Table 4.

Recommended Surveillance for Individuals with Alport Syndrome

System/
Concern		EvaluationFrequency
Renal Regular follow up by nephrologist to incl urinalysis, renal function assessment, & blood pressure determination 1
  • Annually if urine microalbumin-creatinine ratio <30 mg/g or urine protein-creatinine ratio <0.2 mg/mg
  • Every 6 mos if urine microalbumin-creatinine ratio >30 mg/g or urine protein-creatinine ratio >0.2 mg/mg
For at-risk transplant recipients 2: monitor for development of anti-glomerular basement membrane antibody-mediated glomerulonephritis.Monthly for 1st 12 mos post transplant
Hearing Audiologic eval starting at age 6-7 yrsEvery 1-2 yrs
Vision Monitor for maculopathy, anterior lenticonus, corneal erosions, & cataracts.Starting in adolescence in males w/a truncating pathogenic variant of COL4A5 & in persons w/ARAS; repeat every 1-2 yrs.
Cardiac Cardiac eval for aortic dilatation (for males w/XLAS) as part of transplant preparationEchocardiogram follow-up interval as determined by findings & directed by cardiologist
1.

Although most females with XLAS exhibit only asymptomatic microhematuria, there can be a significant risk for progression to ESRD [Jais et al 2003]. For this reason, all women with a diagnosis of Alport syndrome need to be monitored regularly for the development of proteinuria and hypertension.

2.

Males with XLAS and a truncating pathogenic variant in COL4A5 and individuals with ARAS

From: Alport Syndrome

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