About immunodeficiency-3

What is immunodeficiency-3?

Hyper-IgM Syndrome (HIM) is a rare genetic (primary) immunodeficiency disorder that is typically inherited as an X-linked recessive genetic trait. Symptoms and physical findings associated with the disorder usually become apparent in the first or second year of life. Hyper-IgM Syndrome may be characterized by recurrent pus-producing (pyogenic) bacterial infections of the upper and lower respiratory tract including the sinuses (sinusitis) and/or the lungs (pneumonitis or pneumonia); the middle ear (otitis media); the membrane that lines the eyelids and the white portions (sclera) of the eyes (conjunctivitis); the skin (pyoderma); and/or, in some cases, other areas. Individuals with Hyper-IgM Syndrome are also susceptible to "opportunistic" infections, i.e., infections caused by microorganisms that usually do not cause disease in individuals with fully functioning immune systems (non-immunocompromised) or widespread (systemic) overwhelming disease by microorganisms that typically cause only localized, mild infections. In individuals with Hyper-IgM Syndrome, such opportunistic infections may include those caused by Pneumocystis carinii, a microorganism that causes a form of pneumonia, or Cryptosporidium, a single-celled parasite (protozoa) that can cause infections of the intestinal tract. In addition, individuals with Hyper-IgM Syndrome are prone to certain autoimmune disorders affecting particular elements of the blood, such as neutropenia, a condition in which there is an abnormal decrease of certain white blood cells (neutrophils). Additional physical findings often associated with the disorder may include enlargement (hypertrophy) of the tonsils, enlargement of the liver and spleen (hepatosplenomegaly), chronic diarrhea and impaired absorption of nutrients by the intestinal tract (malabsorption), and/or other symptoms.

The range and severity of symptoms and physical features associated with this disorder may vary from case to case. Because approximately 70 percent of reported cases of Hyper-IgM Syndrome are inherited as an X-linked recessive genetic trait, the vast majority of affected individuals are male. However, some cases of autosomal recessive and autosomal dominant genetic inheritance have been reported. In addition, a rare acquired form of the disorder has been described in the medical literature.

What are the symptoms for immunodeficiency-3?

Immunodeficiency-3 (common variable 3)is a disorder that impairs the immune system.

  • People with ID-3 are highly susceptible to infection from foreign invaders such as bacteria, or more rarely, viruses, and often develop recurrent infections, particularly in the lungs, sinuses, and ears.
  • Pneumonia is common in people with immunodeficiency -3, chronic bronchitis is also sometimes seen alongside.
  • People may develop Diarrhea, problems absorbing food nutrients, reduced liver function, and impaired blood flow to the liver.
  • It may also lead to a reduced level of blood cells and lymph cells.
  • Painful joints and node swelling is another common condition.
  • Recurrent viral infections like colds, herpes, warts, condyloma, deep skin abscesses, lymph nodes or internal organs, Persistent thrush or fungal infections on the skin or elsewhere, and infections with tuberculosis-like bacteria (also known as Nontuberculous Mycobacteria) are also seen.



Conditions
Low immunity,High risk of infections
Drugs
Immunotherapy,Immunoglobin replacement therapy
Symptoms
Constant infections,Fever,Chills

What are the causes for immunodeficiency-3?

Immunodeficiency, a defect in immunity, impairs the body’s ability to resist infection. Immune disorders caused by a genetic defect are usually evident early in life.

  • Immunodeficiency 3 is caused by a variety of different genetic abnormalities that result in a defect in the capability of immune cells to produce normal amounts of all types of antibodies.
  • Only a few of these defects have been identified, and the cause of most cases of Immunodeficiency 3 is unknown. Many people with Immunodeficiency 3 carry a DNA variation called a polymorphism in a gene known as TACI.
  • However, while this genetic abnormality confers an increased risk of developing Immunodeficiency 3, it alone is not capable of causing
  • Immunodeficiency 3 is also linked to IgA deficiency, a related condition in which only the level of the antibody immunoglobulin A (IgA) is low.
  • In contrast, levels of other antibody types are usually normal or near normal.
  • IgA deficiency typically occurs alone.
  • But in some cases, it may precede the development of IMMUNODEFICIENCY 3 or occur in family members of Immunodeficiency 3 patients.


Conditions
Low immunity,High risk of infections
Drugs
Immunotherapy,Immunoglobin replacement therapy
Symptoms
Constant infections,Fever,Chills

What are the treatments for immunodeficiency-3?

Immunodeficiency 3 can be efficiently treated with immunoglobin replacement therapy. This will replenish the immunoglobins.

  • Immunoglobin replacement therapy is given at frequent intervals for life, and is thought to help reduce bacterial infections and boost immune function.
  • Before therapy begins, plasma donations are tested for known blood-borne pathogens, then pooled and processed to obtain concentrated IgG samples.
  • Infusions can be administered in three different forms: intravenously (IVIg),[19] subcutaneously (SCIg), and intramuscularly (IMIg).
  • These immunoglobins can be administered intravenously either in the veins of arms or legs.
  • Subcutaneous infusions can also be placed in the skin beneath the legs and hands.
  • Intramuscular infusions were used earlier but they are not used today.
  • Immuno competent drugs can also be given.


Conditions
Low immunity,High risk of infections
Drugs
Immunotherapy,Immunoglobin replacement therapy
Symptoms
Constant infections,Fever,Chills

What are the risk factors for immunodeficiency-3?

Inherited gene mutations most commonly cause primary immunodeficiency 3 disorders.

  • Secondary immunodeficiency disorders can be caused by a variety of things, including chronic conditions like diabetes and hypertension, some drugs, and radiation therapy.
  • The persons with a history of long-term hospitalization also face the same issue.
  • Persons with insufficient nutrition will have severe precipitations with this disease.
  • People who have a family history of primary immunodeficiency disorders have a higher risk for developing primary disorders themselves.
  • Age is another important factor in this line. As you age, the organs will have a low number of immune-competent cells.


Conditions
Low immunity,High risk of infections
Drugs
Immunotherapy,Immunoglobin replacement therapy
Symptoms
Constant infections,Fever,Chills

Is there a cure/medications for immunodeficiency-3?

Immunodeficiency 3 can be efficiently treated with immunoglobin replacement therapy. This will replenish the immunoglobins.

  • Immunoglobin replacement therapy is given at frequent intervals for life, and is thought to help reduce bacterial infections and boost immune function.
  • Before therapy begins, plasma donations are tested for known blood-borne pathogens, then pooled and processed to obtain concentrated IgG samples.
  • Infusions can be administered in three different forms: intravenously (IVIg),[19] subcutaneously (SCIg), and intramuscularly (IMIg).
  • These immunoglobins can be administered intravenously either in the veins of arms or legs.
  • Subcutaneous infusions can also be placed in the skin beneath the legs and hands.
  • Intramuscular infusions were used earlier, but they are not used today.


Conditions
Low immunity,High risk of infections
Drugs
Immunotherapy,Immunoglobin replacement therapy
Symptoms
Constant infections,Fever,Chills

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