Severe B Cell Deficiency in Mice Lacking the Tec Kinase Family Members Tec and Btk [PDF]
The cytoplasmic protein tyrosine kinase Tec has been proposed to have important functions in hematopoiesis and lymphocyte signal transduction. Here we show that Tec-deficient mice developed normally and had no major phenotypic alterations of the immune ...
Baltimore, David +7 more
core +3 more sources
Community-acquired Acinetobacter calcoaceticus pneumonia in a patient with agammaglobulinaemia
We herein describe the case of a 38-year-old patient with congenital agammaglobulinemia who presented with community-acquired pneumonia; acute respiratory failure with sepsis ensued requiring ICU admission, mechanical ventilation and vasopressors ...
K. El Gharib +3 more
doaj +1 more source
Good's Syndrome Presenting With Weaning Failure From Ventilation and Myasthenia Gravis in a Post-Thymectomy Patient. [PDF]
This case highlights the importance of long‐term vigilance in patients with thymoma, even after apparently successful resection, as Good's syndrome may manifest years later. Clinicians should maintain a high index of suspicion for Good's syndrome in any thymectomized patient presenting with unexplained infections, diarrhoea, or autoimmune complications,
Mehta AA +4 more
europepmc +2 more sources
Genetic stability of Campylobacter coli in patients with primary antibody deficiencies [PDF]
In the Clinical Communication, Dion et al 1 reported that in patients with severe primary antibody deficiency (PAD), Campylobacter infection is a major cause (6.5%) of chronic or recurrent diarrhea. Moreover, by a molecular study performed in a subset of
Milito, Cinzia, Quinti, Isabella
core +1 more source
Clinical and Genetic Profile of X-Linked Agammaglobulinemia: A Multicenter Experience From India
Background There is paucity of literature on XLA from developing countries. Herein we report the clinical and molecular profile and outcome in a multicenter cohort of patients with XLA from India.
A. Rawat +41 more
semanticscholar +1 more source
Clinic of humoral primary immunodeficiencies in adults. Experience in a tertiary hospital
Background: Primary immunodeficiencies (PID) are characterized by alteration of the components of the immune system. Humoral deficiencies represent 50%.
Julio César Cambray-Gutiérrez +3 more
doaj +1 more source
Tubulointerstitial nephritis complicating IVIG therapy for X-linked agammaglobulinemia [PDF]
BACKGROUND: Patients with X-linked agammaglobulinemia (XLA) develop immune-complex induced diseases such as nephropathy only rarely, presumably because their immunoglobulin (Ig) G concentration is low.
Akane Izu +7 more
core +2 more sources
Since the first clinical description in 1952, immunoglobulin replacement therapy remains the mainstay of treatment of patients with X-linked agammaglobulinemia (XLA). However, this therapy only replaces IgG isotype and does not compensate for the loss of
B. Shillitoe +8 more
semanticscholar +1 more source
Fatal SARS in X-Linked Lymphoproliferative Disease Type 1: A Case Report
X-linked lymphoproliferative disease (XLP1) is an inborn error of immunity (IEI) with severe immune dysregulation caused by a mutation in the SH2D1A gene resulting in the absence or dysfunction of signaling lymphocytic activation molecule (SLAM ...
Ming Hin Chung +5 more
doaj +1 more source
Targeting Brutons Tyrosine Kinase in Chronic Lymphocytic Leukemia at the Crossroad between Intrinsic and Extrinsic Pro-survival Signals [PDF]
Chemo immunotherapies for chronic lymphocytic leukemia (CLL) showed a positive impact on clinical outcome, but many patients relapsed or become refractory to the available treatments.
Facco, Monica +7 more
core +1 more source

