Transplant candidatesFollow-up and Treatment of Renal Transplantation with Nephropathic Cystinosis in Central Taiwan
Section snippets
Methods
A retrospective analysis of the case histories of 1,196 RT patients in our hospital over the past 30 years was performed and showed that there were only 2 patients (siblings) with nephropathic cystinosis after RT. The sisters, aged 5 and 9 years old, were diagnosed with cystinosis by documenting corneal cystine crystal accumulation. After diagnosis they began cysteamine treatment until transplantation. We evaluated their height, central nervous and muscular systems, ocular manifestations,
Results
Two sisters had received renal biopsies before RT. The pathology of the original kidneys showed accumulation of cystine within renal tubules (Fig 1). The elder sister did not receive any renal replacement therapy before RT and received her first allograft at age 19.8 years. The young sister had received hemodialysis for 2 months at age 13.4 years owing to ESRD, and then received an allograft transplant from her mother. They each experienced 1 episode of acute rejection at 6 months after the
Discussion
Renal transplantation and treatment with cysteamine indeed improves the outcome of cystinosis, and this potentially fatal disease is now treatable. Our patients presented with corneal cystine accumulation and nephropathy. After transplantation, they both maintained good allograft function. One of them even successfully gave birth to a child without any complications.
Lawson et al4 and Hory et al9 reported a total of 4 cystinotic children who received RT. Morphologic studies of renal tubules
Conclusion
There are many extrarenal complications resulting from nephropathic cystinosis, but in our 2 cases (siblings), cysteamine therapy was not used to attenuate the intracellular cystine accumulation, and at the latest follow-up only ocular involvement was noted. There was a small sample size in the present study, so it may not be possible to generalize these findings to larger populations. Further studies with a larger number of patients are needed to monitor complications and establish prognoses.
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