Subject(s)
Program Development , Psychology, Adolescent , Adolescent , Emotions , Family Characteristics , Feeding Behavior , Humans , Parent-Child Relations , Sex Education , Sex FactorsABSTRACT
Short stature and short trunk have been reported in thalassaemic patients. We report a study on stature and body proportions in 476 patients (2-36 years old) with beta-thalassaemia major, followed in 12 Italian centres. Auxological data (standing height, sitting height, subischial leg length, target height), haematological data (age at first transfusion, age at start of desferrioxamine [DFX] chelation, mean dose of DFX, ferritin values) and information regarding the presence of endocrine disorders and of bone lesions, were collected and analysed according to the age of the patients, in order to investigate the natural history of the disproportion and the role of siderosis, DFX toxicity and endocrine disorders. Our data indicate that about 18% of thalassaemic patients exhibit short stature; disproportion between the upper and lower body segments is present in 14%; however, a short trunk despite normal stature is present in another 40% of patients. This is due to a spinal growth impairment which starts in infancy and progressively aggravates. We think that a short trunk is peculiar to the disease itself; however, other factors such as hypogonadism, siderosis, or DFX-induced bone dysplasia are probably involved in aggravating the body disproportion in these patients.
Subject(s)
Body Constitution , Body Height , beta-Thalassemia/physiopathology , Adolescent , Adult , Aging , Blood Transfusion , Child , Child, Preschool , Deferoxamine/therapeutic use , Female , Ferritins/blood , Humans , Iron Chelating Agents/therapeutic use , Male , beta-Thalassemia/therapyABSTRACT
The paper reports five cases of Kawasaki's syndrome diagnosed during the period 1985-89. The mean age of patients was 20 months (4/12; 4 years and 5/12). Symptomatology gradually regressed in four of these patients with a return to good health and normal cardiological controls. The fifth patient died suddenly following a cardiorespiratory failure when his general conditions were showing a marked improvement. Given that cardiac involvement is reported to be the sole cause of death in Kawasaki's syndrome, this was probably the cause of the patient's death. This event together with the presence of a number of barely symptomatic forms, which certainly escape correct diagnosis, may explain some of the cases of sudden death in early childhood. In the present case the child died suddenly due to an early stage of heart failure on awakening and may be considered a classic example of "cot death". Although the treatment protocol is well defined, it is not yet known to what extent the cardio-vascular apparatus is influenced by therapy in cases of children with Kawasaki's syndrome.
