• Journal of mucopolysaccharidosis and rare diseases
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• v.3 no.2
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• pp.44-46
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• 2017

• Clinical and Experimental Pediatrics
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• v.52 no.8
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• pp.922-929
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• 2009

Purpose : The aim was to investigate the clinical characteristics and responses to growth hormone (GH) therapy in children with attenuated growth who showed normal GH responses to GH stimulation tests (GHST). Methods : The study included 39 patients with height velocity (HV) of less than 4 cm/yr and normal GHST results. Clinical characteristics of patients were analyzed retrospectively. Results : Eleven were born as small for gestational age (SGA) and 28 as appropriate for age (AGA). In the SGA group, the standard deviation score (SDS) of age and height measured at their first visit was significantly low. Sixteen patients were treated with GH and six of 23 without GH therapy were followed for 1 year after GHST. The mean (range) of HV was 7.7 (4.9 to 11.1) cm/yr in patients with GH therapy and 3.7 (2.7 to 4.5) cm/yr in those without GH therapy, which was statistically significant (P<0.001). In the GH-treated group, HV and difference in height SDS during the treatment increased significantly (P<0.001; P< 0.001, respectively). HV increased after 1 year of GH therapy in the SGA and AGA groups (SGA, P=0.043; AGA, P=0.003). The level of Insulin-like growth factor-I was significantly lower in GH-treated patients with height SDS <-3 than those with ${\geq}3$ (P=0.023). Conclusion : In children with growth failure and normal GHST, HV increases significantly by short-term GH therapy. The assessment of long-term effects of GH therapy is necessary. Moreover, further studies should be considered to evaluate the GH-IGF-I axis due to the possibility of GH insensitivity syndrome.

• Clinical and Experimental Pediatrics
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• v.53 no.3
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• pp.294-299
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• 2010

Although the increasing incidence of central precocious puberty (CPP) in Korea has recently raised public concerns about health and growth problems, there are many areas of uncertainty regarding the pathogenesis, diagnosis, and management of CPP. In this paper, we review the definition of precocity, the assessment of CPP, and the hormonal abnormalities that support the diagnosis. In addition, we review the practical guidelines regarding the clinical use of gonadotropin-releasing hormone analogs in children with CPP. Indications for treatment, determination of dosage, monitoring during treatment, and discontinuation of therapy are discussed.

• The Korean Journal of Physiology
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• v.10 no.1
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• pp.67-71
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• 1976

Effect of Electro Convulsion Therapy (ECT) on the Human Growth Hormone (HGH) response was observed in 32 cases of various mental illness. The patients were fasting overnight and ECT was induced in the morning by application of $90{\sim}120V$ for a period of $0.1{\sim}0.5\;sec$. In all patients, venous blood samples were taken prior to simultaneously with 1 and 4 hours after the ECT. Plasma was immediately seperated and stored in $-20^{\circ}C$ refrigerator. HGH concentrantion was determined by means of radioimmunoassay Results were as follows: 1. Application of electric current was not followed by a significant change in mean plasma HGH of 52 cases. 2 Increases of plasma HGH following ECT were observed in about half cases showing low initial level but in 20 other cases with high initial level of plasma HGH, reasonable decreases after therapy were not found. 3. Plasma concentration of HGH one hour after application of current were always higher than just after the current. 4. There were no differences between HGH responses to a 1st, 2nd or 5th ECT. 5. Discussions were made on the possible modification of HGH response following ECT by the autoregulatory control mechanism.

• Korean Journal of Clinical Laboratory Science
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• v.49 no.4
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• pp.495-497
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• 2017

Thalassemia major is a genetic disorder with a defective synthesis of either the alpha or the beta chain of hemoglobin A. Blood transfusion is crucial for the survival in these patients. Unfortunately, endocrine dysfunction is a very common complication in these patients and is principally due to excessive iron overload as a result of frequent blood transfusions. Although regular blood transfusion may increase life expectancy, disturbances in growth and pubertal development, abnormal gonadal functions, impaired thyroid, parathyroid and adrenal functions, diabetes, and disorderly bone growth are common side effects. We hereby present a case of a 23-year-old, unmarried woman with beta thalassemia major presenting with primary amenorrhea, poor development of secondary sexual character, and short stature. Thorough history, clinical examination, and laboratory investigation, including dynamic function test (insulin tolerance test) were conducted. These tests confirmed that she had multiple endocrinopathies, including hypogonadotropic hypogonadism, growth hormone deficiency, and subclinical adrenal insufficiency, which were caused by iron overload. She required hormone replacement therapy. Early recognition of possible deficiencies in hypothalamo-pituitary-end organ hormones caused by iron overload in thalassemia patients that undergo frequent blood transfusion procedures is essential. Appropriate treatments, including transfusion regimen and chelation therapy, as well as specific treatment of each complication are the crucial for the successful management and improvement of quality of life these patients.

• Clinical and Experimental Pediatrics
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• v.48 no.8
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• pp.865-870
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• 2005

Purpose : The use of growth hormone(GH) to promote growth in normal short children without classical GH deficiency is controversial. Numerous foreign studies have shown the effects of GH therapy in children with idiopathic short stature(ISS) whereas few has been interested in Korea. Therefore, this study is designed to investigate the effects of GH therapy on ISS by observing correlations and changes among various growth parameters such as, insulin-like growth factor-I(IGF-I) and insulin-like growth factor binding protein-3(IGFBP-3). Methods : This study was conducted retrospectively with 15 children with ISS in Chungbuk National University Hospital in Korea. Mean age was $11.44{\pm}2.81$ and the children were treated with 0.66 IU/kg/wk dosage of GH for 1 or 2 years. Also, the growth parameters before and after the GH therapy were observed. Results : Height standard deviation score(HT-SDS) was increased from $-1.85{\pm}0.70$ to $-1.58{\pm}0.56$ at 1 year and to $-1.21{\pm}0.37$ at 2 years after GH therapy. Predicted adult height standard deviation score(PAH-SDS) was also increased from $-2.10{\pm}0.52$ to $-1.67{\pm}0.59$ at 1 year, and to $-0.96{\pm}0.60$ at 2 years. Serum IGF-I and IGFBP-3 levels were significantly increased after 1 year and marginally increased after 2 years of GH therapy. Conclusion : It is concluded that GH therapy has growth promoting effect. The significant increase in IGF-I and IGFBP-3 levels during the GH therapy suggests that IGF-I and IGFBP-3 are useful predictors of response to the use of GH therapy. It is expected that larger patient samples would provide more reliable information about the effect of GH therapy.

• The korean journal of orthodontics
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• v.40 no.4
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• pp.227-238
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• 2010

Objective: The purpose of this study was to analyze the effect of growth hormone treatment (GHT) on craniofacial growth in children of short stature. Methods: Nineteen untreated children of short stature were referred from the Pediatric Department, Yeungnam University Hospital as a subject group. All subjects had lateral cephalograms taken before, after 1 year and after 2 years of growth hormone treatment. As a reference group, we selected 19 normal children with paired sampling who matched the subjects' age and sex, from the Department of Orthodontics, Kyungpook National University Hospital. Results: Before GHT, anterior cranial base length and upper posterior facial height, posterior total facial height, mandibular ramus length, and mandibular corpus length were significantly smaller in the reference group. In angular craniofacial measurements, saddle angle and mandibular plane angle were larger. SNA and SNB were smaller in the reference group. After two years of GHT, growth hormone accelerated growth in several craniofacial components. The posterior total facial height, the anterior, posterior cranial base length, and the mandibular ramus length were increased. And the difference in mandibular plane angle and ANB values compared with the reference group was decreased. Conclusions: GHT over 2 years leads to a craniofacial catch-up growth tendency, which is pronounced in interstitial cartilage and condylar cartilage.

• Journal of Genetic Medicine
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• v.20 no.2
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• pp.60-69
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• 2023

Purpose: Despite enzyme replacement therapy (ERT) and/or allogeneic hematopoietic stem cell transplantation, individuals with mucopolysaccharidosis (MPS) I or II often experience significant growth deficiencies. This study aimed to assess the safety and efficacy of recombinant human growth hormone (hGH) treatment in children diagnosed with MPS I or II. Materials and Methods: A total of nine pediatric patients-four with MPS I and five with MPS II-underwent treatment with ERT and hGH at Samsung Medical Center. Results: The mean hGH dose administered was 0.26±0.03 mg/kg/week. In the MPS I group, three patients showed an increase in height Z-score from -4.09±0.83 to -3.68±0.43 after 1 year of hGH treatment, and to -3.10±0.72 by the end of the hGH regimen. In the MPS II group, while the height Z-score of four patients decreased according to standard growth charts, it improved from 1.61±1.79 to 2.71±1.68 based on the disease-specific growth chart through hGH treatment. Two patients discontinued hGH treatment due to lack of efficacy after 22 and 6 months each of treatment, respectively. No new-onset neurological symptoms or necessity for prosthetic or orthopedic surgery were reported during hGH treatment. Conclusion: This study provides insights into the impact of hGH on MPS patients, demonstrating its potential to reverse growth deceleration in some cases. Further research is needed to explore the long-term effects of hGH on changes in body composition, muscle strength, and bone health in this population.

• The Korean Journal of Pain
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• v.12 no.1
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• pp.27-35
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• 1999

Background: Fibromyalgia is a common syndrome of musculoskeletal pain and fatigue. Lacking distinctive histological or laboratory abnormality in diagnosis, it has often been considered a form of "psychogenic rheumatism". Fibromyalgia causes much distress to the affected patients and often frustrates physicians, who are unable to start rational therapy on any logical disease pathology. Methods: Growth hormone is essential for muscular homeostasis. In the present study, the notion that the stage-4 sleep anomaly typically seen in the fibromyalgia syndrome may disrupt growth hormone secretion was tested. Because growth hormone has a very short half-life, serum levels of somatomedin C were measured; somatomedin C is the major mediator of growth hormone's anabolic actions and is a prerequisite for normal muscle homeostasis. Serum levels of somatomedin C using acid-extraction procedure and two-site immunoradiome-tric assay (IRMA) and number of tender points were measured in 27 female patients with fibromyalgia from 40 to 60 years old and 27 healthy controls. Results: There were no differences in the concentration of somatomedin C between fibromyalgia patients and controls ($mean{\pm}SD$: $178.3{\pm}75.5$ ng/ml versus $166.3{\pm}76.6$ ng/ml; p=0.55). And there were no correlations between number of tender point and serum somatomedin C level by linear regression analysis. Conclusions: These findings did not support that there is a distinctive disruption of the growth hormone-somatomedin C neuroendocrine axis in a fibromyalgia syndrome. But we can not discard the hypothesis that disturbed sleep predispose to muscle pain.

• Clinical and Experimental Pediatrics
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• v.53 no.10
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• pp.892-897
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• 2010

Purpose: This study evaluated the prevalence of the metabolic syndrome (MetS) and risk factors for metabolic derangement in young adults with childhood-onset hypopituitary growth hormone deficiency (ACOHGHD). Methods: Thirty patients with ACOHGHD who were treated with hormone-replacement therapy, aged 18 to 29 years, who visited the Seoul National University Children's Hospital between September 2009 and February 2010 were enrolled. Height, weight, waist circumference, hip circumference, and blood pressure were measured, and the clinical and hormonal features were reviewed retrospectively. We evaluated measures of metabolic derangement in the enrolled patients and in the data of healthy adults aged 20 to 29 years taken from the 2005 Korean National Health and Nutrition Examination Survey (KNHANES) as part of the National Cholesterol Education Program-the Adult Treatment Panel III. Results: Compared with the KNHANES participants, patients with ACOHGHD had significantly large waist circumference (men and women), high systolic blood pressure (BP) (women) and diastolic BP (men), and high serum triglyceride levels (women). The duration of illness correlated significantly with central obesity ($r^2$=0.546, $P$=0.003). The prevalence of MetS was 10% in patients with ACOHGHD and 2.3% in KNHANES participants. The prevalence of central obesity and MetS was higher in patients with ACOHGHD than in KNHANES participants ($P$<0.001 and $P$=0.042, respectively). Conclusion: Abdominal obesity correlated with the duration of illness in patients with ACOHGHD. Waist circumference should be measured in the clinic to prevent MetS, particularly in patients with a long history of ACOHGHD, regardless of age or sex.