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Quoted phrase not found in phrase index: "Immunodeficiency 87 and autoimmunity"
Page 1
Interim analysis: Open-label extension study of leniolisib for patients with APDS.
Rao VK, Kulm E, Šedivá A, Plebani A, Schuetz C, Shcherbina A, Dalm VA, Trizzino A, Zharankova Y, Webster S, Orpia A, Körholz J, Lougaris V, Rodina Y, Radford K, Bradt J, Relan A, Holland SM, Lenardo MJ, Uzel G. Rao VK, et al. J Allergy Clin Immunol. 2024 Jan;153(1):265-274.e9. doi: 10.1016/j.jaci.2023.09.032. Epub 2023 Oct 4. J Allergy Clin Immunol. 2024. PMID: 37797893 Free article.
Resultant immune deficiency and dysregulation lead to recurrent sinopulmonary infections, herpes viremia, autoimmunity, and lymphoproliferation. ...At the data cutoff date (December 13, 2021), median leniolisib exposure was 102 weeks. Overall, 32 patients (87 …
Resultant immune deficiency and dysregulation lead to recurrent sinopulmonary infections, herpes viremia, autoimmunity, and ly …
Clinical and laboratory features associated with macrophage activation syndrome in Still's disease: data from the international AIDA Network Still's Disease Registry.
Triggianese P, Vitale A, Lopalco G, Mayrink Giardini HA, Ciccia F, Al-Maghlouth I, Ruscitti P, Sfikakis PP, Iannone F, de Brito Antonelli IP, Patrone M, Asfina KN, Di Cola I, Laskari K, Gaggiano C, Tufan A, Sfriso P, Dagna L, Giacomelli R, Hinojosa-Azaola A, Ragab G, Fotis L, Direskeneli H, Spedicato V, Dagostin MA, Iacono D, Ali HH, Cipriani P, Sota J, Kardas RC, Bindoli S, Campochiaro C, Navarini L, Gentileschi S, Martín-Nares E, Torres-Ruiz J, Saad MA, Kourtesi K, Alibaz-Oner F, Sevik G, Iagnocco A, Makowska J, Govoni M, Monti S, Maggio MC, La Torre F, Del Giudice E, Hernández-Rodríguez J, Bartoloni E, Emmi G, Chimenti MS, Maier A, Simonini G, Conti G, Olivieri AN, Tarsia M, De Paulis A, Lo Gullo A, Więsik-Szewczyk E, Viapiana O, Ogunjimi B, Tharwat S, Erten S, Nuzzolese R, Karamanakos A, Frassi M, Conforti A, Caggiano V, Marino A, Sebastiani GD, Gidaro A, Tombetti E, Carubbi F, Rubegni G, Cartocci A, Balistreri A, Fabiani C, Frediani B, Cantarini L. Triggianese P, et al. Intern Emerg Med. 2023 Nov;18(8):2231-2243. doi: 10.1007/s11739-023-03408-3. Epub 2023 Oct 12. Intern Emerg Med. 2023. PMID: 37828268 Free PMC article.
Patients with Still's disease classified according to internationally accepted criteria were enrolled in the AutoInflammatory Disease Alliance (AIDA) Still's Disease Registry. Clinical and laboratory features observed during the inflammatory attack complicated by MAS were …
Patients with Still's disease classified according to internationally accepted criteria were enrolled in the AutoInflammatory Disease Allian …
Comparing the levels of CTLA-4-dependent biological defects in patients with LRBA deficiency and CTLA-4 insufficiency.
Catak MC, Akcam B, Bilgic Eltan S, Babayeva R, Karakus IS, Akgun G, Baser D, Bulutoglu A, Bayram F, Kasap N, Kiykim A, Hancioglu G, Kokcu Karadag SI, Kendir Demirkol Y, Ozen S, Cekic S, Ozcan D, Edeer Karaca N, Sasihuseyinoglu AS, Cansever M, Ozek Yucel E, Tamay Z, Altintas DU, Aydogmus C, Celmeli F, Cokugras H, Gulez N, Genel F, Metin A, Guner SN, Kutukculer N, Keles S, Reisli I, Kilic SS, Yildiran A, Karakoc-Aydiner E, Lo B, Ozen A, Baris S. Catak MC, et al. Allergy. 2022 Oct;77(10):3108-3123. doi: 10.1111/all.15331. Epub 2022 May 12. Allergy. 2022. PMID: 35491430
We sought to compare the clinical and laboratory manifestations of both diseases and investigate the role of flow cytometry in predicting the genetic defect in patients with LRBA deficiency and CTLA-4 insufficiency. ...The T-cell subsets showed more deviation to mem …
We sought to compare the clinical and laboratory manifestations of both diseases and investigate the role of flow cytometry in predicting …
Hypogammaglobulinemia: a diagnosis that must not be overlooked.
Pimenta FMCA, Palma SMU, Constantino-Silva RN, Grumach AS. Pimenta FMCA, et al. Braz J Med Biol Res. 2019 Oct 10;52(10):e8926. doi: 10.1590/1414-431X20198926. eCollection 2019. Braz J Med Biol Res. 2019. PMID: 31618370 Free PMC article.
Hypothyroidism was identified in 4/8 (50%) patients. Rhinitis was found in 7/8 (87.5%) and asthma in 3/8 (37.5%) patients. The tomographic findings were consolidations, atelectasis, emphysema, ground glass opacity, budding tree, bronchial thickening, and bronchiectasis. Im …
Hypothyroidism was identified in 4/8 (50%) patients. Rhinitis was found in 7/8 (87.5%) and asthma in 3/8 (37.5%) patients. The tomogr …
The GAIN Registry - a New Prospective Study for Patients with Multi-organ Autoimmunity and Autoinflammation.
Staus P, Rusch S, El-Helou S, Müller G, Krausz M, Geisen U, Caballero-Oteyza A, Krüger R, Bakhtiar S, Lee-Kirsch MA, Fasshauer M, Baumann U, Hoyer BF, Farela Neves J, Borte M, Carrabba M, Hauck F, Ehl S, Bader P, von Bernuth H, Atschekzei F, Seppänen MRJ, Warnatz K, Nieters A, Kindle G, Grimbacher B. Staus P, et al. J Clin Immunol. 2023 Aug;43(6):1289-1301. doi: 10.1007/s10875-023-01472-0. Epub 2023 Apr 21. J Clin Immunol. 2023. PMID: 37084016 Free PMC article.
Of 354 (84.5%) patients who were genetically tested, 248 (59.2%) had a defined monogenetic cause. For 87 (20.8%) patients, no mutation was found and for 19 (4.5%), the result was pending. ...The GAIN registry may serve as a valuable resource for research in the inborn erro …
Of 354 (84.5%) patients who were genetically tested, 248 (59.2%) had a defined monogenetic cause. For 87 (20.8%) patients, no mutatio …
Serum anti-tissue transglutaminase IgA and prediction of duodenal villous atrophy in adults with suspected coeliac disease without IgA deficiency (Bi.A.CeD): a multicentre, prospective cohort study.
Ciacci C, Bai JC, Holmes G, Al-Toma A, Biagi F, Carroccio A, Ciccocioppo R, Di Sabatino A, Gingold-Belfer R, Jinga M, Makharia G, Niveloni S, Norman GL, Rostami K, Sanders DS, Smecuol E, Villanacci V, Vivas S, Zingone F; Bi.A.CeD study group. Ciacci C, et al. Lancet Gastroenterol Hepatol. 2023 Nov;8(11):1005-1014. doi: 10.1016/S2468-1253(23)00205-4. Epub 2023 Sep 9. Lancet Gastroenterol Hepatol. 2023. PMID: 37696284
Histology was evaluated by the local pathologist, with discordant cases (positive tTG-IgA without duodenal villous atrophy or negative tTG-IgA with duodenal villous atrophy) re-evaluated by a central pathologist. The reliability of serum tests for the prediction of duodena …
Histology was evaluated by the local pathologist, with discordant cases (positive tTG-IgA without duodenal villous atrophy or negative tTG-I …
Clinical, Immunological, and Genetic Features in Patients with Immune Dysregulation, Polyendocrinopathy, Enteropathy, X-linked (IPEX) and IPEX-like Syndrome.
Jamee M, Zaki-Dizaji M, Lo B, Abolhassani H, Aghamahdi F, Mosavian M, Nademi Z, Mohammadi H, Jadidi-Niaragh F, Rojas M, Anaya JM, Azizi G. Jamee M, et al. J Allergy Clin Immunol Pract. 2020 Sep;8(8):2747-2760.e7. doi: 10.1016/j.jaip.2020.04.070. Epub 2020 May 16. J Allergy Clin Immunol Pract. 2020. PMID: 32428713
METHODS: The literature search was performed in PubMed, Web of Science, and Scopus databases, and demographic, clinical, immunologic, and molecular data were compared between the IPEX and IPEX-like groups. ...CONCLUSIONS: Patients with IPEX syndrome generally suffer from e …
METHODS: The literature search was performed in PubMed, Web of Science, and Scopus databases, and demographic, clinical, immunologic, …
Prevalence and factors associated with latent autoimmune diabetes in adults (LADA): a cross-sectional study.
Manisha AM, Shangali AR, Mfinanga SG, Mbugi EV. Manisha AM, et al. BMC Endocr Disord. 2022 Jul 8;22(1):175. doi: 10.1186/s12902-022-01089-1. BMC Endocr Disord. 2022. PMID: 35804315 Free PMC article.
BACKGROUND: The Latent Autoimmune Diabetes in Adults (LADA) is a slowly progressive Type 1 diabetes subgroup with onset during middle age. ...The parameters such as mean age, family history of diabetes mellitus status, Fasting Blood Glucose, clinical characteristics, and c …
BACKGROUND: The Latent Autoimmune Diabetes in Adults (LADA) is a slowly progressive Type 1 diabetes subgroup with onset during middle …
Who's your data? Primary immune deficiency differential diagnosis prediction via machine learning and data mining of the USIDNET registry.
Méndez Barrera JA, Rocha Guzmán S, Hierro Cascajares E, Garabedian EK, Fuleihan RL, Sullivan KE, Lugo Reyes SO. Méndez Barrera JA, et al. Clin Immunol. 2023 Oct;255:109759. doi: 10.1016/j.clim.2023.109759. Epub 2023 Sep 9. Clin Immunol. 2023. PMID: 37678719
RESULTS: The twelve PID diagnoses were CVID (1098 patients), DiGeorge syndrome, Chronic granulomatous disease, Congenital agammaglobulinemia, PID not otherwise classified, Specific antibody deficiency, Complement deficiency, Hyper-IgM, Leukocyte adhesion deficien …
RESULTS: The twelve PID diagnoses were CVID (1098 patients), DiGeorge syndrome, Chronic granulomatous disease, Congenital agammaglobulinemia …
Prognostic markers for immunodeficiency-associated primary central nervous system lymphoma.
Kaulen LD, Galluzzo D, Hui P, Barbiero F, Karschnia P, Huttner A, Fulbright R, Baehring JM. Kaulen LD, et al. J Neurooncol. 2019 Aug;144(1):107-115. doi: 10.1007/s11060-019-03208-w. Epub 2019 Jun 13. J Neurooncol. 2019. PMID: 31190317
We aimed to define clinical and imaging features and determine prognostic factors for immunodeficiency-associated PCNSL. METHODS: All PCNSL cases seen at Yale-New Haven Hospital between 2002 and 2017 were retrospectively screened for immunodeficiency. ...RESULTS: 23 …
We aimed to define clinical and imaging features and determine prognostic factors for immunodeficiency-associated PCNSL. METHODS: All …
46 results