• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.9 no.2
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• pp.200-209
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• 2006

Purpose: The purpose of this study was to evaluate the long term outcome and the factors contributing to treatment outcome for chronic functional constipation in children. Methods: Sixty three children were enrolled who had chronic functional constipation and could be followed by telephone contact. They were treated at the Bucheon Soonchunhyang Hospital for more than 1 month and observed from March 2001 to June 2005. We analyzed the clinical features, symptoms and signs, as well as the course and results of treatment. Results: The male to female ratio was 35 (55.6%) : 28 (44.4%). The mean age at the onset of symptoms and diagnosis was $21.1{\pm}23.5$ (1.9~84.0) months and $47.1{\pm}34.2$ (6.9~138.0) months, respectively. The mean defecation frequency before treatment was $3.2{\pm}2.3$ (0.5~10.0) times per week. The symptoms associated with constipation were as follows: soiling 34 (54.0%) which was more common in males than females, large stools in 30 (47.6%), decreased bowel movements less than three times a week in 20 (31.7%), straining during defecation in 19 (30.2%) and retentive posturing 19 (30.2%). The mean duration of follow-up was $34.2{\pm}14.6$ (3.6~60.0) months and 44 (69.8%) patients had their symptoms resolve ("success") and 19 (30.2%) were not resloved ("fail") from the constipation. The time for recovery from soiling, straining during defecation and retentive posturing after treatment was $4.3{\pm}2.4$ (1.0~36.0), $5.0{\pm}1.4$ (0.8~36.0) and $5.0{\pm}3.1$ (1.0~36.0) months, respectively. A relapse of the constipation occurred in 15 (23.8%) patients, 9 (60%) boys and 6 (40%) girls. The time to relapse after cessation of treatment was $2.9{\pm}1.9$ (1.0~6.0) months and the only risk factor associated with relapse was the initial duration of treatment. Conclusion: Most of the patients had resolution of symptoms within five months after treatment; relapse occurred within three months after the interruption of treatment. The duration of treatment was important for recovery and for the prevention of relapse in the constipated children. Thus a long term maintenance of therapy and follow-up is necessary for chronic functional constipation in children.

• Tuberculosis and Respiratory Diseases
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• v.50 no.4
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• pp.409-414
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• 2001

Background : Legionella pneumophila has been recognized as an important cause of community-acquired pneumonia(CAP) requiring hospitalization. However, epidemiological data on the occurrence of legionella-related pneumonia is unavailable in Korea. The purpose of this study was to evaluate the etiological importance of legionella pneumophila serogroup 1 in patients hospitalized with CAP. Method : The CAP patients over 16 year-old were recruited from July 1999 to June 2000 at the Chunchon Sacred Heart Hospital. Fifty four patients (male 29, female 25, average age $63.8{\pm}15.3$) were included in this study. A diagnosis of a legionella pneumophila infection was based on a urinary antigen test using the Binax Company enzyme immunoassay. The severity of pneumonia was assessed using the Fine's PORT scoring system. Result : The average Fine's PORT score was 99.7(${\pm}44.9$). According to the risk classification proposed by the Infectious Disease Society of America, the number of patients in each class(from class I to class V) were 6(11.1%), 13(24.1%), 9(16.7%), 14(25.8%), and 12(22.2%), respectively. Thirty two patients(59.3%) were initially admitted to the intensive care unit. The mortality rate was 16.7%(9 in 54). In all patients, urinary antigens to Legionella pnewnophila serogroup 1 were not detected. Conclusion : Legionella pnewnophila may play little role in causing adult CAP in Korea. Therefore, the routine use of macrolide in the empirical treatment of the CAP patients based upon the ATS guidelines(1993) in Korea should be reevaluated.

• Tuberculosis and Respiratory Diseases
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• v.53 no.2
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• pp.161-172
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• 2002

Background : To Investigate the association between bronchial anthracofibrosis (AF) and tuberculosis (TB), and the clinical utility of a polymerase chain reaction (PCR) on bronchial specimens for rapid diagno-sis of active pulmonary TB in patients with bronchial AF. Method : Thirty patients (25 women and 5 men ranging in age from 53 to 88), who were diagnosed with bronchial AF by a bronchoscopic exami-nation, were enrolled in this study. PCR targeting the IS6110 segment of Mycobacterium tuberculosis was performed on the bronchial wash fluid and anthracofibrotic bronchial tissue. The PCR results were compared with the bacteriological, histological, and clinical findings. Results : Eighteen of the 30 patients (60%) were associated with TB, nine of whom were confirmed as having active TB. The remaining 9 had a past history of TB. The sputum or bronchial aspirate AFB smear, culture, and histological findings were positive in 4 (13%), 9 (30%), and 5 (17%) patients, respectively. PCR of the AF tissue and bronchial wash fluid was positive in 5 (17%) and 11 (37%) of the 30 patients, respectively. PCR was more sensitive than the AFB smears for diagnosing pulmonary TB (22 % us 89 %, respectively, p<0.05). All 5 patients with positive AF tissue PCR results also had both histological findings and positive bronchial wash fluid PCR results. Of the 3 patients with positive PCR but negative bacteriological or histological results, 2 of these patients appeared to have active tuberculosis on a clinical basis. Conclusion: Although TB-PCR did not reveal an increased association between bronchial AF and TB compared with traditional methods, PCR on the bronchial wash fluid appears to be useful for the rapid diagnosis of pulmonary TB in patients with bronchial AF. TB-PCR on AF bronchial tissue itself did not yield additional benefits for diagnosing TB, which suggests that an AF lesion itself may not be an active or original site of the infection, but a secondary change of TB.

• 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.

• Clinical and Experimental Pediatrics
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• v.45 no.5
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• pp.646-653
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• 2002

Purpose : The incidence of type 2 diabetes mellitus in children has been increasing worldwide recently, which is thought to be related to the increasing prevalence of obesity. We investigated to evaluate the incidence and the characteristics of type 2 diabetes mellitus in children and also analysed the relationship between intrauterine growth retardation and type 2 diabetes mellitus. Methods : We investigated 25 children diagnosed as type 2 diabetes mellitus between March 1990 and December 2000. The analysis was performed retrospectively with medical records based on the clinical characteristics and laborotory findings. Results : Incidence of type 1 and type 2 diabetes mellitus in children has been increasing since 1990. We demonstrated an increase in the percentage of type 2 diabetes mellitus children from 5.3% in 1990 to 21.0% in 2000. Sixty eight percent of patients(17/25) were classified as obese group. Initial symptoms at first visit were polyuria, polydipsia and polyphagia 48%(12/25), asymptomatic glycosuria 40% (10/25), weight loss 8%(2/25) and obesity 4%(1/25). The mean age at diagnosis was $12.9{\pm}1.8$ years. 64%(16/25) of patients had positive family history of type 2 diabetes mellitus. Autoanti-bodies were positive in 18.1%(4/22) of patients. Twenty eight percent (7/25) of patients had an associated disease and two patients had fatty liver in association with obesity. Treatment consisted of diet, exercise, education and oral hypoglycemic agents. Three patients were treated with insulin as well as oral hypoglycemic agents because of poor blood glucose control. Long-term diabetic complications occurred in 4 patients. Intrauterine growth retardtion was found in 34.6%(9/25); 88.9% (8/9) of these patients were non-obese group. Conclusion : The increase in the incidence of type 2 diabetes mellitus in children is thought to be related to the increasing prevalence of obesity. The non-obese group of patients might be associated with intrauterine growth retardation.

• Clinical and Experimental Pediatrics
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• v.53 no.3
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• pp.420-427
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• 2010

Purpose : Several complications can occur in patients who received bone marrow transplantation (BMT) during childhood and adolescence. This study aims to investigate endocrine dysfunctions after BMT so that better care can be provided to care for long-term survivors of BMT. Methods : One hundred patients (61 males, 39 females) were included in this study. Clinical parameters such as initial diagnosis, age at BMT, conditioning regimen, presence of graft-versus-host disease (GVHD), growth pattern, thyroid function, and pubertal status were retrospectively reviewed to evaluate risk factors associated with endocrine dysfunction. Results : Height standard deviation score (SDS) at BMT, after 1 year of BMT, and at the last visit were $0.08{\pm}1.04$, $-0.09{\pm}1.02$, and $-0.27{\pm}1.18$, respectively (P =0.001). Height SDS significantly decreased in patients who received total body irradiation (TBI) (P =0.017). One of the patients who received TBI demonstrated growth hormone deficiency. Thirty (31.9%) of 94 patients had compensated hypothyroidism. Incidence of compensated hypothyroidism was higher among those who had GVHD (odds ratio 2.82, P =0.025). Of the 32 patients (17 males, 15 females) who were over 14 years in male and 13 years in female at the last visit, 16 (3 males, 13 females) had increased luteinizing hormone (LH) or follicle-stimulating hormone (FSH). Abnormal elevation of LH or FSH was more common in females (odds ratio 30.3, P =0.001). Conclusion : The most common endocrine dysfunction was ovarian insufficiency. Regular check-up for endocrine function needs to be required due to high incidence of endocrine dysfunction in patients with BMT.

• Clinical and Experimental Pediatrics
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• v.53 no.3
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• pp.392-396
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• 2010

Purpose : The association between iron deficiency anemia and febrile convulsion in infants has been examined in several studies with conflicting results. Therefore, the authors aimed to evaluate the precise relationship involved. Methods : In this case-control study, the authors assessed 100 children with a diagnosis of febrile convulsion, aged between 9 months and 2 years, during January 2007 to July 2009. The control group consisted of 100 febrile children without convulsion; controls were closely matched to the cases by age, gender, and underlying disease. Results : The mean ages of the febrile convulsion and control group were $16.3{\pm}7.4$ and $15.8{\pm}6.1$ months, respectively, and the two groups had no differences in clinical features. Iron deficiency anemia (Hb <10.5 gm/dL) was more frequent in the febrile convulsion group than in the control group, although there was no statistical significance. Unexpectably, the RDW (red blood cell distribution width) was significantly lower and the MCNC (mean corpuscular hemoglobin concentration) was significantly higher among seizure cases than among the controls (P <0.05). There is no statistical difference between simple and complex febrile groups in the clinical and laboratory profiles. On multiple logistic regression analysis, iron deficiency anemia was more frequent, but the RDW was lower, among the cases with febrile convulsion, compared with the controls. Conclusions : Our study suggests that the iron deficiency anemia is associated with febrile convulsion, and screening for iron deficiency anemia should be considered in children with febrile convulsions.

• The Korean Journal of Pain
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• v.6 no.2
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• pp.192-198
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• 1993

In 1984, a magnetic resonance spectrometer(magnetic resonance analyser, MRA $I_{TM}$) was developed by Sigrid Lipsett and Ronald J. Weinstock in the USA, Biomedical applications of the spectrometer have been examined by Dr. Hoang Van Duc(pathologist, USC), and Nakamura, et al(Japan). From their theoretical views, the biophysical functions of this machine are to analyse and synthesize a healthy tissue and organ resonance pattern, and to detect and correct an abnormal tissue and organ resonance pattern. All of the above functions are based on Quantum physics. The healthy tissue and organ resonance patterns are predetermined as standard magnetic resonance patterns by digitizing values based on peak resonance emissions(response levels or high pitched echo-sounds amplified via human body). In clinical practice, a counter or neutralizing resonance pattern calculated by the spectrometer can correct a phase-shifted resonance pattern(response levels or low pitched echo-sounds) of a diseased tissue and organ. By administering the counter resonance pattern into the site of pain and trigger point, it is possible to readjust the phase-shifted resonance pattern and then to alleviate pain through regulation of the neurotransmitter function of the nervous system. For assessing clinical effectiveness of pain relief with MRA $I_{TM}$ this study was designed to estimate pain intensity by the patient's subjective verbal rating scale(VRS such as graded to no pain, mild, moderate and severe) before application of it, to evaluate an amount of pain relief as applied the spectrometer by the patients subjective pain relief scale(visual analogue scale, VAS, 0~100%), and then to observe a continuation of pain relief following its application for managing acute and chronic pain in the 102 patients during an 8 months period beginning March, 1993. An application time of the spectrometer ranged from 15 to 30 minutes daily in each patient at or near the site of pain and trigger point when the patient wanted to be treated. The subjects consisted of 54 males and 48 females, with the age distribution between 23~40 years in 29 cases, 41~60 years in 48 cases and 61~76 years in 25 cases respectively(Table 1). The kinds of diagnosis and the main site of pain, the duration of pain before the application, and the frequency of it's application were recorded on the Table 2, 3 and 4. A distinction between acute and chronic pain was defined according to both of the pain intervals lasting within and over 3 months. The results of application of the spectrometer were noted as follows; In 51 cases of acute pain before the application, the pain intensities were rated mild in 10 cases, moderate in 15 cases and severe in 26 cases. The amounts of pain relief were noted as between 30~50% in 9 cases, 51~70% in 13 cases and 71~95% in 29 cases. The continuation of pain relief appeared between 6~24 hours in two cases, 2~5 days in 10 cases, 6~14 days in 4 cases, 15 days in one case, and completely relived of pain in 34 cases(Table 5~7). In 51 cases of chronic pain before the application, the pain intensities were rated mild in 12 cases, moderate in l8 cases and severe in 21 cases. The amounts of pain relief were noted as between 0~50% in 10 cases, 51~70% in 27 cases and 71~90% in 14 cases. The continuation of pain relief appeared to have no effect in two cases. The level of effective duration was between 6~12 hours in two cases, 2~5 days in 11 cases, 6~14 days in 14 cases, 15~60 days in 9 cases and in 13 cases the patient was completely relieved of pain(Table 5~7). There were no complications in the patients except a mild reddening and tingling sensation of skin while applying the spectrometer. Total amounts of pain relief in all of the subjects were accounted as poor and fair in 19(18.6%) cases, good in 40(39.2%) cases and excellent in 43(42.2%) cases. The clinical effectiveness of MRA $I_{TM}$ showed variable distributions from no improvements to complete relief of pain by the patient's assessment. In conclusion, we suggest that MRA $I_{TM}$ may be successful in immediate and continued pain relief but still requires several treatments for continued relief and may be gradually effective in pain relief while being applied repeatedly.

• The Journal of the Korean bone and joint tumor society
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• v.20 no.2
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• pp.54-59
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• 2014

Purpose: We report the diagnosis, treatment outcomes and prognosis of the patients with soft tissue malignant myoepithelioma in the extremities. Materials and Methods: We retrospectively reviewed 6 patients with soft tissue malignant myoepithelioma in the extremities who were treated at our institution between 2008 and 2014. Two patients received unplanned excision at another hospital and remaining 4 patients underwent the biopsy procedures and received wide excision at our hospital. Results: There were 3 men and 3 women with mean age of 41 (33-54) years. The average follow up was 28 (9-45) months. Among the 6 patients, only 4 patients underwent biopsy procedures under the impression of malignant soft tissue sarcoma. Surgical margins for these 4 patients were negative. Two patients who had unplanned excision received another re-excision and one of them showed no residual tumor in the resected specimen. Local recurrences were developed in all patients and distant metastasis in 4 patients. All 4 patients who developed distant metastasis died due to disease progression. Among the 2 patients who developed local recurrence only, one patient has another local recurrence after re-operation and remaining one patient is no evidence of disease for 2 years after resection of locally recurred mass. Conclusion: Soft tissue malignant myoepithelioma in the extremities is a rare disease and shows an aggressive behavior. Appropriate biopsy under the impression of soft tissue malignancy is necessary and complete surgical resection with wide margins is the recommended treatment of choice.

• Journal of Gastric Cancer
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• v.5 no.3 s.19
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• pp.169-173
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• 2005

Background: Gastric polyps encompass a wide variety of lesions that most commonly arise from the gastric epithelium. However, coincidental gastric carcinomas have rarely been reported, being found in $1.5{\sim}2.1%$ of patients with hyperplastic polyps. The sizes and the pathologies of polyps seem to be important in the application of treatment. Therefore, it is necessary to classily gastric polypoid lesions after a gastrectomy. Materials and Methods: During a follow-up endoscopy study, 23 patients were found to have developed gastric polyps after a gastrectomy. Most of those polyps were removed by using an endoscopic polypectomy. We performed clinical and pathologic evaluations of the gastric polyps in the remainding in the stomach after a gastrectomy, Results: The mean age of the patients was 64.5 years old with the incidence of polyps remainding in the stomach after a gastrectomy increasing after the first year following the gastrectomy. The sizes of the polyps ranged from 0.3cm to 3.5cm in diameter and the numbers of polyps below 1.0cm were 19 (82.6%). The anastomotic site was the most prevalent place 10 (43.2%), followed by the cardia 6 (26.0%) and the body 4 (17.3%). Among 23 gastric polypoid lesions Yamada types of gastric polyps in the remainding in the stomach were as follows: 1 case in type I, 12 cases in type II, 9 cases in type III, 1 case in type IV. The pathologic diagnoses of the polyps were hyperplastic polyps in 6 cases, tubular adenomas in 2 cases and inflammatory polyps in 15 cases. Conclusion: Endoscopic polypectomy is believed to be important in assessing the precise diagnosis of gastric polyps remainding in the stomach. In this study, hyperplastic polyps were found to have no malignant potential, despite their sizes. As a result aggressive biopsy with a polypectomy of gastric polyp afier gastrectomy is recommended and frequent follow-up be performed.