RESUMO
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.
