Hemolytic uremic syndrome and kidney transplantation in uncontrolled donation after circulatory death (DCD): A two-case report.
anti-C5 treatment
ischemia/reperfusion injury (IRI)
thrombotic microangiopathy (TMA)
uncontrolled donation after circulatory death (uDCD)
Journal
Clinical nephrology. Case studies
ISSN: 2196-5293
Titre abrégé: Clin Nephrol Case Stud
Pays: Germany
ID NLM: 101638685
Informations de publication
Date de publication:
2021
2021
Historique:
received:
09
11
2020
accepted:
11
03
2021
entrez:
4
6
2021
pubmed:
5
6
2021
medline:
5
6
2021
Statut:
epublish
Résumé
Hemolytic uremic syndrome (HUS) is a rare disease characterized by microangiopathic hemolysis, thrombocytopenia, and renal involvement. Complement-mediated atypical HUS (aHUS) is a result of genetic defects in the alternative complement pathway components or regulators. The introduction of eculizumab has improved renal and overall survival of aHUS patients. Nowadays, given organ shortage, it is necessary to consider kidney transplantation (KT) even in protocols with a high risk of HUS recurrence, such as from donation after circulatory death (DCD) donors. Here, we describe two patients with HUS who underwent a KT from an uncontrolled DCD (uDCD). The first patient, affected by aHUS due to a heterozygous deletion in CFHR3-CFHR1 and a novel heterozygous variant in CFHR5 gene, underwent a KT with eculizumab prophylaxis. The patient did not experience a post-transplant aHUS recurrence. The second patient, who experienced an HUS episode characterized by a hypertensive crisis and with no underlying mutations in complement system genes, underwent a KT without eculizumab prophylaxis. At day 5, anti-complement treatment commenced due to hematological signs of thrombotic microangiopathy (TMA). After the introduction of eculizumab, we observed a stabilization of kidney function and hematological remission. We present herein two different patients with HUS who both underwent successful KT from uDCD donation under the umbrella of eculizumab therapy. Taking into account the importance of increasing the number of organs available for transplantation, uDCD could represent an additional resource in this subset of HUS patients.
Sections du résumé
BACKGROUND
BACKGROUND
Hemolytic uremic syndrome (HUS) is a rare disease characterized by microangiopathic hemolysis, thrombocytopenia, and renal involvement. Complement-mediated atypical HUS (aHUS) is a result of genetic defects in the alternative complement pathway components or regulators. The introduction of eculizumab has improved renal and overall survival of aHUS patients. Nowadays, given organ shortage, it is necessary to consider kidney transplantation (KT) even in protocols with a high risk of HUS recurrence, such as from donation after circulatory death (DCD) donors. Here, we describe two patients with HUS who underwent a KT from an uncontrolled DCD (uDCD).
CASE SUMMARY
METHODS
The first patient, affected by aHUS due to a heterozygous deletion in CFHR3-CFHR1 and a novel heterozygous variant in CFHR5 gene, underwent a KT with eculizumab prophylaxis. The patient did not experience a post-transplant aHUS recurrence. The second patient, who experienced an HUS episode characterized by a hypertensive crisis and with no underlying mutations in complement system genes, underwent a KT without eculizumab prophylaxis. At day 5, anti-complement treatment commenced due to hematological signs of thrombotic microangiopathy (TMA). After the introduction of eculizumab, we observed a stabilization of kidney function and hematological remission.
CONCLUSION
CONCLUSIONS
We present herein two different patients with HUS who both underwent successful KT from uDCD donation under the umbrella of eculizumab therapy. Taking into account the importance of increasing the number of organs available for transplantation, uDCD could represent an additional resource in this subset of HUS patients.
Identifiants
pubmed: 34084691
doi: 10.5414/CNCS110434
pmc: PMC8170123
doi:
Types de publication
Case Reports
Langues
eng
Pagination
59-66Informations de copyright
© Dustri-Verlag Dr. K. Feistle.
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