WBR0470
| Author | [[PageAuthor::Rim Halaby, M.D. [1]]] |
|---|---|
| Exam Type | ExamType::USMLE Step 1 |
| Main Category | MainCategory::Immunology |
| Sub Category | SubCategory::Hematology |
| Prompt | [[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?]] |
| Answer A | AnswerA::Elevated IgM levels |
| Answer A Explanation | AnswerAExp::Elevated IgM levels are seen in hyper-IgM syndrome. |
| Answer B | AnswerB::Elevated IgE levels |
| Answer B Explanation | AnswerBExp::Elevated IgE levels are seen in hyper-IgE syndrome. |
| Answer C | AnswerC::Elevated IgG levels |
| Answer C Explanation | AnswerCExp::Elevated IgG levels are seen following re-exposure to an antigen. Monocloncal IgG gammopathy may mean multiple myeloma. |
| Answer D | AnswerD::Elevated IgA levels |
| Answer D Explanation | AnswerDExp::Elevated IgA levels may also be seen in multiple myeloma. |
| Answer E | AnswerE::Elevated levels of all immunoglobulins |
| Answer E Explanation | AnswerEExp::Elevated levels of all immunoglobulins is not characteristic of any syndrome. |
| Right Answer | RightAnswer::B |
| Explanation | [[Explanation::Hyper-IgE syndrome (HIES) or Job's syndrome is a primary immunodeficiency 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. Educational Objective: 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. Reference:
Minegishi Y. Hyper-IgE syndome. Curr Opin Immunol. 2009;21(5):487-492 |
| Approved | Approved::No |
| Keyword | WBRKeyword::job's syndrome, WBRKeyword::job's, WBRKeyword::syndrome, WBRKeyword::IgE, WBRKeyword::hyper, WBRKeyword::coarse, WBRKeyword::facies, WBRKeyword::eczema, WBRKeyword::retained, WBRKeyword::primary, WBRKeyword::teeth, WBRKeyword::primary teeth, WBRKeyword::Buckley, WBRKeyword::hyperimmunoglobulin, WBRKeyword::HIES, WBRKeyword::abscess, WBRKeyword::staphylococcal |
| Linked Question | Linked:: |
| Order in Linked Questions | LinkedOrder:: |