Nineteen patients with PHO with onset in childhood are presented. In 7 cases the syndrome was found in other family members too. In clinical, X-ray scintigraphic, arteriographic, and plethysmographic investigations, joint affection was found in 14 patients. Clubbing of the fingers and toes was seen in all the patients and a periosteal reaction was found in the long bones in 11 patients. Arteriography was carried out in 5 patients and showed hypervascularization and arteriovenous anastomoses in the clubbed fingers. The gas analysis of the arterial blood did not show any pathological changes. The histological finding showed poorly vascularized and oedematous periarticular tissue and ossification in the periosteal area, while in the synovial membrane, slight, nonspecific inflammatory changes were seen.

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