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Showing 2 results for Short Stature

Akrami S M, Yousefzadeh G R,
Volume 65, Issue 9 (12-2007)
Abstract

Background: Hutchinson-Gilford Progeria Syndrome (HGPS) is a very rare genetic disorder with a frequency of 1 in 8 million live births. It is characterised by premature aging phenotype. The median age at death is 13.4 years. It is an autosomal dominat disease due to a de novo point mutation in the Lamin A gene exon 11 in the majority of cases. More than 100 cases have been reported world wide.
Case report: We describe here an exceptionally long-lived patient with HGPS, who is alive at age 36. She was referred by a cardiologist to our endocrinology clinic to be worked up for presence of a metabolic or genetic disorder before a heart surgery.
Results: Having more attention of clinicians about very rare diseases and referring the patients to geneticist are the main goals of this case report as well as describing the disease.


Samira Shiri , Mahmoud Ghasemi, Khairollah Asadollahi, Maryam Deldar , Kourosh Sayehmiri ,
Volume 79, Issue 9 (12-2021)
Abstract

Background: Short children face many problems throughout their lives. Consumption of growth hormone and the drugs such as Letrozole, Oxandrolone, and Anastrozole can increase the growth of children and adolescents. There is not an overall estimate of the effects of Letrozole, Oxandrolone, and Anastrozole on the growth of children and adolescents with Idiopathic Short Stature (ISS). There are different estimates of the effect of growth hormone and the drugs Letrozole, Oxandrolone, and Anastrozole on growth in children and adolescents with Idiopathic Short Stature. Therefore, the aim of this study was to compare the efficacy of growth hormone, Letrozole, Oxandrolone, Anastrozole, and a combination of growth hormone and aromatase inhibitors on height gain in children and adolescents with idiopathic short stature using meta-analysis method.
Methods: Search was done in databases such as; Scopus, Web of Science(ISI), and PubMed using keywords: Idiopathic, Short Stature, Body Height, Aromatase Inhibitors, Growth hormone, Letrozole, Oxandrolone, Anastrozole. Randomized clinical trial studies ,that have investigated the efficacy of growth hormone and aromatase inhibitors on height gain in children and adolescents with short stature, were selected. Height standard deviation score (HSDS) before intervention and after treatment has been used to measure the rate of height increase in various studies. This study was written based on the PRISMA checklist and the heterogeneity of this study was evaluated using the Q statistic and I2 index.
Results: Height increase index (HSDS) was obtained with the use of growth hormone combined with one of the aromatase inhibitors (0.38-3.58=95%CI) (SMD=0.98), with the use of growth hormone (0.62-1.14=95%CI) (SMD=0.88), with the use of Letrozole (0.51-16.51=95%CI) (SMD=0.83), with the use of Oxandrolone (0.99-0.99=95%CI) (SMD=0.56), and with the use of Anastrozole (0.00-0.63=95%CI) (SMD=0.31), which shows that all these drugs have a significant effect on height increase. (P<0.05)
Conclusion: Although the effect of growth hormone on height increase was greater than that of aromatase inhibitors, but according to our results, adding an aromatase inhibitor to growth hormone can increase the effectiveness of it.


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