Long-term follow-up including extensive complement analysis of a pediatric C3 glomerulopathy cohort.

ABSTRACT:

Background

C3 glomerulopathy (C3G) is a rare kidney disorder characterized by predominant glomerular depositions of complement C3. C3G can be subdivided into dense deposit disease (DDD) and C3 glomerulonephritis (C3GN). This study describes the long-term follow-up with extensive complement analysis of 29 Dutch children with C3G.

Methods

Twenty-nine C3G patients (19 DDD, 10 C3GN) diagnosed between 1992 and 2014 were included. Clinical and laboratory findings were collected at presentation and during follow-up. Specialized assays were used to detect rare variants in complement genes and measure complement-directed autoantibodies and biomarkers in blood.

Results

DDD patients presented with lower estimated glomerular filtration rate (eGFR). C3 nephritic factors (C3NeFs) w

SUBMITTER: Michels MAHM 

PROVIDER: S-EPMC8921070 | biostudies-literature | 2022 Mar

REPOSITORIES: biostudies-literature