중증 호흡부전 신생아에서 사망의 조기예측 기준

박국인 ( Kook In Park ),남궁란 ( Ran Namgung ),이철 ( Chul Lee ),한동관 ( Dong Gwan Han ) 대한주산의학회 1990 Perinatology Vol.1 No.1

We conducted a retrospective study of early prediction of death based on arterial blood gas study and ventilatory indices in newborn infants with severe respiratory failure underwent conventional mechanical ventilation. Of 41 babies with severe respiratory failure admitted to a neonatal intensive care unit of Yonsei University Medical Center from Jan. 1986 to June 1989,7(17%) ultimately died. Thirty two of the 41 infants had hyaline membrane disease, 13 had pneumonia & / or sepsis, 13 had severe asphyxia, 4 had perisitent pulmonary hypertension, and 4 had meconium aspiration syndrome. There was a significant association between adverse outcome and severity of respirea-tory failure; patients with a single a/A PO2 ≤ 0.03 had mortality of 100%(4 of 4), those with a single OI≥ 60 had mortality 80%(4 of 5), those with a single PaO2 ≤ l8mmHg had mortality 100%(4 of 4), and those with a single Aa DO2 > 645mmHg had mortality 100%(6 of 6). An Aa DO2 criterion of greater than or equal to 640mmHg for 4 hours producecd a mortality of l00%(6 of 6) and an PaO2 < 50mmHg for a 28 hours interval was associated with a 83% mortality(5 of 6). Based on this study, we recommend that each NICU study its various patient groups to determine what quantitative indices, if any, allow reliable prediction of outcome and which specific index values allow discrimination between favorable and unfavorable outcomes. A predictive set of indices, once validated for a particular NICU, can then be used to indentify those infants who are likely to have an unfavorable outcome. Treatment options for those infants, whether conventional or experimental, can then be critically appraised.

수액 제한된 부당 및 적정 저출생체중아에서 생후 체중변화

윤신원 ( Shin Won Yoon ),남궁란 ( Ran Namgung ),이철 ( Chul Lee ) 대한주산의학회 2002 Perinatology Vol.13 No.4

연구목적:수액 제한요법을 시행 받았던 저출생체중아중 부당경량아(SGA)와 적정체중아(AGA)에서 초기 생리적 체중감소 양상을 비교하여 체내 수분변화의 차이를 살펴보고, 초기 생리적 체중감소에 영향 주는 요인을 비교 분석하며, 적정체중아에서 생후 첫 1주에 관찰되는 세 단계의 이뇨기가 부당 경량아에서도 나타나는지 조사하였다. 방법:1989년 1월부터 1996년 12월까지 7년동안 연세의대 세브란스병원 신생아집중치료실에 입원하여 기계적환기요법 및 수액제한요법을 시행 받은 51명의 저출생체중아중 부당 저출생체중아(SGA군, n=20)와 적정 저출생체중아(AGA군, n=31)로 분류하여 생후 첫 5일간 수액투여량, 생후 체중변화, 소변량, 혈청 전해질농도, 뇨 비중 및 이뇨시작(소변량 >3ml/kg/day)시기 등에 대한 차이를 환자의 병록지를 이용하여 후향적으로 비교 분석하였다. 결과:1) SGA군의 평균 재태주령은 30.4±1.6주(mean±SD), 평균 출생체중은 970±180g 이었고 AGA군의 평균 재태주령은 30±1.7주, 평균 출생체중은 1437±276g으로 출생체중이 SGA군에서 유의하게 적었다(p<0.01). 2) 체중감소율은 생후 첫 5일 동안 SGA군이 AGA군 보다 적었으며(p=0.01), 생후 5일째 평균 체중감소율은 SGA군 12.1±5.3%, AGA군 15.8±4.3%로 SGA군에서 유의하게 적었다(p<0.01). 3) 소변량은 생후 첫 5일 동안 SGA군에서 의미 있게 적었으며(p<0.05), 생후 2일과 3일째에 SGA가 AGA보다 유의하게 적었다(p<0.01). 이뇨시작은 SGA군에서 생후 5일째, AGA군에서는 생후 2일째부터 나타났다. 결론:수액 제한요법을 시행 받은 저출생체중아에서 SGA군은 AGA군과 비교할 때 생후 첫 5일간 체중감소율이 적었으며, 이는 SGA에서 AGA와 비교하여 소변량이 적고 이뇨시작이 늦기 때문으로 생각된다. 기계적환기요법을 요하는 저출생체중아의 수액요법시 SGA와 AGA사이의 수액균형의 차이가 고려되어야 할 것으로 사료된다. Objective: To determine the differences in water balance between preterm small for gestational age (SGA) and appropriate for gestational age (AGA) infants, we compared the difference in initial physiologic weight loss tiring the first five days of life and studied associated factors influencing the initial physiologic weight loss in preterm SGA and AGA infants. Methods: We retrospectively analyzed water and electrolyte balance in 51 preterm low birth weight infants of whom 20 were preterm SGA infants (gestational age, 30.4 ±1.6weeks; birth weight, 970± 1 80g) and 31 pretenu AGA infants (30 ± 1.7 weeks; 1437 ± 276g) matched by gestation. Results: The percent weight loss was significantly lower in SGA than in AGA infants (p=0.01) . The percent weight loss on the fifth day of life were 12.1 ±5.3%in SGA and 15.8 ±4.3%in AGA infants, respectively (p=<0.01). SGA infants had significantly lower urine output on the second and third day of life compared to AGA infants. Diuresis started on the second day of life in AGA infants and on the fifth day of life in SGA infants. Conclusion: The initial physiologic weight loss in SGA infants occurred in a smaller degree compared with AGA infants. This was associated with lower urine volume and the delay in onset of diuresis in SGA infants. These differences in water balance in SGA and AGA infants should be considered in the management of preterm low birth weight infants on mechanical ventilation.

Angiotensin 2 Receptor Blocker를 사용한 산모로부터 출생 후 급성 신부전증을 보인 신생아 1례

이철 ( Chul Lee ),박민수 ( Min Soo Park ),박국인 ( Kook In Park ),남궁란 ( Ran Namgung ),이순민 ( Soon Min Lee ),은호선 ( Ho Seon Eun ),라정진 ( Jeong Jin Ra ) 대한주산의학회 2012 Perinatology Vol.23 No.4

Hypertension is common medical problem encountered during pregnancy. However medication administered for maternal hypertension may cause fetal or neonatal complications. Angiotensin converting enzyme inhibitor or angiotensin II receptor blocker are rarely used during pregnancy, and there are few reports about the effect of them, because administration of these drugs during pregnancy may cause oligohydramnios, renal tubular dysplasia, hypocalvaria, pulmonary hypoplasia, intrauterine growth retardation, neonatal anuria and persistent ductus arteriosus. We report a case of neonatal acute renal failure by angiotensin II receptor blocker during pregnancy. In this case, the neonate with meconium aspiration was admitted to neonatal intensive care unit (NICU). During the NICU stay, neonatal anuria occurred, and there was a medical history that his mother took Candesartan Cilexeril (Atacand(R)), one of angiotensin II receptor blockers during pregnancy. The neonate showed intrinsic acute renal failure, so fluid was restricted and diuretics were administered to the neonate, and after 10 days, anuria improved.

신생아에서 연부 조직 종괴로 진단된 선천성 전신성 파종성 악성 횡문근종양 1례

박영아 ( Young A Park ),김정은 ( Jeong Eun Kim ),남궁란 ( Ran Namgung ),박민수 ( Min Soo Park ),박국인 ( Kook In Park ),이철 ( Chul Lee ) 대한주산의학회 2010 Perinatology Vol.21 No.3

Malignant rhabdoid tumor (MRT) was first described as a “rhabdomyosarcomatoid” variant of Wilms tumor, but was later identified as a distinct entity. MRTs are extremely rare and highly aggressive neoplasm with poor outcome, occurring mostly in kidneys and central nervous system, less frequently in extrarenal sites. MRTs are referred to as “congenital” when it is diagnosed at or immediately after birth. There are few reports about congenital disseminated MRT at birth. We report a case of congenital malignant rhabdoid tumor presenting as a soft tissue mass in the right arm at birth although the autopsy revealed the systemic disseminated tumors through the whole body with typical microscopic findings.

선천성 무섬유소원혈증

김희수 ( Hee Soo Kim ),박국인 ( Kook In Park ),남궁란 ( Ran Namgung ),이철 ( Chul Lee ),한동관 ( Dong Gwan Han ),김길영 ( Kir Young Kim ),김현숙 ( H. S. Kim ) 대한주산의학회 1992 Perinatology Vol.3 No.2

임상 ; 한국인 모유의 영양소와 영아의 성장과의 관계 분석 연구

이철 ( Chul Lee ),박민수 ( Min Soo Park ),박국인 ( Kook In Park ),남궁란 ( Ran Namgung ),이순민 ( Soon Min Lee ),민경복 ( Kyong Bok Min ),은호선 ( Ho Sun Eun ) 대한주산의학회 2012 Perinatology Vol.23 No.4

목적 : 본 연구는 건강한 수유부와 정상 만삭아를 대상으로 모유의 조성이 영아 성장에 미치는 영향을 확인하고자 하 였다. 방법 : 2011년 10월부터 2012년 3월까지 강남세브란스병원, 서울의 수유 센터 두 곳을 방문한 건강한 수유부와 영 아 173명을 대상으로 하였다. 수집된 모유는 Semisolid-state mid-infrared milk analyzer (MIRIS(R) Human Milk Analyzer, HMA, Miris AB, Uppsala, Sweden)를 이용하여 모유 1 mL를 1분간 분석기에 넣어 측정하였다. 결과 : 모유 성분의 분석을 통하여 섭취량/권장량(%)을 표준 체중 5 percentile 이상인 군과 5 percentile 미만인 군으로 나누어 비교한 결과, 열량은 130.2±34.1%, 68.2±31.7%, 단백질은 133.4±33.9%, 75.0±28.3%, 지방질은 189.6±94.7 %, 91.9±63.2%, 탄수화물은 120.7±17.6%, 72.3±26.5%으로 두 군간에 유의한 차이를 보였다(P<0.05). 표준 체중 25 percentile 이상인 군과 25 percentile 미만인 군으로 나누었을 때도 열량은 135.3±34.3%, 99.0±33.9% 이었으며, 단백 질은 136.7±35.0%, 108.0±33.9%, 지방질은 197.1±97.6, 141.6±79.8%, 탄수화물은 125.1±16.5%, 95.0±22.0%으로 두 군간에 유의한 차이를 보였다(P<0.05). Multiple logistic regression을 이용하여 분석한 결과 표준 체중이 5% 미만인 군에 영향을 미치는 인자는 탄수화물이 가장 유의한 인자이었고(P<0.05), 25% 미만인 군에 영향을 미치는 인자는 단백 질과 탄수화물이었다(P<0.05). 결론 : 본 연구에서는 영아의 체중이 작은 경우 모유 성분의 영양소가 유의하게 낮음을 확인하였다. 이를 통해 출생 후 체중 증가가 유의하게 적고, 성장 곡선상 낮은 체중 분포를 보일 경우 적극적인 모유수유와 더불어 수유부의 식습관 개 선을 통한 적정한 영양공급 및 나아가 영양 강화를 이루어 신생아의 성장 증가를 도모해야 하겠다. Purpose : The aim of this study was to determine the effect of the macronutrient composition of breast milk from Korean women on the growth of infants. Methods : 173 healthy lactating women and breast-fed infants who visited Gangnam Severance Hospital and two breast-feeding centers in Seoul from October 2011 to March 2012 were recruited. We checked the birth weight and body weight of infants while collecting breast milk from the mothers, and analyzed the macronutrient component of breast milk with a mid-infrared milk analyzer (MIRIS(R) Human Milk Analyzer, HMA, Miris AB, Uppsala, Sweden). Group analysis was performed depending on more or less than 5 percentile and 25 percentile of body weight. Results : The amount of daily intake/RDA for calories, proteins, lipids and carbohydrates of breast milk were significantly lower in the less than 5 percentile and 25 percentile group (P <0.05). Using multiple logistic regression analysis, the significant nutrient component that was insufficient in the less than 5 percentile and less than 25 percentile of body weight group respectively was carbohydrates (P <0.05). Conclusion : We conclude that each macronutrient level of breast milk is statistically low in infants with less body weight. These results suggest that nutritional consideration of breast milk is necessary for the growth of breast-fed infants.

신생아기에 진단된 미토콘드리아 호흡 사슬 결함 1례

최경민 ( Kyoung Min Choi ),권해식 ( Hae Sik Kweon ),이동우 ( Dong Woo Lee ),남궁란 ( Ran Namgung ),박민수 ( Min Soo Park ),이철 ( Chul Lee ),이영목 ( Young Mok Lee ),이진성 ( Jin Sung Lee ) 대한주산의학회 2003 Perinatology Vol.14 No.1

미토콘드리아 대사질환은 지방산 산화장애, 피루브산염 대사장애 및 호흡사슬 결함으로 분류되는데, 호흡 사슬 결함의 신생아기 임상증상은 반복적인 무호흡, 경련이 동반된 혼수, 선천성 젖산혈증, 긴장저하, 간기능부전, 비후형 심근증 등이며, 진단은 세포질과 미토콘드리아의 산화환원 상태를 반영하는 지표인 혈장의 젖산, 피루브산염, 케톤체의 측정과 그 molar ratios로 한다. 본 저자등은 신생아에서 심한 대사성 산증과 동반되어 혼수, 호흡부전, 간기능장애, 신부전증 등 다발성 장기 기능 부전소견을 보이며, 젖산 산혈증(>2.5mM), 증가된 젖산/피루브산염 비(>20), 케톤뇨 등이 확인되어 선천성 대사이상 중 미토콘드리아 호흡사슬 결함으로 진단된 1례를 보고한다. Mitochondrial diseases are classified into the three major categories, defects of fatly acid oxidation, defects of pyruvate metabolism, and defects of the respiratory chain, and all of these cause severe neuron-logic dysfunction in the newborn period. Defects of the mitochondrial respiratory chain present as recurrent apnea, seizures, congenital lactic acidosis, hypotonia, hepatic dysfunction and hypertrophic cardiomyopathy in the neonatal period. Laboratory findings of hyperlactataemia (>2.5mM), elevated lactate/ pyruvate (L/P) ratio (>20) and ketone body ratio (>2) suggest the diagnosis of mitochondrial respiratory chain defects. We report a case of mitochondrial respiratory chain defect diagnosed in the neonatal period presenting with multiorgan failure consisting of severe metabolic acidosis, comatous mental state, respiratory distress, hepatic dysfunction, renal failure with lactic acidosis (24mM), increased L/P ratios (55.6) and ketonuria (increased ratio of 3-hydroxybutyrate/acetoacetate).

정상 신생아의 선천성 비루관폐쇄 발생빈도에 관한 보고

이상렬(Sang Yeul Lee),정학성(Hak Sung Chung),김홍복(Hong Bok Kim),남궁란(Ran Namgung),한동관(Dong Gwan Han) 대한안과학회 1989 대한안과학회지 Vol.30 No.1

부당경량아에서 면역글로부린 G-아형의 농도 및 태반통과에 관한 연구

박국인 ( Kook In Park ),김동수 ( Dong Soo Kim ),남궁란 ( Ran Namgung ),이철 ( Chul Lee ),한동관 ( Dong Gwan Han ),강명서 ( Myung Seo Kang ),권오헌 ( Oh Hun Kwon ) 대한주산의학회 1991 大韓周産醫學會雜誌 Vol.2 No.2

산전스테로이드 투여가 수액제한 요법을 시행받은 극저출생체중아의 수액 균형 및 예후에 미치는 영향

김정년 ( Jeong Nyun Kim ),이철 ( Chul Lee ),박민수 ( Min Soo Park ),남궁란 ( Ran Namgung ),박국인 ( Kook In Park ),한동관 ( Dong Gwan Han ) 대한주산의학회 1998 大韓周産醫學會雜誌 Vol.9 No.2