• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.4 no.1
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• pp.1-9
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• 2001

Purpose: Gastroesophgeal reflux (GER) is defined as involuntary movement of gastric contents into esophagus. Relaxation of lower esophageal sphincter caused by immature anatomical development in newborn and young infants produces GER frequently. We wanted to know whether the frequency of GER is influenced by feeding types and position or not. We studied in 16 subjects according to feeding types (breast feeding group: BFG-7, formula feeding group: FFG-9) who admitted to the Soonchunhyang university hospital for recurrent regurgitation with 24 hr esophageal pH monitoring from August 1996 to July 1999. Methods: We compared two groups by number of reflux episode, reflux rate, longest episode, numbers of episodes lasting >5 minutes, longest episode in upright position and longest episode in supine position. We used Mann-Whitney test for statistical analysis. Results: 1) The subjects were 7 infants in BFG and 9 infants in FFG, 16 in total, and mean age was 2.1, and 2.6 months for BFG and FFG, respectively. 2) The reflux numbers were $244{\pm}151/day$, $275{\pm}155/day$ for BFG and FFG, respectively. 3) The reflux rate was $14{\pm}15%$ for BFG and $28{\pm}22%$ for FFG. It was lower in BFG. 4) The longest episode was $20{\pm}28$ minutes for BFG and $58{\pm}66$ minutes for FFG. It was significantly longer in FFG. 5) The numbers of episodes lasting >5 minutes were $5{\pm}6$ for BFG and $9{\pm}3$ for FFG. 6) The longest episode in upright position was $10{\pm}8$ minutes for BFG and $40{\pm}47$ minutes for FFG. It was significantly shorter in BFG. 7) The longest episode in supine position was $18{\pm}29$ minutes for BFG and $52{\pm}66$ minutes for FFG. It was significantly shorter in BFG. Conclusion: Breast feeding is strongly recommended to reduce the regurgitation in infancy. It is an another benefit of breast feeding.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.17 no.2
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• pp.55-62
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• 2017

Gaucher disease (GD) is an autosomal recessive lysosomal storage disorder caused by beta-glucosidase deficiency. An 18 month-old male with hepatosplenomegaly, anemia, thrombocytopenia, and growth retardation referred to our hospital. The patient showed neurological symptoms, such as supranuclear gaze palsy and developmental delay. Bone marrow biopsy performed to rule out malignancy and the results revealed no malignant cell; however, abnormal histiocytes suggesting storage disease was noted. Based on hepatosplenomegaly, bicytopenia and unexplained neurologic manifestations, enzyme activity and genetic analysis were conducted emergently with a strong suspicion of GD. Beta-glucosidase activity in leukocyte was decreased. GBA sequencing to confirm the diagnosis revealed compound heterozygous pathogenic variants (i.e., c.754T>A, c.887G>A), both previously reported as the cause of neuronopathic GD. Under the diagnosis of type 3 GD, the patient immediately received enzyme replacement therapy (ERT). After 17 months of ERT, the size of spleen decreased, and hemoglobin and platelet count returned to normal. In addition, the activity of chitotriosidase and angiotensin converting enzyme decreased. However, myoclonic movement and generalized seizure occurred at the age of 19 months and antiepileptic drug was started. Other neurological deterioration including supranuclear gaze palsy and developmental delay also persisted. A new therapy to overcome neurologic problems should be developed for patients with type 3 GD.

• Journal of the korean academy of Pediatric Dentistry
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• v.35 no.4
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• pp.766-770
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• 2008

Gingival fibromatosis is a rare condition characterized by varying degrees of gingival hyperplasia. Gingival fibromatosis usually occurs as an isolated disorder or can be associated with a variety of other syndromes. It usually appears at the time of eruption of permanent dentition but, can develop at the time of eruption of the primary dentition and rarely at birth. It may deform palatal contour and subsequently restrict the tongue movement, resulting in interference during speech and mastication. In addition, it incapacitates maintenance of normal lip closure. A 14-month-old girl visited the department of pediatric dentistry, Yonsei University Dental Hospital, for the congenital gingival overgrowth. There was no one in the family, who showed similar pattern of gingival growth. The intraoral clinical examination revealed generalized severe gingival enlargement throughout the maxillary and the mandibular arches. Enlarged gingival tissue was pink and had firm consistency. She was referred for chromosomal analysis, which confirmed absence of any known syndrome. Under local anesthesia, "Punch-biopsy" was performed on the labial area, and the specimen was histologically diagnosed as gingival fibromatosis. For she did not have any medical problem nor familiar history, she was diagnosed as having idiopathic gingival fibromatosis. Regarding her age and behavior, close follow-up was decided.

• Journal of radiological science and technology
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• v.39 no.3
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• pp.361-368
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• 2016

As the tertiary industry is being developed, many problems are introduced in an industrial area in Korea. This study aims to investigate the residents' health conditions, such as the prevalence of metabolic syndrome and fatty liver diagnosed by ultrasonography, comparing the urban with the industrial areas. The subjects were 378 adults in the urban area and 376 adults in the industrial area. Subject variables accounted for were gender, age, drinking habits, smoking habits, physical activities, waist circumferences and BMI. In blood tests, fasting blood glucose, triglyceride, HDL, blood pressures, total cholesterol, AST, ALT and GGT were examined. Also, the presence and absence of fatty liver, diagnosed using ultrasonography, was investigated. As a result, the prevalence of metabolic syndrome were 43.7% in the urban area and 51.6% in the industrial area, hence it was significantly higher in the industrial area than in the urban area. Fasting blood glucose, triglyceride and waist circumference in the distribution of the individual components were 47.4, 53.7, and 16.1% in the urban area and 54.5, 61.5, and 22.9% in the industrial area respectively, so it was substantially higher in the industrial area than in the urban area. On the other hand, HDL, diastolic blood pressure, and fatty liver were 25.7, 43.4, and 44.7% in the urban area and 21.3, 19.9, and 36.7% in the industrial area respectively and thus it was much higher in the urban area than in the industrial area. In conclusion, this study shows that there were significant differences between the residents' health condition, comparing the urban area to the industrial area. Therefore, the precaution and aggressive management of metabolic syndrome may be necessary, in accordance with the characteristics of the industrial zone.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.12 no.2
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• pp.108-112
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• 2012

Glycogen storage disease type III (GSD type III, OMIM #232400) is a rare autosomal recessive disease caused by a deficiency of the glycogen-debranching enzyme (GDE) with a mutation in the AGL gene (OMIM *610860). It is known to be bifunctional enzyme, that is, having two independent catalytic activities; 1,4-${\alpha}$-D-glucan 4-${\alpha}$-D-glycosyltransferase (EC 2.4.1.25) and amylo-1,6-glucosidase (EC 3.2.1.33) that occur at separate active sites on a single polypeptide chain. Most patients with GSD type III usually have symptoms related to decreased glycogenolysis in liver and muscles, such as hepatomegaly, hypoglycemia, failure to thrive, hyperlipidemia, muscle weakness and cardiomyopathy (type IIIa), however some patients show symptoms restricted to liver (type IIIb). GSD type III is diagnosed by enzyme test through liver or muscle biopsy or mutation analysis of the AGL gene. We report the case of GSD type III proven by gene study after liver biopsy, which revealed c.476delA, c.3444_3445insA in exon 6, 27 of AGL gene in Korean patient.

• Journal of Yeungnam Medical Science
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• v.2 no.1
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• pp.191-202
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• 1985

As the result of a significant improvement in the economic situation and development of scientific techniques in Korea during the last 20 years, the life expectancy of the Korean people has lengthened considerably and as a result the number of old aged people has markedly increased. Such an increase of the number of aged people brought about many problems. Authors would like to take a medical look at the "Problem of old aged people" presently facing us in Korea. Currently the number of people over 65 has increased rapidly and is 1,620,000, 4% of total population. But it is still much lower than 8.9% in Japan, 10.7% in U.S.A., and 14.9% in the United Kingdom. Over 25% of these aged people were found to have at least more than one disease which requires medical care. Diseases occur in the circulatory system, 30.9%, respiratory system, 17.1%, digestive system 8.6%, mental disorders, 8.4%, malignant neoplasms, 7.0%. About 51% of the aged over 65 are under medical security benefit, mostly with partial coverage plan. Their clinic visit rate was very low (2.0% in 1981), which might be due to financial reasons. Since diseases affecting the aged progress chronically, early detection and long term care are utter most important. However there is almost no special facility, long term care center or geriatric specialist. For proper management of medical problems in the growing population of the Korean eldery expansion of medical security coverage, greater number of specialized facilities, education of geriatric special manpower and efficient operating system should be established.

• Journal of the korean academy of Pediatric Dentistry
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• v.46 no.4
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• pp.343-352
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• 2019

Abnormal orofacial functions such as lip incompetency in the period of growth and development can cause morphological anomalies of the craniofacial complex. Therefore, it is crucial to make an early diagnosis based on the evaluation of the myofunctional conditions, and to make appropriate treatment plans. The objectives of this study were to quantitatively evaluate the standard lip closing force (LCF) of each age in the elementary school children, and to evaluate the relationships between LCF and affecting factors. The sample consisted of 765 children who were 7 - 12 years old in Jeonju city. Clinical examination about occlusal conditions and lip competency, and LCF measurement were performed by a single examiner. LCF was measured three times for each children with the LCF measuring device. The LCF was correlated positively with age in both sexes. The distribution of LCF groups was correlated significantly with Angle's classes and lip competency (p = 0.016, 0.004). The proportion of children with high LCFs was greater in the "competent lip" group, whereas the proportion of those with low LCFs was greater in the "incompetent lip" group.

• Food Science of Animal Resources
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• v.26 no.4
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• pp.418-425
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• 2006

This study was conducted to evaluate extraction properties of crude saponin and ginsenosides, and their effects on sensory properties of emulsified pork sausage. Non-dried ginseng root was boiled in 0 (e.g., 100% distilled water), 20, 40, 60, 80 or 100% ethanol, and powdered by a freezing dry method. Weight of dried powder for the 0% ethanol extraction was 20% of initial non-dried ginseng weight, while $20{\sim}80%$ and 100% ethanol extractions resulted in approximately 15 and 10% of their initial weights, respectively. On the other hand, crude saponin content in the dried powder was linearly increased for a higher ethanol content where 100% ethanol extraction resulted in 123.52 mg/g. LC/MS analysis of crude saponin for quantifying ginsenosides showed that Rb1, Rb2 and Rc were significantly (p<0.05) higher levels for both 80 and 100% ethanol extractions. In the case of Rg1 ginsenoside, 60, 80 and 100% ethanol extractions resulted in significantly (p<0.05) higher levels. Emulsified pork sausages containing 0, 1 or 2% ginseng extracts were smoked or non-smoked and their sensory characteristics and preference were evaluated. Smoking process significantly (p<0.05) decreased juiciness and tenderness, but the treatment significantly (p<0.05) improved flavor and consumer preference. It was particularly noticed that a 2% addition of ginseng extract prevented the adverse effects of smoking process on juiciness and tenderness while the 2% addition significantly (p<0.05) improved consumer preference. The current results implied that addition of ginseng extract in emulsified pork sausage could improve sensory quality.

• Clinical and Experimental Pediatrics
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• v.45 no.10
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• pp.1263-1272
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• 2002

Purpose : Rett syndrome(RTT) is an X-linked dominant neurodevelopmental disorder affecting 1 per 10,000-15,000 female births worldwide. It was initially described by Andreas Rett in 1966. RTT involves developmental regression characterized stereotypic hand movements, tremors, gait apraxia, seizures, deceleration of head growth after the age of 6-18 months. The disease-causing gene was identified as MECP2 on chromosome Xq28. We carried out mutational analysis of MECP2 genes in RTT patients. Methods : Whole blood(5 cc) of 34 sporadic RTT patients was collected in EDTA-anticoagulated tubes. Genomic DNA was extracted from peripheral blood using the E.Z.N.A. blood DNA kit. Four exons of the MECP2 gene were amplified by PCR in 34 Korean with RTT. We carried out PCR divided the exon three into two parts and the exon four into five parts. Primer sequences designed by Amir et al. in 1999 were almost used(AF030876). Sequencing primers used were the same as PCR. DNA sequencing reactions were performed using an ABI 377 DNA sequencer and ABI PRISM dye terminator cycle sequencing reaction kit(Perkin-elmer). The results were compared with the normal DNA sequence(X99686). To confirm the change of sequence on novel mutations, RFLP analysis was performed. Results : The MECP2 mutations were detected in 23(67.6%) of the 34 patients. The mutations consisted of 12 different types including nine missense and three nonsense mutations. Of these, three (L100V, G161E and T311M) mutations were newly identified. Most of the mutations discovered are located within MBD(39.1%) and TRD(39.1%). In this study, three(T158M, R270X, R306C) mutations were identified high frequency. Conclusion : MECP2 gene was also an important cause of Korean RTT patients. MECP2 gene study is an important tool for diagnosis of Korean RTT patients.

• Journal of the Korean Academy of Child and Adolescent Psychiatry
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• v.14 no.1
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• pp.12-25
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• 2003

Objectives：As increasing number of new antidepressants have been being introduced in clinical practice, pharmacological understanding has been broadened. These changes mandate new information and theories to be incorporated into the treatment process of children with depressive disorders. In light of newly coming knowledge, this review intended to recapitulate the characteristics of new antidepressants and to consider the pivotal issues to develope guidelines for the treatment of depression in childhood and adolescence. Methods：Searching the Pub-Med online database for the articles with the key words of 'new', 'antidepressants' and 'children' ninety-seven headings of review articles were obtained. The author selected the articles of pertinent subjects in terms of either treatment guideline or psychopharmacology of new antidepressants. When required, articles about the clinical effectiveness of individual antidepressants were separatedly searched. In addition, the safety information of new antidepressants was acquired by browsing the official sites of the United States Food and Drugs Administration and Department of Health and Human Services. Results：1) For the clinical course, treatment phase, and treatment outcome, the reviews or treatment guidelines adopted the information from adult treatment guidelines. 2) Systematic and critical reviews unambiguously concluded that selective serotonin reuptake inhibitors(SSRIs) excelled tricyclic antidepressants( TCAs) for both efficacy and side effect profiles, and were recommend for the first-line choice for the treatment of children with depressive disorders. 3) New antidepressants generally lacked treatment experiences and randomized controlled clinical trials. 4) SSRIs and other new antidepressants, when used together, might result in pharmacokinetic and/or pharmacodynamic drug-to-drug interaction. 5) The difference of the clinical effectiveness of antidepressants between children and adults should be addressed from developmental aspects, which required further evidence. Conclusion：Treatment guidelines for the pharmacological treatment of childhood and adolescence depression could be constructed on the basis of clinical trial findings and practical experiences. Treatment guidelines are to best serve as the frame of reference for a clinician to make reasonable decisions for a particular therapeutic situation. In order to fulfill this role, guidelines should be updated as soon as new research data become available.