To determine whether human X-linked neonatal diabetes mellitus, enteropathy and endocrinopathy syndrome (IPEX; MIM 304930) is the genetic equivalent of the scurfy (sf) mouse, we sequenced the human ortholog (FOXP3) of the gene mutated in scurfy mice (Foxp3), in IPEX patients. We found four non-polymorphic mutations. Each mutation affects the forkhead/winged-helix domain of the scurfin protein, indicating that the mutations may disrupt critical DNA interactions.

Newly diagnosed children appear to be protected from severe hypoglycemia. Rates increase with lower glycated hemoglobin, especially when mean HbA1c is < 8.0%. Younger children, who may be more susceptible to the adverse effects of neuroglycopenia, are at a particular risk of significant hypoglycemia.

Sleep impairs counterregulatory-hormone responses to hypoglycemia in patients with diabetes and normal subjects.

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Increased emphasis on strict glycaemic control of insulin dependent diabetes mellitus (IDDM) in young patients may be expected to cause increases in rates of significant hypoglycaemia. To evaluate whether this is the case for a large population based sample of IDDM children and adolescents rates of severe (coma, convulsion) and moderate (requiring assistance for treatment) hypoglycaemia were studied prospectively over a four year period.A total of 709 patients were studied yielding 2027 patient years of data (mean (SD) age: 12.3 (4.4); range 0-18 years, duration IDDM: 4.9 (3.8) years). Details of hypoglycaemia were recorded at clinic visits every three months when glycated haemoglobin (HbA1 c ) was also measured.Overall the incidence of severe hypoglycaemia was 7.8 and moderate was 15.4 episodes/100 patient years. Over the four years mean (SD) clinic HbA1 c steadily fell from 10.2 (1.6)% in 1992 to 8.8 (1.5)% in 1995. In parallel with this there was a dramatic increase in the rate of hypoglycaemia, especially in the fourth year of the study, when severe hypoglycaemia increased from 4.8 to 15.6 episodes/100 patient years. This increase was particularly marked in younger children (<6 years) in whom severe hypoglycaemia increased from 14.9 to 42.1 episodes/100 patient years in 1995.It is concluded that attempts to achieve improved metabolic control must be accompanied by eVorts to minimise the eVects of significant hypoglycaemia, particularly in the younger age group. (Arch Dis Child 1998;78:111-115) Keywords: hypoglycaemia; insulin dependent diabetes mellitus; glycaemic control Hypoglycaemia is the most common acute complication of the treatment of insulin dependent diabetes mellitus (IDDM) and its occurrence often restricts attempts to improve glycaemic control. This is particularly critical for younger patients who may be even more susceptible to severe hypoglycaemia 1 as a result of the interaction of both physiological and behavioural factors. A number of reports have described the incidence and clinical factors associated with significant hypoglycaemia in childhood but many of these have become outdated with recent changes to management goals and practices.2-5 For example, the greater emphasis on improved metabolic control and intensified treatment after the release of the Diabetes Control and Complications Trial (DCCT) results is likely to have altered the epidemiology of this acute complication of treatment. 6 We have recently reported results of a study performed over a three year period in which we found a high incidence of significant hypoglycaemia in children and adolescents. 7 Episodes were rare in the first year from diagnosis but were more frequent in children aged <6 years and in those with lower glycated haemoglobin (HbA1 c ). We now report a continuation of the same study, now completed over four years, and analysed to determine the relationship between glycaemic control and rates of hypoglycaemia. Although the association between lower HbA1 c and the incidence of severe hypoglycaemia h...