Association between body composition and pulmonary function in children and young people with cystic fibrosis.

OBJECTIVES: Body mass index (BMI) has significant limitations when assessing nutritional status in pediatric patients with cystic fibrosis (CF). We evaluated whether measurements of lean body mass (LBM) and fat mass (FM) are more sensitive nutritional parameters by testing their association with pulmonary function in adolescent patients with CF. METHODS: Sixty-nine male and female adolescents with CF were studied (age: 14.5 ± 2.3, BMI: 19.5 ± 2.3 kg/m2). Dual-energy x-ray absorptiometry (DXA) was used to measure total and segmental (appendicular, truncal) body composition (FM, LBM bone mineral density, and content) as routine care to monitor bone health. Correlation and multiple regression analyses were performed to assess the association among body composition variables and forced expiratory volume in 1 s (FEV1). We also evaluated the influence of the F508del mutation on body composition. RESULTS: FEV1 was significantly associated with total (r = 0.68, P <0.001), truncal (r = 0.71, P <0.001), and appendicular (r = 0.67, P <0.001) LBM, whereas it was not associated with total (r = 0.02, P = 0.89) and truncal (r = 0.04, P = 0.77) FM. BMI had a significant but weaker correlation with FEV1 (r = 0.52, P <0.001) compared with LBM. LBM was the only significant predictor of FEV1 in fully adjusted regression models. CONCLUSIONS: LBM is a significant predictor of pulmonary function in CF adolescent patients. DXA scanning performed as part of routine bone health monitoring in CF can provide important body composition data relevant to clinical interventions that optimize nutritional status. DXA reference data for LBM in non-adult populations are needed to enhance diagnostic assessment and monitor clinical progression of CF.

Improved growth, tolerance and intake with an extensively hydrolysed peptide feed in infants with complex disease.

BACKGROUND & AIMS: Infants with complex medical conditions often display faltering growth due to elevated nutritional requirements, poor intake and intolerance of feeding with malabsorption and maldigestion. As a result their nutritional management can be extremely challenging and enteral nutritional support is required. This study aimed to investigate the effectiveness, tolerance and acceptability of nutritional support with a specially formulated, paediatric peptide feed in infants with complex disease and signs of growth faltering with their current nutritional management. METHODS: This prospective intervention study investigated gastrointestinal (GI) tolerance, nutritional intake and compliance with feeding, anthropometry and growth in 18 infants (mean age 6.11 months ± 4.69, mean weight 4.97 kg ± 1.71) during 28 days of enteral nutritional support with a paediatric (1 kcal/ml) readymade peptide feed. RESULTS: GI tolerance to nutritional support with a peptide enteral feed was good and either improved or remained stable over the study. Compliance was excellent (94.0% ± 12.6), total energy intake improved (+23 ± 42 kcal/kg, p = 0.037) and mean weight (0.61 kg ± 0.31, p = 0.0001), length (1.89 ± 1.77 cm, p = 0.0001), head circumference (1.33 ± 1.29 cm, p = 0.001), weight for length Z score (p < 0.05), and weight for age Z score (p < 0.05) significantly improved. Sixty one percent (n = 11) of the infants showed signs of increased growth velocity, moving upwards in terms of their centiles. All 18 infants continued with the paediatric, peptide enteral feed once the study was complete. CONCLUSIONS: This prospective study showed that nutrition support with a specially formulated, paediatric peptide readymade feed was well tolerated, helped to promote growth, and can be considered suitable for use in infants with complex disease and faltering growth who are unable to tolerate a whole protein feed.

Vitamin D in children with cystic fibrosis.

PURPOSE: The Cystic Fibrosis Trust in 2007 published a recommended target of 75-150 nmol/L for 25-hydroxyvitamin D (25-OHD). In 2008 we found that only 10% of pancreatic insufficient (PI) children met this target. An increase in supplementation was implemented and a repeat audit performed in 2010. METHODS: PI children ≥1 year under sole-care in our regional centre were included. Vitamin D3 supplementation increased by >450% to either 3800 IU/day liquid or 800 IU daily plus 20,000 IU weekly tablets. In 2010 pancreatic sufficient (PS) children were also audited separately. RESULTS: The median 25-OHD level increased from 51.5 nmol/L in 2008 (n=78, 10% >75 nmol/L) to 72 nmol/L in 2010 (n=72, 51% >75 nmol/L), p<0.0001. In PS children (n=15 in 2010) 87% had 25-OHD levels <75 nmol/L. CONCLUSIONS: A substantial increase in supplementation led to a significant increase in 25-OHD levels but around half still failed to reach the recommended target.

End-of-life decision-making, the principle of double effect, and the devil's choice: a response to Roger Magnusson.

Recently, the principle of double effect has come under scrutiny by Magnusson who believes it provides a subterfuge for those who act so as to end the lives of their patients. Specifically, he argues that the conceptual distinction between foresight and intention is dubious and, moreover, renders patients vulnerable to involuntary euthanasia. At the same time, Magnusson wants to protect doctors from criminal liability when faced with (what he understands to be) a "devil's choice" between ending the life of a patient or under-treating pain. Hence, Magnusson proposes that, subject to specific conditions, a so-called "defence of necessity" be recognised through either common law doctrine or legislation. However, to safeguard this defence, he must rely on what he most wants to reject: a fundamental aspect of the principle of double effect.