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.


OBJECTIVE
To investigate the spectrum and prevalence of abdominal US findings in these patients.


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.
Papadaki
Marina G
MG
1st Department of Paediatrics, University of Athens, Agia Sofia Children's Hospital, Athens, Greece. papadaki1@yahoo.com
Kattamis
Antonios C
AC
Papadaki
Irene G
IG
Menegas
Damianos G
DG
Georgakopoulou
Theano P
TP
Mavrommati-Metaxotou
Anna
A
Kattamis
Christos A
CA
D000005:Abdomen / Q000000981:diagnostic imaging*; D000293:Adolescent; D000328:Adult; D000755:Anemia, Sickle Cell / Q000000981:diagnostic imaging*; D002648:Child; D002675:Child, Preschool; D002769:Cholelithiasis / Q000000981:diagnostic imaging / Q000209:etiology; D005260:Female; D006529:Hepatomegaly / Q000000981:diagnostic imaging / Q000209:etiology; D006801:Humans; D007223:Infant; D007674:Kidney Diseases / Q000000981:diagnostic imaging / Q000209:etiology; D008297:Male; D008875:Middle Aged; D010182:Pancreatic Diseases / Q000000981:diagnostic imaging / Q000209:etiology; D013163:Splenomegaly / Q000000981:diagnostic imaging / Q000209:etiology; D013789:Thalassemia / Q000000981:diagnostic imaging*; D014463:Ultrasonography