OBJECTIVE: In a multicenter study, we evaluated the relationships between the extent and severity of bronchiectasis on CT and clinical symptoms, spirometric abnormality, and sputum characteristics. SUBJECTS AND METHODS: The study population included 261 patients with symptomatic, physiologically significant bronchiectasis, who were enrolled in another study evaluating the clinical efficacy ...

We present a prepubertal male cystic fibrosis patient with high circulating oestrogen levels (as a consequence of sever cystic-fibrosis-related hepatobiliary disease) who subsequently developed a large pelvic arteriovenous malformation. This has not previously been described in patients with cystic fibrosis, despite the association between high oestrogen levels and arteriovenous malformations. ...

In this study, intra-individual variation of resting energy expenditure (REE) in adults with cystic fibrosis (CF) and the effect of measurement duration were determined. Twelve adults with CF and chronic Pseudomonas aeruginosa (Ps. aeruginosa) infection and 12 healthy volunteers, matched for age and sex were studied whilst clinically stable on ...

Lipid peroxidation biomarkers and antioxidant status were measured in 76 cystic fibrosis (CF) patients and compared to 40 control subjects. Univariate and multivariate statistics were performed in this study. Results showed that indicators of lipid peroxidation were higher in CF patients than in controls; thiobarbituric acid reactants and autoantibodies against ...

AIMS: To study and compare the perspectives of relapsed alcohol-dependent patients and their family members regarding relapse precipitants. DESIGN: Observational, cross-sectional survey. SETTING: An addiction centre in northern India. PARTICIPANTS: Thirty consecutive male patients attending the centre with relapse of alcohol dependence following previous inpatient treatment, and their respective family ...

Abstract Although biological markers such as carbohydrate-deficient transferrin (CDT), gamma-glutamyl transferase (GGT) and mean corpuscular volume (MCV) have been used as indicators for heavy alcohol consumption and alcoholism little information is available on the utitlity of these markers in detecting relapses. In this study the value of the biological markers ...

We measured the concentrations of several serum and urinary fibrosis markers, which are metabolites of extracellular matrix, in schistosomiasis patients to investigate their relationship with the ultrasonographic scoring system and with parasitologic data. This study was conducted in patients with various stages of the disease evaluated by ultrasonography (intestinal disease ...

We have conducted a double-blind study to assess the possible involvement of the human herpesviruses (HHVs) HHV6, HHV7, Epstein-Barr virus (EBV), and cytomegalovirus in chronic fatigue syndrome (CFS) patients compared to age-, race-, and gender-matched controls. The CFS patient population was composed of rigorously screened civilian and Persian Gulf War ...

A literature review was conducted on the excess use of alcohol by methadone patients. Although the rate of alcohol abuse among methadone patients was found to be high (compared to general population estimates), the rate was comparable to individuals who engaged in risk-taking behaviors and individuals under stress, such as ...

OBJECTIVE: This report describes the unusual presentation of Clostridium difficile colitis in five patients with cystic fibrosis and the role of CT in first suggesting the correct diagnosis in this group of patients. Because of the absence of watery diarrhea and the presence of abdominal bloating and decreased stooling, cystic ...

STUDY OBJECTIVES: (1) To determine the relationship between IgE levels and the prevalence of allergic bronchopulmonary aspergillosis (ABPA) in cystic fibrosis (CF) patients, (2) to establish the usefulness of assessing atopy as an identifying risk factor for ABPA, (3) to evaluate the clinical course of patients receiving and not receiving ...

BACKGROUND: Right lower quadrant abdominal pain may pose a diagnostic problem in patients with cystic fibrosis. Abdominal ultrasound examination, used commonly in the diagnostic work-up, may reveal abnormalities of the appendix. However, interpretation of such findings is problematic, because the appearance of the gastrointestinal system during routine examination has not ...

PURPOSE: Previous studies have shown that the Scandipharm Flutter airway clearance device has increased the ability of cystic fibrosis patients to expectorate mucus. Studies that show the effects of the Flutter on pulmonary function among the pediatric cystic fibrosis patients are limited. Thus, we embarked on a study to assess ...

We present two types of "new" foreign body granulomas caused by the injectable aesthetic microimplants Bioplastique and Artecoll. We report the clinical and histopathologic findings in five patients who had undergone treatment of mainly facial contour abnormalities with one of these implants. All patients were women, and the mean age ...

Airway nitric oxide concentrations in patients with cystic fibrosis or primary ciliary dyskinesia syndrome have been shown to be lower than in healthy subjects. Decreased NO concentrations may contribute to impaired ciliary clearance, respiratory tract infections, or obstructive lung disease in these conditions. Nasal and exhaled NO concentrations were compared ...

BACKGROUND: Cystic fibrosis related diabetes (CFRD) has become increasingly common with the increasing longevity of patients with cystic fibrosis. The diagnosis of CFRD is important as its development may lead to a clinical deterioration which may be reversed with treatment. The oral glucose tolerance test (OGTT) is the method of ...

A retrospective analysis of files of patients with cystic fibrosis and pulmonary exacerbations was performed to investigate whether an individual dosage of tobramycin once established by serum level determination allows a reliable prediction of the adequate dosage in a consecutive exacerbation. All patients hospitalized > or = 2 times between ...

OBJECTIVES: To demonstrate the association between orthostatic intolerance and the chronic fatigue syndrome (CFS) in adolescents and to delineate the form that orthostatic intolerance takes in these children. STUDY DESIGN: We investigated the heart rate and blood pressure (BP) responses to head-up tilt (HUT) in 26 adolescents aged 11 to ...

Airways inflammation has been associated with increased nitric oxide (NO) in the exhaled breath. It was, therefore, questioned whether exhaled NO could act as an indicator of the severity of airways inflammation in the chronic suppurative lung diseases cystic fibrosis (CF) and bronchiectasis. NO levels in a single exhalation were ...

A decreased basal and/or stimulated activity of the G-protein/adenylyl cyclase (AC) system in peripheral blood cells has been proposed to represent a trait marker for alcoholism. However, AC activity may underlie state-dependent changes, which may impair a proper interpretation of AC activity measurements. Our study examined systematically the AC activity ...

Ciprofloxacin is effective for treating pulmonary infection in adult cystic fibrosis patients, and demonstrates excellent efficacy against Pseudomonas aeruginosa, but its use in paediatric cystic fibrosis patients has been limited because quinolone-induced cartilage toxicity has been observed in juvenile animals and has been considered a potential risk for children. Children ...

This study investigated the relations between nasal transepithelial electric potential difference (PD) and the phenotype and genotype of cystic fibrosis (CF) adult patients. Basal nasal PD was measured in 95 adult CF patients who were classified into three groups of nasal PD (expressed as absolute values) according to the 10th ...

PURPOSE: We compared MRI of the membranous labyrinth in patients with chronic non-neoplastic inner ear disease and MR signs of labyrinthine fibrosis and controls depending on their age, in order to establish whether there were any MR differences regarding patient age groups, control age groups and between the patients and ...

OBJECTIVES: The objective of this study was to assess the dye 2', 7'-dichlorofluorescein (DCF) assay in screening for alterations in polymorphonuclear cell (PMN) and monocyte (MC) oxidative burst of cystic fibrosis (CF) patients. STUDY DESIGN: 56 CF patients aged between 2 and 20 years were investigated. Purified cells were stimulated ...

BACKGROUND: Alpha-1 antitrypsin (alpha 1-AT) is the most abundant proteinase inhibitor within the lung. We have recently reported the surprising observation that cystic fibrosis patients with mild to moderate deficiency of alpha 1-antitrypsin have significantly better pulmonary function than non-deficient patients. This study may have been biased as it did ...

Fifty-five patients with oral submucosal fibrosis and an equal number of patients with no evidence of the disease were studied. All patients underwent upper gastrointestinal endoscopy and any abnormality was noted. Multiple oesophageal biopsies were obtained from the upper end of the oesophagus and from any endoscopically observed abnormality. The ...

Glucagon-producing neuroendocrine tumors typically present with a characteristic constellation of symptoms including necrolytic migratory erythema, non-insulin-dependent diabetes, weight loss, anemia, glossitis, and an increased thrombotic tendency. Most glucagonomas are solid and arise in the body or tail of the pancreas. We report two cases of cystic glucagonoma, one found incidentally ...

Carbohydrate-deficient transferrin (CDT) is a useful indicator of excessive alcohol consumption with higher sensitivity and specificity than other markers that are used. In the present study, CDT was analysed in 161 patients hospitalized in a surgical ward to evaluate whether history of drinking and chronic alcohol misuse are important determinants ...

BACKGROUND & AIMS: The diagnosis of hemochromatosis is now possible for C282Y homozygous patients using noninvasive molecular genetic tests. The aim of this study was to define noninvasive factors predictive of severe fibrosis (bridging fibrosis or cirrhosis) to avoid unnecessary liver biopsies in such patients. METHODS: Clinical and biological data ...

Little is known about the utility of collateral reports in substantiating self-report for individuals assessed in nonalcoholism treatment contexts. This study examined the concordance of 581 pairs of medical patient and collateral responses to a commonly used alcohol screening instrument, the CAGE Questions, as well as to reports of the ...

Improved survival has been associated with better nutritional status in patients with cystic fibrosis (CF). In this study we examined the relationship between nutritional state and other measures of clinical severity in adult patients with CF, attending a regional centre. Eighty-one patients (median age 21 years) were studied. Patients with ...

Chronic fatigue syndrome (CFS) has been widely studied by neuroimaging techniques in recent years with conflicting results. In particular, using single-photon emission computed tomography (SPECT) and perfusion tracers, hypoperfusion has been found in several brain regions, although the findings vary across research centers. The objective of this study was to ...

BACKGROUND: Patients with cystic fibrosis have an increased risk of hyperoxaluria, and of subsequent nephrocalcinosis and calcium-oxalate urolithiasis. Oxalate homoeostasis is controlled, in part, by the intestinal bacterium, Oxalobacter formigenes. The loss of this bacterium from the gut flora is associated with an increased risk of hyperoxaluria and calcium-oxalate urolithiasis. ...

A 53-year-old Japanese female, who had been suffering from idiopathic pulmonary fibrosis (IPF) for 3 years, was admitted with dyspnoea. The important laboratory finding on admission was a serum CA19-9 level of 649 U/mL. Despite steroid treatment for IPF, her respiratory condition gradually deteriorated with the increase of serum CA19-9 ...

Decreased bone density and increased risk of fractures are seen in patients with cystic fibrosis. Suboptimal vitamin D levels, nutrition problems, hypogonadism, inactivity, corticosteroid use, and cytokines may contribute to the low bone mass seen in these patients. Treatment recommendations must be individualized and may include nutrition, vitamin D, estrogen ...

Cystic fibrosis is characterized by the accumulation of thick viscous purulent secretions. Recombinant human deoxyribonuclease I (rhDNase) breaks down extracellular DNA, which contributes to the increased viscosity of sputum. A multinational, open-label study was conducted in 974 cystic fibrosis patients with moderate lung disease [forced vital capacity (FVC) 40-70% of ...

BACKGROUND: Irinotecan (CPT-11) is metabolized by esterase to form a SN-38, which is further conjugated by UGT1A1. Genetic polymorphism has been shown in a promoter region of UGT1A1 and is related to its activity. We investigated whether there might be an inter-individual difference in pharmacokinetics of SN-38 and its glucuronide, ...

The aim of this study was to assess the prevalence of ataxia of stance in different types of alcohol-dependent patients. Posturographic measurements were performed in 82 abstinent alcohol-dependent patients and 54 healthy controls in order to analyse postural control. According to Lesch and co-workers, alcohol dependence was classified as total ...

The purpose of this article is to review published studies on the efficacy of old and new mucus clearance techniques and to develop recommendations for different groups of patients. Mucus clearance is a problem in cystic fibrosis, bronchiectasis, and many other pulmonary conditions. Percussion and postural drainage (P & PD) ...

Nitric oxide (NO) can be detected in exhaled gas in human subjects. It is produced by nitric oxide synthase (NOS) and is rapidly metabolized to nitrite and nitrate (NO2/NO3). Exhaled NO is reported to be elevated in patients with asthma, bronchiectasis, or upper respiratory tract infection. Recent reports have shown ...

We investigated whether chronic fatigue syndrome (CFS) patients have physical and/or cardiovascular de-conditioning, in 273 CFS patients and 72 healthy controls. We used laboratory tests to assess haematological, biochemical, endocrinological and immunological systems. The cardiovascular system was assessed by echocardiography and carotid echography. Body composition was determined by dual energy ...

Hemoptysis is common in patients with cystic fibrosis (CF). Bleeding may vary in severity, ranging from minor blood-streaking of sputum to expectoration of significant quantities of blood. Major hemoptysis, defined as bleeding greater than 240 ml/24 h, represents a medical emergency. Bronchial artery embolization (BAE) is one of the treatment ...

The objective of this study was to evaluate the clinical, radiological investigation profiles, and ciliary function and ultrastructure in Chinese patients with Kartagener's syndrome (presence of dextrocardia, sinusitis and bronchiectasis). All patients with dextrocardia were assessed for the presence of sinusitis and bronchiectasis in our hospital network. Patients identified with ...

We examined the protein and fat nutritional status of 65 cystic fibrosis patients aged 4-26 y (x +/- SD: 11.2 +/- 5.6 y). Patients were treated with pancreatic enzyme extracts to improve nutrient absorption; in addition, most patients were supplemented with vitamins A and E. Results were compared with those ...

OBJECTIVE: To assess the outcome of epididymectomy as a method of treating epididymal pathology. PATIENTS AND METHODS: Forty-one patients who underwent epididymectomy between 1990 and 1995 were analysed retrospectively; their clinical records were reviewed and their satisfaction with the outcome and relief of symptoms assessed using a questionnaire and/or telephone ...

BACKGROUND: Hermansky-Pudlak syndrome is characterized by oculocutaneous albinism, a storage-pool deficiency, and lysosomal accumulation of ceroid lipofuscin, which causes pulmonary fibrosis and granulomatous colitis in some cases. All identified affected patients in northwest Puerto Rico are homozygous for a 16-bp duplication in exon 15 of a recently cloned gene, HPS. ...

A marked influx of inflammatory cells occurs into the airways of patients with cystic fibrosis (CF), which may contribute to the development of lung injury. Leukocyte-endothelial adhesion molecules play a crucial role in the recruitment of inflammatory cells, and soluble forms of these molecules have been shown to increase in ...

AIMS: To assess the value of opportunistic screening in Accident and Emergency (A&E) for patients with alcohol-related problems and provision of an intervention. DESIGN: Screening of A&E attendees for the purpose of recruitment to a randomized trial of a counselling intervention. SETTING: A General Hospital A&E department. PARTICIPANTS: All 17,000 ...

OBJECTIVES: Transforming growth factor beta1 (TGF-beta1) is a potent immunosuppressive cytokine that promotes fibrosis by enhancing the synthesis of extracellular matrix components. The repair process following lung allograft injury is due to rejection or infection replaces lung parenchyma by fibrotic tissue, leading to pulmonary dysfunction. The role of TGF-beta1 in ...

The diagnosis of cystic fibrosis (CF) is not always certain, despite extensive clinical evaluation, multiple sweat chloride tests, and genotype analysis. We hypothesized that nasal transepithelial potential difference measurements have a useful role in this situation. In 11 patients without an established diagnosis of CF, results of simultaneous nasal potential ...