60 Questions
Which of the following is a classification system for immunodeficiency?
Primary vs secondary
What is the term used to describe a temporary deficiency of antibodies in infants?
Transient hypogammaglobulinemia of infancy
Which of the following is an example of an antibody deficiency syndrome?
XLA
What is the term used to describe immunodeficiency caused by defects in (T) cell-mediated immunity?
SCID
Which immunodeficiency syndrome is characterized by a deficiency in the terminal complement pathway?
Terminal complement pathway deficiency
What type of infections are commonly associated with immunodeficiency?
Opportunistic
What is the term used to describe infections that are unusually severe, protracted, or not responding to standard therapy?
Severe
What is the term used to describe infections that occur frequently in individuals with immunodeficiency?
Frequent
What are the key clinical features of XLA (X-linked agammaglobulinemia)?
Deficiency of B cells
What are the key clinical features of SCID (Severe Combined Immunodeficiency)?
Deficiency of (T) cells
Which of the following is true about opportunistic infections?
They are usually severe and protracted
What is the classification of immunodeficiency where the immune defect is secondary to another disease process?
Secondary immunodeficiency
Which age group is more susceptible to developing immunodeficiency due to extremes of age?
Infants
What is the main type of infection associated with antibody-deficiency immunodeficiency?
Bacterial infections
Which type of immunodeficiency affects both antibody production and T cells?
Combined immunodeficiency
What is the term used to describe age-related changes in the immune system that result in greater susceptibility to infection and reduced response to vaccination?
Immunosenscence
What is the main factor contributing to reduced T and B cell receptor diversity in immunosenescence?
Telomere shortening
Which of the following is NOT a characteristic of immunosenescence?
Expansion of T cell pool
What is the current research focus regarding immunosenescence and T cells?
Cytomegalovirus
What is the term used to describe immune dysregulation in immunodeficiency syndromes, which manifests as uncontrolled inflammation and autoimmune diseases?
Immune dysregulation
Which of the following is a common immunological condition in older people characterized by low levels of immunoglobulin G (IgG)?
Antibody deficiency
Which of the following disorders is caused by a deficiency of Bruton's tyrosine kinase (Btk)?
X-Linked agammaglobulinemia
Which disorder is characterized by a deficiency of CD40 ligand on T cells, impairing affinity maturation of B cells and resulting in recurrent bacterial infections?
X-Linked hyper-IgM syndrome
Which type of immunodeficiency refers to deficiencies in CD4 T cells and is often associated with opportunistic, viral, fungal, and mycobacterial infections?
Cellular immunodeficiency
Which disorder is characterized by absent or non-functional T cells and often presents with rash, failure to thrive, chronic diarrhea, and various infections?
Severe combined immunodeficiency (SCID)
Which of the following is a common cause of Severe combined immunodeficiency (SCID)?
All of the above
Which disorder is caused by migration defects in the 3rd and 4th branchial arches and is characterized by absent parathyroids, cleft palate, congenital heart defects, and thymic aplasia?
DiGeorge syndrome
Which deficiency leads to specific susceptibility to Neisseria species, while the rest of the immune system remains intact?
Deficiency of terminal complement components C5-C9
Which of the following is not a primary antibody deficiency disorder?
Antibody deficiency
Which type of immunodeficiency can be treated with stem cell transplantation or gene therapy to reconstitute T and B cell populations?
Severe combined immunodeficiency (SCID)
Which antibody deficiency is primarily characterized by low levels of IgG?
IgG deficiency
What is the cardinal feature of antibody deficiency?
Recurrent bacterial infections
What is the potential consequence of not treating antibody deficiency early and aggressively?
Irreversible lung damage (bronchiectasis)
Which condition can cause secondary antibody deficiency?
Nephrotic syndrome
Which of the following is true about primary immunodeficiencies?
They are caused by defects in the immune system itself.
Which age group is more susceptible to developing immunodeficiency due to extremes of age?
Elderly
What is the term used to describe immune dysregulation in immunodeficiency syndromes, which manifests as uncontrolled inflammation and autoimmune diseases?
Autoinflammation
What is the main type of infection associated with antibody-deficiency immunodeficiency?
Bacterial infections
Which of the following is an example of an opportunistic infection that may suggest immunodeficiency?
Candida infection in the eye
Which of the following infections is not considered abnormal in individuals without immunodeficiency?
Cold sores
What type of infections are characteristic of immunodeficiency?
Unusual and severe infections
What factors can influence infection frequency in individuals with immunodeficiency?
All of the above
Which of the following is a characteristic of immunosenescence?
Greater susceptibility to infection
Which type of immunodeficiency affects both antibody production and T cells?
Combined immunodeficiency
What is the term used to describe a temporary deficiency of antibodies in infants?
Transient hypogammaglobulinemia of infancy
Which disorder is characterized by absent or non-functional T cells and often presents with rash, failure to thrive, chronic diarrhea, and various infections?
Severe Combined Immunodeficiency (SCID)
Which of the following is NOT a primary immunodeficiency syndrome?
Transient hypo gamma anaemia of infancy
What is the term used to describe a temporary deficiency of antibodies in infants?
Transient hypo gamma anaemia of infancy
Which disorder is caused by migration defects in the 3rd and 4th branchial arches and is characterized by absent parathyroids, cleft palate, congenital heart defects, and thymic aplasia?
DiGeorge syndrome
Which of the following is true about opportunistic infections?
They are commonly associated with immunodeficiency
Which antibody is the first to be produced during pregnancy?
IgM
At what age does an infant have normal adult levels of IgG?
At birth
What is the term used to describe a period of relative antibody deficiency around 6 months of age?
Transient hypo gamma globulinemia of infancy
Which type of immunodeficiency is characterized by decreased levels of IgG antibodies in infants?
Transient hypo gamma globulinemia of infancy
Which of the following is one of the two examples of primary antibody deficiency mentioned in the text?
Anaemia
Where do B cells mature?
Bone marrow
Which process is involved in the maturation of B cells?
V(D)J recombination
Which disorder is characterized by a deficiency of Bruton's tyrosine kinase (Btk)?
XLA
Which immunodeficiency syndrome is characterized by a deficiency in the terminal complement pathway?
C3 deficiency
Which type of immunodeficiency affects both antibody production and T cells?
Combined immunodeficiency
Study Notes
Immunodeficiency Disorders: Understanding the Risk Factors and Manifestations
- Elderly individuals have increased susceptibility to infection due to factors such as reduced mobility, poor nutrition, impaired wound healing, and co-morbidities.
- Antibody deficiency, characterized by low levels of immunoglobulin G (IgG), is a common immunological condition in older people, leading to recurrent pyogenic infections of the respiratory tract and sometimes gut infections.
- Antibody deficiency can be caused by physiological factors, such as transient hypogammaglobulinemia of infancy, or secondary factors like renal or skin disorders.
- Primary antibody deficiency disorders include X-Linked agammaglobulinemia, X-Linked hyper-IgM syndrome, and Common Variable Immunodeficiency.
- X-Linked agammaglobulinemia, also known as Bruton's disease, is caused by a deficiency of Bruton's tyrosine kinase (Btk), leading to a maturation arrest of B cells and absence of immunoglobulin production.
- X-Linked hyper-IgM syndrome is characterized by a deficiency of CD40 ligand on T cells, impairing affinity maturation of B cells and resulting in recurrent bacterial infections.
- Early recognition and aggressive treatment of intercurrent infections are essential in managing antibody deficiency disorders to prevent irreversible lung damage.
- Cellular immunodeficiency refers to deficiencies in CD4 T cells, often seen in conditions like advanced HIV infection, and is associated with opportunistic, viral, fungal, and mycobacterial infections.
- Severe combined immunodeficiency (SCID) is a life-threatening primary immunodeficiency characterized by absent or non-functional T cells and often presents with rash, failure to thrive, chronic diarrhea, and various infections.
- Common causes of SCID include deficiencies in common gamma chain, JAK3, and RAG1/2, which are essential for T and B cell maturation.
- SCID can be treated with stem cell transplantation or gene therapy to reconstitute T and B cell populations.
- DiGeorge syndrome, caused by migration defects in the 3rd and 4th branchial arches, is characterized by absent parathyroids, cleft palate, congenital heart defects, and thymic aplasia, leading to variable immunodeficiency and possible autoimmune manifestations.
- Deficiency of terminal complement components C5-C9 leads to specific susceptibility to Neisseria species, while the rest of the immune system remains intact. Diagnosis is done through functional complement assays.
Test your knowledge on immunodeficiency disorders with this informative quiz! Explore the risk factors, manifestations, and treatment options for various conditions such as antibody deficiency, cellular immunodeficiency, severe combined immunodeficiency (SCID), and more. Challenge yourself and learn about the complexities of the immune system.
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