British medical bulletin
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The haemolytic uraemic syndrome (HUS) is characterized by the triad of thrombocytopenia, microangiopathic haemolytic anaemia and acute renal failure. HUS may be classified as either diarrhoeal-associated or non-diarrhoeal/atypical (aHUS). aHUS has recently been shown to be a disease of complement dysregulation, with 50% of cases involving the complement regulatory genes, factor H (CFH), membrane cofactor protein (MCP; CD46), and factor I (IF). However, incomplete penetrance of mutations in each of these genes is reported. ⋯ The reported precipitating events predominantly cause endothelial injury. Discovery of these mutations has revealed important genotype-phenotype correlations. MCP-HUS has a better prognosis and a better outcome after transplantation than either CFH-HUS or IF-HUS.
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British medical bulletin · Jan 2006
ReviewIdiopathic intracranial hypertension and visual function.
Idiopathic intracranial hypertension is a relatively common condition (incidence up to 19/100 000/year in the high-risk group of obese women in reproductive age range) causing headaches with papilloedema. Detailed investigations are required to exclude other causes of raised intracranial pressure. The condition may be self-limiting or enter a chronic phase with significant morbidity because of headache and visual loss. ⋯ Management of hypertension is initially medical, utilizing a combination of managed weight reduction and diuretic therapy. Cerebrospinal fluid (CSF) diversion surgery may be required to stabilize vision. Options include neurosurgical shunting by lumbar-peritoneal of ventriculo-peritoneal routes or by optic nerve sheath fenestration or both. High category evidence from randomized trials to guide management decisions is lacking. This article sets out to guide current best practice.
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Leprosy is a granulomatous disease affecting the skin and nerves caused by Mycobacterium leprae. It continues to be a significant public health problem. Multidrug therapy (MDT) cures the infection, but immunological reactions may occur and neuropathy may lead to disability and deformity. It is important that the manifestations of the condition are recognized as early as possible so that early nerve damage can be identified and treated rapidly.
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British medical bulletin · Jan 2006
ReviewPreventing incapacity in people with musculoskeletal disorders.
Musculoskeletal disorders are among the most common causes of sickness absence, long-term incapacity for work and ill-health retirement. The number of Incapacity Benefit (IB) recipients in the United Kingdom has trebled since 1979, despite improvement in objective measures of health. Most of the trend is in non-specific conditions (largely subjective complaints, often with little objective pathology or impairment). ⋯ Rehabilitation should be directed to overcome biopsychosocial obstacles to recovery and return to work. These principles are fundamental to better clinical and occupational management and minimizing incapacity. Sickness absence and incapacity from non-specific musculoskeletal conditions could be reduced by 33-50%, but that depends on getting all stakeholders onside and a fundamental shift in thinking about these conditions-in health care, in the workplace and in society.
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Delirium is a common presentation of acute physical illness in older people. When complicating a hospital admission it is an independent predictor of poor outcomes and is poorly detected. Up to 50% of delirium in older people develops after admission to hospital. ⋯ Controlled studies demonstrate the potential to reduce incident delirium by 30-40%, and these interventions are essentially the provision of high-quality care. The routine use of risk prediction rules for all older people admitted to general hospitals would identify those at greatest risk and allow the implementation of care plans that incorporate strategies for prevention and the detection of early symptoms. There is now sufficient evidence to recommend that this should become routine practice.