• Clinical and Experimental Pediatrics
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• v.51 no.12
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• pp.1342-1345
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• 2008

Megalencephalic leukoencephalopathy with subcortical cysts (MLC) is a rare white matter disorder, first described in the early 1990s. The brain in patients with MLC appears swollen on MRI, with diffuse white matter abnormalities; in addition, there is an invariable presence of subcortical cysts, primarily in the anterior temporal region sparing the deep white matter, basal ganglia, thalami, and cerebellum. Patients with MLC present with macrocephaly and neurological abnormalities such as motor deterioration, ataxia, spasticity, and cognitive deficits. We report a twenty-month-old boy who presented with seizures and macrocephaly, delay in development, and abnormal brain MRI findings compatible with the diagnosis of MLC. The brain MRI revealed bilateral hypersignal intense subcortical white matter regions in the frontal, temporal, and parietal lobes on T2-weighted images, which were not yet associated with cystic changes. During follow-up, the frequency of seizures decreased after anticonvulsant medication was started, but the head circumference remained above the 97th percentile, and the patient continued to have developmental delay.

• Proceedings of the Korean Institute Of Construction Engineering and Management
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• 2007.11a
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• pp.68-74
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• 2007

Critical Chain Project Management(CCPM) is a new project management system paradigm which maintains the advantages of PERT/CPM and improves the shortcomings of it. In CCPM the task durations are determined as 50% time estimates, ie average time discarding the their contingency. CCPM determines the critical chain the constraint of a projects considering the logical precedence relationship and resource conflict resolution. Project buffer is located at the end of critical chain to absorb the variations of critical chain. The size of project buffer is usually calculated as the half of the sum of critical chain length. Also feeding buffer is inserted after each non-critical chain which feeding into the critical chain to prevent the time delay of critical chain from uncertainties of non-critical chains. Resource buffer can be utilized to improve the availability of resources of critical chain. Buffer management is a project execution and control mechanism. Buffers are classified into 3 zones. They are OK zone, Watch and Plan zone and Expediting zone. If the project status is within Watch and Plan zone, contingency plan is established. And if it changes into Expediting zone, the preplanned contingency plan are executed to recover the time delay. In CCPM the workers are asked to work with relay runner work mechanism that they work fast if possible and report their completion to project manager for the succeeding task to start as soon as possible. The task durations are not considered as the promised time schedule. The multi-tasking is prohibited.

• Korean Journal of Health Psychology
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• v.19 no.4
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• pp.929-955
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• 2014

The purpose of this study was to 1) explore the effectiveness of a college-level self-management (SM) course in improving a target behavior and reducing impulsivity, 2) investigate improvements in other non-target self-control behaviors(generalization of self-control), and 3) determine whether change in impulsivity could predict the generalization of self-control. A total of 128 College students who took the SM course were included. Participants completed the computerized delay-discounting task, self-report impulsiveness scale, and general self-control behavior questionnaire at the beginning and end of the course. After participants had defined their target behaviors, they self-monitored and recorded their behaviors everyday throughout the course. Results revealed that 63% of the participants successfully changed their target behaviors after the course. Although differing depending on the type of target behavior, thoes who successfully changed their target behaviors reported decreases in impulsivity and increases in other self-control behaviors such as physical exercise, healthy diet, study habits, and time management, after the course. Furthermore, the decrease of impulsivity significantly predicted the generalization of self-control in the exercise and diet groups. The results indicate that reduced impulsivity is related to improvements in a target behavior and general self-control behaviors in other spheres. Implications and limitations are also discussed.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.13 no.2
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• pp.80-85
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• 2017

Angelman syndrome is a rare disorder caused by deletion or inactivation of genes on the maternally inherited chromosome 15. This neurodevelopmental disorder is characterized by developmental and intellectual delay, speech impairment, sleep disturbance, seizures, motor dysfunction, and frequent laughing or smiling. Orofacial characteristics include a prominent mandible, large mouth, prominent cheeks, a tendency to rest the tongue between the dental arches, excessive drooling, and excessive chewing behavior. Patients with this syndrome usually require general anesthesia even in a simple operation, because of risk of perioperative seizure during dental procedure. This is a case report about dental treatment of a 3-year-old female patient with Angelman syndrome under general anesthesia. This case suggests that the dental treatment under general anesthesia can be considered a safe component for the uncooperative, delayed developmental patients with underlying disease. Also, periodic dental exam appointment should be made to provide the patients with preventive treatments and to make them remain familiar with the dental environment.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.20 no.1
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• pp.29-35
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• 2020

Joubert syndrome (JS) is a rare genetic disorder that is characterized by ataxia, hypotonia, developmental delay, respiratory abnormalities such as apnea-hyperpnea, and abnormal eye movements. The pathognomonic diagnostic finding is the "molar tooth sign" (MTS) on brain magnetic resonance imaging (MRI), described as cerebellar vermis hypoplasia or dysplasia, thick and horizontally oriented superior cerebellar peduncles, and an abnormally deep interpeduncular fossa. JS is characterized by genetic heterogeneity: pathogenic variants in over 30 genes have been identified to date. The CEP290 protein, which is on chromosome 12q21.3, is most frequently mutated in patients with JS, especially with renal involvement. Here, we report a case of JS in a 14-year-old male patient with end-stage renal disease. To the best of our knowledge, this is the first Korean report of a patient with JS due to CEP290 mutation (c.6012-12T> A) whose diagnosis was confirmed after repetitive MRI. We suggest consultation with an experienced neuro-radiologist and follow-up MRI studies to detect a "hidden" MTS if clinical findings suggest a diagnosis of JS. Furthermore, even in the absence of an MTS, whole exome sequencing should be considered.

• Clinical and Experimental Pediatrics
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• v.52 no.2
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• pp.242-246
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• 2009

The clinical features of ring chromosome 13 include mental and growth retardation, CNS anomalies, facial dysmorphism, cardiac defects, genital malformations, limb anomalies, skeletal deformities and anal malformations. Although many cases of ring chromosome 13 have been reported worldwide, only 6 cases have been reported in Korea, and the latter cases were not mosaic but pure ring chromosome 13. Here we report a case with mosaic ring chromosome 13. The baby boy was born at 37 weeks of gestation by induced vaginal delivery due to intrauterine growth restriction (IUGR). He was the second baby of a 28-year-old hepatitis B carrier mother and a 32-year-old father. There was no family history of chromosomal anomalies. The baby was a symmetric IUGR with a birth weight of 1,860 g, length of 44.8 cm, and head circumference of 29.4 cm. The physical examination revealed microcephaly, trigonocephaly, flat occiput, large ears, short neck and dysmorphic facial features, including microophthalmia, hypertelorism, antimongoloid slanting palpebral fissures, a flat nasal bridge, and micrognathia. The karyotype of this patient performed by peripheral blood lymphocytes was 46,XY,r(13)(p13q34)/45,XY,-13/46,XY,dic r(13;13)(p13q34;p13q34). The baby showed failure to thrive, hypotonia, and developmental delay. We report the first case of mosaic ring chromosome 13 in a male baby in Korea and compare this case with other Korean cases of non-mosaic ring chromosome 13.

• Journal of the korean academy of Pediatric Dentistry
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• v.39 no.4
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• pp.412-417
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• 2012

Mucopolysaccharidosis (MPS) is a disorder which is caused by the defect of the lysosomal enzyme that is essentially needed for resolution of glycosaminoglycans (GAGs). Metabolite of GAGs will accumulate in the lysosome of cells and will result in the dysfunction of cells, tissues, and organs. Eventually, patients will manifest both mental retardation and physical disorders. In worst cases, mucopolysaccharidosis can cause premature death. The current clinical types have been classified as MPS from type I to type IX according to the defect of certain enzyme. The dental complications have been reported as delay of eruption, enamel hypoplasia, microdontia, malocclusion, condylar defects, gingival hyperplasia and dentigerous cystlike follicle. This clinical report presents the case of a boy with MPS type II, Hunter Syndrome which has various dental complications.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.15 no.1
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• pp.44-48
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• 2015

Mucopolysaccharidosis (MPS) IIIA is a lysosomal storage disorder caused by abnormalities of the enzyme Heparan N-sulfatase that is required for degradation of heparan sulfate. The patient in this study was a 5 year-old boy who presented with macrocephaly and developmental delay. Urinary excretion of glycosaminoglycan was increased (26 g/moL creatinine, reference range: <7 g/moL creatinine) and a distinct band of heparan sulfate was shown in electrophoresis. Heparan N-sulfatase activity was significantly decreased in skin fibroblasts (0.2 pmoL/min/mg protein, reference range: 9-64 pmoL/min/mg protein). PCR and direct sequencing analysis of the SGSH gene showed compound heterozygous mutations: c.1040C>T (p.S347F) and c.703G>A (p.D235N). This is the first report for a Korean patient with MPS IIIA who was confirmed by biochemical investigation and molecular genetic analyses.

• Journal of the Korea Academia-Industrial cooperation Society
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• v.17 no.4
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• pp.43-51
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• 2016

Preventing the forgery and alteration of test reports is important, in order to maintain the reliability of military supplies. Based on a survey of reports on testing institutions and a case study of established electronic document systems in the public sector, we propose a system architecture, which helps prevent the forgery and alteration of test reports with legal force. The proposed architecture takes advantage of both the time stamp that records the time a document is received and a server synchronized with the testing institution. Using the proposed system architecture, the user is able to request a test and receive reports from the testing institution without delay. Also, a defense agency is able to conveniently prove the authenticity of the test reports and utilize the statistical data collected by the architecture. Therefore, we expect that the proposed system architecture will help defense agencies to prevent the forgery and alteration of test reports and ensure their reliability and quality.

• Korean Journal of Construction Engineering and Management
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• v.19 no.5
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• pp.111-120
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• 2018

Recently, Domestic public construction projects are experiencing a great deal of disputes because of the growing uncertainty about the criteria for calculating the prolongation cost. In addition, researchers have been studying various systems and proper cost estimates in an effort to reduce the uncertainty of these systems and the occurrence of disputes. However, there is no standard yet for social consensus. Meanwhile, The study on the classification system according to the recognition standard of accounting has been systematically studied. As a result, the concepts of accrual and cash basis are defined separately. The purpose of this study is to verify the possibility of applying the concept of 'accrual basis' to the Standard for calculation of prolongation cost. Therefore, As a result of analyzing the occurrence pattern of Job-site overhead cost, it is confirmed that actual costs can not be calculated by the cash-basis method. In particular, the implications of the necessity of the accrual-basis method should be more strictly indicated in the case of items such as indirect labor costs and welfare benefits. In addition, the contractor 's claim report and the appraisal report were examined. As a result, it was confirmed that the calculation situations of prolongation costs are biased to the cash-basis method. In this way, it is suggested that necessary to supplement the calculation standard of the actual costs from the point of view of accrual basis.