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,LALD Header

The Lysosomal Acid Lipase (LAL) Deficiency Knowledge Centre is intended to provide healthcare professionals with accurate disease awareness and treatment information for patients with this condition.

LAL deficiency is an autosomal recessive lysosomal storage disorder resulting in marked lysosomal accumulation of cholesteryl esters and triglycerides. LAL deficiency has multi-organ involvement including the liver, spleen, and cardiovascular system. The two major phenotypes of this condition are a rapidly progressive form in infancy (formerly known as Wolman disease) and a later-onset progressive form in children and adults (formerly known as cholesteryl ester storage disease, CESD).

Users of this educational resource can expect to find clinical content arranged within three main sections:

Disease Awareness: including epidemiology, pathophysiology, genotypic presentation, diagnosis, prognosis and more…

Treatment: presents the drug characteristics, safety and efficacy of sebelipase alfa – the first and only licenced treatment for LAL deficiency

Resources: useful external links, events calendar and a video channel with short expert video discussions on topics such as diagnostic approach and current prognosis.

Developed by EPG Health Media (Europe) Ltd for epgonline.org, in collaboration and with content provided by Alexion Pharma GmbH

,Cystic Fibrosis Knowledge Centre

Cystic fibrosis (CF) is a genetic condition caused by a defect in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR) chloride channel. In people with CF, the protein does not work effectively, resulting in thick, sticky secretions at epithelial membranes – the underlying defect in CF.1 Cystic fibrosis can have multi-organ involvement with a range of complications and comorbidities reported.

The aim of the Cystic Fibrosis Knowledge Centre is to provide healthcare professionals with accurate clinical information including disease awareness information, emerging and established treatment options and current best practice based on recently updated guidelines by the European Cystic Fibrosis Society (ECFS). A resources section contains external links to upcoming medical conferences and patient support groups.

This resource is developed by EPG Health Media for epgonline.org, supported by an independent educational grant from Vertex.

References:
1. Boucher RC. Cystic Fibrosis. In: Kasper DL, Braunwald E, Fauci AS, Hauser SL, Longo DL, Jameson JL et al., editors. Harrison's Internal Medicine. 16th ed. New York: McGraw-Hill; 2005. p. 1-9.​

Latest News

Medical Videos

An Animation of The Hepatitis C Virus and How it Affects the Body
An Animation of The Hepatitis C Virus and How it Affects the Body
Discussion of Hepatitis C in the UK House of Lords June 2011
Discussion of Hepatitis C in the UK House of Lords June 2011

Recent Drug Updates

Medical Journal Abstracts on Hepatology

Elafibranor, an Agonist of the Peroxisome Proliferator-Activated Receptor-α and -δ, Induces Resolution of Nonalcoholic Steatohepatitis Without Fibrosis Worsening.

Sun 01 May 2016 -  Elafibranor, an Agonist of the Peroxisome Proliferator-Activated Receptor-α and -δ, Induces Resolution of Nonalcoholic Steatohepatitis Without Fibrosis Worsening.

Background & Aims Elafibranor is an agonist of the peroxisome proliferator−activated receptor-α and peroxisome proliferator−activated receptor-δ. Elafibranor improves ...

Radiofrequency ablation of hepatocellular carcinoma: Mono or multipolar?

Tue 01 Mar 2016 -  Radiofrequency ablation of hepatocellular carcinoma: Mono or multipolar?

Background and aims: Thermo-ablation by radiofrequency is recognized as a curative treatment for early-stage hepatocellular carcinoma. However, local recurrence may occur because of incomplete ...

Clinical Guidelines

EASL Clinical Practice Guidelines on the management of benign liver tumours

Apr 2016

Benign liver tumours are a heterogeneous group of lesions with different cellular origins, as..

... summarized by an international panel of experts sponsored by the World Congress of Gastroenterology in 1994.

These lesions are frequently found incidentally as a consequence of the widespread use of imaging tests and often have a benign course. Some of these lesions are of greater clinical relevance than others, and the aim of these recommendations is to provide a contemporary aid for the practical diagnosis and management of the more common benign tumours.

These include haemangiomas, focal nodular hyperplasia (FNH) and hepatocellular adenoma (HCA).

EASL Clinical Practice Guidelines on the prevention, diagnosis and treatment of gallstones

Apr 2016

Gallstones or cholelithiasis are a major public health problem in Europe and other developed..

... countries and affect up to 20% of the population.

Gallstone disease is the most common gastrointestinal disorder for which patients are admitted to hospitals in European countries.

The interdisciplinary care for patients with gallstone disease has advanced considerably during recent decades thanks to a growing insight into the pathophysiological mechanisms and remarkable technical developments in endoscopic and surgical procedures. In contrast, primary prevention for this common disease is still in its infancy.

The EASL Clinical Practice Guidelines (CPG) on the prevention, diagnosis and therapy of gallstones aim to provide current recommendations on the following issues:
1. Prevention of gallstones
2. Diagnosis of gallbladder stones
3. Medical therapy of gallbladder stones
4. Surgical therapy of gallbladder stones
5. Diagnosis of bile duct stones
6. Endoscopic and surgical therapy of bile duct stones
7. Diagnosis and therapy of intrahepatic stones
8. Therapy of gallstones during pregnancy

Clinical Trials

Association Between Autologous Transfusion and Recurrence Among Patients With HCC After Resection (AT/HCC)

Fri 01 Jan 2016

This prospective study aims to determine whether autotransfusion of red blood cells salvaged during liver resection is associated with the recurrence of hepatocellular ...

Effects of Empagliflozin on Liver Fat Content, Energy Metabolism and Body Composition in Patients With Type 2 Diabetes (EmLiFa)

Tue 01 Dec 2015

The effects of empagliflozin treatment on hepatocellular lipid content, liver energy metabolism and body composition will be investigated in a multicentre, prospective, placebo-controlled, ...

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