CONGENITAL ABSENCE OF THE INTRAHEPATIC BILE DUCTS.
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Heterotopic ossification is often a severe clinical complication of joint arthroplasty, neurologic trauma, and muscle injury. In rare genetic disorders, such as fibrodysplasia ossificans progressiva, heterotopic ossification can be crippling and often leads to premature death. Reliable animal models of heterotopic ossifications that mimic pathologies seen in man would be invaluable for the development of new treatments to combat heterotopic ossification. Various methods used to induce heterotopic ossification in animals including the use of bone morphogenetic proteins, urinary tract epithelia, and transformed cell lines are described. Genetic animal models of heterotopic ossification and various miscellaneous examples of heterotopic ossification in animals are described. Finally, the use of transgenic mice to manipulate bone morphogenetic protein expression is discussed as a possible future animal model of heterotopic ossification.
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OBJECTIVE: To assess whether lateral semicircular canal (LSCC) ossification is more advanced than that in the cochlear basal turn, in order to judge the value of the former as a predictor. METHODS: Retrospective review of 33 paediatric patients from our cochlear implant programme, with profound sensorineural hearing loss after bacterial meningitis. Magnetic resonance imaging (MRI), computed tomography (CT) scans and operative findings were reviewed. RESULTS: On CT, LSCC ossification scores were more advanced than those for the cochlear basal turn in 69.9 per cent of implanted ears. Forty-five per cent (15/33) of children had ossification at surgery. In predicting this, the sensitivity of CT LSCC ossification was 90 per cent and that of MRI LSCC ossification was 83.3 per cent. CONCLUSIONS: The more advanced ossification found in the LSCC, compared with that in the cochlear basal turn, adds to previous findings of LSCC pathology predicting cochlear ossification. Surprisingly, CT of the LSCC appears to be no less valuable than MRI in pre-operative cochlear implant assessment of post-meningitic children.
PURPOSE: Evaluation of age-related differences in the incidence, length and topographic location of ossifications in the stylohyoid chain. METHOD: Panoramic radiographs of 380 patients (718 reviewed stylohyoid chains), subdivided into 4 age groups (up to 20 years, 21 to 40 years, 41 to 60 years, older than 60 years), were reviewed and examined for the incidence, length and topographic location of stylohyoidal ossification. RESULTS: 221 (30.8%) of 718 reviewed stylohyoidal chains showed radiological variants (elongation of the stylold process and/or ossification of the stylohyoid ligament). With increasing age, there was an increase in the incidence and length of stylohyoidal ossifications (p < 0.01). A significant linear correlation between the length of the stylohyoidal ossifications and age was only found in the young age group (up to 20 years; p < 0.01). In the young age group(up to 20 years), there was also a preferred presence of isolated locations in the superior stylohyoidal segment. With increasing age, there was a pronounced presence of ossifications in the middle and inferior stylohyoid segments and combinations of ossified varibilities. CONCLUSION: Stylohyoidal ossifications show age-related differences in incidence, length and topography and can also be put down to different pathological events. Stylohyoidal ossifications gain a different importance in adult patients from that in young people.
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Lectin binding sites and immunohistochemically detectable fibronectin and carbonic anhydrase II (CA II) were examined in induced bone structure of calcifying epithelioma of Malherbe. Pathological features of ossification were particularly evident in the border zone between tumor epithelium and stroma, and epithelial foci adjacent to sites of bone formation were conspicuously basophilic. The use of lectin binding in these foci coincided with the basophilic epithelial zones: Con A, RCA-1, PNA, SBA, and WGA bound strongly, indicating the presence of glucose (Glu), mannose (Man), galactose (Gal), N-acetyl-D-galactosamine (GalNAC), and N-acetyl-D-glucosamine (GlcNAC). Fibronectin was also detected in the same epithelial-mesenchymal interacting layers as the positive lectin-binding sites. Staining for CA II was strongly positive in giant cells and in epithelial zones in the bone-inducing areas. The bond induction mechanism in the stromal tissue of calcifying epithelioma of Malherbe may initially involve the action CA II in the epithelial-mesenchymal interacting zone, which then brings about ossification of the matrix which is fibronectin-positive and rich in lectin-binding sites.
STUDY DESIGN: The present study analyzed the restriction fragment length polymorphism patterns of alpha 2(XI) collagen, bone morphogenetic protein-2, alkaline phosphatase, and tumor necrosis factor-alpha genes in patients with ossification of the posterior longitudinal ligament. This study investigates the genetic polymorphism of bone-induced factors in patients with ossification of the posterior longitudinal ligament and compares it with healthy control subjects. OBJECTIVES: To clarify the genetic markers linked to ossification of the posterior longitudinal ligament. SUMMARY OF BACKGROUND DATA: Ossification of the posterior longitudinal ligament is a genetic disease associated with abnormal calcium metabolism involving the posterior longitudinal ligament. Previous genetic studies have not identified the pathologic mechanism of ossification of the posterior longitudinal ligament. Histopathologic studies of ossification of the posterior longitudinal ligament and the animal model, the spinal hyperostotic mouse, have revealed an increase in Type XI collagen and bone morphogenetic protein-2 expression. METHODS: Eighteen Japanese patients with ossification of the posterior longitudinal ligament and 51 healthy, unrelated control subjects were investigated for the restriction fragment length polymorphism patterns of COL11A2, bone morphogenetic protein-2, alkaline phosphatase, and tumor necrosis factor-alpha, genes with various restriction endonucleases. RESULTS: The gene frequencies of COL11A2 obtained with BamHl (10.0 kb fragment) and HindIII (19.0 kb fragment) observed in patients with ossification of the posterior longitudinal ligament were higher compared with control subjects (0.43 and 0.14, respectively). These differences were statistically significant (BamHl P = 0.018; Hindlll P = 0.046). Two new restriction fragment length polymorphism patterns were detected of the bone morphogenetic protein-2 gene with Mspl and Taql and one already known restriction fragment length polymorphism pattern of the tumor necrosis factor-alpha gene with Ncol. However, they were not significantly different from the control subjects. CONCLUSIONS: Seven restriction fragment length polymorphisms of COL11A2 gene were identified. Two of them (BamHl, 10.0/10.0 kb genotype; HindIII, 19.0/19.0 kb genotype) were significantly different in patients with ossification of the posterior longitudinal ligament.
BACKGROUND: For nearly a century, the mechanical failure of calcified heart valves was attributed to a passive degenerative process. Recently, several case reports described bone formation in surgically excised heart valves and suggested an unexpected process of tissue repair. METHODS AND RESULTS: We studied the prevalence and pathology of heterotopic ossification in 347 surgically excised heart valves (256 aortic, 91 mitral) in 324 consecutive patients (182 men, 142 women; mean age 68 years) who underwent cardiac valve replacement surgery between 1994 and 1998. The valves were examined microscopically to determine the prevalence and features of bone formation and remodeling. Two hundred eighty-eight valves (83%) had dystrophic calcification. Mature lamellar bone with hematopoietic elements and active bone remodeling were present in 36 valves (13%) with dystrophic calcification. Endochondral bone formation, similar to that seen in normal fracture repair, was identified in 4 valves. Microfractures were present in 92% of all valves with ossification. Neoangiogenesis was found in all valves with ossification. Bone morphogenetic proteins 2 and 4 (BMP 2/4), potent osteogenic morphogens, were expressed by myofibroblasts and preosteoblasts in areas adjacent to B- and T-lymphocyte infiltration in valves where ossification was identified. Mast cells were present in calcified and ossified valves and were especially prominent in atheromatous regions. CONCLUSIONS: Heterotopic ossification consisting of mature lamellar bone formation and active bone remodeling is a relatively common and unexpected finding in end-stage valvular heart disease and may be associated with repair of pathological microfractures in calcified cardiac valves.
Some histological analyses of the ossification of the posterior longitudinal ligament (OPLL) have been reported, but no ultrastructural studies of the ligamentum flavum (LF) in patients with OPLL have been published to date. To understand the pathology of the ossification of the spinal ligament, we examined, by electron microscopy, ultrastructural changes in the LF in cases of OPLL and made a comparison with the LF in cervical spondylotic myelopathy (CSM). Subjects were three men and two women with cervical OPLL who underwent longitudinal spinous process-splitting laminoplasty. During surgery, a small piece of the LF was collected from C2-C3 to C7-T1 and was then analyzed by light and electron microscopy. We observed atrophic elastic bundles with a two-layer structure and disarrangement, a partially torn area, the disappearance of microfibrils, and an enlarged interstitium with an irregular alignment of collagen fibrils. We observed some properties of a cell preceding its death: the initial phase may be the disappearance of the plasma-membrane, followed by the scattering of many organellae around its degenerated nucleus. Finally, many extracellular plasma membrane-invested particles that resemble matrix vesicles remain there without phagocytosis. These results suggest that ultrastructural abnormalities exist in the spinal ligament in cases of ossification of the spinal ligament.
Calcification of pituitary adenomas as a histopathologic finding is reported in up to 25% of all degenerated adenomas. On the other hand, ossification of these adenomas is an extremely rare finding, with only one reported case, a prolactinoma. Here, we report a case of somatotropinoma in a 42-year-old acromegalic female. The adenoma, about 2 cm in diameter, was removed by the transsphenoidal route. Pathologic examination revealed ossification areas in the adenoma and the patient showed dramatic progress with relief of acromegaly signs and normalization of serum growth hormone levels. This case seems to be a unique report of osteoid metaplasia of a somatotropinoma.
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This report gives radiological and pathological evidence of bilateral ossification of the aponeurotic tendons of insertion of the pectineal muscles in a dog. This was associated with a severe grade 3 hip dysplasia of which the pathological changes are described. The ossification of the tendons is considered to have resulted from excessive and continous tension of the pectineus muscles. Various aetiological factors are discussed and some substantiation provided for the theory that pathology of the pectineal muscles and their tendons of insertion may be a contributing factor in the pathogenesis of hip dysplasia.