Rituximab therapy in ROHHAD(NET) syndrome.

OBJECTIVES: Rapid-onset obesity with hypoventilation, hypothalamic dysfunction, autonomic dysregulation, and neural-crest tumour (ROHHAD(NET)) is a rare syndrome presenting in early childhood associated with high morbidity and mortality. There is no specific diagnostic biomarker and diagnosis is based on clinical features. An autoimmune origin has been postulated. CASE PRESENTATION: Management is largely supportive. We report a case of a five-year old female who presented in respiratory arrest after 6-months of rapid weight gain. She had central hypoventilation, central diabetes insipidus, growth hormone deficiency and hyperprolactinaemia. She displayed elevated interleukin-6 levels on cytokine serology which normalised after rituximab treatment. After rituximab treatment, her weight reduced significantly from greatly above the 99.6th to the 50th centile in 12 months. CONCLUSIONS: This response possibly reflects an underlying, immune-inflammatory pathology driving excess adiposity in this condition. Potentially, other aspects of ROHHAD(NET) may be mediated through autoimmune dysregulation in which case rituximab may provide benefits for prognosis and survival.

Feeding growth restricted premature neonates: a challenging perspective.

Nutrition in the postnatal period is essential to achieve optimal growth and maintain biochemical normality. Feeding growth-restricted premature neonates remains a big challenge for pediatricians and neonatologists. The choice of milk is one of the biggest challenges. Breast milk is recommended, although feeding with preterm formulas can ensure a more consistent delivery of optimal levels of nutrients. The timing of introduction of feeds and the rate of advancement of those feeds in preterm infants are both topics of significant controversy. Early feeding is advantageous because it improves the functional adaptation of the gastrointestinal tract and reduces the duration of total parenteral nutrition. A faster rate of advancement will also reduce the duration of need for parenteral nutrition. Despite this, enteral feeding is often delayed and is often slowly increased in high-risk infants because of a possible increased risk of necrotizing enterocolitis (NEC). Growth-restricted neonates are at increased risk of developing NEC due to a combination of antenatal and postnatal disturbances in gut perfusion. If enteral feeding is introduced earlier and advanced more quickly, this may lead to increased risk of NEC, but slower feeds extend the duration of parenteral nutrition and its risks and may have adverse consequences for survival, growth, and development. Premature infants pose a significant nutritional challenge. Overall, we would suggest the preferential use of human breast milk, early minimal enteral feeds, and standardized feeding protocols with cautious advancements of feeds to facilitate gastrointestinal adaptation and reduce the risk of NEC, however further research is needed.