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https://www.readbyqxmd.com/read/29021487/severe-infection-of-pseudomonas-aeruginosa-during-eculizumab-therapy-for-paroxysmal-nocturnal-hemoglobinuria
#1
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
#2
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
#3
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
#4
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...
September 7, 2017: Epilepsia
https://www.readbyqxmd.com/read/28875535/disrupted-intrinsic-and-remote-functional-connectivity-in-heterotopia-related-epilepsy
#5
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
#6
Wenyu Liu, Bo Yan, Dongmei An, Running Niu, Yingying Tang, Xin Tong, Qiyong Gong, Dong 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 (64 direction) and 3T structural MRIs were performed in 29 PNH patients (mean age 27.3 years), and 16 patients underwent a second scan (average time between 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
#7
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
#8
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
#9
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
#10
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
https://www.readbyqxmd.com/read/28808414/diiron-dithiolate-hydrides-complemented-with-proton-responsive-phosphine-amine-ligands
#11
Michaela R Carlson, Ryan Gilbert-Wilson, Danielle R Gray, Joyee Mitra, Thomas B Rauchfuss, Casseday P Richers
The reaction of Fe2(pdt)(CO)6 with two equivalents of Ph2PC6H4NH2 (PNH2) affords the amido hydride HFe2(pdt)(CO)2(PNH2)(PNH) {[H1H](0), pdt(2-) = CH2(CH2S(-))2}. Isolated intermediates in this conversion include Fe2(pdt)(CO)5-(κ(1)-PNH2) and Fe2(pdt)(CO)4(κ(2)-PNH2). X-ray crystallographic analysis of [H1H](0) shows that the chelating amino/amido-phosphine ligands occupy trans-dibasal positions. The (31)P NMR spectrum indicates that [H1H](0) undergoes rapid proton exchange between the amido and amine centers...
July 7, 2017: European Journal of Inorganic Chemistry
https://www.readbyqxmd.com/read/28796431/acute-kidney-injury-in-a-postpartum-woman-with-paroxysmal-nocturnal-hemoglobinuria-a-case-report-and-literature-review
#12
Masatoshi Nishimoto, Masaru Matsui, Hideo Tsushima, Kaori Tanabe, Miho Tagawa, Ken-Ich Samejima, Yasuhiro Akai, Yoshihiko Saito
Paroxysmal nocturnal hemoglobinuria is a rare clonal hematopoietic stem cell disorder characterized by intravascular hemolysis, hemoglobinuria, and inflammatory thrombotic state. Intravascular hemolysis in paroxysmal nocturnal hemoglobinuria (PNH) can lead to acute and chronic renal injury through hemoglobin-mediated toxicity. A 32-year-old pregnant woman with myelodysplastic syndrome was admitted to our hospital with severe preeclampsia. Shortly after an urgent caesarean section, she became obtunded and showed signs of acute kidney injury (AKI) with anuria, severe intravascular hemolysis, and hypermagnesemia...
August 10, 2017: Hemodialysis International
https://www.readbyqxmd.com/read/28781057/multiple-lacunar-infarcts-in-paroxysmal-nocturnal-hemoglobinuria
#13
Alex Tiburtino Meira, Gabriel Sampaio Froehner, André Petean Trindade, Silméia Garcia Zanati Bazan, Gabriel Pereira Braga, Rodrigo Bazan
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired disease characterized by clonal hematopoietic stem cell disorder, with increased mortality and morbidity. Venous thrombosis is the most common cause of mortality in PNH. The relationship between PNH and cerebrovascular disease is unclear; few cases are reported in the literature, most of them related to cerebral venous thrombosis; In PNH the involvement of intracranial and extracranial arterial sites is very rare. We report a case of a 49-year-old woman who has a medical history of diabetes mellitus, hypertension, and PNH and presented multiple lacunar strokes in a routine consultation with a hematologist...
August 3, 2017: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
https://www.readbyqxmd.com/read/28761231/renal-manifestations-in-paroxysmal-nocturnal-hemoglobinuria
#14
R Ram, K P Adiraju, S Gudithi, K V Dakshinamurty
Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired chronic disorder characterized by a triad of clinical features - hemolytic anemia, pancytopenia, and thrombosis. Not many reports of renal involvement in PNH are available in literature. We present a case series of PNH with renal involvement. We present the data of PNH patients who attended to Departments of General Medicine and Nephrology at a government-run tertiary care institute in South India. The diagnosis of PNH in these patients during initial phase, between 1998 and 2004 was based on sucrose lysis and Ham's test...
July 2017: Indian Journal of Nephrology
https://www.readbyqxmd.com/read/28761230/renal-biopsy-in-paroxysmal-nocturnal-hemoglobinuria-an-insight-into-the-spectrum-of-morphologic-changes
#15
V Puri, A Gandhi, S Sharma
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired, potentially life-threatening disease of blood, characterized by complement-induced intravascular hemolytic anemia and thrombosis. PNH can sometimes present directly with renal manifestations, without showing any hematological manifestation. It, therefore, becomes essential for clinicians and pathologists to be aware of the spectrum of renal changes in PNH. The aim of this study was to document the morphologic changes observed in renal biopsies in patients with PNH...
July 2017: Indian Journal of Nephrology
https://www.readbyqxmd.com/read/28748287/presence-of-acute-and-chronic-renal-failure-in-patients-with-paroxysmal-nocturnal-hemoglobinuria-results-of-a-retrospective-analysis-from-the-spanish-pnh-registry
#16
Ana Villegas, Ramiro Núñez, Anna Gaya, María Victoria Cuevas-Ruiz, José Miguel Bosch, Anna Carral, Beatriz Arrizabalaga, María Isabel Gómez-Roncero, Asunción Mora, Pilar Bravo, Esperanza Lavilla, Carmen Monteserín, Belén Hernández, Pilar Martínez-Barranco, Isidro Jarque, María Anunciación Urquía, Gloria García-Donas, Salut Brunet, Fernando Ataulfo González, Álvaro Urbano
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, life-threatening blood disease. With the advent of eculizumab treatment, renal function has substantially improved, although no data from real-world clinical practice are available. An observational, retrospective, multicenter study was conducted in Spain on clinical data obtained from outpatient visits of patients with PNH (Spanish PNH Registry) who had experienced acute (ARF) or chronic (CRF) renal failure. Of the 128 patients registered (April 2014), 60 were diagnosed with classic PNH...
July 26, 2017: Annals of Hematology
https://www.readbyqxmd.com/read/28728504/the-diverse-expression-of-the-wt1-gene-in-patients-with-acquired-bone-marrow-failure-syndromes
#17
Yahong You, Jiali Huo, Shihong Lu, Yingqi Shao, Meili Ge, Jun Shi, Xingxin Li, Jinbo Huang, Zhendong Huang, Jing Zhang, Min Wang, Neng Nie, Yizhou Zheng
Acquired bone marrow failure syndromes (aBMFS) encompass a wide range of diseases. A study to investigate WT1 expression in BM was conducted in 387 patients with aBMFS in China. The WT1 level in patients with aplastic anemia (AA) was significantly lower than that in patients with paroxysmal nocturnal hemoglobinuria (PNH, p = .023) and myelodysplastic syndrome (MDS, p < .001). In addition, the WT1 level in patients with MDS significantly increased as the disease progressed to an advanced stage. Patients with hypoplastic MDS had a differentiated expression level of WT1 compared with that of NSAA (p < ...
July 21, 2017: Leukemia & Lymphoma
https://www.readbyqxmd.com/read/28718265/haemoglobinuria-and-portal-venous-thrombosis-in-a-young-male
#18
Zain Ul Abideen, Munnam Sohail Jafar, Nasir Hameed, Ahmad Malik
Paroxysmal nocturnal haemoglobinuria is a non-malignant stem cell disorder due to acquired somatic mutations in cell surface anchored proteins CD55 and CD59. Both have a compliment inhibitory role and their deficiency leads to intravascular haemolysis. This paper reports a challenging case of a 25 years old male who presented with generalized weakness, exertional dyspnoea and episodic early morning haematuria. Recently, he started developing progressive abdominal distention and dull generalized abdominal pain...
April 2017: Journal of Ayub Medical College, Abbottabad: JAMC
https://www.readbyqxmd.com/read/28692147/persisting-hyperbilirubinemia-in-patients-with-paroxysmal-nocturnal-hemoglobinuria-pnh-chronically-treated-with-eculizumab-the-role-of-hepatocanalicular-transporter-variants
#19
Ferras Alashkar, Susanne N Weber, Colin Vance, Dörte Herich-Terhürne, Ulrich Dührsen, Frank Lammert, Alexander Röth
BACKGROUND: Eculizumab-treated paroxysmal nocturnal hemoglobinuria (PNH) patients (pts) show a dramatic decrease in serum lactate dehydrogenase (LDH) activities and bilirubin concentrations. However, some pts remain hyperbilirubinemic, possibly indicating an inadequate response due to extravascular hemolysis. METHODS: Mutation analyses of hepatocanalicular transporter/nuclear receptor variants (ABCB4, ABCB11, ATP8B1, NR1H4) were performed in eight (five of eight males; mean age 38 years [range 26-68 years]) out of the 174 pts with PNH/-clone at our department due to a persistent increase in total bilirubin concentrations (median 3...
July 10, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28675769/outcomes-of-allogeneic-stem-cell-transplantation-in-patients-with-paroxysmal-nocturnal-hemoglobinuria-with-or-without-aplastic-anemia
#20
Sung-Eun Lee, Sung Soo Park, Young-Woo Jeon, Jae-Ho Yoon, Byung-Sik Cho, Ki-Sung Eom, Yoo-Jin Kim, Seok Lee, Chang-Ki Min, Hee-Je Kim, Seok-Goo Cho, Dong-Wook Kim, Woo-Sung Min, Jong Wook Lee
OBJECTIVE: The aim of this study was to evaluate the long-term outcomes of allogeneic stem cell transplantation (SCT) in patients with paroxysmal nocturnal hemoglobinuria (PNH) with or without aplastic anemia (AA). METHOD: A total of 33 patients with PNH clones who underwent allogeneic SCT were analyzed. RESULTS: After a median follow-up of 57 months (range, 6.0-151.3), the 5-year estimated overall survival rate was 87.9±5.7%. Four patients died of transplant-related mortality (TRM)...
July 4, 2017: European Journal of Haematology
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