James K Stoller

Pulmonary hypertension after liver transplantation in patients with antecedent hepatopulmonary syndrome : A report of 2 cases and review of the literature

Hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PPHTN) are distinct clinical entities that may complicate liver disease. Although HPS and PPHTN are different, several reports describe 6 patients in whom both conditions have occurred, either concurrently or sequentially, sometimes with the onset of PPHTN after liver transplantation. The current report extends this sparse experience by reporting 2 patients who underwent liver transplantation for HPS and who developed pulmonary hypertension after liver transplantation. This experience calls for better understanding of the pathogenesis of HPS and PPHTN and ways to better predict their occurrence. Liver Transpl 12:1278‐1282, 2006.

A review of augmentation therapy for alpha-1 antitrypsin deficiency

Introduction: Alpha-1 antitrypsin deficiency (AATD) is a relatively common, but under-recognized condition which manifests commonly with liver cirrhosis and emphysema. Specific therapy for lung-affected individuals with AATD is augmentation therapy, which consists of intravenous infusion of purified human plasma-derived alpha-1 antitrypsin (AAT). Augmentation therapy was first approved by the United States Food and Drug Administration (FDA) in 1987 for emphysema associated with severe AATD and today, six augmentation therapy preparations, all of which derive from pooled human plasma, have received FDA approval. Areas covered: This paper reviews augmentation therapy for AATD, including the various available commercial preparations, their processing and biochemical differences, evidence regarding biochemical and clinical efficacy, patterns of clinical use, adverse effect profiles, cost-effectiveness and potential uses in conditions other than emphysema associated with AATD. Novel and emerging strategies for treating AATD are briefly discussed next, including alternative dosing and administration strategies, recombinant preparations, small molecule inhibitors of neutrophil elastase and of AAT polymerization, autophagy-enhancing drugs and gene therapy approaches. Expert opinion: We conclude with a discussion of our approach to managing patients with AATD and use of augmentation therapy.

Emotional intelligence competencies provide a developmental curriculum for medical training

Since healthcare faces challenges of access, quality, and cost, effective leadership for healthcare is needed. This need is especially acute among physicians, whose demanding training focuses on scientific and clinical skills, eclipsing attention to leadership development. Among the competencies needed by leaders, emotional intelligence (EI) – defined as the ability to understand and manage oneself and to understand others and manage relationships – has been shown to differentiate between great and average leaders. In this context, teaching EI as part of the medical training curriculum is recommended. Furthermore, because physicians’ developmental needs evolve over the course of prolonged training, specific components of EI (e.g., teambuilding, empathy, and negotiation) should be taught at various phases of medical training. Consistent with the concept of a spiral curriculum, such EI competencies should be revisited iteratively throughout training, with differing emphasis and increasing sophistication to meet evolving needs. For example, teamwork training is needed early in undergraduate medical curricula to prompt collaborative learning. Teamwork training is also needed during residency, when physicians participate with differing roles on patient care teams. Training in EI should also extend beyond graduate medical training to confer the skills needed by clinicians and by faculty in academic medical centers.

Augmentation therapy in α-1 antitrypsin deficiency

Background: α-1 Antitrypsin deficiency is a genetic disorder that leads to early-onset emphysema. Recently, exogenous supplementation of the enzyme has become a therapeutic alternative. Objective: To review the role of so-called augmentation therapy with pooled human plasma α-1 antitrypsin as a specific treatment for emphysema caused by α-1 antitrypsin deficiency. Methods: The authors performed a Medline (1966 – 2007) search with the keywords ‘α-1 antitrypsin deficiency’ and ‘therapy’. The authors focused on articles regarding biochemical and clinical efficacy. Results/conclusion: Augmentation therapy has been shown to raise antiprotease serum and epithelial lining fluid levels above the ‘protective threshold’ value. Evidence suggests that this approach slows the decline in lung function, could reduce infection rates, might enhance survival, and is well tolerated. Questions about the cost-effectiveness of this therapy remain.

Transudative chylothorax: report of two cases and review of the literature.

Transudative chylothorax is a rare entity that has been associated with a limited range of clinical settings. To date, transudative chylothoraces have been described in only 13 patients, most commonly as a result of hepatic cirrhosis. Recognition of the transudative nature of these effusions is important to avoid unnecessary diagnostic testing and inappropriate management strategies. This report describes the presentation, diagnosis and management of two patients with transudative chylothoraces, and provides a brief review of the relevant literature.

Alpha-1 antitrypsin (AAT) deficiency - what are the treatment options?

Alpha-1 antitrypsin (AAT) deficiency is an under-recognized genetic condition that predisposes to liver disease and early-onset emphysema. Although AAT is mainly produced in the liver, its main function is to protect the lung against proteolytic damage from neutrophil elastase. The most common mutation responsible for severe AAT deficiency, the so-called Z variant, reduces serum levels by promoting polymerization of the molecule within the hepatocyte, thereby reducing secretion. Serum levels below the putative protective threshold level of 11 micromolar (μmol/L) increase the risk of emphysema. In addition to the usual treatments for emphysema, infusion of purified AAT from pooled human plasma represents a specific therapy for AAT deficiency and raises serum and epithelial lining fluid levels above the protective threshold. Substantial evidence supports the biochemical efficacy of this approach, particularly for the weekly infusion regimen. Definitive evidence of clinical efficacy is still needed, as the two available randomized controlled trials showed non-significant trends towards slowing rates of loss of lung density on lung computerized axial tomography. However, concordant results of prospective cohort studies suggest that augmentation therapy has efficacy in slowing the rate of decline of lung function in patients with moderate airflow obstruction and severe deficiency of AAT. Overall, augmentation therapy is well-tolerated and, despite its failure to satisfy criteria for cost-effectiveness, is recommended because it is the only currently available specific therapy for AAT deficiency.

Developing leadership competencies among medical trainees: five-year experience at the Cleveland Clinic with a chief residents' training course

Objectives: Challenges in healthcare demand great leadership. In response, leadership training programs have been developed within academic medical centers, business schools, and healthcare organizations; however, we are unaware of any well-developed programs for physicians-in-training. Methods: To address this gap, we developed a two-day leadership development course for chief residents (CRs) at the Cleveland Clinic, framed around the concept of emotional intelligence. This paper describes our five-year experience with the CRs leadership program. Results: Since inception, 105 CRs took the course; 81 (77%) completed before-and-after evaluations. Participants indicated that they had relatively little prior knowledge of the concepts that were presented and that the workshop greatly enhanced their familiarity with leadership competencies. Qualitative analysis of open-ended responses indicated that attendees valued the training, especially in conflict resolution and teamwork, and indicated specific action plans for applying these skills. Furthermore, the workshop spurred some participants to express plans to learn more about leadership competencies. Conclusions: This study extends prior experience in offering an emotional intelligence-based leadership workshop for CRs. Though the program is novel, further research is needed to more fully understand the impact of leadership training for CRs and for the institutions and patients they serve.

Respiratory Care Work Assignment Based on Work Rate Instead of Work Load

BACKGROUND: We previously reported a new management variable, work rate, defined as work load due per hour, based on cumulative standard treatment times. We found that work rates were unachievable (ie, exceeded 1 hour of scheduled work per hour of available labor) for 75% of scheduled due times, despite presumed achievable average work load. OBJECTIVE: To determine the optimal strategy for creating work assignments based on work rate. METHODS: A focus group used root-cause analysis to identify ways to balance assignments based on work rate. We surveyed employees to assess their willingness to start earlier. We determined the ratio of scheduled to unscheduled work during a 12-month period. The scheduled work comprised administering small-volume nebulizer, metered-dose inhaler, noninvasive ventilation, and mechanical ventilation. The unscheduled work consisted of all other modalities. We also developed a spreadsheet model to assess the effect of shifting the start time on work-rate distribution in a representative 24-hour period. RESULTS: The focus group determined that starting treatments 1 hour earlier would help. Fifteen of the 24 clinicians surveyed responded, and 13 of the respondents were willing to start earlier. The scheduled work load averaged approximately 55% of the total work load, but there was high variability per assignment area (range 0–99%). The spreadsheet model showed that shifting treatment start times improved the distribution of work rate throughout the day, but did not guarantee that labor demand never outstrips supply. CONCLUSIONS: Our studies to date suggest that: basing assignments on average work load leads to periods of unachievable work rate, resulting in missed treatments and staff dissatisfaction. We have only limited ability to reduce peaks in work rate, but staggering treatment times is effective. Fair assignment of work should differentiate scheduled from unscheduled work.

Respiratory Therapist-Managed Arterial Catheter Insertion and Maintenance Program: Experience in a Non-Teaching Community Hospital

BACKGROUND: Usual practice in community health-care settings indicates that arterial catheters are inserted by physicians. In the context of a respiratory therapist (RT)-managed arterial catheter placement protocol being implemented in our community hospital, the current study describes the implementation and outcomes of this RT-managed arterial catheter insertion and maintenance program. METHODS: Tuality Healthcare is a 215-bed community health-care system (10-bed ICU) in Hillsboro, Oregon. With the goal of enhancing the quality of ICU care, an RT-managed multidisciplinary team was implemented to lead the delivery of protocolized ventilator liberation, arterial catheter insertion, and arterial blood gas utilization. Preparation for the program included didactic teaching, simulation-based training, and precepted procedural experience. A database was created for audit and quality improvement purposes. Outcomes and arterial blood gas utilization data were obtained from the audit database and from the hospital electronic health record. RESULTS: During the 4-y period (March 1, 2012, to April 31, 2016), 256 arterial catheter insertion attempts were made by a team of 12 qualified RTs. The success rate for the initial placement attempt by RT was high (94.5% [242 of 256]). Sixty-three percent of arterial lines were placed in patients to help manage severe sepsis/septic shock. No ischemic or infectious complications were reported during the study period. Nearly 40% (96 of 242) of the successful placements by RTs on initial attempts were performed during the night shift, when intensivists were not physically present in the ICU. CONCLUSIONS: This experience establishes the feasibility of an RT-managed arterial catheter placement program in a community ICU. The RT-managed program was characterized by a high degree of success and safety and allowed arterial catheter placement at times when intensivists were not available in the ICU. This experience extends the sparse reported experience of RT-managed arterial catheter placement programs and underscores the value of RTs as members of the ICU team.