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Journal Review: Porphyric neuropathy
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Pdf Summary
Porphyric neuropathy is a neurological manifestation of acute hepatic porphyrias, characterized by a motor predominant axonal neuropathy that primarily affects the upper extremities. Diagnosis involves testing for porphyrin precursors in a urine sample. Treatment includes intravenous heme and avoidance of trigger medications. The exact mechanisms behind the neuropathy are not fully understood. Clinical manifestations include autonomic dysfunction, peripheral neuropathy, and central nervous system symptoms. Electrodiagnostic studies aid in diagnosis. Early diagnosis and treatment are important to prevent complications.
Keywords
Porphyric neuropathy
neurological manifestation
acute hepatic porphyrias
axonal neuropathy
diagnosis
urine sample
treatment
intravenous heme
trigger medications
complications
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