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

Berislav Perić

Niko Krakar

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Acta Stomatol Croat, Vol. 39, br. 3, 2005. 311A S CIntroductionThe term porphyria encompasses a group of dis-eases of uncharacteristic symptomatology which all exhibit excessive excretion of one or more porphy-rins, porphyrinogens and/or porphyrin precursors in the urine and faeces (1).The incidence of porphyria ranges between 1: 10 000 and 1:50 000 (1). Four exogenous factors - drugs, steroids, hunger and infection - can induce transition from the latent form of the disease into the acute form (3). Thus it is important for the dentist to know which drugs can cause an attack of porphyria, and those which can be applied safely.PathophysiologyPorphyrias are a group of diseases caused by defective enzymes on the biosynthetic pathway of heme (4).Heme, a pigment which contains iron, is a non-protein active ingredient of haemoproteins which are present in all tissues. Heme is mainly formed in bone marrow, where it synthesises in haemoglobin and in the liver, where it synthesises in cytochromes, of which the most important is cytochrome P-450 which most frequently participants in drug metabo-lism (5).Heme biosynthesis occurs with the assistance of eight different enzymes which catalyse individual Dental Treatment of the Patient with PorphyriaBerislav Perić1Niko Krakar21Clinical Department of Oral Surgery University Hospital “Dubrava”, Zagreb2General Hospital  “Dr. Ivo Pedišić”, SisakActa Stomat Croat2005; 311-314REVIEWReceived: May 5, 2004Address for correspondence:Berislav PerićClinical Department of Oral SurgeryUniversity Hospital “Dubrava”Av. G. Šuška 6, 10040 ZagrebCroatiae-mail:  berislav.peric@t-com.zg.hrSummaryPorphyria is a group of inherited diseases caused by defective en-zymes 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 be-cause of the effect of these compounds/products on the nervous system and skin.  Numerous drugs, such as local anaesthetics (lidocaine), an-tibiotics (clindamycin, erythromycin, metronidazole) and others, can be precipitating factors and cause acute attacks. Patients with porphyria can safely be given bupivacaine, amoxycillin, clavulanic acid, acethyl-salicilic 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.Key words:  porphyria, drugs.B. Perić et al. Dental Treatment of PorphyriaActa Stomatol Croat, Vol. 39, br. 3, 2005.312  A S Csteps. Depending on the specific enzyme defect dif-ferent types of porphyria can be differentiated. In all porphyrias there is a raised level of synthetase del-taaminolevulinic acid (ALA-synthetase), of the first enzyme on the biosynthetic pathway of heme (4). Heme inhibits further formation of ALA-synthetase (1). Certain drugs and hormones stimulate hepato-cytes to increase the formation of ALA synthetase, heme and cytochromes (P-450 (3).The consequence of the enzyme defect is the formation of abnormal amounts of precursor hemes (porphyrins) which accumulate in the tissues and are excreted in the urine and faeces (4). Clinical signs nearly always occur from the effect of these com-pounds on the nervous system and skin.Classification of porphyriaAccording to the tissue in which porphyrins accumulate, porphyrias can be classified as eryth-ropoietic, in which porphyrins accumulate in bone marrow, and hepatic, in which porphyrins accumu-late in the liver. The erythropoietic group includes: congenital erythropoietic porphyria, erythropoietic protoporphyria and X-linked siderblastic anaemia. The hepatic group includes: acute intermittent por-phyria, cutanea tarda porphyria, hereditary cop-roporphyria, variegate porphyria and porphyria defect ALA dehydratase.A third group represents hepatoerythropoietic porphyrias which combine erythropoietic and hepat-ic porphyria (1, 6).The most frequent forms of porphyria are:• Acute intermittent porphyria (AIP).• Late skin porphyria.• Hereditary coproporphyria• Variegate porphyria (South African genetic por-phyria) (1, 4, 6).Acute intermittent porphyriaFor the dentist the most important, and at the same time the most frequent acute porphyria, is acute intermittent porphyria (AIP).AIP is a hepatic porphyria. The prevalence of AIP in the world ranges from around 1:1000-10 000, with greater frequency in Scandinavian countries and Great Britain (3, 6). It manifests after puberty and is most frequent in women. Drugs are usual-ly the precipitating factor. The majority of harmful drugs in acute porphyria induce hepatic ALA syn-thetase and enzyme cytochrome P-450. Other dis-eases, surgical operations, stress, menstrual cycle (second half) pregnancy, psychological problems, alcohol have also been reported to be precipitating factors.Attacks develop over several hours or days and last from several days to several weeks. Symp-toms arise as a result of damage to visceral nerves. Most frequently they include abdominal pain, nau-sea, vomiting, diarrhea and opstipation. The uri-nary bladder may be involved with urine retention, incontinence, dysurea and polakysurea. Tachycardia is usual with hypertension, perspiration and restless-ness, caused by damage to the autonomic nervous system or high level of catecholamines in the blood. Motor neuropathy often occurs. Muscular weakness usually starts in the shoulders and arms, although it can involve any neurone, including the cerebral nerves. Severe paralysis and respiratory insufficien-cy may develop. Acute attacks carry a significant risk of death due to respiratory paralysis in 10-25% of patients (7).Acute attacks require hospitalisation and infu-sions of glucose and i.v. administration of heme, which suppresses synthesis of the enzyme ALA syn-thetase by a feedback mechanism (3-5, 8, 9).Late skin porphyria (Porphyria cutanea tarda) is the most frequent of all porphyrias. However, it is characterised only by skin symptoms, photosensi-tivity and the occurrence of bullae. Alcohol, iron and estrogens are reported to be precipitating fac-tors (6).Hereditary coproporphyria is a hepatic porphyr-ia. Acute attacks cannot be differentiated from those of AIP, and the same therapy is carried out.Variegate porphyria is characterised by acute episodes of neurological and psychic disorders, with sensitivity of the skin to sun and also to mechanical stimulation. It is treated as for AIP.Acta Stomatol Croat, Vol. 39, br. 3, 2005. 313 A S CB. Perić et al. Dental Treatment of PorphyriaDrugs and porphyriasWhen confronted with a patient suffering from an acute form of porphyria it is extremely impor-tant for the dentist to be aware of the drugs which are known to be precipitating factors. Many lists have been published on drugs which are classified as harmful or safe for application in those suffering from AIP (7, 10-12). Such lists are similar in many ways, due to the fact that they are compiled on the basis of in vitro study, experimental animal studies, case presentations and structural similarities with drugs that are known to be harmful to those suf-fering from AIP (e.g. new barbiturates or sulphon-amides are considered harmful) (3.10). In the first case a false-positive result is often possible. The drawback of the second is the difference in physi-ological processes of the human and animal organ-ism, and the third case is founded on anecdotal evi-dence (3). Thus, according to Moore, it is likely that all drugs which are marked as “safe” for application in porphyria are safe, while those which are marked “unsafe” are not necessarily so, but should be used with caution (10).The harmlessness of local anaesthetics remains a controversial subject because of experimental evi-dence of the porphyriopathy of local anaesthetics tested on animals (lidocaine) and cell cultures (lido-caine, mepivacaine, bupivacaine) (10-12). For exam-ple, Nordman et al reports that prilocaine, lidocaine, bupivacaine and mepivacaine are “dangerous”, and procaine “safe” (11). Moore reports that lidocaine is unsafe, and bupivacaine, procaine and prilocaine are safe (10). Moore and McColl report that mepi-vacaine is unsafe, prilocaine questionable, and bupi-vacaine and procaine safe (7). Nevertheless, clini-cal experience has shown that these medications can be applied to patients with acute porphyrias with no noticeable side-effects.It is recommended that bupivacaine is used for infiltrational and conductive anaesthesia (0.25-0.5%) with adrenaline (1:200 000), and for topical lidocaine (gel 2%, spray 4-10%) (13).Penicillin antibiotics can be used (penicillin G, penicillin V, amoxycillin, clavulanic acid), as opposed to clindamycin, erythromycin, metronida-zole (2). However, situations have been described in which clindamycin was applied as a “safe drug” because it had previously been used in the patient with no negative consequence with regard to induc-tion of AIP (5).Of the analgesics AS acid, paracetamol and ibu-profen can safely be applied.Corticosteroids, which are used in the treatment of certain oral conditions such as lichen, recurrent aphthous stomatitis and pemphigus, can induce AIP (14).Of other therapeutic factors the following should be mentioned: ethanol which is used as a vehiku-lum in solutions for mouth rinsing, carbamazepine which is used in the therapy of neuralgia n. trigemi-nus, and chlorzoxazone, a muscle relaxant of central effect, which can also induce AIP (2, 3).Table 1 gives a comprehensive list of drugs which are of practical importance for the dentist, ensuring safe use in patients with acute forms of porphyria (2).ConclusionTreatment of patients with acute forms of por-phyria represents a challenge for the dentist. With this in mind we give below general guidelines for carrying out safe and successful therapy in such patients.1. While talking to the patient the dentist should ask about the frequency of acute episodes and when the last attack occurred. What factors induced it and whether there are prodromal symptoms which precede acute episodes. In what way were such attacks treated and was hospitalisation nec-essary?2. The dentist should determine the name of the physician treating the disease and briefly discuss with him/her the plan for dental therapy. It is necessary to decide which anaesthetic, analge-sic, antibiotic and sedative can be included in the therapy and to discuss emergency therapy protocol in the case of an acute attack.3. The dentist should inform the patient about the harmfulness of strong carbohydrate diets, which are prescribed for such patients, oral health and taking relevant contra-measures with regard to increased hygiene, mouth rins-B. Perić et al. Dental Treatment of PorphyriaActa Stomatol Croat, Vol. 39, br. 3, 2005.314  A S Cing with chlorzoxazone solution (which does not contain ethanol) and frequent check-ups (every 3-6 months).4. In view of the history data the dentist should decide whether to refer the patient for treatment in an appropriate specialist institution (5).

Dental Treatment of the Patient with Porphyria

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Acta Stomatol Croat, Vol. 39, br. 3, 2005. 307A S CUvodNazivak porfirija obuhvaća skupinu bolesti neka-rakteristične simptomatologije kojima je zajednič-ka prekomjerna ekskrecija jednoga ili više porfina, porfirogena i/ili porfinskih prekursora u urinu i u stolici (1).Čestoća porfirije kreće se između 1 na 10 000 i 1: 50 000 (1). Četiri egzogena čimbenika - lijeko-vi, steroidi, gladovanje i infekcija - mogu potaknu-ti prijelaz iz latentnog oblika bolesti u akutan (3). Zbog toga je stomatologu važno poznavati lijekove koji bi mogli uzrokovati napad porfirije, i one koje možemo nesmetano upotrijebiti.PatofiziologijaPorfirije su skupina bolesti uzrokovanih defekt-nim enzimima na biosintetskom putu hema (4).Hem, pigment koji sadrži željezo, neprotein-ski je djelatni sastojak hemoproteina koji postoji u svim tkivima. Hem se najviše stvara u koštanoj srži, gdje se ugrađuje u hemoglobin, te u jetrima, gdje se ugrađuje u citokrome. Među njima je najvažni-ji citokrom P-450 koji najčešće sudjeluje u metabo-lizmu lijekova (5).Biosinteza hema odvija se uz pomoć osam razli-čitih enzima koji kataliziraju pojedine pomake. Ovi-sno o defektu pojedinog enzima razlikujemo i razne vrste porfirija. Zajedničko svim porfirijama jest porast razine sintetaze deltaaminolevulinske kiseli-ne (ALA-sintetaze), prvog enzima na biosintetsko-Stomatološki pristup bolesniku s porfirijomBerislav Perić1Niko Krakar21Klinički zavod za oralnu kirurgiju Kliničke bolnice “Dubrava”, Zagreb2Opća bolnica  “Dr. Ivo Pedišić”, SisakActa Stomat Croat2005; 307-310PREGLEDNI RADPrimljeno: 5. svibnja 2004.Adresa za dopisivanje:Berislav PerićE-mail:  berislav.peric@zg.htnet.hrSažetakPorfirije su skupina nasljednih bolesti uzrokovanih defektnim enzi-mima na biosintetskom putu hema. Ovisno o defektu pojedinog enzima razlikujemo i razne vrste porfirija. Posljedica defekta enzima jest stva-ranje abnormalnih količina prekursora hema (porfirina) koji se naku-pljaju 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, eri-tromicin, metronidazol) i dr. spadaju u precipitirajuće čimbenike i mo-gu 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 lije-čenja pacijenta s nekim akutnim oblikom porfirije.Ključne riječi: porfirija, lijekovi.B. Perić i sur. Stomatološki pristup bolesniku s porfirijomActa Stomatol Croat, Vol. 39, br. 3, 2005.308  A S Cme putu hema (4). Hem koči daljnje stvaranje ALA-sintetaze (1). Neki lijekovi i hormoni potiču hepa-tocite na povećano stvaranje ALA sintetaze, hema i citokroma P-450 (3). Posljedica defekta enzima jest stvaranje abnor-malnih količina prekursora hema (porfirina). Oni se nakupljaju u tkivima, a izlučuju mokraćom i stoli-com (4). Klinički znaci gotovo potpuno proizlaze iz učinka tih spojeva na živčani sustav i kožu.Podjela porfirijaPorfirije se mogu podijeliti prema tkivu u koje-mu se nagomilavaju porfirini na eritropoetske, u kojima se porfirini nagomilavaju u koštanoj srži, i na hepatičke, u kojima se porfirini nakupljaju u jetri-ma. U eritropoetske se ubrajaju: kongenitalna eri-tropoetska porfirija, eritropoetinska protoporfiri-ja i X-vezana sideroblastična anemija. U hepatičke spadaju: akutna intermitentna porfirija, kasna kož-na porfirija, hereditarna koproporfirija, varijegatna porfirija i porfirija defekta ALA dehidrataze.Treću skupinu čine hepatoeritropoetske porfirije koje su kombinacija eritropoetske i hepatičke por-firije (1, 6).Češći oblici porfirija jesu:• akutna intermitentna porfirija (AIP),• kasna kožna porfirija,• hereditarna koproporfirija i• porfirija varijegata (južnoafrička genetička por-firija) (1, 4, 6).Akutna intermitentna porfirija Za stomatologe najvažnija a ujedno i najčešća porfirija jest akutna intermitentna porfirija (AIP).AIP se ubraja u hepatičke porfirije. Prevalenci-ja AIP-a u svijetu kreće se oko 1 na 1000-10 000, s većom čestoćom u skandinavskim zemljama i u Velikoj Britaniji (3, 6). Ona se manifestira nakon puberteta i češća je u žena. Lijekovi su najčešći precipitirajući čimbenik. Većina škodljivih lijekova kod akutnih porfirija inducira jetrenu ALA sintetazu i enzime citokroma P-450. Kao precipitirajući čim-benici navode se i druge bolesti, kirurški zahvati, stres, menstrualni ciklus (druga polovica), trudnoća, psihološki problemi, uživanje alkohola.Napadaji se razvijaju kroz nekoliko sati ili dana i traju od nekoliko dana do nekoliko tjedana. Simpto-mi nastaju kao posljedica oštećenja visceralnih živa-ca. Najčešća je bol u trbuhu, mučnina, povraćanje, proljev ili opstipacija. Može biti zahvaćen i mokrać-ni mjehur, pa se javljaju retencija urina, inkontinen-cija, disurija i polakisurija. Obično se zapažaju i tahikardija, hipertenzija, znojenje i nemir, koji proi-zlaze iz oštećenja autonomnoga živčanog sustava ili visokih razina katekolamina u krvi. Česta je i motor-na neuropatija. Mišićna slabost većinom počinje u ramenima i u rukama, no zna zahvatiti bilo koje neu-rone, uključujući i moždane živce. Može se razviti teška paraliza i respiratorna insuficijencija. Akutni napadi još nose znatan rizik od smrtnosti zbog res-piratorne paralize u 10-25% pacijenata (6).Akutni napadi zahtijevaju hospitalizaciju i infu-zije glukoze i i.v. davanje hema koji mehanizmom povratne sprege suzbija sintezu enzima ALA sinte-taze (3-5, 8, 9).Kasna kožna porfirija (Porphiria cutanea tar-da) najčešća je od svih porfirija, no karakterizirana je samo kožnim simptomima, fotosenzitivnošću te nastankom bula. Kao precipitirajući čimbenici navo-de se alkohol, željezo i estrogeni (6).Hereditarna koproporfirija ubraja se u hepatičke porfirije. Akutne napadaje nije moguće razlikovati od onih u AIP-a, a provodi se i jednaka terapija.Varijegatnu porfiriju karakteriziraju akutne epi-zode neuroloških i psihijatrijskih poremećaja, a uz njih postoji i osjetljivost kože na sunce, ali i na mehaničke podražaje. Liječi se jednako kao i AIP.Lijekovi i porfirijeStomatologu kojega posjeti bolesnik s nekom od akutnih oblika porfirija najvažnije je poznava-ti lijekove koji spadaju u precipitirajuće čimbeni-ke. Publicirano je mnogo lista lijekova klasificira-nih kao štetnih ili pak sigurnih za uporabu u obolje-lih od AIP-a (7, 10-12). Navedene liste imaju mno-go nepodudarnosti. Razlog tomu je što su naprav-ljene na temelju studija in vitro, eksperimentalnih studija na životinjama, prikaza slučaja te struktur-nih sličnosti s lijekovima koji su poznati kao štetni Acta Stomatol Croat, Vol. 39, br. 3, 2005. 309 A S CB. Perić i sur. Stomatološki pristup bolesniku s porfirijomza oboljele od AIP-a (npr. novi barbiturati ili sulfo-namidi smatrali bi se štetnima) (3, 10). U prvome slučaju često je moguć lažno-pozitivni rezultat. Dru-goj metodi je mana razlika u fiziološkim procesima ljudskog i životinjskog organizma, a treća se meto-da temelji na anegdotalnim dokazima (3). Zato je, prema Moore-u, vjerojatno da su svi lijekovi koji su označeni ‘sigurnima’ za uporabu kod porfirija uisti-nu sigurni, a oni koji su označeni ‘nesigurnima’ to ne moraju biti, no potrebno ih je upotrebljavati s oprezom (10).Neškodljivost lokalnih anestetika ostaje i dalje sporna tema s obzirom na eksperimentalne dokaze o porfirogenosti lokalnih anestetika ispitivanih na živo-tinjama (lidokain) i staničnim kulturama (lidokain, mepivakain, bupivakain) (10, 12). Na primjer, Nor-Lokalni anestetici / Local anaesthetics Antimikotici / AntifungalsBupivakain / Bupivacaine U Amfotericin / Amphotericin USOLidokain / Lidocaine U Flukonazol / Fluconazole USIOMepivakain / Mepivacaine USO Griseofulvin IProkain / Procaine U Ketokonazol / Ketoconazole ITetrakain / Tetracaine U Antivirusni lijekovi / AntiviralsAnalgetici i antireumatici /  Analgesics and anti-inflammatoriesAciklovir / Aciclovir UAntihistaminici / AntihistaminesAspirin U Epinefrin / Epinephrine UIbuprofen U Kortikosteroidi / CorticosteroidsParacetamol U Deksametazon / Dexamethasone UAntibiotici / Antibiotics Sedativi i hipnotici / Sedatives and hypnoticsAmoksicilin / Amoxycillin U Diazepam USOAmpicilin / Ampicillin U Lorazepam UBenzilpenicilin / Benzylpenicillin U Oksazepam / Oxazepam USOKlavulanska kiselina / Clavulanic acid U Antikonvulzivi / EpilepsyKloksacilin / Cloxacillin U Karbomazepin / Carbamazepine IPhenoxymethylpenicillin U Miorelaksansi / Central muscle relaxantsPivampicillin USIO Klorzoksazon / Chlorzoxazone ICefaleksin / Cefalexin USO Ostalo / MiscellaneousKlindamicin / Clindamycin I Fluoridi UEritromicin / Erythromycin I Glukoza UTetraciklin / Tetracycline USIO Imunoglobulini UMetronidazol / Metronidazole USIO Vitamini UTablica 1. Kategorizacija lijekovaTable 1. Categorization of the drugsLegenda / Legend:U - Upotrebljavati / UseUSO - Upotrebljavati s oprezom / Use with cautionUSIO - Upotrebljavati s iznimnim oprezom / Use with extreme cautionI - Izbjegavati / Avoiddman i sur. navode prilokain, lidokain, bupivakain i mepivakain kao ‘opasne’, a prokain kao ‘siguran’ (11). Moore navodi lidokain kao ‘nesiguran’, a bupi-vakain, prokain i prilokain kao ‘sigurne’ (10). Moo-re i McColl označavaju mepivakain kao ‘nesiguran’, a prilokain kao ‘upitan’, a bupivakain i prokain kao ‘sigurne’ (7). No unatoč tome klinička iskustva poka-zuju da su se ta sredstva upotrebljavala u pacijenata s akutnim porfirijama bez opaženih nuspojava.U Tablici 1 dan je širi popis lijekova, koji su od praktičnoga značenja za stomatologe i sigurnost nji-hove uporabe u pacijenata s akutnim oblicima por-firije (2).Preporuka je da se za infiltracijsku i provodnu anesteziju rabi bupivakain (0,25-0,5%) s adrenali-nom (1:200 000), a za topikalnu lidokain (gel 2%, spray 4-10%) (13).B. Perić i sur. Stomatološki pristup bolesniku s porfirijomActa Stomatol Croat, Vol. 39, br. 3, 2005.310  A S CPenicilinski antibiotici mogu se rabiti (Penicilin G, Penicilin V, amoksicilin, klavulanska kiselina), za razliku od klindamicina, eritromicina, metroni-dazola (2). Unatoč tome opisan je i slučaj da se je klindamicin upotrebljavao kao ‘siguran lijek’ jer se i prije davao pacijentu bez posljedica u smislu indu-ciranja AIP (5). Od analgetika sigurna je uporaba acetilsalicilne kiseline, paracetamola i ibuprofena.Kortikosteroidi koji se upotrebljavaju u liječe-nju nekih oralnih stanja, kao što su lihen, rekuren-tni aftozni stomatitis i pemfigus, mogu inducirati AIP (14).Od ostalih terapijskih čimbenika treba spomenu-ti etanol koji se rabi kao vehikulum u otopinama za ispiranje usta, karbamazepin koji se rabi u terapi-ji neuralgija n. trigeminusa i klorzoksazon, mišični relaksans središnjega djelovanja koji također mogu potaknuti AIP (2, 3).ZaključakTretiranje pacijenata s akutnim formama porfi-rija izazov je za stomatologa. U skladu s tim daje-mo opće smjernice da bi se nad takvim pacijentima mogla provesti sigurna i uspješna terapija.1. U razgovoru s pacijentom informirati se o čestoći akutnih epizoda i kada je bio zadnji napadaj, koji su čimbenici koji ga iniciraju te postoje li pro-dromalni simptomi koji prethode akutnim epizo-dama. Na koji su se način do sada takvi napadi liječili i je li bila potrebna hospitalizacija? 2. Saznati koji liječnik primarno liječi bolest i s njim kratko raspraviti o planu stomatološke terapije. Potrebno je izložiti koje je anestetike, analgetike, antibiotike i sedative moguće uključiti u terapiju te raspraviti o hitnom terapijskom protokolu u slučaju akutnoga napadaja. 3. Stomatolog treba obavijestiti pacijenta o štet-nosti za oralno zdravlje jakih ugljikohidratnih dijeta, koje se prepisuju takvim pacijentima, te o potrebnim protumjerama kao što su pojačana higijena, ispiranje usta otopinom klorheksidina (koja ne sadrži etanol) i češći kontrolni pregledi (svakih 3-6 mjeseci). 4. S obzirom na sve anamnestičke podatke, stoma-tolog treba odlučiti hoće li uputiti pacijenta na liječenje u specijalističku ustanovu (5). Literatura1. VRHOVAC B i sur. Interna medicina. Zagreb: Naklada Lje-vak, 2003: 1326-31.2. WEB:UCT:AC:ZA/DEPTS/PORPHYRIA. 2004.3. BROOWN RS, HAYS GL, JEANSONNE MJ, LUSK SS. The managment of a dental abscess in a patient with acute intermittent porphyria. A case report. Oral Surg Oral Med Oral Pathol 1992; 575-8.4. BROWN GJ, WELBURY RR. The management of por-phyria in dental practice. Br Dent J 2002; 193: 145-6. 5. MOORRE AW 3RD, COKE JM. Acute porphyric disor-ders. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2000; 90: 257-62. 6. HARRISON’S. Principi interne medicine. Split: Placebo, 1997.7. MOORE MR, McCOLL KEL. Therapy of the acute por-phyrias. Clin Biochem 1989; 22: 181-8. 8. ROSE L, KAYE D. Internal medicine for dentistry. 2 nd ed. St. Louis: CV Mosby 1990; 1102-6. 9. HRVATSKO DRUŠTVO FIZIOLOGA. Patofiziologija. Zagreb: Jumena 1990: 234-5. 10. MOORE MR. International review of drugs in acute por-phyria. Int J Biochem 1980;1089-97.11. NORDMANN Y, FOGIEL JM, DEYBACH JC. Porphyries hepatiques en odonto-stomatologie. Actual Odontostomatol 1988;183-9.12. DISLER PB, MOORE MR. Drug therapy in acute porphyr-ias. Clin Dermatol 1985;112-24.13. WWW.PORPHYRIA-EUROPE.COM 14. TSCHUDY DP, VALSAMIS M, MAGNUSSEN CR. Acute intermittent porphyria: clinical and selected research aspects. Ann Intern Med 1975; 83: 851-64.

Dental Treatment of the Patient with Porphyria

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