지원사업
학술연구/단체지원/교육 등 연구자 활동을 지속하도록 DBpia가 지원하고 있어요.
커뮤니티
연구자들이 자신의 연구와 전문성을 널리 알리고, 새로운 협력의 기회를 만들 수 있는 네트워킹 공간이에요.
논문 기본 정보
- 자료유형
- 학술저널
- 저자정보
- 발행연도
- 2017.1
- 수록면
- 495 - 498 (4page)
이용수
초록· 키워드
오류제보하기
Thalassemia major is a genetic disorder with a defective synthesis of either the alpha or the beta chain of hemoglobin A. Blood transfusion is crucial for the survival in these patients. Unfortunately, endocrine dysfunction is a very common complication in these patients and is principally due to excessive iron overload as a result of frequent blood transfusions. Although regular blood transfusion may increase life expectancy, disturbances in growth and pubertal development, abnormal gonadal functions, impaired thyroid, parathyroid and adrenal functions, diabetes, and disorderly bone growth are common side effects. We hereby present a case of a 23-year-old, unmarried woman with beta thalassemia major presenting with primary amenorrhea, poor development of secondary sexual character, and short stature. Thorough history, clinical examination, and laboratory investigation, including dynamic function test (insulin tolerance test) were conducted. These tests confirmed that she had multiple endocrinopathies, including hypogonadotropic hypogonadism, growth hormone deficiency, and subclinical adrenal insufficiency, which were caused by iron overload. She required hormone replacement therapy.
Early recognition of possible deficiencies in hypothalamo-pituitary-end organ hormones caused by iron overload in thalassemia patients that undergo frequent blood transfusion procedures is essential. Appropriate treatments, including transfusion regimen and chelation therapy, as well as specific treatment of each complication are the crucial for the successful management and improvement of quality of life these patients.
목차
등록된 정보가 없습니다.
참고문헌 (12)
참고문헌 신청
Al-Elq AH / 2004 / Endocrinopathies in patients with thalassemia / Saudi Med J 25 (10) : 1347 ~ 1351
Alireza Abdollah Shamshirsaz / 2003 / Metabolic and endocrinologic complications in beta-thalassemia major: a multicenter study in Tehran / BMC Endocrine Disorders 3 (1) : 4
Ang AL / 2014 / History of myocardial iron loading is a strong risk factor for diabetes mellitus and hypogonadism in adult with beta thalassemia major / Eur J Haematol 92 (3)
De P / 2014 / A review of endocrine disorders in thalassaemia / Open J Endocr Metab Dis 4 (2) : 25 ~ 34
De Sanctis V / 2013 / Growth and endocrine disorders in thalassemia:The international network on endocrine complications in thalassemia (I-CET) position statement and guidel
Alireza Abdollah Shamshirsaz / 2003 / Metabolic and endocrinologic complications in beta-thalassemia major: a multicenter study in Tehran / BMC Endocrine Disorders 3 (1) : 4
Ang AL / 2014 / History of myocardial iron loading is a strong risk factor for diabetes mellitus and hypogonadism in adult with beta thalassemia major / Eur J Haematol 92 (3)
De P / 2014 / A review of endocrine disorders in thalassaemia / Open J Endocr Metab Dis 4 (2) : 25 ~ 34
De Sanctis V / 2013 / Growth and endocrine disorders in thalassemia:The international network on endocrine complications in thalassemia (I-CET) position statement and guidel
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