Kidney Transplantation in Patients with Atypical Hemolytic Uremic Syndrome due to Complement Factor H Deficiency: Impact of Liver Transplantation

Kim, Se-Jin; Park, Eujin; Min, Sang Il; Yi, Nam Joon; Ha, Jong‐Won; Ha, Il Soo; Cheong, Hae Il; Kang, Hee Gyung

doi:10.3346/jkms.2018.33.e4

Cited by 4 publications

(2 citation statements)

References 35 publications

(45 reference statements)

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“…Despite the high risk of aHUS recurrence in patients with CFH mutations, prophylactic administration of eculizumab has been demonstrated to effectively prevent aHUS recurrence in a significant proportion of patients. Additionally, since CFH is primarily produced in the liver, liver transplantation could be an alternative treatment option [ 39 , 40 ].…”

Section: Evaluation Prevention and Treatment Of Post-kidney Transplan...mentioning

confidence: 99%

Characteristics and management of thrombotic microangiopathy in kidney transplantation

Cho

Jung

et al. 2023

Korean J Transplant

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Thrombotic microangiopathy is not a rare complication of kidney transplantation and is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury with extensive thrombosis of the arterioles and capillaries. Various factors can cause thrombotic microangiopathy after kidney transplantation, including surgery, warm and cold ischemia-reperfusion injury, exposure to immunosuppressants, infection, and rejection. Many recent studies on atypical hemolytic uremic syndrome have described genetic abnormalities related to excessive activation of the alternative complement pathway. The affected patients' genetic backgrounds revealed significant genetic heterogeneity in several genes involved in complement regulation, including the complement factor H, complement factor H-related proteins, complement factor I, complement factor B, complement component 3, and CD46 genes in the alternative complement pathway. Although clinical studies have provided a better understanding of the pathogenesis of diseases, the diverse triggers present in the transplant environment can lead to thrombotic microangiopathy, along with various genetic predispositions, and it is difficult to identify the genetic background in various clinical conditions. Given the poor prognosis of posttransplant thrombotic microangiopathy, further research is necessary to improve the diagnosis and treatment protocols based on risk factors or genetic predisposition, and to develop new therapeutic agents.

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Section: Evaluation Prevention and Treatment Of Post-kidney Transplan...mentioning

confidence: 99%

Characteristics and management of thrombotic microangiopathy in kidney transplantation

Cho

Jung

et al. 2023

Korean J Transplant

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“…If a patient has a CFH mutation and experiences multiple relapses, liver transplantation can be considered because the majority of plasma CFH is produced by the liver. There have been several reports of successful liver transplantation that led to no further relapses [96]. While successful liver transplantation can cure CFH-associated recurrent aHUS, recipients of liver transplantation require immunosuppressive agents to prevent rejection, which leads to an immunocompromised state and an increased risk of infection and malignancy.…”

Section: Treatment Of Pediatric Ahusmentioning

confidence: 99%

Consensus regarding diagnosis and management of atypical hemolytic uremic syndrome

Lee

Kang

et al. 2020

Korean J Intern Med

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Thrombotic microangiopathy (TMA) is defined by specific clinical characteristics, including microangiopathic hemolytic anemia, thrombocytopenia, and pathologic evidence of endothelial cell damage, as well as the resulting ischemic end-organ injuries. A variety of clinical scenarios have features of TMA, including infection, pregnancy, malignancy, autoimmune disease, and medications. These overlapping manifestations hamper differential diagnosis of the underlying pathogenesis, despite recent advances in understanding the mechanisms of several types of TMA syndrome. Atypical hemolytic uremic syndrome (aHUS) is caused by a genetic or acquired defect in regulation of the alternative complement pathway. It is important to consider the possibility of aHUS in all patients who exhibit TMA with triggering conditions because of the incomplete genetic penetrance of aHUS. Therapeutic strategies for aHUS are based on functional restoration of the complement system. Eculizumab, a monoclonal antibody against the terminal complement component 5 inhibitor, yields good outcomes that include prevention of organ damage and premature death. However, there remain unresolved challenges in terms of treatment duration, cost, and infectious complications. A consensus regarding diagnosis and management of TMA syndrome would enhance understanding of the disease and enable treatment decision-making.

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Deceased vs living donor grafts for pediatric simultaneous liver‐kidney transplantation: A single‐center experience

Gautier

Monakhov

Tsiroulnikova

et al. 2020

Clinical Laboratory Analysis

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Introduction:In conditions of limited experience of pediatric simultaneous liver-kidney transplantation (SLKT) using grafts from living and deceased donors, there is a certain need to validate the approach.Patients: The retrospective study of 18 pediatric patients who received SLKT between 2008 and 2019.Results: Grafts were obtained from both living and deceased donors. The patients' age ranged from 2 to 16 years (9 years ±4). The body weight of the children varied from 9.5 to 39 kg (22 kg ±9). The follow-up period lasted from 1 to 109 months (median 38 months ±35). The various graft combinations were used in both groups. There was no mortality during the follow-up. There was no significant difference in baseline parameters in recipients who received grafts from living and deceased donors except age (7.5 years ±2.2 vs 11.8 years ±4.1; P = .038). Rate of complications > grade II was higher among recipients of deceased donor SLKT (7.7% vs 60%; OR, 7.8; 95% CI, 1.04-58.48; P = .044). All the patients are alive with both grafts functioning. All the living donors returned to the normal life. Conclusion:SLKT is a safe and effective procedure for children with both simultaneous end-stage liver disease and end-stage renal disease. Both living donor partial liver and kidney transplantation and deceased donor liver-kidney transplantation can be considered as safe and feasible options.

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Kidney Transplantation in Patients with Atypical Hemolytic Uremic Syndrome due to Complement Factor H Deficiency: Impact of Liver Transplantation

Cited by 4 publications

References 35 publications

Characteristics and management of thrombotic microangiopathy in kidney transplantation

Characteristics and management of thrombotic microangiopathy in kidney transplantation

Consensus regarding diagnosis and management of atypical hemolytic uremic syndrome

Deceased vs living donor grafts for pediatric simultaneous liver‐kidney transplantation: A single‐center experience

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