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Flashcards in PEDIATRICS Deck (115)
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1

Genetics in Noonan's syndrome

Common AD disorder due to mutation to genes involved in RAS-MAP-K pathway
Can affect both males and females (big difference between Turners)

2

Thickened helices

Noonans syndrome

3

Severe h/a and dizziness in pt with Noonans syndrome -- > screen for

Arnold Chiari malformation

4

Cardiac malformations that contrast Noonans vs Turners

Noonans: right sided heart lesions, HOCM
Turners: left sided

5

Diseases associated with Turners

Lymphedema
DM-1
Hashimotos
Autoimmune disorders

6

Difference between Sotos syndrome and Beckwith-Wiedemann

BW features hypoglycemia during newborn period, macroglossia, umbilical hernia and/or omphalocele.

7

Tumors assoc with Beckwith Wiedemann

10% will go on to develop small round blue cell tumors, most commonly Wilm's.

8

Croup pathogens

RSV, influenza, adenovirus, and parainfluenza virus ** most common.

9

Moderate/severe croup tx

oral or IM corticosteroids, INHALED racemic epinephrine.

10

Pathogens for Epiglottitis

USED to be H. influenza type B.
Now, strep, non-typable H influenza and viral agents.

11

Subglottic narrowing

croup

12

Supraglottic narrowing

epiglottitis

13

Epispadias is associated with/

Bladder extrophy

14

Where is the testicle usually found in cryptorchidism?

Inguinal canal.

15

Hydrocele is a remnant of what embryological structure

Tunica vaginalis.

16

How do you treat hydocele

It will usually resolve within six months.

17

Abnormality associated with hypospadias

Chordee, a ventral curvature of the peniis. The growth differences between the attenuated ventral urethra and its associated tissues, and the normal growing dorsal tissue, is thought to cause this. It is often apparent during an abreaction.

It is also highly a/w cryptorchidism and inguinal hernias.

18

How many DTaPs do you get

5. Four doses are adequate if the last dose was given on or after the 4th birthday. Should be completed through age 6.

19

If DTaP was given before age of 4 and the fifth vaccine was not given, what vaccine should you administer?

Trap starting at 7 years of age.

20

Vaccine given to children under 7 y.o. who have a contraindication to pertussis vaccine

DT

21

Tx of pertussis

Macrolide like azithromycin.

22

Tx usually given in a pt allergic to penicillin with strep pharyngitis

Azithromycin or other macrolide.

23

Atypical pneuma usually antibiotic class of choice

Azithromycin/macrolide.

24

Iron studies in a thalassemia patient

Normal to high ferritin
Normal to low TIBC

25

Labs in sickle cell

Low Hgb
Elevated reticulocyte count, as it is a hemolytic anemia.

26

Sideroblastic anemia : iron labs

Microcytic, incrased serum ferritin
TIBC saturation is normal or elevated.

27

An alternative to inhaled glucocorticoids as long-term asthma control agent

Montelukast, a leukotriene receptor antagonist

28

Kienbock's disease

AVN and collapse of the lunate so frequently encountered in individuals who overuse the wrist through repetitive compression loading.

29

Downey cells

MONO!

30

Metamyelocytes differentiate into?

Eosinophils
Basophils
Neutrophils