A Case of Anhidrotic Ectodermal Dysplasia with Atrophic Rhinitis / 대한이비인후과학회지

Anhidrotic ectodermal dysplasia is a rare genetic disorder characterized by absence or diminished numbers of structures derived from the ectoderm, and it is reported to be inherited as an x-linked recessive trait. It is recognized clinically by anhidrosis, hypotrichosis, anodontia or reduced numbers of teeth with deformed shape and characteristic facial features. In addition, otolaryngological manifestations include atrophic rhinitis, sensorineural hearing loss, and conductive hearing loss and satyr ear, among others. Early diagnosis of anhidrotic ectodermal dysplasia can prevent fatal hyperpyrexia and appropriate genetic counseling can be followed to make a reasonable future plans for the pediatric patient. A 2-month-old infant was referred with symptoms of intermittent nasal obstruction and crust formation in both nasal cavities. The nasal endoscope demonstrated atrophic changes of nasal mucosa and radiologic study showed an unerupted conical shaped tooth. The diagnosis of anhidrotic ectodermal dysplasia was confirmed with the finger impression test that revealed deficiency of sweat pores. We report a case of anhidrotic ectodermal dysplasia with a review of the literature.

Clinical Analysis of Refractory Epistaxis / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: Epistaxis is a frequently seen disease in the field of otorhinolaryngology. However, many patients have to revisit the hospital because of the failure of the initial treatment, in which cases, the inconvenience is greater due to more severe bleeding. We studied epistaxis patients who visited our service during the last 10 years to analyze the causes of visits and the possible countermeasures. SUBJECTS AND METHOD: We studied retrospectively the clinical cases of 616 emmergency patients and 65 patients admitted for the treatment of epistaxis during the last 10 years, from August 1995 to July 2004. The group I, 505 patients (74.2%), improved with only one treatment. The group II, 176 patients (25.8%), showed refractory epistaxis following the initial treatment. RESULTS: In the group I, Kisselbach's area (68.1%) was the most common site to be treated, followed by Woodruff's area (13.5%), anterior end of inferior turbinate (8.3%), and superior portion of septum (2.0%). In the group II, the most frequent area to be treated was Woodruff's area (46.0%), followed by Kisselbach's area (31.8%), superior portion of septum (4.6%), anterior end of inferior turbinate (4.0%). CONCLUSION: The most important factor in the failure of primary care was because the precise area of bleeding was not found at the time of initial treatment. Therefore, it is important that we must check the areas by endoscope carefully to decrease the failure of initial treatment of epistaxis.