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- W2090960410 abstract "The WAS, an x-linked disorder with the triad of recurrent infections, eczema, and thrombocytopenia with hemorrhage, is a difficult management problem. 1/3 of WAS patients die of thrombo-cytopenia with catastrophic intracranial hemorrhage the principal threat. Because of the experience that splenectomized WAS patients died within a few months of overwhelming sepsis, splenectomy has been considered relatively contraindicated. 18 splenectomized WAS patients were evaluated for their response in terms of hemostatic improvement and subsequent clinical course. 17 of the 18 patients (94%) had elevations of their platelet counts (pc) to normal or near normal levels with control of bleeding. The mean pc rose from 19 to 249 × 103/mm3. Autologous platelet survival became normal post splenectomy in the one patient studied. 7 of the 18 patients are alive with a mean survival of 8 years post splenectomy. 7 of the 7 survivors are on prophylactic antibiotics and have been free of septic complications while on antibiotics. Of the 9 patients who have died, only one was or long term antibiotic coverage which had been stopped 10 days before the onset of his fatal septic episode. Splenectomy appears to offer a useful therapeutic modality for controlling hemorrhage and increasing survival in WAS when combined with the use of prophylactic antibiotics. The critical requirement for continuous antibiotic coverage, however, cannot be overemphasized when considering this form of therapy." @default.
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- W2090960410 date "1978-04-01" @default.
- W2090960410 modified "2023-10-18" @default.
- W2090960410 title "712 SPLENECTOMY AND PROPHYLACTIC ANTIBIOTICS IN THE MANAGEMENT OF THE WISKOTT-ALDRICH SYNDROME (WAS)" @default.
- W2090960410 doi "https://doi.org/10.1203/00006450-197804001-00717" @default.
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