국제 결혼 외국인 산모에서 태어난 신생아의 주산기 문제 및 예후

송은송,최영륜 한국모자보건학회 2008 한국모자보건학회 학술대회 연제집 Vol.2008 No.2

임신 31주 산모의 복부 자상으로 인해 발생한 태아 두부 손상

송은송 ( Eun Song Song ),김수영 ( Soo Young Kim ),최영륜 ( Young Youn Choi ),김윤하 ( Yoon Ha Kim ),송태복 ( Tae Bok Song ) 대한주산의학회 2009 Perinatology Vol.20 No.3

Penetrating trauma in a fetus is uncommon but may cause potentially life-threatening injuries. We experienced a case of preterm infant who delivered by C-section and showed two deep laceration wounds on face, facial nerve palsy, skull fracture and intracranial hemorrhage due to penetrating injury of an abdominal stab wound in a 31 weeks pregnant woman.

다량의 태아-모체간 수혈로 인한 중증 신생아 빈혈 2예

송은송 ( Eun Song Song ),김기환 ( Ki Hwan Kim ),김소연 ( So Youn Kim ),박천학 ( Chun Hak Park ),최영륜 ( Young Youn Choi ) 대한주산의학회 2004 Perinatology Vol.15 No.2

Feto-maternal hemorrhage, the presence of fetal red blood cells in the maternal circulation, occurs in up to 75% of pregnancies. But its volume is usually very small. Feto-maternal hemorrhage of more than 30 ml of whole blood is relatively rare. The key features that lead to early diagnosis are the maternal history, fetal monitoring, the clinical and laboratory findings of anemia and a negative Coombs` test. Diagnosis is confirmed by Kleihauer-Betke test. Perinatal problems include fetal distress, neonatal anemia, hypovolemic shock, and death. The fetal outcome depends on the amount and rate of bleeding. The initial hemoglobin level was a better predictor than the volume of bleeding. We present two cases of severe neonatal anemia associated with massive feto-maternal hemorrhage, which was confirmed by Kleihauer-Betke test.

단일 제대 동맥을 가진 건강한 신생아에서 신장 초음파 검사의 유용성

강주미,송은송,국진화,이형신,김소영,최영륜,Kang, Ju-Mi,Song, Eun-Song,Kuk, Jin-Hwa,Lee, Hyung-Sin,Kim, So-Young,Choi, Young-Youn 대한소아청소년과학회 2003 Clinical and Experimental Pediatrics (CEP) Vol.46 No.9

목 적 : 단일 제대 동맥을 가진 건강한 신생아의 약 1/3에서 위장관 폐색 및 요로 기형을 동반할 수 있으므로 요로 기형을 발견하기 위해 초음파 검사를 관습적으로 시행하고 있지만 실제초음파 검사상 이상이 발견되는 경우는 드물다. 이에 저자는 단일 제대 동맥을 가진 건강한 신생아에서 신장 초음파 검사의 유용성을 알아보고자 본 연구를 실시하였다. 방 법 : 1995년 1월부터 2002년 7월까지 출생 후 이학적 검사상 단일 제대 동맥이 있는 신생아에서 단일 제대 동맥이면서 증후군의 일부이거나 수술적 치료가 필요한 심각한 질환이 동반된 경우는 제외하고 건강한 신생아 총 30명을 대상으로 하였다. 임상적 배경과 출생 72시간 이후 신장 초음파를 실시하였고, 신장초음파상 이상이 있는 경우 지속적 추적관찰과 더불어 배뇨성 방광요도 조영술 및 technetium-99m-mercaptoacetyl-triglycerine(MAG3) 신스캔 검사를 실시하여 수신증의 크기, 폐쇄의 정도 및 신기능 변화를 분석하였다. 결 과 : 총 30명의 남녀비는 1 : 1이었으며 평균 재태 연령은 $38.0{\pm}2.56$주, 평균 출생 체중은 $2,780{\pm}690g$이었다. 단일 제대동맥을 가진 건강한 신생아 30명 중 신장 초음파상 이상이 발견 된 례는 5명으로 16.7%이었고, 의미 있는 요로 기형은 1명으로 3.3%이었다. 이상이 발견된 5명 중 4명은 경도의 수신증으로 추적 검사상 자연 소실되었고, 1명은 심한 수신증으로 배뇨성 방광 요도 조영술상 역류는 없었고 MAG3 검사상 좌측 신장 기능 저하가 보여 외래 추적 관찰 중이다. 결 론 : 단일 제대 동맥을 가진 건강한 신생아에서 신장 초음파 검사상 발견된 신장 이상은 16.7%로 일반인에서 보이는 신장 이상의 빈도와 큰 차이는 없었고 1명을 제외한 4명(80.0%)에서 자연 소실되었으므로, 비용-이익 면에서 볼 때 단일 제대 동맥을 가진 건강한 신생아에서 관습적으로 신장 초음파 검사를 실시하는 것에 대해 고려해 볼 필요가 있다고 사료된다. Purpose : It is known that single umbilical artery is frequently associated with gastrointestinal or urogenital anomaly, however, routine renal sonography has been debated in healthy neonate with isolated single umbilical artery. This study is designed to determine the usefulness of routine renal sonography in apparently healthy infants with an isolated single umbilical artery. Methods : Thirty healthy neonates with a single umbilical artery without a major anomaly from January 1995 to July 2002 were enrolled. The authors investigated the clinical background of babies and their mothers after renal sonography after 72 hours of age. When the abnormalities were found at the first renal sonography, the severity of hydronephrosis and degree of obstruction and renal function were analyzed by follow up renal sonography, voiding cystourethrography(VCUG) and technetium-99m-dimercaptosuccinic acid(DMSA) scan or technetium-99m-mercaptoacetyl-triglycerine (MAG3) scan. Results : Among the 30 healthy patients with isolated single umbilical artery, five patients(16.7%) showed abnormalities on first renal sonography with one major(3.3%) and four(13.4%) minor renal anomaly(minimal or mild hydroneohrosis). One major renal anomaly(severe hydronephrosis) showed severe decreased renal function on MAG3 scan without reflux, and the other four minor regressed spontaneously on follow up study. Conclusion : The value of routine early renal sonograpy for detecting renal anomaly in healthy infants with an isolated single umbilical artery remained unclear because most of the anomalies would regress spontaneously in the follow up study.

Lung torsion after tracheoesophageal fistula repair in an infant

양은미,송은송,장해인,정인석,최영윤 대한소아청소년과학회 2013 Clinical and Experimental Pediatrics (CEP) Vol.56 No.4

Lung torsion is a very rare event that has been reported in only 9 cases in the pediatric literature but has not yet been reported in Korean infants. We present a case of lung torsion after tracheoesophageal fistula repair in an infant. Bloody secretion from the endotracheal tube and chest radiographs and computed tomographic scan results indicated lung torsion. Emergency exploration indicated 180°torsion of the right upper lobe (RUL) and right middle lobe (RML). After detorsion of both lobes, some improvement in the RUL color was observed, but the color change in the RML could not be determined. Although viability of the RML could not be proven, pexy was performed for both the lobes. Despite reoperation, clinical signs and symptoms did not improve. The bronchoscopy revealed a patent airway in the RUL but not in the RML. Finally, the RML was surgically removed. The patient was discharged on the 42nd day after birth.

The first report of a chromosome 1q44 deletion in Korea

조주현,송은송,김희나,오범석,최영윤 대한소아청소년과학회 2014 Clinical and Experimental Pediatrics (CEP) Vol.57 No.6

The deletion of the distal long arm of chromosome 1 is associated with a characteristic facial appearanceand a pattern of associated malformations. Characteristic manifestations include a round face withprominent ‘cupid's bow’ and downturned corners of the mouth, thin vermilion borders of lips, a longupper lip with a smooth philtrum, a short and broad nose, epicanthal folds, apparently low-set ears,micrognathia, microcephaly, abnormal hands and feet, variable cardiac or genital anomalies, moderateto severe mental retardation, and growth retardation. Using fluorescent in situ hybridization (FISH)analysis to map precisely the deletion, we present a case of chromosome 1q44 deletion with craniofacialcharacteristics, multiple congenital anomalies, and growth and psychomotor retardation. Incomparison with other reported cases of 1q43-44 deletion, the subject does not show hydrocephalus,seizure, syn- or polydactyly of hands, and a urogenital anomaly. However, an arachnoid cyst, pinpointdimple on the midline of the forehead, a right-sided supernumerary nipple and auricular pit, polydactylyof the right foot, adducted thumb, and flexion restriction of the proximal interphalangeal joint with asimian line in both hands were observed additionally.

Clinical Utility of Echocardiography for the Diagnosis and Prognosis in Children with Bronchopulmonary Dsyplasia

최영얼,조화진,송은송,정인석,윤남식,최영연,마재숙,조영국 한국심초음파학회 2016 Journal of Cardiovascular Imaging (J Cardiovasc Im Vol.24 No.4

Background: Bronchopulmonary dysplasia (BPD) may result in chronic pulmonary artery hypertension and right ventricular(RV) dysfunction. Various echocardiographic assessments of RV dysfunction have been used to determine whether echocardiographicmeasurements of premature infants with BPD could provide sensitive measures of RV function that correlates with BPDseverity. Methods: Twenty-eight control subjects without BPD (non BPD group), 28 patients with mild BPD, 11 patients with moderateBPD, and six patients with severe BPD underwent echocardiograms with standard measurement such as ejection fraction byM-mode, tricuspid regurgitation pressure gradient, myocardial performance index (MPI) derived from pulse Doppler, and tissueDoppler imaging (TDI) measurements. BPD severity was classified by the NICHD/NHLBI/ORD workshop rating scale. Twenty-eight control subjects without BPD (non BPD group), 28 patients with mild BPD, 11 patients with moderate BPD, and sixpatients with severe BPD underwent echocardiograms with standard measurement such as ejection fraction by M-mode, tricuspidregurgitation pressure gradient, myocardial performance index (MPI) derived from pulse Doppler, and TDI measurements. BPD severity was classified by the NICHD/NHLBI/ORD workshop rating scale. Results: None of the standard echocardiographic findings was significantly different between the control group and BPDgroups. However, mean septal TDI-MPI of the severe BPD group (0.68 ± 0.06) was significantly (p < 0.01) higher than that of thenon-BPD (0.58 ± 0.10) or the mild BPD group (0.59 ± 0.12). In addition, mean RV TDI-MPI of the severe BPD group (0.71 ±0.13) was significantly (p < 0.05) higher than that of the non-BPD group (0.56 ± 0.08) or the mild BPD group (0.60 ± 0.125). Linear regression showed a good correlation between the severity of BPD and RV TDI-MPI (p = 0.01, R = 0.30) or septal TDIMPI(p = 0.04, R = 0.24). Conclusion: Echocardiographic evaluation of RV function based on an assessment of RV TDI-MPI can provide RV dysfunctionparameter in premature infants with BPD.

다문화 가정 신생아의 출생률과 주산기 위험 인자 및 예후 변화: 단일기관에서의 10년간 경험

장해인,최준식,송은송,최영륜 대한신생아학회 2013 Neonatal medicine Vol.20 No.1

Purpose: The aim of this study is to investigate the periodical changes of birth rate, perinatal risk factors and outcome of newborns in multi-cultural families with Korean male and non-Korean, Asian female in Gwangju, Chonnnam province. Methods: We enrolled newborns born in or transferred from other hospital to Chonnam National University Hospital between January 2001 and December 2010. They were categorized into two periods: data from 2001 to 2005 was defined as period I and from 2006 to 2010 was defined as period II. The clinical data of the birth rate, perinatal risk factors and outcome were retrospectively reviewed. Results: Number and rate of newborns in multicultural families increased significantly. The nationality of the mother changed: Vietnamese, Chinese and Cambodian significantly increased; and Japanese and Filipino significantly decreased. As the maternal age tended to be younger in period II, the age gap between the spouses increased, and the paternal age significantly increased, especially in age group of over 45 year-old. Among other perinatal risk factors, premature rupture of membranes and oligohydramnios were decreased. There were no statistical difference in morbidity (except hyperbilirubinemia) and mortality. However, congenital anomalies significantly increased. Conclusion: As the number of newborns in multicultural families has increased, the incidence of congenital anomalies also increased. We carefully presumed this result could be caused by increasing paternal age. Careful concern and management are needed for these families and further prospective studies are needed in a larger number of subjects. 목적: 한국인 남성과 아시아계 결혼이주자 여성과의 다문화 가정을 대상으로, 최근 10년간 빈도와 주산기 위험 인자 및예후에 어떤 변화가 있었는지 알아보고자 한 후향적 연구이다. 방법: 2001년 1월부터 2010년 12월까지 전남대학교병원에서 태어났거나 또는 외부병원에서 분만 후 이송되었던 다문화가정 산모와 신생아를 대상으로 하였다. 2001년부터 2005년까지를 I기, 2006년부터 2010년까지를 II기로 나누어,두 기간 사이에 빈도와 주산기 위험 인자 및 예후를 분석하였다. 결과: 다문화가정 신생아 수와 빈도는 의미있게 증가하였고, 결혼 이주자 산모 국적은 베트남, 중국, 캄보디아는 증가하고, 일본, 필리핀은 감소하였다. 엄마 연령이 더 어려지면서 배우자와의 나이 차가 커졌으며, 특히 45세 이상 고령아빠가 증가하였다. 다른 주산기 위험인자 중, 조기 양막파수와 양수과소증은 감소하였다. 다문화가정 신생아에서 황달 이외 다른 질환의 이환율에 변화는 없었고 사망률도 변화가 없었으나 선천 기형이 증가하였다. 결론: 다문화가정 신생아의 증가와 더불어 이들에서 선천 기형이 증가하는 추세인데, 이는 아빠 나이가 고령화되면서 초래되는 현상일 가능성이 추측되었다. 따라서 다문화 가정에 대한 각별한 관심과 관리가 필요하며, 앞으로 더 많은수를 대상으로 한 전향적 연구가 있어야 할 것으로 사료되었다.

미숙아에서 혈소판 감소증에 의해 발견된 장 혈관종 (Kasabach-Meritt 증후군) 1례

이영진,배설희,송은송,최수진나,김윤하,최영륜 대한신생아학회 2010 Neonatal medicine Vol.17 No.1

Kasabach-Merritt syndrome is a rare thrombocytopenic consumptive coagulopathy associated with a giant hemangioma. We experienced a case of unexplained ascites with thrombocytopenia in a 32 week premature infant. An exploratory laparotomy was performed to determine the cause of the refractory ascites and thrombocytopenia. An intestinal hemangioma was found, but, surgical removal was not performed due to the extensive involvement. Hemangioma was confirmed by SPECT (single-photon emission computed tomography) and the thrombocytopenia was treated with steroid therapy. It is recommended that hemangioma of the visceral organs should be suspected when unexplained thrombocytopenia and disseminated intravascular coagulopathy persist. Kasabach-Merritt 증후군은 거대한 혈관종에 의해 혈소판이 소모되어 감소되는 드문 질환이다. 저자들은 재태주령 32주 미숙아에서 설명되지 않은 복수와 혈소판 감소증의 원인을 찾기 위해 시험적 개복술을 시행하여 장 혈관종을 발견하였으나 광범위하여 수술적 제거를 하지 못하고, SPECT로 확인 후 스테로이드 치료로 혈소판 감소증이 호전된 1례를 경험하였다. 따라서 다른 원인에 의해 설명되지 않는 혈소판 감소증과 파종성 혈관내 응고병증이 있으면 내부 장기의 혈관종을 의심해 볼 필요가 있다고 사료되었다.

Costello syndrome: three sporadic cases

김지윤,김미정,송은송,조영국,최영륜,마재숙 대한소아청소년과학회 2007 Clinical and Experimental Pediatrics (CEP) Vol.50 No.10

Costello syndrome (CS) is a rare multiple congenital abnormality syndrome characterized by a typical coarse face, developmental delay, psychomotor and growth retardation, neurologic abnormalities, cardiac and cutaneous anomalies, severe feeding difficulties with postnatal growth failure, and increased risk of tumors. Since Costello first described it in 1971 and again in 1977, over 100 cases have been reported worldwide. It was recently shown that CS is a congenital condition caused by heterozygous de novo missense mutations affecting the codon for glycine 12 or 13 of the HRAS gene. We experienced three unrelated cases with coarse faces, developmental delays, short statures, macrocephaly, and redundant skin with deep palmar and plantar creases, hypertrophic cardiomyopathy and atrial tachycardia, which are characteristic of CS. Costello 증후군은 특징적 얼굴 형태(coarse face), 발달 장애, 지능 저하, 성장 지연, 신경학적 이상, 심근증, 피부 병변, 수유 장애, 상대적 대두증, 소관절의 과신전, 고형종 발생 증가 등을 특징으로 하는 드문 증후군으로 1977년 Costello에 의해 처음으로 보고되었으며, 이후 전세계적으로 약 100례 이상이 보고되었다. 최근 Costello 증후군은 HRAS 유전자 내의 glycine 12 또는 13 codon을 침범하는 de novo mutation에 의해 발생하는 것으로 알려져 있다. 저자들은 특징적인 얼굴 형태와 지능 저하, 대두증, 손과 발의 과도한 주름, 비후성 심근증과 심방성 빈맥으로 특징지어지는 Costello 증후군으로 진단된 환아 3례를 경험하였기에 이를 보고하는 바이다.