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The carotenoids are potent antioxidants with the ability to quench singlet oxygen and other toxic oxygen species. The aim of this pilot study was to investigate the protective effect of β-carotene on oxidant system in patients with cystic fibrosis (CF) and in patients with bronchiectasis (BE) caused by a reason other than CF. Eighteen children with CF and 15 children with BE followed in the Pediatric Chest Disease Unit of Hacettepe University, and 15 healthy children participated in the study. Compared with the controls, significantly lower plasma levels of β-carotene were found in the CF group and significantly lower plasma levels of vitamin E in the CF and BE groups. The standardization of carotenoid levels for total cholesterol did not significantly attenuate these differences. In addition, there were significantly higher levels of malondialdehyde (a marker of lipid peroxidation) and tumour necrosis factor-α (TNF-α) in children with CF and in children with BE than in normal subjects. After 6 mo of β-carotene supplementation, the plasma levels of βcarotene and vitamin E increased and the plasma levels of TNF-α and malondialdehyde decreased in both groups. Conclusion: Potent antioxidants, β-carotene and vitamin E are deficient in patients with CF and in patients with BE, and they are more susceptible to oxidative damage. These patients may benefit from β-carotene supplementation. [ABSTRACT FROM AUTHOR]

Patients with cystic fibrosis (CF) are underweight and growth retarded. This study tested the link between serum insulin-like growth factor-I (IGF-I) and insulin-like growth factor-binding protein-3 (IGFBP-3) levels and body height, nutritional status, pulmonary function tests and activity of inflammation in 92 subjects with CF (age 2.1-18.8 y). It also analysed the effect of short-term antibiotic treatment and hyperalimentation on IGF-I and IGFBP-3 levels in 33 subjects (age 3.6- 33.7 y) on 41 occasions. Both IGF-I (-1.19 ± 0.17 SD) and IGFBP-3 levels (-0.66 ± 0.12 SD; both p< 0.0001 vs 0) were decreased in cross-sectional measurements. Their standardized values were inversely proportional to age (IGF-I: r=-0.23, p= 0.03; IGFBP-3: r=-0.29, p = 0.005) and positively correlated with SDS of height (IGF-I: r = 0.40, p<0.0001; IGFBP-3: r = 0.36, p = 0.0005) and of mid-arm circumference (IGF-I: r = 0.39, p = 0.0001; IGFBP-3: r = 0.38, p = 0.0002), and with pulmonary function tests. After a short-term course of intensive antibiotic therapy and hyperalimentation, IGF-I normalized (from-0.66 ± 0.20 to 0.00 ± 0.25 SD; p< 0.0001) and IGFBP-3 increased (from -0.78 ± 0.15 to -0.53 ± 0.16 SD; p = 0.002). IGFBP-3 correlated inversely with erythrocyte sedimentation rate (r =-0.40, p = 0.01). [ABSTRACT FROM AUTHOR]

In patients with cystic fibrosis (CF), glucose intolerance preceding diabetes (prediabetes) may have adverse effects on nutritional status and respiratory function, which are reversible after the start of insulin therapy. Respiratory function (forced vital capacity and forced expiratory volume in one second) and body mass index (BMI) were compared retrospectively in a French cohort of 14 patients during the 5 y preceding insulin therapy for diabetes and in 14 age- and sex-matched controls with normal oral glucose tolerance tests. In the diabetic group, all three parameters deviated increasingly from the values in the controls; the differences became statistically different during the 6 mo before insulin therapy. The effect was more important in patients for whom diabetes mellitus was diagnosed on the basis of symptoms of hyperglycaemia than in patients for whom it was diagnosed by systematic screening, but still present in the latter. After insulin was started, respiratory function improved and the BMI returned to normal within 1 y. The annual insulin requirement increased from 0.62 during the first year to 1.25 during the fifth year. Glycosylated haemoglobin (HbA[sub Ic]) values ranged from 6.6 to 7.8%. Only 2 episodes of severe hypoglycaemia were recorded over 42 patient-years of follow-up. The insulin regimen most often used was two daily injections of a mixture of short- and intermediate-acting insulin (n = 10) given with an insulin pen. [ABSTRACT FROM AUTHOR]

Cystic-fibrosis-related diabetes mellitus deterioration of overall clinical status. The purpose of this prospective study was to investigate the influence of insulin on nutrition, lung function and clinical status of cystic fibrosis patients. For a period of 5 y, and at 6-mo intervals, body mass index, forced expiratory volume in 1 sec, Shwachman score, intravenous glucose tolerance test and first-phase insulin response were determined in 30 cystic fibrosis patients (age range 10-35 y) with exocrine pancreatic insufficiency. During the study period, six patients (3M and 3F; age range 15-22 y) developed diabetes and required insulin therapy. The decrease of first-phase insulin response coincided with deterioration of nutritional and clinical status, which improved significantly 6 mo after the institution of insulin. Conclusion: Insulin, as an anabolic hormone, could have an influence on body mass, which may affect pulmonary function and clinical condition in cystic fibrosis. It is important to identify cystic fibrosis individuals at risk of developing diabetes so that early insulin therapy is instituted. is frequently underdiagnosed and associated with. [ABSTRACT FROM AUTHOR]

Fifteen appropriate-for-date premature low-birthweight infants with cystic periventricular leukomalacia (PVL) were studied. The infants were stratified into three birthweight groups: less than 1000 g, 1000 g and greater but less than 1500 g, and 1500 g or greater. Reported and new risk factors for PVL were compared with control patients for all patients and each birthweight group. Hypocarbia was significantly related to cystic PVL, especially in infants with birthweight 1000 g or greater (p < 0.03). Sensitivity to hypocarbia might be decreased in infants with birthweight less than 1000 g due to therapy or prematurity. In the group with birthweight less than 1000 g, the proportion of cystic PVL infants on continuous intra-arterial blood-pressure monitoring tended to be lower than the controls, with an almost significant difference (p = 0.05). The duration of tocolysis was significantly longer in the cystic PVL infants than in the controls when the birthweight was greater than 1500 g (p < 0.04). For some risk factors, a significant difference or a tendency of difference was demonstrated only after stratifying the birthweight. For others, the difference became insignificant after stratification. Assessing risk factors after stratifying by birthweight or degree of prematurity is therefore useful. Conclusion: The results suggest that hypocarbia should be avoided to prevent cystic PVL, especially in infants with birthweight of 1000 g or greater, continuous intra-arterial blood-pressure monitoring may be important in infants with birthweight less than 1000 g, and fetal status should be monitored carefully when the duration of tocolysis is prolonged, especially in infants with birthweight of 1500 g or more. [ABSTRACT FROM AUTHOR]

This study compared the acid steatocrit (AS) results of healthy children with those of sick children with and without gastrointestinal involvement. Stool samples of 166 children were investigated, comprising 50 healthy children, 26 asthma patients, and 90 patients with gastrointestinal problems divided into 34 treated cystic fibrosis (CF) patients with exocrine pancreatic insufficiency, 16 untreated coeliac disease (CD) patients and 40 patients with various gastrointestinal problems. The median values (5th–95th percentile) of AS results were 3.3% (0.0–21%) for healthy children, 4.5% (1.8–22.5%) for asthma patients, 24.7% (2.6–68.2%) for treated CF patients with exocrine pancreatic insufficiency, 19.8% (3–77.7%) for untreated CD patients and 5.5% (1.8–29%) for patients with various gastrointestinal diseases. [ABSTRACT FROM AUTHOR]