• Journal of Chest Surgery
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• 제32권6호
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• pp.510-517
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• 1999

배경: 신생아나 조기영아에서 전폐정맥연결이상은 아직도 외과적으로 쉽게 치료되지 않는 심기형 중 하나이다. 본 연구는 조기 영아기에 외과적으로 교정된 중요한 다른 심기형이 동반되지 않은 전폐정맥연결이상 환아들의 조기 및 중기 성적을 분석하기 위해 시행되었다. 대상 및 방법: 1993년 1월부터 1998년 8월까지 본 교실에서 외과적으로 교정술을 시행받은 전폐정맥연결이상 환아들중 조기 영아 15명의 의무기록을 후향적으로 분석하였다. 남아가 8명, 여아가 7명이었으며 나이는 생후 4일에서 3.5개월(중심나이 생후 22일)이었다. 체중은 1.75 kg에서 4.9kg(평균체중 3.54 kg)이었다. 심기형을 형태별로 보면 심장상부형이 11례, 심장형이 3례, 심장하부형이 1례였다. 6례(40%)에서 폐정맥환류의 협착이 있었다. 13례에서 수술방법으로 완전순환 정지법을 사용하였고 심장상부형과 심장하부형은 우심방 뒤에서 측방으로 접근하여 공통폐정맥과 좌심방을 문합하였는데 매우 가는 비흡수사인 polypropylene 사를 이용하여 연속봉합을 이용하였다. 결과: 1명의 환아가 술후 17일째 패혈증으로 사망하여 수술사망율은 6.7%였다. 이 환아는 술전 인공호홉이 필요하였던 상심실형의 신생아였다. 두명의 만기사망이 있었는데 그 중 1명은 수술 3년 후 집에서 급사하였는데 원인을 알 수 없었고 다른 한명은 술후 폐정맥협착으로 재수술을 시도하고 좋은 상태로 퇴원하였으나 재수술 3개월후 재발된 폐동맥고혈압으로 사망하였다. 두명의 환아에서 폐정맥협착으로 첫 수술 5개월, 10개월후에 각각 재수술이 시행되었다. 이 중 한명은 현재 계속되는 폐동맥고혈압은 있으나 비교적 좋은 상태로 잘 지내고 있다. 만기사망 및 재수술 환아 3명을 제외한 11명(78.6%)의 환아는 25.8$\pm$20.4개월(0.5개월에서 67개월)의 평균 추적기간동안 심초음파검사상 폐정맥환류의 협착이나 폐동맥고혈압의 증거없이 모두 NYHA class I으로 잘 지내고 있다. 결론: 조기 영아에서의 전폐정맥연결이상은 비교적 낮은 수술사망율로 교정 될 수 있었으나 술후 폐정맥 혹은 문합부협착증이 2례(14.3%)에서 발생하였고 재수술의 결과도 좋지 못하였다. 그러나 술후 폐정맥환류에 협착이 없었던 환아들의 상태는 매우 양호하였다.

• Journal of Chest Surgery
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• 제24권10호
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• pp.960-966
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• 1991

From February 1984 through July 1991, 104 infants less than 1 year of age with congenital heart defects underwent open heart repair with conventional cardiopulmonary bypass which occupied 10.7% of all patients with congenital heart defects operated on during same period. There were 66 boys and 38 girls 7 days to 12 months [mean age, 8.2 months]. Four patients were neonates, 8 were 1 to 3 months, 23 were 4 to 6 months, and 69 were 7 to 12 months of age. Mean body weight at repair was 6.9kg and mean BSA, 0.36m2 Indications for operation were intractable congestive heart failure and severe pulmonary hypertension in patients with VSD and severe cyanosis and anoxic spells in patients with TOF. Conditions corrected were VSD[79], TOF[8], AVSD[4], PS[2], PA+IVS[2], TAPVC [2], MR[2], DOLV[l], Truncus arteriosus[1], D-TGA[1], and PA-VSD[1]. Twenty-three of 79 patients with VSD had associated cardiovascular anomalies which included PDA in 16 patients, PS in 9 patients, ASD in 5 patients, LSVC in 2 patients, MR in 1 patient, dextrocardia in 1 patient, and single coronary artery in 1 patient. The hospital mortality rate was 24.0% which was much higher than that of 6% in patients over 1 year of age. The greatest mortality occurred in babies of low weight under 6 months of age, There was no late death. Surviving infants showed marked symptomatic improvement and change in growth patterns. These surgical results were to be overcome with proper pre- and post-operative management and improvement of surgical technique

• Childhood Kidney Diseases
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• 제13권2호
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• pp.222-228
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• 2009

목적 : 선천성 수신증 환아에서 방광요관역류(VUR)가 어떠한 관련이 있는지 또한 요로감염 발생 여부와 어떠한 연관이 있는지 정보가 부족하다. 이에 연구자들은 VCUG를 시행 받은 선천성 수신증 환아를 대상으로, 수신증과 VUR 간의 연관성 및 요로감염 발생 여부에 대하여 분석을 하여 환자관리에 도움이 되는 정보를 마련하고자 하였다. 방법 : 연구는 2002년 1월 1일부터 2008년 12월 31일까지 아주대학교 병원에서 선천성 수신증으로 진단된 생후 1개월 이내의 환아 중, VCUG를 시행 받은 122명을 대상으로 후향적 연구를 하였다. 측정치의 통계분석은 SPSS version 16.0을 사용하여 logistic regression, T-test, chi-square test 등을 실시하였으며, P<0.05인 경우 통계학적으로 유의한 것으로 평가하였다. 결과 : 총 122명의 선천성 수신증 환자 중 남자가 많았고(73%), 주로 편측 수신증을 가진 환자가 많았으며(77%), 그 중 좌측(84.0%) 수신증 환자가 유의하게 많았다. VUR은 총 23명(18.9%)에서 발견되었다. 수신증의 심한 정도는 좌우측에 따른 차이를 보이지 않았으며, VUR의 정도도 좌우측에 따른 차이를 보이지 않았다. 수신증을 1-4단계로 나누었을 때, 각 단계별로 VUR의 발생빈도 차이는 관찰할 수 없었으나, 수신증을 경한 수신증(1-2단계)과 중한 수신증(3-4단계)으로 나누었을 때 중한 수신증에서 VUR의 발생 빈도가 유의하게 높음을 확인할 수 있었다. 또한 영아기 동안에 요로감염 발생을 살펴보면, 수신증이 심하거나 VUR이 있을 경우 그 발생빈도가 높았다. 결론 : 선천성 수신증 환아에서 수신증의 정도가 심할 경우 더욱 VUR의 빈도가 더 높았으며, VUR이 존재할 경우 영아기에 요로감염이 더 흔히 발생함을 알 수 있었다.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• 제36권5호
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• pp.434-437
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• 2010

A melanotic neuroectodermal tumor of infancy (MNTI) is a uncommon osteolytic pigmented neoplasm that primarily affects the jaws of newborn infants. Most patients (> 90%) present with the tumor in the first year of life. Approximately 65% form in the maxilla, 11% in the mandible, 5% in the brain and elsewhere. MNTI is normally benign, but up to 15% may recur and a few have metastasized. Approximately 200 cases of MNTI have been reported but only 2 of them presented as multifocal. A case of MNTI in a 7 month old boy was encountered. The chief complaint was maxillary anterior ridge swelling. The incisional biopsy findings were MNTI. Two months after the first operation, mild swelling of another site was observed. The infant was examined periodically since undergoing two procedures with no recurrence. This case demonstrates the possibility of a multicentric MNTI. We report a multicentric MNTI with a review of the relevant literature.

• Journal of Genetic Medicine
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• 제21권1호
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• pp.14-21
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• 2024

Infantile nystagmus syndrome (INS) refers to congenital forms of nystagmus that are present at birth or during infancy. This syndrome may be caused by afferent visual system disorders or abnormal development of the ocular motor system. INS is a genetically heterogeneous disorder for which there are more than 100 causative genes. Since applying clinical tests for the differential diagnosis of INS can be challenging in early infancy and children, genetic testings such as next-generation sequencing are becoming more important for achieving accurate diagnoses. An improved understanding of the molecular mechanisms of INS may also lead to the development of gene-based therapies for INS. These advantages of genetic testing have the potential to change the diagnostic paradigm of patients with INS. However, the diagnostic pathway based on genetic testing still has several limitations in terms of the therapeutic effect and methodology. This review summarizes genetic and clinical features of INS, and discusses the promise and pitfalls of genetic testing in INS.

• 대한한방소아과학회지
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• 제30권1호
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• pp.45-58
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• 2016

Objectives The purpose of this study is to investigate about chief complaints of Korean pediatrics and adolescent outpatients in the ${\bigcirc}{\bigcirc}$ university hospital for their recent trend. Methods The study was composed of 4,677 new patients aged between 1 month and 20 years who had been visited pediatrics in ${\bigcirc}{\bigcirc}$ university hospital from 2012 January to 2015 December. Results 1. Among those patients, majority was early childhood as 51.4%, followed by early childhood, late childhood, infancy, and adolescence. 2. Chief complaints in this population have been recorded in the fall the most as 29.6%, but the distribution was inconsistent. 3. To categorize, digestive-related chief complaints were the highest as 30.6%, followed by respiratory, supplemental use, growth, psychiatrics. Respiratory-related chief complaints increased dramatically in the fall. 4. In infancy and early childhood, digestive/respiratory complaints were common, whereas, growth complaints were common in late childhood and adolescence. 5. Digestive/respiratory complaints have been over 50% out of all chief complaints annually. Psychiatric and growth-related complaints have been trending up, whereas, supplemental/skin complaints have decreased every year. 6. Specifically, anorexia were the most common in digestive complaints, and rhinitis/sinusitis were top among respiratory complaints. Precocious puberty and developmental disability increased every year. Conclusions The most common chief complaints were digestive and respiratory-related, and precocious puberty and growth issues have been trending up lately. Based on this result, more studies that targets precocious puberty and growth problems are needed.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제20권1호
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• pp.47-54
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• 2017

Purpose: Clinical symptoms associated with Clostridium difficile infection (CDI) can vary widely. Carrier state without apparent symptoms is relatively common during infancy. The objective of this study was to determine the association of C. difficile colonization with bowel habit change and the effect of C. difficile colonization treatment on restoration of normal bowel habit. Methods: Between 2006 and 2014, infants at 1 to 12 months of age with diarrhea for more than 2 weeks who did not improve with conservative care were recruited from Gachon University Gil Medical Center. Infants who were followed up for at least 7 days were included. The presence or absence of C. difficile colonization, effect of metronidazole, and other medical records were reviewed. To determine the association between CDI and bowel habit change, logistic regression analysis was used. Results: Of a total of 126 infants, 74 (58.7%) were male patients. Of the 126 patients, 27 (21.4%) had C. difficile colonization. Significant (p<0.05) risk factors for C. difficile colonization included artificial milk feeding (odds ratio [OR], 4.310; 95% confidence interval [CI], 1.564-11.878), prior rotavirus vaccination (OR, 4.322; 95% CI, 1.018-18.349), and antibiotic use (OR, 4.798; 95% CI, 1.430-16.101). There was improvement in bowel habit after metronidazole therapy (OR, 0.34; 95% CI, 0.15-0.79; p<0.05), regardless of the presence or absence of C. difficile colonization, Conclusion: There was no significant correlation between bowel habit change and C. difficile colonization during infancy. However, metronidazole can be used as an optional method to manage functional gastrointestinal disorders.

• Journal of Chest Surgery
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• 제24권2호
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• pp.115-122
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• 1991

From April 1986 to December 1989, 25 infants under the age of 12 months with tetralogy of Fallot were operated on. Age ranged from 3 to 12 months[mean 8.9$\pm$4.9 months] and mean body weight was 7.8$\pm$ 2.6kg. All the patients were deeply cyanotic, 12 of them experienced anoxic spell. Transannular patch was laid down in 19 patients, in 7 of them monocuspid patch was utilized. Postrepair P RV/LV was measured at operation room in 17 patients[mean 0.48$\pm$0. 14]. Hospital mortality was 20Yo. Causes of deaths include right ventricular failure and low cardiac output. The mortality was closely related with patient`s age and body surface area at operation. Also higher mortality was noticed in patients having major associated anomaly or previous palliative operation, preoperative management with propranolol and transannular repair. 18 patients were followed up for 12 to 50 months with a mean follow-up time of 24 months after operation. There were no late deaths and late ventricular arrhythmia or congestive heart failure was not detected as yet. Redo operation was performed in one case because of residual pulmonic stenosis. Considering several advantages of early primary repair, primary repair of symptomatic infants with tetralogy of Fallot should be encouraged despite somewhat high mortality rate as yet and better results could be anticipated along with improvement of myocardial protection method and postoperative care.

• Journal of Chest Surgery
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• 제21권1호
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• pp.55-61
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• 1988

This report provides follow-up data on 116 patients with congenital cyanotic heart disease, aging 1 month to 13 years [median: 1.8 years], who underwent the modified Blalock-Taussig shunt using polytetrafluoroethylene graft at Seoul National University Hospital between September, 1984 and June, 1987. Among 116 patients complete follow-up studies were done on 95 patients. The mean preoperative arterial oxygen tension was 36 torr. Thirty-Six patients [38%] underwent operation in infancy. Conduit diameters included 4mm [15 cases], 5mm [47 cases], and 6mm [33 cases] sizes. The mean postoperative arterial oxygen tension was 52 torr [P<0.001]. The effectiveness of shunts was evaluated clinically and by shunt murmur, echocardiography and cardiac catheterization with angiography 1 to 31 months after operation. The incidence of shunt occlusion was 9.5% and the mortality was 14.8%. The actuarial patency rate was 83.1 * 6.4% and the actuarial survival rate was 82.5 * 4.5% at 30 months` follow-up for all patients. The effectiveness of the 4mm diameter conduit may be limited. Blalock-Taussig procedure is an effective alternative to the classic B-T shunt in congenital cyanotic heart disease.

• Childhood Kidney Diseases
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• 제8권2호
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• pp.223-228
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• 2004

목적: 요로 감염은 어린이에게 흔한 감염성 질병의 하나로, 특히 어린 영아에서는 증상이 모호하고 신장 손상의 위험성과 신요로계 기형의 동반 가능성이 있는 중요한 질환이다. 저자들은 우리나라 영아에서 월령 6개월 이전에 발생한 발열성 요로 감염의 임상적 특성, 요로계 영상 검사의 유용성과 요로 감염 재발의 위험 인자를 확인하기 위하여 후향적 고찰을 시행하였다. 방법: 1999년 11월부터 2002년 5월 사이에 서울대학교 어린이 병원에서 요로 감염으로 진단받고 치료받은 환아 중, 월령 6개월 이전에 발열로 첫번째 요로 감염을 진단받은 환아를 대상으로 병록지를 고찰하였다. 결과: 대상 환아 90명(남:여 77:13)의 첫번째 발열성 요로 감염의 발병 연령은 $2.5{\pm}1.4$개월이었으며 신장 초음파 검사(90명), 배뇨 방광요도조영술(VCUG[81명])로 모두 45명(방광 요관 역류[VUR] 35명)에서 요로계의 이상이 발견되었다. 1개월 이상-3개월 미만의 남아(30명)에서 초음파 소견이 정상인 경우 VUR이 있을 위험도가 유의하게 낮았다(OR=0.09). 6개월 이상 추적 관찰한 환아 53명(남:여 46:7) 중 29명(남:여 26:3)에서 첫 6개월 동안 요로 감염의 재발이 있었다. 재발군에서 첫번째 요로 감염의 발병 연령이 재발하지 않은 환자군에 비해 높았으나 다른 차이는 발견되지 않았고, 예방적 항생제의 복용이 재발 여부에 영향을 미치지 않았다. 결론: 6개월 미만의 요로 감염 환아는 주로 남아로, 50%에서 요로계의 구조적 이상이 발견되었고, 초음파 검사는 1-3개월의 남아에서 유용하였다. 첫 번째 요로 감염 이후 6개월 이상 추적 관찰한 환자군의 발열성 요로 감염 발생율은 3.7회/환아-년이었으며, 요로계 영상 검사의 이상유무나 예방적 항생제의 투여 여부는 재발과 관련을 보이지 않았다.