CF.

Distribution by Scientific Domains
Distribution within Medical Sciences


Selected Abstracts


Evidence for non-genomic transmission of ecological information via maternal behavior in female rats

GENES, BRAIN AND BEHAVIOR, Issue 1 2007
J. McLeod
Maternal behavior is flexible and programs offspring development. Using a novel manipulation, we demonstrate that rat maternal behavior is sensitive to ecologically relevant stimuli. Long-Evans hooded rat dams (F0) and pups were exposed to a predator condition (cat odor) or a control condition (no odor) for 1 h on the day of parturition. Predator-exposed F0 dams displayed significantly more maternal behavior (licking/grooming, arched-back nursing) relative to control-exposed dams across five subsequent observation days. Female offspring (F1) were raised to adulthood, bred and maternal behavior was observed. F1 dams reared by a predator-exposed F0 dam displayed significantly higher maternal behavior relative to F1 dams reared by a control-exposed F0 dam across 5 days of observation. Increased levels of maternal behavior in predator-reared (PR) F1 dams were evident even in F1 females that had been cross-fostered (CF) from a control-exposed F0 dam, suggesting a non-genomic transmission of increased levels of maternal behavior. Lactating PR F1 dams had significantly elevated estrogen receptor , and , mRNA in the medial preoptic area relative to control-reared (CR) F1 dams. Furthermore, among CR F1 dams, there was no significant difference between those dams that had been CF from predator-exposed F0 dams and those that had been sham CF. These results support the hypothesis that flexible rat maternal behavior can shape offspring development according to current environmental conditions. The results also suggest that estrogen signaling may be part of an epigenetic mechanism by which changes in maternal behavior are passed from F0 to F1 dams. [source]


Evaluation and use of a synthetic quality control material, included in the European external quality assessment scheme for cystic fibrosis,

HUMAN MUTATION, Issue 8 2008
Sarah Berwouts
Abstract Assuring high quality within the field of genetic testing is fundamental, as the results can have considerable impact on the patient and his or her family. The use of appropriate quality control (QC) samples is therefore essential. Diagnostic laboratories mainly use patient samples as QC material, which of course include a maximum of two mutations per sample. Bearing in mind that some assays (such as for cystic fibrosis [CF] testing) can test for more than 100 mutations, multiplex QC materials including more than two mutations could save valuable time and reagents. Based on this need, synthetic multiplex controls have been developed by Maine Molecular Quality Controls, Inc. (MMQCI) for CF. A synthetic control, containing six homozygous mutations and one polymorphism for CF transmembrane conductance regulator (CFTR), was evaluated by distributing it through the CF external quality assessment (EQA) scheme, along with the EQA samples in 2005. A total of 197 participants returned results of the yearly EQA scheme and 133 laboratories participated in the evaluation of the synthetic sample. Respectively, 76% and 73% of the participants were assigned as successful. This evaluation study revealed that the multiplex QC material performed well in the majority of assays and could be useful in method validation, as a tool to challenge interpretation skills, and as potential proficiency testing (PT) material. Hum Mutat 0, 1,8, 2008. © 2008 Wiley-Liss, Inc. [source]


Thermal stability and ablation properties of silicone rubber composites

JOURNAL OF APPLIED POLYMER SCIENCE, Issue 2 2008
Eung Soo Kim
Abstract Effects of incorporation of clay and carbon fiber (CF) into a high temperature vulcanized (HTV) silicone rubber, i.e., poly(dimethylsiloxane) (PDMS) containing vinyl groups, on its thermal stability and ablation properties were explored through thermogravimetric analyses (TGA) and oxy-acetylene torch tests. Natural clay, sodium montmorillonite (MMT), was modified with a silane compound bearing tetra sulfide (TS) groups to prepare MMTS4: the TS groups may react with the vinyl groups of HTV and enhance the interfacial interaction between the clay and HTV. MMTS4 layers were better dispersed than MMT layers in the respective composites with exfoliated/intercalated coexisting morphology. According to TGA results and to the insulation index, the HTV/MMTS4 composite was more thermally stable than HTV/MMT. However, addition of CF to the composites lowered their thermal stability, because of the high thermal conductivity of CF. The time elapsed for the composite specimen, loaded with a constant weight, to break off after the oxy-acetylene flame bursts onto the surface of the specimen was employed as an index for an integrated assessment of the ablation properties, simultaneously taking into consideration the mechanical strength of the char and the rate of decomposition. The elapsed time increased in the order of: HTV < HTV/CF , HTV/MMTS4 < HTV/CF/MMTS4 , HTV/MMT < HTV/CF/MMT. This order was different from the increasing order of the thermal stability determined by TGA results and the insulation index. The decreased degree of crosslinking of the composites with MMTS4 compared with that of the composite with MMT may be unfavorable for the formation of a mechanically strong char and could lead to early rupture of the HTV/MMTS4 specimen. © 2008 Wiley Periodicals, Inc. J Appl Polym Sci, 2008 [source]


Melt mixing of carbon fibers and carbon nanotubes incorporated polyurethanes

JOURNAL OF APPLIED POLYMER SCIENCE, Issue 1 2008
Shahrul Azam Abdullah
Abstract Polyurethane composites filled with carbon fibers (CF) and carbon nanotubes (CNT) were prepared by mixing and injection molding, and its mechanical as well as their thermal properties were investigated. Dynamic mechanical analysis (DMA), thermogravimetry analysis (TGA), and thermal conductivity tests were done, and the properties were evaluated as a function of the filler concentration. The storage modulus of the composites increased with fillers concentration, which also mean the increase of the stiffness, suggest a good adhesion between the polyurethane matrix and the fillers. Addition of more CF and CNT to the composites broadened and lowered the peak of tan , specifies that the polyurethane composite became more elastic because there is a good adhesion between the fillers and the matrix. The addition of carbon fillers improves the thermal stability of the polyurethane. The inclusions of CNT show a better thermal stability when compared with CF. The addition of carbon fillers also increased the thermal conductivity of the polyurethane composites. © 2008 Wiley Periodicals, Inc. J Appl Polym Sci, 2008 [source]


Recycled carbon fiber filled polyethylene composites

JOURNAL OF APPLIED POLYMER SCIENCE, Issue 3 2008
Tony McNally
Abstract Composites of recycled carbon fiber (CF) with up to 30 wt % loading with polyethylene (PE) were prepared via melt compounding. The morphology of the composites and the degree of dispersion of the CF in the PE matrix was examined using scanning electron microscopy, and revealed the CF to be highly dispersed at all loadings and strong interfacial adhesion to exist between the CF and PE. Raman and FTIR spectroscopy were used to characterize the surface chemistry and potential bonding sites of recycled CF. Both the Young's modulus and ultimate tensile stress increased with increasing CF loading, but the percentage stress at break was unchanged up to 5 wt % loading, then decreased with further successive addition of CF. The effect of CF on the elastic modulus of PE was examined using the Halpin-Tsai and modified Cox models, the former giving a better fit with the values determined experimentally. The electrical conductivity of the PE matrix was enhanced by about 11 orders of magnitude on addition of recycled CF with a percolation threshold of 7 and 15 wt % for 500-,m and 3-mm thick samples. © 2007 Wiley Periodicals, Inc. J Appl Polym Sci, 2008 [source]


Genetic Determination of Colles' Fracture and Differential Bone Mass in Women With and Without Colles' Fracture

JOURNAL OF BONE AND MINERAL RESEARCH, Issue 7 2000
Hong-Wen Deng
Abstract Osteoporotic fractures (OFs) are a major public health problem. Direct evidence of the importance and, particularly, the magnitude of genetic determination of OF per se is essentially nonexistent. Colles' fractures (CFs) are a common type of OF. In a metropolitan white female population in the midwestern United States, we found significant genetic determination of CF. The prevalence (K) of CF is, respectively, 11.8% (±SE 0.7%) in 2471 proband women aged 65.55 years (0.21), 4.4% (0.3%) in 3803 sisters of the probands, and 14.6% (0.7%) in their mothers. The recurrence risk (K0), the probability that a woman will suffer CF if her mother has suffered CF is 0.155 (0.017). The recurrence risk (Ks), the probability that a sister of a proband woman will suffer CF given that her proband sister has suffered CF is 0.084 (0.012). The relative risk , (the ratio of the recurrence risk to K), which measures the degree of genetic determination of complex diseases such as CF, is 1.312 (0.145; ,0) for a woman with an affected mother and 1.885 (0.276; ,s) for a woman with an affected sister. A ,-value significantly greater than 1.0 indicates genetic determination of CF. The terms ,0 and ,s are related to the genetic variances of CF. These parameters translate into a significant and moderately high heritability (0.254 [0.118]) for CF. These parameters were estimated by a maximum likelihood method that we developed, which provides a general tool for characterizing genetic determination of complex diseases. In addition, we found that women without CF had significantly higher bone mass (adjusted for important covariates such as age, weight, etc.) than women with CF. [source]


Vitamin K prescribing patterns and bone health surveillance in UK children with cystic fibrosis

JOURNAL OF HUMAN NUTRITION & DIETETICS, Issue 6 2007
D. 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]


Sex differences in weight perception and nutritional behaviour in adults with cystic fibrosis

JOURNAL OF HUMAN NUTRITION & DIETETICS, Issue 2 2001
S. Walters
Introduction Good nutritional status in cystic fibrosis (CF) is associated with improved clinical status and survival. In some conditions where dietary and pharmacological treatment are important (e.g. diabetes), a combination of eating disorders and failure of treatment compliance has been reported. Cases of eating disorders have been reported in CF. Societal pressures on young women to remain slim may compromise optimum clinical management as women are content to remain underweight. Objective To determine whether women have different perception of their weight than men with CF and whether this manifests in different nutritional behaviour. Methods Confidential postal questionnaire to 1870 adults with CF in 1994 known to the Association of Cystic Fibrosis Adults (UK). Participants were asked their own weight and height, and their perception of their weight. Very underweight was defined as < 85% ideal body weight, underweight as 85,94%, normal weight as 95,104%, overweight as 105,114% and very overweight as 115% and over. Results A significantly higher proportion of women than men who are very underweight (29% vs. 11%) or underweight (41% vs. 15%) saw themselves as being of normal weight or overweight. Conversely, a significantly higher proportion of men than women who were normal weight (42% vs. 19%) saw themselves as underweight. All P -values < 0.01. A significantly higher proportion of people who perceived themselves to be underweight were taking oral or enteral food supplements compared with those who did not (77% vs. 30%, P < 0.05). Multiple logistic regression showed that perception of self as underweight was the strongest predictor of taking oral or enteral food supplements (adjusted odds ratio 2.42), even after adjustment for age, sex, overall severity score, body mass index, and seeing a dietitian in the last year. Conclusion Young women with CF tend to overestimate their weight, and young men with CF underestimate their weight when compared with their actual body weight. Perception of self as underweight is reflected in nutritional behaviour, being a significant predictor of taking oral and enteral food supplements. People working with young patients with CF should be aware of these sex differences in weight perception, and work with young women and young men with CF to achieve a realistic perception of body weight and realistic nutritional goals. There is a need for further research into body image, weight perception, eating behaviour and adherence to pancreatic enzyme supplementation and oral and enteral food supplementation in young patients with CF. [source]


Effect of natural and controlled fermentation on flatus-producing compounds of beans (Phaseolus vulgaris)

JOURNAL OF THE SCIENCE OF FOOD AND AGRICULTURE, Issue 10 2003
Marisela Granito
Abstract Fermentation of grain legumes is an efficient method to reduce the concentration of ,-galactosidic compounds that are known to be flatulence producers. Soluble dietary fibre has also been implicated in flatulence production; however, little information exists about the effectiveness of fermentation in diminishing the effects of these compounds. The objective of this work was to study the effect of natural fermentation (NF) and controlled fermentation (CF) on the content of ,-galactosides and dietary fibre in dry beans (Phaseolus vulgaris) for 24, 48, 72 and 96 h. After 48 h, the pH during NF dropped from 6.15 to 4.00 and the nominal acidity increased six times; for CF, however, although the decrease in pH was similar to that for NF, the nominal acidity increased only three times after 48 h. Insoluble fibre content did not change the pH significantly after 96 h for NF and CF. Soluble fibre suffered an apparent removal after 48 h of NF and underwent a sharp reduction of 66% after 96 h of CF. The concentration of stachyose (the main ,-galactoside in raw beans) diminished notably after 48 h and 96 h NF (72% and 95% respectively), whereas with CF only 11% was removed after 96 h. NF of P vulgaris seems to be more effective than CF in reducing the flatulence-producer factors (,-galactosides and soluble dietary fibre). Copyright © 2003 Society of Chemical Industry [source]


Prevalence of incontinence and incontinence-specific quality of life impairment in women with cystic fibrosis,,

NEUROUROLOGY AND URODYNAMICS, Issue 8 2009
M. Vella
Abstract Aims Cystic fibrosis (CF) is the commonest autosomal recessive disorder in Caucasians. With advancing medical technology, the life expectancy has more than double in the last twenty years. This has led to new unforeseen health problems like urinary incontinence. The aim was to establish the prevalence, symptomatic typology, and quality of life impact of incontinence in a population of women with CF. Methods Women were recruited from two London tertiary referral centres for CF. They were asked to fill in a self- completed anonymous King's Health Questionnaire (KHQ) for assessment of symptoms and quality of life Impairment. Their age was also noted. Descriptive statistics are reported. Correlation was assessed using Pearson's Product Moment Correlation Coefficient (r). Results One hundred and forty six women were approached for enrolment. 98(67%) consented to take part in study. Their mean age was 28. Seventy three (74%) reported symptoms of urinary incontinence, which in all cases was perceived as stress urinary incontinence. Of these 44(60%) reported mild stress incontinence, 16(22%) moderate stress incontinence and 13(18%) severe stress incontinence. Continent and incontinent women perceived their general health to be similarly poor (mean score 40 vs 44 points). Strong positive correiations were found between age and stress urinary incontinence severity (r=0.742; P <0.001) and between age and total King's Health Questionnaire score (r=0.828; P <0.001). Conclusions Women with CF have a higher prevalence of stress urinary incontinence than the general population, with both the prevalence and associated quality of life impairment increasing with age. Neurourol. Urodynam. 28:986,989, 2009. © 2009 Wiley-Liss, Inc. [source]


Effects of a short-term rehabilitation program on airway inflammation in children with cystic fibrosis,,

PEDIATRIC PULMONOLOGY, Issue 6 2010
Alexander Moeller MD
Abstract Background Respiratory therapy in cystic fibrosis (CF) consists of airway clearance, infection control, and reduction of airway inflammation. It is well recognized that physical activity as well as daily chest physiotherapy, enhance airway clearance. We investigated the effects of pulmonary rehabilitation, including physical activity and chest physiotherapy, on airway inflammation in children with CF. Methods Eighteen children with stable CF (six females), aged 8.2,16.2 years, participating in a 3-week multidisciplinary inpatient rehabilitation program were recruited. Assessment at the beginning and the end of the program included clinical score, pulmonary function test, exhaled breath condensate (EBC) and sputum analysis. Sputum supernatant and EBC were analyzed for interleukin (IL)-1b, 6, 8, 10, 12, tumor necrosis factor-alpha (TNF-,) and LTB4. Results Median (IQR) symptom scores decreased from 19 [23] to 16 [21], P,=,0.005. Vital capacity and FVC increased significantly (P,<,0.05). However no difference was found for the total sputum cells and sputum as well as EBC cytokines between the two visits. Significant correlations were found for sputum IL-1 (+), IL-6 (,), and IL-8 (+) to total sputum cell count and neutrophils and for IL-8 to TNF-,. Conclusions We have shown that a short-term inpatient rehabilitation for children with stable CF with intensive physical activity mainly improve subjective clinical symptoms and measures of lung function such as VC and FVC but does not influence airflow obstruction and airway inflammation as assessed by sputum and EBC analysis. Pediatr Pulmonol. 2010; 45:541,551. © 2010 Wiley-Liss, Inc. [source]


Motivational interviewing for adherence problems in cystic fibrosis

PEDIATRIC PULMONOLOGY, Issue 3 2010
Alistair J.A. Duff
Abstract This review focuses on adherence in cystic fibrosis (CF), and the factors known to influence it. In particular, it discusses the importance of effective communication in clinical settings and considers the evidence for the effectiveness of motivational interviewing (MI), to increase adaptation and adherence in physical health and CF. The review also contains an overview of the key concepts of MI, its' practice in medical settings and recommendations on how to adopt MI techniques in the routine care of people with CF. Pediatr Pulmonol. 2010; 45:211,220. © 2010 Wiley-Liss, Inc. [source]


Using index of ventilation to assess response to treatment for acute pulmonary exacerbation in children with cystic fibrosis,

PEDIATRIC PULMONOLOGY, Issue 8 2009
FRACP, Paul D. Robinson MRCPCH
Abstract Background The use of alternative more sensitive measures has become a focus of research in CF. The utility of indexes of ventilation, Lung Clearance Index (LCI) and peak aerobic capacity (peak VO2), were studied as assessment tools in gauging response to intravenous (IV) therapy in acute pulmonary exacerbation, in comparison to the more commonly used index of forced expiratory volume in 1,sec (FEV1). The utility of a previously published clinical score was further explored. Methods Patients aged 8,18 years admitted for IV antibiotic treatment of a pulmonary exacerbation were recruited. Spirometry, plethysmography, multiple breath nitrogen washout, exercise testing, and Cystic Fibrosis Clinical Score (CFCS) were performed on admission and prior to discharge. Results Twenty-eight patients were recruited, with a mean (range) age of 13.7 (8; 17) years, 16 female and 12 male. Mean (range) admission FEV1 was 61.4 (28; 92)% predicted, or z -score ,3.09 (,6.15; ,0.52), FVC 83.0 (38; 120)% predicted, or z -score ,1.71 (,5.66; ,1.17), and Shwachman,Kulczycki 68.9 (50; 90). FEV1 increased by 7.0% (P,<,0.01) from admission to discharge. Mean (range) admission LCI, 10.10 (6.87; 14.83), decreased by 3.8% (P,=,0.03). Mean (range) admission peak VO2 (ml/kg/min), 31.2 (23.4; 45.4), increased on discharge by 6.6% (P,<,0.01). Proposed clinical thresholds, based on the available variability data, highlighted the heterogeneity of response in lung function tests. Mean (range) admission CFCS, 26.5 (19; 39), decreased to 19.9 (13; 31) on discharge, a 25.2% improvement (P,<,0.01). CFCS demonstrated improvement in 27 of 28 patients. Changes in peak VO2 (r,=,,0.50, P,=,0.02) and LCI (r,=,0.48, P,=,0.01) correlated with CFCS change. Conclusions In children with mild-to-moderate CF, whilst statistically significant improvement in both LCI and peak VO2 were seen, heterogeneity of response was evident. The most consistent improvement was seen in CFCS. Correlation of LCI and peak VO2 with change in clinical score (CFCS) was seen. The full clinical significance of these changes in LCI and peak VO2 needs to be evaluated further with additional variability data. The CFCS may be useful in the assessment of response to treatment in CF but requires formal validation. Pediatr Pulmonol. 2009; 44:733,742. © 2009 Wiley-Liss, Inc. [source]


Relation of bone mineral density with clinical and laboratory parameters in pre-pubertal children with cystic fibrosis

PEDIATRIC PULMONOLOGY, Issue 7 2009
Nazan Cobanoglu MD
Abstract To study bone mineral density (BMD) of pre-pubertal cystic fibrosis (CF) children, and its relation with clinical and laboratory parameters, we enrolled 16 CF (8 girls) (4,8 years), and 16 control children (8 girls) (4,8 years). After anthropometric measurements, BMD, serum calcium, phosphorus, total alkaline phosphatase (ALP), 25-hydroxy vitamin D (25-OHD), parathyroid hormone, osteocalcin, tumor necrosis factor (TNF)-,, soluble TNF-, receptor 2 (sTNFR2), and soluble IL-2 receptor (sIL-2R) levels, and urinary calcium and hydroxyproline excretions were assessed. Disease severity of CF patients was determined with Shwachman,Kulczycki clinical and Brasfield radiological scoring systems. The mean Shwachman,Kulczycki and Brasfield scores of CF patients were indicating well-controlled disease. The anthropometric measurements, mean BMD values, and serum calcium, phosphorus and parathyroid hormone levels were within normal range and similar in both groups. Serum osteocalcin levels were lower, and ALP and 25-OHD levels were higher in CF. Although 24-hr urinary calcium excretions was higher in CF patients, hydroxyproline excretions were similar in both groups. There was no difference between two groups for the serum levels of sIL-2R, TNF-, and sTNFR2. Children with low vertebral z -scores had higher serum sIL-2R levels in both groups, but the same relation could not be shown for TNF-, and sTNFR2. We may speculate that younger, healthier and well-nourished patients with CF may have normal BMD, but the bone disease develop as patients get older because of the other contributing factors. Future well-designed longitudinal studies with large cohorts might show a relation with BMD and cytokines in CF. Pediatr Pulmonol. 2009; 44:706,712. © 2009 Wiley-Liss, Inc. [source]


Reproducibility of spirometry during cystic fibrosis pulmonary exacerbations,,

PEDIATRIC PULMONOLOGY, Issue 11 2008
Don B. Sanders MD
Abstract Objectives: To compare the within day variation of spirometry between hospital admission, discharge, and outpatient follow up among children with cystic fibrosis (CF) hospitalized for a pulmonary exacerbation. Hypothesis: Within day variation of spirometry will be greater at hospital admission than at hospital discharge or outpatient follow up. Methods: We performed a retrospective review of spirometry data for all patients with CF ,6 years old admitted to our pediatric CF center for a pulmonary exacerbation in 2004 or 2005. For patients who had previously performed spirometry successfully, measurements were used from one admission only during 2004,2005 if the spirometry occurred within 3 days of hospital admission, 3 days of discharge, or at a follow up clinic visit when well. We compared the within day coefficients of variation (CV) for FVC, FEV1, and FEF25,75 between time points using the Wilcoxon signed rank-test. We also determined the change in spirometry that is likely to be beyond measurement variability during inpatient treatment of a pulmonary exacerbation. Results: Spirometry data were available from 40 subjects at admission and follow up and 35 at hospital discharge. There was no significant difference in CV at admission, discharge, and follow up for FVC, FEV1, or FEF25,75. The mean (SD) CV was 3.1% (2.7) for FVC, 3.2% (2.1) for FEV1, and 9.7% (7.0) for FEF25,75 at admission, 2.8% (2.2) for FVC, 3.1% (2.1) for FEV1, and 8.1% (6.7) for FEF25,75 at discharge, and 2.7% (1.7) for FVC, 2.8% (2.0) for FEV1, and 8.4% (7.8) for FEF25,75 at follow up. These are similar to previous reports of outpatients with CF. The improvement in spirometry that exceeded measurement variability for our cohort was 80 ml for FVC, 70 ml for FEV1, and 220 ml/sec for FEF25,75. Conclusions: The presence of an acute pulmonary exacerbation in children and adolescents with CF does not substantially contribute to the within day variation in spirometry. Within day variation of spirometry for children with CF during pulmonary exacerbations is similar to previously reported values from clinically stable CF patients. Pediatr. Pulmonol. 2008; 43:1142,1146. © 2008 Wiley-Liss, Inc. [source]


Assessment of body composition in pediatric patients with cystic fibrosis

PEDIATRIC PULMONOLOGY, Issue 10 2008
Greg D. Wells PhD
Abstract Rationale Cystic fibrosis (CF) leads to pathological changes in organs that express the cystic fibrosis transmembrane conductance regulator (CFTR), including secretory cells of the digestive tract and the pancreas. Maintaining nutritional sufficiency is challenging for CF patients and therefore accurate monitoring is important for their clinical management. Purpose The objectives of this study were to evaluate the effectiveness of skinfold measurements as an accurate method for determining body composition (fat mass (FM) and lean body mass (LBM)) of this population, using dual-energy X-ray absorptiometry (DEXA) as a gold standard comparison and to determine the most accurate equation for this calculation in children with CF. Methods Fifty-five pediatric patients with CF participated in the study. FM and LBM calculated via four methods: Slaughter, Durnin, Durenberg (2-site and 4-site). The relationship between the methods and DEXA results were estimated by intraclass-correlation coefficient (ICC) and Bland and Altman analyses. Results The Slaughter method was the most accurate (ICC of 0.92 for FM and 0.99 for LBM) and displayed the least bias over the range of FM and LBM in CF patients. In addition, the results of Bland Altman analyses comparing each skinfold method to DEXA, revealed that the results were evenly distributed along the range of values for the Slaughter calculation, whereas the other three methods under and over estimated % fat results at the upper and lower ends of the range respectively. Conclusion We therefore conclude that the Slaughter method may be used for body composition assessment of pediatric CF patients. This provides clinical teams with a simple, accurate and non-invasive method that can be used to monitor nutritional status in pediatric patients with CF. Pediatr Pulmonol. 2008; 43:1025,1032. © 2008 Wiley-Liss, Inc. [source]


Doll-like face: Is it an underestimated clinical presentation of cystic fibrosis?

PEDIATRIC PULMONOLOGY, Issue 7 2008
Mehmet Kose MD
Abstract Cystic fibrosis (CF) is the most prevalant inheritable chronic disease in caucasian children. The clinical syndrome of kwashiorkor is well-recognized complication of CF. The edema of the face can be seen in kwashiorkor. As doll-like face is very rare and underestimated clinical presentation of CF patients complicated with hypoproteinemia we evaluated demographic features and laboratory characteristics of 5 patients diagnosed as CF with doll-like face. Methods: Between June 2005 and January 2008, 115 children were diagnosed as having CF enrolled in our center. Five infants were diagnosed as CF with doll-like face before the age of 6 months participitated in study. Results: The incidence of doll-like face younger than the 6 months of age were 9.4% in our center. 48 infants diagnosed as CF without doll-like face before the age of 6 months participitated in the study as controls (group2). Physical examination revealed doll-like face and pitting edema of lower extremities in group 1. Their weight and length were under the third centile. Laboratory findings of group 1 include: mean hemoglobin 7.6g/dl; mean total protein 4.4 g/dl; albumin 2.3 g/dl. When compared control group in order to; 11.4 g/dl (range 7.6,17.9); 6.2 g/dl (range 4.0,8.8); 4.7 g/dl (range 2.1,5.8). mean hemoglobin, total protein and albumin values were lower in group 1. Conclusion: In a subgroup of patients, doll-like face may be the presenting manifestation of CF. Especially in developing countries clinicians should be aware of in patients with malnutrition and doll-like face and CF should be considered in differential diagnosis. Pediatr Pulmonol. 2008; 43:634,637. © 2008 Wiley-Liss, Inc. [source]


The repeatability of submaximal endurance exercise testing in cystic fibrosis,

PEDIATRIC PULMONOLOGY, Issue 1 2007
MB BCh BAO, Sinead C. Barry BSc
Abstract Submaximal endurance cycle ergometer exercise tests are used to measure the efficacy of an exercise intervention, but the repeatability of these tests in patients with cystic fibrosis (CF) has not been established. The purpose of this study was to examine the repeatability of submaximal endurance testing in stable CF. Fifteen adults with CF underwent two submaximal endurance tests carried out over a 7-day period. A subset of six subjects returned 28 days later for a third submaximal endurance test. Workload was set at 80% of maximum workload and exercise was performed to exhaustion. Oxygen consumption, minute ventilation, tidal volume, carbon dioxide output, respiratory rate, heart rate, and oxygen saturation were measured at rest, at end exercise and at four matched times during the submaximal endurance tests (20, 40, 60, and 80% of exercise duration calculated from the first endurance test). Submaximal endurance test time was highly repeatable with no significant learning effect identified on multiple testing. Submaximal endurance exercise time demonstrated a variability of 5.7% which is consistent with high levels of repeatability. Metabolic, ventilatory and cardiac variables were all also highly reproducible between test days. Submaximal endurance testing is repeatable in stable CF, confirming that submaximal endurance tests are a reliable tool for assessment of therapeutic benefit in patients with CF. Pediatr Pulmonol. 2007; 42:75,82. © 2006 Wiley-Liss, Inc. [source]


Clinical scoring systems in cystic fibrosis

PEDIATRIC PULMONOLOGY, Issue 7 2006
Gaudenz M. Hafen MD
Abstract The first cystic fibrosis (CF) scoring system was published in 1958. Since then, many other scoring systems were developed. Clinical parameters, details about statistical evaluations, and recent strategic uses of scores were identified. Several similar scores aiming to assess chronic illness severity (Shwachman-Kulczycki score and a modification, Cooperman, Berneze-score and the NIH score) have not been evaluated and are out of date, given the changing natural history of CF. Of the current scoring systems, the modified Shwachman score by Doershuk is perhaps most reliable for describing follow-up studies. Scores designed for acute changes and short-term evaluation were also developed. The modified Huang score may be useful in the prognostic evaluation of patients with end-stage disease. It could also be used for discrimination of adult patients with differing disease severity and for longitudinal evaluation. Scores assessing pulmonary exacerbations could help provide consensus among clinicians regarding the need for intervention. Most of these scores require further evaluation. Although scores could provide an objective measure of disease severity, progression, need for and response to interventions, including value in selecting patients for lung transplantation and as an outcome measure for research studdies, no scoring system can fulfill all these objectives. Nevertheless, there is a need for the development of a modern day longitudinal score that is sensitive, valid and reproducible, to reflect the milder disease status of patients. Pediatr Pulmonol. 2006; 41: 602,617. © 2006 Wiley-Liss, Inc. [source]


Effects of sildenafil on pulmonary hypertension and exercise tolerance in severe cystic fibrosis-related lung disease,

PEDIATRIC PULMONOLOGY, Issue 4 2006
Gregory S. Montgomery MD
Abstract Cystic fibrosis (CF) patients with advanced lung disease are at risk for developing pulmonary vascular disease and pulmonary hypertension, characterized by progressive exercise intolerance beyond the exercise-limiting effects of airways disease in CF. We report on a patient with severe CF lung disease who experienced clinically significant improvements in exercise tolerance and pulmonary hypertension without changing lung function during sildenafil therapy. Pediatr Pulmonol. © 2006 Wiley-Liss, Inc. [source]


Processing of CFTR: Traversing the cellular maze,How much CFTR needs to go through to avoid cystic fibrosis?

PEDIATRIC PULMONOLOGY, Issue 6 2005
Margarida D. Amaral PhD
Abstract Biosynthesis of the cystic fibrosis transmembrane conductance regulator (CFTR), like other proteins aimed at the cell surface, involves transport through a series of membranous compartments, the first of which is the endoplasmic reticulum (ER), where CFTR encounters the appropriate environment for folding, oligomerization, maturation, and export from the ER. After exiting the ER, CFTR has to traffic through complex pathways until it reaches the cell surface. Although not yet fully understood, the fine details of these pathways are starting to emerge, partially through identification of an increasing number of CFTR-interacting proteins (CIPs) and the clarification of their roles in CFTR trafficking and function. These aspects of CFTR biogenesis/degradation and by membrane traffic and CIPs are discussed in this review. Following this description of complex pathways and multiple checkpoints to which CFTR is subjected in the cell, the basic question remains of how much CFTR has to overcome these barriers and be functionally expressed at the plasma membrane to avoid CF. This question is also discussed here. Pediatr Pulmonol. © 2005 Wiley-Liss, Inc. [source]


Usefulness of a program of hospital-supervised physical training in patients with cystic fibrosis

PEDIATRIC PULMONOLOGY, Issue 2 2004
Attilio 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]


Standardized procedure for measurement of nasal potential difference: An outcome measure in multicenter cystic fibrosis clinical trials,

PEDIATRIC PULMONOLOGY, Issue 5 2004
Thomas A. Standaert PhD
Abstract Patients with cystic fibrosis (CF) can be discriminated from healthy subjects by measurement of the nasal potential difference, which has become a useful outcome measure for therapies directed toward correcting defective electrolyte transport in CF. A standard operating procedure was developed by a CF Foundation clinical trials network, to be followed by all sites performing collaborative studies. Key variables in the measurement included type of voltmeter, exploring probe, reference electrodes, and solutions used to assess both sodium transport and chloride conductance. Eight sites submitted data on 3,8 normal and 4,5 CF subjects. Baseline voltage, an index of sodium transport, was ,18.2,±,8.3 mV (mean,±,SD) for normals, and ,45.3,±,11.4 mV for CF patients. There was no CFTR-mediated chloride secretion in CF subjects, as evidenced by the lack of response to perfusion with zero chloride,+,beta agonist solutions (+3.2,±,3.5 mV) vs. that in normals (,23.7,±,10.2 mV). The standardized nasal potential difference measurement minimizes variability between operators and study sites. Valid and consistent results can be attained with trained operators and attention to technical details. These data demonstrate the procedure to be sufficient for multicenter studies in the CF Foundation network. Pediatr Pulmonol. 2004; 37:385,392. © 2004 Wiely-Liss, Inc. [source]


Cystic fibrosis and infantile hypertrophic pyloric stenosis: Is there an association?

PEDIATRIC PULMONOLOGY, Issue 5 2002
Khalid S. Kakish MD
Abstract Cystic fibrosis (CF) is the most common lethal genetic disease among Caucasian populations. The generally accepted incidence of CF in the United States is 1 in 3,200 in the Caucasian population. Intestinal obstructions and atresias have been described among patients with CF. An association of CF with infantile hypertrophic pyloric stenosis (IHPS) has not been previously documented. A review in our clinic of 72 patients with CF revealed IHPS in two. The incidence of 2.7% is greater than the 0.3% incidence expected in the general population. This ninefold increase in IHPS in patients with CF suggests an association between the two and warrants further investigation. Pediatr Pulmonol. 2002; 33:404,405. © 2002 Wiley-Liss, Inc. [source]


Acceleration of lung disease in children with cystic fibrosis after Pseudomonas aeruginosa acquisition

PEDIATRIC PULMONOLOGY, Issue 4 2001
Michael R. Kosorok PhD
Abstract As part of the ongoing Wisconsin Cystic Fibrosis (CF) Neonatal Screening Project, we had the unique opportunity to study the longitudinal relationship between Pseudomonas aeruginosa (Pa) acquisition and infection and developing lung disease in children with CF. The primary objective was to determine whether acquisition of Pa was associated with a measurable change in the progression of lung disease. Two outcome measures were used to study 56 patients who were diagnosed through newborn screening: 1) Wisconsin additive chest radiograph score (WCXR), based on the average of scores from a pulmonologist and a radiologist, and 2) the highest forced expired volume in 1 sec (FEV1)/forced vital capacity (FVC) ratio. We used two measures of Pa acquisition: 1) time of first positive protocol-determined oropharyngeal (with cough) culture, and 2) the magnitude of antibody titer detected by ELISA assays, using as antigen a crude cell lysate, purified exotoxin A, or an elastase toxoid prepared from three Pa strains. Other predictor variables included age, pancreatic status, height-for age, and weight-for-age-percentiles. The best regression model for predicting changes in the WCXR included time to first positive culture and antibody titer for Pa elastase. Prior to Pa acquisition, WCXR worsened by 0.45 points/year (P,>,0.25); after Pa acquisition, the rate of worsening increased significantly (P,<,0.001) to 1.40 points/year. Each antibody titer level (log base 2) increased the score by 0.48 points (P,<,0.001). The best regression model for predicting change in the FEV1/FVC included only time to first positive culture. Prior to Pa acquisition, the FEV1/FVC ratio declined by 1.29%/year; after Pa infection, the rate of decrease significantly accelerated to 1.81%/year (P,=,0.001). Our data show that Pa acquisition is associated with declining pulmonary status in children with CF, and that this effect is probably gradual rather than precipitous. Because these patients were diagnosed and treated aggressively, our estimates of the effects of Pa acquisition may be conservative. We also conclude that the WCXR appears to be more sensitive than FEV1/FVC in detecting early changes in lung disease associated with CF. Pediatr Pulmonol. 2001; 32:277,287. © 2001 Wiley-Liss, Inc. [source]


Metabolic alkalosis with hypoelectrolytemia in infants with cystic fibrosis

PEDIATRICS INTERNATIONAL, Issue 3 2002
Stojka Fustik
Abstract Background: Infants with cystic fibrosis (CF) can develop episodes of hyponatremic hypochloremic dehydration with metabolic alkalosis when they sweat excessively, which is not caused by sweating in normal infants. We investigated the incidence of the metabolic alkalosis with hypoelectrolytemia in CF infants, the possible risk factors for its occurrence and the importance of the manifestation in the diagnosis of CF. Methods: In order to evaluate the incidence and the risk factors for the development of this sweat-related metabolic disorder in CF, we reviewed the records of all children diagnosed as having CF before the age of 12 months in a 10-year period. Data analysis included medical history data, clinical features, biochemical parameters (blood pH, serum bicarbonate, sodium, chloride and potassium levels), sweat chloride test values, as well as genetic analysis data. Results: The prevalence of metabolic alkalosis in association with low serum electrolyte concentrations (hyponatremia, hypochloremia, and hypokalemia) in infant CF population in our region was 16.5%. We found no season predilection in its occurrence. Early infant age, breast-feeding, delayed CF diagnosis, heat exhaustion and the presence of severe CF transmembrane conductance regulator mutations are predisposed factors for the development of metabolic alkalosis with hypoelectrolytemia. Conclusions: The results from our study suggest that metabolic alkalosis with hypoelectrolytemia is a relatively common manifestation of CF in infancy. The possibility of CF should be seriously considered in any infant with this metabolic disorder. [source]


Compassion Fatigue and Psychological Distress Among Social Workers: A Validation Study

AMERICAN JOURNAL OF ORTHOPSYCHIATRY, Issue 1 2006
Richard E. Adams PhD
Few studies have focused on caring professionals and their emotional exhaustion from working with traumatized clients, referred to as compassion fatigue (CF). The present study had 2 goals: (a) to assess the psychometric properties of a CF scale, and (b) to examine the scale's predictive validity in a multivariate model. The data came from a survey of social workers living in New York City following the September 11, 2001, terrorist attacks on the World Trade Center. Factor analyses indicated that the CF scale measured multiple dimensions. After overlapping items were eliminated, the scale measured 2 key underlying dimensions,secondary trauma and job burnout. In a multivariate model, these dimensions were related to psychological distress, even after other risk factors were controlled. The authors discuss the results in light of increasing the ability of professional caregivers to meet the emotional needs of their clients within a stressful environment without experiencing CF. [source]


Treatment of lobar atelectasis with bronchoscopically administered recombinant human deoxyribonuclease in cystic fibrosis?

THE CLINICAL RESPIRATORY JOURNAL, Issue 2 2008
Anne Marie McLaughlin
Abstract Introduction:, Recombinant human deoxyribonuclease (rhDNase) reduces sputum viscosity and improves pulmonary function in cystic fibrosis (CF). Objective:, The objective of this study was to describe our experience in which rhDNase (Pulmozyme; Roche, Basel, Switzerland) was administered by bronchoscopic instillation into atelectatic lobes in five adults with CF. Conclusion:, We found this method successful in treating lobar atelectasis, which was resistant to conventional therapy with antibiotics and physiotherapy. In all but one of the cases we described, administration of DNase in this manner resulted in a radiographic and clinical improvement of the atelectasis. We recommend that respiratory physicians consider this as a second line treatment in the management of atelectasis. Please cite this paper as: McLaughlin AM, McGrath E, Barry R, Egan JJ and Gallagher CG. Treatment of lobar atelectasis with bronchoscopically administered recombinant human deoxyribonuclease in cystic fibrosis? The Clinical Respiratory Journal 2008; 2: 123,126. [source]


Births in wild black and gold howler monkeys (Alouatta caraya) in Northern Argentina

AMERICAN JOURNAL OF PRIMATOLOGY, Issue 3 2009
Silvana Peker
Abstract Previous studies on births in nonhuman primates suggest that births are expected to occur at night to avoid predators. Here, we describe birth-related behaviors in wild black and gold howler monkeys, Alouatta caraya and address the various ideas proposed in the literature about the timing of births in group-living nonhuman primates. We collected data on females' birth-related behaviors through continuous focal observations and scan samples. Focal observations on females giving birth were taken for the remainder of the day after noticing a female was in labor. We recorded behaviors and the spatial distribution of the whole group using scan samples taken every 10,min from sunrise to sunset the same day of birth. We recorded five births at the continuous forest (CF) over a 25 months period (January 2004,December 2004 and September 2005,September 2006) and two births in the fragmented forest (FF) over a 13 months period (September 2005,September 2006). From these, four births were during daylight (two at CF and two at FF) and three during the night at CF. Our descriptions of A. caraya births contribute to a growing data set on the timing of parturition in wild nonhuman primates and suggest that a clear pattern of nocturnal births is not universal across nonhuman primate species. Am. J. Primatol. 71:261,265, 2009. © 2008 Wiley-Liss, Inc. [source]


Combined Double Lung,Liver Transplantation for Cystic Fibrosis Without Cardio-Pulmonary By-Pass

AMERICAN JOURNAL OF TRANSPLANTATION, Issue 10 2007
V. Corno
Sequential bilateral single lung-liver transplantation (SBSL-LTx) is a therapeutic option for patients with end stage lung and liver disease (ESLLD) due to cystic fibrosis (CF). A few cases have been reported, all of them were performed with the use of cardio-pulmonary by-pass (CPB). We performed SBSL-LTx in three young men affected by CF. All the recipients had respiratory failure and portal hypertension with hypersplenism. Along with lung transplants, two patients received a whole liver graft and one an extended right graft from an in situ split liver. During transplantation neither CPB nor veno-venous by-pass (VVB) were employed. Immunosuppression was based on basiliximab, tacrolimus, steroids and azathioprine. The three recipients are alive with a median follow-up of 670 days (range 244,1533). Combined SBSL-LTx is a complex but effective procedure for the treatment of ESLLD due to CF, not necessarily requiring the use of CPB or VVB. [source]