BACKGROUND: To clarify the clinical manifestations of pediatric complement component C3 glomerulonephritis (C3GN), we retrospectively evaluated differences in the clinicopathological findings and prognosis between C3GN and immune-complex-mediated membranoproliferative glomerulonephritis (IC-MPGN). METHODS: Thirty-seven patients diagnosed with "idiopathic MPGN" were enrolled in this retrospective study. The patients were divided into two groups, with Group 1 consisting of 19 patients diagnosed with IC-MPGN and Group 2 consisting of 18 patients diagnosed with C3GN. The clinical findings and the prognosis were investigated for both groups. RESULTS: Thirteen patients in Group 2 were identified by mandatory annual school screening for urinary abnormalities. The incidence of macro-hematuria and the frequency of low serum C4 values were lower in Group 2 patients than in Group 1 patients. At the time of the second renal biopsy, urinary protein excretion, incidence of hematuria, frequency of low serum C3 values, and scores for mesangial proliferation, glomerular sclerosis, and interstitial fibrosis were higher in Group 2 patients than in Group 1 patients. At the most recent follow-up examination, the number of patients categorized as non-responding or with end-stage renal disease was higher in Group 2 patients than in Group 1 patients. CONCLUSIONS: Our results suggest that the treatment response and prognosis of patients with C3GN are worse than those of patients with IC-mediated MPGN. Therefore, in the clinical context regarding treatment options and prognosis, it may be useful to classify idiopathic MPGN as C3GN or IC-MPGN. In addition, long-term follow-up of C3GN is necessary.
BACKGROUND: To clarify the clinical manifestations of pediatric complement component C3 glomerulonephritis (C3GN), we retrospectively evaluated differences in the clinicopathological findings and prognosis between C3GN and immune-complex-mediated membranoproliferative glomerulonephritis (IC-MPGN). METHODS: Thirty-seven patients diagnosed with "idiopathic MPGN" were enrolled in this retrospective study. The patients were divided into two groups, with Group 1 consisting of 19 patients diagnosed with IC-MPGN and Group 2 consisting of 18 patients diagnosed with C3GN. The clinical findings and the prognosis were investigated for both groups. RESULTS: Thirteen patients in Group 2 were identified by mandatory annual school screening for urinary abnormalities. The incidence of macro-hematuria and the frequency of low serum C4 values were lower in Group 2 patients than in Group 1 patients. At the time of the second renal biopsy, urinary protein excretion, incidence of hematuria, frequency of low serum C3 values, and scores for mesangial proliferation, glomerular sclerosis, and interstitial fibrosis were higher in Group 2 patients than in Group 1 patients. At the most recent follow-up examination, the number of patients categorized as non-responding or with end-stage renal disease was higher in Group 2 patients than in Group 1 patients. CONCLUSIONS: Our results suggest that the treatment response and prognosis of patients with C3GN are worse than those of patients with IC-mediated MPGN. Therefore, in the clinical context regarding treatment options and prognosis, it may be useful to classify idiopathic MPGN as C3GN or IC-MPGN. In addition, long-term follow-up of C3GN is necessary.
Authors: Andrew S Bomback; Richard J Smith; Gaetano R Barile; Yuzhou Zhang; Eliot C Heher; Leal Herlitz; M Barry Stokes; Glen S Markowitz; Vivette D D'Agati; Pietro A Canetta; Jai Radhakrishnan; Gerald B Appel Journal: Clin J Am Soc Nephrol Date: 2012-03-08 Impact factor: 8.237
Authors: Jean Hou; Glen S Markowitz; Andrew S Bomback; Gerald B Appel; Leal C Herlitz; M Barry Stokes; Vivette D D'Agati Journal: Kidney Int Date: 2013-09-25 Impact factor: 10.612
Authors: Peter F Zipfel; Christine Skerka; Qian Chen; Thorsten Wiech; Tim Goodship; Sally Johnson; Veronique Fremeaux-Bacchi; Clara Nester; Santiago Rodríguez de Córdoba; Marina Noris; Matthew Pickering; Richard Smith Journal: Mol Immunol Date: 2015-04-28 Impact factor: 4.407
Authors: Rubén Martínez-Barricarte; Meike Heurich; Francisco Valdes-Cañedo; Eduardo Vazquez-Martul; Eva Torreira; Tamara Montes; Agustín Tortajada; Sheila Pinto; Margarita Lopez-Trascasa; B Paul Morgan; Oscar Llorca; Claire L Harris; Santiago Rodríguez de Córdoba Journal: J Clin Invest Date: 2010-09-13 Impact factor: 14.808
Authors: Yuzhou Zhang; Nicole C Meyer; Kai Wang; Carla Nishimura; Kathy Frees; Michael Jones; Louis M Katz; Sanjeev Sethi; Richard J H Smith Journal: Clin J Am Soc Nephrol Date: 2012-01-05 Impact factor: 8.237
Authors: Daniel P Gale; Elena Goicoechea de Jorge; H Terence Cook; Rubén Martinez-Barricarte; Andreas Hadjisavvas; Adam G McLean; Charles D Pusey; Alkis Pierides; Kyriacos Kyriacou; Yiannis Athanasiou; Konstantinos Voskarides; Constantinos Deltas; Andrew Palmer; Véronique Frémeaux-Bacchi; Santiago Rodriguez de Cordoba; Patrick H Maxwell; Matthew C Pickering Journal: Lancet Date: 2010-08-25 Impact factor: 79.321
Authors: Agustín Tortajada; Hugo Yébenes; Cynthia Abarrategui-Garrido; Jaouad Anter; Jesús M García-Fernández; Rubén Martínez-Barricarte; María Alba-Domínguez; Talat H Malik; Rafael Bedoya; Rocío Cabrera Pérez; Margarita López Trascasa; Matthew C Pickering; Claire L Harris; Pilar Sánchez-Corral; Oscar Llorca; Santiago Rodríguez de Córdoba Journal: J Clin Invest Date: 2013-06 Impact factor: 14.808
Authors: Sanjeev Sethi; Fernando C Fervenza; Yuzhou Zhang; Ladan Zand; Nicole C Meyer; Nicolò Borsa; Samih H Nasr; Richard J H Smith Journal: Kidney Int Date: 2012-12-12 Impact factor: 10.612
Authors: Keri A Drake; Natalie Ellington; Jyothsna Gattineni; Jose R Torrealba; Allen R Hendricks Journal: Pediatr Nephrol Date: 2019-10-30 Impact factor: 3.714