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患儿,男,2岁。两年前出现口唇青紫,呈持续性,冬季紫绀加重,无不适症状,入院前两个月紫绀加重,全身皮肤粘膜青紫,唇、口腔粘膜及四肢末端紫绀明显。在病程中患儿饮食、智力均正常,无啼哭和活动后气急、紫绀加重及蹲踞现象。父母系姨表兄妹结婚。体检:T37.1℃,P100次/min,R34次/min,体重15kg。皮肤粘膜呈灰谒色,以口唇紫绀明显。指趾末端紫绀,无杵状指。无鼻扇,双肺、心、肝、脾正常。血RBC4.5℃×10~(12)/L,Hb130g/L,WBC5.6×10~9/L,N0.63,E0.02,L0.35;Hb电泳正常,高铁Hb还原试验正常。心脏彩色多普勒检查无异常。抽取的静脉血呈棕褐色,在空气中放量2h不变色。诊断为先天性高铁血红蛋白血症。予吸氧无效,改给亚甲蓝20mg加于10％GS20ml中静脉注射,15min后青紫消失,观察2天紫绀消失出院。
Children, male, 2 years old. Appearing two years ago, lips were purple, persistent, cyanosis increased in winter, no symptoms, cyanosis increased two months before admission, systemic skin mucosal bruising, lips, oral mucosa and extremities cyanosis. During the course of children’s diet, intelligence are normal, no crying and post-exercise urgency, cyanosis increased and squatting phenomenon. Parents aunt cousins marriage. Physical examination: T37.1 ℃, P100 times / min, R34 times / min, weight 15kg. Mucosal grayish Ye color to clear cyanotic lips. Finger toe end of cyanosis, without clubbing means. No nose fans, lungs, heart, liver, spleen normal. Blood RBC4.5 ℃ × 10 ~ (12) / L, HB130g / L, WBC5.6 × 10 ~ 9 / L, N0.63, E0.02, L0.35; Hb electrophoresis normal, high-speed rail Hb reduction test is normal. Heart color Doppler examination no abnormalities. Extraction of venous blood was tan, heavy volume in the air 2h does not change color. Diagnosis of congenital methemoglobinemia. Give oxygen is invalid, change to methylene blue 20mg plus 10% GS20ml in the intravenous injection, after 15min bruising disappeared, observed 2 days cyanosis disappeared.