임상 ; 당뇨병 산모에서 출생한 신생아의 선천성 기형 진단에서의 산전 정밀 초음파 검사의 역할

김영아 ( Young A Kim ),임진아 ( Gin A Lim ),이병섭 ( Byong Sop Lee ),심재윤 ( Jae Yoon Shim ),원혜성 ( Hye Sung Won ),김애란 ( Ai Rhan Kim ),김기수 ( Ki Soo Kim ),이필량 ( Pil Ryang Lee ),김암 ( Ahm Kim ),피수영 ( Soo Young Pi ) 대한주산의학회 2010 Perinatology Vol.21 No.3

목적: 당뇨병 산모아는 비당뇨병 산모아에 비해 선천성 기형의 빈도가 높다. 연구자들은 당뇨병 산모에서 산전정밀 초음파의 태아 기형 선별 검사로서의 유용성을 확인하고 신생아에 대한 출생 후 진단적 검사의 필요성에 대하여 조사하였다. 방법: 2004년 1월부터 2009년 2월까지 서울아산병원에서 임신성 또는 임신전성 당뇨로 진단받고 태아 기형의 검사 목적으로 정밀 초음파가 시행 된 218명과 이들에서 출생한 226명의 신생아를 대상으로 하여 의무기록을 후향적으로 검토하였다. 산전 진단 소견과 출생 후 진찰 및 초음파 검사 결과와의 차이점을 비교하였다. 결과: 산전 정밀초음파 검사 및 산후 진찰 및 초음파 검사 상 각각 32명(14.2%) 및 35명(15.5%)에서 이상소견이 발견되었다. 산전 진단에서 정상이었던 194명의 신생아 중 7례(3.6%)에서만 산후에 선천성 기형이 발견되었으며 이들은 모두 신생아기 진찰로 진단되거나 의심할 수 있었다. 임신 24-28주경 공복 혈당이 120 mg/dL이상인 경우와 인슐린 치료를 받은 경우 선천성 기형의 발생이 의미 있게 증가하였다. 결론: 숙련된 산과의사에 의하여 시행된 산전의 정밀 초음파 검사는 당뇨병 산모아의 선천성 기형의 발견 및 신생아기 치료방침 결정에 유용하였다. 당뇨 산모아에서 산전 정밀 초음파 상 이상이 없었던 경우 신생아기의 추가적인 진단적 초음파 검사는 필요하지 않을 것으로 생각된다. Objective: Infants of diabetic mothers have higher incidence of congenital malformations compared with those of non-diabetic mothers. We investigated the usefulness of prenatal level II or “targeted” ultrasonography (TUS) as a diagnostic tool to identify congenital abnormalities in infants of diabetic mothers. Methods: We retrospectively reviewed the medical records of 218 mothers diagnosed as pregestational or gestational diabetes in whom prenatal TUS was performed in Asan Medical Center between 2004 and 2009. The prenatal TUS findings were compared with the congenital abnormalities found in the infants (n=226) of the diabetic mothers by physical examination and ultrasonography (including echocardiography). Maternal risk factors associated with congenital anomalies were also investigated. Results: The incidence of congenital anomalies was 14.2% (n=32) in prenatal TUS and 15.5% (n=35) postnatally, respectively. Only 7 cases (3.6%) out of the 194 infants with normal prenatal TUS findings were found to have congenital abnormalities diagnosed postnatally. All of the abnormalities in these 7 infants could be detected or suspected by physical examination after birth. Maternal fasting glucose level >120 mg/dL and insulin therapy were significant risk factors for predicting congenital abnormalities in infants of diabetic mothers. Conclusion: Prenatal TUS performed by experienced obstetricians had a good reliability in the diagnosis of congenital anomalies in infants of diabetic mothers. Need for universal screening of ultrasound or echocardiography in these infants should be questioned especially in the cases in which prenatal TUS was performed.

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 저출생체중아에서의 소장천공

김대연,김성철,김애란,김기수,피수영,김인구,Kim, Dae-Yeon,Kim, Seong-Chul,Kim, Ai-Rhan,Kim, Ki-Soo,Pi, Soo-Young,Kim, In-Koo 대한소아외과학회 2001 소아외과 Vol.7 No.2

With the advances in neonatal intensive care, pediatric surgeons experience very low birth weight infants, weighing <1,500 g, more frequently. We report our 14 cases of very low birth weight infants with intestinal perforations without congenital causes, at the Asan Medical Center during the 11-year period from 1989 to 2000. The average birth weight was 919 g(563-1,490), and average gestational age was 206 days(161-286). There were nine males and five females, Operation was performed at an average age of 14.0 days(3-38). Ten neonates with symptomatic PDA were given indomethacin in an attempt to close the ductus. Bowel perforation involved the jejunum in two and ileum in twelve. At laparotomy, there were seven focal intestinal perforations, five typical NEC, one intussusception, and an unknown cause, Four neonates underwent resection and anastomosis of the bowel, and nine underwent exteriorization. One underwent resection and anastomosis after peritoneal drainage. Four patients had postoperative complications; two leakage of anastomosis, one stoma necrosis, and one internal herniation. Seven of fourteen patients survived(50.0 %). Seven patients died of septic complication. There was a significant difference in the birth weight and gestational age in survivors compared with those who died(p<0.05). There was an increased risk of bowel perforation in indomethacin treatment for PDA. Careful clinical observation and keen judgment are essential for this particular group of infants.

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.

치료 원칙 변화에 따른 신생아 Bochdalek 탈장의 예후

서진영,남소현,김대연,김성철,김애란,김기수,피수영,김인구,Seo, Jin-Young,Nam, So-Hyun,Kim, Dae-Yeon,Kim, Seong-Chul,Kim, Ai-Rhan E.,Kim, Ki-Soo,Pi, Soo-Young,Kim, In-Koo 대한소아외과학회 2006 소아외과 Vol.12 No.2

1989년 3월부터 2005년 5월까지 울산대학교 의과대학 서울아산병원 소아외과에서 치료받은 신생아 Bochdalek 탈장 67예를 대상으로 하였다. 1989년 3월부터 1999년 말까지 조기수술, 과호흡, 수술시 흉관의 삽입을 시행한 I기(33예)와, 2000년 1월부터 2005년 5월까지 지연 수술, 최소한의 호흡 요법과 고빈도 환기 요법, 일산화질소 흡입 요법을 병행하고, 수술시 흉관의 삽입을 제한한 II기(34예)로 나누어 생존율을 비교하였다. I기의 경우 33예 중 20예가 생존하여 60.6 %, II기의 경우 34예 중 25예가 생존하여 73.5 %의 신생아기 생존율을 보였으나 통계학적 유의성은 없었다. 하지만 II기에서 생존율이 향상된 경향을 보임으로, II기에 불필요한 수술은 피할 수 있었던 것으로 생각되며, 나쁜 예후 인자를 갖는 예에서 치료 원칙의 변화가 의미 있는 역할을 했을 것으로 생각된다. 지연수술, 호흡요법, 일산화질소 흡입요법 각각의 영향에 대한 조사를 할 수 없었던 것은 이번 연구의 한계이다. 그러나 수술 전에 충분하게 제반 상태가 안정된 후에 시행하는 지연 수술이 바람직하다고 생각되며, 수술시 흉관 삽입은 피하는 것이 좋겠다. 수술 전후의 고빈도 환기 요법과 에 대해서는 좀 더 연구가 필요할 것으로 보인다. There are considerable controversies in the management of congenital diaphragmatic hernia. By 1997, early operation, routine chest tube on the ipsilateral side and maintainingrespiratory alkalosis by hyperventilation were our principles (period I). With a transition period from 1998 to 1999, delayed operation with sufficient resuscitation, without routine chest tube, and permissive hypercapnia were adopted as our practice. High frequency oscillatory ventilation (HFOV) and nitric oxide (NO) were applied, if necessary, since year 2000(period II). Sixty-seven cases of neonatal Bochdalek hernia from 1989 to 2005 were reviewed retrospectively. There were 33 and 34 cases in period I and II, respectively. The neonatal survival rates were 60.6 % and 73.5 %, respectively, but the difference was not significant. In period I, prematurity, low birth weight, prenatal diagnosis, inborn, and associated anomalies were considered as the significant poor prognostic factors, all of which were converted to nonsignificant in period II. In summary, improved survival was not observed in later period. The factors considered to be significant for poor prognosis were converted to be nonsignificant after change of the management principle. Therefore, we recommend delayed operation after sufficient period of stabilization and the avoidance of the routine insertion of chest tube. The validity of NO and HFOV needs further investigation.

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.

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.

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.

미숙아망막병증의 출생 후 위험인자

김태임(Tae Im Kim),손준홍(Jun Hong Sohn),윤영희(Young Hee Yoon),김기수(Ki Soo Kim),피수영(Soo Young Pi) 대한안과학회 2000 대한안과학회지 Vol.41 No.5

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.