Isojima Tsuyoshi, Sakazume Satoru, Hasegawa Tomonobu, Ogata Tsutomu, Nakanishi Toshio, Nagai Toshiro, Yokoya Susumu
Department of Pediatrics, Teikyo University School of Medicine, Tokyo, Japan.
Department of Pediatrics, Dokkyo Medical University Koshigaya Hospital, Koshigaya, Japan.
Clin Pediatr Endocrinol. 2017;26(3):153-164. doi: 10.1297/cpe.26.153. Epub 2017 Jul 27.
We recently published growth references for Japanese individuals with Noonan syndrome (NS). However, it is uncertain whether these references can be used to evaluate the longitudinal growth of children with NS. In addition, these charts did not include detailed values suitable for clinical practice, and they did not include weight-for-height (WFH) charts. In the present study, we validated the references and established new WFH charts for children with NS. In addition, we investigated the growth patterns of these children by comparing them with those of children with Turner syndrome (TS), as well as with those of the normal population. To validate our reference values, we enrolled 32 subjects from our previous study with data available at both a younger (≤ 5 yr) and an older age (≥ 15 yr). We then investigated longitudinal changes in NS-specific standard deviation scores (SDSs) for height in these subjects. There was no significant difference between the initial and later SDSs (mean difference: -0.12, 95% confidence interval: -0.26-0.023, = 0.10), suggesting that the references could be applied in clinical practice. We also confirmed that the growth patterns of children with NS in each index are significantly different from those of children with TS. In conclusion, we confirmed auxological reference values for Japanese children with NS.
我们最近公布了日本努南综合征(NS)患者的生长参考标准。然而,这些参考标准能否用于评估NS患儿的纵向生长尚不确定。此外,这些图表没有包含适合临床实践的详细数值,也没有包含身高别体重(WFH)图表。在本研究中,我们验证了这些参考标准,并为NS患儿建立了新的WFH图表。此外,我们通过将这些患儿与特纳综合征(TS)患儿以及正常人群进行比较,研究了他们的生长模式。为了验证我们的参考值,我们从之前的研究中招募了32名在较年幼(≤5岁)和较年长(≥15岁)时均有数据的受试者。然后,我们调查了这些受试者中NS特异性身高标准差评分(SDS)的纵向变化。初始SDS和后期SDS之间无显著差异(平均差异:-0.12,95%置信区间:-0.26至0.023,P = 0.10),这表明这些参考标准可应用于临床实践。我们还证实,NS患儿各指标的生长模式与TS患儿显著不同。总之,我们确定了日本NS患儿的体格学参考值。