Insulin dependent XXXX in thalassaemia.

@entity1 @entity6 @entity409 @entity10200 @entity1270 @entity299 @entity68 @entity1374
@entity6 was observed in 29 of 448 @entity1 with thalassaemia major attending seven Italian centres. Twelve @entity1 , at onset of clinical @entity6 , presented with an asymptomatic @entity10200 , 13 with @entity1270 , and four with @entity1270 . All were diagnosed after 1979, at a mean age of 17 years. Mean age at diagnosis of @entity6 was lower in @entity1 born in the last two decades. In these @entity1 transfusions were started at a younger age and pre-transfusion haemoglobin concentration, serum ferritin concentration, incidence of @entity409 , and the presence of a family history of @entity6 were higher than in @entity1 born previously. Although 27 (93%) cases had @entity68 chelating treatment the mean serum ferritin concentration was 5600 micrograms/l; 25 (92%) of these @entity1 had signs of @entity409 . The determination of C peptide in 10 @entity1 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 @entity6 was followed in most @entity1 by the appearance of other @entity299 , or both. Fourteen @entity1 died within three years of presenting with overt @entity6 . Haemosiderosis, @entity409 , and genetic factors seemed to be crucial in @entity6 development. Thalassaemic @entity1 developing clinical @entity6 are at high risk for other complications and should be strictly monitored, especially for @entity1374 .
