Clinical Indicators of Severity of Sickle Cell Anaemia in Children in Khartoum

Abstract

Sickle cell anaemia has a wide variation in the severity of clinical presentation. A six months descriptive comparative hospital based study was conducted in the sickle cell clinic and three main Teaching Hospitals in Khartoum State. Sixty nine children with severe sickle cell anaemia and a control group of seventy children with sickle cell anaemia without evidence of disease severity were included in the study. The objectives of the study were to document the criteria for the severity of sickle cell anaemia in Sudanese children, and to describe the clinical pattern of severe sickle cell anaemia in children and its complications, and also to identify the possible contributing factors for disease severity. Most of the children with severe sickle cell anaemia had more than four episodes of vasoocclusive crises per year (59.4%) followed by proteinurea and haematuria in (37.7%) of them, (8.7%) had history of stroke, (7.2%) had more than four times blood transfusion per year, (5.8%) were diagnosed as having osteonecrosis, while acute chest syndrome, recurrent priapism and bilateral proliferative retinopathy occurred in (1.4%.). 11 Patients with severe sickle cell anaemia mainly presented with pallor (85.5%), fever (55.1%), jaundice (53.6%) and painful episodes (49.3%). Hepatomegaly was detected in (78.3%) and splenomegaly in (34.8%). They had mean haemoglobin concentration of 6.5gm/dl, total white blood cell count 12312/cumm, reticulocytes count 8.1% and fetal haemoglobin of 6.6%. There was no significant statistical difference between the children with severe sickle cell anaemia and the control group except for the percentage of patients who presented with increasing pallor, the extent of hospitalization and leukocytosis. Low haemoglobin concentration was significantly associated with stroke in children with severe sickle cell anaemia, while high haemoglobin concentration was associated with osteonecrosis.