急性间歇性血卟啉病的临床观察和护理..docVIP

急性间歇性血卟啉病的临床观察和护理 摘要: 目的 根据温州市第三人民医院收治的3名急性间歇性血卟啉病患者的诊治和护理过程，总结出对此病的临床观察和护理的经验，对急性发作期护理、缓解期护理、心理护理、出院后护理进行细致总结，提供临床参考。方法 结合温州市第三人民医院2010-2015年收治的3名患者的临床观察和护理进行报道和分析。结果 三例患者都有其特殊的临床特点和多发的临床症状，经过周详的临床观察和护理，三例患者病情都得到了控制，并顺利出院，患者出院后在医务人员的指导下和家属帮助下仍进行着院外护理。 结论 急性间歇性血卟啉病尚无根治方法，可针对不同阶段的临床症状进行密切临床观察和对症治疗，再适当进行心理护理，出院后仍要继续进行院外护理，才能良好地控制病情，改善生活质量。 关键词：急性间歇性血卟啉病；临床观察；护理 OBJECTIVE: According to our hospital three acute intermittent porphyria patients blood diagnosis, treatment and care process, summed up the experience of clinical observation and care of this disease, acute exacerbation of care, ease of care, psychological care, meticulous care after discharge summary, provided clinical reference. Method combines hospital where 2010 - 2015 Clinical treated three patients and care coverage and analysis. Results Three patients have their specific clinical features and clinical symptoms of multiple, through careful clinical observation and care, three patients with the disease have been brought under control, and was discharged after being discharged under the guidance of medical staff is still engaged perfect care. Conclusion no cure for acute intermittent porphyries’ method, close clinical observation and symptomatic treatment for the different stages of clinical symptoms, and appropriate psychological care, still continue to care after discharge, can well control the disease, improve living quality. Keywords: acute intermittent porphyry; clinical observation; nursing 血卟啉病又称为血紫质病，系机体在血红素合成过程中酶活性发生变化或缺陷，从而导致卟啉或卟啉前体物质累积于组织、血液或出现于尿中，并表现出各种症状。其中，最常见的类型即为急性间歇性血卟啉病（AIP），为一种常染色体显性遗传性疾病，由于胆色素原（PBG）脱氨酶(HMB合成酶)活性缺乏所致。间歇性腹痛、神经精神症状、光感性皮肤损害μmol/L。尿PBG阳性，?孕酮125.6nmol/L?，诊断为孕酮增高导致的急性间歇性血卟啉病。 1.3例3 患者男，72岁，腹痛伴恶心、呕吐，重度黄疸，患者自述平时爱饮酒，并有神经官能症病史。入院时体温38.5℃，血压120/80mmHg，呼吸20次/分，心率90次/分。立位腹平片显示“肠管扩张伴多发气液平面”， 胃镜、心电图检查均无异常，肝穿刺活检显示肝细胞肿胀，汇管区炎细胞浸润；血检：ALT500IU/L，AST203 IU/L，TBIL352.0μmol/L，μmol/L，溶血相关实验阴性。经保肝补液治疗，转氨酶和胆红素恢复正常。但2周后，腹痛、黄疸加重，尿检发现尿液为橘红色，尿PBG阳性，287nmol/g，?严重发作需要用血红素治疗，每日3mg/kg，人白蛋白与羟高铁血红素重组形成血红素