• Journal of Chest Surgery
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• 제16권2호
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• pp.147-156
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• 1983

Over a period from May, 1977 to SEptember, 1982, 101 cases ofopen heart surgerywere done under cardiopulmonary bypass. There were 50 male and 51 female patients, and the ages of the patients ranged from 19 months to 48 years. Sixty-nine cases were congenital heart disease and 32 cases were acquired heart disease, which consisted of 30 valvular disease, 1 IVC obstruction, and 1 myxoma. Among the 30 cases of valvular disease, 12 MVR, 4 MVR+TAP, 2 MVT+AVR, 1 MAP, and 11 OMC were done. There were 3 operative deaths (17.5%) in 16 MVR, 1 in 2 MVR+AVR, and 1 in 11 OMC. Operative mortality in 69 congenital heart disease was 13.0% ; 3 deaths (6.7%) in 45 acyanotic and 6(25.0%) in 24 cyanotic cases. The overall mortality for 101 cases was 14.8%; 13.0% for congenital and 18.8% for acquired heart disease.

• Clinical and Experimental Pediatrics
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• 제49권9호
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• pp.917-929
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• 2006

Over the last several decades there has been a remarkable change in the therapeutic strategy of congenital heart disease. Development of new tools and devices, accumulations of experience, technical refinement have positively affected the outcome of interventional treatment. Many procedures including atrial septostomy, balloon valvuloplasty, balloon dilation of stenotic vessel with or without stent implantation, transcatheter occlusion of abnormal vascular structure, transcatheter closure of patent arterial duct and atrial septal defect, are now performed as routine interventional procedures in many institutes. In diverse conditions, transcatheter techniques also provide complementary and additive role in combination with surgery. Intraoperative stent implantation on stenotic vessels, perventricular device insertion, and hybrid stage 1 palliative procedure for hypoplastic left heart syndrome have been employed in high risk patients for cardiac surgery with encouraging results. Transcatheter closure of ventricular septal defect has been performed safely showing comparable result with surgery. Investigational procedures such as percutaneous valve insertion and valve repair are expected to replace the role of surgery in certain group of patients in the near future. Continuous evolvement in this field will contribute to reduce the risk and suffering from congenital heart disease, while surgery will be still remained as a gold standard for significant portion of congenital heart disease.

• Clinical and Experimental Pediatrics
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• 제53권2호
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• pp.190-194
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• 2010

목 적 : 애성 및 음성 변동은 심장병 환자에서 관찰될 수 있는데 이러한 음성 변동 정도를 음향학적 지표의 평가로 분석하여, 소아에서 선천성 심장병 종류에 따른 음성 변동을 음향학적 요소로 분석하고자 하였다. 방 법 : 수술 전 선천성 심장병 환아 94명을 연구 대상으로 하였고 심실 중격 결손, 동맥관 개존, 심방 중격 결손, 폐동맥 협착, 활로 사징이 포함되었으며, 음성 변동을 조사하기 위한 음향 지표 분석은 통증 자극을 통하여 얻어진 음성 자료를 multi-dimensional voice program system을 이용하여 평가하였다. 연구에 사용된 음향 지표로 average fundamental frequency, length of analyzed sample, jitter percent, shimmer, noise to harmonic ratio를 분석하였다. 결 과 : 음향 지표인 average fundamental frequency은 동맥관 개존, 심실 중격 결손, 활로 사징 경우에서 유의한 감소를 나타냈으며, length of analyzed sample은 연구 대상 선천성 심장병 환아에서 대조군에 비해 의미있는 차이를 나타내지 않았다. Jitter percent, shimmer, noise to harmonic ratio는 심실 중격 결손, 동맥관 개존, 심방 중격 결손에서 대조군에 비해 증가되었다. 이러한 음성 변동은 연구 대상 선천성 심장병 중 동맥관 개존에서 음도변이, 강도변이, 음성의 소음 정도가 가장 뚜렷하게 나타났으며, 동맥관 개존, 심실 중격 결손, 심방 중격 결손 순으로 그 음성 변동의 특징을 나타냈다. 결 론 : 수술 전 선천성 심장병 환아에서 음성을 음향학적 지표로 비교 분석한 결과 음향학적 지표들이 심장 질환의 유형별 차이를 보이는데 좌우 단락 심질환군에서 현저한 비정상적 음향학적 지표를 나타냈다.

• 대한유전성대사질환학회지
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• 제22권2호
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• pp.53-57
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• 2022

다양한 아미노산 및 지질 대사 질환에서 피부와 모발의 변화가 관찰된다. 탈모증이 관찰되는 경우 장병성 선단피부염(아연 대사 장애), 비오티니다아제 결핍증 (비타민 B), 다발성 카르복실라제 결핍증, acrodermatitis acidemica 등 아미노산 및 비타민 대사 결함을 의심해볼 수 있다. 또한 부서지기 쉬운 모발이 관찰되는 경우 아르기니노숙신산뇨증 또는 시트룰린혈증 및 점액다당증을 의심해볼 수 있다. 건조하고 두꺼워진 인설을 가진 피부 또는 비늘증은 중성지질 축적 질환, 지방산 대사 장애, 콜레스테롤 합성 및 대사 장애와 관련하여 나타날 수 있다. 수포성 병변은 장병성 선단피부염, 비오티니다아제 결핍증, 홀로카르복실라제 합성효소 결핍증, acrodermatitis acidemica 등에서 나타날 수 있다.

• Journal of Chest Surgery
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• 제26권2호
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• pp.115-121
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• 1993

Between March, 1988 and July, 1992, 114 patients underwent Open Heart Surgery under hypothermic cardiopulmonary bypass. There were 29 cases of congenital heart anomalies (25％), and 85 cases of acquired heart diseases (75％) consisting of 53 cases of valvular heart disease, 31 cases of ischemic heart disease, and a case of left atrial myxoma. The age distribution of 114 cases was 4 to 73 years, and mean age was 43 years old consisting of mean age of congenital heart disease 23, valvular heart disease 47, and ischemic heart disease 57 years old. Overall operative mortality was about 7.9％.

• Journal of Chest Surgery
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• 제28권2호
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• pp.193-195
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• 1995

Congenital lobar emphysema is an uncommon disease affecting newborns and infants with respiratory distress. This report describes a 1.9kg premature baby of 31weeks gestation who developed respiratory distress symptoms of congenital lobar emphysema involved left upper lobe.Left upper lobe lobectomy was carried out and good result was obtained. So, we present one case report with literature review.

• Tuberculosis and Respiratory Diseases
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• 제78권4호
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• pp.450-454
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• 2015

Although tuberculosis is highly prevalent worldwide, congenital tuberculosis is one of the least common manifestations of the disease. The diagnosis is usually difficult because of the non-specific clinical presentation and the lack of awareness of maternal disease prior to pregnancy and delivery. We present the case of a preterm neonate with congenital tuberculosis, born to a previously healthy mother who had developed severe disseminated tuberculosis during her pregnancy. Once the diagnosis was confirmed in the mother, the congenital infection was confirmed by isolation of Mycobacterium tuberculosis in gastric aspirates, and positive polymerase chain reaction in a cerebrospinal fluid examination. Treatment for tuberculosis with a four-drug regimen resulted in an adequate clinical response in both the mother and infant.

• Clinical and Experimental Pediatrics
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• 제49권11호
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• pp.1125-1139
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• 2006

In 1991, the Ministry of Health & Social affairs adopted a nationwide service program for neonatal screening of phenylketonuria, galactosemia, maple syrup urine disease, homocystinuria, histidinemia & congenital hypothyroidism for newborns delivered from low class pregnant women registered in health centers. Government decreased the test items from six to two, PKU & congenital hypothyroidism to increase test numbers with same budget from 1995. Government decided to test PKU & hypothyroidism for all newborns from 1997. 78 laboratories wanted to participate for neonatal screening test in 1999. Government didn't decide laboratory center for a certain district and placed responsibility on free competition. Government are planning to test 573,000 newborns from 1998, Government decided to screen 6 items PKU, congenital hypothyroidism, maple syrup urine disese, homocystinuria, galactosemia and congenital adrenal hyperplasia from 2006. 17 laboratores are participating now. The cost of screening test is supported by both the federal government and local government on a 40-60 basis. In case a patient with an inherited metabolic disease is diagnosed by screening of government program, special milk is provided at government's expense. Interlaboratory quality control was started 6 times a year from 1994. According to the government project, 3,707,773 newborns were screened. 86 PKU, 718 congenital hypothyroidism were detected. So incidence of PKU is 1/43,114 and congenital hypothyroidism is 1/4,612. Maeil dairy company produced new special formula for PKU, MMA and PA, MSUD, urea cycle disorder, homocystinuria, isovaleric acidemia from Oct. 1999. The cost benefit of performing screening procedures coupled with treatment has been estimated to be as high as 1.77 times in PKU, 11.11 times in congenital hypothyroidism than cost without screening. We are trying to increase the budget to test all newborns for Tandem mass sereening & Wilson disease from 2008. Now it is a very important problem to decrease laboratory numbers of neonatal screening in Korea. So we are considering 4-5 central laboratories which cover all newborns and are equipped with tandem mass spectrometer & enzyme immunoassay for TSH, 17OHP & enzyme colorimetric assay for galactose.

• Clinical and Experimental Pediatrics
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• 제55권8호
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• pp.297-300
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• 2012

Symptomatic pulmonary arterial hypertension (PAH) in patients with isolated atrial septal defect (ASD) is rare during infancy. We report a case of isolated ASD with severe PAH in an infant who developed airway obstruction as cardiomegaly progressed. The patient presented with recurrent severe respiratory insufficiency and failure to thrive before the repair of the ASD. Echocardiography confirmed volume overload on the right side of heart and severe PAH (tricuspid regurgitation [TR] with a peak pressure gradient of 55 to 60 mmHg). The chest radiographs demonstrated severe collapse of both lung fields, and a computed tomography scan showed narrowing of the main bronchus because of an intrinsic cause, as well as a dilated pulmonary artery compressing the main bronchus on the left and the intermediate bronchus on the right. ASD patch closure was performed when the infant was 8 months old. After the repair of the ASD, echocardiography showed improvement of PAH (TR with a peak pressure gradient of 22 to 26 mmHg), and the patient has not developed recurrent respiratory infections while showing successful catch-up growth. In infants with symptomatic isolated ASD, especially in those with respiratory insufficiency associated with severe PAH, extrinsic airway compression should be considered. Correcting any congenital heart diseases in these patients may improve their symptoms.

• Journal of Chest Surgery
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• 제17권2호
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• pp.305-314
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• 1984

From May 1977 to April 1984, 204 cases of open heart surgery were performed under cardiopulmonary bypass. There were 99 male and 105 female patients ranging in age from 19 months to 58 years. 136 cases [66.7%] were congenital heart disease, and 68 cases [33.3%] were acquired heart disease, which were 66 valvular disease [97.1%], 1 IVC obstruction, and 1 myxoma. There were 136 congenital heart anomaly with 16 operative deaths [11.8%], consisting of 94 acyanotic cases with 7 death [7.4%] and 42 cases of cyanotic cases with 9 deaths [21.4%]. In 66 patients of acquired valvular disease, 52 valves were implanted; 47 mitral valve replacement with 4 death [8.5%] and 5 double valve replacement [MVR+AVR] with 1 death [20%]. Postoperative, warfarin sodium was medicated with checking prothrombin time. Finally, the operative mortality was 11.8% in congenital anomaly, and 11.8% in acquired heart disease, overall mortality rate was 8.5%.