What is the pathophysiology behind Acute Intermittent Porphy
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Prachi Praveen
What is the pathophysiology behind Acute Intermittent Porphyria leading to seizures in patients?
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Dr. K●●●●●p S●●●h
Dr. K●●●●●p S●●●h Internal Medicine
AIP is an autosomal dominant disease that results from defects in the enzyme porphobilinogen-deaminase. This enzyme speeds the conversion of porphobilinogen to hydroxymethylbilane. In AIP, the porphyrin precursors, porphobilinogen and amino-levulinic acid (ALA), accumulate. The predominant problem appears to be neurologic damage that leads to peripheral and autonomic neuropathies and psychiatric manifestations. [2] Although levels of porphobilinogen and ALA are always elevated during acute attacks, how this leads to the symptomatic disease is still unclear because most patients with the genetic defect have excessive porphyrin secretion but no symptoms. ... Read more
Oct 30, 2020Like1
P●●●●i P●●●●●n
P●●●●i P●●●●●n General Medicine
Thank you
Nov 10, 2020Like