The common childhood renal disorders in Nigeria are post-infectious acute glomerulonephritis (PIAGN), nephrotic syndrome, hypertension, congenital anomalies and urinary tract infection. Children with PIAGN often present with hypertension, circulatory overload and hypoalbuminaemia. Nephrotic syndrome is characterized by a paucity of minimal change and a poor prognosis. Posterior urethral valves and hydronephrosis are the most frequent congenital anomalies. Children with congenital anomalies present late with advanced disease. The commonest malignancy is nephroblastoma, but Burkitt's lymphoma of the kidney also occurs. Both acute and chronic renal failure present depressing and distressing clinical problems, with an aggressive course and a high morbidity and mortality. Poor socio-economy has adverse effects on the presentation, management and follow-up of Nigerian children with renal disorders.
Alport syndrome is a heterogeneous genetic disease involving the basement membrane of the glomeruli, inner ear, retina, and lens capsule. It typically manifests as progressive glomerulopathy that frequently results in end-stage renal disease, high-tone sensorineural deafness, and ocular abnormalities of anterior lenticonus and yellow and white dots and flecks on the macular of the retina. In this report, we describe the cases of 2 siblings: 15- and 13-year-old boys of pure African descent with the <i>COL4A5</i> gene mutation. Both children had the classical features of Alport syndrome haematuria, proteinuria, progressive sensorineural high-tone hearing loss, and ocular abnormalities. Their renal abnormalities initially regressed on therapy with angiotensin-converting enzyme inhibitors but reoccurred, depicting the need for early diagnosis as the early institution of this therapy before significant glomerulopathy is advocated.
We report here on a child who over a period of 8 years was admitted several times to hospitals in different states of Nigeria based on fictitious illnesses described by his mother. The child had various unnecessary, expensive and invasive investigations followed by treatment with harmful drugs. The evolution of this case of Munchausen syndrome by proxy is described in order to alert paediatricians in developing countries to a problem which is described frequently in more affluent societies. We believe this is the first such case to be recorded in West Africa.
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