9 research outputs found
Corrigendum: Increase in pediatric recurrent fever evaluations during the first year of the COVID-19 pandemic in North America
[This corrects the article DOI: 10.3389/fped.2023.1240242.]
Increase in pediatric recurrent fever evaluations during the first year of the COVID-19 pandemic in North America
The impact of the COVID-19 pandemic on new diagnoses of recurrent fevers and autoinflammatory diseases is largely unknown. The Childhood Arthritis and Rheumatology Research Alliance (CARRA) PFAPA/AID Working Group aimed to investigate the impact of the COVID-19 pandemic on the number of pediatric patients evaluated for recurrent fevers and autoinflammatory diseases in North America. The absolute number of new outpatient visits and the proportion of these visits attributed to recurrent fever diagnoses during the pre-pandemic period (1 March 2019–29 February 2020) and the first year of the COVID-19 pandemic (1 March 2020–28 February 2021) were examined. Data were collected from 27 sites in the United States and Canada. Our results showed an increase in the absolute number of new visits for recurrent fever evaluations in 21 of 27 sites during the COVID-19 pandemic compared to the pre-pandemic period. The increase was observed across different geographic regions in North America. Additionally, the proportion of new visits to these centers for recurrent fever in relation to all new patient evaluations was significantly higher during the first year of the pandemic, increasing from 7.8% before the pandemic to 10.9% during the pandemic year (p < 0.001). Our findings showed that the first year of the COVID-19 pandemic was associated with a higher number of evaluations by pediatric subspecialists for recurrent fevers. Further research is needed to understand the reasons behind these findings and to explore non-infectious triggers for recurrent fevers in children
Recommended from our members
Increase in pediatric recurrent fever evaluations during the first year of the COVID-19 pandemic in North America.
The impact of the COVID-19 pandemic on new diagnoses of recurrent fevers and autoinflammatory diseases is largely unknown. The Childhood Arthritis and Rheumatology Research Alliance (CARRA) PFAPA/AID Working Group aimed to investigate the impact of the COVID-19 pandemic on the number of pediatric patients evaluated for recurrent fevers and autoinflammatory diseases in North America. The absolute number of new outpatient visits and the proportion of these visits attributed to recurrent fever diagnoses during the pre-pandemic period (1 March 2019-29 February 2020) and the first year of the COVID-19 pandemic (1 March 2020-28 February 2021) were examined. Data were collected from 27 sites in the United States and Canada. Our results showed an increase in the absolute number of new visits for recurrent fever evaluations in 21 of 27 sites during the COVID-19 pandemic compared to the pre-pandemic period. The increase was observed across different geographic regions in North America. Additionally, the proportion of new visits to these centers for recurrent fever in relation to all new patient evaluations was significantly higher during the first year of the pandemic, increasing from 7.8% before the pandemic to 10.9% during the pandemic year (p < 0.001). Our findings showed that the first year of the COVID-19 pandemic was associated with a higher number of evaluations by pediatric subspecialists for recurrent fevers. Further research is needed to understand the reasons behind these findings and to explore non-infectious triggers for recurrent fevers in children
Recommended from our members
Corrigendum: Increase in pediatric recurrent fever evaluations during the first year of the COVID-19 pandemic in North America.
[This corrects the article DOI: 10.3389/fped.2023.1240242.]
Recommended from our members
Increase in Pediatric Recurrent Fever Evaluations During the First Year of the COVID-19 Pandemic in North America
The impact of the COVID-19 pandemic on new diagnoses of recurrent fevers and autoinflammatory diseases is largely unknown. The Childhood Arthritis and Rheumatology Research Alliance (CARRA) PFAPA/AID Working Group aimed to investigate the impact of the COVID-19 pandemic on the number of pediatric patients evaluated for recurrent fevers and autoinflammatory diseases in North America. The absolute number of new outpatient visits and the proportion of these visits attributed to recurrent fever diagnoses during the pre-pandemic period (1 March 2019-29 February 2020) and the first year of the COVID-19 pandemic (1 March 2020-28 February 2021) were examined. Data were collected from 27 sites in the United States and Canada. Our results showed an increase in the absolute number of new visits for recurrent fever evaluations in 21 of 27 sites during the COVID-19 pandemic compared to the pre-pandemic period. The increase was observed across different geographic regions in North America. Additionally, the proportion of new visits to these centers for recurrent fever in relation to all new patient evaluations was significantly higher during the first year of the pandemic, increasing from 7.8% before the pandemic to 10.9% during the pandemic year ( \u3c 0.001). Our findings showed that the first year of the COVID-19 pandemic was associated with a higher number of evaluations by pediatric subspecialists for recurrent fevers. Further research is needed to understand the reasons behind these findings and to explore non-infectious triggers for recurrent fevers in children
Consensus treatment plans for periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome (PFAPA): a framework to evaluate treatment responses from the childhood arthritis and rheumatology research alliance (CARRA) PFAPA work group
Abstract
Background
Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is the most common periodic fever syndrome in children. There is considerable heterogeneity in management strategies and a lack of evidence-based treatment guidelines. Consensus treatment plans (CTPs) are standardized treatment regimens that are derived based upon best available evidence and current treatment practices that are a way to enable comparative effectiveness studies to identify optimal therapy and are less costly to execute than randomized, double blind placebo controlled trials. The purpose of this project was to develop CTPs and response criteria for PFAPA.
Methods
The CARRA PFAPA Working Group is composed of pediatric rheumatologists, infectious disease specialists, allergists/immunologists and otolaryngologists. An extensive literature review was conducted followed by a survey to assess physician practice patterns. This was followed by virtual and in-person meetings between 2014 and 2018. Nominal group technique (NGT) was employed to develop CTPs, as well as inclusion criteria for entry into future treatment studies, and response criteria. Consensus required 80% agreement.
Results
The PFAPA working group developed CTPs resulting in 4 different treatment arms: 1. Antipyretic, 2. Abortive (corticosteroids), 3. Prophylaxis (colchicine or cimetidine) and 4. Surgical (tonsillectomy). Consensus was obtained among CARRA members for those defining patient characteristics who qualify for participation in the CTP PFAPA study.
Conclusion
The goal is for the CTPs developed by our group to lead to future comparative effectiveness studies that will generate evidence-driven therapeutic guidelines for this periodic inflammatory disease
Single amino acid charge switch defines clinically distinct proline-serine-threonine phosphatase-interacting protein 1 (PSTPIP1)-associated inflammatory diseases
Background: Hyperzincemia and hypercalprotectinemia (Hz/Hc) is a
distinct autoinflammatory entity involving extremely high serum
concentrations of the proinflammatory alarmin myeloid-related protein
(MRP) 8/14 (S100A8/S100A9 and calprotectin).
Objective: We sought to characterize the genetic cause and clinical
spectrum of Hz/Hc.
Methods: Proline-serine-threonine phosphatase-interacting protein 1
(PSTPIP1) gene sequencing was performed in 14 patients with Hz/Hc, and
their clinical phenotype was compared with that of 11 patients with
pyogenic arthritis, pyoderma gangrenosum, and acne (PAPA) syndrome.
PSTPIP1-pyrin interactions were analyzed by means of immunoprecipitation
and Western blotting. A structural model of the PSTPIP1 dimer was
generated. Cytokine profiles were analyzed by using the multiplex
immunoassay, and MRP8/14 serum concentrations were analyzed by using an
ELISA.
Results: Thirteen patients were heterozygous for a missense mutation in
the PSTPIP1 gene, resulting in a p.E250K mutation, and 1 carried a
mutation resulting in p. E257K. Both mutations substantially alter the
electrostatic potential of the PSTPIP1 dimer model in a region critical
for protein-protein interaction. Patients with Hz/Hc have extremely high
MRP8/14 concentrations (2045 +/- 1300 mu g/mL) compared with those with
PAPA syndrome (116 +/- 74 mu g/mL) and have a distinct clinical
phenotype. A specific cytokine profile is associated with Hz/Hc. Hz/Hc
mutations altered protein binding of PSTPIP1, increasing interaction
with pyrin through phosphorylation of PSTPIP1.
Conclusion: Mutations resulting in charge reversal in the y-domain of
PSTPIP1 (E -> K) and increased interaction with pyrin cause a distinct
autoinflammatory disorder defined by clinical and biochemical features
not found in patients with PAPA syndrome, indicating a unique
genotype-phenotype correlation for mutations in the PSTPIP1 gene. This
is the first inborn autoinflammatory syndrome in which inflammation is
driven by uncontrolled release of members of the alarmin family