Maqbool Alam, Munir Akmal Lodhi, Hassan Ahmed Hassan.
Clinico-pathological profile of sickle cell disease in children.
Pak J Pathol Jan ;13(2):7-11.

The objective of this study is to highlight the clinical, hematological and biochemical findings in children having sickle cell disease in the Southwestern part of Saudi Arabia. A prospective study was designed at Armed Forces Hospital- Sharourah in the Southwestern part of the Saudi Arabia. The study period was of two years from Jan 2001 to Dec 2002. Thiry-four children with homozygous sickle cell disease were followed over a period of two years. Clinical,hematological and biochemical features during each admission were recorded and analyzed. The mean age of the patients was 6.4± 4.9 years. Twenty-one (61.76%) were female and 13 (38.24%) were male. About 79% hospital admissions were due to painful crises. Splenomegaly, hepatomegaly and cholelithiasis were present in 14.70%, 5.88% and 20.58% of patients respectively. Mean hemoglobin concentration was 7.6±1.4 g/dL. Mean corpuscular volume (MCV) and mean corpuscular hemoglobin (MCH) were 86.9±11.1 fL and 26.6±3.5 pg respectively. Platelet, leucocyte and reticulocyte counts were increased. Fetal hemoglobin (Hb F) levels were from 7 to 28% with a mean of 17.57±5.41%. Hyperbilirubinemia and high lactate dehydrogenase (LDH) concentration were recorded. Analysis of clinico-pathological manifestations suggests that many patients homozygous sickle cell disease from Southwestern region of Saudi Arabia suffered from painful crises but severest complications are rare in these children probably due to high fetal haemoglobin.

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