Optimising immunisation in children with 22q11 microdeletion.
22q11 microdeletion syndrome
DiGeorge syndrome
immunisation
velo-cardiofacial syndrome
Journal
Therapeutic advances in vaccines and immunotherapy
ISSN: 2515-1355
Titre abrégé: Ther Adv Vaccines Immunother
Pays: England
ID NLM: 101718382
Informations de publication
Date de publication:
2020
2020
Historique:
received:
20
12
2019
accepted:
21
07
2020
entrez:
5
11
2020
pubmed:
6
11
2020
medline:
6
11
2020
Statut:
epublish
Résumé
The condition known as 22q11 microdeletion syndrome has a broad phenotypic spectrum, with many affected individuals experiencing mild-to-moderate immunodeficiency. Currently, there are significant variations in live vaccine practices and immunological testing prior to live vaccine administration due to safety concerns and limited established guidelines. Queensland Children's Hospital (QCH) Child Development Unit, offers a state-wide 22q11 microdeletion clinic. This is a retrospective single-centre review, capturing the majority of children with 22q11 microdeletion in Queensland, Australia. We describe the live vaccination status of 134 children, age 0 to 18 years under our care between 2000 and 2018, adverse events following immunisation (AEFI) and the proportion of children who received additional pneumococcal coverage. An immunological investigation pathway prior to live vaccine administration is proposed. Of the 134 children, 124 were eligible for live vaccinations as per the Australian National Immunisation Program: 82% had received dose one of measles, mumps and rubella (MMR) vaccine, 77% had completed MMR dose two and 66% had completed varicella immunisation. There were no AEFI notifications reported. Of the total sample of children, 18% received a fourth dose of conjugate pneumococcal vaccine (Prevenar 7 or 13) and 16% received a dose of Pneumovax 23 from 4 years of age. Immunology workup practices were demonstrated to vary widely prior to live vaccine administration. Most patients' immune profiles were consistent with mild-to-moderate immunodeficiency. We propose an immunological investigation and vaccination pathway with the aim of providing guidance and consistency to clinicians caring for children with 22q11 microdeletion.
Sections du résumé
BACKGROUND
BACKGROUND
The condition known as 22q11 microdeletion syndrome has a broad phenotypic spectrum, with many affected individuals experiencing mild-to-moderate immunodeficiency. Currently, there are significant variations in live vaccine practices and immunological testing prior to live vaccine administration due to safety concerns and limited established guidelines.
METHODS
METHODS
Queensland Children's Hospital (QCH) Child Development Unit, offers a state-wide 22q11 microdeletion clinic. This is a retrospective single-centre review, capturing the majority of children with 22q11 microdeletion in Queensland, Australia. We describe the live vaccination status of 134 children, age 0 to 18 years under our care between 2000 and 2018, adverse events following immunisation (AEFI) and the proportion of children who received additional pneumococcal coverage. An immunological investigation pathway prior to live vaccine administration is proposed.
RESULTS
RESULTS
Of the 134 children, 124 were eligible for live vaccinations as per the Australian National Immunisation Program: 82% had received dose one of measles, mumps and rubella (MMR) vaccine, 77% had completed MMR dose two and 66% had completed varicella immunisation. There were no AEFI notifications reported. Of the total sample of children, 18% received a fourth dose of conjugate pneumococcal vaccine (Prevenar 7 or 13) and 16% received a dose of Pneumovax 23 from 4 years of age. Immunology workup practices were demonstrated to vary widely prior to live vaccine administration. Most patients' immune profiles were consistent with mild-to-moderate immunodeficiency.
CONCLUSION
CONCLUSIONS
We propose an immunological investigation and vaccination pathway with the aim of providing guidance and consistency to clinicians caring for children with 22q11 microdeletion.
Identifiants
pubmed: 33150298
doi: 10.1177/2515135520957139
pii: 10.1177_2515135520957139
pmc: PMC7580130
doi:
Types de publication
Journal Article
Langues
eng
Pagination
2515135520957139Informations de copyright
© The Author(s), 2020.
Déclaration de conflit d'intérêts
Conflict of interest: The authors declare that there is no conflict of interest.
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