Abrami syndrome (also known as Widal-Abrami disease or enterohepatic syndrome) is a rare liver disease characterized by the accumulation of fats in liver cells (hepatocytes).
This disease was first described by the French physician Louis-Albert Abrami in 1879. He discovered fat deposits in the liver during an autopsy of a patient with progressive jaundice. Later, in 1903, the French pathologist Ernest Vidal confirmed and expanded Abrami's description, so the disease is sometimes also called Vidal-Abrami disease.
With Abra syndrome, triglycerides accumulate in the cytoplasm of hepatocytes, which leads to the development of fatty liver degeneration. This is accompanied by progressive jaundice, hepatosplenomegaly, ascites and other signs of liver failure.
The reasons for the development of Abrami syndrome are not completely clear. It is assumed that the underlying cause is a violation of lipid metabolism and lipoprotein transport. The disease is more common in middle-aged and elderly women.
The diagnosis is made on the basis of the clinical picture, data from laboratory and instrumental research methods. Treatment is usually ineffective and is supportive in nature. The prognosis is poor, with mortality reaching 60% within 2 years of diagnosis.
Abramo Syndrome (AS) is a rare and severe disease characterized by a combination of gastrointestinal disorders and hepatolienal failure. This disease occurs exclusively in women, which serves as one of the pathogenetic mechanisms of its formation.
First described by the French doctor M. Abramory in 1902 as isolated cases of intestinal disorders. The term "Abramo syndrome" is considered outdated, has no official confirmation and is not widely used. The disease has several synonyms. The most common remains “vidal abreamo disease” (named after the research doctor