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Flashcards in Pediatrics Deck (59)
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1

6+ month child with recurrent sinopulmonary and GI infections, small lymph nodes
Diagnosis and treatment?

Bruton's X- linked gammaglobulinemia
low B cells, no Ig
Scheduled IVIG, no live vax (other vax don't give good protection)

2

Teen with recurrent sinopulmonary and GI infections, small lymph nodes
Diagnosis and treatment?

Combined variable immunodeficiency
-mild XLA
2 out of 3 Ig forms absent
Scheduled IVIG

3

Previously health child with anaphylactic reaction after blood transfusion
Dx, Tx?

IgA Deficiency
May also have recurrent sinopulmonary and GI infections

4

Bruton's X- linked gammaglobulinemia presentation, why?

6+ month child with recurrent sinopulmonary and GI infections, small lymph nodes
low B cells, no Ig

5

Combined variable immunodeficiency presentation

Teen with recurrent sinopulmonary and GI infections, chronic pulmonary disease

6

Chronic Granulomatous Disease Pathology and lab values

No respiratory burst, Adensoine deaminase deficiency
Macs eat but dont kill
Skin abcesses and pulm infections
Catalase + bugs
Inc WBC, IgM and IgG
Nitro blue positive

7

DiGeorge Syndrome presentation

thymic hypoplasia, low T cells
recurrent PCP or fungal infections
3rd pharyngeal pouch malformations
Cardiac defects
Hypocalcemia

8

Wiscott-Aldrich presentation

Boys (x linked)
Eczema, low platelets, normal infections
low WBC, plts
Inc IgA and IgE
Need BM transplant

9

Infant with delayed separation of the umbilical cord

Leukocyte adhesion deficiency
Toxic vital signs but no pus
get biopsy

10

Why do tet spells occur and how to help?

Tet spell: RVOT obstruction, deox blood goes into aorta instead of lungs
-cres-decres systolic ejection murmur, single S2 sound

Knee to Chest (squatting): increases SVR to help push blood into the lungs and reduce hypoxia/cyanosis

11

Xray finding: pelvis or long bone, lamellated periosteal reaction/ moth-eaten/ onion skinning

Ewing Sarcoma

12

Xray finding: soap bubble appearance in the epiphysis of a long bone

Giant cell tumor

13

Methemoglobinemia presentation and labs

cyanosis after exposure to dapsone/nitrites/anesthetics/oxidizing agents
Pulse ox is low 85%ish but PaO2 is normal

-oxidized Hb wont let go of the O2

14

Methemoglobinemia work up and treatment

co-oximetry to measure Hb v. Methemoglobinemia levels
Tx: Methylene Blue or high dose Ascorbic acid (vitC)

15

Thalassemia minor presentation

microcytic anemia
nl RDW
compensatory retic count elevation due to bone marrow response

16

Treatment of rheumatic heart disease

benzathine penicillin G IM q4weeks to prevent recurrence and progression
may also need cardiac monitoring for mitral valve issues

17

Transient tachypnea of the newborn presentation and pathology

tachypnea, clear lung sounds, intercostal retractions, nasal flaring
CXR: inc lung vol, fluid in interlobar fissures
premature or c section
delayed reabsorption of alveolar fluid 2/2 dec hormonal signals that would have been released during labor

18

Cyanosis in the first 24 hours, single S2, narrow mediastinum (egg on a string)
No improvement with O2
Diagnosis, next step?

Transposition of Great Vessels
give prostaglandins to keep PDA open
get an echo
Surgery

No murmur=ASD
murmur= VSD or PDA

19

Kawasaki Disease present and pathophysiology

acute vasculitis of small and medium arteries, persistent release of pro-inflammatory cytokines
-FEVER: 5+ days, unresponsive to tx
-BL non exudative CONJUCTIVITIS
-polymorphous RASH
-erythema, edema, desquam of hands and feet
-Cervical LAD, 1.5+ cm

20

Labs of Kawasaki Disease

Inc ESR, CRP
hypoalbuminemia
sterile pyuria
leukocytosis, anemia, thrombocytosis

21

Complications of Kawasaki Disease

Coronary artery aneurysms
MI

22

Primary amenorrhea ages

13 with no secondary characteristics
15 with secondary characteristics

23

Most common cause of neonatal sepsis

GBS

24

Presentation of absence seizures

<20 sec
don't respond to voice or touch
presence of automatisms

25

Why are pts with sickle cell at inc risk for pneumococcal infections?

Splenic autoinfarction occurs at an early age
inc risk of infection with encapsulated organisms

26

Signs that point to iron deficiency anemia

heavy menses, decreased erythrocytes. thrombocytosis
*need to r/o or tx IDA before Hb electrophoresis because it may mask a thalassemia*

27

Fanconi Anemia presentation and pathophysiology

Defective DNA repair
Bone Marrow Failure-Aplastic Anemia
short, thumb abnormalities, abnormal skin pigmentation

28

<1 infant with hypotonia, constipation, irritability, oculobulbar weakness (absent gag reflex, ptosis), hyporeflexia

Botulism

29

batteries, magnets, sharp objects in esophagus or symptomatic
vs.
small blunt, non toxic, radiopaque object

immediate removal with flexible endoscopy

observation, repeat Xray at 24 hours

30

Toxo
vs.
CMV

macrocephaly and parenchymal calcifications

microcephaly and periventricular calcifications