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Cystic fibrosis [keywords]
- Correlation of intracystic pressure with cyst volume, length of common channel, biochemical changes in bile and histopathological changes in liver in choledochal cyst. [Journal Article]
- J Indian Assoc Pediatr Surg 2014 Jan; 19(1):10-6.
This study was undertaken to establish correlation between intracholedochal cystic pressure (ICCP) with biochemical changes in bile, liver histology, cyst wall histology, length of common channel, and cyst volume.In this cross-sectional study, ICCP was measured after surgical access before any mobilization by direct cyst cannulation. Bile was then aspirated for biochemical estimation (bilirubin, amylase, lipase, pH, and electrolytes). Common channel length and cyst volume were determined by preoperative magnetic resonance cholangiopancreatography (MRCP). Liver histology was assessed under parenchymal, bile duct, and portal parameters. Cyst wall was examined for ulceration, inflammation, fibrosis, and metaplasia.ICCP was recorded in 31 patients; mean and median ICCP were 15.64 and 14 mmHg, respectively (range = 6-30 mmHg). Cases with median ICCP < 14 had median cyst volume of 48 cc (range = 36-115) and amylase 2052 IU/L (range = 190-5052) whereas those with ICCP ≥ 15 had volume of 20 cc (range = 10-100) (P = 0.004) and amylase 36 IU/L (range = 0-2806) (P = 0.0004) suggesting inverse correlation. No significant correlation was found with bilirubin and electrolytes. ICCP directly correlated with parenchymal changes like hepatocellular damage (P = 0.002) and cholestasis (P = 0.001). It also correlated with bile duct changes. ICCP inversely correlated with cyst wall changes (P = 0.003, 0.0001, 0.023, 0.0013, respectively). High pressure cysts had normal pancreaticobiliary junction.High-pressure cysts tend to be smaller but have more severe backpressure changes in liver parenchyma. Low-pressure cysts have high volume and higher levels of amylase and lipase and therefore have more severe cyst wall changes.
- Vaccines for preventing influenza in people with cystic fibrosis. [JOURNAL ARTICLE]
- Cochrane Database Syst Rev 2014 Mar 6.:CD001753.
Viral respiratory tract infections in people with cystic fibrosis have a deteriorating effect on their lung function and disease progression. Annual influenza vaccination is therefore commonly recommended for people with cystic fibrosis.To assess the effectiveness of influenza vaccination for people with cystic fibrosis.We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register which comprises of references identified from comprehensive electronic database searches and handsearching of relevant journals and abstract books of conference proceedings. We also contacted the companies which market the influenza vaccines used in the trials to obtain further information about randomised controlled trials.Date of the most recent search of the Cochrane Cystic Fibrosis and Genetic Disorders Group's Cystic Fibrosis Trials Register: 08 July 2013.All randomised and quasi-randomised trials (published or unpublished) comparing any influenza vaccine with a placebo or with another type of influenza vaccine.Two authors independently assessed study quality and extracted data. Additional information was obtained by contacting the investigators when it was indicated.Four studies enrolling a total of 179 participants with cystic fibrosis (143 (80%) were children aged 1 to 16 years) were included in this review. There was no study comparing a vaccine to a placebo or a whole virus vaccine to a subunit or split virus vaccine. Two studies compared an intranasal applied live vaccine to an intramuscular inactivated vaccine and the other two studies compared a split virus to a subunit vaccine and a virosome to a subunit vaccine (all intramuscular). The incidence of all reported adverse events was high depending on the type of influenza vaccine. The total adverse event rate ranged from 48 out of 201 participants (24%) for the intranasal live vaccine to 13 out of 30 participants (43%) for the split virus vaccine. With the limitation of a statistical low power there was no significant difference between the study vaccinations. None of the events were severe. All study influenza vaccinations generated a satisfactory serological antibody response. No study reported other clinically important benefits.There is currently no evidence from randomised studies that influenza vaccine given to people with cystic fibrosis is of benefit to them. There remains a need for a well-constructed clinical study, that assesses the effectiveness of influenza vaccination on important clinical outcome measures.
- Psychological therapies for thalassaemia. [JOURNAL ARTICLE]
- Cochrane Database Syst Rev 2014 Mar 6.:CD002890.
Thalassaemia is a group of genetic blood disorders characterised by the absence or reduction in the production of haemoglobin. Severity is variable from less severe anaemia, through thalassaemia intermedia, to profound severe anaemia (thalassaemia major). In thalassaemia major other complications include growth retardation, bone deformation, and enlarged spleen. Blood transfusion is required to treat severe forms of thalassaemia, but this results in excessive accumulation of iron in the body (iron overload), removed mostly by a drug called desferrioxamine through 'chelation therapy'. Non-routine treatments are bone marrow transplantation (which is age restricted), and possibly hydroxyurea, designed to raise foetal haemoglobin level, thus reducing anaemia. In addition, psychological therapies seem appropriate to improving outcome and adherence to medical treatment.To examine the evidence that in people with thalassaemia, psychological treatments improve the ability to cope with the condition, and improve both medical and psychosocial outcomes.We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Haemoglobinopathies Trials Register which comprises of references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings. Searches on the Internet were also performed.Date of the most recent search of the Group's Haemoglobinopathies Trials Register: 11 November 2013.All randomised or quasi-randomised controlled trials comparing the use of psychological intervention to no (psychological) intervention in people with thalassaemia.No trials of psychological therapies have been found in the literature for inclusion in this review.There are currently no results to be reported.As a chronic disease with a considerable role for self-management, psychological support seems appropriate for managing thalassaemia. However, from the information currently available, no conclusions can be made about the use of specific psychological therapies in thalassaemia. This systematic review has clearly identified the need for well-designed, adequately-powered, multicentre, randomised controlled trials assessing the effectiveness of specific psychological interventions for thalassaemia.
- The Tumor Necrosis Factor α (-308 A/G) Polymorphism Is Associated with Cystic Fibrosis in Mexican Patients. [Journal Article]
- PLoS One 2014; 9(3):e90945.
Environmental and genetic factors may modify or contribute to the phenotypic differences observed in multigenic and monogenic diseases, such as cystic fibrosis (CF). An analysis of modifier genes can be helpful for estimating patient prognosis and directing preventive care. The aim of this study is to determine the association between seven genetic variants of four modifier genes and CF by comparing their corresponding allelic and genotypic frequencies in CF patients (n = 81) and control subjects (n = 104). Genetic variants of MBL2 exon 1 (A, B, C and D), the IL-8 promoter (-251 A/T), the TNFα promoter (TNF1/TNF2), and SERPINA1 (PI*Z and PI*S) were tested in CF patients and control subjects from northeastern Mexico by PCR-RFLP.The TNF2 allele (P = 0.012, OR 3.43, 95% CI 1.25-9.38) was significantly associated with CF under the dominant and additive models but was not associated with CF under the recessive model. This association remained statistically significant after adjusting for multiple tests using the Bonferroni correction (P = 0.0482). The other tested variants and genotypes did not show any association with the disease.An analysis of seven genetic variants of four modifier genes showed that one variant, the TNF2 allele, appears to be significantly associated with CF in Mexican patients.
- Adaptation of Pseudomonas aeruginosa in Cystic Fibrosis Airways Influences Virulence of Staphylococcus aureus In Vitro and Murine Models of Co-Infection. [Journal Article]
- PLoS One 2014; 9(3):e89614.
Cystic fibrosis (CF) airways disease represents an example of polymicrobial infection whereby different bacterial species can interact and influence each other. In CF patients Staphylococcus aureus is often the initial pathogen colonizing the lungs during childhood, while Pseudomonas aeruginosa is the predominant pathogen isolated in adolescents and adults. During chronic infection, P. aeruginosa undergoes adaptation to cope with antimicrobial therapy, host response and co-infecting pathogens. However, S. aureus and P. aeruginosa often co-exist in the same niche influencing the CF pathogenesis. The goal of this study was to investigate the reciprocal interaction of P. aeruginosa and S. aureus and understand the influence of P. aeruginosa adaptation to the CF lung in order to gain important insight on the interplay occurring between the two main pathogens of CF airways, which is still largely unknown. P. aeruginosa reference strains and eight lineages of clinical strains, including early and late clonal isolates from different patients with CF, were tested for growth inhibition of S. aureus. Next, P. aeruginosa/S. aureus competition was investigated in planktonic co-culture, biofilm, and mouse pneumonia model. P. aeruginosa reference and early strains, isolated at the onset of chronic infection, outcompeted S. aureus in vitro and in vivo models of co-infection. On the contrary, our results indicated a reduced capacity to outcompete S. aureus of P. aeruginosa patho-adaptive strains, isolated after several years of chronic infection and carrying several phenotypic changes temporally associated with CF lung adaptation. Our findings provide relevant information with respect to interspecies interaction and disease progression in CF.
- Macrolide therapy in adults and children with non-cystic fibrosis bronchiectasis: a systematic review and meta-analysis. [Journal Article]
- PLoS One 2014; 9(3):e90047.
A systematic review and meta-analysis was conducted to evaluate the efficacy and safety of macrolide therapy in adults and children with bronchiectasis.We searched the PUBMED, EMBASE, CENTRAL databases to identify relevant studies. Two reviewers evaluated the studies and extracted data independently. The primary outcome was the number of bronchiectasis exacerbations. Secondary outcomes included exacerbation-related admissions, quality of life (QoL), spirometry, 6-minute walk test (6MWT) and adverse events.Nine eligible trials with 559 participants were included. Six were conducted on adults, and the remaining on children. Macrolide therapy significantly reduced the number of patients experiencing one or more exacerbation in adults [risk ratio (RR) = 0.59; 95% CI, 0.40 to 0.86; P = 0.006; I2 = 65%] and children [RR = 0.86; 95% CI, 0.75-0.99; P = 0.04; I2 = 0%], but not the number of patients with admissions for exacerbation. Macrolide therapy was also associated with reduced frequency of exacerbations in adults (RR = 0.42; 95% CI, 0.29 to 0.61; P<0.001; I2 = 64%) and children (RR = 0.50; 95% CI, 0.35 to 0.71; P<0.001). Pooled analyses suggested that spirometry, including FEV1 and FVC, were significantly improved in adults but not in children. Macrolide therapy improved the QoL (WMD, -6.56; 95% CI, -11.99 to -1.12; P = 0.02; I2 = 86%) but no significant difference in 6MWT (WMD, 4.15; 95% CI, -11.83 to 20.13; P = 0.61; I2 = 31%) and the overall adverse events (RR, 0.96; 95% CI, 0.82 to 1.13; P = 0.66; I2 = 0%) in adults. However, reports of diarrhea and abdominal discomforts were higher with macrolide therapy.Macrolide maintenance therapy, both in adults and children, was effective and safe in reducing bronchiectasis exacerbations, but not the admissions for exacerbations. In addition, macrolide administration in adults was associated with improvement in QoL and spirometry, but not 6WMT. Future studies are warranted to verify the optimal populations and clarify its potential effects on antimicrobial resistance.
- Evaluation of micronutrient profile of North Indian children with cystic fibrosis: a case control study. [JOURNAL ARTICLE]
- Pediatr Res 2014 Mar 6.
Background:The micronutrient levels in cystic fibrosis (CF) children are not available from developing countries where nutritional profile of children is quite different from western countries.Methods:Levels of fat soluble vitamins (A, D, E) and trace metals (iron, copper, zinc) were measured in 27 CF cases and 27 controls.Results:CF cases had significantly low levels of all studied micronutrients compared to controls and the levels were even lower in cases with exacerbation than stable CF cases. Prevalence of vitamins D, vitamin E, iron, copper, and zinc deficiency was significantly high in cases than controls, whereas vitamin A deficiency was almost equal in both groups.Conclusions:The prevalence of vitamins A, D, E and iron, copper, and zinc deficiency was high in CF cases and their levels were significantly lower in cases than controls. CF cases should be regularly monitored for these micronutrients and appropriate supplementation should be considered.Pediatric Research (2014); doi:10.1038/pr.2014.30.
- Defining severity in non-cystic fibrosis bronchiectasis. [Journal Article]
- Expert Rev Respir Med 2014 Apr; 8(2):249-62.
Bronchiectasis is a common disease with important impacts on patient's quality of life and on healthcare systems in terms of healthcare costs, hospitalisations and mortality. After decades of being regarded as an orphan disease there are an increasing numbers of treatments available, or in late-stage clinical trials. Assessment of disease severity is fundamental to clinical management. To date, however, there is no agreed definition of what constitutes mild, moderate or severe bronchiectasis. These terms are often applied to radiological appearances, but while important, computed tomography findings are not sufficiently precise to capture the complex impact of the disease. Studies are beginning to define the risk factors for mortality, hospital admissions, exacerbations and impaired quality of life in bronchiectasis, allowing us to propose new definitions of what constitutes severe bronchiectasis. An improved understanding of severity and prognosis in this disease will aid clinical decision making and the application of new therapies.
- Laser photocoagulation in patients with retinopathy of prematurity undergoing chest physiotherapy. [Journal Article]
- J Pediatr Ophthalmol Strabismus 2013.:e4-5.
The authors report the correlation between chest physiotherapy and increased risk of vitreous hemorrhage after laser photocoagulation therapy in patients with retinopathy of prematurity (ROP). Two premature neonates, the first born at 26 weeks and the second at 31 weeks gestational age, both underwent chest physiotherapy, the first for congenital cystic fibrosis and bronchopulmonary dysplasia and the second for premature alveolar atelectasis. Both were diagnosed as having ROP that necessitated laser photocoagulation treatment at 34 and 37 weeks, respectively. The first patient developed bilateral vitreous hemorrhage and bilateral cataracts. The second patient developed vitreous hemorrhage in one eye. Chest physiotherapy in neonates with ROP significantly increases the risk of hemorrhage after laser photocoagulation in an otherwise fragile retinal vasculature.