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001-es BibID:	BIBFORM111993
035-os BibID:	(cikkazonosító)1149 (WoS)001016877300001 (Scopus)85163968050
Első szerző:	Balázs Gergely (csecsemő- és gyermekgyógyász, neonatológus)
Cím:	Prevention of Chronic Morbidities in Extremely Premature Newborns with LISA-nCPAP Respiratory Therapy and Adjuvant Perinatal Strategies / Gergely Balázs, András Balajthy, István Seri, Thomas Hegyi, Tibor Ertl, Tamás Szabó, Tamás Röszer, Ágnes Papp, József Balla, Tamás Gáll, György Balla
Dátum:	2023
ISSN:	2076-3921
Megjegyzések:	Less invasive surfactant administration techniques, together with nasal continuous airway pressure (LISA-nCPAP) ventilation, an emerging noninvasive ventilation (NIV) technique in neonatology, are gaining more significance, even in extremely premature newborns (ELBW), under 27 weeks of gestational age. In this review, studies on LISA-nCPAP are compiled with an emphasis on short- and long-term morbidities associated with prematurity. Several perinatal preventative and therapeutic investigations are also discussed in order to start integrated therapies as numerous organ-saving techniques in addition to lung-protective ventilations. Two thirds of immature newborns can start their lives on NIV, and one third of them never need mechanical ventilation. With adjuvant intervention, these ratios are expected to be increased, resulting in better outcomes. Optimized cardiopulmonary transition, especially physiologic cord clamping, could have an additively beneficial effect on patient outcomes gained from NIV. Organ development and angiogenesis are strictly linked not only in the immature lung and retina, but also possibly in the kidney, and optimized interventions using angiogenic growth factors could lead to better morbidity-free survival. Corticosteroids, caffeine, insulin, thyroid hormones, antioxidants, N-acetylcysteine, and, moreover, the immunomodulatory components of mother's milk are also discussed as adjuvant treatments, since immature newborns deserve more complex neonatal interventions.
Tárgyszavak:	Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban folyóiratcikk
Megjelenés:	Antioxidants. - 12 : 6 (2023), p. 1-36. -
További szerzők:	Balajthy András (1988-) (általános orvos) Seri István Hegyi Thomas Ertl Tibor Szabó Tamás (1968-) (gyermekgyógyász) Röszer Tamás (1979-) (orvos, biológus) Papp Ágnes (1967-) (gyermekgyógyász, pulmonológus) Balla József (1959-) (belgyógyász, nephrológus) Gáll Tamás (1982-) (molekuláris biológus, mikrobiológus) Balla György (1953-) (csecsemő és gyermekgyógyász, neonatológus)
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001-es BibID:	BIBFORM066281
Első szerző:	Balogh Enikő (molekuláris biológus)
Cím:	Impaired Immunosuppressive Effect of Bronchoalveolar Mesenchymal Stem Cells in Hypersensitivity Pneumonitis : preliminary findings / Eniko Balogh, Bela Nagy Jr., Agnes Gyetvai, Zsolt Bene, Zoltan Hendrik, Viktoria Jeney, Peter Nagy, Agnes Papp, Jozsef Balla, Gyorgy Balla, Janos Kappelmayer, Bela Nagy
Dátum:	2018
ISSN:	1552-4949
Megjegyzések:	Bronchoalveolar mesenchymal stem cells (MSCs) play an important role in the maintenance of lung integrity. Therapeutic application of bone marrow-derived MSCs reduced chronic bronchial inflammation in idiopathic pulmonary fibrosis, and improved the ratio of survivors in sepsis with pneumonia. This study investigated the effect of MSCs from bronchoalveolar lavage fluid (BALF) of hypersensitivity pneumonitis (HP) on T-cell function under in vitro conditions.METHODS:Bronchoalveolar MSCs were obtained via bronchoscopy with BAL from children with severe subacute HP. As control, BALF MSCs were assessed from children without any inflammatory lung disease. Isolated MSCs were characterized via immunophenotyping by flow cytometry and confocal laser scanning microscopy. HP-derived and healthy separated peripheral blood mononuclear cells (PBMCs) were stimulated by 5 ?g/mL phytohemagglutinin in the presence of HP-derived or control MSCs in 5-day cultures. Proliferation and activation of T-cells were characterized by the mean fluorescence intensity (MFI) of 5,6-carboxyfluorescein-diacetat succinimidyl ester (CFSE) and CD25, CD69 as well as HLA-DR surface positivities, respectively.RESULTS:HP-derived MSCs showed significantly lower level of CD73, CD90, and CD105 expression compared to control MSCs in both flow cytometric and confocal microscopic experiments. MSCs from HP did not reduce T-cell proliferation based on CFSE MFI values, while the level of CD25 expression on both control and HP-derived CD4+ and CD8+ T-cells was significantly reduced by normal MSCs, while HP-derived MSCs did not have any significant effect. The level of other activation markers was not markedly modulated by MSCs.CONCLUSIONS:BALF MSCs from HP are unable to downregulate the proliferation and activation of T-cells that may support the development of recurrent intrapulmonary inflammation in HP. ? 2016 Clinical Cytometry Society.
Tárgyszavak:	Orvostudományok Elméleti orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban T-lymphocyte proliferation and activation bronchoalveolar lavage hypersensitivity pneumonitis mesenchymal stem cells
Megjelenés:	Cytometry Part B-Clinical Cytometry. - 94 : 2 (2018), p. 363-368. -
További szerzők:	Nagy Béla Jr. (1980-) (labordiagnosztikai szakorvos) Gyetvai Ágnes Bene Zsolt (1981-) (orvos) Hendrik Zoltán (1986-) (orvos) Jeney Viktória (1971-) (vegyész, kémia tanár) Nagy Péter (1971-) (biofizikus) Papp Ágnes (1967-) (gyermekgyógyász, pulmonológus) Balla József (1959-) (belgyógyász, nephrológus) Balla György (1953-) (csecsemő és gyermekgyógyász, neonatológus) Kappelmayer János (1960-) (laboratóriumi szakorvos) Nagy Béla (1949-) (csecsemő- és gyermekgyógyász, gyermek-tüdőgyógyász)
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001-es BibID:	BIBFORM090236
Első szerző:	Sümegi Andrea (biológus)
Cím:	A novel splice site indel alteration in the EIF2AK3 gene is responsible for the first cases of Wolcott-Rallison syndrome in Hungary / Sümegi Andrea, Hendrik Zoltán, Gáll Tamás, Felszeghy Enikő, Szakszon Katalin, Antal-Szalmás Péter, Beke Lívia, Papp Ágnes, Méhes Gábor, Balla József, Balla György
Dátum:	2020
ISSN:	1471-2350 1471-2350
Megjegyzések:	Background: Wolcott-Rallison Syndrome (WRS) is a rare autosomal recessive disease that is the most common cause of neonatal diabetes in consanguineous families. WRS is caused by various genetic alterations of the Eukaryotic Translation Initiation Factor 2-Alpha Kinase 3 (EIF2AK3) gene. Methods: Genetic analysis of a consanguineous family where two children were diagnosed with WRS was performed by Sanger sequencing. The altered protein was investigated by in vitro cloning, expression and immunohistochemistry. Results: The first cases in Hungary, ? two patients in one family, where the parents were fourth-degree cousins - showed the typical clinical features of WRS: early onset diabetes mellitus with hyperglycemia, growth retardation, infection-induced multiple organ failure. The genetic background of the disease was a novel alteration in the EIF2AK3 gene involving the splice site of exon 11? intron 11?12 boundary: g.53051_53062delinsTG. According to cDNA sequencing this created a new splice site and resulted in a frameshift and the development of an early termination codon at amino acid position 633 (p.Pro627AspfsTer7). Based on in vitro cloning and expression studies, the truncated protein was functionally inactive. Immunohistochemistry revealed that the intact protein was absent in the islets of pancreas, furthermore insulin expressing cells were also dramatically diminished. Elevated GRP78 and reduced CHOP protein expression were observed in the liver. Conclusions: The novel genetic alteration causing the absence of the EIF2AK3 protein resulted in insufficient handling of severe endoplasmic reticulum stress, leading to liver failure and demise of the patients. taa, km
Tárgyszavak:	Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban folyóiratcikk Wolcott-Rallison syndrome EIF2AK3 gene Endoplasmic reticulum stress PERK protein Splice site variant Indel alteration
Megjelenés:	BMC Medical Genetics. - 21 : 61 (2020), p. 1-12. -
További szerzők:	Hendrik Zoltán (1986-) (orvos) Gáll Tamás (1982-) (molekuláris biológus, mikrobiológus) Felszeghy Enikő Noémi (1970-) (gyermekgyógyász) Szakszon Katalin (1977-) (csecsemő- és gyermekgyógyász, klinikai genetikus) Antal-Szalmás Péter (1968-) (laboratóriumi szakorvos) Beke Lívia Papp Ágnes (1967-) (gyermekgyógyász, pulmonológus) Méhes Gábor (1966-) (patológus) Balla József (1959-) (belgyógyász, nephrológus) Balla György (1953-) (csecsemő és gyermekgyógyász, neonatológus)
Pályázati támogatás:	GINOP-2.3.2-15-2016-00043 GINOP EFOP-3.6.2-16-2017-00006 EFOP OTKA-112233 OTKA ED_18-1-2019-0028 Egyéb
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