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Atypical HUS

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https://www.readbyqxmd.com/read/29152520/a-case-of-escherichia-coli-hemolytic-uremic-syndrome-in-a-10-year-old-male-with-severe-neurologic-involvement-successfully-treated-with-eculizumab
#1
Malia Rasa, James Musgrave, Keith Abe, Len Tanaka, Konstantine Xoinis, Bruce Shiramizu, Gretchen Foskett, Rhiana Lau
Hemolytic uremic syndrome (HUS) can be classified as typical and atypical, and the treatment recommendations currently differ between the 2 types. Eculizumab is recommended as first-line treatment for atypical HUS; however, its use in typical HUS has been controversial. We report a case of a 10-year-old male with severe neurologic impairment who was successfully treated with eculizumab, which was started 4 days after onset of neurologic symptoms. Our case supports the use of eculizumab in typical HUS with neurologic involvement, even when given later in the course, as the pathophysiology of typical HUS has been shown to involve activation of the complement pathway, similar to atypical HUS...
October 2017: Journal of Investigative Medicine High Impact Case Reports
https://www.readbyqxmd.com/read/29142942/complement-activation-induces-neutrophil-adhesion-and-neutrophil-platelet-aggregate-formation-on-vascular-endothelial-cells
#2
Magdalena Riedl, Damien G Noone, Meraj A Khan, Fred G Pluthero, Walter H A Kahr, Nades Palaniyar, Christoph Licht
Introduction: Atypical hemolytic uremic syndrome is a thrombotic microangiopathy, which is linked to hereditary or autoimmune defects in complement activators or regulators present in blood and on vascular endothelial cells. Acute thrombotic microangiopathy episodes are typically preceded by infections, which by themselves would not be expected to manifest HUS. Thus, it is possible that the host immune response contributes to the precipitation of aHUS. However, the mechanisms involved are not fully understood...
January 2017: KI Reports
https://www.readbyqxmd.com/read/28947259/-hemolytic-and-uremic-syndrome-and-related-thrombotic-microangiopathies-treatment-and-prognosis
#3
C Rafat, P Coppo, F Fakhouri, V Frémeaux-Bacchi, C Loirat, J Zuber, E Rondeau
Major achievements in the understanding of thrombotic microangiopathies (TMA) have not only resulted in a reclassification of TMA but most of all they have culminated in the design of new treatments and have enabled clinicians to better delineate their prognosis. Recent multicenter studies have improved our understanding of the prognosis of atypical hemolytic and uremic syndromes (aHUS). More specifically, they have highlighted the role of genetic testing on predicting the recurrence of aHUS, the risk of chronic kidney disease and the recurrence following kidney transplantation...
September 22, 2017: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/28939980/targeted-exome-sequencing-in-anti-factor-h-antibody-negative-hus-reveals-multiple-variations
#4
R W Thergaonkar, Ankita Narang, Bahadur Singh Gurjar, Pradeep Tiwari, Mamta Puraswani, Himanshi Saini, Aditi Sinha, Binuja Varma, Mitali Mukerji, Pankaj Hari, Arvind Bagga
BACKGROUND: Genetic susceptibility to atypical hemolytic uremic syndrome (aHUS) may lie within genes regulating or activating the alternate complement and related pathways converging on endothelial cell activation. METHODS: We tested 32 Indian patients of aHUS negative for antibodies to complement factor H for genetic variations in a panel of 15 genes, i.e., CFH, CFHR1-5, CFI, CFB, C3, CD46, MASP2, DGKE, ADAMTS13, THBD and PLG using next-generation DNA sequencing and for copy number variation in CFHR1-3...
September 22, 2017: Clinical and Experimental Nephrology
https://www.readbyqxmd.com/read/28899876/treatment-and-management-of-children-with-haemolytic-uraemic-syndrome
#5
REVIEW
Patrick R Walsh, Sally Johnson
Haemolytic uraemic syndrome (HUS), comprising microangiopathic haemolytic anaemia, thrombocytopaenia and acute kidney injury, remains the leading cause of paediatric intrinsic acute kidney injury, with peak incidence in children aged under 5 years. HUS most commonly occurs following infection with Shiga toxin-producing Escherichia coli (STEC-HUS). Additionally, HUS can occur as a result of inherited or acquired dysregulation of the alternative complement cascade (atypical HUS or aHUS) and in the setting of invasive pneumococcal infection...
September 12, 2017: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/28835862/late-onset-cobalamin-disorder-and-hemolytic-uremic-syndrome-a-rare-cause-of-nephrotic-syndrome
#6
Gianluigi Ardissino, Michela Perrone, Francesca Tel, Sara Testa, Amelia Morrone, Ilaria Possenti, Francesco Tagliaferri, Robertino Dilena, Francesca Menni
Hemolytic uremic syndrome (HUS) is an unrare and severe thrombotic microangiopathy (TMA) caused by several pathogenetic mechanisms among which Shiga toxin-producing Escherichia coli infections and complement dysregulation are the most common. However, very rarely and particularly in neonates and infants, disorders of cobalamin metabolism (CblC) can present with or be complicated by TMA. Herein we describe a case of atypical HUS (aHUS) related to CblC disease which first presented in a previously healthy boy at age of 13...
2017: Case Reports in Pediatrics
https://www.readbyqxmd.com/read/28729338/a-new-therapeutic-strategy-for-atypical-hus
#7
Joel L Moake
No abstract text is available yet for this article.
July 20, 2017: Blood
https://www.readbyqxmd.com/read/28720077/hemolytic-uremic-syndrome-as-the-presenting-manifestation-of-wt1-mutation-and-denys-drash-syndrome-a-case-report
#8
Joseph L Alge, Scott E Wenderfer, John Hicks, Mir Reza Bekheirnia, Deborah A Schady, Jamey S Kain, Michael C Braun
BACKGROUND: Hemolytic uremic syndrome (HUS) can occur as a primary process due to mutations in complement genes or secondary to another underlying disease. HUS sometimes occurs in the setting of glomerular diseases, and it has been described in association with Denys-Drash syndrome (DDS), which is characterized by the triad of abnormal genitourinary development; a pathognomonic glomerulopathy, diffuse mesangial sclerosis; and the development of Wilms tumor. CASE PRESENTATION: We report the case of a 46, XX female infant who presented with HUS and biopsy-proven thrombotic microangiopathy...
July 18, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28711077/acute-kidney-injury-in-pregnancy
#9
Belinda Jim, Vesna D Garovic
Pregnancy-related acute kidney injury (AKI) has declined in incidence in the last three decades, although it remains an important cause of maternal and fetal morbidity and mortality. Pregnancy-related causes of AKI such as preeclampsia, acute fatty liver of pregnancy, HELLP (Hemolysis, Elevated Liver function tests, Low Platelets) syndrome, and the thrombotic microangiopathies (thrombotic thrombocytopenic purpura, atypical hemolytic-uremic syndrome [HUS]) exhibit overlapping features and often present as diagnostic dilemmas...
July 2017: Seminars in Nephrology
https://www.readbyqxmd.com/read/28682564/-complement-factor-b-mutation-in-atypical-hemolytic-uremic-syndrome-rare-cause-of-rare-disease
#10
Luca Visconti, Valeria Cernaro, Gianluigi Ardissino, Martina Sgarbanti, Domenico Ferrara, Giuseppe Visconti, Domenico Santoro, Michele Buemi
Hemolytic uremic syndrome (HUS) is a rare disease characterized by microangiopathic hemolysis, platelet consumption and multiple organ failure with predominant renal involvement. In the most of cases (85-90%), it is associated with enteric infection due to Shiga-toxin or verocytotoxin (STEC-VTEC)-producer Escherichia coli. Rarely, in about 10-15% of cases, HUS develops in the presence of a disorder of alternative complement pathway regulation and it is defined atypical (aHUS). We describe the case of a 65-year-old man who came to our attention with a clinical presentation of aHUS and a clinical course characterized by rapidly progressive acute renal failure (ARF), which required renal replacement treatments, and by a stable clinical picture of hematological impairment as a marker of a non-severe and self-limiting form...
April 2017: Giornale Italiano di Nefrologia: Organo Ufficiale Della Società Italiana di Nefrologia
https://www.readbyqxmd.com/read/28647502/alternative-complement-pathway-hemolytic-assays-reveal-incomplete-complement-blockade-in-patients-treated-with-eculizumab
#11
Bénédicte Puissant-Lubrano, Sylvain Puissochet, Nicolas Congy-Jolivet, Dominique Chauveau, Stéphane Decramer, Arnaud Garnier, Antoine Huart, Nassim Kamar, David Ribes, Antoine Blancher
Eculizumab is a monoclonal anti-C5 antibody used in the treatment of atypical hemolytic uremic syndrome (aHUS). We monitored complement inhibition in 16 eculizumab-treated patients suffering from HUS or transplant rejection (not aHUS patients). Blood samples were obtained one to four weeks after the last eculizumab injection. We observed that eculizumab efficiently blocked the terminal pathway (TP) through classical pathway (CP) activation measured by kinetic hemolytic assay (HA) (<10%) but incompletely blocked the TP through alternative pathway (AP) activation measured by rabbit (APH50>23%) or chicken erythrocytes HA (AP100>15%)...
June 21, 2017: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/28509134/two-cases-of-atypical-hemolytic-uremic-syndrome-ahus-and-eosinophilic-granulomatosis-with-polyangiitis-egpa-a-possible-relationship
#12
Mercedes Cao, Tamara Ferreiro, Bruna N Leite, Francisco Pita, Luis Bolaños, Francisco Valdés, Angel Alonso, Eduardo Vázquez, Juan Mosquera, María Trigás, Santiago Rodríguez
Atypical hemolytic uremic syndrome (aHUS) is a rare disease characterized by hemolysis, thrombocytopenia, and renal failure. It is related to genetic mutations of the alternative complement pathway and is difficult to differentiate from other prothrombotic microangiopathies. Eosinophilic granulomatosis with polyangiitis (EGPA) (Churg-Strauss syndrome, CSS) is a systemic ANCA-associated vasculitis and a hypereosinophilic disorder where eosinophils seem to induce cell apoptosis and necrosis and therefore, vasculitis...
May 2017: CEN Case Reports
https://www.readbyqxmd.com/read/28447417/diagnostic-approach-to-microangiopathic-hemolytic-disorders
#13
REVIEW
K Kottke-Marchant
Thrombotic micro-angiopathies (TMA) are a group of related disorders that are characterized by thrombosis of the microvasculature and associated organ dysfunction, and encompass congenital, acquired, and infectious etiologies. A hall mark of TMAs is the fragmentation of erythrocytes by the microvascular thrombi, resulting in a hemolytic anemia. There are several distinct pathophysiologies leading to microangiopathic hemolysis, ranging from decreased degradation of von Willebrand factor as seen in thrombotic thrombocytopenic purpura (TTP) to endothelial damage facilitated by Escherichia coli shiga toxin or complement dysregulation, seen in shiga toxin-related hemolytic-uremic syndrome (Stx-HUS) and complement-mediated TMA (also called atypical hemolytic-uremic syndrome), respectively...
May 2017: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/28439485/treatment-of-atypical-hemolytic-uremic-syndrome-in-the-era-of-eculizumab
#14
Rawaa Ebrahem, Salam Kadhem, Quoc Truong
Hemolytic-uremic syndrome (HUS) is the triad of microangiopathic hemolytic anemia (MAHA), thrombocytopenia, and acute kidney injury (AKI); the main cause of multi-organ failure is related to thrombotic microangiopathy (TMA). Atypical HUS (aHUS) is a disease of uncontrolled complement activation associated with a high mortality rate and most cases progress to end-stage renal disease. About 50% of patients with this syndrome carry mutations in genes that encode complement proteins. Also, aHUS constitutes an over-activation of the complement pathway which is either inherited, acquired, or both...
March 23, 2017: Curēus
https://www.readbyqxmd.com/read/28419995/hemolytic-uremic-syndrome-and-kidney-transplantation-a-case-series-and-review-of-the-literature
#15
Sabrina Milan Manani, Grazia Maria Virzì, Anna Giuliani, Anna Clementi, Alessandra Brocca, Daniela Dissegna, Francesca Martino, Emanuele Stefano Giovanni d''Amore, Claudio Ronco
BACKGROUND: Hemolytic uremic syndrome (HUS) can be triggered by Shiga toxin producing Escherichia coli (STEC) infection or it can be defined as atypical HUS (aHUS) if it is related to uncontrolled complement activation. aHUS is characterized by a high incidence of recurrence after kidney transplantation, and it can also occur de novo in transplant recipients. Eculizumab is used both to prevent and to treat aHUS following kidney transplantation. In this paper, we report our centre experience and we present 4 cases of HUS in patients who underwent kidney transplantation...
2017: Nephron
https://www.readbyqxmd.com/read/28416508/hus-and-atypical-hus
#16
REVIEW
T Sakari Jokiranta
Hemolytic uremic syndrome (HUS) is a thrombotic microangiopathy characterized by intravascular hemolysis, thrombocytopenia, and acute kidney failure. HUS is usually categorized as typical, caused by Shiga toxin-producing Escherichia coli (STEC) infection, as atypical HUS (aHUS), usually caused by uncontrolled complement activation, or as secondary HUS with a coexisting disease. In recent years, a general understanding of the pathogenetic mechanisms driving HUS has increased. Typical HUS (ie, STEC-HUS) follows a gastrointestinal infection with STEC, whereas aHUS is associated primarily with mutations or autoantibodies leading to dysregulated complement activation...
May 25, 2017: Blood
https://www.readbyqxmd.com/read/28387984/practical-issues-in-using-eculizumab-for-children-with-atypical-hemolytic-uremic-syndrome-in-the-acute-phase-a-review-of-4-patients
#17
Chikako Terano, Kenji Ishikura, Riku Hamada, Yasuhiro Yoshida, Wataru Kubota, Yusuke Okuda, Shunsuke Shinozuka, Ryoko Harada, Sunao Iyoda, Yoshihiro Fujimura, Yuko Hamasaki, Hiroshi Hataya, Masataka Honda
AIM: Recently eculizumab, a monoclonal antibody to C5, was found to improve the disease course of atypical hemolytic uremic syndrome (aHUS) and has been recommended as the first line treatment by an international consensus guideline. However, several practical issues in the use of eculizumab for the acute phase of aHUS have yet to be resolved. METHODS: Children who received eculizumab with diagnosis of aHUS between March 2010 and December 2015 at Tokyo Metropolitan Children's Medical Center were enrolled...
April 7, 2017: Nephrology
https://www.readbyqxmd.com/read/28356668/targeting-renin-angiotensin-system-in-malignant-hypertension-in-atypical-hemolytic-uremic-syndrome
#18
V Raghunathan, S K Sethi, M A Dragon-Durey, M Dhaliwal, R Raina, P Jha, S B Bansal, V Kher
Hypertension is common in hemolytic uremic syndrome (HUS) and often difficult to control. Local renin-angiotensin activation is believed to be an important part of thrombotic microangiopathy, leading to a vicious cycle of progressive renal injury and intractable hypertension. This has been demonstrated in vitro via enhanced tissue factor expression on glomerular endothelial cells which is enhanced by angiotensin II. We report two pediatric cases of atypical HUS with severe refractory malignant hypertension, in which we targeted the renin-angiotensin system by using intravenous (IV) enalaprilat, oral aliskiren, and oral enalapril with quick and dramatic response of blood pressure...
March 2017: Indian Journal of Nephrology
https://www.readbyqxmd.com/read/28353273/clinical-characteristics-of-children-with-hemolytic-uremic-syndrome-in-hangzhou-china
#19
Shui-Ai Zhao, Bo-Tao Ning, Jian-Hua Mao
BACKGROUND: Hemolytic uremic syndrome (HUS) is a main cause of acute renal failure in children. This study aimed to analyze the clinical characteristics of HUS. METHODS: A retrospective analysis was performed in 46 children with sporadic HUS. RESULTS: Of the 46 HUS patients, 20 (43.5%) were diarrhea-related HUS, and 26 (56.5%) were atypical HUS. Anemia, edema, oliguria, hemoglobinuria and hypertension were the most common manifestations. Thrombocytopenia, hyponatremia, hypocalcemia, hyperkalemia, metabolic acidosis, increased fibrinogen and hypocomplementemia were found in most patients...
April 2017: World Journal of Pediatrics: WJP
https://www.readbyqxmd.com/read/28347544/ocular-involvement-in-atypical-haemolytic-uraemic-syndrome
#20
A Sampedro López, B Domínguez Moro, J M Baltar Martin, C Garcia Monteavaro, J J Barbón García
CASE REPORT: The case is presented of a young man with an atypical haemolytic-uraemic syndrome (aHUS), complicated with bilateral serous retinal detachment, cotton wool spots, and a branch artery occlusion. Treatment with plasmapheresis, haemodialysis and systemic eculizumab led to the blood and urine parameters returning to normal, as well as resolution of the retinal anomalies. Genetic analysis show both mutations in complement factor H and C3. DISCUSSION: Haemolytic-uraemic syndrome (HUS) is a thrombotic microangiopathy characterised by microangiopathic haemolytic anaemia, thrombocytopenia, and acute renal failure...
March 24, 2017: Archivos de la Sociedad Española de Oftalmología
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