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Acute Intermittent Porphyria Treatment & Management

  • Author: Thomas G DeLoughery, MD; Chief Editor: Emmanuel C Besa, MD  more...
 
Updated: Dec 23, 2015
 

Approach Considerations

The treatment goal for acute attacks of porphyria is to decrease heme synthesis and reduce the production of porphyrin precursors. High doses of glucose can inhibit heme synthesis and are useful for treatment of mild attacks. United Kingdom guidelines recommend administering 5% glucose in 0.9% sodium chloride solution, infused intravenously at a rate of 2 L/24 h. Intravenous glucose in water solutions (eg, dextrose 5% or 10% [D5W, D10W]), should be avoided as they may aggravate hyponatremia.[8]

Patients experiencing severe attacks, especially those with severe neurologic symptoms, should be treated with hematin in a dose of 4 mg/kg/d for 4 days. Once hematin is initiated, glucose therapy no longer has a role.[8]

Pain can be remarkably severe, and pain control is best achieved with narcotics. Laxatives and stool softeners should be administered with the narcotics to avert exacerbating existing constipation.

Symptomatic treatment also includes the use of beta-blockers to control tachycardia and prevent arrhythmia; beta- blockers, clonidine, or other recommended antihypertensives can also be used to treat hypertensive crisis. Nausea and vomiting can be controlled with olanzapine, lorazepam, or prochlorperazine.[9]

Treat seizures with gabapentin (Neurontin). Most classic antiseizure medicines can lead to acute porphyria attacks.

A minority of patients with acute intermittent porphyria experience recurrent attacks. In addition to avoidance of precipitating factors, treatment options that may be considered in those cases include gonadotrophin-releasing hormone analogues (for women with attacks related to their menstrual cycles) and prophylactic hematin infusions.[8]

Rarely, liver transplantation may be indicated for patients with intractable recurrent attacks that are life-threatening or severely affect quality of life. Liver transplantation cures AIP.[8]

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Diet

The patient should receive a high-carbohydrate diet during the attack. If the patient is unable to eat, intravenous glucose should be administered. Between attacks, eating a balanced diet is more important than eating one rich in glucose.

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Contributor Information and Disclosures
Author

Thomas G DeLoughery, MD Professor of Medicine, Pathology, and Pediatrics, Divisions of Hematology/Oncology and Laboratory Medicine, Associate Director, Department of Transfusion Medicine, Division of Clinical Pathology, Oregon Health and Science University School of Medicine

Thomas G DeLoughery, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Blood Banks, American College of Physicians, American Society of Hematology, International Society on Thrombosis and Haemostasis, Wilderness Medical Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Marcel E Conrad, MD Distinguished Professor of Medicine (Retired), University of South Alabama College of Medicine

Marcel E Conrad, MD is a member of the following medical societies: Alpha Omega Alpha, American Association for the Advancement of Science, American Association of Blood Banks, American Chemical Society, American College of Physicians, American Physiological Society, American Society for Clinical Investigation, American Society of Hematology, Association of American Physicians, Association of Military Surgeons of the US, International Society of Hematology, Society for Experimental Biology and Medicine, SWOG

Disclosure: Partner received none from No financial interests for none.

Chief Editor

Emmanuel C Besa, MD Professor Emeritus, Department of Medicine, Division of Hematologic Malignancies and Hematopoietic Stem Cell Transplantation, Kimmel Cancer Center, Jefferson Medical College of Thomas Jefferson University

Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American Society of Clinical Oncology, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Hematology, New York Academy of Sciences

Disclosure: Nothing to disclose.

Additional Contributors

Clarence Sarkodee Adoo, MD, FACP Consulting Staff, Department of Bone Marrow Transplantation, City of Hope Samaritan BMT Program

Clarence Sarkodee Adoo, MD, FACP is a member of the following medical societies: American College of Physicians-American Society of Internal Medicine, American Society of Hematology, American Society of Clinical Oncology

Disclosure: Nothing to disclose.

References
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  8. [Guideline] Stein P, Badminton M, Barth J, Rees D, Stewart MF, British and Irish Porphyria Network. Best practice guidelines on clinical management of acute attacks of porphyria and their complications. Ann Clin Biochem. 2013 May. 50 (Pt 3):217-23. [Medline]. [Full Text].

  9. Pischik E, Kauppinen R. An update of clinical management of acute intermittent porphyria. Appl Clin Genet. 2015. 8:201-14. [Medline]. [Full Text].

  10. Willandt B, Langendonk JG, Biermann K, Meersseman W, D'Heygere F, George C, et al. Liver Fibrosis Associated with Iron Accumulation Due to Long-Term Heme-Arginate Treatment in Acute Intermittent Porphyria: A Case Series. JIMD Rep. 2015 Jun 21. 50 (Pt 3):217-23. [Medline].

  11. Delaby C, To-Figueras J, Deybach JC, et al. Role of two nutritional hepatic markers (insulin-like growth factor 1 and transthyretin) in the clinical assessment and follow-up of acute intermittent porphyria patients. J Intern Med. 2009 Apr 23. epub ahead of print. [Medline].

  12. Syal K, Bhatt R, Singh S, Ohri A. Acute intermittent porphyria. J Anaesthesiol Clin Pharmacol. 2015 Apr-Jun. 31 (2):261-3. [Medline]. [Full Text].

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  14. Anderson KE, Bloomer JR, Bonkovsky HL, et al. Recommendations for the diagnosis and treatment of the acute porphyrias. Ann Intern Med. 2005 Mar 15. 142(6):439-50. [Medline].

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Table 1. Drugs Thought Safe in Porphyria*
Acetazolamide acetylcholine



Actinomycin D



Acyclovir



Adenosine monophosphate



Adrenaline



Alclofenac



Allopurinol



Alpha tocopheryl



Acetate



Amethocaine



Amiloride



Aminocaproic acid



Aminoglycosides



Amoxicillin



Amphotericin



Ampicillin



Ascorbic acid



Aspirin



Atenolol



Atropine



Azathioprine



Beclomethasone



Benzhexol HCl



Beta-carotene



Biguanides



[Bromazepam]



Bromides



Buflomedil HCl



Bumetanide



Bupivacaine



Buprenorphine



Buserelin



Butacaine SO4



Canthaxanthin



Carbimazole



[Carpipramine HCl]



Chloral hydrate



[Chlormethiazole]



[Chloroquine]



[Chlorothiazide]



Chlorpheniramine



Chlorpromazine



Ciprofloxacin



Cisapride



Cisplatin



Clavulanic acid



Clofibrate



Clomiphene



Cloxacillin



Co-codamol



Codeine phosphate



Colchicine



[Corticosteroids]



Corticotrophin (adrenocorticotropic hormone [ACTH])



Coumarins



Cyclizine



Cyclopenthiazide



Cyclopropane



[Cyproterone acetate]



Danthron



Desferrioxamine



Dexamethasone



[Dextromoramide]



Dextrose



Diamorphine



Diazoxide



Dicyclomine HCl



Diflunisal



Digoxin



Dihydrocodeine



Dimercaprol



Dimethicone



Dinoprost



Diphenoxylate HCl



Dipyridamole



[Disopyramide]



Domperidone



Doxorubicin HCl



Droperidol



[Estazolam]



Ethacrynic acid



Ethambutol



[Ethinyl oestradiol]



Ethoheptazine citrate



Etoposide



Famotidine



Fenbufen



[Fenofibrate]



Fenoprofen



Fentanyl



Flucytosine



Flumazenil



Fluoxetine HCl



Flurbiprofen



Fluvoxamine



Maleate



Folic acid



Fructose



Fusidic acid



Follicle-stimulating hormone



Gentamicin



Glafenine



Glucagon



Glucose



Glyceryl trinitrate



Goserelin



Guanethidine



Guanfacine HCl



Haem arginate



[Haloperidol]



Heparin



Heptaminol HCl



Hexamine



[Hydrocortisone]



Ibuprofen



Indomethacin



Insulin



Iron



Josamycin



[Ketamine]



Ketoprofen



Ketotifen



Labetalol



Luteinizing hormone–releasing hormone



Liquorice



Lithium



Salts lofepramine



Loperamide



[Lorazepam]



Magnesium-sulphate



[Mebendazole]



Mecamylamine



Meclofenoxate HCl



Meclozine



Mefloquine HCl



[Melphalan]



Meptazinol



Mequitazine



Metformin



Methadone



[Methotrimeprazine]



Methylphenidate



Methyluracil



Metipropranolol



Metopimazine



Metoprolol



[Metronidazole]



[Midazolam]



Minaprine HCl



Minaxolone



Morphine



Nadolol



Naftidrofuryl



Oxalate



[Naproxen sodium]



Natamycin



Nefopam HCl



Neostigmine



Netilmicin



Niflumic acid



Nitrous oxide



Norfloxacin



Ofloxacin



Oxolinic acid



Oxybuprocaine



[Oxyphenbutazone]



Oxytocin



[Pancuronium bromide]



Paracetamol



Paraldehyde



Parapenzolate Br



Penicillamine



Penicillin



Pentolinium



Pericyazine



Pethidine



Phenformin



Phenoperidine



Phentolamine mesylate



Pipotiazine



Palmitate



Piracetam



Pirbuterol



Pirenzepine



Pizotifen



[Prazosin]



[Prednisolone]



Primaquine



Probucol



Procainamide HCl



Procaine



Prochlorperazine



Proguanil HCl



Promazine



Propantheline Br



Propofol



Propranolol



Propylthiouracil



[Proxymetacaine]



Pseudoephedrine HCl



Pyridoxine



[Pyrimethamine]



Quinidine



Quinine



[Ranitidine]



Reserpine



Resorcinol



Salbutamol



Senna



Sodium bromide



Sodium ethylenediaminetetraacetic acid



Sodium fusidate



Sorbitol



Streptomycin



Sulbutiamine



Sulindac



Sulfadoxine



Suxamethonium



Talampicillin



Temazepam



Tetracaine



[Tetracyclines]



Thiouracils



Thyroxine



Tiaprofenic acid



Ticarcillin



Tienilic acid



Timolol maleate



Tolazoline



Tranexamic acid



Triacetyloleandomycin



Triamterene



Triazolam



[Trichlormethiazide]



Trifluoperazine



Trimeprazine



Tartrate



Trimetazidine HCl



Tripelennamine



Tubocurarine



Vancomycin



[Vincristine]



Vitamins



Warfarin sodium



Zidovudine



Zinc Preparations



*Bracketed [] drugs are those in which experimental evidence of porphyrin genicity is conflicting.
Table 2. Drugs Thought Unsafe in Porphyria
Alcuronium



*Alphaxalone



Alphadolone



Alprazolam



Aluminium



Preparations



Amidopyrine



Aminoglutethimide Aminophylline



Amiodarone



*Amitriptyline



[Amphetamines]



*Amylobarbitone



Antipyrine



*Auranofin



*Aurothiomalate



Azapropazone



Baclofen



*Barbiturates



*Bemegride



Bendrofluazide



Benoxaprofen



Benzbromarone



[Benzylthiouracil]



[Bepridil]



Bromocriptine



Busulphan



*Butylscopolamine Captopril



*Carbamazepine



*Carbromal



*Carisoprodol



[Cefuroxime]



[Cephalexin]



[Cephalosporins]



[Cephradine]



[Chlorambucil]



*Chloramphenicol



*Chlordiazepoxide *Chlormezanone



Chloroform



*Chlorpropamide



Cinnarizine



Clemastine



[Clobazam]



[Clomipramine HCl]



[Clonazepam]



Clonidine HCl



*Clorazepate



Cocaine



[Colistin]



Co-trimoxazole



Cyclophosphamide



Cycloserine



Cyclosporin



Danazol



*Dapsone



Dexfenfluramine



Dextropropoxyphene Diazepam



*Dichloralphenazone *Diclofenac Na



Dienoestrol



Diethylpropion



Dihydralazine



*Dihydroergotamine



Diltiazem



*Dimenhydrinate



*Diphenhydramine



[Dothiepin HCl]



Doxycycline



*Dydrogesterone



*Econazole NO3



*Enalapril



Enflurane



*Ergot compounds



Ergometrine maleate Ergotamine tartrate



*Erythromycin



*Estramustine



Ethamsylate



*Ethanol



Ethionamide



*Ethosuximide



*Ethotoin



Etidocaine



Etomidate



Fenfluramine



*Flucloxacillin



*Flufenamic acid



Flunitrazepam



Flupenthixol



Flurazepam



*Frusemide



*Glibenclamide



*Glutethimide



*Glipizide



Gramicidin



*Griseofulvin



[Haloperidol]



*Halothane



*Hydantoins



*Hydralazine



*Hydrochlorothiazide *Hydroxyzine



Hyoscine



*Imipramine



Iproniazid



Isometheptene mucate



[Isoniazid]



Kebuzone



Ketoconazole



*Levonorgestrel



Lignocaine



*Lisinopril



Loprazolam



Loxapine



*Lynestrenol



Lysuride



Maleate



Maprotiline HCl



Mebeverine HCl



*Mecillinam



*Medroxyprogesterone



[Mefenamic acid]



Megestrol acetate



*Mephenytoin



Mepivacaine



*Meprobamate



Mercaptopurine



Mercury compounds



Mestranol



[Metapramine HCl]



Methamphetamine



Methohexitone



Methotrexate



Methoxyflurane



Methsuximide



*Methyldopa



*Methylsulphonal



*Methyprylone



Methysergide



*Metoclopramide



Metyrapone



Mianserin HCl



Miconazole



[Mifepristone]



Minoxidil



*Nandrolone



*Nalidixic acid



Natamycin



*Nandrolone



[Nicergoline]



*Nifedipine



*Nikethamide



Nitrazepam



*Nitrofurantoin



Nordazepam



Norethynodrel



*Norethisterone



[Nortriptyline]



Novobiocin



*Oral contraceptives



*Orphenadrine



Oxanamide



[Oxazepam]



Oxybutynin HCl



Oxycodone



*Oxymetazoline



*Oxyphenbutazone



Oxytetracycline



Paramethadione



Pargyline



*Pentazocine



Perhexiline



Phenacetin



Phenelzine



*Phenobarbitone



Phenoxybenzamine



*Phensuximide



*Phenylbutazone



Phenylhydrazine



*Phenytoin



Pipebuzone



Pipemidic



Acid



Piritramide



*Piroxicam



*Pivampicillin



*Pivmecillinam



Prazepam



Prenylamine



*Prilocaine



*Primidone



[Probenecid]



*Progesterone



Progabide



Promethazine



[Propanidid]



*Pyrazinamide



Pyrrocaine



Quinalbarbitone



Rifampicin



Simvastatin



Sodium aurothiomalate



Sodium oxybate



[Sodium valproate]



*Spironolactone



Stanozolol



Succinimides



*Sulfacetamide



*Sulfadiazine



*Sulfadimidine



*Sulfadoxine



*Sulfamethoxazole *Sulfasalazine



*Sulfonylureas



Sulfinpyrazone



Sulpiride



Sulthiame



Sultopride



*Tamoxifen



*Terfenadine



Tetrazepam



*Theophylline



*Thiopentone Na



Thioridazine



Tilidate



Tinidazole



*Tolazamide



*Tolbutamide



Tranylcypromine



Trazodone HCl



Trimethoprim



[Trimipramine]



Troxidone



Valproate



Valpromide



Veralipride



*Verapamil



*Vibramycin



Viloxazine HCl



[Vinblastine]



[Vincristine]



Zuclopenthixol



*These drugs have been associated with acute attacks of porphyria.



†Bracketed [] drugs are those in which experimental evidence of porphyringenicity is conflicting.



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