ECFS best practice guidelines: the 2018 revision

Developments in managing CF continue to drive dramatic improvements in survival. As newborn screening rolls-out across Europe, CF centres are increasingly caring for cohorts of patients who have minimal lung disease on diagnosis. With the introduction of mutation-specific therapies and the prospect of truly personalised medicine, patients have the potential to enjoy good quality of life in adulthood with ever-increasing life expectancy. The landmark Standards of Care published in 2005 set out what high quality CF care is and how it can be delivered throughout Europe. This underwent a fundamental re-write in 2014, resulting in three documents; center framework, quality management and best practice guidelines. This document is a revision of the latter, updating standards for best practice in key aspects of CF care, in the context of a fast-moving and dynamic field.In continuing to give a broad overview of the standards expected for newborn screening, diagnosis, preventative treatment of lung disease, nutrition, complications, transplant/end of life care and psychological support, this consensus on best practice is expected to prove useful to clinical teams both in countries where CF care is developing and those with established CF centres. The document is an ECFS product and endorsed by the CF Network in ERN LUNG and CF Europe.     

Renal endothelin receptor type B upregulation in rats with low or high renin hypertension

OBJECTIVES: To evaluate the role of endothelin-1 (ET-1) in hypertension, we investigated density and distribution of ETA and ETB receptors in hearts and kidneys of deoxycorticosterone acetate (DOCA)-salt and 1 kidney -- 1 clip (1K1C) hypertensive rats. METHODS: Five groups of uninephrectomized Wistar rats were put on a low salt diet. Three groups of rats drank tap water and two groups received saline. One group of each regimen received DOCA subcutaneously and two corresponding groups without DOCA served as controls. The fifth group of rats had the renal artery clipped to induce 1K1C hypertension. At 6 weeks, mean arterial pressure (MAP) was recorded and membrane binding assays using 125I-ET-1 were carried out. RESULTS: MAP was increased from control 122 +/- 3 to 155 +/- 6 and 218 +/- 11 mmHg in DOCA-salt and 1K1C rats, respectively, and cardiac weight index was increased. ETA receptors were predominantly expressed in the heart, whereas ETB receptors were predominant in the kidney. In the kidneys, the density of the ETB receptor subtype was upregulated in DOCA-salt and 1K1C rats from 160 +/- 8 to 217 +/- 12 and 190 +/- 2 fmol/mg (P < 0.05), respectively, and ETA tended to be downregulated (P = 0.057). Plasma renin activity was decreased in DOCA-salt rats from 17 +/- 3 to 0.17 +/- 0.01 ng/ml per h and increased in 1K1C rats on low salt diet to 30 +/- 5 ng/ml per h. CONCLUSIONS: Since ETB is the predominant endothelin receptor in the kidneys, upregulation of the ETB receptor mediating vasodilation and downregulation of the ETA receptor mediating vasoconstriction would be compatible with a mainly renal counter-regulatory effect of endothelin-1 to hypertension. Both low and high renin models of hypertension may be affected.     

Influence of Rapid Fluid Loading on Airway Structure and Function in Healthy Humans

BackgroundThe present study examined the influence of rapid intravenous fluid loading (RFL) on airway structure and pulmonary vascular volumes using computed tomography imaging and the subsequent impact on pulmonary function in healthy adults (n = 16).Methods and ResultsTotal lung capacity (ΔTLC = ?6%), forced vital capacity (ΔFVC = ?14%), and peak expiratory flow (ΔPEF = ?19%) decreased, and residual volume (ΔRV = +38%) increased post-RFL (P < .05). Airway luminal cross-sectional area (CSA) decreased at the trachea, and at airway generation 3 (P < .05), wall thickness changed minimally with a tendency for increasing in generation five (P = .13). Baseline pulmonary function was positively associated with airway luminal CSA; however, this relationship deteriorated after RFL. Lung tissue volume and pulmonary vascular volumes increased 28% (P < .001) post-RFL, but did not fully account for the decline in TLC.ConclusionsThese data suggest that RFL results in obstructive/restrictive PF changes that are most likely related to structural changes in smaller airways or changes in extrapulmonary vascular beds.     

Revascularization for chronic critical lower limb ischemia in octogenarians is worthwhile

OBJECTIVE: Advanced age is considered a relative contraindication for surgical revascularization in patients with peripheral arterial occlusive disease. Our aim was to analyze the usefulness of endovascular and surgical revascularization in patients older than 80 years with chronic critical leg ischemia (CLI). Our hypothesis was that the clinical benefit of lower extremity revascularization is limited in octogenarians. METHODS: This was a prospective cohort study with a 1-year follow-up. Subjects included a consecutive series (January 1999 to June 2004) of patients presenting with CLI. Revascularization cohorts were either open surgical or endovascular with conservatively treated patients as a reference group. Prospective follow-up occurred after 30 days and 2, 6, and 12 months. The primary end point was sustained clinical success, defined as a categorical upward shift in clinical symptoms according to Rutherford, without major amputation and without the need for repeated target extremity revascularization (TER). Secondary clinical success was defined accordingly, including repeated TER. Mortality, major amputation, and TER were separately calculated end points. All results were stratified for age categories of nonoctogenarians (<80 years) and octogenarians (> or =80 years). Cumulative outcome was determined by the Kaplan-Meier method, and differences were assessed by log-rank tests. Multivariable analysis was performed by using Cox proportional regression. RESULTS: A total of 376 patients (158 women; mean age, 75.8 +/- 10.7 years) with 416 critically ischemic limbs were analyzed. Overall, 150 patients (39.9%) were older than 80 years, and 85 limbs were treated surgically (26 octogenarians; 30.6%), 207 limbs (96 octogenarians; 46.4%) were treated by endovascular means, and 124 limbs (45 octogenarians; 36.3%) were treated conservatively, including delayed revascularization procedures. Both sustained and secondary clinical success rates, as well as limb salvage rates, were higher in the revascularization cohorts as compared with conservatively treated patients, regardless of age category (P < .001, P < .001, and P = .006, respectively, by Cox proportional hazard model). Mortality was significantly higher in octogenarians (P = .006 by Cox proportional hazard model), particularly within 30 days after surgical revascularization (hazard ratio, 5.35; 95% confidence interval, 1.15-24.9). Patient age category did not affect the rate of major amputations or TER. CONCLUSIONS: Individually tailored revascularization improves the outcome of CLI in octogenarians as well as in nonoctogenarians; even so, endovascular revascularization should be preferred in octogenarians because of the higher mortality associated with surgery.     

Intermethod agreement and interobserver correlation of radiologic acromiohumeral distance measurements

The acromiohumeral distance (AHD) measured on conventional, true anteroposterior radiographs taken with the patient standing and the arm held in neutral rotation can predict the presence or absence of a large, chronic rotator cuff tears, and narrowing of the acromiohumeral distance can predict irreparability of rotator cuff tears. The relation between AHD measured on conventional radiographs and the integrity of the rotator cuff tendons is well known. Conversely, the correlation between the AHD on conventional radiographs and that measured on magnetic resonance imaging (MRI) or computed tomography (CT) scans is unknown. It has also not been determined which method correlates better with fatty infiltration of the rotator cuff muscles and might thereby be a better prognostic value for the outcome of rotator cuff repair. Conventional radiographs, plus MRI and or CT studies taken at the same time of patients with rotator cuff tears, shoulder instability, primary glenohumeral osteoarthritis, or idiopathic frozen shoulder, were randomly selected, and the AHD was measured both on conventional radiographs and on MRI or CT. The values were correlated with the stage of fatty degeneration of the rotator cuff muscles in the cases of rotator cuff tears. The AHD on conventional radiographs was larger than on MRI and CT, and correlation of the values measured (conventional radiography vs MRI and CT) could be demonstrated. For rotator cuff diseases, a significant but moderate correlation (r = 0.6; P < .05) between conventional and MRI was identified, and the conventional values were approximately 0.6 mm greater than the corresponding MRI values. It is, therefore, possible to use the AHD read on MRI or CT studies for prognostic reasons, provided the values obtained are converted accordingly and remembering that the correlation of the values is only moderate.