Paraplegia in a thalassaemic patient with short stature.

Extramedullary hematopoiesis (EMH) is a normal compensatory reaction that occurs in almost all chronic hemolytic anemia, especially in transfusion independent thalassemia intermedia, and can involve many organs or tissues, including the epidural space leading to spinal cord compression syndrome. We present a case of EMH in a 29 year old woman with thalassemia major, regularly transfused since the time of diagnosis (age 21 months), who presented with sudden muscle weakness, difficulty walking and maintaining the upright position. Magnetic Resonance Imaging (MRI) of the thoracic spine showed spinal cord compression secondary to extramedullary hematopoiesis in the spinal canal, leading to early therapy.

Vertebral collapse in thalassemia intermedia: case report.

Over the last few decades, the use of regular blood transfusions and adequate iron chelation beginning in the first years of life has modified the clinical picture and the natural course of thalassemia major. With the rise in the average age of these patients new problems have emerged, in particular bone disease: osteopenia, osteoporosis and the increased risk of fractures have become important causes of morbidity in a population whose longevity is continuously increasing. The Authors describe the case of a 41 year old patient affected by clinical thalassemia intermedia who presented with vertebral collapse after mild trauma.

Effects of acarbose in beta-thalassaemia major patients with normal glucose tolerance and hyperinsulinism.

The pathogenesis of diabetes in thalassaemia is complex and multifactorial. Understanding the sequence of abnormalities in the progression from normal glucose tolerance to impaired glucose tolerance may help in the formulation of ways to intervene in this process. In our study, we assessed the effects of acarbose, an alpha-glucosidase inhibitor, in five young adult thalassaemic patients with hyperinsulinism and normal oral glucose tolerance test (OGTT).

Effects of acarbose in patients with beta-thalassaemia major and abnormal glucose homeostasis.

Seventeen TM patients with impaired glucose tolerance (IGT) or non-insulin dependent diabetes mellitus (NIDDM) and hyperinsulinism were treated for 12 months with acarbose (100 mg. orally with breakfast, lunch and evening meals). An improvement in glucose tolerance was observed in 2 out of 11 TM patients with IGT and in all TM patients with NIDDM.

Growth hormone secretion in adult patients with thalassaemia.

Background And Objectives: Growth retardation and short stature are frequent clinical features of patients with beta-thalassaemia major. Dysfunction of the GH-IGF-1 axis has been described in many thalassaemic children and adolescents with short stature and reduced growth velocity. Several studies have demonstrated that recombinant GH treatment improves growth velocity in these patients, although response to the treatment is variable and not predictable.

Potential myocardial iron content evaluation by magnetic resonance imaging in thalassemia major patients treated with Deferoxamine or Deferiprone during a randomized multicenter prospective clinical study.

The purpose of this study was to evaluate if the variations of heart magnetic resonance imaging in beta-thalassemia major patients treated with Deferoxamine B mesylate (DF) or Deferiprone (L1) chelation therapy is a useful tool of the indirect myocardial iron content determination. For this reason, a prospective study was carried out. Seventy-two consecutive patients with beta-thalassemia major (35 treated with DF and 37 with L1) were studied.

Deferiprone versus deferoxamine in patients with thalassemia major: a randomized clinical trial.

Deferiprone has been suggested as an effective oral chelation therapy for thalassemia major. To assess its clinical efficacy, we compared deferiprone with deferoxamine in a large multicenter randomized clinical trial. One-hundred forty-four consecutive patients with thalassemia major and serum ferritin between 1500 and 3000 ng/ml were randomly assigned to deferiprone (75 mg/kg/day) (n = 71) or deferoxamine (50 mg/kg/day) (n = 73) for 1 year.

Impaired glucose homeostasis in young adult thalassemic patients: a pilot study with acarbose.

This study investigated the effects of the alpha-glucosidase inhibitor, acarbose, on glycemic control and insulin secretion in thalassemic patients with impaired glucose tolerance. The safety and tolerability of the drug were also evaluated. Nine patients (4 men and 5 women, aged 20-34 years) with beta-thalassemia major received a standardized nutritional test load prior to and following 3 months treatment with acarbose 100 mg t.