• Neonatal Medicine
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• v.28 no.3
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• pp.99-107
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• 2021

Purpose: Studies have been conducted on the prevalence and infant mortality rate of congenital anomalies; however, studies on child mortality are rare. Therefore, we evaluated the characteristics of deaths associated with congenital anomalies among children born in Korea who died within 5 years of age. Methods: Birth-to-death cohort linked data of children under the age of 5 years from 2010 to 2013, and statistical data on the cause of death by age from 1999 to 2019, both provided by the Korea National Statistical Office's Microdata Integrated Service, were retrospectively investigated. We investigated the trends and characteristics of mortality associated with congenital anomalies. Results: Among 1,858,945 children, 6,510 children who died were under 5 years of age, and among them, 1,229 deaths were associated with congenital anomalies, while 5,281 deaths were due to other causes. Deaths associated with congenital anomalies accounted for 18.9% of all deaths. When comparing congenital anomalies by systems, anomalies of the cardiovascular system (52.6%) were the most common. The mortality rate associated with congenital anomalies and those of other causes showed similar declining trends in 21 years. Conclusion: The mortality rate of congenital anomalies during the first 5 years of life did not increase differently from the prevalence of congenital anomalies but rather decreased. Deaths associated with congenital anomalies accounted for 20.5% of all infant deaths and 12.1% of child deaths, since the major causes of death in infants and children are slightly different, continuous and careful monitoring is required.

• Journal of Chest Surgery
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• v.20 no.4
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• pp.659-672
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• 1987

Nitrofen [2,4-dichlorophenyl-P-nitrophenyl ether] is a diphenyl ether herbicide used for pre and post-emergent control of broad leafed weeds. This chemical was known to induce a variety of congenital cardiovascular anomalies with diaphragmatic hernia and hydronephrosis in the rate fetuses. The present study was conducted to produce congenital cardiovascular anomalies in the rat fetuses by oral nitrofen administration at the indicated doses and days of gestation, and to find the characteristics of nitrofen-induced cardiovascular anomalies. All the observed fetuses were removed from the pregnant Sprague-Dawley rats sacrificed on the twenty-first day of gestation. They were preserved in 10 per cent formalin and dissection for examination were carried out under a dissecting microscope using forceps and scissors. Following results and conclusion were based on dissecting microscopic findings on 482 offsprings. 1. The eleventh day of gestation was the most sensitive day for nitrofen induction of congenital cardiovascular anomalies in the rat. This incidence was dose-related in rats exposed on the eleventh day of gestation. 2. Ventricular septal defect was the most common single anomaly that represented more than half of the total cardiovascular anomalies, followed by aortic arch anomalies and tetralogy of Fallot. 3. Cardiac anomalies derived from infundibular maldevelopment such as tetralogy of Fallot and pulmonary atresia with ventricular septal defect were only observed in the eleventh gestation day treated group. 4. Aortic arch anomalies were found in high frequency and the great majority were characteristically anomalous right subclavian artery with left aortic arch. Key words; nitrofen, congenital cardiovascular anomalies.

• Journal of Korean Foot and Ankle Society
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• v.16 no.3
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• pp.148-155
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• 2012

There are many different type of congenital toe anomalies such as syndactyly, polydactyly which are more common, and less frequently macrodactyly and curly toe. Congenital anomaly of hand can decrease the hand function and easy to be visualized, so the early treatment of anomaly is natural and recommended. On the other hand, Congenital anomaly of foot rarely decrease the foot function and was hidden in the shoe, so treatment of anomaly was delayed frequently. However, the surgery can be needed, as the foot getting grown-up, discomfort of shoe fitting or intractable plantar keratosis due to secondary deformation of foot can occur. A distinct feature and surgical consideration was compared with congenital anomaly of hand and it should be taken into account in the treatment of adult toe anomalies.

• Journal of Chest Surgery
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• v.24 no.8
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• pp.741-750
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• 1991

Between April, 1986 and July, 1991, 477 patients underwent open heart surgery with hypothermic cardiopulmonary bypass. There were 242 patients [50.7%] of acyanotic congenital anomalies, 34 patients [7.1%] of cyanotic congenital anomalies, and 187 patients [39.2%] of acquired heart diseases, 8 patients [1.7%] of coronary artery diseases, and 6 patients [1.3%] of mixed anomalies. Among the 276 congenital cardiac anomalies, 147 patients [53.3%] were male and 129 patients [46.7%] were female, ranged in age from 2 years to 58 years. Among the 187 acquired heart diseases, 72 patients [38.5%] were male and 115 patients [61.5%] were female, ranged in age from 10 years to 68 years. The common congenital defect were VSD and ASD in acyanotic cardiac patients, and TOF in cyanotic cardiac patients. Among the 187 acquired heart diseases, 180 patients underwent operation for cardiac valvular diseases, 4 patients were resected left atrial myxoma, and 3 patients underwent operation for aortic regurgitation with ascending aortic aneurysm. The operative mortality rate was 1.2% in acyanotic cardiac patients, 11.8% in cyanotic cardiac patients, and 6.9% in acquired cardiac patients, with overall mortality rate 4.2%.

• Journal of Chest Surgery
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• v.13 no.4
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• pp.414-417
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• 1980

During the period from 1958 to July 31, 1980, there were 1764 Cardiovascular Surgical cases consisting of 1166 congenital and 598 acquired lesions in the Department of Cardiothoracic Surgery, Seoul National University Hospital. For 1764 cases 1162 open heart surgery and 602 conventional operations were performed. Among 1166, congenital lesions 266 adult cases were found. Adult cases occupy 15.1% of the total cases consisting of 24.5% of acyanotic and 19.9% of cyanotic group. Over all operative mortality of adult cases was 6.8% compared with 12.4% of total cases. Left over congenital cardiovascular anomalies in Korea can be safely operated at the present time. The oldest operated patient among adult congenital cardiovascular anomalies was 57 years old female atrial septal defect case. For 1162 cases open heart surgery bubble oxygenators were utilized except 2 membrane oxygenators. Various kinds of bubble oxygenators have been used but recently Shiley**** oxygenators are being used exclusively with satisfactory results.

• Journal of Chest Surgery
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• v.19 no.4
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• pp.663-671
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• 1986

100 cases of open heart surgery were done in the Dept. of Thoracic and Cardiovascular Surgery, Won Kwang University Hospital from July, 1984 to October, 1986. l. Among the 100 cases, there were 51 cases [51%] of acyanotic congenital heart anomalies, 10 cases [10%] of cyanotic congenital heart anomalies and 39 cases [39%] of acquired heart disease. 2. The age distribution of 100 cases was 18 months to 56 years old and mean age was 10.8 years old in congenital heart anomalies and 34.7 years old of acquired heart disease. 3. The overall mortality was 8%. and the mortality in each entity is 5.9% in congenital acyanotic cases, 10% in congenital cyanotic cases and 10.3% in acquired valvular heart disease. 4. For myocardial protection, high concentration potassium of cold blood cardioplegic solution [30mEq/L] had been used, associated with topical cooling of ice-slush.

• Neonatal Medicine
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• v.16 no.1
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• pp.89-93
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• 2009

Maternal diabetes is known to have teratogenic effects which increase the risk for congenital anomalies, such as caudal dysplasia, cardiac defects, hydronephrosis, and small left colon syndrome. Infants of diabetic mothers have a 10-fold higher frequency of anomalies in the central nervous system and a 5-fold higher frequency of congenital heart defects. However, jejunal atresia combined with multiple anomalies of the face, ears, and hands has rarely been reported. Herein we report a neonate born to a diabetic mother, who had hemifacial microsomia, displacement of the lacrimal ducts, polydactyly of the right hand, microtia of the right ear and proximal jejunal atresia presenting as bile regurgitation on the 1st day of life.

• Advances in pediatric surgery
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• v.7 no.1
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• pp.7-14
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• 2001

Congenital anomalies of the head and neck region such as preauricular sinus and skin tag, thyroglossal duct cyst, branchial anomaly, cystic hygroma and dermoid cyst are common in pediatric population. It is important for pediatricians and pediatric surgeons to be familiar with the embryology and the anatomical characteristic of these lesions in order to diagnose and treat them properly. Three hundred and nineteen patients with congenital head and neck anomalies treated at Hanyang University Hospital between 1980 and 1999 were reviewed to determine the relative frequency of the anomalies and to analyze the method of management. Eight-four (25.1 %) of 335 lesions were preauricular sinus and skin tag, 81 (24.2 %) were thyroglossal duct cyst, 81 (24.2 %) branchial anomaly, 58 (17.3 %) cystic hygroma and 31 were (9.2 %) dermoid cyst. The male-to-female ratio was 1.4:1. Thyroglossal duct cyst most commonly present at 3-5years, however branchial anomalies commonly are diagnosed in children younger than 1 year. Preauricular sinus showed familial tendency in three patients and was bilateral is 33.8 %. Most head and neck anomalies in children have specific clinical and anatomical characterics. A careful history and physical examination is very useful for diagnosis and proper management. Experienced pediatric surgeons should do the initial surgery since the recurrence rate after incomplete surgical excision can be high.

• Journal of Chest Surgery
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• v.17 no.4
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• pp.804-810
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• 1984

Two hundred twenty one cases of open heart surgery were done in the Department of Thoracic and Cardiovascular Surgery, Pusan National University Hospital from July, 1981 to October, 1984. 1.There were 154 cases [73%] of congenital anomalies and 57 cases [27%] of acquired valvular heart diseases. Among the congenital cases, 128 cases were acyanotic and 26 cases were cyanotic. Among the 57 cases of acquired valvular replacement surgery, 3 cases had open heart commissurotomy, one had Kay annuloplasty. 2.The age distribution of the congenital acyanotic anomalies ranged from 5 to 32 years with mean age of 12.8 years, the congenital cyanotic anomalies from 3 to 29 years with mean age of 14.2 years and the acquired valvular diseases from 9 to 51 years with mean age of 30 years. The difference of sex distribution was no significance. 3.Three methods for debubbling process were used in our institute, in 133 cases, the vent was inserted into the left ventricular apex, in 61 cases inserted into the left atrium through right superior pulmonary vein and in 17 cases used needle aspiration only. 4.For cardioplegia, the GIK solution was infused repeatedly from 30 to 40 minutes interval and brought excellent results for myocardial protection during open heart surgery. 5.Overall mortality was 7.6%. The mortality along with each disease is 1.56% in congenital acyanotic cases, 26.9% in congenital cyanotic cases and 12.3% in acquired valvular disease.

• Clinical and Experimental Pediatrics
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• v.62 no.2
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• pp.68-74
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• 2019

Purpose: To investigate the prevalence of occult spinal dysraphism (OSD) and subsequent neurosurgery in pediatric patients with isolated or combined dorsal midline cutaneous stigmata with or without other congenital malformations. Methods: We carried out a retrospective review of patients who underwent sonography or magnetic resonance imaging (MRI) for OSD because of suspicion of dorsal midline cutaneous stigmata (presumed to be a marker for OSD) between January 2012 and June 2017. Information about patient characteristics, physical examination findings, spinal ultrasound and MRI results, neurosurgical notes, and accompanying congenital anomalies was collected. Results: Totally 250 patients (249 ultrasound and one MRI screening) were enrolled for analysis. Eleven patients underwent secondary MRI examinations. The prevalence of OSD confirmed by an MRI was 2.4% (6 patients including one MRI screening). Five patients (2%) had tethered cord and underwent prophylactic neurosurgery, 3 of whom had a sacrococcygeal dimple and a fibrofatty mass. Prevalence of tethered cord increased as markers associated with a sacrococcygeal dimple increased (0.5% of the isolated marker group, 8.1% of the 2-marker group, and 50% of the 3-marker group). Incidence of OSD with surgical detethering in 17 other congenital anomaly patients was 11.8%, which was higher than the 1.3% in 233 patients without other congenital anomalies. Conclusion: Our results suggest that the presence of dorsal midline cutaneous stigmata, particularly fibrofatty masses, along with a sacrococcygeal dimple is associated with OSD or cord tethering requiring surgery. OSD should be suspected in patients with concurrent occurrence of other congenital anomalies.