Hypoproteinemia and edema in infants with cystic fibrosis of the pancreas

Growth and pulmonary outcomes during the first 2 y of life of breastfed and formula-fed infants diagnosed with cystic fibrosis through the Wisconsin Routine Newborn Screening Program

“…Breastfeeding was historically discouraged for CF infants because of concerns about protein energy malnutrition, which is manifested by hypoproteinemia, hyponatremia, edema, and anemia (28)(29)(30)(31)(32)(33). Despite these reports, a 1990 survey showed that 77% of CF centers encouraged breastfeeding, with nearly 37% of CF centers recommending exclusive breastfeeding (ExBF) (34).…”

Edema, Anemia, Hypoproteinemia, and Acrodermatitis Enteropathica

“…2,4,6-8 Liver involvement is variable, but when present, manifest as hepatomegaly and mild elevation of transaminases, which are reversible once proper nutritional support is initiated. 4,5,9 Pulmonary symptoms usually present after the development of the edema. 4,5,9 Early recognition of this clinical symptom complex and initiation of appropriate nutritional supplementation and pancreatic enzymes are essential to improve outcome.…”

“…4,5,9 Pulmonary symptoms usually present after the development of the edema. 4,5,9 Early recognition of this clinical symptom complex and initiation of appropriate nutritional supplementation and pancreatic enzymes are essential to improve outcome. 2,4,5 The gold standard for the diagnosis of cystic fibrosis is by demonstrating elevated sweat chloride in the presence of pancreatic exocrine deficiency, chronic pulmonary disease, meconium ileus, or a positive family history.…”

“…There has been a reported increased incidence of hypoalbuminemia with infants being fed either by soy-based formulas or breast milk. 4,9 The hypoalbuminemia following feeding with a soy-based formula is due primarily to a different and inadequate composition of protein derived from soybean as compared with cow's milk. 6 The hypoproteinemia among breast-fed infants is due to loss of proteins through stools.…”

Soy Protein-based Formulas: Recommendations for Use in Infant Feeding

“…10 Before the routine supplementation of soy protein formulas with methionine, infants with undiagnosed, untreated cystic fibrosis were particularly at risk for severe hypoalbuminemia and edema when fed soy proteins, a risk that remains in soy, cow milk, and breastfed infants with cystic fibrosis until the initiation of pancreatic enzyme therapy. 11,12 Carnitine, which is required for the optimal mitochondrial oxidation of long-chain fatty acids, is deficient in foods of plant origin and is added to soy formula to the level in breast milk, as is taurine, an amino acid that is abundant in human milk. Taurine functions as an antioxidant and, along with glycine, is a major conjugate of bile acids in early infancy.…”