Primary biliary cirrhosis

Primary biliary cirrhosis (PBC) is a chronic and slowly progressive cholestatic liver disease of autoimmune etiology characterized by injury of the intrahepatic bile ducts that may eventually lead to liver failure. Affected individuals are usually in their fifth to seventh decades of life at time of diagnosis, and 90% are women. Annual incidence is estimated between 0.7 and 49 cases per million-population and prevalence between 6.7 and 940 cases per million-population (depending on age and sex). The majority of patients are asymptomatic at diagnosis, however, some patients present with symptoms of fatigue and/or pruritus. Patients may even present with ascites, hepatic encephalopathy and/or esophageal variceal hemorrhage. PBC is associated with other autoimmune diseases such as Sjogren's syndrome, scleroderma, Raynaud's phenomenon and CREST syndrome and is regarded as an organ specific autoimmune disease. Genetic susceptibility as a predisposing factor for PBC has been suggested. Environmental factors may have potential causative role (infection, chemicals, smoking). Diagnosis is based on a combination of clinical features, abnormal liver biochemical pattern in a cholestatic picture persisting for more than six months and presence of detectable antimitochondrial antibodies (AMA) in serum. All AMA negative patients with cholestatic liver disease should be carefully evaluated with cholangiography and liver biopsy. Ursodeoxycholic acid (UDCA) is the only currently known medication that can slow the disease progression. Patients, particularly those who start UDCA treatment at early-stage disease and who respond in terms of improvement of the liver biochemistry, have a good prognosis. Liver transplantation is usually an option for patients with liver failure and the outcome is 70% survival at 7 years. Recently, animal models have been discovered that may provide a new insight into the pathogenesis of this disease and facilitate appreciation for novel treatment in PBC

Primary biliary cirrhosis

Primary biliary cirrhosis (PBC) is an immune-mediated chronic cholestatic liver disease with a slowly progressive course. Without treatment, most patients eventually develop fibrosis and cirrhosis of the liver and may need liver transplantation in the late stage of disease. PBC primarily affects women (female preponderance 9–10:1) with a prevalence of up to 1 in 1,000 women over 40 years of age. Common symptoms of the disease are fatigue and pruritus, but most patients are asymptomatic at first presentation. The diagnosis is based on sustained elevation of serum markers of cholestasis, i.e., alkaline phosphatase and gamma-glutamyl transferase, and the presence of serum antimitochondrial antibodies directed against the E2 subunit of the pyruvate dehydrogenase complex. Histologically, PBC is characterized by florid bile duct lesions with damage to biliary epithelial cells, an often dense portal inflammatory infiltrate and progressive loss of small intrahepatic bile ducts. Although the insight into pathogenetic aspects of PBC has grown enormously during the recent decade and numerous genetic, environmental, and infectious factors have been disclosed which may contribute to the development of PBC, the precise pathogenesis remains enigmatic. Ursodeoxycholic acid (UDCA) is currently the only FDA-approved medical treatment for PBC. When administered at adequate doses of 13–15 mg/kg/day, up to two out of three patients with PBC may have a normal life expectancy without additional therapeutic measures. The mode of action of UDCA is still under discussion, but stimulation of impaired hepatocellular and cholangiocellular secretion, detoxification of bile, and antiapoptotic effects may represent key mechanisms. One out of three patients does not adequately respond to UDCA therapy and may need additional medical therapy and/or liver transplantation. This review summarizes current knowledge on the clinical, diagnostic, pathogenetic, and therapeutic aspects of PBC

A Case of Recurrent Secondary Aortoenteric Fistula 4 Months after Surgery Treated by Endovascular Coiling of the Aortic Stump and Bilateral Chimney Stent Grafts to Renal Arteries

The treatment of recurrent aortoenteric fistula (AEF) previously repaired by surgery is challenging, with a high mortality rate. Open repair is often limited by “hostile abdomen,” while endovascular treatment is difficult when the distance between the aortic stump and the origin of the renal arteries is short, with high risk of their occlusion. We describe a recurrent AEF repaired by surgery 4 months earlier, treated by endovascular coiling of the aortic stump after deployment of 2 renal artery stent grafts with the chimney technique

Predictors of the multiwire technique use in carotid artery stenting

Objectives: The multiwire technique (MWT) in carotid artery stenting (CAS), characterized by the use of more than one guidewire to support guiding catheter in patients with hostile neck anatomy, increases procedural time, enhancing the risk of periprocedural stroke. The aim of the present study was to identify which factors are predictors of the MWT use in CAS, in order to stratify patients with longer procedure at potential higher risk of periprocedural stroke.Methods: The study retrospectively included patients who underwent CAS for stenotic plaque between January 2015 and December 2019. Exclusion criteria was incomplete clinical data. For each patients were registered clinical data, main aortic arch and supra-aortic vessel anatomical features, carotid plaque characteristics, and procedural details. The sample was divided in two group on the basis of the number of guides used during the stenting procedure: one guide (standard technique, ST) or more than one guidewire (MWT) to support the guiding catheter. Differences between groups were tested by Chi-square text or Fisher's exact test and Mann-Whitney U test. Logistic regression analysis was used to identify predictors for the use of the MWT. The area under the ROC (AUC) curve was used to assess performance of the model to predict the use of the multiwire technique.Results: The final sample included 146 of the 204 (71%) patients who underwent CAS during the study period. The median age of the patients was 79 years (IQR 71-83 years) with 47/146 (32%) females. CAS was performed with MWT in 17/146 (12%) of the cases. MWT was used more likely in patients with aortic arch type II or III as compared to ST (71% vs 37%, P = 0.02) while plaques with heavy concentric calcifications were more frequent in ST as compared to MWT (38% vs 12%, P = 0.03). At multivariable analysis aortic arch type II or III (OR 5.08, 95% CI 1.48-17.93, P < 0.01), plaque stenosis > 79% (OR 4.13, 95% CI 1.03-16.61, P = 0.04), and plaque heavy concentric calcifications (OR 0.19, 95% CI 0.04-0.94, P = 0.04) were independent predictors of MWT use. The model showed an AUC of 0.827 (95% CI 0.756-0.884) for the prediction of the MWT use during CAS.Conclusions: Aortic arch type II or III, carotid plaque with stenosis higher than 79% of the lumen or without heavy concentric calcifications were predictors for the use of the MWT during CAS. These features should be considered during planning of CAS as hallmark of vascular stiffness and therefore of higher procedure complexity

Spyglass percutaneous transhepatic lithotripsy of symptomatic recurrent lithiasis of the intrahepatic bile duct with distal stenosis

In patients with symptomatic intrahepatic biliary lithiasis, the treatment is usually discussed by a multidisciplinary team. Although hepatectomy is considered as definitive treatment, when lobar atrophy is absent, endoscopic procedures are frequently proposed as first-line treatment due the low invasiveness and for sparing liver parenchyma. Percutaneous route is used in case of peroral approach failure. We present a case of recurrent symptomatic intrahepatic biliary lithiasis of the right posterior hepatic duct, sustained by downstream biliary stenosis. Peroral cholangioscopy failed to visualize the stone for the accompanying stenosis. Thus, the patient was successfully treated with percutaneous transhepatic lithotripsy performed with Spyglass direct visualization system II (Boston Scientific Inc., Natick, Massachusetts, USA). During the procedure, the biopsy of the biliary stenosis revealed fibrosis, which was treated by cholangioplasty with cutting balloon. After 15 months, the patient is asymptomatic, with moderate residual stenosis in absence of calculi at follow-up magnetic resonance cholangiography

Qualitative and quantitative chest CT parameters as predictors of specific mortality in COVID-19 patients

Purpose: To test the association between death and both qualitative and quantitative CT parameters obtained visually and by software in coronavirus disease (COVID-19) early outbreak. Methods: The study analyzed retrospectively patients underwent chest CT at hospital admission for COVID-19 pneumonia suspicion, between February 21 and March 6, 2020. CT was performed in case of hypoxemia or moderate-to-severe dyspnea. CT scans were analyzed for quantitative and qualitative features obtained visually and by software. Cox proportional hazards regression analysis examined the association between variables and overall survival (OS). Three models were built for stratification of mortality risk: clinical, clinical/visual CT evaluation, and clinical/software-based CT assessment. AUC for each model was used to assess performance in predicting death. Results: The study included 248 patients (70% males, median age 68 years). Death occurred in 78/248 (32%) patients. Visual pneumonia extent > 40% (HR 2.15, 95% CI 1.2–3.85, P = 0.01), %high attenuation area – 700 HU > 35% (HR 2.17, 95% CI 1.2–3.94, P = 0.01), exudative consolidations (HR 2.85–2.93, 95% CI 1.61–5.05/1.66–5.16, P < 0.001), visual CAC score > 1 (HR 2.76–3.32, 95% CI 1.4–5.45/1.71–6.46, P < 0.01/P < 0.001), and CT classified as COVID-19 and other disease (HR 1.92–2.03, 95% CI 1.01–3.67/1.06–3.9, P = 0.04/P = 0.03) were significantly associated with shorter OS. Models including CT parameters (AUC 0.911–0.913, 95% CI 0.873–0.95/0.875–0.952) were better predictors of death as compared to clinical model (AUC 0.869, 95% CI 0.816–0.922; P = 0.04 for both models). Conclusions: In COVID-19 patients, qualitative and quantitative chest CT parameters obtained visually or by software are predictors of mortality. Predictive models including CT metrics were better predictors of death in comparison to clinical model