• Journal of Chest Surgery
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• 제5권1호
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• pp.13-18
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• 1972

In 1835,Schlesinger first described a case of subisthmlc lower thoracic aortic coarctation. Since Olim`s unsuccessful reconstructive surgery in 1949 and Beattie`s first successful resection with homograft replacement on such a lesion in 1951 were reported,about 20 cases of atypical aortic coarctation had been treated by definitive surgery until 1964. In Korea, only 2 cases of atypical aortic coarctation treated by bypass graft were reported until now. This is the third case-report treated by reconstructive surgery. The patient,11 year old girl who had 2 year history of headache, visual weakness, intermittent claudlcation, and general weakness, was first diagnosed of having the hypertension due to atypical coarctation by the findings of high blood pressure[170/110mmHg] at the upper extremity and weak pulsation on both femoral artery,murmur on the epigastrium, absence of aortic knob, and aorto graphy. Aortography demonstrated the isolated segmental narrowing[length 5cm, diameter 0.4cm] at the level of aortic hiatus 2cm above celiac arterial origin, the dilated right 9th, 10th, 11th intercostal arteries with multiple dimunitive collaterals and no associated abnormalities in the other arteries. Preoperatlve positive findings were strong positive mantoux test, high AST[720 units]. transient mild cardiomegaly with right lung infiltration on chest X-ray and suggestive left ventricular hypertrophy on ECG. On December 1970, through separate left thoracotomy and abdominal approach, bypass graft between descending thoracic aorta and abdominal aorta below renal artery was performed. The operation was first successful with satisfactory reduction of hypertension on the upper trunk[postoperatlve 130/80mmHg] and strong pulsation on the lower extremities[postop. O, postop. 140/100mmHg]. However,6 weeks after surgery, she expired of sudden hemoptysis and shock due to anastomotic leak within the thorax. Operative finding disclosed that the affected aorta was firm, with rich periaortic fibrosis and the outer diameter of stenotic site was not attenuated. Histopathology of the resected specimen was also compatible with primary arteritis.

• Tuberculosis and Respiratory Diseases
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• 제68권6호
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• pp.358-362
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• 2010

Intralobar pulmonary sequestration is a rare congenital lung anomaly. It is defined as a portion of nonfunctioning lung parenchyma that receives its blood supply from an anomalous systemic artery. Patients often present with chronic or recurrent pneumonia. A chest radiograph may show a cystic lesion with air-fluid levels in the lung base. A high index of suspicion is needed for a diagnosis. Surgical removal of a symptomatic intralobar pulmonary sequestration is generally the treatment of choice. Identifying the aberrant artery is a difficult problem when resecting a pulmonary sequestration. The thoracic and abdominal aortas are the most common origins for the abnormal blood supply. However, arterial supply from the celiac artery is quite rare. We present a case of intralobar pulmonary sequestration with the blood supply originating from the celiac artery.

• 디지털융복합연구
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• 제14권6호
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• pp.363-370
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• 2016

본 연구는 중년여성을 대상으로 12주간 순환근력운동이 신체구성성분과 기초체력에 어떠한 영향을 미치는지를 분석하기 위해 실시되었다. 최근 6개월 동안 규칙적으로 운동을 하지 않았으며, 질병 및 신체적 이상소견이 없는 중년여성 34명을 대상으로 12주간 순환근력운동을 실시하였으며, 신체구성성분검사와 기초체력검사항목으로 평가하였다. 결과에서 신체구성성분검사는 체중, 체지방률, 허리둘레 모든 항복에서 통계적으로 유의한 차이를 보였으며, 기초체력측정에서는 악력과 제자리멀리뛰기에서 어느 정도의 향상을 보이기는 했지만 통계적으로 유의한 차이가 나타나지 않았다. 그러나 상대악력, 앉아윗몸 앞으로 굽히기, 교차윗몸일으키기, 10m왕복달리기, 20m왕복오래달리기에서는 통계적으로 유의한 차이가 나타난 것으로 보았을 때, 12주간의 순환근력운동이 중년여성의 신체구성성분과 기초체력에서 효과를 보여 비만 개선과 체력증진을 위한 운동프로그램으로 권장될 수 있을 것으로 사료된다.

• Childhood Kidney Diseases
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• 제17권2호
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• pp.143-148
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• 2013

신경섬유종은 드문 전신 질환으로 여러 장기를 침범하며 특히 다양한 크기의 혈관을 침범하여 혈관병을 발생시켜 대동맥 협착, 모야모야병, 신동맥 협착 등을 일으킨다. 이로 인하여 약 0.4-6.4%의 환자에서 고혈압 증상이 있으며 이의 원인으로 신혈관 협착이 가장 흔하다. 특히 약물에도 조절되지 않는 고혈압은 혈관 협착 등이 원인이 될 수 있기 때문에 도플러 초음파 검사나 전산화 혈관 조영술 등의 정밀 검사가 필요하다. 이에 대한 치료는 약물 치료, 피부경유 혈관경유혈관성형술, 수술적 치료 등의 병합 요법으로 이루어지며 재협착 발생률이 높기 때문에 주의 깊은 추적 관찰이 필요하다. 본 저자들은 제 1형 신경섬유종증에 합병된 모야모야병에서 신장동맥 협착을 동반한 고혈압을 진단받고 치료한 1례를 경험하였기에 보고하는 바이다.

• Neonatal Medicine
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• 제28권3호
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• pp.99-107
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• 2021

Purpose: Studies have been conducted on the prevalence and infant mortality rate of congenital anomalies; however, studies on child mortality are rare. Therefore, we evaluated the characteristics of deaths associated with congenital anomalies among children born in Korea who died within 5 years of age. Methods: Birth-to-death cohort linked data of children under the age of 5 years from 2010 to 2013, and statistical data on the cause of death by age from 1999 to 2019, both provided by the Korea National Statistical Office's Microdata Integrated Service, were retrospectively investigated. We investigated the trends and characteristics of mortality associated with congenital anomalies. Results: Among 1,858,945 children, 6,510 children who died were under 5 years of age, and among them, 1,229 deaths were associated with congenital anomalies, while 5,281 deaths were due to other causes. Deaths associated with congenital anomalies accounted for 18.9% of all deaths. When comparing congenital anomalies by systems, anomalies of the cardiovascular system (52.6%) were the most common. The mortality rate associated with congenital anomalies and those of other causes showed similar declining trends in 21 years. Conclusion: The mortality rate of congenital anomalies during the first 5 years of life did not increase differently from the prevalence of congenital anomalies but rather decreased. Deaths associated with congenital anomalies accounted for 20.5% of all infant deaths and 12.1% of child deaths, since the major causes of death in infants and children are slightly different, continuous and careful monitoring is required.

• Clinical and Experimental Pediatrics
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• 제64권10호
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• pp.531-537
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• 2021

Background: Multisystem inflammatory syndrome in children (MIS-C) is a new hyperinflammatory variant that evolved during the coronavirus disease 2019 pandemic. Although the precise pathophysiology of MIS-C is uncertain, it is thought to be due to immune dysregulation occurring after recovery from acute infection. Purpose: Our study aimed to analyze the clinical spectrum, laboratory parameters, imaging characteristics, treatment strategies, and short-term outcomes of children with a diagnosis of MIS-C. Methods: This retrospective and prospective observational study included children less than 16 years of age who were admitted to the pediatric unit of a tertiary care teaching hospital in south India between August 2020 to January 2021 with a diagnosis of MIS-C according to World Health Organization criteria. Results: Twenty-one children were included in the analysis; all had fever with variable combinations of other symptoms. The mean age was 6.9 years; 71.4% were male. Gastrointestinal (80.9%) and cardiovascular (80.9%) systems were the most commonly affected. The majority of children had elevated inflammatory markers, and 16 (76.2%) had echocardiographic abnormalities mimicking Kawasaki disease. Eleven children (52.4%) required intensive care admission, 3 (14.3%) required supplemental oxygen, and 4 (19%) required inotropes. Nine (42.9%) were treated with intravenous immunoglobulin alone, 6 (28.6%) with steroids alone, and 3 (14.3%) with steroids and immunoglobulin. The median hospital stay was 6 days; there were no fatalities. Overweight/obesity, elevated ferritin, and mucocutaneous involvement were significantly associated with a prolonged hospital stay (≥7 days). Sixteen children (76.2%) were followed up till now and all of them had no clinical concerns. Conclusion: MIS-C is an emerging disease with variable presentation. A high index of suspicion is necessary for its early identification and appropriate management. Further research is essential for developing optimal treatment strategies.

• Journal of Genetic Medicine
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• 제18권2호
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• pp.142-146
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• 2021

Alagille syndrome (AGS) is a rare autosomal dominant inherited disorder, with major clinical manifestations of bile duct paucity, cholestasis, cardiovascular anomaly, ophthalmic abnormalities, butterfly vertebrae, and dysmorphic facial appearance. It is caused by heterozygous mutations in JAG1 or NOTCH of the Notch signaling pathway presenting with variable phenotypic penetrance and involving multiple organ systems. The following case report describes a unique case of a 16-year-old female with AGS who presented with the primary complaint of renovascular hypertension. She had a medical history of ventricular septal defect and polycystic ovary syndrome. The patient had a dysmorphic facial appearance including frontal bossing, bulbous tip of the nose, a pointed chin with prognathism, and deeply set eyes with mild hypertelorism. Stenoocclusive changes of both renal arteries, celiac artery, lower part of the abdominal aorta, and left intracranial artery, along with absence of the left internal carotid artery were found on examination. Whole exome sequencing was performed and revealed a pathologic mutation of JAG1, leading to the diagnosis of AGS. Reverse phenotyping detected butterfly vertebrae and normal structure and function of the liver and gallbladder. While the representative symptom of AGS in most scenarios is a hepatic problem, in this case, the presenting clinical features were the vascular anomalies. Clinical manifestations of AGS are diverse, and this case demonstrates that renovascular hypertension might be in some cases a presenting symptom of AGS.

• 대한통합의학회지
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• 제11권3호
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• pp.185-194
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• 2023

Purpose : This study aimed to investigate the relationship between the characteristics of hemodialysis patients and the occurrence of sarcopenia through a comprehensive literature review. Methods : A systematic literature search was conducted to identify eligible studies in the Cochrane library, PubMed and Embase. In this review, we included all papers published since the initiative's inception and summarized results as of december 2022. Studies that investigated association between sarcopenia diagnosis and hemodialysis patients (aged≥18 years) were included. Ultimately, 16 studies met our selection criteria. The risk of bias was assessed using the Newcastle-Ottawa scale. Results : Fourteen of the sixteen studies (88 %) reported that significant association between sarcopenia diagnosis and hemodialysis patients. However, two studies reported no association between sarcopenia diagnosis and hemodialysis patients. As a factor statistically related to sarcopenia in hemodialysis patients, Mortality (6 studies, 38 %), age (5 studies, 31 %), body composition (4 studies, 25 %), physical activity (2 studies, 13 %), diabetes (2 studies, 13 %), cardiovascular abnormalities (1 studies, 6 %), nutritional status (3 studies, 19 %), and gender (3 studies, 19 %). Conclusion : Our findings highlight the necessity of developing a physical therapy program that accurately reflects the health status of hemodialysis patients. To further investigate the association between the diagnosis of sarcopenia and hemodialysis patients, it is recommended to conduct large-scale longitudinal studies using standardized diagnostic criteria and evaluation methods, as well as analyze potential risk factors. Consequently, this study emphasizes the importance and potential of developing physical therapy programs that effectively address the health consequences associated with hemodialysis. The significance of this research lies in its ability to provide valuable insights and lay the foundation for future studies focused on developing preventive and therapeutic interventions targeting muscle wasting syndrome resulting from hemodialysis.

• 대한영상의학회지
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• 제83권2호
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• pp.372-377
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• 2022

일측성 폐정맥 폐쇄는 총폐정맥이 좌심방내로 연결되는 못하는 드문 심혈관계 기형이다. 일측성 폐정맥 폐쇄는 흔히 어린 시기에 진단이 되며, 성인이 된 이후에 진단이 되는 경우는 극히 드물다. 성인 환자에서 활동 시 호흡곤란과 객혈이 흔한 임상증상이다. 폐실질의 이상은 폐정맥 폐쇄의 간접적인 소견이며, 간질성 폐질환으로 나타날 수 있다. 우리는 62세 여자 환자에서 일측성 간질성 폐질환을 보이는 일측성 폐정맥 폐쇄의 증례와 12년간의 흉부 X선 사진 소견의 변화를 보고하고자 한다.

• 대한영상의학회지
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• 제83권4호
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• pp.846-860
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• 2022

지속적 좌측상대정맥은 드문 선천성 흉부 혈관 기형이다. 지속적 좌측상대정맥은 일반적으로 혈역학적 영향이 없지만 몇 가지 유형의 지속적 좌측상대정맥과 동반되는 심장 기형은 임상 증상을 유발할 수 있다. 지속적 좌측상대정맥의 존재는 심장 박동기 또는 중심 정맥 카테터를 배치할 때 왼쪽 쇄골 하 접근을 통한 심장으로의 카테터 삽입을 어렵게 만들 수 있다. 우연히 발견된 지속적 좌측상대정맥을 인식하는 것은 혈관 손상과 같은 합병증을 예방할 수 있다. 또한 지속적 좌측상대정맥과 유사한 위치에 있는 다양한 혈관들을 감별하는 것은 향후 흉부혈관시술을 위해서 필요하다. 이번 임상 화보는 지속적 좌측상대정맥의 다중 검출기 컴퓨터단층촬영 소견을 설명하고 감별이 필요한 다른 혈관들에 대해 요약해 보고자 한다.