Journal of Rare Cardiovascular Diseases最新文献_第2页

Amyloid cardiomyopathy: the different facets of a not so rare disease (RCD code III 3A.1, III 3A.2) 淀粉样心肌病:一种不那么罕见的疾病的不同方面(RCD代码III 3A)。1、3、3、a、2)

Journal of Rare Cardiovascular Diseases Pub Date : 2020-01-27 DOI: 10.20418/JRCD.VOL4NO3.394

Ioannis Boutsikos, M. Dova, Aristea Tsaroucha, D. Chatzis

{"title":"Amyloid cardiomyopathy: the different facets of a not so rare disease (RCD code III 3A.1, III 3A.2)","authors":"Ioannis Boutsikos, M. Dova, Aristea Tsaroucha, D. Chatzis","doi":"10.20418/JRCD.VOL4NO3.394","DOIUrl":"https://doi.org/10.20418/JRCD.VOL4NO3.394","url":null,"abstract":"Amyloidosis is a rare disorder which can affect multiple organs, with cardiac amyloidosis (CA) being one of its main clinical manifestations. CA has 2 distinct subtypes, AL amyloidosis and transthyretin amyloidosis (ATTR), with different histopathological appearances and subsequently different treatment strategies. The diagnostic methods available include multiple modalities to properly detect CA, ranging from the surface electrocardiogram and simple 2D‐echocardiography to more sophisticated methods such as specific biomarkers, speckle tracking echocardiography, or even bone scintigraphy with radioisotopes. However, the definitive diagnosis is reached with an endomyocardial biopsy or a biopsy from peripheral tissue. CA is a particularly challenging disorder in terms of patient management, which ranges from symptomatic treatment for heart failure symptoms to special medication and multiple drug regimens. For AL amyloidosis, a 3‐drug regimen is used as a first‐line therapy including cyclophosphamide, a proteasome inhibitor, and dexamethasone. For ATTR amyloidosis, specific medications are used such as Tafamidis, depending on the specific stage of the disease. Clinical trials of new drugs are pending with the ultimate goal of improving treatment rates and quality of life in patients with any type of CA. JRCD 2019; 4 (2): 34–41","PeriodicalId":37488,"journal":{"name":"Journal of Rare Cardiovascular Diseases","volume":"69 1","pages":"34-41"},"PeriodicalIF":0.0,"publicationDate":"2020-01-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"90527142","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 1

Sinus of Valsalva aneurysm dissecting the inter ventricular septum with rupture into the right ven tricle (RCD code: I 1B.O) Valsalva动脉瘤窦切室间隔并破裂至右三心室(RCD代码:1b1 . o)

Journal of Rare Cardiovascular Diseases Pub Date : 2020-01-27 DOI: 10.20418/JRCD.VOL4NO3.377

N. Oryshchyn, Y. Ivaniv, L. Kulyk, Oksana Predzemirska

引用次数: 1

Journal of Rare Cardiovascular Diseases now included in Directory of Open Access Journals (DOAJ)! 罕见心血管疾病杂志现已列入开放获取期刊目录(DOAJ)!

Journal of Rare Cardiovascular Diseases Pub Date : 2020-01-27 DOI: 10.20418/JRCD.VOL4NO3.402

P. Podolec

引用次数: 1

The potential role of the immune system and its modulation in rare diseases 免疫系统及其调节在罕见疾病中的潜在作用

Journal of Rare Cardiovascular Diseases Pub Date : 2019-08-19 DOI: 10.20418/JRCD.V4I2.379.G296

P. Podolec

引用次数: 0

Prognostic value of inflammatory markers in acute coronary syndrome in a population with premature cardiovascular disease (RCD code: VIII) 急性冠状动脉综合征炎症标志物在早发性心血管疾病人群中的预后价值(RCD代码:VIII)

Journal of Rare Cardiovascular Diseases Pub Date : 2019-08-09 DOI: 10.20418/JRCD.VOL4NO2.373

A. Pura, Marta Wilk, M. Olszowska

{"title":"Prognostic value of inflammatory markers in acute coronary syndrome in a population with premature cardiovascular disease (RCD code: VIII)","authors":"A. Pura, Marta Wilk, M. Olszowska","doi":"10.20418/JRCD.VOL4NO2.373","DOIUrl":"https://doi.org/10.20418/JRCD.VOL4NO2.373","url":null,"abstract":"Introduction: Inflammation plays a significant role in the development of atherosclerosis, and inflammatory markers could be used in risk assessment of patients with ischaemic heart disease. The use of such markers could be beneficial in younger patients, since in this group, prevention is more effective than treatment of myocardial infarctions, as long‐term prognoses are often unfavourable. Aim: Our goal was to examine the value of inflammatory biomarkers as an assessment tool for cardiovascular risk in a population with a premature ischaemic heart disease. Materials and Methods: We analysed laboratory test results of 100 consecutive patients hospitalized in the John Paul II Hospital in Krakow, Poland between 2014–2017. Inclusion criteria was cardiovascular disease diagnosed in coronarography under the age of 55 years for women and 45 years for men. We excluded patients with incomplete data and acute infections. The remaining 90 patients were divided into groups based on the reason of admission (myocardial infarction or elective diagnostics). Results: White blood cell count (median of 6.990 × 10 3 per/μl in comparison to 8.535 × 10 3 /μl) and absolute neutrophil count (median of 4060 × 10 3 /μl and 5360 × 10 3 /μl ) were lower in the group admitted for diagnostics. Although inflammatory biomarkers (platelet distribution width, white blood cells) were within normal ranges, we observed higher values (above the medians for studied population) in the group admitted to hospital due to acute coronary syndrome. Conclusion: Inflammatory biomarkers could be useful in the assessment of cardiovascular risk in patients with a premature ischaemic heart disease. Since the measured values of the inflammatory biomarkers were within normal range in the examined population, further studies should be conducted to determine appropriate cut‐off values. JRCD 2019; 4 (2): 37–41.","PeriodicalId":37488,"journal":{"name":"Journal of Rare Cardiovascular Diseases","volume":"25 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-08-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"73073668","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Wunderlich’s syndrome associated with anticoa gulant treatment, heart failure, and post-radiation vasculopathy (RCD code: I-O) Wunderlich综合征与抗凝治疗、心力衰竭和放疗后血管病变相关(RCD代码:I-O)

Journal of Rare Cardiovascular Diseases Pub Date : 2019-08-06 DOI: 10.20418/JRCD.VOL4NO2.370

A. Pytlewski, Mateusz Siwek, Leszek Drabik, Miłosz Delikat, Dawid Śmietana, E. Sobieraj, P. Iwaszczuk, A. Mazurek, W. Płazak

{"title":"Wunderlich’s syndrome associated with anticoa gulant treatment, heart failure, and post-radiation vasculopathy (RCD code: I-O)","authors":"A. Pytlewski, Mateusz Siwek, Leszek Drabik, Miłosz Delikat, Dawid Śmietana, E. Sobieraj, P. Iwaszczuk, A. Mazurek, W. Płazak","doi":"10.20418/JRCD.VOL4NO2.370","DOIUrl":"https://doi.org/10.20418/JRCD.VOL4NO2.370","url":null,"abstract":"Background: Wunderlich’s syndrome (WS) is an extremely rare condition, characterised by spontaneous haemorrhage into the subcapsular, perirenal, and retroperitoneal spaces. The most common cause is renal angiomyolipoma. Classic symptoms, such as acute flank pain, flank mass, and hypovolemic shock, known as Lenk’s triad, have limited sensitivity and specificity for detecting WS. High mortality may be related to the difficulties in diagnosis, poor disease recognition, and non‐specific symptoms and signs. Computed tomography (CT) is the method of choice for the diagnosis of WS. Depending on the severity of symptoms, a conservative or invasive approach is reasonable. Methods and results: We report the case of a 58‐year‐old male with multivalvular heart defect, atrial fibrillation, and exacerbation of chronic heart failure complicated with nontraumatic bleeding into the retroperitoneal space. CT revealed a massive (14x11x26 cm) retroperitoneal and perirenal haematoma. The patient was successfully treated with transfusion of red blood cells, fresh frozen plasma, and intravenous fluids. Simultaneously, heart failure treatment was initiated. In view of the chronic and idiopathic character of the haematoma and successful conservative treatment, there was no indication for surgery. Discussion: Several factors might have contributed to the bleeding. First, the patient was receiving anticoagulant treatment to reduce the risk of arterial thromboembolism in atrial fibrillation. Another possible cause is post‐radiation vasculopathy, an adverse effect of previous radiation therapy for malignant granuloma. The third factor which could have contributed to bleeding was the exacerbation of chronic heart failure and pulmonary hypertension. JRCD 2019; 4 (2): 51-55.","PeriodicalId":37488,"journal":{"name":"Journal of Rare Cardiovascular Diseases","volume":"23 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-08-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"83706737","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 2

Foetal 2:1 atrioventricular block in a patient with Timothy syndrome (LQT8) (RCDD code: VI-1B-1.2) 1例Timothy综合征(LQT8)患者胎儿2:1房室传导阻滞(RCDD代码:VI-1B-1.2)

Journal of Rare Cardiovascular Diseases Pub Date : 2019-08-05 DOI: 10.20418/JRCD.VOL4NO2.361

P. Yubbu, H. Chew, Y. Yakob, S. Lua, H. Samion

引用次数: 0

Partial recovery of left ventricular function in dilated cardiomyopathy as a result of tuberculosis treatment (RCD code: III-1B.9.o) 扩张型心肌病肺结核治疗后左心室功能部分恢复(RCD代码:III-1B.9.o)

Journal of Rare Cardiovascular Diseases Pub Date : 2019-07-31 DOI: 10.20418/JRCD.VOL4NO2.371

A. Wileczek, M. Trawinska, S. Stec

引用次数: 0

Current views on the use of interferons in the treatment of polycythaemia vera (RCD code: VIII) 干扰素治疗真性红细胞增多症的现状(RCD代码:VIII)

Journal of Rare Cardiovascular Diseases Pub Date : 2019-05-08 DOI: 10.20418/JRCD.VOL4NO2.363

Anna Prochwicz, E. Szczepanek, D. Krochmalczyk

引用次数: 0

Effusive-constrictive pericarditis in a patient with end-stage renal disease (RCD code: VI-4D.1) 终末期肾病患者积液性缩窄性心包炎1例(RCD代码:VI-4D.1)

Journal of Rare Cardiovascular Diseases Pub Date : 2019-04-25 DOI: 10.20418/JRCD.VOL4NO2.350

A. Wojtkowska, A. Tomaszewski, Magdalena Zakościelna, E. Czekajska-Chehab, A. Wysokiński

{"title":"Effusive-constrictive pericarditis in a patient with end-stage renal disease (RCD code: VI-4D.1)","authors":"A. Wojtkowska, A. Tomaszewski, Magdalena Zakościelna, E. Czekajska-Chehab, A. Wysokiński","doi":"10.20418/JRCD.VOL4NO2.350","DOIUrl":"https://doi.org/10.20418/JRCD.VOL4NO2.350","url":null,"abstract":"Renal disease, especially end‐stage renal disease, is associated with possible pericardial involvement. We report the case of a 51‐year‐old patient with a history of end‐stage‐renal disease, currently receiving chronic haemodialysis. The patient was admitted to the ward due decreased exercise tolerance and elevation of inflammatory markers. Features of active exudative‐constrictive pericarditis were detected upon imaging studies (echocardiography, computed tomography). The haemodialysis regimen was intensified and anti‐inflammatory treatment was administered. In the follow‐up examination, the patient presented with improved clinical status and echocardiography revealed a decreased amount of pericardial fluid with no pattern of constriction. The elevation of CRP and evidence of active pericardial inflammation upon contrast enhancement seen in CT may help to identify patients with potentially reversible forms of pericardial con‐ striction. In this group of patients, in addition to intensification of dialysis, anti‐inflammatory therapy should be considered to prevent the need for pericardiectomy. JRCD 2019; 4 (2): 47-50.","PeriodicalId":37488,"journal":{"name":"Journal of Rare Cardiovascular Diseases","volume":"54 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-04-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"83572751","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0