Interdisciplinary Expert Council Resolution. Kidney transplantation in patients with atypical  hemolytic-uremic syndrome: clinical and organizational-methodical aspects of patient  management

Experts of the Union of Pediatricians of Russia have developed modern guidelines on management of children with atypical hemolytic uremic syndrome (aHUS). aHUS is ultra-rare (orphan) disease with progressive and relapsing course during the life with potentially life-threatening state. This article covers issues of disease etiology, pathogenesis, and classification. Specific attention is paid to the diagnosis, differential diagnosis, and therapy of aHUS according to the evidence-based approaches.

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Modern Approaches to the Management of Children with Atypical Hemolytic Uremic Syndrome

Эмирова

Abaseeva

Баранов³

et al. 2022

Pediatr. farmakol.

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Atypical hemolytic-uremic syndrome in children

Burlutskaya

Статова

Ustyuzhaninа

et al. 2022

Innovacionnaâ medicina Kubani

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Background: Atypical hemolytic-uremic syndrome (aHUS) is a rare progressive form of systemic thrombotic microangiopathy (TMA), which develops due to uncontrolled activation of the alternative pathway of the complement system and is characterized by a predominant lesion of the vessels of the renal microcirculatory bed.Clinical cases description: The article describes two clinical cases of aHUS, which demonstrate the difficulties of differential diagnosis of typical and atypical HUS at the initial stages of the disease.Conclusion: Given the lack of clear laboratory criteria of aHUS, it is necessary to carry out a complex and consistent differential diagnosis of various TMA options, the exclusion of which makes it possible to make a correct diagnosis more accurately.

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Atypical hemolytic-uremic syndrome: evolution of treatment and impact of clinical and genetic characteristics on possibility of eculizumab withdrawal

Prokopenko

2022

Nefrologiâ (St.-Peterbg.)

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Atypical hemolytic uremic syndrome (aHUS) is a rare variant of thrombotic microangiopathy (TMA) associated with uncontrolled activation of alternative complement pathway due to mutations in complement regulatory protein genes or antibodies formation to regulators. Clinical manifestations of aHUS can be triggered by infections, sepsis, pregnancy, autoimmune diseases, organ transplantation, and other complement-activating conditions. Previously, the only treatment option for aHUS was plasma therapy – fresh frozen plasma infusions or plasma exchange, but its effectiveness was insufficient. Currently, targeted treatment available – recombinant monoclonal antibodies against complement C5 protein – eculizumab with high efficiency in achieving aHUS remission, renal function recovery, and preventing TMA at kidney transplantation. For a long time, the question of the optimal duration of treatment and the possibility of eculizumab discontinuing remained unresolved. It was shown that aHUS relapses developed in 20-35 % of patients after discontinuation of complement-blocking therapy. The article presents an overview of a large number of studies of eculizumab treatment outcomes and the possibility of its withdrawal, including a French prospective multicenter study that identified risk factors for aHUS relapse after eculizumab discontinuation: the presence of rare variants of complement genes, female gender, increased soluble C5b-9 plasma level. In patients who did not have rare genetic variants, the risk of relapse was less than 5 %. In general, eculizumab discontinuation after achieving complete remission of aHUS and renal function recovery in patients with low risk of recurrence can provide better tolerability of maintenance treatment, and decrease the incidence of infectious complications and the financial burden on the healthcare system.

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Interdisciplinary Expert Council Resolution. Kidney transplantation in patients with atypical hemolytic-uremic syndrome: clinical and organizational-methodical aspects of patient management

Cited by 3 publications

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Modern Approaches to the Management of Children with Atypical Hemolytic Uremic Syndrome

Modern Approaches to the Management of Children with Atypical Hemolytic Uremic Syndrome

Atypical hemolytic-uremic syndrome in children

Atypical hemolytic-uremic syndrome: evolution of treatment and impact of clinical and genetic characteristics on possibility of eculizumab withdrawal

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