• Journal of Chest Surgery
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• 제43권6호
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• pp.725-728
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• 2010

횡문근육종은 영아에서 발생하는 가장 흔한 심장 내 양성종양으로 많은 수에서 그 크기가 감소하여 자연 소멸되는 것으로 알려져 있다. 하지만 일부 환자에서는 성장의 기능적 문제를 야기하여 수술적 치료를 필요로 하기도 한다. 환아는 산전 초음파에서 심실 내 다발성 종양이 발견되었으며 출생 직후부터 심한 좌심실 유출로 폐쇄로 인한 심장기능부전이 발생하였다. 생후 1일째 응급으로 수술을 시행하였으며 대동맥 절개를 통한 경판막 접근법으로 유출로를 막고 있는 종양의 일부분만을 절제하였다. 수술 후 시행한 초음파에서 유출로 폐쇄가 완전히 없어진 것을 확인할 수 있었으며, 3년간 정기적으로 경과 관찰한 결과 남아있는 종양은 그 크기가 점차 줄어드는 것을 확인할 수 있었다.

• Clinical and Experimental Pediatrics
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• 제54권6호
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• pp.260-266
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• 2011

Purpose: Infantile hepatic hemangioendothelioma (IHHE) is the most common type of hepatic vascular tumor in infancy. We conducted this study to review our clinical experience of patients with IHHE and to suggest management strategies. Methods: We retrospectively analyzed the medical records of 23 IHHE patients (10 males, 13 females) treated at the Asan Medical Center between 1996 and 2009. Results: Median age at diagnosis was 38 days (range, 1 to 381 days). Seven patients (30%) were diagnosed with IHHE based on sonographically detected fetal liver masses, 5 (22%) were diagnosed incidentally in the absence of symptoms, 5 (22%) had congestive heart failure, 3 (13%) had skin hemangiomas, 2 (9%) had abnormal liver function tests, and 1 (4%) had hepatomegaly. All diagnoses were based on imaging results, and were confirmed in three patients by histopathology analysis. Six patients were observed without receiving any treatment, whereas 12 received corticosteroids and/or interferonalpha. One patient with congestive heart failure and a resectable unilobar tumor underwent surgical resection. Three patients with congestive heart failure and unresectable tumors were managed by hepatic artery embolization with/without medical treatment. At a median follow-up of 29 months (range, 1 to 156 months), 21 (91%) patients showed complete tumor disappearance or >50% decrease in tumor size. One patient died due to tumor-related causes. Conclusion: IHHE generally has a benign clinical course with low morbidity and mortality rates. Clinical course and treatment outcome did not differ significantly between medically treated and non-treated groups. Surgically unresectable patients with significant symptoms may be treated medically or with hepatic artery embolization.

• 대한전기학회:학술대회논문집
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• 대한전기학회 1999년도 하계학술대회 논문집 G
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• pp.3253-3255
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• 1999

출생시 국내에서 영아 사망률은 약 1%에 이르고 태아의 질병발생과 사망은 계속적으로 일어나고 있으므로 저가의 태아감시기술의 개발이 절실하다. 이를 위하여 본 연구는 임산부의 복부로부터 태아의 움직임과 심음을 검출하는 증폭기의 개발을 목표로 한다. 검출된 신호는 듣거나 녹음할 수 있으며. A/D 변환할 경우 PC에서 태아의 심음을 분석할 수 있게 한다. 개발된 증폭기를 이용하여 잡음에 노출된 일반 대학병원 환경에서 30명의 임산부를 대상으로 임상실험을 수행한 결과, 저잡음 특성을 나타내고. 빠른 경우 22주에서도 태아의 심음을 검출할 수 있었고. 심음의 주기검출이 가능하였다.

• 대한소아치과학회지
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• 제35권2호
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• pp.319-323
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• 2008

에드워드 증후군은 18번 세염색체(trisomy)성 질환으로 신체의 모든 기관에 다수의 영향을 주며 정신 지체, 발육 지연, 호흡 곤란, 선천성 심장 질환 등의 전신 질환과 손가락의 굴곡변형과 족부후방돌출(rocker-bottom feet)의 소견을 보인다. 산모가 에드워드 증후군 환아를 임신했을 경우 양수과다, 작은 태반, 단일 제대 동맥의 소견을 보인다. 에드워드 증후군을 가진 환아는 생존율이 매우 낮다. 절반이 자궁 내에서 사망하며, 출생아의 50%는 생존율이 2개월이고, $5{\sim}10%$는 생존율이 1년 정도이다. 에드워드 증후군을 가진 환아가 충치 치료를 주소로 내원하였다. 환아의 전신 질환과 심장 수술 병력, 저체중, 기도확보 유지가 어려운 점을 고려하여 전신마취 하에 치과 치료를 시행하였다. 저자는 에드워드 증후군 환아의 치과 치료 후 다소의 지견을 얻었기에 보고하는 바이다.

• Clinical and Experimental Reproductive Medicine
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• 제37권1호
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• pp.41-48
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• 2010

목 적: 체외수정시술 시 황체기 보강은 근주, 경질, 경구 등의 다양한 방법으로 이루어지고 있는데, 경구 투여법은 환자의 순응도가 높고 비교적 안전한 방법이다. 본 연구에서는 황체기 보강을 근주로 시작하여 경구 투여로 전환하는 방법의 효용성과 전환 시기에 따른 결과를 비교 분석하고자 하였다. 연구방법: 2003년 9월부터 2009년 6월까지 분당서울대학교병원에서 체외수정시술을 시행받은 환자에서 배아 이식 후 태아 심박동까지 확인된 76주기를 대상으로 하였다. 과배란유도는 GnRH agonist long protocol (n=7) 또는 GnRH antagonist protocol (n=66)을 이용하였으며 3주기에서는 냉동배아 이식을 시행하였다. 황체기 보강을 위하여 난자 채취일부터 매일 progest in oil 50 mg을 근주하였고, 태아 심박동이 확인된 후 임신 6~7주 (n=17) 또는 8주 이후 (n=59)에 micronized progesterone (Utrogestan, Laboratoires Besins International, France) 300 mg을 매일 경구로 투여하였다. 결 과: 대상군 전체의 유산율은 3.9% (3/76)이었으며, 경구 투여 전환시의 임신 주수는 평균 8주 4일 (난자 채취일로부터 $46{\pm}5.8$일)이었다. 황체기 보강을 임신 6~7주 사이에 경구 투여로 전환한 17주기 중에서 1례의 자연유산이 확인되었으며 유산 시 주수는 9주 4일이었다. 8주 이후에 경구 투여로 전환한 59주기 중에서는 2례의 자연유산이 확인되었으며 (11주 3일, 11주 4일), 두 군의 자연유산율은 각각 5.6%와 3.4%로 두 군 간에 통계적으로 유의한 차이는 없었다 (p=0.678). 결 론: 체외수정시술 후 황체기 보강을 시행하는 경우 태아 심박동이 확인된 이후에 근주 투여를 경구 투여로 전환하는 방법은 비교적 낮은 유산율을 보임을 확인하였으며, 특히 8주 이전에 전환하는 것도 유용한 방법이라 사료된다.

• Clinical and Experimental Pediatrics
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• 제58권10호
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• pp.392-397
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• 2015

Purpose: Alagille syndrome is a complex hereditary disorder that is associated with cardiac, hepatic, skeletal, ocular, and facial abnormalities. Mutations in the Notch signaling pathway, such as in JAG1 and NOTCH2, play a key role in embryonic development. A cardiac or hepatic presentation is a critical factor for determining the prognosis. Methods: We conducted a retrospective study of 41 patients with Alagille syndrome or a JAG1 mutation between 1983 and 2013. Results: The first presentations were jaundice, murmur, cyanosis, and small bowel obstruction at a median age of 1.0 months (range, 0-24 months). The JAG1 mutation was found in 27 of the 28 genetically-tested patients. Cardiovascular anomalies were identified in 36 patients, chronic cholestasis was identified in 34, and liver transplantation was performed in 9. There was no significant correlation between the severity of the liver and cardiac diseases. The most common cardiovascular anomaly was peripheral pulmonary stenosis (83.3%), with 13 patients having significant hemodynamic derangement and 12 undergoing surgical repair. A total bilirubin level of >15 mg/dL with a complex surgical procedure increased the surgical mortality (P=0.022). Eight patients died after a median period of 2.67 years (range, 0.33-15 years). The groups with fetal presentation and with combined severe liver and heart disease had the poorest survival (P<0.001). Conclusion: The group with combined severe liver and heart disease had the poorest survival, and a multidisciplinary approach is necessary to improve the outcome.

• Asian-Australasian Journal of Animal Sciences
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• 제26권8호
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• pp.1057-1064
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• 2013

p70 ribosomal S6 kinase (p70S6K) can integrate nutrient and growth factor signals to promote cell growth and survival. We report our molecular characterization of the complementary DNA (cDNA) that encodes the goat p70S6K gene 40S ribosomal S6 kinase 1 (S6K1) (GenBank accession GU144017) and its 3' noncoding sequence in Inner Mongolia Cashmere goats (Capra hircus). Goat S6K1 cDNA was 2,272 bp and include an open reading frame (ORF) of 1,578 bp, corresponding to a polypeptide of 525 amino acids, and a 694-residue 3' noncoding sequence with a polyadenylation signal at nucleotides 2,218 to 2,223. The relative abundance of S6K1 mRNA was measured by real-time PCR in 6 tissues, and p70S6K expression was examined by immunohistochemistry in heart and testis. The phosphorylation of p70S6K is regulated by mitogen-activated protein kinase (MAPK) signaling in fetal fibroblasts.

• Clinical and Experimental Pediatrics
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• 제54권11호
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• pp.443-455
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• 2011

Neonatal mortality rate (NMR) or infant mortality rate (IMR) are the rate of deaths per 1,000 live births at which babies of either less than four weeks or of one year of age die, respectively. The NMR and IMR are commonly accepted as a measure of the general health and well-being of a population. Korea's NMR and IMR fell significantly between 1993 and 2009 from 6.6 and 9.9 to 1.7 and 3.2, respectively. Common causes of infantile death in 2008 had decreased compared with those in 1996 such as other disorders originating in the perinatal period, congenital malformation of the heart, bacterial sepsis of newborns, disorders related to length of gestation and fetal growth, intra-uterine hypoxia, birth asphyxia. However, some other causes are on the increase, such as respiratory distress of newborn, other respiratory conditions originating in the perinatal period, other congenital malformation, diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism. In this study, we provide basic data about changes of NMR and IMR and the causes of neonatal and infantile death from 1983 to 2009 in Korea.

• Asian Pacific Journal of Cancer Prevention
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• 제15권22호
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• pp.9557-9565
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• 2014

MiRNAs are endogenous, single stranded ~22-nucleotide non-coding RNAs (ncRNAs) which are transcribed by RNA polymerase II and mediate negative post-transcriptional gene regulation through binding to 3'untranslated regions (UTR), possibly open reading frames (ORFs) or 5'UTRs of target mRNAs. MiRNAs are involved in the normal physiology of eukaryotic cells, so dysregulation may be associated with diseases like cancer, and neurodegenerative, heart and other disorders. Among all cancers, lung cancer, with high incidence and mortality worldwide, is classified into two main groups: non-small cell lung cancer and small cell lung cancer. Recent promising studies suggest that gene expression profiles and miRNA signatures could be a useful step in a noninvasive, low-cost and repeatable screening process of lung cancer. Similarly, every stage of lung development during fetal life is associated with specific miRNAs. Since lung development and lung cancer phenomena share the same physiological, biological and molecular processes like cell proliferation, development and shared mRNA or expression regulation pathways, and according to data adopted from various studies, they may have partially shared miRNA signature. Thus, focusing on lung cancer in relation to lung development in miRNA studies might provide clues for lung cancer diagnosis and prognosis.

• 한국응용약물학회:학술대회논문집
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• 한국응용약물학회 2003년도 Annual Meeting of KSAP : International Symposium on Pharmaceutical and Biomedical Sciences on Obesity
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• pp.87-87
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• 2003

Taurine (2-aminoethanesulfonic acid, $^{+}NH_3CH_2CH_2{SO_3}^{-}$) is endogenous $\beta$-amino acid which is essential in fetal nutrition and development and is present in abundant quantities in several tissues of fetus. In utero, taurine deficiency causes abnormal development and abnormal function of brain, retina, kidney and myocardium. Thus, transfer of taurine into fetus is important during embryonic development. Taurine transporter (TauT) has 12 hydrophobic membrane -spanning domains, which is typical of the $Na^{+}$- and $Cl^{-}$-dependent transporter gene family. Among the various biosynthetic enzymes of taurine, cysteine sulfinic acid decarboxylase (CSD) is the rate-limiting enzyme for biosynthesis of taurine. However, the enzyme activities of taurine biosynthesis are limited in early stage of embryonic development. To analyze the expression period of TauT and CSD during embryonic development, we have investigated the gene expression of TauT and CSD using reverse transcriptase polymerase chain reaction (RT-PCR) in mouse and chicken embryos. RT-PCR anaylsis revealed that both TauT and CSD mRNAs were already expressed at Day-4.5 in mouse embryo. In chicken whole embryo, TauT and CSD mRNAs began to appear on developing times of 48 hrs and 12 hrs, respectively. TauT mRNA was detected in the organs of heart, brain and eye of the day-3 chicken embryo. Our data show that TauT and CSD mRNAs were expressed in early stage of embryonic development.