C5 insufficiency syndrome
Introduction
Introduction to C5 dysfunction syndrome C5 dysfunction syndrome, also known as familial C5 dysfunction, was first reported by Miller in 1968. Mainly found in infants, characterized by a normal C5 content in serum, but functional defects. basic knowledge The proportion of illness: 0.001% Susceptible people: mainly found in babies Mode of infection: non-infectious Complications: suppurative meningitis
Cause
Causes of C5 dysfunction syndrome
(1) Causes of the disease
Autosomal abnormalities, which are dominant or recessive, have not yet been determined, and there is a familial trend.
(two) pathogenesis
Complement in the host's immune response to pathogenic microorganisms, mainly through immunomodulation, enhance the chemotaxis and immune adhesion of phagocytic cells (including neutrophils), play and dissolve antigenic effects, when the primary nature of complement When the immune function is incomplete, the susceptibility to various infections is increased, and various autoimmune diseases are easily secondary. This disease is caused by C5 insufficiency and chemokine production disorder in serum, but the specific pathogenesis is still unknown.
Prevention
C5 dysfunction syndrome prevention
Avoid overwork and exercise properly. The purpose of physical exercise is to improve the body's ability to resist disease and promote the recovery of the body. You can choose the appropriate exercise method according to your own specific situation, but the amount of exercise should not be too large, you must persist every day, persevere, in order to receive good results.
Complication
C5 dysfunction syndrome complications Complications, purulent meningitis
In severe cases, it may be accompanied by exfoliative exudative dermatitis, sepsis, and purulent meningitis.
Symptom
C5 dysfunction syndrome symptoms common symptoms thin diarrhea
Sebum exudative dermatitis occurs from infancy, and can start from any part, quickly spread to the whole body, accompanied by chronic diarrhea, weight loss, low living ability, due to poor anti-infective ability of patients, it is easy to have repeated bacterial infections ( Gram-negative bacteria, such as otitis media, pneumonia, enteritis.
Examine
Examination of C5 dysfunction syndrome
Serum total complement (CH50) and C5 levels, swimming motility and antigen were in the normal range, C5 activity decreased, and the serum of children could not enhance the phagocytosis of granulocytes and staphylococci by normal phagocytic cells. Other neutrophils function normally, but some cases may be associated with T cell dysfunction and hyperglobulinemia.
Diagnosis
Diagnosis and identification of C5 dysfunction syndrome
According to infant onset, clinical manifestations and laboratory tests confirmed.
