• Annals of Pediatric Endocrinology and Metabolism
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• v.23 no.4
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• pp.235-239
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• 2018

Most cases of congenital hyperthyroidism are autoimmune forms caused by maternal thyroid stimulating antibodies. Nonautoimmune forms of congenital hyperthyroidism caused by activating mutations of the thyrotropin receptor (TSHR) gene are rare. A woman gave birth to a boy during an emergency cesarean section at 33 weeks of gestation due to fetal tachycardia. On the 24th day of life, thyroid function tests were performed due to persistent tachycardia, and hyperthyroidism was confirmed. Auto-antibodies to TSHR, thyroid peroxidase, and thyroglobulin were not found. The patient was treated with propylthiouracil and propranolol, but hyperthyroidism was not well controlled. At 3 months of age, the patient had craniosynostosis and hydrocephalus, and underwent a ventriculoperitoneal shunt operation. Direct sequencing of the TSHR gene showed a heterozygous mutation of c.1899C>A (p.Asp633Glu) in exon 10. No mutations were discovered in any of the parents in a familial genetic study. We have reported a case of sporadic nonautoimmune congenital hyperthyroidism, by a missense mutation of the TSHR gene, for the first time in South Korea.

• Journal of Chest Surgery
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• v.17 no.4
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• pp.593-606
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• 1984

With the ligation of patent ductus arteriosus by Gross in 1938, surgeons first entered the field of congenital heart disease. Interruption of a ductus is one of the most satisfactory and curative operations in the field of surgery for congenital heart disease. 27 cases of isolated patent ductus arteriosus were operated from Jan. 1978 to July 1984 at the Department of Thoracic & Cardiovascular Surgery in Kyung-Hee University Hospital. Retrospective clinical analysis of these patients were: 1. Sex ratio, female: male, was 2:1. 2. Mean age at operation was 9.85\ulcorner.58 years. The youngest patient was a 23 month-old girl and the oldest one was a 24 year-old male. 3. More than half of the patients had less than 50 percentile of growth retardation. 4. Chief complaints of the patients were frequent URI [52%], dyspnea on exertion [33%], generalized weakness [22%], palpitation [7%], but 7 patients [26%] had no subjective symptoms. 5. Continuous machinery murmur could be heard at the 2nd or 3rd intercostal space on the left sternal border in 22 patients [81%]. The other S patients made systolic murmur with accentuation of the second heart sound and those were associated with pulmonary hypertension. 6. Radiologic findings of Chest P-A were cardiac enlargement in 15 patients [55%], enlargement of pulmonary conus and/or increasing density of pulmonary vascularity in 20 patients [74%]. 7. Electrocardiographic findings of the patients were within normal limit in 13 patients [48%], LVH in 4 patients [15%], biventricular hypertrophy in 3 patients [11%]. 8; echocardiogram was obtained from 11 patients. Ductus was directly visualized in 7 patients. Left atrial enlargement is the secondary change of left to right shunt, 10 patients had LA/Ao ratio more than 1.2. 9. Cardiac catheterization performed in 25 patients. The mean value of the results were:SO2[PA-RV]= 14.72\ulcorner6.01%, Qp/Qs=2.22\ulcorner.80, peak systolic pulmonary arterial pressure=48.28\ulcorner1.60 mmHg. 10. 26 patients were operated through the left posterolateral thoracotomy: closure of ductus by double ligation in 14 cases, triple ligation in 5 cases, and division with suture in 8 cases. One patient suffer from aneurysmal rupture of main pulmonary artery, endocarditis, hemopericardium was treated with cardiopulmonary bypass via median sternotomy and closure of ductus through the ruptured main pulmonary artery. 11.There was no death associated with the operation, but 3 cases were experienced with intraoperative rupture around the ductus resulting in massive bleeding. The other complications were transient hoarseness in one patient, atelectasis in left lower lobe in 3 patients, and postoperative systemic hypertension in 4 patients with unknown etiology. 12. Pulse pressure was reduced, 11.47+5.92 mmHg, postoperatively, as compare to preoperative status. 13. Intraoperative wedge lung biopsy from lingular segment for the evaluation of the pulmonary vascular disease was taken in S patients with severe pulmonary hypertension. The result was Heath-Edward grade I in one case, grade II in two cases, and grade III in two cases.

• Journal of Chest Surgery
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• v.36 no.8
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• pp.559-565
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• 2003

By improving the flow pattern in Fontan circuit, total cavopulmonary connection (TCPC) could result in a better outcome than atriopulmonary connection Fontan operation. For the patients with impaired hemodynamics after atriopulmonary Fontan connection, conversion to TCPC can be expected to bring hemodynamic and functional improvement. We studied the results of the revision of the previous Fontan connection to TCPC in patients with failed Fontan circulation. Material and method: From October1979 to June 2002, eight patients who had failed Fontan circulation, underwent revision of previous Fontan operation to TCPC at Yonsei University Hospital. Intracardiac anomalies of the patients were tricuspid atresia (n=4) and other functional single ventricles (n=4). Mean age at TCPC conversion was 14.0$\pm$7.0 years (range, 4.6~26.2 years) and median interval between initial Fontan operation and TCPC was 7.5 years (range, 2.4~14.3 years). All patients had various degree of symptoms and signs of right heart failure. NYHA functional class was 111 or IV in six patients. Paroxysmal atrial fibrillation (n:f), cyanosis (n=2), intraatrial thrombi (n=2), and protein losing enteropathy (PLE) (n=3) were also combined. The previous Fontan operation was revised to extracardiac conduit placement (n=7) and intraatrial lateral tunnel (n=1). Result: There was no operative death. Major morbidities included deep sternal infection (n=1), prolonged pleural effusion over two weeks (n=1), and temporary junctional lachyarrhythrnia (n=1). Postoperative central venous Pressure was lower than the preoperative value (17.9$\pm$3.5 vs. 14.9$\pm$1.0, p=0.049). Follow-up was complete in all patients and extended to 50,1 months (mean, 30.3$\pm$ 12.8 months). There was no late death. All patients were in NYHA class 1 or 11. Paroxysmal supraventricular tachycardia developed in a patient who underwent conversion to intraatrial lateral tunnel procedure, PLE was recurred in two patients among three patients who had had PLE before the convertsion. There was no newly developed PLE. Conclusion: Hemodynamic and functional improvement could be expected for the patients with Fontan circulatory failure after atriopulmonary connection by revision of their previous circulation to TCPC. The conversion could be performed with low risk of morbidity and mortality.

• Journal of Chest Surgery
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• v.40 no.12
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• pp.837-842
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• 2007

Background: Closure of the ductus arteriosus is often delayed in premature infants, which creates a hemodynamically significant left to right shunt that exerts an adverse effect on the normal development and growth of these babies. We reviewed out experience on surgical closure of patent ductus arteriosus via axillary minithoracotomy in premature infants. Material and Method: From April 2002 to October 2006, 20 premature infants whose gestation was under 37 weeks underwent surgical closure of patent ductus arteriosus as a result of complications or contra-indications for the use of indomethacin. Their mean gestational age was 28.8+3.4 weeks, ranging from 25+3 to 34+6 weeks, and the average age at operation was $15.6{\pm}6.3$ days. The mean body weight at operation was $1,174{\pm}416\;g$, ranging from 680 to 2,100g; 16 infants were under 1,500 and 9 infants were under 1,000 g. The procedures were performed in the newborn intensive care unit via $2{\sim}3\;cm$ long axillary minithoracotomy with the infant in the lateral position with left arm abduction. The mean size of the patent ductus arteriosus was $3.8{\pm}0.3\;mm$. For the most part, the ductus was closed with clips; 2 infants in whom the ductus was ruptured while dissection was being performed underwent ductal division. Result: Ten of twelve infants who had been ventilator dependent preoperatively could be successfully weaned from the ventilator at a mean duration of 9.7 days after the operation. There was no procedure-related complication or death. Two infants eventually died of the conditions not related to the operation; one from sepsis at postoperative 131 days and the other from pneumonia at postoperative 41 days, respectively. Conclusion: Surgical closure of the patent ductus arteriosus improved the hemodynamic instability and so promoted the successful growth and normal development of premature infants. Considering the low surgical risk along with the reduced invasiveness, early and aggressive surgical intervention is highly recommended.

• Journal of Chest Surgery
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• v.30 no.9
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• pp.869-875
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• 1997

Perimembranous ventri ular septal defects(PMVSDS) are the most common type of ventricular septal defects(VSDs) and consist morphologically of deficiency of the membranous septum and variable portions of the adjacent muscular septum. Repair of VSD has begun via a right ventriculotomy. Even with this exposure, however, it mght lead to ventricular dysfunction. Transatrial exposure of VSDs is luiown to a versatile approach to PMVSDS and even malaligunent defects can be repaired by this method. Although transatrial exposure can be improved by taking down'the atrioventricular valve at the annulus, surgeons have been hesitant to do so because of concern for valvular competence. Therefore, this study was undertaken to clarity the effects of transamlular approach of tricuspid valve (TATV) at operation of PMVSD. During last 5 years, twenty eight cases from 96 patients of PMVSD were closed by TATV and follow up study was done from 3 months to 33 months and results were obtained as follows. 1. Age at operation was fr m 4 months to 38 years and most patients(17, 62%) were above 5 years. 2. Preoperative pulmonary-systemic flow ratio(QPIQS) was ranged from 1 to 2.8 and 22 patients(79%) were less than 2. 3. Peak systolic pulmonary artery pressure was below 30mmHg in 8, 30-50mmHg in 17, above 50mmHg in 3 patients and 25 patients(89%) were less than 50mmHg. 4. Preoperative tricuspid regurgitation(TR) is none in 12, trivial in 6, mild in 3, moderate in 5, severe in 2 patients but postoperative TR was none in 18, trivial in 6, mild in 4 patients, so TR in most patients had decreased or not. 5. Indications for operation were based on the presence of a significant shunt. However, in patients with small shunts, indications for operation were included additional factors, tricuspid valve pouch, RVOT obstruction(right ventricular outflow tract obstruction), subacute bacterial endocarditis and associated anomalies. 6. There were no hospital deaths and residual shunts in postoperative echocardiography. Therefore TATV is especially a good method in PMVSn where patients have trcuspid valve pouch. And it is a safe and effective technique that improves exposure for PMVSD repair and does not adversely affect tricuspid valvular competence.

• Journal of Chest Surgery
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• v.29 no.2
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• pp.177-184
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• 1996

Between 1987 and 1994, 21 patients were treated surgically for aortic aneurysm involving the thoracoabdominal aorta. There were 11 males and 10 females, and their age ranged from 20 to 67 years old and mean age was 41.5 years. Many complained of back pain, chest pain or discomfort, and flank pain, but three patients were asymptomatic. 15 patients had chronic dissection (71.4%) and 6 had nondissecting fusiform or saccular aneurysm(28.5%), and of those 15 patients with chronic dissection, 6(28.5%) had atherosclerosis assniated with hypertension, 5 (23.8%) were Martian syndrome, and 2 (9.5%) were associated with pregnancy. The diameter of an aneurysm ranged from 6cm to 12cm, and their extent was classified as type I in 7(33.3%), type II in 8(38.1%), type III in 3(14.3%), and type IV in 3(14.3%) patients based on Crawford classification for TAA . Diseased aorta was replaced with artificial vascular graft in all but one patient. In whom the aortic tear site due to pseudoaneurysm was closed by primary suture. For the spinal cord protection during the operation, we used partial cardiopulmonary bypass (FV-FA or PA-FA bypass) in 12 patients (57.1%), Biopump (LA-FA bypass) in 4(19.0%), total circulartory arrest and CPB in 2 (9.5%), Gott's heparinized shunt in 1(4.7%), and simple aortic cross clamping in 2 (9.5%). The most common complication after the operation was hoarseness due to unilateral vocal cord palsy which onured in 5 patients (23.8%), and the next common complication was wound infection in 4 patients(19.0%), paraplegia in 2 patients (9.5%), chylothorax in 1 patient(4.7%). The hospital mortality rate was 9.5% (2deaths), and there was no late death. Our experience shows that the graft replacement of TAAA had reasonable rate of mortality, low rate of serious complication, and provided good post operative state of the pati nts, and since the thoracoabdominal aortic operation is not a high risky procedure anymore, we recommend a radical operation for the indicated patients.

• Journal of Chest Surgery
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• v.35 no.6
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• pp.439-448
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• 2002

Pulmonary artery banding (PAB) in the functional univentricular heart (UVH) is a palliative procedure for staging toward the Fontan procedure; however, it is known to be a risk factor. Material and method： The records of all 37 patients with functional UVHs who underwent surgical palliation using PAB between September 1989 and August 1999 were reviewed retrospectively. We investigated the aortic arch obstruction, the development and progression of subaortic stenosis after PAB, and risk factor of mortality according to surgical method. Result： In 37 neonates and infants with single ventricular physiology, aortic arch obstruction was combined in 7. There were 6 early deaths (16.2%) after PAB and 3 late deaths (8.1%) after Fontan operation. The actuarial overall survival including early mortality at 3 and 5 years were 80.7$\pm$6.6%, 72.2$\pm$8.2% respectively. Among 31 patients who survived PAB, 27 patients (87.1 %) could become candidates for Fontan operation； 22 patients (71.0%) completed Fontan operation with 3 deaths and 5 were waiting bidirectional cavopulmonary shunt(BCPS) or Fontan operation (follow-up mean 4.5 year, minimal 2 year). Subaortic stenosis developed in 8 patients after PAB (8/29, 27.6%)； 3 cases in the patients without arch anomaly (3/22, 13.6%) and 5 in those with arch anomaly (5/7, 71.4%). The subaortic stenosis was managed with Damus-Kaye-Stansel procedure (DKS) in 6 patients without operative mortality and conal septum resection in 2 without long-term survivor. Analysis of risk factors established that aortic arch obstruction was strongly associated with subaortic stenosis (p＜0.001). The only risk factor of late mortality was Fontan procedure without staged palliation by BCPS (p=0.001). Conclusion： PAB is effective as an initial palliative step in functional UVH. And the high risk group of patients with aortic obstruction can undergo effective short-term PAB as an initial palliative step, with subsequent DKS for subaortic stenosis. This strategy, initial PAB and careful surveillance, and early relief of subaortic stenosis can maintain acceptable anatomy and hemodynamics for later Fontan procedures.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.6 no.2
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• pp.129-139
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• 2003

Purpose: The aim of the study was to evaluate the long-term outcome of glycogen storage disease (GSD) type 1 with particular reference to hepatic adenoma and hepatocellular carcinoma, and to analyze risk factors affecting the development of hepatic adenoma in GSD type 1. Methods: Forty-three GSD type 1 patients (31 males and 12 females, mean age $13.9{\pm}6.4$ years) were analyzed retrospectively. Hepatic adenoma was detected on abdominal USG and diagnosed on histologic examination. Clinical profiles were compared between patients with hepatic adenoma (n=16) and age-matched controls without hepatic adenoma (n=16). Results: 1) Of 43 GSD type 1 patients, 16 (37.2%) had hepatic adeoma. Hepatic adenoma was detected at the age of mean $14.2{\pm}4.1$ years (range: 7.9~25.7 years). Fourteen (87.5%) adenomas were multiple at detection. 2) Comparison of the clinical profiles between adenoma group and non-adenoma group revealed that age at first introduction of uncooked cornstarch treatment was significantly late in adenoma group compared with non-adenoma group ($9.1{\pm}5.2$ years vs. $3.0{\pm}1.8$ years, p=0.003). Portocaval shunt surgery was performed in 11 (68.8%) patients in adenoma group and 3 (18.8%) in non-adenoma group (p=0.004). Hepatic adenoma developed mean $5.8{\pm}4.2$ years after shunt operation. 3) One patient was diagnosed as hepatocellular carcinoma at the age of 25.7 years. Conclusion: Hepatic adenoma is an important late complication of GSD type 1 with the risk of malignant transformation. Early introduction of cornstarch therapy with strict metabolic control is needed to prevent the development of hepatic adenoma in GSD type 1.

• Journal of Chest Surgery
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• v.37 no.2
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• pp.154-159
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• 2004

The arteriovenous fistula (AVF), which maintains satisfactory blood flow, is necessary to the patients of end-stage renal disease for the long term hemodialysis. We performed the snuffbox fistula as the first operation for hemodialysis vascular access. This study was performed to investigate the patency rates, complications, risk factors for occlusion of the AVF, and the types of reoperations. Material and Method: We performed 146 snuffbox fistulas from Jun. 1994 to Dec. 2001 The records of the patients except six patients who were lost from follow up were analyzed retrospectively, Mean age and male:female ratio were 52$\pm$15 years (range, 17∼79 years) and 80 : 60 respectively. Diabetes mellitus and hypertension were combined in 47 patients and 101 respectively. Preoperative levels of creatinine and potassium were 9.09$\pm$3.68 mg/dL (range, 2.55∼20.09 mg/dL) and 4.7$\pm$0.9 mmol/L (range, 2.3∼8.1 mmol/L). One hundred thirteen cases of the snuffbox fistulas were done at left side hand and the others at right hand. Result: Mean follow up period of the patients was 41.8$\pm$31.0 months (range, 0.2∼108,8 months). During the follow up period, 35 occlusions of AVF occurred and these AVFs were patent for 9.8$\pm$10.1 months (range, 0.1∼40.4 months). The patency rates of f month, and 1, 2, 3, 5 years were known as 92.8, 80.2, 73.8, 71.3, 69.6% respectively. Right sided snuffbox fistulas (p-value=0.045) and old age (p-value=0.048) were revealed as significant risk factors for occlusion of AVF. The postoperative complications consisted of occlusions of AVF caused by intimal hyperplasia of vein in 24, thrombosis in nine, stenosis of anastomosis site in three, and venous hypertensions in two. After the first operation 37 patients underwent 86 reoperations. Conclusion: The snuffbox fistulas showed acceptable patency rates and low complication rates. The snuffbox fistulas as the first operation for AVF formation can be a good option for the patients with end-stage renal disease.

• Journal of Chest Surgery
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• v.24 no.2
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• pp.123-134
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• 1991

Twenty eight patients had undergone repair of an isolated complete atrioventricular septal defect between April 1986 and September 1990 in Seoul National University Children`s Hospital. The group comprised 13 male and 15 female patients. They ranged in age from 2 months to 8 years[mean 18.6months] and in weight from 3. 4kg to 23kg[mean 9.0$\pm$4.6kg]. They were analysed as Rastelli type A in 17 patients, Rastelli type B in 2 patients, and Rastelli type C in 9 patients. Seven patients had concomitant Down`s syndrome. All patients had large left-to-right shunt[mean pulmonary to systemic flow ratio 3.5 $\pm$2.2 ranging from 0.68 to 10.0] and high pulmonary systolic pressure[mean 74$\pm$18.8mmHg, ranging from 35 to 110]. In 11 patients, one patch technique was used to close the atrial and ventricular septal defect and 16 patients were undergone by two patch technique. We urgently managed only one patient by pulmonary artery banding whose anatomy was Rastelli type C and severe mitral regurgitation was identified. Postoperative complete A - V block was noted in 3 patients, two of whom were dead in operating room due to combined LVOTO and myocardial failure, and one patient with Rastelli type C was undergone by VVI type permanent pacemaker insertion 1wk later after two patch technique, but we had to manage him by modified Konno operation and total correction due to LVOTO and VSD leakage and severe mitral regurgitation 3 years later. Another two reoperation cases due to severe mitral regurgitation after two patch technique were undergone, one of whom we managed by mitral annuloplasty 3 months later but aggravated mitral regurgitation made us to control him by MVR 3 months later. Another one case of VSD leakage and tricuspid regurgitation was managed by total correction but she died of respiratory insufficiency 14 days later. We experienced pulmonary hypertensive crisis in 3 patients, who were dead in two cases comparing with one control case. So operative mortality is 9/27[33.6%], in one patch group of 3/11[29.2%] comparing with two patch group of 6/16[37.5%]. In summary, causes of death were pump weaning failure, myocardial failure and low cardiac output syndrome and pulmonary hypertensive crisis, resp. failure, complete AV block. Mean follow up period is 15.8$\pm$10.7 months[ranging from 3months to 37 months]