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International Journal of Pediatrics، جلد ۱۲، شماره ۱۰، صفحات ۱۹۰۹۸-۱۹۱۰۱
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عنوان انگلیسی Nephropathic Cystinosis in Children: A Ten Year Experience from a Pediatric Nephrology Center in Mashhad, North East of Iran
چکیده انگلیسی مقاله Background: Nephropathic cystinosis is a rare autosomal recessive lysosomal storage disease. Its clinical manifestations include: failure to thrive, fanconi syndrome, ocular findings, growth retardation and end stage renal disease.Methods: This was a retrospective chart review of patients with a diagnosis of nephropathic cystinosis over a ten year period in Dr. Sheikh Children’s Hospital in Mashhad, North East of Iran.Results: 20 patients were included in the study. The most common symptoms leading to the diagnosis were failure to thrive, polyuria, and polydipsia. Kidney involvement was reported in 77% of patients. End stage renal disease was reported in 10% and 2 patients had died because of that. Extrarenal manifestations included: hypothyroidism (25%), anemia (55.5%) and hypophosphatemic rickets (80%).Conclusion: The most important complication of nephropathic cystinosis is end stage kidney disease. Early diagnosis and timely treatment with cysteamin can prevent or delay the complications.
کلیدواژه‌های انگلیسی مقاله cystinosis, cysteamine, ESRD, Nephropathy
نویسندگان مقاله | Nasrin Moazzen
Clinical Research Development Unit of Akbar hospital, Faculty of medicine, Mashhad University of Medical Sciences, Mashhad, Iran.


| Fatemeh Shahrahmani
Medical Student, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran.


| Anoush Azarfar
Department of Pediatric Nephrology, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran.


| Yalda Ravanshad
Department of Social Medicine, Azad University of Medical Sciences, Mashhad, Iran.


| Alireza Ataei Nakhaee
Department of Pediatrics, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran.


| Ali Khakshour
Clinical Research Development Unit of Akbar hospital, Faculty of medicine, Mashhad University of Medical Sciences, Mashhad, Iran.


| mojtaba lotfi
Clinical Research Development Unit of Akbar hospital, Faculty of medicine, Mashhad University of Medical Sciences, Mashhad, Iran.


| Sepideh Bagheri
Clinical Research Development Unit of Akbar hospital, Faculty of medicine, Mashhad University of Medical Sciences, Mashhad, Iran.
نشانی اینترنتی https://jpp.mums.ac.ir/article_25304.html
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