@entity8 ( @entity6994 ) is characterized by recurrent episodes of macroscopic @entity811 accompanied by @entity1729 or persistent asymptomatic microscopic @entity811 with or without @entity644 . @entity6994 may involve one or more members of a family. Three generations of a cohort of 110 @entity1 with biopsy-proven @entity6994 , living in Southern Italy, were checked for urinalysis, and the relative risk (RR) of developing the disease was evaluated. A total of 19 unrelated familial, 37 suspected, and 54 sporadic cases of @entity6994 were identified. Renal survival was estimated by the Kaplan-Meier method for censored data and compared by use of the log-rank test. More than 50% of the @entity1 with @entity6994 clustered in kindred with more than two probably affected relatives. In 19 unrelated @entity6994 families, 8 had single-generation (SG) and 11 multigenerational (MG) involvement showing a prevalent vertical transmission of the trait. The RR was 16 times higher in first-degree relatives (odds ratio [OR], 16.4; 95% confidence interval [CI], 5.7 to 47.8; P < 0.0001) and >2 times higher, even if NS, in second-degree relatives (OR, 2.4; 95 % CI, 0.7 to 7.9; P = 0.145). The clinical and histologic picture of familial and sporadic @entity6994 appeared to be similar. The 20-yr renal survival rate from the apparent onset of the disease was significantly poorer in @entity1 with familial (41%) than in @entity1 with sporadic (94%) @entity6994 (P = 0.003). Furthermore, 15-yr renal survival from the time of renal biopsy was significantly worse in familial @entity6994 (P = 0.02); @entity454 was present in 64% of familial and only in 8% of @entity1 with sporadic @entity6994 . Finally, renal survival was significantly worse in @entity1 belonging to families with SG rather than with MG involvement (P = 0.03). These data show, for the first time, that familial @entity6994 may be considered a nonbenign disease that occurs frequently in first-degree relatives. Familial @entity6994 has a poorer outcome than sporadic @entity6994 . Therefore, an accurate family history and urinalysis in all family members is urgently recommended in clinical practice. This procedure might avoid late referral of subjects with persistent and underestimated @entity1494 and late diagnosis of the disease.

Increased risk of @entity454 in XXXX .

@entity1 :: ('9606', 'Species') :: ['patients'] @entity644 :: ('MESH:D011507', 'Disease') :: ['proteinuria'] @entity8 :: ('MESH:D007674', 'Disease') :: ['Primary IgA nephropathy'] @entity1494 :: ('MESH:D001745', 'Disease') :: ['urinary abnormalities'] @entity6994 :: ('60498', 'Gene') :: ['IgAN'] @entity1729 :: ('MESH:D012141', 'Disease') :: ['upper respiratory tract infections'] @entity811 :: ('MESH:D006417', 'Disease') :: ['hematuria'] @entity454 :: ('MESH:D007676', 'Disease') :: ['end-stage renal disease']
@entity8 :: (MESH:D007674,Disease) :: ['familial IgA nephropathy']
