Document Detail


Joubert syndrome with nephronophthisis in neurofibromatosis type 1.
MedLine Citation:
PMID:  21743231     Owner:  NLM     Status:  In-Data-Review    
Abstract/OtherAbstract:
Joubert syndrome (JS) is a rare developmental disorder of the central nervous system, characterised by brainstem and cerebellar malformations, hypotonia, episodic hyperapnea and apnea and mental retardation. It may be associated other systemic abnormalities like ocular (e.g., retinal dysplasia, etc.), oculomotor, musculoskeletal and renal (e.g., cystic dysplasia, nephronophthisis), with renal failure. We describe a case of JS with nephronophthisis in neurofibromatosis Type 1 leading to end-stage renal disease, a association that has never been described earlier in the medical literature to the best of our knowledge.
Authors:
Javed Ahmed; Uma S Ali
Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Saudi journal of kidney diseases and transplantation : an official publication of the Saudi Center for Organ Transplantation, Saudi Arabia     Volume:  22     ISSN:  1319-2442     ISO Abbreviation:  Saudi J Kidney Dis Transpl     Publication Date:    2011 Jul-Aug
Date Detail:
Created Date:  2011-07-11     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9436968     Medline TA:  Saudi J Kidney Dis Transpl     Country:  Saudi Arabia    
Other Details:
Languages:  eng     Pagination:  788-91     Citation Subset:  IM    
Affiliation:
Department of Pediatric Nephrology, B.J. Wadia Hospital for Children, Parel, Mumbai, India.
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