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Flashcards in Pediatrics section within UW Deck (219)
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1

Gold standard for duchenne diagnosis?

Genetic testing. Shows deletion of dystrophin gene: Xp21

2

Onset of Duchene vs Becker

Duchene by 2-3 years old, Becker 5-15 years old.

3

What enzyme levels are reaised in DMD?

Serum creatine phosphokinase and aldolase

4

Treatment for subluxation of radial head

Hyperpronation of forearm, supination of forearm and flexion of the elbow.

5

What is the first and second most common posterior fossa tumor?

1) cerebellar astrocytoma 2) medullobastoma (cerebellar vermis)

6

Posterior vermis syndrome is characterized by?

Truncal and gait ataxia

7

Lesch Nyan syndrome secondary to deficiency in?

hypoxanthine-guanine phosphoribosyl transferase (HPRT). X linked recessive disorder. Enzyme is involved with purine metabolism.

8

Sx of Lesch Nyan

First appears at 6 months w/ vomiting and dystonia. Then progressive mental retardation, choreoathetosis, spasticity, dysarthric speech, dystonia, self injury. Xs uric acid builds up leading to gouty arthritis, tophus formation and obstructive nephropathy

9

IF you see a boy with gout, suspect?

Lesch Nyan (usually gouty arhritis manifests when you're 50 )

10

Marfan mutation of what gene

fibrillin 1 gene

11

What vitamin has shown to improve morbidity and mortality in measles

Vitamin A. through immune enhancement as well as regeneration of GI and respiratory eptihelium

12

Alpha adrenergic and beta adrenergic effects of epinephrine for asthma?

Alpha 1 adrenergic: vasoconstriction - increases blood pressure and decreases upper airway edema, reduces bronchial secretions, and mucosal edema. Beta 2 adrenergic: promotes smooth muscle relaxation, decreases systemic release of inflammatory mediators. These are effects of epinephrine.

13

In cases of croup, you should administer what before considering intubation?

Racemic epiphephrine. Reduces need for intubation

14

Metanephros is embryologic precursor for ?

Renal parenchyma

15

Wilms tumor arises from which embryogenic structure?

Metanephros

16

Three most common pediatric cancers?

Leukemia, CNS and neuroblastoma (TN says leukemia, CNS and lymphomas)

17

Neuroblastomas arise from which embryonic structure?

Neural crest cells => precursor of adrenal medulla and sympathetic chains

18

Most common location of neuroblastoma

Abdomen = adrenals or retroperitoneal ganglia

19

For neuroblastoma what can be seen on CT scan and plain x-ray

calcifications and hemorrhages

20

For neuroblastoma levels of what are elevated in serum and urine?

catecholamines and their metabolites (HVA and VMA)

21

Mesoneprhos

seminal vesicles, epidiydmis, ejaculatory ducts, and ductus deferens

22

Wilms tumor aka

Nephroblastoma

23

Freidrich Ataxia?

A/R condition. Parents should get genetic counseling for prenatal diagnosis with affected child.

24

Ppt of epiglotitis. Most concerning complication?

Rapid progression, drooling, sore throat, dysphagia, airway obstruction is most concerning complication.

25

Sudden LOC, disorientation, slow gain of consciousness?

Seizure

26

Post ictal paralysis aka

Todd's paralysis

27

Todd's paralysis

Post ictal condition that improves with restoration of motor function within 24 hours

28

What is micrognathia

Undersized jaw

29

Symptoms of Digeorge syndrome

CATCH 22. Cardiac anomalies (conotruncal defects, cyanotic CHD), Abnormal facies such as micrognathia and low set ears, Thymic hypoplasia (immunodeficiency, lymphopenia causing susceptibility to viral and fungal infections, poor T cell help could affect B cells and bacterial infections also), Cognitive impariment, Hypoparathyroid/hypocalcemia

30

Digeorge ngenetic abnormality?

Microdeltion of 22q.11