Tilbery C P, Sanvito W L, Espindola E S
Arq Neuropsiquiatr. 1979 Jun;37(2):151-7. doi: 10.1590/s0004-282x1979000200005.
The study of six patients with acute intermittent porphyria is reported. Clinical and biochemical studies have been performed during the long hospitalization of the patiets. The main disautonomic aspects were the arterial hypertension and the tachycardia in four patients, cardiac arrest in three patients and respiratory arrest in five patients. The cause of this problems is probably the involvment of the autonomic adrenergic system. The authors observed also three patients with hyponatremia probably resulting from inappropriate secretion of antidiuretic hormone. The prognosis of the acute intermittent porphyria is worse if the patients have disautonomic symptoms; three patients died and two had neurological sequalae (motor tetraparesis).
本文报告了对6例急性间歇性卟啉病患者的研究。在患者长期住院期间进行了临床和生化研究。主要的自主神经功能紊乱方面包括4例患者出现动脉高血压和心动过速,3例患者发生心脏骤停,5例患者出现呼吸骤停。这些问题的原因可能是自主肾上腺素能系统受累。作者还观察到3例低钠血症患者,可能是抗利尿激素分泌不当所致。如果患者出现自主神经功能紊乱症状,急性间歇性卟啉病的预后更差;3例患者死亡,2例有神经后遗症(肢体运动性瘫痪)。