title: Splenic function in Omani children with sickle cell disease: correlation with severity index, hemoglobin phenotype, iron status, and alpha-thalassemia trait.
abstract: The prevalence of functional asplenia in Omani children with sickle cell disease (SCD) has not been previously defined. In this study, the authors aim to compare the natural history of splenic dysfunction in their patients to other reports. The splenic function was studied in 72 Omani patients with sickle cell disease (50 homozygous for hemoglobin S (HbS-S), 11 double heterozygotes for HbS and beta(0)-thalassemia (HbS-beta(0)-thal), 5 HbS-beta(+)-thal, 5 patients with hemoglobin S-D disease, and 1 child with hemoglobin S oman trait) aged 4.8-16 years, using (99m)Tc-labeled tin colloid scintigraphy. The study revealed 4 groups according to their colloid uptake: group I included 20 patients (28%) with normal splenic function; group II, 6 patients (8%) with mild hyposplenism; group III, 20 (28%) with severe hyposplenism; and group IV, 26 (36%) patients with functional asplenia. Overall, more than 60% of them had preserved splenic function. Except for HbS-beta(+) patients, the developmental pattern of hyposplenism was not different among the different Hb phenotypes. Factors associated with preservation of spleen function in these patients were larger splenic size (p < .01), less clinical severity (p < .05), lower MCH (p < .01), higher HbF (p < .001), and presence of alpha-thalassemia trait (p < .05).
authors.affiliation: Department of Child Health, Haematology/Oncology Unit, College of Medicine, Sultan Qaboos University, Al-Khod, Muscat, Oman.
authors.forename: Yasser A
authors.initials: YA
authors.lastname: Wali
authors.forename: Zakia
authors.initials: Z
authors.lastname: Al-Lamki
authors.forename: Samir S
authors.initials: SS
authors.lastname: Hussein
authors.forename: Hadia
authors.initials: H
authors.lastname: Bererhi
authors.forename: Dilip
authors.initials: D
authors.lastname: Kumar
authors.forename: Shah
authors.initials: S
authors.lastname: Wasifuddin
authors.forename: Mathew
authors.initials: M
authors.lastname: Zachariah
authors.forename: Kanjaksha
authors.initials: K
authors.lastname: Ghosh
mesh_terms: D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000150:complications* / Q000453:epidemiology / Q000235:genetics; D002648:Child; D002675:Child, Preschool; D005260:Female; D006453:Hemoglobinopathies / Q000150:complications; D006454:Hemoglobins / Q000032:analysis / Q000235:genetics; D006801:Humans; D007501:Iron / Q000097:blood; D008297:Male; D009850:Oman / Q000453:epidemiology; D010641:Phenotype; D015995:Prevalence; D011877:Radionuclide Imaging; D012307:Risk Factors; D012720:Severity of Illness Index; D013158:Splenic Diseases / Q000000981:diagnostic imaging / Q000453:epidemiology / Q000209:etiology*; D013667:Technetium; D017085:alpha-Thalassemia / Q000150:complications
chemical_list: D006454:Hemoglobins; D013667:Technetium; D007501:Iron