Long-term oxygen therapy in children with sickle cell disease and hypoxaemia

Liguoro, Ilaria; Arigliani, Michele; Singh, Bethany; Rees, David C.; Inusa, Baba Pd; Gupta, Atul

doi:10.1136/archdischild-2020-319347

Cited by 5 publications

(14 citation statements)

References 30 publications

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“…TCD velocities (right mean cerebral artery) pre‐ and postintervention in children with sickle cell disease and sleep‐disordered breathing: effect of adenotonsillectomy and oxygen. * 81 TCD, transcranial doppler [Color figure can be viewed at wileyonlinelibrary.com]…”

Section: Resultsmentioning

confidence: 99%

“…Mean nocturnal oxygen saturation (SpO 2 ) pre‐ and postintervention in children with sickle cell disease and sleep‐disordered breathing: cumulative effect and subgroup analysis (adenotonsillectomy—T&A; oxygen; HU). * 81 HU, hydroxyurea; T&A, tonsillectomy & adenoidectomy [Color figure can be viewed at wileyonlinelibrary.com]…”

Section: Resultsmentioning

confidence: 99%

“…Mean annual rate per patient of vaso‐occlusive pain crises pre‐ and postintervention (adenotonsillectomy—T&A; oxygen) in children with sickle cell disease and sleep‐disordered breathing: cumulative effect and subgroup analysis. * 81 T&A, tonsillectomy & adenoidectomy [Color figure can be viewed at wileyonlinelibrary.com]…”

Section: Resultsmentioning

confidence: 99%

“…A total of 11 papers 54,[74][75][76][77][78][79][80][81][82][83] focusing on different treatment options of SDB in children with SCD were identified and grouped according to the specific intervention to perform meta-analysis (Table 8). Four hundred and forty-three children (180 males, 40.6%, WM age 5.9 years) from six studies evaluating clinical effects of T&A, 54,74-78 80 patients (39 males, 49%, WM age 9.9 years) from two studies on HU 79,80 and 42 (21 males, 50%, WM age 11.8 years) from three studies on night oxygen [81][82][83] were included in the analysis (Table 8).…”

Section: Therapeutic Interventionsmentioning

confidence: 99%

“…13,15,53,69 We analyzed the impact of different interventions to treat SDB (T&A and oxygen) and no effect was shown on the mean annual rate of acute pain crises per patient. 74,75,78,[81][82][83] However, it should be pointed out that SCD patients with OSA or nocturnal hypoxemia are more likely to be started on HU, which represents a confounder difficult to adjust for (especially in retrospective studies) when evaluating the association between SDB and vaso-occlusive pain crises. Future prospective studies should definitely aim to further clarify the relationship between SDB and acute/chronic pain in SCD.…”

Section: Therapeutic Interventionsmentioning

confidence: 99%

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The burden of sleep disordered breathing in children with sickle cell disease

Liguoro

Arigliani

Tan

et al. 2021

Pediatric Pulmonology

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Children with sickle cell disease (SCD) have an increased risk of sleep disordered breathing (SDB) compared with the general pediatric population. There has been a growing research interest on this field in recent years, yet many questions regarding risk factors and clinical implications of SDB remain unclear. The aim of this review is to provide a concise narrative and systematic synthesis of the available evidence on the epidemiology, clinical presentation, complications, and management, of SDB in children with SCD. An electronic search was conducted on studies published from the 1st of January 2000 to the 31st of December 2020 in PubMed/Medline, Scopus, and Cochrane databases. All studies focusing on SDB in children with SCD aged from 0 to 20 years were included. Studies were eligible for inclusion if available in the English language. A quantitative synthesis of the included studies was performed.Only studies focusing on specific treatment outcomes were included in a metaanalytic process. A total of 190 papers were initially identified. After screening the title and abstract, 112 articles were evaluated for eligibility. At the end of the selection process, 62 studies were included in the analysis. Sleep disordered breathing is associated with worse neurological, neurocognitive, and cardiological outcomes, whereas the association with frequency or severity of vaso-occlusive pain events and acute chest syndrome was not clarified. Therapeutic interventions like adenotonsillectomy or oxygen supplementation may result in a significant increase in mean nocturnal oxygen saturation but effective clinical implications remain still unclear.

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Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

Section: Therapeutic Interventionsmentioning

confidence: 99%

Section: Therapeutic Interventionsmentioning

confidence: 99%

See 3 more Smart Citations

The burden of sleep disordered breathing in children with sickle cell disease

Liguoro

Arigliani

Tan

et al. 2021

Pediatric Pulmonology

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Oxygen saturation thresholds in managing sickle cell disease at US children's hospitals

Yadav,

Munir,

Chan

et al. 2024

Pediatric Blood & Cancer

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BackgroundAdequate oxygen saturation (SpO2) is crucial for managing sickle cell disease (SCD). Children with SCD are at increased risk for occult hypoxemia; therefore, understanding SpO2 threshold practices would help identify barriers to oxygen optimization in a population sensitive to oxyhemoglobin imbalances. We investigated SpO2 cutoff levels used in clinical algorithms for management of acute SCD events at children's hospitals across the United States, and determined their consistency with recommended national guidelines (SpO2 > 95%).MethodsClinical pathways and algorithms used for the management of vaso‐occlusive crisis (VOC) and acute chest syndrome (ACS) in SCD were obtained and reviewed from large children's hospitals in the United States.ResultsResponses were obtained from 94% (140/149) of eligible children's hospitals. Of these, 63 (45%) had available clinical algorithms to manage VOC and ACS. SpO2 cutoff was provided in 71.4% (45/63) of clinical algorithms. Substantial variation in SpO2 cutoff levels was noted, ranging from ≥90% to more than 95%. Only seven hospitals (5% of total hospitals and 15.6% of hospitals with clinical algorithms available) specified oxygen cutoffs that were consistent with national guidelines. Hospitals geographically located in the South (46.8%; n = 29/62) and Midwest (54.8%; n = 17/31) were more likely to have VOC and ACS clinical algorithms, compared to the Northeast (26.5%; n = 9/34) and West (36.4%; n = 8/22).ConclusionThere is inconsistency in the use of clinical algorithms and oxygen thresholds for VOC and ACS across US children's hospitals. Children with SCD could be at risk for insufficient oxygen therapy during adverse acute events.

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