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PLoS One. 2014 May 19;9(5):e98054. doi: 10.1371/journal.pone.0098054. eCollection 2014.

Pancreatic endocrine and exocrine function in children following near-total pancreatectomy for diffuse congenital hyperinsulinism.

Author information

1
Department of Paediatric Endocrinology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, United Kingdom; Institute of Child Health, University College London, London, United Kingdom.
2
Department of Paediatric Endocrinology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, United Kingdom; Institute of Child Health, University College London, London, United Kingdom; Departments of Paediatric Endocrinology, Ankara Pediatric Hematology and Oncology Training Hospital, Ankara, Turkey.
3
Department of Paediatric Endocrinology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, United Kingdom.
4
Institute of Biomedical and Clinical Science, University of Exeter Medical School, Exeter, United Kingdom.

Abstract

CONTEXT:

Congenital hyperinsulinism (CHI), the commonest cause of persistent hypoglycaemia, has two main histological subtypes: diffuse and focal. Diffuse CHI, if medically unresponsive, is managed with near-total pancreatectomy. Post-pancreatectomy, in addition to persistent hypoglycaemia, there is a very high risk of diabetes mellitus and pancreatic exocrine insufficiency.

SETTING:

International referral centre for the management of CHI.

PATIENTS:

Medically unresponsive diffuse CHI patients managed with near-total pancreatectomy between 1994 and 2012.

INTERVENTION:

Near-total pancreatectomy.

MAIN OUTCOME MEASURES:

Persistent hypoglycaemia post near-total pancreatectomy, insulin-dependent diabetes mellitus, clinical and biochemical (faecal elastase 1) pancreatic exocrine insufficiency.

RESULTS:

Of more than 300 patients with CHI managed during this time period, 45 children had medically unresponsive diffuse disease and were managed with near-total pancreatectomy. After near-total pancreatectomy, 60% of children had persistent hypoglycaemia requiring medical interventions. The incidence of insulin dependent diabetes mellitus was 96% at 11 years after surgery. Thirty-two patients (72%) had biochemical evidence of severe pancreatic exocrine insufficiency (Faecal elastase 1<100 µg/g). Clinical exocrine insufficiency was observed in 22 (49%) patients. No statistically significant difference in weight and height standard deviation score (SDS) was found between untreated subclinical pancreatic exocrine insufficiency patients and treated clinical pancreatic exocrine insufficiency patients.

CONCLUSIONS:

The outcome of diffuse CHI patients after near-total pancreatectomy is very unsatisfactory. The incidence of persistent hypoglycaemia and insulin-dependent diabetes mellitus is very high. The presence of clinical rather than biochemical pancreatic exocrine insufficiency should inform decisions about pancreatic enzyme supplementation.

PMID:
24840042
PMCID:
PMC4026387
DOI:
10.1371/journal.pone.0098054
[Indexed for MEDLINE]
Free PMC Article

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