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65 Cards in this Set
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Decreased production of B cells.
X-linked recessive defect in a tyrosine kinase gene associated with low levels of all classes of immunoglobulins. Associated with recurrent Bacterial infections after 6 months of age, when levels of maternal IgG antibody decline. Occurs in Boys (X-linked) |
Bruton's agammaglobulinemia
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X-linked recessive defect in a tyrosine kinase gene associated with low levels of all classes of immunoglobulins
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Bruton's agammaglobulinemia
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Decreased production of B cells.
X-linked recessive defect in a tyrosine kinase gene associated with low levels of all classes of immunoglobulins. Associated with recurrent Bacterial infections after 6 months of age, when levels of maternal IgG antibody decline. Occurs in Boys (X-linked) |
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Associated with recurrent Bacterial infections after 6 months of age, when levels of maternal IgG antibody decline.
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Bruton's agammaglobulinemia
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Decreased production of B cells.
X-linked recessive defect in a tyrosine kinase gene associated with low levels of all classes of immunoglobulins. Associated with recurrent Bacterial infections after 6 months of age, when levels of maternal IgG antibody decline. Occurs in Boys (X-linked) |
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Bruton's agammaglobulinemia is also known as
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X-linked agammaglobulinemia
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Decreased production of B cells.
X-linked recessive defect in a tyrosine kinase gene associated with low levels of all classes of immunoglobulins. Associated with recurrent Bacterial infections after 6 months of age, when levels of maternal IgG antibody decline. Occurs in Boys (X-linked) |
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Bruton's agammaglobulinemia affects which demographics?
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Boys (x-linked). Associated with recurrent Bacterial infections after 6 months of age, when levels of maternal IgG antibody decline.
(B for Bruton, B cells, Bacterial infections after 6 months, and Boys) |
Decreased production of B cells.
X-linked recessive defect in a tyrosine kinase gene associated with low levels of all classes of immunoglobulins. Associated with recurrent Bacterial infections after 6 months of age, when levels of maternal IgG antibody decline. Occurs in Boys (X-linked) |
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Bruton's agammaglobulinemia has a defect in which gene?
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a tyrosine kinase gene associated with low levels of all classes of immunoglobulins
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Decreased production of B cells.
X-linked recessive defect in a tyrosine kinase gene associated with low levels of all classes of immunoglobulins. Associated with recurrent Bacterial infections after 6 months of age, when levels of maternal IgG antibody decline. Occurs in Boys (X-linked) |
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what goes wrong in Bruton's agammaglobulinemia?
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Decreased production of B cells. (B for Bruton, B cells, Bacterial infections after 6 months, and Boys)
X-linked recessive defect in a tyrosine kinase gene associated with low levels of all classes of immunoglobulins |
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Thymus and parathyroids fail to develop owing to failure of development of the 3rd and 4th pharyngeal pouches. Presents with Tentany owing to hypocalcemia. Recurrent viral and fungal infections due to T-cell deficiency. Congenital defects of heart and great vessels. 22q11 delection.
Decreased production of T cells. |
Thymic aplasia / DiGeorge syndrome
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Thymus and parathyroids fail to develop owing to failure of development of the 3rd and 4th pharyngeal pouches. Presents with Tentany owing to hypocalcemia. Recurrent viral and fungal infections due to T-cell deficiency. Congenital defects of heart and great vessels. 22q11 delection.
Decreased production of T cells. |
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What fails to develop in DiGeorge?
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3rd and 4th pharyngeal pouches fail to develop -> thymus and parathyroids failing to develop.
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Thymus and parathyroids fail to develop owing to failure of development of the 3rd and 4th pharyngeal pouches. Presents with Tentany owing to hypocalcemia. Recurrent viral and fungal infections due to T-cell deficiency. Congenital defects of heart and great vessels. 22q11 delection.
Decreased production of T cells. |
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What disease -> hypocalcemia
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DiGeorge / thymic aplasia
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Thymus and parathyroids fail to develop owing to failure of development of the 3rd and 4th pharyngeal pouches. Presents with Tentany owing to hypocalcemia. Recurrent viral and funal infections due to T-cell deficiency. Congenital defects of heart and great vessels. 22q11 delection.
Decreased production of T cells. |
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What disease -> Congenital defects of heart and great vessels.
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DiGeorge / thymic aplasia
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Thymus and parathyroids fail to develop owing to failure of development of the 3rd and 4th pharyngeal pouches. Presents with Tentany owing to hypocalcemia. Recurrent viral and funal infections due to T-cell deficiency. Congenital defects of heart and great vessels. 22q11 delection.
Decreased production of T cells. |
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22q11 delection.
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DiGeorge / thymic aplasia
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Thymus and parathyroids fail to develop owing to failure of development of the 3rd and 4th pharyngeal pouches. Presents with Tentany owing to hypocalcemia. Recurrent viral and funal infections due to T-cell deficiency. Congenital defects of heart and great vessels. 22q11 delection.
Decreased production of T cells. |
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types of infections seens in DiGeorge / thymic aplasia?
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Recurrent viral and fungal infections due to T-cell deficiency.
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Thymus and parathyroids fail to develop owing to failure of development of the 3rd and 4th pharyngeal pouches. Presents with Tentany owing to hypocalcemia. Recurrent viral and funal infections due to T-cell deficiency. Congenital defects of heart and great vessels. 22q11 delection.
Decreased production of T cells. |
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Defect in early stem-cell differentiation. Presents with recurrent viral, bacterial, fungal and protozoal infections. May have multiple causes (e.g. failure to synthesize MHC II antigents, defective IL-2 receptors, or adenosine deaminase deficiency)
Decreased production of B and T cells |
Severe combined Immunodeficiency (SCID)
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Defect in early stem-cell differentiation. Presents with recurrent viral, bacterial, fungal and protozoal infections. May have multiple causes (e.g. failure to synthesize MHC II antigens, defective IL-2 receptors, or adenosine deaminase deficiency)
Decreased production of B and T cells |
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3 causes of SCID
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failure to synthesize MHC II antigents,
defective IL-2 receptors, or adenosine deaminase deficiency |
Defect in early stem-cell differentiation. Presents with recurrent viral, bacterial, fungal and protozoal infections. May have multiple causes (e.g. failure to synthesize MHC II antigents, defective IL-2 receptors, or adenosine deaminase deficiency)
Decreased production of B and T cells |
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types of infections seen in SCID
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Presents with recurrent viral, bacterial, fungal and protozoal infections.
(Basically EVERYTHING) |
Defect in early stem-cell differentiation. Presents with recurrent viral, bacterial, fungal and protozoal infections. May have multiple causes (e.g. failure to synthesize MHC II antigents, defective IL-2 receptors, or adenosine deaminase deficiency)
Decreased production of B and T cells |
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Adenosine deaminase deficiency can lead to what disease?
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SCID
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Defect in early stem-cell differentiation. Presents with recurrent viral, bacterial, fungal and protozoal infections. May have multiple causes (e.g. failure to synthesize MHC II antigents, defective IL-2 receptors, or adenosine deaminase deficiency)
Decreased production of B and T cells |
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Defective IL-2 receptors can lead to what disease?
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SCID
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Defect in early stem-cell differentiation. Presents with recurrent viral, bacterial, fungal and protozoal infections. May have multiple causes (e.g. failure to synthesize MHC II antigents, defective IL-2 receptors, or adenosine deaminase deficiency)
Decreased production of B and T cells |
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Defective IL-12 recepts can lead to what disease?
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IL-12 receptor deficiency
(defective IL-2 receptors lead to SCID) |
decreased activation of T-cells
presents with disseminated mycobacterial infections |
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IL-12 receptor deficiency leads to what types of infections?
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disseminated mycobacterial infections
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decreased activation of T-cells
presents with disseminated mycobacterial infections |
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IL-12 receptor deficiency leads to decreased activation of what cells?
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T cells
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decreased activation of T-cells
presents with disseminated mycobacterial infections |
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decreased activation of T-cells
presents with disseminated mycobacterial infections |
IL-12 receptor deficiency
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decreased activation of T-cells
presents with disseminated mycobacterial infections |
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Defect in CD40 ligand on CD4 T helper cells leads to inability to class switch. Presents early in life with severe pyogenic infections. High levels of IgM; very low levels of IgG, IgA, and IgE.
decreased activation of B-cells |
hyper IgM syndrome
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Defect in CD40 ligand on CD4 T helper cells leads to inability to class switch. Presents early in life with severe pyogenic infections. High levels of IgM; very low levels of IgG, IgA, and IgE.
decreased activation of B-cells |
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Defect in CD40 ligand on CD4 T helper cells leads to inability to class switch.
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hyper IgM syndrome
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Defect in CD40 ligand on CD4 T helper cells leads to inability to class switch. Presents early in life with severe pyogenic infections. High levels of IgM; very low levels of IgG, IgA, and IgE.
decreased activation of B-cells |
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Presents early in life with severe pyogenic infections.
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hyper IgM syndrome
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Defect in CD40 ligand on CD4 T helper cells leads to inability to class switch. Presents early in life with severe pyogenic infections. High levels of IgM; very low levels of IgG, IgA, and IgE.
decreased activation of B-cells |
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High levels of IgM; very low levels of IgG, IgA, and IgE.
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hyper IgM syndrome
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Defect in CD40 ligand on CD4 T helper cells leads to inability to class switch. Presents early in life with severe pyogenic infections. High levels of IgM; very low levels of IgG, IgA, and IgE.
decreased activation of B-cells |
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hyper IgM syndrome has a defect in which ligand?
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Defect in CD40 ligand on CD4 T helper cells leads to inability to class switch.
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Defect in CD40 ligand on CD4 T helper cells leads to inability to class switch. Presents early in life with severe pyogenic infections. High levels of IgM; very low levels of IgG, IgA, and IgE.
decreased activation of B-cells |
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How does IgM syndrome present?
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Presents early in life with severe pyogenic infections.
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Defect in CD40 ligand on CD4 T helper cells leads to inability to class switch. Presents early in life with severe pyogenic infections. High levels of IgM; very low levels of IgG, IgA, and IgE.
decreased activation of B-cells |
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X-linked defect in the ability to mount an IgM response to capsular polysaccharides of bacteria. Associated with elevated IgA levels, normal IgE levels, and low IgM levels.
Triad of symptoms includes recurrent pyogenic Infections, thrombocytopenic Purpura, Eczema (WIPE) decreased activation of B-cells |
Wiskott-Aldrich syndrome
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X-linked defect in the ability to mount an IgM response to capsular polysaccharides of bacteria. Associated with elevated IgA levels, normal IgE levels, and low IgM levels.
Triad of symptoms includes recurrent pyogenic Infections, thrombocytopenic Purpura, Eczema (WIPE) decreased activation of B-cells |
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Associated with elevated IgA levels, normal IgE levels, and low IgM levels.
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Wiskott-Aldrich syndrome
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X-linked defect in the ability to mount an IgM response to capsular polysaccharides of bacteria. Associated with elevated IgA levels, normal IgE levels, and low IgM levels.
Triad of symptoms includes recurrent pyogenic Infections, thrombocytopenic Purpura, Eczema (WIPE) decreased activation of B-cells |
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Triad of symptoms includes recurrent pyogenic Infections, thrombocytopenic Purpura, Eczema (WIPE)
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Wiskott-Aldrich syndrome
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X-linked defect in the ability to mount an IgM response to capsular polysaccharides of bacteria. Associated with elevated IgA levels, normal IgE levels, and low IgM levels.
Triad of symptoms includes recurrent pyogenic Infections, thrombocytopenic Purpura, Eczema (WIPE) decreased activation of B-cells |
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how does Wiskott-Aldrich syndrome present?
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Triad of symptoms includes recurrent pyogenic Infections, thrombocytopenic Purpura, Eczema (WIPE)
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X-linked defect in the ability to mount an IgM response to capsular polysaccharides of bacteria. Associated with elevated IgA levels, normal IgE levels, and low IgM levels.
Triad of symptoms includes recurrent pyogenic Infections, thrombocytopenic Purpura, Eczema (WIPE) decreased activation of B-cells |
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Antibody levels in Wiskott-Aldrich syndrome?
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elevated IgA levels, normal IgE levels, and low IgM levels.
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X-linked defect in the ability to mount an IgM response to capsular polysaccharides of bacteria. Associated with elevated IgA levels, normal IgE levels, and low IgM levels.
Triad of symptoms includes recurrent pyogenic Infections, thrombocytopenic Purpura, Eczema (WIPE) decreased activation of B-cells |
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Thrombocytopenic purpura might be a sign of which syndrome?
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Wiskott-Aldrich syndrome
Triad of symptoms includes recurrent pyogenic Infections, thrombocytopenic Purpura, Eczema (WIPE) |
X-linked defect in the ability to mount an IgM response to capsular polysaccharides of bacteria. Associated with elevated IgA levels, normal IgE levels, and low IgM levels.
Triad of symptoms includes recurrent pyogenic Infections, thrombocytopenic Purpura, Eczema (WIPE) decreased activation of B-cells |
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Failure of gamma-interferon production by helper T cells. Neutrophils fail to respond to chemotactic stimuli. Presents with recurrent "cold" (noninflamed) staphylococcal abscesses, eczema, coarse facies, retained primary teeth, and high levels of IgE.
decreased activation of macrophages |
Job's syndrome
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Failure of gamma-interferon production by helper T cells. Neutrophils fail to respond to chemotactic stimuli. Presents with recurrent "cold" (noninflamed) staphylococcal abscesses, eczema, coarse facies, retained primary teeth, and high levels of IgE.
decreased activation of macrophages |
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etiology of Job's syndrome
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Failure of gamma-interferon production by helper T cells. Neutrophils fail to respond to chemotactic stimuli.
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Failure of gamma-interferon production by helper T cells. Neutrophils fail to respond to chemotactic stimuli. Presents with recurrent "cold" (noninflamed) staphylococcal abscesses, eczema, coarse facies, retained primary teeth, and high levels of IgE.
decreased activation of macrophages |
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presentation of Job's syndrome
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Presents with recurrent "cold" (noninflamed) staphylococcal abscesses, eczema, coarse facies, retained primary teeth, and high levels of IgE.
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Failure of gamma-interferon production by helper T cells. Neutrophils fail to respond to chemotactic stimuli. Presents with recurrent "cold" (noninflamed) staphylococcal abscesses, eczema, coarse facies, retained primary teeth, and high levels of IgE.
decreased activation of macrophages |
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Eczema is seen in which 2 diseases?
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Wiskott-Aldrich syndrome
Job's syndrome |
Failure of gamma-interferon production by helper T cells. Neutrophils fail to respond to chemotactic stimuli. Presents with recurrent "cold" (noninflamed) staphylococcal abscesses, eczema, coarse facies, retained primary teeth, and high levels of IgE.
decreased activation of macrophages |
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Immunoglobulin levels in Jobs synd.
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high IgE
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Failure of gamma-interferon production by helper T cells. Neutrophils fail to respond to chemotactic stimuli. Presents with recurrent "cold" (noninflamed) staphylococcal abscesses, eczema, coarse facies, retained primary teeth, and high levels of IgE.
decreased activation of macrophages |
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decreased activation of macrophages is seen in
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Jobs syndrome
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Failure of gamma-interferon production by helper T cells. Neutrophils fail to respond to chemotactic stimuli. Presents with recurrent "cold" (noninflamed) staphylococcal abscesses, eczema, coarse facies, retained primary teeth, and high levels of IgE.
decreased activation of macrophages |
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Defect in LFA-1 adhesion proteins on phagocytes. Presents early with severe pyogenic and fungal infections and delayed separation of umbilicus.
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Leukocyte adhesion deficiency syndrome
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Defect in LFA-1 adhesion proteins on phagocytes. Presents early with severe pyogenic and fungal infections and delayed separation of umbilicus.
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etiology of Leukocyte adhesion deficiency syndrome
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Defect in LFA-1 adhesion proteins on phagocytes.
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Defect in LFA-1 adhesion proteins on phagocytes. Presents early with severe pyogenic and fungal infections and delayed separation of umbilicus.
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presentation of Leukocyte adhesion deficiency syndrome
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Presents early with severe pyogenic and fungal infections and delayed separation of umbilicus..
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Defect in LFA-1 adhesion proteins on phagocytes. Presents early with severe pyogenic and fungal infections and delayed separation of umbilicus.
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delayed separation of umbilicus can be a sign of which disease
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Leukocyte adhesion deficiency syndrome
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Defect in LFA-1 adhesion proteins on phagocytes. Presents early with severe pyogenic and fungal infections and delayed separation of umbilicus.
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Autosomal recessive.
Defect in microtubular function and lysosomal emptying of phagocytic cells. Presents with recurrent pyogenic infections by staphylococci and streptococci, partial albinism, and peripheral neuropathy. |
Chediak-Higashi disease
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Autosomal recessive.
Defect in microtubular function and lysosomal emptying of phagocytic cells. Presents with recurrent pyogenic infections by staphylococci and streptococci, partial albinism, and peripheral neuropathy. |
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Chediak-Higashi disease has what inheritance pattern?
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Autosomal recessive.
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Autosomal recessive.
Defect in microtubular function and lysosomal emptying of phagocytic cells. Presents with recurrent pyogenic infections by staphylococci and streptococci, partial albinism, and peripheral neuropathy. |
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etiology of Chediak-Higashi disease
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Defect in microtubular function and lysosomal emptying of phagocytic cells.
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Autosomal recessive.
Defect in microtubular function and lysosomal emptying of phagocytic cells. Presents with recurrent pyogenic infections by staphylococci and streptococci, partial albinism, and peripheral neuropathy. |
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Defect in microtubular function and lysosomal emptying of phagocytic cells.
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Chediak-Higashi disease
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Autosomal recessive.
Defect in microtubular function and lysosomal emptying of phagocytic cells. Presents with recurrent pyogenic infections by staphylococci and streptococci, partial albinism, and peripheral neuropathy. |
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presentation of Chediak-Higashi disease
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Presents with recurrent pyogenic infections by staphylococci and streptococci, partial albinism, and peripheral neuropathy.
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Autosomal recessive.
Defect in microtubular function and lysosomal emptying of phagocytic cells. Presents with recurrent pyogenic infections by staphylococci and streptococci, partial albinism, and peripheral neuropathy. |
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Presents with recurrent pyogenic infections by staphylococci and streptococci, partial albinism, and peripheral neuropathy.
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Chediak-Higashi disease
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Autosomal recessive.
Defect in microtubular function and lysosomal emptying of phagocytic cells. Presents with recurrent pyogenic infections by staphylococci and streptococci, partial albinism, and peripheral neuropathy. |
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partial albinism can be a sign of which disease?
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Chediak-Higashi disease
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Autosomal recessive.
Defect in microtubular function and lysosomal emptying of phagocytic cells. Presents with recurrent pyogenic infections by staphylococci and streptococci, partial albinism, and peripheral neuropathy. |
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Defect in phagocytosis of neutrophils owing to lack of NADPH oxidase activity or similar enzymes. Presents with marked susceptibility to opportunistic infection with bacteria, especially S. aureus, E. coli, and Aspergillus.
Diagnosis confirmed with negative nitroblue tetrazolium dye reduction test. |
Chronic granulomatous disease
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Defect in phagocytosis of neutrophils owing to lack of NADPH oxidase activity or similar enzymes. Presents with marked susceptibility to opportunistic infection with bacteria, especially S. aureus, E. coli, and Aspergillus.
Diagnosis confirmed with negative nitroblue tetrazolium dye reduction test. |
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etiology of Chronic granulomatous disease
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Defect in phagocytosis of neutrophils owing to lack of NADPH oxidase activity or similar enzymes.
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Defect in phagocytosis of neutrophils owing to lack of NADPH oxidase activity or similar enzymes. Presents with marked susceptibility to opportunistic infection with bacteria, especially S. aureus, E. coli, and Aspergillus.
Diagnosis confirmed with negative nitroblue tetrazolium dye reduction test. |
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Defect in phagocytosis of neutrophils owing to lack of NADPH oxidase activity or similar enzymes.
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Chronic granulomatous disease
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Defect in phagocytosis of neutrophils owing to lack of NADPH oxidase activity or similar enzymes. Presents with marked susceptibility to opportunistic infection with bacteria, especially S. aureus, E. coli, and Aspergillus.
Diagnosis confirmed with negative nitroblue tetrazolium dye reduction test. |
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presentation of Chronic granulomatous disease
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Presents with marked susceptibility to opportunistic infection with bacteria, especially S. aureus, E. coli, and Aspergillus.
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Defect in phagocytosis of neutrophils owing to lack of NADPH oxidase activity or similar enzymes. Presents with marked susceptibility to opportunistic infection with bacteria, especially S. aureus, E. coli, and Aspergillus.
Diagnosis confirmed with negative nitroblue tetrazolium dye reduction test. |
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negative nitroblue tetrazolium dye reduction test is used to diagnose which disease?
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Chronic granulomatous disease
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Defect in phagocytosis of neutrophils owing to lack of NADPH oxidase activity or similar enzymes. Presents with marked susceptibility to opportunistic infection with bacteria, especially S. aureus, E. coli, and Aspergillus.
Diagnosis confirmed with negative nitroblue tetrazolium dye reduction test. |
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T-cell dysfunction specifically against Candida albicans. Presents with skin and mucous membrane Candida infections.
Idiopathic dysfunction of T cells. |
chronic mucocutaneous candidiasis
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T-cell dysfunction specifically against Candida albicans. Presents with skin and mucous membrane Candida infections.
Idiopathic dysfunction of T cells. |
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what type of cells are dysfunctional in chronic mucocutaneous candidiasis?
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T cells
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T-cell dysfunction specifically against Candida albicans. Presents with skin and mucous membrane Candida infections.
Idiopathic dysfunction of T cells. |
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Deficiency in a specific class of immunoglobulins--possibly due to a defect in isotype switching. Selective IgA deficiency is the most common selective immunoglobulin deficiency.
Presents with sinus and lung infections; milk allergies and diarrhea are common. Idiopathic dysfunction of B cells |
Selective immunoglobulin deficiency
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Deficiency in a specific class of immunoglobulins--possibly due to a defect in isotype switching. Selective IgA deficiency is the most common selective immunoglobulin deficiency.
Presents with sinus and lung infections; milk allergies and diarrhea are common. Idiopathic dysfunction of B cells |
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most common selective immunoglobulin deficiency
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selective IgA deficiency
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Deficiency in a specific class of immunoglobulins--possibly due to a defect in isotype switching. Selective IgA deficiency is the most common selective immunoglobulin deficiency.
Presents with sinus and lung infections; milk allergies and diarrhea are common. Idiopathic dysfunction of B cells |
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how does Selective Immunoglobulin Deficiency present?
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Presents with sinus and lung infections; milk allergies and diarrhea are common.
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Deficiency in a specific class of immunoglobulins--possibly due to a defect in isotype switching. Selective IgA deficiency is the most common selective immunoglobulin deficiency.
Presents with sinus and lung infections; milk allergies and diarrhea are common. Idiopathic dysfunction of B cells |
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Presents with sinus and lung infections; milk allergies and diarrhea are common.
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Selective immunoglobulin deficiency
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Deficiency in a specific class of immunoglobulins--possibly due to a defect in isotype switching. Selective IgA deficiency is the most common selective immunoglobulin deficiency.
Presents with sinus and lung infections; milk allergies and diarrhea are common. Idiopathic dysfunction of B cells |
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Defect in DNA repair enzymes with associated IgA deficiency. Presents with cerebellar problems (ataxia) and spider angiomas (telangiectasia)
Idiopathic dysfunction of B cells |
Ataxia-telangiectasia
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Defect in DNA repair enzymes with associated IgA deficiency. Presents with cerebellar problems (ataxia) and spider angiomas (telangiectasia)
Idiopathic dysfunction of B cells |
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how does Ataxia-telangiectasia present?
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Presents with cerebellar problems (ataxia) and spider angiomas (telangiectasia)
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Defect in DNA repair enzymes with associated IgA deficiency. Presents with cerebellar problems (ataxia) and spider angiomas (telangiectasia)
Idiopathic dysfunction of B cells |
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what immunoglobulin deficiency is present in Ataxia-telangiectasia?
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IgA deficiency
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Defect in DNA repair enzymes with associated IgA deficiency. Presents with cerebellar problems (ataxia) and spider angiomas (telangiectasia)
Idiopathic dysfunction of B cells |