• Clinical and Experimental Pediatrics
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• v.51 no.6
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• pp.650-654
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• 2008

Glycogen storage disease (GSD) and mucopolysaccharidosis (MPS) are both independently inherited disorders. GSD is a member of a group of genetic disorders involving enzymes responsible for the synthesis and degradation of glycogen. GSD leads to abnormal tissue concentrations of glycogen, primarily in the liver, muscle, or both. MPS is a member of a group of inherited lysosomal storage diseases, which result from a deficiency in specific enzymatic activities and the accumulation of partially degraded acid mucopolysaccharides. A case of a 16-month-old boy who presented with hepatomegaly is reported. The liver was four finger-breadth-palpable. A laboratory study showed slightly increased serum AST and ALT levels. The liver biopsy showed microscopic features compatible with GSD. The liver glycogen content was 9.3% which was increased in comparison with the reference limit, but the glucose-6-phosphatase activity was within the normal limit. These findings suggested GSD other than type I. Bony abnormalities on skeletal radiographs, including an anterior beak and hook-shaped vertebrae, were seen. The mucopolysaccharide concentration in the urine was increased and the plasma iduronate sulfatase activity was low, which fulfilled the diagnosis criteria for Hunter syndrome (MPS type II). To the best of the authors' knowledge, this is the first case of GSD and Hunter syndrome being identified at the same time.

• Journal of radiological science and technology
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• v.31 no.2
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• pp.115-121
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• 2008

Purpose: Aortic Dissection is very dangerous, prognostic disease, which the bloodstream flow out of the true lumen of the aorta by the bursting of aortic intima resulting in a rapid dissociation of inner and outer layer from the media. It is difficult to diagnose aortic dissection clinically by normal X-ray. This study was to investigate the occurrence frequency by age and number of patients who are identified to be aortic dissection by CT (Computed Tomography) scan. Materials and methods: We investigated the trend of yearly fluctuation, gender, age, and department of clinical research of the 112 patients who conducted CT scan in C- University Hospital for two years from January 2005 to December 2006. The MIP and SSD which reconstructed CT image and the VRT image were obtained for the accurate observation. The result was investigated by comparing normal X-ray and CT scan. Results and Conclusion: 1. The yearly check of 112 patients conducted CT scan showed 37 people (41.9%) in 2005, and it was increased to 65 (58.1%) in 2006 by 1.4 times. 2. The gender distribution of patients given a CT scan showed 45 males (40.1%), and female 67 (59.9 %). The aortic dissection patients were 9 (20%) out of 45 males, 21 (31.3%) out of 67 females and women were 1.6 times more than men. Women are also 1.5 times more than men in the number of examinee. 3. The age distribution of patient's who conducted CT scan revealed that there was no patient under 30 years old while 88.3% of all patients were through 41 to 80 years old. The higher the age was, the higher the occurrence of aortic dissection was. The difference in the occurrence frequency of age was statistically significant (p<0.01). 4. The departments that requested CT scan were the emergency department 46 (41.1%), circulatory internal medicine 37 (33.0%), chest surgery 13 (11.6%), and others 6 (14.3%). The combined ratio of emergency medicine and circulatory internal medicine was 74.1% of all. The results show that the aortic dissection is a very dangerous disease whose patients visit mainly via the emergency room. 5. The aortic dissection patients had normal X-ray readings in 22 (73.3%) out of 30, and only 8 (26.7 percent) are abnormal in the X-ray diagnosis. Therefore, the CT scan needs to be enforced in order to assess accurately the disease of aortic dissection.

• Journal of radiological science and technology
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• v.31 no.2
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• pp.149-159
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• 2008

The purposes of this study are to analyze abnormal dilatation of the extrahepatic bile ducts by using transabdominal ultrasound, to confirm the existence of bile ducts diseases and their interrelationship, and for it to give a new theoretical basis for the technical access to extrahepatic bile ducts, upon which to analyze the ripple effects of the scan training. After teaching technical access process based on the new theory about extrahepatic bile duct to the thirty students who are studying ultrasonography, we allocated three hours per one student (30 mins ${\times}$ 6 times) to focus on the training of scanning skill. Training has been performed by one-to-one method. For evaluation, all the students have to perform the scans on (1) confluence of the right and left hepatic ducts (extrahepatic bile ducts and cystic duct), (2) the suprapancreatic bile duct, (3) the intrapancreatic bile duct, (4) intrapapilla Duct, based on the clearly divided concept. The existing training and methods have had low confidency about transabdominal ultrasonography of the extrahepatic bile duct and had limitation with which they could image only the suprapancreatic bile duct. The evaluation after finishing the train based on the new theory, however, all the students (30students) can access to (1) confluence of the right and left hepatic ducts(extrahepatic bile ducts and cystic duct), (2) the suprapancreatic bile duct objectively. 24 students can access to (3) the intrapancreatic bile duct and only one student can even make an image for (4) the intrapapilla Duct Though the evaluation on extrahepatic bile duct has to be performed with multi-sided method considering intrahepatic cause, bile duct cause and pathophysiological cause, only if we can image the extrahepatic bile duct to ampular of Vater objectively and confidently, we can greatly reduce invasive procedure such as ERCP, which is for the purpose of simple differential diagnosis and painful to the patients. Therefore if we concentrate on the scanning train based on the new theory to raise the confidency about ultrasonography, the effect will be doubled.

• Journal of Veterinary Clinics
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• v.20 no.2
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• pp.237-241
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• 2003

A case of juvenile onset generalized demodicosis of one year old, intact female Japanese Chin dog weighing 3.1kg was presented to the Small Animal Clinic of the College of Veterinary Medicine of Kyungpook National University. The diagnosis was made based on the physical examination, deep skin scrapings, hematology, serum chemistry, endocrinologic evaluation and bacterial culture. Numerous D. canis mites of various stages were observed in multiple skin scraping samples. CBC, serum chemistry, $T_3$, $T_4$ and free $T_4$ values were within normal range and Staphylococcus intermedius was isolated in bacterial culture of pustules. The dog was healthy other than skin lesions at the presentation. The three different treatment protocols were tried for the case. No clinical improvement was observed during 13 weeks of orally given daily basis ivermectin therapy at 600ug/kg and 2 weeks of spot-on weekly basis selamectin therapy at 30 mg/kg with cephalexin given orally twice a day at 25 mg/kg. However, a remarkable remission was seen by 0.1% amitraz dip on weekly basis with 4% chlorhexidine bath given twice a week. The secondary staphylococcal infection and accompanied pruritus was almost disappeared in two weeks and she was recovered clinically normal in 9 weeks of therapy. The therapy was continued for 4 more weeks to prevent relapse. The dog is clinically normal and being monitored for development of any abnormal dermatological signs for the time being.

• Journal of the korean academy of Pediatric Dentistry
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• v.35 no.4
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• pp.750-756
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• 2008

Supernumerary teeth are frequently found in the anterior portion of the maxilla and develop as a result of abnormal proliferation of the dental lamina during tooth germ formation, caused by genetic or environmental factors. They may result in various complications, such as eruption interference, displacement, rotation of adjacent teeth, diastema, eruption into the nasal cavity, and development of dentigerous cyst. The optimal time for surgical extraction of supernumerary teeth has been a controversial issue. Someone prefer early surgical extraction because supernumerary teeth can cause eruption interference and displacement of adjacent teeth, eventually altering occlusion. Others prefer to delay surgical extraction until $8{\sim}10$ years of age in consideration of root maturation of the adjacent teeth and also patient's behavior. When surgical extraction of supernumerary teeth is postponed, there is possibility that impacted supernumerary teeth in the inverted or horizontal position move toward the nasal cavity, hard palate, or premolar area. When such intraosseous tooth migration is combined with the vertical growth of the maxilla, surgical approach becomes even harder. Therefore, possibility of intraosseous tooth migration should be considered as an important factor when deciding appropriate time for surgical extraction. We are presenting cases of mesiodens which showed intraosseous migration during $6{\sim}7$ years of follow-up period since the first diagnosis had been made at the $2{\sim}3$ years of age.

• 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.

• Tuberculosis and Respiratory Diseases
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• v.59 no.3
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• pp.298-305
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• 2005

Background : Obstructive sleep apnea syndrome (OSAS) is believed to have multifactorial causes. The major risk factors for OSAS are obesity, narrowed upper airways, and abnormal cranial-facial structures. A genetic basis for OSAS has been also suggested by reports of families with many members affected. This study analyzed the HLA typing in patients with OSAS to determine the possible role of genetics in OSAS. Methods : Twenty-five Korean patients with OSAS (1 woman and 24 men; age range 30-66 years) were enrolled in this study. A diagnosis of OSAS was made using full-night polysomnography. The control group consisted of 200 healthy Korean people. Serologic typing of the HLA-A and B alleles was performed in all patients using a standard lymphocyte microcytotoxicity test. Analysis of the polymorphic second exons of the HLA-DRB1 gene was performed using a polymerase chain reaction-sequence specific oligonucleotide probe. Results : The allele frequency of HLA-A11 was significantly lower in patients with OSAS compared with the controls (p<0.05). The HLA-B allele frequencies in the patients and controls had a similar distribution. Analysis of the HLADRB1 gene polymorphisms showed an increased frequency of DRB1*09 in the OSA patients compared with the controls (p<0.05). When the analysis was performed after dividing the OSAS patients according to the severity of apnea, the allele frequency of HLA-DRB1*08 was significantly higher in the severe OSA patients (apnea index >45) than in the controls (p<0.05). Conclusion : This study revealed an association between OSAS and the HLA-A11 and DRB1*09 alleles as well as association between the disease severity and the HLA-DRB1*08 allele in Korean patients. These results suggest that genetics plays an important role in both the development and the disease severity of OSAS.

• Journal of Yeungnam Medical Science
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• v.17 no.2
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• pp.146-154
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• 2000

Background and Purpose: Radioopaque lesions are commonly seen in patients who received thoracic radiotherapy for various kinds of thoracic neoplasm, But therir exact diagnos are sometimes uncertain. Patients and Methods: We examined simple chest radiograph and computed tomogram(CT) of 69 patients who received thoracic radiotherapy for lung cancer and were follow up at least 6 months in Yeungnam University Medical Center. Results: Of the 69 patients. thirty-eight patients showed radioopaque lesions in their chest radiographs except radiation fibrosis; radiation pneumonitis was witnessed in 24 patients. infectious pneumonia in 8 patients, and recurrence in 6 patients. In radiation pneumonitis patients, the pneumonitis occurred usually between 50 to 130 days after receiving radiation therapy, and interval between pneumonitis and fibrosis is 21 to 104 days. Simple chest radiographs of radiation pneumonitis(24 patients) represented ground glass opacities or consolidation in 4 cases(type I, 17%), reticular of reticulonodular opacities in 10 cases(type II, 42%), irregular patchy consolidations in 2 cases( type III, 8%), and consolidation with fibrosis in 8 cases(type IV, 33%), CT represent ground glass opacities or consolidation in 5 cases(type I, 29%), irregular nodular opacities in 3 cases(type II, 19%), irregular opacity beyond radiation fields in 3 cases(type III, 18%), and consolidation with fibrosis in 6 cased(type IV, 35%). The CT of four patients who represented type II on simple chest radiographs reveal type I and III, and CT of two patients with clinical symptoms who had no abnormal finding on simple radiograph revealed type I. Conclusions: In conclusion, computed tomogram is superior to the simple radiograph when trying to understand the pathologic process of radiation pneumonitis and provide confidence in the diagnosis of radiation induced lung disease.

• Pediatric Infection and Vaccine
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• v.5 no.2
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• pp.221-229
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• 1998

Purpose : The symptom of urinary tract infection in children is non-specific and systemic compared to that of adults. So the diagnosis of UTI in children can be delayed. If UTI in children is not appropriately managed, it may he resulted in renal failure, hypertension, growth failure in adolescence because there are already documented renal scar or urinary tract abnormality in infection period. Especially upper UTI that involve renal parenchyme may be result in fatal complication. The purpose of this study is analyzing the relationship between acute reactive marker and $^{99m}TC$-DMSA renal scan in upper urinary tract infection. Methods : This study included 56 children admitted at Dankook University Hospital Pediatric Department in Jan. 1995~May. 1998. We analyzed quantatively the results of acute reactive marker(CRP, ESR, WBC), pyuria, fever and compared to those of sonographically find ing and $^{99m}TC$-DMSA renal scan. Comparison between groups were performed by the chi-square (x2) test and a p value of less than 0.05 was considered statistically significant. Results : 1) The number of boys less than 1 year of age was larger than that of girls. But the number of boys more than 1 year of age was reversed. 2) The higher me level of reactive marker (CRP, WBC), the more the probability of upper UTI. 3) The higher fever, the more the probability of upper UTI. 4) The more pyuria, the more probability of upper UTI. 5) The more higher the grade of vesicoureteral reflux, the more probability of upper UTI. 6) $^{99m}TC$-DMSA renal scan is more sensitive and more specific diagnostic tool than renal sonogram. Conclusion : The appearance of an abnormal $^{99m}TC$-DMSA renal scan is correlated with acute reactive marker (CRP, ESR, WBC), fever, pyuria. $^{99m}TC$-DMSA renal scan can be a good valuable predictor tool in upper UTI. So we can start early treatment and decrease the incidence of complication of upper urinary tract by above indicators before knowing the result of urine culture. And we can follow up the patients in more good relationships with their parents by telling them the duration of treatment and follow-up plan.

• Pediatric Infection and Vaccine
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• v.10 no.2
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• pp.200-207
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• 2003

Purpose : Malaria is known to have been eradicated for a few decades through persistent national health program in South Korea. However, malaria caused by Plasmodium vivax has started to reappear incidiously among military personnel near to DMZ since 1993. After then, the number of malarial cases have been increased abruptly year by year. We analyzed the children of indigenous malaria who were diagnosed by peripheral blood smear and malarial antibody test with regards to epidemiologic and clinical manifestations. Methods : The study 13 cases below 15 years of age, who were confirmed as vivax malaria during from January 2000 to August 2003. We retrospectively analyzed epidemiologic data, clinical manifestations, laboratory findings and therapeutic responses. Results : All of 13 cases were indigenous and tested positive for Plasmodium vivax. Of 13 patients, 9 were male and 4 were female. Mean age of onset was $9.5{\pm}3.6$ years old. Ilsan(n=9) was the most prevalent area, the most patients(n=11) were presented in summer (from June to August). A characteristic feature of periodic 3 day fever in patients with P.vivax infection was reported in only 2 among 13 cases. Thrombocytopenia was most prominent findings, which was accompanied by 12 of 13 patients and pancytopenia was appeared in 3 patients on this study. The therapeutic responses of hydroxycholoquine were very good in all cases, and abnormal laboratory findings were recovered and no relapse during follow-up period. Conclusion : Vivax malaria is indigenous in Korea near to DMZ, but recently endemic area seemed to be extended southward. Plasmodium vivax is the cause of indigenous malaria of children. As for children with high fever accompanying thrombocytopenia in endemic area of Korea, malaria must be included in differential diagnosis whether the type of fewer is periodic 3 day fever or not. Malaria antibody test is helpful as a screening test for malaria.