Augmentation Therapy for Alpha-1 Antitrypsin Deficiency: Patient Experiences With Self-Infusion, Home Providers, and Clinics.

Care of Peripheral Venous Catheter Sites: Advantages of Transparent Film Dressings Over Tape and Gauze

Alpha 1 antitrypsin (A1AT) is the major plasma serine protease inhibitor that is produced in liver cells. A1AT deficiency is recognized globally as a common genetic cause of liver disease in children, which results from mutations in the SERine Protease INhibitor A1 (SERPINA1) gene. The importance of A1AT deficiency in Viet Nam is unclear. The aim of this study was to determine the A1AT variants present in paediatric patients with liver diseases in order to clarify whether A1AT deficiency is present in Viet Nam. A1AT studies were carried out in 130 children with liver disease of indeterminate aetiology. A1AT levels were determined by immunoturbidimetry. Phenotype analysis of A1AT was performed by isoelectric focusing (IEF) in all patients. Genotype analyses to determine A1AT mutations were performed by direct sequencing. We identified a rare variant of A1AT named Zbristol. The Zbristol appeared to be deficient in the plasma to about the same degree as the PI S protein resulting in low concentration of A1AT in one of these two Vietnamese patients. No other deficient A1AT allele was detected, although 11 patients (8.5%) showed a reduced serum concentration of A1AT. These are the first two cases of a rare A1AT deficiency allele to be found in Viet Nam clearly inferring that A1AT deficiency is not just a disease of Caucasians. As such, the laboratory diagnosis of A1AT deficiency including A1AT concentration determination and phenotype and genotype testing should form part of the routine differential diagnosis of paediatric liver disease of indeterminate aetiology in Vietnamese patients.

With new patient-centric and scientific networks being created, Prof Chorostowska-Wynimko explored how these initiatives, such as the European Alpha-1 Research Collaboration (EARCO) and the European Reference Network-LUNG Alpha1 Antitrypsin Deficiency (ERN-LUNG AATD Core Network), will help to advance the management of alpha 1-antitrypsin deficiency (AATD) patients. EARCO plans to create a registry to gather information from centres across Europe and ERN-LUNG AATD plans to ensure highly specialised healthcare for AATD patients, including reliable AATD diagnostics across European laboratories. Explaining in more detail the plans for the new EARCO registry, Dr Barrecheguren argued the case for another AATD registry to gather large-scale data that clinical trials cannot provide. She provided an overview of the new EARCO prospective follow-up registry, to be launched next year, which will integrate existing national AATD registries, enhance long-term follow-up and quality of data, and facilitate research and quality improvements across healthcare systems. Discussing one of the first initiatives of the ERN-LUNG AATD Core Network, Dr Ferrarotti explored how to align AATD testing across Europe with the creation of European LAB-NET, an initiative first involving six European centres that will co-operate to collect, develop, verify, and make reference materials available for molecular and biochemical tests to correctly diagnose AATD and provide quality control in the laboratory diagnosis. Dr Greulich reported on a post-hoc pooled analysis from the RAPID-randomised controlled trial (RAPID-RCT) and the RAPID-open label extension (RAPID-OLE) study, which compared the safety and tolerability of adverse event (AE) rates for two different alpha-1 antitrypsin (AAT) dosing patterns, weekly infusions of 60 mg/kg AAT, and bi-weekly infusions of 120 mg/kg AAT. Results showed there were no significant differences for exposure-adjusted event rates (p=0.850), infusion-adjusted event rates (p=0.344), and serious treatment emergent AE (TEAE) (p=1.0); TEAE occurring in the first 24 and 48 hours were comparable for both groups. Prof Sandhaus presented the results of a telephone survey from the USA AlphaNet organisation of self-infusion practices in 555 patients with AATD. The survey found that 7.9% of respondents self-administered AAT and 92.1% who did not. Of the 44 patients who self-administered AAT, 95.4% reported being very satisfied and 4.6% were satisfied with their treatment.

Alpha 1-antitrypsin (AAT) deficiency is a genetic disorder that primarily affects the lungs and liver. While AAT deficiency is one of the most common genetic disorders in the Caucasian population, it is extremely rare in Asians. Here, we report the case of a 36-year-old Korean woman with AAT deficiency who visited the emergency department of our hospital for the treatment of progressive dyspnea that had begun 10 years ago. She had never smoked. Chest computed tomography revealed panlobular emphysema in both lungs, which suggested AAT deficiency. The serum AAT level was 33 mg/dL (reference interval: 90-200 mg/dL). Four exons of the SERPINA1 gene, which is responsible for AAT deficiency, and their flanking regions were analyzed by PCR-direct sequencing. The patient was found to have 1 missense mutation (c.230C>T, p.Ser77Phe; Siiyama) and 1 frameshift mutation (c.1158dupC, p.Glu387ArgfsX14; QOclayton). This is the first Korean case of AAT deficiency confirmed by genetic analysis and the second case of a compound heterozygote of Siiyama and QOclayton, the first case of which was reported from Japan.

Severe alpha-1 antitrypsin (AAT) deficiency is a proven genetic risk factor for COPD, but there is marked variation in the development of COPD among AAT deficient subjects. To investigate familial aggregation of lung function in subjects with AAT deficiency, we estimated heritability for forced expiratory volume in 1 s (FEV₁) and FEV₁/forced vital capacity (FVC) in 378 AAT deficient subjects from 167 families in the AAT Genetic Modifiers Study; all subjects were verified homozygous for the Z AAT deficiency allele. Heritability was evaluated for models that included and excluded an ascertainment correction, as well as for models that excluded, included and were stratified by a cigarette smoking covariate. In models without an ascertainment correction, and in all models without a covariate for smoking, no evidence for familial aggregation of lung function was observed. In models conditioned on the index proband with covariates for smoking, post-bronchodilator FEV₁/FVC demonstrated significant heritability (0.26 ± 0.14, p = 0.03). When we limited the analysis to subjects with a smoking history, post-bronchodilator FEV₁demonstrated significant heritability (0.47 ± 0.21, p = 0.02). Severity rate phenotypes were also assessed as potential phenotypes for genetic modifier studies. Significant heritability was found with all age-of-onset threshold models that included smoking and ascertainment adjustments. Using the t-distribution, the heritability estimates ranged from 0.43 to 0.64, depending on the age-of-onset of FEV₁decline used for the severity rate calculation. Correction for ascertainment and consideration of gene-by-smoking interactions will be crucial for the identification of genes that may modify susceptibility for COPD in families with AAT deficiency.

US Centers for Disease Control guidelines recommend replacement of peripheral intravenous (IV) catheters no more frequently than every 72 to 96 hours. Routine replacement is thought to reduce the risk of phlebitis and bloodstream infection. Catheter insertion is an unpleasant experience for patients and replacement may be unnecessary if the catheter remains functional and there are no signs of inflammation. Costs associated with routine replacement may be considerable. This is an update of a review first published in 2010. To assess the effects of removing peripheral IV catheters when clinically indicated compared with removing and re-siting the catheter routinely. For this update the Cochrane Vascular Trials Search Co-ordinator searched the Cochrane Vascular Specialised Register (March 2015) and CENTRAL (2015, Issue 3). We also searched clinical trials registries (April 2015). Randomised controlled trials that compared routine removal of peripheral IV catheters with removal only when clinically indicated in hospitalised or community dwelling patients receiving continuous or intermittent infusions. Two review authors independently assessed trial quality and extracted data. Seven trials with a total of 4895 patients were included in the review. The quality of the evidence was high for most outcomes but was downgraded to moderate for the outcome catheter-related bloodstream infection (CRBSI). The downgrade was due to wide confidence intervals, which created a high level of uncertainty around the effect estimate. CRBSI was assessed in five trials (4806 patients). There was no significant between group difference in the CRBSI rate (clinically-indicated 1/2365; routine change 2/2441). The risk ratio (RR) was 0.61 (95% CI 0.08 to 4.68; P = 0.64). No difference in phlebitis rates was found whether catheters were changed according to clinical indications or routinely (clinically-indicated 186/2365; 3-day change 166/2441; RR 1.14, 95% CI 0.93 to 1.39). This result was unaffected by whether infusion through the catheter was continuous or intermittent. We also analysed the data by number of device days and again no differences between groups were observed (RR 1.03, 95% CI 0.84 to 1.27; P = 0.75). One trial assessed all-cause bloodstream infection. There was no difference in this outcome between the two groups (clinically-indicated 4/1593 (0.02%); routine change 9/1690 (0.05%); P = 0.21). Cannulation costs were lower by approximately AUD 7.00 in the clinically-indicated group (mean difference (MD) -6.96, 95% CI -9.05 to -4.86; P ≤ 0.00001). The review found no evidence to support changing catheters every 72 to 96 hours. Consequently, healthcare organisations may consider changing to a policy whereby catheters are changed only if clinically indicated. This would provide significant cost savings and would spare patients the unnecessary pain of routine re-sites in the absence of clinical indications. To minimise peripheral catheter-related complications, the insertion site should be inspected at each shift change and the catheter removed if signs of inflammation, infiltration, or blockage are present.

CRISPR (clustered regularly interspaced short palindromic repeats) genome editing holds promise in the treatment of genetic diseases that currently lack effective long-term therapies. Patients with alpha-1 antitrypsin (AAT) deficiency develop progressive lung disease due to the loss of AAT's antiprotease function and liver disease due to a toxic gain of function of the common mutant allele. However, it remains unknown whether CRISPR-mediated AAT correction in the liver, where AAT is primarily expressed, can correct either or both defects. Here we show that AAV delivery of CRISPR can effectively correct Z-AAT mutation in the liver of a transgenic mouse model. Specifically, we co-injected two AAVs: one expressing Cas9 and another encoding an AAT guide RNA and homology-directed repair template. In both neonatal and adult mice, this treatment partially restored M-AAT in the serum. Furthermore, deep sequencing confirmed both indel mutations and precise gene correction in the liver, permitting careful analysis of gene editing events in vivo. This study demonstrates a proof of concept for the application of CRISPR-Cas9 technology to correct AAT mutations in vivo and validates continued exploration of this approach for the treatment of patients with AAT deficiency.

Providing care at the end of life: do Medicare rules impede good care?

British Journal of NursingVol. 23, No. Sup8 Research and GuidelinesThe One Million Global Catheters PIVC worldwide prevalence studyEvan AlexandrouEvan AlexandrouSearch for more papers by this authorEvan AlexandrouPublished Online:6 May 2014https://doi.org/10.12968/bjon.2014.23.Sup8.S16AboutSectionsView articleView Full TextPDF/EPUB ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareShare onFacebookTwitterLinked InEmail View article References Ahlqvist M, Berglund B, Nordstrom G, Klang B, Wirén M, Johansson E (2010) A new reliable tool (PVC assess) for assessment of peripheral venous catheters. J Eval Clin Pract 16(6): 1108–15. doi: https://doi.org/10.1111/j.1365-2753.2009.01278.x Crossref, Medline, Google ScholarKagel EM, Rayan GM (2004) Intravenous catheter complications in the hand and forearm. J Trauma 56(1): 123–7. doi: https://doi.org/10.1097/01.TA.0000058126.72962.74 Crossref, Medline, Google ScholarMaki DG, Kluger DM, Crnich CJ (2006) The risk of bloodstream infection in adults with different intravascular devices: a systematic review of 200 published prospective studies. Mayo Clin Proc 81(9): 1159–71. http://bit.ly/R9JTjG Google ScholarWebster J, Clarke S, Paterson Det al. (2008) Routine care of peripheral intravenous catheters versus clinically indicated replacement: randomised controlled trial. BMJ 337: a339. doi: https://doi.org/http://dx.doi.org/10.1136/bmj.a339 Crossref, Medline, Google ScholarZingg W, Pittet D (2009) Peripheral venous catheters: an under-evaluated problem. Int J Antimicrob Agents 34(Suppl 4): S38–42. doi: https://doi.org/10.1016/S0924-8579(09)70565-5 Crossref, Medline, Google Scholar FiguresReferencesRelatedDetailsCited byThe effects of ultrasound guidance on first-attempt success for difficult peripheral intravenous catheterization: a systematic review and meta-analysis20 January 2023 | European Journal of Emergency Medicine, Vol. 30, No. 2Ultraschallgesteuerte periphere Venenpunktion bei schlechtem Venenstatus8 February 2023 | Die Anaesthesiologie, Vol. 23The effect of clinical guideline education on the knowledge and practice of nurses for peripheral intravenous catheter placement based on short message service: A quasi-experimental study30 May 2022 | The Journal of Vascular Access, Vol. 24Ultrasound-Guided Peripheral Intravenous Catheter Insertion Training Reduces Use of Midline Catheters in Hospitalized Patients With Difficult Intravenous Access27 September 2021 | Journal of Patient Safety, Vol. 18, No. 3The impact of a notched peripheral intravenous catheter on the first attempt success rate in hospitalized adults: Block-randomized trial2 February 2021 | The Journal of Vascular Access, Vol. 23, No. 2Development of a Three-Dimensionally Printed Ultrasound-Guided Peripheral Intravenous Catheter PhantomCureus, Vol. 23The Role of Ultrasound in the Ambulatory Setting9 March 2021Difficult intravenous access as an independent predictor of delayed care and prolonged length of stay in the emergency department19 August 2020 | Journal of the American College of Emergency Physicians Open, Vol. 1, No. 6Subcutaneous hydration and medications infusions (effectiveness, safety, acceptability): A systematic review of systematic reviews24 August 2020 | PLOS ONE, Vol. 15, No. 8Nurse-Performed Ultrasound-Guided Technique for Difficult Peripheral Intravenous Access in Critically Ill Patients: A Randomized Controlled Trial3 April 2020 | Journal of the Association for Vascular Access, Vol. 25, No. 2Better With UltrasoundChest, Vol. 157, No. 2Accès veineux central en radiologie interventionnelleLa Presse Médicale, Vol. 48, No. 10Vessel health and preservation: An integrative review14 November 2018 | Journal of Clinical Nursing, Vol. 28, No. 7-8Road map for improvement: Point prevalence audit and survey of central venous access devices in paediatric acute care6 October 2016 | Journal of Paediatrics and Child Health, Vol. 53, No. 2Indications for Peripheral, Midline, and Central Catheters: Summary of the Michigan Appropriateness Guide for Intravenous Catheters RecommendationsJournal of the Association for Vascular Access, Vol. 21, No. 3Ultrasonography Versus Landmark for Peripheral Intravenous Cannulation: A Randomized Controlled TrialAnnals of Emergency Medicine, Vol. 68, No. 1Development of the UK Vessel Health and Preservation (VHP) framework: a multi-organisational collaborativeJournal of Infection Prevention, Vol. 17, No. 2Effectiveness of a training program in compliance with recommendations for venous lines care30 July 2015 | BMC Infectious Diseases, Vol. 15, No. 1 23 April 2014Volume 23Issue Sup8ISSN (print): 0966-0461ISSN (online): 2052-2819 Metrics History Published online 6 May 2014 Published in print 23 April 2014 Information© MA Healthcare LimitedPDF download

Rationale: The genetic condition alpha-1 antitrypsin deficiency (AATD) is often unrecognized. In adults, AATD can cause common obstructive pulmonary disease and/or liver disease. We previously developed a machine learning model using claims data from a large United States database (Komodo Health), with the goal of identifying patients with AATD symptoms who were undiagnosed but likely had AATD. The objective of this study was to calibrate, refine, and evaluate the diagnostic performance of the model using deidentified patient data from the Cleveland Clinic Electronic Medical Record (EMR) for application within this clinical system. Methods: Cleveland Clinic EMR deidentified patient records from January 1, 1998 to August 15, 2024 were divided into two cohorts; 1) the positive cohort included patients with AATD, as indicated by an AATD diagnosis code and confirmed either by genetic testing or by serum alpha-1 antitrypsin levels; 2) the negative cohort included patients who had symptoms and comorbidities similar to AATD but no records of AATD diagnosis or treatments, in addition to randomly selected control patients. For model training and calibration, only data until the first AATD diagnosis or treatment were used. The data were first split into calibration and validation subsets (80% and 20%, respectively). The model was refined on the calibration data using 3-fold cross-validation, and the calibrated model was then evaluated on the hold-out validation data (unseen during training) to predict AATD. Receiver operating characteristic (ROC) and precision-recall (PR) curves, as well as areas under the curve (AUC) were generated. The final calibrated model was further validated by two Cleveland Clinic pulmonary physicians (UH, JKS) who independently reviewed 60 patient records and assigned probabilities of AATD. Results: Of 21,166 records analyzed, the calibration and the validation datasets consisted of 582 positive and 5864 negative patients. The calibrated model achieved high levels of performance (ROC-AUC=0.89, PR-AUC=0.62) on the hold-out validation subset in distinguishing AATD positive from negative cases (Figure 1). At a discrimination threshold of 0.90, the model achieved high sensitivity=0.70, high specificity=0.92, and balanced accuracy=0.81. Conclusions: A previously developed machine learning model for identifying patients with AATD was successfully calibrated and validated using patient data from the Cleveland Clinic EMR. As measured by ROC and PR on unseen validation data, the model achieved high levels of performance, demonstrating differentiation between AATD and similar conditions or randomly selected controls.

THE incidence of intraoperative awareness with explicit recall is approximately 1–2 cases/1,000, with high-risk situations including cardiac surgery, trauma surgery, and emergency obstetric interventions.1,2There can be severe and persistent psychiatric sequelae to awareness, which may recur years after the awareness event. The most severe consequence is post–traumatic stress disorder (PTSD), which can present with depression, anxiety attacks, or debilitating flashbacks.Because the triggers of PTSD in this population are often healthcare settings and healthcare workers, many such patients will avoid further medical care. In patients who do return for surgical intervention, there is often a distrust of anesthesiologists, as well as fears related to PTSD flashbacks or a further awareness event.3As such, a therapeutic modality that could attenuate the PTSD response to the surgical environment would be of great benefit in minimizing subsequent perioperative psychiatric sequelae. Here, we report the successful treatment of a patient with PTSD after intraoperative awareness by psychotherapy and desensitization to the operating room.The patient was a 49-yr-old, 77-kg woman with a history of scoliosis, pseudoarthrosis, and other orthopedic problems who presented for a staged posterior–anterior lumbar spine fusion. Of note, the patient had a family history of malignant hyperthermia, and she had experienced cardiac arrest under ether during a hip surgery in 1968. It is unclear whether the arrest was due to malignant hyperthermia. Since that time, she had undergone more than 30 surgical procedures, with no adverse events. The posterior portion of the case proceeded uneventfully, and she presented 1 week later for the anterior intervention.The patient had a 20-gauge intravenous catheter placed in her right hand preoperatively and was premedicated with 2 mg midazolam and 100 μg fentanyl. The patient was given 100 mg propofol and 7 mg vecuronium for induction; tracheal intubation proceeded uneventfully. Anesthetic maintenance initially consisted of 80 μg · kg−1· min−1propofol, 0.05 μg · kg−1· min−1remifentanil, and 50% nitrous oxide in oxygen. Because of the patient's history of nausea and vomiting, nitrous oxide was discontinued shortly after surgical incision, and infusion rates were increased. At this time, arterial catheter placement was attempted in the right radial artery. During the placement of the arterial line, the intravenous catheter on the right hand ceased functioning, which interrupted the delivery of anesthetic. There was an increase in heart rate, but this was also the period directly after incision. After discovery of the malfunctioning intravenous catheter, the propofol and remifentanil infusions were transferred to a second peripheral intravenous catheter. No further benzodiazepine was administered.Postoperatively, the patient reported that she had been aware, unable to move, and in pain for a portion of the case. She expressed relief when the anesthesiologist confirmed that there was a period during which she could have been aware, because it validated her experience. After supportive visits by the anesthesiology staff and resident, the patient was referred for psychological evaluation and treatment. Selected points of her outpatient psychological treatment are described below.The patient experiences intraoperative awareness.Upon her first outpatient psychiatric evaluation, the patient endorsed daily intrusive thoughts about the surgical event that were "very upsetting." She reported fear of having surgery again and distress when exposed to reminders of her surgery (such as a "surgical blue" color or medical television shows). She also experienced an almost complete loss of interest in activities that were enjoyable to her before her surgery, as well as a feeling of distance and detachment. She stated that she had difficulty falling asleep and reported moderate to severe hypervigilance.The patient reported continued symptoms of PTSD and avoidance behaviors. She was reassured that her symptoms were normal responses to a traumatic experience. The rationale and description of exposure-based therapy for the treatment of PTSD was given, and questions were answered. A list of avoided situations was constructed and ranked according to the level of anxiety each provoked. The patient was instructed to engage in one of the lower ranked items, lying on her back in a dark room, for 20 min each day.The patient reported that she had engaged in behaviors at home that she had been previously avoiding, including watching television shows with medical themes. Prolonged exposure to the trauma memory was initiated during therapy. The patient completed a first exposure and was coached on remaining in the first person, including as many sensory details as possible.Supportive counseling was provided. "In vivo " exposure was done in the magnetic resonance imaging simulator one time each month. "Homework" assignments were given to continue engaging in previously avoided activities.Supportive counseling and cognitive behavioral therapy were provided. The patient and her psychologist went to a partially equipped operating room. The patient sat in a chair in the operating room and discussed her thoughts, emotions, and sensations elicited by the operating room environment. The patient seemed to focus on the anesthesia cart for much of the time and was visibly distressed.Supportive counseling and cognitive behavioral therapy was provided. Repeated in vivo exposure to surgery cues was performed in a fully equipped operating room that was not in use. Initial anxiety scores and anxiety scores at 20 min were reported by the patient and recorded by her psychologist (fig. 1).The patient, her psychologist, and two anesthesiologists met in an operating room in preparation for a colonoscopy the patient would be undergoing later in the month. The anesthesiologists (G.A.M. and N.N.N.) were not involved in the original case but answered questions about awareness and general anesthesia. After the meeting, the patient reported being very satisfied with the exchange and said that she was grateful for the opportunity.The patient reported that she underwent a colonoscopy procedure the previous week that was both "good and bad." She stated that her interaction with the anesthesiologist before the procedure was unsatisfying in that he was insensitive regarding her awareness experience. The patient elected to have her colonoscopy at a hospital closer to her home, so the anesthesiologists involved in the therapeutic process were not present for the actual procedure. Despite the unsatisfying encounter with her anesthesiologist on that day, she had no physical or psychological complications during or after the procedure. The patient's PTSD was deemed by her psychologist to be in remission.The incidence of PTSD after intraoperative awareness is still controversial. Prospective studies have found an incidence of 0–44%,4–6whereas retrospective cases series suggest an incidence of 2–56%.7–10The diagnosis of PTSD requires (1) exposure to a traumatic event; (2) reexperiencing of the traumatic event in recurrent thoughts, nightmares, or flashbacks; (3) avoidance of stimuli associated with the trauma; (4) hyperarousal states; (5) greater than 1 month duration of symptoms; and (6) functional impairment.11Particularly problematic in the case of awareness patients is that the cues associated with the trauma are usually related to healthcare settings and healthcare workers. There is thus the potential for avoidance of further medical care or surgical intervention. Risk factors for PTSD include severity of trauma, female sex, middle age, single status, psychiatric history, low education status, and borderline personality disorder.11In patients experiencing awareness, a severe early emotional response was the only predictor found in one major study of PTSD and late psychological symptoms.10Treatment modalities for PTSD include cognitive behavioral therapy, exposure or "flooding" therapy, eye-movement desensitization reprocessing, and antidepressant medication.11–13Guiding the patient through the reexploration of traumatic memories is a key element of PTSD therapy, but in vivo exposure to traumatic cues has been found to be more effective.12Cognitive behavioral therapy attempts to restructure the intellectual framework within which the traumatic memory is processed.13To our knowledge, there is only one case report of cognitive behavioral therapy and exposure in the treatment of PTSD in a patient experiencing awareness.13In that case, however, the in vivo exposure was limited to visiting the hospital in which the surgery—a cesarean delivery—was performed.The current case represents the first report of in vivo exposure to an operating room setting in a patient with PTSD after awareness during general anesthesia. It is important to note that the patient went through a graded exposure, starting with a reexploration of the memory of traumatic events and concluding with time in an actual operating room. This case is also unique because (1) anesthesiologists were involved in the therapeutic process and (2) the therapy and desensitization occurred before and in anticipation of another medical intervention. The patient was particularly grateful to be in the operating room with anesthesiologists who could address her questions. Although we cannot ascribe a strict causal relation, we believe that the graded exposure to the operating room setting and the participation of anesthesiologists in the therapy enabled the patient to undergo a further intervention in a hospital setting without psychological sequelae such as flashbacks, panic attacks, or anxiety. This case suggests that psychotherapy in conjunction with exposure and desensitization to the operating room may be an effective treatment for PTSD in patients who have experienced intraoperative awareness. Further investigation, such as case series analysis and randomized study, is warranted to validate this therapeutic technique.

Background The benefits of pulmonary rehabilitation (PR) on fatigue-resistant skeletal muscle fibre type I have been found to be smaller in COPD patients with alpha-1 antitrypsin deficiency (AATD) than in those without AATD. Alpha-1 antitrypsin (AAT) augmentation therapy was suggested as a potential factor of influence. Whether this finding mirrors different improvements in 6-min walk distance (6MWD) between both groups remains unknown. Methods 140 patients with AATD-related COPD (phenotype PiZZ, FEV 1 : 31 ± 8%pred.) and 280 COPD patients without AATD (FEV 1 : 31 ± 8%pred.) were matched for baseline 6MWD and included in a retrospective analysis. AATD patients were divided into those “on” (AATD AUG+ ) or “off” (AATD AUG- ) augmentation therapy. 6MWD was assessed pre and post an inpatient 4-week PR program. Plasma level of creatinine was analysed at baseline. Results In AATD and COPD patients with comparable initial 6MWD (331 ± 106 m and 326 ± 101 m, p = n.s.), improvements in 6MWD following PR were similar (+49 ± 49 m and +53 ± 52 m, intra-group change: p AUG+ : +51 ± 55 m, AATD AUG- : +47 ± 40 m, p = n.s.). Only in AATD group, higher baseline creatinine levels and lower 6MWD were positive predictors for the PR-related increase in 6MWD. Conclusions Independently of the genetic variant of AAT, COPD patients achieved the same training-related benefit in 6MWD. Augmentation therapy showed no effect on 6MWD adaptation during PR.

346 Ultra Long Versus Standard Long Peripheral Intravenous Catheters: A Randomized Controlled Trial of Ultrasound-Guided Catheter Survival

To determine the overall and per-day risk of complications of short peripheral intravenous (PIV) catheters placed for indefinite periods. During 5 months, general pediatric patients receiving intravenous therapy through short PIV catheters were monitored. Patient and catheter characteristics were recorded, complications were noted, and rolled semiquantitative cultures of removed catheters were performed. Major endpoints were infection and phlebitis. Per-day risk of complications and catheter colonization (>15 colony-forming units) were calculated. University children's hospital. General pediatric ward inpatients with PIV. We studied 642 Teflon catheters in place >24 hours (mean, 3.7 days) in 525 patients. There were no cases of catheter sepsis (0%; 95% confidence interval [CI95], 0%-0.6%), one possible insertion-site infection (0.2%; CI95, 0.004%-0.9%), and seven cases of phlebitis (1.1%; CI95, 0.4%-2.3%). Catheter colonization occurred in 92 (26%) of 348 catheters cultured. Neither the per-day risk of phlebitis nor of catheter colonization increased significantly with placement >3 days. Current guidelines recommend replacement of PIV catheters in adults within 2 to 3 days; no recommendations are made for children. Our findings and those of others indicate that the overall risk of PIV catheter complications in children is extremely low and would not be reduced substantially by routine catheter replacement.

Peripheral intravenous catheters (PIVCs) are common devices used across many healthcare settings. This quality improvement project aims to proactively remove PIVCs as soon as possible by empowering nurses and providers to clinically evaluate the necessity of every PIVC on a daily basis on a general hospital medical unit. Specific criteria were established to assess PIVC necessity. Cases of PIVCs not meeting established criteria are escalated to providers for a decision point. The PIVC removal times documented within the electronic medical record were analyzed to compare precriteria PIVC dwell times to postcriteria dwell times. The time between removal of a patient's last PIVC and patient discharge was analyzed to determine if more PIVCs are being removed sooner after becoming clinically unnecessary. Significantly fewer PIVCs (decrease of 14.4%) are being removed on the day of discharge in the postintervention time frame, whereas more PIVCs are being removed one (increase of 5.5%) or two (increase of 4.0%) days before the day of discharge. A strategic project to critically evaluate PIVCs on a daily basis and remove PIVCs not meeting criteria for use was successful in proactively removing PIVCs. Hospitals should evaluate PIVC practice, monitor daily usage, and strategically intervene to remove unneeded PIVCs.