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Arch Dis Child Fetal Neonatal Ed 1999;80:F209-F212 ( May )

Permanent neonatal diabetes mellitus: clinical presentation and epidemiology in Oman

Bhasker Bappal,a Palany Raghupathy,a Vasantha de Silva,b Saleh Mohamed Al Khusaibyc

a Paediatric Endocrinology, Royal Hospital, Muscat, Oman, b Department of Clinical Pathology, c Department of Neonatology

Correspondence to: Dr B Bhasker Paediatric Endocrinology and Metabolic Unit, Royal Hospital, PO Box 1331, SEEB, Postcode 111, Muscat, Sultanate of Oman.

Accepted 26 November 1998

AIM---To estimate the accurate incidence and prevalence of permanent neonatal diabetes mellitus, and to determine the clinical profile of this condition in the Sultanate of Oman.
METHODS---All children diagnosed as having permanent neonatal diabetes mellitus between 1991 and 1995 in Oman were included in the study.
RESULTS---The mean incidence was 2.2 per 100 000 live births/year and the prevalence among under 5s during 1995 was 2.0/100 000. Intrauterine growth retardation was noted in all (mean birthweight 1.86 kg), and diabetic ketoacidosis (mean plasma glucose 34.4 (SD 8.7) mmol/l, mean pH 7.17 (SD 0.09) in 80%. Hypertriglyceridaemia (mean serum triglyceride 19.06 (6.13) mmol/) was constant. No infant had clinical or immunological evidence of congenital viral infections. None had C-peptide excretion or circulating islet cell antibody during diagnosis or follow up. The other important features were parental consanguinity in all, HLA DR3/DR4 association in 80%, development of autoimmune hypothyroidism in one and observation of autoimmune disorders (insulin dependent diabetes mellitus and Hashimoto's thyroiditis) in family members.
CONCLUSIONS---These findings strongly suggest an immune mediated aetiology for diabetes mellitus. The reported incidence of permanent insulin dependent neonatal diabetes mellitus in Oman is the highest in the world.


Keywords: permanent neonatal insulin dependent diabetes mellitis; epidemiology; Oman


© 1999 by Archives of Disease in Childhood






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