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https://www.readbyqxmd.com/read/28646526/adamts13-test-and-or-plasmic-clinical-score-in-management-of-acquired-thrombotic-thrombocytopenic-purpura-a-cost-effective-analysis
#1
Chong H Kim, Sierra C Simmons, Lance A Williams Iii, Elizabeth M Staley, X Long Zheng, Huy P Pham
BACKGROUND: The ADAMTS13 test distinguishes thrombotic thrombocytopenic purpura (TTP) from other thrombotic microangiopathies (TMAs). The PLASMIC score helps determine the pretest probability of ADAMTS13 deficiency. Due to inherent limitations of both tests, and potential adverse effects and cost of unnecessary treatments, we performed a cost-effectiveness analysis (CEA) investigating the benefits of incorporating an in-hospital ADAMTS13 test and/or PLASMIC score into our clinical practice...
June 23, 2017: Transfusion
https://www.readbyqxmd.com/read/28646244/increased-soluble-vcam-1-and-normal-p-selectin-in-cystic-fibrosis-a-cross-sectional-study
#2
Jan K Nowak, Irena Wojsyk-Banaszak, Edyta Mądry, Andrzej Wykrętowicz, Patrycja Krzyżanowska, Sławomira Drzymała-Czyż, Agata Nowicka, Andrzej Pogorzelski, Ewa Sapiejka, Wojciech Skorupa, Mariusz Szczepanik, Aleksandra Lisowska, Jaroslaw Walkowiak
PURPOSE: As life expectancy in cystic fibrosis (CF) increases, questions regarding its potential impact on cardiovascular health arise. Soluble vascular cell adhesion molecule 1 (sVCAM-1), P-selectin (sP-selectin) are proposed as biomarkers of cardiovascular disease. We aimed to: compare their concentrations in clinically stable CF patients and healthy subjects (HS) and verify whether they independently correlate with CF characteristics. METHODS: Serum sVCAM-1 and sP-selectin levels were measured using ELISA...
June 23, 2017: Lung
https://www.readbyqxmd.com/read/28645643/management-of-thrombotic-thrombocytopenic-purpura
#3
P Coppo
Daily therapeutic plasma exchange (TPE) transformed the historically fatal prognosis of acquired, anti-ADAMTS13 antibody-mediated thrombotic thrombocytopenic purpura (TTP), leading to the current overall survival rates of >80%. However, relapses occur in up to 40% of patients and refractory disease with fatal outcomes still occurs, typically within the first days of management. In this context, the introduction of rituximab has been the second major breakthrough in TTP management. Rituximab is now routinely recommended during the acute phase, typically in patients with a suboptimal response to treatment, and increasingly as frontline therapy, with high response rates in the following weeks...
June 20, 2017: Transfusion Clinique et Biologique: Journal de la Société Française de Transfusion Sanguine
https://www.readbyqxmd.com/read/28638625/early-eculizumab-use-in-atypical-haemolytic-uraemic-syndrome-in-a-jehovah-s-witness-refusing-blood-products
#4
May Al-Shaghana, Andrew Bentall, Mark D Jesky, William Lester, Graham Lipkin
Thrombotic microangiopathy (TMA) is characterized by microscopic angiopathic haemolytic anaemia, thrombocytopenia and organ injury. Supportive therapies include the use of blood products. Recently the terminal complement inhibitor eculizumab has been approved in atypical haemolytic uraemic syndrome (aHUS) in some countries. We report the case of a 23-year-old female Jehovah's Witness presenting with vaginal haemorrhage from thrombocytopaenia, severe anaemia (nadir Hb 28 g/L) and anuric acute kidney injury with TMA secondary to aHUS...
June 2017: Oxford Medical Case Reports
https://www.readbyqxmd.com/read/28637667/thrombocytopenia-in-pregnancy
#5
Douglas B Cines, Lisa D Levine
Thrombocytopenia develops in 5-10% of women during pregnancy or in the immediate post-partum period. A low platelet count is often an incidental feature, but it can also provide a biomarker of a coexisting systemic or gestational disorder and a potential reason for a maternal intervention or treatment that might pose harm to the fetus. This chapter reflects our approach to these issues with an emphasis on advances in understanding and managing the more common causes of thrombocytopenia in pregnancy made over the past 5-10 years...
June 21, 2017: Blood
https://www.readbyqxmd.com/read/28626544/atypical-hemolytic-uremic-syndrome-a-brief-review
#6
REVIEW
Kuixing Zhang, Yuxin Lu, Kevin T Harley, Minh-Ha Tran
Atypical hemolytic uremic syndrome (aHUS) is a disease characterized by the triad of microangiopathic hemolytic anemia, thrombocytopenia and acute kidney injury. The histopathologic lesions of aHUS include thrombotic microangiopathy involving the glomerular capillaries and thrombosis involving arterioles or interlobar arteries. Extra-renal manifestations occur in up to 20% of patients. The majority of aHUS is caused by complement system defects impairing ordinary regulatory mechanisms. Activating events therefore lead to unbridled, ongoing complement activity producing widespread endothelial injury...
June 1, 2017: Hematology Reports
https://www.readbyqxmd.com/read/28621343/current-evidence-on-the-discontinuation-of-eculizumab-in-patients-with-atypical-haemolytic-uraemic-syndrome
#7
COMMENT
Manuel Macia, Fernando de Alvaro Moreno, Tina Dutt, Ingela Fehrman, Karine Hadaya, Christoph Gasteyger, Nils Heyne
Background. Atypical haemolytic uraemic syndrome (aHUS) is a rare, life-threatening disorder for which eculizumab is the only approved treatment. Life-long treatment is indicated; however, eculizumab discontinuation has been reported. Methods. Unpublished authors' cases and published cases of eculizumab discontinuation are reviewed. We also report eculizumab discontinuation data from five clinical trials, plus long-term extensions and the global aHUS Registry. Results. Of six unpublished authors' cases, four patients had a subsequent thrombotic microangiopathy (TMA) manifestation within 12 months of discontinuation...
June 2017: Clinical Kidney Journal
https://www.readbyqxmd.com/read/28614243/different-types-of-glomerulonephritis-associated-with-the-dysregulation-of-the-complement-alternative-pathway-in-2-brothers-a-case-report
#8
Pei Chen, Li Zhu, Feng Yu, Sha-Sha Han, Si-Jun Meng, Wei-Yi Guo, Hong Zhang, Yan Song
RATIONALE: C3 glomerulonephritis (C3GN) and complement-mediated hemolytic uremic syndrome (HUS) both result from the abnormal regulation of the complement system. A significant number of patients with C3GN or complement-mediated HUS have mutations of more than 1 complement protein. This discovery has had a major impact on identifying the underlying cause of familial C3GN or complement-mediated HUS. PATIENT CONCERNS: We report the cases of 2 brothers (herein referred to as patient II-1 and patient II-9), both with complement disorders that differed in their clinical and genetic features...
June 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28612003/discontinuation-of-peritoneal-dialysis-after-late-initiation-of-eculizumab-in-a-case-of-familial-atypical-hemolytic-uremic-syndrome-a-case-report
#9
Rafael Alonso Valente, Giannina Elena García Rodríguez, Yanina García Marcote, Manuel Fidalgo Díaz, Vanesa Becerra Mosquera, Daniel Novoa García, Teresa Cordal Martínez, Cándido Díaz Rodríguez
BACKGROUND: Atypical hemolytic-uremic syndrome is caused by a thrombotic microangiopathy and manifests itself with hemolytic anemia, thrombocytopenia, and organ ischemia. Its etiology is a mutation affecting the genes encoding for proteins of the complement system. Early treatment with eculizumab (8.6 months from the moment of presentation), a humanized monoclonal antibody against complement, is shown to be effective in controlling symptoms and reversing organ damage. We present a patient with a mutation not previously described in the literature...
January 2017: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/28611987/complement-in-kidney-transplantation
#10
REVIEW
Marek Cernoch, Ondrej Viklicky
The complement system is considered to be an important part of innate immune system with a significant role in inflammation processes. The activation can occur through classical, alternative, or lectin pathway, resulting in the creation of anaphylatoxins C3a and C5a, possessing a vast spectrum of immune functions, and the assembly of terminal complement cascade, capable of direct cell lysis. The activation processes are tightly regulated; inappropriate activation of the complement cascade plays a significant role in many renal diseases including organ transplantation...
2017: Frontiers in Medicine
https://www.readbyqxmd.com/read/28610947/impaired-balance-is-related-to-the-progression-of-diabetic-complications-in-both-young-and-older-adults
#11
Daisuke Kukidome, Takeshi Nishikawa, Miki Sato, Yoshiko Nishi, Risa Shimamura, Junji Kawashima, Seiya Shimoda, Hiroshi Mizuta, Eiichi Araki
AIMS: To investigate the balance ability in younger and older adults with diabetes and evaluate the associations between balance ability and microvascular complications. METHODS: This cross-sectional observational study compared 162 participants and 177 controls with and without type 2 diabetes, respectively. Balance ability was assessed using two static (one-legged stance and postural sway area) and two dynamic (Timed Up and Go [TUG] and Functional Reach) tests...
June 4, 2017: Journal of Diabetes and its Complications
https://www.readbyqxmd.com/read/28609342/cutaneous-collagenous-vasculopathy-report-of-two-cases-presenting-as-disseminated-telangiectasias-and-review-of-the-literature
#12
Laure Bondier, Mathilde Tardieu, Perrine Leveque, Isabelle Challende, Nicole Pinel, Marie T Leccia
Cutaneous collagenous vasculopathy is a recently described idiopathic microangiopathy characterized by acquired diffuse cutaneous telangiectasias and specific histological features: dilated capillaries in the superficial dermis, with walls thickened by hyaline material containing collagen IV by immunohistochemistry. The authors describe 2 cases and review all cases reported in the literature to date, 34 cases including our own. Cases were mainly observed in women (sex ratio 0.41), median age 63.5 (16-85). Hypertension and diabetes seem more frequent in these patients than in the general population...
June 1, 2017: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/28607917/thrombotic-microangiopathy-associated-with-synthetic-cannabinoid-receptor-agonists
#13
Michael Karass, Savneek Chugh, Gabriela Andries, Aleksandra Mamorska-Dyga, John C Nelson, Praveen N Chander
Marijuana is one of the most commonly used recreational drugs in the United States. As marijuana is illegal in the majority of countries, the use of readily available and unregulated synthetic cannabinoids (SCBs) has increased. Little is known about the potential adverse effects of SCBs especially in regards to their nephrotoxicity. Case reports of acute kidney injury (AKI) from acute tubular injury secondary to their use have been reported. However, the exact pathology, mechanism, and extent of renal injury remain unknown...
2017: Stem Cell Investigation
https://www.readbyqxmd.com/read/28607779/thrombotic-microangiopathy-secondary-to-intravenous-abuse-of-opana%C3%A2-er
#14
Kamia Thakur, Vaibhav Agrawal, Ashley Kass, Lauren M Dimarino, R Patrick Dorion, Joseph Vadakara
Opana ER (oxymorphone) is an opioid drug available throughout the United States, and intravenous abuse of the crushed oral formulation has been associated with drug-induced thrombotic microangiopathy. In this abstract, we describe two young patients who lived together and used Opana ER intravenously. Both presented with microangiopathic hemolytic anemia that mimicked thrombotic thrombocytopenic purpura (TTP). Treating this condition poses a clinical challenge, as it is difficult to distinguish it from TTP. The role for plasma exchange is not clear but can be used while awaiting the results of the ADAMTS-13 activity, but ultimately supportive care with drug discontinuation is the recommended therapy of choice...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28598975/vitamin-d-deficiency-and-clinical-correlations-in-systemic-sclerosis-patients-a-retrospective-analysis-for-possible-future-developments
#15
Amelia Chiara Trombetta, Vanessa Smith, Emanuele Gotelli, Massimo Ghio, Sabrina Paolino, Carmen Pizzorni, Amber Vanhaecke, Barbara Ruaro, Alberto Sulli, Maurizio Cutolo
OBJECTIVE: Assessment of serum 25-hydroxyvitamin D (25(OH)D) correlations with clinical parameters and evaluation of the efficacy of standard oral supplementation in systemic sclerosis (SSc) patients. METHODS: 154 SSc patients were recruited, in all seasons. Serum 25(OH)D concentrations were evaluated using LIAISON 25-OH (Diasorin, Italy). Medsger disease severity scale (DSS), nailfold videocapillaroscopy (NVC) and all instrumental exam contemplated by international guidelines were performed...
2017: PloS One
https://www.readbyqxmd.com/read/28597458/clinical-scoring-systems-in-thrombotic-microangiopathies
#16
Pavan K Bendapudi, Vivek Upadhyay, Lova Sun, Marisa B Marques, Robert S Makar
No abstract text is available yet for this article.
July 2017: Seminars in Thrombosis and Hemostasis
https://www.readbyqxmd.com/read/28596415/hemolytic-uremic-syndrome-in-pregnancy-and-postpartum
#17
Alexandra Bruel, David Kavanagh, Marina Noris, Yahsou Delmas, Edwin K S Wong, Elena Bresin, François Provôt, Vicky Brocklebank, Caterina Mele, Giuseppe Remuzzi, Chantal Loirat, Véronique Frémeaux-Bacchi, Fadi Fakhouri
BACKGROUND: Pregnancy is associated with various forms of thrombotic microangiopathy, including hemolytic uremic syndrome. A previous small French study suggested that pregnancy-associated hemolytic uremic syndrome was to be included in the spectrum of atypical hemolytic uremic syndrome linked to complement alternative pathway dysregulation. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: We sought to retrospectively analyze the presentation, outcome, and frequency of complement alternative pathway gene variants in a larger international (France, United Kingdom, Italy) cohort of patients with pregnancy-associated hemolytic uremic syndrome...
June 8, 2017: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/28596255/thrombotic-thrombocytopenic-purpura-associated-with-pegylated-interferon-alfa-2a-use-in-a-patient-with-polycythemia-vera
#18
Radhika Gangaraju, Soo J Kim, Jing-Fei Dong, Sabina Swierczek, Josef T Prchal
Pegylated interferon alfa-2a (pegIFNa) is being increasingly used for treatment of myeloproliferative neoplasms; however, its side effects, including autoimmune complications, are not unusual. We report on a 47-year-old woman with polycythemia vera (PV) treated with pegIFNa and in complete hematologic remission who developed thrombotic thrombocytopenic purpura (TTP). To our knowledge, thrombotic microangiopathy has been reported as a side effect of interferon (IFN) use in patients with hepatitis and chronic myeloid leukemia, but not in those with PV...
June 2017: Journal of the National Comprehensive Cancer Network: JNCCN
https://www.readbyqxmd.com/read/28596204/malignant-hypertension-as-a-rare-cause-of-thrombotic-microangiopathy
#19
Guramrinder Thind, Karthik Kailasam
Malignant hypertension can occasionally be associated with microangiopathic haemolytic anaemia. A 38-year-old male presented with nausea, vomiting, loss of appetite and oliguria for 2 weeks. He was diagnosed with hypertensive emergency with cardiac and renal dysfunction. Interestingly, further workup was diagnostic for the presence of thrombotic microangiopathy (TMA): haemoglobin =12.7 g/dL, indirect bilirubin =2.0 mg/dL, haptoglobin ≤6 mg/dL, platelet count =121 000/μL and schistocytes on peripheral smear...
June 8, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28595537/capillaroscopic-findings-in-primary-fibromyalgia
#20
Sevdalina Lambova, Ulf Müller-Ladner
: Introduction: Although Raynaud's phenomenon (RP) is observed in a significant proportion of patients with primary fibromyalgia, the available data on capillaroscopic findings in primary fibromyalgia are scarce. OBJECTIVE: The aim of the study was to evaluate the capillaroscopic pattern in patients with primary fibromyalgia. PATIENTS AND METHODS: 26 patients with primary fibromyalgia (25 women and 1 man) were included in the study. Mean age was 55±10 years...
June 7, 2017: Current Rheumatology Reviews
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