(D84.1) Defects in the complement system

More details coming soon

Icon
263 079 in individuals diagnosis defects in the complement system confirmed
Icon
14 071 deaths with diagnosis defects in the complement system
Icon
5% mortality rate associated with the disease defects in the complement system

Diagnosis defects in the complement system is diagnosed Men are 4.10% more likely than Women

136 927

Men receive the diagnosis defects in the complement system

6 596 (4.8 %)

Died from this diagnosis.

100
95
90
85
80
75
70
65
60
55
50
45
40
35
30
25
20
15
10
5
0
126 152

Women receive the diagnosis defects in the complement system

7 475 (5.9 %)

Died from this diagnosis.

Risk Group for the Disease defects in the complement system - Men and Women aged 0-5

Icon
In Men diagnosis is most often set at age 0-89
Icon
Less common in men the disease occurs at Age 90-95+Less common in women the disease occurs at Age 90-95+
Icon
In Women diagnosis is most often set at age 0-89

Disease Features defects in the complement system

Icon
Absence or low individual and public risk
Icon

Defects in the complement system - what does this mean

Defects in the complement system occur when mutations in the genes that encode the proteins involved in the complement system lead to an impaired ability of the body to respond to foreign substances, resulting in an increased risk of infection, autoimmune disorders, and other inflammatory conditions.

What happens during the disease - defects in the complement system

Defects in the complement system occur when the proteins responsible for mediating the immune response are unable to function properly. this can lead to an increased risk of infection, autoimmune disorders, and other inflammatory conditions. in some cases, these defects can also lead to an increased risk of developing certain types of cancer.

Clinical Pattern

More details coming soon

How does a doctor diagnose

  • Complete blood count (CBC)
  • C3 and C4 levels
  • CH50 assay
  • Flow cytometry
  • ELISA testing
  • Western blot analysis
  • Complement receptor 1 (CR1) assay
  • Antinuclear antibody (ANA) testing
  • Immunofluorescence assay (IFA)

Treatment and Medical Assistance

Main goal of the treatment: Treating Defects in the Complement System
  • Administering intravenous immunoglobulin (IVIG) to replace missing complement proteins.
  • Providing antibiotics to treat any infections that result from the complement deficiency.
  • Administering hematopoietic stem cell transplantation (HSCT) to replace the defective stem cells.
  • Performing plasma exchange therapy to remove abnormal complement proteins.
  • Prescribing medications to suppress the immune system.
  • Providing genetic counseling to help affected individuals and their families understand the condition and its inheritance.
Icon
8 Days of Hospitalization Required
Icon
42 Hours Required for Outpatient Treatment

Defects in the complement system - Prevention

The best way to prevent defects in the complement system is to ensure that your diet is rich in essential nutrients such as vitamins, minerals, and antioxidants. eating a balanced diet, getting regular exercise, and avoiding smoking and excessive alcohol consumption can help keep the complement system functioning properly. additionally, getting regular medical check-ups and following your doctor's advice can help identify any deficiencies in the complement system before they become a problem.