RESUMO
Hereditary complement C3 deficiency is associated with recurrent bacterial infections and proliferative glomerulonephritis. We describe a case of an adult with complete deficiency of complement C3 due to homozygous mutations in C3 gene: c.1811delT (Val604Glyfs*2), recurrent bacterial infections, crescentic glomerulonephritis, and end-stage renal failure. Following isolated kidney transplantation he would remain C3 deficient with a similar, or increased, risk of infections and glomerulonephritis. As C3 is predominantly synthesized in the liver, with a small proportion of C3 monocyte derived and kidney derived, he proceeded to simultaneous liver-kidney transplantation. The procedure has been successful with restoration of his circulating C3 levels, normal liver and kidney function at 26 months of follow-up. Simultaneous liver-kidney transplant is a viable option to be considered in this rare setting.
Assuntos
Glomerulonefrite , Falência Renal Crônica , Transplante de Rim , Adulto , Complemento C3/genética , Humanos , Rim , Falência Renal Crônica/cirurgia , Fígado , MasculinoRESUMO
BACKGROUND: The aim of this study was to evaluate the midterm results of a brachio-axillary arteriovenous graft (BA-AVG) for the provision of vascular access haemodialysis patients. MATERIALS AND METHODS: A cohort retrospective consecutive single-centre study of 46 patients undergoing BA-AVG using the Gore Acuseal, from November 2015 to October 2019 was conducted. Data on patient demographics, comorbidities, medical therapy, and complications were collated for the initial endpoints of primary patency, primary assisted patency, and secondary patency. A subgroup analysis included outcomes in patients over 70 years old and events (complications) per AVG per year. Data were subjected to Kaplan-Meier survival estimator with log-rank analysis and test of probability. RESULTS: The mean age of the cohort was 63.5 years with male predominance (male, n = 27, 59%). A total of 37 (80%) patient procedures were conducted with elective settings as well as on an emergency basis with a 91.3% technical success rate. The most common complication was grade I steal syndrome (8.7%), followed by graft infections (4.3%), median nerve neuropraxia (4.3%), and postoperative bleeding (2%), demonstrating a 0.1 per AVG complication per 2 years. Median primary patency, primary assisted patency, and secondary patency over a mean follow-up period of 28 months was 5.5, 12.5, and 18 months, respectively, with no associated 30-day mortality. CONCLUSION: BA-AVG with midterm longevity and low complications may serve as an alternative access type when a suitable site is not identified. The AVG patency rate in the elderly or patients with limited life expectancy is promising. However, more robust data are needed to confirm the benefit of AVG in this cohort.