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Related Concept Videos

Peripheral Arterial Disease II: Clinical Manifestations and Diagnostic Evaluation01:21

Peripheral Arterial Disease II: Clinical Manifestations and Diagnostic Evaluation

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Clinical manifestationsPeripheral Arterial Disease (PAD) manifests through a range of symptoms, from the characteristic intermittent claudication to atypical presentations and severe complications in advanced stages. Intermittent claudication, a hallmark symptom of PAD, presents as exercise-induced muscle pain that typically resolves within minutes of rest. This pain is reproducible and stems from inadequate blood flow, leading to the accumulation of lactic acid produced during anaerobic...
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Related Experiment Video

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Pediatric-Onset Chronic Inflammatory Demyelinating Polyneuropathy: A Multicenter Study.

Gamze Sarıkaya Uzan1, Atay Vural2, Deniz Yüksel3

  • 1Division of Child Neurology, Department of Pediatrics, Dokuz Eylül University Faculty of Medicine, İzmir, Turkey.

Pediatric Neurology
|May 28, 2023
PubMed
Summary

Pediatric chronic inflammatory demyelinating polyneuropathy (CIDP) treatment with intravenous immunoglobulin (IVIg) or IVIg plus steroids showed equal efficacy. MRI findings may correlate with clinical severity but not treatment response in pediatric CIDP patients.

Keywords:
ChildhoodChronic inflammatory demyelinating polyneuropathyIVIgImmunotherapyNeurofascin antibodiesSteroids

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Area of Science:

  • Neurology
  • Immunology
  • Pediatrics

Background:

  • Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare autoimmune disorder affecting peripheral nerves.
  • Pediatric-onset CIDP presents unique diagnostic and therapeutic challenges.
  • Understanding the clinical and demographic features of pediatric CIDP is crucial for effective management.

Purpose of the Study:

  • To evaluate the clinical and demographic characteristics of pediatric-onset CIDP in Turkey.
  • To assess the efficacy of different treatment modalities for pediatric CIDP.
  • To investigate the association between MRI findings and clinical outcomes in pediatric CIDP.

Main Methods:

  • Retrospective review of clinical data from pediatric CIDP patients (2010-2021).
  • Evaluation based on the 2021 Joint Task Force Guideline for CIDP management.
  • Comparison of treatment outcomes between intravenous immunoglobulin (IVIg) monotherapy and IVIg plus steroid combination therapy.
  • Analysis of magnetic resonance imaging (MRI) characteristics and their correlation with clinical severity.

Main Results:

  • A total of 43 pediatric patients (51.2% male) were included.
  • Significant improvement in modified Rankin scale (mRS) scores was observed post-treatment for all patients.
  • First-line treatments (IVIg vs. IVIg + steroids) showed comparable efficacy (P > 0.05).
  • Abnormal MRI findings were associated with higher pretreatment mRS scores (P < 0.05).

Conclusions:

  • First-line immunotherapy, including IVIg and IVIg plus steroids, demonstrated equal efficacy in treating pediatric CIDP.
  • MRI features may be linked to disease severity but do not appear to influence treatment response in pediatric CIDP.