BackgroundPaediatric rheumatology service in Sub-Sahara African is virtually not available as there is a shortage of paediatric rheumatologists and other rheumatology health professionals. We aim to describe the clinical spectrum and the frequencies of paediatric rheumatic diseases (PRDs) in Lagos State University Teaching Hospital (LASUTH), Lagos, Nigeria.MethodsThis is a retrospective review of patients with PRDs seen over a five year period (March 2010 to February 2016) at the rheumatology clinic and children ward of LASUTH. We reviewed the folders of 57 patients from our records. The demographics, baseline laboratory features, clinical diagnosis, treatment patterns and patient outcomes were extracted and analyzed. Clinical and laboratory characteristics between patients with Juvenile idiopathic arthritis (JIA) and patients with juvenile connective tissue diseases (JCTD) were compared using Fisher’s exact test.ResultsFifty seven patients were studied with a female to male ratio of 3 to 1 (Female: 43; M: 14). The mean age at presentation in years was 14 ± 4.4 years (range: 1.5–22 years). The mean duration of symptoms before diagnosis was 18.4 ± .9 months (range: 2–60 months). The diagnostic types of PRDs included 28(49.1%) cases of JIA. These were made up of 14 cases of polyarticular JIA, nine cases of oligoarticular JIA and 5 cases of systemic onset JIA. Others were 18 (24.6%) cases of juvenile systemic lupus erythematosus (JSLE), 3 (5.3%) cases of joint hypermobility syndrome, 2 (3.5%) cases of juvenile systemic sclerosis, 2 (3.5%) cases of fibromyalgia, 2 (3.5%) cases of plantar fasciitis, 1 (1.6%) case of juvenile dermatomyositis (JDM), 1 (1.6%) case of juvenile polymyositis-systemic lupus erythematosus (PM-SLE) overlap, 1 (1.6%) case of secondary bilateral knee osteoarthritis from Blount disease, 1 (1.6%) case of secondary osteoporosis from childhood leukemia and 1 (1.6%) case of Osgood-Schlatter’s disease. Constitutional symptoms and extra-articular diseases were significantly more frequent among JCTD cases than among the JIA cases (Constitutional symptoms: 100% vs 83.3%, p = 0.003; extra-articular disease: 100% vs 10.7%, p = 0.001). The percentage mortality in this study was 10.5% while 20 (35.1%) of the patients were lost to clinic follow up.ConclusionThe pattern of PRDs observed in this study is similar to that described in South African and North American series but it differs from patterns reported in Asian series. Although hitherto largely unrecognized, PRDs may constitute a substantial cause of morbidity and mortality in black Africans.
Juvenile systemic lupus erythematosus (JSLE) is a complex multisystemic autoimmune disorder of unknown cause. It accounts for about one in five cases of SLE. The tendency for SLE to run a fulminant course when it starts in childhood has made JSLE a potentially more severe disease than adult SLE. Reports of JSLE from sub-Saharan Africa are scanty in spite of the increasing reports of adult SLE. We conducted a 4-year retrospective study of JSLE cases seen at the Lagos State University Teaching Hospital between January 2010 and December 2014. Out of the 12 patients studied, eight were girls and four were boys. All patients had positive antinuclear antibody and extractable nuclear antibody tests. Anti-dsDNA antibody was positive in 10 patients. Eight patients had renal disease while four patients had neuropsychiatric manifestations. Haematological abnormalities and constitutional symptoms were present in all patients. Patients were treated with pulse methylprednisolone, oral prednisolone, hydroxychloroquine and azathioprine. Three patients also received rituximab. In conclusion, JSLE exists in Nigeria and exhibits clinical and immunological characteristics similar to its pattern in other parts of the world. It is, however, diagnosed late and is possibly being underdiagnosed as there is no paediatric rheumatologist in the country.
Uraemic optic neuropathy (UON) is an acute but reversible loss of vision in patients with end-stage renal failure. Hypoperfusion of the posterior ciliary arteries, the major blood supply to the optic nerve head, results in ischaemic injury to the nerve. Anaemia, hypertension and elevated nitrogen urea level have been identified as factors contributing to optic nerve neuropathy. Toxic uraemic metabolites affect nerve conduction and their removal with dialysis results in improved vision. Optic neuropathy is a rare complication of uraemia in children. This is the first case of optic neuropathy related to uraemia in a child with end-stage renal disease in Nigeria.
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