Evaluation of Gastric Emptying in Cystic Fibrosis Using Bedside Ultrasonography.

OBJECTIVES: Although gastric emptying rate (GER) influences incretin response and nutrient absorption, both defective in people with cystic fibrosis (CF), there are few studies in this group, and the circadian rhythm is unknown. We employed an ultrasonographic technique to assess GER in people with CF following liquid and mixed meals and also evaluated the circadian rhythm, compared to normal controls. METHODS: Thirteen pancreatic-insufficient adults with CF and 10 healthy controls underwent a fasting oral glucose tolerance test (OGTT; liquid meal) and on a second visit underwent a fasting mixed meal test (MMT) thrice on the same day. GER was measured during the OGTT and each MMT using a 3.5-MHz abdominal transducer probe at baseline and 30, 60, 90, and 120 minutes. RESULTS: Controls had normal GER for both OGTT and MMT. Compared to controls, people with CF had delayed GER60 for OGTT (mean 46%), which increased with time (P = .001), and also delayed GER90 for MMT in the morning (mean 56%), afternoon (58%), and evening (59%). The GER in the control group was better at all time points throughout the day compared to patients with CF. CONCLUSIONS: This is the first study to use ultrasonography, an inexpensive bedside technique, to assess GER in adults with CF. For the first time, we have shown that adults with CF have delayed GER for both liquid and mixed meals, and this delay continues throughout the day.

Long-term effect of insulin treatment in cystic fibrosis-related diabetes.

BACKGROUND: Although insulin treatment confers short-term benefit in cystic fibrosis-related diabetes (CFRD), few studies have compared its long-term effect on the clinical outcome. OBJECTIVES: In this study, we aimed to investigate the long-term impact of insulin treatment on pulmonary function, nutritional status and hospital admissions in patients with CFRD. METHODS: We reviewed pulmonary function, body mass index (BMI) and hospital admissions 5 years before and 3 years after insulin therapy in 42 adult CFRD patients. RESULTS: Prior to treatment, over a period of 5 years, the annual rate of change in forced expiratory volume in 1 s (FEV(1)) was -3.2%, forced vital capacity (FVC) -2.5%, and BMI -0.07%. At treatment of CFRD (baseline), the mean FEV(1) was 51.6% predicted (range 24-96), FVC 66.4% (range 29-103) and BMI 19.5 (range 15.3-29.5). At 3 months following insulin treatment, there was a significant improvement in all parameters, which was maintained at 1 year for FEV(1) (55.1%; p < 0.002), 2 years for FVC (72.1%; p < 0.01) and at 3 years for BMI (20.4%; p < 0.002). After 3 months, FEV(1) declined at a rate similar to that before treatment (-3.2 vs. -3.1% per year; p = 0.77), such that the mean FEV(1) after treatment returned to pretreatment baseline values at 34 months. There was no difference in the number of hospital admissions with insulin treatment. CONCLUSIONS: Insulin enhances the nutritional state and temporarily improves pulmonary function in CFRD patients, on average delaying the decline in FEV(1) by 34 months.

Lessons to be learned: a case study approach. Bronchiolitis obliterans organising pneumonia: a reversible cause of respiratory failure.

A 70-year-old lady was admitted to hospital through the casualty department with suspected bilateral community acquired pneumonia. Although she received intravenous antibiotics, high concentrations of supplemental inhaled oxygen and continuous positive airway pressure therapy there was failure to respond. Abnormal levels of peripheral blood neutrophil and eosinophil counts as well as computerised tomography chest scan appearances suggested bronchiolitis obliterans organising pneumonia. The diagnosis was confirmed by a lung biopsy obtained by video-assisted thoracoscopy under general anaesthesia. She made a prompt and complete recovery with oral steroid treatment.

Long-term survival of patients with pulmonary disease undergoing coronary artery bypass surgery.

OBJECTIVES: We sought to investigate the long-term survival of patients with obstructive, restrictive and chronic obstructive pulmonary disease (COPD) as defined by the Global Initiative for Chronic Obstructive Lung Disease (GOLD). METHODS: A prospective database was retrospectively analysed and cross-correlated with the UK strategic tracking service to evaluate survival after primary coronary artery bypass grafts (CABG). Univariate and multivariate Cox regression analyses were performed. Three separate multivariate analyses were performed: COPD GOLD criteria for obstructive and/or restrictive lung disease, forced expiratory volume in 1 s (FEV1), forced vital capacity (FVC) and the FEV1/FVC ratio to investigate the effect of FEV1 and FVC individually. RESULTS: We analysed 13 337 primary CABG procedures. The median follow-up was 7 years. Univariate analysis demonstrated that obstructive (P < 0.0001), restrictive (P < 0.0001) and mixed obstructive and restrictive pulmonary disease (P < 0.0001), and COPD as defined by the GOLD criteria (P < 0.0001), are all significant factors determining long-term survival. Cox regression analysis identified age, diabetes, moderate LV, poor LV, peripheral vascular disease, dialysis, left internal mammary artery (LIMA) usage, EuroSCORE, cardiopulmonary bypass and creatinine kinase muscle-brain isoenzyme as significant factors in addition to pulmonary disease that determine long-term survival. Moderate and severe COPD defined by GOLD criteria were significant factors determining long-term survival, but mild COPD had no significant effect. Obstructive and restrictive lung disease were both significant factors determining long-term survival. Restrictive lung disease, however, carried a greater prognostic significance (higher hazard ratio 2.2 vs 1.6) than obstructive. LIMA utilization in patients with COPD was not associated with an increased intensive care unit stay, re-intubation rate or in-hospital mortality rate. CONCLUSIONS: Pulmonary disease is a significant factor determining long-term survival. Patients with severe COPD still have a relatively good long-term survival and should not be denied surgery. LIMA utilization in patients with COPD results in a significantly increased long-term survival, without an increased intensive care unit stay, re-intubation rate or in-hospital mortality rate.

Impact of chronic obstructive pulmonary disease severity on surgical outcomes in patients undergoing non-emergent coronary artery bypass grafting.

OBJECTIVES: Although the association between chronic obstructive pulmonary disease (COPD) and adverse surgical outcomes has been previously demonstrated, the impact of COPD severity on postoperative mortality and morbidity remains unclear. Our objective was to analyse the prognostic implication of COPD stages as defined by the Global Initiative for Chronic Obstructive Lung Disease. METHODS: Between September 1997 and April 2010, 13,638 patients undergoing first time isolated CABG were retrospectively reviewed, of whom 2421 patients were excluded due to lack of spirometry records or restrictive pattern on spirometry. The remaining 11,217 patients were divided into three groups: group 1 (including patients with normal spirometry and patients with mild COPD (FEV1/FVC ratio<70%, FEV1≥80% predicted), group 2 (moderate COPD: FEV1/FVC ratio<70%, 50%≤FEV1<80% predicted) and group 3 (severe COPD: FEV1/FVC ratio<70%, FEV1<50% predicted). Logistic regression was used to examine the effect of COPD severity on early mortality and morbidity, after adjusting for differences in patient characteristics. RESULTS: Early mortality in the three groups was 1.4, 2.9 and 5.7% respectively (P<0.001). Similarly, a consistent trend of increasing frequency of postoperative complications with advanced COPD stage was noted. On multivariate analysis, severe COPD was found to be significantly associated with early mortality [adjusted OR, 2.31 (95% CI) (1.23-4.36)], P=0.01. CONCLUSIONS: The severity of COPD as defined by spirometry can be a prognostic marker in patients undergoing CABG. Spirometric criteria may help refining currently used operative risk scores.

Empyema due to a highly transmissible Pseudomonas aeruginosa strain in an adult cystic fibrosis patient.

Chronic pulmonary infection with Pseudomonas aeruginosa occurs in up to 85 % of individuals with cystic fibrosis (CF) by the time they reach adulthood, and is the major cause of morbidity and mortality: nearly all patients die from progressive respiratory failure due to repeated pulmonary exacerbations. However, despite the predilection of this organism for the lungs of CF people, infection of the pleura is much less common and is not well described in the CF population. We describe what is believed to be the first case of pleural empyema due to a particularly pathogenic transmissible strain of P. aeruginosa (the Liverpool epidemic strain) in an adult CF patient.

Simultaneous bilateral spontaneous pneumothorax in an adult patient with cystic fibrosis.

Pneumothorax is a common complication in cystic fibrosis and is associated with worsening of lung function. However, bilateral simultaneous pneumothorax in cystic fibrosis is a rare condition. We describe the case of a 17-year-old female with cystic fibrosis who presented with spontaneous pneumothorax. Clinically, she presented right-sided chest pain and progressive breathlessness. The pneumothorax failed to resolve after the initial treatment (chest drainage). However, the patient was later successfully treated with additional chest drainage and talc pleurodesis. We also discuss the etiology and management of pneumothorax in patients with cystic fibrosis, since pneumothorax is associated with increased morbidity and mortality among such patients.

Management of cystic fibrosis related diabetes: a survey of UK cystic fibrosis centers.

INTRODUCTION: Cystic fibrosis related diabetes (CFRD), a poor prognostic factor in cystic fibrosis (CF), is an increasing problem and guidelines regarding its management have recently been published. However, the evidence base for CFRD screening and diagnosis is not comprehensive and its current management in the UK is unknown. We therefore conducted a questionnaire survey of all recognized UK CF centers to assess clinical practice and determine adherence to these recent recommendations. METHODS: A questionnaire survey (regarding screening, diagnosis, treatment and monitoring of CFRD) sent to all 45 recognized CF centers (>50 patients) in the UK. RESULTS: Completed questionnaires were returned by 37 centers (82%). Although 35 (95%) centers screened patients annually for CFRD, 12 (34%) used a single investigation whereas the remaining 23 (66%) used two or more methods. As regards diagnosis only 11 (30%) used the recommended combination of oral glucose tolerance test (OGTT) and serial glucose monitoring, with 18 (49%) using OGTT alone. Insulin was the preferred treatment of choice in 36 (97%) centers, and pediatric centers were less likely to use oral hypoglycemic agents (4/17 vs. 9/16; chi(2) = 3.6, P < 0.05). 29 (78%) centers carried out an annual diabetes review and in 34 (92%) patients were cared for jointly between the CF and endocrinology teams. Glycosylated hemoglobin and serial glucose monitoring were the most common investigations used to monitor glycemic control. CONCLUSIONS: This survey highlights the disparities in the management of CFRD with regards to screening and diagnostic practice, and poor adherence to national guidelines.

Impact of 18F-FDG PET scan on the prevalence of benign thoracic lesions at surgical resection / Impacto do estudo com 18F-FDG PET sobre a prevalência de achados de lesões torácicas benignas em ressecções cirúrgicas

OBJECTIVE: The main utility of 18-fluorodeoxyglucose positron emission tomography (FDG-PET) lies in the staging of lung cancer. However, it can also be used to differentiate indeterminate pulmonary lesions, but its impact on the resection of benign lesions at surgery is unknown. The aim of this study was to compare the prevalence of benign lesions at thoracotomy carried out for suspected lung cancer, before and after the introduction of PET scanning in a large thoracic surgical centre. MATERIALS AND METHODS: We reviewed our prospectively recorded surgical database for all consecutive patients undergoing thoracotomy for suspected or proven lung cancer and compared the prevalence of benign lesions in 2 consecutive 2-year groups, before (group I) and after (group II) the introduction of FDG-PET scan respectively. RESULTS: Surgical resection was performed on 1233 patients during the study period. The prevalence of benign lesions at surgery in groups I and II was similar (44/626 and 41/607, both 7 percent), and also in group II between those who underwent FDG-PET scan and the remainder (21/301 and 20/306 respectively, both 7 percent). In group II, of the 21 patients with benign lesions, who underwent FDG-PET, 19 had a false positive scan (mean standardised uptake value 5.3 [range 2.6-12.7]). Of these, 13 and 4 patients respectively had non-diagnostic bronchoscopy and percutaneous transthoracic lung biopsy pre thoracotomy. There was no difference in the proportion of different benign lesions resected between group I and those with FDG-PET in group II. CONCLUSION: The introduction of FDG-PET scanning has not altered the proportion of patients undergoing thoracotomy for ultimately benign lesions, mainly due to the avidity for the isotope of some non-malignant lesions. Such false positive results need to be considered when patients with unconfirmed lung cancer are contemplated for surgical resection.

OBJETIVO: A principal utilidade da tomografia por emissão de pósitrons com 18-fluordeoxiglicose (FDG-PET) está no estadiamento do câncer de pulmão. Porém, ela também pode ser utilizada para diferenciar lesões pulmonares indeterminadas, mas seu impacto na ressecção cirúrgica de lesões benignas é desconhecido. O objetivo deste estudo foi comparar a prevalência de lesões benignas em toracotomias feitas por suspeição de câncer de pulmão, antes e após a introdução do FDG-PET, em um centro de referência de cirurgia torácica. MATERIAIS E MÉTODOS: Os autores analisaram, prospectivamente, uma base de dados cirúrgicos de todos os pacientes consecutivos submetidos a toracotomia por câncer de pulmão suspeito ou comprovado e compararam a prevalência de lesões benignas em dois grupos ao longo de dois anos consecutivos, respectivamente antes (grupo I) e depois (grupo II) da introdução da FDG-PET. RESULTADOS: Ressecção cirúrgica foi feita em 1.233 pacientes durante o período do estudo. A prevalência de lesões benignas na cirurgia nos grupos I e II foi similar (44/626 e 41/607, ambas correspondendo a 7 por cento), e também no grupo II, entre aqueles submetidos a FDG-PET e os restantes (21/301 e 20/306 respectivamente, ambos correspondendo a 7 por cento). No grupo II, dos 21 pacientes com lesões benignas submetidos a FDG-PET, 19 tiveram um estudo falso-positivo (valor médio padrão de captação 5.3 [faixa 2.6-12.7]). Desses, respectivamente 13 e 4 pacientes tiveram broncoscopia não diagnóstica e biópsia transtorácica percutânea de pulmão antes da toracotomia. Não houve diferença na proporção de lesões benignas diferentes ressecadas entre o grupo I e aqueles submetidos a FDG-PET no grupo II. CONCLUSÃO:A introdução da FDG-PET não alterou a proporção de pacientes submetidos a toracotomia por lesões benignas, principalmente devido à avidez pelo isótopo de algumas lesões não malignas. Tais resultados falsos-positivos devem ser considerados nos casos em que se contempla a possibilidade de ressecção cirúrgica em pacientes com câncer de pulmão não confirmado.

Simultaneous bilateral spontaneous pneumothorax in an adult patient with cystic fibrosis / Pneumotórax espontâneo simultâneo bilateral em um paciente adulto com fibrose cística

Pneumothorax is a common complication in cystic fibrosis and is associated with worsening of lung function. However, bilateral simultaneous pneumothorax in cystic fibrosis is a rare condition. We describe the case of a 17-year-old female with cystic fibrosis who presented with spontaneous pneumothorax. Clinically, she presented right-sided chest pain and progressive breathlessness. The pneumothorax failed to resolve after the initial treatment (chest drainage). However, the patient was later successfully treated with additional chest drainage and talc pleurodesis. We also discuss the etiology and management of pneumothorax in patients with cystic fibrosis, since pneumothorax is associated with increased morbidity and mortality among such patients.

O pneumotórax é uma complicação comum na fibrose cística e está associado com a piora da função pulmonar. Entretanto, o pneumotórax simultâneo bilateral na fibrose cística é um achado raro. Nesse artigo é descrito o caso de uma paciente de 17 anos com fibrose cística que cursou com pneumotórax espontâneo simultâneo bilateral. Clinicamente ela apresentou dor torácica à direita e dificuldade respiratória progressiva. Embora o pneumotórax não tenha respondido bem ao tratamento inicial (drenagem torácica), ela foi posteriormente tratada com drenagem e pleurodese com talco, com sucesso. São discutidos também a etiologia e a conduta nesta condição, que está associada com o aumento da morbidade e mortalidade.