Alport syndrome epidemiology and demographics

Alport’s syndrome or hereditary nephritis is considered one of the most common familial nephropathies. Its incidence is 1 in 50,000 – 1/100,000 live births in the United States.^[1] Since the original description, male gender has been noted to be associated with worse severity, suggesting maternal transmittance. Onset of symptoms often starts during infancy in X-linked Alport’s syndrome; patients reach ESRD during adolescence.^[1] Patients with autosomal recessive Alport’s syndrome sometimes progress less rapidly; although they may still progress to ESRD early, they may reach ESRD during adult life.^[1]

Worldwide, Alport’s syndrome is not predominant in a specific race, ethnicity, or within a geographic distribution.^[2][3][4] In the USA, however, Western states have a significantly higher rate of Alport’s syndrome and is up to two-fold more common than other regions within the USA.

Although a hereditary disorder, spontaneous mutations comprise approximately 15-20% of new cases of Alport’s syndrome.^[5]

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