Home Parenteral Nutrition for Children: What Are the Factors Indicating Dependence and Mortality?

Abstract

Parenteral nutrition (PN) in children with short bowel syndrome is crucial and lifesaving. Taking care of such patients requires interprofessional practice and multiple team resource management. Home PN (HPN) usage allows patients and families to live regular lives outside hospitals. We share our experiences for the last two decades and identify the risk factors for complications and mortality. A retrospective study of HPN patients was conducted between January 2000 and February 2022. Medical records of age, body weight, diagnosis, length of residual intestines, HPN period, central line attempts, complications, weaning, and survival were collected and analyzed. The patients were classified as HPN free, HPN dependent, and mortality groups. A total of 25 patients received HPN at our outpatient clinic, and one was excluded for the adult age of disease onset. There were 13 patients (54.1%) who were successfully weaned from HPN until the record-enroled date. The overall mortality rate was 20.8% (five patients). All mortality cases had prolonged cholestasis, Child Class B or C, and a positive Pediatric End-Stage Liver Disease (PELD) score. For HPN dependence, extended resection and multiple central line placement were two significant independent factors. Cholestasis, Child Class B or C, and positive PELD score were the most important risk factors for mortality. The central line-related complication rate was not different in all patient groups. The overall central line infection rate was 1.58 per 1000 catheter days. Caution should be addressed to prevent cholestasis and intestinal failure-associated liver disease during the HPN period, to prevent mortality. By understanding the risks of HPN dependence and mortality, preventive procedures could be addressed earlier.

Keywords: Pediatric End-Stage Liver Disease (PELD) score; catheter-related bloodstream infection; central line-associated bloodstream infection (CLABI); intestinal failure-associated liver disease (IFALD); parenteral nutrition; parenteral nutrition-associated cholestasis (PNAC); parenteral nutrition-associated liver disease (PNALD).

Figure 1

The scatter diagram between small intestinal length and catheter-related infection times. Correlation coefficient, r = −0.237 (−0.584 to 0.183) and p = 0.263. Trend line by locally weighted scatterplot smoothing and span = 60%.

Figure 2

(a) Kaplan–Meier survival curve of all-cause mortality in HPN patients. (b) Comparison between HPN-free and HPN-dependent patients (HPN-dependent and mortality groups). It was noted than the overall survival was significantly longer in HPN-free patients than in HPN-dependent patients (logrank test: p = 0.003).

Figure 3

Kaplan–Meier estimated survival rates of pediatric patients with HPN, considering the different risk factors: (a) major colon loss (>50%), (b) residual small intestine length <10% predicted, (c) Child-Turcotte-Pugh Classification, and (d) Pediatric End-stage Liver Disease (PELD) score. A, B, and C stand for Child Class A, B, and C, respectively.