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https://www.readbyqxmd.com/read/28646906/modulation-of-oxidative-phosphorylation-and-redox-homeostasis-in-mitochondrial-ndufs4-deficiency-via-mesenchymal-stem-cells
#1
Marlen Melcher, Katharina Danhauser, Annette Seibt, Özer Degistirici, Fabian Baertling, Arun Kumar Kondadi, Andreas S Reichert, Werner J H Koopman, Peter H G M Willems, Richard J Rodenburg, Ertan Mayatepek, Roland Meisel, Felix Distelmaier
BACKGROUND: Disorders of the oxidative phosphorylation (OXPHOS) system represent a large group among the inborn errors of metabolism. The most frequently observed biochemical defect is isolated deficiency of mitochondrial complex I (CI). No effective treatment strategies for CI deficiency are so far available. The purpose of this study was to investigate whether and how mesenchymal stem cells (MSCs) are able to modulate metabolic function in fibroblast cell models of CI deficiency. METHODS: We used human and murine fibroblasts with a defect in the nuclear DNA encoded NDUFS4 subunit of CI...
June 24, 2017: Stem Cell Research & Therapy
https://www.readbyqxmd.com/read/28646532/acquired-bilateral-telangiectasia-macularis-eruptiva-perstans-a-unique-clinical-feature-of-photodamaging-rather-than-a-subtype-of-cutaneous-mastocytosis
#2
Hye-Rim Moon, Young Jae Kim, Joon Min Jung, Chong Hyun Won, Mi Woo Lee, Jee Ho Choi, Sung Eun Chang
Telangiectasia macularis eruptiva perstans (TMEP) is a rare subtype of cutaneous mastocytosis, characterized by telangiectatic tan to brown macules on the trunk and extremities. Although TMEP has been descried as an uncommon disease in the literature, we often encounter patients with TMEP lesions in the outpatient clinic. We aimed to assess the clinical and histopathological characteristics of acquired bilateral TMEP, and the pathophysiological mechanism of acquired bilateral TMEP among these patients. We retrospectively reviewed 30 patients (28 men and 2 women) with acquired bilateral TMEP; multiple telangiectatic dark red to brown macules that were symmetrically distributed...
June 23, 2017: Journal of Dermatology
https://www.readbyqxmd.com/read/28644950/hematopoietic-cell-transplantation-in-fanconi-anemia-and-dyskeratosis-congenita-a-minireview
#3
REVIEW
Mouhab Ayas
Bone marrow failure syndrome is an epithet of bone marrow failure (all or single-cell lineage) that is attributable to an underlying genetic aberration usually with a constellation of somatic abnormalities. Multiple inheritance patterns have been described in these disorders; many are transmitted in an autosomal recessive pattern, which may consequently lead to a higher prevalence of such illnesses in homogeneous societies such as Saudi Arabia, where consanguineous marriages are not uncommon. At King Faisal Specialist Hospital and Research Center, the most common entity referred for allogeneic hematopoietic cell transplantation (HCT) is Fanconi anemia, followed by pure red aplasia, and, less commonly, dyskeratosis congenita, congenital neutropenia, and others...
June 15, 2017: Hematology/oncology and Stem Cell Therapy
https://www.readbyqxmd.com/read/28641093/hematological-parameters-and-red-blood-cell-morphological-abnormality-of-glucose-6-phosphate-dehydrogenase-deficiency-co-inherited-with-thalassemia
#4
Jutharat Pengon, Saovaros Svasti, Sumalee Kamchonwongpaisan, Phantip Vattanaviboon
OBJECTIVE/BACKGROUND: Glucose-6-phosphate dehydrogenase (G-6-PD) deficiency and thalassemia are genetically independent hemolytic disorders. Co-inheritance of both disorders may affect red blood cell pathology to a greater extent than normally seen in either disorder alone. This study determines the prevalence and evaluates hematological changes of G-6-PD deficiency and thalassemia co-inheritance. METHODS: G-6-PD deficiency was screened from 200 male thalassemia blood samples using a fluorescent spot test...
June 15, 2017: Hematology/oncology and Stem Cell Therapy
https://www.readbyqxmd.com/read/28637614/classical-inherited-bone-marrow-failure-syndromes-with-high-risk-for-myelodysplastic-syndrome-and-acute-myelogenous-leukemia
#5
REVIEW
Sharon A Savage, Carlo Dufour
The inherited marrow failure syndromes (IBMFS) are a heterogeneous group of diseases characterized by failure in the production of one or more blood lineage. The clinical manifestations of the IBMFS vary according to the type and number of blood cell lines involved, including different combinations of anemia, leukopenia, and thrombocytopenia. In some IBMFS, systemic non-hematologic manifestations, including congenital malformations, mucocutaneous abnormalities, developmental delay, and other medical complications, may be present...
April 2017: Seminars in Hematology
https://www.readbyqxmd.com/read/28637327/effect-of-simvastatin-on-baboon-endometriosis
#6
Hugh S Taylor, Myles Alderman, Thomas M D'Hooghe, Asgerally T Fazleabas, Duleba J Antoni
Endometriosis, a common disorder affecting women of reproductive age is characterized by ectopic growth of the endometrial tissues, altered steroid hormone response and inflammation. Previous studies revealed that statins, selective inhibitors of the key step of mevalonate pathway, inhibit growth of endometrial stromal cells in vitro and reduce endometriotic lesions in murine models of endometriosis. This study evaluated the effects of simvastatin on the development of endometriosis in a baboon model of this disease...
June 16, 2017: Biology of Reproduction
https://www.readbyqxmd.com/read/28632811/management-of-poorly-controlled-indolent-systemic-mastocytosis-using-narrowband-uvb-phototherapy
#7
Zain Husain, Dylan Waterman, Kathleen Ellison, Jennifer A DeSimone
The mastocytoses comprise a group of proliferative stem cell disorders defined by the abnormal accumulation of mast cells (MCs) in the skin or other body tissues including the bone marrow, gastrointestinal tract, and liver. Systemic mastocytosis is defined by the presence of one major and one minor criterion or 3 minor criteria delineated by the World Health Organization (WHO). We present the case of a 57-year-old woman with a 10-year history of red-brown pruritic maculopapular lesions on the upper and lower extremities and trunk who was originally diagnosed with cutaneous mastocytosis...
May 2017: Cutis; Cutaneous Medicine for the Practitioner
https://www.readbyqxmd.com/read/28630094/type-i-ifn-is-necessary-and-sufficient-for-inflammation-induced-red-blood-cell-alloimmunization-in-mice
#8
David R Gibb, Jingchun Liu, Prabitha Natarajan, Manjula Santhanakrishnan, David J Madrid, Stephanie C Eisenbarth, James C Zimring, Akiko Iwasaki, Jeanne E Hendrickson
During RBC transfusion, production of alloantibodies against RBC non-ABO Ags can cause hemolytic transfusion reactions and limit availability of compatible blood products, resulting in anemia-associated morbidity and mortality. Multiple studies have established that certain inflammatory disorders and inflammatory stimuli promote alloimmune responses to RBC Ags. However, the molecular mechanisms underlying these findings are poorly understood. Type I IFNs (IFN-α/β) are induced in inflammatory conditions associated with increased alloimmunization...
June 19, 2017: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/28627345/-effects-of-microrna-21-inhibitor-on-apoptosis-of-type-ii-alveolar-epithelial-cells-in-rats-with-hyperoxia-induced-acute-lung-injury
#9
Lei Shi, Ying He, Bing Bai, Miao Chen
OBJECTIVE: To observe the effects of microRNA-21 (miR-21) inhibitor on apoptosis of type II alveolar epithelial cells (AEC II) in rats with hyperoxia-induced acute lung injury (HALI). METHODS: Eighty Sprague-Dawley (SD) rats were divided into air-control group, hyperoxia injury group, empty-virus control group (200 μL solution with lentivirus was dropped into the nasal) and miR-21 inhibitor pretreatment group (200 μL solution with lentivirus contained miR-21 inhibitor was dropped through the nasal) by random number table...
March 2017: Zhonghua Wei Zhong Bing Ji Jiu Yi Xue
https://www.readbyqxmd.com/read/28626156/a-method-to-establish-a-mouse-model-of-bone-marrow-microenvironment-injury
#10
Wenzhe Cheng, Quanhu Ge, Longfei Wan, Xiaoyi Wang, Xueling Chen, Xiangwei Wu
A normal bone marrow microenvironment plays a very important role in the normal functioning of hematopoietic stem cells. Once disturbed, this microenvironment can become favorable for the occurrence of blood disorders, cancers, and other diseases. Therefore, further studies on the bone marrow microenvironment should be performed to reveal regulatory and stem cell fate determination mechanisms and promote the development of bone marrow transplantation, tissue repair and regenerative medicine, and other fields...
June 13, 2017: Experimental Animals
https://www.readbyqxmd.com/read/28624906/pathogen-reduction-inactivation-of-products-for-the-treatment-of-bleeding-disorders-what-are-the-processes-and-what-should-we-say-to-patients
#11
Giovanni Di Minno, David Navarro, Carlo Federico Perno, Mariana Canaro, Lutz Gürtler, James W Ironside, Hermann Eichler, Andreas Tiede
Patients with blood disorders (including leukaemia, platelet function disorders and coagulation factor deficiencies) or acute bleeding receive blood-derived products, such as red blood cells, platelet concentrates and plasma-derived products. Although the risk of pathogen contamination of blood products has fallen considerably over the past three decades, contamination is still a topic of concern. In order to counsel patients and obtain informed consent before transfusion, physicians are required to keep up to date with current knowledge on residual risk of pathogen transmission and methods of pathogen removal/inactivation...
June 18, 2017: Annals of Hematology
https://www.readbyqxmd.com/read/28622016/endoplasmic-reticulum-stress-is-involved-in-nucleus-pulposus-degeneration-and-attenuates-low-ph-induced-apoptosis-of-rat-nucleus-pulposus-cells
#12
Zhi-Yang Xie, Lu Chen, Feng Wang, Lei Liu, Cong Zhang, Kun Wang, Feng Cai, Arjun Sinkemanni, Xin Hong, Xiao-Tao Wu
The microenvironment of degenerative intervertebral disk (IVD) is characteristic of a high concentration of lactic acid and low pH levels, whereas the underlying mechanism has not been clearly defined. Endoplasmic reticulum (ER) is the hub of interactions between environmental signals and cellular biological functions, the malfunction of which is closely involved in the pathogenesis of multiple disorders, including IVD degeneration (IVDD). This research mainly aims at exploring what role ER stress plays in the natural process of IVDD and pH-induced apoptosis of rat nucleus pulposus (NP) cells (NPCs)...
June 16, 2017: DNA and Cell Biology
https://www.readbyqxmd.com/read/28610635/cellular-function-reinstitution-of-offspring-red-blood-cells-cloned-from-the-sickle-cell-disease-patient-blood-post-crispr-genome-editing
#13
Jianguo Wen, Wenjing Tao, Suyang Hao, Youli Zu
BACKGROUND: Sickle cell disease (SCD) is a disorder of red blood cells (RBCs) expressing abnormal hemoglobin-S (HbS) due to genetic inheritance of homologous HbS gene. However, people with the sickle cell trait (SCT) carry a single allele of HbS and do not usually suffer from SCD symptoms, thus providing a rationale to treat SCD. METHODS: To validate gene therapy potential, hematopoietic stem cells were isolated from the SCD patient blood and treated with CRISPR/Cas9 approach...
June 13, 2017: Journal of Hematology & Oncology
https://www.readbyqxmd.com/read/28606401/monoclonal-gammopathy-with-both-nemaline-myopathy-and-amyloid-myopathy
#14
Min Wang, Lin Lei, Hai Chen, Li Di, Mi Pang, Yan Lu, Lu Lu, Xin-Ming Shen, Yuwei Da
Monoclonal gammopathies due to plasma cell dyscrasias can induce diverse rare neuromuscular disorders. Deposition of monoclonal antibody light chains in skeletal muscle causes amyloid myopathy. Monoclonal gammopathy is occasionally associated with sporadic late-onset nemaline myopathy. Here we report a monoclonal gammopathy patient with both sporadic late-onset nemaline myopathy and amyloid myopathy. The diagnoses were based on immunofixation electrophoresis of urine, and serum for free light chain assay, Congo red staining and Thioflavin S staining of muscle biopsies, as well as immunohistochemical staining and electron-microscopic observation...
May 11, 2017: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/28598573/successful-treatment-of-tacrolimus-related-pure-red-cell-aplasia-and-autoimmune-hemolytic-anemia-with-rituximab-in-a-pediatric-cardiac-transplant-patient
#15
Chenue Abongwa, Ghada Abusin, Ayman El-Sheikh
Acquired pure red cell aplasia (PRCA) and autoimmune hemolytic anemia (AIHA) are rare complications of immunosuppression in pediatric solid organ transplant patients. We report a 14-month-old female child who developed Coombs positive hemolytic anemia and reticulocytopenia while on tacrolimus after cardiac transplantation. She was successfully treated with rituximab after failing treatment with corticosteroids and intravenous immunoglobulins. Clinicians should consider PRCA differential diagnosis in a patient presenting with reticulocytopenia and hemolysis...
June 9, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28592631/why-measure-carbohydrate-deficient-transferrin
#16
(no author information available yet)
The acute or chronic harmful use of alcohol is among the top five risk factors for disease, disability and death worldwide.1 Monitoring alcohol consumption is an important aspect of the management of alcohol-use disorders and may include subjective self-reported questionnaires or objective tests.1,2-6 Such tests include measuring alcohol or alcohol metabolite levels or biomarkers such as liver enzymes or red cell indices.2-6 Carbohydrate deficient transferrin (CDT) is a biomarker used for assessing chronic alcohol misuse...
June 2017: Drug and Therapeutics Bulletin
https://www.readbyqxmd.com/read/28584708/analyses-of-antioxidant-status-and-nucleotide-alterations-in-genes-encoding-antioxidant-enzymes-in-patients-with-benign-and-malignant-thyroid-disorders
#17
Nur Siti Fatimah Ramli, Sarni Mat Junit, Ng Khoon Leong, Nurhanani Razali, Jaime Jacqueline Jayapalan, Azlina Abdul Aziz
BACKGROUND: Synthesis of thyroid hormones and regulation of their metabolism involve free radicals that may affect redox balance in the body. Thyroid disorders causing variations in the levels of thyroid hormones may alter cellular oxidative stress. The aim of this study was to measure the antioxidant activities and biomarkers of oxidative stress in serum and red blood cells (RBC) of patients with benign and malignant thyroid disorders and to investigate if changes in the antioxidant activities in these patients were linked to alterations in genes encoding the antioxidant enzymes...
2017: PeerJ
https://www.readbyqxmd.com/read/28574201/standardized-high-sensitivity-flow-cytometry-testing-for-paroxysmal-nocturnal-hemoglobinuria-in-children-with-acquired-bone-marrow-failure-disorders-a-single-center-u-s-study
#18
Rachel E Donohue, Andrea N Marcogliese, Ghadir S Sasa, M Tarek Elghetany, Alka A Redkar, Alison A Bertuch, Choladda V Curry
BACKGROUND: Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired hematopoietic stem cell disorder that has not been well documented in children, particularly those with acquired bone marrow failure disorders (ABMFD) - acquired aplastic anemia (AAA) and myelodysplastic syndrome (MDS). Therefore, we sought to determine the prevalence of PNH populations in children with ABMFD. METHODS: PNH testing was performed in children with an ABMFD diagnosis using high sensitivity (≥0...
June 2, 2017: Cytometry. Part B, Clinical Cytometry
https://www.readbyqxmd.com/read/28572880/effect-of-medicinal-mushrooms-on-blood-cells-under-conditions-of-diabetes-mellitus
#19
REVIEW
Taras Vitak, Borys Yurkiv, Solomon Wasser, Eviatar Nevo, Natalia Sybirna
Diabetes mellitus (DM) is the third most common non-infectious disease leading to early disability and high mortality. Moreover, the number of patients is growing every year. The main symptom of DM is hyperglycemia. Increased levels of blood glucose activate polyol, hexosamine, and protein kinase metabolic pathways cause the intensification of non-enzymatic glycosylation and nitration of macromolecules. This, in turn, leads to the development of oxidative and nitrative stresses and secondary complications, such as different kinds of micro- and macroangiopathies...
May 15, 2017: World Journal of Diabetes
https://www.readbyqxmd.com/read/28559914/changes-in-cerebral-oxygenation-associated-with-intradialytic-blood-transfusion-in-patients-with-severe-anemia-undergoing-hemodialysis
#20
Kiyonori Ito, Susumu Ookawara, Yuichiro Ueda, Haruhisa Miyazawa, Masaya Kofuji, Hideyuki Hayasaka, Takayuki Uchida, Katsunori Yanai, Hiroki Ishii, Mitsutoshi Shindo, Taisuke Kitano, Keiji Hirai, Yoshio Kaku, Taro Hoshino, Kaoru Tabei, Yoshiyuki Morishita
BACKGROUND: Hemodialysis (HD) patients frequently suffer from severe anemia caused by various hemorrhagic disorders in addition to renal anemia. Intradialytic blood transfusion is sometimes performed; however, the cerebral oxygenation changes associated with this procedure remain unclear. METHODS: Sixteen HD patients with severe anemia who required intradialytic blood transfusion were included (12 men and 4 women; mean age, 64.8 ± 9.8 years). Cerebral regional oxygen saturation (rSO2) was monitored using near-infrared spectroscopy, and cerebral fractional oxygen extraction (FOE) was calculated before and after HD...
January 2017: Nephron Extra
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