Yasemin ISIK BALCI, Semra DEMIRLENK, Semra BALIN, Özlem ÖZLER

TALASEMİ HASTALARINDA ABDOMİNAL ULTRASONOGRAFİ BULGULARI

AMAÇ:Bu çalışmanın amacı, Denizli Devlet Hastanesi Talasemi Merkezinde izlenen talasemi major vetalasemi intermedia hastalarının rutin abdominal ultrasonografik incelemeleri seyrinde görülenhepatosplenomegali dışındaki abdominal solid organlar ile ilgili bulgularını değerlendirmektir.GEREÇ ve YÖNTEMLER:Bu çalışmaya, 73'ü talasemi major, 5'i talasemi intermedia olmak üzere, yaşları 2ile 30 yıl arasında, 40 kız, 38 erkek hasta alınmıştır.BULGULAR:Safra kesesinde taş ve çamur (%8.9) en sık görülen bulgu idi. Aksesuar dalak 6 hastada (%7.7),karaciğerde ekojenite artışı 2 hastada (%2.6), karaciğerde hemanjiom 1 hastada(%1.3), portal ven trombozu 1hastada(%1.3) saptandı. Bir talasemi major hastasında, bilateral renal kist ve sağ renal taş ve beraberinde safrakesesinde taş görüldü.SONUÇ:Talasemi hastalarında abdominal ultrasonografik inceleme, komplikasyonların önlenmesi ve etkiliklinik izlem için düzenli aralıklar ile yapılmalıdır

Anahtar Kelimeler:

Abdominal ultrasonografi, talasemi major, talasemi intermedia

Abdominal Ultrasonographic Findings in Patients with Thalassemia

OBJECTIVE: The aim of this study was to evaluate the type and prevalance of abdominal ultrasonographic abnormalities except hepatosplenomegaly in patients with thalassemia major and thalassemia intermedia. MATERIAL and METHODS: Seventy-eight patients; 73 thalassemia major, 5 thalassemia intermedia followed in Denizli State Hospital Thalassemia Center were included in the study. The patients' ages ranged from 2 to 30 years, with 40 females, 38 males. RESULTS: The most frequent ultrasonografic findings were cholelithiasis (8.9%), accesory spleen (7.7%), hepatic grade I-II echogenity (2,6%), hepatic hemangioma (1.3%) and portal vein thrombosis (1.3%). One case was diagnosed with polycystic kidney disease with coexisting nephrolitiasis and cholelithiasis. CONCLUSION: Abdominal ultrasonographic imaging of patients with thalassemia patients should be done regularly to prevent complications and for effective clinical observation.

Keywords:

Abdominal ultrasonography, thalassemia major, thalassemia intermedia,

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1. Lanzskowsky P. Thalassemias. In: Lanzskowsky P, editor. Manuel of pediatric hematology and oncology, 4th ed. Elsevier Academic Pres, California, 2005:181- 91.
2. Goldfarb A, Grisaru D, Gimmon Z, Okon E, Lebensart P, Rachmilewitz EA High incidence of cholelithiasis in older patients with homozygous beta-thalassemia. Acta Haematol 1990;83:120-2.
3. Dewey KW, Grossman H, Canale Cholelithiasis in thalassemia major. Radiology 1970;96:385-8.
4. Borgna-Pignatti C, De Stefano P, Pajno D, Tomasi G, Gatti C. Cholelithiasis in children with thalassemia major: an ultrasonographic study. J Pediatr 1981;99:243-4.
5. Galanello R, Piras S, Barella S, Leoni GB, Cipollina MD, Perseu L, Cao A. Cholelithiasis and Gilbert's syndrome in homozygous beta-thalassaemia. Br J Haematol 2001;115:926-8.
6. Papadaki MG, Kattamis AC, Papadaki IG, Menegas DG, Georgakopoulou TP, Mavrommati-Metaxotou A, Kattamis CA. Abdominal ultrasonographic findings in patients with sickle-cell anaemia and thalassaemia intermedia. Pediatr Radiol 2003;33:515-21.
7. Holcomb GW Jr, Holcomb GW. Cholelithiasis in infants, children, and adolescents. Pediatr Rev 1990;11:268-74.
8. Portincasa P, Di Ciaula A, Vendemiale G, Palmieri V, Moschetta A, VanbergHenegouwen GP, Palasciano G. Gallbladder motility and cholesterol crystallization in bile from patients with pigment and cholesterol gallstones. Eur J Clin Invest 2000;30:317-24.
9. Origa R, Galanello R, Perseu L, Tavazzi D, Domenica Cappellini M, Terenzani L, Forni GL, Quarta G, Boetti T, Piga A. Cholelithiasis in thalassemia major. Eur J Haematol 2009;82:22-5.
10. Au WY, Cheung WC, Chan GC, Ha SY, Khong PL, Ma ES. Risk factors for hyperbilirubinemia and gallstones in Chinese patients with b thalassemia syndrome. Haematologica 2003;88:220-2.
11. Portincasa P, Moschetta A, Berardino M, Di-Ciaula A, Vacca M, Baldassarre G, Pietrapertosa A, Cammarota R, Tannoia N, Palasciano G. Impaired gallbladder motility and delayed orocecal transit contribute to pigment gallstone and biliary sludge formation in betathalassemia major adults. World J Gastroenterol 2004;10:2383-90.
12. Kalayci AG, Albayrak D, Güneş M, Incesu L, Agaç R. The incidence of gallbladder stones and gallbladder function in beta-thalassemic children. Acta Radiol 1999;40: 440-3.
13. Senaati S, Gumruk FU, Delbakhsh P, Balkanci F, Altay C. Gallbladder pathology in pediatric beta-thalassemic patients. A prospective ultrasonographic study. Pediatr Radiol 1993;23:357-9.
14. Cohen A, Gayer R, Mizamir J: Long-term effect of splenectomy on transfusion requirements in thalasemia major.Am J Hematol 1989;30:254
15. Al-Hawsawi ZM, Haouimi AS, Hassan RA, Tarawah AM. Portal vein thrombosis after splenectomy for betathalassemia major. Saudi Med J 2004;25:225-8
16. Fujita F, Lyass S, Otsuka K, Giordano L, Rosenbaum DL, Khalili TM, Phillips EH. Portal vein thrombosis following splenectomy: identification of risk factors. Am Surg 2003;69:951-6.