XXXX in adults: a challenging cause of organic hyperinsulinism.

@entity1 @entity11448 @entity11449 @entity9608 @entity5 @entity1373 @entity11243 @entity751 @entity460
BACKGROUND: @entity11448 in adults has been reintroduced into the differential diagnosis of organic hyperinsulinism by the description of 'noninsulinoma @entity751 (NIPHS)'. MATERIALS AND METHODS: Pathologic specimens of all adult @entity1 (n = 66) operated on for organic hyperinsulinism were re-examined. Five @entity1 fulfilled the histomorphological criteria of @entity11448 . Retrospective review of clinical presentation, results of 72-h fasts, intravenous @entity11243 tolerance tests, pre- and intraoperative localization studies and surgical therapy was performed. RESULTS: In contrast to NIPHS, fasting tests became positive after 8-14 h. @entity11243 tests were positive and preoperative imaging showed negative results in all @entity1 . At first operation distal pancreatic resections were performed in three @entity1 , resection of the pancreatic body in one @entity1 and biopsy of the pancreatic tail in one @entity1 . Two of three @entity1 with recurrent disease had to be reoperated. One @entity1 showed a coexistence of @entity11448 and @entity9608 and is part of a kindred with autosomal dominantly inherited 'familial islet-cell @entity11449 '. CONCLUSIONS: Surgical exploration is indicated only after thorough biochemical diagnosis. An aggressive strategy for preoperative localization including selective arterial @entity460 stimulation testing seems justified. There may be a combination of @entity11448 and @entity5 . A link between beta-cell @entity1373 and progression to @entity9608 based on not yet known genetic causes can be suspected.
