OC92 Single centre experience of parenteral nutrition in patients with mitochondrial neurogastrointestinal encephalomyopathy (MNGIE) after liver transplant

• Published in: Frontline gastroenterology 2024-07, Vol.15 (Suppl 1), p.A62-A63
• Main Authors: Sandhu, Kirn, Coskun, Enes, Gurung, Sabrina, O’Leary, Kate, Hind, Jonathan, Dogra, Harween
• Format: Article
• Language: English
• Subjects: Abstracts; Glucose; Lipids; Liver transplants; Meals; Nitrogen; Nutrition; Nutritional status; Ostomy; Parenteral nutrition; Patients; Standard scores
• ISSN: 2041-4137; 2041-4145
• DOI: 10.1136/flgastro-2024-bspghan.88

Mitochondrial neurogastrointestinal encephalomyopathy (MNGIE) is a rare condition characterised by gastrointestinal dysmotility and neurological manifestations such as ptosis, external ophthalmoplegia, peripheral neuropathy and leukoencephalopathy. It is caused by mutations in the TYMP (thymidine phosphorylase) gene resulting in toxic levels of plasma thymidine causing deoxyribonucleoside imbalances impairing DNA replication and over-time disabling mitochondrial function.1 2 It typically occurs in the second decade and patients usually die from severe malnutrition and gastrointestinal (GI) complications.2 Current therapeutic strategies aim to normalise metabolic derangement by removing toxic metabolites or restoring thymidine phosphorylase activity. Hepatic tissue has a high expression of thymidine phosphorylase therefore liver transplant (LT) may restore the biochemical imbalance.3 We describe three patients with MNGIE in table 1 who have undergone LT for chronic liver disease but have persistent gastrointestinal dysmotility resulting in intestinal failure requiring parenteral nutrition(PN). Two of the patients (A and B) are siblings with B having a more severe phenotype. Attempts were made to feed the patients enterally via jejunal route but there were problems with recurrent nasojejunal, gastro-jejunal tubes displacement likely due to dysmotility and poor feed tolerance (high gastrostomy losses, vomiting and abdominal pain).Abstract OC92 Table 1Demographics of MNGIE patients Patient Age Sex Weight/BMI (Z-score) Age at LT Pre-transplant nutrition Enteral intake post transplant PN composition A 16yrs F 27.7 kg/ 12.75 kg/m2 (Z: -5.20) 15yrs Normal diet, fortisips 3 meals a day, snacks plus 100–200mls fortisips 1600ml over 12hrs PN 4 nights a week, nitrogen 0.28 g/kg/day glucose 6.9 g/kg/day, lipid 1.1 g/kg/day, PN providing 57% of EAR B 13yrs M 23.5 kg 11.1 kg/m2 (Z: -6.67) 12yrs Normal diet (trialled NG feeds, 250mls of Paediasure Compact with poor tolerance) 1 meal a day (chicken and rice) 600ml over 18hrs nitrogen 0.39 g/kg/day, glucose: 9.6 g/kg/day, lipid 1.5 g/kg/day (4x week) PN providing 102% of EAR C 18yrs M 41.6 kg/ 14.7 kg/m2 (Z: -4.04) 17yrs 1300ml Nutrini peptisorb energy with vitajoule via NG tube 1 bottle of Peptisip, orange juice, fizzy drinks, food intake can be 2–3 meals a day PN volume varies from lipid to non lipid days 2100/1700mls nitrogen 0.25 g/kg/day, glucose 8 g/kg/day, lipid 0.3 g/kg/day (3x week) PN providing 72% of EAR NG:nasog