Outpatient Pain Management in Children With Chronic Pancreatitis: A Scoping Systematic Review.

OBJECTIVES: Although pain management is central to pediatric chronic pancreatitis (CP) care, no evidence-based guidelines exist. In this scoping systematic review, we sought promising strategies for CP pain treatment in children. METHODS: We systematically reviewed literature on pain management in children and adults with CP, and 2 conditions with similar pain courses: juvenile idiopathic arthritis and sickle cell disease. RESULTS: Of 8997 studies identified, 287 met inclusion criteria. There are no published studies of analgesic medications, antioxidants, dietary modification, integrative medicine, or regional nerve blocks in children with CP. In adults with CP, studies of nonopioid analgesics, pancreatic enzymes, and dietary interventions have mixed results. Retrospective studies suggest that endoscopic retrograde cholangiopancreatography and surgical procedures, most durably total pancreatectomy with islet autotransplant, improve pain for children with CP. Follow-up was short relative to a child's life. Large studies in adults also suggest benefit from endoscopic therapy and surgery, but lack conclusive evidence about optimal procedure or timing. Studies on other painful pediatric chronic illnesses revealed little generalizable to children with CP. CONCLUSIONS: No therapy had sufficient high-quality studies to warrant untempered, evidence-based support for use in children with CP. Multicenter studies are needed to identify pain management "best practices."

Advocacy for Improving Nutrition in the First 1000 Days to Support Childhood Development and Adult Health.

Maternal prenatal nutrition and the child's nutrition in the first 2 years of life (1000 days) are crucial factors in a child's neurodevelopment and lifelong mental health. Child and adult health risks, including obesity, hypertension, and diabetes, may be programmed by nutritional status during this period. Calories are essential for growth of both fetus and child but are not sufficient for normal brain development. Although all nutrients are necessary for brain growth, key nutrients that support neurodevelopment include protein; zinc; iron; choline; folate; iodine; vitamins A, D, B6, and B12; and long-chain polyunsaturated fatty acids. Failure to provide key nutrients during this critical period of brain development may result in lifelong deficits in brain function despite subsequent nutrient repletion. Understanding the complex interplay of micro- and macronutrients and neurodevelopment is key to moving beyond simply recommending a "good diet" to optimizing nutrient delivery for the developing child. Leaders in pediatric health and policy makers must be aware of this research given its implications for public policy at the federal and state level. Pediatricians should refer to existing services for nutrition support for pregnant and breastfeeding women, infants, and toddlers. Finally, all providers caring for children can advocate for healthy diets for mothers, infants, and young children in the first 1000 days. Prioritizing public policies that ensure the provision of adequate nutrients and healthy eating during this crucial time would ensure that all children have an early foundation for optimal neurodevelopment, a key factor in long-term health.

Nutritional Status Improved in Cystic Fibrosis Patients with the G551D Mutation After Treatment with Ivacaftor.

BACKGROUND: The cystic fibrosis (CF) transmembrane conductance regulator (CFTR) gating mutation G551D prevents sufficient ion transport due to reduced channel-open probability. Ivacaftor, an oral CFTR potentiator, increases the channel-open probability. AIM: To further analyze improvements in weight and body mass index (BMI) in two studies of ivacaftor in patients aged ≥6 years with CF and the G551D mutation. METHODS: Patients were randomized 1:1 to ivacaftor 150 mg or placebo every 12 h for 48 weeks. Primary end point (lung function) was reported previously. Other outcomes included weight and height measurements and CF Questionnaire-Revised (CFQ-R). RESULTS: Studies included 213 patients (aged ≤ 20 years, n = 105; aged > 20 years, n = 108). In patients ≤20 years, adjusted mean change from baseline to week 48 in body weight was 4.9 versus 2.2 kg (ivacaftor vs. placebo, p = 0.0008). At week 48, change from baseline in mean weight-for-age z-score was 0.29 versus -0.06 (p < 0.0001); change in mean BMI-for-age z-score was 0.26 versus -0.13 (p < 0.0001). In patients >20 years, adjusted mean change from baseline to week 48 in body weight was 2.7 versus -0.2 kg (p = 0.0003). Mean BMI change at week 48 was 0.9 versus -0.1 kg/m(2) (p = 0.0003). There was no linear correlation evident between changes in body weight and improvements in lung function or sweat chloride. Significant CFQ-R improvements were seen in perception of eating, body image, and sense of ability to gain weight. CONCLUSIONS: Nutritional status improved following treatment with ivacaftor for 48 weeks.

Metabolic effects of infection and postnatal steroids.

Optimal development of the newborn depends on rapid accretion of substrate in the neonatal period, particularly in the premature infant. Steroids and infection not only induce catabolism, but associated endogenous responses reprioritize crucial substrate to restore homeostasis. The result is a protein/energy deficit and concomitant delay in growth and development. Innovative feeding strategies and novel therapies are needed to reduce the impact of catabolism in this population.