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https://www.readbyqxmd.com/read/28323922/fibrogenesis-imperfecta-ossium-and-response-to-human-growth-hormone-a-potential-novel-therapy
#1
Sanjay Kumar Bhadada, Vandana Dhiman, Soham Mukherjee, Sameer Aggarwal, Amanjit Bal, Suja P Sukumar, Ashwani Sood, Dinesh Chandra Sharma, Niranjan Khandelwal, Anil Bhansali, Wim Van Hul, Sudhaker D Rao
Context: Fibrogenesis imperfecta ossium (FIO) is a rare bone disease manifested by generalized bone pain, fragility fractures, progressive disability, and extensive mineralization defect on bone biopsy. The pathogenesis of the disease is unknown and currently there is no effective treatment. Objective: To report on the effect of recombinant human growth hormone (rhGH) therapy in FIO. Design: An observational study in two patients. Setting: Endocrinology Clinic in an Academic Institution Patients or Other Participants: Two siblings with FIO Intervention(s): rhGH was administered subcutaneously at a dose of 1U daily for one year...
February 28, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28322994/the-role-of-mirna-223-in-cancer-function-diagnosis-and-therapy
#2
REVIEW
Yunliang Gao, Le Lin, Tao Li, Jinrui Yang, Yongbao Wei
MicroRNAs (miRNAs) constitute a large family of small, non-coding RNAs with the capacity to regulate gene expression post-transcriptionally. miRNAs appear to hold promise of mechanistic explanations for various physiological and pathological processes. miRNA-223 is highly conserved and preferentially expressed in the hematopoietic system in regulation of myeloid differentiation. Recently, increasing evidence suggests that miRNA-223 may also play an essential part in both hematological malignancies and solid tumors...
March 17, 2017: Gene
https://www.readbyqxmd.com/read/28322187/influence-of-genetic-background-on-hematologic-and-histopathologic-alterations-during-acute-granulocytic-anaplasmosis-in-129-svev-and-c57bl-6j-mice-lacking-type-i-and-type-ii-interferon-signaling
#3
Jennifer L Johns Marielle L Discipulo Amanda L Koehne Kaitlin A Moorhead And Claude M Nagamine
The role of host type I IFN signaling and its interaction with other immune pathways during bacterial infections is incompletelyunderstood. Type II IFN signaling plays a key role during numerous bacterial infections including granulocytic anaplasmosis (GA)caused by Anaplasma phagocytophilum infection. The function of combined type I and type II IFN signaling and their potentialsynergism during GA and similar tick-borne diseases is a topic of current research investigation. The goal of this study was toevaluate 2 mouse models of absent type I/type II IFN signaling in experimental A...
March 20, 2017: Comparative Medicine
https://www.readbyqxmd.com/read/28321122/the-emerging-roles-of-tumor-derived-exosomes-in-hematological-malignancies
#4
REVIEW
M Boyiadzis, T L Whiteside
Exosomes are small (30-150 nm) membranous vesicles of endocytic origin produced by all cells under physiological and pathological conditions. They have recently emerged as vehicles for intercellular transfer of molecular and genetic contents from parent to recipient cells. Exosome-mediated transfer of proteins or genes (RNA, miRNA, DNA) results in reprogramming of recipient cell functions. Exosomes carry and deliver information that is essential for health, and they participate in pathological events, including malignant transformation...
March 21, 2017: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
https://www.readbyqxmd.com/read/28296681/adenovirus-hepatitis-clinicopathologic-analysis-of-12-consecutive-cases-from-a-single-institution
#5
Kurt B Schaberg, Neeraja Kambham, Richard K Sibley, John P T Higgins
Adenoviruses are common pathogens that usually cause self-limited infections. However, in the immunocompromised host they can cause severe infections involving multiple organs including the liver. A search of the pathology database at Stanford University Medical Center (1995 to 2016) identified 12 cases of adenovirus hepatitis including biopsy and autopsy specimens. There were 8 pediatric patients, 7 of which had received orthotropic liver transplants and 1 of which was receiving chemotherapy for lymphoblastic leukemia...
March 14, 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28295628/pathophysiological-aspects-of-red-blood-cells-in-end-stage-renal-disease-patients-resistant-to-recombinant-human-erythropoietin-therapy
#6
Hara T Georgatzakou, Vassilis L Tzounakas, Anastasios G Kriebardis, Athanassios D Velentzas, Effie G Papageorgiou, Artemis I Voulgaridou, Apostolos C Kokkalis, Marianna H Antonelou, Issidora S Papassideri
OBJECTIVE: Modified, bio-reactive red blood cells (RBCs) and RBC-derived microvesicles likely contribute to the hematological and cardiovascular complications in end-stage renal disease (ESRD). This study assesses the physiological profile of red blood cells (RBCs) in end-stage renal disease (ESRD) patients receiving standard or high doses of recombinant human erythropoietin (rhEPO). METHOD: Blood samples from twenty-eight patients under sustained hemodialysis, responsive or not to standard rhEPO administration were examined for RBC morphology, fragility, hemolysis, redox status, removal signaling, membrane protein composition and microvesiculation before and after dialysis...
March 10, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28295050/network-analysis-reveals-crosstalk-between-autophagy-genes-and-disease-genes
#7
Ji-Ye Wang, Wei-Xuan Yao, Yun Wang, Yi-Lei Fan, Jian-Bing Wu
Autophagy is a protective and life-sustaining process in which cytoplasmic components are packaged into double-membrane vesicles and targeted to lysosomes for degradation. Accumulating evidence supports that autophagy is associated with several pathological conditions. However, research on the functional cross-links between autophagy and disease genes remains in its early stages. In this study, we constructed a disease-autophagy network (DAN) by integrating known disease genes, known autophagy genes and protein-protein interactions (PPI)...
March 15, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28290981/case-report-of-clinical-vignette-osteopetrosis
#8
John B Moore, Thanh D Hoang, Alfred F Shwayhat
INTRODUCTION: Osteopetrosis is a connective tissue disorder resulting from abnormally dense bone predisposing patients to fracture. The clinical pattern of fractures across time and space as well as suggestive radiographic findings usually raises diagnostic suspicion. Multiple genetic mutations resulting in dysfunctional osteoclasts have been implicated in the pathogenesis of osteopetrosis with variable inheritance patterns. In severe cases, usually inherited in an autosomal recessive pattern, the medullary cavity important in the production of normal blood cell progenitors is replaced by defective endochondral bone, leading to pancytopenia and consequential extramedullary hematopoiesis...
March 2017: Military Medicine
https://www.readbyqxmd.com/read/28273655/new-agents-that-target-senescent-cells-the-flavone-fisetin-and-the-bcl-xl-inhibitors-a1331852-and-a1155463
#9
Yi Zhu, Ewald J Doornebal, Tamar Pirtskhalava, Nino Giorgadze, Mark Wentworth, Heike Fuhrmann-Stroissnigg, Laura J Niedernhofer, Paul D Robbins, Tamara Tchkonia, James L Kirkland
Senescent cells accumulate with aging and at sites of pathology in multiple chronic diseases. Senolytics are drugs that selectively promote apoptosis of senescent cells by temporarily disabling the pro-survival pathways that enable senescent cells to resist the pro-apoptotic, pro-inflammatory factors that they themselves secrete. Reducing senescent cell burden by genetic approaches or by administering senolytics delays or alleviates multiple age- and disease-related adverse phenotypes in preclinical models...
March 8, 2017: Aging
https://www.readbyqxmd.com/read/28271647/mycobacterium-kansasii-pneumonia-with-mediastinal-lymphadenitis-in-a-patient-with-acute-myeloid-leukemia-successful-treatment-to-stem-cell-transplantation
#10
Yeon Geun Choi, Sung Yeon Cho, Dong Gun Lee, Eunjung Yim, Hyonsoo Joo, Seongyul Ryu, Jae Ki Choi, Hee Je Kim
Non-tuberculous mycobacterial (NTM) disease is a relatively rare cause of neutropenic fever in patients with hematologic malignancies. During the neutropenic period, performing invasive procedures for microbiological or pathological confirmation is difficult. In addition, the optimal treatment duration for NTM disease in patients with leukemia, especially prior to stem cell transplantation (SCT), has not been documented. Therefore, we report a case of pneumonia with necrotizing lymphadenitis caused by Mycobacterium kansasii diagnosed during chemotherapy being performed for acute myeloid leukemia...
January 19, 2017: Infection & Chemotherapy
https://www.readbyqxmd.com/read/28270940/synchronous-occurrence-of-chronic-myeloid-leukemia-and-mantle-cell-lymphoma
#11
Prajwol Pathak, Ying Li, Brian Allen Gray, William Stratford May, Merry Jennifer Markham
Chronic myeloid leukemia (CML) and mantle cell lymphoma (MCL) are hematologic malignancies that originate from different oligopotent progenitor stem cells, namely, common myeloid and lymphoid progenitor cells, respectively. Although blastic transformation of CML can occur in the lymphoid lineage and CML has been related to non-Hodgkin lymphoma on transformation, to our knowledge, de novo and synchronous occurrence of CML and MCL has not been reported. Herein, we report the first case of synchronous CML and MCL in an otherwise healthy 38-year-old man...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28265009/preclinical-mammalian-safety-studies-of-epharna-dopc-nanoliposomal-epha2-targeted-sirna
#12
Michael J Wagner, Rahul Mitra, Mark J McArthur, Wallace Baze, Kirstin Barnhart, Sherry Wu, Cristian Rodriguez-Aguayo, Xinna Zhang, Robert L Coleman, Gabriel Lopez-Berestein, Anil K Sood
To address the need for efficient and biocompatible delivery systems for systemic siRNA delivery, we developed 1,2-Dioleoyl-sn-Glycero-3-Phosphatidylcholine (DOPC) nanoliposomal EphA2-targeted therapeutic (EPHARNA). Here, we performed safety studies of EPHARNA in murine and primate models. Single dosing of EPHARNA was tested at 5 concentrations in mice (N=15 per group) and groups were sacrificed on days 1, 14, and 28 for evaluation of clinical pathology and organ toxicity. Multiple dosing of EPHARNA was tested in mice and Rhesus macaques twice weekly at two dose levels in each model...
March 6, 2017: Molecular Cancer Therapeutics
https://www.readbyqxmd.com/read/28246450/protective-effect-of-rutin-in-comparison-to-silymarin-against-induced-hepatotoxicity-in-rats
#13
M Kasi Reddy, A Gopala Reddy, B Kala Kumar, D Madhuri, G Boobalan, M Anudeep Reddy
AIM: The aim of this study is to evaluate the hepatoprotective effect of rutin (RTN) in comparison to silymarin (SLM) against acetaminophen (APAP)-induced hepatotoxicity in rats. MATERIALS AND METHODS: Male Wistar albino rats (n=24) of 3 months age were equally divided into four groups. Group 1 served as normal control. Hepatotoxicity was induced in the remaining three groups with administration of 500 mg/kg po APAP from day 1-3. Groups 2, 3, and 4 were subsequently administered orally with distilled water, 25 mg/kg of SLM, and 20 mg/kg of RTN, respectively, for 11 days...
January 2017: Veterinary World
https://www.readbyqxmd.com/read/28239179/unsuspected-lung-pathology-in-autopsies-of-children-with-cancer
#14
María Elena Y Furuya, Alicia Rodríguez-Velasco, María Carmen Rodríguez-Zepeda, Enrique López-Aguilar, Mario H Vargas, Martha Sciandra-Rico, Rocío Cárdenas-Navarrete, Olivia Madrigal-Muñiz, Carina Feria-Kaiser, Elba E Delgado-González, Miguel Ángel Villasís-Keeverz
BACKGROUND: Although pulmonary involvement is common in patients with cancer, its frequency and nature is seldom reported in the medical literature. OBJECTIVE: To determine the frequency and type of lung pathological conditions revealed by autopsy in children with cancer. METHODS: All reports from autopsies performed in children with cancer from 1989 to 2012 in a pediatric hospital were reviewed. RESULTS: In the analyzed period, 118 autopsies (10...
January 2017: Revista de Investigación Clínica; Organo del Hospital de Enfermedades de la Nutrición
https://www.readbyqxmd.com/read/28228103/disease-manifestations-and-burden-of-illness-in-patients-with-acid-sphingomyelinase-deficiency-asmd
#15
REVIEW
Margaret M McGovern, Ruzan Avetisyan, Bernd-Jan Sanson, Olivier Lidove
Acid sphingomyelinase deficiency (ASMD), a rare lysosomal storage disease, is an autosomal recessive genetic disorder caused by different SMPD1 mutations. Historically, ASMD has been classified as Niemann-Pick disease (NPD) types A (NPD A) and B (NPD B). NPD A is associated with a uniformly devastating disease course, with rapidly progressing psychomotor degeneration, leading to death typically by the age of 3 years, most often from respiratory failure. In contrast, the clinical phenotype and life expectancy of patients with NPD B may vary widely...
February 23, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28223802/fe3o4-based-plga-nanoparticles-as-mr-contrast-agents-for-the-detection-of-thrombosis
#16
Jia Liu, Jie Xu, Jun Zhou, Yu Zhang, Dajing Guo, Zhigang Wang
Thrombotic disease is a great threat to human health, and early detection is particularly important. Magnetic resonance (MR) molecular imaging provides noninvasive imaging with the potential for early disease diagnosis. In this study, we developed Fe3O4-based poly(lactic-co-glycolic acid) (PLGA) nanoparticles (NPs) surface-modified with a cyclic Arg-Gly-Asp (cRGD) peptide as an MR contrast agent for the detection of thrombosis. The physical and chemical characteristics, biological toxicity, ability to target thrombi, and biodistribution of the NPs were studied...
2017: International Journal of Nanomedicine
https://www.readbyqxmd.com/read/28222323/a-case-report-of-the-clear-cell-variant-of-gallbladder-carcinoma
#17
Ravi Maharaj, Christo Cave, Kevin Sarran, Nigel Bascombe, Dilip Dan, Wesley Greaves, Wayne A Warner
INTRODUCTION: Clear cell gallbladder carcinoma accounts for less than 1% of all gallbladder malignancies and demonstrates its unique histopathological characteristics in patients with no prior medical illness or familial predisposition. PRESENTATION OF CASE: Here we present a case of a 56-year-old female, with no prior medical conditions presented with a 2-month history of upper abdominal pain. Routine hematological and biochemical tests were unremarkable. An abdominal ultrasound revealed the presence of a gallbladder calculi, and a fundic mass while magnetic resonance cholangiopancreatography revealed a 8...
January 12, 2017: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/28218709/pet-imaging-for-initial-staging-and-therapy-assessment-in-multiple-myeloma-patients
#18
REVIEW
Clément Bailly, Rodolphe Leforestier, Bastien Jamet, Thomas Carlier, Mickael Bourgeois, François Guérard, Cyrille Touzeau, Philippe Moreau, Michel Chérel, Françoise Kraeber-Bodéré, Caroline Bodet-Milin
Multiple myeloma (MM) is a hematological neoplasm characterized by the clonal proliferation of malignant plasma cells in the bone marrow. MM results in diffuse or focal bone infiltration and extramedullary lesions. Over the past two decades, advances have been made with regard to the diagnosis, staging, treatment, and imaging of MM. Computed tomography (CT) and magnetic resonance imaging (MRI) are currently recommended as the most effective imaging modalities at diagnostic. Yet, recent data from the literature suggest that positron emission tomography combined with computed tomography (PET/CT) using (18)F-deoxyglucose (FDG) is a promising technique for initial staging and therapeutic monitoring in this pathology...
February 18, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28213738/cytomegalovirus-infection-in-patients-with-lupus-nephritis-clinical-and-laboratory-features-and-therapeutic-considerations
#19
Lei Zhang, Jianling Tao, Yubing Wen, Li Li, Xueyi Wu, Xuewang Li, Xuemei Li
To better clarify the clinical features and therapeutic strategy of CMV infection in lupus nephritis patients, we retrospectively surveyed a total of 40 lupus nephritis patients, who had been hospitalized and underwent renal biopsy and diagnosed as having CMV infection during their hospitalization at our institution within the last 10 years. The percentage of CMV infections in the entire hospitalized lupus nephritis population was 5.3% (40/755). The principal clinical features of the 40 CMV-infected patients were hematological disorders (n = 25), fever (n = 21), liver dysfunction (n = 19), and respiratory symptoms (n = 12)...
February 17, 2017: Clinical and Experimental Medicine
https://www.readbyqxmd.com/read/28187089/clinical-outcome-of-neoadjuvant-radiochemotherapy-in-locally-advanced-cervical-cancer-results-of-an-open-prospective-multicenter-phase-2-study-of-the-north-eastern-german-society-of-gynecological-oncology
#20
Dominique Koensgen, Jalid Sehouli, Antje Belau, Martin Weiss, Matthias B Stope, Vivien Grokopf, Michael Eichbaum, Peter Ledwon, Werner Lichtenegger, Marek Zygmunt, Günter Köhler, Alexander Mustea
OBJECTIVE: The aim of this study was to determine the response rate, toxicity, operability, and surgical complication rate of neoadjuvant concomitant radiochemotherapy (cRCH) (ifosfamide + carboplatin) followed by radical hysterectomy plus external-beam radiotherapy with curative intention in locally advanced primary inoperable stages IIB and IIIB squamous cell cervical cancer. METHODS: Patients with cervical cancer from 8 departments were enrolled. Patients received 3 cycles of ifosfamide 1...
March 2017: International Journal of Gynecological Cancer
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