RESUMO
BACKGROUND: Neuroendocrine tumours (NETs) are neoplastic yet behave differently to typical cancers. Despite often being slow growing, they can lead to significant gastrointestinal complications including intestinal failure (IF). The use of home parenteral nutrition (HPN) in neoplastic conditions is rising, primarily for palliation and bridging through treatments for cancer, but remains a challenging decision with a paucity of high-grade evidence-based guidance. METHODS: A retrospective analysis of patients with NET on HPN was performed. Data collected included the cause of IF, complications encountered with HPN and changes in nutritional assessments. RESULTS: Eight patients were identified, all with metastatic NET. Median weight improved following HPN commencement and line sepsis was the sole complication. All patients had stabilisation and optimisation of nutritional and hydration status. CONCLUSIONS: HPN is commenced to improve or maintain patients' nutritional status during often lifelong treatment. The principle aim in providing HPN was to improve survival and quality of life. While NETs are cancers, our case series demonstrates the potential of HPN to actively support longer term survival in the subgroup of patients who develop IF.
RESUMO
Polyglucosan inclusion body myopathy (PIBM) is a recently described gastrointestinal neuromuscular disease. Given its rarity, very little is known of its natural history and prognosis. In this case report, we present the first case of PIBM and resultant intestinal failure in which, following 7 years of home parenteral nutrition, gastrointestinal symptoms subsided and nutrition autonomy was restored.