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Z Uworld Step 2 > Pediatrics > Flashcards

Pediatrics Flashcards

1
Q

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

A

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

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2
Q

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

A

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

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3
Q

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

A

IgA Deficiency

May also have recurrent sinopulmonary and GI infections

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4
Q

Bruton’s X- linked gammaglobulinemia presentation, why?

A

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

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5
Q

Combined variable immunodeficiency presentation

A

Teen with recurrent sinopulmonary and GI infections, chronic pulmonary disease

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6
Q

Chronic Granulomatous Disease Pathology and lab values

A 
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
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7
Q

DiGeorge Syndrome presentation

A 
thymic hypoplasia, low T cells
recurrent PCP or fungal infections
3rd pharyngeal pouch malformations
Cardiac defects
Hypocalcemia
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8
Q

Wiscott-Aldrich presentation

A 
Boys (x linked)
Eczema, low platelets, normal infections
low WBC, plts
Inc IgA and IgE
Need BM transplant
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9
Q

Infant with delayed separation of the umbilical cord

A

Leukocyte adhesion deficiency
Toxic vital signs but no pus
get biopsy

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10
Q

Why do tet spells occur and how to help?

A

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

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11
Q

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

A

Ewing Sarcoma

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12
Q

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

A

Giant cell tumor

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13
Q

Methemoglobinemia presentation and labs

A

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

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14
Q

Methemoglobinemia work up and treatment

A

co-oximetry to measure Hb v. Methemoglobinemia levels

Tx: Methylene Blue or high dose Ascorbic acid (vitC)

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15
Q

Thalassemia minor presentation

A

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

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16
Q

Treatment of rheumatic heart disease

A

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

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17
Q

Transient tachypnea of the newborn presentation and pathology

A

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

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18
Q

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

A

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

No murmur=ASD
murmur= VSD or PDA

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19
Q

Kawasaki Disease present and pathophysiology

A

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
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20
Q

Labs of Kawasaki Disease

A

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

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21
Q

Complications of Kawasaki Disease

A

Coronary artery aneurysms

MI

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22
Q

Primary amenorrhea ages

A

13 with no secondary characteristics

15 with secondary characteristics

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23
Q

Most common cause of neonatal sepsis

A

GBS

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24
Q

Presentation of absence seizures

A

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

25
Q

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

A

Splenic autoinfarction occurs at an early age

inc risk of infection with encapsulated organisms

26
Q

Signs that point to iron deficiency anemia

A

heavy menses, decreased erythrocytes. thrombocytosis

need to r/o or tx IDA before Hb electrophoresis because it may mask a thalassemia

27
Q

Fanconi Anemia presentation and pathophysiology

A

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

28
Q

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

A

Botulism

29
Q

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

A

immediate removal with flexible endoscopy

observation, repeat Xray at 24 hours

30
Q

Toxo
vs.
CMV

A

macrocephaly and parenchymal calcifications

microcephaly and periventricular calcifications

31
Q

First step in precocious puberty work up

A

Measure Bone Age

32
Q

Most common pneumonia pathogen in CF pts
Child
vs.
Adult

A

Staph Aureus

Pseudomonas

33
Q

Complications of chronic giardiasis

A

Lactose intolerance, weight loss, vitamin deficiency

34
Q

Enzyme and mechanism impaired in Von Gierke Disease

A

Glucose 6 phosphatase deficiency

Impaired glycogen to glucose conversion

35
Q

Enzyme deficiency and impaired mechanism in Galactosemia

A

Galactose-1-phosphate uridyl transferase deficiency

Cant break down galactose

36
Q

12 month milestones

Gross/fine motor, language, social

A 
Stands, first steps, throw a ball
Pincer grasp
Handful of words
Separation anxiety
Follows 1 step commands
37
Q

18 month milestones

Gross/fine motor, language, social

A

Run, kick a ball
2-4 tower cube, remove clothes
10-25 words, 1 body part
Understands mine, pretend play

38
Q

2 year milestones

Gross/fine motor, language, social

A

Stairs with same feet, jump
6 cube tower, copy a line
50+ words, 2 word phrases
2 step commands, parallel play, toilet training

39
Q

3 year milestones

Gross/fine motor, language, social

A

Stairs alternating feet, tricycle
Copies circle, uses utensils
3 word sentences, 75% understandable
Age/gender, imaginative play

40
Q

4 year milestones

Gross/fine motor, language, social

A

1 foot balance/hop
Copies a cross
Colors, 100% understandable
Cooperative play

41
Q

5 year milestones

Gross/fine motor, language, social

A

Skips, catch a ball
Copies a square, ties shoes, dress/bathe, write letters
Count to 10, 5 word sentences
Friends, completes toilet training

42
Q

Screening in beckwidth-wiedemann syndrome patients

A

Abd US and alpha fetoprotein
Inc risk of wilms tumor or hepatoblastoma

  • Abd US/AFP q3 mo from age 1-4
  • Abd US q3 mo from 4-8
  • Renal US q3? 8-teen
43
Q

Test needed for Kawasaki patients

A

Echo to look for coronary artery aneurysms

44
Q

Hyposthenuria

What is it? Who gets it?

A

Inability of kidneys to concentrate urine
Sickle cell trait/disease
Polyuria, low urine spec grav, nl Na

45
Q

Lesch-Nyhan Syndrome Deficiency

A

hypoxanthine-guanine phosphoribosyltransferase deficiency

Self mutilation, gouty arthritis

46
Q

Hereditary spherocytosis triad and test

A

Coombs neg anemia, jaundice, splenomegaly

Inc retics, hyperbili, Eosin-5-maleimide binding, acidified glycerol lysis tests

47
Q

Alport syndrome pathophys, presentation, biopsy findings

A

IV collagen defective in GBM, cochlea, eye
-Boy <10, gross hematuria after URI, fam hx of hearing loss, CKD

GBM- lamellated due to longitudinal splitting of lamina densa
Normal compliment levels

48
Q

Treatment for otitis externa

A

Topical fluoroquinolone (cipro) +/- topical steroid

49
Q

MCC pediatric stroke

A

Sickle Cell Disease

50
Q

Precocious puberty, irregular (one-sided) cafe au lait macules, polyostotic fibrous dysplasia (recurrent fractures)
Disease?

A

McCune-Albright Syndrome

51
Q

Angular cheilitis, stomatitis, glossitis, normocytic normochromic anemia, seb dermatitis
Vit deficiency?

A

Vit B2 riboflavin

52
Q

Additional Vaccinces for HbSS

A

PPSV 23 and MCV 4 at 2 yrs old

53
Q

fever, AMS, focal seizure, dx? Work up?

A

encephalitis

MRI

54
Q

Barium enema shows narrowed distal colon with proximal dilation, finding? dx?

A

transition point

Hirschsprungs disease- get rectal biopsy

55
Q

palpable purpura, thrombocytopenia, arthralgia/arthritis, abdominal pain, renal disease, dx?

A

Henoch-schonlein purpura

56
Q

Presentation of HUS, why?

A

Diarrhea cause by E. Coli O157 H7

  • Shiga toxin damages endothelial cells in renal glomeruli
  • platelets are activated and form microthrombi that shear RBCs

Normocytic anemia, thrombocytopenia, edema after a diarrheal illness

57
Q

MCC of CAH

A

21-hydroxylase deficiency

-Elevated 17-hydroxyprogesterone

58
Q

Congenital syphilis findings

A

Rhinorrhea, abnl long bone Xrays, desquamating/bullous rash

59
Q

Congenital Rubella findings

A

Cataracts, Heart issues like PDA

Z Uworld Step 2 (1 decks)

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