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P42 The curious case of HbKöln in the nighttime: an unusual case of apparent nocturnal hypoxaemia
  1. Tom Chambers,
  2. Rory Cairns and
  3. Nanak Singh
  1. Barts Health NHS Trust, London


A 23 year old female presents with snoring, unrefreshing sleep, and somnolence. Body mass index is 29kg/m2. Examination is unremarkable. She has a history of coeliac disease and unstable haemoglobin Köln (HbKöln).

Overnight respiratory polygraphy shows mean saturations of 88.6% and a saturation time <90% of 89.4% (figure 1).

What could be the cause of her low oxygen saturations?

Abstract P42 Figure 1

Overnight respiratory polygraph study showing a) full night oximetry trace with mean SpO2 of 88.6% and b) five minutes of representative flow data with oximetry trace. Apnoea-hypopnea index on the study was 1.1/hr and oxygen desaturation index was 1.2/hr. There was no suggestion Of hypoventilation on the study. Oximeter signal quality 98.4%

Investigations Unremarkable chest radiograph, spirometry, and gas transfer

Capillary blood gas pH 7.44, pCO2 4.76 KPa, pO2 13.5 KPa, Hb 133, sO2 97.2%, FO2Hb 94%, FCOHb 1.6%, FMetHb 1.7%, sO2 96.7%,BE 0.3, HCO3 24.9. Simultaneous pulse oximeter: sO2 89%.

Case explanation

Oximeters compare absorption spectra of oxyhaemoglobin and deoxyhaemoglobin in red (660nm) and infra-red (940nm) light to estimate oxygen saturation (figure 2). HbKöln is an autosomal dominant haemoglobinopathy with absorption spectra shifted upwards in the range of 600–900nm,1 resulting in reduced saturations measured by oximetry compared to the blood gas.

Key learning points

Abstract P42 Figure 2

Extinction curves showing differing absorption of red and infra-red light of normal adult oxyhaemoglobin and deoxyhaemoglobin. By comparing absorption of red and infrared light, oximeters estimate current oxygen saturations. Verhovsek et al. Am. J. Haematol. 2010; 85: 882–885

Oximeters are imperfect They can be inaccurate in carbon monoxide poisoning, dyshemoglobinemia, dark skinned patients, low saturation ranges, in patients with nail varnish and other situations.2

Clinical context, and family history, are key. Modern blood gas analysers use co-oximetry to measure absorption at >100 wavelengths to accurately distinguish ‘normal’ haemoglobin from other species.

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