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==Differentiating Wiskott-Aldrich Syndrome From Other Disorders of Humoral Imuunodeficiency==
==Differentiating Wiskott-Aldrich Syndrome From Other Disorders of Humoral Imuunodeficiency==
Wiskott-Aldrich Syndrome should be differentiated from other disorders leading to [[hypogammaglobulinemia]] and defects of [[humoral immunity]]. The following conditions may be considered as differentials:<ref name="pmid11091267">{{cite journal |vauthors=Fischer A |title=Severe combined immunodeficiencies (SCID) |journal=Clin. Exp. Immunol. |volume=122 |issue=2 |pages=143–9 |date=November 2000 |pmid=11091267 |pmc=1905779 |doi= |url=}}</ref>
Wiskott-Aldrich Syndrome should be differentiated from other disorders leading to [[hypogammaglobulinemia]] and defects of [[humoral immunity]]. The following conditions may be considered as differentials:
{|
{|
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Disorder  
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Disorder  
Line 8: Line 8:
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Laboratory Findings
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Laboratory Findings
|-
|-
! align="center" style="background:#DCDCDC;" + |[[Wiskott-Aldrich syndrome|'''Wiskott-Aldrich Syndrome''']]
! align="center" style="background:#DCDCDC;" + |[[Wiskott-Aldrich syndrome|Wiskott-Aldrich Syndrome]]
| align="left" style="background:#F5F5F5;" + |
| align="left" style="background:#F5F5F5;" + |
* [[Mutation]] in [[WAS]] [[gene]]
* [[Mutation]] in [[WAS]] [[gene]]
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* Fewer and smaller [[platelets]]
* Fewer and smaller [[platelets]]
|-
|-
! align="center" style="background:#DCDCDC;" + |[[X-linked agammaglobulinemia|'''X-Linked (Bruton) Agammaglobulinemia''']]
! align="center" style="background:#DCDCDC;" + |[[X-linked agammaglobulinemia|X-Linked (Bruton) Agammaglobulinemia]]
| align="left" style="background:#F5F5F5;" + |
| align="left" style="background:#F5F5F5;" + |
* Defect in [[tyrosine kinase]] [[gene]] ([[Bruton's tyrosine kinase|BTK]])
* Defect in [[tyrosine kinase]] [[gene]] ([[Bruton's tyrosine kinase|BTK]])
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* Decreased [[immunoglobulins]] of all classes  
* Decreased [[immunoglobulins]] of all classes  
|-
|-
! align="center" style="background:#DCDCDC;" + |[[IgA deficiency|'''Selective IgA Deficiency''']]
! align="center" style="background:#DCDCDC;" + |[[IgA deficiency|Selective IgA Deficiency]]
| align="left" style="background:#F5F5F5;" + |
| align="left" style="background:#F5F5F5;" + |
* [[Stem cell]] defect (Transferrable with [[Bone marrow transplant|marrow transplant]])
* [[Stem cell]] defect (Transferrable with [[Bone marrow transplant|marrow transplant]])
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* Normal [[IgG]] and [[IgM]] levels
* Normal [[IgG]] and [[IgM]] levels
|-
|-
! align="center" style="background:#DCDCDC;" + |[[Common variable immunodeficiency|'''Common Variable Immunodeficiency''']]
! align="center" style="background:#DCDCDC;" + |[[Common variable immunodeficiency|Common Variable Immunodeficiency]]
| align="left" style="background:#F5F5F5;" + |
| align="left" style="background:#F5F5F5;" + |
* Defective [[B cell]] differentiation
* Defective [[B cell]] differentiation
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* Decreased [[immunoglobulins]]
* Decreased [[immunoglobulins]]
|-
|-
! align="center" style="background:#DCDCDC;" + |[[Job's syndrome|'''Autosomal dominant hype IgE syndrome (Job's Syndrome)''']]
! align="center" style="background:#DCDCDC;" + |[[Job's syndrome|Autosomal dominant hype IgE syndrome (Job's Syndrome)]]
| align="left" style="background:#F5F5F5;" + |
| align="left" style="background:#F5F5F5;" + |
* Defieciency of [[T helper 17 cell|Th17 cells]] due to [[STAT3]] [[mutation]]
* Defieciency of [[T helper 17 cell|Th17 cells]] due to [[STAT3]] [[mutation]]
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* Decreased levels of [[interferon gamma]] (IFN-gamma)
* Decreased levels of [[interferon gamma]] (IFN-gamma)
|-
|-
! align="center" style="background:#DCDCDC;" + |[[Severe combined immunodeficiency|'''Severe combined immunodeficiency (SCID)''']]
! align="center" style="background:#DCDCDC;" + |[[Severe combined immunodeficiency|Severe combined immunodeficiency (SCID)]]
| align="left" style="background:#F5F5F5;" + |
| align="left" style="background:#F5F5F5;" + |
* Defective [[Interleukin-2 receptor|interleukin-2 receptor gamma chain]]  
* Defective [[Interleukin-2 receptor|interleukin-2 receptor gamma chain]]  
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* Absent [[T cells]] on [[flow cytometry]]
* Absent [[T cells]] on [[flow cytometry]]
|-
|-
! align="center" style="background:#DCDCDC;" + |[[Ataxia telangiectasia|'''Ataxia Telangiectasia''']]
! align="center" style="background:#DCDCDC;" + |[[Ataxia telangiectasia|Ataxia Telangiectasia]]
| align="left" style="background:#F5F5F5;" + |
| align="left" style="background:#F5F5F5;" + |
* Defect in [[ATM|ATM gene]]
* Defect in [[ATM|ATM gene]]
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* [[Cerebellar]] atrophy
* [[Cerebellar]] atrophy
|-
|-
! align="center" style="background:#DCDCDC;" + |[[Hyper IgM Syndrome Type 1|'''Hyper IgM Syndrome''']]
! align="center" style="background:#DCDCDC;" + |[[Hyper IgM Syndrome Type 1|Hyper IgM Syndrome]]
| align="left" style="background:#F5F5F5;" + |
| align="left" style="background:#F5F5F5;" + |
* Defective [[CD40L]] ([[CD40L|CD40 ligand]]) on [[T helper cell|Th cells]] leading to [[class switching]] defect
* Defective [[CD40L]] ([[CD40L|CD40 ligand]]) on [[T helper cell|Th cells]] leading to [[class switching]] defect

Revision as of 16:54, 14 December 2018

Differentiating Wiskott-Aldrich Syndrome From Other Disorders of Humoral Imuunodeficiency

Wiskott-Aldrich Syndrome should be differentiated from other disorders leading to hypogammaglobulinemia and defects of humoral immunity. The following conditions may be considered as differentials:

Disorder Mechanism Characteristic Features Clinical Presentation Laboratory Findings
Wiskott-Aldrich Syndrome
X-Linked (Bruton) Agammaglobulinemia
Selective IgA Deficiency
  • Serum IgA < 7 mg/dl
  • Normal IgG and IgM levels
Common Variable Immunodeficiency
  • Defective B cell differentiation
  • May be acquired in 20-30 years of age
Autosomal dominant hype IgE syndrome (Job's Syndrome)
  • Distinctive coarse facies
  • Cold (non-inflammatory) Staphylococcal abscesses
  • Retained primary teeth
  • Eczema
Severe combined immunodeficiency (SCID)
Ataxia Telangiectasia
Hyper IgM Syndrome
  • Malignancy: can cause the reduction in the immunoglobulin production.
  • Viral infections: such as Epstein-Barr virus, HIV, cytomegalovirus are other causes of hypogammaglobulinemia..
  • Side effect of certain medications: Some drugs include systemic glucocorticoids, phenytoin, and carbamazepine, have been associated with IgG deficiency.
  • Other causes of primary humoral immunodeficiencies.
  • Smoking: may cause IgG2 subclass deficiency.
  • Protein-losing conditions: enteropathies, nephrotic syndrome, burns, and other traumas may cause abnormal loss of immunoglobulins.
An ECG in a person with a potassium level of 1.1 showing the classical ECG changes of ST segment depression, inverted T waves, large U waves, and a slightly prolonged PR interval. By James Heilman, MD - Own work, CC BY-SA 3.0


An ECG in a person with a potassium level of 1.1 showing the classical ECG changes of ST segment depression, inverted T waves, large U waves, and a slightly prolonged PR interval. By James Heilman, MD - Own work, CC BY-SA 3.0


{{#ev:youtube|7TWu0_Gklzo}}

Table

Complications Polymyositis Dermatomyositis
Malignancy