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Hemolytic Uremic Syndrome

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A novel compound heterozygous mutation in DGKE in a Chinese patient causes atypical hemolytic uremic syndrome.

Jitong Li, Yinsen Song, Yaodong Zhang, Hongjiang Li, Ming Tian, Di Li, Shufeng Zhang, Guanghai Cao, Cuihua Liu,

Objectives: DGKE mutations can lead to hemolysis and thrombus in patients with atypical hemolytic uremic syndrome (aHUS). However, the sequence variants of DGKE in Chinese patients with aHUS have not been reported, and the protein function and crystal structure of DGKE remain unresolved.Methods: Targeted exome sequencing was accomplished in one ... Read more >>

Hematology (Hematology (Amsterdam, Netherlands))
[2020, 25(1):101-107]

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Unravelling the effect of a potentiating anti-Factor H antibody on atypical hemolytic uremic syndrome associated factor H variants

Gillian Dekkers, Mieke Brouwer, Jorn Jeremiasse, Angela Kamp, Robyn Biggs, Gerard van Mierlo, Scott Lauder, Suresh Katti, Taco Kuijpers, Theo Rispens, Ilse Jongerius,

The complement system plays an important role in our innate immune system. Complement activation results in clearance of pathogens, immune complex and apoptotic cells. The host is protected from complement-mediated damage by several complement regulators. Factor H (FH) is the most important fluid-phase regulator of the alternative pathway of the ... Read more >>

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Prophylactic amoxicillin for the prevention of meningococcal infection in infants with atypical hemolytic uremic syndrome under treatment with eculizumab: a report of two cases.

Kazuki Tanaka, Naoya Fujita, Satoshi Hibino,

Eculizumab, a humanized monoclonal antibody to complement C5, is a therapeutic drug for atypical hemolytic-uremic syndrome (aHUS) that inhibits the terminal pathway of complement. Patients on eculizumab therapy may become more susceptible to infection with capsule-forming bacteria, including meningococci. Therefore, meningococcal vaccination is required for patients who are on eculizumab ... Read more >>

CEN Case Rep (CEN case reports)
[2020, :]

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Peripheral Ulcerative Keratitis Secondary to Atypical Hemolytic Uremic Syndrome.

Amit V Mishra, Danielle C Cadieux, Harald Gjerde, Darrell R Lewis,

PURPOSE:To report a case of peripheral ulcerative keratitis secondary to atypical hemolytic uremic syndrome. METHODS:We report the case of a 76-year-old man who presented with bilateral aggressive peripheral ulcerative keratitis. Clinical examinations and investigations are reported from the patients' admission. RESULTS:The patient had an extended workup for autoimmune and infectious ... Read more >>

Cornea (Cornea)
[2020, :]

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[The complement system-a "hot topic" not only for kidney diseases].

Kerstin Amann, Christoph Daniel, Maike Büttner-Herold,

Increasing interest in the role of the complement system in systemic and renal disease is based on new pathophysiological and therapeutic insights of the recent past and particularly in genetic analyses in children with atypical hemolytic uremic syndrome (aHUS). aHUS is the prototypical systemic disease associated with excessive activation of ... Read more >>

Pathologe (Der Pathologe)
[2020, :]

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Isolated thrombotic microangiopathy of the small intestine in a patient with atypical hemolytic uremic syndrome - a case report.

Christoph Nunius, Maike Büttner-Herold, Simone Bertz, Mario Schiffer, Bjoern Buchholz,

BACKGROUND:Atypical hemolytic uremic syndrome (aHUS) is a rare disease characterized by systemic thrombotic microangiopathy (TMA) reflected by hemolysis, anemia, thrombocytopenia and systemic organ injury. The optimal management of aHUS-patients when undergoing kidney transplantation to prevent recurrence in the allograft is eculizumab, an approved recombinant antibody targeting human complement component C5. ... Read more >>

BMC Nephrol (BMC nephrology)
[2020, 21(1):104]

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A child with atypical hemolytic uremic syndrome: Is there a rationale to stop eculizumab?

Tanja Kersnik Levart,

Clin. Nephrol. (Clinical nephrology)
[2020, 93(4):215-216]

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Antibody response to lipopolysaccharides and recombinant proteins of Shiga toxin (Stx) producing Escherichia coli (STEC) in children with hemolytic uremic syndrome in Poland.

Waldemar Rastawicki, Karolina Śmietańska, Natalia Rokosz-Chudziak, Tomasz Wołkowicz,

Typical hemolytic uremic syndrome (STEC-HUS), caused by Shiga toxin (Stx)-producing Escherichia coli (STEC), is a serious, life-threating disease that mainly affects children. Bacteriological and genetic tests are commonly used in the routine laboratory diagnosis of STEC-HUS; however, serological methods have emerged as useful and reliable diagnostic tools, especially when bacterial ... Read more >>

Lett. Appl. Microbiol. (Letters in applied microbiology)
[2020, :]

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Epidemiology of Atypical Hemolytic Uremic Syndrome: A Systematic Literature Review.

Kevin Yan, Kamal Desai, Lakshmi Gullapalli, Eric Druyts, Chakrapani Balijepalli,

Atypical hemolytic uremic syndrome (aHUS) is a rare but severe disorder that frequently has a genetic component and results from the overactivation of the alternative complement pathway. As research moves toward improved diagnosis and therapy of aHUS, it will be important to better understand its epidemiology. Our objective was to ... Read more >>

Clin Epidemiol (Clinical epidemiology)
[2020, 12:295-305]

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Complement C3 mutation causing atypical hemolytic uremic syndrome successfully treated with eculizumab.

Moataz Ellithi, Mahum Shahid, Hafez Mohammad Abdullah, Jonathan Bleeker,

Hematol Transfus Cell Ther (Hematology, transfusion and cell therapy)
[2020, :]

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Streptococcus Pneumoniae-associated Thrombotic Microangiopathy in an Immunosuppressed Adult.

Yumi Ichikawa, Masato Murata, Makoto Aoki, Jun Nakajima, Yuta Isshiki, Yusuke Sawada, Kazunori Fukushima, Kiyohiro Oshima,

AbstractA 62-year-old male who was receiving prednisolone and methotrexate for scleroderma and rheumatoid arthritis complained of diarrhea and vomiting, and was transferred to our hospital for detailed examination and treatment of renal dysfunction and thrombocytopenia. Hemolytic anemia and crushed erythrocytes were found during the patient’s course; therefore, we suspected thrombotic ... Read more >>

(Open Medicine)
[2020, 15:204-210]

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Atypical Hemolytic Uremic Syndrome following Acute Type A Aortic Dissection.

Eigo Ikushima, Manabu Hisahara, Takuya Nishijima, Hikaru Uchiyama, Tatsushi Onzuka, Yoshie Ochiai, Tsuyoshi Muta, Shigehiko Tokunaga,

Atypical hemolytic uremic syndrome (aHUS) is a thrombotic microangiopathy (TMA)-related disease that manifests as a triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury (AKI) and is caused by uncontrolled activation of the complement system. We report the case of a 61-year-old woman with acute type A aortic dissection ... Read more >>

Case Rep Hematol (Case reports in hematology)
[2020, 2020:2467953]

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Cobalamin c deficiency associated with antifactor h antibody-associated hemolytic uremic syndrome in a young adult.

C Philipponnet, J Desenclos, M Brailova, J Aniort, J L Kemeny, C Deville, V Fremeaux-Bacchi, B Souweine, A E Heng,

BACKGROUND:Thrombotic microangiopathy (TMA) syndromes are characterized by the association of hemolytic anemia, thrombocytopenia and organ injury due to arteriolar and capillary thrombosis. CASE PRESENTATION:We report the first case of adult onset cobalamin C (Cbl C) disease associated with anti-factor H antibody-associated hemolytic uremic syndrome (HUS). A 19-year-old woman was admitted ... Read more >>

BMC Nephrol (BMC nephrology)
[2020, 21(1):96]

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Genetic Disorders of the Glomerular Filtration Barrier.

Anna S Li, Jack F Ingham, Rachel Lennon,

The glomerular filtration barrier is a highly specialized capillary wall comprising fenestrated endothelial cells, podocytes, and an intervening basement membrane. In glomerular disease, this barrier loses functional integrity, allowing the passage of macromolecules and cells, and there are associated changes in both cell morphology and the extracellular matrix. Over the ... Read more >>

Clin J Am Soc Nephrol (Clinical journal of the American Society of Nephrology : CJASN)
[2020, :]

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Evaluation of a rapid turn-over, fully-automated ADAMTS13 activity assay: a method comparison study.

Jan Stratmann, Josephine-Nana Ward, Wolfgang Miesbach,

Thrombotic thrombocytopenic purpura (TTP) is a life-threatening thrombotic microangiopathy caused by severely reduced activity of the von-Willebrand factor-cleaving protease ADAMTS13, mainly caused by anti-ADAMTS-13 antibodies. Although several test systems for ADAMTS13 measurement exist, long turn-around times hamper the usability in daily practice. We performed a method comparison study for two ... Read more >>

J. Thromb. Thrombolysis (Journal of thrombosis and thrombolysis)
[2020, :]

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Low efficacy of vaccination against serogroup B meningococci in patients with atypical hemolytic uremic syndrome.

Nils Mülling, Hana Rohn, Ulrich Vogel, Heike Claus, Benjamin Wilde, Ute Eisenberger, Andreas Kribben, Oliver Witzke, Anja Gäckler,

BACKGROUND:The C5 complement inhibitor eculizumab is first-line treatment in atypical hemolytic uremic syndrome (aHUS) going along with a highly increased risk of meningococcal infections. Serogroup B meningococci (MenB) are the most frequently encountered cause for meningococcal infections in Europe. Efficacy of the protein-based MenB-vaccine Bexsero in aHUS has not been ... Read more >>

Biosci. Rep. (Bioscience reports)
[2020, 40(3):]

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Thrombocytopenia-Associated Multiple Organ Failure.

Trung C Nguyen,

Thrombocytopenia-associated multiple organ failure is a clinical phenotype encompassing a spectrum of syndromes associated with disseminated microvascular thromboses. Autopsies performed in patients that died with thrombotic thrombocytopenic purpura, hemolytic uremic syndrome, or disseminated intravascular coagulation reveal specific findings that can differentiate these 3 entities. Significant advancements have been made in ... Read more >>

Crit Care Clin (Critical care clinics)
[2020, 36(2):379-390]

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Typical and Atypical Hemolytic Uremic Syndrome in the Critically Ill.

Carlos L Manrique-Caballero, Sadudee Peerapornratana, Cassandra Formeck, Gaspar Del Rio-Pertuz, Hernando Gomez Danies, John A Kellum,

Hemolytic uremic syndrome is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. Disseminated intravascular coagulation, thrombotic thrombocytopenic purpura, and hemolytic uremic syndrome have a similar clinical presentation. Diagnostic needs to be prompt to decrease mortality, because identifying the different disorders can help to tailor specific, effective therapies. However, ... Read more >>

Crit Care Clin (Critical care clinics)
[2020, 36(2):333-356]

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Genetic Testing in Pediatric Kidney Disease.

Veronica Arora, Kanav Anand, Ishwar Chander Verma,

The advent of next gene sequencing technology has led to the publication of a profusion of papers on monogenic contributions to pediatric kidney disorders. It started with the discovery of mutations in the podocin gene in steroid resistant nephrotic syndrome (SRNS). It is realized now that genetic disorders contribute to ... Read more >>

Indian J Pediatr (Indian journal of pediatrics)
[2020, :]

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Rasburicase in hemolytic uremic syndrome related to Shiga toxin-producing Escherichia coli: a report of nine cases.

Alejandro Balestracci, Luciana Meni Battaglia, Sandra Mariel Martin, Ismael Toledo,

BACKGROUND:Hyperuricemia might induce additional renal damage in children with hemolytic uremic syndrome related to Shiga toxin-producing Escherichia coli (STEC-HUS). A few case reports have shown rasburicase to be effective in decreasing serum uric acid (UA) and improving renal function. However, there is only one report on the use of rasburicase ... Read more >>

Pediatr. Nephrol. (Pediatric nephrology (Berlin, Germany))
[2020, :]

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Prognostic value of urinary TGF-β1 in hemolytic uremic syndrome: A pilot study.

Alejandro Balestracci, Adriana Haydeé Roy, María Gracia Caletti,

BACKGROUND:Transforming growth factor β1 (TGF-β1) is the main profibrotic cytokine. Its urinary excretion reflects intrarenal production; thus, we conjectured that it is elevated during hemolytic uremic syndrome related to Shiga-toxin-producing Escherichia coli (STEC-HUS). In this pilot study, we explored the ability of baseline TGF-β1 excretion (exposure variable) to predict renal ... Read more >>

Pediatr Int (Pediatrics international : official journal of the Japan Pediatric Society)
[2020, 62(3):371-378]

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CFHR Gene Variations Provide Insights in the Pathogenesis of the Kidney Diseases Atypical Hemolytic Uremic Syndrome and C3 Glomerulopathy.

Peter F Zipfel, Thorsten Wiech, Emma D Stea, Christine Skerka,

Sequence and copy number variations in the human CFHR-Factor H gene cluster comprising the complement genes CFHR1, CFHR2, CFHR3, CFHR4, CFHR5, and Factor H are linked to the human kidney diseases atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathy. Distinct genetic and chromosomal alterations, deletions, or duplications generate hybrid or ... Read more >>

J. Am. Soc. Nephrol. (Journal of the American Society of Nephrology : JASN)
[2020, 31(2):241-256]

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A97 TACROLIMUS-ASSOCIATED HEMOLYTIC UREMIC SYNDROME IN A PEDIATRIC LIVER TRANSPLANT RECIPIENT.

K W Wong, S Gilmour,

AbstractBackgroundTacrolimus associated hemolytic uremic syndrome (HUS) has been reported in renal and pediatric cardiac transplantation. Furthermore, in adults receiving liver transplant, there are reports of multiorgan failure, death, and end stage renal failure secondary to tacrolimus induced HUS [J NEPHROL 2003; 16: 580–585]. Although tacrolimus is commonly used in pediatric ... Read more >>

J Can Assoc Gastroenterol (Journal of the Canadian Association of Gastroenterology)
[2020, 3(Suppl 1):113-114]

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European Society for Immunodeficiencies (ESID) and European Reference Network on Rare Primary Immunodeficiency, Autoinflammatory and Autoimmune Diseases (ERN RITA) Complement Guideline: Deficiencies, Diagnosis, and Management.

Nicholas Brodszki, Ashley Frazer-Abel, Anete S Grumach, Michael Kirschfink, Jiri Litzman, Elena Perez, Mikko R J Seppänen, Kathleen E Sullivan, Stephen Jolles,

This guideline aims to describe the complement system and the functions of the constituent pathways, with particular focus on primary immunodeficiencies (PIDs) and their diagnosis and management. The complement system is a crucial part of the innate immune system, with multiple membrane-bound and soluble components. There are three distinct enzymatic cascade ... Read more >>

J. Clin. Immunol. (Journal of clinical immunology)
[2020, :]

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Atypical hemolytic uremic syndrome: a case report.

B M D B Basnayake, A W M Wazil, N Nanayakkara, S M D K Samarakoon, E M S K Senavirathne, B U E W D R Thangarajah, N Karunasena, R M B S S Mahanama,

BACKGROUND:Thrombotic microangiopathy is a pathological condition comprised of microvascular thrombosis involving any organ of the body leading to thrombocytopenia, Coombs-negative hemolytic anemia, and end-organ damage. The most common forms of thrombotic microangiopathies are Shiga toxin-producing Escherichia coli-mediated hemolytic uremic syndrome, thrombotic thrombocytopenic purpura, and atypical hemolytic uremic syndrome. The atypical ... Read more >>

J Med Case Rep (Journal of medical case reports)
[2020, 14(1):11]

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