https://com-peds-pulmonary.sites.medinfo.ufl.edu/files/2012/09/Guidelines_for_Management_of_Infants_with_CF.pdf

Cystic Fibrosis Foundation Evidence-Based Guidelines for Management of Infants with Cystic Fibrosis

Growth status in children with cystic fibrosis based on the National Cystic Fibrosis Patient Registry data: evaluation of various criteria used to identify malnutrition

Malnutrition is an adverse prognostic factor in cystic fibrosis, influencing the course of pulmonary disease and correlating inversely with survival. A positive energy balance between energy intake and the combination of total energy expenditure, energy losses and growth-related energy cost is essential to maintain normal nutritional status. Before starting nutritional supplementation, it is important to rule out pathological conditions that may have a deleterious effect on nutritional status: persistent exocrine pancreatic insufficiency, chronic bacterial pulmonary colonization, impaired glucose tolerance, specific nutritional deficits and associated disorders leading to a decrease of energy intake. Several methods are available, ranging from boosted oral nutrition to behavioural intervention, oral supplementation, enteral nutrition and, rarely, parenteral nutrition. The use of elemental nutrients for either oral supplementation or enteral nutrition seems of no nutritional benefit and is more expensive than conventional polymeric nutrients. Provided that the goals of the nutritional supplementation are fulfilled, simpler is often better.

Cystic fibrosis: nutritional consequences and management.

Increased energy expenditure in young children with cystic fibrosis

Direct transesterification of plasma fatty acids for the diagnosis of essential fatty acid deficiency in cystic fibrosis

There is now sufficient evidence to prove that the maintenance of a high energy normally balanced diet prevents malnutrition/growth retardation and may well enhance prognosis in patients with CF. Most patients are able to tolerate the normal to high fat content without undue problems with steatorrhoea and with the recent advent of more effective enzyme replacement therapy, this should be even less of a problem. Conversely, there is an equally large bulk of evidence to indicate that the maintenance of a low fat diet, while controlling symptoms from steatorrhoea in some patients, is energy depriving and produces growth failure. In children or teenagers who are presently growth retarded, installation of a high energy intake may improve growth in some, but not in others. Further investigation of the latter patients is required to evaluate their persistent anorexia and advantages and disadvantages of nutritional supplementation by invasive techniques. Certainly growth and wellbeing can be vastly improved. However, such studies may well have to be multicentered to obtain sufficient patients to control for the many variables involved, in order to demonstrate the effects on pulmonary function.

Chronic undernutrition/growth retardation in cystic fibrosis.

Previously we have reported that 37% of infants with cystic fibrosis diagnosed by neonatal screening with the dried blood spot immunoreactive trypsin assay have pancreatic sufficiency. However, 34 of the 78 infants had pancreatic function tests an average 2.3 years after diagnosis, thus it was possible that the percentage with neonatal pancreatic sufficiency was underestimated, due to the loss of pancreatic function with time in some infants.



To assess this hypothesis we have assessed pancreatic function at the time of diagnosis in a further 20 infants since the completion of the previous study. Results of fecal fat determinations and/or pancreatic stimulation tests indicate that 10 (50%) of these infants have pancreatic sufficiency. Combining these results with those of the previous study, 31 of 64 patients (48%) have pancreatic sufficiency at this early age.



We have also monitored the progression of pancreatic disease in the 39 patients with pancreatic sufficiency recognized to date. Eleven have developed pancreatic insufficiency and require enzyme replacement therapy. Five others have shown further improvement of colipase secretion with age.



We conclude that the dried blood immunoreactive trypsin screening program for cystic fibrosis does recognize patients with pancreatic sufficiency, and at diagnosis nearly half the patients are in this category. To date, 28% of patients with pancreatic sufficiency have demonstrated a variable decline in pancreatic function with age. Pediatr Pulmonol. 1991; Supp 7: 69–71.

Assessment of pancreatic function in screened infants with cystic fibrosis.

Dietary intakes were measured over a period of 5 days in 36 malnourished and 36 well-nourished patients with cystic fibrosis. Both energy and protein intakes were significantly less in the malnourished patients for the two age groups studied: 4-9.99 years (p less than 0.01 for both parameters), and 10-16 years (p less than 0.05 and p less than 0.01, respectively). In both age groups and both patient groups, average protein intakes were well in excess of the recommended daily intake, but energy intake in the malnourished patients was below the recommended daily intake. Nutritional supplementation of 10 malnourished patients with a polymeric formula, infused overnight via a gastrostomy tube, resulted in a seven-fold increase in weight gain (p less than 0.001) and a doubling of linear growth velocity (p less than 0.01) over a period of 18 months, compared to the 18 months prior to gastrostomy feeding. Measurements of total body nitrogen in eight of these patients demonstrated a 38% increase in body nitrogen content over 12 months, indicating a replenishment of the protein deficit.

M.A. Gruca

Papers

Chronic undernutrition/growth retardation in cystic fibrosis.

Assessment of pancreatic function in screened infants with cystic fibrosis.

Nutritional status, growth and development in children undergoing intensive treatment for cystic fibrosis.