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Peds Clerkship > Allergy/Imm + Heme/Onc > Flashcards

Allergy/Imm + Heme/Onc Flashcards

1
Q

DRESS syndrome:

A

(drug rash, eosinophilia, systemic sx)

erythema multiforme w/ eosinophilia + systemic sx, usually due to anticonvulsants

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2
Q

Maternal IgG passes to fetus via ___ and lasts ____. IgA passes via ___

A

IgG via placenta (lasts 3-6 months)

IgA via breast milk

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3
Q

Chronic granulmatous disease:
cause?
presentation?
dx?

A

XR ∆NADPH oxidase → phagocytic defect

recurrent abscesses

Dx nitroblue tetrazolium (NBT) test

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4
Q

Wiskott-Aldrich syndrome:
inheritance pattern?
presentation?
dx?

A

XR

thrombocytopenia (petechiae), recurrent infx, eczema

Dx CBC (↓plt), ↓IgM, ↑IgA, ↑IgE

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5
Q 
Bruton agammaglobulinemia: 
cause?
presentation?
dx?
tx?
A

XR B-cell defect → lack of all Ig types

recurrent bacterial infx (OM, PNA, etc.) after 6 months since mom’s Ig confers passive immunity

Dx ↓serum Ig levels

Tx repeated IVIG

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6
Q

T-cell defect:
cause?
dx?

A

likely DiGeorge syndrome (CATCH-22)

Dx intradermal Candida test

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7
Q

Combined variable immunodeficiency:
cause?
presentation?
a/w?

A

acquired B-cell defect (>10 y/o)

lack of all Ig types but normal B-cell levels

↑risk of lymphomas

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8
Q

SCID (“bubble boy disease”):
cause?
presentation?
tx?

A

XR or AR ∆adenosine deaminase → lymphocytes can’t make DNA to proliferate (**both B- and T-cells)

no T/B cells = severe infx → death at young age

Tx bone marrow txp

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9
Q

Job syndrome: presentation?

A

FATED – coarse Facies, Abscesses, retained primary Teeth, ↑↑IgE, Derm issues (eczema)

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10
Q

Asplenia:
presentation?
dx?

A

recurrent encapsulated bacterial infx (SHiN – Strep pneumo, H. influenza, Neisseria)
*common in SCD kids

Dx CBC w/ smear (Howell-Jolly bodies)

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11
Q

Hereditary angioedema:
cause?
presentation?

A

AD ∆C1 esterase inhibitor → random activation of complement pathway

recurrent episodes of edema w/ positive family hx

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12
Q

IgA deficiency:
presentation?
dx?

A

recurrent respiratory infx + diarrhea + anaphylatic transfusion rxns (reacts to donor serum IgA)

Dx ↓IgA

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13
Q

Complement deficiency:
cause/presentation?
dx?

A

C5-C9 deficiency predisposes to recurrent Neisseria infx

Dx CH50 test

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14
Q

Juvenile rheumatoid arthritis (Still disease):
type __ hypersensitivity
tx?

A

RF+ (anti-IgG Fc domain) → type 3 HS

Tx NSAIDs > MTX > PO steroids

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15
Q 
Polyarticular JRA =
Pauciarticular JRA=
    dx?
    tx?
Systemic JRA: presentation?
A

Polyarticular JRA: >5 joints

Pauciarticular JRA: 0-4 joints, can present w/ anterior uveitis (iridocyclitis)

    • Dx slit-lamp eye exam
    • Tx PO/OU steroids

Systemic JRA: systemic sx prior to arthritis, rheumatoid rash, high spiking fevers

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16
Q

Juvenile dermatomyositis:
presentation?
dx?

A

presents w/ progressive muscle weakness, skin rash, fatigue

Dx ↑CPK

17
Q

Neonatal lupus:

A
    • skin lesions (maternal ab-ag complexes cross placenta)

- - 3° heart block (anti-SSA attacks fetal heart conduction)

18
Q

Systemic lupus erythematosus (SLE):
type __ HS
dx?
tx?

A

3

Dx screen w/ ANA, confirm w/ anti-dsDNA or anti-Smith (also: has incr IgG)

Tx low-dose aspirin

19
Q

ITP:
presentation?
dx?
tx?

A

autoantibodies against gpIIb/IIIa → platelet sequestration in spleen → petechiae, purpura

Dx ↓plt/↑MKC

Tx RhoGAM (if Rh+), IVIG (if Rh-), steroids (2nd line), splenectomy (if severe)

20
Q

Fanconi anemia:
cause?
presentation?
tx?

A

AR ∆DNA repair genes → aplastic anemia + short stature, hypopigmented areas, eye/ear deformities

Tx androgens + hematopoietic drugs (EPO, neupogen, etc.)

21
Q

Anemia of prematurity:
dx?
tx?

A

MCC anemia in premature and SGA infants

Dx CBC shows profound anemia w/ ↓reticulocytes

Tx iron supplements

22
Q

Transient erythroblastopenia of childhood:
dx?
tx?

A

RBC aplasia in children following a viral infx

Dx CBC shows profound anemia w/ no reticulocytes

Tx supportive care (lasts 1-2 months, may need pRBCs)

23
Q

Blackfan-Diamond anemia: presentation?

A

macrocytic RBC aplasia + short stature, webbed neck, cleft lip, shield chest, triphalangeal thumbs

24
Q

MC organism?
Sickle cell + sepsis:
Sickle cell + osteomyelitis:
Sickle cell + aplastic crisis:

A

Sickle cell + sepsis: Strep pneumo

Sickle cell + osteomyelitis: Salmonella

Sickle cell + aplastic crisis: Parvo B19

25
Q

Sickle cell trait:
presentation?
dx?

A

MC sx is painless gross hematuria

Dx Hb electrophoresis shows HbS of 35-40%

26
Q

Barts disease
pathyphys?
presentation?
dx?

A

4 α-chain deletion → fetal hydrops

Dx post-mortem autopsy

27
Q 
HbH disease
pathyphys?
presentation?
dx?
tx?
A

3 α-chain deletion → severe anemia sx

Dx screen w/ CBC (microcytic anemia + ↓RDW), confirm w/ ↑HbH

Tx frequent transfusions

28
Q 
β-thalassemia major
pathyphys?
presentation?
dx?
tx?
A

homozygous ∆β-chain → severe anemia sx

Dx screen w/ CBC (microcytic anemia + ↓RDW), confirm w/ ↑HbF

Tx frequent transfusions


29
Q

β-thalassemia minor
pathyphys?
presentation?
dx?

A

heterozygous ∆β-chain → mild anemia sx

Dx screen w/ CBC (microcytic anemia + ↓RDW), confirm w/ ↑HbA2

Tx reassurance

30
Q

Kasabach-Merritt phenomenon:
pathyphys?
tx?

A

large vascular anomalies (kaposiform hemangioendothelioma, tufted angioma) sequester RBCs, platelets, and clotting factors → anemia, thrombocytopenia, coagulopathy

Tx steroids + IFN-α

31
Q

ALL:
pathophys?
Dx?
Tx?

ALL prognosis: good? poor?

A

pre-B-cell proliferation (CALLA+/TdT+) → infiltration of RES (LAD, splenomegaly) + BM (pancytopenia)

Dx bone marrow bx shows >25% lymphoblasts

Tx chemotherapy

good px w/ age 1-10, hyperdiploidy, WBC <50, and TEL/AML1 on t(12;21)

poor px w/ Philadelphia chromosome on t(9;22)

32
Q

Hodgkin lymphoma:
presentation?
dx?
tx?

A

B-cell proliferation into Reed-Sternberg cells → B-type sx (fever, weight loss, night sweats)

Dx CXR to look for mediastinal mass
then LN bx

Tx chemotherapy

33
Q

Langerhans cell histiocytosis: presentation?

A

Langerhans cell proliferation → eosinophilic granuloma → painful, solitary lytic bone lesion (MC site is skull)

34
Q

Hereditary spherocytosis:
dx?
tx?

A

AR ∆spectrin → hemolytic anemia + jaundice in newborn

Dx CBC shows spherocytes + abnormal osmotic fragility test

Tx splenectomy

35
Q

Von Willebrand disease:
dx?
tx?

A

AD ∆vWF → MCC hemorrhagic diathesis

Dx vWF levels + activity (ristocetin cofactor activity)

Tx DDAVP

36
Q

Tumor lysis syndrome:
labs?
dx?
tx?

A

lysis of cancer cells → hyperkalemia + hyperuricemia + renal failure

Dx renal panel + ↑uric acid

Tx allopurinol

37
Q

Cause of Dactylitis in Sickle Cell:

Acute chest syndrome in SC:
presentation?
tx?

A

sickle cell anemia → vaso-occlusion → vascular necrosis of metacarpals/metatarsals → acute onset painful swelling of hands and feet

PNA-like presentation
Dx CXR shows infiltrate
Tx azithromycin + ceftriaxone

Peds Clerkship (13 decks)

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