小儿肠衰竭患者的青春期和生长。

Puberty and growth in patients with pediatric intestinal failure.

机构信息

Department of Surgery, Boston Children's Hospital and Harvard Medical School, Boston, MA, United States of America; Center for Advanced Intestinal Rehabilitation, Boston Children's Hospital and Harvard Medical School, Boston, MA, United States of America.

Department of Surgery, Boston Children's Hospital and Harvard Medical School, Boston, MA, United States of America.

出版信息

J Pediatr Surg. 2022 Jun;57(6):1045-1049. doi: 10.1016/j.jpedsurg.2022.01.057. Epub 2022 Feb 14.

DOI:10.1016/j.jpedsurg.2022.01.057

PMID:35287963

Abstract

BACKGROUND

Pediatric intestinal failure (PIF) affects nutrition, metabolism, and endocrine development, but its downstream impact on puberty is unknown.

METHODS

A retrospective review was performed of patients age >8 years with PIF managed at an intestinal rehabilitation program. Outcomes of interest were peak height velocity (PHV), age at PHV, and age at pubertal onset (Tanner stage 2). Outcomes were stratified by sex and compared to established norms.

RESULTS

Of 110 patients with PIF, 54.5% were male. Compared to the CDC 50th percentile, PHV in PIF patients was similar for females (8.09±2.36 vs. 7.37 cm/yr;p = 0.23) but significantly higher for males (9.27±2.56 vs. 7.91 cm/yr;p = 0.038). Age at PHV in PIF patients was significantly younger for both males (12.31±2.14 vs. 13.38 years;p = 0.049) and females (10.70±1.06 vs. 11.71 years;p = 0.001). PIF patients reached pubertal onset earlier than published norms; this was significant for males (12.41±1.80 vs. 13.44 years;p = 0.014), but not for females (10.45±1.81 vs. -11.15 years;p = 0.13). The mean height-for-age Z-score was -1.2, with 20% of patients having a Z-score less than -2.

CONCLUSIONS

Pubertal onset and growth are neither delayed nor diminished in patients with PIF. The high incidence of short stature, however, highlights the importance of optimizing prepubertal linear growth to attain full height potential.

TYPE OF STUDY

Prognosis study (Retrospective cohort study).

摘要

背景

儿科肠衰竭（PIF）会影响营养、代谢和内分泌发育，但它对青春期的下游影响尚不清楚。

方法

对在肠康复计划中接受治疗的年龄>8 岁的 PIF 患者进行回顾性分析。感兴趣的结果是峰值身高速度（PHV）、PHV 年龄和青春期开始年龄（Tanner 分期 2）。根据性别对结果进行分层，并与既定标准进行比较。

结果

在 110 例 PIF 患者中，54.5%为男性。与 CDC 第 50 百分位相比，PIF 患者的 PHV 在女性中相似（8.09±2.36cm/yr；p=0.23），但在男性中显著更高（9.27±2.56cm/yr；p=0.038）。PIF 患者的 PHV 年龄在男性（12.31±2.14 岁比 13.38 岁；p=0.049）和女性（10.70±1.06 岁比 11.71 岁；p=0.001）中均明显更年轻。PIF 患者达到青春期开始的年龄早于公布的标准；这在男性中是显著的（12.41±1.80 岁比 13.44 岁；p=0.014），但在女性中不显著（10.45±1.81 岁比 -11.15 岁；p=0.13）。身高年龄 Z 评分的平均值为-1.2，有 20%的患者 Z 评分小于-2。