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Lisowska Aleksandra - - 2010
Small intestine bacterial overgrowth (SIBO) has been reported in cystic fibrosis (CF) patients. However, the potential link to intestinal inflammation has not been studied so far. Therefore, we aimed to assess whether SIBO correlates with intestinal inflammation in CF patients. As a preliminary study, we assessed whether calprotectin is detectable ...
Werlin Steven L - - 2010
Treatment with pancreatic enzymes fails to completely correct malabsorption and gastrointestinal symptoms in patients with cystic fibrosis (CF). The aim of the present study was to examine the small intestine of patients with CF without overt evidence of gastrointestinal disease using capsule endoscopy (CE). Patients with CF received the agile ...
Sumida Yoshio Y Center for Digestive and Liver Diseases, Department of Pathology, Nara City Hospital, - - 2010
  The biological basis of variability in histological progression of non-alcoholic fatty liver disease (NAFLD) remains unknown. Dehydroepiandrosterone (DHEA), the most abundant steroid hormone, has been shown to influence sensitivity to reactive oxygen species, insulin sensitivity and expression of peroxisome proliferator-activated receptor-α. Our aim was to determine whether more histologically ...
Fricker-Hidalgo Hélène - - 2010
Allergic bronchopulmonary aspergillosis (ABPA) is a frequent complication in cystic fibrosis patients. The diagnosis remains difficult and requires a combination of clinical, radiological, biological, and mycological criteria. The aim of this study was to analyze the added value of two recombinant antigens, rAspf4 and rAspf6, associated with the detection of ...
Marinho Carolina Coimbra - - 2010
Non-invasive markers of fibrosis have been used to diagnose liver fibrosis in a variety of diseases. Hyaluronic acid (HA) and collagen IV (C-IV) levels were measured in the sera of patients from an endemic area for schistosomiasis in Brazil to diagnose and to rank the intensity of liver fibrosis. Seventy-nine ...
Cobano─člu Nazan - - 2010
We investigated the presence of anti-neutrophil cytoplasmic antibodies (ANCA) in the serum and bronchoalveolar lavage fluid (BALF) of 21 cystic fibrosis (CF), 7 idiopathic bronchiectasis (IBR), and 11 control children and the relation between ANCA and any bacteria grown in BALF. Six of the CFs, but none of the IBRs ...
Robroeks Charlotte M H H T - - 2010
In cystic fibrosis (CF), airway inflammation causes an increased production of reactive oxygen species, responsible for degradation of cell membranes. During this process, volatile organic compounds (VOCs) are formed. Measurement of VOCs in exhaled breath of CF patients may be useful for the assessment of airway inflammation. This study investigates ...
Becker S - - 2010
BACKGROUND AND AIM: Nephrogenic systemic fibrosis (NSF) is a highly debilitating disorder primarily affecting the skin, but also other organ compartments. So far, it has only occurred in patients suffering from acute or chronic renal failure, with almost all of them having been exposed to gadolinium-based contrast agents (GBCA). The ...
Seifert Clarice M - - 2010
BACKGROUND: There is significant debate on the influence of inflammatory mucosal disease on paranasal sinus pneumatization (PSP) and temporal bone pneumatization (TBP) in cystic fibrosis patients (CF). It is often assumed that mucosal disease of the paranasal sinuses will negatively influence development and pneumotization of the paranasal sinuses and temporal ...
Zemanick Edith T - - 2010
Pulmonary exacerbations are a major cause of morbidity in cystic fibrosis (CF) and likely contribute to lung function decline. Exacerbations are often associated with characteristic airway bacteria [CF related bacteria (CFRB)]. However, some patients do not have CFRB detected by culture during exacerbations. We sought to determine the proportion of ...
Jambhekar Supriya K - - 2010
PURPOSE: To report two patients with associated conditions in addition to cystic fibrosis. METHODS: We reviewed our database and report two patients with cystic fibrosis who had associated conditions. These patients also had novel disease causing CFTR mutations on full gene sequence analysis. RESULTS: We identified 2 patients with novel ...
Georgiopoulou Vasiliki V - - 2010
Survival of patients with cystic fibrosis (CF) has improved, resulting in increased exposure of patients to cardiovascular risk factors. Diabetes mellitus is common in patients with CF; however, less is known about lipid abnormalities in this population. In this study, the prevalence of lipid abnormalities was investigated in a contemporary ...
Olesen Hanne Vebert - - 2010
BACKGROUND: Cystic fibrosis (CF) affects the lungs causing infections and inflammation. Surfactant protein D (SP-D) is an innate defense lectin primarily secreted in the lungs. We investigated the influence of the SP-D Met11Thr polymorphism on CF lung function; and serum SP-D as a marker for CF lung disease. METHODS: For ...
Meeus Mira - - 2011
To evaluate the criterion validity and internal consistency of the International Physical Activity Questionnaire-short form (IPAQ-sf) in Chronic Fatigue Syndrome (CFS) patients. Fifty-six CFS patients completed the IPAQ-sf after they wore a tri-axial accelerometer and filled out activity diaries during 1 week. Spearman rank correlation coefficients and Cronbach's Alpha were ...
Zhao Xiao - - 2010
OBJECTIVES: To establish and compare the distance and angle from the limen nasi to the sphenoid ostium in pediatric patients with normal sinonasal anatomy vs pediatric patients with cystic fibrosis (CF). DESIGN: Retrospective review of computed tomographic images. SETTING: Tertiary university-based medical center. PARTICIPANTS: Patients (newborn to age 20 years) ...
Goetz Danielle M - - 2010
Late presentation of congenital diaphragmatic hernia (CDH) is unusual, especially in patients with cystic fibrosis (CF). To our knowledge, cases of CDH in CF patients and the combined effects on lung function have not been previously described. Here we report two cases of late presenting CDH in CF patients and ...
Shimada Kenji - - 2010
OBJECTIVES: To review our experience with female children who have unilateral renal agenesis or renal dysplasia associated with cystic lesions in the reproductive system. METHODS: Between 1991 and 2007, we treated 26 patients with unilateral renal agenesis or renal dysplasia associated with pelvic, retrovesical or interlabial cystic lesions. In 16 ...
van der Doef H P J - - 2010
In Cftr-/- mice that mostly die because of intestinal obstruction, intestinal expression of Clca3 is decreased, whereas upregulation of Clca3 results in amelioration of intestinal disease. The aim of the study was to investigate whether the p.S357N variant in CLCA1, the human orthologue of Clca3, acts as a modifier gene ...
Fallahi Gholamhossein - - 2010
Cystic fibrosis (CF) is a hereditary disease, characterized by chronic pulmonary disease, pancreatic insufficiency and abnormal electrolytes in the sweat. In order to evaluate the clinical manifestations and laboratory findings of Iranian children with CF during a 10-year period, 243 CF patients, with a median age of 5 months, were ...
Walkowiak Jaros?aw - - 2010
BACKGROUND: Most methods used for the assessment of severe steatorrhea in cystic fibrosis (CF) are sensitive. In fact, the tests show their usefulness in a borderline zone of the results. Yet, the existing data related to acid steatocrit (AS) are still contradictory. Therefore, in the present study we have aimed ...
Lupsor Monica - - 2010
BACKGROUND/AIMS: Transient elastography (TE) is a noninvasive method for predicting liver fibrosis, mainly validated in patients with viral hepatitis. Information is still limited concerning its performance in non-alcoholic steatohepatitis (NASH) patients. We aimed to assess the value of TE in the prediction of fibrosis stage in NASH as well as ...
Gal Sol?ne Le - - 2010
We retrospectively investigated 76 patients with cystic fibrosis for the presence of Pneumocystis jirovecii, by performing real-time PCR and nested-PCR assays on 146 archival sputum specimens. P. jirovecii was detected in only 1 patient (1.3%) showing that in our region (Brest, France), the fungus is rarely involved in pulmonary colonization ...
O'Brien Catherine E CE College of Pharmacy, Department of Pharmacy Practice, University of Arkansas for Medical Sciences, Little Rock, AR 72205, USA. - - 2010
To describe the use of lubiprostone for constipation in 3 adults with cystic fibrosis (CF). This case series describes the use of lubiprostone for the treatment of constipation in 3 adults with CF (mean +/- SD length of therapy 17.3 +/- 1.5 mo). All 3 patients were prescribed lubiprostone 24 ...
Menten Renaud - - 2010
BACKGROUND: Hepatic involvement is frequent in patients with cystic fibrosis (CF), with focal biliary cirrhosis being the pathognomonic hepatic manifestation. In around one-quarter of CF patients, it results in CF-associated liver disease (CFLD). This occurs as a relatively early complication with the majority of patients presenting in childhood or their ...
Jarad N A - - 2010
BACKGROUND: Magnetic resonance imaging (MRI) has been shown to be a useful tool to evaluate the volume of the pancreas. There is currently no information about the size of the spleen in cystic fibrosis (CF) patients. PATIENTS AND METHODS: We investigated 51 adult volunteers: 28 pancreatic insufficient CF patients [13 ...
Scheenstra Renske J - - 2010
CONCLUSION: Our results indicate that repeated treatment courses with tobramycin 10 mg/kg (twice daily for 3 weeks) may be safely applied in cystic fibrosis (CF) patients with respect to ototoxicity. The risk of hearing loss in this patient group is less than expected, which could be explained by either unfavourable ...
Druce M R - - 2010
BACKGROUND: Fibrosis is a hallmark of neuroendocrine tumours (NETs) arising in the jejunum and ileum and may manifest in the mesentery and elsewhere. It is clinically important and once-established, there are few effective therapies. AIM: To examine the frequency, radiological manifestations and clinical significance of intra-abdominal fibrosis in a patient ...
Bruno Savino - - 2010
The objective of this study is to determine the efficacy and safety of peginterferon alfa-2a (40 KD)/ribavirin in patients with advanced fibrosis. Data from 341 genotype 1/4 patients (99 with bridging fibrosis/cirrhosis) treated for 48 weeks and 1547 genotype 2/3 patients (380 with bridging fibrosis/cirrhosis) treated for 16 or 24 ...
Gailhouste Luc - - 2010
Imaging of supramolecular structures by multiphoton microscopy offers significant advantages for studying specific fibrillar compounds in biological tissues. In this study, we aimed to demonstrate the relevance of Second Harmonic Generation (SHG) for assessing and quantifying, without staining, fibrillar collagen in liver fibrosis. We first showed the relationship between SHG ...
Chapman Anna L P - - 2010
Decreased expired nitric oxide (eNO) is commonly observed in cystic fibrosis (CF) patients and is usually explained by dysregulation of NO synthase (NOS) isoforms in respiratory tract epithelium. Later stages of this disease are accompanied by intense airway infiltration of phagocytes with high NOS activity, abundant levels of the hemoprotein ...
Bulitta J B - - 2010
Despite the promising activity of ceftazidime against Pseudomonas aeruginosa and Burkholderia cepacia, there has not yet been a study that directly compared the pharmacokinetics (PK) of ceftazidime in cystic fibrosis (CF) patients and healthy volunteers by population PK methodology. We assessed the population PK and PK/pharmacodynamic (PD) breakpoints of ceftazidime ...
Veronez L - - 2010
This study was designed to use volumetric capnography to evaluate the breathing pattern and ventilation inhomogeneities in patients with chronic sputum production and bronchiectasis and to correlate the phase 3 slope of the capnographic curve to spirometric measurements. Twenty-four patients with cystic fibrosis (CF) and 21 patients with noncystic fibrosis ...
Houwen Roderick H - - 2010
OBJECTIVES: Various definitions for distal intestinal obstruction syndrome (DIOS), meconium ileus equivalent, and constipation in patients with cystic fibrosis (CF) are used. However, an unequivocal definition for DIOS, meconium ileus equivalent, and constipation is preferred. The aims of this study were, therefore, to seek consensus on the definitions for DIOS ...
Walkowiak Jaros?aw - - 2010
BACKGROUND: The coexistence of cystic fibrosis (CF) and celiac disease (CD) has been reported. To our knowledge there is no study directly comparing the incidence of CD in CF patients to that in the general population at the same time. There is no published data on genetic predisposition to CD ...
Porro Chiara - - 2010
Microparticles (MPs) are membrane vesicles released during cell activation and apoptosis. MPs have different biological effects depending on the cell from they originate. Cystic fibrosis (CF) lung disease is characterized by massive neutrophil granulocyte influx in the airways, their activation and eventually apoptosis. We investigated on the presence and phenotype ...
Sardi Ana Sofia Del Castillo AS Hospital Santo Tomás, Ciudad de Panamá, República de Panamá. - - 2010
Hypertrophic cardiomyopathy is a rare, autosomal dominant cardiac disorder characterized by hypertrophy of the interventricular septum and mitral valve abnormalities. A 25-year old female, second gestation, with a diagnosis of hypertrophic cardiomyopathy for four years and history of mild intermittent asthma controlled with sporadical use of corticosteroids. On physical exam, ...
Mayer Beate - - 2010
BACKGROUND: In this study, the data of eight new patients in conjunction with previously reported cases with piperacillin-induced immune hemolytic anemia (PIHA) are described. STUDY DESIGN AND METHODS: Five patients with cystic fibrosis and three patients with other disorders developed massive hemolysis after administration of piperacillin. Serologic tests were carried ...
Kerr Jonathan R - - 2010
Chronic fatigue syndrome/myalgic encephalomyelitis (CFS/ME) is a neuro-immune disease of uncertain pathogenesis. Human parvovirus B19 infection has been shown to occur just prior to development of the onset of CFS/ME in several cases, although B19 seroprevalence studies do not show any significant differences between CFS/ME and controls. In this study, ...
van der Doef H P J - - 2010
BACKGROUND: The aims of this study were to determine prevalence, risk factors and treatment of constipation in patients with Cystic Fibrosis (CF), as well as the diagnostic value of abdominal radiography. METHODS: A cohort of 214 pediatric CF patients was investigated. Furthermore, 106 abdominal radiographs of CF patients with or ...
Yazdanpanah Mohammad Javad MJ Department of Dermatology, Ghaem Hospital, Mashhad University of Medical Sciences, Mashhad , Iran. - - 2009
A seventeen-year-old male presented with a restriction in the oral cavity. The oral mucosa was white and he could not protrude his tongue. For 4 years he had habitually held a powdery material containing betel nut in the oral vestibule for several minutes a day. A biopsy of the labial ...
Martha B - - 2010
Although the mucoid form of Pseudomonas aeruginosa (Pa) is largely responsible for the progression of lung disease in cystic fibrosis (CF), the relationship between factors relating daily-care regimes to mucoidy acquisition are as yet poorly investigated. Fifty-two CF patients registered at the CF centre of Dijon, France, were retrospectively evaluated ...
Hansen C R - - 2010
BACKGROUND: Achromobacter xylosoxidans infection may cause conspicuous chronic pulmonary inflammation in cystic fibrosis (CF) patients similar to Pseudomonas aeruginosa and the Burkholderia cepacia complex (Bcc). Evolution in lung function was compared in chronically infected patients. Cytokine concentrations in CF patients with and without chronic infection were compared to healthy controls. ...
Walsh Aimee C AC School of Medicine and Public Health, University of Wisconsin, Madison, Wisconsin, - - 2010
A comparison of the longitudinal progression of lung disease in cystic fibrosis patients identified through newborn screening (NBS) in cohorts located in two different countries has never been performed and was the primary objective of this study. The study included 56 patients in Brittany diagnosed through NBS between 1989 and ...
Ramos Regina Terse Trindade - - 2009
OBJECTIVE: Obstructive sleep apnea syndrome (OSAS) in cystic fibrosis (CF) patients may be associated with the presence of upper airway obstruction caused by chronic infection and nasal polyposis that may also contribute to OSAS severity. Our objective was to identify the profile of findings in CF and OSAS patients by ...
Reichel Martin - - 2010
BACKGROUND: Acid sphingomyelinase (ASM; EC 3.1.4.12) hydrolyses membrane sphingomyelin into the bioactive lipid ceramide and is thus involved in different cellular processes such as differentiation, immunity, or cell death. Activation of ASM has been reported in particular in conjunction with the cellular stress response to several external stimuli, and increased ...
Nery Fabiano G - - 2010
Alcoholism is highly prevalent among bipolar disorder (BD) patients, and its presence is associated with a worse outcome and refractoriness to treatment of the mood disorder. The neurobiological underpinnings that characterize this comorbidity are unknown. We sought to investigate the neurochemical profile of the dorsolateral prefrontal cortex (DLPFC) of BD ...
Matel Julia L - - 2009
Cystic fibrosis (CF) is mostly recognized for its pulmonary morbidity, but the earliest manifestations of the disease are related to its gastrointestinal and nutritional derangements. Destruction of acinar pancreatic tissue, pancreatic ductular obstruction, and lack of enzymatic activity lead to malabsorption (particularly of fats), diarrhea, and failure to thrive. A ...
Bernaba Bob N - - 2010
Anthracyclines are known to have acute cardiotoxicity. Anthracycline-induced dilated cardiomyopathy may have late onset and present years after administration of the drug. Several studies have described the clinical findings in patients with late-onset cardiomyopathy, including electrocardiography, exercise testing, echocardiography, and histological findings in endomyocardial biopsies; however, there is little information ...
Szollosi I - - 2011
Background: Whether autonomic dysfunction contributes to tachycardia in cystic fibrosis (CF) is unknown. Methods: Heart rate variability (HRV) was assessed to determine high frequency power and the low/high frequency power ratio (HF, LF/HF) as markers of vagal and sympathovagal balance, respectively, under spontaneous and controlled breathing (15 breaths per minute ...
Bahuleyan Biji - - 2009
Cystic prolactinomas are considered not amenable to dopamine agonist therapy. We present the results of dopamine agonist therapy in six patients with cystic prolactinomas. The inclusion criteria of patients were: (i) cystic macroadenomas with the cyst occupying more than 50% of the tumour volume; (ii) a serum prolactin value more ...
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