Prognostic factors for pediatric patients with severe intestinal motility disorders: a single institution's
Keisuke Yano1, Mitsuru Muto1, Koshiro Sugita1
1Department of Pediatric Surgery, Research Field in Medical and Health Sciences, Medical and Dental Area, Research and Education Assembly, Kagoshima University, Kagoshima, Japan.
Insights
Severe pediatric intestinal motility disorder (IMD) has a high mortality rate. Cholestasis is a key prognostic factor, highlighting the need to manage liver disease and infections associated with long-term nutrition support.
Area of Science:
- Pediatric Gastroenterology
- Intestinal Motility Disorders
- Nutritional Support
Background:
- Severe intestinal motility disorder (IMD) in children poses significant health challenges.
- Long-term total parenteral nutrition (TPN) is often required for management, carrying associated risks.
Purpose of the Study:
- To determine prognostic factors for severe pediatric intestinal motility disorder (IMD).
- To identify predictors of outcomes in children with severe IMD requiring prolonged TPN.
Main Methods:
- Retrospective review of medical records for 14 pediatric patients with severe IMD requiring TPN for ≥60 days.
- Analysis of patient characteristics, IMD types (isolated hypoganglionosis, extensive aganglionosis, chronic idiopathic intestinal pseudo-obstruction), and outcomes.
Main Results:
- Cholestasis was identified as a significant negative prognostic factor (p=0.005).
- Mortality rate was high at 50%, with intestinal failure-associated liver disease (IFALD) and catheter-related blood stream infections (CRBSI) being primary causes of death.
- No significant difference in mortality was observed among the different types of severe IMD.
Conclusions:
- Severe IMD remains a condition with high mortality.
- Cholestasis is a critical predictor of prognosis in pediatric severe IMD.
- Preventing and managing IFALD and CRBSI associated with long-term TPN is crucial for improving survival rates.
Purpose:
To identify the prognostic factors for pediatric severe intestinal motility disorder (IMD).
Methods:
We reviewed the medical records of patients with severe IMD, who required total parenteral nutrition (TPN) for ≥ 60 days at our institution between April, 1984 and March, 2023, examining their characteristics to identify prognostic factors.
Results:
The types of IMD in the 14 patients enrolled in this study were as follows: isolated hypoganglionosis (IHG, n = 6), extensive aganglionosis (EAG: n = 6), and chronic idiopathic intestinal pseudo-obstruction (CIIP, n = 2). There was no significant difference in mortality among the three types of severe IMD. Weaning-off TPN and the use of the colon were not significant prognostic factors, but cholestasis was a significant prognostic factor (p = 0.005). There was a high mortality rate (50%), with the major causes of death being intestinal failure-associated liver disease (IFALD) following hepatic failure, and catheter-related blood stream infection (CRBSI). One IHG patient underwent small bowel transplantation but died of acute rejection.
Conclusion:
Severe IMD is still associated with a high mortality rate and cholestasis predicts the prognosis. Thus, preventing or improving IFALD and CRBSI caused by long-term TPN is important for reducing the mortality rate.
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