WBR0470
| 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. |
| 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 |
| Linked Question | Linked:: |
| Order in Linked Questions | LinkedOrder:: |