ABSTRACT
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/therapySubject(s)
Glomerulonephritis/drug therapy , Heparin/therapeutic use , Child , Female , Glomerulonephritis/pathology , Humans , Kidney/pathology , MaleSubject(s)
Glomerulonephritis/drug therapy , Heparin/therapeutic use , Lupus Erythematosus, Systemic/drug therapy , Child , Female , Glomerulonephritis/etiology , Glomerulonephritis/pathology , Humans , Immunosuppressive Agents/therapeutic use , Kidney/pathology , Lupus Erythematosus, Systemic/pathologyABSTRACT
IgM and IgA dosed in cord sera of 400 infants. IgM are present in all samples; 16.25% of them show levels 25 mg/100 cm3. IgA are present in 5.75%, but only 2.25% show levels 6 mg/100 cm3. Our controls have confirmed they depend on maternal blood contamination. Correlations between IgM and IgA levels and perinatal infections are considered. Further studies are required to determine the presumptive nature of infectious agents.
