Hereditary Coproporphyria Follow-up

Author: Thomas G DeLoughery, MD; Chief Editor: Emmanuel C Besa, MD more...

Updated: Jan 10, 2012

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Further Inpatient Care

Admit patients with severe attacks for close monitoring.
Monitor patients with paralysis for signs of respiratory compromise.

Patients with recurrent attacks may benefit from a program of chronic hematin infusion. For example, women with severe symptoms at the time of their menses can have 1 dose of 4 mg/kg before the onset of their period.

Avoid medicines that can provoke an attack. The list of medications to avoid is long; however, only a few have been implicated clearly in porphyria. Patients also should avoid overconsumption of alcohol and avoid fasting.

Most patients (60-80%) who have an acute attack of porphyria never have another. Avoiding precipitating factors also helps prevent attacks. Researchers consider coproporphyria a less severe disease than AIP.

Teach patients the importance of avoiding unsafe drugs and fasting.
For excellent patient education resources, visit eMedicine's Esophagus, Stomach, and Intestine Center and Muscle Disorders Center. Also, see eMedicine's patient education articles Abdominal Pain in Adults, Constipation in Adults, and Chronic Pain.

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, and Wilderness Medical Society

Disclosure: Nothing to disclose.

Specialty Editor Board

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

Disclosure: Takeda Millenium Honoraria Speaking and teaching

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

Disclosure: Medscape Salary Employment

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, and Southwest Oncology Group

Disclosure: No financial interests None None

Rajalaxmi McKenna, MD, FACP Southwest Medical Consultants, SC, Department of Medicine, Good Samaritan Hospital, Advocate Health Systems

Rajalaxmi McKenna, MD, FACP is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology, and International Society on Thrombosis and Haemostasis

Disclosure: Nothing to disclose.

Chief Editor

Emmanuel C Besa, MD Professor, Department of Medicine, Division of Hematologic Malignancies, 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 College of Clinical Pharmacology, American Federation for Medical Research, American Society of Clinical Oncology, American Society of Hematology, and New York Academy of Sciences

Disclosure: Nothing to disclose.

References

Whatley SD, Mason NG, Woolf JR, et al. Diagnostic strategies for autosomal dominant acute porphyrias: retrospective analysis of 467 unrelated patients referred for mutational analysis of the HMBS, CPOX, or PPOX gene. Clin Chem. Jul 2009;55(7):1406-14. [Medline].
Barbaro M, Kotajärvi M, Harper P, Floderus Y. Identification of an AluY-mediated deletion of exon 5 in the CPOX gene by MLPA analysis in patients with hereditary coproporphyria. Clin Genet. Jan 13 2011;[Medline].
Ventura P, Cappellini MD, Rocchi E. The acute porphyrias: a diagnostic and therapeutic challenge in internal and emergency medicine. Intern Emerg Med. Aug 2009;4(4):297-308. [Medline].
Billoo AG, Lone SW. A family with acute intermittent porphyria. J Coll Physicians Surg Pak. May 2008;18(5):316-8. [Medline].
Corrigall AV, Campbell JA, Siziba K, Kirsch RE, Meissner PN. The expression of protoporphyrinogen oxidase in human tissues. Cell Mol Biol (Noisy-le-grand). Jul 1 2009;55(2):89-95. [Medline].
Roshal M, Turgeon J, Rainey PM. Rapid quantitative method using spin columns to measure porphobilinogen in urine. Clin Chem. Feb 2008;54(2):429-31. [Medline].
Harper P, Wahlin S. Treatment options in acute porphyria, porphyria cutanea tarda, and erythropoietic protoporphyria. Curr Treat Options Gastroenterol. Dec 2007;10(6):444-55. [Medline].
Ma E, Mar V, Varigos G, Nicoll A, Ross G. Haem arginate as effective maintenance therapy for hereditary coproporphyria. Australas J Dermatol. May 2011;52(2):135-8. [Medline].
Anderson KE. The Porphyrias. In: Zakim D, Boyer TD, eds. Hepatology: A Textbook of Liver Disease. Philadelphia, Pa:. WB Saunders;1996:417-463.
Anderson KE, Bloomer JR, Bonkovsky HL, et al. Recommendations for the diagnosis and treatment of the acute porphyrias. Ann Intern Med. Mar 15 2005;142(6):439-50. [Medline].
Bickers DR, Pathak MA, Lim HW. The Porphyrias. In: Fitzpatrick B, et al, eds. Dermatology in General Medicine. New York, NY:. McGraw-Hill, Inc;1993:1854-1893.
Bonkovsky HL, Barnard GF. Diagnosis of porphyric syndromes: a practical approach in the era of molecular biology. Semin Liver Dis. 1998;18(1):57-65. [Medline].
Daniell WE, Stockbridge HL, Labbe RF, et al. Environmental chemical exposures and disturbances of heme synthesis. Environ Health Perspect. Feb 1997;105 Suppl 1:37-53. [Medline].
Elder GH, Smith SG, Smyth SJ. Laboratory investigation of the porphyrias. Ann Clin Biochem. Sep 1990;27 ( Pt 5):395-412. [Medline].
Gorchein A. Drug treatment in acute porphyria. Br J Clin Pharmacol. Nov 1997;44(5):427-34. [Medline].
Hahn M, Bonkovsky HL. Multiple chemical sensitivity syndrome and porphyria. A note of caution and concern. Arch Intern Med. Feb 10 1997;157(3):281-5. [Medline].
Kalman DR, Bonkovsky HL. Management of acute attacks in the porphyrias. Clin Dermatol. Mar-Apr 1998;16(2):299-306. [Medline].
Kauppinen R, Mustajoki P. Prognosis of acute porphyria: occurrence of acute attacks, precipitating factors, and associated diseases. Medicine (Baltimore). Jan 1992;71(1):1-13. [Medline].
Laiwah AC, McColl KE. Management of attacks of acute porphyria. Drugs. Nov 1987;34(5):604-16. [Medline].
Martasek P. Hereditary coproporphyria. Semin Liver Dis. 1998;18(1):25-32. [Medline].
Massey EW. Neuropsychiatric manifestations of porphyria. J Clin Psychiatry. Jun 1980;41(6):208-13. [Medline].
Mattern SE, Tefferi A. Acute porphyria: the cost of suspicion. Am J Med. Dec 1999;107(6):621-3. [Medline].
Moore MR. The biochemistry of heme synthesis in porphyria and in the porphyrinurias. Clin Dermatol. Mar-Apr 1998;16(2):203-23. [Medline].
Murphy GM. The cutaneous porphyrias: a review. The British Photodermatology Group. Br J Dermatol. Apr 1999;140(4):573-81. [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.

View Table List

Read more about Hereditary Coproporphyria on Medscape

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.

Hereditary Coproporphyria Follow-up

Further Inpatient Care

Further Outpatient Care

Deterrence/Prevention

Prognosis

Patient Education