Monday 22 August 2016

DiGeorge Syndrome | Crack PGIMER NOV 2016


Which is found in DiGeorge Syndrome?

A. Tetany 
B. Eczema 
C. Mucocutaneous candidiasis 
D. Absebnt B and Tcells
E. Total absence of T cells



The Answer is . (A) Tetany; (C) Mucocntaneous candidiasis
• Di George’s syndrome is a developmental defect involving the endodermal derivative of 3rd and 4th pharyngeal pouches, whih leads to aplasia or hypoplasia of the thymus and parathyroid glands
• Circulating T-cells are reduced
• Tetany (hypocalcemic) can occur.
• Humoral immunity is largelyunaffected.
• Low immunity against viral and fungal infection ; mucocutaneous candidiasis occurs.
• Congential cardiac anomalies (Fallot’s tetralogv) including great vessels of heart, facial deformities are prominent.
• Eczema is seen in wiskot-Aidrich syndrome.
• The individuals have a small but histologically normal thymus allowing most patients to develop functional T-cells

in number that may or may not be adequate for host defense.