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First‐year growth in children with Noonan syndrome: Associated with feeding problems?

机译:Noonan综合征儿童的一年生长:与喂养问题有关吗?

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Children with Noonan syndrome show rapid decline of growth in the first year of life and feeding problems are present in over 50%. The aim of this study was to explore whether growth decelerates because of feeding problems or other Noonan syndrome‐related factors. We performed a retrospective, longitudinal cohort study of clinically and genetically diagnosed subjects with Noonan syndrome ( n ?=?143). Questionnaires about the phenotypic–genotypic profile and reported feeding problems were sent to eligible subjects. Data on first‐year growth was obtained from growth charts. Ninety‐one participants were excluded because of different criteria. A total of 52 subjects with Noonan syndrome were included. The largest decline in weight and length standard deviation score (SDS) occurred in the first 2.5 months after birth (?1.93 and ?1.15, respectively), with feeding problems causing a decline of 0.57 SDS in the remaining months. At 1 year, children with feeding problems were on average 290 g lighter and 0.8 cm shorter than children without feeding problems. Weight gain was also negatively influenced by having a PTPN11 mutation ( n ?=?39) and a higher gestational age, whereas children of parents with Noonan syndrome and with a higher birth weight gained more weight. Growth in length was reduced by having cardiac surgery and a higher gestational age, but positively influenced by birth length and maternal height. Growth in children with Noonan syndrome is impaired right after birth and only partially associated with feeding problems. In addition, several specific Noonan syndrome‐related factors seem to influence growth in the first year.
机译:Noonan综合征的儿童表现出生命的第一年的增长迅速下降,饲养问题超过50%。本研究的目的是探讨是否因为喂养问题或其他日死综合征相关因素而逐渐减缓。我们对Noonan综合征进行了临床和转基因受试者进行了回顾性,纵向队列研究(N?=?143)。关于表型 - 基因型外形的问卷和报告的饲养问题被送到符合条件的受试者。从生长图表中获得了一年增长的数据。由于标准不同,排除了九十一位参与者。共纳了共有52名中非南综合征的受试者。在出生后的前2.5个月内最大的重量和长度标准偏差评分(SDS)(分别为1.93和?1.15)发生,饲养问题导致剩余月份下降0.57秒。在1年,饲养问题的儿童平均平均290克更轻,比儿童短0.8厘米,而不会喂食问题。重量增益也受到PTPN11突变(N?= 39)和更高的孕龄的负面影响,而Noonan综合征的父母的孩子患有更高的产量较高。通过心脏手术和更高的妊娠年龄,长度的生长减少,但受出生长度和母体高度的积极影响。在出生后,Noonan综合征的儿童的生长受损,并且仅部分与喂养问题有关。此外,几种特定的中午综合征相关因素似乎在第一年影响了增长。

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