Learning objectives Describe porphyria Recognize the symptoms and signs of porphyria Anesthetic management of a patient with porphyria Definition and mechanisms Porphyrias are a heterogeneous group of inherited genetic disorders of heme biosynthesis The heme biosynthetic pathway is most active in the liver and bone marrow Porphyrins are organic cyclical compounds found in heme, the iron-containing ring structure found in hemoglobin, myoglobin, and all of the cytochromes Classification Classification Acute porphyrias: Potential to develop acute neurovisceral crises Acute intermittent porphyria (AIP) Variegate porphyria (VP) Hereditary coproporphyria (HCP) 5-aminolaevulinic acid (ALA) dehydrase deficiency Triggers for an acute crisis Fasting Dehydration Infection Drugs Endogenous hormones Stress Smoking Alcohol Non-acute: Do not deteriorate into acute crises, less relevant for anesthesiologists Porphyria cutanea tarda Congenital erythropoietic porphyria Erythropoietic protoporphyria Signs and symptoms Presentation of an acute crisis Almost all patients have severe abdominal pain, usually associated with tachycardia Symptoms and signs of acute crises vary greatly and can mimic other conditions Risk factors Women are 4 to 5 times more likely to develop crises in their early thirties Pathophysiology Treatment Once an acute crisis has been diagnosed, management consists of the following: Remove or treat potential triggering factors and avoid a catabolic state Administration of i.v
In the context of diabetes models, it may contribute to heart attack, impaired wound healing, stroke, and kidney damage
Severe neonatal RDS is associated with reduced pulmonary and CBMC expression of GR, indicating significant dysregulation of this receptor during the progression of the disease
Myeloperoxidase catalyzes the conjugation of serotonin to thiols via free radicals and tryptamine-4,5-dione
