Armellino, A.

Abstract Home parenteral nutrition (HPN) is a life‐sustaining therapy traditionally used as a bridge to enteral autonomy or intestinal transplantation. Increasingly, it is used for intractable feeding intolerance (IFI), which can occur near the end of life (EOL) in children with severe neurological impairment (SNI). In these cases, HPN use differs from its historical role and requires tailored outpatient planning. We report a retrospective case series of four children with SNI who developed IFI and received HPN as part of their EOL care. The pediatric palliative care team (PPCT) was involved in all cases during HPN decision‐making and planning. The pediatric nutrition support team (PNST) and PPCT collaborated to provide individualized, goal‐directed care. Patients ranged from 1 to 12 years old, with HPN durations between 5 weeks and 2 years. All were enrolled in hospice while receiving HPN. Plans were made through shared decision‐making, reflecting each family's values, and included tailored approaches to laboratory monitoring, febrile episodes, line care, growth assessment, and follow‐up. HPN at EOL requires careful ethical consideration, particularly of autonomy because families often find comfort in providing nutrition. IFI is often unanticipated, and prognoses are uncertain. With the combined expertise of PNST and PPCT, HPN can be used meaningfully at EOL, supporting family values, easing suffering, and creating space for peace and connection.

AbstractCongenital nephrotic syndrome (CNS) is a complex condition that requires multidisciplinary care. Hyperlipidemia is a characteristic feature with elevation of serum cholesterol and triglycerides. Little evidence is available to guide treatment of dyslipidemia in infants with CNS. We describe successful treatment of severe hypertriglyceridemia through dietary changes in a boy with CNS. A 9‐day‐old boy presented to the emergency department with lower extremity edema caused by deep venous thrombosis. Laboratory evaluation identified hypoalbuminemia, nephrotic‐range proteinuria, and a pathogenic variant of the NPHS1 gene. The initial triglyceride concentration of 369 mg/dl increased to 3096 mg/dl by 5 weeks of age, when his diet consisted of breast milk. Refrigerated breast milk was skimmed by removing the top layer after allowing it to separate for 24 h. This process was repeated prior to use. Skimmed breast milk was supplemented with medium‐chain triglyceride oil and an infant protein powder. After 2 days, the triglyceride concentration declined to 481 mg/dl and, by day 10, to 148 mg/dl. When breast milk supply decreased, a 1:1 ratio of skimmed maternal breast milk to an elemental, very low‐fat formula was utilized. The triglyceride concentration remained below 400 mg/dl for the first year of life, except when skimmed breast milk was not available during hospitalization. Severe hypertriglyceridemia caused by CNS can present in the neonatal period and be difficult to manage. In our patient, skimmed maternal breast milk was successful in reducing the triglyceride concentration and should be considered a therapeutic option for children with hyperlipidemia caused by CNS.