Idiopathic retroperitoneal fibrosis of the bladder

A patient is described with isolated idiopathic retroperitoneal fibrosis (IRPF) of the bladder causing overhydration due to postrenal oliguria. IRPF is known to spread to the bladder; restriction of the bladder, however, is extremely rare. The patient was successfully treated with corticosteroids. The potential role of corticosteroids in the management of IRPF is discussed.     

An elderly case of idiopathic retroperitoneal fibrosis]

An elderly case of idiopathic retroperitoneal fibrosis (IRPF) with bilateral ureteral obstruction was reported. A 74-year-old man was admitted with complaints of general fatigue and loss of appetite. An elastic hard mass with a smooth surface was palpated in the left upper quadrant of the abdomen. Abnormal ESR and CRP were noticed. Abdominal ultrasonography showed both a homoechoic mass which surrounded the aorta in the retroperitoneal space and bilateral hydronephrosis. Computed tomography revealed a retroperitoneal mass involving bilateral ureters. Magnetic resonance imaging demonstrated a mass with a sharp image and signal intensity. Furthermore the morphological relationship between the mass and the ureters and major vessels clearly indicated the characteristics of benign retroperitoneal fibrosis. Based upon the above findings, a diagnosis of IRPF was made. After confirming the histological diagnosis by biopsy, treatment consisting of bilateral ureterolysis, intra-abdominal transposition of ureters and oral administration of prednisolone was performed, resulting in a normalization of laboratory findings. Analysis of 86 previously cases of IRPF reported up to 1990 in Japan, revealed the peak age to be in the 7th decade with predominance in males (males: females = 57:29).     

IgG4‐related retroperitoneal fibrosis: a newly characterized disease

Retroperitoneal fibrosis (RPF) is a rare disease characterized by chronic, nonspecific inflammatory and sclerotic or fibrotic tissue in the periaortic or periiliac retroperitoneum that encases adjacent structures. There will be a series of clinical manifestations once the proliferated fibrous tissues encase the abdominal aorta, iliac arteries and urinary duct. RPF is generally divided into two types: idiopathic retroperitoneal fibrosis (IRPF) without identified pathogenesis, making up about two‐thirds of cases, and secondary retroperitoneal fibrosis. Recent studies on Immunoglobulin G4‐related disease (IgG4‐RD) reveal that abundant infiltration of IgG4 positive plasma cells is found in biopsies on the mass of RPF of some IRPF patients, which is identified as one spectrum of IgG4‐RD and is named IgG4‐related RPF. IgG4‐related RPF is often misdiagnosed as retroperitoneal visceral malignancy and is treated with surgery. In addition, because of its good response to glucocorticoid, early detection and treatment is important. We review the definition, epidemiology, clinical features, diagnostic criteria, treatment and prognosis of IgG4‐related RPF in this article to raise awareness of this newly characterized disease.     

Challenge to overcome: Nonstructural protein 5A-P32 deletion in direct-acting antiviral-based therapy for hepatitis C virus

Interferon(IFN)-based therapy for hepatitis C virus(HCV) infection has recently been replaced by IFNfree direct-acting antiviral(DAA)-based therapy, which has been established as a 1~(st) line therapy with high efficacy and tolerability due to its reasonable safety profile. Resistance-associated substitutions(RASs) have been a weakness of DAA-based therapy. For example, combination therapy with daclatasvir and asunaprevir(DCV/ASV) is less effective for HCV genotype 1-infected patients with Y93H as a nonstructural protein 5A RAS. However, the problem regarding RASs has been gradually overcome with the advent of recently developed DAAs, such as sofosbuvir-based regimens or combination therapy with glecaprevir and pibrentasvir. Despite the high efficiency of DAA-based therapy, some cases fail to achieve viral eradication. P32 deletion, an NS5A RAS, has been gradually noticed in patients with DCV/ASV failure. P32 deletion has been sporadically reported and the prevalence of this RAS has been considered to be low in patients with DCV/ASV failure. Thus, the picture of P32 deletion has not been fully evaluated. Importantly, currently-commercialized DAA-based combination therapy was not likely to be effective for patients with P32 deletion. Exploring and overcoming this RAS is essential for antiviral therapy for chronic hepatitis C.     

Acute renal failure and infectious mononucleosis

Renal failure is a rare complication of infectious mononucleosis, and is usually due to acute interstitial nephritis. Outcome has been variable with spontaneous recovery, death, and chronic renal failure being described. Only two patients have been reported as requiring dialysis. In some cases the aetiology of the interstitial nephritis has been open to doubt and may have been drug-induced. The case of a patient with acute renal failure associated with infectious mononucleosis and in whom interstitial nephritis appears to have been totally related to the underlying disease is described. There was an excellent response to treatment with high doses of methyl prednisolone given intravenously.     

Bioinspired Topographic Surface Modification of Biomaterials

Physical surface modification is an approach that has been investigated over the last decade to reduce bacterial adhesion and improve cell attachment to biomaterials. Many techniques have been reported to modify surfaces, including the use of natural sources as inspiration to fabricate topographies on artificial surfaces. Biomimetics is a tool to take advantage of nature to solve human problems. Physical surface modification using animal and vegetal topographies as inspiration to reduce bacterial adhesion and improve cell attachment has been investigated in the last years, and the results have been very promising. However, just a few animal and plant surfaces have been used to modify the surface of biomaterials with these objectives, and only a small number of bacterial species and cell types have been tested. The purpose of this review is to present the most current results on topographic surface modification using animal and plant surfaces as inspiration to modify the surface of biomedical materials with the objective of reducing bacterial adhesion and improving cell behavior.     

New Approaches and Understandings in the Growth of Cubic Silicon Carbide

In this review paper, several new approaches about the 3C-SiC growth are been presented. In fact, despite the long research activity on 3C-SiC, no devices with good electrical characteristics have been obtained due to the high defect density and high level of stress. To overcome these problems, two different approaches have been used in the last years. From one side, several compliance substrates have been used to try to reduce both the defects and stress, while from another side, the first bulk growth has been performed to try to improve the quality of this material with respect to the heteroepitaxial one. From all these studies, a new understanding of the material defects has been obtained, as well as regarding all the interactions between defects and several growth parameters. This new knowledge will be the basis to solve the main issue of the 3C-SiC growth and reach the goal to obtain a material with low defects and low stress that would allow for realizing devices with extremely interesting characteristics.     

Combined oral anticoagulants and antiplatelets: benefits and risks

Combined antiplatelet and anticoagulant therapy has been suggested for those clinical conditions in which conventional antithrombotic regimens have shown suboptimal efficacy, and in patients with indication for both: antiplatelet and anticoagulant therapy. Clinical trials aimed at assessing the clinical benefit of the association with respect to mono-therapy have been conducted in patients with atrial fibrillation, in patients with recent myocardial infarction, and in patients with prosthetic heart valves. Overall, a favorable benefit-risk profile of combined therapy in comparison to anticoagulant alone has been observed in patients with mechanical prosthetic heart valves and in those with coronary artery disease while no clear advantage has been shown in patients with atrial fibrillation. In almost all these studies, however, a higher risk of major bleeding has been observed in patients receiving combined therapy in comparison to patients receiving warfarin alone. Thus, a combined regimen of anticoagulant and antiplatelet therapy should be reserved for selected patients at high risk of thromboembolic events who have a low risk of bleeding.     

Sclerosing Cholangitis Associated with Autoimmune Hemolytic Anemia and Hyperthyroidism

A patient presented with autoimmune hemolytic anemia and sclerosing cholangitis. Six years later he was found to be hyperthyroid. Although sclerosing cholangitis has been associated with other autoimmune disorders, it has not been reported in association with either autoimmune hemolytic anemia or hyperthyroidism. Sclerosing cholangitis is a chronic fibrosing inflammatory disease process which may include parts or all of the biliary tree including the gallbladder. Although this disorder has been reported to occur with a variety of autoimmune disorders, it has not been previously associated with autoimmune hemolytic anemia and hyperthyroidism.     

Patient and technique survival after treatment shifts between CAPD and haemodialysis in a single centre

In the last decade, there has been a renewed interest in peritoneal dialysis and this modality has been proposed as a possible alternative to haemodialysis in the care of patients with end-stage renal disease. Attempts have been made to compare various aspects of these two modes of dialysis (4,6). Clinical trials have been performed particularly comparing CAPD with haemodialysis in the treatment of certain subgroups of patients, such as children or patients with diabetic nephropathy (1,5).     

Coagulation Factor IX in Normal and Haemophilia B Plasma

S ummary . Coagulation factor IX has been isolated in a partially purified form from normal human plasma. Studies have been performed of the coagulant properties of this factor, of its activation by contact product and subsequent reaction with factor VIII. A similar chromatographic procedure with DE AE cellulose has been used to study the plasma of a patient with haemophilia B. A fraction has been isolated with similar physico-chemical properties to normal factor IX but lacking coagulant activity.     

Colony stimulating factors

The genes for a number of growth factors that stimulate human hematopoietic and lymphoid cells in vitro have recently been cloned and recombinant molecules provided for clinical trials. For three of these (erythropoietin, G-CSF, and GM-CSF), phase I and II studies have been completed and promising results have been obtained. Of particular relevance to the field of bone marrow transplantation (BMT) has been the finding that G-CSF and GM-CSF could shorten the period of neutropenia in patients treated with chemotherapy, including regimens requiring BMT support. Doses of up to 240 micrograms/m2 of GM-CSF have been well tolerated and have increased the peripheral blood neutrophil count in a dose-dependent manner. At higher doses, eosinophils and monocytes were also increased. A continuous infusion over at least 2 h was found to be superior to bolus administration in terms of both efficacy and reduced side effects. These have usually been mild, but bone pain, headache, fatigue and elevated temperature have been encountered. The rise in neutrophil numbers shortly after initiating treatment with GM-CSF is probably due to neutrophil demargination. After a few days increased bone marrow cellularity has also been noted. In addition to these effects on cell numbers, enhancement of granulocyte and monocyte functions has been documented. However, a major concern with the use of G-CSF and GM-CSF in cancer patients, particularly those with hematopoietic malignancies, is the potential of these molecules to stimulate malignant cells.(ABSTRACT TRUNCATED AT 250 WORDS)     

Deletion of chromosome 20q in myelodysplasia can occur in a multipotent precursor of both myeloid cells and B cells

Deletions of the long arm of chromosome 20 are associated with several myeloid malignancies and, in particular, with myeloproliferative disorders and myelodysplastic syndromes (MDS). Together with deletions of chromosome 5q and chromosome 7q, chromosome 20q deletions have previously been thought to be restricted to myeloid cells in patients with MDS. We report here that deletion of chromosome 20q in MDS can arise in a multipotent precursor of both myeloid cells and B cells. Clonal Epstein-Barr virus (EBV)-transformed cell lines, both with and without a 20q deletion, have been isolated from a patient with MDS. Moreover, these cell lines have been shown to provide a useful physical mapping tool and have been used to confirm the interstitial nature of the 20q deletion. Microsatellite polymerase chain reaction (PCR) and PCR analysis of PGK gene methylation have been used to study highly purified populations of peripheral blood cells. The 20q deletion was detectable by microsatellite PCR in peripheral blood granulocytes and monocytes but not in B cells or T cells. Clonality of the different lineages followed the same pattern as the 20q deletion. This represents the first report in which a chromosome abnormality associated with MDS has been immortalized in an EBV-transformed lymphoblastoid cell line. Furthermore, our data show that patients with apparent myeloid restriction of a chromosome deletion may in fact have a disease arising in a multipotent cell with both myeloid and lymphoid potential.     

Chemiluminescence measurement of phagocytic activity of pulmonary macrophages in sarcoid alveolitis

The phagocytic activity of alveolar macrophages in a group of patients with stage I and stage II fresh sarcoidosis has been studied in comparison with a group of healthy volunteers. The phagocytic activity has been measured by means of chemiluminescent emission recording. While no difference in the emission of chemiluminescence has been detected in polymorphonuclear leukocytes of patients and control subjects, a greater emission of chemiluminescence has been noted in the alveolar macrophages of sarcoid patients. Such a difference is statistically significant; its possible relation to alveolar inflammation has been considered.     

Percutaneous approaches to mitral valve regurgitation

Mitral regurgitation is a common problem associated with significant morbidity and mortality. Mitral valve surgery has been the treatment of choice for symptomatic patients with severe mitral regurgitation or asymptomatic patients with high-risk clinical features. However, a significant number of patients remain untreated related mainly due to a projected high surgical risk. Therefore, alternative percutaneous treatments including indirect annuloplasty, which takes advantage of the coronary sinus, and direct annuloplasty have recently been explored. Most recently, promising results of the first randomized trial comparing conventional mitral valve surgery to percutaneous therapy with a clip creating a double orifice much like the surgical Alfieri approach have been presented. Finally, percutaneous mitral valve replacement in an animal model has been pursued. This review serves to familiarize the reader with some anatomical concepts and devices for percutaneous mitral repair.     

Arrêt cardiaque extrahospitalier et pandémie de la COVID-19

Since the appearance of the COVID-19 pandemic in 2020, the direct mortality related to COVID-19 infections has been monitored worldwide, with a daily count of the number of deaths due to COVID-19. Several measures have been undertaken in the societal and professional field, and the healthcare systems have been reorganized to limit the virus spread, and to cope with the surge of hospital admissions for COVID-19. Questions have been raised regarding the indirect effect of the pandemic, with uncertainties regarding the impact of delays in non-COVID diseases management, due to lockdown, postponement of non-urgent medical consultations and interventions, and decrease in screening. Sudden cardiac death could have been impacted by all those changes, and is generally a good surrogate of public health. In the current article, we review the impact of the COVID-19 pandemic on the epidemiology and outcome of sudden cardiac death.     

Recent advances in the treatment of the spondyloarthropathies

PURPOSE OF REVIEW: Recently, there has been renewed interest in the spondyloarthropathy family of chronic inflammatory rheumatic conditions, which has been fueled to a large extent by the biologic era. Over the period of the past 2 years in particular, there have been several notable advances. First, there have been a number of large, high-quality randomized controlled trials evaluating the tumor necrosis factor (TNF) blockers and conservative treatments such as physiotherapy and nonsteroidal anti-inflammatory drugs for use in spondyloarthropathy. This has paved the way for the development of better tools to assess outcome in these patients both in daily practice and in the context of clinical trials. This review uses a systematic approach to outline the most recent (within the last 2 years) and the most pertinent advances in the treatments of the spondyloarthropathies, with particular emphasis on ankylosing spondylitis and psoriatic arthritis. RECENT FINDINGS: Supervised group exercise programs maintain flexibility and posture in patients with ankylosing spondylitis, and spa therapy is a cost-effective treatment option in ankylosing spondylitis. Nonsteroidal anti-inflammatory drugs have a role in symptom modification and, more importantly, may prevent structural disease progression in patients with ankylosing spondylitis when administered continuously at a fixed dose. TNF blockers have been evaluated in a number of randomized controlled trials in ankylosing spondylitis and psoriatic arthritis and have been demonstrated to be safe and effective in the short-term management of these diseases. Longer-term trials are awaited with radiographic outcomes to comment on their disease-modifying properties and their long-term safety and efficacy profiles. SUMMARY: There has been renewed interest in the spondyloarthropathy family of disorders, with an explosion in the number of trials evaluating outcome with the TNF blockers. To date, no cure has been found for the disease, but these agents are emerging as the best therapeutic option available for patients with ankylosing spondylitis and psoriatic arthritis to date.     

How to proceed in Helicobacter pylori-positive chronic gastritis refractory to first- and second-line eradication therapy

Helicobacter pylori is a widespread disease causing most of the peptic ulcer diseases and low-grade mucosa-associated lymphoreticular tissue (MALT) lymphoma. Moreover, H. pylori is a proven environmental risk factor for gastric carcinoma and it has been recognized as a type 1 carcinogen factor. A combination of drugs has been proposed, using a proton pump inhibitor (PPI), amoxicillin, clarithromycin, metronidazole and tetracycline to treat the infection. Since 1996, according to the European guidelines, the first-line approach using PPI, amoxicillin and clarithromycin or metronidazole has been suggested. Seven days of quadruple therapy with PPI (or ranitidine), tetracycline, bismuth salts and metronidazole has been reserved as second-line treatment. To improve the eradication rate of the triple therapy, a different combination of the available antibiotics has been proposed, consisting of a 10-day sequential regimen. A second-line levofloxacin-amoxicillin-based triple therapy given for 10 days has been proposed, obtaining a high eradication rate, suggesting this regimen to be a suitable retreatment option in eradication failure. A third-line treatment with rifabutin-based regimen has been proposed.