Sugimura K
Nagoya University College of Medical Technology.
Nihon Rinsho. 1995 Jun;53(6):1418-21.
The clinical features of acute intermittent porphyria (AIP) are described in this chapter. AIP is inherited as an autosomal dominant pattern of inheritance. Prevalence in Japan is 1.5 in 100,000. Attacks are more frequent in women of 20s to 40s. The common clinical pattern of symptom involves acute abdominal pain, psychiatric disturbances, and acute neuropathy. The nerve biopsy shows segmental demyelination and axonal degeneration. Many small vacuolations are distinctively seen in all of the cell components of the nerve. Clinical diagnosis is not difficult when doctors keep the possibility of AIP in their minds in cases of abdominal pain, weakness and mental symptoms. The major trust of treatment is avoidance of acute attacks which is almost entirely dependent upon avoidance of porphyrogenic drugs. The intravenous administration of heme and glucose is important and effective therapy for acute attacks of porphyria.
本章描述了急性间歇性卟啉病（AIP）的临床特征。AIP以常染色体显性模式遗传。在日本，其患病率为十万分之一点五。20多岁至40多岁的女性发作更为频繁。常见的症状模式包括急性腹痛、精神障碍和急性神经病变。神经活检显示节段性脱髓鞘和轴突变性。在神经的所有细胞成分中都能明显看到许多小空泡。当医生在遇到腹痛、虚弱和精神症状时考虑到AIP的可能性，临床诊断并不困难。治疗的主要要点是避免急性发作，这几乎完全依赖于避免使用致卟啉药物。静脉注射血红素和葡萄糖是治疗卟啉病急性发作的重要且有效的疗法。
