Live Vaccine Use and Safety in DiGeorge Syndrome

Hofstetter, Annika M.; Jakob, Kathleen; Klein, Nicola P.; Dekker, Cornelia L.; Edwards, Kathryn M.; Halsey, Neal A.; Baxter, Roger; Williams, Sarah E.; Graham, Philip L.; LaRussa, Philip

doi:10.1542/peds.2013-0831

Cited by 42 publications

(52 citation statements)

References 30 publications

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“…We did not study the tolerance after receiving the live vaccines in this population because we did not have the data. In other studies, the rate of mild adverse effects was similar to the general population (Hofstetter et al, ; Suksawat et al, ).…”

Section: Discussionsupporting

confidence: 65%

Risk factors of clinical dysimmune manifestations in a cohort of 86 children with 22q11.2 deletion syndrome: A retrospective study in France

Mahe¹,

Nagot

Portalès³

et al. 2019

American J of Med Genetics Pt A

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In this study, we describe the biological immune profiles and clinical dysimmune manifestations (infections, autoimmune diseases, and allergies) of patients with 22q11.2 deletion syndrome with the aim of determining risk factors for clinical events. This retrospective study concerned all the patients with 22q11 deletion syndrome attending the Montpellier University Hospital from January 1, 1992, to December 31, 2014 who had at least one immune investigation before the age of 18.We analyzed the clinical features, biological tests and the course of infections, autoimmunity, and allergy of 86 children. Among these 86 children, 48 (59%) had a low T lymphocyte level. Twenty-nine patients (34%) had a severe infection. The only risk factor for severe infection was the low level of CD4+ T-cells (OR: 3.3; 95% confidence interval (CI) [1.020-11.108]). Eleven patients (13%) developed an autoimmune disease; the only risk factor was an antecedent of severe infection (OR: 4.1; 95% CI [1.099-15.573]). Twenty-three patients (27%) had allergic episodes. A low level of CD8+ T-cells (OR: 3.2; 95% CI [1.07-9.409]) was significantly associated with allergy manifestations. Patients with 22q11 deletion syndrome have a high rate of dysimmune manifestations. We found statistic correlations among CD4+ T-cell count, infectious manifestations, and autoimmunity.

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Section: Discussionsupporting

confidence: 65%

Risk factors of clinical dysimmune manifestations in a cohort of 86 children with 22q11.2 deletion syndrome: A retrospective study in France

Mahe¹,

Nagot

Portalès³

et al. 2019

American J of Med Genetics Pt A

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“…We demonstrated that the risk of adverse events is low is this population as long as they have T cells. These data have been confirmed by an independent group and a large survey spearheaded by the CDC . The three studies found that administration of live viral vaccines was safe in this population.…”

Section: Managementsupporting

confidence: 53%

“…These data have been confirmed by an independent group and a large survey spearheaded by the CDC. 157,158 The three studies found that administration of live viral vaccines was safe in this population. Nevertheless, there is ongoing concern due to the recent description from our group of delayed onset granulomatous inflammation in immunodeficient individuals from vaccine-strain rubella.…”

Section: Immunologymentioning

confidence: 99%

Chromosome 22q11.2 deletion syndrome and DiGeorge syndrome

Sullivan

2018

Immunological Reviews

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Summary Chromosome 22q11.2 deletion syndrome is the most common microdeletion syndrome in humans. The effects are protean and highly variable, making a unified approach difficult. Nevertheless, commonalities have been identified and white papers with recommended evaluations and anticipatory guidance have been published. This review will cover the immune system in detail and discuss both the primary features and the secondary features related to thymic hypoplasia. A brief discussion of the other organ system involvement will be provided for context. The immune system, percolating throughout the body can impact the function of other organs through allergy or autoimmune disease affecting organs in deleterious manners. Our work has shown that the primary effect of thymic hypoplasia is to restrict T cell production. Subsequent homeostatic proliferation and perhaps other factors drive a Th2 polarization, most obvious in adulthood. This contributes to atopic risk in this population. Thymic hypoplasia also contributes to low regulatory T cells and this may be part of the overall increased risk of autoimmunity. Collectively, the effects are complex and often age‐dependent. Future goals of improving thymic function or augmenting thymic volume may offer a direct intervention to ameliorate infections, atopy, and autoimmunity.

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“…Related studies have revealed that many patients with 22q11.2 deletion syndrome with low T cell count had no serious adverse events after receiving mump-measles-rubella and varicella vaccines [25,26]. The rate of mild adverse effects was similar to that seen in the general population [25].…”

Section: Discussionmentioning

confidence: 75%