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

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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
End-stage renal disease
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
Type: article (author version)
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)

Submitter: 佐々木 聡

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