先天性高胰岛素血症非典型形式的外科治疗

The surgical management of atypical forms of congenital hyperinsulinism.

作者信息

Capito Carmen, de Lonlay Pascale, Verkarre Virginie, Jaubert Francis, Rahier Jacques, Nihoul-Fékété Claire, Aigrain Yves

机构信息

Department of Pediatric Surgery, AP-HP Hôpital Necker-Enfants Malades, Université Paris Descartes, Paris, France.

出版信息

Semin Pediatr Surg. 2011 Feb;20(1):54-5. doi: 10.1053/j.sempedsurg.2010.10.003.

DOI:10.1053/j.sempedsurg.2010.10.003

PMID:21186006

Abstract

Beyond the 2 classical forms of congenital hyperinsulinism, focal and diffuse, we report our experience on the surgical treatment of atypical forms. We define 2 subtypes among these atypical forms of hyperinsulinism: in case of a giant focal form the surgical strategy is the same as in focal forms. In case of hyperinsulinism caused by a mosaic, our experience suggests the benefit of a limited resection from the tail to the body of the pancreas.

摘要

除了先天性高胰岛素血症的两种经典形式，即局灶性和弥漫性之外，我们报告了我们对非典型形式手术治疗的经验。我们在这些非典型高胰岛素血症形式中定义了两种亚型：对于巨大局灶性形式，手术策略与局灶性形式相同。对于由嵌合体引起的高胰岛素血症，我们的经验表明从胰尾到胰体进行有限切除是有益的。