The goal of the Microalbuminuria in Untreated Boys with Alport Syndrome study is to gather information about critical clinical time points such as when patients with small amounts of protein (microalbuminuria) in their urine progress to larger amounts (overt proteinuria). Large amounts of protein in the urine is often an early sign of kidney disease.

Information needs to be collected in boys who are not taking medications known as angiotensin converting enzyme inhibitor (ACEI) or angiotensin receptor blocker (ARB) in order to obtain accurate data about the length of time between the onset of microalbuminuria and the start of overt proteinuria. This new information will give physicians a better understanding of how to treat patients with Alport syndrome.

The information we gather by conducting this study will aid in planning future clinical trials because the identification of time points in disease progression, such as microalbuminuria and overt proteinuria, could reduce the time necessary to show a clinical benefit of a new treatment option.

The study has been approved by the University of Minnesota's Institutional Review Board.

Study Aims

1. To determine the average ages of onset of microalbuminuria and overt proteinuria in untreated boys with Alport syndrome
2. To determine the average duration of microalbuminuria before transition to overt proteinuria in untreated boys with Alport syndrome

This study does not involve treatment and is anticipated to last 3-5 years.

Inclusion Criteria:

• Diagnosis of Alport syndrome, confirmed by skin biopsy, kidney biopsy, or molecular genetic analysis
• Diagnosis of Alport syndrome, based on presence of hematuria and confirmed diagnosis of Alport syndrome in a first-degree relative
• Male gender
• Absence of overt proteinuria, defined as urine protein:creatinine ratio less than 0.2 mg/mg
• Subject is not currently receiving treatment with an angiotensin converting enzyme inhibitor (ACEI) or angiotensin receptor blocker (ARB)

Exclusion Criteria:

• Female gender
• Presence of overt proteinuria
• Current treatment with ACEI or ARB
• End-stage kidney disease (on dialysis or kidney transplant recipient)

The study population is comprised of approximately 30 males ranging in age from 0 - 18 at the time of enrollment.

2007-2009