• Journal of Chest Surgery
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• 제47권6호
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• pp.536-540
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• 2014

Due to its low prevalence and because there is lack of awareness about it, Loeys-Dietz syndrome is often misdiagnosed as Marfan syndrome, which has similar skeletal abnormalities and aortic pathology. However, the differential diagnosis between these two connective tissue diseases is critical because they correspond to different surgical indications and surgical decision-making. We report two cases of successful thoracoabdominal aortic replacement in patients with previously undiagnosed Loeys-Dietz syndrome.

• Biomolecules & Therapeutics
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• 제30권5호
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• pp.399-408
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• 2022

Diabetic cardiomyopathy (DCM) is described as abnormalities of myocardial structure and function in diabetic patients without other well-established cardiovascular factors. Although multiple pathological mechanisms involving in this unique myocardial disorder, mitochondrial dysfunction may play an important role in its development of DCM. Recently, considerable progresses have suggested that mitochondrial biogenesis is a tightly controlled process initiating mitochondrial generation and maintaining mitochondrial function, appears to be associated with DCM. Nonetheless, an outlook on the mechanisms and clinical relevance of dysfunction in mitochondrial biogenesis among patients with DCM is not completely understood. In this review, hence, we will summarize the role of mitochondrial biogenesis dysfunction in the development of DCM, especially the molecular underlying mechanism concerning the signaling pathways beyond the stimulation and inhibition of mitochondrial biogenesis. Additionally, the evaluations and potential therapeutic strategies regarding mitochondrial biogenesis dysfunction in DCM is also presented.

• Journal of Chest Surgery
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• 제46권3호
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• pp.237-239
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• 2013

An ectopic thyroid is caused by abnormalities in migration of the thyroid during development and rarely occurs in the thoracic cavity or the abdominal cavity. We report the case of a 64-year-old female who had abnormal findings from a thyroid hormone test during follow-up after thyroid cancer surgery. Based on the radioisotope diagnostic test, an ectopic thyroid inside the thoracic cavity was suspected. Through surgical treatment, the patient was diagnosed with ectopic intrapulmonary thyroid. Ectopic intrapulmonary thyroid is reported to be very rare and the case is described along with a literature review.

• Journal of Chest Surgery
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• 제55권1호
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• pp.77-80
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• 2022

Kartagener syndrome (KS) is a rare disease with an incidence of 1 in 20,000 to 30,000 births. There is no cure for KS, and conservative medical treatments are used to relieve symptoms and prevent disease progression. Lung transplantation (LT) is the only treatment option for end-stage KS. Since patients with KS have anatomical abnormalities such as situs inversus totalis, which often require surgery to correct, most reports are related to surgical techniques. Reports about morphological adaptations and changes in transplanted lung structure after LT in patients with KS are rare. We performed LT in a patient with KS and observed morphological adaptation of the lungs for 6 months on chest computed tomography using a quantitative evaluation tool (Chest Image Platform; Harvard University Disability Resources, Cambridge, MA, USA).

• Integrative Medicine Research
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• 제3권4호
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• pp.204-210
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• 2014

The aim of this review was to understand the effects of ${\beta}$-adrenergic stimulation on oxidative stress, structural remodeling, and functional alterations in the heart and cerebral artery. Diverse stimuli activate the sympathetic nervous system, leading to increased levels of catecholamines. Long-term overstimulation of the ${\beta}$-adrenergic receptor (${\beta}AR$) in response to catecholamines causes cardiovascular diseases, including cardiac hypertrophy, stroke, coronary artery disease, and heartfailure. Although catecholamines have identical sites of action in the heart and cerebral artery, the structural and functional modifications differentially activate intracellular signaling cascades. ${\beta}AR$-stimulation can increase oxidative stress in the heart and cerebral artery, but has also been shown to induce different cytoskeletal and functional modifications by modulating various components of the ${\beta}AR$ signal transduction pathways. Stimulation of ${\beta}AR$ leads to cardiac dysfunction due to an overload of intracellular $Ca^{2+}$ in cardiomyocytes. However, this stimulation induces vascular dysfunction through disruption of actin cytoskeleton in vascular smooth muscle cells. Many studies have shown that excessive concentrations of catecholamines during stressful conditions can produce coronary spasms or arrhythmias by inducing $Ca^{2+}$-handling abnormalities and impairing energy production in mitochondria, In this article, we highlight the different fates caused by excessive oxidative stress and disruptions in the cytoskeletal proteome network in the heart and the cerebral artery in responsed to prolonged ${\beta}AR$-stimulation.

• Journal of Korean Academy of Oral Health
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• 제42권4호
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• pp.238-241
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• 2018

Williams syndrome is a rare congenital disorder with various physical abnormalities and characterized by facial, oral, and dental features. Individuals with Williams syndrome typically have eating disorders in the early childhood, which lead to prolonged night feeding. Prolonged night feeding is a risk factor for rampant dental caries. Williams syndrome is caused by the microdeletion of chromosome 7, resulting in elastin deficiency. Elastin is integral to cardiovascular health. Many patients with Williams syndrome have complex cardiovascular abnormalities that must be considered a part of dental management. Complications related to cardiovascular diseases may induce adverse effects such as dangerously elevated blood pressure. This may occur in patients during stressful dental treatment. In addition, characteristics of auditory hyperalgesia and anxiety disorders among patients with William syndrome, complicate receiving routine dental management. Therefore, dental treatment under sedation or general anesthesia may be preferable for patients with Williams syndrome; in particular, patients who are very uncooperative and/or needs extensive dental treatment. A thorough evaluation of each patient's physical condition is required before making decisions regarding dental treatment. Careful monitoring and preparation for emergencies are very important during and shortly after dental treatment under general anesthesia or sedation. Monitoring is critical until vital signs have stabilized and return to normal. A 28-month-old man diagnosed as having Williams syndrome, visited the Dental Hospital of ${\bigcirc}{\bigcirc}$ University for the management of rampant dental caries. We reported on the management of this patient who had peripheral pulmonic stenosis, and received dental treatment under general anesthesia. We also reviewed the characteristics of Williams syndrome and discussed considerations for dental treatment under general anesthesia.

• Clinical and Experimental Reproductive Medicine
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• 제19권1호
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• pp.95-101
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• 1992

During the years 1983 to 1991, cytogenetic analysis was performed on 19 women with Turner syndrome in order to find out the incidence of symptoms and signs according to the classification of chromosome abnormalities. 1. All of them showed short stature and the mean height in 7 adults was $140.71{\pm}5.26cm$. 2. Among the 19 patients with Turner syndrome, 7 (36.8%) had 45, XO karyotype, 7 (36.8%) had 46, Xi (Xq), and remained 5 (26.3%) had mosaicism. 3. Five patients with mosaicism had 45, X/46, XX (2), 45, X/46, Xi (Xq) (2) and 45, X/47, XXX (1), respectively. 4. Patients with 45, XO and 46, Xi (Xq) had amenorrhea, whereas only 33% (1/3) of patients with mosaicism had amenorrhea. Total incidence of amenorrhea was 84.6% (11/13). 5. Abnormal external genitalia was detected in 63.6% of patients. The incidence of abnormality in patients with mosaicism was lower than that of other groups. 6. OMPC and deafness were detected in 3 of 19 patients. 7. Two cases of cardiovascular abnormalities were found in patients with 45, XO. This study suggests that gnenetic counselling according to the classification of chromosomal abnormalities could be needed in patients with Turner syndrome.

• Journal of Chest Surgery
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• 제21권4호
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• pp.711-715
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• 1988

Pulmonary arteriovenous fistula is a congenital vascular malformation originated from abnormalities of capillary development. Fistulas may develop only in the lung or may be associated with similar abnormalities of the skin, mucous membrane, and the other organs. It may occur with hereditary hemorrhagic telangietasia[Rendu-Osier-Weber syndrome]. Recently we have experienced a case of the pulmonary arteriovenous fistula associated with Schwannoma in 20 year old male man. Tennis ball sized bright reddish mass which was composed of variable sized vascular channels filled with blood clots was located in the just subpleural portion of left lower lobe. Left lower lobectomy was done. Microscopic findings showed variable sized numerous blood vessels embedding in the parenchyma. There was a thumb-tip sized brown nodular mass which was turned out to be Schwannoma at the left 7th thoracic paravertebral area.

• Journal of Chest Surgery
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• 제35권11호
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• pp.835-838
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• 2002

편평상피 세포암으로 우측 폐 상엽 절제술을 시행한 68세 남자 환자에서 나타난 기엥바레 증후군에 대하여 보고하고자 한다. 환자는 수술 후 6일 째 갑작스럽고 양측성의 하지의 근력약화 및 호흡부전과 감각실조를 호소했다. 응급으로 기관내 삽관 후 인공환기기를 거치했다. 근력 약화 후 2일, 20일, 40일째 전기진단적 검사를 시행하였다. 운동신경전도장애가 현저하게 나타났다. 지속적인 운동말단신경잠시, 전도 시간의 분산, 부분적인 운동신경전도차단이 나타났으며 이와 같은 것들은 기엥바레 증후군의 진단적 특징이다. 보조적인 치료와 함께 정주적 면역 글로불린의 부가적인 사용을 시행하였으며 병세는 근력 약화 후 6주만에 회복되었다.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제25권5호
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• pp.432-440
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• 2022

Purpose: Eating disorders often result in somatic complications, including cardiac abnormalities. Cardiac abnormalities may involve any part of the heart, including the cardiac conduction system, and can lead to sudden cardiac death. The current study aimed to evaluate the incidence of cardiac complications in pediatric patients with eating disorders and their associated factors. Methods: We retrospectively analyzed patients aged 10-18 years who were diagnosed with DSM-V (Diagnostic and Statistical Manual of Mental Disorder-V) eating disorders and underwent electrocardiography (ECG) and/or echocardiography between January 2015 and May 2020. Results: In total, 127 patients were included, of whom 113 (89.0%) were female. The median body mass index (BMI) was 15.05±3.69 kg/m². Overall, 74 patients (58.3%) had ECG abnormalities, with sinus bradycardia being the most common abnormality (91.9%). Patients with ECG abnormalities had significantly lower BMI (14.35±2.78 kg/m² vs. 16.06± 4.55 kg/m², p<0.001) than patients without ECG abnormalities, as well as lower phosphorus and higher cholesterol levels. Among the 46 patients who underwent echocardiographic evaluation, 23 (50.0%) had echocardiographic abnormalities, with pericardial effusion being the most common (60.9%). The median left ventricular mass (LVM) and ejection fraction were 67.97±21.25 g and 66.91±28.76%, respectively. LVM and BMI showed a positive correlation (r=0.604, p<0.001). After weight gain, the amount of pericardial effusion was reduced in 3 patients, and 30 patients presented with normal ECG findings. Conclusion: Cardiac abnormalities are relatively frequent in patients with eating disorders. Physicians should focus on this somatic complication and careful monitoring is required.