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Flashcards in Peds Deck (26)
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1
Q

child with small or absent lymphoid tissue and recurrent sinopulmonary and gastrointestinal infections

A

X-linked/Bruton agammaglobulinemia (results from a failure of B cell development) Treatment=monthly IV Ig infusion

2
Q

severe-combined immunodeficiency; child with severe, recurrent viral, fungal, and bacterial. infections and failure to thrive is caused by what?

A

adenosine deaminase deficiency=impaired T cell development

3
Q

child with disseminated bacterial infections with encapsulated bacteria (strep pneumo, neisseria meningiditis)

A

complement deficiency

4
Q

recurrent skin and pulmonary infections with catalase positive organisms (staph and serratia)

A

impaired oxidative burst in chronic granulomatous disease

5
Q

child with hypocalcemia, cardiac defects, failure to thrive, recurrent infections

A

thymic hypoplasia with DiGeorge syndrome (22q11.2 microdeletion)

6
Q

baby/child with hepatosplenomegaly and cherry red macula on exam

A

Niemann-Pick Disease

7
Q

what is deficient in Niemann Pick Disease?

A

sphingomyelinase dificiency

8
Q

cherry red macula with NO hepatosplenomegaly

A

Tay-Sachs Disease

9
Q

what is deficient in Tay-Sachs disease?

A

B-hexosaminidase A deficiency

10
Q

galactocerebrosidase deficiency

A

Krabbe disease

11
Q

glucocerebrosidadse deficiency

A

Gaucher disease

12
Q

lysosomal hydrolase deficiency with coarse facial features and corneal clouding

A

Hurler syndrome

13
Q

what are the signs and sx in CHARGE syndrome?

A
  • Coloboma
  • Heart defects
  • choanal Atresia
  • Retardation of growth/development
  • Genitourinary abnormalities
  • Ear abnormalities
14
Q

what are the signs and sx in VACTERL?

A
  • Vertebral
  • Anal atresia
  • Cardiac
  • TE fistula
  • Renal
  • Limb
15
Q

jaundiced baby that starts at 3-5 days and peaks at 2 weeks; no signs inadequate breast feeding or dehydration

A

breast milk jaundice

16
Q

jaundiced baby that runs yellow within the first week of life; has difficulty eating/latching and is dehydrated

A

breast feeding jaundice

17
Q

treatment for breastfeeding jaundice

A

increase frequency and duration of feeds to stimulate milk production

18
Q

growth failure and recurrent respiratory infections in infants is concerning for___

A

CF (pancreatic insufficiency; may also see greasy stools

19
Q

what is the dosing for HPV vaccine?

A
  • <15 years old= 2 doses 6 months apart
  • >15 years old= 3 doses
20
Q

most ocmmon cause of acute unilateral cervical lymphadenitis in children

A

staph aureus or strep pyogenes

21
Q

rapidly spreading rash in children <5 years of age with fever and sloughing off of skin

A

staphylococcal scalded skin syndrome

22
Q

rash that starts on the face and moves caudally; may have suboccipital or postauricular lymphadenopathy

A

rubella= German measles= togavirus

23
Q

treatment of group A strep/ strep pyogenes pharyngitis?

A

10 days of oral amoxicillin or penicillin G

24
Q

Klinefelter syndrome

A

47 XXY male, tall with long limbs, gynecomastia, small penis, sparse facial hair, small firm testes

25
Q

lab findings characteristeic of Klinefelter syndrome

A

elevated FSH and TSH, low testosterone, decreased sperm count

26
Q

pt with klinefelters are at increased risk of developing _____

A

extragonadal germ cell tumor