Abstract
We report on a young woman with congenital methaemoglobinaemia detected by a pulse oximeter during anaesthesia. Investigation of the patient and her family showed that the methaemoglobinaemia resulted from a recessive deficiency of NADH-diaphorase enzyme. A knowledge of the working principles and limitations of pulse oximetry is essential to determine appropriaie management in desaturation episodes during the perioperative period.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Schwartz JM, Reiss AL, Jaffe ER (1985) Hereditary methemoglobinemia with deficiency of NADH cytochrome b5 reductase. In: Stanbury JB, Wyngaarden JB, Fredrickson DS, Goldstein JL, Brown MS (eds) The metabolic basis of inherited disease. McGraw-Hill, New York, pp 1654–1665
Alexander CM, Teller LE, Gross JB (1989) Principles of pulse oximetry: theoretical and practical considerations. Anesth Analg 68: 368–376
Ralston AC, Webb RK, Runciman WB (1991) Potential errors in pulse oximetry I. Pulse oximeter evaluation. Anaesthesia 46: 202–206
Scott EM, McGraw JC (1962) Purification and properties of diphosphopyridine nucleotide diaphorase of human erythrocytes. J Biol Chem 237: 249–252
Eisenkraft JB (1988) Pulse oximeter de-saturation due to methemoglobinemia. Anesthesiology 68: 279–282
Watcha MF, Connor MT, Hing AV (1989) Pulse oximetry in methemoglobinemia. Arch Pediatr Adolesc Med 143:845–847
Chisholm DG, Sturt H (1994) Congenital methaemoglobinaemia detected by preoperative pulse oximetry. Can J Anaesth 41: 519–522
Anderson ST, Hajduczek J, Barker SJ (1988) Benzocaine-induced methemoglobinemia in an adult: accuracy of pulse oximetry with methemoglobinemia. Anesth Analg 67:1099–1101
Sprung J, Bourkee DL, Mackenzie CF, Thomas P (1994) Chronic methemoglobinemia: improving hemoglobin saturation monitoring during anesthesia. J Clin Monit 10: 267–269
Gabel RA, Bunn HF (1974) Hereditary methemoglobinemia as a cause of cyanosis during anesthesia. Anesthesiology 40:516–518
Tanishima K, Tanimoto K, Tomoda A, Mawatari K, Matsukawa S, Yoneyama Y, Ohkuwa H, Takazakura E (1985) Hereditary methemoglobinemia due to cytochrome b5 reductase deficiency in blood cells without associated neurologic and mental disorders. Blood 66: 1288–1291
Scott EM, Hoskins DD (1958) Hereditary methemoglobinemia in Alaskan Eskimos and Indians. Blood 13: 795–802
Tanishima K (1987) Hereditary methemoglobinemia. Tanpakushitsu Kakusan Koso 32: 870–876 (in Japanese)
Stoelting RK, Miller RD (1994) Monitoring. In: Stoelting RK, Miller RD (eds) Basics of anesthesia, 3rd ed. Churchill Livingtone, New York, pp 201–215
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Sugahara, K., Sadohara, T., Kawaguchi, T. et al. NADH-diaphorase deficiency identified in a patient with congenital methaemoglobinaemia detected by pulse oximetry. Intensive Care Med 24, 706–708 (1998). https://doi.org/10.1007/s001340050648
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s001340050648