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Printable Handouts
Navigable Slide Index
- Introduction
- Learning objectives
- Primary immune deficiencies (PIDs)
- The immune system at a glance
- Immune responses
- Genetic architecture of PID
- When to suspect immunodeficiency
- Function of key players in the immune system
- Clinical manifestations
- Immunology investigations
- Specific examples
- PID prevalence in the UK
- Disorders of innate immunity
- Pattern recognition receptors
- TLR3 deficiency – HSV encephalitis
- MyD88 deficiency – invasive bacterial disease
- Neutrophil defects
- Chronic granulomatous disease - generalities
- Chronic granulomatous disease - pathogenesis, presentation and treatment
- Diagnosis by flow-cytometry of neutrophils
- Antibody deficiencies
- Humoral immunodeficiencies
- X-linked agammaglobulinaemia
- Bruton’s X-linked antibody deficiency
- Common Variable Immunodeficiency Disorder
- Investigation to CONFIRM antibody deficiency
- Investigation for CAUSE of antibody deficiency
- Antibody deficiency treatment
- Primary antibody deficiency: key points
- T cell defects
- Severe Combined Immune Deficiency
- Laboratory evaluation at 5 months of age
- Lymphocytes counts in children
- Case study: pathology
- Case study: diagnosis in this child
- SCID
- PID associated lymphocyte development blocks: T- B- NK-
- PID associated lymphocyte development blocks: T- B+ NK-
- PID associated lymphocyte development blocks: T- B- NK+
- Lymphocyte phenotypes of different SCIDs
- Workup and Management of SCID
- Treatment and prognosis
- 2q11 syndrome
- Summary
Topics Covered
- Introduction to Primary Immune Deficiencies (PIDs)
- The immune system function and PIDs
- Disorders of innate immunity
- Antibody deficiencies
- T cell defects
- PIDs early diagnosis and treatment
Links
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External Links
Talk Citation
Patel, S.Y. (2020, November 30). Primary immunodeficiency disorders [Video file]. In The Biomedical & Life Sciences Collection, Henry Stewart Talks. Retrieved October 6, 2024, from https://doi.org/10.69645/NJFO8259.Export Citation (RIS)
Publication History
Financial Disclosures
- Dr. Smita Y. Patel has not informed HSTalks of any commercial/financial relationship that it is appropriate to disclose.
Other Talks in the Series: The Immune System - Key Concepts and Questions
Transcript
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0:00
Hello, my name is Smita Patel and I'm a clinical immunologist working
at the Oxford University Hospitals, NHS Foundation Trust.
I'm going to be talking about primary immunodeficiency disorders.
0:16
The learning objectives for this lecture are to gain
understanding of primary immune deficiencies and how they are classified,
to recognize how primary immune deficiencies present and relate
that presentation to the function and pathogenesis of the disease,
to understand how we investigate patients and undertake treatment.
I will cover specific conditions which may be
encountered within the setting of internal medicine.
0:45
The primary immunodeficiency disorders are a heterogeneous group of
disorders characterized by poor or absent function of the immune system,
this predisposes affected individuals to infections,
autoimmunity, inflammation, atopy, and malignancy.
There are almost 400 genetically defined primary immune deficiencies,
and understanding how the infections relate to the development and function
of the immune system is key to further investigation and hence treatment.
It may be, because primary immune deficiencies are rare,
that there is often a delay in diagnosis,
but there are conditions which will be seen by general physicians and it's
important to at least consider primary immune deficiency in the differential diagnosis.
Forms of antibody deficiency are the most common form of
primary immune deficiency and may present in adults,
however, most present in childhood,
and therefore, a complete history is crucial.