(D80) Immunodeficiency with predominantly antibody defects

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230 829 in individuals diagnosis immunodeficiency with predominantly antibody defects confirmed
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3 237 deaths with diagnosis immunodeficiency with predominantly antibody defects
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1% mortality rate associated with the disease immunodeficiency with predominantly antibody defects

Diagnosis immunodeficiency with predominantly antibody defects is diagnosed Men are 29.67% more likely than Women

149 656

Men receive the diagnosis immunodeficiency with predominantly antibody defects

1 396 (0.9 %)

Died from this diagnosis.

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81 173

Women receive the diagnosis immunodeficiency with predominantly antibody defects

1 841 (2.3 %)

Died from this diagnosis.

Risk Group for the Disease immunodeficiency with predominantly antibody defects - Men and Women aged 0-5

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In Men diagnosis is most often set at age 0-89
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Less common in men the disease occurs at Age 90-95+Less common in women the disease occurs at Age 35-39, 85-95+
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In Women diagnosis is most often set at age 0-34, 40-84

Disease Features immunodeficiency with predominantly antibody defects

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Absence or low individual and public risk
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Immunodeficiency with predominantly antibody defects - what does this mean

Immunodeficiency with predominantly antibody defects occurs when the body is unable to produce enough antibodies to fight off infection. this type of immunodeficiency can be caused by genetic defects, certain medications, or other medical conditions.

What happens during the disease - immunodeficiency with predominantly antibody defects

Immunodeficiency with predominantly antibody defects is caused by a genetic defect in the b-cell maturation pathway, leading to a decrease in the production of functional antibodies. this defect can be caused by mutations in genes that are important for b-cell maturation, such as the genes that encode for the components of the b-cell receptor or the signaling pathways that are important for b-cell development. as a result, the patient is unable to produce functional antibodies to fight off infections, leading to recurrent and often severe infections.

Clinical Pattern

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How does a doctor diagnose

  • Complete Blood Count (CBC)
  • Serum Immunoglobulin levels
  • Flow cytometry
  • T-cell subset analysis
  • B-cell subset analysis
  • T-cell proliferation assays
  • Antibody titers to common pathogens
  • T-cell receptor excision circles (TRECs) assay
  • Genetic testing for known immune deficiencies
  • Skin testing for delayed hypersensitivity
  • Lymphocyte transformation test

Treatment and Medical Assistance

Main Goal: To improve the body's ability to fight infections
  • Administering replacement immunoglobulin
  • Administering vaccines
  • Antibiotic treatment for infections
  • Monitoring for signs of infection
  • Avoiding contact with people who are ill
  • Maintaining good hygiene
  • Avoiding contact with animals
  • Eating a healthy, balanced diet
  • Exercising regularly
  • Managing stress
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7 Days of Hospitalization Required
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126 Hours Required for Outpatient Treatment

Immunodeficiency with predominantly antibody defects - Prevention

Immunodeficiency with predominantly antibody defects can be prevented by maintaining a healthy lifestyle, including regular exercise, adequate sleep, and a balanced diet. vaccines can help prevent certain infections, and regular check-ups with a healthcare provider can help identify any underlying conditions that may increase the risk of immunodeficiency.

Specified forms of the disease

(D80.0) Hereditary hypogammaglobulinaemia
(D80.1) Nonfamilial hypogammaglobulinaemia
(D80.2) Selective deficiency of immunoglobulin A [IgA]
(D80.3) Selective deficiency of immunoglobulin G [IgG] subclasses
(D80.4) Selective deficiency of immunoglobulin M [IgM]
(D80.5) Immunodeficiency with increased immunoglobulin M [IgM]
(D80.6) Antibody deficiency with near-normal immunoglobulins or with hyperimmunoglobulinaemia
(D80.7) Transient hypogammaglobulinaemia of infancy
(D80.8) Other immunodeficiencies with predominantly antibody defects
(D80.9) Immunodeficiency with predominantly antibody defects, unspecified