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Platelet disorder

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https://www.readbyqxmd.com/read/29147753/defective-wnt-signaling-associates-with-bone-marrow-fibrosis-a-cross-sectional-cohort-study-in-a-family-with-wnt1-osteoporosis
#1
R E Mäkitie, R Niinimäki, S Kakko, T Honkanen, P E Kovanen, O Mäkitie
This study explores bone marrow function in patients with defective WNT1 signaling. Bone marrow samples showed increased reticulin and altered granulopoiesis while overall hematopoiesis was normal. Findings did not associate with severity of osteoporosis. These observations provide new insight into the role of WNT signaling in bone marrow homeostasis. INTRODUCTION: WNT signaling regulates bone homeostasis and survival and self-renewal of hematopoietic stem cells. Aberrant activation may lead to osteoporosis and bone marrow pathology...
November 16, 2017: Osteoporosis International
https://www.readbyqxmd.com/read/29145678/-thrombocytosis-and-thrombocytopenia-background-and-clinical-relevance
#2
Kai Wille, Parvis Sadjadian, Martin Griesshammer
Due to the central role of platelets in hemostasis, the clinical relevance of quantitative changes in platelet counts (< 150 G/l or > 450 G/l) may be significant. Thrombopoesis (= production of platelets) occurs in the bone marrow, and the hormone thrombopoetin takes control on its regulation.In thrombocytosis, primary causes have to be distinguished from the far more common reactive (= secondary) reasons. The most important form of primary thrombocytosis occurs in myeloproliferative neoplasms especially in essential thrombocythemia (ET)...
November 2017: Deutsche Medizinische Wochenschrift
https://www.readbyqxmd.com/read/29144585/therapeutic-potential-of-low-dose-il-2-in-immune-thrombocytopenia-an-analysis-of-3-cases
#3
Jiakui Zhang, Yanjie Ruan, Xuanxuan Xu, Huiping Wang, Qianshan Tao, Jun Lu, Linhuan Xia, Qiuye Zhang, Jeffrey Wang, Yiping Wang, Zhimin Zhai
Immune thrombocytopenia (ITP) is an acquired immune-mediated disorder with regulatory T cells (Tregs) reduction. Recent studies have shown that low-dose interleukin-2 can preferentially induce regulatory T cell expansion in vivo, and therefore offers a therapeutic strategy against immune thrombocytopenia. We have demonstrated in a previous study that Tregs and platelet counts significantly improve in an adult with ITP following low-dose IL-2 treatment. Here we report the efficacy of low-dose interleukin-2 in another three adults with immune thrombocytopenia who failed the first-line treatment...
November 16, 2017: Cytometry. Part B, Clinical Cytometry
https://www.readbyqxmd.com/read/29144506/soluble-p-selectin-promotes-retinal-ganglion-cell-survival-through-activation-of-nrf2-signaling-after-ischemia-injury
#4
Kishan Kapupara, Yao-Tseng Wen, Rong-Kung Tsai, Shun-Ping Huang
Retinal ischemic injuries play an important role in the pathogenesis of several eye disorders. Inflammation and oxidative stress are key players in ischemic injuries. Following retinal ischemia, vascular endothelial cells and leukocytes express several inflammatory adhesion receptors, such as selectins and cell adhesion molecules. P-selectin stimulates leukocyte recruitment to platelet aggregates and has an important role in vascular homeostasis and inflammatory leukocyte extravasation. Soluble P-selectin can be neuroprotective through competitive binding to the receptors of endogenous P-selectin molecules...
November 16, 2017: Cell Death & Disease
https://www.readbyqxmd.com/read/29143464/mean-platelet-diameter-measurements-to-classify-inherited-thrombocytopenias
#5
K Fixter, D J Rabbolini, B Valecha, M-C Morel-Kopp, S Gabrielli, Q Chen, W S Stevenson, C M Ward
INTRODUCTION: Mean platelet volume (MPV) assists the differential diagnosis of inherited thrombocytopenia (IT) but lacks standardisation and varies between automated analysers. Classification of IT based on mean platelet diameter (MPD) has been proposed by an international collaborative study but has not been validated. METHODS: To assess the applicability of MPD to classify forms of IT, digital images of blood films from patients with established genetic causes for IT were generated, and the MPD measured (ZEISS Axio-scanner and Image J software) by a blinded reviewer...
November 16, 2017: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/29141318/-ras-associated-autoimmune-leukoproliferative-disorder-a-report-of-2-cases-and-literature-review
#6
T Y He, C R Li, Y Xia, F F Liang, Y Luo, J Yang
Objective: To investigate the clinical features and genetic characteristics of cases with Ras-associated autoimmune leukoproliferative disorder(RALD). Method: Characteristics of clinical data and gene mutation of the first two cases in China with RALD were retrospectively analyzed. The related literature was searched by using search terms "NRAS" , "KRAS" or "RALD" . Result: Case1, a seven-year-seven-month old girl, was admitted due to "thrombocytopenia and splenomegaly for three years" . Palpation showed enlargement of submandibular lymph nodes and hepatosplenomegaly...
November 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/29136727/-the-459th-case-arthralgia-fever-rash-and-thrombocytopenia
#7
J Z Shen, Y Wang, M Y Fang
The differential diagnoses of reactive arthritis presenting as arthralgia should be considered as diverse disorders, especially when the symptoms cannot be fully explained by some definite diseases. Do not ignore the indication of bone marrow aspiration. We reported a 50-year-old woman who complained of arthralgia, recurrent fever and rash 9 months ago. Laboratory exams showed mild leukopenia, anemia, thrombocytopenia and increased lymphocyte proportion. She was treated with glucocorticoid after the diagnosis of connective tissue disease was suspected...
November 1, 2017: Zhonghua Nei Ke za Zhi [Chinese Journal of Internal Medicine]
https://www.readbyqxmd.com/read/29135309/screening-for-platelet-function-disorders-with-multiplate-and-platelet-function-analyzer
#8
Floor C J I Moenen, Minka J A Vries, Patricia J Nelemans, Katrien J M van Rooy, Jeannique R R A Vranken, Paul W M Verhezen, Rick J H Wetzels, Hugo Ten Cate, Harry C Schouten, Erik A M Beckers, Yvonne M C Henskens
Light transmission aggregation (LTA) is the gold standard for the diagnosis of platelet function disorders (PFDs), but it is time-consuming and limited to specialized laboratories. Whole-blood impedance aggregometry (Multiplate) and platelet function analyzer (PFA) may be used as rapid screening tools to exclude PFDs. The aim of this study is to assess the diagnostic performance of Multiplate and PFA for PFDs, as detected by LTA.Data from preoperative patients, patients referred to the hematologist for bleeding evaluation, and patients with a diagnosed bleeding disorder were used...
November 14, 2017: Platelets
https://www.readbyqxmd.com/read/29134118/an-overview-of-platelet-products-prp-prgf-prf-etc-in-the-iranian-studies
#9
REVIEW
Seyed Ahmad Raeissadat, Marzieh Babaee, Seyed Mansour Rayegani, Zahra Hashemi, Amir Ali Hamidieh, Parviz Mojgani, Hossein Fouladi Vanda
Aim: The aim of the study was to carry out a review of published studies on various platelet products in Iranian studies. Materials & methods: Electronic databases were searched for relevant articles. Two review authors independently extracted data via a tested extraction sheet, and disagreements were resolved by a meeting with a third review author. Results: Bone disorders (25%), wound and fistula (16%), dental and gingival disorders (14%) and osteoarthritis (11%) have more relative frequency based on different fields...
November 2017: Future Science OA
https://www.readbyqxmd.com/read/29130141/sj%C3%A3-gren-s-syndrome-initially-presented-as-thrombotic-thrombocytopenic-purpura-in-a-male-patient-a-case-report-and-literature-review
#10
REVIEW
Xiaohan Xu, Tienan Zhu, Di Wu, Lu Zhang
Thrombotic thrombocytopenic purpura (TTP) is a potentially lethal multisystem disorder which could be caused by autoimmune diseases. However, the concomitant occurrence of TTP and Sjögren's syndrome (SS) is an extremely uncommon scenario, especially in male patients. A 56-year-old Chinese male was admitted for the appearance of diffuse ecchymosis. Then he gradually developed transient slurred speech, progressive confusion, agitation, extremity weakness, and fever. Laboratory investigations suggested anemia, thrombocytopenia, significantly increased lactic dehydrogenase, schistocytes in peripheral blood smear, and a disintegrin-like metalloproteinase with thrombospondin motif type 1 member 13 (ADAMTS13) activity deficiency with high inhibitor titers...
November 13, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/29122692/blockade-of-platelet-activating-factor-receptor-attenuates-abnormal-behaviors-induced-by-phencyclidine-in-mice-through-down-regulation-of-nf-%C3%AE%C2%BAb
#11
The-Vinh Tran, Se Jin Park, Eun-Joo Shin, Hai-Quyen Tran, Ji Hoon Jeong, Choon-Gon Jang, Yu Jeung Lee, Seung-Yeol Nah, Toshitaka Nabeshima, Hyoung-Chun Kim
Accumulating evidence suggests that neuroinflammation is one of the important etiologic factors of abusive and neuropsychiatric disorders. Platelet-activating factor (PAF) is potent proinflammatory lipid mediat1or and plays a pivotal role in neuroinflammatory disorders through the specific PAF receptor (PAF-R). Phencyclidine (PCP) induces a psychotomimetic state that closely resembles schizophrenia. Here, we investigated the role of PAF-R in the abnormal behaviors induced by PCP in mice. Repeated treatment with PCP resulted in a significant increase in PAF-R gene expression in the prefrontal cortex (PFC) and in the hippocampus...
November 6, 2017: Brain Research Bulletin
https://www.readbyqxmd.com/read/29122152/biologic-agents-for-the-treatment-of-hypereosinophilic-syndromes
#12
Fei Li Kuang, Amy D Klion
Hypereosinophilic syndromes (HES) are a heterogeneous group of rare disorders defined by the presence of marked peripheral or tissue eosinophilia resulting in end-organ damage. Although conventional therapies, including glucocorticoids, hydroxyurea, and IFN-α, are initially effective in reducing eosinophilia and symptoms in a majority of patients with platelet-derived growth factor mutation-negative HES, the development of resistance and treatment-related toxicity are common. In contrast, targeted therapy with the tyrosine kinase inhibitor, imatinib, is well tolerated but effective only in the subset of patients with HES with a primary myeloid disorder...
November 2017: Journal of Allergy and Clinical Immunology in Practice
https://www.readbyqxmd.com/read/29117459/receptor-homodimerization-plays-a-critical-role-in-a-novel-dominant-negative-p2ry12-variant-identified-in-a-family-with-severe-bleeding
#13
Stuart J Mundell, David Rabbolini, Sara Gabrielli, Qiang Chen, Riyaad Aungraheeta, James L Hutchinson, Tatjana Kilo, Joel Mackay, Christopher M Ward, William Stevenson, Marie-Christine Morel-Kopp
BACKGROUND: Although inherited platelet disorders are still underdiagnosed worldwide, advances in molecular techniques are improving disease diagnosis and patient management. OBJECTIVE: To identify and characterize the mechanism underlying the bleeding phenotype in a Caucasian family with an autosomal dominant P2RY12 variant. METHODS: Full blood count, platelet aggregometry, flow cytometry and western-blotting were performed before NGS. Detailed molecular analysis of the identified P2Y12R receptor's variant was subsequently performed in mammalian cells over-expressing receptor constructs...
November 8, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/29114092/inhibition-of-cd147-cluster-of-differentiation-147-ameliorates-acute-ischemic-stroke-in-mice-by-reducing-thromboinflammation
#14
Rong Jin, Adam Y Xiao, Rui Chen, D Neil Granger, Guohong Li
BACKGROUND AND PURPOSE: Inflammation and thrombosis currently are recognized as critical contributors to the pathogenesis of ischemic stroke. CD147 (cluster of differentiation 147), also known as extracellular matrix metalloproteinase inducer, can function as a key mediator of inflammatory and immune responses. CD147 expression is increased in the brain after cerebral ischemia, but its role in the pathogenesis of ischemic stroke remains unknown. In this study, we show that CD147 acts as a key player in ischemic stroke by driving thrombotic and inflammatory responses...
November 7, 2017: Stroke; a Journal of Cerebral Circulation
https://www.readbyqxmd.com/read/29111429/megakaryocyte-and-polyploidization
#15
REVIEW
Stefania Mazzi, Larissa Lordier, Najet Debili, Hana Raslova, William Vainchenker
In mammals, platelets are produced in the blood by cytoplasmic fragmentation of megakaryocytes (MKs). Platelet production is thus dependent on both the MK number and size. During differentiation, MKs switch from a division by mitosis to polyploidization by endomitosis to increase their size. The endomitotic process includes several successive rounds of DNA replication with an entry in mitosis with a failure in late cytokinesis and a defect in karyokinesis. This leads to a giant cell with a modal ploidy at 16N and one multilobulated nucleus...
October 27, 2017: Experimental Hematology
https://www.readbyqxmd.com/read/29109595/the-role-of-hematological-indices-in-patients-with-acute-coronary-syndrome
#16
REVIEW
Jan Budzianowski, Konrad Pieszko, Paweł Burchardt, Janusz Rzeźniczak, Jarosław Hiczkiewicz
An increased systemic and local inflammation plays a key role in the pathophysiology of acute coronary syndrome (ACS). This review will discuss the role of hematological indices: white blood cells (WBC), neutrophil to lymphocyte ratio (NLR), red cell distribution width (RDW), and platelet indices, that is, platelet to lymphocyte ratio (PLR), mean platelet volume (MPV), and platelet distribution width (PDW) in the case of ACS. In recent years, a strong interest has been drawn to these indices, given that they may provide independent information on pathophysiology, risk stratification, and optimal management...
2017: Disease Markers
https://www.readbyqxmd.com/read/29108898/thymidine-phosphorylase-a-potential-new-target-for-treating-cardiovascular-disease
#17
REVIEW
Wei Li, Hong Yue
We recently found that thymidine phosphorylase (TYMP), also known as platelet-derived endothelial cell growth factor, plays an important role in platelet activation in vitro and thrombosis in vivo by participating in multiple signaling pathways. Platelets are a major source of TYMP. Since platelet-mediated clot formation is a key event in several fatal diseases, such as myocardial infarction, stroke and pulmonary embolism, understanding TYMP in depth may lead to uncovering novel mechanisms in the development of cardiovascular diseases...
October 20, 2017: Trends in Cardiovascular Medicine
https://www.readbyqxmd.com/read/29108448/the-relationship-between-neutrophil-lymphocyte-platelet-lymphocyte-ratio-and-cognitive-functions-in-bipolar-disorder
#18
Demet Sağlam Aykut, Filiz Civil Arslan, Evrim Özkorumak Karagüzel, Gizem Aral, Serdar Karakullukçu
OBJECTIVE: Inflammation is an important factor in pathophysiology of bipolar disorder. Neutrophil-lymphocyte ratio (NLR) and platelet-lymphocyte ratio (PLR) analysis are used to predict peripheral inflammation. The aim of this study is to calculate neutrophil-lymphocyte and platelet-lymphocyte ratios, which are inflammatory markers, and investigate their effect on cognitive functions in euthymic patients with objective bipolar disorder. METHOD: Twenty - eight patients with type-I bipolar disorder and 22 healthy controls matched for age, gender and educational status were included in the study...
November 6, 2017: Nordic Journal of Psychiatry
https://www.readbyqxmd.com/read/29108103/adamts13-and-von-willebrand-factor-a-dynamic-duo
#19
REVIEW
Kieron South, David A Lane
VWF is a key player in haemostasis, acting as a carrier for FVIII and capturing platelets at sites of vascular damage. To capture platelets, it must undergo conformational changes, both within its A1 domain and at the macromolecular level through A2 domain unfolding. Its size and this function are regulated by the metalloproteinase, ADAMTS13. Recently, it has been shown that ADAMTS13 undergoes a conformational change upon interaction with VWF and that this enhances its activity towards its substrate. This review summarises recent work on these conformational transitions, describing how they are controlled...
November 6, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/29103820/an-overview-of-the-biology-of-platelet-rich-plasma-and-microneedling-as-potential-treatments-for-alopecia-areata
#20
REVIEW
Lauren C Strazzulla, Lorena Avila, Kristen Lo Sicco, Jerry Shapiro
Platelet-rich plasma and microneedling have been investigated recently as potential therapeutic options for the treatment of hair disorders. Evidence from laboratory studies indicates that these treatments enhance growth factor production that in turn facilitates hair follicle development and cycling. Several small studies and case reports have presented encouraging findings regarding the use of these treatments for alopecia areata. Future investigations will be needed to validate these therapeutic techniques for patients with alopecia areata and further refine which subtypes of the disease these methods are best indicated for...
November 3, 2017: Journal of Investigative Dermatology
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