infantile cortical hyperostosis

(redirected from Caffey-Silverman syndrome)
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Related to Caffey-Silverman syndrome: Caffey Syndrome

hyperostosis

 [hi″per-os-to´sis]
excessive growth of bony tissue. adj., adj hyperostot´ic.
frontal internal hyperostosis (hyperostosis fronta´lis inter´na) a new formation of bone tissue protruding in patches on the internal surface of the cranial bones in the frontal region, most commonly affecting women near menopause.
generalized cortical hyperostosis a hereditary disorder beginning during puberty, marked by osteosclerosis of the skull, mandible, clavicles, ribs, and diaphyses of long bones, associated with elevated blood alkaline phosphatase.
infantile cortical hyperostosis a syndrome seen in infants under six months of age, marked by fever, arthralgias, and swelling and cortical thickening of facial, trunk, and long bones. Called also Caffey's disease.

in·fan·tile cor·ti·cal hy·per·os·to·sis

[MIM*114000]
neonatal subperiosteal bone formation over many bones, especially the mandible, clavicles, and the shafts of long bones; it follows fever, usually appearing before 6 months of age and disappearing during childhood; familial cases are inherited as autosomal dominant.

Caffey disease

An autosomal dominant condition MIM 114000 characterised by fever, inflammation, swelling of affected bones (tibia, mandible, ulna and facial bones) and restlessness.

Molecular pathology
Caffey disease is linked to a mutation of COL1A1.

infantile cortical hyperostosis

Caffey disease An AD condition characterized by early onset hyperostosis and neo-osteogenesis, of facial and, less commonly, long bones, soft tissue swelling, hyperirritability, dysphagia, fever, pleuritis Lab ↑ ESR, alk phos

Caffey,

John Patrick, U.S. physician, radiologist, and pediatrician, the father of pediatric radiology, 1895-1978.
Caffey-Silverman syndrome - Synonym(s): Caffey syndrome
Caffey disease - Synonym(s): Caffey syndrome
Caffey syndrome - neonatal subperiosteal bone formation over many bones, especially the mandible and clavicles and the shafts of long bones. Synonym(s): Caffey disease; Caffey-Silverman syndrome; infantile cortical hyperostosis