TY - JOUR
T1 - Splenic function in Omani children with sickle cell disease
T2 - Correlation with severity index, hemoglobin phenotype, iron status, and α-thalassemia trait
AU - Wali, Yasser A.
AU - Al-Lamki, Zakia
AU - Hussein, Samir S.
AU - Bererhi, Hadia
AU - Kumar, Dilip
AU - Wasifuddin, Shah
AU - Zachariah, Mathew
AU - Ghosh, Kanjaksha
PY - 2002/10
Y1 - 2002/10
N2 - 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 (Hb S-S), 11 double heterozygotes for HbS and β0-thalassemia (HbS-β0-thal), 5 HbS-β+-thal, 5 patients with hemoglobin S-D disease, and 1 child with hemoglobin S oman trait) aged 4.8-16 years, using 99mTc-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-β+ 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 α-thalassemia trait (p < .05).
AB - 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 (Hb S-S), 11 double heterozygotes for HbS and β0-thalassemia (HbS-β0-thal), 5 HbS-β+-thal, 5 patients with hemoglobin S-D disease, and 1 child with hemoglobin S oman trait) aged 4.8-16 years, using 99mTc-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-β+ 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 α-thalassemia trait (p < .05).
KW - Colloid scintigraphy
KW - Sickle cell disease
KW - Splenic function
UR - http://www.scopus.com/inward/record.url?scp=0036786019&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0036786019&partnerID=8YFLogxK
U2 - 10.1080/08880010290097314
DO - 10.1080/08880010290097314
M3 - Article
C2 - 12217195
AN - SCOPUS:0036786019
SN - 0888-0018
VL - 19
SP - 491
EP - 500
JO - Pediatric Hematology and Oncology
JF - Pediatric Hematology and Oncology
IS - 7
ER -