[Precocious puberty during chronic adrenal insufficiency. Another example of complex drag-effect endocrinopathy?].
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Biomedical subjects
Publications and source records attributed to M Forest.
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This paper focuses on the development of a portal in the World Wide Web (WWW), which captures and locates quality information for patients with chronic kidney disease (CKD). It examines the problems patients face when accessing and understanding information gleaned from Web sites and describes an idea from a Research Board Member to facilitate patient access to quality information. The idea germinated into the development of a patient specific Web site, providing one stop access and links to appropriate CKD information, assessed by patients and health professionals. Collaboration between the EDTNA/ERCA Research Board and CEAPIR the European Federation of Kidney Patients has enhanced the project.
Concerning these six cases, the authors recall the rarity of osseous localization of Hydatidiform disease, isolated as a rule, arising in the adult and preferentially involving the ilium, spinal column, femur, humerus and scapula. Being slowly progressive, the condition is revealed by pain, swelling and above all spontaneous fractures (long bones), or signs of root or medullary compression (spinal column), followed sometimes by fistula formation. Radiological diagnosis is often difficult with an osteolytic appearance without appreciable reactional osteogenesis. As for laboratory investigations, they are only of value in orientation; immunofluorescence being the weakest. The extent of osseous lysis frequently demands extensive surgery. From the anatomical standpoint, the hydatid variety of Echinococcosis has a specific intra-osseous development: beginning in the spongy bone, the architecture is intermediary between the hydatid appearance and the alveolar variety. Some cases however comprise a unilocular appearance with a suspicion of adventitial reaction. Extension into the soft parts frequently adopts a macroscopic appearance identical with that of visceral forms. Bony lysis is dependant on osteoclastic resorption or foci of necrosis while reactional osteogenesis is reduced. Medullary cellular reactions are determinant in the anatomical progress: osteitis or histiocyte and plasmodial reaction to the cuticle fragments.
Bone formation by the sarcoma is exclusive, without any cartilaginous component; the pattern of the tumour being sometimes quite similar to dysplasia lesional tissue. It seems to be a tissular transformation rather than an unicellular sarcomatous change. Among the etiological factors, except the part of the radiotherapy, periostal changes enhanced by a surgical procedure with acrylic cement in the femoral shaft are perhaps contributing to the development of a sarcoma whose pattern is quite particular.
The morphological study of Giant Cell Tumors of Bone collected at the Orthopaedic Department of C.H.U. Cochin, with high resolution radiography of macroscopical samples added to topographical cytology allows to report the following facts: --Mononuclear cells have cytonuclear criteria between histiocyte and fibroblast cells, quite apart from Chondroblastoma and Chondromyxoid fibroma. Cytological transitions are observed between mononuclear and giant cells. --Osteoid formation is usually connected with the lysis or the blow-up of the neighbouring cortex. Without secondary changes, collagenisation is reduced. --A vascular network is appearing between the cortex and the tumor, factor of some radiological evolutions, those aspects being nearly similar to aneurysmal bone cyst. --The grading of the tumour has no valuable architectural support ; mitotic activity is equally of no value for the assesment of a recurrence. Nuclear analysis with granular clumping of chromatin, thickening of the nuclear rim and increase in size and number of nucleoli was only a help to identify sarcomas. The latter are primary-associated with quiescent-appearing lesions or merging on multiple tumoral sampling. Giant cell sarcoma, except fibroblastic-like forms, can keep a differenciated cytology with frequent amitosis. --Tumor polymorphism is warrant of some histological pitfalls ; the most rewarding territories for the analysis are in the long bones the upper epiphyseal areas. Whereas, the frequency of secondary changes enhance the additional value of topographical inprint cytology for a correct morphological appraisal.
2 giant cells tumors were examined under electron microscope. The study confirmed that basic cellular population was mononuclear cells which demonstrated a tendancy toward phagocytosis and fibrillar material production. These cells had some appearance of macrophages or fibroblasts.
The authors have treated nineteen cases of desmoid fibroma of the limbs of which two were intraosseous. The severity of the lesion was greater when sited proximally in the limbs. In many patients, the tumours grew slowly and gave little incapacity. A large number of recurrences were found after surgical excision. The treatment, however, must be based on surgical excision which should be as extensive as possible. Radiotherapy should only be used after the tumour has recurred several times. In view of reports of spontaneous healing, hormone therapy was tried but no conclusions could be drawn from these trials.
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The authors report the pathological and clinical findings in five cases of chondrosarcoma resembling chondroblastic chondrosarcomas as described by Lichtenstein and Bernstein in 1959 and, above all, clear cell chondrosarcomas as described by Unni et al. (1976). These chondrosarcomas have a double peculiarity:--topographic, for their site is often epiphyseal :--anatomica, for they associate, in the usual chondrosarcomatous sectors, areas rich in clear cells, with a rich blood supply, and in some cases a diffuse reactional osteogenesis. Although the long term prognosis is similar to that of all chondrosarcomas, their course is sometimes very slow. One should thus distinguish carefully these tumours from chondroblastomas in spite of certain topographic and radiological similarities.
Quantitative bone histomorphometry and evaluation of blood parameters have been performed in 24 patients with hepatic cirrhosis. 13 patients show osteoporosis which, in 8 of them, is associated with osteoclastic hyperactivity but without elevation of blood parathormon. All patients have hypocalcemia and 14 of them hyperosteidosis. These results are compared with data of the literature on bone morphometry and phosphocalcic metabolism during hepatic cirrhosis.
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