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[Congenital hyperinsulinism of infancy: surgical treatment in 60 cases of focal form].
- Source :
-
Archives de pediatrie : organe officiel de la Societe francaise de pediatrie [Arch Pediatr] 2005 Mar; Vol. 12 (3), pp. 258-63. - Publication Year :
- 2005
-
Abstract
- Congenital hyperinsulinism of infancy is a severe disease that leads to important brain damage. Two different forms of the disease have been identified by pathologists: a diffuse and a focal form. A specific genetic anomaly identified in focal forms has never been described in diffuse ones. However, for most of authors, failure of medical treatment results in near-total pancreatectomy in all cases, which ends in diabetus. The aim of this retrospective study was to assess the results of elective partial pancreatectomy performed in 60 cases of focal form of hyperinsulinism over the last 18 years. Fifty-eight patients were cured with euglycemia at both fasting and hyperglycaemic tests without insulin-dependent diabetes mellitus. One patient is still in hypoglycaemia from unrecognized lesion; insulin-dependent diabetes mellitus occurred in one case nine years after surgery (a near-total pancreatectomy has been performed because of unknown focal form, in 1985).
- Subjects :
- Biopsy
Child
Child, Preschool
Congenital Hyperinsulinism classification
Congenital Hyperinsulinism diagnosis
Congenital Hyperinsulinism genetics
Congenital Hyperinsulinism pathology
Female
Follow-Up Studies
Humans
Infant
Infant, Newborn
Male
Mutation
Pancreas pathology
Postoperative Complications
Retrospective Studies
Time Factors
Treatment Outcome
Congenital Hyperinsulinism surgery
Pancreatectomy
Subjects
Details
- Language :
- French
- ISSN :
- 0929-693X
- Volume :
- 12
- Issue :
- 3
- Database :
- MEDLINE
- Journal :
- Archives de pediatrie : organe officiel de la Societe francaise de pediatrie
- Publication Type :
- Academic Journal
- Accession number :
- 15734120
- Full Text :
- https://doi.org/10.1016/j.arcped.2004.09.017