Abstract:
AIMS:To compare adult heights of GH-treated and GH-untreated patients with Silver-Russell syndrome (SRS) who were epigenotyped. METHODS:This was a nonrandomized retrospective study with matched controls at a single center. Molecular analysis of 32 out of 37 GH-treated patients (16 females) revealed IGF2-H19 epimutations in 12 and maternal uniparental disomy of chromosome 7 (matUPD7) in 5 patients; 15 were negative. At start of GH, mean age was 7.2 years and mean height -3.34 standard deviation score (SDS). Mean GH dose used was 51 µg/kg·day, mean duration of therapy was 5.6 years. Puberty was blocked by GnRH analogs in 16 patients. The untreated group comprised 13 individuals (5 females, mean age 6.8 years and mean height -3.34 SDS). End points were adult height and overall height gain. RESULTS:GH-treated patients reached an adult height of -2.12 ± 0.98 SDS gaining 1.22 SDS in comparison to baseline. Adult height SDS of the untreated was -3.13 ± 1.37 SDS. The matched treated patients were significantly taller than their untreated counterparts. Outcome was dependent on height at start of GH and duration of therapy. Height gain was highest in the shortest patients. CONCLUSIONS:GH improved adult height in SRS to a comparable degree as reported in nonsyndromic SGA children. A trend toward a better outcome in matUPD7 needs confirmation in larger cohorts.
journal_name
Horm Res Paediatrjournal_title
Hormone research in paediatricsauthors
Binder G,Liebl M,Woelfle J,Eggermann T,Blumenstock G,Schweizer Rdoi
10.1159/000354658subject
Has Abstractpub_date
2013-01-01 00:00:00pages
193-200issue
3eissn
1663-2818issn
1663-2826pii
000354658journal_volume
80pub_type
临床试验,杂志文章abstract:BACKGROUND/AIMS:The diagnostic criteria for polycystic ovary syndrome (PCOS) in adolescence are controversial, primarily because the diagnostic pathological features used in adult women may be normal pubertal physiological events. Hence, international pediatric and adolescent specialty societies have defined criteria t...
journal_title:Hormone research in paediatrics
pub_type: 杂志文章
doi:10.1159/000375530
更新日期:2015-04-01 00:00:00
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journal_title:Hormone research in paediatrics
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doi:10.1159/000315656
更新日期:2011-01-01 00:00:00
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journal_title:Hormone research in paediatrics
pub_type: 临床试验,杂志文章
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journal_title:Hormone research in paediatrics
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journal_title:Hormone research in paediatrics
pub_type: 临床试验,杂志文章,多中心研究
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journal_title:Hormone research in paediatrics
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journal_title:Hormone research in paediatrics
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journal_title:Hormone research in paediatrics
pub_type: 临床试验,杂志文章,多中心研究
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journal_title:Hormone research in paediatrics
pub_type: 杂志文章
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journal_title:Hormone research in paediatrics
pub_type: 临床试验,杂志文章
doi:10.1159/000356920
更新日期:2014-01-01 00:00:00
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journal_title:Hormone research in paediatrics
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journal_title:Hormone research in paediatrics
pub_type: 杂志文章
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更新日期:2015-01-01 00:00:00
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journal_title:Hormone research in paediatrics
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更新日期:2015-01-01 00:00:00
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journal_title:Hormone research in paediatrics
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更新日期:2018-01-01 00:00:00
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更新日期:2010-01-01 00:00:00
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journal_title:Hormone research in paediatrics
pub_type: 临床试验,杂志文章,多中心研究
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更新日期:2018-01-01 00:00:00
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journal_title:Hormone research in paediatrics
pub_type: 杂志文章
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更新日期:2011-01-01 00:00:00
abstract:BACKGROUND:A UK study showed final height in Turner syndrome (TS) girls receiving growth hormone is affected by age at pubertal induction and oxandrolone (Ox). Using data from that study, we analysed the effect of timing of oral ethinylestradiol (EE2) and Ox on height velocity (HV), bone maturation and pubertal progres...
journal_title:Hormone research in paediatrics
pub_type: 杂志文章,多中心研究,随机对照试验
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journal_title:Hormone research in paediatrics
pub_type: 杂志文章
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更新日期:2017-01-01 00:00:00
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journal_title:Hormone research in paediatrics
pub_type: 临床试验,杂志文章
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更新日期:2016-01-01 00:00:00
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journal_title:Hormone research in paediatrics
pub_type: 杂志文章
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更新日期:2010-01-01 00:00:00
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