Results 61 to 70 of about 8,636 (197)

Atypical hemolytic uremic syndrome and mutation analysis of factor H gene in two Tunisian families [PDF]

, 2010
Imen Habibi1,Imen Sfar1,Walid Ben Alaya1, Jihen Methlouthi2, Abdelkrim Ayadi2, Mounira Brahim2, Jacques Blouin3, Raoudha Dhagbouj1, Thouraya Ben Rhomdhane1, Mouna Makhlouf1, Houda Aouadi1, Saloua Ayed-Jendoubi1, Véronique Fremeaux-bacchi3 ...
et al, Walid Ben Alaya, Imen Habibi, Imen Sfar   +3 more
core  

Complement mutations in Diacylglycerol Kinase-E-associated atypical hemolytic uremic syndrome. [PDF]

, 2014
32 p.-4 tab-3 fig.-2 tab. suppl.Background and objectives Atypical hemolytic uremic syndrome is characterized by vascular endothelial damage caused by complement dysregulation.
Roldán, María Luisa Justa, Rodríguez de Córdoba, Santiago, Sheila Pinto, Sánchez-Corral, Pilar, Maria Luisa Justa Roldan, Marta Adragna, Adragna, Marta, López, Laura, Laura Lopez, Hoppe, Bernd, Antonia Peña, Peña, Antonia, López-Trascasa, Margarita, Sánchez Chinchilla, Daniel, Margarita Lopez Trascasa, Daniel Sánchez Chinchilla, Santiago Rodríguez de Córdoba, Bernd Hoppe, Pilar Sánchez-Corral, Pinto, Sheila   +19 more
core   +1 more source

Reduced membrane attack complex formation in umbilical cord blood during Eculizumab treatment of the mother: a case report

BMC Nephrology, 2019
Background Atypical hemolytic uremic syndrome (aHUS) is a disorder of the microvasculature with hemolytic anemia, thrombocytopenia and acute kidney injury.
Subagini Nagarajah, Martin Tepel, Christian Nielsen, Kristian Assing, Yaseelan Palarasah, Lise Lotte Torvin Andersen, Lotte Borg Lange, Claus Bistrup   +7 more
doaj   +1 more source

Discontinuation of Eculizumab treatment after hematological remission in patients with atypical and drug-induced hemolytic uremic syndrome

Romanian Journal of Internal Medicine, 2022
Introduction. The aim was to evaluate the effect of therapeutic plasma exchange (TPE) and eculizumab on hematological and renal survival in atypical hemolytic uremic syndrome (aHUS), and additionally, to examine the reliability of discontinuation of ...
Yeter Hasan H., Derici Ulver, Arinsoy Turgay, Altok Kadriye, Erten Yasemin, Guz Galip   +5 more
doaj   +1 more source

Complement Inhibition in the Clinic: Are We Doing Enough to Protect Patients From Infection?

European Journal of Immunology, Volume 56, Issue 7, July 2026.
Excessive complement activation is implicated in a broad range of diseases. Therapeutic approaches targeting the complement cascade, from pathway‐selective inhibition to terminal blockade, can effectively control disease activity. However, increasing degrees of complement inhibition are associated with a heightened susceptibility to bacterial, viral ...
Serena Bettoni, Ebba Qviberg, Edward Chaloner, Hayley Lavender, Maisem Laabei   +4 more
wiley   +1 more source

Critical appraisal of eculizumab for atypical hemolytic uremic syndrome [PDF]

, 2016
Lilian M Pereira Palma,1 Craig B Langman2  1Pediatric Nephrology, State University of Campinas (UNICAMP), Campinas, São Paulo, Brazil; 2The Feinberg School of Medicine, Northwestern University, and the Ann and Robert H.
Palma LMP, Langman CB
core  

First Revision of the Guidelines for the Diagnosis and Management of Remethylation Disorders

Journal of Inherited Metabolic Disease, Volume 49, Issue 4, July 2026.
ABSTRACT This guideline summarizes diagnostic and therapeutic approaches based on a systematic literature review and evidence evaluation using the GRADE methodology. Given the limited high‐quality data, expert consensus was additionally obtained through a modified Delphi process.
Giorgia Olivieri, Andrea Bordugo, Birute Burnyte, Alessandro Costetti, Maria‐Luz Couce, Luisa Diogo, Francois Feillet, D. Sean Froese, Benedetta Greco, Jean‐Louis Gueant, Luciana Hannibal, Friederike Hörster, Viktor Kožich, Giancarlo La Marca, Irini Manoli, Fanny Mochel, Lorenzo Orazi, Belén Pérez, Manuel Schiff, Bernd C. Schwahn, Ida Schwartz, Karolina M. Stepien, Jolanta Sykut‐Cegielska, Trine Tangeraas, Rolf H. Zetterström, Carlo Dionisi‐Vici, Martina Huemer   +26 more
wiley   +1 more source

Plasma Volume to Achieve Remission in Atypical Hemolytic Uremic Syndrome [PDF]

, 2019
Introduction: Atypical hemolytic uremic syndrome (HUS) is defined as a heterogeneous group of disorders. Plasma infusion or plasma exchange is the rescue therapy for this life-threatening syndrome.
Nakysa, Hooman, Mahdaynia, Soheila, Hoseini-Shamsabadi, Rozita, Otukesh, Hasan, Nickavar, Azar   +4 more
core   +2 more sources

Complement activation in atypical hemolytic uremic syndrome and scleroderma renal crisis: a critical analysis of pathophysiology

Brazilian Journal of Nephrology, 2018
Scleroderma is an autoimmune disease that affects multiple systems. While pathophysiologic mechanisms governing the development of scleroderma are relatively poorly understood, advances in our understanding of the complement system are clarifying the ...
Roman Zuckerman, Arif Asif, Eric J. Costanzo, Tushar Vachharajani   +3 more
doaj   +1 more source

Vitamin‐Responsive Disorders: From Molecular Basis to Clinical Presentation and Therapy

Journal of Inherited Metabolic Disease, Volume 49, Issue 4, July 2026.
ABSTRACT Vitamin‐dependent cofactors are essential for numerous metabolic reactions, and defects affecting their uptake, conversion, utilisation, or regeneration constitute a heterogeneous group of inherited metabolic disorders (IMDs). Although dietary vitamin intake is sufficient to sustain coenzyme synthesis in healthy individuals, it is insufficient
Cécile Acquaviva, Yann Nadjar, Manuel Schiff, Christine Vianey‐Saban, Alain Fouilhoux, Jean‐François Benoist   +5 more
wiley   +1 more source