Lysosomal Acid Lipase (LAL) is a key enzyme involved in lipid metabolism, responsible for hydrolysing the cholesteryl esters and triglycerides. Wolman Disease represents the early onset phenotype of LAL deficiency rapidly leading to death. Cholesterol Ester Storage Disease is a late onset phenotype that occurs with fatty liver, elevated aminotransferase levels, hepatomegaly and dyslipidaemia, t...

Autoimmune hepatitis (AIH) is a complex liver disease of unknown cause which results in immune-mediated liver injury with varied clinical presentations. Seronegative AIH follows a similar course to autoantibody-positive disease and diagnosis may be challenging. There are no single serologic tests of sufficient diagnostic specificity, and delay in appropriate treatment may lead to progression of...

BACKGROUND Digestive and liver diseases (LD) are among the most common causes of mortality and morbidity in Iran and throughout the world. We have aimed to report the etiology and outcome of gastrointestinal and LD that needed admission in a typical tertiary referral hospital in Tehran during the last decade. METHODS Shariati Hospital Gastroenterology and Liver Disease Department (GI & LD) was ...

Impaired kidney function is a well-recognized complication following liver transplantation (LT). Studies of this complication in children have been limited by small numbers and insensitive outcome measures. Our aim was to define the prevalence of, and identify risk factors for, post-LT kidney dysfunction in a multicenter pediatric cohort using measured glomerular filtration rate (mGFR). We cond...

Epilepsy is an uncommon comorbidity of Parkinson's disease (PD) and has been considered not directly associated with PD. We present five patients (3 men and 2 women; ages 49-85) who had concomitant PD and cryptogenic epilepsy. Although rare, epilepsy can coexist with PD and their coexistence may influence the progression of PD. While this may be a chance association, an evolving understanding o...

BACKGROUND Worldwide, chronic liver disease is a major cause of morbidity and mortality. Causes of elevated serum alanine aminotransferase (ALT) levels vary depending on the population under study. The aim of this study is to evaluate the frequency and causes of persistently elevated ALT levels in patients of the Gastroenterology (GI) Clinics in Ghaem and Emam Reza Hospitals in Mashhad, Iran. M...

1. Mitochondria and microsomal fractions have been isolated from liver biopsies from patients with alcoholic cirrhosis, cryptogenic cirrhosis or chronic aggressive hepatitis. 2. Cirrhotic livers yieled fewer mitochondria than normal liver. 3. The most significant change was a decrease in mitochondrial respiratory control. Cirrhotic microsomal fractions had a 50% diminution in cytochrome b5 and ...

Patients with chronic active hepatitis or alcoholic cirrhosis have serum antibodies to many more serotypes of Escherichia coli than do patients with primary biliary cirrhosis or cryptogenic cirrhosis, or normal controls. They also have antibodies against more serotypes than cirrhotic patients with a portacaval shunt. These observations suggest that factors other than shunting of blood away from...