Growth and development in thalassaemia major patients with severe bone lesions due to desferrioxamine
Nine transfusion-dependent beta-thalassaemia major patients (seven males and two females), aged 4-15 years, with growth retardation and severe rickets-like radiological lesions due to continuous subcutaneous chelation therapy with desferrioxamine (45-75 mg/kg body weight, 6-7 time/week), were seen i...
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| Veröffentlicht in: | European journal of pediatrics 1996-05, Vol.155 (5), p.368-372 |
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| container_title | European journal of pediatrics |
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| creator | DE SANCTIS, V PINAMONTI, A DI PALMA, A SPROCATI, M ATTI, G GAMBERINI, M. R VULLO, C |
| description | Nine transfusion-dependent beta-thalassaemia major patients (seven males and two females), aged 4-15 years, with growth retardation and severe rickets-like radiological lesions due to continuous subcutaneous chelation therapy with desferrioxamine (45-75 mg/kg body weight, 6-7 time/week), were seen in our centre during the last 8 years. Serum ferritin levels ranged from 976 to 4115 micrograms/l. There was a progressive decline in growth velocity in these patients 2-3 years before the appearance of rickets-like radiological lesions. All patients underwent surgery to correct genu valgum and/or slipped capital epiphyses. The final height was below the 3rd percentile in six patients (SDS: from -2.9 to -5.2). The short stature was mainly due to a disproportion between upper and lower segments. Six of the patients had an associated sensorineural hearing loss.
Our data emphasize the importance of an accurate surveillance of the toxic effects of desferrioxamine treatment and warn of the risk of overtreating patients with low iron overload and also suggest a possible individual idiosyncrasy to the adverse effects of chelation therapy. |
| doi_str_mv | 10.1007/BF01955263 |
| format | Article |
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Our data emphasize the importance of an accurate surveillance of the toxic effects of desferrioxamine treatment and warn of the risk of overtreating patients with low iron overload and also suggest a possible individual idiosyncrasy to the adverse effects of chelation therapy.</description><identifier>ISSN: 0340-6199</identifier><identifier>EISSN: 1432-1076</identifier><identifier>DOI: 10.1007/BF01955263</identifier><identifier>PMID: 8741032</identifier><identifier>CODEN: EJPEDT</identifier><language>eng</language><publisher>Heidelberg: Springer</publisher><subject>Adolescent ; Antidotes - adverse effects ; beta-Thalassemia - complications ; beta-Thalassemia - drug therapy ; beta-Thalassemia - physiopathology ; Biological and medical sciences ; Blood diseases ; Body height ; Body weight ; Bone Diseases - chemically induced ; Bone Diseases - complications ; Bone lesions ; Chelation ; Chelation therapy ; Chelation Therapy - adverse effects ; Child ; Child, Preschool ; Deferoxamine ; Deferoxamine - adverse effects ; Deferoxamine - therapeutic use ; Epiphysis ; Female ; Ferritin ; General and cellular metabolism. Vitamins ; Growth ; Growth rate ; Hearing loss ; Humans ; Iron ; Lead poisoning ; Male ; Medical sciences ; Pharmacology. Drug treatments ; Physical growth ; Rickets ; Surgery ; Velocity</subject><ispartof>European journal of pediatrics, 1996-05, Vol.155 (5), p.368-372</ispartof><rights>1996 INIST-CNRS</rights><rights>Springer-Verlag 1996</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c339t-b534a9f0efd8e00ecf985d650a17922c9518da558104712d7cd269e87cb533803</citedby><cites>FETCH-LOGICAL-c339t-b534a9f0efd8e00ecf985d650a17922c9518da558104712d7cd269e87cb533803</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,776,780,27901,27902</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=3073607$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/8741032$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>DE SANCTIS, V</creatorcontrib><creatorcontrib>PINAMONTI, A</creatorcontrib><creatorcontrib>DI PALMA, A</creatorcontrib><creatorcontrib>SPROCATI, M</creatorcontrib><creatorcontrib>ATTI, G</creatorcontrib><creatorcontrib>GAMBERINI, M. R</creatorcontrib><creatorcontrib>VULLO, C</creatorcontrib><title>Growth and development in thalassaemia major patients with severe bone lesions due to desferrioxamine</title><title>European journal of pediatrics</title><addtitle>Eur J Pediatr</addtitle><description>Nine transfusion-dependent beta-thalassaemia major patients (seven males and two females), aged 4-15 years, with growth retardation and severe rickets-like radiological lesions due to continuous subcutaneous chelation therapy with desferrioxamine (45-75 mg/kg body weight, 6-7 time/week), were seen in our centre during the last 8 years. Serum ferritin levels ranged from 976 to 4115 micrograms/l. There was a progressive decline in growth velocity in these patients 2-3 years before the appearance of rickets-like radiological lesions. All patients underwent surgery to correct genu valgum and/or slipped capital epiphyses. The final height was below the 3rd percentile in six patients (SDS: from -2.9 to -5.2). The short stature was mainly due to a disproportion between upper and lower segments. Six of the patients had an associated sensorineural hearing loss.
Our data emphasize the importance of an accurate surveillance of the toxic effects of desferrioxamine treatment and warn of the risk of overtreating patients with low iron overload and also suggest a possible individual idiosyncrasy to the adverse effects of chelation therapy.</description><subject>Adolescent</subject><subject>Antidotes - adverse effects</subject><subject>beta-Thalassemia - complications</subject><subject>beta-Thalassemia - drug therapy</subject><subject>beta-Thalassemia - physiopathology</subject><subject>Biological and medical sciences</subject><subject>Blood diseases</subject><subject>Body height</subject><subject>Body weight</subject><subject>Bone Diseases - chemically induced</subject><subject>Bone Diseases - complications</subject><subject>Bone lesions</subject><subject>Chelation</subject><subject>Chelation therapy</subject><subject>Chelation Therapy - adverse effects</subject><subject>Child</subject><subject>Child, Preschool</subject><subject>Deferoxamine</subject><subject>Deferoxamine - adverse effects</subject><subject>Deferoxamine - therapeutic use</subject><subject>Epiphysis</subject><subject>Female</subject><subject>Ferritin</subject><subject>General and cellular metabolism. Vitamins</subject><subject>Growth</subject><subject>Growth rate</subject><subject>Hearing loss</subject><subject>Humans</subject><subject>Iron</subject><subject>Lead poisoning</subject><subject>Male</subject><subject>Medical sciences</subject><subject>Pharmacology. Drug treatments</subject><subject>Physical growth</subject><subject>Rickets</subject><subject>Surgery</subject><subject>Velocity</subject><issn>0340-6199</issn><issn>1432-1076</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>1996</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><sourceid>BENPR</sourceid><recordid>eNpFkMFLwzAYxYMoc04v3oWAnoTql6ZpmqMON4WBFz2XrPnKMtpmJq3T_96IZR4-3uH93vvgEXLJ4I4ByPvHBTAlRJrzIzJlGU8TBjI_JlPgGSQ5U-qUnIWwhQgrVkzIpJAZA55OCS692_cbqjtDDX5i43Ytdj21He03utEhaGytpq3eOk93urfRDXRvYyZE3iNduw5pg8G6LlAzIO1drAo1em_dl25th-fkpNZNwItRZ-R98fQ2f05Wr8uX-cMqqThXfbIWPNOqBqxNgQBY1aoQJhegmVRpWinBCqOFKBhkkqVGVibNFRayikleAJ-R67_enXcfA4a-3LrBd_FlyVTGeAwKGanbP6ryLgSPdbnzttX-u2RQ_g5a_g8a4auxcli3aA7ouGD0b0Zfh0o3tdddZcMB4yB5Hu8HJNV8yg</recordid><startdate>19960501</startdate><enddate>19960501</enddate><creator>DE SANCTIS, V</creator><creator>PINAMONTI, A</creator><creator>DI PALMA, A</creator><creator>SPROCATI, M</creator><creator>ATTI, G</creator><creator>GAMBERINI, M. 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Vitamins</topic><topic>Growth</topic><topic>Growth rate</topic><topic>Hearing loss</topic><topic>Humans</topic><topic>Iron</topic><topic>Lead poisoning</topic><topic>Male</topic><topic>Medical sciences</topic><topic>Pharmacology. Drug treatments</topic><topic>Physical growth</topic><topic>Rickets</topic><topic>Surgery</topic><topic>Velocity</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>DE SANCTIS, V</creatorcontrib><creatorcontrib>PINAMONTI, A</creatorcontrib><creatorcontrib>DI PALMA, A</creatorcontrib><creatorcontrib>SPROCATI, M</creatorcontrib><creatorcontrib>ATTI, G</creatorcontrib><creatorcontrib>GAMBERINI, M. 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R</au><au>VULLO, C</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Growth and development in thalassaemia major patients with severe bone lesions due to desferrioxamine</atitle><jtitle>European journal of pediatrics</jtitle><addtitle>Eur J Pediatr</addtitle><date>1996-05-01</date><risdate>1996</risdate><volume>155</volume><issue>5</issue><spage>368</spage><epage>372</epage><pages>368-372</pages><issn>0340-6199</issn><eissn>1432-1076</eissn><coden>EJPEDT</coden><abstract>Nine transfusion-dependent beta-thalassaemia major patients (seven males and two females), aged 4-15 years, with growth retardation and severe rickets-like radiological lesions due to continuous subcutaneous chelation therapy with desferrioxamine (45-75 mg/kg body weight, 6-7 time/week), were seen in our centre during the last 8 years. Serum ferritin levels ranged from 976 to 4115 micrograms/l. There was a progressive decline in growth velocity in these patients 2-3 years before the appearance of rickets-like radiological lesions. All patients underwent surgery to correct genu valgum and/or slipped capital epiphyses. The final height was below the 3rd percentile in six patients (SDS: from -2.9 to -5.2). The short stature was mainly due to a disproportion between upper and lower segments. Six of the patients had an associated sensorineural hearing loss.
Our data emphasize the importance of an accurate surveillance of the toxic effects of desferrioxamine treatment and warn of the risk of overtreating patients with low iron overload and also suggest a possible individual idiosyncrasy to the adverse effects of chelation therapy.</abstract><cop>Heidelberg</cop><cop>Berlin</cop><pub>Springer</pub><pmid>8741032</pmid><doi>10.1007/BF01955263</doi><tpages>5</tpages></addata></record> |
| fulltext | fulltext |
| identifier | ISSN: 0340-6199 |
| ispartof | European journal of pediatrics, 1996-05, Vol.155 (5), p.368-372 |
| issn | 0340-6199 1432-1076 |
| language | eng |
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| source | MEDLINE; Springer Nature - Complete Springer Journals |
| subjects | Adolescent Antidotes - adverse effects beta-Thalassemia - complications beta-Thalassemia - drug therapy beta-Thalassemia - physiopathology Biological and medical sciences Blood diseases Body height Body weight Bone Diseases - chemically induced Bone Diseases - complications Bone lesions Chelation Chelation therapy Chelation Therapy - adverse effects Child Child, Preschool Deferoxamine Deferoxamine - adverse effects Deferoxamine - therapeutic use Epiphysis Female Ferritin General and cellular metabolism. Vitamins Growth Growth rate Hearing loss Humans Iron Lead poisoning Male Medical sciences Pharmacology. Drug treatments Physical growth Rickets Surgery Velocity |
| title | Growth and development in thalassaemia major patients with severe bone lesions due to desferrioxamine |
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