• Clinical and Experimental Pediatrics
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• 제55권9호
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• pp.350-353
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• 2012

An 18-year-old boy was admitted with chest discomfort, nausea, and dyspnea at rest. At the age of 3 years, he underwent muscle biopsy and dystrophin gene analysis owing to an enlarged calf muscle and elevated serum kinase level (6,378 U/L) without overt weakness; based on the results, Becker muscular dystrophy (BMD) was diagnosed. The dystrophin gene showed deletion of exons 45 to 49. He remained ambulant and could step upstairs without significant difficulties. A chest roentgenogram showed cardiomegaly (cardiothoracic ratio, 54%), and his electrocardiogram (ECG) showed abnormal ST-T wave, biatrial enlargement, and left ventricular hypertrophy. The 2-dimensional and M-mode ECGs showed a severely dilated left ventricular cavity with diffuse hypokinesis. The systolic indices were reduced, including fractional shortening (9%) and ejection fraction (19%). Despite receiving intensive medical treatment, he died from congestive heart failure 5 months after the initial cardiac symptoms. We report a case of BMD with early-onset dilated cardiomyopathy associated with deletion of exons 45 to 49. Early cardiomyopathy can occur in BMD patients with certain genotypes; therefore, careful follow-up is required even in patients with mild phenotypes of BMD.

• 한국동물위생학회지
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• 제40권1호
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• pp.67-70
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• 2017

A 4 months old, intact male Shih-tzu was referred to the Veterinary Medical Teaching Hospital of Chungnam National University for evaluation of the cause of cardiac murmur and syncope. Thoracic radiography revealed right-sided cardiomegaly. Echocardiography showed marked hypertrophy of right ventricular free wall and obstruction of right ventricular outflow tract, indicating double chambered right ventricle (DCRV). The dog was medicated with atenolol and sildenafil for DCRV, however, clinical signs did not control by medication. Balloon dilation for DCRV was performed to ameliorate patient's clinical signs. The peak systolic pressure gradient across the obstruction region was decreased and clinical signs was improved by balloon dilation procedure. This is the first case report of balloon dilation for the treatment of canine DCRV in South Korea.

• Journal of Chest Surgery
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• 제10권1호
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• pp.148-155
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• 1977

The clinical findings with cardioangiography and successful surgical treatment of a 10 year old girl with double-outlet right ventricle is reported at The Dept. of Thoracic and Cardiovascular Surgery, Korea University Hospital, College of Medicine. The patient has been suffered from intermittent cyanosis, palpitation, and exertional dyspnea since 1 year after NFSD, and a holosystolic ejection murmur of grade 4 at the left 3rd intercostal space with mild cyanosis of the lips was the only physical findings at the time of this admission. Cardiac catheterization revealed ventricular septal defect with left to right shunt of 43% and right to left shunt of 10.2%. On cardioangiography from the left ventricle revealed all of the left ventricular outflow shunted into the right ventricle through the large ventricular septal defect, and the aorta originated from the infundibular chamber of the right ventricle with left, anterior sided pulmonary artery. The atria, viscera, and ventricles were normally located, and right ventricular out-flow was narrowed with infundibular hypertrophy and pulmonary valvular stenosis. Surgical correction was accomplished by closure of the ventricular septal defect in such a way that left ventricular outflow was routed via a Teflon felt prosthetic tunnel to the aorta, and pulmonary valvulotomy with infundibulectomy Was done to pass Hegar`s dilator No. 15 for reconstruction of the right ventricular outflow tract. The patient tolerated complete repair and has continued to improve over a period of three months after operation with normal school life. Details of the disease and method of repair are presented with related references.

• Journal of Chest Surgery
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• 제23권5호
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• pp.962-967
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• 1990

The anomalous pulmonary venous return of the entire left lung was an extremely rare congenital anomaly. The reported surgical experience with correction of this disorder was limited. The 3-year-old female patient underwent an operation upon the unilateral total anomalous pulmonary venous return from the left lung, in which the left superior pulmonary vein drained into innominate vein and the left inferior pulmonary vein into the coronary sinus, in Yeungnam University Hospital. The symptoms were nonspecific except frequent upper respiratory infection. Cyanosis was not seen. On auscultatory findings, a grade 2/6 systolic ejection murmur was audible over left second intercostal space of left sternal border and second heart sound had an increased pulmonary component which was widely splitted. The electrocardiogram demonstrated a right ventricular hypertrophy and right axis deviation and chest X-ray showed slightly increased pulmonary vascularity and bulged pulmonary conus. The echocardiogram demonstrated increased right atrial, ventricular, and pulmonary arterial dimension, and also secundum atrial septal defect and enlarged coronary sinus. The cardiac catheterization confirmed the left-to-right with a Qp/Qs of 2.0: 1 and oxygen step-up was seen in pulmonary artery, right ventricle, right atrium, and left innominate vein, and the catheter was not been introduced into the left pulmonary vein. A median sternotomy incision was done. Left superior pulmonary vein was drained to the innominate vein through anomalous vertical vein and the left inferior pulmonary vein drained to right atrium through the coronary sinus. The diversion of the left inferior pulmonary vein to posterior wall of left atrium was done after division in the proximity of coronary sinus. The anomalous vertical vein was diverted to base of left atrial auricle and then a atrial septal defect was sutured directly. The postoperative course was uneventful and she was discharged on the eleventh postoperative day. In the postoperative follow-up-2 months, she has been well without specific problems.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제7권1호
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• pp.87-91
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• 2004

저자들은 복통과 구토에 동반된 전신 부종을 주소로 내원하여 저알부민혈증과 말초혈액 호산구증가증의 임상 소견으로 단백 소실성 위장증을 동반한 호산구성 위장관염으로 의심되었으나, 내시경검사 소견, 조직 소견, 방사선 소견과 저절로 좋아지는 일과성 임상 경과 등을 종합하여 말초혈액의 호산구 증가증과 조직 내에서 호산구의 심한 침윤을 보인 CMV 감염 소아 Menetrier병 1례를 보고하고자 한다. CMV 감염과 관련된 소아 Menetrier병의 특징적인 임상 증상과 소견을 염두에 두어야 하며, 향후 CMV 감염과 말초 혈액 호산구증가증과 조직내 호산구 침윤의 관련성에 대하여 임상적 해석이 필요하리라 판단된다.

• 한국임상수의학회지
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• 제32권1호
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• pp.101-104
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• 2015

4년령의 수컷 골든 리트리버 견이 복부 팽만 및 호흡곤란을 주증상으로 내원하였으며, 신체 검사 결과 부정맥과 심잡음이 청진되었다. 흉복부 방사선 검사 결과 전반적인 심비대, 흉수 및 복수가 관찰되었다. 심초음파 검사에서 비정상적인 승모판 및 삼첨판의 움직임과 역류가 관찰되었으며, 좌심실의 편심성 비대와 좌심방 비대가 보였다. 컬러 도플러 영상에서 확장기에 승모판 입구로부터 좌심실로 유입되는 와류가 관찰되었으며, 수축기에는 좌심방 및 우심방으로의 와류가 보였다. 도플러 검사로 확장기 승모판 유입 속도가 매우 증가하였으며 pressure half time이 지연되었다는 것을 알 수 있었다. 심초음파 검사 결과에 기초하여 승모판 협착증과 병발된 승모판 이형성증과 삼첨판 이형성증을 진단하였다.

• Journal of Chest Surgery
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• 제28권2호
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• pp.136-142
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• 1995

A clinical study was performed on 69 cases of isolated PDA surgically treated at the Department of Thoracic and Cardiovascular surgery of Kyung-Hee University Hospital from Mar. 1986 to Feb. 1994. Retrospective clinical analysis of these patients were as follows: 1.23 males and 46 females ranged in age from 16 days to 49 years. [mean 8.69yrs.,sex ratio M:F=1:2 2. Chief complaints were frequent URI in 44%, dyspnea on exertion in 16%,palpitation in 8%, easy fatigability in 6%, and no subjective symptoms in 26%. 3. On auscultation, typical continuous machinery murmur heard in 84%, and systolic murmur in 16% on Lt 2nd or 3rd intercostal space. 4. Simple chest x- ray showed increased pulmonary vascularity in 67%, cardiomegaly in 61%,and within normal limit in 16%. 5. EKG findings were LVH in 42%, biventricular hypertrophy in 17%, RVH in 3%, and within normal limit in 38%. 6. Echocardiogram was performed from all patient, and direct visualization of ductus in 93% 7. Cardiac catheterization was performed in 39 patients. The mean value of the results were;Differance SaO2[MPA-RV =11.03$\pm$ 5.26%,Qp/Qs=2.44$\pm$1.35,systolic pulmonary arterial pressure=40.69 $\pm$ 17.69mmHg. 8. 66 patients were operated through the left posterolateral thoracoctomy ; closure of ductus by double ligation in 43 cases, triple ligation in 23 cases.3 patients were operated by simple closure under cardiopulmonary bypass. 9. There was no death associated with the operation. The operative complications were atelectasis in 8 cases, pneumonia in 4 cases recannalization in 2 cases, and hoarseness in one case. 10. Systemic diastolic pressure was increased 8.12$\pm$ 0.13mmHg, and pulse pressure was decreased about 9.52 $\pm$ 1.87mmHg.

• Asian-Australasian Journal of Animal Sciences
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• 제10권5호
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• pp.528-533
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• 1997

Twenty four female Sprague-Dawley rats weighing about 190 g were used to examine changes in muscle cAMP concentrations and steady-state levels of skeletal muscle ${\alpha}$-actin mRNA during chronic administration of cimaterol, a ${\beta}$-adrenergic agonist. Cimaterol was mixed in a powdered rat diet at 10 mg/kg diet. At 3 and 21 days after the start of treatment, skeletal muscle and heart samples were collected for the measurement of cAMP concentrations and skeletal muscle ${\alpha}$-actin mRNA levels. Cimaterol increased (p < 0.01) body weight gain gradually during the first seven days of the trial period, but not thereafter. Most skeletal muscle weights and the ratio of muscle weight to body weight were increased (p < 0.05) by cimaterol treatment both at 3 and 21 days. Heart weight was also increased (p < 0.05) by cimaterol treatment at 3 and 21 days, but the ratio of heart weight to body weight was increased (p < 0.05) only at 3 day. Cimaterol decreased (p < 0.05) cAMP concentration of gastrocnemius muscle at both 3 and 21 days after treatment. However, cimaterol tended (p = 0.07) to increase cAMP concentration at 3 days in the heart. Cimaterol tended (p = 0.08) to increase the steady-state level of ${\alpha}$-actin mRNA by 60% in gastrocnemius muscle at 3 days but had no effect at 21 days. The results indicate that the pattern of hypertrophic response to chronic dietary administration of cimaterol is different between cardiac and skeletal muscle. In skeletal muscles it appears that the hypertrophy induced by cimaterol is partly due to stimulated myofibrillar protein synthesis at a pre-translational level.

• 대한한의학방제학회지
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• 제28권3호
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• pp.271-280
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• 2020

Hypertension has been approved to cause disharmony between the heart and kidney such as cardiac hypertrophy and kidney dysfunction. In traditional oriental medicine Paeo-tang (PET) has been shown to have effects on blood circulation improvement. However, the beneficial effect of PET on hypertension remains unknown. In this study, we investigated that PET attenuates blood pressure and improves cardiovascular and renal function in NG-nitro-L-arginine methylester (L-NAME) rat model. Hypertensive rat models were induced by the administration of L-NAME (40 mg/kg/day) and then PET (50 or 100 mg/kg/day) or Olmetec was treated for 2 weeks. PET treatment significantly suppressed the systolic blood pressure and decreased intima-media thickness in the thoracic aorta. PET ameliorated endothelium-dependent and independent vascular relaxation in the L-NAME-induced vascular dysfunction. PET ameliorated the functional decline in the kidney such as albumin and blood urea nitrogen in plasma. These results demonstrated that PET possesses protective effects against L-NAME-induced hypertension.

• The Korean Journal of Physiology and Pharmacology
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• 제22권4호
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• pp.447-456
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• 2018

Angiotensin-(1-9) [Ang-(1-9)], generated from Ang I by Ang II converting enzyme 2, has been reported to have protective effects on cardiac and vascular remodeling. However, there is no report about the effect of Ang-(1-9) on pulmonary hypertension. The aim of the present study is to investigate whether Ang-(1-9) improves pulmonary vascular remodeling in monocrotaline (MCT)-induced pulmonary hypertensive rats. Sprague-Dawley rats received Ang-(1-9) ($576{\mu}g/kg/day$) or saline via osmotic mini-pumps for 3 weeks. Three days after implantation of osmotic mini-pumps, 50 mg/kg MCT or vehicle were subcutaneously injected. MCT caused increases in right ventricular weight and systolic pressure, which were reduced by co-administration of Ang-(1-9). Ang-(1-9) also attenuated endothelial damage and medial hypertrophy of pulmonary arterioles as well as pulmonary fibrosis induced by MCT. The protective effects of Ang-(1-9) against pulmonary hypertension were inhibited by Ang type 2 receptor ($AT_2R$) blocker, but not by Mas receptor blocker. Additionally, the levels of LDH and inflammatory cytokines, such as $TNF-{\alpha}$, MCP-1, $IL-1{\beta}$, and IL-6, in plasma were lower in Ang-(1-9) co-treated MCT group than in vehicle-treated MCT group. Changes in expressions of apoptosis-related proteins such as Bax, Bcl2, Caspase-3 and -9 in the lung tissue of MCT rats were attenuated by the treatment with Ang-(1-9). These results indicate that Ang-(1-9) improves MCT-induced pulmonary hypertension by decreasing apoptosis and inflammatory reaction via $AT_2R$.