OVERVIEW
Benign recurrent intrahepatic cholestasis is also known as benign familial intrahepatic cholestasis because of the familial occurrence in some patients. The etiology is unknown and may not be due to a single cause. The first attack often occurs in early childhood and may be recurrent. The episodes are quite similar to obstructive jaundice, but the liver histology shows microcholestatic cholestatic changes, and liver function and liver histology return to normal in the intervals, and the prognosis is good.
Etiology
The etiology of the disease is unknown and may be due to congenital defects in bile acid-dependent and non-bile acid-dependent excretory functions. Some patients have a family history. In addition, there are seasonal episodes of the disease, during which allergies, such as skin rashes, occur, thus requiring attention to the influence of environmental factors on the disease.
Symptoms
Clinical symptoms of the disease can appear at any age, and are more common in children and adolescents. It is characterized by intermittent recurrent episodes of bruising. The attack period lasts from 1 to 6 months, occasionally for more than 1 year. Before the attack, there is usually no clear trigger, and each attack has a short prodromal period, which is mainly manifested by weakness, loss of appetite, vomiting, diarrhea, deepening of urine color, and itching of the skin, followed by a gangrenous stage, progressive aggravation of jaundice and itching of the skin, light-colored stools, fatty diarrhea, and absence of fever and abdominal pain. Physical examination showed hepatomegaly without splenomegaly. Sleep is often interfered with due to itching and weight loss.
Examination
1. Laboratory examination
Elevated serum bilirubin, mainly increased conjugated bilirubin, serum bile acids and alkaline phosphatase are markedly increased, ALT is normal or slightly high, and intermittent laboratory tests are normal.
2. PTC or cholangiography examination
The bile ducts inside and outside the liver are normal.
Diagnosis
The diagnosis of this disease mainly depends on clinical history and clinical manifestations, and the combination of ultrasound, liver biopsy, retrograde cholangiography and percutaneous transhepatic cholangiopancreatography can assist the diagnosis. Diagnostic criteria: (1) episodes of significant jaundice and severe itching without any symptoms between episodes; (2) exclusion of other risk factors for intrahepatic sludgy jaundice, such as drugs or pregnancy; (3) episodes of biochemical tests consistent with intrahepatic sludgy jaundice, and imaging suggests that the intra- and extrahepatic biliary system is normal; (4) biliary plugs can be detected in hepatopenetrated tissues under light microscopy.
Treatment
The disease is self-limiting and can recover by itself. If there is itching, it can be treated symptomatically, and cholestyramine, phenobarbital, adrenocorticotropic hormone can be tried. If jaundice persists for a long time, fat-soluble vitamins and medium-chain fatty acids can be supplemented appropriately.
Questions you may be concerned about
How to treat benign recurrent intrahepatic cholestasis?
Benign recurrent intrahepatic cholestasis is treated with medication and surgery.
The pathogenesis of benign recurrent intrahepatic cholestasis is still unclear, and it is thought to be related to genes and hereditary factors. The symptoms can appear at any age, and the clinical manifestations are mainly intermittent recurrent episodes of cholestasis, with fatigue, loss of appetite, deepening of the color of the urine, itching of the skin, and jaundice, etc. The main treatment modalities are medication.
At present, the main treatment is to take medication, commonly used drugs, such as ursodeoxycholic acid, can increase the secretion of bile acid so that the composition of bile changes, reduce the cholesterol in the bile and cholesterol lipids, which is conducive to the gradual dissolution of cholesterol, and improve the symptoms of jaundice and itchiness to a certain extent.
If there is itching, it can be treated symptomatically, and can be tried with calamine powder, phenobarbital, adrenocorticotropic hormone and so on. If jaundice persists for a long time, fat-soluble vitamins and medium-chain fatty acids can be supplemented appropriately.
Secondly, if the jaundice is severe, biliary drainage can be carried out through hepatic bile duct drainage, which is mainly applicable to biliary stasis caused by narrowing of ducts, mainly through surgical incision of the narrowed hepatic bile ducts, implantation of drainage tubes or biliary stents, and continuous drainage after propping up the narrowed ducts.
Benign recurrent intrahepatic cholestasis can be treated by consulting a medical doctor, and the medication should be applied according to the doctor’s instructions.