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논문 기본 정보
- 자료유형
- 학술저널
- 저자정보
- 저널정보
- 대한소아내분비학회 Annals of Pediatirc Endocrinology & Metabolism Annals of Pediatirc Endocrinology & Metabolism 제22권 제1호
- 발행연도
- 2017.1
- 수록면
- 60 - 64 (5page)
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초록· 키워드
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Hemochromatosis is an inherited or secondary disorder caused by excessive iron storage leading to multiple organ damage. We describe 2 patients with diabetes mellitus caused by hemochromatosis secondary to multiple blood transfusions due to severe aplastic anemia. Subject 1, who was diagnosed with severe aplastic anemia at 15 years of age, received multiple red blood cell transfusions before he underwent autologous peripheral blood stem cell transplantation (PBSCT) at 22 years of age. At 21 years of age, hyperglycemia was detected with increased hemoglobin A1c and serum ferritin levels, 9.7% and 12,910 ng/mL (normal range, 20–320 ng/mL), respectively. The 24-hour urine C-peptide level was normal with negative antiglutamic acid decarboxylase antibody. Subsequently, metformin and an iron-chelating agent were administered. However, an intensive insulin regimen was necessary 2 years after the onset of diabetes. Subject 2, who was diagnosed with severe aplastic anemia at 2 years of age, received multiple blood transfusions until she underwent haploidentical PBSCT at 13 years of age. At 11 years of age, she developed diabetes mellitus with a high serum ferritin level (12,559.8 ng/mL). She is currently 18 years old and has been treated with an intensive insulin regimen and estrogen/progesterone replacement therapy because of hypogonadotropic hypogonadism. It is presumed that the loss of insulin secretory capacity and insulin resistance played a role in the pathogenesis of diabetes mellitus due to hemochromatosis in these cases.
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참고문헌 (20)
참고문헌 신청
Aregbesola A / 2013 / Body iron stores and the risk of type 2 diabetes in middle-aged men / Eur J Endocrinol 169 : 247 ~ 253
Burt MJ / 1996 / Haemochromatosis: a clinical update / Med J Aust 164 : 348 ~ 351
Cario H / 2010 / Guidelines for diagnosis and treatment of secondary iron overload in patients with congenital anemia / Klin Padiatr 222 : 399 ~ 406
Creighton Mitchell T / 2014 / Diabetes and hemochromatosis / Curr Diab Rep 14 : 488 ~
European Association For The Study Of The Liver / 2010 / EASL clinical practice guidelines for HFE hemochromatosis / J Hepatol 53 : 3 ~ 22
Burt MJ / 1996 / Haemochromatosis: a clinical update / Med J Aust 164 : 348 ~ 351
Cario H / 2010 / Guidelines for diagnosis and treatment of secondary iron overload in patients with congenital anemia / Klin Padiatr 222 : 399 ~ 406
Creighton Mitchell T / 2014 / Diabetes and hemochromatosis / Curr Diab Rep 14 : 488 ~
European Association For The Study Of The Liver / 2010 / EASL clinical practice guidelines for HFE hemochromatosis / J Hepatol 53 : 3 ~ 22
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