|Title||Primary immune deficiency 1|
By the end of the training period trainees will, in respect of primary immune deficiencies, their presentation, investigation and treatment, be able to:
- analyse, synthesise, evaluate and apply knowledge
- perform a range of technical and clinical skills and procedures
- demonstrate the attitudes and behaviours necessary for professional practice as a consultant clinical scientist dealing with the complexities, uncertainties and tensions of professional practice at this level.
Knowledge and understanding
By the end of the training period the trainee will be able to demonstrate the ability to analyse, evaluate and synthesise relevant knowledge and its application to their professional practice in relation to:
- appropriate laboratory investigations
- therapeutic interventions:
- putative mechanisms of action of various immunological therapies
- principles of the various therapeutic options to guide the most appropriate treatment for an individual patient
- the biology of all PIDs
- diagnostic criteria and their application for all PIDS (for a non-exhaustive list see www.esid.org/clinical-diagnostic-criteria-for-pid-73-0)
- the tests used in diagnosis for all PIDS, does not need to include details of specific tests done in one of or fewer centres in the UK
- the principles of management for all PIDS
- first-line treatments and onward referral (e.g. PCP prophylaxis for a new SCID) and shared care (of a periodic fever patient) of rarer PIDS
- CVID, SpAD, IGAD, XLA,HIGM syndromes, transient hypogamma of infancy Job’s, CGD, HAE
- understand when to suspect PID and how to investigate.
Note: for details of primary immunodeficiency diseases, see:
- Report of IUIS Scientific Committee. Clin and Exp Immunology 1999;118 (Suppl):1–28.
- Chapel HM. IUIS report on immunodeficiency disease: an update. Clin Exp Immunol. 2003;132:9–15.
- Bonilla FA, Geha RS. Update on primary immunodeficiency diseases. J Allergy Clin Immunol 2006; 117(2 Suppl Mini-Primer):S435–41.
Technical and clinical skills
By the end of the training period the trainee will be able to demonstrate a critical understanding of current relevant research, theory and knowledge and its application to the performance of the following technical procedures and laboratory skills:
- the principles and application of particular tests including quantification of immunoglobulin, flow cytometry, complement components, etc.
- interpretation of the results, any action that may be required for unusual results and basic QC and QA for the curriculum topics listed above.
By the end of the training period the trainee will be able to apply knowledge of primary immune deficiencies, their presentation, investigation and treatment, to perform the clinical skills necessary to manage under supervision the following.
- history taking
- physical examination
- selection of appropriate ancillary investigations
- formulating differential diagnoses.
To use knowledge of therapeutic options and understanding of the principles of their actions to aid clinical decision making.
Familiarity with national guidance on appropriate test selection and use where relevant, e.g. NICE.
Attitudes and behaviours
This module has no attitude and behaviours information.