Η πρωτοπαθής χολοκαλαιμία

    Η αιτία της πρωτοπαθής χολοκαλαιμίας δεν είναι ακόμη γνωστή και μπορεί να έχει σχέση με την αυτοάνοση. Η πρωτοπαθής χολοκαλαιμία συχνά συνυπάρχει με άλλες αυτοάνοσες ασθένειες όπως η ρευματοειδής αρθρίτιδα, το σύνδρομο σιτοειδούς, η σκληροδέρμα, η χρόνια λεμφώδης θυρεοειδίτιδα κ.λπ., και είναι πιο συχνή στις μεσήλικες γυναίκες.

　　Επειδή η πρωτοπαθής χολοκαλαιμία μπορεί να έχει σχέση με την αυτοάνοση, οι επιπλοκές της νόσου είναι οι εξής:

　　1φθαρής δέρματος

　　Η παρούσα κατάσταση για την αντιμετώπιση της φθαρής δέρματος δεν έχει ακόμη κλασική και αποτελεσματική θεραπεία. Η χορήγηση ανιόντων ανταλλαγής阴离子 exchange resin胆胺 είναι η πρώτη γραμμή θεραπείας για την αντιμετώπιση της φθαρής δέρματος. Αν ο ασθενής δεν μπορεί να αντέξει τις παρενέργειες της胆胺, η ριφομπικίνη μπορεί να χρησιμοποιηθεί ως δεύτερη γραμμή θεραπείας. Η ριφομπικίνη μπορεί να ελέγξει καλά τα συμπτώματα φθαρής της PBC, αλλά δεν είναι αποτελεσματική για όλους τους ασθενείς. Η αποτελεσματικότητά της είναι συνήθως1months before it becomes significant. Rifampin may achieve an antipruritic effect by changing the internal environment of bile acids in liver cells and improving the biochemical indicators of PBC patients. Nalmephene, Naltrexone, and other opiate drugs can be used for patients who are ineffective with cholestyramine and rifampin. There are many other methods (such as ultraviolet light, light therapy, and plasma apheresis therapy, etc.) used for the control of pruritus in PBC, but none have been confirmed by regular clinical trials. For intractable pruritus that cannot be controlled, liver transplantation surgery can be performed.

　　2, osteoporosis

　　After the clear diagnosis of PBC, bone density should be measured regularly, and then every2years follow-up once. Educate patients to develop good living habits (such as regular作息, quit smoking), and supplement vitamin D and calcium. Postmenopausal women are recommended to use hormone replacement therapy, and it is best to administer it through the skin. If osteoporosis is very obvious, biophosphonate treatment can be used.

　　about11% of PBC patients have osteoporosis. Supplementing calcium and vitamin D can prevent demineralization, the recommended doses are1000 ~1200mg/d and25000 ~50000 IU, once a week2~3time. Recent research has found that raloxifene can prevent bone loss and reduce serum cholesterol.60mg/d, take continuously1years, can increase the bone mineral density of the lumbar spine (LS2BMD), without liver toxicity. In addition, alendronate can effectively increase bone mass.

　　3, SICCA syndrome

　　All PBC patients should be asked about symptoms such as dry eyes, dry mouth, and difficulty swallowing, and female patients should also be asked about sexual difficulties, and appropriate treatment measures should be taken if they exist.

　　4, Raynaud's syndrome

　　For patients in cold areas, the treatment of Raynaud's syndrome is a difficult problem, patients should avoid exposing their hands and feet to cold environments, and smokers should quit smoking. Calcium channel blockers can be used if necessary, but they may worsen lower esophageal sphincter dysfunction.

　　5, portal hypertension

　　PBC patients can develop pre-sinusoidal portal hypertension before liver cirrhosis, and the treatment of portal hypertension in liver cirrhosis patients is the same as that of other types of liver cirrhosis. However, the efficacy of beta-blockers for non-cirrhotic pre-sinusoidal portal hypertension needs to be confirmed, and shunt surgery can be considered if necessary. It is recommended that PBC patients be screened for the presence of esophageal varices at the time of the first clear diagnosis, and after that2once a year. If varices are found, measures should be taken to prevent bleeding.

　　6, a lack of fat-soluble vitamins

　　Hyperbilirubinemia can be accompanied by a lack of fat-soluble vitamins and poor calcium absorption, and in patients without jaundice, little is known about the level of fat-soluble vitamins and the value of oral supplementation. Fat-soluble vitamin supplementation is best given in a water-soluble form. Monthly subcutaneous injection of vitamin K can correct coagulopathy secondary to vitamin K deficiency.

　　7, thyroid disease

　　Thyroid disease can affect about15% ~25In % of PBC patients, it usually exists before the onset of PBC. It is recommended to measure the serum thyroid hormone level in patients diagnosed with PBC and to check regularly.

　　Primary biliary cirrhosis often coexists with other immune diseases such as rheumatoid arthritis, Sjögren's syndrome, systemic sclerosis, chronic lymphocytic thyroiditis, etc., and is more common in middle-aged women, with insidious onset, slow progression, mild early symptoms, and patients generally in good condition, with no significant decrease in appetite and weight, about10％ of patients may have no symptoms at all. For patients with unexplained chronic progressive obstructive jaundice, especially those with steatorrhea, the etiology and progression of the disease should be understood in detail, whether there are other immune diseases, and attention should be paid to distinguishing from secondary biliary cirrhosis and jaundice caused by other reasons.

　　Since the etiology of primary biliary cirrhosis is unknown, there is no effective prevention method for the disease. Try not to eat foods that damage the liver. Maintain the habit of exercise and a good attitude.

　　Primary biliary cirrhosis is mainly judged clinically according to the following criteria: significantly elevated triglycerides, serum bile acids, conjugated bilirubin, alkaline phosphatase and glutamyl transferase, etc., normal or slightly to moderately elevated transaminases, positive anti-mitochondrial antibodies in the blood, elevated IgM, prolonged prothrombin time, positive bilirubin in urine, and normal or decreased bilirubinogen in urine.

　　Imaging: Ultrasound, endoscopic retrograde cholangiopancreatography, CT, percutaneous liver biopsy cholangiography, etc., to understand whether there is expansion of bile ducts inside and outside the liver and diseases that cause obstructive jaundice outside the liver.

　　The treatment of primary biliary cirrhosis mainly includes adequate rest, high-protein, high-carbohydrate, high-vitamin, low-fat diet, daily fat