Biomedical subjects
Publications and source records attributed to J G Swart.
Four cases of lipoid proteinosis (Urbach-Wiethe disease) are presented. The incidence, clinical features, relevant special investigations and possible treatment of the disease are reviewed. These patients present with hoarseness and lesions of the skin and mucosa. The condition is often misdiagnosed by general practitioners and otolaryngologists.
We report on a family with the Rapp-Hodgkin ectodermal dysplasia syndrome. Four affected family members are described and a review of the literature is given.
The morphology of the aqueductus vestibuli enjoys considerable attention as it may possibly play a role in the cause of Ménière's disease. In this study, silicone rubber casts of human temporal bones were used, and the aqueductus vestibuli was then measured by means of a reflection microscope. By this method the morphometry of the aqueductus vestibuli was determined accurately.
People who are prone to motion sickness have a directional preponderance of nystagmus to the left. Centrifuging will change this preponderance to the right in most people, and at the same time reduce their tendency towards motion sickness but only with regard to air travel.
Fifteen patients with chronic otitis media, where the organism cultured was Pseudomonas aeruginosa, had previously been treated with a variety of antibiotics, ear drops and surgical procedures. In 8 patients the discharge persisted. Between October 1986 and August 1987 these patients were treated with a combination of antibiotics (including piperacillin, ceftriaxone, amikacin and tobramycin) specific for Pseudomonas administered intravenously. In 7 patients the infection cleared completely. Follow-up ranged from 1 month to 1 year (average 4.5 months). The regimen described could be of value in patients who do not respond to standard methods of treatment.
The classic oral features of hypophosphatasia, namely, premature tooth loss and large pulp spaces, were found in a young adult woman with bone and joint pains. A study of 22 family members revealed several with dental abnormalities such as abnormal enamel, dentin, or cementum formation, decreased mandibular bone density, and abnormally large pulp spaces. Only the propositus' sister fulfilled the biochemical criteria for hypophosphatasia. Biochemical examination of an extracted tooth from this sister showed phosphate and alkaline phosphatase values that were 7 to 10 times lower than normal and reduced concentrations of the essential cofactors Zn++ and Co++. The spectrum of dental abnormalities is reviewed. This family study reveals that enamel hypoplasia, increased pulp spaces, and premature tooth loss are present not only in the deciduous but also in the permanent dentition. These findings should draw the dentist's attention to this condition.
The pupils at a school for black hearing-disabled children were clinically investigated and the findings were analysed with regard to age, sex, and nature and possible cause of deafness. A total of 169 children were examined. The majority were boys, and 95% were hearing-disabled before the age of 3 years. One-tenth of the children had a positive family history. An apparent cause could be established with a degree of certainty in only 30% of cases.
The diagnosis and radiological findings in a 48-year-old woman with paranasal sinus Aspergillus fumigatus infection is described. The patient had had chronic post-nasal discharge and sinusitis for 7 years. She was treated by total removal of all infected material.
The endolymphatic sacs of ten vervet monkeys were surgically ablated unilaterally, and the animals were assigned predetermined survival times ranging from 6 months to 5 years. The left and right ears were prepared in anatomical continuity for light microscopic study. Four experimental ears had openings in their membranous labyrinths of unknown etiology and were not considered acceptable for correlative studies. The remaining five experimental ears showed mild cochlear endolymphatic hydrops, which was localized to the apical regions or was more severe in the apical regions than elsewhere. Four ears also showed saccular endolymphatic hydrops. The sensory and neural structures in both experimental and control ears were normal, and none of the control ears showed endolymphatic hydrops. None of the animals developed severe endolymphatic hydrops or the cochleovestibular symptoms that occur in human subjects with Meniere's disease. The results of this experiment indicate that dysfunction of the endolymphatic sac may be a contributing factor, but probably is not the sole cause of Meniere's disease.
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During the 11-year period from 1974 to 1984, 222 cases of non-meningitic extrapulmonary tuberculosis in the head and neck region were seen at the ear, nose and throat departments of the academic hospitals of the University of Pretoria. A retrospective analysis revealed that three structures were mainly involved--the cervical lymph nodes, larynx and middle ear. The diagnoses were all confirmed on histological examination, revealing a positive biopsy rate for tuberculosis of 5% (222) of the total number of specimens submitted for the same period (4,357). Females had more cervical node involvement than males. Ninety per cent of all patients were black and the largest group was under 10 years old. A high index of suspicion of tuberculosis is important in the differential diagnosis of neck swellings, hoarseness and otorrhoea.
To compare the clinical results, complications and morbidity of osteotomies, secondary cleft repairs and ridge augmentations in which frozen femoral head homologous bone or autologous bone was used, a series of 61 patients with follow-up periods of 1 to 6 years has been reviewed. Long-term clinical observation shows that, with a correct indication and surgical technique, frozen homologous bone is a good alternative to autologous bone in clinical grafting procedures.
Cowden's syndrome or multiple hamartoma and neoplasia syndrome is a hereditary disorder combining multiple hamartomas of many organs. These hamartomas are derived from ectodermal, mesodermal, and endodermal tissues and may undergo spontaneous malignant changes. Small papular cutaneous lesions and papillomatous outgrowth and papular lesions of the oral mucosa are the most predominant features of the syndrome. Since the orocutaneous lesions often precede the associated malignant degenerations, they may serve as a diagnostic signal for the probably developing neoplasia in other organs. The presence of extensive papillomatous and papular lesions in the oral mucosa combined with skin lesions should therefore alert the clinician to suspect the presence of Cowden's syndrome, the early diagnosis of which is important in order that the concomitant malignant changes might be discovered at the earliest possible moment. Four cases of Cowden's disease are presented, with emphasis on the oral manifestations of this syndrome.
A five-year follow-up study of 26 patients who underwent subperiosteal onlay augmentation of the mandible using corticocancellous bone from the iliac crest is presented. An overall postoperative reduction in mandibular height of 44% was found. The different rates of resorption between the various suggested augmentation methods are discussed, and suggestions are given to prevent the rapid resorption that occurs during the immediate postoperative period.
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