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血卟啉病(Porphyria)是少见的一种先天性卟啉代谢障碍疾病,临床表现复杂多样,无特异性,以腹痛、皮肤及精神神经三大症状群为表现,类型各异,极易误诊。现将我科收治肝性血卟啉病(肝炎型)1例报告如下。 患者,男,34岁,工人,住院号107595,因持续尿黄、双眼及皮肤黄染伴反复腹部胀痛、食欲差、呕吐5~+月,在当地按急性病毒性肝炎治疗,症状无明显好转于1989年9月9日来本院检查,化验肝功能异常,按急性黄疸型病毒性肝炎收住院治疗,经积极护肝及对症治疗,病情好转,肝功能正常。但仍有尿色黄、双眼及皮肤黄染,住院47天出院,继续门诊治疗。因上述症状反复出现,尿色黄、双眼及皮肤黄染加深,肝功能异常,拟急性黄疸型病毒性肝炎再次住院。住院第2天观察尿色呈咖啡样,留置尿液可变咖啡色,经反复查尿卟啉呈阳性而明确诊断。 讨论 血卟淋病为一种先天性卟啉代谢紊乱的疾病,肝
Porphyria is a rare disease of congenital porphyria metabolic disorder. Its clinical manifestations are complex and diverse, nonspecific, and are characterized by three groups of symptoms of abdominal pain, skin and neuropsychiatry, different types and easily misdiagnosed . Now we receive liver hematoporphyrin disease (hepatitis type) in 1 case reported as follows. Patient, male, 34 years old, worker, hospital number 107595, due to persistent urine yellow, yellow eyes and skin with repeated abdominal pain, poor appetite, vomiting 5 ~ + month, according to the local treatment of acute viral hepatitis, no significant symptoms Improvement September 9, 1989 to our hospital for examination, test abnormal liver function, according to acute jaundice virus hepatitis hospital treatment, the active liver and symptomatic treatment, the condition improved, normal liver function. But there are still urine yellow, yellow eyes and skin, hospitalized 47 days discharged, continue out-patient treatment. Recurrent because of the above symptoms, urine yellow, dark yellow eyes and skin deepening, abnormal liver function, acute jaundice viral hepatitis re-hospitalized. On the second day of hospitalization, urine color was observed as coffee-like, indwelling urine variable brown, after repeated examination of urinary porphyrin was positive and a clear diagnosis. Discussion Hematoxylin and porphyria is a disorder of congenital porphyrin metabolism, liver