Porphyria hepatic - Altmeyers Encyclopedia

Definition
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Group of porphyrin diseases, which are based on a disturbance of the liver cell function (multiple or malformation of porphyrins). The term "acute hepatic porphyria" is used to describe the potentially intermittent acute form. Acute hepatic porphyria may currently be in a clinically manifest phase, see also porphyria syndrome, acute. After spontaneous remission or therapy, the porphyria process is in a subclinical phase (latency phase). The stability of the latency phase depends on prophylactic measures, especially on avoiding porphyrinogenic provocation factors.

Classification
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Acute hepatic porphyrias:
- Porphyria acuta intermittens (PAI)
- Porphyria variegata (PV)
- Coproporphyria hereditaria (HC).
Chronic hepatic porphyrias:
- Porphyria cutanea tarda (PCT).

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Diagnosis
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The diagnosis of acute hepatic porphyria is confirmed by:

Determination of the two porphyrin precursors: delta-aminolevulinic acid, porphobilinogen.
Determination of porphyrins in urine.

Note(s)
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Main Prohibited and Allowed Medicines and Agents for Acute Hepatic Porphyria:

Prohibited (known as porphyria triggers):
- Antidiabetics, antihypertensives (clonidine, hydralazine, sprinolocaton, methyldopa), antiphlogistics (especially phenylbutazone, pyrazolone derivatives, ibuprofen), anticonvulsants (carbamazepine, hydantoins, primidone), antirheumatic drugs (diclofenac)
- Barbiturates, chloroquine and derivatives, griseofulvin, dapsone, diazepam, diazeptics (halothane), nitrofurantoin, ranitidine, steroid hormones (estrogens and progesterone), sulphonamides, theophylline, alcohol.
Prohibited (allegedly not porphyria-inducing potency):
- Acetylsalicylic acid, narcotics (halothane), salicylic acid, cephalosporins, dexamethasone, cimetidine, digitalis preparations, fentanyl, flunitrazepam, heparin, penicillins, prednisolone, procaine, promethazine, reserpine, tetracyclines.

Literature
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Doss MO et al. (2003) Arneistoistofficants in acute hepatic porphyria and recommendations for anesthesia. In: Red List 2003, online version, http://www.rote-liste.de/Online/texte/anhang/porhyrien.html