• The Journal of the Korea Contents Association
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• v.11 no.6
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• pp.279-288
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• 2011

Nursing college students' continuous clinical experiences play an important part in the development of their nursing competency. In reality, it is difficult all of students to have enough clinical experiences. We can think of simulation learning as a solution to this problem. This research attempted to develop learning contents of simulation scenario regarding pediatric nursing. We organized the contents focusing on nursing process. The application of simulation scenario showed that the educational effect. In conclusion, this research suggests that nursing college students can supply high-quality nursing services to children and their families by developing and application of simulation scenario with various cases.

• Journal of the Korea Academia-Industrial cooperation Society
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• v.13 no.7
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• pp.3016-3022
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• 2012

The purpose of this study is to measure and evaluate the degree of the pain using tools such as faces, glasses, chips, and colors for 96 children in three general hospitals. The children preferred faces, glasses, chips and colors in order, and they preferred orange color for pain-free and black for intense pain. The pain score was significantly high with the children who did not have experience in hospital except colors tool in the difference by means of general characteristics(faces, glasses, chips; p<0.05). Faces, glasses, chips, colors tools are pain assessment tools which are easy simple to handle by children including family and medical personnel. We expect these tools will be used in many ways to assess pain according to a child's age in a clinical setting.

• Clinical and Experimental Pediatrics
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• v.50 no.8
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• pp.789-793
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• 2007

Basal cell nevus syndrome (BCNS), also known as Gorlin syndrome, is a rare autosomal dominant disorder characterized by variable manifestations, including multiple basal cell carcinomas, odontogenic keratocysts of the jaw, skeletal anomalies including scoliosis and bifid ribs, palmar and plantar pits, calcification of the falx cerebri, and biparietal frontal bossing. We report a case of a 9-year-old boy with the clinical features of basal cell nevus syndrome, in which a PTCH gene mutation was confirmed by DNA testing.

• Clinical and Experimental Pediatrics
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• v.49 no.3
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• pp.298-304
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• 2006

Purpose : We compared the asthma predictive index(API) and the modified asthma predictive index (mAPI) of the Tuscon Children's Respiratory Study Group in Korean children with recurrent wheezing. We investigated the atopic profiles and presence of allergen sensitization of each risk group, and ascertained the significant clinical risk factors. Methods : Two hundred and sixty two children, who visited for recurrent wheezing from 1998 to 2005, were enrolled and divided into groups by API and mAPI. We investigated the history of the patients and their families, atopic profiles, and sensitization to aeroallergen and food allergens. Twenty nine children were followed up to 6 years of age and we evaluated the sensitivity, specificity and positive and negative predictive value of both indices. Results : The high risk group of API were of older age, were more likely to be sensitized to aeroallergen(P=0.001) and food allergen(P=0.034) and had higher levels of total eosinophil count, eosinophil percent, serum ECP, total IgE, and D.p-, D.f-specific IgE. High risk group of mAPI showed higher levels of atopic markers such as egg-, milk-, D.p- and D.f-specific IgE. Even though API did not include allergen sensitization, the high risk group was more significantly sensitized to common allergens than the low risk group. Twenty nine children were followed up until 6 years of age; therefore 15 children were diagnosed as asthma, clinically. The sensitivity, specificity, positive and negative predictive values of mAPI were higher than API. Conclusion : Both high risk groups of API and mAPI had higher levels of atopic markers and were more sensitized to common allergens. These findings suggest that sensitization to aeroallergens and food allergens are more objective markers as asthma predictive indices. In addition, mAPI is a more reliable index in predicting asthma in Korean children with recurrent wheezing than is API. But only 29 patients were followed until the age of 6, so we need to include more children with long term follow up for future study.

• Clinical and Experimental Pediatrics
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• v.48 no.10
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• pp.1132-1138
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• 2005

Purpose : The mucopolysaccharidoses (MPSs) are a heterogeneous group of lysosomal storage disorders. They are caused by a deficiency of the enzymes involved in the degradation of glycosaminoglycans. Early recognition is important because recombinant enzyme replacement therapy is now available for MPS. We studied the clinical characteristics of 80 MPS children with the object of determining the epidemiological, clinical and radiological features in Korean MPS children. Methods : Diagnosis of MPS was confirmed by skin fibroblast enzyme analysis in 80 patients between February 1995 and December 2004. Charts were retrospectively reviewed for clinical and radiological findings, as well as for intelligence and speech evaluations. Results : Hunter syndrome (MPS type II) was the most prevalent type, appearing in 51/80 cases (64 %), followed by Sanfilippo syndrome (MPS III-18%), Hurler syndrome (MPS I-15%), and Morquio syndrome (MPS IV-4%). The average age at diagnosis was 5.5 years (range 1 to 20), and the male-to-female ratio was 4.7 : 1. Typical radiographic changes were observed in 45/54 cases (83%). Mitral regurgitation was the most common cardiac defect. Moderate to profound mental retardation and hearing loss were present in 14/35 cases (56%) and 33/38 cases (82%), respectively. Four MPS II patients had bone marrow transplantation, with mixed outcomes. Five MPS I patients are currently on enzyme replacement therapy. Conclusion : Our study showed a high proportion of MPS II cases (64%), which may represent population variability. By studying the clinical features of these patients, we hope to alert pediatricians of the warning signs of MPS.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.17 no.1
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• pp.1-10
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• 2017

Purpose: This study aimed to investigate the perception and emotional experiences in rare and intractable diseases for caregivers of pediatric patients with mitochondrial diseases in order to provide therapeutic interventions for patients, caregivers, and families. Methods: A total of 83 caregivers of pediatric patients with mitochondrial diseases were recruited from the pediatric mitochondrial disease clinics of the Gangnam Severance Hospital in South Korea. Participants completed the survey about their perception of mitochondrial disease and emotional experiences after the diagnosis, and these clinical data were analyzed accordingly. Results: Surveys from a total of 83 caregivers of patients were analyzed, and the patients' age ranged from 6 to 12 years (33%), followed by ages 1 to 6 years (30%). Children with mitochondrial diseases were between 0 and 0.5 years of age at the time of first symptom onset (43%), and the duration of illness lasted more than 10 years in most cases (42%). Prior to diagnosis of mitochondrial diseases, the amount of awareness the caregivers had was 'Not at all' for both rare and intractable diseases and mitochondrial diseases in 44 cases and 68 cases, respectively. For the caregivers' emotional experiences, the most common initial responses were 'Discouraged/despair', 'Helpless/lethargic', and 'Disconcerted'. 'Anxious', 'Committed to treatment', and 'Responsibility as family members' were the most common emotional responses from the caregivers, followed by 'Disconcerted' and 'Helpless/lethargic'. Conclusion: It is important to consider the level of perception and emotional experiences of caregivers and patients with rare and intractable mitochondrial diseases for planning treatment programs.

• Journal of agricultural medicine and community health
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• v.41 no.3
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• pp.119-128
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• 2016

Objective: This study was to investigate the knowledge, health belief, and vaccination behavior on hepatitis A among university students. Methods: A self-administered questionnaire survey was conducted from $3^{rd}$ to $25^{th}$ March, 2014 and 197 subjects were enrolled in statistical analysis. Results: The result showed that the score for knowledge of the subjects on hepatitis A was $4.59{\pm}3.06$ out of 15 points, for health belief $2.39{\pm}0.28$ out of 4 points, and the vaccination rate of the subjects was 12.7%. There was significant difference in hepatitis A knowledge score according to experience of hepatitis A check-up and hepatitis A vaccination history of family members, and in health belief according to gender. For hepatitis A vaccination there was significant difference according to experience of hepatitis A check-up, family history of liver disease, hepatitis A vaccination history of family members, and education history of hepatitis A. As a result of logistic regression analysis experience of hepatitis check-up and hepatitis A vaccination history of family members were significant factors for hepatitis A vaccination. Conclusions: The level of knowledge, health belief for hepatitis A of the subjects was low and vaccination rate also low. The experience of hepatitis A check-up and hepatitis A vaccination history of family members were factors affecting hepatitis A vaccination. It would be necessary to develop programs for improving level of knowledge and health belief and raising the rate of hepatitis A vaccination for the university students in Korea.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.6 no.1
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• pp.10-14
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• 2010

Vitamin D-resistant rickets(VDRR) is hereditary disease manifesting marked hypophosphatemia caused by renal tubular loss of phosphate into urine and an associated decrease in the calcium and phosphorous ion product. VDRR is identified by clinical symptoms, such as in the limbs, gait disturbance, dwarfism, familial occurrence, bowlegs, and knock-knees, as well as by laboratory findings. Dental findings are enlarged chambers and extension of the pulp horns into the cusp tips in both the primary and the permanent dentition in contrast to the vitamin D-deficient rickets. The major oral manifestations are multiple spontaneous abscesses in a caries-free dentition. We reported the clinical features and treatment of a 6-year-old boy with vitamin D-resistant rickets referred to the department of pediatric dentistry in Kyung Hee University for multiple spontaneous periapical abscesses and gingival fistula without severe dental caries.

• Clinical and Experimental Pediatrics
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• v.50 no.5
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• pp.489-492
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• 2007

Idiopathic renal hypouricemia is a disorder characterized by impaired urate handling in the renal tubules. This disease usually produces no symptoms, but hematuria, uric acid nephrolithiasis or acute renal failure may develop. A defect in the SLC22A12 gene, which encodes the human urate transporter, is the known major cause of this disorder. We describe a 10-month-old boy with idiopathic renal hypouricemia. He was diagnosed with transient pseudohypoaldosteronism at admission, but hypouricemia was accidentally found through follow-up study. By gene analysis, his diagnosis was confirmed to idiopathic renal hypouricemia. In addition, we report a mutation in the human urate transporter 1 (hURAT1) gene identified in his family.

• Childhood Kidney Diseases
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• v.12 no.1
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• pp.111-115
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• 2008

Vitamin D-dependent rickets(VDDR) is a rare autosomal disorder, characterized by hypocalcemia, hypophosphatemia, increased alkaline phosphatase, secondary hyperparathyroidism and many other clinical features. Type I VDDR is due to congenital defects of renal 1${\alpha}$-hydroxylase, the enzyme responsible for the conversion of 25-(OH)D3 to 1,25-$(OH)_2D3$. Type II VDDR arise from target organ resistance to 1,25-$(OH)_2D3$. Unilateral renal aplasia is generally thought to result from a lack of induction of the metanephric blastema from the ureteral bud, which may be secondary to ureteral bud maldevelopment and/or to a problem with the formation of the mesonephric duct. The incidence of unilateral renal aplasia is approximately 1/500-3,200. Type 1 VDDR associated with unilateral renal aplasia has not been reported yet. Thus we report a case of a 3 month old female infant diagnosed as type 1 VDDR with unilateral aplasia of kidney.