Pub Date : 2020-01-27DOI: 10.20418/JRCD.VOL4NO3.394
Ioannis Boutsikos, M. Dova, Aristea Tsaroucha, D. Chatzis
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
{"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}
Pub Date : 2020-01-27DOI: 10.20418/JRCD.VOL4NO3.377
N. Oryshchyn, Y. Ivaniv, L. Kulyk, Oksana Predzemirska
Sinus of Valsalva aneurysm (SVA) is a rare congenital heart disease. A possible complication of SVA is rupture into a heart chamber, causing volume overload, which could lead to heart failure and the requirement for surgical correction. Transthoracic echocardiography is a readily available diagnostic tool for assessment, while other methods of cardiac imaging (cardiac CT and MRI) confirm the diagnosis and are necessary for preoperative assessment of the pathology. We present a very rare case of right sinus of Valsalva aneurysm dissecting the interventricular septum which ruptured into the right ventricle, causing volume overload of the right heart chambers and progressive heart failure. The diagnosis was established by transthoracic echocardiography and confirmed by ECG‐gated CT. Successful surgical correction resulted in regression of dilatation and dysfunction of heart chambers and to functional recovery of the patient. JRCD 2019; 4 (3): 51-54.
{"title":"Sinus of Valsalva aneurysm dissecting the inter ventricular septum with rupture into the right ven tricle (RCD code: I 1B.O)","authors":"N. Oryshchyn, Y. Ivaniv, L. Kulyk, Oksana Predzemirska","doi":"10.20418/JRCD.VOL4NO3.377","DOIUrl":"https://doi.org/10.20418/JRCD.VOL4NO3.377","url":null,"abstract":"Sinus of Valsalva aneurysm (SVA) is a rare congenital heart disease. A possible complication of SVA is rupture into a heart chamber, causing volume overload, which could lead to heart failure and the requirement for surgical correction. Transthoracic echocardiography is a readily available diagnostic tool for assessment, while other methods of cardiac imaging (cardiac CT and MRI) confirm the diagnosis and are necessary for preoperative assessment of the pathology. We present a very rare case of right sinus of Valsalva aneurysm dissecting the interventricular septum which ruptured into the right ventricle, causing volume overload of the right heart chambers and progressive heart failure. The diagnosis was established by transthoracic echocardiography and confirmed by ECG‐gated CT. Successful surgical correction resulted in regression of dilatation and dysfunction of heart chambers and to functional recovery of the patient. JRCD 2019; 4 (3): 51-54.","PeriodicalId":37488,"journal":{"name":"Journal of Rare Cardiovascular Diseases","volume":"146 1","pages":"51-54"},"PeriodicalIF":0.0,"publicationDate":"2020-01-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"80993533","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}
Pub Date : 2020-01-27DOI: 10.20418/JRCD.VOL4NO3.402
P. Podolec
{"title":"Journal of Rare Cardiovascular Diseases now included in Directory of Open Access Journals (DOAJ)!","authors":"P. Podolec","doi":"10.20418/JRCD.VOL4NO3.402","DOIUrl":"https://doi.org/10.20418/JRCD.VOL4NO3.402","url":null,"abstract":"","PeriodicalId":37488,"journal":{"name":"Journal of Rare Cardiovascular Diseases","volume":"62 1","pages":"33"},"PeriodicalIF":0.0,"publicationDate":"2020-01-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"88823880","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}
Pub Date : 2019-08-19DOI: 10.20418/JRCD.V4I2.379.G296
P. Podolec
In an original article featured in this issue entitled ‘Prognostic value of inflammatory markers in acute coronary syndrome in a population with premature cardiovascular disease (RCD code: VIII)’, the potential of inflammatory markers in the management of acute coronary syndromes in patients with premature car‐ diovascular disease is described. This study is a valu‐ able contribution to the body of evidence in the field of immune mechanisms in cardiovascular diseases.
{"title":"The potential role of the immune system and its modulation in rare diseases","authors":"P. Podolec","doi":"10.20418/JRCD.V4I2.379.G296","DOIUrl":"https://doi.org/10.20418/JRCD.V4I2.379.G296","url":null,"abstract":"In an original article featured in this issue entitled ‘Prognostic value of inflammatory markers in acute coronary syndrome in a population with premature cardiovascular disease (RCD code: VIII)’, the potential of inflammatory markers in the management of acute coronary syndromes in patients with premature car‐ diovascular disease is described. This study is a valu‐ able contribution to the body of evidence in the field of immune mechanisms in cardiovascular diseases.","PeriodicalId":37488,"journal":{"name":"Journal of Rare Cardiovascular Diseases","volume":"200 1","pages":"33"},"PeriodicalIF":0.0,"publicationDate":"2019-08-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"73283810","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}
Pub Date : 2019-08-09DOI: 10.20418/JRCD.VOL4NO2.373
A. Pura, Marta Wilk, M. Olszowska
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.
{"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}
Pub Date : 2019-08-06DOI: 10.20418/JRCD.VOL4NO2.370
A. Pytlewski, Mateusz Siwek, Leszek Drabik, Miłosz Delikat, Dawid Śmietana, E. Sobieraj, P. Iwaszczuk, A. Mazurek, W. Płazak
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.
{"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}
Pub Date : 2019-08-05DOI: 10.20418/JRCD.VOL4NO2.361
P. Yubbu, H. Chew, Y. Yakob, S. Lua, H. Samion
Long QT syndrome (LQTS) may be a cause of foetal bradyarrhythmia and an important cause of death in children with arrhythmia. We present the case of a patient of Kadazan Iban descent with LQTS. He was detected prenatally to have foetal 2:1 atrioventricular (AV) block and tetralogy of Fallot. His postnatal electrocardiogram revealed a functional 2:1 AV block with QTc interval of 690 ms. Dysmorphism and cutaneous syndactyly of both hands and feet pointed to a diagnosis of classical Timothy syndrome (TS) type 1. This diagnosis was confirmed molecularly with a heterozygous mutation c.1216G>A. p. (Gly406Arg) at exon 8A in the CACNA1C gene. To the best of our knowledge, this is the first reported case of TS in a Kadazan Iban child. JRCD 2019; 4 (2): 42-46.
{"title":"Foetal 2:1 atrioventricular block in a patient with Timothy syndrome (LQT8) (RCDD code: VI-1B-1.2)","authors":"P. Yubbu, H. Chew, Y. Yakob, S. Lua, H. Samion","doi":"10.20418/JRCD.VOL4NO2.361","DOIUrl":"https://doi.org/10.20418/JRCD.VOL4NO2.361","url":null,"abstract":"Long QT syndrome (LQTS) may be a cause of foetal bradyarrhythmia and an important cause of death in children with arrhythmia. We present the case of a patient of Kadazan Iban descent with LQTS. He was detected prenatally to have foetal 2:1 atrioventricular (AV) block and tetralogy of Fallot. His postnatal electrocardiogram revealed a functional 2:1 AV block with QTc interval of 690 ms. Dysmorphism and cutaneous syndactyly of both hands and feet pointed to a diagnosis of classical Timothy syndrome (TS) type 1. This diagnosis was confirmed molecularly with a heterozygous mutation c.1216G>A. p. (Gly406Arg) at exon 8A in the CACNA1C gene. To the best of our knowledge, this is the first reported case of TS in a Kadazan Iban child. JRCD 2019; 4 (2): 42-46.","PeriodicalId":37488,"journal":{"name":"Journal of Rare Cardiovascular Diseases","volume":"54 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-08-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"82128625","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}
Pub Date : 2019-07-31DOI: 10.20418/JRCD.VOL4NO2.371
A. Wileczek, M. Trawinska, S. Stec
We present the case of a young patient with dilated cardiomyopathy (DCM) and concomitant pulmonary tuberculosis (TB), emphasising the need for an interdisciplinary approach when considering underlying aetiology. A 36‐year‐old male was admitted to the hospital due to signs of acute heart failure (HF). After diagnostic work‐up, which included laboratory examination, echocardiography, and coronary angiography, a diagnosis of DCM was established and therapy was initiated. Despite optimal medical therapy for HF, the patient’s condition did not improve. During further diagnostic workup, pulmonary tuberculosis was identified. A significant reduction in HF signs and symptoms and improvement in left ventricular ejection fraction occurred only after TB was treated with 6 months of therapy. The presented case study illustrates the necessity to perform a complete diagnostic workup to identify reversible causes of DCM. JRCD 2019; 4 (2): 56-58.
{"title":"Partial recovery of left ventricular function in dilated cardiomyopathy as a result of tuberculosis treatment (RCD code: III-1B.9.o)","authors":"A. Wileczek, M. Trawinska, S. Stec","doi":"10.20418/JRCD.VOL4NO2.371","DOIUrl":"https://doi.org/10.20418/JRCD.VOL4NO2.371","url":null,"abstract":"We present the case of a young patient with dilated cardiomyopathy (DCM) and concomitant pulmonary tuberculosis (TB), emphasising the need for an interdisciplinary approach when considering underlying aetiology. A 36‐year‐old male was admitted to the hospital due to signs of acute heart failure (HF). After diagnostic work‐up, which included laboratory examination, echocardiography, and coronary angiography, a diagnosis of DCM was established and therapy was initiated. Despite optimal medical therapy for HF, the patient’s condition did not improve. During further diagnostic workup, pulmonary tuberculosis was identified. A significant reduction in HF signs and symptoms and improvement in left ventricular ejection fraction occurred only after TB was treated with 6 months of therapy. The presented case study illustrates the necessity to perform a complete diagnostic workup to identify reversible causes of DCM. JRCD 2019; 4 (2): 56-58.","PeriodicalId":37488,"journal":{"name":"Journal of Rare Cardiovascular Diseases","volume":"58 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-07-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"82279023","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}
Pub Date : 2019-05-08DOI: 10.20418/JRCD.VOL4NO2.363
Anna Prochwicz, E. Szczepanek, D. Krochmalczyk
Interferon alpha is a molecule associated with stimulation of immune system cells, resulting in an anti‐proliferative and immunomodulatory effect. It has been demonstrated that interferon reduces the number of platelets, leukocytes, and erythrocytes in patients suffering from chronic myeloproliferative diseases. In this paper, we present an overview of selected research evaluating the efficacy and adverse effects of various recombinant interferons used in the treatment of polycythaemia vera. We have analysed previously reported studies on the use of interferon. Interferon alfa‐2a was the first interferon approved for standard treatment of polycythaemia vera, while the next was pegylated interferon alfa‐2a. We also present recent results from studies on a newly modified molecule, ropeginterferon, a mono‐pegylated form of interferon alfa‐2b. Interferons reduce the number of phlebotomies required in patients with polycythaemia vera, accompanied by a resolution of typical disease symptoms. Treatment is well tolerated by the majority of patients. JRCD 2019; 4 (2): 34-36.
{"title":"Current views on the use of interferons in the treatment of polycythaemia vera (RCD code: VIII)","authors":"Anna Prochwicz, E. Szczepanek, D. Krochmalczyk","doi":"10.20418/JRCD.VOL4NO2.363","DOIUrl":"https://doi.org/10.20418/JRCD.VOL4NO2.363","url":null,"abstract":"Interferon alpha is a molecule associated with stimulation of immune system cells, resulting in an anti‐proliferative and immunomodulatory effect. It has been demonstrated that interferon reduces the number of platelets, leukocytes, and erythrocytes in patients suffering from chronic myeloproliferative diseases. In this paper, we present an overview of selected research evaluating the efficacy and adverse effects of various recombinant interferons used in the treatment of polycythaemia vera. We have analysed previously reported studies on the use of interferon. Interferon alfa‐2a was the first interferon approved for standard treatment of polycythaemia vera, while the next was pegylated interferon alfa‐2a. We also present recent results from studies on a newly modified molecule, ropeginterferon, a mono‐pegylated form of interferon alfa‐2b. Interferons reduce the number of phlebotomies required in patients with polycythaemia vera, accompanied by a resolution of typical disease symptoms. Treatment is well tolerated by the majority of patients. JRCD 2019; 4 (2): 34-36.","PeriodicalId":37488,"journal":{"name":"Journal of Rare Cardiovascular Diseases","volume":"72 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-05-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"85465194","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}
Pub Date : 2019-04-25DOI: 10.20418/JRCD.VOL4NO2.350
A. Wojtkowska, A. Tomaszewski, Magdalena Zakościelna, E. Czekajska-Chehab, A. Wysokiński
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.
{"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}
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