Hudgins Kerstin
School of Nursing, Fayetteville State University, Fayetteville, North Carolina.
Crit Care Nurs Q. 2019 Apr/Jun;42(2):192-197. doi: 10.1097/CNQ.0000000000000253.
Diagnosis of porphyria is often difficult due to the range of symptoms, which are common in many other disorders, hence frequently leading to misdiagnosis. Attacks can be triggered through sunlight and contact with diverse substances, including medications. Signs are severe, appear quickly, and tend to last from days to weeks, affecting the skin, mentation, the digestive, cardiovascular, nervous, and muscular system. Neuropathy signifies a severe and potentially life-threatening attack. Porphyria is mainly an inherited disorder, but can be acquired, and emerges in adults and children. The exact rates of porphyria are unknown and vary around the world. Diagnosis, clinical presentation, treatment, and disease management are introduced.
由于症状范围广泛，血卟啉病的诊断往往很困难，这些症状在许多其他疾病中也很常见，因此常常导致误诊。发作可由阳光和接触多种物质（包括药物）引发。症状严重，出现迅速，往往持续数天至数周，影响皮肤、精神状态、消化、心血管、神经和肌肉系统。神经病变意味着严重且可能危及生命的发作。血卟啉病主要是一种遗传性疾病，但也可能后天获得，可发生于成人和儿童。血卟啉病的确切发病率未知且在世界各地有所不同。本文介绍了其诊断、临床表现、治疗及疾病管理。
