Category: AIP

Porphyrinogenic drugs, acute porphyria, hormonal status and glucose metabolism

A PubMed study by Matkovic LB1, D’Andrea F, Fornes D, San Martín de Viale LC, Mazzetti MB.

Abstract

This work deals with the study of how porphyrinogenic drugs modeling acute porphyrias interfere with the status of carbohydrate-regulating hormones in relation to key glucose enzymes and to porphyria, considering that glucose modulates the development of the disease. … Thus, porphyria-inducing drugs AIA and DDC dramatically altered the status of hormones that regulate carbohydrate metabolism increasing insulin levels and reducing GC production, metabolization and plasmatic levels.

In this acute porphyria model, gluconeogenic and glycogenolytic blockages caused by PEPCK and GP depressed activities, respectively, would be mainly a consequence of the negative regulatory action of insulin on these enzymes. GC could also contribute to PEPCK blockage both because they were depressed by the treatment and because they are positive effectors on PEPCK. These disturbances in carbohydrates and their regulation, through ALA-S de-repression, would enhance the porphyria state promoted by the drugs on heme synthesis and destruction. This might be the mechanism underlying the “glucose effect” observed in hepatic porphyrias. The statistical correlation study performed showed association between all the variables studied and reinforce these conclusions.

 

PMID: 21889565   DOI: 10.1016/j.tox.2011.08.014   Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.
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Acute intermittent porphyria -diagnostic and treatment traps

A PubMed case study by Cojocaru IM, Sapira V, Socoliuc G, Hertea C, Balea M, Ursache C, Cojocaru M.

Abstract

Acute intermittent porphyria (AIP) is a rare metabolic disease defined by mutations coding the deaminaze enzyme of porphobilinogen (PBGD). Porphyrias are somewhat misdiagnosed as a consequence of light symptoms in patients. Acute forms of porphyria can be life-threatening, so a correct diagnosis and an accurate treatment are highly important. The authors presented the case of a 38-years-old patient admitted for persistent abdominal pain that previously presented two generalized convulsive seizures. The diagnosis of AIP was established by the raised concentration of urinary porphyrins. Despite treatment with carbohydrates and hemines, the clinical picture of the patient worsened, with tetraplegia and severe respiratory failure. The patient died seven weeks after the initial presentation of the disease.

PMID: 22788092