• Journal of The Korean Society of Inherited Metabolic disease
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• v.23 no.1
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• pp.25-30
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• 2023

Leigh syndrome is a rare progressive neurodegenerative mitochondrial disorder with clinical and genetic heterogeneity. Recently, balletic IARS2 variants have been identified in a number of patients presenting broad clinical phenotypes from Leigh and West syndrome to a rare syndrome CAGSSS characterized by cataracts, growth hormone deficiency, sensory neuropathy, sensorineural hearing loss, and skeletal dysplasia syndrome (OMIM#616007). We describe a child with Korean Leigh syndrome with urologic manifestations resulting from a compound heterozygote mutation in IARS2. A 5-year-old girl visited the emergency room with a complaint of abdominal pain accompanied by abdominal distension. Abdominal-pelvic CT showed a markedly distended urinary bladder without definite obstructive lesions. She was diagnosed with neurogenic bladder dysfunction based on a urodynamic study. She had global delayed development due to neurologic regression after 6 months of age and a history of bilateral cataract surgery at the age of 2 years. Her brain magnetic resonance imaging showed symmetrically increased signal intensities in the bilateral putamen and caudate nuclei with diffuse cerebral atrophy. No gene variants were identified through whole-mitochondrial genome analysis. Whole exome sequencing was performed for diagnosis, and compound heterozygous pathogenic variants were identified in IARS2: c.2446C>T (p. Arg816Ter) and c.2450G>A (p. Arg817His). To the best of our knowledge, this is the first case report of bladder dysfunction manifestation in a patient with IARS2-related Leigh syndrome. Thus, it broadens the clinical and genetic spectrum of IARS2-associated diseases.

• Journal of dental hygiene science
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• v.1 no.1
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• pp.28-38
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• 2001

The aim of this study was to analyze the dental caries of chilren's deciduous teeth in relation to their mothers' socioeconomic factors and their oral health beliefs to increase the effect on children's oral health. One hundred seventy four children and their mothers were selected for the study. The children were 5 and 6 years old in children house in Kun-san, Korea. Research was conducted by oral examination, their mothers' socioeconomic factors and dental health belief model. The finding of analysis can be summaried as follows; In regard to the caries in deciduous teeth status, the five-year-old boy children's dft index was 3.39 and that of the girls of the same age was 2.76. The dft index of the boys of six was 3.86 and that of the girls of the same age was 3.27. The five-year-old boy children had the highest df rate(81.8%), and their dt rate was more than their ft rate. But for the six-year-old girl children, ft rate was higher than dt rate. For the five years old, the boy children's ft rate was significantly higher than that of the girls(p<0.05). Concerning the caries in deciduous teeth states by socioeconomic factors, their oral health status was significantly different according to mother employment and householder age. The df rate of the preschoolers with working mothers(84.7%) was significantly higher than that of those with nonworking mothers(66.7%)(p<0.05). The dft index of the group whose households were over forty was highest with 4.07, followed by the children of the households aged 35 to 39 with 3.83, those of the households aged 25 to 29 with 3.33, and those of the households aged 30 to 34 with 2.15. The difference between the groups was significant(p<0.05). As to the difference of maternal dental health beliefs by maternal socioeconomic factors, households educational background and whether to live with parents or parents-in-law under the same roof made a significant difference to maternal oral health beliefs. The mothers whose households were college graduates or better-educated were stronger in oral health susceptibility and seriousness(p<0.01, p<0.05). The salience was recognized more by those who lived with parents or parents-in-law than by the others who didn't(p<0.05). The dft index of the preschoolers was related to maternal dental health beliefs. That was lower when their mothers perceived dental health benefit better.

• Journal of Nutrition and Health
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• v.9 no.2
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• pp.68-86
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• 1976

The primary purpose of this study is to evaluate the correct nutritional status on pre-school children in Korea. Furthermore, it made an attempt to find and define nutrional problems, and assist in establishment on their nutritional improvement plan. For this, food intake and health condition (physical, clinical, biochemical and parasitological) survey on 109 Pre-school children in both sexes, randomly selected from Yang-Gu area in Gang-Won province and Rea-ju area in Kyong-gy Province, were conducted by means of three-day records, during the two periods of Spring and Fall season in 1975. The results obtained are summerized as follows: 1. The food intake; Average food intake of the subjects per day were $508{\sim}647g$ ($83{\sim}91%$ in vegetable foods and $5.5{\sim}11.7%$ in animal foods) in Yang-gu area, and $587{\sim}698g$ ($88{\sim}89%$ in vegetable foods and $6.3{\sim}7.6%$ in animal foods) in Rea-ju area. 2. The intake of energy and nutrients; a) Calory intake. Average energy intake of subjects per day in Yang-gu area$(1120{\sim}1415kcal)$ were all lower than the Korean Recommended Dietary Allowances (RDA) in either Spring and Fall survey, whereas the subjects in Rea-ju area were lower intake $(1213{\sim}1418kcal)$ than the RDA in the Spring but higher intake$(1516{\sim}1755kcal)$ than the RDA in the Fall, and the average intake were similar level with that of RDA. b) Protein intake. Average protein intake of the subjects per day in Yang-gu area $(33{\sim}43g)$ girl subjects in Rea-ju area $(35{\sim}39g)$ were lower than the RDA in either Spring and Fall survey, whereas the boy subjects in Rea-ju area$(36{\sim}38g)$ were lower in Spring and higher $(49{\sim}57g)$ in the Fall than that of the RDA, but the average $(43{\sim}47g)$ were similar level with the RDA. The protein intake from animal sources in all subjects were much lower $(5.5{\sim}11.7\;of\;total\;protein)$ than the RDA. c) Fat intake. Average fat intake were very lower in all subjects of both area $(14{\sim}24g\;in\;Yang-gu,\;10{\sim}12g\;in\;Rea-ju)$ than that of RDA which is recommended $12{\sim}14%$ of total energy to be supplied from fat. d) Calcium intake. Average calcium intake were very low in all subjects of both area $(264{\sim}355mg\;in\;Yang-gu\;and\;283{\sim}429mg\;in\;Rea-ju)$, especially, these in Spring were about a half level of the RDA, and it was much increased in the Fall due to increased intake of milk, but it was still not enough than the RDA. e) Vitamin A intake. Average intake of V.A ($703{\sim}1465\;IU$ in Ynag-gu and $750{\sim}1521\;IU$ in Rea-ju) were also lower than the RDA, moreover their V-A sources were mainly vegetable, so that the V-A supply might be critical one for the subjected. f) Riboflavin intake. Average riboflavin intake on all subjects in both area except boys in Rea-ju area in Fall, were very lower than the RDA. 3. The physical status; a) Average weight and height of boys aged 4 and 5 in Yang-gu area and girls of aged 5 in Rea-ju area were lower than those of Korean Standard of 1967 report, but those by age of girls in Yang-gu area and boys in Rea-ju area were a little heigher than the Korean Standard. It is, hower, present Korean standard of physical status might be somehow heigher than the 1967, since the socio-economical situation has been much improved during past a decade. So that, if one considered on this sense, the physical status of the subjects on this survey might be somehow lower than those of present Korean standard. b) Average upper arm circumference in both area were no difference each other, and their mean values of age 4, 5 and 6 in boy and girl were 15.6, 16.5, 16.4 and 15.5, 16.5, 16.4cm respectively. c) Average chest girth of boys were similar to those of Korean standard whereas the girls were smaller than the Korea standard. The average head circumference also showed similar tendency with the chest girth. 4. The clinical findings; The most popular clinical signs were angular stomatitis and dental caries, and boys had more heigher incidence then the girls. 5. The biochemical findings; a) Hemoglobin and anemia Average Hb value of boys and girls were 11.4 and 10.9g per 100 ml of blood respectively. The incidence of anemia (Hb value below 11 g/100 ml, by WHO) was increased by age, and girls had more heigher incidence than the boy (34% : 48%). The incidence of anemia in age of 4,5, and 6 in boys and girls were 28%, 41% 34%, and 33%, 50%, 49% respectively. The degree of the anemia was not severe, and the anemia of there subjects may be caused mainly low intake of better quality protein and low iron intake as well. b) Hematocrit. Average Ht value of whole subject were $39.9{\sim}41.6%$. c) Blood plasma protein. Average blood plasma protein contents of whole subjects were $6.6{\sim}7.4gm$ per 100 ml. The incidence of deficient range (<6.0g%, by ICNND) was only one girl of age 4 in yang Gu area. 6. Parasitological findgs; The most popular parasitism were asicris lumbicoides and trichocephalus trichiura, and about 2/3 of the whole subjects were suffering one or more of these parasitism.

• Research in Community and Public Health Nursing
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• v.14 no.2
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• pp.263-273
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• 2003

Objectives: The purpose of this study was to identify the family phenomenon and characteristics by ICNP such as family shape, life standard. area, and development stage. Method: 1. Sample size was 115. 2. Data analysis method included frequency analysis including ratio. Results: 1. On the distribution of the family phenomena by family shape, the nuclear family showed a higher ratio on the distribution in family communication and the unhealthy life style. The nuclear family was related to children andhad a higher ratio on the inadequate care management of the sick member. The extended family showed the highest ratio on the inadequate care management of sick member. 2. On the distribution of the family phenomena by family life standard, the family with medium life standard showed a higher ratio on the inappropriate family coping, whereas the family with lower life standard appeared to have a higher ratio on the inadequate care management of the sick member. 3. On the distribution of the family phenomena by area. the large city area showed the highest ratio on the inappropriate family coping, the medium and small city area appeared to have the highest ratio on the unhealthy life style. The county area showed the highest ratio on the inadequate care management of sick member. 4. On the distribution of the family phenomena by development stage, the family in rearing period showed a higher ratio on the inappropriate family coping and the lack of family intimacy. Families with preschool children showed a higher ratio on the unhealthy life style, and families with school age children showed the highest ratio on the inappropriate family coping. Families with adolescents appeared to have the highest ratio on the disturbance in family communication, and families with launching young adults showed the highest ratio on the inadequate care management of sick member. 5. On the distribution of the family characteristics by family shape, the nuclear family showed a higher ratio on the family characteristics such as less communication chances among family members and neglect of general child rearing, whereas the third generation family appeared to have a higher ratio on the characteristics such as overburden of housewife's role and short of caring among family members. 6. On the distribution of the family characteristics by family life standard, the family of medium life standard showed higher ratio on the family characteristics such as a few communication chance among family and overburden of housewife's role, and the family of lower life standard appeared to higher ratio on the family characteristics such as short of caring among family members. 7. On the distribution of the family characteristics by area. the large city area showed a higher ratio on the family characteristics such as overburden of housewife's role and neglect of general child rearing. The medium and small city area appeared to have a higher ratio on the family characteristics such as less communication chancec among family members. The county area showed a higher ratio on the family characteristics such as short-term care among family members. 8. On the distribution of the family characteristics by development stage, the family with rearing period showed a higher ratio on the family characteristics such as neglect of general child rearing. Families with preschool children showed a higher ratio on the family characteristics such as less communication chancec among family members. Families with school age children showed a higher ratio on the family characteristics such as overburden of housewife's role. Families with adolescents appeared to have a higher ratio on the family characteristics such as less communication chances among family members. Families with launching young adults showed a higher ratio on the family characteristics such as less communication chances and short-term care among family members.

• Women's Health Nursing
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• v.7 no.4
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• pp.518-535
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• 2001

Nursing practices should be based on the understanding of human beings. In order to understand human beings, it is important to study the lifestyles and thoughts of people in their natural environment. In this sense, the cultural aspects of a society need to be studied for a culture-bound nursing service. Child care, which is an important element of nursing, is also strongly influenced by the culture of a society. Therefore, a cultural study is necessary to understand the child-rearing practices of any society. The major purpose of this dissertation is to provide basic foundations for developing a culture-based theory for nursing intervention through studying traditional cultural elements of child care in Korean society. The study examined child-rearing practices in a small village on Koje Island in the southern part of Korea. It utilized ethnographic methodologies including participatory observations and in-depth interviews. The study participants were 9 Korean mothers living on Koje Island. The average age was 52. The data were collected between July in 1998 and December in 1999. The average number of interviews per person was 7-8, and the duration of each interview was approximately 2 hours. The data were analyzed using the Spradley Analytical Method. The following 9 major child-rearing aspects of mothers on Koje Island were discovered as a result of the study: 1. Firstly, mothers on Koje Island were mostly concerned about the "Old Birth Goddess' Curse", especially during their child's early years. This concern was evidenced by their careful behavior when their child was very young and by their praying to the Old Birth Goddess not to be jealous of their babies. 2. Secondly, they wished their children to live a different and better life than themselves. It was represented by their strong motivation toward their children's education as well as their expectation for their children's success. In traditional Korean culture, Korean people think that the rise and fall of the household depend on their offsprings. Therefore, Korean mothers wish their children attain to a higher level of social status through education. 3. Third, mothers are concerned about their children's righteousness. Mothers on Koje island expect their children to live with discretion, justice, strength, respect, harmony, and to do their best in life. 4. Next was an 'anticipation of their children's happy marriage'. The attributes of this category were an 'anxiety about their children's married life', and 'an expectation of a good spouse for their children'. Because Korean people believe that only a son can continue the bloodline of a family, especially Korean mothers have a great concern of the possibility of their daughters not having a son after marriage. Also they have different expectations toward their daughter-in-laws than son-in-laws. 5. Korean mothers also derived their satisfaction from their son. It was characterized by 'excessive affection toward their son', 'dependency on their son', and 'being afraid of their married daughter having a girl like themselves'. Korean society has been a patriarchy. Therefore, a son is beloved as someone who will take care of his old parents, be in charge of ancestral rites, and provide a daughter-in-law who can conceive a son. 6. The sixth category concerned 'the differences in their expectations for their children'. The attributes in this category were 'different expectations depending on their children's gender', 'different expectations depending on their children's ability', and a 'great sympathy toward children with low abilities'. Korean mothers expect their son to become better than their daughter. 7. The seventh category was related to their 'roles in child-caring practices'. Traditionally a child was raised in an extended family system in Korea So it was not the sole duty of a mother to bring up the child. Korean mothers used to receive much help rasing children from their in-laws, and family members. On the other hand, many children grew up by themselves, because their mothers were very busy taking care of housework. Furthermore, many children also grew up in poverty. 8. Mothers also had issues related to 'conflicts in child rearing'. They were characterized by 'lack of understanding', 'rudeness of children', and 'giving vent to one's anger'. 9. Finally, mothers regretted not doing their best in child-rearing practices. It was characterized by a 'bitter feeling of repentance', 'feeling irritated', and 'feeling of unsatisfaction'.

• Korean Journal of Environment and Ecology
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• v.28 no.5
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• pp.542-549
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• 2014

This study was conducted to investigate the body size, sexual size dimorphism (SSD), and related environmental factors between Red-tongued viper snakes (Gloydius ussuriensis) inhabiting two different places, i.e., Jeju Island and its islet Gapado, and to provide data required to maintain species diversity from May, 2006 until June, 2009. The snout-vent length of the Red-tongued viper snake population inhabiting Jeju Island was found to be 242-532 mm ($422.0{\pm}46.7mm$, n = 100) in females and 296-580 mm ($434.5{\pm}51.7mm$, n = 63) in males. In contrast, the snout-vent length was observed to be 205-395 mm ($335{\pm}43.6mm$, n = 55) in female and 215-430 mm ($328{\pm}39.4mm$, n = 73) in male Red-tongued viper snakes inhabiting Gapado. These data demonstrated the snout-vent length of both female and male Red-tongued viper snakes on Jeju Island to be larger than those on Gapado (Female t = 17.343, df = 115, P<0.001; Male = 19.128, df = 101, P<0.001). SSD was measured to be -0.03 in the Red-tongued viper snake population on Jeju Island, with more or less larger sizes in the males, while it was 0.02 in the Red-tongued viper snake population in the Gapado, with a little larger sizes in the females. The reason for this difference in the snake populations between Jeju Island and Gapado may be due to adaption to the different ecological environments. In addition, as SSD, the snout-vent length of the Red-tongued viper snake populations and in young vipers was somewhat higher in the males than in the females on Jeju Island (t = -2.011, df = 117, P<0.05). However, no significant differences were observed in the snout-vent length of the young and the general Red-tongued viper snake populations on Gapa Island. For the population on Jeju island, the head length (F = 6.318, $df_{1,2}$=1,117, P<0.05), head width (F=8.090, $df_{1,2}$=1,117, P<0.01), inter eye length (F=15.898, $df_{1,2}$=1,117, P<0.001), and tail length (F=238.488, $df_{1,2}$=1,111, P<0.001) were all larger in the males, while females showed higher body mass (F=64.111, $df_{1,2}$=1,114, P<0.001). In the case of the Gapa Island population, no significant differences in the head length, head width, and inter eye length between females and males were observed, while the males had a longer tail length (F=168.555, $df_{1,2}$=1,74, P<0.001) and the females were heavier (F=17.812, $df_{1,2}$=1,76, P<0.001). Though no significant differences were found in the head length, head width, and inter eye length, the tail length (F=67.793, $df_{1,2}$=1,72, P<0.001) and body mass (F=4.558, $df_{1,2}$=1,72, P<0.05) were higher in the young male Red-tongued viper snakes than in the females. The snout-vent length, head length, head width, and inter eye length, which did not display SSD in the young Red-tongued viper snake populations, were higher in the male Red-tongued viper snake populations than in the female population from Jeju Island, implying that SSD in the Red-tongued viper snake population on Jeju Island is expressed due to environmental effects during their growth.