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https://www.readbyqxmd.com/read/29907460/clinical-and-genetic-predictors-of-atypical-hemolytic-uremic-syndrome-phenotype-and-outcome
#1
Franz Schaefer, Gianluigi Ardissino, Gema Ariceta, Fadi Fakhouri, Marie Scully, Nicole Isbel, Åsa Lommelé, Varant Kupelian, Christoph Gasteyger, Larry A Greenbaum, Sally Johnson, Masayo Ogawa, Christoph Licht, Johan Vande Walle, Véronique Frémeaux-Bacchi
Atypical hemolytic uremic syndrome (aHUS) is a rare, genetic, life-threatening disease. The Global aHUS Registry collects real-world data on the natural history of the disease. Here we characterize end-stage renal disease (ESRD)-free survival, the rate of thrombotic microangiopathy, organ involvement and the genetic background of 851 patients in the registry, prior to eculizumab treatment. A sex-specific difference was apparent according to age at initial disease onset as the ratio of males to females was 1...
June 12, 2018: Kidney International
https://www.readbyqxmd.com/read/29902403/fulminant-meningococcal-sepsis-due-to-non-groupable-em-neisseria-meningitidis-em-in-a-patient-receiving-eculizumab
#2
Victoria Hall, Rekha Pai Mangalore, Simon He, Kerrie Stevens, Jason A Trubiano, Natasha E Holmes, Benjamin P Howden, Jason C Kwong
No abstract text is available yet for this article.
June 18, 2018: Medical Journal of Australia
https://www.readbyqxmd.com/read/29898960/antibody-inhibition-of-properdin-prevents-complement-mediated-intravascular-and-extravascular-hemolysis
#3
Damodar Gullipalli, Fengkui Zhang, Sayaka Sato, Yoshiyasu Ueda, Yuko Kimura, Madhu Golla, Takashi Miwa, Jianxiang Wang, Wen-Chao Song
Paroxysmal nocturnal hemoglobinuria (PNH) is a serious blood disorder characterized by dysregulated complement activation on blood cells. Eculizumab, the current standard therapy and a humanized anti-C5 mAb, relieves anemia and thrombosis symptoms of PNH patients by preventing complement-dependent intravascular hemolysis (IVH). However, up to 20% of PNH patients on long-term eculizumab treatment still suffer from significant anemia and are transfusion dependent because of extravascular hemolysis (EVH) of C3-opsonized PNH erythrocytes...
June 13, 2018: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/29897310/an-unusual-pattern-of-peritubular-capillary-injury-involving-apoptosis-in-a-renal-transplant-patient
#4
John K Brealey, John Cassidy, Jim Manavis
Microvascular injury is an important factor in renal allograft survival. Repeated episodes of endothelial injury from chronic antibody-mediated rejection typically manifest at the ultrastructural level as circumferential multilayering of remodeled glomerular basement membrane material and peritubular capillary basal lamina. In contrast to this typical pattern of microvascular injury, a renal transplantation case is presented in which focally dilated and multilayered segments of peritubular capillary basal lamina bearing lipid droplets were interspersed with ultrastructurally normal unilayered segments of basal lamina devoid of lipid droplets...
June 13, 2018: Ultrastructural Pathology
https://www.readbyqxmd.com/read/29895518/disseminated-cryptococcosis-associated-with-administration-of-eculizumab
#5
Megan Clancy, Ryan McGhan, Jeremy Gitomer, Ana Marie Inocencio, Christina Aldrich, Roberto Iaderosa, Ryan Stevens
PURPOSE: A case report of fatal disseminated cryptococcosis in a patient treated with eculizumab is presented along with a review of literature suggesting a possible etiologic mechanism. SUMMARY: A 23-year-old man with a history of minimal change nephrotic syndrome was hospitalized for acute kidney injury and abdominal pain and swelling. He was found to have disseminated pneumococcal disease, including peritonitis, bacteremia, and pulmonic endocarditis. The patient developed evidence of microangiopathic hemolytic anemia, leading to a diagnosis of atypical hemolytic uremic syndrome, and was started on eculizumab...
June 12, 2018: American Journal of Health-system Pharmacy: AJHP
https://www.readbyqxmd.com/read/29883245/fatal-meningococcemia-due-to-neisseria-meningitidis-serogroup-y-in-a-vaccinated-child-receiving-eculizumab
#6
Meltem Polat, Selçuk Yüksel, Nuriye Ünal Şahin
Life-threatening and fatal meningococcal infections have occurred in patients treated with eculizumab. Herein we describe an 11-year-old boy with atypical hemolytic uremic syndrome treated with eculizumab who developed fatal meningococcemia due to Neisseria meningitidis serogroup Y 16 months after receiving two doses of meningococcal conjugate vaccine (MenACWY-D) while on oral penicillin prophylaxis. Clinicians need to maintain a high index of suspicion for invasive meningococcal disease in patients taking eculizumab treatment, regardless of meningococcal vaccination or antimicrobial prophylaxis status...
June 8, 2018: Human Vaccines & Immunotherapeutics
https://www.readbyqxmd.com/read/29874282/a-computational-model-for-the-evaluation-of-complement-system-regulation-under-homeostasis-disease-and-drug-intervention
#7
Nehemiah Zewde, Dimitrios Morikis
The complement system is an intricate defense network that rapidly removes invading pathogens. Although many complement regulators are present to protect host cells under homeostasis, the impairment of Factor H (FH) regulatory mechanism has been associated with several autoimmune and inflammatory diseases. To understand the dynamics involved in the pivotal balance between activation and regulation, we have developed a comprehensive computational model of the alternative and classical pathways of the complement system...
2018: PloS One
https://www.readbyqxmd.com/read/29870459/novel-approaches-to-block-complement
#8
Georg A Böhmig, Markus Wahrmann, Farsad Eskandary, Lionel Rostaing
The complement system may contribute in many ways to transplant injury, being a promising target for specific therapeutic interventions. There is evidence that the monoclonal anti-C5 antibody eculizumab is effective in the prevention and treatment of early antibody-mediated rejection (ABMR), but terminal complement blockade might be of limited efficiency in chronic rejection. Given the diversity of immunological events triggered by activation steps upstream to C5, in particular, opsonin and anaphylatoxin formation through C3 cleavage, one may argue that, in the specific context of ABMR, inhibition of antibody-triggered classical pathway (CP) activation might be beneficial...
June 4, 2018: Transplantation
https://www.readbyqxmd.com/read/29862099/atypical-haemolytic-uraemic-syndrome-associated-with-clostridium-difficile-infection-successfully-treated-with-eculizumab
#9
Joshua M Inglis, Jeffrey A Barbara, Rajiv Juneja, Caroline Milton, George Passaris, Jordan Y Z Li
Clostridium difficile infection is a rare precipitant of atypical haemolytic uraemic syndrome (aHUS). A 46-year-old man presented with watery diarrhoea following an ileocaecal resection. He developed an acute kidney injury with anaemia, thrombocytopaenia, raised lactate dehydrogenase, low haptoglobin, and red cell fragments. Stool sample was positive for C. difficile toxin B. He became dialysis-dependent as his renal function continued to worsen despite treatment with empiric antibiotics and plasma exchange...
2018: Case Reports in Nephrology
https://www.readbyqxmd.com/read/29858281/c5b9-formation-on-endothelial-cells-reflects-complement-defects-among-patients-with-renal-thrombotic-microangiopathy-and-severe-hypertension
#10
Sjoerd A M E G Timmermans, Myrurgia A Abdul-Hamid, Judith Potjewijd, Ruud O M F I H Theunissen, Jan G M C Damoiseaux, Chris P Reutelingsperger, Pieter van Paassen
Background Severe hypertension can induce thrombotic microangiopathy (TMA) in the renal vasculature, the occurrence of which has been linked to mechanical stress to the endothelium. Complement defects may be the culprit of disease in patients who present with severe renal disease and often progress to ESRD, despite BP control. Methods We studied a well defined cohort of 17 patients with hypertension-associated TMA to define the prevalence of complement defects by a specific ex vivo serum-based microvascular endothelial cell assay...
June 1, 2018: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/29850957/thrombosis-and-anti-phospholipid-syndrome-a-5-year-update-on-treatment
#11
REVIEW
Cecilia Beatrice Chighizola, Pier Luigi Meroni
PURPOSE OF REVIEW: The aim of this review is to provide an update of the therapeutic tools for thrombotic anti-phospholipid syndrome (APS), focusing on the last 5 years. RECENT FINDINGS: Early studies appointed anticoagulation at moderate intensity as the mainstay of treatment of thrombotic APS; in the last 5 years, the strategy has not much mutated. Some uncertainties regarding the role of direct oral anticoagulants and the optimal regimen for arterial thrombotic APS still persist: high-intensity anticoagulation, anticoagulation plus anti-platelet agent, and double anti-platelet agents being the possible alternatives...
May 31, 2018: Current Rheumatology Reports
https://www.readbyqxmd.com/read/29843966/cd59-deficiency-presenting-as-polyneuropathy-and-moyamoya-syndrome-with-endothelial-abnormalities-of-small-brain-vessels
#12
Christian Klemann, Janbernd Kirschner, Sandra Ammann, Horst Urbach, Olaf Moske-Eick, Barbara Zieger, Myriam Ricarda Lorenz, Klaus Schwarz, Soroush Doostkam, Stephan Ehl, Rudolf Korinthenberg
CD59 is involved in lymphocyte signal transduction and regulates complement-mediated cell lysis by inhibiting the membrane attack complex. In the cases reported so far, congenital isolated CD59 deficiency was associated with recurrent episodes of hemolytic anemia, peripheral neuropathy, and strokes. Here, we report on a patient from a consanguineous Turkish family, who had a first episode of hemolytic anemia at one month of age and presented at 14 months with acute Guillain-Barré syndrome (GBS). The child suffered repeated infection-triggered relapses leading to the diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP)...
April 13, 2018: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/29807376/-thrombotic-microangiopathies
#13
Jörg Schubert, Michael Dechant
Thrombotic microangiopathies are almost devastating diseases leading to death at high frequency if untreated. They consist of at least five distinct entities, TTP, HUS, aHUS, TMA due to drug interference, systemic disease or post therapy TMA. Around 10 years ago there was only one established therapeutic approach as plasmapheresis. Meanwhile, there are new drugs been licensed or within licensing process. Patients with atypical HUS can be treated successfully by the Complement inhibitor Eculizumab. In addition, there is a new inhibitor of von-Willebrand-Polymerisation available...
June 2018: Deutsche Medizinische Wochenschrift
https://www.readbyqxmd.com/read/29803280/atypical-hemolytic-uremic-syndrome
#14
REVIEW
Bradley P Dixon, Ralph A Gruppo
Atypical hemolytic uremic syndrome is a rare life-threatening disease of unregulated complement activation. Untreated, the prognosis is generally poor; more than one-half of patients die or develop end-stage renal disease within 1 year. Atypical hemolytic uremic syndrome is characterized by thrombotic microangiopathy with evidence of hemolysis, thrombocytopenia, and renal impairment. This systemic disease affects the kidneys, brain, heart, lungs, gastrointestinal tract, pancreas, and skin. Acquired and genetic abnormalities of complement regulation may be identified in approximately 70% of patients...
June 2018: Pediatric Clinics of North America
https://www.readbyqxmd.com/read/29794727/therapeutic-effects-of-various-therapeutic-strategies-on-non-exudative-age-related-macular-degeneration-a-prisma-compliant-network-meta-analysis-of-randomized-controlled-trials
#15
Yanli Wei, Hongxia Liao, Jian Ye
PURPOSE: Age-related macular degeneration (AMD) is a chronic progressive central retinal disease. Geographic atrophy (GA) is a late stage of dry AMD (DAMD) and is a slowly but inexorably progressive disease that causes irreversible blindness over time. We aimed to assess various therapeutic strategies for DAMD and GA treatment by network meta-analysis. METHODS: We searched PubMed, Embase, and the Cochrane Library to identify randomized controlled trials (RCTs) of atrophic AMD treatments published prior to December 16, 2017...
May 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29779928/the-diagnosis-and-clinical-management-of-the-catastrophic-antiphospholipid-syndrome-a-comprehensive-review
#16
REVIEW
Ricard Cervera, Ignasi Rodríguez-Pintó, Gerard Espinosa
The catastrophic antiphospholipid syndrome (CAPS) is a life-threating variant of the antiphospholipid syndrome characterized by the development of multiple thrombosis in a short period of time, usually ending up in the failure of function of several vital organs. Most CAPS episodes are related to a prothrombotic situation or precipitating factor such as infections, surgical procedures or malignant diseases. In patients with CAPS, the development of multiple thrombosis leads to an important cytokine release that worsens the already critical patient's situation...
May 17, 2018: Journal of Autoimmunity
https://www.readbyqxmd.com/read/29778558/successful-kidney-transplant-with-eculizumab-thymoglobulin-and-belatacept-therapy-in-a-highly-sensitised-patient-with-atypical-haemolytic-uraemic-syndrome-due-to-factor-h-mutation
#17
John Fredy Nieto-Ríos, Mónica Zuluaga-Quintero, Diana Carolina Bello-Márquez, Arbey Aristizabal-Alzate, Catalina Ocampo-Kohn, Lina María Serna-Higuita, Lina Arias, Gustavo Zuluaga-Valencia
Atypical haemolytic uremic syndrome is a disease caused by complement regulation abnormalities that generally progresses to chronic end-stage renal disease with a high rate of recurrence in kidney transplantation and a high risk of graft loss. Anti-complement therapy has improved the prognosis of these patients, achieving disease remission in most cases, increasing the likelihood of a successful kidney transplant and increasing patient and graft survival. Drugs with low risk of induction of thrombotic microangiopathies such as belatacept and mycophenolate have also been used with satisfactory results...
May 16, 2018: Nefrología: Publicación Oficial de la Sociedad Española Nefrologia
https://www.readbyqxmd.com/read/29774464/eculizumab-in-stec-hus-need-for-a-proper-randomized-controlled-trial
#18
Sebastian Loos, Jun Oh, Markus J Kemper
Hemolytic uremic syndrome caused by Shiga toxin-producing E. coli (STEC-HUS) is often associated with a severe morbidity including neurological involvement and a mortality of 1-5%. Although STEC-HUS is often self-limited, improvement of treatment strategies is needed for cases with complications and, among others, plasma exchange/plasmapheresis and use of antibiotics have been advocated. With the availability of the complement blocker eculizumab, now a standard treatment of atypical HUS, several series have addressed its use in STEC-HUS, with variable response; randomized controlled trials are lacking...
May 17, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29768958/bevacizumab-induced-atypical-hemolytic-uremic-syndrome-and-treatment-with-eculizumab
#19
Anusha Vakiti, Daulath Singh, Ravi Pilla, Muhamad Alhaj-Moustafa, Kelly W Fitzpatrick
Bevacizumab (Avastin) is a recombinant humanized monoclonal antibody used for the management of various solid malignancies including colorectal, lung, brain, renal, and ovarian cancers as well as age-related macular degeneration of the eye. It is a vascular endothelial growth factor inhibitor which exhibits its action by blocking the growth of blood vessels in cancerous tissue. Common side effects include hypertension, fatigue, headaches, and increased risk of infections. Atypical hemolytic uremic syndrome is a serious side effect associated with bevacizumab due to its anti-angiogenic effect...
January 1, 2018: Journal of Oncology Pharmacy Practice
https://www.readbyqxmd.com/read/29750742/an-atypical-case-of-atypical-hemolytic-uremic-syndrome
#20
Tine Francois, Johan Vande Walle, Evelyn Dhont, Werner Keenswijk
We present the case of a 2-month-old infant presenting with pallor and laboratory results showing: hemoglobin 5.1 (10 to 1.5) g/dL, MCV 94.7 (75 to 105) fL, leukocytes 17.4 (7 to 15) ×10/μL, platelets 259 (150 to 450) ×10/μL, hyperbilirubinemia and renal dysfunction. A hemolytic anemia with tubular injury secondary to hemoglobinuria was suspected. Hyperhydration and packed cells were given but she deteriorated. Fluid overload with anuria further complicated the course necessating hemodialysis. Atypical hemolytic uremic syndrome was suspected and eculizumab was administered resulting in rapid improvement...
May 10, 2018: Journal of Pediatric Hematology/oncology
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