Insulin dependent diabetes in thalassaemia.

Diabetes mellitus was observed in 29 of 448 patients with thalassaemia major attending seven Italian centres. Twelve patients, at onset of clinical diabetes, presented with an asymptomatic glycosuria, 13 with ketosis, and four with ketoacidosis. All were diagnosed after 1979, at a mean age of 17 years. Mean age at diagnosis of diabetes was lower in patients born in the last two decades. In these patients transfusions were started at a younger age and pre-transfusion haemoglobin concentration, serum ferritin concentration, incidence of liver disease, and the presence of a family history of diabetes were higher than in patients born previously. Although 27 (93%) cases had iron chelating treatment the mean serum ferritin concentration was 5600 micrograms/l; 25 (92%) of these patients had signs of liver impairment. The determination of C peptide in 10 patients showed a wide variation in pancreatic beta cell function, and insulin requirements ranged between 0.15 and 1.72 U/kg body weight. Metabolic control was generally poor. The onset of diabetes mellitus was followed in most patients by the appearance of other endocrine or cardiac complications, or both. Fourteen patients died within three years of presenting with overt diabetes. Haemosiderosis, liver infections, and genetic factors seemed to be crucial in diabetes development. Thalassaemic patients developing clinical diabetes mellitus are at high risk for other complications and should be strictly monitored, especially for thyroid impairment.

Nutritional and technological characteristics of new broad bean flaked products.

The effects of the technological processes (soaking in water or alkaline solutions, drying, puree preparation) and the supplementation with maize flour on the nutritional value and on the organoleptic characteristics of broad bean (Vicia faba, L. major) flakes have been studied. Protein content was not affected by technological process. The addition of maize flour decreased the protein content of the final product depending on the amount of the maize flour added. Amino acid composition showed a decrease of tryptophan due to technological processing. Supplementation with maize flour improved the amino acid pattern and, except for tryptophan, the amount of essential amino acids in the flakes supplemented with 25% or more maize flour well compared with the provisional pattern by F.A.O. In vitro digestibility trials did not evidence significant changes due to technological processes or to integration of broad beans with maize flour. Broad bean toxic factors (vicine and convicine glycosides) were only slightly affected by the alkaline treatment of the flakes. Glycosides content decreased with the increasing supplementation with maize flour but the relationship was not linear. The organoleptic tests were positive for texture and taste, whereas the appearance of the products should be improved.

Height of children off therapy after acute lymphoblastic leukemia.

A total of 290 children off therapy after acute lymphoblastic leukemia, in continuous complete remission for at least 2 years, were evaluated for height at the onset of the disease and at the most recent clinical visit (median time after suspension of treatment 4 years 4 months, range 2 years-11 years 3 months). All patients had been treated with multidrug schedules; intrathecal drugs had been given to 84% of the patients for prevention of CNS involvement, associated with radiotherapy. The height percentiles at the most recent examination were shifted downward significantly compared with the expected pattern (p less than 0.001). The effect on stature was much more marked in girls, with a reduction of height percentiles at most recent examination from expected and from diagnosis; in males there was a reduction from diagnosis to latest follow-up, but the values were within the limits of normal. The short stature was mostly observed in pubertal girls and in patients who had undergone radiotherapy.