• 제목/요약/키워드: CDH2

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늦게 발현된 선천성 횡경막 탈장 1예 (A Case of Late Presenting Congenital Diaphragmatic Hernia)

  • 송지은;권오건;김영호;이혜란
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • 제12권2호
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    • pp.246-250
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    • 2009
  • 평소 건강하게 지내다가 갑자기 시작된 반복적인 구토와 보채는 증상으로 5개월 여아가 병원에 내원하였고, 단순 흉부 방사선 검사에서 비정상적 음영이 좌측 흉부에서 관찰되었다. 이어 시행된 흉부 전산화 단층촬영에서 선천성 횡경막 탈장으로 진단되어, 흉부외과에서 수술적 치료 시행하였으며 수술 결과 횡경막 좌측후외방의 Bochdalek 형식의 선천성 횡격막 탈장으로 확인되었고, 수술적 치료 후 증상의 호전을 경험하였기에 문헌고찰과 함께 보고하는 바이다.

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Outcomes after Extracorporeal Membrane Oxygenation in Neonates with Congenital Diaphragmatic Hernia: A Single-Center Experience

  • Choi, Wooseok;Cho, Won Chul;Choi, Eun Seok;Yun, Tae-Jin;Park, Chun Soo
    • Journal of Chest Surgery
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    • 제54권5호
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    • pp.348-355
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    • 2021
  • Background: Congenital diaphragmatic hernia (CDH) is a rare disease often requiring mechanical ventilation after birth. In severe cases, extracorporeal membrane oxygenation (ECMO) may be needed. This study analyzed the outcomes of patients with CDH treated with ECMO and investigated factors related to in-hospital mortality. Methods: Among 254 newborns diagnosed with CDH between 2008 and 2020, 51 patients needed ECMO support. At Asan Medical Center, a multidisciplinary team approach has been applied for managing newborns with CDH since 2018. Outcomes were compared between hospital survivors and nonsurvivors. Results: ECMO was established at a median of 17 hours after birth. The mean birth weight was 3.1±0.5 kg. Twenty-three patients (23/51, 45.1%) were weaned from ECMO, and 16 patients (16/51, 31.4%) survived to discharge. The ECMO mode was veno-venous in 24 patients (47.1%) and veno-arterial in 27 patients (52.9%). Most cannulations (50/51, 98%) were accomplished through a transverse cervical incision. No significant between-group differences in baseline characteristics and prenatal indices were observed. The oxygenation index (1 hour before: 90.0 vs. 51.0, p=0.005) and blood lactate level (peak: 7.9 vs. 5.2 mmol/L, p=0.023) before ECMO were higher in nonsurvivors. Major bleeding during ECMO more frequently occurred in nonsurvivors (57.1% vs. 12.5%, p=0.007). In the multivariate analysis, the oxygenation index measured at 1 hour before ECMO initiation was identified as a significant risk factor for in-hospital mortality (odds ratio, 1.02; 95% confidence interval, 1.01-1.04; p=0.05). Conclusion: The survival of neonates after ECMO for CDH is suboptimal. Timely application of ECMO is crucial for better survival outcomes.

기도결찰을 시행한 토끼 태자의 실험적 횡격막탈장에서 Lamellar body counts (Lamellar Body Counts in Fetal Rabbits' Experimental Diaphragmatic Hernia and Tracheal Ligation)

  • 전용순;정수진;이정녀
    • Advances in pediatric surgery
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    • 제17권2호
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    • pp.179-187
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    • 2011
  • Experimental tracheal ligation (TL) has been shown to reverse the pulmonary hypoplasia associated with congenital diaphragmatic hernia (CDH) and to normalize gas exchange. The purpose of this study was to determine whether the TL would correct the surfactant deficiency present in the fetal rabbit model of CDH by using lamellar body count. Lamellar bodies are synthesized and secreted by the type II pneumocytes of fetal lung. The phospholipids present in these bodies constitute the major component of pulmonary surfactant. Twenty-one pregnant New Zealand rabbits underwent hysterotomy and fetal surgery on gestational day 24. Two fetuses of each pregnant rabbit were operated. In the fetus of one end of bicornuate uterus, left DH was created by excision of fetal diaphragm through open thoracotomy (DH Group). In the fetus of the other end of bicornuate uterus, left DH and TL were created (TL Group). The fetuses were delivered by Cesarean section on gestational day 31. Fourteen in control group, 12 in the DH group and 13 in TL group were born alive. En bloc excision of lungs, bronchi and trachea was done in all newborn rabbits. A five Fr catheter was inserted through trachea and repeated irrigations with 10 cc normal saline were done. The irrigated fluid was centrifuged at $280{\times}g$ for 5 minutes and the lamellar bodies were counted with the upper level fluid in platelet channel of electronic cell counter. The average lamellar body counts were $37.1{\pm}14.2{\times}10^3/{\mu}L$ in control group, $11.5{\pm}4.4 {\times}10^3/{\mu}L$ in DH group, and $6.5{\pm}0.9{\times}10^3/{\mu}L$ in TL group. Lamellar body count in DH group was lower than in control group and did not increase after TL. This study shows TL has no therapeutic effect on decreased surfactant level of CDH and the pregnant rabbit is appropriate for the animal model of CDH.

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토끼에서 태아수술에 의한 횡경막탈장과 기도결찰 (Experimental Diaphragmatic Hernia and Tracheal Ligtion in a Fetal Rabbit Model)

  • 조마해;김우기
    • Advances in pediatric surgery
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    • 제6권1호
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    • pp.1-9
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    • 2000
  • Despite of advances in perinatal management and treatment modalities congenital diaphragmatic hernia(CDH) remains a frustrating problem. Although the sheep has proven to be a reliable experimental model for the production of intrauterine CDH, the rabbit may have some advantages. These include lower cost, smaller body size, year-round availability, high number of fetuses per pregnancy, and short gestational period. To evaluate the feasibility of the rabbit model of CDH, twenty-seven pregnant New Zealand rabbits were utilized. Hysterotomy and an operative procedure for creating a diaphragmatic defect on gestational day 24 or 25, in two fetuses of each pregnant rabbit were performed. In one fetus of one cornu of the uterus, the left fetal diaphragm was excised through an open thoracotomy(DH group). In another fetus in the other cornu, CDH was created and the trachea clipped(Surgiclip, USSC, Norwalk, Conn., USA) (TL group). Delivery was by Cesarean section on 30 days of gestation. Among twenty- seven pregnant rabbits, 12 in the DH group and eight in the TL group were born alive. The most common herniated organ was the left lobe of the liver. In thee DH group, the lungs were hypoplastic with decreased lung weight/body weight ratio, reduced numbers of alveoli, thicker media of the pulmonary arteries, and immature alveoli. In TL group, the alveoli were more mature and did not differ from the control animals. In conclusion, (1) pulmonary hypoplasia develops in the fetal rabbit diaphragmatic hernia model and (2) simultaneous tracheal ligation prevents pulmonary hypoplasia.

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Differentially Expressed Genes by Inhibition of C-terminal Src Kinase by siRNA in Human Vascular Smooth Muscle Cells and Their Association with Blood Pressure

  • Hong, Kyung-Won;Shin, Young-Bin;Kim, Koan-Hoi;Oh, Berm-Seok
    • Genomics & Informatics
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    • 제9권3호
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    • pp.102-113
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    • 2011
  • C-terminal SRC kinase (CSK) is a ubiquitously expressed, cytosolic enzyme that phosphorylates and inactivates several SRC family protein tyrosine kinases. Recent genomewide association studies have implicated CSK in the regulation of blood pressure. The current study aim is to determine the blood pressure association of the genes regulated by CSK down-regulation. The CSK mRNA expression was downregulated in vascular smooth muscle cells using small interfering RNA (siRNA). CSK mRNA levels fell by 90% in cells that were treated with CSK siRNA; the RNA from these cells was examined by microarray using the Illumina HumanRef-8 v3 platform, which comprises 24,526 reference mRNA probes. On treatment with CSK siRNA, 19 genes were downregulated by more than 2-fold and 13 genes were upregulated by more than 2-fold. Three (CANX, SLC30A7, and HMOX1) of them revealed more than 3 fold differential expression. Interestingly, the HMOX1 SNPs were associated with diastolic blood pressure in the 7551 Koreans using Korea Association REsource data, and the result was supported by the other reports that HMOX1 linked to blood vessel maintenance. Among the remaining 29 differentially expressed genes, seven (SSBP1, CDH2, YWHAE, ME2, PFTK1, G3BP2, and TUFT1) revealed association with both systolic and diastolic blood pressures. The CDH2 gene was linked to blood pressures. Conclusively, we identified 32 differentially expressed genes which were regulated by CSK reduction, and two (HOMX1 and CDH2) of them might influence the blood pressure regulation through CSK pathway.

Acinetobacter calcoaceticus C10에서 Cyclohexanol Dehydrogenase의 생합성 억제 (Repression of Cyclohexanol Dehydrogenase in Acinetobacter calcoaceticus C10)

  • 박희동;박종성;이인구
    • Current Research on Agriculture and Life Sciences
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    • 제5권
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    • pp.68-74
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    • 1987
  • A. calcoaceticus C10은 CL 배지에서 ${\varepsilon}$-caprolactone 및 succinate에 의해 생육에 아무 영향도 받지 않았으나 adipate와 xylose에 의해서 균의 생육도가 증가하였다. 특히 이 균이 탄소원으로 이용할 수 없는 포도당에 의해서도 생육도가 증가하였으며 6시간 배양 후 0.2%의 포도당을 첨가한 경우에는 16시간 후 CL 배지에서 생육한 것보다 약 2배의 생육도를 냐타내었다. A. calcoacelicus C10이 생산하는 cyclohexanol dehydrogenase(CDH)는 ${\varepsilon}$-caprolactone, succinate, xylose 및 포도당에 의해 이화물 억제를 받지 않았으나 사이클로헥사놀 대사경로의 최종생산물인 adipate에 의하여 생합성 억제를 받았다. CL 배지에 0.1%의 adipate를 첨가한 배지에서는 CDH가 다소 유도되었으나 0.2%의 adipate를 첨가한 배지에서는 CDH가 거의 유도되지 않아 0.5%의 adipate를 첨가한 것과 비슷한 억제효과를 나타내었다.

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만성매일두통 환자에 대한 홍화 약침 치료 : 예비연구 (The effect of Carthami-Semen acupuncture on chronic daily headache: A pilot study)

  • 김민경;박성욱;정우상;문상관;박정미;고창남;조기호;김영석;배형섭
    • 대한약침학회지
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    • 제13권4호
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    • pp.109-118
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    • 2010
  • Objectives : This pilot study is designed to reveal the effect of Carthami-semen acupuncture treatment on chronic daily headache. Methods : 1. All the voluntary subjects were diagnosed as CDH. 2. Carthami-semen acupuncture is applied 2 times a week for 4 weeks. 3. The patients were assessed by Headache impairment test score(HIT), Short Form 36 (SF-36) Health Survey, and Headache Free Days(HFD) before and after 4 weeks treatment, and two weeks the treatment finished. Results : The result were as follows ; 1. HIT score decreased from $65.3{\pm}5.5$ to $53.1{\pm}9.4$, and furthermore declined to $49.5{\pm}8.0$ after 2 weeks the treatment finished. 2. HFD increased from 19.1% to 31.5% during the treatment time. Moreover it showed continuous effectiveness after 2 weeks the treatment finished. 3. SF-36 showed significant improvement on all of the QOL items. Conclusions : This pilot study suggests that Carthami-semen acupuncture treatment has an effect on the CDH patients.

Methylation Status and Expression of E-cadherin in Oral Squamous Cell Carcinomas Compared t6 Benign Oral Epithelial Lesions

  • Son, Hyun-Jin;Chu, Jung-Youb;Cho, Eui-Sic;Lee, Dong-Geun;Min, Myung-Gee;Lee, Suk-Keun;Cho, Nam-Pyo
    • International Journal of Oral Biology
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    • 제31권2호
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    • pp.27-32
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    • 2006
  • Expression of invasion/metastasis suppressor, E-cadherin, is reduced in many types of human carcinomas. Although somatic and germline mutations in the CDH1, which encodes the human E-cadherin, have frequently been reported in cases with diffuse gastric and lobular breast cancers, irreversible genetic inactivations are rare in other human carcinomas. Recently, it has been well documented that some genes in human cancers may be inactivated by altered CpG methylation. Herein, we determined the expression and methylation status of E-cadherin in oral squamous cell carcinoma(SCC) by immunohistochemistry and methylation-specific PCR. The expression of E-cadherin was significantly higher in the well-differentiated oral SCCs than the moderately or poorly differentiated ones. None of eight tested benign epithelial hyperplasias showed aberrant methylation, whereas five of 12 oral squamous cell carcinomas showed aberrant methylation. When we compared E-cadherin expression with methylation status, oral SCCs with normal methylation showed a higher expression of E-cadherin than those with methylation. These findings suggest that aberrant CpG methylation of CDH1 promoter region is closely associated with transcriptional inactivation and might be involved in tumor progression of the oral mucosa.

유료 방송 시스템에 적합한 ID기반의 2 라운드 그룹키 동의 프로토콜 (Two-round ID-based Group Key Agreement Fitted for Pay-TV System)

  • 김현주;남정현;김승주;원동호
    • 정보보호학회논문지
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    • 제15권1호
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    • pp.41-55
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    • 2005
  • 그룹키 통의 프로토콜은 일련의 그룹을 형성하는 다수의 통신 참여자들이 공개된 통신망을 통해 안전하고 효율적인 방법으로 그룹의 세션키를 설정하기 위한 목적으로 설계된다. 본 논문에서는 유료 방송 시스템과 같은 그룹중심의 응용이나 서비스에 적합한 ID 기반의 2 라운드의 그룹키 동의 프로토콜을 제안하고, 이의 안전성을 CDH 가정과 BDDH 가정에 기반하여 랜덤 오라클 모델에서 증명한다. 제안하는 프로토콜은 Nam이 제안한 3 라운드 그룹키 동의 프로토콜을 기초로 설계된 것으로, 개인식별정보에 기반한 암호 시스템을 사용하여 키 관리 절차를 보다 간단히 하였으며, 새로운 인증 메카니즘을 사용하여 키 전송 메시지들의 길이를 줄이고 결합적 단순성을 제공하였다. 또한 제안하는 프로토콜은 전송되는 메시지들에 대한 인증을 묶음(batch) 기법을 사용하여 검증하도록 설계하여 효율성을 더욱 개선 시 켰다.

선천성 횡격막 탈장증 교정 후 발견된 활주형 탈장 1예 (A Case of Sliding Hiatal Hernia associated with Bochdalek Hernia Repair)

  • 남석진;김현학;이석구
    • Advances in pediatric surgery
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    • 제2권2호
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    • pp.129-132
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    • 1996
  • This is a case report of a sliding hiatal hernia with severe gastroesophageal reflux(GER) after repair of congenital diaphragmatic hernia(CDH). It was not possible to determine whether the hiatal hernia is a de novo lesion which was missed at the original operation or a consequence of overzealous repair of the Bochdalek defect at the expense of weakening of the diaphragmatic crura. This case demonstrates that a sliding hiatal hernia can be a cause of severe gastroesophageal reflux that should be managed surgically.

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