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Successful Kidney Transplantation for End-Stage Renal Disease in Marfan's Syndrome
Makoto Ryosaka1, Kazuya Omoto, Taiji Nozaki
1Department of Urology, Tokyo Women's Medical University, 8-1 Kawada-cho, Shinjuku-ku, Tokyo 162-8666, Japan.
Abstract:
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.
Insights
Kidney transplantation may be suitable for Marfan syndrome patients with end-stage renal disease (ESRD). A patient with Marfan syndrome and ESRD successfully received a kidney transplant without vascular complications.
Area of Science:
- Cardiology
- Nephrology
- Genetics
Background:
- Marfan syndrome, a genetic connective tissue disorder, commonly presents with life-threatening aortic dissections and aneurysms.
- End-stage renal disease (ESRD) in Marfan syndrome patients is rare, with no reported cases of kidney transplantation.
- The risk of iliac artery dissection or aneurysm in these patients remains unknown.
Purpose of the Study:
- To report the first case of kidney transplantation in a patient with Marfan syndrome and ESRD.
- To assess the safety and efficacy of kidney transplantation in this patient population.
- To evaluate vascular complications, specifically iliac artery dissection or aneurysm, post-transplantation.
Main Methods:
- A patient with Marfan syndrome and ESRD secondary to aortic dissection underwent living donor kidney transplantation.
- Post-transplant monitoring included assessment of renal function, graft status, and vascular imaging (ultrasonography).
- Follow-up included evaluation for microhematuria, proteinuria, and vascular complications over two years.
Main Results:
- The patient's renal function normalized post-transplantation, with stable graft function for two years.
- No vascular complications, including iliac artery aneurysm or dissection, were observed during the follow-up period.
- Microhematuria and proteinuria remained negative, indicating successful graft function.
Conclusions:
- Kidney transplantation appears to be a viable option for Marfan syndrome patients with ESRD.
- Careful long-term observation is crucial to monitor for potential vascular complications.
- This case suggests that ESRD patients with Marfan syndrome may benefit from transplantation if carefully selected and monitored.
Related Concept Videos
01:28Kidney Transplant I: Introduction
01:26Kidney Transplant II: Surgical Procedure