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We report the cases of two adult patients with cystic fibrosis affecting the pancreas and liver, who also had severe vitamin E deficiency and neurologic disease. The most prominent clinical features were abnormal eye movements, diminished reflexes, decreased vibratory and position sense, ataxia, and muscle weakness. Treatment with intramuscular injections of vitamin E partially corrected the neurologic deficits. Vitamin E absorption tests documented severe malabsorption, which was later alleviated by the addition of dessicated ox bile to the regimen of alpha-tocopheryl acetate. These studies suggest that a decreased intraluminal concentration of bile salts is an important factor in the development of severe vitamin E deficiency and in the poor response to oral replacement therapy that is seen in some patients with cystic fibrosis. [ABSTRACT FROM AUTHOR]

Introduction: Voluntary cough (VC) and the laryngeal expiration reflex (LER) provoke stress urinary incontinence (SUI). The aim of this article is to analyze the effectiveness of these stimuli on the timing of urinary leaks., Material and Methods: Urodynamic testing using pressure catheters was performed on 123 subjects with history of SUI. The LER was triggered using the induced reflex cough test (iRCT). Each subject was tested with VC and LER and leaked with one or both stimuli. The occurrence and timing of leaks were recorded., Results: The peak and average intra-abdominal pressures were 16-19% greater for LER compared to VC. Of the 123 subjects, LER caused leak in 118 (96%), VC in 71 (58%) and both in 66 (54%). For LER compared to VC, leak was more likely to occur during or immediately after the first expiratory effort. The electromyogram for VC and LER were similar., Conclusions: The iRCT reliably initiated the LER and triggered SUI more effectively than VC. During VC, the smooth muscle of the internal urethral sphincter (IUS) starts to contract during inspiration, and constriction of the IUS continues into the expiratory phase; this increased urethral tonicity would lessen the likelihood of SUI. We refer to this as the inspiration closure reflex (ICR). With LER the inspiration would not take place, and the first expiratory effort would be against a non-constricted IUS, making leak more likely. Our findings disprove the pressure transmission theory. The internal and external urethral sphincters may both increase urethral closure pressure and resistance., Competing Interests: Disclosure Drs. Addington, Stephens and Miller have a stock interest in Pneumoflex Systems, Inc. These authors were not compensated for this study. The data is part of an FDA application by Pneumoflex Systems LLC, ClinicalTrials.gov Identifier: NCT00801203., (Copyright by Polish Urological Association.)

Aspiration is a common result of stroke, and may lead to lung infections and pneumonia. Cough may prevent this aspiration and thus prevent the pneumonia. We review the four types of cough usually used to assess aspiration risk: voluntary cough (VC), reflex cough (RC), the laryngeal expiration reflex (LER), and cough on swallow (CoS). VC is easy to test but starts with an inspiration that may cause aspiration, and is controlled by cortico-brainstem pathways that may not be involved in influencing aspiration. RC also starts with an inspiration, and requires instrumental intervention, but is more relevant to protecting the lungs. The LER starts with an expiration, so is 'anti-aspiration', and is easy to test, but its neural mechanisms have not been fully analysed. CoS can be tested at the same time as direct observations of aspiration, but little is known about its neural mechanisms. Each method has its advocates, and the purpose of the review is to discuss how each may be applied and how the information from each may be assessed and valued., (Copyright © 2011 Elsevier Ltd. All rights reserved.)

Background: In the last 3 decades, there has been an unexplained increase in the prevalence of asthma and hay fever., Objective: We sought to determine whether there is an association between childhood vaccination and atopic diseases, and we assessed the self-reported prevalence of atopic diseases in a population that included a large number of families not vaccinating their children., Methods: Surveys were mailed to 2964 member households of the National Vaccine Information Center, which represents people concerned about vaccine safety, to ascertain vaccination and atopic disease status., Results: The data included 515 never vaccinated, 423 partially vaccinated, and 239 completely vaccinated children. In multiple regression analyses there were significant ( P < .0005) and dose-dependent negative relationships between vaccination refusal and self-reported asthma or hay fever only in children with no family history of the condition and, for asthma, in children with no exposure to antibiotics during infancy. Vaccination refusal was also significantly ( P < .005) and negatively associated with self-reported eczema and current wheeze. A sensitivity analysis indicated that substantial biases would be required to overturn the observed associations., Conclusion: Parents who refuse vaccinations reported less asthma and allergies in their unvaccinated children. Although this relationship was independent of measured confounders, it could be due to differences in other unmeasured lifestyle factors or systematic bias. Further research is needed to verify these results and investigate which exposures are driving the associations between vaccination refusal and allergic disease. The known benefits of vaccination currently outweigh the unproved risk of allergic disease.

Objective: The internal branch of the superior laryngeal nerve (ibSLN) conveys impulses for the laryngeal cough reflex, which protects the laryngeal aditus and prevents the development of aspiration pneumonia. The purpose of this study was to determine the effect of bilateral anesthesia of the ibSLN on the cough reflex after inhalation of a nebulized chemoirritant solution of tartaric acid., Design: Prospective, clinical investigation., Setting: Outpatient., Participants: Nine healthy volunteers., Interventions: Bilateral injections of 2% lidocaine solution without epinephrine into the paraglottic space containing the ibSLN., Main Outcome Measures: The tidal volume after inhalation of a nebulized 20% tartaric acid solution and forced vital capacity (FVC) were measured before and after injection. Data were analyzed using the Wilcoxon signed ranks, Mann-Whitney, and sign tests., Results: Complete anesthesia of the ibSLN abolished the laryngeal cough reflex. Postinjection tidal volumes were significantly lower than preinjection volumes (p<.01). The decrease in tidal volumes for six subjects with complete bilateral anesthesia was significantly larger than the decrease in tidal volumes for three subjects with partial anesthesia (p<.05). FVC in both the six subjects with complete bilateral anesthesia and the three subjects with partial anesthesia did not significantly change from preinjection to postinjection. None of the subjects in this study had complications or adverse respiratory sequelae., Conclusion: Tartaric acid-induced cough may be useful in assessing the integrity of the laryngeal cough reflex after anesthesia or in patients with neurologic injury who are at risk of developing aspiration pneumonia. It may also be useful in making the decision whether to resume oral feeding.

Objective: To determine the effectiveness of a new reflex cough test, using nebulized tartaric acid, in the evaluation of the laryngeal cough reflex and the development of aspiration pneumonia., Study Design: In this two-phase study, the cough test assessed the cough reflex in 161 stroke subjects. Phase 1 was a double-blinded prospective study of 40 subjects scheduled to have both modified barium swallow and the reflex cough test. Phase 1 subjects with an abnormal cough test showed an increased pneumonia incidence, and therefore, phase 2 was not blinded. In phase 2, 121 subjects were evaluated using the cough test; 38 received a modified barium swallow. Test results were compared using the Fisher exact test., Results: A total of 131 subjects from both phases had a normal reflex cough test; none developed pneumonia (p < .01). Thirty subjects from both phases had abnormal reflex cough test results; 5 developed pneumonia. Modified barium swallow findings did not reliably indicate the risk for developing pneumonia. Specificity of a normal reflex cough test was 100%., Conclusion: The reflex cough test reliably evaluated the laryngeal cough reflex and the associated risk of developing aspiration pneumonia in stroke patients. Testing the laryngeal cough reflex may significantly reduce morbidity, mortality, and costs in stroke patients.

A growing body of research confirms the existence of a powerful connection between socioeconomic status and health. This research has implications for both clinical practice and public policy and deserves to be more widely understood by physicians. Absolute poverty, which implies a lack of resources deemed necessary for survival, is self-evidently associated with poor health, particularly in less developed countries. Over the past two decades, economic decline or stagnation has reduced the incomes of 1.6 billion people. Strong evidence now indicates that relative poverty, which is defined in relation to the average resources available in a society, is also a major determinant of health in industrialized countries. For example, persons in U.S. states with income distributions that are more equitable have longer life expectancies than persons in less egalitarian states. There are numerous possible approaches to improving the health of poor populations. The most essential task is to ensure the satisfaction of basic human needs: shelter, clean air, safe drinking water, and adequate nutrition. Other approaches include reducing barriers to the adoption of healthier modes of living and improving access to appropriate and effective health and social services. Physicians as clinicians, educators, research scientists, and advocates for policy change can contribute to all of these approaches. Physicians and other health professionals should understand poverty and its effects on health and should endeavor to influence policymakers nationally and internationally to reduce the burden of ill health that is a consequence of poverty.

Mortality from asthma in Chicago is among the highest in the nation for 5-34-year-olds. Data for all hospitalizations among Chicago residents less than 35 years of age were examined to define characteristics that may be contributing to morbidity and mortality from asthma. From 1987 through 1989, the average annual age-adjusted hospitalization rate among persons less than 35 years of age in Chicago was 3.57 per 1000 persons. There were significant associations of community hospitalization rate with median income (r = -0.61, p < 0.001) and with proportion of community asthma hospitalizations using Medicaid, Medicare, or self-payment (r = 0.69, p < 0.001). Among 18-34-year-old men, asthma hospitalization rates for Medicaid recipients were 17.4-34.1-fold higher than among men using other forms of insurance. Asthma admissions using Medicaid, Medicare, or self-payment were more likely than those using other forms of insurance to present through the emergency department (79.3% and 66.4%, respectively, p < 0.001) and be discharged against medical advice (1.8% vs. 0.7%, respectively, p < 0.001). These data suggest that differential access to or utilization of health care may be contributing to asthma morbidity in Chicago.

Fever was unchanged by chemotherapy in ten patients with bacteriologically positive pulmonary tuberculosis. Blood cultures were positive for Gram-positive organisms in six patients and for Gram-negative organisms in four patients. The same organism was present in sputa and blood in six patients and in urine and blood in two patients. Leukocytosis was not found, and roentgenographic findings did not suggest superinfection. Nine of the ten patients survived. Blood cultures must be obtained in patients with pulmonary tuberculosis whose fever is not altered by antituberculous chemotherapy, so that concomitant septicemia is not neglected.