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Sample records for generalized cystic resolution

  1. Generalized cystic resolution: a metric for assessing the fundamental limits on beamformer performance.

    PubMed

    Guenther, Drake A; Walker, William F

    2009-01-01

    Existing methods for characterizing the imaging performance of ultrasound systems do not clearly quantify the impact of contrast, spatial resolution, and signal-to-noise ratio (SNR). Although the beamplot, contrast resolution metrics, SNR measurements, ideal observer methods, and contrast-detail analysis provide useful information, it remains difficult to discern how changes in system parameters affect these metrics and clinical imaging performance. In this paper, we present a rigorous methodology for characterizing the pulse-echo imaging performance of arbitrary ultrasound systems. Our metric incorporates the 4-D spatio-temporal system response, which is defined as a function of the individual beamformer channel weights. The metric also incorporates the individual beamformer channel electronic SNR. Whereas earlier performance measures dealt solely with contrast resolution or echo signal-to-noise ratio, our metric combines them so that tradeoffs between these parameters are easily distinguishable. The new metric quantifies an arbitrary system's contrast resolution and SNR performance as a function of cyst size, beamformer channel weights, and beamformer channel SNR. We present a theoretical derivation of the unified performance metric and provide simulation and experimental results highlighting the metric's utility. We compare the fundamental performance limits of 2 beamforming strategies: the dynamic focus finite impulse response (FIR) filter beamformer and the spatial matched filter (SMF) beamformer to the performance of the conventional delay-and-sum (DAS) beamformer. Results from this study show that the SMF beamformer and the FIR beamformer offer significant gains in beamformer SNR and contrast resolution compared with the DAS beamformer, respectively. The metric clearly distinguishes the performance of the SMF beamformer, which enhances system sensitivity, from the FIR beamformer, which optimizes system contrast resolution. Finally, the metric provides one

  2. High spatial resolution magnetic resonance imaging of cystic adventitial disease of the popliteal artery.

    PubMed

    Maged, Ismaeel M; Turba, Ulku C; Housseini, Ahmed M; Kern, John A; Kron, Irving L; Hagspiel, Klaus D

    2010-02-01

    High spatial resolution magnetic resonance imaging (MRI) of patients with cystic adventitial disease can demonstrate connections between cysts in the adventitia and the adjacent joint, which is important for successful treatment. The inability to identify these during surgery can lead to a recurrence; thus, high spatial resolution MRI has the potential to affect therapy. This article presents the high spatial resolution MRI findings of cystic adventitial disease in a series of three consecutive patients and discusses the relevance of these findings to the etiology and therapy.

  3. Cystic resolution: a performance metric for ultrasound imaging systems.

    PubMed

    Ranganathan, Karthik; Walker, William F

    2007-04-01

    This paper describes a metric that can be used to characterize the resolution of arbitrary broadband coherent imaging systems. The metric is particularly suited to medical ultrasound because it characterizes scanner performance using the contrast obtained by imaging anechoic cysts of various sizes that are embedded in a speckle-generating background, accounting for the effect of electronic noise. We present the theoretical derivation of the metric and provide simulation examples that demonstrate its utility. We use the metric to compare a low-cost, handheld, C-scan system under development in our laboratory to conventional ultrasound scanners. We also present the results of simulations that were designed to evaluate and optimize various parameters in our system, including the f/# and apodization windows. We investigate the impact of electronic noise on our system and quantify the tradeoffs associated with quantization in the analog to digital converter. Results indicate that an f/1 receive aperture combined with 10-bit precision and a signal-to-noise ratio (SNR) of 0 dB per channel would result in adequate image quality.

  4. High Spatial Resolution MRI of Cystic Adventitial Disease of the Iliofemoral Vein Communicating with the Hip Joint

    SciTech Connect

    Michaelides, Michael; Pantziara, Maria Ioannidis, Kleanthis

    2013-05-14

    Venous cystic adventitial disease (CAD) is an extremely rare entity, and so far less than 20 cases have been described in the literature. Herein, we describe the imaging findings of CAD of iliofemoral vein in a 51-year-old woman who presented with leg swelling with special emphasis on high spatial resolution MRI, which demonstrated communication of the cyst with the hip joint. To our knowledge, this is the first description of high spatial resolution MRI findings in venous CAD supporting a new theory about the pathogenesis of venous CAD.

  5. Children and adolescents with cystic fibrosis display moderate bone microarchitecture abnormalities: data from high-resolution peripheral quantitative computed tomography.

    PubMed

    Braun, C; Bacchetta, J; Braillon, P; Chapurlat, R; Drai, J; Reix, P

    2017-08-09

    We investigated whether bone microstructure assessed by high-resolution peripheral quantitative tomography (HR-pQCT) could be altered in children and teenagers with cystic fibrosis (CF). In comparison to their healthy counterparts, bone microstructure was mildly affected at the tibial level only. Cystic fibrosis-related bone disease (CFBD) may alter bone health, ultimately predisposing patients to bone fractures. Our aim was to assess bone microstructure using high-resolution peripheral quantitative tomography (HR-pQCT) in a cohort of children and teenagers with CF in comparison to age-, puberty-, and gender-matched healthy volunteers (HVs). In this single-center, prospective, cross-sectional study, we evaluated the HR-pQCT bone parameters of CF patients and compared them to those of the healthy volunteers. At a median age of 15.4 [range, 10.5-17.9] years, 37 CF patients (21 boys) with 91% [range, 46-138%] median forced expiratory volume in 1 s were included. At the ultradistal tibia, CF patients had a smaller bone cross-sectional area (579 [range, 399-1087] mm(2)) than HVs (655 [range, 445-981] mm(2)) (p = 0.027), related to a decreased trabecular area, without any significant differences for height. No other differences were found (trabecular number, separation, thickness, or distribution) at the radial or tibial levels. Bone structure was different in patients receiving ursodeoxycholic acid and those bearing two F508del mutations. In our cohort of children and teenagers with good nutritional and lung function status, bone microstructure evaluated with HR-pQCT was not severely affected. Minimal microstructure abnormalities observed at the tibial level may be related to the cystic fibrosis transmembrane conductance regulator defect alone; the long-term consequences of such impairment will require further evaluation.

  6. 851 resected cystic tumors of the pancreas: A 33-year experience at the Massachusetts General Hospital

    PubMed Central

    Valsangkar, Nakul P.; Morales-Oyarvide, Vicente; Thayer, Sarah P.; Ferrone, Cristina R.; Wargo, Jennifer A.; Warshaw, Andrew L.; Castillo, Carlos Fernández-del

    2013-01-01

    Background The objective of this study was to identify trends in the diagnosis and treatment of cystic neoplasms of the pancreas using a retrospective review of patients from a surgical database at an academic referral center during a 33-year period. Methods Patient characteristics, including demographics, pathology, and survival, were analyzed over 5 time periods between 1978 and 2011. Results A total of 851 consecutive patients underwent resection for a cystic neoplasm of the pancreas during a 33-year period. Sixty-five percent of patients were female, and mean age was 60 years. The most common pathologic diagnoses were intraductal papillary mucinous neoplasm (38%), mucinous cystic neoplasm (23%), serous cystadenoma (16%), and cystic neuroendocrine neoplasm (7%). There was a stepwise increase in the number of resections across time periods (67 between 1978 and 1989; 376 between 2005 and 2011), with a parallel increase in the proportion of incidentally discovered lesions (22% to 50%). Diagnosis of intraductal papillary mucinous neoplasm was very uncommon in the first 2 time periods (before the first recognition of intraductal papillary mucinous neoplasm as a distinct entity) but predominated in the last 2 (41% and 49%), and cystic neuroendocrine neoplasms, which constituted 3% of the cystic neoplasms in the first time-period, now comprise more than 8% of pancreatic cystic neoplasms. The proportion of malignant neoplasms decreased over time (41% between 1978 and 1989; 12% between 2005 and 2011), reflecting probably the earlier diagnosis and treatment of premalignant neoplasms. Although operative mortality was minimal (4/849, 0.5%), the postoperative complication rate was 38%. Overall 5-year survival for all mucinous lesions was 87%. Conclusion Cystic neoplasms of the pancreas are being diagnosed and treated with increasing frequency. At present, most are incidentally discovered intraductal papillary mucinous neoplasms. (Surgery 2012;152:S4–12.) PMID:22770958

  7. Construction of a general human chromosome jumping library, with application to cystic fibrosis

    SciTech Connect

    Collins, F.S.; Drumm, M.L.; Cole, J.L.; Lockwood, W.K.; Woude, G.F.V.; Iannuzzi, M.C.

    1987-02-27

    In many genetic disorders, the responsible gene and its protein product are unknown. The technique known as reverse genetics, in which chromosomal map positions and genetically linked DNA markers are used to identify and clone such genes, is complicated by the fact that the molecular distances from the closest DNA markers to the gene itself are often too large to traverse by standard cloning techniques. To address this situation, a general human chromosome jumping library was constructed that allows the cloning of DNA sequences approximately 100 kilobases away from any starting point in genomic DNA. As an illustration of its usefulness, this library was searched for a jumping clone, starting at the met oncogene, which is a marker tightly linked to the cystic fibrosis gene that is located on human chromosome 7. Mapping of the new genomic fragment by pulsed field gel electrophoresis confirmed that it resides on chromosome 7 within 240 kilobases downstream of the met gene. The use of chromosome jumping should be applicable to any genetic locus for which a closely linked DNA marker is available.

  8. Chapter Iii: Resolutions of the General Assembly

    NASA Astrophysics Data System (ADS)

    2007-12-01

    The members of the Resolutions Committee 2003-2006 were Christopher J. Corbally (chair, USA), Jocely S. Bell Burnell (UK), Matthew Colless (Australia) Georges Meylan (Switzerland), Silvia Torres-Peimbert (Mexico), Rachel L. Webster (Australia), and Robert Williams (USA).

  9. Generalizing the nonlocal-means to super-resolution reconstruction.

    PubMed

    Protter, Matan; Elad, Michael; Takeda, Hiroyuki; Milanfar, Peyman

    2009-01-01

    Super-resolution reconstruction proposes a fusion of several low-quality images into one higher quality result with better optical resolution. Classic super-resolution techniques strongly rely on the availability of accurate motion estimation for this fusion task. When the motion is estimated inaccurately, as often happens for nonglobal motion fields, annoying artifacts appear in the super-resolved outcome. Encouraged by recent developments on the video denoising problem, where state-of-the-art algorithms are formed with no explicit motion estimation, we seek a super-resolution algorithm of similar nature that will allow processing sequences with general motion patterns. In this paper, we base our solution on the Nonlocal-Means (NLM) algorithm. We show how this denoising method is generalized to become a relatively simple super-resolution algorithm with no explicit motion estimation. Results on several test movies show that the proposed method is very successful in providing super-resolution on general sequences.

  10. Resolution of generalized dermatophytosis without treatment in dogs.

    PubMed

    Medleau, L; Chalmers, S A

    1992-12-15

    Natural resolution of generalized dermatophytosis reportedly is common in large-animal species. In dogs with generalized dermatophytosis, antifungal treatment usually is prescribed, because the prevalence of natural resolution of dermatophytosis in dogs is not known. Five dogs, 6 to 18 months old, were diagnosed as having generalized dermatophytosis. Each dog was treated with an inert substance given orally with food, once daily. Signs of disease resolved in 3 of the dogs after 4 to 8 weeks of treatment with the inert substance. Two dogs did not improve, so treatment with ketoconazole was initiated. Resolution was achieved in these 2 dogs after 6 weeks of treatment with ketoconazole.

  11. In utero resolution of microcystic congenital cystic adenomatoid malformation after prenatal betamethasone therapy: A report of three cases and a literature review.

    PubMed

    Yamashita, Akiko; Hidaka, Nobuhiro; Yamamoto, Ryo; Nakayama, Soichiro; Sasahara, Jun; Ishii, Keisuke; Mitsuda, Nobuaki

    2015-09-01

    Fetal congenital cystic adenomatoid malformation (CCAM) can progress to nonimmune hydrops, and the mortality rate of CCAM with hydrops is reported to be nearly 100%. We describe three microcystic CCAM cases in which the fetal condition improved after maternal betamethasone therapy. The median gestational age at steroid administration was 23 5/7 weeks' gestation. The CCAM decreased in size in all cases. Our series showed a 100% hydrops resolution rate (2/2) and a 100% survival rate (3/3). Our experience suggests the efficacy of betamethasone treatment on fetuses with microcystic CCAM who have fluid collection or are at risk of developing hydrops. © 2014 Wiley Periodicals, Inc.

  12. General anesthetic octanol and related compounds activate wild-type and delF508 cystic fibrosis chloride channels

    PubMed Central

    Marcet, Brice; Becq, Frédéric; Norez, Caroline; Delmas, Patrick; Verrier, Bernard

    2004-01-01

    Cystic fibrosis transmembrane conductance regulator (CFTR) Cl− channel is defective during cystic fibrosis (CF). Activators of the CFTR Cl− channel may be useful for therapy of CF. Here, we demonstrate that a range of general anesthetics like normal-alkanols (n-alkanols) and related compounds can stimulate the Cl− channel activity of wild-type CFTR and delF508-CFTR mutant. The effects of n-alkanols like octanol on CFTR activity were measured by iodide (125I) efflux and patch-clamp techniques on three distinct cellular models: (1) CFTR-expressing Chinese hamster ovary cells, (2) human airway Calu-3 epithelial cells and (3) human airway JME/CF15 epithelial cells which express the delF508-CFTR mutant. Our data show for the first time that n-alkanols activate both wild-type CFTR and delF508-CFTR mutant. Octanol stimulated 125I efflux in a dose-dependent manner in CFTR-expressing cells (wild-type and delF508) but not in cell lines lacking CFTR. 125I efflux and Cl− currents induced by octanol were blocked by glibenclamide but insensitive to 4,4′-diisothiocyanatostilbene-2,2′-disulfonic acid, as expected for a CFTR Cl− current. CFTR activation by octanol was neither due to cell-to-cell uncoupling properties of octanol nor to an intracellular cAMP increase. CFTR activation by octanol requires phosphorylation by protein kinase-A (PKA) since it was prevented by H-89, a PKA inhibitor. n-Alkanols chain length was an important determinant for channel activation, with rank order of potencies: 1-heptanol<1-octanol<2-octanol<1-decanol. Our findings may be of valuable interest for developing novel therapeutic strategies for CF. PMID:14967738

  13. General anesthetic octanol and related compounds activate wild-type and delF508 cystic fibrosis chloride channels.

    PubMed

    Marcet, Brice; Becq, Frédéric; Norez, Caroline; Delmas, Patrick; Verrier, Bernard

    2004-03-01

    1. Cystic fibrosis transmembrane conductance regulator (CFTR) Cl(-) channel is defective during cystic fibrosis (CF). Activators of the CFTR Cl(-) channel may be useful for therapy of CF. Here, we demonstrate that a range of general anesthetics like normal-alkanols (n-alkanols) and related compounds can stimulate the Cl(-) channel activity of wild-type CFTR and delF508-CFTR mutant. 2. The effects of n-alkanols like octanol on CFTR activity were measured by iodide ((125)I) efflux and patch-clamp techniques on three distinct cellular models: (1). CFTR-expressing Chinese hamster ovary cells, (2). human airway Calu-3 epithelial cells and (3). human airway JME/CF15 epithelial cells which express the delF508-CFTR mutant. 3. Our data show for the first time that n-alkanols activate both wild-type CFTR and delF508-CFTR mutant. Octanol stimulated (125)I efflux in a dose-dependent manner in CFTR-expressing cells (wild-type and delF508) but not in cell lines lacking CFTR. (125)I efflux and Cl(-) currents induced by octanol were blocked by glibenclamide but insensitive to 4,4'-diisothiocyanatostilbene-2,2'-disulfonic acid, as expected for a CFTR Cl(-) current. 4. CFTR activation by octanol was neither due to cell-to-cell uncoupling properties of octanol nor to an intracellular cAMP increase. CFTR activation by octanol requires phosphorylation by protein kinase-A (PKA) since it was prevented by H-89, a PKA inhibitor. 5. n-Alkanols chain length was an important determinant for channel activation, with rank order of potencies: 1-heptanol<1-octanol<2-octanol<1-decanol. Our findings may be of valuable interest for developing novel therapeutic strategies for CF.

  14. Cystic Fibrosis

    MedlinePlus

    Cystic fibrosis (CF) is an inherited disease of the mucus and sweat glands. It affects mostly your lungs, pancreas, liver, intestines, sinuses, and sex organs. CF causes your mucus to be thick and sticky. ...

  15. Cystic hygroma

    MedlinePlus

    ... white blood cells. This material is called embryonic lymphatic tissue. After birth, a cystic hygroma most often ... 198. Tower RL II, Camitta BM. Abnormalities of lymphatic vessels. In: Kliegman RM, Stanton BF, St Geme ...

  16. Cystic fibrosis - resources

    MedlinePlus

    Resources - cystic fibrosis ... The following organizations are good resources for information on cystic fibrosis : Cystic Fibrosis Foundation -- www.cff.org March of Dimes -- www.marchofdimes.org/baby/cystic-fibrosis-and- ...

  17. Cystic fibrosis.

    PubMed

    Ratjen, Felix; Bell, Scott C; Rowe, Steven M; Goss, Christopher H; Quittner, Alexandra L; Bush, Andrew

    2015-05-14

    Cystic fibrosis is an autosomal recessive, monogenetic disorder caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. The gene defect was first described 25 years ago and much progress has been made since then in our understanding of how CFTR mutations cause disease and how this can be addressed therapeutically. CFTR is a transmembrane protein that transports ions across the surface of epithelial cells. CFTR dysfunction affects many organs; however, lung disease is responsible for the vast majority of morbidity and mortality in patients with cystic fibrosis. Prenatal diagnostics, newborn screening and new treatment algorithms are changing the incidence and the prevalence of the disease. Until recently, the standard of care in cystic fibrosis treatment focused on preventing and treating complications of the disease; now, novel treatment strategies directly targeting the ion channel abnormality are becoming available and it will be important to evaluate how these treatments affect disease progression and the quality of life of patients. In this Primer, we summarize the current knowledge, and provide an outlook on how cystic fibrosis clinical care and research will be affected by new knowledge and therapeutic options in the near future. For an illustrated summary of this Primer, visit: http://go.nature.com/4VrefN.

  18. Spirometer-triggered high-resolution computed tomography and pulmonary function measurements during an acute exacerbation in patients with cystic fibrosis.

    PubMed

    Robinson, T E; Leung, A N; Northway, W H; Blankenberg, F G; Bloch, D A; Oehlert, J W; Al-Dabbagh, H; Hubli, S; Moss, R B

    2001-04-01

    To evaluate a high-resolution computed tomography (HRCT) scoring system, clinical parameters, and pulmonary function measurements in patients with cystic fibrosis (CF) before and after therapy for a pulmonary exacerbation. Patients (n = 17) were evaluated by spirometer-triggered HRCT imaging, clinical parameters, and pulmonary function tests (PFTs) before and after treatment. HRCT scans were reviewed by 3 radiologists using a modified Bhalla scoring system. Bronchiectasis, bronchial wall thickening, and air trapping were identified in all subjects on initial evaluation. The initial total HRCT score correlated significantly with the Brasfield score (r = -.91, P <.001) and several PFT measures. After treatment, there were improvements in the acute change clinical score (ACCS) (P <.001), most pulmonary function measurements, and total HRCT score (P <.05). Bronchiectasis, bronchial wall thickening, and air trapping did not significantly change. Mucus plugging subcomponent HRCT score, slow vital capacity (SVC), forced expiratory volume in 1 second (FEV(1)), and forced vital capacity (FVC) (percent predicted) and reversible and total HRCT scores were most sensitive to change by effect size analysis. Improvements occurred with treatment in total and reversible HRCT scores, PFTs, and ACCS. Total and reversible HRCT scores and percent predicted SVC, FEV1, and FVC were the most sensitive to change. The greatest change was seen in the mucus plugging subcomponent HRCT score.

  19. General Semantics and the "Spirit" of the Resolution.

    ERIC Educational Resources Information Center

    Simerly, Greggory

    The Cross Examination Debate Association (CEDA) strives to eliminate the perceived abuses of intercollegiate debate: machine gun delivery, the overemphasis of evidence cards, and the use of "squirrel cases" (cases so narrow that they ignore most of the resolution or are actually off the topic). CEDA philosophically and, at least to some…

  20. 29 CFR 96.53 - Audit resolution generally.

    Code of Federal Regulations, 2012 CFR

    2012-07-01

    ... promptly evaluate findings and recommendations reported by auditors and the corrective action plan developed by the recipient to determine proper actions in response to audit findings and recommendations... the final approved audit report is received by the DOL official(s) responsible for audit resolution. ...

  1. 29 CFR 96.53 - Audit resolution generally.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... promptly evaluate findings and recommendations reported by auditors and the corrective action plan developed by the recipient to determine proper actions in response to audit findings and recommendations... the final approved audit report is received by the DOL official(s) responsible for audit resolution. ...

  2. 29 CFR 96.53 - Audit resolution generally.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... promptly evaluate findings and recommendations reported by auditors and the corrective action plan developed by the recipient to determine proper actions in response to audit findings and recommendations... the final approved audit report is received by the DOL official(s) responsible for audit resolution. ...

  3. 29 CFR 96.53 - Audit resolution generally.

    Code of Federal Regulations, 2013 CFR

    2013-07-01

    ... promptly evaluate findings and recommendations reported by auditors and the corrective action plan developed by the recipient to determine proper actions in response to audit findings and recommendations... the final approved audit report is received by the DOL official(s) responsible for audit resolution. ...

  4. 29 CFR 96.53 - Audit resolution generally.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ... promptly evaluate findings and recommendations reported by auditors and the corrective action plan developed by the recipient to determine proper actions in response to audit findings and recommendations... the final approved audit report is received by the DOL official(s) responsible for audit resolution. ...

  5. Cystic fibrosis

    MedlinePlus

    ... cannot be prevented. Screening those with a family history of the disease may detect the CF gene in many carriers. Alternative ... FJ. Cystic fibrosis. In: Goldman L, Schafer AI, eds. Goldman's Cecil Medicine . 25th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap ...

  6. What Causes Cystic Fibrosis?

    MedlinePlus

    ... page from the NHLBI on Twitter. What Causes Cystic Fibrosis? A defect in the CFTR gene causes cystic ... in the severity of the disease. How Is Cystic Fibrosis Inherited? Every person inherits two CFTR genes—one ...

  7. Generalized alopecic and cystic dermatosis in a cat: a counterpart to the hairless mouse phenotype or a unique congenital dermatosis?

    PubMed

    Neuber, Ariane E; Van Den Broek, Adri H M; Rhind, Susan M; Hill, Peter B; Thoday, Keith L

    2006-02-01

    A 2-year-old, male neutered, domestic semi-long-haired cat was presented with a 1.5-year history of progressive, initially nonpruritic alopecia and malodorous greasy exudate affecting the distal extremities, trunk and neck but sparing the head and tail. The extensive alopecia and 'seborrhoea' were associated with severe thickening of the skin and fold formation on the dorsal head and distal extremities as well as the lateral thorax and abdomen. The hair was easily epilated, numerous milia were seen on the ventral abdomen and the caudal and lateral thighs, and mild paronychia was present. Histopathological examination of skin biopsies revealed marked cystic dilation of hair follicles and sebaceous glands with follicular hypoplasia, infundibular hyperkeratosis and variable associated inflammation. Systemic glucocorticoid therapy in combination with topical washes with chlorhexidine and miconazole resulted in a marked improvement and some hair regrowth, but the cat was subsequently lost to follow-up. The dermatosis resembles a number of conditions in other species, but it is not clear whether it is a counterpart to the hairless mutant mouse or is a unique dermatosis.

  8. Anxiety and depression in adults with cystic fibrosis: a comparison between patients and the general population in Sweden and three other European countries.

    PubMed

    Backström-Eriksson, Lena; Sorjonen, Kimmo; Bergsten-Brucefors, Agneta; Hjelte, Lena; Melin, Bo

    2015-10-14

    Cystic fibrosis (CF) is the most common autosomal recessive life-shortening disease among Caucasians. Studies exploring the prevalence of anxiety and depression in adult CF patients are few, show inconsistent findings and rarely include comparisons with general populations. Prevalence and degree of anxiety and depression were investigated in adult CF patients in Sweden, Belgium, Germany and the UK, and compared to corresponding general population data. Adult non-transplanted CF patients from the three largest CF-centres (out of four) in Sweden (N = 129; Age range 18-70 years; 50 % women) completed the Hospital Anxiety and Depression Scale (HADS). Studies using HADS in adult CF populations in the UK, Germany, and Belgium were included, as well as HADS normative data from the corresponding general populations. No elevated risk for anxiety and depression was found among the CF patients. However, a Country x Group interaction effect emerged; CF patients experienced a higher degree of anxiety than the general population in Sweden, but not in the other countries, though this finding did not remain significant in a logistic regression analysis. In Sweden the effect was limited to women. A Country x Group interaction effect was also found for Depression; CF patients experienced lower degree of depression than the general population in Sweden, Germany and the UK, but not in Belgium/Netherlands. Contrary to earlier outcomes, the present results do not indicate any general elevated risk for anxiety and depression among CF patients. Anxiety was slightly higher in the Swedish CF population, compared to the general population; this finding was not seen in the other countries. Depression among CF patients was lower than or similar to that in the general populations in the studied countries.

  9. Arctic storms simulated in atmospheric general circulation models under uniform high, uniform low, and variable resolutions

    NASA Astrophysics Data System (ADS)

    Roesler, E. L.; Bosler, P. A.; Taylor, M.

    2016-12-01

    The impact of strong extratropical storms on coastal communities is large, and the extent to which storms will change with a warming Arctic is unknown. Understanding storms in reanalysis and in climate models is important for future predictions. We know that the number of detected Arctic storms in reanalysis is sensitive to grid resolution. To understand Arctic storm sensitivity to resolution in climate models, we describe simulations designed to identify and compare Arctic storms at uniform low resolution (1 degree), at uniform high resolution (1/8 degree), and at variable resolution (1 degree to 1/8 degree). High-resolution simulations resolve more fine-scale structure and extremes, such as storms, in the atmosphere than a uniform low-resolution simulation. However, the computational cost of running a globally uniform high-resolution simulation is often prohibitive. The variable resolution tool in atmospheric general circulation models permits regional high-resolution solutions at a fraction of the computational cost. The storms are identified using the open-source search algorithm, Stride Search. The uniform high-resolution simulation has over 50% more storms than the uniform low-resolution and over 25% more storms than the variable resolution simulations. Storm statistics from each of the simulations is presented and compared with reanalysis. We propose variable resolution as a cost-effective means of investigating physics/dynamics coupling in the Arctic environment. Future work will include comparisons with observed storms to investigate tuning parameters for high resolution models. Sandia National Laboratories is a multi-program laboratory managed and operated by Sandia Corporation, a wholly owned subsidiary of Lockheed Martin Corporation, for the U.S. Department of Energy's National Nuclear Security Administration under contract DE-AC04-94AL85000. SAND2016-7402 A

  10. [News in cystic fibrosis].

    PubMed

    Delaisi, B

    2013-08-01

    The improvement over the last two decades in the treatment of cystic fibrosis led to an increase in life expectancy approaching 40 years at birth. Logically, the population of adult patients has been increasing and is currently 50% of patients followed in France. These therapeutic advances have justified the establishment in 2003 of a generalized neonatal screening for cystic fibrosis. The latest data of this screening show an incidence of CF of 1/5359 live births, far below the incidence of 1/2500 which was widely accepted twenty years ago. The performance of this screening is currently based on the dosage of trypsin immuno reactive, followed in case of exceeding the threshold of a search of the 30 most common mutations, can detect around 96% of 150 to 200 CF cases every year. Therefore, the possibility of a false negative of the screening cannot be excluded and evocative symptoms of cystic fibrosis, even for children born after 2003, will lead to prescribe a sweat test. While treatments available so far goal consequences of cystic fibrosis, a new therapeutic class to correct the functional defect of the mutated protein, called CFTR modulators, is emerging. Ivacaftor, leader of this new class, belonging to the category of "CFTR potentiator" got its access on the market in September 2012 for patients carrying the G551D mutation. New other molecules, named "CFTR correctors" which can have synergistic effect with ivacaftor and concern patients carrying the most common mutation--DF 508--are under development. Copyright © 2013. Published by Elsevier Masson SAS.

  11. Cystic Fibrosis

    PubMed Central

    Asay, Lyal D.

    1965-01-01

    Cystic fibrosis, a disease thought to be transmitted as a recessive genetic trait, is found as a disease in about one in 1,000 to one in 10,000 births. It involves all of the exocrine glands with presenting symptoms dependent upon the extent of involvement of any group of glands. Many aspects of the disease can be corrected by substitution therapy. This applies particularly to the use of animal pancreas for the steatorrhea and salt for prevention of heat prostration. Unfortunately, the obstructive pulmonary disease with secondary bronchial infections can only be treated symptomatically by the use of mucus thinning agents, postural drainage, and antibiotics. Nevertheless, longevity can be increased and a great deal of hope offered to the families of these unfortunate children by careful supervision of their medical care. ImagesFigure 1.Figure 2.Figure 3.Figure 4.Figure 5.Figure 6.Figure 7.Figure 8.Figure 9.Figure 10.Figure 11. PMID:14288148

  12. Stochastically established resolution analysis helps to determine empirical tuning parameters in general interpolation schemes

    NASA Astrophysics Data System (ADS)

    Ye, Z.; Chiao, L.

    2013-12-01

    Resolution analysis has been a crucial appraisal procedure in general estimation problems to help with the correct interpretation. However, complete resolution information is usually inaccessible due to the sizeable matrix inversion involved with the construction of the resolution matrix. Furthermore, there are not explicit forward kernels embedded within formulations for popular interpolation algorithms such as the kriging and the minimum curvature gridding schemes. Stochastic simulation has recently been proposed to make resolution evaluation for sizeable inverse problems tractable. We generalize the method of getting resolution information to the popular interpolation schemes. There are usually certain empirically determined tuning parameters involved in these interpolation schemes, for example, the ideal function and radius of influence for fitting the semi-variogram in the kriging method and the relative weighting of the membrane stress term in the minimum curvature gridding scheme. We show that our proposed resolution analysis not only provide the crucial spatial resolution pattern, more importantly, it helps to determine those critical tuning parameters that have been determined empirically and arbitrarily. Keywords: resolution analysis; stochastic simulation; kriging; minimum curvature gridding

  13. Coreference resolution: A review of general methodologies and applications in the clinical domain

    PubMed Central

    Zheng, Jiaping; Chapman, Wendy W.; Crowley, Rebecca S.; Savova, Guergana K.

    2011-01-01

    Coreference resolution is the task of determining linguistic expressions that refer to the same real-world entity in natural language. Research on coreference resolution in the general English domain dates back to 1960s and 1970s. However, research on coreference resolution in the clinical free text has not seen major development. The recent US government initiatives that promote the use of electronic health records (EHRs) provide opportunities to mine patient notes as more and more health care institutions adopt EHR. Our goal was to review recent advances in general purpose coreference resolution to lay the foundation for methodologies in the clinical domain, facilitated by the availability of a shared lexical resource of gold standard coreference annotations, the Ontology Development and Information Extraction (ODIE) corpus. PMID:21856441

  14. A general CFD framework for fault-resilient simulations based on multi-resolution information fusion

    NASA Astrophysics Data System (ADS)

    Lee, Seungjoon; Kevrekidis, Ioannis G.; Karniadakis, George Em

    2017-10-01

    We develop a general CFD framework for multi-resolution simulations to target multiscale problems but also resilience in exascale simulations, where faulty processors may lead to gappy, in space-time, simulated fields. We combine approximation theory and domain decomposition together with statistical learning techniques, e.g. coKriging, to estimate boundary conditions and minimize communications by performing independent parallel runs. To demonstrate this new simulation approach, we consider two benchmark problems. First, we solve the heat equation (a) on a small number of spatial ;patches; distributed across the domain, simulated by finite differences at fine resolution and (b) on the entire domain simulated at very low resolution, thus fusing multi-resolution models to obtain the final answer. Second, we simulate the flow in a lid-driven cavity in an analogous fashion, by fusing finite difference solutions obtained with fine and low resolution assuming gappy data sets. We investigate the influence of various parameters for this framework, including the correlation kernel, the size of a buffer employed in estimating boundary conditions, the coarseness of the resolution of auxiliary data, and the communication frequency across different patches in fusing the information at different resolution levels. In addition to its robustness and resilience, the new framework can be employed to generalize previous multiscale approaches involving heterogeneous discretizations or even fundamentally different flow descriptions, e.g. in continuum-atomistic simulations.

  15. Cystic fibrosis - nutritional considerations

    MedlinePlus

    ... this page: //medlineplus.gov/ency/article/002437.htm Cystic fibrosis - nutrition To use the sharing features on this page, please enable JavaScript. Cystic fibrosis (CF) is a life-threatening disease that causes ...

  16. Multi-resolution community detection based on generalized self-loop rescaling strategy

    NASA Astrophysics Data System (ADS)

    Xiang, Ju; Tang, Yan-Ni; Gao, Yuan-Yuan; Zhang, Yan; Deng, Ke; Xu, Xiao-Ke; Hu, Ke

    2015-08-01

    Community detection is of considerable importance for analyzing the structure and function of complex networks. Many real-world networks may possess community structures at multiple scales, and recently, various multi-resolution methods were proposed to identify the community structures at different scales. In this paper, we present a type of multi-resolution methods by using the generalized self-loop rescaling strategy. The self-loop rescaling strategy provides one uniform ansatz for the design of multi-resolution community detection methods. Many quality functions for community detection can be unified in the framework of the self-loop rescaling. The resulting multi-resolution quality functions can be optimized directly using the existing modularity-optimization algorithms. Several derived multi-resolution methods are applied to the analysis of community structures in several synthetic and real-world networks. The results show that these methods can find the pre-defined substructures in synthetic networks and real splits observed in real-world networks. Finally, we give a discussion on the methods themselves and their relationship. We hope that the study in the paper can be helpful for the understanding of the multi-resolution methods and provide useful insight into designing new community detection methods.

  17. Language in Context: MEG Evidence for Modality-General and -Specific Responses to Reference Resolution

    PubMed Central

    2016-01-01

    Abstract Successful language comprehension critically depends on our ability to link linguistic expressions to the entities they refer to. Without reference resolution, newly encountered language cannot be related to previously acquired knowledge. The human experience includes many different types of referents, some visual, some auditory, some very abstract. Does the neural basis of reference resolution depend on the nature of the referents, or do our brains use a modality-general mechanism for linking meanings to referents? Here we report evidence for both. Using magnetoencephalography (MEG), we varied both the modality of referents, which consisted either of visual or auditory objects, and the point at which reference resolution was possible within sentences. Source-localized MEG responses revealed brain activity associated with reference resolution that was independent of the modality of the referents, localized to the medial parietal lobe and starting ∼415 ms after the onset of reference resolving words. A modality-specific response to reference resolution in auditory domains was also found, in the vicinity of auditory cortex. Our results suggest that referential language processing cannot be reduced to processing in classical language regions and representations of the referential domain in modality-specific neural systems. Instead, our results suggest that reference resolution engages medial parietal cortex, which supports a mechanism for referential processing regardless of the content modality. PMID:28058272

  18. Variable-resolution frameworks for the simulation of tropical cyclones in global atmospheric general circulation models

    NASA Astrophysics Data System (ADS)

    Zarzycki, Colin

    The ability of atmospheric General Circulation Models (GCMs) to resolve tropical cyclones in the climate system has traditionally been difficult. The challenges include adequately capturing storms which are small in size relative to model grids and the fact that key thermodynamic processes require a significant level of parameterization. At traditional GCM grid spacings of 50-300 km tropical cyclones are severely under-resolved, if not completely unresolved. This thesis explores a variable-resolution global model approach that allows for high spatial resolutions in areas of interest, such as low-latitude ocean basins where tropical cyclogenesis occurs. Such GCM designs with multi-resolution meshes serve to bridge the gap between globally-uniform grids and limited area models and have the potential to become a future tool for regional climate assessments. A statically-nested, variable-resolution option has recently been introduced into the Department of Energy/National Center for Atmospheric Research (DoE/NCAR) Community Atmosphere Model's (CAM) Spectral Element (SE) dynamical core. Using an idealized tropical cyclone test, variable-resolution meshes are shown to significantly lessen computational requirements in regional GCM studies. Furthermore, the tropical cyclone simulations are free of spurious numerical errors at the resolution interfaces. Utilizing aquaplanet simulations as an intermediate test between idealized simulations and fully-coupled climate model runs, climate statistics within refined patches are shown to be well-matched to globally-uniform simulations of the same grid spacing. Facets of the CAM version 4 (CAM4) subgrid physical parameterizations are likely too scale sensitive for variable-resolution applications, but the newer CAM5 package is vastly improved in performance at multiple grid spacings. Multi-decadal simulations following 'Atmospheric Model Intercomparison Project' protocols have been conducted with variable-resolution grids. Climate

  19. Evaluation of Cloud Parameterizations in a High Resolution Atmospheric General Circulation Model Using ARM Data

    SciTech Connect

    Govindasamy, B; Duffy, P

    2002-04-12

    Typical state of the art atmospheric general circulation models used in climate change studies have horizontal resolution of approximately 300 km. As computing power increases, many climate modeling groups are working toward enhancing the resolution of global models. An important issue that arises when resolution of a model is changed is whether cloud and convective parameterizations, which were developed for use at coarser resolutions, will need to be reformulated or re-tuned. We propose to investigate this issue and specifically cloud statistics using ARM data. The data streams produced by highly instrumented sections of Cloud and Radiation Testbeds (CART) of ARM program will provide a significant aid in the evaluation of cloud and convection parameterization in high-resolution models. Recently, we have performed multiyear global-climate simulations at T170 and T239 resolutions, corresponding to grid cell sizes of 0.7{sup 0} and 0.5{sup 0} respectively, using the NCAR Community Climate Model. We have also a performed climate change simulation at T170. On the scales of a T42 grid cell (300 km) and larger, nearly all quantities we examined in T170 simulation agree better with observations in terms of spatial patterns than do results in a comparable simulation at T42. Increasing the resolution to T239 brings significant further improvement. At T239, the high-resolution model grid cells approach the dimensions of the highly instrumented sections of ARM Cloud and Radiation Testbed (CART) sites. We propose to form a cloud climatology using ARM data for its CART sites and evaluate cloud statistics of the NCAR Community Atmosphere Model (CAM) at higher resolutions over those sites using this ARM cloud climatology. We will then modify the physical parameterizations of CAM for better agreement with ARM data. We will work closely with NCAR in modifying the parameters in cloud and convection parameterizations for the high-resolution model. Our proposal to evaluate the cloud

  20. iPAINT: a general approach tailored to image the topology of interfaces with nanometer resolution

    NASA Astrophysics Data System (ADS)

    Aloi, A.; Vilanova, N.; Albertazzi, L.; Voets, I. K.

    2016-04-01

    Understanding interfacial phenomena in soft materials such as wetting, colloidal stability, coalescence, and friction warrants non-invasive imaging with nanometer resolution. Super-resolution microscopy has emerged as an attractive method to visualize nanostructures labeled covalently with fluorescent tags, but this is not amenable to all interfaces. Inspired by PAINT we developed a simple and general strategy to overcome this limitation, which we coin `iPAINT: interface Point Accumulation for Imaging in Nanoscale Topography'. It enables three-dimensional, sub-diffraction imaging of interfaces irrespective of their nature via reversible adsorption of polymer chains end-functionalized with photo-activatable moieties. We visualized model dispersions, emulsions, and foams with ~20 nm and ~3° accuracy demonstrating the general applicability of iPAINT to study solid/liquid, liquid/liquid and liquid/air interfaces. iPAINT thus broadens the scope of super-resolution microscopy paving the way for non-invasive, high-resolution imaging of complex soft materials.Understanding interfacial phenomena in soft materials such as wetting, colloidal stability, coalescence, and friction warrants non-invasive imaging with nanometer resolution. Super-resolution microscopy has emerged as an attractive method to visualize nanostructures labeled covalently with fluorescent tags, but this is not amenable to all interfaces. Inspired by PAINT we developed a simple and general strategy to overcome this limitation, which we coin `iPAINT: interface Point Accumulation for Imaging in Nanoscale Topography'. It enables three-dimensional, sub-diffraction imaging of interfaces irrespective of their nature via reversible adsorption of polymer chains end-functionalized with photo-activatable moieties. We visualized model dispersions, emulsions, and foams with ~20 nm and ~3° accuracy demonstrating the general applicability of iPAINT to study solid/liquid, liquid/liquid and liquid/air interfaces. i

  1. Bilateral Cystic Adrenal Neuroblastoma with Cystic Liver metastasis

    PubMed Central

    Aslan, Mine; Kalyoncu, Ayse Ucar; Habibi, Hatice Arioz; Ozdemir, Gul Nihal; Koc, Basak; Adaletli, Ibrahim

    2017-01-01

    Bilateral congenital cystic adrenal neuroblastoma (NB) with cystic liver metastasis is a very rare condition and only few cases have been reported in the literature. Herein we report a case of a congenital bilateral cystic adrenal NB with cystic liver metastasis and briefly discuss characteristic imaging features of cystic NB. PMID:28163998

  2. Voice Disorder in Cystic Fibrosis Patients

    PubMed Central

    Lourenço, Bruna Mendes; Costa, Kauê Machado; da Silva Filho, Manoel

    2014-01-01

    Cystic fibrosis is a common autosomal recessive disorder with drastic respiratory symptoms, including shortness of breath and chronic cough. While most of cystic fibrosis treatment is dedicated to mitigating the effects of respiratory dysfunction, the potential effects of this disease on vocal parameters have not been systematically studied. We hypothesized that cystic fibrosis patients, given their characteristic respiratory disorders, would also present dysphonic symptoms. Given that voice disorders can severely impair quality of life, the identification of a potential cystic fibrosis-related dysphonia could be of great value for the clinical evaluation and treatment of this disease. We tested our hypothesis by measuring vocal parameters, using both objective physical measures and the GRBAS subjective evaluation method, in male and female cystic fibrosis patients undergoing conventional treatment and compared them to age and sex matched controls. We found that cystic fibrosis patients had a significantly lower vocal intensity and harmonic to noise ratio, as well as increased levels of jitter and shimmer. In addition, cystic fibrosis patients also showed higher scores of roughness, breathiness and asthenia, as well as a significantly altered general grade of dysphonia. When we segregated the results according to sex, we observed that, as a group, only female cystic fibrosis patients had significantly lower values of harmonic to noise ratio and an abnormal general grade of dysphonia in relation to matched controls, suggesting that cystic fibrosis exerts a more pronounced effect on vocal parameters of women in relation to men. Overall, the dysphonic characteristics of CF patients can be explained by dysfunctions in vocal fold movement and partial upper airway obstruction, potentially caused by the accumulation of mucus and chronic cough characteristic of CF symptomatology. Our results show that CF patients exhibit significant dysphonia and suggest they may

  3. Voice disorder in cystic fibrosis patients.

    PubMed

    Lourenço, Bruna Mendes; Costa, Kauê Machado; da Silva Filho, Manoel

    2014-01-01

    Cystic fibrosis is a common autosomal recessive disorder with drastic respiratory symptoms, including shortness of breath and chronic cough. While most of cystic fibrosis treatment is dedicated to mitigating the effects of respiratory dysfunction, the potential effects of this disease on vocal parameters have not been systematically studied. We hypothesized that cystic fibrosis patients, given their characteristic respiratory disorders, would also present dysphonic symptoms. Given that voice disorders can severely impair quality of life, the identification of a potential cystic fibrosis-related dysphonia could be of great value for the clinical evaluation and treatment of this disease. We tested our hypothesis by measuring vocal parameters, using both objective physical measures and the GRBAS subjective evaluation method, in male and female cystic fibrosis patients undergoing conventional treatment and compared them to age and sex matched controls. We found that cystic fibrosis patients had a significantly lower vocal intensity and harmonic to noise ratio, as well as increased levels of jitter and shimmer. In addition, cystic fibrosis patients also showed higher scores of roughness, breathiness and asthenia, as well as a significantly altered general grade of dysphonia. When we segregated the results according to sex, we observed that, as a group, only female cystic fibrosis patients had significantly lower values of harmonic to noise ratio and an abnormal general grade of dysphonia in relation to matched controls, suggesting that cystic fibrosis exerts a more pronounced effect on vocal parameters of women in relation to men. Overall, the dysphonic characteristics of CF patients can be explained by dysfunctions in vocal fold movement and partial upper airway obstruction, potentially caused by the accumulation of mucus and chronic cough characteristic of CF symptomatology. Our results show that CF patients exhibit significant dysphonia and suggest they may

  4. Hurricane Forecasting with the High-resolution NASA Finite-volume General Circulation Model

    NASA Technical Reports Server (NTRS)

    Atlas, R.; Reale, O.; Shen, B.-W.; Lin, S.-J.; Chern, J.-D.; Putman, W.; Lee, T.; Yeh, K.-S.; Bosilovich, M.; Radakovich, J.

    2004-01-01

    A high-resolution finite-volume General Circulation Model (fvGCM), resulting from a development effort of more than ten years, is now being run operationally at the NASA Goddard Space Flight Center and Ames Research Center. The model is based on a finite-volume dynamical core with terrain-following Lagrangian control-volume discretization and performs efficiently on massive parallel architectures. The computational efficiency allows simulations at a resolution of a quarter of a degree, which is double the resolution currently adopted by most global models in operational weather centers. Such fine global resolution brings us closer to overcoming a fundamental barrier in global atmospheric modeling for both weather and climate, because tropical cyclones and even tropical convective clusters can be more realistically represented. In this work, preliminary results of the fvGCM are shown. Fifteen simulations of four Atlantic tropical cyclones in 2002 and 2004 are chosen because of strong and varied difficulties presented to numerical weather forecasting. It is shown that the fvGCM, run at the resolution of a quarter of a degree, can produce very good forecasts of these tropical systems, adequately resolving problems like erratic track, abrupt recurvature, intense extratropical transition, multiple landfall and reintensification, and interaction among vortices.

  5. How Is Cystic Fibrosis Treated?

    MedlinePlus

    ... page from the NHLBI on Twitter. How Is Cystic Fibrosis Treated? Cystic fibrosis (CF) has no cure. However, ... help oral pancreatic enzymes work better. Treatments for Cystic Fibrosis Complications A common complication of CF is diabetes . ...

  6. Cystic Fibrosis: Diet and Nutrition

    MedlinePlus

    ... Right Sport for You Healthy School Lunch Planner Cystic Fibrosis: Diet and Nutrition KidsHealth > For Teens > Cystic Fibrosis: ... Food Enzyme Supplements Beating the Frustration What Is Cystic Fibrosis? At lunch, Lindsay often gets bored with having ...

  7. A resolution insensitive parameterization of cloud overlap for general circulation models

    NASA Astrophysics Data System (ADS)

    Bergman, J. W.; Rasch, P. J.

    2001-05-01

    Radiative fluxes produced in an atmospheric general circulation model (GCM) can be very sensitive to how the overlap of clouds from different vertical levels is specified. This becomes particularly problematic as the vertical resolution of GCMs increases and idealized overlap assumption, such as random overlap, yield pathological results. We develop a parameterization of cloud overlap that is relatively insensitive to changes of resolution. The relationship between overlap and the correlation of a 'cloudiness' function is recognized and exploited to prescribe cloud overlap in terms of a decorrelation length scale Lc. Cloud fraction at each level together with Lc are used here to prescribe the entire sub-grid (i.e., on unresolved horizontal scales) cloud distribution for the purposes of radiative transfer. This distribution and the radiative fluxes calculated from it are insensitive to changes of vertical resolution if Lc is at least twice the thickness of a model level, that is, if the model resolution is sufficient to resolve the cloud distribution. The overlap parameterization is implemented into two radiative transfer formulations: the independent column approximation, in which a separate radiative transfer calculation is performed for each permutation of clear and cloudy conditions within individual levels, and a modified plane-parallel calculation, which closely approximates ICA fluxes at a fraction of the computational cost. The sensitivity of radiative fluxes to the specified decorrelation length scale is discussed in the context of idealized cloud distributions and those from a GCM.

  8. RAISHIN: A High-Resolution Three-Dimensional General Relativistic Magnetohydrodynamics Code

    NASA Technical Reports Server (NTRS)

    Mizuno, Yosuke; Nishikawa, Ken-Ichi; Koide, Shinji; Hardee, Philip; Fishman, Gerald J.

    2006-01-01

    We have developed a new three-dimensional general relativistic magnetohydrodynamic (GRMHD) code, RAISHIN, using a conservative, high resolution shock-capturing scheme. The numerical fluxes are calculated using the Harten, Lax, & van Leer (HLL) approximate Riemann solver scheme. The flux-interpolated, constrained transport scheme is used to maintain a divergence-free magnetic field. In order to examine the numerical accuracy and the numerical efficiency, the code uses four different reconstruction methods: piecewise linear methods with Minmod and MC slope-limiter function, convex essentially non-oscillatory (CENO) method, and piecewise parabolic method (PPM) using multistep TVD Runge-Kutta time advance methods with second and third-order time accuracy. We describe code performance on an extensive set of test problems in both special and general relativity. Our new GRMHD code has proven to be accurate in second order and has successfully passed with all tests performed, including highly relativistic and magnetized cases in both special and general relativity.

  9. Cystic Lesions of the Mediastinum.

    PubMed

    Vargas, Daniel; Suby-Long, Thomas; Restrepo, Carlos S

    2016-06-01

    Cystic lesions are commonly seen in the mediastinum, and they may arise from virtually any organ. The vast majority of these lesions are benign and result in no symptoms. When large, cysts may produce symptoms related to compression of adjacent structures. The most common mediastinal cysts are pericardial and foregut duplication cysts. Both computed tomography and magnetic resonance are routinely used to evaluate these lesions. Although computed tomography offers superior spatial resolution, magnetic resonance is useful in differentiating cysts that contain proteinaceous material from solid lesions. Occasionally, cysts arise from solid lesions, such as thymoma or teratoma. Although cysts are alike in appearance, location helps narrowing the differential diagnoses.

  10. A general resolution of intractable problems in polynomial time through DNA Computing.

    PubMed

    Sanches, C A A; Soma, N Y

    2016-12-01

    Based on a set of known biological operations, a general resolution of intractable problems in polynomial time through DNA Computing is presented. This scheme has been applied to solve two NP-Hard problems (Minimization of Open Stacks Problem and Matrix Bandwidth Minimization Problem) and three co-NP-Complete problems (associated with Hamiltonian Path, Traveling Salesman and Hamiltonian Circuit), which have not been solved with this model. Conclusions and open questions concerning the computational capacity of this model are presented, and research topics are suggested.

  11. Living with Cystic Fibrosis

    MedlinePlus

    ... page from the NHLBI on Twitter. Living With Cystic Fibrosis If you or your child has cystic fibrosis (CF), you should learn as much as you can ... with your doctors to learn how to manage CF. Ongoing Care Having ongoing medical care by a ...

  12. Chromomycosis: Subcutaneous cystic type.

    PubMed

    Agrawal, S N; Bhise, P R; Sony, P R

    2000-01-01

    A 38-year -old male farmer presented with a solitary, asymptomatic, cystic lesion on the palm since last four years. He underwent excision of this cyst two times during this period but the lesion recurred near the same site. The histopathology and the microbiological examination led to the diagnosis of the rare subcutaneous cystic type of chromomycosis.

  13. Insight into Resolution Enhancement in Generalized Two-Dimensional Correlation Spectroscopy

    PubMed Central

    Ma, Lu; Sikirzhytski, Vitali; Hong, Zhenmin; Lednev, Igor K.; Asher, Sanford A.

    2014-01-01

    Generalized two-dimensional correlation spectroscopy (2D COS) can be used to enhance spectral resolution in order to help differentiate highly overlapped spectral bands. Despite the numerous extensive 2D COS investigations, the origin of the 2D spectral resolution enhancement mechanism(s) are not completely understood. In the work here we studied the 2D COS of simulated spectra in order to develop new insights into the dependence of the 2D COS spectral features on the overlapping band separations, their intensities and bandwidths, and their band intensity change rates. We find that the features in the 2D COS maps that derive from overlapping bands are determined by the spectral normalized half-intensities and the total intensity changes of the correlated bands. We identify the conditions required to resolve overlapping bands. In particular, 2D COS peak resolution requires that the normalized half-intensities of a correlating band have amplitudes between the maxima and minima of the normalized half-intensities of the overlapping bands. PMID:23452492

  14. Generalized recovery algorithm for 3D super-resolution microscopy using rotating point spread functions

    PubMed Central

    Shuang, Bo; Wang, Wenxiao; Shen, Hao; Tauzin, Lawrence J.; Flatebo, Charlotte; Chen, Jianbo; Moringo, Nicholas A.; Bishop, Logan D. C.; Kelly, Kevin F.; Landes, Christy F.

    2016-01-01

    Super-resolution microscopy with phase masks is a promising technique for 3D imaging and tracking. Due to the complexity of the resultant point spread functions, generalized recovery algorithms are still missing. We introduce a 3D super-resolution recovery algorithm that works for a variety of phase masks generating 3D point spread functions. A fast deconvolution process generates initial guesses, which are further refined by least squares fitting. Overfitting is suppressed using a machine learning determined threshold. Preliminary results on experimental data show that our algorithm can be used to super-localize 3D adsorption events within a porous polymer film and is useful for evaluating potential phase masks. Finally, we demonstrate that parallel computation on graphics processing units can reduce the processing time required for 3D recovery. Simulations reveal that, through desktop parallelization, the ultimate limit of real-time processing is possible. Our program is the first open source recovery program for generalized 3D recovery using rotating point spread functions. PMID:27488312

  15. Generalized recovery algorithm for 3D super-resolution microscopy using rotating point spread functions

    NASA Astrophysics Data System (ADS)

    Shuang, Bo; Wang, Wenxiao; Shen, Hao; Tauzin, Lawrence J.; Flatebo, Charlotte; Chen, Jianbo; Moringo, Nicholas A.; Bishop, Logan D. C.; Kelly, Kevin F.; Landes, Christy F.

    2016-08-01

    Super-resolution microscopy with phase masks is a promising technique for 3D imaging and tracking. Due to the complexity of the resultant point spread functions, generalized recovery algorithms are still missing. We introduce a 3D super-resolution recovery algorithm that works for a variety of phase masks generating 3D point spread functions. A fast deconvolution process generates initial guesses, which are further refined by least squares fitting. Overfitting is suppressed using a machine learning determined threshold. Preliminary results on experimental data show that our algorithm can be used to super-localize 3D adsorption events within a porous polymer film and is useful for evaluating potential phase masks. Finally, we demonstrate that parallel computation on graphics processing units can reduce the processing time required for 3D recovery. Simulations reveal that, through desktop parallelization, the ultimate limit of real-time processing is possible. Our program is the first open source recovery program for generalized 3D recovery using rotating point spread functions.

  16. Renormalization of total sets of states into generalized bases with a resolution of the identity

    NASA Astrophysics Data System (ADS)

    Vourdas, A.

    2017-08-01

    A total set of states for which we have no resolution of the identity (a ‘pre-basis’), is considered in a finite dimensional Hilbert space. A dressing formalism renormalizes them into density matrices which resolve the identity, and makes them a ‘generalized basis’, which is practically useful. The dresssing mechanism is inspired by Shapley’s methodology in cooperative game theory, and it uses Möbius transforms. There is non-independence and redundancy in these generalized bases, which is quantified with a Shannon type of entropy. Due to this redundancy, calculations based on generalized bases are sensitive to physical changes and robust in the presence of noise. For example, the representation of an arbitrary vector in such generalized bases, is robust when noise is inserted in the coefficients. Also in a physical system with a ground state which changes abruptly at some value of the coupling constant, the proposed methodology detects such changes, even when noise is added to the parameters in the Hamiltonian of the system.

  17. Cystic Lung Diseases: Algorithmic Approach.

    PubMed

    Raoof, Suhail; Bondalapati, Praveen; Vydyula, Ravikanth; Ryu, Jay H; Gupta, Nishant; Raoof, Sabiha; Galvin, Jeff; Rosen, Mark J; Lynch, David; Travis, William; Mehta, Sanjeev; Lazzaro, Richard; Naidich, David

    2016-10-01

    Cysts are commonly seen on CT scans of the lungs, and diagnosis can be challenging. Clinical and radiographic features combined with a multidisciplinary approach may help differentiate among various disease entities, allowing correct diagnosis. It is important to distinguish cysts from cavities because they each have distinct etiologies and associated clinical disorders. Conditions such as emphysema, and cystic bronchiectasis may also mimic cystic disease. A simplified classification of cysts is proposed. Cysts can occur in greater profusion in the subpleural areas, when they typically represent paraseptal emphysema, bullae, or honeycombing. Cysts that are present in the lung parenchyma but away from subpleural areas may be present without any other abnormalities on high-resolution CT scans. These are further categorized into solitary or multifocal/diffuse cysts. Solitary cysts may be incidentally discovered and may be an age related phenomenon or may be a remnant of prior trauma or infection. Multifocal/diffuse cysts can occur with lymphoid interstitial pneumonia, Birt-Hogg-Dubé syndrome, tracheobronchial papillomatosis, or primary and metastatic cancers. Multifocal/diffuse cysts may be associated with nodules (lymphoid interstitial pneumonia, light-chain deposition disease, amyloidosis, and Langerhans cell histiocytosis) or with ground-glass opacities (Pneumocystis jirovecii pneumonia and desquamative interstitial pneumonia). Using the results of the high-resolution CT scans as a starting point, and incorporating the patient's clinical history, physical examination, and laboratory findings, is likely to narrow the differential diagnosis of cystic lesions considerably. Copyright © 2016 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.

  18. The use of high resolution computerized tomography (HRCT) of the chest in evaluating the effect of tobramycin solution for inhalation in cystic fibrosis lung disease.

    PubMed

    Nasr, Samya Z; Sakmar, Ermelinda; Christodoulou, Emmanuel; Eckhardt, Boris P; Streetman, Daniel S; Strouse, Peter J

    2010-05-01

    To compare the usefulness of HRCT of the chest versus spirometric measures (PFTs) in evaluating the effect of tobramycin solution for inhalation (TSI) in cystic fibrosis (CF). Thirty-two CF patients with mostly mild lung disease age > or = 6 years, were enrolled in a double-blind, placebo-controlled pilot study. Patients were chronically colonized with Pseudomonas aeruginosa for at least 6 months prior to and at enrollment. If patients were on TSI, they were taken off for at least 3 months prior to enrollment. Duration was 6 months; 31 subjects completed the study. HRCT and PFTs were evaluated at baseline, after 28 days of treatment and at the end of the study. Study medication was administered as 5 ml nebulized treatment twice a day for 28 days followed by 28 days off (one cycle). Study consisted of three cycles. Two radiologists scored all films using a validated system. A total HRCT score consists of the sum of subscores: linear opacities, hyperinflation, nodular opacities, peribronchial thickening, mucous plugging, and bronchiectasis; each subscore could range from 0 to 80, with potential total scores varying from 0 to 480. The percent of the maximum possible HRCT score was then calculated and used for all comparisons. Using two tailed paired t-test, the percent maximum HRCT score decreased by 1.4 +/- 2.6% (mean +/- SD) (P = 0.049) and 0.3 +/- 2.8% (P = 0.63) for the TSI group and decreased by 0.1 +/- 1.5% (P = 0.74) and increased by 0.6 +/- 1.8% (P = 0.23) for the placebo group between visits 1 and 2, and visits 1 and 3, respectively. The data were then analyzed using a mixed model utilizing changes in scores over the durations of the study for each group. The change of HRCT score for the TSI group was -0.24/day (P = 0.02) and -0.03/day (P = 0.22), and for the control group the change was -0.01 (P = 0.93) and 0.02 (P = 0.29) between visits 1 and 2, and visits 1 and 3 respectively. FEF(25-75)% and FEV(1)% changes were not statistically significant using both

  19. Generalized multiple kernel framework for multiclass geospatial objects detection in high-resolution remote sensing images

    NASA Astrophysics Data System (ADS)

    Li, Xiangjuan; Sun, Xian; Sun, Hao; Li, Yu; Wang, Hongqi

    2012-01-01

    Multiclass geospatial objects detection within complex environments is a challenging problem in remote-sensing areas. In this paper we propose a novel, generalized kernel-based learning framework for the purpose of enhanced object detection. There are two novel areas. (1) Multisource information, including shape, feature points, and appearance, was extracted to give a comprehensive representation of the objects. We improved a shape descriptor and introduced a two-level spatial pyramid to represent appearance, both global and local. Therefore, basis kernels were formed, one for each feature. (2) In order to illustrate the effect of each kind of feature on each pyramid level, a generalized and weighted combination method was first used to combine all of the levels and then the features. The weights and the classifier model are based on the support vector machine framework for obtaining balance between all basis kernels. This classifier was transformed into a powerful detector by using a sliding window. The reported results are for the detection on high-resolution remote-sensing images. This study demonstrates that the proposed generalized and weighted combination of kernels can yield better performance compared with traditional single-kernel classifier and other combination methods.

  20. Social cognitive conflict resolution: contributions of domain-general and domain-specific neural systems.

    PubMed

    Zaki, Jamil; Hennigan, Kelly; Weber, Jochen; Ochsner, Kevin N

    2010-06-23

    Cognitive control mechanisms allow individuals to behave adaptively in the face of complex and sometimes conflicting information. Although the neural bases of these control mechanisms have been examined in many contexts, almost no attention has been paid to their role in resolving conflicts between competing social cues, which is surprising given that cognitive conflicts are part of many social interactions. Evidence about the neural processing of social information suggests that two systems--the mirror neuron system (MNS) and mental state attribution system (MSAS)--are specialized for processing nonverbal and contextual social cues, respectively. This could support a model of social cognitive conflict resolution in which competition between social cues would recruit domain-general cognitive control mechanisms, which in turn would bias processing toward the MNS or MSAS. Such biasing could also alter social behaviors, such as inferences made about the internal states of others. We tested this model by scanning participants using functional magnetic resonance imaging while they drew inferences about the social targets' emotional states based on congruent or incongruent nonverbal and contextual social cues. Conflicts between social cues recruited the anterior cingulate and lateral prefrontal cortex, brain areas associated with domain-general control processes. This activation was accompanied by biasing of neural activity toward areas in the MNS or MSAS, which tracked, respectively, with perceivers' behavioral reliance on nonverbal or contextual cues when drawing inferences about targets' emotions. Together, these data provide evidence about both domain-general and domain-specific mechanisms involved in resolving social cognitive conflicts.

  1. A High Resolution Coupled General Circulation Model for Subseasonal Forecast: Impact of Horizontal Resolution on Simulation and Retrospective Forecast of Intraseasonal Oscillations

    NASA Astrophysics Data System (ADS)

    Vintzileos, A.; Saha, S.; Pan, H.; Johansson, A.; Thiaw, C.

    2005-05-01

    General circulation models are notorious for their misrepresentation of Tropical Intraseasonal Oscillations. Hitherto, some improvements due to (i) increased vertical resolution, (ii) the type of convective parameterizations and (iii) coupling to ocean have been reported. Here we investigate the impact of increasing horizontal resolution in a high vertical resolution atmospheric component of a coupled ocean - atmosphere model. The high vertical resolution assures consistency with the horizontal discretization in both coarse and dense configurations. A series of retrospective forecast of Tropical Intraseasonal Oscillations from 1997 to 2004 performed with the GFS-T126L64/MOM3 is compared to the results of the operational version of the NCEP coupled seasonal forecasting dynamical system CFS i.e., GFS-T62L64/MOM3. In order to account for the effects of model drift and initialization shocks to the TIO, we further extend this comparison to long coupled experiments where the initial adjustment period is removed.

  2. Early bronchiectasis in cystic fibrosis detected by surveillance CT.

    PubMed

    Pillarisetti, Naveen; Linnane, Barry; Ranganathan, Sarath

    2010-08-01

    There is emerging evidence that cystic fibrosis lung disease begins early in infancy. Newborn screening allows early detection and surveillance of pulmonary disease and the possibility of early intervention in this life-shortening condition. We report two children with cystic fibrosis who underwent a comprehensive assessment from diagnosis that included measurement of lung function, limited-slice high-resolution CT and BAL performed annually. Early aggressive surveillance enabled significant lung disease and bronchiectasis to be detected during the first few years of life and led to a change in management, highlighting a clinical role for CT scanning during the preschool years in children with cystic fibrosis.

  3. Learning about Cystic Fibrosis

    MedlinePlus

    ... Cystic Fibrosis: Your Genes, Your Health [yourgenesyourhealth.org] Multimedia educational site including screening and treatment information. Hosted by the Dolan DNA Learning Center at Cold Spring Harbor Laboratory. What is ...

  4. Epidural Cystic Spinal Meningioma

    PubMed Central

    Zhang, Ji; Chen, Zheng-he; Wang, Zi-feng; Sun, Peng; Jin, Jie-tian; Zhang, Xiang-heng; Zhao, Yi-ying; Wang, Jian; Mou, Yong-gao; Chen, Zhong-ping

    2016-01-01

    Abstract Cystic spinal meningioma (CSM) is an uncommon meningioma variant. Extradural CSMs are particularly rare and difficult to distinguish from other intraaxial tumors. This study presents a case of a 36-year-old woman with intraspinal extradual CSM at the thoracolumbar spine. She experienced persistent weakness, progressive numbness, and sensory disturbance in the right lower limb. Magnetic resonance imaging (MRI) of the patient revealed an irregular cystic mass at the thoracic 11 to lumbar 3 levels dorsally. This case was misdiagnosed as other neoplasms prior to surgery because of the atypical radiographic features and location of the tumor. Extradural CSMs should be considered in the differential diagnosis of intraspinal extradural cystic neoplasms. Complete removal of cystic wall provides an optimal outcome, rendering the lesion curable. PMID:26986119

  5. Cystic Adenomatoid Odontogenic Tumor

    PubMed Central

    Grover, Sonal; Rahim, Ahmed Mujib Bangalore; Parakkat, Nithin Kavassery; Kapoor, Shekhar; Mittal, Kumud; Sharma, Bhushan; Shivappa, Anil Bangalore

    2015-01-01

    Adenomatoid Odontogenic Tumor (AOT) is a well-established benign epithelial lesion of odontogenic origin. Rightfully called “the master of disguise,” this lesion has been known for its varied clinical and histoarchitectural patterns. Not only does AOT predominantly present radiologically as a unilocular cystic lesion enclosing the unerupted tooth (which is commonly mistaken as a dentigerous cyst) but the lesion also presents rarely with a cystic component histopathologically. We present one such unusual case of cystic AOT associated with an impacted canine, mimicking a dentigerous cyst. The present case aims to highlight the difference between cystic AOT and dentigerous cyst radiographically. The exact histogenesis of AOT and its variants still remains obscure. An attempt has been made to hypothesize the new school of thought regarding the origin of AOT. PMID:26579317

  6. Diabetes in cystic fibrosis.

    PubMed

    Bridges, Nicola

    2013-05-01

    Cystic fibrosis related diabetes (CFRD) is a common complication of cystic fibrosis, caused by a fall in insulin secretion with age in individuals with pancreatic insufficiency. CFRD is associated with worse clinical status and increased mortality. Treatment of CFRD with insulin results in sustained improvements in lung function and nutrition. While clinical experience with insulin treatment in CF has increased, the selection of who to treat and glycaemic targets remain unclear. Copyright © 2013. Published by Elsevier Ltd.

  7. A simple method for determining the spatial resolution of a general inverse problem

    NASA Astrophysics Data System (ADS)

    An, Meijian

    2012-09-01

    The resolution matrix of an inverse problem defines a linear relationship in which each solution parameter is derived from the weighted averages of nearby true-model parameters, and the resolution matrix elements are the weights. Resolution matrices are not only widely used to measure the solution obtainability or the inversion perfectness from the data based on the degree to which the matrix approximates the identity matrix, but also to extract spatial-resolution or resolution-length information. Resolution matrices presented in previous spatial-resolution analysis studies can be divided into three classes: direct resolution matrix, regularized/stabilized resolution matrix and hybrid resolution matrix. The direct resolution matrix can yield resolution-length information only for ill-posed inverse problems. The regularized resolution matrix cannot give any spatial-resolution information. The hybrid resolution matrix can provide resolution-length information; however, this depends on the regularization contribution to the inversion. The computation of the matrices needs matrix operation, however, this is often a difficult problem for very large inverse problems. Here, a new class of resolution matrices, generated using a Gaussian approximation (called the statistical resolution matrices), is proposed whereby the direct determination of the matrix is accomplished via a simple one-parameter non-linear inversion performed based on limited pairs of random synthetic models and their inverse solutions. Tests showed that a statistical resolution matrix could not only measure the resolution obtainable from the data, but also provided reasonable spatial/temporal resolution or resolution-length information. The estimates were restricted to forward/inversion processes and were independent of the degree of inverse skill used in the solution inversion; therefore, the original inversion codes did not need to be modified. The absence of a requirement for matrix operations during

  8. Cystic Fibrosis (CF) Respiratory Screen: Sputum

    MedlinePlus

    ... Cystic Fibrosis (CF) Chloride Sweat Test Lungs and Respiratory System Cystic Fibrosis: Diet and Nutrition Cystic Fibrosis Cystic Fibrosis: Diet and Nutrition Lungs and Respiratory System Contact Us Print Resources Send to a friend ...

  9. Cystic Fibrosis (CF) Respiratory Screen: Sputum

    MedlinePlus

    ... Cystic Fibrosis (CF) Chloride Sweat Test Lungs and Respiratory System Cystic Fibrosis: Diet and Nutrition Cystic Fibrosis Cystic Fibrosis: Diet and Nutrition Lungs and Respiratory System Contact Us Print Resources Send to a Friend ...

  10. What's it Like to Have Cystic Fibrosis?

    MedlinePlus

    ... CPR: A Real Lifesaver Kids Talk About: Coaches Cystic Fibrosis KidsHealth > For Kids > Cystic Fibrosis Print A A ... strength to deal with cystic fibrosis. What Is CF? Cystic fibrosis (CF) is a disease that causes ...

  11. Generalized high-spectral-resolution lidar technique with a multimode laser for aerosol remote sensing.

    PubMed

    Cheng, Zhongtao; Liu, Dong; Zhang, Yupeng; Liu, Chong; Bai, Jian; Wang, Dan; Wang, Nanchao; Zhou, Yudi; Luo, Jing; Yang, Yongying; Shen, Yibing; Su, Lin; Yang, Liming

    2017-01-23

    High-spectral-resolution lidar (HSRL) is a powerful tool for atmospheric aerosol remote sensing. The current HSRL technique often requires a single longitudinal mode laser as the transmitter to accomplish the spectral discrimination of the aerosol and molecular scattering conveniently. However, single-mode laser is cumbersome and has very strict requirements for ambient stability, making the HSRL instrument not so robust in many cases. In this paper, a new HSRL concept, called generalized HSRL technique with a multimode laser (MML-gHSRL), is proposed, which can work using a multimode laser. The MML-gHSRL takes advantage of the period characteristic of the spectral function of the interferometric spectral discrimination filter (ISDF) thoroughly. By matching the free spectral range of the ISDF with the mode interval of the multimode laser, fine spectral discrimination for the lidar return from each longitudinal mode can be realized. Two common ISDFs, i.e., the Fabry-Perot interferometer (FPI) and field-widened Michelson interferometer (FWMI), are introduced to develop the MML-gHSRL, and their performance is quantitatively analyzed and compared. The MML-gHSRL is a natural but significant generalization for the current HSRL technique based on the IDSF. It is potential that this technique would be a good entrance to future HSRL developments, especially in airborne and satellite-borne aerosol remote sensing applications.

  12. GENFIRE: A generalized Fourier iterative reconstruction algorithm for high-resolution 3D imaging

    DOE PAGES

    Pryor, Alan; Yang, Yongsoo; Rana, Arjun; ...

    2017-09-05

    Tomography has made a radical impact on diverse fields ranging from the study of 3D atomic arrangements in matter to the study of human health in medicine. Despite its very diverse applications, the core of tomography remains the same, that is, a mathematical method must be implemented to reconstruct the 3D structure of an object from a number of 2D projections. Here, we present the mathematical implementation of a tomographic algorithm, termed GENeralized Fourier Iterative REconstruction (GENFIRE), for high-resolution 3D reconstruction from a limited number of 2D projections. GENFIRE first assembles a 3D Fourier grid with oversampling and then iteratesmore » between real and reciprocal space to search for a global solution that is concurrently consistent with the measured data and general physical constraints. The algorithm requires minimal human intervention and also incorporates angular refinement to reduce the tilt angle error. We demonstrate that GENFIRE can produce superior results relative to several other popular tomographic reconstruction techniques through numerical simulations and by experimentally reconstructing the 3D structure of a porous material and a frozen-hydrated marine cyanobacterium. As a result, equipped with a graphical user interface, GENFIRE is freely available from our website and is expected to find broad applications across different disciplines.« less

  13. Venus atmosphere simulated by a high-resolution general circulation model

    NASA Astrophysics Data System (ADS)

    Sugimoto, Norihiko

    2016-07-01

    An atmospheric general circulation model (AGCM) for Venus on the basis of AFES (AGCM For the Earth Simulator) have been developed (e.g., Sugimoto et al., 2014a) and a very high-resolution simulation is performed. The highest resolution of the model is T319L120; 960 times 480 horizontal grids (grid intervals are about 40 km) with 120 vertical layers (layer intervals are about 1 km). In the model, the atmosphere is dry and forced by the solar heating with the diurnal and semi-diurnal components. The infrared radiative process is simplified by adopting Newtonian cooling approximation. The temperature is relaxed to a prescribed horizontally uniform temperature distribution, in which a layer with almost neutral static stability observed in the Venus atmosphere presents. A fast zonal wind in a solid-body rotation is given as the initial state. Starting from this idealized superrotation, the model atmosphere reaches a quasi-equilibrium state within 1 Earth year and this state is stably maintained for more than 10 Earth years. The zonal-mean zonal flow with weak midlatitude jets has almost constant velocity of 120 m/s in latitudes between 45°S and 45°N at the cloud top levels, which agrees very well with observations. In the cloud layer, baroclinic waves develop continuously at midlatitudes and generate Rossby-type waves at the cloud top (Sugimoto et al., 2014b). At the polar region, warm polar vortex zonally surrounded by a cold latitude band (cold collar) is well reproduced (Ando et al., 2016). As for horizontal kinetic energy spectra, divergent component is broadly (k>10) larger than rotational component compared with that on Earth (Kashimura et al., in preparation). Finally, recent results for thermal tides and small-scale waves will be shown in the presentation. Sugimoto, N. et al. (2014a), Baroclinic modes in the Venus atmosphere simulated by GCM, Journal of Geophysical Research: Planets, Vol. 119, p1950-1968. Sugimoto, N. et al. (2014b), Waves in a Venus general

  14. Management Issues for Adolescents with Cystic Fibrosis

    PubMed Central

    Withers, Adelaide Lindsay

    2012-01-01

    The healthy adolescent will encounter major changes in biological and psychosocial domains. The adolescent period can be greatly affected by a chronic illness. Cystic fibrosis is a terminal illness that can significantly affect an adolescent's biological, mental and psychosocial health. This paper discusses general issues to consider when managing an adolescent with a chronic medical condition, and specifically how cystic fibrosis may impact upon puberty, body image, risk-taking behaviours, mental health, independence, nonadherence, reproductive health, transition, lung transplantation, and end of life care. PMID:22991662

  15. Thymic basaloid carcinoma: a clinicopathologic study of 12 cases, with a general discussion of basaloid carcinoma and its relationship with adenoid cystic carcinoma.

    PubMed

    Brown, Jeffrey G; Familiari, Ubaldo; Papotti, Mauro; Rosai, Juan

    2009-08-01

    aggressive behavior and significant mortality. In this paper, we review the pertinent literature and discuss the possible relationship of thymic BC with thymic adenoid cystic carcinoma, as well as BCs and adenoid cystic carcinomas at other sites.

  16. High-resolution in vivo diffusion imaging of the human brain with generalized slice dithered enhanced resolution: Simultaneous multislice (gSlider-SMS).

    PubMed

    Setsompop, Kawin; Fan, Qiuyun; Stockmann, Jason; Bilgic, Berkin; Huang, Susie; Cauley, Stephen F; Nummenmaa, Aapo; Wang, Fuyixue; Rathi, Yogesh; Witzel, Thomas; Wald, Lawrence L

    2017-03-05

    To develop an efficient acquisition for high-resolution diffusion imaging and allow in vivo whole-brain acquisitions at 600- to 700-μm isotropic resolution. We combine blipped-controlled aliasing in parallel imaging simultaneous multislice (SMS) with a novel slab radiofrequency (RF) encoding gSlider (generalized slice-dithered enhanced resolution) to form a signal-to-noise ratio-efficient volumetric simultaneous multislab acquisition. Here, multiple thin slabs are acquired simultaneously with controlled aliasing, and unaliased with parallel imaging. To achieve high resolution in the slice direction, the slab is volumetrically encoded using RF encoding with a scheme similar to Hadamard encoding. However, with gSlider, the RF-encoding bases are specifically designed to be highly independent and provide high image signal-to-noise ratio in each slab acquisition to enable self-navigation of the diffusion's phase corruption. Finally, the method is combined with zoomed imaging (while retaining whole-brain coverage) to facilitate low-distortion single-shot in-plane encoding with echo-planar imaging at high resolution. A 10-slices-per-shot gSlider-SMS acquisition was used to acquire whole-brain data at 660 and 760 μm isotropic resolution with b-values of 1500 and 1800 s/mm(2) , respectively. Data were acquired on the Connectome 3 Tesla scanner with 64-channel head coil. High-quality data with excellent contrast were achieved at these resolutions, which enable the visualization of fine-scale structures. The gSlider-SMS approach provides a new, efficient way to acquire high-resolution diffusion data. Magn Reson Med, 2017. © 2017 International Society for Magnetic Resonance in Medicine. © 2017 International Society for Magnetic Resonance in Medicine.

  17. Self-management education for cystic fibrosis.

    PubMed

    Savage, Eileen; Beirne, Paul V; Ni Chroinin, Muireann; Duff, Alistair; Fitzgerald, Tony; Farrell, Dawn

    2011-07-06

    Self-management education may help patients with cystic fibrosis and their families to choose, monitor and adjust treatment requirements for their illness, and also to manage the effects of illness on their lives. Although self-management education interventions have been developed for cystic fibrosis, no previous systematic review of the evidence of effectiveness of these interventions has been conducted. To assess the effects of self-management education interventions on improving health outcomes for patients with cystic fibrosis and their caregivers We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register (date of the last search: 23 February 2011).We also searched databases through EBSCO (CINAHL; Psychological and Behavioural Sciences Collection; PsychInfo; SocINDEX) and Elsevier (EMBASE) and handsearched relevant journals and conference proceedings (date of the last searches: 30th March 2011). Randomised controlled trials, quasi-randomised controlled trials or controlled clinical trials comparing different types of self-management education for cystic fibrosis or comparing self-management education with standard care or no intervention. Two authors assessed trial eligibility and risk of bias. Three authors extracted data. Four trials (involving a total of 269 participants) were included. The participants were children with cystic fibrosis and their parents or caregivers in three trials and adults with cystic fibrosis in one trial. The trials compared four different self-management education interventions versus standard treatment: (1) a training programme for managing cystic fibrosis in general; (2) education specific to aerosol and airway clearance treatments; (3) disease-specific nutrition education; and (4) general and disease-specific nutrition education. Training children to manage cystic fibrosis in general had no statistically significant effects on weight after six to eight weeks, mean difference -7.74 lb (95% confidence

  18. Self-management education for cystic fibrosis.

    PubMed

    Savage, Eileen; Beirne, Paul V; Ni Chroinin, Muireann; Duff, Alistair; Fitzgerald, Tony; Farrell, Dawn

    2014-09-08

    Self-management education may help patients with cystic fibrosis and their families to choose, monitor and adjust treatment requirements for their illness, and also to manage the effects of illness on their lives. Although self-management education interventions have been developed for cystic fibrosis, no previous systematic review of the evidence of effectiveness of these interventions has been conducted. To assess the effects of self-management education interventions on improving health outcomes for patients with cystic fibrosis and their caregivers We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register (date of the last search: 22 August 2013).We also searched databases through EBSCO (CINAHL; Psychological and Behavioural Sciences Collection; PsychInfo; SocINDEX) and Elsevier (Embase) and handsearched relevant journals and conference proceedings (date of the last searches: 01 February 2014 ). Randomised controlled trials, quasi-randomised controlled trials or controlled clinical trials comparing different types of self-management education for cystic fibrosis or comparing self-management education with standard care or no intervention. Two authors assessed trial eligibility and risk of bias. Three authors extracted data. Four trials (involving a total of 269 participants) were included. The participants were children with cystic fibrosis and their parents or caregivers in three trials and adults with cystic fibrosis in one trial. The trials compared four different self-management education interventions versus standard treatment: (1) a training programme for managing cystic fibrosis in general; (2) education specific to aerosol and airway clearance treatments; (3) disease-specific nutrition education; and (4) general and disease-specific nutrition education. Training children to manage cystic fibrosis in general had no statistically significant effects on weight after six to eight weeks, mean difference -7.74 lb (i.e. 3.51 kg) (95

  19. Performance of a reconfigured atmospheric general circulation model at low resolution

    NASA Astrophysics Data System (ADS)

    Wen, Xinyu; Zhou, Tianjun; Wang, Shaowu; Wang, Bin; Wan, Hui; Li, Jian

    2007-07-01

    Paleoclimate simulations usually require model runs over a very long time. The fast integration version of a state-of-the-art general circulation model (GCM), which shares the same physical and dynamical processes but with reduced horizontal resolution and increased time step, is usually developed. In this study, we configure a fast version of an atmospheric GCM (AGCM), the Grid Atmospheric Model of IAP/LASG (Institute of Atmospheric Physics/State Key Laboratory of Numerical Modeling for Atmospheric Sciences and Geophysical Fluid Dynamics), at low resolution (GAMIL-L, hereafter), and compare the simulation results with the NCEP/NCAR reanalysis and other data to examine its performance. GAMIL-L, which is derived from the original GAMIL, is a finite difference AGCM with 72×40 grids in longitude and latitude and 26 vertical levels. To validate the simulated climatology and variability, two runs were achieved. One was a 60-year control run with fixed climatological monthly sea surface temperature (SST) forcing, and the other was a 50-yr (1950 2000) integration with observational time-varying monthly SST forcing. Comparisons between these two cases and the reanalysis, including intra-seasonal and inter-annual variability are also presented. In addition, the differences between GAMIL-L and the original version of GAMIL are also investigated. The results show that GAMIL-L can capture most of the large-scale dynamical features of the atmosphere, especially in the tropics and mid latitudes, although a few deficiencies exist, such as the underestimated Hadley cell and thereby the weak strength of the Asia summer monsoon. However, the simulated mean states over high latitudes, especially over the polar regions, are not acceptable. Apart from dynamics, the thermodynamic features mainly depend upon the physical parameterization schemes. Since the physical package of GAMIL-L is exactly the same as the original high-resolution version of GAMIL, in which the NCAR Community

  20. Androgen excess in cystic acne.

    PubMed

    Marynick, S P; Chakmakjian, Z H; McCaffree, D L; Herndon, J H

    1983-04-28

    We measured hormone levels in 59 women and 32 men with longstanding cystic acne resistant to conventional therapy. Affected women had higher serum levels of dehydroepiandrosterone sulfate, testosterone, and luteinizing hormone and lower levels of sex-hormone-binding globulin than controls. Affected men had higher levels of serum dehydroepiandrosterone sulfate and 17-hydroxyprogesterone and lower levels of sex-hormone-binding globulin than controls. To lower dehydroepiandrosterone sulfate, dexamethasone was given to men, and dexamethasone or an oral contraceptive pill, Demulen (or both), was given to women. Of the patients treated for six months, 97 per cent of the women and 81 per cent of the men had resolution or marked improvement in their acne. The dose of dexamethasone required to reduce dehydroepiandrosterone sulfate levels was low, rarely exceeding the equivalent of 20 mg of hydrocortisone per day. We conclude that most patients with therapeutically resistant cystic acne have androgen excess and that lowering elevated dehydroepiandrosterone sulfate results in improvement or remission of acne in most instances.

  1. New animal models of cystic fibrosis: what are they teaching us?

    PubMed Central

    Keiser, Nicholas W.; Engelhardt, John F.

    2013-01-01

    Purpose of review Cystic fibrosis is the first human genetic disease to benefit from the directed engineering of three different species of animal models (mice, pigs, and ferrets). Recent studies on the cystic fibrosis pig and ferret models are providing new information about the pathophysiology of cystic fibrosis in various organ systems. Additionally, new conditional cystic fibrosis transmembrane conductance regulator (CFTR) knockout mice are teaching unexpected lessons about CFTR function in surprising cellular locations. Comparisons between these animal models and the human condition are key to dissecting the complexities of disease pathophysiology in cystic fibrosis. Recent findings Cystic fibrosis pigs and ferrets have provided new models to study the spontaneous development of disease in the lung and pancreas, two organs that are largely spared overt spontaneous disease in cystic fibrosis mice. New cystic fibrosis mouse models are now interrogating CFTR functions involved in growth and inflammation at an organ-based level using conditional knockout technology. Together, these models are providing new insights on the human condition. Summary Basic and clinical cystic fibrosis research will benefit greatly from the comparative pathophysiology of cystic fibrosis mice, pigs, and ferrets. Both similarities and differences between these three cystic fibrosis models will inform pathophysiologically important mechanisms of CFTR function in humans and aid in the development of both organ-specific and general therapies for cystic fibrosis. PMID:21857224

  2. Martian atmospheric gravity waves simulated by a high-resolution general circulation model

    NASA Astrophysics Data System (ADS)

    Kuroda, Takeshi; Yiǧit, Erdal; Medvedev, Alexander S.; Hartogh, Paul

    2016-07-01

    Gravity waves (GWs) significantly affect temperature and wind fields in the Martian middle and upper atmosphere. They are also one of the observational targets of the MAVEN mission. We report on the first simulations with a high-resolution general circulation model (GCM) and present a global distributions of small-scale GWs in the Martian atmosphere. The simulated GW-induced temperature variances are in a good agreement with available radio occultation data in the lower atmosphere between 10 and 30 km. For the northern winter solstice, the model reveals a latitudinal asymmetry with stronger wave generation in the winter hemisphere and two distinctive sources of GWs: mountainous regions and the meandering winter polar jet. Orographic GWs are filtered upon propagating upward, and the mesosphere is primarily dominated by harmonics with faster horizontal phase velocities. Wave fluxes are directed mainly against the local wind. GW dissipation in the upper mesosphere generates a body force per unit mass of tens of m s^{-1} per Martian solar day (sol^{-1}), which tends to close the simulated jets. The results represent a realistic surrogate for missing observations, which can be used for constraining GW parameterizations and validating GCMs.

  3. Crystal Structure of Human General Transcription Factor TFIIE at Atomic Resolution.

    PubMed

    Miwa, Kohei; Kojima, Rieko; Obita, Takayuki; Ohkuma, Yoshiaki; Tamura, Yasushi; Mizuguchi, Mineyuki

    2016-10-23

    In eukaryotes, RNA polymerase II requires general transcription factors to initiate mRNA transcription. TFIIE subunits α and β form a heterodimer and recruit TFIIH to complete the assembly of the pre-initiation complex. Here, we have determined the crystal structure of human TFIIE at atomic resolution. The N-terminal half of TFIIEα forms an extended winged helix (WH) domain with an additional helix, followed by a zinc-finger domain. TFIIEβ contains the WH2 domain, followed by two coiled-coil helices intertwining with TFIIEα. We also showed that TFIIEα binds to TFIIEβ with nanomolar affinity using isothermal titration calorimetry. In addition, mutations on the residues involved in the interactions resulted in severe growth defects in yeast. Lack of the C-terminal region of yeast TFIIEβ causes a mild growth defect in vivo. These findings provide a structural basis for understanding the functional mechanisms of TFIIE in the context of pre-initiation complex formation and transcription initiation. Copyright © 2016 Elsevier Ltd. All rights reserved.

  4. [Atypical intraperitoneal cystic masses].

    PubMed

    Domínguez-Pérez, S T; Baeza-Herrera, C; Villalobos-Castillejos, A; González-Mateos, T; Aguilar-Venegas, M

    2010-01-01

    Omental, mesenteric and retroperitoneal cystic masses are very rare in pediatric population. They usually present as asymptomatic abdominal tumors and only occasionally a preoperative diagnosis is made. We presented 4 cases presented to our hospital during a 2-year period. All patients were male with an age ranged from 5 months to 7 years. Three patients had previous diagnosis of cystic mass by abdominal ultrasound. Three patients presented with acute abdomen and one patient with bowel obstruction. All patients underwent successful resection of the mass with no perioperative mortality.

  5. Future climate of the Caribbean from a super-high-resolution atmospheric general circulation model

    NASA Astrophysics Data System (ADS)

    Hall, Trevor C.; Sealy, Andrea M.; Stephenson, Tannecia S.; Kusunoki, Shoji; Taylor, Michael A.; Chen, A. Anthony; Kitoh, Akio

    2013-07-01

    Present-day (1979-2003) and future (2075-2099) simulations of mean and extreme rainfall and temperature are examined using data from the Meteorological Research Institute super-high-resolution atmospheric general circulation model. Analyses are performed over the 20-km model grid for (1) a main Caribbean basin, (2) sub-regional zones, and (3) specific Caribbean islands. Though the model's topography underestimates heights over the eastern Caribbean, it captures well the present-day spatial and temporal variations of seasonal and annual climates. Temperature underestimations range from 0.1 °C to 2 °C with respect to the Japanese Reanalysis and the Climatic Research Unit datasets. The model also captures fairly well sub-regional scale variations in the rainfall climatology. End-of-century projections under the Intergovernmental Panel on Climate Change SRES A1B scenario indicate declines in rainfall amounts by 10-20 % for most of the Caribbean during the early (May-July) and late (August-October) rainy seasons relative to the 1979-2003 baselines. The early dry season (November-January) is also projected to get wetter in the far north and south Caribbean by approximately 10 %. The model also projects a warming of 2-3 °C over the Caribbean region. Analysis of future climate extremes indicate a 5-10 % decrease in the simple daily precipitation intensity but no significant change in the number of consecutive dry days for Cuba, Jamaica, southern Bahamas, and Haiti. There is also indication that the number of hot days and nights will significantly increase over the main Caribbean basin.

  6. Cystic Fibrosis: Brazilian ENT Experience

    PubMed Central

    Sih, Tania; Godinho, Ricardo; Franco, Leticia Paiva; Piltcher, Otávio

    2012-01-01

    Most published studies about Cystic Fibrosis (CF) are European or North American. There are still few publications about the characteristics of fibrocystic populations in developing countries. The incidence of cystic fibrosis (CF) in Brazil varies among different regions (1 : 10,000 in Minas Gerais, 1 : 9,500 in Paraná, 1 : 8,700 in Santa Catarina, and 1 : 1600 in Rio Grande do Sul). The prevalence of the DF508 mutation also varies according to population: 33% in Sao Paulo, 49% in Rio Grande do Sul, 27% in Santa Catarina, and 52% in Minas Gerais. Cough and nasal obstruction are the most common symptoms. The variation in nasal polyposis prevalence may be explained by population genotypic characteristics in a country that spans a continent. Findings on nasal endoscopy and computed tomography (CT) have better correlation than do this information compared with surgical and clinical history. Microbiologic studies suggest a high level of early contamination of the airways. Sensorineural hearing loss (SNHL) occurs in these patients as a result of ototoxic antibiotics. The data compiled in this paper is useful, but also lead to the general agreement that more research would be welcome due to the unique characteristics of this country. PMID:22611403

  7. A Variable Resolution Atmospheric General Circulation Model for a Megasite at the North Slope of Alaska

    NASA Astrophysics Data System (ADS)

    Dennis, L.; Roesler, E. L.; Guba, O.; Hillman, B. R.; McChesney, M.

    2016-12-01

    The Atmospheric Radiation Measurement (ARM) climate research facility has three siteslocated on the North Slope of Alaska (NSA): Barrrow, Oliktok, and Atqasuk. These sites, incombination with one other at Toolik Lake, have the potential to become a "megasite" whichwould combine observational data and high resolution modeling to produce high resolutiondata products for the climate community. Such a data product requires high resolutionmodeling over the area of the megasite. We present three variable resolution atmosphericgeneral circulation model (AGCM) configurations as potential alternatives to stand-alonehigh-resolution regional models. Each configuration is based on a global cubed-sphere gridwith effective resolution of 1 degree, with a refinement in resolution down to 1/8 degree overan area surrounding the ARM megasite. The three grids vary in the size of the refined areawith 13k, 9k, and 7k elements. SquadGen, NCL, and GIMP are used to create the grids.Grids vary based upon the selection of areas of refinement which capture climate andweather processes that may affect a proposed NSA megasite. A smaller area of highresolution may not fully resolve climate and weather processes before they reach the NSA,however grids with smaller areas of refinement have a significantly reduced computationalcost compared with grids with larger areas of refinement. Optimal size and shape of thearea of refinement for a variable resolution model at the NSA is investigated.

  8. [Intestinal cystic duplication in infants and the etiology of intussusception].

    PubMed

    Kasis, A; Sabo, E; Mogilner, J G; Boss, J

    1993-11-15

    2 infants, 3 months old and 8 months, respectively, with restlessness and vomiting were each found to have ileocolic intussusception with barium filling defects. Laparotomy disclosed in each a dome-shaped structure, 2 cm and 0.6 cm in greatest diameter, respectively, on the antimesenteric side of the ileal wall. Histological examination showed cystic duplication of the ileum. It is suggested that manual reduction generally fails when cystic duplication is an etiological factor, and surgery is then mandatory.

  9. A Variable Resolution Stretched Grid General Circulation Model: Regional Climate Simulation

    NASA Technical Reports Server (NTRS)

    Fox-Rabinovitz, Michael S.; Takacs, Lawrence L.; Govindaraju, Ravi C.; Suarez, Max J.

    2000-01-01

    The development of and results obtained with a variable resolution stretched-grid GCM for the regional climate simulation mode, are presented. A global variable resolution stretched- grid used in the study has enhanced horizontal resolution over the U.S. as the area of interest The stretched-grid approach is an ideal tool for representing regional to global scale interaction& It is an alternative to the widely used nested grid approach introduced over a decade ago as a pioneering step in regional climate modeling. The major results of the study are presented for the successful stretched-grid GCM simulation of the anomalous climate event of the 1988 U.S. summer drought- The straightforward (with no updates) two month simulation is performed with 60 km regional resolution- The major drought fields, patterns and characteristics such as the time averaged 500 hPa heights precipitation and the low level jet over the drought area. appear to be close to the verifying analyses for the stretched-grid simulation- In other words, the stretched-grid GCM provides an efficient down-scaling over the area of interest with enhanced horizontal resolution. It is also shown that the GCM skill is sustained throughout the simulation extended to one year. The developed and tested in a simulation mode stretched-grid GCM is a viable tool for regional and subregional climate studies and applications.

  10. [New tools in cystic fibrosis].

    PubMed

    Dournes, G; De Boeck, K; Bui, S; Vermeulen, F; Ramalho, A; Chateil, J-F; Laurent, F; Fayon, M

    2016-12-01

    The use of 3 novel tools available for the diagnosis and treatment in cystic fibrosis are described here. 1) The lung clearance index is a sensitive method which can detect functional impairment in the first months after birth. 2) Detailed morphological analyses of the lung can be performed with the new MRI sequences, without any contrast medium or risk of radiation. The analysis of functional MRI data (perfusion, diffusion, ventilation, inflammation) will be possible, and these data will be correlated to morphological data. The exploration of other organs such as the sinuses, liver and abdomen during the same examination represents another definite advantage. 3) Organoïds are a good example of personalized medicine. This tool explores CFTR function and treatment response in each of the 2000 or so known CFTR mutations. These tests are limited to specialized centers, mostly within a research context. However, their generalization after standardization is expected in the near future.

  11. Cystic Fibrosis: Prenatal Screening and Diagnosis

    MedlinePlus

    ... Management Education & Events Advocacy For Patients About ACOG Cystic Fibrosis: Prenatal Screening and Diagnosis Home For Patients Search ... Screening and Diagnosis FAQ171, June 2017 PDF Format Cystic Fibrosis: Prenatal Screening and Diagnosis Pregnancy What is cystic ...

  12. Accurate and general treatment of electrostatic interaction in Hamiltonian adaptive resolution simulations

    NASA Astrophysics Data System (ADS)

    Heidari, M.; Cortes-Huerto, R.; Donadio, D.; Potestio, R.

    2016-10-01

    In adaptive resolution simulations the same system is concurrently modeled with different resolution in different subdomains of the simulation box, thereby enabling an accurate description in a small but relevant region, while the rest is treated with a computationally parsimonious model. In this framework, electrostatic interaction, whose accurate treatment is a crucial aspect in the realistic modeling of soft matter and biological systems, represents a particularly acute problem due to the intrinsic long-range nature of Coulomb potential. In the present work we propose and validate the usage of a short-range modification of Coulomb potential, the Damped shifted force (DSF) model, in the context of the Hamiltonian adaptive resolution simulation (H-AdResS) scheme. This approach, which is here validated on bulk water, ensures a reliable reproduction of the structural and dynamical properties of the liquid, and enables a seamless embedding in the H-AdResS framework. The resulting dual-resolution setup is implemented in the LAMMPS simulation package, and its customized version employed in the present work is made publicly available.

  13. Modeling of Arctic Storms with a Variable High-Resolution General Circulation Model

    SciTech Connect

    Taylor, Mark A.; Roesler, Erika Louise; Bosler, Peter Andrew

    2015-08-01

    The Department of Energy’s (DOE) Biological and Environmental Research project, “Water Cycle and Climate Extremes Modeling” is improving our understanding and modeling of regional details of the Earth’s water cycle. Sandia is using high resolution model behavior to investigate storms in the Arctic.

  14. The Cystic Fibrosis Intestine

    PubMed Central

    De Lisle, Robert C.; Borowitz, Drucy

    2013-01-01

    The clinical manifestations of cystic fibrosis (CF) result from dysfunction of the cystic fibrosis transmembrane regulator protein (CFTR). The majority of people with CF have a limited life span as a consequence of CFTR dysfunction in the respiratory tract. However, CFTR dysfunction in the gastrointestinal (GI) tract occurs earlier in ontogeny and is present in all patients, regardless of genotype. The same pathophysiologic triad of obstruction, infection, and inflammation that causes disease in the airways also causes disease in the intestines. This article describes the effects of CFTR dysfunction on the intestinal tissues and the intraluminal environment. Mouse models of CF have greatly advanced our understanding of the GI manifestations of CF, which can be directly applied to understanding CF disease in humans. PMID:23788646

  15. Abdominal cystic lymphangioma mimicking appendicitis.

    PubMed

    Wake, Sarah; Abhyankar, Aruna; Hutton, Kim

    2013-06-01

    A cystic lymphangioma arising within the abdomen is a rare entity in children. It may present with an abdominal mass and symptoms of abdominal pain, vomiting, and anorexia. These nonspecific clinical symptoms are often attributed to more common acute pediatric conditions. In this report, we describe two pediatric cases of intra-abdominal cystic lymphangioma that were initially diagnosed and treated as appendicitis. True diagnosis was only achieved on surgical excision and pathological investigation of cystic material.

  16. Resolution Dependence of Equatorial Precipitation Activities Represented in a General Circulation Model with No Cumulus Parameterization

    NASA Astrophysics Data System (ADS)

    Nakajima, K.; Yamada, Y.; Takahashi, Y. O.; Ishiwatari, M.; Ohfuchi, W.; Hayashi, Y. Y.

    2015-12-01

    An aquaplanet experiment is performed to explore how representation of equatorial precipitation activities in a GCM depends on its horizontal resolution. The numerical model utilized is AFES, an AGCM optimized for the Earth Simulator (Ohfuchi et al 2004). For the sake of simplicity, we adopt no cumulus parametarizetion scheme, although large scale condensation scheme is retained. We compare the results with increasing horizontal resolution from T39 to T319. Other setups follows those of the CONTROL case of the Aqua Planet Experiment (APE) Project (Neale and Hoskins, 2000; Blackburn and Hoskins, 2013). We can identify two kinds of resolution dependences; one is zonal and the other is meridional. Zonally, at lower horizontal resolution, precipitation occurs as disturbances with the scale of a few gridpints, and propagate both eastward and westward. As the resolution increases, zonal scale of precipitation disturbances decreases, and there appears a hierarchical structure: grid scale disturbances move westward, while they are organized into eastward moving, Kelvin wave like envelopes. The westward motion of grid scale precipitation structure can be related to the advection of inhomogeniety of moisture by the zonal mean wind. Another kind of large scale organization identified is the north-south "zigzag" structure of the distribution of grid scale precipitation disturbances, which has zonal wavenumber two and propagates westward. This structure appear only when the model can resolve the moist equatorial radius of deformation, and can be interpreted as moist mixed-Rossby gravity wave. The Mixed-Rossby gravity wave and the Kelvin wave like structure occur superposed to each other. Features similar to the above can also be noted in some of the AGCM runs with cumuls parameterizations in the APE (Blackburn et al, 2013; Williamson et al 2013; Nakajima et al 2013). Acknowledgement:Numerical experiments were performed at the Earth Simulator, JAMSTEC.

  17. Treatment and prognosis of rectal prolapse in cystic fibrosis.

    PubMed

    Stern, R C; Izant, R J; Boat, T F; Wood, R E; Matthews, L W; Doershuk, C F

    1982-04-01

    Rectal prolapse occurred in 112 (18.5%) of 605 cystic fibrosis patients. In 48 patients prolapse preceded diagnosis of cystic fibrosis, but physicians (pediatricians, pediatric and general surgeons, and proctologists) rarely appreciated its importance as a symptom of this disease. Prolapses frequently cease with institution of pancreatic enzyme replacement therapy following diagnosis of cystic fibrosis. However, even when the disease remains undiagnosed, the symptom is often transient and frequently resolves at 3-5 yr of age. Prolapse occurring initially after cystic fibrosis is diagnosed rarely responds to manipulation of diet or enzyme doses. Many patients develop a method of reduction which involves voluntary abdominal, perineal, and gluteal muscles and does not require manual pressure on the prolapsed segment. Most patients do not need specific treatment for the prolapse. Surgery is rarely necessary. A sweat test should be obtained on any child who has had even a single episode of rectal prolapse.

  18. Benign cystic peritoneal mesothelioma.

    PubMed Central

    Bhandarkar, D S; Smith, V J; Evans, D A; Taylor, T V

    1993-01-01

    The well defined but rare entity of benign cystic mesothelioma is reported. The aetiology of this neoplasm remains obscure. The presenting features make a precise preoperative diagnosis difficult; information provided by computed tomography and cytology may help. A firm diagnosis can only come from electron microscopic or immunohistochemical examination of the tumour. Diagnostic accuracy and diligent follow up are essential because, although the tumour is considered benign, it does tend towards local recurrence. Images PMID:8227441

  19. Cystic pulmonary hydatidosis

    PubMed Central

    Sarkar, Malay; Pathania, Rajnish; Jhobta, Anupam; Thakur, Babu Ram; Chopra, Rajesh

    2016-01-01

    Cystic echinococcosis (CE) is a zoonotic parasitic disease caused by the larval stages of the cestode Echinococcus granulosus. Worldwide, pulmonary hydatid cyst is a significant problem medically, socially, and economically. Surgery is the definitive therapy of pulmonary hydatidosis. Benzimidazoles may be considered in patients with a surgical contraindication. This review will focus on pathogenesis, lifecycle, clinical features, and management of pulmonary hydatid disease. PMID:27051107

  20. Using the Variable-Resolution General Circulation Model CAM-SE to Simulate Regional Tropical Cyclone Climatology

    NASA Astrophysics Data System (ADS)

    Zarzycki, C. M.; Jablonowski, C.; Taylor, M. A.

    2012-12-01

    The ability of General Circulation Models (GCMs) to resolve tropical cyclones in the climate system has traditionally been difficult due to issues such as small storm size and the existence of key thermodynamic processes requiring significant parameterization. At traditional GCM grid resolutions of 50-300 km tropical cyclones are severely under-resolved, if not totally unresolved. Recent improvements in computational ability as well as advances in GCM model design now allow for simulations with grid spacings as small as 10-25 km. At these resolutions, models are able to more effectively capture key dynamical features of tropical cyclones. This paper explores a variable-resolution global model approach that allows for high spatial resolutions in areas of interest, such as low-latitude ocean basins where tropical cyclogenesis occurs. Such GCM designs with multi-resolution meshes serve to bridge the gap between globally uniform grids and limited area models and have the potential to become a future tool for regional climate assessments. A statically-nested, variable-resolution option has recently been introduced into the National Center for Atmospheric Research (NCAR) Community Atmosphere Model's (CAM) Spectral Element (SE) dynamical core. The SE dynamical core is also known as the 'High-Order Method Modeling Environment' (HOMME). We present aquaplanet climate experiments which showcase the ability of nested meshes to produce realistic tropical cyclones selectively in high resolution grids embedded within a global domain. We also evaluate model performance when coupled to an active land model and forced with historical sea surface temperatures by comparing multi-year results from variable-resolution CAM-SE to other globally-uniform high resolution tropical cyclone studies recently completed by the climate modeling community. Specific focus is paid to intensity profiles and track densities as well as the interannual variability in storm count in tropical regions of

  1. Cystic fibrosis and coeliac disease

    PubMed Central

    Goodchild, Mary C.; Nelson, R.; Anderson, Charlotte M.

    1973-01-01

    Two children, unrelated, in whom cystic fibrosis had been diagnosed at the ages of 14 weeks and 10 months, respectively, were found subsequently to suffer from coeliac disease as well. Both children are responding well to dietary gluten withdrawal and to conventional treatment for cystic fibrosis. It is suggested that cystic fibrosis may predispose to the development of coeliac disease. Therefore, jejunal biopsy may be a useful investigation in the occasional child with cystic fibrosis, who presents with unusual features, and who fails to thrive as well as expected. ImagesFIG. 2FIG. 3FIG. 5FIG. 6 PMID:4517654

  2. Neonatal cystic fibrosis screening test

    MedlinePlus

    Cystic fibrosis screening - neonatal; Immunoreactive trypsinogen; IRT test; CF - screening ... better nutrition, growth, and lung function. This screening test helps doctors identify children with CF before they ...

  3. Clostridium difficile pancolitis in adults with cystic fibrosis.

    PubMed

    Barker, H C; Haworth, C S; Williams, D; Roberts, P; Bilton, D

    2008-09-01

    We report three cases of Clostridium difficile pancolitis in adults with cystic fibrosis (CF) in whom the presenting symptoms were atypical. All three required treatment with systemic steroids, in addition to oral vancomycin and metronidazole to achieve resolution of the colitis. This experience suggests that C. difficile colitis should be considered in individuals with CF presenting with non-specific abdominal symptoms.

  4. Simulations of Madden-Julian Oscillation in High Resolution Atmospheric General Circulation Model

    NASA Astrophysics Data System (ADS)

    Deng, Liping; Stenchikov, Georgiy; McCabe, Matthew; Bangalath, HamzaKunhu; Raj, Jerry; Osipov, Sergey

    2014-05-01

    The simulation of tropical signals, especially the Madden-Julian Oscillation (MJO), is one of the major deficiencies in current numerical models. The unrealistic features in the MJO simulations include the weak amplitude, more power at higher frequencies, displacement of the temporal and spatial distributions, eastward propagation speed being too fast, and a lack of coherent structure for the eastward propagation from the Indian Ocean to the Pacific (e.g., Slingo et al. 1996). While some improvement in simulating MJO variance and coherent eastward propagation has been attributed to model physics, model mean background state and air-sea interaction, studies have shown that the model resolution, especially for higher horizontal resolution, may play an important role in producing a more realistic simulation of MJO (e.g., Sperber et al. 2005). In this study, we employ unique high-resolution (25-km) simulations conducted using the Geophysical Fluid Dynamics Laboratory global High Resolution Atmospheric Model (HIRAM) to evaluate the MJO simulation against the European Center for Medium-range Weather Forecasts (ECMWF) Interim re-analysis (ERAI) dataset. We specifically focus on the ability of the model to represent the MJO related amplitude, spatial distribution, eastward propagation, and horizontal and vertical structures. Additionally, as the HIRAM output covers not only an historic period (1979-2012) but also future period (2012-2050), the impact of future climate change related to the MJO is illustrated. The possible changes in intensity and frequency of extreme weather and climate events (e.g., strong wind and heavy rainfall) in the western Pacific, the Indian Ocean and the Middle East North Africa (MENA) region are highlighted.

  5. Some lessons and thoughts from development of an old-fashioned high-resolution atmospheric general circulation model

    NASA Astrophysics Data System (ADS)

    Ohfuchi, Wataru; Enomoto, Takeshi; Yoshioka, Mayumi K.; Takaya, Koutarou

    2014-05-01

    Some high-resolution simulations with a conventional atmospheric general circulation model (AGCM) were conducted right after the first Earth Simulator started operating in the spring of 2002. More simulations with various resolutions followed. The AGCM in this study, AFES (Agcm For the Earth Simulator), is a primitive equation spectral transform method model with a cumulus convection parameterization. In this presentation, some findings from comparisons between high and low-resolution simulations, and some future perspectives of old-fashioned AGCMs will be discussed. One obvious advantage of increasing resolution is capability of resolving the fine structures of topography and atmospheric flow. By increasing resolution from T39 (about 320 km horizontal grid interval) to T79 (160 km), to T159 (80 km) to T319 (40 km), topographic precipitation over Japan becomes increasingly realistic. This feature is necessary for climate and weather studies involving both global and local aspects. In order to resolve submesoscale (about 100 km horizontal scale) atmospheric circulation, about 10-km grid interval is necessary. Comparing T1279 (10 km) simulations with T319 ones, it is found that, for example, the intensity of heavy rain associated with Baiu front and the central pressure of typhoon become more realistic. These realistic submesoscale phenomena should have impact on larger-sale flow through dynamics and thermodynamics. An interesting finding by increasing horizontal resolution of a conventional AGCM is that some cumulus convection parameterizations, such as Arakawa-Schubert type scheme, gradually stop producing precipitation, while some others, such as Emanuel type, do not. With the former, the grid condensation increases with the model resolution to compensate. Which characteristics are more desirable is arguable but it is an important feature one has to consider when developing a high-resolution conventional AGCM. Many may think that conventional primitive equation

  6. Overview of the clinical problem: facts and current issues of mucinous cystic neoplasms of the pancreas.

    PubMed

    Jeurnink, S M; Vleggaar, F P; Siersema, P D

    2008-11-01

    Pancreatic cystic lesions are uncommon and consist of pseudocysts, congenital cysts and cystic neoplasms including mucinous cystic neoplasms, intraductal papillary mucinous neoplasms and serous cystic neoplasms. Mucinous cystic neoplasms are large septated cysts without connection to the ductal system, characterised by the presence of thick-walled ovarian-type stroma and mucin. They occur predominantly in women and often are malignant. Therefore, surgical resection is recommended. Intraductal papillary mucinous neoplasms are neoplasms with tall, columnar, mucin-containing epithelium involving the main pancreatic ducts or major side branches. Intraductal papillary mucinous neoplasms occur in men and women in their 60s and 70s and may differentiate into malignant neoplasms. Therefore, surgical resection is mandatory. Serous cystic neoplasms appear as multiple cysts lined with cubic flat epithelium containing glycogen-rich cells with clear cytoplasm. They mainly occur in women in their 50s and are generally benign. Therefore, a conservative approach is recommended. As both mucinous cystic neoplasm and intraductal papillary mucinous neoplasms have a high malignant potential, it is important to differentiate between the various pancreatic cystic lesions. Several imaging techniques and tumour markers have been evaluated. Nonetheless, definitive guidelines to differentiate between serous cystic neoplasms, mucinous cystic neoplasms and intraductal papillary mucinous neoplasms are still poorly defined. A number of management issues regarding these neoplasms are still under debate, for example which imaging technique to use, differentiation between malignant or benign lesions and the preferred treatment modality for each pancreatic cystic neoplasm. Further research may lead to a definitive guideline for the diagnosis and treatment of mucinous cystic neoplasms, intraductal papillary mucinous neoplasms and serous cystic neoplasms.

  7. Multilocular Cystic Renal Cell Carcinoma or Cystic Nephroma?

    PubMed Central

    Cortez-Betancourt, Roberto; Alías-Melgar, Alejandro; Botello-Gómez, Pedro Jair; Ramírez-Garduño, Emilio; Trujillo-Vázquez, Eric Iván; Torres-Santos, Yosimart; Mata-Martínez, José Antonio; Carreño- de la Rosa, Fernando

    2016-01-01

    The incidence of Multilocular cystic renal cell carcinoma (MCRCC) in literature is very low and confounding MCRCC with cystic nephroma (CN) is even more unusual. The aim of this report is to present a case of MCRCC and emphasize the importance of the preoperative radiologic evaluation and immunohistochemical staining confirmation to obtain an accurate diagnosis. A 73-year-old woman presented with a history of 4-month right flank pain. CT showed a Bosniak type III renal mass. After laparoscopic partial nephrectomy the initial report was cystic nephroma. Immunohistochemical staining was performed being positive for Epithelial Membrane Antigen thus changing the diagnosis to MCRCC. Multilocular cystic renal cell carcinoma cannot reliably be distinguished from cystic nephroma neither by physical examination nor by radiologic evaluation; immunohistochemical staining assay is useful to differentiate between these conditions allowing an accurate diagnosis and proper follow-up. PMID:28074169

  8. Robot-assisted excision of cervical cystic hygroma through a retroauricular hairline approach: a case report.

    PubMed

    Lin, Frank Cheau-Feng; Yang, Tsung-Lin; Tung, Min-Che; Tsai, Stella Chin-Shaw

    2016-06-09

    Cystic hygroma is a rare benign abnormality of the lymphatic system generally occurring in young children less than 2 years old. The standard transcervical surgical treatment of cystic hygroma may often leave a permanent scar in the neck region. We report a case of cystic hygroma in a 19-month-old Asian baby girl successfully treated with robot-assisted excision through a hairline neck-lift approach. We present the use of the Yang's retractor as an instrumental advancement to this surgical approach. Treatment options for cystic hygroma may be surgical or nonsurgical. We report a case of cystic hygroma in a 19-month-old child successfully treated with robot-assisted excision through a small concealed retroauricular hairline approach. This is the first report in the medical literature of treating cystic hygroma with a minimally invasive robot-assisted excision via a small, concealed, hairline incision.

  9. Current perspectives on pancreatic serous cystic neoplasms: Diagnosis, management and beyond

    PubMed Central

    Zhang, Xiao-Peng; Yu, Zhong-Xun; Zhao, Yu-Pei; Dai, Meng-Hua

    2016-01-01

    Pancreatic cystic neoplasms have been increasingly recognized recently. Comprising about 16% of all resected pancreatic cystic neoplasms, serous cystic neoplasms are uncommon benign lesions that are usually asymptomatic and found incidentally. Despite overall low risk of malignancy, these pancreatic cysts still generate anxiety, leading to intensive medical investigations with considerable financial cost to health care systems. This review discusses the general background of serous cystic neoplasms, including epidemiology and clinical characteristics, and provides an updated overview of diagnostic approaches based on clinical features, relevant imaging studies and new findings that are being discovered pertaining to diagnostic evaluation. We also concisely discuss and propose management strategies for better quality of life. PMID:27022447

  10. Cystic Fibrosis: Diet and Nutrition

    MedlinePlus

    ... Getting an X-ray Cystic Fibrosis: Diet and Nutrition KidsHealth > For Kids > Cystic Fibrosis: Diet and Nutrition Print A A A What's in this article? ... is someone who knows all about food and nutrition. Each kid is different, but most kids with ...

  11. Cystic Fibrosis: Diet and Nutrition

    MedlinePlus

    ... in the Operating Room? Cystic Fibrosis: Diet and Nutrition KidsHealth > For Kids > Cystic Fibrosis: Diet and Nutrition A A A What's in this article? CF ... is someone who knows all about food and nutrition. Each kid is different, but most kids with ...

  12. Stride search: A general algorithm for storm detection in high-resolution climate data

    DOE PAGES

    Bosler, Peter A.; Roesler, Erika L.; Taylor, Mark A.; ...

    2016-04-13

    This study discusses the problem of identifying extreme climate events such as intense storms within large climate data sets. The basic storm detection algorithm is reviewed, which splits the problem into two parts: a spatial search followed by a temporal correlation problem. Two specific implementations of the spatial search algorithm are compared: the commonly used grid point search algorithm is reviewed, and a new algorithm called Stride Search is introduced. The Stride Search algorithm is defined independently of the spatial discretization associated with a particular data set. Results from the two algorithms are compared for the application of tropical cyclonemore » detection, and shown to produce similar results for the same set of storm identification criteria. Differences between the two algorithms arise for some storms due to their different definition of search regions in physical space. The physical space associated with each Stride Search region is constant, regardless of data resolution or latitude, and Stride Search is therefore capable of searching all regions of the globe in the same manner. Stride Search's ability to search high latitudes is demonstrated for the case of polar low detection. Wall clock time required for Stride Search is shown to be smaller than a grid point search of the same data, and the relative speed up associated with Stride Search increases as resolution increases.« less

  13. Stride Search: a general algorithm for storm detection in high-resolution climate data

    NASA Astrophysics Data System (ADS)

    Bosler, Peter A.; Roesler, Erika L.; Taylor, Mark A.; Mundt, Miranda R.

    2016-04-01

    This article discusses the problem of identifying extreme climate events such as intense storms within large climate data sets. The basic storm detection algorithm is reviewed, which splits the problem into two parts: a spatial search followed by a temporal correlation problem. Two specific implementations of the spatial search algorithm are compared: the commonly used grid point search algorithm is reviewed, and a new algorithm called Stride Search is introduced. The Stride Search algorithm is defined independently of the spatial discretization associated with a particular data set. Results from the two algorithms are compared for the application of tropical cyclone detection, and shown to produce similar results for the same set of storm identification criteria. Differences between the two algorithms arise for some storms due to their different definition of search regions in physical space. The physical space associated with each Stride Search region is constant, regardless of data resolution or latitude, and Stride Search is therefore capable of searching all regions of the globe in the same manner. Stride Search's ability to search high latitudes is demonstrated for the case of polar low detection. Wall clock time required for Stride Search is shown to be smaller than a grid point search of the same data, and the relative speed up associated with Stride Search increases as resolution increases.

  14. Effect of AMOC collapse on ENSO in a high resolution general circulation model

    NASA Astrophysics Data System (ADS)

    Williamson, Mark S.; Collins, Mat; Drijfhout, Sybren S.; Kahana, Ron; Mecking, Jennifer V.; Lenton, Timothy M.

    2017-06-01

    We look at changes in the El Niño Southern Oscillation (ENSO) in a high-resolution eddy-permitting climate model experiment in which the Atlantic Meridional Circulation (AMOC) is switched off using freshwater hosing. The ENSO mode is shifted eastward and its period becomes longer and more regular when the AMOC is off. The eastward shift can be attributed to an anomalous eastern Ekman transport in the mean equatorial Pacific ocean state. Convergence of this transport deepens the thermocline in the eastern tropical Pacific and increases the temperature anomaly relaxation time, causing increased ENSO period. The anomalous Ekman transport is caused by a surface northerly wind anomaly in response to the meridional sea surface temperature dipole that results from switching the AMOC off. In contrast to a previous study with an earlier version of the model, which showed an increase in ENSO amplitude in an AMOC off experiment, here the amplitude remains the same as in the AMOC on control state. We attribute this difference to variations in the response of decreased stochastic forcing in the different models, which competes with the reduced damping of temperature anomalies. In the new high-resolution model, these effects approximately cancel resulting in no change in amplitude.

  15. Stride search: A general algorithm for storm detection in high-resolution climate data

    SciTech Connect

    Bosler, Peter A.; Roesler, Erika L.; Taylor, Mark A.; Mundt, Miranda R.

    2016-04-13

    This study discusses the problem of identifying extreme climate events such as intense storms within large climate data sets. The basic storm detection algorithm is reviewed, which splits the problem into two parts: a spatial search followed by a temporal correlation problem. Two specific implementations of the spatial search algorithm are compared: the commonly used grid point search algorithm is reviewed, and a new algorithm called Stride Search is introduced. The Stride Search algorithm is defined independently of the spatial discretization associated with a particular data set. Results from the two algorithms are compared for the application of tropical cyclone detection, and shown to produce similar results for the same set of storm identification criteria. Differences between the two algorithms arise for some storms due to their different definition of search regions in physical space. The physical space associated with each Stride Search region is constant, regardless of data resolution or latitude, and Stride Search is therefore capable of searching all regions of the globe in the same manner. Stride Search's ability to search high latitudes is demonstrated for the case of polar low detection. Wall clock time required for Stride Search is shown to be smaller than a grid point search of the same data, and the relative speed up associated with Stride Search increases as resolution increases.

  16. Respiratory infections in patients with cystic fibrosis undergoing lung transplantation.

    PubMed

    Lobo, Leonard J; Noone, Peadar G

    2014-01-01

    Cystic fibrosis is an inherited disease characterised by chronic respiratory infections associated with bronchiectasis. Lung transplantation has helped to extend the lives of patients with cystic fibrosis who have advanced lung disease. However, persistent, recurrent, and newly acquired infections can be problematic. Classic cystic fibrosis-associated organisms, such as Staphylococcus aureus and Pseudomonas aeruginosa, are generally manageable post-transplantation, and are associated with favourable outcomes. Burkholderia cenocepacia poses particular challenges, although other Burkholderia species are less problematic. Despite concerns about non-tuberculous mycobacteria, especially Mycobacterium abscessus, post-transplantation survival has not been definitively shown to be less than average in patients with these infections. Fungal species can be prevalent before and after transplantation and are associated with high morbidity, so should be treated aggressively. Appropriate viral screening and antiviral prophylaxis are necessary to prevent infection with and reactivation of Epstein-Barr virus and cytomegalovirus and their associated complications. Awareness of drug pharmacokinetics and interactions in cystic fibrosis is crucial to prevent toxic effects and subtherapeutic or supratherapeutic drug dosing. With the large range of potential infectious organisms in patients with cystic fibrosis, infection control in hospital and outpatient settings is important. Despite its complexity, lung transplantation in the cystic fibrosis population is safe, with good outcomes if the clinician is aware of all the potential pathogens and remains vigilant by means of surveillance and proactive treatment.

  17. Management of the Upper Airway in Cystic Fibrosis

    PubMed Central

    Illing, Elisa A.; Woodworth, Bradford A.

    2015-01-01

    Purpose of Review Upper airway disease engenders significant morbidity for patients with cystic fibrosis and is increasingly recognized as having a much greater role in pulmonary outcomes and quality of life than originally believed. Widespread disparate therapeutic strategies for cystic fibrosis chronic rhinosinusitis underscore the absence of a standardized treatment paradigm. This review outlines the most recent evidence-based trends in the management of upper airway disease in cystic fibrosis. Recent Findings The unified airway theory proposes that the sinuses are a focus of initial bacterial colonization which seeds the lower airway and may play a large role in maintaining lung infections. Mounting evidence suggests more aggressive treatment of the sinuses may confer significant improvement in pulmonary disease and quality of life outcomes in cystic fibrosis patients. However, there is a lack of high-level evidence regarding medical and surgical management of cystic fibrosis chronic rhinosinusitis that makes generalizations difficult. Summary Well designed clinical trials with long-term follow-up concerning medical and surgical interventions for cystic fibrosis sinus disease are required to establish standardized treatment protocols, but increased interest in the sinuses as a bacterial reservoir for pulmonary infections has generated considerable attention. PMID:25250804

  18. Two buffer PAGE system-based SSCP/HD analysis: a general protocol for rapid and sensitive mutation screening in cystic fibrosis and any other human genetic disease.

    PubMed

    Liechti-Gallati, S; Schneider, V; Neeser, D; Kraemer, R

    1999-07-01

    The large size of many disease genes and the multiplicity of mutations complicate the design of an adequate assay for the identification of disease-causing variants. One of the most successful methods for mutation detection is the single strand conformation polymorphism (SSCP) technique. By varying temperature, gel composition, ionic strength and additives, we optimised the sensitivity of SSCP for all 27 exons of the CFTR gene. Using simultaneously SSCP and heteroduplex (HD) analysis, a total of 80 known CF mutations (28 missense, 22 frameshift, 17 nonsense, 13 splicesite) and 20 polymorphisms was analysed resulting in a detection rate of 97.5% including the 24 most common mutations worldwide. The ability of this technique to detect mutations independent of their nature, frequency, and population specificity was confirmed by the identification of five novel mutations (420del9, 1199delG, R560S, A613T, T1299I) in Swiss CF patients, as well as by the detection of 41 different mutations in 198 patients experimentally analysed. We present a three-stage screening strategy allowing analysis of seven exons within 5 hours and analysis of the entire coding region within 1 week, including sequence analysis of the variants. Additionally, our protocol represents a general model for point mutation analysis in other genetic disorders and has already been successfully established for OTC deficiency, collagene deficiency, X-linked myotubular myopathy (XLMTM), Duchenne and Becker muscular dystrophy (DMD, BMD), Wilson disease (WD), Neurofibromatosis I and II, Charcot-Marie-Tooth disease, hereditary neuropathy with liability to pressure palsies, and defects in mitochondrial DNA. No other protocol published so far presents standard SSCP/HD conditions for mutation screening in different disease genes.

  19. [Cystic fibrosis in 2008].

    PubMed

    Durieu, I; Josserand, R Nove

    2008-11-01

    To describe the epidemiological, physiopathological, clinical and therapeutic knowledge concerning cystic fibrosis (CF). Important modifications in the health organization of the care concerning this orphan disease have been implemented in France. The life expectancy has dramatically increased, as well as the knowledge concerning the pathological structure and function of the CFTR gene and protein. This will lead to the development of emerging drug treatments for this lethal disease. The life expectancy is predicted to exceed 40 years for children born in the 2000s. As a result, there has been a tremendous growth of the adult population that reached 40% of the overall approximately 5000 patients included in the CF French registry (Observatoire National de la Mucoviscidose). Lung disease remains the primary cause of morbidity and mortality. The characteristic phenotypic presentation associates bronchial and rhinosinusal symptoms, pancreatic insufficiency and liver disease. Bronchial damage leads to progressive chronic respiratory insufficiency. Diabetes mellitus and osteoporosis frequently appears in adulthood. Neonatal screening has been implemented in France since 2002. It will prevent delayed diagnosis and its deleterious consequences. Some atypical cases of CF presenting only with one or two organ system involvement can be diagnosed in adulthood. Isolated chronic rhinosinusitis, bronchiectasis, congenital bilateral absence of vas deferens, recurrent pancreatitis, allergic bronchopulmonary aspergillosis, and some case of cholangitis may so revealed late form of cystic fibrosis. The health care is organized in cystic fibrosis centres. Despite gene discovery, treatment still remains symptomatic, based on intensive pulmonary and nutritional treatments. Challenges for new treatments are to correct the basic defect, either by gene therapy or by pharmacological modulation of the abnormal physiological processes.

  20. Screening for cystic fibrosis.

    PubMed Central

    Dodge, J A; Ryley, H C

    1982-01-01

    Practicable methods are now available for whole population screening of neonates for cystic fibrosis. Although diagnosis and treatment of the disease from birth has not yet been unequivocally shown to improve prognosis, existing evidence suggests that this is likely. Further ethical reasons are proposed in support of neonatal diagnosis and early treatment. The development of tests for prenatal diagnosis and carrier detection is under active investigation, and raises ethical problems for heterozygotes and their medical advisers. The heavy financial and emotional burden this disease imposes on the patient and the family should not be underestimated when policy decisions are made. PMID:7138066

  1. Cystic fibrosis and sleep.

    PubMed

    Katz, Eliot S

    2014-09-01

    Sleep disturbances are frequently observed in cystic fibrosis (CF). The resultant sleep fragmentation, short sleep duration, and gas-exchange abnormalities are postulated to contribute to the neurocognitive, cardiovascular, and metabolic abnormalities associated with CF. There are no outcomes data to establish the optimal procedure for screening and treating CF patients for sleep-related respiratory abnormalities. Therapy with supplemental oxygen and bilevel ventilation are widely considered to be effective in the short term, but there are few evidence-based data to support long-term improvements in morbidity and mortality. Copyright © 2014 Elsevier Inc. All rights reserved.

  2. Correcting circulation biases in a lower-resolution global general circulation model with data assimilation

    NASA Astrophysics Data System (ADS)

    Canter, Martin; Barth, Alexander; Beckers, Jean-Marie

    2017-02-01

    In this study, we aim at developing a new method of bias correction using data assimilation. This method is based on the stochastic forcing of a model to correct bias by directly adding an additional source term into the model equations. This method is presented and tested first with a twin experiment on a fully controlled Lorenz '96 model. It is then applied to the lower-resolution global circulation NEMO-LIM2 model, with both a twin experiment and a real case experiment. Sea surface height observations are used to create a forcing to correct the poorly located and estimated currents. Validation is then performed throughout the use of other variables such as sea surface temperature and salinity. Results show that the method is able to consistently correct part of the model bias. The bias correction term is presented and is consistent with the limitations of the global circulation model causing bias on the oceanic currents.

  3. Correcting circulation biases in a lower-resolution global general circulation model with data assimilation

    NASA Astrophysics Data System (ADS)

    Canter, Martin; Barth, Alexander; Beckers, Jean-Marie

    2016-12-01

    In this study, we aim at developing a new method of bias correction using data assimilation. This method is based on the stochastic forcing of a model to correct bias by directly adding an additional source term into the model equations. This method is presented and tested first with a twin experiment on a fully controlled Lorenz '96 model. It is then applied to the lower-resolution global circulation NEMO-LIM2 model, with both a twin experiment and a real case experiment. Sea surface height observations are used to create a forcing to correct the poorly located and estimated currents. Validation is then performed throughout the use of other variables such as sea surface temperature and salinity. Results show that the method is able to consistently correct part of the model bias. The bias correction term is presented and is consistent with the limitations of the global circulation model causing bias on the oceanic currents.

  4. Excision methods for high resolution shock capturing schemes applied to general relativistic hydrodynamics

    SciTech Connect

    Hawke, Ian; Loeffler, Frank; Nerozzi, Andrea

    2005-05-15

    We present a simple method for applying excision boundary conditions for the relativistic Euler equations. This method depends on the use of reconstruction-evolution methods, a standard class of high-resolution shock-capturing methods. We test three different reconstruction schemes, namely, total variation diminishing, piecewise parabolic method (PPM) and essentially nonoscillatory. The method does not require that the coordinate system is adapted to the excision boundary. We demonstrate the effectiveness of our method using tests containing discontinuities, static test fluid solutions with black holes, and full dynamical collapse of a neutron star to a black hole. A modified PPM scheme is introduced because of problems arisen when matching excision with the original PPM reconstruction scheme.

  5. Fetal MRI as Complementary Study of Congenital Cystic Adenomatoid Malformation During Pregnancy: A Single Case Report

    PubMed Central

    Miranda-Paanakker, Alberto; Gomez-Leal, Paloma; Navarro-Sanchez, Patricia; Bueno-Crespo, Andres; Martinez-Cendan, Juan Pedro; Remezal-Solano, Manuel

    2016-01-01

    Fetal lung masses are rare findings in prenatal ultrasound scanning in general population, of which congenital cystic adenomatoid malformation is the most commonly diagnosed type. This paper reports a single case of congenital cystic adenomatoid malformation detected at our hospital and the subsequent clinical follow-up using ultrasound scanning and fetal magnetic resonance imaging. PMID:27186452

  6. First-trimester septated cystic hygroma and cavum velum interpositum cyst.

    PubMed

    Sherer, David M; Dalloul, Mudar; Miller, Michelle J; Kheyman, Mila; Zinn, Harry L; Abulafia, Ovadia

    2011-07-01

    First-trimester septated cystic hygroma, frequently noted during general obstetric first-trimester screening, is strongly associated with fetal aneuploidy and structural anomalies and is considered an ominous finding. We present the case of a fetus with a first-trimester septated cystic hygroma and cavum velum interpositum cyst.

  7. High-resolution interpolation of climate scenarios for Canada derived from general circulation model simulations

    Treesearch

    D. T. Price; D. W. McKenney; L. A. Joyce; R. M. Siltanen; P. Papadopol; K. Lawrence

    2011-01-01

    Projections of future climate were selected for four well-established general circulation models (GCMs) forced by each of three greenhouse gas (GHG) emissions scenarios recommended by the Intergovernmental Panel on Climate Change (IPCC), namely scenarios A2, A1B, and B1 of the IPCC Special Report on Emissions Scenarios. Monthly data for the period 1961-2100 were...

  8. Atomic Structure of the Cystic Fibrosis Transmembrane Conductance Regulator.

    PubMed

    Zhang, Zhe; Chen, Jue

    2016-12-01

    The cystic fibrosis transmembrane conductance regulator (CFTR) is an anion channel evolved from the ATP-binding cassette (ABC) transporter family. In this study, we determined the structure of zebrafish CFTR in the absence of ATP by electron cryo-microscopy to 3.7 Å resolution. Human and zebrafish CFTR share 55% sequence identity, and 42 of the 46 cystic-fibrosis-causing missense mutational sites are identical. In CFTR, we observe a large anion conduction pathway lined by numerous positively charged residues. A single gate near the extracellular surface closes the channel. The regulatory domain, dephosphorylated, is located in the intracellular opening between the two nucleotide-binding domains (NBDs), preventing NBD dimerization and channel opening. The structure also reveals why many cystic-fibrosis-causing mutations would lead to defects either in folding, ion conduction, or gating and suggests new avenues for therapeutic intervention. Copyright © 2016 Elsevier Inc. All rights reserved.

  9. Cystic fibrosis since 1938.

    PubMed

    Davis, Pamela B

    2006-03-01

    Cystic fibrosis (CF) was distinguished from celiac disease in 1938. Then, it was a pathologic diagnosis, life expectancy was approximately 6 months, and the autosomal recessive disease was believed to arise from abnormal mucus plugging exocrine ducts. Death often occurred from lung infection. Discovery of the sweat electrolyte defect in 1953 and standardization of the sweat test in 1959 allowed identification of milder cases, and CF was no longer considered only a disorder of mucus. In 1955, establishment of centers with programs of aggressive, comprehensive care initiated striking improvement in longevity. The pillars of care established then (attention to nutrition, airway clearance, treatment of lung infection) remain today. In 1983, chloride transport was identified as the basic physiologic CF defect, accompanied by increased sodium reabsorption. In 1980, we learned that inflammation contributes independently to lung disease and constitutes an independent therapeutic target. In 1989, the discovery of the CF gene demonstrated the basic defect to be in a cAMP-regulated chloride channel. This afforded new diagnostic tests, opportunities for research, and prospects for using the gene as therapy. Since then, substantial advances in basic and clinical research catalyzed therapeutic improvements: median survival age now exceeds 30 years. The Cystic Fibrosis Foundation center network provides not only opportunity to conduct clinical trials but also means to disseminate new therapies. In the future, treatments directed at the basic defect can be expected, with concomitant improvements in morbidity and mortality.

  10. Cystic fibrosis in Uruguay.

    PubMed

    Luzardo, Gerardo; Aznarez, Isabel; Crispino, Beatriz; Mimbacas, Adriana; Martínez, Liria; Poggio, Rossana; Zielenski, Julian; Tsui, Lap-Chee; Cardoso, Horacio

    2002-03-31

    We conducted clinical and genetic analyses of 52 cystic fibrosis (CF) patients in Uruguay, which is about half of the known affected individuals in the country. A relatively high proportion had a mild presentation, characterized by pancreatic sufficiency (28%), a strong pulmonary component (97%), and borderline sweat electrolyte measurements (25%). Mutational analysis of CF chromosomes demonstrated a relatively low incidence of the DeltaF508 allele (40%) and a large number of other cystic fibrosis conductance regulator mutations, with an overall detection rate of about 71%. Fifteen different mutations were detected in our patients: DeltaF508, G542X, R1162X, G85E, N1303K, R334W, R75Q, R74W, D1270N, W1282X, DeltaI507, 2789+5G-->A, R1066C, -816C/T, R553X, as well as RNA splicing variant IVS8-5T. This group of Uruguayan CF patients has some characteristics in common with other populations of similar origin (Hispanics), as well as some unique characteristics.

  11. Cystic fibrosis related diabetes.

    PubMed

    O'Shea, Donal; O'Connell, Jean

    2014-08-01

    Improved life expectancy in cystic fibrosis (CF) has led to an expanding population of adults with CF, now representing almost 50 % of the total CF population. This creates new challenges from long-term complications such as diabetes mellitus (DM), a condition that is present in 40 %-50 % of adults with CF. Cystic fibrosis-related diabetes (CFRD) results from a primary defect of insulin deficiency and although sharing features with type 1 (DM1) and type 2 diabetes (DM2), it is a clinically distinct condition. Progression to diabetes is associated with poorer CF clinical outcomes and increased mortality. CFRD is not associated with an increased risk of cardiovascular disease and the prevalence of microvascular complications is lower than DM1 or DM2. Rather, the primary goal of insulin therapy is the preservation of lung function and optimization of nutritional status. There is increasing evidence that appropriate screening and early intervention with insulin can reverse weight loss and improve pulmonary function. This approach may include targeting postprandial hyperglycemia not detected by standard diagnostic tests such as the oral glucose tolerance test. Further clinical research is required to guide when and how much to intervene in patients who are already dealing with the burden of one chronic illness.

  12. Different manifestations of calcifying cystic odontogenic tumor.

    PubMed

    Utumi, Estevam Rubens; Pedron, Irineu Gregnanin; da Silva, Leopoldo Penteado Nucci; Machado, Gustavo Grothe; Rocha, André Caroli

    2012-01-01

    The calcifying cystic odontogenic tumor normally presents as a painless, slow-growing mass, involving both maxilla and mandible, primarily the anterior segment (incisor/canine area). It generally affects young adults in the third to fourth decades, with no gender predilection. Computerized tomography images revealed important characteristics that were not detected by panoramic radiography, such as fenestration, calcification and tooth-like structures. The typical microscopic feature of this lesion is the presence of variable amounts of aberrant epithelial cells, without nuclei, which are named "ghost cells". In addition, dysplastic dentine can be found and occasionally the cyst can be associated with an area of dental hard tissue formation resembling an odontoma. The treatment for calcifying cystic odontogenic tumor involves simple enucleation and curettage. The purpose of this article is to present two different manifestation of calcifying cystic odontogenic tumor in which computerized tomography, associated to clinical features, served as an important tool for diagnosis, adequate surgical planning and follow-up of patients.

  13. [Large cystic neurinoma: a case report].

    PubMed

    Miyagi, A; Maeda, K; Sugawara, T

    1997-07-01

    We report a case of large cystic acoustic neurinoma. A 52-year-old male was admitted to hospital with a history of progressive dysphagia, gait disturbance and diplopia for 2 months. On admission, neurological examinations revealed Bruns' type nystagmus to the left side, hypesthesia in the distribution of the second and third divisions of the left trigeminal nerve, and partial paresis of cranial nerves IX, X, and XII on the left side, and truncal ataxia. A pure-tone threshold audiogram indicated the presence of 32 dB hearing loss in the left ear. Speech discrimination was 80%. Caloric vestibular responses were absent on the left side. Skull radiographs with polytomographs of the internal auditory canal (IAC) were normal. Bony changes in the IAC were not found by high-resolution bone-window computed tomography (CT) scan. A plain CT scan revealed a large low-attenuated cystic mass in the left cerebellopontine angle (CPA), which was associated with displacement of the fourth ventricle. An enhanced CT scan demonstrated a thin rim-enhancement in the cyst wall. Magnetic resonance imaging (MRI) scans disclosed a large rim-enhanced cystic mass extending superiorly into the tentorial incisura and inferiorly into the foramen magnum. At surgery via a left suboccipital approach, a large cystic mass was found at the left CPA arising from the VIIIth nerve, and compressing the Vth, VIth, VIIth and lower cranial nerves. The cyst was filled with a xanthochromic fluid and was firmly attached to the internal auditory meatus (IAM). However no tumor extension into the IAM was confirmed. The tumor was excised completely. The postoperative course was uneventful, except for impairment of the VIIth and VIIIth nerves. At 6 months after the first operation, the facial nerve had improved up to grade III (Hause-Brackmann stage). Histological examinations revealed a typical benign acoustic neurinoma with predominant representation of Antoni B tissues. The cyst wall contained numerous abnormal

  14. Cystic tumors of the pancreas

    PubMed Central

    Morana, Giovanni; Guarise, Alessandro

    2006-01-01

    Cystic tumors of the pancreas are less frequent than solid lesions and are often detected incidentally, as many of these lesions are small and asymptomatic. However, they may be associated with pancreatitis or have malignant potential. With advancements in diagnostic imaging, cystic lesions of the pancreas are being detected with increasing frequency. Many lesions can cause a pancreatic cyst, most being non-neoplastic while approximately 10% are cystic tumors, ranging from benign to highly malignant tumors. With increasing experience it is becoming clear that the prevalence of pseudocyst among cystic lesions of the pancreas is lower than usually presumed. A presumptive diagnosis of pseudocyst based on imaging appearance alone can cause a diagnostic error, and neoplastic cysts of the pancreas are particularly susceptible to this misdiagnosis, which can result in inappropriate treatment. Cystic tumors of the pancreas are formed by serous or mucinous structures showing all stages of cellular differentiation. According to the WHO classification, they can be subdivided on the basis of their histological type and biological behavior into benign tumors, borderline tumors, and malignant tumors. Cystic pancreatic tumors can be subdivided into peripheral (serous cystadenomas, mucinous cystic tumors, solid and papillary epithelial neoplasms, cystic islet cell tumors), which do not communicate with the main pancreatic duct, and ductal tumors (mucinous tumor), according to their site of origin. On the basis of imaging criteria alone, it can be very difficult to differentiate non-tumoral cystic lesions from neoplastic ones. The management of these patients is complex, and it is important to correlate imaging findings with knowledge of the patient’s symptoms and of the natural history and predictors of malignancy in pancreatic cysts. PMID:16861136

  15. Gastrointestinal Manifestations of Cystic Fibrosis

    PubMed Central

    2016-01-01

    Cystic fibrosis has historically been considered a pulmonary disease, but with the increasing life expectancy of these patients, gastrointestinal manifestations are becoming more important. Furthermore, nutritional status is closely linked to pulmonary function and, thus, overall mortality. This article discusses gastrointestinal manifestations (which involve nutritional, pancreatic, hepatobiliary, and, in particular, gastrointestinal tract issues) of cystic fibrosis as well as management of the disease. In addition, the article discusses studies that have been critical to our understanding of gastrointestinal manifestations of cystic fibrosis. PMID:27330503

  16. Nutritional Issues in Cystic Fibrosis.

    PubMed

    Solomon, Missale; Bozic, Molly; Mascarenhas, Maria R

    2016-03-01

    The importance of maintaining adequate nutrition in patients with cystic fibrosis has been well known for the past 3 decades. Achieving normal growth and maintaining optimal nutrition is associated with improved lung function. Comprehensive and consistent nutritional assessments at regular intervals can identify those at risk of nutritional failure and uncover micronutrient deficiencies contributing to malnutrition. Management of malnutrition in cystic fibrosis should follow a stepwise approach to determine the causes and comorbidities and to develop a nutritional plan. Nutritional management is crucial at every stage in a person's life with cystic fibrosis and remains a cornerstone of management.

  17. A bizarre abdominal cystic lesion.

    PubMed

    Zucchini, Giorgia; Pezzilli, Raffaele; Ricci, Claudio; Casadei, Riccardo; Santini, Donatella; Calculli, Lucia; Corinaldesi, Roberto

    2010-09-06

    In spite of careful intraoperative precautions and gauze counts, mistakes can still occur during surgery. In the case reported, a retained gauze leaved during a surgical approach for removing a solid-cystic papillary tumor localized in the pancreatic tail, caused both persistent abdominal discomfort and the presence of an abdominal cystic lesion at imaging techniques. When a previous operative history is present, a foreign body should be taken into account in the differential diagnosis of a patient with an intra-abdominal cystic mass. Finally, radio-opaque marker should be routinely used by surgeons in order to reach a correct diagnosis in operated patients having retained gauze.

  18. Pneumothorax in cystic fibrosis

    PubMed Central

    Kioumis, Ioannis P.; Zarogoulidis, Konstantinos; Huang, Haidong; Li, Qiang; Dryllis, Georgios; Pitsiou, Georgia; Machairiotis, Nikolaos; Katsikogiannis, Nikolaos; Papaiwannou, Antonis; Lampaki, Sofia; Porpodis, Konstantinos; Zaric, Bojan; Branislav, Perin; Mpoukovinas, Ioannis; Lazaridis, George

    2014-01-01

    Pneumothorax is recognized as a common and life-threatening complication in cystic fibrosis (CF) patients, especially in those who are infected with P. aeruginosa, B. cepacia or Aspergillus, need enteral feeding, are diagnosed as suffering from allergic bronchopulmonary aspergillosis (ABPA), developed massive hemoptysis, and their respiratory function is seriously compromised. Structural impairment and altered airflow dynamics in the lungs of CF patients are considered as the main predisposing factors, but also inhaled medications and non-invasive positive pressure ventilation (NIPPV) could increase the risk of pneumothorax. Clinical presentation could range from dramatic to very mild. Management of spontaneous pneumothorax occurring to patients with CF is essentially similar to that for non-CF patients. Therapeutic options include intercostal tube drainage, video-assisted thoracoscopic surgery (VATS), and medical or surgical pleurodesis. Pneumothorax increases both short- and long-term morbidity and mortality in CF patients and causes significant deterioration of their quality of life. PMID:25337406

  19. Simulation of the Small-Scale Dust Activities and Their Mutual Interactions on the Atmospheric Dynamics Using a High-Resolution Mars General Circulation Model

    NASA Astrophysics Data System (ADS)

    Kuroda, T.; Kadowaki, M.

    2017-06-01

    We show the simulation results of our high-resolution Mars general circulation model including the dust lifting processes for the investigations of the meteorological features which invoke dust storms and subsequent enhancement of small-scale waves.

  20. Cystic fibrosis in pregnancy.

    PubMed Central

    Kent, N E; Farquharson, D F

    1993-01-01

    OBJECTIVE: To review the outcomes of pregnancies in women with cystic fibrosis (CF) and to address issues pertinent to the obstetric care of such women. DATA SOURCES: English-language case reports and case series published from 1960 to 1991 identified through a search of MEDLINE and Index Medicus. The terms of reference were "cystic fibrosis" and "pregnancy". Not all the reports reviewed addressed all the outcomes under consideration. STUDY SELECTION: A total of 20 reports citing cases of pregnancy in women with CF. DATA EXTRACTION: Outcomes included the number of spontaneous abortions, pregnancies continued beyond 20 weeks, preterm deliveries, maternal deaths at 6 months and 2 years after delivery and perinatal deaths. Breast-feeding was addressed. Measures to assess the severity of maternal disease included the mean age at diagnosis of CF, weight gain during pregnancy, pulmonary function studies if available and the need for pancreatic enzyme replacement therapy. DATA SYNTHESIS: Of 217 pregnancies in 162 women spontaneous abortion occurred in 10 (4.6%). Pregnancy progressed beyond 20 weeks in 81.6% of cases; 24.3% of the deliveries were preterm. The maternal death rate did not exceed that among age-related women with CF who were not pregnant. The rate of perinatal death was 7.9%. Breast milk was not hypernatremic. Poor outcomes were associated with a weight gain of less than 4.5 kg and a forced vital capacity of less than 50% of the predicted value. CONCLUSIONS: Premature labour and delivery remain a significant risk for pregnant women with CF, contributing to a high rate of perinatal death. Maternal illness and death result from deteriorating pulmonary function. Breast-feeding is not contraindicated. Attention to energy intake and pulmonary function is important. PMID:8374843

  1. Molecular Diagnosis of Cystic Fibrosis.

    PubMed

    Deignan, Joshua L; Grody, Wayne W

    2016-01-01

    This unit describes a recommended approach to identifying causal genetic variants in an individual suspected of having cystic fibrosis. An introduction to the genetics and clinical presentation of cystic fibrosis is initially presented, followed by a description of the two main strategies used in the molecular diagnosis of cystic fibrosis: (1) an initial targeted variant panel used to detect only the most common cystic fibrosis-causing variants in the CFTR gene, and (2) sequencing of the entire coding region of the CFTR gene to detect additional rare causal CFTR variants. Finally, the unit concludes with a discussion regarding the analytic and clinical validity of these approaches. Copyright © 2016 John Wiley & Sons, Inc.

  2. Haemophilus infection in cystic fibrosis.

    PubMed Central

    Rayner, R J; Hiller, E J; Ispahani, P; Baker, M

    1990-01-01

    Twenty seven patients with cystic fibrosis under the age of 12 years and 27 matched patients with asthma were followed up in a prospective study for one year. The isolation rate of non-capsulated strains of Haemophilus influenzae from cough swabs and sputum specimens taken at routine clinic visits every two months was significantly greater in cystic fibrosis than in asthma. Haemophilus para-influenzae was equally common in both groups. During exacerbations the isolation rate of H influenzae in cystic fibrosis was significantly greater than at other times, whereas in asthma there was no significant difference. The distribution of biotypes of H influenzae and H parainfluenzae was similar in the two groups. In cystic fibrosis, biotype I was associated with exacerbations. Biotype V was more common than in previous studies, but was not associated with exacerbations. PMID:2185699

  3. [Lung physiotherapy in cystic fibrosis].

    PubMed

    Gursli, S; Haanaes, O C

    1991-02-28

    This article is intended as a brief practical guide for physicians and physiotherapists concerned with the treatment of cystic fibrosis. Physiotherapeutic techniques for the treatment of chest diseases have been developed and modified as advances have taken place in the medical management of cystic fibrosis. The article describes forced expiratory technique, positive expiratory pressure, postural drainage, autogenic drainage and other techniques. Patients with cystic fibrosis live longer and have a better quality of life than ever before, but progressive deterioration of lung function will always be their most serious problem. Physical activity and chest physiotherapy are essential parts of all treatment regimens for cystic fibrosis. It is important to realize that the physiotherapist is a very important member of the team which includes nurses, physicians-and the patient.

  4. Newborn screening for cystic fibrosis.

    PubMed

    Castellani, Carlo; Massie, John; Sontag, Marci; Southern, Kevin W

    2016-08-01

    Since the late 1970s when the potential of the immunoreactive trypsinogen assay for early identification of infants with cystic fibrosis was first recognised, the performance of newborn blood spot screening (NBS) has been continually assessed and its use has gradually expanded. NBS for cystic fibrosis is a cost-effective strategy and, if standards of care are fully implemented and robust management pathways are in place, has a positive effect on clinical outcomes. In the past decade, NBS has undergone rapid expansion and an unprecedented number of infants with cystic fibrosis have access to early diagnosis and care. Cystic fibrosis NBS has now moved on from the development phase and is entering an era of consolidation. In the future, research should focus on the rationalisation and optimisation of existing programmes, with particular attention to bioethical implications such as unwanted detection of carriers and inconclusive diagnoses. Copyright © 2016 Elsevier Ltd. All rights reserved.

  5. Physical training for cystic fibrosis.

    PubMed

    Bradley, J; Moran, F

    2008-01-23

    Physical training may form an important part of the care package for people with cystic fibrosis. To determine whether a prescribed regimen of physical training produces improvement or prevents deterioration in physiological and clinical outcomes in cystic fibrosis compared to no training. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register which comprises references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings. Date of the most recent search: September 2007. All randomised and quasi-randomised controlled clinical trials in which a prescribed regimen of physical training is compared to no physical training in people with cystic fibrosis. Two authors independently selected studies for inclusion, assessed methodological quality and extracted data. Of the 26 studies identified, seven studies which included 231 participants, met the inclusion criteria. This review does provide some limited evidence from both short- and long-term studies that aerobic or anaerobic physical training has a positive effect on primary outcomes (exercise capacity, strength and lung function) but improvements are not consistent between studies. Conclusions about the efficacy of physical training in cystic fibrosis are limited by the small size, short duration and incomplete reporting of most of the studies included in this review. Physical training is already part of the care package offered to most people with cystic fibrosis and there is a lack of evidence to actively discourage this. The benefits obtained from including physical training in a package of care may be influenced by the type of training programme. Further research is needed to assess comprehensively the benefits of exercise programmes in people with cystic fibrosis and the relative benefits of the addition of aerobic versus anaerobic versus a combination of both types of physical training to the care

  6. Pneumococcal vaccines for cystic fibrosis.

    PubMed

    Burgess, Laura; Southern, Kevin W

    2014-08-05

    Invasive pneumococcal disease is associated with significant mortality and many countries have introduced routine pneumococcal vaccination into their childhood immunisation programmes. Whilst pneumococcal disease in cystic fibrosis is uncommon, pneumococcal immunisation may offer some protection against pulmonary exacerbations caused by this pathogen. In the USA and UK pneumococcal vaccination is currently recommended for all children and adults with cystic fibrosis. To assess the efficacy of pneumococcal vaccines in reducing morbidity in people with cystic fibrosis. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Cystic Fibrosis Trials Register, which comprises references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings. In addition, the pharmaceutical manufacturers of the polysaccharide and conjugate pneumococcal vaccines were approached.Date of the most recent search: 15 May 2014. Randomised and quasi-randomised controlled trials comparing pneumococcal vaccination (with either a polysaccharide or conjugate pneumococcal vaccine) with non-vaccination or placebo in children or adults with cystic fibrosis were eligible for inclusion. No relevant trials were identified. There are no trials included in this review. As no trials were identified we cannot draw conclusions on the efficacy of routine pneumococcal immunisation in people with cystic fibrosis in reducing their morbidity or mortality. As many countries now include pneumococcal immunisation in their routine childhood vaccination schedule it is unlikely that future randomised controlled trials will be initiated. Rigorously conducted epidemiological studies may offer the opportunity to evaluate the efficacy of pneumococcal vaccination in reducing morbidity and mortality in people with cystic fibrosis.

  7. Pneumococcal vaccines for cystic fibrosis.

    PubMed

    Burgess, Laura; Southern, Kevin W

    2012-09-12

    Invasive pneumococcal disease is associated with significant mortality and many countries have introduced routine pneumococcal vaccination into their childhood immunisation programmes. Whilst pneumococcal disease in cystic fibrosis is uncommon, pneumococcal immunisation may offer some protection against pulmonary exacerbations caused by this pathogen. In the USA and UK pneumococcal vaccination is currently recommended for all children and adults with cystic fibrosis. To assess the efficacy of pneumococcal vaccines in reducing morbidity in people with cystic fibrosis. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Cystic Fibrosis Trials Register, which comprises references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings. In addition, the pharmaceutical manufacturers of the polysaccharide and conjugate pneumococcal vaccines were approached.Date of the most recent search: 10 July 2012. Randomised and quasi-randomised controlled trials comparing pneumococcal vaccination (with either a polysaccharide or conjugate pneumococcal vaccine) with non-vaccination or placebo in children or adults with cystic fibrosis were eligible for inclusion. No relevant trials were identified. There are no trials included in this review. As no trials were identified we cannot draw conclusions on the efficacy of routine pneumococcal immunisation in people with cystic fibrosis in reducing their morbidity or mortality. As many countries now include pneumococcal immunisation in their routine childhood vaccination schedule it is unlikely that future randomised controlled trials will be initiated. Rigorously conducted epidemiological studies may offer the opportunity to evaluate the efficacy of pneumococcal vaccination in reducing morbidity and mortality in people with cystic fibrosis.

  8. High-resolution numerical simulation of Venus atmosphere by AFES (Atmospheric general circulation model For the Earth Simulator)

    NASA Astrophysics Data System (ADS)

    Sugimoto, Norihiko; AFES project Team

    2016-10-01

    We have developed an atmospheric general circulation model (AGCM) for Venus on the basis of AFES (AGCM For the Earth Simulator) and performed a high-resolution simulation (e.g., Sugimoto et al., 2014a). The highest resolution is T639L120; 1920 times 960 horizontal grids (grid intervals are about 20 km) with 120 vertical layers (layer intervals are about 1 km). In the model, the atmosphere is dry and forced by the solar heating with the diurnal and semi-diurnal components. The infrared radiative process is simplified by adopting Newtonian cooling approximation. The temperature is relaxed to a prescribed horizontally uniform temperature distribution, in which a layer with almost neutral static stability observed in the Venus atmosphere presents. A fast zonal wind in a solid-body rotation is given as the initial state.Starting from this idealized superrotation, the model atmosphere reaches a quasi-equilibrium state within 1 Earth year and this state is stably maintained for more than 10 Earth years. The zonal-mean zonal flow with weak midlatitude jets has almost constant velocity of 120 m/s in latitudes between 45°S and 45°N at the cloud top levels, which agrees very well with observations. In the cloud layer, baroclinic waves develop continuously at midlatitudes and generate Rossby-type waves at the cloud top (Sugimoto et al., 2014b). At the polar region, warm polar vortex surrounded by a cold latitude band (cold collar) is well reproduced (Ando et al., 2016). As for horizontal kinetic energy spectra, divergent component is broadly (k > 10) larger than rotational component compared with that on Earth (Kashimura et al., in preparation). We will show recent results of the high-resolution run, e.g., small-scale gravity waves attributed to large-scale thermal tides. Sugimoto, N. et al. (2014a), Baroclinic modes in the Venus atmosphere simulated by GCM, Journal of Geophysical Research: Planets, Vol. 119, p1950-1968.Sugimoto, N. et al. (2014b), Waves in a Venus general

  9. What Are the Signs and Symptoms of Cystic Fibrosis?

    MedlinePlus

    ... Twitter. What Are the Signs and Symptoms of Cystic Fibrosis? The signs and symptoms of cystic fibrosis (CF) ... respiratory, digestive, or reproductive systems of the body. Cystic Fibrosis Figure A shows the organs that cystic fibrosis ...

  10. Eating disorders in patients with cystic fibrosis.

    PubMed

    Raymond, N C; Chang, P N; Crow, S J; Mitchell, J E; Dieperink, B S; Beck, M M; Crosby, R D; Clawson, C C; Warwick, W J

    2000-06-01

    This study was designed to examine rates of eating disorders and psychopathology in patients with cystic fibrosis (CF). Fifty-eight CF patients and 43 healthy control participants were evaluated using structured psychiatric interviews and rating scales. Two control participants and no CF patients were diagnosed with an eating disorder. Additionally, 11 CF patients were diagnosed with one or more psychiatric disorders. Group means on the rating scales did not show clinically meaningful elevations in either group. These data indicate no evidence for elevated rates of eating disorders in CF patients. Similarly, rates of other psychiatric disorders in the CF group were not greater than the prevalence reported in the general population.

  11. [Cystic fibrosis: centers and care networks organization].

    PubMed

    Bellon, G

    2012-05-01

    More than 20 years after the gene discovery, without specific treatment, the observed improvement of the cystic fibrosis prognosis appears due to management's organization as well as early diagnosis (neonatal screening) or progress in symptomatic treatment. The CF Centers (CRCM) official recognition was a necessary step before generalization of routine neonatal screening (October, 2002). Actually French CF management relies on three levels of organization: CF centers, regional care networks and French CF Society, in close relationship with patients association (Vaincre la Mucoviscidose). Copyright © 2012 Elsevier Masson SAS. All rights reserved.

  12. Quantitative high-resolution renal perfusion imaging using 3-dimensional through-time radial generalized autocalibrating partially parallel acquisition.

    PubMed

    Wright, Katherine L; Chen, Yong; Saybasili, Haris; Griswold, Mark A; Seiberlich, Nicole; Gulani, Vikas

    2014-10-01

    Dynamic contrast-enhanced (DCE) magnetic resonance imaging (MRI) examinations of the kidneys provide quantitative information on renal perfusion and filtration. However, these examinations are often difficult to implement because of respiratory motion and their need for a high spatiotemporal resolution and 3-dimensional coverage. Here, we present a free-breathing quantitative renal DCE-MRI examination acquired with a highly accelerated stack-of-stars trajectory and reconstructed with 3-dimensional (3D) through-time radial generalized autocalibrating partially parallel acquisition (GRAPPA), using half and quarter doses of gadolinium contrast. Data were acquired in 10 asymptomatic volunteers using a stack-of-stars trajectory that was undersampled in-plane by a factor of 12.6 with respect to Nyquist sampling criterion and using partial Fourier of 6/8 in the partition direction. Data had a high temporal (2.1-2.9 seconds per frame) and spatial (approximately 2.2 mm) resolution with full 3D coverage of both kidneys (350-370 mm × 79-92 mm). Images were successfully reconstructed with 3D through-time radial GRAPPA, and interframe respiratory motion was compensated by using an algorithm developed to automatically use images from multiple points of enhancement as references for registration. Quantitative pharmacokinetic analysis was performed using a separable dual-compartment model. Region-of-interest (ROI) pharmacokinetic analysis provided estimates (mean (SD)) of quantitative renal parameters after a half dose: 218.1 (57.1) mL/min per 100 mL; plasma mean transit time, 4.8 (2.2) seconds; renal filtration, 28.7 (10.0) mL/min per 100 mL; and tubular mean transit time, 131.1 (60.2) seconds in 10 kidneys. The ROI pharmacokinetic analysis provided estimates (mean (SD)) of quantitative renal parameters after a quarter dose: 218.1 (57.1) mL/min per 100 mL; plasma mean transit time, 4.8 (2.2) seconds; renal filtration, 28.7 (10.0) mL/min per 100 mL; and tubular mean transit time

  13. Parameterized Radiative Convective Equilibrium Across a Range of Domains: A Unifying Tool for General Circulation Models and High Resolution Models

    NASA Astrophysics Data System (ADS)

    Silvers, L. G.; Stevens, B. B.; Mauritsen, T.; Marco, G. A.

    2015-12-01

    The characteristics of clouds in General Circulation Models (GCMs) need to be constrained in a consistent manner with theory, observations, and high resolution models (HRMs). One way forward is to base improvements of parameterizations on high resolution studies which resolve more of the important dynamical motions and allow for less parameterizations. This is difficult because of the numerous differences between GCMs and HRMs, both technical and theoretical. Century long simulations at resolutions of 20-250 km on a global domain are typical of GCMs while HRMs often simulate hours at resolutions of 0.1km-5km on domains the size of a single GCM grid cell. The recently developed mode ICON provides a flexible framework which allows many of these difficulties to be overcome. This study uses the ICON model to compute SST perturbation simulations on multiple domains in a state of Radiative Convective Equilibrium (RCE) with parameterized convection. The domains used range from roughly the size of Texas to nearly half of Earth's surface area. All simulations use a doubly periodic domain with an effective distance between cell centers of 13 km and are integrated to a state of statistical stationarity. The primary analysis examines the mean characteristics of the cloud related fields and the feedback parameter of the simulations. It is shown that the simulated atmosphere of a GCM in RCE is sufficiently similar across a range of domain sizes to justify the use of RCE to study both a GCM and a HRM on the same domain with the goal of improved constraints on the parameterized clouds. The simulated atmospheres are comparable to what could be expected at midday in a typical region of Earth's tropics under calm conditions. In particular, the differences between the domains are smaller than differences which result from choosing different physics schemes. Significant convective organization is present on all domain sizes with a relatively high subsidence fraction. Notwithstanding

  14. Renal diseases in adults with cystic fibrosis: a 40 year single centre experience.

    PubMed

    Wilcock, M J; Ruddick, A; Gyi, K M; Hodson, M E

    2015-10-01

    There is a sizable literature describing renal disease in patients with cystic fibrosis. Previous studies have focused on single disease processes alone, most commonly renal stone disease or acute kidney injury. In this study we report for the first time on the prevalence of all forms of renal disease in a cystic fibrosis population. A retrospective review of adult patients with cystic fibrosis attending the Adult Cystic Fibrosis Department at the Royal Brompton Hospital was carried out by searching the department's database to identify patients with renal problems and subsequently retrieving clinical information from medical notes. The prevalence of all renal diseases in our population was 5.1 %. The most commonly identified problem was renal stones. At 2.0 % the prevalence of renal stones in adult patients with cystic fibrosis was comparable to the general population. A range of other renal diseases were identified, the next most common being drug-induced acute kidney injury. A range of cystic fibrosis independent and attributable diseases has been identified but no cystic fibrosis specific disease. In contrast to other cystic fibrosis centres no increased prevalence of renal stones was found.

  15. PULMONARY CYSTIC ECHINOCOCCOSIS

    PubMed Central

    Santivanez, Saul; Garcia, Hector H.

    2012-01-01

    Pulmonary cystic echinococosis, a zoonosis caused by the larvae of the dog tapeworm Echinococcus granulosus, is considered as a major public health problem in those countries where dogs are used to care for large herds because of the incapacitating effects produced in affected population. The ratio lung:liver involvement is higher in children than in adults. A higher proportion of lung cases are discovered incidentally on a routine x-ray evaluation; the majority of infected people remain asymptomatic until the cyst enlarges sufficiently to cause symptoms. The majority of symptoms are caused by mass effect from the cyst volume; the presence of complications caused by cysts broke changes the clinical presentation; the principal complication is cyst rupture, producing cough, chest pain, hemoptysis, or vomica. Diagnosis is obtained by imaging evaluation (Chest X-ray or CT scan), supported by serology in the majority of cases. Surgery is the main therapeutic approach, having as principal objective, the removal of the parasite, preventing intraoperative dissemination; the use of pre surgical chemotherapy reduces the chances of seeding and recurrence; treatment using benzimidazoles is the preferred treatment when surgery is not available, or complete removal is not feasible PMID:20216420

  16. Spontaneous pneumomediastinum and hemopneumothoraces secondary to cystic lung metastasis.

    PubMed

    Park, Sung Il; Choi, Eugene; Lee, Heung Bum; Rhee, Yang Keun; Chung, Myoung Ja; Lee, Yong Chul

    2003-01-01

    We report a case of a cystic metastasis to the lung from an angiosarcoma of the scalp in a 75-year-old man who complained of hemoptysis. A chest CT scan showed multiple thin-walled pulmonary cysts, bilateral pneumothoraces, small nodules and pneumomediastinum. Histologic examination revealed pleural infiltration of angiosarcoma cells. One month later, a high-resolution CT scan showed that the cysts had rapidly developed into large lesions.

  17. Cystic Lesions in Autoimmune Pancreatitis.

    PubMed

    Gompertz, Macarena; Morales, Claudia; Aldana, Hernán; Castillo, Jaime; Berger, Zoltán

    2015-01-01

    Autoimmune pancreatitis (AIP) can be chronic or recurrent, but frequently completely reversible after steroid treatment. A cystic lesion in AIP is a rare finding, and it can mimic a pancreatic cystic neoplasm. Difficulties in an exact diagnosis interfere with treatment, and surgery cannot be avoided in some cases. We report the history of a 63-year-old male presenting with jaundice and pruritus. AIP was confirmed by imaging and elevated IgG4 blood levels, and the patient completely recovered after corticosteroid therapy. One year later, he presented with a recurrent episode of AIP with elevated IgG4 levels, accompanied by the appearance of multiple intrapancreatic cystic lesions. All but 1 of these cysts disappeared after steroid treatment, but the remaining cyst in the pancreatic head was even somewhat larger 1 year later. Pancreatoduodenectomy was finally performed. Histology showed the wall of the cystic lesion to be fibrotic; the surrounding pancreatic tissue presented fibrosis, atrophy and lymphoplasmacytic infiltration by IgG4-positive cells, without malignant elements. Our case illustrates the rare possibility that cystic lesions can be part of AIP. These pseudocysts appear in the pancreatic segments involved in the autoimmune disease and can be a consequence of the local inflammation or related to ductal strictures. Steroid treatment should be initiated, after which these cysts can completely disappear with recovery from AIP. Surgical intervention may be necessary in some exceptional cases.

  18. Cystic Neoplasms of the Pancreas

    PubMed Central

    Tran Cao, Hop S.; Kellogg, Benjamin; Lowy, Andrew M.; Bouvet, Michael

    2015-01-01

    Whereas pancreatic duct adenocarcinoma (PDA) is a well-studied (but still poorly understood) disease with a dismal prognosis, cystic neoplasms of the pancreas form a more recently recognized group of pancreatic tumors. They are diverse and variable in their pathologic characteristics, clinical course, and outcomes,1–3 although all portend a better overall prognosis than PDA. In recent years, with the improved sensitivity and increasing use of cross-sectional imaging in clinical practice, these lesions are more commonly identified,4 with many being discovered incidentally. Indeed, large radiological series using computed tomography (CT) or magnetic resonance imaging (MRI) have reported detection rates of pancreatic cystic lesions between 1.2% and almost 20%,5,6 approaching the 24.3% prevalence rate in an autopsy series by Kimura and colleagues.7 Although most of these lesions are pseudocysts, a significant portion consist of cystic neoplasms, which are estimated to represent 10% to 15% of all primary pancreatic cystic lesions.8 Given the growing clinical relevance of these tumors, a keen understanding of their natural history and pathophysiology is needed. This article reviews pancreatic cystic neoplasms, with a focus on the challenges encountered in their diagnosis and treatment. PMID:20159515

  19. Cystic Fibrosis (CF): Chloride Sweat Test

    MedlinePlus

    ... to 2-Year-Old Cystic Fibrosis (CF) Chloride Sweat Test KidsHealth > For Parents > Cystic Fibrosis (CF) Chloride Sweat Test Print A A A What's in this ... en el sudor What It Is A chloride sweat test helps diagnose cystic fibrosis (CF) , an inherited ...

  20. Cystic Fibrosis (CF): Chloride Sweat Test

    MedlinePlus

    ... 1- to 2-Year-Old Cystic Fibrosis (CF) Chloride Sweat Test KidsHealth > For Parents > Cystic Fibrosis (CF) Chloride Sweat Test A A A What's in this ... cloruro en el sudor What It Is A chloride sweat test helps diagnose cystic fibrosis (CF) , an ...

  1. What's it Like to Have Cystic Fibrosis?

    MedlinePlus

    ... Room? What Happens in the Operating Room? Cystic Fibrosis KidsHealth > For Kids > Cystic Fibrosis A A A What's in this article? What ... a condition she's known all her life — cystic fibrosis (say: SIS-tik fi-BRO-sus). Her parents ...

  2. Diagnosis and treatment of cystic lung disease

    PubMed Central

    Park, Sanghoon; Lee, Eun Joo

    2017-01-01

    Cystic lung disease (CLD) is a group of lung disorders characterized by the presence of multiple cysts, defined as air-filled lucencies or low-attenuating areas, bordered by a thin wall (usually < 2 mm). The recognition of CLDs has increased with the widespread use of computed tomography. This article addresses the mechanisms of cyst formation and the diagnostic approaches to CLDs. A number of assessment methods that can be used to confirm CLDs are discussed, including high-resolution computed tomography, pathologic approaches, and genetic/ serologic markers, together with treatment modalities, including new therapeutic drugs currently being evaluated. The CLDs covered by this review are lymphangioleiomyomatosis, pulmonary Langerhans cell histiocytosis, Birt-Hogg-Dube syndrome, lymphocytic interstitial pneumonia/follicular bronchiolitis, and amyloidosis. PMID:28264540

  3. Radioisotope therapy of cystic craniopharyngeomas

    SciTech Connect

    Strauss, L.; Sturm, V.; Georgi, P.; Schlegel, W.; Ostertag, H.; Clorius, J.H.; Van Kaick, G.

    1982-09-01

    Eighteen patients suffering from cystic craniopharyngeoma were treated with intracavitary irradiation. The beta-emitting radioisotope /sup 90/y (2.25 MeV) was instilled into the cyst following stereotactic puncture of the space-occupying lesion. The surgical approach was planned using angiograms and reconstructed transmission computer tomography (TCT) coronal and saggital sections. Therapy was devised to deliver 20,000 rad to the cyst's wall. Eleven patients received follow-up TCT examinations after four months. Eight of 11 patients had a significant volume decrease in the craniopharyngeoma cyst. In two patients, the cystic volume remained unchanged; one had progression of disease. It is concluded that the intracavitary treatment of cystic craniopharyngeoma will result in a reduction of the size of the space-occupying lesion.

  4. [Intestinal cystic duplication. Case report].

    PubMed

    Herranz Barbero, Ana; Prat Ortells, Jordi; Muñoz Fernández, M Elena; Castañón García-Alix, Montserrat; Figueras Aloy, Josep

    2017-08-01

    Intestinal cystic duplications are rare congenital anomalies, with an estimated incidence of approximately 1:4500 autopsies. The etiopathogenesis is uncertain. These duplications are cystic, tubular or diverticular structures lined with gastrointestinal mucosa. They share a common smooth muscle wall with the gastrointestinal tract but usually their lumens do not communicate with each other. Gastric duplication cysts represent 7-9% of the gastrointestinal tract duplication. They can be diagnosed prenatally by fetal ultrasound; magnetic resonance imaging characterizes the cyst and excludes other malformations. Postnatal ultrasound shows a characteristic double walled cyst. Newborns are usually asymptomatic, although nonspecific gastrointestinal symptoms, intestinal obstruction due to mass effect, volvulus or infection are described. In asymptomatic patients, clinical follow-up and periodic image controls are recommended. Elective surgical resection is the treatment of choice, using minimally invasive technique whenever possible. A case of prenatally suspected intestinal cystic duplication is presented. Sociedad Argentina de Pediatría.

  5. Nutritional management of cystic fibrosis.

    PubMed Central

    MacDonald, A

    1996-01-01

    Nutritional support is an integral part of the management of cystic fibrosis patients. It is arguably best provided by a qualified dietitian and nutritional care sister working in conjunction with the rest of the cystic fibrosis team. The patient's nutritional needs should be assessed, regularly reviewed, and nutritional treatment tailored to meet the changing clinical and psychosocial needs of the patient. Nutritional intervention is not without complications, and in particular attention to normal feeding behaviour and vigilance when instituting supplementary nutrition may prevent many feeding difficulties. PMID:8660059

  6. Imaging of Cystic and Cyst-like Lesions of the Mediastinum with Pathologic Correlation

    PubMed Central

    Ödev, Kemal; Arıbaş, Bilgin K.; Nayman, Alaaddin; Arıbaş, Olgun K.; Altınok, Tamer; Küçükapan, Ahmet

    2012-01-01

    Cystic masses of the mediastinum are a heterogenous group of asymptomatic or symptomatic, congenital, infectious, or neoplastic lesions. For early and correct diagnosis, evaluation, and optimal patient management of cystic mediastinal masses in infants, children, or adults imaging plays an important role. A non-invasive and sensitive imaging modality is an efficient and cost-effective tool. Multidetector computed tomography (MDTC) with volumetric acquisition provides fast acquisition of high resolution images and multiplanar reconstruction. Both 2D and 3D imaging in mediastinal imaging help in surgical planning and assessing resectability of mediastinal lesions. MR imaging has many advantages over other modalities for detecting and identifying cystic, or fluid-filled mediastinal masses, because of its intrinsic high soft tissue contrast and direct multiplanar imaging capabilities. However, histological tissue analysis may be required to differentiate a cystic lesion from other cyst-like or low-attenuation lesions. PMID:22919547

  7. The clinical anatomy of cystic artery variations: a review of over 9800 cases.

    PubMed

    Andall, R G; Matusz, P; du Plessis, M; Ward, R; Tubbs, R S; Loukas, M

    2016-07-01

    While laparoscopic cholecystectomy can be a routine procedure when biliary anatomy is normally located, cystic artery variations can easily disorientate the inexperienced surgeon to the anatomy of the hepatobiliary triangle. This study presents the clinically important anatomical variations of the cystic artery. PubMed, Medline, Cochrane Database of Systematic Reviews, and Google Scholar databases were searched to conduct a review of the existing English literature on the clinically important cystic artery variations. An aberrant vessel was defined as a vessel that originated from an atypical source and/or one that was present in a specimen in addition to the normal vessel. The cystic artery originated typically from the right hepatic artery (79.02 %) and was found in the hepatobiliary triangle in only 5427 of 6661 (81.5 %) cases. Clinically important cystic artery variations are (1) the cystic artery located anterior to the common hepatic duct or common bile duct found in 485 of 2704 (17.9 %) and 228 of 4202 (5.4 %) of cases, respectively, (2) the cystic artery located inferior to the cystic duct found in 38 of 770 (4.9 %) of cases, (3) short cystic arteries found in 98 of 1037 (9.5 %) cases and (4) multiple cystic arteries found in (8.9 %) of cases. These variations are common in the general population and can lead to inadvertent ligation of biliary ducts or aberrant vessels. Therefore, it is important for the hepatobiliary surgeon to be aware of these vascular anomalies to avoid operative complications.

  8. Three-dimensional structures of tropical nonmigrating tides in a high-vertical-resolution general circulation model

    NASA Astrophysics Data System (ADS)

    Sakazaki, Takatoshi; Sato, Kaoru; Kawatani, Yoshio; Watanabe, Shingo

    2015-03-01

    This paper investigates nonmigrating tides from the ground to the lower mesosphere using data from a high-resolution general circulation model (KANTO GCM), as well as observational data from the Sounding of the Atmosphere using Broadband Emission Radiometry instrument on board the Thermosphere-Ionosphere-Mesosphere Energetics and Dynamics satellite and from GPS radio occultation measurements obtained with the COSMIC/FORMOSAT-3 mission. We extract nonmigrating tides using a composite as a function of universal time in physical space, without performing a zonal wave number decomposition. The KANTO GCM clearly demonstrates that tropical nonmigrating tides are regarded as gravity waves excited by diabatic heating enhanced over two major continents, specifically Africa and South America. They propagate zonally, in a direction away from their sources; that is, west and eastward propagating waves are dominant on the western and eastern sides of the continents, respectively. These characteristics are observed in two satellite data sets as well, except that the amplitudes in the KANTO GCM are larger than those in the observations. Seasonal variations of nonmigrating tides are also investigated. It is suggested that filtering owing to the stratopause semiannual oscillation, as well as diabatic heating in the troposphere, is important for the seasonal variations of nonmigrating tides in the stratosphere and the lower mesosphere.

  9. Simulation of a dust episode over Eastern Mediterranean using a high-resolution atmospheric chemistry general circulation model

    NASA Astrophysics Data System (ADS)

    Abdel Kader, Mohamed; Zittis, Georgios; Astitha, Marina; Lelieveld, Jos; Tymvios, Fillipos

    2013-04-01

    An extended episode of low visibility took place over the Eastern Mediterranean in late September 2011, caused by a strong increase in dust concentrations, analyzed from observations of PM10 (Particulate Matter with <10μm in diameter). A high-resolution version of the atmospheric chemistry general circulation model EMAC (ECHAM5/Messy2.41 Atmospheric Chemistry) was used to simulate the emissions, transport and deposition of airborne desert dust. The model configuration involves the spectral resolution of T255 (0.5°, ~50Km) and 31 vertical levels in the troposphere and lower stratosphere. The model was nudged towards ERA40 reanalysis data to represent the actual meteorological conditions. The dust emissions were calculated online at each model time step and the aerosol microphysics using the GMXe submodel (Global Modal-aerosol eXtension). The model includes a sulphur chemistry mechanism to simulate the transformation of the dust particles from the insoluble (at emission) to soluble modes, which promotes dust removal by precipitation. The model successfully reproduces the dust distribution according to observations by the MODIS satellite instruments and ground-based AERONET stations. The PM10 concentration is also compared with in-situ measurements over Cyprus, resulting in good agreement. The model results show two subsequent dust events originating from the Negev and Sahara deserts. The first dust event resulted from the transport of dust from the Sahara on the 21st of September and lasted only briefly (hours) as the dust particles were efficiently removed by precipitation simulated by the model and observed by the TRMM (Tropical Rainfall Measuring Mission) satellites. The second event resulted from dust transport from the Negev desert to the Eastern Mediterranean during the period 26th - 30th September with a peak concentration at 2500m elevation. This event lasted for four days and diminished due to dry deposition. The observed reduced visibility over Cyprus

  10. Survival rates in cystic fibrosis.

    PubMed Central

    Wilmott, R W; Tyson, S L; Dinwiddie, R; Matthew, D J

    1983-01-01

    Life tables were calculated for 273 British children with cystic fibrosis for the period 1974-9. There was a marked improvement in survival rates in the meconium ileus group compared with the 1969-73 data, but there was little improvement in patients presenting later with other symptoms. PMID:6639137

  11. Nutritional management of cystic fibrosis.

    PubMed

    Goodchild, M C

    1987-01-01

    Cystic fibrosis patients have an increased requirement for calories and probably for all the major nutrients. The newer, enteric-coated granular preparations of pancreatic enzyme are more effective than preceding preparations and should permit a normal fat intake. Recent work has emphasized the interdependence of respiratory disease and nutrition.

  12. Cystic lumphangioma of the colon

    SciTech Connect

    Agha, F.P.; Francis, I.R.; Simms, S.M.

    1983-10-01

    Cystic lymphangioma is a rare benign lesion of the gastrointestinal tract, in which the colon is the least frequntly involved site. A case is reported displaying the characteristic radiographic features of an extramucosal intramural mass lesion in a patient with concurrent cystadenocarcinoma of the pancreas, in whom the possibility of a metastatic lesion to the colon could not be excluded except by surgical resection.

  13. [Therapeutic update in cystic fibrosis].

    PubMed

    Durupt, S; Nove Josserand, R; Durieu, I

    2014-06-01

    We present the recent therapeutic advances in the cystic fibrosis care. It concerns improvements in symptomatic treatment with the development of dry powder inhaled antibiotics that improved quality of life, and innovative treatments namely the modulators of the cystic fibrosis transmembrane protein conductance regulator (CFTR), molecules which act specifically at the level of the defective mechanisms implied in the disease. The life expectancy of cystic fibrosis patients born after 2000, is estimated now to be about 50 years. This improvement of survival was obtained with the organization of the care within the specialized centers for cystic fibrosis (Centre de ressource et de compétences de la mucoviscidose) and remains still based on heavy symptomatic treatments. Dry powder inhaled antibiotics constitute a significant time saving for patients to whom all the care can achieve two hours daily. Since 2012, the modulators of CFTR, molecules allowing a pharmacological approach targeted according to the type of the mutations, allows a more specific approach of the disease. Ivacaftor (Kalydeco(®)) which potentialises the function of the CFTR protein expressed on the cellular surface is now available for patients with the G551D mutation. Lumacaftor is going to be tested in association with ivacaftor in patients with the F508del mutation, that is present in at least 75% of the patients. The ataluren which allows the production of a functional protein CFTR in patients with a no sense mutation is the third representing of this new therapeutic class. We presently have numerous symptomatic treatments for the cystic fibrosis care. The development of CFTR modulators, today available to a restricted number of patients treated with ivacaftor represents a very promising therapeutic avenue. It will represent probably the first step to a personalized treatment according to CFTR genotype.

  14. Elastase-mediated phosphatidylserine receptor cleavage impairs apoptotic cell clearance in cystic fibrosis and bronchiectasis

    PubMed Central

    Vandivier, R. William; Fadok, Valerie A.; Hoffmann, Peter R.; Bratton, Donna L.; Penvari, Churee; Brown, Kevin K.; Brain, Joseph D.; Accurso, Frank J.; Henson, Peter M.

    2002-01-01

    Cystic fibrosis is characterized by an early and sustained influx of inflammatory cells into the airways and by release of proteases. Resolution of inflammation is normally associated with the orderly removal of dying apoptotic inflammatory cells through cell recognition receptors, such as the phosphatidylserine receptor, CD36, and αv integrins. Accordingly, removal of apoptotic inflammatory cells may be impaired in persistent inflammatory responses such as that seen in cystic fibrosis airways. Examination of sputa from cystic fibrosis and non–cystic fibrosis bronchiectasis patients demonstrated an abundance of apoptotic cells, in excess of that seen in patients with chronic bronchitis. In vitro, cystic fibrosis and bronchiectasis airway fluid directly inhibited apoptotic cell removal by alveolar macrophages in a neutrophil elastase-dependent manner, suggesting that elastase may impair apoptotic cell clearance in vivo. Flow cytometry demonstrated that neutrophil elastase cleaved the phosphatidylserine receptor, but not CD36 or CD32 (FcγRII). Cleavage of the phosphatidylserine receptor by neutrophil elastase specifically disrupted phagocytosis of apoptotic cells, implying a potential mechanism for delayed apoptotic cell clearance in vivo. Therefore, defective airway clearance of apoptotic cells in cystic fibrosis and bronchiectasis may be due to elastase-mediated cleavage of phosphatidylserine receptor on phagocytes and may contribute to ongoing airway inflammation. PMID:11877474

  15. Poly Cystic Ovarian Syndrome: An Updated Overview

    PubMed Central

    El Hayek, Samer; Bitar, Lynn; Hamdar, Layal H.; Mirza, Fadi G.; Daoud, Georges

    2016-01-01

    Poly Cystic Ovarian Syndrome (PCOS) is one of the most common metabolic and reproductive disorders among women of reproductive age. Women suffering from PCOS present with a constellation of symptoms associated with menstrual dysfunction and androgen excess, which significantly impacts their quality of life. They may be at increased risk of multiple morbidities, including obesity, insulin resistance, type II diabetes mellitus, cardiovascular disease (CVD), infertility, cancer, and psychological disorders. This review summarizes what the literature has so far provided from guidelines to diagnosis of PCOS. It will also present a general overview about the morbidities associated with this disease, specifically with its more severe classic form. Finally, the review will stress on the various aspects of treatment and screening recommendations currently used in the management of this condition. PMID:27092084

  16. Poly Cystic Ovarian Syndrome: An Updated Overview.

    PubMed

    El Hayek, Samer; Bitar, Lynn; Hamdar, Layal H; Mirza, Fadi G; Daoud, Georges

    2016-01-01

    Poly Cystic Ovarian Syndrome (PCOS) is one of the most common metabolic and reproductive disorders among women of reproductive age. Women suffering from PCOS present with a constellation of symptoms associated with menstrual dysfunction and androgen excess, which significantly impacts their quality of life. They may be at increased risk of multiple morbidities, including obesity, insulin resistance, type II diabetes mellitus, cardiovascular disease (CVD), infertility, cancer, and psychological disorders. This review summarizes what the literature has so far provided from guidelines to diagnosis of PCOS. It will also present a general overview about the morbidities associated with this disease, specifically with its more severe classic form. Finally, the review will stress on the various aspects of treatment and screening recommendations currently used in the management of this condition.

  17. Laparoscopic management of enlarged cystic duct.

    PubMed

    Nowzaradan, Y; Meador, J; Westmoreland, J

    1992-12-01

    After laparoscopic exploration of the common bile duct, or when a patient has acute cholecystitis, the cystic duct is sometimes edematous and too large to be ligated safely with an Endoclip. In such cases, ligation of the cystic duct with an Endoloop offers a solution to the problem. The standard technique for application of an Endoloop consists of dividing the cystic duct and then applying the Endoloop. This becomes more difficult if, after the cystic duct is divided, loss of traction on the common bile duct results in retraction of the divided cystic stump outside of the laparoscopic field of view. To avoid this difficulty, the authors apply an Endoloop with the grasping forceps on the cystic duct before the duct is divided so that it cannot retract from operative view and for this task developed an instrument that allows simultaneous introduction of both grasping forceps and the Endoloop through a single port.

  18. Biomarkers in Paediatric Cystic Fibrosis Lung Disease.

    PubMed

    Ramsey, Kathryn A; Schultz, André; Stick, Stephen M

    2015-09-01

    Biomarkers in cystic fibrosis are used i. for the measurement of cystic fibrosis transmembrane regulator function in order to diagnose cystic fibrosis, and ii. to assess aspects of lung disease severity (e.g. inflammation, infection). Effective biomarkers can aid disease monitoring and contribute to the development of new therapies. The tests of cystic fibrosis transmembrane regulator function each have unique strengths and weaknesses, and biomarkers of inflammation, infection and tissue destruction have the potential to enhance the management of cystic fibrosis through the early detection of disease processes. The development of biomarkers of cystic fibrosis lung disease, in particular airway inflammation and infection, is influenced by the challenges of obtaining relevant samples from infants and children for whom early detection and treatment of disease might have the greatest long term benefits.

  19. Cystic Fibrosis Research | NIH MedlinePlus the Magazine

    MedlinePlus

    ... please turn Javascript on. Feature: Steady Advances Against Cystic Fibrosis Cystic Fibrosis Research Past Issues / Fall 2012 Table of Contents "Remarkable strides in cystic fibrosis research over the past two decades have culminated ...

  20. [Historical compilation of cystic fibrosis].

    PubMed

    Navarro, Salvador

    2016-01-01

    Cystic fibrosis is the most common life-shortening recessively inherited disorder in the Caucasian population. The genetic mutation that most frequently provokes cystic fibrosis (ΔF508) appeared at least 53,000years ago. For many centuries, the disease was thought to be related to witchcraft and the "evil eye" and it was only in 1938 that Dorothy H. Andersen characterized this disorder and suspected its genetic origin. The present article reviews the pathological discoveries and diagnostic and therapeutic advances made in the last 75 years. The review ends with some considerations for the future. Copyright © 2015 Elsevier España, S.L.U. and AEEH y AEG. All rights reserved.

  1. Pharmacogenetics of cystic fibrosis treatment.

    PubMed

    Carter, Suzanne C; McKone, Edward F

    2016-08-01

    Cystic fibrosis (CF) is genetic autosomal recessive disease caused by reduced or absent function of CFTR protein. Treatments for patients with CF have primarily focused on the downstream end-organ consequences of defective CFTR. Since the discovery of the CFTR gene that causes CF in 1989 there have been tremendous advances in our understanding of the genetics and pathophysiology of CF. This has recently led to the development of new CFTR mutation-specific targeted therapies for select patients with CF. This review will discuss the characteristics of the CFTR gene, the CFTR mutations that cause CF and the new mutation specific pharmacological treatments including gene therapy that are contributing to the dawning of a new era in cystic fibrosis care.

  2. Mesenteric cystic lymphangioma mimicking malignancy.

    PubMed

    Hureibi, Khalid; Sunidar, Osama A

    2014-09-01

    Mesenteric cystic lymphangiomas are benign tumours arising from the mesentery, and have no known aetiology. Patients might be discovered incidentally to have asymptomatic mesenteric cysts, or they can present with symptoms such as pain, nausea and vomiting. A 27-year-old man presented with vague abdominal pain, loss of appetite, postprandial fullness and significant weight loss. There was no lymphadenopathy, and abdominal examination was unremarkable. CT showed a mesenteric mass and a diagnosis of abdominal lymphoma was suggested. There was no evidence of pulmonary tuberculosis on chest X-ray and the purified protein derivative test was negative. On laparotomy, a 5×9×7 cm sessile cyst containing thick white fluid and arising from the ileal mesentery was found and completely removed. Histopathology proved a diagnosis of mesenteric cystic lymphangioma. The patient made uneventful recovery, and was asymptomatic on clinical follow-up after 6 weeks. 2014 BMJ Publishing Group Ltd.

  3. On the Maxwell-Stefan approach to diffusion: a general resolution in the transient regime for one-dimensional systems.

    PubMed

    Leonardi, Erminia; Angeli, Celestino

    2010-01-14

    The diffusion process in a multicomponent system can be formulated in a general form by the generalized Maxwell-Stefan equations. This formulation is able to describe the diffusion process in different systems, such as, for instance, bulk diffusion (in the gas, liquid, and solid phase) and diffusion in microporous materials (membranes, zeolites, nanotubes, etc.). The Maxwell-Stefan equations can be solved analytically (only in special cases) or by numerical approaches. Different numerical strategies have been previously presented, but the number of diffusing species is normally restricted, with only few exceptions, to three in bulk diffusion and to two in microporous systems, unless simplifications of the Maxwell-Stefan equations are considered. In the literature, a large effort has been devoted to the derivation of the analytic expression of the elements of the Fick-like diffusion matrix and therefore to the symbolic inversion of a square matrix with dimensions n x n (n being the number of independent components). This step, which can be easily performed for n = 2 and remains reasonable for n = 3, becomes rapidly very complex in problems with a large number of components. This paper addresses the problem of the numerical resolution of the Maxwell-Stefan equations in the transient regime for a one-dimensional system with a generic number of components, avoiding the definition of the analytic expression of the elements of the Fick-like diffusion matrix. To this aim, two approaches have been implemented in a computational code; the first is the simple finite difference second-order accurate in time Crank-Nicolson scheme for which the full mathematical derivation and the relevant final equations are reported. The second is based on the more accurate backward differentiation formulas, BDF, or Gear's method (Shampine, L. F. ; Gear, C. W. SIAM Rev. 1979, 21, 1.), as implemented in the Livermore solver for ordinary differential equations, LSODE (Hindmarsh, A. C. Serial

  4. Precipitation response to solar geoengineering in a high-resolution tropical-cyclone permitting coupled general circulation model

    NASA Astrophysics Data System (ADS)

    Irvine, P. J.; Keith, D.; Dykema, J. A.; Vecchi, G. A.; Horowitz, L. W.

    2016-12-01

    Solar geoengineering may limit or even halt the rise in global-average surface temperatures. Evidence from the geoMIP model intercomparison project shows that idealized geoengineering can greatly reduce temperature changes on a region-by-region basis. If solar geoengineering is used to hold radiative forcing or surface temperatures constant in the face of rising CO2, then the global evaporation and precipitation rates will be reduced below pre-industrial. The spartial and frequency distribution of the precipitation response is, however, much less well understood. There is limited evidence that solar geoengineering may reduce extreme precipitation events more that it reduces mean precipitation, but that evidence is based on relatively course resolution models that may to a poor job representing the distribution of extreme precipitation in the current climate. The response of global and regional climate, as well as tropical cyclone (TC) activity, to increasing solar geoengineering is explored through experiments with climate models spanning a broad range of atmospheric resolutions. Solar geoengineering is represented by an idealized adjustment of the solar constant that roughly halves the rate of increase in radiative forcing in a scenario with increasing CO2 concentration. The coarsest resolution model has approximately a 2-degree global resolution, representative of the typical resolution of past GCMs used to explore global response to CO2 increase, and its response is compared to that of two tropical cyclone permitting GCMs of approximately 0.5 and 0.25 degree resolution (FLOR and HiFLOR). The models have exactly the same ocean and sea-ice components, as well as the same parameterizations and parameter settings. These high-resolution models are used for real-time seasonal prediction, providing a unified framework for seasonal-to-multidecadal climate modeling. We assess the extreme precipitation response, comparing the frequency distribution of extreme events with

  5. A multidecadal simulation of Atlantic tropical cyclones using a variable-resolution global atmospheric general circulation model

    NASA Astrophysics Data System (ADS)

    Zarzycki, Colin M.; Jablonowski, Christiane

    2014-09-01

    Using a variable-resolution option within the National Center for Atmospheric Research/Department of Energy Community Atmosphere Model (CAM) Spectral Element (SE) global model, a refined nest at 0.25° (˜28 km) horizontal resolution located over the North Atlantic is embedded within a global 1° (˜111 km) grid. The grid is designed such that fine grid cells are located where tropical cyclones (TCs) are observed to occur during the Atlantic TC season (June-November). Two simulations are compared, one with refinement and one control case with no refinement (globally uniform 1° grid). Both simulations are integrated for 23 years using Atmospheric Model Intercomparison Protocols. TCs are tracked using an objective detection algorithm. The variable-resolution simulation produces significantly more TCs than the unrefined simulation. Storms that do form in the refined nest are much more intense, with multiple storms strengthening to Saffir-Simpson category 3 intensity or higher. Both count and spatial distribution of TC genesis and tracks in the variable-resolution simulation are well matched to observations and represent significant improvements over the unrefined simulation. Some degree of interannual skill is noted, with the variable-resolution grid able to reproduce the observed connection between Atlantic TCs and the El Niño-Southern Oscillation (ENSO). It is shown that Genesis Potential Index (GPI) is well matched between the refined and unrefined simulations, implying that the introduction of variable-resolution does not affect the synoptic environment. Potential "upscale" effects are noted in the variable-resolution simulation, suggesting stronger TCs in refined nests may play a role in meridional transport of momentum, heat, and moisture.

  6. Pseudomembranous colitis in cystic fibrosis.

    PubMed

    Nagakumar, Prasad

    2013-05-01

    Cystic fibrosis (CF) patients may require frequent courses of antibiotics and repeated hospital admissions. Although children with CF have high carriage rate for C.difficile, they rarely develop colitis. Pseudomembranous colitis is more common in adult post lung transplant CF patients. Although rare, paseudomembranous colitis should be considered in CF patients presenting with abdominal symptoms even in the absence of diarrhoea. Copyright © 2013 Elsevier Ltd. All rights reserved.

  7. Etanercept-induced cystic acne.

    PubMed

    Kashat, Maria; Caretti, Katherine; Kado, Jessica

    2014-07-01

    Tumor necrosis factor α antagonists are potent biologics used to treat a variety of autoimmune disorders such as rheumatoid arthritis, ankylosing spondylitis, Crohn disease, psoriasis, and psoriatic arthritis. These medications are known to have many side effects (eg, infusion reactions, cytopenia, risk for infection, heart failure); however, only a few cases of acne vulgaris have been associated with the use of these biologics, particularly infliximab and adalimumab. We report a rare case of etanercept-induced cystic acne.

  8. Description and validation of a scoring system for tomosynthesis in pulmonary cystic fibrosis.

    PubMed

    Vult von Steyern, Kristina; Björkman-Burtscher, Isabella M; Höglund, Peter; Bozovic, Gracijela; Wiklund, Marie; Geijer, Mats

    2012-12-01

    To design and validate a scoring system for tomosynthesis (digital tomography) in pulmonary cystic fibrosis. A scoring system dedicated to tomosynthesis in pulmonary cystic fibrosis was designed. Three radiologists independently scored 88 pairs of radiographs and tomosynthesis examinations of the chest in 60 patients with cystic fibrosis and 7 oncology patients. Radiographs were scored according to the Brasfield scoring system and tomosynthesis examinations were scored using the new scoring system. Observer agreements for the tomosynthesis score were almost perfect for the total score with square-weighted kappa >0.90, and generally substantial to almost perfect for subscores. Correlation between the tomosynthesis score and the Brasfield score was good for the three observers (Kendall's rank correlation tau 0.68, 0.77 and 0.78). Tomosynthesis was generally scored higher as a percentage of the maximum score. Observer agreements for the total score for Brasfield score were almost perfect (square-weighted kappa 0.80, 0.81 and 0.85). The tomosynthesis scoring system seems robust and correlates well with the Brasfield score. Compared with radiography, tomosynthesis is more sensitive to cystic fibrosis changes, especially bronchiectasis and mucus plugging, and the new tomosynthesis scoring system offers the possibility of more detailed and accurate scoring of disease severity. Tomosynthesis is more sensitive than conventional radiography for pulmonary cystic fibrosis changes. The radiation dose from chest tomosynthesis is low compared with computed tomography. Tomosynthesis may become useful in the regular follow-up of patients with cystic fibrosis.

  9. Disease modifying anti-rheumatic drugs in people with cystic fibrosis-related arthritis.

    PubMed

    Thornton, Judith; Rangaraj, Satyapal

    2012-09-12

    Arthritis remains a relatively infrequent complication of cystic fibrosis, but is a cause of significant morbidity when it does occur. Two distinct types of arthritis are described in cystic fibrosis: cystic fibrosis-related arthropathy and hypertrophic osteoarthropathy. Management of arthritis in people with cystic fibrosis is uncertain and complex because of the underlying disease and its treatment. To review the effectiveness and safety of disease-modifying anti-rheumatic drugs for the management of arthritis related to cystic fibrosis in adults and children. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Cystic Fibrosis Trials Register which comprises references identified from comprehensive electronic database handsearches of relevant journal and abstract books of conference proceedings.Date of most recent search: 10 July 2012. Randomised controlled trials which compared the efficacy and safety of disease-modifying anti-rheumatic drugs (e.g. methotrexate, gold, sulfasalazine, penicillamine, leflunomide, hydroxychloroquine and newer agents such as biologic disease modifying agents and monoclonal antibodies) with each other, with no treatment or with placebo for cystic fibrosis-related arthropathy or hypertrophic osteoarthropathy. No relevant studies were identified. No studies were included in this review. Although it is generally recognised that cystic fibrosis-related arthritis can be episodic and resolve spontaneously, treatment with analgesics and anti-inflammatory agents may be needed. But when episodic symptoms progress to persistent disease, disease-modifying anti-rheumatic drugs may be needed to limit the course of the disease. It is disappointing that no randomised controlled trials to rigorously evaluate these drugs could be found. This systematic review has identified the need for a well-designed adequately powered randomised controlled trial to assess the efficacy and safety of disease-modifying anti-rheumatic drugs for the

  10. Investigating Cenozoic climate change in tectonically active regions with a high-resolution atmospheric general circulation model (ECHAM5)

    NASA Astrophysics Data System (ADS)

    Mutz, Sebastian; Ehlers, Todd; Li, Jingmin; Werner, Martin; Stepanek, Christian; Lohmann, Gerrit

    2016-04-01

    Studies of Cenozoic palaeo-climates contribute to our understanding of contemporary climate change by providing insight into analogues such as the Pliocene (PLIO), and by evaluation of GCM (General Circulation Models) performance using the Mid-Holocene (MH) and the Last Glacial Maximum (LGM). Furthermore, climate is a factor to be considered in the evolution of ecology, landscapes and mountains, and in the reconstruction of erosion histories. In this study, we use high-resolution (T159) ECHAM5 simulations to investigate pre-industrial (PI) and the the above mentioned palaeo-climates for four tectonically active regions: Alaska (St. Elias Range), the US Northwest Pacific (Cascade Range), western South America (Andes) and parts of Asia (Himalaya-Tibet). The PI climate simulation is an AMIP (Atmospheric Model Intercomparison Project) style ECHAM5 experiment, whereas MH and LGM simulation are based on simulations conducted at the Alfred Wegner Institute, Bremerhaven. Sea surface boundary conditions for MH were taken from coupled atmosphere-ocean model simulations (Wei and Lohmann, 2012; Zhang et al, 2013) and sea surface temperatures and sea ice concentration for the LGM are based on GLAMAP project reconstructions (Schäfer-Neth and Paul, 2003). Boundary conditions for the PLIO simulation are taken from the PRISM (Pliocene Research, Interpretation and Synoptic Mapping) project and the employed PLIO vegetation boundary condition is created by means of the transfer procedure for the PRISM vegetation reconstruction to the JSBACH plant functional types as described by Stepanek and Lohmann (2012). For each of the investigated areas and time slices, the regional simulated climates are described by means of cluster analyses based on the variability of precipitation, 2m air temperature and the intra-annual amplitude of the values. Results indicate the largest differences to a PI climate are observed for LGM and PLIO climates in the form of widespread cooling and warming

  11. Effects of ocean grid resolution on tropical cyclone-induced upper ocean responses using a global ocean general circulation model

    NASA Astrophysics Data System (ADS)

    Li, Hui; Sriver, Ryan L.

    2016-11-01

    Tropical cyclones (TCs) have the potential to influence regional and global climate through interactions with the upper ocean. Here we present results from a suite of ocean-only model experiments featuring the Community Earth System Model, in which we analyze the effect of tropical cyclone wind forcing on the global ocean using three different horizontal ocean grid resolutions (3°, 1°, and 0.1°). The ocean simulations are forced with identical atmospheric inputs from the Coordinated Ocean-Ice Reference Experiments version 2 (COREv2) normal year forcing conditions, featuring global blended TC winds from a fully coupled CESM simulation with a 25 km atmosphere. The simulated TC climatology shows good agreement with observational estimates of annual TC statistics, including annual frequency, intensity distributions, and geographic distributions. Each ocean simulation is composed of a 5 year spin-up with COREv2 normal year forcing, followed by 18 months with blended TC winds. In addition, we conduct corresponding control simulations for each grid resolution configuration without blended TC winds. We find that ocean horizontal and vertical grid resolutions affect TC-induced heat and momentum fluxes, poststorm cold wake features, and ocean subsurface temperature profiles. The responses are amplified for smaller grid spacing. Moreover, analyses show that the annually accumulated TC-induced ocean heat uptake is also sensitive to ocean grid resolution, which may have important implications for modeled ocean heat budgets and variability.

  12. Cystic and ductal tumors of the pancreas: diagnosis and management.

    PubMed

    Scoazec, J Y; Vullierme, M P; Barthet, M; Gonzalez, J M; Sauvanet, A

    2013-04-01

    Incidentally discovered cystic tumors of the pancreas (CTP) are an increasingly frequent entity. It is essential to differentiate lesions whose malignant potential is either nil or negligible (pseudocyst, serous cystadenoma, simple cysts) from lesions with intermediate malignant potential (intraductal papillary mucinous tumor of the pancreas [IPMN] involving the secondary ducts, cystic endocrine tumor) or those with high malignant potential (mucinous cystadenoma, solid pseudopapillary tumors and IPMN involving the main pancreatic duct). The approach to defining malignant potential is based on diagnostic CT scan, magnetic resonance imaging (MRI), and endoscopic ultrasound (EUS), often complemented by EUS-guided cyst puncture for biochemical and cytological analysis of cyst fluid. Surgery for diagnostic purposes should be avoided because of its significant morbidity. For pseudocysts, simple cysts and serous cystadenomas, abstention is the general rule. Resection, preserving as much pancreatic parenchyma as possible, is the rule for IPMN involving the main pancreatic duct, mucinous cystadenomas, solid and pseudopapillary tumors, and cystic endocrine tumors. Resection is rarely indicated at the outset for IPMN involving secondary pancreatic ducts; morphologic observation is the general rule and preventive excision may be indicated secondarily. Good collaboration between surgeons, radiologists and endosonographists is necessary for optimal management of CTP.

  13. Pleural effusions in non-transplanted cystic fibrosis patients.

    PubMed

    Belanger, Adam R; Nguyen, Kimtuyen; Osman, Umar; Gilbert, Christopher R; Allen, Katie; Al Rais, Ahmad Farid; Yarmus, Lonny; Akulian, Jason A

    2017-07-01

    Pleural effusions are considered rare in cystic fibrosis (CF) patients. There is a paucity of available information in the literature concerning the nature and significance of pleural effusions in non-transplanted CF patients. We conducted a multicenter retrospective evaluation of non-transplanted adult CF patients. Given the small sample size, only descriptive statistics were performed. A total of 17 CF patients with pleural effusion were identified, of whom 9 patients underwent thoracentesis. The crude incidence of pleural effusion was 43 per 10,000 person-years in hospitalized CF patients at large CF centers. All sampled effusions were inflammatory in nature. All samples submitted for culture grew at least one organism. Pleural effusions are rare in adult non-transplanted CF patients. These fluid collections appear to be quite inflammatory with a higher rate of empyema than in the general population. Copyright © 2016 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.

  14. Negative sweat tests and cystic fibrosis.

    PubMed Central

    Sarsfield, J K; Davies, J M

    1975-01-01

    Two brothers are described with chronic suppurative pulmonary disease. One has classical cystic fibrosis with complete pancreatic involvement and abnormal sweat test. The other had incomplete pancreatic disease with repeatedly normal sweat tests. The implications of a negative sweat test in patients with cystic fibrosis are discussed. Images FIG. PMID:1147688

  15. Clinical Assessment of Standard and Generalized Autocalibrating Partially Parallel Acquisition Diffusion Imaging: Effects of Reduction Factor and Spatial Resolution

    PubMed Central

    Andre, J.B.; Zaharchuk, G.; Fischbein, N.J.; Augustin, M.; Skare, S.; Straka, M.; Rosenberg, J.; Lansberg, M.G.; Kemp, S.; Wijman, C.A.C.; Albers, G.W.; Schwartz, N.E.; Bammer, R.

    2012-01-01

    BACKGROUND AND PURPOSE PI improves routine EPI-based DWI by enabling higher spatial resolution and reducing geometric distortion, though it remains unclear which of these is most important. We evaluated the relative contribution of these factors and assessed their ability to increase lesion conspicuity and diagnostic confidence by using a GRAPPA technique. MATERIALS AND METHODS Four separate DWI scans were obtained at 1.5T in 48 patients with independent variation of in-plane spatial resolution (1.88 mm2 versus 1.25 mm2) and/or reduction factor (R = 1 versus R = 3). A neuroradiologist with access to clinical history and additional imaging sequences provided a reference standard diagnosis for each case. Three blinded neuroradiologists assessed scans for abnormalities and also evaluated multiple imaging-quality metrics by using a 5-point ordinal scale. Logistic regression was used to determine the impact of each factor on subjective image quality and confidence. RESULTS Reference standard diagnoses in the patient cohort were acute ischemic stroke (n = 30), ischemic stroke with hemorrhagic conversion (n = 4), intraparenchymal hemorrhage (n = 9), or no acute lesion (n = 5). While readers preferred both a higher reduction factor and a higher spatial resolution, the largest effect was due to an increased reduction factor (odds ratio, 47 ± 16). Small lesions were more confidently discriminated from artifacts on R = 3 images. The diagnosis changed in 5 of 48 scans, always toward the reference standard reading and exclusively for posterior fossa lesions. CONCLUSIONS PI improves DWI primarily by reducing geometric distortion rather than by increasing spatial resolution. This outcome leads to a more accurate and confident diagnosis of small lesions. PMID:22403781

  16. Incidental pancreatic cystic neoplasms in an asymptomatic healthy population of 21,745 individuals

    PubMed Central

    Chang, Ye Rim; Park, Joo Kyung; Jang, Jin-Young; Kwon, Wooil; Yoon, Jeong Hee; Kim, Sun-Whe

    2016-01-01

    Abstract Although incidental pancreatic cystic neoplasms are being diagnosed with increasing frequency, little is known about the accurate prevalence of pancreatic cysts in the general population. The aims of this study were to evaluate the crude prevalence rate of pancreatic cystic neoplasms in asymptomatic healthy adults, and calculate the age- and sex-adjusted nationwide prevalence rate. A total of 21,745 asymptomatic individuals who underwent abdominal computed tomography (CT) as a health screening examination were enrolled between 2003 and 2013 at the Seoul National University Hospital Healthcare System Gangnam Center. Nationwide population data of 2010 were collected from the National Statistical Office, Korea. Incidental pancreatic cystic neoplasms were found in 457 individuals whose mean age was 58.7 years. The types of neoplasms were reviewed by 2 separate designated radiologists and the final diagnosis was made as follows: intraductal papillary mucinous neoplasm: 376 (82%), serous cystic neoplasm: 19 (4%), mucinous cystic neoplasm: 7 (2%), and indeterminate cysts: 55 (12%). Eight cases underwent operation. The crude prevalence rate was 2.1% and the age- and sex-adjusted expected nationwide prevalence was 2.2%. The prevalence increased with age. Here, we reported the first large-scale study among the healthy population to find out the prevalence rate of pancreatic cystic neoplasms; the age- and sex-adjusted prevalence was 2.2%, and increased with age. Further investigations regarding the clinical implications of incidental pancreatic neoplasms are necessary. PMID:28002329

  17. A feasibility study of a prototype PET insert device to convert a general-purpose animal PET scanner to higher resolution.

    PubMed

    Wu, Heyu; Pal, Debashish; O'Sullivan, Joseph A; Tai, Yuan-Chuan

    2008-01-01

    We developed a prototype system to evaluate the feasibility of using a PET insert device to achieve higher resolution from a general-purpose animal PET scanner. The system consists of a high-resolution PET detector, a computer-controlled rotation stage, and a custom mounting plate. The detector consists of a cerium-doped lutetium oxyorthosilicate array (12 x 12 crystals, 0.8 x 1.66 x 3.75 mm(3) each) directly coupled to a position-sensitive photomultiplier tube (PS-PMT). The detector signals were fed into the scanner electronics to establish coincidences between the 2 systems. The detector was mounted to a rotation stage that is attached to the scanner via the custom mounting plate after removing the transmission source holder. The rotation stage was concentric with the center of the scanner. The angular offset of the insert detector was calibrated via optimizing point-source images. In all imaging experiments, coincidence data were collected from 9 angles to provide 180 degrees sampling. A (22)Na point source was imaged at different offsets from the center to characterize the in-plane resolution of the insert system. A (68)Ge point source was stepped across the axial field of view to measure the sensitivity of the system. A 23.2-g mouse was injected with 38.5 MBq of (18)F-fluoride and imaged at 3 h after injection for 2 h. The transverse image resolution of the PET insert device ranges from 1.1- to 1.4-mm full width at half maximum (FWHM) without correction for the point-source dimension. This corresponds to approximately 33% improvement over the resolution of the original scanner (1.7- to 1.8-mm FWHM) in 2 of the 3 directions. The sensitivity of the device is 0.064% at the center of the field, 46-fold lower than the sensitivity of an existing animal PET scanner. The mouse bone scan had improved image resolution using the PET insert device over that of the existing animal PET scanner alone. We have demonstrated the feasibility of using a high-resolution insert

  18. [Bronchopulmonary infection in cystic fibrosis].

    PubMed

    Munck, Anne; Bingen, Edouard

    2003-01-15

    Bronchopulmonary infection determines the vital prognosis of the patients with cystic fibrosis. Following Staphylococcus aureus infection, patients are colonized or cocolonized by Pseudomonas aeruginosa, greatly involved in the pulmonary deterioration; intensive antibiotic treatment of primocolonisation helps to prevent or delay chronic colonisation. Chronic colonization needs a rational long term antibiotic strategy to prevent the occurrence of multiresistant germs; antibiotic cures are performed every 3 or 4 months before pulmonary exacerbation symptoms. Antibiotherapy, physiotherapy and nutritional management helps to increase the survival and quality of life.

  19. Endocrine Disorders in Cystic Fibrosis.

    PubMed

    Blackman, Scott M; Tangpricha, Vin

    2016-08-01

    Cystic fibrosis is frequently complicated by endocrine disorders. Diabetes can be expected to affect most with CF and pancreatic insufficiency and varies widely in age of onset, but early identification and treatment improve morbidity and mortality. Short stature can be exacerbated by relative delay of puberty and by use of inhaled corticosteroids. Bone disease in CF causes fragility fractures and should be assessed by monitoring bone mineral density and optimizing vitamin D status. Detecting and managing endocrine complications in CF can reduce morbidity and mortality in CF. These complications can be expected to become more common as the CF population ages. Copyright © 2016 Elsevier Inc. All rights reserved.

  20. Cystic schwannoma of the pelvis.

    PubMed

    Jindal, T; Mukherjee, S; Kamal, M R; Sharma, R K; Ghosh, N; Mandal, S N; Das, A K; Karmakar, D

    2013-01-01

    Schwannomas are benign tumours that arise from the Schwann cells of nerve fibres. They commonly occur in the head and neck, mediastinum and extremities. They are extremely rare in the pelvis. These are usually slow-growing tumours and are often detected incidentally. Preoperative diagnosis is extremely difficult as there are no definitive signs on imaging. Aspiration biopsy is often inconclusive or misleading. Surgical excision is both diagnostic and therapeutic. As these tumours are often large in size, open excision is most commonly performed. We describe a case of a large, cystic schwannoma of the pelvis causing bladder outlet obstruction and bilateral hydroureteronephrosis. Complete surgical excision was performed laparoscopically.

  1. Disease modifying anti-rheumatic drugs in people with cystic fibrosis-related arthritis.

    PubMed

    Thornton, Judith; Rangaraj, Satyapal

    2009-01-21

    Arthritis remains a relatively infrequent complication of cystic fibrosis, but is a cause of significant morbidity when it does occur. Two distinct types of arthritis are described in cystic fibrosis: cystic fibrosis-related arthropathy and hypertrophic osteoarthropathy. Management of arthritis in people with cystic fibrosis is uncertain and complex because of the underlying disease and its treatment. To review the effectiveness and safety of disease-modifying anti-rheumatic drugs (DMARDs) for the management of arthritis related to cystic fibrosis in adults and children. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register which comprises references identified from comprehensive electronic database handsearches of relevant journal and abstract books of conference proceedings.Date of most recent search: May 2008 Randomised controlled trials which compared the efficacy and safety of DMARDs (e.g. methotrexate, gold, sulfasalazine, penicillamine, leflunomide, hydroxychloroquine and newer agents such as biologic disease modifying agents and monoclonal antibodies) with each other, with no treatment or with placebo for cystic fibrosis-related arthropathy or hypertrophic osteoarthropathy. No relevant studies were identified. No studies were included in this review. Although it is generally recognised that cystic fibrosis-related arthritis can be episodic and resolve spontaneously, treatment with analgesics and anti-inflammatory agents may be needed. But when episodic symptoms progress to persistent disease, DMARDs may be needed to limit the course of the disease. It is disappointing that no randomised controlled trials to rigorously evaluate DMARDs could be found. This systematic review has identified the need for a well-designed adequately powered randomised controlled trial to assess the efficacy and safety of DMARDs for the management of cystic fibrosis-related arthropathy and hypertrophic osteoarthropathy in adults and children with

  2. Potential shift correction in multivariate curve resolution of voltammetric data. General formulation and application to some experimental systems.

    PubMed

    Alberich, Arístides; Díaz-Cruz, José Manuel; Ariño, Cristina; Esteban, Miquel

    2008-01-01

    A new mathematical algorithm is proposed to correct the progressive potential shift of some voltammetric signals that decrease the linearity of the data. The corrected data matrix can be further analysed by Multivariate Curve Resolution by Alternating Least Squares (MCR-ALS) and the vector including the potential shift corrections can be fitted to specific equations such as that by DeFord-Hume. A detailed discussion is given on the different cases of potential shift correction, and, in some of them, mathematical simulation is made or experimental systems [Cd(ii)-glutathione and Zn(ii)-glycine] are analysed.

  3. A general strategy for developing cell-permeable photo-modulatable organic fluorescent probes for live-cell super-resolution imaging

    PubMed Central

    Pan, Deng; Hu, Zhe; Qiu, Fengwu; Huang, Zhen-Li; Ma, Yilong; Wang, Yina; Qin, Lingsong; Zhang, Zhihong; Zeng, Shaoqun; Zhang, Yu-Hui

    2014-01-01

    Single-molecule localization microscopy (SMLM) achieves super-resolution imaging beyond the diffraction limit but critically relies on the use of photo-modulatable fluorescent probes. Here we report a general strategy for constructing cell-permeable photo-modulatable organic fluorescent probes for live-cell SMLM by exploiting the remarkable cytosolic delivery ability of a cell-penetrating peptide (rR)3R2. We develop photo-modulatable organic fluorescent probes consisting of a (rR)3R2 peptide coupled to a cell-impermeable organic fluorophore and a recognition unit. Our results indicate that these organic probes are not only cell permeable but can also specifically and directly label endogenous targeted proteins. Using the probes, we obtain super-resolution images of lysosomes and endogenous F-actin under physiological conditions. We resolve the dynamics of F-actin with 10 s temporal resolution in live cells and discern fine F-actin structures with diameters of ~80 nm. These results open up new avenues in the design of fluorescent probes for live-cell super-resolution imaging. PMID:25410769

  4. A Variable-Resolution Stretched-Grid General Circulation Model and Data Assimilation System with Multiple Areas of Interest: Studying the Anomalous Regional Climate Events of 1998

    NASA Technical Reports Server (NTRS)

    Fox-Rabinovitz, Michael S.; Takacs, Lawrence; Govindaraju, Ravi C.; Atlas, Robert (Technical Monitor)

    2002-01-01

    The new stretched-grid design with multiple (four) areas of interest, one at each global quadrant, is implemented into both a stretched-grid GCM (general circulation model) and a stretched-grid data assimilation system (DAS). The four areas of interest include: the U.S./Northern Mexico, the El Nino area/Central South America, India/China, and the Eastern Indian Ocean/Australia. Both the stretched-grid GCM and DAS annual (November 1997 through December 1998) integrations are performed with 50 km regional resolution. The efficient regional down-scaling to mesoscales is obtained for each of the four areas of interest while the consistent interactions between regional and global scales and the high quality of global circulation, are preserved. This is the advantage of the stretched-grid approach. The global variable resolution DAS incorporating the stretched-grid GCM has been developed and tested as an efficient tool for producing regional analyses and diagnostics with enhanced mesoscale resolution. The anomalous regional climate events of 1998 that occurred over the U.S., Mexico, South America, China, India, African Sahel, and Australia are investigated in both simulation and data assimilation modes. Tree assimilated products are also used, along with gauge precipitation data, for validating the simulation results. The obtained results show that the stretched-grid GCM and DAS are capable of producing realistic high quality simulated and assimilated products at mesoscale resolution for regional climate studies and applications.

  5. High resolution general purpose D-layer experiment for EISCAT incoherent scatter radars using selected set of random codes

    NASA Astrophysics Data System (ADS)

    Turunen, T.; Westman, A.; Häggström, I.; Wannberg, G.

    2002-09-01

    The ionospheric D-layer is a narrow bandwidth radar target often with a very small scattering cross section. The target autocorrelation function can be obtained by transmitting a series of relatively short coded pulses and computing the correlation between data obtained from different pulses. The spatial resolution should be as high as possible and the spatial side lobes of the codes used should be as small as possible. However, due to the short pulse repetition period (in the order of milliseconds) at any instant, the radar receives detectable scattered signals not only from the pulse illuminating the D-region but also from 3 5 ambiguous-range pulses, which makes it difficult to produce a reliable estimate near zero lag of the autocorrelation function. A new experimental solution to this measurement problem, using a selected set of 40-bit random codes with 4 µs elements giving 600 m spatial resolution is presented. The zero lag is approximated by dividing the pulse into two 20-bit codes and computing the correlation between those two pulses. The lowest altitudes of the E-layer are measured by dividing the pulse into 5 pieces of 8 bits, which allows for computation of 4 lags. In addition, coherent integration of data from four pulses is used for obtaining separately the autocorrelation function estimate for the lowest altitudes and in cases when the target contains structures with a long coherence time. Design details and responses of the experiment are given, and analysed test data are shown.

  6. Aspergillus infections in cystic fibrosis.

    PubMed

    King, Jill; Brunel, Shan F; Warris, Adilia

    2016-07-05

    Patients with cystic fibrosis (CF) suffer from chronic lung infection and airway inflammation. Respiratory failure secondary to chronic or recurrent infection remains the commonest cause of death and accounts for over 90% of mortality. Bacteria as Staphylococcus aureus, Pseudomonas aeruginosa and Burkholderia cepacia complex have been regarded the main CF pathogens and their role in progressive lung decline has been studied extensively. Little attention has been paid to the role of Aspergillus spp. and other filamentous fungi in the pathogenesis of non-ABPA (allergic bronchopulmonary aspergillosis) respiratory disease in CF, despite their frequent recovery in respiratory samples. It has become more apparent however, that Aspergillus spp. may play an important role in chronic lung disease in CF. Research delineating the underlying mechanisms of Aspergillus persistence and infection in the CF lung and its link to lung deterioration is lacking. This review summarizes the Aspergillus disease phenotypes observed in CF, discusses the role of CFTR (cystic fibrosis transmembrane conductance regulator)-protein in innate immune responses and new treatment modalities. Copyright © 2016. Published by Elsevier Ltd.

  7. Internal irradiation for cystic craniopharyngioma

    SciTech Connect

    Kobayashi, T.; Kageyama, N.; Ohara, K.

    1981-12-01

    The authors report the results of internal irradiation with labeled chromic phosphate (32P) and gold-198 (198Au) colloid in eight cases of cystic craniopharyngiomas. They used a newly developed dosimetric formula, by which the radiation dose at the cyst wall and at any point far from the radioactive source can be calculated. Ten courses of irradiation in eight patients were carried out by injection of either 32P or 198Au colloid into the cyst through an Ommaya drainage system that had been placed at craniotomy. Follow-up studies ranging from 13 to 156 months revealed that all cysts were effectively treated, with elimination of fluid or collapse of the cyst. This was confirmed by Conray cystography and/or computerized tomography. Not only the dose delivered to the wall but also the thickness of the cyst wall and the location of the cyst are important factors in planning internal irradiation. A safe and adequate dose to the cyst wall could range between 9000 to 30,000 rads for craniopharyngioma. This treatment is suitable for large cysts that are thought to be difficult to remove radically, recurrent cysts resistant to previous treatment, or multiple cysts. Internal irradiation may also be applicable in other cystic intracranial tumors if dosimetry is calculated accurately.

  8. A Multi-wavelength High-resolution study of the S255 Star-forming Region: General Structure and Kinematics

    NASA Astrophysics Data System (ADS)

    Zinchenko, I.; Liu, S.-Y.; Su, Y.-N.; Kurtz, S.; Ojha, D. K.; Samal, M. R.; Ghosh, S. K.

    2012-08-01

    We present observational data for two main components (S255IR and S255N) of the S255 high mass star-forming region in continuum and molecular lines obtained at 1.3 mm and 1.1 mm with the Submillimeter Array (SMA), at 1.3 cm with the Very Large Array, and at 23 and 50 cm with the Giant Metrewave Radio Telescope. The angular resolution was from ~2'' to ~5'' for all instruments. With the SMA we detected a total of about 50 spectral lines of 20 different molecules (including isotopologues). About half of the lines and half of the species (in particular N2H+, SiO, C34S, DCN, DNC, DCO+, HC3N, H2CO, H2CS, SO2) have not been previously reported in S255IR and partly in S255N at high angular resolution. Our data reveal several new clumps in the S255IR and S255N areas through their millimeter wave continuum emission. Masses of these clumps are estimated at a few solar masses. The line widths greatly exceed expected thermal widths. These clumps have practically no association with NIR or radio continuum sources, implying a very early stage of evolution. At the same time, our SiO data indicate the presence of high-velocity outflows related to some of these clumps. In some cases, strong molecular emission at velocities of the quiescent gas has no detectable counterpart in the continuum. We discuss the main features of the distribution of NH3, N2H+, and deuterated molecules. We estimate properties of decimeter wave radio continuum sources and their relationship with the molecular material.

  9. Vitamin K and the management of patients with cystic fibrosis.

    PubMed Central

    Durie, P R

    1994-01-01

    OBJECTIVE: To assess the advisability of routine vitamin K supplementation in patients with cystic fibrosis (CF). DATA SOURCES: Studies identified through a MEDLINE search with the use of MeSH terms vitamin K, cystic fibrosis, PIVKA-II (protein induced by vitamin K absence-II), coagulation abnormality and cystic fibrosis, and hepatic disorder and cystic fibrosis. STUDY SELECTION: Six articles published between January 1981 and December 1992 were selected: one general review of vitamin K in infancy and five studies involving clinical trials of vitamin K supplementation or screening for fat-soluble vitamins, vitamin K or PIVKA-II in patients with CF. Review articles on nutrition in patients with CF, technical reports, letters, comments and case studies not bearing directly on these issues were excluded. DATA EXTRACTION: Findings in these articles were analysed and compared to determine whether routine supplementation in all patients with CF is indicated, whether specific subgroups of these patients are susceptible to vitamin K deficiency and areas in which future research is needed. RESULTS: There is no consensus on routine vitamin K supplementation in patients with CF. Studies have found a few cases of vitamin K deficiency among the population of people with CF. In addition, various factors--including pancreatic failure, liver disease, bowel resection and long-term use of antibiotics--can put some of these patients at risk of vitamin K deficiency. CONCLUSIONS: Specific indications for routine vitamin K supplementation in all patients with CF have not yet been identified. Pending further studies, it would be prudent to consider routine supplementation in patients with CF and severe noncholestatic and cholestatic liver disease, major small-bowel resection, pancreatic insufficiency or lung disease necessitating frequent use of antibiotics. A stronger body of evidence is needed as a basis for clinical strategies. PMID:7922929

  10. Estimates of Bottom Flows and Bottom Boundary Layer Dissipation of the Oceanic General Circulation from Global High-Resolution Models

    DTIC Science & Technology

    2009-02-27

    Penduff et , • , ». r HI J m 1 mno m • , -„„,, . . * . , general circulation [e.g., Munk and Wunsch, 1998...model variables and all grid points in the vertical and horizontal directions, because of the unfeasibly large stor- age and analysis computers that

  11. High resolution interpolation of climate scenarios for the conterminous USA and Alaska derived from general circulation model simulations

    Treesearch

    Linda A. Joyce; David T. Price; Daniel W. McKenney; R. Martin Siltanen; Pia Papadopol; Kevin Lawrence; David P. Coulson

    2011-01-01

    Projections of future climate were selected for four well-established general circulation models (GCM) forced by each of three greenhouse gas (GHG) emissions scenarios, namely A2, A1B, and B1 from the Intergovernmental Panel on Climate Change (IPCC) Special Report on Emissions Scenarios (SRES). Monthly data for the period 1961-2100 were downloaded mainly from the web...

  12. Fungi in cystic fibrosis and non-cystic fibrosis bronchiectasis.

    PubMed

    Moss, Richard B

    2015-04-01

    Bronchiectasis is a pathologic bronchial dilatation with loss of function that can result from multiple inflammatory and infectious injuries to the conducting airways of the lung. Molds, particularly the filamentous fungus Aspergillus fumigatus, have been implicated as a common cause of both cystic fibrosis (CF) and non-CF bronchiectasis, the latter primarily in patients with severe asthma. The pathogenesis of mold-associated bronchiectasis is usually due to atopic sensitization to mold allergens in the presence of active chronic endobronchial fungal infection with host innate and adaptive immune deviation to a Th2-dominated inflammation, a condition known as allergic bronchopulmonary aspergillosis (ABPA) (or allergic bronchopulmonary mycosis if a non-Aspergillus mold is implicated). Diagnostic criteria of ABPA continue to evolve, while treatment relies upon downregulation of the allergic inflammatory response with immunomodulatory agents and antifungal pharmacotherapy.

  13. Tropical cyclone activity in a warmer climate as simulated by a high-resolution coupled general circulation model: changes in frequency and air-sea interaction.

    NASA Astrophysics Data System (ADS)

    Scoccimarro, Enrico; Gualdi, Silvio; Navarra, Antonio

    2010-05-01

    This study investigates the possible changes that the greenhouse global warming might generate in the characteristics of the tropical cyclones (TCs). The analysis has been performed using climate scenario simulations carried out with a fully coupled high-resolution global general circulation model (INGV-SXG) with a T106 atmospheric resolution. The capability of the model to reproduce a reasonably realistic TC climatology has been assessed by comparing the model results from a simulation of the XX Century with observations. The model appears to be able to simulate tropical cyclone-like vortices with many features similar to the observed TCs. The simulated TC activity exhibits realistic geographical distribution, seasonal modulation and interannual variability, suggesting that the model is able to reproduce the major basic mechanisms that link the TC occurrence with the large scale circulation. The results from the climate scenarios reveal a substantial general reduction of the TC frequency when the atmospheric CO2 concentration is doubled and quadrupled. The reduction appears particularly evident for the tropical north west Pacific (NWP) and north Atlantic (ATL). In the NWP the weaker TC activity seems to be associated with a reduced amount of convective instabilities. In the ATL region the weaker TC activity seems to be due to both the increased stability of the atmosphere and a stronger vertical wind shear. Despite the generally reduced TC activity, there is evidence of increased rainfall associated with the simulated cyclones. Using the new fully coupled CMCC model (CMCC_MED), with a T159 atmospheric resolution, we found a significant modulation of the Ocean Heat Transport (OHT) induced by the TC activity. Thus the possible changes that greenhouse induced global warming during 21st century might generate in the characteristics of the TC-induced OHT have been analyzed.

  14. Cystic avascular necrosis of the triquetrum.

    PubMed

    Albtoush, Omar M; Esmadi, Mohammad; Al-Omari, Mamoon H

    2013-01-01

    Carpal bones are rarely affected by avascular necrosis (AVN) in the absence of fractures. The lunate is the most frequently affected carpal bone, followed by the scaphoid and the capitate. The triquetrum is rarely affected by AVN. We report a case of multiple cystic changes in the triquetrum in a patient with a history of trauma. He was treated by below elbow Colles plaster cast for 3 months, with no improvement. Cystic changes resulted from irreversible AVN of the triquetrum. This is the first case to be reported in the literature with cystic AVN changes in the triquetrum.

  15. Cystic Pheochromocytoma Presenting as Adrenal Cyst

    PubMed Central

    Abdulsalam, Mohammed Shafi; Satish, Priyanka; Janakiraman, Raghunath Keddy; Singh, Shivshankar

    2016-01-01

    Pheochromocytomas are usually solid tumours. But it can present as cystic lesions in the adrenal gland. Cystic lesions in adrenal gland with hypertension needs attention to rule out pheochromocytoma. If ignored, it may lead to hypertensive emergency, multisystem crisis and death. Early diagnosis with biochemistry, Computed Tomography (CT) or Magnetic Resonance Imaging (MRI) of abdomen, proper functional imaging like Meta Iodo Benzyl Guanidine (MIBG) scan is essential. Proper preoperative preparation is important to prevent hypertensive crisis during and after surgery. We are reporting a case of cystic pheochromocytoma in a young male. PMID:28050427

  16. Cystic Pheochromocytoma Presenting as Adrenal Cyst.

    PubMed

    Abdulsalam, Mohammed Shafi; Ganapathy, Vijaya; Satish, Priyanka; Janakiraman, Raghunath Keddy; Singh, Shivshankar

    2016-11-01

    Pheochromocytomas are usually solid tumours. But it can present as cystic lesions in the adrenal gland. Cystic lesions in adrenal gland with hypertension needs attention to rule out pheochromocytoma. If ignored, it may lead to hypertensive emergency, multisystem crisis and death. Early diagnosis with biochemistry, Computed Tomography (CT) or Magnetic Resonance Imaging (MRI) of abdomen, proper functional imaging like Meta Iodo Benzyl Guanidine (MIBG) scan is essential. Proper preoperative preparation is important to prevent hypertensive crisis during and after surgery. We are reporting a case of cystic pheochromocytoma in a young male.

  17. Pityriasis rubra pilaris in the setting of HIV infection: clinical behaviour and association with explosive cystic acne.

    PubMed

    Martin, A G; Weaver, C C; Cockerell, C J; Berger, T G

    1992-06-01

    The development of pityriasis rubra pilaris (PRP) in three patients with human immunodeficiency virus (HIV) infection is described. Two of the patients had onset of severe generalized cystic acne concomitant with their development of PRP. PRP and acne conglobata should be added to the group of cutaneous disorders that can present in a more virulent manner in the setting of HIV infection. The association of cystic acne with PRP and their response to treatment are discussed.

  18. Heart involvement in cystic fibrosis: A specific cystic fibrosis-related myocardial changes?

    PubMed

    Labombarda, Fabien; Saloux, Eric; Brouard, Jacques; Bergot, Emmanuel; Milliez, Paul

    2016-09-01

    Cystic fibrosis is a complex multi-systemic chronic disease characterized by progressive organ dysfunction with development of fibrosis, possibly affecting the heart. Over the last four decades pathological, experimental, and clinical evidence points towards the existence of a specific myocardial involvement in cystic fibrosis. Multi-modality cardiac imaging, especially recent echocardiographic techniques, evidenced diastolic and/or systolic ventricular dysfunction in cystic fibrosis leading to the concept of a cystic fibrosis-related cardiomyopathy. Hypoxemia and inflammation are among the most important factors for heart involvement in cystic fibrosis. Cystic Fibrosis Transmembrane Regulator was found to be involved in the regulation of cardiomyocyte contraction and may also account for cystic fibrosis-related myocardial dysfunction. This review, mainly focused on echocardiographic studies, seeks to synthesize the existing literature for and against the existence of heart involvement in cystic fibrosis, its mechanisms and prognostic implications. Careful investigation of the heart function may be helpful for risk stratification and therapeutic decisions in patients with cystic fibrosis.

  19. Effects of Tropical Cyclones on Ocean Heat Transport as simulated by a High Resolution Coupled General Circulation Model

    NASA Astrophysics Data System (ADS)

    Scoccimarro, E.; Gualdi, S.; Bellucci, A.; Sanna, A.; Vichi, M.; Manzini, E.; Fogli, P.; Navarra, A.; Oddo, P.

    2010-12-01

    Tropical cyclones (TCs) activity and their relationship with the Northern hemispheric Ocean Heat Transport (OHT) is investigated. The analysis has been performed using 20C3M (20th Century) and A1B (21st Century) IPCC scenario climate simulations obtained running a state-of-the-art atmosphere-ocean-seaice coupled global model, with high-resolution in the atmosphere. The capability of the model to reproduce a realistic TC climatology has been assessed by comparing the model results from the simulation of the 20th Century with observations. The model is able to simulate tropical cyclone-like vortices with many features similar to the observed TCs. The simulated TC activity exhibits realistic structure, geographical distribution and interannual variability, indicating that the model is able to reproduce the major basic mechanisms that link the TC activity with the large scale circulation. The TC-induced ocean cooling is well represented and the TCs activity increases significantly the poleward OHT out of the tropics, but also increases the heat transport into the deep tropics. This effect, investigated looking at the 100 most intense Northern hemisphere TCs, is strongly correlated to the TC-induced momentum flux at the surface of the ocean. TCs frequency and intensity appear to be substantially stationary through the whole 1950- 2069 period. Also the effect of the TCs induced OHT) does not significantly change during the simulated period.

  20. Cystic mesothelioma of the peritoneum.

    PubMed

    Datta, R V; Paty, P B

    1997-10-01

    A 48-year-old man presented with a 3-month history of weight loss and progressive right lower quadrant abdominal pain. His medical history was notable for appendectomy at age 17. Ultrasonography and computed tomography of the abdomen revealed a 12 cm multicystic mass in the right paracolic space. At laparotomy a large serous cyst was found arising from the lateral wall of the cecum, and four additional small cysts were found on the small bowel mesentery, greater omentum, liver capsule, and right hemi-diaphragm. Complete removal of the tumor was accomplished by right colectomy with extraperitoneal dissection of the large cyst and simple excision of the four smaller cysts. Final pathology with immunohistochemical staining confirmed cystic mesothelioma of the peritoneum. In this report we discuss the diagnostic workup and treatment of this rare disease.

  1. Laboratory diagnosis of cystic fibrosis.

    PubMed

    Webster, H L

    1983-01-01

    The demonstration of abnormally high concentrations of electrolytes in eccrine sweat is still the only practical laboratory procedure available for diagnosis of cystic fibrosis. Properly performed, the sweat test is very reliable, but there are many published reports that all of the methods in current use frequently generate incorrect diagnoses. Analysis of potential for error in sweat test methods shows that of the three essential phases involved, stimulation, collection, and analysis, the major cause of intrinsic inaccuracy occurs in the collection process. In this case the problem is due to condensate formation, which leads to the subsequent analysis of nonrepresentative sweat. Human error is also an important cause of false results and is a direct function of the number of critical manual operations involved in the technic. This review provides a critical examination of sweat test methods, identifying problem areas and suggesting ways to improve procedures in the interests of clinically reliable laboratory data in support of diagnosis.

  2. Cystic cerebellar astrocytomas in childhood.

    PubMed

    Griffin, T W; Beaufait, D; Blasko, J C

    1979-07-01

    Thirty-nine patients with low grade cystic cerebellar astrocytomas were treated at the University of Washington and Children's Orthopedic Hospital in Seattle, Washington, between 1955 and 1977; 29 were treated with partial or complete resection alone, and 10 received radiation therapy after various types of surgical procedures. With a mean follow-up time of 7 years, the survival rate for patients who had complete resections of their primary disease was 100%. The relapse-free survival rate was 82%. The relapse-free survival rate for patients treated primarily with partial resection alone was 36%. Postoperative irradiation after partial resection for both primary and recurrent disease resulted in a relapse-free survival rate of 83%. If complete tumor excision is not possible, postoperative radiation therapy is recommended following partial resection.

  3. Chronic pancreatitis and cystic fibrosis

    PubMed Central

    Witt, H

    2003-01-01

    Recent discoveries of trypsinogen and trypsin inhibitor mutations in patients with chronic pancreatitis (CP) support the hypothesis that an inappropriate activation of pancreatic zymogens to active enzymes within the pancreatic parenchyma starts the inflammatory process. Current data suggest that CP may be inherited dominant, recessive, or complex as a result of mutations in the above mentioned or yet unidentified genes. Evaluation of patients with CP should include genetic testing. Cystic fibrosis (CF) is an autosomal recessive inherited disorder caused by mutations in the CF transmembrane conductance regulator (CFTR) gene and is characterised by pancreatic insufficiency and chronic bronchopulmonary infection. The progression and severity of pulmonary disease differs considerably between people with identical CFTR mutations and does not seem to correlate with the type or class of the CFTR mutation. The identification of further disease modifying genetic factors will increase the pathophysiological understanding and may help to identify new therapeutic targets. PMID:12651880

  4. Cystic echinococcosis in southern Brazil.

    PubMed

    de la Rue, Mario L

    2008-01-01

    Cystic echinococcosis (CE) is very common in the southern part of Rio Grande do Sul State where sheep and cattle raising is the most important economical activity. Prevalence in intermediate hosts is well known due to reports in slaughterhouses while human reports are of discussed value. This is due to underreporting of cases and few epidemiological studies make it difficult to assess the situation of echinococcosis in the population. Whereas cattle infection rate is more or less stable around 12% of the slaughter animals, in sheep there has been an increasing number in the last five years. Some efforts have been done to control the zoonosis but no effective results were obtained so far. Probably educational efforts to change human practices (feeding dogs with raw viscera), periodic treatment of dogs with praziquantel and joined actions with slaughterhouses could bring more attention to improve some control measures.

  5. Alcaligenes infection in cystic fibrosis.

    PubMed

    Tan, Kenneth; Conway, Steven P; Brownlee, Keith G; Etherington, Christine; Peckham, Daniel G

    2002-08-01

    The aim of this study was to investigate the effect of chronic Alcaligenes species infection of the respiratory tract on the clinical status of patients with cystic fibrosis. We conducted a retrospective case-controlled study. The microbiological records of all patients attending the Leeds Regional Pediatric and Adult Cystic Fibrosis Units from 1992-1999 were examined. Chronic Alcaligenes infection was defined as a positive sputum culture on at least three occasions over a 6-month period. These patients were compared with controls matched for age, gender, respiratory function, and Pseudomonas aeruginosa infection status. Respiratory function tests, anthropometric data, Shwachman-Kulczycki score, Northern chest x-ray score, intravenous and nebulized antibiotic treatment, and corticosteroid treatment were compared from 2 years before to 2 years after Alcaligenes infection. From a clinic population of 557, 13 (2.3%) fulfilled the criteria for chronic infection. The median age at acquisition of infection was 17.2 years (range, 6.5-33.6). There was no significant difference in the changes of percentage predicted values for FEV(1), FVC, FEF(25-75), or Shwachman-Kulczycki and Northern chest x-ray scores, or in weight, height, and body mass index z-scores between Alcaligenes-infected cases and controls. There was also no significant difference in the use of antibiotics (intravenous and nebulized) or corticosteroids (inhaled and oral). We conclude that in our clinic, chronic infection with Alcaligenes species was uncommon. Chronically infected patients showed no excess deterioration in clinical or pulmonary function status from 2 years before to 2 years after primary acquisition.

  6. Epidural Cystic Spinal Meningioma: A Case Report.

    PubMed

    Zhang, Ji; Chen, Zheng-he; Wang, Zi-feng; Sun, Peng; Jin, Jie-tian; Zhang, Xiang-heng; Zhao, Yi-ying; Wang, Jian; Mou, Yong-gao; Chen, Zhong-ping

    2016-03-01

    Cystic spinal meningioma (CSM) is an uncommon meningioma variant. Extradural CSMs are particularly rare and difficult to distinguish from other intraaxial tumors. This study presents a case of a 36-year-old woman with intraspinal extradual CSM at the thoracolumbar spine. She experienced persistent weakness, progressive numbness, and sensory disturbance in the right lower limb. Magnetic resonance imaging (MRI) of the patient revealed an irregular cystic mass at the thoracic 11 to lumbar 3 levels dorsally. This case was misdiagnosed as other neoplasms prior to surgery because of the atypical radiographic features and location of the tumor. Extradural CSMs should be considered in the differential diagnosis of intraspinal extradural cystic neoplasms. Complete removal of cystic wall provides an optimal outcome, rendering the lesion curable.

  7. [Cystic fibrosis in a woman aged seventy].

    PubMed

    Ras, Janneke E; van Velzen, Edwin; van Berkhout, Ferdinand Teding; van den Brand, Joop J G

    2010-01-01

    A seventy-year-old woman was admitted to hospital with a Staphylococcus aureus respiratory tract infection. She had a history of extensive bronchiectasis and allergic bronchopulmonary aspergillosis (ABPA). Cystic fibrosis (CF) was suspected and cystic fibrosis transmembrane conductance regulator (CFTR) gene analysis showed F508del and R117H-7T mutations. In these mutations there is residual activity in the chloride channel in the cell membrane coded by the CFTR gene. This results in a much milder disease pattern varying from no disease at all to isolated organ disease. This type of disease is known as non-classical cystic fibrosis. In our patient the diagnosis of cystic fibrosis was made exceptionally late in life.

  8. Radiological description of cystic pancreatic tumors.

    PubMed

    Rodríguez Torres, C; Larrosa López, R

    2016-01-01

    Although most cystic pancreatic lesions are pseudocysts, it is important to do a thorough differential diagnosis with true cystic tumors because cystic tumors are potentially malignant. Sometimes computed tomography and magnetic resonance imaging cannot establish the definitive diagnosis, making it necessary to perform other imaging tests such as endoscopic ultrasound, which in addition to morphological information, can also enable cytologic and biochemical analysis of the lesion through puncture and aspiration of its contents. Combining all these findings nearly always provides enough diagnostic information to allow the appropriate approach in each case. This article describes the specific morphological characteristics for each cystic pancreatic tumor on computed tomography, magnetic resonance imaging, and endoscopic ultrasound and reviews the guidelines for managing these types of lesions.

  9. Cervical Cystic Hygroma in an Adult

    PubMed Central

    Derin, Serhan; Şahan, Murat; Dere, Yelda; Çullu, Neşat; Şahan, Leyla

    2014-01-01

    Cystic hygromas/lymphangiomas are extremely rare malformations in adults. They are usually seen in infants and children under 2 years of age. En bloc resection is difficult due to the adhesive characteristics of the tumors. Inadequate surgical intervention often leads to recurrent disease. We report herein the case of a cystic hygroma/lymphangioma that presented as an uncommon mass on the cervical region in an adult, together with its histopathological, radiologic, and operative features. PMID:25548704

  10. Antibiotic-associated colitis and cystic fibrosis.

    PubMed

    Pokorny, C S; Bye, P T; MacLeod, C; Selby, W S

    1992-09-01

    The use of antibiotics in patients with cystic fibrosis is widespread, and fecal carriage of Clostridium difficile occurs in up to 50% of these patients; however, antibiotic-associated colitis appears to be a rare occurrence. The reasons why this is so remain unknown. A case of antibiotic-associated colitis occurring in a patient with cystic fibrosis is described. Possible mechanisms for the rarity of antibiotic-associated colitis are reviewed and implications for prompt diagnosis and therapy are discussed.

  11. Cervical solitary long segment cystic Schwannoma.

    PubMed

    Vikram, Muthusubramanian; Pande, Anil; Vasudevan, Madhabushi Chakravarthy; Ravi, Ramamurthi

    2010-04-01

    Cystic neurofibromas are very rare and are of separate entity; occurrence in the cervical spine extending over a long segment intra-durally is very rare. There is a paucity of literature available on cervical cystic Schwannoma and its MRI characteristics. As the preoperative diagnosis of these tumors helps in planning the surgery for this rare tumor the imaging findings reported herein may aid in the preoperative diagnosis and management.

  12. [Retrocervical cystic hygroma: about 35 cases].

    PubMed

    Masmoudi, Aida; Neji, Kaled; Kacem, Sémia; Boudhraa, Kaled; Jabnoun, Samy; Chelli, Hella; Reziga, Hédi; Zouari, Faouzia; Khrouf, Naima; Chaabouni, Habiba; Siala, Soumaya

    2002-03-01

    Retrocervical cystic hygroma is a congenital defect associated to chromosomic anomalies. We report a retrospective study about 35 cystic hygroma autopsies colliged in C.M.N.T in 10 years. Antenatal sonography has a sensibility 94.5%. Genetic abnormalities dominated by trisomie 13 Turner syndrome dad found in 11.5%. Medical abortion has done in 48.5%. A multidisciplinary management autorized to understand etiopathogeny of this defect.

  13. Colonic disorders in adult cystic fibrosis.

    PubMed

    Chaun, H

    2001-09-01

    By 1996, the median survival of patients with cystic fibrosis (CF) in North America had increased to 31 years. With the markedly improved life expectancy, many CF patients are now adults. There is an associated increased risk of certain colonic disorders, and the emergence of other previously unrecognized disorders, in adult CF patients. The distal intestinal obstruction syndrome (DIOS), which is more common in older patients, is a frequent cause of abdominal pain. Intussusception may complicate DIOS; other differential diagnoses include appendiceal disease, volvolus, Crohn's disease, fibrosing colonopathy and colonic carcinoma. The diagnosis of acute appendicitis, although uncommon in patients with CF, is often delayed, and appendiceal abscess is a frequent complication. The prevalence of Crohn's disease in CF has been shown to be 17 times that of the general population. Right-sided microscopic colitis is a recently recognized entity in CF of uncertain clinical significance. Fibrosing colonopathy has been confined mostly to children with CF, attributed to the use of high strength pancreatic enzyme supplements, but it has been reported in three adults. Nine cases of carcinoma of the large intestine have been reported worldwide, associated with an apparent excess risk of digestive tract cancers in CF. Despite high carrier rates of Clostridium difficile in patients with CF, pseudomembranous colitis is distinctly rare, but severe cases complicated by toxic megacolon have been reported. In these patients, watery diarrhea is often absent. Adult CF patients with refractory or unexplained intestinal symptoms merit thorough investigations.

  14. Adenoid cystic carcinoma of breast: Recent advances

    PubMed Central

    Miyai, Kosuke; Schwartz, Mary R; Divatia, Mukul K; Anton, Rose C; Park, Yong Wook; Ayala, Alberto G; Ro, Jae Y

    2014-01-01

    Adenoid cystic carcinoma (ACC) of the breast is a rare special subtype of breast cancer characterized by the presence of a dual cell population of luminal and basaloid cells arranged in specific growth patterns. Most breast cancers with triple-negative, basal-like breast features (i.e., tumors that are devoid of estrogen receptor, progesterone receptor, and human epidermal growth factor receptor 2 expression, and express basal cell markers) are generally high-grade tumors with an aggressive clinical course. Conversely, while ACCs also display a triple-negative, basal-like phenotype, they are usually low-grade and exhibit an indolent clinical behavior. Many discoveries regarding the molecular and genetic features of the ACC, including a specific chromosomal translocation t(6;9) that results in a MYB-NFIB fusion gene, have been made in recent years. This comprehensive review provides our experience with the ACC of the breast, as well as an overview of clinical, histopathological, and molecular genetic features. PMID:25516849

  15. Records of the General Conference (28th, Paris, France, October 25 to November 16, 1995). Volume 1: Resolutions [and] Volume 2: Reports--Programme Commissions, Administrative Commission, Legal Committee.

    ERIC Educational Resources Information Center

    United Nations Educational, Scientific, and Cultural Organization, Paris (France). General Conference. Cultural Organization, Paris (France). Section for Technical and Vocational Education.

    The records of the 28th UNESCO general conference are divided into three parts. The first part contains the resolutions adopted by the UNESCO General Conference and the list of officers of the General Conference and of the Commissions and Committees. The second part contains the reports of the five UNESCO Program Commissions, the Administrative…

  16. [Cystic fibrosis: how to use pulmonary function tests].

    PubMed

    Counil, F P; Karila, C; Le Bourgeois, M; Matecki, S; Lebras, M N; Couderc, L; Fajac, I; Reynaud-Gaubert, M; Bellet, M; Gauthier, R; Denjean, A

    2007-06-01

    Neonatal screening for cystic fibrosis (CF) leads to early dedicated specialist care for all patients. Pulmonary function tests (PFT) are mandatory for routine monitoring of CF patients. The aim of this article is to review the current guidelines for PFTs in CF, particularly the type of test, the age and the clinical status of the patient. The regular use of spirometry is generally accepted. Many other tests are used but their clinical value in the routine follow-up of CF patients remains to be established. Further efforts should be made to evaluate the value of PFTs in CF, particularly in very young children.

  17. Analysis of cystic fibrosis gene mutations in children with cystic fibrosis and in 964 infertile couples within the region of Basilicata, Italy: a research study

    PubMed Central

    2014-01-01

    Introduction Cystic fibrosis is the most common autosomal recessive genetic disease in the Caucasian population. Extending knowledge about the molecular pathology on the one hand allows better delineation of the mutations in the CFTR gene and the other to dramatically increase the predictive power of molecular testing. Methods This study reports the results of a molecular screening of cystic fibrosis using DNA samples of patients enrolled from January 2009 to December 2013. Patients were referred to our laboratory for cystic fibrosis screening for infertile couples. In addition, we identified the gene mutations present in 76 patients affected by cystic fibrosis in the pediatric population of Basilicata. Results In the 964 infertile couples examined, 132 subjects (69 women and 63 men) resulted heterozygous for one of the CFTR mutations, with a recurrence of carriers of 6.85%. The recurrence of carriers in infertile couples is significantly higher from the hypothetical value of the general population (4%). Conclusions This study shows that in the Basilicata region of Italy the CFTR phenotype is caused by a small number of mutations. Our aim is to develop a kit able to detect not less than 96% of CTFR gene mutations so that the relative risk for screened couples is superimposable with respect to the general population. PMID:25304080

  18. COMPARISON BETWEEN SEDATION AND GENERAL ANESTHESIA FOR HIGH RESOLUTION COMPUTED TOMOGRAPHIC CHARACTERIZATION OF CANINE IDIOPATHIC PULMONARY FIBROSIS IN WEST HIGHLAND WHITE TERRIERS.

    PubMed

    Roels, Elodie; Couvreur, Thierry; Farnir, Frédéric; Clercx, Cécile; Verschakelen, Johny; Bolen, Géraldine

    2017-02-23

    Canine idiopathic pulmonary fibrosis is a progressive interstitial lung disease mainly affecting West Highland white terriers. Thoracic high-resolution computed tomographic (T-HRCT) findings for Canine idiopathic pulmonary fibrosis acquired under general anesthesia have been described previously. However, the use of general anesthesia may be contraindicated for some affected dogs. Sedation may allow improved speed and safety, but it is unknown whether sedation would yield similar results in identification and grading of Canine idiopathic pulmonary fibrosis lesions. The aim of this prospective, observational, method-comparison, case-control study was to compare findings from T-HRCT images acquired under sedation versus general anesthesia for West Highland white terriers affected with Canine idiopathic pulmonary fibrosis (n = 11) and age-matched controls (n = 9), using the glossary of terms of the Fleischner Society and a scoring system. Ground-glass opacity was identified in all affected West Highland white terriers for both sedation and general anesthesia acquisitions, although the Ground-glass opacity extent varied significantly between the two acquisitions (P < 0.001). Ground-glass opacity was the sole lesion observed in control dogs (n = 6), but was less extensive compared with affected West Highland white terriers. Identification and grading of a mosaic attenuation pattern differed significantly between acquisitions (P < 0.001). Identification of lesions such as consolidations, nodules, parenchymal and subpleural bands, bronchial wall thickening, and bronchiectasis did not differ between acquisitions. The present study demonstrated that T-HRCT obtained under sedation may provide different information than T-HRCT obtained under general anesthesia for identification and grading of some Canine idiopathic pulmonary fibrosis lesions, but not all of them. These differences should be taken into consideration when general anesthesia is contraindicated and sedation is

  19. Abnormal Ion Permeation through Cystic Fibrosis Respiratory Epithelium

    NASA Astrophysics Data System (ADS)

    Knowles, M. R.; Stutts, M. J.; Spock, A.; Fischer, N.; Gatzy, J. T.; Boucher, R. C.

    1983-09-01

    The epithelium of nasal tissue excised from subjects with cystic fibrosis exhibited higher voltage and lower conductance than tissue from control subjects. Basal sodium ion absorption by cystic fibrosis and normal nasal epithelia equaled the short-circuit current and was amiloride-sensitive. Amiloride induced chloride ion secretion in normal but not cystic fibrosis tissue and consequently was more effective in inhibiting the short-circuit current in cystic fibrosis epithelia. Chloride ion-free solution induced a smaller hyperpolarization of cystic fibrosis tissue. The increased voltage and amiloride efficacy in cystic fibrosis reflect absorption of sodium ions across an epithelium that is relatively impermeable to chloride ions.

  20. Cystic tumors of the pancreas: imaging and management.

    PubMed

    Dewhurst, Catherine E; Mortele, Koenraad J

    2012-05-01

    Cystic tumors of the pancreas are a subset of rare pancreatic tumors that vary from benign to malignant. Many have specific imaging findings that allow them to be differentiated from each other. This article (1) reviews the imaging features of the common cystic pancreatic lesions, including serous microcystic adenoma, mucinous cystic tumor, intraductal papillary mucinous tumor, and solid pseudopapillary tumor, and including the less common lesions such as cystic endocrine tumors, cystic metastases, cystic teratomas, and lymphangiomas; and (2) provides comprehensive algorithms on how to manage the individual lesions, with recommendations on when to reimage patients. Copyright © 2012 Elsevier Inc. All rights reserved.

  1. Development of cystic embryoid bodies with visceral yolk-sac-like structures from mouse embryonic stem cells using low-adherence 96-well plate.

    PubMed

    Yasuda, Emiko; Seki, Yuji; Higuchi, Takatoshi; Nakashima, Fumio; Noda, Tomozumi; Kurosawa, Hiroshi

    2009-04-01

    Cystic embryoid bodies with visceral yolk-sac-like structure (cystic EB-Vs) are used as a model for the study of early extraembryonic tissue formation containing visceral endoderm-like derivatives. In this study, we optimized the cell density of embryonic stem (ES) cells for developing cystic EB-Vs in a low-adherence 96-well plate. When ES cells were seeded at a density of 4000 cells/well, the cystic EB-Vs were most efficiently developed from ES cells via forming multicellular spherical aggregates called embryoid bodies (EBs). The suspension culture in the low-adherence plate was preferable for developing EBs into cystic EB-Vs rather than the attachment culture in the plate coated with 0.1% gelatin. The seeding cell density of 4000 cells/well was always superior to 1000 cells/well in the efficiency of cystic EB-V development. Because the high-cell density culture generally raises the limitation of oxygen and nutrient supplies, we investigated the effects of low-oxygen and low-nutrient conditions on the development of cystic EB-Vs. It was found that low oxygen tension was not a factor for promoting the development of cystic EB-Vs. It was suggested that a low-nutrient medium is preferred for developing cystic EB-Vs rather than a sufficient-nutrient medium. In conclusion, the suspension culture in the low-adherence 96-well plate seeded with 4000 ES cells/well was optimum for developing cystic EB-Vs. The low-nutrient condition may be one of the factors for promoting the development of cystic EB-Vs.

  2. A canine case with cystic meningioma showing miraculous reduction of the cystic lesion

    PubMed Central

    WADA, Masae; HASEGAWA, Daisuke; HAMAMOTO, Yuji; ASAI, Atsushi; SHOUJI, Akane; CHAMBERS, James; UCHIDA, Kazuyuki; FUJITA, Michio

    2015-01-01

    A 12-year-old spayed female Labrador retriever was presented with forebrain signs. Brain MRI revealed a huge cystic lesion with the thickened falx in the frontal region. The brain parenchyma surrounding the lesion showed significant signs of a mass effect and also increased intracranial pressure. However, the dog suddenly became lucid after about two weeks, and an MRI scan one month after the initial study revealed a dramatically shrunken cystic lesion. The dog survived for over a year until it was euthanized for other reasons, and the brain lesion was diagnosed as a cystic meningioma histologically. To the authors’ knowledge, this is the first report that described the reduction of the cystic lesion of a cystic meningioma in dogs. PMID:26256491

  3. High HCV subtype heterogeneity in a chronically infected general population revealed by high-resolution hepatitis C virus subtyping.

    PubMed

    Rodriguez-Frias, F; Nieto-Aponte, L; Gregori, J; Garcia-Cehic, D; Casillas, R; Tabernero, D; Homs, M; Blasi, M; Vila, M; Chen, Q; Vargas, V; Castells, Ll; Viladomiu, Ll; Genesca, J; Minguez, B; Augustin, S; Riveiro-Barciela, M; Carbonell, J; Perales, C; Soria, M E; Asensio, M; Llorens, M; Ordeig, L; Godoy, C; Buti, M; Esteban, R; Pumarola, T; Esteban, J I; Quer, J

    2017-10-01

    This study aimed to characterize the chronically infected general hepatitis C virus (HCV) population in Barcelona using a highly sensitive subtyping method that can identify the 67 recognized HCV subtypes and diagnose mixed infection by various genotypes/subtypes in a single individual. The resulting information has implications for selecting optimal direct-acting antiviral (DAA) treatment for each patient and establishing public healthcare policies in our setting. Consecutive HCV patients (treatment-naïve or interferon-based failures) attending Vall d'Hebron Hospital outpatient clinics from February 2015 to May 2016 (N=1473) were included in the study. Patient samples were characterized using HCV subtyping by next-generation ultra-deep pyrosequencing. The following genotypes (G) were found: G1 (1126/1473 (76.4%)), G4 (145/1473 (9.8%)), G3 (135/1473 (9.2%)), G2 (51/1473 (3.5%)), and G5 (1/1473 (0.1%)). Twenty-two subtypes were seen: 1b (790/1473 (53.6%)), 1a (332/1473 (22.5%)), 3a (133/1473 (9.0%)), 4d (105/1473 (7.1%)), 4a (29/1473 (2.0%)), and 2c (25/1473 (1.7%)), with 16 low-prevalence subtypes accounting for the remaining 3.0% (44/1473). There was a worrisome 1.0% (15/1473) of mixed infections. G2 (51/1473 (3.5%)) showed a high level of heterogeneity. Analyses by age groups showed a predominance of G1b over G1a (428/506 (84.6%) vs. 24/506 (4.7%)) in patients born before 1950 (N=506/1473), and similar percentages of these subtypes in those born between 1951 and 1975 (N=834/1473) (315/834, 37.8% vs. 266/834, 31.9%) and after 1976 (N=133/1473) (47/133, 35.3% vs. 42/133, 31.6%). Subtype distribution showed a higher level of heterogeneity than was expected, particularly for G2. Prevalence of mixed infections was around 1%. HCV subtype distribution related to patient age group suggested that patients born from 1936 to 1975 in our setting should undergo screening for the infection. Next-generation sequencing enabled better classification of candidates for DAA

  4. Cystic adrenal lesions: focus on pediatric population (a review)

    PubMed Central

    CARSOTE, MARA; GHEMIGIAN, ADINA; TERZEA, DANA; GHEORGHISAN-GALATEANU, ANCUTA AUGUSTINA; VALEA, ANA

    2017-01-01

    Background and aim The cysts may potentially affect any organ; adrenals cysts are rare. This is a review of the literature regarding adrenal cysts, focusing on children and young adults. General data Three major types have been described: pure cysts (endothelial, epithelial, and hemorrhagic or pseudocyst), parasitic (as hydatid) cysts and cystic part of a tumour (most frequent are neuroblastoma, ganglioneuroma, pheocromocytoma, and teratoma). The complications are: bleeding, local pressure effects; infection; rupture (including post-traumatic); arterial hypertension due to renal vessels compression. Adrenal hemorrhage represents a particular condition associating precipitating factors such as: coagulation defects as Factor IX or X deficiency, von Willebrand disease, thrombocytopenia; antiphospholipid syndrome; previous therapy with clopidogrel or corticosteroids; the rupture of a prior tumour. At birth, the most suggestive features are abdominal palpable mass, anemia, and persistent jaundice. Adrenal insufficiency may be found especially in premature delivery. The hemorrhage is mostly self-limiting. Antenatal ultrasound diagnosis of a cyst does not always predict the exact pathology result. The most important differential diagnosis of adrenal hemorrhage/hemorrhagic cyst is cystic neuroblastoma which is highly suggestive in the presence of distant metastases and abnormal catecholamine profile. The major clue to differentiate the two conditions is the fact that the tumor is stable or increases over time while the adrenal hemorrhage is expected to remit within one to two weeks. Conclusion Pediatric adrenal cysts vary from simple cysts with a benign behavior to neoplasia- related lesions displaying severe prognosis as seen in cystic neuroblastoma. A multidisciplinary team is required for their management which is conservative as close follow-up or it makes necessary different surgical procedures in cases with large masses or if a malignancy suspicion is presented

  5. Neural correlates of specific and general Pavlovian-to-Instrumental Transfer within human amygdalar subregions: a high-resolution fMRI study.

    PubMed

    Prévost, Charlotte; Liljeholm, Mimi; Tyszka, Julian M; O'Doherty, John P

    2012-06-13

    It is widely held that the interaction between instrumental and Pavlovian conditioning induces powerful motivational biases. Pavlovian-Instrumental Transfer (PIT) is one of the key paradigms demonstrating this effect, which can further be decomposed into a general and specific component. Although these two forms of PIT have been studied at the level of amygdalar subregions in rodents, it is still unknown whether they involve different areas of the human amygdala. Using a high-resolution fMRI (hr-fMRI) protocol optimized for the amygdala in combination with a novel free operant task designed to elicit effects of both general and specific PIT, we demonstrate that a region of ventral amygdala within the boundaries of the basolateral complex and the ventrolateral putamen are involved in specific PIT, while a region of dorsal amygdala within the boundaries of the centromedial complex is involved in general PIT. These results add to a burgeoning literature indicating different functional contributions for these different amygdalar subregions in reward-processing and motivation.

  6. Altitude distribution of tropospheric ozone over the Northern Hemisphere during 1996, simulated with a chemistry-general circulation model at two different horizontal resolutions

    NASA Astrophysics Data System (ADS)

    Kentarchos, A. S.; Roelofs, G. J.; Lelieveld, J.

    2001-01-01

    The spatial/temporal variability of the vertical distribution of tropospheric ozone in the Northern Hemisphere (NH) over a period of 1 year (1996) is studied with a coupled chemistry-general circulation model. The model is used at two different horizontal resolutions (T30: 3.75°×3.75° and T63: 1.875°×1.875°) and is nudged towards European Centre for Medium Range Weather Forecasts analyses for 1996, using a four-dimensional assimilation technique (newtonian relaxation), to enable direct comparisons of observations and model results. Overall, the model reproduces satisfactorily the magnitude and seasonal variability of the vertical ozone distribution observed at six selected locations. Discrepancies occur, however, at remote locations in the subtropical Atlantic and tropical Pacific where ozone concentrations throughout the free troposphere are overestimated by the fourth version of the European Centre Hamburg Model (ECHAM4)-T30. A considerable improvement is evident at T63, which can be attributed, at least partially, to less efficient transport of ozone precursors from the polluted continents at higher resolution. In the upper troposphere/tropopause region, short-term ozone variations are better reproduced at higher resolution. The origin of tropospheric ozone is examined by decomposing its seasonal variation in the model into ozone from the stratosphere and ozone produced within the troposphere. Differences in the NH annual tropospheric ozone budget for 1996, between T30 and T63 mean amounts are relatively small. The tropospheric ozone budget is dominated by photochemical production and destruction (2716 and 2684 Tg, respectively), while the net ozone flux from the stratosphere is estimated to be 436 Tg, and dry deposition is estimated to be 487 Tg.

  7. Immunosuppressive drug therapy for preventing rejection following lung transplantation in cystic fibrosis.

    PubMed

    Saldanha, Ian J; Akinyede, Oluwaseun; Robinson, Karen A

    2015-11-10

    For people with cystic fibrosis and advanced pulmonary damage, lung transplantation is an available and viable option. However, graft rejection is an important potential consequence after lung transplantation. Immunosuppressive therapy is needed to prevent episodes of graft rejection and thus subsequently reduce morbidity and mortality in this population. There are a number of classes of immunosuppressive drugs which act on different components of the immune system. There is considerable variability in the use of immunosuppressive agents after lung transplantation in cystic fibrosis. While much of the research in immunosuppressive drug therapy has focused on the general population of lung transplant recipients, little is known about the comparative effectiveness and safety of these agents in people with cystic fibrosis. This is an update of a previously published review. To assess the effects of individual drugs or combinations of drugs compared to placebo or other individual drugs or combinations of drugs in preventing rejection following lung transplantation in people with cystic fibrosis. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register and scanned references of the potentially eligible study. We also searched the www.clinicaltrials.gov registry to obtain information on unpublished and ongoing studies.Date of latest search: 19 May 2015. Randomised and quasi-randomised studies. We independently assessed the studies identified from our searches for inclusion in the review. Should eligible studies be identified and included in future updates of the review, we will independently extract data and assess the risk of bias. While two studies met our inclusion criteria, we did not include them in the review because the investigators of the studies did not report any information specific to people with cystic fibrosis. Our attempts to obtain this information have not yet been successful. We will include any provided data in future

  8. A General Method for Constructing Atomic-Resolution RNA Ensembles using NMR Residual Dipolar Couplings: The Basis for Inter-helical Motions Revealed

    PubMed Central

    Salmon, Loïc; Bascom, Gavin; Andricioaei, Ioan; Al-Hashimi, Hashim M.

    2013-01-01

    The ability to modulate alignment and measure multiple independent sets of NMR residual dipolar couplings (RDCs) has made it possible to characterize internal motions in proteins at atomic resolution and with timescale sensitivity ranging from picoseconds up to milliseconds. The application of such methods to the study of RNA dynamics, however, remains fundamentally limited by the inability to modulate alignment and by strong couplings between internal and overall motions that complicate the quantitative interpretation of RDCs. Here, we address this problem by showing that RNA alignment can be generally modulated, in a controlled manner, by variable elongation of A-form helices, and that the information contained within the measured RDCs can be extracted even in the presence of strong couplings between motions and overall alignment via structure-based prediction of alignment. Using this approach, four RDC data sets, and a broad conformational pool obtained from a 8.2µs molecular dynamics simulation, we successfully construct and validate an atomic resolution ensemble of HIV-1 TAR RNA. This ensemble reveals local motions in and around the bulge involving changes in stacking and hydrogen bonding interactions, which are undetectable by traditional spin relaxation and that drive global changes in inter-helical orientation. This new approach broadens the scope of using RDCs in characterizing the dynamics of nucleic acids. PMID:23473378

  9. An Ultrahigh Resolution Structure of TEM-1 beta-Lactamase Suggests a Role for Glu166 as the General Base in Acylation

    SciTech Connect

    Minasov, George; Wang, Xiaojun; Shoichet, Brian K.

    2010-03-08

    Although TEM-1 {beta}-lactamase is among the best studied enzymes, its acylation mechanism remains controversial. To investigate this problem, the structure of TEM-1 in complex with an acylation transition-state analogue was determined at ultrahigh resolution (0.85 {angstrom}) by X-ray crystallography. The quality of the data was such as to allow for refinement to an R-factor of 9.1% and an R{sub free} of 11.2%. In the resulting structure, the electron density features were clear enough to differentiate between single and double bonds in carboxylate groups, to identify multiple conformations that are occupied by residues and loops, and to assign 70% of the protons in the protein. Unexpectedly, even at pH 8.0 where the protein was crystallized, the active site residue Glu166 is clearly protonated. This supports the hypothesis that Glu166 is the general base in the acylation half of the reaction cycle. This structure suggests that Glu166 acts through the catalytic water to activate Ser70 for nucleophilic attack on the {beta}-lactam ring of the substrate. The hydrolytic mechanism of class A {beta}-lactamases, such as TEM-1, appears to be symmetrical, as are the serine proteases. Apart from its mechanistic implications, this atomic resolution structure affords an unusually detailed view of the structure, dynamics, and hydrogen-bonding networks of TEM-1, which may be useful for the design of inhibitors against this key antibiotic resistance target.

  10. Emerging treatments in cystic fibrosis.

    PubMed

    Jones, Andrew M; Helm, Jennifer M

    2009-10-01

    There are a number of potential drugs for the treatment of cystic fibrosis (CF) currently undergoing clinical studies. A number of antibacterials formulated for delivery by inhalation are at various stages of study; these include dry-powder inhaler versions of colistin, tobramycin and ciprofloxacin, and formulations of azteonam, amikacin, levofloxacin, ciprofloxacin and fosfomycin/tobramycin for nebulization. Clinical trials of anti-inflammatory agents, including glutathione, phosphodiesterase-5 inhibitors such as sildenafil, oral acetylcysteine, simvastatin, methotrexate, docosahexaenoic acid, hydroxychloroquine, pioglitazone and alpha1-antitrypsin, are ongoing. Ion channel modulating agents, such as lancovutide (Moli1901, duramycin) and denufosol, which activate alternate (non-CF transmembrane regulator [CFTR]) chloride channels, and GS 9411, a sodium channel antagonist, are now at the stages of clinical study and if successful, will offer a new category of therapeutic agent for the treatment of CF. Correction of the underlying gene effect, either by agents that help to correct the dysfunctional CFTR, such as ataluren, VX-770 and VX-809, or by gene transfer (gene therapy), is a particularly exciting prospect as a new therapy for CF and clinical studies are ongoing. This article reviews the exciting potential drug treatments for CF currently being evaluated in clinical studies, and also highlights some of the challenges faced by research and clinical teams in assessing the efficacy of potential new therapies for CF.

  11. Cystic fibrosis: a clinical view.

    PubMed

    Castellani, Carlo; Assael, Baroukh M

    2017-01-01

    Cystic fibrosis (CF), a monogenic disease caused by mutations in the CFTR gene on chromosome 7, is complex and greatly variable in clinical expression. Airways, pancreas, male genital system, intestine, liver, bone, and kidney are involved. The lack of CFTR or its impaired function causes fat malabsorption and chronic pulmonary infections leading to bronchiectasis and progressive lung damage. Previously considered lethal in infancy and childhood, CF has now attained median survivals of 50 years of age, mainly thanks to the early diagnosis through neonatal screening, recognition of mild forms, and an aggressive therapeutic attitude. Classical treatment includes pancreatic enzyme replacement, respiratory physiotherapy, mucolitics, and aggressive antibiotic therapy. A significant proportion of patients with severe symptoms still requires lung or, less frequently, liver transplantation. The great number of mutations and their diverse effects on the CFTR protein account only partially for CF clinical variability, and modifier genes have a role in modulating the clinical expression of the disease. Despite the increasing understanding of CFTR functioning, several aspects of CF need still to be clarified, e.g., the worse outcome in females, the risk of malignancies, the pathophysiology, and best treatment of comorbidities, such as CF-related diabetes or CF-related bone disorder. Research is focusing on new drugs restoring CFTR function, some already available and with good clinical impact, others showing promising preliminary results that need to be confirmed in phase III clinical trials.

  12. [Azithromycin therapy in cystic fibrosis].

    PubMed

    Máiz Carro, Luis; Cantón Moreno, Rafael

    2004-03-06

    Progressive lung disease, caused by chronic endobronchial colonization, is the major cause of morbidity and mortality in patients with cystic fibrosis (CF). Several pathogens, including Staphylococcus aureus and Pseudomonas aeruginosa are responsible for this effect. The steadily improving prognosis of CF has been attributed to the use of antibiotics with activity against these organisms. Despite a significant increase in the amount of published material demonstrating the potential role of macrolide antibiotics as antiinflammatory agents and their effects on bacterial virulence, their mechanism of action in CF patients is still unknown. Although there is a limited number of clinical trials assessing the efficacy and safety of azithromycin (AZM) in CF, increasing evidence suggests that 3 to 6-month AZM treatment in CF patients is safe and well tolerated. This treatment results in clinical improvement, decreasing the number of pulmonary exacerbations and increasing pulmonary function. Therefore, chronic treatment with AZM should be considered in CF patients added to conventional therapy. Clinical experience with macrolides other than AZM in CF patients is very limited.

  13. Cystic hygroma colli as the first echographic sign of the fetal akinesia sequence.

    PubMed

    Witters, I; Moerman, P H; Van Assche, F A; Fryns, J P

    2001-01-01

    We report first trimester cystic hygroma colli with subsequent resolution and development of a fetal akinesia deformation sequence. Neuropathological examination of the brain showed intra- and extracellular white matter edema while spinal cord, peripheral nerves and muscles were normal. Hygroma colli as the first echographic sign of subsequent severe fetal akinesia sequence without muscular dystrophy as seen in the Lethal Multiple Pterygium syndrome has not been previously reported.

  14. Rehabilitation with Cystic Fibrosis: From Utopia to Reality.

    ERIC Educational Resources Information Center

    Goldberg, Richard T.; And Others

    1980-01-01

    The paper dispels some of the myths regarding cystic fibrosis (a genetic metabolism disorder), provides information on the latest developments in rehabilitation, summarizes research in the field, and projects future needs of the patient with cystic fibrosis. (SBH)

  15. CXCR4+ granulocytes reflect fungal cystic fibrosis lung disease.

    PubMed

    Carevic, Melanie; Singh, Anurag; Rieber, Nikolaus; Eickmeier, Olaf; Griese, Matthias; Hector, Andreas; Hartl, Dominik

    2015-08-01

    Cystic fibrosis airways are frequently colonised with fungi. However, the interaction of these fungi with immune cells and the clinical relevance in cystic fibrosis lung disease are incompletely understood.We characterised granulocytes in airway fluids and peripheral blood from cystic fibrosis patients with and without fungal colonisation, non-cystic fibrosis disease controls and healthy control subjects cross-sectionally and longitudinally and correlated these findings with lung function parameters.Cystic fibrosis patients with chronic fungal colonisation by Aspergillus fumigatus were characterised by an accumulation of a distinct granulocyte subset, expressing the HIV coreceptor CXCR4. Percentages of airway CXCR4(+) granulocytes correlated with lung disease severity in patients with cystic fibrosis.These studies demonstrate that chronic fungal colonisation with A. fumigatus in cystic fibrosis patients is associated with CXCR4(+) airway granulocytes, which may serve as a potential biomarker and therapeutic target in fungal cystic fibrosis lung disease.

  16. Rehabilitation with Cystic Fibrosis: From Utopia to Reality.

    ERIC Educational Resources Information Center

    Goldberg, Richard T.; And Others

    1980-01-01

    The paper dispels some of the myths regarding cystic fibrosis (a genetic metabolism disorder), provides information on the latest developments in rehabilitation, summarizes research in the field, and projects future needs of the patient with cystic fibrosis. (SBH)

  17. Cystic precursors to invasive pancreatic cancer

    PubMed Central

    Matthaei, Hanno; Schulick, Richard D.; Hruban, Ralph H.; Maitra, Anirban

    2011-01-01

    Improvements in the sensitivity and quality of cross-sectional imaging have led to increasing numbers of patients being diagnosed with cystic lesions of the pancreas. In parallel, clinical, radiological, pathological and molecular studies have improved the systems for classifying these cysts. Patients with asymptomatic serous cystic neoplasms can be managed conservatively with regular monitoring; however, the clinical management of patients with intraductal papillary mucinous neoplasms and mucinous cystic neoplasms is far more challenging, as it is difficult to determine whether these lesions will progress to malignancy. Fortunately, prospective studies have helped to establish that proposed clinical and radiological criteria (the Sendai guidelines) can be used to guide the care of patients with cystic lesions of the pancreas. Despite this progress in imaging and clinical guidelines, sensitive and specific tests have not yet been developed that can reliably predict the histology and biological properties of a cystic lesion. Such biomarkers are urgently needed, as noninvasive precursors of pancreatic cancer are curable, while the vast majority of invasive pancreatic adenocarcinomas are not. PMID:21383670

  18. Cystic duct carcinoma mimicking a middle bile duct tumour

    PubMed Central

    Francisco, Elsa; Mendes, Miguel; Vale, Sílvio; Esteves, Joana

    2015-01-01

    Cystic duct carcinoma was defined by Farrar as a tumour restricted to the cystic duct, making it a rare disease. The authors describe a case of a cystic duct carcinoma that fulfils Farrar’s strict diagnostic criteria and that became clinically relevant by compressing the common hepatic duct, thus causing cholestasis. A cholecystectomy was performed with en bloc resection of the cystic and extrahepatic bile duct with a regional lymphadenectomy. PMID:25819819

  19. Challenges in pulmonary fibrosis. 3: Cystic lung disease.

    PubMed

    Cosgrove, Gregory P; Frankel, Stephen K; Brown, Kevin K

    2007-09-01

    Cystic lung disease is a frequently encountered problem caused by a diverse group of diseases. Distinguishing true cystic lung disease from other entities, such as cavitary lung disease and emphysema, is important given the differing prognostic implications. In this paper the features of the cystic lung diseases are reviewed and contrasted with their mimics, and the clinical and radiographic features of both diffuse (pulmonary Langerhans' cell histiocytosis and lymphangioleiomyomatosis) and focal or multifocal cystic lung disease are discussed.

  20. Challenges in pulmonary fibrosis · 3: Cystic lung disease

    PubMed Central

    Cosgrove, Gregory P; Frankel, Stephen K; Brown, Kevin K

    2007-01-01

    Cystic lung disease is a frequently encountered problem caused by a diverse group of diseases. Distinguishing true cystic lung disease from other entities, such as cavitary lung disease and emphysema, is important given the differing prognostic implications. In this paper the features of the cystic lung diseases are reviewed and contrasted with their mimics, and the clinical and radiographic features of both diffuse (pulmonary Langerhans' cell histiocytosis and lymphangioleiomyomatosis) and focal or multifocal cystic lung disease are discussed. PMID:17726170

  1. Cystic Meningioma Masquerading as a Metastatic Tumor: A Case Report

    PubMed Central

    Ramanathan, Nithya; Kamaruddin, Khairul Azmi; Othman, Aizzat; Mustafa, Fadhli; Awang, Mohamed Saufi

    2016-01-01

    Cystic meningioma is a rare form of intracranial meningioma. Meningiomas are typically solid tumors but may rarely have cystic components. The diagnosis of cystic meningioma is clinically challenging as the finding of multiple intra-axial tumors, including metastatic tumors, is relatively common. We report a case of cystic meningioma initially diagnosed as a metastatic tumor from a recurrence of acute lymphoid leukemia. However, postoperative histopathological examination demonstrated an atypical meningioma. PMID:27418876

  2. [Cystic fibrosis in a 70-year-old woman].

    PubMed

    Bruun, Lene Søndberg; Jensen, Michael Skov

    2002-05-06

    Cystic fibrosis is usually diagnosed in early childhood, and patients rarely live beyond the age of 40. We present a case of a 70-year-old woman, in whom cystic fibrosis was diagnosed with the rare mutation, R117C. Cystic fibrosis should therefore also be considered in older patients.

  3. Living with Cystic Fibrosis: A Guide for the Young Adult.

    ERIC Educational Resources Information Center

    Cystic Fibrosis Foundation, Atlanta, GA.

    Intended for the young adult with cystic fibrosis, the booklet provides information on dealing with problems and on advances in treatment and detection related to the disease. Addressed are the following topics: description of cystic fibrosis; inheritance of cystic fibrosis; early diagnosis; friends, careers, and other matters; treatment;…

  4. Living with Cystic Fibrosis: A Guide for the Young Adult.

    ERIC Educational Resources Information Center

    Cystic Fibrosis Foundation, Atlanta, GA.

    Intended for the young adult with cystic fibrosis, the booklet provides information on dealing with problems and on advances in treatment and detection related to the disease. Addressed are the following topics: description of cystic fibrosis; inheritance of cystic fibrosis; early diagnosis; friends, careers, and other matters; treatment;…

  5. Nebulized and oral thiol derivatives for pulmonary disease in cystic fibrosis.

    PubMed

    Nash, Edward F; Stephenson, Anne; Ratjen, Felix; Tullis, Elizabeth

    2009-01-21

    Cystic fibrosis is an inherited condition resulting in thickened, sticky respiratory secretions. Respiratory failure, due to recurrent pulmonary infection and inflammation, is the most common cause of mortality. Muco-active therapies (e.g. dornase alfa and nebulized hypertonic saline) may decrease sputum viscosity, increase airway clearance of sputum, reduce infection and inflammation and improve lung function. Thiol derivatives, either oral or nebulized, have shown benefit in other respiratory diseases. Their mode of action is likely to differ according to the route of administration. There are several thiol derivatives, and it is unclear which of these may be beneficial in cystic fibrosis. To evaluate the efficacy and safety of nebulized and oral thiol derivatives in people with cystic fibrosis. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register, comprising references identified from comprehensive electronic database searches, hand searches of relevant journals, abstract books and conference proceedings.Most recent search: November 2008. Randomized and quasi-randomized controlled trials comparing nebulized or oral thiol derivatives to placebo or another thiol derivative in people with cystic fibrosis. The authors independently assessed trials for inclusion, analysed methodological quality and extracted data. Searches identified 18 trials; eight (seven older than 10 years) (234 participants) are included. Three trials of nebulized thiol derivatives were identified (one compared 20% n-acetylcysteine to 2% n-acetylcysteine; another compared sodium-2-mercaptoethane sulphonate to 7% hypertonic saline; and another compared glutathione to 4% hypertonic saline). Although generally well-tolerated with no significant adverse effects, there was no evidence of significant clinical benefit in our primary outcomes in participants receiving these treatments.Five studies of oral thiol derivatives were identified. Three studies compared n

  6. Nebulized and oral thiol derivatives for pulmonary disease in cystic fibrosis.

    PubMed

    Tam, Julian; Nash, Edward F; Ratjen, Felix; Tullis, Elizabeth; Stephenson, Anne

    2013-07-12

    Cystic fibrosis is an inherited condition resulting in thickened, sticky respiratory secretions. Respiratory failure, due to recurrent pulmonary infection and inflammation, is the most common cause of mortality. Muco-active therapies (e.g. dornase alfa and nebulized hypertonic saline) may decrease sputum viscosity, increase airway clearance of sputum, reduce infection and inflammation and improve lung function. Thiol derivatives, either oral or nebulized, have shown benefit in other respiratory diseases. Their mode of action is likely to differ according to the route of administration. There are several thiol derivatives, and it is unclear which of these may be beneficial in cystic fibrosis. To evaluate the efficacy and safety of nebulized and oral thiol derivatives in people with cystic fibrosis. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register, comprising references identified from comprehensive electronic database searches, hand searches of relevant journals, abstract books and conference proceedings.Most recent search: 13 June 2013.We also conducted a PubMed search on 26 February 2013 for relevant published articles. Randomized and quasi-randomized controlled trials comparing nebulized or oral thiol derivatives to placebo or another thiol derivative in people with cystic fibrosis. The authors independently assessed trials for inclusion, analysed risk of bias and extracted data. Searches identified 23 trials; nine trials (255 participants) are included, of these seven trials are more than 10 years old. Three trials of nebulized thiol derivatives were identified (one compared 20% N-acetylcysteine to 2% N-acetylcysteine; another compared sodium-2-mercaptoethane sulphonate to 7% hypertonic saline; and another compared glutathione to 4% hypertonic saline). Although generally well-tolerated with no significant adverse effects, there was no evidence of significant clinical benefit in our primary outcomes in participants receiving

  7. Non-cystic fibrosis bronchiectasis: its diagnosis and management.

    PubMed

    Stafler, Patrick; Carr, Siobhán B

    2010-06-01

    An increase in the frequency of diagnosing non-cystic fibrosis bronchiectasis in children is due to heightened awareness of the disease and the wider availability of high-resolution computed tomography. The most common underlying conditions leading to bronchiectasis include infections, immunodeficiency, aspiration and primary ciliary dyskinesia. Treatment centres on airway clearance with aggressive antibiotic regimens and physiotherapy; more specific approaches are available for some of the underlying conditions. A high index of suspicion that a child may have underlying bronchiectasis must be maintained in the presence of prolonged or recurrent "wet/productive" cough. The classic definition of bronchiectasis is of irreversible bronchial dilatation; however, at the milder end of the spectrum it appears that radiographic changes may be reversible. Untreated, in its severest form bronchiectasis can progress to end stage pulmonary failure in adult life. In this article, we review its pathogenesis and diagnosis and the evidence base for available treatments.

  8. Florid cystic endosalpingiosis of the uterus

    PubMed Central

    Heatley, M; Russell, P

    2001-01-01

    A 73 year old woman presented with a right sided adnexal cystic mass. At laparotomy, this proved to be a benign serous ovarian cyst and an aggregation of thin walled subserosal and soft tissue cysts and spongy nodules up to 16 mm in diameter involving the side wall of the uterus and adjacent parametrium. These were removed by total abdominal hysterectomy and bilateral salpingo-oophorectomy. Histologically, the cystic spaces and smaller acini were lined by benign tubo-endometrioid epithelium, with smaller areas typical of serous differentiation and rare microfoci of endocervical-type mucinous epithelium. These features indicated multidirectional Mullerian differentiation in a process that, overall, was consistent with so called florid cystic endosalpingiosis. This lesion is to be distinguished from other benign conditions including multicystic mesothelioma, endometriosis, endocervicosis, florid deep glands of the uterine cervix, and deep Nabothian cysts of the uterine cervix. Key Words: uterus • endosalpingiosis • cysts PMID:11328842

  9. [Cystic lesions--diagnosis and conservative treatment].

    PubMed

    Olas, Jacek; Bucka, Jolanta; Dworak, Agata; Golański, Bartłomiej

    2003-01-01

    Effective treatment of cystic lesions that we can apply in the outpatient clinic is the target for seeking a new solution in finding treatment that will produce a better percentage of recovered patients. At this moment non-surgical treatment of cystic lesions is compare with traditional methods of treatment i.e. cyst content aspiration and injection of steroids, with methods which lead to obliteration of cyst lumen and in this way closing the space being the reservoir for the cyst content. Local application of hialuronidase, aspiration, and then steroid injection is gaining more and more attention of clinicians. We also pay much more attention to local fibrinogen injection as a safe and effective method of treatment, which can be useful in the treatment of cystic lesions and topical treatment of bursitis.

  10. A rare case of bilateral cystic partially differentiated nephroblastoma recurring as bilateral cystic Wilms tumour.

    PubMed

    Kurian, Jujju Jacob; Ninan, Pradeep Joseph

    2015-04-15

    Childhood cystic partially differentiated nephroblastoma (CPDN) is an uncommon renal neoplasm. Bilateral CPDN or CPDN co-existing with a cystic nephroma/Wilms tumour is extremely rare. Treatment of CPDN is by complete surgical excision. Although local recurrences are uncommon, distant metastases have not been described. We present a case of bilateral CPDN that, after complete excision, recurred as bilateral cystic Wilms tumour. To the best of our knowledge, this is the first reported case in the literature where a bilateral CPDN has recurred as bilateral Wilms tumour.

  11. Cystic adrenal lesions: focus on pediatric population (a review).

    PubMed

    Carsote, Mara; Ghemigian, Adina; Terzea, Dana; Gheorghisan-Galateanu, Ancuta Augustina; Valea, Ana

    2017-01-01

    The cysts may potentially affect any organ; adrenals cysts are rare. This is a review of the literature regarding adrenal cysts, focusing on children and young adults. Three major types have been described: pure cysts (endothelial, epithelial, and hemorrhagic or pseudocyst), parasitic (as hydatid) cysts and cystic part of a tumour (most frequent are neuroblastoma, ganglioneuroma, pheocromocytoma, and teratoma). The complications are: bleeding, local pressure effects; infection; rupture (including post-traumatic); arterial hypertension due to renal vessels compression. Adrenal hemorrhage represents a particular condition associating precipitating factors such as: coagulation defects as Factor IX or X deficiency, von Willebrand disease, thrombocytopenia; antiphospholipid syndrome; previous therapy with clopidogrel or corticosteroids; the rupture of a prior tumour. At birth, the most suggestive features are abdominal palpable mass, anemia, and persistent jaundice. Adrenal insufficiency may be found especially in premature delivery. The hemorrhage is mostly self-limiting. Antenatal ultrasound diagnosis of a cyst does not always predict the exact pathology result. The most important differential diagnosis of adrenal hemorrhage/hemorrhagic cyst is cystic neuroblastoma which is highly suggestive in the presence of distant metastases and abnormal catecholamine profile. The major clue to differentiate the two conditions is the fact that the tumor is stable or increases over time while the adrenal hemorrhage is expected to remit within one to two weeks. Pediatric adrenal cysts vary from simple cysts with a benign behavior to neoplasia- related lesions displaying severe prognosis as seen in cystic neuroblastoma. A multidisciplinary team is required for their management which is conservative as close follow-up or it makes necessary different surgical procedures in cases with large masses or if a malignancy suspicion is presented. Recently, laparoscopic approach is regarded as

  12. Cystic angiomatosis, a heterogeneous condition

    PubMed Central

    Najm, Aurélie; Soltner-Neel, Elise; Le Goff, Benoît; Guillot, Pascale; Maugars, Yves; Berthelot, Jean-Marie

    2016-01-01

    Abstract Background: Cystic angiomatosis (CA) is a rare disorder causing bony cysts. It displays some similarity to Gorham–Stout disease (GSD), but has a much better local prognosis, despite the larger number of cysts. These 2 conditions also differ in terms of their location, visceral involvement, and response to treatment. Methods: We report 4 cases of CA, including 1 sclerosing form, which we compare with cases from a literature review performed with PRISMA methodology. Results: We reviewed 38 articles describing 44 other patients. Mean age at diagnosis for the 48 patients (our 4 patients + the 44 from the review) was 22.5 years, and 28 of the patients were men. The femur was involved in 81% (n = 39), the pelvis in 73% (n = 35), the humerus in 52% (n = 25), the skull in 48% (n = 23), and the vertebrae in 44% (n = 21). Visceral lymphangiomatosis (either clinical, or detected on autopsy) was also reported in 35% (n = 18) of the patients. The spleen was the most frequently involved organ (n = 12), followed by the lungs and pleura (n = 8). Liver cysts and/or chylothorax were rarely reported (5 cases), but were invariably fatal. Radiation therapy on bone or soft tissue masses was ineffective, as was interferon alpha, in the 2 patients in which this drug was tested. The efficacy of bisphosphonate was at best equivocal. Conclusion: The progression of CA is unpredictable and treatments effective against GSD, such as bisphosphonates and radiotherapy, have proved ineffective for this condition. New treatments are thus urgently required. PMID:27787381

  13. Venous thromboembolism in cystic fibrosis.

    PubMed

    Takemoto, Clifford M

    2012-02-01

    The incidence of venous thromboembolism (VTE) is increasing in the pediatric population. Individuals with cystic fibrosis (CF) have an increased risk of thrombosis due to central venous catheters (CVCs), as well as acquired thrombophilia secondary to inflammation, or deficiencies of anticoagulant proteins due to vitamin K deficiency and/or liver dysfunction. CVC-associated thrombosis commonly results in line occlusion, but may develop into serious life-threatening conditions such as deep venous thrombosis (DVT), superior vena cava syndrome or pulmonary embolism (PE). Post-thrombotic syndrome (PTS) may be a long complication. Local occlusion of the catheter tip may be managed with instillation of thrombolytics (such as tPA) within the lumen of the catheter; however, CVC-associated thrombosis involving the proximal veins is most often is treated with systemic anticoagulation. Initial treatment with heparin is a standard approach, but thrombolytic therapy, which may carry higher bleeding risks, should be considered for life and limb threatening episodes of VTE. Recommended duration of anticoagulation with low molecular weight heparin (LMWH) or warfarin ranges from 3 to 6 months for major removable thrombotic risks; longer anticoagulation is considered for recurrent thrombosis, major persistent thrombophilia, or the continued presence of a major risk factor such as a CVC. While CVCs are the most common risk for development of VTE in children, studies have not demonstrated a clear benefit with routine use of systemic thromboprophylaxis. The incidence and risk factors of VTE in CF patients will be reviewed and principles of diagnosis and management will be summarized.

  14. Cystic fibrosis in adult age.

    PubMed

    Lerín, M; Prados, C; Martínez, M T; Maíz, L; Girón, R; Solé, A; Cabanillas, J J; Alvarez-Sala, R

    2014-01-01

    To know the prevalence of the patients diagnosed of cystic fibrosis (CF) older than 18 years old of five specific Spanish Units and to analyze their clinical, genetic and microbiological characteristics. Observational, cross-sectional, descriptive study of patients diagnosed with CF at age or older than 18 years. The variables analyzed were: current age, age at diagnosis, sex, nationality, lung function parameters, pathologies presented at diagnosis, microbiological features and genetic findings. Eigthy nine patients (14.8% of the total of 600 CF patients followed at the participating units), of which 45 patients were female (50.6%) and 44 were males (49.4%), were included with a mean age at diagnosis of 36.4 years. Eigthy one patients (91%) were Spaniards. The sweat test was diagnostic in 77 (86.5%) of the patients studied. The sweat test was diagnostic in 77 of the 89 patients studied (86.5%). The most frequently detected mutations were F508del/other and G542X/other, and the most frequent clinical findings at diagnosis were the presence of bronchiectasis in 33 patients (37.1%) followed by sterility in 12 patients (13.5%). The most common colonizing organisms were meticillin-sensitive Staphylococcus aureus (S.aureus) (23.6%) and Pseudomonas aeruginosa (P. aeruginosa) (13.5%). Most patients presented a mild obstructive ventilatory defect and had no pancreatic involvement. The sweat test used to be indeterminate. CF is also a disease which diagnosis can be in adulthood. CF patients diagnosed in adulthood have a mild lung function and lower incidence of pancreatic involvement, so their prognosis tends to be favorable. Copyright © 2014 Elsevier España, S.L. All rights reserved.

  15. Cystic echinococcosis in Southern Israel.

    PubMed

    Ben-Shimol, Shalom; Sagi, Orli; Houri, Ohad; Bazarsky, Elina; Berkowitz, Anat; Bulkowstein, Shlomi; Barrett, Chiya; Greenberg, David

    2016-01-01

    The aim of this retrospective, population-based study was to characterize demographically and clinically cystic-echinococcosis (CE) in southern Israel, between 2005 and 2012. Newly-diagnosed (nd-CE) and past-diagnosed (pd-CE, diagnosed before the study) cases were defined. Two populations live in southern-Israel, receiving medical treatment at a single hospital: the Jewish and the Bedouin populations (resembling resource-rich and resource-poor populations, respectively). 126 CE cases were identified; 55 nd-CE and 71 pd-CE. Mean annual nd-CE incidence per 100,000 in the Bedouin and Jewish populations were 2.7 ± 1.2 and 0.4 ± 0.3, respectively (P<0.001). None of the Bedouin and 86.5% of the Jewish patients were born outside Israel. Liver and lung involvement were recorded in 85.7% and 15.1% of overall-CE, respectively. Abdominal pain, cough, fever, eosinophilia and asymptomatic disease were documented in 63.6%, 32.7%, 27.3%, 41.5% and 12.7% of nd-CE, respectively. Serology sensitivity for first test and any positive test were 67.3% and 83.3%, respectively. Computed tomography, ultrasonography and X-ray diagnosis were documented in 79.2%, 58.4% and 17.0% of overall-CE, respectively, with ultrasonography mainly used in liver-CE and X-ray in lung-CE. Treatment included surgery and albendazole in 50.0% and 55.3% of CE, respectively. We conclude that CE is endemic in southern-Israel among the Bedouin population, while disease is probably mainly imported in the Jewish population. Liver involvement and eosinophilia rates were high compared with those of other endemic regions, possibly due to differences in the timing of diagnosis. These findings may help developing treatment and prevention strategies.

  16. Enteral tube feeding for cystic fibrosis.

    PubMed

    Morton, Alison; Wolfe, Susan

    2015-04-09

    Enteral tube feeding is routinely used in many cystic fibrosis centres when oral dietary and supplement intake has failed to achieve an adequate nutritional status. The use of this method of feeding is assessed on an individual basis taking into consideration the patients age and clinical status. To examine the evidence that in people with cystic fibrosis, supplemental enteral tube feeding improves nutritional status, respiratory function, and quality of life without significant adverse effects. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register which comprises references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings. We also contacted the companies that market enteral feeds and reviewed their databases.Date of the most recent search of the Group's Cystic Fibrosis Trials Register: 13 February 2015.Date of the most recent hand search of PubMed and conference abstract books: 13 February 2015. All randomised controlled trials comparing supplemental enteral tube feeding for one month or longer with no specific intervention in people with cystic fibrosis. The searches identified 38 trials; however, none were eligible for inclusion in this review. There are no trials included in this review. Supplemental enteral tube feeding is widely used throughout the world to improve nutritional status in people with cystic fibrosis. The methods mostly used, nasogastric or gastrostomy feeding, are expensive and may have a negative effect on self-esteem and body image. Reported use of enteral tube feeding suggests that it results in nutritional and respiratory improvement; but, efficacy has not been fully assessed by randomised controlled trials. It is acknowledged, however, that performing a randomised controlled trial would be difficult due to the ethics of withholding an intervention in a group of patients whose nutritional status necessitates it.

  17. Liver Disease in Cystic Fibrosis: an Update

    PubMed Central

    Parisi, Giuseppe Fabio; Di Dio, Giovanna; Franzonello, Chiara; Gennaro, Alessia; Rotolo, Novella; Lionetti, Elena; Leonardi, Salvatore

    2013-01-01

    Context Cystic fibrosis (CF) is the most widespread autosomal recessive genetic disorder that limits life expectation amongst the Caucasian population. As the median survival has increased related to early multidisciplinary intervention, other manifestations of CF have emergedespecially for the broad spectrum of hepatobiliary involvement. The present study reviews the existing literature on liver disease in cystic fibrosis and describes the key issues for an adequate clinical evaluation and management of patients, with a focus on the pathogenetic, clinical and diagnostic-therapeutic aspects of liver disease in CF. Evidence Acquisition A literature search of electronic databases was undertaken for relevant studies published from 1990 about liver disease in cystic fibrosis. The databases searched were: EMBASE, PubMed and Cochrane Library. Results CF is due to mutations in the gene on chromosome 7 that encodes an amino acidic polypeptide named CFTR (cystic fibrosis transmembrane regulator). The hepatic manifestations include particular changes referring to the basic CFTR defect, iatrogenic lesions or consequences of the multisystem disease. Even though hepatobiliary disease is the most common non-pulmonary cause ofmortalityin CF (the third after pulmonary disease and transplant complications), only about the 33%ofCF patients presents clinically significant hepatobiliary disease. Conclusions Liver disease will have a growing impact on survival and quality of life of cystic fibrosis patients because a longer life expectancy and for this it is important its early recognition and a correct clinical management aimed atdelaying the onset of complications. This review could represent an opportunity to encourage researchers to better investigate genotype-phenotype correlation associated with the development of cystic fibrosis liver disease, especially for non-CFTR genetic polymorphisms, and detect predisposed individuals. Therapeutic trials are needed to find strategies of

  18. Enteral tube feeding for cystic fibrosis.

    PubMed

    Conway, Steven; Morton, Alison; Wolfe, Susan

    2012-12-12

    Enteral tube feeding is routinely used in many cystic fibrosis centres when oral dietary and supplement intake has failed to achieve an adequate nutritional status. The use of this method of feeding is assessed on an individual basis taking into consideration the patients age and clinical status. To examine the evidence that in people with cystic fibrosis, supplemental enteral tube feeding improves nutritional status, respiratory function, and quality of life without significant adverse effects. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register which comprises references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings. We also contacted the companies that market enteral feeds and reviewed their databases.Date of the most recent search of the Group's Cystic Fibrosis Trials Register: 03 September 2012.Date of the most recent hand search of PubMed and conference abstract books: 15 June 2012. All randomised controlled trials comparing supplemental enteral tube feeding for one month or longer with no specific intervention in people with cystic fibrosis. Thirty-one trials were identified by the searches; however, none were eligible for inclusion in this review. There are no trials included in this review. Supplemental enteral tube feeding is widely used throughout the world to improve nutritional status in people with cystic fibrosis. The methods mostly used, nasogastric or gastrostomy feeding, are expensive and may have a negative effect on self-esteem and body image. Reported use of enteral tube feeding suggests that it results in nutritional and respiratory improvement; but, efficacy has not been fully assessed by randomised controlled trials. It is acknowledged, however, that performing a randomised controlled trial would be difficult due to the ethics of withholding an intervention in a group of patients whose nutritional status

  19. The Cystic Fibrosis of Exocrine Pancreas

    PubMed Central

    Wilschanski, Michael

    2013-01-01

    The cystic fibrosis transmembrane conductance regulator (CFTR) protein is highly expressed in the pancreatic duct epithelia, and permits anions and water to enter the ductal lumen. This results in an increased volume of alkaline fluid allowing the highly concentrated proteins secreted by the acinar cells to remain in a soluble state. This work will expound on the pathophysiology and pathology caused by the malfunctioning CFTR protein with special reference to ion transport and acid-base abnormalities both in humans and animal models. We will also discuss the relationship between cystic fibrosis (CF) and pancreatitis, and outline present and potential therapeutic approaches in CF treatment relevant to the pancreas. PMID:23637307

  20. Cardiac lymphangioma presenting as intrapericardial cystic mass.

    PubMed

    Lone, Nazir A; Naikoo, Bashir A; Khan, Naseer A

    2016-01-01

    Cystic lymphangioma usually confined to head and neck is a well-recognized tumor that occurs during childhood. However, a cardiac lymphangioma is exceptionally uncommon and a particularly rare form of disease. We report a case of cystic lymphangioma arising from the right ventricular wall, and presenting as pericardial mass in a young female, who presented with a history of exercise intolerance in the form of breathlessness on exertion and palpitations. The management of such a case was a difficult task; however, she underwent near total resection of the mass, and is doing well for the last 2 years.

  1. Genetics of Cystic Fibrosis: Clinical Implications.

    PubMed

    Egan, Marie E

    2016-03-01

    Cystic fibrosis (CF) is a common life-shortening autosomal recessive genetic disorder caused by mutations in the gene that encodes for the cystic fibrosis transmembrane conductance regulator protein (CFTR). Almost 2000 variants in the CFTR gene have been identified. The mutational classes are based on the functional consequences on CFTR. New therapies are being developed to target mutant CFTR and restore CFTR function. Understanding specific CF genotypes is essential for providing state-of-the art care to patients. In addition to the variation in CFTR genotype, there are several modifier genes that contribute to the respiratory phenotype.

  2. Diagnosis of Adult Patients with Cystic Fibrosis.

    PubMed

    Nick, Jerry A; Nichols, David P

    2016-03-01

    The diagnosis of cystic fibrosis (CF) is being made with increasing frequency in adults. Patients with CF diagnosed in adulthood typically present with respiratory complaints, and often have recurrent or chronic airway infection. At the time of initial presentation individuals may appear to have clinical manifestation limited to a single organ, but with subclinical involvement of the respiratory tract. Adult-diagnosed patients have a good response to CF center care, and newly available cystic fibrosis transmembrane receptor-modulating therapies are promising for the treatment of residual function mutation, thus increasing the importance of the diagnosis in adults with unexplained bronchiectasis.

  3. Precision genomic medicine in cystic fibrosis

    PubMed Central

    Chang, Eugene H.; Zabner, Joseph

    2015-01-01

    The successful application of precision genomic medicine requires an understanding of how a person’s genome can influence their disease phenotype and how medical therapies can provide personalized therapy to one’s genotype. In this review, we highlight advances in precision genomic medicine in cystic fibrosis (CF), a classic autosomal recessive genetic disorder. We discuss genotype-phenotype correlations in CF, genetic and environmental modifiers of disease, and pharmacogenetic therapies that target specific genetic mutations thereby addressing the primary defect of cystic fibrosis. PMID:26073768

  4. Sonographic-pathologic correlation of complex cystic breast lesions

    PubMed Central

    Pongrattanaman, Saravech; Prueksadee, Jenjeera

    2013-01-01

    Objective To understand the pathologic basis for sonographic features of complex cystic lesions. Methods From 2 646 female patients underwent breast sonography at King Chulalongkorn Memorial Hospital from January 2005 through December 2010, 103 cystic lesions were included. Pathologic confirmation was performed by fine-needle aspiration (n=42), core needle biopsy (n=6), excision (n=54) and mastectomy (n=1). Complex cystic breast masses were classified into 3 types as followings; thick outer wall and/or thick internal septa (type I); thick septation and thick wall were defined as equal or more than 0.5 cm, masses containing mixed cystic and solid components (at least 50% of cystic component) (type II), predominantly solid with eccentric cystic foci (at least 50% of solid component) (type III). Results In 103 complex cystic masses, there are 27 lesions (26%) classified as type I cystic breast masses, 37 lesions (36%) as type II cystic breast masses and 39 lesions (38%) type III cystic breast masses, 26 lesions (25.2%) are proved to be malignant. All of type I cystic breast masses in our study are benign, and 14 (38%) of type II cystic breast masses and 12 lesions (31%) of type III cystic breast lesions are proved to be malignant. Conclusions Type II and III lesions should suggest possibility of malignancy and biopsy should be performed in all lesions. All type I lesion in this study are benign. None of other parameters we included in this study (size or margin) can effectively differentiate between benign or malignant cystic breast lesions. Also, grading of the malignant lesions by using type of cystic breast mass cannot be applied.

  5. Global distribution of gravity wave fields and their seasonal dependence in the Martian atmosphere simulated in a high-resolution general circulation model

    NASA Astrophysics Data System (ADS)

    Kuroda, Takeshi; Medvedev, Alexander; Yiğit, Erdal; Hartogh, Paul

    2016-10-01

    Gravity waves (GWs) are small-scale atmospheric waves generated by various geophysical processes, such as topography, convection, and dynamical instability. On Mars, several observations and simulations have revealed that GWs strongly affect temperature and wind fields in the middle and upper atmosphere. We have worked with a high-resolution Martian general circulation model (MGCM), with the spectral resolution of T106 (horizontal grid interval of ~67 km), for the investigations of generation and propagation of GWs. We analyzed for three kinds of wavelength ranges, (1) horizontal total wavenumber s=21-30 (wavelength λ~700-1000 km), (2) s=31-60 (λ~350-700 km), and (3) s=61-106 (λ~200-350 km). Our results show that shorter-scale harmonics progressively dominate with height during both equinox and solstice. We have detected two main sources of GWs: mountainous regions and the meandering winter polar jet. In both seasons GW energy in the troposphere due to the shorter-scale harmonics is concentrated in the low latitudes in a good agreement with observations. Orographically-generated GWs contribute significantly to the total energy of disturbances, and strongly decay with height. Thus, the non-orographic GWs of tropospheric origin dominate near the mesopause. The vertical fluxes of wave horizontal momentum are directed mainly against the larger-scale wind. Mean magnitudes of the drag in the middle atmosphere are tens of m s-1 sol-1, while instantaneously they can reach thousands of m s-1 sol-1, which results in an attenuation of the wind jets in the middle atmosphere and in tendency of their reversal.

  6. A general protocol of ultra-high resolution MR angiography to image the cerebro-vasculature in 6 different rats strains at high field.

    PubMed

    Pastor, Géraldine; Jiménez-González, María; Plaza-García, Sandra; Beraza, Marta; Padro, Daniel; Ramos-Cabrer, Pedro; Reese, Torsten

    2017-09-01

    Differences in the cerebro-vasculature among strains as well as individual animals might explain variability in animal models and thus, a non-invasive method tailored to image cerebral vessel of interest with high signal to noise ratio is required. Experimentally, we describe a new general protocol of three-dimensional time-of-flight magnetic resonance angiography to visualize non-invasively the cerebral vasculature in 6 different rat strains. Flow compensated angiograms of Sprague Dawley, Wistar Kyoto, Lister Hooded, Long Evans, Fisher 344 and Spontaneous Hypertensive Rat strains were obtained without the use of contrast agents. At 11.7T using a repetition time of 60ms, an isotropic resolution of up to 62μm was achieved; total imaging time was 98min for a 3D data set. The visualization of the cerebral arteries was improved by removing extra-cranial vessels prior to the calculation of maximum intensity projection to obtain the angiograms. Ultimately, we demonstrate that the newly implemented method is also suitable to obtain angiograms following middle cerebral artery occlusion, despite the presence of intense vasogenic edema 24h after reperfusion. The careful selection of the excitation profile and repetition time at a higher static magnetic field allowed an increase in spatial resolution to reliably detect of the hypothalamic artery, the anterior choroidal artery as well as arterial branches of the peri-amygdoidal complex and the optical nerve in six different rat strains. MR angiography without contrast agent can be utilized to study cerebro-vascular abnormalities in various animal models. Copyright © 2017 The Authors. Published by Elsevier B.V. All rights reserved.

  7. Detection of cystic structures using pulsed ultrasonically induced resonant cavitation

    NASA Technical Reports Server (NTRS)

    Bar-Cohen, Yoseph (Inventor); Kovach, John S. (Inventor)

    2002-01-01

    Apparatus and method for early detection of cystic structures indicative of ovarian and breast cancers uses ultrasonic wave energy at a unique resonance frequency for inducing cavitation in cystic fluid characteristic of cystic structures in the ovaries associated with ovarian cancer, and in cystic structures in the breast associated with breast cancer. Induced cavitation bubbles in the cystic fluid implode, creating implosion waves which are detected by ultrasonic receiving transducers attached to the abdomen of the patient. Triangulation of the ultrasonic receiving transducers enables the received signals to be processed and analyzed to identify the location and structure of the cyst.

  8. Oscillating devices for airway clearance in people with cystic fibrosis.

    PubMed

    Morrison, Lisa; Agnew, Jennifer

    2014-07-20

    Chest physiotherapy is widely prescribed to assist the clearance of airway secretions in people with cystic fibrosis. Oscillating devices generate intra- or extra-thoracic oscillations orally or external to the chest wall. Internally they create variable resistances within the airways, generating controlled oscillating positive pressure which mobilises mucus. Extra-thoracic oscillations are generated by forces outside the respiratory system, e.g. high frequency chest wall oscillation. To identify whether oscillatory devices, oral or chest wall, are effective for mucociliary clearance and whether they are equivalent or superior to other forms of airway clearance in the successful management of secretions in people with cystic fibrosis. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register comprising references identified from comprehensive electronic database searches and hand searches of relevant journals and abstract books of conference proceedings. Latest search of the Cystic Fibrosis Trials Register: 13 January 2014. Randomised controlled studies and controlled clinical studies of oscillating devices compared with any other form of physiotherapy in people with cystic fibrosis. Single-treatment interventions (therapy technique used only once in the comparison) were excluded. Two authors independently applied the inclusion criteria to publications and assessed the quality of the included studies. The searches identified 68 studies with a total of 288 references; 35 studies (total of 1050 participants) met the inclusion criteria. Studies varied in duration from up to one week to one year; 20 of the studies were cross-over in design. The studies also varied in type of intervention and the outcomes measured, furthermore data were not published in sufficient detail in most of these studies, so meta-analysis was limited. Few studies were considered to have a low risk of bias in any domain. It is not possible to blind participants and

  9. Cystic hypersecretory ductal carcinoma of the breast: a rare cause of cystic breast mass.

    PubMed

    Song, Sun Wha; Whang, In Yong; Chang, Eun Deok

    2011-11-01

    We present the case of a surgically confirmed, invasive, cystic hypersecretory ductal carcinoma (CHDC) of the breast in a 43-year-old woman. The initial sonography showed a complex cyst, which required a core biopsy; however, the diagnosis was delayed as the patient refused to undergo the biopsy and the cyst decreased in size, as seen on follow-up sonography. Excision biopsy was performed, and invasive CHDC was diagnosed after regrowth of the cystic lesion. Meticulous sonographic evaluation of a cystic breast mass is always important, and pathology confirmation must be considered if the lesion shows features suspicious for malignancy, as a CHDC could be the cause of a cystic breast mass.

  10. Psychological interventions for cystic fibrosis.

    PubMed

    Glasscoe, C A; Quittner, A L

    2003-01-01

    As survival estimates for cystic fibrosis (CF) steadily increase long-term management has become an important focus for intervention. Psychological interventions are largely concerned with emotional and social adjustments, adherence to treatment and quality of life, however no systematic review of such interventions has been undertaken for this disease. To describe the extent and quality of effectiveness studies utilising psychological interventions for CF and whether these interventions provide significant psychosocial and physical benefits in addition to standard care. Relevant trials were identified from searches of Ovid MEDLINE, the Cochrane trial registers for CF and Depression, Anxiety and Neurosis Groups and PsychINFO; unpublished trials were located through professional networks and Listserves. Most recent search: April 2003. This review included RCTs and quasi-randomised trials. Study participants were children and adults diagnosed with CF, and their immediate family members. Psychological interventions were from a broad range of modalities and outcomes were primarily psychosocial, although physical outcomes and cost effectiveness were also considered. Two reviewers independently selected relevant trials and assessed their methodological quality. For binary and continuous outcomes a pooled estimate of treatment effect was calculated for each outcome. This review is based on the findings of eight studies, representing data from a total of 358 participants. Studies fell into four conceptually similar groups: (1) gene pre-test education counselling for relatives of those with CF (one study); (2) biofeedback, massage and music therapy to assist physiotherapy (three studies); (3) behavioural intervention to improve dietary intake in children up to 12 years (three studies); and (4) self-administration of treatments to improve quality of life in adults (one study). Interventions were largely educational or behavioural, targeted at specific treatment concerns

  11. The diagnosis of cystic fibrosis.

    PubMed

    De Boeck, Kris; Vermeulen, Francois; Dupont, Lieven

    2017-06-01

    Establishing the diagnosis of cystic fibrosis (CF) is straight forward in the majority of patients: they present with a clear clinical picture (most frequently chronic respiratory symptoms plus malabsorption), the sweat chloride value is>60mmol/L and two known disease causing CFTR mutations are identified. In less than 5% of subjects, mainly those with a milder or limited phenotype, the diagnostic process is more complex, because initial diagnostic test results are inconclusive: sweat chloride concentration in the intermediate range, less than 2 CF causing mutations identified or both. These patients should be referred to expert centers where bioassays of CFTR function like nasal potential difference measurement or intestinal current measurement can be done. Still, in some patients, despite symptoms compatible with CF and some indication of CFTR dysfunction (e.g. only intermediate sweat chloride value), diagnostic criteria are not met (e.g. only 1 CFTR mutation identified). For these subjects, the term CFTR related disorder (CFTR-RD) is used. Patients with disseminated bronchiectasis, congenital bilateral absence of the vas deferens and acute or recurrent pancreatitis may fall in this category. CF has a very wide disease spectrum and increasingly the diagnosis is being made during adult life, mainly in subjects with milder phenotypes. In many countries, nationwide CF newborn screening (NBS) has been introduced. In screen positive babies, the diagnosis of CF must be confirmed by a sweat test demonstrating a sweat chloride concentration above 60mmol/L. To achieve the benefit of NBS, every baby in whom the diagnosis of CF is confirmed must receive immediate follow-up and treatment in a CF reference center. CF NBS is not full proof: some diagnoses will be missed and in some babies the diagnosis cannot be confirmed nor ruled out with certainty. Screening algorithms that include gene sequencing will detect a high number of such babies that are screen positive with an

  12. Employment and work disability in adults with cystic fibrosis.

    PubMed

    Laborde-Castérot, Hervé; Donnay, Carole; Chapron, Jeanne; Burgel, Pierre-Régis; Kanaan, Reem; Honoré, Isabelle; Dusser, Daniel; Choudat, Dominique; Hubert, Dominique

    2012-03-01

    As a result of prolonged survival, more patients with cystic fibrosis (CF) participate in the labour force. The aim of this study was to evaluate their education, occupation levels and risk factors for work disability. 207 patients answered a self-administered questionnaire about their educational level and work status. Independently, medical records were reviewed for illness severity indicators. 39 patients (19%) were students, 117 (57%) were in the labour force, 13 (6%) were seeking employment and 38 (18%) were inactive. CF patients had a higher educational level and were more likely to hold skilled jobs and to work part time than the general population. FEV1 and educational level were the strongest predictive factors of disability. Many CF patients have access to professional life. Their higher educational levels improve the chances of attaining employment, which highlights the need for career counselling. Working part time helps to maintain employment despite declining health. Copyright © 2011 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.

  13. Achromobacter xylosoxidans respiratory tract infection in cystic fibrosis patients.

    PubMed

    Lambiase, A; Catania, M R; Del Pezzo, M; Rossano, F; Terlizzi, V; Sepe, A; Raia, V

    2011-08-01

    The aims of this study were to evaluate the frequency of Achromobacter xylosoxidans infection in a cohort of cystic fibrosis patients, to investigate antimicrobial sensitivity, to establish possible clonal likeness among strains, and to address the clinical impact of this infection or colonization on the general outcome of these patients. The study was undertaken between January 2004 and December 2008 on 300 patients receiving care at the Regional Cystic Fibrosis Center of the Naples University "Federico II". Sputum samples were checked for bacterial identification. For DNA fingerprinting, pulsed-field gel electrophoresis (PFGE) was carried out. Fifty-three patients (17.6%) had at least one positive culture for A. xylosoxidans; of these, 6/53 (11.3%) patients were defined as chronically infected and all were co-colonized by Pseudomonas aeruginosa. Of the patients, 18.8% persistently carried multidrug-resistant isolates. Macrorestriction analysis showed the presence of seven major clusters. DNA fingerprinting also showed a genetic relationship among strains isolated from the same patients at different times. The results of DNA fingerprinting indicate evidence of bacterial clonal likeness among the enrolled infected patients. We found no significant differences in the forced expiratory volume in 1 s (FEV(1)) and body mass index (BMI) when comparing the case group of A. xylosoxidans chronically infected patients with the control group of P. aeruginosa chronically infected patients.

  14. Cystic echinococcosis in Mundari tribe-members of South Sudan.

    PubMed

    Stewart, Barclay T; Jacob, Joseph; Finn, Timothy; Lado, Mounir; Napoleon, Robert; Brooker, Simon; Sidhu, Paul S; Kolaczinski, Jan

    2013-09-01

    Many neglected tropical diseases, including the zoonotic disease cystic echinococcosis (hydatidosis), are endemic to East Africa. However, their geographical distribution is heterogenous and incompletely characterized. The aim of this study was to determine if Mundari pastoralists harbor endemic human hydatidosis. The survey was conducted in cattle camps randomly selected from accessible sites provided by officials in Terekeka, South Sudan. Following informed consent, a questionnaire collected demographic data and hydatid exposure risk. A systematic sonographic abdominal exam was performed using General Electric's LOGIQ Book XP with a 3C-RS 2-5 MHz curvilinear transducer. Six hundred and ten individuals were screened from 13 camps. Four infections were identified, all in women. The prevalence of abdominal hydatid disease in the Mundari tribe-members in cattle camps was 0·7% and all individuals reporting at least one high-risk exposure to hydatid disease. Cystic echinococcosis is endemic among Mundari pastoralists; however, it would appear to be less endemic than in neighboring tribes.

  15. Cystic echinococcosis in Mundari tribe-members of South Sudan

    PubMed Central

    Stewart, Barclay T; Jacob, Joseph; Finn, Timothy; Lado, Mounir; Napoleon, Robert; Brooker, Simon; Sidhu, Paul S; Kolaczinski, Jan

    2013-01-01

    Many neglected tropical diseases, including the zoonotic disease cystic echinococcosis (hydatidosis), are endemic to East Africa. However, their geographical distribution is heterogenous and incompletely characterized. The aim of this study was to determine if Mundari pastoralists harbor endemic human hydatidosis. The survey was conducted in cattle camps randomly selected from accessible sites provided by officials in Terekeka, South Sudan. Following informed consent, a questionnaire collected demographic data and hydatid exposure risk. A systematic sonographic abdominal exam was performed using General Electric’s LOGIQ Book XP with a 3C-RS 2–5 MHz curvilinear transducer. Six hundred and ten individuals were screened from 13 camps. Four infections were identified, all in women. The prevalence of abdominal hydatid disease in the Mundari tribe-members in cattle camps was 0.7% and all individuals reporting at least one high-risk exposure to hydatid disease. Cystic echinococcosis is endemic among Mundari pastoralists; however, it would appear to be less endemic than in neighboring tribes. PMID:24139620

  16. Successfully treated congenital cystic adenomatoid malformation by open fetal surgery

    PubMed Central

    Fan, Dazhi; Wu, Shuzhen; Wang, Rui; Huang, Yi; Fu, Yao; Ai, Wen; Zeng, Meng; Guo, Xiaoling; Liu, Zhengping

    2017-01-01

    Abstract Background: Congenital cystic adenomatoid malformation (CCAM) is a rare hamartomatous cystic lesion. Open fetal surgery currently provides a potential therapeutic option for management of a fetus with CCAM diagnosis. Case Summary: A 22-year-old G2P0 woman presented at   weeks’ gestation for evaluation of a fetus with a left lung lesion and diagnosed as CCAM at   weeks’ gestation. Open fetal surgery was performed to resection the lesion at   weeks’ gestation under deep maternal general anesthesia. The mother presented at   weeks after open fetal surgery with preterm premature rupture of membranes (PPROM) and underwent cesarean delivery at   weeks’ gestation. A vigorous woman infant of 1955 g, with good Apgar score, was delivered. At 1 month, 4 years, and present, 5 years after birth, she has continued to do well without any obvious deficit and both respiration and circulation were well maintained. Conclusion: We present one case of CCAM which was cured by open fetal surgery and continued to do well at follow-up of 5 years. The success of treatment provided preliminary experience for further carrying out such interventions in China. PMID:28079822

  17. Too much salt, too little soda: cystic fibrosis.

    PubMed

    Quinton, Paul M

    2007-08-25

    Cystic fibrosis (CF) of the pancreas is the most widely accepted name of the most common fatal inherited single gene defect disease among Caucasians. Its incidence among other races is thought to be significantly less, but mutations in the gene have been reported in most, if not all, major populations. This review is intended to give general concepts of the molecular as well as physiological basis of the pathology that develops in the disease. First, an overview of the organ pathology and genetics is presented, followed by the molecular structure of the gene product (cystic fibrosis transmembrane conductance regulator, CFTR), its properties, functions, and controls as currently understood. Second, since mutations appear to be expressed primarily as a defect in electrolyte transport, effects and mechanisms of pathology are presented for two characteristically affected organs where the etiology is best described: the sweat gland, which excretes far too much NaCl ("salt") and the pancreas, which excretes far too little HCO3(- )("soda"). Unfortunately, morbidity and mortality in CF develop principally from refractory airway infections, the basis of which remains controversial. Consequently, we conclude by considering possible mechanisms by which defects in anion transport might predispose the CF lung to chronic infections.

  18. Cancer risk among lung transplant recipients with cystic fibrosis.

    PubMed

    Fink, Aliza K; Yanik, Elizabeth L; Marshall, Bruce C; Wilschanski, Michael; Lynch, Charles F; Austin, April A; Copeland, Glenn; Safaeian, Mahboobeh; Engels, Eric A

    2017-01-01

    Previous studies demonstrated increased digestive tract cancers among individuals with cystic fibrosis (CF), particularly among lung transplant recipients. We describe cancer incidence among CF and non-CF lung recipients. We used data from the US transplant registry and 16 cancer registries. Standardized incidence ratios (SIRs) compared cancer incidence to the general population, and competing risk methods were used for the cumulative incidence of colorectal cancer. We evaluated 10,179 lung recipients (1681 with CF). Risk was more strongly increased in CF recipients than non-CF recipients for overall cancer (SIR 9.9 vs. 2.7) and multiple cancers including colorectal cancer (24.2 vs. 1.7), esophageal cancer (56.3 vs. 1.3), and non-Hodgkin lymphoma (61.8 vs. 9.4). At five years post-transplant, colorectal cancer was diagnosed in 0.3% of CF recipients aged <50 at transplant and 6.4% aged ≥50. CF recipients have increased risk for colorectal cancer, suggesting a need for enhanced screening. Copyright © 2016 European Cystic Fibrosis Society. All rights reserved.

  19. Craniofacial morphology in children with cystic fibrosis.

    PubMed

    Hellsing, E; Brattström, V; Strandvik, B

    1992-04-01

    Cystic fibrosis (CF) is a hereditary metabolic disorder with clinical symptoms of abnormal mucus production. This blocks the airways, gives pancreatic insufficiency, and increases sweat electrolytes. The progressive respiratory disease often leads to respiratory insufficiency and cor pulmonale. The aim of the present investigation was to examine the facial morphology in children with cystic fibrosis. The sample comprised 11 children with cystic fibrosis, who were divided in two groups, one with gastrointestinal disorders and the other with predominantly respiratory insufficiency. Eleven healthy children with normal occlusions were selected as controls. Lateral skull radiographs obtained in natural head posture were digitized, and linear and angular variables for the different groups calculated and compared statistically. The cystic fibrosis group showed open bite, decreased posterior facial height, increased mandibular and craniocervical inclination. Additionally, within the CF-group, the children with respiratory insufficiency differed more from the controls than the children with gastrointestinal disorders. Despite the small number of subjects, the facial morphology of the CF children showed a similar pattern to that of children with nasal respiratory obstruction due to enlarged adenoids or tonsils.

  20. Cystic lesion around the hip joint

    PubMed Central

    Yukata, Kiminori; Nakai, Sho; Goto, Tomohiro; Ikeda, Yuichi; Shimaoka, Yasunori; Yamanaka, Issei; Sairyo, Koichi; Hamawaki, Jun-ichi

    2015-01-01

    This article presents a narrative review of cystic lesions around the hip and primarily consists of 5 sections: Radiological examination, prevalence, pathogenesis, symptoms, and treatment. Cystic lesions around the hip are usually asymptomatic but may be observed incidentally on imaging examinations, such as computed tomography and magnetic resonance imaging. Some cysts may enlarge because of various pathological factors, such as trauma, osteoarthritis, rheumatoid arthritis, or total hip arthroplasty (THA), and may become symptomatic because of compression of surrounding structures, including the femoral, obturator, or sciatic nerves, external iliac or common femoral artery, femoral or external iliac vein, sigmoid colon, cecum, small bowel, ureters, and bladder. Treatment for symptomatic cystic lesions around the hip joint includes rest, nonsteroidal anti-inflammatory drug administration, needle aspiration, and surgical excision. Furthermore, when these cysts are associated with osteoarthritis, rheumatoid arthritis, and THA, primary or revision THA surgery will be necessary concurrent with cyst excision. Knowledge of the characteristic clinical appearance of cystic masses around the hip will be useful for determining specific diagnoses and treatments. PMID:26495246

  1. Ivacaftor for patients with cystic fibrosis.

    PubMed

    Wainwright, Claire E

    2014-10-01

    Ivacaftor is an oral bioavailable potentiator of the cystic fibrosis transmembrane conductance regulator protein. It is the first therapeutic agent that has been registered for clinical use which targets the basic defect in people with cystic fibrosis who carry a G551D mutation or other rarer specific gating mutations. Clinical trials have shown consistent and impressive clinical benefit that appears to be sustained over time in people with cystic fibrosis who carry a G551D mutation and similar benefits have been seen in those who carry rarer gating mutations. Ivacaftor is orally administered twice daily with a dose that does not vary between children aged 6 years through to adult life in patients with G551D. It appears to be well tolerated although there are potential interactions with drugs that are metabolised through CYPP450 CYP3A. Ivacaftor is also currently being trialled in combination with correctors for patients with the most common mutation of cystic fibrosis transmembrane conductance regulator the F508del mutation.

  2. Cystic fibrosis year in review 2016.

    PubMed

    Savant, Adrienne P; McColley, Susanna A

    2017-08-01

    In this article, we highlight cystic fibrosis (CF) research and case reports published in Pediatric Pulmonology during 2016. We also include articles from a variety of journals that are thematically related to these articles, or are of special interest to clinicians. © 2017 Wiley Periodicals, Inc.

  3. Spontaneous hepatocellular adenoma with marked cystic degeneration.

    PubMed

    Kobayashi, M; Enzan, H; Araki, K; Kawai, S; Kitagawa, N

    1999-01-01

    We report a case of spontaneous hepatocellular adenoma with marked cystic degeneration in the non-cirrhotic liver. A 36 year-old Japanese woman with neither history of liver diseases nor use of oral contraceptives and steroids, complained of a 6 kg weight loss over 6 months. Barium meal study revealed an extramural compression along the fornix of the stomach. Abdominal ultrasonography (UC) and computed tomography (CT) demonstrated a mass in the left lateral segment of the liver that measured 11.6x9.5 cm with cystic lesions. Laboratory data on admission showed no significant findings. Celiac angiography revealed a hyper-vascular mass. Surgical exploration revealed a soft mass arising and protruding from the left lateral segment of the liver. Partial resection of the left lateral segment was performed. Histologically, the tumor was surrounded by a thin fibrous pseudocapsule. The neoplastic cells resembling normal hepatocytes around the tumor were large, pale and arranged in thick, irregular cords. Neither mitotic figures nor foci of dysplasia were present. The central portions of the tumor showed marked cystic degeneration. The tumor was histologically diagnosed as hepatocellular adenoma (HCA). HCA with cystic degeneration has been rarely reported.

  4. Exercise is medicine in cystic fibrosis.

    PubMed

    Wheatley, Courtney M; Wilkins, Brad W; Snyder, Eric M

    2011-07-01

    Exercise activates adrenergic and purinergic pathways that regulate activity of ion channels on airway epithelia cells and sweat glands. Therefore, we hypothesize that exercise is not only an important therapy for cystic fibrosis (CF) patients by facilitating systemic improvements but, more importantly, that exercise can improve the pathophysiological ion dysregulation at a cellular level, thereby enhancing quality of life in CF.

  5. Cystic echinococcosis: Future perspectives of molecular epidemiology

    USDA-ARS?s Scientific Manuscript database

    Human cystic echinococcosis (CE) has been conceived to be caused predominantly by Echinococcus granulosus sensu stricto (the dog-sheep strain). Recent molecular approaches on CE, however, have revealed that human cases are also commonly caused by another species, Echinococcus canadensis. All indices...

  6. Nutritional assessment in children with cystic fibrosis

    USDA-ARS?s Scientific Manuscript database

    Optimal nutrition, including consuming 35–40% of calories (kcal) as fat, is a vital part of the management of cystic fibrosis (CF), and involves accurate assessment of dietary intake. We compared 3 methods of nutritional assessment in 8– to 14-year-old children (n=20) with CF: 1) a 24-h Dietary Reca...

  7. Zinc supplementation in children with cystic fibrosis

    USDA-ARS?s Scientific Manuscript database

    Cystic fibrosis (CF) leads to malabsorption of macro- and micronutrients. Symptomatic zinc deficiency has been reported in CF but little is known about zinc homeostasis in children with CF. Zinc supplementation (Zn suppl) is increasingly common in children with CF but it is not without theoretcial r...

  8. Diabetes mellitus in patients with cystic fibrosis.

    PubMed

    Alves, Crésio de Aragão Dantas; Aguiar, Renata Arruti; Alves, Ana Cláudia S; Santana, Maria Angélica

    2007-01-01

    Cystic fibrosis-related diabetes (CFRD) is the principal extra-pulmonary complication of cystic fibrosis, occurring in 15-30% of adult cystic fibrosis patients. The number of cystic fibrosis patients who develop diabetes is increasing in parallel with increases in life expectancy. The aim of this study was to review the physiopathology, clinical presentation, diagnosis and treatment of CFRD. A bibliographic search of the Medline and Latin American and Caribbean Health Sciences Literature databases was made. Articles were selected from among those published in the last twenty years. Insulin deficiency, caused by reduced beta-cell mass, is the main etiologic mechanism, although insulin resistance also plays a role. Presenting features of type 1 and type 2 diabetes, CFRD typically affects individuals of approximately 20 years of age. It can also be accompanied by fasting, non-fasting or intermittent hyperglycemia. Glucose intolerance is associated with worsening of nutritional status, increased morbidity, decreased survival and reduced pulmonary function. Microvascular complications are always present, although macrovascular complications are rarely seen. An oral glucose tolerance test is recommended annually for patients > or = 10 years of age and for any patients presenting unexplained weight loss or symptoms of diabetes. Patients hospitalized with severe diseases should also be screened. If fasting hyperglycemia persists for more than 48 h, insulin therapy is recommended. Insulin administration remains the treatment of choice for diabetes and fasting hyperglycemia. Calories should not be restricted, and patients with CFRD should be managed by a multidisciplinary team.

  9. Dental treatment for people with cystic fibrosis.

    PubMed

    Harrington, N; Barry, P J; Barry, S M

    2016-06-01

    To describe the nature and consequences of the multi-system genetic condition cystic fibrosis with a view to ensuring optimal dental treatment planning for these patients. A literature search was conducted to identify the key medical and dental manifestations of cystic fibrosis. These findings are discussed and utilised to create recommendations for treatment planning in patients with cystic fibrosis for the practising dental practitioner. Cystic fibrosis is a complex, lethal, multisystem autosomal recessive disorder resulting from mutations on chromosome 7 which result in dysfunction of an ion channel that sits on epithelial surfaces. Respiratory disease remains the leading cause of mortality. Survival has greatly increased in recent decades secondary to improved treatment and specialist care. Specific dental manifestations of the disease may result from the condition itself or complications of treatment. Modification of patient management may be necessary to provide optimum patient care. The pathophysiology and clinical manifestations are relevant to practicing dental practitioners and inform recommendations to be utilised to ensure optimal treatment planning for these patients.

  10. [New prospects in cystic fibrosis treatment].

    PubMed

    Prados, C; Serrano, S; Alvarez-Sala, R; Villamor, J

    1997-04-01

    Only a few years ago, cystic fibrosis (CF) was considered the most frequent genetic disease in childhood, although survival has increased considerably in recent years owing to improved treatment. We discuss treatments that are still relevant as well as others that are under investigation now, aiming for better understanding of the disease and the therapies that have improved quality of life for CF patients.

  11. Bilateral Adenoid Cystic Carcinoma of the Orbit.

    PubMed

    Branson, Sara V; McClintic, Elysa; Yeatts, R Patrick

    A 41-year-old woman with adenoid cystic carcinoma of the left lacrimal gland underwent extended left orbital exenteration and postoperative external beam radiation therapy. She presented 1 year postoperatively with contralateral right orbital involvement. The authors report a rare case of adenocystic carcinoma of the lacrimal gland with bilateral orbital involvement.

  12. Kelvin waves and ozone Kelvin waves in the quasi-biennial oscillation and semiannual oscillation: A simulation by a high-resolution chemistry-coupled general circulation model

    NASA Astrophysics Data System (ADS)

    Watanabe, Shingo; Takahashi, Masaaki

    2005-09-01

    Equatorial Kelvin waves and ozone Kelvin waves were simulated by a T63L250 chemistry-coupled general circulation model with a high vertical resolution (300 m). The model produces a realistic quasi-biennial oscillation (QBO) and a semiannual oscillation (SAO) in the equatorial stratosphere. The QBO has a period slightly longer than 2 years, and the SAO shows rapid reversals from westerly to easterly regimes and gradual descents of westerlies. Results for the zonal wave number 1 slow and fast Kelvin waves are discussed. Structure of the waves and phase relationships between temperature and ozone perturbations coincide well with satellite observations made by LIMS, CLAES, and MLS. They are generally in phase (antiphase) in the lower (upper) stratosphere as theoretically expected. The fast Kelvin waves in the temperature and ozone are dominant in the upper stratosphere because the slow Kelvin waves are effectively filtered by the QBO westerly. In this simulation, the fast Kelvin waves encounter their critical levels in the upper stratosphere when zonal asymmetry of the SAO westerly is enhanced by an intrusion of the extratropical planetary waves. In addition to the critical level filtering effect, modulations of wave properties by background winds are evident near easterly and westerly shears associated with the QBO and SAO. Enhancement of wave amplitude in the QBO westerly shear is well coincident with radiosonde observations. Increase/decrease of vertical wavelength in the QBO easterly/westerly is obvious in this simulation, which is consistent with the linear wave theory. Shortening of wave period due to the descending QBO westerly shear zone is demonstrated for the first time. Moreover, dominant periods during the QBO westerly phase are longer than those during the QBO easterly phase for both the slow and fast Kelvin waves.

  13. Adaptive smoothing of high angular resolution diffusion-weighted imaging data by generalized cross-validation improves Q-ball orientation distribution function reconstruction.

    PubMed

    Metwalli, Nader S; Hu, Xiaoping P; Carew, John D

    2010-09-01

    Q-ball imaging (QBI) is a high angular resolution diffusion-weighted imaging (HARDI) technique for reconstructing the orientation distribution function (ODF). Some form of smoothing or regularization is typically required in the ODF reconstruction from low signal-to-noise ratio HARDI data. The amount of smoothing or regularization is usually set a priori at the discretion of the investigator. In this article, we apply an adaptive and objective means of smoothing the raw HARDI data using the smoothing splines on the sphere method with generalized cross-validation (GCV) to estimate the diffusivity profile in each voxel. Subsequently, we reconstruct the ODF, from the smoothed data, based on the Funk-Radon transform (FRT) used in QBI. The spline method was applied to both simulated data and in vivo human brain data. Simulated data show that the smoothing splines on the sphere method with GCV smoothing reduces the mean squared error in estimates of the ODF as compared with the standard analytical QBI approach. The human data demonstrate the utility of the method for estimating smooth ODFs.

  14. Histochemical technique: a general method for quantitative enzyme assays of single cell extracts with a time resolution of seconds and a reading precision of femtomoles

    SciTech Connect

    Outlaw, W.H. Jr.; Springer, S.A.; Tarczynski, M.C.

    1985-03-01

    Biochemists who study single cells have been constrained by the lack of a general methodology of high time resolution and high measurement sensitivity for quantitatively assaying enzyme activities using natural substrates in solution. The methods the authors describe will remove this limitation. In brief, nanogram tissue samples are dissected from frozen-dried tissue. The samples are extracted in microdroplets of assay cocktail. The enzyme activity, indicated fluorometrically by the oxidation/reduction of NAD(P), is followed in real time on a computer display. With these methods, assays of pyruvate orthophosphate dikinase on samples enriched in bundlesheath cells and mesophyll cells of Flaveria brownii yielded the predictable results. Assays of this enzyme in guard cells dissected from Vicia faba leaflets gave results like those recently reported by another laboratory for protoplasts derived from these cells. The results of assays by this method and by enzymic cycling for NAD(P) triose-P dehydrogenase were comparable. Phosphoenolpyruvate carboxylase, the most extensively studied enzyme activity, was present at high levels in guard cells, which has been demonstrated previously in other reports based on diverse assay approaches.

  15. Cystic Renal Disease in the Domestic Ferret

    PubMed Central

    Jackson, Courtnye N; Rogers, Arlin B; Maurer, Kirk J; Lofgren, Jennifer LS; Fox, James G; Marini, Robert P

    2008-01-01

    Cystic renal diseases in domestic ferrets are a common anecdotal finding but have received scant systematic assessment. We performed a 17-y, case-control retrospective analysis of the medical records of 97 ferrets housed at our institution between 1987 and 2004, to determine the prevalence and morphotypes of cystic renal diseases in this species. Histologic sections stained with hematoxylin and eosin, Masson trichrome, or periodic acid–Schiff were evaluated by a comparative pathologist, and statistical analysis of hematologic and serum chemistry values was correlated with morphologic diagnosis. Of the 97 available records, 43 were eliminated due to lack of accompanying tissues. Of the 54 remaining cases, 37 (69% prevalence) had documented renal cysts, and 14 of the 54 ferrets (26%) had primary polycystic disease consisting of either polycystic kidney disease affecting renal tubules or, more commonly, glomerulocystic kidney disease. Secondary polycystic lesions were identified in 11 ferrets (20%), and 12 ferrets (22%) exhibited focal or isolated tubular cysts only as an incidental necropsy finding. Ferrets with secondary renal cysts associated with other developmental anomalies, mesangial glomerulopathy, or end-stage kidney disease had hyperphosphatemia and elevated BUN in comparison with those with primary cystic disease and elevated BUN compared with those without renal lesions. Although reflecting institutional bias, these results implicate primary and secondary cystic renal diseases as highly prevalent and underreported in the domestic ferret. In addition to the clinical implications for ferrets as research subjects and pets, these findings suggest a potential value for ferrets as a model of human cystic renal diseases. PMID:18524174

  16. Endoscopic Ultrasound-Guided Treatment of Pancreatic Cystic and Solid Masses

    PubMed Central

    2015-01-01

    Pancreatic tumor is one of the most difficult diseases to diagnose and treat because of its anatomical location and characteristics. Recently, there have been several innovative trials on the treatment of pancreatic tumors using endoscopic ultrasound (EUS) because it allows selective access to the difficult to reach target organ along the gastrointestinal tract and can differentiate vessels by color Doppler. Among these trials, several have investigated EUS-guided ethanol lavage with or without paclitaxel for pancreatic cystic tumors. These studies show a 33% to 79% complete resolution rate with a favorable safety profile. Compared to EUS-guided ethanol lavage for pancreatic cystic tumors, EUS-guided radiofrequency ablation is considered a less invasive treatment method for pancreatic cancer. Although there are still several difficulties and concerns about complications, one clinical study reported 72.8% feasibility with favorable safety, and therefore, we anticipate the results of ongoing studies with these new less invasive techniques. PMID:26240804

  17. Metastatic pancreatic carcinoma masquerading as cystic lung disease: a rare presentation.

    PubMed

    Stern, Emily; Huseini, Taha; Kuok, YiJin; Lake, Fiona

    2017-09-01

    This 52-year-old male ex-smoker presented with a six-month history of progressive breathlessness and weight loss. He deteriorated acutely, and was admitted with severe type 1 respiratory failure. Apart from diffuse coarse crackles on chest auscultation, physical examination was unremarkable. High-resolution computed tomography (HRCT) showed diffuse cystic changes throughout the lungs. A diagnosis of pulmonary Langerhans cell histiocytosis (PLCH) was considered. Further workup identified a coincidental pancreatic lesion of uncertain significance, which remained indeterminate on magnetic resonance imaging (MRI) and on positron emission tomography (PET). Transbronchial biopsy revealed enteric differentiated adenocarcinoma exhibiting lepidic spread, and autopsy later confirmed primary pancreatic malignancy. This case demonstrates that metastatic pancreatic malignancy can present with severe respiratory failure and masquerade as cystic lung disease.

  18. Physical exercise training for cystic fibrosis.

    PubMed

    Radtke, Thomas; Nolan, Sarah J; Hebestreit, Helge; Kriemler, Susi

    2015-06-28

    Physical exercise training may form an important part of regular care for people with cystic fibrosis. This is an update of previously published reviews. To determine the effects of physical exercise training compared to no training on aerobic exercise capacity, forced expiratory volume in one second, health-related quality of life and other patient-relevant (secondary) outcomes in cystic fibrosis. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register which comprises references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings.Date of the most recent search: 10 March 2015. All randomised and quasi-randomised controlled clinical trials comparing exercise training of any type and duration with conventional care in people with cystic fibrosis. Two authors independently selected studies for inclusion, assessed methodological quality and extracted data. Of the 48 studies identified, 13 studies which included 402 participants, met the inclusion criteria. The numbers in each study ranged from nine up to 72 participants; one study was in adults, six were in children and adolescents and six studies included all age ranges. Four studies of hospitalised participants lasted less than one month and nine studies were outpatient-based, lasting between two months and three years. The studies included participants with a wide range of disease severity and employed differing levels of supervision with a mixture of types of training. There was also wide variation in the quality of the included studies.This systematic review shows limited evidence from both short- and long-term studies that in people with cystic fibrosis aerobic or anaerobic physical exercise training or a combination of both has a positive effect on aerobic exercise capacity, pulmonary function and health-related quality of life. Although improvements are not consistent between studies and ranged

  19. Omega-3 fatty acids for cystic fibrosis.

    PubMed

    Oliver, Colleen; Jahnke, Nikki

    2011-08-10

    Studies suggest that a diet rich in omega-3 essential fatty acids may have beneficial anti-inflammatory effects for chronic conditions such as cystic fibrosis. To determine whether there is evidence that omega-3 polyunsaturated fatty acid supplementation reduces morbidity and mortality and to identify any adverse events associated with supplementation. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group's Trials Register comprising references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings. Authors and persons interested in the subject of the review were contacted.Date of last search: 10 March 2011. Randomised controlled trials in people with cystic fibrosis comparing omega-3 fatty acid supplements with placebo. Two authors independently selected studies for inclusion, extracted data and assessed the risk of bias of the studies. The searches identified 13 studies; four studies with 91 participants were included. Two studies compared omega-3 fatty acids to olive oil for six weeks. One study compared a liquid dietary supplement containing omega-3 fatty acids to one without for six months. One study compared omega-3 fatty acids and omega-6 fatty acids to a control (capsules with customised fatty acid blends) for three months. Only one short-term study (19 participants) comparing omega-3 to placebo reported a significant improvement in lung function and Shwachman score and a reduction in sputum volume in the omega-3 group. Another study (43 participants) demonstrated a significant increase in serum phospholipid essential fatty acid content and a significant drop in the n-6/n-3 fatty acid ratio following omega-3 fatty acid supplementation compared to control. The longer-term study (17 participants) demonstrated a significant increase in essential fatty acid content in neutrophil membranes and a significant decrease in the leukotriene B4 to leukotriene B5 ratio

  20. Quantitative comparison using Generalized Relative Object Detectability (G-ROD) metrics of an amorphous selenium detector with high resolution Microangiographic Fluoroscopes (MAF) and standard flat panel detectors (FPD).

    PubMed

    Russ, M; Shankar, A; Jain, A; Setlur Nagesh, S V; Ionita, C N; Scott, C; Karim, K S; Bednarek, D R; Rudin, S

    2016-02-27

    A novel amorphous selenium (a-Se) direct detector with CMOS readout has been designed, and relative detector performance investigated. The detector features include a 25μm pixel pitch, and 1000μm thick a-Se layer operating at 10V/μm bias field. A simulated detector DQE was determined, and used in comparative calculations of the Relative Object Detectability (ROD) family of prewhitening matched-filter (PWMF) observer and non-prewhitening matched filter (NPWMF) observer model metrics to gauge a-Se detector performance against existing high resolution micro-angiographic fluoroscopic (MAF) detectors and a standard flat panel detector (FPD). The PWMF-ROD or ROD metric compares two x-ray imaging detectors in their relative abilities in imaging a given object by taking the integral over spatial frequencies of the Fourier transform of the detector DQE weighted by an object function, divided by the comparable integral for a different detector. The generalized-ROD (G-ROD) metric incorporates clinically relevant parameters (focal-spot size, magnification, and scatter) to show the degradation in imaging performance for detectors that are part of an imaging chain. Preliminary ROD calculations using simulated spheres as the object predicted superior imaging performance by the a-Se detector as compared to existing detectors. New PWMF-G-ROD and NPWMF-G-ROD results still indicate better performance by the a-Se detector in an imaging chain over all sphere sizes for various focal spot sizes and magnifications, although a-Se performance advantages were degraded by focal spot blurring. Nevertheless, the a-Se technology has great potential to provide breakthrough abilities such as visualization of fine details including of neuro-vascular perforator vessels and of small vascular devices.

  1. Quantitative comparison using generalized relative object detectability (G-ROD) metrics of an amorphous selenium detector with high resolution microangiographic fluoroscopes (MAF) and standard flat panel detectors (FPD)

    NASA Astrophysics Data System (ADS)

    Russ, M.; Shankar, A.; Jain, A.; Setlur Nagesh, S. V.; Ionita, C. N.; Scott, C.; Karim, K. S.; Bednarek, D. R.; Rudin, S.

    2016-03-01

    A novel amorphous selenium (a-Se) direct detector with CMOS readout has been designed, and relative detector performance investigated. The detector features include a 25μm pixel pitch, and 1000μm thick a-Se layer operating at 10V/μm bias field. A simulated detector DQE was determined, and used in comparative calculations of the Relative Object Detectability (ROD) family of prewhitening matched-filter (PWMF) observer and non-pre-whitening matched filter (NPWMF) observer model metrics to gauge a-Se detector performance against existing high resolution micro-angiographic fluoroscopic (MAF) detectors and a standard flat panel detector (FPD). The PWMF-ROD or ROD metric compares two x-ray imaging detectors in their relative abilities in imaging a given object by taking the integral over spatial frequencies of the Fourier transform of the detector DQE weighted by an object function, divided by the comparable integral for a different detector. The generalized-ROD (G-ROD) metric incorporates clinically relevant parameters (focal- spot size, magnification, and scatter) to show the degradation in imaging performance for detectors that are part of an imaging chain. Preliminary ROD calculations using simulated spheres as the object predicted superior imaging performance by the a-Se detector as compared to existing detectors. New PWMF-G-ROD and NPWMF-G-ROD results still indicate better performance by the a-Se detector in an imaging chain over all sphere sizes for various focal spot sizes and magnifications, although a-Se performance advantages were degraded by focal spot blurring. Nevertheless, the a-Se technology has great potential to provide break- through abilities such as visualization of fine details including of neuro-vascular perforator vessels and of small vascular devices.

  2. Implications of carrier identification in newborn screening for cystic fibrosis.

    PubMed

    Parsons, E P; Clarke, A J; Bradley, D M

    2003-11-01

    To investigate the psychosocial implications for families whose infant was identified as a cystic fibrosis carrier by newborn screening. Prospective psychosocial assessment. Primary care. (a) families of an affected infant identified by screening (n = 9); (b) families of a carrier infant identified by screening (n = 10). group of mothers from the general population (n = 82). Questionnaires and semistructured interviews. Attitude to screening, assessments of the mother/baby relationship, anxiety, wellbeing. All families were in favour of screening, with no evidence that the mother/baby relationship, anxiety or wellbeing had been adversely affected. Parents, however, did identify problems in terms of the service delivery protocol and genetic counselling practice. Six months after disclosure, carrier identification was not perceived by parents to be problematic.

  3. Liver transplantation for hepatic cirrhosis in cystic fibrosis.

    PubMed Central

    Noble-Jamieson, G; Valente, J; Barnes, N D; Friend, P J; Jamieson, N V; Rasmussen, A; Calne, R Y

    1994-01-01

    Five children with cystic fibrosis complicated by hepatic cirrhosis received liver grafts. They all had portal hypertension with varices and three had variceal bleeding; respiratory function was only moderately impaired, but four were colonised with pseudomonas and one with aspergillus. Liver transplantation was well tolerated and there was no increase in respiratory or other early postoperative complications. Four of the children were fully well from 14 to 35 months after transplantation; the most recently transplanted had problems from a biliary stricture. In spite of the need for immunosuppression there was no increase in infection and respiratory function improved or remained stable. Once the children were stabilised after transplantation their nutrition and general health were greatly improved. PMID:7979532

  4. Adenoid Cystic Carcinoma Mimicking an Oroantral Fistula: A Case Report

    PubMed Central

    Monteiro, Bárbara Vanessa de Brito; Grempel, Rafael Grotta; Gomes, Daliana Queiroga de Castro; Godoy, Gustavo Pina; Miguel, Márcia Cristina da Costa

    2013-01-01

    Introduction Adenoid cystic carcinoma (ACC) is one of the most frequent malignant salivary gland tumors, which commonly affects the minor salivary glands of the mouth and is rare in the nose and paranasal sinuses. In the maxillary sinus, ACC can mimic inflammatory diseases and has a poor prognosis. Objective To report a case of a 50-year-old man with ACC of the maxillary sinus whose clinical findings in the alveolar ridge mimicked an oroantral fistula. Case Report An excisional biopsy was performed and histopathologic analysis revealed ACC. Lung metastases and residual tumor in the maxillary sinus were detected by imaging methods. In view of the poor general health of the patient, no new surgical intervention was performed and he was only treated by radiotherapy and follow-up. Conclusion Although rare in the maxillary sinus, ACC should be included in the differential diagnosis of lesions affecting this site. PMID:25992095

  5. Asymptomatic carriage of Clostridium difficile in patients with cystic fibrosis.

    PubMed

    Peach, S L; Borriello, S P; Gaya, H; Barclay, F E; Welch, A R

    1986-09-01

    Faecal samples from 37 patients with cystic fibrosis and 40 control patients at the Brompton Hospital and the London Chest Hospital were examined for the presence of Clostridium difficile. The organism was isolated from 2 (17%) of control patients who were receiving antibiotics and from one (3.6%) of control patients who had no antimicrobial treatment. Thirty two per cent of the patients with cystic fibrosis excreted C difficile, though none of them had diarrhoea. Two of the three isolates from control patients and nine of the 12 isolates from patients with cystic fibrosis produced toxin B (cytotoxin) in vitro. Toxin B was present in the stools of one of the control patients and three of the patients with cystic fibrosis; toxin A (enterotoxin) was not detected in the faeces of the patients with cystic fibrosis. Two cytotoxigenic strains of C difficile isolated from patients with cystic fibrosis were examined in hamsters; both were virulent, and the animals died.

  6. Asymptomatic carriage of Clostridium difficile in patients with cystic fibrosis.

    PubMed Central

    Peach, S L; Borriello, S P; Gaya, H; Barclay, F E; Welch, A R

    1986-01-01

    Faecal samples from 37 patients with cystic fibrosis and 40 control patients at the Brompton Hospital and the London Chest Hospital were examined for the presence of Clostridium difficile. The organism was isolated from 2 (17%) of control patients who were receiving antibiotics and from one (3.6%) of control patients who had no antimicrobial treatment. Thirty two per cent of the patients with cystic fibrosis excreted C difficile, though none of them had diarrhoea. Two of the three isolates from control patients and nine of the 12 isolates from patients with cystic fibrosis produced toxin B (cytotoxin) in vitro. Toxin B was present in the stools of one of the control patients and three of the patients with cystic fibrosis; toxin A (enterotoxin) was not detected in the faeces of the patients with cystic fibrosis. Two cytotoxigenic strains of C difficile isolated from patients with cystic fibrosis were examined in hamsters; both were virulent, and the animals died. PMID:3093537

  7. Diabetes mellitus and bone disease in cystic fibrosis.

    PubMed

    Curran, David R; McArdle, John R; Talwalkar, Jaideep S

    2009-10-01

    Patients with cystic fibrosis are frequently affected with pancreatic insufficiency and are predisposed to the development of diabetes mellitus (DM) and bone demineralization. Cystic fibrosis-related diabetes mellitus is a clinical entity distinct from type 1 and type 2 diabetes, with important implications for the nutritional and pulmonary health of cystic fibrosis patients. This form of diabetes owes largely to insulin deficiency, but alterations in insulin sensitivity and hepatic glucose production have also been described. Therapy for cystic fibrosis-related diabetes differs substantially from type 2 DM, with careful attention to prandial glycemic excursions crucial to controlling its metabolic effects. Bone disease, including osteopenia and osteoporosis, also occurs with increased frequency in cystic fibrosis, owing to defects in intestinal absorption, chronic inflammation, lung disease, low body weight, and gonadal dysfunction. The pathogenesis, implications, diagnosis, and therapy of cystic fibrosis-related bone demineralization are discussed, with attention to recommended approaches to prevention of and treatment of established bone disease.

  8. Pregnancy and cystic fibrosis: Approach to contemporary management

    PubMed Central

    Tay, George; Callaway, Leonie; Bell, Scott C

    2014-01-01

    Over the previous 50 years survival of patients with cystic fibrosis has progressively increased. As a result of improvements in health care, increasing numbers of patients with cystic fibrosis are now considering starting families of their own. For the health care professionals who look after these patients, the assessment of the potential risks, and the process of guiding prospective parents through pregnancy and beyond can be both challenging and rewarding. To facilitate appropriate discussions about pregnancy, health care workers must have a detailed understanding of the various important issues that will ultimately need to be considered for any patient with cystic fibrosis considering parenthood. This review will address these issues. In particular, it will outline pregnancy outcomes for mothers with cystic fibrosis, issues that need to be taken into account when planning a pregnancy and the management of pregnancy for mothers with cystic fibrosis or mothers who have undergone organ transplantation as a result of cystic fibrosis. PMID:27512443

  9. Cystic fibrosis lung disease in adult patients.

    PubMed

    Vender, Robert L

    2008-04-01

    As the longevity of all patients with cystic fibrosis (CF) continues to increase (median 2005 survival=36.8 years), more adult patients will be receiving their medical care from nonpediatric adult-care providers. Cystic fibrosis remains a fatal disease, with more than 80% of patients dying after the age of 18 years, and most deaths resulting from pulmonary disease. The changing epidemiology requires adult-care providers to become knowledgeable and competent in the clinical management of adults with CF. Physicians must understand the influence of specific genotype on phenotypic disease presentation and severity, the pathogenic factors determining lung disease onset and progression, the impact of comorbid disease factors such as CF-related diabetes and malnutrition upon lung disease severity, and the currently approved or standard accepted therapies used for chronic management of CF lung disease. This knowledge is critical to help alleviate morbidity and improve mortality for the rapidly expanding population of adults with CF.

  10. Cystic fibrosis and physiological responses to exercise.

    PubMed

    Williams, Craig A; Saynor, Zoe L; Tomlinson, Owen W; Barker, Alan R

    2014-12-01

    Cardiopulmonary exercise testing is underutilized within the clinical management of patients with cystic fibrosis. But within the last 5 years, there has been considerable interest in its implementation, which has included deliberations by the European Cystic Fibrosis Society about incorporating this method within the clinical assessment of patients. This review examines the current use of cardiopulmonary exercise testing in assessing the extent and cause(s) of exercise limitation from a pediatric perspective. Examples of the measured parameters and their interpretation are provided. Critical synthesis of recent work in the oxygen uptake (VO2) kinetics response to and following exercise is also discussed, and although identified more as a research tool, its utilization advances researchers understanding of the cardiovascular, respiratory and muscular limitations to exercise tolerance. Finally, exercise and its application in therapeutic interventions are highlighted and a number of recommendations made about the utility of exercise prescription.

  11. Diffuse Cystic Lung Disease. Part I

    PubMed Central

    Vassallo, Robert; Wikenheiser-Brokamp, Kathryn A.; McCormack, Francis X.

    2015-01-01

    The diffuse cystic lung diseases (DCLDs) are a group of pathophysiologically heterogenous processes that are characterized by the presence of multiple spherical or irregularly shaped, thin-walled, air-filled spaces within the pulmonary parenchyma. Although the mechanisms of cyst formation remain incompletely defined for all DCLDs, in most cases lung remodeling associated with inflammatory or infiltrative processes results in displacement, destruction, or replacement of alveolar septa, distal airways, and small vessels within the secondary lobules of the lung. The DCLDs can be broadly classified according to underlying etiology as those caused by low-grade or high-grade metastasizing neoplasms, polyclonal or monoclonal lymphoproliferative disorders, infections, interstitial lung diseases, smoking, and congenital or developmental defects. In the first of a two-part series, we present an overview of the cystic lung diseases caused by neoplasms, infections, smoking-related diseases, and interstitial lung diseases, with a focus on lymphangioleiomyomatosis and pulmonary Langerhans cell histiocytosis. PMID:25906089

  12. Diffuse Cystic Lung Disease. Part II

    PubMed Central

    Vassallo, Robert; Wikenheiser-Brokamp, Kathryn A.; McCormack, Francis X.

    2015-01-01

    The diffuse cystic lung diseases have a broad differential diagnosis. A wide variety of pathophysiological processes spanning the spectrum from airway obstruction to lung remodeling can lead to multifocal cyst development in the lung. Although lymphangioleiomyomatosis and pulmonary Langerhans cell histiocytosis are perhaps more frequently seen in the clinic, disorders such as Birt-Hogg-Dubé syndrome, lymphocytic interstitial pneumonia, follicular bronchiolitis, and light-chain deposition disease are increasingly being recognized. Obtaining an accurate diagnosis can be challenging, and management approaches are highly disease dependent. Unique imaging features, genetic tests, serum studies, and clinical features provide invaluable clues that help clinicians distinguish among the various etiologies, but biopsy is often required for definitive diagnosis. In part II of this review, we present an overview of the diffuse cystic lung diseases caused by lymphoproliferative disorders, genetic mutations, or aberrant lung development and provide an approach to aid in their diagnosis and management. PMID:25906201

  13. Diffuse Cystic Lung Disease. Part I.

    PubMed

    Gupta, Nishant; Vassallo, Robert; Wikenheiser-Brokamp, Kathryn A; McCormack, Francis X

    2015-06-15

    The diffuse cystic lung diseases (DCLDs) are a group of pathophysiologically heterogenous processes that are characterized by the presence of multiple spherical or irregularly shaped, thin-walled, air-filled spaces within the pulmonary parenchyma. Although the mechanisms of cyst formation remain incompletely defined for all DCLDs, in most cases lung remodeling associated with inflammatory or infiltrative processes results in displacement, destruction, or replacement of alveolar septa, distal airways, and small vessels within the secondary lobules of the lung. The DCLDs can be broadly classified according to underlying etiology as those caused by low-grade or high-grade metastasizing neoplasms, polyclonal or monoclonal lymphoproliferative disorders, infections, interstitial lung diseases, smoking, and congenital or developmental defects. In the first of a two-part series, we present an overview of the cystic lung diseases caused by neoplasms, infections, smoking-related diseases, and interstitial lung diseases, with a focus on lymphangioleiomyomatosis and pulmonary Langerhans cell histiocytosis.

  14. Inflammation and its genesis in cystic fibrosis.

    PubMed

    Nichols, David P; Chmiel, James F

    2015-10-01

    The host inflammatory response in cystic fibrosis (CF) lung disease has long been recognized as a central pathological feature and an important therapeutic target. Indeed, many believe that bronchiectasis results largely from the oxidative and proteolytic damage comprised within an exuberant airway inflammatory response that is dominated by neutrophils. In this review, we address the longstanding argument of whether or not the inflammatory response is directly attributable to impairment of the cystic fibrosis transmembrane conductance regulator or only secondary to airway obstruction and chronic bacterial infection and challenge the importance of this distinction in the context of therapy. We also review the centrality of neutrophils in CF lung pathophysiology and highlight more recent data that suggest the importance of other cell types and signaling beyond NF-κB activation. We discuss how protease and redox imbalance are critical factors in CF airway inflammation and end by reviewing some of the more promising therapeutic approaches now under development.

  15. Ivacaftor: a review of its use in patients with cystic fibrosis.

    PubMed

    Deeks, Emma D

    2013-09-01

    Ivacaftor (Kalydeco™) is a potentiator of the cystic fibrosis transmembrane conductance regulator (CFTR) and is the first drug that treats an underlying cause of cystic fibrosis to be licensed for use. Ivacaftor increases the open probability (i.e. gating) of CFTR channels with the G551D mutation, thus enhancing chloride transport, and is indicated in a number of countries for the treatment of cystic fibrosis in patients aged ≥6 years who carry this mutation. This review focuses on pharmacological, clinical efficacy and tolerability data relevant to the use of ivacaftor in this indication. In two 48-week, double-blind, phase III trials in patients aged ≥12 (STRIVE) or 6-11 (ENVISION) years with cystic fibrosis and the G551D mutation, oral ivacaftor 150 mg every 12 h significantly improved lung function relative to placebo, when used in combination with standard care. Significant improvements in pulmonary exacerbation risk (in STRIVE) as well as bodyweight and some aspects of health-related quality of life (both studies) were also seen with the drug versus placebo. Moreover, the beneficial effects of ivacaftor on parameters such as lung function and bodyweight were maintained over up to 96 weeks of treatment in an ongoing open-label extension of these studies. Ivacaftor was generally well tolerated, with headache, oropharyngeal pain, upper respiratory tract infection and nasal congestion being among the most common adverse events. Thus, ivacaftor expands the current treatment options for patients with cystic fibrosis who have the G551D mutation. Its potential for use in patients with other CFTR mutations is also of interest.

  16. Customer satisfaction survey to improve the European cystic fibrosis external quality assessment scheme.

    PubMed

    Berwouts, Sarah; Dequeker, Elisabeth

    2011-08-01

    The Cystic Fibrosis European Network, coordinated from within the Katholieke Universiteit Leuven, is the provider of the European cystic fibrosis external quality assessment (EQA) scheme. The network aimed to seek feedback from laboratories that participated in the cystic fibrosis scheme in order to improve services offered. In this study we analysed responses to an on-line customer satisfaction survey conducted between September and November 2009. The survey was sent to 213 laboratories that participated in the cystic fibrosis EQA scheme of 2008; 69 laboratories (32%) responded. Scores for importance and satisfaction were obtained from a five-point Likert scale for 24 attributes. A score of one corresponded to very dissatisfied/very unimportant and five corresponded to very satisfied/very important. Means were calculated and placed in a two-dimensional grid (importance-satisfaction analysis). Means were subtracted from each other to obtain gap values (gap-analysis). No attribute had a mean score below 3.63. The overall mean of satisfaction was 4.35. Opportunities for improvement enclosed clarity, usefulness and completeness of the general report and individual comments, and user-friendliness of the electronic datasheet. This type of customer satisfaction survey was a valuable instrument to identify opportunities to improve the cystic fibrosis EQA scheme. It should be conducted on a regular basis to reveal new opportunities in the future and to assess effectiveness of actions taken. Moreover, it could be a model for other EQA providers seeking feedback from participants. Overall, the customer satisfaction survey provided a powerful quality of care improvement tool.

  17. Investigating self-efficacy, disease knowledge and adherence to treatment in adolescents with cystic fibrosis.

    PubMed

    Faint, Nicholas R; Staton, Janelle M; Stick, Stephen M; Foster, Juliet M; Schultz, André

    2017-05-01

    Patient adherence is integral to the effectiveness of prescribed treatment, and is associated with beneficial disease outcomes, yet in adolescents with cystic fibrosis, adherence is often sub-optimal. Multiple factors may contribute to treatment adherence, including disease knowledge and self-efficacy. In adolescents with cystic fibrosis: (i) to compare the disease knowledge of adolescents and their parents before transition to adult care; (ii) to determine the relationship between disease knowledge (adolescent, parent) and adherence; and (iii) to evaluate self-efficacy and its association with disease knowledge and adherence. Adolescents with cystic fibrosis and their parents were recruited from a tertiary children's hospital. Disease knowledge and self-efficacy was assessed using the Knowledge of Disease Management-CF and General Self-Efficacy Scales respectively. Using pharmacy records, medication possession ratio was calculated to measure treatment adherence in the preceding year. Thirty-nine adolescent (aged 12-17 (median 14) years) and parent pairs were recruited. Adherence to hypertonic saline, but not other medications, was significantly associated with disease knowledge in adolescents (r (2)  = 0.40, P = 0.029). Mean (SD) adolescent self-efficacy was 30.8 (4.0), and not associated with disease knowledge or adherence. Mean (SD) disease knowledge was less in adolescents than parents (55 (16)% and 72 (14)% respectively, P < 0.001). Disease knowledge is sub-optimal in adolescents with cystic fibrosis, even in the 2 years immediately before transition to adult care. Given that adherence with some treatments has been associated with disease knowledge our results suggest the need for educational interventions in adolescents with cystic fibrosis to optimise self-management and health outcomes. © 2017 Paediatrics and Child Health Division (The Royal Australasian College of Physicians).

  18. Use of the Cystic Fibrosis Foundation's extensive sputum-culturing protocol for patients without cystic fibrosis: implications for infection control and antimicrobial resistance.

    PubMed

    Brown, Jennifer

    2014-05-01

    The US Cystic Fibrosis Foundation has guidelines for culturing respiratory tract specimens from patients with cystic fibrosis. Pulmonary physicians were surveyed regarding their use of these extensive cystic fibrosis culture protocols for patients without cystic fibrosis. The survey results and a discussion of the implications of these practices are reported.

  19. Nutrition in patients with cystic fibrosis: a European Consensus.

    PubMed

    Sinaasappel, M; Stern, M; Littlewood, J; Wolfe, S; Steinkamp, G; Heijerman, Harry G M; Robberecht, E; Döring, G

    2002-06-01

    This document is the result of an European Consensus conference which took place in Artimino, Tuscany, Italy, in March 2001 involving 33 experts on nutrition in patients with cystic fibrosis, organised by the European Cystic Fibrosis Society, and sponsored by Axcan-Scandipharm, Baxter, Dr Falk Pharma, Fresenius, Nutricia, SHS International, Solvay Pharmaceuticals (major sponsor). The purpose of the conference was to develop a consensus document on nutrition in patients with cystic fibrosis based on current evidence.

  20. Case report: a left upper quadrant complex cystic mass.

    PubMed

    Choi, Lydia; Mirani, Neena; Kim, Steve H

    2012-01-01

    A 61-year-old woman presented with left upper quadrant/flank pain and increasing abdominal girth. CT scan showed a large complex, multi-cystic lesion in the left abdomen. Laparotomy revealed a large retroperitoneal mass attached to the left kidney. Left nephrectomy was performed and pathology demonstrated a benign cystic nephroma. Surgical oncologists should be aware of this rare renal lesion as the clinical and radiographic presentation may mimic other intra-abdominal cystic lesions.

  1. Recurrent cystic adventitial disease of the popliteal artery: successful treatment with percutaneous transluminal angioplasty.

    PubMed

    Maged, Ismaeel M; Kron, Irving L; Hagspiel, Klaus D

    2009-01-01

    Cystic adventitial disease (CAD) is a rare vascular condition that most commonly affects the popliteal artery. Percutaneous transluminal angioplasty (PTA) is generally not considered a valid therapeutic option due to high recurrence rate. We report a case of CAD of the popliteal artery that recurred after surgical cyst enucleation that was successfully treated with PTA. To the best of our knowledge, this is the first case of successful PTA for the treatment of recurrent CAD of the popliteal artery.

  2. Inspiratory muscle training for cystic fibrosis.

    PubMed

    Houston, Brian W; Mills, Nicola; Solis-Moya, Arturo

    2008-10-08

    Cystic fibrosis is the most common life-limiting genetic condition in Caucasians and the life-expectancy of those newly diagnosed is increasing. Inspiratory muscle training may be a way of improving the lung function and quality of life of people with cystic fibrosis. Hence there is a need to establish whether this intervention is beneficial. To determine the effect of inspiratory muscle training on health-related quality of life, pulmonary function and exercise tolerance. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials register comprising of references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings.Date of most recent search: April 2008. Randomised or quasi-randomised clinical controlled trials comparing different inspiratory muscle training regimens with each other or a control in people with cystic fibrosis. Three review authors independently applied the inclusion and exclusion criteria to publications and assessed the quality of the included studies. Seven studies were identified. Of these six studies with 140 participants met the review inclusion criteria. There was wide variation in the quality of the included studies. Data were not published in sufficient detail or with sufficiently similar outcome measures in these studies to perform meta-analyses. We have not found any evidence to suggest that this treatment is either beneficial or not. We would advise that practitioners evaluate on a case-by-case basis whether or not to employ this therapy. We recommend that future studies make more use of health-related quality of life and exercise tolerance measures; and that there is an agreement upon a single standard measure of classifying the clinical status of the participants.

  3. [Multilocular cystic nephroma. Preoperative diagnosis with CAT].

    PubMed

    González Escalante, A; García Riestra, V; Fernández García, M L; Relea Sarabia, A; Beiras Garcia, M; Varela Salgado, M; Sarandeses Portela, A; Alcázar Otero, J J

    1993-01-01

    We report a case of multilocular cystic nephroma in a 45-year-old female patient who had been referred for urological evaluation on detecting a mass in the left lumbar region. Patient work up included CT evaluation, which proved to be the most useful in making the diagnosis. The literature is reviewed with special reference to the nature of this lesion and the existing controversy on whether it is an embryonary tumor or a congenital malformation.

  4. Congenital cystic eye with optic nerve.

    PubMed

    Holland, Lee; Haridas, Anjana; Phillips, Gael; Sullivan, Timothy

    2015-07-15

    Congenital cystic eye (CCE) is a rare condition caused by failure of invagination of the optic vesicle resulting in a persistent cyst replacing the eye. An associated optic nerve attached to the cyst is a rarely reported phenomenon that has been sparsely described histologically, with no immunohistochemistry reported previously. The authors present a case of CCE with optic nerve tissue inserting into the cyst, and present the histological and immunohistochemical findings.

  5. Mature cystic teratoma (dermoid cyst) in the sylvian fissure: a case report and review of the literature.

    PubMed

    Li, Qiang; You, Chao; Zan, Xin; Chen, Ni; Zhou, Liangxue; Xu, Jianguo

    2012-02-01

    Mature cystic teratoma (dermoid cyst) inside the Sylvian fissure is rare. A 14-year-old boy presented with 2 episodes of generalized tonic-clonic seizures. Using a fat-suppressed, T1-weighted sequence, magnetic resonance imaging revealed a hypointense nonenhancing mass in the left Sylvian fissure. He underwent left pterional craniotomy for total tumor resection. The pathological diagnosis was mature cystic teratoma (dermoid cyst). Headache and seizures are the leading symptoms. Cyst rupture causes inflammation of cholesterol crystals, and the cyst contents may cause seizure. Surgical resection is the treatment of choice, but radical resection is not advised if critical neurovascular structure can be injured.

  6. Cystic fibrosis: a mucosal immunodeficiency syndrome

    PubMed Central

    Cohen, Taylor Sitarik; Prince, Alice

    2013-01-01

    Cystic fibrosis transmembrane conductance regulator (CFTR) functions as a channel that regulates the transport of ions and the movement of water across the epithelial barrier. Mutations in CFTR, which form the basis for the clinical manifestations of cystic fibrosis, affect the epithelial innate immune function in the lung, resulting in exaggerated and ineffective airway inflammation that fails to eradicate pulmonary pathogens. Compounding the effects of excessive neutrophil recruitment, the mutant CFTR channel does not transport antioxidants to counteract neutrophil-associated oxidative stress. Whereas mutant CFTR expression in leukocytes outside of the lung does not markedly impair their function, the expected regulation of inflammation in the airways is clearly deficient in cystic fibrosis. The resulting bacterial infections, which are caused by organisms that have substantial genetic and metabolic flexibility, can resist multiple classes of antibiotics and evade phagocytic clearance. The development of animal models that approximate the human pulmonary phenotypes—airway inflammation and spontaneous infection—may provide the much-needed tools to establish how CFTR regulates mucosal immunity and to test directly the effect of pharmacologic potentiation and correction of mutant CFTR function on bacterial clearance. PMID:22481418

  7. Lung disease in mice with cystic fibrosis.

    PubMed Central

    Kent, G; Iles, R; Bear, C E; Huan, L J; Griesenbach, U; McKerlie, C; Frndova, H; Ackerley, C; Gosselin, D; Radzioch, D; O'Brodovich, H; Tsui, L C; Buchwald, M; Tanswell, A K

    1997-01-01

    The leading cause of mortality and morbidity in humans with cystic fibrosis is lung disease. Advances in our understanding of the pathogenesis of the lung disease of cystic fibrosis, as well as development of innovative therapeutic interventions, have been compromised by the lack of a natural animal model. The utility of the CFTR-knockout mouse in studying the pathogenesis of cystic fibrosis has been limited because of their failure, despite the presence of severe intestinal disease, to develop lung disease. Herein, we describe the phenotype of an inbred congenic strain of CFTR-knockout mouse that develops spontaneous and progressive lung disease of early onset. The major features of the lung disease include failure of effective mucociliary transport, postbronchiolar over inflation of alveoli and parenchymal interstitial thickening, with evidence of fibrosis and inflammatory cell recruitment. We speculate that the basis for development of lung disease in the congenic CFTR-knockout mice is their observed lack of a non-CFTR chloride channel normally found in CFTR-knockout mice of mixed genetic background. PMID:9399953

  8. Mycobacterium abscessus and Children with Cystic Fibrosis

    PubMed Central

    Sermet-Gaudelus, Isabelle; Le Bourgeois, Muriel; Pierre-Audigier, Catherine; Offredo, Catherine; Guillemot, Didier; Halley, Sophie; Akoua-Koffi, Chantal; Vincent, Véronique; Sivadon-Tardy, Valérie; Ferroni, Agnès; Berche, Patrick; Scheinmann, Pierre; Lenoir, Gérard

    2003-01-01

    We prospectively studied 298 patients with cystic fibrosis (mean age 11.3 years; range 2 months to 32 years; sex ratio, 0.47) for nontuberculous mycobacteria in respiratory samples from January 1, 1996, to December 31, 1999. Mycobacterium abscessus was by far the most prevalent nontuberculous mycobacterium: 15 patients (6 male, 9 female; mean age 11.9 years; range 2.5–22 years) had at least one positive sample for this microorganism (versus 6 patients positive for M. avium complex), including 10 with >3 positive samples (versus 3 patients for M. avium complex). The M. abscessus isolates from 14 patients were typed by pulsed-field gel electrophoresis: each of the 14 patients harbored a unique strain, ruling out a common environmental reservoir or person-to-person transmission. Water samples collected in the cystic fibrosis center were negative for M. abscessus. This major mycobacterial pathogen in children and teenagers with cystic fibrosis does not appear to be acquired nosocomially. PMID:14720400

  9. [Treatment of Cystic Fibrosis with CFTR Modulators].

    PubMed

    Tümmler, B

    2016-05-01

    Personalized medicine promises that medical decisions, practices and products are tailored to the individual patient. Cystic fibrosis, an inherited disorder of chloride and bicarbonate transport in exocrine glands, is the first successful example of customized drug development for mutation-specific therapy. There are two classes of CFTR modulators: potentiators that increase the activity of CFTR at the cell surface, and correctors that either promote the read-through of nonsense mutations or facilitate the translation, folding, maturation and trafficking of mutant CFTR to the cell surface. The potentiator ivacaftor and the corrector lumacaftor are approved in Germany for the treatment of people with cystic fibrosis who carry a gating mutation such as p.Gly551Asp or who are homozygous for the most common mutation p.Phe508del, respectively. This report provides an overview of the basic defect in cystic fibrosis, the population genetics of CFTR mutations in Germany and the bioassays to assess CFTR function in humans together with the major achievements of preclinical research and clinical trials to bring CFTR modulators to the clinic. Some practical information on the use of ivacaftor and lumacaftor in daily practice and an update on pitfalls, challenges and novel strategies of bench-to-bedside development of CFTR modulators are also provided.

  10. Environmental scan of cystic fibrosis research worldwide.

    PubMed

    Kelly, Janice

    2017-05-01

    Cystic fibrosis (CF) is a rare fatal genetic disease, affecting 70,000 to 100,000 people worldwide [1]. Numerous countries have specific charitable organizations dedicated to CF, with many funding research to find a cure or effective control for the disease. Cystic Fibrosis Canada, the largest charity in Canada dedicated to funding research and care in CF, conducted an environmental scan of these organizations to learn and better understand their research goals and funding process. A set of questions was sent to 25 CF charitable organizations around the world by email. Responses were consolidated to identify common practices and innovative approaches. Among respondents, there were variations in the amount of funds invested in research annually and the number of studies supported. Common themes identified included practicing an open call for research applications, evaluating applications using a peer review process, and placing an increased emphasis on patient engagement. Innovative approaches included funding one larger project; funding a series of sub-projects on a common theme; partially funding a research project; and, indefinitely funding part of a researcher's salary. Among CF charitable organizations, there are numerous approaches to research funding. Both similarities and differences were noted between these organizations, all of which share the common goal of working towards improving quality of life and survival for people with CF. Copyright © 2016 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.

  11. Chloride and potassium channels in cystic fibrosis airway epithelia

    NASA Astrophysics Data System (ADS)

    Welsh, Michael J.; Liedtke, Carole M.

    1986-07-01

    Cystic fibrosis, the most common lethal genetic disease in Caucasians, is characterized by a decreased permeability in sweat gland duct and airway epithelia. In sweat duct epithelium, a decreased Cl- permeability accounts for the abnormally increased salt content of sweat1. In airway epithelia a decreased Cl- permeability, and possibly increased sodium absorption, may account for the abnormal respiratory tract fluid2,3. The Cl- impermeability has been localized to the apical membrane of cystic fibrosis airway epithelial cells4. The finding that hormonally regulated Cl- channels make the apical membrane Cl- permeable in normal airway epithelial cells5 suggested abnormal Cl- channel function in cystic fibrosis. Here we report that excised, cell-free patches of membrane from cystic fibrosis epithelial cells contain Cl- channels that have the same conductive properties as Cl- channels from normal cells. However, Cl- channels from cystic fibrosis cells did not open when they were attached to the cell. These findings suggest defective regulation of Cl- channels in cystic fibrosis epithelia; to begin to address this issue, we performed two studies. First, we found that isoprenaline, which stimulates Cl- secretion, increases cellular levels of cyclic AMP in a similar manner in cystic fibrosis and non-cystic fibrosis epithelial cells. Second, we show that adrenergic agonists open calcium-activated potassium channels, indirectly suggesting that calcium-dependent stimulus-response coupling is intact in cystic fibrosis. These data suggest defective regulation of Cl- channels at a site distal to cAMP accumulation.

  12. Percutaneous Image-Guided Aspiration and Sclerosis of Adventitial Cystic Disease of the Femoral Vein

    SciTech Connect

    Johnson, Jason M.; Kiankhooy, Armin; Bertges, Daniel J.; Morris, Christopher S.

    2009-07-15

    Adventitial cystic disease (ACD), also known as cystic mucoid or myxomatous degeneration, is a rare vascular disease mainly seen in arteries. Seventeen cases have been reported in the world literature. We report the first known case of ACD successfully treated with percutaneous image-guided ethanol sclerosis. Computed tomography showed a cystic mass adherent to the wall of the common femoral vein. An ultrasound examination revealed a deep venous thrombosis of the leg, secondary to extrinsic compression of the common femoral vein. Three years prior to our procedure, the cyst was aspirated, which partially relieved the patient's symptoms. Over the following 3 years the patient's symptoms worsened and a 10-cm discrepancy in thigh size developed, in addition to the deep venous thrombosis associated with lower-extremity edema. Using ultrasound guidance and fluoroscopic control, the cyst was drained and then sclerosed with absolute ethanol. The patient's symptoms and leg swelling resolved completely within several weeks. Follow-up physical examination and duplex ultrasound 6 months following sclerosis demonstrated resolution of the symptoms and elimination of the extrinsic compression effect of the ACD on the common femoral vein.

  13. Pathologic and Radiologic Correlation of Adult Cystic Lung Disease: A Comprehensive Review

    PubMed Central

    Parimi, Vamsi; Taddonio, Michale; Kane, Joshua Robert; Yeldandi, Anjana

    2017-01-01

    The presence of pulmonary parenchymal cysts on computed tomography (CT) imaging presents a significant diagnostic challenge. The diverse range of possible etiologies can usually be differentiated based on the clinical setting and radiologic features. In fact, the advent of high-resolution CT has facilitated making a diagnosis solely on analysis of CT image patterns, thus averting the need for a biopsy. While it is possible to make a fairly specific diagnosis during early stages of disease evolution by its characteristic radiological presentation, distinct features may progress to temporally converge into relatively nonspecific radiologic presentations sometimes necessitating histological examination to make a diagnosis. The aim of this review study is to provide both the pathologist and the radiologist with an overview of the diseases most commonly associated with cystic lung lesions primarily in adults by illustration and description of pathologic and radiologic features of each entity. Brief descriptions and characteristic radiologic features of the various disease entities are included and illustrative examples are provided for the common majority of them. In this article, we also classify pulmonary cystic disease with an emphasis on the pathophysiology behind cyst formation in an attempt to elucidate the characteristics of similar cystic appearances seen in various disease entities. PMID:28270943

  14. Diffusion-weighted MR imaging of solid and cystic lesions of the pancreas.

    PubMed

    Wang, Yi; Miller, Frank H; Chen, Zongming E; Merrick, Laura; Mortele, Koenraad J; Hoff, Frederick L; Hammond, Nancy A; Yaghmai, Vahid; Vahid, Yaghmai; Nikolaidis, Paul

    2011-01-01

    Diffusion-weighted magnetic resonance (MR) imaging is increasingly used in the detection and characterization of pancreatic lesions. Diffusion-weighted imaging may provide additional information to radiologists evaluating patients who have cystic or solid neoplasms of the pancreas. Because of greater freedom of motion of water molecules in fluid-rich environments, simple cysts in the pancreas have higher signal intensity on diffusion-weighted images with a b value of 0 sec/mm2 and lower signal intensity on high-b-value images. High apparent diffusion coefficient (ADC) values can be obtained on ADC maps because of the T2 “shine-through” effect. In contrast, solid neoplasms of the pancreas show increased signal intensity relative to the pancreas on diffusion-weighted images with a b value of 0 sec/mm2 and relatively high signal intensity on high-b-value images. Diffusion-weighted imaging can help detect solid pancreatic neoplasms with extremely dense cellularity or extracellular fibrosis by demonstrating significantly low ADC values, and these neoplasms may be better detected on diffusion-weighted MR images because of better contrast, although the resolution is generally worse. However, diffusion-weighted imaging may not be capable of helping definitively characterize solid lesions as inflammatory or neoplastic because of an overlap in ADC values between the two types. For example, it is difficult to distinguish poorly differentiated pancreatic adenocarcinoma from mass-forming pancreatitis at diffusion-weighted imaging because of similarly low ADC values attributed to dense fibrosis.

  15. Lack of correlation between pulmonary disease and cystic fibrosis transmembrane conductance regulator dysfunction in cystic fibrosis: a case report

    PubMed Central

    2010-01-01

    Introduction Mutations in both alleles of the cystic fibrosis transmembrane conductance regulator gene result in the disease cystic fibrosis, which usually manifests as chronic sinopulmonary disease, pancreatic insufficiency, elevated sodium chloride loss in sweat, infertility among men due to agenesis of the vas deferens and other symptoms including liver disease. Case presentation We describe a pair of African-American brothers, aged 21 and 27, with cystic fibrosis. They were homozygous for a rare frameshift mutation in the cystic fibrosis transmembrane conductance regulator 3791delC, which would be expected to cause significant morbidity. Although 80% of cystic fibrosis patients are colonized with Pseudomonas aeruginosa by eight years of age, the older brother had no serum opsonic antibody titer to P. aeruginosa by age 13 and therefore would have failed to mount an effective antibody response to the alginate (mucoid polysaccharide) capsule of P. aeruginosa. He was not colonized with P. aeruginosa until 24 years of age. Similarly, the younger brother was not colonized with P. aeruginosa until age 20 and had no significant lung disease. Conclusion Despite a prevailing idea in cystic fibrosis research that the amount of functional cystic fibrosis transmembrane conductance regulator predicts clinical status, our results indicated that respiratory disease severity in cystic fibrosis exhibits phenotypic heterogeneity. If this heterogeneity is, in part, genetic, it is most likely derived from genes outside the cystic fibrosis transmembrane conductance regulator locus. PMID:20420703

  16. Lack of correlation between pulmonary disease and cystic fibrosis transmembrane conductance regulator dysfunction in cystic fibrosis: a case report.

    PubMed

    Levy, Hara; Cannon, Carolynn L; Asher, Daniel; García, Christopher; Cleveland, Robert H; Pier, Gerald B; Knowles, Michael R; Colin, Andrew A

    2010-04-26

    Mutations in both alleles of the cystic fibrosis transmembrane conductance regulator gene result in the disease cystic fibrosis, which usually manifests as chronic sinopulmonary disease, pancreatic insufficiency, elevated sodium chloride loss in sweat, infertility among men due to agenesis of the vas deferens and other symptoms including liver disease. We describe a pair of African-American brothers, aged 21 and 27, with cystic fibrosis. They were homozygous for a rare frameshift mutation in the cystic fibrosis transmembrane conductance regulator 3791delC, which would be expected to cause significant morbidity. Although 80% of cystic fibrosis patients are colonized with Pseudomonas aeruginosa by eight years of age, the older brother had no serum opsonic antibody titer to P. aeruginosa by age 13 and therefore would have failed to mount an effective antibody response to the alginate (mucoid polysaccharide) capsule of P. aeruginosa. He was not colonized with P. aeruginosa until 24 years of age. Similarly, the younger brother was not colonized with P. aeruginosa until age 20 and had no significant lung disease. Despite a prevailing idea in cystic fibrosis research that the amount of functional cystic fibrosis transmembrane conductance regulator predicts clinical status, our results indicated that respiratory disease severity in cystic fibrosis exhibits phenotypic heterogeneity. If this heterogeneity is, in part, genetic, it is most likely derived from genes outside the cystic fibrosis transmembrane conductance regulator locus.

  17. Anti-inflammatory drugs and analgesics for managing symptoms in people with cystic fibrosis-related arthritis.

    PubMed

    Thornton, Judith; Rangaraj, Satyapal

    2016-01-21

    Arthritis remains a relatively infrequent complication of cystic fibrosis, but is a cause of significant morbidity when it does occur. Two distinct types of arthritis are described in cystic fibrosis: cystic fibrosis-related arthropathy (CFA) and hypertrophic pulmonary osteoarthropathy (HPO). Management of arthritis in people with cystic fibrosis is uncertain and complex because of the underlying disease and its intense treatment. This is an update of a previously published review. To review the effectiveness and safety of pharmacological agents for the symptomatic management of cystic fibrosis-related arthritis in adults and children with cystic fibrosis. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register which comprises references identified from comprehensive electronic database searches, handsearches of relevant journals and abstract books of conference proceedings.Date of most recent search: 19 January 2016. Randomised controlled studies which compared the efficacy and safety of anti-inflammatory and analgesic agents (e.g. non-steroidal anti-inflammatory agents, systemic corticosteroids, intra-articular corticosteroids) with each other, with no treatment or with placebo for CFA and HPO. No relevant studies were identified. No studies were included in this review. Although it is generally recognised that CFA may be episodic and resolve spontaneously, treatment with analgesics and anti-inflammatory agents may be needed. While this approach may be sufficient to manage symptoms, it is disappointing that no randomised controlled trials to rigorously evaluate these agents were found, nor could the authors identify any quasi-randomised. This systematic review has identified the need for a well-designed adequately-powered randomised controlled trial to assess the efficacy and safety of pharmacological agents for the symptomatic management of cystic fibrosis-related arthritis (CFA and HPO) in adults and children with cystic fibrosis

  18. Oral calorie supplements for cystic fibrosis.

    PubMed

    Smyth, Rosalind L; Rayner, Oli

    2017-05-04

    Poor nutrition occurs frequently in people with cystic fibrosis and is associated with other adverse outcomes. Oral calorie supplements are used to increase total daily calorie intake and improve weight gain. However, they are expensive and there are concerns they may reduce the amount of food eaten and not improve overall energy intake. This is an update of a previously published review. To establish whether in people with cystic fibrosis, oral calorie supplements: increase daily calorie intake; and improve overall nutritional intake, nutritional indices, lung function, survival and quality of life. To assess adverse effects associated with using these supplements. We searched the Cochrane Cystic Fibrosis Trials Register comprising references from comprehensive electronic database searches, handsearches of relevant journals and abstract books of conference proceedings. We contacted companies marketing oral calorie supplements.Last search: 18 October 2016. Randomised or quasi-randomised controlled trials comparing use of oral calorie supplements for at least one month to increase calorie intake with no specific intervention or additional nutritional advice in people with cystic fibrosis. We independently selected the included trials, assessed risk of bias and extracted data. We contacted the authors of included trials and obtained additional information for two trials. We identified 21 trials and included three, reporting results from 131 participants lasting between three months and one year. Two trials compared supplements to additional nutritional advice and one to no intervention. Two of the included trials recruited only children. In one trial the risk of bias was low across all domains, in a second trial the risk of bias was largely unclear and in the third mainly low. Blinding of participants was unclear in two of the trials. Also, in one trial the clinical condition of groups appeared to be unevenly balanced at baseline and in another trial there were

  19. Cystic Fibrosis Associated with Worse Survival After Liver Transplantation.

    PubMed

    Black, Sylvester M; Woodley, Frederick W; Tumin, Dmitry; Mumtaz, Khalid; Whitson, Bryan A; Tobias, Joseph D; Hayes, Don

    2016-04-01

    Survival in cystic fibrosis patients after liver transplantation and liver-lung transplantation is not well studied. To discern survival rates after liver transplantation and liver-lung transplantation in patients with and without cystic fibrosis. The United Network for Organ Sharing database was queried from 1987 to 2013. Univariate Cox proportional hazards, multivariate Cox models, and propensity score matching were performed. Liver transplant and liver-lung transplant were performed in 212 and 53 patients with cystic fibrosis, respectively. Univariate Cox proportional hazards regression identified lower survival in cystic fibrosis after liver transplant compared to a reference non-cystic fibrosis liver transplant cohort (HR 1.248; 95 % CI 1.012, 1.541; p = 0.039). Supplementary analysis found graft survival was similar across the 3 recipient categories (log-rank test: χ(2) 2.68; p = 0.262). Multivariate Cox models identified increased mortality hazard among cystic fibrosis patients undergoing liver transplantation (HR 2.439; 95 % CI 1.709, 3.482; p < 0.001) and liver-lung transplantation (HR 2.753; 95 % CI 1.560, 4.861; p < 0.001). Propensity score matching of cystic fibrosis patients undergoing liver transplantation to non-cystic fibrosis controls identified a greater mortality hazard in the cystic fibrosis cohort using a Cox proportional hazards model stratified on matched pairs (HR 3.167; 95 % CI 1.265, 7.929, p = 0.014). Liver transplantation in cystic fibrosis is associated with poorer long-term patient survival compared to non-cystic fibrosis patients, although the difference is not due to graft survival.

  20. A single-tube multiplex system for the simultaneous detection of 10 common cystic fibrosis mutations.

    PubMed

    Axton, R A; Brock, D J

    1995-01-01

    We describe a nonisotopic single-tube polymerase chain reaction (PCR) multiplex system that detects 10 of the more common cystic fibrosis (CF) mutations (delta F508, delta I507, V520F, G551D, G542X, R553X, R117H, 621 + 1G-->T, N1303K, A455E). The use of this method detects approximately 90% of the CF alleles in the British population. Five exons of the CF gene are amplified simultaneously by PCR, followed by an overnight triple enzyme restriction digest, and then resolved by high-resolution gel acrylamide electrophoresis.

  1. Understanding how cystic fibrosis mutations disrupt CFTR function: from single molecules to animal models.

    PubMed

    Wang, Yiting; Wrennall, Joe A; Cai, Zhiwei; Li, Hongyu; Sheppard, David N

    2014-07-01

    Defective epithelial ion transport is the hallmark of the life-limiting genetic disease cystic fibrosis (CF). This abnormality is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR), the ATP-binding cassette transporter that functions as a ligand-gated anion channel. Since the identification of the CFTR gene, almost 2000 disease-causing mutations associated with a spectrum of clinical phenotypes have been reported, but the majority remain poorly characterised. Studies of a small number of mutations including the most common, F508del-CFTR, have identified six general mechanisms of CFTR dysfunction. Here, we review selectively progress to understand how CF mutations disrupt CFTR processing, stability and function. We explore CFTR structure and function to explain the molecular mechanisms of CFTR dysfunction and highlight new knowledge of disease pathophysiology emerging from large animal models of CF. Understanding CFTR dysfunction is crucial to the development of transformational therapies for CF patients.

  2. Utility of gram stain in evaluation of sputa from patients with cystic fibrosis.

    PubMed Central

    Sadeghi, E; Matlow, A; MacLusky, I; Karmali, M A

    1994-01-01

    The utility of sputum Gram stain in assessing salivary contamination and in predicting the presence of pathogens on the basis of morphology was investigated in 287 respiratory specimens from patients with cystic fibrosis. Where acceptability for culture was defined as a leukocyte/squamous epithelial cell ratio of > 5, 76.6% (220 of 287) of respiratory specimens received in the laboratory were considered acceptable. Unacceptable specimens were more common in younger patients. The positive predictive value of the Gram stain for growth from acceptable sputum samples was 98% for Pseudomonas aeruginosa, 84.4% for Pseudomonas cepacia, 86.3% for Staphylococcus aureus, and 100% for Haemophilus influenzae. In cystic fibrosis patients, as has been reported for respiratory specimens in general, Gram stain of respiratory specimens in helpful for interpreting culture results. PMID:7510312

  3. First Isolations of Segniliparus rugosus from Patients with Cystic Fibrosis▿

    PubMed Central

    Butler, W. Ray; Sheils, Catherine A.; Brown-Elliott, Barbara A.; Charles, Nadege; Colin, Andrew A.; Gant, Mary J.; Goodill, John; Hindman, Diane; Toney, Sean R.; Wallace, Richard J.; Yakrus, Mitchell A.

    2007-01-01

    We report three cases of the new genus Segniliparus isolated from patients with cystic fibrosis. All isolates were unambiguously identified by 16S rRNA gene sequencing as Segniliparus rugosus (GenBank accession no. AY 60892). Drug susceptibility results that may enhance treatment for cystic fibrosis patients with this opportunistic pathogen are presented. PMID:17670929

  4. Magnesium in cystic fibrosis--Systematic review of the literature.

    PubMed

    Santi, Maristella; Milani, Gregorio P; Simonetti, Giacomo D; Fossali, Emilio F; Bianchetti, Mario G; Lava, Sebastiano A G

    2016-02-01

    The metabolism of sodium, potassium, and chloride and the acid-base balance are sometimes altered in cystic fibrosis. Textbooks and reviews only marginally address the homeostasis of magnesium in cystic fibrosis. We performed a search of the Medical Subject Headings terms (cystic fibrosis OR mucoviscidosis) AND (magnesium OR hypomagnes[a]emia) in the US National Library of Medicine and Excerpta Medica databases. We identified 25 reports dealing with magnesium and cystic fibrosis. The results of the review may be summarized as follows. First, hypomagnesemia affects more than half of the cystic fibrosis patients with advanced disease; second, magnesemia, which is normally age-independent, relevantly decreases with age in cystic fibrosis; third, aminoglycoside antimicrobials frequently induce both acute and chronic renal magnesium-wasting; fourth, sweat magnesium concentration was normal in cystic fibrosis patients; fifth, limited data suggest the existence of an impaired intestinal magnesium balance. Finally, stimulating observations suggest that magnesium supplements might achieve an improvement in respiratory muscle strength and mucolytic activity of both recombinant and endogenous deoxyribonuclease. The first comprehensive review of the literature confirms that, despite being one of the most prevalent minerals in the body, the importance of magnesium in cystic fibrosis is largely overlooked. In these patients, hypomagnesemia should be sought once a year. Furthermore, the potential of supplementation with this cation deserves more attention. © 2015 Wiley Periodicals, Inc.

  5. A case of mistaken identity: giant cystic pheochromocytoma.

    PubMed

    Antedomenico, Elena; Wascher, Robert A

    2005-01-01

    A 39-year-old Marshall Islands woman was referred for evaluation of an abdominal mass. Medical history was significant only for pulmonary tuberculosis and scrofula. The patient denied a personal or family history of pancreatic or endocrine disease. Physical examination revealed normal vital signs and a 12-cm mildly tender left upper quadrant mass. A computed tomography scan revealed an 11-cm cystic mass contiguous with the distal pancreas and closely associated with the hilum of the spleen and the left kidney. Based on these findings and the patient's history, a cystic neoplasm of the pancreas was suspected, and she was subsequently taken to the operating room for exploration. Intraoperatively, the patient became markedly hypertensive with manipulation of the mass, requiring intravenous nitroprusside. Histopathological evaluation confirmed the diagnosis of cystic pheochromocytoma (PCC). The patient's postoperative course was uncomplicated. Cystic PCCs may not present with the classic prodromal symptoms associated with solid PCCs. This case represents the complex and unsuspected presentation of an extremely rare functional cystic neoplasm. A high index of suspicion for cystic PCC is necessary when confronted with cystic lesions in the vicinity of the adrenal glands. Failure to recognize cystic PCC before resection may lead to uncontrollable hypertension in the operating room, with potentially serious consequences.

  6. Cystic adventitial disease of popliteal artery with significant stenosis

    PubMed Central

    Gupta, Ranjana; Mittal, Puneet; Gupta, Praveen; Jindal, Nancy

    2013-01-01

    Cystic adventitial disease of popliteal artery is a rare condition of unknown etiology which usually presents in middle-aged men. We present Doppler and computed tomography angiography findings in a case of cystic adventitial disease with significant obstruction of popliteal artery, with secondary narrowing of popliteal vein. PMID:24082480

  7. Giant ranula of the neck: differentiation from cystic hygroma.

    PubMed

    Macdonald, Andre J; Salzman, Karen L; Harnsberger, H Ric

    2003-04-01

    Occasionally, diving ranulas may attain large dimensions (giant ranula); gross involvement of the submandibular and parapharyngeal spaces makes differentiation from other cystic neck masses, particularly cystic hygroma, difficult. As diving ranula and cystic hygroma are managed with different surgical approaches, avoidance of this pitfall is critical. Our purpose was to review the imaging findings of giant ranula and compare them with those of cystic hygroma to define those features that are helpful in differentiating these different disease entities. We conducted a retrospective review of all cases of ranulas that had been surgically treated at our institution in a 15-year period. These were compared with cases of cystic hygroma that involved the same anatomic regions. Images were analyzed for anatomic location and morphology, with specific attention paid to those characteristics that might assist differentiation. Giant ranula was defined as any ranula that significantly involved the paraphyngeal space in addition to the submandibular space. Six patients with giant ranula and fifteen patients with cystic hygroma were reviewed. All giant ranulas retained tapered communication with the sublingual space and were homogeneous, thin-walled, anatomically defined, fluid-containing masses. One infected lesion enhanced, and another previously operated lesion demonstrated mild septation. Cystic hygroma commonly did not communicate with the sublingual space and had features of lobulation, septation and heterogeneity. Additional involvement of spaces not typically involved by ranula further assisted differentiation. Although giant ranulas may superficially resemble cystic hygroma, several imaging features allow confident differentiation of these two entities.

  8. Cystic Hygroma in an Adult; a Case Report

    PubMed Central

    Veeraraghavan, G; Denny, C; Lingappa, A

    2009-01-01

    Lymphangioma is a benign infiltrative malformation of the lymphatic channels. Cystic lymphangioma or cystic hygroma is a subtype of lymphangioma which exhibits large macroscopic cystic space histologically. The cause of cystic hygroma is believed to be developmental defect or primary Multilocular cystic malformation of dilated lymphatic channels. Cystic hygroma is a common and distinct entity that is not manifested in the oral cavity but occurs in the neck as a large, deep diffuse swelling. They are usually found in the posterior triangle of the neck. They often cross the midline, reaching axilla and mediastinum. Such localization verifies the complexity and extent of the lymphatic system in the cervical region when compared to other regions of the body. The five main locations where cystic hygroma can occur are, cervical (75–90%), axillary (20%), inguinal, retroperitoneal and thoracic. They usually appear as solitary lesions. They are usually infiltrative, often separating fascial planes and incorporating nerves, muscles, and blood vessels. They are fluctuant, freely mobile, compressible, painless and transilluminate well. The skin overlying the lesion is normal and usually there is no associated lymphadenopathy. Various treatment modalities have been tried. Surgery has been the main form of treatment, but total removal is not possible in all cases because of the extent of the lesion, which sometimes involves vital structures. We report a case of cystic hygroma in a young male patient. PMID:21483540

  9. Rare cystic liver lesions: A diagnostic and managing challenge

    PubMed Central

    Bakoyiannis, Andreas; Delis, Spiros; Triantopoulou, Charina; Dervenis, Christos

    2013-01-01

    Cystic formations within the liver are a frequent finding among populations. Besides the common cystic lesions, like simple liver cysts, rare cystic liver lesions like cystadenocarcinoma should also be considered in the differential diagnosis. Thorough knowledge of each entity’s nature and course are key elements to successful treatment. Detailed search in PubMed, Cochrane Database, and international published literature regarding rare cystic liver lesions was carried out. In our research are included not only primary rare lesions like cystadenoma, hydatid cyst, and polycystic liver disease, but also secondary ones like metastasis from gastrointestinal stromal tumors lesions. Up-to date knowledge regarding diagnosis and management of rare cystic liver lesions is provided. A diagnostic and therapeutic algorithm is also proposed. The need for a multidisciplinary approach by a team including radiologists and surgeons familiar with liver cystic entities, diagnostic tools, and treatment modalities is stressed. Patients with cystic liver lesions must be carefully evaluated by a multidisciplinary team, in order to receive the most appropriate treatment, since many cystic liver lesions have a malignant potential and evolution. PMID:24282350

  10. Omega-3 fatty acids for cystic fibrosis.

    PubMed

    Oliver, Colleen; Watson, Helen

    2016-01-05

    Studies suggest that a diet rich in omega-3 essential fatty acids may have beneficial anti-inflammatory effects for chronic conditions such as cystic fibrosis. This is an updated version of a previously published review. To determine whether there is evidence that omega-3 polyunsaturated fatty acid supplementation reduces morbidity and mortality and to identify any adverse events associated with supplementation. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group's Trials Register comprising references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings. Authors and persons interested in the subject of the review were contacted.Date of last search: 13 August 2013. Randomised controlled trials in people with cystic fibrosis comparing omega-3 fatty acid supplements with placebo. Two authors independently selected studies for inclusion, extracted data and assessed the risk of bias of the studies. The searches identified 15 studies; four studies with 91 participants (children and adults) were included; duration of studies ranged from six weeks to six months. Two studies were judged to be at low risk of bias based on adequate randomisation but this was unclear in the other two studies. Three of the studies adequately blinded patients, however, the risk of bias was unclear in all studies with regards to allocation concealment and selective reporting.Two studies compared omega-3 fatty acids to olive oil for six weeks. One study compared a liquid dietary supplement containing omega-3 fatty acids to one without for six months. One study compared omega-3 fatty acids and omega-6 fatty acids to a control (capsules with customised fatty acid blends) for three months. Only one short-term study (19 participants) comparing omega-3 to placebo reported a significant improvement in lung function and Shwachman score and a reduction in sputum volume in the omega-3 group. Another

  11. Cystic fibrosis heterozygote screening in 5,161 pregnant women

    SciTech Connect

    Witt, D.R.; Hallam, P.; Blumberg, B.; Fishbach, A.

    1996-04-01

    A screening program for cystic fibrosis (CF) heterozygotes was conducted in a large HMO prenatal population, to evaluate the level of interest among eligible patients, the effectiveness of prescreening education, attitudes toward the screening process, psychological effects, and utilization of prenatal diagnosis and its outcomes. The heterozygote identification rate and frequency of specific CFTR mutations were also assessed. Identified carriers were offered genetic counseling and testing of male partners. Prenatal diagnosis was offered if both partners were identified as carriers. A total of 5,161 women underwent carrier testing; 947 others completed survey instruments only. The acceptance rate of screening was high (78%), and pretest education by videotape was generally effective. Adverse psychological effects were not reported. Participants generally found screening to be desirable and useful. Screening identified 142 female heterozygotes, 109 couples in which the male partner was not a carrier, and 7 high-risk couples. The incidence of R117H mutations was much higher than expected. The number of identified carriers was much lower in Hispanics than in Caucasians. We conclude that large-scale prenatal screening for CF heterozygotes in the absence of a family history of CF is an acceptable method for identifying couples at risk for affected fetuses. Sufficient pretest education can be accomplished efficiently, test insensitivity is well accepted, adverse psychological events are not observed, and general patient satisfaction is high. 66 refs., 1 fig., 8 tabs.

  12. Cystic fibrosis: need for mass deployable screening methods.

    PubMed

    Sengar, Aditya Singh; Agarwal, Anirudh; Singh, Manish K

    2014-10-01

    Cystic fibrosis (CF) is an autosomal recessive disease caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. CFTR is a member of the adenosine triphosphate (ATP)-binding cassette superfamily of proteins and it functions as a chloride channel. CFTR largely controls the working of epithelial cells of the airways, the gastrointestinal tract, exocrine glands, and genitourinary system. Cystic fibrosis is responsible for severe chronic pulmonary disorders in children. Other maladies in the spectrum of this life-limiting disorder include nasal polyposis, pansinusitis, rectal prolapse, pancreatitis, cholelithiasis, insulin-dependent hyperglycemia, and cirrhosis. This review summarizes the recent state of art in the field of cystic fibrosis diagnostic methods with the help of CF literature published so far and proposes new research domains in the field of cystic fibrosis diagnosis.

  13. Pain is an underestimated symptom in cystic fibrosis.

    PubMed

    Masson, Alexandra; Kirszembaum, Maya; Sermet-Gaudelus, Isabelle

    2017-08-12

    Life expectancy is increasing in cystic fibrosis and new aspects of the disease have to be taken into account in cystic fibrosis care. Pain is encountered among 70% of adult and pediatric patients with cystic fibrosis. This symptom is underestimated by the multidisciplinary team. It has been reported as impacting quality of life and adherence to treatments. The location of pain is inconstant among the different studies but the major symptoms are headaches, gastrointestinal, and chest pain. Pain is different for each patient and requires careful evaluation using questionnaires some of which specifically developed for patients with cystic fibrosis. Medical and nonmedical treatment such as ostheopathy or sophrology may relieve pain symptoms but have to be adjusted in the frame of a global personalized care. Pain maybe an underestimated symptom among patients with cystic fibrosis and impacts negatively on quality of life. VIDEO ABSTRACT.

  14. Mesenteric lymphangioma mimicking a cystic ovarian mass on imaging.

    PubMed

    Hitzerd, Emilie; van Hamont, Dennis; Pijnenborg, Johanna M A

    2016-02-01

    Pelvic cystic masses are frequently observed in women. Most lesions are benign and of ovarian origin. However, non-ovarian lesions can be easily confused with cystic ovarian masses on imaging, which hampers diagnostic and therapeutic management. In this report, a rare case of mesenteric lymphangioma mimicking an ovarian cystic mass, discovered as an incidental finding on orthopaedic MRI in an adult female, is presented. The report highlights the sometimes difficult diagnostic process of pelvic cystic masses, due to an extensive differential diagnosis and the fact that imaging is often inconclusive. Even though most cystic masses are of ovarian origin, non-ovarian causes can mimic ovarian masses and should be considered as differential diagnoses. Surgical exploration may be necessary to exclude malignant causes. 2016 BMJ Publishing Group Ltd.

  15. Cystic Fibrosis Revisited - a Review Study.

    PubMed

    Klimova, Blanka; Kuca, Kamil; Novotny, Michal; Maresova, Petra

    2017-01-01

    Cystic fibrosis (CF) is an incurable, chronic disease, which causes severe damages to respiratory and digestive tracts. It is the most common genetically inherited disease among caucasians. This disease is caused by defects in CF genes, the so-called mutations in cystic fibrosis transmembrane conductance regulator (CFTR) gene population. At present over 100,000 people suffer from this disease worldwide. The purpose of this review study is to describe the pathophysiology of CF and provide the latest information on its diagnosis and treatment therapies with respect to the improvement of patient's quality of life and emphasis on targeted specialized care. The methodological approaches include a method of literature review of available sources exploring the issue of cystic fibrosis both from a global and specific perspective point of view. A search was performed in the databases PubMed, MEDLINE, Web of Science, Scopus, Springer and ScienceDirect. Furthermore, other sources cited in the analyzed studies were also examined. On the basis of evaluation of these literature sources, the research issue was explored. The main benefits (e.g., specialized centres for the treatment of CF exist or a new breakthrough in the gene therapy of CF has been made) and limitations (e.g., comorbidity of CF, lifelong and costly treatment, or adverse impact on patient's and caregiver's quality of life) in the treatment of narcolepsy are highlighted. CF requires an integrated treatment approach in specialized CF centers, involving various factors contributing to a better patient's state of health in the form of relevant and well-balanced non-pharmacological and pharmacological therapies. In addition, further large scale clinical trials are needed in order to develop compounds that are aimed at the most common classes of CFTR. Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.org.

  16. Cystic fibrosis therapeutics: the road ahead.

    PubMed

    Hoffman, Lucas R; Ramsey, Bonnie W

    2013-01-01

    A great deal of excitement and hope has followed the successful trials and US Food and Drug Administration approval of the drug ivacaftor (Kalydeco), the first therapy available that targets the underlying defect that causes cystic fibrosis (CF). Although this drug has currently demonstrated a clinical benefit for a small minority of the CF population, the developmental pathway established by ivacaftor paves the way for other CF transmembrane conductance regulator (CFTR) modulators that may benefit many more patients. In addition to investigating CFTR modulators, researchers are actively developing numerous other innovative CF therapies. In this review, we use the catalog of treatments currently under evaluation with the support of the Cystic Fibrosis Foundation, known as the Cystic Fibrosis Foundation Therapeutics Pipeline, as a platform to discuss the variety of candidate treatments for CF lung disease that promise to improve CF care. Many of these approaches target the individual components of the relentless cycle of airway obstruction, inflammation, and infection characteristic of lung disease in CF, whereas others are aimed directly at the gene defect, or the resulting dysfunctional protein, that instigates this cycle. We discuss how new findings from the laboratory have informed not only the development of novel therapeutics, but also the rationales for their use and the outcomes used to measure their effects. By reviewing the breadth of candidate treatments currently in development, as well as the recent progress in CF therapies reflected by the evolution of the therapeutics pipeline over the past few years, we hope to build upon the optimism and anticipation generated by the recent success of Kalydeco.

  17. Analyzing the Effect of Tropical Cyclones on the Upper Ocean Using an Ocean General Circulation Model with Varying Horizontal Grid Resolution

    NASA Astrophysics Data System (ADS)

    Li, H.; Sriver, R. L.

    2015-12-01

    Tropical cyclones (TCs) have the potential to influence regional and global climate through their interactions with the upper ocean. Here we present results from a suite of ocean-only model experiments featuring the Community Earth System Model (CESM), in which we analyze the effect of tropical cyclone wind forcing on the global ocean using three different horizontal ocean grid resolutions (3˚, 1˚, and 0.1˚). The ocean simulations are forced with identical atmospheric inputs from the Coordinated Ocean-Ice Reference Experiments version 2 (COREv2) normal year forcing conditions, featuring global blended TC winds from a fully-coupled CESM simulation with a 25 km atmosphere [Small et al., 2014]. The simulated TC climatology shows good agreement with observational estimates of annual TC statistics, including annual frequency, intensity distributions, and geographic distributions. Each simulation is 10 years, which includes a 5-year spin up and 5 years of TC-wind forcing. In addition, we conduct corresponding control simulations for each grid resolution configuration without applied TC forcing. We will discuss the TC-induced ocean responses across a variety of spatial and temporal scales. A key highlight of this work is analyzing the effect of ocean horizontal grid resolution on TC-induced ocean responses, particularly at resolutions capable of simulating mesoscale ocean eddies.

  18. The Dynamic General Vegetation Model MC1 over the United States and Canada at a 5-arcminute resolution: model inputs and outputs

    Treesearch

    Ray Drapek; John B. Kim; Ronald P. Neilson

    2015-01-01

    Land managers need to include climate change in their decisionmaking, but the climate models that project future climates operate at spatial scales that are too coarse to be of direct use. To create a dataset more useful to managers, soil and historical climate were assembled for the United States and Canada at a 5-arcminute grid resolution. Nine CMIP3 future climate...

  19. Benign Cystic Mesothelioma Misdiagnosed as Peritoneal Carcinomatosis

    PubMed Central

    Shin, Hyun Deok; Kim, Suk Bae

    2016-01-01

    Benign cystic mesothelioma (BCM) is a rare benign disease that forms multicystic masses in the abdomen, pelvis, and retroperitoneum. It occurs predominantly in young to middle-aged women. The majority of cases were associated with a history of abdominal or pelvic operation, a history of endometriosis, and pelvic inflammatory disease. We present a unique case of BCM which is different to the previous cases. The patient was a 52-year-old man showing features of peritoneal carcinomatosis accompanied by ascites on abdominal computed tomography scans. We herein report a case of BCM misdiagnosed with peritoneal carcinomatosis. PMID:27403112

  20. CYSTIC FIBROSIS: MICROBIOLOGY AND HOST RESPONSE

    PubMed Central

    Zemanick, Edith T.

    2016-01-01

    THE EARLIEST DESCRIPTIONS OF LUNG DISEASE IN PEOPLE WITH CYSTIC FIBROSIS (CF) DEMONSTRATED THE INVOLVEMENT OF THREE INTERACTING PATHOPHYSIOLOGICAL ELEMENTS IN CF AIRWAYS: MUCUS OBSTRUCTION, INFLAMMATION, AND INFECTION. OVER THE PAST 7 DECADES, OUR UNDERSTANDING OF CF RESPIRATORY MICROBIOLOGY AND INFLAMMATION HAS EVOLVED WITH THE INTRODUCTION OF NEW TREATMENTS, WITH INCREASED LONGEVITY, AND WITH INCREASINGLY SOPHISTICATED LABORATORY TECHNIQUES. IN THIS CHAPTER, WE WILL REVIEW THE CURRENT STATE OF UNDERSTANDING OF THE ROLES OF INFECTION AND INFLAMMATION AND THEIR ROLES IN DRIVING LUNG DISEASE. WE WILL ALSO DISCUSS HOW THIS CONSTANTLY EVOLVING INFORMATION IS USED TO INFORM CURRENT THERAPEUTIC STRATEGIES, MEASURES AND PREDICTORS OF DISEASE SEVERITY, AND RESEARCH PRIORITIES. PMID:27469179

  1. Vitamin K supplementation for cystic fibrosis.

    PubMed

    Jagannath, Vanitha A; Fedorowicz, Zbys; Thaker, Vidhu; Chang, Anne B

    2015-01-18

    Cystic fibrosis is a genetic disorder which can lead to multiorgan dysfunction. Malabsorption of fat and fat-soluble vitamins (A, D, E, K) may occur and can cause subclinical deficiencies of some of these vitamins. Vitamin K is known to play an important role in both blood coagulation and bone formation. Supplementation with vitamin K appears to be one way of addressing the deficiency, but there is very limited agreement on the appropriate dose and frequency of use of these supplements. To assess the effects of vitamin K supplementation in people with cystic fibrosis and to determine the optimal dose and route of administration of vitamin K for both routine and therapeutic use. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group's Trials Register comprising references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings.Most recent search: 08 October 2014. Randomised and quasi-randomised controlled trials of all preparations of vitamin K used as a supplement compared to either no supplementation (or placebo) at any dose or route and for any duration, in children or adults diagnosed with cystic fibrosis (by sweat test or genetic testing). Two authors independently screened papers, extracted trial details and assessed their risk of bias. Two trials (total of 32 participants) each lasting one month were included in the review and were assessed as having a moderate risk of bias. One was a dose-ranging parallel group trial in children (aged 8 to 18 years); and the other (with an older cohort) had a cross-over design comparing supplements to no treatment, but no separate data were reported for the first intervention period. Neither of the trials addressed any of the primary outcomes (coagulation, bone formation and quality of life). Both trials reported the restoration of serum vitamin K and undercarboxylated osteocalcin levels to the normal range after one month of

  2. [Measurement of pulmonary inflammation in cystic fibrosis].

    PubMed

    Fayon, M; Chiron, R; Abely, M

    2008-06-01

    Lung inflammation is a pivotal phenomenon in the pathogenesis of cystic fibrosis. Inflammation can be measured and quantified within a research perspective, as well as in daily clinical practice. In this review paper, the "Inflammation Task Force" of the "Société Française de Mucoviscidose" has reviewed the literature regarding the various techniques currently available (bronchoalveolar lavage, sputum analysis, nasal wash and brushing, exhaled breath condensates, carbon monoxide and nitric oxide, and systemic measurements (plasma and urine)). The interpretation of all these determinations in children and adults is also discussed.

  3. [New therapeutic developments in cystic fibrosis].

    PubMed

    Bui, S; Macey, J; Fayon, M; Bihouée, T; Burgel, P-R; Colomb, V; Corvol, H; Durieu, I; Hubert, D; Marguet, C; Mas, E; Munck, A; Murris-Espin, M; Reix, P; Sermet-Gaudelus, I

    2016-12-01

    Since the discovery of chloride secretion by the Cystic Fibrosis Transport regulator CFTR in 1983, and CFTR gene in 1989, knowledge about CFTR synthesis, maturation, intracellular transfer and function has dramatically expanded. These discoveries have led to the distribution of CF mutations into 6 classes with different pathophysiological mechanisms. In this article we will explore the state of art on CFTR synthesis and its chloride secretion function. We will then explore the consequences of the 6 classes of mutations on CFTR protein function and we will describe the new therapeutic developments aiming at correcting these defects. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

  4. Airway clearance therapy in cystic fibrosis patients.

    PubMed

    Pisi, Giovanna; Chetta, Alfredo

    2009-08-01

    Cystic fibrosis (CF) is the most common life-shortening inherited disease affecting Caucasian people. In CF, the major feature of lung disease is the retention of mucus due to impaired clearance of abnormally viscous airway secretions. Airway clearance techniques (ACTs) may significantly improve mucociliary clearance and gas exchange, thereby being of clinical benefit in reducing pulmonary complications in CF patients. ACTs include conventional chest physiotherapy, active cycle of breathing techniques, autogenic drainage, positive expiratory pressure and high-frequency chest compression. In order to suit the needs of patients, families and care-givers, ACTs need to be individually and continuously adapted.

  5. Cystic fibrosis-associated liver disease.

    PubMed

    Herrmann, Ulrike; Dockter, Gerd; Lammert, Frank

    2010-10-01

    Liver disease is increasingly common in cystic fibrosis (CF). As new therapeutic options emerge, life expectancy increases and common hepatobiliary manifestations impact on quality of life and survival of CF patients. Hepatobiliary abnormalities in CF vary in nature and range from defects attributable to the underlying CFTR gene defect to those related to systemic disease and malnutrition. Today complications of liver disease represent the third most frequent cause of disease-related death in patients with CF. Here we review molecular and clinical genetics of CF, including genetic modifiers of CF-associated liver disease, and provide practical recommendations for genetic testing, diagnosis and treatment of hepatobiliary manifestations in CF.

  6. Genetic therapies for cystic fibrosis lung disease.

    PubMed

    Sinn, Patrick L; Anthony, Reshma M; McCray, Paul B

    2011-04-15

    The aim of gene therapy for cystic fibrosis (CF) lung disease is to efficiently and safely express the CF transmembrane conductance regulator (CFTR) in the appropriate pulmonary cell types. Although CF patients experience multi-organ disease, the chronic bacterial lung infections and associated inflammation are the primary cause of shortened life expectancy. Gene transfer-based therapeutic approaches are feasible, in part, because the airway epithelium is directly accessible by aerosol delivery or instillation. Improvements in standard delivery vectors and the development of novel vectors, as well as emerging technologies and new animal models, are propelling exciting new research forward. Here, we review recent developments that are advancing this field of investigation.

  7. [Nutrition, cystic fibrosis and the digestive tract].

    PubMed

    Olveira, Gabriel; Olveira, Casilda

    2008-05-01

    The prevalence of hyponutrition in cystic fibrosis is high although it may vary according to the different studies. Detection of hyponutrition should be done by combining different methods, depending on their availability. However, the simplest and most validated criterion is to measure at each visit the weight (and height in children) in order to calculate the body mass index and categorizing hyponutrition according to absolute criteria: in adults < 18.5 kg/m(2), and in children as percentiles of the body mass index. Worsening of the nutritional status is directly related with the decrease in lung function parameters and it has been proposed as a morbidity (and even mortality) predictive factor in people with cystic fibrosis, independently of the level of pulmonary dysfunction. Exocrine pancreatic insufficiency is present is approximately 70-90% of the patients with cystic fibrosis and the genotype-phenotype correlation is high. Most of the patients with exocrine pancreatic insufficiency tolerate a high-fat diet provided that they are treated with pancreatic enzymes at appropriate doses. The prevalence of diabetes increases with age, reaching up 40% of the cases in patients older than 30 years. Clinical liver involvement is less prevalent (it approximately affects 1/3 of the patients). Other intestinal complications such as meconial ileus, gastroesophageal reflux, obstruction of the distal intestine, or fibrosing colon disease may also condition malnourishment. In patients with cystic fibrosis, a usual high-fat diet providing 120%-150% of the recommended calories is advised. If the nutritional goals are not achieved or maintained with diet modifications, artificial supplements may be added, although the recommendation for their use has not been endorsed by solid scientific evidences. The most frequently used preparations usually are polymeric or hypercaloric. The indications for enteral (through a tube, especially gastrostomy) or parenteral nutritional support are

  8. Vitamin D Deficiency in Cystic Fibrosis

    PubMed Central

    Hall, William B.; Sparks, Amy A.; Aris, Robert M.

    2010-01-01

    Cystic Fibrosis is the most common inherited genetic respiratory disorder in the Western World. Hypovitaminosis D is almost universal in CF patients, likely due to a combination of inadequate absorption, impaired metabolism, and lack of sun exposure. Inadequate levels are associated with the high prevalence of bone disease or osteoporosis in CF patients, which is associated with increased morbidity including fractures, kyphosis, and worsening pulmonary status. Treatment goals include regular monitoring 25 hydroxyvitamin D (25OHD) levels with aggressive treatment for those with levels <75 nmol/L (<30 ng/mL). More research is needed to determine optimal supplementation goals and strategies. PMID:20148079

  9. Influenza immunization in children with cystic fibrosis.

    PubMed

    Adlard, P; Bryett, K

    1987-01-01

    Nineteen children with cystic fibrosis and aged between 5 and 13 years were randomized to receive two doses at monthly intervals of either a split-virion influenza vaccine (MFV-Ject, Institut Merieux) or a sub-unit vaccine (Fluvirin, Evans). In those completing the study, there was a satisfactory serological response. There was no statistically significant difference between the immunogenicity of the two vaccines as evaluated by haemagglutination inhibition or single radial haemolysis tests. The incidence of local side-effects was similar in the two groups.

  10. Vitamin K supplementation for cystic fibrosis.

    PubMed

    Jagannath, Vanitha A; Thaker, Vidhu; Chang, Anne B; Price, Amy I

    2017-08-22

    Cystic fibrosis is a genetic disorder which can lead to multiorgan dysfunction. Malabsorption of fat and fat-soluble vitamins (A, D, E, K) may occur and can cause subclinical deficiencies of some of these vitamins. Vitamin K is known to play an important role in both blood coagulation and bone formation. Supplementation with vitamin K appears to be one way of addressing the deficiency, but there is very limited agreement on the appropriate dose and frequency of use of these supplements. This is an updated version of the review. To assess the effects of vitamin K supplementation in people with cystic fibrosis and to determine the optimal dose and route of administration of vitamin K for both routine and therapeutic use. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group's Trials Register comprising references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings.Most recent search: 30 January 2017. Randomised and quasi-randomised controlled trials of all preparations of vitamin K used as a supplement compared to either no supplementation (or placebo) at any dose or route and for any duration, in children or adults diagnosed with cystic fibrosis (by sweat test or genetic testing). Two authors independently screened papers, extracted trial details and assessed their risk of bias. Two trials (total of 32 participants) each lasting one month were included in the review and were assessed as having a moderate risk of bias. One was a dose-ranging parallel group trial in children (aged 8 to 18 years); and the other (with an older cohort) had a cross-over design comparing supplements to no treatment, but no separate data were reported for the first intervention period. Neither of the trials addressed any of the primary outcomes (coagulation, bone formation and quality of life). Both trials reported the restoration of serum vitamin K and undercarboxylated osteocalcin

  11. Vaccine strategies against cystic fibrosis pathogens

    PubMed Central

    Le Moigne, Vincent; Gaillard, Jean-Louis; Herrmann, Jean-Louis

    2016-01-01

    ABSTRACT A great number of cystic fibrosis (CF) pathogens such as Pseudomonas aeruginosa, the Burkholderia cepacia and the Mycobacterium abscessus complex raised difficult therapeutic problems due to their intrinsic multi-resistance to numerous antibiotics. Vaccine strategies represent one of the key weapons against these multi-resistant bacteria in a number of clinical settings like CF. Different strategies are considered in order to develop such vaccines, linked either to priming the host response, or by exploiting genomic data derived from the bacterium. Interestingly, virulence factors synthesized by various pathogens might serve as targets for vaccine development and have been, for example, evaluated in the context of CF. PMID:26618824

  12. The Changing Microbial Epidemiology in Cystic Fibrosis

    PubMed Central

    LiPuma, John J.

    2010-01-01

    Summary: Infection of the airways remains the primary cause of morbidity and mortality in persons with cystic fibrosis (CF). This review describes salient features of the epidemiologies of microbial species that are involved in respiratory tract infection in CF. The apparently expanding spectrum of species causing infection in CF and recent changes in the incidences and prevalences of infection due to specific bacterial, fungal, and viral species are described. The challenges inherent in tracking and interpreting rates of infection in this patient population are discussed. PMID:20375354

  13. "Cystic fibrotics could survive cholera, choleraics could survive cystic fibrosis"; hypothesis that explores new horizons in treatment of cystic fibrosis.

    PubMed

    Azimi, Arsalan

    2015-12-01

    Cystic fibrosis, the most common inherited disease of white population, is a disease of CFTR channels, in which mucosal function of many organs especially respiratory tract is impaired. Decreased mucociliary clearance and accumulation of mucus in airways facilitates colonization of infectious microorganisms, followed by infection. Following chronic infection, persistent inflammation ensues, which results in airway remodeling and deterioration of mucociliary clearance and result in a vicious cycle. Here, it is hypothesized that cholera toxin (CT) could ameliorate symptoms of cystic fibrosis as CT could dilute the thickened mucus, improve mucociliary clearance and alleviate airway obstruction. CT strengthens immunity of airway mucosa and it could attenuates bacterial growth and reduce persistency of infection. CT also modulates cellular immune response and it could decrease airway inflammation, hinder airway remodeling and prevent respiratory deterioration. Thereby it is hypothesized that CT could target and ameliorate many of pathophysiologic steps of the disease and it explores new horizons in treatment of CF. Copyright © 2015 Elsevier Ltd. All rights reserved.

  14. Purely cystic adrenal lesion in a newborn evolving into a solid neuroblastoma.

    PubMed

    Gali, Shapira; Anat, Ilivitzki

    2015-02-01

    Purely cystic neuroblatomas are often discovered prenatally. As the main differential diagnosis is adrenal hemorrhage, follow-up sonography is warranted after birth. Cystic neuroblastomas are expected to evolve into lesions of mixed echogenicity with cystic and solid components. We present a rare case of a purely cystic left-sided adrenal lesion in a newborn, suggesting an adrenal hemorrhage, which on follow-up sonography evolved into a purely solid mass with poor vascularization, diagnosed as a cystic neuroblastoma. We suggest that even purely cystic adrenal masses in the newborn should be closely followed up with sonography, as they may represent purely cystic neuroblastomas. © 2015 Wiley Periodicals, Inc.

  15. Cystic fibrosis pulmonary guidelines: airway clearance therapies.

    PubMed

    Flume, Patrick A; Robinson, Karen A; O'Sullivan, Brian P; Finder, Jonathan D; Vender, Robert L; Willey-Courand, Donna-Beth; White, Terry B; Marshall, Bruce C

    2009-04-01

    Cystic fibrosis (CF) is a genetic disease characterized by dehydration of airway surface liquid and impaired mucociliary clearance. As a result, there is difficulty clearing pathogens from the lung, and patients experience chronic pulmonary infections and inflammation. Clearance of airway secretions has been a primary therapy for those with CF, and a variety of airway clearance therapies (ACTs) have been developed. Because ACTs are intrusive and require considerable time and effort, it is important that appropriate techniques are recommended on the basis of available evidence of efficacy and safety. Therefore, the Cystic Fibrosis Foundation established a committee to examine the clinical evidence for each therapy and provide guidance for their use. A systematic review was commissioned, which identified 7 unique reviews and 13 additional controlled trials that addressed one or more of the comparisons of interest and were deemed eligible for inclusion. Recommendations for use of the ACTs were made, balancing the quality of evidence and the potential harms and benefits. The committee determined that, although there is a paucity of controlled trials that assess the long-term effects of ACTs, the evidence quality overall for their use in CF is fair and the benefit is moderate. The committee recommends airway clearance be performed on a regular basis in all patients. There are no ACTs demonstrated to be superior to others, so the prescription of ACTs should be individualized. Aerobic exercise is recommended as an adjunctive therapy for airway clearance and for its additional benefits to overall health.

  16. Variable weight training in cystic fibrosis.

    PubMed

    Strauss, G D; Osher, A; Wang, C I; Goodrich, E; Gold, F; Colman, W; Stabile, M; Dobrenchuk, A; Keens, T G

    1987-08-01

    A six-month pilot study of variable weight training (VWT) was undertaken to assess its impact on body weight, pulmonary function, muscle size and strength, and social function in 12 adolescent and adult patients with moderately severe cystic fibrosis. Exercise for patients with cystic fibrosis (CF) has often been recommended as an adjunct to physical therapy, although aerobic exercise has not resulted in weight gain in CF. Compared to a three-month control period, six months of VWT resulted in significant increase in weight (2.88 kg, p less than .02), muscle size (1.6 to 1.8 cm upper arm, p less than .01), strength (increase from 16 to 32 muscle groups at normal strength, p less than .005), and decrease in residual volume (1.77 L, p less than .03) and RV/TLC (12.4 percent, p less than .02). There was no significant improvement in other measures of pulmonary or social function. VWT appears to be a form of exercise in which even moderately ill CF patients can engage safely, leading to desired weight gain and increased strength. These results warrant further study of the effects of VWT on pulmonary function and CF morbidity.

  17. Cystic peritoneal mesothelioma: report of a case.

    PubMed

    Cavallaro, Andrea; Berretta, Massimiliano; Lo Menzo, Emanuele; Cavallaro, Vincenzo; Zanghì, Antonio; Di Vita, Maria; Cappellani, Alessandro

    2011-01-01

    Benign multicystic peritoneal mesothelioma (BMPM) is a rare disease with good short-term prognosis and rare malignant transformation. However, its biological significance remains unexplained. A neoplastic origin is considered by many authors to require a surgical excision, based on the high recurrence and progressive growth rate of the tumors. However, alternative or integrative treatment options have also been proposed. A 45-year-old woman presented to our unit with a history of occasional discomfort and pain in the left hip. On physical examination, we noticed a tough-elastic, fixed mass located in the iliac fossa. Computed tomography scan detected a mass with multiseptated cystic-like areas. Due to the similarity of these findings to a primitive sarcomatous tumor of the retroperitoneum, an arteriographic study was also performed. The patient underwent en bloc resection of the mass, including a segment of the sigmoid colon. The final pathologic diagnosis was cystic mesothelioma. Further studies are needed to better understand the etiology and pathogenesis of this rare disease, and to define a more tailored treatment plan.

  18. Lessons learned from metabolomics in cystic fibrosis.

    PubMed

    Muhlebach, Marianne S; Sha, Wei

    2015-12-01

    Cystic fibrosis is a mono-genetic multi-system disease; however, respiratory manifestations cause the main morbidity and mortality where chronic bacterial infections lead to bronchiectasis and ultimately respiratory failure. Metabolomics allows a relatively complete snapshot of metabolic processes in a sample using different mass spectrometry methods. Sample types used for discovery of biomarkers or pathomechanisms in cystic fibrosis (CF) have included blood, respiratory secretions, and exhaled breath to date. Metabolomics has shown distinction of CF vs. non-CF for matrices of blood, exhaled breath, and respiratory epithelial cultures, each showing different pathways. Severity of lung disease has been addressed by studies in bronchoalveolar lavage and exhaled breath condensate showing separation by metabolites that the authors of each study related to inflammation; e.g., ethanol, acetone, purines. Lipidomics has been applied to blood and sputum samples showing associations with lung function and Pseudomonas aeruginosa infection status. Finally, studies of bacteria grown in vitro showed differences of bacterial metabolites to be associated with clinical parameters. Metabolomics, in the sense of global metabolomic profiling, is a powerful technique that has allowed discovery of pathways that had not previously been implicated in CF. These may include purines, mitochondrial pathways, and different aspects of glucose metabolism besides the known differences in lipid metabolism in CF. However, targeted studies to validate such potential metabolites and pathways of interest are necessary. Studies evaluating metabolites of bacterial origin are in their early stages. Thus further well-designed studies could be envisioned.

  19. Cystic fibrosis chronic rhinosinusitis: A comprehensive review

    PubMed Central

    Chaaban, Mohamad R.; Kejner, Alexandra; Rowe, Steven M.

    2013-01-01

    Background: Advances in the care of patients with cystic fibrosis (CF) have improved pulmonary outcomes and survival. In addition, rapid developments regarding the underlying genetic and molecular basis of the disease have led to numerous novel targets for treatment. However, clinical and basic scientific research focusing on therapeutic strategies for CF-associated chronic rhinosinusitis (CRS) lags behind the evidence-based approaches currently used for pulmonary disease. Methods: This review evaluates the available literature and provides an update concerning the pathophysiology, current treatment approaches, and future pharmaceutical tactics in the management of CRS in patients with CF. Results: Optimal medical and surgical strategies for CF CRS are lacking because of a dearth of well-performed clinical trials. Medical and surgical interventions are supported primarily by level 2 or 3 evidence and are aimed at improving clearance of mucus, infection, and inflammation. A number of novel therapeutics that target the basic defect in the cystic fibrosis transmembrane conductance regulator channel are currently under investigation. Ivacaftor, a corrector of the G551D mutation, was recently approved by the Food and Drug Administration. However, sinonasal outcomes using this and other novel drugs are pending. Conclusion: CRS is a lifelong disease in CF patients that can lead to substantial morbidity and decreased quality of life. A multidisciplinary approach will be necessary to develop consistent and evidence-based treatment paradigms. PMID:24119602

  20. Medullary cystic disease: a family study.

    PubMed

    Chen, H C; Chang, J M; Tsai, J H; Lai, Y H

    1998-03-01

    Medullary cystic disease of the kidney is characterized by progressive tubulointerstitial disease with medullary cyst formation and secondary glomerular sclerosis. We treated a patient with chronic renal failure and investigated the family history of renal disease. The patient, an 18-year-old woman, was admitted due to poor appetite and fatigue for several months. Findings on physical examination were normal except for a pale conjunctiva. Urinalysis revealed only mild proteinuria with clear sediment. The hemogram showed normocytic normochromic anemia with hemoglobin 86 g/L. The patient was azotemic and her creatinine clearance rate was 10.7 mL/min. Renal sonography showed contraction of both kidneys with a marked increase in cortical echogenicity. One small cyst was found in the medullary area. Computed tomography (CT) and magnetic resonance imaging revealed several medullary cysts. Percutaneous renal biopsy showed focal and periglomerular sclerosis, marked tubular atrophy, and interstitial fibrosis. Ten of her family members were examined for renal function, and by sonography and CT. Five had medullary cysts, and three of the five showed abnormal renal function. Medullary cystic disease should be considered in the differential diagnosis of patients with renal disease and a positive family history.

  1. [Vitamin E deficiency in cystic fibrosis].

    PubMed

    Muñoz, C; Polanco, I; Hernanz, A; Carrasco, S; Barea, I; Murga, M L; Arroba, M L; Codoceo, R

    1987-12-01

    Plasma vitamin E levels were measured by high performance liquid chromatography in 42 children with cystic fibrosis and were correlated with the following parameters: sex, age, time of follow-up, clinical evolution (Schwachman score), vitamin E/cholesterol and faecal fat excretion. All children in this study received oral alfa-tocoferol (50-100 mg daily) from the diagnosis. According to the vitamin E level patients were distributed in two groups. Group I: 27 patients (64.3%) with normal concentrations. Group II: 15 patients (35.7%) with decreases plasma levels but without clinical manifestations. Steatorrhea was present in all children except 4 patients from group I and one patient from group II. On the other hand, vitamin E/cholesterol was normal in 80% of patients with vitamin E deficiency (group II). We did not find any correlation between plasma vitamin E levels and the different clinical and biological parameters studied. Further studies should be carried out to throw more light on the mechanism underlying the pathogenesis of vitamin E deficiency in patients with cystic fibrosis.

  2. Cystic fibrosis mortality trends in France.

    PubMed

    Bellis, Gil; Cazes, Marie-Hélène; Parant, Alain; Gaimard, Maryse; Travers, Cécile; Le Roux, Evelyne; Ravilly, Sophie; Rault, Gilles

    2007-05-01

    In 1992 France set up a national cystic fibrosis observatory (Observatoire national de la mucoviscidose, ONM) to monitor the state of health of patients on an annual basis. Using the ONM data, this study estimates the main indicators for life expectancy and assesses the total number of cystic fibrosis patients. The data for the years 1994 to 2003 are divided into 3-year periods. Life tables are drawn up for these periods, from which mean and median lengths of life are determined. Using the most recent life table, the number of births in 2003 and the incidence of the disease, the total population of patients can be estimated, assuming a stationary population. In 2001-2003, life expectancy at birth of patients registered with the ONM was 39.1 years and median length of life was 36.4 years. These results, substantially better than those of 1994-1996, are linked to improved conditions of patient inclusion in the ONM database, to improvements in their healthcare, but also to the limitations of the life tables. Based on the 2003 data, the total theoretical number of patients is 6490, and coverage by the ONM database is thus 63.2%. These provisional results demonstrate the need to convert the ONM observatory into a registry providing exhaustive coverage of all patients.

  3. Comparative biology of cystic fibrosis animal models.

    PubMed

    Fisher, John T; Zhang, Yulong; Engelhardt, John F

    2011-01-01

    Animal models of human diseases are critical for dissecting mechanisms of pathophysiology and developing therapies. In the context of cystic fibrosis (CF), mouse models have been the dominant species by which to study CF disease processes in vivo for the past two decades. Although much has been learned through these CF mouse models, limitations in the ability of this species to recapitulate spontaneous lung disease and several other organ abnormalities seen in CF humans have created a need for additional species on which to study CF. To this end, pig and ferret CF models have been generated by somatic cell nuclear transfer and are currently being characterized. These new larger animal models have phenotypes that appear to closely resemble human CF disease seen in newborns, and efforts to characterize their adult phenotypes are ongoing. This chapter will review current knowledge about comparative lung cell biology and cystic fibrosis transmembrane conductance regulator (CFTR) biology among mice, pigs, and ferrets that has implications for CF disease modeling in these species. We will focus on methods used to compare the biology and function of CFTR between these species and their relevance to phenotypes seen in the animal models. These cross-species comparisons and the development of both the pig and the ferret CF models may help elucidate pathophysiologic mechanisms of CF lung disease and lead to new therapeutic approaches.

  4. Cystic Fibrosis Transmembrane Conductance Regulator (CFTR)

    PubMed Central

    Corradi, Valentina; Vergani, Paola; Tieleman, D. Peter

    2015-01-01

    The cystic fibrosis transmembrane conductance regulator (CFTR) is a member of the ATP-binding cassette (ABC) transporter superfamily. CFTR controls the flow of anions through the apical membrane of epithelia. Dysfunctional CFTR causes the common lethal genetic disease cystic fibrosis. Transitions between open and closed states of CFTR are regulated by ATP binding and hydrolysis on the cytosolic nucleotide binding domains, which are coupled with the transmembrane (TM) domains forming the pathway for anion permeation. Lack of structural data hampers a global understanding of CFTR and thus the development of “rational” approaches directly targeting defective CFTR. In this work, we explored possible conformational states of the CFTR gating cycle by means of homology modeling. As templates, we used structures of homologous ABC transporters, namely TM(287–288), ABC-B10, McjD, and Sav1866. In the light of published experimental results, structural analysis of the transmembrane cavity suggests that the TM(287–288)-based CFTR model could correspond to a commonly occupied closed state, whereas the McjD-based model could represent an open state. The models capture the important role played by Phe-337 as a filter/gating residue and provide structural information on the conformational transition from closed to open channel. PMID:26229102

  5. Targeting ion channels in cystic fibrosis.

    PubMed

    Mall, Marcus A; Galietta, Luis J V

    2015-09-01

    Mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene cause a characteristic defect in epithelial ion transport that plays a central role in the pathogenesis of cystic fibrosis (CF). Hence, pharmacological correction of this ion transport defect by targeting of mutant CFTR, or alternative ion channels that may compensate for CFTR dysfunction, has long been considered as an attractive approach to a causal therapy of this life-limiting disease. The recent introduction of the CFTR potentiator ivacaftor into the therapy of a subgroup of patients with specific CFTR mutations was a major milestone and enormous stimulus for seeking effective ion transport modulators for all patients with CF. In this review, we discuss recent breakthroughs and setbacks with CFTR modulators designed to rescue mutant CFTR including the common mutation F508del. Further, we examine the alternative chloride channels TMEM16A and SLC26A9, as well as the epithelial sodium channel ENaC as alternative targets in CF lung disease, which remains the major cause of morbidity and mortality in patients with CF. Finally, we will focus on the hurdles that still need to be overcome to make effective ion transport modulation therapies available for all patients with CF irrespective of their CFTR genotype.

  6. New horizons for cystic fibrosis treatment.

    PubMed

    Fajac, Isabelle; De Boeck, Kris

    2017-02-01

    Cystic fibrosis is an inherited multi-system disease associated with chronic lung infection, malabsorption, salt loss syndromes, male infertility and leading to numerous comorbidities. The landscape in cystic fibrosis care has changed markedly with currently more adult patients than children in many countries. Over 2000 different mutations in the CFTR gene have been reported and the majority are extremely rare. Understanding how CFTR mutations translate to disturbed synthesis or function of the CFTR protein has opened the way to 'personalized' treatments to correct the basic defect. The first 2 drugs have reached the clinic: a CFTR potentiator to augment CFTR channel function, and the combination of this potentiator with a corrector to increase CFTR expression at the cell membrane. To obtain robust correction of CFTR expression at the cell membrane, combinations of correctors with additive efficacy are under investigation. Other mutation type-specific treatments under clinical investigation are premature stop codon-read through drugs and antisense oligonucleotides that correct the basic defect at the mRNA level. Restoring the defective gene by gene editing can already be achieved ex vivo. Mutation agnostic treatments are explored as well: stabilizing CFTR expression at the cell membrane, circumventing the CFTR channel by blocking or activating other ion channels, and gene therapy. Combinations of these therapies can be anticipated. The pipeline of corrective strategies under clinical investigation is increasing continuously and a rising number of pharmaceutical companies are entering the field. Copyright © 2016 Elsevier Inc. All rights reserved.

  7. Cystic Fibrosis and the Nervous System.

    PubMed

    Reznikov, Leah R

    2017-05-01

    Cystic fibrosis (CF) is a life-shortening autosomal recessive disorder caused by mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR). CFTR is an anion channel that conducts bicarbonate and chloride across cell membranes. Although defective anion transport across epithelial cells is accepted as the basic defect in CF, many of the features observed in people with CF and organs affected by CF are modulated by the nervous system. This is of interest because CFTR expression has been reported in both the peripheral and central nervous systems, and it is well known that the transport of anions, such as chloride, greatly modulates neuronal excitability. Thus it is predicted that in CF, lack of CFTR in the nervous system affects neuronal function. Consistent with this prediction, several nervous system abnormalities and nervous system disorders have been described in people with CF and in animal models of CF. The goal of this special feature article is to highlight the expression and function of CFTR in the nervous system. Special emphasis is placed on nervous system abnormalities described in people with CF and in animal models of CF. Finally, features of CF that may be modulated by or attributed to faulty nervous system function are discussed. Copyright © 2016 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.

  8. Imaging the Abdominal Manifestations of Cystic Fibrosis

    PubMed Central

    McDermott, S.; Chan, V. O.; Ridge, C. A.

    2017-01-01

    Cystic fibrosis (CF) is a multisystem disease with a range of abdominal manifestations including those involving the liver, pancreas, and kidneys. Recent advances in management of the respiratory complications of the disease has led to a greater life expectancy in patients with CF. Subsequently, there is increasing focus on the impact of abdominal disease on quality of life and survival. Liver cirrhosis is the most important extrapulmonary cause of death in CF, yet significant challenges remain in the diagnosis of CF related liver disease. The capacity to predict those patients at risk of developing cirrhosis remains a significant challenge. We review representative abdominal imaging findings in patients with CF selected from the records of two academic health centres, with a view to increasing familiarity with the abdominal manifestations of the disease. We review their presentation and expected imaging findings, with a focus on the challenges facing diagnosis of the hepatic manifestations of the disease. An increased familiarity with these abdominal manifestations will facilitate timely diagnosis and management, which is paramount to further improving outcomes for patients with cystic fibrosis. PMID:28250993

  9. Stereotaxic intracavitary irradiation for cystic craniopharyngiomas

    SciTech Connect

    Pollack, I.F.; Lunsford, L.D.; Slamovits, T.L.; Gumerman, L.W.; Levine, G.; Robinson, A.G.

    1988-02-01

    Stereotaxic intracavitary irradiation with instillation of phosphorus-32 (/sup 32/P) colloidal chromic phosphate was performed in nine patients with cystic craniopharyngiomas. Serial neurological, ophthalmological, neuroendocrinological, and radiological examinations were performed before and after treatment. Dosimetry was determined based on a computerized tomography (CT) estimation of tumor volume, and was calculated to provide a tumoricidal dose (200 to 300 Gy) to the cyst wall. The follow-up period ranged from 14 to 45 months (mean 27 months). After treatment, all nine patients showed improvement of symptoms and radiological evidence of cyst regression. Because of an expanding solid component producing recurrent symptoms, one patient required a craniotomy 14 months after isotope instillation. Three of five patients with impaired visual acuity before surgery had significant improvement in acuity after treatment. Preoperative visual field defects in eight patients improved in four after /sup 32/P therapy. Of seven patients with preoperative endocrine abnormalities, one individual showed almost complete normalization and another had improvement in endocrine function. Patients who exhibited residual neuroendocrine function before isotope instillation developed no significant deterioration in endocrine status during the follow-up period. The findings suggest that stereotaxic intracavitary irradiation is a safe and effective treatment which should be considered as the initial surgery for cystic craniopharyngiomas.

  10. [Adenoid cystic carcinoma of the lacrimal gland].

    PubMed

    Holz, F G; Tetz, M; Born, I A; Völcker, H E

    1992-07-01

    A 38-year-old female presented with a left-sided peripheral N. VI-paresis and negative neuroradiologic work-up. After one year she developed an incomplete N. III paresis, numbness of her face correlating to N. V1 and V2, reduced lacrimal secretion as well as a palpable mass at the lateral orbital rim. Magnetic resonance imaging revealed an orbital tumor extending into the cavernous sinus. Biopsy disclosed an adenoid cystic carcinoma of the lacrimal gland with basaloid, cribriform and tubular pattern. Therefore, an exenteration with postoperative radiation therapy was performed. Immunohistochemical findings included positive reactions for keratin (KL-1 and AE 1 + 3), Vimentin and Egp 34. By determination of Ki 67 the proliferative activity of the tumor was found to be 15%. Our case report demonstrates that even in absence of an exophthalmus or pain a malignant orbital tumor may be assumed as the underlying cause for a combined monolateral oculomotor and trigeminal paresis. Immunohistochemical results support the notion, that the adenoid cystic carcinoma of the lacrimal gland may arise from precursor cells of the terminal duct system.

  11. CFTR protein repair therapy in cystic fibrosis.

    PubMed

    Quintana-Gallego, Esther; Delgado-Pecellín, Isabel; Calero Acuña, Carmen

    2014-04-01

    Cystic fibrosis is a single gene, autosomal recessive disorder, in which more than 1,900 mutations grouped into 6 classes have been described. It is an example a disease that could be well placed to benefit from personalised medicine. There are currently 2 very different approaches that aim to correct the basic defect: gene therapy, aimed at correcting the genetic alteration, and therapy aimed at correcting the defect in the CFTR protein. The latter is beginning to show promising results, with several molecules under development. Ataluren (PTC124) is a molecule designed to make the ribosomes become less sensitive to the premature stop codons responsible for class i mutations. Lumacaftor (VX-809) is a CFTR corrector directed at class ii mutations, among which Phe508del is the most frequent, with encouraging results. Ivacaftor (VX-770) is a potentiator, the only one marketed to date, which has shown good efficacy for the class iii mutation Gly551Asp in children over the age of 6 and adults. These drugs, or a combination of them, are currently undergoing various clinical trials for other less common genetic mutations. In the last 5 years, CFTR has been designated as a therapeutic target. Ivacaftor is the first drug to treat the basic defect in cystic fibrosis, but only provides a response in a small number of patients. New drugs capable of restoring the CFTR protein damaged by the most common mutations are required.

  12. Cystic echinococcosis in Greece. Past and present.

    PubMed

    Sotiraki, S; Chaligiannis, I

    2010-09-01

    Cystic echinococcosis is a zoonotic disease with a wide geographical distribution, Greece included, and is considered to be a serious problem for the public health and the livestock economy. Although the disease was widespread in Greece since ancient times, cystic echinococcosis was identified as a serious problem around 1970, and since then national surveillance programmes are running, based on meat inspection and stray dogs management. Ever since, there are official records of the parasite's prevalence in humans and livestock which show a continuous decline. More precisely, human hydatidosis, according to the official records, declines from an annual incidence of 14.8 per 100,000 inhabitants during 1967-1971 to 0.3 in 2008. Late surveys reveal that in Greece the prevalence of echinococcosis was 23-39.2% for sheep, 7.6-14.7% for goats, 0% in cattle and 0.6% in pigs, while further molecular analyses in Southern Greece showed the existence of the genotypes G1 and G3 in sheep and G7 in goats in that area. All data presented demonstrate that the parasite is still present in Greece. Surveillance is nowadays being performed under EU regulations but it is highly important to improve and adopt corrective and preventive measures to avoid animal and human infection.

  13. Oscillating devices for airway clearance in people with cystic fibrosis.

    PubMed

    Morrison, Lisa; Innes, Stephanie

    2017-05-04

    Chest physiotherapy is widely prescribed to assist the clearance of airway secretions in people with cystic fibrosis. Oscillating devices generate intra- or extra-thoracic oscillations orally or external to the chest wall. Internally they create variable resistances within the airways, generating controlled oscillating positive pressure which mobilises mucus. Extra-thoracic oscillations are generated by forces outside the respiratory system, e.g. high frequency chest wall oscillation. This is an update of a previously published review. To identify whether oscillatory devices, oral or chest wall, are effective for mucociliary clearance and whether they are equivalent or superior to other forms of airway clearance in the successful management of secretions in people with cystic fibrosis. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register comprising references identified from comprehensive electronic database searches and hand searches of relevant journals and abstract books of conference proceedings. Latest search of the Cystic Fibrosis Trials Register: 27 April 2017.In addition we searched the trials databases ClinicalTrials.gov and the WHO International Clinical Trials Registry Platform. Latest search of trials databases: 26 April 2017. Randomised controlled studies and controlled clinical studies of oscillating devices compared with any other form of physiotherapy in people with cystic fibrosis. Single-treatment interventions (therapy technique used only once in the comparison) were excluded. Two authors independently applied the inclusion criteria to publications and assessed the quality of the included studies. The searches identified 76 studies (302 references); 35 studies (total of 1138 participants) met the inclusion criteria. Studies varied in duration from up to one week to one year; 20 of the studies were cross-over in design. The studies also varied in type of intervention and the outcomes measured, data were not

  14. Prenatal screening for cystic fibrosis carriers: an economic evaluation.

    PubMed

    Rowley, P T; Loader, S; Kaplan, R M

    1998-10-01

    The cloning of the CFTR gene has made it technically possible to avert the unwanted birth of a child with cystic fibrosis (CF). Several large trials offering prenatal CF carrier screening suggest that such screening is practical and that identified carriers generally use the information obtained. Therefore, a critical question is whether the cost of such screening is justified. Decision analysis was performed that used information about choices that pregnant women were observed to make at each stage in the Rochester prenatal carrier-screening trial. The cost of screening per CF birth voluntarily averted was estimated to be $1,320,000-$1,400,000. However, the lifetime medical cost of the care of a CF child in today's dollars was estimated to be slightly>$1,000,000. Therefore, despite both the high cost of carrier testing and the relative infrequency of CF conceptions in the general population, the averted medical-care cost resulting from choices freely made are estimated to offset approximately 74%-78% of the costs of a screening program. At present, if it is assumed that a pregnancy terminated because of CF is replaced, the marginal cost for prenatal CF carrier screening is estimated to be $8,290 per quality-adjusted life-year. This value compares favorably with that of many accepted medical services. The cost of prenatal CF carrier screening could fall to equal the averted costs of CF patient care if the cost of carrier testing were to fall to $100.

  15. Prenatal screening for cystic fibrosis carriers: an economic evaluation.

    PubMed Central

    Rowley, P T; Loader, S; Kaplan, R M

    1998-01-01

    The cloning of the CFTR gene has made it technically possible to avert the unwanted birth of a child with cystic fibrosis (CF). Several large trials offering prenatal CF carrier screening suggest that such screening is practical and that identified carriers generally use the information obtained. Therefore, a critical question is whether the cost of such screening is justified. Decision analysis was performed that used information about choices that pregnant women were observed to make at each stage in the Rochester prenatal carrier-screening trial. The cost of screening per CF birth voluntarily averted was estimated to be $1,320,000-$1,400,000. However, the lifetime medical cost of the care of a CF child in today's dollars was estimated to be slightly>$1,000,000. Therefore, despite both the high cost of carrier testing and the relative infrequency of CF conceptions in the general population, the averted medical-care cost resulting from choices freely made are estimated to offset approximately 74%-78% of the costs of a screening program. At present, if it is assumed that a pregnancy terminated because of CF is replaced, the marginal cost for prenatal CF carrier screening is estimated to be $8,290 per quality-adjusted life-year. This value compares favorably with that of many accepted medical services. The cost of prenatal CF carrier screening could fall to equal the averted costs of CF patient care if the cost of carrier testing were to fall to $100. PMID:9758600

  16. Cystic degeneration of neuro endocrine tumor of pancreas and Crohn's disease: true or coincidental association?

    PubMed

    Hedfi, Mohamed; Abbasi, Imed; Intissar, Chibani; Salwa, Ammar; Chouchen, Adnen

    2016-01-01

    Pancreatic endocrine tumors (PETs) or islet cell tumors are rare lesions, the incidence of which is estimated to be less than 1 per 100,000 person-years in the general population. PETs can be divided into functional (exhibit a distinct clinical syndrome due to hormone hypersecretion) and non-functional tumors. The majority of PETs are non-functional. In spite of their rarity, cystic neoplasms of the pancreas are characterized by existing or potential malignancy that cannot be ignored during decisive process with regard to the choice of treatment. The purpose of this workis to find an association with Crohn's disease and cystic degeneration of a neuroendocrine tumor of the pancreas. Crohn's disease may affect extraintestinal organs, including the pancreas. In such cases, It seems certain that many patients diagnosed with Crohn disease (CD) are predisposed to a wider spectrum of cancers. We present a case of pancreatic cyst with no typical features of pseudocyst in the medical interview, with history of Crohn's disease, treated by caudal pancreatectomy. We tried to evaluate the clinical and morphological features of so-called cystic neoplasms associated with inflammatory bowel disease and to define their pathological characteristics.

  17. Subepithelial fibrosis and degradation of the bronchial extracellular matrix in cystic fibrosis.

    PubMed

    Durieu, I; Peyrol, S; Gindre, D; Bellon, G; Durand, D V; Pacheco, Y

    1998-08-01

    Cystic fibrosis is a genetic disease caused by mutations of the cystic fibrosis transmembrane conductance regulator gene. Chronic inflammation and proteolysis lead to progressive damage of the bronchial wall. Extracellular matrix determines the structural organization and the mechanical properties of lung airways. It was thus examined in nine patients with cystic fibrosis (six bronchial biopsies and three lobectomies) in order to assess its level of alteration. The submucosal changes in matrix protein distribution were analyzed by immunochemistry and electron microscopy: the subepithelial basal lamina was thinned; an acellular collagen fiber layer composed of interstitial collagens (types I and III) subtended by tenascin and devoid of elastin-associated microfibrils was deposited beneath the basal lamina; this dense fibrous deposit generally formed a thick layer and could extend into the bronchial wall; the bronchial elastic framework lost arborescent distribution and appeared slender, packed, or lacunar; ultrastructural observation gave evidence for elastic and collagenic fiber lysis. Proteolytic activity is probably the major cause of matrix degradation. Fibrosis appears as a repair process rather than as an active fibrogenesis. The reversibility of extracellular matrix alterations is an important challenge and various interventions such as anti-inflammatory treatments can be targeted to halt or reverse this degradation process.

  18. Application of mass spectrometry to study proteomics and interactomics in cystic fibrosis.

    PubMed

    Balch, William E; Yates, John R

    2011-01-01

    The cystic fibrosis transmembrane conductance regulator (CFTR) does not function in isolation, but rather in a complex network of protein-protein interactions that dictate the physiology of a healthy cell and tissue and, when defective, the pathophysiology characteristic of cystic fibrosis (CF) disease. To begin to address the organization and operation of the extensive cystic fibrosis protein network dictated by simultaneous and sequential interactions, it will be necessary to understand the global protein environment (the proteome) in which CFTR functions in the cell and the local network that dictates CFTR folding, trafficking, and function at the cell surface. Emerging mass spectrometry (MS) technologies and methodologies offer an unprecedented opportunity to fully characterize both the proteome and the protein interactions directing normal CFTR function and to define what goes wrong in disease. Below we provide the CF investigator with a general introduction to the capabilities of modern mass spectrometry technologies and methodologies with the goal of inspiring further application of these technologies for development of a basic understanding of the disease and for the identification of novel pathways that may be amenable to therapeutic intervention in the clinic.

  19. Biliary liver cirrhosis secondary to cystic fibrosis: a rare indication for liver transplantation.

    PubMed

    Sańko-Resmer, J; Paczek, L; Wyzgał, J; Ziółkowski, J; Ciszek, M; Alsharabi, A; Grzelak, I; Paluszkiewicz, R; Patkowski, W; Krawczyk, M

    2006-01-01

    As more effective therapies prolong the lives of patients with cystic fibrosis, there are now more patients in this population diagnosed with liver diseases. Secondary biliary cirrhosis is not a rare complication of mucoviscidosis. It is diagnosed in 20% of patients with mucoviscidosis; in 2% it is accompanied by portal hypertension. On average patients with portal hypertension and its complications are 12 years old. Liver transplantation is an accepted method of treatment for children with cystic fibrosis and portal hypertension. It eliminates the cause of the portal hypertension, decreases life-threatening medical conditions, and improves their nutritional status and quality of life. Despite immunosuppressive treatment they do not seem to beat increased risk of upper respiratory tract infections. On the contrary improved respiratory function and status are generally observed. We present our first case of orthotopic liver transplantation performed in a 29-year-old man with cystic fibrosis. The donor was a 42-year-old woman who died of a ruptured cerebral aneurysm. The surgery was performed in September 2004. The patient received immunosuppression based on steroids, basiliximab, tacrolimus, and mycophenolic acid due to renal insufficiency. Antibiotic (meropenem) and antiviral prophylaxis (gancyclovir) were used. A 6-month period of observation confirmed the clinical data from the pediatric population-a good prognosis with improved nutritional status, respiratory function, and quality of life.

  20. A case report: Giant cystic parathyroid adenoma presenting with parathyroid crisis after Vitamin D replacement

    PubMed Central

    2012-01-01

    Background Parathyroid adenoma with cystic degeneration is a rare cause of primary hyperparathyroidism. The clinical and biochemical presentation may mimic parathyroid carcinoma. Case presentation We report the case of a 55 year old lady, who had longstanding history of depression and acid peptic disease. Serum calcium eight months prior to presentation was slightly high, but she was never worked up. She was found to be Vitamin D deficient while being investigated for generalized body aches. A month after she was replaced with Vitamin D, she presented to us with parathyroid crisis. Her corrected serum calcium was 23.0 mg/dL. She had severe gastrointestinal symptoms and acute kidney injury. She had unexplained consistent hypokalemia until surgery. Neck ultrasound and CT scan revealed giant parathyroid cyst extending into the mediastinum. After initial medical management for parathyroid crisis, parathyroid cystic adenoma was surgically excised. Her serum calcium, intact parathyroid hormone, creatinine and potassium levels normalized after surgery. Conclusion This case of parathyroid crisis, with very high serum calcium and parathyroid hormone levels, is a rare presentation of parathyroid adenoma with cystic degeneration. This case also highlights that Vitamin D replacement may unmask subclinical hyperparathyroidism. Consistent hypokalemia until surgery merits research into its association with hypercalcemia. PMID:22840059

  1. Human Genome Project and cystic fibrosis--a symbiotic relationship.

    PubMed

    Tolstoi, L G; Smith, C L

    1999-11-01

    When Watson and Crick determined the structure of DNA in 1953, a biological revolution began. One result of this revolution is the Human Genome Project. The primary goal of this international project is to obtain the complete nucleotide sequence of the human genome by the year 2005. Although molecular biologists and geneticists are most enthusiastic about the Human Genome Project, all areas of clinical medicine and fields of biology will be affected. Cystic fibrosis is the most common, inherited, lethal disease of white persons. In 1989, researchers located the cystic fibrosis gene on the long arm of chromosome 7 by a technique known as positional cloning. The most common mutation (a 3-base pair deletion) of the cystic fibrosis gene occurs in 70% of patients with cystic fibrosis. The knowledge gained from genetic research on cystic fibrosis will help researchers develop new therapies (e.g., gene) and improve standard therapies (e.g., pharmacologic) so that a patient's life span is increased and quality of life is improved. The purpose of this review is twofold. First, the article provides an overview of the Human Genome Project and its clinical significance in advancing interdisciplinary care for patients with cystic fibrosis. Second, the article includes a discussion of the genetic basis, pathophysiology, and management of cystic fibrosis.

  2. Systemic corticosteroid and isotretinoin treatment in cystic acne.

    PubMed

    Karvonen, S L; Vaalasti, A; Kautiainen, H; Reunala, T

    1993-12-01

    Prednisolone combined with erythromycin was given to 6 patients with cystic acne. The treatment responses were compared to those in 6 patients with cystic acne receiving isotretinoin and erythromycin and also to those in 3 patients with acne fulminans treated with prednisolone and erythromycin. During the first 4 weeks cystic acne showed a clear improvement in 5 out of 6 patients in both treatment groups. A similar improvement occurred in all 3 patients with acne fulminans. When corticosteroid was stopped, 2 out of 5 patients with cystic acne had a relapse and needed isotretinoin for complete control. In the isotretinoin-treated group, one patient with cystic acne needed prednisolone because the acne worsened to an ulcerative form. Slightly elevated liver enzymes, possibly due to erythromycin treatment, were observed in 2 patients with cystic acne and in one patient with acne fulminans. The present results show that prednisolone combined with erythromycin is an effective treatment during the early stages of cystic and febrile acne, but isotretinoin is needed for long-term control.

  3. Diagnosing cystic fibrosis-related diabetes: current methods and challenges.

    PubMed

    Prentice, Bernadette; Hameed, Shihab; Verge, Charles F; Ooi, Chee Y; Jaffe, Adam; Widger, John

    2016-07-01

    Cystic fibrosis-related diabetes (CFRD) is the end-point of a spectrum of glucose abnormalities in cystic fibrosis that begins with early insulin deficiency and ultimately results in accelerated nutritional decline and loss of lung function. Current diagnostic and management regimens are unable to entirely reverse this clinical decline. This review summarises the current understanding of the pathophysiology of CFRD, the issues associated with using oral glucose tolerance tests in CF and the challenges faced in making the diagnosis of CFRD. Medline database searches were conducted using search terms "Cystic Fibrosis Related Diabetes", "Cystic Fibrosis" AND "glucose", "Cystic Fibrosis" AND "insulin", "Cystic Fibrosis" AND "Diabetes". Additionally, reference lists were studied. Expert commentary: Increasing evidence points to early glucose abnormalities being clinically relevant in cystic fibrosis and as such novel diagnostic methods such as continuous glucose monitoring or 30 minute sampled oral glucose tolerance test (OGTT) may play a key role in the future in the screening and diagnosis of early glucose abnormalities in CF.

  4. Surgical Outcome of Idiopathic Epiretinal Membranes with Intraretinal Cystic Spaces

    PubMed Central

    Shiode, Yusuke; Morizane, Yuki; Toshima, Shinji; Kimura, Shuhei; Kumase, Fumiaki; Hosokawa, Mio; Hirano, Masayuki; Doi, Shinichiro; Takahashi, Kosuke; Hosogi, Mika; Fujiwara, Atsushi; Shiraga, Fumio

    2016-01-01

    Objective To investigate the occurrence ratio, localization, and surgical outcomes of intraretinal cystic spaces in idiopathic epiretinal membranes (ERMs). Methods We retrospectively reviewed the charts of 432 eyes of 398 consecutive patients with idiopathic ERM who underwent vitrectomy and ERM peeling from January 2012 to September 2015. We selected cases with intraretinal cystic space prior to surgery, detected by spectral-domain optical coherence tomography. We then evaluated the effects of ERM peeling on intraretinal cystic spaces, best corrected visual acuity, and central retinal thickness at 6 months after surgery. Results Twenty-four eyes (5.5%) showed intraretinal cystic spaces before surgery, present in the inner retinal layer (the inner group) in 9 eyes, in the outer retinal layer (the outer group) in 6 eyes, and in both the inner and the outer retinal layers (the combined group) in 9 eyes. Additionally, 30 eyes with ERM but without any presence of intraretinal cystic space were selected randomly and classified as the no cyst group. At 6 months after surgery, the disappearance rate of cystic spaces was significantly greater for the outer group than for the inner group (83.3% and 11.1%, respectively, P = 0.011). The mean best corrected visual acuity improved significantly after surgery in the inner group, the outer group, and the no cyst group (P < 0.05 for all three groups) but did not improve in the combined group (P = 0.58). The mean central retinal thickness decreased significantly after surgery in the inner group, the combined group, and the no cyst group (P < 0.05). Conclusions Intraretinal cystic spaces were observed in 5.5% of preoperative idiopathic ERM cases. Following surgery, the cystic spaces in the outer retinal layer disappeared at higher rates than those in the inner retinal layer, suggesting that the pathophysiologies of these cystic spaces are different. PMID:27992520

  5. Air Trapping and Airflow Obstruction in Newborn Cystic Fibrosis Piglets

    PubMed Central

    Adam, Ryan J.; Michalski, Andrew S.; Bauer, Christian; Abou Alaiwa, Mahmoud H.; Gross, Thomas J.; Awadalla, Maged S.; Bouzek, Drake C.; Gansemer, Nicholas D.; Taft, Peter J.; Hoegger, Mark J.; Diwakar, Amit; Ochs, Matthias; Reinhardt, Joseph M.; Hoffman, Eric A.; Beichel, Reinhard R.; Meyerholz, David K.

    2013-01-01

    Rationale: Air trapping and airflow obstruction are being increasingly identified in infants with cystic fibrosis. These findings are commonly attributed to airway infection, inflammation, and mucus buildup. Objectives: To learn if air trapping and airflow obstruction are present before the onset of airway infection and inflammation in cystic fibrosis. Methods: On the day they are born, piglets with cystic fibrosis lack airway infection and inflammation. Therefore, we used newborn wild-type piglets and piglets with cystic fibrosis to assess air trapping, airway size, and lung volume with inspiratory and expiratory X-ray computed tomography scans. Micro–computed tomography scanning was used to assess more distal airway sizes. Airway resistance was determined with a mechanical ventilator. Mean linear intercept and alveolar surface area were determined using stereologic methods. Measurements and Main Results: On the day they were born, piglets with cystic fibrosis exhibited air trapping more frequently than wild-type piglets (75% vs. 12.5%, respectively). Moreover, newborn piglets with cystic fibrosis had increased airway resistance that was accompanied by luminal size reduction in the trachea, mainstem bronchi, and proximal airways. In contrast, mean linear intercept length, alveolar surface area, and lung volume were similar between both genotypes. Conclusions: The presence of air trapping, airflow obstruction, and airway size reduction in newborn piglets with cystic fibrosis before the onset of airway infection, inflammation, and mucus accumulation indicates that cystic fibrosis impacts airway development. Our findings suggest that early airflow obstruction and air trapping in infants with cystic fibrosis might, in part, be caused by congenital airway abnormalities. PMID:24168209

  6. Stimulatory and inhibitory histamine receptors in canine cystic duct.

    PubMed Central

    Clanachan, A. S.; Courtney, D. F.; Scott, G. W.

    1982-01-01

    1 The effects of histamine receptor stimulation were assessed on the resistance of the canine cystic duct in vivo and on the contractility of circular muscle preparations of canine cystic duct in vitro. 2 In anaesthetized dogs, the H1-receptor agonist, 2-pyridylethylamine (0.05 to 15 mumol, i.a.), elicited dose-dependent increases in cystic duct resistance, whereas the H2-receptor agonist, 4-methylhistamine (0.05 to 15 mumol, i.a.) decreased cystic duct resistance. These responses were antagonized by the H1-receptor antagonist, diphenhydramine, and the H2-receptor antagonist, cimetidine, respectively. 3 Histamine (0.1 to 3000 nmol, i.a.) also increased cystic duct resistance in vivo. In the presence of diphenhydramine, the stimulatory effect of histamine was antagonized and slight decreases in cystic duct resistance became apparent. Cimetidine or prazosin also antagonized the stimulatory effects of histamine. 4 Histamine (1 to 100 microM) or 2-pyridylethylamine (1 to 100 microM) contracted, whereas 4-methylhistamine (1 to 100 microM) relaxed, circular muscle preparations of cystic duct. These excitatory and inhibitory responses were antagonized by diphenhydramine and cimetidine, respectively. 5 These results indicate that the canine cystic duct possesses excitatory H1- and inhibitory H2-receptors. The predominant effect of histamine is an H1-receptor-mediated increase in cystic duct resistance. Histamine, which may be released in association with cholecystitis, may exert significant effects on the regulation of bile flow in and out of the gallbladder and may contribute to gallbladder stasis during biliary disease. PMID:7150877

  7. Physiological impact of abnormal lipoxin A₄ production on cystic fibrosis airway epithelium and therapeutic potential.

    PubMed

    Higgins, Gerard; Ringholz, Fiona; Buchanan, Paul; McNally, Paul; Urbach, Valérie

    2015-01-01

    Lipoxin A4 has been described as a major signal for the resolution of inflammation and is abnormally produced in the lungs of patients with cystic fibrosis (CF). In CF, the loss of chloride transport caused by the mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) Cl(-) channel gene results in dehydration, mucus plugging, and reduction of the airway surface liquid layer (ASL) height which favour chronic lung infection and neutrophil based inflammation leading to progressive lung destruction and early death of people with CF. This review highlights the unique ability of LXA4 to restore airway surface hydration, to stimulate airway epithelial repair, and to antagonise the proinflammatory program of the CF airway, circumventing some of the most difficult aspects of CF pathophysiology. The report points out novel aspects of the cellular mechanism involved in the physiological response to LXA4, including release of ATP from airway epithelial cell via pannexin channel and subsequent activation of and P2Y11 purinoreceptor. Therefore, inadequate endogenous LXA4 biosynthesis reported in CF exacerbates the ion transport abnormality and defective mucociliary clearance, in addition to impairing the resolution of inflammation, thus amplifying the vicious circle of airway dehydration, chronic infection, and inflammation.

  8. Physiological Impact of Abnormal Lipoxin A4 Production on Cystic Fibrosis Airway Epithelium and Therapeutic Potential

    PubMed Central

    Higgins, Gerard; McNally, Paul; Urbach, Valérie

    2015-01-01

    Lipoxin A4 has been described as a major signal for the resolution of inflammation and is abnormally produced in the lungs of patients with cystic fibrosis (CF). In CF, the loss of chloride transport caused by the mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) Cl− channel gene results in dehydration, mucus plugging, and reduction of the airway surface liquid layer (ASL) height which favour chronic lung infection and neutrophil based inflammation leading to progressive lung destruction and early death of people with CF. This review highlights the unique ability of LXA4 to restore airway surface hydration, to stimulate airway epithelial repair, and to antagonise the proinflammatory program of the CF airway, circumventing some of the most difficult aspects of CF pathophysiology. The report points out novel aspects of the cellular mechanism involved in the physiological response to LXA4, including release of ATP from airway epithelial cell via pannexin channel and subsequent activation of and P2Y11 purinoreceptor. Therefore, inadequate endogenous LXA4 biosynthesis reported in CF exacerbates the ion transport abnormality and defective mucociliary clearance, in addition to impairing the resolution of inflammation, thus amplifying the vicious circle of airway dehydration, chronic infection, and inflammation. PMID:25866809

  9. Enteral tube feeding for individuals with cystic fibrosis: Cystic Fibrosis Foundation evidence-informed guidelines.

    PubMed

    Schwarzenberg, Sarah Jane; Hempstead, Sarah E; McDonald, Catherine M; Powers, Scott W; Wooldridge, Jamie; Blair, Shaina; Freedman, Steven; Harrington, Elaine; Murphy, Peter J; Palmer, Lena; Schrader, Amy E; Shiel, Kyle; Sullivan, Jillian; Wallentine, Melissa; Marshall, Bruce C; Leonard, Amanda Radmer

    2016-11-01

    Nutrition is integral to the care of individuals with cystic fibrosis (CF). Better nutritional status is associated with improved pulmonary function. In some individuals with CF, enteral tube feeding can be useful in achieving optimal nutritional status. Current nutrition guidelines do not include detailed recommendations for enteral tube feeding. The Cystic Fibrosis Foundation convened an expert panel to develop enteral tube feeding recommendations based on a systematic review of the evidence and expert opinion. These guidelines address when to consider enteral tube feeding, assessment of confounding causes of poor nutrition in CF, preparation of the patient for placement of the enteral feeding tube, management of the tube after placement and education about enteral feeding. These recommendations are intended to guide the CF care team, individuals with CF, and their families through the enteral tube feeding process.

  10. Flow in Idealised Compliant Human Cystic Duct Models

    NASA Astrophysics Data System (ADS)

    Al-Atabi, M.; Chin, S. B.; Beck, S.; Luo, X. Y.

    The functions of the cystic duct and the role of its complicated geometry (in particular the valves of Heister), in the delivery of bile flow have always been a subject of speculation. This paper reports on an experimental investigation of the flow in idealised, compliant cystic duct models. The valves of Heister were idealised using eight semi-circular alternating baffles fitted inside the compliant tubes. These tubes were arranged in configurations representing the anatomical configurations of real cystic ducts. Models both with and without baffles were tested for comparison. The results show that the valves of Heister seem to play a role in facilitating both the filling and emptying of the gallbladder.

  11. Late presentation of congenital cystic adenomatoid malformation of the lung

    SciTech Connect

    Hulnick, D.H.; Naidich, D.P.; McCauley, D.I.; Feiner, H.D.; Avitabile, A.M.; Greco, M.A.; Genieser, N.B.

    1984-06-01

    Although most often recognized in neonates and young children, congenital cystic adenomatoid malformation of the lung (CCAM) occasionally appears in later years. Three patients, aged 35, 24, and 7 years, are reported. Chest radiographs in each case suggested a localized patchy density, a cystic mass, or a multicystic mass, but computed tomography (CT) best demonstrated the cystic and solid components while ruling out bronchiectasis or major bronchial obstruction. Bronchography contributed no further diagnostic information compared with CT. Each patient underwent lobectomy. Histologically, the characteristic overgrowth of bronchiolar elements replacing normal parenchymal architecture was accompanied by some superimposed inflammatory change.

  12. Cystic fibrosis pulmonary guidelines: treatment of pulmonary exacerbations.

    PubMed

    Flume, Patrick A; Mogayzel, Peter J; Robinson, Karen A; Goss, Christopher H; Rosenblatt, Randall L; Kuhn, Robert J; Marshall, Bruce C

    2009-11-01

    The natural history of cystic fibrosis lung disease is one of chronic progression with intermittent episodes of acute worsening of symptoms frequently called acute pulmonary exacerbations These exacerbations typically warrant medical intervention. It is important that appropriate therapies are recommended on the basis of available evidence of efficacy and safety. The Cystic Fibrosis Foundation therefore established a committee to define the key questions related to pulmonary exacerbations, review the clinical evidence using an evidence-based methodology, and provide recommendations to clinicians. It is hoped that these guidelines will be helpful to clinicians in the treatment of individuals with cystic fibrosis.

  13. [A case of cystic pheochromocytoma mimicking liver abscess].

    PubMed

    Toyoshima, Yuta; Hosokawa, Yukinari; Takada, Satoshi; Hayashi, Yoshiki; Fujimoto, Kiyohide; Hirao, Yoshihiko

    2011-07-01

    A 64-year-old man presented to our hospital feeling ill with epigastralgia. Computed tomography (CT) showed right suprarenal cystic tumor. High urinary catecholamine level was noted. Based on metaiodobenzylguanidine (MIBG) scintigraphy, magnetic resonance imaging and blood tests, preoperative diagnosis was adrenal pheochromocytoma. En-bloc resection of the tumor and the right kidney was performed. The cyst contained yellowish serous fluid, which had a catecholamine level about 3,000 times that in the blood. The histological diagnosis was cystic pheochromocytoma. Pathogenesis of cystic adrenal tumor is discussed briefly.

  14. Cystic change in primary paediatric optic nerve sheath meningioma.

    PubMed

    Narayan, Daniel; Rajak, Saul; Patel, Sandy; Selva, Dinesh

    2016-08-01

    Primary optic nerve sheath meningiomas (PONSM) are rare in children. Cystic meningiomas are an uncommon subgroup of meningiomas. We report a case of paediatric PONSM managed using observation alone that underwent cystic change and radiological regression. A 5-year-old girl presented with visual impairment and proptosis. Magnetic resonance (MR) imaging demonstrated a PONSM. The patient was left untreated and followed up with regular MR imaging. Repeat imaging at 16 years of age showed the tumour had started to develop cystic change. Repeat imaging at 21 years of age showed the tumour had decreased in size.

  15. Spectrum and Classification of Cystic Neoplasms of the Pancreas.

    PubMed

    Greer, Jonathan B; Ferrone, Cristina R

    2016-04-01

    As patients are living longer and axial imaging is more widespread, increasing numbers of cystic neoplasms of the pancreas are found. Intraductal papillary mucinous neoplasms and mucinous cystic neoplasms are the most common. The revised Sendai guidelines provide a safe algorithm for expectant management of certain cystic neoplasms; however, studies are ongoing to identify further subgroups that can be treated nonoperatively. For those patients with high-risk clinical features or symptoms, surgical resection can be performed safely at high-volume pancreatic centers. Accurate diagnosis is critical for accurate decision making.

  16. Non-invasive ventilation for cystic fibrosis.

    PubMed

    Moran, Fidelma; Bradley, Judy M; Piper, Amanda J

    2017-02-20

    Non-invasive ventilation may be a means to temporarily reverse or slow the progression of respiratory failure in cystic fibrosis by providing ventilatory support and avoiding tracheal intubation. Using non-invasive ventilation, in the appropriate situation or individuals, can improve lung mechanics through increasing airflow and gas exchange and decreasing the work of breathing. Non-invasive ventilation thus acts as an external respiratory muscle. This is an update of a previously published review. To compare the effect of non-invasive ventilation versus no non-invasive ventilation in people with cystic fibrosis for airway clearance, during sleep and during exercise. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register comprising references identified from comprehensive electronic database searches, handsearching relevant journals and abstract books of conference proceedings. We searched the reference lists of each trial for additional publications possibly containing other trials.Most recent search: 08 August 2016. Randomised controlled trials comparing a form of pressure preset or volume preset non-invasive ventilation to no non-invasive ventilation used for airway clearance or during sleep or exercise in people with acute or chronic respiratory failure in cystic fibrosis. Three reviewers independently assessed trials for inclusion criteria and methodological quality, and extracted data. Ten trials met the inclusion criteria with a total of 191 participants. Seven trials evaluated single treatment sessions, one evaluated a two-week intervention, one evaluated a six-week intervention and one a three-month intervention. It is only possible to blind trials of airway clearance and overnight ventilatory support to the outcome assessors. In most of the trials we judged there was an unclear risk of bias with regards to blinding due to inadequate descriptions. The six-week trial was the only one judged to have a low risk of bias for all

  17. European Cystic Fibrosis Society Standards of Care: Quality Management in cystic fibrosis.

    PubMed

    Stern, Martin; Bertrand, Dominique Pougheon; Bignamini, Elisabetta; Corey, Mary; Dembski, Birgit; Goss, Christopher H; Pressler, Tanja; Rault, Gilles; Viviani, Laura; Elborn, J Stuart; Castellani, Carlo

    2014-05-01

    Since the earliest days of cystic fibrosis (CF) treatment, patient data have been recorded and reviewed in order to identify the factors that lead to more favourable outcomes. Large data repositories, such as the US Cystic Fibrosis Registry, which was established in the 1960s, enabled successful treatments and patient outcomes to be recognized and improvement programmes to be implemented in specialist CF centres. Over the past decades, the greater volumes of data becoming available through Centre databases and patient registries led to the possibility of making comparisons between different therapies, approaches to care and indeed data recording. The quality of care for individuals with CF has become a focus at several levels: patient, centre, regional, national and international. This paper reviews the quality management and improvement issues at each of these levels with particular reference to indicators of health, the role of CF Centres, regional networks, national health policy, and international data registration and comparisons. Copyright © 2014 European Cystic Fibrosis Society. All rights reserved.

  18. Evaluation of high-resolution melting (HRM) for mutation scanning of selected exons of the CFTR gene.

    PubMed

    Krenková, P; Norambuena, P; Stambergová, A; Macek, M

    2009-01-01

    Hereby we present evaluation of high-resolution melting for mutation scanning applied to the cystic fibrosis transmembrane conductance regulator gene. High resolution melting was used for mutation scanning of selected samples derived from cystic fibrosis patients with a known cystic fibrosis transmembrane conductance regulator genotype. We tested 19 different disease-causing cystic fibrosis transmembrane conductance regulator mutant genotypes located within six exons of the cystic fibrosis transmembrane conductance regulator gene (4, 7, 10, 11, 14b and 22). Normalized melting curves of tested samples were compared to sequenced-verified wildtype samples. Determined mutations are as follows: p.F508del, p.I507del, p.G551D, p.R347P, c.1717- 1G>A, c.621+1G>T, p.Y122X, p.I336K, p.R553X, c.2789+5G>A, c.574delA, c.1811+1G>C, p.L1335F, p.L1335P, p.L1324P and p.M470V and represent minimally 76.5 % of all cystic fibrosis alleles detected in the Czech cystic fibrosis population. All analysed samples with mutant genotypes were unambiguously distinguished from wild-type samples. High-resolution melting analysis enabled reliable detection of all single-nucleotide polymorphism classes and 1- or 3- base pair deletions. We examined the specificity, sensitivity and precision of this methodology. High-resolution melting analysis is an economical, sensitive and specific close-tube method and has a high utility for the detection of unknown mutations in cystic fibrosis DNA diagnostics.

  19. Annual maximum 5-day rainfall total and maximum number of consecutive dry days over Central America and the Caribbean in the late twenty-first century projected by an atmospheric general circulation model with three different horizontal resolutions

    NASA Astrophysics Data System (ADS)

    Nakaegawa, T.; Kitoh, A.; Murakami, H.; Kusunoki, S.

    2014-04-01

    We simulated changes in annual maximum 5-day rainfall (RX5D) and annual maximum number of consecutive dry days (CDD) in Central America, Mexico, and the Caribbean with three different horizontal resolution atmospheric global general circulation models (AGCMs) and quantified the uncertainty of the projections. The RX5Ds and CDDs were projected to increase in most areas in response to global warming. However, consistent changes were confined to small areas: for RX5D, both coastal zones of northern Mexico and the Yucatan Peninsula; for CDD, the Pacific coastal zone of Mexico, the Yucatan Peninsula, and Guatemala. All three AGCMs projected that RX5Ds and CDDs averaged over only the land area and over the entire area (land and ocean) would increase. The dependence of RX5D probability density functions on the horizontal resolutions was complex. Precipitation unrelated to tropical cyclones was primarily responsible for the projected increases in the frequency of RX5Ds greater than 300 mm.

  20. Other mucoactive agents for cystic fibrosis.

    PubMed

    Bye, Peter T P; Elkins, Mark R

    2007-03-01

    This review examines specific mucoactive agents from three classes: expectorants, which add water to the airway; ion-transport modifiers, which promote ion and water transport across the epithelium of the airway; and mucokinetics, which improve cough-mediated clearance by increasing airflow or reducing sputum adhesivity. The agents are isotonic and hypertonic saline, mannitol, denufosol and beta-agonists. Our understanding of these agents has recently improved through pre-clinical research, clinical trials and, in particular, extensive research into the nature of the liquid lining the surface of the airway, both in health and in cystic fibrosis (CF). For each agent, recent research is reviewed, highlighting the evidence for possible mechanisms of action and for clinical efficacy in CF, as well as the implications for the optimal clinical application of the agent.

  1. Control programme for cystic echinococcosis in Uruguay

    PubMed Central

    Irabedra, Pilar; Ferreira, Ciro; Sayes, Julio; Elola, Susana; Rodríguez, Miriam; Morel, Noelia; Segura, Sebastian; dos Santos, Estela; Guisantes, Jorge A

    2016-01-01

    Cystic echinococcosis is a highly endemic parasitic zoonosis that is present in the Southern Cone countries of America. For several decades, various prevention and control programmes have been implemented in different countries and regions, with varying results. In Uruguay, a new control programme was implemented in 2006 that employed new strategies for canine diagnosis and treatment, dog population control, diagnosis in humans, epidemiological surveillance, and health education, including community participation. The control programme in Uruguay addresses the control and surveillance of the disease from a holistic perspective based on Primary Health Care, which has strengthened the community’s participation in developing and coordinating activities in an interdisciplinary manner. Similarly, the control programme that is currently implemented is based on a risk-focused approach. The surveillance and control measures were focused on small villages and extremely poor urban areas. In this study, the strategies used and the results obtained from 2008-2013 are analysed and discussed. PMID:27223652

  2. Clostridium difficile in patients with cystic fibrosis.

    PubMed

    Welkon, C J; Long, S S; Thompson, C M; Gilligan, P H

    1985-08-01

    One hundred seven patients with cystic fibrosis (CF) and 54 other patients with risk factors for Clostridium difficile-associated disease were entered into a bacteriologic study to compare the rate of recovery of C difficile and cytotoxin in feces with occurrence of diarrhea and to investigate potentially protective or permissive relationships of fecal flora. Toxigenic C difficile was recovered from 22% of CF patients and 11% of patients with other diagnoses. Unlike the latter group, the majority (12/15) of CF patients who had cytotoxin recovered had formed stools and no history of diarrhea. Explanations for the lack of symptoms are speculative. Stool flora of CF patients was significantly more likely to include several bacteria with known inhibitory effects on C difficile. Recovery of C difficile and cytotoxin, however, was not associated with the decrease in rate of recovery or the mean bacterial count of any bacterium of fecal flora.

  3. High dose Nutrizym 22 in cystic fibrosis.

    PubMed

    Shah, A; Dinwiddie, R; Madge, S; Prescott, P; Hudson, G

    1993-09-01

    New high dose pancreatic enzyme preparations could be potentially helpful to cystic fibrosis (CF) patients. The purpose of this study was to compare the efficacy of the new high dose pancreatic enzyme preparation, Nutrizym 22 with the standard preparation Nutrizym GR. Twenty-five CF children (aged 7-16 years) entered the study and 22 completed it; 3 did not, due to non-compliance. All were taking Nutrizym GR for at least 2 weeks before entering the study. A randomised double blind, crossover method using standard Nutrizym GR or double strength Nutrizym 22 capsules was carried out over two consecutive 14-day periods. Crossover analyses of variance showed no statistically significant differences in actual weight gain, appetite, abdominal pain, stool consistency or faecal fat during the prestudy and study periods. It is concluded that half the capsule numbers of the high strength preparation are just as effective as the standard capsule dosage.

  4. Dosimetry for radiocolloid therapy of cystic craniopharyngiomas.

    PubMed

    Rojas, E Leticia; Al-Dweri, Feras M O; Lallena, Antonio M; Bodineau, Coral; Galán, Pedro

    2003-09-01

    The dosimetry for radiocolloid therapy of cystic craniopharyngiomas is investigated. Analytical calculations based on the Loevinger and the Berger formulas for electrons and photons, respectively, are compared with Monte Carlo simulations. The role of the material of which the colloid introduced inside the craniopharyngioma is made of as well as that forming the cyst wall is analyzed. It is found that the analytical approaches provide a very good description of the simulated data in the conditions where they can be applied (i.e., in the case of a uniform and infinite homogeneous medium). However, the consideration of the different materials and interfaces produces a strong reduction of the dose delivered to the cyst wall in relation to that predicted by the Loevinger and the Berger formulas.

  5. Control programme for cystic echinococcosis in Uruguay.

    PubMed

    Irabedra, Pilar; Ferreira, Ciro; Sayes, Julio; Elola, Susana; Rodríguez, Miriam; Morel, Noelia; Segura, Sebastian; Santos, Estela Dos; Guisantes, Jorge A

    2016-05-24

    Cystic echinococcosis is a highly endemic parasitic zoonosis that is present in the Southern Cone countries of America. For several decades, various prevention and control programmes have been implemented in different countries and regions, with varying results. In Uruguay, a new control programme was implemented in 2006 that employed new strategies for canine diagnosis and treatment, dog population control, diagnosis in humans, epidemiological surveillance, and health education, including community participation. The control programme in Uruguay addresses the control and surveillance of the disease from a holistic perspective based on Primary Health Care, which has strengthened the community's participation in developing and coordinating activities in an interdisciplinary manner. Similarly, the control programme that is currently implemented is based on a risk-focused approach. The surveillance and control measures were focused on small villages and extremely poor urban areas. In this study, the strategies used and the results obtained from 2008-2013 are analysed and discussed.

  6. Autogenic Drainage in Children With Cystic Fibrosis.

    PubMed

    Corten, Lieselotte; Morrow, Brenda M

    2017-04-01

    Airway clearance is an essential part of the management of cystic fibrosis (CF) as it facilitates clearance of viscous pulmonary secretions. This review aimed to determine the effect of autogenic drainage (AD) and assisted autogenic drainage (AAD) compared with no, sham, or other types of airway clearance in children with CF. Two pediatric randomized cross-over trials were identified on the use of AD in children with CF; no studies were available on the use of AAD. In one study AD had a positive influence on the Huang score, and is preferred over postural drainage in this population. We could not determine the efficacy of AD and AAD in children with CF. We recommend the implementation of pediatric-specific randomized controlled trials with adequate sample sizes, appropriate clinical outcome measures, and analysis of adverse effects.

  7. Infantile Cystic Hygroma: An Unusual Perioperative Course

    PubMed Central

    Saini, Suman; Dayal, Madhu; Gupta, Amita

    2017-01-01

    Airway management of an infant with a giant cervical lump may be a difficult task. The anesthesiologist must be prepared to face associated challenges during securing the airway in such patients. We report our experience with One year old infant who presented with huge cystic hygroma in the cervical region leading to recurrent episodes of respiratory tract infection and distress. Surgical removal was needed as sclerotherapy proved ineffective in reducing its size. Proseal laryngeal mask airway was used as a conduit after inhalational induction since airway could not be maintained with bag and mask. The child was tracheostomized postoperatively and also had a prolonged Intensive Care Unit stay. Difficulties encountered in intubation and postoperative management of this child are discussed in this report.

  8. Genetic therapies for cystic fibrosis lung disease

    PubMed Central

    Sinn, Patrick L.; Anthony, Reshma M.; McCray, Paul B.

    2011-01-01

    The aim of gene therapy for cystic fibrosis (CF) lung disease is to efficiently and safely express the CF transmembrane conductance regulator (CFTR) in the appropriate pulmonary cell types. Although CF patients experience multi-organ disease, the chronic bacterial lung infections and associated inflammation are the primary cause of shortened life expectancy. Gene transfer-based therapeutic approaches are feasible, in part, because the airway epithelium is directly accessible by aerosol delivery or instillation. Improvements in standard delivery vectors and the development of novel vectors, as well as emerging technologies and new animal models, are propelling exciting new research forward. Here, we review recent developments that are advancing this field of investigation. PMID:21422098

  9. Scoliosis in cystic fibrosis - an appraisal

    SciTech Connect

    Paling, M.R.; Spasovsky-Chernick, M.

    1982-03-01

    An unusually high prevalence (10%) of scoliosis is described in a series of 151 patients aged four years and older with cystic fibrosis. The scolioses were of the late onset (juvenile and adolescent) type, being typically thoracic with the curve convex to the right, although there was no significant preference for either sex. No direct relationship was found between the spinal curvature and the severity or distribution of the lung disease, although the worse scolioses tended to occur in patients with relatively severe pulmonary involvement. There was no evidence of metabolic bone disease as a predisposing cause. Some indication of a familial tendency towards scoliosis was apparent, and a genetic or constitutional basis is postulated with an unknown precipitating factor.

  10. Nutrition and growth in cystic fibrosis.

    PubMed

    Hankard, Régis; Munck, Anne; Navarro, Jean

    2002-01-01

    Malnutrition is a common complication of chronic diseases in children and may lead to growth impairment (stunting). Malnutrition in cystic fibrosis (CF) results from increased energy expenditure, decreased energy intakes, malabsorption of ingested nutrients because of pancreatic insufficiency and chronic inflammation. Malnutrition and high levels of inflammatory cytokines affect IGF-1 production through interrelated mechanisms. Nutritional support was shown to improve both nutritional status and outcome in CF. However, some nutrients have a direct effect on the disease. n-3 fatty acids supplementation is able to correct lipid abnormalities resulting from a primary mechanism. Moreover, n-3 fatty acids have a direct effect on the inflammatory response, decreasing eicosanoid synthesis and modulating nuclear transcriptional factors nuclear factor kappaB and peroxisome proliferator-activated receptors gamma. Nutritional support may be considered part of the care of the CF patient together with antibiotics, pancreatic enzymes and physiotherapy, influencing significantly the evolution of the disease.

  11. Voxel-based dose calculation in radiocolloid therapy of cystic craniopharyngiomas

    NASA Astrophysics Data System (ADS)

    Treuer, H.; Hoevels, M.; Luyken, K.; Gierich, A.; Hellerbach, A.; Lachtermann, B.; Visser-Vandewalle, V.; Ruge, M.; Wirths, J.

    2015-02-01

    Very high doses are administered in radiocolloid therapy of cystic craniopharyngiomas. However individual dose planning is not common yet mainly due to insufficient image resolution. Our aim was to investigate whether currently available high-resolution image data can be used for voxel-based dose calculation for short-ranged β-emitters (32P,90Y,186Re) and to assess the achievable accuracy. We developed a convolution algorithm based on voxelized dose activity distributions and dose-spread kernels. Results for targets with 5-40 mm diameter were compared with high-resolution Monte Carlo calculations in spherical phantoms. Voxel size was 0.35 mm. Homogeneous volume and surface activity distributions were used. Dose-volume histograms of targets and shell structures were compared and γ index (dose tolerance 5%, distance to agreement 0.35 mm) was calculated for dose profiles along the principal axes. For volumetric activity distributions 89.3% ± 11.9% of all points passed the γ test (mean γ 0.53  ±  0.16). For surface distributions 33.6% ± 14.8% of all points passed the γ test (mean γ 2.01  ±  0.60). The shift of curves in dose-volume histograms was -1.7 Gy ± 7.6 Gy (-4.4 Gy ± 24.1 Gy for 186Re) in volumetric distributions and 46.3% ± 32.8% in surface distributions. The results show that individual dose planning for radiocolloid therapy of cystic craniopharyngiomas based on high-resolution voxelized image data is feasible and yields highly accurate results for volumetric activity distributions and reasonable dose estimates for surface distributions.

  12. Global impact of bronchiectasis and cystic fibrosis

    PubMed Central

    Redondo, Margarida; Keyt, Holly; Dhar, Raja

    2016-01-01

    Educational aims To recognise the clinical and radiological presentation of the spectrum of diseases associated with bronchiectasis. To understand variation in the aetiology, microbiology and burden of bronchiectasis and cystic fibrosis across different global healthcare systems. Bronchiectasis is the term used to refer to dilatation of the bronchi that is usually permanent and is associated with a clinical syndrome of cough, sputum production and recurrent respiratory infections. It can be caused by a range of inherited and acquired disorders, or may be idiopathic in nature. The most well recognised inherited disorder in Western countries is cystic fibrosis (CF), an autosomal recessive condition that leads to progressive bronchiectasis, bacterial infection and premature mortality. Both bronchiectasis due to CF and bronchiectasis due to other conditions are placing an increasing burden on healthcare systems internationally. Treatments for CF are becoming more effective leading to more adult patients with complex healthcare needs. Bronchiectasis not due to CF is becoming increasingly recognised, particularly in the elderly population. Recognition is important and can lead to identification of the underlying cause, appropriate treatment and improved quality of life. The disease is highly diverse in its presentation, requiring all respiratory physicians to have knowledge of the different “bronchiectasis syndromes”. The most common aetiologies and presenting syndromes vary depending on geography, with nontuberculous mycobacterial disease predominating in some parts of North America, post-infectious and idiopathic disease predominating in Western Europe, and post-tuberculosis bronchiectasis dominating in South Asia and Eastern Europe. Ongoing global collaborative studies will greatly advance our understanding of the international impact of bronchiectasis and CF. PMID:28210295

  13. Prevalence of Intracranial Atherosclerotic Stenosis Using High-Resolution Magnetic Resonance Angiography in the General Population: The Atherosclerosis Risk in Communities Study.

    PubMed

    Suri, Muhammad Fareed K; Qiao, Ye; Ma, Xiaoye; Guallar, Eliseo; Zhou, Jincheng; Zhang, Yiyi; Liu, Li; Chu, Haitao; Qureshi, Adnan I; Alonso, Alvaro; Folsom, Aaron R; Wasserman, Bruce A

    2016-05-01

    Intracranial atherosclerotic stenosis (ICAS) is a common cause of stroke, but little is known about its epidemiology. We studied the prevalence of ICAS and its association with vascular risk factors using high-resolution magnetic resonance angiography in a US cardiovascular cohort. The Atherosclerosis Risk in Communities (ARIC) study recruited participants from 4 US communities from 1987 to 1989. Using stratified sampling, we selected 1980 participants from visit 5 (2011-2013) for high-resolution 3T-magnetic resonance angiography. All images were analyzed in a centralized laboratory, and ICAS was graded as: no stenosis, <50% stenosis, 50% to 69% stenosis, 70% to 99% stenosis, and complete occlusion. We calculated per-vessel and per-person prevalence of ICAS (weighted for n=6538 visit 5 participants) and also estimated the US prevalence. We used multivariable logistic regression to identify variables independently associated with ICAS. Subjects who had an adequate magnetic resonance angiography (n=1765) were aged 67 to 90 years, 41% were men, 70% were white, and 29% were black. ICAS was prevalent in 31% of participants and 9% had ICAS ≥50%. Estimated US prevalence of ICAS ≥50% for 65 to 90 years old was 8% for whites and 12% for blacks. Older age, black race, higher systolic blood pressure, and higher low-density lipoprotein cholesterol levels were associated with increased odds of ICAS, whereas higher levels of high-density lipoprotein cholesterol and use of cholesterol-lowering medications were associated with decreased odds of ICAS. Body mass index and smoking were not associated with ICAS. The prevalence of ICAS in older adults is high, and it could be a target for primary prevention of stroke and dementia in this population. © 2016 American Heart Association, Inc.

  14. Cystic fibroadenoma of the breast: a case report.

    PubMed

    Durak, Merih Güray; Karaman, Ilgın; Canda, Tülay; Balci, Pınar; Harmancioğlu, Omer

    2011-01-01

    Fibroadenoma is the most common breast tumor in adolescent and young women. Fibroadenomas that consist of sclerosing adenosis, papillary apocrine metaplasia, epithelial calcifications, and/or cysts greater than 3 mm are considered as complex fibroadenoma. The relative risk of developing breast cancer in patients with complex fibroadenoma is increased, compared to women with noncomplex fibroadenoma. Extensive cystic degeneration in a fibroadenoma, so called "cystic fibroadenoma" is very rare. Herein, we present a case of such a lesion in a 43-year-old female who has been on follow-up for fibrocystic changes of the breast, and discuss both radiological and histopathologic differential diagnosis of this lesion with other cystic lesions of the breast, including cystic papilloma. The patient is free of disease after 17 months of clinical follow-up.

  15. Sinonasal adenoid cystic carcinoma presenting as an orbital mass.

    PubMed

    Kwon, Robert O; Lyon, David B; Floyd, Michael; Girod, Douglas A

    2010-01-01

    A 61-year-old man with a history of sinus polyposis and prior sinus surgery presented with left-sided retrobulbar pain and headache. He was found to have left-sided proptosis, and imaging studies showed a large left medial orbital soft-tissue mass. Incisional biopsy revealed adenoid cystic carcinoma. Further evaluation revealed extensive sinonasal adenoid cystic carcinoma. The patient underwent en bloc maxillectomy with orbital exenteration and partial rhinectomy, followed by radiation therapy. The medial orbit is an unusual location for adenoid cystic carcinoma, which the authors believe was a secondary manifestation due to perineural spread from the sinuses via the ethmoidal nerves. Adenoid cystic carcinoma should be included in the differential diagnosis of tumors of the medial orbit, especially if there is a history of sinonasal disease.

  16. Posterior left atrial wall hematoma mimicking cystic intracavitary atrial mass.

    PubMed

    Bahnacy, Yasser; Suresh, Cheriyil; Dawoud, Hamed; Zubaid, Mohammad

    2010-10-01

    Atrial myxoma is the most common benign primary tumor of the heart most commonly in the left atrium (LA). Cystic or cavitated intracardiac masses are rare. We report the case of a 43-year-old male patient admitted with chest infection, hemoptysis, and severe respiratory distress, who had to be ventilated. Chest computed tomography showed bilateral lung consolidation with large mass occupying the region of the LA. Transthoracic echocardiography and transesophageal echocardiography showed a large intracavitary left atrial cystic mobile mass. Open-heart surgical exploration did not show any mass inside the LA. A posterior left atrial wall hematoma was found and evacuated. Biopsies confirmed the presence of blood clots. Posterior left atrial wall hematoma may appear as left atrial intracavitary cystic mass and should be included in the differential diagnosis of cystic left atrial mass.

  17. Screening for cystic fibrosis: what every NP should know.

    PubMed

    Nicholson, Krysta N

    2013-09-10

    In a chronic childhood disease such as cystic fibrosis, it is important for nurse practitioners to be knowledgeable about the disease process, methods of mutation identification, and diagnostic criteria. Multiple studies have shown improved prognosis for patients diagnosed early.

  18. A pulmonary mucinous cystic tumour of borderline malignancy.

    PubMed

    Bacha, D; Ayadi-Kaddour, A; Smati, B; Kilani, T; El Mezni, F

    2008-06-01

    We report a well-documented case of pulmonary mucinous cystic tumour of borderline malignancy involving the left lower lobe. The lesion was found incidentally by chest radiograph and CT scan with a provisional diagnosis of bronchioloalveolar carcinoma. The tumour was 4 cm in its greatest dimension, cystic and filled with gelatinous mucus. Microscopically, the neoplastic mucinous epithelium was composed of cuboidal cells with focally nuclear stratification and mild to moderate nuclear atypia. The patient has remained free from recurrence or metastases for 6 years. Pulmonary mucinous cystic tumour of borderline malignancy is a rare, recently described neoplasm, which spans a spectrum of tumours with malignant potential. The recent World Health Organization classification of lung tumours does not recognize this entity, which has a very good prognosis, and as such should be distinguished from classic pulmonary adenocarcinoma. Histological diagnosis can be difficult to distinguish from cystic bronchioloalveolar carcinoma or metastatic mucinous adenocarcinoma.

  19. Giant cystic sacral schwannoma mimicking tarlov cyst: a case report.

    PubMed

    Attiah, Mark A; Syre, Peter P; Pierce, John; Belyaeva, Elizaveta; Welch, William C

    2016-05-01

    To present a rare case of a giant schwannoma of the sacrum mimicking a Tarlov cyst. A 58-year-old woman had a 1-year history of low back pain. MRI revealed a large cystic mass in the sacral canal with bony erosion. Radiological diagnosis of Tarlov cyst was made. The patient underwent surgical treatment for the lesion, which revealed a solid mass. Histopathological examination of the tumor confirmed the diagnosis of schwannoma. The postoperative course was uneventful and the patient has had significant improvement in her pain 1 month postoperatively. Giant cystic schwannoma of the sacrum is a very rare diagnosis overlooked by practitioners for more common cystic etiologies, but its treatment is significantly different. Care should be taken to include this diagnosis in a differential for a cystic sacral mass.

  20. Clinical Scenarios in Chronic Kidney Disease: Cystic Renal Diseases.

    PubMed

    Meola, Mario; Samoni, Sara; Petrucci, Ilaria

    2016-01-01

    Cysts are frequently found in chronic kidney disease (CKD) and they have a different prognostic significance depending on the clinical context. Simple solitary parenchymal cysts and peripelvic cysts are very common and they have no clinical significance. At US, simple cyst appears as a round anechoic pouch with regular and thin profiles. On the other hand, hereditary polycystic disease is a frequent cause of CKD in children and adults. Autosomal dominant polycystic kidney disease (ADPKD) and autosomal recessive polycystic kidney disease (ARPKD) are the best known cystic hereditary diseases. ADPKD and ARPKD show a diffused cystic degeneration with cysts of different diameters derived from tubular epithelium. Medullary cystic disease may be associated with tubular defects, acidosis and lithiasis and can lead to CKD. Acquired cystic kidney disease, finally, is secondary to progressive structural end-stage kidney remodelling and may be associated with renal cell carcinoma. © 2016 S. Karger AG, Basel.

  1. Cystic Pancreatic Lymphangioma - Diagnostic Role of Endoscopic Ultrasound.

    PubMed

    Carvalho, Diana; Costa, Mariana; Russo, Pedro; Simas, Luís; Baptista, Teresa; Ramos, Gonçalo

    2016-01-01

    Pancreatic cystic lymphangiomas are rare benign lesions that arise from lymphatic vessels, accounting for less than 0.2% of all pancreatic cysts. Typically it is asymptomatic and discovery occurs during imaging exams for non-pancreatic disease. In the past, a definite diagnosis was made through surgery, with complete resection of all tumoral tissue to prevent recurrence. Nowadays, the development of endoscopic ultrasound (EUS) made it possible to identify these cysts combining morphologic ultrasound features, macroscopic aspirated fluid appearance, biochemical and cytological evaluation of the sample. We report two cases of cystic pancreatic lymphangioma diagnosed through EUS, allowing conservative management without surgery. These cases show that cystic pancreatic lymphangioma should be considered in the differential diagnosis of cystic pancreatic lesions and that EUS is an important tool for their recognition.

  2. Complementary and alternative medicine use in children with cystic fibrosis.

    PubMed

    Giangioppo, Sandra; Kalaci, Odion; Radhakrishnan, Arun; Fleischer, Erin; Itterman, Jennifer; Lyttle, Brian; Price, April; Radhakrishnan, Dhenuka

    2016-11-01

    To estimate the overall prevalence of complementary and alternative medicine use among children with cystic fibrosis, determine specific modalities used, predictors of use and subjective helpfulness or harm from individual modalities. Of 53 children attending the cystic fibrosis clinic in London, Ontario (100% recruitment), 79% had used complementary and alternative medicine. The most commonly used modalities were air purifiers, humidifiers, probiotics, and omega-3 fatty acids. Family complementary and alternative medicine use was the only independent predictor of overall use. The majority of patients perceived benefit from specific modalities for cystic fibrosis symptoms. Given the high frequency and number of modalities used and lack of patient and disease characteristics predicting use, we recommend that health care providers should routinely ask about complementary and alternative medicine among all pediatric cystic fibrosis patients and assist patients in understanding the potential benefits and risks to make informed decisions about its use. Copyright © 2016 Elsevier Ltd. All rights reserved.

  3. Modular microfluidic system as a model of cystic fibrosis airways

    PubMed Central

    Skolimowski, M.; Weiss Nielsen, M.; Abeille, F.; Skafte-Pedersen, P.; Sabourin, D.; Fercher, A.; Papkovsky, D.; Molin, S.; Taboryski, R.; Sternberg, C.; Dufva, M.; Geschke, O.; Emnéus, J.

    2012-01-01

    A modular microfluidic airways model system that can simulate the changes in oxygen tension in different compartments of the cystic fibrosis (CF) airways was designed, developed, and tested. The fully reconfigurable system composed of modules with different functionalities: multichannel peristaltic pumps, bubble traps, gas exchange chip, and cell culture chambers. We have successfully applied this system for studying the antibiotic therapy of Pseudomonas aeruginosa, the bacteria mainly responsible for morbidity and mortality in cystic fibrosis, in different oxygen environments. Furthermore, we have mimicked the bacterial reinoculation of the aerobic compartments (lower respiratory tract) from the anaerobic compartments (cystic fibrosis sinuses) following an antibiotic treatment. This effect is hypothesised as the one on the main reasons for recurrent lung infections in cystic fibrosis patients. PMID:23908680

  4. Slower rise of exhaled breath temperature in cystic fibrosis.

    PubMed

    Bade, Geetanjali; Gupta, Sumita; Kabra, Sushil Kumar; Talwar, Anjana

    2015-02-01

    To measure exhaled breath temperature in patients with cystic fibrosis. 17 patients (6-18 years) with cystic fibrosis and 15 age- and gender-matched healthy controls were recruited in this cross sectional study. Exhaled breath temperature was measured in subjects recruited in both the groups with a device X-halo and analyzed as plateau temperature achieved and rate of temperature rise. Patients with cystic fibrosis showed no significant difference in plateau temperature [34.4(32.3-34.6) versus 33.9 (33.0-34.4)oC; P=0.35] while mean (SEM.) rate of temperature rise was significantly less in patients [0.09 (0.01) versus 0.14 (0.02) ƼC/s ; P=0.04] as compared to controls. There was a slower rise of exhaled breath temperature in patients with cystic fibrosis whereas plateau temperature was not significantly different from controls.

  5. Cystic hygroma in a tactical aviator: a case report.

    PubMed

    Oakes, Michael J; Sherman, Bret E

    2004-12-01

    Cystic hygroma of the neck is a benign, congenital tumor of lymphatic origin rarely seen in adults, with approximately 100 cases presented in the literature. The etiology of this lesion is poorly understood, with spontaneous occurrence, upper respiratory infection, or trivial trauma being suggested. We present a recent unique case report of an adult male tactical aviator who experienced a progressively enlarging cystic hygroma of the neck over a 2-week period without history of infection or obvious trauma. This case is unusual in that there may have been an element of continuous microtrauma to the area given the individual's occupation and required flight head gear. A successful surgical dissection and removal of the cystic hygroma was performed. A discussion and brief literature review of cystic hygroma in adulthood is presented.

  6. Cystic fibrosis in adults. From researcher to practitioner.

    PubMed Central

    Marelich, G P; Cross, C E

    1996-01-01

    The Cystic Fibrosis Foundation currently tracks about 20,000 Americans with cystic fibrosis, an autosomal recessive genetic disease that leads to multisystem complications. With the institution of better therapeutic regimens over the past 2 decades, more patients with this disease are surviving to adulthood. Within the past decade, both clinical and basic science research in the field of cystic fibrosis has progressed at a rapid rate. The intent of this review is to introduce readers to the molecular, cellular, and systemic disorders of this disease. We discuss treatment strategies involving antibiotics, nutrition, immune-response mediators, chest physiotherapy, and sputum-active agents with respect to the airway dysfunction of cystic fibrosis. Other common complications, recent developments, transplantation, and gene therapy are also reviewed. PMID:8732732

  7. Benign Cystic Peritoneal Mesothelioma Revealed by Small Bowel Obstruction

    PubMed Central

    Bray Madoué, Kaimba; Boniface, Moifo; Annick Laure, Edzimbi; Pierre, Herve

    2016-01-01

    Benign cystic peritoneal mesothelioma is a rare tumor which frequently occurs in women of reproductive age. Abdominal pain associated with pelvic or abdominal mass is the common clinical presentation. We report the case of a 22-year-old woman with a pathological proved benign cystic mesothelioma of the peritoneum revealed by a small bowel obstruction and a painful left-sided pelvic mass with signs of psoitis. Contrast enhanced abdominal CT-scan demonstrated a large pelvic cystic mass with mass effect on rectosigmoid and pelvic organs. The patient underwent surgical removal of the tumor. Pathological examination revealed the diagnosis of benign cystic mesothelioma of the peritoneum. The outcome was excellent with a 12-month recoil. PMID:27066288

  8. Florid Cystic Endosalpingiosis (Müllerianosis) in Pregnancy

    PubMed Central

    Montero-Balaguer, Beatriz; Desantes-Real, Domingo; Perales-Marín, Alfredo

    2016-01-01

    Cystic endosalpingiosis refers to the existence of heterotopic cystic müllerian tissue resembling structures of the fallopian tubes. We report a case of florid cystic endosalpingiosis discovered in a pregnant woman during a scheduled cesarean section and review the current knowledge of this disease. A 30-year-old woman with a twin pregnancy attended the hospital day unit at term. The first twin was in a breech presentation and a cesarean section was scheduled. During the procedure the uterine fundus and part of the body were seen completely seeded with multitude of cyst-like structures resembling hydatids of Morgagni. The immunohistochemistry analysis showed a positive expression for PAX8 (Box-8), CK7, and estrogen and progesterone receptors. The lesions did not disappear after pregnancy. Cystic endosalpingiosis should be always borne in mind, even in pregnancy, when it comes to making the differential diagnosis of a pelvic or systemic multicystic mass. PMID:27668111

  9. Calcified cystic lymphangioma of the mesentery: case report.

    PubMed

    Buccoliero, Anna Maria; Castiglione, Francesca; Maio, Vincenza; Morelli, Caterina; Martin, Alessandra; Messineo, Antonio; Taddei, Gian Luigi

    2009-01-01

    Mesenteric cysts are rare and are associated with heterogeneous pathological conditions. We describe an extraordinary case of mesenteric calcified cystic lymphangioma in a 10-year-old boy. To the best of our knowledge only 6 mesenteric calcified cystic lymphangioma have been reported. The patient was admitted with abdominal pain and vomiting. Abdominal X-ray and computed tomography scan documented a calcified cyst which dislocated and compressed the ileum. Laparatomy revealed that the cyst arose in the mesentery at 100 cm from the ileocecal valve. The cystic wall was composed of fibrosclerotic calcified tissue and had an endothelial lining. In the adjacent fibrofatty stroma there were distended hemolymphatics. Postoperative recovery was uneventful. At follow-up 6 months after surgery the patient is well. Calcified cystic lymphangioma of the mesentery should be considered in the differential diagnosis of an intra-abdominal calcified cyst in children.

  10. Cystic fibrosis-related diabetes: a distinct condition.

    PubMed

    Cano Megías, Marta; González Albarrán, Olga

    2015-01-01

    Cystic fibrosis is the most common fatal inherited autosomal recessive disease in Caucasians, affecting approximately one out of every 2,000 births. Survival of patients with cystic fibrosis has significantly improved due to advances in respiratory and nutritional care, and their current average life expectancy is 30-40 years. Development of cystic fibrosis-related diabetes is a comorbidity that increases with age and may reach a prevalence up to 50% in adults. Its development is associated to impaired lung function and nutritional status, and early diagnosis and treatment are therefore essential to improve quality of life and performance status. Insulin therapy for diabetes and other early carbohydrate metabolism disorders may improve lung function and nutritional status of patients with cystic fibrosis.

  11. Benign Cystic Mesothelioma: A Rare Cause for Scrotal Swelling

    PubMed Central

    Aber, A.; Tahir, A.; Arumuham, V.; Smith, G.; Almpanis, S.

    2012-01-01

    Benign cystic mesothelioma of the tunica vaginalis is a rare occurrence. It usually presents with painless gradual swelling in the scrotum. These types of benign mesotheliomas typically occur in the peritoneum and usually affect young to middle-aged patients. We present in this case an unusual case of benign cystic mesothelioma of the tunica vaginalis in a 77-year-old male patient. PMID:22675366

  12. [MALIGNANT TUMORS IN OVARIAN MATURE CYSTIC TERATOMAS INTRAOPERATIVE DIAGNOSTIC BASIS].

    PubMed

    Khachatryan, A

    2016-11-01

    Extremely rare ovarian primary tumors formed in a mature cystic teratomaare described in the literature. This research work studies the frequency of malignant mature cystic teratoma, as well as their clinical and morphological features and necessity of intraoperative histological examination of all teratomas. Cases histories of 56 patients, suffering from ovarian mature cystic teratomahave been studied in MC Shengavit in the period of 2003 - 2015. Among them 4 patients with the somatic malignancies were identified. Morphological methods, which are considered to be "gold standard" of tumor investigation, were used in staining the slides with hematoxylin - eosin. According to the literature the secondary malignant transformation rarely occurs and is typical in postmenopausal women, with a frequency of 0.17-3%. According to the results of our study, malignant tumors in mature cystic teratomas were observed in 4 (7,14%) from the total number of mature cystic teratomas (n=56). There was not revealed a correlation between the duration of the complaints, age of the patients, sizes of ovarian mature teratoma and malignization degree. Thus, the greatest difficulties of clinical diagnosis of malignant tumors in the ovarian mature cystic teratomas were in the early stage of the disease, because of a variety of clinical manifestations, not pathognomonic for malignization. All mentioned symptoms may be observed in the patients with usual mature cystic teratomas. Тhis cases confirm the necessity to take tissue samples from the other ovary for intraoperative histopathological evaluation in each case of mature cystic teratomas. It is necessary to examine a large number of tumor sites, to prevent errors in the assessment of the maturity degree of teratoma.

  13. Cystic Meningioma Simulating Arachnoid Cyst: Report of an Unusual Case

    PubMed Central

    Jorge, Docampo; Nadia, Gonzalez; Claudio, Vazquez; Carlos, Morales; Eduardo, Gonzalez-Toledo

    2014-01-01

    The purpose of this paper is to show an unusual case of meningioma simulating arachnoid cyst on CT scan and MRI, diagnosed in a 63-year-old woman evaluated for headache and vision disorders. The meningioma shown is predominantly cystic with a small mural nodule enhancing after gadolinium and exhibiting diffusion restriction. Cystic portion of the tumor is hypodense on CT, and evidences fluid signal intensity on T1- and T2-weighted MR imaging. PMID:25057425

  14. Cystic meningioma simulating arachnoid cyst: report of an unusual case.

    PubMed

    Docampo, Jorge; Jorge, Docampo; Gonzalez, Nadia; Nadia, Gonzalez; Vazquez, Claudio; Claudio, Vazquez; Morales, Carlos; Carlos, Morales; Gonzalez-Toledo, Eduardo; Eduardo, Gonzalez-Toledo

    2014-01-01

    The purpose of this paper is to show an unusual case of meningioma simulating arachnoid cyst on CT scan and MRI, diagnosed in a 63-year-old woman evaluated for headache and vision disorders. The meningioma shown is predominantly cystic with a small mural nodule enhancing after gadolinium and exhibiting diffusion restriction. Cystic portion of the tumor is hypodense on CT, and evidences fluid signal intensity on T1- and T2-weighted MR imaging.

  15. Gamma Knife Radiosurgery for the Treatment of Cystic Cerebral Metastases

    SciTech Connect

    Ebinu, Julius O.; Lwu, Shelly; Monsalves, Eric; Arayee, Mandana; Chung, Caroline; Laperriere, Normand J.; Kulkarni, Abhaya V.; Goetz, Pablo; Zadeh, Gelareh

    2013-03-01

    Purpose: To assess the role of Gamma Knife radiosurgery (GKRS) in the treatment of nonsurgical cystic brain metastasis, and to determine predictors of response to GKRS. Methods: We reviewed a prospectively maintained database of brain metastases patients treated at our institution between 2006 and 2010. All lesions with a cystic component were identified, and volumetric analysis was done to measure percentage of cystic volume on day of treatment and consecutive follow-up MRI scans. Clinical, radiologic, and dosimetry parameters were reviewed to establish the overall response of cystic metastases to GKRS as well as identify potential predictive factors of response. Results: A total of 111 lesions in 73 patients were analyzed; 57% of lesions received prior whole-brain radiation therapy (WBRT). Lung carcinoma was the primary cancer in 51% of patients, 10% breast, 10% colorectal, 4% melanoma, and 26% other. Fifty-seven percent of the patients were recursive partitioning analysis class 1, the remainder class 2. Mean target volume was 3.3 mL (range, 0.1-23 mL). Median prescription dose was 21 Gy (range, 15-24 Gy). Local control rates were 91%, 63%, and 37% at 6, 12, and 18 months, respectively. Local control was improved in lung primary and worse in patients with prior WBRT (univariate). Only lung primary predicted local control in multivariate analysis, whereas age and tumor volume did not. Lesions with a large cystic component did not show a poorer response compared with those with a small cystic component. Conclusions: This study supports the use of GKRS in the management of nonsurgical cystic metastases, despite a traditionally perceived poorer response. Our local control rates are comparable to a matched cohort of noncystic brain metastases, and therefore the presence of a large cystic component should not deter the use of GKRS. Predictors of response included tumor subtype. Prior WBRT decreased effectiveness of SRS for local control rates.

  16. Reduced upper airway nitric oxide in cystic fibrosis.

    PubMed Central

    Balfour-Lynn, I M; Laverty, A; Dinwiddie, R

    1996-01-01

    Nitric oxide (NO) produced within the respiratory tract is detectable in exhaled and nasal air. Its synthesis may be induced by inflammatory cytokines and reduced by glucocorticoids. Increased concentrations have been found in asthma and bronchiectasis. In this study, NO concentrations were determined in 63 children with cystic fibrosis, of whom 13 were on inhaled steroids (mean age 13.3 years) and 50 were not (mean age 12.3 years); 57 normal children (mean age 12.2 years) were also studied. NO was measured by chemiluminescence analyser, exhaled NO following a relaxed vital capacity manoeuvre, and nasal NO with the breath held following a full inspiration. Mean concentration of exhaled NO in cystic fibrosis patients (no steroids) was 4.7 parts per billion (ppb) (95% confidence interval (CI) 4.0 to 5.3); this did not differ from values in normal children (mean 4.8 ppb, 95% CI 3.8 to 5.8) or in cystic fibrosis patients on inhaled steroids (mean 3.6 ppb, 95% CI 2.5 to 4.8). Nasal concentrations were significantly lower in cystic fibrosis patients, with or without inhaled steroids, than in normal children (cystic fibrosis, no inhaled steroids: 460 ppb, 95% CI 399 to 520; cystic fibrosis, inhaled steroids: 522 ppb, 95% CI 313 to 730, v normal children: 1024 ppb, 95% CI 896 to 1152, p < 0.0001). Considering the inflammatory nature of cystic fibrosis, it is surprising exhaled NO levels were not increased, but this may have been due to alteration in NO diffusion through thick mucus. The low nasal NO concentrations, which are probably the result of impaired flow from the paranasal sinuses, may contribute to the recurrent respiratory infections typical of cystic fibrosis. PMID:8984918

  17. Cystic choroid plexus papilloma in the cavum septum pellucidum.

    PubMed

    Tuchman, Alexander; Kalhorn, Stephen P; Mikolaenko, Irina; Wisoff, Jeffrey H

    2009-12-01

    A choroid plexus papilloma is a rare CNS neoplasm arising from the neuroepithelial lining of the choroid plexus. A third ventricular location of a choroid plexus papilloma is rare compared with the more common sites in the lateral and fourth ventricles. Cystic choroid plexus papilloma represents an infrequent subtype that may present diagnostic ambiguity. The authors present a case of cystic choroid plexus papilloma within a cavum septum pellucidum that radiographically mimicked neurocysticercosis.

  18. Cystic pancreatic neuroendocrine tumors: To date a diagnostic challenge.

    PubMed

    Caglià, Pietro; Cannizzaro, Maria Teresa; Tracia, Angelo; Amodeo, Luca; Tracia, Luciano; Buffone, Antonino; Amodeo, Corrado; Cannizzaro, Matteo Angelo

    2015-09-01

    Cystic PNETs are an uncommon neoplasms increasingly detected in current clinical practice which often present a diagnostic challenges to both the experienced radiologist and pathologist. The aim of this study was to review the available literature to summarize current data that compare and evaluate both the clinical and pathologic features of cystic pancreatic neuroendocrine tumors. A systematic review of the current literature was performed using the search engines EMBASE and PubMed to identify all studies reporting on cystic pancreatic neuroendocrine tumors. The MeSH search terms used were "cystic pancreatic neuroendocrine tumors", "endocrine neoplasms", and "pancreatic cysts". Multiple combinations of the keywords and MeSH terms were used. The clinical evaluation of cystic pancreatic lesions appears to suffer from same limitations despite the improvement in the diagnostic tools. Subsequently, we highlight diagnostic pitfalls and differential diagnosis of these cystic tumors. In this review we discuss current advances in the application of the imaging modalities and characteristics features with special emphasize on endoscopic ultrasound (EUS), and EUS guide fine needle aspiration (EUS-FNA). Cystic neuroendocrine tumor in the pancreas underlines the clinical impact of endoscopic ultrasound in the work-up of patients with unclear lesions in the pancreas. EUS-FNA cytology and cyst fluid analysis is a useful adjunct to abdominal imaging for the diagnosis of pancreatic cystic lesions. Due to the evident diagnostic difficulties, we hypothesize that cyst fluid characteristics, including cytomorphological features, is the most accurate test to achieve a preoperative diagnosis and to provide a basis for prognostic prediction. Copyright © 2015 IJS Publishing Group Limited. Published by Elsevier Ltd. All rights reserved.

  19. Pulmonar collision tumor: metastatic adenoid cystic carcinoma and lung adenocarcinoma.

    PubMed

    Blanco, M; García-Fontán, E; Ríos, J; Rivo, J E; Fernández-Martín, R; Cañizares, M A

    2012-01-01

    We report an extraordinary case of collision tumor consisting of a lung adenocarcinoma and a metastatic adenoid cystic carcinoma in a 56 year-old man. He was diagnosed with a pulmonary nodule 11 years after treatment of an adenoid cystic carcinoma of the right maxillary sinus. A non-small cell carcinoma was observed when a transbronchial biopsy was performed. The other component of the nodule was only diagnosed with pathological examination of the resection specimen.

  20. Early airway infection, inflammation, and lung function in cystic fibrosis

    PubMed Central

    Nixon, G; Armstrong, D; Carzino, R; Carlin, J; Olinsky, A; Robertson, C; Grimwood, K

    2002-01-01

    Aims: To determine the relation between lower airway infection and inflammation, respiratory symptoms, and lung function in infants and young children with cystic fibrosis (CF). Methods: A prospective study of children with CF aged younger than 3 years, diagnosed by a newborn screening programme. All were clinically stable and had testing as outpatients. Subjects underwent bronchial lavage (BL) and lung function testing by the raised volume rapid thoracoabdominal compression technique under general anaesthesia. BL fluid was cultured and analysed for neutrophil count, interleukin 8, and neutrophil elastase. Lung function was assessed by forced expiratory volume in 0.5, 0.75, and 1 second. Results: Thirty six children with CF were tested on 54 occasions. Lower airway infection shown by BL was associated with a 10% reduction in FEV0.5 compared with subjects without infection. No relation was identified between airway inflammation and lung function. Daily moist cough within the week before testing was reported on 20/54 occasions, but in only seven (35%) was infection detected. Independent of either infection status or airway inflammation, those with daily cough had lower lung function than those without respiratory symptoms at the time of BL (mean adjusted FEV0.5 195 ml and 236 ml respectively). Conclusions: In young children with CF, both respiratory symptoms and airway infection have independent, additive effects on lung function, unrelated to airway inflammation. Further studies are needed to understand the mechanisms of airway obstruction in these young patients. PMID:12244003

  1. Ovarian Mature Cystic Teratoma: Challenges of Surgical Management

    PubMed Central

    Sinha, Abha; Ewies, Ayman A. A.

    2016-01-01

    Although ovarian mature cystic teratomas are the commonest adnexal masses occurring in premenopausal women, there are many challenges faced by gynecologists on deciding upon the best surgical management. There is uncertainty, lack of consensus, and variation in surgical practices. This paper critically analyzes various surgical approaches and techniques used to treat these cysts in an attempt to outline a unified guidance. MEDLINE and EMBASE databases were searched in January 2015 with no date limit using the key words “ovarian teratoma” and “ovarian dermoid.” The search was limited to articles in English language, humans, and female. The two authors conducted the search independently. The laparoscopic approach is generally considered to be the gold standard for the management. Oophorectomy should be the standard operation except in younger women with a single small cyst. The risk of chemical peritonitis after contents spillage is extremely rare and can certainly be overcome with thorough peritoneal lavage using warmed fluid. There is a place for surveillance in some selected cases. PMID:27110246

  2. Cystic fibrosis in Finland: a molecular and genealogical study.

    PubMed

    Kere, J; Norio, R; Savilahti, E; Estivill, X; de la Chapelle, A

    1989-08-01

    The incidence of cystic fibrosis (CF) in Finland is one tenth that in other Caucasian populations. To study the genetics of CF in Finland, we used a combined molecular and genealogical approach. Out of the 20 Finnish families with a living CF patient, 19 were typed for eight closely linked restriction fragment length polymorphisms (RFLP) at the MET, D7S8, and D7S23 loci. The birthplaces of the parents and grandparents were traced using population registries. Allele and haplotype frequencies in Finland are similar to those of other European and North American populations, but are modified by sampling: two regional CF gene clusters, evidently the results of a founder effect, were identified. Generally, the gene was evenly distributed over the population, carrier frequency being estimated at approximately 1.3%. We conclude that CF in Finland is caused by the common Caucasian mutation(s), and that the low frequency of the gene can be explained by a negative sampling effect and genetic drift.

  3. Mechanisms of gastro-oesophageal reflux in cystic fibrosis.

    PubMed Central

    Cucchiara, S; Santamaria, F; Andreotti, M R; Minella, R; Ercolini, P; Oggero, V; de Ritis, G

    1991-01-01

    Abnormal degrees of gastro-oesophageal reflux (GOR) were detected by 24 hour intraoesophageal pH measurement in 12 of 14 children (mean age 7.9 years; range 5 months-16 years) affected by cystic fibrosis and complaining of symptoms suggesting GOR. These patients underwent combined recording of distal oesophageal motility and intraluminal pH in order to investigate mechanisms of GOR. Inappropriate lower oesophageal sphincter relaxation was the most common mechanism of reflux in all patients. Other mechanisms (appropriate relaxation or lowered pressure of the lower oesophageal sphincter, increased intragastric pressure) were detected less frequently. Frequency of inappropriate lower oesophageal sphincter relaxations was significantly higher in patients with cystic fibrosis than in other study groups (symptomatic GOR, GOR disease complicated by respiratory complaints). Inappropriate lower oesophageal sphincter relaxations occurred with the same frequency in patients with cystic fibrosis and in a group of children with GOR disease complicated by oesophagitis. Abnormalities of distal oesophageal contractions such as decreased amplitude or uncoordinated waves were also recorded in cystic fibrosis patients. Seven patients with cystic fibrosis completed a therapeutic trial for eight weeks consisting of postural treatment and oral cisapride, a new prokinetic drug. The oesophageal acid exposure improved in only three patients. We conclude that pathologic GOR is commonly associated with cystic fibrosis. The predominant reflux mechanism in these patients is a transient inappropriate lower oesophageal sphincter relaxation rather than a low steady state basal lower oesophageal sphincter pressure. PMID:2039253

  4. The clinical spectrum of chronic appendiceal abscess in cystic fibrosis.

    PubMed

    Allen, E D; Pfaff, J K; Taussig, L M; McCoy, K S

    1992-10-01

    To describe the varied characteristics seen in patients with cystic fibrosis who develop chronic abscess formation secondary to unrecognized appendicitis. Patient series. Cystic Fibrosis Care Centers in Columbus, Ohio, and Tucson, Ariz. Five patients with cystic fibrosis who developed chronic abdominal abscesses secondary to occult appendicitis are described. Two patients developed fistula formation with purulent fluid drainage before diagnosis. One patient developed an extensive psoas abscess. Another presented with prolonged fever of unknown origin. These patients were identified by retrospective review of the past 20-year experience at two Cystic Fibrosis Care Centers. Development of chronic abdominal abscess related to unrecognized appendicitis is a rare but important complication in patients with cystic fibrosis. Prompt diagnosis depends on physician familiarity with the varied presentations of this entity. Diagnostic abdominal computed tomography and/or ultrasonography should particularly be considered when patients with cystic fibrosis present with pain, mass, or drainage from the right flank; prolonged fever; a limp; or failure of suspected meconium ileus equivalent syndrome to respond promptly to cathartic measures.

  5. Cystic Odontoma in a Patient with Hodgkin's Lymphoma

    PubMed Central

    Costa, Victor; Caris, Adriana Rocha; León, Jorge Esquiche; Ramos, Carolina Judica; Jardini, Vaneska; Kaminagakura, Estela

    2015-01-01

    Cystic odontoma is a rare entity, which is characterized by the association of a cyst with complex/compound odontoma. The aim of this study was to report the case of a 5-year-old male patient diagnosed previously with Hodgkin's lymphoma and treated successfully with chemotherapy and radiotherapy, who developed a mandibular odontogenic lesion. Physical examination revealed a swelling on the right side of the mandible. Radiographically, a well-defined radiolucent area surrounded by radiopaque material was observed. An incisional biopsy was performed and microscopic analysis showed a cystic lesion consisting of an atrophic epithelium comprising 2-3 cell layers and the absence of inflammation in the cystic capsule. The cyst was decompressed and the lesion was removed after 3 months of follow-up. Microscopic analysis of the surgical specimen showed a cystic hyperplastic epithelium surrounded by an intense chronic inflammatory cell infiltrate, which was in close contact with mineralized tissue resembling dentin and cementum. The final diagnosis was cystic odontoma. Since chemotherapy can affect the growth and development of infant teeth, a relationship between chemotherapy-associated adverse events and cystic odontoma is suggested in the present case. PMID:26618008

  6. Hormonal abnormalities of the pancreas and gut in cystic fibrosis.

    PubMed

    Adrian, T E; McKiernan, J; Johnstone, D I; Hiller, E J; Vyas, H; Sarson, D L; Bloom, S R

    1980-09-01

    We have investigated the effect of cystic fibrosis on alimentary hormones in 10 children by measuring the pancreatic and gut hormone rsponse to a milk drink. Plasma insulin and gastric inhibitory peptide were both significantly reduced (P < 0.05 and P < 0.005, respectively, at 15 min) in the patients with cystic fibrosis, compared with controls, even though the early glucose rise was greater in the former group (P < 0.05 at 15 min). Fasting levels of pancreatic polypeptide were significantly lower in the fibrocystic children (P < 0.01), and the normal response to milk was completely abolished in these patients (P < 0.001). Fasting plasma enteroglucagon concentrations were grossly abolished in the cystic fibrosis patients (P < 0.001) and these remained elevated throughout the test. No significant differences were seen in basal or postmilk responses of plasma glucagon, gastrin, secretin, vasoactive intestinal peptide, or motilin in cystic fibrosis. It would thus appear that the pancreatic polypeptide cell is more susceptible to the effects of the disease process than the beta or alpha cell in cystic fibrosis. Some aspects of the abnormalities in the gastrointestinal endocrine system were similar to those seen in celiac disease and tropical sprue and may, therefore, effect a similar hormonal response in these patients with cystic fibrosis to those with mucosal damage.

  7. Advanced endoscopic ultrasound management techniques for preneoplastic pancreatic cystic lesions

    PubMed Central

    Arshad, Hafiz Muhammad Sharjeel; Bharmal, Sheila; Duman, Deniz Guney; Liangpunsakul, Suthat; Turner, Brian G

    2017-01-01

    Pancreatic cystic lesions can be benign, premalignant or malignant. The recent increase in detection and tremendous clinical variability of pancreatic cysts has presented a significant therapeutic challenge to physicians. Mucinous cystic neoplasms are of particular interest given their known malignant potential. This review article provides a brief but comprehensive review of premalignant pancreatic cystic lesions with advanced endoscopic ultrasound (EUS) management approaches. A comprehensive literature search was performed using PubMed, Cochrane, OVID and EMBASE databases. Preneoplastic pancreatic cystic lesions include mucinous cystadenoma and intraductal papillary mucinous neoplasm. The 2012 International Sendai Guidelines guide physicians in their management of pancreatic cystic lesions. Some of the advanced EUS management techniques include ethanol ablation, chemotherapeutic (paclitaxel) ablation, radiofrequency ablation and cryotherapy. In future, EUS-guided injections of drug-eluting beads and neodymium:yttrium aluminum agent laser ablation is predicted to be an integral part of EUS-guided management techniques. In summary, International Sendai Consensus Guidelines should be used to make a decision regarding management of pancreatic cystic lesions. Advanced EUS techniques are proving extremely beneficial in management, especially in those patients who are at high surgical risk. PMID:27574295

  8. Rhinosinusitis in cystic fibrosis: not a simple story.

    PubMed

    Babinski, Dariusz; Trawinska-Bartnicka, Maria

    2008-05-01

    Chronic inflammation of sinuses and nasal mucosa is found in 74-100% patients suffering from cystic fibrosis, whereas nasal polyps in 6-44% patients. The aim of this paper is to assess rhinosinusitis types taking into account the forms of cystic fibrosis and the kind of CFTR gene mutation. The author presents material of 126 cystic fibrosis patients, 90 with typical clinical features and 36 with atypical phenotype. Genetic tests were carried out to determine the genotype of CFTR gene. The sample was divided into four groups according to the genotype effect on the chloride canal function. Cytological examination of nasal mucosa was carried out in all the patients. In 71.5% of patients with cystic fibrosis, infectious chronic non-specific rhinosinusitis was found. Other types of rhinosinusitis--acute infectious, chronic allergic and non-allergic with eosinophilia were found in 21.4% of patients, whereas in 7.1% of patients no clinical symptoms of rhinosinusitis were found. Nasal polyps were found in 23 (18.3%) patients with cystic fibrosis: in 21 patients with a typical form and in 2 patients with an atypical form. Nasal polyps were more frequent in groups with the genotype consisting of both "strong" mutations than in the group with unknown or "mild" mutations. Rhinosinusitis in cystic fibrosis patients is not homogenous pathology. Infectious chronic non-specific rhinosinusitis is found the most frequently, but other forms of rhinosinusitis appear quite often and they require proper treatment.

  9. Increased oral bioavailability of ciprofloxacin in cystic fibrosis patients.

    PubMed Central

    Christensson, B A; Nilsson-Ehle, I; Ljungberg, B; Lindblad, A; Malmborg, A S; Hjelte, L; Strandvik, B

    1992-01-01

    The altered pharmacokinetic properties of, e.g., aminoglycosides in cystic fibrosis patients have to be considered when pulmonary exacerbations are treated. Since reported data on ciprofloxacin, a fluorinated quinolone, are conflicting, we compared intravenous and oral administration in cystic fibrosis patients when treating them for mild symptoms of pulmonary infection. All of the patients were colonized with Pseudomonas species. Ciprofloxacin was administered orally (15 mg/kg of body weight) or intravenously (6 mg/kg) twice a day for at least 10 days during separate treatment periods. Five healthy volunteers received single intravenous and oral doses. Pharmacokinetic evaluations were performed at first dose and at steady state. The results showed that cystic fibrosis patients have increased oral bioavailability of ciprofloxacin (80% in cystic fibrosis patients versus 57% in volunteers) and increased total clearance (688 ml/min in CF patients versus 528 ml/min in volunteers). Our data indicate that the pharmacokinetic properties of ciprofloxacin are altered in cystic fibrosis patients with mild symptoms of pulmonary exacerbations and that the changes most probably are due to cystic fibrosis per se or to the impact of chronic infection. PMID:1489195

  10. US Cystic Fibrosis Foundation and European Cystic Fibrosis Society consensus recommendations for the management of non-tuberculous mycobacteria in individuals with cystic fibrosis: executive summary

    PubMed Central

    Olivier, Kenneth N; Saiman, Lisa; Daley, Charles L; Herrmann, Jean-Louis; Nick, Jerry A; Noone, Peadar G; Bilton, Diana; Corris, Paul; Gibson, Ronald L; Hempstead, Sarah E; Koetz, Karsten; Sabadosa, Kathryn A; Sermet-Gaudelus, Isabelle; Smyth, Alan R; van Ingen, Jakko; Wallace, Richard J; Winthrop, Kevin L; Marshall, Bruce C; Haworth, Charles S

    2016-01-01

    Non-tuberculous mycobacteria (NTM) are ubiquitous environmental organisms that can cause chronic pulmonary infection, particularly in individuals with pre-existing inflammatory lung disease, such as cystic fibrosis (CF). Pulmonary disease (PD) caused by NTM has emerged as a major threat to the health of individuals with CF, but remains difficult to diagnose and problematic to treat. In response to this challenge, the US Cystic Fibrosis Foundation (CFF) and the European Cystic Fibrosis Society (ECFS) convened a panel of 19 experts to develop consensus recommendations for the screening, investigation, diagnosis and management of NTM-PD in individuals with CF. PICO (population, intervention, comparison, outcome) methodology and systematic literature reviews were employed to inform draft recommendations, which were then modified to achieve consensus and subsequently circulated for public consultation within the USA and European CF communities. We have thus generated a series of pragmatic, evidence-based recommendations as an initial step in optimising management for this challenging condition. PMID:26678435

  11. Trafficking and function of the cystic fibrosis transmembrane conductance regulator: a complex network of posttranslational modifications

    PubMed Central

    McClure, Michelle L.; Barnes, Stephen; Brodsky, Jeffrey L.

    2016-01-01

    Posttranslational modifications add diversity to protein function. Throughout its life cycle, the cystic fibrosis transmembrane conductance regulator (CFTR) undergoes numerous covalent posttranslational modifications (PTMs), including glycosylation, ubiquitination, sumoylation, phosphorylation, and palmitoylation. These modifications regulate key steps during protein biogenesis, such as protein folding, trafficking, stability, function, and association with protein partners and therefore may serve as targets for therapeutic manipulation. More generally, an improved understanding of molecular mechanisms that underlie CFTR PTMs may suggest novel treatment strategies for CF and perhaps other protein conformational diseases. This review provides a comprehensive summary of co- and posttranslational CFTR modifications and their significance with regard to protein biogenesis. PMID:27474090

  12. Modulation of Heavy Rainfall in the Middle East and North Africa by Madden-Julian Oscillation Using High Resolution Atmospheric General Circulation Model

    NASA Astrophysics Data System (ADS)

    Deng, L.; Stenchikov, G. L.; McCabe, M. F.; Bangalath, H. K.

    2014-12-01

    Recently, the modulation of subtropical rainfall by the dominant tropical intraseasonal signal of the Madden-Julian Oscillation (MJO), has been explored through the discussion of the MJO-convection-induced Kelvin and Rossby wave related teleconnection patterns. Our study focuses on characterizing the modulation of heavy rainfall in the Middle East and North Africa (MENA) region by the MJO, using the Geophysical Fluid Dynamics Laboratory (GFDL) global High Resolution Atmospheric Model (HIRAM) simulations (25-km; 1979-2012) and a combination of available atmospheric products from satellite, in-situ and reanalysis data. The observed Hadley Centre Global Sea Ice and Sea Surface Temperature (HadISST) and the simulated SST from GFDL's global coupled carbon-climate Earth System Models (ESM2M) are employed in HIRAM to investigate the sensitivity of the simulated heavy rainfall and MJO to SST. The future trend of the extreme rainfalls and their links to the MJO response to climate change are examined using HIRAM simulations of 2012-2050 with the RCP4.5 and RCP 8.5 scenarios to advance the possibility of characterization and forecasting of future extreme rainfall events in the MENA region.

  13. Expression of cystic fibrosis transmembrane conductance regulator corrects defective chloride channel regulation in cystic fibrosis airway epithelial cells

    NASA Astrophysics Data System (ADS)

    Rich, Devra P.; Anderson, Matthew P.; Gregory, Richard J.; Cheng, Seng H.; Paul, Sucharita; Jefferson, Douglas M.; McCann, John D.; Klinger, Katherine W.; Smith, Alan E.; Welsh, Michael J.

    1990-09-01

    The cystic fibrosis transmembrane conductance regulator (CFTR) was expressed in cultured cystic fibrosis airway epithelial cells and Cl- channel activation assessed in single cells using a fluorescence microscopic assay and the patch-clamp technique. Expression of CFTR, but not of a mutant form of CFTR (ΔF508), corrected the Cl- channel defect. Correction of the phenotypic defect demonstrates a causal relationship between mutations in the CFTR gene and defective Cl- transport which is the hallmark of the disease.

  14. Airway Clearance Devices for Cystic Fibrosis

    PubMed Central

    2009-01-01

    Executive Summary Objective The purpose of this evidence-based analysis is to examine the safety and efficacy of airway clearance devices (ACDs) for cystic fibrosis and attempt to differentiate between devices, where possible, on grounds of clinical efficacy, quality of life, safety and/or patient preference. Background Cystic fibrosis (CF) is a common, inherited, life-limiting disease that affects multiple systems of the human body. Respiratory dysfunction is the primary complication and leading cause of death due to CF. CF causes abnormal mucus secretion in the airways, leading to airway obstruction and mucus plugging, which in turn can lead to bacterial infection and further mucous production. Over time, this almost cyclical process contributes to severe airway damage and loss of respiratory function. Removal of airway secretions, termed airway clearance, is thus an integral component of the management of CF. A variety of methods are available for airway clearance, some requiring mechanical devices, others physical manipulation of the body (e.g. physiotherapy). Conventional chest physiotherapy (CCPT), through the assistance of a caregiver, is the current standard of care for achieving airway clearance, particularly in young patients up to the ages of six or seven. CF patients are, however, living much longer now than in decades past. The median age of survival in Canada has risen to 37.0 years for the period of 1998-2002 (5-year window), up from 22.8 years for the 5-year window ending in 1977. The prevalence has also risen accordingly, last recorded as 3,453 in Canada in 2002, up from 1,630 in 1977. With individuals living longer, there is a greater need for independent methods of airway clearance. Airway Clearance Devices There are at least three classes of airway clearance devices: positive expiratory pressure devices (PEP), airway oscillating devices (AOD; either handheld or stationary) and high frequency chest compression (HFCC)/mechanical percussion (MP

  15. Vorinostat in Treating Patients With Locally Advanced, Recurrent, or Metastatic Adenoid Cystic Carcinoma

    ClinicalTrials.gov

    2017-09-15

    Recurrent Oral Cavity Adenoid Cystic Carcinoma; Recurrent Salivary Gland Carcinoma; Salivary Gland Adenoid Cystic Carcinoma; Stage III Major Salivary Gland Cancer AJCC v7; Stage III Oral Cavity Adenoid Cystic Carcinoma AJCC v6 and v7; Stage IVA Major Salivary Gland Cancer AJCC v7; Stage IVA Oral Cavity Adenoid Cystic Carcinoma AJCC v6 and v7; Stage IVB Major Salivary Gland Cancer AJCC v7; Stage IVB Oral Cavity Adenoid Cystic Carcinoma AJCC v6 and v7; Stage IVC Major Salivary Gland Cancer AJCC v7; Stage IVC Oral Cavity Adenoid Cystic Carcinoma AJCC v6 and v7; Tongue Carcinoma

  16. Cystic fibrosis transmembrane conductance regulator-modifying medications: the future of cystic fibrosis treatment.

    PubMed

    Pettit, Rebecca S

    2012-01-01

    To review and evaluate cystic fibrosis transmembrane conductance regulator (CFTR) modulators for the treatment of cystic fibrosis (CF). Literature was accessed through MEDLINE (1977-January 2012), the Cochrane Library, and International Pharmaceutical Abstracts (1977-March 2012). Search terms included ivacaftor, VX-770, VX-809, ataluren, PTC 124, CFTR modulator, and cystic fibrosis. All English-language articles identified from the data sources were evaluated for inclusion. Clinical trials and relevant review articles were evaluated for each CFTR modulator. CF is caused by a mutation in the gene that encodes for the CFTR protein; mutations can be separated into 5 different classes. Ivacaftor is a new CFTR potentiator that helps the CFTR channel open properly in patients with the CFTR mutation, G551D. Patients in one study had significant decreases in sweat chloride values and increases in pulmonary function tests. Ivacaftor was approved by the Food and Drug Administration (FDA) to be taken orally at a dose of 150 mg twice a day in G551D CF patients older than 6 years. Additional studies are investigating the use of ivacaftor in other gating mutations and in younger patients. VX-809 is a CFTR corrector that modulates the folding and trafficking of CFTR. VX-809 was originally studied alone in patients with F508del mutation but is now being used in combination with ivacaftor in Phase 2 studies. Ataluren allows the read through of premature stop codons, and studies in patients with CF with nonsense mutations show an increase in chloride transportation. Ataluren requires 3 times a day dosing and is currently in a Phase 3 placebo-controlled study. Three new agents, ivacaftor, VX-809, and ataluren, target the basic defects in CFTR production. Ivacaftor was recently FDA approved, while the other 2 agents are still in clinical trials. Patients with CF will benefit from personalized medicine based on their specific genotype.

  17. Abnormal electrochemical skin conductance in cystic fibrosis.

    PubMed

    Hubert, Dominique; Brunswick, Philippe; Calvet, Jean-Henri; Dusser, Daniel; Fajac, Isabelle

    2011-01-01

    Electrochemical skin conductance measurement is an active electrophysiologic method in which incremental low direct voltage is applied on the skin. It generates a current due to reverse iontophoresis which previous studies suggested to be mostly related to chloride anion movements. As sweat chloride movements upon electric stimulation were likely to be impaired in cystic fibrosis (CF) patients, we designed a proof-of-concept study to measure electrochemical skin conductance in CF patients and control subjects and to test the ability of this method to discriminate CF from controls. Electrochemical skin conductance was measured in 41 adult patients with classical CF and 20 healthy control subjects. Patients placed their hands and feet on nickel electrodes and an incremental low direct voltage was applied on the anode during 2min. The resulting voltage on the cathode and the current generated between anode and cathode were measured and from them, two electrochemical skin conductance variables were calculated: ESC, obtained when a low voltage of 1.6V was applied, and dESC which took into account electrochemical skin conductances obtained when low and high voltages were applied. ESC measurements on hands and feet were significantly different in CF patients (on feet: 75±10μSi), as compared with control subjects (62±13μSi, p<0.0001); dESC was also significantly different and more discriminative in CF patients (on feet: 34±24μSi), as compared with control subjects (93±24μSi, p<0.0001). dESC measurement provided a diagnostic specificity of 1 and a sensitivity of 0.93. These results show that electrochemical skin conductance which is easily and rapidly measured is abnormal in CF patients. Trial registry name in the European Clinical Trials Database (eudraCT): "EZSCAN MUCO1: Mesure de la conductance cutanée par chronoampérométrie", N°EUDRACT: 2007-A00221-52. Copyright © 2010 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.

  18. Diffuse cystic lung diseases: differential diagnosis.

    PubMed

    Baldi, Bruno Guedes; Carvalho, Carlos Roberto Ribeiro; Dias, Olívia Meira; Marchiori, Edson; Hochhegger, Bruno

    2017-01-01

    Diffuse cystic lung diseases are characterized by cysts in more than one lung lobe, the cysts originating from various mechanisms, including the expansion of the distal airspaces due to airway obstruction, necrosis of the airway walls, and parenchymal destruction. The progression of these diseases is variable. One essential tool in the evaluation of these diseases is HRCT, because it improves the characterization of pulmonary cysts (including their distribution, size, and length) and the evaluation of the regularity of the cyst wall, as well as the identification of associated pulmonary and extrapulmonary lesions. When combined with clinical and laboratory findings, HRCT is often sufficient for the etiological definition of diffuse lung cysts, avoiding the need for lung biopsy. The differential diagnoses of diffuse cystic lung diseases are myriad, including neoplastic, inflammatory, and infectious etiologies. Pulmonary Langerhans cell histiocytosis, lymphangioleiomyomatosis, lymphocytic interstitial pneumonia, and follicular bronchiolitis are the most common diseases that produce this CT pattern. However, new diseases have been included as potential determinants of this pattern. RESUMO As doenças pulmonares císticas difusas se caracterizam pela presença de cistos envolvendo mais de um lobo pulmonar, que se originam por diversos mecanismos, incluindo dilatação dos espaços aéreos distais por obstrução, necrose das paredes das vias aéreas e destruição do parênquima. Essas doenças apresentam evolução variável. A TCAR é fundamental na avaliação dessas doenças uma vez que permite uma melhor caracterização dos cistos pulmonares, incluindo sua distribuição, tamanho, extensão e regularidade das paredes, assim como a determinação de outras lesões pulmonares e extrapulmonares associadas. Frequentemente a TCAR é suficiente para a definição etiológica dos cistos pulmonares difusos, associada a achados clínicos e laboratoriais, sem a necessidade

  19. Genetic analysis of hispanic individuals with cystic fibrosis

    SciTech Connect

    Grebe, T.A.; Doane, W.W.; Norman, R.A.; Rhodes, S.N. ); Seltzer, W.K. ); DeMarchi, J.; Silva, D.K.; Gozal, D.; Bowman, C.M.; Accurso, F.J.; Jain, K.D. )

    1994-03-01

    The authors have performed molecular genetic analysis of Hispanic individuals with cystic fibrosis (CF) in the southwestern United States. Of 129 CF chromosomes analyzed, oly 46% (59/129) carry [Delta]F508. The G542X mutation was found on 5% (7/129) of CF chromosomes. The 3849+10kbC[yields]T mutation, detected primarily in Ashkenazi Jews, was present on 2% (3/129). R1162X and R334W, mutations identified in Spain and Italy, each occurred on 1.6% (2/129) of CF chromosomes. W1282X and R553X were each detected once. G551D and N1303K were not found. Overall, screening for 22 or more mutations resulted in detection of only 58% of CF transmembrane conductance regulator gene mutations among Hispanic individuals. Analysis of KM19/XV2c haplotypes revealed an unusual distribution. Although the majority of [Delta]508 mutations are on chromosomes of B haplotypes, the other CF mutations are on A and C haplotypes at higher-than-expected frequencies. These genetic analysis demonstrate significant differences between Hispanic individuals with CF and those of the general North American population. Assessment of carrier/affected risk in Hispanic CF individuals cannot, therefore, be based on the mutation frequencies found through studies of the general population but must be adjusted to better reflect the genetic makeup of this ethnic group. Further studies are necessary to identify the causative mutation(s) in this population and to better delineate genotype/phenotype correlations. These will enable counselors to provide more accurate genetic counseling. 22 refs., 2 tabs.

  20. Cystic meningioma: radiological, histological, and surgical particularities in 43 patients.

    PubMed

    Boukobza, Monique; Cebula, Helene; Pop, Raoul; Kouakou, Fulbert; Sadoun, Amirouche; Coca, Hugo Andres; Polivka, Marc; Diemidio, Paolo; Ganau, Mario; George, Bernard; Froelich, Sebastien; Proust, Francois; Chibbaro, Salvatore

    2016-10-01

    The presence of cysts is a rare occurrence for intracranial meningiomas in adults. We report our experience in a large consecutive series of cystic meningiomas. We prospectively collected data for a dedicated database of cystic meningioma cases between January 2004 and December 2011 in two tertiary neurosurgical centers. Studied data included preoperative imaging, surgical records, and pathology reports. Among 1214 surgeries for intracranial meningioma, we identified 43 cases of cystic meningioma, corresponding to an incidence of 3.5 %. The most common localization was the hemispheric convexity (17/43 cases). Twenty-eight patients had intratumoral cysts, nine peritumoral, and five mixed intra and extratumoral. In 29 patients with available diffusion imaging, ADC coefficients were significantly lower in grade II-III tumors compared to grade I (p = 0.01). Complete resection of the cystic components was possible in 27/43 patients (63 %); partial resection in 4/43 (9 %); in 6/43 (14 %) cyst resection was not possible but multiple biopsies were performed from the cystic walls; in another 6/43 (14 %) the cystic wall was not identified during surgery. Cells with neoplastic features were identified within the cyst walls at pathology in 26/43 cases (60 %). All patients were followed-up for 24 months; long-term follow-up was available only in 32 patients for an average period of 49 months (range, 36-96 months). No recurrence requiring surgery was observed. Cystic meningiomas are rare. Cells with neoplastic features are often identified within the cyst walls. Complete cyst resection is recommendable when considered technically feasible and safe.