| Title | Pediatric kidney transplantation for cystinosis. | | Author(s) | West JC, Goodman SI, Schröter GP, Bloustein PA, Hambidge KM, Weil R | | Source | J Pediatr Surg 1977 Oct; 12(5):651-5. | | MeSH | Adolescent
Child
Cystine
Cystinosis
Female
Graft Rejection
Humans
Kidney
Kidney Transplantation
Male
Thyroid Gland
Time Factors
Transplantation, Homologous
| | Abstract | In an experience of more than 700 kidney transplants, three recipients are known to have had congenital cystinosis. A cadaver kidney transplant in a 10 yr old child with nephropathic cystinosis functioned well for 7 1/2 yr before it was rejected and the graft was free of recurrent cystinosis after that interval. This prolongation of life was associated with marked accumulation of cystine in the patient's thyroid gland, presenting as a mass in the neck. If additional longevity is achieved in this patient, abnormal intracellular cystine accumulation may be expected in other host tissues. Retransplantation was carried out with the father as donor, accepting the possibility of increased cystine levels in the retransplant. This patient's age of 18 yr identifies him as one of the longest known survivors of infantile nephropathic cystinosis. | | Language | eng | | Pub Type(s) | Case Reports
Journal Article
Research Support, U.S. Gov't, P.H.S.
| | PubMed ID | 335039 |
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