Cystic Fibrosis: From Protein-Energy Malnutrition to Obesity with Dyslipidemia

Advances in early diagnosis and treatment have increased the survival of patients with cystic fibrosis (CF) to 30-40 years of age[1]. Interventions in nutritional status are recommended since an adequate body mass index (BMI) has been shown to be positively correlated with pulmonary function[2]. However, the development of obesity may influence the onset of CF-related diabetes[3]. We report here the case of a malnourished infant with CF who progressed to obesity at a reference center in the state of São Paulo, Brazil. A female patient was born premature (33 weeks), weight 2,235g, length 43.5cm. At 2 months of age, CF was diagnosed, with pancreatic insufficiency (two sweat chloride >60 mEq/L and steatocrit >10%) associated with the anemia and low-protein form (Hb 7.0 g/dL; total proteins 3.0 g/dL; albumin 1.7 g/dL). Pancreatic enzyme replacement and complementation of maternal milk with an appropriate formula were started. At 3 mo 25 d, she weighed 4,745g and measured 54cm. Diarrhea, dehydration with electrolytic imbalance (Na 130 mEq/L, K 2.4 mEq/L and Cl 87mEq/L) and metabolic alkalosis (pH 7.54; bicarbonate 35,7; BE +11.5) started at 5 months of age and pseudo-Bartter syndrome was diagnosed. At 6 mo 12 d, the baby weighed 4,500g and started to receive a high-calorie, high-protein diet. At 9 mo, she weighed 6,995g and measured 64cm and at 1 year and 4 months 10,470 g and 75.5 cm. She had 5 episodes of prolonged hospitalization from 5 mo to 11 mo, when she was fed enterally (high-calorie, high-protein diet with a concentrated formula enriched with dextrin maltose and/or a nutritional supplement).