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We studied eight affected and four unaffected individuals from a Colombian family with autosomal dominant diffuse high bone density. Affected individuals have normal, proportional height and high serum alkaline phosphatase activity. Radiographically, affected members exhibit generalized,
In 1984-1989 at the First Paediatric Clinic of the Motol Faculty Hospital six patients with the diagnosis of infantile cortical hyperostosis (Caffey-Silvermann syndrome) were hospitalized. Non-familial concurrence of the disease was involved. All children developed the disease before the age of 4.5
Prostaglandin E1 (PGE1) infusion is usually administered for short periods to maintain patency of ductus arteriosus in infants with cyanotic heart disease. Prolonged therapy may be necessary while patients are awaiting surgical treatment. Several side effects occur at the onset of the treatment,
The acquired hyperostosis syndrome (AHS) is a chronic inflammatory disorder of bone of unknown etiology. It is accompanied by circumscribed hyperostosis which can be associated with ossifying lesions at sites of tendinous and ligamentary insertions and erosive or non-erosive arthritis. The
A 56-year-old Japanese woman with a history of palmoplantar pustulosis was admitted for examination due to left femur pain. Radiography and computed tomography showed thickening of the bone on the outer portion of the left femur. Bone scintigraphy of the left femur showed intense radioactive uptake.
Hyperostosis generalisata with striations of the bones falls within the large group of sclerotic bone dysplasias and is characterised by a marked widening of cortical bone, sclerosis of the base of the skull, and dense striations in the cancellous bone. All the bones of the skeleton, with the
The authors report on 8 patients with the dominant type of endosteal hyperostosis (Worth's disease), found in the same kindred over 3 generations. Three were males, five females, from 5 to 49 years of age. Major radiographic features were an increased density of bones of the skull and spine, with
Periosteal hyperostosis (exostosis) was identified in 5.9% (11/188) of DBA/1 male mice 10-14 weeks old used for collagen-induced arthritis (CIA) efficacy testing of immunomodulatory biologics. Mice with and without CIA in the affected limb, and also control and treated groups, were involved, with
Cranial hyperostosis is a common secondary manifestation of intracranial meningiomas. This may occur with or without neoplastic invasion, apart or even remote from the growth. Alkaline phosphatase (AP) is one of many enzymes produced by meningiomas and is known to possess indirect ossifying
We describe a boy aged nine months with infantile cortical hyperostosis in association with cystic fibrosis. Symmetrical periosteal thickening was present in the clavicles, ribs, femora, humeri, ulnae and radii. Periosteal hyperostosis of the humerus developed in association with an increase in the
The first reported Spanish family with autosomal dominant endosteal hyperostosis is presented and two members in two different generations studied. Neurological involvement with sensorineural hearing loss, chronic intracranial hypertension, and mild corticospinal tract abnormalities were found in
The common side effects associated with the use of prostaglandins in newborn infants include apnoea, hyperthermia, diarrhoea, skin flushing and oedema. Periosteal reaction or cortical thickening of the bones, also known as cortical hyperostosis, is associated with a prolonged use of prostaglandins.
The ultrastructure and the immunohistochemical pattern of the cells which are responsible for the bone resorption in the cortical infantile hyperostosis were investigated. The osteoclasts present a great positivity to MB1 antigen and a low positivity to OKM5. Mononuclear cells with primary
We have examined the alkaline phosphatase (AP) activity of primary calvaria-derived osteoblast-like cells from the twy (tip-toe walking Yoshimura) and normal ICR control mouse. The twy mouse displays elevated osseous formation particularly in the spine, and the pathophysiological features resemble