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https://www.readbyqxmd.com/read/28644950/hematopoietic-cell-transplantation-in-fanconi-anemia-and-dyskeratosis-congenita-a-minireview
#1
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/28644353/extraintestinal-manifestations-of-celiac-disease-effectiveness-of-the-gluten-free-diet
#2
Hilary Jericho, Naire Sansotta, Stefano Guandalini
OBJECTIVE: The aim of the study was to evaluate the effectiveness of the gluten-free diet (GFD) on extraintestinal symptoms in pediatric and adult celiac populations at the University of Chicago. METHODS: We conducted a retrospective chart review of the University of Chicago Celiac Center clinic charts from January 2002 to October 2014. Demographics, serologic testing, intestinal biopsies, and extraintestinal symptoms at presentation, 12, 24, and >24 months were recorded...
July 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28644307/x-linked-sideroblastic-anemia-in-a-malay-boy-with-alas2-s568g-mutation
#3
Thomas A K Susanto, Rajat Bhattacharyya
Dimorphism in peripheral blood film was noted in a 16 year old Malay boy with anemia who was eventually diagnosed with X-linked sideroblastic anemia. A mutation in ALAS2 S568G was identified which has not been described previously in a Malay ethnic group.
July 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28644162/would-lipophilic-statin-therapy-as-a-prognostic-factor-improve-survival-in-patients-with-uterine-cervical-cancer
#4
Moo-Kon Song, Byoung-Sub Shin, Chung-Sik Ha, Won-Young Park
OBJECTIVES: In vitro studies showed that lipophilic statins inhibit cell growth, adhesion, and invasion and induce apoptosis in cancer cell lines. In uterine cervical cancer, several important factors including age, stage, anemia, lymphovascular invasion, lymph node metastases, and parametrial spread were known to significantly predict survival. We investigated whether statin therapy as a prognostic factor would significantly predict survival in cervical cancer. METHODS: Patients with stages IB to IV cervical cancer who received radical hysterectomy and/or para-aortic lymph node dissection were included...
June 23, 2017: International Journal of Gynecological Cancer
https://www.readbyqxmd.com/read/28644101/safety-and-cost-efficiency-of-a-restrictive-transfusion-protocol-in-patients-with-traumatic-brain-injury
#5
Laura B Ngwenya, Catherine G Suen, Phiroz E Tarapore, Geoffrey T Manley, Michael C Huang
OBJECTIVE Blood loss and moderate anemia are common in patients with traumatic brain injury (TBI). However, despite evidence of the ill effects and expense of the transfusion of packed red blood cells, restrictive transfusion practices have not been universally adopted for patients with TBI. At a Level I trauma center, the authors compared patients with TBI who were managed with a restrictive (target hemoglobin level > 7 g/dl) versus a liberal (target hemoglobin level > 10 g/dl) transfusion protocol. This study evaluated the safety and cost-efficiency of a hospital-wide change to a restrictive transfusion protocol...
June 23, 2017: Journal of Neurosurgery
https://www.readbyqxmd.com/read/28643980/-hiatal-hernia-current-diagnostic-and-therapeutic-management
#6
Pierre Allemann, Valentine Guarnero, Alain Schoepfer, Nicolas Demartines, Markus Schäfer
Hiatal hernia is a frequent pathology. Two types have been described : sliding hernia and paraesophageal hernia (PEH). The first one is the most frequent and is associated with reflux disease. Patients with PEH have a large variety of symptoms, such as chest pain, dyspnea, regurgitation, iterative pneumonia and iron-deficiency anemia. Operative management is necessary for sliding hernia with reflux disease and for symptomatic PEH, respectively. In patients with asymptomatic PEH, operative risk must be balanced with the risk of postoperative complications...
June 14, 2017: Revue Médicale Suisse
https://www.readbyqxmd.com/read/28643539/prevalence-of-iron-deficiency-anemia-and-iron-deficiency-in-a-pediatric-population-with-inflammatory-bowel-disease
#7
Fábia Susana Ginja de Carvalho, Inês Ambrósio de Medeiros, Henedina Antunes
OBJECTIVES: Iron deficiency is the most common cause of anemia in children with inflammatory bowel disease, although the real prevalence is unknown. Intravenous iron is suggested as the first line treatment. This study aims to determine the prevalence of iron deficiency anemia in children with inflammatory bowel disease followed in a Pediatric Gastroenterology Unit of a tertiary center and to evaluate this unit's experience with intravenous iron. MATERIALS AND METHODS: A retrospective cohort study was designed involving children with inflammatory bowel disease followed in that unit between January 2001 and April 2016...
June 23, 2017: Scandinavian Journal of Gastroenterology
https://www.readbyqxmd.com/read/28643177/targeting-dna-repair-and-replication-stress-in-the-treatment-of-ovarian-cancer
#8
REVIEW
Junko Murai
Approximately half of high-grade serous epithelial ovarian cancers incur alterations in genes of homologous recombination (BRCA1, BRCA2, RAD51C, Fanconi anemia genes), and the rest incur alterations in other DNA repair pathways at high frequencies. Such cancer-specific gene alterations can confer selective sensitivity to DNA damaging agents such as cisplatin and carboplatin, topotecan, etoposide, doxorubicin, and gemcitabine. Originally presumed to inhibit DNA repair, PARP inhibitors that have recently been approved by the FDA for the treatment of advanced ovarian cancer also act as DNA damaging agents by inducing PARP-DNA complexes...
June 22, 2017: International Journal of Clinical Oncology
https://www.readbyqxmd.com/read/28642830/a-rare-case-of-esophageal-adenocarcinoma-with-urinary-bladder-metastasis
#9
Heather Katz, Rahoma E Saad, Krista Denning, Toni O Pacioles
Metastatic esophageal adenocarcinoma to the urinary bladder is extremely rare. We describe a previously healthy 49-year-old female with recent diagnosis of adenocarcinoma of the gastroesophageal junction with metastatic disease to the liver. Biopsy was positive for human epidermal growth factor receptor 2 (HER2) by Fluorescence In Situ Hybridization (FISH). She received six cycles of Cisplatin, 5-Fluorouracil, and Herceptin and subsequently developed symptomatic anemia and hematuria. Cystoscopy with retroflexion was performed and she received a transurethral resection of bladder tumor with fulguration...
2017: Case Reports in Gastrointestinal Medicine
https://www.readbyqxmd.com/read/28642774/increasing-provasculature-complexity-in-the-arabidopsis-embryo-may-increase-total-iron-content-in-seeds-a-hypothesis
#10
Hannetz Roschzttardtz, Sofía Bustos, Maria F Coronas, Miguel A Ibeas, Susana Grant-Grant, Joaquín Vargas-Pérez
Anemia due to iron deficiency is a worldwide issue, affecting mainly children and women. Seed iron is a major source of this micronutrient for feeding, however, in most crops these levels are too low to meet daily needs. Thus, increasing iron allocation and its storage in seeds can represent an important step to enhance iron provision for humans and animals. Our knowledge on seed iron homeostasis is mainly based on studies performed in the model plant Arabidopsis thaliana, where iron accumulates in endodermis cells surrounding the embryo provasculature...
2017: Frontiers in Plant Science
https://www.readbyqxmd.com/read/28642072/recombinant-human-thrombopoietin-promotes-platelet-engraftment-and-improves-prognosis-of-patients-with-myelodysplastic-syndromes-and-aplastic-anemia-after-allogeneic-hematopoietic-stem-cell-transplantation
#11
Hong Wang, Man Huang, Ying Zhao, Jia-Qian Qi, Chun Chen, Ya-Qiong Tang, Hui-Ying Qiu, Cheng-Cheng Fu, Xiao-Wen Tang, De-Pei Wu, Chang-Geng Ruan, Yue Han
Poor platelet graft function (PPGF) is a significant complication after allogeneic hematopoietic stem cell transplantation (allo-HSCT). However, no optimal treatment has been recommended. This study investigated aspects of platelet recovery after allo-HSCT, including prognostic value and the effect of recombinant human thrombopoietin (rhTPO). We retrospectively analyzed 275 patients who received allo-HSCT in our center. Of them, 135 (49.1%) patients had good platelet graft function (GPGF) and 140 (50.9%) had PPGF...
June 19, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28641660/-comparison-of-laboratory-indexes-and-therapeutic-efficacy-between-two-kinds-of-different-rbc-transfusion-in-aiha-patients
#12
Jing-Jing Gao, Xiong-Peng Zhu, Ming-Quan Wang
OBJECTIVE: To investigate the effect of 2 kinds of red blood cells (RBC) on the laboratorial indexes and therapeutic efficacy of patients with autoimmune hemolytic anemia (AIHA). METHODS: The clinical data of 120 patients with AIHA from June 2015 to June 2016 were analyzed retrospectively. These 120 patients were divided into A goup and B group. The patients in A group (60 cases) were infused with washed RBC, while the patients in B group (60 cases) were infused with WBC-deplated RBC...
June 2017: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/28641642/-clinical-efficacy-of-cellular-immuotherapy-combined-with-bortezomib-for-the-treatment-of-patients-with-multiple-myeloma
#13
Yi-Ming Wang, Hao Long, Dan Yang, Jiang-He Shao
OBJECTIVE: To study the clinical efficacy of cellular immunotherapy combined with bortezomib for treatment of patients with multiple myeloma. METHODS: A total of 76 patients with multiple myeloma in our hospital from October 2012 to October 2013 were selected and randomly divided into 2 groups: the patients in 1 group (38 cases) were treated with cellular immunotherapy combined with chemotherapy including bortezomib (combined therapy group), the patients in other group(38 cases) were treated with only chemotherapy including bortezomib(single chemotherapy as control group)...
June 2017: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/28641620/-role-of-microparticles-derived-from-acute-promyelocytic-leukemia-cells-in-coagulopathy
#14
Ying-Mei Zhang, Bo Chen, Long-Yue Wu, Jin-Xiao Hou, Jin-Yue Fu
OBJECTIVE: To evaluate the role of microparticle (MP) derived from acute promyelocytic leukemia (APL) cells and tissue factor (TF) carried by the MP in hypercoagulable state, and the effect of treatment with cytotoxic chemotherapy/differentiating agents on procoagulant activity (PCA) of these MP. METHODS: Bone marrow mononuclear cells (BMMNC) were extracted from 5 APL patients and 5 sex- and age- matched patients with iron deficiency anemia as controls. The cells were cultured in vitro for 48 h, then MP-rich culture medium and MP-free culture medium were harvested and MP was further obtained from certain volume of MP-rich culture medium...
June 2017: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/28641096/hematopoietic-stem-cell-transplantation-for-sickle-cell-anemia-the-changing-landscape
#15
REVIEW
Adetola A Kassim, Deva Sharma
Allogeneic hematopoietic stem cell transplantation (HSCT) remains the only curative therapy for sickle cell disease (SCD); however, its use is limited by lack of suitable human leukocyte antigen (HLA)-matched donors and decreased application in older patients with significant morbidity. Myeloablative, HLA-identical sibling transplantation in children with SCD offers excellent long-term survival, with overall and event-free survival rates of 95% and 92%, respectively. However, the risk of graft-versus-host-disease, infections, infertility, and other long-term transplant complications, further limits its widespread use...
June 15, 2017: Hematology/oncology and Stem Cell Therapy
https://www.readbyqxmd.com/read/28640661/attrition-from-antiretroviral-treatment-services-among-pregnant-and-non-pregnant-patients-following-adoption-of-option-b-in-haiti
#16
Jean Wysler Domercant, Nancy Puttkammer, Paul Young, Krista Yuhas, Kesner François, Reynold Grand'Pierre, David Lowrance, Michelle Adler
BACKGROUND: Access to antiretroviral therapy (ART) has expanded in Haiti because of the adoption of Option B+ and the revision of treatment guidelines. Retention in care and treatment varies greatly and few studies have examined retention rates, particularly among women enrolled in Option B+. OBJECTIVE: To assess attrition among pregnant and non-pregnant patients initiating ART following adoption of Option B+ in Haiti. METHODS: Longitudinal data of adult patients initiated on ART from October 2012 through August 2014 at 73 health facilities across Haiti were analyzed using a survival analysis framework to determine levels of attrition...
2017: Global Health Action
https://www.readbyqxmd.com/read/28639991/evaluation-of-a-nutritional-support-intervention-in-malnourished-hiv-infected-children-in-bamako-mali
#17
Julie Jesson, Aba Coulibaly, Mariam Sylla, Clémentine N'diaye, Fatoumata Dicko, David Masson, Valériane Leroy
BACKGROUND: We assessed a nutritional support intervention in malnourished HIV-infected children in a HIV-care program of the University Hospital Gabriel Touré, Bamako, Mali. METHODS: All HIV-infected children <15 years were diagnosed for malnutrition between 07 and 12/2014. Malnutrition was defined according to the WHO growth standards with Z-scores. Two types were studied: acute malnutrition (AM) and chronic malnutrition (CM). All participants were enrolled in a 6-month prospective interventional cohort, receiving Ready-To-Use Therapeutic Food, according to type of malnutrition...
June 19, 2017: Journal of Acquired Immune Deficiency Syndromes: JAIDS
https://www.readbyqxmd.com/read/28639946/assessment-of-left-ventricular-systolic-function-in-patients-with-iron-deficiency-anemia-by-three-dimensional-speckle-tracking-echocardiography
#18
Qiao Zhou, Jiaqi Shen, Yue Liu, Runlan Luo, Bijun Tan, Guangsen Li
OBJECTIVE: The present study aimed to evaluate left ventricular (LV) systolic function in patients with iron deficiency anemia (IDA) by 3 dimensional speckle-tracking echocardiography (3DSTE). METHODS: Participants were grouped by hemoglobin (Hb) levels in order to study the effect of anemia on cardiac function. Group A included 40 healthy volunteers. Eighty-three patients who were diagnosed with IDA were divided into 2 groups according to the Hb level. Group B (Hb 9 g/dL) included 44 patients, while group C (Hb 6-9 g/dL) included 39 patients...
June 22, 2017: Anatolian Journal of Cardiology
https://www.readbyqxmd.com/read/28639730/evaluation-of-thoracic-radiographs-as-a-screening-test-for-dogs-and-cats-admitted-to-a-tertiary-care-veterinary-hospital-for-noncardiopulmonary-disease
#19
Christine L Keyserling, Yekaterina Buriko, Bridget M Lyons, Kenneth J Drobatz, Anthony J Fischetti
Thoracic radiographs are used as a screening tool for dogs and cats with a variety of disorders that have no clinical signs associated with thoracic structures. However, this practice has never been supported by an evidence-based study. The objective of this retrospective observational study was to determine if certain canine and feline populations have a higher proportion of radiographic abnormalities, and whether any of these abnormalities are associated with patient hospitalization and outcome. Patients were excluded if current or previous examinations revealed evidence of primary respiratory or cardiac disease, malignant neoplasia, or an abnormal breathing pattern consistent with pulmonary pathology...
June 22, 2017: Veterinary Radiology & Ultrasound
https://www.readbyqxmd.com/read/28639307/anti-d-in-a-mother-hemizygous-for-the-variant-rhd-dnb-gene-associated-with-hemolytic-disease-of-the-fetus-and-newborn
#20
Kelli M Quantock, Genghis H Lopez, Catherine A Hyland, Yew-Wah Liew, Robert L Flower, Frans J Niemann, Arthur Joyce
BACKGROUND: Individuals with the partial D phenotype when exposed to D+ red blood cells (RBCs) carrying the epitopes they lack may develop anti-D specific for the missing epitopes. DNB is the most common partial D in Caucasians and the clinical significance for anti-D in these individuals is unknown. STUDY DESIGN AND METHODS: This article describes the serologic genotyping results and clinical manifestations in two group D+ babies of a mother presenting as group O, D+ with alloanti-D...
June 21, 2017: Transfusion
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