Dietetic Department, Birmingham Children's Hospital, Birmingham, UK.
J Hum Nutr Diet. 2012 Jun;25(3):209-16. doi: 10.1111/j.1365-277X.2012.01229.x. Epub 2012 Feb 9.
In children with inherited metabolic disorders (IMD), feeding difficulties are often assumed to be inherent, although there is little evidence describing their frequency or severity. The present study aimed to describe feeding patterns/difficulties among children with IMD on protein-restricted diets from one centre.
Data from an observational, pilot study of 20 IMD children, nine females (median age, 2.7 years; range, 1-6 years) were compared with data obtained from a retrospective historical group of 15 healthy children (HC), 12 females, aged 1-5 years (median 3.0 years). Caregivers completed a feeding assessment questionnaire, and three separate video recordings were taken of each child eating at home.
The main feeding problems identified by the caregivers' questionnaire in the IMD group (compared to HC) were: poor appetite (55% versus 7%; P = 0.004), limited food variety (55% versus 27%; P = 0.04) and lengthy mealtimes (70% versus 20%; P = 0.006). During mealtimes, children from the IMD group were more likely to vomit, exhibit negative behaviour, get distracted and self-feed less often. From video recordings of meals, although the median meal duration was similar for the two groups (18 min IMD versus 16 min HC), the HC ate twice the quantity of food (3.4 mouthfuls min(-1) versus 1.5 mouthfuls min(-1) ; P < 0.001). During mealtimes, IMD caregivers were less likely to talk to their children (median parent to child communications: IMD group, seven in 10 min; HC, 17 in 10 min). Eighty-three percent of IMD children regularly ate alone.
In children with IMD on protein restrictions, severe feeding difficulties were common. Caregivers need to focus more attention on the social aspects of feeding. Further larger scale studies are required.
在患有遗传性代谢疾病（IMD）的儿童中，喂养困难通常被认为是与生俱来的，尽管很少有证据描述其频率或严重程度。本研究旨在描述来自一个中心的接受蛋白质限制饮食的 IMD 儿童的喂养模式/困难。
对 20 名 IMD 儿童（9 名女性，中位数年龄为 2.7 岁；范围为 1-6 岁）的观察性、试点研究的数据与来自 15 名健康儿童（HC）的回顾性历史组数据进行了比较，该组 12 名女性，年龄为 1-5 岁（中位数 3.0 岁）。护理人员完成了喂养评估问卷，并在家中对每个孩子进行了三次单独的进食录像。
在 IMD 组中，护理人员通过问卷确定的主要喂养问题（与 HC 相比）为：食欲不振（55%比 7%；P=0.004）、食物种类有限（55%比 27%；P=0.04）和用餐时间长（70%比 20%；P=0.006）。在用餐期间，IMD 组的儿童更容易呕吐、表现出消极行为、分心和自我进食频率较低。从进餐的录像来看，虽然两组的平均进餐时间相似（IMD 组 18 分钟，HC 组 16 分钟），但 HC 组进食的食物量是 IMD 组的两倍（IMD 组每分钟 3.4 口，HC 组每分钟 1.5 口；P<0.001）。在用餐期间，IMD 组的护理人员与孩子交流的次数较少（中位数：IMD 组，每 10 分钟 7 次；HC 组，每 10 分钟 17 次）。83%的 IMD 儿童经常独自进食。
在接受蛋白质限制饮食的 IMD 儿童中，严重的喂养困难很常见。护理人员需要更加关注喂养的社交方面。需要进一步进行更大规模的研究。
