Literature DB >> 20856047

Saldino-Mainzer syndrome: nephronophthisis, retinitis pigmentosa, and cone-shaped epiphyses.

Carmen Mortellaro1, Lucilla Bello, Angela Pucci, Alberta Greco Lucchina, Mario Migliario.   

Abstract

Saldino-Mainzer syndrome is part of a group disorders, the conorenal syndromes, that are characterized by cone-shaped epiphyses with chronic renal disease in childhood and are variously associated with retinitis pigmentosa, cerebral ataxia, and/or abnormalities of the proximal epiphyses and femur metaphyses. Saldino-Mainzer syndrome usually has sporadic presentation. The present report shows the unusual findings of a 23-year-old woman, affected by the Saldino-Mainzer syndrome and has undergone kidney transplantation, highlights the possible association with maxillofacial and cephalometric abnormalities.

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Year:  2010        PMID: 20856047     DOI: 10.1097/SCS.0b013e3181ec69bb

Source DB:  PubMed          Journal:  J Craniofac Surg        ISSN: 1049-2275            Impact factor:   1.046


  3 in total

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2.  Combined NGS approaches identify mutations in the intraflagellar transport gene IFT140 in skeletal ciliopathies with early progressive kidney Disease.

Authors:  Miriam Schmidts; Valeska Frank; Tobias Eisenberger; Saeed Al Turki; Albane A Bizet; Dinu Antony; Suzanne Rix; Christian Decker; Nadine Bachmann; Martin Bald; Tobias Vinke; Burkhard Toenshoff; Natalia Di Donato; Theresa Neuhann; Jane L Hartley; Eamonn R Maher; Radovan Bogdanović; Amira Peco-Antić; Christoph Mache; Matthew E Hurles; Ivana Joksić; Marija Guć-Šćekić; Jelena Dobricic; Mirjana Brankovic-Magic; Hanno J Bolz; Gregory J Pazour; Philip L Beales; Peter J Scambler; Sophie Saunier; Hannah M Mitchison; Carsten Bergmann
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Review 3.  Emerging role of primary cilia as mechanosensors in osteocytes.

Authors:  An M Nguyen; Christopher R Jacobs
Journal:  Bone       Date:  2012-11-28       Impact factor: 4.398

  3 in total

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