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- W2725487152 abstract "Hermansky Pudlak Syndrome is a rare autosomal recessive disorder which results in oculocutaneous albinism, bleeding problems due to functional platelet abnormality and systemic complications like IBD and pulmonary fibrosis. Presence of platelet functional abnormality leads to spontaneous rectal bleeding in IBD patients with HPS which makes scoring of disease activity difficult. Patients with HPS also have associated vWF deficiency. Bleeding manifestations in these patients can be successfully addressed with Desmopressin. We report a rare case of this syndrome with Crohns colitis which was successfully treated with Desmopressin and Azathioprine. Keyword :IBD-Inflammatory bowel disease, HPS- Hermansky Pudlak Syndrome, vWF -Von Willi Brand Factor, CDAI - Crohns Disease Activity Index" @default.
- W2725487152 created "2017-07-14" @default.
- W2725487152 creator A5078131824 @default.
- W2725487152 date "2016-11-23" @default.
- W2725487152 modified "2023-09-28" @default.
- W2725487152 title "AN INTERESTING CASE OF REFRACTORY IBD - HERMANSKY PUDLAK SYNDROME" @default.
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- W2725487152 hasPublicationYear "2016" @default.
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