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At least 865 records · Page 48Linked to original sources
PubMed1980

Meningiomatous changes in the optic canan: a polytomographic study.

Eleven patients who had unilateral insidious compression of the optic nerve but exhibited no detectable abnormality on plain skull radiographs or non-tomographic views of the optic canal were studied. Paracanalicular meningioma was proved surgically in 10. Complex-motion tomography demonstrated characteristic alterations in the bone forming the optic canal in all cases. These changes consisted of canal enlargement, changes in contour, and an atypical radiographic appearance of the cortical bone, seen as (a) diffuse, spongy, non-homogeneous thickening of the canal margins; (b) multiple tiny saw-tooth-like exostoses projecting into the canal lumen; or (c) short, well-circumscribed areas of smooth bone thickening. Other techniques such as angiography and pneumoencephalography were not as helpful in the diagnosis as complex-motion tomography.

Adult↗
PubMed1981

Radiographic features of the hand in diffuse idiopathic skeletal hyperostosis (DISH): comparison with normal subjects and acromegalic patients.

Radiographs of the hands of 25 men and 9 women with diffuse idiopathic skeletal hyperostosis (DISH) were compared with age- and sex-matched controls and acromegalic patients. Characteristics of DISH included broadening and "arrowheading" of the distal phalangeal tufts, enlarged sesamoid bones, increased cortical width of tubular bones, prominent enthesopathy in the proximal phalanges, exostoses (particularly at the metacarpophalangeal heads), and new bone in the joint capsule. There was no consistent change in soft-tissue measurements or cartilage width between DISH and control patients. Acromegaly could be differentiated from DISH by the relative lack of enthesopathy, marked soft-tissue changes, and increased cartilage width. Any metabolic factor responsible for the changes in DISH apparently acts primarily on the enthesis and bone but has little effect on soft-tissue or cartilage growth.

Acromegaly↗
PubMed1984

Type IX Ehlers-Danlos syndrome. A new variant with pathognomonic radiographic features.

The authors describe 7 male patients from two unrelated families who presented with what appears to be a heretofore undiagnosed X-linked variant of Ehlers-Danlos syndrome. Unlike the eight previously reported types, this variant is manifested by specific skeletal abnormalities, including occipital exostoses, widening and bowing of multiple long bones at tendinous and ligamentous insertion sites, and deformed clavicles. Major clinical complications include genitourinary problems, chronic diarrhea with malabsorption, and/or syncopal episodes. Laboratory tests suggest that this variant may represent the true lysyl-oxidase-deficient form of Ehlers-Danlos syndrome.

Adolescent↗
PubMed2004

Effect of C-reactive protein on gene expression in vascular endothelial cells.

C-reactive protein (CRP) is significantly associated with the risk of ischemic cardiovascular disease in epidemiological studies. To explore if CRP has a functional role, we investigated its effect on the gene expression profile of vascular endothelial cells. Human vascular endothelial cells (human umbilical vein endothelial cells and human aortic endothelial cells) were incubated with CRP at various concentrations (0-10 mug/ml). Microarray analysis showed that a total of 11 genes increased (IL-8, core promoter element binding protein, activin A, monocyte chemoattractant protein 1, Exostoses 1, Cbp/p300-interacting transactivator with Glu/Asp-rich COOH-terminal domain 2, plasminogen activator inhibitor 1, fibronectin-1, gravin, connexin43, and sortilin-related receptor-1) and 6 genes decreased (methionine adenosyltransferase 2A, tryptophan-rich basic protein, reticulocalbin 1, membrane-associated RING-CH protein VI, cytoplasmic dynein1, and annexin A(1)) by more than twofold for their mRNA levels. IL-8 was the most significantly upregulated gene (13.6-fold), which demonstrated a clear dose- and time-dependent pattern revealed by quantitative real-time PCR. Cell adhesion assay showed that CRP enhanced the monocyte adhesion to endothelial cell monolayer by 2-fold (P < 0.01), which was partially blocked by an anti-IL-8 antibody (34.2% inhibition, P < 0.01). Inhibition of ERK MAPK pathway using U0126 prevented CRP-induced IL-8 upregulation, and Western blot analysis revealed a rapid activation of ERK1/2 after CRP stimulation. These data showed that CRP can significantly influence gene expressions in vascular endothelium. The CRP-responsive genes suggested that CRP may have a broad functional role in cell growth and differentiation, vascular remodeling and solid tumor development.

Aorta↗
PubMedDate not supplied

Role of transtympanic endoscopy of the middle ear in the diagnosis of perilymphatic fistula in patients with sensorineural hearing loss or vertigo.

BACKGROUND: The diagnosis of perilymphatic fistula (PLF) is often difficult, and therefore the condition can be overlooked. Tympanoscopy presents an alternative procedure for visualising the middle ear anatomy, and it may help to diagnose PLF. AIM: The aim of this study was to evaluate the use of middle ear endoscopy in establishing the diagnosis of PLF and in defining its incidence in patients with sensorineural hearing loss and/or vertigo and tinnitus. SUBJECTS AND METHODS: Two hundred and sixty-five patients (22-80 years of age, mean 48 years) were prospectively and consecutively referred for middle ear examination with tympanoscopy. Tympanoscopy was performed using endoscopes with visual angles of 5 and 25 degrees and an outer diameter of 1.7 mm. The round window niche (with its secondary membrane), the oval window with a stapes superstructure, a part of the facial recess and the area in the fissula ante fenestram were examined and video-recorded. RESULTS: For 1 patient, tympanoscopy revealed fistula in the round window membrane that was covered with a fibrinous layer. In 4 cases abnormal mucosal shining appeared in the round window, but no PLF was present. In 7 cases the tympanic cavity could not be visualised because of the adhesive tympanic membrane, abnormal anatomy or the prominent exostoses of the external ear canal. In 6 cases a postendoscopic middle ear infection was found. No permanent tympanic membrane perforation occurred in any of the patients in this study. CONCLUSIONS: Tympanoscopy is a rapid examination tool with which to verify certain areas of the middle ear anatomy, but it is of limited value for ruling out the presence of PLF.

Adult↗
PubMedDate not supplied

Osteochondroma of the sacrum with a correlative radiographic and histological evaluation.

Primary benign sacral tumors are rare representing only 1-4% of solitary exostoses and constituting 4% of all solitary spinal tumor. The tumor usually comes to attention in childhood or adolescence. The treatment of symptomatic lesions is resection. We present here a case of a sacral osteochondroma with characteristic histological and radiological features in an adolescent presenting with radicular pain.

Adolescent↗
PubMed1994

Oxalosis associated with aluminum bone disease: a new type of mixed renal osteodystrophy.

An 18-year-old man suffering from uremia was hospitalized because of severe skeletal pain and multiple exostoses. The patient had been treated for 5 years by regular hemodialysis; primary oxalosis was the suspected cause of uremia. Transiliac bone biopsy and subsequent histomorphometric analysis of undecalcified bone specimen as well as specific aluminum staining revealed a picture of oxalosis combined with osteomalacia and a marked accumulation of aluminum at the front of mineralization. This combination of histological features of oxalosis and aluminum bone disease suggests a new type of mixed renal osteodystrophy.

Adolescent↗
PubMed1996

Trichorhinophalangeal syndrome type III.

Trichorhinophalangeal syndrome (TRPS) type III is a newly defined clinical entity. This symptom complex is inherited as an autosomal dominant trait and clinically characterized by growth retardation, craniofacial abnormalities, severe brachydactyly and sparse hair. In addition, absence of mental retardation and cartilaginous exostoses are required for the diagnosis of TRPS III. To further delineate this newly recognized entity, we report on a patient from a Turkish family segregating TRPS III in 7 family members. The patient had a very short stature (147 cm, < 3rd standard deviation), a thin upper lip and a prominent lower lip, a pear-shaped nose, stubby fingers and toes with cone-shaped epiphyses and sparse scalp hair. Scanning electron microscopy findings and results of energy-dispersive X-ray microanalysis are presented in such a patient for the first time.

Abnormalities, Multiple↗
PubMed1982

Abnormal copper metabolism and deficient lysyl oxidase activity in a heritable connective tissue disorder.

Biochemical abnormalities were studied in two brothers with bladder divericulas, inguinal hernias, slight skin laxity, and hyperelasticity and skeletal abnormalities including occipital exostoses. Lysyl oxidase activity was low in the medium of cultured skin fibroblasts, this abnormality being accompanied by reduced conversion of the newly synthesized collagen into the soluble form. Copper concentrations were markedly elevated in the cultured skin fibroblasts, but decreased in the serum and hair. Serum cerulophasmin levels were also low. The reduced lysyl oxidase activity is suggested to be responsible for ther clinical manifestations, but the deficiency in this copper-dependent enzyme may be secondary to the abnormalities in the metabolism of the cation. Nevertheless, a mutation directly affecting both lysyl oxidase and an intracellular copper transport protein cannot be excluded. The disease is tentatively classified as one subtype of the Ehlers-Danlos syndrome.

Adult↗
PubMedDate not supplied

Otolaryngic osteopathology in 14th century mid-america. The crow creek massacre.

Over 486 people were massacred and buried communally at the Crow Creek Village Site, South Dakota, about 1350 AD. Osseous pathology in the craniofacial area of the victims' skeletons is reviewed and compared to data from other indigenous skeletal populations past and present. The Crow Creek mastoid x-rays are analyzed and compared to four other studies which span over 1000 years of Dakota Territory history. Findings regarding external auditory canal exostoses in the different groups are compared. Unusual findings in the craniofacial area are assessed. Insight into longitudinal epidemiology in one portion of the U.S. is possible through this study.

Adolescent↗
PubMed1993

Osteoma of the internal auditory canal.

The use of MRI for the evaluation of lesions in the internal auditory canal presents a potential pitfall in the diagnosis of bony lesions of the IAC, because bone is poorly visualized with this method of imaging. The presence of marrow in an osteoma might aid in its detection, since fat in the marrow has a bright signal intensity of T1-weighted imaging. Computed tomography remains the imaging modality of choice for bony lesions of the temporal bone. We demonstrate a case of IAC osteoma in which surgical removal resulted in improvement of symptoms. The gross and microscopic appearance of the IAC osteoma in this case is similar to the characteristic findings of osteomas of the EAC. This suggests that the criteria applied to osteomas and exostoses of the EAC may also be used to differentiate bony lesions of the IAC.

Diagnosis, Differential↗
PubMed1996

Subungual exostosis of the foot.

Eleven subungual exostoses of the foot (10 on the hallux, 1 on the third toe) were studied. The initial symptom was subungual pain. When a subungual mass of fibrous tissue appeared, the nail was pushed up and in one case the mass became infected. X-rays exhibited a bone mass protruding from the terminal phalanx on the dorsomedial aspect of the toe in all cases. All patients underwent surgical excision of the lesions with partial onychectomy. Three layers were identified in five cases: a cap of fibrous tissue, a middle zone of hyaline cartilage with enchondral ossification, and a deep zone of cancellous bone. In three other cases, the histological pattern was pleomorphic and poorly characterized. The study shows that most subungual bone masses exhibited the pathological features of conventional osteochondromas. Nonetheless, a small number of lesions were pleomorphic and differed from osteochondromas, with abundant fibrous tissue merging irregularly into scattered islets of cartilage that was not organized in columns. Radical excision of the mass achieved complete relief of symptoms and recovery without recurrences in all cases.

Adolescent↗
PubMed1997

Functional evaluation of the 10-year outcome after modified Evans repair for chronic ankle instability.

The Evans tenodesis is an operative treatment for chronic ankle instability with good short-term results. The disadvantage of impaired hind foot kinematics and restricted motion has been described, and only few reports of long-term results can be found. No techniques have been used to assess the outcome objectively. We wanted to determine whether a modified Evans procedure led to a satisfactory clinical and functional outcome. Nineteen patients were available at a 10-year follow-up. The clinical examination included a detailed questionnaire and stress radiographs. Foot function was evaluated with plantar pressure distribution measurements during walking and peroneal reaction time measurements elicited on a rapidly tilting platform (recorded with surface electromyography). High subjective patient satisfaction was contrasted with a high rate of residual instability, pain, and swelling. The radiographs showed an increased number of exostoses. The gait analysis revealed reduced peak pressures under the lateral heel and increased values under the longitudinal arch. The reaction times of the peroneal muscles were shorter on the operated side (significant: peroneus longus). The persistent clinical problems as well as the functional changes indicate that the disturbed ankle joint kinematics permanently alter foot function and may subsequently support the development of arthrosis. Therefore, the Evans procedure should only be applied if anatomical reconstruction of the lateral ankle ligaments is not feasible.

Adult↗
PubMed2003

Developmental pathways in musculoskeletal neoplasia: involvement of the Indian Hedgehog-parathyroid hormone-related protein pathway.

There are many crucial genes and signaling pathways in the proper development of an organism. Pathologies may arise from a deregulation of these pathways. The Indian Hedgehog-PTH-related protein (Ihh-PTHrP) pathway is vital in the proper development of endochondral bones, such as the long bones. The Ihh-PTHrP pathway regulates the rate at which chondrocytes within the growth plate proliferate and differentiate. Thus, this pathway allows for the longitudinal growth of bones. However, a disruption in this pathway may lead to enchondromas and osteochondromas, which are both childhood cartilaginous neoplasms. Recently, our lab identified a mutant receptor for PTHrP in enchondroma samples. Mice expressing this mutant receptor and mice with increased Ihh activity develop conditions similar to human enchondromatosis. Linkage analysis shows an association between EXT genes and osteochondromas in hereditary multiple exostoses syndrome. Studies in Drosophila and mice suggest EXT gene products play a role in the diffusion of hedgehog proteins. A mutation in EXT genes may result in an abnormal Ihh diffusion pattern leading to an osteochondroma. There are agents that inhibit Hedgehog signaling. These agents may be useful in the treatment of enchondromas and osteochondromas. This review will discuss the discovery of the Ihh-PTHrP pathway and its involvement in neoplasia, and will suggest possible novel therapeutic agents in the treatment of these cartilaginous neoplasms.

Bone Development↗
PubMed2001

Canalplasty: review of 100 cases.

Canalplasty is the surgical procedure whereby the external auditory meatus is widened. The indications include exostoses, stenosing external otitis and widening for surgical access. One hundred consecutive ears operated on by one surgeon are reported. The surgical technique is described in detail, paying particular attention to bone removal from the anterior canal wall. In this paper the majority of cases were occasioned by soft tissue rather than bony stenosis. The re-stenosis rate was four per cent and in each case this was associated with the use of a middle temporal artery flap. Partial, transient, delayed facial palsy occurred in two per cent, probably relating to thermal injury transmitted from the burr. A full, spontaneous recovery of facial function occurred in each case. This is a safe and effective technique for canal widening.

Ear Canal↗
PubMed1986

Effect of life in industrialized societies on hearing in natives of Easter Island.

In an effort to determine whether life in industrialized societies can have an effect on hearing, 90 natives from Easter Island over 45 years old were evaluated. They underwent complete clinical and audiological assessment and were divided into groups according to those having lived only on the island or those having lived in modern civilization. With all factors being equal, except exposure to modern civilization, our results showed that living in civilized societies has a significant negative effect on hearing; the severity is directly proportional to the years of exposure. The median hearing thresholds of natives always living on the island (men and women combined) was found to be similar to those of female citizens of the United States; there was no significant difference in hearing thresholds between men and women among these natives. These results suggest that there are no significant inherent racial differences nor significant inherent differences between males and females in the sensitivity of hearing. A relationship between aging and hearing was noted and interpreted as true or intrinsic presbycusis. There were no cases of otosclerosis, Meniere's disease, or exostoses of the external ear canal among natives who had always lived on the island. Pure natives were found not to be prone to otitis media in spite of having an extremely high incidence of severe upper respiratory tract allergies.

Aged↗
PubMed1999

Osteochondroma with compression of the spinal cord. A report of two cases.

We report two cases of vertebral osteochondroma. In one patient a solitary cervical lesion presented as entrapment neuropathy of the ulnar nerve and in the other as a thoracic tumour associated with hereditary multiple exostoses producing paraplegia. We highlight the importance of an adequate preoperative evaluation in such patients.

Adolescent↗
PubMed2005

Ulnar focal cortical indentation: a previously unrecognised form of ulnar dysplasia.

Deformity of the forearm due to growth disturbance of the ulna occurs in a number of conditions such as ulnar deficiency, multiple exostoses, and neurofibromatosis. We report a previously unrecognised form, caused by focal cortical indentation. We have treated five children with this condition, three girls and two boys; the mean age at presentation was 5 years (2 to 8). The deformity was first recognised about the age of two years, and progressed gradually. The radiological findings were the same in all cases. The focal cortical indentation was seen at the distal end of the ulna with anteromedial bowing and dysplasia. The radial head was dislocated posterolaterally. In one patient the histological findings at the site of indentation were of a fold of tissue resembling periosteum, which interfered with enchondral ossification. Treatment by ulnar lengthening using an external fixator and osteotomy which corrected both the ulnar deformity and reduced the dislocated radial head in two cases gave the best results.

Bone Diseases, Developmental↗