| dc.description.abstract | The landmark contributions to the recognition and understanding of sickle-cell disease at the clinical, cellular and molecular levels are reviewed. The variable natural history of the disease with environmental and socio-economic factors is noted, and the devastating clinical course of the disease in West Africa stressed. The literature on the skeletal manifestations of sickle-cell disease is reviewed, with special emphasis on contributions from Africa.
The general clinical syndrome of sickle-cell disease in 207 Nigerian patients studied, is presented. The general clinical features of sickle-cell disease are similar to previous accounts from West Africa, but differ from reports from non-malarial countries in the incidence of florid infective complications and the frequency of severe anaemia. Details are presented of 266 major skeletal complications encountered in the 207 Nigerian sicklers; Fyogenic osteomyelitis was present in 129 patients septic arthritis in 31, avascular necrosis of the femoral head in 75, pathological fracture in 26 and miscellaneous complications in 5 patients. Infection comprised 70% of the 266 skeletal complications in this series.
Acute osteomyelitis is a commoner and more florid disease among sicklers in Nigeria than in non-malarial countries. Salmonella comprised 50% of the positive cultures; and 80% of Salmonella acute osteomyelitis was multifocal. A combination of chloramphenicol and cloxacillin was found to be the best guess antibiotic in acute osteomyelitis in Ibadan. The incidence of progression from acute to chronic osteomyelitis was higher when pus was evacuated by open drainage than by needle aspiration. Chronic osteomyelitis was commoner and more difficult to treat than in non-malarial countries. Sinographic findings in 14 patients with chronic discharging osteomyelitis of 18 Iong bones are described. This investigation contributed to the choice of definitive treatment and the type of operative procedure in 13 (72%) of the 18 long bones. Operative treatment of chronic osteomyelitis was safe, and the results were comparable with those in patients without haemoglobinopathy, but conservative treatment yielded poor results. Septic arthritis was commoner and more florid than in sicklers in non-malarial countries. Its pattern was similar to that described in patients without haemoglo-binopathy, apart from the high incidence of multifocal
joint infection in HbSS, the marked preponderance of gram-negative infection, and the high complication rate. The pattern of avascular necrosis o the femoral head was similar to recent reports from non-malarial countries. The early results of rotation upper femoral osteotomy in young children with Perthes-like necrosis appeared encouraging; but conservative treatment yielded poor results. Pathological fracture complicating osteomyelitis was commoner than in non-malarial countries. It was similar in pattern to fracture complicating bone infarcts
in West Africa, except for a preponderance in male HbSS patients under 10 years of age, with left-sided, whole-diaphysial, gram-negative osteomyelitis. These findings are discussed and contrasted with the patterns of major skeletal complications in sicklers elsewhere. It is concluded that pyogenic skeletal infections are commoner and more florid here than in non-malarial countries. They significantly impair the quality of life, and are a major cause of crippling of many surviving sickle-cell patients in Nigeria. | en_US |
