Nutritional Status at Diagnosis, During, and After Treatment in Adolescents and Young Adults with Cancer.

Purpose: Malnutrition is commonly observed during cancer treatment, while some cancer survivors are at risk of overweight and obesity. This study investigated nutritional status during and after treatment in adolescents and young adults (AYA) with cancer. Methods: A retrospective chart review of AYA diagnosed with cancer was conducted. Data were collected monthly during treatment, then annually for 3 years of follow-up. Results: Of 93 AYA, 8% were underweight at diagnosis versus 20% during treatment (p = 0.012). Forty-four percent experienced ≥5% loss of weight (LOW) during treatment, and 23% of those were not referred to a dietitian. While 47% were referred to a dietitian at some point during treatment, 77% did not have dietetic involvement in the month after reaching greatest percentage LOW. Different tumor types were associated with different risks of LOW. Eighty-six percent with acute lymphoblastic leukemia (ALL)/lymphoblastic lymphoma (LL) and 86% with acute myeloid leukemia had ≥5% LOW during treatment, compared with 17% with Hodgkin lymphoma (p < 0.0001). In year 3 of follow-up, 36% of all AYA were overweight or obese versus 25% at diagnosis (p = 0.2). Overweight/obesity was more common in ALL/LL survivors than other tumor types (67% vs. 14%, p = 0.037). No patients had dietitian involvement in year 3 of follow-up. Conclusions: AYA, particularly those with ALL/LL, are at risk of significant weight loss during treatment and overweight and obesity during survivorship. Dietetic involvement was inconsistent in this cohort. These data may guide which diagnoses warrant preemptive dietetic input during treatment and highlight the importance of dietetic involvement in survivorship.

New insights into carnitine-acylcarnitine translocase deficiency from 23 cases: Management challenges and potential therapeutic approaches.

Carnitine acyl-carnitine translocase deficiency (CACTD) is a rare autosomal recessive disorder of mitochondrial long-chain fatty-acid transport. Most patients present in the first 2 days of life, with hypoketotic hypoglycaemia, hyperammonaemia, cardiomyopathy or arrhythmia, hepatomegaly and elevated liver enzymes. Multi-centre international retrospective chart review of clinical presentation, biochemistry, treatment modalities including diet, subsequent complications, and mode of death of all patients. Twenty-three patients from nine tertiary metabolic units were identified. Seven attenuated patients of Pakistani heritage, six of these homozygous c.82G>T, had later onset manifestations and long-term survival without chronic hyperammonemia. Of the 16 classical cases, 15 had cardiac involvement at presentation comprising cardiac arrhythmias (9/15), cardiac arrest (7/15), and cardiac hypertrophy (9/15). Where recorded, ammonia levels were elevated in all but one severe case (13/14 measured) and 14/16 had hypoglycaemia. Nine classical patients survived longer-term-most with feeding difficulties and cognitive delay. Hyperammonaemia appears refractory to ammonia scavenger treatment and carglumic acid, but responds well to high glucose delivery during acute metabolic crises. High-energy intake seems necessary to prevent decompensation. Anaplerosis utilising therapeutic d,l-3-hydroxybutyrate, Triheptanoin and increased protein intake, appeared to improve chronic hyperammonemia and metabolic stability where trialled in individual cases. CACTD is a rare disorder of fatty acid oxidation with a preponderance to severe cardiac dysfunction. Long-term survival is possible in classical early-onset cases with long-chain fat restriction, judicious use of glucose infusions, and medium chain triglyceride supplementation. Adjunctive therapies supporting anaplerosis may improve longer-term outcomes.

Highlights from the nutrition guidelines for cystic fibrosis in Australia and New Zealand.

Optimal nutrition care is important in the management of cystic fibrosis (CF). This paper summarises the '2017 Nutrition Guidelines for Cystic Fibrosis in Australia and New Zealand (NZ)'. CF dietitians formulated 68 practice questions which were used to guide a systematic literature search and review of the evidence for nutrition in CF. Identified papers underwent quality and evidence assessment using the American Dietetic Association quality criteria checklist and the National Health and Medical Research Council of Australia (NHMRC) rankings. Evidence statements, graded recommendations and practice points were developed covering core nutrition topics (assessment and nutrition interventions including oral, enteral and micronutrient supplementation); nutrition-related co-morbidities (including pancreatic insufficiency, CF-related diabetes, bone health and distal intestinal obstruction syndrome); and key new topic areas (genetic modulator therapies, overweight/obesity and complementary therapies). This paper showcases highlights from the guidelines, focussing on new topic areas and geographic and climate considerations for vitamin D, salt and hydration.