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cystic fibrose

A N Ø Schultz, N Høiby, X C Nielsen, T Pressler, K Dalhoff, M Duno, A Buchard, H K Johansen, H Wang, C S Dalbøge
Ciprofloxacin (CIP) is frequently used when treating cystic fibrose (CF) patients with intermittent Pseudomonas aeruginosa (P. aeruginosa) lung colonization. However, approximately 20% of the patients progress to chronic infection despite early intervention. The aim of this study, was to investigate the pharmacokinetics of CIP, to evaluate if CYP3A4-related metabolism is involved and to find the optimal dose needed to eradicate intermittently colonizing bacteria in the lungs of CF patients. Methods An open-label, prospective pharmacokinetic study was performed...
March 2017: Pediatric Pulmonology
Maria da Conceição Marinho Sousa Ribeiro Oliveira Reisinho, Bárbara Pereira Gomes
Objectives: to search for nursing interventions focused on the improvement of quality of life and promotion of self-care of adolescents suffering from the Cystic Fibrosis. Method: literature review. The inclusion criteria were: primary studies and studies with interventions developed by nurses in the adolescent population with Cystic Fibrosis, using Portuguese, Spanish, French and English with no time limit, and supported by the databases Scopus, Web of Science and CINAHL...
December 8, 2016: Revista Latino-americana de Enfermagem
Stephanie Demeyer, Kris De Boeck, Peter Witters, Katrien Cosaert
UNLABELLED: Cystic fibrosis is a life shortening hereditary disease, primarily leading to progressive pulmonary infection and exocrine pancreatic dysfunction. Several gastrointestinal complications other than malabsorption can arise during the disease course and with the progressively increasing life span of patients with CF; new and more rare complications are being recognized. We review the literature on gastrointestinal manifestations in CF, excluding the liver and pancreas. CONCLUSION: We describe the clinical presentation and treatment of more common conditions like gastroesophageal reflux, small intestinal bacterial overgrowth, intussusception, meconium ileus, distal intestinal obstruction syndrome, and constipation, and we also discuss what is known on celiac disease, appendicitis, fibrosing colonopathy, inflammation and inflammatory bowel disease and gastrointestinal cancer...
July 2016: European Journal of Pediatrics
M Leipig, K Abenthum, B Wollanke, I Czupalla, I Esposito, K Steiger, W Hermanns, N Herbach
A 16-year-old Friesian gelding with relapsing colic was humanely destroyed during diagnostic laparotomy due to suspected abdominal neoplasia. On post-mortem examination, the pancreas appeared as a firm mass (20 × 8 × 8 cm). The cut surface had a lobular structure with multiple cavities. Histological examination revealed severe chronic fibrosing pancreatitis with acinar-ductal metaplasia and duct dysplasia, which was considered to be the cause of the recurrent colic. Formation of tubular complexes within a background of acinar-ductal metaplasia is similar to the regressive lesions detected in the human pancreas in the context of inflammation, duct obstruction, cystic fibrosis and neoplasia...
August 2015: Journal of Comparative Pathology
Lisa P Lavelle, Sinead H McEvoy, Elaine Ni Mhurchu, Robert G Gibney, Colm J McMahon, Eric J Heffernan, Dermot E Malone
Cystic fibrosis (CF) is the most common lethal autosomal recessive disease in the white population. Mutation of the CF transmembrane conductance regulator gene on chromosome 7 results in production of abnormally viscous mucus and secretions in the lungs of patients with CF. A similar pathologic process occurs in the gastrointestinal tract, pancreas, and hepatobiliary system. Inspissated mucus causes luminal obstruction and resultant clinical and radiologic complications associated with the disease process. Pancreatic involvement can result in exocrine and endocrine insufficiency, pancreatic atrophy, fatty replacement, or lipomatous pseudohypertrophy...
May 2015: Radiographics: a Review Publication of the Radiological Society of North America, Inc
Minoru Inomata, Soichiro Ikushima, Nobuyasu Awano, Keisuke Kondoh, Kohta Satake, Masahiro Masuo, Yuji Kusunoki, Atsuko Moriya, Hiroyuki Kamiya, Tsunehiro Ando, Noriyo Yanagawa, Toshio Kumasaka, Takashi Ogura, Fumikazu Sakai, Arata Azuma, Akihiko Gemma, Tamiko Takemura
BACKGROUND: Clinical evaluation to differentiate the characteristic features of pulmonary fibrosis and emphysema is often difficult in patients with combined pulmonary fibrosis and emphysema (CPFE), but diagnosis of pulmonary fibrosis is important for evaluating treatment options and the risk of acute exacerbation of interstitial pneumonia of such patients. As far as we know, it is the first report describing a correlation among clinical, radiological, and whole-lung pathological features in an autopsy cases of CPFE patients...
June 28, 2014: BMC Pulmonary Medicine
Sarah Claus, Claudius Weiler, Joerg Schiewe, Wolfgang Friess
In the last decades, dry powder inhalation has become a very attractive option for pulmonary drug delivery to treat lung diseases like cystic fibroses and lung infections. In contrast to the traditional pulmonary application of drugs for asthma and chronic obstructive pulmonary disease, these therapies require higher lung doses to be administered. The developments and improvements toward high dose powder pulmonary drug delivery are summarized and discussed in this chapter. These include the invention and improvement of novel inhaler devices as well as the further development of formulation principles and new powder engineering methods...
January 2014: European Journal of Pharmaceutics and Biopharmaceutics
Audrey Baldessari, Jessica Snyder, Joel Ahrens, Robert Murnane
A 36-year-old male chimpanzee (Pan troglodytes) assigned to a life-long sign language communication project presented for sudden death. No other clinical or clinical pathological abnormalities were noted and given the signalment, death due to cardiac failure was suspected. Necropsy findings revealed moderate cardiomegaly and other chronic age-related findings including focal renal tubular cystic dilation and gingival hyperplasia. Histologic evaluation of the heart revealed interstitial fibrosing cardiomyopathy characterized by severe interstitial myocardial fibrosis replacing and separating myofibers within all chambers of the heart, especially the left ventricle, interventricular septum and subvalvular areas...
2013: Pathobiology of Aging & Age related Diseases
Florian Lang, Jakob Voelkl
INTRODUCTION: Expression of serum-and-glucocorticoid-inducible kinase-1 (SGK1) is low in most cells, but dramatically increases under certain pathophysiological conditions, such as glucocorticoid or mineralocorticoid excess, inflammation with TGFβ release, hyperglycemia, cell shrinkage and ischemia. SGK1 is activated by insulin and growth factors via phosphatidylinositide-3-kinase, 3-phosphoinositide-dependent kinase and mammalian target of rapamycin. SGK1 sensitive functions include activation of ion channels (including epithelial Na(+) channel ENaC, voltage gated Na(+) channel SCN5A transient receptor potential channels TRPV4 - 6, Ca(2+) release activated Ca(2+) channel Orai1/STIM1, renal outer medullary K(+) channel ROMK, voltage gated K(+) channels KCNE1/KCNQ1, kainate receptor GluR6, cystic fibrosis transmembrane regulator CFTR), carriers (including Na(+),Cl(-) symport NCC, Na(+),K(+),2Cl(-) symport NKCC, Na(+)/H(+) exchangers NHE1 and NHE3, Na(+), glucose symport SGLT1, several amino acid transporters), and Na(+)/K(+)-ATPase...
June 2013: Expert Opinion on Investigational Drugs
Konosuke Morimoto, William J Janssen, Mayumi Terada
RATIONALE: Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive fibrosing interstitial pneumonia. The pathogenicity of IPF has been widely investigated but still remains to be clarified. Efferocytosis, the specialized recognition and ingestion of apoptotic cells by phagocytes, is essential for the resolution of inflammation in the lungs and repair of injured tissues. Impaired efferocytosis contributes to the pathogenesis of chronic lung diseases such as emphysema and cystic fibrosis...
December 2012: Respiratory Medicine
Kei Nakajima, Haruki Oshida, Toshitaka Muneyuki, Masafumi Kakei
Pancreatic exocrine insufficiency (PEI) is often observed in patients with pancreatic diseases, including chronic pancreatitis, cystic fibrosis, and tumors, or after surgical resection. PEI often results in malnutrition, weight loss and steatorrhea, which together increase the risk of morbidity and mortality. Therefore, nutritional interventions, such as low-fat diets and pancreatic enzyme replacement therapy (PERT), are needed to improve the clinical symptoms, and to address the pathophysiology of pancreatic exocrine insufficiency...
2012: Core Evidence
Tamara N Pereira, Peter J Lewindon, Ristan M Greer, Anita C Hoskins, Richard M Williamson, Ross W Shepherd, Grant A Ramm
OBJECTIVES: Liver disease contributes to significant morbidity and mortality in cystic fibrosis (CF). Although all patients with CF express the defective CF transmembrane conductance regulator in cholangiocytes, many develop asymptomatic fibrosing liver disease. Only some develop cirrhosis, with pathogenesis remaining enigmatic. Available noninvasive diagnostic tools do not identify patients at risk before development of advanced fibrosis. We conducted a pilot study to identify genes associated with hepatic injury and fibrosis on liver biopsy that may help elucidate determinants of CF-associated liver disease (CFLD)...
March 2012: Journal of Pediatric Gastroenterology and Nutrition
G Terheggen, D Dieninghoff, E Rietschel, U Drebber, W Kruis, L Leifeld
OBJECTIVE: Fibrosing colonopathy (FC) is a rare entity associated with cystic fibrosis (CF). Until now, patients with stricturing FC have usually been treated surgically. In this instance, we aimed at avoiding surgery by applying a new conservative approach. - METHODS: Case report on an adult with CF who developed persistent abdominal pain due to a non-passable stricture in the right transverse colon. Histology confirmed fibrosing colonopathy. - RESULTS: Initially we treated the patient with prednisolone pulse therapy and additive antibiotic therapy...
September 12, 2011: European Journal of Medical Research
James M Littlewood, Gary J Connett, Suntje Sander-Struckmeier, Friederike Henniges
OBJECTIVE: At the request of the Medicines and Healthcare Regulatory Agency and in agreement with the appropriate authorities, an observational, multi-center, non-interventional, post-authorization safety study of high-strength pancreatic enzymes was conducted. RESEARCH DESIGN AND METHODS: Patients with exocrine pancreatic insufficiency due to cystic fibrosis (CF) who had previously taken high doses of pancreatic enzymes received pancreatin 40,000 capsules (Creon 40,000 Minimicrospheres, Abbott GmbH, Hanover, Germany) as part of their normal treatment for up to 2 years...
March 2011: Expert Opinion on Drug Safety
Ming Ye, Pin Tu, Gui-mei Li, Mei-zhao LE, Mao-hong Zhang
OBJECTIVE: To explore the ultrastructural changes of hepatocyte fibrogenesis in cholelithiasis in biliary tract. METHODS: l0 liver biopsies were taken from the patients suffered from gallstone and choledocholithiasis during surgical treatment and the ultrastructural changes were observed under electromicroscope. RESULTS: There were plentiful collagenous microfibrils (CMFs) grown within some hepatocytes. These CMFs distributed locally or diffusely in cytoplasm even extended into nucleus...
December 2010: Zhonghua Gan Zang Bing za Zhi, Zhonghua Ganzangbing Zazhi, Chinese Journal of Hepatology
Robert J Kuhn, Andres Gelrud, Anne Munck, Steven Caras
Exocrine pancreatic insufficiency (EPI) is associated with conditions including cystic fibrosis (CF), chronic pancreatitis (CP), and pancreatic surgery (PS). The symptoms include maldigestion, malnutrition, weight loss, flatulence, and steatorrhea. Pancreatic enzyme replacement therapy (PERT) is the standard treatment for EPI; it is regulated in many countries and most recently in the USA following a US FDA mandate for all PERT manufacturers to submit new drug applications. Pancrelipase delayed-release capsules (CREON®, Abbott, Marietta, GA, USA) have been available in Europe since 1984 and in the USA since 1987; a new formulation was the first PERT to gain approval in the USA in 2009...
December 2010: Advances in Therapy
L Schmitt, M Wiebel, F Frese, C Dehnert, C Zugck, P Bärtsch, H Mairbäurl
When ventilating large volumes of air during exercise, airway fluid secretion is essential for airway function. Since these are impaired in cystic fibrosis and exercise-induced asthma, it was the aim of this study to determine how exercise affects airway Na(+) and Cl(-) transport and whether changes depend on exercise intensity. Nasal potential was measured in Ringer's solution, with amiloride to block Na(+) transport, and in low chloride-containing isoproterenol to assess Cl(-) channels. Nasal potential was measured at rest and during submaximal and maximal bicycle ergometer exercise in individuals with cystic fibrosis, exercise-induced asthma and controls...
February 2011: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
Michael O Stormon, Wan F Ip, Lynda Ellis, Susanne Schibli, Johanna M Rommens, Peter R Durie
BACKGROUND: : Because the acinar cells of the exocrine pancreas in patients with Shwachman-Diamond syndrome (SDS) are severely depleted, we hypothesized that a similar deficiency may be present in acinar cells of the parotid gland. PATIENTS AND METHODS: : We determined serum pancreatic isoamylase and parotid amylase activities in 16 patients with SDS, 13 healthy controls, and 13 disease controls (cystic fibrosis or fibrosing pancreatitis). Parotid amylase and electrolyte concentrations were measured in stimulated parotid gland secretions...
July 2010: Journal of Pediatric Gastroenterology and Nutrition
Brian H Eisner, Michael P Kurtz, Mukesh G Harisinghani
MRI is an imaging modality that is used in the evaluation and treatment of renal cancer. Although less frequently used than CT, MRI is useful in several aspects of renal cancer evaluation and may have some advantages. MRI has shown promising results for renal cancer staging (especially determination of vascular invasion by tumor), in differentiating malignant from benign complex cystic renal lesions, and in the treatment and follow-up of minimally invasive ablative therapies for renal cancer. In addition, new research shows promise for novel applications of MRI...
May 2010: Journal of Endourology
Florian Lang, Agnes Görlach
Heterocyclic indazole derivatives are claimed in patent WO2008138448 as inhibitors of the serum- and glucocorticoid-inducible-kinase 1 (SGK1) and drugs for the pharmacological treatment of SGK1-related diseases, such as diabetes, obesity, metabolic syndrome, systemic and pulmonary hypertension, cardiac fibrosis, hypertrophy and insufficiency, arteriosclerosis, glomerulosclerosis, nephrosclerosis, nephritis, nephropathy, deranged electrolyte excretion, fibrosing and inflammatory disease (e.g., liver cirrhosis, lung fibrosis, rheumatism, arthrosis, Crohn s disease, chronic bronchitis, radiation fibrosis, sclerodermia, cystic fibrosis, scar formation and Alzheimer' disease), tumor growth, peptic ulcers and some disorders hitherto not conclusively shown to involve SGK1...
January 2010: Expert Opinion on Therapeutic Patents
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