Persistent Morbidity and Mortality of Protein Calorie Malnutrition in Young Infants with CF

Cystic Fibrosis Presenting With Dermatitis

“…3 PEM occurs in 5% to 13% of infants with CF and is associated with increased morbidity and mortality. 3,4 Dermatitis as an initial presentation of CF is rare and has been attributed to deficiencies in zinc, protein, and essential fatty acids (EFAs). 2,5,6 The rash typically occurs 1 month before the onset of edema and other clinical manifestations.…”

“…2,[4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19] The dermatitis typically presents as erythematous, scaling papules that later progress to extensive coalescing, desquamating plaques. The eruption is usually first noted in the perineum anywhere from several days to 15 months after birth, with subsequent spread to the perioral region, extremities, and trunk.…”

A 4-month-old boy with acrodermatitis enteropathica-like symptoms

“…When CF presents with kwashiorkor, it is associated with high morbidity and mortality [1]. Early recognition and the instigation of appropriate nutritional supplementation and pancreatic enzymes is essential for improving the outcome, as in our case [6,7,12,13,15].…”

“…Up to 10% of patients with CF presented with hypoproteinemia, delayed development, anemia, and edema due to the kwashiorkor caused by pancreatic insufficiency. Cutaneous symptoms as a presenting sign in CF are rare [1,5,6,12,15]. The skin eruption is usually seen between the 3rd to 7th months of age and is seen about a month before edema and severe malnutrition.…”

Nutrition in cystic fibrosis: a historical overview

“…Eight of the babies were not taking pancreatin supplements. All nine had low zinc levels and six had Pseudomonas aeruginosa cultured from tracheal aspirates (Abman et al 1986). …”