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Alagille syndrome

Disease name

This entry is made by Compilation and application of scientific encyclopedia entries of "Science Popularization China" authentication.

Korean English

(Chief Physician) Review

Department of Gastroenterology, General Hospital of Beijing Military Region

Alagille syndrome is a chronic disease with phenotypic characteristics Cholestasis The most common reason for Multi system Dominance of Hereditary disease 。 This syndrome was first reported by Alagille et al. in 1969. The organs involved in Alagille syndrome include liver, heart, bone, eyes and face, etc. The incidence of this disease reported abroad is about 1/70000. In recent years, China began to pay attention to the disease.

Foreign name

Alagille syndrome

Visiting department

GI Medicine

Multiple population

Infancy

Common diseases

Liver, heart, bones, eyes, face, etc

Common causes

Congenital intrahepatic bile duct dysplasia

common symptom

Severe itching, prominent forehead, large distance between eyes and nose, small and sharp chin, etc

Chinese name

Alagille syndrome

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essential information

Foreign name: Alagille syndrome
Visiting department: GI Medicine
Multiple population: Infancy
Common location: Liver, heart, bones, eyes, face, etc

Common causes: Congenital intrahepatic bile duct dysplasia
common symptom: Severe itching, prominent forehead, large distance between eyes and nose, small and sharp chin, etc
Chinese name: Alagille syndrome

pathogeny

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Congenital intrahepatic bile duct Dysplasia, manifested as obvious enlargement of liver, most of which are seen under microscope Gate management area There is no bile duct, sometimes dysplastic bile duct can be seen, mostly without obvious lumen, accompanied by obvious Cholestasis Phenomenon and mild fibrosis in portal area, testis can be seen Interstitial fibrosis Etc.

clinical manifestation

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Both men and women can suffer from the disease. Mild disease occurs within 3 months after birth jaundice ， Intrahepatic cholestasis Is the main feature of the disease; serious itch , prominent forehead, large distance between eyes and nose, small and pointed chin; Pulmonary valve Audible Systolic murmur ； The anterior arch of the spine is split, not fused, has no scoliosis, and has varying degrees of intelligence Developmental retardation ； There may be testicular dysplasia.

diagnosis

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According to typical symptoms and Liver biopsy Can be diagnosed. This disease can be diagnosed only when there are three or more of the following:

1. Intrahepatic bile duct Hypoplasia ；

2. Surroundings Pulmonary artery stenosis ；

3. Typical Facial features ；

4. Spine Anterior arch Fission;

five Immediate relatives More than one person has Alagille syndrome.

differential diagnosis

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Alagille syndrome is Infancy chronic cholestatic liver diseases Is one of the important reasons. The early diagnosis of this syndrome is difficult, and it is very easy to be misdiagnosed as Biliary atresia We must be alert in clinical work. adopt Hepatic puncture Pathological examination Eye examination and spinal radiography are helpful for early identification and correct diagnosis.

treatment

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No special treatment available Cholagogue Or traditional Chinese medicine to treat Cholestasis , and supplement Fat soluble vitamin 。

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