Successful Kidney Transplantation for End-Stage Renal Disease in Marfan's Syndrome
Marfan’s syndrome is a systemic disorder of the connective tissue caused by mutations in the extracellular matrix protein fibrillin-1, with aortic dissection and aneurysm being its most life-threatening manifestations. Kidney transplantation for end-stage renal disease (ESRD) in patients with Marfan...
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| Format: | Article |
| Language: | English |
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Wiley
2013-01-01
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| Series: | Case Reports in Transplantation |
| Online Access: | http://dx.doi.org/10.1155/2013/809613 |
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| author | Makoto Ryosaka Kazuya Omoto Taiji Nozaki Kazuhiko Yoshida Yugo Sawada Hajime Hirano Tomokazu Shimizu Hideki Ishida Kazunari Tanabe |
| author_facet | Makoto Ryosaka Kazuya Omoto Taiji Nozaki Kazuhiko Yoshida Yugo Sawada Hajime Hirano Tomokazu Shimizu Hideki Ishida Kazunari Tanabe |
| author_sort | Makoto Ryosaka |
| collection | DOAJ |
| description | Marfan’s syndrome is a systemic disorder of the connective tissue caused by mutations in the extracellular matrix protein fibrillin-1, with aortic dissection and aneurysm being its most life-threatening manifestations. Kidney transplantation for end-stage renal disease (ESRD) in patients with Marfan’s syndrome has not been reported in the literature, and the rate of the incidence of dissection or aneurysm in the iliac artery is unknown. Here, we present a patient with Marfan’s syndrome with ESRD due to severe renal ischemia caused by massive bleeding from thoracoabdominal aortic dissection leading to transplant surgery of a living kidney procured from the patient’s mother. After kidney transplantation, the renal function normalized without vascular complications, and stable graft function along with negative results for both microhematuria and proteinuria continued for two years. Also, vascular complication such as aneurysm or dissection of the iliac artery was not observed using ultrasonography during the follow-up period. ESRD patients with Marfan’s syndrome might be suitable for kidney transplantation, but long-term and careful observations are needed. |
| format | Article |
| id | doaj-art-a05d1c572b524310a271e109264f04f4 |
| institution | Kabale University |
| issn | 2090-6943 2090-6951 |
| language | English |
| publishDate | 2013-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Transplantation |
| spelling | doaj-art-a05d1c572b524310a271e109264f04f42025-02-03T06:42:20ZengWileyCase Reports in Transplantation2090-69432090-69512013-01-01201310.1155/2013/809613809613Successful Kidney Transplantation for End-Stage Renal Disease in Marfan's SyndromeMakoto Ryosaka0Kazuya Omoto1Taiji Nozaki2Kazuhiko Yoshida3Yugo Sawada4Hajime Hirano5Tomokazu Shimizu6Hideki Ishida7Kazunari Tanabe8Department of Urology, Tokyo Women’s Medical University, 8-1 Kawada-cho, Shinjuku-ku, Tokyo 162-8666, JapanDepartment of Urology, Tokyo Women’s Medical University, 8-1 Kawada-cho, Shinjuku-ku, Tokyo 162-8666, JapanDepartment of Urology, Tokyo Women’s Medical University, 8-1 Kawada-cho, Shinjuku-ku, Tokyo 162-8666, JapanDepartment of Urology, Tokyo Women’s Medical University, 8-1 Kawada-cho, Shinjuku-ku, Tokyo 162-8666, JapanDepartment of Urology, Tokyo Women’s Medical University, 8-1 Kawada-cho, Shinjuku-ku, Tokyo 162-8666, JapanDepartment of Urology, Tokyo Women’s Medical University, 8-1 Kawada-cho, Shinjuku-ku, Tokyo 162-8666, JapanDepartment of Urology, Tokyo Women’s Medical University, 8-1 Kawada-cho, Shinjuku-ku, Tokyo 162-8666, JapanDepartment of Urology, Tokyo Women’s Medical University, 8-1 Kawada-cho, Shinjuku-ku, Tokyo 162-8666, JapanDepartment of Urology, Tokyo Women’s Medical University, 8-1 Kawada-cho, Shinjuku-ku, Tokyo 162-8666, JapanMarfan’s syndrome is a systemic disorder of the connective tissue caused by mutations in the extracellular matrix protein fibrillin-1, with aortic dissection and aneurysm being its most life-threatening manifestations. Kidney transplantation for end-stage renal disease (ESRD) in patients with Marfan’s syndrome has not been reported in the literature, and the rate of the incidence of dissection or aneurysm in the iliac artery is unknown. Here, we present a patient with Marfan’s syndrome with ESRD due to severe renal ischemia caused by massive bleeding from thoracoabdominal aortic dissection leading to transplant surgery of a living kidney procured from the patient’s mother. After kidney transplantation, the renal function normalized without vascular complications, and stable graft function along with negative results for both microhematuria and proteinuria continued for two years. Also, vascular complication such as aneurysm or dissection of the iliac artery was not observed using ultrasonography during the follow-up period. ESRD patients with Marfan’s syndrome might be suitable for kidney transplantation, but long-term and careful observations are needed.http://dx.doi.org/10.1155/2013/809613 |
| spellingShingle | Makoto Ryosaka Kazuya Omoto Taiji Nozaki Kazuhiko Yoshida Yugo Sawada Hajime Hirano Tomokazu Shimizu Hideki Ishida Kazunari Tanabe Successful Kidney Transplantation for End-Stage Renal Disease in Marfan's Syndrome Case Reports in Transplantation |
| title | Successful Kidney Transplantation for End-Stage Renal Disease in Marfan's Syndrome |
| title_full | Successful Kidney Transplantation for End-Stage Renal Disease in Marfan's Syndrome |
| title_fullStr | Successful Kidney Transplantation for End-Stage Renal Disease in Marfan's Syndrome |
| title_full_unstemmed | Successful Kidney Transplantation for End-Stage Renal Disease in Marfan's Syndrome |
| title_short | Successful Kidney Transplantation for End-Stage Renal Disease in Marfan's Syndrome |
| title_sort | successful kidney transplantation for end stage renal disease in marfan s syndrome |
| url | http://dx.doi.org/10.1155/2013/809613 |
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