Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0851184 (thinning)
 11,252 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A new clinico-pathologic entity is described. It is defined as osteofibrous dysplasia of long bones, and is based on twenty two personal observations to which are added seventeen cases from the literature. This dysplasic congenital lesion is clearly differentiated from fibrous dysplasia by clinical, radiographic and histological characteristics and by its clinical course. These features may be summarised as follows: 1) Slight predominance of the male sex. 2) Very early age of onset either at birth or in the first years of life. 3) Site almost exclusively tibial, sometimes also in the fibula. Localisation predominantly in the middle third of the tibial diaphysis, but sometimes in the distal or proximal third. In the fibula, it is always at the distal third. 4) The lesion is painless and generally causes bony enlargement. There is often slight anterior bowling and more rarely, slight varus of valgus bowing. Pathological fracture may occur; rarely there is a pseudarthrosis. 5) The radiographic appearances are very characteristic, with enlargement of the bone, intracortical osteolytic lesions with thinning or disappearance of the external cortex, sclerotic reaction on the medullary aspect, and narrowing of the medullary canal. 6) The histological features are also typical, consisting of fibrous tissue enclosing bone trabeculae lined by osteoblasts and a "zonal" architectural pattern. 7) Sometimes the lesion tends to heal spontaneously in the very early years of life; in other cases it is moderatley progressive. It relapses frequently after curettage, but such recurrences are generally non-progressive. In some cases slight anterior bowing persists permanently. 8) Surgery should be restricted to patients over the age of five in whom the lesion is extensive, with imminent or actual pathological fracture, and to the rare cases of pseudarthrosis. The results are good even in cases of relapse or pseudarthrosis. The correction of residual bowing, if indicated, can safely be carried out with one or more osteotomies at the age of ten to twelve years.
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PMID:Osteofibrous dysplasia of long bones a new clinical entity. 102 9

A patient suffering from thalassaemia, with extreme osteoporosis, coarse trabeculation and cortical thinning of the bones, developed a pathological fracture of the left hip. This was treated by a single dose of radiotherapy. It is suggested that the radiotherapy facilitated the healing process by eliminating the causative factor of the fracture, which was the expanding and over-proliferating bone marrow.
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PMID:Pathological fracture in haemoglobinopathy: treatment by irradiation. 842 18

The radiographic features of hydatid disease of bone have been reviewed in 16 Kuwaiti patients. The majority of the lesions were in the lower limb or pelvis, and the commonest presentation was with a pathological fracture. The radiological signs seen most often were lucent lesions in the bone, associated with expansion of the bone and thinning of the cortex. In patients with these signs soft tissue calcification appeared to be highly suggestive of hydatid disease. The disease was diagnosed before operation in only half the patients. In areas where the disease is endemic hydatid disease should be suspected when the lesions described are seen on plain films.
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PMID:The value of plain film findings in hydatid disease of bone. 849 74

Enchondroma is a benign growth of cartilage arising in the bone metaphysis as a solitary or multiple primary lesions. The form of multiple enchondromatosis with unilateral predominance is termed Ollier's disease. We have recently treated a case of Ollier's disease with the chief complaint of deformity of the left hand. The patient was an 11-year-old boy. Radiographic examination showed honeycombed clear spaces in the metaphyses of the middle and proximal phalanges of the left ring and little fingers as well as of the fourth and fifth metacarpals, and thinning of the cortex of these bones, but with no evidence of pathological fracture. The tumors of the left fourth and fifth metacarpal bones and of the phalanges of the left ring and little fingers were removed, and the metacarpophalangeal joint of the little finger was capsulotomized. The patient was free from recurrence 19 months after surgery. With regard to the prognosis of Ollier's disease, malignant transformation into chondrosarcoma or osteosarcoma has been reported of the chondroma. Since Ollier's disease is self-limited in that it usually stops spontaneously as the patient grows, and since the cartilaginous lesions in occasional cases may regress or even disappear, any cartilaginous lesions that are still active or painful after termination of the growth period should be examined thoroughly under suspicion of undergoing malignant transformation.
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PMID:A case of Ollier's disease of the hand. 901 45

Four cases of proved hemophiliac pseudotumors caused by intraosseous bleeding are reported. Five lesions were found at the uncommon locations involving the cranial vault, mandible, phalanx, distal femur and distal tibia. The conventional radiographic and computed tomographic findings are expansile osteolytic destruction, cortical thinning, partial breaking cortex or pathological fracture, and sometimes associated soft tissue mass. Ultrasonographic feature of one case at the phalanx shows cortical expansion and thinning contained mixed echogenicity in the medullary canal with soft tissue extension. T99m DTPA of one case at the distal femur shows increased vascular flow and uptake at right distal tibia and left distal femur.
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PMID:Hemophiliacs bone pseudotumors. 1008 44

The authors report a case of pathologic fracture of the distal tibia associated with Charcot-Marie-Tooth disease. Pathologic fracture was visible four weeks after initial pain. Treatment consisted in a short leg walking cast for six weeks. Charcot-Marie-Tooth disease is a slowly progressive neurogenic muscular atrophy affecting the distal parts of the lower limbs. The muscular atrophy is responsible for radiographic bony changes including narrowing of the shaft with thinning of the cortex, rarefaction at the end of the long bones and relative widening of the medullary cavity. Pathologic fractures in neuromuscular disease are rare; a few cases have been reported following application of very small forces. The authors draw attention to the increased risk of pathologic fractures in patients with neuromuscular disease. Ambulatory treatment of fractures should be used whenever possible; prolonged immobilization could result in further loss of function.
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PMID:[Pathologic fracture of the tibia associated with Charcot-Marie-Tooth disease]. 1021 11

Angiosarcoma associated with fibrous dysplasia is very rare. We have recently experienced two cases of angiosarcoma that secondarily arose from fibrous dysplasia. The first patient, a 55 year-old man, had noticed a deformity in the right upper arm since he was five years old. At the age of 25 years, polyostotic fibrous dysplasia was diagnosed by X-ray examination. The patient complained of swelling and pain around the left shoulder. The diaphysis of the humerus was mostly non-observable due to severe bone destruction by tumor invasion and there was a large soft tissue tumor. Biopsy examination revealed grade II or III hemangioendothelioma with typical histologic findings of fibrous dysplasia. After interscapulothoracic amputation for wide tumor resection, he died of DIC. Autopsy revealed multiple liver metastatic lesions of angiosarcoma. The second patient was a 66-year-old woman. She presented with a pathological fracture in the right tibia, due to an osteolytic lesion with cortical bone swelling and thinning. The histologic diagnosis was fibrous dysplasia without any sarcomatous changes. However, after a 3-time recurrence, angiosarcoma arose from the same lesion. She is now still alive with disease-free period of 6.5 years after amputation.
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PMID:Two cases of secondary angiosarcoma arising from fibrous dysplasia. 1065 Jul 91

Unicameral, or solitary, bone cysts are unusual tumors seen in the ends of long bones in skeletally immature persons. The etiology of these lesions is poorly understood. Various hypotheses have included dysplastic processes, synovial cysts, and abnormalities in the local circulation. Most patients present with a nondisplaced pathologic fracture, but occasionally cysts are found incidentally. Plain radiographs typically show a symmetric lesion with cortical thinning and expansion of the cortical boundaries. Once diagnosed, unicameral bone cysts continue to be a treatment dilemma. Traditional methods, such as prednisolone therapy, usually involve multiple anesthetics and injections and are associated with high recurrence rates. Major surgical procedures, such as wide exposure, curettage, and bone grafting, may be somewhat more effective, but still carry with them significant morbidity and recurrence rates. Newer techniques involving percutaneous grafting with allograft or bone substitutes or a combination of the two are promising in light of their low complication rate and lower reoperation rate.
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PMID:Unicameral bone cysts. 1095 Nov 10

The benign fibrous histiocytoma is a rare tumor with only a few descriptions. In order to better define the optimal therapeutic procedure and the necessity of surgery we retrospectively analyzed the patients of the orthopedic department. Benign fibrous histiocytoma occurred in the femur (n = 3), pelvis (n = 2), humerus, tibia, fibula, rib and spine. Pain as the cardinal symptom and a median age of 28 years are factors that differentiate benign fibrous histiocytoma from other metaphyseal fibrous lesions such as the nonossifying fibroma. Radiographic investigation of this entity showed osteolytic lesions with eccentric thinning of the cortex and small fissures. To a variable extent, sclerosis was found in the margin of the lesions. Computer tomography revealed dense soft tissue in the lesions, but one lesion was filled with fluid. The tumor was restricted to bone, with no periosteal or soft tissue reaction. Magnetic resonance imaging showed enhancement of the tumor after administration of contrast medium. All lesions had uniform histological findings. Although no pathologic fracture occurred in any case, local expansion of the tumor was indicated by cortical thinning, small fissures and pain. This implied that the patients needed prophylactic curettage and bone grafting. Surgery restricted to the osteolytic area was sufficient to prevent recurrence.
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PMID:Benign fibrous histiocytoma of bone: a report of ten cases and review of literature. 1240 38

Osseous hydatid disease is caused by the parasitic tapeworm Echinococcus. The species most responsible for hydatid disease is Echinococcus granulosus, endemic especially in sheep-rearing districts like Mediterranean countries and Australia. In Turkey, the exact incidence of human hydatid disease is not known, but < or =34% of asymptomatic farmers have positive serology. Bone hydatidosis is rare, making up 0.5% to 4% of all cases. Patients usually present with pain, swelling, or pathological fracture. There are no specific radiographic signs in affected bone. In the later stages, lytic lesions with a trabeculated pattern, with or without sclerosis, may be seen. Computed tomography (CT) is still the best method for diagnosis and posttherapy follow-up of osseous hydatidosis. On CT, skeletal cystic hydatidosis appears as one or several closely related, well-defined, osteolytic lesions. There may be bone expansion, cortical thinning, cortical destruction, sclerosis, honeycomb appearance, and extension into adjacent soft tissues.
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PMID:Hydatid bone disease of the femur. 1929 69


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