Of 880 patients who attended the paediatric cardiology unit, University College Hospital, Ibadan, during a ten-year period, 635 (72.2%) had congenital cardiac malformations. Ventricular septal defects were the commonest lesions (35% of the 635 cases), followed by patent ductus arteriosus (22%), Fallot's tetralogy (10%), pulmonary stenosis (9%) and atrial septal defects (7.5%). Coarctation of the aorta was uncommon (2%) and aortic stenosis rare (0.6%). The overall sex incidence was even. Aetiological factors were ascertained in 72 cases (11%). In 60% of these cases intra-uterine rubella was responsible and in 18% perinatal asphyxia. In most patients the cardiac malformation was detected late; consequently complications, such as heart failure, pulmonary hypertension, and polycythaemia were common, even in new patients. Sixty-seven patients (11%) died in hospital, mostly from surgical intervention, heart failure, hypoxaemia, bronchopneumonia and associated extracardiac defects. It is suspected that hypocalcaemia, resulting from vitamin D deficiency may be a cause of the observed low prevalence of obstructive aortic lesions. We suggest that cardiac evaluation be performed at birth in postnatal clinics and in immunization centres, in order to facilitate early detection and treatment of congenital heart disease.

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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.

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