Effect of Growth Hormone Therapy in Patients with Noonan Syndrome: A Retrospective Study

Background: Noonan syndrome is an autosomal dominant condition with an incidence of 1:1000 to 1:2500. The disorder is associated with distinct dysmorphic features, cardiac anomalies, developmental delay and delayed puberty. Short stature is a recognised feature of Noonan syndrome. Objectives: The aim of this study is to assess the effect of growth hormone treatment in patients with Noonan syndrome. Methods: Retrospective data was collected from patients with Noonan syndrome treated with growth hormone. The results were analysed with variables expressed as mean values and standard deviation scores. Results: Twelve Noonan syndrome patients (M: F = 10:2) treated with growth hormone were identified. The mean age of starting growth hormone was 8 years, with baseline height standard deviation score of -2.96 (range: -1.64 to -5.54). The height standard deviation score significantly improved to -2.50 (P = 0.0035) and then -2.22 (P = 0.0025), following one and two years of treatment, respectively. The average height velocity for the patients prior to starting treatment was 5.16cm/year (range: 2.4 - 8.2 cm/year), which significantly improved to 7.76cm/year (ranging from 4.1 to 12.8 cm/year) after one year of growth hormone treatment (P = 0.020) and to 6.51cm/year at the end of two years. Conclusions: Our study has shown that growth hormone treatment significantly improves the height standard deviation score of patients with Noonan syndrome over a two-year course of growth hormone therapy without any side effects. Further research is required to analyse the long-term effect of growth hormone therapy in patients with Noonan syndrome, including the impact on final adult height.

Download Full-text

Occurrence of Cranial Neoplasms in Pediatric Patients with Noonan Syndrome Receiving Growth Hormone: Is Screening with Brain MRI prior to Initiation of Growth Hormone Indicated?

Hormone Research in Paediatrics ◽

10.1159/000479107 ◽

2017 ◽

Vol 88 (6) ◽

pp. 423-426 ◽

Cited By ~ 5

Author(s):

Kanthi Bangalore Krishna ◽

Pedro Pagan ◽

Oscar Escobar ◽

Jadranka Popovic

Keyword(s):

Growth Hormone ◽

Brain Tumors ◽

Hormone Therapy ◽

Noonan Syndrome ◽

Growth Hormone Treatment ◽

Growth Hormone Therapy ◽

Brain Mri ◽

Hormone Treatment ◽

Tumor Burden ◽

Primary Brain Tumors

Noonan syndrome (NS) is associated with short stature. Growth hormone treatment has been FDA approved for use in these patients. Children with NS are at a higher risk of developing benign and malignant proliferative disorders, primary brain tumors being one of them. Since growth hormone therapy can worsen the tumor burden, screening with a brain MRI prior to growth hormone initiation in NS patients is strongly recommended. Here we present two NS patients who developed different primary brain tumors while being on growth hormone therapy.

Download Full-text

ORIGINAL ARTICLE: Should short children born small for gestational age with a distance to target height <1 standard deviation score be excluded from growth hormone treatment?

Clinical Endocrinology ◽

10.1111/j.1365-2265.2010.03833.x ◽

2010 ◽

Vol 73 (3) ◽

pp. 355-360

Author(s):

Annemieke J. Lem ◽

Sandra W. K. De Kort ◽

Maria A. J. De Ridder ◽

Anita C. S. Hokken-Koelega

Keyword(s):

Growth Hormone ◽

Standard Deviation ◽

Gestational Age ◽

Small For Gestational Age ◽

Growth Hormone Treatment ◽

Standard Deviation Score ◽

Hormone Treatment ◽

Target Height ◽

Deviation Score ◽

Short Children

Download Full-text

Effect of One-Year Growth Hormone Therapy on Cardiometabolic Risk Factors in Boys with Obesity

BioMed Research International ◽

10.1155/2020/2308124 ◽

2020 ◽

Vol 2020 ◽

pp. 1-8

Author(s):

Jing Wu ◽

Fei Zhao ◽

Yuan Zhang ◽

Jiang Xue ◽

Jiangying Kuang ◽

...

Keyword(s):

Growth Hormone ◽

Hormone Therapy ◽

Human Growth Hormone ◽

Growth Hormone Treatment ◽

Growth Hormone Therapy ◽

Recombinant Human Growth Hormone ◽

Human Growth ◽

Hormone Treatment ◽

Risk Markers ◽

One Year

It has been recognized that people with obesity are more likely to have low growth hormone secretion. Recent studies have also confirmed that the abnormalities of the growth hormone/insulin-like growth factor 1 axis were associated with cardiovascular complications in people with obesity. However, little is known about whether recombinant human growth hormone therapy could improve cardiovascular and metabolic risks in obese children. This study aims to evaluate the effect of one-year growth hormone therapy on obesity-related comorbidities and to assess the safety in Chinese boys with obesity. Eighteen boys with obesity were treated with recombinant human growth hormone for one year. Anthropometric measurements, endocrine testing, and cardiovascular risk markers were performed in all obese boys in baseline, and follow-up visits were performed at 3 months, 6 months, 9 months, and one year, respectively. After one year of recombinant human growth hormone treatment, the body mass index standard deviation scores decreased (P<0.001) and insulin-like growth factor 1 levels increased (P<0.001). GH treatment also reduced low density lipoprotein cholesterol (P<0.001), total cholesterol (P<0.001), triglycerides (P=0.042), and alanine aminotransferase (P=0.027) when compared with the baseline. One-year of recombinant human growth hormone treatment could improve cardiometabolic risk markers, without adverse effects on glucose homeostasis in boys with obesity.

Download Full-text

Assessment of anterior-posterior spinal curvatures in children suffering from hypopituitarism

BMC Endocrine Disorders ◽

10.1186/s12902-019-0462-4 ◽

2019 ◽

Vol 19 (1) ◽

Author(s):

Magdalena Kobylińska ◽

Roksana Malak ◽

Katarzyna Majewska ◽

Andrzej Kędzia ◽

Włodzimierz Samborski

Keyword(s):

Growth Hormone ◽

Hormone Therapy ◽

Growth Hormone Treatment ◽

Growth Hormone Therapy ◽

Alpha Angle ◽

Hormone Treatment ◽

The Body ◽

Body Posture ◽

Spinal Curvatures ◽

Anterior Posterior

Abstract Background Body posture may be disordered by vestibular dysfunction, neurological disorders, problems with the distribution of muscle tone, brain injuries, and other dysfunctions. Growth hormone deficiency (GHD) can lead to many disorders, particularly of the musculoskeletal system. During treatment with recombinant human growth hormone (rhGH), an increase in muscle mass and an improvement in bone structure can be observed in children suffering from hypopituitarism from GHD. Methods The study involved 33 children suffering from hypopituitarism with GHD (9 girls and 24 boys), aged 10–14 years old. Measurements of the magnitude of their anterior–posterior spinal curvatures were made using an inclinometer. The children were examined at the medianus of the sacrum bone, the Th12–L1 intervertebral area, and the C7–Th1 intervertebral area. In order to characterize the anterior–posterior curvature of the spine, the results were compared with the general norms reported by Saunders. Statistical calculations were carried out using the statistical package Statistica 10 PL. Results Lumbar lordosis angles were higher in the patients currently receiving growth hormone (GH) treatment than in those who had yet to receive it. There is a statistically significant positive correlation between the length of growth hormone treatment and the alpha angle. There are also statistically significant correlations between age at the beginning of growth hormone therapy and the angle of lordosis. Statistically significant correlations were also seen between age at the beginning of growth hormone therapy and the alpha angle. Conclusions Although there may be changes in posture at the beginning of rhGH treatment, the sooner growth hormone therapy begins, the better the body posture. The longer the growth hormone treatment, the better the posture, as expressed by the alpha angle in the sagittal plane.

Download Full-text

Long-acting Growth Hormone Therapy: A REAL3 Alternative to Daily Growth Hormone Treatment?

The Journal of Clinical Endocrinology & Metabolism ◽

10.1210/clinem/dgaa074 ◽

2020 ◽

Vol 105 (4) ◽

pp. e1921-e1924

Author(s):

Vaneeta Bamba ◽

Michael A Levine

Keyword(s):

Growth Hormone ◽

Hormone Therapy ◽

Growth Hormone Treatment ◽

Growth Hormone Therapy ◽

Hormone Treatment ◽

Daily Growth ◽

Long Acting

Download Full-text

Is a Combination of a GnRH Agonist and Recombinant Growth Hormone an Effective Treatment to Increase the Final Adult Height of Girls with Precocious or Early Puberty?

International Journal of Endocrinology ◽

10.1155/2018/1708650 ◽

2018 ◽

Vol 2018 ◽

pp. 1-10 ◽

Cited By ~ 1

Author(s):

Wei Song ◽

Fei Zhao ◽

Shuang Liang ◽

Guimei Li ◽

Jiang Xue

Keyword(s):

Growth Hormone ◽

Standard Deviation ◽

Adult Height ◽

Combined Therapy ◽

Standard Deviation Score ◽

Height Standard Deviation Score ◽

Early Puberty ◽

Deviation Score ◽

Final Adult Height ◽

Rhgh Treatment

The aim of treatment for idiopathic central precocious puberty (ICPP) is to increase final adult stature, for which gonadotropin-releasing hormone agonist (GnRHa) is the gold standard. Early puberty is frequently similar to ICPP, with pubertal onset only slightly advanced. Short stature may result from early pubertal onset. Some studies have suggested that recombinant human growth hormone (rhGH) should be combined with a GnRHa to improve adult height, while others have not. Here, the aim was to compare the efficacy of combined GnRHa and rhGH treatment with GnRHa or rhGH treatment alone, or no therapy, for the improvement of the final height of girls with ICPP or early puberty. Electronic databases of randomized and quasi-randomized controlled trials, in which the efficacy of GnRHa preparations was compared with that of rhGH for the treatment of children with precocious or early puberty, were searched and a meta-analysis conducted. Five studies of early puberty and four studies of ICPP were identified. There were no statistically significant differences between final adult height standard deviation score and initial height standard deviation score in the treatment of early puberty (GnRHa and rhGH versus rhGH alone or no treatment). The overall analysis of the data failed to indicate any benefit of combined therapy, while individual reports suggested that in specific instances combined therapy may be beneficial in preserving or reclaiming growth potential and improving adult height.

Download Full-text

The Frequency of Bleeding and Height of Adolescent Haemophiliacs

Thrombosis and Haemostasis ◽

10.1055/s-0038-1646647 ◽

1979 ◽

Vol 41 (02) ◽

pp. 286-290 ◽

Cited By ~ 2

Author(s):

J L Bem ◽

M J Painter ◽

A Aronstam ◽

J H Patrick ◽

R E Newcombe

Keyword(s):

Standard Deviation ◽

Standard Deviation Score ◽

Height Standard Deviation Score ◽

Deviation Score

SummaryThe relation between the height of adolescent haemophiliacs and their bleeding frequency has been studied. 45 haemophiliacs aged 10-19 years were divided into 3 groups: small, medium and tall, using a Height Standard Deviation Score. The average bleeding frequency per 100 days in the group of small haemophiliacs was 8.71 ± SD 4.47, in the medium height group 10.18 ± SD 6.71, while the tall individuals bled in average 15.97 ± SD 3.15 every 100 days. There was no relationship between age and bleeding frequency.

Download Full-text