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https://www.readbyqxmd.com/read/28074147/cerebellar-ataxia-and-sensory-ganglionopathy-associated-with-light-chain-myeloma
#1
Panagiotis Zis, Dasappaiah Ganesh Rao, Bart E Wagner, Lucinda Nicholson-Goult, Nigel Hoggard, Marios Hadjivassiliou
BACKGROUND: Cerebellar ataxia with sensory ganglionopathy is a rare neurological combination that can occur in some hereditary ataxias including mitochondrial diseases and in gluten sensitivity. Individually each condition can be a classic paraneoplastic neurological syndrome. We report a patient with this combination who was diagnosed with light-chain myeloma ten years after initial presentation. CASE PRESENTATION: A 65-year-old Caucasian lady was referred to our Ataxia Clinic because of a 6-year history of progressive unsteadiness and a 2-year history of slurred speech...
2017: Cerebellum & Ataxias
https://www.readbyqxmd.com/read/28070238/hematological-disorders-and-pulmonary-hypertension
#2
REVIEW
Rajamma Mathew, Jing Huang, Joseph M Wu, John T Fallon, Michael H Gewitz
Pulmonary hypertension (PH), a serious disorder with a high morbidity and mortality rate, is known to occur in a number of unrelated systemic diseases. Several hematological disorders such as sickle cell disease, thalassemia and myeloproliferative diseases develop PH which worsens the prognosis. Associated oxidant injury and vascular inflammation cause endothelial damage and dysfunction. Pulmonary vascular endothelial damage/dysfunction is an early event in PH resulting in the loss of vascular reactivity, activation of proliferative and antiapoptotic pathways leading to vascular remodeling, elevated pulmonary artery pressure, right ventricular hypertrophy and premature death...
December 26, 2016: World Journal of Cardiology
https://www.readbyqxmd.com/read/28060579/a-comparison-of-the-metabolic-complications-and-hospital-course-of-severe-anorexia-nervosa-by-binge-purge-and-restricting-subtypes
#3
Melanie Rylander, John T Brinton, Allison L Sabel, Philip S Mehler, Jennifer L Gaudiani
This study examines adult patients with severe, life-threatening anorexia nervosa who were admitted to an inpatient, medical stabilization unit between October 1, 2008 and December 31, 2014. Specifically, the study compares anorexia nervosa, binge purge subtype (AN-BP) and anorexia nervosa, restricting subtype (AN-R) on admission measures, hospital course, and outcomes. Of the 232 patients, 46% (N = 108) had AN-BP. Patients with AN-R manifested a higher frequency of underweight-mediated medical complications, including bone marrow dysfunction, hepatic dysfunction, and hypoglycemia...
January 6, 2017: Eating Disorders
https://www.readbyqxmd.com/read/28041853/stem-cells-in-male-sexual-dysfunction-are-we-getting-somewhere
#4
REVIEW
Mohammad Ayodhia Soebadi, Uros Milenkovic, Emmanuel Weyne, Fabio Castiglione, Maarten Albersen
INTRODUCTION: Stem cells for sexual disorders are steadily being introduced into clinical trials. Two conditions of importance are the main target for this line of treatment, especially when regarding the wide array of translational and basic science highlighting the potential advantages of regenerative therapy: erectile dysfunction (ED) and more recently Peyronie disease (PD). Cellular therapy offers a treatment modality that might reverse disease progression. It would be used in a curative setting, in contrast to other pharmaceutical agents that are currently available...
December 29, 2016: Sexual Medicine Reviews
https://www.readbyqxmd.com/read/28024491/-children-s-nk-t-cell-lymphoma-associated-hemophagocytic-syndrome-clinical-analysis-of-6-cases
#5
Yong-Zhi Zheng, Hao Zheng, Jian Li, Shao-Hua LE, Xue-Ling Hua, Zai-Sheng Chen, Ling Zheng, Mei Li, Yi-Qiao Chen, Qin-Li Gao, Jing-Hui Yang, Jian-Da Hu
OBJECTIVE: To study the clinical features, treatment and prognosis of patients with NK/T cell lymphoma-associated hemophagocytic syndrome(NK/T-LAHPS). METHODS: Retrospective analysis was used to explore the clinical data of 6 children with NK/T-LAHPS who were admitted in Department of Pediatric Hematology of Fujian Medical University Union Hospital from July 2012 to June 2016. The 6 patients included 4 boys and 2 girls, with a median age of 4 years(range 1.75 to 11)...
December 2016: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/28008770/the-budget-impact-and-cost-effectiveness-of-defibrotide-for-treatment-of-veno-occlusive-disease-with-multi-organ-dysfunction-in-patients-post-hematopoietic-stem-cell-transplant
#6
David L Veenstra, Gregory F Guzauskas, Kathleen F Villa, Denise M Boudreau
BACKGROUND: A Phase-3 study of defibrotide compared with historical controls demonstrated a 23% improvement in 100-day survival post-hematopoietic stem cell transplantation (HSCT) among patients with veno-occlusive disease with multi-organ dysfunction (VOD with MOD). AIM: To estimate the budget impact and cost-effectiveness of introducing defibrotide to a transplant center. METHODS: We developed a budget impact model from the perspective of a bone-marrow transplant center...
December 23, 2016: Journal of Medical Economics
https://www.readbyqxmd.com/read/28006767/a-2-year-longitudinal-prospective-study-of-the-effects-of-eltrombopag-on-bone-marrow-in-patients-with-chronic-immune-thrombocytopenia
#7
Russell K Brynes, Raymond S M Wong, Maung M Thein, Kalpana K Bakshi, Paul Burgess, Dickens Theodore, Attilio Orazi
BACKGROUND: The long-term effects of eltrombopag on bone marrow (BM) reticulin and/or collagen deposition in previously treated adults with chronic immune thrombocytopenia (ITP) were assessed. METHODS: Three BM biopsies were collected at baseline and after 1 and 2 years of eltrombopag treatment. Specimens were centrally processed, stained for reticulin and collagen, independently reviewed by 2 hematopathologists, and rated according to the European Consensus 0-3 scale of marrow fibrosis (MF)...
December 23, 2016: Acta Haematologica
https://www.readbyqxmd.com/read/27998927/niche-wnt5a-regulates-the-actin-cytoskeleton-during-regeneration-of-hematopoietic-stem-cells
#8
Christina Schreck, Rouzanna Istvánffy, Christoph Ziegenhain, Theresa Sippenauer, Franziska Ruf, Lynette Henkel, Florian Gärtner, Beate Vieth, M Carolina Florian, Nicole Mende, Anna Taubenberger, Áine Prendergast, Alina Wagner, Charlotta Pagel, Sandra Grziwok, Katharina S Götze, Jochen Guck, Douglas C Dean, Steffen Massberg, Marieke Essers, Claudia Waskow, Hartmut Geiger, Mathias Schiemann, Christian Peschel, Wolfgang Enard, Robert A J Oostendorp
Here, we show that the Wnt5a-haploinsufficient niche regenerates dysfunctional HSCs, which do not successfully engraft in secondary recipients. RNA sequencing of the regenerated donor Lin(-) SCA-1(+) KIT(+) (LSK) cells shows dysregulated expression of ZEB1-associated genes involved in the small GTPase-dependent actin polymerization pathway. Misexpression of DOCK2, WAVE2, and activation of CDC42 results in apolar F-actin localization, leading to defects in adhesion, migration and homing of HSCs regenerated in a Wnt5a-haploinsufficient microenvironment...
January 2017: Journal of Experimental Medicine
https://www.readbyqxmd.com/read/27994856/c3-glomerulopathy-in-adults-a-distinct-patient-subset-showing-frequent-association-with-monoclonal-gammopathy-and-poor-renal-outcome
#9
Isaac E Lloyd, Alexander Gallan, Hunter K Huston, Kalani L Raphael, Dylan V Miller, Monica P Revelo, Mazdak A Khalighi
BACKGROUND: C3 glomerulopathy (C3G) includes both C3 glomerulonephritis (C3GN) and dense deposit disease (DDD) and is defined by C3-dominant deposits on immunofluorescence. Dysfunction of the alternative pathway (AP) of complement is central to the pathophysiology of C3G and young patients often harbor genetic alterations of AP mediators. Recently, a link between C3G and paraproteinemia has been established. We performed this study to better characterize older patients with C3G where this association is more frequently seen...
December 2016: Clinical Kidney Journal
https://www.readbyqxmd.com/read/27981571/haematopoietic-prolyl-hydroxylase-1-deficiency-promotes-m2-macrophage-polarization-and-is-both-necessary-and-sufficient-to-protect-against-experimental-colitis
#10
Sophie Van Welden, Martine De Vos, Ben Wielockx, Simon J Tavernier, Melissa Dullaers, Sara Neyt, Benedicte Descamps, Lindsey Devisscher, Sarah Devriese, Lien Van den Bossche, Tom Holvoet, Ann Baeyens, Carmen Correale, Silvia D'Alessio, Christian Vanhove, Filip De Vos, Bruno Verhasselt, Georg Breier, Bart N Lambrecht, Sophie Janssens, Peter Carmeliet, Silvio Danese, Dirk Elewaut, Debby Laukens, Pieter Hindryckx
Prolyl hydroxylase domain-containing proteins (PHD) regulate the adaptation of cells to hypoxia. Pan-hydroxylase inhibition is protective in experimental colitis in which PHD1 plays a prominent role. However, it is currently unknown how PHD1 targeting regulates this protection and which cell type(s) are involved. Here we demonstrated that Phd1 deletion in endothelial and haematopoietic cells (Phd1(f/)(f) Tie2:cre) protected mice from dextran sulphate sodium (DSS)-induced colitis with reduced epithelial erosions, immune cell infiltration and colonic microvascular dysfunction, whereas the response of Phd2(f/+) Tie2:cre and Phd3(f/)(f) Tie2:cre mice to DSS was similar to their littermate controls...
December 16, 2016: Journal of Pathology
https://www.readbyqxmd.com/read/27979832/cellular-senescence-promotes-adverse-effects-of-chemotherapy-and-cancer-relapse
#11
Marco Demaria, Monique N O'Leary, Jianhui Chang, Lijian Shao, Su Liu, Fatouma Alimirah, Kristin Koenig, Catherine Le, Natalia Mitin, Allison M Deal, Shani Alston, Emmeline C Academia, Sumner Kilmarx, Alexis Valdovinos, Boshi Wang, Alain de Bruin, Brian K Kennedy, Simon Melov, Daohong Zhou, Norman E Sharpless, Hyman Muss, Judith Campisi
Cellular senescence suppresses cancer by irreversibly arresting cell proliferation. Senescent cells acquire a pro-inflammatory senescence-associated secretory phenotype. Many genotoxic chemotherapies target proliferating cells non-specifically, often with adverse reactions. In accord with prior work, we show that several chemotherapeutic drugs induce senescence of primary murine and human cells. Using a transgenic mouse that permits tracking and eliminating senescent cells, we show that therapy-induced senescent (TIS) cells persist and contribute to local and systemic inflammation...
December 15, 2016: Cancer Discovery
https://www.readbyqxmd.com/read/27941791/bone-marrow-derived-immature-myeloid-cells-are-a-main-source-of-circulating-supar-contributing-to-proteinuric-kidney-disease
#12
Eunsil Hahm, Changli Wei, Isabel Fernandez, Jing Li, Nicholas J Tardi, Melissa Tracy, Shikha Wadhwani, Yanxia Cao, Vasil Peev, Andrew Zloza, Jevgenijs Lusciks, Salim S Hayek, Christopher O'Connor, Markus Bitzer, Vineet Gupta, Sanja Sever, David B Sykes, David T Scadden, Jochen Reiser
Excess levels of protein in urine (proteinuria) is a hallmark of kidney disease that typically occurs in conjunction with diabetes, hypertension, gene mutations, toxins or infections but may also be of unknown cause (idiopathic). Systemic soluble urokinase plasminogen activator receptor (suPAR) is a circulating factor implicated in the onset and progression of chronic kidney disease (CKD), such as focal segmental glomerulosclerosis (FSGS). The cellular source(s) of elevated suPAR associated with future and progressing kidney disease is unclear, but is likely extra-renal, as the pathological uPAR is circulating and FSGS can recur even after a damaged kidney is replaced with a healthy donor organ...
January 2017: Nature Medicine
https://www.readbyqxmd.com/read/27941768/androgens-in-women-after-allogeneic-hematopoietic-cell-transplantation-impact-of-chronic-gvhd-and-glucocorticoid-therapy
#13
Y Björk, E Smith Knutsson, C Ankarberg-Lindgren, A-K Broman, I Andersson, L Björkman, J Magnusson, K Bergmark, H Anderson, P-O Andersson, M Brune
Low androgen levels may contribute to sexual dysfunction in women after allogeneic hematopoietic cell transplantation (alloHCT). However, data on serum androgens in women after alloHCT are limited. The aim of this study was to assess androgen levels and their association with chronic GvHD (cGvHD) and glucocorticoid (GC) therapy. Included were 65 allografted women, 33 with cGvHD, and 23 of these were on GC therapy. Controls were 94 healthy, age-matched women. Supportive study groups were women after autologous HCT (autoHCT; n=20) and non-transplanted women on GC therapy (n=26)...
December 12, 2016: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/27931514/vascular-alterations-in-a-murine-model-of-acute-graft-versus-host-disease-are-associated-with-decreased-serum-levels-of-adiponectin-and-an-increased-activity-and-vascular-expression-of-indoleamine-2-3-dioxygenase
#14
Peter M Schmid, Abdellatif Bouazzaoui, Karin Schmid, Christoph M Birner, Christian Schach, Lars S Maier, Ernst Holler, Dierk H Endemann
Graft-versus-host disease (GVHD) is the limiting complication after bone marrow transplantation (BMT), and its pathophysiology seems to be highly influenced by vascular factors. Our study aimed at elucidating possible mechanisms involved in vascular GVHD. For this purpose, we used a fully MHC-mismatched model of BALB/c mice conditioned according to two different intensity protocols with total body irradiation and transplantation of allogeneic (C57BL/6) or syngeneic bone marrow cells and splenocytes. Mesenteric resistance arteries were studied in a pressurized myograph...
November 2016: Cell Transplantation
https://www.readbyqxmd.com/read/27929539/sensitivity-of-hematopoietic-stem-cells-to-mitochondrial-dysfunction-by-sdhd-gene-deletion
#15
José Antonio Bejarano-García, África Millán-Uclés, Iván V Rosado, Luís Ignacio Sánchez-Abarca, Teresa Caballero-Velázquez, María José Durán-Galván, José Antonio Pérez-Simón, José I Piruat
It is established that hematopoietic stem cells (HSC) in the hypoxic bone marrow have adapted their metabolism to oxygen-limiting conditions. This adaptation includes suppression of mitochondrial activity, induction of anerobic glycolysis, and activation of hypoxia-inducible transcription factor 1α (Hif1α)-dependent gene expression. During progression of hematopoiesis, a metabolic switch towards mitochondrial oxidative phosphorylation is observed, making this organelle essential for determining cell fate choice in bone marrow...
December 8, 2016: Cell Death & Disease
https://www.readbyqxmd.com/read/27928380/facial-dystonia-with-facial-grimacing-and-vertical-gaze-palsy-with-round-the-houses-sign-in-a-29-year-old-woman
#16
J Crespi, G Bråthen, P Quist-Paulsen, J Pagonabarraga, C Roig-Arnall
A 29-year-old woman developed progressive dysarthria and coordination problems from the age of 15. Examination showed dysarthria, facial dystonia, bibrachial dystonia, hyperreflexia, ataxia, and emotional incontinence. Downward supranuclear gaze palsy was prominent with a "Round the Houses" sign. Magnetic resonance imaging of the brain and medulla, electroneurography, and cerebrospinal fluid were normal. A computed tomography scan showed hepatosplenomegaly. This combination of progressive neurological symptoms together with hepatosplenomegaly was suggestive of inborn error of metabolism...
February 2016: Neuro-ophthalmology
https://www.readbyqxmd.com/read/27927779/depletion-of-gut-microbiota-protects-against-renal-ischemia-reperfusion-injury
#17
Diba Emal, Elena Rampanelli, Ingrid Stroo, Loes M Butter, Gwendoline J Teske, Nike Claessen, Geurt Stokman, Sandrine Florquin, Jaklien C Leemans, Mark C Dessing
An accumulating body of evidence shows that gut microbiota fulfill an important role in health and disease by modulating local and systemic immunity. The importance of the microbiome in the development of kidney disease, however, is largely unknown. To study this concept, we depleted gut microbiota with broad-spectrum antibiotics and performed renal ischemia-reperfusion (I/R) injury in mice. Depletion of the microbiota significantly attenuated renal damage, dysfunction, and remote organ injury and maintained tubular integrity after renal I/R injury...
December 7, 2016: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/27927037/clinical-hepatotoxicity-associated-with-antifungal-agents
#18
Ioannis Kyriakidis, Athanasios Tragiannidis, Silke Munchen, Andreas H Groll
Invasive fungal diseases (IFDs) are a leading cause of morbidity and mortality among immunocompromised patients with bone marrow failure syndromes, hematological malignancies, hematopoietic stem cell transplantation (HSCT), those admitted in intensive care units (ICUs) and those with prolonged febrile neutropenia. IFDs occur in a setting of multiple morbidities and are associated with case fatality rates between 30 and 70%. Along with the development of classes and compounds, the last two decades have seen substantial improvements in the prevention and management of these infections and an overall increased use of antifungal agents...
December 16, 2016: Expert Opinion on Drug Safety
https://www.readbyqxmd.com/read/27922691/microarray-expression-profiles-of-genes-in-lung-tissues-of-rats-subjected-to-focal-cerebral-ischemia-induced-lung-injury-following-bone-marrow-derived-mesenchymal-stem-cell-transplantation
#19
Yue Hu, Liu-Lin Xiong, Piao Zhang, Ting-Hua Wang
Ischemia-induced stroke is the most common disease of the nervous system and is associated with a high mortality rate worldwide. Cerebral ischemia may lead to remote organ dysfunction, particular in the lungs, resulting in lung injury. Nowadays, bone marrow-derived mesenchymal stem cells (BMSCs) are widely studied in clinical trials as they may provide an effective solution to the treatment of neurological and cardiac diseases; however, the underlying molecular mechanisms remain unknown. In this study, a model of permanent focal cerebral ischemia-induced lung injury was successfully established and confirmed by neurological evaluation and lung injury scores...
January 2017: International Journal of Molecular Medicine
https://www.readbyqxmd.com/read/27922667/pituitary-sex-hormones-enhance-the-pro%C3%A2-metastatic-potential-of-human-lung-cancer-cells-by-downregulating-the-intracellular-expression-of-heme-oxygenase%C3%A2-1
#20
Ahmed Abdelbaset-Ismail, Daniel Pedziwiatr, Gabriela Schneider, Jacek Niklinski, Radoslaw Charkiewicz, Marcin Moniuszko, Magda Kucia, Mariusz Z Ratajczak
We report that human lung cancer cell lines express functional receptors for pituitary sex hormones (SexHs) and respond to stimulation by follicle‑stimulating hormone (FSH), luteinizing hormone (LH), and prolactin (PRL). Expression of these receptors has also been confirmed in patient lung cancer samples at the mRNA level. Stimulation of human lung cancer cell lines with FSH, LH, or PRL stimulated migration and chemotaxis, and some cell lines responded by enhanced proliferation. Moreover, priming of human lung cancer cells by exposing them to pituitary SexHs resulted in enhanced seeding efficiency of injected human lung cancer cells into bone marrow, liver, and lungs in an immunodeficient mouse model...
January 2017: International Journal of Oncology
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