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Small Bowel Obstruction Caused by an Aberrant Congenital Band in a Child
Min Ha Kwak,김애숙,Ji Hae Kang,최성민,김두권,이동석,하동엽,김성우 대한소아소화기영양학회 2012 Pediatric gastroenterology, hepatology & nutrition Vol.15 No.3
Small bowel obstruction due to congenital band is not only rare in children, but also difficult to diagnose, because common symptoms such as vomiting and abdominal pain are observed in patients. In order to prevent a fatal result, an anomalous congenital band should be considered in the discriminative diagnosis of intestinal obstruction in children who have no previous experience of operation or intraperitoneal inflammation. This report presents a 4-year-old boy who was admitted with abdominal pain and nonbilious vomiting for a day. The initial suspicion was for acute gastroenteritis. However, after further investigation and performance of surgery, the intestinal obstruction associated with a congenital band was confirmed. No recurrence was observed during the 8-month follow-up period. Thus early confirmation based on radiologic study is a crucial factor for the diagnosis of small bowel obstruction caused by a congenital band.
김선영 ( Seon Young Kim ),구회경 ( Hoe Kyoung Koo ),최용성 ( Yong Sung Choi ) 경희대학교 경희의료원 2022 慶熙醫學 Vol.37 No.1
Congenital constriction band syndrome is a rare disease, also called amniotic band syndrome. The etiology of this disease is not well known and there are no definitive diagnostic criteria yet. It causes anomalies that involve in various organs, however hand deformities are the most common. We present a case of congenital constriction band syndrome with multiple anomaly types in hand and foot such as amputation of fingers and toes, syndactyly and constriction ring. The syndactyly was found on the right hand from the first finger to the fifth finger. The patient was admitted to the neonatal intensive care unit, and the evaluation of neural defects and craniofacial anomalies was normal. Rehabilitation treatment was performed on the right hand through a consultation. Z-plasty surgery was performed at 2 and 6 months of age.
선천성 수부 윤상 수축대 증후군에 대한 미세현미경을 이용한 수술적 교정
황호,김용규,황종익,Hwang, Ho,Kim, Yong-Kyu,Whang, Jong-Ick 대한미세수술학회 2005 Archives of reconstructive microsurgery Vol.14 No.1
The Z-plasty has been known as a most common surgical treatment of congenital circumferential constriction band syndrome of the hand. There were thirteen patients of congenital circumferential constriction band syndrome of the hand. All patients underwent Z-plasty under microscope. we did microsurgical dissection to minimize vascular, neural and lymphatic injury, and then considering secondary correction and scar contracture, tried to preserve as much subcutaneous fat and skin flap as possible without any excision. There were less skin necrosis and lymphedema as a result of vascular compromise. Using microscope offers several advantages. First, preventing vascular, neural and lymphatic injury. Second, getting an accurate suture approximation. finally, preserving as much subcutaneous fat and skin flap as possible without any excision. The outcome of digit growth and contour can be excellent than we expected. As a results, we believe that correction of congenital circumferential constriction band syndrome of the hand under the microscope have better results.
한영미,이나래,배미혜,박경희,신진홍,김대성,변신연 대한신생아학회 2016 Neonatal medicine Vol.23 No.3
This paper reports the brain magnetic resonance imaging (MRI) findings of a case of merosin-deficient congenital muscular dystrophy (MDCMD) in a neonate and dis- cusses the spectrum of brain involvement in MDCMD. A neonate presented hypo- tonia, increased serum creatine kinase levels, and polymicrogyria and subcortical heterotopia on brain MRI involving both posterior temporal and occipital lobes. Al- though these findings suggested Fukuyama muscular dystrophy, muscle biopsy show- ed dystrophic changes and an absence of merosin staining. We found that compound heterozygous mutation for c.2049_2050delAG (p.R683fs) and c.5866-2A>G in the LAMA2 gene which encodes Laminin-α2. To our knowledge, this is the second Korean case of MDCMD with polymicrogyria and subcortical heterotopias. This case shows that a range of brain structural malformations can be found in children with MDCMD and that the classification of congenital muscular dystrophy (CMD) is not complete yet, as indicated previously in reports suggesting other unclassified forms of CMD.
고식적 Damus-Kaye-Stansel 술식의 임상적 적용 및 결과
임홍국,이창하,김수진,김웅한,이철,이길수,이재웅,신성호,황성욱 대한흉부외과학회 2008 Journal of Chest Surgery (J Chest Surg) Vol.41 No.1
Background: The Damus-Kaye-Stansel (DKS) procedure is a proximal MPA-ascending aorta anastomosis used to relieve systemic ventricular outflow tract obstructions (SVOTO) and pulmonary hypertension. The purpose of this study was to review the indications and outcomes of the DKS procedure, including the DKS pathway and semilunar valve function. Material and Method: A retrospective review of 28 patients who underwent a DKS procedure between May 1994 and April 2006 was performed. The median age at operation was 5.3 months (13 days∼38.1 months) and body weight was 5.0 kg (2.9∼13.5 kg). Preoperative pressure gradients were 25.3±15.7 mmHg (10∼60 mmHg). Eighteen patients underwent a preliminary pulmonary artery banding as an initial palliation. Preoperative main diagnoses were double outlet right ventricle in 9 patients, double inlet left ventricle with ventriculoarterial discordance in 6, another functional univentricular heart in 5, Criss-cross heart in 4, complete atrioventricular septal defect in 3, and hypoplastic left heart variant in 1. DKS techniques included end-to-side anastomosis with patch augmentation in 14 patients, classical end-to-side anastomosis in 6, Lamberti method (double-barrel) in 3, and others in 5. The bidirectional cavopulmonary shunt and Fontan procedure were concomitantly performed in 6 and 2 patients, respectively. Result: There were 4 hospital deaths (14.3%), and 3 late deaths (12.5%) with a follow-up duration of 62.7±38.9 months (3.3∼128.1 months). Kaplan-Meier estimated actuarial survival was 71.9%±9.3% at 10 years. Multivariate analysis showed right ventricle type single ventricle (hazard ratio=13.960, p=0.004) and the DKS procedure as initial operation (hazard ratio=6.767, p=0.042) as significant mortality risk factors. Four patients underwent staged biventricular repair and 13 received Fontan completion. No SVOTO was detected after the procedure by either cardiac catheterization or echocardiography except in one patient. There was no semiulnar valve regurgitation (>Gr II) or semilunar valve-related reoperation, but one patient (3.6%) who underwent classical end-to-side anastomosis needed reoperation for pulmonary artery stenosis caused by compression of the enlarged DKS pathway. The freedom from reoperation for the DKS pathway and semilunar valve was 87.5% at 10 years after operation. Conclusion: The DKS procedure can improve the management of SVOTO, and facilitate the selected patients who are high risk for biventricular repair just after birth to undergo successful staged biventricular repair. Preliminary pulmonary artery banding is a safe and effective procedure that improves the likelihood of successful DKS by decreasing pulmonary vascular resistance. The long-term outcome of the DKS procedure for semilunar valve function, DKS pathway, and relief of SVOTO is satisfactory.
양막대로 인한 환상협착대를 동반한 선천성 다발성 사지기형아 1례
오봉수(BS Oh),박형배(HB Park),오보훈(BH Oh),주갑순(KS Ju) 대한산부인과학회 1982 Obstetrics & Gynecology Science Vol.25 No.2
최근 저자들은 제태 기간 44주로, 자연 정상분만한 남아에서 양막대로 인한 기형으로 추정되는 다발성 사지 기형아를 경험하였기에 약간의 문헌적 고찰과 더불어 보고하는 바이다. A case of congenital multiple limbs anomalies with ring constrictions due to amnionic band is presented here in with a brief review of the literatures.
Small bowel obstruction caused byan anomalous congenital band in an infant
성태정,조지웅 대한소아청소년과학회 2008 Clinical and Experimental Pediatrics (CEP) Vol.51 No.2
저자들은 발열과 식욕저하를 주소로 패혈증 의증으로 전원 된 27일된 신생아에서 구토와 혈변 등은 없었으나, 입원 당시 복부팽만과 단순복부촬영에서 보인 소장 확장소견으로 개복술을 실시한 결과 선천성 밴드에 의해 내탈장된 소장이 압박되어 유발된 장폐색증 1예를 경험하였기에 문헌고찰과 함께 보고하는 바이다.