Image Based Questions On Immune System

Pneumatoceles in CXR 

Staph aureus Hyper lgE Syndrome - AD inheritance, recurrent abscesses, Staph aureus, IgE > 2000 IU /ml.

Wiskott- Aldrich Syndrome 

WASP gene on Chromosome X · Triad RET- Recurrent infections, Eczema (skin rash), Immunodeficiency probably, Thrombocytopenia with small sized platelets.

Ataxia Telangiectasia 

Defect in ATM gene (Chromosome 11),  Cerebellar ataxia · Oculocutaneous telangiectasia · Recurrent Sinopulmonary infections, Defective DNA repair · ↑ed risk of lymphoreticular malignancies a Adenocarcinoma.

Digeorge Syndrome 

Microdeletion of Chr 22q11 · Short Philtrum. Mandibular Hypoplasia · Dysmorphism, Hypotelorism with antimongoloid slant · Prominent bridge of nose · Absent thymic shadow on mediastinal X-Ray.

Mnemonic-  CATCH 22 

C- Conotruncal abnormalies A - Abnormal face T -Thymic hypoplasia C --- cleft palate H - Hypocalcemia - due to hyperparathyroidism, 22 - Chr 22q11microdeleon.

Nitroblue Tetrazolium Test (NBT) 

Screening test for Chronic granulomatous disease.

Chronic Granulomatous disease (CGD) · Confirmatory Test → DHR (Dihydrorhodamine assay by Fluid cytometry).

No Blue granules (CGD). Macrophages with bluish granules present. They are able to reduce the Colorless NBT dye (Normal).

Henoch Schoenlein Purpura (HSP) 

Palpable purpura with 1 or more of o Diffuse abdominal pain,  Arthritis or arthralgia,  Renal involvement,  Any biopsy lgA deposition.

Coronary Artery Aneurysms 

Coronary artery aneurysms [Giant > 8mm] seen on coronary angiography · DOC for Kawasaki disease: Iv lg.

Juvenile Dermatomyositis 

Goron papules · Flat topped · Erythematous or violaceous papules or plaques over bony prominences  E.g., MCP, DIP, PIP joints. Heliotrope Rash · Bluish - violet discoloration of eyelids ± periorbital edema.

Also Read: KEY POINTS AND RECOMMENDATIONS IN PEDIATRIC ADVANCED LIFE SUPPORT

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