Hemolytic Uremic Syndrome
- PDF / 1,017,151 Bytes
- 11 Pages / 595.276 x 790.866 pts Page_size
- 89 Downloads / 194 Views
Hot Topic Article (B Dixon and E Nehus, Section Editors)
Hemolytic Uremic Syndrome Gema Ariceta, MD, PhD Address Division of Pediatric Nephrology, Hospital Universitari Vall d’Hebron, Universitat Autonoma Barcelona, Hospital Infantil 2ª planta, 119-129, 08035, Barcelona, Spain Email: [email protected]
* Springer Nature Switzerland AG 2020
Keywords Hemolytic uremic syndrome I Shiga toxin-producing Escherichia coli I Thrombotic microangiopathy I Complement
Abstract Purpose of review The emergence of specific treatment of atypical hemolytic uremic syndrome (aHUS) using eculizumab has allowed renal function recovery and safe kidney transplantation (KT). Early and precise recognition of complement-mediated (CM) atypical HUS (aHUS) is challenging and differs between children and adults. Our aim is to facilitate a decision-making process on HUS management. Recent findings Hemolytic uremic syndrome (HUS) represents a heterogeneous group of diseases characterized by systemic endothelial cell injury, most often involving kidney, and which leads to thrombotic microangiopathy (TMA). Many inherited factors and environmental triggers cause HUS, but in children and the young, 90% are related to Shiga toxin-producing-Escherichia coli (STEC). Most non-STEC-HUS present primary complement (C) dysregulation and defective protection against C injury at the endothelial surface. Before eculizumab, CM-aHUS mortality was 60%, and progression to end stage kidney disease the rule, with frequent relapses after KT. Worldwide, the most common questions are who could benefit the most from C blockade, the most appropriate monitoring strategy for these individuals, and optimal duration of treatment with eculizumab. Summary HUS is a life-threatening disease with poor outcome if untreated. Early and clear distinction between aHUS from other primary TMA (thrombotic thrombocytopenic-purpura, STEC-HUS), rare non-CM-aHUS, and secondary TMA is required as clinical features overlap. The current approach is terminology consensus, classification based on disease pathogenic mechanisms, and patient risk stratification.
Introduction Hemolytic uremic syndrome (HUS) is an entity defined by the simultaneous occurrence of non-immune microangiopathic anemia, thrombocytopenia, and kidney injury [1, 2••]. This syndrome was first described in four patients in 1955 by Gasser et al. [3]. In 1964, Gianantonio et al. [4] published a large outbreak of 54
sporadic cases that took place in Argentina that were preceded by bloody diarrhea. In 1982, Karmali et al. [5] identified verotoxin (a shiga-like toxin) (VT) produced by Escherichia coli (VTEC) as the etiological agent causing HUS, allowing identification of the most frequent pathogenic mechanism. Other severe forms of
Hot Topic Article (B Dixon and E Nehus, Section Editors) “atypical” HUS (aHUS) without diarrhea, previously named as “diarrhea negative HUS,” were subsequently recognized, and few years later, familial cases of HUS were first described [6]. In 1997, familial aHUS was linked to complement FH (CFH)
Data Loading...
