Bone density study at the distal radius, using pQCT analysis, in Greek thalassemic patients.

BACKGROUND: Expansion of marrow spaces in thalassemia patients contributes to reduced bone density and increased risk of fractures. Peripheral quantitative computed tomography (pQCT) measures the volumetric bone mineral density (vBMD) independently in different compartments. OBJECTIVE AND METHODS: To study the regional bone structure in thalassemia, we performed pQCT (XCT-2000 Scanner, Stratec Inc.) in 57 young adult patients with beta-thalassemia major, aged 21-44 years (32 females). The vBMDs (total, trabecular, cortical + subcortical), Stress-Strain Index (SSI) and the cross-sectional area (CSA) were determined at the "4% site" of the distal radius; data were compared to reference values. RESULTS: Total vBMD, trabecular, cortical plus subcortical vBMD in thalassemics were significantly lower than in healthy adults (p<0.005). Thalassemic males had significant higher values of vBMD, SSI and CSA than females (p<0.005). CONCLUSIONS: In Greek thalassemic patients a significant reduction in bone density, associated with increased morbidity and risk of fractures, has been detected on pQCT examination.

Abdominal ultrasonographic findings in patients with sickle-cell anaemia and thalassaemia intermedia.

BACKGROUND: The high prevalence and heterogeneity of clinical and haematological phenotypes of sickle-cell anaemia and thalassaemia intermedia (TI) syndromes in the Greek population constitute a major challenge in routine medical practice. PURPOSE: To investigate the spectrum and prevalence of abdominal US findings in these patients. MATERIALS AND METHODS: The study included 105 patients, 13 homozygous for sickle-cell anaemia (S/S) and 92 with TI syndromes (Th/S, beta(thal)/beta(thal )or beta(thal)/deltabeta(thal)). The patients' ages ranged from 1 to 54 years, with 49 males and 56 females. RESULTS: At the time of the examination, 41% of the patients had undergone splenectomy and 23.8% had undergone cholecystectomy. The most frequent US findings (percentages refer to all patients) were: hepatomegaly (70.5%), splenomegaly (48.6%) and cholelithiasis (20%). A bright liver was identified in 3.8% and focal hepatic lesions were present in two patients of the TI group. Functional disorders of the gallbladder concerned mainly patients with sickle-cell syndromes. Of those with S/S, 30.8% had a shrunken spleen. An echogenic pancreas was identified in one patient in each group. Increased renal echogenicity was observed in 17.6% of patients with sickle-cell syndromes. One case of polycystic kidney disease was diagnosed and coexisted with S/S. CONCLUSIONS: The value of US imaging in detecting complications of sickle-cell and TI syndromes is still debated. To our knowledge, there are no previous reports concerning the frequency and spectrum of abdominal US findings in patients with these syndromes.