An acute attack of variegate porphyria complicated by severe autonomic neuropathy.
A patient with familial variegate porphyria is described. She was admitted in an acute attack and developed severe dysautonomia manifested clinically by postural hypotension, tachycardia and constipation with severe abdominal pain. Whilst formal testing of autonomic function demonstrated the presence of marked abnormalities, peripheral nerve conduction studies were normal.
D000208:Acute Disease; D000328:Adult; D000622:Aminolevulinic Acid / Q000652:urine; D001342:Autonomic Nervous System Diseases / Q000150:complications*; D005260:Female; D006801:Humans; D011162:Porphobilinogen / Q000652:urine; D011164:Porphyrias / Q000150:complications* / Q000175:diagnosis / Q000652:urine; D011166:Porphyrins / Q000652:urine; D012871:Skin Diseases / Q000150:complications* / Q000175:diagnosis
D011166:Porphyrins; D011162:Porphobilinogen; D000622:Aminolevulinic Acid