Klin Padiatr 2012; 224(04): 259-265
DOI: 10.1055/s-0032-1312612
Review
© Georg Thieme Verlag KG Stuttgart · New York

Oxygen Saturation in Pulse Oximetry in Hemoglobin Anomalies

Sauerstoffsättigungen in der Pulsoximetrie bei Hämoglobinanomalien
B. Zur
1   Institut für Klinische Chemie und Klinische Pharmakologie, Universitätsklinik Bonn, Germany
,
S. Bagci
2   Abteilung Neonatologie, Zentrum für Kinderheilkunde, Universitätsklinik Bonn, Germany
,
M. Ludwig
1   Institut für Klinische Chemie und Klinische Pharmakologie, Universitätsklinik Bonn, Germany
,
B. Stoffel-Wagner
1   Institut für Klinische Chemie und Klinische Pharmakologie, Universitätsklinik Bonn, Germany
› Author Affiliations
Further Information

Publication History

Publication Date:
19 July 2012 (online)

Abstract

Background:

Pulse oximetry is an essential diagnostic method in pediatric emergency medicine and pediatric intensive care. However, if undetected hemoglobin anomalies are the underlying cause measurements of low oxygen saturation can be interpreted incorrectly or may lead to unnecessary examinations. In 2 recently discovered hemoglobin anomalies, Hb Bonn and Hb Venusberg, this resulted in extensive and repeat cardiopulmonary examinations. This review aims to provide an overview of hemoglobin anomalies causing low oxygen saturation.

Methods:

We describe the methods required for differential diagnosis of hemoglobin anomalies, such as hemoglobin electrophoresis, High Performance Liquid Chromatography, hemoglobin gene sequencing and spectral photometry, and the difficulties with the interpretation of results. Furthermore, with a review of the literature we provide an extensive overview of hemoglobin anomalies which result in low oxygen saturation measurement in pulse oximetry. With the examples of Hb Bonn, a novel hemoglobin mutation of the proximal α1-globin, which results in false low pulse oximetry measurements of oxygen saturation, and Hb Venusberg, a low oxygen-affine hemoglobin mutation of the β-globin, we highlight the difficulties arising from the respective case histories.

Conclusion:

In pediatric medicine, hemoglobin anomalies must be included in the diagnosis as a possible underlying cause of low oxygen saturation in case of ambiguous or conflicting pulse oximetry findings.

Zusammenfassung

Hintergrund:

Die Pulsoximetrie ist ein essenzielles diagnostisches Verfahren in der pädiatrischen Notfall- und Intensivmedizin. Niedrige Sauerstoffsättigungen in der Pulsoximetrie können bei verschiedenen Hämoglobinanomalien aber auch falsch interpretiert werden oder zu unnötigen Untersuchungen führen. Bei den kürzlich entdeckten Hämoglobinanomalien, Hb Bonn und Hb Venusberg, führte dies zu aufwendigen und wiederholten kardiopulmologischen Untersuchungen, was uns dazu veranlasste, eine Übersicht über Hämoglobinanomalien, die niedrige Sauerstoffsättigungen in der Pulsoximetrie verursachen, zu verfassen.

Methoden:

Diese Übersicht beschreibt zum einen die Methoden, die für eine konsequente Diagnostik von Hämoglobinanomalien notwendig sind, wie Hämoglobin-Elektrophorese, High Performance Liquid Chromatography, Hämoglobin-Gen-Sequenzierung und Spektralphotometrie, und stellt die Probleme der Interpretation dar. Zum anderen wurde Literaturrecherche herangezogen, um einen möglichst großen Überblick über Hämoglobinanomalien, die in der Puls­oximetrie zu niedrigen Sättigungen führen, zu verschaffen. Anhand von Hb Bonn, eine neue Hämoglobinmutation des proximalen α1-Globins, das pulsoximetrisch zu falsch niedrig gemesse­ner Sauerstoffsättigung führt, und Hb Venusberg, eine niedrig sauerstoffaffine Hämoglobinmutation des β-Globins, wird beispielhaft aufgezeigt, welche Schwierigkeiten sich aus der Krankengeschichte ergeben.

Schlussfolgerung:

In der Pädiatrie sollten Hämoglobinanomalien als Ursachen niedriger Sauerstoffsättigungen in differenztialdiagnostische Überlegungen miteinbezogen werden, wenn es zu unklaren oder widersprüchlichen Befunden bei der Pulsoximetrie kommt.

 
  • References

  • 1 Abdulmalik O, Safo MK, Lerner NB et al. Characterization of hemoglobin bassett (alpha94Asp→Ala), a variant with very low oxygen affinity. Am J Hematol 2004; 77: 268-276
  • 2 Bowes WA, Corke BC, Hulka J. Pulse oximetrie: A review of the theory, accuracy, and clinical applications. Obstet Gynecol 1989; 74: 541
  • 3 Cecil WT, Thorpe KJ, Fibuch EE et al. A clinical evaluation of the accuracy of the Nellcor N-100 an Ohmeda 3700 pulse oximeters. J Clin Monit 1988; 4: 31
  • 4 Chebbo A, Tfaili A, Jones SF. Hypoventilation syndromes. Med Clin North Am 2011; 95: 1189-1202
  • 5 de-Wahl Granelli A, Wennergren M, Sandberg K et al. Impact of pulse oximetry screening on the detection of duct depent congenital heart disease: a Swedish prospective screening study in 39821 newborns. BMJ 2009; 338a3037
  • 6 Deyell R, Jackson S, Spier S et al. Low oxygen saturation by pulse oximetry may be associated with a low oxygen affinity hemoglobin variant, hemoglobin Titusville. J Pediatr Hematol Oncol 2006; 28: 100-102
  • 7 Ewer AE, Middleton LJ, Furmston AT et al. Pulse oximetry screening for congenital heart defects in newborn infants (PulsOx): a test accuracy study. Lancet 2011; 378: 785-794
  • 8 Ferrazza AM, Martolini D, Valli G et al. Cardiopulmonary exercise testing in the functional and prognostic evaluation of patients with pulmonary diseases. Respiration 2009; 77: 3-17
  • 9 Fitzgerald RK, Johnson A. Pulse oximetry in sickle cell anemia. Crit Care Med 2001; 29: 1803-1806
  • 10 Fouzas S, Priftis KN, Anthracopulos MB. Pulse Oximetry in Pediatric Practice. Pediatrics 2011; 128: 740-752
  • 11 Göbel U, Görtner L, Niehus T. Hematologic or immunologic disease as cause of cardiopulmonary distress. Klin Padatr 2009; 221: 273-274
  • 12 Gortner L, Möller J, Reiss I. Akutes, nicht obstruktives Lungenversagen (ARDS+ALI) im Kindesalter. [ARDS+ALI in Childhood-Treatment Guideline]. Klin Padiatr 2011; 223: 440-444
  • 13 Hayashi A, Shimizu A, Yamamura Y et al. Hemoglobins M: identification of Iwate, Boston, and Saskatoon variants. Science 1966; 152: 207-208
  • 14 Hermansen CL, Lorah KN. Respiratory Distress in the Newborn. Am Fam Physician 2007; 76: 987-994
  • 15 Hohl RJ, Sherburne AR, Feeley JE et al. Low pulse oximeter-measured hemoglobin oxygen saturations with hemoglobin Cheverly. Am J Hematol 1998; 59: 181-184
  • 16 Huehns ER, Hecht F, Yoshida A et al. Hemoglobin-Seattle (agr2beta276 Glu): An Unstable Hemoglobin Causing Hemolytic Anemia. Blood 1970; 36: 209-218
  • 17 Hünseler C, Lange L, Kribs A et al. Primary Ciliary Dyskinesia Causing Neonatal Respiratory Distress. Klin Padiatr 2009; 221: 440-443
  • 18 Huicho L, Pawson IG, León-Velarde F et al. Oxygen saturation and heart rate in healthy school children and adolescens living at high altitude. Am J Hum Biol 2001; 13: 761-770
  • 19 Imamura T, Fujita S, Ohta Y et al. Hemoglobin Yoshizuka (G10(1008)β Asparagine – Aspatic Acid): a New Variant with a Reduced Oxygen Affinity from a Japanese Familiy. J Clin Invest 1969; 48: 2341-2348
  • 20 Katoh R, Miyake T, Arai T. Unexpectedly low pulse oximeter readings in a boy with unstable hemoglobin Köln. Anesthesiology 1989; 70: 112
  • 21 Khemani RG, Thomas NJ, Venkatachalam V et al. Comparison of SpO2 to PaO2 based markers of lung disease severity for children with acute lung injury. Crit Care Med 2012; 40: 1309-1316
  • 22 Kleihauer E. Anomale Hämoglobine und Thalassämiesyndrome: Grundlagen und Klinik. ecomed Verlagsgesellschaft 1996; 180-183
  • 23 Kuji A, Satoh Y, Kikuchi K et al. The anesthetic management of a patient with haemoglobin M(Iwate). Anesth Analg 2001; 93: 1192-1193
  • 24 Luo HY, Irving I, Prior J et al. Hemoglobin Titusville, a Low Oxygen Affinity Variant Hemoglobin, in a Family of Northern European Backgraound. Am J Hematol 2004; 77: 384-386
  • 25 Mau MK, Yamasato KS, Yamamoto LG. Normal oxygen saturation values in pediatric patients. Hawaii Med J 2005; 64 (42) 44-45
  • 26 Mendelson Y. Pulse Oximetry: theory and applications for noninvasive monitoring. Clin Chem 1992; 38+9: 1601-1607
  • 27 Morita K, Fukuzawa J, Onodera S et al. Hemoglobin Kansas found in a patient with polycythemia. Ann Hematol 1992; 65: 229-231
  • 28 Nagel RL, Lynfield J, Johnson J et al. Hemoglobin Beth Israel – A Mutant Causing Clinically Apperent Cyanosis. N Engl J Med 1976; 295: 125-130
  • 29 Oeverland B, Skatvedt O, Kari J et al. Pulseoximetry: sufficient to diagnose severe sleep apnea. Sleep Med 2002; 3: 133-138
  • 30 Pianosi P, Charge TD, Esseltine DW et al. Pulse oximetry in sickle cell disease. Arch Dis Child 1993; 68: 735-738
  • 31 Poyart C, Schaad O, Kister J et al. Hemoglobin Saint Mande [beta 102 (G4) Asn-Tyr]. Functional studies and structural modelling reveal an altered T state. Eur J Biochem 1990; 194: 343-348
  • 32 Rackoff WR, Kunkel N, Silber JH et al. Pulse oximetry and factors associated with hemoglobin oxygen desaturation in children with sickle cell disease. Blood 1993; 81: 3422-3427
  • 33 Ralston AC, Webb RK, Runciman WB. Potential errors in pulse oximetry. Anaesthesia 1991; 46: 291-295
  • 34 Rosa J, Labie D, Wajcman H et al. Haemoglobin I Toulouse: ß-66 (E 10) Lys→Glu: a new abnormal haemoglobin with a mutation localized on the E 10 prophyrin surrounding zone. Nature 1996; 223: 190-196
  • 35 Rosychuk RJ, Hudson-Mason A, Eklund D et al. Discrepancies between arterial oxygen saturation and functional oxygen saturation measured with pulse oximetrie in very preterm infants. Neonatology 2012; 101: 14-19
  • 36 Sarikonda KV, Ribeiro RS, Herrick JL et al. Hemoglobin lansing: A novel haemoglobin variant causing falsely decreased oxygen saturation by pulse oximetry. Am J Hematol 2009; 84: 541
  • 37 Schnapp LM, Cohen NH. Pulse oximetry. Uses and abuses. Chest 1990; 98: 1244-1250
  • 38 So CC, Chan AY, Chow EY et al. Haemoglobin Bonn in a Chinese family as cause of spurious hypoxaemia measured by pulse oximetry. J Clin Pathol 2010; 63: 947-949
  • 39 Starkey CR, Davies L, Hoyer JD et al. Clinical manifestations of hemoglobin Chico at high altitude. J Pediatr Hematol Oncol 2006; 28: 760-762
  • 40 Stenson B, Brocklehurst P, Tarnow-Mordi W. Increased 36-Week Survival with High Oxygen Saturation Target in Extremely Preterm Infants. N Engl J Med 2011; 364: 1680-1682
  • 41 Tautz J, Merkel C, Loersch F. Implication of pulse oxymetry screening for detection of congenital heart defects. Klin Padiatr 2010; 222: 291-295
  • 42 Vargas MH, Heyaime-Lalane J, Pérez-Rodriguez L et al. Day-night fluctuation of pulse oximetry: an exploratory study in pediatric inpatients. Rev Invest Clin 2008; 60: 303-310
  • 43 Zijlstra WG, Buursma A, Meeuwsen-van der Rost WP. Absorption spectra of human fetal and adult oxyhemoglobin, de-Oxyhemoglobin, carboxyhemoglobin, and methemoglobin. Clin Chem 1991; 37+9: 1633-1638
  • 44 Zur B, Mayer-Hubner B, Ludwig M et al. A 14 year old boy with chronic cyanosis, mild anemia, and limited physical resistance to stress. Clin Chem 2012; 58: 332-336
  • 45 Zur B, Hornung A, Breuer B et al. A Novel Hemoglobin, Bonn, Causes Falsely Decreased Oxygen Saturation Measurements in Pulse Oximetry. Clin Chem 2008; 54: 594-596