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Idiopathic periosteal hyperostosis with dysproteinemia. A new clinical entity
R B Goldbloom1, P B Stein, A Eisen
1Paediatric Medical Service, Department of Radiology, Montreal Children's Hospital, Quebec.
Insights
This study describes a rare, transient illness in two children involving fever, bone pain, and subperiosteal new bone formation. Symptoms resolved completely, suggesting a temporary immune response.
Area of Science:
- Pediatrics
- Rheumatology
- Immunology
Background:
- Transient illnesses in children can present with complex symptoms.
- Subperiosteal new bone formation is a radiographic finding seen in various conditions.
- Dysproteinemia indicates abnormalities in serum protein levels.
Observation:
- Two unrelated children presented with fever, bone pain, and inability to walk following a respiratory infection.
- Radiographs revealed subperiosteal new bone formation, which resolved over time.
- Tibial biopsy showed right-angled new bone formation without inflammation, alongside mucinous edema.
Findings:
- The illness was characterized by transient fever, bone pain, and radiographic evidence of periosteal hyperostosis.
- Serum protein analysis showed hypoalbuminemia and elevated alpha2 and gamma globulins, particularly IgG.
- Bone marrow exhibited an increased number of plasma cells.
Implications:
- This transient condition, potentially linked to an immune response post-infection, has not been previously described.
- Understanding this entity is crucial for accurate pediatric diagnosis and management.
- Further research is needed to identify the specific etiologic agent and underlying mechanisms.
Abstract:
Two unrelated children, suffering from a transient illness characterized by fever, bone pain and tenderness, inability to walk, x-ray evidence of subperiosteal new bone formation and dysproteinemia, are described. In both children the onset followed a respiratory infection. The fever lasted for about three weeks in 1 patient and two months in the other. As fever subsided, the x-ray evidence of periosteal hyperostosis gradually disappeared, and the electrophoretic pattern of the serum proteins returned to normal. Tibial biopsy in 1 patient showed subperiosteal new bone formation of the right-angled type, with no inflammation and with the presence of mucinous edema. Plasma cells were unusually abundant in the bone marrow. The serum protein disturbance was characterized by hypoalbuminemia and striking elevation of the alpha2 and gamma globulins. Detailed serum protein studies performed in 1 patient showed the hypergammaglobulinemia to be due primarily to an increase in the IgG fraction. No etiologic agent was identified, and recovery was complete in both patients. The entity does not appear to have been described previously.
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