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Article: Growth, puberty and endocrine function in beta-thalassaemia major

TitleGrowth, puberty and endocrine function in beta-thalassaemia major
Authors
Issue Date1997
PublisherFreund Publishing House, Ltd. The Journal's web site is located at http://www.freundpublishing.com/Journal_Pediatric_Endocrinology_Metabolism/JPEMprev.htm
Citation
Journal Of Pediatric Endocrinology And Metabolism, 1997, v. 10 n. 2, p. 175-184 How to Cite?
AbstractAlthough delay in onset of puberty is a common cause of growth failure in adolescent thalassaemic patients, growth retardation could also be due to iron overload, the toxic effects of desferrioxamine, or the development of other endocrinopathies such as GH insufficiency or primary hypothyroidism. Abnormal body proportions with truncal shortening are commonly seen and could be due to the disease itself, iron toxicity, delay in puberty or the toxic effects of desferrioxamine. The absence of a pubertal growth spurt during spontaneous or induced puberty is detrimental to the achievement of a normal final adult height. Low serum IGF-I and normal GH reserve in short thalassaemic children imply that a state of relative GH resistance exists. The rise in IGF-I and improvement in growth with GH therapy suggest that this GH resistance is only partial. Although the results of short-term GH therapy are encouraging, the impact of treatment on final height on non-GH deficient short thalassaemic children remains uncertain. Multiple endocrinopathies, including hypogonadism, hypothyroidism and diabetes mellitus, occur mainly in older patients who tend to have high serum ferritin levels. Prognosis for survival is greatly improved if the serum ferritin is kept below 2000 μg/l by regular chelation. Chelation therapy initiated early before the accumulation of a significant iron burden or dosages of desferrioxamine in excess of 50 mg/kg/day should be avoided. Serum ferritin should be checked regularly and the 'toxicity index' should be used to monitor chelation therapy. In cases of delayed puberty, sexual development should be induced at an appropriate age.
Persistent Identifierhttp://hdl.handle.net/10722/79925
ISSN
2015 Impact Factor: 0.912
2015 SCImago Journal Rankings: 0.493
ISI Accession Number ID
References

 

DC FieldValueLanguage
dc.contributor.authorLow, LCKen_HK
dc.date.accessioned2010-09-06T08:00:22Z-
dc.date.available2010-09-06T08:00:22Z-
dc.date.issued1997en_HK
dc.identifier.citationJournal Of Pediatric Endocrinology And Metabolism, 1997, v. 10 n. 2, p. 175-184en_HK
dc.identifier.issn0334-018Xen_HK
dc.identifier.urihttp://hdl.handle.net/10722/79925-
dc.description.abstractAlthough delay in onset of puberty is a common cause of growth failure in adolescent thalassaemic patients, growth retardation could also be due to iron overload, the toxic effects of desferrioxamine, or the development of other endocrinopathies such as GH insufficiency or primary hypothyroidism. Abnormal body proportions with truncal shortening are commonly seen and could be due to the disease itself, iron toxicity, delay in puberty or the toxic effects of desferrioxamine. The absence of a pubertal growth spurt during spontaneous or induced puberty is detrimental to the achievement of a normal final adult height. Low serum IGF-I and normal GH reserve in short thalassaemic children imply that a state of relative GH resistance exists. The rise in IGF-I and improvement in growth with GH therapy suggest that this GH resistance is only partial. Although the results of short-term GH therapy are encouraging, the impact of treatment on final height on non-GH deficient short thalassaemic children remains uncertain. Multiple endocrinopathies, including hypogonadism, hypothyroidism and diabetes mellitus, occur mainly in older patients who tend to have high serum ferritin levels. Prognosis for survival is greatly improved if the serum ferritin is kept below 2000 μg/l by regular chelation. Chelation therapy initiated early before the accumulation of a significant iron burden or dosages of desferrioxamine in excess of 50 mg/kg/day should be avoided. Serum ferritin should be checked regularly and the 'toxicity index' should be used to monitor chelation therapy. In cases of delayed puberty, sexual development should be induced at an appropriate age.en_HK
dc.languageengen_HK
dc.publisherFreund Publishing House, Ltd. The Journal's web site is located at http://www.freundpublishing.com/Journal_Pediatric_Endocrinology_Metabolism/JPEMprev.htmen_HK
dc.relation.ispartofJournal of Pediatric Endocrinology and Metabolismen_HK
dc.titleGrowth, puberty and endocrine function in beta-thalassaemia majoren_HK
dc.typeArticleen_HK
dc.identifier.openurlhttp://library.hku.hk:4550/resserv?sid=HKU:IR&issn=0334-018X&volume=10 &issue=No 2&spage=175&epage=184&date=1997&atitle=Growth,+Puberty+and+Endocrine+Function+in+Beta-Thalassaemia+Majoren_HK
dc.identifier.emailLow, LCK: lcklow@hkucc.hku.hken_HK
dc.identifier.authorityLow, LCK=rp00337en_HK
dc.description.naturelink_to_subscribed_fulltext-
dc.identifier.pmid9364350en_HK
dc.identifier.scopuseid_2-s2.0-0031006274en_HK
dc.identifier.hkuros22426en_HK
dc.relation.referenceshttp://www.scopus.com/mlt/select.url?eid=2-s2.0-0031006274&selection=ref&src=s&origin=recordpageen_HK
dc.identifier.volume10en_HK
dc.identifier.issue2en_HK
dc.identifier.spage175en_HK
dc.identifier.epage184en_HK
dc.identifier.isiWOS:A1997XA69900002-
dc.publisher.placeIsraelen_HK
dc.identifier.scopusauthoridLow, LCK=7007049461en_HK

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