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
- 저널정보
- 대한소아내분비학회 Annals of Pediatirc Endocrinology & Metabolism Annals of Pediatirc Endocrinology & Metabolism 제22권 제4호
- 발행연도
- 2017.1
- 수록면
- 272 - 275 (4page)
이용수
초록· 키워드
오류제보하기
Management of congenital hyperinsulinemia of infancy (CHI) is challenging. A 4-month-old female infant with persistent hypoglycemia and elevated insulin levels was diagnosed with CHI. Gallium-68 DOTANOC positron emission tomography/computed tomography (PET/CT) scan (68Ga-labeled [1,4,7,10-tetraazacyclododecane-N,N’,N’’,N’’’-tetraacetic acid]-1-NaI3-octreotide) demonstrated focal disease in the body of the pancreas. Genetic studies indicated paternal inheritance, making focal disease likely. She was started on diazoxide therapy with partial improvement in blood glucose levels. Due to a suboptimal response to diazoxide and the likelihood of focal disease amenable to surgery, a laparoscopic subtotal pancreatectomy with preservation of the head of the pancreas was performed. The biopsy demonstrated diffuse hyperplastic pancreatic islet cells on immunohistochemistry, indicative of diffuse rather than focal disease. Paternal inheritance is a recognized indicator of focal disease. Gallium-68 DOTANOC PET/CT scan is the only available imaging modality in South India as 18F-L-dihydroxyphenylalanine (DOPA) PET/CT scan is not available at present. A laparoscopic approach reduces the postoperative recovery time and morbidity in such patients. The absence of 18F-L-DOPA PET/CT scan and the limited supply of diazoxide makes the management of this complex condition more challenging in developing countries.
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등록된 정보가 없습니다.
참고문헌 (17)
참고문헌 신청
Al-Shanafey S / 2009 / Laparoscopic pancreatectomy for persistent hyperinsulinemic hypoglycemia of infancy / J Pediatr Surg 44 : 134 ~ 138
Banerjee I / 2011 / The contribution of rapid KATP channel gene mutation analysis to the clinical management of children with congenital hyperinsulinism / Eur J Endocrinol 1
Beltrand J / 2012 / Glucose metabolism in 105 children and adolescents after pancreatectomy for congenital hyperinsulinism / Diabetes Care 35 : 198 ~ 203
Chandran S / 2013 / Paternally inherited ABCC8 mutation causing diffuse congenital hyperinsulinism / Endocrinol Diabetes Metab Case Rep 2013 : 130041 ~
Desai MP / 1998 / Persistent hyperinsulinemic hypoglycemia of infancy / Indian Pediatr 35 : 317 ~ 328
Banerjee I / 2011 / The contribution of rapid KATP channel gene mutation analysis to the clinical management of children with congenital hyperinsulinism / Eur J Endocrinol 1
Beltrand J / 2012 / Glucose metabolism in 105 children and adolescents after pancreatectomy for congenital hyperinsulinism / Diabetes Care 35 : 198 ~ 203
Chandran S / 2013 / Paternally inherited ABCC8 mutation causing diffuse congenital hyperinsulinism / Endocrinol Diabetes Metab Case Rep 2013 : 130041 ~
Desai MP / 1998 / Persistent hyperinsulinemic hypoglycemia of infancy / Indian Pediatr 35 : 317 ~ 328
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