• Clinical and Experimental Pediatrics
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• v.55 no.7
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• pp.224-231
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• 2012

Prader-Willi syndrome (PWS) is a complex multisystem genetic disorder characterized by hypothalamic-pituitary dysfunction. The main clinical features include neonatal hypotonia, distinctive facial features, overall developmental delay, and poor growth in infancy, followed by overeating with severe obesity, short stature, and hypogonadism later in development. This paper reviews recent updates regarding the genetic aspects of this disorder. Three mechanisms (paternal deletion, maternal disomy, and deficient imprinting) are recognized. Maternal disomy can arise because of 4 possible mechanisms: trisomy rescue (TR), gamete complementation (GC), monosomy rescue (MR), and postfertilization mitotic nondisjunction (Mit). Recently, TR/GC caused by nondisjunction at maternal meiosis 1 has been identified increasingly, as a result of advanced maternal childbearing age in Korea. We verified that the d3 allele increases the responsiveness of the growth hormone (GH) receptor to endogenous GH. This paper also provides an overview of endocrine dysfunctions in children with PWS, including GH deficiency, obesity, sexual development, hypothyroidism, and adrenal insufficiency, as well as the effects of GH treatment. GH treatment coupled with a strictly controlled diet during early childhood may help to reduce obesity, improve neurodevelopment, and increase muscle mass. A more active approach to correct these hormone deficiencies would benefit patients with PWS.

• Childhood Kidney Diseases
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• v.13 no.1
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• pp.14-20
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• 2009

Growth retardation is a common consequenc of chronic kidney disease (CKD) in childhood. Many recent clinical and experimental data indicate that growth failure in CKD is mainly due to a relative GH insensitivity and functional IGF-I deficiency. Glucocorticoids also glucocorticoids interfere with the integrity of the somatotropic hormone axis at various levels. Over the past 10 years, recombinant growth hormone (rhGH) has been used to help short children with chronic kidney disease. A GH dosage of 0.35 mg/kg/week (28 IU/$m^2$/week) appears efficient and safe. Some clinical trial data show that final height will be within the normal target height range when GH treatment is continued for many years without remarkable adverse events.

• Korean Journal of Animal Reproduction
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• v.22 no.1
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• pp.81-87
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• 1998

This experiment was carried out with 12 Korean native heifers(8~12month old, body weight, 160~240kg) raised at a farm of Chang-Soo Livestock Cooperatives to evaluate the effects of rGH(recombinant growth hormone) on serum concentrations of growth hormone, estrogen, and IGF-I, weight gain, teat volume gain and processing enzyme activity of IGF-I, binding protein III at 28 day intervals. Animals used were injected with 250mg rGH at 14 day intervals from December to Ferbruary in 1994. The significant difference was found in the group of treatment on the 4th week in the endogenous GH(p<.01) and 8th week in estrogen and IGF-I(p<.05) after injectin of rGH in Korean native heifers. There were significant differences between control group and treatment group in weight and teat volume on 8th week after treatment(p<.05). Processing enzyme activity before injection of rGH were low. However, heifers injected with 250mg of rGH showed that processing enzyme activity of IGF binding protein was highly increased throughout the experiment. Present results suggest that injection of exogenous rGH to heifers can increase the growth performance and udder development of Korean native heifers by the endogenous hormonal changes.

• 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.

• Biomolecules & Therapeutics
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• v.8 no.4
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• pp.285-292
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• 2000

The effects of the members of the same nuclear receptor superfamily (all-trans retinoic acid (RA), thyroid hormone(T3) or hydrocortisone) on proliferation and differentiation in the SK-N-SH neuroblastoma (NB) cell lines were studied. NB cells were treated with RA, T3, or hydrocortisone at concentration of 10$^{-6}$ M or 10$^{-8}$ M for 3 days or 7 days. RA induced concentration- and time-dependent morphologic differentiation(neurite outgrowth and microtubule-associated protein expression) and growth inhibition in NB cells. Treatment of 10$^{-7}$ M T3 for 7 days increased viability and differentiation of NB cells. Treatment of 10$^{-6}$ M hydrocortisone for 7 days increased viability of NB cells. Although these three effectors are members of the same receptor superfamily, the regulation of brain development may be carried out in a different manner.

• Journal of Korean Medicine for Obesity Research
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• v.9 no.1
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• pp.59-69
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• 2009

Objectives This study was designed to evaluate the difference of between Kyungohkgo and Kyungohkgo Ga Nokyong effects of growth Methods We divided male Spraque-Dawley rats into 6 groups. They were Normal group, Growth deficiency rat with insufficient nutrition diet group, Growth deficiency rat with 0.1% Kyungohkgo group, Growth deficiency rat with 0.2% Kyungohkgo group, Growth deficiency rat with 0.1% Kyungohkgo Ga Nokyong group and Growth deficiency rat with 0.2% Kyungohkgo Ga Nokyong group. They were administered for 5 weeks. We measured body weight, serum growth hormone, insulin-like growth factor and thyroid stimulating hormone, RBC, concentration of Hb and PCV ratio, total WBC and its composition, the values of plasma glutamic oxaloacetic transaminase(GOT) and glutamic pyruvic transaminase(GPT) activities. Results 1. In body weight, Kyungohkgo 0.1%, Kyungohkgo Ga Nokyong 0.1%, 0.2% groups were showed sighnificantly different than control group. But That groups were not showed significantly different than each others. 2. In serum growth hormone, insulin-like growth factor and thyroid stimulating hormone, we obtained the results of tendency to increase in Kyungohkgo Ga Nokyong group, however these values showed no significantly different. 3. In the counts of RBC, Kyungohkgo group and Kyungohkgo Ga Nokyong group were showed significantly different than control group. But these values showed no significantly different. In the concentration of Hb, Kyungohkgo Ga Nokyong 0.2%group was showed significantly different than control group. Kyungohkgo Ga Nokyong 0.2% group and Kyungohkgo 0.2% group were showed significantly different than control group. 4. The counts of total WBC and its composition showed no significantly different in all treatment groups. 5. The values of plasma glutamic oxaloacetic transaminase(GOT) and glutamic pyruvic transaminase(GPT) activities showed no significantly different in all treatment groups. Conclusions So Kyungohkgo Ga Nokyong and Kyungohkgo have an effect of promoting growth of rats. And We know that the effect of Kyungohkgo Ga Nokyong is better than Kyungohkgo.

• 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.

• Asian-Australasian Journal of Animal Sciences
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• v.29 no.10
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• pp.1451-1457
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• 2016

A comparative study was performed to investigate the efficacy of KiFAY as a feed additive on performance parameters, thyroid, and pancreatic hormone levels in broilers. Ninety birds (Vencobb 400) were randomly divided into three groups viz., Control (no DL-methionine supplementation), Treatment1 (containing added DL-methionine) and Treatment 2 (containing KiFAY and without DL-methionine supplementation). The performance parameters (weekly body weight, body weight gain, feed intake, and feed consumption ratio) were recorded and calculated during the whole study of 4 weeks. Analyses of insulin and insulin-like growth factor (IGF 1), triiodothyronine (T3), thyroxine (T4) and thyroid stimulating hormone (TSH) were performed at the end of the study. The results show that birds on supplementation of KiFAY performed significantly (p<0.001) better than other treatments. The weekly body weight, body weight gain, feed in-take and feed consumption ratio improved in KiFAY treated birds. The study found an increase in insulin and IGF1 levels (p<0.001) in KiFAY compared with the other treatments. Serum T3, T4, and TSH levels in the Treatment 2 were higher than other treatments (p<0.001). The KiFAY supplementation was able to improve performance with associated responses at a hormonal level in broilers.

• Journal of Aquaculture
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• v.12 no.2
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• pp.79-89
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• 1999

The recombinant bovine somatotropin (rBST) was administered to the oliver flounder, Paralichthys olivaceus, to know the effects and optimal administration frequency and dosage of the hormone. The experiment was conducted with three different treatment groups (A, B and C) designated based on the duration and administration frequency of rBST and one control (D) from April 14, 1996 to March 16, 1997. The fish of hormone treated groups grew 7.86 to 10.07% (47.45 to 60.75 g in weight) better than the control at the end of the experiment (P<0.05). No significant differences in their growth were detected among treatment groups. The distinct growth improvement was recognized four weeks after completion of the first four hormone administration. When considering water temperatures measured from the experimental tanks, the effect of rBST on the flounder was greater during the period showing relatively lower temperature. The survival rates were higher in treatment groups than in the control, revealing 98.3% (A), 98.4% (B), 97.7% (C) and 93.1% (D) during the first stage of culture; 92.7% (A), 91.3% (B), 86.7% (C) and 80.0% (D) during the second stage of culture.

• Journal of mucopolysaccharidosis and rare diseases
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• v.3 no.2
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• pp.41-43
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• 2017

Prader-Willi syndrome (PWS) is a genetic disorder that is considered, especially on child, to cause poor feeding, hypotonia, failure to thrive, developmental delay and hypogonadism which is known to affect between 1 in 10,000 and 30,000 people. The children with PWS are viewed as affected by growth hormone (GH) insufficiency, although the exact mechanisms of GH deficiency are not fully understood. However, the benefits of GH treatment in children with PWS are well established. Myers, et al. (2006), Grugni, et al. (2016) indicated its positive effects on linear growth, body composition, motor function, respiratory function and psychomotor development. Despite of its effectiveness and advantages had been well known and proven in many other studies, there is only one recombinant GH product that is approved for PWS in Korea, $Genotropin^{(R)}$, till now. A phase III clinical study of GH treatment with $Eutropin^{TM}$, in 34 Korean PWS children is in progress, which is expected to have comparable effects and safety profile with the active control by assessing auxological changes such as height standard deviation score, body composition changes such as lean body mass and percent body fat, motor and cognitive development using Bayley scale, and safety profiles.