Hall Rachael, Gardner Kate, Rees David C, Chakravorty Subarna
Department of Haematology, Imperial College London, London, UK.
Department of Haematological Medicine, King's College London, London, UK.
BMJ Paediatr Open. 2018 Oct 23;2(1):e000302. doi: 10.1136/bmjpo-2018-000302. eCollection 2018.
To assess the prevalence of high body mass index (BMI) in children with sickle cell disease and assess correlation between BMI and disease severity.
Retrospective chart review followed by statistical analysis.
A single tertiary paediatric clinic in inner city London.
All patients with sickle cell disease, including homozygous haemoglobin (HbSS) and compound heterozygous Hb (HbSC), age 2-18 years receiving clinical care at the centre, were included in the study.
Height and weight measurements, steady-state laboratory blood tests, hospital admission rates, adjunct therapy such as hydroxycarbamide or blood transfusions and obstructive sleep apnoea (OSA) data were obtained from the hospital electronic patient records.
To study the prevalence of high BMI and to identify any correlation between BMI and disease severity.
385 patients were included. 64 children (17%) were overweight or obese, of which a significantly higher number of children with HbSC were obese or overweight (23 out of 91, 25%) compared with those with HbSS (36 out of 273, 13%), p≤0.001. No correlation was found between high BMI and presence of OSA, and markers of disease severity such as admission rates, fetal haemoglobin or lactate dehydrogenase levels.
High BMI did not correlate with disease severity in this cohort of patients with sickle cell disease. Obesity was more prevalent in females and those with HbSC. Further prospective studies are needed to determine long-term effects of BMI in disease severity and outcome.
评估镰状细胞病患儿高体重指数（BMI）的患病率，并评估BMI与疾病严重程度之间的相关性。
回顾性病历审查及后续统计分析。
伦敦市中心的一家三级儿科诊所。
纳入该研究的为所有年龄在2至18岁、在该中心接受临床护理的镰状细胞病患者，包括纯合血红蛋白（HbSS）和复合杂合血红蛋白（HbSC）患者。
从医院电子病历中获取身高和体重测量值、稳态实验室血液检查结果、住院率、羟基脲或输血等辅助治疗以及阻塞性睡眠呼吸暂停（OSA）数据。
研究高BMI的患病率，并确定BMI与疾病严重程度之间的相关性。
共纳入385例患者。64名儿童（17%）超重或肥胖，其中HbSC患儿中超重或肥胖的人数显著多于HbSS患儿（91例中有23例，25%），而HbSS患儿中这一比例为273例中有36例，13%，p≤0.001。未发现高BMI与OSA的存在以及疾病严重程度指标（如住院率、胎儿血红蛋白或乳酸脱氢酶水平）之间存在相关性。
在这组镰状细胞病患者中，高BMI与疾病严重程度无关。肥胖在女性和HbSC患者中更为普遍。需要进一步的前瞻性研究来确定BMI对疾病严重程度和预后的长期影响。
