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At least 1,477 records · Page 82Linked to original sources
PubMed1990

Fluoride, aluminum, and phosphate kinetics in cryolite workers.

Exposure to cryolite (Na3AlF6) dust may result in skeletal fluorosis. Eight male workers at a cryolite concentrator participated in a 4-day study after 5 days of vacation. Dust exposures were 0.16 to 21.2 mg/m3. Urine was collected before work began and during two 4-hour periods. Preshift urine fluoride concentrations increased during the week. Fluoride concentrations in postshift urine and serum both correlated with the dust exposures. Serum fluoride concentrations decreased with a half-life of 3.3 to 6.9 hours after work. Fluoride clearance was 40.5 to 76.5 mL/min at urinary flow rates of 0.89 to 2.21 mL/min. Serum aluminum concentrations varied without relation to the exposure, but the urinary aluminum excretion correlated with the fluoride levels. Preshift serum-phosphate concentrations increased significantly during the week, possibly indicating changes in mineral metabolism. For monitoring of individual uptake of cryolite dust, serum fluoride measurements are most useful.

Aluminum↗
PubMed2003

Vertebral body osteonecrosis without vertebral collapse.

STUDY DESIGN: A case of idiopathic focal vertebral body osteonecrosis without vertebral collapse is reported. OBJECTIVES: To report a rare case of focal vertebral osteonecrosis and to clarify the features of imaging studies. SUMMARY OF BACKGROUND DATA: There are no previous reports describing focal vertebral body necrosis without collapse in the literature. METHODS: A case report and literature review are presented. RESULTS: Imaging studies demonstrated: 1) sclerotic change on computed tomography scan; 2) low intensity on T1, high intensity on T2 with clear margin, and no gadolinium enhancement on magnetic resonance imaging; and 3) no uptake on bone scan. Histologic examination revealed empty lacunas, fatty necrosis with vacuolar degeneration, and cell debris compatible with bone marrow necrosis. CONCLUSION: Vertebral body osteonecrosis must be considered a possible diagnosis of patients presenting with low intensity on T1, high intensity on T2 with clear margin, and no gadolinium enhancement on magnetic resonance imaging.

Adult↗
PubMed2003

Raine syndrome: report of a family with three affected sibs and further delineation of the syndrome.

We describe three affected sibs with Raine syndrome born to a consanguineous Turkish couple. Clinical findings and post-mortem assessment are presented. We have added previously unreported meso and severe telebrachyphalangy and urogenital anomalies to the clinical spectrum. Appositional new bone formation may be mistaken for fractures and callus formation--both prenatally by ultrasound and postnatally in radiographs. Further research is required to detect the underlying metabolic and molecular defects of this autosomal recessive syndrome.

Abnormalities, Multiple↗
PubMed2004

Sclerosing bone dysplasias: neurologic assessment and management.

PURPOSE OF REVIEW: Sclerosing bone dysplasias are rare genetic disorders of bone remodeling in which excessive bone formation takes place, resulting in encroachment on neural structures. The infant usually appears normal at birth, and the first sign of a problem only comes when a neurologic deficit develops, usually in the form of an acute facial palsy. Although less than 300 cases have been published, these conditions should always be considered in the differential diagnosis of facial nerve palsy, especially in children. RECENT FINDINGS: This review highlights the neurologic presentation and assessment as well as the management of sclerosing bone dysplasias. An exciting development is the recent discovery of the sclerosteosis (SOST) gene, which is involved with excessive bone formation in sclerosteosis and Van Buchem disease. Researchers in bone metabolism and pharmaceutical companies are now utilizing this knowledge to develop a medicine for osteoporosis. SUMMARY: In children and young adults an acute facial palsy, especially if it is recurrent, as well as a conductive hearing loss may be the first sign of a sclerosing bone dysplasia.

Biopsy, Needle↗
PubMedDate not supplied

Microgeodic disease affecting the hands and feet of children.

Twenty cases of microgeodic disease affecting the hands or feet of children are presented, and the relevant literature is reviewed. Clinical features include chilblain-like appearance and tenderness of the involved digits. Radiographically patchy osteoporosis of the diaphysis and/or sclerosis of the diaphysis and rarefaction of the metaphysis with cortical erosion is evident, often accompanied by subperiosteal new bone formation. Characteristically, this condition occurs in winter and resolves spontaneously within 6 months clinically and radiographically. This lesion appears to result from vascular insufficiency of the hands and feet causing bone infarction secondary to intense vasospasm precipitated by cold.

Chilblains↗
PubMed1981

Biochemical and ultrastructural demonstration of elastin accumulation in the skin lesions of the Buschke-Ollendorff syndrome.

The Buschke-Ollendorff syndrome is an association of cutaneous lesions, dermatofibrosis lenticularis disseminata, with osteopoikilosis. This condition is inherited in an autosomal dominant pattern. In order to clarify the biochemical nature of the skin lesions, we have examined 12 patients with the Buschke-Ollendorf syndrome, representing 2 unrelated kindreds. Histologically, the lesions were characterized by excessive amounts of unusually broad, interlacing elastic fibers in the dermis. Digestion of skin secretions with pancreatic elastase completely removed these fibers. Electron microscopy of the dermis further revealed markedly branched elastic fibers without fragmentation. The accumulation of elastin in the skin was also demonstrated by measurements of desmosine employing a radioimmunoassay. The desmosine content of the skin lesions increased 3- to 7-fold when compared to the skin either from healthy controls or from uninvolved skin adjacent to a lesion. The results indicate that the skin lesions of the Buschke-Ollendorff syndrome are connective tissue nevi of the elastin type. Cell cultures from these patients may provide a convenient model to study the control mechanisms involved in elastin metabolism.

Desmosine↗
PubMed1977

Juvenile elastoma and osteopoikilosis (the Buschke--Ollendorff syndrome).

Sixteen patients from seven different families with the Buschke-Ollendorff syndrome have been studied. Osteopoikilosis was found in two-thirds of the patients radiologically examined and all but two had skin involvement. The predominant clinical pattern consisted of grouped skin coloured papules and discs that were distributed asymmetrically and which usually had presented at an early age. The skin lesions showed the characteristic histological changes of juvenile elastoma which, it is suggested, is the specific dermatological abnormality of the Buschke-Ollendorff syndrome.

Adult↗
PubMed1986

Bone lesions in chronic granulocytic leukaemia.

Radiographic abnormalities in bone are unusual during the stable phase of chronic granulocytic leukaemia (CGL). A rare situation is reported in which a patient developed three distinctive skeletal lesions simultaneously in different anatomical sites. Firstly, in both fibulae symmetrical punched out and permeative lesions were present throughout much of the shafts, being most prominent in the mid-diaphyseal regions. Secondly, the tibiae were slightly porotic and showed localized periosteal reactions, whereas in the fibulae there was extensive cloaking by a similar but much more intensive reaction. Thirdly, multiple osteosclerotic lesions were present in the pubic bones and in the proximal ends of the femora and humeri. Concurrently, fluctuant, culture negative swellings were present on the extremities. Histological examination of the material from the subcutaneous and lytic lesions showed only areas of fibrosis with islands of haematopoietic tissue, including scanty megakaryocytes. Bone marrow trephine biopsy showed the presence of myelofibrosis with islands of haematopoietic tissue typical of CGL without any evidence of blastic transformation.

Adult↗