Review and recommendations on management of adult female thalassemia patients with hypogonadism based on literature review and experience of ICET-A network specialists

dc.contributor.authorDe Sanctis, Vincenzoen_ZA
dc.contributor.authorSoliman, Ashraf T.en_ZA
dc.contributor.authorElsedfy, Hebaen_ZA
dc.contributor.authorAlbu, Aliceen_ZA
dc.contributor.authorAl Jaouni, Soaden_ZA
dc.contributor.authorAnastasi, Salvatoreen_ZA
dc.contributor.authorBisconte, Maria Graziaen_ZA
dc.contributor.authorCanatan, Duranen_ZA
dc.contributor.authorChristou, Soteroulaen_ZA
dc.contributor.authorDaar, Shahinaen_ZA
dc.contributor.authorDi Maio, Salvatoreen_ZA
dc.contributor.authorKholy, Mohamed Elen_ZA
dc.contributor.authorKhater, Doaaen_ZA
dc.contributor.authorElshinawy, Mohameden_ZA
dc.contributor.authorKilinc, Yurdanuren_ZA
dc.contributor.authorMattei, Robertoen_ZA
dc.contributor.authorMosli, Hala H.en_ZA
dc.contributor.authorQuota, Alessandraen_ZA
dc.contributor.authorRoberti, Maria Graziaen_ZA
dc.contributor.authorSobti, Praveenen_ZA
dc.contributor.authorAL Yaarubi, Saifen_ZA
dc.contributor.authorCanpisi, Saveriaen_ZA
dc.contributor.authorKattamis, Christosen_ZA
dc.date.accessioned2018-10-26T09:35:36Z
dc.date.available2018-10-26T09:35:36Z
dc.date.issued2017
dc.descriptionCITATION: De Sanctis, V., et al. 2017. Review and recommendations on management of adult female thalassemia patients with hypogonadism based on literature review and experience of ICET-A network specialists. Mediterranean Journal of Hematology and Infectious Diseases, 9(1):e2017001, doi:10.4084/mjhid.2017.001.
dc.descriptionThe original publication is available at https://www.mjhid.org
dc.description.abstractBackground: Multi-transfused thalassemia major (TM) patients frequently develop severe endocrine complications, mainly due to iron overload, anemia and chronic liver disease, which require prompt diagnosis, treatment and follow-up by specialists. The most common endocrine complication documented is hypogonadotropic hypogonadism which increases with age and associated comorbidities. It is thus important for physicians to have a clear understanding of the pathophysiology and management of this disorder. Also to be aware of the side effects, contraindications and monitoring of sex steroid therapy. In this paper practical ICET-A recommendations for the management of hypogonadism in adult females with TM are addressed. Methods: In March 2015, the Coordinator of the International Network of Clinicians for Endocrinopathies in Thalassemia and Adolescent Medicine (ICET-A) conducted a two-step survey to assess the attitudes and practices of doctors in the ICET-A network taking care of adult female TM patients with hypogonadism. They were clinically characterized by the absence of pubertal development, or discontinuation or regression of the maturation of secondary sex characteristics, and biochemically by persistent low FSH, LH and estradiol levels. Recently a supplementary survey on adult female hypogonadism in TM was undertaken within the ICET-A network. Results: The completed questionnaires were returned by 16 of 27 specialists (59.2%) following 590 female TM patients over the age of 18 years; 315 patients (53.3%) had hypogonadism and only 245 (74.6%) were on hormone replacement therapy (HRT). Contraceptive oral pills (COC) were the first treatment choice in 11 centres (68.7%). A wide range of COCs were used with different progestin contents. In general, the patients’ compliance to treatment was reported as good in 81.2 % of centres. The frequency of required tests for follow-up HRT, in addition to the regular check-up for thalassemia, was variable in the participating centres. Conclusions: Doctors taking care of TM patients should have sound knowledge of the pathophysiology of hypogonadism in adult females with TM. They should know the potential effects of HRT including advantages and disadvantages of estrogen and progestins. Moreover, they should keep in consideration the emotional needs of these patients dreaming to attain a full pubertal development.en_ZA
dc.description.urihttps://www.mjhid.org/index.php/mjhid/article/view/2017.001
dc.description.versionPublisher's version
dc.format.extent10 pages
dc.identifier.citationDe Sanctis, V., et al. 2017. Review and recommendations on management of adult female thalassemia patients with hypogonadism based on literature review and experience of ICET-A network specialists. Mediterranean Journal of Hematology and Infectious Diseases, 9(1):e2017001, doi:10.4084/mjhid.2017.001
dc.identifier.issn2035-3006 (online)
dc.identifier.otherdoi:10.4084/mjhid.2017.001
dc.identifier.urihttp://hdl.handle.net/10019.1/104614
dc.language.isoen_ZAen_ZA
dc.publisherPAGEpress
dc.rights.holderAuthors retain copyright
dc.subjectThalassemia -- Treatmenten_ZA
dc.subjectEndocrine glands -- Diseasesen_ZA
dc.subjectHypogonadism -- Pathologyen_ZA
dc.titleReview and recommendations on management of adult female thalassemia patients with hypogonadism based on literature review and experience of ICET-A network specialistsen_ZA
dc.typeArticleen_ZA
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