低汗性外胚葉形成不全症の鼻腔内痂皮

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  • Nasal Crust in a Case of Hypohidrotic Ectodermal Dysplasia
  • 臨床 低汗性外胚葉形成不全症の鼻腔内痂皮
  • リンショウ テイカンセイ ガイハイヨウ ケイセイ フゼンショウ ノ ビコウ ナイカヒ

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<p>Hypohidrotic ectodermal dysplasia is a hereditary syndrome characterized mainly by the triad of less hair, less sweat and less teeth. It is associated with hypoplasia of the ectoderm caused by a genetic abnormality, and treatment remains mainly symptomatic. I encountered a case in a boy who presented with rhinostenosis at one month after birth. There were no anatomic abnormalities of the nasal cavity, however, the entire nasal cavity was filled with crust associated with atrophic nasal inflammation. Nose breathing was disturbed because of the pervasive crust and the child developed recurrent episodes of upper respiratory tract infection with a bad odor of the nasal mucus. Provision of care to prevent nasal crust formation for 4 years 6 months at the outpatient department led to improvement of the nose breathing and the bad odor of the nasal mucus. Herein, I report the method and the outcome.</p>

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