• Tuberculosis and Respiratory Diseases
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• v.44 no.4
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• pp.907-913
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• 1997

Background : Congenital bronchoesophageal fistula(BEF) presented in adult life is a rare disorder and has characteristic clinical findings such as paroxysmal cough after water ingestion and recurrent respiratory infections. It usually manifested recurrent pneumonia and chronic cough with purulent phlegmon which was mis-or under-diagnosed as chronic bronchitis, bronchiectasis or lung abscess so forth. Methods : We reviewed retrospectively 13 cases of congenital BEF in adult of Paik Hospital, College of Medicine, Inje University including 22 cases of congenital BEF previously reported in literature of Korea from 1979 through 1995. Results : The mean age at diagnosis was $40.2{\pm}14.3$. There was no difference in sex ratio(Male : Female 18 : 17). The most common symptom was cough(91.4%), followed by chronic sputum(74.3), hemoptysis(25.7), and paroxysmal nocturnal cough at specific position(20%). Twenty one of 31 patients who were able to review have the most specific sign, Ono's sign presented as paroxysmal cough after liquid ingestion. By classification of Braimbridge-Keith, Fourteen(45.1%) of 31 patients were group I (associated with esophageal diverticulum), 15(48.4%) were group II (simple fistula), and group Ill and IV was one case in each. The opening of fistula confined to right lower lobe in 26(76.5%), left lower lobe in 6(17.6%), and left main bronchus in 2(5.9%) cases. Conclusion : Congenital bronchoesophageal fistula is uncommon disorder which has characteristic histories and specific symptoms such as chronic and recurrent lower respiratory infections, and paroxysmal cough after liquid ingestion. Medical attention and careful history should be done in patients who have localized recurrent lower respiratory infections in right lower lobe.

• Journal of Chest Surgery
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• v.36 no.3
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• pp.136-141
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• 2003

We assessed the intermediate-term result of tricuspid annuloplasty (TAP) for tricuspid valve regurgitation (TR) associated with congenital heart disease in adults. Risk factors for residual TR were also analysed. Material and Method: From August 1989 to June 2001, seventy three adult patients, 51 females and 22 males, underwent TAP for TR associated with various congenital heart disease. Their age ranged from 46 years to 73 years (mean:43). Associated heart anomalies were atrial septal defect (55), ventricular septal defect (6), partial anomalous pulmonary venous return (4) and others (8). Preoperative and post-operative TR velocities were 3.25 m/sec and 2.56 m/sec respectively, and the types of TAP were De Vega in 43, Kay in 18 and Ring annuloplasty in 12. Postoperative follow-up duration was 2,347 patient-month (mean: 32.6 months), and 134 two-dimensional echocardiographic examinations were done during this period. Residual TR greater than III/IV was considered as TAP failure. Result: TAP failure was observed in 7 patients (9.6%), and one patient among them underwent tricuspid valve replacement. Risk factors for TAP failure were diagnosis other than atrial septal defect (p=0.001), preoperative (p=0.038) and postoperative (p=0.028) high TR velocity. There was no statistical significance in terms of TAP methods. Conclusion: Careful evaluation of valve morphology and aggressive surgical intervention are mandatory for the repair of TR with preoperative or residual RV pressure overload.

• Korean Journal of Cleft Lip And Palate
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• v.2 no.1_2
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• pp.29-41
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• 1999

Cleft lip and palate is the most frequent congenital facial deformity of the orofacial area. Successful management of patients with cleft lip / palate requires a multidiciplinary approach from birth to adult stage. Coordinated treatment by the cleft palate team is an essential requirement to obtain optimum treatment results. One of the negative effect of the early surgical interventions of lip and palate is a significant incidence of maxillary growth restriction that produces secondary deformities of the jaws and malocclusion that includes congenital missing of lateral incisor, malformed teeth, rotation or ectopic position of upper anterior teeth, and it has been thought due to the resistance of palatal scar tissue. In Orthodontic treatment for cleft lip / palate patients, expansion of upper dental arch or palatal suture is often needed to correct posterior and/or anterior cross bite and align upper teeth. Various appliances such as hyrax, quad-helix, fan-type expansion screw and jointed-fan type expander can be used for palatal expansion. In the orthodontic treatment of the cleft lip / palate patient, we must consider patient age and severity of palatal constriction for proper appliance selection, and must pay special attention to maintain the treatment results.

• The Korean Journal of Pharmacology
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• v.28 no.1
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• pp.49-60
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• 1992

This study was designed to evaluate the responses of cardiovascular system to endothelin (ET) and neuropeptide Y (NPY) in 12 week-old SHR treated with or without enalapril (ENP) for 6 weeks. The diastolic blood pressure and heart rate were lower in ENP-treated SHR than in control. The pressor response to intravenous, but not intracerebroventricular, ET or NPY was attenuated by ENP treatment. The chronotropic action induced by electrical stimulation was attenuated by ENP or ET. The negative chronotropic action of ET was blocked by yohimbine. The increase in aortic tension induced by electrical field stimulation (EFS) was depressed in ENP-treated group as compared with non-treated group, and enhanced by ET, but not NPY, in the non-treated group. The ET-induced increase in tension was enhanced by removal of endothelium in the control group but not in ENP-treated group. The plasma concentration of norepinephrine and ET-induced increase in concentration of norepinephrine and epinephrine in plasma were decreased in ENP-treated group. These results suggest that preventive effect of enalapril on the development of hypertension may result from depressing vasoactive action of endothelin and neuropeptide Y, and sympathetic neurotransmission at peripheral nervous system.

• Childhood Kidney Diseases
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• v.2 no.2
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• pp.161-168
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• 1998

Purpose : Lots of congenital anomalies of urinary tract including hydronephrosis are detected in fetus and neworn by popular use of prenatal ultrasonography. But there are little data available in Korea about natural course of hydronephrosis diagnosed antenatally by ultrasonography. So we intended to help management of these patients by analizing the follow up data of the neonates with hydronephrosis diagnosed antenatally. Methods : We evaluated 22 patients with neonatal hydronephrosis(33 renal units) who were diagnosed prenatally and confirmed postnatally. Especially patients with suspected ureteropelvic junction obstruction were followed regulary with renal ultrasonography and diuretic renography for 8-24 months. Results : 1) The etiologies of neonatal hydronephrosis diagnosed prenatally were suspected ureteropelvic junction obstruction($69.9\%$), vesicoureteral reflux($15.1\%$), primary megaureter($3.0\%$), double ureter with ureterocele($3.0\%$), ureteral stricture($3.0\%$), multicystic dysplastic kidney(3.0$\%$), and ureterovesical junction obstruction(3.0$\%$). 2) The follow up results of 23 renal units of suspected ureteropelvic junction obsruction: Except 4 renal units with palpable abdominal mass, of the remained 19 renal units, 14 units($73.6\%$) were improved spontaneously, 3 units($15.7\%$) remained stable, only2 units($10.5\%$) were aggravated. Conclusion : We concluded that in most cases of hydronephrosis there is no need for immediate surgery, and that nonoperative approach, using serial ultrasonography and diuretic renogram, is safe management of neonatal hydronephrosis diagnosed anteratally.

• Neonatal Medicine
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• v.18 no.1
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• pp.14-22
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• 2011

Neonatal bleeding is a common problem encountered in nursery rooms or neonatal intensive care units, especially among premature infants. Furthermore, owing to recent remarkable improvement of neonatology, survival rates of preterm neonates have increased; hence, neonatal bleeding cannot be emphasized enough. Since the total blood volume of neonates is small, bleeding can be one of the causes of morbidities and mortalities. Therefore, rapid diagnosis and immediate therapy is urgently needed. The patient's medical history including a familial history of a bleeding disorder or of a previously affected infant who suffered from bleeding along with maternal and neonatal drugs can provide important diagnostic clues. Presence of bleeding with or without petechiae and ecchymoses in a healthy term or late preterm infant with thrombocytopenia but normal prothrombin time and activated partial thromboplastin time strongly suggests a congenital bleeding disorder. For a sick infant who is bleeding from multiple sites, an acquired disorder such as disseminated intravascular coagulation is suspected. Intracranial hemorrhage in term or late preterm infants without a history of birth trauma is highly suggestive of coagulation disorders. The purpose of this review is to summarize recent advances in diagnostic methods is as well as basic concepts of neonatal hemostatic disorders. First, an outline of background information will be presented followed by a discussion of primary and secondary hemostatic disorders as well as inherited and acquired disorders.

• Proceedings of the Korea Water Resources Association Conference
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• 2004.05b
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• pp.731-736
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• 2004

강우-유출과정의 수문학적 현상을 보다 정확히 분석하고 예측하는 기법으로 강우에 의한 유출의 반응을 나타내는 지체시간, 도달시간 등 수문학적 반응시간을 유역의 지형형태학적 인자들과 연계하는 방법이 많이 이용되고 있다. 이에 본 연구에서는 Clark방법과 지형형태학적 순간단위도(GIUH)를 이용하여 계측유역의 강우-유출반응을 모의하였고, 이를 관측된 값과 비교하여 미계측유역의 적용성 여부를 검토해보았다. 대상 유역의 하상지형인자 및 지형형태학적 특성은 Arc-View를 이용하여 구하였으며, 이를 기존의 문헌자료와 비교해보았다. Clark방법의 매개변수의 결정에 있어서 시간-면적곡선은 HEC-1의 무차원 식을 이용하였고, 도달시간은 Kirpich 공식을 이용하여 구하였으며, 저류상수는 Clark방법에 의해 추정된 순간단위도의 첨두유량이 Horton의 차수비의 함수로 구한 철두유량과 같아지는 값으로 결정하였다. 본 연구는 전적비교를 출구점으로하는 유역면적 $8.5km^2$인 설마천을 대상유역으로 하였으며, 모의된 강우-유출반응과 비교하기 위해 사용된 강우사상은 2002년의 8월 4일과 2002년 10월 6일의 10분 단위 우량이다. Clark방법과 GIUH를 이용하여 모의한 유출곡선과 관측된 유출곡선을 비교해본 결과 첨두유량은 8월의 강우사상 때는 $21\%$크게, 10월의 강우사상 때는 $35\%$작게 나타났다. 첨두시간은 모의된 경우가 각각 10분, 20분 빨리 도달하였다. 또한 이러한 결과는 유역의 도달시긴에 가장 민감하게 반응함을 알 수 있었다. 따라서, 유역의 도달시간 산정에 주의를 요한다면 프랙탈 차원이 유사한 미계측유역의 수문곡선 산정에 있어서 Clark방법과 GIUH를 이용하는 방법도 유용하다고 사료된다. 주는 각 수문인자 중 강우시간분포와 유효우량 산정방법 그리고 유출모형에 대해 자자 검토하였으며, 최종적으로 면적에 따른 임계지속기간과 유출량의 변화를 검토해 보았다.이를 각각의 경우의 해석해 결과와 비교${\cdot}$분석하였다. 후방추적 퍼프모형은 전방추적 퍼프모형에 비하여 사용된 퍼프수와 관계없이 작은 오차를 발생하였으며, 전체적으로 퍼프 모형이 입자모형보다는 훨씬 적은 수의 계산을 통해서도 작은 오차를 나타낼 수 있다는 것을 알 수 있었다. 그러나 Gaussian 분포를 갖는 퍼프모형은 전단흐름에서의 긴 유선형 농도분포를 모의할 수 없었고, 이에 관한 오차는 전단계수가 증가함에 따라 비선형적으로 증가하였다. 향후, 보다 다양한 흐름영역에서 장${\cdot}$단점 분석 및 오차해석을 수행한 후에 각각의 Lagrangian 모형의 장점만을 갖는 모형결합 방법을 제시할 수 있을 것으로 판단된다.mm/$m^{2}$로 감소한 소견을 보였다. 승모판 성형술은 전 승모판엽 탈출증이 있는 두 환아에서 동시에 시행하였다. 수술 후 1년 내 시행한 심초음파에서 모든 환아에서 단지 경등도 이하의 승모판 폐쇄 부전 소견을 보였다. 수술 후 조기 사망은 없었으며, 합병증으로는 유미흉이 한 명에서 있었다. 술 후 10개월째 허혈성 확장성 심근증이 호전되지 않아 Dor 술식을 시행한 후 사망한 예를 제외한 나머지 6명은 특이 증상 없이 정상 생활 중이다 결론: 좌관상동맥 페동맥이상 기시증은 드물기는 하나, 영유아기에 심근경색 및 허혈성 심근증 또는 선천성 승모판 폐쇄 부전등을 초래하는 심각한 선천성 심질환이다. 그러나 진단 즉시 직접 좌관상동맥-대동맥 이식술로 수술적 교정을 해줌으로써 좋은 성적을 기대할 수 있음을 보여주었다.특히 교사들이 중요하

• Journal of Chest Surgery
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• v.31 no.3
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• pp.304-307
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• 1998

Aneurysm of the sinus of Valsalva on mostly congenital disease that develops more frequently in Orientals, is very low in incidence. In most cases, aneurysm of the sinus of Valsalva extends to intracardiac and results in ruptures into the right ventricle or atrium. The likelihood of extracardiac aneurysm of the sinus of Valsalva is very low. Cases of extracardiac aneurysm are usually accompanied by aortic regurgitation and can cause right ventricle outflow tract obstruction, myocardial ischemia, and myocardial infarction due to compression by aneurysm. Since the aneurysm can rupture in the intrapericardium and cause cardiogenic shock or sudden death, definite diagnosis and management are important. If confirmed, it is preferable operform a surgical correction. We report here, with a literature review, a case where myocardial ischemia and aortic egurgitation caused by aneurysm developed in the left coronary and noncoronary sinus, and were surgically corrected with satisfactory esults.

• Journal of Chest Surgery
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• v.32 no.6
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• pp.504-509
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• 1999

Background: Heart transplantation is considerated for a selected certain group of complicated congenital heart disease in neonates because corrective surgery is very difficult and has high mortality. Precise planning of transplantation is necessary to adequately fit the donor heart to the recipient. Material and Method: We have performed 4 neonatal pig heart transplantations to test the technical feasibility. Experiment 1: The transplantation was performed using the same technique as the adult heart transplantation. Experiment 2: The transplantation for hypoplastic left heart syndrome was simulated as we reconstructed the whole aortic arch with donor aorta. Experiment 3: The heart transplantation was done with radical pulmonary artery reconstruction. Experiment 4: The experiment was performed for a long term survival. Result: Preoperative planning was very important for adequate fitting. All animals could be weaned from cardiopulmonary bypass, however, two animals died due to bleeding at pulmonary artery and left atrium. Conclusion: We concluded that the neonatal heart transplantation can be applied in some complicated Further using animal model is mandatory.

• Journal of the korean academy of Pediatric Dentistry
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• v.38 no.1
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• pp.56-61
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• 2011

Cornelia de Lange syndrome(CdLS) is a well-described multiple malformation syndrome typically involving proportionate small stature, developmental delay, specific facial features, major malformations, and behavioral abnormalities. Dental issues include micrognathia, crowding of teeth, small teeth, absent teeth, poor oral hygiene, and periodontal disease. We present a case of CdLS in a 10-year-old girl. Micrognathia, crowding of teeth, and bifid uvula are the characteristic features of this case. Dental treatments for this child including preventive and restorative procedures were performed under outpatient general anesthesia.