Journal of Disability and Oral Health

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Cover Date:
December 2012
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Pre-transplant dental management of a patient with nephropathic cystinosis

Doi: 10.4483/JDOH_012Martins04

Abstract Nephropathic cystinosis (NC) is an autosomal recessive metabolic disorder that is characterised by intracellular cystine deposits in the lysosomes of several tissues, leading to multiple organ involvement and renal failure. A 23-year-old male patient diagnosed with NC was referred to our clinic for dental treatment before his second kidney transplant. He presented with growth retardation, chronic renal failure, partial retinal blindness, photophobia, hypothyroidism, mild swallowing difficulties, and diabetes. An oral examination showed dry lips, fissured tongue partially covered with biofilm, several teeth revealing clinical aspects compatible with enamel hypoplasia, and generalised extensive carious lesions and gingivitis. A radiographic examination revealed the presence of multiple root dilacerations and changes on the trabecular bone pattern. This paper discusses the pre-transplant dental treatment of a NC patient, including oral features clinical guidelines for dental management and challenges which may be encountered when providing care.

Key words: Special Care Dentistry, Chronic renal failure, cystinosis, dental management, Fanconi syndrome.

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Fabiana Martins, Marcio Augusto de Oliveira, Nathalie Pepe Medeiros de Rezende, Paulo Sérgio da Silva Santos, Marina Gallottini

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  1. Index to JDOH Volume 13 2012
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  1. Thanks to reviewers 2012
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