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.

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