eMedicine Specialties > Hematology > Heme Synthesis and Disorders

Porphyria, Hereditary Coproporphyria: Follow-up

Author: Thomas G DeLoughery, MD, Professor of Medicine and Pathology, Divisions of Hematology/Oncology and Laboratory Medicine, Associate Director, Department of Transfusion Medicine, Division of Clinical Pathology, Oregon Health Sciences University
Contributor Information and Disclosures

Updated: Nov 4, 2009

Follow-up

Further Inpatient Care

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

Further Outpatient Care

  • 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.

Deterrence/Prevention

  • 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.

Prognosis

  • 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.

Patient Education

Miscellaneous

Medicolegal Pitfalls

  • The most common diagnostic error in proporphyria is to label patients with mild elevations of urine coproporphyrins as having porphyria.
 


More on Porphyria, Hereditary Coproporphyria

Overview: Porphyria, Hereditary Coproporphyria
Differential Diagnoses & Workup: Porphyria, Hereditary Coproporphyria
Treatment & Medication: Porphyria, Hereditary Coproporphyria
Follow-up: Porphyria, Hereditary Coproporphyria
References
Further Reading
[ CLOSE WINDOW ]

References

  1. 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].

  2. 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].

  3. Billoo AG, Lone SW. A family with acute intermittent porphyria. J Coll Physicians Surg Pak. May 2008;18(5):316-8. [Medline].

  4. 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].

  5. 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].

  6. 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].

  7. Anderson KE. The Porphyrias. In: Zakim D, Boyer TD, eds. Hepatology: A Textbook of Liver Disease. Philadelphia, Pa:. WB Saunders;1996:417-463.

  8. 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].

  9. 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.

  10. 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].

  11. 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].

  12. Elder GH, Smith SG, Smyth SJ. Laboratory investigation of the porphyrias. Ann Clin Biochem. Sep 1990;27 ( Pt 5):395-412. [Medline].

  13. Gorchein A. Drug treatment in acute porphyria. Br J Clin Pharmacol. Nov 1997;44(5):427-34. [Medline].

  14. 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].

  15. Kalman DR, Bonkovsky HL. Management of acute attacks in the porphyrias. Clin Dermatol. Mar-Apr 1998;16(2):299-306. [Medline].

  16. 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].

  17. Laiwah AC, McColl KE. Management of attacks of acute porphyria. Drugs. Nov 1987;34(5):604-16. [Medline].

  18. Martasek P. Hereditary coproporphyria. Semin Liver Dis. 1998;18(1):25-32. [Medline].

  19. Massey EW. Neuropsychiatric manifestations of porphyria. J Clin Psychiatry. Jun 1980;41(6):208-13. [Medline].

  20. Mattern SE, Tefferi A. Acute porphyria: the cost of suspicion. Am J Med. Dec 1999;107(6):621-3. [Medline].

  21. Moore MR. The biochemistry of heme synthesis in porphyria and in the porphyrinurias. Clin Dermatol. Mar-Apr 1998;16(2):203-23. [Medline].

  22. Murphy GM. The cutaneous porphyrias: a review. The British Photodermatology Group. Br J Dermatol. Apr 1999;140(4):573-81. [Medline].

  23. Peters TJ, Sarkany R. Porphyria for the general physician. Clin Med. May-Jun 2005;5(3):275-81. [Medline].

  24. Poh-Fitzpatrick MB. Clinical features of the porphyrias. Clin Dermatol. Mar-Apr 1998;16(2):251-64. [Medline].

  25. Tefferi A, Colgan JP, Solberg LA Jr. Acute porphyrias: diagnosis and management. Mayo Clin Proc. Oct 1994;69(10):991-5. [Medline].

  26. Zaider E, Bickers DR. Clinical laboratory methods for diagnosis of the porphyrias. Clin Dermatol. Mar-Apr 1998;16(2):277-93. [Medline].

[ CLOSE WINDOW ]

Keywords

hereditary coproporphyria, porphyria, heme, porphyrin, acute porphyria, porphyrias, acute intermittent porphyria, cutaneous porphyria, porphyria tarda

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

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

Medical Editor

Clarence Sarkodee-Adoo, MD, Consulting Staff, Department of Bone Marrow Transplantation, City of Hope Samaritan BMT Program
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

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

CME Editor

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, 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 Hematology, and New York Academy of Sciences
Disclosure: Nothing to disclose.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.