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WISKOTT ALDRICH SYNDROME - A CASE REPORT

Journal: University Journal of Medicine and Medical Specialities (Vol.3, No. 1)

Publication Date:

Authors : ;

Page : 138-143

Keywords : :Wiskott-Aldrich syndrome; eczema; thrombocytopenia; pneumococcal meningitis;

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Abstract

Wiskott Aldrich syndrome is a rare X linked recessive disorder with a clinical triad of eczema, thrombocytopenia and recurrent infections. Due to a profound deficiency of T- and Blymphocyte function, infections are common in classic WAS and may involve all classes of microorganisms. These infections may include upper and lower respiratory infections such as otitis media, sinusitis and pneumonia. More severe infections such as systemic sepsis, meningitis and severe viral infections are less frequent. Immunologically, there is poor antibody response to polysaccharide antigens, progressive decrease in T cell number and function, and a characteristic immunoglobulin profile reflected in low IgM, elevated normal IgA, elevated IgE and normal low IgG levels. This is a unique and original case report of a patient, a case of Wiskott Aldrich syndrome to present with acute pneumococcal meningitis. Pneumococcal meningitis is one of the dreaded and life threatening manifestations of immunodeficiency in patients with Wiskott Aldrich syndrome. Complete recovery with prompt antibiotic treatment is possible inspite of the constraints posed by severe immunodeficiency

Last modified: 2017-02-23 18:16:31