Reading...
Play button
Play button
Use LEFT and RIGHT arrow keys to navigate between flashcards;
Use UP and DOWN arrow keys to flip the card;
H to show hint;
A reads text to speech;
20 Cards in this Set
- Front
- Back
|
What immune cell deficiency presents with recurrent infections with encapsulated bacteria such as Staphylococcus and Haemophilus influenzae?
|
B cell deficiency
|
|
|
What immune cell deficiency presents with recurrent infections with encapsulated bacteria such as Staphylococcus and Haemophilus influenzae?
|
B cell deficiency
|
|
|
What immune cell deficiency presents with recurrent fungal, viral or protozoal infections?
|
T cell deficiency
|
|
|
X linked hypogammaglobinemia(Bruton's agammaglobinemia) has low levels of all immunoglobulins due to what underlying deficiency?
|
Deficiency of B cell tyrosine kinase receptors leading to failure of differentiation of pre B cells to mature B cells.
|
|
|
X linked hypogammaglobinemia(Bruton's agammaglobinemia) has low levels of all immunoglobulins due to what underlying deficiency?
|
Deficiency of B cell tyrosine kinase receptors leading to failure of differentiation of pre B cells to mature B cells.
|
|
|
How does Bruton's manifest clinically?
|
Recurrent bacterial infections
|
|
|
Who typically gets Bruton's agammaglobinemia and why?
|
Young boys due to the X linked recessive inheritance.
|
|
|
How is Bruton's agammaglobinemia treated?
|
Treat with pooled immunoglobulin Ig.
|
|
|
At what age do most congenital B cell immunodeficiencies manifestt?
|
About 6 months, as levels of maternal IgG acquired transplacentally during the fetal period begin to fall.
|
|
|
What is the most common selective Ig deficiency, and how do patients present?
|
Selective IgA deficiency causes recurrent sinus and lung infections(recall IgA is typically present in mucus).
|
|
|
What can occur when patients with selective IgA deficiency receive a blood transfusion?
|
Anaphylactic reaction if the patients have anti-IgA antibodies that react against IgA in the donor serum.
|
|
|
What embryological process is defective in DiGeorge syndrome?
|
Development of 3rd and 4th pharyngeal arches, and subsequent aplasia of thymus(3rd arch) and parathyroids(third arch: inferior parathyroids, 4th arch: superior parathyroid.
|
|
|
What immune deficiency is part of DiGeorge syndrome and how is it treated?
|
Deficit of T cells due to thymic aplasia results in fungal, viral and protozoal infections(Pneumocystis pneumonia(PCP) and Candida albicans). Treat with fetal thymic transplant.
|
|
|
What electrolyte disturbance is seen in DiGeorge syndrome?
|
Hypocalcemia(and tetany) due to failure of parathyroid development.
|
|
|
What are the clinical and laboratory manifestations of hyper IgM syndrome?
|
Clinical: recurrent pyogenic bacterial infections early in life.
|
|
|
What are laboratory manifestations of hyper IgM syndrome?
|
See high IgM, but low IgG, IgA and IgE.
|
|
|
What is the underlying genetic defect in hyper IgM syndrome?
|
A mutation in CD40 ligand gene, leads to a defective CD40L on T cell surfaces. Without the proper CD40L, CD40 signaling, B cells cannot switch isotypes from IgMto other classes.
|
|
|
To what infections are patients with interleukin-12(IL-12) receptor deficiency predisposed?
|
Disseminated mycobacterial infections, because IL-12 is involved in development of the cell mediated Th1 response against mycobacteria.
|
|
|
What types of immune cells are defective in severe combined immunodeficiency disease(SCID) and how is it inherited?
|
B and T cells. Most cases(75%) are X linked.
|
|
|
How does SCID clinically manifest and how is it treated?
|
Patients are predisposed to recurrent bacterial, viral, fungal and protozoal infections. Treat with bone marrow transplant.
|
