Dental Treatment of the Patient with Porphyria

Porfirije su skupina nasljednih bolesti uzrokovanih defektnim enzimima na biosintetskom putu hema. Ovisno o defektu pojedinog enzima razlikujemo i razne vrste porfirija. Posljedica defekta enzima jest stvaranje abnormalnih količina prekursora hema (porfirina) koji se nakupljaju u tkivima i izlučuju mokraćom i stolicom. Klinički znaci gotovo potpuno proizlaze iz učinka tih spojeva na živčani sustav i kožu. Mnogi lijekovi, kao lokalni anestetici (lidokain), antibiotici (klindamicin, eritromicin, metronidazol) i dr. spadaju u precipitirajuće čimbenike i mogu uzrokovati akutne napade. Pacijentima s porfirijom sa sigurnošću se mogu dati bupivakain, amoksicilin, klavulanska kiselina, acetilsalicilna kiselina, paracetamol (1,2). Zato je stomatologu važno imati određene smjernice u slučaju liječenja pacijenta s nekim akutnim oblikom porfirije.Porphyria is a group of inherited diseases caused by defective enzymes on the biosynthetic pathway of heme. Depending on the specific enzyme defect different forms of porphyria can be differentiated. The consequence of the enzyme defect is the formation of abnormal amounts of porphyrins or precursors which accumulate in the tissues and are excreted in the urine and faeces. Almost all the clinical signs occur because of the effect of these compounds/products on the nervous system and skin. Numerous drugs, such as local anaesthetics (lidocaine), antibiotics (clindamycin, erythromycin, metronidazole) and others, can be precipitating factors and cause acute attacks. Patients with porphyria can safely be given bupivacaine, amoxycillin, clavulanic acid, acethylsalicilic acid and paracetamol (1, 2). Thus it is important for the dentist to have certain guidelines with regard to the treatment of patients with acute form of porphyria