• Clinical and Experimental Pediatrics
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• 제59권sup1호
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• pp.25-28
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• 2016

Phelan-McDermid syndrome is a rare genetic disorder caused by the terminal or interstitial deletion of the chromosome 22q13.3. Patients with this syndrome usually have global developmental delay, hypotonia, and speech delays. Several putative genes such as the SHANK3, RAB, RABL2B, and IB2 are responsible for the neurological features. This study describes the clinical features and outcomes of Korean patients with Phelan-McDermid syndrome. Two patients showing global developmental delay, hypotonia, and speech delay were diagnosed with Phelan-McDermid syndrome via chromosome analysis, fluorescent in situ hybridization, and multiplex ligation-dependent probe amplification analysis. Brain magnetic resonance imaging of Patients 1 and 2 showed delayed myelination and severe communicating hydrocephalus, respectively. Electroencephalography in patient 2 showed high amplitude spike discharges from the left frontotemporoparietal area, but neither patient developed seizures. Kidney ultrasonography of both the patients revealed multicystic kidney disease and pelviectasis, respectively. Patient 2 experienced recurrent respiratory infections, and chest computed tomography findings demonstrated laryngotracheomalacia and bronchial narrowing. He subsequently died because of heart failure after a ventriculoperitoneal shunt operation at 5 months of age. Patient 1, who is currently 20 months old, has been undergoing rehabilitation therapy. However, global developmental delay was noted, as determines using the Korean Infant and Child Development test, the Denver developmental test, and the Bayley developmental test. This report describes the clinical features, outcomes, and molecular genetic characteristics of two Korean patients with Phelan-McDermid syndrome.

• 간호행정학회지
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• 제6권1호
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• pp.135-146
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• 2000

The purpose of this study was to develope, based on the Nursing Intervention Classification (NIC) system. a set of standardized nursing interventions which had been validated. and their associated activities. for use with nursing diagnoses related to home health care for women who have had a caesarian delivery and for their newborn babies. This descriptive study for instrument development had three phases: first. selection of nursing diagnoses. second, validation of the preliminary home health care interventions. and third, application of the home care interventions. In the first phases, diagnoses from 30 nursing records of clients of the home health care agency at P. medical center who were seen between April 21 and July 30. 1998. and from 5 textbooks were examined. Ten nursing diagnoses were selected through a comparison with the NANDA (North American Nursing Diagnosis Association) classification In the second phase. using the selected diagnoses. the nursing interventions were defined from the diagnoses-intervention linkage lists along with associated activities for each intervention list in NIC. To develope the preliminary interventions five-rounds of expertise tests were done. During the first four rounds. 5 experts in clinical nursing participated. and for the final content validity test of the preliminary interventions. 13 experts participated using the Fehring's Delphi technique. The expert group evaluated and defined the set of preliminary nursing interventions. In the third phases, clinical tests were held at in a home health care setting with two home health care nurses using the preliminary intervention list as a questionnaire. Thirty clients referred to the home health care agency at P. medical center between October 1998 and March 1999 were the subjects for this phase. Each of the activities were tested using dichotomous question method. The results of the study are as follows: 1. For the ten nursing diagnoses. 63 appropriate interventions were selected from 369 diagnoses interventions links in NlC., and from 1.465 associated nursing activities. From the 63 interventions. the nurses expert group developed 18 interventions and 258 activities as the preliminary intervention list through a five-round validity test 2. For the fifth content validity test using Fehring's model for determining lCV (Intervention Content Validity), a five point Likert scale was used with values converted to weights as follows: 1=0.0. 2=0.25. 3=0.50. 4=0.75. 5=1.0. Activities of less than O.50 were to be deleted. The range of ICV scores for the nursing diagnoses was 0.95-0.66. for the nursing interventions. 0.98-0.77 and for the nursing activities, 0.95-0.85. By Fehring's method. all of these were included in the preliminary intervention list. 3. Using a questionnaire format for the preliminary intervention list. clinical application tests were done. To define nursing diagnoses. home health care nurses applied each nursing diagnoses to every client. and it was found that 13 were most frequently used of 400 times diagnoses were used. Therefore. 13 nursing diagnoses were defined as validated nursing diagnoses. Ten were the same as from the nursing records and textbooks and three were new from the clinical application. The final list included 'Anxiety', 'Aspiration. risk for'. 'Infant behavior, potential for enhanced, organized'. 'Infant feeding pattern. ineffective'. 'Infection'. 'Knowledge deficit'. 'Nutrition, less than body requirements. altered', 'Pain'. 'Parenting'. 'Skin integrity. risk for. impared' and 'Risk for activity intolerance'. 'Self-esteem disturbance', 'Sleep pattern disturbance' 4. In all. there were 19 interventions. 18 preliminary nursing interventions and one more intervention added from the clinical setting. 'Body image enhancement'. For 265 associated nursing activities. clinical application tests were also done. The intervention rate of 19 interventions was from 81.6% to 100%, so all 19 interventions were in c1uded in the validated intervention set. From the 265 nursing activities. 261(98.5%) were accepted and four activities were deleted. those with an implimentation rate of less than 50%. 5. In conclusion. 13 diagnoses. 19 interventions and 261 activities were validated for the final validated nursing intervention set.

• 여성건강간호학회지
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• 제8권2호
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• pp.301-313
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• 2002

The purpose of this study was to develop the frame for standardized manual for Sanhujoriwon by finding out the status of the management of women & newborn in Sanhujoriwon, like postpartum care center. The subjects were 95 staffs of 22 Sanhujoriwon agreed on oral consent, in Seoul and Bundang, Korea. Data were collected from Sep. 15 to Oct. 24, 2001. The instrument used for this study was a structured questionnaire consisted of 16 items of general characteristics & educational characteristics about postpartum care, 31 items of degree of management of women's postpartum care (Cronbach's $\alpha$.93 ), 24 items of degree of management of newborn's care(Cronbach's $\alpha$.94 ), 10 items of methods of management of women's postpartum care, 8 items of methods management of newborn's care. The data were analyzed by the SPSS/PC+ program using frequency, percentage, mean, standard deviation, t-test, ANOVA and Pearson correlation coefficient. The results of the study were as follows ; 1. The mean of the degree of management for postpartum women was 4.1. The mean score for the degree of management of general postpartum care for women was 4.00 & traditional postpartum care(Sanhujori) was 4.20. The degree of management of traditional postpartum care was higher than general postpartum care for women. 2. The degree of management for newborn : The mean score for the degree of management of newborn was 4.37. 3.There was a strong positive correlation between general postpartum care and traditional postpartum care($r=.744^{**}$), and postpartum mother care and newborn care($r=.798^{**}$). 4.The basic frame for the management of the women and newborn in Sanhujoriwon. 1) For women: Integrated postpartal care Physical management : Vital sign & BP check, contraction of uterus, form and amount of lochia, management of personal hygiene, management of breast & breast-feeding, management of postpartum exercise, prevention of infection, symptom & sign of high risk and prevention & management of high risk condition; Emotional-psychological management: assessment and management of mother-baby attachment, emotional state; Educational management : education of vaccination schedule, urinary incontinence, rearing infant, breast-feeding ; and Environmental management : temperature, humidity, disinfection, cleaning, light, infection control integrated with 6 principles of Sanhujori. 2) For newborn Physical management : check of vital sign, management of umbilical cord, jaundice, prevention of infection, management of diaper rash; Emotional-psychological management : assessment of sleep, crying, activity, response of mother-baby attachment; and Environmental management : temperature, humidity, disinfection, cleaning, light, infection control integrated with 6 principles of Sanhujori. In conclusion Sanhujoriwon must be health care center for the postpartum women and newborn. Therefore, the establishment of various laws and regulations in such a way to meet the realistic needs of Sanhujoriwon as a health care center for women and infants future health should be done. The standardized management manual based on the results is absolutely required above all.

• Clinical and Experimental Pediatrics
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• 제53권6호
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• pp.694-700
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• 2010

Purpose: To assess the validity of individual and combined prognostic effects of severe bronchopulmonary dysplasia (BPD), brain injury, retinopathy of prematurity (ROP), and parenteral nutrition associated cholestasis(PNAC). Methods: We retrospectively analyzed the medical records of 80 extremely low birthweight (ELBW) infants admitted to the neonatal intensive care unit (NICU) of the Severance Children's Hospital, and who survived to a postmenstrual age of 36 weeks. We analyzed the relationship between 4 neonatal morbidities (severe BPD, severe brain injury, severe ROP, and severe PNAC) and poor outcome. Poor outcome indicated death after a postmenstrual age of 36 weeks or survival with neurosensory impairment (cerebral palsy, delayed development, hearing loss, or blindness) between 18 and 24 months of corrected age. Results: Each neonatal morbidity correlated with poor outcome on univariate analysis. Multiple logistic regression analysis revealed that the odds ratios (OR) were 4.9 (95% confidence interval [CI], 1.0-22.6; $P$=0.044) for severe BPD, 13.2 (3.0-57.3; $P$<.001) for severe brain injury, 5.3 (1.6-18.1; $P$=0.007) for severe ROP, and 3.4 (0.5-22.7; $P$=0.215) for severe PNAC. Severe BPD, brain injury, and ROP were significantly correlated with poor outcome, but not severe PNAC. By increasing the morbidity count, the rate of poor outcome was significantly increased (OR 5.2; 95% CI, 2.2-11.9; $P$<.001). In infants free of the above-mentioned morbidities, the rate of poor outcome was 9%, while the corresponding rates in infants with 1, 2, and more than 3 neonatal morbidities were 46%, 69%, and 100%, respectively. Conclusion: In ELBW infants 3 common neonatal mornidifies, severe BPD, brain injury and ROP, strongly predicts the risk of poor outcome.

• 임상간호연구
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• 제20권3호
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• pp.313-325
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• 2014

Purpose: This study aimed to develop the protocol of kangaroo care for premature infants and to evaluate its effects including physiologic responses of premature infants, their parents'anxiety, and neonatal nurses'perception regarding the kangaroo care. Methods: Kangaroo care protocol was developed through a literature review and validation of an expert group. The developed kangaroo care protocol was tested with 27 preterm infants, 24 parents, and 60 nurses in one neonatal intensive care unit in S hospital. Data were collected from December 2012 to February 2013 and analyzed by descriptive statistics and t-test using the SPSS/Win statistical program. Results: Physiologic responses including heart rate, respiration rate, oxygen saturation, body temperature among premature infants received the kangaroo care were maintained within normal limit. The anxiety levels of the parents who provided kangaroo care for their infants were significantly decreased as it was compared before and after care (t=3.43, p=.002). There were no significant differences in nurses'perceptions about kangaroo care before and after the application of the protocol. Conclusion: The developed kangaroo care protocol is proved to be effective and can be used safely in nursing practice.

• 한국식생활문화학회지
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• 제11권2호
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• pp.217-228
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• 1996

Infanthood is a crucial period when mental and emotional developments take place along with physical growth. Adequate supply of nutrition is essential during this period and there is an urgent need for proper education and guidance on balanced nutrition. Therefore, this study was designed to assess to current status of lactation and to investigate mothers' perspectives on human milk and formular milk. As random samples for this study, a total of 352 mothers having infant under the age of 4 were surveyed. 27.3% of mother surveyed were breast-feeding, 38.0% were formula-feeding and 34.7% were mixed-feeding. According to the result of this study, the percentage of breast feeding was on the decrease, while the dependence on formular milk was on the rise. The main reason of formula-feeding was insufficient secretion of human milk on mothers part. The major factors that affect to select feeding method were employment status, degree of education, family income and age. Also, the interviewees desired the product fortified with special ingredients that are hot contained in formular milk currently available in the market. The most concerned problem of formula milk was indigestion and the interviewees wanted the formular milk to be improved digestibility.

• 보건행정학회지
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• 제1권1호
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• pp.54-71
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• 1991

Health of a nation is quite often represented by the statistics such as infant death rate and maternal mortality rate. It is indisputable that maternal child health(MCH) is the basis of health of a nation. MCH is also one of the cardinal component of primary health care. The importance of MCH is conspicuous especially in the developing countries. In Korea, People in the rural communities still have high access barrier to basic health care needs, including MCH services. Access to quality care during pregnancy and delivery seems to be the crucial factor in preventing deaths in women and children. The beneficial effects of prenatal and postnatal care on the outcome of pregnancy for mother and child, and those of health professional-attended institutional delivery on the health of mother and child have been well documented in many studies. Recognizing these effects, the government of Korea received IBRD loan of $30 million in 1979 for th purpose of constructing 89 rural MCH centers. The construction is complete now and all 89 MCH centers are under operation ti imporve primary health care for mothers and children in Korea. However, it has been observed over time that overall performance of public MCH centers is declining. The decline has been attributed partly to low quality services by public MCH centers, poor management by health center mangers, competition with for-profit private clinics, and to the development of national health insurance. This study investigates the utilization by rural communities in Korea of MCH services provided by public sector health centers deemed to be physically and financially accessible to the community but suboptimally used. It seeks also to determine the factors that influence people's utilizations. This study sets out to discover a desirable form of MCH center from among alternative forms of centers, thereby to construct a MCH model.

• Journal of Korean Neurosurgical Society
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• 제52권4호
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• pp.417-419
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• 2012

Tumors of the central nervous system are common in the pediatric population and constitute the second most prevalent tumor type in children. Within this group, spinal cord tumors are relatively rare and account for 1 to 10% of all pediatric central nervous system tumors. We describe a very rare case of an intradural extramedullary spinal cord tumor with a subcutaneous mass and discuss its clinical presentation, pathogenesis, and treatment. A male infant was delivered normally, with uneventful development. At 16 days post-delivery, his family took him to a pediatrician because of a mass on his upper back. Magnetic resonance imaging of the thoracic spine revealed a well-demarcated soft-tissue mass with central cystic change or necrosis at the subcutaneous layer of the posterior back (T2-7 level). Another mass was found with a fat component at the spinal canal of the T1-3 level, which was intradural extramedullary space. After six weeks, the spinal cord tumor and subcutaneous mass were grossly total resected; pathologic findings indicated an atypical myxoid spindle cell neoplasm, possibly nerve sheath in type. The final diagnosis of the mass was an atypical myxoid spindle cell neoplasm. The postoperative course was uneventful, and the patient was discharged after nine days without any neurological deficit. We report a rare case of an intradural extramedullary spinal tumor with subcutaneous mass in a neonate. It is necessary to monitor the patient's status by examining consecutive radiologic images, and the symptoms and neurological changes should be observed strictly during long-term follow-up.

• 농업과학연구
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• 제40권4호
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• pp.359-365
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• 2013

The aim of this study was to produce enzymatic hydrolysis of ${\alpha}$-LA, ${\beta}$-LG and BSA with alcalase for the possible application of hypoallergenic foods toward cow's milk allergenic infant. The molecular weights of most of the peptides in hydrolysates from ${\alpha}$-LA, ${\beta}$-LG and BSA by alcalase were below 3,000 dalton. Antigenesity of ${\alpha}$-LA, ${\beta}$-LG and BSA hydrolysates to rabbit anti-${\alpha}$-LA antiserum, ${\beta}$-LG antiserum and BSA antiserum were remarkably decreased by more than $10^{-3}$ at 20% inhibitionrate. Antigenesity of polyvalent antigenic peptide in ${\alpha}$-LA, ${\beta}$-LG and BSA hydrolysates to specific rabbit anti-${\alpha}$-LA antiserum, ${\beta}$-LG antiserum and BSA antiserum was determined by PCS test using guina-pig. Hydrolysates of ${\alpha}$-LA, ${\beta}$-LG and BSA with less than 3,000 dalton did not show polyvalent antigenic reaction against rabbit antiserum. Hydrolysates of ${\alpha}$-LA, ${\beta}$-LG and BSA could be a source for the manufacturing of hypoallergenic food.

• 한국농공학회지
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• 제33권3호
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• pp.73-80
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• 1991

In Korea, small-scale reclaimed areas have been suffering from many problems because of the lack of comprehensive developing strategy although considerable investments have been inputed by the public sector since 1970's. For 3 reclaimed sites in Chonnam Province chosen as case study areas, the analysis, the third attempt of widely-spanned studies on areal conditions, concentrated on their living conditions. Its results were as follows : 1. Although rural residents have increased their concern on health and medical services, the public sector can only serve basic and emergent level of them, and therefore, the private sector, which is mostly placed in urban areas and costed much higher than public one, has the dominant share of those services. So, because those costs are a great expense to rural residents, their supplying system should be planned with special reference to lightening the economic burden of them. 2. By the development of locally fitted programs and innovative systems, the rural education should be qualitatively improved to deal successfully with its small scale. That will result in increasing the schooling rate to schools in rural communities, lightening the economic burden of rural residents on education services and finally activating them to contribute the betterment of rural education. 3. Servicing level of water supply, sewerage and garbage disposal in the reclaimed areas has been remaining at the lower order even in other rural areas. In the design of rural water supply, average daily consumption per capita should be changed according to the composition rate of fishing households and variety of the source of water supply. 4. Most of rural residents in the reclaimed areas want to establish the welfare facilites for the infant, youth, aged and public bath However in the long-term basis, the cooperative production and processing facilities should be considered for modernized efficient farming.