婴幼儿先天性歪嘴哭综合征(附4例报告)

目的总结歪嘴哭综合征患儿的临床特征及合并畸形。方法报告4例歪嘴哭综合征患儿的临床表现，并通过有关检查确定其合并的先天畸形情况。结果4例歪嘴哭患儿，3例合并先天性心脏病，1例未发现先天畸形。结论对于歪嘴哭综合征患儿应仔细检查，以便及时发现其他畸形并予以必要的医学干预。     

婴幼儿铅中毒脑病临床特点及预后分析

目的 探讨铅中毒脑病的临床特点及预后。方法 回顾分析衡阳市中心医院儿科2002~2003年26例铅中毒脑病患儿的临床资料及随访结果。结果 26例铅中毒脑病患儿临床均表现为频繁的抽搐伴意识障碍，中度以上贫血。伴呕吐23例、腹泻21例、发热11例、肝大5例。26例患儿治疗前血铅平均为8.486μmol/L，尿铅平均为3.598μmol/L，Hb均低于90g/L，最低55g/L。血钙均低于1.86mmol/L， 最低1.03mmol/L。脑脊液蛋白均增高，22例心肌酶谱增高，提示心肌损害明显。头颅CT/MRI仅1例脑室系统轻度扩张，全部病例经驱铅治疗后无一例死亡，1年后随访,继发性癫疒间4例，脑瘫4例。结论铅中毒脑病是铅中毒的严重类型，预后不良。对不明原因的无热惊厥者应注意及时行血铅检查，以便早期诊断，防止漏诊。 Abstract Objective Study the clinical characteristics and prognosis of lead poisoning encephalopathy.Methods We reviewed and analyzed the clinical data and the results of the 26 cases of lead poisoning encephalopathy.Results All the 26 cases had frequen convulsion,accompanied by disturbance of consciousness and above middle pitch of anemia.Among the 26 cases vomit ting was found in 23 cases,diarrhea in 21 cases,fever in 11 cases,live swelling in 5 cases.Supplemental examination:before treatment,the average of blood lead level was 8.486μmol/L，urine lead level was 3.598μmol/L,Hb was lower than 90g/ L,the lowest being 5g/L.The blood calcium was all lower than 1.86 mmol/ L,the lowest being 1.03 mmol/L.Protein of cerebrospinal fluid in all cases was over normal.Myocardial enzyme of 22 cases was over normal,suggesting myocardials injured obviously.Only 1 case was found ventricle system expanding lightly in CT/MRI.All cases had no death after treatment of driving lead.The 12 month follow up result suggested that second epilepsy was found in 4 cases and cerebral palsy in 4 cases.Conclusion The lead poisoning encephalopathy is the most serious type of the lead poisoning,and prognosis is not good. The patient of convulsion with unknown reason,and no fever,should be checked the blood lead for early diagnosis in order to prevent from leaking diagnosis. Key wordsLead poisoning;Encephalopathy;Clinical characteristics;Prognosis      

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目的探讨铅中毒脑病的临床特点及预后。方法回顾分析衡阳市中心医院儿科2002～2003年26例铅中毒脑病患儿的临床资料及随访结果。结果26例铅中毒脑病患儿临床均表现为频繁的抽搐伴意识障碍,中度以上贫血。伴呕吐23例、腹泻21例、发热11例、肝大5例。26例患儿治疗前血铅平均为8.486μmol/L,尿铅平均为3.598μmol/L,Hb均低于90g/L,最低55g/L。血钙均低于1.86mmol/L,最低1.03mmol/L。脑脊液蛋白均增高,22例心肌酶谱增高,提示心肌损害明显。头颅CT/MRI仅1例脑室系统轻度扩张,全部病例经驱铅治疗后无一例死亡,1年后随访,继发性癫疒间4例,脑瘫4例。结论铅中毒脑病是铅中毒的严重类型,预后不良。对不明原因的无热惊厥者应注意及时行血铅检查,以便早期诊断,防止漏诊。