Comparison of diagnostic methods for pancreatic insufficiency in infants with cystic fibrosis.

OBJECTIVE: The objective of the present study is to compare daily weight gain and laboratory analysis (72-hour fecal fat and steatocrit) with fecal elastase-1 (EL-1) when diagnosing pancreatic insufficiency (PI) in infants with cystic fibrosis (CF). METHODS: A total of 39 infants with CF, diagnosed consecutively by newborn screening at 2 referral centers, were included in the study. Daily weight gain and results of laboratory analysis of stool samples were compared using the κ coefficient and the receiver operator characteristic (ROC) curve. RESULTS: Using the criterion of low daily weight gain, the frequency of PI was 92.3%; using the 72-hour fecal fat, steatocrit, and fecal EL-1 tests, the frequency was 42.3%, 86.2%, and 84.6%, respectively. EL-1 was used as the reference test. It was observed that the criteria of low daily weight gain (<50th percentile) and abnormal steatocrit, used together, showed the highest sensitivity (91.3%) and specificity (83.3%) for the diagnosis of PI. CONCLUSIONS: When fecal EL-1 analysis is not immediately available, low daily weight gain associated with abnormal steatocrit can be adopted as a criterion for initiating pancreatic enzyme replacement therapy in infants with CF; however, EL-1 testing should be performed later for confirmation of PI.

Oral magnesium supplementation in children with cystic fibrosis improves clinical and functional variables: a double-blind, randomized, placebo-controlled crossover trial.

BACKGROUND: Magnesium is one of the most important minerals in the body. Although some studies reported that patients with cystic fibrosis (CF) lack magnesium, no international study has assessed the importance of oral magnesium supplementation in CF patients. OBJECTIVE: We prospectively investigated the long-term effect of oral magnesium supplementation on respiratory muscle strength by using manuvacuometry and the Shwachman-Kulczycki (SK) score among children and adolescents with CF. DESIGN: This double-blind, randomized, placebo-controlled crossover study included 44 CF patients (aged 7-19 y; 20 males) who were randomly assigned to receive magnesium (n = 22; 300 mg/d) or placebo (n = 22) for 8 wk with a 4-wk washout period between trials. All patients were undergoing conventional treatment of CF. The experimental protocol included clinical evaluation, assessment of urinary concentration of magnesium, and manuvacuometric measurements [maximal inspiratory pressure (MIP) and maximal expiratory pressure (MEP)]. MIP was the primary outcome. RESULTS: Urinary magnesium increased after the administration of magnesium (change: 36.38 mg/d after magnesium compared with 0.72 mg/d after placebo; P < 0.001). Moreover, MIP and MEP significantly improved only after magnesium administration (change in MIP: 11% predicted after magnesium compared with 0.5% predicted after placebo; change in MEP: 11.9% predicted after magnesium compared with 0.8% predicted after placebo; P < 0.001 for both). Magnesium administration had a beneficial effect on clinical variables assessed by the SK score (change: 4.48 points after magnesium compared with -1.30 points after placebo; P < 0.001). CONCLUSION: Oral magnesium supplementation helped improve both the SK score and respiratory muscle strength in pediatric patients with CF.

Prevalence of hyponatremia at diagnosis and factors associated with the longitudinal variation in serum sodium levels in infants with cystic fibrosis.

OBJECTIVE: To determine the prevalence of hyponatremia at diagnosis in patients with cystic fibrosis and identify the factors associated with changes in serum sodium concentration over time. STUDY DESIGN: This longitudinal study investigated whether variations in serum sodium concentration were associated with age, diet, infection status, and climate/temperature. Multivariate analysis was performed using the random-effects model for longitudinal data. RESULTS: Hyponatremia at diagnosis was observed in 19 of the 20 patients (95%). Factors identified as associated with variations in serum sodium concentration were diet (P = .008) and climate/temperature (P = .005). Intake of solid foods appeared to greatly increase the serum sodium concentration (increase of 5 mEq/L after introduction of solid foods); however, a confounding factor between diet and age cannot be definitively ruled out. Climate/temperature contributed in an inverse way; a 1°C-increase in ambient temperature was associated with a 0.5-mEq/L decrease in serum sodium concentration. CONCLUSION: Infants with cystic fibrosis who feed on breast milk or formula and live in a high-temperature environment are at increased risk for hyponatremia, even when receiving a higher salt intake in accordance with recommendations.

Pediatric functional constipation treatment with Bifidobacterium-containing yogurt: a crossover, double-blind, controlled trial.

AIM: To evaluate the treatment of pediatric functional chronic intestinal constipation (FCIC) with a probiotic goat yogurt. METHODS: A crossover double-blind formula-controlled trial was carried out on 59 students (age range: 5-15 years) of a public school in Belo Horizonte, MG, Brazil, presenting a FCIC diagnostic, according to Roma III criteria. The students were randomized in two groups to receive a goat yogurt supplemented with 10(9) colony forming unit/mL Bifidobacterium longum (B. longum) (probiotic) daily or only the yogurt for a period of 5 wk (formula). Afterwards, the groups were intercrossed for another 5 wk. Defecation frequency, stool consistency and abdominal and defecation pain were assessed. RESULTS: Both treatment groups demonstrated improvement in defecation frequency compared to baseline. However, the group treated with probiotic showed most significant improvement in the first phase of the study. An inversion was observed after crossing over, resulting in a reduction in stool frequency when this group was treated by formula. Probiotic and formula improved stool consistency in the first phase of treatment, but the improvement obtained with probiotic was significantly higher (P = 0.03). In the second phase of treatment, the group initially treated with probiotic showed worsening stool consistency when using formula. However, the difference was not significant. A significant improvement in abdominal pain and defecation pain was observed with both probiotic and formula in the first phase of treatment, but again the improvement was more significant for the group treated with B. longum during phase I (P < 0.05). When all data of the crossover study were analyzed, significant differences were observed between probiotic yogurt and yogurt only for defecation frequency (P = 0.012), defecation pain (P = 0.046) and abdominal pain (P = 0.015). CONCLUSION: An improvement in defecation frequency and abdominal pain was observed using both supplemented and non-supplemented yogurt, but an additional improvement with B. longum supplementation was obtained.

Management of gastroesophageal reflux disease and erosive esophagitis in pediatric patients: focus on delayed-release esomeprazole.

OBJECTIVE: To review the literature on the treatment of gastroesophageal reflux disease (GERD) with emphasis on proton pump inhibitors (PPIs), particularly on delayed-release esomeprazole, and to identify properties and adverse effects of PPIs observed in the treatment of GERD in children and adolescents. SOURCES: Electronic search of PubMed/Medline and Cochrane Collaboration databases, and of abstracts on DDW, NASPGHAN, and ESPGHAN. We focused on controlled and randomized studies published since 2000 and identified reviews that presented a consensual position, and directives published within the last 10 years. MAIN RESULTS: PPIs are considered better antisecretory agents than H(2)-receptor antagonists. Although all PPIs are similar, they are not identical in their pharmacologic properties. For example, the acid-suppressive effect of esomeprazole, the S-isomer of omeprazole, persists for more than 16 hours after administration of the morning dose. Therefore, it can control acidity after night meals better than a single dose of omeprazole. Moreover, the onset of the suppressive effect of esomeprazole is faster. It achieves acid inhibition faster than other PPIs. CONCLUSION: Currently, the mainstream treatment for GERD in children is a PPI. Although PPIs are safe drugs, effective in healing erosive esophagitis, and in relieving symptoms, studies with esomeprazole have shown that this drug has as powerful an ability to inhibit acid secretion as omeprazole. It also seems that some pharmacologic properties of esomeprazole are actually better for the treatment of GERD.