失盐型先天性肾上腺皮质增生是新生儿虚脱和重度低钠血症的主要病因、但作为新生儿阿迪森氏危象的特有征象,低渗性脱水并发颅内高压却很少为人熟知。本文报告一例三周龄男婴,因相继出现厌食、腹泻、呕吐1周入院。足月正常分娩,出生时体重3.1kg。其家人均健康。检查:体温低(直肠温35℃、消耗性重病容。重度脱水、烦燥、前囟紧张、矢状骨缝增宽(头围32.5cm)。眼底检查无异常。染色体正常。外生殖器表型正常。血清钠103mmol/L、钾7.9mmol/L、氯79mmol/L、尿素氮17.2mmol/L、标准碳酸氢11mmol/L,PH7.23,17-α羟基黄体酮389mmol/L;尿钠41mmol/L、钾17mmol Loss of salt congenital adrenal hyperplasia is a major cause of neonatal collapse and severe hyponatremia, but as a unique sign of neonatal Addison’s crisis, hypotonic dehydration complicated by intracranial hypertension is rarely well-known. This article reports a three-week-old baby boy, one after another due to anorexia, diarrhea and vomiting. Full-term normal delivery, birth weight 3.1kg. Their families are healthy. Check: low body temperature (rectal temperature 35 ℃, severe demyelination, severe dehydration, irritability, anterior fontanel, sagittal suture widened (head circumference 32.5cm.) Fundus examination was normal. Chromosome normal genitalia phenotype Normal serum sodium 103mmol / L, potassium 7.9mmol / L, chlorine 79mmol / L, urea nitrogen 17.2mmol / L, standard bicarbonate 11mmol / L, PH7.23,17-alpha hydroxy progesterone 389mmol / L; / L, potassium 17mmol