신생아 및 영아기의 허쉬슈프렁병 진단

김대연,김성철,김경모,김애란,김기수,김정선,구현우,윤종현,김진천,피수영,김인구,Kim, Dae-Yeon,Kim, Seong-Chul,Kim, Kyung-Mo,Kim, Ellen Ai-Rhan,Kim, Ki-Soo,Kim, Jung-Sun,Goo, Hyun-Woo,Yoon, Chong-Hyun,Kim, Jin-Cheon,Pil, Soo-Young,Kim, I 대한소아외과학회 2002 소아외과 Vol.8 No.1

Diagnosing Hirschsprung's disease is a clinical challenge. Hirschsprung's disease should be considered in any child who has a history of constipation dating back to the newborn period. We examined diagnostic methods and their results retrospectively in 37 neonates and infants who underwent both barium enema and anorectal manometry for the diagnosis of Hirschsprungs disease at Asan Medical Center between January 1999 and April 2001. Two radiologists and a surgeon repeatedly reviewed both of the diagnostic results. In anorectal manometry, thirty-four studies were in agreement with the definitive diagnosis, giving an overall diagnostic accuracy of 91.9 % (neonate; 100 %, infant; 85.7 %). The accuracy and specificity of barium enema was lower than those of anorectal manometry, but sensitivity was higher. There was no significant difference between the two methods. Both studies showed findings consistent with the final diagnosis. However, discordant results needed further evaluation or close observation to diagnose accurately. We conclude that Hirschsprungs disease should not be diagnosed by only one diagnostic method.

미세아의 외과적 문제점들

김대연,김성철,김애란,김기수,피수영,김인구,Kim, Dae-Yeon,Kim, Seong-Chul,Kim, Ellen Ai-Rhan,Kim, Ki-Soo,Pi, Soo-Young,Kim, In-Koo 대한소아외과학회 2006 소아외과 Vol.12 No.1

Recent advances in neonatal management have resulted in a dramatic increase in survival of very low birth weight infants. These critically ill infants, however, continue to pose significant challenges in management and ethics. There is little information on the outcome of the micropremie (birth weight less than 800 g) that require surgery. The records of 171 micropremies treated over a 15 year period (beginning in 1989) at Asan Medical Center was reviewed retrospectively. Forty-one (24.0 %) infants required surgical interventions by pediatric surgeons. There were 90 boys and 81 girls. The smallest infant, weighed 396g at birth, had esophageal atresia and died before surgery. The smallest survivor, birth weight 645 g, received anenterostomy for necrotizing enterocolitis at the weight of 590 g. The gestational age of the group rangedfrom 21 to 36 weeks. The most common surgical problem was inguinal hernia. There were 20 inguinal hernias, and repairs were performed on17 infants. Excluding 2 cases, hernia repair was performed at the time of discharge. There was only one recurrence of adirect inguinal hernia. Necrotizing enterocolitis developed in 17 patients, 11 were operated upon, two had peritoneal drainages, and 9 had enterostomies. Five of 11 surgical infants died after operation and three of the nonsurgical infants died of various complications. Although micropremies have potentially high risks of serious complications and death, the outcome can improve with careful surgical observation and judgment.

선천성 식도무공증의 임상적 고찰

김성철,김대연,김애란,김기수,피수영,김인구,Kim, Seong-Chul,Kim, Dae-Yeon,Kim, Ellen Ai-Rhan,Kim, Ki-Soo,Pi, Soo-Young,Kim, In-Koo 대한소아외과학회 2003 소아외과 Vol.9 No.1

This study reviews 14 years' experience treating esophageal atresia with special emphasis on the clinical profile and outcome. From May 1989 to February 2003, 65 cases of esophageal atresia (EA) were treated at Asan Medical Center. Boys outnumbered girls 2.4 to 1. Prematutity and low birth weight were 27.7% and 38.5%. Esophageal atresia with distal tracheoesophageal fistula (TEF) was the most common type (87.7%), followed by pure EA and H type fistula. Forty-six patients (70.8%) had one or more associated anomalies, cardiac malformations were the most common. Duodenal atresia was found in 7 cases. There were 6 patients (9.2 %) with VATER cluster. VACTERL cluster was present in 18 patients (27.7%), one of who fulfilled the complete syndrome. Waterston group A, B and C made up 21.5%, 40.0% and 38.5% of the total group. Surgical treatment was attempted in 63 patients and deferred in 2 who had severe associated malformations. For EA with distal TEF, primary esophago esophagostomy was carried out in 51 cases, and division of TEF and gastrostomy in 4 cases and no operation in 2 cases. For pure EA, colonic graft was done in 2 after gastrostomy and esophagostomy, and esophago esophagostomy was performed in 2 after gastrostomy. Two patients with pure EA are waiting for the second operation after gastrostomy. Division of TEF was carried out in 2 cases with H type TEF. The overall survival rate was 76.9%, and survival by Waterston classification was 100% in group A, 80.8% in B and 60.0% in C. Thorough workup for associated anomalies, interdepartmental approach and more careful surgical decision and technique are required to improve the outcome of EA.

극소 저출생 체중아에서 비타민 A 보충요법의 만성 폐질환에 대한 예방 효과

김훈기 ( Hun Gy Kim ),박성종 ( Sung Jong Park ),이정주 ( Jung Ju Lee ),김영돈 ( Young Don Kim ),김경아 ( Kyueng Ah Kim ),김애란 ( Ellen Ai Rhan Kim ),김기수 ( Ki Soo Kim ),피수영 ( Soo Young Pi ) 대한주산의학회 2001 大韓周産醫學會雜誌 Vol.12 No.3

만삭아에 발생한 괴사성 장염

김대연,김성철,김경모,김애란,김기수,피수영,김인구,Kim, Dae-Yeon,Kim, Seong-Chul,Kim, Kyung-Mo,Kim, Ellen Ai-Rhan,Kim, Ki-Soo,Pi, Soo-Young,Kim, In-Koo 대한소아외과학회 2003 소아외과 Vol.9 No.1

Necrotizing Enterocolitis (NEC) is usually a disease of premature infants, but occasionally it affects the term neonate. Twenty-five infants with NEC were treated at Asan Medical Center between January 2000 and December 2002, and 13 of them were term infants. In each case, the diagnosis of NEC was established by a clinical illness fulfilling the Bell's stage II or III NEC as modified by Walsh or by surgical findings. There were six males and seven females. The birth weight was from 1,960 to 3,700 g. The age at diagnosis was from 1 to 40 days. Four patients had congenital heart disease: one of who had hypothyroidism and cleft palate. Abdominal distension was present in all, and bloody stools in four. One patient had history of hypoglycemia, three had Rota viral infection. Eight patients had leucopoenia (<$5.0{\times}10^9/L$), seven had thrombocytopenia (<$100{\times}10^9/L$), and three severe thrombocytopenia (<$50{\times}10^9/L$). Laparotomy was required in 10 of the 13 patients. Indications for operation in the acute phase were failure to respond to aggressive medical therapy in five, and perforation in three patients. There were two late phase operations for intestinal stricture and fistula. There were no operative complications. Ten of thirteen patients survived (76.9%). Two patients died of septic complication. There was a delayed death due to heart failure. There was a significant difference in survival according to platelet count ($50{\times}10^9/L$) (p<0.05). Congenital heart disease and Rota viral infection are associated with NEC in term infants and thrombocytopenia and leucopoenia may be surgical indications.

신생아 중환자실에서 extended spectrum β-lactamase를 생성하는 Klebsiella pneumoniae 집단 보균 발생의 분자 역학적 조사 및 추적관찰

전누리,김미나,정재심,김양수,김애란,김기수,피수영,Jun, Nu-Lee,Kim, Mi-Na,Jeong, Jae-Sim,Kim, Yang-Soo,Kim, Ellen Ai-Rhan,Kim, Ki-Soo,Pi, Soo-Young 대한소아청소년과학회 2006 Clinical and Experimental Pediatrics (CEP) Vol.49 No.2

목 적 : 2000년 6월과 7월에 본원 신생아 중환자실에서는 ESBL 생성 K. pneumoniae에 의한 패혈증이 집단 발생하여 전체 환아에 대한 보균 상태를 파악하고 감염관리를 위해 감시배양을 실시하였다. 당시 집단 보균 상태임을 발견하여 보균자에 대한 격리를 실시한 결과 집단보균을 해결할 수 있었으나 대부분의 환아가 보균 상태로 퇴원하였다. ESBL 생성 K. pneumoniae는 대변 내 균이 장착되어서 입원 환아들간에 집단 감염을 일으킬 수 있으며, 이러한 균주에 의한 집단감염은 치료와 예후에 중요한 인자로 작용될 것으로 생각된다. 이에 저자들은 집단보균 발생시 분리된 ESBL 생성 K. pneumoniae의 분자 역학적 특징과 보균환아들의 추적관찰 결과를 알아보고자 본 연구를 시행하였다. 방 법 : 2000년 7월 28일부터 12월 30일까지 입원환아를 대상으로 직장내 도말법으로 감시배양검사를 시행하였다. 감시배양검사는 재원환아들에서 1주 간격으로 시행하였고, 신환은 입원 당일에 시행하였다. 균주의 형별 검사를 위해서 Pulsed-field gel electrophoresis(PFGE)를 시행하였고, 보균 상태로 퇴원했던 환아들은 외래에서 대변 배양검사로 추적 관찰하였다. 결 과 : 감시 배양기간 중 총 80명(28.5%)의 환아에서 보균이 확인되었고, 퇴원시 5명(6.3%)에서 음성이 확인되었다. PFGE를 시행한 65명의 타이핑 결과, 일회의 PFGE를 시행한 53명에서 분리된 균주의 염색체 유전자 분획양상은 집단클론 6가지, 단독클론 10가지형으로 분류되었고, 집단 클론 중 A형이 28명(52.8%)으로 가장 많았고, B형이 11명(20.8%), C, D, F, G형이 각각 1명(1.9%)이었다. 2회 이상 검사를 시행한 12명 중 초기검사에서는 A형이 10명(83.3%)으로 월등히 많았고 B형은 2명(16.7%)이었으며, 추적검사에서 분획 양상이 변화된 경우는 6명(50%)이었고, 이들은 A나 B로 변화된 경우가 각각 2명(33.3%)이었다. 변화되지 않은 6명(50%)은 모두 A형으로 남아 있었다. 월별 PFGE 양상은 처음 배양시 집단클론 A, B, C, D형 4가지와 단독클론 세 가지형이었으나 감염 관리를 하면서 11월부터 집단클론 A, B 두 가지형으로 나타나는 양상을 보였고, A형이 더 우세하였다. 보균된 상태로 퇴원한 75명 중 외래 추적관찰이 가능했던 30명을 대상으로 대변 배양검사를 시행한 결과 모두 음성이 확인되었다. 결 론 : 다양한 클론의 균주에 의한 집단보균 상태는 감염 관리를 하면서 단일 클론으로 변화하는 양상을 보였고, A형이 우세한 것으로 보아 집단 보균을 일으킨 유형 중 주 집단 클론은 A형이었음을 알 수 있었다. ESBL 생성 K. pneumoniae 보균 상태는 중환자실내에 입원기간 중에는 거의 지속되지만, 지역사회로 복귀하면 전부 해제되는 것으로 보인다. Purpose : The aims of this study included assessment of molecular-epidemiologic features during an outbreak of colonization of extended spectrum ${\beta}$-lactamase producing Klebsiella pneumoniae(ESBL-KPN) and re-evaluation of their colonized status one year later. Methods : Rectal swab cultures for ESBL-KPN from all hospitalized infants and newly admitted infants were obtained during the outbreak of colonization from July to December, 2000. The pattern of XbaI-digested chromosomal DNA of isolates were analyzed by pulsed-field gel electrophoresis. Weekly rectal swab cultures were obtained during the outbreak until patients were either discharged or decolonized. Patients discharged after being colonized had follow up stool cultures a year later. Results : A total of 80 patients(28.5 percent) were colonized. Of those, 53 whose pulsed-field gel electrophoresis(PFGE) was possible only once, were ESBL-KPN grouped into six cluster clones and 10 single clones : 28 patients(52.8 percent) were colonized with type A, the most common clone, followed by type B in 11 patients(20.8 percent). Of those 12 patients in whom serial PFGE was done more than twice, type A was predominant. Narrowed-down in strains occurred from types A, B, C, D and three single clones at initiation of the study into types A and type B after three months of strict infection control. Among 75 patients(93.7 percent) who were sent home after being colonized, 30 patients were re-called for stool cultures a year later : All of them were decolonized. Conclusion : This study demonstrates the importance of infection control as the diversity of ESBL-KPN strains could be narrowed into fewer strains. Colonization of ESBL-KPN could be reversed upon return to the community.

체외수정시술로 출생한 쌍생아의 임상적 경과에 대한 비교 분석

김경아,민우경,임재우,전누리,원혜성,김정훈,김애란,이필량,이인식,김기수,김암,피수영,Kim, Kyung-Ah,Min, Uoo-Gyung,Lim, Jae-Woo,Jun, Nu-Lee,Won, Hye-Sung,Kim, Chung-Hoon,Kim, Ellen Ai-Rhan,Lee, Pil-Ryang,Lee, In-Sik,Kim, Ki-Soo,Kim, Ahm,Pi 대한소아청소년과학회 2003 Clinical and Experimental Pediatrics (CEP) Vol.46 No.3

목 적 : 불임의 증가와 함께 체외수정시술에 의한 신생아의 출생이 점차 증가하고 있으나, 이들에 대한 임상적 예후와 경과에 대한 연구가 미비한 실정으로 본원에서 출생한 체외수정 쌍생아의 임상적 경과 및 예후를 알고자 본 연구를 시행하였다. 방 법 : 1995년 1월부터 2000년 6월까지 6년간 서울아산병원에서 분만된 신생아 460쌍 중 250쌍의 자연 수정에 의한 쌍생아(대조군)와 체외 수정 시술에 의해 출생한 156쌍의 쌍생아를 대상으로 재태연령, 출생체중, 성비, 입원률, 1분/5분 Apgar 점수, 동맥관개존증의 발생 및 인도신 치료 여부, 신생아 호흡 곤란 증후군, 일과성 빈호흡, 황달, 미숙아망막증, 뇌출혈, 괴사성 장염, 패혈증, 전해질 이상, 선천적 기형의 발생 유무를 비교하였으며, 입원기간과 사망률을 알아보고 비교 분석하였다. 또한 산모의 나이와 출산력 및 조기양막파열, 임신성 고혈압, 조기진통의 발생 여부와 임신 중의 입원횟수와 입원기간을 비교하였다. 결 과 : 6년간 본원에서 출생한 쌍생아는 총 460쌍이었으며 이중 체외수정시술에 의해 출생한 쌍생아는 156쌍(34.1%)이었다. 산모의 과거력과 임신성 합병증, 임신기간, 출산 형태에 대한 비교에서 산모의 나이, 출산력, 입원기간 등은 두 군 사이에 차이가 없었으나, 체외수정시술을 받은 산모에서 조기진통의 횟수가 많았고, 입원 횟수가 의미있게 많았다. 분만 형태의 비교에서 체외수정시술을 받았던 군이 자연수정군에 비해 수술로 분만한 경우가 의미있게 많았으며(93% vs 82%) 응급 수술보다 정규 수술이 의미있게 많았다. 신생아의 비교에 있어서 몸무게, 성비, 동맥관개존증의 발생 및 인도신 치료 여부, 신생아 호흡 곤란 증후군, 일과성 빈호흡, 황달, 미숙아망막증, 뇌출혈, 괴사성 장염, 패혈증, 선천적 기형의 발생 유무, 생존율, 입원기간 등은 두 군간에 의미 있는 차이가 없었고, 1분, 5분 Apgar 점수가 자연수정군에서 높았으며, 전해질의 이상소견이 체외수정시술군에서 의미있게 많았다. 체외수정시술에 의한 쌍생아군에서 첫 번째 아가와 두번째 아가를 비교하였을 때 출생체중, 신생아 집중치료실 입원율, 입원기간 등은 차이는 없었으나, 신생아 호흡 곤란 증후군, 동맥간개존증, 패혈증, 괴사성장염 등은 두번째 아가에서 유의하게 많았다. 결 론 : 체외수정시술에 의한 쌍생아의 출생은 전체 쌍생아 출생아의 34.1%로 많은 부분을 차지하였고, 산과적 합병증 및 주산기 예후를 비교하였을 때 체외수정군과 자연수정군이 유의한 차이가 없었다. Purpose : To examine various neonatal outcomes and perinatal factors resulting from assisted reproduction compared to that of spontaneous conception. Methods : This is a retrospective study. The control cases were all twins of spontaneous conception born between periods from January 1995 to June 2000. The study cases were identified from twins conceived by assisted reproduction in the same time peried. A total of 460 sets of twins consisted of 250 twins of spontaneous conception and 156 twins of assisted reproduction were studied. The primary outcomes were neonatal morbidity and mortality and the secondary outcomes were perinatal factors including number, length and cost of hospitalization for the delivery. Results : No differences were seen in various neonatal factors including gestational age, birth weight and incidences of respiratory distress syndrome, patent ductus arteriosus, necrotizing enterocolitis, hyperbilirubinemia, sepsis, intraventricular hemorrhage and the length of hospitalizations. Lower one minute and five minute Apgar scores and frequently encountered electrolyte abnormalities were observed in neonates of assisted reproduction. In general, the second twin of assisted reproduction had increased incidences of respiratory distress syndrome, sepsis and necrotizing enterocolitis than the first twin. Increased frequencies of preterm labor, hospitalization and elective cesarean section were seen among mothers who underwent artifical conception. However, overall hospital costs in terms of mothers hospitalization for the delivery and neonates hospitalization did not show differences. Conclusion : Assisted reproduction twins had similar neonatal morbidities, mortalities and perinatal morbidities compared to those born by spontaneous conception.

신생아기에 진단된 Netherton 증후군 1례

이은희,김애란,김기수,조범진,고재경,피수영,Lee, Eun-Hee,Kim, Ellen Ai-Rhan,Kim, Ki-Soo,Cho, Beom-Jin,Koh, Jai-Kyoung,Pi, Soo-Young 대한소아청소년과학회 2003 Clinical and Experimental Pediatrics (CEP) Vol.46 No.4

Netherton 증후군은 어린선양 피부병증, 머리카락이상, 아토피 소질 등 3가지 임상양상을 특징으로 하는 드문 질병이다. 저자들은 임상소견, 피부조직 검사와 머리카락 현미경 소견을 통하여 신생아기에 Netherton 증후군으로 진단하고 추적 관찰하고 있기에 간단한 문헌 고찰과 함께 보고하는 바이다. Netherton's syndrome is an unusual disorder which consists of triad of ichtyosiform dermatosis, multiple defects of hair shaft and an atopic diathesis. The finding of bamboo hair is pathognomic in Netherton's syndrome and the ichthyosiform dermatosis may consist of either ichtyosis linearis circumflexa or congenital ichthyosiform erythroderma. Often, variability in the clinical features leads to a delay in diagnosis in many cases. We report a case of Netherton's syndrome diagnosed in the neonatal period. The patient presented with severe ichthyosis and confirmed microscopically distinctive bamboo hair.

융모막혈관종증에 의해 발생한 태아수종 1례

서주희 ( Ju Hee Seo ),김애란 ( Ellen A Kim ),김기수 ( Ki Soo Kim ),피수영 ( Soo Young Pi ) 대한주산의학회 2009 Perinatology Vol.20 No.3

Apart from the common etiologies of neonatal hydrops fetalis, a miscellaneous group of rare causes of them include abnormalities of the placenta, namely, chorangioma (chorioangioma). Chorangiomatosis is multiple small chorangiomas, a rare tumor occurring in 1% of pregnancies, of varying sizes in the placenta. When chorangioma is large enough it can cause high output cardiac failure of the fetus and results in non-immune hydrops. We report a case of neonatal hydrops fetalis caused by placental chorangiomatosis in which the placenta could have been easily overlooked. We suggest evaluating every placenta for biopsy in cases of neonatal hydrops fetalis especially when the other causes of hydrops fetalis have been ruled out.

괴사성 장염과 선천성 심질환을 지닌 신생아의 영양관리

김애란 한국소아심장연구회 2004 소아심장 Vol.8 No.1