Yogaman: An Inexpensive, Anatomically-detailed Chest Tube Placement Trainer.

INTRODUCTION: Opportunities for chest tube placement in emergency medicine training programs have decreased, making competence development and maintenance with live patients problematic. Available trainers are expensive and may require costly maintenance. METHODS: We constructed an anatomically-detailed model using a Halloween skeleton thorax, dress form torso, and yoga mat. Participants in a trial session completed a survey regarding either their comfort with chest tube placement before and after the session or the realism of Yogaman vs. cadaver lab, depending on whether they had placed <10 or 10 or more chest tubes in live patients. RESULTS: Inexperienced providers reported an improvement in comfort after working with Yogaman, (comfort before 47 millimeters [mm] [interquartile ratio {IQR}, 20-53 mm]; comfort after 75 mm [IQR, 39-80 mm], p=0.01). Experienced providers rated realism of Yogaman and cadaver lab similarly (Yogaman 79 mm [IQR, 74-83 mm]; cadaver lab 78 mm [IQR, 76-89 mm], p=0.67). All evaluators either agreed or strongly agreed that Yogaman was useful for teaching chest tube placement in a residency program. CONCLUSION: Our chest tube trainer allowed for landmark identification, tissue dissection, pleura puncture, lung palpation, and tube securing. It improved comfort of inexperienced providers and was rated similarly to cadaver lab in realism by experienced providers. It is easily reusable and, at $198, costs a fraction of the price of available commercial trainers.

Wilson disease: Health-related quality of life and risk for depression.

BACKGROUND: Wilson disease is an autosomal recessive disorder of copper metabolism and requires lifelong medical treatment. Therefore, the analysis of quality of life has gathered more attention. Aims of this study were to examine risk for depression and health-related quality of life in patients suffering from Wilson disease. METHODS: Sixty-eight patients were included in this retrospective cross sectional study. The Personal Health Questionnaire-9 Depression Scale was used to assess depression. The Short Form-36 Health Survey questionnaire was used to assess health-related quality of life. RESULTS: The Personal Health Questionnaire-9 indicated that 21% (14/68) of patients were at risk for major depressive disorders (scores>10) and 35% (24/68) were at risk for mild depression (scores 5-9). Women had significantly lower life quality scores than men. Primary neurologic disease manifestation was associated with significantly lower total Short Form-36 and subdimension scores compared with primary hepatic or mixed presentation. Overall, patients with Wilson disease experienced higher quality of life than patients with other chronic liver diseases. CONCLUSIONS: As patients with Wilson disease have a high risk for depressive disorders, active assessment for depression is mandatory. Patients with primary neurological symptoms are at higher risk for reduction of life quality.

Coagulation Parameters in Wilson Disease.

BACKGROUND & AIMS: Wilson disease (WD) is an autosomal recessive disorder of copper metabolism. Alterations of copper metabolism have been associated with changes in coagulation factors. The aim of the present study was the analysis of coagulation factors in WD patients. METHODS: 100 patients attending a tertiary WD outpatient clinic were analyzed in a prospective cross sectional cohort study. Out of peripheral venous blood samples coagulation factors were assessed including: full blood count, INR, partial thromboplastin time (PTT), clotting factors II, V, VII, VIII, IX, X, XI, XII, XIII, von Willebrand factor/-antigen, fibrinogen, antithrombin III, protein S, protein C, activated protein C (APC) resistance. Subgroup analyses of the blood tests were performed for sex, initial clinical presentation, WD treatment and liver function. RESULTS: Subgroup analysis by liver function showed decreased levels of factors II, V, VII and X. Subgroup analysis by gender or clinical course of the disease did not reveal significant coagulation changes. In patients treated with trientine significantly decreased levels of factors II, VII and antithrombin III and increased von Willebrand factor/-antigen levels were detected. Factor VIII levels were significantly reduced in patients receiving zinc. CONCLUSION: Although significant differences of some coagulation parameters in subgroup analysis were found, no clinically relevant alterations of the coagulation system in WD patients could be detected.

Nature as capital: Advancing and incorporating ecosystem services in United States federal policies and programs.

The concept of nature as capital is gaining visibility in policies and practices in both the public and private sectors. This change is due to an improved ability to assess and value ecosystem services, as well as to a growing recognition of the potential of an ecosystem services approach to make tradeoffs in decision making more transparent, inform efficient use of resources, enhance resilience and sustainability, and avoid unintended negative consequences of policy actions. Globally, governments, financial institutions, and corporations have begun to incorporate natural capital accounting in their policies and practices. In the United States, universities, nongovernmental organizations, and federal agencies are actively collaborating to develop and apply ecosystem services concepts to further national environmental and economic objectives. Numerous federal agencies have begun incorporating these concepts into land use planning, water resources management, and preparations for, and responses to, climate change. Going forward, well-defined policy direction will be necessary to institutionalize ecosystem services approaches in federal agencies, as well as to guide intersector and interdisciplinary collaborative research and development efforts. In addition, a new generation of decision support tools are needed to further the practical application of ecosystem services principles in policymaking and commercial activities. Improved performance metrics are needed, as are mechanisms to monitor the status of ecosystem services and assess the environmental and economic impacts of policies and programs. A greater national and international financial commitment to advancing ecosystem services and natural capital accounting would likely have broad, long-term economic and environmental benefits.

Increased Prevalence of Subcutaneous Lipomas in Patients With Wilson Disease.

GOALS: To determine the prevalence and characteristics of lipomas in patients with Wilson disease. BACKGROUND: Wilson disease is an autosomal recessive disorder resulting in copper accumulation in the liver and the central nervous tissue. Subcutaneous lipomas were often noted by the authors during clinical examinations of patients with Wilson disease. This is the first study to analyze the prevalence and progression of lipoma development in patients with Wilson disease. STUDY: Eighty consecutive patients attending a tertiary care center were examined for the presence of subcutaneous lipomas. RESULTS: Subcutaneous lipomas could be detected during the examination of 21 (26%) of the 80 patients with Wilson disease. Multiple subcutaneous lipomas were present in 16 (76%) of the 21 affected patients. Lipomas were mainly found on the extremities and the trunk. Neither initial presentation nor decoppering treatment influenced the presence or course of lipomas in these patients. CONCLUSIONS: Subcutaneous lipoma formation is more common in patients with Wilson disease than in the general population. We suggest that the presence of lipomas contributes to the differential diagnosis of Wilson disease.

Individual and organizational predictors of pediatric psychiatric inpatient admission in connecticut hospitals: a 6 month secondary analysis.

The objective of this study is to test the hypotheses that bipolar disorders or depressive disorders, minority status, and the presence of pediatric inpatient psychiatric unit will be individual predictors of pediatric psychiatric inpatient admission, and to provide a model that will evaluate which individual and organizational characteristics predict pediatric psychiatric inpatient. For this purpose, a secondary analysis of the medical records of 1,520 pediatric patient visits between January 1, 2008 and June 30, 2008, was conducted using univariate and multivariate logistic regression. Independent predictors of pediatric psychiatric inpatient admission were presence of bipolar and depressive disorders, greater average daily census, and increasing operating margin. Minority status was a significant predictor of not being admitted, as was presence of an anxiety disorder, greater total margin and older age. The results indicate that both individual and organizational factors impact disposition outcomes in particular subsets of pediatric patients who present to emergency departments for psychiatric reasons.

Zinc monotherapy is not as effective as chelating agents in treatment of Wilson disease.

BACKGROUND & AIMS: Wilson disease is a genetic disorder that affects copper storage, leading to liver failure and neurologic deterioration. Patients are treated with copper chelators and zinc salts, but it is not clear what approach is optimal because there have been few studies of large cohorts. We assessed long-term outcomes of different treatments. METHODS: Patients in tertiary care centers were retrospectively analyzed (n = 288; median follow-up time, 17.1 years) for adherence to therapy, survival, treatment failure, and adverse events from different treatment regimens (chelators, zinc, or a combination). Hepatic treatment failure was defined as an increase in activity of liver enzymes (aspartate aminotransferase, alanine aminotransferase, and γ-glutamyltransferase) >2-fold the upper limit of normal or >100% of baseline with an increase in urinary copper excretion. RESULTS: The median age at onset of Wilson disease was 17.5 years. Hepatic and neuropsychiatric symptoms occurred in 196 (68.1%) and 99 (34.4%) patients, respectively. Hepatic treatment failure occurred more often from zinc therapy (14/88 treatments) than from chelator therapy (4/313 treatments; P < .001). Actuarial survival, without transplantation, showed an advantage for chelating agents (P < .001 vs zinc). Changes in treatment resulted mostly from adverse events, but the frequency did not differ between groups. Patients who did not respond to zinc therapy showed hepatic improvement after reintroduction of a chelating agent. CONCLUSIONS: Treatments with chelating agents or zinc salt are effective in most patients with Wilson disease; chelating agents are better at preventing hepatic deterioration. It is important to identify patients who do not respond to zinc therapy and have increased activities of liver enzymes, indicating that a chelating agent should be added to the therapeutic regimen.

Identification and characterization of asparagine deamidation in the light chain CDR1 of a humanized IgG1 antibody.

Despite technological advances, detection of deamidation in large proteins remains a challenge and the use of orthogonal methods is needed for unequivocal assignment. By a combination of cation-exchange separation, papain digestion, and a panel of mass spectrometry techniques we identified asparagine deamidation in light chain complementarity determining region 1 (CDR1) of a humanized IgG1 monoclonal antibody. The reaction yields both Asp and isoAsp, which were assigned by Edman degradation and by isoAsp detection using protein isoaspartate methyltransferase. The deamidated antibody variants were less potent in antigen binding compared to the nondegraded antibody. Changes in near-UV CD spectra, susceptibility to papain cleavage in an adjacent CDR2 loop, and the tendency of the newly formed isoAsp to undergo isomerization suggest local perturbations in the structure of the isoAsp-containing antibody.

Wilson protein expression, copper excretion and sweat production in sweat glands of Wilson disease patients and controls.

BACKGROUND: In Wilson disease, copper is not sufficiently excreted into bile due to the absence or malfunction of the Wilson protein copper ATPase in the excretory pathway of hepatocytes. Copper is found in sweat. It is unknown if the Wilson protein plays a role in copper excretion into sweat. It is the aim of this study to investigate Wilson protein expression in sweat glands and analysing its effects on copper excretion into sweat in controls and patients with Wilson disease. METHODS: Immunofluorescent analysis of the Wilson protein in skin samples from normal rat, LEC rat and human skin biopsies were performed. Pilocarpin-induced sweat gland stimulation by iontophoretic transfer adapted from the methods used for cystic fibrosis sweat test was used for sweat induction. Sweat volume, sweat copper concentration, serum ceruloplasmin and serum copper were analysed in 28 Wilson patients and 21 controls. RESULTS: The Wilson protein is expressed in human and rat sweat gland epithelia. Copper concentration in sweat is not significantly different between controls and Wilson patients. Wilson patients produce significantly smaller volumes of sweat compared to controls. Sweat production is partially reversible in Wilson patients under medical treatment for Wilson disease or after liver transplantation CONCLUSION: Wilson patients show a reduced sweat production with unaltered sweat copper concentration. The Wilson protein might play an important role in physiological sweat production.

Analysis of the human Atox 1 homologue in Wilson patients.

AIM: To analyze the metallochaperone antioxidant-1 (Atox1) gene sequence in Wilson disease patients. METHODS: Mutation analysis of the four exons of the Atox1 gene including the intron- exon boundaries was performed in 63 Wilson disease patients by direct sequencing. RESULTS: From 63 selected patients no mutations were identified after the entire coding region including the intron- exon boundaries of Atox1 were sequenced. One known polymorphism within the Atox1 gene (5'UTR -99 T>C) in 31 (49%) of the Wilson patients as well as one previously undescribed variation (5'UTR -68 C>T) in 2 of the Wilson patients could be detected. Statistical analyses revealed that the existence of a variation within the Atox1- gene showed a tendency towards an earlier onset of the disease. CONCLUSION: Based on the data of this study, no major role can be attributed to Atox1 in the pathophysiology or clinical variation of Wilson disease.

Copper toxicosis gene MURR1 is not changed in Wilson disease patients with normal blood ceruloplasmin levels.

AIM: To analyze our Wilson disease patient cohort (n=106) for alterations in the gene coding for MURR1. METHODS: Patients with an established diagnosis of Wilson disease but normal ceruloplasmin blood levels were chosen for our study (n = 14). Patients with two known disease-causing mutations in the ATP7B gene were not included. The three exons of the human MURR1 gene were sequenced after amplification of the genomic DNA by polymerase chain reaction. RESULTS: Our study did not reveal any mutations leading to an amino acid change in the MURR1 sequence of Wilson disease patients. A polymorphism at 472 bp of the coding sequence could be confirmed. CONCLUSION: The MURR1 gene plays no role in the pathogenesis of Wilson disease patients with normal serum ceruloplasmin levels.

Analysis of the human homologue of the canine copper toxicosis gene MURR1 in Wilson disease patients.

Wilson disease is a human disorder of copper metabolism resulting in toxic copper accumulation. Patients present with a high clinical variability, even when sharing identical mutations. MURR1, the gene causing canine copper toxicosis in Bedlington terriers, maps to chromosome 2 in humans, a region different to the Wilson gene locus. MURR1 might influence human copper metabolism and the clinical presentation of Wilson disease patients. This study analyzed MURR1 gene sequence in Wilson disease patients and MURR1 gene transcription in selected patients. Mutation analysis of three exons of the MURR1 gene including the intron-exon boundaries was performed in 63 Wilson disease patients by direct sequencing. Of the 63 Wilson patients 19 (30%) had basepair changes in the MURR1 gene. Three intronic base pair changes, one new sequence variation and two known polymorphisms were detected, including the GAT/GAC heterozygous state at codon Asn 164 in 15 (24%) of the analyzed patients. This suggests that GAT/GAC heterozygous state at codon Asn 164 is associated with an earlier onset of disease.

Child and adolescent psychiatry. A state administrator's view.

In the course of the nation's evolution toward managed-care service delivery and financing models, state administration of Medicaid and other public-sector health care services has become increasingly complex. This article reviews and comments on key policy issues that state administrators face in the organization and financing of Medicaid-managed behavioral health care services. The author offers recommendations for states that are considering additional reforms.