primary intestinal lymphangiectasia
Introduction
Introduction to primary small intestinal lymphatic dilatation Primary enteric lymphangiectasis (primaryenteric lymphangiectasis) is characterized by structural defects in the small intestinal mucosa that result in lymphatic vessel dilatation and functional obstruction, and do not normally accept chylomicrons and lymphatic drainage. basic knowledge The proportion of illness: 0.005% Susceptible people: no special people Mode of infection: non-infectious Complications: edema
Cause
Causes of primary intestinal lymphangiectasis
Primary factor (35%):
Congestion of congenital small intestinal lymphatic drainage, the intracranial chyle and other small intestinal lymphatic vessels such as the serosal layer and the mesenteric lymphatic vessels are expanded, the range depends on the level of obstruction, and the pressure in the intestinal lymphatic vessels is increased due to obstruction, which damages the chylomicrons. And the absorption of fat-soluble vitamins also hinders the recirculation of small intestinal lymphocytes into the peripheral blood. A large amount of small intestinal lymph leaks into the intestinal lumen, which can form true lymphatic vessels - small intestinal fistula, small intestinal lymph, chylomicrons, proteins and lymphocytes. Directly into the intestinal lumen, causing loss of protein and lymphocytes, chylomicrons are isolated from the lamina propria and dilated lymphatic vessels, lymphatic vessels of the serosa and mesentery are dilated, leakage can cause ascites in the chyle, and obstruction of the thoracic duct leads to chylothorax Fluid.
Secondary factors (65%):
There are many causes of secondary small bowel lymphadenopathy, including extensive abdominal or retroperitoneal lymphoma, retroperitoneal fibrosis, chronic pancreatitis, mesenteric tuberculosis or sarcoidosis, Crohn's disease, and even constrictive pericarditis and chronic Congestive heart failure, primary intestinal lymphatic dilatation, also known as Milroy disease, is caused by the developmental deformity of the lymphatic vessels, often involving lymphatic vessels in other parts of the body.
Prevention
Primary small bowel lymphadenopathy prevention
There is no effective preventive measure for this disease. Early detection and early diagnosis are the key to the prevention and treatment of this disease.
Complication
Primary intestinal lymphangiectasis complications Complications edema
Fatty diarrhea causes loss of vitamin D. Severe steatorrhea can cause low calcium convulsions. Macular edema can cause blindness, but it is rare.
Symptom
Symptoms of primary small bowel lymphadenopathy common symptoms diarrhea edema hypoproteinemia malabsorption syndrome convulsions steatorrhea
Edema and diarrhea are the main symptoms. The edema begins intermittently and then becomes persistent. In a few cases, the edema is asymmetrical. Asymmetry edema starts from the infant or childhood, and is mostly congenital Milroy disease, such as occurs in the upper arm. There may be yellow nails, because the patient has a large amount of protein loss, causing severe hypoproteinemia, and later edema becomes symmetrical. Most patients have mild steatorrhea and malabsorption syndrome, such as appearing within 10 years old, there will be Stunting, most patients have diarrhea, about half of the patients have chyle in the stool, fat diarrhea causes vitamin D loss, severe steatorrhea can cause low calcium convulsions, macular edema can cause blindness, but rare, in the course of the disease, about half Abdominal or thoracic chyle effusion may occur, and hypocholesterolemia may be distinguished from ascites of nephrotic syndrome.
The loss of immunoglobulin causes a significant decrease in blood IgA, IgM and IgG, only 50% of normal people, but the patient's response to the antigen is still intact, can produce the corresponding antibodies, lymphocyte leakage can cause peripheral blood lymphocytes Reduction, this is a characteristic clinical manifestation. The loss of lymphocytes is mainly T cells, which can seriously affect the cellular immune function. Although the immune function is low, opportunistic infections are rare, and 75% of small intestinal X-ray examinations show abnormalities. The Conniventes flap is large and thick due to edema, with increased secretion and a slight expansion of the intestine. At this time, lymphangiography is almost abnormal.
Examine
Examination of primary small intestinal lymphatic dilatation
Intestinal mucosa biopsy: pathologically, the structure of the intestinal villi is severely distorted, and the central chyle tube of the villi is obviously dilated. It contains lipid-rich macrophages. Under electron microscope, there are chylomicrons in the extracellular space between the intestinal cells and the lamina propria.
Small bowel X-ray examination is often abnormal, showing improvement of mucosal edema and malabsorption. Lymphatic angiography shows peripheral and visceral lymphatic dysplasia and absence of retroperitoneal lymph nodes. The biopsy features of jejunal mucosa have an intrinsic membrane and submucosal lymphatic expansion because The obvious retention of the dilated lymphatic vessels makes the villi a rod.
Diagnosis
Diagnosis and diagnosis of primary small intestinal lymphatic dilatation
Young patients have edema, especially asymmetry, with hypoproteinemia. Those who cannot explain liver disease or kidney disease should be suspected of this disease; if there is both a decrease in immunoglobulin and a decrease in lymphocytes in peripheral blood, it is more likely. Intestinal protein loss can be detected by 131I, or 51Cr-albumin. The diagnosis of this disease depends on small intestinal mucosal biopsy. The pathologically seen intestinal villus structure is severely distorted. The central chyle tube of the villi is obviously dilated, which is filled with lipid. Macrophages, under the electron microscope, there are chylomicrons in the extracellular space between intestinal cells and lamina propria, which are diagnostic.
Celiac disease is caused by allergy to gluten, so it is also known as gluten allergic bowel disease. The gluten is mainly found in wheat, but all cereals except millet and corn have different degrees. Gluten contains gluten. (Glutenin) and gliadin are toxic, which can reduce the formation and secretion of digestive enzymes, which can cause dull and thick intestinal villi, infiltration of lamina propria, and decrease of intestinal mucosal absorption area, resulting in sugar, protein, Absorption of fat, vitamins, inorganic salts, trace elements and water, and complex clinical symptoms, often accompanied by anemia, cyanosis, osteomalacia, osteoporosis, muscle weakness, peripheral neuralgia, secondary parathyroid function Decreased, extrahepatic manifestations such as hyperparathyroidism.
