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Predicting respiratory failure and outcome in pediatric Guillain-Barré syndrome
Joyce Roodbol1, Rudolf Korinthenberg2, Esmee Venema3
1Department of Neurology Erasmus MC- Sophia Children's Hospital, University Medical Center Rotterdam, the Netherlands; Department of Paediatric Neurology Erasmus MC- Sophia Children's Hospital, University Medical Center Rotterdam, the Netherlands.
Insights
A new model, EGRIS-Kids, accurately predicts respiratory failure in children with Guillain-Barré syndrome (GBS). The GBS disability score predicts recovery of walking ability, aiding personalized GBS patient care.
Area of Science:
- Pediatric Neurology
- Clinical Prognostication
- Epidemiology of Neurological Disorders
Background:
- Guillain-Barré syndrome (GBS) presents with variable clinical courses and outcomes, including respiratory failure and mobility impairment.
- Existing prognostic models like EGRIS are not accurate for pediatric GBS cases.
- There is a need for reliable prognostic tools tailored to children with GBS.
Purpose of the Study:
- To identify prognostic factors for respiratory failure and inability to walk in pediatric GBS.
- To develop a novel clinical prognostic model, EGRIS-Kids, for individual risk assessment in children with GBS.
Main Methods:
- A multicenter retrospective cohort study analyzed data from children (<18 years) diagnosed with GBS.
- Two independent cohorts from Germany, Switzerland, Austria, and The Netherlands were included.
- Primary outcomes assessed were respiratory failure and independent walking ability at one year post-diagnosis.
Main Results:
- The developed EGRIS-Kids model incorporates age, cranial nerve involvement, and GBS disability score at admission.
- EGRIS-Kids predicts respiratory failure risk (4-50%) with an AUC of 0.71.
- A lower GBS disability score at nadir was the strongest predictor for recovery of independent walking (OR 0.43).
Conclusions:
- EGRIS-Kids accurately predicts respiratory failure risk in pediatric GBS.
- The GBS disability score at admission effectively predicts the inability to walk.
- These tools enable personalized monitoring and treatment strategies for children with GBS.
Background:
Guillain-Barré syndrome (GBS) has a highly variable clinical course and outcome as indicated by the risk of developing respiratory failure and residual inability to walk. Prognostic models as Erasmus GBS Respiratory Insufficiency Score (EGRIS) developed in adult patients are inaccurate in children. Our aim was to determine the prognostic factors of respiratory failure and inability to walk in children with GBS and to develop a new clinical prognostic model for individual patients (EGRIS-Kids).
Methods:
A multicenter retrospective cohort study was performed using the data of children (younger than 18 years) fulfilling the diagnostic criteria for GBS from the NINDS. This study was performed in two independent cohorts from centers in Germany, Switzerland, Austria (N = 265, collected 1989-2002) and The Netherlands (N = 156, collected 1987-2016). The predicted main outcomes were occurrence of respiratory failure during the disease course and inability to walk independent at one year after diagnosis.
Results:
In the combined cohort of 421 children, 79 (19%) required mechanical ventilation and one patient died. The EGRIS-kids was developed including: age, cranial nerve involvement and GBS disability score at admission, resulting in a 9 point score predicting risks of respiratory failure ranging from 4 to 50% (AUC = 0.71). A lower GBS disability score at nadir was the strongest predictor of recovery to independent walking (at one month: OR 0.43 95%CI 0.25-0.74).
Conclusions:
EGRIS-Kids and GBS disability score at admission accurately predict the risk of respiratory failure and inability to walk respectively in children with GBS, as tools to personalize the monitoring and treatment.
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