Hereditary sensory neuropathy type I

Hereditary sensory neuropathy type I (HSN I) is a slowly progressive neurological disorder characterised by prominent predominantly distal sensory loss, autonomic disturbances, autosomal dominant inheritance, and juvenile or adulthood disease onset. The exact prevalence is unknown, but is estimated as very low. Disease onset varies between the 2nd and 5th decade of life. The main clinical feature of HSN I is the reduction of sensation sense mainly distributed to the distal parts of the upper and lower limbs. Variable distal muscle weakness and wasting, and chronic skin ulcers are characteristic. Autonomic features (usually sweating disturbances) are invariably observed. Serious and common complications are spontaneous fractures, osteomyelitis and necrosis, as well as neuropathic arthropathy which may even necessitate amputations. Some patients suffer from severe pain attacks. Hypacusis or deafness, or cough and gastrooesophageal reflux have been observed in rare cases. HSN I is a genetically heterogenous condition with three loci and mutations in two genes (SPTLC1 and RAB7) identified so far. Diagnosis is based on the clinical observation and is supported by a family history. Nerve conduction studies confirm a sensory and motor neuropathy predominantly affecting the lower limbs. Radiological studies, including magnetic resonance imaging, are useful when bone infections or necrosis are suspected. Definitive diagnosis is based on the detection of mutations by direct sequencing of the SPTLC1 and RAB7 genes. Correct clinical assessment and genetic confirmation of the diagnosis are important for appropriate genetic counselling and prognosis. Differential diagnosis includes the other hereditary sensory and autonomic neuropathies (HSAN), especially HSAN II, as well as diabetic foot syndrome, alcoholic neuropathy, neuropathies caused by other neurotoxins/drugs, immune mediated neuropathy, amyloidosis, spinal cord diseases, tabes dorsalis, lepra neuropathy, or decaying skin tumours like amelanotic melanoma. Management of HSN I follows the guidelines given for diabetic foot care (removal of pressure to the ulcer and eradication of infection, followed by the use of specific protective footwear) and starts with early and accurate counselling of patients about risk factors for developing foot ulcerations. The disorder is slowly progressive and does not influence life expectancy but is often severely disabling after a long duration of the disease

The Role of Medical Education in Reducing Health Care Disparities: The First Ten Years of the UCLA/Drew Medical Education Program

BACKGROUND: The University of California, Los Angeles (UCLA)/Charles R. Drew University Medical Education Program was developed to train physicians for practice in underserved areas. The UCLA/Drew Medical Education Program students receive basic science instruction at UCLA and complete their required clinical rotations in South Los Angeles, an impoverished urban community. We have previously shown that, in comparison to their UCLA counterparts, students in the Drew program had greater odds of maintaining their commitment to medically disadvantaged populations over the course of medical education. OBJECTIVE: To examine the independent association of graduation from the UCLA/Drew program with subsequent choice of physician practice location. We hypothesized that participation in the UCLA/Drew program predicts future practice in medically disadvantaged areas, controlling for student demographics such as race/ethnicity and gender, indicators of socioeconomic status, and specialty choice. DESIGN: Retrospective cohort study. PARTICIPANTS: Graduates (1,071) of the UCLA School of Medicine and the UCLA/Drew Medical Education Program from 1985–1995, practicing in California in 2003 based on the address listed in the American Medical Association (AMA) Physician Masterfile. MEASUREMENTS: Physician address was geocoded to a California Medical Service Study Area (MSSA). A medically disadvantaged community was defined as meeting any one of the following criteria: (a) federally designated HPSA or MUA; (b) rural area; (c) high minority area; or (d) high poverty area. RESULTS: Fifty-three percent of UCLA/Drew graduates are located in medically disadvantaged areas, in contrast to 26.1% of UCLA graduates. In multivariate analyses, underrepresented minority race/ethnicity (OR: 1.57; 95% CI: 1.10–2.25) and participation in the Drew program (OR: 2.47; 95% CI: 1.59–3.83) were independent predictors of future practice in disadvantaged areas. CONCLUSIONS: Physicians who graduated from the UCLA/Drew Medical Education Program have higher odds of practicing in underserved areas than those who completed the traditional UCLA curriculum, even after controlling for other factors such as race/ethnicity. The association between participation in the UCLA/Drew Medical Education Program and physician practice location suggests that medical education programs may reinforce student goals to practice in disadvantaged communities

High pressure diamond-like liquid carbon

We report density-functional based molecular dynamics simulations, that show that, with increasing pressure, liquid carbon undergoes a gradual transformation from a liquid with local three-fold coordination to a 'diamond-like' liquid. We demonstrate that this unusual structural change is well reproduced by an empirical bond order potential with isotropic long range interactions, supplemented by torsional terms. In contrast, state-of-the-art short-range bond-order potentials do not reproduce this diamond structure. This suggests that a correct description of long-range interactions is crucial for a unified description of the solid and liquid phases of carbon.Comment: 4 pages, 5 figure

Do Physicians with Self-Reported Non-English Fluency Practice in Linguistically Disadvantaged Communities?

BackgroundLanguage concordance between physicians and patients may reduce barriers to care faced by patients with limited English proficiency (LEP). It is unclear whether physicians with fluency in non-English languages practice in areas with high concentrations of people with LEP.ObjectiveTo investigate whether physician non-English language fluency is associated with practicing in areas with high concentrations of people with LEP.DesignCross-sectional cohort study.ParticipantsA total of 61,138 practicing physicians no longer in training who participated in the California Medical Board Physician Licensure Survey from 2001-2007.MeasuresSelf-reported language fluency in Spanish and Asian languages. Physician practice ZIP code corresponding to: (1) high concentration of people with LEP and (2) high concentration of linguistically isolated households.MethodsPractice location ZIP code was geocoded with geographic medical service study designations. We examined the unadjusted relationships between physician self-reported fluency in Spanish and selected Asian languages and practice location, stratified by race-ethnicity. We used staged logistic multiple variable regression models to isolate the effect of self-reported language fluency on practice location controlling for age, gender, race-ethnicity, medical specialty, and international medical graduate status.ResultsPhysicians with self-reported fluency in Spanish or an Asian language were more likely to practice in linguistically designated areas in these respective languages compared to those without fluency. Physician fluency in an Asian language [adjusted odds ratio (AOR) = 1.77; 95% confidence intervals (CI): 1.63-1.92] was independently associated with practicing in areas with a high number of LEP Asian speakers. A similar pattern was found for Spanish language fluency (AOR = 1.77; 95% CI: 1.43-1.82) and areas with high numbers of LEP Spanish-speakers. Latino and Asian race-ethnicity had the strongest effect on corresponding practice location, and this association was attenuated by language fluency.ConclusionsPhysicians who are fluent in Spanish or an Asian language are more likely to practice in geographic areas where their potential patients speak the corresponding language

Fundamental Reform of Payment for Adult Primary Care: Comprehensive Payment for Comprehensive Care

Primary care is essential to the effective and efficient functioning of health care delivery systems, yet there is an impending crisis in the field due in part to a dysfunctional payment system. We present a fundamentally new model of payment for primary care, replacing encounter-based imbursement with comprehensive payment for comprehensive care. Unlike former iterations of primary care capitation (which simply bundled inadequate fee-for-service payments), our comprehensive payment model represents new investment in adult primary care, with substantial increases in payment over current levels. The comprehensive payment is directed to practices to include support for the modern systems and teams essential to the delivery of comprehensive, coordinated care. Income to primary physicians is increased commensurate with the high level of responsibility expected. To ensure optimal allocation of resources and the rewarding of desired outcomes, the comprehensive payment is needs/risk-adjusted and performance-based. Our model establishes a new social contract with the primary care community, substantially increasing payment in return for achieving important societal health system goals, including improved accessibility, quality, safety, and efficiency. Attainment of these goals should help offset and justify the costs of the investment. Field tests of this and other new models of payment for primary care are urgently needed

The providers of health services in Lebanon: a survey of physicians

BACKGROUND: Emerging from civil distress carries with it major challenges to reforming a health system. One such challenge is to ensure an adequate supply of competent human resources. The objective of this study was to assess the supply of physicians in Lebanon in 1998, with an assessment of their practice patterns and capacity building. METHODS: Lists of members of physician's associations were examined to determine the number of physicians in Lebanon and their geographical distribution. A self-administered survey targeted 388 physicians (5%) randomly stratified by the five regions of Lebanon. Some 377 providers reported information on their demographic profile, practice patterns and development. Further, information on continuing education activities was acquired. RESULTS: In Lebanon, the overall physician-to-population ratio was 248 per 100, 000, characterized by an evident maldistribution at the intracountry regional level. Physicians worked 38 hours per week examining on average 21 patients per day, with an average time of 30 minutes spent per visit. They also reported spending 11% of their time waiting for patients. Respondents reported a very wide range of income, with 90% earning less than USD 2,000 per month. Moreover, the continuing education profile revealed a total of 43.7 hours per year, similar to that required for board certification in many developed countries. Conference attendance was the dominant continuing education activity (95% of respondents) and consumed most of the time allotted for continuing education, reported at 32 hours per year. DISCUSSION AND CONCLUSION: Various economic indicators point to an oversupply of physicians in Lebanon and a poor allocation of their time for capacity building. Therefore, it is crucial for decision-makers to closely monitor the increasing supply of providers and institute appropriate intervention strategies, taking into consideration appropriate provision of good-quality services and ensuring that continuing education activities are well established, organized and monitored