Question

Kostmann syndrome is a severe congenital neutropenia. Which of the following is characteristic of this condition?

a) Severe bacterial infections
b) Bone marrow disorders
c) Severe viral infections
d) Autoimmune disorders

Answer 1

Kostmann syndrome is associated with severe bacterial infections due to a genetic mutation leading to neutrophil dysfunction. It is a primary immunodeficiency focused on neutrophils, unlike SCID, which affects multiple immune cells.

Step-by-step explanation:

Kostmann syndrome, also known as severe congenital neutropenia, is characterized by severe bacterial infections. This condition results from a genetic mutation leading to the dysfunction of neutrophils, a type of white blood cell that forms an essential part of the immune system. Neutrophils are vital for combating bacterial infections, and their absence or malfunction in Kostmann syndrome leaves individuals highly susceptible to recurrent and severe bacterial illnesses. Unlike conditions like severe combined immunodeficiency (SCID), which affect multiple aspects of the immune system, including B-cell and T-cell function, Kostmann syndrome primarily involves neutrophil deficiency. It is important to distinguish this condition from secondary immunodeficiencies, which occur not from genetic causes but as a result of external factors like infection, metabolic disturbances, or medical treatments.

Answer 2

Kostmann syndrome is a severe congenital neutropenia. a) Severe bacterial infections is characteristic of this condition.

Step-by-step explanation:

Kostmann syndrome, also known as severe congenital neutropenia, is characterized by a a)severe reduction in the number of neutrophils, a type of white blood cell essential for fighting bacterial infections.

Neutrophils play a crucial role in the immune system's defense against bacteria, and their deficiency in Kostmann syndrome leaves individuals highly susceptible to severe bacterial infections.

Individuals with Kostmann syndrome often experience recurrent and life-threatening bacterial infections, including those affecting the skin, respiratory tract, and other organs. The condition typically presents early in life, and affected individuals may face challenges in maintaining overall health due to the increased susceptibility to bacterial pathogens.

Options b), c), and d) are not characteristic of Kostmann syndrome. Bone marrow disorders (Option b) may cause neutropenia, but Kostmann syndrome specifically refers to a congenital form. Severe viral infections (Option c) and autoimmune disorders (Option d) are not the primary features of Kostmann syndrome, as the main concern lies in the susceptibility to bacterial infections associated with neutropenia.