Table of Contents

HK J Paediatr (New Series)
Vol 17. No. 1, 2012

HK J Paediatr (New Series) 2012;17;24-30

Original Article

Daytime Sleepiness and Obstructive Sleep Apnoea from Children to Young Adults with Beta-thalassaemia Intermedia: A Pilot Study

KCC Chan, AM Li, WCW Chu, RCH Li, SC Ling, HL Yuen, CK Li


Objective: This pilot study assessed the prevalence of daytime sleepiness and obstructive sleep apnoea (OSA) from children to young adults with beta-thalassaemia intermedia. Methods: Patients diagnosed with beta-thalassaemia intermedia and attended the four participating hospitals (Prince of Wales, Tuen Mun, Queen Elizabeth and Princess Margaret) for regular evaluations were recruited. All subjects were examined by a paediatrician and underwent overnight polysomnography (PSG). The subjects were also invited to undergo magnetic resonance imaging (MRI) of the upper airway. Serum haemoglobin and ferritin levels were measured after overnight PSG. A detailed sleep apnoea questionnaire and a Chinese version of the Epworth Sleepiness Scale were completed by parents of subjects under 18 years old or by subjects themselves if they were aged 18 or more. Findings: A total of 19 beta-thalassaemia intermedia patients (15 males and 4 females) aged 3-28 years were enrolled. Their mean age at diagnosis was 3.54 years. Four subjects were found to have OSA using obstructive apnoea hypopnoea index (OAHI) >1 as cut-off. Epworth sleepiness score, haemoglobin and ferritin levels, PSG parameters and sleep related symptoms were similar between subjects with and without OSA. All 4 OSA subjects and 7 non-OSA patients underwent MRI, and no significant differences between their upper airway parameters were noted. Conclusion: Prevalence of OSA maybe higher amongst patients with beta-thalassaemia intermedia but this has to be confirmed by further studies with larger sample size.

目的:這項研究初步評估兒童及青壯年中型β地中海貧血患者,日間嗜睡和阻塞性睡眠呼吸暫停(OSA)的患病率。方法:本研究所招募的相關患者,均為下列四所參與本研究的醫院病人(威爾斯親王醫院、屯門醫院、伊利沙伯醫院和瑪嘉烈醫院),這些患者被診斷患有中型β地中海貧血並定期進行評估。所有病例均接受兒科醫師體檢,並行整夜多導睡眠圖檢查(PSG)。同時也應邀接受上呼吸道的磁力共振素描檢查(MRI)。整夜多導睡眠圖檢查後測量血清鐵蛋白和血紅蛋白的水平。18歲以上的患者親自作答詳細的睡眠窒息症問卷及中文版Epworth嗜睡量表,18歲以下的患者則由父母作答。結果:本研究共招募19例中間型β地中海貧血患者(男性15例、女性4例),年齡3 ~28歲。診斷該病時的平均年齡為3.54歲。應用阻塞性呼吸暫停低通氣指數(OAHI) >1作為臨界值,發現4例為OSA。這4例病人的Epworth嗜睡評分、血紅蛋白和鐵蛋白水準、整夜多導睡眠圖參數和睡眠相關症狀與無OSA的病人之間無顯著差異。4名OSA受試者和7名非OSA受試者均進行了MRI檢查,其上呼吸道參數間並無顯著差異。結論:中型β地中海貧血患者OSA的患病率可能增高,但這還需要更大樣本量的研究進一步證實。

Keyword : Beta-thalassaemia; Magnetic resonance imaging; Sleep apnoea, obstructive



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