benign paroxysmal peritonitis syndrome
Introduction
Introduction to benign paroxysmal peritonitis syndrome Benign Paroxysmal Peritonitis Syndrome is also known as Familial Mediterranean Fever Syndrome, hereditary familial amyloidosis, and Seigal-Cattan-Mamon syndrome. The disease is more common in the Mediterranean coastal areas, such as Jews and Arabs in Spain and Portugal. It is more common in adolescents. More men than women, children can also develop disease but are rarer than adults. basic knowledge The proportion of illness: 0.002% Susceptible people: good for young men Mode of infection: non-infectious Complications: urticaria, polyneuritis
Cause
The cause of benign paroxysmal peritonitis syndrome
The cause of this disease is unknown, may be a familial autosomal recessive hereditary disease, a type of amyloidosis.
Peritoneal congestion and edema, scattered cellulose-like substance deposition or amyloid deposition, may be accompanied by a small amount of exudate, which contains serous fibrin, neutrophils, lymphocytes, while other organs such as liver, spleen, kidney, Tissues such as heart and lung may also have amyloid deposits.
Prevention
Benign paroxysmal peritonitis prevention
The disease is a genetic disease, and prenatal and postnatal care is the best preventive measure for this disease.
Complication
Benign paroxysmal peritonitis complications Complications urticaria polyneuritis
Accompanied by urticaria, polyneuritis, multiple serositis.
Symptom
Benign paroxysmal peritonitis symptoms Common symptoms Abdominal tenderness Abdominal pain Ascites Asymmetric joint pain Bloating Low fever Chest pain
Mainly have paroxysmal fever or irregular fever, abdominal pain, abdominal distension, can be diffuse or limited to a certain part, often accompanied by chest pain, joint pain, neurological dysfunction, seizures are intermittent periodic, interictal symptoms Can be completely relieved, female patients often in the menstrual period, physical examination showed abdominal tenderness, hepatomegaly, and ascites positive.
Examine
Examination of benign paroxysmal peritonitis syndrome
1. In the attack period, white blood cells often rise, erythrocyte sedimentation rate increases, 2 globulin increases, urine protein is positive, and there may be tubular urine.
2. There may be mild abnormalities in liver function when the liver is involved.
3. The examination of the chest and ascites is serous fibrin.
4. The Congo red test was intravenously injected with 0.2% congo red solution at 0.22 ml/kg body weight. After 4 minutes and 1 hour, 10 ml of venous blood was taken for colorimetric examination. The percentage of the stain remaining in the serum was significantly reduced. Histopathological examination revealed amyloid deposition.
Diagnosis
Diagnosis and differentiation of benign paroxysmal peritonitis syndrome
diagnosis
Paroxysmal intermittent fever, abdominal pain, chest pain, joint pain, and multiple serositis in the Mediterranean region, combined with its family history should consider the possibility of this disease, because the amyloid can quickly absorb Congo red, feasible Congo red test Liver histopathological examination can confirm the diagnosis of amyloid deposition.
Differential diagnosis
Identification with acute peritonitis, retroperitoneal fibrosis and other diseases.
