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Growth and endocrine function after near total pancreatectomy for hyperinsulinaemic hypoglycaemia
A T Soliman1, I Alsalmi, A Darwish
1Department of Paediatrics, Royal Hospital, Muscat, Oman.
Insights
Children undergoing near-total pancreatectomy for persistent hyperinsulinaemic hypoglycaemia of infancy (PHHI) experience significant growth retardation and impaired insulin secretion. Despite normal developmental milestones, they show low insulin-like growth factor-I (IGF-I) levels and defective linear growth.
Area of Science:
- Pediatric Endocrinology
- Metabolic Disorders
- Surgical Outcomes
Background:
- Persistent hyperinsulinaemic hypoglycaemia of infancy (PHHI) is a rare cause of severe hypoglycemia in newborns.
- Near-total pancreatectomy is a treatment option for PHHI when medical management fails.
- Infants undergoing this extensive surgery often present with macrosomia and birth complications.
Purpose of the Study:
- To evaluate the long-term growth, endocrine function, and developmental outcomes in children who underwent near-total pancreatectomy for PHHI.
- To investigate the relationship between impaired insulin secretion and growth factors post-surgery.
- To assess exocrine pancreatic function and overall development in this cohort.
Main Methods:
- Retrospective study of seven children who underwent near-total pancreatectomy (95-98%) for PHHI.
- Assessment of growth parameters (height, weight, growth velocity), hormonal levels (growth hormone, IGF-I), and glucose metabolism (oral glucose tolerance, C-peptide response).
- Evaluation of exocrine pancreatic function through fecal fat content and serum albumin levels.
Main Results:
- Children exhibited significant growth retardation, with height and weight below the 5th centile and low growth velocity.
- Impaired insulin secretion was evident, with decreased C-peptide response to glucagon and significantly low IGF-I concentrations.
- Despite normal growth hormone response, linear growth was defective; three developed diabetes, and two had impaired glucose tolerance.
Conclusions:
- Near-total pancreatectomy for PHHI leads to significant growth retardation and impaired insulin secretion, independent of normal growth hormone response.
- Low IGF-I production may be linked to defective insulin secretion, impacting linear growth.
- While exocrine function is generally preserved, long-term endocrine complications like diabetes are a concern.
Abstract:
Seven children, with a mean (SD) age of 4.6 (2.1) years, who as infants (21 (7.5) days) underwent near total (95-98%) pancreatectomy for persistent hyperinsulinaemic hypoglycaemia of infancy (PHHI) were studied. At birth all the infants were macrosomic. Four infants had been born after a difficult labour, of whom three had moderate birth asphyxia and respiratory distress. All had normal thyroid function. After surgery transient hyperglycaemia was manifest in six of the children and required insulin treatment for 5.8 (3.8) weeks, and transient hypoglycaemia was encountered in one child and responded well to increased carbohydrate intake and diazoxide for three weeks. Six of the children rapidly crossed down their length and weight centiles during the first year after surgery. At the end of the first year these children were at or below the 5th centile of height and weight for their age and gender. After a period of 4.6 (2.1) years, their mean (SD) height score was -2.57 (0.5), growth velocity 3.9 (0.75) cm/year, and growth velocity SD score -2.1 (0.55)l these were significantly low and denoted significant growth retardation. The growth hormone peak responses to provocation with clonidine were normal (13.5 (2.8) micrograms/l). However, the circulating insulin-like growth factor-I (IGF-I) concentrations were significantly decreased (79 (34) ng/ml). Three of the children developed diabetes at two and a half, five, and seven years after surgery, two others had impaired oral glucose tolerance and six out of the seven children had an impaired C peptide response to glucagon. Defective insulin secretion in these children might directly inhibit IGF-I synthesis in the liver. The body mass index of the pancreatectomised children was 14.9 (0.5) and was normal for age and gender; they had a normal 72 hour faecal fat content and normal serum albumin concentration. These data indicated grossly adequate exocrine pancreatic function. It appears that children requiring near total pancreatectomy for PHHI have normal developmental milestones but defective linear growth with impaired insulin secretion and low IGF-I production despite normal growth hormone response to provocation.