Pane Marika, Vasta Isabella, Messina Sonia, Sorleti Domenica, Aloysius Annie, Sciarra Federico, Mangiola Fortunato, Kinali Maria, Ricci Enzo, Mercuri Eugenio
Department of Child Neurology, Policlinico Gemelli, Largo Gemelli 00168, Roma, Italy.
Eur J Paediatr Neurol. 2006 Sep-Nov;10(5-6):231-6. doi: 10.1016/j.ejpn.2006.08.008. Epub 2006 Oct 10.
The aim of the study was to conduct a survey using a dedicated questionnaire to estimate feeding difficulties, gastrointestinal involvement and weight gain in a population of 118 Duchenne muscular dystrophy (DMD) patients (age range 13.80-35.8 years). All the answers were entered in a database and the data analysed subdividing the cohort into age groups (3-9, 9-13, 13-18, 18-24, 24-30, 30-36 years). The results indicate that chewing difficulties are frequent and become increasingly present with age, associated with a progressive increase of the duration of meals. Episodes of choking or other clinical signs of swallowing difficulties are in contrast much less frequent even after age 18. Aspiration pneumonia were also not very frequent and only occurred in 7/118. Clinical signs of gastroesophageal reflux requiring treatment were only found in 5 while 43/118 complained of constipation requiring treatment. Very few of our patients had their weight above 2 SD (n = 4) and this was always found in patients between 9 and 18 years while after this age there was an increasing number of patients with weight below 2 SD. The results of our survey suggest that although choking is one of the most feared complications in patients with DMD, clinical signs of swallowing abnormalities are infrequent when collecting clinical information retrospectively. Further studies using an objective evaluation such as videofluoroscopy are needed to identify minor signs that may not be obvious on clinical examination.
本研究的目的是使用一份专门的问卷进行一项调查，以评估118例杜氏肌营养不良症（DMD）患者（年龄范围为13.80 - 35.8岁）的喂养困难、胃肠道受累情况和体重增加情况。所有答案都录入数据库，并将队列按年龄组（3 - 9岁、9 - 13岁、13 - 18岁、18 - 24岁、24 - 30岁、30 - 36岁）进行数据分析。结果表明，咀嚼困难很常见，且随着年龄增长愈发明显，同时进餐时间也逐渐延长。相比之下，即使在18岁以后，呛噎发作或其他吞咽困难的临床体征也少得多。吸入性肺炎也不常见，仅在118例中有7例发生。需要治疗的胃食管反流临床体征仅在5例中发现，而118例中有43例主诉有需要治疗的便秘。我们的患者中体重超过2个标准差的极少（n = 4），且这种情况总是出现在9至18岁的患者中，而在此年龄之后，体重低于2个标准差的患者数量逐渐增加。我们的调查结果表明，尽管呛噎是DMD患者最令人担忧的并发症之一，但回顾性收集临床信息时吞咽异常的临床体征并不常见。需要进一步使用诸如视频荧光透视等客观评估方法进行研究，以识别临床检查中可能不明显的轻微体征。
