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At least 1,117 records · Page 62Linked to original sources
PubMed1977

Ear canal hyperostosis--surfer's ear. An improved surgical technique.

The increased populatiry of surfing has produced a marked augmentation in the incidence of ear canal exostosis. However, when it becomes moderately severe, I prefer to call it "hyperostosis." Exposure to cold ocean water for many years can be an important etiologic factor in hyperostosis. There is a serious risk, and a high incidence of tympanic membrane perforations during the removal of large external canal hyperostosis. This injury can be prevented by placing a sheet of Silastic against the tympanic membrane beforehand. I describe the method. Serious degrees of hyperostosis, causing transient hearing loss and otitis externa, are increasingly common in coastal towns, where cold-water surfing is a popular year-around sport.

Adult↗
PubMed1990

A survey of otologic training in US residency programs.

Otologic cases are dwindling in numbers, jeopardizing the clinical training of our residents. A survey was performed to determine the magnitude of the problem facing our training programs. Seventy-three of 79 questionnaires distributed were returned. Results of the survey indicate that facial nerve decompression, exostosis repair, and stapedectomy were, on the average, infrequently performed by residents. Training for these seldom-performed procedures consists of temporal bone laboratory experience, close operative supervision, and the use of private patients for resident training. The majority (84%) of respondents did not feel that fellowship training was required to perform otologic surgery on graduation or that it should be required for obtaining hospital privileges in seldom-performed cases. Approximately three quarters (76%) of the program directors consider the training of seldom-performed procedures to be a moderate-to-serious problem facing otologic training today.

Exostoses↗
PubMed1976

Congenital generalized fibromatosis. An African case with gingival hypertrophy and other unusual features.

What is believed to be the first reported case of congenital generalized fibromatosis in an African infant is described. Features in our patient, which were not noted in previous reports of the disease, include gingival hypertrophy, ankylosis of joints, skeletal hyperostosis, and lymphatic dilation of the ileal villi. Corticosteroid therapy was tried in the patient, but did not produce any beneficial effect.

Ankylosis↗
PubMed1998

Genetic influence on the prevalence of torus palatinus.

Torus palatinus (TP) represents a benign anatomic variation. It has been suggested that genetic factors play a leading role in its occurrence. The purpose of the present study was to determine, by segregation analysis, the inheritance of TP. Data were collected from members of 37 randomly selected Israel Jewish families and analyzed using the segregation analysis. Vertical transmission of TP was found in 19 families suggesting autosomal dominant transmission, which was supported by the results of the segregation analysis test. A significantly higher number of affected offspring (60.3%) was observed compared to the expected figure (50%) for an autosomal dominant trait with full penetrance. This is explained by the high gene frequency of TP and the relatively high proportion of homozygous parents.

Adult↗
PubMed1997

External auditory exostosis in prehistoric Chilean populations: a test of the cold water hypothesis.

Over one thousand prehistoric crania (n = 1,149) from northern Chile were analyzed to determine if the presence of external auditory exostosis (EAE) was a type of subsistence-induced pathology, a consequence of habitual fishing in the cold water of the Pacific Ocean, rather than genetically determined. To test this occupational hypothesis, the sample was divided according to chronology, type of economy, site elevation, and sex. The crania came from 43 sites, including the coast, lowland valleys (100-2,000 m), and highlands (2,000 to 4,000 m) with a time frame of 7,000 B.C. to the Inca era (1500 A.D.). There was a significant association between EAE, environment, and sex. The coastal inhabitants had the highest prevalence of EAE with 30.7% (103/336), followed by 2.3% (6/24) for the valley people and 0% (0/549) for highlanders. Coastal and valley men were significantly more affected than their female counterparts. Contrary to expectations, there was no significant association between EAE and economy and/or chronology. In the Arica area, the early Chinchorro fishers, without agriculture, had 27.7% (26/94) EAE, the subsequent agro-pastoralists, 42.7% (32/75), and the late Arican agro-pastoral fishers had 35.6% (36/101) EAE. Apparently, with the advent of agriculture, the coastal Arican populations increased their ocean harvests, rather than decreased them, to gain a surplus in order to trade with nonmaritime groups.

Chile↗
PubMed1998

Disrupted expression of matrix genes in the growth plate of the mouse cartilage matrix deficiency (cmd) mutant.

Chondrodysplasia in the autosomal recessive cartilage matrix deficiency (cmd) mutant is caused by lack of the proteoglycan aggrecan arising from a mutation in the gene. Homozygous cmd/cmd mice are characterized by disorganisation of chondrocytes in the growth plate, disproportionate dwarfism, cleft palate, and perinatal lethality. We have studied the impact of the aggrecan deficiency on the expression of other matrix genes during the differentiation of chondrocytes in the growth plate of cmd/cmd 18.5 day fetuses. Compared with the wild-type, there are significant differences in the growth plates of cmd mutants in the combinations of co-expression of genes encoding the glycoprotein link protein, proteoglycan syndecan 3, collagens alpha 1 (X) [Col10a1], alpha 2(XI) [Col11a2], and the alternative transcripts of alpha 1 (II) [Col2a1 type IIA form], and alpha 1 (IX) [Col9a1 long and short forms]. The discordance of gene expression in cmd chondrocytes may be additional factors contributing to the disrupted cellular architecture of the growth plate resulting from the primary absence of aggrecan.

Aggrecans↗
PubMed1990

Chondrosarcoma of bone in children.

Chondrosarcoma of bone rarely occurs in children. This report analyzes the clinicopathologic features in a series of 47 patients with conventional chondrosarcoma who were less than 17 years of age. Of the 47 cases, 14 were from the Mayo Clinic files, and 33 were from our consultation files. Most of the lesions occurred in the trunk and upper ends of the long bones, with the humerus being the most frequent skeletal site. Twelve of the 47 tumors were secondary. The radiographic findings were the same as those seen in adult chondrosarcoma. Pathologically, the tumors were low grade. En bloc resection is the treatment of choice because of the high incidence of local recurrence with lesser surgical margins. Prognosis in childhood chondrosarcoma is no different from that in adult chondrosarcoma. None of the patients with follow-up data had metastasis.

Adolescent↗
PubMed2000

The mechanism of formation of bony spurs (enthesophytes) in the achilles tendon.

OBJECTIVE: To investigate the early stages in the formation of bony spurs in relation to normal enthesis development. METHODS: Histologic sections of rat Achilles tendons, stained with toluidine blue or Masson's trichrome, were examined in animals ranging from 2 weeks to 1 year of age. Further material prepared for immunohistochemistry was labeled with monoclonal antibodies for laminin and type IV collagen to highlight the presence of small blood vessels at the enthesis. Sections of small spurs from the Achilles tendons of elderly humans were also examined for comparison. RESULTS: As a part of normal development, bone grows into the Achilles tendon as the calcaneus enlarges. Ossification is preceded by vascular invasion, which occurs along rows of enthesis fibrocartilage cells. Small bony spurs develop when ossification at one point on the enthesis outstrips that on either side. CONCLUSION: Bony spurs can develop in the Achilles tendon without the need for preceding microtears or any inflammatory reaction, and they form by endochondral ossification of enthesis fibrocartilage. The increased surface area created at the tendon-bone junction may be an adaptive mechanism to ensure the integrity of the interface in response to increased mechanical loads.

Achilles Tendon↗
PubMed1982

A new form of metaphyseal chondrodysplasia in two sibs: surgical treatment of tracheobronchial malacia and scoliosis.

We describe a 19-year-old male with a previously unrecognized form of disproportionate short stature, tracheobronchial malacia, and progressive scoliosis and his 28-year-old sister with the same but milder condition. The clinical characteristics were short limbs and digits and thoracolumbar scoliosis. Bone films showed progression from marked metaphyseal dysplasia of tubular bones in childhood to short and broad bones with mild dysplasia of the joints in adulthood. The vertebrae and the intervertebral plates were only mildly affected in spite of marked scoliosis. Trachea and bronchi were reinforced with surrounding acrylate mesh before surgical treatment of the scoliosis. Affected sibs of both sexes and healthy parents suggest an autosomal recessive mode of inheritance.

Abnormalities, Multiple↗
PubMed1984

The Jansen type of metaphyseal chondrodysplasia: confirmation of dominant inheritance and review of radiographic manifestations in the newborn and adult.

We describe a mother and infant girl with the Jansen type of metaphyseal chondrodysplasia. The transmission of the syndrome from mother to daughter in this family establishes this as a dominant condition. Characteristic radiographic manifestations of the disorder were obvious at birth. The mother and infant illustrate the two extremes of age where the radiographic manifestations are less characteristic than in middle childhood, since the marked metaphyseal calcifications are absent.

Adult↗
PubMed1989

Type II collagen screening in the human chondrodysplasias.

Abnormalities of type II collagen have been considered strong candidates for causing human condrodysplasias. We have employed peptide mapping to screen for several types of type II colagen abnormalities in cartilage samples from 66 patients with 20 separate disorders. Except for achondrogenesis type II (Langer-Saldino) and spondyloepiphyseal dysplasia (SED) congenita in which abnormalities have been described and diastrophic dysplasia in which the changes were probably secondary, no abnormalities were detected. Within the limitations of the screening technique, the results combined with other data from the literature suggest that abnormalities of this molecule are not common causes of chondrodysplasias outside of the achondrogenesis type II-SED congenita family of disorders.

Adolescent↗
PubMed1991

Fibrochondrogenesis in male twins at 24 weeks gestation.

Fibrochondrogenesis is a rare lethal chondrodysplasia. Only 5 cases have been reported. We report on a pair of affected twins diagnosed at 24 weeks of gestation. Occurrence in sibs and consanguinity in the parents in a previous report support autosomal recessive transmission.

Alkaline Phosphatase↗