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Paeds Vivasallergy-and-immunology

Paeds Vivas · allergy-and-immunology

Antibody deficiencies — branching viva

Branching viva on antibody deficiencies in children: recognising the recurrent-infection pattern that earns an immunoglobulin work-up, interpreting the work-up functionally with a vaccine response, classifying into a primary inborn error or a secondary cause, deciding who needs immunoglobulin replacement, and protecting the lung.

branching clinical structured oral1 min readVerification in progress

Target exams

RACP DCEMRCPCH ClinicalRCPSC Pediatrics
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Study tools

Target exams

RACP DCEMRCPCH ClinicalRCPSC Pediatrics
Prompt
Outpatient clinic: a seven-month-old boy presents with his second lobar pneumonia this year, both requiring intravenous antibiotics. His maternal uncle died in infancy. On examination his tonsils are absent and there are no palpable lymph nodes. Total IgG, IgA and IgM are all markedly low. The examiner asks: what is your framework, what is the most likely diagnosis, how will you confirm it, and what are the principles of long-term management — then branches to a thriving toddler with a single low IgG and asks you to justify why you would NOT start immunoglobulin.

Opening question

Take me through your framework when a child presents with recurrent infection that you suspect might be due to an antibody deficiency. [1] [2]

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References5ShowHide
  1. [1]Boyle JM, Buckley RH. Population prevalence of diagnosed primary immunodeficiency diseases in the United States. J Clin Immunol, 2007.PMID 17577648
  2. [2]Bousfiha A, Moundir A, Tangye SG, et al. The 2022 Update of IUIS Phenotypical Classification for Human Inborn Errors of Immunity. J Clin Immunol, 2022.PMID 36198931
  3. [3]Winkelstein JA, Marino MC, Lederman HM, et al. X-linked agammaglobulinemia: report on a United States registry of 201 patients. Medicine (Baltimore), 2006.PMID 16862044
  4. [4]Ameratunga R, Gillis D, Steele R Diagnostic criteria for common variable immunodeficiency disorders. J Allergy Clin Immunol Pract, 2016.PMID 27587325
  5. [6]Seidel MG, Kindle G, Gathmann B, et al. The European Society for Immunodeficiencies (ESID) Registry Working Definitions for the Clinical Diagnosis of Inborn Errors of Immunity. J Allergy Clin Immunol Pract, 2019.PMID 30776527
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