Abnormal liver functions as a result of total parenteral nutrition in a patient with short-bowel syndrome

The pathogenesis of total parenteral nutrition (TPN)-induced liver cholestasis is poorly understood. Cholestasis generally occurs late in TPN therapy in association with elevated serum alkaline phosphatase and total bilirubin concentrations. Such factors as preexisting medical conditions, excessive nutrient infusion, amino-acid deficiency, absence of enteral stimulation, protracted duration of therapy, continuous infusion schedule, and hypoalbuminemia have all been suggested as possible etiologies. Various treatments have been proposed for the correction of TPN-induced cholestasis including administration of bile salt and antimicrobial therapies. To avoid potential hepatic complications associated with TPN, certain preventive measures can be considered. Administration of energy substrates should not be excessive. A mixed-fuel system that includes lipids should be implemented. TPN should be cycled if it will be used long term, and initiation of enteral nutrition should begin as soon as possible.     

Pregnancy in a patient with chronic intestinal pseudo-obstruction on long-term parenteral nutrition

Parenteral nutrition support is provided in most instances for short intervals during pregnancy in conditions where oral/enteral intake is severely compromised. Few reports describe the use of parenteral nutrition from conception to delivery. We report the case of a 30-year-old woman suffering from a severe form of chronic intestinal pseudo-obstruction on long-term parenteral nutrition because of malabsorption and malnutrition. Pregnancy and delivery developed uneventfully. The fetus grew normally throughout pregnancy. Our patient needed only slight modifications in her parenteral nutrition regimen during lactation. There were no metabolic complications during pregnancy. We conclude that female patients even with severe forms of gastrointestinal diseases, such as chronic intestinal pseudo-obstruction requiring long-term home parenteral nutrition, can conceive and carry successfully a pregnancy to term.     

Characterization of posthospital bloodstream infections in children requiring home parenteral nutrition

Background: Home parenteral nutrition (HPN) is lifesaving for children with intestinal failure. Catheter‐associated bloodstream infections (CA‐BSI) are common in hospitalized patients receiving parenteral nutrition (PN), but data evaluating CA‐BSI in children receiving HPN are limited. Objective: To determine the incidence and characteristics of CA‐BSI in children receiving HPN. Methods: Medical records of 44 children receiving HPN during a 3‐year period were reviewed. End points were CA‐BSI during the initial 6 months after discharge. CA‐BSI was defined as isolation of pathogens from blood requiring antimicrobial therapy. Results: The primary indication for HPN was short bowel syndrome (46%), and 59 BSI were documented during the initial 6 months of HPN in 29 (66%) children. Of CA‐BSI, polymicrobial infections accounted for 52%; gram‐positive, 29%; gram‐negative, 17%; and fungal, 2%. CA‐BSI incidence per 1000 catheter‐days was highest during the first month posthospital discharge (72 episodes; 95% confidence interval [CI], 45.4–109.6). CA‐BSI incidence density ratio for children receiving HPN for >90 days compared with those receiving HPN for <30 days was 2.2 (P < .05). Logistic regression revealed that Medicaid insurance and age <1 year were associated with increased risk for CA‐BSI (odds ratio [OR], 4.4 [95% CI, 1.13–16.99] and 6.6 [1.50–28.49], respectively; P < .05). Conclusions: The incidence of CA‐BSI in children receiving HPN is highest during the first month posthospital discharge. Strategies to address care in the immediate posthospital discharge period may reduce the burden of infectious complications of HPN.     

Renal function in patients on long-term home parenteral nutrition and in intestinal transplant recipients

ObjectiveA decrease of renal function was described in patients on long-term home parenteral nutrition (HPN) for benign intestinal failure. The risk for chronic renal failure (CRF) due to frequent episodes of dehydration despite optimal HPN, is an indication for intestinal transplantation (ITx). ITx is the solid organ transplant at highest risk for developing CRF. The aim of this study was to compare the prevalence and the probability of CRF occurring in adults on HPN and after ITx.MethodsA cross-sectional and retrospective follow-up study was carried out in 2011. Renal function was evaluated at cross-sectional and at time of starting HPN or ITx, by serum creatinine concentration (mg/dL) and estimated glomerular filtration rate (eGFR), according to the Modification of Diet in Renal Disease equation (mL·min·1.73 m²). CRF was defined as eGFR <60. Duration of follow up was from time of starting treatment to time of cross-sectional.ResultsWe enrolled 33 patients on HPN and 22 who had undergone ITx. The frequency of CRF was 6% in the HPN group and 9% in the ITx group (P = 0.67) at start of treatment, and 21% and 54%, respectively (P = 0.01) at the time of the cross-sectional evaluation. During the follow-up, the annual decline of eGFR was 2.8% and 14.5%, respectively (P = 0.02). The 5-y probability of maintaining an eGFR ≥60 was 84% in the HPN group and 44% in the ITx group (P < 0.001).ConclusionsThe decrease of renal function and the risk for developing CRF are greater after ITx than during HPN. The risk for CRF on HPN, as a criterion for ITx, should be revised.     

Maintenance of parenteral nutrition volume reduction, without weight loss, after stopping teduglutide in a subset of patients with short bowel syndrome

Background: Teduglutide was discontinued after being tested for ≥ 24 weeks in patients with parenteral nutrition (PN) ‐dependent short bowel syndrome in a clinical trial for efficacy to reduce PN volume. This study was describes change in body mass index (BMI) and PN volume over 12 months in patients who stopped drug after the clinical trial. Methods: Prescribed PN volume, weight, and complications were reported. Patients with stable (NEUT, n = 15) or decreased (DEC, n = 7) PN volume by 12 months after stopping drug (NEUT/DEC, n = 22) were compared to those who had increased PN volume (INC, n = 15). With drug response defined by ≥20% reduction from pre‐drug PN volume to end of drug therapy, 12 INC and 13 NEUT/DEC patients were drug responders. Results: Eleven of 20 eligible sites reported data for 39 of 53 eligible study participants, with follow‐up data for 37. INC patients had shorter colon and less frequently had colon in continuity than NEUT/DEC. BMI was decreased at 3, 6, and 12 months relative to the first off‐drug visit in INC patients (P = .001), but not in NEUT/DEC patients. Change in BMI off‐drug was predicted by colon and small bowel length, baseline BMI, and on‐drug change in PN volume (adjusted R2 = 0.708). Conclusions: Gastrointestinal anatomy, baseline BMI, and PN volume reduction on‐drug predicted change in BMI off‐drug. Whether this response would be maintained for a longer time or in the context of a challenging clinical situation has not been evaluated.     

ASPEN definitions in pediatric intestinal failure

Pediatric intestinal failure (PIF) is a relatively rare disease entity that requires focused interdisciplinary care and specialized nutrition management. There has long been a lack of consensus in the definition of key terms related to PIF because of its rarity and a plethora of small studies rather than large trials. As such, the American Society for Parenteral and Enteral Nutrition (ASPEN) PIF Section, composed of clinicians from a variety of disciplines caring for children with intestinal failure, is uniquely poised to provide insight into this definition void. This document is the product of an effort by the Section to create evidence-based consensus definitions, with the goal of allowing for appropriate comparisons between clinical studies and measurement of long-term patient outcomes. This paper has been approved by the ASPEN Board of Directors.