Primary Immunodeficiency Disorders (PID)
Primary Immunodeficiency Disorders (PID) are a group of inherited disorders characterized by a malfunction or deficiency in the immune system, leaving individuals more susceptible to infections. These disorders affect various components of the immune system, including white blood cells, antibodies, and complement proteins.
Types of Primary Immunodeficiency Disorders (PID) :
Common Variable Immunodeficiency (CVID):
CVID is characterized by low levels of serum immunoglobulins and an increased susceptibility to bacterial, viral, and fungal infections.
Selective IgA Deficiency:
Individuals with this disorder have low levels of immunoglobulin A (IgA), leading to recurrent respiratory, gastrointestinal, and other infections.
X-Linked Agammaglobulinemia (XLA):
XLA primarily affects males and is characterized by the absence of mature B cells and immunoglobulins, resulting in severe and recurrent bacterial infections.
Severe Combined Immunodeficiency (SCID):
SCID is a group of disorders characterized by a severe impairment in both T cell and B cell immunity, leading to life-threatening infections early in life.
Hyper IgM Syndrome:
This disorder results from a defect in class-switch recombination, leading to elevated levels of IgM and reduced levels of other immunoglobulins, resulting in recurrent bacterial infections.
DiGeorge Syndrome:
DiGeorge Syndrome is caused by a deletion of a portion of chromosome 22, resulting in abnormalities in the development of the thymus and parathyroid glands, leading to immune deficiencies and other health issues.
Chronic Granulomatous Disease (CGD):
CGD is characterized by defects in the phagocytic cells’ ability to destroy certain bacteria and fungi, leading to recurrent infections and the formation of granulomas.
Wiskott-Aldrich Syndrome:
This X-linked disorder affects males and is characterized by eczema, low platelet counts, and immune deficiencies, leading to an increased susceptibility to infections and an increased risk of developing autoimmune disorders and cancer.
Types of Primary Immunodeficiency Disorders (PID) :
Common Variable Immunodeficiency (CVID):
CVID is characterized by low levels of serum immunoglobulins and an increased susceptibility to bacterial, viral, and fungal infections.
Selective IgA Deficiency:
Individuals with this disorder have low levels of immunoglobulin A (IgA), leading to recurrent respiratory, gastrointestinal, and other infections.
X-Linked Agammaglobulinemia (XLA):
XLA primarily affects males and is characterized by the absence of mature B cells and immunoglobulins, resulting in severe and recurrent bacterial infections.
Severe Combined Immunodeficiency (SCID):
SCID is a group of disorders characterized by a severe impairment in both T cell and B cell immunity, leading to life-threatening infections early in life.
Hyper IgM Syndrome:
This disorder results from a defect in class-switch recombination, leading to elevated levels of IgM and reduced levels of other immunoglobulins, resulting in recurrent bacterial infections.
DiGeorge Syndrome:
DiGeorge Syndrome is caused by a deletion of a portion of chromosome 22, resulting in abnormalities in the development of the thymus and parathyroid glands, leading to immune deficiencies and other health issues.
Chronic Granulomatous Disease (CGD):
CGD is characterized by defects in the phagocytic cells’ ability to destroy certain bacteria and fungi, leading to recurrent infections and the formation of granulomas.
Wiskott-Aldrich Syndrome:
This X-linked disorder affects males and is characterized by eczema, low platelet counts, and immune deficiencies, leading to an increased susceptibility to infections and an increased risk of developing autoimmune disorders and cancer.
Common Symptoms:
Recurrent infections, including bacterial, viral, and fungal infections
Failure to thrive or poor growth
Chronic Diarrhoea or gastrointestinal issues
Persistent skin rashes or infections
Delayed wound healing
Autoimmune conditions
Family history of immune disorders
Treatments:
Immunoglobulin Replacement Therapy (IVIG)
Antibiotic Therapy
Stem Cell Transplantation
Gene Therapy
Supportive Care
Frequently Asked Questions
Can PID be cured?
PID is typically managed rather than cured. Treatment aims to control symptoms, prevent infections, and improve quality of life.
Is PID hereditary?
Many forms of PID are inherited, meaning they are passed down from parents to their children through genes.
Can individuals with PID live normal lives?
With appropriate management and treatment, many individuals with PID can lead relatively normal lives.
Are there any lifestyle changes recommended for individuals with PID?
Maintaining good hygiene practices, avoiding contact with sick individuals, and staying up-to-date with vaccinations are recommended lifestyle changes for individuals with PID.
Can individuals with PID receive vaccinations?
While some vaccinations may be safe for individuals with PID, others, such as live vaccines, may be contraindicated. It is essential to consult with a healthcare provider for personalized recommendations.
What is the long-term outlook for individuals with PID?
The long-term outlook for individuals with PID varies depending on the specific disorder, its severity, and the effectiveness of treatment. Regular monitoring and management are crucial for optimizing outcomes.
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