Renal failure due to tubulointerstitial nephropathy in an infant with cranioectodermal dysplasia

Obikane, Katsuyuki; Nakashima, Taiji; Watarai, Yoshihiko et al.

Pediatric Nephrology, 2006, 21(4), 574-576

Permalink : https://hdl.handle.net/2115/8501

このアイテムのアクセス数:1,556件（2026-03-15 08:01 集計）

閲覧可能ファイル 

ファイル フォーマット サイズ 閲覧回数 説明
PediatNephrol21_p574 pdf 1.12 MB 1,564

論文情報

ファイル出力 EndNote Basic出力 Mendeley出力

アクセス権
open access
URI
https://hdl.handle.net/2115/8501
タイトル
Renal failure due to tubulointerstitial nephropathy in an infant with cranioectodermal dysplasia
著者
Obikane, Katsuyuki ; Nakashima, Taiji ; Watarai, Yoshihiko ; Morita, Ken ; Cho, Kazutoshi ; Tonoki, Hidefumi ; Nagata, Michio ; Sasaki, Satoshi
著者名
Obikane, Katsuyuki
著者名
Nakashima, Taiji
著者名
Watarai, Yoshihiko
著者名
Morita, Ken
著者名
Cho, Kazutoshi
著者名
Tonoki, Hidefumi
著者名
Nagata, Michio
著者名
Sasaki, Satoshi
言語
英語
キーワード
Cranioectodermal dysplasia
Tubulointerstitial nephropathy
Oligohydramnios
End-stage renal disease
Nephronophthisis
発行日
2006-04
出版者
Springer
収録物名
Pediatric Nephrology
巻(号)
21(4)
ページ
574-576
抄録
Cranioectodermal dysplasia (CED) is a rare autosomal recessive disease with characteristic craniofacial, skeletal, and ectodermal-derived tissue abnormalities. In this disease, tubulointerstitial nephropathy (TIN) has been reported as one of the life-threatening combinations. Here we report a sporadic case of CED showing signs of renal failure during the perinatal period. Renal biopsy at the age of 6 months revealed TIN consisting of marked interstitial fibrosis with inflammatory cell infiltration accompanied by scattered tubular atrophy. Glomeruli were often sclerosed and others showed prominent immaturity; the findings are supportive of progressive deterioration of renal function in this infant. This case suggests that TIN in CED can occur during the fetal period and progress rapidly, leading to end-stage renal failure in infancy.
資源タイプ
学術雑誌論文
出版タイプ
Accepted Manuscript
権利情報
The original publication is available at www.springerlink.com
PISSN
0931-041X
EISSN
1432-198X
関連情報
PMID
16491415
関連情報 (isVersionOf)
出版社版DOI
https://doi.org/10.1007/s00467-006-0031-8