Renal Transplantation in Patients with Cystinosis – A Case Series
Cystinosis is a rare autosomal recessive lysosomal storage disorder causing intracellular accumulation of cystine in different organs, leading to several organ dysfunctions. Renal involvement is the most serious manifestation of cystinosis leading to end-stage kidney disease (ESKD). Here, we report...
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| Format: | Article |
| Language: | English |
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Wolters Kluwer Medknow Publications
2024-12-01
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| Series: | Indian Journal of Transplantation |
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| Online Access: | https://journals.lww.com/10.4103/ijot.ijot_4_24 |
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| author | Yashwanth Raj Thiagarajan Dinesh Kannabhiran Kanakaraj Arumugham Rajan Ravichandran |
| author_facet | Yashwanth Raj Thiagarajan Dinesh Kannabhiran Kanakaraj Arumugham Rajan Ravichandran |
| author_sort | Yashwanth Raj Thiagarajan |
| collection | DOAJ |
| description | Cystinosis is a rare autosomal recessive lysosomal storage disorder causing intracellular accumulation of cystine in different organs, leading to several organ dysfunctions. Renal involvement is the most serious manifestation of cystinosis leading to end-stage kidney disease (ESKD). Here, we report the outcomes of four patients with cystinosis who underwent kidney transplantation. All the patients were young males. Renal manifestation included Fanconi syndrome and progressive decline in glomerular filtration rate (GFR). One of the patients had nephrotic syndrome with focal segmental glomerulosclerosis. The median age at ESKD was 10 years. Post-transplant, all patients were maintained on tacrolimus-based triple immunosuppression. All patients received cysteamine therapy. The median follow-up duration was 7.5 years. All patients maintained excellent graft function during follow-up except one. The median estimated GFR was 92.6 mL/min/1.73 m2. One patient lost the graft 6 years after transplant due to T-cell-mediated rejection. Kidney transplantation can be safely performed in patients with cystinosis with good long-term outcomes. This is the first case series regarding cystinosis patients who received kidney transplantation from India. |
| format | Article |
| id | doaj-art-33ceca414469435987c937fad79e9072 |
| institution | Kabale University |
| issn | 2212-0017 2212-0025 |
| language | English |
| publishDate | 2024-12-01 |
| publisher | Wolters Kluwer Medknow Publications |
| record_format | Article |
| series | Indian Journal of Transplantation |
| spelling | doaj-art-33ceca414469435987c937fad79e90722025-01-07T06:12:53ZengWolters Kluwer Medknow PublicationsIndian Journal of Transplantation2212-00172212-00252024-12-0118443643910.4103/ijot.ijot_4_24Renal Transplantation in Patients with Cystinosis – A Case SeriesYashwanth Raj ThiagarajanDinesh KannabhiranKanakaraj ArumughamRajan RavichandranCystinosis is a rare autosomal recessive lysosomal storage disorder causing intracellular accumulation of cystine in different organs, leading to several organ dysfunctions. Renal involvement is the most serious manifestation of cystinosis leading to end-stage kidney disease (ESKD). Here, we report the outcomes of four patients with cystinosis who underwent kidney transplantation. All the patients were young males. Renal manifestation included Fanconi syndrome and progressive decline in glomerular filtration rate (GFR). One of the patients had nephrotic syndrome with focal segmental glomerulosclerosis. The median age at ESKD was 10 years. Post-transplant, all patients were maintained on tacrolimus-based triple immunosuppression. All patients received cysteamine therapy. The median follow-up duration was 7.5 years. All patients maintained excellent graft function during follow-up except one. The median estimated GFR was 92.6 mL/min/1.73 m2. One patient lost the graft 6 years after transplant due to T-cell-mediated rejection. Kidney transplantation can be safely performed in patients with cystinosis with good long-term outcomes. This is the first case series regarding cystinosis patients who received kidney transplantation from India.https://journals.lww.com/10.4103/ijot.ijot_4_24cysteaminecystinosisfanconi syndromerenal transplant |
| spellingShingle | Yashwanth Raj Thiagarajan Dinesh Kannabhiran Kanakaraj Arumugham Rajan Ravichandran Renal Transplantation in Patients with Cystinosis – A Case Series Indian Journal of Transplantation cysteamine cystinosis fanconi syndrome renal transplant |
| title | Renal Transplantation in Patients with Cystinosis – A Case Series |
| title_full | Renal Transplantation in Patients with Cystinosis – A Case Series |
| title_fullStr | Renal Transplantation in Patients with Cystinosis – A Case Series |
| title_full_unstemmed | Renal Transplantation in Patients with Cystinosis – A Case Series |
| title_short | Renal Transplantation in Patients with Cystinosis – A Case Series |
| title_sort | renal transplantation in patients with cystinosis a case series |
| topic | cysteamine cystinosis fanconi syndrome renal transplant |
| url | https://journals.lww.com/10.4103/ijot.ijot_4_24 |
| work_keys_str_mv | AT yashwanthrajthiagarajan renaltransplantationinpatientswithcystinosisacaseseries AT dineshkannabhiran renaltransplantationinpatientswithcystinosisacaseseries AT kanakarajarumugham renaltransplantationinpatientswithcystinosisacaseseries AT rajanravichandran renaltransplantationinpatientswithcystinosisacaseseries |