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302 Cards in this Set
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A 10 year-old girl presents to the emergency department complaining of nausea, emesis, fatigue, and abdominal pain. Her mother states that her thirst is unsatiable and has been urinating much more frequently than usual. She has also been losing weight over the last 3 weeks. On examination, the patient appears tired. She has dry mucous membranes and delayed capillary refill. Her abdomen is soft with no masses. Bedside glucose > 500 mg/dL. Laboratory analysis reveals serum glucose of 628 mg/dL, arterial pH of 7.20, serum carbon dioxide level of 12 mEq/L, and urinalysis is positive for ketones and glucose. What is the first step in management? AAdminister long-acting insulinBAdminister bolus of sodium bicarbonateCAdminister dextrose infusionDAdminister a 20 mL/kg bolus of 0.9% saline |
Administer a 20 mL/kg bolus of 0.9% saline |
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DKA, whats the first step of management |
Administer a 20 mL/kg bolus of 0.9% saline this is to try and ensure proper hydration - fluid resus is critical in DKA and is thus the first initial step of management |
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treatment of DKA |
should address correction of fluid loss with IV fluids, correction ofhyperglycemia with insulin, correction of electrolyte disturbances and acid-base balance and treatment of concurrent infection if present. Fluid resuscitation is always the first best step in management of DKA |
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A 5-week-old boy presents to the emergency department with progressive nonbilious projectile vomiting for one week. Past medical history reveals that he was born full term without any complications and has been growing well. Physical examination reveals a palpable olive-shaped mass in the epigastrum. Which of the following laboratory findings is expected?AHyperglycemic metabolic acidosis BHypochloremic hypokalemic metabolic alkalosis CHyponatremic hypochloremic metabolic alkalosis DHypernatremic hyperchloremic metabolic acidosis EHyponatremic hyperkalemic metabolic acidosis |
Hypochloremic hypokalemic metabolic alkalosis |
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pyloric stenosis would result in prolonged vomiting resulting in |
Hypochloremic hypokalemic metabolic alkalosis |
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how does prolonged vomiting cause hypochloremic hypokalemic metabolic alkalosis |
Prolonged vomiting and resultant loss of gastric hydrochloric acid (HCI) produces hypochloremia and metabolic alkalosis. In an attempt to maintain acid-base balance, the cellular hydrogen/potassium pump releases hydrogen into theextracellular space. This outflux of hydrogen causes a reverse influx of potassium via the pump, which reduces extracellularpotassium levels and causes hypokalemia. |
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manament of pyloric stensosis |
correct electrolyte disturbance surgical pyloromyotomy |
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Prolonged vomiting and resultant loss of |
gastric hydrochloric acid (HCl) produces a hypochloremic,hypokalemic metabolic alkalosis. |
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A diagnosis of juvenile rheumatoid arthritis includes all of the following criteria except:AOnset before age 16BExclusion of other causes of arthritisCArthritis lasting for at least 6 weeksDPositive rheumatoid factorEArthritis in at least one joint |
Positive rheumatoid factor Not all subtypes of JRA are RF positive. |
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juvenile RA definition |
arthritis lasting for at least 6 weeks with onset before age 16, affecting 2 or more joints |
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juvenille RA thought to be due to |
autoimmune origin |
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classic signs of juvenille RA |
persistent (≥16 weeks) joint pain, swelling, and stiffness, but JRA can be associated withiridocyclitis, generalized adenopathy, splenomegaly, rash, and fever lasting more than a few days |
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There are subtypes of JRA corresponding to degree of involvement: |
systemic-onset JRA (Still's disease), polyarticular-onset JRA, and pauciarticular-onset JRA. RF and ANAs should be tested in patient's suspected of having JRA as they may help to distinguish between subtypes. RF and ANA are absent in Still's disease, ANAs are present in up to 75% of pauci-articular cases, and RF is often positive is polyarticular JRA. |
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A 6-year-old boy presents to the pediatrician's office with 4 weeks of fatigue and shortness of breath. His medical history is notable for poor follow-up with yearly check-ups, but his mother notes that he has been fairly healthy for the duration of his life, except for a "bad sore throat that needed antibiotics" about 6 months ago. On examination, the boy appears fatigued but is appropriately oriented and responsive, and is generally non-toxic appearing. He is afebrile. Cardiac examination is notable for a widely split S2, a quiet S1, and point of maximum impulse displaced to the left. What is the next best step in the management of this patient?AReassurance & routine careBDigoxinCShort-term course of clindamycinDIM CeftriaxoneELong-term penicillin |
Long-term penicillin |
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rheumatic heart disease is caused by |
autoimmune cross-reactivity following a Streptococcal infection. Development of antibody-mediated mitral valve damage is common. Long-term penicillin is suitable for acute treatment and prophylaxis from complications. |
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whats given by acute rheumatic heart disease and prophylaxis from complications |
long term penicillin |
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A 5-month-old infant with distinctive facies and palmar creases develops acute lymphoblastic leukemia. Chromosomal studies would be most likely to reveal which of the following? ATrisomy 21 B45, XO CTrisomy 18 D47, XXY ETrisomy 13 |
Trisomy 21 |
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with Down syndrome children having a roughly 300-fold increased chance of developing childhood leukemia (both acute lymphoblastic leukemia andacute myeloid leukemia) compared with their unaffected peers. The leukemia can even develop in utero. |
true |
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other associations of DS |
hypothyroidism, undescended testes with risk for later tumor development, severecongenital heart disease (notably endocardial cushion defects and ventricular septal defects), duodenal atresia, and, in later life, early onset Alzheimer disease. |
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A 5-week-old child is seen after experiencing multiple long bone fractures. The parents explain that the patient has been seen by multiple pediatric orthopedists after three fractures in the past week. Physical examination is normal, but a hearing test reveals significant hearing loss. Which is the most likely diagnosis in this patient?ARickets BAlport’sdisease COsteomalacia DChildabuse EOsteogenesis imperfecta |
Osteogenesis imperfecta |
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Patients with osteogenesis imperfecta typically have a |
history of multiple fractures with minimal trauma. Physical examination may reveal hearing loss and blue sclera. The condition is caused by abnormal Type I collagen. |
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osteogenesis imperfecta manifests as |
multiple fractures with minimal trauma, hearing loss, and blue sclera. |
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A 17-year old boy comes in to your office complaining of allergic rhinitis. He tells you he gets these symptoms every September when the new school year starts. His medical history is significant for childhood asthma, and his family history is strongly positive for eczema.Which type of medication will allow him to best manage his symptoms as of now? AIntranasal antihistamines BIntranasal cromolyn sodium CIntranasal decongestants DIntranasal glucocorticoidsEOral antihistamines |
D Intranasal glucocorticoids Topical intranasal glucocorticoids are currently known to be the most effective way to manage moderate allergic rhinitis, and they have a better safety profile compared to oral corticosteroids (i.e. fewer systemic adverse effects). |
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Allergic rhinitis features |
Allergic rhinitis presents as allergy-type symptoms most often involving rhinorrhea, sneezing, problems with smell, and itching affecting the nose, mouth, eyes, throat, and skin. It is triggered by an allergen such as pollen, mould, dust, or petdander. This boy's allergies apparently have a seasonal onset - common in allergic rhinitis, and the reason why a subset is also called seasonal allergic rhinitis. |
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allergic rhinitis treatment |
The most effective treatment is to avoid trigger environments, however medications can be very effective depending on the severity of the symptoms. Antihistamines are effective for short-term relief. Topical intranasal corticosteroids are currently known to be the most effective way to manage moderate allergic rhinitis, and they have a better safety profilecompared to oral corticosteroids (i.e. fewer systemic adverse effects). Other treatment options include decongestants, nasal washes, leukotriene inhibitors, and, if symptoms are unremitting/refractory, immunotherapy. |
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treatment for mild allergic rhinitis |
intranasal antihistamines? |
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treatment for moderate allergic rhinitis |
intranasal CS |
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Depending on the severity of symptoms, environment-avoidance, nasal washes, medications, and immunotherapy are available options for treatment of allergic rhinitis. However, for moderate symptoms, topical (spray) intranasal corticosteroids are the most effective management. |
true |
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A new mother brings her 10 day old son in for evaluation of a pink and watery eye. Mother admits she had no prenatal care and is worried something might have happened in utero. She said that the child was born in a well-respected local hospital, though. Patient's vitals are blood pressure 70/40 mmHg, pulse 130, and temperature 37C. On physical exam, the infant's right eye is pink, mildly swollen and with watery discharge. A culture of the fluid is taken. What is the next step in managing this patient?A0.5% erythromycin ophthalmic ointmentBOral erythromycinCOral ceftriaxoneDWarm compressesEContinue to monitor as outpatientFSilver nitrate eye dropsGHospitalization and ceftriaxone |
Oral erythromycin |
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The first line treatment for suspected chlamydial conjuncitivits is |
oral erythromycin; however a high level of suspicion should remain for other sexually transmitted infections, like Chlamydia, HIV, or syphillis. |
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chlamydial conjunctivities notes |
This patent's mother had no prenatal care and, therefore, possibly had an undiagnosed health condition while pregnant, such as an STD. After birth, neonates are treated with prophylactic 0.5% erythromycin ophthalmic ointment to preventgonococcal ophthalmia neonatorum. Silver nitrate was initially used, but it is currently used infrequently in developed countries, as it is more likely to cause chemical irritation of the eyes.Gonococcal conjunctivitis is typically seen 2-5 days after birth and manifests as a purulent conjunctivitis with swollen eyelids. Diagnosis can be made by culture of the exudate, which shows gram negative diplococci. These infants should also be evaluated for co-infection with Chlamydia and other sexually transmitted diseases (HIV and syphillis). Treatment for gonococcal infection is a single dose of intravenous or intramuscular ceftriaxone, and topical erythromycin alone is ineffective in confirmed infections. Depending on the severity of the conjunctivitis, hospitalization may be warranted for monitoring.This neonate most likely has Chlamydial conjunctivitis, which can be seen days about 5-14 days after birth. These patients can present with a range of findings from mild swelling and watery discharge to marked eyelid swelling andchemosis. Culture of exudate is the gold standard for diagnosis this condition, though nucleic acid amplification tests may yield results more quickly. Treatment for this infection is with 14 days of oral erythromycin. Untreated conjunctivitiswill persist and can cause corneal scarring, so outpatient monitoring without treatment would not be recommended. Also, followup is warranted, as oral erythromycin is only 80% effective as a first treatment, and a second course is sometimes necessary. |
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A 30-year-old female who is known HIV positive gives birth to a healthy term infant. The mother received appropriateprenatal care and required Zidovudine with Nevirapine prior to her C-section delivery. What is the best course of management for the healthy infant? AAdmit to NICU for evaluation of cardiovascular symptomsExplain BRun HIV western blot on infant to determine if a transfer of infection occurredWestern blots use antibodies in testing. Hence, it cannot be used because infants born to HIV positivemothers are always seropositive due to maternally derived IgG antibodies to the virus. Both the ELISAand Western blot will be unhelpful in children under 18 months.COrder a 6 week course of Zidovudine for the infantExplainDPerform Chest X-ray to evaluate for possible Lymphocytic Interstitial Pneumonia (LIP)LIP is one of the common respiratory manifestations of AIDS in the pediatric population. In this scenario, however, the baby is healthy and asymptomatic, and requires no additional radiographs to check for respiratory manifestations such as LIP or PCP.ENo additional management is needed as child has no symptoms and unlikely to have HIV |
Order a 6 week course of Zidovudine for the infantAlthough the transmission of HIV from mother to infant has decreased due to the administration of antiretroviralmedications, this decrease is only noted when followed by a 6-week treatment of the infant with Zidovudine. Studies have shown that treatment of the infant has decreased the vertical transmission rate to about 2%. The currentstandard of care is to start infants from HIV positive mothers on 6 weeks of Zidovudine, NNRTI or protease inhibitors. |
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If a mother with HIV, Even if mom has been treated with ARVs, the neonate must also receive a 6-week course of ARV treatment to maximally prevent chance of vertical trasmission. |
TRUE |
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Although the transmission of HIV from mother to infant has decreased due to the administration of antiretroviralmedications, this decrease is only noted when followed by a 6-week treatment of the infant with Zidovudine. Studies have shown that treatment of the infant has decreased the vertical transmission rate to about 2%. The current standard of care is to start infants from HIV positive mothers on 6 weeks of Zidovudine, NNRTI or protease inhibitors. |
TRUE |
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7 year old Jordan comes to the emergency room with severe pain in his right elbow after he tripped and fell on the sidewalk. The arm is visibly deformed and the x-ray confirms a fracture of the distal portion of the humerus. As you explain how you are going to fix his arm, he casually states that this is the 5th cast he's needed. Probing further, you discover that he has had multiple previous fractures with minor trauma, and he is often covered in bruises from the tiniest bumps. You go back and do a thorough physical exam and you find that the whites of his eyes have a bluish tint and he wears hearing aids. He has mild scoliosis. What is the pathophysiology underlying his condition?AMutation in the fibrillin 1 geneBMutation in the collagen 3 geneCMutation in type 1 collagenDMutation in the dystrophin geneEMutations in the fibroblast growth factor gene |
Mutation in type 1 collagen dx = osteogenesis imperfecta |
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features of osteogenesis imperfecta |
There are 4 diffferent types of osteogenesis imperfecta (OI), all of which are caused by a mutation in the collagen type 1 gene. Collagen 1 is a component of bone, ligaments, dentin and sclera, therefore all these tissues are affected. Type 1 OI is the most common and mildest form, which presents with multiple fractures and easy bruising, but is not severely deforming. It can also be associated with hearing loss and dentinogenesis imperfecta (dysplasia and discloration of the teeth). The most characteristic sign pointing to the diagnosis of osteogenesis imperfecta in this case is the blue sclera, which occurs because the sclera is thinner than normal due to the deficiency of collagen, so that the underlying tissue can be seen. The other conditions listed above do not cause this manifestation. |
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Probably the greatest collagen mnemonic of all time: Strong, slippery, bloody, bowel movement. |
Strong = Type 1 collagen, found in bone, dentin Slippery = Type 2 collagen, found in hyaline cartilage Bloody = Type 3 collagen, found in blood vessel walls Bowel Movement = Type 4 collagen, found in Basement Membranes |
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Osteogenesis imperfecta is a condition where the collagen type 1 gene is mutated. Type 1 OI is the most common and mildest form, which presents with multiple fractures and easy bruising, but is not severely deforming. |
true |
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A 14-year old girl is brought in to see you by her mother for evaluation of an outer ear infection. The girl tells you that she is on the high-school swim team and that several of her teammates have developed ear infections and pruritic,erythematous rashes as well. What's the most likely organism responsible for these infections?AStaphylococcus epidermidisNot classically associated with otitis externa from swimming.BPseudomonas aeruginosaPseudomonas aeruginosa often colonizes water that is not changed frequently or is otherwise unclean, and can cause otitis externa and pseudomonas folliculitis - the rash described in the vignette.CEscherichia coliNot classically associated with otitis externa from swimming.DStreptococcus pneumoniaeExplain |
Pseudomonas aeruginosaPseudomonas aeruginosa often colonizes water that is not changed frequently or is otherwise unclean, and can cause otitis externa and pseudomonas folliculitis - the rash described in the vignette. |
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otitis externa two most common organisms |
Pseudomonas aerginosa and staph. aureus |
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otitis externa |
Otitis externa (outer ear infection) is also called "swimmer's ear" for a reason! First, constant water exposure to the ear canal can reduce the amount of cerumen (earwax) that is present, leading to maceration of the canal. Pseudomonas aeruginosa and Staphylococcus aureus are two types of bacteria that flourish in (particularly unclean) water, and will have an easier time invading macerated skin. Furthermore, the outer ear canal is normally slightly acidic (low pH). Frequent exposure to water can slightly raise the pH, making the canal more favourable for bacterial overgrowth by Pseudomonas or S. aureus. |
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A 3-year-old boy is brought to your office by his mother, who reports that he has been coughing for the past 3 weeks. The cough seems to come in bouts lasting several minutes. The coughing is sometimes severe enough to cause him to vomit. About 6 weeks ago, she reports that he had a very mild cold that went away on its own, and that this new cough seemed to happen spontaneously. The child's past medical history is benign, with no previous serious infections or illnesses. On exam, the child has a fever of 38˚ C (100.4˚ F) and otherwise normal vital signs. His oropharynx is erythematous but does not have any frank lesions or exudates. Lungs are clear to auscultation, and the cardiac exam is benign. A chest X-ray is obtained and is also normal. What is the most likely diagnosis in this patient?APertussisBTuberculosisCBacterial pneumoniaDReactive airway diseaseERhinovirus |
A Pertussis |
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The 3 phases of pertussis: |
the catarrhal phase (like the common cold), the paroxysmal phase (bouts of coughing), and the convalescent phase (resolution). Treatment is largely supportive, but antibiotics can reduce the duration of symptoms. |
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A newborn born at 37 weeks by C-section to a diabetic mother is found to have dyspnea an hour after delivery with a respiratory rate of 70. Exam shows nasal flaring and slight acrocyanosis. Chest X-ray reveals perihilar streaking and patchy infiltrates. What is the pathophysiology of his condition?AInfectionBIncreased insulin levelsCDeficient levels of surfactantDRetained fetal alveolar fluidECongenital heart abnormality |
D Retained fetal alveolar fluid |
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Deficient levels of surfactant |
This is the cause of neonatal respiratory distress syndrome. Patients are normally more premature and CXR shows diffuse ground glass opacities. |
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Transient tachypnea of the newborn (TTN) is a relatively benign condition that is caused by |
a failure of adequate clearance of fluid from alveoli at birth. Treat with a few days of FiO2. |
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Transient tachypnea of the newborn (TTN) CXR findings |
shows diffuse perihilar streaking due to vascular swelling, patchy infiltrates, and sometimes air trapping (darker lung fields). |
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A newborn child is being examined for concerns of ambiguous genitalia. Vital signs include a pulse of 155/min and ablood pressure of 60/40 mm Hg. Genital examination reveals blind ending vaginal pouch and clitoris measuring 1.5 cm. Masses are palpable in the inguinal canals bilaterally. Serum laboratory studies are shown below: Na: 140 mEq/L K: 4.5 mEq/L Cl: 110 mEq/L HCO3: 25 mEq/L Ultrasonography shows the absence of the uterus or fallopian tubes. Which of the following is the most likely karyotypeand hormonal abnormality in this patient?AKaryotype XY with elevated ratio of testosterone to DHT BKaryotype XX with decreased 17-hydroxyprogesteroneExplainCKaryotype XX with elevated ratio of testosterone to DHTExplainDKaryotype XY with elevated 17-hydroxyprogesteroneExplainEKaryotype XX with elevated 17-hydroxyprogesteroneExplain |
Karyotype XY with elevated ratio of testosterone to DHT |
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An XY patient with elevated 17-hydroxyprogesterone would likely have congenital adrenal hyperplasia due to 21-hydroxylase deficiency. The external genitalia would be normal, and the child would not be expected to have a blind vaginal pouch as seen in this infant. Also, electrolyte abnormalities, particularly hyponatremiaand hyperkalemia, are frequently associated with these abnormalities. |
true |
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A 12-year-old boy presents to your clinic for a yearly check-up. Neither he nor his mother have any new issues since their last visit 1 year ago. At the end of the visit, you review his growth curves, which show that his weight and weight-for-height have crossed several percentile lines upward; his weight and weight-for-height are now both at the 95th percentile. Which of the following treatments is indicated for children like this one?AVery-low-calorie dietBMaintenance of weight with increasing ageCLoss of weight as rapidly as possibleDPharmacologic treatment with appetite-suppressing agentsESurgical treatment with gastric banding or bypass |
B Maintenance of weight with increasing age |
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whats the approach to an obese child |
The ideal way to approach obesity in children is not so much weight loss as it is maintenance of weight as the childgrows in height. Introducing healthy foods and regular exercise are key. |
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A 7-year-old boy is brought to the urgent care clinic by his mother, who noticed "bumps on his bottom." He has no major past medical history, but is unvaccinated. Upon interviewing the child and his mother, you find out that he has also been having 5/10 pain in both of his knees, as well as a dull stomach ache that does not localize to any one quadrant, and that seems to come and go. The rash appeared about 1 week before the onset of the stomach and joint pain. On exam, you note diffuse tenderness of the abdomen to palpation, but no distention or masses were palpated. Adermatologic survey reveals palpable purpura over the buttocks and upper thighs. What is the most important sequela of the pathology affecting this patient?APeripheral neuropathyBRenal failure requiring dialysisCSeizure disorderDUveitisEIntussusception |
EIntussusception |
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HSP is associated with |
intussusception |
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HSP is an IgA-mediated vasculitis that presents with a classic pattern of lower body rash, abdominal pain, andarthralgia. The most important complication not to be missed in children with HSP is |
intussusception |
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A three-month-old male is brought to the emergency department by his mother for epistaxis of 2 hours' duration. The mother denies any trauma and reports the infant was sleeping when she noticed the nose bleed. Past medical history is significant for severe eczema and multiple episodes of thrush. Physical examination reveals active bleeding of the left nasal septum, diffusely dry skin, and multiple, small, red spots measuring 1-2 mm on the lower abdomen. What is the most likely diagnosis? Avon Willebrand disease BWiskott-Aldrich Syndrome ExplainCRocky Mountain Spotted Fever ExplainDImmune thrombocytopenic purpura ExplainEMeningococcemia |
Wiskott-Aldrich Syndrome |
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Wiskott-Aldrich Syndrome |
von Willebrand disease is an autosomal dominant disorder caused by qualitatively or quantitatively deficient levels of von Willebrand factor. Patients commonly present with easy bruising, recurrent epistaxis, menorrhagia, or prolonged bleeding after surgery or dental extraction. Family history may reveal relatives with clotting disorders. Laboratory findings reveal prolonged activated partial thromboplastin (aPTT) in severe disease as a result of concurrent factor VIII deficiency. Von Willebrand factor normally binds to and protects factor VIII from rapid breakdown in the blood. |
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Wiskott-Aldrich syndrome is an X-linked recessive disorder characterized by the clinical triad ofthrombocytopenia, eczema, and immunodeficiency. On the board exam, look for a male child with petechiae, dry skin, and a history of recurrent infections. |
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ITP |
Immune thrombocytopenic purpura is caused by autoantibodies formed against specific platelet membrane glycoproteins. These patients have an isolated thrombocytopenia caused by peripheral platelet destruction and present with petechiae, purpura, bruising, epistaxis, and menorrhagia. |
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N. meningitidis is a gram negative |
diplococcus |
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Meningococcemia is caused by |
dissemination of Neisseria meningitidis into the bloodstream.Neisseria meningitidis is a Gram-negative diplococcus that is spread by aerosol droplets and contact with respiratory secretion. Patients often have viral-like symptoms such as fever, sore throat, and rhinorrhea before the development of overt septic shock. Key features of fulminant meningococcemia include prominent petechiae and purpura, hypotension, DIC, acidosis, adrenal hemorrhage,multiorgan failure, and possible coma. A large percentage of these patients also develop meningitis. On the board, look for a college student to present to the emergency room with these symptoms. Treatment includes a combination of dexamethasone, vancomycin, and ceftriaxone. |
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A 16-year-old African American female is brought to the emergency room after a motor vehicle accident. Her past medical history is significant for recurrent diarrhea and frequent sinus infections that she manages with saline rinses. Her vitals are as follows: HR 125 bpm, BP 90/40 mmHg, RR 25/min. On examination, her extremities are cool and her conjunctiva appear pale. Her lab values are as follows: hemoglobin: 9 g/dL, hematocrit: 38%, WBC: 4,000/mm3, PLTs: 90,000/mm3.She is rapidly transfused with several units of whole blood. Within a few minutes, the patient develops dyspnea and her blood pressure drops. Epinephrine is administered and she is aggressively resuscitated with fluids and pressors. After recovery, what condition is this patient at increased risk for? ALymphomaBPeripheral neuropathyCUlcerative colitisDGastric cancerEEndocarditis |
Ulcerative colitis |
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Selective IgA deficiency |
is the most common primary immunodeficiency. It is often asymptomatic, but since IgA is key in safeguarding our mucosal barriers, these patients are at risk for recurrent sinopulmonary and gastrointestinal infections. Beware of anaphylactic reactions to transfused blood contained IgA antibody. |
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A 10-year-old female presents to clinic for her well child exam. She has high foot-arches, and she has an Xray that shows the following:Credit: Wikimedia CommonsWhen you see her in clinic, she is developmentally age-appropriate. The mother states that her father, paternal grandmother, and paternal aunt also have similar foot and back problems. What is the most likely etiology for this patient's condition?ADeficiency of galatactocerebrosidase BArysulfatase A deficiencyI'm SureFeeling LuckyNo ClueCDefective production of proteins involved with structure and function of peripheral nerves or myelin sheathDInflammation of CNSEInflammation of endoneurium |
C Defective production of proteins involved with structure and function of peripheral nerves or myelin sheathThis is characteristic of Charcot-Marie-Tooth disease. |
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Charcot-Marie-Toothe Disease |
is a genetically heterogenous disease of proteins related to peripheral nerve axons and the myelin sheath that commonly presents with high arches and scoliosis. |
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You are informed by an obstetrician at your hospital that one of her patients is in active labor. She also notes thatprenatal ultrasound revealed that the baby has hypoplastic left heart syndrome (HLHS). Which of the following otherwise pathological heart lesions will be necessary to ensure the initial survival of this child?AConotruncal defectBAtrial septal defect aloneCRight ventricular hypertrophyDPatent ductus arteriosus aloneEPatent ductus arteriosus and atrial septal defect |
E Patent ductus arteriosus and atrial septal defect |
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A child with born with hypoplastic left heart requires both a patent ductus arteriosus and an atrial septal defect to maintain life. |
true |
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A 7-year-old boy recently discharged from the hospital returns to the emergency room with several days of diarrhea. He had been treated for a systemic infection with broad spectrum IV antibiotics. What is the most likely etiology?AC. difficileBDrug toxicityCResistant gut pathogenDNew community-acquired infectionEFactitious disorder |
C. difficile |
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Post-antibiotic diarrhea should make you think of |
Clostridium difficile overgrowth causing colitis. |
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Penicillins,cephalosporins, clindamycin, macrolides, and fluoroquinolones have all been linked to C. difficile colitis. The risk of C. difficile colitis is particularly elevated after hospitalization for antibiotic treatment. |
true |
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A fourteen-year-old male is brought to the office by his father with the complaint of being the shortest student in this grade. The father states that both he and the patient’s mother were “late bloomers” and did not achieve average height until 17 years of age. Past medical history is unremarkable. The growth curve reveals that the patient was at the 50th percentile for height and weight at birth, experienced a gradual deceleration in growth velocity until 3 years of age, and has maintained a normal growth velocity at the 3rd percentile curve since. Physical examination reveals slightly immature physical appearance for acne and Tanner stage 2 genitalia and pubic hair. What is the most likely diagnosis?AKlinefelter syndrome BConstitutional growth delay CFamilial short stature DHypopituitarism EKallmansyndrome |
Constitutional growth delay |
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Constitutional growth delay is the most common cause of |
prepubertal short stature and pubertaldelay. The parents of these parents will often describe themselves as “late bloomers.” |
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delayed puberty |
Delayed puberty is defined in boys as the absence of testicular enlargement after 13 years of age and in girls by the absence of breast growth after 12 years of age. Constitutional growth delay is a variant of normal growth and is the most common cause of prepubertal short stature and pubertal delay. Patients are normal-sized at birth but shortly thereafter experience a deceleration in linear growth that results in the downward crossing of growth percentile until approximately 3 years of age. After 3 years of age, these individuals resume a normal growth velocity for the remainder of their prepubertal years. At the expected time of puberty, these individuals appear to “drop off” the growth curve because of the delay in onset of the pubertal growth spurt in relation to their peers. The onset of puberty, the pubertalgrowth spurt, and catch-up growth occur alter than average in these patients, and they eventually attain normal adult stature and sexual development. The bone age X-ray of these individuals are often younger than their chronologic age. On the boards, constitutional growth delay is often presented as a child whose parents were “late bloomers” and have average adult height. |
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A six month old male comes to your office for a routine check-up. You are not able to palpate the right testis in the scrotum, but the remainder of the physical exam is normal. Looking back in his charts, you see that this same finding was noted on his discharge from the newborn nursery. He has missed most of his follow up appointments since then. Which of the following is the most appropriate management of his condition?AThe undescended testis should be removed prophylactically as there has likely been some malignant changeBSurgical correction can be done electively for cosmetic effects, but it is not necessaryCNothing needs to be done. The testes will spontaneously descend by 2 years of age.DThe testes should be surgically fixated as soon as possible to allow easy screening for malignancy and increase his chances of fertility in the futureEThe testes should be surgically fixated to reduce the risk of malignancy, but he will be infertile |
The testes should be surgically fixated as soon as possible to allow easy screening for malignancy and increase his chances of fertility in the future |
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Cryptorchidism is the failure of one or both testes to descend into the scrotum by birth, and increases risk of infertilityand testicular malignancy. Spontaneous descent of the testes will occur in the majority of infants within the first 3 months of life, but fewer than 1% descend after 1 year. Orchiopexy between 6-12 months of life increases the likelihood that fertility will be preserved. |
true |
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A 1-year-old boy underwent scheduled, open surgical repair of a large ventricular septal defect. The surgery was uncomplicated. On post-operative day 2, the child develops dyspnea and is noted to have muffled heart sounds on exam. Rate and rhythm are regular; there are no extra heart sounds. The lungs are clear to auscultation bilaterally. The wound is closed, non-exudative, and non-erythematous. What is the most likely diagnosis in this child?APericardial effusionBRespiratory depression due to analgesic regimenCRespiratory depression due to inhaled anestheticDRe-opening of defectESurgical site infection |
A Pericardial effusion |
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Muffled heart sounds in the days following major heart surgery should always make one think of a developing |
pericardial effusion. An echo should be done to characterize the extent of the effusion. |
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Heart surgery is a major risk factor for |
pericardial effusion in children and adults |
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A 5-year-old male is brought to the emergency department by ambulance after being found unresponsive.History reveals the patient was found in a bathroom of a neighbor's home where he was being watched for the day. Several prescription medication bottles were opened and next to the patient including acetaminophen, clonazepam, glyburide,and metoprolol. Laboratory studies reveal blood glucose of 110 mg/dL. Physical examination reveals an unresponsive male with no signs of external trauma.Pupils are 4 mm, equal and reactive to light bilaterally. Vitals reveal a blood pressure of 110/60 mmHg, heart rate of 120 beats/min, respiratory rate of 6 breaths/min, and an oxygen saturation of 99% by non-rebreather mask. Which is the most likely cause of all the symptoms in this patient?AEpidural hematoma BAcetaminophen overdose CBeta-blocker overdose DSulfonylurea overdose EBenzodiazepine overdose |
EBenzodiazepine overdose |
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bendo OD |
Benzodiazepine overdose can present as an unresponsive patient with otherwise normal physical examination. This patient has a normal glucose, normal vital signs except a slightly reduced respiratory rate, and no signs of head trauma. Given his normal sized pupils and generalized obtunded state, a clonazepam overdose should be suspected. |
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Typical symptoms of BZD overdose include |
decreased mentation, delirium, headache, weakness, and slurred speech. Signs include mild respiratory depression,hypothermia,dilated pupils, and hyporeflexia. This patient has a normal glucose, normal vital signs except a slightly reducedrespiratory rate, and no signs of head trauma. Given his normal sized pupils and generalized obtunded state, aclonazepam overdose should be suspected. |
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A twelve year old boy is brought to his pediatrician for a well-child visit. He is generally healthy, but his grades in school have recently slipped. On further questioning, his mother says that his teachers have all commented on his inability to pay attention in school. He stares blankly into space for a few seconds and doesn’t seem to listen to instruction. EEG shows a 3 Hz spike and wave pattern. The boy is prescribed the first line medication for this condition. What is the mechanism of action of this medication? ABlocks T-type calcium channelsBInhibits GABA reuptakeCInhibits GABA transaminaseDBlocks voltage-gated sodium channelsEIncreases chloride channel opening frequency |
Blocks T-type calcium channels |
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1st line tx for absent seizures |
ethosuzimide next valproic acid |
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moa of ethosuzimide |
works by blocking T-type calcium channels in the thalamus that might normally sustain the rhythm of discharges during a seizure. |
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AE of ethosuzimide |
It has few side effects, including Gi distress, fatigue, headache, and rarely, Stevens-Johnson syndrome. |
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A 15-year-old male presents to the emergency department with the complaint of a bee sting. History reveals the patient has been stung once before with only a local reaction. Physical examination reveals audible wheezes, edema of the posterior pharynx and diffuse cutaneous hives. Which is the most appropriate initial step in management in this patient?ADiphenhydramineBIntramuscular epinephrine 1:10,000CFamotidineDRanitidineEIntramuscular epinephrine 1:1,000 |
E Intramuscular epinephrine 1:1,000 |
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Intramuscular administration is the dilution of 1:1,000. Intravenous infusion ofepinephrine is required the 1:10,000 formulation is used. |
true |
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anaphylactic medication notes |
This patient is presenting with an anaphylactic reaction to a bee sting. Findings typically include cutaneous urticaria or angioedema, dyspnea, wheezing or airway obstruction as well asgastrointestinal and cardiovascular manifestations. The most important steps in management ofanaphylaxis is airway stabilization followed by administration of epinephrine and obtainingintravenous access. The type of epinephrine used with intramuscular administration is the dilutionof 1:1,000. When an intravenous infusion of epinephrine is required the 1:10,000 formulation is used. It has also been shown that simultaneous H1 and H2 receptor blockade may be superior to H1 blockade alone. For this reason diphenhydramine plus an H2 blocker are commonly administered along withintravenous fluids and methylprednisilone. |
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A five-week old baby boy is brought to the hospital after continuous, non-bilious projectile vomiting. His mother says he has not been able to keep food down for the past few days and that he seems weak. On physical exam, the abdomen is non-distended but a small mass can be palpated below the costal margin. Which of the following is the most likely diagnosis?AAnnular pancreasBIntestinal volvulusCTracheoesophageal fistulaDIleal atresiaECongenital pyloric stenosis |
Congenital pyloric stenosis |
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A newborn female is born at 40 weeks to a mother who had routine prenatal care. A Cesarean-section was performed after some complications occurred during delivery. On physical exam on day 2, the infant is found to have a pustular rash on a red base on her face and blue-gray marks on her lower back. She was also found to have a shorter left leg and asymmetrical skin folds in the groin. What is the next step in caring for this patient?APelvic USBOrtolani and Barlow ManeuversCTopical SteroidsDCall Child Protective ServicesEContinue to monitor patient as normal |
Ortolani and Barlow Maneuvers |
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Unilateral leg shortening and abnormal skin fold in a newborn should raise suspicion for developmental dysplasia of the hip. Both Ortolani and Barlow maneuvers can be helpful for detecting newborns with this anomaly. |
true |
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A 6-year-old girl is admitted to the hospital for 48 hours of vomiting, the etiology of which is unknown. This child has been admitted to the same hospital 3 times within the past 6 months for the same complaint. While in the hospital, however, the vomiting tends to stop soon after admission. Aside from the recent string of hospitalizations, her past medical history is benign. She is up to date on all of her vaccinations. Extensive screening for ingested toxins and infectious etiologies has yielded no answers. Imaging has been similarly benign. Her mother seems very eager to figure out what is going on, and is very involved in the care of her child; she stays in her daughter's room during every admission. After ensuring proper fluid and electrolyte balance, what is the next best step in the management of this patient?AFamily consultation with psychiatryBRepeat imaging of the head and abdomenCConsultation with genetic specialistsDConsultation with neurosurgeryE24-hour monitoring and discharge with instructions to follow up with their primary doctor |
Family consultation with psychiatry |
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Factitious disorders hinge on the desire to receive medical attention, and in Munchausen disorder by proxy, it is the parent or caregiver of a child feigning symptoms in the child. |
true |
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A previously healthy 10-year-old Asian boy presents to your office with fever, sore throat, and cough over the past week. He denies any cold symptoms, rhinnorhea, diarrhea, or changes in appetite. His mother notes that he has lost weight since the last time he saw you about 6 months ago for immunizations and a well child visit. Previously, his weight was at the 35th percentile, and currently, he is at the 20th percentile. On physical exam he is afebrile, breathing comfortable with no nasal discharge, and has a clear posterior oropharynx. He has significant non-tender cervical lymphadenopathy bilaterally. His heart has a regular rate and rhythm with no murmurs, rubs or gallops, but he has diminished breath sounds at the right base. Besides sending the child for a chest x-ray, what is the next best step in diagnosing the patient?ARapid strep throat testBViral panel nasal swabCLymph node biopsyDTuberculin skin testEBartonella serology |
Tuberculin skin test |
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A 4 year- old girl with repeated episodes of runny nose presents to your office. Her mother is concerned that her daughter seems to have a persistent postnasal drip even during the summer months. Although the child is up to date on all her immunizations, she has also had about 8 episodes of pneumonia in the winter, which lasted a few weeks at a time. On physical exam, she is afebrile and generally breathing comfortably, coughing slightly with expiratory wheezes and has a runny nose. She also appears underweight. Upon closer nasal inspection, you notice colorless nasal drainage with bilateral nasal polyps. Which of the following diagnostic tests is most likely associated with this clinical scenario?AIndirect Laryngoscopy BChest X-rayCHead CT DSweat Chloride Test ESerum immunoglobulins |
Sweat Chloride Test |
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Cystic fibrosis can present with persistent rhinorrhea, nasal polyps, and increased respiratory infections. The sweat chloride test is the best screening test for cystic fibrosis. |
true |
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whats the best screening test for cystic fibrosis |
sweat test |
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You are in the newborn nursery completing an examination on a female infant who was born 12 hours ago. She was full term and weighed 3650g. She had an uncomplicated vaginal delivery with an Apgar score of 9. She has begun breastfeeding and is latching on well. On examination, you notice that her right foot is not entirely symmetrical to the left one. The right forefoot is adducted, while the midfoot and hindfoot are normal. There is full range of motion at the ankle and at the subtalar joint. What is the most common cause of this foot deformity?AChromosomal anomalyBEhlers-Danlos SyndromeCMaternal use of fluoroquinolone during pregnancyDMaternal diabetesEAmniotic bandFIntrauterine molding |
Intrauterine molding |
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Metatarsus adductus is a positional deformity that is primarily due to intrauterine molding. The majority of cases resolve spontaneously; more severe cases need further intervention. |
true |
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A 7-year old boy presents to the ED with fever, headache and generalized muscle pains, particularly in his calves, for the past 3 days. He also complains of mild abdominal pain and nausea. On exam he appears toxic and there is a rosy, blanching maculopapular rash which began on his wrists and ankles yesterday, and has spread to cover his extremities today. The rash is beginning to involve his palms and soles as well. His mother cannot remember anything unusual in the past couple of weeks, but when probed she recalls removing an attached tick from his leg last week.Which of the following is the best approach to management?AOrder rickettsial antibody titers; initiate analgesics and antipyretics while awaiting resultsExplainBOrder Borrelia titers and start on doxycyclineBorrelia is the causative organism in Lyme disease, and this is not the typical presentation of Lyme.CStart regimen of ChloramphenicolExplainDObtain blood cultures and start regimen of CeftriaxoneExplainEStart regimen of doxycyclineExplain |
Start regimen of doxycycline |
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Although there is the concern of using tetracyclines in children under 8 years old due to the risk of tooth discoloration, this side effect is dose dependent, and the dose of doxycycline used in the treatment of Rocky Mountain Spotted Fever (RMSF) is highly unlikely to cause tooth discoloration. In addition, RMSF can be a life-threatening illness, and the benefit of using tetracycline far outweighs the risk. |
true |
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RMSF is a tick-borne infectious disease. The blanching maculopapular rash occurs after 2-3 days of symptom onset, and begins on the wrists/ankles, later spreading to the extremities, then the whole body. Involvement of palms and soles is pathognomonic. Treat with doxycycline within 4 days of disease onset. |
true |
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tick bites infections tx |
doxycycline within 4 days of onset |
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A 6-day-old boy born to Amish parents has had low hemoglobin levels since day 1 of life. Physical exam reveals moderate hepatosplenomegaly and some skin ulcers. He is diagnosed with pyruvate kinase deficiency. Which of the following is a finding you expect to find on his labs?AIncreased haptoglobinBIncreased osmotic fragility of red blood cellsCReticulocytosisDLow ironEElevated folic acidFDecreased LDH |
CReticulocytosis |
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RBCs are destroyed in the spleen in persons with PK deficiency. Lab values would include elevated LDH, reduced haptoglobin, and reticulocytosis. |
true |
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puruvate kinase deficiency is the most common cause of |
congenital non-spherocytic chronichemolytic anemia. |
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pyruvate kinase deficiency |
Pyruvate kinase (PK) deficiency is the most common cause of congenital non-spherocytic chronichemolytic anemia. This autosomal recessive disorder is caused by a lack of PK in erythrocytes, which lack mitochondria and depend on PK for glucose metabolism and energy production. The cause of hemolysis is unclear, but it may relate to deficient ATP production and failed maturation of RBCs, causing them to be destroyed in the spleen. Pyruvate Kinase deficiency is especially prevalent among Pennsylvania Amish. Diagnosis is usually done by genetic testing, although assay of erythrocytic PK enzymatic activity is a possible test as well. Lab results are consistent with extravascular hemolysis because thehemolysis occurs in the spleen. These would be elevated LDH, reduced haptoglobin, andreticulocytosis. Other signs include hepatosplenomegaly, pigmented gallstones, iron overload(especially, but not only, after repeat transfusions), folic acid deficiency secondary to increased requirements (needed for increased cell production of reticulocytosis), skin ulcers, and sometimes even hydrops fetalis. |
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pk deficiency dx |
genetic testing or assay of pk enz activity |
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A pregnant woman presents to the labor and delivery department; she has received inconsistent prenatal care, with her last visit being 4 months ago. She reports that she does not have any past medical history, and that she has not really had any problems throughout this pregnancy. On admission, her screening labs were notable for a fingerprick glucose of 300mg/dL. A follow-up HbA1c was measured at 7.7%. Ultrasound shows a fetus at the 65th percentile for weight at 39 weeks, which is the gestational age estimated by this mother. This woman's newborn is at greatest risk for which of the following complications?AHemolytic anemiaBType I DiabetesCHyperglycemiaDHypoglycemiaEType II Diabetes |
D Hypoglycemia |
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Excess insulin in a newborn shunts the now-limited glucose supply intracellularly, leading tohypoglycemia. This is why breastfeeding as soon as possible after birth is recommended in IDM. |
true |
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a child who is born who is hypoglycemic may have |
small penis |
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Infants of diabetic mothers (IDM) are at risk for a number of obstetric complications. Of the choices listed above, hypoglycemia would be the most likely outcome for this child. Glucose crosses theplacental barrier, but insulin does not, meaning that the fetus will have been exposed to a high level of glucose in utero. The fetus' pancreas will produce a commensurate amount of insulin to maintain a normal blood glucose concentration. When the baby is born, however, the maternal supply ofglucose ends, leaving the newborn with an excessively high level of insulin in its circulation. This excess insulin (in the setting of normal insulin sensitivity) shunts further glucose intracellularly, leading to hypoglycemia. Hypoglycemia in the newborn can present as jitteriness, hypotonia,irritability, lethargy, apnea, bradycardia, and even seizures. |
true |
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A neonate is seen for respiratory distress six hours after birth. History reveals a premature birth with no complications and full prenatal care. Physical examination reveals a scaphoid abdomen with bowel sounds heard in the lower anterior thorax. Which of the following additional findings is most likely?AFluid accumulation in lungBLung hypoplasiaCBowel sounds more prominent on the rightDAspiration pneumoniaEMeckel's diverticulum |
B Lung hypoplasia |
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pulmonary hypoplasia |
In pediatric pulmonary hypoplasia, infants most commonly present with respiratory distress and cyanosis in the first minutes or hours of life. It can be severe and may be associated with circulatory insufficiency, requiring aggressive resuscitative measures. |
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A father frantically calls your office because he is home alone with the kids and his 4 year old son just started bleeding profusely from his nose. There was no preceding trauma. This has never happened before. Which of the following statements would not be appropriate advice to help this father manage his child's nosebleed?AInsert gauze wetted with drops of nasal decongestant sprayBPinch on the anterior soft portion of the nose for 10 minutesI'm SureFeeling LuckyNo ClueCApply a warm wet washcloth over the nose to relieve the dryness of the nasal passagesDHave the child seated upright and leaning forward |
CApply a warm wet washcloth over the nose to relieve the dryness of the nasal passages |
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Epistaxis is a fairly common presentation in the pediatric population. Managing uncomplicatednosebleeds includes upright + forward-leaning posture, application of pressure to the anterior of the nose, and potentially gauze wetted with decongestant spray. |
true |
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mgmt of epistaxis |
includes upright + forward-leaning posture, application of pressure to the anterior of the nose, and potentially gauze wetted with decongestant spray. |
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5 month-old male presents with vomiting, blood in his stool, and intermittent periods of what appears to be abdominal pain. His parents report attacks where he is very fussy and kicks his legs in the air. Which is the most likely diagnosis in this patient?AIntussusception BHernia ExplainCMeckel diverticulum ExplainDAppendicitis ExplainEVolvulus Explain |
intussusception |
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classic triad for intussusception |
vomiting abdo pain blood per retum |
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A 16-year-old recently immigrated African HIV patient who has not been adhering to his antiretroviral regimen presents to your office with intermittent fevers and headache for the past few weeks. On physical exam he is afebrile with nuchal rigidity and sensitivity to light. One month ago his CD4 count was 20 cells/uL. After doing a lumbar puncture, you report elevated opening pressure of 220mm H2O and you see the following under a microscope following staining. What is the best treatment?Credit: Wikimedia CommonsACorticosteroidsBRifampin with isoniazidCAmphotericin B followed by flucytosine and fluconazoleDFluconazoleEExtended-course oral tetracycline |
Amphotericin B followed by flucytosine and fluconazole |
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Cryptococcus neofromans is a fungal infection that causes meningitis in immunocompromised hosts and is treated with amphotericin B. |
true |
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A 12-year-old boy presents to your office with 8 weeks of leg pain. The pain is mostly located in his thigh and was initially mild; now he says the pain is so bad that he cannot walk on his leg. Neither increasing nor decreasing activity alleviates the pain, which tends to be worse at night. He reports that using NSAIDs help temporarily, but that the pain always returns. He walks with a limp. Both he and his mother deny any recent trauma to the leg. On exam, the patient is afebrile, and there are no tissue changes overlying the leg. An X-ray obtained of the leg is shown. What is the most likely diagnosis of this lesion?Credit: National Library of MedicineAStress fractureBOsteoid osteomaCAcute osteomyelitisDOsteoblastomaEOsteopenic lesion |
B Osteoid osteoma |
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Osteoid osteoma is a benign bone tumor affect usually boys in their 2nd decade. On X-ray, they appear as a nidus of hypodense sclerosis surrounded by hyperdense sclerosis. |
true |
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Necrotizing enterocolitis (NEC) is a disease of the intestines that is most commonly seen in sick and/or preterm neonates. Which of the following is not correct of NEC?ARespiratory distress increases the risk of NECExplainBThe underlying cause of NEC is fetal hypertensionExplainCAir found in the portal vein indicates severe NECTrue.DSepticemia increases the risk of NECExplainELow Apgar scores increase the risk of NEC |
BThe underlying cause of NEC is fetal hypertension The underlying cause of NEC is an initial ischemic injury to bowel wall, leading to bacterial invasion and subsequent necrosis. |
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The underlying cause of NEC is an initial ischemic injury to bowel wall, leading to bacterial invasion and subsequent necrosis. |
true |
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Necrotizing enterocolitis is most commonly seen in sick and/or preterm newborns in the 2nd or 3rd week of life. Bloating, feeding intolerance, bilious vomiting, bloody/loose stools, temperature instability, and sometimes sepsis are key signs to look out for. |
true |
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A 2-day old newborn presents with a fever, followed the next day by a vesicular eruption. He then begins to develop signs of respiratory distress. Three days before his birth, his mother had a similar but less severe rash on her face, trunk, and arms which was itchy. What should have been given to her baby after he was born?AAmpicillin and GentamicinExplainBVaricella-zoster immune globulinCVaricella Zoster vaccineDAcyclovirExplainECorticosteroids |
Varicella-zoster immune globulin |
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Neonatal varicella is a serious infection with infant mortality as high as 30%. Infants born to mothers symptomatic around time of delivery should receive VZIG, and if the infant actually develops NV, should receive a 10-day course of acyclovir. |
true |
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if an infant born to a mother who is symptomatic with varicella how should the infant be managed |
mother should get VZIG around time of delivery |
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if an infant actually develops neonatal varicella due to mother transmission during birth how should you treat the infant |
10 day course of acyclovir |
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A 25-day-old baby girl is brought to the emergency department by her parents with a fever of 100.6˚ F. Her parents have not noticed any other concerning signs or symptoms and physical exam is notable only for mild tachycardia. What should be your next step?ACBC, CMP, UA, blood and urine cultures, lumbar punctureBCBC, BMP, and UACDischarge home and follow up in 1 weekDDischarge home with a list of symptoms that should prompt returnEInpatient observation for 24 hours |
ACBC, CMP, UA, blood and urine cultures, lumbar puncture |
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All children under 60 days old with documented fever and no clear source should receive a fullsepsis workup. |
true |
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A newborn is found on exam to have excessive drooling and respiratory distress. What is the best diagnostic test for the condition that is concerning you?AV-Q perfusion scanBCT scanCPlace feeding tube and take an X-rayDPulmonary Function TestingEBarium swallowFChest xray |
Place feeding tube and take an X-ray |
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In any newborn with respiratory distress and excessive drooling, you must rule out a |
tracheo-esophageal fistula and esophageal atresia |
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esophageal atresia |
is often suggested on prenatal ultrasound withpolyhydramnios. To diagnose, place a naso- or orogastric feeding tube, take an X-ray, and look for coiling of the tube in the atretic esophagus. |
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A flashlight is shown into a newborn's eyes. What principle could potentially explain this finding?Credit: Wikimedia CommonsAAnticipationBIncomplete penetranceCLoss of heterozygosityDCo-dominanceEVariable expressivity |
C Loss of heterozygosity This is described as "two-hit-hypothesis". |
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If a patient inherits or develops a mutation in a tumor suppressor gene, the complementary allele must be deleted or mutates before a cancer develops - this is the 'two-hit' hypothesis, representing a loss of heterozygosity. |
true |
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A 1-day-old male is noted to have feeding intolerance after several episodes of bilious vomiting. His temperature is 36.1C (97.0F), pulse is 190 beats/min, respirations are 30 breaths/min and blood pressure is 80/40 mm Hg. On physical exam, there is a palpable epigastric mass. An abdominal radiograph is performed on which gastric and duodenal air is seen, and a nasogastric tube is placed which returns bilious secretions. Which of the following is most likely the cause of the patient's symptoms? AImperforate anus ExplainBEsophageal atresia Esophageal atresia would present with regurgitation of feeds. It is often associated with a tracheoesophageal fistula. CPyloric stenosis ExplainDDuodenal atresia ECongenital gastritis |
esophageal atresia |
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Obstructions of the GI tract proximal to the ampulla of Vater present with non-bilious vomiting whereas distal obstructions present with bilious vomiting. |
true |
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A 15-year-old male presents to his pediatrician with a two-month history of progressively worsening dyspnea, productive cough, and wheezing. Physical examination reveals bilateral hyperresonance to percussion, decreased breath sounds bilaterally, and distant heart sounds. There is a slight distention of his abdomen along with a palpable abdominal fluid wave. A chest radiograph shows an increased anterio-posterior diameter with bilateral flattening of the diaphragm. Initial laboratory tests show alanine aminotransferase (ALT) of 120 U/L (normal: 10-40 U/L), aspartate aminotransferase (AST) of 100 U/L (10-59 (U/L), total bilirubin of 3.4 mg/dL (0.2-1.3 mg/dL). Which of the following is the most likely diagnosis in this patient?AStatus asthmaticus BPanacinar emphysema CBronchiectasis DViral hepatitis EAlcoholic cirrhosis |
Panacinar emphysema Although AAT deficiency is commonly seen in younger patients, it can still present in middle-aged patients. AAT deficiency is considered a panacinar emphysema, as opposed to centroacinar emphysema secondary to respiratory unit damage fromsmoking cigarettes. Think of AAT deficiency as a differential diagnosis when a patient presents with signs and symptoms of COPD and have never smoked in theirlife. |
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Although AAT deficiency is commonly seen in younger patients, it can still present in middle-aged patients. AAT deficiency is considered a panacinar emphysema, as opposed to centroacinar emphysema secondary to respiratory unit damage from smoking cigarettes. Think of AAT deficiency as a differential diagnosis when a patient presents with signs and symptoms of COPD and have never smoked in their life. |
true |
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A1A deficiency |
Alpha-1 antitrypsin deficiency is an inherited disorder that results in early onset panacinaremphysema as well as cirrhosis of the liver. Patients typically present with signs and symptoms of COPD at an early age and may not even have a history of smoking. This patient presents with signs and symptoms of emphysema due to its progressive nature. Thedistended abdomen along with the fluid wave points to signs of having ascites secondary tocirrhosis. Furthermore, even if this patient had been a smoker or an alcohol abuser, at such a young age it would be very unlikely to have COPD and/or cirrhosis. AAT is an acute phase reactant that is synthesized in the liver and released from hepatocytes. Thegenetic defect in alpha-1 antitrypsin (AAT) deficiency alters the configuration of the AAT molecule, which prevents its release from hepatocytes. AAT normally inhibits elastic fiber breakdown around respiratory units caused by protease and elastase enzymes. An overabundance of elastase and trypsin in the lungs can result in the breakdown of alveolarsurfactant proteins, and thus decreased alveolar compliance. Furthermore, the inability of AAT to be released from hepatocytes results in an abnormal intracellular accumulation of AAT within theendoplasmic reticulum that leads to liver destruction. This hepatic destruction can eventually lead to liver cirrhosis and even hepatocellular carcinoma. AST, ALT, and bilirubin levels elevate above normal when hepatocyte damage occurs and bilirubinis backed up within the hepatic sinusoids. This can eventually lead to jaundice.Major Takeaways: Although AAT deficiency is commonly seen in younger patients, it can still present in middle-aged patients. AAT deficiency is considered a panacinar emphysema, as opposed to centroacinar emphysema secondary to respiratory unit damage from smoking cigarettes. Think of AAT deficiency as a differential diagnosis when a patient presents with signs and symptoms of COPD and have never smoked in their life. |
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A 16-year-old girl presents to the emergency room because of a panic attack. She began feeling tingling around her mouth and became nervous at this symptom, causing her to hyperventilate. The tingling has persisted, and she also reports having had muscle cramps and spasms for the past week. A CMP reveals normal electrolytes except for a low serum calcium. A review of the patient's chart reveals thyroid surgery 1 month ago for a suspicious nodule. Which of the following findings is most likely to be also present in this patient?AHigh parathyroid hormoneBLow calcitoninCLow parathyroid hormoneDMicrocytic anemiaEHigh calcitoninMain ExplanationThis girl is likely experiencing hypoparathyroidism because of destruction of parathyroid tissue during her nodule excision. Hypoparathyroidism causes hypocalcemia, which leads to a number of clinical manifestations including the Trousseau & Chvostek signs, muscle cramps, tetany, andperioral paresthesias. Importantly, hyperventilation such as during panic attacks can exacerbateperioral paresthesias, but those paresthesias in this case would have been initially caused by low serum calcium.Major Takeaway:Unintended removal of some parathyroid tissue is an unfortunate complication of thyroid surgery. The resultant low PTH levels can cause serum hypocalcemia, which manifests as the symptom complex described above. |
Low parathyroid hormone |
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Unintended removal of some parathyroid tissue is an unfortunate complication of thyroid surgery. The resultant low PTH levels can cause serum hypocalcemia, which manifests as the symptom complex described above. |
true |
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A 5-month old female is brought to clinic by her mother because she has been turning blue. On examination, there is a diastolic murmur in the tricuspid region and a widely split S2. The mother also adds that she was taking medication for her bipolar disorder while she was pregnant. What is the most likely etiology for this patient's condition?AAtrial septal defectBPatent ductus arteriosusCVentricular septal defectDEisenmenger syndromeEEbstein's anomaly |
E Ebstein's anomaly |
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Use of lithium during pregnancy is associated with an increased risk for Ebstein's anomaly, which presents as cyanosis in a newborn. |
true |
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lithium in pregnancy is associated with increased risk of |
ebsteins anomaly |
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An eighteen year old girl is brought to the emergency room by her roommate after symptoms of a severe headache and altered mental status. She is found to have a fever of 101.6F. After imaging confirms no anatomical defects, a lumbar puncture is performed, revealing increased neutrophils and decreased protein levels in the cerebrospinal fluid. Which defense mechanism was initially bypassed in this infection?AIgA in the nasopharynxBIgG in the nasopharynxCIgM in the bloodstreamDIgA in the stomachEIgG in the bloodstreamFIgM in the stomach |
AIgA in the nasopharynx |
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Neisseria meningitidis can colonize the nasopharynx. In susceptible individuals, the organism is able to overcome mucosal defenses, particularly by using a virulence factor called IgA protease. |
true |
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A medically-trained parent of a child admitted with croup (laryngotracheobronchitis) approaches you at the hospital and asks you what medication you can give to their child to reduce their "croupscore" and subsequently their hospital stay. You're hoping to get a reference letter from this parent - what do you say to them?ANebulized SalbutamolExplainBOral BudesonideExplainCIV Penicillin GExplainDOral or IM DexamethasoneOral or IM dexamethasone (0.6mg/kg, single dose) has been shown to reduce croupscores and thus make hospital stays shorter.EOral AzithromycinExplain |
D Oral or IM DexamethasoneOral or IM dexamethasone (0.6mg/kg, single dose) has been shown to reduce croupscores and thus make hospital stays shorter. |
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Croup is a viral inflammation affecting the upper airway, larynx, trachea, and bronchi. It is usually caused by parainfluenza viruses. Steroids have been shown to reduce croup scores and length of hospital stay. If treating mild-moderate croup, steroids decrease the need for other interventions such as nebulized adrenaline as well. |
true |
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At a well-child visit for a 4-year old boy, his mother complains of his constant bedwetting. He has never been completely dry, and wets the bed 1-2 nights a week. The mother expresses her frustration because she was able to potty train her older two daughters when they were 2.5-3 years old, and she does not know why it is taking little Nicky so long to catch up. On further questioning, there is no daytime incontinence, no urgency or frequency, no constipation or fecal incontinence, weight loss or excessive thirst. He is not allowed to drink excessively at night. He has no significant past medical history and is otherwise developing appropriately. What should your next step in management be?ARestrict his daily fluid intake to 50% of his current intakeBThe fact that he has never been continent is a negative prognostic factor. Start desmopressin acetate.CReassure and educate the familyDCheck his HbA1c levelEHe should be very lightly punished every time he wets his bed. Negative reinforcement will help to train him. |
CReassure and educate the family |
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Most children become toilet-trained between 2-4 years of age. If the child still has urinary incontinence ≥ 5 years of age, they are clinically defined as having enuresis. Treatment includes reassurance, behavioural conditioning with bedwetting alarms, and medications. |
true |
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enuresis def |
urinary incontinence >= 5yo |
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A concerned father comes to your office because every other night for the past 2 weeks, his four year old daughter has woken up the entire house screaming in her sleep. This happens about 2 hours after she has gone to bed, and he usually finds her sweating, screaming and thrashing around in the bed. She appears terrified, however any attempts to wake her up or console her are in vain. In the morning, it seems that she does not remember a thing. Which of the following statements is most accurate?AThis condition is characterized by rapid entry into REM sleep within minutes of falling asleepBThis condition is associated with sudden loss of motor tone in response to strong emotionCShe will need to undergo a sleep latency testDThis is a nightmare. Parents can be reassured that nothing serious is wrongESleepwalking occurs during the same phase of the sleep cycle |
Sleepwalking and night terrors both occur during the deepest stages of non-REM sleep (stages 3 and 4). |
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Night terrors, like sleepwalking, occur in the deepest stages of non-REM sleep (3 and 4). Thus, avoiding activities that prolong the deep stages of sleep may reduce the frequency of night terrors. |
true Parents are often very worried about night terrors, but they need to be reassured that the child is not in pain and that they can let the episode run its course. It is helpful to avoid stress, irregular sleep schedules and sleep deprivation in these children, because all these prolong the deep stages of sleep where night terrors occur. |
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During a routine well-child visit, the 24-year-old mother of a healthy 5-year-old boy inquires about her persistent acne, which she says is not significantly alleviated by over-the-counter acne treatments. She inquires about Isotretinoin, which her friend has found helpful. While you would not prescribe any medications to her directly, what should she be counseled about?AConjunctivitisBUse of oral contraceptives to prevent pregnancyCDecrease in bone mineral densityDHypercholesterolemiaEPhotosensitivity caused by the drug |
Use of oral contraceptives to prevent pregnancy |
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Isotretinoin is contraindicated during pregnancy because of its |
teratogenic effects |
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isotretinoin |
Isotretinoin is a form of retinoic acid which is contraindicated during pregnancy due to its constellation of teratogenic effects. Isotretinoin and its metabolites cross the placenta and causecraniofacial, CNS, cardiovascular, thymic and parathyroid gland malformations/abnormalities. There is also some evidence that use of isotretinoin during pregnancy is associated with lower IQ scores in children.While all of the other choices listed are side effects of using isotretinoin, the most important consideration is that a woman taking the drug must not become pregnant. |
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On a primary care rotation in Ghana, you meet a child with an ulcerating dermatitis. Credit: Wikimedia CommonsHe also has pitting edema, a distended abdomen, thin hair, and is missing a few teeth. What would you expect to see on biopsy of his liver?AEnlarged liver with fatty infiltratesBNo pathologyCSmall liver with micronodular cirrhosisDA circumscribed massESmall liver with macronodular cirrhosis |
A Enlarged liver with fatty infiltrates |
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kwashiokor (protein deficiency) |
The defining sign of Kwashiorkor (protein deficiency) in a malnourished child is gross pitting edemaof the ankles and feet and abdominal distension. Complications are multi-systemic: impaired liver function, impaired energy production, and impaired immunity become a major problem. |
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A 15-year-old girl presents to your office with concerns from persistent, low-grade cough, fever and fatigue. She reports these symptoms have been occurring for the past 2 months, but that she has otherwise been healthy for the previous year. The only significant item on her past medical history is infectious mononucleosis 2 years ago.Her vital signs are normal, but you note a 10-pound weight loss since last year's exam. Physical exam reveals several enlarged, non-tender and firm cervical lymph nodes. Cardiovascular exam reveals normal S1/S2 heart sounds with no murmurs, rubs or gallops. The lungs are clear to auscultation bilaterally. You also note splenomegaly on abdominal exam. A chest X-ray would show what pathologic finding indicative of this patient's underlying disease?AMediastinal massBLobar pneumoniaCDiffuse pulmonary infiltratesDPerihilar infiltratesECardiomegaly |
A Mediastinal mass |
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Hodgkin's Lymphoma tends to affect age groups in a distinct bimodal pattern - peaking in young adulthood and again in patients over 50 years of age. The diagnosis requires histologic analysis of excised lymph node tissue, but a mediastinal mass on CXR is present in 75% of affected children and can suggest the diagnosis. |
true |
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A 10-year old boy is brought to his pediatrician due to recent onset of dysarthria. His parents have noticed that he was previously very talkative, but now appears taciturn and moody. When he does speak, he has difficulty forming his words correctly. On physical exam, the child has mild tenderness to palpation in his right upper quadrant. On fundoscopic exam, there are yellow-brown deposits in the corneo-scleral junction. The physician diagnoses the child with a disease that occurs due to the accumulation of a substance that is toxic to tissues. How is this substance predominantly excreted in the body?AConjugation to plasma proteins BExcretion into bile CGlomerular filtration DIntestinal excretion ELiver cytochrome activity FLysosomal storage |
B Excretion into bile |
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Wilson disease is a disorder resulting from |
impaired copper excretion into bile. Copper overload and deposition in tissues leads to predominantly hepatic and neuropsychiatric symptoms. |
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A 5-day-old infant is being evaluated in the neonatal intensive care unit for increased lethargy.Birth history is significant for Cesarean section at 36 weeks of gestation due to non-reassuring fetal heart tones, neonatal hypoglycemia, and ambiguous genitalia. Physical examination reveals a hypotensive, lethargic infant with decreased muscular tone. Laboratory findings reveal a serum sodium concentration of 128 mEq/L and a serum potassium concentration of 6.1 mEq/L. What is the most likely diagnosis?AConn syndrome B17a-hydroxylasedeficiency C21-hydroxylase deficiency DCushing disease EPrimary adrenal insufficiency |
21-hydroxylase deficiency |
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A 21-hydroxylase deficiency is the most common form of |
congenital adrenal hyperplasia |
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females with congenital adrenal hyperplasia due to 21 hydroxylase def will have |
ambiguous external genitalia Other features include hyperkalemia, hyponatremia, hypoglycemia, and shock within several days of birth. |
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males with congenital adrenal hyperplasia due to 21 hydroxylase def will have |
normal external genitalia Other features include hyperkalemia, hyponatremia, hypoglycemia, and shock within several days of birth. |
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A 4-year-old girl with a corrected aortic stenosis is seen by her oncologist for a follow up visit. She was recently diagnosed with acute lymphoblastic leukemia and initiated chemotherapy a week before through a peripherally inserted central line. At the visit her mother reports the she is better but "seems more tired all the time" and has been bruising easily. Physical examination shows several tender non-blanching petechiae on the pads of the fingers and toes, several dark non-tender petechiae on her palms and soles, and small linear hemorrhages beneath the fingernails. Fundoscopic examination shows several tiny areas of hemorrhage on the retinae bilaterally. Cardiac examination is notable for a II/VI systolic ejection murmur that seems to have worsened in comparison to the last visit. Which of the following is the most likely diagnosis in this patient?ADilated cardiomyopathyBBleeding diathesis secondary to thrombocytopeniaCInfective endocarditisDAcute rheumatic feverEHypertrophic cardiomyopathy |
Infective endocarditis |
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Infective endocarditis occasionally manifests clinically with |
Osler's nodes, Janeway lesions, and Roth spots. It is most commonly caused by S. aureus or S. viridans. |
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IE features |
Infective endocarditis (IE) is an infection of the endocardial surfaces of the heart, most commonly the heart valves. IE occurs when a microorganism is introduced to heart surfaces (such as during a surgical procedure), adheres to the surfaces, and begins to invade the valves and surrounding areas. Most cases of IE are caused by streptococcal and staphyloccocal microorganisms. IE is relatively uncommon in children, and precipitating risk factors includecongenital heart disease. The patient in question has a history of aortic stenosis which was corrected with surgery, and a recent hospitalization with placement of an indwelling central catheter. Her exam is notable for the classic findings of infective endocarditis, including Osler nodes (painful petechiae on the fingertips), Janeway lesions (non-tender petechiae on the hands and soles), and Roth spots (pinpoint retinal hemorrhages). The nidus of infection is likely her repaired aortic valve, and the route of infection was likely through the peripherally inserted central catheter(PICC). Once the microorganism is identified, the preferred treatment of IE is the administration of antibioticsintravenously. |
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central lines can cause |
IE due staph aureus or strep viridans |
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A 9-month old term male presents for increased work of breathing. Per his mother, the infant has had rhinorrhea,watery eyes and a dry cough for the last three days. This morning, the mother noticed the infant working harder tobreathe and brought him to clinic for evaluation. Past medical history is notable for an episode of bronchiolitis at 6 months of age requiring hospitalization for one day. Sick contacts include two children in daycare with viral illnesses. Family history is significant for asthma in an older sister. Home medications include vitamin D supplementation. The patient has no known drug allergies. On examination, the infant has increased work of breathing with tracheal tugging, nasal flaring and intercostal andsubcostal retractions. Vitals are: T 37˚C, HR 160, RR 40, BP 90/55, and O2 saturation 85% on room air. On examination, the infant has diffuse wheezing in both lung fields. Which of the following is evidence-based management for this patient?APrednisoloneBHypertonic saline nebulizationCContinuous albuterol nebulizationDChest radiographEContinuous pulse oximetry |
Continuous pulse oximetry |
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For bronchiolitis, current guidelines state that supplemental oxygen should be given, along with continuous pulse oximetry to monitor response to therapy. |
true |
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A 15-month-old boy with no past medical history is brought in to his pediatrician's office for a regular check-up. His mother reports that he had an upper respiratory infection about 2 months ago, but that he has been otherwise healthy since his last visit. Routine screening CBC reveals a hemoglobin level of 6.8g/dL, 5000 WBCs/µL, and 400,000 platelets/µL. A follow-up CBC with smear returns the same results, as well as a finding of a reticulocyte count at 0.5%. MCV is 95fL. Which of the following is the most likely diagnosis in this patient?ATransient erythroblastopenia of childhoodBSickle cell traitCDiamond-Blackfan anemiaDPhysiologic anemiaEFanconi anemia |
Transient erythroblastopenia of childhood |
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whats transient erythroblastopenia of childhood |
Transient erythroblastopenia of childhood (TEC) is a temporary aplastic anemia, often associated with a recent viral infection. It is a self-resolving condition that does not require treatment. |
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A 2-year-old exclusively breast fed African American child is brought to your emergency department with a pain in his leg after a mild fall. He also has been having an increase of upper respiratory infections. On physical examination, the patient presents with a prominent forehead, significant dental caries, bumpy ribs, flattened occiput, normal sinus rhythm and bowed extremities. You immediately send for radiographs and observe a greenstick fracture of the left leg and some fraying at the ends of the distal femur with decreased mineralization around the epiphyses. What is your next best step for treatment besides a cast for his leg?AVitamin D 2000 international units/day for six weeksBFollow-up in 1 year to rule out Blount’s diseaseCVitamin K1 0.5mg doseDRest and referral to orthopedic surgeonEFerrous sulfate 3mg/kg for four weeks |
Vitamin D 2000 international units/day for six weeks |
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The vitamin D content of breast milk is low even in a vitamin D sufficient mother, and exclusively breast-fed infants will only ingest only 10 to 40 IU/day of vitamin D in the absence of sun exposure or supplement. Furthermore, thevitamin D content of breast milk is lower in mothers with dark skin, which makes this patient at extreme risk forVitamin D deficiency. The decreased mineralization around the epiphyses as well as bow-legs with prominent forehead and bumpy ribs are clear indications for patients with vitamin D deficiency. Typically the bumpy ribs, or rachitic rosary, is due to a deficiency of calcium from the low vitamin D, which results in lack of mineralization and overgrowth of costochondraljoint cartilage. Also, patients with Vitamin D deficiency are known to have increase risk for upper respiratory functions. Major Takeaways: The vitamin D content of breast milk is low even in a vitamin D sufficient mother, and exclusivelybreast-fed infants thus vitamin D of 2000 international units/day for six weeks is needed. |
true |
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An infant is born with truncated fingers with deep circumferential indentations. A photograph is shown below. What is the pathogenesis of this condition?Credit: Wikimedia CommonsAVascular disease in the motherBAbnormal strands of amniotic tissueCAbdominal trauma during pregnancyDSpontaneous mutations in homeobox domainsEIngestion of alcohol by the mother during pregnancy |
B Abnormal strands of amniotic tissue |
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Amniotic Band Syndrome can present in a variety of ways - it depends upon the body part being constricted by the bands. Look for a pattern to the deformation and query for complications during pregnancy to help suggest the diagnosis. |
true |
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A newborn presents with severely misshapen vertebrae and a significant narrowing of the anus. He is also found to have a tracheo-esophageal fistula. Which of the following problems is NOT likely to also be found in this patient?ACardiac defectsBGenitourinary malformationsCRenal agenesisDMissing thumbs/underdeveloped forearms |
Genitourinary malformations |
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VACTERL association |
(vertebral defects, anal atresia, cardiac defects, tracheo-esophageal fistula, renal anomalies and limb development issues) The genitourinary system is typically not affected. |
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'VACTERL associations' are congenital anatomical defects that typically occur in groups. If you see 1 or more in aneonate, ensure you look for the presence of any others. |
true |
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As part of a well-child visit, the mother of a 10-year-old girl with no past medical history asks about the changes associated with puberty in her daughter. The daughter currently has no pubic hair and only her papillae are raised above her chest wall. What can you tell the mother about the first changes she will see next in her daughter?ASmall mounds forming at the breasts and areolae around the nipples and/or the development of straight, scant pubic hair along the labiaBSome spotting of blood within the underwearCIntermittent light vaginal bleeding & diffuse abdominal painDCoarse, curly pubic hair development in advance of breast budding and areolae enlargementEAreolae forming without breast buds always occurring simultaneously with the development of straight, scant pubic hair |
Small mounds forming at the breasts and areolae around the nipples and/or the development of straight, scant pubic hair along the labia |
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Tanner staging is a scale of 1-5 used to grade secondary sexual development. In females, Tanner staging is based on breast and pubic hair development, and Tanner stage II is the first stage of puberty, which is associated with breastbuds, increased areolar diameter, and scant pubic hair. |
The first signs of puberty in females are breast budding and possibly the development of scant, straight pubic hairalong the labia. Either or both of these signs may arise, but they do not have to occur together. These findings are consistent with Tanner stage II for the breasts and genitalia. Menarche typically occurs within 2 years of the onset ofTanner stage II in girls.In boys, the first sign of puberty (i.e. Tanner stage II) is testicular enlargement with or without the development of scant, straight pubic hair primarily at the base of the penis. |
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A 14-year-old girl is admitted to the hospital for inpatient treatment of anorexia nervosa. By her report, she has not eaten anything for the past 4 days, having drank only water. Her admission physical exam is notable for hyperreflexia, as well as positive Chvostek and Trousseau signs. Deficiencies in which of the following serum ions could be responsible for these neurologic findings?AChlorideBBicarbonateCSodium and potassiumDHydrogenEMagnesium and calcium |
Magnesium and calcium |
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Hypomagnesemia and hypocalcemia often co-occur, and have similar clinical signs. These include neurological (e.g. Trousseau's, Chvostek's) and cardiac (e.g. prolonged QT interval) symptoms. |
true |
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what are the signs for hypomagnesemia and hypocalcemia |
neurological trousseaus sign chveosteks sign cardiac prolonged QT interval |
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You are evaluating a 5-year-old boy for the first time. He has received spotty medical care as his mother had been uninsured until this point. He is up to date on his routine vaccinations, but has received little other health care. His mother notes that when he was born, she was told that he had a small "hole inside of his heart." She is not aware of any other health issues, although she does note that he seems to get tired more easily as of late. On exam, the boy is afebrile. You note a I/VI holosystolic murmur at the lower left sternal border. The point of maximum impulse is displaced leftward. There is no clubbing or edema visible on exam. The lungs are clear to auscultation. Which of the following is most likely to be observed on chest X-ray?ALeft-shifted tracheaBBibasilar pulmonary infiltratesCBlunted costovertebral anglesDDilated aortic rootECardiomegaly |
Cardiomegaly |
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vsd has what sort of murmur |
pansystolic aka holosytolic In a child, worsening fatigue, a holosystolic murmur, and a displaced point of maximal impulse should make you think of a ventricular septal defect. Both left and right ventricles are likely to be enlarged in a patient like this, who has had little medical care. |
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A distraught mother brings in her 8-year-old child when she learns about lead contamination in his elementary school'sdrinking water. Which is the most appropriate screening test?ANeurologicexam ExplainBOphthalmologic exam ExplainCBlood lead level DPeripheral blood smear for basophilic stippling Peripheral blood smear for basophilic stippling would be present in lead toxicity but it is notascreening test.EErythrocyte protoporphyrin levels |
Blood lead level |
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Blood lead levels are the first and most accurate method used to screen children for lead poisoning. |
true |
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You are working the night shift in the ER and your next patient is an 18-month old girl who has been brought in by her parents because of an awful sounding cough. She has had a runny nose and a low grade fever for the past 2 days, with some hoarseness and a rough-sounding cough that started this afternoon. This evening she began making some high pitched sounds when taking breaths, and she seemed to be having a bit of trouble breathing so they brought her in. She is alert and does not appear to be in acute distress. She has a temperature of 101˚F with RR of 50/min and O2 saturation of 97%. There is an audible inspiratory stridor which worsens when she starts to cry during the examination. You hear an occasional barking cough. Her pharynx is mildly erythematous with normal tonsils and no exudate. There are minimal suprasternal and intercostal retractions, and there is adequate air entry on chest auscultation. Which of the following is the best step in management?AAP and lateral radiographs of the neckBTrial of bronchodilator therapy and oral steroidsCRacemic epinephrine and IM corticosteroid therapyDStart IV antibioticsEIntubation |
Racemic epinephrine and IM corticosteroid therapy |
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croup fxs |
Croup (laryngotracheobronchitis) is a viral infection of the upper airways most often seen in the pediatric population between 6 and 36 months, and classically presents with a "barking" cough. Most cases can be managed on an outpatient basis and will resolve within 3-7 days. |
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A 15-year-old male was playing in his high school basketball game when he suddenly became short of breath and immediately fell unconscious. A family history of similar episodes occurring in a number of other family members was discovered, and a few relatives had passed away at a young age. The patient had been previously healthy to his knowledge. What was the cause of this patient’s event? AConcentric hypertrophy with systolic dysfunction BConcentric hypertrophy with diastolic dysfunction CIschemia-induced cardiac dilation DEccentric hypertrophy with diastolic dysfunction EEccentric hypertrophy with systolic dysfunction |
Concentric hypertrophy with diastolic dysfunction |
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Sudden cardiac arrest/death in a young athlete is often due to hypertrophic cardiomyopathy, which is often inherited in an autosomal dominant fashion. Remember that a thickened left ventricle, from either a congenital condition (HOCM) or acquired (hypertension), is stiff and has poor diastolic filling, leading to diastolic heart failure. |
true |
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A couple who recently migrated from Nigeria brings their 4-year old daughter to the hospital with severe pain in her back and legs. She has been very fatigued with low energy for the past two days. She has had similar episodes in the past, and this is her fifth episode this year. On physical exam, you find an irritable, crying child with subconjunctival pallor and mild scleral icterus. She is tachycardic. Her chest is clear to auscultation. Her abdomen is soft and non-tender, with the tip of the spleen barely palpable. She is reluctant to move her lower extremities and screams when you attempt passive movements. Her lab results show a Hb of 6.5mg/dL. She is currently on no medications. Her parents are frustrated with seeing her suffer and want better management of her condition. Which of the following is not currently recommended as part of her long term management?APneumococcal vaccineBDaily folic acid supplementationCProphylactic splenectomyDHydroxyureaEDaily Penicillin |
Prophylactic splenectomy |
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in sickle cell disease that results in vaso-occlusive crises whats the mainstay of treatment |
hydration and analgesia |
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Patients with sickle cell disease have abnormally shaped red blood cells which do not flow as smoothly through blood vessels. If vaso-occlusive crises do occur, hydration and analgesia are the mainstays of treatment. |
true |
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A full-term eight pound baby boy is noted to have large scrotum at delivery. The physician is able to palpate both testicles within the scrotum. The scrotum further enlarges when the physician applies gentle pressure to the newborn's abdomen. The physician is able to transilluminate the enlarged scrotum. Which of the following developmental defects is present in this child? AFailed fusion of the processus vaginalis BWeakened transversalis fascia CDilitation of the pampiniform plexus DHerniation of bowel through the inguinal canal ECryptorchidism |
Failed fusion of the processus vaginalis |
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Hydroceles increase in size when pressure is applied to the abdominal wall and transluminate with a strong light. They occur due to failed fusion of the processes vaginalis. |
Craniopharyngioma |
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Histologic evaluation of the tumor reveals a monotonous collection of small, round, darkly stained cells. He is eventually diagnosed with a primitive neuroectodermal tumor (PNET) of the CNS. What is the embryologic origin of the cells in this tumor?AHematopoietic stem cellBMesodermCNeural tubeDEndodermENeural crest |
Neural crest |
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Primitive Neuroectodermal Tumors (PNETs) are a group of highly malignant tumors derived from |
neural crest cells. |
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A newborn is examined and found to have polydactyly, meningomyelocele, and rocker-bottom feet. The mother did not obtain prenatal care. Based on these findings, the most likely diagnosis in this patient is which of the following?ATurner syndrome BTrisomy 18 CTrisomy 21 DTrisomy 13 EFragile X syndrome |
Trisomy 13 Trisomy 13, or Patau syndrome, is characterized by mental retardation, polydactyly,holoprosencephaly, meningomyelocele, rocker-bottom feet, cleft palate, eye malformations, and GI/cardiac/renal disease. |
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Which of the following is the first vaccine series that will be completed in a child's life, assuming that the child is following the standard immunization schedule?AMeasles, mumps, and rubella (MMR)BHaemophilus influenza B (Hib)CHepatitis BDDiphtheria, pertussis, and acellular pertussis (DTaP)ERotavirus |
CHepatitis B |
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Hepatitis B vaccine is the first vaccine given to children, often shortly after birth. It is recommended to be completed by the time the child is 2 months old. |
true |
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A child presents to the office for a health maintenance examination. The mother has no concerns and the child has had no medical problems to date. The mother states the child sleeps through the night and is feeding well. Physical examination reveals the child is able to sit unsupported and transfer a toy from one hand to the other. He is babbling throughout the examination. The most likely age of the child is?A2 month oldB9 month oldC12 month oldD15 month oldE6 month old |
6 month old |
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developmental milestones |
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A 16-year-old girl presents with pain in her right lower quadrant. She has not had any fevers, but has felt fatigued. Additionally, she complains of new irregularity of her menstrual cycles. She normally has a period every 28 days, but most recently she started bleeding only yesterday, 6 weeks after her last cycle. Her past medical history is otherwise negative. Physical exam reveals mild tachycardia and a blood pressure of 110/70. What is your next step?AAbdomen and pelvis CTBUrinalysis with urine cultureCPelvic ultrasoundDUrine pregnancy testEPelvic bimanual exam |
Urine pregnancy test |
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Remember the classic triad for ectopic pregnancy: |
abdominal pain, vaginal bleeding, and missed menstrual cycle. Also remember the classic pearl: "All female patients are pregnant until proven otherwise". |
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A 15-year-old boy presents to the office with the complaints of painful urination and urethral discharge. History reveals two occasions of unprotected intercourse in the past two weeks. Physical examination reveals a purulent urethral discharge. A specimen of the discharge is obtained for culture and the results are pending. Along with appropriate pharmacological management, which of the following should also be done?ANotify the boy’s parentsBNotify the sexual partnersCCounsel him on the importance of safe sexual practicesDTerminate him from your practice followingtreatmentENotify the state health department |
Counsel him on the importance of safe sexual practices |
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There are exceptions to the rules of treating minors. Minors do not need consent for emergentintervention, treatment of alcohol and drug-related issues, treatment during pregnancy, and treatment of sexually transmitted diseases. |
true |
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A 1-day-old female is seen in the nursery for a physical examination. History reveals a normal spontaneous vaginal delivery. The mother received full prenatal care and plans to breastfeed exclusively. Vital signs are stable. Physical examination reveals a bulging, blue dome-shaped, translucent membrane at the level of the vaginal introitus. Which is the most appropriate test to confirm the diagnosis in this patient?APelvic examination ExplainBPelvic ultrasound CHormone analysis Hormone analysis would provide little benefit in the work-up of this patient. DDirect needle aspiration ExplainEComputed tomography |
pelvic ultrasound |
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Direct needle aspiration is useful in the presence of a periurethral cyst but would not be indicated in this patient. |
true |
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Pelvic ultrasound is the study of choice in patients suffering from |
imperforate hymen |
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A 6 day old newborn born at 31 weeks gestation with a low birth weight is in the NICU and develops new acute onset bloody diarrhea. He also vomited twice after his last feeding. On exam you find a distended, tender abdomen with decreased bowel sounds. What do you expect to see on abdominal xray?ANo abnormalitiesBMalrotationCAir-fluid levels proximal to a point of obstructionDPneumocystis intestinalisEPeritoneal abscess |
Pneumocystis intestinalis |
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NEC classically occurs in low birthweight, sick, or premature newborns during the first few weeks of life. Symptoms include acute onset vomiting, bloody diarrhea, and temperature instability. Radiological findings includepneumocystis intestinalis, and if more severe, air in the portal vein and/or free air in the abdomen. |
true |
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A 4-year-old girl develops abdominal cramps, diarrhea and tenesmus after coming home from daycare one day. Her stools are bloody, contain mucous and are small in volume. She has a fever of 103.3ºF. Stool sample showspolymorphonuclear leukocytes on a methylene blue stain. CBC measured at an urgent care clinic reveals WBC of 14. What treatment is recommended?ALoperamide, with fluid and electrolyte repletionExplainBAzithromycinCCiprofloxacinExplainDFluid and electrolyte replacement onlyExplain |
Azithromycin |
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A mother brings in her 21-day old infant complaining that the child has had projectile vomiting after feeding for several days. He has a good appetite, but is losing weight. The mother thinks there may have been a similar problem with the baby's paternal uncle, who needed surgery when he was an infant. Which of the following findings is a characteristic sign of this condition?ABilious vomitBilious vomit would be present only if foodstuff was allowed to reach the small intestine, which is not the case in this infant. (See bilious vomit, think duodenal or lower, i.e. post-pylorus)BHypochloremic alkalosisCGeneralized abdominal distensionExplainDCurrant jelly stoolExplain |
BHypochloremic alkalosis |
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pyloric stenosis classically results in what metabolic lab changes |
A classic lab finding ishypochloremic, hypokalemic metabolic alkalosis due to loss of chloride, hydrogen, and potassium ions via emesis. |
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A 10 year-old girl presents to the emergency department complaining of headaches, nausea, fatigue, and abdominal pain. Her mother states that her thirst is unsatiable and has been urinating much more frequently than usual. On examination, the patient appears tired. She has dry mucous membranes and delayed capillary refill. Her abdomen is soft with no masses. Bedside glucose is 410 mg/dL. Laboratory analysis reveals sodium of 132, chloride of 91,bicarbonate of 12 meq/L, BUN of 37 and creatinine of 1.8 mg/dL. What is the most likely cause of her metabolic acidosis?AKetoacidosisExplainBDiarrheaExplainCRenal tubular acidosisRenal tubular acidosis causes non-gap metabolic acidosis. This patient has an anion gap of 29.DUremiaUremia is a cause of anion-gap metabolic acidosis. However, this vignette is a classic story fordiabetic ketoacidosis. |
ketoacidosis |
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Anion gap can be calculated as [Sodium] - [Chloride] - [Bicarbonate] which is 29 in this vignette. Causes of anion-gap metabolic acidosis can be remembered using the mnemonic MUDPILES: |
Methanol Uremia Diabetic ketoacidosis Propylene glycol Iron poisoning/isoniazid Lactic acidosis Ethylene glycol Sepsis/Salicylates |
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The mother of a 12-year-old boy brings her son into your office because he has been "going to the bathroom all thetime" for the past month. She says that "he's thirsty all the time, but it seems like it just goes in and out." She has also noticed areas of darker skin around the skin folds of his arms and neck. Otherwise he has had no other symptoms: nonausea, vomiting, or diarrhea; no pain with urination; no changes in the color of his urine. The patient's exam is notable for weight greater than the 95th percentile, and you note that his growth chart indicates that he has been crossing percentile lines for several years now. Hyperpigmented areas are visible on the antecubital fossae and on the lateral aspects of his neck. Which of the following lab results would be diagnostic of the process affecting this child?ALow serum C-peptide levelsB2+ glucose on urine dipstickCFasting glucose >125mg/dLD2+ ketones on urine dipstickEFasting glucose >100mg/dL |
Fasting glucose >125mg/dL |
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T2DM can be diagnosed in 4 different ways: |
1. Fasting glucose greater than or equal to 126mg/dL (the correct answer in this question) 2. Random glucose greater than or equal to 200mg/dL 3. Plasma glucose greater than or equal to 200mg/dL two hours after an oral glucose challenge (1.75g/kg) 4. Hemoglobin A1c greater than or equal to 6.5% |
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T2DM is becoming an increasingly common diagnosis in children. Two common ways you will diagnose this is by looking for fasting glucose greater than or equal to 126mg/dL, or a HbA1c ≥ 6.5%. |
true |
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A 25-year-old G1P1 female brings her 2-day-old infant to the emergency department with the complaints that he has been lethargic, irritable, and not feeding well since she brought him home. The infant was born via a normal vaginal delivery at 33 weeks gestation. Physical examination reveals an elevated temperature and a bulging anterior fontanel. Which of the following would most likely be found in a cerebrospinal fluid sample from this patient? AOpening pressure ↑, lymphocytes ↑, protein ↑, glucose ↓ ExplainBOpening pressure ↑, PMNs ↑, protein ↓, glucose ↑ In bacterial meningitis, protein should be elevated and glucose should be very low because the bacteria consume it. COpening pressure normal, lymphocytes ↑, protein normal, glucose normal ExplainDOpening pressure ↑, PMNs ↑, protein ↑, glucose ↑ ExplainEOpening pressure ↑, PMNs ↑, protein ↑, glucose ↓ |
EOpening pressure ↑, PMNs ↑, protein ↑, glucose ↓ |
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Bacterial meningitis in a newborn is likely due to Streptococcus agalactiae and a CSF exam shows increased PMNs and protein and decreased glucose. |
true |
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An 10-year-old boy presents to the emergency department complaining of severe scrotal pain. History reveals that four days prior his left testicle became swollen and mildly painful. He states the pain has progressively worsened and is why his father brought him to the hospital today. He denies sexual activity, fever, nausea and vomiting, or urinary symptoms. He does not recall any traumatic events prior to the onset of his symptoms. Physical examination reveals a small firm, blue mass at the top of his left testicle. Which is the most likely diagnosis in this patient?AHydrocele ExplainBEpididymitis Epididymitis is typically caused by an infection and can be either acute or chronic in nature. Patients present with testicular pain and swelling, as well as fever, erythema and pyuria. Pain may be relieved by scrotal elevation. Diagnosis is aided by performing a UA, Doppler ultrasound to assess for increased vascularity or by surgical exploration.CTesticular cancer ExplainDTesticular torsion ExplainETorsion of the testicular appendage |
ETorsion of the testicular appendage |
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Torsion of the testicular appendage presents as an acute onset of unilateral scrotal pain and swelling in the absence of fever, nausea, vomiting or urinary symptoms. A blue mass may be present at the top of the involved testis. |
true |
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A 6-year-old girl currently undergoing treatment for non-Hodgkin lymphoma presents to the emergency departmentwith worsening nausea, vomiting, diarrhea and muscle cramps. The mother reports that her daughter has not urinatedin the past 24 hours. Stat labs obtained by the triage nurse reveal the following:Sodium: 140Potassium: 5.1Calcium: 4.5Bicarbonate: 26BUN: 29Creatinine: 2.2What is the most likely cause of this patient's presentation?AAdverse effects of chemotherapy drugsBMetastatic disease to the kidneysCDehydrationDTumor lysis syndromeENeutropenia |
Tumor lysis syndrome |
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TLS is a potentially fatal complication of chemotherapy. Patients are at greatest risk for acute kidney injury, which usually presents as anuria. TLS can present as a variety of symptoms including nausea, vomiting, diarrhea, lethargy,heart failure, seizures, muscle cramps and sudden death. |
true |
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complication of chemotherapy |
tumor lysis syndrome Patients are at greatest risk for acute kidney injury, which usually presents as anuria. TLS can present as a variety of symptoms including nausea, vomiting, diarrhea, lethargy,heart failure, seizures, muscle cramps and sudden death. |
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A 18-month-old boy was brought to your office by his father who complained that the patient has fever and ear pain. History reveals the child began yanking on his ear three days ago and complaining of pain. The mother reports a temperature of 100.0°F this morning and a decreased appetite. Vital signs reveal a temperature of 101.4ºF. Physical examination reveals erythema and decreased mobility of the right tympanic membrane. Four days later the childreturns to the office with no improvement of symptoms. Physical examination reveals a bulging tympanic membranebilaterally with erythema and a meniscus of fluid behind the membrane. Which of the following is the most appropriate pharmacological management at this time?AAmoxicillin ExplainBAzithromycinAzithromycin may be given in the case of a penicillin allergy, but is not as first-line therapy foramoxicillin-resistant organisms. CCeftriaxone (Rocephin) injectionExplainDVancomycinExplainEAmoxicillin/clavulanate (Augmentin) |
Amoxicillin/clavulanate (Augmentin) |
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First-line therapy for amoxicillin-resistant otitis media is amoxicillin/clavulanic acid. |
true |
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An infant is born small for gestational age and being evaluated by a physician. On physical exam, the physician notes a smooth philtrum of the lip, microcephaly, and midfacial hypoplasia. The most likely teratogen that this infant was exposed to prenatally is which of the following?AThalidomide ExplainBAlcohol ExplainCLithium ExplainDDiazepam |
Alcohol |
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Characteristics of fetal alcohol syndrome (FAS) are |
microcephaly, smooth philtrum, cardiac septal defect, small palpebral fissure, midfacial hypoplasia, and microphthalmia. |
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A 27-year old woman comes to the office complaining of breast pain. She has been breastfeeding her 6-week old son since birth, but her current pain is making this quite uncomfortable. On examination there is a localized area oferythema, tenderness and warmth to her left breast, with some degree of induration. The nipple is cracked slightly. Tender axillary lymphadenopathy is appreciated. The woman is febrile with a temperature of 101.4˚F, and complains of generalized malaise. What is the most appropriate management of her condition?AShe most likely has mastitis. Start her on anti-Staphylococcal antibiotics and continuebreastfeedingBThis is normal irritation associated with breastfeeding. Recommend a cold compress, andbreastfeeding from the right breast only.ExplainCShe most likely has mastitis. Stop breastfeeding and start her on antibiotics covering S. aureus.Breastfeeding is part of the recommended therapy for mastitis. Emptying of the affected breast will improve the pain and help empty the milk from the affected area. DShe most likely has a breast abscess. Perform an incision and drainage and stop breastfeedingExplainEShe most likely has an inflammatory breast cancer. Send her for ultrasound and breast biopsy |
AShe most likely has mastitis. Start her on anti-Staphylococcal antibiotics and continuebreastfeeding |
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Mastitis is an inflammation of the breast most often due to S. aureus infection during the first 2 months ofbreastfeeding. Antibiotic treatment should be given, but breastfeeding in the affected breast should also continue. |
true |
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A 10-year-old boy presents to the emergency department following a fall while climbing a tree. He is unable to hold his right hand up at the wrist and that it remains flexed at that joint. Also, he is unable to extend at the wrist or digits. Sensation is diminished along the lateral aspect of the hand on the dorsal surface as well as on the posterior forearmbut is intact along the triceps. The boy has a normal triceps reflex but diminished brachioradialis reflex. X-ray of the right upper extremity would most likely reveal which of the following injuries? AFracture of the distal radius ExplainBFracture in the midshaft of the humerus ExplainCFracture of the distal ulna Patients with nephrotic syndrome can develop swelling of the soft tissues in the hands and feet as a result of hypoalbuminemia due to loss of protein in the urine. The hands and feet are typically not painful or tender to palpation as seen in this child. His presentation is more consistent withdactylitis due to sickle cell disease.DAcromioclavicular joint separation EFracture in the surgical neck of the humerus Explain |
Acromioclavicular joint separation |
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Dactylitis presents with symmetrical painful swelling of the hands, feet, or both, in a young childwith sickle cell disease. |
true Dactylitis is a specific type of vaso-occlusive crisis caused by infarction of the small bones of the hands and feet and manifests as extremely painful, usually symmetrically, swelling of the hands or feet (or both). The extremities can also be mildly erythematousand are usually very tender to touch. Patients are also often mildly febrile. Radiographically, only soft tissue swelling can be appreciated acutely, making dactylitis difficult to distinguish from other diseases on the differential, includingosteomyelitis, which does not demonstrate immediate radiographical changes on plain film. Dactylitis is the most common initial presentation of sickle cell disease, accounting for 50% of cases of children who present under the age of 2 years. It is uncommon in older children. |
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A 16-year-old male is brought to the office for examination. He comes with his mother, who tells you he has anintellectual disability that was diagnosed when he was very little. The patient has a long face with a large jaw and large, everted ears. The patient is also noted to have macroorchidism. Which of the following is the most likely etiology of this patient's condition?ATrinucleotide repeat of CTGExplainBDeletion in gene encoding dystrophinExplainCTrinucleotide repeat of CGGExplainDTrinucleotide repeat of GAAThis is characteristic of Friedreich ataxia, which typically presents with frequent falling, nystagmus,pes cavus, kyphoscoliosis, and hypertrophic cardiomyopathy.ETrinucleotide repeat of CAGExplain |
Trinucleotide repeat of CGG This is characteristic of Fragile X syndrome. |
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Fragile X syndrome is a trinucleotide repeat disease characterized by repeat of CGG on the X chromosome. It is most often seen in men and young boys, and is associated with developmental delay and macroorchidism. |
true |
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A 4-year-old child presents to your office for a well child visit. If you were to observe this child in a social situation, which of the following is the highest social and gross motor interaction you are most likely to observe if the patient has met the developmental milestones? |
Has an imaginary friend and participates in imaginative play |
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A four year old child will typically present with the ability to skip, alternate feet down stairs, catch ball, and participate in imaginative play. See the comprehensive list of developmental milestones above. |
true |
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A 6 month-old boy has been spitting up his meals since he was one month old and has also had frequent, colickybehavior after feeding. He is otherwise asymptomatic with no significant findings on physical exam. His growth has been stabilized at 90th percentile. What is the most likely diagnosis?A Gastroesophageal reflux (GER) |
Gastroesophageal reflux (GER) Gastroesophageal reflux is a common cause of vomiting in very young children that almost always resolves on its own by 1 year of age. |
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A 12 year old boy presents a one day history of nausea, vomiting, fever, non-bloody diarrhea and cramping abdominal pain. His fever is 101.6 F and he is having 5-6 loose stools per day. He was in his normal, healthy state before this stated. His mother mentions that he was helping cook chicken cutlets two days ago. Stool culture grows gram-negative, lactose-negative organisms that form translucent colonies on MacConkey agar which are positive forSalmonella O antigen. What is the best course of treatment? AAmoxicillinBTrimethoprim-sulfamethoxazole (TMP-SMX)CReplace fluids and electrolytesDCiprofloxacinEPiperacillin-tazobactam |
Replace fluids and electrolytes |
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In general, otherwise healthy individuals with a suspected salmonella infection need supportive therapy and no antibiotics. |
true |
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A 2 year old child whose family recently immigrated from Korea is brought to the Emergency Department with a fever of 102.8F that has lasted 6 days. He has swollen cervical lymph nodes, injected conjunctiva, erythematous lips and tongue and red swollen hands. His older brother was recently sick with a viral upper respiratory infection. Lab tests show a normal urinalysis, WBC 14, Hct 25, MCV 88, and ESR of 35 mm/hr. How should this child be treated?AAcetaminophen and oral fluidsExplainBDoxycycline for 5-10 daysExplainCAmoxicillin and clavulanic acidAugmentin combines Amoxicillin, a penicillin antibiotic, with Clavulanic acid, a β-lactamase inhibitor. It is widely used to treat many bacterial infections including UTIs, respiratory tract infections, soft tissue infections, and dental infections. Antibiotics should be avoided in children without bacterial infections.DAspirin and intravenous immunoglobulins |
Aspirin and intravenous immunoglobulins |
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Important keys to a KD diagnosis include a fever lasting >5 days, cracked lips, and injected conjunctiva. Admit, and treat with IVIG and aspirin immediately. |
true |
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A mother calls the office with the complaint that her 5-year-old son has sustained a chemical burn. History reveals he was playing under the sink and accidentally spilled an alkaline drain cleaner all over his arms and legs. The nearest hospital is a few miles away. Which of the following are the most appropriate instructions to give the mother? ATake the patient into the shower right away and keep the water running over him for 30 minutes before bringing him to the emergency department BCover the burned areas with triple antibiotic ointment until he can be seen in the office the following day. C Bring the patient into the emergency department as soon as possible D Wash the burned areas with a mixture of water and vinegar and bring the patient to theEmergency department ExplainE Wrap the burned areas in sterile dressings before bringing him to the emergency department. |
Wait for 4-5 days and reevaluate if she has not passed the bead |
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Most objects that make it through the throat to the stomach are able to pass completely through the GI system. |
true |
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A 2-year-old female presents to the office with her mother for a health maintenance examination.The mother states thechild “gets into everything in our house.” She is afraid that her daughter is going to put herself in a dangerous situation. History reveals the house has childproof cabinets, a baby gate for the stairs, and an appropriate car seat. Their home has a pool and a fenced in back yard. The mother would like to know the greatest risk for her child. She is informed the most common cause of death in this age group is?AChild abuseExplainBSuffocation ExplainCMotor vehicle accidents Motor vehicle accidents are the second leading cause of death in this age group. DElectrical burnsExplainEDrowning |
Drowning |
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Unintentional injury is the major cause of death in children aged 1-4 with drowning being the leading cause of unintentional injury. |
true |
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A 7-year-old boy is brought to the pediatrician's office for a yearly check-up. His father is concerned about his son's height, stating that his growth appears to be slowing down. Looking at the boy's growth curves, you see that his height curve followed the 5th percentile until about age 4, when he began to “fall off” of the curve. His height is now below the 3rd percentile, but his weight is at the 85th percentile. His father shows you a bone age radiograph taken last week. The radiologist's report estimates that the child's bone age is 5. The father reports that he and the boy's mother both underwent puberty at a “normal” age.ASerum calcium and parathyroid hormone levelsBHead CTCFull karyotype analysisDFibroblast growth factor receptor 3 gene sequencingESerum IGF-1, somatomedin C, and IGF-BP3 measurement |
Serum IGF-1, somatomedin C, and IGF-BP3 measurement |
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GHD is diagnosed by a combination of growth charting, serum screening, and GH stimulation testing. It is then treated by administering recombinant GH injections several times per week until the child’s adult height potential is reached. |
true |
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A 2-week-old infant girl is brought to the hospital following a seizure. She was born at 39 weeks gestation following an uncomplicated pregnancy. Her neonatal course has been complicated by persistent jaundice. Her mother notes that she has been sleepier over the last few days. She says that she exclusively breastfeeds her daughter. Laboratory results are remarkable for normal alanine and aspartate aminotransferases but unconjugated bilirubin of 30mg/dL. Hercomplete blood count is within normal limits, and peripheral blood smear is unremarkable. Which of the following is the most likely underlying cause of her seizure? AAbsence of UDP-glucuronosyl transferase ExplainBIncreased intestinal absorption of bilirubin Though inflammatory bowel diseases such as Crohn disease and ulcerative colitis can increase the risk of colon cancer, they are not due to a mutation within the DNA mismatch repair mechanism. CDecreased level of bilirubin conjugation enzyme ExplainDDefective liver excretion of bilirubin ECongenital hemolytic disorder |
Defective liver excretion of bilirubin |
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Lynch syndrome is an autosomal dominant disorder that is caused by a germline mutation in one of several DNA mismatch repair (MMR) genes or loss of expression of MSH2 due to deletion. |
true |
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A 6-year-old girl with no past medical history is brought to the emergency room after coughing up blood at home. Her mother reports that she had been sick with what appeared to be a "chest cold," for the last few days. About 2 weeks ago, when they returned from their visit to relatives living in Arizona, she had been in her usual state of health. She also reports that her daughter's chest has been hurting, and that she has been running a fever at home. The patient has not been vomiting, had diarrhea, or been exposed to any sick contacts that the mother is aware of. On exam, the child is febrile to 39.2C with otherwise stable vital signs. Her lung exam is largely benign except for a few expiratory wheezes and decreased breath sounds over the right middle lobe. Cardiovascular exam is benign. Achest X-ray is obtained and shows moderate hilar lymphadenopathy and a single thin-walled cavitary lesion. What is the most likely diagnosis in this child?AReactive airway diseaseBRSCHistoplasmosisDCoccidioidomycosisEBacterial pneumonia |
Coccidioidomycosis |
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A 2-day-old infant presents with cyanosis that has progressively worsened since birth. Interestingly, the patient has had no respiratory distress. On physical exam, you hear a clear single second heart sound with no thrills or rubs. You immediately request a chest radiography which demonstrates no cardiomegaly and normal pulmonary vasculature. On ECHO, the parasagittal plane imaging shows the aorta rising anteriorly from the right ventricle. Given the clinical scenario, which of the following is likely the best treatment option?AArterial switch procedure (Jatene procedure) |
Arterial switch procedure (Jatene procedure) |
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The ductus arteriosus typically closes in the first few hours of life. and the best procedure is the arterial switch procedure, which is performed in the first two weeks of life, as the left ventricle will still be able to support the systemic circulation. |
true The initial maintenance is prostaglandin E1 to sustain patency of the ductus arteriosus and until the surgical procedure is able to be performed. However, this is unlikely to treat the patient’s underlying problem and is only used for maintenance. |
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A 17-year-old overweight male, who has recently begun a new diet plan, presents to his primary care physician complaining that his friends have been telling him that he looks “a little yellow” sometimes. After a short history and a physical examination, the physician assures him that he is healthy. Laboratory studies reveal an elevated unconjugated bilirubin. One week later, his bilirubin is measured as normal. Of the following mechanisms, the one which best explains the pathogenesis of the most likely diagnosis is?AAbsent glucoronyl transferase activity ExplainBIncreased intravascular hemolysis ExplainCReduced glucuronyl transferase activity |
Reduced glucuronyl transferase activity |
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Gilbert syndrome is a benign genetic disorder that presents as asymptomatic, transient rises inunconjugated bilirubin due to low activity of glucuronyl transferase. It can be exacerbated by fasting. |
true |
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A five-year-old child is brought into the ED by his parents, who report altered mental status after he fell and hit his head on the sidewalk. The radiologist's analysis of the CT reveals no evidence of trauma, but did uncover a well-circumscribed 3cm mass at the tectal plate (see below). Credit: Wikimedia CommonsYou consult the neurosurgeon on call, who recommends immediate excision. The surgery was successful and resulted in complete resection with clear margins. Following the surgery, the pathologist's report describes the tumor cells as being bipolar-appearing neurons with long, "hair-like" processes on H&E stain. Additionally, the pathologist notes the tumor is WHO grade I.This vignette and description is consistent with what type of brain tumor? |
juvenile pilocytic astrocytoma |
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juvenile pilocytic astrocytoma |
Pilocytic astrocytomas are low-grade (WHO grade I) gliomas characterized by hair-like bipolar neurons. They tend to grow as single lesions in the cerebellar hemispheres or near the 3rd ventricle, though they have also been observed in the cerebral hemispheres as well. The massestend to be well-circumscribed and easily resected, provided they are easily accessible for surgery. Brain tumors can have a wide range of clinical presentations, or can be occult or subtle in nature.Pilocytic astrocytomas mainly affect children aged 5-14 years, and arise at a rate of 2 in 100,000 people. Some sporadic cases have been associated with "gain" mutations in chromosome 7q34 at the BRAF locus. |
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A 6 year old girl presents with new-onset acute abdominal and pelvic pain. She is having trouble describing the character of the pain, but says the pain is on the right side of her belly and also points to the area above her pubic bone. She denies nausea or vomiting, has no recent sick contacts, and review of systems is otherwise negative. Other than mild tachycardia, her vital signs are normal. What should be your next step in diagnosis?AAbdominal ultrasoundBAbdomen and pelvis CT scanCPelvic ulstrasoundPelvic ultrasound may reveal ovarian pathology, but at this age and with the description of flank pain, abdominal ultrasound is more useful to look for both kidney stones and appendicitis.DPelvic exam without speculumExplainEAbdominal xray |
Abdominal ultrasound |
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In female children presenting with pelvic pain, abdominal/pelvic ultrasound should be the first ordered to aid diagnosis whenever possible. Pelvic exam can be distressful to a child and should be avoided if there is no indication in the history and other causes can be explored first. |
true |
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A 10-year-old girl presents to her pediatrician complaining of pain in her right wrist preventing her from writing in school. Her mother mentions that two days ago she had elbow pain which they believed was from too much basketball, because last week after a basketball game she complained of left knee pain for a few days. She has been otherwise healthy except for a bad sore throat about a month ago. On exam she has a fever of 100.6F and a II/VI systolic murmur best heard at the apex. While labs are pending, you order an electrocardiogram, which reveals a prolonged PR interval. What other findings might you expect?AMetabolic acidosisBElevated erythrocyte sedimentation rate (ESR)CRetinal hemorrhagesDPositive anti-nuclear antibodies (ANA)ERash on cheeks and bridge of nose |
Elevated erythrocyte sedimentation rate (ESR) |
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Rheumatic fever is diagnosed by the presence of a Group A Strep infection and using the Jones Criteria. |
true |
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pathophys of rheumatic fever |
Pathophysiology is antibody cross-reactivity between GAS's M protein and antigens in myocardium, joints, and CNS tissue. This molecular mimicry causes a Type II hypersensitivity reaction. Rheumatic heart disease can be temporary or chronic. It can predispose patients to left heartdilation, left ventricular hypertrophy, congestive heart failure,arrhythmia, and bacterial endocarditis. |
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jones criteria |
The five major manifestations spell JONES (pretend the heart is shaped like an O): Joints: migratory arthritis, predominantly of large jointsCarditis and valvulitisNodules (subcutaneous)Erythema marginatumSydenham chorea (central nervous system involvement) The four minor manifestations are:ArthralgiaFeverElevated acute phase reactants (erythrocyte sedimentation rate [ESR], C-reactive protein [CRP])Prolonged PR interval |
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rheumatic fever |
This patient meets the Jones criteria for acute rheumatic fever, most common in children aged 5-15 years old and 2-4 weeks after a pharyngeal (not skin) infection by group A streptococcus. Jones criteria requires Group A Streptococcus (GAS) infection followed by 2 major or 1 major and 2 minor manifestations, listed below. |
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An eighteen year old college student presents to the student health clinic complaining of purulent discharge and pain on urination. A culture is taken and he is treated with ceftriaxone and azithromycin. A gram stain of the discharge is done and gram-negative diplococci are observed. Which of the following carbohydrates can be fermented by the infectious agent?AGlucose, maltose, and lactoseBMaltose and lactoseCGlucose onlyDLactose onlyEMaltose onlyFSorbitol onlyGGlucose and lactoseHGlucose and maltose |
Glucose only |
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Neisseria meningitidis is a Gram-negative diplococcus that ferments both glucose and maltose. |
true |
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A 3-year-old girl presents to your clinic with an 8 day history of fever, vomiting and watery diarrhea. There has been mild improvement in the last few weeks as the frequency of the patient's stool has decreased in the last few days, and the stool is more formed. Mother denies the patient has had any seizures and reports the child has had a few sick contacts. Patient goes to a day care about 4 days a week and three other children have had similar symptoms. Mother denies any history of travel or changes in diet. On physical exam the patient has normal vital signs with a moderate amount of stool in her diaper. She has an otherwise normal exam with no concerns regarding hydration. You prescribe fluids to the patient. What is the best treatment option available to help prevent further breakout of this patient's most likely condition? ABoiling all the drinking water at the day care centerExplainBPenicillin treatment for the affected childrenExplainCReturn back to daycare with stool well contained in diaper |
Return back to daycare with stool well contained in diaper |
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Viral gastroenteritis, which can be caused by rotavirus, is an acute and self-limited disease that does not require pharmacologic therapy. Initial therapy is directed toward correcting fluid deficit and electrolyte imbalance. |
true |
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A 4-year-old boy is brought to clinic because his mother has noticed enlarged lymph nodes and he seems to bebruising easily when he plays outside. He has seemed fatigued and irritable recently. Physical exam reveals severalbruises on his extremities and petechiae on his trunk. CBC reveals Hb 8.0g/dL, WBC 58,000/mL, and Platelets44,000/mL.After bone marrow biopsy and cytogenetics, he is diagnosed with Acute Lymphoblastic Leukemia. What feature onbone marrow biopsy is required to classify a leukemia as acute?A<20% erythroblastic cellsB<20% adipose cellsC>20% blast cellsD>50% blast cellsE>20% cellularity |
>20% blast cells |
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In acute leukemias, blast forms predominate - accounting for at least 20% of the total cellularity of a bone marrow biopsy sample. |
true |
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A previously healthy 5-year-old boy has been febrile for about 2 days and presents to your office with complaints of abrupt onset of vomiting and difficulty drinking fluids. He denies any diarrhea, rhinorrhea or upper respiratory symptoms. His mother notes that he just started kindergarten and there have been a few other children out sick. On physical examination, he is sleepy but arousable, and febrile with a temperature of 101.9F. His posterior oropharynx is erythematous with an inflamed uvula and enlarged symmetric masses (see image below) . He also has enlarged, tender anterior cervical lymphadenopathy. He is quite resistant to the physical examination, constantly moving his head. He moves his head vigorously in an effort to avoid your physical examination. A throat culture is currently pending. What is likely the best initial treatment? |
Oral penicillin |
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This is a classic case of streptococcal tonsillitis. The first line treatment is 10 days of oral penicillin |
true |
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A 20-month old child is brought to your office by his mother, who has noticed that he looks pale and seems to get tired more easily than other children when he is playing. He has not had any recent infections or unexplained fevers. She denies bleeding from the gums or easy bruising. On exam, you notice facial and subconjunctival pallor. He is mildly tachycardic. There is no jaundice, lymphadenopathy or organomegaly. He is reluctant to let go of his bottle during the exam, and you find out from his mom that he often drinks up to six 8oz bottles of whole milk per day. Which of the following is a reason why excess intake of cow's milk can result in this child's symptoms?AThe child is most likely lactose intolerant, and the resulting inflammation causes decreased absorption of iron from the gutExplainBCows milk protein can irritate the lining of the gut, causing chronic blood lossCThe calcium in milk interferes with the absorption of folic acid from the gutExplainDThe alkaline components in milk affect the activity of intrinsic factor, decreasing uptake ofvitamin B12ExplainECow's milk is low in calories, therefore the child's fatigue is due to inadequate intake of calories |
Cows milk protein can irritate the lining of the gut, causing chronic blood loss |
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Iron deficiency anemia is often seen in children under 2 years who are fed excess cow's milk. The reason is threefold:cow's milk is a poor source of dietary iron; certain milk proteins can irritate the gut lining and cause chronic, occult blood loss; and last, the calcium ions in milk can interfere with iron absorption in our gut. |
true |
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Acute leukemia comprises what percent of pediatric malignancies? |
30% |
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A 10 day old infant is brought to the ER by his parents because he is having difficulty feeding and is jaundiced. He was born at 36 weeks gestation via spontaneous vaginal delivery when mother presented to preterm premature rupture of the membrane. After birth, the infant did well and was discharged on day 6. The infant was breast fed every 2-3 hours for 20 to 30 minutes per breast. The infant was fine until 2 days ago when he was having difficulty feeding and sleepier than usual. When the infant was awake he was hard to console and fussy. The infant is afebrile, but had 5 episodes of non-bloody, non-billious emesis since yesterday.Vitals:body temperature: 95Fblood pressure 80/49pulse: 155respirations: 38Physical Exam:General: face, neck and chest is jaundicedHead and Neck: full with no bulging fontanelles, dry mucous membrane, scleral icterus, full range of motion of the neckNeurological Exam: lethargic and decreased toneCardiovascular, respiratory, and abdominal exam: normalWhat is the most important step in management?Aorder a CT scan of the headExplainBobtain C-reactive protein levelsExplainCdiscontinue breastfeedingThe infant is presenting with a number of clinical findings (e.g. difficulty feeding, fussiness, andlethargy) that are not well explained by breastfeeding jaundice.Dperform a lumbar puncture and collect blood cultures |
perform a lumbar puncture and collect blood cultures |
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Infants that are lethargic and/or inconsolable need to have a full sepsis work up performed which includes a CBC, UA, lumbar puncture, blood culture, and urine culture. |
true |
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You are in the newborn nursery completing an examination on a female infant who was born 12 hours ago. She was full term and weighed 3650g. She had an uncomplicated vaginal delivery with an Apgar score of 9. She has begunbreastfeeding and is latching on well. On examination, you notice that her right foot is not entirely symmetrical to the left one. The right forefoot is adducted, while the midfoot and hindfoot are normal. The forefoot can be passively manipulated into abduction. There is full range of motion at the ankle and at the subtalar joint. What is the most appropriate management of her deformity at this time?ASerial castingBStretching exercisesCSpecial outflare shoesDSurgical correctionEObservation and reassurance |
EObservation and reassurance |
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Metatarsus adductus is a foot deformity caused by intrauterine moulding. Treatment will depend on how rigid the foot is when manipulated. The majority of cases resolve spontaneously, with only observation and reassurance to theparents. |
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An infant born at 26 weeks gestation is found, on fundoscopic exam, to have an ophthalmic condition thought to be related to incomplete retinal vascularization and increased permeability of immature vessels. More than half her retinais involved with stage III disease, and dilated, tortuous vessels are seen around the optic disc on exam. What should be done to manage her condition? |
Laser photocoagulation |
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The majority of premature infants with ROP heal without treatment. However, when threshold ROP is reached, the indicated treatment is ablation of the peripheral avascular retina by laser photocoagulation. |
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treatment for retinopathy of prematurity if it doesnt heal by itself |
ablation with laser photocoagulation |
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You receive a call from a frantic mother one day because an outbreak of chickenpox has been reported at her son Jim's daycare. She is concerned about her older daughter Carrie, who just started chemotherapy treatment for ALL. The whole family was at the hospital visiting her yesterday, including Jim. Carrie is vaccinated but Jim is not. Neither has had chickenpox before. Their mom has heard how bad infections can be in kids undergoing chemotherapy, and she is extremely worried for Carrie. What is the best course of management?ABoth should receive the chickenpox vaccine at this timeBJim should get supportive treatment if he gets sick, and Carrie should get prophylactic acyclovirCSince Jim has no lesions, he is not contagious, so reassure the mom that she has nothing to worry about.DJim should receive the chickenpox vaccine and Carrie should receive varicella zoster immunoglobulin (VZIG)EBoth should receive prophylactic acyclovir |
Jim should receive the chickenpox vaccine and Carrie should receive varicella zosterimmunoglobulin (VZIG) |
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Jim should receive the chickenpox vaccine and Carrie should receive varicella zosterimmunoglobulin (VZIG) |
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The varicella vaccine is recommended in healthy patients who have or have not been exposed to the highly contagious virus. As it is a live attenuated vaccine, it is NOT recommended in immunocompromised individuals. Instead, these patients receive VZIG: varicella zoster immunoglobulin. |
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A 2-year-old male is brought to the emergency department for high fever and rash. History reveals the fever has been ongoing for five days. His pediatrician saw him two days ago and felt the fever and rash were likely due to a non-specific viral infection. Since his appointment, he has been refusing to eat solids and has only been taking sips from his bottle and cups. He has become even more irritable. Vital signs reveal a blood pressure of 90/60 mmHg, a heart rate of 110/min, a temperature of 39.6 C(103.2 F), and a respiratory rate of 25/min. Physical examination reveal conjunctival injection bilaterally. His tongue is bright red and edematous. Tonsils are small but erythematous and without exudate. There is a 1.5 cm lymph node along the left anterior cervical chain and a 2 cm supraclavicular lymph node on the right. He has a diffuse, erythematous maculopapular type rash on his chest, abdomen and back extending into the groin region. His hands and feet are red and painful to touch. The patient is admitted to the hospital for dehydration and treatment of his underlying condition. Laboratory analysis reveals a white blood cell count of 13,000 cells/µL with a normal differential, mild anemia, and an elevated erythrocyte sedimentation rate. In addition to immediate intravenous hydration, which of the following is the next appropriate step in management? AAcetaminophen BIbuprofen CCeftriaxone DIntravenous immunoglobulin EAspirin and intravenous immunoglobulin |
Aspirin and intravenous immunoglobulin |
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Kawasaki Disease may be complicated by the development of coronary artery aneurysm. |
IVIG and aspirin significant reduce the incidence of coronary artery aneurysm formation. |
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A 40-year-old woman comes to the clinic because she has been having episodes of fever and chills every 3 days for the past several weeks. She recently returned from Southeast Asia, where she was in an automobile accident and received a blood transfusion. On physical examination today, she is noted to be jaundiced and to havehepatosplenomegaly. A blood smear reveals ring-shaped structure in a few of her red blood cells. The most likely infection is which of the following?Credit: Wikimedia CommonsAPlasmodium BTrypanosoma ExplainCLeishmania ExplainDToxoplasma Toxoplasma cause toxoplasmosis and tends to infect lymph nodes, brain, and muscle.EBabesia microti Explain |
Plasmodium |
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Thick and thin films is routinely used for malaria diagnosis; thick films should be stained withGiemsa or Field stain, thin films with Giemsa or Leishman stain. Within the erythrocytes the merozoites grow first to a ring-shaped form and then to a larger trophozoite form |
true VUR is the retrograde flow of urine from the bladder, up to the ureters or kidneys. VUR predisposes to recurrent infections and renal scarring, which can eventually lead to renal failure. VUR can be managed in three ways: surveillance, antibiotic prophylaxis or surgical repair. |
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VUR inevitably progresses with time, eventually resulting in renal scarring |
VUR is not inevitably progressive. Reflux spontaneously resolves in many young children, but this is less likely as puberty approaches. However, VUR that is bilateral, or grade 4 or 5 at presentation, is less likely to resolve spontaneously |
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A 32-year-old gravida 1 para 1 woman is in her 26th week of gestation and has an oral glucose tolerance result of 155mg/dL. For which of the following will her newborn be at increased risk?AHyperglycemiaBAnemiaCNeonatal SeizureDHypercalcemiaECoarctation of the aorta |
Neonatal Seizure |
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Infants born to mothers with diabetes are at risk for a host of conditions, including neonatal seizures. Diabetes screening during antenatal care is vital. |
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A concerned mother brings her 3-year-old daughter to the pediatrician because of "on-and-off fevers" for the past week. She reports that her daughter has been having daily fevers to 103F each morning and that she wakes up looking quite sick. Later during the day, however, she appears normal and has a normal body temperature. The fevers sometimes return later in the day. Her mother also reports that the child has a "salmon-colored rash" that is most prominent when she is having a fever. On exam, the patient is currently febrile to 102F and has a macular, pink rash mainly present over the axillae and waist. You also note hepatosplenomegaly and several swollen joints that are not particularly tender. CBC is notable for leukocytosis to 20,000 and thrombocytosis to 750,000. The differential is normal. What is the most likely diagnosis in this child?ASystemic onset juvenile idiopathic arthritisBAcute lymphoblastic leukemiaCSystemic lupus erythematosusDKawasaki diseaseEOsteosarcoma |
Systemic onset juvenile idiopathic arthritis JIA is an autoimmune condition characterized by an inflammatory response primarily attacking the joints. Joint pain, fevers, and a 'salmon-pink' rash are classic symptoms. Treatment includes NSAIDs for mild disease, orimmunosuppressants such as corticosteroids or methotrexate for more severe disease. |
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A 6-month-old patient in your practice is brought in for a well-child visit. His mother is eager to introduce solid foods, but is worried about the development of allergies. The patient's family history is significant for atopic dermatitis in his father and peanut and egg allergies in his mother. What is your recommendation for this parent?AAny and all foods (except honey) can be gradually introduced after 4-6 months of age |
Any and all foods (except honey) can be gradually introduced after 4-6 months of age |
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Children over 6 months of age can safely have any solid food introduced into their diet (except honey!). |
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