Evaluation of the glucocorticoid, mineralocorticoid, and adrenal androgen secretion dynamics in a large cohort of patients aged 6-18 years with transfusion-dependent ?-thalassemia major, with an emphasis on the impact of cardiac iron load

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dc.contributor.authorUcar, Ahmet
dc.contributor.authorOner, Nergiz
dc.contributor.authorOzek, Gulcihan
dc.contributor.authorCetincakmak, Mehmet Guli
dc.contributor.authorAbuhandan, Mahmut
dc.contributor.authorYildirim, Ali
dc.contributor.authorKaya, Cemil
dc.date.accessioned2024-04-24T16:02:39Z
dc.date.available2024-04-24T16:02:39Z
dc.date.issued2016
dc.departmentDicle Üniversitesien_US
dc.description.abstractThe variable presence of adrenal insufficiency (AI) due to hypocortisolemia (HC) in patients with thalassemia is well established; however, the prevalence of adrenocortical hypofunction (ACH) in the zona glomerulosa and zona reticularis of the adrenal cortex is unknown. To establish the prevalence of ACH, we examined the cortisol response to 1-A mu g and 250-A mu g ACTH tests, plasma aldosterone (A)/plasma renin activity (PRA) ratio, and serum dehydroepiandrosterone sulfate (DHEAS) levels in a large cohort of patients with thalassemia, and to investigate the impact of total body iron load (TBIL) on adrenocortical function. The setting used was University hospital and government-based tertiary care center. One hundred twenty-one (52 females) patients with beta-thalassemia major (beta-TM) and 72 healthy peers (38 females) were enrolled. The patients underwent a 250-A mu g cosyntropin test if their peak cortisol was < 500 nmol/L in a 1-A mu g cosyntropin test. Magnetic resonance imaging (MRI) was performed to assess the MRI-based liver iron content and cardiac MRI T2* iron. The associations between ACH and TBIL were investigated. The patients with thalassemia had lower ACTH, cortisol, DHEAS, and A/PRA values compared with the controls (p < 0.001). Thirty-nine patients (32.2 %) had HC [primary (n = 1), central (n = 36), combined (n = 2)], and 47 (38.8 %) patients had reduced DHEAS levels; 29 (24.0 %) patients had reduced A/PRA ratios. Forty-six (38.0 %) patients had hypofunction in one of the adrenal zones, 26 (21.5 %) had hypofunction in two adrenal zones, and 9 (7.4 %) had hypofunction in all three zones. Patient age and TBIL surrogates were significant independent parameters associated with ACH. Cardiac MRI T2* iron was the only significant parameter that predicted the severity of ACH at a cut-off of 20.6 ms, with 81 % sensitivity and 78 % specificity. Patients with thalassemia have a high prevalence of AI due to HC and zona glomerulosa and zona reticularis hypofunction. TBIL surrogates can predict ACH, but cardiac iron was the only surrogate that was adequately sensitive to predict the severity of ACH.en_US
dc.description.sponsorshipSanliurfa Thalassemia Societyen_US
dc.description.sponsorshipThe authors would like to thank all of the patients and parents included in the study. The authors would also like to thank Dr. Huseyin Demirbilek for his critical review of the manuscript and David Chapman for editing the manuscript. The authors also thank Omega Statistics, Ltd., and Mark Behar for the statistical analyses, the Sanliurfa Thalassemia Society for partial financial support, and the thalassemia nurses Ayfer Askin and Harun Cagan for their active involvement in recruiting patients for the study.en_US
dc.identifier.doi10.1007/s12020-016-0872-2
dc.identifier.endpage248en_US
dc.identifier.issn1355-008X
dc.identifier.issn1559-0100
dc.identifier.issue1en_US
dc.identifier.pmid26839091
dc.identifier.scopus2-s2.0-84975871966
dc.identifier.scopusqualityQ2
dc.identifier.startpage240en_US
dc.identifier.urihttps://doi.org/10.1007/s12020-016-0872-2
dc.identifier.urihttps://hdl.handle.net/11468/14868
dc.identifier.volume53en_US
dc.identifier.wosWOS:000377934000027
dc.identifier.wosqualityQ2
dc.indekslendigikaynakWeb of Science
dc.indekslendigikaynakScopus
dc.indekslendigikaynakPubMed
dc.language.isoenen_US
dc.publisherSpringeren_US
dc.relation.ispartofEndocrine
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/closedAccessen_US
dc.subjectThalassemia Majoren_US
dc.subjectAdrenocorticalen_US
dc.subjectMineralocorticoiden_US
dc.subjectCortisolen_US
dc.subjectDehydroepiandrosterone Sulfateen_US
dc.subjectCardiac Mri T2*Ironen_US
dc.subjectTotal Body Iron Loaden_US
dc.titleEvaluation of the glucocorticoid, mineralocorticoid, and adrenal androgen secretion dynamics in a large cohort of patients aged 6-18 years with transfusion-dependent ?-thalassemia major, with an emphasis on the impact of cardiac iron loaden_US
dc.titleEvaluation of the glucocorticoid, mineralocorticoid, and adrenal androgen secretion dynamics in a large cohort of patients aged 6-18 years with transfusion-dependent ?-thalassemia major, with an emphasis on the impact of cardiac iron load
dc.typeArticleen_US

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