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Sickle cell morbidity profile in Omani children
1Department of Paediatrics, Nizwa Hospital, Nizwa, Sultanate of Oman. efjay@omantel.net.om
Insights
Sickle cell disease in Omani children presents a severe morbidity profile, with vaso-occlusive crises and weight faltering being most common. This highlights the need for improved management and prevention strategies for pediatric sickle cell disease.
Area of Science:
- Pediatrics
- Hematology
- Genetics
Background:
- Sickle cell disease (SCD) poses a significant global health challenge, particularly in pediatric populations.
- Understanding the specific morbidity patterns in different ethnic and geographic groups is crucial for targeted interventions.
Purpose of the Study:
- To define the morbidity profile of sickle cell disease in Omani children.
- To identify common complications and assess disease severity in this cohort.
Main Methods:
- Retrospective analysis of 97 Omani children (aged <= 12 years) with SCD admitted between July 1999 and June 2000.
- Data collection included admission reasons, frequency, complications, and anthropometric measurements.
- Comparison of weight faltering with age- and sex-matched non-sickle cell disease controls.
Main Results:
- Vaso-occlusive crises (83%) and severe anemia (12%) were primary admission reasons.
- Significant weight faltering was observed in 68% of children with SCD compared to 28% of controls (p < 0.001).
- Other complications included hypersplenism, avascular necrosis, acute chest syndrome, and splenic sequestration; 71% had moderately severe to severe disease.
Conclusions:
- Omani children with sickle cell disease exhibit a severe morbidity profile.
- Meteorological and genetic factors may contribute to this pattern.
- There is an urgent need for enhanced management strategies and preventive measures for pediatric SCD.
Abstract:
In order to define the morbidity profile of sickle cell disease in Omani children, we analysed data on 97 children (53 boys, 44 girls) aged < or = 12 years admitted under our care in a regional referral hospital between July 1999 and June 2000. Ninety of them had sickle cell anaemia (HbSS disease) and seven had sickle cell thalassaemia (beta zero). Their mean (SD) steady-state Hb was 7.9 (1.2), range 6-10 g/dl. They were admitted on 316 occasions during the 12-month period. The number of admissions per child ranged from one to 12 (mean 3.3). Vaso-occlusive crises were the main reason for admission (83%), followed by severe anaemia (12%) and infections (4%). During the study period, 31% received blood transfusions. Weight faltering was very common, 68% falling below the 5th percentile of the National Center for Health Statistics reference curves compared with 28% of age- and sex-matched non-sicklers (p < 0.001). Other complications included hypersplenism (four), ischaemic necrosis of the femoral head (two), and one case each of acute chest syndrome, acute splenic sequestration, cholelithiasis and pathological fracture of a lumbar vertebra. Overall, 71% of the children had moderately severe or severe disease. This pattern seems to be attributable, at least in part, to meteorological and genetic factors. The severe morbidity profile reported in this study underscores the need to continue the search for optimal management modalities, including the often emotion-laden issue of prevention.