• 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.

• Clinical and Experimental Pediatrics
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• v.51 no.4
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• pp.401-408
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• 2008

Purpose : Transcatheter closure of atrial septal defects (ASD) is currently established therapy as an alternative to surgery. But rarely, complications are reported in some studies. We report early and intermediate term complications associated with transcatheter closure of atrial septal defects using the Amplatzer septal occluder (ASO). Methods : From June 2003 to May 2006, 64 patients underwent transcatheter closure of secundum ASD or patent foramen ovale using the ASO. The ratio of male to female was 1:2.4, the median age was 17 years (range: 2.6-64 years) and their median weight was 47.5 kg (range: 2.6-64 kg). Results : The median diameter of ASD measured with transthoracic or transesophageal echocardiography was 15 mm (range: 6-28 mm), the median balloon stretched diameter was 18 mm (range: 6.5-34 mm), and the median size of device was 19.5 mm (range: 6-36 mm), was little difference with balloon stretched diameter. There were 10 cases of complications: arrhythmia (2), device malformation (2), aorta to right atrial fistula (1), hemolytic anemia (1), mitral valve encroachment (1), malposition (1), residual shunt (1), and inferior vena cava perforation (1). Conclusion : Transcatheter closure of ASD using ASO is effective and safe therapy. However, significant complications such as aorta to atrial fistula, atrial erosion, or device embolization can happen, so an appropriate selection of patient and device in relevance to size and anatomy of ASD is important for successful closure.

• Tuberculosis and Respiratory Diseases
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• v.54 no.3
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• pp.330-337
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• 2003

Background : Low spirometric forced vital capacity(FVC) in conjunction with a normal or high ratio of the forced expiratory volume at 1 second to the forced vital capacity($FEV_1$/FVC%) has traditionally been classified as a restrictive abnormality. However, the gold-standard diagnosis of a restrictive pulmonary impairment requires a measurement of the total lung capacity (TLC). This study was performed to determine the predictive value of spirometric measurements of the FVC for diagnosing a restrictive pulmonary abnormality. Methods : Test results from 1,371 adult patients who undertook both spirometry and lung volume measurements on the same visit from January 1999 to December 2000 were enrolled in this study. The test values for the FVC, the TLC that was below 80% of predicted value, and a $FEV_1$/FVC% that was below 70%, were classified as being abnormal. Results : Of the 1,371 patients, 353 patients had a reduced a FVC. Of these patients, 186 patients had a reduced TLC. Therefore, the positive predictive value was 52.7%. Of the 196 patients with a normal $FEV_1$/FVC% and a reduced FVC, 148(75.5%) patients had a lower TLC. Thirty eight (24.2%) patients out of 157 patients with a low $FEV_1$/FVC% and a low FVC showed a restrictive defect. Conclusion : Spirometry is useful to rule out a restrictive pulmonary abnormality, but a restrictive pattern on the spirometry dose not mean there is a true restrictive disease. For the patients with a low FVC, TLC measurements are essential for diagnosing a restrictive pulmonary impairment.

• Journal of Yeungnam Medical Science
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• v.16 no.2
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• pp.228-236
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• 1999

Background: Thyrotoxic periodic paralysis is an uncommon illness characterized by intermittent flaccid paralysis of skeletal muscle, usually accompanied by hypokalemia, in patient with hyperthyroidism. However, the pathophysiology of thyrotoxic periodic paralysis remains largely unexplained and controversial. This report describes the clinical and biochemical findings in 19 patients with thyrotoxic periodic paralysis who were examined at the Yeungnam University Medical Center(YUMC) during the past decade. Methods: The medical records of 997 YUMC patients, seen between 1986 and 1996, with diagnosis of hyperthyroidism were reviewed. Nineteen patients out of 997 hyperthyroidism patients were diagnosed, and examined by history, physical examination, serum electrolyte value, and thyroid function test during paralysis. On the basis of these results, comparisons were made on age, sex, precipitating factors, timing, affected limbs, prognosis, serum potassium and serum phosphate and thyroid hormone levels. Results: The prevalence of periodic paralysis in hyperthyroidism was 1.9 percent and the male to female prevalence ratio was 30:1 and in all patients, the development of perodic paralysis was correlated with hyperfunctional state of the thyroid gland. Eleven cases of periodic paralysis were associated with hypokalemia and their thyroid hormone levels were significantly more increased than those of the patients without hypokalemia. Interestingly, our study shows the recurrence of paralysis after treatment. Conclusion: Although the precise pathophysiology of the disease is as yet undefined and controversial, it occurs primarily in Asians with an overwhelming male preponderance and prevalence of 2 percent in hyperthyroidism. The interactive roles of thyroid hormone, Na-K pump, and genetically inherited defect in the cellular membrane potential of the skeletal muscle can be speculated. Further investigation will be needed to firmly establish the mechanism of thyrotoxic periodic paralysis.

• Childhood Kidney Diseases
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• v.4 no.2
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• pp.111-119
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• 2000

Purpose : Henoch-$Sch{\ddot{o}}nlein$ purpura(HSP) is a common pediatric discase presenting most frequently with skin, gastrointestinal, joint and renal manifestations. The prognosis of HSP is mainly determined by the involvement of the kidney, but prognostic markers have not been established. We evaluated the patients who have HSP nephritis with nephrotic syndrome. Method : Clinical manifestations and laboratory findings were observed and analyzed in 34 cases with HSP which were manifested by nephrotic syndrome hospitalized at Kyung Hee university Hospital during the period from Jan. 1990 to Dec. 1998. Results : 1) Male to female ratio was 1.3:1, and mean age at onset was 8.3 year. 2) Mean duration from symptom onset to renal biopsy was 10.5 weeks. 3) Proportion of patients presenting with acute nephritis was 32.4$\%$, gross hematuria 17.6$\%$, microscopic hematuria 50$\%$. 4) The findings of renal biopsy were 20 cases of grade II, 11 cases of grade III, 2 cases of grade I, 1 case of grade IV according to classification by ISKDC. 5) Patients with grade I were recovered with no residual defect, but patients with grade IV shows active renal disease(states C). Conclusion : Among the 디le patients with Henoch-$Sch{\ddot{o}}nlein$ purpura accompanying nephrotic syndrome, more aggressive treatment might be needed in patients showing crescents formation on renal biopsy. A prospective study will be needed to explore the progression of this disease.

• Clinical and Experimental Pediatrics
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• v.45 no.12
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• pp.1519-1527
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• 2002

Purpose : Although influenza virus is one of the most important causes of acute respiratory tract infections(ARTIs) in children, virus isolation is not popular and there are only a few clinical studies on influenza in Korea. We evaluated the epidemiologic and clinical features of ARTIs by influenza virus in children. Methods : From February 1995 to August 2001, nasopharyngeal aspirations were obtained and cultured for the isolation of influenza virus in children admitted with ARTIs. The medical records of patients with influenza virus infection were reviewed retrospectively. Results : Respiratory viruses were isolated in 997(22.0%) out of 4,533 patients examined, and influenza virus was isolated in 164 cases(3.6%). Influenza virus was isolated year after year mainly from December to April of next year. The ratio of male and female was 1.9 : 1 with a median age of 15 months. The most common clinical diagnosis of influenza virus infection was pneumonia, and fever and cough developed in most patients. There was no difference between influenza A and B infection in clinical diagnoses and symptoms. All patients recovered without receiving antiviral treatment except for one patient diagnosed with pneumonia who had underlying disease of Down syndrome with ventricular septal defect. Conclusion : ARTIs caused by influenza virus developed every winter and spring during the period of study. Because fatal complication can develop in the high risk group, prevention, early diagnosis and proper management of influenza should be emphasized.

• Journal of the Korean Institute of Traditional Landscape Architecture
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• v.36 no.3
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• pp.115-127
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• 2018

This study was attempted to find out the value of cultural assets through the clear diagnosis and prescription of the dead and weakness factors of the Population of Retusa Fringe Trees in Pyeongji-ri, Jinan(Natural Monument No. 214), The results are as follows. First, Since the designation of 13 natural monuments in 1968, since 1973, many years have passed since then. In particular, despite the removal of some of the buried soil during the maintenance process, such as retreating from the fence of the primary school after 2010, Second, The first and third surviving tree of the designated trees also have many branches that are dead, the leaves are dull, and the amount of leaves is small. vitality of tree is 'extremely bad', and the first branch has already been faded by a large number of branches, and the amount of leaves is considerably low this year, so that only two flowers are bloomed. The second is also in a 'bad'state, with small leaves, low leaf density, and deformed water. The largest number 1 in the world is added to the concern that the s coverd oil is assumed to be paddy soils. Third, It is found that the composition ratio of silt is high because it is known as '[silty loam(SiL)]'. In addition, the pH of the northern soil at pH 1 was 6.6, which was significantly different from that of the other soil. In addition, the organic matter content was higher than the appropriate range, which is considered to reflect the result of continuous application for protection management. Fourth, It is considered that the root cause of failure and growth of Jinan pyeongji-ri Population of Retusa Fringe Trees group is chronic syndrome of serious menstrual deterioration due to covered soil. This can also be attributed to the newly planted succession and to some of the deaths. Fifthly, It is urgent to gradually remove the subsoil part, which is estimated to be the cause of the initial damage. Above all, it is almost impossible to remove the coverd soil after grasping the details of the soil, such as clayey soil, which is buried in the rootstock. After removal of the coverd soil, a pestle is installed to improve the respiration of the roots and the ground with Masato. And the dead 4th dead wood and the 5th and 6th dead wood are the best, and the lower layer vegetation is mown. The viable neck should be removed from the upper surface, and the bark defect should undergo surgery and induce the development of blindness by vestibule below the growth point. Sixth, The underground roots should be identified to prepare a method to improve the decompression of the root and the respiration of the soil. It is induced by the shortening of rotten roots by tracing the first half of the rootstock to induce the generation of new roots. Seventh, We try mulching to suppress weed occurrence, trampling pressure, and soil moisturizing effect. In addition, consideration should be given to the fertilization of the foliar fertilizer, the injection of the nutrients, and the soil management of the inorganic fertilizer for the continuous nutrition supply. Future monitoring and forecasting plans should be developed to check for changes continuously.

• Tuberculosis and Respiratory Diseases
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• v.42 no.4
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• pp.555-568
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• 1995

Background: It is most physiologic to measure the diffusing capacity of the lung by using oxygen, but it is so difficult to measure partial pressure of oxygen in the capillary blood of the lung that in clinical practice it is measured by using carbon monoxide, and single-breath diffusing capacity method is used most widely. However, since the process of withholding the breath for 10 seconds after inspiration to the total lung capacity is very hard to practice for patients who suffer from cough, dyspnea, etc, the intra-breath lung diffusing capacity method which requires a single exhalation of low-flow rate without such process was devised. In this study, we want to know whether or not there is any significant difference in the diffusing capacity of the lung measured by the single-breath and intra-breath methods, and if any, which factors have any influence. Methods: We chose randomly 73 persons without regarding specific disease, and after conducting 3 times the flow-volume curve test, we selected forced vital capacity(FVC), percent of predicted forced vital capacity, forced expiratory volume within 1 second($FEV_1$), percent of forced expiratory volume within 1 second, the ratio of forced expiratory volume within 1 second against forced vital capacity($FEV_1$/FVC) in test which the sum of FVC and $FEV_1$ is biggest. We measured the diffusing capacity of the lung 3 times in each of the single-breath and intra-breath methods at intervals of 5 minutes, and we evaluated which factors have any influence on the difference of the diffusing capacity of the lung between two methods[the mean values(ml/min/mmHg) of difference between two diffusing capacity measured by two methods] by means of the linear regression method, and obtained the following results: Results: 1) Intra-test reproducibility in the single-breath and intra-breath methods was excellent. 2) There was in general a good correlation between the diffusing capacity of the lung measured by a single-breath method and that measured by the intra-breath method, but there was a significant difference between values measured by both methods($1.01{\pm}0.35ml/min/mmHg$, p<0.01) 3) The difference between the diffusing capacity of the lung measured by both methods was not correlated to FVC, but was correlated to $FEV_1$, percent of $FEV_1$, $FEV_1$/FVC and the gradient of methane concentration which is an indicator of distribution of ventilation, and it was found as a result of the multiple regression test, that the effect of $FEV_1$/FVC was most strong(r=-0.4725, p<0.01) 4) In a graphic view of the difference of diffusing capacity measured by single-breath and intra-breath method and $FEV_1$/FVC, it was found that the former was divided into two groups in section where $FEV_1$/FVC is 50~60%, and that there was no significant difference between two methods in the section where $FEV_1$/FVC is equal or more than 60% ($0.05{\pm}0.24ml/min/mmHg$, p>0.1), but there was significant difference in the section, less than 60%($-4.5{\pm}0.34ml/min/mmHg$, p<0.01). 5. The diffusing capacity of the lung measured by the single-breath and intra-breath method was the same in value($24.3{\pm}0.68ml/min/mmHg$) within the normal range(2%/L) of the methane gas gradient, and there was no difference depending on the measuring method, but if the methane concentration gradients exceed 2%/L, the diffusing capacity of the lung measured by single-breath method became $15.0{\pm}0.44ml/min/mmHg$, and that measured by intra-breath method, $11.9{\pm}0.51ml/min/mmHg$, and there was a significant difference between them(p<0.01). Conclusion: Therefore, in case where $FEV_1$/FVC was less than 60%, the diffusing capacity of the lung measured by intra-breath method represented significantly lower value than that by single-breath method, and it was presumed to be caused largely by a defect of ventilation-distribution, but the possibility could not be excluded that the diffusing capacity of the lung might be overestimated in the single-breath method, or the actual reduction of the diffusing capacity of the lung appeared more sensitively in the intra-breath method.