نتایج جستجو برای: severe combined immune deficiency

تعداد نتایج: 1039889  

Journal: :The Journal of clinical investigation 1979
M S Hershfield N M Kredich D R Ownby H Ownby R Buckley

The cytotoxic nucleoside 2'-deoxyadenosine is excreted in excessive amounts by individuals with genetic deficiency of adenosine deaminase, and may be in part responsible for the severe combined immune dysfunction from which they suffer. Earlier studies from this laboratory showed that 2'-deoxyadenosine causes the irreversible inactivation of the enzyme S-adenosylhomocysteine hydrolase by an act...

Journal: :Journal of microbiology, immunology, and infection = Wei mian yu gan ran za zhi 2010
Alexandra Santos Andrea Dias Ana Cordeiro Carolina Cordinhã Sónia Lemos Graça Rocha Emília Faria

The bacilli Calmette-Guérin (BCG) vaccine is administered to all newborns in countries where tuberculosis is endemic. Immunocompromised hosts, namely patients with human immunodeficiency virus infection or primary immunodeficiencies, are especially prone to serious complications from this vaccine. We report three cases of BCG disease in children with primary immunodeficiencies: one with a parti...

Journal: :PLoS Pathogens 2006
Kathie-Anne Walters Michael A Joyce Jill C Thompson Maria W Smith Matthew M Yeh Sean Proll Lin-Fu Zhu T. J Gao Norman M Kneteman D. Lorne Tyrrell Michael G Katze

The severe combined immunodeficiency disorder (SCID)-beige/albumin (Alb)-urokinase plasminogen activator (uPA) mouse containing a human-mouse chimeric liver is currently the only small animal model capable of supporting hepatitis C virus (HCV) infection. This model was utilized to characterize the host transcriptional response to HCV infection. The purpose of these studies was to investigate th...

Bahrami, Ahmad , Haghighi Aski, Behzad , Kalantar, Sara , Manafi Anari, Ali , Talebi, Saeid , Zare Mahmood Abadi, Ramin ,

Digeorge syndrome is caused by microdeletion of a large region of chromosome 22q11.2 lead to the abnormal development of the third and fourth pharyngeal pouches. This syndrome is characterized by hypoparathyroidism, cellular immune deficiency secondary to thymic hypoplasia, congenital heart disease and dysmorphic facial features. In this case report, we describe a 4month old boy who presented w...

Journal: :Clinical infectious diseases : an official publication of the Infectious Diseases Society of America 2009
Marion Malphettes Laurence Gérard Maryvonnick Carmagnat Gaël Mouillot Nicolas Vince David Boutboul Alice Bérezné Raphaële Nove-Josserand Vincent Lemoing Laurent Tetu Jean-François Viallard Bernard Bonnotte Michel Pavic Julien Haroche Claire Larroche Jean-Claude Brouet Jean-Paul Fermand Claire Rabian Claire Fieschi Eric Oksenhendler

BACKGROUND Common variable immunodeficiency (CVID) is a primary immune deficiency defined by defective antibody production. In most series, a small proportion of patients present with opportunistic infections (OIs). METHODS The French DEFI study has enrolled patients with primary hypogammaglobulinemia and allows a detailed clinical and immunologic description of patients with previous OIs and...

Journal: :iranian journal of allergy, asthma and immunology 0
elahe rashidi immunology, asthma and allergy research institute, tehran university of medical sciences, tehran, iran mohammad reza fazlollahi immunology, asthma and allergy research institute, tehran university of medical sciences, tehran, iran sara zahedifard department of pathology, medical faculty, shahid beheshti university of medical sciences, tehran, iran azadeh talebzadeh immunology, asthma and allergy research institute, tehran university of medical sciences, tehran, iran anoshirvan kazemnejad department of biostatistics, faculty of medical sciences, tarbiat modares university, tehran, iran shiva saghafi immunology, asthma and allergy research institute, tehran university of medical sciences, tehran, iran

mannose-binding lectin (mbl) is a protein of innate immune system that is involved in opsonization and complement activation. mbl deficiency is associated with predisposition to infectious diseases; however subnormal levels are also seen in healthy subjects. the aim of this study was to investigate the prevalence and clinical manifestation of mbl deficiency in patients with increased susceptibi...

Journal: :The Journal of pediatrics 2016
Yao Ding John D Thompson Lisa Kobrynski Jelili Ojodu Guisou Zarbalian Scott D Grosse

OBJECTIVE To evaluate the expected cost-effectiveness and net benefit of the recent implementation of newborn screening (NBS) for severe combined immunodeficiency (SCID) in Washington State. STUDY DESIGN We constructed a decision analysis model to estimate the costs and benefits of NBS in an annual birth cohort of 86 600 infants based on projections of avoided infant deaths. Point estimates a...

2010
Otfried Kistner Brian A. Crowe Walter Wodal Astrid Kerschbaum Helga Savidis-Dacho Nicolas Sabarth Falko G. Falkner Ines Mayerhofer Wolfgang Mundt Manfred Reiter Leopold Grillberger Christa Tauer Michael Graninger Alois Sachslehner Michael Schwendinger Peter Brühl Thomas R. Kreil Hartmut J. Ehrlich P. Noel Barrett

The recent emergence and rapid spread of a novel swine-derived H1N1 influenza virus has resulted in the first influenza pandemic of this century. Monovalent vaccines have undergone preclinical and clinical development prior to initiation of mass immunization campaigns. We have carried out a series of immunogenicity and protection studies following active immunization of mice, which indicate tha...

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