Renal 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 retrospective study is a follow up of 21 cystinosis children transplanted in Labafinejad Hospital, Tehran, Iran. Results: Three cystinosis patients involved by primary non-function because of graft vein thrombosis and/or severe acute tubular necrosis. The remaining cystinosis patients had excellent graft survival rate and only one patient lost the graft 3 years post-transplant due to noncompliance. The graft survival rate after excluding the patients with primary non-function was 100%, 94%, 94% and 94% at 1,3,5 and 10 years after transplant. The mean serum creatinine in patients with functioning graft 10 years after transplant was 1.6 mg/dl. Conclusion: We showed that cystinosis patients had good graft function in long term after transplantation.
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...متن کامل
BACKGROUND Cystinosis is a rare lysosomal disorder leading to end stage renal disease in more than 90 % of patients before 20 years of age. Data about safety and efficiency of renal transplantation in patients with cystinosis is scarce. We evaluated long-term outcomes of renal transplantation in adult patients with cystinosis. METHODS Data of renal transplantation (n = 31) in 30 adult patient...متن کامل
Background and Objective: Some neurologic complications may be detected after renal transplantation in children including tremor, peripheral neuropathy, altered level of consciousness and seizure. The aim of our study was to detect the prevalence of seizure after renal transplantation and its risk factors in pediatric age groups. Methods: In this descriptive study, 102 patients 5-14 yea...متن کامل
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...متن کامل
Background: Combined Liver and Kidney Transplantation (CLKT) is a recognized treatment option for a select group of paediatric patients with severe liver and kidney disease. The aim of this study is to report Indication and outcome of CLKT in our center and to compare the results with the Isolated Kidney Transplantation (IKT). Patients and method: We retrospectively reviewed children who underw...متن کامل
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 کامل
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دوره 1 شماره 2
صفحات 10- 12
تاریخ انتشار 2015-08
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