Monitoring for microalbuminuria and proteinuria should be initiated by age 1 year in at risk children, or as soon as a diagnosis of Alport syndrome is established, and repeated at least annually.
Affected individuals with overt proteinuria (urine protein-creatinine ratio persistently > 0.2 mg/mg, or urinary protein excretion > 4 mg/m2/h in a timed collection) should receive treatment with ACE inhibitor therapy.
Treatment with ACE inhibitor therapy should be considered in affected boys with microalbuminuria in whom the risk of ESRD by age 30 is high.
Second line treatment with an ARB (losartan) or aldosterone inhibition (spironolactone).
Comments by evaluators:
This guideline is for children with Alport syndrome.
Very little evidence. Mainly expert opinion.
The authors of this manuscript are paediatric and adult nephrologists. Pharmacology and genetics input would be useful.