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Yingqi Shao, Sizhou Feng, Jinbo Huang, Jiali Huo, Yahong You, Yizhou Zheng
BACKGROUND: Dyskeratosis congenita (DC) is an inherited telomeropathy characterized by mucocutaneous dysplasia, bone marrow failure, cancer predisposition, and other somatic abnormalities. Cells from patients with DC exhibit short telomere. The genetic basis of the majority of DC cases remains unknown. METHODS: A 2 generational Chinese Han family with DC was studied using targeted capture and next-generation sequencing to identify the underlying DC-related mutations...
March 7, 2018: BMC Medical Genetics
Caleb K Y Yip, Sumit Bansal, Siew Ying Wong, Aik Jiang Lau
Galeterone and abiraterone acetate are anti-androgens developed for the treatment of metastatic castration-resistant prostate cancer. In the present study, we investigated the effect of these drugs on dehydroepiandrosterone (DHEA) sulfonation catalyzed by human liver and intestinal cytosols and human recombinant sulfotransferase enzymes (SULT2A1, SULT2B1b, and SULT2E1), and compared their effects to those of other anti-androgens (cyproterone acetate, spironolactone, and danazol). Each of these chemicals (10 μM) inhibited DHEA sulfonation catalyzed by human liver and intestinal cytosols...
February 7, 2018: Drug Metabolism and Disposition: the Biological Fate of Chemicals
Harshit Khurana, Pankaj Malhotra, Man Updesh Sachdeva, Neelam Varma, Parveen Bose, Uday Yanamandra, Subhash Varma, Alka Khadwal, Deepesh Lad, Gaurav Prakash
OBJECTIVES: Danazol is an attenuated androgen and is used in the treatment of aplastic anemia (AA) in resource constraint settings. We chose to study the role of CD4+ CD25high CD127low FoxP3+ T regulatory cells (T-regs) in the pathophysiology of AA and their response to treatment with Danazol alone or in combination with immunosuppressive treatment (IST). METHODS: T-regs' percentages of 25 acquired idiopathic AA patients and 25 healthy controls who completed study protocol were analyzed by performing multicolor flowcytometry on peripheral blood samples...
February 8, 2018: Hematology (Amsterdam, Netherlands)
Wenqing Yu, Wei Qu, Zhiyong Wang, Chunhong Xin, Rui Jing, Yinghui Shang, Huilin Zou, Hua Wang, Sizhou Feng
RATIONALE: Sjogren's syndrome(SS) is a chronic autoimmune disease, which damages exocrine glands especially salivary and lacrimal glands, with xerostomia and xerophthalmia as common symptoms. PATIENT CONCERNS: We report a case of a 49-year-old woman presented with pancytopenia. Her laboratory examinations lead us diagnose her as Sjogren's syndrome complicating pancytopenia. She had neurological symptoms during her treatment, which represent only 4.5% of Sjogren's syndrome complicating damage in nervous system...
December 2017: Medicine (Baltimore)
Xueping Luo, Zefeng Xu, Bing Li, Tiejun Qin, Peihong Zhang, Hongli Zhang, Liwei Fang, Lijuan Pan, Naibo Hu, Shiqiang Qu, Yue Zhang, Gang Huang, Robert Peter Gale, Zhijian Xiao
Low-dose thalidomide and prednisone alone or combined are effective therapies in some persons with primary myelofibrosis (PMF) and anemia with or with RBC transfusion dependence. Danazol is also effective in some persons with PMF and anemia. Responses to these drugs are typically incomplete and not sustained. It is unclear whether adding danazol to thalidomide and prednisone would improve efficacy. We retrospectively compared the outcomes of 88 subjects with PMF and anemia receiving thalidomide and prednisone without (n = 46) or with danazol (n = 42)...
January 15, 2018: Blood Cancer Journal
Álvaro José Mayorga, Gerardo José Ayestas-Moreno
BACKGROUND: Hereditary angioedema is a disease which manifests itself with episodes of spontaneous edema on skin, mucosal and airway. Treatment includes acute and prophylactic approach to minimize the attacks and severity. In many parts of the world, androgen derivatives, antifibrinolytic and fresh frozen plasma are the therapies available for prophylaxis. CLINICAL REPORT: 16 years old teenager of without history of immune decease, has in the course of 1-year repetitive episodes of painless, non-pruritic angioedema, does not respond to antihistamine therapy, corticosteroids or adrenaline; fresh frozen plasma is applied in 1 occasion exacerbating episode with severity...
October 2017: Revista Alergia Mexico: Organo Oficial de la Sociedad Mexicana de Alergia e Inmunología, A.C
Rodrigo T Calado, Diego V Clé
Despite significant progress in transplantation by the addition of alternative hematopoietic stem cell sources, many patients with inherited bone marrow failure syndromes are still not eligible for a transplant. In addition, the availability of sequencing panels has significantly improved diagnosis by identifying cryptic inherited cases. Androgens are the main nontransplant therapy for bone marrow failure in dyskeratosis congenita and Fanconi anemia, reaching responses in up to 80% of cases. Danazol and oxymetholone are more commonly used, but virilization and liver toxicity are major adverse events...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
Amy Y Hafez, Micah A Luftig
Epstein-Barr virus (EBV) is an oncogenic herpesvirus that is ubiquitous in the human population. Early after EBV infection in vitro, primary human B cells undergo a transient period of hyper-proliferation, which results in replicative stress and DNA damage, activation of the DNA damage response (DDR) pathway and, ultimately, senescence. In this study, we investigated DDR-mediated senescence in early arrested EBV-infected B cells and characterized the establishment of persistent DNA damage foci. We found that arrested EBV-infected B cells exhibited an increase in promyelocytic leukemia nuclear bodies (PML NBs), which predominantly localized to markers of DNA damage, as well as telomeric DNA...
December 1, 2017: Viruses
Andrew T Kuykendall, Savan Shah, Chetasi Talati, Najla Al Ali, Kendra Sweet, Eric Padron, David A Sallman, Jeffrey E Lancet, Alan F List, Kenneth S Zuckerman, Rami S Komrokji
Ruxolitinib is a JAK1/2 inhibitor that is effective in managing symptoms and splenomegaly related to myelofibrosis (MF). Unfortunately, many patients must discontinue ruxolitinib, at which time treatment options are not well defined. In this study, we investigated salvage treatment options and clinical outcomes among MF patients who received and discontinued ruxolitinib outside the context of a clinical trial. Among 145 patients who received ruxolitinib, 23 died while on treatment, 58 remained on treatment at time of analysis, leaving 64 people available for analysis...
March 2018: Annals of Hematology
Gulsen Akoglu, Belgin Kesim, Gokhan Yildiz, Ahmet Metin
BACKGROUND: Hereditary angioedema is a rare autosomal dominantly inherited immunodeficiency disorder characterized by potentially life-threatening angioedema attacks. OBJECTIVE: We aimed to investigate the clinical and genetic features of a family with angioedema attacks. METHODS: The medical history, clinical features and C1-INH gene mutation of a Turkish family were investigated and outcomes of long-term treatments were described. RESULTS: Five members had experienced recurrent swellings on the face and extremities triggered by trauma...
September 2017: Anais Brasileiros de Dermatologia
Zahari Vinarov, V Katev, D Radeva, S Tcholakova, N D Denkov
OBJECTIVE: This study aims to clarify the role of surfactant and drug molecular structures on drug solubility in micellar surfactant solutions. SIGNIFICANCE: (1) Rationale for surfactant selection is provided; (2) the large data set can be used for validation of the drug solubility parameters used in oral absorption models. METHODS: Equilibrium solubility of two hydrophobic drugs and one model hydrophobic steroid in micellar solutions of 19 surfactants was measured by HPLC...
April 2018: Drug Development and Industrial Pharmacy
N Saghafi, H Rhkhshandeh, N Pourmoghadam, L Pourali, M Ghazanfarpour, A Behrooznia, F Vafisani
Breast pain (mastalgia) often precedes menstrual period, which is of mild to moderate severity. This study was performed to determine the effectiveness of chamomile on pain control of cyclic mastalgia. This double-blind randomised controlled clinical trial was conducted on 60 patients with mastalgia referred to the breast clinic of an academic hospital, Mashhad University of Medical Sciences. The patients were randomly allocated into two groups: chamomile (n = 30) and placebo (n = 30). Primary outcomes were: (1) assessment of the visual analogue scale (VAS) and (2) assessment of the breast pain chart (BPC) 8 weeks after initial intervention...
October 26, 2017: Journal of Obstetrics and Gynaecology: the Journal of the Institute of Obstetrics and Gynaecology
Jan-Willem Groen, Sissi Grosfeld, Wichor M Bramer, Miranda F Ernst, Magriet M Mullender
CYCLIC AND NON-CYCLIC BREAST-PAIN: A systematic review on pain reduction, side effects, and quality of life for various treatments. BACKGROUND: No clear systematic-review on all the various treatment regimen for (Non-) cyclical-breast-pain currently exists. OBJECTIVES: The aim of this study was to assess the various forms of therapy for treatment of breast-pain and the evidence for their effectiveness. SEARCH STRATEGY: Search-terms included 'mastalgia' and 'therapy' or 'hormones' or 'nsaid' or 'psychotherapy' or 'analgesia' or 'surgery', and synonyms...
December 2017: European Journal of Obstetrics, Gynecology, and Reproductive Biology
Eric S Surrey, Ahmed M Soliman, Hongbo Yang, Ella Xiaoyan Du, Bowdoin Su
INTRODUCTION: Hysterectomy and laparoscopy are common surgical procedures used for the treatment of endometriosis. This study compares outcomes for women who received either procedure within the first year post initial surgery. METHODS: The study used data from the Truven Health MarketScan claims databases from 2004 to 2013 to identify women aged 18-49 years who received an endometriosis-related laparoscopy or hysterectomy. Patients were excluded if they did not have continuous insurance coverage from 1 year before through 1 year after their endometriosis-related procedure, if they were diagnosed with uterine fibroids prior to or on the date of surgery (i...
November 2017: Advances in Therapy
Maria Szubert, Magdalena Ziętara, Jacek Suzin
Endometriosis with its estimated incidence rate of ∼7-10% of women of reproductive age is a disease with the wide spectrum of symptoms depending on form and localization of endometrial foci. One clinical form of endometriosis is deep infiltrating endometriosis (DIE), most difficult to manage and generating a lot of direct and indirect treatment costs. We search the literature from PubMed database to establish the role of conservative treatment of DIE. Randomised controlled trials are lacking but in experts opinion hormonal treatment should be the first-line treatment in DIE...
January 2018: Gynecological Endocrinology
Fei-Er Feng, Ru Feng, Min Wang, Jia-Min Zhang, Hao Jiang, Qian Jiang, Jin Lu, Hui Liu, Jun Peng, Ming Hou, Jian-Liang Shen, Jing-Wen Wang, Lan-Ping Xu, Kai-Yan Liu, Xiao-Jun Huang, Xiao-Hui Zhang
BACKGROUND: Primary immune thrombocytopenia is a severe bleeding disorder. About 50-85% of patients achieve initial remission from first-line therapies, but optimal second-line treatment remains a challenge. All-trans retinoic acid (ATRA) has an immunomodulatory effect on haemopoiesis, making it a possible treatment option. We aimed to evaluate the efficacy and safety of ATRA plus danazol versus danazol in non-splenectomised patients with corticosteroid-resistant or relapsed primary immune thrombocytopenia...
October 2017: Lancet Haematology
Simon Panzer, Ingrid Pabinger
No abstract text is available yet for this article.
October 2017: Lancet Haematology
Emmanuelle Weber, Quitterie Reynaud, Romain Fort, Stéphane Durupt, Pascal Cathébras, Isabelle Durieu, Jean-Christophe Lega
BACKGROUND: Corticosteroid sparing is required in 15% to 40% of adults with persistent or chronic primary immune thrombocytopenic purpura (ITP). Herein, the efficacy of immunomodulatory drugs (dapsone, interferon alpha, danazol, and hydroxychloroquine as second-third-line therapies in ITP is investigated. METHODS: MEDLINE was searched for studies that included patients with persistent or chronic primary ITP and published before the end of December 2014. Two investigators independently extracted data regarding study design, patient characteristics, dosage schedule, time to response, and occurrence of adverse events...
September 2017: Medicine (Baltimore)
Ashima Taneja, Satinder Kaur, R K Soni, Bhanupriya, Jaspreet Kaur, Laveen Singla
INTRODUCTION: Endometriosis is an oestrogen-dependent disorder, manifests during reproductive years and is associated with pain and infertility. There is considerable debate about the effectiveness of various interventions for pain relief. AIM: To evaluate the efficacy of Levonorgestrel Intrauterine System (LNG-IUS) and Danazol in postoperative pain relief for patients with endometriosis. MATERIALS AND METHODS: Hundred patients with diagnosis of endometriosis, who were treated laparoscopically, entered the study to receive either danazol (600 mg once daily) or LNG-IUS (inserted during immediate post operative period) postsurgery, for pain relief...
July 2017: Journal of Clinical and Diagnostic Research: JCDR
Hai-Zhi Liu, Xiao-Xue Han, Jia Liu, Feng-Cheng Zhu, Rui-Man Li
OBJECTIVE: To study the effect of Taoren Quyu Decoction (TQD) on endometrial cells in patients with endometriosis (EMs) and EMs in rats. METHODS: A total of 60 female Wistar rats were randomly divided into 4 groups, namely, normal group, model group, positive group and TQD group, each group having 15 rats. Except the normal group, EMs model was established in the other three groups by transplanting the rat autologous endometrium. After 4 weeks of intragastric administration, blood, eutopic and ectopic endometrial tissues of rats in each group were collected to detect the serum levels of estrogen (E2), cancer antigen 125 (CA125), endometrial antibody (EMAb), and expressions of microvessel density (MVD), vascular endothelial growth factor (VEGF) and angiopoietin (Ang-2)...
July 2017: Asian Pacific Journal of Tropical Medicine
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