Use of an In‐line Digestive Cartridge With Enteral Nutrition Improves the Weight Trajectory of 2 Children With Cystic Fibrosis Complicated by Another Medical Diagnosis

Giguere-Rich, Catherine; Mathew, Amy; Reid, Elizabeth; Autore, Kimberly; Guill, Margaret F.

doi:10.1002/ncp.10080

Cited by 7 publications

(18 citation statements)

References 26 publications

(81 reference statements)

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“…This mandates that the clinician has a high index of suspicion for this condition and highlights the importance of early laboratory evaluation . Growth failure in EPI is often multifactorial and is attributed to the mismatch between reduced intake, intestinal malabsorption and excessive losses, and increased energy needs . Most of the inherited syndromes with EPI are multisystem disorders, and the presence of extra‐GI manifestations will also help direct the diagnosis (Table ).…”

Section: Clinical Presentation and Laboratory Investigationsmentioning

confidence: 99%

“…All current pancrelipase preparations are porcine in origin and available in various formulations (eg, capsules with enteric‐coated beads vs non–enteric‐coated tablets). Most preparations are capsules containing enteric‐coated microspheres or microtablets that are intended to release the enzymes in a pH milieu of 5.5–6 within the proximal small bowel . The capsules must be swallowed without chewing.…”

Section: Treatmentmentioning

confidence: 99%

“…Energy recommendations for CF patients with EPI are 110%–200% of the caloric requirements of healthy children of the same age, with 30%–40% of the calories from fat . Energy needs are adjusted based on individual weight‐gain patterns.…”

Section: Treatmentmentioning

confidence: 99%

“…Beyond infancy, high‐energy formulas with 1.5–2 kcal/mL are usually preferred . Evidence‐based guidelines for administration of PERT for enteral tube feeding have not been established . Current consensus on PERT management includes oral administration of PERT prior to the bolus feeds; however, at the time the guidelines were written, there were no recommended methods for enzyme administration with nocturnal tube feedings .…”

Section: Treatmentmentioning

confidence: 99%

“…The practice of mixing enzymes with the enteral feeds can potentially lead to tube occlusion and inconsistent enzyme delivery . A digestive lipase cartridge (RELiZORB, Alcresta Therapeutics, Inc., Newton, MA, USA) was introduced in 2015 and is approved for use in children (>5 years of age) and adults . This cartridge has also been used in younger children.…”

Section: Treatmentmentioning

confidence: 99%

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Management of Exocrine Pancreatic Insufficiency in Children

2019

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The diagnosis of exocrine pancreatic insufficiency (EPI) can be difficult, as symptoms may be nonspecific. A delayed diagnosis of EPI can negatively impact health through poor weight gain, impaired growth, and malabsorption of nutrients. Because of active growth and development, children are more vulnerable to the consequences of untreated EPI. Pancreatic enzyme replacement therapy is the cornerstone of management and offers both symptomatic relief and improvement in clinical outcomes. Additionally, a high-energy diet with unrestricted fat and supplementation with fat-soluble vitamins is often required to optimize growth and prevent nutrition deficiencies. Cystic fibrosis (CF) is the most common condition in children that causes EPI, and improvement in nutrition management is associated with improved pulmonary function and increased survival. Currently, the management of other conditions leading to EPI in children is not well studied, and inferences from the CF literature are often necessary in caring for these patients. (Nutr Clin Pract. 2019;34(suppl 1):S27-S42)

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Section: Clinical Presentation and Laboratory Investigationsmentioning

confidence: 99%

Section: Treatmentmentioning

confidence: 99%

Section: Treatmentmentioning

confidence: 99%

Section: Treatmentmentioning

confidence: 99%

Section: Treatmentmentioning

confidence: 99%

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Management of Exocrine Pancreatic Insufficiency in Children

2019

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Improvements in anthropometric measures and gastrointestinal tolerance in patients with cystic fibrosis by using a digestive enzyme cartridge with overnight enteral nutrition

Hendrix

Flume

First³

et al. 2022

Nut in Clin Prac

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Background: Patients with cystic fibrosis (CF) and pancreatic insufficiency are at risk for suboptimal fat absorption, inability to maintain weight, poor growth, and increased gastrointestinal (GI) symptoms due to malabsorption. Enteral nutrition (EN) is used to supplement caloric intake and requires pancreatic enzyme replacement for effective digestion. We evaluated the relationship between long-term use of an in-line digestive enzyme cartridge with EN and changes in anthropometric measures and GI symptoms in patients with CF.Methods: This is a single-center, retrospective case review of patients with CF using a digestive enzyme cartridge with EN. Data were collected from the patient medical records and included weight, height, body mass index, EN regimen, and reported GI symptoms.Results: Thirteen pediatric and five adult patients with a mean age of 12.6 years used a digestive enzyme cartridge with EN for a period of 3-27 months. Most patients (n = 14) had been using oral digestive enzymes with EN before using the digestive enzyme cartridge, whereas four started from the onset of EN. The indications to convert from oral enzymes to the digestive enzyme cartridge included poor growth (72.2%) and poor tolerance of EN (69.2%). There was a reduction in reported GI symptoms after initiating use of the digestive enzyme cartridge.After 12 months of digestive cartridge use, there were improvements in anthropometrics.Conclusions: This real-world experience with prolonged use of a digestive enzyme cartridge with EN demonstrated improved clinical outcomes and a reduction in GI symptoms in patients with CF.

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Association of in‐line digestive enzyme cartridge with enteral feeds on improvement in anthropometrics among pediatric patients with cystic fibrosis

Shrivastava,

Shaw,

Lee

et al. 2024

Nut in Clin Prac

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BackgroundApproximately 85% of patients with cystic fibrosis (CF) have exocrine pancreatic insufficiency (EPI) with 10% requiring supplemental nighttime enteral tube feedings. Administration of pancreatic enzyme replacement therapy (PERT) with nighttime feedings is fraught with challenges. RELiZORB (Alcresta Therapeutics, Inc), an in‐line lipase cartridge, delivers PERT continuously with enteral feedings. Outcomes related to the use of this in‐line lipase cartridge are lesser known. This project evaluated anthropometrics related to in‐line lipase cartridge use among pediatric patients with CF already receiving oral PERT therapy prior to nighttime enteral feedings.MethodsRetrospective chart review was performed on 29 patients with CF and EPI receiving supplemental tube feedings and utilizing in‐line lipase cartridge for a continuous 12 month period between 2015 and 2019. Anthropometrics were evaluated 12 months before and after initiation of in‐line lipase cartridge.ResultsCompared with mean height z score at 6‐months pre–in‐line lipase cartridge, mean height z score at 6‐months post–in‐line‐lipase cartridge (adjusted mean difference [AMD] = 0.2540; 95% CI = [0.0487, 0.4592]; P = 0.0153) and mean height z score at 12‐months post–in‐line lipase cartridge (AMD = 0.2684; 95% CI = [0.0203, 0.5166]; P = 0.0340) were significantly higher. Mean weight z score at 12‐months post–in‐line‐lipase‐cartridge neared statistical significance compared with 6‐months pre–in‐line lipase cartridge (AMD = 0.2816; 95% CI = [−0.0003, 0.5634]; P = 0.0502) when excluding seven patients with advanced lung disease (forced expiratory volume in the first second of expiration of 40%). Weight‐for‐length or body mass index did not significantly differ compared with pre–in‐line lipase cartridge.ConclusionUse of in‐line lipase cartridge with enteral feeds improved anthropometrics, especially height, in pediatric patients with CF.

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Use of an In‐line Digestive Cartridge With Enteral Nutrition Improves the Weight Trajectory of 2 Children With Cystic Fibrosis Complicated by Another Medical Diagnosis

Cited by 7 publications

References 26 publications

Management of Exocrine Pancreatic Insufficiency in Children

Management of Exocrine Pancreatic Insufficiency in Children

Improvements in anthropometric measures and gastrointestinal tolerance in patients with cystic fibrosis by using a digestive enzyme cartridge with overnight enteral nutrition

Association of in‐line digestive enzyme cartridge with enteral feeds on improvement in anthropometrics among pediatric patients with cystic fibrosis

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