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Podocyte Infolding Glomerulopathy: A Case Series Report and Literature Review
Background: Podocyte infolding glomerulopathy (PIG) is a peculiar and very rare manifestation in renal pathology. Its underlying pathogenesis mechanism and clinical characteristics remain unclear due to sparse reports. Objective: To further elucidate the clinical profile of PIG by carefully reportin...
Autores principales: | , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2023
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9918010/ https://www.ncbi.nlm.nih.gov/pubmed/36769735 http://dx.doi.org/10.3390/jcm12031088 |
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author | Feng, Yunlin Wang, Wei Zou, Yurong Chen, Tingyu Wang, Wei Li, Guisen Wang, Amanda Y. Zhang, Ping |
author_facet | Feng, Yunlin Wang, Wei Zou, Yurong Chen, Tingyu Wang, Wei Li, Guisen Wang, Amanda Y. Zhang, Ping |
author_sort | Feng, Yunlin |
collection | PubMed |
description | Background: Podocyte infolding glomerulopathy (PIG) is a peculiar and very rare manifestation in renal pathology. Its underlying pathogenesis mechanism and clinical characteristics remain unclear due to sparse reports. Objective: To further elucidate the clinical profile of PIG by carefully reporting our four cases and a comprehensive review of cases in the literature. Methods: This study retrospectively reviewed four cases of PIG from 2010 to 2022 in our centre. Clinical and pathological profiles were reported. PIG cases in the literature were searched in the MEDLINE database and analysed together with our cases. Results: Four cases of PIG identified from our centre and 40 cases from the current literature were reported. The pooled analysis of these 44 cases indicated 79.5% (35/44) were females, 93.2% (41/44) were East Asians, and 63.6% (28/44) were reported in Japan. The average age was 42.0 ± 12.5 years old. The average amount of proteinuria at the time of renal biopsy was 3.06 ± 3.2 g/day. The most reported comorbidities were connective tissue diseases, mainly systemic lupus erythematosus, and 20.5% (9/44) of the cases did not have any contaminant disease. Most of the cases (81.8%, 36/44) had been treated with immunosuppressants, of which a combination of corticosteroids and one other type of immunosuppressant was most commonly reported. In addition, 45.4% (20/44) and 34.1% (15/44) of the cases had achieved complete response and partial response, respectively, after treatment. Whole exosome sequencing indicated mutations in the INF2 gene. Conclusions: PIG is a rare condition and seen in relatively younger populations, often associated with connective tissue diseases clinically and one or two other glomerulopathies histologically. The outcomes following immunosuppressive treatment are relatively good. Mutations in INF2 might be involved in the development of PIG; however, the implications of these results need to be investigated. |
format | Online Article Text |
id | pubmed-9918010 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-99180102023-02-11 Podocyte Infolding Glomerulopathy: A Case Series Report and Literature Review Feng, Yunlin Wang, Wei Zou, Yurong Chen, Tingyu Wang, Wei Li, Guisen Wang, Amanda Y. Zhang, Ping J Clin Med Article Background: Podocyte infolding glomerulopathy (PIG) is a peculiar and very rare manifestation in renal pathology. Its underlying pathogenesis mechanism and clinical characteristics remain unclear due to sparse reports. Objective: To further elucidate the clinical profile of PIG by carefully reporting our four cases and a comprehensive review of cases in the literature. Methods: This study retrospectively reviewed four cases of PIG from 2010 to 2022 in our centre. Clinical and pathological profiles were reported. PIG cases in the literature were searched in the MEDLINE database and analysed together with our cases. Results: Four cases of PIG identified from our centre and 40 cases from the current literature were reported. The pooled analysis of these 44 cases indicated 79.5% (35/44) were females, 93.2% (41/44) were East Asians, and 63.6% (28/44) were reported in Japan. The average age was 42.0 ± 12.5 years old. The average amount of proteinuria at the time of renal biopsy was 3.06 ± 3.2 g/day. The most reported comorbidities were connective tissue diseases, mainly systemic lupus erythematosus, and 20.5% (9/44) of the cases did not have any contaminant disease. Most of the cases (81.8%, 36/44) had been treated with immunosuppressants, of which a combination of corticosteroids and one other type of immunosuppressant was most commonly reported. In addition, 45.4% (20/44) and 34.1% (15/44) of the cases had achieved complete response and partial response, respectively, after treatment. Whole exosome sequencing indicated mutations in the INF2 gene. Conclusions: PIG is a rare condition and seen in relatively younger populations, often associated with connective tissue diseases clinically and one or two other glomerulopathies histologically. The outcomes following immunosuppressive treatment are relatively good. Mutations in INF2 might be involved in the development of PIG; however, the implications of these results need to be investigated. MDPI 2023-01-30 /pmc/articles/PMC9918010/ /pubmed/36769735 http://dx.doi.org/10.3390/jcm12031088 Text en © 2023 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Article Feng, Yunlin Wang, Wei Zou, Yurong Chen, Tingyu Wang, Wei Li, Guisen Wang, Amanda Y. Zhang, Ping Podocyte Infolding Glomerulopathy: A Case Series Report and Literature Review |
title | Podocyte Infolding Glomerulopathy: A Case Series Report and Literature Review |
title_full | Podocyte Infolding Glomerulopathy: A Case Series Report and Literature Review |
title_fullStr | Podocyte Infolding Glomerulopathy: A Case Series Report and Literature Review |
title_full_unstemmed | Podocyte Infolding Glomerulopathy: A Case Series Report and Literature Review |
title_short | Podocyte Infolding Glomerulopathy: A Case Series Report and Literature Review |
title_sort | podocyte infolding glomerulopathy: a case series report and literature review |
topic | Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9918010/ https://www.ncbi.nlm.nih.gov/pubmed/36769735 http://dx.doi.org/10.3390/jcm12031088 |
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