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208 Cards in this Set
- Front
- Back
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Combined Immunodeficiencies
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SCID
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T-cell Deficiency
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Di George sydnrome
Chronic mucocutaneous candidiasis |
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Humoral Deficiency
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Bruton's agammaglobulinemia
IgA deficiency CVID |
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Phagocytosis
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Chrnoic granulomatous disease
Chediak-Higashi syndrome Job syndrome |
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Other
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Wiskott-Aldrich
Ataxia-telangiectasia |
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acute disseminated form of Langherhan's cell histiocytosis
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Letterer-Siwe Disease
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what are the sxs
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skeleton involved, lymphadenoathy, HSM,
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s/s: progressive cerebellar ataxia (first 6yrs), humoral and cellular immunodeficiency, oculocutaenous telangeictasisas, chronic sinopulmonary infection, increased risk of malignancy
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ataxia-telangiectasia
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thrombocytopenia, eczema, and increased susceptibility to infection (males-x-linked recessive)
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Wiskott-Aldrich Syndrome (low IgM, increased IgA
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resp tract infections, Urinary tract, infections and gi infections
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selective IgA deficiency (IgA is major immunoglobulin in upper airway)
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severe recurrent infections of skin and lymph nodes, osteomyeitis HSM
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Chronic Granulomatous Disease (impaired neutrophils)
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hypocomplimentemia in which renal diseases
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lupus nephritis and PSGN, not in HSP or MCD
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increased IgE with eosinophilia, recurrent Staph infxns, pruritic eczematoid dermatitis
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Job Syndrome (neutrophil chemotactic defect)
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GVH Disease (acute)
donor lymphocytes against host MHC antigens |
abnormal LFTs->choleestatic hepatitis
diarrhea-> enteritis erythroderma |
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GVH (chronic)
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dry eyes and mouth --> Sjorgrne's syndroem
SLE Scldroderma primary biliary cirrhosis tx: corticosteroids, Abs to T cells |
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irritability may be a sign of what in a child with a prolonged fever (>7 days)
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Irritability may be the only way that a young child can express pain or discomfort, including meningeal irritation, headache from intracranial irritation and simple exhaustion.
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fever of more than 5 days, rash is a classic finding, particularly when combined with conjunctivitis and refusal to walk, due to painful, swollen feet
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think about Kawasaki Disease
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sandpaper rash
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Scarlet Fever
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caused by parvovirus B19. There is frequently an associated low-grade fever (100 to 101) with a rash appearing 7 to 10 days later. The characteristic rash starts as facial erythema -- the "slapped cheek" appearance. This can spread to the trunk and have an erythematous macular appearance. Central clearing of the rash appears, giving a lacy appearance. The rash often lasts longest on the extremities, where it has a lacy, reticular appearance.
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Erythema infectiosum - Also called fifth disease, t
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After a prodrome of fever (over 101), cough, coryza, and conjunctivitis, this maculopapular rash starts on the neck, behind the ears, and along the hairline. It spreads downward, and reaches the feet in 2-3 days. Immunization is very effective in preventing this infection.
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measles
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This macular or maculopapular rash starts on the trunk and spreads to the arms and neck. There is usually less involvement of the face and legs. The rash is preceded by 3-4 days of high fevers, which end as the rash appears. It is usually seen in children less than 2 years old.
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roseola
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The rash starts on the trunk and spreads to the extremities and head. Each lesion progresses from an erythematous macule to papule to vesicle to pustule, and then crusts over. Lesions at various stages of development are seen in the same area of the body. There is usually a mild fever. The disease is self-limited, lasting about 1 week.
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varicella
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strawberry tongue: erythematous tongue with prominent papillae
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streptococcal pharyngitis and Kawasaki disease
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DKA sxs (random glucose >200mg/dl
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abdominal pain and vomiting, with no fevver, dehydration, altered mental status 2/2 electrolyte imbalances or dehydration, tachypnea,
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Hypotonic/Hyponatremic
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(Na<130 mEq/dl)
Mild forms can be seen in viral gastroenteritis. More severe forms (sodium <120mg/dl) can be seen in free water or diluted formula replacement, or in adrenal insufficiency. |
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isotonic dehydration
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(Na = 130-145 mEq/dl).
This is the most common type of dehydration in children, including children presenting with acute gastroenteritis and in DKA. |
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Hypertonic/Hypernatremic
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(Na>145 mEq/dl)
This is seen most commonly in breastfeeding failure, use of inappropriate rehydration solutions (boiled milk) and diabetes insipidus. |
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hyperglycemia and metabolic acidosis caused by elevated serum ketones and lactic acidosis, which then affect electrolytes and serum osmolarity
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DKA is characterized by
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some consequences of hypoglycemia
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tachycardia, tremulousness, diaphoresis, confusion, irritability, hunger, lethargy, slurred speech, loss of consciousness, and seizures.
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treatment of cerebral edema
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If cerebral edema is suspected, it must be treated immediately. Treatment includes mannitol 0.25-1 mg/kg IV, decreasing the IV fluid rate, and elevating the head of the patient's bed.
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207: idiopathic pulmonary hemosiderosis
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A disease of unknown etiology characterized by recurrent hemorrhaging from pulmonary capillaries: hemoptysis. BAL would show hemosiderin laden macrophages
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bronchoalveolar lavage
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Bronchoalveolar lavage (BAL) is a medical procedure in which a bronchoscope is passed through the mouth into the lungs and fluid is squirted into a small part of the lung and then recollected for examination. BAL is typically performed to diagnose lung disease. In particular, BAL is commonly used to diagnose infections in people with immune system problems, pneumonia in people on ventilators, some types of lung cancer, and scarring of the lung (interstitial lung disease).
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unilateral nasal discharge, obstruction, and fould smelling discharge, purulent, maldorou,sb.oody discharge
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examine for a nasal foreign body
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210. dangerous consequence of staph pneumonia
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tension pneumothorax. tx by inserting a needle or cath into the 2nd or 3rd intercostal space in midclavicular line
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bacterial tracheitis
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severe adn life-threatening sequelae of viral laryngotracheobronchitis. days of viral URI -> acute elevation of temp --> resp ditress, biphasic stridor. tx: establishign an airway with intubation and IV antibiotics.
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pus in middle meatus of the nose is draining from...
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maxillary sinux, frontal, ro anterior ethmoid sinuses
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tx of sinusitis
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antibiotics for 10-14 days
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inspiratory stridor
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croup: extrathoracid airway collapses on inspiration
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treatment of croup
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supportive, but racemic epi and corticosteroids reduce length of time in ER and hospital
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fever, cough and tachypnea in a child with Sickle Cell Anemia
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manifestations of pneumonia, pulmonary thromboemboli, or sepsis. need to manage patient immediately.
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triad of situs invertsus, chronic sinusitis and otitis media, and airway disease
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Kartagener's syndorme: primary ciliary dyskineesia in 50% of pts
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congenital cystoid adenomatous malformation
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congential dysfunction of the development of the bronchioles
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inclusion conjunctivits and afebrile pneumonia in the neonate, think..
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chlamydia trachomatis (pneumonitis syndrome)
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recurrent pneumonia in an otherwise heatly child...think
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foreign body/anatomical blockade of airway
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what happens to your acid/base system if you loose xs potassium (ex: from diuretics)
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you have a metabolic alkalosis (K exits the cell, H goes into the cell). You can then get compensatory hypoventilation to increase PCO2
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when assessing an acutely ill child, what are the three things you need to do first
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ABCs: establish a patent airway, assess breathing and respiratory distress, assess that there has been no circulatroy compromise.
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What is Cushing's triad?
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hypertension, bradycardia and respiratory depression are signs of increased intracranial pressure, but will not occur until the patient is near the point of uncal herniation
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Name 3 metabolic causes of increased ICP.
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Metabolic causes of increased ICP include Reye's syndrome, liver failure, and electrolyte disturbances. These are diffuse encephalopathies and are unlikely to present with focal findings. Increased ICP can occur and can present with altered mental status and papilledema.
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Would you expect to see papilledema with meningitis?
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no, Meningitis usually presents with altered mental status (irritability, lethargy, coma), fever and signs of meningeal irritation. Focal findings are rare, as is papilledema. In fact, finding papilledema should prompt you to seriously consider other diagnoses.
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The constellation of anemia, basophilic stippling and constipation in a child with radiodense material in the rectum points to the diagnosis of
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lead intoxication. The presence of cerebral edema and seizures raises the diagnosis to acute lead poisoning.
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What is Zellwegger Syndrome?
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rare AR condition causign progressive degeneration of liver and kidneys. Usuallyfatal in 6-12 months. GENERALIZED HYPOTONIA, imparied neurologic function
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Upslanting palpebral fissures, small ears, a flattened midface, epicanthal folds, hypotonia and redundant nuchal skin are common features of infants with
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Down syndrome
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lab diagnosis for Down's Syndrome
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Lymphocyte Karyotype
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what endocrine abnormality are down's syndrome babies at risk for
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hypothyroidism
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lymphedema in utero, which is the cause of many of the physical findings such as the webbed neck, low placement of ears, edema of the hands and feet, hyperconvex nails and a "shield" chest with widely spaced nipples. Coarctation of the aorta is found in about 20% of affected girls. Short stature is common, and some girls are not diagnosed until early adolescence when they present with short stature and delayed sexual maturation (due to gonadal dysgenesis). Most have a normal IQ.
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Turner's Syndrome
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Clinical features include microphthalmia, microcephaly and severe mental retardation, polydactyly, cleft lip and palate, cardiac and renal defects, umbilical hernias, and cutis aplasia.
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Patau's (Trisomy 13)
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Clinical features include severe mental retardation, prominent occiput, micrognathia, low-set ears, short neck, overlapping fingers, heart defects, renal malformations, limited hip abduction, and rocker-bottom feet.
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Edward's (Trisomy 18)
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What is the dif between a holosystolic murmur and an ejection murmur
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A "holosystolic" murmur is S1 coincident, meaning that the murmur starts with S1, not after it. An ejection murmur is also systolic but does not start until after S1 because there is a delay from S1 to the onset of ejection - the isovolumic contraction time.
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What is a holosystolic murmur associated with?
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A VSD, mitral insufficiency and tricuspid insufficiency.A VSD causes a holosystolic murmur because flow through the VSD starts with the onset of ventricular contraction.
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What is an ejection murmur associated with?
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Ejection murmurs occur with aortic and pulmonic valve stenosis.
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differential of hepatomegaly in an infant
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The differential of hepatomegaly in an infant includes congestive heart failure, congenital infections, inborn errors of metabolism, anemias, and less commonly tumors.
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Still's murmur
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Innocent murmur: This is often described as musical or vibratory, and is heard best at the left lower sternal border in the supine position.
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widely split, fixed S2 in a child aged 3-5 years
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pathognomonic physical finding of an atrial septal defect.The systolic murmur is due to increased flow across a normal pulmonic valve.
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when does coarctation of the aorta present?
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infancy or at any age beyond because it tends to be a progressive problem, gradually getting more severe over a number of years. Coarctation presents with a murmur, hypertension in the upper extremities, and a discrepancy between the upper and lower extremity blood pressures.
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what congenital heart defects typically present in infancy?
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Ventricular septal defects, aortic and pulmonic stenosis, patent ductus arteriosus, and tetralogy of Fallot
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There is a prominent systolic ejection click just after S1, and a harsh systolic ejection murmur.
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pulmonic stenosis
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vibratory and low pitched, and is heard best at the left lower sternal border. The vibratory quality is the most characteristic feature of an innocent murmur.
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innocent murmur
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Which congenital heart defects can present with CHF in the newborn?
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VSD, Aortic Stenosis, coarctation of the aorta, large PDA
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pathophysiology behind CHF in children 2/2 a VSD
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The infant with a VSD is supplying enough blood to the tissues to meet the body's metabolic needs, but is doing it from an elevated filling pressure (due to the left ventricular volume overload). The adequate cardiac output is achieved by creating a high adrenergic state and by activating the renin-angiotensin system. Many of the symptoms of CHF in the infant are caused by these neurohormonal changes.
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why are VSD typically not heard at birth, but wks later
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Newborns have elevated pulmonary vascular resistance. Since the systemic and pulmonary vascular resistances (PVR) are nearly equal, there is no reason for blood to shunt through the VSD. Classic teaching is that VSD murmurs are not heard in the nursery, and that was the case for Tyler. The murmur of a VSD will appear when the PVR drops, usually at a few days to weeks of age.
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treatment of CHF 2/2 a VSD in an infant
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Digoxin and diuretics
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What is Eisenmenger's syndrome?
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The pulmonary vasculature constricts in response to exposure to high pressure and high flow. With chronic exposure to these conditions the pulmonary vasculature will develop permanent changes and loses the ability to relax, even if the VSD is closed. This causes the pulmonary vascular resistance to exceed the systemic resistance, and shunting through the VSD will shift to right to left. The patient will then develop cyanosis, progressing to polycythemia, heart failure and death. Most patients with Eisenmenger's disease will live into their twenties, but with a very impaired quality of life.
Patients with a large unrepaired VSD will universally develop Eisenmenger's disease. In general this will not occur before 2 years of age, but VSD closure is now recommended at earlier ages to completely prevent development of Eisenmenger's disease. |
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fever + petechial rash
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think meningococcemia
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5 cyanotic heart diseases
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1. Truncus Arteriosus
2. Transposition of the Great Vessels 3. Tricuspid Atresia 4. Tetralogy of Fallot 5. Total Anomalous Pulmonary Venous Return |
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Signs and Sxs of Kawasaki's Disease
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"CRASH and BURN"
Conjunctivitis Rash Adenopathy Strawberry Tongue Hand and Feet Edema/Swelling Burn: Fever x 5days tx: high dose ASA and IVIG within 10days |
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fever and petechiae on palms and soles of feet
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RMSF
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fever and sandpaper/sun-burn type rash
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Toxic Shock Syndrome
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treatment of undescended testes if not palpated in the inguinal canal
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orchiopexy after 12 months of age
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treatment of undescended testes if palpated in the inguinal canal
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hormonal therapy with LH-releasing hormone is controversial, but practiced?
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gynecoogical tumor assoc with elevated beta-HCG
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seminoma/dysgerminoma. most common gemr cell tumor. tx: radiation
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what type of ulcers do chancroids and herpes cause
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PAINFUL ULCERS
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what type of ulcers do lymphogranuloma venereum (LGV) and syphilis cause?
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PAINLESS ULCERS
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PID treatment
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Doxycycline for 2 wks.
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Chlamydia treatment
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Zithromax or Coxy, Erythromycin, Levofloxacinor ofloxacing for 7 days
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Dx of HSV
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Tzanck smear, tx: acyclovir
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Dx of syphillis and tx
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T. pallidum on dark-field microscopy and penicllin
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Dx of chancroid and tx
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Gram stain reveals gram-positive cocci arranged in boxcar formation. Tx: Azithromycin
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Dx and Tx of Lymphogranuloma Venereum
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Elevated Ab titers on complement fixation and microimmunofluorescence tests.
tx; Azithromycin |
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when someone is suspected to have Ghonorrhea, what should you do?
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treat for BOTH gonorrhea and chlamydia
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microcytic anemia, increased RDW, decreased ferritin, decreased TIBC
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Fe deficiency anemia
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poikilocytosis
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variation in SHAPE of RBCs
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anisocytosis
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variation in SIZE of RBCs
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most common causes of hypochromic microcytic anemia
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Fe deficiency, Anemia of Chronic Disease and Thalassemia Syndromes
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4 sickle cell crises
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vaso-occlusive, aplastic (parvovirus), sequestration, hemolytic
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increased osmotic fragility, increased retic count, Positive family hx, and splenomegaly.
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Hereditary spherocytosis (negative coombs)
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Fever, anemia, thrombocytopenia, renal dyfunction, and neurologic abnormalitity
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"FAT RN": TTP
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child with epistaxis, prolonged bleeding time, and nml platelet count
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von Willebrand's Disease
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what is the most common cause of amblyopia
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strabismus
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watery, itchy red eyes with edema to the conjunctiva and lids,
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allergic conjnctivitis: IgE medisated rxn 2/2 trigtgers like pollen or dust
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conjunctivitis with lymph nodes
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viral etiology: watery, red eyes with preauricular lymph nodes
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most common cuases of viral conjunctivitis
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adenovirus and coxsackievir
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mucopurulent dishcage, red eyes, adn edema of the conjunctiva
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bacterial: H. influenza and S. pneuminae are typical
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what is dacryostenosis
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congential nasolacrimal duct obstrution: see chronic tearing (overflowing), erythema 2/2 rubbing
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4 mo old child with an exudative eye discharge and a painful, red lacrimal sac
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dacrocystitis
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sxs of orbital cellulitis
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inflammation ofthe orgbital tissuesBEHIND the septum: PAINFUL extraocular motion, proptosis, decreased vision, erythema, eddema: IV Abx b/c EMERGENCY
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sxs of periorbital cellulitis
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infl of eyeleids and periorbital tissue ANTERIOR to the septum: erythema, edema, NO PAIN with extraocular mvmts. tx: ceftriaxone
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most common organisms causing periorbital and orbital cellulitis
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SHIP: S. aureus, H. influenza, S. pneumoniae
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Leukocira (white pupilary reflext), strabismus, orbital inflammation, hyphema (blood layering anterior to the iris)
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Retinoblastoma: most common orbital primary malignant tumor in children
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2 common comp of otitis media
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overall: hearing loss, intracranial: meningitis
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"swimmer's ear": ear painw ith mvmt of pinna, pruritis, edema of ear canal, palpable lymph nodes
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otitis externa: most common is P. aeruginosa
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Meniere's triad
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vertigo, tinnitus, and hearing loss
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What two diuretics asre ototoxic
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FUROSEMIDE and ethacrynic acid
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Which Abx are ototoxic?
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AMINOGLYCOSIDES, Gentamicin, erythromycin, Minocycline, Quinolones
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Which Chemotherapeutics are ototoxic?
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Cisplatin, Vinblastine
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Ototoxic Antimalarials?
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quinine, chlororquine, mefloquine
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Ototoxic Antiarrhythmiccs
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Quinidine
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Ototoxic Salicylates
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Aspirin
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Paraneoplastic syndrome of Nasopharyngeal Carcinoma
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Clubbing, fever, SIADH
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endocrine disorder assoc with HLA-DR3 and DR-4
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Type I DM
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insulin increaseed, normal or decreased, but insulin resistance
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Type II DM
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hyperglycemia >300mg/dL, acidosis pH<7.30, Bicarbonate < 15 mEq/L
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DKA: medical emergency b/c can progess to coma and death
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sxs of DKA
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polyuria, polydipsia, fatigue, HA, nausea, vomiting, tachycardia, ari hunger
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most severe complication of DKA management
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cerebral edema: if glucose levels fall too quickly. Insulin should be given 0.1U/kg/hr.
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which DM has a greater family hx
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Type II DM
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Traid of hemochromatosis. what can cause hemochromatosis. labs?
hemocrhomatosis: increase storage of iron in the form of hemosiderin |
triad: cirrhosis, bronzing of skin, DM
cuases: hereditary, neonatal, transfusion induced. Lab: increased ferritin adn transferrin |
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tx of hemochromatosis
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chleation with desferoxamine
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3 y/o boy that is unconscious, flushed face, pulse of 160, RR of 16, BP of 40/20. recalt temp of 36.2. unusual odor on his breath. he ahs a GTC seizure
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DKA and check serum glucose
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triad of hyperthyroidism with diffuse goiter, opthalmopahthy, dermopathy, gradual onset: 6-12 mo
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Juvenile Graves' Disease
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medical tx of hyperthyroidism
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Propylthiouracil 5-10 mg/kg/day q8h PO and Propranolol 0.5-1 mg/kg/day PO, thyroidectomy
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acquired hypothyroidism
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Hashimoto's thyroidits is most common cause. see growth deceleration. tx: l-thyroxine 10 micrograms/kg/day
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voracious appetite (c or s weight gain or loss, heat intolerance, emotionla labiity, restlessness, sx sweating, stools, poor sleep. declining school performance, flushed and fidgety, and warm
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hyperthyroidism: Grave's Disease most comon.
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Acute onsetn of hyperthermia, seere tachycardia leadin to cardiogenic shock, CNS manifestations, diaphroesis, N/V
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throid storm which may be life threatening.
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clinical manifestations of hypercalcemia should clue you into what endocrine abnormality: muscle weakness, anoreixa, N/V/C, polydipsia, polyuria, weight loss, fever
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hyperparathyroidism: PTH causes an increase in Ca, decrease in P.
can be primary or 2/2 chronic renal failure, liver disease, or vitamin D deficiency |
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combination of hyponatermia and hyperkalemia clue you into what congenital problem
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congenital adrenal hyperplasia: most commonly caused by 21-hyrdoxylase deficiency. can have severe salt wasting due to decreased aldosterone and cortisol. Females have ambiguosu genitalia.
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urgent tests with suspected congential adrenal hyperplasia
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1. serum glucose
2. serum electrolytes (others: cortisol, testosterone, 17-OH progesterone) |
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Addison's Disease
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Primary Adrenal Insufficiency, destruction of adrenal cortex: autoimmune, TB
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sxs: weakness, nausea, vomiting, weight loss, HA, emotional lability, salt craving. physical : postural hypotension, hyperpigmentation
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Addison's Disease (primary adrenal insufficiency).
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increase in epinephrine and norepi, wkth HA, palpitations, diaphoresis, abdominal pain and flushing
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pheochromocytoma of the adrenal medulla. if only norepi is high, then think of extra-adrenal tumor
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what endocrine abnormality can cause pubertal delay
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hypopituitarism (decrease in LH and FSH)
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what endocrine abnormality can cause hypoglycemi and micropenis in a neonate
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hypopituitarism (decreased ACTH and GH)
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what endocrine abnormality can cause poor linear growth and hypoglycemia
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GH deficiency
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how do cortisol and GH regulate insulin
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GH decreses insulin and Cortisol increses insulin?
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etiology of gigantism, rapid linear growth, caors facial features, nelarging hadns and feet
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Pituitary adenoma causing increased GH
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normal female pubertal progression
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thelarche -> height growth spurt -> pubic hair -> menarche (13 y/o)
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normal male pubertal progression
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testicular enlargemetn -> penile enlargement -> height groth spur (14 y/o) -> pubic hairmm
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Kallman Syndrome
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X-linked hypogonadotropic hypogonadism affecting males sand females, associated with anosmia, cleft lip/palate, and other midline defects
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primary amenorrhea: definition and most common cause
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no menstural flow by age 16. most common cause is Tuner's Syndrome
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primary amenorrhea in an unambiguous female. Presence of testes in inguinal hernia
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Testicular feminization: androgen insensitivity syndrome. XY males with testess appears as unambiguous female
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normal testes (both gonads are testes) but undervirilization of external genitalia
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Male pseudohermaphroditism 2/2 androgen insensitivty or enzyme defects int estosterone syntheiss.
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pink macules and papules, initially on face adn spred inferiorly within 24 hrs
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Rubella (German Measles)
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eriythematrous macules and papules initialy along hairline, spreading inferiorly w/in 2-3 daiys, fade in 4-6 d with desquamation. hint: bluish-white papules on erythematous base appear on day 1-2 of fever, over buccal mucosa, adjacent to second molars
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Koplik's spots with Measles (rubeola)
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stomatitis (vesicles rapidly opne to painful ulcers). Gray blisters on hands and feet on background erythema
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Hand-foot-mouth disease (Coxsackie A virus)
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2-6 mm blanchable macules that first appear peripherally on wrists,forearms,k ankles, palms and soles. spreads to trunk proiximal extremitie adn face w/in 6-18 hours. evolve to deep red papules adn petechiae over 1-3 days. W/in 2-4 daiys, exanthem is no longer blanchable
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RMSF (Rickettsia rickettsii)
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"slapped cheks"-red papules coalesce on face. retiulate rash on buttocks and upper arms taht spreads. Palmos and soles may be involved. mucous membranes may have red spots
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Fifth disease (eriythema infectiousum ) (parvovirus B19)
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discrete, pink macules, papules, and petechiae over trunk, extremities, adn palate
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meningococemia (n. meningitidis)
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erythematous macules over arms and legs evolve itno hemorrhagic, painful pustules w/in 2-3 days
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Gonococcemia (n. gonorrhea)
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painless "button-lke" chancre with indurated borders
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Primary Syphilis
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multipel, dscrete, firm "ham-colored" papules scattered symmetrically over trunk, palms, soles, adn genitals; condyloma lata-soft lfat topped pink papules in anogenital region
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secondary syphillis
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erythema chronicum migrans-expanidng, erytheatous, annular plaque with central clearing
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Lyme Disease (Borrelia burdorferi)
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erythematous macules and plaques appear in stocking and glove distribution 1-3 days after onset of fever. Spreads to involve trunk and extremieits w/in 2 daiys, lasts an average of 12 days
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Kawasaki's disease
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raised patches on lower back with orange-peel texture.
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Tuberous sclerosis and Shagreen patches
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red, vascular noduels on face that may resemble aggravated acne
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Tuberous sclerosis and Adenoma sebaceum
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hemangioma variant; appears as sharply marginated, red or purple macule, commonly distruibuted unilarterally on face; presenta t birth adn never disapperas; lesion grows propritonallt o siz eof person, may develop pappula ran dnodular areas
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Sturge-Weber syndrome adn port-wine stain
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janeway lesion asn osler's nodes
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Janeway Lesions: multiple, hemorrhagic, nontender macules on fingers and toes. Osler's nodes: tender, violaceous subcutaneous nodules on palms and soles.
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palpable purpura (small vessel damage), arthritis, and abdominal pain.
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HSP
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what's the problem with accutane (oral isotretinooin)
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teratogenic
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threadlike burrows in the interdigital areas, growin, elbows, ankles
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think scabies
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signs and sxs of scabies
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pruritis, 1-2mm red papules, some with excoritaitos, crustina gn scalin. tx with lindane or permethrin in infants
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umbilicated (dome-shaped), skin-colored or pearly-white papules. what is tx
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think molluscum contagiosum. it is ia self-liited, contagious, vrial infection transmitted by direct contact. it is caused by a poxvirus. tx: curettage, cryosurgery, sometimes observation
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what causes scarlet fever
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GAS
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what is scarlet fever?
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toxin-mediatd dz with sore throat, high fever, and mucous membrane eirythema. "strawberry tongue"
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linear petechiae evident in body folds after strep infectoin
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pastia's sign of scarlet fever
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what can SJS progress to
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TEN
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how do you differentiate psoriasis from Reiter's syndrome witha psoriatic rash
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Reiter's syndrome will inolve the mucous membranes, Psoriasis does not.
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digitalis toxicity in infants
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vomiting
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major criteria in rheumatic fever
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carditis, arthritis, erythema marginatu, chorea, and subcutaneous nodules (joints, carditis, nodules, erythem, sydenham's chorea)
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minor for RF
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arthalgias, fever or hx of RF, increased ESr, Pos CRP, increase WBC and anemai, prolonged PR and QT on ECg. most common finding is arthralgias
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hereditary angioedema
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AD pattern , defect in complement -> increased kinin and edema in skin, sometimes throat and gi
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ellis0van creveld syndrome assoc with what congenital heart defect
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atrial septal defects
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digitalis toxicity in infants
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vomiting
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major criteria in rheumatic fever
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carditis, arthritis, erythema marginatu, chorea, and subcutaneous nodules (joints, carditis, nodules, erythem, sydenham's chorea)
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minor for RF
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arthalgias, fever or hx of RF, increased ESr, Pos CRP, increase WBC and anemai, prolonged PR and QT on ECg. most common finding is arthralgias
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hereditary angioedema
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AD pattern , defect in complement -> increased kinin and edema in skin, sometimes throat and gi
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ellis0van creveld syndrome assoc with what congenital heart defect
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atrial septal defects
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supraventricular tachycardia and tx
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rapid heart rate, little variability in rate, and P wave before eveiry QRS. Tx by stimulating the vagus nerver b/c increased SVT can lead to hydrops fetalis.
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a pulse greater than 250 should clue you into
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tachyarrhiythmia.
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chf from any cause can cause what
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cyanosis, pallor, dyspnea, tachypnea, tachycardia, and cardimegaly
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Ebstein's anomaly
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Downward displacemnt of the tricuspid valve. confirmed by RV hypertrophy and RV conduction defects.
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spindle shaped swelling of finger joints. systemic sxs: constituaionl dz, rheuamtoid rash, high spiking fevrs. diffuse rash
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JRA
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left axis deviation and hypertrophy of the LV can happen in what cyanotic heart defect
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Tricuspid Atresia can cause a hypoplastic RV leading to left axis deviation. the rest cause right axis deviation
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egg on a string, early cyanosis, normal sized heart,
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transposition of the great vessels
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marfan syndrome eye finding
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subluxatin of the ocular lens (ectopia lentis) also dilatation of aroticc root and ascendign aorta, aorti caneurysm, xs height for age, long and thi extremities
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Williams syndrome
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supraventricular aortic stenosis mostly, also hypercalcemia in infancy, MR, and prominent thick lips, long philthrum, short stature, cog impairments
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long QT
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can cuase syncopal episodses in late childhood and adolescnce. arrhythmias may be noted, like v.fib.
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complication of neonatal lupus
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thrombocytopenia, neutropenia, rash, liver dysfunction, and congenital heart block
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CHF presentation in an infant
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weak, diaphoretic, poor weight gain, tacypnea w/ retractions. lungs: cracckles, wheezes.
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nocturnal abdomainal pain adn gi bleeding with pos fam hx, discomfort, vomiting, anorexia, weight loss, hematemeis or melena
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peptic ulcer disease. pain is most common
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painless intermittent rectal bleeding in early childhood
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Mecke's diverticulum. dx by meckel's scan
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1st week of life with billous vomiting, adn/or intermittent abdominal pain. acute presntation caused by volvulus of hte intestines.
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malrotation with volvulus (films show a dilated stomach adn proximal loops of bowel and "curl Q" twist on upper GI when barium through malrotated intestines. Billous vomiting 2/2 obstruction.
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4 wk old with nonbillous vomiting for 10 days
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pyloric stenosis. often see metabolic alkalosis with low K and Chloride.
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RUQ pain with fever, vomiting, nausea,k adn jaundice....
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acute cholecystitis. confirm with u/s of gallbladder. may happen in child with SCD
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intussusception
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infant btween 4-10 months with sudden onset of intermittent colicky abd pain. passage of stool with blood and mucus, like current jell. Contrast enaema exam can be benefitical and diagnositc
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protein requirement to prevent kwashiokor
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8% of diet
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Williams Syndrome
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WILLIAMS:
Weight (low at birth, slow to gain) Iris (stellate iris) Long philtrum Large mouth Increased Ca++ Aortic stenosis (and other stenoses) Mental retardation Swelling around eyes (periorbital puffiness) |
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Hemolytic Anemia
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"Remember to decrease the RATE of IV fluids in these patients":
Renal failure Anemia (microangiopathic, hemolytic) Thrombocytopenia Encephalopathy (TTP) |
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Pyloric Stenosis
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Pyloric stenosis is 3 P's:
Palpable mass Paristalsis visible Projectile vomiting (2-4 weeks after birth) |
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what happens with XS Vitamin A, E and K
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Excess vitamin A: Anomalies (teratogenic)
Excess vitamin E: Enterocolitis (necrotizing enterocolitis) Excess vitamin K: Kernicterus (hemolysis) |
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Beckwith-Wideman Syndrome
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HOMO:
Hypoglycemia Omphalocel Macroglossia/ Macrosomia Organomegaly |
