• 제목/요약/키워드: Congenital defects

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식도주위 열공 탈장 -1례 보고- (Paraesophageal Hiatal Hernia)

  • 이원진
    • Journal of Chest Surgery
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    • 제28권11호
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    • pp.1067-1070
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    • 1995
  • We experienced a case of congenital paraesophageal hiatal hernia[Type;IV in seventeen day-old female and treated through the right thoracotomy, reduction of the herniated viscera ,stomach and some part of transverse colon and omentum by gentle finger push, and narrowing the esopahgeal hiatus. Paraesophageal hiatal hernia accounts for only 5% per cent of all diaphragmatic defects but is a potentially dangerous lesion due to compressed lung by the herniated viscera. Symptoms are related to this, including exertional dyspnea, vomiting, cough, Tachypnea but noncyanotic, etc. Barium study shows that the stomach has herniated into the right pleural cavity. The speckled appearance in the herniated stomach in the herniated stomach was due to food material. It strongly suggests paraesophageal hiatal hernia. The operation was done. We report the case with the brief review of literatures.

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Pulmonary Root Translocation with the Lecompte Maneuver: For Transposition of the Great Arteries with Ventricular Septal Defect and Pulmonary Stenosis

  • Yoon, Dong Woog;Kim, Tae Ho;Shim, Man-shik;Jun, Tae-Gook;Jang, Jae Seok
    • Journal of Chest Surgery
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    • 제48권5호
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    • pp.351-354
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    • 2015
  • A five-month-old boy who had undergone previously transcatheter balloon atrioseptostomy at 3 days of age for complete transposition of the great arteries with ventricular septal defect and pulmonary stenosis underwent pulmonary root translocation with the Lecompte maneuver. This operation has the advantages of maintaining pulmonary valve function, preserving the capacity for growth, and avoiding problems inherent to the right ventricular to pulmonary artery conduit. This patient progressed well for 9 months postoperatively and we report this case of pulmonary root translocation with the Lecompte maneuver.

Surgical Extraction of an Embolized Atrial Septal Defect Occluder Device into Pulmonary Artery after Percutaneous Closure

  • Yolcu, Mustafa;Kaygin, Mehmet Ali;Ipek, Emrah;Ulusoy, Fatih Rifat;Erkut, Bilgehan
    • Journal of Chest Surgery
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    • 제46권2호
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    • pp.135-137
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    • 2013
  • An atrial septal defect is the most common type of congenital heart disease among adults. Surgical repair or percutaneous closure of the defect is the treatment options. Even though percutaneous closure seems to be less risky than surgical repair, it may result in fatal complications like device embolism, cardiac perforation and tamponade. Herein we report a case of the embolism of a device into the pulmonary artery after one hour of percutaneous closure in which the embolized device was surgically removed and the defect was closed with a pericardial patch.

Fontan Revision with Y-Graft in a Patient with Unilateral Pulmonary Arteriovenous Malformation

  • Lee, Jeong-woo;Park, Jeong-Jun;Goo, Hyun Woo;Ko, Jae Kon
    • Journal of Chest Surgery
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    • 제50권3호
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    • pp.207-210
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    • 2017
  • The extracardiac conduit Fontan procedure is the last surgical step in the treatment of patients with a functional single ventricle. An acquired pulmonary arteriovenous malformation may appear perioperatively or postoperatively due to an uneven hepatic flow distribution. Here we report a case of a bifurcated Y-graft Fontan operation in a 15-year-old male patient with a unilateral pulmonary arteriovenous malformation after an extracardiac conduit Fontan operation.

Early onset of colorectal cancer in a 13-year-old girl with Lynch syndrome

  • Ahn, Do Hee;Rho, Jung Hee;Tchah, Hann;Jeon, In-Sang
    • Clinical and Experimental Pediatrics
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    • 제59권1호
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    • pp.40-42
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    • 2016
  • Lynch syndrome is the most common inherited colon cancer syndrome. Patients with Lynch syndrome develop a range of cancers including colorectal cancer (CRC) and carry a mutation on one of the mismatched repair (MMR) genes. Although CRC usually occurs after the fourth decade in patients with Lynch syndrome harboring a heterozygous MMR gene mutation, it can occur in children with Lynch syndrome who have a compound heterozygous or homozygous MMR gene mutation. We report a case of CRC in a 13-year-old patient with Lynch syndrome and congenital heart disease. This patient had a heterozygous mutation in MLH1 (an MMR gene), but no compound MMR gene defects, and a K-RAS somatic mutation in the cancer cells.

Airway Improvement with Medium-Term Compression Duration after Right Pulmonary Artery Anterior Translocation

  • Jang, Woo Sung;Kim, Jae Bum;Kim, Jae Hyun;Choi, Hee Jeong
    • Journal of Chest Surgery
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    • 제51권1호
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    • pp.57-60
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    • 2018
  • Left main bronchus compression occasionally occurs in patients with cardiac disease. A 19-month-old female patient weighing 6.7 kg was admitted for recurrent pneumonia and desaturation. S he had an atrial septal defect (AS D) with a right aortic arch. Her left main bronchus had been compressed between the enlarged right pulmonary artery (RPA) and the descending thoracic aorta for 14 months. We conducted ASD closure and RPA anterior translocation via sternotomy. The left main bronchus compression was relieved despite the medium-term duration of compression.

Townes-Brocks Syndrome Associated with Hypothyroidism in a Korean Newborn : A Case Report

  • Park, Shin-Young;Lee, Woo-Ryoung
    • Journal of Genetic Medicine
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    • 제5권2호
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    • pp.136-138
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    • 2008
  • Townes-Brocks 증후군은 이형성 귀, 청력 상실, preaxial polydactyly, triphalangeal thumbs, 쇄항, 신기형, 선천성 심질환, 정신 지체 등을 동반하는 다기형의 상염색체 우성 유전질환이다. 그러나 갑상선 기능저하증은 Townes-Brocks 증후군의 흔한 특징은 아니다. 현재까지 갑상선 기능저하증과 동반된 Townes-Brocks 증후군은 3례가 보고된 바 있다. 저자들은 한국에서 최초로 갑상선 기능저하증과 동반된 Townes-Brocks 증후군 1례를 보고한다.

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A case of partial trisomy 3p syndrome with rare clinical manifestations

  • Han, Dong-Hoon;Chang, Ji-Young;Lee, Woo-In;Bae, Chong-Woo
    • Clinical and Experimental Pediatrics
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    • 제55권3호
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    • pp.107-110
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    • 2012
  • Partial trisomy 3p results from either unbalanced translocation or $de$ $novo$ duplication. Common clinical features consist of dysmorphic facial features, congenital heart defects, psychomotor and mental retardation, abnormal muscle tone, and hypoplastic genitalia. In this paper, we report a case of partial trisomy 3p with rare clinical manifestations. A full-term, female newborn was transferred to our clinic. She had cleft lip-plate, dysgenesis of the corpus callosum, patent ductus arteriosus, pulmonary hypertension, and severe right-sided hydronephrosis, associated with ureteropelvic junction obstruction. Cytogenetic investigation revealed partial trisomy 3p; 46,XX,der(4)t(3;4)(p21.1;p16). The karyotype of her father showed a balanced translocation, t(3;4)(p21.1;p16). Therefore, the size of duplication can be an important factor.

성인에서 발견된 좌측 폐동맥 형성부전증 1례 (A Case of Left Pulmonary Artery Hypoplasia in Adult)

  • 이승현;최광호;이흥범;이용철;이양근
    • Tuberculosis and Respiratory Diseases
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    • 제46권1호
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    • pp.116-121
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    • 1999
  • Unilateral hypoplasia of the pulmonary artery is an uncommon anomaly, which commonly develops in combination with congenital cardiovascular defects such as tetralogy of Fallot, patent ductus arteriosus and septal defect of atrium or ventricle, but may also present as an isolated lesion. We have recently experienced a case of the left pulmonary artery hypoplasia in adult by chance of during the general health screen, which diagnosed by chest X-ray, chest spiral CT, lung perfusion and ventilation scan, digital substraction angiogram and bronchoscopy, then presented hereby with the review of relevant literature.

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Cardiac biomarkers for early detection of heart diseases in small animals

  • Hyun, Chang-Baig
    • 한국임상수의학회:학술대회논문집
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    • 한국임상수의학회 2009년도 춘계학술대회
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    • pp.137-150
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    • 2009
  • Background: Heart failure can develop secondary to various heart diseases (e.g. mitral valvular insufficiency, congenital heart defects, heart worm infection). The clinical signs of heart failure can be confused with those of other conditions, such as respiratory disease. Therefore, specific, sensitive, rapid and inexpensive blood tests for heart failure are desirable. Cardiac troponins, natriuretic peptides and cytokines have been more recently used as indicators of heart disease in humans and animals. These peptides are sensitive to changes in vasoconstriction and dilation within the heart and are used for the diagnosis and prognosis of heart failure. Methods: Previously developed and newly developed cardiac biomarkers will be discussed for understating clinical implications and diagnostic values in heart diseases in small animals

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