SEARCH · PubMed Health
Explore indexed PubMed citations for clinical trials, systematic reviews and public health research. Read source abstracts and follow each citation to its original PubMed record.
Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.
A case is presented of progressive acro-osteolysis of the hands combined with progressive mandibular ramus osteolysis and multiple cortical defects. The features of acro-osteolysis are reviewed and classified.
A case of a rare, non-familial, generalized, congenital, osseous dysplasia is described with features which have previously been described under the name 'acro-osteolysis'. Basilar invagination followed defective bone formation in the skull and this produced occipital headache and progressive neurological deterioration with involvement of the lower cranial nerves and cerebellar function. Hydrocephalus and valvular impaction of the cerebellum in the foramen magnum were demonstrated. Partial relief was obtained by foramen magnum decompression; death supervened from infection and respiratory inadequacy at the age of 20.
A mother and son with acro-osteolysis (Hajdu-Cheney syndrome) are described. In addition to osteolysis of the distal phalanges, these patients have a generalized osseous dysplasia with osteoporosis, premature loss of teeth, short stature and a distinctive facial appearance. In one of the cases an enlarged sella turcica was associated with no abnormality of endocrine function. A biopsy specimen taken from an area of active osteolysis in a phalanx was studied by light and electron microscopy. There was active replacement of central medullary bone by a fibrous and angiomatous process characterized by the presence of small, thick-walled vessels and an unusual number of interspersed nerve fibers and mast cells. A neurovascular dysfunction with local release of osteolytic mediators may be involved in the pathogenesis of the disorder, but the nature of the osteolytic factor is unknown.
A six year old girl. With a clinical picture of acro-osteolysis idiopathic non familial associated with other anomalies is presented. Literature is reviewed and the clinical, radiologic, pathologic and etiopathogenetic features are commented, pointing out the diferent associated abnormalities that other authors did not consign: facial, anacraneal dysplasia, epiphyseal separations and dislocations of radius, and peroneal incurvation.
Occupational acro-osteolysis is a serious disease definitely related to the inhalation of agents involved in the polymerization of vinyl chloride. After variable periods of latency, the more or less characteristic clinical picture appears. A Raynaud's phenomenon is usually a premonitory sign indicating that alterations in bone will probably ensue. Scleroderma-like skin lesions and systemic involvement of variable degree occur frequently. The pathophysiology of the disease is unknown, but obstructive lesions of small peripheral arteries are thought to cause osteolysis of the distal phalanges of the fingers.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
The report concerns a description of a case of hereditary acroosteolysis with peculiar clinical and X-ray symptoms. It is being stressed that the neurological changes in different cases differ by their manifold signs and are sometimes the prevalent syndrome. The authors conducted a genetical analysis of 11 cases of hereditary acroosteolysis, which were seen in Dagestan. The paper contains some considerations of the differencies of hereditary acroosteolysis from other similar disorders.
Hadjdu-Cheney syndrome is a rare type of syndrome characterized by acro-osteolysis, dolichocephaly with multiple Wormian bones, absence of frontal sinuses and joint laxity. A case of this syndrome is presented. A histological study of the osteolytic lesion revealed destruction characterized by microfractures with a poor reparative process. It is postulated that an abnormality of osteoblast or osteoid function is the pathogenesis of this syndrome.
Four cases of non-familiar acropathia ulcero-mutilans (Bureau-Barriere) are described. In all cases there was a history of alcoholism, abnormal liver function, painless foot ulcers and polyneuritis. The radiological changes were confined to the lower extremities. There were osteolytic changes in the metatarsal heads and the bases of the proximal phalanges, particularly of the great toes; in addition, there was general demineralisation of the bones of the feet. One case was remarkable in that the tarsal bones and the proximal protions of the metatarsals were also involved. A variety of bone deformities were observed, and there were pathological fractures as well as subluxation and dislocation. The differential diagnosis between familial and non-familial acro-osteolysis, and of some other bone abnormalities is discussed. A multifactorial pathogenesis of the disease is suggested.
Vinyl chloride (VC) disease is a multisystem disorder incorporating Raynaud's phenomenon, acro-osteolysis, thrombocytopenia, portal fibrosis, and hepatic and pulmonary dysfunction. Immunological and immunochemical investigations showed the presence of circulating immune complexes in 19 out of 28 patients with the disease and in a further two out of 30 workers exposed to VC. The immunological data were reviewed in relation to the clinical picture of the disease and to the available evidence on the metabolism of VC. The results suggest that VC disease is an immune complex disorder and that the immune response is initiated by the adsorption of VC or a metabolite on to tissue or plasma protein.
Although polyvinyl chloride has been produced from vinyl chlride monomer for more than 40 years, recognition of toxicity among vinyl chloride polymerization workers is more recent. In the mid 1960s, workers involved in cleaning polymerization tanks were found to have acro-osteolysis. In 1974, the same population of workers was found to be at risk for an unusual type of hepatic fibrosis and angiosarcoma of the liver. We describe two cases of vinyl chloride-associated liver injury, one of hepatic fibrosis and one of angiosarcoma. Histologic features of these lesions are similar to the hepatic fibrosis and angiosarcomas resulting from chronic exposure to inorganic arsenicals. Preliminary studies suggest that the toxicity of vinyl chloride may result from formation, during high-dose exposure, of active metabolites by mixed function oxidases of the liver. Epidemiologic studies indicate an increased incidence not only of liver disease, but also of cancers of the brain, lung, and possibly other organs.