Persistent morbidity and mortality of protein calorie malnutrition in young infants with CF.

Young infants with cystic fibrosis (CF) are at risk for developing symptomatic protein-calorie malnutrition (PCM) characterized by hypoalbuminemia, edema, and anemia. We reviewed the hospital charts of all infants less than 12 months of age referred to our CF clinic between 1979 and 1982 and found nine patients with PCM (13%). Their courses were characterized by age less than 7 months (nine patients), frequent use of soy formula (eight patients), presence of gastrointestinal or respiratory symptoms before being diagnosed with CF (eight patients), severe respiratory distress often requiring mechanical ventilation (five patients), significant infections (three patients), and high mortality (five patients). We further noted that eight infants were not on pancreatic enzyme therapy at the onset of PCM. This clinical study extends the findings of earlier reports of PCM in patients with CF by showing that this syndrome persists as a major source of morbidity and mortality in young infants with CF, especially those fed soy formula and not receiving pancreatic enzyme supplements. Because PCM can develop rapidly and may be the presenting sign of CF in young infants, we speculate that early diagnosis of CF with institution of pancreatic supplements may decrease incidence and severity of PCM in young patients with CF.