Paeds Cases · allergy-and-immunology
Explaining a primary immunodeficiency diagnosis and immunoglobulin replacement — OSCE
Communication and structured-discussion OSCE on explaining a new diagnosis of common variable immunodeficiency to the family of an eight-year-old girl referred for recurrent sinopulmonary infection and emerging bronchiectasis — including what the diagnosis means, why immunoglobulin replacement is needed, the practicalities of long-term therapy, infection-prevention and vaccination strategy, genetic counselling and the school and transition plan, and addressing the family's anxiety about the lifelong nature of the condition and the father's hope that the recurrent infections were 'just a phase'.
On this page
Study tools
Target exams
Candidate instructions (8-minute station)
You are the paediatric registrar in the general paediatric clinic. An eight-year-old girl has been referred for recurrent otitis media, sinusitis and pneumonia, with two hospital admissions in the past year and new crackles and wheeze suggesting early bronchiectasis. Her blood tests show low IgG and IgA and a poor response to a pneumococcal vaccine challenge, and the immunology team has confirmed a diagnosis of common variable immunodeficiency — a primary antibody deficiency. [7]
Your tasks are: [1]
- Explain the diagnosis of common variable immunodeficiency in language the family can understand. [7]
- Explain why immunoglobulin replacement therapy is needed, what it involves practically, and what it will and will not achieve. [7]
- Cover the infection-prevention and vaccination strategy, including which vaccines are safe and which need coordination with immunology. [5]
- Address the genetic counselling, the school and transition plan, and the father's belief that the recurrent infections were "just a phase". [1]
You should name the immunology team as the long-term lead for her care and explain what ongoing surveillance will involve. [5]
References3ShowHide
- [7]Fried AJ; Bonilla FA Pathogenesis, diagnosis, and management of primary antibody deficiencies and infections. Clin Microbiol Rev, 2009.PMID 19597006
- [5]de Vries E; European Society for Immunodeficiencies (ESID) members Patient-centred screening for primary immunodeficiency, a multi-stage diagnostic protocol designed for non-immunologists: 2011 update. Clin Exp Immunol, 2012.PMID 22132890
- [1]Subbarayan A; Colarusso G; Hughes SM; Gennery AR; Slatter M; Cant AJ; Barge D; Flood T; Abinun M; Hambleton S Clinical features that identify children with primary immunodeficiency diseases. Pediatrics, 2011.PMID 21482601