Association of Increased Pulmonary Interleukin-6 with the Priming Effect of Intra-Amniotic Lipopolysaccharide on Hyperoxic Lung Injury in a Rat Model of Bronchopulmonary Dysplasia

Kim, Do-Hyun,Choi, Chang Won,Kim, Ee-Kyung,Kim, Han-Suk,Kim, Beyong Il,Choi, Jung-Hwan,Lee, Myong Jin,Yang, Eun Gyeong S. Karger AG 2010 NEONATOLOGY Vol.98 No.1

<P><I>Background:</I> The authors previously demonstrated the priming effect of intra-amniotic lipopolysaccharide (LPS) on hyperoxic lung injury in a rat model of bronchopulmonary dysplasia (BPD). <I>Objectives:</I> To investigate the mechanism underlying this priming effect by determining biochemical profiles in a rat model of BPD. <I>Methods:</I> The rat model involved intra-amniotic LPS administration and postnatal hyperoxia (85%). The mRNA expressions of <I>interleukin-6 (IL-6), vascular endothelial growth factor (VEGF), VEGF receptor-2 (VEGFR-2), basic fibroblast growth factor (bFGF),</I> and <I>transforming growth factor </I>β<SUB><I>1</I></SUB><I> (TGF-</I>β<SUB><I>1</I></SUB><I>),</I> as well as the protein levels of IL-6, VEGF, and protein carbonyl in lung tissue were compared between the LPS plus hyperoxia, the LPS only, the hyperoxia only, and the control groups. <I>Results:</I> Morphometric analysis of lung tissues demonstrated that alveolarization was significantly inhibited only in the LPS plus hyperoxia group. IL-6 protein levels and its mRNA expression in the lungs were significantly increased only in the LPS plus hyperoxia group. Neither LPS nor hyperoxia increased IL-6 in the lungs independently. <I>bFGF</I> mRNA expression was significantly decreased in the LPS-treated groups. VEGF protein levels were significantly reduced by hyperoxia, whereas protein carbonyl levels were increased by intra-amniotic LPS or hyperoxia. No additional significant change to VEGF or protein carbonyl levels was produced by intra-amniotic LPS or hyperoxia. There were no significant differences in the mRNA expressions of <I>VEGF, VEGFR-2,</I> and <I>TGF-</I>β<SUB><I>1</I></SUB>. <I>Conclusions:</I> The priming effect of intra-amniotic LPS on hyperoxic lung injury may be associated with IL-6 elevation in the lungs.</P><P>Copyright © 2009 S. Karger AG, Basel</P>

Neonatal Morbidities Associated with Histologic Chorioamnionitis Defined Based on the Site and Extent of Inflammation in Very Low Birth Weight Infants

Kim, Su Yeong,Choi, Chang Won,Jung, Euiseok,Lee, Juyoung,Lee, Jin A,Kim, Haeryoung,Kim, Ee-Kyung,Kim, Han-Suk,Kim, Beyong Il,Choi, Jung-Hwan The Korean Academy of Medical Sciences 2015 JOURNAL OF KOREAN MEDICAL SCIENCE Vol.30 No.10

Misdiagnosis of fetus-in-fetu as meconium peritonitis

Kim, Yoon-Joo,Sohn, Se-Hyung,Lee, Ju-Young,Sohn, Jin-A,Lee, Eun-Hee,Kim, Ee-Kyung,Choi, Chang-Won,Kim, Han-Suk,Kim, Beyong-Il,Choi, Jung-Hwan The Korean Pediatric Society 2011 Clinical and Experimental Pediatrics (CEP) Vol.54 No.3

Fetus-in-fetu (FIF) is a rare congenital condition in which a fetiform mass is detected in the host abdomen and also in other sites such as the intracranium, thorax, head, and neck. This condition has been rarely reported in the literature. Herein, we report the case of a fetus presenting with abdominal cystic mass and ascites and prenatally diagnosed as meconium pseudocyst. Explorative laparotomy revealed an irregular fetiform mass in the retroperitoneum within a fluid-filled cyst. The mass contained intestinal tract, liver, pancreas, and finger. Fetal abdominal cystic mass has been identified in a broad spectrum of diseases. However, as in our case, FIF is often overlooked during differential diagnosis. FIF should also be differentiated from other conditions associated with fetal abdominal masses.

Effects of Acute Hyperoxia on Changes of Hyaluronic Acid and Histopathologic Morphology of Lung in Neonatal Rats

Kim, Beyong Il,Choi, Jung-Hwan,Yun, Chong Ku,Chi, Je G. Seoul National University 1994 The seoul journal of medicine Vol.35 No.2

Misdiagnosis of fetus-in-fetu as meconium peritonitis

Yoon Joo Kim,최중환,이주영,Jin A Sohn,Eun Hee Lee,Ee Kyung Kim,Chang Won Choi,김한석,Beyong Il Kim 대한소아청소년과학회 2011 Clinical and Experimental Pediatrics (CEP) Vol.54 No.3

Fetus-in-fetu (FIF) is a rare congenital condition in which a fetiform mass is detected in the host abdomen and also in other sites such as the intracranium, thorax, head, and neck. This condition has been rarely reported in the literature. Herein, we report the case of a fetus presenting with abdominal cystic mass and ascites and prenatally diagnosed as meconium pseudocyst. Explorative laparotomy revealed an irregular fetiform mass in the retroperitoneum within a fluid-filled cyst. The mass contained intestinal tract, liver, pancreas, and finger. Fetal abdominal cystic mass has been identified in a broad spectrum of diseases. However, as in our case, FIF is often overlooked during differential diagnosis. FIF should also be differentiated from other conditions associated with fetal abdominal masses.

A case of McKusick-Kaufman syndrome

Son, Se-Hyung,Kim, Yoon-Joo,Kim, Eun-Sun,Kim, Ee-Kyung,Kim, Han-Suk,Kim, Beyong-Il,Choi, Jung-Hwan The Korean Pediatric Society 2011 Clinical and Experimental Pediatrics (CEP) Vol.54 No.5

McKusick-Kaufman syndrome (MKS) is an autosomal recessive multiple malformation syndrome characterized by hydrometrocolpos (HMC) and postaxial polydactyly (PAP). We report a case of a female child with MKS who was transferred to the neonatal intensive care unit of Seoul National University Children's Hospital on her 15th day of life for further evaluation and management of an abdominal cystic mass. She underwent abdominal sonography, magnetic resonance imaging, genitography and cystoscopy which confirmed HMC with a transverse vaginal septum. X-rays of the hand and foot showed bony fusion of the left third and fourth metacarpal bones, right fourth dysplastic metacarpal bone and phalanx, right PAP and hypoplastic left foot with left fourth and fifth dysplastic metatarsal bones. In addition, she had soft palate cleft, mild hydronephroses of both kidneys, hypoplastic right kidney with ectopic location and mild rotation, uterine didelphys with transverse vaginal septum and low-type imperforated anus. She was temporarily treated with ultrasound-guided transurethral aspiration of the HMC. Our patient with HMC and PAP was diagnosed with MKS because she has two typical abnormality of MKS and she has no definite complications of retinal disease, learning disability, obesity and renal failure that develop in Bardet-Biedl syndrome, but not in MKS until 33 months of age. Here, we describe a case of a Korean patient with MKS.

미숙아에서 모세 혈관종의 임상양상

김선녀 ( Seon Nyo Kim ),이주영 ( Juyoung Lee ),신승한 ( Seung Han Shin ),최창원 ( Chang Won Choi ),김이경 ( Ee Kyung Kim ),김한석 ( Han Suk Kim ),김병일 ( Beyong Il Kim ),최중환 ( Jung Hwan Choi ) 대한주산의학회 2015 Perinatology Vol.26 No.3

Purpose: Capillary hemangiomas occur more frequently in preterm infants. We aimed to describe the clinical course of capillary hemangiomas in preterm infants. Methods: The records of preterm infants with a gestational age (GA) of <35 weeks who were admitted to two tertiary neonatal intensive care units from January 2004 to December 2013 and had capillary hemangiomas were reviewed retrospectively. Subgroup analysis of between infants of GA <30 weeks and GA 30-34+6 weeks were done and ad hoc analysis comparing study population and matched preterm infants without hemangioma for investigation of differences in clinical characteristics. Results: Of the 2,772 preterm infants, 112 (4%) infants developed capillary hemangiomas. The majority (91.9 %) of them had a solitary hemangiomas with the trunk was the most commonly involved site (43%). Three quarters of the patients were treated with topical corticosteroid, propranolol or laser treatment. When we divided this population as who were born before or after GA 30 weeks, there was no difference at postmenstrual age (PMA) of onset of capillary hemangiomas (median [IQR], 36+4 [30+5-40+5] vs. 36+2 [33+6-41+1] weeks, P= 0.275). The age at involution of capillary hemangiomas was also not differ between two groups (median [IQR], 7.75 [3.75-12.25] vs. 7.5 [4-13.75] months, P=0.425). There were no statistical differences between preterm infants with capillary hemangiomas and their age, weight and sex matched control preterm infants without hemangiomas in the neonatal and maternal factors. Conclusion: The development of capillary hemangiomas occurred at approximately 36 to 37weeks of PMA regardless of prematurity in preterm infants. Capillary hemangiomas of preterm infants resolved spontaneously and disappear completely by around 7 months of corrected age.

임상 ; 식이부전으로 소장 조영술을 시행 받은 조산아 46례의 임상적 고찰

손세형 ( Se Hyung Son ),김윤주 ( Yoon Joo Kim ),이주영 ( Ju Young Lee ),이은희 ( Eun Hee Lee ),손진아 ( Jin A Sohn ),김은선 ( Eun Sun Kim ),김이경 ( Ee Kyung Kim ),김한석 ( Han Suk Kim ),김병일 ( Beyong Il Kim ),최중환 ( Jung Hwan 대한주산의학회 2010 Perinatology Vol.21 No.2

목적: 조산아에서 식이 진행이 잘 되지 않는 경우에 소장조영술 검사를 시행해 볼 수 있다. 그러나 현재까지 조산아에서 소장조영술의 안정성과 유효성 평가가 확립되어 있지 않다. 본 연구를 통해 소장조영술의 최근 경험을 조사하여 조산아에서 식이진행이 되지 않을 경우에 소장조영술 검사에 대해 기초자료를 제시하고자 한다. 방법: 2005년 1월부터 2008년 10월까지 서울대학교 병원 어린이병원 신생아 중환자실에 입원하여 소장조영술을 시행한 46명의 조산아들을 연구대상으로 하여 의무기록을 후향적으로 분석하였다. 결과: 평균 재태기간과 평균출생체중은 각각 30(+1)±3(+5)주(24-36주), 1,435±623g (460-2,810g)이었다. 소장조영술을 시행한 평균 수태후 연령은 37(+2)±4(+2)주(중간값 36(+2)주, 27(+2)-45(+4)주)였고 이는 생후 46.8±27.3일(5-126일)에 시행되었다. 소장조영술 검사를 시행한 결과에서 기계적 장폐색이 없었던 경우는 29명(63%)이었고 이들 중 22명(47.8%)은 위장관 이행 시간이 연장되어 있었다. 그러나 이들은 결국 완전 장관 영양에 도달하였고 도달하는 데 걸린 시간은 평균 11.2일이었다(중간값 6일). 소장조영술을 시행한 신생아들을 출생체중에 따라 1.5kg 미만 군과 1.5kg 이상 군으로 분류하였을 때 각각 27명, 19명이었다. 1.5 kg 이상 군에서는 검사를 더 일찍 시행하였다(P=0.027). 1.5kg 미만 군에서는 27명 중 15명(55.6%)에서 인공호흡기 치료를 받거나 인큐베이터 내에서 치료 중으로 침상 옆에서 검사를 시행하였다. 1.5kg 미만 군에서는 신생아괴사성장염, 태변연관 장폐색 및 위장관 수술 과거력이 1.5kg 이상 군보다 유의하게 많았다(P=0.044). 소장조영술 시행 시 사용한 조영제는 바륨, 텔레브릭스, 가스트로그라핀이었고 조영제 사용 후 관련된 합병증으로 구토 및 조영제 정체가 있었으나 대부분 호전되었고 흡인이나 패혈증 등의 부작용은 없었다. 결론: 식이 부전이 있는 조산아에서 적극적인 소장조영술 검사는 식이부전의 원인을 밝히고 이를 해결하는 데에 도움이 되므로 식이를 진행하는 데에 도움이 될 것이다. Objective: Small bowel series (SBS) may be a useful test in identifying the causes of feeding intolerance in preterm infants. Unfortunately, little is known about the safety and effectiveness of SBS in preterm infants. By scrutinizing our recent experiences in performing SBS in preterm infants, we aim to provide basic data concerning the use of SBS in preterm infants with feeding intolerance. Methods: Between January 2005 and October 2008, SBS was done in 46 preterm infants with feeding intolerance, who were admitted to the neonatal intensive care unit (NICU) of Seoul national university children`s hospital. We conducted a retrospective analysis of these preterm infants. Results: The average gestational age and birth weight of the 46 infants were, 30(+1)±3(+5) weeks, 1,435±623 g, respectively. The mean postconceptional age when SBS was done, was 37(+2)±4(+2) weeks, 46.8±27.3days. SBS proved that 29 infants did not have mechanical obstructions and 22 of these infants had delayed bowel transit time. Eventually, 35 infants attained full feeding except those who underwent operation. SBS was done at their bedside in 15 out of 27 infants in the group less than 1.5kg. History of NEC, meconium related ileus, and gastrointestinal operation were more frequent in the group less than 1.5kg. The adverse effects of SBS in preterm infants included vomiting, delayed transit of the contrast media, all of which resolved in the end and there were no reports of aspiration or sepsis. Conclusion: Actively performing SBS test in preterm infants with feeding intolerance will promote feeding advancement by discovering the cause of feeding intolerance and subsequent management of the specific problem.

백혈병모양 반응을 보이는 극소저출생체중아의 주산기 인자와 신생아기 예후

이현주 ( Hyun Ju Lee ),심규홍 ( Gyu Hong Shim ),김은선 ( Eun Sun Kim ),이진아 ( Jin A Lee ),최창원 ( Chang Won Choi ),김이경 ( Ee Kyung Kim ),김한석 ( Han Suk Kim ),김병일 ( Beyong Il Kim ),최중환 ( Jung Hwan Choi ) 대한주산의학회 2009 大韓周産醫學會雜誌 Vol.20 No.1

목적: 미숙아에서 백혈병모양 반응이 있는 경우 기관지폐 형성 이상의 발생이 유의하게 증가한다고 알려져 있다. 이에 저자들은 극소저출생체중아에서 백혈병모양 반응과 주산기 인자 및 미숙아 합병증에 대한 관계를 알아보고자 본 연구를 시행하였다. 방법: 2005년 6월부터 2008년 6월까지 서울대학교병원과 분당 서울대학교병원 신생아 중환자실에 입원한 환아 중 출생 체중이 1,500 g 미만인 극소저출생체중아를 대상으로 후향적 환자 대조군 연구를 시행하였다. 백혈병모양 반응이 있는 환아는 비슷한 재태주령과 출생체중의 백혈구 증가증이 없는 (WBC counts, <35,000 cells/mm3) 대조군과 비교하였다. 백혈병모양 반응은 출생 후 1개월 이내에 백혈구 수치가 40,000 cells/mm3 이상이거나 절대 호중구 수가 30,000 cells/mm3 이상인 경우로 정의하였다. 결과: 주산기 인자에서 성별, 1분과 5분의 Apgar 점수, 산모의 말초혈액내 백혈구 수, 산전 스테로이드 투여 여부, 산모의 연령은 두 군간의 차이가 없었다. 양수내 양성 세균배양여부(67% vs 11%, P<0.01), 양수내 U. urealyticum 집락여부(52% vs 10%, P<0.01), 조직학적 융모양막염(81% vs 33%, P<0.01), 제대혈관염(50% vs 7%, P<0.01)은 백혈병모양 반응군에서 유의하게 높은 빈도를 나타냈다. 미숙아 합병증으로 호흡곤란 증후군, 동맥관 개존증, 괴사성 장염, 패혈증, 뇌실내 출혈, 기관지폐형성 이상은 두 군간의 유의한 차이를 보이지 않았다. 결론: 극소저출생체중아에서 생후 1개월 이내의 백혈병모양 반응은 기관지폐 형성 이상과 다른 미숙아 합병증과 연관이 없었고 융모양막염과 연관이 있었다. 백혈병모양 반응은 산전 염증반응에 대한 백혈구의 일시적인 동역학(kinetics)적 변화로 신생아의 임상적 경과에 크게 영향을 주지 않을 것으로 생각된다. Purpose: Leukemoid reaction (LR) represents inflammatory reaction in very low birth weight (VLBW) infants, and has been reported to relate with the development of bronchopulmonary dysplasia (BPD). This study was done to assess the relationship between perinatal characteristics and neonatal outcome of leukemoid reaction in VLBW infants. Methods: Three hundred and seventy two VLBW infants admitted to the NICUs of SNUCH and SNUBH between June 2005 to June 2008 were studied in a retrospective case-controlled manner. Of these VLBW infants, 32 LR (+) infants were compared with 83 LR (-) infants who were matched for gestational age and birth weight with respect to perinatal and neonatal clinical characteristics. LR was defined as a white blood cell (WBC) count of more than 40,000 cells/mm3 or absolute neutrophil count of more than 30.000 cells/mm3 during the first month of life. Results: Of 413 VLBW infants, There was no significant difference between the LR (+) and LR (-) infants in sex, Apgar scores, maternal peripheral WBC count, the use of antenatal steroids and maternal age. Comparing with LR (-) infants, LR (+) infants had a higher positive rate for amniotic fluid culture (67% vs 11%; P≤0.01), U. ureaplasma in amniotic fluid (52% vs 10%; P=0.01), histologic chorioamnionitis (81% vs 33%; P=0.01) and funisitis (50% vs 7%; P=0.01). The incidences of respiratory distress syndrome, patent ductus arteriosus, necrotizing enterocolitis, sepsis, intraventricular hemorrhage and BPD showed no significant difference between LR (+) and LR (-) infants. Conclusion: LR during the first month of life in VLBW infants was associated with chorioamnionitis, but not with the incidence of bronchopulmonary dysplasia or other neonatal morbidities. These findings suggest that LR may be a simple reflection of transient accelerated neutrophil production induced by prenatal inflammation without significant neonatal consequences.

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.