Chronic pancreatitis in children
Introduction
Introduction to chronic pancreatitis in children Chronic pancreatitis (chronic pancreatitis) refers to the local or diffuse chronic progressive inflammation of the pancreas, which is progressive, persistent, and irreversible. Increasingly increased pancreatic parenchymal damage leads to a progressive decline in pancreatic endocrine and exocrine functions. basic knowledge The proportion of the disease: 0.2% (the incidence of pancreas and biliary dysfunction is higher) Susceptible people: children Mode of infection: non-infectious Complications: Diabetes Pancreatic cancer
Cause
Causes of childhood chronic pancreatitis
Chronic calcific pancreatitis (25%):
(1) hereditary pancreatitis: an autosomal recessive hereditary disease. The gene of hereditary pancreatitis is located on the long arm of chromosome 7, and the arginine at position 117 of trypsinogen is replaced by histidine. Causes the self-digestion of the pancreas and induces pancreatitis.
Pathological findings included pancreatic atrophy, fibrosis and calcification, almost atrophic acinar cells, ductal obstruction and extensive fibrosis, and islet cells intact.
(2) Adolescent tropical (nutritional) pancreatitis: tropical (nutritive) pancreatitis is a common cause of chronic pancreatitis in children, seen in southern India, Indonesia, and in some tropical regions of Africa near the equator. The population is due to malnutrition and consumption of tapioca flour, which contains toxic glycosides and is clinically similar to other types of chronic pancreatitis.
Chronic obstructive pancreatitis (25%):
(1) Pancreatic schizophrenia: The incidence of pancreatic schizophrenia in the general population is 5% to 15%, which is the most common malformation of the pancreas. Because the dorsal and ventral pancreatic primordia cannot be fused, it leads to pancreatic tail, pancreatic body and Part of the pancreatic head drains through the relatively narrow accessory pancreatic duct, rather than through the main pancreatic duct. Many scholars believe that pancreatic schizophrenia is associated with recurrent pancreatitis. ERCP can diagnose pancreatic schizophrenia, and nipple sphincter incision can help.
(2) Abdominal trauma: After abdominal trauma, the recessive injury of the pancreatic duct can lead to stenosis, pseudocyst formation and chronic obstruction.
Idiopathic fibrotic pancreatitis (30%):
Idiopathic fibrotic pancreatitis is rare and can have abdominal pain or obstructive jaundice, and diffuse fibrous tissue hyperplasia can be seen in the glands.
Other (10%):
Hyperlipidemia type I, IV, V patients can develop pancreatitis, transient hyperlipidemia can occur in pancreatitis, so elevated blood lipids in acute pancreatitis must be retested after improvement, other causes include gallbladder Fibrosis, sclerosing cholangitis, inflammatory bowel disease, etc.
Pathogenesis
There are two main pathological types of chronic pancreatitis: calcification and obstructiveness, both of which are rare in children. In children, chronic calcific pancreatitis is seen in hereditary pancreatitis and idiopathic pancreatitis. The pancreas is hard and can be touched with calcium and stone during surgery. When a sticky plug containing various proteins such as digestive enzymes, mucopolysaccharides and glycoproteins is fused in the lumen of the catheter, occlusion occurs, and calcium carbonate precipitates to form intraductal stones. Toxic metabolites aggravate damage to the pancreas.
Trypsin is a glycoprotein that prevents the precipitation of calcium salts in normal pancreatic juice. Chronic ethanol intake and protein deficiency reduce the synthesis of trypsin, decreased levels of pancreatic protein and the formation of secondary calcium stones. There are many common pathways leading to calcific pancreatitis.
Congenital malformations or acquired diseases such as tumors, fibrosis or damaging stenosis, obstructive pancreatitis occurs when the main pancreatic duct is blocked, inflammation occurs in the pancreatic epithelium, is replaced by fibrous tissue, free radicals and antioxidants are deficient in chronic pancreas Both the formation and development of inflammation play an important role.
Prevention
Prevention of chronic pancreatitis in children
Hereditary chronic recurrent pancreatitis should be a good consultation for genetic diseases; adolescent tropical pancreatitis should prevent protein malnutrition, balance diet; prevent hyperlipidemia, hypercalcemia; prevent vitamin D poisoning and overnutrition; Thorough treatment of acute pancreatitis to prevent the disease from delaying.
Complication
Pediatric complications of chronic pancreatitis Complications, diabetic pancreatic cancer
Often complicated by absorption disorders and growth dysplasia, and diabetes, pancreatic cancer and so on.
Symptom
Symptoms of chronic pancreatitis in children Common symptoms Vitamin deficiency Diabetic abdominal pain Abnormal liver function Fat-soluble vitamin deficiency Nausea and vomiting Fatty tissue necrosis
The common initial clinical symptoms in patients with chronic pancreatitis are recurrent acute pancreatitis, which gradually causes damage to the pancreas and progressively weakens the function of the pancreas, but in some patients this process of worsening the disease Not obvious, there may be no pain or other special manifestations, only when this progressive latent lesion develops to the middle or late stage, the clinical manifestations of the patient are obvious when pancreatic insufficiency occurs.
Abdominal pain
Most children with chronic pancreatitis have intermittent or chronic abdominal pain. The cause of the pain is not very clear. It is mainly manifested in the upper abdomen, quarter ribs or around the umbilicus. There is also pain in the back. Vomiting and fever are not common, sometimes pain. It can last for a few weeks, and the degree of pain varies, but as the disease progresses, the pain can be gradually reduced.
2. Absorption disorders and dysplasia
This clinical manifestation is mainly due to postprandial abdominal pain, which limits dietary intake and insufficient nutrient supply. In addition, the poor function of pancreas affects the absorption of fat and protein, and steatorrhea is more than protein diarrhea. Significantly reduced, although there is severe steatorrhea, fat-soluble vitamin absorption disorder is less common, only the absorption of vitamin B12 is more obvious, blood lipids and urinary amino acids are increased.
3. Diabetes
Abnormal glucose metabolism is impaired by the release of insulin and glucagon, which can sometimes occur early in the disease. Diabetes is the most common clinical manifestation of adolescent tropical pancreatitis in calcified pancreatitis and alcoholic pancreatitis It can also be seen that while insulin secretion is reduced due to islet cell damage, dysfunction caused by fibrosis around the islets reduces insulin release into the blood.
4. Malignant tumor of the pancreas
For some patients with chronic pancreatitis, the incidence of pancreatic cancer is significantly increased. The level of this risk varies with the cause, and may be the natural outcome of some chronic pancreatitis. There are no reports of pancreatic malignancies in patients with pancreatitis.
5. Other
Chronic pancreatitis with ascites is rare, with pleural effusion is more rare, and some may cause pancreatic adipose tissue necrosis due to the escape of pancreatic enzyme into the skin, forming erythema and nodules, and some patients may have abdomen The mass may be an inflammatory mass of the pancreas, or it may be a pseudocyst or a benign or malignant tumor of the pancreas.
Many patients have a history of recurrent acute pancreatitis. Abdominal pain is the most prominent and common symptom. In most cases, the severity of abdominal pain is reduced and the duration is reduced over time. In calcified pancreatitis, with The calcification of the pancreas, the pain is alleviated, but the endocrine function is insufficient. When 98% of the pancreatic function is lost, the exocrine function is insufficient. The lack of pancreatic exocrine function can lead to malnutrition, bulimia and growth disorders. Lack of nutrition, especially fat-soluble vitamins, vitamin B12 and the lack of essential fatty acids, steatorrhea can occur in severe cases, the amount of feces in children is significantly increased, fecal acid stench or stench, diabetes can occur, but generally not serious.
The age of onset of hereditary pancreatitis is more common in 5 to 10 years old. Because of family history, the diagnosis is very easy. The patient has severe abdominal pain with nausea, vomiting, physical examination and clinical process similar to acute pancreatitis caused by other causes. Acute Symptoms relieved after 4 to 8 days, and pancreatic tissue damage will be aggravated after each episode. The interictal period is generally acceptable. The asymptomatic period can be several weeks or years. When developing chronic pancreatitis, amylase and Lipase can be normal in acute attacks, CT or ultrasonography shows a shrinking, calcified pancreas with ductal dilatation, ERCP can be found in dilated or narrow ductal stones, many years later, develop pancreatic insufficiency, late complications including diabetes, Arterial thrombosis, pancreatic cancer and other tumors in the abdomen.
Examine
Examination of chronic pancreatitis in children
Laboratory inspection
1. General laboratory inspection
(1) Serum amylase may increase in the acute attack period, but in most cases, serum amylase does not increase due to pancreatic juice secretion disorder.
(2) Fecal microscopy has a large amount of fat droplets and undigested muscle fibers.
(3) Some cases were positive for urine sugar reaction and glucose tolerance test.
2. Pancreatic external secretion function test
(1) Pancretiny-secretin test (PS test): It is the only method for directly detecting pancreatic secretion function in chronic pancreatitis when it is not serious. Perform PS sensitivity test before the test. After 12 hours of fasting, the intramuscular injection of metoclopramide and diazepam was performed before the intraoperative injection of 2% tetracaine in the throat. The gastrointestinal double lumen was inserted under the monitor of the fluorescent screen. The gastric drainage hole was located in the antrum of the stomach. Located at the lower end of the descending part of the duodenum, when the duodenal juice became clear and alkaline reaction, 10 to 20 minutes of duodenal juice was collected for experimental control, intravenous injection of secretin 2 ~ 3U / kg, each The duodenal juice was collected once every 10 minutes for 2 times. Then, the secretin was slowly injected intravenously with 1 to 2 U/kg, and the duodenal juice was collected once every 10 minutes for 3 times. The normal range of the test.
After administration of trypsin or secretin, the pancreatic juice outflow can occur, and the bicarbonate and pancreatic enzyme excretion are lower than normal.
(2) benzoyl-tyrosyl-p-aminobenzoic acid test (BT-PABA test, bentimomide test): 3 days before the test, trypsin, sulfonamides, vitamin B2 and other drugs that have an effect on the test are prohibited. Fasting in the evening, oral BT-PABA 15mg/kg (about PABA 5 mg/kg), can be taken with normal breakfast, can drink water regularly, collect 6h urine, determine the content of PABA, the result is the amount of PABA excreted in urine The ratio of taking PABA indicates that under normal circumstances, the 6-month urine PABA excretion rate is 60% to 87%, with an average of 70%, and the discharge rate of children with chronic pancreatitis is <60%. This test is more accurate in the advanced stage (sensitivity is about 70). %), but the sensitivity of early or mild chronic pancreatitis is low, and false positives may occur in hepatobiliary diseases, kidney diseases, and small bowel diseases.
Film degree exam
1. Abdominal plain film can be seen in pancreatic calcification or pancreatic duct stones.
2. B super visible pancreatic calcification, pancreatic duct stones, pancreatic duct dilatation, pancreatic localized or diffuse enlargement or atrophy, pancreatic pseudocyst and other changes.
3. CT scan showed irregular contour of the pancreas, pancreatic calcification, expansion or irregularity of the pancreatic duct, and atrophy of the gland.
4. Endoscopic retrograde cholangiopancreatogra-
Phy, ERCP) Pancreatic duct angiography shows that the lumen is dilated or irregularly beaded, with calcification or stones, and pseudocysts.
5. Pancreatic angiography Pancreatic angiography through the splenic artery or duodenal mesenteric angiography can be seen in the internal pancreatic artery segmental stenosis or occlusion or aneurysm, the pancreatic vein can also be narrow or occlusion.
Diagnosis
Diagnosis and diagnosis of chronic pancreatitis in children
diagnosis
Chronic pancreatitis is easy to diagnose according to the patient's typical history of pancreatitis and chronic signs in imaging. More typical patients have recurrent abdominal pain, vomiting and elevated serum amylase. Some patients have malabsorption and growth. Barriers, fat-soluble vitamin deficiency is rare.
1. Pancreatic enzyme assay: Blood amylase and blood lipase are elevated during acute attack.
2. Glucose intolerance: Lack of fat-soluble vitamins, hypoglobulinemia, abnormal liver function.
3. Pancreatic function test: such as radionuclide fat test, CCK-secrelin, BT-PABA test, etc., without any pancreatic function test, it is sensitive enough to diagnose mild early or moderate pancreatitis.
4. Abdominal plain film: The diagnosis of chronic pancreatitis is established when abdominal pancreatic calcification is seen in the abdominal plain film.
5. ERCP: The highest sensitivity for the diagnosis of chronic pancreatitis.
Differential diagnosis
It is differentiated from other causes such as steatorrhea, diabetes, upper abdomen mass, and jaundice.
