• Neonatal Medicine
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• v.18 no.2
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• pp.404-408
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• 2011

Scimitar syndrome (SS) is a rare congenital anomaly characterized by hypoplasia of the right lung and partially anomalous pulmonary venous drainage to the inferior vena cava. The term scimitar derives from the shadow created by the anomalous pulmonary vein on chest X-ray that closely resembles that of a curved Turkish sword. It rarely presents as an isolated abnormality. Various cardiac and non-cardiac anomalies have been association with SS, such as right lung hypoplasia, dextroposition of the heart, hypoplasia of the right pulmonary artery, systemic arterial blood supply to the right lower lung from the infra-diaphragmatic aorta, and a secundum type of atrial septal defect. However, an imperforate anus has not been reported previously in association with SS. We describe the first case of infantile scimitar syndrome accompanied with an imperforate anus in a newborn who presented with tachypnea and right pulmonary atelectasis.

• Neonatal Medicine
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• v.25 no.1
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• pp.49-52
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• 2018

Arterial tortuosity syndrome (ATS) is a very rare autosomal recessive connective tissue disease characterized by generalized elongation and tortuosity of the medium- to large-sized arteries, and other systemic connective tissue manifestations. To date, this disease entity has not been reported in Korea. We report a case of ATS diagnosed in a neonate who presented with severe elongation and tortuosity of the aorta and its major branches, as well as the intracranial arteries. Additionally, the patient presented with a tortuous dilatation of the inferior vena cava, an aneurysmal dilatation of the extra-hepatic bile ducts, and an inguinal and sliding hiatal hernia. The diagnosis was confirmed using DNA sequencing analysis, and the patient demonstrated a compound heterozygosity for two novel mutations (c.738delG [p.Gln247Serfs*33] and c.362T>C [p.Ile121Thr]) in exon 2 of the SLC2A10 gene. Genetic analysis also confirmed that both parents were heterozygous carriers of the responsible mutations. Owing to such clinical manifestations, ATS is often misdiagnosed as other connective tissue diseases including Loeys-Dietz syndrome, Marfan syndrome, and Ehlers-Danlos syndrome. In patients presenting with a high index of suspicion, thorough clinical evaluation and screening for ATS including computed tomography or magnetic resonance angiography and target gene analysis are necessary for early diagnosis and management.

• Journal of Veterinary Clinics
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• v.9 no.1
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• pp.333-366
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• 1992

Safety of recombinant porcine somatotropin administration on pig was studied using 32 Landrace x Yorkshire crossbred pigs. The starting body weight ranged from 55.5kg to 65.3kg. Eight pigs were allotted to each low dose group of sustained releasing rPST(SL), high dose group of sustained releasing rPST(SH), daily injection group of rPST(DI), and control group(C). Pigs in SL group and SH group were injected subcutaneously twice in 3 week-interval with 1000$\mu\textrm{g}$ and 2000$\mu\textrm{g}$ of sustained releasing rPST per kg body weight, respectively. Pigs in DI group were injected intramuscularly with 100$\mu\textrm{g}$ of rPST everyday for 6 weeks. Blood was collected from anterior vena cava just before the first treatment, and at four weeks and six weeks of experiment. Hematological parameters and blood chemical parameters indicating liver function, kidney function, electrolyte metabolism, mineral metabolism and lipid metabolism were determined. Necropsy and urinalysis were performed after final blood collection. The results were summarized as follows, and it is concluded that rPST administration does not affect pig health negatively. 1. rPST administration did not affect kidney function as manisfested by BUN, creatinine and urinalysis. 2. rPST administration did not affect liver function as manisfested by total protein, albumin, serum AST activity serum ALT activity serum ALP activity, serum LDH activity, serum GGT activity and serum SDH activity. 3. rPST administration did not affect skeletal muscle, cardiac muscle and brain as manifasted by serum AST activity and serum LDH activity. 4. rPST administration increased blood glucose level within normal range. 5. rPST administration did not affect lipid metabolism as manisfested by triglyceride, cholesterol, and phospholipid concentrati on. 6. rPst administration dia not affect mineral metabolism as manisfested by calcium, phosphorus, magnesium and iron concentration. 7. rPST administration did not affect electrolyte metabolism as manisfested by Na, K, chloride concentration. 8. rPST administration did not affect erythrocyte count, leukocyte count, thrombocyte count, and plasma fibrinogen level.

• Journal of Veterinary Clinics
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• v.30 no.1
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• pp.36-40
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• 2013

Caval syndrome is a life threatening condition caused by heavy worm burden in right cardiac chambers including right atrium, right ventricle, and vena cava and results in mechanical interference in tricuspid valvular excursion and blood flow to lower pulmonary arteries. A mechanical worm removal is the choice of option for treating this condition in dogs. In this study, we developed a new mechanical worm removal method using modified extraction brushes for treating dogs with caval syndrome. With this newly developed method, we successfully removed heartworms in two dogs with caval syndrome. Although the number of cases applied was small, authors found this method can be a good alternative retrieval method for mechanical worm removal in dogs.

• Journal of Chest Surgery
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• v.31 no.6
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• pp.619-623
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• 1998

A 51-year-old male with chronic renal failure had marked swelling and tenderness of the right arm. Venography revealed central vein occlusion involving stenosis of right proximal subclavian vein, right internal jugular vein, and left distal innominate vein, and obstruction of right brachiocephalic vein. Multiple obstruction of these veins was thought to have resulted from repeated subclavian catheterization. Right subclavian-superior vena cava was bypassed with 10 mm Gore-tex vascular graft and then left subclavian vein with 8 mm Gore-tex vascular graft was bypassed to the 10 mm Gore-tex vascular graft. The results were excellent.

• Journal of Chest Surgery
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• v.12 no.1
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• pp.33-42
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• 1979

The present study reports 41 cases of congenital and acquired heart diseases, who received open heart surgery under extracorporeal circulation [ECC] by Sarns Heart-Lung-Machine [HLM] at the Department of Thoracic and Cardiovascular Surgery, Hanyang University Hospital during the` period between July 1975 and February 1979. The priming of pump oxygenator was carried out by the hemodilution method using Hartman`s solution, whole blood, and fresh human plasma. The rate of hemodilution was in the average of 50.8 ml/kg. ECC was performed at the average perfusion flow rate of 85.0 ml/kg/min [2.43 L./ kg/2] and at moderate hypothermia. In the total cardiopulmonary bypass, arterial pressure ranged between 55 mmHg and 90 mmHg, but generally maintaining over 70 mmHg. Patient age ranged between 2 and 54 year old, in congenital heart diseases, between 2 and 28, in acquired heart diseases, between 17 and 54 Sex ratio of male to female was 20:21. The cases include a case of pulmonary valvular stenosis, 4 cases of atrial septal defect, 9 cases of ventricular septal defect, 9 cases of tetralogy of Fallot, 5 cases of pentalogy of Fallot, 3 cases of atypical multiple anomalies 7 cases of mitral stenosis or insufficiency, a case of myxoma in left atrium, and a case of ruptured aneurysm of Valsalva`s sinus. The surgical managements were 16 valvulotomy for pulmonary valvular stenosis, 2 Teflon patch graft closure and 5 simple suture closure of atrial septal defect, 16 Teflon patch graft closure and 5 simple suture closure of ventricular septal defect, 12 pericardial patch graft for infundibular stenosis of right ventricle, one anastomosis between left superior vena cava and right atrium, 2 open mitral commissurotomy, 5 mitral valve replacement using Starr-Edward`s ball valve, porcine xenograft by Hancock, by Carpentier-Edward, or Angell-Shiley, one removal of left atrial myxoma, and a repair of ruptured aneurysm of Valsalva`s sinus. Four [9.7%] out 41 cases expired postoperatively and the rest of 37 cases survived with satisfactory results. The causes of death were one coronary embolism in tetralogy of Fallot, 2 postoperative lower cardiac output in atypical multiple anomalies, and one right heart failure in large: ventricular septal defect with pulmonary hypertension.

• Journal of Chest Surgery
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• v.38 no.10 s.255
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• pp.729-732
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• 2005

A 15-year-old male was admitted with right-sided chest pain and cough for one month. On chest computed tomographic scan, a $10\times15\times16$ em-sized huge mediastinal mass was occupied in the right hemithorax. Radiologically, it seemed that the tumor was severely adhesive on the heart and the superior vena cava. Therefore we decided on chemotherapy and radiotherapy first instead of surgery. The tumor marker was nearly normalized afterwards, but the tumor size was seemed to be bigger on chest tomographic scan. This suggests the growing teratoma syndrome. After the successful resection, he showed symptomatic improvement and is being followed up without any symptoms in an out patient department up to now.

• Neonatal Medicine
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• v.16 no.1
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• pp.71-75
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• 2009

There are few cases of partial trisomy of 9q, known as partial 9q trisomy syndrome with low birth weight, microcephaly, hypotelorism, beaked nose, small lip, long finger, hypertrophic pyloric stenosis, ventricular septal defect, and mental retardation. We report partial trisomy of 9q derived from a paternal chromosome, which has different features of other syndromes, including prematurity, atrial and ventricular septal defect, patent ductus arteriosus, persistent left superior vena cava, congenital hydronephrosis, and scrotal hernia.

• Journal of Chest Surgery
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• v.35 no.10
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• pp.740-744
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• 2002

Retrograde cerebral perfusion under hypothermic circulatory arrest is a simple and useful adjunct to avoid cerebral ischemic injury in the treatment of aortic arch pathology. In the surgery of distal aortic arch and proximal descending aortic lesions through the left thoracotomy incision, right atrium-retrograde cerebral perfusion (RA-RCP) through a venous cannula positioned into the right atrium is simpler than retrograde cerebral perfusion through superior vena cava. The time limits for RA-RCP during aortic arch reconstruction have yet to be clarified. We, herein, present a case with uneventful recovery after RA-RCP of 94 minutes during reconstruction of aortic arch and descending aorta. These data suggest that RA-RCP, as an adjunct to hypothermic circulatory arrest, may prolong the circulatory arrest time and thus prevent ischemic injury of the brain, even when RA-RCP exceeds 90 minutes.

• Journal of Chest Surgery
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• v.46 no.1
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• pp.41-48
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• 2013

Background: We present our 12-year experience of pulmonary thromboendarterectomy in patients with chronic thromboembolic pulmonary hypertension. Materials and Methods: Between January 1999 and March 2011, 16 patients underwent pulmonary thromboendarterectomy. Eleven patients (69%) were classified as functional class III or IV based on the New York Heart Association (NYHA) classification. Seven patients had a history of inferior vena cava filter insertion, and 5 patients showed coagulation disorders. Pulmonary thromboendarterectomy was performed during total circulatory arrest with deep hypothermia in 14 patients. Results: In-hospital mortality and late death occurred in 2 patients (12.5%) and 1 patient (6.3%), respectively. Extracorporeal membrane oxygenation support was required in 4 patients who developed severe hypoxemia after surgery. Thirteen of the 14 survivors have been followed up for 54 months (range, 2 to 141 months). The pulmonary arterial systolic pressure and cardiothoracic ratio on chest radiography was significantly decreased after surgery ($76{\pm}26$ mmHg vs. $41{\pm}17$ mmHg, p=0.001; $55%{\pm}8%$ vs. $48%{\pm}3%$, p=0.003). Tricuspid regurgitation was reduced from $2.1{\pm}1.1$ to $0.7{\pm}0.6$ (p=0.007), and the NYHA functional class was also improved to I or II in 13 patients (81%). These symptomatic and hemodynamic improvements maintained during the late follow-up period. Conclusion: Pulmonary thromboendarterectomy for chronic thromboembolic pulmonary hypertension shows good clinical outcomes with acceptable early and long term mortality.