Open AccessJournal Article
Hypocomplementaemia due to a genetic deficiency of beta 1H globulin.
R A Thompson,M H Winterborn +1 more
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An 8-month-old Asian boy who presented with the haemolytic uraemic syndrome was found to have a low haemologic complement, and in particular a very low C3 level, with a normal C4 level, which indicated increased alternative pathway activation.Abstract:
An 8-month-old Asian boy who presented with the haemolytic uraemic syndrome was found to have a low haemolytic complement, and in particular a very low C3 level, with a normal C4 level. These abnormalities persisted after recovery and were not associated with the presence of circulating C3 nephritic factor. A clinically healthy 3-year-old brother was found to have an identical complement profile, which indicated increased alternative pathway activation. Both brothers had normal levels of the C3b inactivator, but very low levels of beta 1H globulin (less than 10% of a reference standard serum). The parents, who were first cousins, had half-normal levels of beta 1H globulin, and low levels were found in other members of the family, indicating that the defect was inherited.read more
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Atypical Hemolytic–Uremic Syndrome
Marina Noris,Giuseppe Remuzzi +1 more
TL;DR: Current concepts about the pathobiology of atypical hemolytic–uremic syndrome are reviewed and its diagnosis and management are reviewed.
Journal ArticleDOI
Syndromes of Thrombotic Microangiopathy
James N. George,Carla M. Nester +1 more
TL;DR: This review article covers the diverse pathophysiological pathways that can lead to microangiopathic hemolytic anemia and a procoagulant state with or without damage to the kidneys and other organs.
Journal ArticleDOI
The human complement factor H: functional roles, genetic variations and disease associations.
Santiago Rodríguez de Córdoba,Jorge Esparza-Gordillo,Elena Goicoechea de Jorge,Margarita López-Trascasa,Pilar Sánchez-Corral +4 more
TL;DR: This review summarises the current knowledge of the role of factor H in health and disease and outlines the functional domains responsible for these regulatory activities in factor H.
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Hemolytic Uremic Syndrome
Marina Noris,Giuseppe Remuzzi +1 more
TL;DR: In this paper, a review summarizes current concepts about the epidemiological findings, the pathological and clinical aspects of STEC-HUS, pneumococcal HUS, and aHUS and their diagnosis and management.
Journal ArticleDOI
Atypical hemolytic uremic syndrome
TL;DR: Case reports and two phase II trials show an impressive efficacy of the complement C5 blocker eculizumab, suggesting it will be the next standard of care.
References
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Journal ArticleDOI
Modulation of the alternative complement pathways by beta 1 H globulin.
TL;DR: The kinetics of inactivation of C3b by C3B inactivator and beta 1 H are first order, suggesting that potentiation is not a multistep process.
Journal Article
Activation of the Alternate Pathway of Human Complement by Rabbit Cells
TL;DR: Findings show that a rabbit cell surface component can activate the alternate pathway of complement in human serum and suggest that this activation does not involve antibody.
Journal ArticleDOI
ISOLATION OF ß1F-GLOBULIN FROM HUMAN SERUM AND ITS CHARACTERIZATION AS THE FIFTH COMPONENT OF COMPLEMENT
TL;DR: The hypothesis that C'5 and C'6 form a functional unit was supported by the finding that both components interact with each other in solution resulting in the formation of a complex.
Journal ArticleDOI
Reactive lysis: the complement-mediated lysis of unsensitized cells i. the characterization of the indicator factor and its identification as c7
R. A. Thompson,P. J. Lachmann +1 more
TL;DR: The phenomenon of reactive lysis represents complement-mediated lysis of unsensitized cells initiated at the C5 stage by a stable complex (C56) which was generated by complement activation at a distance.
Journal ArticleDOI
Modulation of C3b hemolytic activity by a plasma protein distinct from C3b inactivator.
Keith Whaley,Shaun Ruddy +1 more
TL;DR: A human plasma protein binds to cell-bound C3b, the major cleavage product of the third component of complement, and thereafter no longer functions in either the classical or alternative pathways.
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