IgA nephropathy with presentation of nephrotic syndrome at onset in children

被引:23
作者
Shima, Yuko [1 ]
Nakanishi, Koichi [1 ]
Sato, Masashi [1 ]
Hama, Taketsugu [1 ]
Mukaiyama, Hironobu [1 ]
Togawa, Hiroko [1 ]
Tanaka, Ryojiro [2 ]
Nozu, Kandai [3 ]
Sako, Mayumi [4 ]
Iijima, Kazumoto [3 ]
Suzuki, Hiroyuki [1 ]
Yoshikawa, Norishige [5 ]
机构
[1] Wakayama Med Univ, Dept Pediat, 811-1 Kimiidera, Wakayama, Wakayama 6418509, Japan
[2] Hyogo Prefectural Kobe Childrens Hosp, Dept Nephrol, Kobe, Hyogo, Japan
[3] Kobe Univ, Dept Pediat, Grad Sch Med, Kobe, Hyogo, Japan
[4] Natl Ctr Child Hlth & Dev, Div Clin Trials, Ctr Clin Res & Dev, Tokyo, Japan
[5] Wakayama Med Univ, Clin Res Ctr, Wakayama, Wakayama, Japan
关键词
IgA nephropathy; Nephrotic syndrome; End-stage renal disease; Remission of proteinuria; OXFORD CLASSIFICATION; CONTROLLED-TRIAL; PROTEINURIA; INHIBITORS; REMISSION; OUTCOMES; THERAPY; DISEASE;
D O I
10.1007/s00467-016-3502-6
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
Despite a low incidence, nephrotic syndrome (NS) can present with IgA nephropathy (IgAN). The clinical characteristics and long-term outcomes of pediatric patients with IgAN presenting with NS (NS-IgAN) at onset have not been fully elucidated. We retrospectively analyzed 426 patients, and compared clinical and pathological (Oxford) findings between those with NS-IgAN and those with non-NS-IgAN. Among 426 patients, 30 (7.0 %) had NS-IgAN. Logistic analyses showed that male sex (OR: 7.6, p = 0.0002), M1 (OR: 10.3, p = 0.002), and E1 (OR: 15.2, p = 0.0001) were significantly related to NS. The mean observation period was 6.2 +/- 3.2 years. Although NS-IgAN was associated with significantly lower renal survival than non-NS-IgAN according to Kaplan-Meier analysis (p = 0.02), renal survival of NS-IgAN was good (92.4 % at 10 years). The most significant prognostic factor for renal survival was remission of proteinuria after treatment, and NS at onset is also a significant prognostic factor for renal survival after adjusting for remission of proteinuria. Twenty children with NS-IgAN were treated with prednisolone alone, or prednisolone and immunosuppressant. Remission of proteinuria occurred in 21 patients. Three cases of NS-IgAN progressed to stage III-V chronic kidney disease at the most recent observation. They all demonstrated heavy proteinuria after the 2-year initial treatment. The significant factor for persistent proteinuria at 5 years was S1 in NS-IgAN. The most significant factor for renal survival was responsiveness to treatment, not NS itself. As modifiable acute lesions are the dominant pathological findings in NS-IgAN, histological improvements achieved by appropriate treatments can result in a favorable prognosis.
引用
收藏
页码:457 / 465
页数:9
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