Porphyrias: a group of inherited or acquired metabolic disorders involving heme biosynthesis (1,2)
Symptoms mediated by overproduction of porphyrins or precursors in heme biosyntheti...
Symptoms of acute porphyria attacks (1,2)
Psychiatric: depression, disorientation, psychosis
Gastrointestinal: abdominal pain (constant, poorly localized), vomiting, constipation
Neurol...
Neuropsychiatric: avoidance of precipitating factors (e.g., alcohol or o...
Acute porphyria attack: inpatient monitoring as above
Preoperative considerations (12)[C]
Careful consideration of all anesthetic choices to avoi...
Howard M, Hall A, Ramsay R. Congenital erythropoietic porphyria (Gunther disease)—long-term follow up of a case and review. Dermatol Online J. 20...
E80.20 Unspecified porphyria
E80.0 Hereditary erythropoietic porphyria
E80.21 Acute intermittent (hepatic) porphyria
E80.29 Other porphyria
E80.2 Other and unspecified porphyria
E80.1 Porphyria ...
Symptoms of acute porphyria classically include severe nonfocal gastrointestinal pain and neuropsychiatric disturbances.
Mainstays of treatment for acute porphyria attacks include oral ...
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Porphyria cutanea tarda: hands
<bold>Fig GI 22-5 Acute intermittent porphyria.</bold> Adynamic ileus simulating mechanical obstruction on (A) supine and (B) upright views.
<bold>Fig GI 22-5 Acute intermittent porphyria.</bold> Adynamic ileus simulating mechanical obstruction on (A) supine and (B) ...
<bold>FIGURE 2.13</bold> The heme biosynthetic pathway. Inherited defects of each of the heme biosynthetic enzymes except <i>δ</i>-aminolevulinic acid synthase have been described and lead to the clinical disorders known as the porphyrias.
<bold>FIGURE 2.13</bold> The heme biosynthetic pathway. Inherited defects of each of the heme biosynthetic enzymes except <...