• Clinical and Experimental Pediatrics
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• v.53 no.9
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• pp.845-851
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• 2010

Purpose: Recombinant human growth hormone (rhGH) has been widely used to treat short stature. However, there are some concerns that growth hormone treatment may induce skeletal maturation and early onset of puberty. In this study, we investigated whether rhGH can directly affect the neuronal activities of of gonadotropin-releasing hormone (GnRH). Methods: We performed brain slice gramicidin-perforated current clamp recording to examine the direct membrane effects of rhGH on GnRH neurons, and a whole-cell voltage-clamp recording to examine the effects of rhGH on spontaneous postsynaptic events and holding currents in immature (postnatal days 13-21) and adult (postnatal days 42-73) mice. Results: In immature mice, all 5 GnRH neurons recorded in gramicidin-perforated current clamp mode showed no membrane potential changes on application of rhGH (0.4, $1{\mu}g/mL$). In adult GnRH neurons, 7 (78%) of 9 neurons tested showed no response to rhGH ($0.2-1{\mu}g/mL$) and 2 neurons showed slight depolarization. In 9 (90%) of 10 immature neurons tested, rhGH did not induce any membrane holding current changes or spontaneous postsynaptic currents (sPSCs). There was no change in sPSCs and holding current in 4 of 5 adult GnRH neurons. Conclusion: These findings demonstrate that rhGH does not directly affect the GnRH neuronal activities in our experimental model.

• Journal of Genetic Medicine
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• v.18 no.2
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• pp.110-116
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• 2021

Microdeletions of chromosome 22q11.2 are one of the most common microdeletions occurring in humans, and is known to be associated with a wide range of highly variable features. These deletions occur within a cluster of low copy repeats (LCRs) in 22q11.2, referred to as LCR22 A-H. DiGeorge (DGS)/velocardiofacial syndrome is the most prevalent form of a 22q11.2 deletions, caused by mainly proximal deletions between LCR22 A and D. As deletions of distal portion to the DGS deleted regions has been extensively studied, the recurrent distal 22q11.2 microdeletions distinct from DGS has been suggested as several clinical entities according to the various in size and position of the deletions on LCRs. We report a case of long-term follow-up of a female diagnosed with a 22q11.2 distal deletion syndrome, identified a deletion of 1.9 Mb at 22q11.21q11.23 (chr22: 21,798,906-23,653,963) using single nucleotide polymorphism array. This region was categorized as distal deletion type of 22q11.2, involving LCR22 D-F. She was born as a preterm, low birth weight to healthy non-consanguineous Korean parents. She showed developmental delay, growth retardation, dysmorphic facial features, and mild skeletal deformities. The patient underwent a growth hormone administration due to growth impairment without catch-up growth. While a height gain was noted, she had become overweight and was subsequently diagnosed with pre-diabetes. Our case could help broaden the genetic and clinical spectrum of 22q11.2 distal deletions.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.15 no.1
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• pp.9-17
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• 2015

Glycogen storage disease (GSD) type Ia is rare inborn metabolic disorder, caused by glucose-6-phosphatase deficiency. It characterized by hepatomegaly, hypoglycemia, lactic acidosis, hypertriglyceridemia, and hyperuricemia and it is usually manifested in the infantile period. In addition, it is also associated with growth failure, pubertal delay, anemia, platelet dysfunction, osteopenia, and pulmonary hypertension. Hepatocellular adenoma and renal dysfunction are frequent late complications. Delayed diagnosis and inappropriate therapy lead to many complications such as growth failure, osteoporosis, refractory gout, renal failure, hepatocellular carcinoma (HCC), and pulmonary hypertension. Here, two Korean sisters diagnosed with GSD Ia, aged 33 and 36 respectively, were described and compared to recent articles about four adults with late diagnosis of GSD Ia. One sister had typical manifestations of GSD Ia including short stature (height, 145 cm), multiple hepatic adenoma, chronic kidney disease stage IV, and severe osteoporosis, whereas the older sister had normal stature (162 cm), one tiny hepatic nodule, and normal renal function. Direct sequencing of G6PC in two sisters identified a homozygous splicing mutation, c.645G>T, which is a prevalent mutation in Korea. Interestingly, our cases and four adults from recent reports had asymptomatic mild hypoglycemia and various manifestations including renal failure, HCC, fatty liver, or uncontrolled hyperlipidemia. These adult cases represent not only heterogenous phenotype to genotype within family members with GSD Ia but also long-term complications such as gouty arthritis, renal failure, and osteoporosis in untreated adult GSD Ia patients. In addition, lactic academia and hypertriglyceridemia are good markers of GSD Ia to distinguish from metabolic disease.

• Childhood Kidney Diseases
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• v.10 no.2
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• pp.142-151
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• 2006

Purpose : Growth retardation is one of the serious problems in children with nephropathy requiring long-term steroid therapy. We observed the efficacy and safety of recombinant human growth hormone(rhGH) on the growth in children with long-term steroid therapy. Methods : We studied 60 children(male 47, female 13) with nephropathy who received rhGH(1 U/kg/week) for more than 0.5 years($1.39{\pm}1.12$). Their mean age was 11.0 years($11.17{\pm}2.62$). They received steroid therapy from January 1987 through July 2005, and the mean duration of steroid therapy was $4.32{\pm}2.97$ years. Among the patients, there were 32 nephrotic syndrome, 9 IgA nephropathy, 4 mesangial proliferative glomerulonephritis, 4 focal segmental glomerulosclerosis, 2 Henoch $Sch\ddot{o}nlein$ nephritis, 2 Alport syndrome and 7 other cases. Data were gathered on the growth parameters, such as growth velocity, height standard deviation score(SDS), IGF-1, IGFBP-3, bone mass density(BMD) and general chemistry changes. Results : Height velocity increased significantly with rhGH therapy from $3.29{\pm}1.95$ to $8.66{\pm}3.75$(cm/yr) and height SDS decreased from $-0.72{\pm}0.93$ to $-1.04{\pm}0.86$ at one year after steroid therapy but increased to $-0.55{\pm}0.96$ at one year after rhGH administration(P<0.05). BMD improved from $0.71{\pm}0.14$ to $0.79{\pm}0.15g/cm^2$(P<0.05). IGF-1 increased from $445.09{\pm}138.01$ to $506.62{\pm}181.31ng/mL$(P<0.05). IGFBP-3 decreased from $4073.75{\pm}700.78$ to $3933.61{\pm}789.25ug/L$ numerically, but there was no statistically significant difference(P=0.533). Conclusion : The administration of rhGH in the short stature patients who received long-term steroid therapy showed improvement in growth parameters such as SDS, growth velocity, and BMD without significant side-effects or changes in the biochemical parameters.

• The Korean Journal of Physiology
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• v.10 no.2
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• pp.11-16
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• 1976

In hold that the pulmonary functions which is important situation that the national physical power, at firstly, from age 10 to age 29 young men and women 1,540 person for the progress of grew up, and them to by each aged viewing transition for pulmonary functions physical examination record is as following; and to measure normal Person with comparison by each others pulmonary functions was gained as the results of follows. 1) Frequency of Breathing: It was same the male and female age at 10 per minute over or less of 20 times, but, at age 16 to 17 was $15{\sim}16$ times, and had to precensed compare constant value. 2) Vital Capacity: At the grew up Pregress term as the body stature, age 10 to 17 or 18, had to showing rapidly increasing, but the after Probably constant. The comparison value in man and women in progress of growth up was about $200{\sim}300ml$, and after growth of progress are about 1,500ml, add in men was presence more highly value. 3) Tidal Volume: At a9e 10 was 365m1, but, it was continuing increasing, an4 at age 20 is about 500m1, and the different in men and women about $20{\sim}30$ times and had precense highly value at men. 4) Breathing holding time: It was presence same at male and femele 10 to 13, from 35 second to $37{\sim}38$ seconds, and had to Presence a slight increasing, but, may has to final of the growth progrees age at 14, it should be presence rapidly increasing and the after probably presence as same value but, than female at male is high record average value of $7{\sim}8$ seconds.

• Annals of Pediatric Endocrinology and Metabolism
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• v.23 no.4
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• pp.204-209
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• 2018

Purpose: Growth hormone transduction defect (GHTD) is characterized by severe short stature, impaired STAT3 (signal transducer and activator of transcription-3) phosphorylation and overexpression of the cytokine inducible SH2 containing protein (CIS) and p21/CIP1/WAF1. To investigate the role of p21/CIP1/WAF1 in the negative regulation of the growth hormone (GH)/GH receptor and Epidermal Growth Factor (EGF)/EGF Receptor pathways in GHTD. Methods: Fibroblast cultures were developed from gingival biopsies of 1 GHTD patient and 1 control. The protein expression and the cellular localization of p21/CIP1/WAF1 was studied by Western immunoblotting and immunofluorescence, respectively: at the basal state and after induction with $200-{\mu}g/L$ human GH (hGH) (GH200), either with or without siRNA CIS (siCIS); at the basal state and after inductions with $200-{\mu}g/L$ hGH (GH200), $1,000-{\mu}g/L$ hGH (GH1000) or 50-ng/mL EGF. Results: After GH200/siCIS, the protein expression and nuclear localization of p21 were reduced in the patient. After successful induction of GH signaling (control, GH200; patient, GH1000), the protein expression and nuclear localization of p21 were reduced. After induction with EGF, p21 translocated to the cytoplasm in the control, whereas in the GHTD patient it remained located in the nucleus. Conclusion: In the GHTD fibroblasts, when CIS is reduced, either after siCIS or after a higher dose of hGH (GH1000), p21's antiproliferative effect (nuclear localization) is also reduced and GH signaling is activated. There also appears to be a positive relationship between the 2 inhibitors of GH signaling, CIS and p21. Finally, in GHTD, p21 seems to participate in the regulation of both the GH and EGF/EGFR pathways, depending upon its cellular location.

• Journal of Nutrition and Health
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• v.31 no.8
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• pp.1338-1346
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• 1998

North Korea has been experiencing a serious food shortage. However, the extent and magnitude of malnutrition and health problems are virturally unknown because a nationwide food and nutritional survey has never been conducted. To get a better udnerstanding of the situation., an exhaustive literature search on the health and nutritiona conditions of North Koreans was performed. We also obtained anthropometric data on 274 North Korean Defectors who are recently resettlers in the South. Some(137) measurements were taken at the time of entry to the South, and the remaining data was from the health and nutrition mail survey conducted by us on North Korean refugeee re-settlers. Health and inutritional status of North Koreans was found to be less than desirable as shown in high infant mortality rates, high mortality and morbidity rates of malnutriton-related infectious deseases, and smaller body sizes. Striking differences were noted in all of the above health and nutrition indicators between north and South Koreans. Considering the fact that North and South Koreans are homogeneous in their genetic back-ground, ours study results clearly demonstrate that the health and nutrition of North Koreas has deteriorated with the dhronic food shortage, which might have sorsened with a recent food crissi. The smaller and thinner adult stature proevailing in North Korea could play a significant negative role in the country's economy, since lower lean body mass has been associated with reduced work productivity that could hinder economic growth and development.

• Fashion & Textile Research Journal
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• v.14 no.2
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• pp.286-293
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• 2012

The purpose of this study was conducted to provide basic information on developing highschool-uniform pants with more suitable fit. To carry out this study, questionnaires were administered to 187 students in high schools in Gwangju. The collected data were analyzed with frequencies statistics, t-test, ANOVA using the SPSS WIN 18.0. The results are as follows; As results of research on highschool-uniform regulation, 88% of the highschool in Gwangju allowed their students to wear school uniform pants. As results of investigation on the school-uniform pants sizing system of manufacturing firm, it was found that there's a trend towards increasing a large size with the change body type. The size labelling for school uniform pants was waist circumference-stature. There were differences in pants sizes according to the manufacturing firm and body measurements such as hip circumference, in seam length, pants length. As results of survey on wearing conditions, the selection of school uniform pants has increased in winter season. The most important body measurement in the choice of purchase was thigh circumference. In fit of uniform pants, respondents answered that waist circumference and hip circumference became smaller according to the physical growth.

• Childhood Kidney Diseases
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• v.13 no.2
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• pp.256-260
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• 2009

The Russell-Silver syndrome (RSS) is a disease characterized by intrauterine growth retardation with preserved head circumference, facial dysmorphism and short stature. Reported renal and urinary manifestations of RSS include horseshoe kidney, renal tubular acidosis, hydronephrosis, ureteropelvic obstruction and vesicoureteral reflux. Here we report a case of end-stage renal disease associated with RSS, which, to the best of our knowledge, has not been reported yet.

• Asian Oncology Nursing
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• v.8 no.2
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• pp.120-127
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• 2008

Purpose: The purpose of this study was secondary analysis to explore about risk factors with breast cancer on a basis of primary literature. Methods: This study was searched articles by using CINAHL, MEDLINE, Riss4u, Internet website regarding breast cancer. This study searched for the journal published in Korea and foreign countries from 2000 to 2008, about risk factors of breast cancer. This study was reviewed 42 articles (5 experimental study, 35 survey, 1 qualitative study, 1 report) suitable for the research objectives. Results: Magnitude of risk breast cancer (++) was age, geographic region, family history, mutations in BRCA1, BRCA2 genes and in other penetrance genes, radiation, history of benign breast disease, late age of menopause, early age of menarch, nulliparity and older age at first birth, high mammographic breast density, high insulin-like growth factor 1 level. Magnitude of risk factor (+) was hormone replacement therapy, oral contraceptives use, obesity, tall stature, alcohol consumption, high prolactin level, high saturated fat and well-done meat intake, polymorphisms in low penetrance gene, high socioeconomic status. Conclusion: A breast cancer screening protocol according to magnitude of risk factors is needed for disease prevention. The nurses need to educate and counsel women with risk factors of breast cancer.