Results 41 to 50 of about 5,617 (179)

X-Linked Agammaglobulinemia

Medicine, 1985
We have defined the clinical presentation and course of X-linked agammaglobulinemia (X-LA) by means of a multi-center retrospective survey of 96 patients. Infections were the most common presenting feature of patients with X-LA. The most frequent infections involved the upper respiratory tract (75%), lower respiratory tract (65%), gastrointestinal ...
H M, Lederman, J A, Winkelstein
openaire   +2 more sources

Autoimmunity and Inflammation in X-linked Agammaglobulinemia [PDF]

Journal of Clinical Immunology, 2014
In the past, XLA was described as associated with several inflammatory conditions, but with adequate immune globulin treatment, these are presumed to have diminished. The actual prevalence is not known.A web-based patient survey was conducted December 2011- February 2012.
Hernandez-Trujillo, Vivian P, Scalchunes, Chris, Cunningham-Rundles, Charlotte, Ochs, Hans D, Bonilla, Francisco A, Paris, Ken, Yel, Leman, Sullivan, Kathleen E   +7 more
openaire   +5 more sources

Disseminated Spiroplasma apis Infection in Patient with Agammaglobulinemia, France

Emerging Infectious Diseases, 2018
We report a disseminated infection caused by Spiroplasma apis, a honeybee pathogen, in a patient in France who had X-linked agammaglobulinemia. Identification was challenging because initial bacterial cultures and direct examination by Gram staining were
Nicolas Etienne, Laurent Bret, Cécile Le Brun, Hervé Lecuyer, Josquin Moraly, Fanny Lanternier, Olivier Hermine, Agnès Ferroni, Marc Lecuit, Sabine Pereyre, Laure Beven, Olivier Lortholary   +11 more
doaj   +1 more source

Gastrointestinal Manifestations in X-linked Agammaglobulinemia [PDF]

Journal of Clinical Immunology, 2017
X-linked agammaglobulinemia is a primary humoral immunodeficiency characterized by hypogammaglobulinemia and increased susceptibility to infection. Although there is increased awareness of autoimmune and inflammatory complications in X-linked agammaglobulinemia (XLA), the spectrum of gastrointestinal manifestations has not previously been fully ...
Sara Barmettler, Iris M. Otani, Jasmit Minhas, Roshini S. Abraham, Yenhui Chang, Morna J. Dorsey, Zuhair K. Ballas, Francisco A. Bonilla, Hans D. Ochs, Jolan E. Walter   +9 more
openaire   +2 more sources

Gastrointestinal disorders next to respiratory infections as leading symptoms of X-linked agammaglobulinemia in children – 34-year experience of a single center [PDF]

, 2016
Introduction: Respiratory tract infections constitute the most frequent manifestation of X-linked agammaglobulinemia (XLA). There are not many papers elucidating gastrointestinal (GI) disorders, including inflammatory bowel disease (IBD), in such ...
Malgorzata M. Pac, Józef P. Ryżko, Teresa Jackowska, Ewa A. Bernatowska, Jarosław Kierkuś, Joanna Cielecka-Kuszyk, Bożena Mikołuć   +6 more
core   +1 more source

X-linked agammaglobulinemia

Medicine, 2006
X-linked agammaglobulinemia (XLA) is a primary immunodeficiency caused by mutations in the gene for Bruton tyrosine kinase (BTK) that result in the deficient development of B lymphocytes and hypogammaglobulinemia. Because the disorder is uncommon, no single institution has had sufficient numbers of patients to develop a comprehensive clinical picture ...
Jerry A, Winkelstein, Mary C, Marino, Howard M, Lederman, Stacie M, Jones, Kathleen, Sullivan, A Wesley, Burks, Mary Ellen, Conley, Charlotte, Cunningham-Rundles, Hans D, Ochs   +8 more
openaire   +3 more sources

X-linked agammaglobulinemia with bronchiectasis and infection： a case report [PDF]

Zhenduanxue lilun yu shijian
X-linked agammaglobulinemia (XLA) is a rare disease characterized by severe hypogammaglobulinemia, antibody deficiency, and recurrent infections. This study reports a case of X-linked agammaglobulinemia combined with bronchiectasis and infection.
LIN Jiayuan, CHENG Qijian, CHEN Ling
doaj   +1 more source

Phylogeny of Tec Family Kinases: Identification of a Pre-Metazoan Origin of Btk, Bmx, Itk, Tec, Txk and the Btk Regulator SH3BP5 [PDF]

, 2008
It is generally considered mammals and birds have five Tec family kinases (TFKs): Btk, Bmx (also known as Etk), Itk, Tec, and Txk (also known as Rlk). Here, we discuss the domains and their functions and regulation in TFKs.
Ortutay, Csaba, Beston F. Nore, Nore, Beston F., Vihinen, Mauno, C.I. Edvard Smith, Smith, C. I. Edvard, Csaba Ortutay, Mauno Vihinen   +7 more
core   +1 more source

Close linkage of probe p212 (DXS178) to X-linked agammaglobulinemia. [PDF]

, 1989
Segregation analysis was performed in three families affected in X-linked agammaglobulinemia (XLA) with five polymorphic DNA probes linked to the disease locus.
DUSE, MARZIA, S. Guioli, B. Arveiler, B. Bardoni, I. Oberlé, A. Ugazio, G. d. Saint, L. D. Notarangelo, J. L. Mandel, P. Panina   +9 more
core   +3 more sources

Clinical, Molecular, Immunological Properties and Our Clinical Experiences in Patients Diagnosed with X-linked Agamaglobulinemia

Journal of Behçet Uz Children's Hospital, 2023
Objective: As a primary immunodeficiency X-linked agammaglobulinemia (XLA) that develops due to Bruton tyrosine kinase signal transduction protein deficiency which progresses with antibody deficiency was firstly described by an American pediatrician ...
Ezgi Balkarlı, Selime Özen Bölük, Ömer Akçal, İlke Taşkırdı, İdil Akay Haci, Nesrin Gülez, Ferah Genel   +6 more
doaj   +1 more source