小児遺伝性球状赤血球症の外科治療

概要

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During the 17-year period, 1968 to 1984, eighteen children underwent splenectomy for hereditary spherocytosis (HS) at our department. There were 9 males and 9 females. Patient's ages ranged 3 years to 12 years (average 6 years). Eleven patients (61%) had a history of familial transmission of HS. Anemia and/or jaundice were the most common presenting symptoms. Splenomegaly was noted in 16 (89%) of patients at time of initial physical examinations. The clinical diagnosis of HS were confirmed in all prtients by the presence of spherocytes in the peripheral blood, an elevated serum bilirubin level, reticulocytosis, a negative result of Coombs' test, as well as a positive result of the osmotic fragility test. Accessory spleen was found in 10 (56%) of the patients, Splenectomy and cholecystectomy were performed simultaneously in one patient who had a preoperative sonographic diagnosis of cholelithiasis. All 18 patients improved following splenectomy. Ther was no case of postsplenectomy sepsis (overwhelming postsplenectomy infection) in our series.
日本小児外科学会の論文
1986-08-20