Outcomes of kidney transplantation in Alport syndrome compared with other forms of renal disease

Abstract

Introduction: Alport syndrome is an inherited renal disease characterized by hematuria, renal failure, hearing loss and a lamellated glomerular basement membrane. Patients with Alport syndrome who undergo renal transplantation have been shown to have patient and graft survival rates similar to or better than those of patients with other renal diseases.Methods: In this national case series, based in Beaumont Hospital Dublin, we studied the cohort of patients who underwent renal transplantation over the past 33 years, recorded prospectively in the Irish Renal Transplant Registry, and categorized them according to the presence or absence of Alport syndrome. The main outcomes assessed were patient and renal allograft survival.Results: Fifty-one patients diagnosed with Alport syndrome in Beaumont Hospital received 62 transplants between 1982 and 2014. The comparison group of non-Alport patients comprised 3430 patients for 3865 transplants. Twenty-year Alport patient survival rate was 70.2%, compared to 44.8% for patients with other renal diseases (p = .01). Factors associated with patient survival included younger age at transplantation as well as differences in recipient sex, donor age, cold ischemia time, and episodes of acute rejection. Twenty-year graft survival was 46.8% for patients with Alport syndrome compared to 30.2% for those with non-Alport disease (p = .11).Conclusions: Adjusting for baseline differences between the groups, patients with end-stage kidney disease (ESKD) due to Alport syndrome have similar patient and graft survival to those with other causes of ESKD. This indicates that early diagnosis and management can lead to favorable outcomes for this patient cohort.

Clinical Characteristics and Long-Term Prognosis of Alport Syndrome: A Retrospective Single-Center Study

“…Most of the patients with AS who progress to ESRD have a better prog nosis than those with other kidney diseases. However, 2-5 % of transplant patients are likely to have graft failure due to posttransplant antiGBM nephritis caused due to allo antibodies to the donor kidney GBM 7,16,17) . The factors as sociated with the prognosis of AS include gender, family history, age at onset, hearing loss, ocular abnormalities, and abnormalities in EM findings 1,14) .…”

Glomerulonefritis antimembrana basal glomerular postrasplante renal en una adolescente con síndrome de Alport. Informe de caso

“…8 Una publicación más reciente en Irlanda identificó a 51 pacientes con síndrome de Alport a lo largo de 33 años con supervivencia del injerto de 20 años en 70% de los pacientes con síndrome de Alport vs 44.8% para otras enfermedades renales; en esa serie no se identificaron casos de glomerulonefritis antimembrana basal glomerular postrasplante. 9 Gumber y su grupo (2012) reportaron una serie de 31 pacientes, 21% con disfunción aguda del injerto en la que se identificaron 4 pacientes (12%) con glomerulonefritis antimembrana basal glomerular. 10 Gillion, en Bélgica, publicó (2018) el reporte de 43 años en el que identificaron 73 pacientes trasplantados por síndrome de Alport, solo 1.4% de ellos resultó con glomerulonefritis antimembrana basal glomerular.…”

<b>A Comprehensive</b> <b>Review on Inherited Sensorineural Hearing Loss and Their Syndromes</b>

“…and is characterized by HL with renal failure, lamellated glomerular basement membrane, and hematuria. In the case of nephritis, AS with ultra-structural faults in BGM (glomerular basement membranes) of affected individuals, altered and affected the protein structure [330]. Renal transplantation, in affected individuals with AS, shows graft and tolerant survival rates as compared to affect individuals of other renal diseases.…”

“…Patients suffered in "ESKD" (end-stage kidney disease), owing to AS have analogous patients and grafts survival to those affected individuals with other reported causes of "ESKD". Early management and diagnosis indicate positive results in individuals of the affected group [330]. It also diagnosed with anomalies of several ocular phenotypes, including Corneal and retinal manifestations [331,332].…”