Primary Immunodeficiencies Overview Quiz

A group of disorders where the immune system is weakened due to genetic defects, present from birth, often manifesting in early childhood with frequent infections.

A classification of primary immunodeficiency that involves problems with phagocytes, the cells that engulf and destroy pathogens.

A rare genetic disorder characterized by impaired phagocytes, leading to recurrent infections, especially with fungal organisms like Aspergillus.

A classification of primary immunodeficiency that involves issues with the complement system, a group of proteins essential for immune responses.

A classification of primary immunodeficiency that involves defects in B lymphocytes, the cells responsible for producing antibodies.

A classification of primary immunodeficiency that involves problems with T lymphocytes, the cells responsible for cell-mediated immunity.

A classification of primary immunodeficiency where both B and T cell functions are impaired.

A treatment option for primary immunodeficiency, involving the replacement of defective cells with healthy stem cells.

A group of genetic disorders that affect the body's ability to fight infections. They are classified based on the specific component of the immune system affected.

Patients with this condition present with recurrent infections, failure to thrive, developmental delay, and may have severe bacterial infections. The onset of symptoms starts early in life.

A genetic disorder that affects the development of B cells, resulting in a deficiency of antibodies. It is an X-linked recessive disorder, meaning that males are more likely to be affected.

A rare genetic disorder that affects the development of the thymus, leading to a deficiency of T cells. This results in a weakened immune system and an increased risk of infections.

A group of severe disorders that affect both T and B cells, resulting in a severely weakened immune system. These patients are highly susceptible to infections and often require bone marrow transplantation for treatment.

A treatment approach that involves the use of antibiotics to prevent infections in individuals with primary immunodeficiency.

A rare inherited condition that affects the complement system. It is associated with certain bacterial infections, particularly Neisseria meningitidis.

A specific type of SCID caused by a deficiency in the enzyme adenosine deaminase (ADA). This deficiency affects the development and function of T cells and B cells.

A type of immunodeficiency that develops after birth due to various factors, such as infections, medications, or malnutrition.

A group of drugs used to modify the immune system's response, either to suppress it (e.g., in autoimmune diseases) or to enhance it (e.g., in organ transplantation).

The process by which HIV infection progresses over time, eventually leading to AIDS. It involves stages of viral replication, immune system decline, and opportunistic infections.

A type of virus that primarily targets the immune system's CD4+ T cells, which are crucial for fighting infections. This weakens the immune system and makes the body vulnerable to opportunistic infections.

A type of HIV that is found predominantly in West Africa.

The process by which HIV enters a cell by binding to its CD4 receptor and a co-receptor (CCR5 or CXCR4), leading to the fusion of the viral envelope with the cell membrane and the entry of the viral genome.

The enzyme encoded by the HIV virus that transcribes its RNA genome into DNA. This is a critical step in the viral replication cycle.

The enzyme encoded by the HIV virus that integrates the newly synthesized DNA into the host cell's genome, allowing the viral genes to be expressed and produce new viral particles.

HIV mutations in variable loops of gp120 protein can escape antibody recognition without compromising viral fitness.

HIV can evade immune responses by blocking antibody access to epitopes by using different strategies like glycosylation, extending variable loops, oligomerization, and steric hindrance.

HIV Nef protein downregulates MHC class I expression on infected cells, making them invisible to cytotoxic T lymphocytes (CTLs).

HIV mutations in internal proteins like Tat and protease can lead to resistance to CTL response and may potentially reduce viral fitness.

HIV can establish a latent state in resting T cells, where it does not replicate, allowing it to evade immune clearance.

Mutations in the CCR5 gene can confer resistance or delay the progression of HIV infection. A truncated CCR5 receptor prevents the virus from entering the cell.

The Berlin patient received a bone marrow transplant from a donor with a mutated CCR5 gene, which resulted in the removal of HIV from his body.

HAART (highly active antiretroviral therapy) is a combination of antiviral drugs that can effectively control HIV infection, delaying or preventing progression to AIDS.

The period during which HIV infection is present but no symptoms are experienced. It can last for years and is characterized by a low level of virus in the body.

The period during which HIV infection causes noticeable symptoms, such as fever, weight loss, and skin infections. Immune function is weakened during this stage.

The final stage of HIV infection, where the immune system is severely compromised. This is characterized by opportunistic infections, cancers, and a high risk of death.

A large, rapid loss of immune cells (specifically CD4+ T cells) in the gut-associated lymphoid tissue (MALT) during the initial phase of HIV infection.

A specific type of HIV that primarily targets cells expressing the CCR5 receptor, found on the surface of some immune cells (like T cells and macrophages). This is the most common type of HIV.

A specific type of HIV that targets cells expressing both CCR5 and CXCR4 receptors. This type of HIV is often associated with faster progression to AIDS.

A complex combination of antiretroviral drugs used to suppress HIV replication and manage HIV infection. It can improve quality of life and slow the progression to AIDS.

The ability of HIV to escape immune surveillance and continue to replicate in the body, despite immune responses. This is due to the high mutation rate of HIV.