Journal of nephrology
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Journal of nephrology · Nov 2002
Hyperbaric oxygen therapy for calcific uremic arteriolopathy: a case series.
Calcific uremic arteriolopathy (CUA), also referred to as calciphylaxis, is a syndrome of small vessel calcification of unknown etiology causing painful violaceous skin lesions that progress to non-healing ulcers and gangrene. It is observed mainly in patients with end-stage renal disease, is associated with high morbidity and mortality and has no standard treatment at the present time. Although parathyroidectomy (PTX) has been advocated in some cases, other studies have not found this effective. ⋯ In conclusion, CUA appears to result from a multitude of predisposing and/or sensitizing events that are commonly present in the uremic milieu. The specific factors that induce this disorder in an individual patient are not known. The present retrospective study supports a role of HOT in many cases of CUA, especially considering that, in the absence of severe II(nd) HPTH, there are very few therapeutic options.
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The clinical spectrum of monogenic renal diseases is wide ranging, from autosomal dominant polycystic kidney disease to very rare inherited disorders. The genes involved in most of these diseases have been identified. ⋯ The challenges of the future will be to understand phenotypic variability and the molecular mechanisms of disease, and to design pharmacological tools to stop or retard its progression. The post-gene era has begun and the field of research in monogenic disorders is wide open.