• Title/Summary/Keyword: Congenital defects

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Isolated Aortic Valve Replacement with the Ionescu-Shiley Xenograft Valve (대동맥판 치환수술의 임상성적)

  • 김종환
    • Journal of Chest Surgery
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    • v.18 no.1
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    • pp.46-53
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    • 1985
  • A single aortic valve replacement using the lonescu-Shiley bovine pericardial xenograft valve was performed in 66 consecutive patients during the period from February, 1979 to June, 1984. They were 49 males and 17 females with ages ranging from 9 to 61 [mean, 31.113.1] years, and 9 of them were children younger than 15 years of age. Twenty-seven patients [40.9%] required the combined operative procedures to either other valvular lesions or congenital defects. There were 9 early deaths within 30 days of surgery [operative mortality rate, 13.6%] and 2 late deaths thereafter [late mortality rate, 3.0%; or 1.75%/patient-year]. The 57 early survivors were followed for a total duration of 114.2 patient-years [mean, 24.016.0 months]. Four patients experienced thromboembolic complication with no death [3.50%/patient-year]; one died from intracranial bleeding related to anticoagulation [0.88%/patient-year]; one recovered from prosthetic valve endocarditis [0.88%/patient-year]; and four developed aortic regurgitant murmur with none or minimal cardiac symptoms and they were classified into cases of tissue valve failure [3.50%/patient-year]. The actuarial survival rate was 82.34.7% at 6 years, and the actuarial probabilities of freedom from thromboembolism and valve failure were 93.33.9% and 89.15.8% at postoperative 6 years respectively Symptomatic improvement was excellent in most late survivors at the follow-up end with the mean of NYHA Classes of 1.040.19 while the one was 2.290.67 at the time of operation. Excluding the higher operative mortality rates, these clinical results are fully comparable with the ones of reports from the major institutions using the porcine aortic or the bovine pericardial tissue valves and warrants the continued use of the xenograft valve in the aortic position. The importance of more detailed preoperative evaluation of the myocardial function and the need of improved myocardial preservation during surgery for the improved early clinical results were discussed.

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Plastic bronchitis in children: 2 cases (소아 증식성 기관지염 2례)

  • Kim, Yeo Hyang;Choi, Hee Jung;Kim, Jung Ok;Hyun, Myung Chul
    • Clinical and Experimental Pediatrics
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    • v.52 no.7
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    • pp.832-836
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    • 2009
  • Plastic bronchitis is a rare disorder characterized by the formation of extensive, obstructing endobronchial casts. It is associated with asthma and complex cardiac defects such as those requiring the Fontan procedure. The treatment of plastic bronchitis comprises conventional therapy involving spontaneous expectoration and bronchoscopic removal and specific therapy with several new drugs. Herein, we describe the cases of 2 patients diagnosed with plastic bronchitis accompanied with a different underlying disease, which were treated with inhaled corticosteroid and low-dose oral clarithromycin.

Clinical Significance of Uptake Difference on DMSA Scintigraphy in Pediatric Urinary Tract Infection

  • Kim, Byung Kwan;Choi, Won Jee;Yim, Hyung Eun;Yoo, Kee Hwan
    • Childhood Kidney Diseases
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    • v.20 no.2
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    • pp.63-68
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    • 2016
  • Purpose: Disruption of normal renal development can lead to congenital anomalies of the kidney and urinary tract, including renal hypodysplasia. We aimed to clarify whether small kidney size affects clinical manifestations in children with urinary tract infection (UTI). Methods: One hundred fifty-four patients who had their first symptomatic UTI between January 2014 and June 2015 were enrolled in this study. Differences in kidney size were estimated based on percent uptake of $^{99m}Tc-$ dimercaptosuccinic acid (DMSA) in scintigraphy. The patients who showed more than 10% difference in kidney size on DMSA scintigraphy with none or minimal cortical defects were included in group A. (group A, n=17). Laboratory, clinical, and imaging results were compared with those of the other patients (group B, n=137). Results: Group A had a relatively higher incidence of vesicoureteral reflux than group B (44% vs 20%, P<0.05). The levels of plasma neutrophil gelatinase-associated lipocalin (NGAL) and serum C-reactive protein were significantly higher in group A (193 [64-337] vs 91 [59-211] ng/mL and 4.1 [0.5-11.9] vs 2.1 [0.7-5.3] ng/mL, respectively; all P <0.05). Linear regression analysis revealed that plasma NGAL level strongly correlated with the difference in renal uptake in DMSA scintigraphy in group A ($R^2=0.505$). Conclusion: The difference in kidney size could influence the clinical course and severity of pediatric UTI.

Developmental Anomalies of Central Nervous System in Human

  • Chi, Je G.
    • Toxicological Research
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    • v.17
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    • pp.11-16
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    • 2001
  • The development of the central nervous system is a continuous process during the embryonic and fetal periods. For a better understanding of congenital anomalies of central nervous system, three major events of normal development, i.e., neurulation (3 to 4 weeks), brain vesicle formation (4 to 7 weeks) and mantle formation (over 8 weeks) should be kept in mind. The first category of anomalies is neural tube defect. Neural tube defects encompass all the anomalies arise in completion of neurulation. The second category of central nervous system anomalies is disorders of brain vesicle formation. This is anomaly that applies for "the face predicts the brain". Holoprosencephaly covers a spectrum of anomalies of intracranial and midfacial development which result from incomplete development and septation of midline structures within the forebrain or prosencephalon. The last category of central nervous system malformation is disorders involving the process of mantle formation. In the human, neurons are generated in two bursts, the first from 8 to 10 weeks and next from 12 to 14 weeks. By 16 weeks, most of the neurons have been generated and have started their migration into the cortex. Mechanism of migration disorders are multifactorial. Abnormal migration into the cortex, abnormal neurons, faulty neural growth within the cortex, unstable pial-glial border, degeneration of neurons, neural death by exogenous factors are some of the proposed mechanism. Agyria-pachygyria are characterized by a four-layerd cortex. Polymicrogyria is gyri that are too numerous and too small, and is morphologically heterogeneous. Cortical dysplasia is characterized by the presence Q[ abnormal neurons and glia arranged abnormally in focal areas of the cerebral cortex. Neuroglial malformative lesions associated with medically intractable epilepsy are hamartia or hamartoma, focal cortical dysplasia and microdysgenesis.ysgenesis.

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Corrected transposition of the great arteries: surgical treatment of associated anomalies (교정형 대혈관전위증동반된 심혈관기형의 수술요)

  • Kim, Gi-Bong;No, Jun-Ryang;Seo, Gyeong-Pil
    • Journal of Chest Surgery
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    • v.17 no.3
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    • pp.371-380
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    • 1984
  • Corrected transposition of the great arteries [C-TGA] is one of the rare congenital heart disease in which there is both a discordant atrioventricular relationship and transposition of the great vessels. With this arrangement, systemic venous blood passes through the right atrium into the morphologic left ventricle and out the pulmonary artery. Pulmonary venous blood returns to the left atrium, flows into the morphologic right ventricle and out the aorta. Thus, in the rare case when no additional cardiac anomaly is present, a hemodynamically normal heart exists. But more often they are symptomatic as a result of one or several of the commonly associated defects. This paper describes 13 patients who underwent repair of one or more cardiac anomalies associated with corrected transposition at SN UH, from June 1976 through June 1984. 1.8 were males and 5 females, with ages ranging from 3 years to 27 years. 2. Segmental anatomy was {S,L,L} in 12, or {I,D,D} in 1. 3.Associated anomalies were ventricular septal defect in 10, pulmonary outflow tract obstruction in 6, tricuspid insufficiency in. 4, atrial septal defect in 3, subaortic stenosis in 1, mitral insufficiency in 1, and patent ductus arteriosus in 1. 4.None had complete heart block preoperatively, and 3 developed complete heart block intraoperatively. But one of them recovered sinus rhythm on the postoperative 7th day spontaneously. 5.There were 3 cases of hospital morality. But there was no morality since Dec. 1980. 6.Patients with single ventricle, hypoplastic ventricle or those who had palliative surgery alone are not included in this review.

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Efficacy of the Maze Procedure for Atrial Fibrillation Associated with Atrial Septal Defect

  • Shim, Hunbo;Yang, Ji-Hyuk;Park, Pyo-Won;Jeong, Dong Seop;Jun, Tae-Gook
    • Journal of Chest Surgery
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    • v.46 no.2
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    • pp.98-103
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    • 2013
  • Background: Atrial fibrillation (AF) is a common complication in elderly patients with atrial septal defect (ASD). The purpose of this study was to examine the efficacy of the maze procedure in these patients. Materials and Methods: Between February 2000 and May 2011, 46 patients underwent the maze procedure as a concomitant operation with ASD closure. Three patients who underwent a right-sided maze were excluded, and one patient was lost to follow-up. The mean follow-up duration was $3.2{\pm}2.5$ years. Electrocardiography was performed 1 month, 3 months, 6 months, and 1 year after surgery, and checked annually after that. Results: AF persisted in 4 patients after surgery. One year after surgery, among 38 patients, 55.3% remained in sinus rhythm without antiarrhythmic drugs. However, when including the patients who took antiarrhythmic drugs, 92.1% were in sinus rhythm. Freedom from AF recurrence at 3 months, 6 months, 1 year, 2 years, 3 years, and 5 years after surgery were $97.4{\pm}2.6$, $94.4{\pm}3.8$, $91.2{\pm}4.9$, $87.8{\pm}5.8$, $79.5{\pm}7.6$, and $68.2{\pm}12.4$, respectively. There was no early mortality after operation. Conclusion: Concomitant treatment with the maze procedure and ASD closure is safe and effective for restoring the sinus rhythm.

Two Cases of Surgically Created Aneurysms of the Sinus of Valsalva (Valsalva 동의 선천성 동맥류 파열: 2례 수술 경험)

  • 이성행
    • Journal of Chest Surgery
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    • v.10 no.1
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    • pp.133-139
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    • 1977
  • Two cases of congenital aneurysm of sinus of Valsalva, ruptured into the right ventricle, and associated with ventricular septal defects, were undergone intracardiac repair with the aid of extracorporeal circulation using Bentley bubble oxygenator and moderate hypothermia. Case 1. A 20 year old male, with the chief complaints of palpitation and dyspnea, was admitted to Kyungpook National University Hospital on Dec. 16, 1976. Continuous machinery murmur was heard best at left 3rd. intercostal space along the sternal border. Retrograde aortography disclosed aneurysm of the right coronary cusp, which ruptured into the right ventricle. Utilizing cardiac bypass and moderate hypothermia, the right ventricle was opened and aneurysm was closed by direct sutures. Associated ventricuar septal defect was directly ,closed and suture line was reinforced by Dacron patch. Total bypass time was 112 minutes and total aortic cross clamping time was 37 minutes. Assist ventilation was carried out for 28 hours postoperatively. His postoperative course was smooth except removal o1 substernal hematoma and he was .discharged on 24th postoperative day. Case 2. A 28 year old man was admitted to our Hospital on June 9, 1976. two weeks prior to this admission, suddenly he had collapsed while he was walking on the street. Following `this episode, palpitation, dyspnea on exertion and frequent respiratory infection developed. Grade IV systolic murmur was heard best at 3rd intercostal space along the sternal border. Retrograde aortography confirmed the diagnosis of rupture of aneurysm of the sinus Valsalva ruptured into the right ventricle. Under the cardiopulmonary bypass the right ventricle was opened and ruptured aneurysm and infracristal ventricular septal defect were directly closed and reinforced with Dacron patch. Postoperative course was uneventful and he was discharged on 14th postoperative day. The pathogenesis of aneurysm of the sinus Valsalva and mode of diagnosis were discussed. Principle of surgical repair was presented.

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Hybrid Procedure for Muscular Ventricular Septal Defects -2 case reports- (근육형 심실중격결손에 대한 하이브리드 수술법 -2예 보고-)

  • Choi, Seon-Uoo;Yang, Ji-Hyuk;Jun, Tae-Gook;Park, Pyo-Won;Min, Sun-Kyung;Kang, I-Seok
    • Journal of Chest Surgery
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    • v.41 no.6
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    • pp.747-750
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    • 2008
  • Although surgical closure is the standard approach for a muscular ventricular septal defect, the procedure may be complicated by poor visualization and the need for incision on the ventricle. Another approach is, catheter-based intervention. However, it also has limitations. A hybrid procedure, the intraoperative combined use of an interventional device may reduce the procedure's invasiveness. We successfully managed two cases of muscular ventricular septal defect with a hybrid procedure. We report here on these 2 cases along with a review of the literature.

Practical Communication Strategies to Improve the Surgical Outcomes in a Pediatric Cardiac Intensive Care Unit (소아심장외과 중환자실에서의 실무의사소통 프로토콜이 수술 후 성과에 미치는 영향)

  • Uhm, Ju-Yeon;Lee, Worlsook
    • Journal of Korean Academy of Nursing Administration
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    • v.21 no.3
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    • pp.243-253
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    • 2015
  • Purpose: The purpose of this study was to identify the impact of practical communication strategies (PCS) on the reduction of AEs (Adverse Events) in pediatric cardiac ICU (PCICU). Methods: Intra-operative findings and care plans were documented and shared between staff members on a daily basis from the day of operation to the day of general ward transfer. Incidence of AEs was investigated in all patients who were admitted to the PCICU and was compared with incidence of AEs one year after establishment of PCS. Results: The study population consisted of 216 patients in pre-PCS group and 156 patients in post-PCS group. Incidence of readmission decreased from 6.0% (13/216) in pre-PCS group to 0.6% (1/156) in post-PCS group (${\chi}^2=7.23$, p=.010). Incidence of other major complications decreased from 4.2% (9/216) to 0.6% (${\chi}^2=6.66$, p=.012). Minor AEs such as intervention omission, order error, and protocol misunderstanding were reduced from 23.3 cases per 100 patient-days to 7.5 cases per 100 patient-days (${\chi}^2=20.31$, p<.001). Conclusion: Handover protocol is an effective strategy to reduce AEs for critically ill patients after pediatric cardiac surgery. Efforts to develop effective communication strategies should be continued and outcome research about communication strategies for patient safety should be further studied.

ORAL CHARACTERISTIC OF ALAGILLE SYNDROME - A CASE REPORT (Alagille 증후군을 가진 환자의 구강내 특징에 대한 증례보고)

  • Kim, Tae-Wan;Kim, Young-Jin
    • The Journal of Korea Assosiation for Disability and Oral Health
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    • v.3 no.1
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    • pp.17-21
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    • 2007
  • Alagille syndrome is an autosomal dominant genetic disorder and occurs in approximately 1 in 100,000 live births. Diagnostic criteria was established by Alagille. It is mainly caused by a mutation in the Jagged1 gene. Major clinical features of this syndrome are paucity of intrahepatic bile duct with cholestasis, characteristic facies, cardiac murmur, defects of vertebrae, and embryotoxon. And minor clinical features are mental retardation, renal involvement, growth retardation, other skeletal abnormalities, a high-pitched voice. The surviving prognosis of Alagille syndrome patients depends on the severity of cardiovescular malformation in the early ages of infant. However, with the increasing years, it depends on the severity of the liver disease. Cholestasis causes congenital jaundice, malnutrition and growth retardation. Also, the increase of serum cholesterol level cause xanthoma and pruritus. Even though the severity of these problems are reduce with age, there is cases where there is no way but liver transplantation. For oral features of Alagille syndrome patients, green discoloration of entire dentition, induced by bilirubin infiltration into dentinal tubules, is especially. Also, xanthoma on gingiva and partial hypodontia have been reported. This report is on the oral features of an Alagille syndrome patient who visited to Kyung-Pook University Hospital.

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