English (United Kingdom)

https://curated-unify.zendy.io/wp-json/zendy-region/v1/featured_content/oa?rat=en

https://curated-unify.zendy.io/wp-json/zendy-region/v1/highlighted_journal/

Global: Zendy Plus annual

Presents the access of premium content as premium feature

Premium Content

Presents the keyphrase highlighting as premium feature

Keyphrase Highlighting

Presents the summarisation as premium feature

Summarisation

Insights

Presents the pdf analysis as premium feature

PDF Analysis

Presents the zaia usage as premium feature

ZAIA

Global: Zendy Tools annual

Global: Zendy Free Plan

Global: Zendy Tools monthly

Global: Zendy Plus monthly

Growth hormone insensitivity syndrome (GHIS) is a pathological state characterised by disturbance of the normal relationships between growth hormone (GH) secretion, insulin-like growth factor I (IGF-I) synthesis and GH action. Laron syndrome (LS) is the most severe form and is related to defects of the GH receptor gene. Twenty-seven cases of IS from 8 European countries and Australia were characterised clinically and endocrinologically. Clinical features (median) were; age 2.8-22.6 years, 12 males, 15 females, birth weight -0.72 SDS, birth length -1.59 SDS. Hypoglycaemia occurred in 33% and micropenis in 58% of males. Height was -6.0 SDS, weight -3.2 SDS, % weight for height 111.3. Bone age was delayed in 19 of the 27 patients. Endocrine values (median) were; GH 17μg/L, IGF-I<5th centile, with% increment during IGF-I generation test <20%. IG1411313-3 was <5th centile, GH-BP was low or undetectable in 20 and normal in 7 subjects.Treatment with recombinant IGF-I offered the only form of effective therapy. Treatment of 13 patients with IGF-I, 120μg/kg bid induced a change in mean height velocity from 4.1cm/year before treatment to 10.2cm/year at 6 months and 8.8cm/year at 12 months. Adverse effects were minimal. Facial appearance showed a change in maturity associated with capital hair growth. Further studies to define the optimum dose regimen of IGF-I are in progress.

clinical pediatric endocrinology

Growth Hormone Insensitivity Syndrome (GHIS): Clinical Presentation and Effects of Treatment with Recombinant Human Insulin-like Growth Factor I (rhIGF-I) European Experience