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奥尼赖病(Onyalai)是以口腔粘膜和嘴唇出血性大疱为特征的一种急性紫癜性综合征,曾在非洲流行。常见于11～20岁的青少年。作者最近观察到1例45天的土耳其男孩患本病,表现为严重胃肠道出血,经大剂量静脉内甲基强的松龙治疗后迅速恢复。病例报告男性婴儿非近亲结婚,第1产,突然面色苍白、瘀斑、水疱、口腔粘膜和嘴唇变色,血便天。患儿和母亲无感染、免疫接种和服药,包括阿斯匹林。体检发现,患儿重4200克,高55cm,头围37.5cm,身体有瘀斑和出血性大疱伴口腔粘膜下出血,硬腭和嘴唇最为突出,腹部隆起,但肝脾无肿大。实验室检查示贫血(Hb 10.92克/dl,Hct32%),网状细胞正常(0.2%),正成红细胞增多(3%),白细胞轻度增多,血小板明显减少(36×10~3/μl)。Coomb’s试验阴性,骨髓检查示正成红细胞增多,巨核细胞增加,腹部钡灌肠和超声检查未发现肠套迭或其它异常。患儿母亲血小板计数326×10~3/μl,无血小板减少的病史。诊断奥尼赖综合征后
Onyalai is an acute Purpuric Syndrome characterized by buccal mucosa and hernia of the lip, which was endemic in Africa. Common in 11 to 20-year-old adolescents. The authors recently observed a 45-day-old Turkish boy who developed the disease, presenting with severe gastrointestinal bleeding, recovering rapidly after high-dose intravenous methylprednisolone treatment. Case reports Male infants non-relatives marry, the first production, suddenly pale, ecchymosis, blisters, oral mucosa and lips discoloration, bloody days. Children and mothers free of infection, immunizations and medication, including aspirin. Physical examination found that children weighing 4200 grams, 55cm high, head circumference 37.5cm, body ecchymosis and hemorrhagic bullous bleeding with oral submucosa, the palate and lips most prominent, abdominal bulge, but no enlarged liver and spleen. Laboratory tests showed anemia (Hb 10.92 g / dl, Hct 32%), normal reticulocytes (0.2%), positive erythrocytes (3%), mild leukocytosis and decreased platelets (36 × 10-3 / μl) . Coomb’s test negative, bone marrow examination showed positive polycythemia, megakaryocytes, abdominal barium enema and ultrasound examination found no intussusception or other abnormalities. Children with mothers platelet count 326 × 10 ~ 3 / μl, no history of thrombocytopenia. After diagnosis of O’Neil syndrome