ESPE Abstracts

Background: A neonate, born at 34 weeks gestation by caesarean section for foetal distress, was severely growth retarded at birth. Deranged liver functions were noted at birth with (alanine transaminase (ALT), aspartate transamninase (AST) and gamma-glutamyl transfererase (GGT) recorded as 102, 228 and 1078 U/l respectively. The GGT rose to a peak of 3877 U/l at 6 months of age. The clinical course of the neonate was associated with failure to thrive and intermittent hyperglycaemia (although keto-acidosis was not observed early in the phase of the disease). The insulin and C-peptide levels were <0.5 and <0.1 U/l respectively. A trial of glibencamide failed to control the hyperglycaemia. Investigations for inborn errors of metabolism were normal.

Objective and hypotheses: It has been postulated that insulin deficiency in utero contributes to growth retardation. Wide fluctuations of the blood glucose and poor weight gain characterized the post natal course. As there was scanty subcutaneous tissue, continuous intravenous insulin infusion was instituted resulting in better weight gain.

Method: A review of the clinical case record. Permission was obtained from the parent to use the clinical records for presentation.

Results: A liver biopsy revealed marked glycogen deposition.

Conclusion: The association of neonatal diabetes with growth retardation suggests that insulin deficiency in utero may have contributed to the growth retardation. Although glycogen deposition in the liver may occur with insulin deficiency, the reasons for the markedly elevated liver enzymes are not fully understood.