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Canine fucosidosis in English Springer spaniels is the only animal model of the neurovisceral lysosomal storage disease fucosidosis available for preclinical therapeutic trials. For this reason, it is crucial to identify critical time points in disease progression, and if there are particular lesions associated with specific aspects of neurologic dysfunction. Historical records of 53 canine fucosidosis cases from 1979 to 2009 containing a neurologic dysfunction score assessing motor, behavioral and sensory dysfunction were interrogated by statistical analysis. Motor and behavioral dysfunction scores assessing gait deficits and apprehensive behavior first significantly increased at 12-17 months, and increased at each 6-month interval thereafter. Sensory dysfunction scores, assessing hearing loss, balance and vision deterioration, did not significantly increase until 18-23 months, and coincided with a rapid decline in neurologic function. Regression analysis incorporating published neuropathology data, measured by image analysis, identified neuroinflammation and apoptotic cell death as significant informative predictors of increasing neurologic dysfunction. These findings indicate that the level of neuropathology required to induce consistent and conspicuous clinical signs in canine fucosidosis is reached by approximately 12 months of age in the absence of other disease processes. Significant association between neuroinflammation and apoptotic cell death also suggests that specifically targeting these lesions combined with enzyme replacement in future studies may reduce disease burden in fucosidosis. Overall, examining this historical clinical data to identify associations between the extent of neuropathology and degree of clinical dysfunction provides a useful reference tool for monitoring disease and evaluating therapeutic trials conducted in canine fucosidosis.

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An autosomal recessive form of cerebellar abiotrophy occurs in Australian Kelpie dogs. Clinical signs range from mild ataxia with intention tremor to severe ataxia with seizures. A whole-genome mapping analysis was performed using Affymetrix Canine SNP array v2 on 11 affected and 19 control dogs, but there was no significant association with disease. A homozygosity analysis identified a three megabase region likely to contain the disease mutation. The region spans 29.8-33 Mb on chromosome 3, for which all affected dogs were homozygous for a common haplotype. Microsatellite markers were developed in the candidate region for linkage analysis that resulted in a logarithm of odds score suggestive of linkage. The candidate region contains 29 genes, none of which are known to cause ataxia.

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Anesthesia crisis resource management (ACRM) was developed by David Gaba, MD, and colleagues at Stanford University in the early 1990s. Derived from cockpit resource management of the aviation industry, ACRM addresses the issues of human performance and patient safety in anesthesia. Due to the inherent complexity of our dynamic work environment, we are frequently faced with situations that could escalate into critical incidents. ACRM explains the role of personal and environmental factors that can contribute to the evolution of critical incidents and provides the practitioner with some behavioral and intellectual guidelines to manage the risks more effectively. ERR WATCH is an acronym I developed to interpret the principles of ACRM from the nurse anesthetist's perspective. It provides a quick review of the major principles of ACRM, which are Environment, Resources, Reevaluation, Workload, Attention, Teamwork, Communication, and Help. Used together with good clinical management, these principles may provide an edge in solving complex problems and improving performance.

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High fidelity simulators are a new technology for training and research in anesthesiology. The simulators accurately replicate the anesthetist's entire work environment, including the patient, anesthesia machine, monitors, and drugs. The trainee is allowed to develop and refine his skills in anesthesia management in a safe and realistic environment. By tailoring the scenario, both basic and advanced management skills can be taught. Learners can be exposed to serious or uncommon anesthetic events which they may not otherwise see during their training. Videotaping and case logs provide unbiased recording of the session. Simulators also allow the use of new instructional techniques that are untenable in a real operating room, such as allowing critical incidents to develop fully to teach management skills or redoing cases that were poorly handled. Allowing the student to make critical errors, pausing the simulation to discuss management, or replaying the case with different management techniques are valuable teaching tools. Research studies in human performance, learning and cognition, and anesthesia crisis management training are currently underway and may produce improved training methods for novice and expert anesthesia practitioners alike.

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Interactions between warfarin and penicillins have been infrequently reported. A case report of a single patient who experienced the effects of a warfarin-nafcillin interaction as well as a warfarin-dicloxacillin interaction is presented. Clinical effects of this interaction were documented primarily through changes in prothrombin time (PT) and the need for higher warfarin dosing. While the patient received nafcillin, warfarin doses were increased to as much as 4.5 times the previous amounts needed to provide adequate anticoagulation. During dicloxacillin therapy, warfarin doses were gradually decreased, but stabilized to a maintenance dose higher than the patient's pre-nafcillin dose. The dicloxacillin-warfarin interaction appears similar to that noted during nafcillin-warfarin combination.

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Use of recombinant human erythropoietin has been advocated for therapy in anemic patients with end-stage renal disease and to enhance the harvesting of autologous red blood cells from healthy patients scheduled for elective orthopedic surgery. The authors report the case of a diabetic patient with moderate chronic renal failure and cholelithiasis but whose religious beliefs forbade the use of transfusion therapy. She underwent successful cholecystectomy only after treatment with recombinant human erythropoietin.

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Human papillomavirus infection is probably the most prevalent sexually transmitted disease in the United States. In adults, it is associated with condylomata acuminata and with neoplastic changes ranging from dysplasia to carcinoma. Infected mothers may transmit human papillomavirus during the perinatal period; affected children face prolonged, difficult treatment for respiratory papillomatosis. Prevention of infection remains the best approach, since diagnostic and therapeutic methods are suboptimal.

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About 2 percent of current cases of acquired immunodeficiency syndrome are among patients less than 13 years of age. When a woman infected by the human immunodeficiency virus (HIV) becomes pregnant, her neonate has approximately a 40 percent chance of becoming infected vertically in the perinatal period. Experience in detecting HIV-infected pregnant women and in diagnosing their affected neonates has been less than satisfactory. In this review, the epidemiology, pathogenesis, transmission, clinical manifestations, diagnosis, and treatment-prevention of perinatal HIV infection are discussed.

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Sexually transmitted diseases (STDs) have reached epidemic proportion in the United States and have captured the attention of both laypersons and health care professionals. Of special concern is that most STDs can be transmitted vertically to the offspring of infected mothers. Since the advent of acquired immunodeficiency syndrome, other STDs have been at risk of being relatively disregarded. This paper, the second of two parts, reviews issues of prevalence, morbidity, mortality, diagnosis, prevention, and treatment of group B streptococcal and chlamydial infections as they affect the maternal-fetal dyad.

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An analysis was made of the death rates from accidents in children aged 0-14 by health districts in England and Wales during the five year periods 1974-79 and 1980-84. Death rates were generally higher in the north and west of England and lower in the south and east. Rates were higher in urban areas than in rural areas. There was a more than five-fold difference between the highest and lowest rates by districts during both periods. There was a very strong correlation with social deprivation. Greater efforts are required to reduce unnecessary deaths and disability from childhood accidents. The lessons already learned in many parts of the world must now be put into much wider use and practice in the United Kingdom.

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