• Korean Journal of Breeding Science
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• v.41 no.4
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• pp.520-524
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• 2009

A newly developed rice variety "Pungmi 1" is a japonica rice(Oryza sativa L.) with high grain quality and multi-resistant to diseases. It was developed by the rice breeding team of Yeongnam Agricultural Research Institute (YARI), RDA. in 2004. This variety derived from a cross between "YR13616Acp 1", having short culm and multi-resistance to biotic stresses and "Milyang 122" with good grain quality. It has short stature of 73cm in culm length and mid-early flowering date of Aug. 13. This variety is moderately resistant to leaf blast showing durable resistance of lower 10% diseased leaf araea in sequential planting meothod. Milled rice kernel of "Pungmi 1" is translucent, clear in chalkness and good at eating quality in panel test. Milling recovery and head rice ratios were comparable to Milyang 122, while it has low protein content. The milled rice yield potential of "Pungmi 1" is about 5.59 MT/ha at ordinary fertilizer level of local adaptability test. This cultivar would be adaptable to Yeongnam inland plains and southern coastal areas of Yeongnam province at ordinary transplanting as well as after barly cultivation.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.6 no.2
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• pp.94-98
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• 2010

Axenfeld-Rieger syndrome is a rare autosomal dominant disorder characterized by various ocular and extraocular malformations. The incidence of Axenfeld-Rieger syndrome has been estimated to be 1 per 200,000. The syndrome is characterized by short body stature, delayed bone age, and deficient or arrested development of neural crest cells involving the anterior chamber of the eyes, facial bones, teeth, periumbilical skin, and cardiovascular system. This is a case of a 6 year-old girl, who came to the department of the pediatric dentistry, Yonsei University Dental Hospital, for evaluation and treatment of multiple congenital missing permanent teeth. The patient presented typical dental, craniofacial, and systemic features of Axenfeld-Rieger syndrome, such as glaucoma, oval pupil, heterochromatic iris, umbilical hernia, and delayed bone age. On the panoramic view, 3 primary teeth were missing and 13 permanent tooth germs were absent. On the lateral cephalograph, underdevelopment of the maxilla and normal growth pattern of the mandible were confirmed. Periodic dental follow-up is planned for evaluation and interceptive treatment of her dental and craniofacial problems. Denture or removable space maintainer with a pontic is considered for esthetic and functional restoration. In addition, orthognathic surgery is also planned in future to prevent further midfacial skeletal deformation. Early diagnosis of Axenfeld-Rieger syndrome is very important to prevent exacerbation of complications, such as glaucoma and skeletal deformities.

• Journal of Genetic Medicine
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• v.16 no.2
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• pp.55-61
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• 2019

Purpose: Genetic defects in the nuclear-encoded mitochondrial aminoacyl-tRNA synthetases were first identified as causes of various disorders in 2007. Variants in IARS2, which encodes a mitochondrial isoleucyl-tRNA synthetase, were first reported in 2014. These variants are associated with diverse phenotypes ranging from CAGSSS (CAtaracts, Growth hormone deficiency, Sensory neuropathy, Sensorineural hearing loss, and Skeletal dysplasia) and Leigh syndrome to isolated nonsyndromic cataracts. Here, we describe the phenotypic and genetic spectrum of Korean patients with IARS2-related disorders. Materials and Methods: Using whole-exome sequencing followed by Sanger sequencing, we identified five patients with IARS2 mutations. Their medical records and brain magnetic resonance images were reviewed retrospectively. Results: All five patients presented with developmental delay or regression before 18 months of age. Three patients had bilateral cataracts, but none had hearing loss or sensory neuropathy. No evidence of skeletal dysplasia was noted, but two had short stature. One patient had cardiomyopathy and another exhibited renal tubulopathy and hypoparathyroidism. Their brain imaging findings were consistent with Leigh syndrome. Interestingly, we found the recurrent mutations p.R817H and p.V105Dfs*7 in IARS2. Conclusion: To our knowledge, this is the first report of Korean patients with IARS2-related disorders. Our findings broaden the phenotypic and genotypic spectrum of IARS2-related disorders in Korea and will help to increase clinical awareness of IARS2-related neurodegenerative diseases.

• Journal of the korean academy of Pediatric Dentistry
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• v.45 no.1
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• pp.115-122
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• 2018

Noonan syndrome is characterized by distinctive facial features, short stature, and congenital heart disease. It is a congenital genetic disorder with a prevalence of between 1/1,000 and 1/2,500 in both genders. An 11-year-old boy with Noonan syndrome visited the hospital with an ectopically positioned tooth. A pulmonary stenosis was diagnosed and his growth and development were delayed. In many cases of this diseases there is obvious hemostasis, which he was not experiencing. His facial appearance showed characteristic features of Noonan syndrome. The patient showed a dental class II relationship, labioversion of the upper anterior teeth, and a shallow overbite. Radiographic examination revealed that the upper right canine was ectopically positioned, which led to root resorption of the upper right lateral incisor. A lateral cephalometric radiograph revealed a craniofacial pattern that was within normal limits. Surgical opening and button attachment on the impacted upper right canine were performed and traction was applied on the impacted tooth using a removable appliance. This patient was mildly affected by Noonan syndrome and showed some dental problems. However, few studies have reported the oral characteristics of Noonan syndrome despite its high incidence. Thus, this case report describes the oral features and management of Noonan syndrome.

• Journal of Preventive Medicine and Public Health
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• v.7 no.1
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• pp.131-138
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• 1974

The author has conducted survey on the status of physical growth and morbidity of the children for christian children's fund programme, as a means of collecting basic data for the anticipated establishment of a health planning. A total 345 children aged 9 to 16 underwent C.C.F. programme while as a control, a total of 480 children of same ages from the middle-class school children in Jeonju area was also studied. As results of survey conducted for a period of one month (form July 1 to 31, 1974) on a total 429 children in 347 households living in Jeonju area. I. Socio-economic background 1. By educational status of the children, 39.9 per cent of the total children was attending at primary school, 33.8 per cent in middle school and 15.6 per cent in high school. 2. The greatest proportion or 28.8 per cent of the household head were engaged in labor, 17.9 per cent in peddler and 13.2 per cent in retail. 3. As for the living standard of the households, low class constitued 90.1 per cent, middle and high classes only 9.9 per cent. 4. 39.5 per cent of the households had their own house, 39.1 per cent lived in rent deposit house or rooms and 14.6 per cent in monthly rented house and rooms. II. Physical growth and nutritional status 1. The growth of children for C.C.F. programme in terms of height was found to be slightly smaller than the school children. The ages frm 9 to 16 corespond to the 'secondary growth and replenishment period and this period was regarded to be the one most affected by environmental and nutritional factors of all the other periods of growth and developmet. 2. The body weight of the children for C.C.F. presented a quite different pattern from that of the school children. The above findings appeared thin-and-long stature from the famillies with higher living standard while those from the household with low standard of living had a short-and-plump one. 3. According to the values of Rohrer's index, the children of C.C.F carried a higher degree of 'replenishment' than the children in Jeonju area and adolesecence comes later for the girls under C.C.F. programme. III. Morbidity 1. The monthly prevalence rate was 110.0 per thousand persons for the children under C.C.F. programme. 2. The total number of case was classified by timing of the incidence as follws. 40.0 per cent was constituted by diseases carried over from tile previous month and 60.0 per cent by new incidences. 3. The diseases were broken down by W.H.O. disease classification into the greatest proportion or 39.1 per thousand person constituted by disease of the digestive system.

• Journal of the korean academy of Pediatric Dentistry
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• v.36 no.2
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• pp.298-304
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• 2009

XLH (X-linked hypophosphatemic rickets) is a form of rickets which is resistant to the usual dose of vitamin D and inherited in a X-linked dominant manner. It is also known as vitamin D-resistant rickets or familial hypophosphatemic rickets. Here we report a 6-year-and-6-month-old female patient of XLH who is diagnosed with in SNUB. She was referred from local clinic for impaction of maxillary left permanent incisor and its treatment. She presents bowing deformities of the legs, short stature, enlargement of wrist and ankles and spontaneous dental abscesses of clinically sound teeth delayed eruption, taurodontism, delayed apical closure, enlarged pulp chambers, and absent or poorly defined lamina dura. The purpose of this case is to review the literatures of XLH and report the dental and medical characteristics of this patient.

• Journal of Physiology & Pathology in Korean Medicine
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• v.18 no.6
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• pp.1762-1768
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• 2004

The Korean formula medicine, Gami-Yukmi-Jihwang-Tang (GYJT) has been used for growing slowly, short of stature, incomplete development, fatigue, weak child, growing pain of child. However, it is still unclear how GYJT has an effect on experimental models. In the present study, the author investigated the immune-enhancing effect of GYJT. Forced swimming test (FST) was performed as a model of activity test in mice and measured blood urea nitrogen (BUN), aspartate aminotransferase (AST), alanine aminotransferase (ALT), lactic dehydrogenase (LDH), glucose (Glc) and total protein (TP) in the serum. GYJT (1, 0.1 and 0.01 g/㎏) were orally administered to mice, once per day for 7 days using a feeding atraumatic needle. After 3 days, on FST, the immobility time was significantly decreased in the GYJT (0.01g/㎏/day)-fed group (120.75±5.71s) in comparison with the saline-fed group (153.80±10.74s). After 7 days, the immobility time was significantly decreased in the GYJT (0.1 and 0.01g/㎏/day)-fed group (125.67±5.36s and 107.67±3.71s) in comparison with the saline-fed group (167.67±12.99s). In addition, the contents of BUN and Glc in the blood serum were significantly decreased and the contents of AST, ALT and LDH were also decreased in the GYJT (1g/㎏/day)-fed group. However, the content of TP was not changed. The present results suggest that GYJT may be useful for the anti-fatigue and immune-enhancing agent. Also, the author investigated the effect of GYJT on the production of cytokines in human T-cell line, MOLT-4 cells. However, GYJT has not affected the production of IFN­γ, IL-2, IL-4. These results suggest that GYJT has immune-enhancing effect but does not affect T cell-mediated production of cytokines in the immune function improvement.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.4 no.2
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• pp.82-87
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• 2008

Cleidocranial dysplasia (CCD) is an autosomal dominant skeletal dysplasia and is caused by mutation in the CBAFA1 gene of 6p21 chromosome band. Patients with CCD express skeletal dysplasia such as hypoplastic/aplastic clavicle, brachycephalic skull, midface hypoplasia and moderate short stature. In addition to skeletal dysplasia, specific symptoms may appear in respiratory organs, auditory area, and the more distinguished, dentition. Dental findings include: delayed eruption of permanent tooth, multiple supernumerary tooth more than five, malocclusion, etc. In Patients presenting excessive SNT, complications of SNT could be prevented and will be managed through pertinently timed treatment such as tooth extraction, using space maintainer, and orthodontic management after early diagnosis. This case is about the treatment of eruption disorders in permanent teeth owing to SNT in CCD patients, who are three family members in the $3^{rd}$ generation inherited from maternal grandfather through atavism. We performed the extraction of numerous SNT and orthodontic treatment on them in this case. On evaluating panoramic and cephalometric views, some classical signs of skeletal dysplasia due to CCD were recognized in a pool of three patients, the clavicle was distinctively displayed in all patients.

• 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.

• Clinical and Experimental Pediatrics
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• v.51 no.3
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• pp.248-255
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• 2008

Purpose : The purpose of this study was to evaluate the attitudes of middle-school students about height and their behaviors related to height gain. Methods : One thousand four hundred twenty two middle-school students were included. We requested that the students complete a battery of questionnaires asking about problems associated with height. Results : There was a clear difference between real height and desired height. Most students (83.7%) had height dissatisfaction. Most students (91.1%) were concerned about their height. Few students made efforts to improve their height, even though most students had height dissatisfaction and were concerned about their height. Herbal medicine had a tendency to become popularized in treatments for height. Students generally did not judge their friends by height, but there was a small tendency to ignore shorter friends and to feel a sense of inferiority around taller friends. Most students had a greater desire to have a beautiful face than a good body, especially to be tall. Most students thought that there would be some handicaps in life if they had a short stature. Conclusion : The attitudes and concerns about height in adolescence should be understood, and recognized as one aspect of treatment for problems associated with height.