• 제목/요약/키워드: cardiomyopathy

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일차 심장 이식 후 발생한 Cardiac Allograft Vasculopathy의 치료로서의 심장 재이식 - 1예 보고 - (Heart Retransplantation in a Patient with Cardiac Allograft Vasculopathy after Primary Heart Transplantation? - A case report -)

  • 심만식;성기익;김욱성;이영탁;전은석;박표원
    • Journal of Chest Surgery
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    • 제43권1호
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    • pp.73-76
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    • 2010
  • Cardiac allograft vasculopathy (CAV)는 심장 이식 환자의 장기 생존율을 결정하는 중요한 합병증으로서 원위부 혈관의 미만성 병변을 가지는 것이 특징으로 재관류요법을 적용하기 어렵고 성적이 좋지 않다. CAV에 대한 치료로서 심장 재이식은 급성거부반응으로 재이식을 하는 경우보다 예후가 좋고 일차 심장이식 후의 결과와 비슷한 정도로 보고되고 있다. 이에 저자들은 28세 남자 환자로 8년 전에 확장성 심근증으로 일차 심장이식을 받은 뒤 만성 거부 반응으로 CAV가 발생하여 경피적 관상동맥 확장술을 시행하였으나 재협착과 심부전의 반복으로 더 이상의 재관류요법이 어려운 환자에게 심장 재이식을 하여 치료하였기에 보고하는 바이다.

소아환자에서 양심실 순환보조를 중개로 한 후 발생한 급성신부전 환자에서의 심장 이식수술 치험 1예 (A Case Report of Heart Transplantation Bridged by Bi-ventricular Assist Device in a Pediatric Patient of Prerenal Type ARF)

  • 나용준;곽재건;김진현;오세진;이재항;김웅한
    • Journal of Chest Surgery
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    • 제39권11호
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    • pp.854-857
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    • 2006
  • 환자는 8세 여자 환자로 확장성 심근병증을 진단 받은 환자이다. 환자는 심부전으로 인하여 양심실 순환보조를 15일간 시행하였다. 15일 중 후반기 7일은 급성 신부전증이 발생하여 지속적 정맥-정맥간 혈액투석을 시행하였다. 이후 뇌사상태의 A형 혈액형을 가진 심장 공여자에게서 심장을 이식받아 심장이식술을 시행하였다. 면역 억제제는 급성 신부전증을 고려하여 사용하였다. 신부전 상해는 2주간 지속되었고, 심장 이식술 후 약 14일 후 배뇨가 시작되었다. 환자는 수술 후 12일째에 시행한 우심실 조직검사에서 특별한 거부반응의 증거가 없었으며, 면역요법 후 수술 후 52일째에 특별한 문제 없이 퇴원하였다. 환자는 술 후 약 14개월간 조직검사에서 면역 거부반응 없이 외래 경과관찰 중이다.

심장이식 환자에서 Cyclosporine에 의한 중추신경독성 -1례 보고- (Cyclosporine-Assoc iated Central Neurotox ic its after Hearat Transplantat ion 1 Case Report)

  • 김용희;송현;송명근
    • Journal of Chest Surgery
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    • 제30권11호
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    • pp.1136-1138
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    • 1997
  • 승모판막 폐쇄부전증으로 승모판막 치환술을 받은 45세 남자가 확장성 심근증으로 심장이식수술을 받았다. 환자는 수술전 면역억제를 위하여 cyclosporine 400 mg, Immuran 250 mg과 Solumedrol 500 mg을 투약하였 다 술후 cyclosporine을 2 mg/kg/day로 정주했는데 술후 8시간이 지나도 혼수상태가 지속되어 cyclosporine을 1 mg/kg/day로 감량하였다. 당시 혈장내 cyclosporine농도는 345$\mu\textrm{g}$/L, 크레아티닌 수치는 1.8 mg/dl였으며 마그 네슘 수치는 정상수준이 였다. 환자의 의식은 술후 31시간째 완전히 회복되었으나 술후 36시간경부터 전신근 력약화, 초조감, 환청 및 환각을 호소하였다. 신경증상들은 술후 4일째 정상으로 회복되었으며, 환자는 술후 28일째 퇴원하였고 12개월째 후유증은 보이지 않고 있다.

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Microalbuminuria in children with urinary tract infection

  • Kwak, Byung-Ok;Chung, So-Chung;Kim, Kyo-Sun
    • Clinical and Experimental Pediatrics
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    • 제53권9호
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    • pp.840-844
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    • 2010
  • Purpose: Microalbuminuria is defined as increased urinary albumin excretion (30-300 mg/day) or microalbumin/creatinine ratio (30-300 mg/g) in a spot urine sample. Although microalbuminuria is a predictor of clinical nephropathy and cardiomyopathy, few studies have investigated microalbuminuria in children with urinary tract infection (UTI). Therefore, we compared the spot urine microalbumin/creatinine ratio in pediatric UTI patients with that of control subjects. Methods: We investigated the correlation between the ratio in children with UTI and age, height, weight, blood pressure, glomerular filtration rate (GFR), hematuria, vesicoureteral reflux, renal parenchymal defect, and renal scar, and its predictability for UTI complications. Results: We studied 66 patients (42 boys, 24 girls) and 52 healthy children (24 boys, 28 girls). The mean microalbumin/creatinine ratio in UTI patients was statistically significantly increased compared to the control group ($340.04{\pm}321.36mg/g$ vs. $225.68{\pm}154.61mg/g$, $P$=0.0141). The mean value of spot urine microalbumin/creatinine ratio ($384.70{\pm}342.22mg/g$ vs. $264.92{\pm}158.13mg/g$, $P$=0.0341) in 1-23 months age patient group showed statistically significant increase compared to control group. Microalbumin/creatinine ratio showed negative correlation to age (r=-0.29, $P$=0.0167), body surface area (BSA) (r=-0.29, $P$=0.0173) and GFR (r=-0.26, $P$=0.0343). The presence of hematuria ($P$=0.0169) was found to be correlated. Conclusion: The spot urine microalbumin/creatinine ratio in children with UTI was significantly greater than that in normal children, and it was positively correlated with GFR. This ratio is a potential prescreening and prognostic marker in UTI patients. Further studies are required to validate the predictability of microalbuminuria in pediatric UTI patients.

Fructus Amomi Cardamomi Extract Inhibits Coxsackievirus-B3 Induced Myocarditis in a Murine Myocarditis Model

  • Lee, Yun-Gyeong;Park, Jung-Ho;Jeon, Eun-Seok;Kim, Jin-Hee;Lim, Byung-Kwan
    • Journal of Microbiology and Biotechnology
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    • 제26권11호
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    • pp.2012-2018
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    • 2016
  • Coxsackievirus B3 (CVB3) is the main cause of acute myocarditis and dilated cardiomyopathy. Plant extracts are considered as useful materials to develop new antiviral drugs. We had previously selected candidate plant extracts, which showed anti-inflammatory effects. We examined the antiviral effects by using a HeLa cell survival assay. Among these extracts, we chose the Amomi Cardamomi (Amomi) extract, which showed strong antiviral effect and preserved cell survival in CVB3 infection. We investigated the mechanisms underlying the ability of Amomi extract to inhibit CVB3 infection and replication. HeLa cells were infected by CVB3 with or without Amomi extract. Erk and Akt activities, and their correlation with virus replication were observed. Live virus titers in cell supernatants and viral positive- and negative-strand RNA amplification were measured. Amomi extract significantly increased HeLa cell survival in different concentrations ($100-10{\mu}g/ml$). CVB3 capsid protein VP1 expression (76%) and viral protease 2A-induced eIF4G1 cleavage (70%) were significantly decreased in Amomi extract ($100{\mu}g/ml$) treated cells. The levels of positive- (20%) and negative-strand (80%) RNA were dramatically decreased compared with the control, as revealed by reverse transcription-PCR. In addition, Amomi extract improved mice survival (51% vs 26%) and dramatically reduced heart inflammation in a CVB3-induced myocarditis mouse model. These results suggested that Amomi extract significantly inhibited Enterovirus replication and myocarditis damage. Amomi may be developed as a therapeutic drug for Enterovirus.

"상한론"에 나타난 계(悸)와 관련된 처방들의 현대 질환 범위 고찰 (Study on Diseases Scope of Prescriptions Related with the Palpitation in "Shanghanlun")

  • 박미선;김영목
    • 동의생리병리학회지
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    • 제29권1호
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    • pp.1-10
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    • 2015
  • This article is a study on palpitation of which disease cause, disease mechanism and formulas were analyzed with reference to annotations on "Shanghanlun" and "Jinkuiyaolue". And the scope of modern diseases related with palpitation was drawn by research on clinical papers. The source books are "Zhujieshanghanlun" and "Jinkuiyaoluefanglun" and the clinical papers are searched in China Academic Journals(CAJ) of China National Knowledge Infrastructure(CNKI). 13 clauses in "Shanghanlun" and 9 clauses in "Jinkuiyaolue" and 12 formulas are related with palpitation. Disease mechanisms of palpitation were classified as yang deficiency, yin deficiency, qi deficiency, blood deficiency, retained fluid, cold, etc and these days, qi stagnation, phlegm turbidity, blood stasis and fire heat are also considered as disease mechanisms. Modern diseases related with palpitation are arrhythmia(extrasystole, atrial fibrillation, bradycardia, tachycardia, sick sinus syndrome, atrioventricular block), vascular diseases(arterial occlusion, phlebothrombosis, Buerger's disease, coronary artery disease, vasculitis), blood pressure disorder(hypertension, hypotension) and heart diseases such as heart failure, angina pectoris, myocardial infarction, myocarditis, cardiomyopathy, pericardial effusion. And diseases related with psychological change(cardiac neurosis, anxiety neurosis, neurosis, depression, hyperthyroidism, hypothyroidism), pyrexia, anemia, drug intoxication, etc are also related with palpitation. Zhen Wu Tang showing an efficacy in dilating blood vessels and strengthening cardiac function, Wuling Powder with diuretic effect and Fried Glycyrrhizae Decoction acting on the ${\beta}$ receptor are applied to heart failure in different ways. Fried Glycyrrhizae Decoction(308 cases), Zhen Wu Tang(154), Wuling Powder(54), Xiao Chaihu Tang(34), Sini San(20) are reported to have been clinically applied to cardiovascular diseases and Zhen Wu Tang and Wuling Powder mainly applied to heart failure, Fried Glycyrrhizae Decoction, Lizhong Wan, Sini San and Zhen Wu Tang chiefly applied to arrhythmia related diseases. This study focuses on the general research and consideration on clinical applications and is a preliminary study to understand relations between Korean Medicine's symptoms and categories of modern diseases.

Pressure-Overload Cardiac Hypertrophy Is Associated with Distinct Alternative Splicing Due to Altered Expression of Splicing Factors

  • Kim, Taeyong;Kim, Jin Ock;Oh, Jae Gyun;Hong, Seong-Eui;Kim, Do Han
    • Molecules and Cells
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    • 제37권1호
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    • pp.81-87
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    • 2014
  • Chronic pressure-overload cardiac hypertrophy is associated with an increased risk of morbidity/mortality, largely due to maladaptive remodeling and dilatation that progresses to dilated cardiomyopathy. Alternative splicing is an important biological mechanism that generates proteomic complexity and diversity. The recent development of next-generation RNA sequencing has improved our understanding of the qualitative signatures associated with alternative splicing in various biological conditions. However, the role of alternative splicing in cardiac hypertrophy is yet unknown. The present study employed RNA-Seq and a bioinformatic approach to detect the RNA splicing regulatory elements involved in alternative splicing during pressure-overload cardiac hypertrophy. We found GC-rich exonic motifs that regulate intron retention in 5' UTRs and AT-rich exonic motifs that are involved in exclusion of the AT-rich elements that cause mRNA instability in 3' UTRs. We also identified motifs in the intronic regions involved in exon exclusion and inclusion, which predicted splicing factors that bind to these motifs. We found, through Western blotting, that the expression levels of three splicing factors, ESRP1, PTB and SF2/ASF, were significantly altered during cardiac hypertrophy. Collectively, the present results suggest that chronic pressure-overload hypertrophy is closely associated with distinct alternative splicing due to altered expression of splicing factors.

Long-term cardiac composite risk following adjuvant treatment in breast cancer patients

  • Choi, Hong Bae;Yun, Sangchul;Cho, Sung Woo;Lee, Min Hyuk;Lee, Jihyoun;Park, Suyeon
    • 대한종양외과학회지
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    • 제14권2호
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    • pp.102-107
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    • 2018
  • Purpose: Cardiotoxicity is a serious late complication of breast cancer treatment. Individual treatment risk of specific drugs has been investigated. However, studies on the evaluation of the composite risk of chemotherapeutic agents are limited. Methods: We retrospectively analyzed the medical records of breast cancer patients who received adjuvant treatment and had available serial echocardiography results. Patients were assigned to subgroups based on chemotherapy containing anthracyclines (A), anthracyclines and taxanes (A+T), and radiotherapy (RT). The development of cardiac disease and serial ejection fraction (EF) were reviewed. EF decline up to 10% from baseline was considered grade 1 cardiotoxicity and EF decline >20% or absolute value <50% was considered grade 2 cardiotoxicity. The most recent medical records and echocardiography results over 1 year of chemotherapy completion were also reviewed. Late cardiotoxicity was defined as a lack of recovery of EF decline or aggravated EF decline from baseline. Results: In total, 123 patients were evaluated. A small reduction in EF was observed after chemotherapy in both chemotherapy groups. There were no significant differences between groups A and A+T in EF decline following chemotherapy. We could not find any differences in composite risk between the chemotherapy groups and the RT group during follow-up. Late cardiotoxicity was seen in 15.45% of patients. During follow-up, three patients were diagnosed with dilated cardiomyopathy. Conclusion: There was no significant composite risk elevation following adjuvant treatment of breast cancer. However, late cardiotoxicity was considerable and further research in this direction is necessary.

Clinical and molecular characterization of Korean children with infantile and late-onset Pompe disease: 10 years of experience with enzyme replacement therapy at a single center

  • Kim, Min-Sun;Song, Ari;Im, Minji;Huh, June;Kang, I-Seok;Song, Jinyoung;Yang, Aram;Kim, Jinsup;Kwon, Eun-Kyung;Choi, Eu-Jin;Han, Sun-Ju;Park, Hyung-Doo;Cho, Sung Yoon;Jin, Dong-Kyu
    • Clinical and Experimental Pediatrics
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    • 제62권6호
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    • pp.224-234
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    • 2019
  • Purpose: Pompe disease (PD) is an autosomal recessive disorder caused by a deficiency of acid alpha-glucosidase resulting from pathogenic GAA variants. This study describes the clinical features, genotypes, changes before and after enzyme replacement therapy (ERT), and long-term outcomes in patients with infantile-onset PD (IOPD) and late-onset PD (LOPD) at a tertiary medical center. Methods: The medical records of 5 Korean patients (2 male, 3 female patients) diagnosed with PD between 2002 and 2013 at Samsung Medical Center in Seoul, Republic of Korea were retrospectively reviewed for data, including clinical and genetic characteristics at diagnosis and clinical course after ERT. Results: Common initial symptoms included hypotonia, cyanosis, and tachycardia in patients with IOPD and limb girdle weakness in patients with LOPD. Electrocardiography at diagnosis revealed hypertrophic cardiomyopathy in all patients with IOPD who showed a stable disease course during a median follow-up period of 10 years. Patients with LOPD showed improved hepatomegaly and liver transaminase level after ERT. Conclusion: As ERT is effective for treatment of PD, early identification of this disease is very important. Thus, patients with IOPD should be considered candidates for clinical trials of new drugs in the future.

당뇨 합병증과 군령탕 구성성분의 네트워크 약리학 분석 및 효능 예측 (Network Pharmacology Analysis and Efficacy Prediction of GunryeongTang Constituents in Diabetic Complications)

  • 윤정주;김혜윰;태애림;이호섭;강대길
    • 대한한의학방제학회지
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    • 제32권1호
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    • pp.11-28
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    • 2024
  • Objectives : GunRyeong-Tang(GRT) is a traditional herbal prescription that combines Oryeongsan and Sagunja-tang. This study employed network analysis methods on the components of GRT and target genes related to diabetes complications to predict the improvement effects of GRT on diabetes complications. Methods : The collection of active compounds of GRT and related target genes involved the utilization of public databases and the PubChem database. We selected diabetes complication-related genes using GeneCards and confirmed their correlation through comparative analysis with the target genes of GRT. We constructed a network using Cytoscape 3.9.1 and conducted topological analysis. To predict the mechanism, we performed functional enrichment analysis based on Gene Ontology (GO) biological processes and Kyoto Encyclopedia of Genes and Genomes (KEGG) pathways. Results : Through network analysis, 234 active compounds and 1361 related genes were collected from GRT. A total of 9,136 genes related to diabetes complications were collected, and 1,039 target genes overlapping with the components of GRT were identified. The core genes of this network were TP53, INS, AKT1, ALB, and EGFR. In addition, GRT significantly reduced the H9c2 cell size and the expression of myocardial hypertrophy biomarkers (ANP, BNP), which were increased by high glucose (HG). Conclusions : Through this study, we were able to predict the activity and mechanism of action of GRT on diabetes and diabetic complications, and confirmed the potential of GRT as a treatment for diabetes complications through the effect of GRT on improving myocardial hypertrophy for diabetic cardiomyopathy.