• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.7 no.2
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• pp.284-288
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• 2004

Spontaneous perforation of the biliary duct is a rare disease that must be treated immediately when diagnosed in infancy. This disease must be suspected in a previously healthy infant who suddenly has abdominal distension, ascites, and intermittent jaundice. The best way to diagnose the leakage of bile in a clinically stable infant is to use radionuclide hepatobiliary scan. In most cases, however, the diagnosis of the perforation of common bile duct is frequently made during the procedure of operative cholangiogram. The prognosis is usually good with early diagnosis. We experienced an infant of 4 months of age who presented with sudden abdominal distension, jaundice and acholic stool, and confirmed the diagnosis of perforation of common bile duct through operative cholangiogram with t-tube insertion. We report a case of spontaneous perforation in an infant with review of the literatures.

• Clinical and Experimental Pediatrics
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• v.46 no.8
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• pp.769-776
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• 2003

Purpose : The survival rate of very low birth weight infants(VLBWI) has improved by virtue of specialized neonatal care. This study was performed to analyze the changes in incidence, survival rate and morbidity of VLBWI who admitted to Chonnam National University Hospital from 1996 to 2001. Methods : We enrolled 565 VLBWI, and compared the incidence and the survival rate according to the birth weight or gestational weeks between period I(1996 to 1998) and period II(1999 to 2001). The mortality rate according to the postnatal age, cause of death, morbidity and days of hospital stay were also compared. Morbidity is categorized into 'short term' which is curable until discharge, and 'long term' causing any types of sequelae after discharge. Results : Incidence of VLBWI significantly increased in period II over period I(6.0% vs. 11.0%, P< 0.001). The survival rate also increased in period II(71.8% vs. 80.1%, P<0.05), especially in 1,000 to 1,249 gm of birth weight(P<0.001) and in 28 to 30 weeks of gestation(P<0.001). The most common cause of death was respiratory distress syndrome in period I; however it was sepsis in period II. Although overall and short term morbidity rate increased, long term morbidity and days of hospital stay didn't increase in period II. Conclusion : Although the incidence of VLBWI significantly increased and the survival improved in period II compared to period I, especially in 1,000 to 1,249 gm of birth weight and 28 to 30 weeks of gestation, 'long term' morbidity rate and hospital days didn't increase.

• Neonatal Medicine
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• v.18 no.2
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• pp.288-292
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• 2011

Purpose: The incidence of methicillin-resistant Staphylococcus aureus (MRSA) infection has increased in children and in neonates, and is particularly associated with frequent use of central venous catheter in very low birth weight (VLBW) infants. It is known that the morbidity and mortality of MRSA infection are low in neonates, as compared with adults. The objective of this study was to examine the difference in clinical characteristics between VLBW infants that survived and those that did not, a catheter-related bloodstream infection (CRBSI) of MRSA. Methods: Thirty-four VLBW infants had laboratory-confirmed bloodstream infection with S. aureus. We examined the incidence, mortality and morbidity of CRBSI, and predictive factors associated with mortality. Results: Twenty-six infants had same pathogen (24 MRSA, 2 Methicillin-sensitive Staphylococcus aureus) in the blood and in the catheter tip. Eight infants (25.8%) died in the CRBSI and they all had MRSA blood infections. Sex ratio, gestational age, duration between blood collection and identification of pathogens, and WBC and platelet count were not significantly different between patients that died from and patients that survived CRBSI of MRSA. C-reactive protein (CRP) was significantly higher in VLBW infants that died. Mean age of onset and hospital day was earlier (9.1${\pm}$6.6 vs. 26.9${\pm}$20.2; P=0.005) and shorter for patients that died (10.1${\pm}$7.0 vs. 73.0${\pm}$32.4; P=0.000). Two survivors had complications of pyogenic arthritis of the lower extremities and soft tissue infection, respectively. Conclusion: Mortality of CRBSI was likely to be high in VLBW infants and might be anticipated by CRP and early onset of disease.

• Neonatal Medicine
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• v.16 no.1
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• pp.36-46
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• 2009

Purpose: To evaluate the neonatal statistics on a national basis, data for birth characteristics and neonatal mortality were collected and analyzed from 57 hospitals in Korea. Methods: Questionnaires were distributed to determine the characteristics of neonatal births and mortality rates in 57 hospitals in Korea during 2007. We analyzed the characteristics of all inborn births and hospitalized neonates in the neonatal care units (NICUs) and compared the results with published Korean data from 1996 and 2002. Results: A total of 40,433 inborn live births were reported from the 57 hospitals during 2007. Pre-term, term, and post-term births comprised 24.2%, 75.6%, and 0.2% of the neonates, respectively. Low birth weight infants (LBWIs), very low birth weight infants (VLBWIs), and extremely low birth weight infants (ELBWIs) made up 22.0%, 4.6%, and 1.7% of the neonates, respectively. A total of 21,957 (collected by gestational period) and 21,356 (collected by birth weight) neonates were hospitalized in the 57 NICUs. Pre-term, term, and post-term neonates comprised 39.8%, 59.8%, and 0.4% of the neonates, respectively. LBWIs, VLBWIs, and ELBWIs made up 37.3%, 9.5%, and 3.3% of the neonates, respectively. Pre-term, term, and post-term neonates had mortality rates of 4.5%, 0.7%, and 3.7%, respectively, while the mortality rates of LBWIs, VLBWIs, and ELBWIs were 4.7%, 15.3%, and 32.2%, respectively. In comparison with prematurity data from 1996 and 2002, LBWIs, and ELBWIs had a marked increase in birth frequency and a decreased mortality rate in Korea during 2007. Conclusion: The number of live births and the survival rate of pre-term neonates, especially VLBWIs and ELBWIs, are increasing. Even though the outcomes of neonatal care are improving, further efforts to manage these premature infants are needed.

• Tuberculosis and Respiratory Diseases
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• v.45 no.1
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• pp.57-67
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• 1998

Purpose : This study was undertaken to determine the preoperative predictors of mortality and morbidity after lung cancer resection. Method: During the period from October 1, 1995 to August 31, 1996, a prospective study was conducted in 92 lung resection candidates diagnosed as lung cancer. For preoperative predictors of nonpulmonary factors, we considered age, sex, weight loss, hematocrit, serum albumin, EKG and concomitant illness, and for those of pulmonary factors, smoking history, presence of pneumonia, dyspnea scale(1 to 4), arterial blood gas analysis with room air breathing, routine pulmonary function test. And predicted postoperative(ppo) pulmonary factors such as PPO-$FEV_1$, ppo-diffusing capacity(DLco), predicted postoperative product(PPP) of ppo-$FEV_1%{\times}ppo$-DLco% and ppo-maximal $O_2$ uptake($VO_2$max) were also considered. Results: There were 78 men and 14 women with a median age of 62 years(range 42 to 82) and a mean $FEV_1$ of $2.37\pm0.06L$. Twenty nine patients had a decreased $FEV_1$ less than 2.0L. Pneumonectomy was performed in 26 patients, bilobectomy in 12, lobectomy in 54. Pulmonary complications developed in 10 patients, cardiac complications in 9, other complications(empyema, air leak, bleeding) in 11, and 16 patients were managed in intensive care unit for more than 48hours. Three patients died within 30 days after operation. The ppo-$VO_2$max was less than 10ml/kg/min in these three patients, but its statistical significance could not be determined due to small number of patients. In multivariate analysis, the predictor related to postoperative death was weight loss(p<0.05), and as for pulmonary complications, weight loss, dyspnea scale, ppo-DLco and extent of resection(p<0.05). Conclusions: Based on this study, preoperative nonpulmonary factors such as weight loss and dyspnea scale are more important than the pulmonary factors in the prediction of postoperative mortality and/or morbodity in lung resection candidates, but exercise pulmonary fuction test may be useful Our study suggests that ppo-$VO_2$max value less than 10ml/kg/min is associated with death after lung cancer resection but further studies are needed to validate this result.

• Tuberculosis and Respiratory Diseases
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• v.48 no.1
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• pp.14-23
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• 2000

Background: The evaluation of candidates for successful lung resection is important. Our study was conducted to determine the preoperative predictors of postoperative mortality and morbidity in lung cancer patients with impaired lung function. Method; Between October 1, 1995 and August 31, 1997, 36 lung resection candidates for lung cancer with $FEV_1$ of less than 2L or 60% of predicted value were included prospectively. Age, sex, weight loss, hematocrit, serum albumin, EKG and concomitant illness were considered as systemic potential predictors for successful lung resection. Smoking history, presence of pneumonia, dyspnea scale(l to 4), arterial blood gas analysis with room air breathing, routine pulmonary function test were also included for the analysis. In addition, predicted postoperative(ppo) pulmonary factors such as ppo-$FEV_1$ ppo-diffusing capacity(DLco), predicted postoperative product(PPP) of ppo-$FEV_1%{\times}$ppo-DLco% and ppo-maximal $O_2$ uptake($VO_2$max) were also measured. Results: There were 31 men and 5 women with the median age of 65 years(range, 44 to 82) and a mean $FEV_1$ of $1.78{\pm}0.06L$. Pneumonectomy was performed in 14 patients, bilobectomy in 8, lobectomy in 14. Pulmonary complications developed in 10 patients; cardiac complications in 3, other complications(empyema, air leak, bleeding) in 4. Twelve patients were managed in the intensive care unit for more than 48 hours. Two patients died within 30 days after operation. The ppo-$VO_2$max was less than 10 ml/kg/min in these two patients. MVV was the only predictor for the pulmonary complications. However, there was no predictor for the post operative death in this study. Conclusions: Based on the results, MVV was the useful predictor for postoperative pulmonary complications in lung cancer resection candidates with impaired lung function In addition, ppo-$VO_2$max value less than 10 ml/kg/min was associated with postoperative death, so exercise pulmonary function test could be useful as preoperative test. But further studies are needed to validate this result.

• Journal of Genetic Medicine
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• v.6 no.2
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• pp.161-165
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• 2009

Pompe disease (glycogen storage disease type II) is an autosomal recessive disorder caused by deficiency of acid-${\alpha}$-glucosidase (GAA) resulting in lysosomal glycogen accumulation in multiple tissue, particularly cardiac and skeletal muscle. The classic infantile form of Pompe disease is characterized by marked cardiomegaly, respiratory failure and severe generalized hypotonia. Most patients die from cardiorespiratory failure or respiratory infection within the first year or two of life without treatment. A "non-classic" phenotype presents with less severe clinical feature and slow progression of disease. We report two patients with non-classic infantile Pompe disease from one family manifested hypertrophic cardiomyopathy and progressive proximal weakness.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.12 no.1
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• pp.42-48
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• 2012

Pompe disease is a rare lysosomal glycogen storage disorder caused by a total or partial deficiency of the acid ${\alpha}$-glucosidase (GAA) enzyme due to the GAA gene mutations. The classic infantile form of Pompe disease is a rapidly progressive multi-organ disease with hypotonia, generalized muscle weakness, and hypertrophic cardiomyopathy, usually leading to death in the first 2 years of life. Enzyme replacement therapy with recombinant human GAA has been shown to be effective and subsequently yielded promising results. Here, we present clinical and genetic characteristics of three Korean non-classic infantile Pompe patients, and the short term efficacy of enzyme replacement therapy. Considering that enzyme replacement therapy can change the natural course of infantile Pompe disease, early diagnosis and early initiation of treatment is critical to improving patient outcomes.

• Journal of Chest Surgery
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• v.35 no.5
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• pp.387-391
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• 2002

Background: Postoperative morbidity and mortality in destroyed lung are relatively high. We tried to identify the prognostic factors affecting postoperative morbidity and mortality in destroyed lung through a retrospective study. Material and method: The retrospective study was undertaken in 112 patients who had undergone pneumonectomy or pleuropneumonectomy for destroyed lung at Severance Hospital from 1970 to 2000. We analyzed the correlation between postoperative morbidity and mortality and etiology, duration of disease, preoperative FEV1, presence or absence of peroperative empyema, operation timing, the side of operation, duration of operation, and operation type. Result: There were 55 men and 57 women, aged 20 to 81 years (mean 44 years). Etiologic diseases were tuberculosis in 86 patients(76.8%) including tuberculos empyema in 20 and tuberculous bronchiectasis in 4, pyogenic empyema in 12(10,7%), bronchiectasis in 12(10.7%), and lung abscess in 2(1.8%). Postoperative morbidity were 25%(n=28) and postoperative mortality was 6%(n=7). The presence of preoperative empyema(p=0.016), pleuropneumonectomy(p=0.037) and preoperative FEV1 of less than 1.75 L(P=0.048) significantly increased the postoperative morbidity, If operation time was less than 300min, postoperative morbidity(p=0.002) and mortality(p=0.03) were significantly low. Conclusion: Postoperative morbidity and mortality in destroyed lung were acceptable. Postoperative morbidity and mortality were significantly low when operation time was less than 300 min. Preoperative existence of empyema, pleuropneumonectomy and preoperative FEV1 of less than 1.75 L significantly increased postoperative morbidity.

• Journal of Chest Surgery
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• v.37 no.4
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• pp.328-334
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• 2004

There are only limited numbers of reports about long-term results of tricuspid valve replacement(TVR) with bioprosthetic and mechanical prostheses. We analyzed risk factors for tricuspid valve replacement and compared long-term clinical results of bioprosthetic and mechanical valves in tricuspid position. Material and Method: We reviewed 77 cases of TVR, which were performed between October 1978 and December 1996. Mean age was 38.8 15.9 years. Bioprostheses were implanted in 26 cases and mechanical prostheses were implanted in 51 cases. Result The operative mortality was 15.6% and late mortality was 12.3%. Survival for bioprosthetic and mechanical valve group at 5, 10 and 13 years was 81.3% vs. 100%, 66.1% vs. 100%, 60.6% vs. 100% (p=0.0175). Free from valve related re-operation for bioprosthetic and mechanical valve group at 5, 10 and 13 years was 100% vs. 93.9%, 100% vs. 93.9% and 58.3% vs. 93.9% (p=0.3274). Linealized incidences of valve related re-operation for bioprosthetic and mechanical valve group was 2.27 %/patient-years and 1.10 %/patient-years. Risk factor analysis showed that presence of preoperative ascites, hepatomegaly larger than 2 finger breaths, poor preoperative NYHA functional class and number of tricuspid valve replacement were risk factors for early mortality, and the use of bioprosthetic valve and number of open heart surgery were risk factors for late mortality. Conclusion: Long-term survival of mechanical valve was superior to bioprosthetic valve in tricuspid position. We recommend mechanical valve in tricuspid position if the patient can be closely followed up.