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全胃肠外营养并发生物素缺乏症是临床急症,其诊断标准和治疗剂量还不统一。作者最近成功地诊洽了3例(男2,女1;年龄11～13个月)。他们均因肠病作过广泛肠切除。术后因反复伤口感染和败血症,长期用广谱抗生素治疗。因不能经消化道喂养而给全胃肠外营养3～4个月后,下睑外眦、面颊、鼻孔周围、口角、会阴、臀部等部位出现红斑疹,体鳞屑样表皮脱落和浆液渗出;继之有头发、眉毛、睫毛乃至全身体毛脱落,皮肤呈蜡样苍白,伴有睑炎和结膜炎;面部皮下脂肪分布异常;此外还表现易激惹、嗜睡;肌张力减退,不能独自坐、站及行走;发育落后、相互行为极少等。局部用磺胺醋酰、制霉菌素、氢化可的松、氧化锌和红花油等均无效。其中1例反复发生严重的酸中毒和心力衰竭。根据皮疹外观和脱发,初诊为锌或必需脂肪酸缺
Total parenteral nutrition and biotin deficiency is a clinical emergency, the diagnostic criteria and the treatment dose is not uniform. The authors recently successfully treated 3 cases (male 2, female 1; age 11 to 13 months). They have extensive bowel resection due to enteropathy. After repeated wound infection and sepsis, long-term use of broad-spectrum antibiotics. Because can not be fed through the digestive tract to give parenteral nutrition after 3 to 4 months, the lower eyelid, cheeks, around the nostrils, mouth, perineum, buttocks and other parts of erythema rash, body scales, exfoliation and serous exudate ; Followed by hair, eyebrows, eyelashes and even body hair loss, pale waxy skin, accompanied by blepharitis and conjunctivitis; facial subcutaneous fat distribution abnormalities; also showed irritability, lethargy; muscle tension decreased, can not sit alone , Standing and walking; development is backward, mutual behavior is minimal and so on. Topical sulfanilamide, nystatin, hydrocortisone, zinc oxide and safflower oil, etc. are invalid. One case of recurrent severe acidosis and heart failure. According to the rash appearance and hair loss, newly diagnosed with zinc or essential fatty acid deficiency