This protocol addresses female patients with X-linked Alport syndrome — a sub-population in whom clinically significant renal disease can develop and in whom nephroprotective intervention is specifically indicated.
Females with X-linked Alport syndrome can present with hypertension, proteinuria, and other risk factors associated with renal failure progression. Renin-angiotensin system (RAS) antagonists are nephroprotective in this group and are indicated when these clinical features are present.
The regimen for this scenario involves renin-angiotensin system (RAS) antagonist therapy, with defined criteria governing patient selection and the timing of initiation — including consideration of whether to act before specific clinical markers have appeared.
DOI: 10.1681/ASN.2012020148
Renin-angiotensin system antagonists are nephroprotective in females with X-linked Alport syndrome and should be used to treat those with hypertension, proteinuria, and other risk factors for renal failure progression.
Again preliminary support, but no evidence, suggests a beneficial effect for the initiation of ACE inhibitor treatment even before the onset of proteinuria.
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