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Intestinal pseudo-obstruction as an initial presentation of systemic sclerosis in two children
O Ortiz-Alvarez1, D Cabral, J S Prendiville
1Department of Pediatrics, University of British, Columbia Vancouver, Canada.
British Journal of Rheumatology
|February 1, 1997
Summary
Systemic sclerosis initially presented as intestinal pseudo-obstruction in two children. Treatments led to symptom improvement, though gastrointestinal radiological changes persisted.
Area of Science:
- Pediatrics
- Gastroenterology
- Rheumatology
Background:
- Systemic sclerosis is a rare autoimmune disease affecting connective tissues.
- Gastrointestinal involvement is common in systemic sclerosis, but initial presentation as intestinal pseudo-obstruction in children is infrequent.
Observation:
- Two pediatric cases of systemic sclerosis are presented.
- Intestinal pseudo-obstruction was the primary presenting symptom in both children.
Findings:
- Both patients showed improvement in gastrointestinal symptoms and skin manifestations after treatment.
- Case 1 received prednisone and penicillamine; Case 2 was treated with methotrexate.
- Radiological evidence of gastrointestinal changes persisted for 3 years (Case 1) and 2 years (Case 2) post-treatment.
Implications:
- Early diagnosis and treatment of systemic sclerosis can lead to symptom resolution in pediatric patients.
- Intestinal pseudo-obstruction should be considered in the differential diagnosis of pediatric gastrointestinal issues.
- Long-term monitoring for persistent radiological changes is warranted even after clinical improvement.