• Journal of Chest Surgery
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• v.38 no.8 s.253
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• pp.538-544
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• 2005

Background: Pulmonary artery banding (PAB) is an initial palliative procedure for a diverse group of patients with congenital cardiac anomalies and unrestricted pulmonary blood flow. We proved the usefulness of PAB through retrospective investigation of the surgical indication and risk analysis retrospectively. Material and Method: One hundred and fifty four consecutive patients (99 males and 55 females) who underwent PAB between January 1986 and December 2003 were included. We analysed the risk factors for early mortality and actuarial survival rate. Mean age was $2.5\pm12.8\;(0.2\sim92.7)$ months and mean weight was $4.5\pm2.7\;(0.9\sim18.0)\;kg$. Preoperative diagnosis included functional single ventricle $(88,\;57.1\%)$, double outlet right ventricle $(22,\;14.2\%)$, transposition of the great arteries $(26,\;16.8\%)$, and atrioventricular septal defect $(11,\;7.1\%)$. Coarctation of the aorta or interrupted aortic arch $(32,\;20.7\%)$, subaortic stenosis $(13,\;8.4\%)$ and total anomalous pulmonary venous connection $(13,\;8.4\%)$ were associated. Result: The overall early mortality was $22.1\%\;(34\;of\;154)$, The recent series from 1996 include patients with lower age $(3.8\pm15.9\;vs.\;1.5\pm12.7,\;p=0.04)$ and lower body weight $(4.8\pm3.1\;vs.\;4.0\pm2.7,\;p=0.02)$. The early mortality was lower in the recent group $(17.5\%;\;16/75)$ than the earlier group $(28.5\%;\;18/45)$. Aortic arch anomaly (p=0.004), subaortic stenosis (p=0.004), operation for subaortic stenosis (p=0.007), and cardiopulmonary bypass (p=0.007) were proven to be risk factors for early death in univariate analysis, while time of surgery (<1996) (p=0.026) was the only significant risk factor in multivariate analysis. The mean time interval from PAB to the second-stage operation was $12.8\pm10.9$ months. Among 96 patients who survived PAB, 40 patients completed Fontan operation, 21 patients underwent bidirectional cavopulmonary shunt, and 35 patients underwent biventricular repair including 25 arterial switch operations. Median follow-up was $40.1\pm48.9$ months. Overall survival rates at 1 year, 5 years and 10 years were $81.2\%\;65.0\%,\;and\;63.5\%$ respectively. Conclusion: Although it improved in recent series, early mortality was still high despite the advances in perioperative management. As for conventional indications, early primary repair may be more beneficial. However, PA banding still has a role in the initial palliative step in selective groups.

• Journal of Chest Surgery
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• v.32 no.7
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• pp.621-627
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• 1999

Background: Sixty five cases with congenitally corrected transposition of the great arteries (CCTGA) indicated for biventricular repair were operated on between 1984 and september 1998. Comparison between the results of the conventional(classic) connection(LV-PA) and the anatomic repair was done. Material and Method: Retrospective review was carried out based on the medical records of the patients. Operative procedures, complications and the long-term results accoding to the combining anomalies were analysed. Result: Mean age was 5.5$\pm$4.8 years(range, 2 months to 18years). Thirty nine were male and 26 were female. Situs solitus {S,L,L} was in 53 and situs inversus{I,D,D} in 12. There was no left ventricular outflow tract obstruction(LVOTO) in 13(20%) cases. The LVOTO was resulted from pulmonary stenosis(PS) in 26(40%)patients and from pulmonary atresia(PA) in 26(40%) patients. Twenty-five(38.5%) patients had tricuspid valve regurgitation(TR) greater than the mild degree that was present preoperatively. Twenty two patients previously underwent 24 systemic- pulmonary shunts previously. In the 13 patients without LVOTO, 7 simple closure of VSD or ASD, 3 tricuspid valve replacements(TVR), and 3 anatomic corrections(3 double switch operations: 1 Senning+ Rastelli, 1 Senning+REV-type, and 1 Senning+Arterial switch opera tion) were performed. As to the 26 patients with CCTGA+VSD or ASD+LVOTO(PS), 24 classic repairs and 2 double switch operations(1 Senning+Rastelli, 1 Mustard+REV-type) were done. In the 26 cases with CCTGA+VSD+LVOTO(PA), 19 classic repairs(18 Rastelli, 1 REV-type), and 7 double switch operations(7 Senning+Rastelli) were done. The degree of tricuspid regurgitation increased during the follow-up periods from 1.3$\pm$1.4 to 2.2$\pm$1.0 in the classic repair group(p<0.05), but not in the double switch group. Two patients had complete AV block preoperatively, and additional 7(10.8%) had newly developed complete AV block after the operation. Other complications were recurrent LVOTO(10), thromboembolism(4), persistent chest tube drainage over 2 weeks(4), chylothorax(3), bleeding(3), acute renal failure(2), and mediastinitis(2). Mean follow-up was 54$\pm$49 months(0-177 months). Thirteen patients died after the operation(operative mortality rate: 20.0%(13/65)), and there were 3 additional deaths during the follow up period(overall mortality: 24.6%(16/65)). The operative mortality in patients underwent anatomic repair was 33.3%(4/12). The actuarial survival rates at 1, 5, and 10 years were 75.0$\pm$5.6%, 75.0$\pm$5.6%, and 69.2$\pm$7.6%. Common causes of death were low cardiac output syndrome(8) and heart failure from TR(5). Conclusion: Although our study could not demonstrate the superiority of each classic or anatomic repair, we found that the anatomic repair has a merit of preventing the deterioration of tricuspid valve regurgitations. Meticulous selection of the patients and longer follow-up terms are mandatory to establish the selective advantages of both strategies.

• Childhood Kidney Diseases
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• v.9 no.2
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• pp.222-230
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• 2005

Purpose : ARC syndrome refers to an association of arthrogryposis, renal tubular dysfunction, and cholestasis. The VPS33B gene was recently identified as the causative gene. So far, 41 cases of ARC syndrome have been reported worldwide, and it has rarely been reported in Korea. This study was conducted to report the clinical findings of seven ARC syndrome cases in Korean children, focusing especially on renal tubular dysfunction. Methods : The hospital records of 7 cases diagnosed as ARC syndrome at Severance Hospital between Mar. 1995 and Aug. 2005 were reviewed and analyzed. Results : Of the 7 cases, 4 were boys and 3 were girls. Six patients(85$\%$) were born with normal birth weight at term, and one was born at preterm. All cases presented with cholestasis and severe jaundice. According to the type of arthrogryposis described by Brown et al, type 3 and 4 were found in 2 patients and type,6, 7, and the undistributed type in one patient respectively. Other associated clinical findings were as follows : failure to thrive in 6(85$\%$), lax skin in 5(71$\%$), and gray platelet syndrome in 4(57$\%$). Urine analysis revealed 6 cases(85$\%$) with proteinuria, 3(43$\%$) with hematuria, 5(71$\%$) with glycosuria, 2(29$\%$) with phosphaturia and 2(29$\%$) with calciuria. Serum electrolytes showed 4 cases(57$\%$) with hyponatremia, 3(43$\%$) with hypokalemia, and 1(14$\%$) with creatinine elevation. Renal tubular dysfunctions were diagnosed as renal tubular acidosis in 6 cases(85$\%$), nephrogenic diabetes insipidus in 2(29$\%$), and Fanconi syndrome in 2(29$\%$). During the follow-up period, 2(29$\%$) had no treatment, 5(85$\%$) had continuous supplementation to correct the electrolyte imbalance and acidosis, and 1(14$\%$) had dialysis. Only one patient had a family history of ARE syndrome in a sibling. Four cases(57$\%$) were diagnosed at the mean age of 8.2 months, and one case was lost during follow-up. Ages of the survived 2 cases were 13 and 25 months, respectively. Conclusion : The rare disease of ARC syndrome Is associated with severe renal dysfunction. However, this study revealed that the renal manifestation of ARC syndrome in Korean children is relatively mild and survival rate is higher than that of previous studies. Contrary to previous reports, this study showed that familial cases are rare and sporadic occurence is possible in Korea. Thus, the diagnosis of this syndrome requires a careful evaluation of the venal function in cases of congenital arthrogryposis, and a mandatory genetic counseling of affected family for prevention of familial occurance. (J Korean Soc Pediatr Nephrol 2005;9:222-230)

• Childhood Kidney Diseases
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• v.5 no.2
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• pp.136-146
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• 2001

Purpose : One of the most difficult problems in the care of children with nephrotic syndrome remains the occurrence of relapses, despite initial response to steroids. Constantinescu reported that rapidity of initial response to steroid therapy could predict fewer relapses in the first year. So we evaluated the changes in serum lipid abnormalities in children with corticosensitive nephrotic syndrome before steroid treatment and the correlation between serum lipid levels and renal function, days to remission. Methods . We analyzed the Medical records of children who were managed by us between October 1994 and August 2000. In 33 patients with corticosensitive nephrotic syndrome, we evaluated the correlation between serum lipid levels and renal function [Creatinine clearance(Ccr)] and proteinuria before steroid treatment, and days to remission defined as the third day when the patient's urine becomes protein free. Results : There were 21 males and 12 females. Median age at presentation was 6.4 years (range: 1.8-17.3 years). Median days to remission were 15.4 days (range 4-42 days) on Prednisolone $60mg/m^2$ daily. The increased levels of triglyceride, total cholesterol, LDL cholesterol, apolipoprotein B, total cholesterol/HDL cholesterol, Lipoprotein(a) were observed. But the level of HDL cholesterol was not increased. Serum albumin was decreased a]id proteinuria was increased before steroid treatment. But Ccr was not decreased. There were negative correlation between serum albumin and total cholesterol (r = -0.5157, P<0.005), LDL cholesterol (r = -0.5543, P<0.005), total cholesterol/HDL cholesterol (r = -0.4506, P<0.01), lipoprotein(a) (r = -0.4570, P<0.025), apolipoprotein B (r = -0.5297, P<0.025), apolipoprotein B/apolipoprotein Al (r = -0.5851, P<0.01), apolipoprotein B/HDL cholesterol (r = -0.4961, P<0.05) before steroid treatment. There was no correlation between proteinuria and serum lipid profiles. Also Ccr and serum lipid profiles were not correlated. There was positive correlation between days to remission and HDL cholesterol (r = +0.4511, P<0.05), apolipoprotein B (r = +0.5190, P<0.05), apolipoprotein B/HDL cholesterol (r = +0.7169, P<0.005). Conclusions : This results reveal that HDL cholesterol, apolipoprotein B and apolipoprotein B/HDL cholesterol can be used as a predictive factor in corticosensitive nephrotic syndrome. We could not determine the significant level of these lipids for insufficient patients number, but these level may predict future relapses of corticosensitive nephrotic syndrome patients and thus may allow to better management and treatment protocols. More data and long term follow up studies should be needed. (J Korean Soc Pediatr Nephrol 2001;5 : 136-46)

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.29 no.5
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• pp.301-314
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• 2003

The purposes of this retrospective study were to assess the change of serum parameters in oral and maxillofacial surgery patients after operation and to determine what laboratory parameters on treatment periods were associated with the recovery of systemic condition. For purposes of assessing systemic nutritional status, several serum parameters were chosen. The sample patients were randomsubjects extracted from three category patient groups- oral cancer, odontogenic abscess, facial bone fracture based on treated patients at department of oral and maxillofacial surgery in Pusan National University Hospital from September 1, 1998, to September 1, 2002. Each groups were consisted with 10 patients. Each patient chart was examined and blood sample parameters were reviewed with clinical signs, symptoms and vital sign at preoperative day, postoperative 1 day, postoperative 1 week. Several parameters were analyzed statistically for extraction of mean values and differences between the periods groups. The findings of serum parameters of cancer, abscess and fracture groups were as follows: 1. In cancer patients, Hb, MCV, albumin, cholesterol, LDH, AST, ALT, neutrophil, platelet, leukocyte, Na, K, Cl, BUN, creatinine were analyzed. Values of Hb, albumin, AST, neutrophil, leukocyte, Cl showed significantly differences according to periods. 2. In abscess patients, CRP, ESR, leukocyte, body temperature, neutrophil were analyzed. Values of CRP, leukocyte, body temperature, neutrophil showed significanlty differences according to periods. 3. In fracture patients, same parameters with cancer patient's were chosen. Values of platelet, Cl only showed significantly differences according to periods. 4. In cancer patients, data regarding correlation was analyzed statistically as Pearson's value. A positive correlation was found between Hb and albumin, K, Na(P<0.05). A positive correlation was also found between neutrophil and leukocyte(P<0.05). Positive correlations were found between cholesterol and ALT, LDH and platelet, creatinine both, Platelet and BUN, Na and K(P<0.01). 5. In abscess patients, Peason's correlation values were analyzed on parameters. A positive correlation was found only between CRP and neutrophil(P<0.05). 6. In fracture patients, The correlations of parameters also were statistically analyzed. Positive correlations were found between MCV and K, albumin and LDH, AST and three parameters of creatinine, Na, Cl, K and neutrophil, neutrophil and three parameters of leukocyte, BUN, K(P<0.05). Positive correlations were found between LDH and AST, ALT and AST, creatinine both(P<0.01). This retrospective clinical study showed the CRP levels only on abscess patients may be useful in determination of clinical infected status, but the levels of other parameters on cancer, fracture patients did not showed significant values as diagnostic aids for clinical status.

• Tuberculosis and Respiratory Diseases
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• v.42 no.1
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• pp.58-66
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• 1995

Background: Acute interstitial pneumonia is a relatively rare form of interstitial pneumonia, since the vast majority of interstitial pneumonia have a more chronic course. It corresponds to the lesion described by Hamman and Rich, as Hamman-Rich disease in 1944. Another name in the clinical literature is accelerated interstitial pneumonia, idiopathic acute respiratory distress syndrome (idiopathic ARDS), and the organizing stage of diffuse alveolar damage. Acute interstitial pneumonia differs from chronic interstitial pneumonia by clinical and pathologic features. Clinically, this disease is characterized by a sudden onset and a rapid course, and reversible disease. Method and Purpose: Five cases of pathologically proven acute interstitial pneumonia were retrospectively studied to define the clinical, radiologic, and pathologic features. Results: 1) The five cases ranged in age from 31 to 77 years old. The onset of illness was acute in all patients, it began with viral-like prodrome 6~40 days prior to shortness of breath, and respiratory failure eventually developed in all patients. In 2 cases, generalized skin rash was accompanied with flu-like symptoms. Etiologic agent could not be identified in any case. 2) All patients had leukocytosis and severe hypoxemia. Pulmonary function test of 3 available cases shows restrictive ventilatory defect, and one survived patient(case 5) has a complete improvement of pulmonary function after dismissal. 3) Diffuse bilateral chest infiltrates were present radiologically. Theses were the ground-glass, consolidation, and reticular densities without honeycomb fibrosis in all patients. The pathologic abnormalities were the presence of increased numbers of macrophages and the formation of hyaline membranes within alveolar spaces. There was also interstitial thickening with edema, proliferation of immature fibroblast, and hyperplasia of type II pneumocyte. In the survived patient(case5), pathologic findings were relatively early stage of acute interstitial pneumonia, such as hyaline membrane with mild interstitial fibrosis. 4) Of the 5 patients, four patients died of respiratory failure 14~90 days after onset of first symptom, and one survived and recovered in symptoms, chest X ray, and pulmonary function test Conclusion: These results emphasize that acute interstitial pneumonia is clinically, radiologically, and pathologically distinct form of interstitial pneumonia and should be separated from the group of chronic interstitial pneumonia. Further studies will be needed to evaluate the pathogenesis and the treatment of acute interstitial pneumonia.

• The Korean Journal of Nuclear Medicine Technology
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• v.13 no.1
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• pp.77-81
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• 2009

Purpose: The PET of the PET/CT (Positron Emission Tomography/Computed Tomography) quantitatively shows the biological and chemical information of the body, but has limitation of presenting the clear anatomic structure. Thus combining the PET with CT, it is not only possible to offer the higher resolution but also effectively shorten the scanning time and reduce the noises by using CT data in attenuation correction. And because, at the CT scanning, the contrast media makes it easy to determine a exact range of the lesion and distinguish the normal organs, there is a certain increase in the use of it. However, in the case of using the contrast media, it affects semi-quantitative measures of the PET/CT images. In this study, therefore, we will be to establish the reliability of the SUV (Standardized Uptake Value) with CT data correction so that it can help more accurate diagnosis. Materials and Methods: In this experiment, a total of 30 people are targeted - age range: from 27 to 72, average age : 49.6 - and DSTe (General Electric Healthcare, Milwaukee, MI, USA) is used for equipment. $^{18}F$- FDG 370~555 MBq is injected into the subjects depending on their weight and, after about 60 minutes of their stable position, a whole-body scan is taken. The CT scan is set to 140 kV and 210 mA, and the injected amount of the contrast media is 2 cc per 1 kg of the patients' weight. With the raw data from the scan, we obtain a image showing the effect of the contrast media through the attenuation correction by both of the corrected and uncorrected CT data. Then we mark out ROI (Region of Interest) in each area to measure SUV and analyze the difference. Results: According to the analysis, the SUV is decreased in the liver and heart which have more bloodstream than the others, because of the contrast media correction. On the other hand, there is no difference in the lungs. Conclusions: Whereas the CT scan images with the contrast media from the PET/CT increase the contrast of the targeted region for the test so that it can improve efficiency of diagnosis, there occurred an increase of SUV, a semi-quantitative analytical method. In this research, we measure the variation of SUV through the correction of the influence of contrast media and compare the differences. As we revise the SUV which is increasing in the image with attenuation correction by using contrast media, we can expect anatomical images of high-resolution. Furthermore, it is considered that through this trusted semi-quantitative method, it will definitely enhance the diagnostic value.

• Radiation Oncology Journal
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• v.14 no.1
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• pp.1-8
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• 1996

Purpose: We tried to evaluate the role of conventional radiotherapy alone or with neoadjuvant chemotherapy in oropharyngeal cancer in terms of survival rates and to identify prognostic factors affecting survival by retrospective analysis. Materials and Methods: Forty seven patients of oropharyngeal cancer were treated by conventional radiotherapy in our hospital from Nov. 1985 to APr. 1993. Of these, twenty six patients were treated by conventional radio-therapy alone, and 21 patients with neoadjuvant chemotherapy of mostly two or more cycles of cisplatin and pepleomycin. The Patient characteristics of radiotherapy alone group and neoadjuvant chemotherapy group were not different generally. Radiotherapy was performed by 6MV-LINAC and the total radiation doses of Primary tumors were 54.0-79.2 Gy and cervical lymph nodes were 55.8-90.0 Gy with a fraction size of 1.8 or 2.0 Gy per day. The range of follow-up periods was 3-102 months and median was 20 months. The range of a9e was 33-79 years old and median was 58 years old. Results : Overall 3-year actuarial survival rate (3YSR) of all patients was $39\%$. The 3YSRS of stage I (n=5), II (n=11), III (n=12) and IV (n=19) were 60, 55, 33 and $32\%$, respectively The 3YSRS of Tl+2, T3+4 and No, N+ were 55, $18\%$ (p=0.005) and 43, $36\%$ (p>0.1), respectively. There was no difference in 3YSRS between radiotherapy alone group and neoadjuvant chemotherapy group (38 vs $43\%$, p>0.1). According to the original site of primary tumor, the 3YSRS of tonsil (n=32), base of tongue (n=8), soft palate or uvula (n=6) and pharyngeal wall (n=1) were 36 38, 67 and $0\%$, respectively The Patients of soft palate or uvular cancer had longer survival than other primaries but the difference was not significant statistically (p>0.1). Of 32 patients of tonsillar cancer, 22 Patients who had primary extension to adjacent tissue showed inferior survival rate to the ones who had not Primary extension, but the difference was marginally significant statistically (24 vs $60\%$, p=0.08). On Cox multivariate analysis in entire patients with variables of age, T stage, N stage, total duration of radiotherapy, the site of primary tumor and the use of neoadjuvant chemotherapy, only T stage was a significant Prognostic factor affecting 3YSR. Conclusion : The difference of 3YASRS of conventional radiotherapy alone group and neoadjuvant chemotherapy group was not significant statistically. These treatments could be effective in oropharyngeal cancer of early stage, especially such as soft palate, uvular or tonsillar cancer which did not extend to adjacent tissue. But in order to improve the survival of patients of most advanced oropharyngeal cancer, other altered fractionated radiotherapy such as hyperfractionation rather than conventional fractionation or multi-modal approach combining radiotherapy and accessible surgery or concurrent chemotherapy might be beneficial.

• Radiation Oncology Journal
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• v.27 no.4
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• pp.181-188
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• 2009

Purpose: The aim of this study was to evaluate the results of postoperative radiotherapy in a case of perihilar cholagiocarcinoma by analyzing overall survival rate, patterns of failure, prognostic factors for overall survival, and toxicity. Materials and Methods: Between January 1998 and March 2008, 38 patients with perihilar cholangiocarcinoma underwent a surgical resection and adjuvant radiotherapy. The median patient age was 59 years (range, 28 to 72 years), which included 23 men and 15 women. The extent of surgery was complete resection in 9 patients, microscopically positive margins in 25 patients, and a subtotal resection in 4 patients. The tumor bed and regional lymphatics initially received 45 Gy or 50 Gy, but was subsequently boosted to a total dose of 59.4 Gy or 60 Gy in incompletely resected patients. The median radiotherapy dose was 59.4 Gy. Concurrent chemotherapy was administered in 30 patients. The median follow-up period was 14 months (range, 6 to 45 months). Results: The 3-year overall survival and 3-year progression free survival rates were 30% and 8%, respectively. The median survival time was 28 months. A multivariate analysis showed that differentiation was the only significant factor for overall survival. The 3-year overall survival was 34% in R0 patients and 20% in R1 patients. No statistically significant differences in survival were found between the 2 groups (p=0.3067). The first site of failure was local in 18 patients (47%). No patient experienced grade 3 or higher acute toxicity and duodenal bleeding developed in 2 patients. Conclusion: Our results suggest that adjuvant RT might be a significant factor in patients with a positive margin following a radical resection. However, there was still a high locoregional recurrence rate following surgery and postoperative radiotherapy. Further study is necessary to enhance the effect of the adjuvant radiotherapy.

• Pediatric Infection and Vaccine
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• v.11 no.2
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• pp.158-169
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• 2004

Purpose : Although influenza is one of the most important cause of acute respiratory tract infections in children, virus isolation is not popular and there are only a few clinical studies on influenza and diagnostic methods. We evaluated the epidemiological and clinical features of influenza in children and rapid antigen detection test(QuickVue influenza test) on fist half of the year 2004 in Busan. Methods : From January 2004 to June 2004, throat swab and nasal secretion were obtained and cultured for the isolation of influenza virus and tested by rapid antigen detection test(QuickVue influenza test) in children with suspected influenza infections. The medical records of patients with influenza virus infection were reviewed retrospectively. Results : Influenza viruses were isolated in 79(17.2%) out of 621 patients examined. Influenza virus was isolated mainly from March to April 2004. The ratio of male and female with influenza virus infection was 1.2 : 1 with median age of 4 years 6month. The most common clinical diagnosis of influenza virus infection was bronchitis. There was no difference between influenza A and B infection in clinical diagnosis and symptoms. All patients recovered without severe complication. The sensitivity obtained for rapid antigen detection test (QuickVue influenza test) was 93.6% and the specificity was 80.2%, the positive predictive value 40.8%, the negative predictive value 98.8%. Conclusion : With rapid antigen detection test, it is possible early detection of influenza in children. reduction in use of antimicrobial agent and early use of antiviral agent.