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Peds Boards > AI > Flashcards

AI Flashcards

1
Q

CVID

A

Failure to differentiate to plasma B cells

  • panhypogammaglobulinemia
  • chronic pulm infections, bronchiectasis
  • recurrent giardia, diarrhea/malabsorption, steatorrhea, protein losing enteropathy
  • noncaseating granulomas and presents as HSM
  • chronic enteroviral meningitis

Frequent association with autoimmune diseases
Lymphoma incidence is increased

Tx: IVIG

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2
Q

Selective IgA deficiency puts you at risk for

A

Ulcerative Colitis
Celiac
Autoimmune diseases: Hashimoto’s, T1DM

Recurrent Giardia - mucosal secretory IgA within GI tract is missing

Recurrent sinus infections.

Anaphylaxis to blood products or IVIG.

Though most are asymptomatic

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3
Q

Hereditary Angioedema is associated with

A

Cut/Resp - Skin swelling of lips, tongue, soft palate, larynx
GI - abd pain, distension, v/d

Both types have DECREASED C4
Type I - C1 inhibitor is low and fxnally low
Type II - C1 inhibitor is nl/elevated but low fxn

Tx: Danazol - increases levels of C1 esterase inhibitor

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4
Q

SLE + recurrent pyogenic infections = what complement deficiency?

A

C2 deficiency.

- increased susceptibility to S pneumo, Neisseria, Haemophilus

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5
Q

Risk of fulminant meningococcal disease

A

C8/terminal complement deficiency

Properdin deficiency

  • X-linked.
  • Properdin, an important complement factor responsible for the stabilization of the alternative C3 convertase, is deficient
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6
Q

HyperIgE syndrome/Job syndrome

A

IgE: 2k to 50k

  • recurrent staph abscess (S aureus)
  • sinopulm infections (S pneumo, H flu)
  • eczematous rash
  • eos

Patho: Neutrophils fail to adhere to the endothelium and enter the tissues. Secretion of chemotactic chemokines and interleukins (e.g., IL-5 and IL-11) bring eosinophils to the tissues, with secondary stimulation of IgE production and the severe atopic picture of eczema. However, the primary neutrophil problem leads to deep abscesses, primarily with Staphylococcus aureus.

Abscesses are usually “COLD” aka lacks inflammation

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7
Q

Wiskott Aldrich - X-linked

A

thrombocytopenia and SMALL platelets
eczema
immunodef - susceptability to bacteria/opportunistic infections

BOYS because X-linked

Low IgM and high IgA

Tx: antibiotic and IVIG for acute infections, possible splenectomy, HSCT if severe

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8
Q

Chronic Granulomatous Disease

A
  • skin infections
  • pneumonia
  • episodes of lymphadenitis cause by S. aureus usually
  • fungal infections and granuloma formation - esp Aspergillus

genetic defect - that prevents phagocytes from producing superoxides and killing organisms.

Dx: Dihydrorhodamine oxidation tests (DHR test) using flow cytometry OR nitroblue tetrazolium NBT test

Usually X-linked but can be AR

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9
Q

Chediak Higashi

A

Defective lysosome degranulation and NK cell fxn

  • recurrent infections of mucous membranes, skin, resp tract
  • partial oculocutaneous albinism (silver hair, fair skin)
  • progressive sensory or motor neuropathy = ataxia
  • photophobia
  • impaired plt aggregation and bleeding
    ==> lymphoma like syndrome

Dx: large granules in the neutrophils, which interfere with chemotaxis, degranulation, and bactericidal activity

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10
Q

Leukocyte Adhesion Deficiency

A
  • high leukocyte/neutrophil count (bc of underlying defect in migration and adherence)
  • delayed umbilical cord stump separation
  • severe gingivitis
  • recurrent bacterial and fungal infections of skin (without the swelling, erythema, warmth, pus at the site)
  • FTT
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11
Q

DiGeorge - CATCH 22

A 
Cardiac abnormality (commonly interrupted aortic arch, truncus arteriosus and tetralogy of Fallot)
Abnormal facies
Thymic aplasia
Cleft palate
Hypocalcemia/hypoparathyroidism
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12
Q

CHARGE

A 
Coloboma of retina
Heart abnormalities (aortic arch)
Atresia of the chonae
Retarded growth
Genital hypoplasia
Ear anomalies
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13
Q

Hyper-IgM

A

Hyper-IgM = x linked disease with mutation that encodes for a ligand on T cells. Problem of T-cell activation and subsequent failure of immunoglobulin isotype switching, leading to high IgM and low IgG and IgA. These patients have severe T-cell deficiency.

Sinopulm infections, diarrhea
Opportunistic - At risk for PCP PNA

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14
Q

Bloom Syndrome

A

Defect in the DNA repair machinery of the cell, leading to genomic instability and increased sensitivity to DNA-damaging agents.

Patients also have reduced numbers of T cells.

Congenital Telangiectatic Erythema

  • Telangiectasias, Photosensitivity***
  • Chelitis, growth deficiency due to primary hypogonadism
  • increased susceptibility to neoplasm
  • “bird like” actions
  • abnormal facies
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15
Q

Apert Syndrome

A
  • Craniosynostosis,

- Syndactyly (webbing of fingers and toes)

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16
Q

X-linked agammaglobulinemia

A
  • defect in the maturation of early B cells into mature B cells, resulting in the absence of plasma cells.
  • healthy until around 4–6 months of age, at which time their transplacentally acquired IgG declines. They then begin having recurrent serious infections with encapsulated and enteroviral infections.

They have normal T-cell immunity.

Diagnosis is made by serum quantitative immunoglobulins.

Treatment consists of antibiotics, no live-virus vaccines, and monthly IV immunoglobulin (IVIG; 300–400 mg/kg/month).

17
Q

What vaccine if given to a household member would pose a risk to an immunocompromised household member?

A

Oral polio vaccine

18
Q

DRESS

A

Drug rash with eosinophilia and systemic symptoms.
May be associated with HHV6 reactivation

  • antiseizure meds, sulfa drugs
  • 2-12 wks after exposure**

Facial edema + erythematous papules. Then caudal spread and development of vesicular and bullous lesions.

Spares mucous membranes unlike SJS and TEN.

Affects organ systems - liver, kidneys, heart, thyroid.

+ eosinophilia
+ atypical lymphocytosis
+ elevated serum transaminases
+ elevated creatinine levels.

Tx: steroids

19
Q

Linear IgA Bullous Dermatosis

A

idiopathic OR drug induced subepidermal blistering disorder

Tx: Dapsone

20
Q

Timing of Drug reactions

A

Serum sickness: 1-2wks up to 3wks
SJS/TEN: 1-4 wks
DRESS: 2-12 wks

21
Q

What drug can induce symptoms of mastocytosis?

A

Dextromethorphan

22
Q

Serum Sickness

A

Type 3 hypersensitivity - IC
Does NOT require prior exposure

Fever
Rash - urticarial wheals, angioedema
Joint pain
Lymphadenopathy
Muscle aches
Proteinuria 
GI symptoms - diarrhea, vomiting

Last 7-10 days and resolve spontaneously.

23
Q

Does anaphylaxis to eggs require special precautions in regards to influenza vaccine?

A

No - give it without special precautions.

24
Q

Chronic Urticaria is associated with…

A

Thyroid disease!
- Initial eval should include TFTS

Chronic urticaria is defined as the presence of hives, with or without angioedema, for a period of 6 weeks or longer.

Peds Boards (26 decks)

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