(D81.7) Major histocompatibility complex class ii deficiency

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37 763 in individuals diagnosis major histocompatibility complex class ii deficiency confirmed

2 729 deaths with diagnosis major histocompatibility complex class ii deficiency

7% mortality rate associated with the disease major histocompatibility complex class ii deficiency

Etiology Pathogenesis Clinical Pattern Diagnostics Treatment Prevention

Diagnosis major histocompatibility complex class ii deficiency is diagnosed Men are 25.48% more likely than Women

23 693

Men receive the diagnosis major histocompatibility complex class ii deficiency

926 (3.9 %)

Died from this diagnosis.

100

14 070

Women receive the diagnosis major histocompatibility complex class ii deficiency

1 803 (12.8 %)

Died from this diagnosis.

Risk Group for the Disease major histocompatibility complex class ii deficiency - Men aged 20-24 and Women aged 0-5

In Men diagnosis is most often set at age 0-14, 20-89

Less common in men the disease occurs at Age 15-19, 90-95+Less common in women the disease occurs at Age 55-59, 90-95+

In Women diagnosis is most often set at age 0-54, 60-89

Disease Features major histocompatibility complex class ii deficiency

Absence or low individual and public risk

Major histocompatibility complex class ii deficiency - what does this mean

Major histocompatibility complex class ii deficiency is a rare genetic disorder caused by a mutation in one or both of the mhcii genes, which are responsible for producing proteins that help regulate the immune system. this leads to an impaired ability to fight off infections, resulting in recurrent and severe respiratory and gastrointestinal infections.

What happens during the disease - major histocompatibility complex class ii deficiency

Major histocompatibility complex class ii deficiency is an inherited disorder caused by a genetic mutation in the hla-dqa1 and hla-dqb1 genes. this mutation results in a lack of functioning mhc class ii molecules, which are responsible for the recognition of foreign antigens by the immune system. without functioning mhc class ii molecules, the immune system is unable to recognize and respond to foreign antigens, leading to an increased risk of infection, autoimmune disorders, and other health complications.

Clinical Pattern

More details coming soon

How does a doctor diagnose

Blood test to check for MHC-II deficiency
Genetic testing to identify a gene mutation
Immunological testing to assess the level of immune response
Flow cytometry to measure the number of MHC-II molecules
Skin biopsy to check for MHC-II expression
X-ray imaging to check for organ involvement
CT scan to look for any abnormalities

Treatment and Medical Assistance

Main goal of the treatment: To improve the functioning of the immune system

Administering immunoglobulin to provide temporary immunity
Replacing the deficient gene with a normal gene through gene therapy
Administering antibiotics to treat infections
Providing nutritional supplements to boost the immune system
Administering immunosuppressants to reduce inflammation
Carrying out regular blood tests to monitor the progress of the treatment
Providing psychological support to reduce stress and anxiety

19 Days of Hospitalization Required

48 Hours Required for Outpatient Treatment

Major histocompatibility complex class ii deficiency - Prevention

Major histocompatibility complex class ii deficiency can be prevented by avoiding exposure to environmental factors that may trigger the disease, such as certain types of infections, as well as by maintaining a healthy lifestyle, including a balanced diet and regular exercise. vaccination against certain infections may also be beneficial in preventing the disease.

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