Factor H autoantibody is associated with atypical hemolytic uremic syndrome in children in the United Kingdom and Ireland [PDF]
Factor H autoantibodies can impair complement regulation, resulting in atypical hemolytic uremic syndrome, predominantly in childhood. There are no trials investigating treatment, and clinical practice is only informed by retrospective cohort ...
Almond, M +23 more
core +1 more source
Factor H gene variants in Japanese: Its relation to atypical hemolytic uremic syndrome [PDF]
Mutations and polymorphisms of factor H gene (FH1) are known to be closely involved in the developmentof atypical hemolytic uremic syndrome (aHUS). Several groups have identified disease risk mutations andpolymorphisms of FH1 for the development of aHUS,
Fujihara Noriko +10 more
core +1 more source
Thrombotic microangiopathies: thrombotic thrombocytopenic purpura / hemolytic uremic syndrome [PDF]
Thrombotic microangiopathies (TMAs) are pathological conditions characterized by generalized microvascular occlusion by platelet thrombi, thrombocytopenia, and microangiopathic hemolytic anemia.
Mastroianni Kirsztajn, Gianna +1 more
core +2 more sources
A young woman presented with an acute ST-segment elevation myocardial infarction. Her clinical course was complicated by cardiogenic shock and acute renal failure. Work-up revealed thrombocytopenia and hemolytic anemia.
Edward Chau, MD, MS +8 more
doaj +1 more source
ABSTRACT Hematologic emergencies are urgent health conditions which result in significant mortality and morbidity unless timely therapeutic measures are taken. Therapeutic success depends on their timely and accurate recognition by hematology laboratory services.
Ganna Shestakova +2 more
wiley +1 more source
Eculizumab experience in an adult patient with atypical hemolytic uremic syndrome
Atypical hemolytic-uremic syndrome is a disease characterized by nonimmune hemolytic anemia, thrombocytopenia, and renal failure. In this study, we present a case of a patient with atypical hemolytic-uremic syndrome treated successfully with eculizumab ...
Funda Sari +6 more
doaj +1 more source
The major autoantibody epitope on factor H in atypical hemolytic uremic syndrome is structurally different from its homologous site in factor H-related protein 1, supporting a novel model for induction of autoimmunity in this disease [PDF]
Atypical hemolytic uremic syndrome (aHUS) is characterized by complement attack against host cells due to mutations in complement proteins or autoantibodies against complement factor H (CFH).
Abarrategui-Garrido +59 more
core +1 more source
ABSTRACT In endemic areas, acute undifferentiated febrile illness has a wide differential with overlapping features that can lead to misdiagnosis. Although triple co‐infection with scrub typhus, dengue, and leptospirosis is extremely rare, high clinical suspicion and early detection are vital to avoid delayed treatment, multi‐organ dysfunction, and ...
Sulav Kumar Jha +5 more
wiley +1 more source
When the environment and mutations affect organ systems [PDF]
Atypical hemolytic uremic syndrome (aHUS) is a rare thrombotic microangiopathy (TMA) with a genetic predisposition. Like other TMAs, it presents clinically with thrombocytopenia and microangiopathic hemolytic anemia, which is accompanied by disruption of
Abu Ghanimeh, Mouhanna +3 more
core +1 more source
Meningococcal Serogroup Y Meningitis Reveals Inborn Factor B Deficiency
We report a novel case of complete complement Factor B deficiency revealed by invasive meningococcal disease. Using combined functional and genetic analyses, we demonstrate that loss of Factor B abolishes alternative pathway activity despite detectable protein levels. These findings provide a mechanistic framework relevant to complement diagnostics and
Camille Bougeard +9 more
wiley +1 more source

