Mastrangelo Antonio, Serafinelli Jessica, Giani Marisa, Montini Giovanni
Pediatric Nephrology, Dialysis and Transplant Unit, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Milan, Italy.
Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy.
Front Pediatr. 2020 May 12;8:205. doi: 10.3389/fped.2020.00205. eCollection 2020.
The kidney is often the target of immune system dysregulation in the context of primary or systemic disease. In particular, the glomerulus represents the anatomical entity most frequently involved, generally as the expression of inflammatory cell invasion or circulant or immune-complex deposition. Glomerulonephritis is the most common clinical and pathological manifestation of this involvement. There are no universally accepted classifications for glomerulonephritis. However, recent advances in our understanding of the pathophysiological mechanisms suggest the assessment of immunological features, biomarkers, and genetic analysis. At the same time, more accurate and targeted therapies have been developed. Data on pediatric glomerulonephritis are scarce and often derived from adult studies. In this review, we update the current understanding of the etiologic events and genetic factors involved in the pathogenesis of pediatric immunologically mediated primitive forms of glomerulonephritis, together with the clinical spectrum and prognosis. Possible new therapeutic targets are also briefly discussed.
在原发性或全身性疾病的背景下，肾脏常常是免疫系统失调的靶器官。特别是肾小球，是最常受累的解剖结构，通常表现为炎症细胞浸润或循环免疫复合物或免疫复合物沉积。肾小球肾炎是这种受累最常见的临床和病理表现。目前尚无普遍接受的肾小球肾炎分类方法。然而，我们对病理生理机制认识的最新进展提示了对免疫特征、生物标志物和基因分析的评估。与此同时，已经开发出了更准确、更有针对性的治疗方法。关于儿童肾小球肾炎的数据很少，且往往来自成人研究。在这篇综述中，我们更新了目前对儿童免疫介导的原发性肾小球肾炎发病机制中涉及的病因学事件和遗传因素的认识，以及临床谱和预后。还简要讨论了可能的新治疗靶点。
