📇 Rhumatologue ·
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Praticien-chercheur
7 articles scientifiques publiés — formation continue solide
✨ Génération du profil synthétique IA en cours…
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Drug safety · 2000
Journal of clinical immunology · 2020
AbstractThis guideline aims to describe the complement system and the functions of the constituent pathways, with particular focus on primary immunodeficiencies (PIDs) and their diagnosis and management. The complement system is a crucial part of the innate immune system, with multiple membrane-bound and soluble components. There are three distinct enzymatic cascade pathways within the complement system, the classical, alternative and lectin pathways, which converge with the cleavage of central C3. Complement deficiencies account for ~5% of PIDs. The clinical consequences of inherited defects in the complement system are protean and include increased susceptibility to infection, autoimmune diseases (e.g., systemic lupus erythematosus), age-related macular degeneration, renal disorders (e.g., atypical hemolytic uremic syndrome) and angioedema. Modern complement analysis allows an in-depth insight into the functional and molecular basis of nearly all complement deficiencies. However, therapeutic options remain relatively limited for the majority of complement deficiencies with the exception of hereditary angioedema and inhibition of an overactivated complement system in regulation defects. Current management strategies for complement disorders associated with infection include education, family testing, vaccinations, antibiotics and emergency planning.
Clinical and experimental immunology · 2019
Summary Good’s syndrome (thymoma and hypogammaglobulinaemia) is a rare secondary immunodeficiency disease, previously reported in the published literature as mainly individual cases or small case series. We use the national UK-Primary Immune Deficiency (UKPID) registry to identify a large cohort of patients in the UK with this PID to review its clinical course, natural history and prognosis. Clinical information, laboratory data, treatment and outcome were collated and analysed. Seventy-eight patients with a median age of 64 years, 59% of whom were female, were reviewed. Median age of presentation was 54 years. Absolute B cell numbers and serum immunoglobulins were very low in all patients and all received immunoglobulin replacement therapy. All patients had undergone thymectomy and nine (12%) had thymic carcinoma (four locally invasive and five had disseminated disease) requiring adjuvant radiotherapy and/or chemotherapy. CD4 T cells were significantly lower in these patients with malignant thymoma. Seventy-four (95%) presented with infections, 35 (45%) had bronchiectasis, seven (9%) chronic sinusitis, but only eight (10%) had serious invasive fungal or viral infections. Patients with AB-type thymomas were more likely to have bronchiectasis. Twenty (26%) suffered from autoimmune diseases (pure red cell aplasia, hypothyroidism, arthritis, myasthenia gravis, systemic lupus erythematosus, Sjögren’s syndrome). There was no association between thymoma type and autoimmunity. Seven (9%) patients had died. Good’s syndrome is associated with significant morbidity relating to infectious and autoimmune complications. Prospective studies are required to understand why some patients with thymoma develop persistent hypogammaglobulinaemia.
Source PubMed · Recherche par auteur (homonymes possibles, vérifier l'affiliation).
Rheumatology (Oxford, England) · 2021 · Journal Article
Cooray S, Omyinmi E, Hong Y, Papadopoulou C, et al.
Clinical and experimental immunology · 2019 · Journal Article
Zaman M, Huissoon A, Buckland M, Patel S, et al.
Clinical chemistry and laboratory medicine · 2018 · Journal Article
Tange CE, Johnson-Brett B, Cook A, Stordeur P, et al.
International immunopharmacology · 2006 · Journal Article
Jolles S, Hughes J
Journal of clinical immunology · 2020 · Journal Article
Brodszki N, Frazer-Abel A, Grumach AS, Kirschfink M, et al.
Drug safety · 2000 · Journal Article
Jolles S, Sewell WA, Leighton C
Journal of clinical immunology · 2023 · Journal Article
Jefferson L, Ramanan AV, Jolles S, Bernatoniene J, et al.