Results 71 to 80 of about 163,168 (216)

Late diagnosis of agammaglobulinemia in an 8-year-old boy

Pediatria i Medycyna Rodzinna, 2016
Chromosome X-linked Bruton agammaglobulinemia is classified as a primary immunodeficiency disorder. It is a genetic condition associated with a mutation in the BTK gene encoding tyrosine kinase.
Małgorzata Sopińska, Karolina Kapusta, Edyta Heropolitańska-Pliszka, Anna Jung, Bolesław Kalicki   +4 more
doaj   +1 more source

Factors Affecting Immune Reconstitution Post‐Allogeneic HSCT in Children: The Case for an Individualized Approach to Vaccination

European Journal of Haematology, EarlyView.
ABSTRACT Allogeneic hematopoietic stem cell transplantation (HSCT) is increasingly used to treat malignant and non‐malignant diseases. Following allogeneic HSCT, patients are particularly vulnerable to vaccine‐preventable diseases (VPD) because conditioning depletes immune cells, including memory cells.
Hélène Buvelot, Frederic Baleydier, Laure Pittet, Geraldine Blanchard‐Rohner   +3 more
wiley   +1 more source

Six‐Year Trends in Real‐World Data Use for Post‐Marketing Surveillance of New Medical Products in Japan

Clinical and Translational Science, Volume 19, Issue 2, February 2026.
ABSTRACT The Ministerial Ordinance on Good Post‐Marketing Study Practice for Drugs was amended by the Ministry of Health, Labour and Welfare (MHLW) in 2018 to clearly define post‐marketing database studies (DBS) as a measure of pharmacovigilance activities for approved medical products in Japan.
Suguru Okami, Kako Shimotsumagari, Yasunari Sadatsuki   +2 more
wiley   +1 more source

Pseudomonas aeruginosa sepsis presenting as oral ecthyma gangrenosum in identical twins with Bruton tyrosine kinase gene mutation: Two case reports and review of the literature

Journal of Microbiology, Immunology and Infection, 2020
X-linked agammaglobulinemia (XLA) is a primary immunodeficiency disease. We reported two 7-month-old identical male twins with Pseudomonas aeruginosa sepsis who initially manifested as oral ecthyma gangrenosum and were finally diagnosed to have XLA.
Sheng-Chieh Lin, Bor-Luen Chiang, Yuarn-Jang Lee, Ya-Ting Chang, Shiuh-Bin Fang   +4 more
doaj   +1 more source

Haematopoietic Stem Cell Transplant for Norovirus-Induced Intestinal Failure in X-linked Agammaglobulinemia

Journal of Clinical Immunology, 2021
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, Mark J. Ponsford, M. Slatter, Jennifer A. Evans, S. Struik, M. Cosgrove, I. Doull, S. Jolles, A. Gennery   +8 more
semanticscholar   +1 more source

Good's Syndrome Presenting With Weaning Failure From Ventilation and Myasthenia Gravis in a Post‐Thymectomy Patient

Respirology Case Reports, Volume 13, Issue 11, November 2025.
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,
Asmita Anilkumar Mehta, Aditya Ashok, Lakshmi Priya Valathara Pradeep, Suchitra Sivadas, George Justin Mothi   +4 more
wiley   +1 more source

X-linked agammaglobulinemia and isolated growth hormone deficiency

The Turkish Journal of Pediatrics, 1998
X-linked agammaglobulinemia and isolated growth hormone deficiency was first described in 1980 and then classified as a different primary immune deficiency.
D Arslan, T Patiroğlu, M Kendirci, S Kurtoğlu   +3 more
doaj  

CVID Enteropathy Associated With Chronic Norovirus Infection: Background, Clinical Features, and Therapeutic Aspects

Reviews in Medical Virology, Volume 35, Issue 6, November 2025.
ABSTRACT Common variable immunodeficiency (CVID) is the most prevalent symptomatic primary immunodeficiency, characterised by impaired antibody production, immune dysregulation, and a broad spectrum of clinical manifestations. Gastrointestinal involvement is frequent, affecting up to 20% of patients and significantly contributing to morbidity and ...
Györgyi Műzes, Ferenc Sipos
wiley   +1 more source

Optimizing Lentiviral Vector Production: Insights Into PiggyBac Transposase and Concatemeric Array Strategies

Biotechnology Journal, Volume 20, Issue 10, October 2025.
Graphical Abstract and Lay Summary Transposase‐mediated integration provides a robust alternative for generating stable lentiviral vector producer cell lines. While yielding slightly lower maximum titers than concatemeric‐array methods, it requires less DNA, accelerates recovery, and delivers more consistent performance, supporting scalable LVV ...
Jona Röscheise, Maximilian Klimpel, Janina Hoffman, Vathsalya Pabbathi, Herbert Dersch, Parameswari Govindarajan, Holger Laux, Kerstin Otte   +7 more
wiley   +1 more source

Immune System‐Related Genetic Risk Factors for Inhibitory Antibody Development in Patients With Hemophilia: Reviewing an Old Problem From a New Perspective—A Narrative Review

Health Science Reports, Volume 8, Issue 10, October 2025.
ABSTRACT Background and Aims Hemophilia A and B are two of the most common bleeding disorders. Genetic risk factors are associated with the development of autoantibodies released in hemophilia patients against alternative factors and are the most important problems associated with the care of these patients. Objective In this study, we reviewed genetic
Fatemeh Zeylabi, Mojtaba Aghaei, Najmaldin Saki   +2 more
wiley   +1 more source