نتایج جستجو برای: t cell immunodeficiency primary

تعداد نتایج: 2753082  

2016
Gholamreza Azizi Nima Rezaei Fatemeh Kiaee Reza Yazdani Abbas Mirshafiey Asghar Aghamohammadi

1. Department of Laboratory Medicine, Imam Hassan Mojtaba Hospital, Alborz University of Medical Sciences, Karaj, Iran 2. Research Center for Immunodeficiencies, Children’s Medical Center, Tehran University of Medical Sciences, Tehran, Iran 3. Primary Immunodeficiency Diseases Network (PIDNet), Universal Scientific Education and Research Network (USERN), Tehran, Iran 4. Department of Immunology...

Journal: :Paediatria Croatica 2021

Background: Schimke immuno-osseous dysplasia (SIOD, OMIM 242900) is a rare, autosomal recessive, pleiotropic disease caused by mutations in the SMARCAL1 gene. SIOD characterized triad of symptoms, i.e., progressive kidney due to focal segmen- tal glomerulosclerosis (FSGS), spondyloepiphyseal and T-cell immunodeficiency. Additionally, heterogeneous neurological symptoms are often observed course...

Journal: :The Journal of Experimental Medicine 1998
Hiroyuki Moriuchi Masako Moriuchi Anthony S. Fauci

It remains controversial whether human T lymphotropic virus type I (HTLV-I) coinfection leads to more rapid progression of human immunodeficiency virus (HIV) disease in dually infected individuals. To investigate whether HTLV-I infection of certain cells can modulate HIV-1 infection of surrounding cells, primary CD4(+) T cells were treated with cell-free supernatants from HTLV-I-infected MT-2 c...

Journal: :Liver transplantation : official publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society 2006
Michela G Schäppi Dominique C Belli Peter C Rimensberger Christophe Chardot Gürkan Kaya Jean-Marie Tiercy Hulya Ozsahin

Fulminant hepatic failure of unknown origin is the most common cause of fulminant hepatitis with high incidence of aplastic anaemia. Furthermore, the association of liver failure and aplastic anaemia has an increased mortality rate. In this report we describe a 16-month-old boy who presented with aplastic anaemia preceding a non-A, non-B, non-C fulminant liver failure. He developed severe graft...

2011
Katrina L. Randall Stephanie S.-Y. Chan Cindy S. Ma Ivan Fung Yan Mei Mehmet Yabas Andy Tan Peter D. Arkwright Wafaa Al Suwairi Saul Oswaldo Lugo Reyes Marco A. Yamazaki-Nakashimada Maria de la Luz Garcia-Cruz Joanne M. Smart Capucine Picard Satoshi Okada Emmanuelle Jouanguy Jean-Laurent Casanova Teresa Lambe Richard J. Cornall Sarah Russell Jane Oliaro Stuart G. Tangye Edward M. Bertram Christopher C. Goodnow

In humans, DOCK8 immunodeficiency syndrome is characterized by severe cutaneous viral infections. Thus, CD8 T cell function may be compromised in the absence of DOCK8. In this study, by analyzing mutant mice and humans, we demonstrate a critical, intrinsic role for DOCK8 in peripheral CD8 T cell survival and function. DOCK8 mutation selectively diminished the abundance of circulating naive CD8 ...

Journal: :Proceedings of the National Academy of Sciences of the United States of America 2004
Yoshiaki Nishimura Tatsuhiko Igarashi Olivia K Donau Alicia Buckler-White Charles Buckler Bernard A P Lafont Robert M Goeken Simoy Goldstein Vanessa M Hirsch Malcolm A Martin

In contrast to simian immunodeficiency viruses (SIVs), which induce immunodeficiency over a 1- to 3-year period, highly pathogenic simian-human immunodeficiency viruses (SHIVs) cause a complete, irreversible, and systemic depletion of CD4(+) T lymphocytes in rhesus monkeys within weeks of infection. By using small-molecule competitors specific for CCR5 and CXCR4 in ex vivo assays, we found that...

2017
Xavier Rios Ivan K. Chinn Jordan S. Orange Celine I. Hanson Nicholas L. Rider

Newborn screening for severe combined immunodeficiency has proven successful in identifying infants with T-cell deficiencies before they become severely ill. Additionally, the newborn screen can detect subtle early phenotypes that may become severe later in life. We present the case of siblings with features suggestive of T-cell lymphopenia identified as having low T-cell receptor excision circ...

2017
Hassan Abolhassani Emily S J Edwards Aydan Ikinciogullari Huie Jing Stephan Borte Marcus Buggert Likun Du Mami Matsuda-Lennikov Rosa Romano Rozina Caridha Sangeeta Bade Yu Zhang Juliet Frederiksen Mingyan Fang Sevgi Kostel Bal Sule Haskologlu Figen Dogu Nurdan Tacyildiz Helen F Matthews Joshua J McElwee Emma Gostick David A Price Umaimainthan Palendira Asghar Aghamohammadi Bertrand Boisson Nima Rezaei Annika C Karlsson Michael J Lenardo Jean-Laurent Casanova Lennart Hammarström Stuart G Tangye Helen C Su Qiang Pan-Hammarström

In this study, we describe four patients from two unrelated families of different ethnicities with a primary immunodeficiency, predominantly manifesting as susceptibility to Epstein-Barr virus (EBV)-related diseases. Three patients presented with EBV-associated Hodgkin's lymphoma and hypogammaglobulinemia; one also had severe varicella infection. The fourth had viral encephalitis during infancy...

Journal: :Proceedings of the National Academy of Sciences 1984

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