title: von Hippel-Lindau syndrome presenting as pancreatic endocrine insufficiency: a case report.
abstract: The visceral manifestations of von Hippel-Lindau syndrome rarely are clinically significant until late in the disease process. Pancreatic endocrine insufficiency in the syndrome is extremely uncommon. We report a case of a 32-year-old woman with von Hippel-Lindau syndrome whose initial diagnosis came to light because of a clinical presentation with complications related to pancreatic endocrine insufficiency.
authors.affiliation: Department of Surgery University of Texas Health Science Center, San Antonio 78284-7842.
authors.forename: R K
authors.initials: RK
authors.lastname: Thompson
authors.forename: J I
authors.initials: JI
authors.lastname: Peters
authors.forename: K R
authors.initials: KR
authors.lastname: Sirinek
authors.forename: B A
authors.initials: BA
authors.lastname: Levine
mesh_terms: D000328:Adult; D000798:Angiomatosis / Q000150:complications*; D003131:Combined Modality Therapy; D010188:Exocrine Pancreatic Insufficiency / Q000175:diagnosis / Q000209:etiology* / Q000473:pathology / Q000628:therapy; D005260:Female; D006801:Humans; D010180:Pancreatectomy; D010643:Phenoxybenzamine / Q000627:therapeutic use; D006623:von Hippel-Lindau Disease / Q000150:complications* / Q000175:diagnosis / Q000473:pathology / Q000628:therapy
chemical_list: D010643:Phenoxybenzamine