1. Characteristics and outcomes of glomerulonephritis with membranoproliferative pattern in children
- Author
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Yanyan Qian, Xiaoshan Tang, Huijun Wang, Jia-Yan Feng, Jiaojiao Liu, Bingbing Wu, Xiaoyan Fang, Hong Xu, Yihui Zhai, Li Sun, Jing Chen, Fengfang Wei, Qian Shen, Linan Xu, Jialu Liu, Jia Rao, and Haimei Liu
- Subjects
medicine.medical_specialty ,business.industry ,Pediatrics, Perinatology and Child Health ,medicine ,Original Article ,Glomerulonephritis ,medicine.disease ,business ,Dermatology - Abstract
BACKGROUND: Membranoproliferative glomerulonephritis (MPGN) is a rare histopathologic pattern of glomerular injury with limited studies in pediatric patients. Characteristics and outcomes of children with MPGN have also remained to be further explored. METHODS: We retrospectively reviewed the clinicopathological features, genetic findings, treatments and outcomes in 17 pediatric patients pathologically diagnosed with MPGN from 2007 to 2020 in the Children’s National Medical Center in China. RESULTS: Median age at disease onset was 9.9 years (IQR, 5.6–11.9 years). Most of the patients (12/17) had nephrotic range of proteinuria, and nephritic-nephrotic syndrome was the most common clinical presentation (35.2%). Secondary causes were identified in eight patients including hepatitis B virus (HBV) infection (n=4), methylmalonic acidemia (MMA, n=2), rheumatoid arthritis (RA, n=1) and Aymé-Gripp Syndrome (n=1). The nine patients with primary MPGN were further identified as immune-complex mediated MPGN (n=8), and unclassifiable MPGN (U-MGPN, n=1). Genetic analyses identified pathogenic variants of MMACHC gene in two cases of MMA and established the diagnosis for Aymé-Gripp syndrome in one case with a de novo variant of MAF gene. Comparing study between the complete or partial remission group (n=8) and non-response group (n=9) showed a significant difference in the timing of renal biopsy (P
- Published
- 2021