For several days, a 28 y/o male has been complaining to co-workers about being e
ID: 3481998 • Letter: F
Question
For several days, a 28 y/o male has been complaining to co-workers about being extremely tired and nauseous in the morning. His friends note that he is pale, although his cheeks appear like he’s been out in the cold. He and his colleagues assume that it is from too much partying since he has been attending a lot parties in the week prior to Halloween. By midmorning, he begins to feel better and he vows to stay in that night and go to bed early. The next morning, he is found by neighbors, comatose and convulsing.
What is the cause of the Px's condition and why would simply giving the Px oxygen not immediately be sufficient?
Explanation / Answer
The diagnosis here may be
1) Acute intermittent porphyria attack - in which there is defective heme synthesis and accumulation of intermediates of heme synthesis leading to symptoms.(the porphyrias can be classified as either hepatic or erythropoietic, depending on whether the heme biosynthetic intermediates that accumulate arise initially from the liver or developing erythrocytes, or as acute or cutaneous, based on their clinical manifestations. Four of the five hepatic porphyrias—acute intermittent porphyria (AIP), hereditary coproporphyria (HCP), variegate porphyria (VP), and ALA-dehydratase porphyria (ADP)—present during adult life with acute attacks of neurologic manifestations and elevated levels of one or both of the porphyrin precursors, aminolevulinic acid and porphobilinogen, and are thus classified as acute porphyrias.)
treatment : During acute attacks,phenothiazines are useful for nausea, vomiting, anxiety, and restlessness.Carbohydrate loading, usually with intravenous glucose (at least 300 g/dL of 10% dextrose in water daily), may be effective in milder acute attacks of porphyria. Hemin should be used initially for severe attacks and for mild attacks that do not respond to carbohydrate loading within 1–2 days. The standard regimen is 3–4 mg/kg of heme, in the form of lyophilized hematin, heme albumin , or heme arginate, infused daily for 4 days.
As the patient has defective heme synthesis(anemia like state),O2 therapy only is not sufficient in acute attack.