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Revista de la Asociación Médica del Centro Médico ABC

2007, Number 3

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An Med Asoc Med Hosp ABC 2007; 52 (3)

Porphyrias: Anesthetic considerations

Fernández DPL, Esquinca CMT, Vanesa RM, Valdespin PR

Language: Spanish
References: 83
Page: 130-142
PDF size: 145.26 Kb.


ABSTRACT

Porphyrias play an essential role in what concerns enzymatic sequences related with oxygen formation and with its transport, storage and consumption. As all forms of life, porphyrias need oxygen to live and, with this objective, they have adapted metabolic lines to employ the energy in biologic processes. Porphyrias may be classified into keen and no-keen ones. In both cases we are talking about metabolic illnesses caused by an autonomic hereditary failure, which provokes enzymatic failure in the process of heme formation. A failure in one of these enzymes generates an accumulation of substrates which are precursors of this failure, with wich a specefic form of keen porphyria is developed. The classic picture of keen porphyria is characterized by intense abdominal pain, nausea, vomiting, severe constipation, and psychiatric and neurological alterations which may progress to a bulbar paralysis. This crisis is developed by a series of risk factors, such as: some medications, alcohol consumption, hypocaloric diets, fasting, infections and menstrual cycles. In this review we will briefly describe porphyria synthesis and the clinical picture of patients with porphyria, with emphasis on the anesthetic implications, particularly the potential danger of anesthetics in terms of more morbility and mortality.


REFERENCES

  1. Thadani H, Deacon A, Peters T. Diagnosis and management of porphyria. BMJ 2000; 320: 1647-1651.

  2. Monroy SS. Porfirias agudas. An Med Asoc Med Hosp ABC 2004; 49 (2): 87-94.

  3. Baker S, Taylor B. Anaesthesia is also risky in patients with porphyria. BMJ 2000; 321: 1023. doi:10.1136/bmj.321.7267.1023.

  4. James MF, Hift RJ. Porphyrias. Review. Br J Anaesth 2000; 85 (1): 143-153.

  5. Hift RJ, Meissner PN. Related articles, an analysis of 112 acute porphyric attacks in Cape Town, South Africa: Evidence that acute intermittent porphyria and variegate porphyria differ in susceptibility and severity. Medicine (Baltimore) 2005 84 (1): 48-60.

  6. McColl Kel, Dover S, Fitzsimons E, Moore MR. Porphyrin metabolism and the porphyrias. In: Wheatherall DJ, Ledingham JGG, Warrell DA (eds). Oxford textbook of medicine. Oxford: Oxford University Press; 1996. p. 1388-1399.

  7. Consolo D, Ouardirhi Y, Wessels C, Girard C. Obstetrical anaesthesia and porphyries. Ann Fr Anesth Reanim 2005; 24 (4): 428-431.

  8. Brodie MJ, Moore MR, Golberg A. Pregnancy and the acute porphyrias. Br J Obstet Gynaecol. 1977; 84: 726-731.

  9. Kantor G, Rolbin SH. Acute intermittent porphyria and caesarean delivery. Can J Anaesth 1992; 39 (3): 282-285.

  10. Allen SC, Rees GA. A previous history of acute intermittent porphyria as a complication of obstetric anaesthesia. Br J Anaesth 1980; 52 (8): 835-838.

  11. Jensen NF, Fiddler DS, Striepe V. Review. Anesthetic considerations in porphyrias. Anesth Analg 1995; 80 (3): 591-599.

  12. Harrison GG, Meissner PN, Hift RJ. Review: Anaesthesia for the porphyric patient. Anaesthesia. 1993; 48 (5): 417-421.

  13. Stoelting RK, Dierdirf SF. Inborn errors of Metabolism: Anesthesia and co-existing disease, 4th ed. Philadelphia, PA: Churchill Livingstone, 2002. p. 455-460.

  14. Gonzalez-Arriaza HL, Bostwick. Acute porphyrias: A case report and review. JM Am J Psychiatry 2003; 160 (3): 450-459.

  15. Palmieri C, Vigushin DM, Peters TJ. Managing malignant disease in patients with porphyria. QJ Med 2004; 97 (3): 115-126.

  16. Winkler AS, Peters TJ, Marsden JT, Deacon AC, Chandler G, MacDougall IC. Erythropoietin treatment in the neuropsychiatric porphyrias. Clin Chim Acta 2003; 338 (1-2): 61-66.

  17. Sassa S. Modern diagnosis and management of the porphyrias. Br J Haematol 2006; 135 (3): 281-292.

  18. Hunter GH. Review anesthetic considerations in hepatic porphyrias. CRNA 1999; 10 (1): 6-14.

  19. Hahn M, Bonkovsky HL. Multiple chemical sensitivity syndrome and porphyria. A note of caution and concern. Review. Arch Intern Med 1997; 157 (3): 281-285.

  20. Sheppard L, Dorman T. Anesthesia in a child with homozygous porphybilinogen deaminase deficiency: A severe form of acute intermittent porphyria. Paediatr Anaesth 2005; 15 (5): 426-428.

  21. García CM, Filipe P, Alexandrino P et al. Familial case of variegate porphyria in Portugal: P08.52. J Europ Acad Dermatol Venereol 2005; 19 (suppl 2): 232.

  22. Gorchein A. Testing for porphoybilinogen in urine. A Clin Chem 2002; 48 (3): 564-566.

  23. Blanloeil Y, Deybach JC, Portier D, Joyau M, Nordmann Y. Anesthesia and hepatic porphyria. Ann Fr Anesth Reanim 1989; 8 (2): 109-125.

  24. Deacon AC, Peters TJ. Identification of acute porphyria: Evaluation of a commercial screening test for urinary porphobilinogen. Ann Clin Biochem 1998; 35 (Pt 6): 726-732.

  25. Moore MR. Biochemistry of porphyria. Review. Int J Biochem 1993; 25 (10): 1353-1368.

  26. Schoenfeld N, Mamet R. Individualized workup - a new approach to the biochemical diagnosis of acute attacks of neuroporphyria. Physiol Res 2007; 55 (suppl 2): S103-S108.

  27. Mamet R, Schoenfeld N. A reliable diagnosis of porphyria should precede any conclusion concerning the safety of a drug in porphyria. Anesthesiology 1999; 91 (2): 583-584.

  28. Jensen NF, Fiddler DS, Striepe V. Anesthetic considerations in porphyrias. Anesth Analg 1995; 80 (3): 591-599.

  29. Liu YP, Lien WC, Fang CC, Lai TI, Chen WJ, Wang HP. ED presentation of acute porphyria. Am J Emerg Med 2005; 23 (2): 164-167.

  30. Gorchein A. Drug treatment in acute porphyria. Review. Br J Clin Pharmacol 1997; 44 (5): 427-434.

  31. Benumof. Anesthesia & Uncommon Diseases, Hepatics Diseases: Hepatic Porphyria. 4th ed. Philadelphia, PA: WB Saunders, 1998. p. 162-163.

  32. Lip GY, McColl KE, Goldberg A, Moore MR. Smoking and recurrent attacks of acute intermittent porphyria. BMJ 1991; 302 (6775): 507.

  33. Mustajoki P, Nordmann Y. Early administration of heme arginate for acute porphyric attacks. Arch Intern Med 1993; 153 (17): 2004-2008.

  34. Tokola O, Mustajoki P, Himberg JJ. Related Articles, Links Haem arginate improves hepatic oxidative metabolism in variegate porphyria. Br J Clin Pharmacol 1988; 26 (6): 753-757.

  35. Tokola O, Mustajoki P, Himberg JJ. Haem arginate improves hepatic oxidative metabolism in variegate porphyria. Br J Clin Pharmacol 1988; 26 (6): 753-757.

  36. Ashley EM. Anaesthesia for porphyria. Br J Hosp Med. 1996; 56 (1): 37-42.

  37. O’Mahoney D, Wathen CG. Hypertension in porphyria - an understated problem. QJM 1996; 89 (2): 161-162.

  38. Reading PJ, Newman PK, Kelly WF, Bateson MC, Rothwell P. Treatment of immobilization hypercalcaemia in acute intermittent porphyria: Experience from three cases. Neurol Neurosurg Psychiat 1997; 62 (4): 421-422.

  39. Mustajoki P, Heinonen. General Anesthesia in “inducible” porphyrias. J Anesthesiology 1980; 53 (1): 15-20. Ann Fr Anesth Reanim 2005; 24 (4): 428-431.

  40. Magnus IA, Jarrett A, Prankerd TA, Rimington C. Erythropoietic protoporphyria. A new porphyria syndrome with solar urticaria due to protoporphyrinaemia. Lancet 1961; 2: 448-51.

  41. Corbin D, Williams A, Thompson D, Buckels J, Elias E. Erythropoietic protoporphyria: Unusual skin and neurological problems after liver transplantation. Herbert A. Gastroenterology 1991; 100 (6): 1753-1757.

  42. Rank JM, Carithers R, Bloomer J. Related. Evidence for neurological dysfunction in end-stage protoporphyric liver disease. Hepatology 1993; 18 (6): 1404-1409.

  43. Gorchein A. Drug safety in porphyria. Lancet 1980; 2 (8186): 152-153.

  44. Consolo D, Ouardirhi Y, Wessels C, Girard C. Obstetrical anaesthesia and porphyrias. Ann Fr Anesth Reanim 2005; 24 (4): 428-431.

  45. McNeill MJ, Bennet .Use of regional anaesthesia in a patient with acute porphyria. Br J Anaesth 1990; 64 (3): 371-373.

  46. Brennan L, Halfacre JA, Woods SD. Regional anaesthesia in porphyria. Br J Anaesth 1990; 65 (4): 594.

  47. Joyau M, Deybach JC, Durand M, Parmentier G, Nordmann Y. Peridural anesthesia with procaine and fentanyl in a parturient with acute intermittent porphyria. Ann Fr Anesth Reanim 1986; 5 (4): 453-455.

  48. Bohrer H, Schmidt H. Related articles. Regional anesthesia as anesthetic technique of choice in acute hepatic porphyria. J Clin Anesth 1992; 4 (3): 259.

  49. HUGHES RT. Acute porphyria in pregnancy. Lancet 1957; 272 (6963): 301-303.

  50. Stevens JJ, Kneeshaw JD. Mitral valve replacement in a patient with acute intermittent porphyria. Anesth Analg 1996; 82 (2): 416-418.

  51. Lock G, Holstege A, Mueller AR, Christe W, Doss MO, Scholmerich J, Neuhaus P. Liver failure in erythropoietic protoporphyria associated with choledocholithiasis and severe post-transplantation polyneuropathy. Liver 1996; 16 (3): 211-217.

  52. Harrison GG, Moore MR, Meissner PN. Porphyrinogenicity of etomidate and ketamine as continuous infusions. Screening in the DDC-primed rat model. Br J Anaesth 1985; 57 (4): 420-423.

  53. Kanbak M. Ketamine in porphyria. Anesth Analg 1997; 84 (6): 1395.

  54. Rizk SF, Jacobson JH 2nd, Silvay G. Ketamine as an induction agent for acute intermittent porphyria. Anesthesiology 1977; 46 (4): 305-306.

  55. Kostrzewska E, Gregor A. Ketamine in Acute Intermittent Porphyria-Dangerous or Safe? Anesthesiology 1979; 49: 376-377.

  56. Silvay G, Miller R, Tausk C. Safety of ketamine in patients with acute intermittent porphyria. Case reports. Acta Anaesthesiol Scand 1979; 23 (4): 329-330.

  57. Kanbak M. Ketamine in porphyria. Anesth Analg 1997; 84 (6): 1395.

  58. Sarantopoulos CD, Bratanow NC, Stowe DF, Kampine JP Uneventful propofol anesthesia in a patient with coexisting hereditary coproporphyria and hereditary angioneurotic edema. Anesthesiology 2000; 92 (2): 607-609.

  59. Meissner PN, Harrison GG, Hift RJ Propofol as an i.v. anaesthetic induction agent in variegate porphyria. Br J Anaesth 1991; 66 (1): 60-65.

  60. Sarantopoulos CD, Bratanow NC, Stowe DF, Kampine JP. Uneventful propofol anesthesia in a patient with coexisting hereditary coproporphyria and hereditary angioneurotic edema. Anesthesiology 2000; 92 (2): 607-609.

  61. Gorchein A. Porphyria, propofol and rats. Br J Anaesth 1996; 76 (1): 172.

  62. Weir PM, Hodkinson BP. Is propofol a safe agent in porphyria? Anaesthesia 1988; 43 (12): 1022-1023.

  63. Meissner PN, Harrison GG, Hift RJ. Propofol as an i.v. anaesthetic induction agent in variegate porphyria. Br J Anaesth 1991 Jan; 66 (1): 60-65.

  64. Hughes PJ. Propofol in acute porphyrias. Anaesthesia 1990; 45 (5): 415.

  65. Mitterschiffthaler G, Theiner A, Hetzel H, Fuith LC. Safe use of propofol in a patient with acute intermittent porphyria. Br J Anaesth 1988; 60 (1): 109-111.

  66. Tidmarsh MA, Baigent DF. Propofol in acute intermittent porphyria. Br J Anaesth 1992; 68 (2): 230.

  67. Kasraie N, Cousins TB. Propofol and the patient with hereditary coproporphyria. Anesth Analg 1993; 77 (4): 862-863.

  68. Bohrer H, Schmidt H, Motsch J. Propofol and the patient with hereditary coproporphyria. Anesth Analg 1994; 78 (6): 1200-1201.

  69. Nordmann Y, Grandchamp B, Phung N, de Verneuil H, Grelier M, Neiré J. Coproporphyrinogenoxidase deficiency in hereditary coproporphyria. Lancet 1977; 1 (8003): 140.

  70. Elcock D, Norris A. Elevated porphyrins following propofol anaesthesia in acute intermittent porphyria. Anaesthesia 1994; 49 (11): 957-958.

  71. Arsivatham SJ, Johnson TW et al. Prolonged loss of consciousness and elevated porphyrins following propofol administrations. Case reports. Anesthesiology 1998; 89: 1029-1031.

  72. Bohrer H, Schmidt H, Martin E, Lux R, Bolsen K, Goerz G. Testing the porphyrinogenicity of propofol in a primed rat model. Br J Anaesth 1995; 75 (3): 334-338.

  73. Evans PR, Graham S, Kumar CM. The use of sevofluorano in acute intermittent porphyria. Anaesthesia 2001; 56: 388-389.

  74. Messmer M, Gerheuser F, Forst H. Desflurane in acute intermittent porphyria. Anaesthesist 2004; 53 (3): 244-248.

  75. Buzaleh AM, García-Bravo M, Navarro S, Moran-Jiménez MJ, Méndez M, Batlle A, Fontanellas A, de Salamanca RE. Volatile anaesthetics induce biochemical alterations in the heme pathway in a B-lymphocyte cell line established from hepatoerythropoietic porphyria patients (LBHEP) and in mice inoculated with LBHEP cells. Int J Biochem Cell Biol 2004; 36 (2): 216-222.

  76. Lin YC, Chen L. Atracurium in a patient with acute intermittent porphyria. Anesth Analg 1990; 71: 440-441.

  77. Bohrer H, Schmidt H, Bach A, Martin E, Kohl B, Bolsen K, Goerz G. Porphyrinogenic effects of atracurium, vecuronium, and pancuronium in a primed rat model. Lab Anim Sci 1994; 44 (4): 326-330.

  78. McColl KE, Thompson GG, Moore. Studies in laboratory animals to assess the safety of anti-inflammatory agents in acute porphyria MRAnn. Rheum Dis 1987; 46 (7): 540-542.

  79. McNeill MJ, Bennet A. Use of regional anaesthesia in a patient with acute porphyria. Br J Anaesth 1990; 64 (3): 371-373.

  80. Chemmanur AT, Bonkovsky HL. Hepatic porphyrias: Diagnosis and management. Clin Liver Dis 2004; 8 (4): 807-838.

  81. Mustajoki P, Mustajoki S, Rautio A, Arvela P, Pelkonen O. Effects of heme arginate on cytochrome P450-mediated metabolism of drugs in patients with variegate porphyria and in healthy men. Clin Pharmacol Ther 1994; 56 (1): 9-13.

  82. Pivalizza EG, Abramson DC, Gottschalk LI. Porphyria, cardiopulmonary bypass, and volatile anesthetics. Anesth Analg 1996; 83 (3): 658.

  83. Ashley EM. Anaesthesia for porphyria. Br J Hosp Med 1996; 56 (1): 37-42.




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