Long-term follow-up of skeletal dysplasia in thalassaemia major.

We report skeletal changes due to deferoxamine (DF) in 15/29 patients with transfusion-dependent thalassaemia major (TM), followed longitudinally for growth assessment. Clinically the earliest signs were decline in height and/or sitting height growth rate, leg and back pain with restricted movement and limb deformity. Radiologically metaphyseal and spinal changes were seen in 5 subjects and vertebral lesions alone in 10. The metaphyseal changes were mild, moderate or severe and affected all long bones, but were most pronounced at wrists and knees. They progressed from widening of the growth plate and defects of metaphyseal margins to appearance of radiolucent pseudocystic areas and, in severe cases, of cupped, rickets-like metaphyses. The spinal changes proceeded from osseous defects of ventral upper and lower edges of vertebrae and biconvex contours of end-plates to platyspondyly with decreased vertebral body height. After DF dose reduction, metaphyseal changes regressed in 2 patients, while they progressed in 3, requiring corrective surgery for severe valgus knee. Spinal abnormalities either remained unchanged or progressed. Final height was very short in patients with spondylometaphyseal lesions, short and disproportionate in patients with only spinal involvement.

[Splenectomy and infection in homozygous beta-thalassemia. The uselessness of penicillin prophylaxis]. / Splenectomia ed infezioni nella beta-talassemia omozigote. Inutilità della profilassi penicillinica.

To investigate the effective usefulness of penicillin prophylaxis in splenectomized patients, we retrospectively focused on a group of sixty-two splenectomized patients affected by thalassemia major. Thirty-six out of 62 has been receiving monthly 1.200.000 Us. of benzathine-penicillin as prophylaxis. The remaining 26 did not receive prophylaxis, but was treated with antibacterial drugs as soon as symptoms of upper respiratory tract infection occurred. During a total period of eleven years of observation we did not observe any pneumococcal sepsis; the incidence of bacterial infections within the two groups is not different. We conclude for the uselessness of penicillin prophylaxis in splenectomized beta-thalassemic patients.

[Yersinia enterocolitica sepsis in splenectomized thalassemic subjects. Description of 2 cases]. / La sepsi da Yersinia enterocolitica nei soggetti talassemici splenectomizzati. Descrizione di due casi.

Two cases of Yersinia enterocolitica septicemia in two splenectomized children with thalassemia major are reported. Human Yersinia enterocolitica septicemia is an uncommon condition, but its frequency may increase in patients having debilitating diseases or blood disorders, as well as a consequence of the splenectomy. Some brief considerations on the pathogenetic factors yielding, to severe infections in splenectomized thalassemic children are discussed. The high number of thalassemic people in Italy makes necessary an early diagnosis in case of septicemia. Particularly important is the blood culture, because of the slowness of the microbic development in stools.