Results 91 to 100 of about 8,781 (227)

X-linked agammaglobulinemia: clinical and immunologic evaluation of six patients

The Turkish Journal of Pediatrics, 1990
The clinical and immunologic features of six patients with X-linked agammaglobulinemia (XLA) are presented. The most common presenting manifestations were respiratory and gastrointestinal tract infections.
F Ersoy, O Sanal, I Tezcan, A I Berkel
doaj  

Relapsing Campylobacter jejuni Systemic Infections in a Child with X-Linked Agammaglobulinemia

Case Reports in Pediatrics, 2013
X-linked agammaglobulinemia (XLA) is a primary immunodeficiency of the humoral compartment, due to a mutation in the Bruton tyrosine kinase (BTK) gene, characterized by a severe defect of circulating B cells and serum immunoglobulins.
Paola Ariganello, Giulia Angelino, Alessia Scarselli, Irene Salfa, Martina Della Corte, Arianna De Matteis, Patrizia D'Argenio, Susanna Livadiotti, Emma C. Manno, Cristina Russo, Andrea Finocchi, Caterina Cancrini   +11 more
doaj   +1 more source

X-Linked Lymphoproliferative Disease Type 1: A Clinical and Molecular Perspective [PDF]

, 2018
X-linked lymphoproliferative disease (XLP) was first described in the 1970s as a fatal lymphoproliferative syndrome associated with infection with Epstein–Barr virus (EBV).
Baldanzi, Bar, Bergsten, Bloch-Queyrat, Booth, Booth, Booth, Bottino, Candotti, Cannon, Cannons, Cannons, Cannons, Cannons, Chan, Chan, Chen, Chu, Chung, Coffey, Cohen, Crotty, Crotty, Czar, Das, Dong, Dong, Dupré, Dutz, Freeman, Gadue, Georgiadis, Ghosh, Gilmour, Grierson, Griewank, Harada, Harrington, Henter, Hislop, Holt, Hron, Jordan, Kageyama, Kanegane, Kim, Kohn, Lankester, Latour, Latour, Li, Loeffel, Ma, Ma, Marsh, Marsh, McCausland, Mejstríková, Menard, Milone, Morra, Nakajima, Nichols, Nichols, Nichols, Ochs, Pachlopnik Schmid, Palendira, Palendira, Parolini, Pasquier, Pawson, Porteus, Poy, Provisor, Purtilo, Purtilo, Qasim, Qi, Rigaud, Rivat, Rougemont, Ruffo, Sayos, Schwartzberg, Seemayer, Sepulveda, Shlapatska, Snow, Sullivan, Sumegi, Sumegi, Talaat, Tangye, Tangye, Tangye, Thorley-Lawson, Veillette, Victora, Worth, Wu, Wu, Wu, Yin, Zhang, Zhao   +105 more
core   +1 more source

X-linked Agammaglobulinemia - Is it Really Rare?

Clinical Pediatrics: Open Access, 2018
Background: X-Linked Agammaglobulinemia is a disease of the immune system in which there is defective development of the B lymphocytes due to which the production of gammaglobulins is markedly reduced; which results in immunodeficiency and high vulnerability to contract fatal infections.
Hui-Ma, Asfia Banu Pasha, Ya-Yuan Zhang, Guo-Ping Zhou, Xiao-Qing Chen, Hai – Guo Yu   +5 more
openaire   +2 more sources

Valutazione della funzionalita' linfocitaria in soggetti con Immunodeficienza Comune Variabile [PDF]

, 2015
Con il termine Sindrome da Immunodeficienza (ID) si intende un insieme di segni e sintomi dovuti a un deficit del funzionamento del sistema immunitario che si traduce in una aumentata suscettibilità alle infezioni in termini di frequenza, durata e ...
SUTERA, DONATELLA
core  

Severe platelet dysfunction in NHL patients receiving ibrutinib is absent in patients receiving acalabrutinib [PDF]

, 2017
The Bruton’s tyrosine kinase (Btk) inhibitor ibrutinib induces platelet dysfunction and causes increased risk of bleeding. Off-target inhibition of Tec is believed to contribute to platelet dysfunction and other side-effects of ibrutinib.
Appleby, Niamh, Bruce, David, Bye, Alex P., Desborough, Michael J., Gibbins, Jon M., Hildyard, Catherine A. T., Hughes, Craig E., Kriek, Neline, Nock, Sophie H., Sage, Tanya, Unsworth, Amanda J.   +10 more
core   +2 more sources

Bone marrow cells in X-linked agammaglobulinemia express pre-B-specific genes (lambda-like and V pre-B) and present immunoglobulin V-D-J gene usage strongly biased to a fetal-like repertoire. [PDF]

, 1993
Michèle Milili, Françoise Le Deist, Geneviève de Saint Basile, Alain Fischer, M. Fougereau, Claudine Schiff   +5 more
openalex   +1 more source

Structural basis for chromosome X-linked agammaglobulinemia: a tyrosine kinase disease. [PDF]

, 1994
Mauno Vihinen, David Vetrie, Hina S. Maniar, HD Ochs, Qili Zhu, Igor Vořechovský, A D Webster, L. D. Notarangelo, Lennart Nilsson, Janusz M. Sowadski   +9 more
openalex   +1 more source

DEVELOPMENT OF REAL-TIME MULTIPLEX PCR FOR THE QUANTITATIVE DETERMINATION OF TREC'S AND KREC'S IN WHOLE BLOOD AND IN DRIED BLOOD SPOTS

Медицинская иммунология, 2015
Primary immunodeficiencies (PID) such as severe combined immunodeficiency (SCID) and X-linked agammaglobulinemia are characterized by the lack of functional Tand B-cells, respectively. Without early diagnosis and prompt treatment children with PID suffer
M. A. Gordukova, I. P. Oskorbin, O. V. Mishukova, S. B. Zimin, N. V. Zinovieva, N. V. Davydova, A. S. Smirnova, I. A. Nikitina, I. A. Korsunsky, M. L. Filipenko, A. P. Prodeus   +10 more
doaj   +1 more source

Immunoglobulin variable heavy chain cDNA sequence from a patient with X-linked agammaglobulinemia [PDF]

, 1991
Frank Mortari, Hans D. Ochs, Ralph Wedgwood, Harry W. Schroeder   +3 more
openalex   +1 more source