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CF Children (cf + child)
Selected AbstractsVitamin K prescribing patterns and bone health surveillance in UK children with cystic fibrosisJOURNAL OF HUMAN NUTRITION & DIETETICS, Issue 6 2007D. S. Urquhart Abstract Background, Bone disease has become an increasingly recognized complication of cystic fibrosis (CF). Although causes of CF bone disease are multifactorial, there has been recent interest in the role of vitamin K in CF bone disease. Aims,and,methods, A questionnaire survey of all UK paediatric CF centre dietitians and centre directors was carried out to ascertain current practice with regard to vitamin K prescribing and bone health surveillance. Results, The survey had a 97% response rate representing 3414 CF children. Twenty-three centre directors and 19 dietitians responded, and at least moderate agreement was noted with kappa scores >0.41 for all but one question assessed. Ninety-three per cent centres report that >90% pancreatic insufficient patients receive vitamins A, D and E, yet only 18% centres routinely supplement vitamin K. The majority (60%) report that <10% of their CF patients receive vitamin K, whilst vitamin K dosage varied from 0.3,0.5 to 10 mg day,1. Only one centre undertook no bone health surveillance, and vitamin D levels are measured in 89%, calcium intake assessed in 82% and dual-energy X-ray absorptiometry scans performed in 61% centres. Discussion, Heterogeneity in both vitamin K prescribing practices and bone health surveillance in CF across the UK were noted, underlining the need for a national consensus on bone health management, as well as acting as a call for longitudinal research into the clinical effectiveness of vitamin K therapy in CF. [source] Does presenting with meconium ileus affect the prognosis of children with cystic fibrosis?PEDIATRIC PULMONOLOGY, Issue 10 2010Jo-Anne Johnson MBChB Abstract It is a matter of debate as to what extent the long-term outcome of cystic fibrosis (CF) is affected by presenting with meconium ileus (MI). We compared long-term clinical outcomes of CF children who presented with MI, to those presenting with other symptoms (non-MI) in an era of non new-born-screening (NBS). We collected annual lung function data between the ages of 8,15 years in terms of percent predicted first second forced expired volume (FEV1%pr), percent predicted forced vital capacity (FVC%pr), and between the ages of 2,15 years annual height and weight Z-scores (HtZ and WtZ respectively) for children attending the Royal Brompton Hospital CF clinic. To be included in the study, subjects had to have at least five pulmonary function tests and five anthropometric measurements recorded over this period. Thirty-eight MI and 76 non-MI subjects were compared. There were no significant differences in genotype, sex, chronic Pseudomonas infection, or pancreatic enzyme use between the two groups. The median age of diagnosis was 1 day (MI) versus 7 months (non-MI). There was a decline in spirometry and anthropometric variables over the study period for both MI and non-MI groups apart from WtZ score in the non-MI group. Mixed model analysis adjusting for potential confounders including genotype, pancreatic status, sex, chronic Pseudomonas aeruginosa lung infection, and age of diagnosis revealed no difference between the two groups in terms of lung function and growth during the time period of the study, however there was a non-significant trend for subjects presenting with MI to do better in all four parameters. We conclude that babies presenting with MI have no worse long-term outcome than those presenting symptomatically later in infancy, despite having undergone invasive procedures in the newborn period. This underscores the importance of early diagnosis and treatment in CF. Pediatr Pulmonol. 2010; 45:951,958. © 2010 Wiley-Liss, Inc. [source] Transforming growth factor-,1 in bronchoalveolar lavage fluid from children with cystic fibrosis,PEDIATRIC PULMONOLOGY, Issue 11 2009William T. Harris MD Abstract Rationale Transforming factor ,1 (TGF-,1) genetic polymorphisms have been identified as a modifier of cystic fibrosis (CF) lung disease severity. However, few data link TGF-,1 protein levels and clinical markers of CF lung disease severity. Objectives To determine the association between protein levels of TGF-,1 in pediatric CF bronchoalveolar lavage fluid (BALF) and clinical parameters of CF lung disease severity. Methods Total TGF-,1 was measured in BALF from 30 pediatric CF patients and 12 non-CF disease controls undergoing clinically indicated flexible bronchoscopy, and compared to four indicators of clinical disease: infection, inflammation, pulmonary function, and recent/recurrent hospitalization. Results TGF-,1 was elevated in CF BALF compared to non-CF controls (135,±,15,pg/ml vs. 57,±,10,pg/ml, P,<,0.01). In CF BALF, increased TGF-,1 was associated with elevated BALF PMN % (r,=,0.67, P,<,0.01). BALF TGF-,1 was increased in CF subjects whose FEV1 after the completion of antibiotic therapy remained below CF age-normative median values (205.9,±,20.5,pg/ml vs. 106.4,±,24.0, P,=,0.01). BALF TGF-,1 was increased in CF children hospitalized in the previous year compared to those not recently hospitalized (169.9,±,21.6,pg/ml vs. 107.5,±,17.5,pg/ml, P,=,0.04). Neither the presence of a bacterial pathogen nor bacterial quantity was associated with BALF TGF-,1. Conclusions In CF, BALF TGF-,1 is elevated compared to non-CF controls. Increased BALF TGF-,1 is associated with neutrophilic inflammation, diminished lung function and recent hospitalization. Further investigation is needed to address mechanisms behind these associations. Pediatr Pulmonol. 2009; 44:1057,1064. ©2009 Wiley-Liss, Inc. [source] Human metapneumovirus and respiratory syncytial virus infections in older children with cystic fibrosisPEDIATRIC PULMONOLOGY, Issue 1 2007Daniel F. Garcia MD Abstract Background: Human metapneumovirus (hMPV) has been isolated from children with acute respiratory infection worldwide. Its epidemiology remains to be defined in children with cystic fibrosis (CF). We describe the epidemiology and clinical impact of hMPV in CF children and compared it to respiratory syncytial virus (RSV). Methods: CF children ages 7,18 years were studied prospectively during the 1998,1999 RSV season. Nasopharyngeal specimens were collected during acute respiratory illnesses and tested for respiratory viruses. Blood specimens were drawn early, mid, and end of the RSV season, and tested for serological evidence of hMPV and RSV infections. Rates of lower respiratory tract illnesses (LRTI) and hospitalizations for pulmonary exacerbations were compared during the time intervals they developed serological evidence of infection to their non-infection intervals. Results: Six of 44 CF children had a virus positive respiratory illness in 56 LTRI events and 18 hospitalizations. Serological evidence of hMPV and RSV infections occurred in 16 and 20 CF children, respectively; 8 had infections with both viruses. A greater proportion of CF children had ,1 LRTI during their infection intervals compared to their non-infection intervals (13/25 vs. 5/25; P,=,0.03). A trend for higher rates of LRTI was observed in the infection intervals compared to non-infection intervals (9.5,±,11.0 vs. 4.2,±,9.9 per 1,000 child-days; P,=,0.06), and it was significantly greater with a more conservative estimate (one event per child per interval; 7.4,±,7.7 vs. 2.6,±,5.4 per 1,000 child-days; P,,0.01). No differences in hospitalizations rates were detected. Conclusion: The infection rates and clinical impact observed for hMPV were comparable to that for RSV in CF children 7,18 years of age. Pediatr Pulmonol. 2007; 42:66,74. © 2006 Wiley-Liss, Inc. [source] Long-term trial of cyproheptadine as an appetite stimulant in cystic fibrosis,PEDIATRIC PULMONOLOGY, Issue 3 2005Douglas N. Homnick MD Abstract Appetite stimulants have been used to help overcome decreased appetite and malnutrition in children and adults with various chronic illnesses, including cystic fibrosis (CF). Stimulants have included megestrol acetate (MA), cyproheptadine hydrochloride (CH), cannabinoids, hydrazine sulfate, anabolic hormones, and growth hormone. Many of these, including MA, have substantial side effects and may not be suitable for prolonged use. We previously studied the effects of CH on weight gain in a short-term (12 week) trial in CF with good results compared to placebo. Side effects were few, and weight gain was significant. In this study, we sought to determine the effects of CH over a longer term in order to assess its suitability for prolonged use. Sixteen CF children and adults enrolled in the original short-term study subsequently entered this study, and 12 completed the 9-month trial. All patients receiving placebo in the original short-term study received CH 4 mg up to four times a day in the long-term study continuation, and those receiving CH in the short-term study continued on the drug. No pill counts were done, and patients were queried at quarterly visits as to their CH use. Anthropometrics and spirometry were also done quarterly, and antibiotic use was quantified. Subjects who had changed from placebo (CH2 group) gained weight significantly over 3,6 months, and those continuing on CH (CH1 group) generally maintained previously gained weight over the duration of the study. Select spirometric measures improved in both groups but not significantly, and side effects were mild. CH appears to be an effective appetite stimulant in CF, and generally maintains its effect over time with an acceptable side-effect profile. Pediatr Pulmonol. © 2005 Wiley-Liss, Inc. [source] Longitudinal pulmonary status of cystic fibrosis children with meconium ileusPEDIATRIC PULMONOLOGY, Issue 4 2004Zhanhai Li PhD Abstract Although meconium ileus (MI) is the earliest manifestation of cystic fibrosis (CF), and is associated with poorer growth, the longitudinal pulmonary progression of CF children with MI is not clear. To test the hypothesis that MI is associated with worse pulmonary outcomes, we prospectively compared from diagnosis to 12 years of age 32 CF children with MI to 50 CF children without MI who were diagnosed during early infancy through neonatal screening. Pulmonary outcome measures included respiratory symptoms, respiratory infections, pathogens, antibiotic usage, hospitalizations, quantitative chest radiology, spirometry, and lung volume determinations. Obstructive lung disease was defined as percent predicted spirometry values below the lower limits of normal. Longitudinal analyses revealed no significant differences in cough, wheezing, respiratory infections, prevalence of and median times to acquisition of Pseudomonas aeruginosa or Staphylococcus aureus, antibiotic usage, and chest radiograph scores between the two groups. However, MI children showed significantly worse forced expiratory volume in 1 sec (FEV1), forced vital capacity (FVC), forced expiratory flow between 25,75% of FVC (FEF25,75), % predicted FEV1, % predicted FEF25,75, and total lung capacity (TLC). These differences were particularly apparent beginning at age 8,10 years. MI children also had higher rates of and shorter median times to obstructive lung disease. Subgroup analyses showed MI children treated surgically and those treated medically had similar pulmonary outcomes. In conclusion, MI children have worse lung function and more obstructive lung disease than those without MI. Such abnormalities are accompanied by reduced lung volume. MI is a distinct CF phenotype with more severe pulmonary dysfunction. Pediatr Pulmonol. 2004; 38:277,284. © 2004 Wiley-Liss, Inc. [source] Usefulness of a program of hospital-supervised physical training in patients with cystic fibrosisPEDIATRIC PULMONOLOGY, Issue 2 2004Attilio Turchetta MD Abstract Exercise is an important part of normal childhood, but the ability to exercise may be impaired in chronic lung diseases such as cystic fibrosis (CF). Improving exercise performance by training is very attractive. The aim of the present study was the evaluation of the effects of a physical aerobic training program, performed in the Children's Hospital and Research Institute "Bambino Gesł" (Rome, Italy) in outpatient CF children, supervised by a physician. Twelve patients (mean forced expiratory flow in 1 sec (FEV1), 71%), age range 12,24 years (16.7 ± 4.4 years), were enrolled. They performed a maximal exercise stress test on the treadmill (modified Bruce protocol) with breath-by-breath determination of oxygen consumption (VO2) to maximum at end-exercise; we measured time of exercise (TE), maximal heart rate (Hrmax) in beats per minute (bpm), and maximal systolic blood pressure (SBPm) in mmHg. The program consisted of 12 weeks of training twice a week. Each training session consisted of walking or running on the treadmill for 30 min at the speed that allowed the child to attain 60% of the maximal heart rate obtained during a baseline stress test for 4 weeks, 70% in the following 4 weeks, and 80% in the last 4 weeks, under strict medical supervision. HR was continously monitored. There was no change in FEV1 and forced vital capacity after the treatment period. Hrmax and SBPm also remained the same (P,=,0.37 and P,=,0.25, respectively). There was a significant increase in TE (P,<,0.002), VO2, VO2/kg, and pulmonary ventilation (VE) (P,<,0.0001, P,<,0.001, and P,<,0.001, respectively). This pilot study showed that a simple training program improves short-term cardiopulmonary fitness in children with CF. Further studies with a larger sample and for a more prolonged time are necessary to assess if sport can have a long-term effect on lung function or survival in CF patients. Pediatr Pulmonol. 2004; 38:115,118. © 2004 Wiley-Liss, Inc. [source] Inconclusive Cystic Fibrosis neonatal screening results: long-term psychosocial effects on parentsACTA PAEDIATRICA, Issue 12 2009Sandra Perobelli Abstract Aim:, Cystic Fibrosis (CF) Newborn Screening occasionally identifies neonates where a CF diagnosis can neither be confirmed nor excluded. To assess how parents of these infants cope with this ambiguous situation. Methods:, Parents of 11 children with Ambiguous Diagnosis (group AD) were compared with parents of 11 children diagnosed with CF through neonatal screening [group Cystic Fibrosis Diagnosis (CFD)] and with parents of 11 Healthy Control children (group HC) matched for gender and age. Results:, The emotional reaction to the inconclusive result was less pronounced in AD than in CFD (p = 0.003), and AD parents considered their infants as healthy as controls. Parents' anxiety about their child's health is stronger in CFD than in AD (p < 0.05) and HC (p < 0.001). Long-term emotional distress was rated similarly in AD and CFD, and greater than in HC (p = 0.0003). The parent/child relationship was less influenced in AD than in the CF group (p = 0.03). Seven AD and CFD parents changed their family planning projects. Conclusion:, Inconclusive neonatal screening results appear to be understood and associated with lower anxiety levels than CF diagnosis. Concern about the child's health is similar to healthy controls and lower than in parents of CF children. [source] Relationship between impulse oscillometry and spirometric indices in cystic fibrosis childrenACTA PAEDIATRICA, Issue 6 2009Ludovic Moreau Abstract Background: The aim of our retrospective study was to determine the relationship between impulse oscillometry (IOS) data and spirometric tests in cystic fibrosis (CF) children. Methods: Thirty CF children aged 4,19 years have performed lung function tests (LFT). A subset of 15 patients repeated LFT on five separate occasions. IOS parameters were respiratory resistance (Rrs), reactance (Xrs) and impedance at 5 Hz (R5, X5, Zr) and the resonant frequency (Fres). Spirometry indices (SI) included forced expiratory volume in 1 sec (FEV1), forced expiratory flow during the middle half of FVC (FEF25,75) and forced vital capacity (FVC). Results: An inverse relationship was observed between raw values of R5, Zr, Fres and SI respectively, and X5 correlated positively with SI. Although significant, these correlations were poor. Receiver operating characteristic curves (ROC) were constructed to identify cutoff points for IOS parameters to discriminate between children according to predefined FEV1 thresholds (percent predicted), generally used to categorize the level of lung function impairment. No acceptable cutoff points can be found for IOS parameters. Trends analyses in the subgroup of 15 patients showed a significant decline of FEV1 between the first and the fifth evaluation. None of the IOS indices demonstrated a consistent tendency, apart from a slight decrease of Fres. Conclusion: IOS measurements presented an insufficient sensitivity to detect and follow bronchial obstruction in CF patients. [source] Reduced gains in fat and fat-free mass, and elevated leptin levels in children and adolescents with cystic fibrosisACTA PAEDIATRICA, Issue 9 2004ML Ahmed Aim: Bodyweight is an important prognostic indicator in children with cystic fibrosis (CF), but the relationships between body composition and clinical outcomes are less clear. We have investigated the role of leptin (a potential satiety factor) and changes in body composition, height and weight with respect to age and clinical outcome. Methods: 143 children (77 boys) with CF and a median age (range) of 5.99 (2.27,17.98) y were followed with annual measurements of height, weight, skinfolds, forced expiratory volume (FEV1), Shwachman score assessment and fasting blood sample. Our control group comprised 40 children (20 boys, 20 girls) aged 8.6,10.2 y at recruitment who were participating in a longitudinal study of growth and puberty. Results: SD scores for height, weight and BMI decreased with age; fat and fat-free mass was lower in both sexes compared to controls. Shwachman score decreased with age in both sexes and was related to fat-free mass in girls, and to both fat-free and fat mass in boys. FEV1 decreased with age only in boys and was related to fat-free mass. Leptin levels by age and by fat mass were higher in CF children compared to controls. Conclusion: Despite improvements in management, contemporary children with CF still gain less body fat and fat-free mass and are shorter than controls. The higher leptin levels we observed may be due to stimulatory effects of inflammatory cytokines and we postulate that they may contribute to the anorexia, poor weight gain and growth of these children. [source] | ||||||||||