The Acute Porphyrias Diagnosis

Acute intermittent porphyria is the most common type of inducible porphyria. Variegate porphyria and hereditary coproporphyria also cause acute porphyric attacks. A fourth disorder, delta-aminolevulinic acid (ALA) dehy-drase deficiency, is very rare and is unlikely to be encountered by most physicians.

several signs and symptoms may occur during an acute porphyric attack, reflecting widespread involvement of the nervous system. The most frequent is abdominal pain which is caused by an autonomic neuropathy. Other features of autonomic neuropathy are tachycardia, hypertension, constipation, and urinary retention. Peripheral neuropathy may develop as the attack progresses and can lead to paralysis in its most severe form. Central nervous system manifestations include organic brain syndrome, depression, and seizures.

The diagnosis of an acute porphyric attack is made by demonstrating increased urinary excretion of the porphyrin precursors ALA and porphobilinogen (PBG). The measurement of urinary porphyrin excretion is not used to establish the diagnosis and may be misleading if the excretion of the porphyrin precursors is not measured. Between attacks the diagnosis is more difficult, but in acute intermittent porphyria the urinary excretion of porphyrin precursors usually remains elevated. The measurement of erythrocyte PBG deaminase activity can also be used to establish the diagnosis of acute intermittent porphyria. In variegate porphyria there is a plasma porphyrin fluorescence marker which is specific for the disorder. Increased fecal excretion of coproporphyrin is found in hereditary coproporphyria.

Peripheral Neuropathy Natural Treatment Options

Peripheral Neuropathy Natural Treatment Options

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