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Nocturnal oxyhemoglobin desaturation and arteriopathy in a pediatric sickle cell disease cohort

Nocturnal oxyhemoglobin desaturation and arteriopathy in a pediatric sickle cell disease cohort
Nocturnal oxyhemoglobin desaturation and arteriopathy in a pediatric sickle cell disease cohort

OBJECTIVE: The purpose of this study of sickle cell disease (SCD) was to determine whether arteriopathy, measurable as intracranial vessel signal loss on magnetic resonance angiography (MRA), was associated with low nocturnal hemoglobin oxygen saturation (SpO2) or hemolytic rate, measurable as reticulocytosis or unconjugated hyperbilirubinemia.

METHODS: Ninety-five East London children with SCD without prior stroke had overnight pulse oximetry, of whom 47 (26 boys, 39 hemoglobin SS; mean age 9.1 ± 3.1 years) also had MRA, transcranial Doppler (TCD), steady-state hemoglobin, and reticulocytes within 34 months. Two radiologists blinded to the other data graded arteriopathy on MRA as 0 (none) or as increasing severity grades 1, 2, or 3.

RESULTS: Grades 2 or 3 arteriopathy (n = 24; 2 with abnormal TCD) predicted stroke/TIA compared with grades 0 and 1 (log-rank χ2 [1, n = 47] = 8.1, p = 0.004). Mean overnight SpO2 correlated negatively with reticulocyte percentage (r = -0.387; p = 0.007). Despite no significant differences across the degrees of arteriopathy in genotype, mean overnight SpO2 was higher (p < 0.01) in those with grade 0 (97.0% ± 1.6%) than those with grades 2 (93.9 ± 3.7%) or 3 (93.5% ± 3.0%) arteriopathy. Unconjugated bilirubin was not associated but reticulocyte percentage was lower (p < 0.001) in those with grade 0 than those with grades 2 and 3 arteriopathy. In multivariable logistic regression, lower mean overnight SpO2 (odds ratio 0.50, 95% confidence interval 0.26-0.96; p < 0.01) predicted arteriopathy independent of reticulocyte percentage (odds ratio 1.47, 95% confidence interval 1.15-1.87; p = 0.003).

CONCLUSION: Low nocturnal SpO2 and reticulocytosis are associated with intracranial arteriopathy in children with SCD. Preventative strategies might reduce stroke risk.

Anemia, Sickle Cell, Cerebral Arterial Diseases, Child, Cohort Studies, Female, Humans, Hyperbilirubinemia, Hypoxia, Ischemic Attack, Transient, Logistic Models, London, Magnetic Resonance Angiography, Magnetic Resonance Imaging, Male, Multivariate Analysis, Oximetry, Oxyhemoglobins, Reticulocytosis, Severity of Illness Index, Stroke, Ultrasonography, Doppler, Transcranial, Journal Article
0028-3878
2406-2412
Dlamini, Nomazulu
9d17b969-fd65-4620-888f-e54006d2e6dd
Saunders, Dawn E
9d877417-e3ee-493d-9477-a66b47c3455d
Bynevelt, Michael
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Trompeter, Sara
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Cox, Timothy C
f53c3285-98ba-44b0-9967-50a0e9a8d37b
Bucks, Romola S
95c31da3-2a01-45e7-a648-76d84a49edc4
Kirkham, Fenella J
1dfbc0d5-aebe-4439-9fb2-dac6503bcd58
Dlamini, Nomazulu
9d17b969-fd65-4620-888f-e54006d2e6dd
Saunders, Dawn E
9d877417-e3ee-493d-9477-a66b47c3455d
Bynevelt, Michael
b193cce3-8cf6-4b82-ab0a-6bb739b6fa29
Trompeter, Sara
14066edb-7c52-486f-86cd-3bcf3986deb4
Cox, Timothy C
f53c3285-98ba-44b0-9967-50a0e9a8d37b
Bucks, Romola S
95c31da3-2a01-45e7-a648-76d84a49edc4
Kirkham, Fenella J
1dfbc0d5-aebe-4439-9fb2-dac6503bcd58

Dlamini, Nomazulu, Saunders, Dawn E, Bynevelt, Michael, Trompeter, Sara, Cox, Timothy C, Bucks, Romola S and Kirkham, Fenella J (2017) Nocturnal oxyhemoglobin desaturation and arteriopathy in a pediatric sickle cell disease cohort. Neurology, 89 (24), 2406-2412. (doi:10.1212/WNL.0000000000004728).

Record type: Article

Abstract

OBJECTIVE: The purpose of this study of sickle cell disease (SCD) was to determine whether arteriopathy, measurable as intracranial vessel signal loss on magnetic resonance angiography (MRA), was associated with low nocturnal hemoglobin oxygen saturation (SpO2) or hemolytic rate, measurable as reticulocytosis or unconjugated hyperbilirubinemia.

METHODS: Ninety-five East London children with SCD without prior stroke had overnight pulse oximetry, of whom 47 (26 boys, 39 hemoglobin SS; mean age 9.1 ± 3.1 years) also had MRA, transcranial Doppler (TCD), steady-state hemoglobin, and reticulocytes within 34 months. Two radiologists blinded to the other data graded arteriopathy on MRA as 0 (none) or as increasing severity grades 1, 2, or 3.

RESULTS: Grades 2 or 3 arteriopathy (n = 24; 2 with abnormal TCD) predicted stroke/TIA compared with grades 0 and 1 (log-rank χ2 [1, n = 47] = 8.1, p = 0.004). Mean overnight SpO2 correlated negatively with reticulocyte percentage (r = -0.387; p = 0.007). Despite no significant differences across the degrees of arteriopathy in genotype, mean overnight SpO2 was higher (p < 0.01) in those with grade 0 (97.0% ± 1.6%) than those with grades 2 (93.9 ± 3.7%) or 3 (93.5% ± 3.0%) arteriopathy. Unconjugated bilirubin was not associated but reticulocyte percentage was lower (p < 0.001) in those with grade 0 than those with grades 2 and 3 arteriopathy. In multivariable logistic regression, lower mean overnight SpO2 (odds ratio 0.50, 95% confidence interval 0.26-0.96; p < 0.01) predicted arteriopathy independent of reticulocyte percentage (odds ratio 1.47, 95% confidence interval 1.15-1.87; p = 0.003).

CONCLUSION: Low nocturnal SpO2 and reticulocytosis are associated with intracranial arteriopathy in children with SCD. Preventative strategies might reduce stroke risk.

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More information

Accepted/In Press date: 1 April 2016
e-pub ahead of print date: 8 November 2017
Published date: 12 December 2017
Additional Information: Copyright © 2017 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.
Keywords: Anemia, Sickle Cell, Cerebral Arterial Diseases, Child, Cohort Studies, Female, Humans, Hyperbilirubinemia, Hypoxia, Ischemic Attack, Transient, Logistic Models, London, Magnetic Resonance Angiography, Magnetic Resonance Imaging, Male, Multivariate Analysis, Oximetry, Oxyhemoglobins, Reticulocytosis, Severity of Illness Index, Stroke, Ultrasonography, Doppler, Transcranial, Journal Article

Identifiers

Local EPrints ID: 417501
URI: https://eprints.soton.ac.uk/id/eprint/417501
ISSN: 0028-3878
PURE UUID: 1c8936c2-fdc4-4c20-9c92-90df71daabf7
ORCID for Fenella J Kirkham: ORCID iD orcid.org/0000-0002-2443-7958

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Date deposited: 01 Feb 2018 17:30
Last modified: 26 Nov 2019 01:51

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