OVERVIEW
OVERVIEW
Hepatic amyloidosis is a metabolic disease caused by extracellular amyloid deposits in the walls of hepatic blood vessels and tissues, and is part of systemic amyloidosis. Amyloid can be deposited locally or systemically, mainly involving tissues of the heart, liver, kidneys, spleen, gastrointestinal, muscle and skin. It is usually classified by the biochemical composition of the amyloidogenic fibrils, which are generally beta-structured amyloidogenic fibrillar protein deposits. Amyloidosis can be categorized as primary or secondary.
Whether medical insurance
Yes
Department
Gastroenterology
Clinical Symptoms
Fatigue, weakness, weight loss, shortness of breath on exertion, edema, and liver enlargement.
Hazards
May lead to secondary infection, cardiac and renal failure, cirrhosis, etc.
Examination
Liver function test, prothrombinogen test, pathologic examination, isotope technetium sulfur colloid liver scan, abdominal ultrasound, CT examination, etc.
Diagnosis
Diagnosis is based on the manifestations of fatigue, weakness, weight loss, shortness of breath after exertion, edema, liver enlargement, etc., combined with liver function tests, pathology tests and imaging tests such as abdominal ultrasound and CT.
Treatment principle
There is no specific treatment for this disease, and the principle is to prevent further deposition of amyloid and promote or accelerate the absorption of deposited amyloid. Symptomatic treatment and supportive therapy are mostly used.
Curability
Symptoms may improve with active treatment.
Dietary advice
Give high protein diet, appropriate sugar intake, adequate vitamin supplementation, avoid alcohol and spicy food.
Causes
Causes
The cause of primary amyloidosis is unknown, while secondary amyloidosis is mostly induced by chronic diseases.
Symptoms and Diagnosis
Typical symptoms
Hepatic amyloidosis develops slowly, and the clinical manifestations are mostly concealed by the primary disease. Common first symptoms include fatigue, weakness, weight loss, shortness of breath after exertion, and edema. Some may manifest as vague pain in the right quarter rib area, hepatomegaly, abdominal effusion, jaundice and so on.
Other symptoms
All systems and organs of the body can be involved at the same time, the liver function damage is relatively mild, and heart enlargement, arrhythmia, and even heart failure can occur when the heart is involved.
Diagnostic basis
1. The first symptoms are fatigue, weakness, weight loss, shortness of breath after exertion, edema and liver enlargement. In some cases, it may also manifest as vague pain in the right quarter rib area, hepatomegaly, abdominal fluid accumulation, jaundice, and so on. All systems and organs of the body can be involved at the same time, and the liver function damage is relatively mild, and heart enlargement, arrhythmia and even heart failure can occur when the heart is involved.2. Auxiliary examination (1) Laboratory examination: liver function test can see the elevation of glutamyltransferase and alkaline phosphatase, which is more than two times of the upper limit of the normal; prolongation of the prothrombinogen time. (2) Isotope technetium (99mTc) sulfur colloid liver scan: it has auxiliary diagnostic value for hepatic amyloidosis and can dynamically observe the progress of the disease and the effect of treatment. (3) Abdominal ultrasound: irregular dense echogenic light clusters in the liver can be seen on ultrasound imaging, but they are not specific. (4) Abdominal CT examination: imaging shows decreased density of the liver. (5) Pathological examination: liver puncture biopsy is the main and most reliable means to confirm the diagnosis of systemic amyloidosis, the amyloid material in the tissues is stained by Congo red and shows green birefringence under polarized microscope, which is of diagnostic value.
Treatment
Treatment guidelines
There is no specific treatment for this disease, and symptomatic treatment and supportive treatment are mostly used.
Drug treatment
Primary amyloidosis is treated with melphalan, prednisone, 10% dimethyl sulfoxide solution and colchicine.
Other treatments
1. Patients with primary amyloidosis can also be treated with autologous stem cell transplantation. 2. Patients with secondary amyloidosis should be actively treated for the underlying disease, and amyloidosis may stop developing or even subside if it is well controlled.
Prognosis
The prognosis of this disease is poor, with a median survival of 13 months, with large individual differences, depending on the scope, degree and progression of the disease and organ involvement, etc.
Nursing care
Daily care
1. Exercise actively to strengthen the resistance of the body. 2. Keep in a good mood and enhance the confidence to overcome the disease. 3.
Diet regulation
Give high protein diet, consume appropriate sugar, and supplement sufficient vitamins. Avoid drinking alcohol and spicy stimulating food.