Document Detail


Surgical management of islet cell dysmaturation syndrome in young children.
MedLine Citation:
PMID:  2218837     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Islet cell dysmaturation syndrome (ICDS) encompasses the causes of infantile hyperinsulinemic hypoglycemia histologically described as islet cell hyperplasia, pancreatic adenomatosis and nesidioblastosis. Eleven infants underwent 14 pancreatic resections for ICDS from 1965 to 1990 at the University of California at Los Angeles Medical Center for severe hypoglycemia unresponsive to medical therapy. Seizures were the presenting symptoms of hypoglycemia in eight infants. Six patients had nesidioblastosis, four had islet cell hyperplasia and one patient had an adenoma with histologically normal pancreatic islet cells. Four neonates underwent 80 per cent pancreatic resection; three with nesidioblastosis required reoperation (90 to 95 per cent resection). Four older infants underwent 80 per cent pancreatic resection but required diazoxide for less than six months postoperatively. Three infants underwent 90 to 95 per cent pancreatic resection. None have required reoperation or postoperative medications. All infants are normoglycemic without pancreatic exocrine insufficiency and none had postoperative complications. Five infants had preoperative neurologic impairment, with three having severe retardation; all showed some improvement postoperatively, but only one infant now has normal findings on neurologic examination. Early diagnosis and aggressive surgical resection should minimize neurologic complications of the ICDS.
Authors:
H S Bjerke; R E Kelly; M E Geffner; E W Fonkalsrud
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Surgery, gynecology & obstetrics     Volume:  171     ISSN:  0039-6087     ISO Abbreviation:  Surg Gynecol Obstet     Publication Date:  1990 Oct 
Date Detail:
Created Date:  1990-11-01     Completed Date:  1990-11-01     Revised Date:  2009-11-11    
Medline Journal Info:
Nlm Unique ID:  0101370     Medline TA:  Surg Gynecol Obstet     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  321-5     Citation Subset:  AIM; IM    
Affiliation:
Division of Pediatric Surgery, University of California, Los Angeles Center for Health Sciences.
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MeSH Terms
Descriptor/Qualifier:
Adenoma / complications,  pathology,  surgery
Combined Modality Therapy
Diazoxide / therapeutic use
Humans
Hyperinsulinism / complications
Hyperplasia / complications,  pathology,  surgery
Hypoglycemia / etiology,  pathology,  surgery
Infant
Infant, Newborn
Islets of Langerhans*
Pancreatectomy
Pancreatic Diseases / complications,  pathology,  surgery*
Pancreatic Neoplasms / complications,  pathology,  surgery
Reoperation
Retrospective Studies
Syndrome
Chemical
Reg. No./Substance:
364-98-7/Diazoxide

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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