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Çocukluk Çağı Membranöz Glomerülonefrit Tanılı Hastaların Klinik Değerlendirmesi

Year 2023, Volume: 17 Issue: 5, 341 - 346, 25.09.2023
https://doi.org/10.12956/tchd.1236249

Abstract

Amaç: Membranöz nefropati (MN), pediatrik popülasyonda erişkin dönemden daha nadir görülen bir immün kompleks hastalığıdır. MN’nin prognozu, spontan tam remisyondan son dönem böbrek hastalığına (ESRD) kadar değişkendir. Çok merkezli geniş çalışmaların olmaması, bu hastalarda tedavi seçeneklerinin ve klinik sonuçların ayrıntılı olarak incelenmesi olasılığını engellemektedir. Bu çalışma, pediatrik hastalarda MN’nin klinik bulguları, tedavisi ve prognozu hakkındaki literatür verilerine katkı sağlamayı amaçlamaktadır.
Gereç ve Yöntemler: Bu çalışmaya 13 hasta dahil edilmiştir. Çalışmaya primer ve sekonder MN glomerülonefrit tanılı hastalar dahil edilmiştir.
Bulgular: Yaş ortalaması 12.29±3.67 yıldı. Yedi (%53.8) hastada (1 olgu spontan remisyon) tam remisyon, 4 (%30.8) hastada kısmi remisyon meydana geldi. 13 hastanın 2’sinde (%15.4) böbrek yetmezliği gelişti. Son kontrolde 6 (%46.2) hastada proteinüri, 4 (%30.8) hastada mikroskobik hematüri saptandı ve 9 hasta halen düşük doz steroid kullanıyordu.
Sonuç: Mevcut bulgular, pediatrik hastalarda MN’nin prognozu ile ilişkili herhangi bir önemli risk faktörü belirlememiştir, ancak pediatrik MN ile ilgili sınırlı verilere katkı sağlayacağı düşünülmektedir. Pediyatrik popülasyonda MN’nin doğal seyri, tedavi seçenekleri ve uzun dönem sonuçları ile ilgili mevcut verilerin çoğu küçük, kontrolsüz vaka serilerinden oluşmaktadır. Bu nedenle, pediatrik MN’nin prognozu ile ilgili faktörleri açık bir şekilde aydınlatmak için daha büyük ölçekli klinik araştırmaların gerekli olduğunu düşünüyoruz.

References

  • Safar-Boueri L, Piya A, Beck LH, Ayalon, R. Membranous nephropathy: diagnosis, treatment, and monitoring in the post-PLA2R era. Pediatr Nephrol 2021; 36:19-30.
  • Zhang D, Wu Y, Zhang C, Zhang W, Zou J, Jiang G. Compared staining of the phospholipase A2 receptor in the glomeruli of Chinese adults and children with idiopathic membranous nephropathy. Pathol Res Pract 2019;215:952-6.
  • Alok A, Yadav A. Membranous Nephropathy. [Updated 2022 Jun 11]. In: StatPearls 2022. Publishing; 2022 Jan. Available from: https://www.ncbi.nlm.nih.gov/books/NBK559169/
  • Moroni G, Ponticelli C. Secondary Membranous Nephropathy. A Narrative Review. Front Med 2020;7:611317.
  • Li J, Chen B, Gao C, Huang J, Wang Y, Zhang S, , et al. Clinical and pathological features of idiopathic membranous nephropathy with focal segmental sclerosis. BMC Nephrol 2019;20:467.
  • Diaz M, Agraz I, Soler MJ. Anti-phospholipase A2 receptor antibody and spontaneous remission in membranous nephropathy. Clin Kidney J 2019;12:33–5.
  • Storrar J, Gill-Taylor T, Chinnadurai R, Chrysochou C, Poulikakos D, Rainone F, et al. A low rate of end-stage kidney disease in membranous nephropathy: A single centre study over 2 decades. PLoS ONE 2022;17:e0276053.
  • Teisseyre M, Boyer-Suavet S, Crémoni M, Brglez V, Esnault V, Seitz-Polski B. Analysis and Management of Rituximab Resistance in PLA2R1-Associated Membranous Nephropathy. Kidney Int Rep 2021;29:6:1183-8.
  • Flynn JT, Kaelber DC, Baker-Smith CM, Blowey D, Carroll AE, Daniels SR, et al. Clinical practice guideline for screening and management of high blood pressure in children and adolescents. Pediatrics 2017;140:e20171904.
  • Levey AS, Coresh J, Balk E, Kausz AT, Levin A, Steffes MW, et al. National Kidney Foundation practice guidelines for chronic kidney disease: evaluation, classification, and stratification. Ann Intern Med 2003;139:137–47.
  • Keri KC, Blumenthal S, Kulkarni V, Beck L, Chongkrairatanakul T. Primary membranous nephropathy: comprehensive review and historical perspective. Postgrad Med J 2019;95:23-31.
  • Couser WG. Primary membranous nephropathy. Clin J Am Soc Nephrol 2017;12:983–97.
  • Cattran D. Management of membranous nephropathy: when and what for treatment. J Am Soc Nephrol 2005;16:1188–94.
  • Luo J, Yuan Y, Tian J, Zhou Z, Su C, Yang F, et al. Clinicopathological Characteristics and Outcomes of PLA2R-Associated Membranous Nephropathy in Seropositive Patients Without PLA2R Staining on Kidney Biopsy. Am J Kidney Dis 2022;80:364-72.
  • Beck LH, Salant DJ. Membranous nephropathy: recent travels and new roads ahead. Kidney Int 2010;77:765–70.
  • Van De Logt AE, Justino J, Vink CH, van den Brand J, Debiec H, Lambeau G, et al. Anti-PLA2R1 antibodies as prognostic biomarker in membranous nephropathy. Kidney Int Rep 2021;6:1677-86.
  • Liu W, Gao C, Dai H, Zheng Y, Dong Z, Gao Y, et al. Immunological pathogenesis of membranous nephropathy: focus on PLA2R1 and its role. Front Immunol 2019;10:1809.
  • Valentini RP, Mattoo TK, Kapur G, Imam A Membranous glomerulonephritis: treatment response and outcome in children. Pediatr Nephrol 2009;24:301–8.
  • Malatesta-Muncher R, Eldin KW, Beck LH Jr, Michael M. Idiopathic membranous nephropathy in children treated with rituximab: report of two cases. Pediatr Nephrol 2018;33:1089–92.
  • Sabiu G, Podesta MA. Membranous Nephropathy: It Is Time to Go Back to the Future. Nephron 2021;145:721-7.
  • Cattran DC, Brenchley PE. Membranous nephropathy: Integrating basic science into improved clinical management. Kidney Int 2017;91:566–74.
  • Salant DJ, Cattran DC. Membranous nephropathy. In: J. Floege, RJ Johnson, J. Feehally. Comprehensive Clinical Nephrology 2015;6:239–51.
  • Pei Y, Cattran D, Greenwood C. Predicting chronic renal insufficiency in idiopathic 
membranous glomerulonephritis. Kidney Int 1992;42:960–6.

Clinical Evaluation of Pediatric Patients Diagnosed with Membranous Glomerulonephritis

Year 2023, Volume: 17 Issue: 5, 341 - 346, 25.09.2023
https://doi.org/10.12956/tchd.1236249

Abstract

Objective: Membranous nephropathy (MN) is a rare immune complex disease in pediatric population then adults. The prognosis of MN is variable, ranging from spontaneous complete remission to end-stage renal disease (ESRD). The lack of large multicenter studies precludes the possibility of examining in detail the treatment options and clinical outcomes in these patients. The present study aimed to expand the literature on the clinical findings, treatment, and prognosis of MN in pediatric patients.
Material and Methods: This single-center retrospective study included 13 patients with a diagnosis of primary and secondary membranous nephropathy.
Results: The mean age of the sample was 12.29±3.67 years. Complete remission occurred in 7 (53.8%) patients (of which 1 case was spontaneous remission), and partial remission occurred in 4 (30.8%) patients. In long-term follow-ups; one patient had chronic kidney disease (CKD) and one patient had end-stage renal disease (ESRD). At the last-follow up, proteinuria was noted in 6 (46.2%) patients and microscopic hematuria was noted in 4 (30.8%) and 9 patients were still using low-dose steroids.
Conclusion: The current findings have not identified any significant risk factors associated with the prognosis of MN in pediatric patients, but are thought to contribute to the limited data on pediatric MN. Most of the available data on the natural history, treatment options, and long-term outcomes of MN in the pediatric population consists of small, uncontrolled case series. Therefore, we think that larger-scale clinical trials are necessary to clearly elucidate the factors related to the prognosis of pediatric MN.

References

  • Safar-Boueri L, Piya A, Beck LH, Ayalon, R. Membranous nephropathy: diagnosis, treatment, and monitoring in the post-PLA2R era. Pediatr Nephrol 2021; 36:19-30.
  • Zhang D, Wu Y, Zhang C, Zhang W, Zou J, Jiang G. Compared staining of the phospholipase A2 receptor in the glomeruli of Chinese adults and children with idiopathic membranous nephropathy. Pathol Res Pract 2019;215:952-6.
  • Alok A, Yadav A. Membranous Nephropathy. [Updated 2022 Jun 11]. In: StatPearls 2022. Publishing; 2022 Jan. Available from: https://www.ncbi.nlm.nih.gov/books/NBK559169/
  • Moroni G, Ponticelli C. Secondary Membranous Nephropathy. A Narrative Review. Front Med 2020;7:611317.
  • Li J, Chen B, Gao C, Huang J, Wang Y, Zhang S, , et al. Clinical and pathological features of idiopathic membranous nephropathy with focal segmental sclerosis. BMC Nephrol 2019;20:467.
  • Diaz M, Agraz I, Soler MJ. Anti-phospholipase A2 receptor antibody and spontaneous remission in membranous nephropathy. Clin Kidney J 2019;12:33–5.
  • Storrar J, Gill-Taylor T, Chinnadurai R, Chrysochou C, Poulikakos D, Rainone F, et al. A low rate of end-stage kidney disease in membranous nephropathy: A single centre study over 2 decades. PLoS ONE 2022;17:e0276053.
  • Teisseyre M, Boyer-Suavet S, Crémoni M, Brglez V, Esnault V, Seitz-Polski B. Analysis and Management of Rituximab Resistance in PLA2R1-Associated Membranous Nephropathy. Kidney Int Rep 2021;29:6:1183-8.
  • Flynn JT, Kaelber DC, Baker-Smith CM, Blowey D, Carroll AE, Daniels SR, et al. Clinical practice guideline for screening and management of high blood pressure in children and adolescents. Pediatrics 2017;140:e20171904.
  • Levey AS, Coresh J, Balk E, Kausz AT, Levin A, Steffes MW, et al. National Kidney Foundation practice guidelines for chronic kidney disease: evaluation, classification, and stratification. Ann Intern Med 2003;139:137–47.
  • Keri KC, Blumenthal S, Kulkarni V, Beck L, Chongkrairatanakul T. Primary membranous nephropathy: comprehensive review and historical perspective. Postgrad Med J 2019;95:23-31.
  • Couser WG. Primary membranous nephropathy. Clin J Am Soc Nephrol 2017;12:983–97.
  • Cattran D. Management of membranous nephropathy: when and what for treatment. J Am Soc Nephrol 2005;16:1188–94.
  • Luo J, Yuan Y, Tian J, Zhou Z, Su C, Yang F, et al. Clinicopathological Characteristics and Outcomes of PLA2R-Associated Membranous Nephropathy in Seropositive Patients Without PLA2R Staining on Kidney Biopsy. Am J Kidney Dis 2022;80:364-72.
  • Beck LH, Salant DJ. Membranous nephropathy: recent travels and new roads ahead. Kidney Int 2010;77:765–70.
  • Van De Logt AE, Justino J, Vink CH, van den Brand J, Debiec H, Lambeau G, et al. Anti-PLA2R1 antibodies as prognostic biomarker in membranous nephropathy. Kidney Int Rep 2021;6:1677-86.
  • Liu W, Gao C, Dai H, Zheng Y, Dong Z, Gao Y, et al. Immunological pathogenesis of membranous nephropathy: focus on PLA2R1 and its role. Front Immunol 2019;10:1809.
  • Valentini RP, Mattoo TK, Kapur G, Imam A Membranous glomerulonephritis: treatment response and outcome in children. Pediatr Nephrol 2009;24:301–8.
  • Malatesta-Muncher R, Eldin KW, Beck LH Jr, Michael M. Idiopathic membranous nephropathy in children treated with rituximab: report of two cases. Pediatr Nephrol 2018;33:1089–92.
  • Sabiu G, Podesta MA. Membranous Nephropathy: It Is Time to Go Back to the Future. Nephron 2021;145:721-7.
  • Cattran DC, Brenchley PE. Membranous nephropathy: Integrating basic science into improved clinical management. Kidney Int 2017;91:566–74.
  • Salant DJ, Cattran DC. Membranous nephropathy. In: J. Floege, RJ Johnson, J. Feehally. Comprehensive Clinical Nephrology 2015;6:239–51.
  • Pei Y, Cattran D, Greenwood C. Predicting chronic renal insufficiency in idiopathic 
membranous glomerulonephritis. Kidney Int 1992;42:960–6.
There are 23 citations in total.

Details

Primary Language English
Subjects ​Internal Diseases
Journal Section ORIGINAL ARTICLES
Authors

Deniz Karakaya 0000-0001-7720-4923

Fatma Yazılıtaş 0000-0001-6483-8978

Evrim Kargın Çakıcı 0000-0002-1697-6206

Tülin Güngör 0000-0002-5881-1565

Evra Çelikkaya 0000-0003-2695-2045

Mehmet Bülbül 0000-0001-9007-9653

Early Pub Date May 24, 2023
Publication Date September 25, 2023
Submission Date January 17, 2023
Published in Issue Year 2023 Volume: 17 Issue: 5

Cite

Vancouver Karakaya D, Yazılıtaş F, Kargın Çakıcı E, Güngör T, Çelikkaya E, Bülbül M. Clinical Evaluation of Pediatric Patients Diagnosed with Membranous Glomerulonephritis. Turkish J Pediatr Dis. 2023;17(5):341-6.


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