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{{WBRQuestion
{{WBRQuestion
|QuestionAuthor={{Rim}}
|QuestionAuthor={{Rim}}, {{AJL}} {{Alison}}
|ExamType=USMLE Step 1
|ExamType=USMLE Step 1
|MainCategory=Immunology
|MainCategory=Immunology
Line 20: Line 20:
|MainCategory=Immunology
|MainCategory=Immunology
|SubCategory=Hematology
|SubCategory=Hematology
|Prompt=A 10 year old boy is brought his mother to the physician's office because his mother says he looks different from his siblings.  On physical examination, the physician notes abscesses, retained primary teeth, and eczema.  Which of the following lab findings are most consistent with the patient's symptoms?
|Prompt=A 10-year-old male is brought by his mother to the physician's office due to her concern that he has a coarse face and an abnormal appearance.  Upon physical examination, you note abscesses, retained primary teeth, and eczema.  Which of the following lab findings are most consistent with the patient's presentation?
|Explanation=[[Hyper-IgE syndrome]] (HIES) or [[Job's syndrome]] is a primary [[immunodeficiency]] caused by a selective deficiency of [[interferon-gamma]] synthesis.  
|Explanation=[[Hyper-IgE syndrome]] (HIES) ([[Job's syndrome]]), a primary [[immunodeficiency]], is caused by a selective deficiency of [[interferon-gamma]] synthesis.  


Signs and symptoms include: elevated [[IgE]] levels, [[atopic dermatitis]] ([[eczema]]), extracellular infections manifested by cold staphylococcal [[abscesses]], with abnormal facies, fractures following minimal trauma and [[scoliosis]], and retention of [[primary teeth]].
Frequent manifestations and findings include elevated [[IgE]] levels, [[atopic dermatitis]] ([[eczema]]), extracellular infections manifested by cold staphylococcal [[abscesses]], with abnormal facies, fractures following minimal trauma, [[scoliosis]], and retention of [[primary teeth]].


Educational Objective:
|EducationalObjectives= [[Hyper-IgE syndrome]], a primary immunodeficiency, is caused by a selective deficiency of interferon-gamma production that resulting in elevated IgE levels, coarse facial features, retained primary teeth, and cold staphylococcal abscesses.
Hyper-IgE syndrome is a primay immunodeficiency caused by a selective deficiency of interferon-gamma production that causes elevated IgE, coarse facial features, retained primary teeth, and cold staphylococcal abscesses.
|References=Minegishi Y. Hyper-IgE syndome. Curr Opin Immunol. 2009;21(5):487-492


Reference:
Minegishi Y. Hyper-IgE syndome. Curr Opin Immunol. 2009;21(5):487-492
|AnswerA=Low IgE levels
|AnswerA=Low IgE levels
|AnswerAExp=Job's syndrome is characterized by hyper IgE levels, not low IgE levels.
|AnswerAExp=[[Job's syndrome]] is characterized by abnormally high IgE levels.
|AnswerB=Elevated IgE levels
|AnswerB=Elevated IgE levels
|AnswerBExp=Elevated IgE level is seen in hyper-IgE syndrome.
|AnswerBExp=Elevated IgE levels are frequently observed in [[hyper-IgE syndrome]].
|AnswerC=Elevated IgG levels
|AnswerC=Elevated IgG levels
|AnswerCExp=Elevated IgG levels are seen following re-exposure to an antigen. Monocloncal IgG gammopathy may mean multiple myeloma.
|AnswerCExp=Elevated IgG levels are frequently observed following re-exposure to an antigen. Monocloncal IgG gammopathy is associated with [[multiple myeloma]].
|AnswerD=Elevated IgA levels
|AnswerD=Elevated IgA levels
|AnswerDExp=Monoclonal elevation of IgA levels may be seen in multiple myeloma.
|AnswerDExp=Monoclonal elevation of IgA levels are frequently observed in patients with [[multiple myeloma]].
|AnswerE=Gradual lowering of all immunoglobulin levels
|AnswerE=Gradual lowering of all immunoglobulin levels
|AnswerEExp=Gradual lowering of levels of all immunoglobulin levels is not characteristic of Hyper-IgE syndrome.  It is characteristic of Wiskott-Aldrich syndrome.
|AnswerEExp=The gradual lowering of immunoglobulin levels, not characteristic of [[hyper-IgE syndrome]], is characteristic of [[Wiskott-Aldrich syndrome]].
|RightAnswer=B
|RightAnswer=B
|WBRKeyword=job's syndrome, job's, syndrome, IgE, hyper, coarse, facies, eczema, retained, primary, teeth, primary teeth, Buckley, hyperimmunoglobulin, HIES, abscess, staphylococcal,
|WBRKeyword=job's syndrome, IgE, coarse facies, eczema, teeth, primary teeth, abscess, staphylococcal, genetics, abnormality, deficiency, dental,
|Approved=No
|Approved=Yes
}}
}}

Revision as of 14:49, 28 July 2014
Author [[PageAuthor::Rim Halaby, M.D. [1], Alison Leibowitz [2] (Reviewed by Alison Leibowitz)]]
Exam Type ExamType::USMLE Step 1
Main Category MainCategory::Immunology
Sub Category SubCategory::Hematology
Prompt [[Prompt::A 10-year-old male is brought by his mother to the physician's office due to her concern that he has a coarse face and an abnormal appearance. Upon physical examination, you note abscesses, retained primary teeth, and eczema. Which of the following lab findings are most consistent with the patient's presentation?]]
Answer A AnswerA::Low IgE levels
Answer A Explanation [[AnswerAExp::Job's syndrome is characterized by abnormally high IgE levels.]]
Answer B AnswerB::Elevated IgE levels
Answer B Explanation [[AnswerBExp::Elevated IgE levels are frequently observed in hyper-IgE syndrome.]]
Answer C AnswerC::Elevated IgG levels
Answer C Explanation [[AnswerCExp::Elevated IgG levels are frequently observed following re-exposure to an antigen. Monocloncal IgG gammopathy is associated with multiple myeloma.]]
Answer D AnswerD::Elevated IgA levels
Answer D Explanation [[AnswerDExp::Monoclonal elevation of IgA levels are frequently observed in patients with multiple myeloma.]]
Answer E AnswerE::Gradual lowering of all immunoglobulin levels
Answer E Explanation [[AnswerEExp::The gradual lowering of immunoglobulin levels, not characteristic of hyper-IgE syndrome, is characteristic of Wiskott-Aldrich syndrome.]]
Right Answer RightAnswer::B
Explanation [[Explanation::Hyper-IgE syndrome (HIES) (Job's syndrome), a primary immunodeficiency, is caused by a selective deficiency of interferon-gamma synthesis.

Frequent manifestations and findings include elevated IgE levels, atopic dermatitis (eczema), extracellular infections manifested by cold staphylococcal abscesses, with abnormal facies, fractures following minimal trauma, scoliosis, and retention of primary teeth.
Educational Objective: Hyper-IgE syndrome, a primary immunodeficiency, is caused by a selective deficiency of interferon-gamma production that resulting in elevated IgE levels, coarse facial features, retained primary teeth, and cold staphylococcal abscesses.
References: Minegishi Y. Hyper-IgE syndome. Curr Opin Immunol. 2009;21(5):487-492]]

Approved Approved::Yes
Keyword WBRKeyword::job's syndrome, WBRKeyword::IgE, WBRKeyword::coarse facies, WBRKeyword::eczema, WBRKeyword::teeth, WBRKeyword::primary teeth, WBRKeyword::abscess, WBRKeyword::staphylococcal, WBRKeyword::genetics, WBRKeyword::abnormality, WBRKeyword::deficiency, WBRKeyword::dental
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