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713 Cards in this Set
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Another name for Henoch-Schonlein purpura
|
Anaphylactoid purpura
|
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Rx for encopresis
|
Behavioral modification and reassurance
If retentive, also must clear mass + stool softeners for 3-6mo |
|
What are genu varum and Blount disease?
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Bowed legs; latter is if persists past 2yo
|
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What is Legg-Calve-Perthes disease + most common presentation?
|
Avascular necrosis of the femoral head
Painlses limp |
|
Limp in overweight adolescents
|
Slipped acpital femoral epiphysis
|
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What is Osgood-Schlatter disease + treatment?
|
Repeated microfracture of tibial tubercle at insertion of patellar tendon
Overuse injury w/ swelling and knee pain Rest |
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Fever, erythema, extremely tender skin, Nikolsky sign, facial crusting
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Staphylococcal scalded skin disease
|
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Rx for human bite
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Hospitalization for antibitoics and debridgement + healing by secondary intention (granulation)
|
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Where are Baker cysts, and how are they managed?
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Popliteal cysts; observation, typically resolve over several years, surgery if associated symptoms
|
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Rx for lice
|
1% lindane, except in babies (risk of neurotoxicity from transdermal absorption, so use 1% permethrin (Nix))
|
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Hole in the tympanic membrane and prurulent drainage
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Acute otitis media
|
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White polyp extending thru tympanic membrane
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Cholesteatoma (congenital or acquired): small sac lined w/ epithelium-containing debris
Can present in childern w/ recurrent otitis media |
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When does umbilical cord normally separate, and what counts as delayed separation?
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10-14 days post birth
>1mo |
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Cause of delayed separation
|
Leukocyte adhesion deficiency
|
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Rx for umbilical granuloma
|
Silver nitrate
|
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Omphalocele is covered by -__ but no ___
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Peritoneum
Skin |
|
Best booster vaccine at 14yo
|
Tdap (tetanus toxoid, reduced diphtheria toxoid, acellular pertussis)
|
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Rx for mammalian bites
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Irrigation and cleaning
If infant, diabetic, or i/c, antibiotic prophylaxis |
|
Long face, large ears, prominent jaw, macroorchidism, hypotonia, repetitive speech, gaze avoidance, hand flapping, mental retardation
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Fragile X syndrome
|
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Inheritance pattern of fragile X syndrome
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Dominant X-linked disorder w/ reduced penetrance in females
|
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Friendly, hypercalcemia, short, blue irides, cardiopulmonary abnormalities
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Williams syndrome (ch. 7)
|
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Yellow-white lesions w/ surroudning erythema + eosinophils in newborn
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Erythema toxicum
|
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Flat vesicular lesions (splotchy red rash) on nape of neck, eyelids, etc., may persist
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Salmon patch (nevus simplex)
|
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Pustules --> hyperpigmented lesion upon rupture
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Pustular melanosis
|
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Yellow-orange sharply edged hairless lesions on head and neck of infants
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Sebaceous nevi
|
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Yellowish white, 1-2mm cysts scattered over the face and gingivae of the neonate
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Milia (on the palate, called Epstein pearls)
|
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Small, firm, white, cold patches of skin in exposed areas and treatment
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Frostnip
Rewarm before numb |
|
Progression and complications of frostbite
|
Stinging --> aching --> numb
Once rewarmed --> red, blotchy, painful Gangrene may develop |
|
Small ulcerated lesions on exposed areas
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Chillblains
|
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Destruction of fat cells caused by exposure to cold weather or a cold object
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Cold panniculitis
|
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Deficiencies: cheilosis, glossitis, ocular problems (keratitis, conjunctivitis, corneal vascularization), seborrheic dermatitis
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Riboflavin deficiency
|
|
Deficiencies: seizures, peripheral neuritis, dermatitis, microcytic anemia
|
Vita B6 deficiency
|
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Four signs of folate deficiency
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Megaloblastic anemia, glossitis, pharyngeal ulcers, impaired immunity
|
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Deficiencies: pellagra
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Niacin
|
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Deficiencies: dermatitis/ seborrhea
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Biotin
|
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Congenital abnormality with bushy eyebrows, hirsutism, limb defects, VSD, mental retardation
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Cornelia de Lange syndrome
|
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Painful limp with normal/mildly elevated WBC and ESR (+ recent URI)
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Transient synovitis (self-limited)
|
|
How soon should NAC be given for acetaminophen poisoning?
|
16 hours
|
|
Rx for salicylate poisoning
|
Acetazolamide, IV sodium bicarb (hemodialysis if needed)
|
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Toxicity: oculogyric crisis, tremors, torticollis, dysphagia
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Phenothiazine
|
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Rx for phenothiazine ingestion
|
Benadryl (IV or IM)
|
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Rx for iron intoxication (GI symptoms + CV collapse)
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Deferoxamine
|
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Rx for methanol intoxication
|
Induce emesis or gastric lavage
IV ethanol Hemodialysis if severe |
|
Excess vitamins: hyperostosis (excess bone growth), hepatomegaly, increased CSF pressure, dry skin
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Vitamin A
|
|
Excess vitamins: skin flushing and pruritis (long-term tachycardia, liver damage, hyperglycemia/uricemia)
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Nicotinic acid (vasodilator)
|
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Excess vitamins; kidney stones, diarrhea, cramps
|
Vitamin C
|
|
Excess vitamins: nausea, diarrhea, weight loss, polyuria, soft tissue calcification
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Vitamin D
|
|
Excess vitamins: sensory neuropathy with altered sensation of touch, pain, and fever
|
Pyridoxine (B6)
|
|
What does the nitroblue tetrazolium test look for?
|
Chronic granulomatous disease (predilection to infection with catalase positive organisms)
|
|
Thrombocytopenia, recurrent infections, ezcema
|
Wiskott-Aldrich syndrome
|
|
Heart defects, abnormal facies, hypocalcemia, thymic aplasia
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DiGeorge syndrome (CATCH)
|
|
Test for DiGeorge syndrome
|
Intradermal skin test w/ Candida albicans (will have no result b/c of T cell deficiency)
|
|
Consequences of congenital cystic adenomatoid malformation (CCAM)
|
Cystic illness, compression of affected lung --> pulmonary hypoplasia (Rx is surgical excision of affected lobe)
|
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Pharyngitis, fever w/ severe sore throat, refusal of food, drooling, noisy breathing
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Retropharyngeal abscess
|
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Fluctuant mass on neck palpation + sore throat
|
Retropharyngeal abscess
|
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Rx for chlamydial infection in newborns
|
Oral macroles (azithromycin): treats both conjunctivitis and pneumonia
|
|
Acid-base abnormality in tension pneumo
|
Uncompensated respiratory acidosis w/ hypoxia but no metabolic acidosis
|
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Rx for bronchiolitis
|
Monitor fluid status/ oxygenation
|
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Infants born to moms w/ gestational DM are at risk for these respiratory problems
|
Peripheral nerve injuries (Erb-Duchenne, phrenic nerve paralysis) --> asymmetric diaphragmatic motion in seesaw manner on chest US
|
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Rx for gas, kerosene, or furniture polish inhalation (hydrocarbons -->dyspnea, cyanosis, respiratory failure)
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ABG/ pulse ox, intubation if necessary
|
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Recurrent "pneumonias," hemoptysis, digital clubbing, anemia
|
Idiopathic pulmonary hemosiderosis
|
|
Diagnostic method for idiopathic pulmonary hemosiderosis
|
BAL w/ hemosiderin-laden macrophages
|
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Idiopathic pulmonary hemosiderosis and hypersensitivity to cow's milk + Rx
|
Heiner syndrome (Rx: remove cow's milk from diet)
|
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Newborn w/ respiratory distress that improves with crying
|
Choanal atresia
|
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Croup presentation + fever, toxic appearance, biphasic stridor
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Bacterial tracheitis
|
|
Rx for bacterial tracheitis
|
Intubation + IV Abx
|
|
Comon infection in dirt-eating children
|
Visceral larva migrans from Toxocara canis (common parasite of dogs)
|
|
Rx for sinusitis
|
Oral Abx for 10-14d
|
|
When are sinuses large enough to be infected?
|
Maxillary/ ethymoid: infancy
Sphenoid: 3-5yo Frontal: 6-10yo |
|
Drooling, avoidance of foods, cough, choking
|
Foreign object in esophagus
|
|
Rx for ear infection
|
Amoxicillin 40mg/kg BID, i.e. 80mg/kg daily (high dose for good penetration)
|
|
Polyps and eosinophilia on nasal smear
|
Allergic rhinitis
|
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Respiratory acidosis w/ metabolic compensation
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Points to chronic airway obstruction, e.g. from hypertrophied tonsils or adenoids
|
|
Ingestions: lethargy, cma, seizures, widened QRS/BBB
|
TCA poisoning
|
|
Ingestions: N, V, diaphoresis, RUQ pain
|
Tylenol
|
|
Rx for TCA ingestion
|
ICU admit, TCA Fab Ab
|
|
Rx for methanol ingestion
|
Ethanol
|
|
Jones criteria: major
|
Carditis, arthritis, erythema marginatum, chorea, subQ nodules
|
|
Jones criteria: minor
|
Arthralgias, fever, Hx of rheumatic fever, increased ESR or CRP, increased WBC, anemia, prolonged PR and QT
|
|
Tetrology of Fallot
|
VSD
RVH RV outflow obstruction (pulm stenosis) Dextroposition of the aorta |
|
Transposition of the great vessels often has these other 2 abnormalities
|
PFO, hypoplastic L heart syndrome
|
|
When should kids w/ FH of early onset CHD be screened hypercholesterolemia?
|
2yo
|
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Edema around neck + FH
|
Hereditary angioedema
|
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Turner's is associated w/ this cardiac condition
|
Aortic coarctation
|
|
Down's Syndrome is associated w/ this cardiac condition
|
VSD/ endocardial cushion defects
|
|
Marfan's is associated with these cardiac conditions
|
Aortic dilation, AR, MR
|
|
Risk of congenital heart disease in population, w/ 1 child, w/ 2 children
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1%, 2-6%, 20-30%
|
|
Murmur of VSD
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Harsh or blowing holosystolic best along L lower sternal border
|
|
2 most common causes of myocarditis
|
Adenovirus and coxsackie virus B
|
|
Heart rate >250 beats/min indicates
|
Tachyarrhythmia (up to 200 can be CHF from many causes)
|
|
3 first-line treatments for SVT + 1 second-line
|
Vagal stimulation with:
Carotid massage Immersion of face in cold water Voluntary straining 2nd line: IV adenosine |
|
Quadruple rhythm (S3 + S4) + TR + middiastolic murmur at lower left sternum
|
Ebstein anomaly
|
|
What is the Ebstein anomaly
|
Downward displacement of the tricuspid valve
|
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Hypoplastic LV --> LAD and LVH is seen in this valvular disease
|
Tricuspid atresia
|
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Congenital heart disease w/ marked, fluffy appearing venous congestion (snowman) on CXR
|
Total anomalous pulmonary venous return
|
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Early cyanosis, normal sized heart, RAD and RVH
|
Transposition of the great vessels
|
|
Thrombocytopenia, bilateral absence of radius, abnormal thumbs, TOF and/or ASD
|
Thrombocytopenia absent radius syndrome
|
|
Short, downslanting palpebral fissues, ptosis, low set malformed ears, webbed neck, shieldlike chest, pulmonic stenosis, cryptorchidism, mental retardation
|
Noonan Syndrome (male Turner Syndrome, seen in both sexes)
|
|
Noonan syndrome is associated w/
|
Advanced paternal age
|
|
Main complication of neonatal lupus
|
Congenital heart block (permanent, usually requires pacing); most other manifestations are self-resolved
|
|
Common complications of pregnancy-induced HTN
|
Neonatal hypoglycemia and polycythemia
(Decreased uteroplacental blood flow --> fetal nutritional deprivation/ hypoxemia --> decreased glycogen storage and relative erythrocytosis) |
|
Symptoms of "hyperviscosity syndrome" in newborns
|
Tremulousness/jitteriness --> seizures due to sludging of blood in cerebral microcirculation
|
|
Rx for hyperviscosity syndrome
|
Parital exchange transfusion
|
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Common complication of postmature infants w/ chronic placental insufficiency (SGA and wasted appearance)
|
Asphyxia and/or meconium aspiration
|
|
Time frame of three types of conjunctivitis
|
Chemical: 6-12hrs of birth, lasts for 1 day, self-limited
Gonococcal: 2-5d after birth Chlamydial: 5-14d after birth |
|
Why are infants born to chlamydia-positive mothers not routinely treated with oral Abx?
|
Increased incidence of hypertrophic pyloric stenosis among neonates having received erythromycin
|
|
What is hyaline membrane disease?
|
Infant respiratory distress syndrome
|
|
Lung compliance, volume, and direction of blood shunt in infant respiratory distress syndrome
|
Decreases, decreases, and right --> left (From PDA or PFO or in lungs)
|
|
Most common TEF is
|
Blind esophageal pouch (below it, distal esophagus is connected to the trachea)
|
|
Teratogens: when is phenytoin dangerous
|
1st trimester
|
|
Teratogens: propanolol
|
Growth retardation, decreased ability to increase HR and CO, hypoglycemia, apnea
|
|
Bile stained vomitus indicates obstruction is where?
|
Distal to ampulla of Vater
|
|
Goat's milk contains inadequate ___ and ___
|
Folate, iron
|
|
Complications of cleft lip/ palate
|
Recurrent otitis media, hearing loss, speech defects
|
|
Which is repaired first, cleft lip or palate?
|
Clef lip within 3mo, cleft palate btwn 6mo and 5 yrs
|
|
Umbilical hernia, distended abdomen, large head/ fontanelles, hypothermia, feeding difficulties, constipation, jaundice
|
Congenital hypothyroidism
|
|
How long do babies with HIV+ moms receive zidovudine for?
|
6 wks
|
|
When is PCP prophylaxis started in HIV+ infants, and with what?
|
6wks, Bactrim
|
|
Weak cry, single umbilical artery, micrognathia, small pelvis, short sternum
|
Edwards Syndrome (also rocker bottom feet and clenched hands w/ index and third fingers overlapping)
|
|
Microcephaly, sloping forehead, scalp cutis aplasia (missing portion of skin and hair), microphthalmia, coloboma, omphalocele, single umbilical artery, hypersensitivity to atropine/ pilocarpine
|
Patau Syndrome
|
|
Another name for endocardial cushion defect?
|
Complete AV canal
|
|
Common lab finding in leukocyte adherence deficiency?
|
Neutrophilia
|
|
Rx for SCID
|
BM transplant (otherwise death in first 12-24mo)
|
|
Supplements needed in infants receiving goat's milk
|
Folate, B12, iron
|
|
Possible infection in babies receiving goat's milk
|
Brucellosis
|
|
FTT, bleeding problems, fontanelle fullness, hemolytic anemia
|
Cystic fibrosis (last three are from deficiencies of vitamins K, A, and E, respectively)
|
|
Type of formula used in galactosemia
|
Soy-based
|
|
Causes of rickets
|
Inadequate vita D, liver failure, renal failure, primary hypophosphatemia
|
|
How does liver failure lead to rickets
|
Low bile salts --> decreased absorption of fat soluble vitamins
|
|
Genetics of primary hypophosphatemia
|
X-linked
|
|
Lower extremity bowing with low Ca/ PO4
|
Familial primary hypophosphatemia
|
|
How to distinguish rickets from Schmid metaphyseal dysplasia
|
Latter has normal Ca, PO4, and alk phos (alk phos almost always increased in rickets)
|
|
Short, leg bowing, waddling gait, fractures, normal labs
|
Schmid metaphyseal dysplasia
|
|
Deficiencies: nightblindness, conjunctivitis, keratomalacia
|
Vitamin A
|
|
Deficiencies: hemolytic anemia in premature infants
|
Vitamin E
|
|
Deficiencies: hoarseness, anorexia, restlessness, aphonia
|
Thiamine (Vita B1)
|
|
Deficiencies: neuritis, edema, cardiac failure
|
Vita B1 (is beriberi)
|
|
Deficiencies: cheilosis, glossitis, corneal vascularization
|
Riboflavin (Vita B2)
|
|
Deficiencies: dermentia, dermatitis, diarrhea
|
Niacin/ Vita B3 (is pellagra)
|
|
Deficiencies: irritability, convulsions, anemia
|
Pyridoxine/ Vita B6 (diff from presentations in adults)
|
|
Deficiencies: depression, hypotension, muscle weakness, abd pain
|
Pantothenic acid
|
|
Deficiencies: dermatitis, seborrhea, anorexia, muscle pain, pallor, alopecia
|
Biotin
|
|
Excess: increased ICP, anorexia, hyperostosis (pain and swelling of long bones), alopecia, poor growth
|
Vita A
|
|
Excess: hypercalcemia, azotemia, N/V/D, calcinosis of tissues
|
Vita D
|
|
Excess: kidney stones
|
Vita C
|
|
Excess: flushing, pruritis
|
Niacin
|
|
Excess: sensory neuropathy
|
Pyridoxine
|
|
Intraventricular hemorrhage is often seen in these newborns and often leads to this complication
|
Preterm
Posthemorrhagic hydrocephalus |
|
Tacypnea, retractions, grunting, maybe cyanosis in a newborn
|
Transient tachypnea of the newborn
|
|
Maculopapular peeling rash, rhinitis, hepatosplenomegaly, lymphadenopathy, saddle nose, interstitial keratitis
|
Congenital syphilis (also Hutchinson teeth, mulberry molars)
|
|
Risks associated w/ maternal cocaine use
|
Vascular accidents
|
|
Causes of apnea in premature infants
|
Idiopathic apnea
|
|
Causes of apnea in term infants
|
Sepsis, reflux, congenital heart disease, seizures, hypoglycemia, airway obstruction
|
|
Signs of meningitis usually absent in neonates
|
Nuchal rigidity, Kernig/ Brudzinski signs
|
|
When to suspect twin-twin transfusion
|
Difference in hematocrit > 15, difference in body weight > 20%
|
|
Donor twin presentation in twin-twin transfusion
|
Oligohydramnios, anemia, hypovolemia, maybe shock
|
|
Recipient twin presentation in twin-twin transfusion
|
Hydramnios, plethora, hyperviscosity, respiratory distress, hyperbili, hypocalcemia, renal vein thrombosis, CHF, convulsions
|
|
Hydramnios refers to
|
Polyhydramnios
|
|
4 conditions associated w/ polyhydramnios
|
Duodenal atresia, TEF, trisomy 18, anencephaly
|
|
Congenital infections: cataracts, IUGR, myocarditis, macular rash, structural heart defects
|
Rubella
|
|
Congenital infections: hydrocephalus, chorioretinitis, intracranial calcifications
|
Toxoplasmosis
|
|
Congenital infections: IUGR, hepatosplenomegaly, jaundice, petechiae, intracranial calcifications, hearing loss
|
CMV
|
|
Most common congenital infection
|
CMV
|
|
Dysmorphic child, bilateral renal agenesis, primary pulmonary hypoplasia
|
Potter sequence/ oligohydramnios
|
|
Aniridia is associated w/
|
Wilms tumor
|
|
Lateral displacement of medial canthia, broad nasal brige, medial hyperplasia of eyebrows, partial albinism, deafness
|
Waardenburg syndrome
|
|
Complications of Sturge-Weber
|
Vascular malformation in trigeminal nerve distribution, grand mal seizures, mental deficiency, hemiparesis or hemianopsia
|
|
Inheritance of tuberous sclerosis
|
AD
|
|
Soft tissue swelling of scalp involving delivery portion of heart that is ecchymotic
|
Caput succedaneum (self resolves)
|
|
Difference between caput succedaneum and cephalohematomas)
|
Latter doesn't cross suture lines
|
|
Fever, muscle rigidity, metabolic and respiratory acidosis, tachycardia, arrhythmia, tachypnea, cyanosis, myoglobinuria, elevated CK, ARF
|
Malignant hyperthermia
|
|
Inheritance of malignant hyperthermia and what the gene codes for
|
AD
Ryanodine receptor (calcium release channel) |
|
Fever, emesis, multiple sclerosis type MRI
|
Acute disseminated encephalomyelitis
|
|
Rx for ADEM
|
High-dose corticosteroids
|
|
Poor growth, arachnodactyly, osteoporosis, dislocated lenses, mental retardation, thromboembolism
|
Homocystinuria (thromboemboli can cause acute infantile hemiplegia)
|
|
Very large head in normal newborn
|
Simple primary craniosynostosis (rx: surgery)
|
|
Seizure activity with no epileptiform activity on EEG
|
Pseudoseizure
|
|
Differences between pseudoseizure and seizure on exam
|
Pseudoseizure: no loss of bowel/bladder control, no self-injury, normal pupillary response to light
|
|
What is Todd paralysis?
|
Paralysis after focal or Jacksonian seizure; lasts 24-48hrs
|
|
Macular cherry red spots, developmental deterioration and sensitivity to noise
|
Tay Sachs disease
|
|
Enzyme absent in Tay Sachs
|
Beta-hexosaminidase A
|
|
Beta-glucosidase deficiency --> increased tone, strabismus, organomegaly, FTT, stridor, psychomotor regression
|
Gaucher disease
|
|
Beta-galactosidase --> angiokeratomas in bathing suit area + severe pain
|
Fabry disease
|
|
Galactocerebroside beta-galactosidase deficiency --> irritability, seizures, hypertonia, optic atrophy
|
Krabbe disease
|
|
Sphingomyelinase deficiency --> hepatosplenomegaly, LAD, psychomotor retardation w/in first 6mo
|
Niemann-Pick disease
|
|
Psychomotor retardation, ptosis, baldness, hypogonadism, facial immobility, neonatal respiratory distress, cataracts
|
Myotonic muscular dystophy
|
|
Difference btwn myotonic muscular dystrophy and other myopathies
|
Distal muscle weakness (instead of proximal)
|
|
Two things that can terminate migraine headaches
|
Vomiting or sleep
|
|
Three causes of CSF lymphocytosis with elevated protein and low glucose
|
TB meninigitis, viral meninigitis, meningeal leukemia, and medulloblastoma
|
|
Cranial nerve abnormalities, unsteady gait 2/2 spasticity, behavioral changes, UMN signs
|
Pontine glioma
|
|
Brain abscess are more commonly seen in patients with
|
Cardiac defects that have R --> L shunts associated
|
|
When are spinal taps contraindicated?
|
Increasing intracranial pressure
|
|
Unilateral pupillary dilation, hemiplegia, focal seizures, depressed conciousness after a fall
|
Epidural hematoma
|
|
3 components of Cushing's triad for increasing ICP
|
Increasing BP, decreasing HR, irregular respiration
|
|
Tumor causing poor growth, diabetes inspidus, papilledema
|
Craniopharyngioma
|
|
Acute ataxia in kids
|
Infection or drug intoxication (others would be more chronic)
|
|
Most common tumors in kids 1-10yo (vs. <1 or >10)
|
Infratentorial (posterior fossa: cerebellar and brainstem) vs. supratentorial
|
|
Storage diseases: acroparesthesia, intermittent painful crises of extremities or abdomen, cataracts
|
Fabry disease (deficiency of alpha-galactosidase)
|
|
Girl who has lost the use of her hands and ability to communicate/socialize
|
Rett syndrome
|
|
Storage diseases: progressive ataxia, weakness, progressive ataxia, gray macular lesions
|
Metachromatic leukodystrophy (deficient activity of galactosyl-3-sulfate-ceramide sulfatase)
|
|
CSF abnormality in Guillain Barre
|
High protein
|
|
Peroneal and intrinsic foot muscle atrophy, later extending to intrinsic hand muscles and proximal legs
|
Charcot-Marie-Tooth
|
|
Where are the intracranial calcifications in congenital CMV vs. toxo?
|
CMV: periventricular
Toxo: cortex |
|
Alternative name, cause, and presentation of Werdnig-Hoffman disease
|
(Infantile progressive) spinal muscular atrophy: anterior horn disease --> hypotonia, weakness, and delayed developmental motor milestones
|
|
Migraine headache + aura that involves unilateral sensory or motor signs
|
Hemiplegic migraine
|
|
Migraines are also known as
|
Migraines
|
|
Headache associated w/ menstruation
|
Vascular headache
|
|
What is echolalia?
|
Automatic repetition of vocalizations made by someone else
|
|
Common drug reaction from phenothiazines
|
Dystonia (neck, arm, or leg spasms)
|
|
Rx for dystonia induced by phenothiazines
|
Diphenhydramine
|
|
What is Sydenham chorea?
|
Acquired chorea of childhood, seeen after group A strep infections (associated w/ rheumatic heart disease)
|
|
Other signs seen with Sydenham chorea
|
Hypotonia, emotionally labile, milkmaid grip, darting tongue, spooning of an extended hand (flexion at wrist and extension of fingers)
|
|
Cafe au laits, axillary freckling, Lisch nodules (hamartomas), bony lesions, optic glioma
|
Neurofibromatosis Type 1 (usually also have neurofibromas)
|
|
PHACE syndrome
|
Posterior fossa malformations
Hemangiomas Arterial anomalies Coarctation or other heart problems Eye abnormalities |
|
Most common first sign of PHACE syndrome
|
Unilateral facial lesion (similar to Sturge-Weber) + blindness
|
|
Large unilateral cafe au lait spots, fibrous dysplasia of bones, precocious puberty, goiter
|
McCune-Albright Syndrome
|
|
Cause of hyaline membrane disease in newborns
|
Inadequate surfactant production in premies (--> atelectasis --> perfused but non-ventilated alveoli --> hypoxemia)
|
|
Most common cause of stroke in childhood
|
SCA
|
|
Teenager with gait ataxia, dysarthria, absence of DTRs, nystagmus
|
Friedrich ataxia
|
|
Affected spinal regions and inheritance of Friedreich ataxia
|
AR
Spinocerebellar tracts, posterior column, pyramidal tract |
|
Non-neuro complications of Friedreich ataxia
|
HCOM, diabetes, skeletal deformities (scoliosis and hammer toes); cardiomyopathy is most common cause of death
|
|
Macrosomia, macroglossia, visceromegaly, omphalocele, hypoglycemia, hyperinsulinemia
|
Beckwith-Widermann syndrome
|
|
Differentiating congenital hypothyroidism from Beckwith-Wiedemann syndrome
|
Hypothyroid: umbilical hernia, macrocephaly
BW: omphalocele, microcephaly, pancreatic overactivitiy (hypoglycemia/ hyperinsulinemia) |
|
4 complications of infants of diabetic mothers
|
Caudal regression syndrome
Duodenal atresia and small L colon Transposition of great vessels Anencephaly and NTDs |
|
Findings of WAGR syndrome
|
Wilms tumor
Aniridia Genitourinary anomaly mental Retardation |
|
Most common symptom with sickle cell trait
|
Painless hematuria
|
|
Complications of severe cough in pertussis
|
Recal prolapse, epistaxis, pneumothoraces
|
|
5 times when jaundice in a newborn should be investigated
|
- If it presents before 24hrs of life
- If > 12 (or >10-14 in premies) - If direct bili > 2 - Rate of increase is > 5/day - Persists after 10-14 days |
|
Progressive pancytopenia and macrocytosis, cafe au lait spots, microcephaly, short, horshoe kidneys, absent thumbs
|
Fanconi's anemia
|
|
Macrocytic pure red aplasia w/ congenital anomalies (short stature, webbed neck, clef lip, shielded chest, triphalangeal thumbs
|
Diamond-Blackfan syndrome (congenital hypoplastic anemia) (Rx is corticosteroids)
|
|
Therapy to remove lesions in Sturge-Weber
|
Argon laser therapy
|
|
Rubeola
|
Measles
|
|
Underlying abnormality in HSP
|
Small vessel vasculitis
|
|
Tetrad of HSP
|
Lower extremity palpable purpura, arthritis/ athralgias, abdominal pain, renal disease
|
|
Rx for HSP
|
Supportive, NSAIDs if in significant pain
|
|
What type of infections are most commonly seen in DiGeorge syndrome?
|
Fungal
|
|
Rx for Kawasaki disease
|
Aspirin and IVIG
|
|
Cyanotic infant with left axis deviation
|
Tricuspid atresia
|
|
Glomerular abnormality in HSP
|
Mesangial deposition of IgA
|
|
Risk factors for infant respiratory distress syndrome
|
Prematurity, maternal diabetes, male sex, cesarean section, perinatal asphyxia
|
|
What decreases the risk of infant RDS?
|
Stress in utero stimulates fetal lung maturity, e.g. prolonged ROM, IUGR, maternal HTN
|
|
Rx for Turner's Syndrome with 46X/46XY
|
Bilateral abdominal gonadectomy (risk of gonadoblastoma)
|
|
Congenital infections: hepatomegaly, cutaneous lesions, jaundice, anemia, rhinorrhea
|
Syphilis
|
|
Gland responsible for adrenarche (e.g. axillary hair growth)
|
Adrenal gland
|
|
Clinical concern with premature adrenarche and thelarche?
|
Limited, usually benign
|
|
Clinical concern with premature pubarche (pubic hair growth before the age of 8)
|
CNS disorder associated in 50%
|
|
Which is covered by a protective membrane, gastroschisis or omphalocele?
|
Ophalocele
|
|
Which is associated with other congenital abnormalities gastroschisis or omphalocele?
|
Omphalocele
|
|
Treatment for both gastroschisis and omphalocele?
|
Vaginal delivery, protective wrapping, orogastric tube, IV antibiotics, surgery
|
|
Vitamin deficiency: sore through, hyperemic and edematous oropharyngeal mucous membranes, cheilitis, stomatitis, glossitis, normocytic-normochromic anemia, seborrheic dermatitis, photophobia
|
Riboflavin
|
|
Vitamin deficiency: infantile fulminant cardiac syndrome with cardiomegaly, tachycardia, cyanosis, dyspnea, and vomiting
|
Infantile beriberi
|
|
Vitamin deficiency: symmetric peripheral neuropathy
|
Dry beriberi
|
|
Vitamin deficiency: neuropathy + cardiac involvement (cardiomegaly, cardiomyopathy, CHF, peripheral edema, tachy)
|
Wet beriberi
|
|
Hypoglycemic seizures, lactic acidosis, and hyperlipidemia in a 3mo w/ a doll face, thin extremities, and protuberant abdomen
|
Von Gierke's disease/ Type 1 glycogen storage disease
|
|
Von Gierke's disease is a deficiency of ?
|
Glucose-6-phosphatase
|
|
Floppy baby in first few weeks of life with hepatomegaly, feeding difficulties, macroglossia, and heart failure (disease + deficiency)
|
Pompe's disease, acid maltase deficiency
|
|
How to distinguish type 1 glycogen storage disease from type III?
|
Type III has elevated liver transaminases, fasting ketosis, and normal lactate and uric acid concentrations
|
|
Presentation and major complication of branching enzyme deficiency (type IV glycogen storage disease)
|
Hepatosplenomegaly and FTT --> cirrhosis
|
|
Rx for clavicular fracture during delivery
|
Nothing!
|
|
Most common first symptom of autism
|
Lack of a social smile
|
|
Triad of McCune Albright syndrome
|
Precocious puberty, cafe au lait spots, and polyostotic fibrous dysplasia (--> multiple bone defects)
|
|
GI tract polyposis and mucocutaneous pigmentation
|
Peutz-Jeghers syndrome
|
|
Marfan's + thromboembolic events
|
Homocystinuria
|
|
Homocystinuria is a deficiency of
|
Cystathionine synthase
|
|
Lab findings in homocystinuria
|
Elevation of both homocysteine and methionine
|
|
Rx for homocystinuria
|
High doses of vita B6; if unresponsive, restriction of methionine along with supplementation of cysteine
|
|
When does Becker's muscular dystrophy present?
|
After 5yo (Duchenne's is before), usually around age 12
|
|
Rx for liquid alkali ingestion?
|
Assess airway; if patent, next step is upper GI endoscopy to assess damage
|
|
How and when does transposition usually present?
|
With cyanosis and tachypnea within the first few days of life
|
|
Ingestions: vomiting and agitation progressing to lethargy and restlessness, with convulsions, hyperammonemia, elevated LFTs and LDH, and hypglycemia
|
Reye's syndrome (salicylate ingestion)
|
|
Biopsy of liver, kidneys, or brain in Reye's syndrome reveals
|
Microvesicular steatosis
|
|
Rx for Reye's
|
Supportive
|
|
Rx for scarlet fever?
|
Penicillin V
|
|
Rx for swallowed foreign object
|
If in esophagus: immediate endoscopic removal
If distal to esophagus: monitor for excretion |
|
Lytic bone lesion + hypercalcemia
|
Langerhans cell histiocytosis (if solitary long bone lesion)
Primary hyperparathyroidism (usually from parathyroid adenoma) causes multiple lesions |
|
Low temp and jaundice in a neonate
|
Do FIN work-up: jaundice can be present in sepsis
|
|
Rx for pertussis
|
Macrolide
|
|
What are Howell-Jolly bodies, and what do they indicate?
|
Nuclear remnants of RBCs that are normally removed by the spleen; indicate asplenia (e.g. in SCD)
|
|
Work-up and Rx for transient synovitis
|
None!
Ibuprofen and restriction of weight-bearing |
|
4 criteria that make septic arthritis more likely than transient synovitis and justify a work-up
|
- Fever
- WBC count > 12 - Refusal to bear weight - ESR > 40 |
|
What does the rash in niacin deficiency look like, and which pts are at risk for niacin deficiency?
|
Sunburn
Pts with bowel diseases (e.g. ulcerative colitis) who have absorption problems |
|
Vitamin deficiencies: irritability, depression, dermatitis, stomatitis, elevated homocysteine levels (--> atherosclerosis)
|
Vita B6 (pyridoxine) deficiency
|
|
How to distinguish myocarditis from rheumatic fever after a viral prodrome?
|
Myocarditis causes CHF symptoms, rheumatic fever causes more systemic symptoms
|
|
In a CF exacerbation, what bug are you making sure to cover for?
|
Pseudomonas
|
|
Antibiotic regimen for CF exacerbation?
|
IV ceftazidine (or Zosyn) + IV aminoglycoside (want double coverage)
|
|
Rx for impetigo
|
Topical mupirocin (or oral erhythromycin)
|
|
Two most common causative agents of impetigo
|
GAS and S. aureus
|
|
Impetigo is associated with this disease a few weeks afterward?
|
PSGN
|
|
When are IV fluids indicated for rehydration after gastro?
|
When clinical symptoms of dehydration are present: decreased skin turgor, dry MM, tachy, decreased urine output, delayed cap refill (2-3sec)
|
|
Endocrine symptoms, paralysis of vertical gaze, eyelid retraction
|
Pinealoma
|
|
Presentation and Rx for mild (Type I) cases of metatarsus adductus (congenital foot deformity)
|
Passive and action motion of the foot overcorrect into abduction
Reassurance |
|
Presentation and Rx for moderate (Type II) cases of metatarsus adductus
|
Motion of the foot corrects into neutral position
Orthoses |
|
Dx and Rx for erythema migrans
|
Lyme Disease
PO Amoxocillin (50mg/kg/day divided TID for 21 days) Doxycycline if >9yo |
|
Two X-linked immunodeficiencies
|
Bruton's and Wiskott-Aldrich
|
|
How to differentiate common variable immunodeficiency (CVID) from Bruton's agammaglobulinemia
|
CVID has decreased IgM, IgG, IgA, and IgE, but normal numbers of circulating B cells (whereas circulating B cells are decreased in Bruton's) and presents with less severe symptoms at a later age (15-35yo instead of 6-9mo)
|
|
Bedwetting is normal until what age?
|
5yo
|
|
Mental retardation, hypoplastic maxilla, long philtrum, and microcephaly
|
Fetal alcohol syndrome
|
|
Elevated 17-hydroxyprogesterone
|
Congenital adrenal hyperplasia
|
|
What does the Guthrie test look for?
|
PKU (presence of metabolic products of phenylalanine in the urine)
|
|
Protein content of human milk vs. formula and advantage it conveys
|
Human milk is 70% whey and 30% casein, and whey is more easily digested and helps improve gastric emptying
|
|
Human milk is associated with ____ reflux and colic than formula
|
Less
|
|
Most common complication of untreated ureteral reflux in children
|
Renal scarring --> ESRD/ HTN
|
|
Most common predisposing factor for acute bacterial sinusitis
|
Viral URI
|
|
Failure to pass meconium within first 24hrs often indicates
|
Hirschsprung's disease
|
|
Intramural air in the bowel indicates
|
Pneumatosis intestinalis, indicating necrotizing enterocolitis
|
|
Rx for NEC
|
Antibiotics
|
|
Rx for respiratory distress from foreign body aspiration
|
Direct laryngoscopy and rigid (NOT flexible) bronchoscopy
|
|
Should preemies should be given vaccinations based on their gestational or chronological age?
|
Chronological
|
|
How much do babies need to weight in order to receive the HBV vaccine?
|
2kg
|
|
Leukocoria, sensitivity to light, and excessive lacrimation
|
Congenital glaucoma
|
|
Rx for nursemaid's elbow (child keeps arm pronated, no swelling or deformity)
|
Rotating hand and forearm to supinated position w/ pressure over radial head (to reduce the annular ligament)
|
|
Most common elbow dislocation
|
Posterior
|
|
Tenderness of lateral epicondyle + pain w/ passive wrist flexion and resisted wrist extension
|
Tennis elbow
|
|
Baseball player w/ pain, crepitation, and loss of motion of arm
|
Osteochondrosis of capitellum (Panner disease)
|
|
Clay colored stools, dark urine, enlarged liver + conjugated hyperbili 1-6wks after birth
|
Biliary atresia
|
|
Rx for breastfeeding jaundice
|
Increase the frequency and duration of feedings to stimulate more milk production
|
|
Rx for bili > 17 and for bili > 25
|
Phototherapy
Exchange transfusion |
|
2wo with bilious vomiting, abdominal distension, and passage of bloodstained stools
|
Midgut volvulus
|
|
Non-bilious projectile vomiting in a 6wo first-born male
|
Pyloric stenosis
|
|
Top 3 causes of ear infections
|
Strep pneumo, non-typable H influenzae, and Moraxella catarrhalis
|
|
Viral causes of rubeola, rubella, and roseola
|
Measles, german measles, HHV6
|
|
Descending rash + fever + lymphadenopathy
|
Rubella
|
|
Risk of rapid drops in sodium level (e.g. from rehydrating with hypotonic solutions)
|
Cerebral edema
|
|
5 long-term neuro sequelae of bacterial meningitis
|
Hearing loss, loss of cognitive functions (e.g. regression of milestones in kids), seizures, mental retardation, spasticity or paresis
|
|
What is alexia
|
Acquired reading disorder subsequent to brain injury in previously literate pt
|
|
Abx choices for septic arthritis
|
<5: nafcillin (or vanc) + 3rd gen cef
>5: nafcillin (or vanc) alone |
|
Gait disturbances, pes cavus, ataxia, absent ankle jerks
|
Friedreich's ataxia
|
|
Proximal and distal hypotonia since birth, normal social and language skills, and tongue fibrillations
|
Infantile spinal muscular atrophy (SMA)
|
|
Another name for infantile SMA
|
Werdnig-Hoffman's syndrome
|
|
Triple bubble on abdominal XR
|
Jejunal atresia
|
|
Knee pain from repetitive injury sports in adolescent males w/ some edema and tenderness over tibial tubercle
|
Osgood-Schlatters Disease
|
|
Another name for and Rx for Osgood-Schlatters Disease
|
Traction apophysitis
Activity restriction, stretching, NSAIDs |
|
How to distinguish patellar tendonitis from traction apophysitis
|
Pts w/ patellar tendonitis have point tenderness at inferior pole of patella
|
|
Knee XR with irregularity/ fracture of the tibial tubercle
|
Traction apophysitis
|
|
Feared complication fo meningococcemia
|
Waterhouse-Friderichsen syndrome (sudden vasomotor collapse and purpura on flanks due to adrenal hemorrhage)
|
|
CF is due to what category of mutation
|
Deletion
|
|
Opsonization defect is most commonly due to ___ and leads to infections with ___ organisms
|
Asplenia
Encapsulated (e.g. S pneumo) |
|
Delayed growth spurt, delayed puberty, and delayed bone age on XR
|
Constitutional growth delay (will eventually reach a normal height)
|
|
Normal birth weight and height, but then growth velocity slows, and child following growth curve at around 5th percentile for multiple years
|
Constitutional growth delay
|
|
Rx for gonococcal conjunctivitis (2nd-5th day of life)
|
Ceftriaxone
|
|
Rx for chlamydial conjunctivitis
|
Oral erythromycin or reduce risk of chlamydial PNA
|
|
Most common cause of polycythemia in newborns
|
Delayed clamping of the umbilical cord --> excess transfer of placental blood
|
|
Most common complications of polycythemia in newborns
|
Respiratory distress, poor feeding, neuro symptoms (lethargy, irritability, seizures)
|
|
Dx and Rx for transposition
|
Echo
Prostaglandin E1 to stabilize (maintains PDA), then surgery |
|
What type of virus is rubella?
|
Toga virus
|
|
Congenital infections: cardiac anomalies (PDA and ASD), "blueberry muffin spots" (purpura), jaundice, hepatosplenomegaly
|
Rubella (+ hearing loss and cataracts)
|
|
Women are at risk of transmitting rubella during which part of pregnancy
|
First four weeks if primary infection
|
|
Pain in the hip that gradually progresses in a child with sickle cell anemia
|
Avascular necrosis of the femoral head
|
|
By 1yo, how much should a child have grown compared to birth weight?
|
Triple birth weight
Increase height by 50% |
|
How to distinguish ADHD from hearing impairment?
|
Hearing impairment also has poor language development and social isolation
|
|
Pt normal at birth, then develops muscle weakness and global wasting
|
Myotonic muscular dystrophy
|
|
Definition of myotonia
|
Delayed muscle relaxation (e.g. inability to release hand after handshake)
|
|
Another name for and inheritance of myotonic muscular dystophy
|
Steinert disease
AD |
|
Other systems involved in myotonic dystophy
|
Endocrine (DM, testicular atorphy, frontal baldness, hypothyroidism)
Immune Neuro |
|
Dx for suspected severe GERD
|
Modified barium swallow with fluorscopy (allows visualization of swallow reflux)
|
|
Dx for suspected PUD
|
Endoscopy (allows for biopsy and culture)
|
|
What is Sandifer syndrome?
|
Condition in which infants arch and become tonic when feeding to protect their airway from refluxing gastric contents
|
|
Dx for suspected Sandifer syndrome
|
Esophageal pH probe
|
|
Rx for eosinophilic esophagitis
|
Corticosteroids and trigger avoidance
|
|
XR with dilated loops of bowel and stomach + "curly Q twist of barium
|
Malrotation
|
|
Rx for malro/volvulus
|
Surgery
|
|
Echogenic bowel on prenatal ultrasound, then dilated loops of bowel w/ bubbly or granular appearance on XR at birth
|
Meconium ileus
|
|
Meconium ileus is pathognomonic for
|
CF
|
|
ASCA is often positive in ___ while p-ANCA is often positive in ____
|
Crohn's
UC |
|
Most serious complication of ulcerative colitis
|
Toxic megacolon
|
|
Rx for Hirschsprung's disease
|
Surgery
|
|
When are reflux meds indicated
|
Other medical problems (reflux-induced apnea or bradycardia, aspiration PNA, poor weight gain)
|
|
Diagnosis of Meckel's diverticulum
|
Radionucleotide scan (technetium)
|
|
Rx of Meckel's
|
Surgical excision
|
|
Dx of lactose intolerance
|
Hydrogen excretion in breath after oral admin of lactose (or acidic stool pH in presence of reducing substances)
|
|
Causes of direct hyperbili (>20) in infants
|
CF, alpha-1 antitrypsin deficiency, galactosemia, tyrosinemia, infection, choledochal cyst
|
|
Moribund appearance indicates dehydration to what extent
|
>10%
|
|
First teeth to erupt
|
Mandibular central incisors (last are second molars)
|
|
Infantile tooth decay, maxillary worse than mandibular
|
From bottles
|
|
Rx for completely avulsed secondary teeth
|
Dental eval w/in 30 min, ensure not a partial avulsion, transport teeth in milk, saline, or someone's mouth
|
|
Caues and largest risk factor for indirect inguinal hernia
|
Patent processus vaginalis
Prematurity |
|
Cause of direct inguinal hernia
|
Weak musculature in inguinal canal
|
|
Rx for indirect inguinal hernia
|
Surgery b/c incarceration is common
|
|
How to distinguish swallowed maternal blood from fetal hemorrhage
|
Apt test (fetal Hb unchanged in response to alkali, adult Hb changes to hematin)
|
|
Rx for NEC
|
Abx, surgery only if perforated
|
|
Dx of EHEC (from uncooked beef)
|
Stool Cx on sorbitol-MacKonkey agar or assay for shiga toxin
|
|
Vitamin deficiency: diffuse tenderness (esp on legs), poor wound healing, evidence of hemorrhage, irritability, swelling, tachypnea, poor appetite
|
Vitamin C
|
|
Vita deficiency: dry skin, poor growth, impaired cognition
|
Vita A (+ night blindness)
|
|
Ingestions: N/V/D, abd pain, GI bleeding, metabolic acidosis, small opacities on XR
|
Iron poisoning
|
|
Ingestions: lethargy, fever, hyperpnea, vomiting, tinnitus, metabolic acidosis, unremarkable XR
|
Aspirin poisoning
|
|
Ingestions: anorexia, bulging fontanelle (pseudotumor cerebri), hyperirritability, vomiting
|
Vita A
|
|
Ingestions: anorexia, N/V/D, headache, polyuria and polydipsia
|
Vita D
|
|
Infant with equinus and varus of the calcaneum and talus, varus of midfoot, and adduction of forefoot
|
Clubfoot (talipes equinovarus)
|
|
Rx for clubfoot
|
Stretching and manipulation, followed by serial plastar casts
|
|
Rx for RSV
|
Aerosolized ribavirin
|
|
Most serious complications of Kawasaki disease
|
Giant aneurysm formation, thrombosis, or rupture of the coronary arteries --> MI (so need to perform an echo)
|
|
5 major Jones Criteria
|
Polyarthritis
Carditis Chorea SubQ nodules Erythema marginatum |
|
3 minor Jones criteria
|
Fever
Arthralgia Previous rheumatic fever |
|
Rx for rheumatic fever
|
Benzathine penicillin G (+ AEDs, salicylates, and codeine for chorea, pericarditis, and arthritis, respectively)
|
|
Embryologic precursor of neuroblastoma
|
Neural crest cells
|
|
Embryologic precursor of Wilms' tumor
|
Metanephros
|
|
Cause of thrombocytopenia in Wiskott-Aldrich syndrome
|
Impaired production
|
|
Cause of thrombocytopenia in ITP
|
Production of Abs against platelets
|
|
Cause of thrombocytopenia in HUS
|
Platelet activation and consumption (also seen in DIC and TTP)
|
|
Storage disease: adolescent w/ chronic fatigue (anemia), easy bruisability (thrombocytopenia), bone pain, pathologic fractures (Erlenmyer flask deformity of distal femur) and wrinkled paper cells in bone marrow
|
Gaucher's disease (deficiency of acid beta-glucosidase)
|
|
Storage disease: infant w/ FTT, hepatospenomegaly, neurodegeneration
|
Niemann-Pick disease (deficient sphingomyelinase)
|
|
Storage disease: infant w/ hepatospenomegaly and skeletal abnormalities
|
GM1 gangliosidosis (deficient beta-galactosidase)
|
|
Storage disease: child with cherry red macula, neuro symptoms, hepatosplenomegaly
|
GM2 gangliosidoses, incl. Tay-Sachs' and Sandhoff's (deficiency of hexosaminidase)
|
|
Storage disease: bone pain and joint swelling
|
Farber disease (deficiency of ceramidase --> accumulation of ceramide esp in joints)
|
|
Formula mixing for powder and concentrate
|
Formula: 2 scoops to 4oz water
Concentrate: 1 part concentrate to 1 part water |
|
Caloric requirement from birth to 2mo per day and for pre-emies
|
100-200cal/kg/day
More (up to 150) for small pre-emies |
|
Pharyngitis, fever, dysphagia, neck stiffness, muffled voice
|
Retropharyngeal abscess (look for increased space btwn trachea and vertebrae on XR)
|
|
Pts with Turner Syndrome are at increased risk for
|
Osteoporosis
|
|
Rx for nephrotic syndrome in kids
|
Prednisone: 60% is minimal change disease, which is very steroid responsive (add cyclophosphamide if needed)
|
|
Differentiate bacterial and viral lymphadenitis
|
Bacterial is usually unilateral (+ tender, warm, and erythematous); S. aureus is most common; viral may have more systemic symptoms (bilateral, fever, pharyngitis, hepatosplenomegaly)
|
|
Non-TB mycobacteria lymphadenopathy
|
Firm, nontender, small lymphadenopathy
Skin becomes thin and violaceous |
|
What is the only congenital heart disorder that does not develop endocarditis
|
ASD
|
|
Side effect of hydroxyurea
|
Bone marrow suppression --> leukopenia, anemia, thrombocytopenia
|
|
Kids with bronchiolitis (RSV infection) are at increased risk for what later in life?
|
Asthma
|
|
Incidence of cyclic vomiting is high in children whose parents have a history of
|
Migraine headaches
|
|
What is the reticulocyte count in spherocytosis?
|
High (up to 9%)
|
|
Adverse reactions to the DTaP vaccine are usually attributed to which component?
|
Pertussis
|
|
Recurrent infections with staph and aspergillus
|
Chronic granulomatous disease (impaired oxidative metabolism, predisposing to abscesses from catalase-producing organisms)
|
|
Most common congenital obstructive urethral lesion in males (--> oliguria in newborns)
|
Posterior urethral valves (can lead to a distended bladder, palpable as abdominal mass)
|
|
Diagnostic test of choice for suspected posterior urethral valves
|
VCUG
|
|
Worrisome xray finding in NEC
|
Pneumoperitoneum (indicating intestinal perforation)
|
|
Neonate with hypocalcemia seizures
|
Think about DiGeorge syndrome
|
|
Neonate with vomiting, bloody diarhea, stool with RBCs and eosinophils
|
Milk protein intolerance (may have a FH of atopy)
|
|
Most common cause of congenital hypothyroidim
|
Thyroid dysgenesis
|
|
Most common complication of supracondylar fracture
|
Entrapment of brachial artery (so check the radial pulse)
|
|
Newborn with evanescent rash with red halos and eosinophils in the skin lesions
|
Erythema toxicum
|
|
Strongest risk factor for intraventricular hemorrhage
|
Prematurity/ low birth weight (also exposure to vascular perfusion injuries)
|
|
Rx for severe asthma exacerbation that is unresponsive to meds
|
Mechanical ventilation (intubation)
|
|
How to distinguish preseptal and orbital cellulitis
|
Eyelid symptoms (e.g. discoloration) indicate preseptal
Decreased eye movements or visual acuity, double vision, or proptosis indicate orbital cellulitis (as well as systemic symptoms) |
|
Rx for preseptal vs. orbital cellulitis
|
Antibiotics for both, but orbital cellulitis requires hospitalization (and possibly surgical decompression)
|
|
Neonatal hypoglycemia, macroglossia, visceromegaly, omphalocele, earlobe crease
|
Beckwith-Widemann syndrome
|
|
Self-mutilation, mental retardation, hyperuricemia, dystonia, choreoathetosis, cerebral palsy
|
Lesch-Nyhan syndrome (deficiency in hypoxanthine-guanine phosphoribosyl transferase)
|
|
Diagnostic test for pyloric stenosis
|
Ultrasound of the abdomen
|
|
Penicillin and amoxicillin have no activity against this common skin flora bug
|
S. aureus
|
|
Rx for bacterial lymphadenitis
|
Dicloxacillin (covers both staph and strep, 2 most common agents)
|
|
Microcephaly and IUGR most likely indicate which class of problems
|
TORCH infection
|
|
Pathogenesis of Meckel's diverticulus
|
Failure of vitelline duct to obliterate during fetal development
|
|
Murmur of large VSDs is generally ____ compared to that of small VSDs
|
Soft
|
|
Newborn with hypoplastic nails, cleft palate, vita K deficiency
|
Fetal hydantoin syndrome (phentyoin use in pregnancy)
|
|
5 common GI complications of Down's
|
Duodenal atresia
Hirschsprung's Esophageal atresia Pyloric stenosis Malro |
|
Type of anemia seen in sickle cell anemia
|
Hemolytic anemia (hemolysis of sickled cells --> reticulocytosis, hyperbili, elevated LDH with low haptoglobin)
|
|
Rx for SCFE
|
Surgical fixation at current degree of slippage to avoid risk of avascular necrosis
|
|
Is an LP necessary with a febrile seizure?
|
No, unless signs of meningitis present, child already on Abx, or not immunized
|
|
How to distinguish a microcytic anemia from iron deficiency vs. thalassemia?
|
RDW: high (>20%) indicates iron deficiency, normal in thalassemia or anemia of chronic disease
|
|
Peripheral blood smear findings in sickle cell
|
Sickled cells and reticulocytosis
|
|
Nocturnal vaginal itching: presumed diagnosis, diagnostic test, and treatment
|
Vulvovaginitis from pinworm infection
Scotch tape test Mebendazole |
|
Most common causes of acute bacterial sinusitis
|
Same as ear infections:
Strep pneumo Non-typable H flu Moraxella |
|
Most common cause of viral meningitis
|
Enteroviruses (PERCH, though not polio: usually echo or coxsackie)
|
|
Physical exam manifestation of endocardial cushion defect
|
Endocardial cushion defect --> pulmonary HTN --> Loud P2 (Rx is early surgery)
|
|
Inspiratory noises w/o other complaints; "epiglottis rolling in from side to side" on direct laryngoscopy
|
Laryngomalacia (aka congenital flaccid larynx)
|
|
Rx for laryngomalacia
|
Reflux precautions
Should resolve by 2yo |
|
Organ with highest M&M after HUS
|
Kidneys
|
|
Why is their jaundice in HUS?
|
Hemolysis (NOT b/c of liver involvement)
|
|
Down's patient w/ torticollis, urinary incontinence, dizzines, UMN signs (leg spasticity, hyperreflexia, positive Babinski)
|
Alantoaxial instability (due to excessive laxity in posterior transverse ligament, causing increased mobility btwn atlas and axis)
|
|
Rx for atlantoaxial instability
|
Surgical fusion of C1 to C2
|
|
How long must disruptive behavior last for conduct disorder diagnosis
|
1yr
|
|
Differentiate btwn conduct disorder and antisocial personality disorder
|
<18yo for conduct, >18 for antisocial
|
|
Differentiate btwn conduct disorder and oppositional defiant disorder
|
Conduct: older teens, violation of social norms
ODD: late childhood, negativity, hostility, defiance |
|
Definition of infantile colic
|
Starts before 3wks, crying >3hrs/day, >3 days/wk, >3wks/mo
|
|
Rx for infantile colic
|
Nothing universal: soothing, simethicone, probiotics
Usually resolves by 4mo |
|
Drug that opens PDA
|
Prostaglandin E1
|
|
Drug that closes PDA
|
Indomethacin
|
|
Rx for labial adhesions (benign fusion of labia minora)
|
Nightly application of estrogen cream + daily petrolatam for 1mo
|
|
Lax, wrinkled abdominal wall, dilated urinary tract, intra-abdominal testes, renal dysfunction
|
Prune belly syndrome
|
|
Prune belly syndrome is associated w/ these extra-abdominal effects
|
Oligohydramnios, clubfeet, hip dislocation, malro
|
|
Anorexia, V, polydipsia, polyuria, fevers, glucosuria w/ normal serum glucose, high urine pH, hyperchloremic metabolic acidosis, albuminuria w/ normal serum protein and albumin
|
Fanconi Syndrome
|
|
How to distinguish Type 1 from Type 2 RTA
|
Type 2 has a normal urine anior gap, where Type 1 (and type 4) have positive urine anion gaps
|
|
What is phimosis
|
Inability to retract the foreskin
|
|
When is phimosis normal?
|
Before 3yo
|
|
What is acute lobar nephronia
|
In between pyelonephritis and renal abscess
|
|
Rx for nephronia
|
Prolonged IV and then PO antibiotics
|
|
Orchiopexy (moving an undescended testicle into the scrotum) reduces the risk of?
|
Testicular torsion (b/c decreases mobility)
Doesn't reduce the risk of testicular malignancy, though does make accessible for periodic exams |
|
What counts as the most sterile urine collection in a child?
|
Suprapubic tap
|
|
PSGN can occur after which two types of strep infections
|
Pharyngitis or impetigo
|
|
Microangiopathic hemolytic anemia, thrombocytopenia from increased platelet utilization, and renal insufficiency from vascular endothelial injury and local fibrin deposition + bloody diarrhea
|
HUS
|
|
Pain relief upon elevation of the testicle
|
Prehn sign: indicates epididymitis
|
|
What is HSP
|
Systemic IgA-mediated vasculitis
|
|
Bag of worms
|
Varicocele
|
|
Rx for varicocele?
|
Reassurance
May require surgery for infertility later on |
|
Fluid-filled area surrounding testicle that transilluminates
|
Hydrocele
|
|
Rx for hydrocele
|
Observe, usually resolves by 1yo; larger ones that persist may need surgery
|
|
Genetics of Alport syndrome
|
X-linked dominant
|
|
Recurrent gross hematuria + dysuria/abdominal pain
|
Idiopathic hypercalciuria (stones may form over time)
|
|
Rx for acute glomerulonephritis with oliguria
|
Restrict fluid intake to avoid hypervolemia
|
|
Elevated cholesterol and triglycerides + edema
|
Nephrotic syndrome
|
|
Is HTN more likely associate with glomerulonephritis or nephrotic syndrome
|
Glomerulonephritis, apparently
|
|
FTT with constipation, weakness, vomiting, polyuria/polydipsia, dehydration + hypokalemia, hypercalciuria, alkalosis
|
Bartter Syndrome (juxtaglomerular hyperplasia): also have hyperaldosteronism and hyperreninemia
|
|
Is mumps usually unilateral or bilateral?
|
Unilateral and spreads to the opposite side
|
|
Viral infection + sleepiness, emesis, abnormal liver fxn, seizures, coma, respiratory distress, loss of DTRs, posturing
|
Reye's syndrome
|
|
Reye's is a ____ caused by ___ and ____
|
Mitochondrial hepatopathy
Influenza or varicella infection + aspirin use |
|
Cause of death in Reye's
|
Cerebral edema and subsequent herniation
|
|
What is Ekiri syndrome?
|
Rare and fatal toxic encephalopathy seen with Shigella infection
|
|
Rx for shigellosis
|
Supportive care and third generation cephalosporin
|
|
Major cause of tinea capitis
|
Tricophyton tonsurans
|
|
Rx for trichophyton tonsurans
|
Oral griseofulvin
|
|
Linear hair loss + regional adenopathy
|
Traction alopecia
|
|
Hairs near area of hair loss that can be extracted w/ gentle traction --> attenuated or catagen bulb at termination of hair shaft (exclamation hair)
|
Alopecia areata
|
|
Is mumps usually unilateral or bilateral?
|
Unilateral and spreads to the opposite side
|
|
Viral infection + sleepiness, emesis, abnormal liver fxn, seizures, coma, respiratory distress, loss of DTRs, posturing
|
Reye's syndrome
|
|
Reye's is a ____ caused by ___ and ____
|
Mitochondrial hepatopathy
Influenza or varicella infection + aspirin use |
|
Cause of death in Reye's
|
Cerebral edema and subsequent herniation
|
|
What is Ekiri syndrome?
|
Rare and fatal toxic encephalopathy seen with Shigella infection
|
|
Rx for shigellosis
|
Supportive care and third generation cephalosporin
|
|
Major cause of tinea capitis
|
Tricophyton tonsurans
|
|
Rx for trichophyton tonsurans
|
Oral griseofulvin
|
|
Linear hair loss + regional adenopathy
|
Traction alopecia
|
|
Hairs near area of hair loss that can be extracted w/ gentle traction --> attenuated or catagen bulb at termination of hair shaft (exclamation hair)
|
Alopecia areata
|
|
Clinical course of leptospirosis (zoonosis)
|
Septicemic phase; then most are anicteric and go on to immune phase w/ meningitic symptoms
10% are icteric and can have liver and kidney dysfunction |
|
Rx for leptospirosis
|
Penicillin and doxycycline
|
|
Typical age of Kawasaki disease
|
<5yo
|
|
Rx for newborn baby with pertussis
|
Erythromycin (mom's Ig is insufficient)
|
|
Diffuse adenopathy, tonsillar enlargement, enlarged spleen, small hemorrhages on soft palate, periorbital swelling
|
EBV
|
|
Diff in EBV
|
Lymphocyte predominance w/ at least 10% atypicals
|
|
Rx for mono
|
Avoid contacts sports b/c of enlarged spleen
|
|
When is it not Kawasaki disease?
|
Teenage pt
|
|
What is dacrocystitis?
|
Infection of nasolacrimal sac
|
|
Most common cause of dacrocystitis
|
Nasolacrimal duct obstruction
|
|
Rx for nasolacrimal duct obstruction
|
Massage and clean w/ warm washcloths; optho referral is not open by 6mo
|
|
What is a hyphema
|
Blood in anterior chamber of eye
|
|
Virus causing erythema infectiosum
|
Parvovirus B19 (Fifth disease)
|
|
Other systems involved in mumps
|
Renal, pancreas, CNS
|
|
Common cause of death in Wiskott-Aldrich
|
Malignancy 2/2 EBV infection in teenage years
|
|
Rx for Lyme disease
|
Penicillin or tetracycline
|
|
Family of ticks associated w/ Lyme vs. RMSF
|
Ixodes vs. Dermacantor
|
|
What type of bug is Borrelia?
|
Spirochete
|
|
Pinworm bug
|
Enterobius vernicularis
|
|
Persistent, nonsuppurative diarrhea
|
Crypto or Giardia
|
|
Abdominal pain, N/V; then eyelid edema, myalgia, fever, and eosinophilia
|
Trichinella spiralis infection from undercooked pork
|
|
Close contacts of meningococcemia pt need
|
Rifampin prophylaxis
|
|
Contraindications to LP
|
Increased ICP WITHOUT open fontanelle; severe thrombocytopenia/ coagulation disorder; CVR distress; skin infection at puncture site
|
|
Hypergammaglobulin E, eczema-like rash, and recurrent severe staph infections
|
Job-Buckley syndrome (disorder of phagocytic chemotaxis)
|
|
Rash after administration of amoxicillin
|
EBV (unknown pathophys, but pathognomonic)
|
|
Culture medium for onorrhea
|
Thayer-Martin media
|
|
Lab test suggestive of chlamydia pneumonia
|
Increased eosinophils in blood
|
|
<1yo w/ URI --> severe respiratory distress, pneumatocele, and pneumothorax
|
Staph pneumonia
|
|
Rx-resistant PNA that develops empyema and needs chest tubes placed
|
Pneumococcal PNA
|
|
4 contraindications for contact sports
|
One eye, hemophilia, single kidney, unexplained syncope
|
|
Radionuclide scan results in testicular torsion vs. epididymitis
|
Decreased uptake in torsion, increased uptake in epididymitis
|
|
Test for herpes
|
Tzanck prep scraping from ulcer w/ multinucleated giant cells and intranuclear inclusions
|
|
Unilateral draining lyphadenitis
|
Lymphogranuloma venereum (caused by subtype of C. trachomatis)
|
|
How to distinguish suppurative adenopathy of chancroid from that of LGV?
|
In chancroid is concurrent; in LGV is after ulcer has healed
|
|
Culture medium for Haemophilus ducreyi (chancroid)
|
Chocolate agar
|
|
Most common injuries in swimmers
|
Shoulder injuries (rotator cuff tendonitis)
|
|
Most common injuries in basketball players
|
Knees (and ankles), esp patellar tendonitis and Osgood-Schlatter
|
|
Common skin conditions in wrestlers
|
Contact dermatitis, fungal infection, HSV, impetigo, staph furunculosis or staph folliculitis
|
|
Most common ski injury
|
Skier's thumb (hyperextension and abduction of thumb)
|
|
Jaundice, hepatomegaly, vomiting, seizures on intro of fruit juice
|
Hereditary fructose intolerance
|
|
Children w/ tyrosinemia have problems w/ these 3 organs, one of which is not improved w/ dietary management
|
Liver (progressive cirrhosis not preventable), kidney, CNS
|
|
Storage disease seen in teens + enzyme deficeincy
|
Gaucher disease, Beta-glucocerebrosidase
|
|
Erlenmeyer flask appearance of bone + anemia, leukopenia, and thrombocytopenia
|
Gaucher disease
|
|
Cause of neonatal thyrotoxicosis
|
Maternally acquired thyrotropin receptor stimulating antibody (which crosses the placenta, unlike TSH)
|
|
How to distinguish btwn ovarian tumor/ exogenous estrogen and precocious puberty
|
Former suppresses pituitary, so no secondary sex characteristics, just isolated premature thelarche or vaginal bleeding w/o pubic hair
|
|
White forelock, heterochromia irides, congenital deafness
|
Waardenburg syndrome (AD)
|
|
Most common cause of hypothyroidism (thyromegaly) in kids
|
Lymphocytic (Hashimoto's) thyroiditis
|
|
2 common causes of Cushing's
|
Glucocorticoid administration and bilateral adrenal hyperplasia
|
|
Short, downslanting palpebral fissues, ptosis, low set and malformed ears, broad and webbed neck, shield chest, cryptorchidism, pulmonary stenosis, hearing loss, bleeding diathesis, mental retardation in 25%
|
Noonan Syndrome
|
|
ITP is usually preceded by a?
|
Viral infection
|
|
RX for severe ITP
|
IVIG, steroids, anti-D Ig, splenectomy if chronic
|
|
Levels of free erythrocyte proporphyrin are elevated in these 2 conditions
|
Iron deficiency and lead poisoning
|
|
Most common acquired RBC aplasia in kids
|
Transient erythroblastopenia of childhood
|
|
Congenital anemia with increased RBC ADA levels, short stature, and cranial/ upper extremity abnormalities
|
Diamond-Blackfan anemia
|
|
Increased levels of HbA2 is seen in
|
Beta-thal train
|
|
What is the Kasabach-Merritt phenomenon?
|
Large vascular anomalies (kaposiform hemangioendothelioma and tufted angioma) sequest platelets and RBCs --> thrombocytopenia, coagulopathy, and microangiopathic hemolytic anemia
|
|
Rx for Kasabach-Merritt phenomenon
|
Steroids, alpha-INF, and vincristine (no surgery! --> excessive bleeding
|
|
Another name for port wine stain
|
Nevus flammeus
|
|
Cells seen in G6PD deficiency
|
Bite cells (RBCs after removal of Heinz bodies)
|
|
Retinoblastoma is most commonly (spontaneous vs. hereditary) and (unilateral vs. bilateral)
|
Spontaneous and unilateral
|
|
Cure rate of retinoblastoma
|
90%, though vision loss is common
|
|
Pts w/ hereditary retinoblastoma are at increased risk of
|
Osteosarcoma
|
|
When is physiologic anemia of infancy seen?
|
Nadir 6-8wks, as erythropoiesis decreases
|
|
Anemia in newborn w/ normal retic count and no ABO incompatibility
|
Fetomaternal transfusion
|
|
How to test for fetomaternal transfusion
|
Keihauer-Betke stain for fetal Hb-containing RBCs in mom's blood
|
|
Most common childhood malignancy
|
Leukemia
|
|
Horseshoe kidney and hemihypertrophy are associated w/
|
Wilms tumor
|
|
Wilms associated w/ abnormalities on chromosome
|
11
|
|
Abdominal mass + fever, irritability, bone pain, limp, and diarrhea
|
Neuroblastoma
|
|
Causes of peripheral eosinophilia
|
Asthma, urticaria, infantile eczema, drug reactions, helminth infections, collagen vascular disease
|
|
Allergic rhinitis causes eosinophilia where?
|
Nasal secretions, not peripheral blood
|
|
5 lab findings in DIC
|
Prolonged PT and PTT, decreased fibrinogen, fibrin split products, increased D-dimer
|
|
3 causes of spherocytes other than hereditary spherocytosis
|
Hyperthermia, G6PD deficiency, ABO incompatibility
|
|
Rx for von Willebrand's disease
|
DDAVP (can also give vWF + Factor VIII if severe)
|
|
Sunburst pattern of bone formation
|
Osteosarcoma
|
|
Diffuse LAD, fever, night sweats, malaise, weight loss, pruritis
|
Hodgkin's
|
|
Dx of suspected Hodgkin's
|
CXR to look for mediastinal mass, then LN biopsy
|
|
What are Howell-Jolly bodies and when are they seen?
|
Small spherical nuclear remnants seen in reticulocytes of pts w/ asplenia or hyposplenia (e.g. in SCA)
|
|
Severe anemia requiring transfusions, heart failure, hepatosplenomegaly, weakness
|
Thalassemia major
|
|
Most common finding on peripheral smear in thalassemia
|
Target cells
|
|
What are target cells?
|
Erythrocyte with membrane too large for its Hb content, seen in alpha-thal, HbC, and liver diseaes
|
|
Pregnancy complications more common in teens
|
Premature delivery, low birth weight, pregnancy-induced HTN, violence risk
|
|
Male Tanner Stages
|
SMR 2: sparse, thin, long pubic hair
SMR 3: darker, curlier pubic hair SMR 4: adult pubic hair w/o full coverage SMR 5: pubic hair to inner thigh (penis lengthening throughout) |
|
Female Tanner Stages
|
Sparse pubic hair in SMR 2 --> medial thigh by SMR5
SMR 2: breast buds SMR 3: larger breasts and areola w/o separate areolar contour SMR 4: elevation of areola SMR 5: areola part of general breast contour |
|
Rx for suicide attempt w/ nml exam and labs
|
Hospitalize
|
|
Swollen, red, irritated uvula and dental decay
|
Bulimia
|
|
Sport concussion recommendations
|
LOC --> evaluate at hospital
No LOC, nml w/in 15 min --> return to game Confusion > 15 min --> out for 1wk Confusion > 1hr --> hospital |
|
Side effects of isoretinoin
|
Dry skin/eyes/mucous membranes, MSK pain, IBD, pseudotumor cerebri, corneal opacities, abnormal LFTs, increased TGs and cholesterol
|
|
Dehydration, facial and arm hirsutism w/ loss of head hair, bradycardia, amenorrhea
|
Anorexia
|
|
Joint swelling in pt with hemophilia
|
Hemarthroses (hemophilic arthropathy), w/ iron deposition and synovial thickening + fibrosis
|
|
Next option when IV access can't be obtained
|
Intraosseous access (easier than in adults b/c have less bone density)
|
|
Why do we avoid subclavian lines in kids
|
High incidence of complications (pneumothorax, hemothorax)
|
|
Differential for T-wave inversion
|
MI, myocarditis, old pericarditis, myocardial contusion, dig toxicity
|
|
Wide-based gait, decreased vibratory/position sense in legs, absent lower DTRs + inheritance pattern
|
Friedrich's ataxia (AR)
|
|
Most common non=CNS complication of Friedrich's ataxia
|
Necrosis and degeneration of cardiac muscle fibers --> myocarditis, myocardial fibrosis, and cardiomyopathy (--> arrhythmias and CHF)
|
|
Unilateral gynecomastia in a 14yo
|
Pubertal gynecomastia: reassure
|
|
Rx for platelets <30,000
|
Corticosteroids (platelet transfusion only if actively bleeding, as can often cause further destruction of platelets; splenectomy if life-threatening bleed)
|
|
Why does a hyperextended neck provide relief in epiglottitis?
|
Maximizes the airway diameter
|
|
Signs of chronic upper airway obstruction since birth, worse while supine, relieved with neck extension
|
Vascular ring (rx: surgery)
|
|
Cyanosis aggravated by feeding and improved with crying
|
Choanal atresia
|
|
Inspiratory stridor exacerbated by any exertion; symptoms appear w/in first 2 weeks of life with progressive increase in severity; no cyanosis
|
Laryngomalacia
|
|
Precocious puberty + signs of severe androgen excess (e.g. severe acne, rapid growth)
|
Precocious pseudo-puberty, a gonadotropin-independent process, often caused by late-onset CAH
|
|
Heart disease associated w/ neonatal lupus
|
Congenital heart block
|
|
Heart disease associated w/ congenital rubella
|
PDA
|
|
Heart disease associated w/ Edward's syndrome
|
VSD
|
|
Heart disease associated w/ Williams' Syndrome
|
Supravalvular aortic stenosis
|
|
Breathholding spells may be associated w/ this abnormality
|
Anemia
|
|
Craniotabes (ping-pong ball sensation over occiput), rachitic rosary (enlargement of costochondral junctions) and thickening of wrists and ankles
|
Vitamin D deficiency rickets
|
|
Lab findings in Vita D deficiency rickets
|
Serum calcifediol level is decreased, alk phos is increased
|
|
XR findings in rickets
|
Cupping and fraying of distal ends of long bones; double contour along lateral outline of radius
|
|
General tenderness, pseudoparalysis (frog position), bluish-purple and spongy/swollen gums over incisors, and depression of sternum
|
Vita C deficiency
|
|
XR findings in scurvy
|
Pencil-point thinness of long bone cortex and sharply outlined epiphyseal ends
|
|
How to differentiate autism and Asperger's
|
Asperger kids are more socially aware and communicative; have normal language development
|
|
Baby born at home with poor suckling and fatigue followed by rigidity, spasms, and opisthotonus + red and inflamed umbilical stump
|
Neonatal tetanus
|
|
Main causes of death in neonatal tetanus
|
Apnea or septicemia
|
|
Prevention of neonatal tetanus
|
Maternal immunization
|
|
Tests used for neonatal HIV diagnosis
|
PCR, viral culture, p24 antigen testing (can't test antibodies b/c maternal Abs cross the placenta)
|
|
Average survival with neonatal HIV
|
AIDS by 1yo, death by 3yo (progresses more rapidly)
|
|
Empiric Abx for septic arthritis
|
IV nafcillin or IV cefazolin
|
|
Mechanism for improvement of tet spells w/ squatting
|
Increased systemic vascular resistance (--> increased blood flow ot pulmonary circulation)
|
|
In child w/ epidural hematoma, features that necessitate emergent craniotomy
|
GCS < 8, increased ICP, pupillary abnormalities, hemiparesis, or cerebellar signs
|
|
Rx for pts with a small epidural hematoma and no focal neuro signs
|
F/u CT in 24 hrs
|
|
Kids with HSP are at risk for this GI problem
|
Intussusception
|
|
Holosystolic murmur over LLSB and rumbling diastolic murmur over apex
|
VSD
|
|
Rx for vaginal foreign body
|
Irrigation with warmed fluid first; if unsuccessful, exam and removal with sedation or under general anesthesia
|
|
Infants who are SGA are at risk for these 7 complications
|
Hypoxia, perinatal asphyxia, meconium aspiration, polycythemia, hypothermia, hypoglycemia, hypocalcemia
|
|
Paralysis of the left hand and ipsilateral Horner syndrome (miosis and ptosis) after delivery
|
Klumpke paralysis (brachial plexus injury to C7, C8, and T1)
|
|
Absent Moro reflex and intact grasp reflex of affected arm; adduction and internal rotation/ pronation of arm
|
Erb-Duchenne palsy (C5 and C6)
|
|
Craniofacial anomalies, fingernail hypoplasia, growth deficiency, dev't delay, cardiac defects, facial clefts
|
Phenytoin use during pregnancy
|
|
Structure affected in Guillain-Barre
|
Peripheral nerves
|
|
What test should always be done in patients w/ apparent subQ emphysema 2/2 severe coughing paroxysms
|
CXR to r/o pneumothorax
|
|
Rx for chronic granulomatous disease?
|
Daily TMP-SMX and gamma-INF 3x/wk
|
|
Coagulopathy, neuropathy, hepatosplenomegaly, pancytopenia, partial oculocutaneous albinism, frequent bacterial infections (S. aureus) and progressive lymphoproliferative syndrome
|
Chediak-Higashi Syndrome
|
|
Findings diagnostic of Chediak-Higashi syndrome
|
Neutropenia and giant lysosomes
|
|
Chronic pruritic dermatitis, recurrent staph infections, eosinophilia, coarse facial features
|
Hyper IgE (Job's) syndrome
|
|
What must be done prior to surgery for pyloric stenosis?
|
Stabilization w/ IVF and K+
|
|
2 abnormalities where corrective surgery should be delayed until school age
|
Umbilical hernia
ASD |
|
Neck swelling w/ rotation to one side, resisting rotation or flexion + diagnostic test
|
Torticollis
Neck XR to r/o cervical spine fracture or dislocation |
|
3 most common causes of acquired torticollis
|
URIs, minor trauma, cervical lymphadenitis
|
|
How to differentiate HSV meningitis from bacterial meningitis
|
HSV meningitis usually has focal neurologic signs, and fewer band forms on diff
|
|
Sail sign on CXR
|
Thymic shadow (appears very large in kids, but this is normal in kids <2yo)
|
|
Infant with FTT, bilateral cataracts, jaundice, and hypoglycemia
|
Galactosemia
|
|
Enzyme deficiency in galactosemia
|
Galactose-1-phosphate uridyl transferase deficiency
|
|
Pts with galactosemia are at increased risk for this infection
|
E. coli neonatal sepsis
|
|
Cataracts in neonatal period
|
Galactokinase deficiency
|
|
Pertussis prevention for close contacts
|
Erythromycin x 14 days (regardless of age, immunization, or symptoms)
|
|
Dx and Rx for acute bacterial sinusitis
|
Amoxicillin based on clinical, not radiographic findings
|
|
Dx for muscular dystrophy
|
Muscle biopsy
|
|
What is postpericardiotomy syndrome?
|
Reactive pericarditis w/ pericardial effusion that occurs after surgery for congenital heart disease
|
|
Dx test for proteinuria w/o hematuria
|
Repeat 2 more times (is most likely just transient proteinuria)
|
|
Rx for Bruton's agammaglobulinemia
|
IVIG infusion
|
|
Rx for suspected congenital diaphragmatic hernia
|
Orogastric tube w/ continuous suction to prevent bowel distension and further lung compression (followed by intubation)
|
|
Rx for exposure to varicella
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Vaccine if within 3-5 days of exposure; varicella IG if immunodeficient
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When is surgery indicated for umbilical hernias?
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If persist to 3-4yo, >2cm, symptomatic, strangulated, or progressively enlarges
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Rx for absence seizures
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Ethosuximide or valproic acid
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2 toxoid vaccines
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Tetanus and diphtheria
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2 live attenuated vaccines
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MMR and chicken pox
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Children with congenital heart disease are at increased risk of this CNS pathology
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Brain abscess
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5 risk factors for brain abscess dev't
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Congenital heart disease, head trauma, infection of jaw/mouth/face/scalp, meningitis, cranial instrumentation
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Trauma to soft palate + stroke symptoms
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Internal carotid artery dissection
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How to distinguish Niemann-Pick from Tay Sachs
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Hepatomegaly and cervical lymphadenopathy only in NP
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Pathophys of infantile botulism
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NOT ingestion of preformed toxin; organisms enter GI tract, where they produce the toxin (a protease that blocks ACh release)
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Dilation of entire ventricular system w/ distinct enlargement of subarachnoid space over cerebral cortex
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Communicating hydrocephalus 2/2 subarachnoid hemorrhage (e.g. from intraventricular hemorrhage)
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Imaging difference btwn Dandy-Walker anomaly and Chiari malformation
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Dandy-Walker: cystic expansion of fourth ventricle
Chiari: protrusion of structures of posterior fossa thru foramen magnum |
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Diagnostic test for pyloric stenosis
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Abdominal ultrasound
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Cause of edema in Turner Syndrome
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Abnormal development of lymphatic network
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Distinction btwn the arthritis in JRA and in rheumatic fever
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Migratory arthritis in rheumatic fever
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Scalp swelling limited to surface of one cranial bone
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Cephalohematoma (subperiosteal hemorrhage)
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Diffuse swelling that crosses suture lines
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Caput succedaneum
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Pain, pallor, poikilothermia, paresthesias, pulselessness, paralysis
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Compartment syndrome
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Increased gastric residual volume in a preterm neonate
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NEC
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Diarrhea, fever, and erythematous rash on abdomen (Rose spots)
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Salmonella poisoning
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Common organisms:
G+ diplococci G+ cocci in clusters G+ rods G- cocci G- rods |
Strep pneumo
Staph Listeria and Bacillus Neisseria Pseudomonas, Haemophilus, Klebsiella, Legionella |
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Neonate w/ irritability, high-pitched cry, poor sleeping, seizures, sweating, sneezing, tachypnea, V/D
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Neonatal abstinence syndrome (from opioid withdrawal)
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Drooling, abdominal distension, rattling breath sounds + atelectasis and gastric distension on XR
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Esophageal atresia
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Rx for epiglottitis
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Intubation w/ preparation for possible tracheostomy
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Slowly developing back pain, neuro dysfunction (e.g. incontinence), and palpable "step-off" at lumbosacral area
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High-grade spondyloisthesis
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Normal at birth, then apathy, weakness, hypotonia, large tongue, sluggish movement, abdominal bloating, umbilical hernia
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Congenital hypothyroidism
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Test for CF if can't colelct enough sweat during pilocarpine iontophoresis
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DNA testing for CFTR mutations
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Lims shorter than trunk and head disproportionately large
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Achondroplasia
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Short but with normal proportions
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GH deficiency
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Signs of congenital hypothyroidism
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Constipation, prolonged jaundice, sluggishness, poor feeding, apnea, choking, macroglossia, excessive sleepiness
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Newborn w/ macrosomia, macroglossia, abdominal wall defects, linear ear creases, organomegaly, hypoglycemia
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Beckwith-Wiedemann Syndrome
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Infants w/ Beckwith-Widemann Syndrome are at increased risk of
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Malignancies (esp wilms, hepatoblastoma, and gonadoblastoma)
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Born normal and then loss of milestones, umbilical hernia, kyphoscoliosis, deafness, cloudy corneas, claw hand deformity (disease + deficiency)
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Hurler syndrome (alpha-L-iduronidase eficiency --> deposition of dermatan and heparan sulfate)
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What is the bone age in kids with constitutional short stature?
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Few years behind chronological age (indicating still time for a growth spurt)
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Inheritance of achondroplasia
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AD
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Short w/ delayed bone age, mental retardation, increased bone density esp in skull, brachydactyly, obesity w/ round facies and short neck, subcapsular cataracts, cutaneous and subQ calcifications, perivascular calcifications of basal ganglia
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Pseudohypoparathyroidism (Albright hereditary osteodystrophy)
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Lab findings in pseudohypoparathyroidism
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High PTH w/ low Ca and high phosphorous (receptors are resistance to PTH)
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Constipation, anorexia, vomiting, polyuria, lethargy after long-term immobilization b/c of fracture
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Immobilization hypercalcemia
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Dx test for immobilization hypercalcemia
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Serum ionized calcium and urinary calcium to creatinine ratio
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Complicaitons of immobilization hypercalcemia (and resultant hypercalciuria)
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Nephropathy, nephrocalcinosis, hypertensive encephalopathy, convulsions
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Hyponatremia, hyperkalemia, hypoglycemia in first 15 days of life
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CAH
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Urine that turns black when exposed to air (disease + deficiency)
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Alkaptonuria (AR), deficiency of homogentisic acid oxidase
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Rx for alkaptonuria
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None needed; supplemental ascorbic acid may delay onset of arthritis in adults
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Cholestasis, elevated LFTs, bleeding into CNS, GI tract, or at umbilical stump
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alpha-1 antitrypsin deficiency
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Hypoglycemia in a preemie is due to
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Decreased glycogen and fat stores
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Lab values in nutritional rickets
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Normal serum calcium (decreased absorption, but released from bone and decreased excretion), low phosphate (due to phosphaturia), high PTH (stimulated by transient hypocalcemia, causing the other findings), elevated alk phos
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Lab findings in vita D resistant rickets + pathophys and inheritance
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Low phosphate, normal calcium (genetic abnormality in reabsorption of phosphate), X-linked
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Numbness, tingling, seizures
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Hypoparathyroidism (--> hypocalcemia and hyperphosphatemia)
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Obesity, mental retardation, hypogonadism, polydactylyl, retinitis pigmentosa w/ night blindness
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Laurence-Moon-Biedl syndrome (AR)
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Sodium and potassium values in pts with central/nephrogenic diabetes insipidus
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High Na (losing water), somewhat high K+
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Lab findings in hyperaldosteronism
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Hypernatremia, hypokalemia (enhancing sodium-potassium exchange), hyperchloremia, alkalosis
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Lab findings in pts with hyperlipidemia (e.g. von Gierke's)
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Pseudo hyponatremia and hypokalemia
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Most common congenital anomaly in Ehlers-Danlos
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MVP
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Abnormalities of upper extremities, hypoplastic radii, thumb abnormalities, cardiac anomalies, missing pec major (chest wall musculature)
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Holt-Oram syndrome
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