• Title/Summary/Keyword: Children under 6 years old

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Enzyme Replacement Therapy in Patients Who Have Mucopolysaccharidosis and Are younger than 5 years old (5세 미만 뮤코다당체침착증 환자에서의 효소 대체 요법)

  • Park, Seong-Won;Son, Yeong-Bae;Kim, Se-Hwa;Jo, Seong-Yun;Ji, Seon-Tae;Jin, Dong-Gyu
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.10 no.1
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    • pp.59-66
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    • 2010
  • Enzyme replacement of therapy (ERT) is one of the most promising therapeutic strategies for the treatment of lysosomal storage disorders. ERT is available in three types of Mucopolysaccharidosis (MPS): for MPS I (Aludrazyme$^{(R)}$), MPS II (Elaprase$^{(R)}$) and MPS VI (Naglazyme$^{(R)}$) patients who are over 5 years old. But recently, early diagnosis can be done by expert clinicians and even in prenatal case. We describe the case of ERT under 5 years old MPS patients. Up to June, 2010 in Samsung Medical Center, there are 6patients who were diagnosed as MPS and started ERT under 5 years old. 3 patients were MPS I, 3 patients were MPS II. 2 patient who was diagnosed as MPS I was female and others were male. Their age at diagnosis were 4 to 37month-old (4, 13, 16, 25, 27, 37 month-old) and they are now 9 to 60 month-old (9, 39, 32, 81, 60 month-old). The youngest patient was started ERT at 4 month-old and others were started at their 13 to 49 month-old (13, 29, 27, 28, 49 month-old). First manifested symptoms of patients were macrocephaly, kyphosis and coarse face appearance. Especially, in 2 of them, one was MPS I and the other was MPS II had elder brother with same disease. And the youngest one was diagnosed by the iduronate-2-sulfatase (IDS) gene analysis from chorionic villi sampling. His mother knew that she was a heterozygous carrier of IDS gene mutation because her younger brother died from MPS II. All of them confirmed as MPS by the enzyme assay in leukocytes and fibroblast skin culture. We started ERT with ${\alpha}$-L-iduronidase(Aldurazyme$^{(R)}$) to MPS I and did recombinant human iduronate-2-sulfatase (Elaprase$^{(R)}$) to MPS II patients as recommended dose as over 5 years old. But for MPS II patient who was 4 month old, we started ERT by recombinant human IDS (Elaprase$^{(R)}$) with reduced dose 0.1 mg/kg and increased dose every 2 weeks by 0.1mg/kg up to 0.5mg/kg IV infusion. During ERT, all patients had no adverse effects and the excretion of GAGs were decreased. We have evaluated other clinical symptoms such as liver/ spleen volume, heart function and neurologic evaluation. We describe a successful ERT to MPS I and MPS II patient under 5 years old without any adverse event. It indicates that ERT in young children are well tolerated and that it has several effects which may confer clinical benefits with long-term therapy.

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Detection of Adenovirus from Respiratory and Alimentary Tract in Pusan, 1999

  • Cho, Kyung-Soon;Kim, Young-Hee
    • Journal of Life Science
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    • v.10 no.2
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    • pp.17-20
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    • 2000
  • Adenovirus which is an important infectious viral agent in respiratory and alimentary tract was investigated in Pusan, 1999. Fifteen cases of adenovirus were detected from stools and throat swabs of suspected patients. Two cases of enteric adenovirus were detected from a 5 years old boy and a 6-month-old boy. Thirteen cases of respiratory adenoviruses were detected from children aged under 10 years old and one adult. From respiratory specimens, 1 case of adenovirus type 2, 1 case of type 5, and 11 cases of type 3 were found. Enterotype 41 was detected from fecal preparations. Adenoviruses appeared mostly during winter months, January, February and December. Adenovirus showed a slowly progressive cytopathic effect on HEp-2 cells, Vero cells and BGM cells at 37$^{\circ}C$, in a 5-7% $CO_{2}$ incubation. An electron microscopic observation exhibited non-enveloped icosahedron with a diameter of 70nm. No significant differences on cytopathic effect and morphological features have been found from specimens of either alimentary tract or respiratory secretions.

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Study on Remarriage of Bereaved or Divorced Old People -Emphasis on successful remarriage in Chonju Area- (사별 및 이혼한 노인의 재혼에 관한 연구 I -전주시 거주 재혼한 노인의 성공사례를 중심으로-)

  • 이정덕
    • Journal of the Korean Home Economics Association
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    • v.35 no.2
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    • pp.255-272
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    • 1997
  • In the point of view, this study aims to examine the life of remarriaged old people after divorce and bereavement. therefore, the purpose of this study is to increase the understanding of married couple's relationship and remarriage in old age and help old people who want to be abused about their remarriage as a coping response of lire for widowhood in old aged. the subject were 14 old people who married over 53 years old bereavement and divorce residing in Chonju, Chonbuk and they had indepth interviews. 1. Most of subject would married with help of matchmaker under the consent a children and relative 2. The main motivation of remarriage was lone lies, however, for men being taken care and for women economic security were also important. 3. Premarriage experience seemed to help spouse adjustment between couples. 4. They matintained close relationship with there biological children. 5. There show a tendency to the bereavement woman remarriage life was happier than divorce woman. 6. When one of the couple had regular income, little economic conflict was there. In most case, wifes were younger than husband and economic stability after husband's death was their main concern and when the husband didn't show definite will to inherit asset to his wife, conflict got bigger.

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The Clinical Effect of Gikwuibosin-tang on Nocturnal Enuresis (기귀보신탕 투여가 야뇨증 환아에 미치는 영향에 관한 임상적 연구)

  • Ju, Bong-Hyun;Jang, Ji-Hye;Jeong, Min-Jeong;Lee, Seung-Yeon
    • The Journal of Pediatrics of Korean Medicine
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    • v.20 no.2
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    • pp.139-146
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    • 2006
  • Objective : The objective of this study is to assess the clinical effect of Gikwuibosin_tang on nocturnal enuretic children. Methods : This clinical study was carried out with 15 cases of children aged 5 to 10 years old who visited the 00 Oriental Clinic between February 2005 and August 2005 and who were diagnosed as the nocturnal enuresis. The data of present symptom and improvement progress was collected a telephone interviews with their mother. Results : The total number of children was 15, 10 of them were male and 5 were female. Sex ratio was 2:1. Patients under 6 years old were 9(60%). After the treatment of oriental medicine der $2{\sim}3$ months, 6 of the children were improved completely and 7 of them were cured quite well, 2 of them were recovered in a half way. the treatment performed very well so there's no case which were not improved at all. And a ratio of the perfect cure was 40%. Following-up survey by telephone, the treatment were performed completely for 8 of them and 4 of them were almost improved but 3 of them were not improved. And a ratio of the perfect cure was 53.3%. Conclusion : Gikwuibasin-tang is effective in the treatment of nocturnal enuretic children. Further study is needed with more cases.

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Analyses of Impacts of the Outpatient Cost Sharing Reduction based on the Difference-in-differences Model (이중차이모델에 의한 건강보험 외래본인부담금 경감제도의 영향 분석)

  • Ahn, Lee-Su
    • The Journal of the Korea Contents Association
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    • v.13 no.11
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    • pp.187-197
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    • 2013
  • Objectives : The purpose of this study was to analyze the effect of outpatient cost sharing reduction on health care utilization of children under 6 years old. Methods : The data in this analysis was the health insurance claims data between August 2006 and July 2008. The study group was divided into two age groups, namely 0-5 years old and 6-10 years old. This study evaluated the impact of policy change on office visits per person and expenditure per visit. In order to do so, the double difference analysis is used. Results: The results showed that outpatient cost-sharing reduction has never really had a huge impact on office visits per person and expenditure per visit. Conclusions: This study showed that the outpatient cost sharing reduction for children under 6 years old policy is not working. Therefore, cost sharing of National Health Insurance by income class is needed.

Hypercalciuria and febrile convulsion in children under 5 years old

  • Gorabi, Vahid Seddighi;Nikkhoo, Bahram;Faraji, Obeidollah;Mohammadkhani, Mona;Mirzaee, Sattar;Rasouli, Mohammad Aziz;Afkhamzadeh, Abdorrahim
    • Clinical and Experimental Pediatrics
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    • v.61 no.4
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    • pp.129-131
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    • 2018
  • Purpose: The association between hypercalciuria and febrile convulsion is controversial. The present study aimed to investigate the statistical association between hypercalciuria and childhood febrile convulsions. Methods: Overall, 160 children aged 6 months to 5 years, including 80 children with febrile convulsion and 80 febrile children without convulsion (comparison group), were recruited. All laboratory tests, including 24-hour urine calcium, were undertaken in an academic clinical laboratory. Results: Forty-five children of the febrile convulsion group (60%) and 30 of the comparison group (40%) had hypercalciuria. There was a significant difference between the 2 groups (P=0.02). Conclusion: Our results indicated that there is a statistical association between convulsion and hypercalciuria in children. Since we found this association with a cross-sectional assessment, further studies, especially prospective and controlled designs, are needed.

Application of array comparative genomic hybridization in Korean children under 6 years old with global developmental delay

  • Lee, Kyung Yeon;Shin, Eunsim
    • Clinical and Experimental Pediatrics
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    • v.60 no.9
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    • pp.282-289
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    • 2017
  • Purpose: Recent advancements in molecular techniques have greatly contributed to the discovery of genetic causes of unexplained developmental delay. Here, we describe the results of array comparative genomic hybridization (CGH) and the clinical features of 27 patients with global developmental delay. Methods: We included 27 children who fulfilled the following criteria: Korean children under 6 years with global developmental delay; children who had at least one or more physical or neurological problem other than global developmental delay; and patients in whom both array CGH and G-banded karyotyping tests were performed. Results: Fifteen male and 12 female patients with a mean age of $29.3{\pm}17.6months$ were included. The most common physical and neurological abnormalities were facial dysmorphism (n=16), epilepsy (n=7), and hypotonia (n=7). Pathogenic copy number variation results were observed in 4 patients (14.8%): 18.73 Mb dup(2)(p24.2p25.3) and 1.62 Mb del(20p13) (patient 1); 22.31 Mb dup(2) (p22.3p25.1) and 4.01 Mb dup(2)(p21p22.1) (patient 2); 12.08 Mb del(4)(q22.1q24) (patient 3); and 1.19 Mb del(1)(q21.1) (patient 4). One patient (3.7%) displayed a variant of uncertain significance. Four patients (14.8%) displayed discordance between G-banded karyotyping and array CGH results. Among patients with normal array CGH results, 4 (16%) revealed brain anomalies such as schizencephaly and hydranencephaly. One patient was diagnosed with Rett syndrome and one with $M{\ddot{o}}bius$ syndrome. Conclusion: As chromosomal microarray can elucidate the cause of previously unexplained developmental delay, it should be considered as a first-tier cytogenetic diagnostic test for children with unexplained developmental delay.

Severe recurrent nocturnal hypoglycemia during chemotherapy with 6-mercaptopurine in a child with acute lymphoblastic leukemia

  • Cho, Eun Mi;Moon, Jung Eun;Lee, Soo Jung;Ko, Cheol Woo
    • Annals of Pediatric Endocrinology and Metabolism
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    • v.23 no.4
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    • pp.226-228
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    • 2018
  • Various endocrine dysfunctions occur during chemotherapy, including hypoglycemia. However, reports of hypoglycemia associated with 6-mercaptopurine (6-MP) are rare. Herein, we report an 8-year-old boy with severe symptomatic hypoglycemia likely due to 6-MP during chemotherapy. He had been diagnosed with acute lymphoblastic leukemia 3 years previously and was in the maintenance chemotherapy period. Treatment included oral dexamethasone, methotrexate, and 6-MP, of which only 6-MP was administered daily. Hypoglycemic symptoms appeared mainly at dawn, and his serum glucose dropped to a minimum of 37 mg/dL. Laboratory findings showed nothing specific other than increased serum cortisol, free fatty acids, ketone, alanine aminotransferase, and aspartate aminotransferase. Under the hypothesis of hypoglycemia due to chemotherapy drugs, we changed the time of 6-MP from evening to morning and recommended him to ingest carbohydrate-rich foods before bedtime. Hypoglycemia improved dramatically, and there was no further episode during the remaining maintenance chemotherapy period. To the best of our knowledge, this is the first report of this type of hypoglycemia occurring in an Asian child including Korean.

Child Rearing Practice of Working Mothers in a Poor Area of Pusan (부산시 영세지역 취업여성들의 영유아 양육실태)

  • Hwang, Yeon-Ja;Park, Jung-Han
    • Journal of Preventive Medicine and Public Health
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    • v.22 no.3 s.27
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    • pp.389-397
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    • 1989
  • To identify the problems in child rearing practice of employed mothers in urban poor area, all of 201 women with children under 6 years old living in Yun San 3 Dong, a poor area of Pusan city, were interviewed with a questionnaire by a trained interviewer from 10th April to 10th May 1989. Among 201 women, 51 women were employed and 150 women were unemployed. Of the employed mothers 78.5% got their jobs because of economical need and 31.4% of them worker for 60-69 hours per week. Their average monthly income was 100,000-190,000 Won in 33.4% and 200,000-290,000 Won in 25.4% Breast milk was fed in 66.0% of the children of unemployed mothers while 49.0 of the children of the employed mothers were breast-fed (p<0.05). The most common reason for not breast feeding was shortage of breast milk among unemployed mothers (58.9%) but that of the employed mothers was their job(63.6%). The basic immunization for children was completed in 70.5% of children of employed mothers as compared with 82.0% of the children of the unemployed mothers were completely immunized (p>0.1). Accident experience rate of children among the employed mothers was 23.5 a and that of the children of the unemployed mothers was 17.3% . The most common cause of acident in children of the unemployed mothers was carelessness while they were playing(34.6%) but in children of the employed mothers it was traffic accident(25.0%) and falling(25.0%). Most of the traffic accidents took place while the children's brother or sister of age under 14 years were baby sitting. When the accident took place 73.1% of the unemployed mothers were just at home, but 58.3% of the employed mothers were out of home for work. In case of the employed mothers, 58.7% of their children were looked after by an adult mainly grandparents, 15.7% by the children's brothers and sisters under 14 years old, and 3.9% of the children were left alone. A majority of the unemployed mothers wanted to get a job if someone could take care of their children. To facilitate the women employment and for the safety and health of the children, good nurseries for working mothers need to be established at a cost they can afford.

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Seroprevalence of Anti-EBV IgG among Various Age Groups from Khon Kaen Province, Thailand

  • Suntornlohanakul, Rabporn;Wanlapakorn, Nasamon;Vongpunsawad, Sompong;Thongmee, Thanunrat;Chansaenroj, Jira;Poovorawan, Yong
    • Asian Pacific Journal of Cancer Prevention
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    • v.16 no.17
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    • pp.7583-7587
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    • 2015
  • Epstein-Barr virus (EBV) is an extremely common herpesvirus that may cause asymptomatic infection or various diseases, including infectious mononucleosis, certain lymphoproliferative diseases and several types of neoplasms. Vaccine development is an important strategy to reduce the burden of EBV-associated diseases and the timing of vaccinations should be before primary infection occurs. In the past, more than 90% of Thai children were infected with EBV in early childhood. Now, due to the improved healthcare system in Thailand, we aim to determine current prevalence of EBV infection among people in different age groups. A total of 538 sera were collected from residents of Khon Kaen province in northeastern Thailand for detecting anti-EBV IgG. Sera of infants under 2-years-old were also tested for anti-EBV IgM and EBV-DNA. The prevalence of anti-EBV IgG was 47.1% (95% CI: 23.3-70.8) in infants aged 0-6 months, 34.9% (95% CI: 23.1-46.7) in those aged 6-24 months, 87.9% (95% CI: 79.5-96.3) in children aged 3-5 years and then maintained at above 95% through adulthood. These seropositivity rates among Thai children remain similar to those found in a previous study conducted 20 years ago. Thai children are still exposed to EBV from an early age. Therefore, a prophylactic vaccine should be given within the first two years of life.