The role of research in improving medical education and health care.

In the last decades, the role of research carried out in the universities, in the medical schools in particular, has been lessened. This was due to changes that occurred within the universities and to the development of several new institutions and organizations specifically devoted to scientific research. Therefore, it is stressed that research at the university level is an extremely important tool in medical education to train the students in view of their medical profession.

Effects of Amanita phalloides toxins on insulin release: in vivo and in vitro studies.

The clinical picture of Amanita phalloides poisoning includes hypoglycaemia, usually related to hepatic damage. In fact, Amanita toxins induce hepatic glycogen depletion in humans and animals. However, in animals morphological changes of pancreatic beta cells are reported, suggesting that an alteration of insulin secretion might be involved in the pathogenesis of hypoglycaemia. Therefore, we determined fasting glucose, insulin and C-peptide levels in ten patients intoxicated by Amanita phalloides and in ten control subjects. Fasting blood samples were drawn on 3 consecutive days, beginning 48-72 h after mushroom ingestion, and glucose, insulin and C-peptide concentrations were determined by routine methods. Serum glucose concentrations did not differ between poisoned subjects and controls, whereas insulin and C-peptide concentrations were significantly higher in poisoned subjects ( P<0.01), with a significant positive correlation ( R=0.97, P<0.001). We also evaluated the effects of alpha-amanitin, the main amatoxin, on in vitro insulin release. Rat islets were incubated with 5 and 50 mg/l alpha-amanitin, in the presence or absence of 5.6 mM glucose. In another protocol, islets were preincubated for 2 h with 5 and 50 mg/l alpha-amanitin in medium containing 5.6 mM glucose. After lavage, islets were incubated with increasing glucose (2.8-22.0 mM) to evaluate insulin release. In vitro, both concentrations of toxin induced insulin release (5 mg/l P<0.02, 50 mg/l P<0.01 vs controls), in the presence of 5.6 mM glucose. Islets preincubated with 5 mg/l alpha-amanitin showed a pattern of glucose-stimulated insulin release similar to controls, whereas islets preincubated with 50 mg/l alpha-amanitin showed an increased basal release with a reduced response to glucose stimulation. These observations show that Amanita toxins might play a role in the clinical context of Amanita poisoning. We demonstrate, for the first time, that alpha-amanitin induces insulin release and may exert a cytotoxic effect on beta cells.

[Growth hormone deficiency in the adult: only an endocrinologic problem?]. / Il deficit di ormone della crescita nell'adulto: un problema solo endocrinologico?

In the literature published during the last decade an increased risk of death due to cerebrovascular and cardiovascular events in growth hormone deficient adults has been reported. A partial reversibility of the syndrome following recombinant growth hormone treatment has also been described. Both these factors have contributed to the proposal of growth hormone therapy not only for children but also for adults. Following the initial enthusiasm, the scientific community is now evaluating various clinical experiences held over recent years and weighing up the results. Present day medicine has to take the economic impact of prescribed therapeutic regimens into consideration; in other words the ratio between cost and benefits must be calculated. The relatively recent issuance of the license for the treatment of growth hormone deficiency in adults using recombinant growth hormone does not allow us to evaluate a possible reduction in the risk of death due to cerebrovascular and cardiovascular events in treated subjects. A much longer observational period will be required. Besides the partial reversibility of the syndrome as a consequence of treatment, it is necessary to single out the selection criteria for the choice of treatment. These could also be useful as indicators of the efficacy of the same treatment.

The Alström syndrome: is it a rare or unknown disease?

The Alström syndrome is a rare, autosomal recessive disorder characterized by retinal degeneration, obesity, progressive hearing impairment, non-insulin-dependent diabetes mellitus and kidney and heart failure. Mental retardation is absent and the extremities are normal. The Alström syndrome gene located on chromosome 2, has been recently identified. The Alström syndrome involves multiple organ systems with a complex interaction between pathways. Phenotypic expression varies considerably, even within sibships. Manifestations observed in some, but not all, Alström syndrome patients include acanthosis nigricans, alopecia, short stature, scoliosis, kyphosis, hyperostosis frontalis interna, muscle dystonia, advanced bone age and subcapsular cataract. Other metabolic and endocrinological abnormalities have been described: hypothyroidism, hypogonadism, diabetes insipidus, growth hormone deficiency, hyperuricemia and hyperlipidemia. In the final stages of the disease, affected individuals exhibit progressive chronic nephropathy with eventual kidney failure. The most frequent causes of death include hepatic dysfunction and congestive heart failure secondary to dilated cardiomyopathy. We have summarized our personal clinical data and the information from the scientific literature on the topic in order to provide an up-to-date review on the Alström syndrome.