Hyperimmunoglobulin E syndrome
Hyperimmunoglobulin E syndrome
Step 1 - FPP
Step 1 - FPP
Atrophy, aplasia, and hypoplasia
Metaplasia and dysplasia
Hyperplasia and hypertrophy
Oncogenes and tumor suppressor genes
Free radicals and cellular injury
Necrosis and apoptosis
Ischemia
Hypoxia
Inflammation
Wound healing
Introduction to the immune system
Cytokines
Innate immune system
Complement system
T-cell development
B-cell development
MHC class I and MHC class II molecules
T-cell activation
B-cell activation, differentiation, and contraction
Cell-mediated immunity of CD4 cells
Cell-mediated immunity of natural killer and CD8 cells
Antibody classes
Somatic hypermutation and affinity maturation
VDJ rearrangement
Contracting the immune response and peripheral tolerance
B- and T-cell memory
Anergy, exhaustion, and clonal deletion
Vaccinations
Type I hypersensitivity
Type II hypersensitivity
Type III hypersensitivity
Type IV hypersensitivity
Immunodeficiencies: T-cell and B-cell disorders: Pathology review
Immunodeficiencies: Combined T-cell and B-cell disorders: Pathology review
Immunodeficiencies: Phagocyte and complement dysfunction: Pathology review
Thymus histology
Spleen histology
Lymph node histology
Amyloidosis
Transplant rejection
Graft-versus-host disease
Cytomegalovirus infection after transplant (NORD)
Post-transplant lymphoproliferative disorders (NORD)
X-linked agammaglobulinemia
Selective immunoglobulin A deficiency
Common variable immunodeficiency
IgG subclass deficiency
Hyperimmunoglobulin E syndrome
Isolated primary immunoglobulin M deficiency
Thymic aplasia
DiGeorge syndrome
Severe combined immunodeficiency
Adenosine deaminase deficiency
Ataxia-telangiectasia
Hyper IgM syndrome
Wiskott-Aldrich syndrome
Leukocyte adhesion deficiency
Chediak-Higashi syndrome
Chronic granulomatous disease
Complement deficiency
Hereditary angioedema
Asplenia
Food allergy
Anaphylaxis
Asthma
Autoimmune hemolytic anemia
Hemolytic disease of the newborn
Rheumatic heart disease
Myasthenia gravis
Graves disease
Pemphigus vulgaris
Serum sickness
Systemic lupus erythematosus
Poststreptococcal glomerulonephritis
Contact dermatitis
Autoimmune polyglandular syndrome type 1 (NORD)
Acute leukemia
Chronic leukemia
HIV (AIDS)
DNA damage and repair
Factor V Leiden
Von Willebrand disease
Flashcards
Hyperimmunoglobulin E syndrome
0 of 9 complete
Questions
USMLE® Step 1 style questions USMLE
0 of 1 complete
A 20-year-old man presents to his primary care physician due to cough and shortness of breath. The patient’s cough is productive of yellow, blood-tinged sputum. The patient has had recurrent pneumonia over the past ten years treated with oral antibiotics. Past medical history is significant for eczema since the newborn period, as well as recurrent pustular and eczematous rashes on the face and scalp throughout childhood. He underwent extraction of his primary teeth at the age of 14 years due to failure of eruption of secondary teeth. Family history is significant for similar symptoms in the patient’s mother. Temperature is 38.7 C (101.7 F), pulse is 90/min, respirations are 25/min and blood pressure is 110/70 mmHg. Physical examination demonstrates a broad nose, prominent lower lip, deep-set eyes with a prominent forehead, and a rough complexion with exaggerated pore size. Auscultation of lungs demonstrates bilateral rales in the lower lung fields and decreased breath sounds at the right lung base. Which of the following laboratory abnormalities is most likely to be observed in this patient?
Key Takeaways
Hyperimmunoglobulin E syndrome (HIES) is a genetic disorder caused by STAT3 gene mutation. It is characterized by Th17 cell deficiency, impaired neutrophil recruitment to sites of infection, and increased serum IgE concentrations. People with HIES physically present with coarse facial features, recurrent staphylococcal abscesses, retained primary teeth, and eczema.