[Minkowski-Chauffard disease in 1 family].
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.
D000293:Adolescent; D000328:Adult; D000745:Anemia, Hemolytic, Congenital / Q000235:genetics* / Q000473:pathology / Q000601:surgery; D002648:Child; D005260:Female; D006801:Humans; D008297:Male; D010375:Pedigree; D013156:Splenectomy
Morbus Minkowski-Chauffard s opisanie na edna familiia.