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Renal failure due to tubulointerstitial nephropathy in an infant with cranioectodermal dysplasia
Title: | Renal failure due to tubulointerstitial nephropathy in an infant with cranioectodermal dysplasia |
Authors: | Obikane, Katsuyuki Browse this author | Nakashima, Taiji Browse this author | Watarai, Yoshihiko Browse this author | Morita, Ken Browse this author | Cho, Kazutoshi Browse this author | Tonoki, Hidefumi Browse this author | Nagata, Michio Browse this author | Sasaki, Satoshi Browse this author |
Keywords: | Cranioectodermal dysplasia | Tubulointerstitial nephropathy | Oligohydramnios | End-stage renal disease | Nephronophthisis |
Issue Date: | Apr-2006 |
Publisher: | Springer |
Journal Title: | Pediatric Nephrology |
Volume: | 21 |
Issue: | 4 |
Start Page: | 574 |
End Page: | 576 |
Publisher DOI: | 10.1007/s00467-006-0031-8 |
PMID: | 16491415 |
Abstract: | 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. |
Rights: | The original publication is available at www.springerlink.com |
Type: | article (author version) |
URI: | http://hdl.handle.net/2115/8501 |
Appears in Collections: | 医学院・医学研究院 (Graduate School of Medicine / Faculty of Medicine) > 雑誌発表論文等 (Peer-reviewed Journal Articles, etc)
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Submitter: 佐々木 聡
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