Structured Transition Protocol for Children with Cystinosis
نویسندگان
چکیده
منابع مشابه
Structured Transition Protocol for Children with Cystinosis
The transition from pediatric to adult medical services has a greater impact on the care of adolescents or young adults with chronic diseases such as cystinosis. This transition period is a time of psychosocial development and new responsibilities placing these patients at increased risk of non-adherence. This can lead to serious adverse effects such as graft loss and progression of the disease...
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Background and Objective: Cystinosis is a rare inherited disease that leads to renal failure. Fanconi syndrome is the major renal involvement in cystinosis patients. Renal transplantation is the treatment of choice in cystinosis children with end-stage renal disease (ESRD). The study aimed to assess the outcome of renal transplantation in Iranian children with cystinosis. Methods: This...
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Eight children with cystinosis (3 with renal transplants, 2 on maintenance haemodialysis, 2 with chronic renal failure, and one with normal renal function) were studied for evidence of hypothyroidism, and compared with a control group of children with chronic renal failure due to other causes. Abnormal thyroid function was present in all the cystinotic patients: thyroxine (T4) low in 1, free th...
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Cystinosis is a rare autosomal recessive disorder characterized by the intralysosomal accumulation of cystine in all tissues due to mutations in the CTNS gene (17p13.3). Infantile nephropatic cystinosis is the most severe and the most frequent form of the disease. It causes renal Fanconi syndrome, leading to end stage renal failure around the age of 10 years if left untreated. Cystine accumulat...
متن کاملrenal transplantation outcome in children with cystinosis
background and objective: cystinosis is a rare inherited disease that leads to renal failure. fanconi syndrome is the major renal involvement in cystinosis patients. renal transplantation is the treatment of choice in cystinosis children with end-stage renal disease (esrd). the study aimed to assess the outcome of renal transplantation in iranian children with cystinosis. methods: this retrosp...
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ژورنال
عنوان ژورنال: Frontiers in Pediatrics
سال: 2017
ISSN: 2296-2360
DOI: 10.3389/fped.2017.00191