Abstract
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, βthal/βthal or βthal/δβthal). 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.
Similar content being viewed by others
References
Konus LO, Ozdemir A, Akkaya A, et al (1988) Normal liver, spleen and kidney dimensions in neonates, infants and children: Evaluation with sonography. AJR 171:1693–1698
Magid D, Fishman EK, Siegelman SS (1984) Computed tomography of the spleen and liver in sickle cell disease. AJR 143:245–249
Hernandez RJ, Sarnaik SA, Lande I, et al (1988) MR evaluation of liver iron overload. J Comput Assist Tomogr 12:91–94
Bond LR, Hatty SR, Horn ME, et al (1987) Gall stones in sickle cell disease in the United Kingdom. BMJ 295:234–236
Everson GT, Nemeth A, Kourourian S, et al (1989) Gallbladder function is altered in sickle hemoglobinopathy. Gastroenterology 96:1307–1316
Nzeh DA, Adedoyin MA (1989) Sonographic pattern of gallbladder disease in children with sickle cell anaemia. Pediatr Radiol 19:290–292
Rao VM, Mapp EM, Wechsler RJ (1987) Radiology of the gastrointestinal tract in sickle cell anemia. Semin Roentgenol 22:195–204
Walker TM, Hambleton IR, Serjeant GR (2000) Gallstones in sickle cell disease: observations from The Jamaican Cohort Study. J Pediatr 136:80–85
Walker TM, Serjeant GR (1996) Biliary sludge in sickle cell disease. J Pediatr 129:443–445
Adler DD, Glazer GM, Aisen AM (1986) MRI of the spleen: normal appearance and findings in sickle cell anemia. AJR 147:843–845
Walker TM, Serjeant GR (1993) Focal echogenic lesions in the spleen in sickle cell disease. Clin Radiol 47:114–116
Shirkhoda A, Wallace S, Sokhandan M (1985) Computed tomography and ultrasonography in splenic infarction. J Can Assoc Radiol 36:29–33
Flyer MA, Haller JO, Sundaram R (1993) Transfusional hemosiderosis in sickle cell anemia: another cause of an echogenic pancreas. Pediatr Radiol 23:140–142
Mapp E, Karasick S, Pollack H, et al (1987) Uroradiological manifestations of S-hemoglobinopathy. Semin Roentgenol 22:186–194
Huntington DK, Hill SC, Hill MC, et al (1991) Sonographic manifestations of medical renal disease. Semin Ultrasound CT MR 12:290–307
Aikimbaev KS, Oguz M, Guvenc B, et al (1996) Spectral pulsed Doppler sonography of renal vascular resistance in sickle cell disease: clinical implications. Br J Radiol 69:1125–1129
Davidson AJ (1996) Increased renal reflectivity in sickle cell disease. Clin Radiol 51:231
Kraus RA, Gaisie G, Young LW (1990) Increased renal parenchymal echogenicity: causes in paediatric patients. Radiographics 10:1009–1018
Namjoshi SP (1999) Punctuate echogenic foci in spleen and increased echogenicity in renal cortex in sickle cell anemia. J Clin Ultrasound 27:52
Walker TM, Serjeant GR (1995) Increased renal reflectivity in sickle cell disease: prevalence and characteristics. Clin Radiol 50:566–569
Zinn D, Haller JO, Cohen HL (1993) Focal and diffuse increased echogenicity in the renal parenchyma in patients with sickle hemoglobinopathies—an observation. J Ultrasound Med 12:211–214
Walker TM, Beardsall K, Thomas PW, et al (1996) Renal length in sickle cell disease: observations from a cohort study. Clin Nephrol 46:384–388
Kimberling WJ, Yium JJ, Jonson AM, et al (1996) Genetic studies in a black family with autosomal dominant polycystic kidney disease and sickle-cell trait. Nephron 72:595–598
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Papadaki, M.G., Kattamis, A.C., Papadaki, I.G. et al. Abdominal ultrasonographic findings in patients with sickle-cell anaemia and thalassaemia intermedia. Pediatr Radiol 33, 515–521 (2003). https://doi.org/10.1007/s00247-003-0950-5
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00247-003-0950-5