Journal of Rare Cardiovascular Diseases最新文献_第4页

Comparison of platelet count reduction in patients with essential thrombocythaemia treated with hydroxyurea and thromboreductin. Single centre experience (RCD code: VIII) 羟脲和降凝素治疗原发性血小板血症患者血小板计数降低的比较。单中心体验(RCD代码:VIII)

Journal of Rare Cardiovascular Diseases Pub Date : 2019-03-01 DOI: 10.20418/JRCD.VOL3NO8.362

Anna Prochwicz, S. Fornagiel, K. Krawczyk, D. Krochmalczyk

{"title":"Comparison of platelet count reduction in patients with essential thrombocythaemia treated with hydroxyurea and thromboreductin. Single centre experience (RCD code: VIII)","authors":"Anna Prochwicz, S. Fornagiel, K. Krawczyk, D. Krochmalczyk","doi":"10.20418/JRCD.VOL3NO8.362","DOIUrl":"https://doi.org/10.20418/JRCD.VOL3NO8.362","url":null,"abstract":"Essential thrombocythemia is one of the Ph-negative myeloproliferative neoplasms treated with hydroxyurea. An alternative strategy may be a therapy with thromboreductin. The aim of the study was to compare the effectiveness of hydroxyurea and thromboreductin treatment, defined by a decrease in the platelet count. The study group consisted of 154 patients with essential thrombocythemia diag‐ nosed and treated at the Outpatient Clinic of Hematology in Krakow, Poland between 1995 and 2016. Patients were included in the study at the start of cytoreductive treatment. 102 patients was treated with hydroxyurea and 52 patients treated with thromboreducin. We set the limit values for the number of platelets on levels : <800 x 10 9 /L, <600 x 10 9 /L, <450 x 10 9 /L and <350 x 10 9 /L. Afterwards, the analysis of the time required to achieve each point was performed. A comparison of hydroxyurea and thromboreductin groups showed that the number of platelets at the beginning of therapy was significantly lower in patients treated with hydroxyurea. Platelets value in the last control was significantly lower in patients treated with thromboreductin than hydroxyurea. The change in total platelet count over the time was significantly higher in the thromboreductin group. Patients treated with thromboreductin had a faster platelets reduction lower than 450 x10 9 /L. Tromboreductin is effective in reducing the number of platelets in patients with resistant essential thrombocythemia or intolerant of hydroxyurea regardless of age. JRCD 2018; 3 (8): 266–270","PeriodicalId":37488,"journal":{"name":"Journal of Rare Cardiovascular Diseases","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"89634674","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

Heart broken by a mosquito; an unusual case of Takotsubo cardiomyopathy (RCD code: III‐5B) 被蚊子咬碎的心;罕见Takotsubo型心肌病1例(RCD代码:III‐5B)

Journal of Rare Cardiovascular Diseases Pub Date : 2019-01-31 DOI: 10.20418/JRCD.VOL3NO8.336

J. Tai, Anam Haider, B. Hussain

引用次数: 0

Non‐ruptured symptomatic splenic artery aneurysm (RCD code: I-1D.1) 未破裂的症状性脾动脉瘤(RCD代码:I-1D.1)

Journal of Rare Cardiovascular Diseases Pub Date : 2019-01-17 DOI: 10.20418/JRCD.VOL3NO8.348

Hossein Farsavian, M. Davoodi, M. Bonyadi, A. Hessami, A. Shamshirian, S. Hashemi, Sina Nazemi, Ashkan Piranviseh

引用次数: 0

Left ventricular non‐compaction – diagnostic challenges (RCD code: III‐5A.1.o) 左心室非压实-诊断挑战(RCD代码:III‐5a . 1.0)

Journal of Rare Cardiovascular Diseases Pub Date : 2019-01-15 DOI: 10.20418/JRCD.VOL3NO8.331

P. Orda, A. Krivickienė, T. Lapinskas, E. Ereminienė

引用次数: 0

Safety of pulmonary vein isolation in atrial fibrillation patients treated with dabigatran when idarucizumab is available (RCDD code: VIII) 依达鲁珠单抗可用时达比加群房颤患者肺静脉隔离的安全性(RCDD代码:VIII)

Journal of Rare Cardiovascular Diseases Pub Date : 2019-01-15 DOI: 10.20418/JRCD.VOL3NO8.337

E. Koźluk, Dariusz Rodkiewicz, A. Piątkowska, P. Matusik, G. Opolski

{"title":"Safety of pulmonary vein isolation in atrial fibrillation patients treated with dabigatran when idarucizumab is available (RCDD code: VIII)","authors":"E. Koźluk, Dariusz Rodkiewicz, A. Piątkowska, P. Matusik, G. Opolski","doi":"10.20418/JRCD.VOL3NO8.337","DOIUrl":"https://doi.org/10.20418/JRCD.VOL3NO8.337","url":null,"abstract":"Patients with atrial fibrillation (AF) are at increased risk of stroke and systemic thromboembolism and prevention of such episodes is ensured by choosing appropriate anticoagulation. In paroxysmal drug‐refractory AF, catheter ablation is the recommended choice of treatment. The decision on whether to stop administration of oral anticoagulant before catheter ablation procedures is often unclear. We present the case of a 67‐year‐old hypertensive woman with a 5‐year history of symptomatic, drug‐refractory paroxysmal AF, who was admitted for pulmonary vein isolation (PVI) and was anticoagulated with dabigatran. After successful transseptal puncture, an intravenous injection of 10 000 units of heparin was administered. Radiofrequency ablation was initiated at the left pulmonary trunk. After the second application of radiofrequency ablation, a drop in arterial blood pressure to 70/50 mmHg was observed. Urgent echocardiography revealed the presence of fluid within the epicardial surface of the left ventricular apex up to 19 mm, behind the right ventricle and right atrium up to 11 mm. Subsequently, all catheters were removed from the left atrium, and 50 mg of protamine sulfate, dopamine, and intravenous fluids were immediately administered. Idarucizumab was urgently delivered to the catheterisation laboratory and was available during patient hospitalisation in the intensive care unit. However, prior to patient discharge, echocardiography revealed only a trace amount of fluid in the pericardium and the use of idarucizumab was not indicated. Interruption of anticoagulation treatment with dabigatran before ablation is not required. Idarucizumab increases the safety of PVI in patients treated with dabigatran. JRCD 2018; 3 (8): 281–283","PeriodicalId":37488,"journal":{"name":"Journal of Rare Cardiovascular Diseases","volume":"26 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-01-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"90697715","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

Five years of Journal of Rare Cardiovascular Diseases 五年的《罕见心血管疾病杂志

Journal of Rare Cardiovascular Diseases Pub Date : 2018-12-05 DOI: 10.20418/JRCD.VOL0NO0.355

P. Podolec

引用次数: 0

Myocardial infarction in Fabry disease – misfortune or companion? Case report and review of the literature (RCD code: III‑3B.2) 法布里病心肌梗死——不幸还是伴生?病例报告和文献回顾(RCD代码:III‑3B.2)

Journal of Rare Cardiovascular Diseases Pub Date : 2018-08-21 DOI: 10.20418/jrcd.vol3no7.335

J. Chmiel, M. Skubera, J. Bednarek, Klaudia Knap, Marta Swarowska Skuza, Stanisława Bazan ‐ Socha, A. Mazurek, L. Tomkiewicz-Pajak, M. Olszowska, P. Podolec, P. Musialek

{"title":"Myocardial infarction in Fabry disease – misfortune or companion? Case report and review of the literature (RCD code: III‑3B.2)","authors":"J. Chmiel, M. Skubera, J. Bednarek, Klaudia Knap, Marta Swarowska Skuza, Stanisława Bazan ‐ Socha, A. Mazurek, L. Tomkiewicz-Pajak, M. Olszowska, P. Podolec, P. Musialek","doi":"10.20418/jrcd.vol3no7.335","DOIUrl":"https://doi.org/10.20418/jrcd.vol3no7.335","url":null,"abstract":"We discuss a 56‑year‑old man with Fabry disease (FD), a genetic X‑linked glycolipid storage disorder. The patient presented at the Emergency Room in a local hospital due to tachycardia‑associated chest pain, which had occurred occasionally in the past, but on that occasion was long‑lasting (>12h) and distressing. The patient had been diagnosed with FD at the age of 42. He presented a range of symptoms characteristic for the condition, including hypertrophic cardiac myopathy with impaired left ventricular relaxation, angiokeratomas, cornea verticillata, hypohydrosis and acroparesthesia. Residual alpha‑galactosidase A activity at diagnosis was ≈3%. The Enzyme Replacement Therapy (ERT) with the agalsidase alpha was induced. A year later pacemaker implantation was performed due to sick sinus syndrome with symptomatic, severe episodes of bradycardia. The initial diagnosis was tachycardia‑associated chest pain with troponin release in the context of FD left ventricular hypertrophy. However, a decision was made to perform an urgent angiographic evaluation to exclude coronary pathology as a potential factor in the clinical picture. Coronary angiography showed a critical, flow-limiting, stenosis of the left anterior descending artery (LAD) which changed the initial type 2 myocardial infarction (MI) diagnosis to the type 1 MI. Percutaneous stent‑assisted treatment was performed with an optimal angiographic and clinical outcome. However, 5 days later the patient developed a minor left hemispheric ischaemic stroke. In conclusion, the clinical course of a rare pathology such as FD may be importantly complicated by other (more common) pathologies. Physicians, in their diagnostic and therapeutic decision‑making, need to be open to thinking beyond the patient label. JRCD 2018; 3 (7): 246–252","PeriodicalId":37488,"journal":{"name":"Journal of Rare Cardiovascular Diseases","volume":"21 1","pages":"246"},"PeriodicalIF":0.0,"publicationDate":"2018-08-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"82226748","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

JRCD is now an open‑access journal and also accepts high‑quality papers beyond the field of rare cardiovascular diseases and disorders JRCD现在是一个开放获取期刊，也接受罕见心血管疾病和疾病领域以外的高质量论文

Journal of Rare Cardiovascular Diseases Pub Date : 2018-08-21 DOI: 10.20418/JRCD.VOL3NO7.338

P. Podolec

引用次数: 0

Clinical Classification of Rare Cardiovascular Diseases and Disorders: 2018 Update 罕见心血管疾病和障碍临床分类:2018年更新

Journal of Rare Cardiovascular Diseases Pub Date : 2018-08-21 DOI: 10.20418/JRCD.VOL3NO7.330

P. Podolec, G. Kopeć, P. Rubis, J. Stępniewski, J. Podolec, M. Komar, L. Tomkiewicz-Pajak, A. Leśniak‑Sobelga, A. Kabłak-Ziembicka, P. Matusik

{"title":"Clinical Classification of Rare Cardiovascular Diseases and Disorders: 2018 Update","authors":"P. Podolec, G. Kopeć, P. Rubis, J. Stępniewski, J. Podolec, M. Komar, L. Tomkiewicz-Pajak, A. Leśniak‑Sobelga, A. Kabłak-Ziembicka, P. Matusik","doi":"10.20418/JRCD.VOL3NO7.330","DOIUrl":"https://doi.org/10.20418/JRCD.VOL3NO7.330","url":null,"abstract":"Rare diseases and disorders constitute important clinical problems. There are many concerns among physicians while planning the diagnostic and treatment process of such a heterogenous group of patients. These concerns arise not only from the rarity of cases, but also from multiple gaps in knowledge on the management of patients with rare diseases and disorders. The commonly accepted prevalence of rare diseases and disorders is 1 per 2 000 in the general population or less. Incidental prevalence and multiplicity of comorbidities result in an inability to gather enough experience at any single centre. Thus, cooperation and the exchange of ideas is important for the management of patients with rare diseases. Classification of rare cardiovascular diseases and disorders (RCDD) is crucial for expanding knowledge in the field of RCDD. It consists of an overview of RCDD, facilitates clinical approaches to patients and makes the creation of registries and databases easier. We hope that the updated RCDD classification will aid medical practice through the contribution to progress in diagnostics and therapy. It also serves as a summary of scientific achievements in the field of RCDD. Without the grouping of specific disorders, it is very difficult to create diagnostic and therapeutic algorithms. The Classification of RCDD was published for the first time in the Journal of Rare Cardiovascular Diseases (JRCD) in 2013 [1]. RCDD classification was discussed during the 2013 European Society of Cardiology Congress held in Amsterdam (www.crcd.eu/?p=2800) and in international journals, including a recent publication of the European Heart Journal [2, 3]. Clinical classification of RCDD takes into account major clinical symptoms and pathologies and is based on common clinical and/or anatomical features.","PeriodicalId":37488,"journal":{"name":"Journal of Rare Cardiovascular Diseases","volume":"7 1","pages":"230"},"PeriodicalIF":0.0,"publicationDate":"2018-08-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"89270852","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: 9

Spontaneous pneumothorax (RCD code: VIII) 自发性气胸(RCD代码:VIII)

Journal of Rare Cardiovascular Diseases Pub Date : 2018-06-25 DOI: 10.20418/JRCD.VOL3NO7.329

Marcin Kunecki, D. Gałuszka, Adrian Rybski, W. Płazak

引用次数: 0