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Bone Marrow Failure

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https://www.readbyqxmd.com/read/28447876/intrinsic-mesenchymal-stem-cell-dysfunction-in-diabetes-mellitus-implications-for-autologous-cell-therapy
#1
Mari van de Vyver
Mesenchymal stem cells derived from bone marrow (BM-MSCs) have multi-functional properties that have made them a promising therapeutic agent for many regenerative, anti-inflammatory, and auto-immune applications. Under chronic pathological conditions, however, BM-MSCs can become functionally compromised due to long term exposure to changes in the systemic and localized stem cell niche micro-environments. In addition to the fact that functionally compromised BM-MSCs may be therapeutically ineffective, the impairment of BM-MSCs is potentially a contributing factor to disease progression and the development of co-morbidities...
April 27, 2017: Stem Cells and Development
https://www.readbyqxmd.com/read/28444757/hypocobalaminaemia-as-a-cause-of-bone-marrow-failure-and-pancytopenia-in-a-cat
#2
E L Stanley, A E Eatroff
CASE REPORT: A male Domestic Short-hair cat was presented for chronic weight loss, lethargy and hyporexia. Complete haematological examination revealed non-regenerative anaemia, neutropenia and thrombocytopenia, as well as Howell-Jolly bodies, anisocytosis, polychromasia and macrocytosis on blood smear evaluation. Histopathological evaluation of bone marrow biopsy disclosed hypocellularity consistent with bone marrow failure. Concurrent hypocobalaminaemia was identified and treated with parenteral cyanocobalamin supplementation...
May 2017: Australian Veterinary Journal
https://www.readbyqxmd.com/read/28440412/a-rare-fanca-gene-variation-as-a-breast-cancer-susceptibility-allele-in-an-iranian-population
#3
Sakineh Abbasi, Mina Rasouli
Fanconi Anemia (FA) is an autosomal recessive syndrome characterized by congenital abnormalities, progressive bone marrow failure and Fanconi anemia complementation group A (FANCA) is also a potential breast and ovarian cancer susceptibility gene. A novel allele with tandem duplication of 13 base pair sequence in promoter region was identified. To investigate whether the 13 base pair sequence of tandem duplication in promoter region of the FANCA gene is of high penetrance in patients with breast cancer and to determine if the presence of the duplicated allele was associated with an altered risk of breast cancer, the present study screened DNA in blood samples from 304 breast cancer patients and 295 normal individuals as controls...
April 20, 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28439268/alveolar-fluid-clearance-in-pathologically-relevant-conditions-in-vitro-and-in-vivo-models-of-acute-respiratory-distress-syndrome
#4
REVIEW
Laura A Huppert, Michael A Matthay
Critically ill patients with respiratory failure from acute respiratory distress syndrome (ARDS) have reduced ability to clear alveolar edema fluid. This reduction in alveolar fluid clearance (AFC) contributes to the morbidity and mortality in ARDS. Thus, it is important to understand why AFC is reduced in ARDS in order to design targeted therapies. In this review, we highlight experiments that have advanced our understanding of ARDS pathogenesis, with particular reference to the alveolar epithelium. First, we review how vectorial ion transport drives the clearance of alveolar edema fluid in the uninjured lung...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28434866/lentiviral-vectors-with-cellular-promoters-correct-anemia-and-lethal-bone-marrow-failure-in-a-mouse-model-for-diamond-blackfan-anemia
#5
Shubhranshu Debnath, Pekka Jaako, Kavitha Siva, Michael Rothe, Jun Chen, Maria Dahl, H Bobby Gaspar, Johan Flygare, Axel Schambach, Stefan Karlsson
Diamond-Blackfan anemia is a congenital erythroid hypoplasia and is associated with physical malformations and a predisposition to cancer. Twenty-five percent of patients with Diamond-Blackfan anemia have mutations in a gene encoding ribosomal protein S19 (RPS19). Through overexpression of RPS19 using a lentiviral vector with the spleen focus-forming virus promoter, we demonstrated that the Diamond-Blackfan anemia phenotype can be successfully treated in Rps19-deficient mice. In our present study, we assessed the efficacy of a clinically relevant promoter, the human elongation factor 1α short promoter, with or without the locus control region of the β-globin gene for treatment of RPS19-deficient Diamond-Blackfan anemia...
April 20, 2017: Molecular Therapy: the Journal of the American Society of Gene Therapy
https://www.readbyqxmd.com/read/28434530/accelerated-cellular-senescence-as-underlying-mechanism-for-functionally-impaired-bone-marrow-derived-progenitor-cells-in-ischemic-heart-disease
#6
Evelien Nollet, Vicky Y Hoymans, Inez R Rodrigus, Dina De Bock, Marc Dom, Viviane O M Van Hoof, Christiaan J Vrints, Emeline M Van Craenenbroeck
BACKGROUND AND AIMS: Bone marrow (BM)-derived progenitor cells are functionally impaired in patients with ischemic heart disease (IHD), thereby hampering the outcome of autologous stem cell therapy. In search for underlying mechanisms for this BM dysfunction, accelerated cellular senescence was explored. METHODS: We analysed telomere length of BM-derived mononuclear cells (MNC) by MMqPCR in patients with coronary artery disease (n = 12), ischemic heart failure (HF; n = 9), non-ischemic HF (n = 7) and controls (n = 10), and related it to their myeloid differentiation capacity...
May 2017: Atherosclerosis
https://www.readbyqxmd.com/read/28432724/fluorescent-aerolysin-flaer-based-paroxysmal-nocturnal-hemoglobinuria-pnh-screening-a-single-center-experience-from-india
#7
K Rahman, R Gupta, G Yadav, N Husein, M K Singh, S Nityanand
BACKGROUND: Fluorescent aerolysin (FLAER) has been recommended as an important part of antibody panel used for flow cytometric detection of paroxysmal nocturnal hemoglobinuria (PNH) clone. This study was aimed to observe the frequency of PNH-positive clones and their sizes in patients screened for various indications. METHOD: A retrospective analysis of 624 patients screened over a period of 30 months. Frequency and size of clone sizes noted, and laboratory parameters were compared among different groups of patient being screened...
April 22, 2017: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/28428897/acute-myeloid-leukemia-with-basophilic-differentiation-transformed-from-myelodysplastic-syndrome
#8
Yasuhiro Tanaka, Atsushi Tanaka, Akiko Hashimoto, Kumiko Hayashi, Isaku Shinzato
Myelodysplastic syndrome (MDS) terminally transforms to acute myeloid leukemia (AML) or bone marrow failure syndrome, but acute myeloid leukemia with basophilic differentiation has been rarely reported. An 81-year-old man was referred to our department for further examination of intermittent fever and normocytic anemia during immunosuppressive treatment. Chromosomal analysis showed additional abnormalities involving chromosome 7. He was diagnosed as having MDS. At the time of diagnosis, basophils had not proliferated in the bone marrow...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28419882/novel-homozygous-fancl-mutation-and-somatic-heterozygous-setbp1-mutation-in-a-chinese-girl-with-fanconi-anemia
#9
Weiqing Wu, Yang Liu, Qinghua Zhou, Qin Wang, Fuwei Luo, Zhiyong Xu, Qian Geng, Peining Li, Hui Z Zhang, Jiansheng Xie
Fanconi Anemia (FA) is a rare genetically heterogeneous disorder with 17 known complement groups caused by mutations in different genes. FA complementation group L (FA-L, OMIM #608111) occurred in 0.2% of all FA and only eight mutant variants in the FANCL gene were documented. Phenotype and genotype correlation in FANCL associated FA is still obscure. Here we describe a Chinese girl with FA-L caused by a novel homozygous mutation c.822_823insCTTTCAGG (p.Asp275LeufsX13) in the FANCL gene. The patient's clinical course was typical for FA with progression to bone marrow failure, and death from acute myelomonocytic leukemia (AML-M4) at 9 years of age...
April 15, 2017: European Journal of Medical Genetics
https://www.readbyqxmd.com/read/28415593/cysteine-and-glycine-rich-protein-2-csrp2-transcript-levels-correlate-with-leukemia-relapse-and-leukemia-free-survival-in-adults-with-b-cell-acute-lymphoblastic-leukemia-and-normal-cytogenetics
#10
Shu-Juan Wang, Ping-Zhang Wang, Robert Peter Gale, Ya-Zhen Qin, Yan-Rong Liu, Yue-Yun Lai, Hao Jiang, Qian Jiang, Xiao-Hui Zhang, Bin Jiang, Lan-Ping Xu, Xiao-Jun Huang, Kai-Yan Liu, Guo-Rui Ruan
Relapse is the major cause of treatment-failure in adults with B-cell acute lymphoblastic leukemia (ALL) achieving complete remission after induction chemotherapy. Greater precision identifying persons likely to relapse is important. We did bio-informatics analyses of transcriptomic data to identify mRNA transcripts aberrantly-expressed in B-cell ALL. We selected 9 candidate genes for validation 7 of which proved significantly-associated with B-cell ALL. We next focused on function and clinical correlations of the cysteine and glycine-rich protein 2 (CSRP2)...
March 21, 2017: Oncotarget
https://www.readbyqxmd.com/read/28415569/interleukin-2-regulatory-t-cell-axis-critically-regulates-maintenance-of-hematopoietic-stem-cells
#11
Sabrina Giampaolo, Gabriela Wójcik, Edgar Serfling, Amiya K Patra
The role of IL-2 in HSC maintenance is unknown. Here we show that Il2-/- mice develop severe anomalies in HSC maintenance leading to defective hematopoiesis. Whereas, lack of IL-2 signaling was detrimental for lympho- and erythropoiesis, myelopoiesis was enhanced in Il2-/- mice. Investigation of the underlying mechanisms of dysregulated hematopoiesis in Il2-/- mice shows that the IL-2-Treg cell axis is indispensable for HSC maintenance and normal hematopoiesis. Lack of Treg activity resulted in increased IFN-γ production by activated T cells and an expansion of the HSCs in the bone marrow (BM)...
March 18, 2017: Oncotarget
https://www.readbyqxmd.com/read/28414293/inherited-gins1-deficiency-underlies-growth-retardation-along-with-neutropenia-and-nk-cell-deficiency
#12
Julien Cottineau, Molly C Kottemann, Francis P Lach, Young-Hoon Kang, Frédéric Vély, Elissa K Deenick, Tomi Lazarov, Laure Gineau, Yi Wang, Andrea Farina, Marie Chansel, Lazaro Lorenzo, Christelle Piperoglou, Cindy S Ma, Patrick Nitschke, Aziz Belkadi, Yuval Itan, Bertrand Boisson, Fabienne Jabot-Hanin, Capucine Picard, Jacinta Bustamante, Céline Eidenschenk, Soraya Boucherit, Nathalie Aladjidi, Didier Lacombe, Pascal Barat, Waseem Qasim, Jane A Hurst, Andrew J Pollard, Holm H Uhlig, Claire Fieschi, Jean Michon, Vladimir P Bermudez, Laurent Abel, Jean-Pierre de Villartay, Frédéric Geissmann, Stuart G Tangye, Jerard Hurwitz, Eric Vivier, Jean-Laurent Casanova, Agata Smogorzewska, Emmanuelle Jouanguy
Inborn errors of DNA repair or replication underlie a variety of clinical phenotypes. We studied 5 patients from 4 kindreds, all of whom displayed intrauterine growth retardation, chronic neutropenia, and NK cell deficiency. Four of the 5 patients also had postnatal growth retardation. The association of neutropenia and NK cell deficiency, which is unusual among primary immunodeficiencies and bone marrow failures, was due to a blockade in the bone marrow and was mildly symptomatic. We discovered compound heterozygous rare mutations in Go-Ichi-Ni-San (GINS) complex subunit 1 (GINS1, also known as PSF1) in the 5 patients...
April 17, 2017: Journal of Clinical Investigation
https://www.readbyqxmd.com/read/28413775/an-interesting-case-of-mycoplasma-pneumonia-associated-multisystem-involvement-and-diffuse-alveolar-hemorrhage
#13
Rashmi Mishra, Edison Cano, Sindhaghatta Venkatram, Gilda Diaz-Fuentes
Severe mycoplasma pneumonia is a rare entity with only 0.5-2% of cases having a fulminant course. We present a 74-year-old woman with hypertension, diabetes mellitus and remote history of marginal zone B-cell lymphoma admitted with abdominal pain and diarrhea of 1-2 days associated with body-aches, dyspnea, dry cough and weight loss for 2-3 weeks. On physical exam, she was febrile, tachypneic, tachycardic and hypoxic on room air. Chest examination revealed diffuse crackles and end-expiratory wheezes. Laboratory tests showed anemia, acute-on-chronic kidney injury and hyaline casts and epithelial cells in the urine analysis...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/28409921/mesenchymal-stem-cell-seeded-regenerated-silk-fibroin-complex-matrices-for-liver-regeneration-in-an-animal-model-of-acute-liver-failure
#14
Lijuan Xu, Shufang Wang, Xiang Sui, Yu Wang, Yinan Su, Li Huang, Yunwei Zhang, Zheng Chen, Qianqian Chen, Haitao Du, Yaopeng Zhang, Li Yan
The main limitation of liver transplantation as a treatment for end-stage liver disease or acute liver failure is the scarcity of liver organ donors. To develop an alternative therapy for acute liver failure, mesenchymal stem cell (MSC)-seeded regenerated silk fibroin (RSF) matrices were evaluated in vitro and in vivo. Adipose-derived mesenchymal stem cells (ADSCs) and bone marrow-derived mesenchymal stem cells (BMSCs) were planted and grown on RSF scaffolds to form a scaffold complex. The RSF-MSC scaffold complex (the experimental group) and neat RSF scaffolds (the control group) were then placed onto the liver surface of mice induced by CCl4 and detected after 5, 7, 14, 28, and 60 days...
April 24, 2017: ACS Applied Materials & Interfaces
https://www.readbyqxmd.com/read/28407835/-recurrent-pulmonary-infection-and-oral-mucosal-ulcer
#15
Fei-Mei Kuang, Lan-Lan Tang, Hui Zhang, Min Xie, Ming-Hua Yang, Liang-Chun Yang, Yan Yu, Li-Zhi Cao
An 8-year-old girl who had experienced intermittent cough and fever over a 3 year period, was admitted after experiencing a recurrence for one month. One year ago the patient experienced a recurrent oral mucosal ulcer. Physical examination showed vitiligo in the skin of the upper right back. Routine blood tests and immune function tests performed in other hospitals had shown normal results. Multiple lung CT scans showed pulmonary infection. The patient had recurrent fever and cough and persistent presence of some lesions after anti-infective therapy...
April 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/28406754/spheroid-coculture-of-hematopoietic-stem-progenitor-cells-and-monolayer-expanded-mesenchymal-stem-stromal-cells-in-polydimethylsiloxane-microwells-modestly-improves-in-vitro-hematopoietic-stem-progenitor-cell-expansion
#16
Kathryn Futrega, Kerry Atkinson, William B Lott, Michael R Doran
While two-dimensional (2D) monolayers of mesenchymal stem/stromal cells (MSCs) have been shown to enhance hematopoietic stem/progenitor cell (HSPC) expansion in vitro, expanded cells do not engraft long term in human recipients. This outcome is attributed to the failure of 2D culture to recapitulate the bone marrow (BM) niche signal milieu. Herein, we evaluated the capacity of a novel three-dimensional (3D) coculture system to support HSPC expansion in vitro. A high-throughput polydimethylsiloxane (PDMS) microwell platform was used to manufacture thousands of uniform 3D multicellular coculture spheroids...
April 2017: Tissue Engineering. Part C, Methods
https://www.readbyqxmd.com/read/28405130/mechanism-underlying-linezolid-induced-thrombocytopenia-in-a-chronic-kidney-failure-mouse-model
#17
Nao Nishijo, Yasuhiro Tsuji, Kazuhisa Matsunaga, Masahiko Kutsukake, Fumiyasu Okazaki, Shiro Fukumori, Hidefumi Kasai, Yoichi Hiraki, Ippei Sakamaki, Yoshihiro Yamamoto, Yoshiharu Karube, Hideto To
OBJECTIVE: To investigate the relationship between renal function and linezolid (LZD)-induced thrombocytopenia and elucidate the underlying mechanism using a chronic renal disease (CRD) mouse model. MATERIALS AND METHODS: CRD was induced in 5-week-old male Institute of Cancer Research (ICR) mice by 5/6 nephrectomy. After this procedure, LZD (25 and 100 mg/kg) was administered intraperitoneally once every day for 28 days. Platelet counts, white blood cell (WBC) counts, and hematocrit (HCT) levels were measured every 7 days...
January 2017: Journal of Pharmacology & Pharmacotherapeutics
https://www.readbyqxmd.com/read/28403691/novel-nlrc4-mutation-causes-a-syndrome-of-perinatal-autoinflammation-with-hemophagocytic-lymphohistiocytosis-hepatosplenomegaly-fetal-thrombotic-vasculopathy-and-congenital-anemia-and-ascites
#18
Jiancong Liang, Danielle N Alfano, James E Squires, Melissa M Riley, W Tony Parks, Julia Kofler, Areeg El-Gharbawy, Suneeta Madan-Kheterpal, Roxanne Acquaro, Jennifer Picarsic
Autoinflammatory diseases are caused by pathologic activation of the innate immune system. Primary hemophagocytic lymphohistiocytosis (HLH) is an aggressive syndrome of excessive immune activation caused by monogenic mutations resulting in cytotoxic cell defects and subsequent failure to eliminate activated macrophages. Secondary HLH is often diagnosed in cases without a known Mendelian inheritance. However, some cases of "secondary" HLH have been shown to harbor mutations with partial dysfunction of the cytotoxic system...
January 1, 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28401593/low-dose-bmp-2-and-msc-dual-delivery-onto-coral-scaffold-for-critical-size-bone-defect-regeneration-in-sheep
#19
Adeline Decambron, Alexandre Fournet, Morad Bensidhoum, Mathieu Manassero, Frédéric Sailhan, Hervé Petite, Delphine Logeart-Avramoglou, Véronique Viateau
Tissue-engineered constructs (TECs) combining resorbable calcium-based scaffolds and mesenchymal stem cells (MSCs) have the capability to regenerate large bone defects. Inconsistent results have, however, been observed, with a lack of osteoinductivity as a possible cause of failure. This study aimed to evaluate the impact of the addition of low-dose bone morphogenetic protein-2 (BMP-2) to MSC-coral-TECs on the healing of clinically-relevant segmental bone defects in sheep. Coral granules were either seeded with autologous MSCs (bone marrow-derived) or loaded with BMP-2...
April 12, 2017: Journal of Orthopaedic Research: Official Publication of the Orthopaedic Research Society
https://www.readbyqxmd.com/read/28399053/short-course-regimens-of-liposomal-amphotericin-b-for-the-treatment-of-mediterranean-visceral-leishmaniasis-in-children-an-11-year-retrospective-study-at-a-tertiary-care-center
#20
Panagiotis Krepis, Ioanna Argyri, Adamantia Krepi, Areti Syrmou, Nikos Spyridis, Maria Tsolia
BACKGROUND: Visceral leishmaniasis (VL) remains an important public health problem in endemic regions. Current anti-leishmanial agents share several limitations including potentially serious side effects and the risk of clinical failure. OBJECTIVES: Aim of the present study was to examine the effectiveness and safety of short-course liposomal amphotericin B (L-AmB) regimens in the treatment of childhood VL in our area. METHODS: The cases of 43 VL patients (20 males, 23 females, mean age 4...
April 10, 2017: Pediatric Infectious Disease Journal
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