Treatment compliance in children and adults with Cystic Fibrosis

ObjectivesTo determine treatment compliance and how compliance was perceived by patients, parents and by a multidisciplinary team in Cystic Fibrosis (CF) patients. Also to analyse the relative importance given to each of the prescribed treatments, reasons for non-adherence and to investigate possible predictors of therapeutic compliance.Patients and methods34 CF patients (21 females), aged between 1.6 and 40.6 years, attending an outpatient CF clinic. Design: cross-sectional. A self-administered questionnaire was given to all patients whilst attending a programmed visit which was used to determine compliance to different treatments (physiotherapy, nutritional supplements, respiratory and digestive medications). Patients were subjectively classified as compliant or non-compliant by medical staff involved in their care.ResultsTreatment compliance was greater for digestive (88.2%) and respiratory medication (61.8%), compared to physiotherapy (41.2%) or nutritional supplements (59%). CF patients considered digestive medication indispensable (94.1%), compared to nutritional supplements (44.1%). Whilst 26.4% of CF patients considered that respiratory medications or nutritional supplements influenced little or nothing in their quality of life. Comparing age groups younger patients were found to be more compliant (10.4 vs. 20.5 years p = 0.008) and had less severe disease (Shwachman score 83.2 vs. 73.9 p = 0.048).ConclusionsCF patients had greater treatment adherence when prescribed digestive and respiratory medications, compared to physiotherapy or nutritional supplements. Therapeutic adherence was found to worsen with age and disease severity, however improved with treatments which were perceived by patients as more important or had a greater influence in their quality of life.     

Effects of nutritional status and dietetic interventions on survival in Cystic Fibrosis patients before and after lung transplantation

BackgroundThis study retrospectively investigated nutritional status, dietetic intervention and intake in Cystic Fibrosis (CF) patients before and after lung transplantation (LTX).MethodsBody Mass Index (BMI), Fat Free Mass Index (FFMI) and nutritional intake were retrieved from 75 out-patients aged 15–53 years. Patients were seen every 3–4 months during the waiting list time (range 0–81 months) and up to 116 months after LTX. Survival was measured in months.ResultsThe median BMI at baseline was 19.2 kg/m² (range: 15.3 to 28.4 kg/m²) with 29 patients (39%) below ≤ 18.5 kg/m². FFMI (measured in 65 patients) had a median of 15.2 kg/m² (range: 11.1 to 22.4 kg/m²) with 39 patients (60%) ≤ 16.7 kg/m² (men) or ≤ 14.6 kg/m² (women). Median energy intake was 2800 kcal, 239 kcal higher than the estimated energy requirement. However, 8 patients consumed ≥ 500 kcal less than recommended. Protein intake was 104 (range 60–187) g or 1.9 g/kg per day. Despite dietetic intervention with oral nutritional supplements (ONS) (36 patients), tube feeding (12 patients), or both (13 patients), BMI and FFMI hardly improved pre-LTX. LTX was performed in 51 patients (68%); 10 patients died during follow-up, median survival time was 41 months. A BMI ≤ 18.5 kg/m² was more prevalent in patients who died before LTX (6/9) or who died after LTX (4/10) than in patients who were still alive on the waiting list (5/15) or who survived LTX (14/41). Results for FFMI were comparable. From 6–12 months post-LTX, BMI and FFMI markedly improved, especially in underweight patients.ConclusionA BMI ≤ 18.5 kg/m² and an FFMI ≤ 16.7 kg/m² (men) or ≤ 14.6 kg/m² (women) appears to impair survival in LTX candidates with CF. Patients maintained a low body weight before LTX. After LTX weight gain is achieved.     

Pancreaticoduodenectomy with pancreaticogastrostomy: assessment of patients' nutritional status, quality of life and pancreatic exocrine function

BACKGROUND: The changes in digestive function of patients with pancreaticoduodenectomy (PD) and pancreaticogastrostomy reconstruction have not been well-documented. The present study sought to assess the nutritional status, quality of life and pancreatic exocrine function in this group of patients. METHODS: The study group consisted of 11 PD with pancreaticogastrostomy patients. The control group consisted of 11 consecutive patients who had subtotal gastrectomy (SG) for distal stomach tumours. RESULTS: The median ages for the PD and SG groups were 57 and 59 years, respectively. The median intervals between surgery to assessment were 68 and 60 weeks, respectively. The PD group attained a mean of 92.7% of their pre-surgery weight compared to 91.3% in the SG group. Both groups had a comparable gastrointestinal quality of life index and Visick scale scores. Exocrine insufficiency using the faecal chymotrypsin test was present in 36% of patients with PD. None of the patients in the SG group had exocrine insufficiency. CONCLUSION: Pancreaticoduodenectomy patients had a significant occurrence of pancreatic exocrine insufficiency compared to the SG group. But patients with PD and pancreaticogastrostomy reconstruction maintained a nutritional status and quality of life similar to those with curative SG for stomach malignancy. Apart from exocrine insufficiency, the concomitant gastrectomy in the PD group is an important factor responsible for their inability to gain weight.     

Inflammatory and immunological biomarkers are not related to survival in adults with Cystic Fibrosis

BackgroundChronic Pseudomonas aeruginosa pulmonary infection is associated with a decline in lung function and reduced survival in people with Cystic Fibrosis (CF). Damaging inflammatory and immunological mediators released in the lungs can be used as markers of chronic infection, inflammation and lung tissue damage.MethodsClinical samples were collected from CF patients and healthy controls. Serum IgG and IgA anti-Pseudomonas antibodies, sputum IL-8 and TNFα, plasma IL-6 and urine TNFr1 were measured by ELISA. Sputum neutrophil elastase (NE), cathepsin S and cathepsin B were measured by spectrophotometric and fluorogenic assays. The relationship between IgG and IgA, inflammatory mediators and long-term survival was determined.ResultsIgG and IL-6 positively correlated with mortality. However, multivariate analysis demonstrated that after adjusting for FEV₁, IgG was not independently related to mortality. A relationship was observed between IgG and IL-6, TNFα, TNFr1 and between IgA and IL8, cathepsin S and cathepsin B.ConclusionsThese data indicate that biomarkers of inflammation are not independent predictors of survival in people with CF.     

Time course and recovery of arterial blood gases during exacerbations in adults with Cystic Fibrosis

IntroductionHypoxia and hypercapnia are closely linked to morbidity and mortality in patients with Cystic Fibrosis (CF). The aims of this study were to describe the changes in blood gases during and following an acute pulmonary exacerbation in adults with CF.MethodsWe performed a prospective observational study of patients with CF admitted for management of an acute exacerbation. Blood gas and spirometric analysis was performed on admission, throughout the treatment period, and 31 days after discharge (day 45).ResultsAt presentation, eight of nineteen patients had evidence of either hypoxia (PaO₂ < 8 kPa) and/or hypercapnia (PaCO₂ > 6.6 kPa). Blood gas parameters stabilized following two weeks of intravenous antibiotic therapy, with little difference evident in between treatment completion and subsequent review following discharge. Hypercapnia reversed in three patients, with persistent hypercapnia evident in two patients.ConclusionIn our study group, hypoxemia and hypercapnia were frequently observed at presentation of the acute exacerbation. Blood gases stabilized following two weeks of intravenous antibiotic therapy, with arterial PCO₂ one month following hospital discharge generally similar to that at time of discharge.     

胰腺癌患者术后的营养状况及特征分析

目的考察胰腺癌患者术后营养状况并对相关特征进行分析。 方法选取2015年1月至2018年6月手术治疗的肿瘤患者149例进行回顾性研究,其中胰腺癌患者105例归为胰腺癌组,其他类型肿瘤患者44例为对照组。应用SPSS 19.0统计学软件对数据进行统计学分析,生化指标、人体测量指标、营养学相关指标等计量资料采用( ±s)表示,组间比较采用两独立t或t’检验。性别、胰腺癌类型、首发症状等计数资料组间比较采用χ²检验。以P<0.05表示差异具有统计学意义。 结果105例胰腺癌患者中,首发症状占比最高的为上腹痛(51.43%)、其次为腹胀(12.38%),第三为黄疸(11.43%);与对照组相比,胰腺癌组患者的上臂中点周径和三头肌皮褶厚度显著增加,而握力显著降低(P<0.05);与对照组相比,胰腺癌组患者的Hb、ALB、TP和PAB含量均显著降低,而TG和TC水平显著提高(P<0.05);与营养良好组相比,营养不良组患者的住院天数及住院费用均显著增加,而KPS得分显著降低,差异有统计学意义(P<0.05)。 结论相比与其他普外科恶性肿瘤,胰腺癌患者的营养不良发生率高,蛋白水平低,预后较差。     

Altered Calcium Homeostasis in Adults with Cystic Fibrosis

Bone mineral density (BMD) in cystic fibrosis (CF) patients falls progressively below normal with advancing age, in part due to steroid administration, low levels of sex hormones, chronic inflammatory disease, physical inactivity, and chronic malabsorption of calcium and/or vitamin D. The purpose of this study was to compare the fractional absorption of ⁴⁵Ca and urinary excretion of calcium in CF subjects and normal controls following a high-calcium breakfast containing ⁴⁵Ca. Seven young men and 5 young women with CF with pancreatic insufficiency were studied on two separate occasions, with and without administration of pancreatic enzymes. Eleven healthy young adults with normal BMD measurements served as controls. Mean T-scores at the lumbar spine and femur were significantly lower in the CF subjects (p<0.002). Following baseline, fasting collections, timed serum and urine samples were obtained for 5 h after the meal. Fractional absorption (FA) of ⁴⁵Ca was estimated by the method of Marshall and Nordin. At baseline, CF subjects had lower mean serum 25-hydroxyvitamin D, calcium and albumin values (p<0.03 for each), slightly, but not significantly (p= 0.12), lower albumin-corrected calcium values, equivalent serum 1,25-dihydroxyvitamin D values and a trend toward a higher mean serum parathyroid hormone (PTH) value (p= 0.10). Without pancreatic enzymes, CF subjects showed significantly impaired calcium absorption (5 h FA: 11.8 ± 0.5 for controls vs 8.9 ± 0.2 for CF subjects, p= 0.02) and excretion (4 h excretion: 0.20 ± 0.08 mg Ca/mg creatinine for controls vs 0.16 ± 0.09 mg Ca/mg for CF subjects, p= 0.025). Addition of pancreatic enzymes did not fully compensate for this deficiency. In addition, CF patients had higher serum PTH values after a high-calcium meal (p= 0.03), suggesting mild secondary hyperparathyroidism. Altered calcium homeostasis is likely to be a factor in the development of bone disease in CF patients. Received: 9 July 1998 / Accepted: 27 December 1998     

Influence of nutritional status in lung transplant recipients

OBJECTIVE: To assess the influence of nutritional status on mortality in lung transplant (LT) recipients. METHODS: A total of 114 patients underwent lung-transplantation between January 1999 and June 2005. Mortality after lung transplantation was examined based upon body mass index (BMI) categories: BMI < 18.5 kg/m(2) (group I); BMI 18.5 to 24.9 kg/m(2) (group II, reference group); BMI 25 to 27.5 kg/m(2) (group III); and BMI > 27.5 kg/m(2) (group IV). Levels of serum albumin and serum prealbumin were determined before transplantation. We constructed a Cox proportional hazards model for overall survival considering mortality as the outcome. The final model was adjusted by age. RESULTS: We analyzed 114 transplants in 112 patients. The population included 44 single and 70 bilateral LTs. Mean recipient age was 53.9 +/- 10.9 years. The hazard ratio of the risk of death in group IV was higher than in the reference group (hazard ratio: 3.55, 95% CI: 1.19 to 10.66; P = .024). Serum prealbumin 27.5 kg/m(2) was a predictor of increased mortality after transplantation. Low pretransplant prealbumin levels in lung recipients were associated with mortality after transplantation.     

不同配方肠内营养支持对胃癌病人术后营养的作用

目的:评定不同配方肠内营养支持对胃癌病人围手术期营养状况的作用。方法:随机将45例进展期胃癌根治术病人分成对照组、实验A组和实验B组,各组15例。对照组为普通输液组;实验A组肠内营养液不含谷氨酰胺;实验B组每升肠内营养液含谷氨酰胺13.0g。实验组术后第2天起给予肠内营养104.5 kJ/(kg.d),连续7 d,分别测定各项营养指标和免疫指标,检测血浆谷胱甘肽(GSH)和血清氨基酸浓度,观察临床转归。结果:除对照组出现2例肺部感染,其余均未发生严重并发症。实验组病人肱三头肌皮皱厚度(TSF)、上臂肌围(AC)和转铁蛋白(TRF)等营养指标下降明显减少,实验B组TSF和TRF更接近术前水平;术后第7天,实验B组病人血浆GSH浓度明显上升(P=0.035),与对照组(P=0.000)、实验A组(P=0.000)呈显著差异;实验B组CD4+和CD4+/CD8+明显高于对照组。实验B组患者血清丝氨酸(P=0.023)、精氨酸(P=0.031)、缬氨酸(P=0.018)、赖氨酸(P=0.034)和蛋氨酸(P=0.030)均有明显升高。结论:胃癌病人术后早期给予特殊肠内营养支持能有效提高GSH水平,增加CD4+细胞比例,显著增加体内必需氨基酸和半必需氨基酸浓度,改善机体营养和免疫状况。     

Long-term impact of liver transplantation on respiratory function and nutritional status in children and adults with cystic fibrosis

Early liver transplant (LT) has been advocated for patients with cystic fibrosis liver disease (CFLD) and evidence of deterioration in nutritional state and respiratory function to prevent further decline. However, the impact of single LT on long-term respiratory function and nutritional status has not been adequately addressed. We performed a retrospective analysis of the outcomes of 40 (21 adult/19 pediatric) patients with CFLD transplanted between 1987 and 2009 with median follow-up of 47.8 months (range 4-180). One and five-year actuarial survival rates were 85%/64% for adult and 90%/85% for pediatric LT cohorts, respectively. Lung function remained stable until 4 years (FEV(1) % predicted; pretransplant 48.4% vs. 45.9%, 4 years posttransplant) but declined by 5 years (42.4%). Up to 4 years posttransplant mean annual decline in FEV(1) % was lower (0.74%; p = 0.04) compared with the predicted 3% annual decline in CF patients with comorbidity including diabetes. Number of courses of intravenous antibiotics was reduced following LT, from 3.9/year pretransplant to 1.1/year, 5 years posttransplant. Body mass index was preserved posttransplant; 18.0 kg/m(2) (range 15-24.3) pretransplant versus 19.6 kg/m(2) (range 16.4-22.7) 5 years posttransplant. In conclusion, LT is an effective treatment for selected patients with cirrhosis due to CFLD, stabilizing aspects of long-term lung function and preserving nutritional status.     

术前肠内营养对食管癌病人术后营养状况的影响分析

目的研究术前肠内营养支持对食管癌病人术后营养状况的影响。方法对2008年1月至2008年12月河北医科大学附属唐山工人医院40例食管癌的临床资料进行分析。将40例食管癌病人随机分为两组,对照组20例,行术后早期肠内营养(EEN);治疗组20例,在EEN基础上术前口服能全素,肠内营养支持。所有病人均于术前1日及术后第8日测量体重,抽取静脉血测定血红蛋白及血清白蛋白含量,并于第8日留24h尿测尿素氮,计算氮平衡。结果治疗组与对照组比较,术后第8日体重变化差异无统计学意义(P>0.05);血红蛋白、血清白蛋白含量及氮平衡比较,治疗组明显优于对照组,差异有统计学意义(P<0.05)。结论术前肠内营养支持能明显改善食管癌病人术后的营养状况。     

Review of Gastrointestinal Motility in Cystic Fibrosis

Gastrointestinal manifestations in patients with cystic fibrosis (CF) are extremely common and have recently become a research focus. Gastrointestinal (GI) dysfunction is poorly understood in the CF population, despite many speculations including the role of luminal pH, bacterial overgrowth, and abnormal microbiome. Nevertheless, dysmotility is emerging as a possible key player in CF intestinal symptoms. Our review article aims to explore the sequelae of defective cystic fibrosis transmembrane conductance regulator (CFTR) genes on the GI tract as studied in both animals and humans, describe various presentations of intestinal dysmotility in CF, review newer diagnostic motility techniques including intraluminal manometry, and review the current literature regarding the potential role of dysmotility in CF-related intestinal pathologies.     

Importance of bacteriology in upper airways of patients with Cystic Fibrosis

BackgroundRecently the influence of the upper airways (UAW) on the general health of a patient with Cystic Fibrosis (CF) has been acknowledged. Surprisingly the microbiology of the upper compartment of the airways receives barely any attention in the treatment of CF. The aim of the present study was to investigate the microbiology of the upper airways in adult patients with CF, to correlate these findings with cultures from the lower airways (LAW) and with clinical characteristics.MethodsIn this cross-sectional study bacteriological and clinical data were gathered from 104 adult patients with CF. UAW samples for culture were collected by nasal lavage and middle meatal swabs; LAW cultures were performed on expectorated sputum or cough swabs. Each patient performed the Rhinosinusitis Outcome Measure (RSOM-31).ResultsIn 72 patients (69.2%) UAW cultures yielded microorganisms other than normal nasal flora and in 50 patients (48.1%) Pseudomonas aeruginosa grew from the UAW cultures. Similarity between UAW and LAW cultures was determined in 50.0% of these 72 patients. In 3 patients P. aeruginosa was cultured from the UAW after successful eradication of P. aeruginosa from the LAW. P. aeruginosa in the UAW did not influence symptoms of sinonasal disease compared to other microorganisms.ConclusionsComparison of UAW and LAW cultures in adult patients with CF showed one or more concordant microorganism in 50.0% of the patients. P. aeruginosa was most frequently cultured from the UAW. P. aeruginosa can be cultured from the UAW after eradication therapy which may suggest persistence of P. aeruginosa in the UAW. We feel this is may be a motive to include the UAW in eradication therapy in Cystic Fibrosis.