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https://www.readbyqxmd.com/read/28333304/further-insights-into-the-impact-of-mouse-follicle-stage-on-graft-outcome-in-an-artificial-ovary-environment
#1
M C Chiti, M M Dolmans, C M Lucci, F Paulini, J Donnez, C A Amorim
STUDY QUESTION: Are mouse preantral follicles differently affected by isolation, encapsulation and/or grafting procedures according to stage? SUMMARY ANSWER: Isolated secondary follicles showed superior ability to survive and grow after transplantation, which was not related to a particular effect of the isolation and/or grafting procedure, but rather to their own ability to induce neoangiogenesis. WHAT IS KNOWN ALREADY: Isolated and encapsulated mouse preantral follicles can survive (6-27%) and grow (80-100%) in a fibrin matrix with a low concentration of fibrinogen and thrombin (F12...
March 17, 2017: Molecular Human Reproduction
https://www.readbyqxmd.com/read/28331055/reticular-dysgenesis-international-survey-on-clinical-presentation-transplantation-and-outcome
#2
Manfred Hoenig, Chantal Lagresle-Peyrou, Ulrich Pannicke, Luigi D Notarangelo, Fulvio Porta, Andrew R Gennery, Mary Slatter, Morton J Cowan, Polina Stepensky, Hamoud Al-Mousa, Daifulah Al-Zahrani, Sung-Yun Pai, Waleed Al Herz, Hubert B Gaspar, Paul Veys, Koichi Oshima, Kohsuke Imai, Hiromasa Yabe, Lenora M Noroski, Nico M Wulffraat, Karl-Walter Sykora, Pere Soler-Palacin, Hideki Muramatsu, Mariam Al Hilali, Despina Moshous, Klaus-Michael Debatin, Catharina Schuetz, Eva-Maria Jacobsen, Ansgar S Schulz, Klaus Schwarz, Alain Fischer, Wilhelm Friedrich, Marina Cavazzana
Reticular Dysgenesis (RD) is a rare congenital disorder defined clinically by the combination of severe combined immunodeficiency (SCID), agranulocytosis and sensorineural deafness. Mutations in the gene encoding Adenylate Kinase 2 (AK2) were identified to cause the disorder. Hematopoietic stem cell transplantation (HSCT) is the only option to cure this otherwise fatal disease. Retrospective data on clinical presentation, genetics and outcome of HSCT were collected from centers in Europe, Asia and North America for a total of 32 patients born between 1982 and 2011...
March 22, 2017: Blood
https://www.readbyqxmd.com/read/28325276/limiting-thymic-precursor-supply-increases-the-risk-of-lymphoid-malignancy-in-murine-x-linked-severe-combined-immunodeficiency
#3
Samantha L Ginn, Claus V Hallwirth, Sophia H Y Liao, Erdahl T Teber, Jonathan W Arthur, Jianmin Wu, Hong Ching Lee, Szun S Tay, Min Hu, Roger R Reddel, Matthew P McCormack, Adrian J Thrasher, Marina Cavazzana, Stephen I Alexander, Ian E Alexander
In early gene therapy trials for SCID-X1, using γ-retroviral vectors, T cell leukemias developed in a subset of patients secondary to insertional proto-oncogene activation. In contrast, we have reported development of T cell leukemias in SCID-X1 mice following lentivirus-mediated gene therapy independent of insertional mutagenesis. A distinguishing feature in our study was that only a proportion of transplanted γc-deficient progenitors were transduced and therefore competent for reconstitution. We hypothesized that reconstitution of SCID-X1 mice with limiting numbers of hematopoietic progenitors might be a risk factor for lymphoid malignancy...
March 17, 2017: Molecular Therapy. Nucleic Acids
https://www.readbyqxmd.com/read/28321326/first-canadian-case-report-of-kidney-transplantation-from-an-hiv-positive-donor-to-an-hiv-positive-recipient
#4
Georges Ambaraghassi, Héloïse Cardinal, Daniel Corsilli, Claude Fortin, Marie-Chantal Fortin, Valérie Martel-Laferrière, Jacques Malaise, Michel R Pâquet, Danielle Rouleau
RATIONALE: Kidney transplantation has become standard of care for carefully selected patients living with human immunodeficiency virus (HIV) and end-stage renal disease (ESRD) in the highly active antiretroviral therapy (HAART) era. American and European prospective cohort studies have reported similar patient and graft survival compared with HIV-negative kidney transplant recipients. Despite an increased rate of acute rejection, partially due to drug interactions, HIV immunovirologic parameter generally remains under control during immunosuppression...
2017: Canadian Journal of Kidney Health and Disease
https://www.readbyqxmd.com/read/28292286/exome-sequencing-identifies-a-novel-ttc37-mutation-in-the-first-reported-case-of-trichohepatoenteric-syndrome-the-s-in-south-africa
#5
Craig Kinnear, Brigitte Glanzmann, Eric Banda, Nikola Schlechter, Glenda Durrheim, Annika Neethling, Etienne Nel, Mardelle Schoeman, Glynis Johnson, Paul D van Helden, Eileen G Hoal, Monika Esser, Michael Urban, Marlo Möller
BACKGROUND: Trichohepatoenteric syndrome (THE-S) or phenotypic diarrhoea of infancy is a rare autosomal recessive disorder characterised by severe infantile diarrhoea, facial dysmorphism, immunodeficiency and woolly hair. It was first described in 1982 in two infants with intractable diarrhoea, liver cirrhosis and abnormal hair structure on microscopy. We report on two siblings from a consanguineous family of Somali descent who, despite extensive clinical investigation, remained undiagnosed until their demise...
March 14, 2017: BMC Medical Genetics
https://www.readbyqxmd.com/read/28288231/-primary-immunodeficiencies-in-seriously-ill-children-report-of-3-clinical-cases
#6
Leticia Yáñez, Pamela Lama, Carolina Rivacoba, Juanita Zamorano, María Angélica Marinovic
Primary immunodeficiency diseases (PID) are congenital disorders secondary to an impaired immune response. Infections, autoimmune disorders, atopy, and lymphoproliferative syndromes are commonly associated with this disorder. OBJECTIVE: To present and discuss 3 infants diagnosed with PID. CLINICAL CASES: The cases are presented of three patients with PID diagnosed during their first admission to a Paediatric Intensive Critical Care Unit. The first patient, a 4-month-old infant affected by a severe pneumonia, and was diagnosed as a Severe Combined Immunodeficiency Disease...
February 2017: Revista Chilena de Pediatría
https://www.readbyqxmd.com/read/28285796/cardiovascular-disease-in-women-with-hiv-1-infection
#7
Massimo Volpe, Alessia Uglietti, Antonella Castagna, Cristina Mussini, Giulia Marchetti, Rita Bellagamba, Teresa Bini, Daniela Mancusi, Roberta Termini
Cardiovascular disease is a leading cause of death in women, nevertheless it is often underestimated in female patients without overt risk factors. The chronic infection by Human Immunodeficiency Virus (HIV) is clearly associated, along with the use of certain antiretroviral drugs and traditional risk factors, with an increased risk of cardiovascular diseases. The aim of this manuscript is to review the epidemiology, risk factors, pathogenesis, diagnostic approach, primary and secondary prevention strategies of cardiovascular disease in HIV-negative and HIV-positive female subjects...
February 27, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28284260/tuberculosis-in-hiv-infected-south-african-children-with-complicated-severe-acute-malnutrition
#8
H Adler, M Archary, P Mahabeer, P LaRussa, R A Bobat
SETTING: Academic tertiary referral hospital in Durban, South Africa. OBJECTIVE: To describe the incidence and diagnostic challenges of tuberculosis (TB) in human immunodeficiency virus (HIV) infected children with severe acute malnutrition (SAM). DESIGN: Post-hoc analysis of a randomised controlled trial that enrolled antiretroviral therapy naïve, HIV-infected children with SAM. Trial records and hospital laboratory results were explored for clinical diagnoses and bacteriologically confirmed cases of TB...
April 1, 2017: International Journal of Tuberculosis and Lung Disease
https://www.readbyqxmd.com/read/28261507/endoscopic-appearance-of-oropharyngeal-and-upper-gi-kaposi-s-sarcoma-in-an-immunocompromised-patient
#9
Umar Darr, Anas Renno, Zubair Khan, Turki Alkully, Maitham A Moslim, Sehrish Kamal, Ali Nawras
Introduction. Kaposi's sarcoma (KS) usually manifests as a cutaneous disease but GI manifestation is often rare. It is associated with human herpes virus-8 (HHV-8) and seen in immunocompromised patients. In the USA, use of highly active antiretroviral therapy (HAART) has drastically reduced incidence of KS in HIV patients. Case Presentation. A 65-year-old male with human immunodeficiency virus (HIV) was admitted to the intensive care unit (ICU) with cardiopulmonary arrest secondary to hyperkalemia of 7.5 meq/L...
2017: Case Reports in Gastrointestinal Medicine
https://www.readbyqxmd.com/read/28260858/systemic-abnormalities-associated-with-retinal-vein-occlusion-in-young-patients
#10
Suthasinee Sinawat, Chavisa Bunyavee, Tanapat Ratanapakorn, Supat Sinawat, Wipada Laovirojjanakul, Yosanan Yospaiboon
OBJECTIVES: To study the systemic abnormalities associated with retinal vein occlusion in patients aged ≤50 years with a particular emphasis on atherosclerotic diseases and thrombophilic disorders. METHODS: Medical charts of patients, aged ≤50 years whose diagnoses were retinal vein occlusions during the period 1995-2015 were retrospectively reviewed. The primary outcome was the number of systemic abnormalities associated with these patients. Secondary outcomes included types of retinal vein occlusion and sites of occlusion...
2017: Clinical Ophthalmology
https://www.readbyqxmd.com/read/28260245/sexuality-and-hiv-education-in-charter-schools-an-exploratory-study-with-principals-in-san-diego-county-california
#11
Eric R Walsh-Buhi, Brandon Dao, Linda Salgin, James Marshall, Rachel Miller, Doug Fisher, Margaret Walsh-Buhi
BACKGROUND: Schools can address critical sexual and reproductive health (SRH) issues among youth. However, little is known about SRH education being implemented in charter schools. Thus, our purpose was to explore implementation of SRH education in charter schools. METHODS: Using purposive sampling, semistructured telephone interviews were conducted with 20 charter school principals in San Diego County, California. Questions were guided by the Kaiser Family Foundation National Survey of Public Secondary School Principals...
April 2017: Journal of School Health
https://www.readbyqxmd.com/read/28259182/an-unexpected-diagnosis-of-human-immunodeficiency-virus-2-infection-in-an-overseas-visitor-a-case-report
#12
Asma Sohail, Lyndal Van Leer, Natasha Holmes
BACKGROUND: Human immunodeficiency virus 2 infection is endemic in West Africa but is also found in parts of Europe, North and South America, and India where it is thought to have been introduced secondary to migration and commercial trade ties. It is less common than Human immunodeficiency virus 1, with differences in pathogenicity, lower rates of transmission, longer asymptomatic period and slower progression to acquired immunodeficiency syndrome. Human immunodeficiency virus 2 is also associated with diagnostic challenges given the lack of commercially available diagnostic tests, and management challenges given intrinsic resistance to many anti-retroviral therapies...
March 4, 2017: BMC Research Notes
https://www.readbyqxmd.com/read/28257650/subcutaneous-immunoglobulins-for-the-treatment-of-a-patient-with-antisynthetase-syndrome-and-secondary-chronic-immunodeficiency-after-anti-cd20-treatment-a-case-report
#13
Patrick Cherin, Christophe de Jaeger, Jean-Charles Crave, Jean-Christophe Delain, Abir Tadmouri, Zahir Amoura
BACKGROUND: Antisynthetase syndrome is a rare and debilitating multiorgan disease characterized by inflammatory myopathy, interstitial lung disease, cutaneous involvement, and frequent chronic inflammation of the joints. Standard treatments include corticosteroids and immunosuppressants. In some cases, treatment resistance may develop. Administration of immunoglobulins intravenously is recommended in patients with drug-resistant antisynthetase syndrome. CASE PRESENTATION: Here, we describe the case of a 56-year-old woman of Algerian origin...
March 4, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28255007/the-human-chorion-contains-definitive-hematopoietic-stem-cells-from-the-15-th-week-of-gestation
#14
Marcus O Muench, Mirhan Kapidzic, Matthew Gormley, Alan G Gutierrez, Kathryn L Ponder, Marina E Fomin, Ashley I Beyer, Haley Stolp, Zhongxia Qi, Susan J Fisher, Alicia Bárcena
We examined the contribution of the fetal membranes, amnion and chorion, to human embryonic and fetal hematopoiesis. A population of cells displaying a hematopoietic progenitor phenotype (CD34(++/+)CD45(low) cells) of fetal origin was present in the chorion at all gestational ages, associated with stromal cells or near blood vessels, but was absent in the amnion. Prior to 15 weeks of gestation, they lacked hematopoietic in vivo engraftment potential. Differences in the chemokine receptor and β1 integrin expression profiles of progenitors between the 1(st) and 2(nd) trimesters suggest that these cells had gestationally-regulated responses to homing signals and/or adhesion mechanisms that influenced their ability to colonize the stem cell niche...
March 2, 2017: Development
https://www.readbyqxmd.com/read/28240614/diagnostic-and-therapeutic-considerations-in-patients-with-hypogammaglobulinemia-after-rituximab-therapy
#15
Ruba Kado, Georgiana Sanders, W Joseph McCune
PURPOSE OF REVIEW: There are no established guidelines for evaluating and treating hypogammaglobulinemia in patients with rheumatic disease who receive B-cell depleting agents. The purpose of this article is to review findings in the work-up and treatment of common variable immunodeficiency (CVID) that can guide our evaluation of patients with autoimmune disease who develop hypogammaglobulinemia after rituximab/B-cell depleting therapy. RECENT FINDINGS: Infection rates are higher in rheumatic disease patients who develop hypogammaglobulinemia than those who do not...
February 24, 2017: Current Opinion in Rheumatology
https://www.readbyqxmd.com/read/28239902/ecthyma-gangrenosum-without-bacteraemia-evidence-in-favour-of-a-broader-definition
#16
L Ferguson, H Chong, M Singh
Ecthyma gangrenosum (EG) is often defined as a cutaneous manifestation of Pseudomonas aeruginosa septicaemia, typically secondary to neutropenia. There is increasing recognition that a broader definition is warranted, as numerous causative organisms and predisposing conditions have been reported. We describe two cases of EG that occurred without bacteraemia. In this atypical subset of cases, the skin is thought to represent the primary inoculation site from which haematogenous spread can occur. The first case occurred in the context of human immunodeficiency virus (HIV) infection, a rarely reported association...
February 27, 2017: Clinical and Experimental Dermatology
https://www.readbyqxmd.com/read/28239602/first-case-of-cd40lg-deficiency-in-ecuador-diagnosed-after-whole-exome-sequencing-in-a-patient-with-severe-cutaneous-histoplasmosis
#17
Luis Alberto Pedroza, Nina Guerrero, Asbjørg Stray-Pedersen, Cristina Tafur, Roque Macias, Greta Muñoz, Zeynep Coban Akdemir, Shalini N Jhangiani, Levi B Watkin, Ivan K Chinn, James R Lupski, Jordan S Orange
Severe infections with Histoplasma capsulatum are commonly observed in patient with secondary immunodeficiency disorders. We report a two and a half years old boy previously healthy with disseminated cutaneous histoplasmosis. Using whole exome sequencing, we found an indel mutation at the CD40LG gene, suggesting a diagnosis of hyper-IgM (HIGM) syndrome, even in the absence of the usual features for the disease. Interestingly, the patient lives in a region endemic for histoplasmosis. The unusual infections in our case suggest that in children with severe histoplasmosis and resident in endemic areas, HIGM syndrome should be considered as a diagnosis...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28190779/lentiviral-transfer-of-%C3%AE-globin-with-fusion-gene-nup98-hoxa10hd-expands-hematopoietic-stem-cells-and-ameliorates-murine-%C3%AE-thalassemia
#18
Hui Fen Zhao, Allistair Abraham, Yoon-Sang Kim, Yong-Dong Wang, Tamara Pestina, Jun Zhan, Keith Humphries, Arthur W Nienhuis, Derek A Persons
Recently, an engineered Homeobox-nucleoporin fusion gene, NUP98-HOXA10HD or NA10HD, was reported to expand and maintain murine hematopoietic stem cells (HSCs). We postulated that NA10HD would increase the number of human γ-globin-expressing cells to therapeutic levels. We developed a double gene lentiviral vector encoding both human γ-globin and NA10HD, which was used to transduce human peripheral blood CD34(+) cells and increased engraftment 2- to 2.5-fold at 15 weeks post-transplantation in immunodeficient mice...
March 1, 2017: Molecular Therapy: the Journal of the American Society of Gene Therapy
https://www.readbyqxmd.com/read/28178109/risk-factors-for-incidence-of-sexually-transmitted-infections-among-women-in-a-human-immunodeficiency-virus-chemoprevention-trial-voice-mtn-003
#19
Zvavahera Mike Chirenje, Holly M Gundacker, Barbra Richardson, Lorna Rabe, Zakir Gaffoor, Gonasagrie Lulu Nair, Brenda Gati Mirembe, Jeanna M Piper, Sharon Hillier, Jeanne Marrazzo
BACKGROUND: In sub-Saharan Africa, there are limited data on the incidence of sexually transmitted infections (STIs) among women, largely because routine screening for asymptomatic infection is not performed. We conducted a secondary analysis to measure STI incidence rates and determine risk factors for new STI acquisition among women enrolled in the VOICE trial. METHODS: We analyzed data from 4843 women screened for chlamydia, gonorrhoea, syphilis, and trichomonas infection at baseline, annually, at interim visits when clinically indicated and at their study termination visit...
March 2017: Sexually Transmitted Diseases
https://www.readbyqxmd.com/read/28178076/pediatric-acute-respiratory-distress-syndrome-in-pediatric-allogeneic-hematopoietic-stem-cell-transplants-a-multicenter-study
#20
Courtney M Rowan, Lincoln S Smith, Ashley Loomis, Jennifer McArthur, Shira J Gertz, Julie C Fitzgerald, Mara E Nitu, Elizabeth A S Moser, Deyin D Hsing, Christine N Duncan, Kris M Mahadeo, Jerelyn Moffet, Mark W Hall, Emily L Pinos, Robert F Tamburro, Ira M Cheifetz
OBJECTIVE: Immunodeficiency is both a preexisting condition and a risk factor for mortality in pediatric acute respiratory distress syndrome. We describe a series of pediatric allogeneic hematopoietic stem cell transplant patients with pediatric acute respiratory distress syndrome based on the recent Pediatric Acute Lung Injury Consensus Conference guidelines with the objective to better define survival of this population. DESIGN: Secondary analysis of a retrospective database...
February 7, 2017: Pediatric Critical Care Medicine
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