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

Katherine Culbreath ¹, Gregory Keefe ¹, Steven J Staffa ², Nicole Wynne ², Frances Grimstad ³, Lissette Jimenez ⁴, Tom Jaksic ¹, Biren P Modi ⁵

Author information

¹Department 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.

²Department of Surgery, Boston Children's Hospital and Harvard Medical School, Boston, MA, United States of America.

³Department of Surgery, Boston Children's Hospital and Harvard Medical School, Boston, MA, United States of America; Division of Gynecology, Boston Children's Hospital and Harvard Medical School, Boston, MA, United States of America.

⁴Center for Advanced Intestinal Rehabilitation, Boston Children's Hospital and Harvard Medical School, Boston, MA, United States of America; Division of GI/Nutrition, Boston Children's Hospital and Harvard Medical School, Boston, MA, United States of America.

⁵Department 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. Electronic address: biren.modi@childrens.harvard.edu.

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).