Pediatric sclerosing cholangitis

Introduction to pediatric sclerosing cholangitis Sclerosing cholangitis (sclerosing cholangitis), also known as fibrotic cholangitis, is a type of cholestasis syndrome. It is caused by inflammation of the intrahepatic and extrahepatic bile ducts, and submucosal and serosal fibrosis of the intrahepatic and extrahepatic bile ducts. Fibrous stenosis and occlusion. Finally, it can develop into biliary cirrhosis and portal hypertension. In a few cases, the gallbladder and pancreatic duct may be involved, and may be accompanied by chronic ulcerative colitis or retroperitoneal fibrosis. basic knowledge The proportion of illness: 0.001% Susceptible people: children Mode of infection: non-infectious Complications: diarrhea, cirrhosis, ulcerative colitis, retroperitoneal fibrosis, cholangiocarcinoma

Causes of sclerosing cholangitis in children

(1) Causes of the disease

1. The cause of the disease is still unclear. It is generally considered to have:

(1) Bacterial or viral infection factors: Chronic biliary tract inflammation causes fibrosis of the bile duct wall, and the lumen gradually narrows.

(2) allergic factors: sometimes accompanied by other autoimmune diseases such as ulcerative colitis and Crohn's disease.

(3) Congenital genetic factors: The literature reports that sclerosing cholangitis may be a genetically predisposed autoimmune disease. Children with close relatives of their parents have a higher chance of developing this disease. Some scholars believe that it is an autosomal recessive. Genetic diseases, children with primary sclerosing cholangitis often have histocompatibility antigen HLA-B8 (specific human leukocyte antigen) significantly higher than the control group, some people think it is an immune function regulation disorder.

2. Classification There are two types of the disease:

(1) Primary: There is no clinical cause for the disease, treatment is difficult, and the prognosis is poor.

(2) Secondary: Can find direct or indirect causes of the disease, such as chronic biliary infection, stones, surgery and trauma, the primary is more common in children.

(two) pathogenesis

Early liver biopsy showed inflammatory reaction in portal vein and portal area, connective tissue hyperplasia, lymphocyte and plasma cell infiltration, interlobular bile duct hyperplasia, bile duct wall fibrosis gradually replaced by fibrous tissue, thickened and hardened wall, stenosis Until the lock, according to the range of bile duct invasion, it is divided into diffuse type, limited type and segment type (also called jump type).

Pediatric sclerosing cholangitis prevention

Go to bed early and get up early and exercise. Insufficient sleep can reduce the body's immune function, and it is also easy to stimulate the fire, causing external injuries. Keep your mind calm. Avoid anger in the spring, do not be too impatient, always keep your peace of mind.

Complications of sclerosing cholangitis in children Complications diarrhea cirrhosis ulcerative colitis retroperitoneal fibrotic cholangiocarcinoma

Often complicated by chronic diarrhea, nutritional disorders, advanced cirrhosis and portal hypertension, a small number of cases can involve the gallbladder and pancreatic duct, gallbladder and (or) bile duct stones, major bile duct stricture and recurrent bacterial cholangitis, may be accompanied Chronic ulcerative colitis or retroperitoneal fibrosis, can occur cholangiocarcinoma, varicose veins around the ileal anastomosis after rectal colectomy (peristomal varices), PSC associated diseases, a lot of literature reports, mainly autoimmune Diseases, the most common of which is IBD, early reports of PSC patients with IBD incidence rate of 25% to 30%, recently reported 54% to 100%; PSC associated with IBD, the most common CUC, up to 70%, Crohn colitis is about 13%, and there is no report that Crohn's bowel disease only affects the small intestine without involving the colon.

Pediatric sclerosing cholangitis symptoms common symptoms high fever nausea portal hypertension jaundice diarrhea pruritus low fever appetite weak liver and hard biliary cirrhosis

Mainly manifested as chronic progressive obstructive jaundice with obvious itching, chronic pain or discomfort in the right upper quadrant, loss of appetite, nausea and vomiting, chronic diarrhea, malnutrition, some children with long-term low fever or high fever, physical examination can reach increased The liver and liver are hard, and the late stage can develop into biliary cirrhosis and portal hypertension. Some children are often accompanied by chronic ulcer, spleen colitis, Crohns disease, etc., and have growth retardation and delayed puberty.

Examination of sclerosing cholangitis in children

1. Liver function test: serum bilirubin is elevated, mainly direct bilirubin, alkaline phosphatase is elevated, often 2 to 3 times of normal value, and transaminase is slightly elevated.

2. Peripheral blood: leukocytosis.

3. Other tests: serum IgM is also higher than normal, IgA, IgG, serum copper is elevated, urinary copper is increased, and serum ceruloplasmin is increased.

4. Percutaneous gallbladder puncture and intraoperative cholangiography: Chen is divided into the following types according to the intrahepatic and external biliary tract development of primary sclerosing cholangitis:

(1) Intrahepatic type: subdivided: Type I: mild outline of bile duct, local stenosis, no obstruction of distal bile duct, type II: linear stenosis of the lumen, mild dilatation of distal bile duct, distal end Bile duct bifurcation is rare, "wild branch", type III: the distal bile duct is completely occluded, the central bile duct is fusiform, cystic or pouchlike, and the narrowing between the dilated segments is "beaded", type IV: distal bile duct Irregular, narrow or occluded, only the central bile duct is full, and there are no bile ducts in the large peripheral liver.

(2) extrahepatic type: subdivided: type I: extrahepatic biliary tract with general or segmental irregular changes, no obvious stenosis, type II: bile duct is segmental stenosis, lumen is smooth or irregular, type III: Irregular stenosis, all extrahepatic bile ducts were bead-like changes, type IV: extremely irregular lumens, dilatation and stenosis, severe lesions, diverticulum-like.

5. Liver and gallbladder MRI examination: It can be found that the intrahepatic bile duct is thickened, the lesion is abnormal, and other diseases such as metastatic liver cancer, pancreatic cancer and other bile duct obstruction diseases can be excluded.

6. B-type ultrasound: the extrahepatic type can not see the common bile duct, the extrahepatic bile duct is thick and thick, and the wall echo is strong with the intrahepatic bile duct dilatation.

Diagnosis and differential diagnosis of pediatric sclerosing cholangitis

diagnosis

Secondary sclerosing cholangitis: There are many direct or indirect causes of the disease, and there is no difficulty in diagnosis.

Primary sclerosing cholangitis can be diagnosed according to the criteria proposed by Myers et al in 1970:

1 progressive obstructive jaundice. 2 no biliary stones. 3 no history of biliary surgery. 4 The bile duct wall is thickened and the lumen is narrow. 5 long-term observation can rule out biliary malignant lesions. 6 complication without ulcerative colitis or Crohn's disease, in order to determine the location of the lesion, the scope and the shape of the uninfected biliary tract, B-mode ultrasound, percutaneous transhepatic angiography (PTC) and duodenal fibers Endoscopy and other examinations.

Differential diagnosis

Different from cholelithiasis, cholecystitis and congenital malformations of the gallbladder and bile duct, laboratory tests and auxiliary examinations can help identify. Primary sclerosing cholangitis should be excluded from other causes of sclerosing cholangitis or bile duct stenosis / obstruction; need to be differentiated from other cholestatic diseases, such as primary biliary cirrhosis, idiopathic adult bile duct reduction, drugs Cholestatic, chronic active hepatitis, alcoholic liver disease, autoimmune hepatitis, etc. In particular, in some patients with atypical PSC, serum ALP is only slightly elevated, while ALT/AST is significantly elevated, which is easily misdiagnosed as AIH.

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