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dc.contributor.authorNeumann, Uta
dc.contributor.authorPoyrazoglu, Şükran
dc.contributor.authorDe Sanctis, Luisa
dc.contributor.authorProbst-Scheidegger, Ursina
dc.contributor.authorNordenstrom, Ana
dc.contributor.authorMilenkovic, Tatjana
dc.contributor.authorKrone, Nils
dc.contributor.authorLawrence, Neil
dc.contributor.authorBacila, Irina
dc.contributor.authorDawson, Jeremy
dc.contributor.authorBryce, Jillian
dc.contributor.authorAli, Salma R.
dc.contributor.authorvan den Akker, Erica L. T.
dc.contributor.authorBachega, Tania A. S. S.
dc.contributor.authorBaronio, Federico
dc.contributor.authorBirkebaek, Niels H.
dc.contributor.authorBonfig, Walter
dc.contributor.authorvan der Grinten, Hedi C.
dc.contributor.authorCosta, Eduardo C.
dc.contributor.authorde Vries, Liat
dc.contributor.authorElsedfy, Heba
dc.contributor.authorGuven, Ayla
dc.contributor.authorHannema, Sabine
dc.contributor.authorIotova, Violeta
dc.contributor.authorvan der Kamp, Hetty J.
dc.contributor.authorLeon, Maria C.
dc.contributor.authorLichiardopol, Corina R.
dc.contributor.authorAhmed, Syed Faisal
dc.contributor.authorYavas, Zehra
dc.contributor.authorVieites, Ana
dc.contributor.authorThankamony, Ajay
dc.contributor.authorTadokoro-Cuccaro, Rieko
dc.date.accessioned2023-02-21T08:14:55Z
dc.date.available2023-02-21T08:14:55Z
dc.date.issued2022
dc.identifier.citationLawrence N., Bacila I., Dawson J., Bryce J., Ali S. R., van den Akker E. L. T., Bachega T. A. S. S., Baronio F., Birkebaek N. H., Bonfig W., et al., "Analysis of therapy monitoring in the International Congenital Adrenal Hyperplasia Registry.", Clinical endocrinology, cilt.97, sa.5, ss.551-561, 2022
dc.identifier.issn0300-0664
dc.identifier.othervv_1032021
dc.identifier.otherav_16af382b-3b8a-4ec7-a49a-6da3f0322f99
dc.identifier.urihttp://hdl.handle.net/20.500.12627/186489
dc.identifier.urihttps://avesis.istanbul.edu.tr/api/publication/16af382b-3b8a-4ec7-a49a-6da3f0322f99/file
dc.identifier.urihttps://doi.org/10.1111/cen.14796
dc.description.abstractObjective Congenital adrenal hyperplasia (CAH) requires exogenous steroid replacement. Treatment is commonly monitored by measuring 17-OH progesterone (17OHP) and androstenedione (D4). Design Retrospective cohort study using real-world data to evaluate 17OHP and D4 in relation to hydrocortisone (HC) dose in CAH patients treated in 14 countries. Patients Pseudonymized data from children with 21-hydroxylase deficiency (21OHD) recorded in the International CAH Registry. Measurements Assessments between January 2000 and October 2020 in patients prescribed HC were reviewed to summarise biomarkers 17OHP and D4 and HC dose. Longitudinal assessment of measures was carried out using linear mixed-effects models (LMEM). Results Cohort of 345 patients, 52.2% female, median age 4.3 years (interquartile range: 3.1-9.2) were taking a median 11.3 mg/m(2)/day (8.6-14.4) of HC. Median 17OHP was 35.7 nmol/l (3.0-104.0). Median D4 under 12 years was 0 nmol/L (0-2.0) and above 12 years was 10.5 nmol/L (3.9-21.0). There were significant differences in biomarker values between centres (p 0.05). Multivariate LMEM showed HC dose decreasing by 1.0 mg/m(2)/day for every 1 point increase in weight standard deviation score. Discussion Registry data show large variability in 17OHP and D4 between centres. 17OHP correlates with D4 well when accounting for age. Prescribed HC dose per body surface area decreased with weight gain.
dc.language.isoeng
dc.subjectKlinik Tıp (MED)
dc.subjectTıp
dc.subjectSağlık Bilimleri
dc.subjectDahili Tıp Bilimleri
dc.subjectİç Hastalıkları
dc.subjectEndokrinoloji ve Metabolizma Hastalıkları
dc.subjectEndokrin ve Otonom Sistemler
dc.subjectEndokrinoloji, Diyabet ve Metabolizma
dc.subjectEndokrinoloji
dc.subjectYaşam Bilimleri
dc.subjectENDOKRİNOLOJİ VE METABOLİZMA
dc.subjectKlinik Tıp
dc.titleAnalysis of therapy monitoring in the International Congenital Adrenal Hyperplasia Registry.
dc.typeMakale
dc.relation.journalClinical endocrinology
dc.contributor.departmentUniversity of Sheffield , ,
dc.identifier.volume97
dc.identifier.issue5
dc.identifier.startpage551
dc.identifier.endpage561
dc.contributor.firstauthorID3440046


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