Noninvasive Assessment of portal Hypertension in Patients With Primary Biliary Cholangitis is Affected by Severity of Cholestasis.

Background & Aims: Noninvasive tests (NITs) for ruling-out clinical significant portal hypertension (CSPH) and high-risk varices (HRVs) in patients with primary biliary cholangitis (PBC) and compensated advanced chronic liver disease (cACLD) are lacking. We evaluated NITs in these patients and the influence of cholestasis on their performance.

Methods: Consecutive patients from the "Italian PBC registry" and 2 United Kingdom large-volume PBC referral centers with upper endoscopy within 6 months from biochemical evaluation and transient elastography were included.

Pathophysiology, Functional Assessment and Prognostic Implications of Nutritional Disorders in Systemic Amyloidosis.

Gastrointestinal involvement is a common clinical feature of patients with systemic amyloidosis. This condition is responsible for invalidating gastrointestinal symptoms, a significant macro and micronutrient deficit, and is a marker of disease severity. Gastrointestinal involvement should be actively sought in patients with systemic amyloidosis, while its diagnosis is challenging in patients with isolated gastrointestinal symptoms.

An Overview of Molecular Mechanisms in Fabry Disease.

Fabry disease (FD) (OMIM #301500) is a rare genetic lysosomal storage disorder (LSD). LSDs are characterized by inappropriate lipid accumulation in lysosomes due to specific enzyme deficiencies. In FD, the defective enzyme is α-galactosidase A (α-Gal A), which is due to a mutation in the gene on the X chromosome.

The Role of New Imaging Technologies in the Diagnosis of Cardiac Amyloidosis.

Cardiac amyloidosis is an infiltrative disorder caused by transthyretin or immunoglobulin free light-chain deposition, which determines clinical disease with similar phenotype but different time course, prognosis and therapy. Multimodality imaging is the cornerstone for disease diagnosis and management. Multimodality imaging has revolutionized the approach to the disease favoring its awareness and simplifying its diagnosis, especially in ATTR cardiac amyloidosis.

Diagnosis and Management of Cardiovascular Involvement in Fabry Disease.

Fabry disease (FD, OMIM 301500) is an X-linked lysosomal storage disease caused by pathogenic variants in the GLA gene. Cardiac involvement is common in FD and is responsible for impaired quality of life and premature death. The classic cardiac involvement is a nonobstructive form of hypertrophic cardiomyopathy, usually manifesting as concentric left ventricular hypertrophy, with subsequent arrhythmogenic intramural fibrosis.

Impact of Post-Procedural Change in Left Ventricle Systolic Function on Survival after Percutaneous Edge-to-Edge Mitral Valve Repair.

Objectives: To investigate how the changes of left ventricle ejection fraction (LVEF) between admission and discharge affected the long-term outcome in patients who underwent percutaneous edge-to-edge mitral valve repair for secondary mitral regurgitation.

Background: An acute impairment of LVEF after surgical repair of mitral regurgitation, known as afterload mismatch, has been associated with increased all-cause mortality. Afterload mismatch after percutaneous edge-to-edge mitral valve repair has been postulated to be a transient phenomenon.

Renal tubular function by lithium clearance in liver cirrhosis.

Increased tubule sodium reabsorption has been largely suspected in liver cirrhosis (LC), however studies in humans have produced contrasting results. Therefore to ascertain the entity of renal sodium handling in LC this study was devised. A total of 13 patients with child A LC were studied along with 26 age-sex matched healthy controls (HC).

Nephrotic edema.

This article starts with a concise synopsis of the history of edema. The role of underfilling, overflow, antidiuretic hormone, and acquaporins is subsequently discussed. Emphasis is given to the use of diuretics in edematous patients.

Glomerular filtration rate in severely overweight normotensive humans.

The study examined whether indexing glomerular filtration rate (GFR) for body surface area is appropriate for people who are severely overweight. Twenty normotensive adult men who were severely overweight but without microalbuminuria were enrolled into this study. The control group consisted of 20 healthy subjects matched for age, sex, and height.

Field evaluation of the efficacy of Romovac 50, a new inactivated, adjuvanted bovine rotavirus vaccine.

An inactivated bovine rotavirus vaccine, prepared with an adjuvant which gives a water-in-oil emulsion (Montanide ISA 50, adjuvant) was used in field trials for prevention of calf neonatal diarrhoea. The vaccine, which was designated as Romovac 50, when injected into cows in their last month of pregnancy, proved to be as effective as the traditional vaccine prepared with Freund's incomplete adjuvant. Thus, the incidence of the naturally occurring neonatal diarrhoea was significantly reduced in calves which were fed mammary secretion from their vaccinated dams, compared to the calves delivered from the unvaccinated cows.