Anesthetic management of living donor liver transplantation for complement factor H deficiency hemolytic uremic syndrome: a case report

Park, Suk-Hee; Kim, Gaab-Soo

doi:10.4097/kjae.2014.66.6.481

Cited by 4 publications

(2 citation statements)

References 16 publications

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“…We were recently informed of an additional patient with a CFH mutation who died from fungal infection 3 months post-CLKT prepared with PE + eculizumab (personal communication from G. Lipkin, Birmingham, UK). Lastly, a child with a CFH mutation has recently been reported who was cured from aHUS after preemptive living-non-related donor LT under PE/PI coverage [146]. Although most groups would rather take the option of kidney transplantation under eculizumab, CKLT should not be discarded and all options should be discussed with patients and families.…”

Section: Kidney Transplantation For Patients With Atypical Hus In 2014mentioning

confidence: 99%

An international consensus approach to the management of atypical hemolytic uremic syndrome in children

et al. 2015

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Atypical hemolytic uremic syndrome (aHUS) emerged during the last decade as a disease largely of complement dysregulation. This advance facilitated the development of novel, rational treatment options targeting terminal complement activation, e.g., using an anti-C5 antibody (eculizumab). We review treatment and patient management issues related to this therapeutic approach. We present consensus clinical practice recommendations generated by HUS International, an international expert group of clinicians and basic scientists with a focused interest in HUS. We aim to address the following questions of high relevance to daily clinical practice: Which complement investigations should be done and when? What is the importance of anti-factor H antibody detection? Who should be treated with eculizumab? Is plasma exchange therapy still needed? When should eculizumab therapy be initiated? How and when should complement blockade be monitored? Can the approved treatment schedule be modified? What approach should be taken to kidney and/or combined liver-kidney transplantation? How should we limit the risk of meningococcal infection under complement blockade therapy? A pressing question today regards the treatment duration. We discuss the need for prospective studies to establish evidence-based criteria for the continuation or cessation of anticomplement therapy in patients with and without identified complement mutations.

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Section: Kidney Transplantation For Patients With Atypical Hus In 2014mentioning

confidence: 99%

An international consensus approach to the management of atypical hemolytic uremic syndrome in children

et al. 2015

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“…Since 2002, approximately 25 patients who have undergone LT alone or CLKT for aHUS have been reported in the literature ( Table 2 ). 7 18 19 20 21 22 23 24 25 26 27 28 29 30 31 The outcomes of LT have been variable, ranging from the demise of the patients from LT complications 23 24 to a successful long-term outcome. 8 9 21 In the current study, we report two more cases with a successful clinical course of CLKT.…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2018

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BackgroundAtypical hemolytic uremic syndrome (aHUS) is a rare disease that is often associated with genetic defects. Mutations of complement factor H (CFH) are the most common genetic defects that cause aHUS and often result in end-stage renal disease. Since CFH is mainly produced in the liver, liver transplantation (LT) has been performed in patients with defective CFH.MethodsThe clinical courses of four kidney allograft recipients who lost their native kidney functions due to aHUS associated with a CFH mutation were reviewed.ResultsSubject A underwent kidney transplantation (KT) twice, aHUS recurred and the allograft kidney failed within a few years. Subject B received a KT and soon experienced a recurrence of aHUS coinciding with infection. Her allograft kidney function has worsened, and she remains on plasma infusion therapy. Subject C underwent LT followed by KT. She is doing well without plasma infusion therapy after combined LT-KT for 3 years. Subject D received KT following LT and is now recurrence-free from aHUS.ConclusionIn patients with aHUS associated with a CFH mutation, KT without LT was complicated with a recurrence of aHUS, which might lead to allograft loss. Conversely, LT was successful in preventing the recurrence of aHUS and thus might be another option for a recurrence-free life for aHUS patients associated with CFH mutation.

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Atypical Hemolytic Uremic Syndrome

Malina

Frémeaux‐Bacchi

Johnson

2023

Pediatric Kidney Disease

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Anesthetic management of living donor liver transplantation for complement factor H deficiency hemolytic uremic syndrome: a case report

Cited by 4 publications

References 16 publications

An international consensus approach to the management of atypical hemolytic uremic syndrome in children

An international consensus approach to the management of atypical hemolytic uremic syndrome in children

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

Atypical Hemolytic Uremic Syndrome

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