BRIEF REPORTS

Brief ReportsHypoinsulinaemia has an Important Role in theDevelopment of Oedema and HepatomegalyDuring Malnutrition

by Ashraf T. Soliman,* MD, Issa Alsalmi,** MD, and Maurice Asfour, MDDepartments of * Pediatrics and **Endocrinology, Royal Hospital, Muscat, Oman

SummaryVarious alterations in hormonal levels hare been suggested to contribute to the development ofnutritional oedema and fatty liver in children with kwashiorkor.1""4 We present an infant who underwentnear-total pancreatectomy at the age of 4 weeks and developed kwashiorkor after 11 weeks. Thesequence of events following surgery can be divided into two phases. The first phase was characterizedby hyperinsulinaemia and hypoglycaemia before feeds. Daring this phase, although the weight gain wasslow (10 g/day) serum albumin (32 g/1) and prealbumin (0.23 g/I) concentrations were maintained withno oedema or hepatomegaly. In the second phase, insulin deficiency prevailed and be was receiving thesame amount of milk (protein)/day (enriched with starch). During that phase he rapidly developedhypoalbnrainaemia (18 g/1), hypoprealbuminaemia (0.1 g/1), oedema, hepatomegaly, and derraatosis.This case demonstrates dearly the important role of defective insulin secretion in the development ofnutritional oedema and hepatomegaly.

Case ReportThis 3-month-old boy was born at 36 weeks ofgestation to a 35-year-old mother who had unevent-ful pregnancy. At birth he was large for date [birthweight (wt) = 3.65 kg], length = 51 cm, and headcircumference = 34 cm. He had mild respiratorydistress for 2 days. Six hours after birth he sufferedfrom non-ketotic hypoglycaemic seizure attack,when his blood glucose (BG) was 0.8 mmol, seruminsulin level (44 mIU/1) and C-peptide level (970pmol/I) were inappropriately high, documentinghyperinsulinaemia. Serum concentrations of free T4(17.6 pmol/1) and TSH (4.5 mlU/ml) were normal.During hypoglycaemia (BG=1.5 mmol/1) serumgrowth hormone (GH) (16.5 /ig/1) and cortisol(1167 nmol/1) concentrations were appropriate.Medical therapy including dextrose infusion 20 mg/kg/min, diazoxide 20 mg/day on two divided dosesand glucagon failed to bring up his BG to anacceptable range (2.6 mmol/1). Subtotal pancreatect-omy was performed at 4 weeks of age. During thefirst 4 weeks after surgery he was exclusively onbreastmilk (around 100 ml/kg/day). His BG levelswere in the normal range after each feed (3.5-6.7

Correspondence: Ashraf T. Soliman, Pediatrics Endocrinol-ogy, Royal Hospital, Seeb 1331, Muscat code 111, Oman.Fax: (968)591530.

mmol/1) and in the hypoglycaemic range before thenext feed (1.9-3.1 mmol/1). At BG = 2.9 mmol/1,serum C-peptide and insulin levels were 517 pmol/1and 5.6 MlU/ml, respectively, and 8-a.m. cortisollevel (815 nmol/1) was elevated. Serum albumin (32 g/1) and prealbumin (0.23 g/1) concentrations werewithin normal limits. His weight gain was slow (10 g/day). His mother was advised to breastfeed him morefrequently and to enrich the breastmilk with starch tomaintain his BG in the normal range. In addition,oral diazoxide was started at 5 mg twice daily. Hisglycaemic control improved with BG ranging be-tween 3.5 and 5.2 before feeds to 4.8-7.2 mmol/1after feeds. His liver was palpable 2 cm below thecostal margin and his weight on discharge = 4.05 kg.He was discharged home on the same treatment.After 1 week his BG increased significantly to 7.5-9.5mmol/1 and diazoxide was stopped; however, hisblood glucose remained in the high range 8.2-11.3mmol/1 denoting impaired insulin secretion. After 4weeks the baby became irritable, anorexic, anddeveloped skin changes on the neck and diaper area.On physical examination he appeared apathetic withpeevish cry, pale, with oedema of the hands and feet,kwashiorkor face, flaky dermatosis, and hepato-megaly (liver palpable 5 cm below the costal margin).Despite manifest oedema his weight was 3.8 kg.Sepsis work-up was negative. Serum electrolytes, andrenal and hepatic functions were normal. Serumconcentrations of albumin (18 g/I), prelabumin (0.1

Journal of Tropical Pediatrics Vol. 42 October 1996 Oxford University Press 1996 297

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BRIEF REPORTS

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