A check up of literature has been made concerning the congenital microspherocytic hemolytic anemia, reporting of some new data about the pathogenesis of the disease. One family is described with CMHA--one father and his two daughters, the two children of his first daughter and the only child of the second daughter, hence confirming the familial character of the disease. All patients have been splenectomized. After the splenectomy they are practically healthy, without hemolytic crises, supporting the statement that splenectomy is the treatment of choice in CMHA therapy.
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The article presents an observation of a 63-year-old female patient suffering from arterial hypertension, coronary artery disease complicated by a persistent form of atrial fibrillation during 1.5 months, and hereditary Minkowski-Chauffard hemolytic anemia. Treatment with cordaron proved to be ineffective, while oral administration of quinidin in a dose of 1 gr (0.
View Article and Find Full Text PDFMinkowski-Chauffard's disease keeps on being primarily a subject for hematologists, but surgeons play a definite role in its treatment, if we take into consideration the encouraging effect of splenectomy. Experience is recorded gained at the Department of Abdominal Surgery of the Research Institute of Gastroenterology and Hematology with 34 patients for a period of 35 years, 13 of them during the last 18 years. A patient is presented with a characteristic clinical pattern, associated with calculosis of the common bile duct, a very rare observation in this disease.
View Article and Find Full Text PDFChir Pediatr
May 1990
Service Chirurgie Pédiatrique, Hôtel-Dieu, Clermont-Ferrand.
Since 1983, the authors have performed a surgical ligation of the splenic artery in 7 patients suffering hypersplenism due to hematological diseases (4 Minkowski-Chauffard, 2 Betathalassemia Major, 1 Idiopathic Thrombocytopenic purpura). The immediate post operative course and long-term outcome of the patients were evaluated on clinical and biological grounds. The early post operative period was not influenced by the underlying hematological disorder.
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