Permanent neonatal diabetes mellitus: clinical presentation and epidemiology in Oman
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.
METHODSAll children
diagnosed as having permanent neonatal diabetes mellitus between 1991 and 1995 in Oman were included in the study.
RESULTSThe 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.
CONCLUSIONSThese
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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