• 제목/요약/키워드: one-year-old infants

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An ANKRD11 exonic deletion accompanied by a congenital megacolon in an infant with KBG syndrome

  • Seo, Go Hun;Oh, Arum;Kang, Minji;Kim, Eun Na;Jang, Ja-Hyun;Kim, Dae Yeon;Kim, Kyung Mo;Yoo, Han-Wook;Lee, Beom Hee
    • Journal of Genetic Medicine
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    • v.16 no.1
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    • pp.39-42
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    • 2019
  • KBG syndrome is an autosomal dominant syndrome presenting with macrodontia, distinctive facial features, skeletal anomalies, and neurological problems caused by mutations in the ankyrin repeat domain 11 (ANKRD11) gene. The diagnosis of KBG is difficult in very young infants as the characteristic macrodontia and typical facial features are not obvious. The youngest patient diagnosed to date was almost one year of age. We here describe a 2-month-old Korean boy with distinctive craniofacial features but without any evidence of macrodontia due to his very early age. He also had a congenital megacolon without ganglion cells in the rectum. A de novo deletion of exons 5-9 of the ANKRD11 gene was identified in this patient by exome sequencing and real-time genomic polymerase chain reaction. As ANKRD11 is involved in the development of myenteric plexus, a bowel movement disorder including a congenital megacolon is not surprising in a patient with KBG syndrome and has possibly been overlooked in past cases.

Current Status of Augmented Reality Picture Books and Preschooler's Immersion (증강현실 그림책 현황과 유아의 몰입도)

  • Han, You Me;Won, Soon Ok
    • Journal of Information Technology Applications and Management
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    • v.29 no.1
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    • pp.47-57
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    • 2022
  • The purpose of this study is to analyze the current status of augmented reality picture books, which have been steadily developed since 2010, as a genre of electronic picture books, and to reveal how children's immersion in augmented reality picture books differs from paper picture books. To this end, 30 augmented reality picture books on the market were analyzed according to genre, life theme, implementation method, and augmented reality scene ratio. As a result of the study, it was found that the genre of picture books was in the order of information fairy tales, daily fairy tales, and historical fairy tales, and there were no traditional or fantasy fairy tales. Animals and plants accounted for about half of the life topics, and in some cases, there were only a few or no other life topics. In the augmented reality implementation scene, it consisted of only one page in the early days, but all pages are now possible to implement augmented reality due to technology development, production cost reduction, and improved hardware performance of smartphones. It was found that the augmented reality implementation method used CD-ROM in the early days, but gradually became possible to implement using only mobile phones and tablets that were easy for readers to access. In addition, after presenting four picture books to eight 5-year-old infants, the immersion time was measured and the immersion behavior was observed. As a result, augmented reality picture books showed higher immersion[immersion time, immersion behavior] than paper picture books, but compared by literature genre, life fairy tales were higher in paper picture books and natural fairy tales in augmented reality picture books. It was higher when presenting augmented reality picture books after presenting paper picture books according to the order of presentation of picture book types. The results of this study suggest that more diverse life topics and augmented reality picture books in the genre of children's literature should be developed to increase the utilization of augmented reality picture books. In addition, considering that there are differences in immersion between types, literary genres, and reading experience [presentation order], it is expected to increase the educational effect by using picture books complementarily.

The actual type of domestic safety accident of children and the safety knowledge according to variables of parents (가정내 유아의 안전사고 유형과 부모의 변인에 따른 안전지식)

  • Kim, Ji-Young;Kim, Na-Rim
    • Journal of the Korea Academia-Industrial cooperation Society
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    • v.18 no.10
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    • pp.454-461
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    • 2017
  • The purpose of this study is to investigate the types of domestic safety accidents involving children and to verify the difference of safety knowledge according to variables of parents. Subjects include 226 parents who have 3-5 year old infants enrolled in kindergartens and day care centers in I-city, Jeollabuk-do. The study tools are modified and complemented questionnaire for the study purpose referred to the preceding research data and the literature. For the processing of collected data, frequency analysis, descriptive statistics, one-way ANOVA, and the Duncan post-test were utilized using the SPSS program. The results of this study are as follows: First, safety accidents occurred at the majority of homes which have infant children. On average, the occurrence frequency was 1.78 times per six months. The accidents involving infants occurred in the order of drop or slip, crash or impact, stricture or jamming, fall, stab or cut, burn, swallow or inhalation. Second, both parents had an average level of safety knowledge. There was a distinction in the areas of 'fall' and 'first aid' according the mother's age. In addition, stay-at-home mothers had the higher level of safety knowledge compared with working mothers in the areas of 'general safety knowledge' and 'fire'. There was a distinction in the area of 'fire' according to the father's age. Given these findings, safety training programs for parents to improve safety knowledge needs to be developed.

A Comparative Study on the Perception of the Impediment Factors between Mothers and Nurses in Nursery (모유수유 저해요인에 대한 산모와 신생아실 간호사간의 인식정도 비교 연구)

  • Lee, Mi-Ja
    • Women's Health Nursing
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    • v.1 no.1
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    • pp.138-152
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    • 1995
  • The condition of nutrition during infancy will greatly affect infants' physical, emotional growth, especially breast feeding is important in their growth and development, and emotional stability, too. Despite such advantages of breast-feeding, its rate has continued to fall year after year in Korea : the 95% rate in 1960's has fallen to 25.4% in 1990. It is known that such a downfall of breast-feeding rate is associated with various factors. The purpose of the study is to examine mother's and nurses' in nursery perception of the impediment factors of breast-feeding : to compare those between of breast-feeding : to compare those between two groups : to provide fundamental data for developing strategies for increasing breast-feeding. The subjects were 45 new mothers from one hospital and the same number of nurses sampled from 3 university hospitals, in Seoul. The data were collected for 11 days from April 12 to April 23, 1994 and a questionnaire was developed based on the interview with 14 nurses and 10 mothers and the literature reviews. Liker's Five-Point Scale was used as measurement. The Data were analyzed using SPSS / PC and descriptive statistics, t-test, ANOVA. The results of study are as follows : 1) Most new mothers get the information about breast-feeding from their mothers, books or newspaper, and relatives in rank than professionals(nurses or doctors). 2) The impediment factors the breast-feeding are categorized as mothers, hospital system, medical personnels and social factors. The most frequently mentioned impediment factors from mothers are 'Difficulty by operational wounds'(3.13), 'Lack of will for breast-feeding'(3.47), 'Insufficient rest and sleep'(3.52) and 'Opposition from husband or his family members'(4.77)in rank. On the other hand those factors from nurses are 'Inadequate nipple condition'(2.37), 'Decreasing milk secretion given medicine after operation'(2.63), 'Mothers knowledge deficit by poorly prepared education'(2.79) and 'Mothers abhorance of breast-feeing'(3.87) in rank. 3) As for the hospital system, the highly perceived impediment factors by mothers are 'Seperation of baby from mother after birth'(2.78), 'Lack of space for breast-feeding in the hospital'(2.93), 'Lack of facility for informing the time for breast-feeding'(3.18) and 'Because of babys' preference artificial nipple by being accustomed to it in hospital(3.97), in rank. Meanwhile, those from nurses are "Seperation of baby from mother after birth",(1.92), "Inconsistency between hospital's nursing time and mother's breast secretion time" scretion time(2.97), "Lack of space for breast-feeding in the hospital"(3.39), and "Lack of facility for informing the time for breast-feeding"(3.74) 4) As for the medical personnels, the highestly perceived Impediment factor from mothers in "Lack of professional nurses for breast-feeding"(2.96), and the lowestly perceived one is "Doctors' reluctance"(4.75). Nurses perceived same as mothers, too. 5) As for the social factors, the highestly perceived impediment factor by mothers and nurses is "Inconvience of social activities"(2.83) and the lowestly ones are "The sense of self-sacrifice"(4.22) by mothers, and "The sense of old fashioned"(4.13) by nurses. 6) The difference of the perception of impediment factors between mothers and nurses is statistically significant only in mother factor.

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A Study for Developing Music Therapy Activity Program for Development of Rudimentary Movement Phase of Spastic Cerebral Palsied Infant : Applying the techniques of Neurological Music Therapy (경직형 뇌성마비 유아의 초보운동단계 발달을 위한 음악치료활동 프로그램 개발 - 신경학적 음악치료의 기법을 활용하여)

  • Lee, Yoon Jin
    • Journal of Music and Human Behavior
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    • v.4 no.2
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    • pp.84-105
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    • 2007
  • Cerebral palsy is a collection of motor disorders resulting from damage to the central nervous system that arise in multiple handicaps including cognitive disorders, speech disorders, epilepsy, perception disorders, and emotion disorders. Today spastic cerebral palsy has become more prevalent because intensive care for newborns has resulted in higher survival rates for very small premature babies. Since the children grow the fastest in order for a development during one year after birth, the therapeutic intervention is provided as early as possible to the children with cerebral palsy. After seven year old, there is no effect of intervention. So, the necessity of early intervention to spastic cerebral palsied infants is increasing. The purpose of this study is to develop the music therapy activity program using the techniques of neurological music therapy(NMT), the therapeutic application of music to dysfunctions due to neurologic disease of the human nervous system, for rudimentary movement phase of spastic cerebral palsied infant. This music therapy activity program was developed on the basis of the major developmental tasks of the rudimentary movement phase, the period that children can acquire the most basic movement function at the 0 to 2. Then the developmental characteristics of spastic cerebral palsy were applied to this music therapy activity program. This music therapy activity program was classified to three domains, those are stability, locomotion, and manipulation. This study has been consisted of three steps, those are the development of the activities, the evaluation of the activities by th panels, and the adjustment and complement of the activities. Reviewing literatures and interviews were done for the development of the activities, and the evaluation the activities was done by seven music therapists. In the evaluation steps, the questionnaire was used for estimating the content validity and application efficiency. The adjustment and complement of the activities were evaluated by the panels who were participating in the music therapy for cerebral palsied children in the clinical setting, and the results of the adjustment and complement were confirmed by the panels. The evaluation was presented in a mean value with the comment of the panels. In conclusion, the music therapy activity program for the spastic cerebral palsied infants using the techniques of NMT was developed on the basis of the major developmental tasks of the rudimentary movement phase. The program is comprised of 38 activities, those are 14 activities for developing the stability, 10 activities for developing the locomotion, and 14 activities for developing the manipulation. The programed activities would bring out the answers in the affirmative for the conformance with infants' development phase, the harmony between the objective and the activity, the conformance with the cerebral palsied infants, the properness of the music and the instruments, and the utility in the clinic field. This results mean that this developed music activity program is appropriate to help spastic cerebral palsied infants progress their movement development by stages.

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Problems in the field of maternal and child health care and its improvement in rural Korea (우리나라 농촌(農村)의 모자보건(母子保健)의 문제점(問題點)과 개선방안(改善方案))

  • Lee, Sung-Kwan
    • Journal of agricultural medicine and community health
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    • v.1 no.1
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    • pp.29-36
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    • 1976
  • Introduction Recently, changes in the patterns and concepts of maternity care, in both developing and developed countries have been accelerating. An outstanding development in this field is the number of deliveries taking place in hospitals or maternity centers. In Korea, however, more than 90% of deliveries are carried out at home with the help of untrained relatives or even without helpers. It is estimated that less than 10% of deliveries are assisted by professional persons such as a physician or a midwife. Taking into account the shortage of professional person i11 rural Korea, it is difficult to expect widespread prenatal, postnatal, and delivery care by professional persons in the near future, It is unrealistic, therefore, to expect rapid development of MCH care by professional persons in rural Korea due to economic and sociological reasons. Given these conditions. it is reasonable that an educated village women could used as a "maternity aid", serving simple and technically easy roles in the MCH field, if we could give such a women incentive to do so. The midwife and physician are assigned difficult problems in the MCH field which could not be solved by the village worker. However, with the application of the village worker system, we could expect to improve maternal and child hoalth through the replacement of untrained relatives as birth attendants with educated and trained maternity aides. We hope that this system will be a way of improving MCH care, which is only one part of the general health services offered at the local health centre level. Problems of MCH in rural Korea The field of MCH is not only the weakest point in the medical field in our country hut it has also dropped behind other developing countries. Regarding the knowledge about pregnancy and delivery, a large proportion of our respondents reported having only a little knowledge, while 29% reported that they had "sufficient" knowledge. The average number of pregnancies among women residing in rural areas was 4.3 while the rate of women with 5 or more pregnancies among general women and women who terminated childbearing were 43 and 80% respectively. The rate of unwanted pregnancy among general women was 19.7%. The total rate for complications during pregnancy was 15.4%, toxemia being the major complication. The rate of pregnant women with chronic disease was 7%. Regarding the interval of pregnancy, the rates of pregnancy within 12 months and within 36 months after last delivery were 9 and 49% respectively. Induced abortion has been increasing in rural areas, being as high as 30-50% in some locations. The maternal death rate was shown 10 times higher than in developed countries (35/10,000 live births). Prenatal care Most women had no consultation with a physician during the prenatal period. Of those women who did have prenatal care, the majority (63%) received such care only 1 or 2 times throughout the entire period of pregnancy. Also, in 80% of these women the first visit Game after 4 months of gestation. Delivery conditions This field is lagging behind other public health problems in our country. Namely, more than 95% of the women deliveried their baby at home, and delivery attendance by a professional person occurred only 11% of the time. Attendance rate by laymen was 78% while those receiving no care at all was 16%. For instruments used to cut the umbilical corn, sterilized scissors were used by 19%, non-sterilized scissors by 63% and 16% used sickles. Regarding delivery sheets, the rate of use of clean sheets was only 10%, unclean sheets, vinyl and papers 72%, and without sheets, 18%. The main reason for not using a hospital as a place of delivery was that the women felt they did not need it as they had previously experience easy deliveries outside hospitals. Difficult delivery composed about 5% of the total. Child health The main food for infants (95%) was breast milk. Regarding weaning time, the rates within one year, up to one and half, two, three and more than three years were 28,43,60,81 and 91% respectively, and even after the next pregnancy still continued lactation. The vaccination of children is the only service for child health in rural Korea. As shown in the Table, the rates of all kinds of vaccination were very low and insufficient. Infant death rate was 42 per 1,000 live births. Most of the deaths were caused by preventable diseases. Death of infants within the neonatal period was 83% meaning that deaths from communicable diseases decreased remarkably after that time. Infant deaths which occurred without medical care was 52%. Methods of improvement in the MCH field 1. Through the activities of village health workers (VHW) to detect pregnant women by home visiting and. after registration. visiting once a month to observe any abnormalities in pregnant women. If they find warning signs of abnormalities. they refer them to the public health nurse or midwife. Sterilized delivery kits were distributed to the expected mother 2 weeks prior to expected date of delivery by the VHW. If a delivery was expected to be difficult, then the VHW took the mother to a physician or call a physician to help after birth, the VHW visits the mother and baby to confirm health and to recommend the baby be given proper vaccination. 2. Through the midwife or public health nurse (aid nurse) Examination of pregnant women who are referred by the VHW to confirm abnormalities and to treat them. If the midwife or aid nurse could not solve the problems, they refer the pregnant women to the OB-GY specialist. The midwife and PHN will attend in the cases of normal deliveries and they help in the birth. The PHN will conduct vaccination for all infants and children under 5, years old. 3. The Physician will help only in those cases referred to him by the PHN or VHW. However, the physician should examine all pregnant women at least three times during their pregnancy. First, the physician will identify the pregnancy and conduct general physical examination to confirm any chronic disease that might disturb the continuity of the pregnancy. Second, if the pregnant woman shows any abnormalities the physician must examine and treat. Third, at 9 or 10 months of gestation (after sitting of the baby) the physician should examine the position of the fetus and measure the pelvis to recommend institutional delivery of those who are expected to have a difficult delivery. And of course. the medical care of both the mother and the infants are responsible of the physician. Overall, large areas of the field of MCH would be served by the VHW, PHN, or midwife so the physician is needed only as a parttime worker.

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Pilot Research about Influential Factors and Efficacy Judgement of Acupoint Sticking in Dog-Days (삼복첩(三伏貼)의 영향 요인 및 임상 효과 판정에 관한 예비 연구)

  • Lee, Sun-Haeng;Kim, Cho-Young;Chang, Gyu-Tae
    • The Journal of Pediatrics of Korean Medicine
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    • v.25 no.1
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    • pp.72-81
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    • 2011
  • Backgrounds: Sticking warm and hot herbs to acupoint in Dog-Days where the strongest Yangqi takes place, Acupoint sticking in Dog-Days is one of Oriental medical treatment to cure winter diseases in the summer. This treatment is widespread in China and Taiwan, and is becoming popular in South Korea nowadays. Objectives: The research has been conducted to evaluate the influencial factors and efficacy in Acupoint sticking in Dog-Days. Methods: We visited one kindergarten in Dog-Days of 2010, and attached pills which are made of Sinapis Semen(白芥子), Corydalis Tuber(玄胡索), Asari Herba Cum Radix(細辛), Juice of Zingiber Officinale(薑汁) to BL-13(肺兪), BL-15(心兪), BL-17(膈兪) of 56 children for 4-6 hours(34 boys, 22 girls, $52.64{\pm}16.85$ months, 19-78 months). Exacerbating effect or non-significant changes in respiratory symptoms before and after the treatment were ified.. The influentialfactors of efficacy were analyzed by Pearson's $x^2$-test and Fisher's exact test. In addition, the efficacy and satisfaction among once, twice, and three times treatment groups were verified by ANOVA. Results: 27 children (48.2%) were effective, but efficacy felt by parents is 45 cases (80.4%). Sex, perspiration, stool, urine, sleep, cold of hands and feet, cold sensitivity, and frequency of Acupoint sticking in Dog-Days has no relation to efficacy(p>0.05). However, the efficacy is poor in 1 and 2 age(p=0.030). Difference among efficacy of once, twice, and three times treatment groups is not significant(p=0.069), whereas there are linear association in frequency of Acupoint sticking in Dog-Days and efficacy(p=0.030). 36 parents (64.3%) are satisfied with Acupoint sticking in Dog-Days, and we observed significant difference among once, twice, three times treatment groups(p=0.041). 19 parents (33.9%) had been known about Acupoint sticking in Dog-Days, but 53 parents (94.6%) wanted repetition, and 41 parents (73.2%) intended to recommend it to acquaintance. Side effects are minor itching in two cases. Twenty seven participants showed positive effect, and forty five people (80.4%) felt beneficial. Sex, perspiration, stool, urine, sleep, cold hands and feet, sensitivity in cold, and frequency of Acupoint sticking in Dog-Days has no relation to the efficacy(p>0.05). Though the efficacy was poor in one and two year old infants, it was linearly correlated with the frequency count and responses from the treatment (p=0.030). Thirty six participants (64.3%) satisfied with the treatment, and so did the first, second, and third participant groups (p=0.041). Even though, only nineteen participants (33.9%) were familiar with Acupoint sticking in Dog-Days, fifty three participants (94.6%) preferred to repeat the treatment, and 41 parents were preferable to recommend the treatment to others. Side effects of minor itching has been reported in a couple cases. Conclusions: Acupoint sticking in Dog-Days is effective in children older than three year old. The effect would be greater when going through the treatment three times. Based on significant satisfaction, intention of repetition and recommendation, strong promotion on Dog-Days are needed to make a better use of Acupoint sticking in Dog-Days for the effective treatment.

One-year evaluation of the national health screening program for infants and children in Korea (영유아 건강검진 시행 초기 1년의 결과 분석)

  • Moon, Jin Soo;Lee, Soon Young;Eun, Baik-Lin;Kim, Seong Woo;Kim, Young Key;Shin, Son Moon;Lee, Hye Kyoung;Chung, Hee Jung
    • Clinical and Experimental Pediatrics
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    • v.53 no.3
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    • pp.307-313
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    • 2010
  • Purpose : Results of the Korea National Health Screening Program for Infants and Children, which was launched in November 2007, were evaluated for future research and policy development. Methods : Data from a total of 2,729,340 cases were analyzed. Five visiting ages, such as 4, 9, 18, 30, and 60 months, were included. Several parameters such as stunting, obesity, and positive rate of developmental screening were also analyzed. Telephone survey was performed in 1,035 users. For the provider survey, 262 doctors participated in our study. Results : The overall participation rate of users was 35.3%. This participation rate showed a decrement tendency to old age and low income. Only 6.9% of users participated in oral screening. Health screening was performed mainly in private clinics (82.6%). The recall rate of 4 months program users at the age of 9 months was 57.3%. The positive rate of screening was 3.1%, and was higher in the low-income group. By telephone survey, users reported that questionnaires were not difficult (94%) and overall satisfaction was good (73%). Longer duration of counseling was related with more satisfied users. Counseling and health education were helpful to users (73.2%). Doctors agreed that this program was helpful to children (98.5%). Conclusion : Korea National Health Screening Program for Infants and Children was launched successfully. Participation rate should be improved, and a quality control program needs to be developed. More intensive support following this program for children of low-income families may lead to effective interventions in controlling health inequality. Periodic update of guidelines is also needed.

A Baseline Survey on Development of Primary Health Care in the Rural Korea -Sanpuk Village, Kumsa-Myun, Yuju- Gun, Kyunggi-Do- (농촌지역의 일차보건사업 개발을 위한 기초조사 연구 - 경기도 여주군 금사면 산북부락을 중심으로 -)

  • Kim, Myung-Ho;Yun, Suk-Woo;Rhee, Hae-Soak
    • Journal of agricultural medicine and community health
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    • v.12 no.1
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    • pp.5-27
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    • 1987
  • It is widely recognized that primary health care in the community is one of the most important and effective health measures in these days. However, it is reality that unsatisfactory health care system, ineffective utilization of health care by the community people in the rural area are hampering better understanding for primary health care. Therefore promoting health for the rural people and increasing understanding about primary health care, the baseline survey in the community focused in examination for safe community water supply was carried out. The survey was conducted through August 25-31, 1986 in order to find out health problems and relevant factors and to define the demographic characteristics of $^*$Sanpuk village, Kumsa-Myun, Yuju-Gun, Kyunggi-Do, Korea. Household survey was carried out for every home by trained interviewers. The major results are found out as follows : 1) 84.2%(400 houses) of total households were surveyed because 15.8%(75 houses) were unable to survey due to either refusal against interview or absence of family. These 400 households were composed of 1,697 residents(male:830, female 867). Educational level of respondents showed 34.1% as elementary school graduated. Religion distribution showed Buddism(23.8%) as the most dominant. 50.7% of respondents married in the area. 2) Most households(91.5%) have lived in their own house in Sanpuk area. Average family size showed 4.3. More than half of residents(64.2%) have used public supplied water tap. Only 1.5% of the households had a flush toliet. The rest of households have still used primitive insanitary latrines. 3) 32.5% of residents have used gas burner for cooking and for heating in the house, and the coal briquet were used for boiler. Lack of convenient public transportation was the chief complaint for their day life. 4) Each household occupied 1,990 pyungs of rice paddy and 1,170 pyungs of ordinary field in average. Beside farming products, mushroom was the highest product. 5) Sixth percent of households in the survey area regularly participated in community meeting one hand and on the other hand 39.5% never participated. Most of respondents closely contacted with their neighbours and they seemed very friendly each other. 6) The prevalence rate of illness and injury during recent 15 days showed 48.3 per 1,000. The prevalence rate of chronic illnesses during the past one year showed 74 per 1,000. Injury and accident lead the higher portion(22.0%) in the former and in the latter pain(arthritis, back-pain) showed 27.0% as the dominant sickness. 87.8% of the ill residents in the former received medical treatment. As the most frequently utilized medical facility, the clinic or hospital were counted. Among the residents suffering from chronic illnesses, 77.3% in Sanpuk area get some kind of medical treatments and they rarely utilized the clinic or hospital. The reason why the patient did not receive any medical care was found out the fact that symptoms of illness was light or mild and economic problems was serious. 7) Average age of marriage showed 21.6 years old in the women and the average duration of marital period was shown for 15.1 years. The married woman in reproductive age in Sanpuk area had experienced pregnancies 4 times in the aver-age including 0.7 time of pregnancy in average were interrupted by induced abortion and 0.3 time by spontaneous abortion respectively. The practicing rate of the family planning of the married woman during reproductive ages showed 70.7% and the tuballigation was found out as the most frequently used contraceptives. 8) Among woman who has children under 2 years old, 70.0% had received the prenatal care for the last pregnancy. However, the average number of prenatal care visitis per woman showed 3.3 times. Fifty-two % of woman who received the postnatal care for the last delivery showed only 37.5%. 9) Immunization rate of the children under 2 years old showed relatively high and looked successful. The breast feeding for these children showed dominantly in the most. Most of the mothers in Sanpuk area had started the supplementary diet during weaning period of their infants of 6th and 7th month after birth. * : Sanpuk area is a demstration area for community development which has been supported by the Community Development Foundation during the part 10 years. The village is relatively closer to urban area such as Seoul, However, it has a similar characteristics shown as a remote village because of geographical location and inconvenient transportation at present.

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Congenital cystic adenomatoid malformation (선천성 낭포성 선종양기형 -1례 보고-)

  • Sun, Kyung;Baek, Kwang-Je;Lee, Chol-Sei;Chae, Sung-Soo;Kim, Hark-Jei;Kim, Hyung-Mook
    • Journal of Chest Surgery
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    • v.17 no.1
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    • pp.118-124
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    • 1984
  • Congenital Cystic Adenomatiod Malformation (C.C.A.M.) is rare, but one of the most common congenital pulmonary anomalies that cause acute respiratory distress in the newborn infants. It is characterized and differentiated from the diffuse pulmonary cystic disease pathologically, i.e. adenomatoid appearance due to marked proliferation of the terminal respiratory components. An 2/12 year old male patient was suffered from respiratory distress and cyanosis on crying since birth, but no specific therapy was given. With progression of symptoms, he came to Korea University Hospital for further evaluation and then transfered to Dept. of Chest Surgery for operative correction under the impression of Congenital Obstructive Emphysema suggested by a pediatrician. On gestational and family history, there was nothing to be concerned such as congenital anomaly. Physical examinations showed; moderate nourishment and development (Wt. 5.5kg), cyanosis on crying, both intercostal and lower sternal retraction on inspiration, Lt. chest building with tympany, Rt. shifting of cardiac dullness, decreased breathing sound with expiratory wheezing on entire Lt. lung field, decreased breathing sound on Rt. upper lung filed, and tachycardia. The remainders were nonspecific. Laboratory findings were normal except WBC $14000/mm^3$ (lymphocyte 70%), Hgb 9.8m%, Hct 28%, negative Mantaux test, and sinus tachycardia and counter-clockwise rotation on EKG. Preoperative simple Chest PA revealed marked hyperlucent entire Lt. lung, herniation of Lt. upper lobe to Rt., collapsed Rt. upper lobe, tracheal deviation and mediastinal shifting to Rt., and no pleural reaction. At operation, after Lt. posterolateral thoracotomy, 4th rib was resected. Operative findings were severe emphysematous changes limited to both lingular segmentectomy was done. The resected specimen showed slight solidity, measuring $8{\times}4.5{\times}2cm$ in size, and small multiple cystic spaces filled with air. Microscopically, entire tissue structures were glandular in appearance, cyst were lined by ciliated columnar epithelium, and occasional cartilages were noted around the cystic spaces. Bronchial elements were dilated but normal pattern on histologically. The patient had a good postoperative courses clinically and radiologically, and discharged on POD 10th without event. The authors report a case of Cogenital Cystic Adenomatoid Malformation (C.C.A.M.)

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