Acute porphyria polyneuropathy and positive treatment effect glucose

Abstract

The athors described the case of acute porphyria polyneuropathy with an effective treatment for glucose. The disease is manifest at the age of 36 years with abdominal pain, bowel clinical paresis. After 3 weeks from the onset of the disease originated tetraparesis due to peripheral nerve lesions with a primary lesion of the proximal extremities. There was a dark urine. There was a tachycardia with a heart rate of 125 1 per minute. The qualitative urine test for porphyria with Ehrlich’s reagent, which was positive. By quantifying total urinary porphyrins porphyrins were increased more than 10 times, porphobilinogen was increased more than 100 times, delta-aminolevulinic acid was increased almost 15-fold. The diagnose was acute porphyria, severe course, sensory-motor polyneuropathy, severe peripheral tetraparesis. The treatment was: glucose given treatment dosage of 100 g, then 250 g of dry matter during the day. Also appointed B vitamins (B1, B6, B12), alpha-lipoic acid 600 mg orally. The was a positive effect of the treatment. The muscle strength triceps muscle and the iliopsoas muscle became 5 points, the strength of the deltoid muscle increased significantly. The strength of the flexors of fingers also increased slightly. Consequently, for treatment of acute porphyria glucose effectively. Glucosa - inhibitor of synthetase enzyme i aminolevulinic acid. Glucose acts weaker arginate gem. Therefore, in the next stage of its inefficiency porphyria treatment prescribed heme arginate. In this case, glucose was effective. Appointment of haem arginate is not needed.