2020 Korean Guidelines for Cardiopulmonary Resuscitation. Part 8. Neonatal Resuscitation

허주선,김수영,박혜원,최용성,박찬욱,조금준,오아영,장은경,김한석,김애란,황성오 대한응급의학회 2021 Clinical and Experimental Emergency Medicine Vol.8 No.S

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제 8장 신생아 소생술

허주선,김수영,박혜원,최용성,박찬욱,조금준,오아영,장은경,김한석,김애란,황성오 대한응급의학회 2021 대한응급의학회지 Vol.32 No.3-1

쌍생아간 수혈 증후군에서의 신생아기 사망률 및 이환율

허주선,김규리,김윤주,신승한,이재명,이주영,손진아,이진아,최창원,김이경,김한석,김병일,최중환 대한신생아학회 2012 Neonatal medicine Vol.19 No.1

Purpose: This study was aimed to evaluate the neonatal mortality and morbidity of infants affected by twin-twin transfusion syndrome (TTTS) compared to the control twins matched for gestational age. Also the perinatal outcomes of donor parts of TTTS twins with their counterpart recipients were compared. Methods: A retrospective case-control study was conducted from infants born at Seoul National University Children’s Hospital and Seoul National University Bundang Hospital between April 2005 and July 2011. Eighteen pairs of TTTS infants were allocated to the TTTS group. The control group consisted of 36 pairs of twin infants unaffected by TTTS who were matched for gestational age. Neonatal deaths and morbidities were recorded. Results: The mortality in TTTS group was significantly higher than control group (27.8% vs. 4.2%, P=0.001). The incidence of acute renal failure (41.2% vs. 9.7%, P<0.001), cardiac ventricular hypertrophy (31.3% vs. 2.9%, P<0.001), congestive heart failure (45.7%vs. 5.6%, P<0.001), grade ≥2 intraventricular hemorrhage (33.3% vs. 11.1%, P=0.012) and grade ≥2 periventricular leukomalacia (24.2% vs. 2.8%, P=0.001) were significantly higher in TTTS group than control group. There was no significant difference in mortality and morbidities between donors and recipients except significantly higher incidence of acute renal failure in donors (70.6%vs. 11.8%, P=0.001). Conclusion: Twin infants affected by TTTS have higher risk of neonatal death and several severe morbidities. These results indicate that alert monitoring and checking about possible morbidities are very important in newborns with TTTS and early intervention is critical for improving the overall outcome of the affected infants. 목적: 분만 후 생존한 쌍생아간 수혈 증후군 환자군과 주수를 짝지은 대조군을 비교함으로써 쌍생아 수혈 증후군의 신생아기 사망률 및 이환율을 분석하고 쌍생아간 수혈 증후군 내의 공혈자와 수혈자 간에도 사망률과 이환률을 비교하여 이를 토대로 쌍생아 수혈 증후군 환자의 진료에 도움이 되고자 한다. 방법: 2005년 4월부터 2011년 7월까지 서울대병원 어린이병원과분당 서울대병원에 입원한 환아를 대상으로 환자-대조군 연구를 시행하였다. 총 18쌍의 쌍생아간 수혈 증후군 환자와 36쌍의 주수를짝지은 쌍생아 대조군을 대상으로 후향적으로 신생아기 사망, 합병증에 대해 의무 기록을 고찰하였다. 결과: 쌍생아 간 출생 체중, 혈색소 차이는 쌍생아간 수혈 증후군에서 대조군에 비해 유의하게 높았다(29.9% vs. 10.1%, P<0.001; 0.2g/dL vs. -0.3 g/dL, P=0.024). 신생아기 사망률은 쌍생아간 수혈 증후군에서 유의하게 높았다(27.8% vs. 4.2%; P=0.001). 두 군간 신생아기 이환율을 비교하였을 때 쌍생아간 수혈 증후군에서 유의하게 높게 나타난 것은 급성 신부전(41.2% vs. 9.7%, P<0.001), 중등도 이상의 삼첨판 역류(12.5% vs. 1.4%, P=0.033), 심실 비대 (31.3% vs. 2.9%, P<0.001), 울혈성 심부전(45.6% vs. 5.6%, P<0.001), 2단계 이상의뇌실내 출혈(45.7% vs. 5.6%, P<0.001), 2단계 이상의 뇌실주위 백질연화증(24.2% vs. 2.8%, P=0.001)이었다. 쌍생아간 수혈 증후군 내의공혈자와 수혈자 간에 신생아기 사망률에는 유의한 차이가 없었으며, 두 군 간에 통계학적으로 유의한 차이가 있었던 합병증은 급성신부전(70.6% vs. 11.8%; P=0.001)이었고 그 외 심부전, 뇌실내 출혈,뇌실주위 백질 연화증을 포함한 다른 합병증에는 차이가 없었다. 결론: 쌍생아간 수혈 증후군 환아는 그렇지 않은 쌍생아에 비해신생아기 사망률 및 몇 가지 중대한 신생아기 질병 이환율이 유의하게 높았다. 따라서 출생 후 환아의 혈역학적 변화 및 발병 가능한 합병증에 대해 집중적으로 감시, 관찰하는 것이 매우 중요하며 발병시조기에 치료하는 것이 환아의 전체적인 예후에 큰 영향을 미칠 것이다.

극소저체중출생아에서 신생아 괴사성 장염의 침범 범위에 따른 예후 및 예측 인자

허주선,정영화,이주영,신승한,김이경,김한석,최중환 대한신생아학회 2015 Neonatal medicine Vol.22 No.2

Purpose: This study aimed to evaluate the prognosis of necrotizing enterocolitis (NEC) according to the extent of involvement, among very low birth weight infants. Furthermore, the predictive factors for extent of involvement were evaluated. Methods: Medical records of all newborns with surgically treated NEC admitted to the neonatal intensive care unit of Seoul National University Children’s Hospital between 2005 and 2013 were reviewed. Infants were grouped according to the extent of involvement of NEC: isolated segment involvement (ISI, n=31) and multi-segment involvement (MSI, n=17). We evaluated the clinical characteristics, outcomes, and pre-operative factors according to symptoms, laboratory and radiologic findings. Results: The incidence of small for gestational age was significantly higher in the MSI than ISI group (12.9% vs. 41.2%, P=0.036). The length of resected bowel was significantly longer (1.7 cm vs. 8 cm, P=0.010), and the incidence of short bowel syndrome (SBS) (0% vs. 23.1%, P=0.023) and mortality (3.2% vs. 23.5%, P=0.047) were significantly higher in the MSI than ISI group. However, there was no significant difference between the two groups in terms of high-output stoma, time of full enteral feeding, extrauterine growth retardation, changes of z-score of body weight between admission and discharge and reoperation. Portal vein gas detected by ultrasonography was the only statistically significant predictive factor of extent of involvement (odds ratio= 13.237, P=0.029). Conclusion: SBS and mortality were higher in MSI NEC compared to ISI NEC. However, there was no difference in the time of full enteral feeding and growth between the two groups. Portal vein gas detected by ultrasonography maybe a predictive factor of extent of NEC.

Atypical Neonatal Marfan Syndrome with p.Glu1073Lys Mutation of FBN1: the First Case in Korea

허주선,송주영,최은영,김은희,김지희,박소은,전지현 대한의학회 2017 Journal of Korean medical science Vol.32 No.1

Neonatal Marfan syndrome (nMFS) is considered to be on the most severe end of the spectrum of type I fibrillinopathies. The common features of nMFS include ascending aortic dilatation, severe mitral and/or tricuspid valve insufficiency, ectopia lentis, arachnodactyly, joint contractures, crumpled ear, loose skin, and pulmonary emphysema.We describe a newborn male diagnosed with nMFS. He presented several atypical features, such as diaphragmatic eventration, severe hydronephrosis with hydroureter, and dilated cisterna magna. Molecular analysis revealed a missense mutation at nucleotide 3217 (c.3217G>A) in exon 26 of the fibrillin-1 (FBN1) gene, resulting in the substitution of a glutamate for a lysine at codon 1073 (E1073K) in the 12th calcium binding epidermal growth factor-like domain of the FBN1 protein. Here we report a rare case of Nmfs with several combined atypical features, such as diaphragmatic eventration, severe hydronephrosis with hydroureter, and dilated cisterna magna. Our report is the first atypical nMFS case with p.Glu1073Lys mutation of FBN1 in Korea and may help clinicians with the diagnosis and follow-up of atypical nMFS.

A case of mucolipidosis II presenting with prenatal skeletal dysplasia and severe secondary hyperparathyroidism at birth

허주선,Ka Young Choi,Se Hyoung Sohn,Curie Kim,Yoon Joo Kim,Seung Han Shin,Jae Myung Lee,이주영,Jin A Sohn,Byung Chan Lim,이진아,최창원,Ee Kyung Kim,Han Suk Kim,Beyong Il Kim,최중환 대한소아청소년과학회 2012 Clinical and Experimental Pediatrics (CEP) Vol.55 No.11

Mucolipidosis II (ML II) or inclusion cell disease (I-cell disease) is a rarely occurring autosomal recessive lysosomal enzyme-targeting disease. This disease is usually found to occur in individuals aged between 6and 12 months, with a clinical phenotype resembling that of Hurler syndrome and radiological findings resembling those of dysostosis multiplex. However, we encountered a rare case of an infant with ML II who presented with prenatal skeletal dysplasia and typical clinical features of severe secondary hyperparathyroidism at birth. A female infant was born at 37+1 weeks of gestation with a birth weight of 1,690 g (<3rd percentile). Prenatal ultrasonographic findings revealed intrauterine growth retardation and skeletal dysplasia. At birth, the patient had characteristic features of ML II, and skeletal radiographs revealed dysostosis multiplex, similar to rickets. In addition, the patient had high levels of alkaline phosphatase and parathyroid hormone,consistent with severe secondary neonatal hyperparathyroidism. The activities of β-D-hexosaminidase and α-N-acetylglucosaminidase were moderately decreased in the leukocytes but were 5- to 10-fold higher in the plasma. Examination of a placental biopsy specimen showed foamy vacuolar changes in trophoblasts and syncytiotrophoblasts. The diagnosis of ML II was confirmed via GNPTAB genetic testing, which revealed compound heterozygosity of c.3091C>T (p.Arg1031X) and c.3456_3459dupCAAC (p.Ile1154GlnfsX3), the latter being a novel mutation. The infant was treated with vitamin D supplements but expired because of asphyxia at the age of 2 months.

A case of Bartter syndrome type I with atypical presentations

이은혜,허주선,이현경,한경희,강희경,하일수,최용,정해일 대한소아청소년과학회 2010 Clinical and Experimental Pediatrics (CEP) Vol.53 No.8

Bartter syndrome (BS) is an autosomal recessively inherited rare renal tubular disorder characterized by hypokalemic metabolic alkalosis and hyperreninemic hyperaldosteronism with normal to low blood pressure due to a renal loss of sodium. Genetically, BS is classified into 5 subtypes according to the underlying genetic defects, and BS is clinically categorized into antenatal BS and classical BS according to onset age. BS type I is caused by loss-of-function mutations in the SLC12A1 gene and usually manifests as antenatal BS. This report concerns a male patient with compound heterozygous missense mutations on SLC12A1 (p.C436Y and p.L560P) and atypical clinical and laboratory features. The patient had low urinary sodium and chloride levels without definite metabolic alkalosis until the age of 32 months, which led to confusion between BS and nephrogenic diabetes insipidus (NDI). In addition, the clinical onset of the patient was far beyond the neonatal period. Genetic study eventually led to the diagnosis of BS type I. The low urinary sodium and chloride concentrations may be caused by secondary NDI, and the later onset may suggest the existence of a genotype-phenotype correlation. In summary, BS type I may have phenotype variability including low urine sodium and chloride levels and later onset. A definitive diagnosis can be confirmed by genetic testing.

이어웰 귀교정기로 치료한 귓바퀴 기형을 가진 신생아 3례

김지희,신윤호,김기은,허주선,전지현,정성훈 대한신생아학회 2017 Neonatal medicine Vol.24 No.3

Auricular deformities occur frequently in newborn infants. Typically, most pediatricians explain to parents that these deformities will get better as child grows older. But, only about 30% of auricular deformities are known to be self-correcting, and there is no reliable model to predict them. If ear molding is initiated during the first days of life with the EarWell System, successful treatment could be possible without pain in a non-surgical way. We present 3 cases of auricular deformities treated with the EarWell System. 2 infants were born with auricular deformities at Gangnam Cha Medical Center and 1 infant visited the outpatient clinic for the treatment of auricular deformities. 5 ears in 3 infants underwent ear molding using the EarWell System. They had it placed on the 20th day after birth. Average treatment time was 18.7 days, and all of them were corrected. Complications were redness, oozing, erosion and mild pressure ulcerations. Early recognition and treatment of the auricular deformity ensure the great prospect of success. Also, it is important for both the parents and the pediatricians to know that auricular deformities could be successfully treated with Earwell System

Epidemiology of Catheter-related Bloodstream Infections in Neonatal Intensive Care Units: A Rapid Systematic Literature Review

에르데네투야 볼로르마,강초록,최영준,허주선,조한나 대한의료관련감염관리학회 2023 의료관련감염관리 Vol.28 No.1

Background: Catheter-related bloodstream infections (CRBSIs) are serious complications in neonatal intensive care units (NICUs). We aimed to assess the incidence of CRBSIs in NICUs worldwide and describe the causative organisms. Methods: We searched PubMed, EMBASE, Cochrane, and KoreaMed databases. We included studies on CRBSIs in NICU settings with data on bacteremia. We performed a random-effects meta-analysis on CRBSI incidence in NICUs, stratified the data according to WHO regions. We compiled data on underlying organisms. Results: Of the 692 studies identified, 71 published between 2011 and 2022 were considered eligible. The pooled incidence of CRBSI per 1000 catheter days in NICUs was 8.66 (95% confidence interval [CI], 7.19; 10.12). Stratifying by WHO regions, the CRBSI incidence per 1000 catheter days was 10.38 (95% CI, 3.86; 16.90) in the Eastern Mediterranean Region (EMR), 11.77 (95% CI, 9.20; 14.35) in the European Union Region (EUR), 5.94 (95% CI, 3.87; 8.00) in the Western Pacific Region (WPR), and 6.71 (95% CI, 4.39; 9.03) in the Region from the Americas (AMR). Of the 2887 bacterial strains, 73.4% (n=2118) were gram-positive bacteria, 18.9% (n=547) were gram-negative bacteria, and 7.8% (n=225) were fungi. Coagulasenegative Staphylococci (n=1380, 65.2%) were the most common pathogen among the grampositive types, followed by Staphylococcus aureus (n=318, 15%). Among the CRBSI gramnegative cultures, Klebsiella spp. (n=201, 36.7%) was the primary pathogen. Conclusion: We found a substantial burden of CRBSIs in NICUs across the globe. Our findings highlight the need to improve the implementation of global and local strategies to reduce CRBSIs in NICUs.

COVID-19 and vaccination during pregnancy: a systematic analysis using Korea National Health Insurance claims data

안기훈,김혜인,이광식,허주선,김호연,조금준,홍순철,오민정,김해중 대한산부인과학회 2022 Obstetrics & Gynecology Science Vol.65 No.6

ObjectiveThis study systematically analyzed coronavirus disease 2019 (COVID-19) and vaccination details during pregnancy byusing the national health insurance claims data. MethodsPopulation-based retrospective cohort data of 12,399,065 women aged 15-49 years were obtained from the KoreaNational Health Insurance Service claims database between 2019 and 2021. Univariate analysis was performed tocompare the obstetric outcomes of pregnant women (ICD-10 O00-O94) and their newborns (ICD-10 P00-P96) with andwithout COVID-19. Univariate analysis was also performed to compare the age and obstetric outcomes of pregnantwomen receiving different types of vaccines. ResultsThe percentage of pregnant women with COVID-19 during pregnancy was 0.11%. Some obstetric outcomes of pregnantwomen with COVID-19, including the rates of preterm birth or cesarean delivery, were significantly better than thoseof pregnant women without COVID-19. The rate of miscarriage was higher in pregnant women with COVID-19 thanwithout COVID-19. However, the outcomes of newborns of women with and without COVID-19 were not significantlydifferent. Regarding vaccination type, obstetric outcomes of pregnant women appeared to be worse with the viralvector vaccine than with the mRNA vaccine. ConclusionTo the best of our knowledge, this is the first study to systematically analyze COVID-19 and vaccination details duringpregnancy using the national health insurance claims data in Korea. The obstetric outcomes in pregnant women withand without COVID-19 and their newborns were similar.