• Title/Summary/Keyword: 선천적

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Primary Pulmonary Hypertension in a Maltese Dog (말티즈 견에서 발생한 원발성 폐동맥 고혈압증)

  • Moon, Hyeong-Sun;Lee, Seung-Gon;Choi, Ran;Park, In-Chul;Hyun, Chang-Baig
    • Journal of Veterinary Clinics
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    • v.24 no.4
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    • pp.613-617
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    • 2007
  • A 1-year-old castrated male Maltese dog(weighing 2.4 kg) was presented with primary complaints of occasional coughing, dyspnea and exercise intolerance. Based on diagnostic findings including paradoxical split S2, diastolic regurgitant murmur, marked dilation of pulmonary artery, right ventricular eccentric hypertrophy with thickening of ventricular septum, severe tricuspid and pulmonic regurgitation(5.4 m/sec and 3.4 m/sec, respectively) and the absence of any congenital intracardiac shunting, obstructive pulmonary diseases and systemic diseases associated with right ventricular pressure overload or pulmonary hyperperfusion, the case was tentatively diagnosed as primary pulmonary hypertension. The dog was treated with furosemide, aspirin and oxygen supplementation. This case report described a rare case of primary pulmonary hypertension in a Maltese dog.

A Case of Unilateral Absence of Left Pulmonary Artery Presenting Hemoptysis (객혈이 선행된 좌폐동맥 형성부전증 1예)

  • Yoon, Hye-Jin;No, Tae-Mook;Kim, Seon-Woong;Park, Dong-Hi;Kim, In-Seek;Kim, Yeon-Jae;Lee, Byung-Ki;Park, Ji-Young;Chung, Jin-Hong
    • Journal of Yeungnam Medical Science
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    • v.20 no.1
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    • pp.71-78
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    • 2003
  • Unilateral absence of a pulmonary artery (UAPA) is a rare congenital anomaly that is frequently associated with other cardiovascular anomalies first reported by Fraentzel in 1968. Most patients who have no associated cardiac anomalies have only minor or absent symptoms. We experienced a case of isolated UAPA in a young female presenting hemoptysis. The chest radiograph showed a small left lung volume and high resolutional CT of chest showed multiple subpleural nodules and centrilobular nodules in parenchyma. The video-assisted thoracoscopic biopsy revealed diffuse dilated vessels in visceral pleura. The pulmonary angiogram confirmed the absence of the left main pulmonary artery.

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Use of the Autogenous Calvarial Bone in Craniofacial Bone Graft (두안면부의 골이식시 자가 두개골의 이용)

  • Woo, Sang-Hyun;Jeong, Jae-Ho;Lee, Dae-Hoon;Choi, See-Ho;Seul, Jung-Hyun
    • Journal of Yeungnam Medical Science
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    • v.4 no.2
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    • pp.75-81
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    • 1987
  • Bone grafts are an integral and important aspect of craniofacial reconstruction. Ribs, tibia, and iliac bone have traditionally used as donor site but each of these has various problems, however using to the calvarial bone as a donor site has several advantage. These are as follows ; there are abundance of material, easy to reach the donor site through coronal incision, minimal pain of donor area, less functional inability, shorter hospitalization, no need of immobilization, hidden scar at donor site, no secondary deformity and appropriate curvature obtained properly selected. From march to December 1987, we experienced three cases of autogenous calvarial bone graft such as congenital saddle nose deformity, fibrous dysplasia on the right side frontal bone, and deviated nose. The results were very excellent without any significant complication. The detail technique of autogenous calvarial bone graft and its advantages compared with the traditional methods of bone grafts are discussed.

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A Case of Pulmonary Alveolar Proteinosis with Spontaneous Resolution (자연 소실된 폐포단백증 1예)

  • Nam, Seung Bum;Park, Kwang Young;Lee, Ho Jin;Jung, Jae Wook;Choi, Yoon Hee;Kim, Hyo Seok;Kim, Cheol Hyeon;Lee, Jae Cheol
    • Tuberculosis and Respiratory Diseases
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    • v.63 no.3
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    • pp.294-298
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    • 2007
  • Pulmonary alveolar proteinosis is a rare disorder that is characterized by the abundant accumulation of surfactant-derived material within the alveolar spaces and distal bronchioles. Although the findings of homogenous ground-glass opacities overlaid by thickened septa, which form a "crazy paving" pattern on high-resolution computed tomography, can assist in making a diagnosis, a lung biopsy is usually required. This disorder has a variable clinical course, from spontaneous resolution to respiratory failure and death. While a whole lung lavage has been the standard treatment since the early 1960s, GM-CSF therapy has been attempted based on the recently suggested pathogenetic mechanism. We report a case of pulmonary alveolar proteinosis that resolved spontaneously after an open lung biopsy.

Unbalanced Translocations of Chromosome 2 and Chromosome 20 in a Two-Generation Family (2대에 걸쳐 나타난 염색체 2번과 20번의 비균형적 전위 1례)

  • Min, Saeah;Lim, Seonwoong;Kim, Youngsook;Lee, Ohkyung
    • Clinical and Experimental Pediatrics
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    • v.45 no.7
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    • pp.917-922
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    • 2002
  • An unbalanced translocation is frequently the result of inheritance of an unbalanced haploid set from a parent with a balanced translocation. Families in which one parent is a balanced translocation carrier fall into the following classes : Those in which none of the possible abnormal offsprings is viable; Those in which one type of offspring, usually the one with the smaller deletion, is born alive; Those in which two types of abnormal offspring are viable. We report a neonate whose karyotype was 46,XX,der(2)t(2;7)(q21;p21.2),der(20)t(2;20)(q21;p13). She was small for her gestational age and had multiple anomalies such as exophthalmos, corneal opacity, short neck, tongue tie, clinodactyly, atrial septal defect, patent ductus arteriosus and ventriculomegaly. Moreover, her mother's karyotype was 46,XX,der(2)t(2;7)(q21;p21.2),del(16)(q22.1),der(20)t(2;20)(q21;p13) but her father had normal karyotype. The same derivative chrosomes were found between mother and her infant, except for del(16)(q22.1) in her mother and these same unbalanced translocations in a two-generation family are extremely rare.

Surgical Treatment of Congenital Brachymetatarsia According to the Number of Affected Rays (이환된 열 수에 따른 선천성 중족골 단축증의 수술적 치료)

  • Chung, Moon-Sang;Baek, Goo-Hyun;Gong, Hyun-Sik;Oh, Joo-Han;Lee, Young-Ho;Yoon, Pil-Whan;Kim, Ji-Hyeung
    • Journal of Korean Foot and Ankle Society
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    • v.10 no.1
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    • pp.24-30
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    • 2006
  • Purpose: To present our treatment protocol and surgical outcome for patients with congenital brachymetatarsia in which treatment was decided according to the number of affected rays. Materials and Methods: Sixty-nine metatarsals in 44 patients with single or multiple congenital brachymetatarsia were included in the study. When a single ray was affected in a foot, we performed a one-stage lengthening using an intercalary autogenous iliac bone graft. We overcame excessively short rays by the double level lengthening at the metatarsal and proximal phalanx as one stage. When multiple rays were affected in one foot, we performed a one-stage combined shortening and lengthening procedure without an iliac bone graft. Results: All patients were satisfied with the cosmetic and functional results. The average length gain by one-stage lengthening in 56 metatarsals of 38 patients was 14 (6-21) mm. Six patients with a combined shortening and lengthening procedure regained a nearly normal parabola of the involved foot. Neurovascular complication was not identified. Conclusion: Satisfactory results were achieved for the treatment of patients with congenital brachymetatarsia, by individualizing the surgical options according to the number of affected rays and general foot appearance.

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A Therapeutic Approach Based on Motor Development in Congenital Muscular Torticollis: A Case Report (선천성 근육 사경 환아에 대한 운동 발달적 치료 접근: 증례보고)

  • Ryu, Je-Yong;Kim, Yu-Jin;Sung, Ju-Yeon;Shim, Jae-Hun;Lee, Gyu-Wan;Oh, Duck-Won
    • Physical Therapy Korea
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    • v.13 no.2
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    • pp.77-84
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    • 2006
  • Although conservative management of congenital muscular torticollis (CMT) has been well documented, relatively little is known about the response to the treatment. The purposes of this case report were to describe the use of a therapeutic approach based on motor development in physical therapy intervention for an infant with CMT and to report the result of the treatment. The patient was a 20-day-old baby boy with left CMT presenting muscular mass in the left sternocleidomastoid muscle. The angle of the lateral head tilt was 20 degrees. The size of muscular mass was 5.3 mm in ultrasonography. Intervention included ultrasonic therapy, soft tissue massage, passive and active range of motion exercises, motor developmental therapy, and parent instruction. The procedures of motor developmental therapy and changes in the amount of lateral head tilt were documented using photography. The size of the mass was decreased to .3 mm before the 5-month follow-up. The patient also maintained a midline head position in the supine position and a midline head alignment during all functional activities. A therapeutic approach based on motor development is a beneficial method for reducing an asymmetrical head and neck position, and facilitating normal development as a component of physical therapy intervention.

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Double Outlet Right Ventricle in a Maltese Dog (말티스견에서 발생한 양대혈관 우심실기시)

  • Lee, Jong-Sung;Choi, Ran;Han, Suk-Hee;Hyun, Chang-Baig
    • Journal of Veterinary Clinics
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    • v.27 no.6
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    • pp.735-739
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    • 2010
  • An 8 month old female Maltese (body weight 3.6 kg) was referred with primary complaints of dyspnea and exercise intolerance. Diagnostic imaging studies revealed marked cardiomegaly and prominent main pulmonary trunk dilation on thoracic radiography, abnormally arisen aortic roots (toward right ventricle) with left-to right shunted perimembraneous ventricular septal defect located underneath of aortic root, aortic root was located to predominantly to the right ventricle and pulmonary regurgitation (peak velocity 4.7 m/s, pressure gradient ~88 mmHg) from pulmonary over-circulation and hypertension on echocardiography, indicating double outlet right ventricle (DORV). The dog was treated with furosemide (1 mg/kg, BID) for reducing volume overload at right ventricle, spironolatcone (1 mg/kg) and enalapril (0.5 mg/kg) for minimizing deleterious cardiac remodeling, and sildenafil (1 mg/kg) for lessening pulmonary over-circulation and hypertension. The clinical condition of this dog was improved after 1 week of medical treatment. The dog is currently survived and regularly monitored.

Anomalous Origin of the Right Coronary Artery from the Pulmonary Artery (우관상동맥의 폐동맥 이상 기시증(ARCAPA))

  • Esther Choi;Jeong Jun Park;Tae Jin Yoon;Young Hwoe Kim;Jae Kon Ko;In Sook Park;Dong Man Seo
    • Journal of Chest Surgery
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    • v.35 no.12
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    • pp.894-897
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    • 2002
  • Anomalous origin of the right coronary artery from the pulmonary artery is a rare congenital anomaly that has generally been found incidentally during autopsy or surgery. Sudden death may occur without antecedent symptoms in apparently healthy, asymptomatic patients and hence operation is recommended when the lesion is recognized. As opposed to the more frequent anomalous origin of the left coronary artery from the pulmonary artery, only a few children with this anomaly have been reported to have undergone surgical treatment. This report describes a 2-year old patient whose diagnosis was made by echocardiography, confirmed by angiocardiography, and successfully corrected by reimplantation of the anomalous coronary artery into the aorta.

Total Repair through Arterial Switch Operation in a Patient with Taussig-Bing Anomaly Undergoing the Modified Damus-Kaye-Stansel Procedure -1 case- (Damus-Kaye-Stansel 술식을 받은 Taussig-Bing 기형의 환자에서 관상동맥 이식을 통한 완전 교정술 - 1예 보고-)

  • 황여주;한미영;전양빈;박철현;박국양;이창하
    • Journal of Chest Surgery
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    • v.37 no.9
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    • pp.796-799
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    • 2004
  • A 52 day-old male infant who had Taussig-Bing anomaly with coarctation of the aorta underwent initial palliative Damus-Kaye-Stansel (DKS) procedure including arch reconstruction because of suspected intramural coronary artery, size discrepancy of great arteries, potential subaortic stenosis, refractory pneumonia, and severe congestive heart failure. Total repair was done 44 months later, which was composed of VSD patch closure, DKS take-down, and arterial switch procedure, We report a successful case of DKS take-down and arterial switch operation for the reuse of native aortic and pulmonary valves rather than Rastelli-type procedure in a patient with Taussig-Bing anomaly having palliative DKS procedure.