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37 Cards in this Set
- Front
- Back
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what are important steps that children with sickle cell should take to prevent pneumococcal sepsis?
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pneumococcal vaccination
prophylactic penicillin BID (til age 5) |
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what are the characteristics of Turner syndrome?
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45, XO
primary amenorrhea tanner stage 2 aortic coarctation |
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what are the characteristics of Lesch-Nyhan syndrome?
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hypoxanthine-guanine phosphoribosyl transferase (HPRT) deficiency
selfmutilation neurologic (retardation, dystonia, spasticity, choreoathetosis) gouty arthritis (*in a child) tophus formation |
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what does a (+) Coombs' test with a (+) osmotic fragility test imply in an jaundiced newborn of N. European descent with anemia?
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hereditary spherocytosis
a (+) Coombs' implies autoimmune anemia a (+) osmotic fragility implies hereditary spherocytosis |
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what are the characteristics of hyper-IgM syndrome (HIM)?
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high levels of IgM
def of IgG def of IgA poor response to immunizations |
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what are the (4) characteristics of X-linked agammaglobulinemia (XLA)?
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recurrent bacterial infections <5 yo
IgG, IgM, IgA 2 standard deviations below nl absent isohemagglutinins or poor response to vaccines <2% CD19+ B-cells |
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describe the typical patient with Osgood-Schlatter disease
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10-17 yo male
knee pain at the tibial tuberosity (site of insertion of the quadraceps tendon) |
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pt with acute onset of HA's and focal neurological symptoms after an episode of acute otitis media/sinusitis?
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brain abscess
(ring-enhancing lesions on CT/MRI) |
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what are the characteristics of acute post-streptococcal glomerulonephritis?
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follows pharyngitis or pyoderma
HTN edema low C3/CH50, normal C4 hematuria proteinuria incr ASO titers |
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what is Friedreich ataxia?
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mcc of spinocerebellar ataxias
neuro - (ataxia, dysarthria) skeletal - (scoliosis, feet) cardiac - (concentric hypertrophic cardiomyopathy) (mcc of death cardiomyopathy/respiratory) |
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what are Koplik spots?
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bluish white lesions on an erythematous buccal mucosa, (or conjunctivae/vaginal mucosa)
*pathognomonic for rubeola (measles) |
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vaccinations for preterm infants should be given according to their _____ age
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chronologic age
(exception: >2kg prior to first HepB) |
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systolic ejection murmur at L-sternal border with a widely split and fixed S2 is suggestive of a _____
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atrial septal defect
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what is the DOC in pregnancy for chlamydial infection
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erythromycin
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what is the mcc of infectious neonatal conjunctivits?
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chlamydia
(20% risk of pneumonia) |
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what is the mcc of congenital hypothyroidism in the US?
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thyroid dysgenesis
(aplasia, hypoplasia, ectopic gland) |
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what should you think of first in a 3-7 year old child in acute respiratory distress, toxic appearance (leaning forward and drooling), stridor and high grade fever?
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epiglottits - mainly due to Hib
(secure the airway with endotracheal intubation) |
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what are the criteria for Kawasaki's disease (mucocutaneous lymph node syndrome)?
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fever for >5days
4 of the 5 symptoms: - bulbar conjunctival injection - desquamation of finger/toe tips, indurative edema - chapped lips, strawberry tongue, oropharnyx injection - morbilliform truncal exanthem - cervical lymphadenopathy (txt: IVIG and high-dose aspirin) |
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failure to pass stool in the firs 24 hrs w/ ileal obstruction is suggestive of meconium ilius, diagnostic of _____
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cystic fibrosis
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what is the genetic defect in cystic fibrosis?
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mutation leading to deletion of a three base pair encoding for phenylalanine (DA508) in the CFTR gene on chromosome 7
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what are the features of a severe tricyclic antidepressant intoxication? (3)
txt? |
seizure
hypotension prolonged QRS (txt: sodium bicarbonate) |
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what is a cephalohematoma?
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subperiosteal hemorrhage
within few hours of birth scalp swelling limited to 1 cranial bone resolve in 2 wks-3 months |
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a diffuse swelling of the scalp that extends across the midline crossing suture lines in a child born vertex is called _____
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caput succedaneum
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what are the characteristics of a child with a craniopharyngioma? (3)
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increased ICP (HAs/V)
bitemporal hemianopsia calcified lesion above the sella (cystic calcified parasellar lesion on MRI is almost diagnostic) |
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what is the mcc of SAH in children?
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arteriovenous malformation (AVM)
(seizures and HAs) |
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what are the characteristics of DiGeorge syndrome children?
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cyanotic heart disease
craniofacial anomalies thymic hypoplasia cognitive impairment hypoparathyroidism (monitor calcium w/ prolonged QT) |
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what organ system has the highest morbidity and mortality after HUS?
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kidneys
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what is hemolytic-uremic syndrome (HUS)?
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caused by a toxin released by e.coli which enters the systemic circulation and injures the endothelial cells in the kidney
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when do you see red currant jelly stools (blood/mucus)?
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intussusception
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what are the diagnostic features of severe combined immune deficiency (SCID)?
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absent lymph nodes/tonsils
lymphopenia absent thymic shadow on CXR abnl T/B/NKiller cell number |
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when should you operate on an umbilical hernia?
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if it persists til age 3-4 y
>2 cm in diameter symptomatic enlarges after age 1-2 y |
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what is the mcc of acute UTI in children?
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vesicoureteral reflux
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mcc of mortality in infants between 1 m and 1 y
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sudden infant death syndrome (SIDS)
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what is the mcc of congenital adrenal hyperplasia?
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21-hydroxylase deficiency
- virilism - salt wasting - incr 17-alpha-hydroxyprogesterone |
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what do you treat neonatal chlamydial conjunctivitis with?
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oral erythromycin
(systemic to prevent chlamydial pneumonia) |
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useful test to discriminate between functional constipation and Hirschsprung disease?
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anorectal manometry
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what age is concerning if no meconium has been passed?
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>48 hrs
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