دورية أكاديمية
أعرض في EDS

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

التفاصيل البيبلوغرافية
العنوان: Long-term follow-up including extensive complement analysis of a pediatric C3 glomerulopathy cohort.
المؤلفون: Michels MAHM; Department of Pediatric Nephrology, Amalia Children's Hospital, Radboud University Medical Center, Radboud Institute for Molecular Life Sciences, Nijmegen, The Netherlands. marloes.michels@radboudumc.nl., Wijnsma KL; Department of Pediatric Nephrology, Amalia Children's Hospital, Radboud University Medical Center, Radboud Institute for Molecular Life Sciences, Nijmegen, The Netherlands., Kurvers RAJ; Department of Pediatric Nephrology, Maastricht University Medical Center, Maastricht, The Netherlands., Westra D; Department of Pediatric Nephrology, Amalia Children's Hospital, Radboud University Medical Center, Radboud Institute for Molecular Life Sciences, Nijmegen, The Netherlands., Schreuder MF; Department of Pediatric Nephrology, Amalia Children's Hospital, Radboud University Medical Center, Radboud Institute for Molecular Life Sciences, Nijmegen, The Netherlands., van Wijk JAE; Department of Pediatric Nephrology, Emma Children's Hospital, Amsterdam University Medical Centers, Amsterdam, The Netherlands., Bouts AHM; Department of Pediatric Nephrology, Emma Children's Hospital, Amsterdam University Medical Centers, Amsterdam, The Netherlands., Gracchi V; Department of Pediatric Nephrology, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands., Engels FAPT; Department of Pediatric Nephrology, Maastricht University Medical Center, Maastricht, The Netherlands., Keijzer-Veen MG; Department of Pediatric Nephrology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht, The Netherlands., Dorresteijn EM; Department of Pediatric Nephrology, Sophia Children's Hospital, Erasmus Medical Center, Rotterdam, The Netherlands., Volokhina EB; Department of Pediatric Nephrology, Amalia Children's Hospital, Radboud University Medical Center, Radboud Institute for Molecular Life Sciences, Nijmegen, The Netherlands.; Department of Laboratory Medicine, Radboud University Medical Center, Nijmegen, The Netherlands., van den Heuvel LPWJ; Department of Pediatric Nephrology, Amalia Children's Hospital, Radboud University Medical Center, Radboud Institute for Molecular Life Sciences, Nijmegen, The Netherlands.; Department of Laboratory Medicine, Radboud University Medical Center, Nijmegen, The Netherlands.; Department of Pediatrics/Pediatric Nephrology and Department of Development and Regeneration, University Hospitals Leuven, Leuven, Belgium., van de Kar NCAJ; Department of Pediatric Nephrology, Amalia Children's Hospital, Radboud University Medical Center, Radboud Institute for Molecular Life Sciences, Nijmegen, The Netherlands.
المصدر: Pediatric nephrology (Berlin, Germany) [Pediatr Nephrol] 2022 Mar; Vol. 37 (3), pp. 601-612. Date of Electronic Publication: 2021 Sep 02.
نوع المنشور: Journal Article; Research Support, Non-U.S. Gov't
اللغة: English
بيانات الدورية: Publisher: Springer International Country of Publication: Germany NLM ID: 8708728 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1432-198X (Electronic) Linking ISSN: 0931041X NLM ISO Abbreviation: Pediatr Nephrol Subsets: MEDLINE
أسماء مطبوعة: Publication: Berlin : Springer International
Original Publication: Berlin : Springer International, c1987-
مواضيع طبية MeSH: Glomerulonephritis* , Glomerulonephritis, Membranoproliferative*/pathology , Kidney Diseases*, Child ; Complement C3 ; Complement C3 Nephritic Factor ; Complement Pathway, Alternative ; Female ; Follow-Up Studies ; Humans ; Male ; Properdin
مستخلص: 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) were detected in 20 patients and remained detectable over time despite immunosuppressive treatment. At presentation, low serum C3 levels were detected in 84% of all patients. During follow-up, in about 50% of patients, all of them C3NeF-positive, C3 levels remained low. Linear mixed model analysis showed that C3GN patients had higher soluble C5b-9 (sC5b-9) and lower properdin levels compared to DDD patients. With a median follow-up of 52 months, an overall benign outcome was observed with only six patients with eGFR below 90 ml/min/1.73 m 2 at last follow-up.
Conclusions: We extensively described clinical and laboratory findings including complement features of an exclusively pediatric C3G cohort. Outcome was relatively benign, persistent low C3 correlated with C3NeF presence, and C3GN was associated with higher sC5b-9 and lower properdin levels. Prospective studies are needed to further elucidate the pathogenic mechanisms underlying C3G and guide personalized medicine with complement therapeutics.
(© 2021. The Author(s).)
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فهرسة مساهمة: Keywords: C3 glomerulonephritis; C3 glomerulopathy; C3 nephritic factor; Children; Complement system; Dense deposit disease
المشرفين على المادة: 0 (Complement C3)
0 (Complement C3 Nephritic Factor)
11016-39-0 (Properdin)
تواريخ الأحداث: Date Created: 20210903 Date Completed: 20220415 Latest Revision: 20220415
رمز التحديث: 20231215
مُعرف محوري في PubMed: PMC8921070
DOI: 10.1007/s00467-021-05221-6
PMID: 34476601
قاعدة البيانات: MEDLINE
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