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Published on: November 9, 2017
Evolution and treatment of childhood chronic inflammatory polyneuropathy
Elsa Rossignol1, Guy D'Anjou, Normand Lapointe
1Service de neurologie, Département de pédiatrie, Centre hospitalier universitaire Sainte-Justine, Montréal, Québec, Canada.
Insights
Chronic inflammatory demyelinating polyneuropathy (CIDP) in children is rare but often treatable. This study reviews 13 pediatric cases, including refractory instances, to inform treatment strategies.
Area of Science:
- Pediatric Neurology
- Clinical Case Series
- Rare Diseases
Background:
- Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare autoimmune disorder affecting peripheral nerves.
- Pediatric CIDP typically responds to standard immunomodulatory therapies.
- A subset of pediatric CIDP cases exhibit resistance to conventional treatments.
Purpose of the Study:
- To describe the clinical course and treatment outcomes of pediatric CIDP patients.
- To review the literature on managing refractory CIDP in pediatric populations.
- To identify potential therapeutic approaches for treatment-resistant pediatric CIDP.
Main Methods:
- Retrospective review of 13 pediatric CIDP patients treated between 1975 and 2005.
- Inclusion of two recent cases with refractory disease.
- Comprehensive literature search on refractory CIDP treatments in children and adults.
Main Results:
- Most pediatric CIDP cases responded to standard therapies (immunoglobulins, corticosteroids, plasmapheresis).
- Two recent cases presented with refractory disease, highlighting treatment challenges.
- Literature review provided insights into alternative and advanced treatment options.
Conclusions:
- While most pediatric CIDP cases are manageable, refractory disease necessitates exploring alternative therapies.
- Understanding treatment responses in both pediatric and adult refractory CIDP is crucial.
- Further research is needed to establish optimal treatment guidelines for refractory pediatric CIDP.
Abstract:
Chronic inflammatory demyelinating polyneuropathy is a rare disease in pediatric patients. The disease usually responds well to standard therapies including immunoglobulins, corticosteroids, and plasmapheresis. However, a minority of cases appear refractory to standard treatments. This report presents the evolution of 13 patients with chronic inflammatory demyelinating polyneuropathy monitored in our pediatric neurology clinic between 1975 and 2005, including two recent patients with refractory diseases. The literature regarding treatment of refractory cases in adults and children is also reviewed.
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