Splenic function in Omani children with sickle cell disease: Correlation with severity index, hemoglobin phenotype, iron status, and α-thalassemia trait

Yasser A. Wali, Zakia Al-Lamki, Samir S. Hussein, Hadia Bererhi, Dilip Kumar, Shah Wasifuddin, Mathew Zachariah, Kanjaksha Ghosh

Research output: Contribution to journalArticle

23 Citations (Scopus)

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 (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

Original languageEnglish
Pages (from-to)491-500
Number of pages10
JournalPediatric Hematology and Oncology
Volume19
Issue number7
DOIs
Publication statusPublished - Oct 2002

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Thalassemia
Hemoglobin
Sickle Cell Anemia
Hemoglobins
Iron
Phenotype
Colloids
Tin
Sickle Hemoglobin
Severity
Heterozygote
Natural History
Radionuclide Imaging
Spleen

Keywords

  • Colloid scintigraphy
  • Sickle cell disease
  • Splenic function

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Hematology
  • Oncology
  • Cancer Research
  • Management of Technology and Innovation

Cite this

Splenic function in Omani children with sickle cell disease : Correlation with severity index, hemoglobin phenotype, iron status, and α-thalassemia trait. / Wali, Yasser A.; Al-Lamki, Zakia; Hussein, Samir S.; Bererhi, Hadia; Kumar, Dilip; Wasifuddin, Shah; Zachariah, Mathew; Ghosh, Kanjaksha.

In: Pediatric Hematology and Oncology, Vol. 19, No. 7, 10.2002, p. 491-500.

Research output: Contribution to journalArticle

Wali, Yasser A. ; Al-Lamki, Zakia ; Hussein, Samir S. ; Bererhi, Hadia ; Kumar, Dilip ; Wasifuddin, Shah ; Zachariah, Mathew ; Ghosh, Kanjaksha. / Splenic function in Omani children with sickle cell disease : Correlation with severity index, hemoglobin phenotype, iron status, and α-thalassemia trait. In: Pediatric Hematology and Oncology. 2002 ; Vol. 19, No. 7. pp. 491-500.
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