Search results for "Overlap syndrome"

Systematic ajmaline challenge in patients with long QT 3 syndrome caused by the most common mutation: a multicentre study

2016

Aims Overlap syndromes of long QT 3 syndrome (LQT3) and the Brugada syndrome (BrS) have been reported. Identification of patients with an overlapping phenotype is crucial before initiation of Class I antiarrhythmic drugs for LQT3. Aim of the present study was to elucidate the yield of ajmaline challenge in unmasking the Brugada phenotype in patients with LQT3 caused by the most common mutation, SCN5A-E1784K. Methods and results Consecutive families in tertiary referral centres diagnosed with LQT3 caused by SCN5A-E1784K were included in the study. Besides routine clinical work-up, ajmaline challenge was performed after informed consent. A total of 23 subjects (11 female, mean age 27 ± 14 yea…

Predictive Scores in Primary Biliary Cirrhosis

2015

GOALS The aim of this study was to assess the long-term outcome of primary biliary cirrhosis (PBC) patients and to test the clinical value of various outcome models, such as the Mayo Risk Score (MRS), in a large single-center cohort in Germany. BACKGROUND PBC is a chronic autoimmune liver disease with a female gender predominance and a peak incidence in the fifth decade of life. PBC is characterized by portal inflammation and immune-mediated destruction of intrahepatic bile ducts in liver histology and the presence of antimitochondrial antibodies in the serum of nearly 95% of patients. In 5% to 20% of patients an overlap syndrome with autoimmune hepatitis (AIH) is diagnosed. Ursodeoxycholic…

Evidence for an overlap syndrome of autoimmune hepatitis and primary sclerosing cholangitis.

1996

Abstract Background/Aims: Autoimmune hepatitis, primary biliary cirrhosis and primary sclerosing cholangitis are chronic liver diseases with probable autoimmune background. Overlapping features have been described for primary biliary cirrhosis and autoimmune hepatitis. In contrast, there have been only a few case reports on an overlap of autoimmune hepatitis and primary sclerosing cholangitis. Methods: We describe three male patients with clinical and histological overlapping features of primary sclerosing cholangitis and autoimmune hepatitis. Results: All initially asymptomatic patients had elevated levels of aminotransferases, alkaline phosphatase, γ-glutamyltranspeptidase and IgG. Anti-n…

Intrahepatic IgG/IgM plasma cells ratio helps in classifying autoimmune liver diseases.

2010

Abstract Background/Aim Plasma cells infiltrate in the liver is a prototype lesion of autoimmune liver diseases. The possible role of plasma cells isotyping (IgM and IgG) in the liver in the diagnostic definition of autoimmune liver disease, and particularly in variant syndromes such as autoimmune cholangitis and the primary biliary cirrhosis/autoimmune hepatitis overlap syndrome, is less defined. Methods We analysed the clinical, serological and histological features of 83 patients with autoimmune liver disease (40 primary biliary cirrhosis, 20 autoimmune hepatitis, 13 primary sclerosing cholangitis, 4 autoimmune cholangitis and 6 overlap syndrome) compared to 34 patients with chronic hepa…

Characterization of the overlap syndrome of primary biliary cirrhosis (PBC) and autoimmune hepatitis: Evidence for it being a hepatitic form of PBC i…

1999

Some patients with autoimmune liver disease present with a clinical and/or histological picture showing characteristic findings of both autoimmune hepatitis (AIH) and primary biliary cirrhosis (PBC). Various names, mostly overlap syndrome, have been used to describe these cases, which have thus far not been more closely characterized. The aim of this study was the comparison of 20 patients with overlapping features to representative patients considered suffering from typical AIH or typical PBC (20 patients in each group). We found these patients to indeed show a very mixed picture of both conditions biochemically, serologically, and histologically. However, closer analysis suggested that al…

15-years mortality in asthma-COPD overlap syndrome (ACOS) elderly population

2015

Background: The coexistence of asthma and chronic obstructive pulmonary disease (Asthma-COPD Overlap Syndrome: ACOS) is increasingly recognized. Data about the prevalence of ACOS in the elderly and its prognostic implications are unknown. Aim: To evaluate the long-term mortality of ACOS in an elderly population. Methods: 1970 subjects, aged >65 yrs, were enrolled in the population-based SA.R.A. Study. ACOS was defined as: not fully reversible airflow limitation + a) history of asthma or b) very positive bronchodilator test (increase in FEV1 ≥15% and ≥400 ml) + atopy + weezing. The prevalence of ACOS and 15-years mortality rates were assessed in 1063 subjects with complete clinical, lung fun…

ASTHMA–COPD OVERLAP SYNDROME (ACOS): PREVALENZA, QUALITÀ DI VITA CORRELATA ALLO STATO DI SALUTE E IMPLICAZIONI PROGNOSTICHE DI UNA NUOVA ENTITÀ NOSOL…

Asthma control in elderly asthmatics. An Italian observational study

2014

SummaryBackgroundThe exponential increase of individuals aged >64 yrs is expected to impact the burden of asthma. We aimed to explore the level of asthma control in elderly subjects, and factors influencing it.MethodsA multicenter observational study was performed on consecutive patients >64 years old with a documented physician-diagnosis of asthma. Sixteen Italian centers were involved in this 6-month project.FindingsA total of 350 patients were enrolled in the study. More than one-third of elderly asthmatic patients, despite receiving GINA step 3–4 antiasthmatic therapy, had an Asthma Control Test score ≤19, with a quarter experiencing at least one severe asthma exacerbation in the previo…

Metabolic Syndrome in Patients with Coexistent Obstructive Sleep Apnea Syndrome and Chronic Obstructive Pulmonary Disease (Overlap Syndrome).

2020

Background: Evidence suggests that metabolic syndrome (MetS) is highly prevalent in patients with obstructive sleep apnea syndrome (OSAS) and chronic obstructive pulmonary disease (COPD). However, ...

Fifteen-year mortality of patients with asthma-COPD overlap syndrome

2016

Abstract Background The coexistence of asthma and chronic obstructive pulmonary disease (asthma–COPD overlap syndrome: ACOS) is increasingly recognized but data about its prevalence and long-term mortality are needed. Methods Prevalence of ACOS and 15-year mortality rates were assessed in 1065 subjects aged > 65 years, enrolled in the SA.R.A. study, with complete clinical, lung functional and follow-up data. Physical performance, disease-related disability, and health-related quality of life (HRQL) were also evaluated. Results ACOS was found in 11.1% of subjects (29.4% of those previously diagnosed with COPD and 19.7% of those with asthma). ACOS was positively associated with impaired physi…