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Quoted phrase not found in phrase index: "Monocytopenia with susceptibility to infections"
Page 1
Mechanisms of resistance to hypomethylating agents and BCL-2 inhibitors.
Desai SR, Chakraborty S, Shastri A. Desai SR, et al. Best Pract Res Clin Haematol. 2023 Dec;36(4):101521. doi: 10.1016/j.beha.2023.101521. Epub 2023 Oct 20. Best Pract Res Clin Haematol. 2023. PMID: 38092478 Review.
Hypomethylating agents(HMA), azacytidine and decitabine are the first line agents for treatment of MDS and a combination with BCL-2 inhibitor, venetoclax, is approved for AML induction in patients above 75 years and is also actively being investigated for use in high risk MDS. Re …
Hypomethylating agents(HMA), azacytidine and decitabine are the first line agents for treatment of MDS and a combination with BCL-2 inhibito …
A novel GATA2 distal enhancer mutation results in MonoMAC syndrome in 2 second cousins.
West RR, Bauer TR, Tuschong LM, Embree LJ, Calvo KR, Tillo D, Davis J, Holland SM, Hickstein DD. West RR, et al. Blood Adv. 2023 Oct 24;7(20):6351-6363. doi: 10.1182/bloodadvances.2023010458. Blood Adv. 2023. PMID: 37595058 Free PMC article.
GATA2 mutations are found in 90% of patients with a GATA2 deficiency phenotype and are largely missense mutations in the conserved second zinc-finger domain. Mutations in an intron 5 regulatory enhancer element are also well described in GATA2 defic …
GATA2 mutations are found in 90% of patients with a GATA2 deficiency phenotype and are largely missense mutations in th …
Epstein-Barr Virus+ Smooth Muscle Tumors as Manifestation of Primary Immunodeficiency Disorders.
Magg T, Schober T, Walz C, Ley-Zaporozhan J, Facchetti F, Klein C, Hauck F. Magg T, et al. Front Immunol. 2018 Feb 27;9:368. doi: 10.3389/fimmu.2018.00368. eCollection 2018. Front Immunol. 2018. PMID: 29535735 Free PMC article. Review.
However, in a small fraction of predominantly pediatric patients, EBV(+) SMTs may occur in patients with primary immunodeficiency disorders (PIDs), such as GATA2 and CARMIL2 deficiency. In secondary immunodeficiencies and when the underlying condition can not be cur …
However, in a small fraction of predominantly pediatric patients, EBV(+) SMTs may occur in patients with primary immunodeficiency disorders …
Morbidity, Mortality, and Therapeutics in Combined Immunodeficiency: Data From the USIDNET Registry.
Durkee-Shock J, Zhang A, Liang H, Wright H, Magnusson J, Garabedian E, Marsh RA, Sullivan KE, Keller MD; USIDNET Consortium. Durkee-Shock J, et al. J Allergy Clin Immunol Pract. 2022 May;10(5):1334-1341.e6. doi: 10.1016/j.jaip.2022.01.042. Epub 2022 Feb 13. J Allergy Clin Immunol Pract. 2022. PMID: 35172220
RESULTS: On univariate analysis, disseminated invasive viral infections and variants in STAT3, GATA2, and, DOCK8 were associated with increased odds of HSCT. Mucocutaneous fungal infections and variants in STAT3 were associated with increased odds of survival …
RESULTS: On univariate analysis, disseminated invasive viral infections and variants in STAT3, GATA2, and, DOCK8 were associat …
Control of erythropoietin gene expression and its use in medicine.
Jelkmann W. Jelkmann W. Methods Enzymol. 2007;435:179-97. doi: 10.1016/S0076-6879(07)35010-6. Methods Enzymol. 2007. PMID: 17998055 Review.
Erythropoietin (EPO) gene expression is under the control of inhibitory (GATA-2, NF-kappaB) and stimulatory (hypoxia-inducible transcription factor [HIF]-2, hepatocyte nuclear factor [HNF]-4alpha [alpha]) transcription factors. EPO deficiency is the main cause of the anemi …
Erythropoietin (EPO) gene expression is under the control of inhibitory (GATA-2, NF-kappaB) and stimulatory (hypoxia-inducible transcription …
MicroRNA-Based Therapy of GATA2-Deficient Vascular Disease.
Hartmann D, Fiedler J, Sonnenschein K, Just A, Pfanne A, Zimmer K, Remke J, Foinquinos A, Butzlaff M, Schimmel K, Maegdefessel L, Hilfiker-Kleiner D, Lachmann N, Schober A, Froese N, Heineke J, Bauersachs J, Batkai S, Thum T. Hartmann D, et al. Circulation. 2016 Dec 13;134(24):1973-1990. doi: 10.1161/CIRCULATIONAHA.116.022478. Epub 2016 Oct 25. Circulation. 2016. PMID: 27780851
Global miRNAnome-screening identified several GATA2-regulated miRNAs including miR-126 and miR-221. Specifically, proangiogenic miR-126 was regulated by GATA2 transcriptionally and targeted antiangiogenic SPRED1 and FOXO3a contributing to GATA2-mediated forma …
Global miRNAnome-screening identified several GATA2-regulated miRNAs including miR-126 and miR-221. Specifically, proangiogenic miR-1 …
Germline GATA2 variant disrupting endothelial eNOS function and angiogenesis can be restored by c-Jun/AP-1 upregulation.
Purgatorio G, Piselli E, Guglielmini G, Falcinelli E, Bury L, Di Battista V, Pellanera F, Milano F, Matteucci C, Mecucci C, Gresele P. Purgatorio G, et al. Haematologica. 2022 May 1;107(5):1072-1085. doi: 10.3324/haematol.2021.278450. Haematologica. 2022. PMID: 34233447 Free PMC article.
Among the spectrum of GATA2 deficiency- associated manifestations thrombosis has been reported in 25% of patients, but the mechanisms are unknown. ...Given the ability of atorvastatin to restore NO production and angiogenesis by GATA2-deficient endothelial ce …
Among the spectrum of GATA2 deficiency- associated manifestations thrombosis has been reported in 25% of patients, but the mec …
Somatic GATA2 mutations define a subgroup of myeloid malignancy patients at high risk for invasive fungal disease.
Vedula RS, Cheng MP, Ronayne CE, Farmakiotis D, Ho VT, Koo S, Marty FM, Lindsley RC, Bold TD. Vedula RS, et al. Blood Adv. 2021 Jan 12;5(1):54-60. doi: 10.1182/bloodadvances.2020002854. Blood Adv. 2021. PMID: 33570623 Free PMC article.
Invasive fungal disease (IFD) can be a severe treatment complication in patients with myeloid malignancies, but current risk models do not incorporate disease-specific factors, such as somatic gene mutations. Germline GATA2 deficiency is associated with a suscept …
Invasive fungal disease (IFD) can be a severe treatment complication in patients with myeloid malignancies, but current risk models do not i …
Possible mechanisms by which silkworm faeces extract ameliorates adenine-induced renal anaemia in rats.
Mei H, Wu N, Huang X, Cui Z, Xu J, Yang X, Zeng F, Wang K. Mei H, et al. J Ethnopharmacol. 2021 Feb 10;266:113448. doi: 10.1016/j.jep.2020.113448. Epub 2020 Oct 3. J Ethnopharmacol. 2021. PMID: 33022342
ETHNOPHARMACOLOGICAL RELEVANCE: Silkworm faeces are the dry faeces of the insect Bombyx mori (Linnaeus) and have historically been used in traditional Chinese medicine to treat blood deficiency and rheumatic pain. Silkworm faeces extract (SFE) is derived from silkworm faec …
ETHNOPHARMACOLOGICAL RELEVANCE: Silkworm faeces are the dry faeces of the insect Bombyx mori (Linnaeus) and have historically been used in t …
Mediastinal and Disseminated Mycobacterium kansasii Disease in GATA2 Deficiency.
Lovell JP, Zerbe CS, Olivier KN, Claypool RJ, Frein C, Anderson VL, Freeman AF, Holland SM. Lovell JP, et al. Ann Am Thorac Soc. 2016 Dec;13(12):2169-2173. doi: 10.1513/AnnalsATS.201603-207BC. Ann Am Thorac Soc. 2016. PMID: 27607353 Free PMC article.
OBJECTIVES: To identify common clinical manifestations and potential risk factors for M. kansasii infection in patients with GATA2 deficiency. ...MEASUREMENTS AND MAIN RESULTS: Nine patients with GATA2 deficiency developed M. kansasii infecti …
OBJECTIVES: To identify common clinical manifestations and potential risk factors for M. kansasii infection in patients with GATA2 …
41 results