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A Case of Diabetes Mellitus due to Hemochromatosis in Patient with CPRCA

Journal of the Korean Pediatric Society 1995;38(2):280-284.
Published online February 15, 1995.
A Case of Diabetes Mellitus due to Hemochromatosis in Patient with CPRCA
Eun Hee Kim, Yong Hwa, Ho Sik, Sung Wom Kim
Department of Pediatrics, ST. Benedict Hospital, Pusan, Korea
선천성 순 적혈구 빈혈 환아에서 혈색소증에서 병발된 이차성 당뇨병 1례
김은희, 신용화, 안호식, 김성원
부산 성분도병원 소아과
Iron overload from repeated blood transfusions in patients with hematologic problems is a problem of clinical significance, because eventually it can lead to secondary hemochromatosis with diabetes. So it is important to diagnose iron overload early and to restrict blood transfusions in these patients. Recently, we experienced one case of secondary hemochromatosis with diabetes in patient wi th congenital pure red cell anemia, diagnosed by serum ferritin level, TIBC, transferrin saturation, random blood sugar, C-peptide and insulin level. This patient had received 30,000ml of RBCs for 9 years. Treatments with insulin mixed spitting therapy because of insulin dependent diabetes clinically, restriction of blood transfusion and steroid therapy for maintenance of Hemoglobin level were done just after discharge. We report this one case with a brief review of the literature.
Key Words: Diabetes Mellitus, Hemochromatosis, Congenital Pure Red Cell Anemia

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