heme-arginate and Peripheral-Nervous-System-Diseases

Acute intermittent porphyria (AIP) is a rare metabolic disorder of heme biosynthesis characterized by enzymatic defect of porphobiligen desaminase with accumulation and increased excretion of porphyrins and their precursors. Clinical picture is characterized by attacks with a triad of abdominal pain, psychiatric disorder and neurological involvement (central and peripheral). Peripheral nervous system manifestations, often precipitated by porphyrinogenic medications are of poor outcome.. We report a new cases A 13-year-old girl who presented several attacks of AIP and developed acute severe axonal motor neuropathy, three weeks after porphyrinogenic medications (Famotidin, Phenobarbital and Nifedipine).. We stress on the importance of early diagnosis of AIP to prevent serious neurological complications often precipitated by medications and the efficiency of heme arginate treatment when administrated early during the attacks.

Topics: Acute Disease; Adolescent; Arginine; Electromyography; Famotidine; Female; Heme; Heme Oxygenase (Decyclizing); Humans; Nifedipine; Peripheral Nervous System Diseases; Phenobarbital; Porphyrias; Porphyrinogens; Time Factors

We report on a patient with acute intermittent porphyria (AIP) who, during treatment with hypertonic glucose, developed peripheral neuropathy. Once haeme-arginate was started, the progression of the neuropathy was attenuated. This suggests that hypertonic glucose may be inadequate in preventing the development of neuropathy in a patient with porphyria. However, haeme-arginate, if started early, can attenuate the progression of porphyric neuropathy.

Topics: Adult; Arginine; Glucose Solution, Hypertonic; Heme; Humans; Male; Peripheral Nervous System Diseases; Porphyria, Acute Intermittent