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Enostoses, osteomas, osteopoikilosis, osteopathia striata, and melorheostosis may simulate other diseases. However, the findings on imaging usually are characteristic enough to indicate the correct diagnosis.
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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.
In Rumania, 26 cases of osteopoikilosis (20 males, 6 females) have been discovered. Five of these cases were familial (hereditary). Only 1 patient also had disseminated lenticular dermatofibrosis. All of the cases were discovered accidentally. All of the cases exibited the classic form of osteopoikilosis with stippling. Evaluation of the cases showed that various osteopoikilosis could be found in the same family and that probably the appearance of the condensation zones was related to the type of stress to which the bones are subjected (particularly pressure). Many cases show a tendency toward calcification in other tissues and not just in the bones (costal cartilage, periosteal reactions, lungs). In one case, a biopsy was made and a swelling of the trabeculae of the bone as well as cartilagenous proliferation was established.
PURPOSE OF THE STUDY: A clinical and radiological assessment was performed on 32 Freeman polyethylene uncemented acetabular cups, at an average of 9 years follow up. This study was dedicated to analyze polyethylene bone direct fixation. MATERIAL: Between 1984 and 1988, 30 patients were operated for 32 total hip prostheses. Mean age of the population at the time of surgery was 66 years. In all cases the prosthesis was a cemented Müller femoral stem with a Freeman uncemented cup. The acetabular component was made with polyethylene with three pegs fitted with an offset hemispheric cup. METHOD: All patients were reviewed at 1, 3, 5 years and maximal follow-up. Their functional evaluation was performed using Merle d'Aubigné's hip rating scale: pain score, motion score and walking score. Radiological assessment was performed to identify acetabular cup migration, bone lysis, and radio lucency. Revision for acetabular cup loosening was compiled. RESULTS: Five patients died, 27 THA were evaluated at maximum follow-up. Seven revisions were performed after the fifth years. Merle d'Aubigné's score was 14 at the time of revision. X-rays evidenced more than 5 mm migration of the cup, with massive bone lysis. Merle d'Aubigné's score was 16 for the 20 remaining THA. Radiologically, 10 cups showed migration less than 3 mm, five of which with lysis. Among the 10 cups without migration, 5 showed sclerosis near the acetabulum. The study evidenced that cases of migration exceeding 5 mm induced a revision during the next 3 years. DISCUSSION: Our study confirms failure of bone polyethylene direct fixation, with 25 per cent of revision after a five years period. This seems to be the result of micro movement, wear and bone lysis. A five years follow up is not sufficient to evaluate a component, since all revisions were performed after five years. For many authors, radiological evaluation of acetabular component migration is an important criterion for uncemented prosthesis. The EBRA computerized method is an accurate method for determining early migration, and can be used for pro and retrospective studies. CONCLUSION: Our study confirms a high rate of failures of the Freeman uncemented cup at an average of nine-year period, as previously reported in literature.
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Langerhans cell histiocytosis is classically considered as totally different from Chester-Erdheim's disease which consists in the infiltration of various parenchymas by macrophagic CD68-positive histiocytes. We report the case of a 46-year-old woman with a long history of diabetes insipidus who presented typical lesions of Langerhans cell histiocytosis on vulvar and skin biopsies as well as bony cellular infiltrates characteristic of Chester-Erdheim's disease. A few months later she presented cerebellar disorders and died after an 18-month course. At autopsy the pons was enlarged, due to numerous cellular infiltrates which were also scattered in the middle cerebellar pedoncles, dentate nuclei, midbrain and hypothalamus. There were S100-protein positive Langerhans cells intermingled with numerous ovoid CD68-positive histiocytes. There are a few reported cases of Chester-Erdheim's disease presenting foci of Langerhans cells histiocytosis in other parenchymas. In addition, there are 10 reported cases with diabetes insipidus and bilateral infiltration of the brain stem and cerebellum, considered as presenting either one type of histiocytosis or the other. Our case demonstrates that both histiocytoses may coexist in the brain and thus correspond in fact to the same pathology in certain particular cases.
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A ten-year-old boy, who had the typical dental findings of dysosteosclerosis such as yellowish, hypoplastic teeth, retarded eruption, which upon eruption, decayed rapidly, is presented. To date this is the first known case reported with a congenital absence of the first permanent molars. Furthermore, SEM evaluation of the enamel and dentin was performed on a tooth from a patient with dysosteosclerosis for the first time. These studies showed weak ultrastructural compositions due to irregular calcification.
The authors give a short account about the clinical histories of two patients: one with malignant systemic mastocytosis resulting in acute myeloid leukaemia, the other with indolent systemic mastocytosis. A brief review is reported about the physiological and pathophysiological role of mastocyte system. Benign and malignant types, classification of mastocyte proliferation are detailed, several distinct characteristics of clinical appearance, main aspects of diagnosis, therapy and prognosis in patients with different forms of systemic mastocytosis are briefly discussed.