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本文报告1例先天性无痛无汗症(CIPA)患儿。女婴,7个月,汉族。生后第8天开始间断发热伴无汗7个月,体温超过38.5℃即哭闹不安,降温后即安静。哭时少泪。6个月出牙后反复咬伤自已双拇指,舌尖下溃疡久不愈。体检:皮肤干燥,舌尖下有一小蚕豆大小的溃疡,双拇指咬伤破溃,特殊检查:针刺骨膜、跟腱及静脉穿刺取血均无痛觉;味觉及嗅觉似乎缺如;1‰毛果云香碱皮内注射局部无汗;组织胺皮内注射局部有风团,周围无发红反应;新斯的明皮下注射无泪。皮肤活检:见有汗腺及毛
This article reports a case of congenital painless and no sweat (CIPA) in children. Baby girl, 7 months old, Han nationality. 8 days after birth began intermittent fever with no sweat 7 months, body temperature over 38.5 ℃ that is crying uneasy, after cooling is quiet. Less tears when crying. 6 months after toothpick repeated bites his own double thumb, tongue ulcer for a long time without healing. Physical examination: dry skin, a small bean-sized ulcer under the tongue, double thumb bite ulceration, special inspection: acupuncture periosteum, Achilles tendon and venous blood were painless; taste and smell seem absent; 1 ‰ hair Rufous intradermal injection of local anti-perspiration; histamine intradermal injection of local wind group, no redness around the reaction; neostigmine subcutaneous injection without tears. Skin biopsy: see sweat glands and hair