Immunological characterization of an Italian PANDAS cohort
This cross-sectional study aimed to contribute to the definition of Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections (PANDAS) pathophysiology. An extensive immunological assessment has been conducted to investigate both immune defects, potentially leading to r...
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| Format: | Article |
| Language: | English |
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Frontiers Media S.A.
2024-01-01
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| Series: | Frontiers in Pediatrics |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fped.2023.1216282/full |
| _version_ | 1827391228494217216 |
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| author | Lucia Leonardi Giulia Lorenzetti Rita Carsetti Eva Piano Mortari Cristiana Alessia Guido Anna Maria Zicari Elisabeth Förster-Waldl Lorenzo Loffredo Marzia Duse Alberto Spalice |
| author_facet | Lucia Leonardi Giulia Lorenzetti Rita Carsetti Eva Piano Mortari Cristiana Alessia Guido Anna Maria Zicari Elisabeth Förster-Waldl Lorenzo Loffredo Marzia Duse Alberto Spalice |
| author_sort | Lucia Leonardi |
| collection | DOAJ |
| description | This cross-sectional study aimed to contribute to the definition of Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections (PANDAS) pathophysiology. An extensive immunological assessment has been conducted to investigate both immune defects, potentially leading to recurrent Group A β-hemolytic Streptococcus (GABHS) infections, and immune dysregulation responsible for a systemic inflammatory state. Twenty-six PANDAS patients with relapsing-remitting course of disease and 11 controls with recurrent pharyngotonsillitis were enrolled. Each subject underwent a detailed phenotypic and immunological assessment including cytokine profile. A possible correlation of immunological parameters with clinical-anamnestic data was analyzed. No inborn errors of immunity were detected in either group, using first level immunological assessments. However, a trend toward higher TNF-alpha and IL-17 levels, and lower C3 levels, was detected in the PANDAS patients compared to the control group. Maternal autoimmune diseases were described in 53.3% of PANDAS patients and neuropsychiatric symptoms other than OCD and tics were detected in 76.9% patients. ASO titer did not differ significantly between the two groups. A possible correlation between enduring inflammation (elevated serum TNF-α and IL-17) and the persistence of neuropsychiatric symptoms in PANDAS patients beyond infectious episodes needs to be addressed. Further studies with larger cohorts would be pivotal to better define the role of TNF-α and IL-17 in PANDAS pathophysiology. |
| first_indexed | 2024-03-08T17:07:37Z |
| format | Article |
| id | doaj.art-3e8d8c638a444ea7abfbf62b1637e67f |
| institution | Directory Open Access Journal |
| issn | 2296-2360 |
| language | English |
| last_indexed | 2024-03-08T17:07:37Z |
| publishDate | 2024-01-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Pediatrics |
| spelling | doaj.art-3e8d8c638a444ea7abfbf62b1637e67f2024-01-04T04:21:09ZengFrontiers Media S.A.Frontiers in Pediatrics2296-23602024-01-011110.3389/fped.2023.12162821216282Immunological characterization of an Italian PANDAS cohortLucia Leonardi0Giulia Lorenzetti1Rita Carsetti2Eva Piano Mortari3Cristiana Alessia Guido4Anna Maria Zicari5Elisabeth Förster-Waldl6Lorenzo Loffredo7Marzia Duse8Alberto Spalice9Department of Maternal, Infantile and Urological Sciences, Sapienza University of Rome, Rome, ItalyDepartment of Pediatrics, University of Rome Tor Vergata, Rome, ItalyB Cell Physiopathology Unit, Immunology Research Area, Bambino Gesù Children Hospital, Rome, ItalyB Cell Physiopathology Unit, Immunology Research Area, Bambino Gesù Children Hospital, Rome, ItalyDepartment of Maternal, Infantile and Urological Sciences, Sapienza University of Rome, Rome, ItalyDepartment of Maternal, Infantile and Urological Sciences, Sapienza University of Rome, Rome, ItalyDivision of Neonatology, Pediatric Intensive Care and Neuropediatrics, Department of Pediatrics & Adolescent Medicine, Center for Congenital Immunodeficiencies, Medical University of Vienna, Vienna, AustriaDepartment of Clinical, Internal Medicine, Anesthesiologic and Cardiovascular Sciences, Sapienza University of Rome, Rome, ItalyDepartment of Maternal, Infantile and Urological Sciences, Sapienza University of Rome, Rome, ItalyDepartment of Maternal, Infantile and Urological Sciences, Sapienza University of Rome, Rome, ItalyThis cross-sectional study aimed to contribute to the definition of Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections (PANDAS) pathophysiology. An extensive immunological assessment has been conducted to investigate both immune defects, potentially leading to recurrent Group A β-hemolytic Streptococcus (GABHS) infections, and immune dysregulation responsible for a systemic inflammatory state. Twenty-six PANDAS patients with relapsing-remitting course of disease and 11 controls with recurrent pharyngotonsillitis were enrolled. Each subject underwent a detailed phenotypic and immunological assessment including cytokine profile. A possible correlation of immunological parameters with clinical-anamnestic data was analyzed. No inborn errors of immunity were detected in either group, using first level immunological assessments. However, a trend toward higher TNF-alpha and IL-17 levels, and lower C3 levels, was detected in the PANDAS patients compared to the control group. Maternal autoimmune diseases were described in 53.3% of PANDAS patients and neuropsychiatric symptoms other than OCD and tics were detected in 76.9% patients. ASO titer did not differ significantly between the two groups. A possible correlation between enduring inflammation (elevated serum TNF-α and IL-17) and the persistence of neuropsychiatric symptoms in PANDAS patients beyond infectious episodes needs to be addressed. Further studies with larger cohorts would be pivotal to better define the role of TNF-α and IL-17 in PANDAS pathophysiology.https://www.frontiersin.org/articles/10.3389/fped.2023.1216282/fullPANDASPANSGABHSTNF-αIL-17immune defects |
| spellingShingle | Lucia Leonardi Giulia Lorenzetti Rita Carsetti Eva Piano Mortari Cristiana Alessia Guido Anna Maria Zicari Elisabeth Förster-Waldl Lorenzo Loffredo Marzia Duse Alberto Spalice Immunological characterization of an Italian PANDAS cohort Frontiers in Pediatrics PANDAS PANS GABHS TNF-α IL-17 immune defects |
| title | Immunological characterization of an Italian PANDAS cohort |
| title_full | Immunological characterization of an Italian PANDAS cohort |
| title_fullStr | Immunological characterization of an Italian PANDAS cohort |
| title_full_unstemmed | Immunological characterization of an Italian PANDAS cohort |
| title_short | Immunological characterization of an Italian PANDAS cohort |
| title_sort | immunological characterization of an italian pandas cohort |
| topic | PANDAS PANS GABHS TNF-α IL-17 immune defects |
| url | https://www.frontiersin.org/articles/10.3389/fped.2023.1216282/full |
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