Congenital Cortical Hyperostosis (Subscribe)  

CAFFEY DISEASE Alternative titles; symbols INFANTILE (CONGENITAL)CORTICAL HYPEROSTOSIS PRENATAL CORTICAL HYPEROSTOSIS, LETHAL, INCLUDED Caffey disease is caused by mutation in the alpha-1 collagen type I gene (COL1A1; 120150). CLINICAL FEATURES Infantile cortical hyperostosis has somewhat unusual features for a hereditary disorder. It rarely if ever appears after 5 months of age and usually...

Disease Database Caffey's disease aka/or Infantile cortical hyperostosis aka/or Roske-De Toni-Caffey-Smyth syndrome aka/or Caffey De Toni Silvermann syndrome may cause or feature - Miscellaneous syndromes Hyperostosis Symptoms and Signs Genu varum X-ray abnormalities Periosteal reaction belong(s) to the category of Autosomal dominant conditions Caffey's disease: Definition(s) via UMLS "A disease of young infants characterized by soft tissue swellings over the affected bones, fever, and irritability, and marked...

J Clin Invest. 2005 May 2; 115(5): 1142¨C1144Caffey disease: an unlikely collagenopathy Francis H. Glorieux Departments of Surgery, Pediatrics, and Human Genetics, McGill University, and Genetics Unit, Shriners Hospital for Children, Montr¨¦al, Quebec, Canada. Infantile cortical hyperostosis (also known as Caffey disease) is characterized by hyperirritability, acute inflammation of soft tissues, and profound...

"infantile cortical hyperostosis"
* etiology unknown
* onset before 5 months of age
* hyperostosis + periosteal reaction
o mandible (80-95%)
o clavicles
...

Caffey disease, or infantile cortical hyperostosis, is a benign, rare, proliferating bone disease affecting infants. Caffey and Silverman first reported this disease as a distinct entity in 1945. Classically, Caffey disease occurs in the early part of the first year of life (<5 mo). It is characterized by a clinical triad...

Also known as: Caffey's syndrome I Caffey-Smyth syndrome Smyth-Caffey syndrome De Toni-Caffey syndrome De Toni-Caffey-Silverman syndrome De Toni-Silverman-Caffey syndrome Roske-De Toni-Caffey syndrome Roske-De Toni-Caffey-Silverman syndrome Roske-De Toni-Caffey-Smyth syndrome Description: Probably familial disease of infants affecting skeleton and adjacent tissues. It is characterized by fever, irritability, swelling of soft tissues, and cortical bone thickening. The mandible is usually involved, less commonly the...

Caffey's disease or Infantile cortical hyperostosis or Roske-De Toni-Caffey-Smyth syndrome or Caffey De Toni Silvermann syndrome. "A disease of young infants characterized by soft tissue swellings over the affected bones, fever, and irritability, and marked by periods of remission and exacerbation. (Dorland, 27th ed)" Features Miscellaneous syndromes Hyperostosis Symptoms and Signs Genu varum X-ray abnormalities Periosteal reaction

Infantile cortical hyperostosis D Suri, D Dayal and M Singh Advanced Pediatrics Center, -160012, India; Keywords: Caffeys disease A 14 week old male infant presented with multiple tender bony swellings involving the legs, forearm, and lower jaw since 1 month of age (fig 1). No history of fever, trauma, or child...

Indian Pediatrics 2005; 42:64-66 Abstract: Infantile cortical hyperostosis (Caffey disease) is characterized by radiological evidence of cortical hyperostosis, soft tissue swellings, fever and irritability. We report a case of Caffey disease highlighting its presentation as pyrexia of unknown origin, appearance on radionuclide bone scintigraphy and our unsatisfactory experience of...

In 1945, Caffey first described infantile cortical hyperostosis, also known as Caffey disease, a self-limited disorder that affects infants and causes bone changes, soft-tissue swelling, and irritability.1 Although the etiology of this condition is not completely understood, familial and sporadic forms appear to exist
Infantile cortical hyperostosis is an inflammatory process...

Infantile cortical hyperostosis Objectives
1. Describe the clinical features of infantile cortical hyperostosis (Caffey's disease)
2. List two other conditions causing periosteal reaction in the same age range as infantile cortical hyperostosis
3. Discuss the natural history of infantile cortical hyperostosis

URL http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&term=%22Hyperostosis%2C+Cortical%2C+Congenital%22%5BMAJR%5D