背景:喂养问题在患有Barth综合征的男孩中很常见,并且可能导致人群生长延迟和肌肉无力的倾向。这项研究的目的是量化和描述这些喂养问题,并检查改变的味觉和感官敏感性作为影响因素。
方法:横截面,两组比较设计用于检查喂养偏好和行为,化学味觉感知,50名4-17岁(n=24)和没有(n=26)Barth的男孩的感觉敏感性。使用用苯基硫脲(PTC)和苯甲酸钠(NaB)饱和的化学测试条测量味觉。父母使用食物清单记录了喂养问题,而感觉敏感性是使用短感觉谱记录的。
结果:Barth的男孩在喂养行为问题方面与典型的同龄人有很大不同。对于和Barth在一起的男孩来说,食物拒绝和食物选择性被确定为存在于50%的样本中,而70%的人发现了与呕吐或吞咽食物有关的问题。大约一半的巴特家庭指出,他们的孩子的饮食习惯与家庭不符,并且经常准备单独的饭菜。正如先前的研究所证明的那样,约有50%的男孩在味觉/嗅觉敏感性方面表现出可能或明确的差异,明显高于对照组。在化学味觉感知测试中,有Barth的男孩更有可能成为PTC的超级品尝者和NaB的非品尝者。品尝者的状态与喂养问题的存在没有直接关系,然而,味道/气味敏感性确实与食物的类型和质地选择性显着相关。
结论:结果表明,至少50-70%的Barth综合征男孩存在喂养问题,并表明行为通常在6个月大之前就存在。味觉的差异可能会影响有Barth的男孩的饮食选择,特别是他们对咸食物的渴望。味觉/嗅觉敏感性似乎也会影响食物的选择性,因此,在这个人群中可能需要考虑,特别是考虑到饮食对心脏功能的影响,能源消耗,和整体增长。
BACKGROUND: Feeding problems are common in boys with Barth syndrome and may contribute to the population\'s propensity for growth delay and muscle weakness. The purpose of this study was to quantify and describe these feeding issues and examine altered taste perception and sensory sensitivity as contributing factors.
METHODS: A cross-sectional, two-group comparison design was used to examine feeding preferences and behaviors, chemical taste perception, and sensory sensitivities in fifty boys with (n=24) and without (n=26) Barth ages 4-17 years. Taste perception was measured using chemical test strips saturated with phenylthiocarbamide (PTC) and sodium benzoate (NaB). Feeding problems were documented by parents using a Food Inventory, while sensory sensitivities were recorded using a Short Sensory Profile.
RESULTS: Boys with Barth differed significantly from typical peers with regards to problem feeding behaviors. For boys with Barth, food refusal and food selectivity were identified as being present in 50% the sample, while 70% of had identified problems related to gagging or swallowing foods. About half of all Barth families noted that their child\'s eating habits did not match the family\'s and that separate meals were often prepared. As demonstrated in previous research, about 50% of boys with Barth demonstrated probable or definite differences in taste/smell sensitivity, which was significantly higher than controls. On tests of chemical taste perception, boys with Barth were significantly more likely to be supertasters to PTC and non-tasters to NaB. Taster-status did not directly relate to the presence of feeding problems, however, taste/smell sensitivity did significantly relate to food selectivity by type and texture.
CONCLUSIONS: Results indicate feeding problems in at least 50-70% of boys with Barth syndrome, and suggest that behaviors are often present before 6 months of age. Differences in taste perception may influence dietary choices in boys with Barth, particularly their craving of salty foods. Taste/smell sensitivity also appears to influence food selectivity, and therefore may be important to consider in this population, particularly in light of dietary influences on cardiac function, energy consumption, and overall growth.