满某某,男,12岁.因皮肤粘腆变黑6年,1年多来两次发作休克入院。6年前患儿皮肤、粘膜渐变黑,伴身软无力,心慌心累,平时不喜活动.在某院诊断为贫血,经治疗无效.入院前1年因进食稍多后恶心腹泻、发热、全身“紫绀”、反应迟钝、心率增快,心音低弱及血压0/0,以革兰氏阴性 Full XX, male, 12 years old. Black skin due to sticky black for 6 years, more than 1 year episode of shock admitted to hospital. Six years ago, children with skin, mucosal gradient black, accompanied by weakness, palpitation, usually do not like activities in a hospital diagnosed with anemia, after treatment ineffective.1 year before admission due to eating a little more after nausea and diarrhea, fever, Systemic “cyanosis”, unresponsive, faster heart rate, low heart sound and blood pressure 0/0, to Gram-negative