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Endocrine. 2019 Dec 18. doi: 10.1007/s12020-019-02159-6. [Epub ahead of print]

Evaluation of endocrine complications in beta-thalassemia intermedia (β-TI): a cross-sectional multicenter study.

Author information

1
Hematology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.
2
Pediatric Hematology-Oncology, Adult Thalassemia Clinic, Tehran, Iran.
3
Thalassemia Unit, Division of Pediatric Hematology-Oncology, First Department of Pediatrics, University of Athens "Agia Sofia" Children's Hospital, Athens, Greece.
4
Department of Pediatric Hematology, Medical Faculty, Çukurova University, Adana, Turkey.
5
Department of Hematology, College of Medicine & Health Sciences, Sultan Qaboos University, Muscat, Oman.
6
Department of Hematology, College of Medicine & Health Sciences, Sultan Qaboos University and Muscat, Sultanate of Oman Pediatric Department, Faculty of Medicine, Alexandria University, Alexandria, Egypt.
7
Department of General Medicine, Sohar Hospital, Sohar, Oman.
8
Department of Pediatrics, Division of Endocrinology, Hamad General Hospital Doha, Qatar and Department of Pediatrics, Division of Endocrinology, Alexandria University Children's Hospital, Alexandria, Egypt.
9
Thalassemia Diagnosis Center of Mediterranean Blood Diseases Foundation, Antalya, Turkey.
10
King Abdullah University Hospital, Princess Rahma Teaching hospital, Irbid, Jordan.
11
Royal Lancaster Infirmary, Lancaster, UK.
12
Archibishop Makarios III Hospital, Thalassaemia Clinic, Nicosia, Cyprus.
13
Department of Endocrinology, Whittington Hospital, University College London, London, UK.
14
Thalassemia Unit, Umberto 1° Hospital, Siracusa, Italy.
15
Children's Hospital Santobono-Pausilipon, Naples, Italy.
16
Pediatric and Adolescent Outpatient Clinic, Quisisana Hospital, Ferrara, Italy. vdesanctis@libero.it.

Abstract

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.

KEYWORDS:

Endocrine complications; Prevalence; β-thalassemia intermedia

PMID:
31853840
DOI:
10.1007/s12020-019-02159-6

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