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2015 ; 4
(4
): 468-79
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Current management of autosomal dominant polycystic kidney disease
#MMPMID26380198
Akoh JA
World J Nephrol
2015[Sep]; 4
(4
): 468-79
PMID26380198
show ga
Autosomal dominant polycystic kidney disease (ADPKD), the most frequent cause of
genetic renal disease affecting approximately 4 to 7 million individuals
worldwide and accounting for 7%-15% of patients on renal replacement therapy, is
a systemic disorder mainly involving the kidney but cysts can also occur in other
organs such as the liver, pancreas, arachnoid membrane and seminal vesicles.
Though computed tomography and magnetic resonance imaging (MRI) were similar in
evaluating 81% of cystic lesions of the kidney, MRI may depict septa, wall
thickening or enhancement leading to upgrade in cyst classification that can
affect management. A screening strategy for intracranial aneurysms would provide
1.0 additional year of life without neurological disability to a 20-year-old
patient with ADPKD and reduce the financial impact on society of the disease.
Current treatment strategies include reducing: cyclic adenosine monophosphate
levels, cell proliferation and fluid secretion. Several randomised clinical
trials (RCT) including mammalian target of rapamycin inhibitors, somatostatin
analogues and a vasopressin V2 receptor antagonist have been performed to study
the effect of diverse drugs on growth of renal and hepatic cysts, and on
deterioration of renal function. Prophylactic native nephrectomy is indicated in
patients with a history of cyst infection or recurrent haemorrhage or to those in
whom space must be made to implant the graft. The absence of large RCT on various
aspects of the disease and its treatment leaves considerable uncertainty and
ambiguity in many aspects of ADPKD patient care as it relates to end stage renal
disease (ESRD). The outlook of patients with ADPKD is improving and is in fact
much better than that for patients in ESRD due to other causes. This review
highlights the need for well-structured RCTs as a first step towards trying newer
interventions so as to develop updated clinical management guidelines.