Renal hypoplasia can be the cause of membranous nephropathy-like lesions
- PDF / 1,473,381 Bytes
- 8 Pages / 595.276 x 790.866 pts Page_size
- 28 Downloads / 175 Views
ORIGINAL ARTICLE
Renal hypoplasia can be the cause of membranous nephropathy‑like lesions Keiichi Takizawa1,2 · Kenichiro Miura1 · Naoto Kaneko1 · Tomoo Yabuuchi1 · Kiyonobu Ishizuka1 · Shoichiro Kanda2 · Yutaka Harita2 · Yuko Akioka1,3 · Shigeru Horita4 · Sekiko Taneda5 · Kazuho Honda6 · Motoshi Hattori1 Received: 25 February 2020 / Accepted: 9 May 2020 © Japanese Society of Nephrology 2020
Abstract Background Renal hypoplasia (RH) is the most common cause of chronic kidney disease in children. In cases of RH, proteinuria is often induced by glomerular hypertrophy and hyperfiltration that is commonly associated with focal segmental glomerulosclerosis. This study reports the first case series of a possible association between RH and membranous nephropathy (MN). Methods Of the 168 children with RH who visited our department between 1999 and 2017, five with overt proteinuria (≥ 1 g/gCr) underwent renal biopsy. We retrospectively reviewed the medical charts and analyzed biopsy specimens using light microscopy (LM), immunofluorescence (IF), and electron microscopy. Results The five children (four boys and one girl) had a median age of 5.5 years at the time of renal biopsy. The median proteinuria was 4.23 g/gCr (range 1.46–14.25), median serum albumin, 2.9 g/dL (range 2.3–3.7), and median estimated glomerular filtration rate, 59.7 mL/min/1.73 m2 (range 36.7–103.6). LM showed segmental spike formation and mesangial hypercellularity and IF study showed segmental granular immunoglobulin G (IgG) staining (IgG1 and IgG3 dominant) along the capillary loops in all five patients. Electron-dense deposits were observed in the subepithelial and mesangial areas. Thus, the pathological studies showed MN-like lesions in all patients. Conclusion Our study suggests that RH can be the cause of MN-like lesions. Keywords Membranous nephropathy · Renal hypoplasia · Proteinuria · Children
Introduction
* Motoshi Hattori [email protected] 1
Department of Pediatric Nephrology, School of Medicine, Tokyo Women’s Medical University, 8‑1 Kawada‑cho, Shinjuku‑ku, Tokyo 162‑8666, Japan
2
Department of Pediatrics, The University of Tokyo, Tokyo, Japan
3
Department of Pediatrics, Saitama Medical University, Saitama, Japan
4
Department of Pathology, Kidney Center, School of Medicine, Tokyo Women’s Medical University, Tokyo, Japan
5
Department of Pathology, School of Medicine, Tokyo Women’s Medical University, Tokyo, Japan
6
Department of Anatomy, Showa University School of Medicine, Tokyo, Japan
Renal hypoplasia (RH) is a common congenital anomaly characterized by a small overall kidney size due to genetic abnormalities and prematurity [1, 2]. RH is the most common cause of chronic kidney disease (CKD) in children [3]. As CKD progresses, urinalysis typically reveals a urineconcentrating defect and mild proteinuria. Increasing proteinuria often indicates the development of focal segmental glomerulosclerosis (FSGS), presumably due to functional overload of the reduced nephrons [4]. Membranous nephropathy (MN) is a glome
Data Loading...
