Journal article
Cystic Fibrosis—An Otolaryngologic Perspective
Otolaryngology--head and neck surgery, Vol.97(4), pp.356-360
10/1987
DOI: 10.1177/019459988709700403
PMID: 3120101
Abstract
Cystic fibrosis (CF) is the most common lethal genetic disorder in white patients. The protean manifestations of the disease result from exocrine gland dysfunction and include chronically debilitating pulmonary and pancreatic compromise and clinically inconsequential (although diagnostically extremely important) sweat electrolyte abnormalities. The subject of this article is the otolaryngologic manifestations of the disease, based on a retrospective analysis of 450 cases. Nasal polyposis and sinusitis occurred in 10% and 11% of patients, respectively, and polypectomy was, after laparotomy, the most common surgical procedure these children underwent. The extent of intranasal surgery for polyposis was found to be inversely proportional to the recurrence rate. A simple polypectomy was relatively ineffective treatment; when performed in conjunction with a Caldwell-Luc and either an intranasal or extranasal ethmoidectomy, the recurrence rate was less than 13%. Otologic problems, found in 8% of patients, included chronic otitis media (2.5%) and acute otitis media (5.5%). Only five patients required pressure-equalizing tubes. Recent genetic advances of immense importance are also described. Although the basic gene defect has yet to be elucidated, by use of a technique known as restriction-fragment-linked polymorphism, the gene associated with CF has been found in the middle of the long arm of chromosome 7. By following gene markers closely associated with this gene, it is possible to do carrier tests within affected families and, if certain criteria are met, perform prenatal diagnosis. Eventual isolation and characterization of the gene will follow, hopefully making prevention possible and treatment more effective.
Details
- Title: Subtitle
- Cystic Fibrosis—An Otolaryngologic Perspective
- Creators
- Ralph Cepero - From the Department of Otolaryngology and Communicative Sciences, Baylor College of MedicineRichard J.H Smith - From the Department of Otolaryngology and Communicative Sciences, Baylor College of MedicineFrancis I Catlin - From the Department of Otolaryngology and Communicative Sciences, Baylor College of MedicineKyle L Bressler - From the Department of Otolaryngology and Communicative Sciences, Baylor College of MedicineGlenn T Furuta - From the Department of Otolaryngology and Communicative Sciences, Baylor College of MedicineKevin C Shandera - From the Department of Otolaryngology and Communicative Sciences, Baylor College of Medicine
- Resource Type
- Journal article
- Publication Details
- Otolaryngology--head and neck surgery, Vol.97(4), pp.356-360
- DOI
- 10.1177/019459988709700403
- PMID
- 3120101
- NLM abbreviation
- Otolaryngol Head Neck Surg
- ISSN
- 0194-5998
- eISSN
- 1097-6817
- Language
- English
- Date published
- 10/1987
- Academic Unit
- Roy J. Carver Department of Biomedical Engineering; Molecular Physiology and Biophysics; Anatomy and Cell Biology; Stead Family Department of Pediatrics; Iowa Neuroscience Institute; Otolaryngology; Internal Medicine
- Record Identifier
- 9984006403802771
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