Long-term survival in Japanese renal transplant recipients with Alport syndrome: a retrospective study
Abstract Background Patients with Alport syndrome (AS) develop progressive kidney dysfunction due to a hereditary type IV collagen deficiency. Survival of the kidney allograft in patients with AS is reportedly excellent because AS does not recur. However, several studies have implied that the type I...
| Main Authors: | , , , , , , , , , , , |
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| Format: | Article |
| Language: | English |
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BMC
2018-10-01
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| Series: | BMC Nephrology |
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| Online Access: | http://link.springer.com/article/10.1186/s12882-018-1052-9 |
| _version_ | 1819120925656743936 |
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| author | Ai Katsuma Yasuyuki Nakada Izumi Yamamoto Shigeru Horita Miyuki Furusawa Kohei Unagami Haruki Katsumata Masayoshi Okumi Hideki Ishida Takashi Yokoo Kazunari Tanabe Japan Academic Consortium of Kidney Transplantation (JACK) |
| author_facet | Ai Katsuma Yasuyuki Nakada Izumi Yamamoto Shigeru Horita Miyuki Furusawa Kohei Unagami Haruki Katsumata Masayoshi Okumi Hideki Ishida Takashi Yokoo Kazunari Tanabe Japan Academic Consortium of Kidney Transplantation (JACK) |
| author_sort | Ai Katsuma |
| collection | DOAJ |
| description | Abstract Background Patients with Alport syndrome (AS) develop progressive kidney dysfunction due to a hereditary type IV collagen deficiency. Survival of the kidney allograft in patients with AS is reportedly excellent because AS does not recur. However, several studies have implied that the type IV collagen in the GBM originates from podocytes recruited from the recipient’s bone marrow-derived cells, suggesting the possibility of AS recurrence. Limited data are available regarding AS recurrence and graft survival in the Japanese population; the vast majority were obtained from living related kidney transplantation (LRKTx). Methods In this retrospective study, twenty-one patients with AS were compared with 41 matched patients without AS from 1984 to 2015 at two centers using propensity scores. Nineteen of the 21 patients with AS underwent LRKTx. The mean post-transplant follow-up period was 83 months in the AS group and 110 months in the control group. Histopathological AS recurrence was assessed by immunoreactivity of α5 (type IV collagen) antibody and electron microscopy. Results The graft survival rate was equivalent between patients with and without AS (86.7% vs. 77.1% and 69.3% vs. 64.2% at 5 and 10 years; p = 0.16, log-rank test). Immunoreactivity to α5 antibody showed strong linear positivity with no focal defect in six patients. Electron microscopy showed no GBM abnormalities in two patients who were exhibiting long-term kidney allograft survival. Conclusions We confirmed that α5 and the GBM structure were histopathologically maintained in the long term after kidney transplantation. The patient and graft survival rates were equivalent between Japanese patients with and without AS. |
| first_indexed | 2024-12-22T06:28:25Z |
| format | Article |
| id | doaj.art-40a8918422c94d2a9645bef69b378b15 |
| institution | Directory Open Access Journal |
| issn | 1471-2369 |
| language | English |
| last_indexed | 2024-12-22T06:28:25Z |
| publishDate | 2018-10-01 |
| publisher | BMC |
| record_format | Article |
| series | BMC Nephrology |
| spelling | doaj.art-40a8918422c94d2a9645bef69b378b152022-12-21T18:35:46ZengBMCBMC Nephrology1471-23692018-10-011911910.1186/s12882-018-1052-9Long-term survival in Japanese renal transplant recipients with Alport syndrome: a retrospective studyAi Katsuma0Yasuyuki Nakada1Izumi Yamamoto2Shigeru Horita3Miyuki Furusawa4Kohei Unagami5Haruki Katsumata6Masayoshi Okumi7Hideki Ishida8Takashi Yokoo9Kazunari Tanabe10Japan Academic Consortium of Kidney Transplantation (JACK)Division of Nephrology and Hypertension, Department of Internal Medicine, The Jikei University School of MedicineDivision of Nephrology and Hypertension, Department of Internal Medicine, The Jikei University School of MedicineDivision of Nephrology and Hypertension, Department of Internal Medicine, The Jikei University School of MedicineDepartment of Medicine, Kidney center, Tokyo Women’s Medical UniversityDepartment of Urology, Tokyo Women’s Medical UniversityDepartment of Urology, Tokyo Women’s Medical UniversityDivision of Nephrology and Hypertension, Department of Internal Medicine, The Jikei University School of MedicineDepartment of Urology, Tokyo Women’s Medical UniversityDepartment of Urology, Tokyo Women’s Medical UniversityDivision of Nephrology and Hypertension, Department of Internal Medicine, The Jikei University School of MedicineDepartment of Urology, Tokyo Women’s Medical UniversityAbstract Background Patients with Alport syndrome (AS) develop progressive kidney dysfunction due to a hereditary type IV collagen deficiency. Survival of the kidney allograft in patients with AS is reportedly excellent because AS does not recur. However, several studies have implied that the type IV collagen in the GBM originates from podocytes recruited from the recipient’s bone marrow-derived cells, suggesting the possibility of AS recurrence. Limited data are available regarding AS recurrence and graft survival in the Japanese population; the vast majority were obtained from living related kidney transplantation (LRKTx). Methods In this retrospective study, twenty-one patients with AS were compared with 41 matched patients without AS from 1984 to 2015 at two centers using propensity scores. Nineteen of the 21 patients with AS underwent LRKTx. The mean post-transplant follow-up period was 83 months in the AS group and 110 months in the control group. Histopathological AS recurrence was assessed by immunoreactivity of α5 (type IV collagen) antibody and electron microscopy. Results The graft survival rate was equivalent between patients with and without AS (86.7% vs. 77.1% and 69.3% vs. 64.2% at 5 and 10 years; p = 0.16, log-rank test). Immunoreactivity to α5 antibody showed strong linear positivity with no focal defect in six patients. Electron microscopy showed no GBM abnormalities in two patients who were exhibiting long-term kidney allograft survival. Conclusions We confirmed that α5 and the GBM structure were histopathologically maintained in the long term after kidney transplantation. The patient and graft survival rates were equivalent between Japanese patients with and without AS.http://link.springer.com/article/10.1186/s12882-018-1052-9Alport syndromeKidney transplantationType IV collagen |
| spellingShingle | Ai Katsuma Yasuyuki Nakada Izumi Yamamoto Shigeru Horita Miyuki Furusawa Kohei Unagami Haruki Katsumata Masayoshi Okumi Hideki Ishida Takashi Yokoo Kazunari Tanabe Japan Academic Consortium of Kidney Transplantation (JACK) Long-term survival in Japanese renal transplant recipients with Alport syndrome: a retrospective study BMC Nephrology Alport syndrome Kidney transplantation Type IV collagen |
| title | Long-term survival in Japanese renal transplant recipients with Alport syndrome: a retrospective study |
| title_full | Long-term survival in Japanese renal transplant recipients with Alport syndrome: a retrospective study |
| title_fullStr | Long-term survival in Japanese renal transplant recipients with Alport syndrome: a retrospective study |
| title_full_unstemmed | Long-term survival in Japanese renal transplant recipients with Alport syndrome: a retrospective study |
| title_short | Long-term survival in Japanese renal transplant recipients with Alport syndrome: a retrospective study |
| title_sort | long term survival in japanese renal transplant recipients with alport syndrome a retrospective study |
| topic | Alport syndrome Kidney transplantation Type IV collagen |
| url | http://link.springer.com/article/10.1186/s12882-018-1052-9 |
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