Causes of death of patients in an institution for the developmentally disabled

The causes of death of 53 severely to profoundly developmentally disabled patients who died in an intermediate care facility were reviewed. Respiratory disease, predominantly pneumonia and aspiration, accounted for 72% of deaths. Seven patients died of nonrespiratory causes, and in 8 patients, no cause of death could be determined, even after a complete autopsy or investigation. The median age at death was 20 years. The weights of these patients' organs at autopsy were lower than those for normal individuals of the same age. The lifespan of these severely impaired individuals continues to be significantly shortened, even with improved methods of care.     

Hyperplasia of islets of langerhans and low serum insulin in cot deaths

Of the fifty-seven cases of cot death studied two-thirds were younger than 3 months, which is also the peak age of infantile hypoglycaemia. Findings from routine necropsy and histology were scarce; in eleven cases they could be regarded as potentially fatal. About half of the infants had had a mild virus-type infection approximately one week before death. Special attention was paid to endocrine pancreas. Insulitis or lymphocytes in the septa were discovered in twelve cases. Hyperplasia of the islets of Langerhans was a common observation; the hyperplasia being either nesidioblastosis-like with clusters of islets around ducti, or diffuse. The average proportion of islet tissue in the whole pancreas parenchyma was around 5% in infants aged 1–6 months, the percentage being significantly greater than in age-matched controls (4.3%). The pancreatic insulin content was also higher in the cot death cases. Serum insulin values were low (mean 4.8 ± 1.2 μU/ml) in cot deaths; in the controls they were twice as high (mean 11.6 ± 1.6 μU/ml) (p < 0.005). The cause of death in this group of cot deaths could thus be (congenital?) hyperplasia of the islets, possibly combined with a lesion in the B-cells caused by a virus. The mechanism of death would be hypoglycaemia.