TY - JOUR
TI - Evaluation of endocrine complications in beta-thalassemia intermedia (β-TI): a cross-sectional multicenter study
AU - Karimi, M.
AU - Zarei, T.
AU - Haghpanah, S.
AU - Azarkeivan, A.
AU - Kattamis, C.
AU - Ladis, V.
AU - Kattamis, A.
AU - Kilinc, Y.
AU - Daar, S.
AU - Alyaarubi, S.
AU - Khater, D.
AU - Wali, Y.
AU - Elshinawy, M.
AU - Almadhani, A.
AU - Yassin, M.
AU - Soliman, A.T.
AU - Canatan, D.
AU - Obiedat, M.
AU - Al-Rimawi, H.
AU - Mariannis, D.
AU - Christodoulides, C.
AU - Christou, S.
AU - Tzoulis, P.
AU - Campisi, S.
AU - Di Maio, S.
AU - De Sanctis, V.
JO - Endocrine Development
PY - 2020
VL - 69
TODO - 1
SP - 220-227
PB - Springer-Verlag
SN - null
TODO - 10.1007/s12020-019-02159-6
TODO - hydroxyurea, adolescent;  adult;  age;  aged;  Article;  beta thalassemia;  beta thalassemia intermedia;  child;  cross-sectional study;  Cyprus;  endocrine disease;  female;  human;  hypogonadism;  hypothyroidism;  Iran;  Jordan;  major clinical study;  male;  non insulin dependent diabetes mellitus;  Oman;  osteopenia;  osteoporosis;  prevalence;  priority journal;  Qatar;  risk factor;  splenectomy
TODO - Background: Data on the prevalence and type of endocrine disorders in β-thalassemia intermedia (β-TI) patients are scarce. This multicenter study was designed to determine the prevalence of endocrine complications and the associated risk factors in a large group of β-TI patients. Methods: In this cross-sectional multicenter study, 726 β-TI patients, aged 2.5–80 years, registered at 12 thalassemic centers, from nine countries, were enrolled during 2017. In a subgroup of 522 patients (mean age 30.8 ± 12.1; range: 2.5–80 years) from Qatar, Iran, Oman, Cyprus, and Jordan detailed data were available. Results: Overall, the most prevalent complications were osteopenia/osteoporosis (22.3%), hypogonadism (10.1%), and primary hypothyroidism (5.3%). In the subgroup multivariate analysis, older age was a risk factor for osteoporosis (Odds ratio: 7.870, 95% CI: 4.729–13.099, P < 0.001), hypogonadism (Odds ratio: 6.310, 95% CI: 2.944–13.521, P < 0.001), and non-insulin-dependent diabetes mellitus (NIDDM; Odds ratio: 17.67, 95% CI: 2.217–140.968, P = 0.007). Splenectomy was a risk factor for osteoporosis (Odds ratio: 1.736, 95% CI: 1.012–2.977, P = 0.045). Hydroxyurea was identified as a “protective factor” for NIDDM (Odds ratio: 0.259, 95% CI: 0.074–0.902, P = 0.034). Conclusions: To the best of our knowledge, this is the largest cohort of β-TI patients with endocrine disorders evaluated in extremely heterogenic thalassemic populations for age, clinical, hematological, and molecular composition. The study demonstrates that endocrine complications are less common in patients with β-TI compared with β-TM patients. However, regular monitoring with timely diagnosis and proper management is crucial to prevent endocrine complications in β-TI patients. © 2019, Springer Science+Business Media, LLC, part of Springer Nature.
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