• 제목/요약/키워드: Congenital defects

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화상에 의한 소구증 환자의 구각부 재건 - 증례보고 - (RECONSTRUCTION OF THE CORNERS OF THE MOUTH IN BURN-INDUCED MICROSTOMIA - A CASE REPORT -)

  • 최영달;변성수;정휘동;남웅;김형준
    • Maxillofacial Plastic and Reconstructive Surgery
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    • 제29권6호
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    • pp.543-547
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    • 2007
  • The lips and corners of the mouth are not only important for appearance but are also essential for facial expression, speech, and nutrition. Defects in these areas can be caused by congenital clefts of the lip and face, trauma, infection, cysts, and excision of benign or malignant tumors. Numerous techniques have been introduced for reconstruction of the lips and corners of the mouth, and in particular, techniques such as the Kazanjian Roopenian I and II, Converse method, Zisser method, Platz and Wepner method. Gillies and Millard method are commonly utilized for elongation and reconstruction of the mouth corner. Few reports exist in the oral and maxillofacial surgery literature regarding correction of microsomia and reconstruction of the corners of the mouth. As such, the authors report a case of the corners of the mouth elongation in a patient with burn-induced microstomia using the Converse flap which yielded a satisfactory outcome.

발살바동 동맥류 파열의 외과적 치료 - 10례 보고 - (Surgical Treatment of Ruptured Sinus of Valsalva Aneurysm)

  • 김우찬;윤정섭;김치경;조규도;왕영필;곽문섭
    • Journal of Chest Surgery
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    • 제29권11호
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    • pp.1207-1211
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    • 1996
  • 본 가톨릭대학교 의과대학 흉부외과학 교실에선 최근 10년간 10예의 발살바동 동맥류 파열환자를 경험하였다. 8명은 선천성이었으며 2명은 세균성 심내막염에 의한 후천성이었다. 동반된 심질환으로는 대동맥판막폐쇄부전증 4예, 심방중격결손증 2예, 심실중격결손증 1례, 승모판막폐쇄부전증 1례 그리고 삼첨판막폐쇄부전증 1례가 있었다. 전례에서 동맥류가 우관상동에서 발생하였으며, 8명의 환자에서 우심실으로 2명의 환자에서 우심방으로 파열하였다. 저자들은 대동맥절개와 동맥류가 파열된 심방 또는 심실절개를 통한 수술을 선호하였으며, 8명의 환자에서 Dacron 첩포를 사용하여 봉합하였고 2명의 환자에서는 단순봉합하였다. 전 환자에서 양호한 결과를 보였으며, 술후 사망예는 없었다.

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이강우심실의 수술요법 (Surgical Treatment of Double Chambered Right Ventricle)

  • 박종호;노준량
    • Journal of Chest Surgery
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    • 제27권5호
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    • pp.353-363
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    • 1994
  • From January 1978 to December 1992, 59 patients of double chambered right ventricle were repaired. Surgical correction consisted of closure of the ventricular septal defect and resection of anomalous muscle bundles through right ventriculotomy [Group I ; 34 patients] or right atriotomy [Group II ; 25 patients]. Between these two groups, there was no difference in the operation time and the postoperative results. All patients survived. In group I, hemodynamically significant residual ventricular septal defect was found in three and reoperations were necessary. In one patient, subacute bacterial endocarditis developed postoperatively. In group II, complete atrioventricular block developed in one and mediastinitis in two. Follow-up period was from 2 to 75 months [mean 17.1 months]. There was no late death. All patients have remained in sinus rhythm except one patient. Careful evaluation of echocardiographic and catheterization data preoperatively and careful examination of the anatomy intraoperatively are necessary so that double chambered right ventricle should not be overlooked, because most ventricular septal defects are now closed through the right atrium. Repair of double chambered right ventricle is also easily performed through the atrial approach. Transatrial repair should be considered as an alternative to the transventricular approach in patients with this congenital heart defect. Successful surgical correction of double chambered right ventricle is expected with excellent long term results.

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Echocardiographic Diagnosis of Subaortic Stenosis with Severe Deformation of Mitral Valve Apparatus in a Dog

  • Chung, Doo-ri;Yoon, Young-min;Hwang, Tae-sung;Choi, Moon-yeong;Jung, Dong-in;Yeon, Seong-chan;Lee, Hee-chun
    • 한국임상수의학회지
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    • 제34권1호
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    • pp.54-57
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    • 2017
  • A 1-year-old castrated male Schnauzer dog was presented with heart murmur. Auscultation revealed systolic murmur located at the left heart base (grade 5/6). There were no remarkable findings on thoracic radiographs. Two-dimensional echocardiography revealed subaortic tunnel-like obstruction at the entrance to the left ventricular outflow tract. Anterior mitral valve leaflet appeared to be tethered to septum with minimal motion. Chordae tendineae was abnormally thickened. Color Doppler analysis revealed turbulent flow starting below the aortic valve. Mitral regurgitation was presented during systole. Spectral Doppler recordings revealed high velocity flow through the aorta and mitral regurgitation. Based on echocardiographic examination, the dog was diagnosed with subaortic stenosis concurrent with mitral dysplasia. The patient was medicated with ${\beta}-blocker$ and diuretics. It has been doing well without apparent clinical signs at 2 year after the diagnosis.

Morphological classification of the moderator band and its relationship with the anterior papillary muscle

  • Lee, Ju-Young;Hur, Mi-Sun
    • Anatomy and Cell Biology
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    • 제52권1호
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    • pp.38-42
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    • 2019
  • This study investigated and classified the various types of moderator band (MB) in relation to the anterior papillary muscle, with the aim of providing anatomical reference information and fundamental knowledge for use when repairing the congenital defects and understanding the conduction system. The study investigated 38 formalin-fixed human hearts of both sexes obtained from donors aged 38-90 years. The MB was evident in 36 of the 38 specimens (94.7%). The morphology of the MB and its connection with the APM took various forms. The MBs that had a distinct shape were classified into three types according to their shape: cylindrical column, long and thin column, and wide and flat column. Types 2 and 3 were the most common, appearing in 15 (41.7%) and 14 (38.9%) of the 36 specimens, respectively, while type 1 was observed in seven specimens (19.4%). Type 3 was divided into subtypes based on their length. The MB usually originated from a single root (91.7%), with the remainder exhibiting double roots. The pairs of roots in the latter cases had different shapes. The originating point of the MB ranged from the supraventricular crest to the apex of the ventricle. The most-common originating point was in the middle (25 of 36 specimens, 69.4%), followed by the upper third (13.9%), the lower third (11.1%), and the top fifth (5.6%) of the interventricular septum. This study has produced fundamental anatomical and clinical information that will be useful when designing cardiac surgical procedures.

Concurrent SHORT syndrome and 3q duplication syndrome

  • Boaz, Alexander M.;Grasso, Salvatore A.;DeRogatis, Michael J.;Beesley, Ellis N.
    • Journal of Genetic Medicine
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    • 제16권1호
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    • pp.15-18
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    • 2019
  • SHORT syndrome is an extremely rare congenital condition due to a chromosomal mutation of the PIK3R1 gene found at 5q13.1. SHORT is a mnemonic representing six manifestations of the syndrome: (S) short stature, (H) hyperextensibility of joints and/or inguinal hernia, (O) ocular depression, (R) Rieger anomaly, and (T) teething delay. Other key aspects of this syndrome not found in the mnemonic include lipodystrophy, triangular face with dimpled chin (progeroid facies, commonly referred to as facial gestalt), hearing loss, vision loss, insulin resistance, and intrauterine growth restriction (IUGR). 3q duplication syndrome is rare syndrome that occurs due to a gain of function mutation found at 3q25.31-33 that presents with a wide array of manifestations including internal organ defects, genitourinary malformations, hand and foot deformities, and mental disability. We present a case of a 2 year and 3 month old male with SHORT syndrome and concurrent 3q duplication syndrome. The patient presented at birth with many of the common manifestations of SHORT syndrome such as bossing of frontal bone of skull, triangular shaped face, lipodystrophy, micrognathia, sunken eyes, and thin, wrinkled skin (progeroid appearance). Additionally, he presented with findings associated with 3q duplication syndrome such as cleft palate and cryptorchidism. Although there is no specific treatment for these conditions, pediatricians should focus on referring patients to various specialists in order to treat each individual manifestation.

Stroke Recurrence in a Patient Twelve Years after Repair of a Secundum Atrial Septal Defect

  • Ok, Taedong;La, Yun Kyung;Cha, Hyun Seo;Cheon, Kyeongyeol;Choi, Bo Kyu;Yi, Gi Jong;Lee, Kyung-Yul
    • 대한신경집중치료학회지
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    • 제11권2호
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    • pp.124-128
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    • 2018
  • Background: Secundum atrial septal defect (ASD) is a common congenital heart defect in adults. Patients with ASDs at high risk of cardiovascular complications undergo either surgical repair or percutaneous device closure. Case Report: We report the case of an 85-year-old male with unusual recurrent cerebral infarctions. The patient has undergone repair of secundum ASD 12 years ago. Evaluation by transesophageal echocardiography revealed a mobile mass at the patch repair site in the left atrium. The mass was surgically removed due to recurrent stroke during the anticoagulation. Conclusion: This case emphasizes the importance of regular cardiac checkup and the need to consider cardioembolic source as being part of the etiology of stroke recurrence, even if the event occurs many years after intracardiac shunt closures.

In Vivo Stem Cell Imaging Principles and Applications

  • Seongje Hong;Dong-Sung Lee;Geun-Woo Bae;Juhyeong Jeon;Hak Kyun Kim;Siyeon Rhee;Kyung Oh Jung
    • International Journal of Stem Cells
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    • 제16권4호
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    • pp.363-375
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    • 2023
  • Stem cells are the foundational cells for every organ and tissue in our body. Cell-based therapeutics using stem cells in regenerative medicine have received attracting attention as a possible treatment for various diseases caused by congenital defects. Stem cells such as induced pluripotent stem cells (iPSCs) as well as embryonic stem cells (ESCs), mesenchymal stem cells (MSCs), and neuroprogenitors stem cells (NSCs) have recently been studied in various ways as a cell-based therapeutic agent. When various stem cells are transplanted into a living body, they can differentiate and perform complex functions. For stem cell transplantation, it is essential to determine the suitability of the stem cell-based treatment by evaluating the origin of stem, the route of administration, in vivo bio-distribution, transplanted cell survival, function, and mobility. Currently, these various stem cells are being imaged in vivo through various molecular imaging methods. Various imaging modalities such as optical imaging, magnetic resonance imaging (MRI), ultrasound (US), positron emission tomography (PET), and single-photon emission computed tomography (SPECT) have been introduced for the application of various stem cell imaging. In this review, we discuss the principles and recent advances of in vivo molecular imaging for application of stem cell research.

분만성 상완 신경총 손상, 선천성근성사경 및 구개열의 병발 (Concurrence of Obstetric Brachial Plexus Injury, Congenital Muscular Torticollis and Cleft Palate)

  • 이한별;박명철;김치선;한재덕;이승재;김세온;임신영
    • Journal of Genetic Medicine
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    • 제8권1호
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    • pp.71-75
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    • 2011
  • 임신성 당뇨 및 조기양막파수를 보인 산모의, 다른 산전 병력 없이 과체중아로 태어난 생후 17일된 남아에서, 분만성 상완 신경총 손상, 선천성근성사경 및 구개열의 병발이 진단 되었다. 동시에 세 질환의 병발이 단순 병발일 수 있으나, 세 질환을 동시에 유발할 수 있는 병인에 의한 것일 수 있으므로, 증례의 분석을 통하여 어머니의 임신성 당뇨에 의하여 이 세 질환의 발생이 가능할 것으로 가정하였다. 모체의고혈당증은 주로 신경능선 조직에서 발생하는 태아구조에 영향을 주는 것으로 알려져 있고, 신경능선 조직에서 분화하는 조직들 중 하나인 구개골의 형성에 영향을 주어 구개열의 발생이 가능할 것으로 보이며, 임신성 당뇨에서 과체중아의 출생 빈도가 증가하므로, 이와 관련된 분만 손상 중 분만성 상완 신경총 손상 및 선천성근성사경의 발생이 가능할 것으로 판단된다. 따라서 임신성 당뇨가 있는 산모의 태아에서 구개열등의 선천성 기형의 위험성이 높으므로 산전 검사 시 유의해야하며, 과체중아에서 분만성 상완 신경총 손상과 선천성근성사경의 위험성이 높으므로 이에 대한 주의와 확인이 필요할 것으로 판단된다. 그러나, 분만성 상완 신경총 손상, 선천성근성사경 및 구개열의 병발이 산모의 임신성 당뇨와 연관된 합병증일 수 있다는 것을 증명하기 위하여 향후 더 많은 증례를 통한 심화된 연구가 필요하다.

심실중격 결손증의 해부학적 분류 및 임상적 고찰 (Anatomic Classification of Ventricular Septal Defects and Clinical Review of 99 Cases)

  • 이철주;이동협;정태은;강면식
    • Journal of Yeungnam Medical Science
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    • 제3권1호
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    • pp.221-227
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    • 1986
  • 영남대학교 의과대학 흉부외과학교실에서는 1984년부터 1986년까지 총 99명의 심실중격 결손증 환자들을 수술 치험하였던 바 다음과 같은 결론을 얻었다. 1. 남여비는 1.2 : 1로 남자가 약간 많았다. 2. 연령분포는 다양하였으며 전체의 $\frac{2}{3}$가 3세에서 12세 사이였다. 3. 체중분포는 10kg미만이 13예이고 21kg이상이 44 예였다. 4. 입원시 주소는 잦은 상기로 감염증이 78예로 제일 많은 빈도를 보였다. 5. 심도자 검사상 폐 체동맥압비가 0.75이상인 경우가 6명, 폐 체 혈관 저항비가 0.5 이상인 경우는 0명, 폐 체 혈류량비가 3이상인 경우가 13명으로 대부분이 중등도의 병변을 보였다. 6. 동반된 심기형은 개방성 난원공, 폐동맥 협착증, 개방성 동맥관 등의 순이었다. 7. 심실중격 결손의 부위는 막변연 유입부 결손이 45예로 제일 많았으며 근육형은 1예로 없었다. 8. 술후 합병증은 완전 혹은 불완전 우각 차단증이 52예로 가장 많았다. 9. 술후 사망은 2예였으며 2%의 발생율을 보였다.

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