Biomedical subjects
Publications and source records attributed to I Brama.
We present a case of liposarcoma of the nasopharynx in a 28-year-old woman. The tumor was incompletely resected through an intraoral approach and supplementary radiotherapy of 50 Gy in a restricted field was therefore administered. Eleven years of follow-up show the patient to be disease free.
Neoplastic transformation has been associated with a variety of structural changes, among which are changes in membrane carbohydrates. Not much is known, though, e.g., how early in the tumourogenic event these changes take place and what effect these changes have on cell growth, invasion, and ability to metastasize. We were able to identify the B-D-Gal(1-3)DGal-NAc as a membrane carbohydrate component present in malignant laryngeal tissue, but not on adjacent normal mucous membrane. This carbohydrate structure was found to be present in metastatic as well as in nonmetastatic tumours. It was also found in well-differentiated as well as poorly differentiated carcinomas. We suggest that changes in carbohydrate components on the cell membrane of the laryngeal cancer cell are an early event in tumour progression and probably are not related to the degree of invasion or the ability to metastasize.
From 1964 to 1984, 25 children with malignant tumors of the nasopharynx were seen, and their progress was followed at The Hospital for Sick Children in Toronto. Two types of malignancies: rhabdomyosarcoma and lymphoepithelioma were most prevalent with eight cases apiece. The presenting signs and symptoms were related to local and/or regional manifestations of disease. No child presented with signs or symptoms related to distant metastatic disease. The diagnosis and treatment of this series of patients are described briefly. The advent of combined treatment modalities in the past decade has improved the prognosis for nasopharyngeal tumors, especially for the rhabdomyosarcomas; in general, however, survival rates are still poor, approximating 50%.
Obstructive sleep apnea (OSA) was diagnosed in 14 infants less than 18 months of age. Snoring, apnea, failure to thrive, developmental delay and recurrent respiratory infections were the main presenting symptoms. The diagnosis was made by polysomnographic studies or overnight monitoring. Adenotonsillectomy resulted in the relief of symptoms and signs in 13 children. In one infant prolonged nasopharyngeal intubation was needed. An increased awareness of OSA in young infants may prevent the delay in diagnosis, will allow early treatment and thus prevent the development of sequela or complications associated with this syndrome.
Patients with Hunter syndrome may have symptoms of hoarseness, stridor and breathing difficulties as a result of laryngeal and tracheal involvement. In planning their evaluation, we must prefer non-invasive methods as X-ray or CT scan, and avoid doing endotracheal intubation or bronchoscopies. Review of adult cases in the literature and description of the only case of a child with Hunter syndrome having life-threatening complications of his upper airways is discussed in this report. In this case and in the literature we cannot exclude intubation or bronchoscopy as a serious aggravating factor, causing further narrowing of the larynx.
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The association between malignant transformation and the loss of ABO(H) blood group antigens was documented by several authors. Most of the work was done on paraffin sections, though a small portion was performed on fresh frozen tissues. We suggest that the specific red cell adherance (SRCA) test can be applied to tissues cultivated in tissue culture for determination of malignant transformation. This study supports this assumption.
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Laryngeal foreign bodies (F.B.) in children are relative rare, especially in infants under one year of age, and the diagnosis and removal are difficult. The history, the clinical and radiologic findings can be misleading. Seventeen patients with laryngeal F.B. (10 of them under one year of age) are presented, and the diagnostic problems and treatment are discussed. Foreign bodies were either objects of a sharp and thin quality, e.g. an eggshell fragment, or large, e.g. a piece of meat, causing apnea and death.
A skin nodule on the external nose, as a presenting feature of acute myeloid leukemia, is described. The child presented with a lesion on the tip of the nose together with the clinical and haematologic features of leukaemia. The histological finding of the nodule was found to be a characteristic leukaemia cutis with leukaemia cell infiltrate in the skin. Epistaxis and external ear infection were also additional features of E.N.T. system involvement, as we find in other leukaemic patients. Since starting chemotherapy the patient has been well, in complete remission, and the lesion on the nose has almost disappeared.
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Ten of 16 patients with Behçet's disease complained of hearing disturbances (62%), and six of them had vertigo (37%). Detailed audiologic and vestibular examinations revealed both cochlear and vestibular abnormalities in those patients. The labyrinth involvement may be ascribed to vasculitis, which is the basic pathologic lesion in Behçet's disease. This inner ear involvement is a late complication of this disease, appearing almost a decade after the initial manifestations.
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