血卟啉病的临床多样性.docVIP

血卟啉病的临床多样性 目录 TOC \o 1-9 \h \z \u 目录 1 正文 1 文1：血卟啉病的临床多样性 1 1 临床特征分布 2 172例血卟啉病的临床特征分布，见表1。 2 2 血卟啉病的常见诱因［1］ 3 3 误诊情况［2～5］ 3 4 讨论 4 文2：Peyronie病的临床诊治研究 6 1流行病学 7 参考文摘引言： 8 原创性声明（模板） 9 文章致谢（模板） 9 正文 血卟啉病的临床多样性 文1：血卟啉病的临床多样性 ［Abstract］ Objective In order to give clinical docto a wider thought and decrease misdiagnosis and mistreatment,we analyzed the various iidious clinical manifestation of Clinical manifestatio,physical sign,laboratory test result of 4 cases in recent 8 yea diagnosed in our hospital were clinical manifestation and motivation of hematoporphyria of 172 internal cases reported in recent 5 yea was Most of the cases were ache,skin lesion and abnormality of nerves and mind were the 3 main symptoms of hematoporphyria,besides,there were several other manifestatio such as athrepsy,cotipation,nausea,vomiting,difficulty walking,bucking,paralysis, and agitation could aggravate and induce kinds of motivation could induce Because of their diveity in clinical manifestation,more and more docto pay close attention to is easy to lead misdiagnosis without the typical clinical of our docto especially working in basic level must inquest case history carefully and diagnose in time with their laboratory test results,thus we can decrease misdiagnosis and mistreatment. ［Key words］ hematoporphyria;diveity;misdiagnosis 血卟啉病又称血紫质病，由先天性和后天性卟啉代谢紊乱所引起的代谢性疾病。主要为卟啉及（或）卟啉前体的产生、排泄增多，在体内聚积，引起多系统损害。本文主要讨论先天性血卟啉病。本病临床表现多种多样，主要临床表现为光感皮肤损害、腹痛及神经精神症状。可隐匿起病，易误诊。本文总结近10年来国内文献报道的172例及收集本院近8年来收治的4例病例，现对其临床表现、诊断及 治疗 进行分析和讨论。 1 临床特征分布 172例血卟啉病的临床特征分布，见表1。 172例病患中，女101例，男71例，女∶男为∶1，年龄为2～72岁，以中青年为主，病程4天至20年不等。临床症状：绝大多数有腹痛（154例），其中剧烈腹痛，阵发绞痛65例。临床收集4例见表2。本院病例中例1、例2、例3均有腹痛，但例2、例3腹部无典型压痛。腹痛可伴有腹泻（2例）、恶心呕吐（85例）；周围神神、运动神经症状83例，包括乏力，全身痛，四肢、背痛，皮肤感觉异常，呼吸麻痹，行走困难；脑部症状62例，其中不明原因抽搐12例，癫痫样发作4例，颅高压12例，脑电图异常7例，四肢瘫痪9例，双枕叶脑梗死至皮质盲2例，声嘶4例；精神症状共79例，其中躁狂2例，有打人行为1例，曾起疑心杀人1例，烦躁、多疑、幻觉、自语呆滞、精神异常33例，神经衰弱25例；皮肤病变37例，其中皮肤色素沉着8例，光感性皮炎27例，水疱、红斑、丘疹2例；非主征以外的症状临床最多见的为恶心呕吐85例，便秘65例，腹泻23例