Suh Jin-Soon, Jang Kyung Mi, Hyun Hyesun, Cho Myung Hyun, Lee Joo Hoon, Park Young Seo, Oh Jae Hyuk, Kim Ji Hong, Yoo Kee Hwan, Chung Woo Yeong, Kim Seong Heon, Kim Keehyuck, Lee Dae Yeol, Lee Jung Won, Cho Min Hyun, Park Hyewon, Koo Ja Wook, Han Kyoung Hee, Yang Eun Mi, Lee Keum Hwa, Shin Jae Il, Cho Heeyeon, Kim Kyo Soon, Ha Il-Soo, Park Yong Hoon, Kang Hee Gyung
Departments of Pediatrics, Bucheon St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Bucheon 14647, Korea.
Yeungnam University Hospital, Daegu 42415, Korea.
J Clin Med. 2020 Jun 30;9(7):2058. doi: 10.3390/jcm9072058.
Immunoglobulin A nephropathy (IgAN) is one of the most common primary glomerulopathies diagnosed in children and adolescents. This study aimed to evaluate the clinical features in and outcomes of pediatric IgAN over the last 30 years. Patients who were diagnosed before age of 18 at 20 centers in Korea were evaluated retrospectively. Of the 1154 patients (768 males, 386 females) with a median follow-up of 5 years, 5.6% ( = 65) progressed to stage 3-5 chronic kidney disease (CKD). The 10- and 20-year CKD-free survival rates were 91.2% and 75.6%, respectively. Outcomes did not differ when comparing those in Korea who were diagnosed prior to versus after the year 2000. On multivariate analysis, combined asymptomatic hematuria and proteinuria as presenting symptoms and decreased renal function at the time of biopsy were associated with progression to CKD, while remission of proteinuria was negatively associated with this outcome. Patients who presented with gross hematuria or nephrotic syndrome tended toward positive outcomes, especially if they ultimately achieved remission. While remission of proteinuria might imply that the disease is inherently less aggressive, it also can be achieved by management. Therefore, more aggressive management might be required for pediatric-onset IgAN.
免疫球蛋白A肾病（IgAN）是儿童和青少年中最常见的原发性肾小球疾病之一。本研究旨在评估过去30年中儿童IgAN的临床特征和预后。对韩国20个中心18岁之前确诊的患者进行回顾性评估。在1154例患者（768例男性，386例女性）中，中位随访时间为5年，5.6%（n = 65）进展为3-5期慢性肾脏病（CKD）。10年和20年无CKD生存率分别为91.2%和75.6%。比较2000年之前和之后在韩国确诊的患者，结果无差异。多因素分析显示，活检时出现无症状血尿和蛋白尿合并症以及肾功能下降与进展为CKD相关，而蛋白尿缓解与该结果呈负相关。出现肉眼血尿或肾病综合征的患者预后倾向于良好，尤其是如果他们最终实现缓解。虽然蛋白尿缓解可能意味着疾病本身侵袭性较小，但也可以通过治疗实现。因此，对于儿童期发病的IgAN可能需要更积极的治疗。
