Transcatheter device occlusion of the left ventricular outflow tract as treatment for severe aortic regurgitation in hypoplastic left heart syndrome.

Aortic regurgitation is a rare but dangerous condition in patients with hypoplastic left heart syndrome (HLHS). We report the case of a 2-year-old girl with HLHS with aortic/mitral stenosis (AS-MS) subtype after stage II palliation via the bilateral bidirectional Glenn (BBDG) procedure, when aortic regurgitation presented with the clinical manifestation of a paroxysmal coronary steal phenomenon provoked by exertion. Transcatheter closure of the left ventricular outflow tract (LVOT) with an Amplatzer(TM) Vascular Plug 4 minimized and finally abolished aortic reflux without compromising either coronary perfusion or atrioventricular (AV) conduction. Midterm follow-up over 3 years revealed complete disappearance of her symptoms, and follow-up catheterization displayed complete LVOT closure. This case illustrates the promising potential of modern cardiac catherization techniques in certain cases and emphasizes that precise assessment of the native aorta and coronary system is critical in HLHS patients.

A new functional assay for the diagnosis of X-linked inhibitor of apoptosis (XIAP) deficiency.

X-linked inhibitor of apoptosis (XIAP) deficiency, caused by mutations in BIRC4, is an immunodeficiency associated with immune dysregulation and a highly variable clinical presentation. Current diagnostic screening tests such as flow cytometry for XIAP expression or lymphocyte apoptosis assays have significant limitations. Based on recent evidence that XIAP is essential for nucleotide-binding and oligomerization domains (NOD)1/2 signalling, we evaluated the use of a simple flow cytometric assay assessing tumour necrosis factor (TNF) production of monocytes in response to NOD2 stimulation by muramyl dipeptides (L18-MDP) for the functional diagnosis of XIAP deficiency. We investigated 12 patients with XIAP deficiency, six female carriers and relevant disease controls. Irrespective of the diverse clinical phenotype, the extent of residual protein expression or the nature of the mutation, the TNF response was severely reduced in all patients. On average, L18-MDP induced TNF production in 25% of monocytes from healthy donors or female carriers, while fewer than 6% of monocytes responded in affected patients. Notably, the assay clearly discriminated affected patients from disease controls with other immunodeficiencies accompanied by lymphoproliferation, hypogammaglobulinaemia or inflammatory bowel disease. Functional testing of the NOD2 signalling pathway is an easy, fast and reliable assay in the diagnostic evaluation of patients with suspected XIAP deficiency.

[Hemorrhagic skin lesions associated with infections]. / Infektionsassoziierte Hautblutungen.

A broad spectrum of bacterial, viral, and parasitic infections is associated with hemorrhagic skin lesions, typically petechiae. The most prominent underlying entity is fulminant bacterial sepsis, which requires urgent and intensive treatment. In most cases, however, a self-limiting viral disease is the underlying cause. Thus, the pediatrician frequently encounters a diagnostic dilemma between timely diagnosis of sepsis and unnecessary invasive diagnostics. This article reviews the broad differential diagnosis and pathophysiology of infection-associated hemorrhagic skin lesions and proposes a diagnostic algorithm for the combination of fever and petechiae.

Capital sectors and workers' health and safety in the United States.

The dual or segmented economy perspective suggests that the U.S. production system within a capitalist world-system can be divided into distinct sectors based on elements such as levels of industrial concentration, foreign involvement, and unionization. The differing organization of these sectors is argued to influence worker health and safety (WHS) outcomes. An economic segmentation model was applied to national occupational health data to examine the relationship between structural divisions in the economy and occupational hazard exposure, injury, and illness. Workers in more global industrial sectors had only average levels of hazardous exposure but a greater likelihood of occupational injury and illness than workers in other sectors of the economy. These differences are related to the structure of work in the various sectors. The findings suggest the need for (1) greater surveillance and reporting of WHS problems through the general health care system; (2) planning of economic and productive activity that takes WHS issues into account; and (3) greater worker organization and power within and between nations to improve WHS.

Theory and method for the cross-national study of health systems.

The comparison of nations' health systems requires some way of categorizing them. As distinct from the topologies of Roemer, Maxwell, and others, which offer no apparent theoretical derivation, the schema suggested here derives from theory of the capitalist political economic world-system as put forth by Wallerstein and others. With the worldwide division of labor entailed in this perspective (core, semi-periphery, and periphery), combined with the strengths of workers' movements, five types of nations' health systems are identified, and illustrative comparisons are made using the contrasting case studies method. The author also discusses seeming exceptions to the general rule of capitalist dependencies with weak workers' movements having inequitous inadequate health systems.

Dispelling myths on ambulance accidents.

There appears to be a self-perpetuating trail of misinformation surrounding the issue of ambulance accidents. As with all problems, we must first acknowledge that a problem does exist and clearly distinguish the facts from the myths. The intent of this article was to clarify some of those misconceptions by using an analysis of one state's four-year experience with ambulance accidents. The next step in solving this problem is to create driver education programs that modify the behavior of ambulance drivers, adjust their attitudes about driving an emergency vehicle and make them fully aware of the hazards encountered in driving an ambulance. In addition, consider adjusting the agency standard operating procedure so that all ambulances must come to a complete stop at all stop signs and red lights to minimize the number of accidents that occur in the intersection. Readers who would like to learn more about this subject should contact their state EMS offices to determine if they offer emergency vehicle operator courses or continuing education programs that deal with ambulance driving. New York State has developed a network of 90 instructors who are qualified to teach the Ambulance Accident Prevention Seminar, a nine-hour classroom-based ambulance driver "attitude adjustment" program. Two other organizations that have developed driving courses geared specifically for ambulance driving are: Failsafe Driving Inc. Alvin F. Davenport, president 2529 San Pablo Ave. Pinole, CA 94564 National Academy for Professional Driving Dick Turner, chairman 1001-A South Interstate 45 P.O. Box 649 Hutchins, TX 75141.

The political economy of workers' health and safety.

The paucity of social science research on workers' health and safety (WHS) is noted and a framework based on class struggle within the capitalist political economic world-system is suggested. It is hypothesized that the historical and current strengths of workers' movements within nation states will largely determine the adequacy of provisions for WHS. This hypothesis was generally confirmed through field studies in Sweden, Finland, the German Democratic Republic, the Federal Republic of Germany, the U.K. and the U.S.A. However, one key aspect, the linkages between the general health services, especially at points of first entry (sometimes inappropriately referred to as primary health care, PHC) and WHS are highly problematic in all systems. Workers and their representatives, as well as concerned WHS and public health workers should develop greater awareness, concern and understanding of this PHC-WHS problem as well as other aspects of WHS.

Cuba and the Philippines: contrasting cases in world-system analysis.

Cuba and the Philippines are countries with broad similarities in historical background yet sharp divergences in political economic developments and relations to the capitalist world-system in recent times. U.S. economic and political interests dominated both countries during the first half of the 20th century. The changes generated by the Cuban revolution resulted in the end of U.S. power in Cuba in 1959. The Philippines, however, remain profoundly dependent on the United States. The approach taken in this article contrasts these countries, asking what the results of their divergent paths are in terms of health and health services. The ability of Cuba and the Philippines to support the primary health care (PHC) approach by fostering socioeconomic justice, authentic citizen participation, and a regionalized health system is examined. It is clear that the last 25 years of socialist-oriented development in Cuba reversed the negative effects of the previous market economy by providing improved social and health services. The success of the political economy and the fully regionalized health system, supportive of the PHC approach in Cuba, is reflected in the high-level health status of the people. In contrast, poverty, gross social and economic inequities, high prevalence of infectious disease, and inaccessible, inadequate, and uncoordinated health services persist in the Philippines after some 85 years of international and national capitalist development. The poor health status of the Philippine people is a direct reflection of this underdeveloped system.

PIP: Cuba and the Philippines are contrasted with respect to histories of US domination, divergent paths in recent times, and the results as reflected in terms of health and health services. The ability of each country to support the primary health care (PHC) approach by fostering socioeconomic justice, citizen participation, and a regionalized health system is examined. The last 25 years of socialist-oriented development in Cuba reversed the negative effects of the previous market economy by providing improved social and health services. Supportive conditions for the PHC approach in Cuba have resulted in the high-level health status of the people. In contrast, poverty, gross social and economic inequities, high prevalence of infectious disease, and inaccessible, inadequate, and uncoordinated health services persist in the Philippines after some 85 years of capitalist development. The poor health status of the people is a direct reflection of this underdeveloped system.

An evaluation of emergency medical technicians' ability to use manual ventilation devices.

Three hundred twenty emergency medical technicians (EMTs) were tested for their ability to ventilate a precalibrated Recording Resusci-Annie (Laerdal Medical Corporation) using both a bag-valve-mask and a pocket mask. More than 50% of the EMTs were not capable of ventilating to the minimum standard using a bag-valve-mask. The study demonstrates that the pocket mask method is far superior to the bag-valve-mask method. If these experimental results are confirmed by clinical findings, we recommend that future educational courses teach the bag-valve-mask as a four-hand/two-person skill, with one rescuer squeezing the bag with both hands and the second rescuer maintaining hyperextension.