Thompson R K, Peters J I, Sirinek K R, Levine B A
Department of Surgery University of Texas Health Science Center, San Antonio 78284-7842.
Surgery. 1989 May;105(5):598-604.
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.
冯·希佩尔-林道综合征的内脏表现很少在疾病进程晚期之前具有临床意义。该综合征中的胰腺内分泌功能不全极为罕见。我们报告一例32岁患有冯·希佩尔-林道综合征的女性病例，其最初诊断是由于出现了与胰腺内分泌功能不全相关的并发症的临床表现才得以明确。
