• Title/Summary/Keyword: child initiated

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A Case of Dihydropteridine Reductase Deficiency (Dihydropteridine Reductase 결핍증 1례)

  • Oh, Se-Jung;Hong, Yong-Hee;Lee, Yong-Wha;Lee, Seung-Tae;Ki, Chang-Seok;Lee, Dong-Hwan
    • Journal of Genetic Medicine
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    • v.6 no.2
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    • pp.170-174
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    • 2009
  • Tetrahydrobiopterin ($BH_4$) deficiency is caused by mutations in genes encoding enzymes involved in the synthesis and regeneration of $BH_4$. The condition is usually accompanied by hyperphenylalaninemia (HPA) and deficiency of neurotransmitter precursors L-dopa and 5-hydroxytryptophan. $BH_4$ deficiency is much rarer than classical phenylketonuria. Dihydropteridine reductase (DHPR) deficiency, an autosomal recessive genetic disorder, is a cause of malignant hyperphenylalaninemia due to $BH_4$ deficiency. When left untreated, DHPR deficiency leads to neurologic deterioration at the age of 4 or 5 months, including psychomotor retardation, tonicity disorders, drowsiness, irritability, abnormal movements, hyperthermia, hypersalivation, and difficulty swallowing. Treatment of DHPR deficiency should be initiated as early as possible with $BH_4$ supplementation and replacement of the neurotransmitter precursors L-dopa and 5-hydroxytryptophan. We report the first case of DHPR deficiency in Korea, a child diagnosed at 9 years of age by genetic testing.

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Fatal Balamuthia Amebic Encephalitis in a Healthy Child: A Case Report with Review of Survival Cases

  • Krasaelap, A.;Prechawit, S.;Chansaenroj, J.;Punyahotra, P.;Puthanakit, T.;Chomtho, K.;Shuangshoti, S.;Amornfa, J.;Poovorawan, Y.
    • Parasites, Hosts and Diseases
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    • v.51 no.3
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    • pp.335-341
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    • 2013
  • Balamuthia mandrillaris is one of the 4 amebas in fresh water and soil that cause diseases in humans. Granulomatous amebic encephalitis (GAE), caused by B. mandrillaris, is a rare but life-threatening condition. A 4-year-old, previously healthy, Thai girl presented with progressive headache and ataxia for over a month. Neuroimaging studies showed an infiltrative mass at the right cerebellar hemisphere mimicking a malignant cerebellar tumor. The pathological finding after total mass removal revealed severe necrotizing inflammation, with presence of scattered amebic trophozoites. Cerebrospinal fluid (CSF) obtained from lumbar puncture showed evidence of non-specific inflammation without identifiable organisms. A combination of pentamidine, sulfasalazine, fluconazole, and clarithromycin had been initiated promptly before PCR confirmed the diagnosis of Balamuthia amebic encephalitis (BAE). The patient showed initial improvement after the surgery and combined medical treatment, but gradually deteriorated and died of multiple organ failure within 46 days upon admission despite early diagnosis and treatment. In addition to the case, 10 survivors of BAE reported in the PubMed database were briefly reviewed in an attempt to identify the possible factors leading to survival of the patients diagnosed with this rare disease.

A case of steroid-induced psychosis in a child having nephrotic syndrome with toxic epidermal necrolysis (신증후군 환아에서 발생한 독성표피괴사용해 치료를 위해 사용된 고용량 스테로이드로 인한 정신질환 1례)

  • Kim, Sae Yoon;Lee, Jae Min;Park, Yong Hoon
    • Clinical and Experimental Pediatrics
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    • v.53 no.3
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    • pp.437-441
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    • 2010
  • Toxic epidermal necrolysis (TEN) and Stevens Johnson syndrome (SJS) are rare, life-threatening mucocutaneous -diseases, usually attributable to drugs and infections. Corticosteroids have been used in the management of TEN for the last 30 years. This remains controversial and is still much debated. TEN can occur despite administration of high doses of systemic corticosteroids. The psychiatric side effects of corticosteroids can include headache, insomnia, depression, and mood disorders with or without psychotic episodes. Steroid-induced psychosis is dealt with by tapering or discontinuing the steroid; antipsychotics are also sometimes used. We report a case of an 11-year-old boy who was admitted with TEN. He had also been diagnosed as having nephrotic syndrome in the past. Remission was achieved through induction therapy and by maintaining the use of steroids. After a full-dose intravenous dexamethasone for TEN, he showed psychotic symptoms. We diagnosed him as having steroid-induced psychosis. We tapered the steroid use and initiated an atypical antipsychotic medication, olazapine and intravenous immunoglobulin (IV-IG). His symptoms dramatically improved and he was discharged.

A Study on Acupoint SP3 in Saam Acupuncture Method (사암도인침법에서 태백혈의 운용에 대한 연구)

  • Kim, Song-Yi;Lee, Soon-Ho;Park, Ji-Yeun;Park, Hi-Joon
    • Korean Journal of Acupuncture
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    • v.31 no.4
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    • pp.179-187
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    • 2014
  • Objectives : Saam acupuncture initiated by Saamdoin is traditional and originative method, which is characterized by applying the five phases theory and mother-child reinforcement-reduction principle to the selection of acupoints and needling manipulation. Our study was aimed to summarize and assess the use of acupoint SP3(Taebaek) in Saam acupuncture treatment and to further understand Saam acupuncture in an aspect of the combination of acupoints. Methods : We searched the data based on "(Do Hae Kyo Kam) Sa-Ahm's acupuncture method" for SP3 used and acupoint combination including SP3. We performed frequency analysis, network analysis, and cluster analysis for quantitative aspect. To understand clinical implication of SP3 with another acupoint, qualitative and descriptive methods were also carried out. Results and Conclusions : In our study, SP3 was frequently used for tonification of lung, spleen, heart, and kidney meridian and sedation of kidney, heart, and lung meridian. For this, many acupoints such as LU8, LU9, KI3, HT8, KI7, LU10 and LR1 were used with SP3. The combination of SP3 and other acupoints were used to treat stroke, common cold, and pain conditions including headache, low back pain, respiratory disease as well as gastroenteric troubles including stomachache, indigestion, vomiting, and constipation. To further understand Saam acupuncture, an understanding of the five transport points based on five elements characters, pathological changes (deficiency and excess) of viscera and bowels, and concept of source point should be preceded.

A Study on the Analysis of School Health Program by the Chronological Events in Korea (우리나라 학교보건사업 변천에 관한 연구)

  • Kim, Sang-Wook
    • Journal of environmental and Sanitary engineering
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    • v.4 no.2 s.7
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    • pp.61-90
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    • 1989
  • The purpose of this study was to identify and name clusters of school health program, and to describe some of the characteristics of administrative supports. The literature, materials and public documents were analysed by the chronological events from 1945 to 1989. The result of this study is as follows : 1. A brief summary of the history of school health program was included as an introduction to the analysis of the current programs of school health. Five current school health-program clusters were identified from findings of a study of programs ; 1) Physical assessment, laboratory examination and health services for the students, 2) health insturction 3) healthful living condition(environmental health), 4) health clinic management 5) administrative supports. 2. The earliest school-based efforts focused on communicable disease prevention by the ministry of health and social affairs. Annual medical inspection(health assessment) for school children for eyes, ears, nose, and throat were mandated nation-wide in 1951 by physical Assessment Act. 3. In 1979, the health instruction of schools to improve the health status of students was improved by health department in the Ministry of Education. 4. Experiences in healthful environment were basic components of the school health program. However, without careful planning and supervision these experiences were not contributed to the goal of school health. The formal program of school health environment were initiated in 1979. 5. In 1980, the guidelines of school health clinic management were prepared by Ministry of Education such as guidance of essential degrees and facilities in school health clinic. 6. Two patterns of administration of school health programs existed in Korea. In one the school health department operated its own health program and in the other the physical education department operated the health program within the school system. The school health department was established in Ministry of Education from 1979 to 1982. Improved school health programs will be a key element in the comprehensive national child health policy whic I will ask the Ministry of Education to develop for the Department.

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Continuous Renal Replacement Therapy in a 4-year-old Child with Rhabdomyolysis Following Parainfluenza Virus Infection and Hyperammonemia due to Isovaleric Acidemia (Parainfluenza virus 감염 후 발생한 횡문근융해증과 isovaleric acidemia로 인한 고암모니아혈증을 가진 소아에서의 지속적 신대체요법)

  • Park, Se Jin;Cho, Soo Yeon;Pai, Ki Soo;Shin, Jae Il
    • Childhood Kidney Diseases
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    • v.17 no.2
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    • pp.132-136
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    • 2013
  • Parainfluenza virus infection is one of the causes of fatal rhabdomyolysis. Rhabdomyolysis can be aggravated by mitochondrial fatty acid ${\beta}$-oxidation disorders during prolonged periods of fasting. Moreover, in patients with late-onset isovaleric acidemia, hyperammonemia may occur following catabolic stress. In the present report, we describe a case of a 4-year-old boy with parainfluenza virus infection and late-onset isovaleric acidemia that rapidly progressed to coma, seizures, and cardiorespiratory collapse. His serum ammonia and creatinine kinase (CK) levels were $385{\mu}Mol/L$ and 23,707 IU/L, respectively. Continuous renal replacement therapy (CRRT) was initiated using continuous venovenous hemodiafiltration, after which the ammonia and CK levels returned to normal. Thus, we recommend the immediate initiation of CRRT in the management of patients with life-threatening rhabdomyolysis and hyperammonemia.

Extended Septal Myectomy for Hypertrophic Obstructive Cardiomyopathy -Report of a case- (비후형 심근증 환아에서 시행한 광범위 중격절제술 - 1예 보고 -)

  • Lee Jae-Hang;Kwak Jae-Gun;Jung Eui-Suk;Oh Se-Jin;Chang Myoung-Woo;Kim Woong-Han
    • Journal of Chest Surgery
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    • v.39 no.10 s.267
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    • pp.775-778
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    • 2006
  • Hypertrophic cardiomyopathy is characterized by inappropriate hypertrophy of the myocardium and is associated with various clinical presentations ranging from complete absence of symptoms to sudden, unexpected death. These are caused by dynamic obstruction of the left ventricular outflow tract and surgical approaches were initiated. But, the complete resection of hypertrophied midventricular septum is impossible by standard, transaortic approach, because of narrow vision and limited approach. And it leads to inadequate excision, will leave residual left ventricular out-flow tract obstruction or systolic anterior motion of mitral leaflet, and limit symptomatic improvement and patient's survival. We report a case of extended septal myectomy for hypertrophic cardiomyopathy of mid-septum in a child. The extended septal myectomy was performed by aortotomy and left ventricular apical incision, and made possible the complete resection of mid-ventricular septum, abnormal papillary muscles and chordae. The patient's symptom was improved and the postoperative course was uneventful.

THE USE OF DISTRACTION OSTEOGENESIS TO TREAT HEMIFACIAL MICROSOMIA: A CASE REPORT (반안면 왜소증 환자에서의 골신장술: 증례보고)

  • Baik, Sung-Mun;Kim, Su-Gwan;Kim, Hak-Kyun;Moon, Seong-Yong
    • Journal of the Korean Association of Oral and Maxillofacial Surgeons
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    • v.33 no.5
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    • pp.559-566
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    • 2007
  • Distraction osteogenesis(DO) is a surgical method of bone formation that involves an osteotomy and sequential stretching of the healing callus by gradual movement and subsequent remodeling. DO is used to correct facial asymmetry, such as in patients with hemifacial microsomia, maxillary or mandibular retrusion, cleft lip and palate, alveolar defects, and craniofacial deficiency. It is accomplished with the aid of a distraction device, which is secured with screws placed directly into bone, for a predetermined length of time. Hemifacial microsomia is characterized by unilateral facial hypoplasia, often with unilateral shortening of the mandible and subsequent malocclusion. Patients with hemifacial microsomia and facial asymmetry have a vertically short maxilla, tilted occlusal plane, and short mandible. Early treatment is necessary to avoid subsequent impaired midfacial growth. The standard treatment of these malformations consists of the application of bone grafts, which can lead to unpredictable growth. The new bone-lengthening procedure represents a limited surgical intervention and opens up a new perspective for treatment, especially in younger children with severe deformities. This report describes a case of hemifacial microsomia(Type-II left-sided hemifacial microsomia). The patient, a 10-year-old child, visited our clinic for facial asymmetry correction. He had a hypoplastic mandible, displaced ear lobe, 10 mm canting on the right side, and malocclusion. We planned DO to lengthen the left mandible in conjunction with a Le Fort I osteotomy for decanting and then perform a right intraoral vertical ramus osteotomy(IVRO). Progressive distraction at a rate of 0.5 mm/12 hours was initiated 7 days postoperatively. The duration of DO was 17 days. The consolidation period was 3 months. Satisfactory results were obtained in our case, indicating that DO can be used successfully for functional, aesthetic reconstruction of the mandible. We report a case involving DO in conjunction with orthognathic surgery for correcting mandibular hypoplasia with a review of the literature.

Human Risk Assessment of Arsenic and Heavy Metal Contamination and Estimation of Remediation Concentration within Abandoned Metal Mine Area (폐금속 광산지역 비소 및 중금속 오염에 대한 인체위해성평가 및 복원농도 설정)

  • Lee, Sang-Woo;Kim, Jeong-Jin;Park, Mi Jeong;Lee, Sang-Hwan;Kim, Soon-Oh
    • Journal of the Mineralogical Society of Korea
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    • v.28 no.4
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    • pp.309-323
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    • 2015
  • This study was initiated to propose the method for human risk assessment suitable to metal mine area. Using a variety of exposure parameters extracted from the investigation of abandoned metal mines, the proposed method was applied to assess the risk of As and heavy metal contamination for inhabitants (male and female adults and child) within an abandoned mine area. Based on the results of risk assessment, in addition, target remediation concentrations of each media (soil, groundwater, and surface water) were estimated. The results indicate that total carcinogenic risk (TCR) and hazard index (HI) representing carcinogenic and non-carcinogenic risks, respectively, were calculated to exceed the tolerable levels (1.00E-6 and 1) with regard to two exposure pathways (groundwater and crop intakes) and As. Thus, the human risk of study area was evaluated to be significant. Based on the target risk (TR) for carcinogens, the remediation concentrations of soil were computed to be 6.83~6.85 mg/kg and 18.41~18.46 mg/kg for As and Pb, respectively. In terms of target hazard index (THI) for non-carcinogens, the remediation concentrations of soil were calculated to be 17.38 mg/kg for Cu and 9.13 mg/kg for As.

The Great Depression in High School Social Science Textbooks : Critiques and Suggestions (대공황에 대한 고등학교 사회과 교과서 서술의 문제점과 개선방안)

  • Kim, Duol
    • KDI Journal of Economic Policy
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    • v.30 no.1
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    • pp.171-209
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    • 2008
  • The Great Depression is one of the most important economic incidents in the twentieth century. A significant and long-lasting impact of this event is the rise of the government intervention to the economy. Under the catastrophic downturn of the economic condition worldwide, people required their government to play an active role for economic recovery, and this $mentalit{\acute{e}}$ prolonged even after the Second World War. Social science textbooks taught at Korean high schools mostly referred to the Great Depression for explaining the reason of government intervention in economy. However, the mainstream view commonly found in the textbooks provides a misleading theological interpretation. It argues that inherent flaws of the market economy causes over-production/under-consumption, and that this mismatch ends up with economic crisis. The chaotic situation was resolved by substitution of the governments for the market, and the New Deal was introduced as the monumental example ('laissez-faire economy ${\rightarrow}$over-production${\rightarrow}$the Great Depression${\rightarrow}$government intervention${\rightarrow}$economic recovery'). Based on economic historians' researches for past three decades, I argue that this mainstream view commits the fallacy of ex-post justification. Unlike what the mainstream view claims, the Great Depression was neither the result of the 'market failure', nor the recovery from the Great Depression but was due to successful government policies. For substantiating this claim, I suggest three points. First, blaming the weakness or instability of the market economy as the cause of the Great Depression is groundless. Unlike what the textbooks describe, the rise of the U.S. stock price during the 1920s cannot be said as a bubble, and there was no sign of under-consumption during the 1920s. On the contrary, a new consensus emerging from the 1980s among economic historians illustrates that the Great Depression was originated from 'the government failure' rather than from the 'market failure'. Policymakers of European countries tried to return to the gold standard regime before the First World War, but discrepancies between this policy and the reality made the world economy vulnerable. Second, the mainstream view identifies the New Deal as Keynesian interventionism and glorifies it for saving the U.S. economy from the crisis. However, this argument is not true. The New Deal was not Keynesian at all. What the U.S. government actually tried was not macroeconomic stabilization but price and quantity control. In addition, New Deal did not brought about economic recovery that people generally believe. Even after the New Deal, industrial production or employment level remained quite low until the late 1930s. Lastly, studies on individual New Deal policies show that they did not work as they were intended. For example, the National Industrial Recovery Act increased unemployment, and the Agricultural Adjustment Act expelled tenants from their land. Third, the mainstream view characterizes the economic order before the Great Depression as laissez-faire, and it tends to attribute all the vice during the Industrial Revolution era to the uncontrolled market economy. However, historical studies show that various economic and social problems of the Industrial Revolution period such as inequality problems, child labor, or environmental problems cannot be simply ascribed to the problems of the market economy. In conclusion, the remedy for all these problems in high school textbooks is not to use the Great Depression as an example showing the weakness of the market economy. The Great Depression should be introduced simply as a historical momentum that had initiated the growth of government intervention. This reform of high school textbooks is imperative for enhancing the right understanding of economy and history.