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Title: [Juvenile hyaline fibromatosis]. Author: Haddad J, Dandach A, Gebran S, Rhayel L, Aftimos G. Journal: Arch Pediatr; 1997 Dec; 4(12):1200-3. PubMed ID: 9538423. Abstract: BACKGROUND: Juvenile hyaline fibromatosis (JHF) is rare disease of autosomal recessive inheritance. CASE REPORT: A 14-year old boy, born to consanguineous parents was admitted because he suffered from multiple tumors since the age of 5 years. These tumors were mobile, painful varying in size, located mainly on the head, the back, and extremities; they were associated with gingival overgrowth. Bone X-rays showed osteolytic lesions. The cognitive development was normal. Biopsy of tumors showed cords of spindle-shaped cells embedded in homogeneous eosinophilic matrix. The child was progressively disabled due to articular changes. Two of his brothers presented the same features. CONCLUSION: This new case of JHF confirms the severity of prognosis of such a fibromatosis presently considered as a hereditary disorder of collagen metabolism.[Abstract] [Full Text] [Related] [New Search]