Whipple’s Disease .......................... - This rare condition is characterised by infiltration of small intestinal mucosa by “ foamy” macrophages which stain positive with periodic acid-Schif f(PAS) reagent. - The disease is multisystem one and almost any organ can be affected , sometimes long before gastrointestinal involvement becomes apparant - Electron microscopy reveals small Gram-positive bacilli (Tropheryma whippelli) within the macrophages. - Villi are widened and flattened; densely packed macrophages occur in the lamina propria. these may obstruct lymphatic drainage, causing fat malabsorbtion Clinical features of Whipple’s disease ..................................................... Gastrointestinal - Diarrhoea, steatorrhoea, weight los, bloating, protein-losing enteropathy, ascites, hepatosplenomegaly Musculoskeletal - Seronegative large joint arthropathy, sacroilitis Cardiac - Pericarditis(10%), myocarditis, endocarditis, coronary arteritis Neurological - Apathy, fits, dementia, myoclonus, meningitis, cranial nerve lesions Pulmonary - Chronic cough, pleurisy, pulmonary infiltrates Haematological - Anemia, lymphadenopathy Other - Fever, pigmentation Management - Fatal if untreated - Responds well initially to penicillin, tetracycline or sulphonamides - Symptoms resolve within a week and biopsy changes revert to normal in a few weeks. - Relapse occurs in up to one-third of patients often wihin the central nervous system, in which 2 weeks of parenteral Penicillin and co-trimoxazole followed by 6-12months of oral-co-trimoxazole are necessary. Ref: Davidson’s Principles and Practice of Medicine, 19th Edition, Pages 795-96
Posted on: Fri, 26 Jul 2013 09:43:48 +0000
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