Hepatic amyloidosis

Introduction to hepatic amyloidosis Amyloidosis, also known as amyloid deposition, is characterized by a variety of causes of amyloid-degraded fibrin as the main form of amyloid deposition in the blood vessel wall and organs, extracellular tissue. Progressive, poor prognosis, hepatic amyloidosis is part of systemic amyloidosis, and amyloidosis found only in the liver has not been reported so far. basic knowledge The proportion of illness: 0.02% Susceptible people: no specific population Mode of infection: non-infectious Complications: renal failure, cirrhosis, ascites

Causes of hepatic amyloidosis

(1) Causes of the disease

The etiology and pathogenesis of amyloidosis are still unclear. It is generally believed that amyloidogenic substances are mainly infiltrated between cells due to various reasons, or deposited under the basement membrane of small blood vessels, or deposited along reticular fiber scaffolds.

(two) pathogenesis

When the lesions proceeded, these sediments pressed and destroyed these tissues leading to organ failure and death. The amyloid denatured fibers were isolated and purified for amino acid sequence analysis and found to have three protein types:

1. The AL type protein is derived from the light chain of the immunoglobulin (especially the variable region), and the N-terminal sequence and the partial region of the immunoglobulin light chain are homologous, including the kappa type and the lambda type, and the lambda type light chain ratio The type is more likely to form amyloid fibrils, found in primary amyloidosis and amyloid deposition associated with multiple myeloma.

2. The AA protein has the same N-terminal sequence as the non-immunoglobulin called AA protein, the amyloid fiber seems to be independent of the light chain, and the main amyloid fiber consists of a protein A different from immunoglobulin. It may be due to the proteolytic action of phagocytic lysosomal enzymes produced by plasma cells, which is found in secondary and familial Mediterranean fever associated with amyloid deposition.

3. AF type protein amyloid fiber is mainly a normal or abnormal prealbumin complex (molecular weight 14000), usually a single amino acid replacement product of transthyretin (pre- albumin), followed by 2 microglobulin, mainly found in Familial amyloid deposition.

Clinically, according to the condition of amyloidosis involving organs, it can be divided into systemic and localized. According to the presence or absence of the original disease, it can be divided into primary, secondary or hereditary (Table 1).

Hepatic amyloidosis prevention

1. Active treatment of primary disease For patients with secondary amyloidosis, such as the control of rheumatoid arthritis, chronic osteomyelitis, tuberculosis and multiple myeloma and other primary diseases, hepatic amyloidosis may subside.

2. Actively exercise the body to enhance the body's immunity, and it is helpful to prevent the occurrence of this disease. In addition, patients should be reminded to adjust their emotions and keep their mood comfortable.

3. The cause of death in patients with hepatic amyloidosis is secondary infection and heart and kidney failure. Therefore, it is necessary to closely observe changes in the condition. Once this happens, it should be rescued immediately to reduce the mortality rate.

Hepatic amyloidosis complications Complications, renal failure, cirrhosis, ascites

There are secondary infections, heart and kidney failure and cirrhosis ascites.

Symptoms of hepatic amyloidosis Common symptoms Amyloidosis, abdominal distension, jaundice, ascites, liver amyloidosis, varicose veins, liver capsule tension

There are no specific symptoms and signs in amyloidosis. The symptoms are determined by the location of the original disease and amyloid deposits, the amount of deposition and the organs and systems involved. The symptoms are often masked by the primary disease.

Secondary, more than 95% of systemic amyloidosis has liver involvement, often manifested as large liver, upper abdominal fullness, anorexia, a few can be severe liver enlargement (hepatic weight can reach more than 7kg), but liver damage Slightly, occasionally high portal hypertension and manifested as esophagus, gastric varices and ascites, etc., very few may have jaundice.

Hepatic amyloidosis

1, liver function (alanine aminotransferase, aspartate aminotransferase), the clotting time is mostly normal, or mild abnormal changes, mostly expressed as gamma glutamyl transpeptidase and alkaline phosphatase increased, bilirubin exceeded 85.5mol / L Rarely, anemia, proteinuria, etc. can occur, and the erythrocyte sedimentation rate can be normal or increased.

2, imaging examination lacks specificity, ultrasound examination is mainly changed to: liver volume increases, liver parenchyma is thick and point-like uniform echo, portal vein can be widened, sometimes ascites can be formed, CT is mainly manifested as: liver, diffuse liver In the low-density area, the enhancement is not obvious, and the blood vessels in the liver are not displaced.

Diagnosis and identification of hepatic amyloidosis

diagnosis

According to the symptoms, signs and auxiliary examinations, a preliminary diagnosis of amyloidosis can be made. The diagnosis requires tissue biopsy. Skin and rectal mucosal biopsy is the most commonly used screening method. Other biopsy sites include gums, nerves, kidneys and liver. Suspected primary hepatic amyloidosis, liver biopsy can be directly performed. For patients with amyloidosis with obvious enlargement of the liver, hepatic rupture or hepatic hemorrhage may occur due to hepatic membrane tension. The clinical should be cautious for the whole body. Gastric and rectal mucosal biopsies are mostly safe and effective in patients with amyloidosis.

Differential diagnosis

Liver amyloidosis needs to be differentiated from various liver diseases that cause hepatomegaly, such as acute hepatitis, cirrhosis caused by various causes, primary or secondary liver cancer, and hepatolenticular degeneration.

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