DefinitionThis section has been translated automatically.
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.
ClassificationThis section has been translated automatically.
- Acute hepatic porphyrias:
- Porphyria acuta intermittens (PAI)
- Porphyria variegata (PV)
- Coproporphyria hereditaria (HC).
- Chronic hepatic porphyrias:
- Porphyria cutanea tarda (PCT).
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DiagnosisThis section has been translated automatically.
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)This section has been translated automatically.
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.
LiteratureThis section has been translated automatically.
- 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
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Incoming links (7)
Coproporphyria hereditaria; Coproporphyria symptomatica; Hydroa vacciniforme; Porphyria cutanea tarda; Sulfadiazine silver; Sulfasalazine; Sulfonamides;Outgoing links (20)
Acetylsalicylic acid; Acute intermittent porphyria; Cephalosporins; Chloroquine; Cimetidine; Coproporphyria hereditaria; Dadps; Dexamethasone; Griseofulvin; Heparin; ... Show allDisclaimer
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Prof. Dr. med. Peter Altmeyer