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Background: Despite early diagnosis and medical interventions, patients with methylmalonic acidemia (MMA) suffer from multi-organ damage and recurrent metabolic decompensations., Methods: We conducted the largest retrospective multi-center cohort study so far, involving five transplant centers (NCCHD, KUH, KUHP, ATAK, and EMC), and identified all MMA patients (n = 38) undergoing LDLT in the past two decades. Our primary outcome was patient survival, and secondary outcomes included death-censored graft survival and posttransplant complications., Results: The overall 10-year patient survival and death-censored graft survival rates were 92% and 97%, respectively. Patients who underwent LDLT within 2 years of MMA onset showed significantly higher 10-year patient survival compared to those with an interval more than 2 years (100% vs. 81%, p = 0.038), although the death-censored graft survival were not statistically different (100% vs. 93%, p = 0.22). Over the long-term follow-up, 14 patients (37%) experienced intellectual disability, while two patients developed neurological complications, three patients experienced renal dysfunction, and one patient had biliary anastomotic stricture. The MMA level significantly decreased from 2218.5 mmol/L preoperative to 307.5 mmol/L postoperative (p = 0.038)., Conclusions: LDLT achieves favorable long-term patient and graft survival outcomes for MMA patients. While not resulting in complete cure, our findings support the consideration of early LDLT within 2 years of disease onset. This approach holds the potential to mitigate recurrent metabolic decompensations, and preserve the long-term renal function., (© 2024 Wiley Periodicals LLC.)

Prediction of the risk of cardiovascular events (CVE's) is important to optimize outcomes after kidney transplantation. Aortoiliac stenosis is frequently observed during pre-transplant screening. We hypothesized that these patients are at higher risk of post-transplant CVE's due to the joint underlying atherosclerotic disease. Therefore, we aimed to assess whether aortoiliac stenosis was associated with post-transplant CVE's. This retrospective, single-center cohort study included adult kidney transplant recipients, transplanted between 2000 and 2016, with contrast-enhanced imaging available. Aortoiliac stenosis was classified according to the Trans-Atlantic Inter-Society Consensus (TASC) II classification and was defined as significant in case of ≥50% lumen narrowing. The primary outcome was CVE-free survival. Eighty-nine of 367 patients had significant aortoiliac stenosis and were found to have worse CVE-free survival (median CVE-free survival: stenosis 4.5 years (95% confidence interval (CI) 2.8-6.2), controls 8.9 years (95% CI 6.8-11.0); log-rank test P < .001). TASC II C and D lesions were independent risk factors for a post-transplant CVE with a hazard ratio of 2.15 (95% CI 1.05-4.38) and 6.56 (95% CI 2.74-15.70), respectively. Thus, kidney transplant recipients with TASC II C and D aortoiliac stenosis require extensive cardiovascular risk management pre-, peri,- and post-transplantation., (© 2021 The Authors. Clinical Transplantation published by John Wiley & Sons Ltd.)

Patients with class II and III obesity and end-stage renal disease are often ineligible for kidney transplantation (KTx) due to increased postoperative complications and technically challenging surgery. Bariatric surgery (BS) can be an effective solution for KTx candidates who are considered inoperable. The aim of this study is to evaluate outcomes of KTx after BS and to compare the outcomes to obese recipients (BMI ≥ 35 kg/m 2 ) without BS. This retrospective, single-center study included patients who received KTx after BS between January 1994 and December 2018. The primary outcome was postoperative complications. The secondary outcomes were graft and patient survival. In total, 156 patients were included, of whom 23 underwent BS prior to KTx. There were no significant differences in postoperative complications. After a median follow-up of 5.1 years, death-censored graft survival, uncensored graft survival, and patient survival were similar to controls (log rank test p = .845, .659, and .704, respectively). Dialysis pre-transplantation (Hazard Ratio (HR) 2.55; 95%CI 1.03-6.34, p = .043) and diabetes (HR 2.41; 95%CI 1.11-5.22, p = .027) were independent risk factors for all-cause mortality. A kidney from a deceased donor was an independent risk factor for death-censored graft loss (HR 1.98; 95%CI 1.04-3.79, p = .038). Patients who received a KTx after BS have similar outcomes as obese transplant recipients., (© 2021 The Authors. Clinical Transplantation published by John Wiley & Sons Ltd.)

The prevalence of end-stage renal failure in Curaçao (Dutch Caribbean) is one of the highest in the world. In 1998, the St. Elisabeth Hospital started a unique trans-Atlantic collaboration with the Academic Medical Center in Amsterdam, the Netherlands, and the Eurotransplant Foundation. The partnership aimed to achieve a structured transplantation program for patients in the Dutch Caribbean, who otherwise would need lifelong dialysis. This study is an analysis of the 10-yr transplantation results of this trans-Atlantic program. In 41 consecutive transplantations performed between January 1998 and April 2007, one-yr graft survival and complication rates were retrospectively studied. Twenty-four men and 17 women with a median age of 54 were transplanted. The median dialysis period prior to transplantation was 6.8 yr. The one-yr graft survival rate was 69% (95% confidence interval: 52-80%). Initially 28 grafts functioned (68%); four grafts showed primary non-function (10%) and delayed graft function developed in nine patients (22%). Ten recipients had 16 post-operative complications. Our trans-Atlantic program affords patients with end-stage renal failure, who otherwise would need lifelong dialysis, a chance to be transplanted., (© 2010 John Wiley & Sons A/S.)