Alagille syndrome

　　Congenital biliary atresia is characterized by marked liver enlargement, with most portal areas showing no bile ducts under the microscope. Sometimes, underdeveloped bile ducts can be seen, which are often without obvious lumens. Accompanied by marked cholestasis and mild fibrosis in the portal areas, there may be interstitial fibrosis in the testes. For liver-gallbladder imbalance, blood and Qi stasis leading to obstruction of the meridians and collaterals. Mild fibrosis of the liver and gallbladder, nutritional imbalance, and so on.

　　The prognosis of liver disease in children with neonatal cholestasis is poor. However, severe liver complications may appear only at the late stage of liver disease and require lifelong follow-up. The chronic progression of the disease may involve chronic active hepatitis, liver cirrhosis, liver failure, viral hepatitis, fatty liver, and various types of liver cirrhosis, among others. Pay attention to differential diagnosis.

　　Both males and females can be affected by the disease, with mild jaundice occurring within 3 months after birth. Cholestasis within the liver is the main feature of this disease, accompanied by severe itching, blood cholesterol levels as high as 50.8 mmol/L, elevated alkaline phosphatase, and normal alanine aminotransferase. The forehead is prominent, the distance between the eyes and nose is large, the chin is small and pointed, and a systolic murmur can be heard in the pulmonary artery valve. The anterior arch of the spine is open and not fused, without scoliosis, with varying degrees of delayed intellectual development, and may have underdeveloped testes.

　　There are no special preventive measures. Taking multiple drugs is prone to drug interactions, affecting the liver's ability to metabolize drugs. Ganoderma lucidum has the effects of protecting the liver and detoxifying, can reduce SGPT, promote liver cell regeneration, enhance liver antioxidant enzymes, and inhibit the liver tissue's liver lipid peroxidation ability, which can obtain significant improvement for patients with hepatitis, liver cirrhosis, and liver cancer. When people with liver disease seek medical treatment, they should inform the doctor of all the drugs they are currently taking as a reference for the doctor's prescription.

　　At the time of diagnosis of Alagille syndrome, in addition to relying on its clinical manifestations, it is also necessary to rely on auxiliary examinations. This disease can be diagnosed by trypsin, serum alkaline phosphatase, cardiovascular angiography, urinalysis, fecal characteristics, and liver biopsy.

The diet of Alagille syndrome patients should be light, easy to digest, with an emphasis on vegetables and fruits, a reasonable diet, and attention to adequate nutrition. In addition, patients should also pay attention to avoiding spicy, greasy, and cold foods.

　　Bile acid sequestrants or traditional Chinese medicine can be given to treat bile stasis and supplement fat-soluble vitamins.

　　1. Treatment for the Underlying Etiology

　　For bile stasis with clear basic etiology, such as possible, strive for radical treatment or control of the underlying disease. For obstructions caused by tumors or stones, radical tumor resection or ERCP stone removal can be performed; repairing bile duct stenosis can restore bile duct drainage; for immunological damage to bile ductules, immunosuppressants may be effective; for drug-induced bile stasis, stop using the relevant drugs in a timely manner.

　　2. Supportive and Symptomatic Treatment

　　Ursodeoxycholic Acid, Phosphatidylcholine Capsules, Adenosine Methionine, Phenobarbital. Traditional Chinese medicine preparations have the effects of promoting bile excretion and reducing enzymes, such as Bupleurum, White Peony,茵陈, Salvia miltiorrhiza, etc.; for the treatment of itching, it can supplement fat-soluble vitamins, calcium, and vitamin D.