Receptor Activator of Nuclear Factor kappa-Beta Ligand/Osteoprotegerin Axis in Adults with Hb S/beta-Thalassemia and beta-Thalassemia Trait

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dc.authoridTOMBAK, ANIL/0000-0002-7195-1845
dc.contributor.authorTombak, Anil
dc.contributor.authorBoztepe, Burcu
dc.contributor.authorAkbayir, Serin
dc.contributor.authorDogru, Gurbet
dc.contributor.authorSungur, Mehmet Ali
dc.date.accessioned2021-12-01T18:48:02Z
dc.date.available2021-12-01T18:48:02Z
dc.date.issued2020
dc.department[Belirlenecek]en_US
dc.description.abstractThere is not enough data about osteoporosis and the role of receptor activator of nuclear factor kappa-Beta ligand (RANKL)/serum osteoprotegerin (OPG) system in patients with double heterozygosity for sickle cell disease and beta-thalassemia [Hb S (HBB: c.20A>T)/beta-thal] and beta-thal trait. Aim of the study was to investigate bone mineral densities (BMD) and the role of RANKL/OPG system in these cases. We studied 58 adults with Hb S/beta-thal, 52 adults with beta-thal trait, 34 healthy subjects as a control group. The BMD was determined by dual-energy X-ray absorptiometry (DEXA). Biochemical markers of bone metabolism (serum calcium, phosphorus, alkaline phosphatase, osteocalcin) parameters that affect bone metabolism (serum parathyroid hormone, thyroid-stimulating hormone, 25-hydroxyvitamin D, OPG, soluble RANKL [sRANKL]) were studied. Femoral neck Z-scores of 93.2% for beta-thal trait, 83.0% for Hb S/beta-thal patients were within the expected range. Lumbar spine Z-scores of 89.1% for beta-thal, 90.2% for Hb S/beta-thal patients were above -2.0 SD. Z-scores of the control group were within the expected range. Median serum sRANKL level was 2.80, 4.52, 5.79 pmol/L in Hb S/beta-thal, beta-thal trait, control groups, respectively (p = 0.010). Median serum OPG level was 1.07, 0.86, 0.86 pmol/L in Hb S/beta-thal, beta-thal trait, control groups, respectively (p < 0.001). beta-Thalassemia trait alone is not a risk factor for osteopenia/osteoporosis and osteoporosis does not develop in premenopausal women and men younger than 50 years with Hb S/beta-thal. However, as we determined lower levels of osteocalcin, compensatory decrease of sRANKL with compensatory increase of OPG, more severe osteoporosis may develop in advanced ages in these patient populations.en_US
dc.description.sponsorshipMersin UniversityMersin University [BAP-TF DTB (AT) 2010-3]en_US
dc.description.sponsorshipThis study was supported by the Mersin University Scientific Research Projects [BAP-TF DTB (AT) 2010-3 code].en_US
dc.identifier.doi10.1080/03630269.2020.1811116
dc.identifier.endpage337en_US
dc.identifier.issn0363-0269
dc.identifier.issn1532-432X
dc.identifier.issue5en_US
dc.identifier.pmid32873083en_US
dc.identifier.scopus2-s2.0-85090149538en_US
dc.identifier.scopusqualityQ3en_US
dc.identifier.startpage334en_US
dc.identifier.urihttps://doi.org/10.1080/03630269.2020.1811116
dc.identifier.urihttps://hdl.handle.net/20.500.12684/10442
dc.identifier.volume44en_US
dc.identifier.wosWOS:000564985500001en_US
dc.identifier.wosqualityQ4en_US
dc.indekslendigikaynakWeb of Scienceen_US
dc.indekslendigikaynakPubMeden_US
dc.indekslendigikaynakScopusen_US
dc.language.isoenen_US
dc.publisherTaylor & Francis Ltden_US
dc.relation.ispartofHemoglobinen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/closedAccessen_US
dc.subjectBone mineral density (BMD)en_US
dc.subjectosteoporosisen_US
dc.subjectosteoprotegerin (OPG)en_US
dc.subjectreceptor activator of nuclear factor kappa-Beta ligand (RANKL)en_US
dc.subjectsickle cell diseaseen_US
dc.subjectthalassemiaen_US
dc.subjectOsteoporosisen_US
dc.subjectOsteoprotegerinen_US
dc.titleReceptor Activator of Nuclear Factor kappa-Beta Ligand/Osteoprotegerin Axis in Adults with Hb S/beta-Thalassemia and beta-Thalassemia Traiten_US
dc.typeArticleen_US

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