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Wolfram syndrome is an autosomal recessive disorder with probable locus heterogeneity. Only insulin-dependent diabetes mellitus and progressive optic-nerve atrophy are necessary to make the diagnosis, but associated findings include diabetes insipidus, sensorineural hearing loss, ataxia, peripheral neuropathy, urinary-tract atony, and psychiatric illnesses. We performed clinical and molecular studies on four consanguineous families with 16 affected individuals. We point out a new phenotypic variant with absent diabetes insipidus, presence of peptic ulcer disease and bleeding tendency secondary to a platelet aggregation defect. The same phenotypic variant turned out to be a genotypic variant with linkage to a second Wolfram syndrome locus (WFS2) on chromosome 4q22-24., (Copyright 2002 Wiley-Liss, Inc.)

An attempt was made by the Jordanian National Center for Diabetes, Endocrine and Genetic Diseases (NCDEGD) to identify all cases of type 1 diabetes among Jordanian children aged 0-14 y. Data were obtained retrospectively for the years 1992-1994 and prospectively for the years 1995 and 1996, including full name, national identifying number, date of birth, date of diagnosis and family history. The incidence was calculated as the number of cases per 100,000 population, according to the national census of 1994. The incidence rate for these years (1992 through 1996) was 2.8, 2.9, 3.2, 3.6 and 3.6 per 100,000 population, respectively. The male:female ratio was (1:1.03). Seasonal variation at clinical onset was noticed, with maximum incidence in the winter months and minimum incidence in the summer months. In conclusion, the incidence of type 1 diabetes mellitus in Jordanian children aged 0-14 y is among the lowest in the region, but is rising.