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      Hypogonadism in thalassemia major patients

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          Highlights

          • •

            Hypogonadism is a common problem in patients with thalassemia major.

          • •

            The cause of hypogonadism is usually iron deposition in pituitary gonadotropic cells.

          • •

            Pituitary MRI is useful for early detection of pituitary iron deposition.

          • •

            Ovarian failure can often occur after bone marrow transplantation.

          • •

            Fertility, using reproductive techniques, is possible and depends on gonadal reserve.

          Abstract

          Despite recent advances in iron chelation therapy, excess iron deposition in pituitary gonadotropic cells remains one of the major problems in thalassemic patients. Hypogonadism, mostly hypogonadotropic hypogonadism, is usually detected during puberty. Early diagnosis and treatment are crucial for normal pubertal development and to reduce the complications of hypogonadism. The risks and benefits of hormonal replacement therapy, especially regarding the thromboembolic event, remain a challenge for providers caring for thalassemic patients.

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          Survival and complications in patients with thalassemia major treated with transfusion and deferoxamine.

          Caterina Borgna-Pignatti, Simone Rugolotto, Piero De Stefano … (2004)
          Seven Italian centers reported data on survival, causes of death and appearance of complications in patients with thalassemia major. The interactions between gender, birth cohort, complications, and ferritin on survival and complications were analyzed. Survival after the first decade was studied for 977 patients born since 1960 whereas survival since birth and complication appearance was studied for the 720 patients born after 1970. Better survival was demonstrated for patients born in more recent years (p<0.00005) and for females (p=0.0003); 68% of the patients are alive at the age of 35 years. In the entire population 67% of the deaths were due to heart disease. There was a significant association between birth cohort and complication-free survival (p<0.0005). The prevalence of complications was: heart failure 6.8%, arrhythmia 5.7%, hypogonadism 54.7%, hypothyroidism 10.8%, diabetes 6.4%, HIV infection 1.7%, and thrombosis 1.1%. Lower ferritin levels were associated with a lower probability of heart failure (hazard ratio =3.35, p<0.005) and with prolonged survival (hazard ratio = 2.45, p<0.005), using a cut-off as low as 1,000 ng/mL. Survival and complication-free survival of patients with thalassemia major continue to improve, especially for female patients born shortly before or after the availability of iron chelation.
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            KiSS-1 neurones are direct targets for leptin in the ob/ob mouse.

            J T Smith, B V Acohido, D K Clifton … (2006)
            Leptin is an adipocyte-derived hormone that acts on the hypothalamus to influence feeding, metabolism and reproduction, but the cellular and molecular targets for the action of leptin in the brain have yet to be fully elucidated. Kisspeptins are encoded by the Kiss1 gene, which is expressed in the hypothalamus and has been implicated in the neuroendocrine regulation of gonadotrophin-releasing hormone secretion. We tested the hypothesis that kisspeptin-expressing neurones are targets for leptin. First, we examined whether leptin regulates the expression of Kiss1 by comparing levels of KiSS-1 mRNA in the arcuate nucleus among groups of mice having different circulating levels of leptin: (i) wild-type (WT); (ii) leptin-deficient ob/ob; and (iii) ob/ob mice treated with leptin. All mice were castrated to control for endogenous concentrations of gonadal steroids. KiSS-1 mRNA was significantly reduced in ob/ob compared to WT mice and levels of KiSS-1 mRNA in ob/ob mice treated with leptin were increased, but not fully restored to that found in WT animals. Second, we performed double-label in situ hybridisation for KiSS-1 mRNA and the leptin receptor (Ob-Rb) mRNA and found that almost one-half (approximately 40%) of KiSS-1 mRNA-expressing cells in the arcuate nucleus expressed Ob-Rb mRNA. These results demonstrate that KiSS-1 neurones are direct targets for regulation by leptin and suggest that the reproductive deficits associated with leptin-deficient states may be attributable, in part, to diminished expression of Kiss1.
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              How I treat thalassemia.

              Eliezer A Rachmilewitz, Christian Giardina (2011)
              The purpose of this article is to set forth our approach to diagnosing and managing the thalassemias, including β-thalassemia intermedia and β-thalassemia major. The article begins by briefly describing recent advances in our understanding of the pathophysiology of thalassemia. In the discussion on diagnosing the condition, we cover the development of improved diagnostic tools, including the use of very small fetal DNA samples to detect single point mutations with great reliability for prenatal diagnosis of homozygous thalassemia. In our description of treatment strategies, we focus on how we deal with clinical manifestations and long-term complications using the most effective current treatment methods for β-thalassemia. The discussion of disease management focuses on our use of transfusion therapy and the newly developed oral iron chelators, deferiprone and deferasirox. We also deal with splenectomy and how we manage endocrinopathies and cardiac complications. In addition, we describe our use of hematopoietic stem cell transplantation, which has produced cure rates as high as 97%, and the use of cord blood transplantation. Finally, we briefly touch on therapies that might be effective in the near future, including new fetal hemoglobin inducers and gene therapy.
              Article 0 comments Cited 89 times – based on 0 reviews     Review now
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                Author and article information

                Contributors
                Sasima Srisukh
                Journal
                Journal ID (nlm-ta): J Clin Transl Endocrinol
                Journal ID (iso-abbrev): J Clin Transl Endocrinol
                Title: Journal of Clinical & Translational Endocrinology
                Publisher: Elsevier
                ISSN (Electronic): 2214-6237
                Publication date PMC-release: 16 August 2016
                Publication date Collection: September 2016
                Publication date (Electronic): 16 August 2016
                Volume: 5
                Pages: 42-45
                Affiliations
                Department of Medicine, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, 10400, Thailand
                Author notes
                [* ]Corresponding author. Fax: 66 2 201 1647. sasima_kim@ 123456yahoo.com
                Article
                Publisher ID: S2214-6237(16)30020-5
                DOI: 10.1016/j.jcte.2016.08.001
                PMC ID: 5644428
                PubMed ID: 29067234
                SO-VID: 46234b0f-601d-4cf2-9d8c-b4b5cd54d4fc
                Copyright © © 2016 The Authors
                License:

                This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

                History
                Date received : 25 May 2016
                Date revision received : 11 August 2016
                Date accepted : 12 August 2016
                Categories
                Subject: Review

                Keywords: hypogonadism,thalassemia
                Data availability:
                Keywords: hypogonadism, thalassemia

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