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https://www.readbyqxmd.com/read/28418828/a-comparison-of-blood-and-cerebrospinal-fluid-cytokines-il-1%C3%AE-il-6-il-18-tnf-%C3%AE-in-neonates-with-perinatal-hypoxia
#1
Darinka Šumanović-Glamuzina, Filip Čulo, Melanie Ivana Čulo, Paško Konjevoda, Marjana Jerković-Raguž
Perinatal hypoxia-ischemia is a specific and important pathological event in neonatal care practice. The data on relationship between the concentrations of cytokines in blood and cerebrospinal fluid (CSF) and perinatal brain injury are scarce. The aim of this study is to evaluate changes in interleukin (IL-1β, IL-6, and IL-18) and tumor necrosis factor alpha (TNF-α) levels in newborns with perinatal hypoxia (PNH). CSF and serum samples of 35 term and near-term (35-40 weeks) newborns with PNH, at the age of 3-96 hours, were analyzed using enzyme-linked immunosorbent assay...
April 18, 2017: Bosnian Journal of Basic Medical Sciences
https://www.readbyqxmd.com/read/28411558/sporadic-periventricular-nodular-heterotopia-classification-phenotype-and-correlation-with-filamin-a-mutations
#2
Wenyu Liu, Bo Yan, Dongmei An, Jiahe Xiao, Fayun Hu, Dong Zhou
OBJECTIVE: The purpose of this study was to better delineate the clinical spectrum of periventricular nodular heterotopia (PNH) in a large patient population after long term follow up. Specifically, this study aimed to relate PNH subtypes to clinical or epileptic outcomes, epileptic discharges and underlying Filamin A (FLNA) mutations by analyzing anatomical features. METHODS: The study included 100 patients with radiologically confirmed nodular heterotopia. Patients' FLNA gene sequences and medical records were analyzed...
April 4, 2017: Epilepsy Research
https://www.readbyqxmd.com/read/28406545/diagnosing-nocturnal-paroxysmal-hemoglobinuria-a-single-center-4-year-experience
#3
T Mercier, T Devos, M Mukovnikova, N Boeckx
INTRODUCTION: Paroxysmal nocturnal hemoglobinuria (PNH) is a rare disease and can present as a wide range of signs and symptoms. As such, the indication for diagnostic testing for PNH is not always straightforward. Therefore, we analyzed all first-time samples tested over a 56-month period to determine the clinical settings with a high probability of detecting a PNH clone. METHODS: We retrospectively analyzed 323 first-time PNH flow cytometry tests, including LDH, cytopenias, direct antiglobulin test (DAT), and clinical indication for testing as available at the time of testing...
April 13, 2017: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/28380398/a-retrospective-study-of-paroxysmal-nocturnal-hemoglobinuria-in-pediatric-and-adolescent-patients
#4
Angela Mercuri, Piero Farruggia, Fabio Timeus, Laura Lombardi, Daniela Onofrillo, Maria Caterina Putti, Marta Pillon, Maria Elena Cantarini, Paola Corti, Gloria Tridello, Massimiliano De Bortoli, Anna Pegoraro, Simone Cesaro
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare disease, especially in children, characterized by intravascular hemolysis, thrombotic events, serious infections and bone marrow failure. We describe 16 patients who were diagnosed with PNH in childhood or adolescence. The time interval between the onset of symptoms and the PNH diagnosis and its treatment were compared in patients with classic PNH versus PNH associated with bone marrow disorder (PNH/BMD). A greater delay in diagnosis was observed in classic PNH compared to PNH/BMD patients...
March 18, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28350378/developing-a-dataset-to-track-aid-for-reproductive-maternal-newborn-and-child-health-2003-2013
#5
Christopher Grollman, Leonardo Arregoces, Melisa Martinez-Alvarez, Catherine Pitt, Timothy Powell-Jackson, Justine Hsu, Giulia Greco, Josephine Borghi
We created a dataset to generate estimates of donor-reported 'official development assistance' and private grants (ODA+) to reproductive, maternal, newborn and child health (RMNCH) by donor, recipient country and activity type over the period 2003-2013. We collected disbursement information from the Organisation for Economic Co-operation and Development Creditor Reporting System (CRS) in January 2015. All 2.1 million records across all sectors were coded based on donor name, project title, short and long descriptions, and CRS code describing the purpose of the disbursement...
March 28, 2017: Scientific Data
https://www.readbyqxmd.com/read/28332730/multicenter-validation-of-a-simplified-method-for-paroxysmal-nocturnal-hemoglobinuria-screening
#6
Arianna Gatti, Luigi Del Vecchio, Massimo Geuna, Matteo G Della Porta, Bruno Brando
BACKGROUND: Paroxysmal nocturnal hemoglobinuria (PNH) diagnostic guidelines recommend single-tube five- to six-color or two-tube four-color assays. PNH clones are detectable in only a fraction of patients at risk, and screening for new PNH cases can be complex and expensive. In this multicenter study, we have validated a simplified, one-tube two-color FLAER-based assay suitable for PNH screening. METHODS: Six laboratories received samples containing spiked PNH leukocyte clones to be analyzed in parallel with a common six-color cocktail (FLAER/CD24/CD45/CD64/CD15/CD14) and a simplified two-color mixture (FLAER/CD15), a shared calibration procedure, and a common analysis protocol...
March 23, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28328837/atypical-presentation-of-paroxysmal-nocturnal-hemoglobinuria-treated-by-eculizumab-a-case-report
#7
Anne Quinquenel, Quentin Maestraggi, Carinne Lecoq-Lafon, Régis Peffault de Latour, Alain Delmer, Amélie Servettaz
RATIONALE: Paroxysmal nocturnal hemoglobinuria (PNH) is a nonmalignant acquired hematopoietic stem cell disease, which can be revealed by hemolytic anemia, thromboembolism, or bonemarrow failure. Thrombosis can occur at any site, but coronary thrombosis is extremely rare. Controlled trials have demonstrated that eculizimab, an inhibitor of the terminal complement cascade, was able to reduce both hemolysis and thrombosis, but its efficacy in cases of PNH with coronary thrombosis is unknown...
March 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28295191/clonal-dominance-of-pnh-another-piece-to-the-jigsaw
#8
Austin G Kulasekararaj
No abstract text is available yet for this article.
April 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28292372/clinico-haematological-features-of-paroxysmal-nocturnal-haemoglobinuria
#9
Maria Khan, Saqib Qayyum Ahmad, Mukarram Bashir, Parvez Ahmed, Muhammad Ayyub
The aim of this study was to determine the frequency of various clinico-haematological features in patients suffering from paroxysmal nocturnal haemoglobinuria (PNH). It was an observational study carried out from October 2008 - January 2016. All the patients of PNH, diagnosed on the basis of clinical and laboratory findings and confirmed by CD55 and CD59 deficiency on red cells by means of flow cytometry, were included in the study. A total of 22 patients were diagnosed which included 18 (81.8%) males and 4 (18...
January 2017: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
https://www.readbyqxmd.com/read/28281210/design-of-a-time-controlled-pulsatile-release-system-for-propranolol-using-the-dry-coated-method-in-vitro-and-in-vivo-evaluation
#10
Kai Chen, Yanyan Wang, Xiumei Gai, Haiying Wang, Yuenan Li, Haoyang Wen, Weisan Pan, Xinggang Yang
The objective of this study was to design a time-controlled pulsatile release (TCPR) system containing propranolol (PNH) as an active pharmaceutical ingredient. Here, the developed dosage forms were coated with hydroxypropyl-methylcellulose (HPMC) and other excipients as barrier layer using dry-coated technology. The influence of HPMC, microcrystalline cellulose (MCC), and lactose in the outer coating and the coating weight on drug release were investigated. Then, a three-factor, five-level central composite design (CCD) and response surface method were used to optimize the formula of the coating...
March 9, 2017: AAPS PharmSciTech
https://www.readbyqxmd.com/read/28256911/paroxysmal-nocturnal-haemoglobinuria-in-a-patient-with-primary-budd-chiari-syndrome-a-contraceptive-challenge
#11
Isabel Friedmann, Jacques Balayla
CASE REPORT: In this report, we describe the unique case of a 21 year-old woman, gravida 1, para 1, with paroxysmal nocturnal haemoglobinuria (PNH) and Budd-Chiari syndrome, as well as severe vaginismus and cervical stenosis, in need of contraception. Herein, we present the clinical considerations and implications taken to arrive at the right contraceptive choice for the patient. DISCUSSION: Budd-Chiari syndrome is defined by the presence of hepatic venous outflow tract obstruction, which may be due to a number of underlying causes...
April 2017: European Journal of Contraception & Reproductive Health Care
https://www.readbyqxmd.com/read/28246555/management-of-thrombosis-in-paroxysmal-nocturnal-hemoglobinuria-a-clinician-s-guide
#12
REVIEW
Morag Griffin, Talha Munir
Paroxysmal nocturnal haemoglobinuria (PNH), an ultra-orphan disease with a prevalence of 15.9 per million in Europe, is a life-threatening disorder, characterized by haemolysis, bone marrow failure and thrombosis. Patients with PNH prior to the availability of eculizumab had a median survival of between 10 and 22 years, with thrombosis accounting for 22-67% of deaths. 29-44% of patients had at least one thrombosis. This paper provides a clinician's guide to the diagnosis, management and complications of PNH, with an emphasis on thrombosis...
March 2017: Therapeutic Advances in Hematology
https://www.readbyqxmd.com/read/28215731/complementopathies
#13
REVIEW
Andrea C Baines, Robert A Brodsky
The complement system is an essential part of the innate immune system that requires careful regulation to ensure responses are appropriately directed against harmful pathogens, while preventing collateral damage to normal host cells and tissues. While deficiency in some components of the complement pathway is associated with increased susceptibility to certain infections, it has also become clear that inappropriate activation of complement is an important contributor to human disease. A number of hematologic disorders are driven by complement, and these disorders may be termed "complementopathies"...
February 6, 2017: Blood Reviews
https://www.readbyqxmd.com/read/28164595/differential-expression-of-cd52-cd14-and-hla-dr-on-cd4-monocytes-in-three-types-of-acquired-bone-marrow-failure-syndromes
#14
Yang Kai, Dai Yuanyuan, Guan Shihe, Mu Xuanxuan, Zhang Hao, Pan Ying, Wu Yuanyuan, Wang Aihua, Sun Beibei, TongYang, Zhou Tingdong
BACKGROUND: Aplastic anemia (AA), paroxysmal nocturnal hemoglobinuria (PNH), and myelodysplastic syndrome (MDS) are the common spectrums of acquired bone marrow failure syndromes (BMFs). Accurate and timely diagnosis is a significant clinical challenge because of the overlapping features. The pathogenesis is not fully understood, but several studies have suggested that defective monocyte functions play an important role. We aimed to find whether the different expressions of CD52, CD14 and HLA-DR on CD4+ monocytes would be helpful in the preliminary diagnosis of acquired BMFs...
September 1, 2016: Clinical Laboratory
https://www.readbyqxmd.com/read/28151558/whole-transcriptome-sequencing-identifies-increased-cxcr2-expression-in-pnh-granulocytes
#15
Kohei Hosokawa, Sachiko Kajigaya, Keyvan Keyvanfar, Wangmin Qiao, Yanling Xie, Angelique Biancotto, Danielle M Townsley, Xingmin Feng, Neal S Young
The aetiology of paroxysmal nocturnal haemoglobinuria (PNH) is a somatic mutation in the X-linked phosphatidylinositol glycan class A gene (PIGA), resulting in global deficiency of glycosyl phosphatidylinositol-anchored proteins (GPI-APs). This study applied RNA-sequencing to examine functional effects of the PIGA mutation in human granulocytes. CXCR2 expression was increased in GPI-AP(-) compared to GPI-AP(+) granulocytes. Macrophage migration inhibitory factor, a CXCR2 agonist, was significantly higher in plasma of PNH patients...
April 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28124080/serologic-response-to-meningococcal-vaccination-in-patients-with-paroxysmal-nocturnal-hemoglobinuria-pnh-chronically-treated-with-the-terminal-complement-inhibitor-eculizumab
#16
Ferras Alashkar, Colin Vance, Dörte Herich-Terhürne, Nicole Preising, Ulrich Dührsen, Alexander Röth
Eculizumab is indicated for the therapy of patients with symptomatic paroxysmal nocturnal hemoglobinuria (PNH). Due to inhibition of terminal complement cascade, patients on eculizumab are susceptible to Neisseria meningitidis infections. The two mainstays to reduce the risk of infection are vaccination and antibiotic prophylaxis. In this retrospective study, serologic response was analyzed after vaccination with a meningococcal vaccine in 23 PNH patients (median age 36 years; range 25 - 88 years; 15 males, 8 females) by measuring serum bactericidal assay (SBA) using rabbit complement (rSBA) titers against meningococcal serogroups A, C, W, and Y...
April 2017: Annals of Hematology
https://www.readbyqxmd.com/read/28100318/-clinical-characteristics-of-clonal-evolution-after-immunosuppressive-therapy-in-children-with-severe-very-severe-aplastic-anemia
#17
Jing-Liao Zhang, Tian-Feng Liu, Li-Xian Chang, Xia Chen, Yuan-Yuan Ren, Cong-Cong Sun, Chao Liu, Wen-Bin An, Yang Wan, Xiao-Juan Chen, Wen-Yu Yang, Shu-Chun Wang, Ye Guo, Yao Zou, Yu-Mei Chen, Xiao-Fan Zhu
OBJECTIVE: To evaluate the clinical characteristics and risk factors of clonal evolution after immunosuppressive therapy (IST) in children with severe/very severe aplastic anemia (SAA/VSAA). METHODS: The clinical data of 231 children with newly-diagnosed SAA/VSAA who received IST were retrospectively studied. The incidence and risk factors of clonal evolution after IST were analyzed. RESULTS: The 5-year overall survival rate of the 231 patients was 82...
January 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/28045484/development-of-autologous-c5-vaccine-nanoparticles-to-reduce-intravascular-hemolysis-in-vivo
#18
Lingjun Zhang, Wen Qiu, Stephen Crooke, Yan Li, Areeba Abid, Bin Xu, M G Finn, Feng Lin
The complement system is emerging as a new target for treating many diseases. For example, Eculizumab, a humanized monoclonal antibody against complement component 5 (C5), has been approved for paroxysmal nocturnal hemoglobinuria (PNH) in which patient erythrocytes are lysed by complement. In this study, we developed vaccines to elicit autologous anti-C5 antibody production in mice for complement inhibition. Immunization of mice with a conservative C5 xenoprotein raised high titers of IgG's against the xenogenous C5, but these antibodies did not reduce C5 activity in the blood...
January 12, 2017: ACS Chemical Biology
https://www.readbyqxmd.com/read/28039017/development-of-pro-inflammatory-phenotype-in-monocytes-after-engulfing-hb-activated-platelets-in-hemolytic-disorders
#19
Rashi Singhal, Sheetal Chawla, Deepak K Rathore, Angika Bhasym, Gowtham K Annarapu, Vandana Sharma, Tulika Seth, Prasenjit Guchhait
Monocytes and macrophage combat infections and maintain homeostatic balance by engulfing microbes and apoptotic cells, and releasing inflammatory cytokines. Studies have described that these cells develop anti-inflammatory properties upon recycling the free-hemoglobin (Hb) in hemolytic conditions. While investigating the phenotype of monocytes in two hemolytic disorders-paroxysmal nocturnal hemoglobinuria (PNH) and sickle cell disease (SCD), we observed a high number of pro-inflammatory (CD14(+)CD16(hi)) monocytes in these patients...
December 28, 2016: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/28028953/opinions-of-old-age-according-to-residents-of-polish-public-nursing-homes-and-members-of-the-universities-of-the-third-age
#20
Mateusz Cybulski, Elżbieta Krajewska-Kułak, Jacek Jamiołkowski
BACKGROUND: Statistical data from the turn of the 21st century shows a significant increase in the average human life span and, what follows, an extension of old age. OBJECTIVES: The aim of this study was to become familiar with the opinions submitted by respondents regarding aging and old age, health problems connected with aging and preferred health behaviors. MATERIAL AND METHODS: The research was conducted between January 3rd 2013 and February 15th 2014 on a group of 200 residents of public nursing homes and 200 members of the University of the Third Age using a questionnaire created by the authors...
September 2016: Advances in Clinical and Experimental Medicine: Official Organ Wroclaw Medical University
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