Frequency of elevated urinary beta 2-microglobulin levels in relatives of patients with asymptomatic...

We studied urinary beta 2-microglobulin levels in a total of 29 apparently healthy relatives (aged 0.8-70 years) of 8 male patients with asymptomatic low-molecular-weight proteinuria in six families. The frequency of levels above the age- and sex-associated 95% confidence limit was 7 of 29 (24%), 4 of 12 (33%) in first-degree relatives, 2 of 6 (33%) fathers, and 2 of 6 (33%) mothers. These frequencies were significantly above those in the general population (P less than 0.01, by a normal distribution test, a binomial distribution, and Poisson distribution test for the sample proportion). The increased frequency in fathers argues against an X-linked pattern of inheritance for this entity, suggesting that there is heterogeneity in the inheritance.