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Related Experiment Videos

[Sharp syndrome in an infant: a case report].

L Ratovo1, E Bérard, J Khaida

  • 1Clinique médicale infantile, CHU de Nice, hôpital de Cimiez, France.

Pediatrie
|January 1, 1992
PubMed
Summary

Sharp syndrome, a rare autoimmune disorder, was diagnosed late in an 11-year-old boy. Treatment with corticosteroids and antimalarial drugs led to satisfactory long-term outcomes.

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Area of Science:

  • Rheumatology
  • Pediatric Autoimmunity
  • Systemic Autoimmune Diseases

Background:

  • Sharp syndrome, also known as mixed connective tissue disease (MCTD), is a rare autoimmune disorder.
  • Late diagnosis in pediatric cases can complicate management and prognosis.

Observation:

  • An 11-year-old boy with late-diagnosed Sharp syndrome experienced disease progression.
  • He presented with myopericardic aggravation, glomerular disease, and seizures with delirium despite initial corticosteroid therapy.

Findings:

  • High-dose steroid therapy initially improved symptoms.
  • Early switch to synthetic antimalarial drugs resulted in sustained satisfactory outcomes over six years.

Implications:

  • This case highlights the importance of timely diagnosis and aggressive management in pediatric Sharp syndrome.
  • Synthetic antimalarial drugs may be a valuable therapeutic option for managing refractory or advanced cases, offering long-term benefits.

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