Beta-Thalassaemia Intermedia : Evaluation of Endocrine and Bone Complications
Objective. Data about endocrine and bone disease in nontransfusion-dependent thalassaemia (NTDT) is scanty. The aim of our study was to evaluate these complications in β-TI adult patients. Methods. We studied retrospectively 70 β-TI patients with mean followup of 20 years. Data recorded included age...
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| creator | Marcon, A. Ulivieri, F. M. Baldini, M. Graziadei, G. Cappellini, Maria Domenica Spinelli, D. Cassin, R. |
| description | Objective. Data about endocrine and bone disease in nontransfusion-dependent thalassaemia (NTDT) is scanty. The aim of our study was to evaluate these complications in β-TI adult patients. Methods. We studied retrospectively 70 β-TI patients with mean followup of 20 years. Data recorded included age, gender, haemoglobin and ferritin levels, biochemical and endocrine tests, liver iron concentration (LIC) from T2*, transfusion regimen, iron chelation, hydroxyurea, splenectomy, and bone mineralization by dual X-ray absorptiometry. Results. Thirty-seven (53%) males and 33 (47%) females were studied, with mean age 41±12 years, mean haemoglobin 9.2±1.5 g/dL, median ferritin 537 (range 14–4893), and mean LIC 7.6±6.4 mg Fe/g dw. Thirty-three patients (47%) had been transfused, occasionally (24/33; 73%) or regularly (9/33; 27%); 37/70 (53%) had never been transfused; 34/70 patients had been splenectomized (49%); 39 (56%) were on chelation therapy; and 11 (16%) were on hydroxyurea. Endocrinopathies were found in 15 patients (21%): 10 hypothyroidism, 3 hypogonadism, 2 impaired glucose tolerance (IGT), and one diabetes. Bone disease was observed in 53/70 (76%) patients, osteoporosis in 26/53 (49%), and osteopenia in 27/53 (51%). Discussion and Conclusions. Bone disease was found in most patients in our study, while endocrinopathies were highly uncommon, especially hypogonadism. We speculate that low iron burden may protect against endocrinopathy development. |
| doi_str_mv | 10.1155/2014/174581 |
| format | Article |
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M. ; Baldini, M. ; Graziadei, G. ; Cappellini, Maria Domenica ; Spinelli, D. ; Cassin, R.</creator><contributor>Ricchi, Paolo</contributor><creatorcontrib>Marcon, A. ; Ulivieri, F. M. ; Baldini, M. ; Graziadei, G. ; Cappellini, Maria Domenica ; Spinelli, D. ; Cassin, R. ; Ricchi, Paolo</creatorcontrib><description>Objective. Data about endocrine and bone disease in nontransfusion-dependent thalassaemia (NTDT) is scanty. The aim of our study was to evaluate these complications in β-TI adult patients. Methods. We studied retrospectively 70 β-TI patients with mean followup of 20 years. Data recorded included age, gender, haemoglobin and ferritin levels, biochemical and endocrine tests, liver iron concentration (LIC) from T2*, transfusion regimen, iron chelation, hydroxyurea, splenectomy, and bone mineralization by dual X-ray absorptiometry. Results. Thirty-seven (53%) males and 33 (47%) females were studied, with mean age 41±12 years, mean haemoglobin 9.2±1.5 g/dL, median ferritin 537 (range 14–4893), and mean LIC 7.6±6.4 mg Fe/g dw. Thirty-three patients (47%) had been transfused, occasionally (24/33; 73%) or regularly (9/33; 27%); 37/70 (53%) had never been transfused; 34/70 patients had been splenectomized (49%); 39 (56%) were on chelation therapy; and 11 (16%) were on hydroxyurea. Endocrinopathies were found in 15 patients (21%): 10 hypothyroidism, 3 hypogonadism, 2 impaired glucose tolerance (IGT), and one diabetes. Bone disease was observed in 53/70 (76%) patients, osteoporosis in 26/53 (49%), and osteopenia in 27/53 (51%). Discussion and Conclusions. Bone disease was found in most patients in our study, while endocrinopathies were highly uncommon, especially hypogonadism. We speculate that low iron burden may protect against endocrinopathy development.</description><identifier>ISSN: 2314-6133</identifier><identifier>EISSN: 2314-6141</identifier><identifier>DOI: 10.1155/2014/174581</identifier><identifier>PMID: 25110660</identifier><language>eng</language><publisher>Cairo, Egypt: Hindawi Puplishing Corporation</publisher><subject>Adult ; Age ; beta-Thalassemia - complications ; Bone density ; Bone diseases ; Bone Diseases - complications ; Bone Diseases, Metabolic - complications ; Chelation therapy ; Complications and side effects ; Diabetes ; Endocrine gland diseases ; Endocrine System Diseases - complications ; Female ; Fractures ; Humans ; Hypothyroidism ; Male ; Metabolism ; NMR ; Nuclear magnetic resonance ; Older people ; Osteoporosis ; Osteoporosis - complications ; Patients ; Population ; Risk factors ; Standard deviation ; Thalassemia</subject><ispartof>BioMed research international, 2014-01, Vol.2014 (2014), p.1-5</ispartof><rights>Copyright © 2014 M. Baldini et al.</rights><rights>COPYRIGHT 2014 John Wiley & Sons, Inc.</rights><rights>Copyright © 2014 M. Baldini et al. M. Baldini et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.</rights><rights>Copyright © 2014 M. Baldini et al. 2014</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c527t-aff145749f9c2e2dba124feb3c4d0f5f964f237f389443518cf71bd405a54aca3</citedby><cites>FETCH-LOGICAL-c527t-aff145749f9c2e2dba124feb3c4d0f5f964f237f389443518cf71bd405a54aca3</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC4109222/pdf/$$EPDF$$P50$$Gpubmedcentral$$Hfree_for_read</linktopdf><linktohtml>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC4109222/$$EHTML$$P50$$Gpubmedcentral$$Hfree_for_read</linktohtml><link.rule.ids>230,314,724,777,781,882,27905,27906,53772,53774</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/25110660$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><contributor>Ricchi, Paolo</contributor><creatorcontrib>Marcon, A.</creatorcontrib><creatorcontrib>Ulivieri, F. M.</creatorcontrib><creatorcontrib>Baldini, M.</creatorcontrib><creatorcontrib>Graziadei, G.</creatorcontrib><creatorcontrib>Cappellini, Maria Domenica</creatorcontrib><creatorcontrib>Spinelli, D.</creatorcontrib><creatorcontrib>Cassin, R.</creatorcontrib><title>Beta-Thalassaemia Intermedia : Evaluation of Endocrine and Bone Complications</title><title>BioMed research international</title><addtitle>Biomed Res Int</addtitle><description>Objective. Data about endocrine and bone disease in nontransfusion-dependent thalassaemia (NTDT) is scanty. The aim of our study was to evaluate these complications in β-TI adult patients. Methods. We studied retrospectively 70 β-TI patients with mean followup of 20 years. Data recorded included age, gender, haemoglobin and ferritin levels, biochemical and endocrine tests, liver iron concentration (LIC) from T2*, transfusion regimen, iron chelation, hydroxyurea, splenectomy, and bone mineralization by dual X-ray absorptiometry. Results. Thirty-seven (53%) males and 33 (47%) females were studied, with mean age 41±12 years, mean haemoglobin 9.2±1.5 g/dL, median ferritin 537 (range 14–4893), and mean LIC 7.6±6.4 mg Fe/g dw. Thirty-three patients (47%) had been transfused, occasionally (24/33; 73%) or regularly (9/33; 27%); 37/70 (53%) had never been transfused; 34/70 patients had been splenectomized (49%); 39 (56%) were on chelation therapy; and 11 (16%) were on hydroxyurea. Endocrinopathies were found in 15 patients (21%): 10 hypothyroidism, 3 hypogonadism, 2 impaired glucose tolerance (IGT), and one diabetes. Bone disease was observed in 53/70 (76%) patients, osteoporosis in 26/53 (49%), and osteopenia in 27/53 (51%). Discussion and Conclusions. Bone disease was found in most patients in our study, while endocrinopathies were highly uncommon, especially hypogonadism. We speculate that low iron burden may protect against endocrinopathy development.</description><subject>Adult</subject><subject>Age</subject><subject>beta-Thalassemia - complications</subject><subject>Bone density</subject><subject>Bone diseases</subject><subject>Bone Diseases - complications</subject><subject>Bone Diseases, Metabolic - complications</subject><subject>Chelation therapy</subject><subject>Complications and side effects</subject><subject>Diabetes</subject><subject>Endocrine gland diseases</subject><subject>Endocrine System Diseases - complications</subject><subject>Female</subject><subject>Fractures</subject><subject>Humans</subject><subject>Hypothyroidism</subject><subject>Male</subject><subject>Metabolism</subject><subject>NMR</subject><subject>Nuclear magnetic resonance</subject><subject>Older people</subject><subject>Osteoporosis</subject><subject>Osteoporosis - complications</subject><subject>Patients</subject><subject>Population</subject><subject>Risk factors</subject><subject>Standard deviation</subject><subject>Thalassemia</subject><issn>2314-6133</issn><issn>2314-6141</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2014</creationdate><recordtype>article</recordtype><sourceid>RHX</sourceid><sourceid>EIF</sourceid><sourceid>ABUWG</sourceid><sourceid>AFKRA</sourceid><sourceid>AZQEC</sourceid><sourceid>BENPR</sourceid><sourceid>CCPQU</sourceid><sourceid>DWQXO</sourceid><sourceid>GNUQQ</sourceid><recordid>eNqN0Utv1DAUBeAIUdGqdMUaFIkNKkrr61eSLpDa0UArFbEpa-uOY3dcJfZgJ0X8-3pIGR6b4k2ulE_Hj1MUr4CcAAhxSgnwU6i5aOBZcUAZ8EoCh-e7mbH94iilO5JXA5K08kWxTwUAkZIcFJ8vzIjVzRp7TAnN4LC88qOJg-nyeFYu77GfcHTBl8GWS98FHZ03JfquvAh5WIRh0zv9k6SXxZ7FPpmjx-9h8fXj8mZxWV1_-XS1OL-utKD1WKG1wEXNW9tqami3QqDcmhXTvCNW2FZyS1ltWdNyzgQ02taw6jgRKDhqZIfFhzl3M63ySbXxY8RebaIbMP5QAZ36-493a3Ub7hUH0lJKc8C7x4AYvk0mjWpwSZu-R2_ClBRIAMk4bf-DCkEb0tSMZ_r2H3oXpujzS8xKZMN-q1vsjXLehnxEvQ1V55zWhFLCttu-n5WOIaVo7O52QNS2erWtXs3VZ_3mzwfZ2V9FZ3A8g7XzHX53T6S9nrHJxFjcYZ67EJI9AN0DvMo</recordid><startdate>20140101</startdate><enddate>20140101</enddate><creator>Marcon, A.</creator><creator>Ulivieri, F. 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M.</au><au>Baldini, M.</au><au>Graziadei, G.</au><au>Cappellini, Maria Domenica</au><au>Spinelli, D.</au><au>Cassin, R.</au><au>Ricchi, Paolo</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Beta-Thalassaemia Intermedia : Evaluation of Endocrine and Bone Complications</atitle><jtitle>BioMed research international</jtitle><addtitle>Biomed Res Int</addtitle><date>2014-01-01</date><risdate>2014</risdate><volume>2014</volume><issue>2014</issue><spage>1</spage><epage>5</epage><pages>1-5</pages><issn>2314-6133</issn><eissn>2314-6141</eissn><abstract>Objective. Data about endocrine and bone disease in nontransfusion-dependent thalassaemia (NTDT) is scanty. The aim of our study was to evaluate these complications in β-TI adult patients. Methods. We studied retrospectively 70 β-TI patients with mean followup of 20 years. Data recorded included age, gender, haemoglobin and ferritin levels, biochemical and endocrine tests, liver iron concentration (LIC) from T2*, transfusion regimen, iron chelation, hydroxyurea, splenectomy, and bone mineralization by dual X-ray absorptiometry. Results. Thirty-seven (53%) males and 33 (47%) females were studied, with mean age 41±12 years, mean haemoglobin 9.2±1.5 g/dL, median ferritin 537 (range 14–4893), and mean LIC 7.6±6.4 mg Fe/g dw. Thirty-three patients (47%) had been transfused, occasionally (24/33; 73%) or regularly (9/33; 27%); 37/70 (53%) had never been transfused; 34/70 patients had been splenectomized (49%); 39 (56%) were on chelation therapy; and 11 (16%) were on hydroxyurea. Endocrinopathies were found in 15 patients (21%): 10 hypothyroidism, 3 hypogonadism, 2 impaired glucose tolerance (IGT), and one diabetes. Bone disease was observed in 53/70 (76%) patients, osteoporosis in 26/53 (49%), and osteopenia in 27/53 (51%). Discussion and Conclusions. Bone disease was found in most patients in our study, while endocrinopathies were highly uncommon, especially hypogonadism. We speculate that low iron burden may protect against endocrinopathy development.</abstract><cop>Cairo, Egypt</cop><pub>Hindawi Puplishing Corporation</pub><pmid>25110660</pmid><doi>10.1155/2014/174581</doi><tpages>5</tpages><oa>free_for_read</oa></addata></record> |
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| subjects | Adult Age beta-Thalassemia - complications Bone density Bone diseases Bone Diseases - complications Bone Diseases, Metabolic - complications Chelation therapy Complications and side effects Diabetes Endocrine gland diseases Endocrine System Diseases - complications Female Fractures Humans Hypothyroidism Male Metabolism NMR Nuclear magnetic resonance Older people Osteoporosis Osteoporosis - complications Patients Population Risk factors Standard deviation Thalassemia |
| title | Beta-Thalassaemia Intermedia : Evaluation of Endocrine and Bone Complications |
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