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https://www.readbyqxmd.com/read/29214126/pharmacologic-complement-inhibition-in-clinical-transplantation
#1
REVIEW
Vasishta S Tatapudi, Robert A Montgomery
Purpose of Review: Over the past two decades, significant strides made in our understanding of the etiology of antibody-mediated rejection (AMR) in transplantation have put the complement system in the spotlight. Here, we review recent progress made in the field of pharmacologic complement inhibition in clinical transplantation and aim to understand the impact of this therapeutic approach on outcomes in transplant recipients. Recent Findings: Encouraged by the success of agents targeting the complement cascade in disorders of unrestrained complement activation like paroxysmal nocturnal hemoglobinuria (PNH) and atypical hemolytic uremic syndrome (aHUS), investigators are testing the safety and efficacy of pharmacologic complement blockade in mitigating allograft injury in conditions ranging from AMR to recurrent post-transplant aHUS, C3 glomerulopathies and antiphospholipid anti-body syndrome (APS)...
2017: Current Transplantation Reports
https://www.readbyqxmd.com/read/29212979/-clinical-features-and-quality-of-life-assessment-in-japanese-pnh-patients-enrolled-in-the-international-pnh-registry
#2
Masatoshi Sakurai, Shinichiro Okamoto
The aim of the present study was to clarify the clinical characteristics and quality of life (QOL) of Japanese patients (N=116) enrolled in the International PNH Registry, compared to the whole registry cohort (N=3,457), censored as of March 2015. The proportion of patients treated with eculizumab was comparable between the two cohorts; the Japanese cohort showed higher levels of lactate dehydrogenase and lower hemoglobin values at baseline. Compared to the whole cohort, the Japanese cohort had a greater incidence of bone marrow failure (67...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/29190926/polymorphism-of-the-abo-gene-associate-with-thrombosis-risk-in-patients-with-paroxysmal-nocturnal-hemoglobinuria
#3
Zhangbiao Long, Yali Du, Hongmin Li, Bing Han
Thrombosis is one of the most common causes of mortality in Paroxysmal nocturnal hemoglobinuria (PNH), but the predisposing factors for thrombosis are yet to be defined. In this study, we outline the clinical characters and the susceptible genes which lead to thrombotic formation in 104 patients with PNH. The results displayed that the genotypes with minor alleles of rs495828 or rs2519093 in the ABO gene were associated with high risk to thrombus formation (OR 5.95, 95% CI 1.90-18.65 and OR 6.3, 95% CI 2.01-19...
November 3, 2017: Oncotarget
https://www.readbyqxmd.com/read/29187158/associations-of-intimate-partner-violence-with-postnatal-health-practices-in-bihar-india
#4
Sabrina C Boyce, Lotus McDougal, Jay G Silverman, Yamini Atmavilas, Diva Dhar, Katherine Hay, Anita Raj
BACKGROUND: Reducing neonatal mortality is a global priority, and improvements in postnatal health (PNH) practices in India are needed to do so. Intimate partner violence (IPV) may be associated with PNH practices, but little research has assessed this relationship. METHODS: A cross-sectional analysis of data from a representative household sample of mothers of neonates 0-11 months old in Bihar, India was conducted. The relationship between lifetime IPV experience (physical violence only, sexual violence only, or both physical and sexual violence) and PNH practices [clean cord care, kangaroo mother care, early initiation of breastfeeding (EIBF), delayed bathing, receipt of a postnatal care visit, exclusive breastfeeding, and current post-partum contraceptive use] was assessed using multivariate logistic regression...
November 29, 2017: BMC Pregnancy and Childbirth
https://www.readbyqxmd.com/read/29147590/paroxysmal-nocturnal-hemoglobinuria-in-pregnancy-a-dilemma-in-treatment-and-thromboprophylaxis
#5
Arpan Patel, Athira Unnikrishnan, Martina Murphy, Robert Egerman, Sarah Wheeler, Ashley Richards, John Wingard
Paroxysmal nocturnal hemoglobinuria (PNH) is a hematologic disorder characterized by an acquired somatic mutation in the phosphatidylinositol glycan class A gene which leads to a higher risk for increased venous and arterial thrombosis. Current treatment for PNH includes eculizumab. Pregnant patients who have PNH have higher risk for thrombosis and hemorrhage with both pregnancy and their underlying PNH. Treatment frequently poses conundrum. The safety and efficacy of eculizumab during pregnancy and breast feeding have not been extensively studied and contraception has been recommended due to potential for teratogenicity...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/29138283/frequency-of-and-reasons-for-paroxysmal-nocturnal-haemoglobinuria-screening-in-patients-with-unexplained-anaemia
#6
James T England, Bakul Dalal, Heather A Leitch
Referral to hematology for anemia is common. In paroxysmal nocturnal hemoglobinuria (PNH), cells deficient in the glycosylphosphatidyl inositol (GPI) anchor are lysed by complement. Eculizumab improves overall survival and quality of life while reducing hemolysis, transfusion requirements, and thrombosis. We evaluated the frequency of screening for PNH in patients with unexplained anemia. Key clinical features, laboratory data, and investigations were recorded for patients referred for anemia since 2010, without a specific cause found...
November 14, 2017: Journal of Clinical Pathology
https://www.readbyqxmd.com/read/29128072/paroxysmal-nocturnal-hemoglobinuria-assessment-by-flow-cytometric-analysis
#7
REVIEW
Mike Keeney, Andrea Illingworth, D Robert Sutherland
Paroxysmal nocturnal hemoglobinuria (PNH) is an uncommon but frequently debilitating disease that, if untreated, may lead to death in up to 35% of patients within 5 years. Assessment of PNH clone size by flow cytometric analysis has increased in importance with the availability of therapeutic treatments, which prevent the hemolysis of red blood cells and, hence, the myriad symptoms that accompany the disease. This article addresses flow cytometric methodologies and highlights areas of importance in implementing testing, not only for classic PNH but also for other related bone marrow failure disorders, such as aplastic anemia and low-grade myelodysplastic syndrome...
December 2017: Clinics in Laboratory Medicine
https://www.readbyqxmd.com/read/29075054/diagnosis-of-paroxysmal-nocturnal-hemoglobinuria-recent-advances
#8
REVIEW
Prabhu Manivannan, Ankur Ahuja, Hara Prasad Pati
Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal hematopoietic stem cell disorder with its protean clinical manifestations. This is due to partial or complete absence of 'glycophosphatidyl-inositol-anchor proteins' (GPI-AP). The main aim of this review is to highlight various diagnostic modalities available, basic principle of each test and recent advances in the diagnosis of PNH. Recently among various tests available, the flow cytometry has become 'the gold standard' for PNH testing. In order to overcome the difficulties encountered by the testing and research laboratories throughout the world, International Clinical Cytometry Society has come up with guidelines regarding the indications for testing, protocol for sample collection, processing, panel of antibodies as well as gating strategies to be used, how to interpret the test and reporting format to be used...
December 2017: Indian Journal of Hematology & Blood Transfusion
https://www.readbyqxmd.com/read/29062687/integrity-of-the-corpus-callosum-in-patients-with-periventricular-nodular-heterotopia-related-epilepsy-by-flna-mutation
#9
Wenyu Liu, Dongmei An, Running Niu, Qiyong Gong, Dong Zhou
OBJECTIVE: To investigate the quantitative diffusion properties of the corpus callosum (CC) in a large group of patients with periventricular nodular heterotopia (PNH) related epilepsy and to further investigate the effect of Filamin A (FLNA) mutation on these properties. METHODS: Patients with PNH (n = 34), subdivided into FLNA-mutated (n = 11) and FLNA-nonmutated patients (n = 23) and healthy controls (n = 34), underwent 3.0 T structural MRI and diffusion imaging scan (64 direction)...
2018: NeuroImage: Clinical
https://www.readbyqxmd.com/read/29043236/efficacy-of-eculizumab-in-paroxysmal-nocturnal-hemoglobinuria-patients-with-or-without-aplastic-anemia-prospective-study-of-a-korean-pnh-cohort
#10
Chul Won Choi, Jun Ho Jang, Jin Seok Kim, Deog-Yeon Jo, Je-Hwan Lee, Sung-Hyun Kim, Yeo-Kyeoung Kim, Jong-Ho Won, Joo Seop Chung, Hawk Kim, Jae Hoon Lee, Min Kyoung Kim, Hyeon-Seok Eom, Shin Young Hyun, Jeong-A Kim, Jong Wook Lee
BACKGROUND: Patients with paroxysmal nocturnal hemoglobinuria (PNH) often have concurrent aplastic anemia (AA). This study aimed to determine whether eculizumab-treated patients show clinical benefit regardless of concurrent AA. METHODS: We analyzed 46 PNH patients ≥18 years of age who were diagnosed by flow cytometry and treated with eculizumab for more than 6 months in the prospective Korean PNH registry. Patients were categorized into two groups: PNH patients with concurrent AA (PNH/AA, N=27) and without AA (classic PNH, N=19)...
September 2017: Blood Research
https://www.readbyqxmd.com/read/29021487/severe-infection-of-pseudomonas-aeruginosa-during-eculizumab-therapy-for-paroxysmal-nocturnal-hemoglobinuria
#11
Toru Kawakami, Hideyuki Nakazawa, Yukifumi Kurasawa, Hitoshi Sakai, Sayaka Nishina, Noriko Senoo, Yasushi Senoo, Fumihiro Ishida
Eculizumab is the complement inhibitor administered to ameliorate intravascular hemolysis in paroxysmal nocturnal hemoglobinuria (PNH). Whether or not the inhibitory mechanism may also increase the susceptibility to non-Neisserial infection is unclear. A 73-year old woman presented with bacteremia, cholecystitis and liver abscess with Pseudomonas aeruginosa. Although she had been neutropenic for 21 years, she had no history of severe infection before eculizumab had been administered. The infection with P. aeruginosa was successfully controlled with antibiotics, granulocyte colony-stimulating factor and cholecystectomy...
October 11, 2017: Internal Medicine
https://www.readbyqxmd.com/read/28958970/hemolytic-anemia-iron-deficiency-and-personal-history-of-deep-vein-thrombosis-consider-paroxysmal-nocturnal-hemoglobinuria
#12
Nicolas Gendron, Jean-Benoit Arlet, Pascale Gaussem, Isabelle Radford-Weiss, Sidonie Dupeux, Jérémie Rosain, Régis Peffault de La Tour, Luc Darnige
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired hematopoietic stem cell disorder that must be correctly diagnose because it is a chronic disease with a real impact on the quality of life and the survival of the patients. PNH screening of all patients with anemia or thrombosis is not recommended. We report the case of a 71-year-old male patient referred for chronic anemia. Anemia work-up revealed a misunderstood association of a hemolytic anemia with a negative direct antiglobulin test and iron deficiency...
October 1, 2017: Annales de Biologie Clinique
https://www.readbyqxmd.com/read/28918528/signaling-of-the-complement-cleavage-product-anaphylatoxin-c5a-through-c5ar-cd88-contributes-to-pharmacological-hematopoietic-stem-cell-mobilization
#13
Kamila Bujko, Sylwia Rzeszotek, Kai Hoehlig, Jun Yan, Axel Vater, Mariusz Z Ratajczak
Several mechanisms have been postulated for orchestrating the mobilization of hematopoietic stem/progenitor cells (HSPCs), and we previously proposed that activation of the complement cascade plays a crucial role in the initiation and execution of the egress of HSPCs from bone marrow (BM) into peripheral blood (PB). In support of this notion, we demonstrated that mice deficient in the mannan-binding lectin (MBL) pathway, which activates the proximal part of the complement cascade, as well as mice deficient in the fifth component of the complement cascade (C5), which is part of the distal part of the complement cascade, are poor mobilizers...
September 16, 2017: Stem Cell Reviews
https://www.readbyqxmd.com/read/28880999/stereo-eeg-diagnostic-and-therapeutic-tool-for-periventricular-nodular-heterotopia-epilepsies
#14
Laura Mirandola, Roberto F Mai, Stefano Francione, Veronica Pelliccia, Francesca Gozzo, Ivana Sartori, Lino Nobili, Francesco Cardinale, Massimo Cossu, Stefano Meletti, Laura Tassi
OBJECTIVE: Periventricular nodular heterotopias (PNHs) are malformations of cortical development related to neuronal migration disorders, frequently associated with drug-resistant epilepsy (DRE). Stereo-electroencephalography (SEEG) is considered a very effective step of the presurgical evaluation, providing the recognition of the epileptogenic zone (EZ). At the same time, via the intracerebral electrodes it is possible to perform radiofrequency thermocoagulation (SEEG-guided RF-TC) with the aim of ablating and/or disrupting the EZ...
November 2017: Epilepsia
https://www.readbyqxmd.com/read/28875535/disrupted-intrinsic-and-remote-functional-connectivity-in-heterotopia-related-epilepsy
#15
W Liu, X Hu, D An, Q Gong, D Zhou
OBJECTIVES: Several neuroimaging studies have examined neural interactions in patients with periventricular nodular heterotopia (PNH). However, features of the underlying functional network remain poorly understood. In this study, we examined alterations in the local (regional) and remote (interregional) cerebral networks in this disorder. METHODS: Twenty-eight subjects all having suffered from PNH with epilepsy, as well as 28 age- and sex- matched healthy controls, were enrolled in this study...
September 6, 2017: Acta Neurologica Scandinavica
https://www.readbyqxmd.com/read/28872216/perilesional-and-contralateral-white-matter-evolution-and-integrity-in-patients-with-periventricular-nodular-heterotopia-and-epilepsy-a-longitudinal-diffusion-tensor-imaging-study
#16
W Liu, B Yan, D An, R Niu, Y Tang, X Tong, Q Gong, D Zhou
BACKGROUND AND PURPOSE: This study aimed to assess the evolution of perinodular and contralateral white matter abnormalities in patients with periventricular nodular heterotopia (PNH) and epilepsy. METHODS: Diffusion tensor imaging (DTI) (64 directions) and 3 T structural magnetic resonance imaging were performed in 29 PNH patients (mean age 27.3 years), and 16 patients underwent a second scan (average time between the two scans 1.1 years). Fractional anisotropy and mean diffusivity were measured within the perilesional and contralateral white matter...
September 5, 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/28850831/region-specific-connectivity-in-patients-with-periventricular-nodular-heterotopia-and-epilepsy-a-study-combining-diffusion-tensor-imaging-and-functional-mri
#17
Wenyu Liu, Dongmei An, Xin Tong, Running Niu, Qiyong Gong, Dong Zhou
OBJECTIVES: Periventricular nodular heterotopia (PNH) is an important cause of chronic epilepsy. The purpose of this study was to evaluate region-specific connectivity in PNH patients with epilepsy and assess correlation between connectivity strength and clinical factors including duration and prognosis. METHODS: Diffusion tensor imaging (DTI) and resting state functional MRI (fMRI) were performed in 28 subjects (mean age 27.4years; range 9-56years). The structural connectivity of fiber bundles passing through the manually-selected segmented nodules and other brain regions were analyzed by tractography...
August 23, 2017: Epilepsy Research
https://www.readbyqxmd.com/read/28833743/paroxysmal-nocturnal-haemoglobinuria-testing-in-blood-transfusion-laboratories-do-they-go-with-the-flow
#18
L Whitby, J White, M Fletcher, A Whitby, C Milkins, D Barnett
Paroxysmal nocturnal haemoglobinuria (PNH) is a rare stem cell disorder causing, in untreated patients, symptoms that include renal damage, thrombosis and increased mortality. When correctly diagnosed and treated, patients have reduced symptoms and normal life expectancies. Historically PNH testing resided within blood transfusion laboratories using techniques that were insensitive, for example, the Ham test. However, technology has evolved and flow cytometry is now regarded as the gold standard methodology...
August 18, 2017: Transfusion Medicine
https://www.readbyqxmd.com/read/28811010/distinct-clinical-characteristics-of-paroxysmal-nocturnal-hemoglobinuria-in-patients-in-southern-taiwan-a-multicenter-investigation
#19
Hui-Ching Wang, Ching-Yuan Kuo, I-Ting Liu, Tsai-Yun Chen, Yu-Hsiang Chang, Shyh-Jer Lin, Shih-Feng Cho, Yi-Chang Liu, Ta-Chih Liu, Sheng-Fung Lin, Chao-Sung Chang
Paroxysmal nocturnal hemoglobinuria (PNH) is an extremely rare acquired disorder. The aim of this study was to investigate the demographics, clinical manifestations, and outcomes of PNH patients in southern Taiwan. Data on PNH patients diagnosed over a 30-year period (1985-2015) were retrospectively collected from four tertiary medical centers in southern Taiwan. Blood samples were collected for hematologic panel testing and flow cytometry detection of PNH clones. Radiologic studies were performed to assess the frequency of complications...
August 2017: Kaohsiung Journal of Medical Sciences
https://www.readbyqxmd.com/read/28808592/a-case-of-fulminant-meningococcemia-it-is-all-in-the-complement
#20
Kellie L Hawkins, Mariah Hoffman, Sonia Okuyama, Sarah E Rowan
Eculizumab is a novel monoclonal antibody that inhibits complement-mediated hemolysis in patients with paroxysmal nocturnal hemoglobinuria (PNH). Complement deficiency is a well-known risk factor for meningococcal infection. We describe a case of a young patient with PNH treated with eculizumab who presented with a life-threatening case of nongroupable meningococcemia. As this new biologic agent becomes more widely prescribed, providers should be aware of the increased risk of meningococcemia. In addition to vaccination, providers may consider the use of oral penicillin for antibiotic prophylaxis against Neisseria meningitidis in these cases of functional complement deficiency...
2017: Case Reports in Infectious Diseases
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