• Title/Summary/Keyword: Growth disorders

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Region Specific Brain Organoids to Study Neurodevelopmental Disorders

  • Praveen Joseph Susaimanickam;Ferdi Ridvan Kiral;In-Hyun Park
    • International Journal of Stem Cells
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    • v.15 no.1
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    • pp.26-40
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    • 2022
  • Region specific brain organoids are brain organoids derived by patterning protocols using extrinsic signals as opposed to cerebral organoids obtained by self-patterning. The main focus of this review is to discuss various region-specific brain organoids developed so far and their application in modeling neurodevelopmental disease. We first discuss the principles of neural axis formation by series of growth factors, such as SHH, WNT, BMP signalings, that are critical to generate various region-specific brain organoids. Then we discuss various neurodevelopmental disorders modeled so far with these region-specific brain organoids, and findings made on mechanism and treatment options for neurodevelopmental disorders (NDD).

A Study on the Correlation between Anterior Tooth Contacts and Cephalometric Profile in Patients with Craniomandibular Disorders (두개하악장애환자의 전치부접촉과 측모두부형태간의 관계에 관한 연구)

  • Byung-Wook Kim;Kyung-Soo Han;Min Shin
    • Journal of Oral Medicine and Pain
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    • v.17 no.2
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    • pp.37-49
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    • 1992
  • 80 patients who presented at Wonkwang University Dental Hospital with craniomandibular disorders were collected for this study. To observe the occlusal contact pattern such as contact numbers, contact force and presence or absence of anterior occlusal contact, the author used T-Scan system (Tekscan, Inc, U.S.A.) with are computerized occlusal analysing system. And to study the correlation between craniofacial profile and occlusal contact pattern, cephalogram were also taken, The cephalometric items related to growth pattern, jaw bone relation and denture pattern were measured and analysed according to routine method by computerized program. The obtained data were statistically processed with SPSS/PC+ package about anterior contact pattern and its craniofacial relationship. The obtained results were as follows : 1. In terms of growth pattern, patients without anterior tooth contacts showed a tendency to downward growth of craniofacial profile. The value in this subjects were significantly different from the value of patients with anterior tooth contacts in items of low gonial angle, Jarabak ratio, SN to GoMe angle, FMA, occlusal plane to mandibular plane angle and ramus height. 2. In terms of jaw bone relationship, patients without anterior tooth contacts showed a tendency to backward growth of craniofacial profile. The value of this patients were significantly different from the value of patients with anterior tooth contacts in items of SNB, ANB, mandibular plane to anterior cranial base ratio, SNPo, NAPo and APDI items. 3. But in denture pattern, no statistically significant difference by the presence or absence of anterior tooth contacts were showed between this patients groups. 4. From this study, it could be proposed that anterior open bite in the patients with craniomandibular disorders would be originated from not dental discrepancy but skeletal discrepancy.

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Short Stature and Growth Hormone Therapy (저신장 소아를 위한 성장호르몬 치료)

  • Park, Yong-Hoon
    • Journal of Yeungnam Medical Science
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    • v.22 no.1
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    • pp.1-12
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    • 2005
  • Normal growth and development is of prime concern during childhood. The treatment of children with growth hormone deficiency has been revolutionized by growth hormone therapy. An improved height outcome with a final height within the target height range has been achieved. However, close follow-up with regular clinical and laboratory monitoring is essential for achieving the desirable height outcome. The theoretical unlimited supply of growth hormone has led to its wide spread use in a variety of disorders other than a growth hormone deficiency. Initially used in children with Turner syndrome, growth hormone is now used to treat chronic renal failure, an idiopathic short stature and intrauterine growth restrictions in addition to a wide array of newly emerging indications. This review summarizes the basics for a proper growth assessment, the differentiation of normal and abnormal growth causes of a short stature, and the indications for growth hormone treatment.

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Radixin Knockdown by RNA Interference Suppresses Human Glioblastoma Cell Growth in Vitro and in Vivo

  • Qin, Jun-Jie;Wang, Jun-Mei;Du, Jiang;Zeng, Chun;Han, Wu;Li, Zhi-Dong;Xie, Jian;Li, Gui-Lin
    • Asian Pacific Journal of Cancer Prevention
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    • v.15 no.22
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    • pp.9805-9812
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    • 2014
  • Radixin, a member of the ERM (ezrin-radixin-moesin) family, plays important roles in cell motility, invasion and tumor progression. It is expressed in a variety of normal and neoplastic cells, including many types of epithelial and lymphoid examples. However, its function in glioblastomas remains elusive. Thus, in this study, radixin gene expression was first examined in the glioblastoma cells, then suppressed with a lentivirus-mediated short-hairpin RNA (shRNA) method.We found that there were high levels of radixin expression in glioblastoma U251cells. Radixin shRNA caused down-regulation of radixin gene expression and when radixin-silenced cells were implanted into nude mice, tumor growth was significantly inhibited as compared to blank control cells or nonsense shRNA cells. In addition, microvessel density in the tumors was significantly reduced. Thrombospondin-1 (TSP-1) and E-cadherin were up-regulated in radixin- suppressed glioblastoma U251 cells. In contrast, MMP9 was down-regulated. Taken together, our findings suggest that radixin is involved in GBM cell migration and invasion, and implicate TSP-1, E-cadherin and MMP9 as metastasis-inducing factors.

Renal Tubular Acidosis (신세뇨관 산증)

  • Park, Hye-Won
    • Childhood Kidney Diseases
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    • v.14 no.2
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    • pp.120-131
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    • 2010
  • Renal tubular acidosis (RTA) is a metabolic acidosis due to impaired excretion of hydrogen ion, or reabsorption of bicarbonate, or both by the kidney. These renal tubular abnormalities can occur as an inherited disease or can result from other disorders or toxins that affect the renal tubules. Disorders of bicarbonate reclamation by the proximal tubule are classified as proximal RTA, whereas disorders resulting from a primary defect in distal tubular net hydrogen secretion or from a reduced buffer trapping in the tubular lumen are called distal RTA. Hyperkalemic RTA may occur as a result of aldosterone deficiency or tubular insensitivity to its effects. The clinical classification of renal tubular acidosis has been correlated with our current physiological model of how the nephron excretes acid, and this has facilitated genetic studies that have identified mutations in several genes encoding acid and base ion transporters. Growth retardation is a consistent feature of RTA in infants. Identification and correction of acidosis are important in preventing symptoms and guide approved genetic counseling and testing.

Molecular characterization in chromosome 11p15.5 related imprinting disorders Beckwith-Wiedemann and Silver-Russell syndromes

  • Shin, Young-Lim
    • Journal of Genetic Medicine
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    • v.18 no.1
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    • pp.24-30
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    • 2021
  • Epigenetics deals with modifications in gene expression, without altering the underlying DNA sequence. Genomic imprinting is a complex epigenetic phenomenon that refers to parent-of-origin-specific gene expression. Beckwith-Wiedemann syndrome (BWS) and Silver-Russell syndrome (SRS) are congenital imprinting disorders with mirror opposite alterations at the genomic loci in 11p15.5 and opposite phenotypes. BWS and SRS are important imprinting disorders with the increase of knowledge of genetic and epigenetic mechanisms. Altered expression of the imprinted genes in 11p15.5, especially IGF2 and CDKN1C, affects fetal and postnatal growth. A wide range of imprinting defects at multiple loci, instead of a restricted locus, has been shown in some patients with either BWS or SRS. The development of new high-throughput assays will make it possible to allow accurate diagnosis, personalized therapy, and informative genetic counseling.

Clinical Problems in ML II and III: Extra-skeletal Manifestations

  • Park, Sung Won
    • Journal of mucopolysaccharidosis and rare diseases
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    • v.2 no.1
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    • pp.5-7
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    • 2016
  • Mucolipidoses II and III alpha/beta (ML II and ML III) are lysosomal disorders in which the essential mannose-6-phosphate recognition marker is not synthesized onto lysosomal hydrolases and other glycoproteins. The disorders are caused by mutations in GNPTAB, which encodes two of three subunits of the heterohexameric enzyme, N-acetylglucosamine-1-phosphotransferase ML II, recognizable at birth, often causes intrauterine growth impairment and sometimes the prenatal "Pacman" dysplasia. The main postnatal manifestations of ML II include gradual coarsening of neonatally evident craniofacial features, early cessation of statural growth and neuromotor development, dysostosis multiplex and major morbidity by hardening of soft connective tissue about the joints and in the cardiac valves. Fatal outcome occurs often before or in early childhood. ML III with clinical onset rarely detectable before three years of age, progresses slowly with gradual coarsening of the facial features, growth deficiency, dysostosis multiplex, restriction of movement in all joints before or from adolescence, painful gait impairment by prominent hip disease. Cognitive handicap remains minor or absent even in the adult, often wheelchair-bound patient with variable though significantly reduced life expectancy. As yet, there is no cure for individuals affected by these diseases. So, clinical manifestations and conservative treatment is important. This review aimed to highlight the extra-skeletal clinical problems in ML II and III.

Effects of In Vitro Vibration Loading on Myogenesis of C2C12 Myoblasts (C2C12 근아세포의 근육 형성에 대한 진동 부하 효과)

  • Yong Chan Jung;Gayoung Kim;Eunyeong Moon;Hanbyul Cho;Chi Hyun Kim
    • Journal of Biomedical Engineering Research
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    • v.45 no.4
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    • pp.195-203
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    • 2024
  • Muscle disorders arise from genetic factors or other external environmental influences, leading to muscle loss and decreased strength. Muscle disease is closely related to age, and with the global progression of aging, there is increasing interest in the prevention and treatment of muscle diseases among the elderly population. Muscles release various substances during contraction and relaxation when exercising, and growth and differentiation are enhanced. Similar effects can be achieved by vibration stimulations of specific frequencies. To examine the response of muscle cells to vibration stimuli, an in vitro vibration device capable of setting various frequencies and amplitudes was developed for use with a 6-well plate. In vitro vibrational stimulation was applied to C2C12 myoblasts to quantify the growth and differentiation of myoblasts, as well as cell apoptosis. 10 Hz vibrational stimuli resulted in increases in ERK signaling and decreases in cell death. Moreover, an increase in the number of exposures to vibration promoted cellular differentiation. In conclusion, 10 Hz vibrational stimuli have the potential to increase muscle growth and differentiation and reduce apoptosis in C2C12 myoblasts.

A Clinical Inquiry into 200 Cases of Children Coming to the Clinic Due to the Symptom of Growth Deficiency (성장장애(成長障碍)를 주소(主訴)로 내원(來院)한 환아(患兒) 200례(例)에 대(對)한 임상적(臨床的) 관찰(觀察))

  • Na, Dong-Gyu
    • Journal of Haehwa Medicine
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    • v.7 no.2
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    • pp.609-620
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    • 1999
  • Over the period between January 1997 and December 1998, herbal medicine was more than three times administered to the patients coming to Na dong gyu's Oriental Medical Clinic on account of the symptom of growth deficiency. According to radiological opinions about the patients providing cooperation for measuring their height and weight as well as their bone age every three months, it was found that the growth plates were not closed. A research was conducted for 200 children randomly selected of patients in prepuberty (they grew by less than 5cm a year before treatment at a age of 12years for female children and 14years for male children). As a result, the following conclusion was drawn: 1. The randomly selected subjects were made up of 116 male and 84 female children in terms of gender. The age direstribution was most 10 to 12 years in 86 children(34.00%), followed by 8-10 years(27.50%) and 12 to 14 years(19.50%). 2. Considering the distribution of sick children's parental height, the fathers of 141 children(70.50%) measured less than 170cm high, the subaverage height, while the mothers of 172 children(86%) measured less than 160cm high, the subaverage height. It was shown that sick childen's height was genetically influenced by their parents. 3. Children patients's weight at a time of birth was most 3.1-3.5kg for 85 children(44%) and less than 2.5kg which came under the range of growth dificiency for 19 children(9.5%). 4. The highest proportian of the children patients with growth dificiency(56.33%) had the symptom of digestive disorders, of which 77 children patients(18.78%) had anorexia, 16.59% of children patients had the high level of respiratory disorders. Both the digestive disorder and the respiratory disorder put together, they had the high rate of 72.92%. Therefore, this indicates that both the digestive disorder and the respiratory disorder have a great effect on children's growth dificiency. 10.74% of chilren patients were shown to have allergic disorders, which indicates that they also exert an effect on growth deficiency. Specifically, 7.07% of the children patients had the high level of obesity, which shows that an excessive uptake of nutrition may rather induce children to have growth dificiency though an appropriate amount of nutritional uptake is necessary. 5. Comparing their bone age and their chronological age, 58 children patients(29.00%) showed that they were the same at the highest percent. 79 children patients(39.50%) showed that thier bone age was lower than their chronological age. And 63 children patients(31.50%) showed that their bone age was higher than their chronological age. 6. As regards the prescription administered to children patients for treating their growth dificiency, Growth tang A related to the kidney, the congenital factor, of the causes for growth dificiency in traditional Oriental Medicine was administered to 108 children patients(54%), whereas the Growth tang B related to the spleen, the acquired factor, was administered to 92 children patients(46%). 7. 116 male children patients with growth dificiency had the average value of growth for one year before treatment, 4.39cm, while 84 female children patients had the average value of growth for one year before treatment, 4.24cm. A total of 200 children had the average value of growth for one year before treatment, 4.33cm. The annual average value of growth in growth curve surveyed by the Korea Pediatrics Society was 5.79cm. Compared to this value, the one year average value of growth for 108 male and female children patients taking Growth tang A was shown be 8.44cm, which indicates a greater growth by 4.12cm(95.37%) in comparison with the average value of growth before treatment, 4.32cm, and a greater growth by 2.65cm(45.77%) compared to 5.79cm, the average value of growth in growth curve surveyed by the Korea Pediatrics Society. Also, the average value of growth before treatment for 92 male and female children patients taking Growth tang B was shown to be 8.47cm, which indicates a greater increase by 4.15cm(96.06%) compared to 4.32cm, the average value of growth before treatment and a greater increase by 2.67cm(46.29%) in comparison with 5.79cm, the average value of growth in growth curve surveyed by the Korea Pediatrics Society. Considering the average value of growth for male and female children patients taking Growth tang A and B, it was shown to be 8.46cm, which indicates a greater increase by 4.14cm(95.81%) compared to 4.32cm, the average value of growth before treatment, and a greater increase by 2.67cm(46.11%) compared to 5.79cm, the average value of growth in growth curve surveyed by the Korea Pediatrics Society.

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The Effect of using Computer & Smart-phone on Decreased Work Efficiency due to Musculoskeletal Disorders ; Mediating Effect of Perceived Musculoskeletal Disorders (컴퓨터 및 스마트폰 사용이 근골격계질환으로 인한 업무능력 저하에 미치는 영향 : 근골격계 질환의 매개효과)

  • Park, Jong-Ho
    • Journal of Distribution Science
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    • v.14 no.3
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    • pp.55-62
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    • 2016
  • Purpose - Average using time of smart-phone for Korean people is 3 hours 39 minutes and most people who are using a computer at home and their workplace can be affected over force to neck and shoulder due to unstable body posture. musculoskeletal disorders which caused by unstable body posture can affect strongly to decrease work efficiency. So this research is designed to measure the effect of using computer & smart-phone on decreased work efficiency due to musculoskeletal disorders and mediating effect between decreased work efficiency and musculoskeletal disorders. Research Design, Data, and Methodology - The author has developed a questionnaire with 6 hypothesis on the basis of previous research result with 5 constructs. The questionnaires were also made by interview and E-mail. 300 copies of questionnaires were distributed and 282 questionnaire were used for the analysis as valid data responses. SPSS ver.21.0 were used and made Cronbach's α and reliability test, correlation, Baron & Kenny 3 step mediated regression analysis. Result - Cronbach's α shows 0.770~0.954 and C.R. is 0.963~0.997 which is higher than 0.7. and AVE was 0.867~0.933. So the data are all acceptable condition. Using for a long time of a computer & smart-phone has a positive effect on musculoskeletal disorders. This means, it can cause musculoskeletal disorders if people use a computer & smart-phone for a long time due to unstable body posture. And musculoskeletal disorders can effect strongly decrease work efficiency. This study also found out that a long time of using computer can cause musculoskeletal disorders rather than using smart-phone a long time. To check mediate effect of musculoskeletal disorders between using a computer & smart-phone and Decreased Work Efficiency, author used 3-step mediated regression analysis of Baron & Kenny (1986). Using a computer for a long time mediate partially and using a smart-phone for a long time mediate completely. This means that using a smart-phone a long time is not the actual reason to decrease work efficiency. But using level of smart-phone is increasing rapidly day by day. So we need to make additional research about this matter seriously. Conclusion - Nowadays, people can not live on without a computer & smart-phone even a moment. But, using a computer for a long time will affect to cause musculoskeletal disorders and it will effect strongly to decrease work efficiency. Before, we thought over that musculoskeletal disorders were diseases of elder people. But, we found out from this study that musculoskeletal disorders can be happen to any people, even children, or workers in heavy industry or engaged in brain work. So we need to be careful when we use a computer for a long time. People also need to be careful to keep correct body posture when using both a computer and smart-phone since a smart-phone became more popular and using time level became longer. Due to increased income and living standard of people, physical growth of young people is so rapid. But the physical environment of society is not suitable for them since it can not follow up the speed of growth. Suitable work table is very important to prevent musculoskeletal disorder which can affect decrease work efficiency. For a person, a society or country, increased productivity is very important since it can directly connected to the job satisfaction. Education and reeducation for the people is also important, but to teach them how to keep good condition of health will be more important since it can increase the quality of work efficiency and quality of life. Computer and Smart-phone is one the best invention of modern society, but it can cause mental and physical disease which can affect decrease work efficiency and productivity. So it is necessary to observe attentively for the situation continually.