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  1. Obstructive sleep apnoea syndrome in children

    Directory of Open Access Journals (Sweden)

    Anna Włodarska

    2016-09-01

    Full Text Available Sleep-related breathing disorders in children are a clinical problem which is more and more often diagnosed by doctors nowadays. They can be the basis for diagnosing obstructive sleep apnoea syndrome that causes a number of complications: lowering the quality of life, behavioural problems, complications involving cardiovascular system. The incidence of obstructive sleep apnoea syndrome in the paediatric population is estimated to be at the level of 2%. The symptoms of obstructive sleep apnoea syndrome can be divided into daytime and night ones. Night symptoms in children include: snoring, apnoea, breathing with open mouth (both during the day and at night, dry tongue and mouth during sleep, agitated sleep in unnatural positions. Among daytime symptoms of obstructive sleep apnoea syndrome there are: irritability, aggressiveness, hyperactivity, attention deficit disorder, delayed development and growth pattern (mainly failure to thrive, learning problems, morning headaches. Parents often do not connect the night and daytime symptoms with the possible development of obstructive sleep apnoea syndrome in their children. The main predisposing factor of obstructive sleep apnoea syndrome in children is adenotonsillar hypertrophy. Effective and in most cases preferred treatment for the management of obstructive sleep apnoea syndrome in children is adenotonsillectomy. Polysomnography and polygraphy are diagnostic tools helpful in the study of sleep-related disorders. The objective of this study was to systematise the knowledge on the epidemiology, aetiology, clinical image and prevention of obstructive sleep apnoea syndrome in children.

  2. Positional therapy in sleep apnoea - one fits all? What determines success in positional therapy in sleep apnoea syndrome.

    Directory of Open Access Journals (Sweden)

    Natascha Troester

    Full Text Available Positional therapy is a simple means of therapy in sleep apnoea syndrome, but due to controversial or lacking evidence, it is not widely accepted as appropriate treatment. In this study, we analysed data to positional therapy with regard to successful reduction of AHI and predictors of success.All consecutive patients undergoing polysomnography between 2007 and 2011 were analysed. We used a strict definition of positional sleep apnoea syndrome (supine-exclusive sleep apnoea syndrome and of therapy used. Patients underwent polysomnography initially and during follow-up.1275 patients were evaluated, 112 of which had supine-exclusive sleep apnoea syndrome (AHI 5-66/h, median 13/h, 105 received positional therapy. With this treatment alone 75% (70/105 reached an AHI <5/h, in the follow-up 1 year later 37% (37/105 of these still had AHI<5/h, 46% (43/105 yielded an AHI between 5 and 10/h. Nine patient switched to APAP due to deterioration, 3 wanted to try APAP due to comfort reasons. At the last follow-up, 32% patients (34/105 were still on positional therapy with AHI <5/h. BMI was a predictor for successful reduction of AHI, but success was independent of sex, the presence of obstructive versus central sleep apnoea, severity of sleep apnoea syndrome or co-morbidities.Positional therapy may be a promising therapy option for patients with positional sleep apnoea. With appropriate adherence it yields a reasonable success rate in the clinical routine.

  3. Snoring and obstructive sleep apnoea syndrome among ...

    African Journals Online (AJOL)

    Background: Obstructive sleep apnoea (OSA) syndrome is a common disorder in the community. Association between hypertension and sleep apnoea and /or snoring has been described. The Berlin questionnaire is a validated instrument that is used to identify individuals who are at risk for OSA. The study aim to describe ...

  4. Compliance with CPAP therapy in patients with the sleep apnoea/hypopnoea syndrome.

    OpenAIRE

    Engleman, H. M.; Martin, S. E.; Douglas, N. J.

    1994-01-01

    BACKGROUND--Continuous positive airway pressure (CPAP) therapy is the treatment of choice for the sleep apnoea/hypopnoea syndrome. Compliance with this relatively obtrusive therapy has not been well studied. METHODS--Usage of CPAP was investigated in 54 patients with sleep apnoea/hypopnoea syndrome (median 36 (range 7-129) apnoeas + hypopnoeas/hour slept) over the first 1-3 months after starting CPAP therapy. In all cases CPAP usage was monitored by hidden time clocks that indicated for how l...

  5. Obstructive sleep apnoea/hypopnoea syndrome in adults with Down syndrome

    OpenAIRE

    Hill, Elizabeth A.

    2016-01-01

    Key points Adults with Down syndrome are predisposed to obstructive sleep apnoea/hypopnoea syndrome (OSAHS) due to overlap between the Down syndrome phenotype and OSAHS risk factors. The prevalence of OSAHS in adults with Down syndrome is estimated at 35?42%. This is up to ten-times higher than in the general adult population. Symptoms of OSAHS, including behavioural and emotional disturbances as well as standard symptoms such as sleepiness, should be monitored as part of regular health surve...

  6. Medico-legal implications of sleep apnoea syndrome: Driving license regulations in Europe

    DEFF Research Database (Denmark)

    Alonderis, A.; Barbee, F.; Bonsignore, M.

    2008-01-01

    Background: Sleep apnoea syndrome (SAS), one of the main medical causes of excessive daytime sleepiness, has been shown to be a risk factor for traffic accidents. Treating SAS results in a normalized rate of traffic accidents. As part of the COST Action B-26, we looked at driving license regulati......Background: Sleep apnoea syndrome (SAS), one of the main medical causes of excessive daytime sleepiness, has been shown to be a risk factor for traffic accidents. Treating SAS results in a normalized rate of traffic accidents. As part of the COST Action B-26, we looked at driving license...... sleep apnoea syndrome is mentioned in 10 countries. A patient with untreated sleep apnoea is always considered unfit to drive. To recover the driving capacity, seven countries rely on a physician's medical certificate based on symptom control and compliance with therapy, whereas in two countries...... it is up to the patient to decide (on his doctor's advice) to drive again. Only FR requires a normalized electroencephalography (EEG)-based Maintenance of Wakefulness Test for professional drivers. Rare conditions (e.g., narcolepsy) are considered a driving safety risk more frequently than sleep apnoea...

  7. Severe obstructive sleep apnoea syndrome in an adult patient with Laron syndrome.

    Science.gov (United States)

    Dagan, Y; Abadi, J; Lifschitz, A; Laron, Z

    2001-08-01

    A 68 year old patient with Laron syndrome (primary growth hormone (GH) resistance-insensitivity due to a molecular defect of the GH receptor) and severe obstructive sleep apnoea syndrome is described. Treatment with continuous positive air pressure therapy resulted in improved nocturnal sleep, daytime alertness and cognitive functions. Copyright 2001 Harcourt Publishers Ltd.

  8. Is there a difference between the STOP-BANG and the Berlin Obstructive Sleep Apnoea Syndrome questionnaires for determining respiratory complications during the perioperative period?

    Science.gov (United States)

    Gokay, Pervin; Tastan, Sevinc; Orhan, Mehmet Emin

    2016-05-01

    This study aimed to compare the efficiency of the STOP-BANG and Berlin Obstructive Sleep Apnoea Syndrome questionnaires for evaluating potential respiratory complications during the perioperative period. Questionnaires that are used to determine obstructive sleep apnoea risk are not widely used for surgical patients. Among the questionnaires that are commonly used for obstructive sleep apnoea screening, it remains unclear whether the STOP-BANG or Berlin Obstructive Sleep Apnoea Syndrome questionnaire is more effective in terms of ease of use, usage period and diagnosis of surgical patients with obstructive sleep apnoea risk. This study was designed as a descriptive and prospective study. The study included 126 patients over 18 years of age who were American Society of Anesthesiologists classification class I-II and underwent laparoscopic cholecystectomy. To determine the potential obstructive sleep apnoea syndrome risk, the STOP-BANG and Berlin questionnaires were administered. Respiratory complications were then observed during the perioperative period. During intubation and extubation, we observed statistically significant differences in difficult intubation, difficult facemask ventilation and desaturation frequency between the high- and low-risk groups for obstructive sleep apnoea syndrome, as determined by the STOP-BANG questionnaire. During extubation, statistically significant differences in coughing, breath-holding and desaturation frequency were observed between the high-risk and low-risk groups, according to the Berlin questionnaire. In the post-anaesthesia care unit, both questionnaires found statistically significant differences between the low- and high-risk groups. Obstructive sleep apnoea syndrome screening questionnaires administered during the preoperative period are useful for predicting perioperative respiratory complications. It may be most useful to administer the STOP-BANG questionnaire as the initial evaluation. Questionnaires may be used to

  9. Craniofacial abnormalities and their relevance for sleep apnoea syndrome aetiopathogenesis in acromegaly

    Czech Academy of Sciences Publication Activity Database

    Dostálová, S.; Šonka, K.; Šmahel, Zbyněk; Weiss, V.; Marek, J.; Hořínek, D.

    2001-01-01

    Roč. 144, - (2001), s. 491-497 ISSN 0804-4643 R&D Projects: GA MZd IZ3575 Institutional research plan: CEZ:AV0Z5039906 Keywords : apnoea syndrome Subject RIV: AC - Archeology, Anthropology, Ethnology Impact factor: 2.133, year: 2001

  10. Sleep apnoea syndrome and 10-year cardiovascular risk in females with type 2 diabetes: relationship with insulin secretion and insulin resistance.

    Science.gov (United States)

    Hermans, Michel P; Ahn, Sylvie A; Mahadeb, Yovan P; Rousseau, Michel F

    2013-03-01

    Obstructive sleep apnoea syndrome (OSAS) is a risk factor for type 2 diabetes mellitus (T2DM) and promotes cardiovascular events, especially in men. The prevalence of sleep apnoea and its association with microvascular and macrovascular diseases and glycaemic control are poorly documented in T2DM women. A total of 305 T2DM women were sleep apnoea diagnosed through (hetero)anamnesis, Epworth's score, oximetry and polysomnography. Sleep apnoea[+] (n = 25) were compared with sleep apnoea[-] (n = 280) regarding cardiovascular risk factors, glucose homeostasis, micro/macrovascular complications and the United Kingdom Prospective Diabetes Study (UKPDS) 10-year risk. Mean (1 SD) age was 66 (12) years, diabetes duration 15 (9) years, sleep apnoea prevalence 8.2% and metabolic syndrome 86%. There were no differences in age, diabetes duration, education, smoking and blood pressure between groups. Sleep apnoea[+] had significantly higher values of body mass index, waist, relative/absolute fat, conicity, visceral fat (all p Women with sleep apnoea had higher UKPDS risk of CAD: 18 (11)% versus 12 (10)% (p = 0.0136). Prevalent micro/macrovascular complications were not different between groups. Sleep apnoea, a frequent comorbidity of T2DM women, is associated with central fat, atherogenic dyslipidaemia, inflammation, worsening β-cell function, poorer glycaemic control and coronary artery disease risk. Sleep apnoea may increase residual vascular risk for microvascular and macrovascular events in T2DM women. Copyright © 2013 John Wiley & Sons, Ltd.

  11. Metabolic aspects of obstructive sleep apnoea syndrome

    Directory of Open Access Journals (Sweden)

    M. R. Bonsignore

    2009-06-01

    Full Text Available Insulin resistance is often associated with obstructive sleep apnoea syndrome (OSAS and could contribute to cardiovascular risk in OSAS. Sleep loss and intermittent hypoxia could contribute to the pathogenesis of the metabolic alterations associated with obesity, a common feature of OSAS. The biology of the adipocyte is being increasingly studied, and it has been found that hypoxia negatively affects adipocyte function. In November 2007, the European Respiratory Society and two EU COST Actions (Cardiovascular risk in OSAS (B26 and Adipose tissue and the metabolic syndrome (BM0602, held a Research Seminar in Düsseldorf, Germany, to discuss the following: 1 the effects of hypoxia on glucose metabolism and adipocyte function; 2 the role of inflammatory activation in OSAS and obesity; 3 the alarming rates of obesity and OSAS in children; 4 the harmful effects of the metabolic syndrome in OSAS; 5 the effects of OSAS treatment on metabolic variables; and 6 the relationship between daytime sleepiness and hormonal and inflammatory responses. Insulin resistance in skeletal muscle, the role of the endocannabinoid system and novel pharmacological approaches to treat insulin resistance were also discussed. As obesity and hypoxia could be the basic links between OSAS and adipocyte dysfunction, further research is needed to translate these new data into clinical practice.

  12. Genetic aspects of hypertension and metabolic disease in the obstructive sleep apnoea-hypopnoea syndrome

    DEFF Research Database (Denmark)

    Riha, R.L.; Diefenbach, K.; Jennum, P.

    2008-01-01

    Though it has long been recognised that there is a hereditary component to the obstructive steep apnoea/hypopnoea syndrome (OSAHS), identifying its genetic basis remains elusive. Hypertension and metabolic syndrome, Like OSAHS, are polygenic disorders, physiologically complex and the product...... phenotyping, which has hampered genetic dissection of these diseases; in addition, sleep-disordered breathing has not been factored into most studies dealing with essential hypertension or metabolic syndrome. Genome-wide scans have yielded inconsistent results in all three disorders under discussion...... for the expression of cardiovascular disease and metabolic syndrome in the context of OSAHS. (C) 2007 Elsevier Ltd. All rights reserved Udgivelsesdato: 2008/2...

  13. The obstructive sleep apnoea syndrome in adolescents.

    Science.gov (United States)

    Marcus, Carole L; Keenan, Brendan T; Huang, Jingtao; Yuan, Haibo; Pinto, Swaroop; Bradford, Ruth M; Kim, Christopher; Bagchi, Sheila; Comyn, Francois-Louis; Wang, Stephen; Tapia, Ignacio E; Maislin, Greg; Cielo, Christopher M; Traylor, Joel; Torigian, Drew A; Schwab, Richard J

    2017-08-01

    The obstructive sleep apnoea syndrome (OSAS) results from a combination of structural and neuromotor factors; however, the relative contributions of these factors have not been studied during the important developmental phase of adolescence. We hypothesised that adenotonsillar volume (ATV), nasopharyngeal airway volume (NPAV), upper airway critical closing pressure (Pcrit) in the hypotonic and activated neuromotor states, upper airway electromyographic response to subatmospheric pressure and the ventilatory response to CO 2 during sleep would be major predictors of OSAS risk. 42 obese adolescents with OSAS and 37 weight-matched controls underwent upper airway MRI, measurements of Pcrit, genioglossal electromyography and ventilatory response to CO 2 during wakefulness and sleep. ATV, NPAV, activated and hypotonic Pcrit, genioglossal electromyography and ventilatory response to CO 2 during sleep were all associated with OSAS risk. Multivariate models adjusted for age, gender, body mass index and race indicated that ATV, NPAV and activated Pcrit each independently affected apnoea risk in adolescents; genioglossal electromyography was independently associated in a reduced sample. There was significant interaction between NPAV and activated Pcrit (p=0.021), with activated Pcrit more strongly associated with OSAS in adolescents with larger NPAVs and NPAV more strongly associated with OSAS in adolescents with more negative activated closing pressure. OSAS in adolescents is mediated by a combination of anatomic (ATV, NPAV) and neuromotor factors (activated Pcrit). This may have important implications for the management of OSAS in adolescents. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.

  14. Management of the obstructive sleep apnoea syndrome: More knowledge required for an optimal choice of treatment modality

    NARCIS (Netherlands)

    Hoekema, A

    2006-01-01

    In the management of the obstructive sleep apnoea syndrome (OSAS), clinicians may consider various conservative, non-invasive and surgical treatment modalities. Continuous positive airway pressure (CPAP) is regarded as the treatment of choice for, especially, moderate to severe OSAS. However, due to

  15. Expiratory timing in obstructive sleep apnoeas.

    Science.gov (United States)

    Cibella, F; Marrone, O; Sanci, S; Bellia, V; Bonsignore, G

    1990-03-01

    Diaphragmatic electromyogram was recorded during NREM sleep in 4 patients affected by obstructive sleep apnoea (OSA) syndrome in order to evaluate the behaviour of expiratory time (TE) in the course of the obstructive apnoea-ventilation cycle. The two components of TE, i.e. time of post-inspiratory inspiratory activity (TPIIA) and time of expiratory phase 2 (TE2) were separately analysed. TPIIA showed a short duration, with only minor variations, within the apnoea, while its duration was more variable and longer in the interapnoeic periods: the longest TPIIA values were associated with the highest inspiratory volumes in the same breaths. This behaviour seemed regulated according to the need of a more or less effective expiratory flow braking, probably as a result of pulmonary stretch receptors discharge. Conversely TE2 showed a continuous gradual modulation, progressively increasing in the pre-apnoeic period, decreasing during the apnoea and increasing in the post-apnoeic period: these TE2 variations seemed related to oscillations in chemical drive. These data show that TE in the obstructive apnoea-ventilation cycle results from a different modulation in its two components and suggest that both mechanical and chemical influences play a role in its overall duration.

  16. The role of nasal CPAP in obstructive sleep apnoea syndrome due to mandibular hypoplasia.

    LENUS (Irish Health Repository)

    Miller, Stanley D W

    2012-02-01

    Melnick Needles syndrome (MNS), Treacher Collins syndrome (TCS) and Pierre Robin syndrome (PRS) are congenital abnormalities with characteristic facial appearances that include micrognathia. A 20-year-old girl with MNS, a 16-year-old boy with TCS and a 12-year-old girl with PRS attended the sleep apnoea clinic at our institution at different times. Diagnostic sleep studies were initially performed on all three patients to confirm the diagnosis of obstructive sleep apnoea syndrome (OSAS). They subsequently commenced nasal CPAP (nCPAP) treatment and their progress was followed. A limited sleep study on the patient with MNS demonstrated moderate\\/severe OSAS with an AHI of 33 events\\/h. Commencement of nCPAP resulted in symptomatic improvement. Overnight oximetry in the patient with TCS showed repeated desaturation to SpO2<90%. Subsequent treatment by nCPAP almost completely abolished the desaturation events. Overnight polysomnography in the patient with PRS demonstrated severe OSAS with an AHI of 49 events\\/h. After 3 years of nCPAP therapy, this patient requested discontinuation of treatment. Subsequent polysomnography without nCPAP revealed an AHI of <5 events\\/h. The use of nCPAP in the patients with MNS and TCS resulted in effective control of their sleep abnormalities. Mandibular growth and enlargement of the posterior airway space led to resolution of OSAS in the patient with PRS. There is a definite role for nCPAP therapy in patients with congenital micrognathia and OSAS. The use of nCPAP may obviate the need for more invasive corrective surgery for OSAS and is not necessarily a life-long requirement.

  17. The development of a screening questionnaire for obstructive sleep apnoea in children with Down syndrome

    Directory of Open Access Journals (Sweden)

    Emma eSanders

    2015-10-01

    Full Text Available Obstructive sleep apnoea is a condition which affects an estimated 50% of children with Down syndrome, particularly in their early years. It can cause serious sequelae in affected children but may not be recognised by parents or health professionals. Routine screening has been recommended in some countries but is not standard practice. There are no validated questionnaire based tools available to screen this population of children for this particular sleep-related disorder. Using existing validated sleep questionnaire items, we have developed a questionnaire to screen children with Down syndrome up to 6 years of age for obstructive sleep apnoea, which corresponds with the recommendations made in UK national guidelines. This paper describes these first steps in demonstrating content validity for a new questionnaire which will be subject to further in-depth psychometric analysis. Relevance, clarity and age-appropriateness was rated for 33 items using a content review questionnaire by a group of 18 health professionals with expertise in respiratory paediatrics, neurodevelopmental paediatrics and sleep physiology. The content validity index was calculated for individual items and contributed to decisions about item inclusion. Scale level content validity index for the modified questionnaire of 14 items was at an accepted level of 0.78. Two parents of children with Down syndrome took part in cognitive interviews after completing the modified questionnaire. We describe the development of this 14 item questionnaire to screen for OSA in children with DS from infancy to 6 years.

  18. Obstructive sleep apnoea syndrome in children and anaesthesia

    Directory of Open Access Journals (Sweden)

    A Rudra

    2010-01-01

    Full Text Available Obstructive sleep apnoea syndrome (OSAS is a common medical disorder among adults, which is increasingly being recognized in children too. It is a breathing disorder characterized by upper airway obstruction with or without intermittent complete obstruction that disrupts normal breathing during sleep. Anatomical and neuromuscular disorders are mainly responsible for this disorder. This disorder leads to a state of chronic hypoxemia, which has significant cardiac, pulmonary and central nervous system implications. Diagnosis of OSAS is based on thorough history and clinical examination along with appropriate sleep studies including polysomnography. The mainstay of treatment of paediatric OSAS is adenotonsillectomy. Good anaesthetic practice in Paediatric patients with OSAS revolves around good and ideal airway management. Early detection of airway obstruction, intense monitoring to warn of impending airway problems and appropriate and early intervention of airway compromise are good anaesthetic practices. Coexisting medical problems should be adequately addressed and safe analgesic techniques in the perioperative period go towards improving outcomes in patients with paediatric OSAS.

  19. Sleep apnoea during upper respiratory infection and metabolic alkalosis in infancy.

    Science.gov (United States)

    Abreu e Silva, F A; MacFadyen, U M; Williams, A; Simpson, H

    1986-01-01

    Three to four hour polygraphic sleep studies were carried out in 10 infants, five with upper respiratory infection and five with metabolic alkalosis secondary to vomiting during and after recovery from illness. During upper respiratory infection, the main abnormality detected was brief (greater than 3 less than 6 seconds) or prolonged (greater than 6 seconds) attacks of obstructive apnoea. Other indices of apnoea were similar to recovery data. Gross body movements were also increased. In infants with metabolic alkalosis indices of central apnoea were significantly increased when compared with recovery or case control data. Prolonged (greater than 15 seconds) attacks of central apnoea and obstructive apnoea (greater than 6 seconds) were only observed during illness. Gross body movements and periodic breathing were also increased. These findings suggest that the functional consequences of apparently 'mild' illnesses in young infants may be greater than is generally suspected and perhaps relevant to mechanism(s) of death in sudden infant death syndrome. PMID:3789786

  20. Can you die from obstructive sleep apnoea syndrome (OSAS)?

    LENUS (Irish Health Repository)

    O'Carroll, G

    2015-02-01

    Studies suggest an independent association between Obstructive Sleep Apnoea Syndrome (OSAS) and cardiovascular death. The purpose of our study is to examine doctors\\' awareness of this association and to determine whether this correlates with recording of OSAS on death certificates. We contacted the Central Statistics Office (CSO) and obtained relevant mention of OSAS on death certificates. We surveyed doctors on their view of OSAS-related deaths, CSO data from 2008-2011 reveal two deaths with OSAS documented as a direct cause and 52 deaths with OSAS as a contributory cause. Seventy-five doctors\\' surveyed (41%) believe OSAS can be a direct cause of death and 177 (96%) believe OSAS can be an indirect cause of death. Only 22 (12%) had putdown OSAS as a cause of death. OSAS is seldom recorded on death certificates. This is at odds with epidemiological forecasts and contrary to an opinion poll from a selection of doctors.

  1. Obstructive Sleep Apnoea

    African Journals Online (AJOL)

    Cheyne-Stokes respiration), obstructive sleep apnoea and mixed or complex sleep apnoea.1. Obstructive sleep apnoea (OSA) is the most common of these three disorders and is defined as airway obstruction during sleep, accompanied by at least ...

  2. [The Overlap Syndrome: association of COPD and Obstructive Sleep Apnoea].

    Science.gov (United States)

    Weitzenblum, E; Chaouat, A; Kessler, R; Canuet, M; Hirschi, S

    2010-04-01

    Chronic obstructive pulmonary disease (COPD) and the obstructive sleep apnoea-hypopnoea syndrome (OSAHS) are both common diseases affecting respectively 10 and 5% of the adult population over 40 years of age. Their coexistence, which is denominated "Overlap Syndrome", can be expected to occur in about 0.5% of this population. Two recent epidemiologic studies have shown that the prevalence of OSAHS is not higher in COPD than in the general population, and that the coexistence of the two conditions is due to chance and not through a pathophysiological linkage. Patients with "overlap" have a higher risk of sleep-related O(2) desaturation than do patients with COPD alone and the same degree of bronchial obstruction. They have an increased risk of developing hypercapnic respiratory failure and pulmonary hypertension when compared with patients with OSAHS alone and with patients with "usual" COPD. In patients with overlap, hypoxaemia, hypercapnia, and pulmonary hypertension can be observed in the presence of mild to moderate bronchial obstruction, which is different from "usual" COPD. Treatment of the overlap syndrome consists of nasal continuous positive airway pressure or nocturnal non-invasive ventilation (NIV), with or without nocturnal O(2). Patients who are markedly hypoxaemic during the daytime (PaO(2)<55-60 mmHg) should be given conventional long-term O(2) therapy in addition to nocturnal ventilation. Copyright 2010 SPLF. Published by Elsevier Masson SAS. All rights reserved.

  3. Obstructive Sleep Apnoea Syndrome and Weight Loss: Review

    Directory of Open Access Journals (Sweden)

    Douglas C. Cowan

    2012-01-01

    Full Text Available Obstructive sleep apnoea (OSA syndrome is common, and obesity is a major risk factor. Increased peripharyngeal and central adiposity result in increased pharyngeal collapsibility, through increased mechanical loading around the upper airway, reduced tracheal traction on the pharynx, and reduced neuromuscular activity, particularly during sleep. Significant and sustained weight loss, if achieved, is likely to be a useful therapeutic option in the management of OSA and may be attempted by behavioural, pharmacological, and surgical approaches. Behavioural therapy programs that focus on aspects such as dietary intervention, exercise prescription patients and general lifestyle counselling have been tested. Bariatric surgery is an option in the severely obese when nonsurgical measures have failed, and laparoscopic adjustable gastric banding and Roux-en-Y gastric bypass are the most commonly employed techniques in the United Kingdom. Most evidence for efficacy of surgery comes from cohort studies. The role of sibutramine in OSA in the obese patients has been investigated, however, there are concerns regarding associated cardiovascular risk. In this paper the links between obesity and OSA are discussed, and the recent studies evaluating the behavioural, pharmacological and surgical approaches to weight loss in OSA are reviewed.

  4. The diagnosis of obstructive sleep apnoea (Pickwickian syndrome)

    International Nuclear Information System (INIS)

    Stark, P.; Aguilar, E.A. Jr.; Robbins, K.T.

    1984-01-01

    Two patients with obstructive sleep apnoea are described and the value of computer tomography in the diagnosis and follow-up is stressed. Narrowing of the oro-pharynx is a major feature in the diagnosis of this condition and is best demonstrated by CT. (orig.) [de

  5. Ambulatory monitoring in the diagnosis and management of obstructive sleep apnoea syndrome

    Directory of Open Access Journals (Sweden)

    Jaime Corral-Peñafiel

    2013-09-01

    Full Text Available Obstructive sleep apnoea (OSA is a highly prevalent disorder associated with complications such as arterial hypertension, cardiovascular diseases and traffic accidents. The resources allocated for OSA are insufficient and OSA is a significant public health problem. Portable recording devices have been developed for the detection of OSA syndrome and have proved capable of providing an equivalent diagnosis to in-laboratory polysomnography (PSG, at least in patients with a high pre-test probability of OSA syndrome. PSG becomes important in patients who have symptoms and certain comorbidities such as chronic obstructive pulmonary disease or stroke, as well as in patients with a clinical history suggesting a different sleep disorder. Continuous positive airway pressure is the most effective treatment in OSA. Ambulatory monitoring of the therapeutic modalities has been evaluated to enhance the care process and reduce costs compared to the conventional approach, without sacrificing efficiency. This review evaluates the role of portable monitoring devices in the diagnostic process of OSA and the search for alternative strategies based on ambulatory management protocols.

  6. Sleep. 5: Driving and automobile crashes in patients with obstructive sleep apnoea/hypopnoea syndrome.

    Science.gov (United States)

    George, C F P

    2004-09-01

    Driving is a complex task involving distinct cognitive, perceptual, motor, and decision making skills. After placing the vehicle on the road, the driver must constantly survey the ever changing roadway environment to keep the vehicle in the lane and moving at an appropriate safe speed. This surveillance involves two distinct visual tasks: estimating and responding to the oncoming curvature and controlling lane position. Driving is therefore a divided attention task involving speed and lane control as well as monitoring. To do this in a safe manner requires careful attention and alertness which can be problematic for patients with obstructive sleep apnoea/hypopnoea syndrome (OSAHS) or other sleep disorders.

  7. Relationship between obstructive sleep apnoea syndrome and sleep bruxism: a systematic review.

    Science.gov (United States)

    Jokubauskas, L; Baltrušaitytė, A

    2017-02-01

    Obstructive sleep apnoea syndrome (OSAS) is a clinical risk factor for sleep bruxism (SB). Both OSAS and SB are reported to be associated with sleep-related arousal reactions, although no clear causative link has been established. An electronic literature search was conducted of the MEDLINE, ScienceDirect, Wiley Online Library, SAGE Journals and EBSCOhost databases covering the period January 2006 and September 2016. Sequential screenings at the title, abstract and full-text levels were performed. The review included observational studies in the English language with a clearly established aim to assess the relationship between OSAS and SB using full-night PSG. The seven-item quality-assessment tool for experimental bruxism studies was used to assess the methodology across the studies. After a comprehensive screening of titles, abstracts and full texts, only three studies that met the pre-defined criteria were finally included in this systematic review. Two studies gave evidence that OSAS is associated with the occurrence of SB events: (i) SB events frequently occur during micro-arousal events consequent on apnoea-hypopnoea (AH) events and (ii) most SB events occur in temporal conjunction with AH events termination. However, one study did not report a strong association between AH and SB events. It can be concluded that there are not enough scientific data to define a clear causative link between OSAS and SB. However, they appear to share common clinical features. Further studies should focus on the intermediate mechanisms between respiratory and SB events. © 2016 John Wiley & Sons Ltd.

  8. Refractory absence epilepsy associated with GLUT-1 deficiency syndrome.

    LENUS (Irish Health Repository)

    Byrne, Susan

    2011-05-01

    GLUT-1 deficiency syndrome (GLUT-1 DS) is a disorder of cerebral glucose transport associated with early infantile epilepsy and microcephaly. We report two boys who presented with refractory absence epilepsy associated with hypoglycorrhachia, both of whom have genetically confirmed GLUT-1 DS. We propose that these children serve to expand the phenotype of GLUT-1 DS and suggest that this condition should be considered as a cause of refractory absence seizures in childhood.

  9. Obstructive sleep apnoea syndrome as a cause of road traffic accidents.

    Science.gov (United States)

    Aguiar, M; Valença, J; Felizardo, M; Caeiro, F; Moreira, S; Staats, R; Bugalho de Almeida, A A

    2009-01-01

    Several studies have demonstrated that obstructive sleep apnoea syndrome (OSAS) patients have a higher rate of road traffic accidents. Our study aimed to analyse any differences in OSAS patients between those who reported having had road traffic accidents and/or near misses and those who did not. We studied 163 patients with OSAS (apnoea- hypopnoea index (AHI)>10/h) diagnosed using nocturnal polysomnography (NPSG), all drivers, 18.4% of whom drove for a living. Patients were asked at their first clinical interview to self-report road traffic accidents and/or near misses over the past 3 years which had been caused by abnormal daytime drowsiness. This allowed patients to be divided into two groups, those who had had road traffic accidents and/or near misses and those who had not. Both were compared as to age, body mass index (BMI), Epworth Sleepiness Scale (ESS), daytime PaO2 and PaCO2, Functional Outcomes of Sleep Questionnaire (FOSQ) test and NPSG data. This latter was total sleep time (TTS), sleep efficiency, sleep stages, arousal index (ARI), AHI, minimal and average SaO2, % of time with SaO2 TDAH) (T test). Group I (no road traffic accidents) No=89 patients; group II (road traffic accidents) No=74 patients. Age (years) was 57.6+/-11.8 vs. 54.7+/-10.9 (ns); male gender, 75% vs. 78.4%; ESS, 12.3+/-5.4 vs. 17.6+/-4.3 (pTDAH (minutes), 98.5+/-63.7 vs. 133.3+/-83.2 (p=0,005). In our experience patients who had road traffic accidents and/or near misses had a more severe OSAS, with higher AHI, excessive daytime sleepiness and lower quality of life.

  10. The influence of the lateral pharyngeal wall anatomy on snoring and sleep apnoea.

    Science.gov (United States)

    Korhan, Ibrahim; Gode, Sercan; Midilli, Rasit; Basoglu, Ozen Kacmaz

    2015-02-01

    To elucidate the variations of the lateral pharyngeal wall anatomy on physical examination and to assess the clinical importance of the examination of the lateral pharyngeal wall on the presence and severity of obstructive sleep apnoea syndrome. The cross-sectional study was conducted at Ege University Medical School, Izmir, Turkey, between May 2010 and April 2011. The patients were divided into four equal groups: Group 1--snoring without apnoea (age 20-40); Group 2--snoring without apnoea (age 40-60); Group 3--apnoea-hypopnoea index 30/hr. Calibrated oropharynx pictures were taken. Distance between palatoglossal and palatopharyngeal arches, height of palatoglossal and palatopharyngeal arches, uvula width, uvula length and distance between tonsils were measured. SPSS 17 was used for statistical analysis. Of the 80 patients in the study, 44 (55%) were men. Mean distance between palatopharyngeal and palatoglossal arches were 1.55 ± 0.34 cm and 2.70 ± 0.43 cm respectively. Mean height of palatopharyngeal and palatoglossal arches were 0.60 ± 0.21 cm and 1.37 ± 0.36 cm respectively (p > 0.05). Mean uvula width and uvula length were 0.80 ± 0.12cm and 1.25 ± 0.27 cm respectively (p > 0.05). Mean distance between tonsils was 2.24 ± 0.56 cm (p > 0.05). Distance between palatopharyngeal arches was significantly different between groups 3 and 4 (p < 0.05). Palatopharyngeal arch anatomy was found to be significantly associated with obstructive sleep apnoea syndrome severity, especially in patients with normal or small tonsil size. Patients with the palatopharyngeal arches, which narrow the oropharyngeal inlet more than the tonsils, should further be investigated with polysomnography.

  11. Insufficient evidence to confirm effectiveness of oral appliances in treatment of obstructive sleep apnoea syndrome in children.

    Science.gov (United States)

    Fox, Nigel A

    2007-01-01

    Searches were made using the Cochrane Central Register of Controlled Trials, Medline, Embase, Latin American and Caribbean Health Sciences Literature, Bibliografia Brasileira de Odontologia and SciELO (the Scientific Electronic Library Online). Studies chosen were randomised controlled trials (RCT) or quasi-RCT comparing all types of oral and functional orthopaedic appliances with placebo or no treatment, in children of 15 years old or younger. Data were independently extracted by two review authors. Authors were contacted for additional information. Risk ratios with 95% confidence intervals were calculated for all important dichotomous outcomes. A total of 384 trials were identified, of which only one, reporting results from a total of 23 patients, was suitable for inclusion in the review. Data provided in the published report did not answer all the questions from this review, but did answer some: the results presented favour treatment. At present there is not sufficient evidence to state that oral appliances or functional orthopaedic appliances are effective in the treatment of obstructive sleep apnoea (OSA) syndrome in children. Oral appliances or functional orthopaedic appliances may be helpful in the treatment of children with craniofacial anomalies which are risk factors for apnoea.

  12. Obstructive sleep apnoea syndrome, endothelial function and markers of endothelialization. Changes after CPAP.

    Science.gov (United States)

    Muñoz-Hernandez, Rocio; Vallejo-Vaz, Antonio J; Sanchez Armengol, Angeles; Moreno-Luna, Rafael; Caballero-Eraso, Candela; Macher, Hada C; Villar, Jose; Merino, Ana M; Castell, Javier; Capote, Francisco; Stiefel, Pablo

    2015-01-01

    This study tries to assess the endothelial function in vivo using flow-mediated dilatation (FMD) and several biomarkers of endothelium formation/restoration and damage in patients with obstructive sleep apnoea (OSA) syndrome at baseline and after three months with CPAP therapy. Observational study, before and after CPAP therapy. We studied 30 patients with apnoea/hypopnoea index (AHI) >15/h that were compared with themselves after three months of CPAP therapy. FMD was assessed non-invasively in vivo using the Laser-Doppler flowmetry. Circulating cell-free DNA (cf-DNA) and microparticles (MPs) were measured as markers of endothelial damage and the vascular endothelial growth factor (VEGF) was determined as a marker of endothelial restoration process. After three month with CPAP, FMD significantly increased (1072.26 ± 483.21 vs. 1604.38 ± 915.69 PU, pDNA and MPs significantly decreased (187.93 ± 115.81 vs. 121.28 ± 78.98 pg/ml, p<0.01, and 69.60 ± 62.60 vs. 39.82 ± 22.14 U/μL, p<0.05, respectively) and VEGF levels increased (585.02 ± 246.06 vs. 641.11 ± 212.69 pg/ml, p<0.05). These changes were higher in patients with more severe disease. There was a relationship between markers of damage (r = -0.53, p<0.005) but not between markers of damage and restoration, thus suggesting that both types of markers should be measured together. CPAP therapy improves FMD. This improvement may be related to an increase of endothelial restoration process and a decrease of endothelial damage.

  13. EVALUATION OF CHRONIC OBSTRUCTIVE PULMONARY DISEASE PATIENTS WITH OBSTRUCTIVE SLEEP APNOEA - OVERLAP SYNDROME

    Directory of Open Access Journals (Sweden)

    Vaddadi Sailendra

    2016-09-01

    Full Text Available BACKGROUND The drop in oxygen saturation during sleep is more than during exercise and patients of COPD who spend more time in sleeping. Significant sleep desaturation and the sleep disturbances are greater in overlap syndrome than in OSA alone. The present study is conducted in Gayathri Vidya Parishad Institute of Healthcare and Medical Technology, Visakhapatnam, AP, India, to find the prevalence of obstructive sleep apnoea in the patients with COPD. AIMS The present study was a cross-sectional study prospectively carried out with an aim to evaluate the breathing disorders during sleep in patients with COPD and to correlate these disorders with the stage of the disease. SETTINGS AND DESIGN The study Cohort was constituted by patients of COPD registered into Chest OPD or admitted in Indoor units of Gayathri Vidya Parishad Institute of Healthcare and Medical Technology, Visakhapatnam, AP, India, from July 2014 to May 2016. A total of thirty six consecutive COPD patients who consented to be enrolled into the study were classified into Mild, Moderate and Severe stages based on the Indian Guidelines for the management of COPD. METHODS AND MATERIAL Spirometric evaluation and bronchial reversibility testing was conducted in all the patients. Arterial Blood Gas Analysis was done using ABL3 arterial blood gas analyser (Radiometer, Copenhagen. POLYSOMNOGRAPHY Patients were hooked to Compumedics ProFusion Polysomnographic Machine (Compumedics Private Limited 2001, USA, by standard gold cups/electrodes. Thereafter, the patients were subjected to a full night sleep study (Overnight polysomnography. The electrode and sensor connection system utilises E-series EEG/PSG system in order to record the PSG study. The impedance of electrodes was checked and set to <10. A total of 20 leads were utilised for the study. The various parameters monitored included Electroencephalogram (EEG, Electro-oculogram (EOG, Electrocardiogram (ECG, chin and leg Electromyogram (EMG

  14. Lessons from healthcare utilization in children with obstructive sleep apnoea syndrome.

    Science.gov (United States)

    Martinez-Beneyto, Paz; Soria Checa, Cristina E; Botella-Rocamora, Paloma; Rincon-Piedrahita, Inés; Garcia Callejo, Francisco J; Algarra, Jaime Marco

    Paediatric Obstructive Sleep Apnoea-Hypopnoea Syndrome (OSAS) is a multisystemic condition affecting child's health status that may be investigated analyzing demand for healthcare. to quantify the frequency of medical consultations in children with OSAS over a 5-year period, compared to a healthy population. A longitudinal, case-control, ambispective study was conducted at a hospital pertaining to the national public health system. 69 consecutive children referred for OSAS were recruited with no diseases other than OSAS so that healthcare demand was purely attributed to this condition. Matched healthy control children were selected to compare these data. Data regarding frequency of the medical consultations were obtained over 5 years: the year of the treatment ("Year0"), 1 and 2 years before ("Year -1" and "Year -2" respectively), and 1 and 2 years after treatment ("Year+1" and "Year+2") RESULTS: Frequentation Index (FI), as ratio between the use of health services by OSAS children and healthy controls was 1.89 during Year-2, and 2.15 during Year-1 (Pde Otorrinolaringología y Cirugía de Cabeza y Cuello. All rights reserved.

  15. Cardiovascular disease in obstructive sleep apnoea syndrome: the role of intermittent hypoxia and inflammation.

    LENUS (Irish Health Repository)

    Garvey, J F

    2012-02-01

    There is increasing evidence that intermittent hypoxia plays a role in the development of cardiovascular risk in obstructive sleep apnoea syndrome (OSAS) through the activation of inflammatory pathways. The development of translational models of intermittent hypoxia has allowed investigation of its role in the activation of inflammatory mechanisms and promotion of cardiovascular disease in OSAS. There are noticeable differences in the response to intermittent hypoxia between body tissues but the hypoxia-sensitive transcription factors hypoxia-inducible factor-1 and nuclear factor-kappaB appear to play a key role in mediating the inflammatory and cardiovascular consequences of OSAS. Expanding our understanding of these pathways, the cross-talk between them and the activation of inflammatory mechanisms by intermittent hypoxia in OSAS will provide new avenues of therapeutic opportunity for the disease.

  16. Craniofacial differences according to AHI scores of children with obstructive sleep apnoea syndrome: cephalometric study in 39 patients

    International Nuclear Information System (INIS)

    Oezdemir, Hueseyin; Mahmutyaziciglu, Kamran; Davsancimath, Halit; Guendogdu, Sadi; Altin, Remzi; Kart, Levent; Soeguet, Ayhan; Tomac, Nazan; Cinar, Fikret; Uzun, Lokman

    2004-01-01

    Cephalometry is useful as a screening test for anatomical abnormalities in patients with obstructive sleep apnoea syndrome (OSAS). To evaluate comprehensively the cephalo metric features of children with OSAS, with or without adenotonsillar hypertrophy, and to elucidate the relationship between cephalometric variables and apnoea-hypopnoea index (AHI) severity. The study population consisted of 39 children, aged 4-12 years, with OSAS. Cephalometry was analysed using 11 measurements of the bony structures, their relationships and the size of the airways. Additionally, adenoid and tonsillar hypertrophy were graded. Cranial base angles (BaSN and BaSPNS) were found to correlate with increasing levels of AHI scores (P 0.05). The length of the mandibular plane (GnGo) and the minimal posterior airway space (MPAS) were inversely correlated with AHI scores (P<0.001). There was positive correlation between MPAS and GnGo (r=0.740, P<0.001), and negative correlation between MPAS and gonial angle (ArGoGn) (r=-0.541, P<0.001). There was significant correlation between cephalometric data and adenotonsillar hypertrophy concerning BaSN, BaSPNS, ArGoGn, GnGoH, BaN-GnGo, MPAS, GnGO and MPH. (orig.)

  17. Obstructive sleep apnoea syndrome, endothelial function and markers of endothelialization. Changes after CPAP.

    Directory of Open Access Journals (Sweden)

    Rocio Muñoz-Hernandez

    Full Text Available This study tries to assess the endothelial function in vivo using flow-mediated dilatation (FMD and several biomarkers of endothelium formation/restoration and damage in patients with obstructive sleep apnoea (OSA syndrome at baseline and after three months with CPAP therapy.Observational study, before and after CPAP therapy.We studied 30 patients with apnoea/hypopnoea index (AHI >15/h that were compared with themselves after three months of CPAP therapy. FMD was assessed non-invasively in vivo using the Laser-Doppler flowmetry. Circulating cell-free DNA (cf-DNA and microparticles (MPs were measured as markers of endothelial damage and the vascular endothelial growth factor (VEGF was determined as a marker of endothelial restoration process.After three month with CPAP, FMD significantly increased (1072.26 ± 483.21 vs. 1604.38 ± 915.69 PU, p< 0.005 cf-DNA and MPs significantly decreased (187.93 ± 115.81 vs. 121.28 ± 78.98 pg/ml, p<0.01, and 69.60 ± 62.60 vs. 39.82 ± 22.14 U/μL, p<0.05, respectively and VEGF levels increased (585.02 ± 246.06 vs. 641.11 ± 212.69 pg/ml, p<0.05. These changes were higher in patients with more severe disease. There was a relationship between markers of damage (r = -0.53, p<0.005 but not between markers of damage and restoration, thus suggesting that both types of markers should be measured together.CPAP therapy improves FMD. This improvement may be related to an increase of endothelial restoration process and a decrease of endothelial damage.

  18. Risk of obstructive sleep apnoea syndrome among in-patients at a ...

    African Journals Online (AJOL)

    We screened for risk of OSA among in-patients with severe mental illness to determine its prevalence ... Keywords: obstructive sleep apnoea; severe mental illness; Nigeria, in-patients .... a physical co-morbidity which was hypertension, none.

  19. Gender differences in patients with obesity hypoventilation syndrome.

    Science.gov (United States)

    BaHammam, Ahmed S; Pandi-Perumal, Seithikurippu R; Piper, Amanda; Bahammam, Salman A; Almeneessier, Aljohara S; Olaish, Awad H; Javaheri, Shahrokh

    2016-08-01

    The role of gender and menopause in obstructive sleep apnoea is well known; however, no study has reported the impact of gender on the clinical presentation and the nocturnal respiratory events in patients with obesity hypoventilation syndrome. Therefore, this study prospectively evaluated differences in the clinical characteristics of women and men with obesity hypoventilation syndrome in a large cohort of patients with obstructive sleep apnoea. During the study period, a total of 1973 patients were referred to the sleep clinic with clinical suspicion of obstructive sleep apnoea. All patients underwent overnight polysomnography, during which time spirometry, arterial blood samples and thyroid tests were routinely obtained. Among 1973 consecutive patients, 1693 (617 women) were diagnosed with obstructive sleep apnoea, among whom 144 suffered from obesity hypoventilation syndrome (96 women). The prevalence of obesity hypoventilation syndrome among women and men was 15.6% and 4.5%, respectively (P obesity hypoventilation syndrome were significantly older than men with obesity hypoventilation syndrome (61.5 ± 11.9 years versus 49.1 ± 12.5 years, P differences between genders regarding symptoms, body mass index, spirometric data or daytime PaCO2 , women with obesity hypoventilation syndrome suffered significantly more from hypertension, diabetes and hypothyroidism. The prevalence of obesity hypoventilation syndrome was higher in post-menopausal (21%) compared with pre-menopausal (5.3%) women (P obesity hypoventilation syndrome. In conclusion, this study reported that among subjects referred to the sleep disorders clinic for evaluation of obstructive sleep apnoea, obesity hypoventilation syndrome is more prevalent in women than men, and that women with obesity hypoventilation syndrome suffer from significantly more co-morbidities. Post-menopausal women with obstructive sleep apnoea have the highest prevalence of obesity hypoventilation syndrome. © 2016

  20. Oral appliance therapy versus nasal continuous positive airway pressure in obstructive sleep apnoea syndrome: a randomised, placebo-controlled trial on self-reported symptoms of common sleep disorders and sleep-related problems

    NARCIS (Netherlands)

    Nikolopoulou, M.; Byraki, A.; Ahlberg, J.; Heymans, M. W.; Hamburger, H. L.; de Lange, J.; Lobbezoo, F.; Aarab, G.

    2017-01-01

    Obstructive sleep apnoea syndrome (OSAS) is associated with several sleep disorders and sleep-related problems. Therefore, the aim of this study was to compare the effects of a mandibular advancement device (MAD) with those of nasal continuous positive airway pressure (nCPAP) on self-reported

  1. Inflammatory cardiovascular risk markers in obstructive sleep apnoea syndrome.

    LENUS (Irish Health Repository)

    Ryan, Silke

    2012-02-01

    Obstructive sleep apnoea syndrome (OSAS) represents a highly prevalent disease and is recognized as a major public health burden. Large-scale epidemiological studies have demonstrated an independent relationship between OSAS and various cardiovascular disorders. The pathogenesis of cardiovascular complications in OSAS is not completely understood, but given the complexity of the disorder, a multifactorial etiology is likely. Inflammatory processes have emerged as critical in the pathogenesis of atherosclerosis in general and they mediate many of the stages of atheroma formation. Circulating levels of several markers of inflammation have been associated with future cardiovascular risk. These markers include cell adhesion molecules such as intercellular adhesion molecule-1 (ICAM-1) and selectins, cytokines such as tumour necrosis factor alpha (TNF-alpha) and interleukin 6 (IL-6), chemokines such as IL-8, and C-reactive protein (CRP). There is increasing evidence that inflammatory processes also play a central role in the cardiovascular pathophysiology of OSAS. This is supported by cell culture and animal studies identifying a preferential activation of inflammatory pathways by intermittent hypoxia (IH), the hallmark of OSAS. A number of studies have selectively examined the expression of inflammatory factors in OSAS patients with different conclusions. These different findings may have been contributed to by a number of methodological factors such as small subject numbers, inadequately matched study populations, particularly in terms of body mass index (BMI), and inclusion of patients with pre-existing cardiovascular or metabolic diseases. This review will focus on the potential role of various inflammatory markers in OSAS with a critical analysis of the current literature.

  2. Simulated driving in obstructive sleep apnoea-hypopnoea : effects of oral appliances and continuous positive airway pressure

    NARCIS (Netherlands)

    Hoekema, Aarnoud; Stegenga, Boudewijn; Bakker, Marije; Brouwer, Wiebo H.; de Bont, Lambert G. M.; Wijkstra, Peter J.; van der Hoeven, Johannes H.

    Impaired simulated driving performance has been demonstrated in obstructive sleep apnoea-hypopnoea syndrome (OSAHS) patients. Although continuous positive airway pressure (CPAP) generally improves simulated driving performance, the effects of oral-appliance (OA) therapy are unknown. The aims of this

  3. Screening for Sleep Apnoea in Mild Cognitive Impairment: The Utility of the Multivariable Apnoea Prediction Index

    Directory of Open Access Journals (Sweden)

    Georgina Wilson

    2014-01-01

    Full Text Available Purpose. Mild cognitive impairment (MCI is considered an “at risk” state for dementia and efforts are needed to target modifiable risk factors, of which Obstructive sleep apnoea (OSA is one. This study aims to evaluate the predictive utility of the multivariate apnoea prediction index (MAPI, a patient self-report survey, to assess OSA in MCI. Methods. Thirty-seven participants with MCI and 37 age-matched controls completed the MAPI and underwent polysomnography (PSG. Correlations were used to compare the MAPI and PSG measures including oxygen desaturation index and apnoea-hypopnoea index (AHI. Receiver-operating characteristics (ROC curve analyses were performed using various cut-off scores for apnoea severity. Results. In controls, there was a significant moderate correlation between higher MAPI scores and more severe apnoea (AHI: r=0.47, P=0.017. However, this relationship was not significant in the MCI sample. ROC curve analysis indicated much lower area under the curve (AUC in the MCI sample compared to the controls across all AHI severity cut-off scores. Conclusions. In older people, the MAPI moderately correlates with AHI severity but only in those who are cognitively intact. Development of further screening tools is required in order to accurately screen for OSA in MCI.

  4. Prevalence of metabolic syndrome in a north Indian hospital-based population with obstructive sleep apnoea

    Science.gov (United States)

    Agrawal, Swastik; Sharma, Surendra K.; Sreenivas, Vishnubhatla; Lakshmy, Ramakrishnan

    2011-01-01

    Background & objectives: Obstructive sleep apnoea (OSA) is known to be associated with cardiovascular risk factors and metabolic syndrome (MS). The burden of MS in patients with OSA in India is unknown. We investigated the prevalence of MS and its components in a cross-sectional study in patients with and without OSA in a hospital-based population of a tertiary health care centre in New Delhi, India. Methods: Consecutive patients undergoing overnight polysomnography in the Sleep Laboratory of the Department of Internal Medicine of All India Institute of Medical Sciences (AIIMS) hospital, New Delhi, were studied. Anthropometry and body composition analysis, blood pressure (BP), fasting blood glucose, insulin resistance by homeostasis model assessment (HOMA-IR) and fasting blood lipid profile were measured. MS was defined using the National Cholesterol Education Program Adult treatment panel III criteria, with Asian cut-off values for abdominal obesity. Results: Of the 272 subjects recruited, 187 (82%) had OSA [apnoea-hypopnoea index (AHI)>5 events/h] while 40 (18%) had a normal sleep study. Prevalence of MS in OSA patients was 79 per cent compared to 48 per cent in non-OSA individuals [OR 4.15, (2.05-8.56), P<0.001]. Prevalence of OSA in mild, moderate and severe OSA was 66, 72 and 86 per cent, respectively (P<0.001). Patients with OSA were more likely to have higher BP [OR: 1.06 (1.02-1.11)], fasting insulin [OR: 1.18 (1.05-1.32)], HOMA-IR [OR: 1.61 (1.11-2.33)] and waist circumference [OR: 1.20 (1.13-1.27)]. Interpretation & conclusions: Our findings suggest that OSA is associated with a 4-fold higher occurrence of MS than patients without OSA. The prevalence of MS increases with increasing severity of OSA, therefore, early detection will be beneficial. PMID:22199102

  5. Sleep apnoea in patients with quadriplegia.

    Science.gov (United States)

    McEvoy, R. D.; Mykytyn, I.; Sajkov, D.; Flavell, H.; Marshall, R.; Antic, R.; Thornton, A. T.

    1995-01-01

    BACKGROUND--This study was undertaken to establish the prevalence of, and the factors contributing towards, sleep disordered breathing in patients with quadriplegia. METHODS--Forty representative quadriplegic patients (time since injury > 6 months, injury level C8 and above, Frankel category A, B, or C; mean (SE) age 35.0 (1.7) years) had home sleep studies in which EEG, EOG, submental EMG, body movement, nasal airflow, respiratory effort, and pulse oximetry (SpO2) were measured. Patients reporting post traumatic amnesia of > 24 hours, drug or alcohol abuse or other major medical illness were excluded from the study. A questionnaire on medications and sleep was administered and supine blood pressure, awake SpO2, spirometric values, height, and neck circumference were measured. RESULTS--A pattern of sustained hypoventilation was not observed in any of the patients. Sleep apnoeas and hypopnoeas were, however, common. Eleven patients (27.5%) had a respiratory disturbance index (RDI, apnoeas plus hypopnoeas per hour of sleep) of > or = 15, with nadir SpO2 ranging from 49% to 95%. Twelve of the 40 (30%) had an apnoea index (AI) of > or = 5 and, of these, nine (75%) had predominantly obstructive apnoeas-that is, > 80% of apnoeas were obstructive or mixed. This represents a prevalence of sleep disordered breathing more than twice that observed in normal populations. For the study population RDI correlated with systolic and diastolic blood pressure and neck circumference. RDI was higher in patients who slept supine compared with those in other postures. Daytime sleepiness was a common complaint in the study population and sleep architecture was considerably disturbed with decreased REM sleep and increased stage 1 non-REM sleep. CONCLUSIONS--Sleep disordered breathing is common in quadriplegic patients and sleep disturbance is significant. The predominant type of apnoea is obstructive. As with non-quadriplegic patients with sleep apnoea, sleep disordered breathing in

  6. Lifestyle modification for obstructive sleep apnoea.

    Science.gov (United States)

    Shneerson, J; Wright, J

    2001-01-01

    Obstructive sleep apnoeas are due to transient closure of the upper airway during sleep and merge into hypopnoeas in which the airway narrows, but some airflow continues. They are due to the forces compressing the airway overcoming those which stabilise its patency. The commonest association is obesity in which fatty tissue is deposited around the airway. Exercise has been recommended as a method of losing weight, but other techniques which achieve this are also thought to improve symptoms due to sleep apnoeas. Sleep hygiene may alter the sleep structure and the control of the upper airway during sleep and thus promote its patency. The objectives of this review are to determine whether weight loss, sleep hygiene and exercise are effective in the treatment of obstructive sleep apnoeas. The Cochrane Airways Group Trials Register, MEDLINE, EMBASE, CINAHL and reference lists of review articles have been searched. Randomised, single or double blind placebo controlled, either parallel group or crossover design studies of any of these interventions were to have been included. No completed trials have been identified. No randomised trial data were available for analysis. There is a need for randomised controlled trials of these commonly used treatments in obstructive sleep apnoeas. These should identify which sub groups of patients with sleep apnoeas benefit most from each type of treatment and they should have clear and standardised outcome measures.

  7. Gastro-Oesophageal Reflux and Apnoea: Is There a Temporal Relationship?

    Science.gov (United States)

    Rossor, Thomas; Andradi, Gwendolyn; Ali, Kamal; Bhat, Ravindra; Greenough, Anne

    2018-01-01

    Gastro-oesophageal reflux (GOR) and apnoea are common in infants; whether there is a causal relationship is controversial. To determine whether there was a temporal relationship between GOR and apnoea, in particular, the frequency of obstructive apnoeas and if the frequency of GOR episodes correlated with apnoea frequency when maturity at testing was taken into account. Polysomnography and pH/multichannel intraluminal impedance (MII) studies were performed. Apnoeas were classified as central, obstructive, or mixed. MII events were classified as acidic (pH reflux event was compared to that in the 5-min period preceding the event and that in a 5-min reflux-free period (control period). Forty infants (median gestational age 29 [range 24-42] weeks) were assessed at a post-conceptional age of 37 (30-54) weeks. Obstructive (n = 580), central (n = 900), and mixed (n = 452) apnoeas were identified; 381 acid reflux events were detected by MII and 153 by the pH probe only. Apnoeas were not more frequent following GOR than during control periods. Both the frequency of apnoeas (p = 0.002) and GOR episodes (p = 0.01) were inversely related to post-conceptional age at testing, but were not significantly correlated with each other when controlled for post-conceptional age. These results suggest that GOR does not cause apnoea. © 2017 S. Karger AG, Basel.

  8. Idiopathic burning mouth syndrome: a common treatment-refractory somatoform condition responsive to ECT.

    Science.gov (United States)

    McGirr, Alexander; Davis, Lindsay; Vila-Rodriguez, Fidel

    2014-04-30

    Somatic symptom disorders are common causes of disability and suffering, and can pose significant management challenges. Idiopathic burning mouth syndrome is a challenging somatic symptom disorder with relatively high prevalence, particularly among post-menopausal women. Here, we present the case of a woman with severe treatment refractory idiopathic burning mouth syndrome and comorbid major depressive disorder, who was successfully treated with bitemporal electroconvulsive therapy. This case highlights the potential effectiveness of electroconvulsive therapy in idiopathic burning mouth syndrome when other treatment options have been exhausted. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

  9. Successful treatment of refractory TAFRO syndrome with elevated vascular endothelial growth factor using thyroxine supplements.

    Science.gov (United States)

    Oka, Satoko; Ono, Kazuo; Nohgawa, Masaharu

    2018-04-01

    Although the clinical significance of hypothyroidism in TAFRO syndrome is unknown, vascular endothelial growth factor (VEGF) levels decreased with improvements in the condition of our refractory TAFRO cases after thyroxine supplement therapy. Our results indicate that elevated VEGF levels are a potential factor in the pathogenesis and anasarca of TAFRO syndrome with hypothyroidism.

  10. Arterial stiffness in people with Type 2 diabetes and obstructive sleep apnoea

    DEFF Research Database (Denmark)

    Hvelplund Kristiansen, M; Banghøj, A M; Laugesen, E

    2018-01-01

    AIMS: To examine whether people with Type 2 diabetes with concurrent obstructive sleep apnoea have increased arterial stiffness as compared with people with Type 2 diabetes without obstructive sleep apnoea. METHODS: In a study with a case-control design, 40 people with Type 2 diabetes and treatment......-naïve moderate to severe obstructive sleep apnoea (Apnoea-Hypopnoea Index ≥15) and a control group of 31 people with Type 2 diabetes without obstructive sleep apnoea (Apnoea-Hypopnoea Index ... was not significantly different between participants with Type 2 diabetes with obstructive sleep apnoea and those without obstructive sleep apnoea (10.7±2.2 m/s vs 10.3±2.1 m/s; P=0.513), whereas oscillometric pulse wave velocity was significantly higher in participants with Type 2 diabetes with obstructive sleep...

  11. Ventilatory support and pharmacological treatment of patients with central apnoea or hypoventilation during sleep

    Directory of Open Access Journals (Sweden)

    D. Pevernagie

    2007-12-01

    Full Text Available The concept of central sleep apnoea or hypoventilation encompasses hypercapnic central hypoventilation, such as obesity hypoventilation syndrome and eucapnic or hypocapnic central sleep apnoea. Among subjects with eucapnic or hypocapnic central sleep apnoea, several therapeutic options are available for those with Cheyne–Stokes respiration (CSR. CSR is frequent in patients with New York Heart Association stage III and IV chronic heart failure, and in various neurological disorders. In these patients, treatment modalities include optimising cardiac condition and drugs, such as theophylline, acetazolamide and/or oxygen. Ventilatory support, such as nasal continuous positive airway pressure (CPAP, bi-level pressure support, or adaptive servo-ventilation (ASV, has been shown to improve CSR in patients with cardiac failure; however, convincing evidence that nasal CPAP improves life expectancy in these patients is lacking. Nevertheless, the treatment of associated obstructive sleep-disordered breathing is indicated per se, as it may improve cardiac function. There is currently no proof that bi-level ventilation is superior to nasal CPAP. The few available studies that have focused on ASV have shown satisfactory control of CSR in cardiac failure patients. While ASV is not a first-line treatment choice, it appears to be superior to oxygen, CPAP and bi-level pressure ventilation in controlling the apnoea/hypopnea index and probably sleep fragmentation. As yet there are no data on mortality and, as such, firm conclusions cannot be drawn as to the role of ASV in the management of cardiac failure patients suffering from CSR. Obesity-related hypoventilation has increased dramatically over recent decades due to the epidemic increase in obesity in the developed countries. Obesity hypoventilation syndrome predisposes to the development of pulmonary hypertension and cor pulmonale. Noninvasive home ventilation is increasingly applied in obese patients with

  12. Mild obstructive sleep apnoea: clinical relevance and approaches to management.

    Science.gov (United States)

    McNicholas, Walter T; Bonsignore, Maria R; Lévy, Patrick; Ryan, Silke

    2016-10-01

    Obstructive sleep apnoea is highly prevalent in the general population worldwide, especially in its mild form. Clinical manifestations correlate poorly with disease severity measured by the apnoea-hypopnoea index (AHI), which complicates diagnosis. Full polysomnography might be more appropriate to assess suspected mild cases because limited ambulatory diagnostic systems are least accurate in mild disease. Treatment options in mild obstructive sleep apnoea include continuous positive airway pressure (CPAP) and oral appliance therapy, in addition to positional therapy and weight reduction when appropriate. The superior efficacy of CPAP in reducing AHI is offset by greater tolerance of oral appliances, especially in mild disease. Although severe obstructive sleep apnoea is associated with adverse health consequences, including cardiometabolic comorbidities, the association with mild disease is unclear, and reports differ regarding the clinical relevance of mild obstructive sleep apnoea. Improved diagnostic techniques and evidence-based approaches to management in mild obstructive sleep apnoea require further research. Copyright © 2016 Elsevier Ltd. All rights reserved.

  13. Mandibular advancement appliance for obstructive sleep apnoea

    DEFF Research Database (Denmark)

    Petri, Niels; Svanholt, Palle; Solow, Beni

    2008-01-01

    The aim of this trial was to evaluate the efficacy of a mandibular advancement appliance (MAA) for obstructive sleep apnoea (OSA). Ninety-three patients with OSA and a mean apnoea-hypopnoea index (AHI) of 34.7 were centrally randomised into three, parallel groups: (a) MAA; (b) mandibular non...

  14. Electrical neuromodulation improves myocardial perfusion and ameliorates refractory angina pectoris in patients with syndrome X : fad or future?

    NARCIS (Netherlands)

    Jessurun, G; Hautvast, RWM; Tio, RA; DeJongste, M

    2003-01-01

    At present, there is no reliable antianginal drug therapy for patients with cardiac syndrome X. Therefore, the effect of electrical neuromodulation on refractory angina pectoris and myocardial perfusion in cardiac syndrome X was assessed. Eight patients (aged 55 +/- 7 years) with heterogeneous

  15. Severe obstructive sleep disorders in Prader-Willi syndrome patients in southern Italy.

    Science.gov (United States)

    Canora, Angelo; Franzese, Adriana; Mozzillo, Enza; Fattorusso, Valentina; Bocchino, Marialuisa; Sanduzzi, Alessandro

    2018-01-09

    Sleep-related disordered breathing (SDB) is very common in paediatric patients affected by Prader-Willi Syndrome (PWS). However, data addressing SBD patterns and their management are lacking. The aim of the present study was to analyse SDB features in 14 PWS patients (age range, 8 months-17 years). Polygraphic registration (PG) during a 12-h nocturnal sleep was performed in all patients. Obstructive and central apnoea indices and oxygen saturation (SpO 2 ) were recorded along with demographic and clinical data. Obstructive sleep apnoea (OSA) was diagnosed in 13/14 patients (92.9%); the mean obstructive apnoea-hypopnea index (OAHI) was 7.6 ± 4.2 events/h with a mean central apnoea index (CAI) of 0.7 ± 1.04 events/h. Time spent with SpO 2 Prader-Willi syndrome. What is New: • Severe obstructive sleep apnoea is the most frequent sleep-related disorder in our case series.

  16. Low-dose aripiprazole for refractory burning mouth syndrome

    Directory of Open Access Journals (Sweden)

    Umezaki Y

    2016-05-01

    Full Text Available Yojiro Umezaki,1 Miho Takenoshita,2 Akira Toyofuku2 1Psychosomatic Dentistry Clinic, Dental Hospital, 2Psychosomatic Dentistry, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo, Japan Abstract: We report a case of refractory burning mouth syndrome (BMS ameliorated with low dose of aripiprazole. The patient was a 66-year-old female who had suffered from chronic burning pain in her tongue for 13 months. No abnormality associated with the burning sensation was detected in the laboratory tests and the oral findings. Considering the clinical feature and the history together, we diagnosed the burning sensation as BMS. The BMS pain was decreased by aripiprazole (powder 1.0 mg/d, though no other antidepressants had satisfying pain relief. It could be supposed that the efficacy of aripiprazole is caused by dopamine stabilization in this case, and BMS might have a subtype that is reactive to aripiprazole. Further studies are needed to confirm the efficacy of aripiprazole for BMS. Keywords: burning mouth syndrome, low-dose aripiprazole, chronic pain

  17. High-intensity interval training improves obstructive sleep apnoea.

    Science.gov (United States)

    Karlsen, Trine; Nes, Bjarne Martens; Tjønna, Arnt Erik; Engstrøm, Morten; Støylen, Asbjørn; Steinshamn, Sigurd

    2016-01-01

    Three hours per week of vigorous physical activity is found to be associated with reduced odds of sleep-disordered breathing. To investigate whether 12 weeks of high-intensity interval training (HIIT) reduced the apnoea-hypopnea index (AHI) in obese subjects with moderate-to-severe obstructive sleep apnoea. In a prospective randomised controlled exercise study, 30 (body mass index 37±6 kg/m 2 , age 51±9 years) patients with sleep apnoea (AHI 41.5±25.3 events/hour) were randomised 1:1 to control or 12 weeks of supervised HIIT (4×4 min of treadmill running or walking at 90%-95% of maximal heart rate two times per week). In the HIIT group, the AHI was reduced by 7.5±11.6 events/hour (within-group pHIIT improved the AHI and self-reported daytime sleepiness in subjects with obese sleep apnoea without any change in the desaturation index and body weight.

  18. Symptom changes in five dimensions of the Positive and Negative Syndrome Scale in refractory psychosis

    NARCIS (Netherlands)

    Woodward, T.S.; Jung, K.; Smith, G.N.; Hwang, H.; Barr, A.M.; Procyshyn, R.M.; Flynn, S.W.; van der Gaag, M.; Honer, W.G.

    2014-01-01

    Refractory psychosis units currently have little information regarding which symptoms profiles should be expected to respond to treatment. In the current study, we provide this information using structural equation modeling of Positive and Negative Syndrome Scale (PANSS) ratings at admission and

  19. Sleep bruxism associated with obstructive sleep apnoea syndrome - A pilot study using a new portable device.

    Science.gov (United States)

    Winck, M; Drummond, M; Viana, P; Pinho, J C; Winck, J C

    Sleep bruxism (SB) and obstructive sleep apnoea syndrome (OSAS) share common pathophysiologic pathways. We aimed to study the presence and relationship of SB in a OSAS population. Patients referred with OSAS suspicion and concomitant SB complains were evaluated using a specific questionnaire, orofacial evaluation and cardio-respiratory polygraphy that could also monitor audio and EMG of the masseter muscles. From 11 patients studied 9 had OSAS. 55.6% were male, mean age was 46.3±11.3 years, and apnea hypopnea index of 11.1±5.7/h. Through specific questionnaire 55.6% had SB criteria. Orofacial examination (only feasible in 3) confirmed tooth wear in all. 77.8% had polygraphic SB criteria (SB index>2/h). Mean SB index was 5.12±3.6/h, phasic events predominated (72.7%). Concerning tooth grinding episodes, we found a mean of 10.7±9.2 per night. All OSAS patients except two (77.8%) had more than two audible tooth-grinding episodes. These two patients were the ones with the lowest SB index (1.0 and 1.4 per hour). Only in one patient could we not detect tooth grinding episodes. There was a statistically significant positive correlation between tooth grinding episodes and SB index and phasic event index (R=0.755, p=0.019 and R=0.737, p=0.023 respectively, Pearson correlation). Mean apnoea to bruxism index was 0.4/h, meaning that only a minority of SB events were not secondary to OSAS. We could not find any significant correlation between AHI and bruxism index or phasic bruxism index (R=-0.632 and R=-0.611, p>0.05, Pearson correlation). This pilot study shows that SB is a very common phenomenon in a group of mild OSAS patients, probably being secondary to it in the majority of cases. The new portable device used may add diagnostic accuracy and help to tailor therapy in this setting. Copyright © 2016 Sociedade Portuguesa de Pneumologia. Published by Elsevier España, S.L.U. All rights reserved.

  20. Apnoeic-hypopnoeic episodes during obstructive sleep apnoea are associated with histological nonalcoholic steatohepatitis.

    Science.gov (United States)

    Mishra, Poonam; Nugent, Clarke; Afendy, Arian; Bai, Chunhong; Bhatia, Priya; Afendy, Mariam; Fang, Yun; Elariny, Hazem; Goodman, Zachary; Younossi, Zobair M

    2008-09-01

    Nonalcoholic fatty liver disease (NAFLD) and obstructive sleep apnoea are associated with metabolic syndrome and atherosclerotic heart disease. This study evaluates the potential association between the NAFLD subtypes and a number of polysomnographical (PSG) parameters. This study included patients undergoing bariatric surgery with extensive clinical and histological data for whom complete PSG data before surgery were also available. Excess alcohol intake and other causes of liver disease were excluded. Apnoea, hypopnoea and apnoea-hypopnoea index (AHI) were calculated as described previously. In this study, a total of 101 patients [77 nonalcoholic steatohepatitis (NASH) and 22 non-NASH controls] with PSG data were included (age 42.9 +/- 11.4 years, body mass index 51.6 +/- 9.5 kg/m(2), fasting serum glucose 117.4 +/- 53.4 mg/dl, fasting serum triglycerides 171.3 +/- 82.9 mg/dl, 58% hypertension and 33% diabetes mellitus). Subjects with histological NASH had significantly lower lowest desaturation (77 vs. 85%, P=0.006), lower mean nocturnal oxygen saturation (91 vs. 93%, P=0.05), higher AHI (35 vs. 22, P=0.03), higher respiratory disturbance index (46 vs. 21, P=0.02) and higher alanine aminotransferase/aspartate aminotransferase ratio (1.4 vs. 1.3, P=0.05) compared with non-NASH controls. In multivariate analysis, the lowest desaturation (P=0.04) was independently associated with histological NASH. Lowest desaturation and mean nocturnal oxygen saturation were significantly lower in subjects with fibrosis (76 vs. 85%, P=0.004 and 90.4 vs. 93.0%, P=0.02). Our results suggest that the frequent nocturnal hypoxic episodes in NAFLD patients may be a risk factor for developing NASH. Additional studies are needed to study the effect of optimizing sleep apnoea management on the outcomes of patients with NAFLD.

  1. Nasal pillows as an alternative interface in patients with obstructive sleep apnoea syndrome initiating continuous positive airway pressure therapy.

    LENUS (Irish Health Repository)

    Ryan, Silke

    2012-02-01

    Side-effects directly due to the nasal mask are common in patients with obstructive sleep apnoea syndrome (OSAS) commencing continuous positive airway pressure (CPAP). Recently, nasal pillows have been designed to overcome these issues. Limited evidence exists of the benefits and effectiveness of these devices. Twenty-one patients (19 male, 49+\\/-10years) with the established diagnosis of OSAS [apnoea\\/hypopnoea index (AHI): 52+\\/-22] and who had a successful CPAP titration were commenced on CPAP therapy (10+\\/-2cmH2O), and randomized to 4weeks of a nasal pillow (P) and a standard nasal mask (M) in a crossover design. Outcome measures were objective compliance, AHI, quality of life, Epworth Sleepiness Score (ESS) and CPAP side-effects. There was no difference in compliance (M versus P: 5.1+\\/-1.9h versus 5.0+\\/-1.7h; P=0.701) and AHI (2.6+\\/-2.7 versus 3.0+\\/-2.9; P=0.509). Quality of life and ESS improved with CPAP, but there was no difference in the extent of improvement between both devices. Usage of nasal pillows resulted in less reported pressure on the face and more subjects found the nasal pillow the more comfortable device. However, there was no clear overall preference for either device at the end of the study (mask=57%, pillow=43%; P=0.513). The applied CPAP pressure did not correlate with compliance, AHI and ESS. Furthermore, no differences in outcome parameters were noted comparing groups with CPAP pressure <10 and >\\/=10cm H(2) O. Nasal pillows are equally effective in CPAP therapy, but do not generally lead to improved compliance.

  2. Oral appliances and functional orthopaedic appliances for obstructive sleep apnoea in children.

    Science.gov (United States)

    Carvalho, Fernando R; Lentini-Oliveira, Débora A; Prado, Lucila Bf; Prado, Gilmar F; Carvalho, Luciane Bc

    2016-10-05

    Apnoea is a breathing disorder marked by the absence of airflow at the nose or mouth. In children, risk factors include adenotonsillar hypertrophy, obesity, neuromuscular disorders and craniofacial anomalies. The most common treatment for obstructive sleep apnoea syndrome (OSAS) in childhood is adeno-tonsillectomy. This approach is limited by its surgical risks, mostly in children with comorbidities and, in some patients, by recurrence that can be associated with craniofacial problems. Oral appliances and functional orthopaedic appliances have been used for patients who have OSAS and craniofacial anomalies because they hold the lower jaw (mandible) forwards which potentially enlarges the upper airway and increases the upper airspace, improving the respiratory function. To assess the effects of oral appliances or functional orthopaedic appliances for obstructive sleep apnoea in children. We searched the following electronic databases: Cochrane Oral Health's Trials Register (to 7 April 2016); Cochrane Central Register of Controlled Trials (CENTRAL; 2016, Issue 3) in the Cochrane Library (searched 7 April 2016); MEDLINE Ovid (1946 to 7 April 2016); Embase Ovid (1980 to 7 April 2016); LILACS BIREME (from 1982 to 7 April 2016); BBO BIREME (from 1986 to 7 April 2016) and SciELO Web of Science (from 1997 to 7 April 2016). We searched ClinicalTrials.gov and the World Health Organization International Clinical Trials Registry Platform for ongoing trials on 7 April 2016. We placed no restrictions on the language or date of publication when searching the electronic databases. All randomised or quasi-randomised controlled trials comparing all types of oral and functional orthopaedic appliances with placebo or no treatment, in children 15 years old or younger. reduction of apnoea to less than one episode per hour. dental and skeletal relationship, sleep parameters improvement, cognitive and phonoaudiological function, behavioural problems, quality of life, side effects

  3. Identifying patients at high risk for obstructive sleep apnoea ...

    African Journals Online (AJOL)

    Background: Obstructive sleep apnoea is associated with significant health consequences. A significant proportion of hospitalized patients at risk for obstructive sleep apnoea were never identified and referred for polysomnography for diagnosis. The objective of this study was to determine the factors associated with high ...

  4. Sleep apnoea: Finnish National guidelines for prevention and treatment 2002-2012.

    Science.gov (United States)

    Laitinen, L A; Anttalainen, U; Pietinalho, A; Hämäläinen, P; Koskela, K

    2003-04-01

    (1) After negotiations with the Finnish Ministry of Social Affairs and Health, a national programme to promote prevention, treatment and rehabilitation of sleep apnoea for the years 2002-2012 has been prepared by the Finnish Lung Health Association on the basis of extensive collaboration. The programme needs to be revised as necessary, because of the rapid development in medical knowledge, and in appliance therapy in particular. (2) Sleep apnoea deteriorates slowly. Its typical features are snoring, interruptions of breathing during sleep and daytime tiredness. Sleep apnoea affects roughly 3% of middle-aged men and 2% of women. In Finland, there are approx. 150,000 sleep apnea patients, of which 15,000 patients have a severe disease, 50,000 patients are moderate and 85,000 have a mild form of the disease. Children are also affected by sleep apnea. A typical sleep apnea patient is a middle-aged man or a postmenopausal woman. (3) The obstruction of upper airways is essential in the occurrence of sleep apnoea. The obstruction can be caused by structural and/or functional factors. As for structural factors, there are various methods of intervention, such as to secure children's nasal respiration, to remove redundant soft tissue, as well as to correct malocclusions. It is possible to have an effect on the functional factors by treating well diseases predisposing to sleep apnoea, by reducing smoking, the consumption of alcohol and the use of medicines impairing the central nervous system. The most important single risk factor for sleep apnoea is obesity. (4) Untreated sleep apnoea leads to an increase morbidity and mortality through heart circulatory diseases and through accidents by tiredness. Untreated or undertreated sleep apnoea deteriorates a person's quality of life and working capacity. (5) The goals of the Programme for the prevention and treatment of sleep apnoea are as follows: (1) to decrease the incidence of sleep apnoea, (2) to ensure that as many patients

  5. Proposed consensus definitions for new-onset refractory status epilepticus (NORSE), febrile infection-related epilepsy syndrome (FIRES), and related conditions.

    Science.gov (United States)

    Hirsch, Lawrence J; Gaspard, Nicolas; van Baalen, Andreas; Nabbout, Rima; Demeret, Sophie; Loddenkemper, Tobias; Navarro, Vincent; Specchio, Nicola; Lagae, Lieven; Rossetti, Andrea O; Hocker, Sara; Gofton, Teneille E; Abend, Nicholas S; Gilmore, Emily J; Hahn, Cecil; Khosravani, Houman; Rosenow, Felix; Trinka, Eugen

    2018-04-01

    We convened an international group of experts to standardize definitions of New-Onset Refractory Status Epilepticus (NORSE), Febrile Infection-Related Epilepsy Syndrome (FIRES), and related conditions. This was done to enable improved communication for investigators, physicians, families, patients, and other caregivers. Consensus definitions were achieved via email messages, phone calls, an in-person consensus conference, and collaborative manuscript preparation. Panel members were from 8 countries and included adult and pediatric experts in epilepsy, electroencephalography (EEG), and neurocritical care. The proposed consensus definitions are as follows: NORSE is a clinical presentation, not a specific diagnosis, in a patient without active epilepsy or other preexisting relevant neurological disorder, with new onset of refractory status epilepticus without a clear acute or active structural, toxic or metabolic cause. FIRES is a subcategory of NORSE, applicable for all ages, that requires a prior febrile infection starting between 2 weeks and 24 hours prior to onset of refractory status epilepticus, with or without fever at onset of status epilepticus. Proposed consensus definitions are also provided for Infantile Hemiconvulsion-Hemiplegia and Epilepsy syndrome (IHHE) and for prolonged, refractory and super-refractory status epilepticus. This document has been endorsed by the Critical Care EEG Monitoring Research Consortium. We hope these consensus definitions will promote improved communication, permit multicenter research, and ultimately improve understanding and treatment of these conditions. Wiley Periodicals, Inc. © 2018 International League Against Epilepsy.

  6. Surgical treatment for medically refractory focal epilepsy in a patient with fragile X syndrome.

    Science.gov (United States)

    Kenmuir, Cynthia; Richardson, Mark; Ghearing, Gena

    2015-10-01

    Medication resistant temporal lobe epilepsy occurs in a small population of patients with fragile X syndrome. We present the case of a 24-year-old man with medically refractory temporal lobe epilepsy and fragile X syndrome who underwent left anterior temporal lobectomy resulting in cessation of seizures. Our patient was diagnosed with fragile X syndrome with a fully mutated, fully methylated FMR1 gene resulting in 572 CGG repeats. He developed seizures initially controlled with Depakote monotherapy, but progressed to become medically refractive to combination treatment with Depakote, lamotrigine and zonisamide. Prolonged video EEG monitoring revealed interictal left temporal sharp waves and slowing as well as subclinical and clinical seizures, each with left temporal onset. 3T MRI was consistent with left mesial temporal sclerosis. After discussing the case in our multidisciplinary surgical epilepsy conference, he was referred for presurgical evaluation including neuropsychological testing and Wada testing. He underwent an asleep left anterior temporal lobectomy, sparing the superior temporal gyrus. Pathology showed neuronal loss and gliosis in the hippocampus and amygdala. Twelve months after surgery, the patient has not experienced a seizure. He is described by his parents as less perseverative and less restless. We have presented the case of a 24 year-old-man with fragile X syndrome who underwent successful left anterior temporal lobectomy for the treatment of medically refractory epilepsy who is now seizure free without further functional impairment. This case report demonstrates the feasibility of surgical treatment for a patient with comorbid fragile X syndrome and mesial temporal sclerosis. Copyright © 2015 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

  7. Low-dose aripiprazole for refractory burning mouth syndrome.

    Science.gov (United States)

    Umezaki, Yojiro; Takenoshita, Miho; Toyofuku, Akira

    2016-01-01

    We report a case of refractory burning mouth syndrome (BMS) ameliorated with low dose of aripiprazole. The patient was a 66-year-old female who had suffered from chronic burning pain in her tongue for 13 months. No abnormality associated with the burning sensation was detected in the laboratory tests and the oral findings. Considering the clinical feature and the history together, we diagnosed the burning sensation as BMS. The BMS pain was decreased by aripiprazole (powder) 1.0 mg/d, though no other antidepressants had satisfying pain relief. It could be supposed that the efficacy of aripiprazole is caused by dopamine stabilization in this case, and BMS might have a subtype that is reactive to aripiprazole. Further studies are needed to confirm the efficacy of aripiprazole for BMS.

  8. Effectiveness of cyclosporine and mycophenolate mofetil in a child with refractory evans syndrome

    Directory of Open Access Journals (Sweden)

    Piero Farruggia

    2011-05-01

    Full Text Available Evans Syndrome is a rare autoimmune disease consisting of hemolytic anemia, thrombocytopenia and/or neutropenia. It may be associated with other autoimmune or lymphoproliferative diseases. Its course can be extremely serious and, rarely, even life-threatening; thus it represents a excellent treatment challenge for the pediatric hematologist. First line treatment consists of steroids and/or immunoglobulin; further therapy with rituximab, vincristine, cyclophosphamide and other immunosuppressive drugs can be considered in unresponsive patients. We describe a baby with refractory Evans Syndrome that was cured by prolonged administration of mycophenolate mofetil and remained disease-free for 4 years after the discontinuation of treatment.

  9. Polycystic ovarian syndrome

    OpenAIRE

    Nina Madnani; Kaleem Khan; Phulrenu Chauhan; Girish Parmar

    2013-01-01

    Polycystic ovarian syndrome (PCOS) is a "multispeciality" disorder suspected in patients with irregular menses and clinical signs of hyperandrogenism such as acne, seborrhoea, hirsutism, irregular menses, infertility, and alopecia. Recently, PCOS has been associated with the metabolic syndrome. Patients may develop obesity, insulin resistance, acanthosis nigricans, Type 2 diabetes, dyslipidemias, hypertension, non-alcoholic liver disease, and obstructive sleep apnoea. Good clinical examinatio...

  10. Refractory Post-Herpetic Neuralgia As An Initial Presentation Of Olfactory Neuroblastoma-Related Ectopic ACTH Syndrome

    Directory of Open Access Journals (Sweden)

    Hsiang-Hung Lin

    2009-03-01

    Full Text Available We report a woman aged 64 years with ectopic adrenocorticotropic hormone (ACTH syndrome caused by olfactory neuroblastoma as an initial presentation of refractory post-herpetic neuralgia. The manifestations such as cushingoid appearance and endocrine abnormalities are compatible with Cushing's syndrome. Brain computed tomography revealed a sellar mass. A biopsy revealed olfactory neuroblastoma. Immunohistochemical staining further defined the tumor as an ACTH-secreting neuroblastoma. Subsequent opportunistic infections by Candida glabrata fungemia and multiple drug-resistant Acinetobacter baumannii pneumonia occurred during hospitalization as a complication of severe hypercortisolism. Before any therapy for Cushing's syndrome and neuroblastoma could be initiated, the patient died from sepsis and multiorgan failure. We propose that Cushing's syndrome is more complex than what clinicians thought, and that meticulous cerebral imaging studies are crucial.

  11. Obstructive sleep apnoea syndrome in a patient with retrosternal goiter: a case report

    International Nuclear Information System (INIS)

    Sevketbeyoglu, H.; Kara, K.; Ince, M.; Karaagac, H.

    2012-01-01

    Full text: Introduction: Obstructive sleep apnoea syndrome (OSAS) is associated with a large number of predisposing factors (obesity, nasal obstruction, adenoid hypertrophy, macroglossia etc.). In addition to these factors goiter and hypothyroidism have been reported to be associated with OSAS. Objectives and tasks: In our case with retrosternal goiter, values of OSAS before and after thyroidectomy were shown. Materials and methods: Seventy-two years old, BMI: 26,8 kg/m 2 , female patient was admitted our hospital because of complaints to stop breathing during sleep, snoring, morning headache and daytime drowsiness. Results: Thorax CT and ultrasonography of thyroid shown retrosternal goiter and left tracheal deviation. Severe OSAS was diagnosed by polysomnography (PSG). Thyroid function tests were normal. Apneahypopnea index (AHI) was 63,1/h. Patients was performed 7 cm H 2 O nasal continuous positive airway pressure (gvrnCPAP). AHI was 11,4/h under nCPAP. One month after OSAS diagnosis the patient underwent thyroidectomy operation. Pathological examination was reported as multinodular GOITER. In postoperative period CPAP treatment couldn't continue, because patient was not compliant. In postoperative 8-th weeks, PSG was performed; AHI was 34,8/h. The patient's weight and BMI didn't change. Conclusion: In our case, despite absence of continued CPAP treatment after thyroidectomy, symptoms and PSG values improved partially. As a result of these findings, especially, compression of upper airway and deterioration of venous circulation of patients with large goiter may lead to an increase in OSAS symptoms. During patients with OSAS are treated with CPAP, goiter needs to be investigated

  12. Oral appliances and functional orthopaedic appliances for obstructive sleep apnoea in children.

    Science.gov (United States)

    Carvalho, F R; Lentini-Oliveira, D; Machado, M A C; Prado, G F; Prado, L B F; Saconato, H

    2007-04-18

    Apnoea is a breathing disorder marked by the absence of airflow at the nose or mouth. In children, risk factors include adenotonsillar hypertrophy, obesity, neuromuscular disorders and craniofacial anomalies. The most common treatment for obstructive sleep apnoea syndrome (OSAS) in childhood is adenotonsillectomy. This approach is limited by its surgical risks, mostly in children with comorbities and, in some patients, by recurrence that can be associated with craniofacial problems. Oral appliances and functional orthopaedic appliances have been used for patients who have OSAS and craniofacial anomalies because they change the mandible posture forwards and potentially enlarge the upper airway and increase the upper airspace, improving the respiratory function. To assess the effectiveness of oral appliances or functional orthopaedic appliances for OSAS in children. A sensitive search was developed for the Cochrane Central Register of Controlled Trials (CENTRAL) (The Cochrane Library 2005, Issue 3); PubMed (January 1966 to September 2005); EMBASE (1980 to September 2005); Lilacs (1982 to September 2005); BBO-Bibliografia Brasileira de Odontologia (1986 to September 2005); and SciELO (1997 to September 2005). There was no restriction of language or source of information. All randomised or quasi-randomised controlled trials comparing all types of oral and functional orthopaedic appliances with placebo or no treatment, in children 15 years old or younger. reduction of apnoea to less than one episode per hour. dental and skeletal relationship, sleep parameters improvement, cognitive and phonoaudiologic function, behavioural problems, drop outs and withdrawals, quality of life, side effects (tolerability), economic evaluation. Data were independently extracted by two review authors. Authors were contacted for additional information. Risk ratios with 95% confidence intervals were calculated for all important dichotomous outcomes. The initial search identified 384 trials

  13. The genetics of obstructive sleep apnoea.

    LENUS (Irish Health Repository)

    Kent, Brian D

    2012-02-01

    PURPOSE OF REVIEW: Obstructive sleep apnoea syndrome (OSAS) is a highly prevalent disorder associated with reduced quality of life and adverse cardiovascular and metabolic sequelae. Recent years have seen an intensification of the research effort to establish the genetic contribution to the development of OSAS and its sequelae. This review explores emerging evidence in this field. RECENT FINDINGS: A genetic basis for sleep-disordered breathing has been demonstrated for discrete disorders such as Treacher-Collins and Down syndromes, but the picture is less clear in so-called idiopathic OSAS. A degree of heritability appears likely in some of the intermediate phenotypes that lead to OSAS, particularly craniofacial morphology. However, only sparse and often contradictory evidence exists regarding the role of specific polymorphisms in causing OSAS in the general population. Similarly, investigations of the cardiovascular sequelae of OSAS have in general failed to consistently find single causative genetic mutations. Nonetheless, evidence suggests a role for tumour necrosis factor-alpha polymorphisms in particular, and large-scale family studies have suggested shared pathogenetic pathways for the development of obesity and OSAS. SUMMARY: As with other common disorders, OSAS is likely to result from multiple gene-gene interactions occurring in a suitable environment. The application of modern genetic investigative techniques, such as genome-wide association studies, may facilitate new discoveries in this field.

  14. Talimogene Laherparepvec and Nivolumab in Treating Patients With Refractory Lymphomas or Advanced or Refractory Non-melanoma Skin Cancers

    Science.gov (United States)

    2018-05-21

    Adenoid Cystic Carcinoma; Adnexal Carcinoma; Apocrine Carcinoma; Eccrine Porocarcinoma; Extraocular Cutaneous Sebaceous Carcinoma; Hidradenocarcinoma; Keratoacanthoma; Malignant Sweat Gland Neoplasm; Merkel Cell Carcinoma; Microcystic Adnexal Carcinoma; NK-Cell Lymphoma, Unclassifiable; Non-Melanomatous Lesion; Paget Disease; Papillary Adenocarcinoma; Primary Cutaneous Mucinous Carcinoma; Refractory Anaplastic Large Cell Lymphoma; Refractory Mature T-Cell and NK-Cell Non-Hodgkin Lymphoma; Refractory Mycosis Fungoides; Refractory Primary Cutaneous T-Cell Non-Hodgkin Lymphoma; Refractory T-Cell Non-Hodgkin Lymphoma; Sezary Syndrome; Signet Ring Cell Carcinoma; Skin Basal Cell Carcinoma; Skin Basosquamous Cell Carcinoma; Skin Squamous Cell Carcinoma; Spiradenocarcinoma; Squamous Cell Carcinoma of Unknown Primary Origin; Stage III Skin Cancer; Stage IV Skin Cancer; Sweat Gland Carcinoma; Trichilemmocarcinoma; Vulvar Squamous Cell Carcinoma

  15. Pattern recognition of obstructive sleep apnoea and Cheyne–Stokes respiration

    International Nuclear Information System (INIS)

    Weinreich, Gerhard; Teschler, Helmut; Armitstead, Jeff

    2008-01-01

    The aim of this study was to assess the validity of an artificial neural network based on flow-related spectral entropy as a diagnostic test for obstructive sleep apnoea and Cheyne–Stokes respiration. A data set of 37 subjects was used for spectral analysis of the airflow by performing a fast Fourier transform. The examined intervals were divided into epochs of 3 min. Spectral entropy S was applied as a measure for the spread of the related power spectrum. The spectrum was divided into several frequency areas with various subsets of spectral entropy. We studied 11 subjects with obstructive apnoeas (n = 267 epochs), 10 subjects with obstructive hypopnoeas (n = 80 epochs), 11 subjects with Cheyne–Stokes respiration (n = 253 epochs) and 5 subjects with normal breathing in non-REM sleep (n = 174 epochs). Based on spectral entropy an artificial neural network was built, and we obtained a sensitivity of 90.2% and a specificity of 90.9% for distinguishing between obstructive apnoeas and Cheyne–Stokes respiration, and a sensitivity of 91.3% and a specificity of 94.6% for discriminating between obstructive hypopnoeas and normal breathing in non-REM sleep. This resulted in an accuracy of 91.5% for identifying flow patterns of obstructive sleep apnoea, Cheyne–Stokes respiration and normal breathing in non-REM sleep. It is concluded that the use of an artificial neural network relying on spectral analysis of the airflow could be a useful method as a diagnostic test for obstructive sleep apnoea and Cheyne–Stokes respiration

  16. Cardiovascular risk in patients with sleep apnoea with or without continuous positive airway pressure therapy

    DEFF Research Database (Denmark)

    Lamberts, Morten; Nielsen, O W; Lip, G Y H

    2014-01-01

    BACKGROUND: The prognostic significance of age and continuous positive airway pressure (CPAP) therapy on cardiovascular disease in patients with sleep apnoea has not been assessed previously. METHODS: Using nationwide databases, the entire Danish population was followed from 2000 until 2011. First......-time sleep apnoea diagnoses and use of CPAP therapy were determined. Incidence rate ratios (IRRs) of ischaemic stroke and myocardial infarction (MI) were analysed using Poisson regression models. RESULTS: Amongst 4.5 million individuals included in the study, 33 274 developed sleep apnoea (mean age 53, 79......% men) of whom 44% received persistent CPAP therapy. Median time to initiation of CPAP therapy was 88 days (interquartile range 34-346). Patients with sleep apnoea had more comorbidities compared to the general population. Crude rates of MI and ischaemic stroke were increased for sleep apnoea patients...

  17. Respiratory and sleep disorders in female children with atypical Rett syndrome caused by mutations in the CDKL5 gene.

    Science.gov (United States)

    Hagebeuk, Eveline E O; van den Bossche, Renilde A S; de Weerd, Al W

    2013-05-01

    In female children with drug-resistant seizures and developmental delay from birth, atypical Rett syndrome caused by mutations in the CDKL5 gene should be considered. Several clinical features resemble classic Rett syndrome. Respiratory and sleep abnormalities are frequently present in Rett syndrome, whereas little is known in patients with CDKL5 mutations. In four genetically confirmed female patients with CDKL5 mutations (age range 2-15 y), the presence of breathing and sleep abnormalities was evaluated using the validated Sleep Disturbance Scale for Children and polysomnography (PSG). The Sleep Disturbance Scale for Children indicated disorders of initiating and maintaining sleep, daytime somnolence, and sleep breathing disorders. In one patient, PSG showed central apnoeas during sleep: her total apnoea-hypopnoea index (AHI) was 4.9, of which the central AHI was 3.4/h. When awake, central apnoeas were present in two of the four female children (central AHI 28/h and 41/h respectively), all preceded by hyperventilation. PSG showed low rapid eye movement (REM) sleep (9.7-18.3%), frequent awakenings, and low sleep efficiency (range 59-78%). Episodic hyperventilation followed by central apnoeas was present while awake in two of four patients. This may indicate failure of brainstem respiratory centres. In addition, low REM sleep, frequent arousals (not caused by apnoeas/seizures), and low sleep efficiency were present. Similar to Rett syndrome, in patients with CDKL5 mutations PSG seems warranted to evaluate breathing and sleep disturbances. © The Authors. Developmental Medicine & Child Neurology © 2012 Mac Keith Press.

  18. Bone marrow immunophenotyping by flow cytometry in refractory cytopenia of childhood

    NARCIS (Netherlands)

    A.M. Aalbers (Anna Maartje); M.M. van den Heuvel-Eibrink (Marry); I. Baumann (Irith); M.N. Dworzak (Michael); H. Hasle (Henrik); F. Locatelli (Franco); B. de Moerloose (Barbara); M. Schmugge; E. Mejstříková (Ester); M. Nováková (Michaela); M. Zecca (Marco); C.M. Zwaan (Christian Michel); J.G. te Marvelde (Jeroen); A.W. Langerak (Anton); J.J.M. van Dongen (Jacques); R. Pieters (Rob); C.M. Niemeyer (Charlotte); V.H.J. van der Velden (Vincent)

    2015-01-01

    textabstractRefractory cytopenia of childhood is the most common type of childhood myelodysplastic syndrome. Because the majority of children with refractory cytopenia have a normal karyotype and a hypocellular bone marrow, differentiating refractory cytopenia from the immune-mediated bone marrow

  19. The role of nocturnal oximetry in obstructive sleep apnoea-hypopnoea syndrome screening

    Directory of Open Access Journals (Sweden)

    Celestina Ventura

    2007-07-01

    Full Text Available Aim: The aim of our study was to evaluate the sensitivity and specificity of Nocturnal Oximetry (NO as a diagnostic screening tool for obstructive sleep apnoea hypopnoea syndrome (OSAHS, compared with polysomnography (PSG as the gold standard. Methodology: 63 patients with clinical suspicion of OSAHS and exclusion of respiratory disease underwent PSG and NO. We then determined NO sensitivity, specificity, positive (PPV and negative predictive values (NPV. Results: OSAHS was diagnosed in 47 patients with a mean age of 54 years. In the evaluation of the percentage of Total Sleep Time (TST with oxygen desaturation below 90%, we found significant differences between patients with OSAHS (25.4 ± 29.7% and without OSAHS (1 ± 1.5%, p<0,001. We used two cutoff points to evaluate sensitivity, specificity, positive (PPV and negative predictive values (NPV, based on the severity of O2 desaturation (StO2<90%. Using the first cutoff point we diagnosed with NO as positive all the patients with TST desaturation values ≥1% of the TST. Under these circumstances we found a sensitivity of 76.6%, a specificity of 75%, a PPV of 90% and an NPV value of 52.2% for our screening test (NO. Using the second cutoff point, we diagnosed with NO as positive all the patients with TST desaturation values ≥5% of the TST. With this method we found a sensitivity of 65.9%, a specificity of 100%, a PPV of 100% and an NPV of 50%. Conclusion: NO is a useful screening test for the diagnosis of OSAHS in patients without respiratory disease. Resumo: Objectivo: Foi objectivo deste estudo determinar a sensibilidade e a especificidade da oximetria nocturna (ON como método de screening diagnóstico para a síndroma de apneia-hipopneia obstrutiva do sono (SAHOS, utilizando como método de referência a polissonografia (PSG. Metodologia: Foram incluídos 63 doentes com suspeita clínica de SAHOS e exclusão de doença respiratória, sendo

  20. SLEEP APNOEA!!! : SNORING & BEYOND

    Indian Academy of Sciences (India)

    SLEEP APNOEA!!! : SNORING & BEYOND · Slide 2 · Snoring · Introduction · Identifiable causes of hypertension · Crucial areas for Snoring & Obstructive Sleep Apnea · Slide 7 · Slide 8 · Slide 9 · Slide 10 · Slide 11 · Slide 12 · Slide 13 · Slide 14 · Slide 15 · Slide 16 · Epidemiology (contd.) Slide 18 · Am I at risk??? Slide 20.

  1. Obstructive sleep apnoea and atopy among middle aged chronic obstructive pulmonary disease and bronchial asthma patients.

    Science.gov (United States)

    Kumar, Raj; Nagar, Devender; Mallick, Adeeb; Kumar, Manoj; Tarke, Chandrakant R; Goel, Nitin

    2013-09-01

    Obstructive sleep apnoea syndrome is associated with significant morbidity. A high prevalence of obstructive sleep apnoea (OSA) symptoms has been reported in patients with asthma and chronic obstructive pulmonary disease (COPD). There are limited studies regarding relationship between atopy and OSA. To study the risk of obstructive sleep apnoea among middle aged chronic obstructive pulmonary disease and asthma patients by a home based sleep study and its association with atopy. Patients with asthma and COPD were evaluated for OSA symptoms by Epworth sleepiness scale (ESS) and Berlin questionnaire (BQ). ESS score > or = 9 was considered as high risk for OSA. Patients having high risk for OSA by ESS and BQ were further evaluated for OSA by home based sleep study. Skin prick test against common allergens was done to diagnose atopy in these patients. Among 400 patients (229, 57.25% male and 171, 42.75% female) 328 were asthmatics and 72 were COPD patients. ESS and BQ was positive in 11.25% (45/400) and 18.25% (73/400) patients respectively. ESS was positive in 10.67% (35/328) of asthma and 13.88% (10/72) of COPD patients. BQ was positive in 18.29% (60/328) of asthmatic and 18.05% (13/72) of COPD patients. Skin prick test was positive in 74.16% patients. The maximum positivity was found in asthmatics (139/155, 89.68%) compared to COPD patients (16/155, 10.32%). Skin prick test was done for 40 patients out of 73 of Asthma and COPD patients who were found positive by ESS and BQ. 72.5% patients were found to be atopic. Out of 19 patients in whom home polysomnography was done, 13 patients consented for skin prick test with common aeroallergens and 9 (69.23%) patients were found to be atopic. There is an increased risk of obstructive sleep apnoea among middle aged chronic obstructive pulmonary disease and asthma patients. Atopy could be associated with OSA. The association needs to be proved in a larger study.

  2. Patient's experience of treatment for sleep apnoea with a mandibular advancement splint.

    Science.gov (United States)

    Bhamrah, Gurprit; Dhir, Arti; Cash, Alex; Ahmad, Sofia; Winchester, Lindsay J

    2015-10-01

    Obstructive sleep apnoea (OSA) is a well recognised clinical disorder in which there is narrowing and repeated collapse of the upper airway during sleep resulting in the cessation of breathing. Patients with mild to moderate sleep apnoea are often provided with mandibular advancement splint (MAS) therapy as a form of first line or definitive treatment. The aims of this audit were to evaluate patient satisfaction and success of MAS therapy. 93 patients diagnosed with sleep apnoea and suitable for a splint were recruited prospectively at Queen Victoria Hospital, East Grinstead between January 2009 and October 2010. A patient satisfaction questionnaire was developed by health professionals involved in the care of patients with sleep apnoea and assessed for face and content validity and reliability. Participants completed the questionnaire six weeks after the splint was fitted. 44% who previously experienced snoring now reported no snoring and 47% reported less snoring since wearing the MAS appliance. 69% reported complete resolution of sleep apnoea symptoms. 37% experienced aching teeth and 33% experienced having a dry throat when wearing the appliance. 86% of sleeping partners felt that their quality of sleep was improved following their partners treatment. The standards set for each criteria in this audit were met. MAS treatment has a key role to play in the management of obstructive sleep apnoea with high rates of patient satisfaction and the majority of patients partners reporting a significant improvement in their own and their partners sleep quality. Copyright © 2014 Royal College of Surgeons of Edinburgh (Scottish charity number SC005317) and Royal College of Surgeons in Ireland. Published by Elsevier Ltd. All rights reserved.

  3. Partial splenic embolization combined with vincristine infusion for the treatment of refractory idiopathic thrombocytopenic purpura and Evans syndrome: observation of its long-term efficacy

    International Nuclear Information System (INIS)

    Chen Shibing

    2011-01-01

    Objective: To observe the long-term efficacy of partial spleen embolization combined with vincristine infusion in treating refractory idiopathic thrombocytopenic purpura (ITP) and Evans syndrome. Methods: During the period of 2000-2007, partial spleen embolization together with vincristine infusion was carried out in 30 patients with refractory idiopathic thrombocytopenic purpura (n=24) or Evans syndrome (n=6). Vincristine infusion (2 mg) via splenic artery was performed before partial spleen embolization procedure. The long-term effectiveness was observed and analyzed. Results: One week after the treatment, the platelet count was increased from preoperative (10.23±8.28) × 10 9 /L to (140.28±85.45) × 10 9 /L in patients with ITP, while the platelet count was increased from preoperative (12±8) × 10 9 /L to (210±60) × 10 9 /L in patients with Evans syndrome. Meanwhile, the hemoglobin level showed an increase in different degrees, from preoperative (63.00±13.62) g/L to postoperative (123.00±13.14) g/L. The therapeutic effectiveness was 100%. During the follow-up time lasting for 3-5 years, recurrence was seen in 11 patients (36.7%) and the overall efficacy rate was 63.3%. Conclusion: For the treatment of refractory idiopathic thrombocytopenic purpura and Evans syndrome, partial spleen embolization combined with vincristine infusion carries reliable long-term efficacy. (author)

  4. Congenital central hypoventilation syndrome and intestinal ...

    African Journals Online (AJOL)

    ... hypoventilation syndrome (CCHS), also called 'Ondine's curse', is characterised by an abnormal ventilatory response to progressive hypercapnia and sustained hypoxaemia. Neonates with this condition experience hypoventilation or apnoea while asleep. Patients may also have congenital intestinal aganglionosis (CIA), ...

  5. Behavioural hyperventilation as a novel clinical condition associated with central sleep apnoea: a report of three cases.

    Science.gov (United States)

    Pevernagie, Dirk; Mariman, An; Vandenbussche, Nele; Tobback, Els; Overeem, Sebastiaan; Delesie, Liesbeth; Janssen, Hennie; Vogelaers, Dirk

    2012-12-01

    Central sleep apnoea (CSA) is a disorder characterised by repetitive episodes of decreased ventilation due to complete or partial reduction in the central neural outflow to the respiratory muscles. Hyperventilation plays a prime role in the pathogenesis of CSA. Chronic heart failure and dwelling at high altitude are classical conditions in which CSA is induced by hyperventilation. Hyperventilation syndrome (HVS) is a prevalent behavioural condition in which minute ventilation exceeds metabolic demands, resulting in haemodynamic and chemical changes that produce characteristic dysphoric symptoms. HVS is frequently caused by anxiety disorders and panic attacks. Until now, medical literature has focussed primarily on daytime symptoms of behavioural hyperventilation. It is currently unknown how this condition may affect sleep. Three cases are reported in which behavioural hyperventilation was associated with occurrence of significant central sleep apnoea, which was not present during normal tidal breathing in steady sleep. Therefore, behavioural hyperventilation should be added to the list of known clinical conditions associated with CSA. Copyright © 2012 Elsevier B.V. All rights reserved.

  6. Developing a successful treatment for co-morbid insomnia and sleep apnoea.

    Science.gov (United States)

    Sweetman, Alexander M; Lack, Leon C; Catcheside, Peter G; Antic, Nick A; Chai-Coetzer, Ching Li; Smith, Simon S; Douglas, James A; McEvoy, R Doug

    2017-06-01

    Insomnia and sleep apnoea are the two most common sleep disorders, found in 6% and 23-50% of the general population respectively. These disorders also frequently co-occur, with 39-58% of sleep apnoea patients reporting symptoms indicative of co-morbid insomnia. When these disorders co-occur, clinicians are faced with difficult treatment decisions, patients experience the additive detrimental impacts of both disorders, and the effectiveness of discrete treatments for each disorder may be impaired. A common finding is that co-morbid insomnia and sleep apnoea (COMISA) is more difficult to treat than either disorder presenting alone. Co-morbid insomnia reduces the initial acceptance of, and later adherence to, continuous positive airway pressure (CPAP) therapy for obstructive sleep apnoea. This has resulted in recent recommendations that treatment approaches should initially target COMISA patients' insomnia to remove this barrier to CPAP treatment, and improve patient outcomes. However, no randomised controlled trial outcomes investigating this treatment approach currently exist. The current article aims to review and integrate recent research examining the prevalence, characteristics, and theoretical mechanistic relationships between co-occurring insomnia and OSA, and discuss previous treatment attempts. Copyright © 2016 Elsevier Ltd. All rights reserved.

  7. Alcohol and the risk of sleep apnoea: a systematic review and meta-analysis.

    Science.gov (United States)

    Simou, Evangelia; Britton, John; Leonardi-Bee, Jo

    2018-02-01

    A systematic review and meta-analysis of the association between alcohol consumption and risk of sleep apnoea in adults. We searched Medline, EMBASE and Web of Science databases from 1985 to 2015 for comparative epidemiological studies assessing the relation between alcohol consumption and sleep apnoea. Two authors independently screened and extracted data. Random effects meta-analysis was used to estimate pooled effect sizes with 95% confidence intervals (CI). Heterogeneity was quantified using I 2 and explored using subgroup analyses based on study exposure and outcome measures, quality, design, adjustment for confounders and geographical location. Publication bias was assessed using a funnel plot and Egger's test. We identified 21 studies from which estimates of relative risk could be obtained. Meta-analysis of these estimates demonstrated that higher levels of alcohol consumption increased the risk of sleep apnoea by 25% (RR 1.25, 95%CI 1.13-1.38, I 2  = 82%, p Country locations. We detected evidence of publication bias (p = 0.001). A further eight included studies reported average alcohol consumption in people with and without sleep apnoea. Meta-analysis revealed that mean alcohol intake was two units/week higher in those with sleep apnoea, but this difference was not statistically significant (p = 0.41). These findings suggest that alcohol consumption is associated with a higher risk of sleep apnoea, further supporting evidence that reducing alcohol intake is of potential therapeutic and preventive value in this condition. Copyright © 2018 The Authors. Published by Elsevier B.V. All rights reserved.

  8. Driving ability in sleep apnoea patients before and after CPAP treatment: evaluation on a road safety platform.

    Science.gov (United States)

    Mazza, S; Pépin, J-L; Naëgelé, B; Rauch, E; Deschaux, C; Ficheux, P; Lévy, P

    2006-11-01

    Sleepiness is considered to be the major cause of increased traffic accidents in patients with obstructive sleep apnoea syndrome (OSAS). Until now, OSAS patients' driving ability has been assessed using driving simulators, but no assessment in a more natural driving environment has been carried out to date. The aim of the present study was to evaluate driving parameters in OSAS and in controls on a road safety platform, and to compare them with attentional in-laboratory measures before and after continuous positive airway pressure treatment. The parameters measured were: reaction time; distance to stop and number of collisions on the platform; maintenance of wakefulness; and sustained, selective and divided attention in laboratory. Patients exhibited much longer reaction times than controls, leading to a lengthening of the vehicle's stopping distance of 8.8 m at 40 km.h(-1) and to twice the number of collisions. Patients did not demonstrate objective sleepiness or selective and sustained attention deficits. Divided attention deficits were found. However, they did not allow the prediction of real driving impairment. After CPAP treatment, there was no longer any difference between patients and controls regarding driving and attention performances. Driving abilities are significantly impaired in obstructive sleep apnoea syndrome. After continuous positive airway pressure treatment, deficits were normalised. This stresses the importance of evaluating attentional parameters in apnoeic patients and of offering continuous positive airway pressure treatment even to non-sleepy subjects.

  9. Refractory Depression, Fatigue, Irritable Bowel Syndrome, and Chronic Pain: A Functional Medicine Case Report.

    Science.gov (United States)

    Plotnikoff, Gregory; Barber, Melissa

    2016-01-01

    Single-disorder or single-organ-system clinical practice guidelines are often of limited usefulness in guiding effective management of patients with chronic multidimensional signs and symptoms. The presence of multiple long-standing medical problems in a given patient despite intensive medical effort suggests that addressing systemic core imbalances could complement more narrowly focused approaches. A 72-year-old man experiencing longstanding depression, fatigue, irritable bowel syndrome, and chronic pain in the context of additional refractory illnesses was assessed and treated, guided by a system-oriented approach to underlying core imbalances termed functional medicine. This patient was referred from a team of clinicians representing primary care, cardiology, gastroenterology, hematology, and psychology. Prior treatment had been unsuccessful in managing multiple chronic comorbidities. Diagnostic assessment included comprehensive stool and nutritional/metabolic laboratory testing. The blood-, urine-, or stool-based measurements of relevant markers for multiple systemic issues, including digestion/absorption, inflammation, oxidative stress, and methylation, identified previously unrecognized root causes of his constellation of symptoms. These functional measurements guided rational recommendations for dietary choices and supplementation. The patient experienced steady and significant improvement in his mental health, fatigue, chronic pain, and irritable bowel syndrome-as well as the unexpected resolution of his chronic idiopathic pancytopenia. The success in this case suggests that other patients with chronic, complex, and treatment-refractory illness may benefit from a system-oriented assessment of core imbalances guided by specialized nutritional/metabolic and digestive laboratory testing.

  10. Duloxetine in the treatment of burning mouth syndrome refractory to conventional treatment: A case report.

    Science.gov (United States)

    Kim, Yeon-Dong; Lee, Ji-Hye; Shim, Jee-Hoon

    2014-06-01

    Patients with burning mouth syndrome (BMS) report burning sensation and pain involving the tongue and oral mucosa without any apparent medical or dental cause. The pathogenesis of this syndrome remains unclear and there is currently no standard treatment. BMS is, therefore, often misdiagnosed and its management is complex. This lack of clinical expertise may result in decreased health-related quality of life and increased psychological distress among patients with BMS. The present case report involves a 77-year-old female patient with BMS refractory to conventional treatment with nerve block and medication, who was successfully treated with duloxetine. Duloxetine may become a new therapeutic option in the management of BMS. © The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

  11. Congenital Central Hypoventilation Syndrome And Intestinal

    African Journals Online (AJOL)

    Congenital central hypoventilation syndrome (CCHS) or 'Ondine's curse' is characterised by an abnormal ventilatory response to progressive hypercapnia and sustained hypoxaemia. During the neonatal period, most patients with this condition present with hypoventilation or apnoea while asleep. Patients with CCHS may ...

  12. Symptom changes in five dimensions of the Positive and Negative Syndrome Scale in refractory psychosis.

    Science.gov (United States)

    Woodward, Todd S; Jung, Kwanghee; Smith, Geoffrey N; Hwang, Heungsun; Barr, Alasdair M; Procyshyn, Ric M; Flynn, Sean W; van der Gaag, Mark; Honer, William G

    2014-12-01

    Refractory psychosis units currently have little information regarding which symptoms profiles should be expected to respond to treatment. In the current study, we provide this information using structural equation modeling of Positive and Negative Syndrome Scale (PANSS) ratings at admission and discharge on a sample of 610 patients admitted to a treatment refractory psychosis program at a Canadian tertiary care unit between 1990 and 2011. The hypothesized five-dimensional structure of the PANSS fit the data well at both admission and discharge, and the latent variable scores are reported as a function of symptom dimension and diagnostic category. The results suggest that, overall, positive symptoms (POS) responded to treatment better than all other symptoms dimensions, but for the schizoaffective and bipolar groups, greater response on POS was observed relative to the schizophrenia and major depression groups. The major depression group showed the most improvement on negative symptoms and emotional distress, and the bipolar group showed the most improvement on disorganization. Schizophrenia was distinct from schizoaffective disorder in showing reduced treatment response on all symptom dimensions. These results can assist refractory psychosis units by providing information on how PANSS symptom dimensions respond to treatment and how this depends on diagnostic category.

  13. Pharyngeal shape and dimensions in healthy subjects, snorers, and patients with obstructive sleep apnoea.

    OpenAIRE

    Rodenstein, D O; Dooms, G; Thomas, Y; Liistro, G; Stanescu, D C; Culée, C; Aubert-Tulkens, G

    1990-01-01

    To characterise the relation between pharyngeal anatomy and sleep related disordered breathing, 17 men with complaints of snoring were studied by all night polysomnography. Ten of them had obstructive sleep apnoea (mean (SD) apnoea-hypopnoea index 56.3 (41.7), age 52 (10) years, body mass index 31.4 (5.3) kg/m2); whereas seven were simple snorers (apnoea-hypopnoea index 6.7 (4.6), age 40 (17) years, body mass index 25.9 (4.3) kg/m2). The pharynx was studied by magnetic resonance imaging in al...

  14. Pembrolizumab in Treating Younger Patients With Recurrent, Progressive, or Refractory High-Grade Gliomas, Diffuse Intrinsic Pontine Gliomas, Hypermutated Brain Tumors, Ependymoma or Medulloblastoma

    Science.gov (United States)

    2018-06-18

    Constitutional Mismatch Repair Deficiency Syndrome; Lynch Syndrome; Malignant Glioma; Progressive Ependymoma; Progressive Medulloblastoma; Recurrent Brain Neoplasm; Recurrent Childhood Ependymoma; Recurrent Diffuse Intrinsic Pontine Glioma; Recurrent Medulloblastoma; Refractory Brain Neoplasm; Refractory Diffuse Intrinsic Pontine Glioma; Refractory Ependymoma; Refractory Medulloblastoma

  15. Study into the use of continuous positive airway pressure in obstructive sleep apnoea-hypopnoea syndrome patients with daytime drowsiness

    Directory of Open Access Journals (Sweden)

    Clélia Maria Ribeiro Franco

    2009-03-01

    Full Text Available Background: Obstructive sleep apnoea-hypopnoea syndrome (OSAHS is a respiratory disorder with high morbidity and mortality. Continuous positive airway pressure (CPAP is the most commonly prescribed conservative treatment for adults with OSAHS. CPAP therapy normalises or decreases OSAHS symptoms and can reduce and prevent OSAHS complications. Aims: To evaluate adherence to nasal CPAP treatment and CPAP impact on daytime drowsiness. Method: A sample of 20 patients evaluated for daytime drowsiness using the Epworth sleepiness scale and interviewed for adherence to nasal CPAP use. Results: There was a significant decrease in the level of daytime sleepiness of the patients users of nasal CPAP (p=0.017; patients not using nasal CPAP experienced a decrease without statistical significance (p=0.162. 100% of CPAP users reported benefits and 50% of these reported related discomforts. Conclusions: Patients with OSAHS that use CPAP have a greater reduced level of sleepiness than those who do not use it. Resumo: Introdução: A síndroma da apneia-hipopneia obstrutiva do sono (SAHOS é um distúrbio respiratório de elevada morbimortalidade. A terapia com pressão positiva contínua das vias aéreas (CPAP representa o tratamento conservador mais prescrito para a SAHOS e tem o intuito de restabelecer a patência das vias aéreas, normalizando o índice de eventos respiratórios obstrutivos, corrigindo os sintomas. Objectivo: Avaliar o impacto do uso do CPAP nasal sobre a hipersonia diurna em portadores de SAHOS. Método: Amostra de vinte doentes portadores de SAHOS diagnosticados por estudo de polissonografia de noite inteira, usuários ou não de CPAP nasal, todos avaliados quanto à hipersonia diurna através da escala de sonolência de Epworth. Resultados: O decréscimo do nível de sonolência diurna dos usuários de CPAP nasal foi significante (p=0,017, enquanto para não usuários de CPAP nasal a m

  16. Endoscopic cyclophotocoagulation in refractory glaucoma after osteo-odonto-keratoprosthesis in Stevens-Johnson syndrome: a case report.

    Science.gov (United States)

    Forlini, Matteo; Adabache-Guel, Tania; Bratu, Adriana; Rossini, Paolo; Mingaine, Mpekethu Sam; Cavallini, Gian Maria; Forlini, Cesare

    2014-01-01

    To report successful treatment of refractive glaucoma in a patient submitted to osteo-odonto-keratoprosthesis surgery for Stevens-Johnson syndrome. An interventional case report. The patient is a 62-year-old Indian man with known Stevens-Johnson syndrome since 1972 secondary to tetracycline therapy, with bilateral dry eye and corneal blindness. He underwent symblepharon release surgery with mucous membrane graft in both eyes. Osteo-odonto-keratoprosthesis surgery was later performed on the left eye. He was submitted to 2 Ahmed valve implants to control secondary glaucoma but visual fields continued to worsen; hence, he underwent endoscopic 140° cyclophotocoagulation with a good control of IOP. Endoscopic cyclophotocoagulation as alternative treatment provides good results in refractory glaucoma after osteo-odonto-keratoprosthesis surgery.

  17. Automated processing of the single-lead electrocardiogram for the detection of obstructive sleep apnoea.

    Science.gov (United States)

    de Chazal, Philip; Heneghan, Conor; Sheridan, Elaine; Reilly, Richard; Nolan, Philip; O'Malley, Mark

    2003-06-01

    A method for the automatic processing of the electrocardiogram (ECG) for the detection of obstructive apnoea is presented. The method screens nighttime single-lead ECG recordings for the presence of major sleep apnoea and provides a minute-by-minute analysis of disordered breathing. A large independently validated database of 70 ECG recordings acquired from normal subjects and subjects with obstructive and mixed sleep apnoea, each of approximately eight hours in duration, was used throughout the study. Thirty-five of these recordings were used for training and 35 retained for independent testing. A wide variety of features based on heartbeat intervals and an ECG-derived respiratory signal were considered. Classifiers based on linear and quadratic discriminants were compared. Feature selection and regularization of classifier parameters were used to optimize classifier performance. Results show that the normal recordings could be separated from the apnoea recordings with a 100% success rate and a minute-by-minute classification accuracy of over 90% is achievable.

  18. Systemic inflammation: a key factor in the pathogenesis of cardiovascular complications in obstructive sleep apnoea syndrome?

    LENUS (Irish Health Repository)

    Ryan, S

    2012-02-01

    Obstructive sleep apnoea syndrome (OSAS) is a highly prevalent disease and is recognised as a major public health burden. Large-scale epidemiological studies have demonstrated an independent relationship between OSAS and various cardiovascular disorders. The pathogenesis of cardiovascular complications in OSAS is not completely understood but a multifactorial aetiology is likely. Inflammatory processes have emerged as critical in the pathogenesis of atherosclerosis at all stages of atheroma formation. Increased levels of various circulating markers of inflammation including tumour necrosis factor alpha (TNFalpha), interleukin 6 (IL6), IL-8 and C-reactive protein (CRP) have been reported as associated with future cardiovascular risk. There is increasing evidence of elevated inflammatory markers in OSAS with a significant fall after effective treatment with continuous positive airway pressure. This evidence is particularly strong for TNFalpha, whereas studies on IL6 and CRP have yielded conflicting results possibly due to the confounding effects of obesity. Cell culture and animal studies have significantly contributed to our understanding of the underlying mechanisms of the association between OSAS and inflammation. Intermittent hypoxia, the hallmark of OSAS, results in activation of pro-inflammatory transcription factors such as nuclear factor kappa B (NF-kappaB) and activator protein (AP)-1. These promote activation of various inflammatory cells, particularly lymphocytes and monocytes, with the downstream consequence of expression of pro-inflammatory mediators that may lead to endothelial dysfunction. This review provides a critical analysis of the current evidence for an association between OSAS, inflammation and cardiovascular disease, discusses basic mechanisms that may be responsible for this association and proposes future research possibilities.

  19. Deep Brain Stimulation of the internal globus pallidus in refractory Tourette Syndrome.

    Science.gov (United States)

    Smeets, A Y J M; Duits, A A; Plantinga, B R; Leentjens, A F G; Oosterloo, M; Visser-Vandewalle, V; Temel, Y; Ackermans, L

    2016-03-01

    Deep Brain Stimulation in psychiatric disorders is becoming an increasingly performed surgery. At present, seven different targets have been stimulated in Tourette Syndrome, including the internal globus pallidus. We describe the effects on tics and comorbid behavioral disorders of Deep Brain Stimulation of the anterior internal globus pallidus in five patients with refractory Tourette Syndrome. This study was performed as an open label study with follow-up assessment between 12 and 38 months. Patients were evaluated twice, one month before surgery and at long-term follow-up. Primary outcome was tic severity, assessed by several scales. Secondary outcomes were comorbid behavioral disorders, mood and cognition. The final position of the active contacts of the implanted electrodes was investigated and side effects were reported. Three males and two females were included with a mean age of 41.6 years (SD 9.7). The total post-operative score on the Yale Global Tic Severity Scale was significantly lower than the pre-operative score (42.2±4.8 versus 12.8±3.8, P=0.043). There was also a significant reduction on the modified Rush Video-Based Tic Rating Scale (13.0±2.0 versus 7.0±1.6, P=0.041) and in the total number of video-rated tics (259.6±107.3 versus 49.6±24.8, P=0.043). No significant difference on the secondary outcomes was found, however, there was an improvement on an individual level for obsessive-compulsive behavior. The final position of the active contacts was variable in our sample and no relationship between position and stimulation effects could be established. Our study suggests that Deep Brain Stimulation of the anterior internal globus pallidus is effective in reducing tic severity, and possibly also obsessive-compulsive behavior, in refractory Tourette patients without serious adverse events or side-effects. Copyright © 2016 Elsevier B.V. All rights reserved.

  20. Autologous stem cell transplantation in refractory Asherman′s syndrome: A novel cell based therapy

    Directory of Open Access Journals (Sweden)

    Neeta Singh

    2014-01-01

    Full Text Available Background : There is substantial evidence that adult stem cell populations exist in human endometrium, and hence it is suggested that either endogenous endometrial stem/progenitor cells can be activated or bone marrow derived stem cells can be transplanted in the uterine cavity for endometrial regeneration in Asherman′s syndrome (AS. Aims and Objectives : The objective was to evaluate the role of sub-endometrial autologous stem cell implantation in women with refractory AS in attaining menstruation and fertility. Setting : Tertiary care referral center. DESIGN: Prospective case series. Materials and Methods : Six cases of refractory AS with failed standard treatment option of hysteroscopic adhesiolysis in the past were included. Mononuclear stem cells (MNCs were implanted in sub-endometrial zone followed by exogenous oral estrogen therapy. Endometrial thickness (ET was assessed at 3, 6, and 9 months. RESULTS: Descriptive statistics and statistical analysis of study variables was carried out using STATA version 9.0. The mean MNC count was 103.3 × 106 (±20.45 with mean CD34+ count being 203,642 (±269,274. Mean of ET (mm at 3 months (4.05 ± 1.40, 6 months (5.46 ± 1.36 and 9 months (5.48 ± 1.14 were significantly (P < 0.05 increased from pretreatment level (1.38 ± 0.39. Five out of six patients resumed menstruation. Conclusion : The autologous stem cell implantation leads to endometrial regeneration reflected by restoration of menstruation in five out of six cases. Autologous stem cell implantation is a promising novel cell based therapy for refractory AS.

  1. Obstructive sleep apnoea is independently associated with the metabolic syndrome but not insulin resistance state

    Directory of Open Access Journals (Sweden)

    Sithole J

    2006-11-01

    Full Text Available Abstract Obstructive sleep apnoea (OSA is a cardio-metabolic disorder. Whether metabolic syndrome (MS, insulin resistance (IR and albuminuria are independently associated with OSA is unclear, but defining the interactions between OSA and various cardiovascular (CV risk factors independent of obesity facilitates the development of therapeutic strategies to mitigate their increased CV risks. We prospectively recruited 38 subjects with OSA and 41 controls. Anthropometric measurements, glucose, lipids, insulin and blood pressure (BP were measured after an overnight fast. IR state was defined as homeostasis model assessment (HOMA value >3.99 and MS diagnosed according to the International Diabetes Federation (IDF criteria. Subjects with OSA were more obese, more insulin resistant, more hyperglycaemic, had higher Epworth score (measure of day time somnolence and systolic blood pressure levels. The prevalence of MS was higher in OSA compared with non-OSA subjects (74% vs 24%, p 103 cm would predict MS in patients with OSA at 75–78% sensitivity and 61–64% specificity. The agreement between MS and IR state in this cohort is poor. Thus, OSA is associated with MS independent of obesity predominantly due to increased triglyceride, glucose and Epworth score values but not IR or microalbuminuria status. This observation suggests an alternative pathogenic factor mediating the increased cardiovascular risk in patients with OSA and MS, other than that due to IR. The independent link between Epworth score and MS in patients with OSA implicates the role of daytime sleepiness and chronic hypoxia as a potential mediator. Given the discordant between MS and IR state, measurement of waist is useful for predicting mainly MS but not insulin resistance status in patients with OSA. Appropriate pharmacological intervention targeting these independent factors is important in reducing the increased CV risks among patients with OSA.

  2. Sexual function in male patients with obstructive sleep apnoea

    DEFF Research Database (Denmark)

    Petersen, Marian; Kristensen, Ellids; Berg, Søren

    2010-01-01

    OBJECTIVE: Our objective was to investigate general and functional aspects of sexuality in male patients with a confirmed diagnosis of obstructive sleep apnoea (OSA) and compare the results with normative data. MATERIALS AND METHODS: We investigated 308 male patients (age 30-69) admitted to a sleep...... of sexuality were worse in patients with (untreated) OSA when compared with normative data. Both aspects were dependent on age, obesity, social factors and concomitant medication but not on the severity of OSA as reflected by the apnoea-hypopnoea index or subjective sleepiness. CONCLUSION: We conclude...... that although sexual dysfunction is more prevalent in OSA patients than in the general population, it is a complex problem relating more to age, obesity, social factors and comorbidity than to the severity of OSA....

  3. At least one in three people with Type 2 diabetes mellitus referred to a diabetes centre has symptomatic obstructive sleep apnoea

    DEFF Research Database (Denmark)

    Storgaard, H; Mortensen, B; Almdal, T

    2014-01-01

    , diabetic complications or medication use. In multiple regression analyses, age, BMI and HDL cholesterol levels were all significant, independent predictors of obstructive sleep apnoea. CONCLUSIONS: At least one third of people with Type 2 diabetes referred to a diabetes clinic in Denmark has symptomatic......AIMS: To investigate the prevalence of symptomatic obstructive sleep apnoea in unselected patients with Type 2 diabetes referred to a tertiary diabetes clinic. METHODS: In a cross-sectional design, all newly referred patients were offered a stepwise screening for obstructive sleep apnoea with: (1...... obstructive sleep apnoea. Our data suggest higher age, a compromised plasma lipid profile and a more obese phenotype in patients with Type 2 diabetes who have obstructive sleep apnoea, highlighting the need to focus on screening and treatment of obstructive sleep apnoea in these patients....

  4. Biofeedback as a first-line treatment for overactive bladder syndrome refractory to standard urotherapy in children.

    Science.gov (United States)

    Ebiloglu, Turgay; Kaya, Engin; Köprü, Burak; Topuz, Bahadır; Irkilata, Hasan Cem; Kibar, Yusuf

    2016-10-01

    Overactive bladder syndrome (OAB) and dysfunctional voiding (DV) are subgroups of lower urinary tract dysfunction (LUTD). Standard urotherapy is the first-line treatment option of OAB in children. The aim was to investigate the use of biofeedback as a first-line treatment option in OAB refractory to standard urotherapy, and determine the factors affecting efficacy. Between 2005 and 2015, we retrospectively analyzed a total of 136 hospital records of children with OAB who had not previously used any anticholinergics and were refractory to standard urotherapy. Patients with urgency and/or urge incontinence and/or making holding maneuvers to suppress urgency were defined as having OAB symptoms, and resolution of these complaints was defined as successful biofeedback therapy. Seventy-three of 136 OAB patients' urgency recovered by biofeedback therapy with the success rate of 53% (p biofeedback therapies, respectively (p Biofeedback can be thought of as the first-line treatment option when standard urotherapy fails in children with OAB. Copyright © 2016 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.

  5. Obstructive Sleep Apnoea | Gardner | South African Family Practice

    African Journals Online (AJOL)

    South African Family Practice. Journal Home · ABOUT THIS JOURNAL · Advanced Search · Current Issue · Archives · Journal Home > Vol 56, No 2 (2014) >. Log in or Register to get access to full text downloads. Username, Password, Remember me, or Register. Obstructive Sleep Apnoea. BM Gardner. Abstract.

  6. Obstructive Sleep Apnoea | Gardner | South African Family Practice

    African Journals Online (AJOL)

    South African Family Practice. Journal Home · ABOUT THIS JOURNAL · Advanced Search · Current Issue · Archives · Journal Home > Vol 56, No 5 (2014) >. Log in or Register to get access to full text downloads. Username, Password, Remember me, or Register. Obstructive Sleep Apnoea. BM Gardner. Abstract.

  7. Ketogenic diet treatment for pediatric super-refractory status epilepticus.

    Science.gov (United States)

    Appavu, Brian; Vanatta, Lisa; Condie, John; Kerrigan, John F; Jarrar, Randa

    2016-10-01

    We aimed to study whether ketogenic diet (KD) therapy leads to resolution of super-refractory status epilepticus in pediatric patients without significant harm. A retrospective review was performed at Phoenix Children's Hospital on patients with super-refractory status epilepticus undergoing ketogenic diet therapy from 2011 to 2015. Ten children with super-refractory status epilepticus, ages 2-16 years, were identified. 4/10 patients had immune mediated encephalitis, including Rasmussen encephalitis, anti-N-methyl-d-aspartate receptor encephalitis, and post-infectious mycoplasma encephalitis. Other etiologies included Lennox Gastaut Syndrome, non-ketotic hyperglycinemia, PCDH19 and GABRG2 genetic epilepsy, New Onset Refractory Status Epilepticus, and Febrile Infection-Related Epilepsy Syndrome. 4/10 patients' EEG features suggested focal with status epilepticus, and 6/10 suggested generalized with status epilepticus. Median hospital length was 61days and median ICU length was 27days. The median number of antiepileptic medications prior to diet initiation was 3.0 drugs, and the median after ketogenic diet treatment was 3.5 drugs. Median duration of status epilepticus prior to KD was 18days. 9/10 patients had resolution of super-refractory status epilepticus in a median of 7days after diet initiation. 8/9 patients were weaned off anesthesia within 15days of diet initiation, and within 1day of achieving ketonuria. 1/10 patients experienced side effects on the diet requiring supplementation. Most patients achieved resolution of status epilepticus on KD therapy, suggesting it could be an effective therapy that can be utilized early in the treatment of children with super refractory status epilepticus. Copyright © 2016. Published by Elsevier Ltd.

  8. Follow-up of conservatively treated sleep apnoea patients

    African Journals Online (AJOL)

    Health of School Children: Treatment of /ntestinal. Helminths and Schistosomiasis (WHO/GDS/IPI/GTD 92.1). Geneva: WHO, 1992. Accepted 17 June 1994. Follow-up of conservatively treated sleep apnoea patients. P. R. Bartel, J. Verster, P. J. Becker. Polysomnograms have been recorded at our laboratory since 1985 for ...

  9. Sleep apnoea in Australian men: disease burden, co-morbidities, and correlates from the Australian longitudinal study on male health

    Directory of Open Access Journals (Sweden)

    Chamara Visanka Senaratna

    2016-10-01

    Full Text Available Abstract Background Obstructive sleep apnoea is a common disorder with under-rated clinical impact, which is increasingly being recognised as having a major bearing on global disease burden. Men are especially vulnerable and become a priority group for preventative interventions. However, there is limited information on prevalence of the condition in Australia, its co-morbidities, and potential risk factors. Methods We used data from 13,423 adult men included in the baseline wave of Ten to Men, an Australian national study of the health of males, assembled using stratified cluster sampling with oversampling from rural and regional areas. Those aged 18–55 years self-completed a paper-based questionnaire that included a question regarding health professional-diagnosed sleep apnoea, physical and mental health status, and health-related behaviours. Sampling weights were used to account for the sampling design when reporting the prevalence estimates. Odds ratios were used to describe the association between health professional-diagnosed sleep apnoea and potential correlates while adjusting for age, country of birth, and body-mass index (BMI. Results Prevalence of self-reported health professional-diagnosed sleep apnoea increased from 2.2 % in age 18–25 years to 7.8 % in the age 45–55 years. Compared with those without sleep apnoea, those with sleep apnoea had significantly poorer physical, mental, and self-rated health as well as lower subjective wellbeing and poorer concentration/remembering (p < 0.001 for all. Sleep apnoea was significantly associated with older age (p < 0.001, unemployment (p < 0.001, asthma (p = 0.011, chronic obstructive pulmonary disease/chronic bronchitis (p = 0.002, diabetes (p < 0.001, hypercholesterolemia (p < 0.001, hypertension (p < 0.001, heart attack (p < 0.001, heart failure (p < 0.001, angina (p < 0.001, depression (p < 0.001, post-traumatic stress disorder (p

  10. Obstructive sleep apnoea among older patients attending the ...

    African Journals Online (AJOL)

    Aim: To determine the risk of obstructive sleep apnoea and sleep difficulties among older patients attending the geriatric clinic at the University College Hospital (UCH), Ibadan, Nigeria. Methods: The Berlin questionnaires was used to assess the risk of OSA in 843 older patients aged 60 years and above at geriatric clinic, ...

  11. Sleeping over a sleep disorder - Awareness of obstructive sleep apnoea as a modifiable risk factor for hypertension and stroke: A survey among health care professionals and medical students

    OpenAIRE

    Sharma, Sushma; Srijithesh, P. R.

    2013-01-01

    Background: Obstructive sleep apnoea (OSA) syndrome is an established and modifiable but under recognized risk factor for common disorders like stroke and hypertension. Objective: To assess awareness level of health care practitioners and medical students about OSA as a risk factor for stroke and hypertension. Methods: Questionnaire based survey with multiple response type and fill in the blanks type questions. The data was compiled and analyzed using SPSS version 19. Results: 180 participant...

  12. Randomised controlled trial of brief intervention with biofeedback and hypnotherapy in patients with refractory irritable bowel syndrome.

    Science.gov (United States)

    Dobbin, A; Dobbin, J; Ross, S C; Graham, C; Ford, M J

    2013-01-01

    Irritable bowel syndrome (IBS) is a common disorder associated with profoundly impaired quality of life and emotional distress. The management of refractory IBS symptoms remains challenging and non-pharmacological therapeutic approaches have been shown to be effective. We compared brief interventions with biofeedback and hypnotherapy in women referred by their GP with refractory IBS symptoms. Patients were randomised to one of two treatment groups, biofeedback or hypnotherapy, delivered as three one-hour sessions over 12 weeks. Symptom assessments were undertaken using validated, self-administered questionnaires. Two of the 128 consecutive IBS patients suitable for the study declined to consider nonpharmacological therapy and 29 patients did not attend beyond the first session. Of the 97 patients randomised into the study, 21 failed to attend the therapy session; 15 of 76 patients who attended for therapy dropped out before week 12 post-therapy. The mean (SD) change in IBS symptom severity score 12 weeks post-treatment in the biofeedback group was -116.8 (99.3) and in the hypnotherapy group -58.0 (101.1), a statistically significant difference between groups (difference=-58.8, 95% confidence interval [CI] for difference [-111.6, -6.1], p=0.029). In 61 patients with refractory IBS, biofeedback and hypnotherapy were equally effective at improving IBS symptom severity scores, total non-gastrointestinal symptom scores and anxiety and depression ratings during 24 weeks follow-up. Biofeedback may prove to be the more cost-effective option as it requires less expertise.

  13. Long-term CPAP treatment improves asthma control in patients with asthma and obstructive sleep apnoea.

    Science.gov (United States)

    Kauppi, Paula; Bachour, Patrick; Maasilta, Paula; Bachour, Adel

    2016-12-01

    Both asthma and obstructive sleep apnoea cause sleep disturbance, daytime sleepiness and diminished quality of life. Continuous positive airway pressure (CPAP) is efficient in reducing symptoms related to sleep apnoea. Here we report the impact of long-term use of CPAP on asthma symptoms. A survey questionnaire was distributed to all of our obstructive sleep apnoea patients with CPAP therapy in 2013. We used the Finnish version of the Asthma Control Test™ (ACT) and a visual analogue scale (0 = no symptoms, 100 = severe asthma symptoms). Asthma was defined as self-reported physician-diagnosed disease and a special reimbursement for asthma medication by the Social Insurance Institution. We sent 2577 questionnaires and received 1586 answers (61 %). One hundred ninety-seven patients were asthmatics with a prevalence of asthma among CPAP users of 13 %. We studied 152 patients (58 females) whose CPAP therapy was initiated after starting asthma medication. Their mean (SD) age was 62 (10) years, duration of CPAP 5.7 (4.7) years and their CPAP daily use was 6.3 (2.4) h. Self-reported asthma severity decreased significantly from 48.3 (29.6) to 33.1 (27.4) (p CPAP (P CPAP in patients with both asthma and obstructive sleep apnoea.

  14. Apnoea of prematurity – discontinuation of methylxanthines in a ...

    African Journals Online (AJOL)

    Background. Methylxanthines such as caffeine have been proven to reduce apnoea of prematurity and are often discontinued at 35 weeks' corrected gestational age (GA). Objective. To ascertain whether a caffeine protocol based on international guidelines is applicable in our setting, where GA is often uncertain. Methods.

  15. High versus standard dose caffeine for apnoea: a systematic review

    NARCIS (Netherlands)

    Vliegenthart, Roos; Miedema, Martijn; Hutten, Gerard J.; van Kaam, Anton H.; Onland, Wes

    2018-01-01

    Placebo-controlled trials have shown that caffeine is highly effective in treating apnoea of prematurity and reduces the risk of bronchopulmonary dysplasia (BPD) and neurodevelopmental impairment (NDI). To identify, appraise and summarise studies investigating the modulating effect of different

  16. Pulsed radiofrequency of brachial plexus under ultrasound guidance for refractory stump pain: a case report

    Directory of Open Access Journals (Sweden)

    Zheng B

    2017-11-01

    Full Text Available Bixin Zheng, Li Song, Hui Liu Department of Pain Management, West China Hospital of Sichuan University, Chengdu, China Abstract: The post-amputation (pain syndrome, including stump pain, phantom limb sensation, and phantom limb pain is common but difficult to treat. Refractory stump pain in the syndrome is an extremely challenging and troublesome clinical condition. Patients respond poorly to drugs, nerve blocks, and other effective treatments like spinal cord stimulation and surgery. Pulsed radiofrequency (PRF technique has been shown to be effective in reducing neuropathic pain. This report describes a patient with persistent and refractory upper limb stump pain being successfully relieved with PRF of brachial plexus under ultrasound guidance after a 6-month follow-up period, suggesting that PRF may be considered as an alternative treatment for refractory stump-neuroma pain. Keywords: ultrasound guidance, pulsed radiofrequency, brachial plexus, refractory stump pain 

  17. Vasoplegic syndrome during Whipple procedure.

    Science.gov (United States)

    Anandaswamy, Tejesh C; Rajappa, Geetha C; Krishnamachar, Harish

    2017-02-01

    Vasoplegic syndrome is an unusual cause of refractory hypotension under general anesthesia. It is commonly described in the setting of cardiac surgery, but rarely seen in noncardiac setting. We describe successful management of vasoplegic syndrome during Whipple procedure with vasopressin infusion. A high index of suspicion and prompt treatment with vasopressin can be lifesaving in patients with risk factors for vasoplegic syndrome who present with severe refractory hypotension and who respond poorly to fluid administration and routine vasopressor infusion. Copyright © 2016 Elsevier Inc. All rights reserved.

  18. Dynamic cycling in atrial size and flow during obstructive apnoea.

    Science.gov (United States)

    Pressman, Gregg S; Cepeda-Valery, Beatriz; Codolosa, Nicolas; Orban, Marek; Samuel, Solomon P; Somers, Virend K

    2016-01-01

    Obstructive sleep apnoea (OSA) is strongly associated with cardiovascular disease. However, acute cardiovascular effects of repetitive airway obstruction are poorly understood. While past research used a sustained Mueller manoeuver to simulate OSA we employed a series of gasping efforts to better simulate true obstructive apnoeas. This report describes acute changes in cardiac anatomy and flow related to sudden changes in intrathoracic pressure. 26 healthy, normal weight participants performed 5-6 gasping efforts (target intrathoracic pressure -40 mm Hg) while undergoing Doppler echocardiography. 14 participants had sufficient echocardiographic images to allow comparison of atrial areas during the manoeuver with baseline measurements. Mitral and tricuspid E-wave and A-wave velocities postmanoeuver were compared with baseline in all participants. Average atrial areas changed little during the manoeuver, but variance in both atrial areas was significantly greater than baseline. Further, an inverse relationship was noted with left atrial collapse and right atrial enlargement at onset of inspiratory effort. Significant inverse changes were noted in Doppler flow when comparing the first beat postmanoeuver (pMM1) with baseline. Mitral E-wave velocity increased 9.1 cm/s while tricuspid E-wave velocity decreased 7.0 cm/s; by the eighth beat postmanoeuver (pMM8) values were not different from baseline. Mitral and tricuspid A-wave velocities were not different from baseline at pMM1, but both were significantly higher by pMM8. Repetitive obstructive apnoeas produce dynamic, inverse changes in atrial size and Doppler flow across the atrioventricular valves. These observations have important implications for understanding the pathophysiology of OSA.

  19. Stiff baby syndrome er en sjælden årsag til neonatal hypertonicitet

    DEFF Research Database (Denmark)

    Rønne, Maria Sode; Nielsen, Preben Berg; Mogensen, Christian Backer

    2014-01-01

    Stiff baby syndrome (hyperekplexia) is a rare genetic disorder. The condition can easily be misdiagnosed as epilepsy or severe sepsis because of hypertonicity and seizure-like episodes and has an increased risk of severe apnoea and sudden infant death. Tapping of the nasal bridge inducing a start...

  20. Transnasal humidified rapid insufflation ventilatory exchange for oxygenation of children during apnoea: a prospective randomised controlled trial.

    Science.gov (United States)

    Riva, T; Pedersen, T H; Seiler, S; Kasper, N; Theiler, L; Greif, R; Kleine-Brueggeney, M

    2018-03-01

    Transnasal humidified rapid insufflation ventilatory exchange (THRIVE) comprises the administration of heated, humidified, and blended air/oxygen mixtures via nasal cannula at rates of ≥2 litres kg -1  min -1 . The aim of this randomized controlled study was to evaluate the length of the safe apnoea time using THRIVE with two different oxygen concentrations (100% vs 30% oxygen) compared with standard low-flow 100% oxygen administration. Sixty patients, aged 1-6 yr, weighing 10-20 kg, undergoing general anaesthesia for elective surgery, were randomly allocated to receive one of the following oxygen administration methods during apnoea: 1) low-flow 100% oxygen at 0.2 litres kg -1  min -1 ; 2) THRIVE 100% oxygen at 2 litres kg -1  min -1 ; and 3) THRIVE 30% oxygen at 2 litres kg -1  min -1 . Primary outcome was time to desaturation to 95%. Termination criteria included SpO 2 decreased to 95%, transcutaneous CO 2 increased to 65 mmHg, or apnoea time of 10 min. The median (interquartile range) [range] apnoea time was 6.9 (5.7-7.8) [2.8-10.0] min for low-flow 100% oxygen, 7.6 (6.2-9.1) [5.2-10.0] min for THRIVE 100% oxygen, and 3.0 (2.4-3.7) [0.2-5.3] min for THRIVE 30% oxygen. No significant difference was detected between apnoea times with low-flow and THRIVE 100% oxygen administration (P=0.15). THRIVE with 30% oxygen demonstrated significantly shorter apnoea times (Prate of transcutaneous CO 2 increase was 0.57 (0.49-0.63) [0.29-8.92] kPa min -1 without differences between the 3 groups (P=0.25). High-flow 100% oxygen (2 litres kg -1  min -1 ) administered via nasal cannulas did not extend the safe apnoea time for children weighing 10-20 kg compared with low-flow nasal cannula oxygen (0.2 litres kg -1  min -1 ). No ventilatory effect was observed with THRIVE at 2.0 litres kg -1  min -1 . NCT02979067. Copyright © 2017 British Journal of Anaesthesia. Published by Elsevier Ltd. All rights reserved.

  1. Magnesium treatment for patients with refractory status epilepticus due to POLG1-mutations

    NARCIS (Netherlands)

    Visser, Nora A.; Braun, Kees P. J.; Leijten, Frans S. S.; van Nieuwenhuizen, Onno; Wokke, John H. J.; van den Bergh, Walter M.

    2011-01-01

    Mutations in the gene encoding of the catalytic subunit of mtDNA polymerase gamma (POLG1) can cause typical Alpers' syndrome. Recently, a new POLG1 mutation phenotype was described, the so-called juvenile-onset Alpers' syndrome. This POLG1 mutation phenotype is characterized by refractory epilepsy

  2. Oral appliance therapy versus nasal continuous positive airway pressure in obstructive sleep apnoea syndrome: a randomised, placebo-controlled trial on self-reported symptoms of common sleep disorders and sleep-related problems.

    Science.gov (United States)

    Nikolopoulou, M; Byraki, A; Ahlberg, J; Heymans, M W; Hamburger, H L; De Lange, J; Lobbezoo, F; Aarab, G

    2017-06-01

    Obstructive sleep apnoea syndrome (OSAS) is associated with several sleep disorders and sleep-related problems. Therefore, the aim of this study was to compare the effects of a mandibular advancement device (MAD) with those of nasal continuous positive airway pressure (nCPAP) on self-reported symptoms of common sleep disorders and sleep-related problems in mild and moderate OSAS patients. In this randomised placebo-controlled trial, sixty-four OSAS patients (52·0 ± 9·6 years) were randomly assigned to an MAD, nCPAP or an intra-oral placebo appliance in a parallel design. All participants filled out the validated Dutch Sleep Disorders Questionnaire (SDQ) twice: one before treatment and one after six months of treatment. With 88 questions, thirteen scales were constructed, representing common sleep disorders and sleep-related problems. Linear mixed model analyses were performed to study differences between the groups for the different SDQ scales over time. The MAD group showed significant improvements over time in symptoms corresponding with 'insomnia', 'excessive daytime sleepiness', 'psychiatric sleep disorder', 'periodic limb movements', 'sleep apnoea', 'sleep paralysis', 'daytime dysfunction', 'hypnagogic hallucinations/dreaming', 'restless sleep', 'negative conditioning' and 'automatic behaviour' (range of P values: 0·000-0·014). These improvements in symptoms were, however, not significantly different from the improvements in symptoms observed in the nCPAP and placebo groups (range of P values: 0·090-0·897). It can be concluded that there is no significant difference between MAD and nCPAP in their positive effects on self-reported symptoms of common sleep disorders and sleep-related problems in mild and moderate OSAS patients. These beneficial effects may be a result of placebo effects. © 2017 John Wiley & Sons Ltd.

  3. Body Fat Distribution, Serum Leptin, And Insulin Resistance In Obese Subjects With Obstructive Sleep Apnoea

    OpenAIRE

    Hassan ZA*,Attia MF**, Ahmed AH**;Hassan HA***,

    2006-01-01

    Obstructive sleep apnoea (OS A) is strongly associated with obesity and is characterized by endocrine and metabolic changes. The aim of the present study is to clarify whether there is interrelationship between body fat, serum leptin, glucose-insulin metabolism and OSA. Subjects and measurements: we studied 23 obese subjects with OSA (13 males,& 10 females; age mean 36 ± 4.4 years; BMI: 31.7 ± 3.6 kg/m2; WHR: 1.2 ± .25 in males and 0.81+.5 in females ;Apnoea Index "AI"( 9.2 ±6.1) event/hour o...

  4. Stiff baby syndrome er en sjælden årsag til neonatal hypertonicitet

    DEFF Research Database (Denmark)

    Rønne, Maria Sode; Nielsen, Preben Berg; Mogensen, Christian Backer

    2014-01-01

    Stiff baby syndrome (hyperekplexia) is a rare genetic disorder. The condition can easily be misdiagnosed as epilepsy or severe sepsis because of hypertonicity and seizure-like episodes and has an increased risk of severe apnoea and sudden infant death. Tapping of the nasal bridge inducing a start...... response is the clinical hallmark. We report cases of two sisters born with stiff baby syndrome with hypertonicity, exaggerated startle reaction and cyanosis. The syndrome has a good prognosis if treated with clonazepam and both cases were developmental normal after one year....

  5. Approaches to refractory epilepsy

    Directory of Open Access Journals (Sweden)

    Jerome Engel

    2014-01-01

    Full Text Available Epilepsy is one of the most common serious neurological conditions, and 30 to 40% of people with epilepsy have seizures that are not controlled by medication. Patients are considered to have refractory epilepsy if disabling seizures continue despite appropriate trials of two antiseizure drugs, either alone or in combination. At this point, patients should be referred to multidisciplinary epilepsy centers that perform specialized diagnostic testing to first determine whether they are, in fact, pharmacoresistant, and then, if so, offer alternative treatments. Apparent pharmacoresistance can result from a variety of situations, including noncompliance, seizures that are not epileptic, misdiagnosis of the seizure type or epilepsy syndrome, inappropriate use of medication, and lifestyle issues. For patients who are pharmacoresistant, surgical treatment offers the best opportunity for complete freedom from seizures. Surgically remediable epilepsy syndromes have been identified, but patients with more complicated epilepsy can also benefit from surgical treatment and require more specialized evaluation, including intracranial EEG monitoring. For patients who are not surgical candidates, or who are unwilling to consider surgery, a variety of other alternative treatments can be considered, including peripheral or central neurostimulation, ketogenic diet, and complementary and alternative approaches. When such alternative treatments are not appropriate or effective, quality of life can still be greatly improved by the psychological and social support services offered by multidisciplinary epilepsy centers. A major obstacle remains the fact that only a small proportion of patients with refractory epilepsy are referred for expert evaluation and treatment.

  6. Refractory primary immune thrombocytopenia with subsequent del(5q) MDS

    DEFF Research Database (Denmark)

    Bech Mortensen, Thomas; Frederiksen, Henrik; Marcher, Claus Werenberg

    2017-01-01

    A patient with refractory primary immune thrombocytopenia (ITP) characterised by severe skin and mucosal bleedings was treated with several ITP-directed therapies including cyclophosphamide. He later developed therapy-related del(5q) myelodysplastic syndrome with no dysplastic morphological featu...

  7. Sex, stress and sleep apnoea: Decreased susceptibility to upper airway muscle dysfunction following intermittent hypoxia in females.

    Science.gov (United States)

    O'Halloran, Ken D; Lewis, Philip; McDonald, Fiona

    2017-11-01

    Obstructive sleep apnoea syndrome (OSAS) is a devastating respiratory control disorder more common in men than women. The reasons for the sex difference in prevalence are multifactorial, but are partly attributable to protective effects of oestrogen. Indeed, OSAS prevalence increases in post-menopausal women. OSAS is characterized by repeated occlusions of the pharyngeal airway during sleep. Dysfunction of the upper airway muscles controlling airway calibre and collapsibility is implicated in the pathophysiology of OSAS, and sex differences in the neuro-mechanical control of upper airway patency are described. It is widely recognized that chronic intermittent hypoxia (CIH), a cardinal feature of OSAS due to recurrent apnoea, drives many of the morbid consequences characteristic of the disorder. In rodents, exposure to CIH-related redox stress causes upper airway muscle weakness and fatigue, associated with mitochondrial dysfunction. Of interest, in adults, there is female resilience to CIH-induced muscle dysfunction. Conversely, exposure to CIH in early life, results in upper airway muscle weakness equivalent between the two sexes at 3 and 6 weeks of age. Ovariectomy exacerbates the deleterious effects of exposure to CIH in adult female upper airway muscle, an effect partially restored by oestrogen replacement therapy. Intriguingly, female advantage intrinsic to upper airway muscle exists with evidence of substantially greater loss of performance in male muscle during acute exposure to severe hypoxic stress. Sex differences in upper airway muscle physiology may have relevance to human OSAS. The oestrogen-oestrogen receptor α axis represents a potential therapeutic target in OSAS, particularly in post-menopausal women. Copyright © 2016 Elsevier B.V. All rights reserved.

  8. Polycystic ovarian syndrome.

    Science.gov (United States)

    Madnani, Nina; Khan, Kaleem; Chauhan, Phulrenu; Parmar, Girish

    2013-01-01

    Polycystic ovarian syndrome (PCOS) is a "multispeciality" disorder suspected in patients with irregular menses and clinical signs of hyperandrogenism such as acne, seborrhoea, hirsutism, irregular menses, infertility, and alopecia. Recently, PCOS has been associated with the metabolic syndrome. Patients may develop obesity, insulin resistance, acanthosis nigricans, Type 2 diabetes, dyslipidemias, hypertension, non-alcoholic liver disease, and obstructive sleep apnoea. Good clinical examination with hematological and radiological investigations is required for clinical evaluation. Management is a combined effort involving a dermatologist, endocrinologist, gynecologist, and nutritionist. Morbidity in addition includes a low "self image" and poor quality of life. Long term medications and lifestyle changes are essential for a successful outcome. This article focuses on understanding the normal and abnormal endocrine functions involved in the pathogenesis of PCOS. Proper diagnosis and management of the patient is discussed.

  9. Traditional Chinese Medicine for Refractory Nephrotic Syndrome: Strategies and Promising Treatments

    Science.gov (United States)

    Tu, Yuan-Chao

    2018-01-01

    Refractory nephrotic syndrome (RNS) is an immune-related kidney disease with poor clinical outcomes. Standard treatments include corticosteroids as the initial therapy and other immunosuppressants as second-line options. A substantial proportion of patients with RNS are resistant to or dependent on immunosuppressive drugs and often experience unremitting edema and proteinuria, cycles of remission and relapse, and/or serious adverse events due to long-term immunosuppression. Traditional Chinese medicine has a long history of treating complicated kidney diseases and holds great potential for providing effective treatments for RNS. This review describes the Chinese medical theories relating to the pathogenesis of RNS and discusses the strategies and treatment options using Chinese herbal medicine. Available preclinical and clinical evidence strongly supports the integration of traditional Chinese medicine and Western medicine for improving the outcome of RNS. Herbal medicine such as Astragalus membranaceus, Stephania tetrandra S. Moore, and Tripterygium wilfordii Hook F can serve as the alternative therapy when patients fail to respond to immunosuppression or as the complementary therapy to improve therapeutic efficacy and reduce side effects of immunosuppressive agents. Wuzhi capsules (Schisandra sphenanthera extract) with tacrolimus and tetrandrine with corticosteroids are two herb-drug combinations that have shown great promise and warrant further studies. PMID:29507594

  10. Sleep apnoea and driving: how can this be dealt with?

    Directory of Open Access Journals (Sweden)

    J. Krieger

    2007-12-01

    Full Text Available Excessive daytime sleepiness has long been known to be associated with an increased risk of often particularly severe traffic accidents. Obstructive sleep apnoea (OSA is among the most prevalent conditions leading to excessive daytime sleepiness, in addition to impaired cognitive function, both of which are likely to impair driving ability. An increased risk of traffic accidents has been demonstrated repeatedly, in association with OSA, as well its normalisation with effective treatment. However, it seems that not all patients are at equal risk, but it is not clear how to identify when and how at-risk patients can be identified. Nevertheless, some European countries have made specific regulations concerning OSA and/or excessive daytime sleepiness and the capacity to obtain or to keep a driving license. Most countries have the general rule that "a driving license should not be given or renewed to any candidate or license holder suffering from a disorder ... likely to compromise safety on the road", without a specific mention of sleepiness and/or sleep apnoea. However, the way in which such a statement is applied and the measures taken to identify unfit drivers vary greatly from country to country. In addition, in those countries that have made specific regulations, no evaluation of their efficacy in reducing sleepiness-related accidents is available. In practice, it is the physician's responsibility to inform the untreated obstructive sleep apnoea patient about the risk associated with their condition, and about the regulations that prevail in their country, if relevant; only in a few countries, is the physician allowed (or compelled to report the unfit patient to the licensing authorities. Although it is generally accepted that the treated patient may be allowed to drive, the specific treatment conditions that eliminate the risk are not clearly established.

  11. Long-term follow-up of pallidal Deep Brain Stimulation in teenagers with refractory Tourette syndrome and comorbid psychiatric disorders: About three cases.

    Science.gov (United States)

    Hauseux, P-A; Cyprien, F; Cif, L; Gonzalez, V; Boulenger, J-P; Coubes, P; Capdevielle, D

    2017-01-01

    Tourette syndrome (TS) is a complex neuropsychiatric disorder associated with comorbid psychiatric disorders. Peak of tic severity typically occurs in early adolescence and impacts quality of life. Since 1999, promising therapeutic effects of Deep Brain Stimulation (DBS) have been reported in tic reduction for adults with refractory TS. The aim of the study was to assess the long-term risk-benefit ratio of pallidal DBS for young patients with refractory TS and severe comorbid psychiatric disorders. We retrospectively assessed the long-term clinical outcomes of three adolescents who underwent pallidal DBS for the treatment of refractory TS. The mean duration of follow-up was 52 months in our case series. We observed that motor tics decreased with posteroventral GPi DBS in all patients, without reaching a continuous significance over the long-term follow-up. Self-reported social inclusion was globally improved, despite lack of efficacy of DBS on comorbid conditions. These findings suggest a long-term therapeutic benefit of early DBS intervention for highly socially impaired young patients suffering from intractable TS with severe comorbid psychiatric conditions. Further studies are needed to determine the most effective targets of DBS on both tics and comorbid psychiatric profile of TS. Copyright © 2016 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.

  12. Successful diuretics treatment of protein-losing enteropathy in Noonan syndrome.

    Science.gov (United States)

    Mizuochi, Tatsuki; Suda, Kenji; Seki, Yoshitaka; Yanagi, Tadahiro; Yoshimoto, Hironaga; Kudo, Yoshiyuki; Iemura, Motofumi; Tanikawa, Ken; Matsuishi, Toyojiro

    2015-04-01

    There are few reports on successful high-dose spironolactone treatment of refractory protein-losing enteropathy (PLE) caused by Fontan procedure. We report successful diuretics treatment with spironolactone and furosemide at standard dose, of refractory PLE in a patient with Noonan syndrome and repaired congenital heart disease. This is the first successful application of diuretics treatment in a patient with refractory PLE without Fontan procedure. This case illustrates that diuretics treatment can be the first-line treatment of PLE regardless of the causative physiology, and can be effective in refractory PLE with Noonan syndrome. © 2015 Japan Pediatric Society.

  13. Efficacy of daytime continuous positive airway pressure titration in severe obstructive sleep apnoea.

    Science.gov (United States)

    Rudkowski, J C; Verschelden, P; Kimoff, R J

    2001-09-01

    The aim of this study was to evaluate manual nasal continuous positive airway pressure (nCPAP) titration during daytime polysomnography compared with conventional overnight titration for patients with severe obstructive sleep apnoea. Thirty-two patients who underwent daytime titration were retrospectively matched (for age, sex, body mass index and apnoea/hypopnoea index (AHI)) to a group titrated overnight during the same period. Successful titration was defined as the identification of the nCPAP level (effective nCPAP (Peff)) required to eliminate respiratory events during all sleep stages. After 3 months of therapy on nCPAP at Peff, nCPAP utilization history was obtained and a group of patients underwent a repeat polysomnogram (PSG) and completed a follow-up Epworth Sleepiness Scale (ESS) score. Initial titration was successful in 91% of daytime patients and 91% of overnight patients. The success of daytime titration was not related to diagnostic AHI or ESS score. Subjective nCPAP utilization was statistically similar in both groups. On the follow-up PSG, there were no significant differences between daytime (n=11) and overnight (n=11) patients in measures of sleep quality or respiratory disturbance. Both groups demonstrated similar and significant improvements in ESS score. These findings suggest that the effective nasal continuous positive airway pressure can be accurately established during daytime titration in a substantial proportion of severe, symptomatic obstructive sleep apnoea patients.

  14. Tocilizumab for refractory organising pneumonia associated with Sjögren's disease.

    Science.gov (United States)

    Justet, Aurelien; Ottaviani, Sebastien; Dieudé, Philippe; Taillé, Camille

    2015-05-14

    Lung involvement in primary Sjögren syndrome occurs in approximately 10-20% of patients. Tocilizumab, an anti-interleukin-6 receptor antibody, has demonstrated efficacy and safety in small series of systemic sclerosis, and systemic lupus erythematosus, but its effect on interstitial lung manifestations of connective tissue diseases is not well known. We report the use of tocilizumab in a refractory organising pneumonia associated with Sjögren's disease. Our observation suggests that tocilizumab could be an alternative therapeutic in refractory organising pneumonia. 2015 BMJ Publishing Group Ltd.

  15. Polycystic ovarian syndrome

    Directory of Open Access Journals (Sweden)

    Nina Madnani

    2013-01-01

    Full Text Available Polycystic ovarian syndrome (PCOS is a "multispeciality" disorder suspected in patients with irregular menses and clinical signs of hyperandrogenism such as acne, seborrhoea, hirsutism, irregular menses, infertility, and alopecia. Recently, PCOS has been associated with the metabolic syndrome. Patients may develop obesity, insulin resistance, acanthosis nigricans, Type 2 diabetes, dyslipidemias, hypertension, non-alcoholic liver disease, and obstructive sleep apnoea. Good clinical examination with hematological and radiological investigations is required for clinical evaluation. Management is a combined effort involving a dermatologist, endocrinologist, gynecologist, and nutritionist. Morbidity in addition includes a low "self image" and poor quality of life. Long term medications and lifestyle changes are essential for a successful outcome. This article focuses on understanding the normal and abnormal endocrine functions involved in the pathogenesis of PCOS. Proper diagnosis and management of the patient is discussed.

  16. Ibrutinib and Azacitidine for Treatment of Higher Risk Myelodysplastic Syndrome

    Science.gov (United States)

    2018-03-01

    Chronic Myelomonocytic Leukemia; de Novo Myelodysplastic Syndrome; Previously Treated Myelodysplastic Syndrome; Refractory Anemia With Excess Blasts in Transformation; Secondary Myelodysplastic Syndrome

  17. CT-guided stellate ganglion blockade vs. radiofrequency neurolysis in the management of refractory type I complex regional pain syndrome of the upper limb

    Energy Technology Data Exchange (ETDEWEB)

    Kastler, Adrian [University Hospital CHU Gabriel Montpied, Radiology Department, Clermont-Ferrand (France); Franche Comte University, I4S Laboratory-EA 4268-IFR 133, Besancon (France); CHU Clermont-Ferrand, Hopital Gabriel Montpied, Clermont-Ferrand (France); Aubry, Sebastien; Kastler, Bruno [University Hospital CHU Jean Minjoz, Radiology and Interventional Pain Unit, Besancon (France); Franche Comte University, I4S Laboratory-EA 4268-IFR 133, Besancon (France); Sailley, Nicolas; Michalakis, Demosthene [University Hospital CHU Jean Minjoz, Radiology and Interventional Pain Unit, Besancon (France); Siliman, Gaye [University Hospital CHU St Jacques, Clinical Investigation Center, Besancon (France); Gory, Guillaume [Franche Comte University, I4S Laboratory-EA 4268-IFR 133, Besancon (France); Lajoie, Jean-Louis [University Hospital CHU Jean Minjoz, Pain evaluation and Management Unit, Besancon (France)

    2013-05-15

    To describe and evaluate the feasibility and efficacy of CT-guided radiofrequency neurolysis (RFN) vs. local blockade of the stellate ganglion in the management of chronic refractory type I complex regional pain syndrome (CRPS) of the upper limb. Sixty-seven patients were included in this retrospective study between 2000 and 2011. All suffered from chronic upper limb type I CRPS refractory to conventional pain therapies. Thirty-three patients underwent stellate ganglion blockade and 34 benefited from radiofrequency neurolysis of the stellate ganglion. CT guidance was used in both groups. The procedure was considered effective when pain relief was {>=}50 %, lasting for at least 2 years. Thirty-nine women (58.2 %) and 28 men (41.8 %) with a mean age of 49.5 years were included in the study. Univariate analysis performed on the blockade and RFN groups showed a significantly (P < 0.0001) higher success rate in the RFN group (67.6 %, 23/34) compared with the blockade group (21.2 %, 7/33) with an odds ratio of 7.76. CT-guided radiofrequency neurolysis of the stellate ganglion is a safe and successful treatment of chronic refractory type I CRPS of the upper limb. It appears to be more effective than stellate ganglion blockade. (orig.)

  18. Circulating cell-derived microparticles in patients with minimally symptomatic obstructive sleep apnoea.

    Science.gov (United States)

    Ayers, L; Ferry, B; Craig, S; Nicoll, D; Stradling, J R; Kohler, M

    2009-03-01

    Moderate-severe obstructive sleep apnoea (OSA) has been associated with several pro-atherogenic mechanisms and increased cardiovascular risk, but it is not known if minimally symptomatic OSA has similar effects. Circulating cell-derived microparticles have been shown to have pro-inflammatory, pro-coagulant and endothelial function-impairing effects, as well as to predict subclinical atherosclerosis and cardiovascular risk. In 57 patients with minimally symptomatic OSA, and 15 closely matched control subjects without OSA, AnnexinV-positive, platelet-, leukocyte- and endothelial cell-derived microparticles were measured by flow cytometry. In patients with OSA, median (interquartile range) levels of AnnexinV-positive microparticles were significantly elevated compared with control subjects: 2,586 (1,566-3,964) microL(-1) versus 1,206 (474-2,501) microL(-1), respectively. Levels of platelet-derived and leukocyte-derived microparticles were also significantly higher in patients with OSA (2,267 (1,102-3,592) microL(-1) and 20 (14-31) microL(-1), respectively) compared with control subjects (925 (328-2,068) microL(-1) and 15 (5-23) microL(-1), respectively). Endothelial cell-derived microparticle levels were similar in patients with OSA compared with control subjects (13 (8-25) microL(-1) versus 11 (6-17) microL(-1)). In patients with minimally symptomatic obstructive sleep apnoea, levels of AnnexinV-positive, platelet- and leukocyte-derived microparticles are elevated when compared with closely matched control subjects without obstructive sleep apnoea. These findings suggest that these patients may be at increased cardiovascular risk, despite being minimally symptomatic.

  19. Brentuximab Vedotin Treatment for Primary Refractory Hodgkin Lymphoma

    Directory of Open Access Journals (Sweden)

    Hung-Bo Wu

    2013-01-01

    Full Text Available Up to 40% of patients with advanced Hodgkin lymphoma (HL become refractory or relapsed after current standard chemotherapy, among which primary refractory HL confers a particularly poor outcome. With intensive salvage chemotherapy and autologous stem cell transplantation, the long-term remission rate for these patients was only 30%, but more selective treatments with higher therapeutic index are needed. We report the experience of using a new anti-CD30 immunotoxin, brentuximab vedotin, in salvage treatment of a 30-year-old woman with primary refractory Hodgkin lymphoma. The patient presented with SVC syndrome due to the bulky mediastinal tumor and was confirmed to have classical Hodgkin lymphoma, nodular sclerosis type, stage IIIA. The tumor responded to induction chemotherapy transiently, but local progression was noted during subsequent cycles of treatment. Salvage radiotherapy to the mediastinal tumor, obtained no remission but was followed by rapid in-field progression and then lung metastasis. She declined stem cell transplantation and received salvage brentuximab vedotin (BV therapy, which induced dramatic shrinkage of tumor without significant side effects. Serial followup of PET/CT imaging confirmed a rapid and continuous complete remission for 12 months. Although durability of the remission needs further observation, this case illustrates the excellent efficacy of brentuximab vedotin in primary refractory Hodgkin lymphoma.

  20. Feasibility of robotic radical prostatectomy for medication refractory chronic prostatitis/chronic pelvic pain syndrome: Initial results

    Directory of Open Access Journals (Sweden)

    Sameer Chopra

    2016-01-01

    Full Text Available Four patients diagnosed with chronic prostatitis/chronic pelvic pain syndrome (CP/CPPS, met criteria for National Institute of Health (NIH Category III prostatitis, failed multiple medicinal treatments and underwent robotic radical prostatectomy (RRP. Median operative time (range: 157 (127–259 min. Validated functional questionnaires responses and NIH CP symptom index (NIH-CPSI score were collected for each patient's status at different time points pre- and post-operatively. Median decreases (range were: International Prostate Symptom Score - 14 (1–19; Sexual Health Inventory for Men - 6 (−14–22; and NIH-CPSI total - 23.5 (13–33. Median length of follow-up (range was 34 (24–43 months. RRP appears to be an option for carefully selected patients with medication-refractory CP/CPPS who understand that baseline sexual function may not be restored postoperatively.

  1. Pink Masks: Obstructive Sleep Apnoea and the Sociology of Diagnosis

    Directory of Open Access Journals (Sweden)

    SHARON HANCOCK

    2013-01-01

    Full Text Available The sociology of diagnosis takes a new look at diagnostic categories, the means by which they are delivered, and the social consequences of diagnosis for patient and professional alike. Considering the social elements which contribute to the recognition of disease categories and their consequences highlights important phenomena which can enrich the thinking of nurses regardless of whether or not they diagnose as part of their practice. However, the principles of the sociology of diagnosis, while widely debated in academe, have yet to penetrate nursing literature. In this discussion article, we use obstructive sleep apnoea in women as an exemplar to illustrate how a clearly material, pathophysiological disorder has, nonetheless, significant social “content.” We demonstrate the social structures and interests which shape obstructive sleep apnoea as a male disease, and the risks, paradoxically, of both under- and over-diagnosis that arise from this social construction. We use this example to exhort nurses to consider how the social and the biological intermesh and shape how we perceive disease and its impact. This should open the door for more responsive and responsible health care.

  2. Impact of sacubitril-valsartan combination in patients with chronic heart failure and sleep apnoea syndrome: the ENTRESTO-SAS study design.

    Science.gov (United States)

    Jaffuel, Dany; Molinari, Nicolas; Berdague, Philippe; Pathak, Atul; Galinier, Michel; Dupuis, Marion; Ricci, Jean-Etienne; Mallet, Jean-Pierre; Bourdin, Arnaud; Roubille, François

    2018-06-01

    Sleep-disordered breathing (SDB) is a highly prevalent co-morbidity in patients with chronic heart failure (CHF) and can play a detrimental role in the pathophysiology course of CHF. However, the best way to manage SDB in CHF remains a matter of debate. Sacubitril-valsartan has been included in the 2016 European Society of Cardiology guidelines as an alternative to angiotensin-converting enzyme inhibitors to further reduce the risk of progression of CHF, CHF hospitalization, and death in ambulatory patients. Sacubitril and valsartan are good candidates for correcting SDB of CHF patients because their known mechanisms of action are likely to counteract the pathophysiology of SDB in CHF. The ENTRESTO-SAS trial is a 3-month, multicentric, prospective, open-label real-life cohort study. Patients eligible for sacubitril-valsartan treatment (i.e. adults with left ventricular ejection fraction ≤35%, who remain symptomatic despite optimal treatment with an angiotensin-converting enzyme inhibitor, a beta-blocker, and a mineralocorticoid receptor antagonist) will be evaluated before and after 3 months of treatment (nocturnal ventilatory polygraphy, echocardiography, laboratory testing, and quality-of-life and SDB questionnaires). The primary outcome is the change in the Apnoea-Hypopnoea Index, before and after 3 months of treatment. One hundred twenty patients are required to detect a significant 20% improvement of the Apnoea-Hypopnoea Index with a power of 90% at an alpha risk of 5%. In the context of the SERVE-HF study, physicians are waiting for new trials and alternative therapies. We sought to assess in the ENTRESTO-SAS trial whether sacubitril-valsartan could improve the outcome of SDB in CHF patients. © 2018 The Authors. ESC Heart Failure published by John Wiley & Sons Ltd on behalf of the European Society of Cardiology.

  3. Auto-titrating continuous positive airway pressure treatment for obstructive sleep apnoea after acute quadriplegia (COSAQ): study protocol for a randomized controlled trial.

    Science.gov (United States)

    Berlowitz, David J; Ayas, Najib; Barnes, Maree; Brown, Douglas J; Cistulli, Peter A; Geraghty, Tim; Graham, Alison; Lee, Bonsan Bonne; Morris, Meg; O'Donoghue, Fergal; Rochford, Peter D; Ross, Jack; Singhal, Balraj; Spong, Jo; Wadsworth, Brooke; Pierce, Robert J

    2013-06-19

    Quadriplegia is a severe, catastrophic injury that predominantly affects people early in life, resulting in lifelong physical disability. Obstructive sleep apnoea is a direct consequence of quadriplegia and is associated with neurocognitive deficits, sleepiness and reduced quality of life. The usual treatment for sleep apnoea is nasal continuous positive airway pressure (CPAP); however, this is poorly tolerated in quadriplegia. To encourage patients to use this therapy, we have to demonstrate that the benefits outweigh the inconvenience. We therefore propose a prospective, multinational randomized controlled trial of three months of CPAP for obstructive sleep apnoea after acute quadriplegia. Specialist spinal cord injury centres across Australia, New Zealand, the UK and Canada will recruit medically stable individuals who have sustained a (new) traumatic quadriplegia (complete or incomplete second cervical to first thoracic level lesions). Participants will be screened for obstructive sleep apnoea using full, portable sleep studies. Those with an apnoea hypopnoea index greater than 10 per hour will proceed to an initial three-night trial of CPAP. Those who can tolerate CPAP for at least 4 hours on at least one night of the initial trial will be randomized to either usual care or a 3-month period of auto-titrating CPAP. The primary hypothesis is that nocturnal CPAP will improve neuropsychological functioning more than usual care alone. The secondary hypothesis is that the magnitude of improvement of neuropsychological function will be predicted by the severity of baseline sleepiness measures, sleep fragmentation and sleep apnoea. Neuropsychological tests and full polysomnography will be performed at baseline and 3 months with interim measures of sleepiness and symptoms of autonomic dysfunction measured weekly. Spirometry will be performed monthly. Neuropsychological tests will be administered by blinded assessors. Recruitment commenced in July 2009. The results of

  4. An unusual occurrence of Kleine-Levin syndrome in a man with refractory immune thrombocytopenic purpura: a case report.

    Science.gov (United States)

    Amirifard, Hamed; Barzkar, Farzaneh; Fazeli, Seyed Amirhossein; Hashemi, Seyed Mehdi

    2015-04-01

    Kleine-Levin syndrome is an extremely rare neurological entity characterized by recurrent episodes of hypersomnia which are sometimes associated with compulsive hyperphagia and behavioral changes. Autoimmunity has recently been proposed as a factor contributing to its pathogenesis. Immune thrombocytopenic purpura is a relatively common autoimmune disease showing a lot of complexity and uncertainty regarding its treatment regimens and its refractory nature in some cases. A 32-year-old Persian White man visited his private hematologist complaining of recent episodes of epistaxis and appearance of petechial lesions 24 hours after receiving a meningococcal vaccine. He had a history of immune thrombocytopenic purpura 13 years before his presentation. Based on his history and laboratory findings, his condition was diagnosed as a relapse of immune thrombocytopenic purpura and was managed accordingly. He did not respond to first-line corticosteroid regimens and later developed neurological symptoms as recurrent episodes of hypersomnia and hyperphagia. After a complete clinical and paraclinical evaluation and ruling out other possible conditions, he was given a diagnosis of Kleine-Levin syndrome. He was followed up for his immune thrombocytopenic purpura and received different treatment regimens none of which were adequately successful except intravenous immunoglobulin that was only temporarily effective. He has had 4 documented self-limited episodes of Kleine-Levin syndrome since his initial presentation. Immune thrombocytopenic purpura may be associated with meningococcal vaccination in adulthood. Responses to treatment in immune thrombocytopenic purpura vary among patients. Our patient only had a transient acceptable response to intravenous immunoglobulin while all other options failed to improve his platelet count. Concurrence of immune thrombocytopenic purpura and Kleine-Levin syndrome supports the role of autoimmunity as the proposed pathophysiological mechanism of

  5. Rituximab for nephrotic syndrome in children.

    Science.gov (United States)

    Iijima, Kazumoto; Sako, Mayumi; Nozu, Kandai

    2017-04-01

    Idiopathic nephrotic syndrome is the most common chronic glomerular disease in children. At least 20 % of children with this syndrome show frequent relapses and/or steroid dependence during or after immunosuppressive therapies, a condition defined as complicated frequently relapsing/steroid-dependent nephrotic syndrome (FRNS/SDNS). Approximately 1-3 % of children with idiopathic nephrotic syndrome are resistant to steroids and all immunosuppressive agents, a condition defined as refractory steroid-resistant nephrotic syndrome (SRNS); these SRNS children have a high risk of end-stage renal failure. Rituximab, a chimeric anti-CD20 monoclonal antibody, has been shown to be effective for patients with complicated FRNS/SDNS and refractory SRNS. This review describes the recent results of rituximab treatment applied to pediatric nephrotic syndrome, as well as those of our recent study, a multicenter, double-blind, randomized, placebo-controlled trial of rituximab for childhood-onset complicated FRNS/SDNS (RCRNS01). The overall efficacy and safety of rituximab for this disease are discussed.

  6. Management of Refractory Noninsulinoma Pancreatogenous Hypoglycemia Syndrome with Gastric Bypass Reversal: A Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Bhavana B. Rao

    2015-01-01

    Full Text Available Background. Roux-en-Y gastric bypass (RYGB is a commonly performed, effective bariatric procedure; however, rarely, complications such as postprandial hypoglycemia due to noninsulinoma pancreatogenous hypoglycemia syndrome (NIPHS may ensue. Management of refractory NIPHS is challenging. We report a case that was successfully treated with RYGB reversal. Case Report. A 58-year-old male with history of RYGB nine months earlier for morbid obesity presented for evaluation of postprandial, hypoglycemic seizures. Testing for insulin level, insulin antibodies, oral hypoglycemic agents, pituitary axis hormone levels, and cortisol stimulation was unrevealing. Computed tomography (CT scan of the abdomen was unremarkable. A 72-hour fast was completed without hypoglycemia. Mixed meal testing demonstrated endogenous hyperinsulinemic hypoglycemia (EHH and selective arterial calcium stimulation testing (SACST was positive. Strict dietary modifications, maximal medical therapy, gastrostomy tube feeding, and stomal reduction failed to alleviate symptoms. Ultimately, he underwent laparoscopic reversal of RYGB. Now, 9 months after reversal, he has markedly reduced hypoglycemia burden. Discussion. Hyperfunctioning islets secondary to exaggerated incretin response and altered intestinal nutrient delivery are hypothesized to be causative in NIPHS. For refractory cases, there is increasing skepticism about the safety and efficacy of pancreatic resection. RYGB reversal may be successful.

  7. Effect of Maximal Apnoea Easy-Going and Struggle Phases on Subarachnoid Width and Pial Artery Pulsation in Elite Breath-Hold Divers.

    Directory of Open Access Journals (Sweden)

    Pawel J Winklewski

    Full Text Available The aim of the study was to assess changes in subarachnoid space width (sas-TQ, the marker of intracranial pressure (ICP, pial artery pulsation (cc-TQ and cardiac contribution to blood pressure (BP, cerebral blood flow velocity (CBFV and cc-TQ oscillations throughout the maximal breath hold in elite apnoea divers. Non-invasive assessment of sas-TQ and cc-TQ became possible due to recently developed method based on infrared radiation, called near-infrared transillumination/backscattering sounding (NIR-T/BSS.The experimental group consisted of seven breath-hold divers (six men. During testing, each participant performed a single maximal end-inspiratory breath hold. Apnoea consisted of the easy-going and struggle phases (characterised by involuntary breathing movements (IBMs. Heart rate (HR was determined using a standard ECG. BP was assessed using the photoplethysmography method. SaO2 was monitored continuously with pulse oximetry. A pneumatic chest belt was used to register thoracic and abdominal movements. Cerebral blood flow velocity (CBFV was estimated by a 2-MHz transcranial Doppler ultrasonic probe. sas-TQ and cc-TQ were measured using NIR-T/BSS. Wavelet transform analysis was performed to assess cardiac contribution to BP, CBFV and cc-TQ oscillations.Mean BP and CBFV increased compared to baseline at the end of the easy phase and were further augmented by IBMs. cc-TQ increased compared to baseline at the end of the easy phase and remained stable during the IBMs. HR did not change significantly throughout the apnoea, although a trend toward a decrease during the easy phase and recovery during the IBMs was visible. Amplitudes of BP, CBFV and cc-TQ were augmented. sas-TQ and SaO2 decreased at the easy phase of apnoea and further decreased during the IBMs.Apnoea increases intracranial pressure and pial artery pulsation. Pial artery pulsation seems to be stabilised by the IBMs. Cardiac contribution to BP, CBFV and cc-TQ oscillations does not

  8. Pembrolizumab Alone or With Idelalisib or Ibrutinib in Treating Patients With Relapsed or Refractory Chronic Lymphocytic Leukemia or Other Low-Grade B-Cell Non-Hodgkin Lymphomas

    Science.gov (United States)

    2017-06-30

    Recurrent Chronic Lymphocytic Leukemia; Recurrent Extranodal Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Lymphoplasmacytic Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Nodal Marginal Zone Lymphoma; Recurrent Small Lymphocytic Lymphoma; Recurrent Splenic Marginal Zone Lymphoma; Refractory Chronic Lymphocytic Leukemia; Refractory Extranodal Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue; Refractory Follicular Lymphoma; Refractory Lymphoplasmacytic Lymphoma; Refractory Nodal Marginal Zone Lymphoma; Refractory Small Lymphocytic Lymphoma; Refractory Splenic Marginal Zone Lymphoma; Richter Syndrome; Waldenstrom Macroglobulinemia

  9. Mandibular advancement appliance for obstructive sleep apnoea: results of a randomised placebo controlled trial using parallel group design

    DEFF Research Database (Denmark)

    Petri, N.; Svanholt, P.; Solow, B.

    2008-01-01

    The aim of this trial was to evaluate the efficacy of a mandibular advancement appliance (MAA) for obstructive sleep apnoea (OSA). Ninety-three patients with OSA and a mean apnoea-hypopnoea index (AHI) of 34.7 were centrally randomised into three, parallel groups: (a) MAA; (b) mandibular non......). Eighty-one patients (87%) completed the trial. The MAA group achieved mean AHI and Epworth scores significantly lower (P group and the no-intervention group. No significant differences were found between the MNA group and the no-intervention group. The MAA group had...

  10. Efficacy and Safety of Rituximab in Children with Refractory Nephrotic Syndrome; A Multicenter Clinical Trial

    Directory of Open Access Journals (Sweden)

    Yo Han Ahn

    2014-06-01

    Conclusions: In this interim analysis of clinical trial to evaluate the efficacy and safety of RTX in children with refractory NS, RTX treatment for refractory NS was safe and effective, especially in patients with DNS.

  11. Wolf-Hirschhorn (4p-) syndrome with West syndrome.

    Science.gov (United States)

    Motoi, Hirotaka; Okanishi, Tohru; Kanai, Sotaro; Yokota, Takuya; Yamazoe, Tomohiro; Nishimura, Mitsuyo; Fujimoto, Ayataka; Yamamoto, Takamichi; Enoki, Hideo

    2016-01-01

    Wolf-Hirschhorn syndrome (WHS) is a chromosome disorder (4p-syndrome) which is characterized by craniofacial features and epileptic seizures. Here, we report a case of WHS with West syndrome, in whom the seizures were refractory to several antiepileptic drugs but were responsive to the addition of lamotrigine. The patient had epileptic spasms at age seven months. The interictal electroencephalogram was hypsarrhythmic. After adding lamotrigine, seizures decreased remarkably, and spasms disappeared. We have identified and described the very rare case of a girl with WHS who also developed West syndrome. In this case, adding lamotrigine to her medications effectively treated the spasms.

  12. Cerebral gigantism with West syndrome.

    Science.gov (United States)

    Ray, Munni; Malhi, P; Bhalla, A K; Singhi, P D

    2003-07-01

    A case of cerebral gigantism (Sotos syndrome) with West syndrome in a one-year-old male child is reported. The case had a large stature, typical facies and neurodevelopmental delay along with infantile spasms, which were refractory to treatment with valproate and clonazepam.

  13. Outcomes in coronary artery disease patients with sleepy obstructive sleep apnoea on CPAP

    NARCIS (Netherlands)

    Peker, Yuksel; Thunstrom, Erik; Glantz, Helena; Wegscheider, Karl; Eulenburg, Christine

    2017-01-01

    Coronary artery disease (CAD) patients with obstructive sleep apnoea (OSA) have increased risk for major adverse cardiovascular and cerebrovascular events (MACCEs) compared with CAD patients without OSA. We aimed to address if the risk is similar in both groups when OSA patients are treated. This

  14. Clinical Features of Refractory Ascites in Outpatients

    Directory of Open Access Journals (Sweden)

    Wanda Regina Caly

    Full Text Available OBJECTIVES: To present the clinical features and outcomes of outpatients who suffer from refractory ascites. METHODS: This prospective observational study consecutively enrolled patients with cirrhotic ascites who submitted to a clinical evaluation, a sodium restriction diet, biochemical blood tests, 24 hour urine tests and an ascitic fluid analysis. All patients received a multidisciplinary evaluation and diuretic treatment. Patients who did not respond to the diuretic treatment were controlled by therapeutic serial paracentesis, and a transjugular intrahepatic portosystemic shunt was indicated for patients who required therapeutic serial paracentesis up to twice a month. RESULTS: The most common etiology of cirrhosis in both groups was alcoholism [49 refractory (R and 11 non-refractory ascites (NR]. The majority of patients in the refractory group had Child-Pugh class B cirrhosis (p=0.034. The nutritional assessment showed protein-energy malnutrition in 81.6% of the patients in the R group and 35.5% of the patients in the NR group, while hepatic encephalopathy, hernia, spontaneous bacterial peritonitis, upper digestive hemorrhage and type 2 hepatorenal syndrome were present in 51%, 44.9%, 38.8%, 38.8% and 26.5% of the patients in the R group and 9.1%, 18.2%, 0%, 0% and 0% of the patients in the NR group, respectively (p=0.016, p=0.173, p=0.012, p=0.012, and p=0.100, respectively. Mortality occurred in 28.6% of the patients in the R group and in 9.1% of the patients in the NR group (p=0.262. CONCLUSION: Patients with refractory ascites were malnourished, suffered from hernias, had a high prevalence of complications and had a high postoperative death frequency, which was mostly due to infectious processes.

  15. A single qualitative study can show same findings as years of quantitative research: Obstructive sleep apnoea as an example

    Directory of Open Access Journals (Sweden)

    Howard Tandeter

    2016-06-01

    Full Text Available Background Many years of quantitative research led to our present knowledge of the symptoms and associated features (S&AF of the obstructive sleep apnoea (OSA syndrome. Aims 1. To prove that a qualitative research approach may identify symptoms and associated features of OSA in less time/effort than that used in a quantitative approach; 2. To describe the experience of patients with OSA and the effects of the syndrome on their quality of life and that of their spouses and families (issues that quantitative methods fail to recognize. Methods We used a narrative inquiry methodology (qualitative research. The sample was selected using the “snowball sampling technique". The sample included 10 patients with moderate to severe OSA who had good adherence to CPAP and significant clinical improvement after treatment, and 3 of the patient’s spouses. Results The following issues were identified: A long pre-diagnosis phase of OSA (20 years in one of the patients; Characteristic S&AF of the syndrome as experienced by patients and their spouses; The need for increased awareness of both the public and the medical establishment in regards to this disorder; Premature ejaculation (not reported previously and nightmares (non-conclusive in the literature were identified and improved with CPAP therapy. Conclusion With the use of quantitative research methods it took decades to discover things that we found in one simple qualitative study. We therefore urge scientists to use more often these qualitative methods when looking for S&AF of diseases and syndromes.

  16. Slagging gasifier refractories. A new pathway to longer refractory life

    Energy Technology Data Exchange (ETDEWEB)

    Schnake, Mark [Harbinson-Walker Refractories Company, Mexico, MO (United States)

    2013-07-01

    Solid fuel slagging gasification to convert coal or petroleum coke feedstocks into syngas has rapidly evolved over the last 25 years. The gasifier is a high temperature, high pressure reaction chamber. Operating temperatures are between 1250 and 1575 C. Pressures will be between 20.4 and 68 atm. Syngas has been typically used for chemical feedstocks, fuel for power plants, or for steam and hydrogen generation in other industrial applications. Ash which comes from the solid fuel during gasification has many impurities. It melts during the gasifier reactor operation forming a liquid that penetrates the refractory lining. Given time, the refractory will wear away from thermal spalling, structural spalling, or overheating of the refractory. In some cases, all three wear mechanisms are seen in the same gasifier lining. Industry users have identified refractory life as one major limiting factor in worldwide use of this technology. Users have stated if the refractory liner can increase on-line availability of the gasifier operation, more industry acceptance of this technology is possible. Harbison-Walker Refractories Company will review destructive factors affecting lining life and discuss new refractory materials that have dramatically increased gasifier lining life and reliability. New refractory materials will be presented and supported by field trial results and post mortem analysis.

  17. Metabolic syndrome, diet and exercise.

    Science.gov (United States)

    De Sousa, Sunita M C; Norman, Robert J

    2016-11-01

    Polycystic ovary syndrome (PCOS) is associated with a range of metabolic complications including insulin resistance (IR), obesity, dyslipidaemia, hypertension, obstructive sleep apnoea (OSA) and non-alcoholic fatty liver disease. These compound risks result in a high prevalence of metabolic syndrome and possibly increased cardiovascular (CV) disease. As the cardiometabolic risk of PCOS is shared amongst the different diagnostic systems, all women with PCOS should undergo metabolic surveillance though the precise approach differs between guidelines. Lifestyle interventions consisting of increased physical activity and caloric restriction have been shown to improve both metabolic and reproductive outcomes. Pharmacotherapy and bariatric surgery may be considered in resistant metabolic disease. Issues requiring further research include the natural history of PCOS-associated metabolic disease, absolute CV risk and comparative efficacy of lifestyle interventions. Copyright © 2016 Elsevier Ltd. All rights reserved.

  18. Sleep apnoea and driving risk: the need for regulation

    Directory of Open Access Journals (Sweden)

    Walter T. McNicholas

    2015-12-01

    Full Text Available Obstructive sleep apnoea syndrome (OSAS is a highly prevalent chronic respiratory disorder with prevalence among adult males of ≥10%. The most common daytime symptom associated with OSAS is excessive sleepiness, which in more severe manifestations can result in sleepiness at the wheel while driving and probably contributes to the substantial increase in accident risk among patients with OSAS. Fortunately, current evidence indicates that successful therapy of OSAS, particularly with continuous positive airway pressure, can bring the accident risk down to levels similar to an equivalent general population. The recognition of the increased driving accident risk in OSAS prompted the Transport and Mobility Directorate of the European Commission to establish a working group on this topic in 2012, which ultimately led to a revision of Annex III of the EU Driving Licence Directive, which is subject to mandatory implementation by European Union member states by December 2015. This directive specifies that patients with moderate or severe OSAS associated with significant daytime sleepiness should be prohibited from driving until effective therapy is established. These new regulations are designed to balance the legitimate objective of public safety with not penalising OSAS patients who are complying with effective therapy. Successful implementation of regulations on driving in OSAS patients must also include measures to educate relevant stakeholders including patients, medical personnel, traffic police and employers in the transport industry. The key objective is to encourage patients with possible OSAS to seek diagnosis and treatment and not to inhibit OSAS patients from coming forward.

  19. Sleep apnoea and driving risk: the need for regulation.

    Science.gov (United States)

    McNicholas, Walter T; Rodenstein, Daniel

    2015-12-01

    Obstructive sleep apnoea syndrome (OSAS) is a highly prevalent chronic respiratory disorder with prevalence among adult males of ≥10%. The most common daytime symptom associated with OSAS is excessive sleepiness, which in more severe manifestations can result in sleepiness at the wheel while driving and probably contributes to the substantial increase in accident risk among patients with OSAS. Fortunately, current evidence indicates that successful therapy of OSAS, particularly with continuous positive airway pressure, can bring the accident risk down to levels similar to an equivalent general population. The recognition of the increased driving accident risk in OSAS prompted the Transport and Mobility Directorate of the European Commission to establish a working group on this topic in 2012, which ultimately led to a revision of Annex III of the EU Driving Licence Directive, which is subject to mandatory implementation by European Union member states by December 2015. This directive specifies that patients with moderate or severe OSAS associated with significant daytime sleepiness should be prohibited from driving until effective therapy is established. These new regulations are designed to balance the legitimate objective of public safety with not penalising OSAS patients who are complying with effective therapy. Successful implementation of regulations on driving in OSAS patients must also include measures to educate relevant stakeholders including patients, medical personnel, traffic police and employers in the transport industry. The key objective is to encourage patients with possible OSAS to seek diagnosis and treatment and not to inhibit OSAS patients from coming forward. Copyright ©ERS 2015.

  20. High alumina refractories

    International Nuclear Information System (INIS)

    Simao, L.C.; Lopes, A.B.; Galvao Filho, N.B.; Souza, R.B. de

    1989-01-01

    High alumina refractories with 92 to 96.5% Al 2 O 3 were produced using brown and white fused as aggregate. Those refractories present only alumina-α and mullite as crystalline mineralogical phase. Other physical and chemical characteristics are similar to the ones found in refractories produced in Brazil, Japan and U.S.A. The most important physical and chemical tests used for the characterization of the raw materials and refractories, complemented by those realized at high temperatures, plus X-ray Difractometry and optical microscopy are presented, besides the refractory formulation and main parameters of production [pt

  1. A simple procedure for measuring pharyngeal sensitivity: a contribution to the diagnosis of sleep apnoea

    OpenAIRE

    Dematteis, M; Levy, P; Pepin, J

    2005-01-01

    Background: Patients with severe apnoea may have an impaired pharyngeal dilating reflex related to decreased pharyngeal sensitivity. The accuracy of a simple new procedure to measure pharyngeal sensitivity and to diagnose sleep disordered breathing (SDB) was investigated.

  2. Nelson syndrome: definition and management.

    Science.gov (United States)

    Barber, T M; Adams, E; Wass, J A H

    2014-01-01

    Nelson syndrome is an important complication of treatment with total bilateral adrenalectomy (TBA) for patients with refractory Cushing's disease. Although early cases of Nelson syndrome often presented with the clinical features of large sellar masses, the modern face of Nelson syndrome has changed primarily due to earlier detection (with highly resolved magnetic resonance imaging (MRI) and sensitive ACTH assays) and greater awareness of the condition, resulting in reduced morbidity and mortality. Although lack of administration of neoadjuvant pituitary radiotherapy post-TBA surgery may predict future development of Nelson syndrome, other predictive factors remain controversial. Therefore, Nelson syndrome should be screened for closely and long-term in all patients with a history of Cushing's disease and TBA. The diagnosis of Nelson syndrome remains controversial, and the pathogenesis of this condition is incompletely understood. Current hypotheses include the "released negative feedback" mechansism (residual pituitary corticotropinoma cells are "released" from the negative feedback effects of cortisol following TBA), and the "aggressive corticotropinoma" mechanism (Nelson syndrome is most likely to develop in those patients with refractory treatments - including TBA - for an underlying aggressive corticotropinoma). Effective management of Nelson syndrome with pituitary surgery and radiotherapy is often a challenge. Other therapies (such as Gamma Knife surgery and temozolomide) play an important role and merit further research into their efficacy and placement in the management pathway of Nelson syndrome. © 2014 Elsevier B.V. All rights reserved.

  3. Health, social and economical consequences of sleep-disordered breathing

    DEFF Research Database (Denmark)

    Jennum, Poul; Kjellberg, Jakob

    2011-01-01

    The objective direct and indirect costs of sleep-disordered breathing (snoring, sleep apnoea (SA) and obesity hypoventilation syndrome (OHS)) and the treatment are incompletely described.......The objective direct and indirect costs of sleep-disordered breathing (snoring, sleep apnoea (SA) and obesity hypoventilation syndrome (OHS)) and the treatment are incompletely described....

  4. [Obstructive sleep apnea syndrome. Analytical study of 63 cases].

    Science.gov (United States)

    Battikh, Mohamed H; Joobeur, Sameh; Ben Sayeh, Mohamed M; Rouetbi, Naceur; Maatallah, Anis; Daami, Monia; el Kamel, Ali

    2004-02-01

    Obstructive sleep apnoea (OSA) is a relatively common disorder, in developed country with prevalence estimated to lie between 2 and 4% in adult population. The diagnosis of this syndrome is made on the basis of characteristic clinical features and the results of nocturnal polysomnography. There is no data concerning the OSA in developing country. It is therefore of interest to determine the clinic and polysomnographic profile of this disease and to landmark factors correlated with severity in our country. This was achieved by studying a set of 63 OSA. The mean of age was 53 + 13 years with sex ratio 1. The means of Epworth sleepiness scale score, BMI and Apnoea/Hypopnoea index (AHI) were respectively 16 + 4, 38.8 + 7 kg/m2 and 51.7 + 28.6. 44% of patients have OSA severe with IAH > 50/h. Arousal index and desaturation index were respectively 36.4 + 21.7 and 49 + 26. Trial of continuous positive airway pressure (CPAP) therapy was proposed first to 40 patients, 17 were able to use CPAP.

  5. Merits of using andalusite-based refractories compared to bauxite-based refractories

    OpenAIRE

    Nyoka, M.; Brazier, D.; Courtney, T.; Parry, R.A.

    2013-01-01

    Historically bauxite-based refractories have been used in applications where andalusite-based refractories could work. Bauxite-based refractories were chosen over andalusite-based refractories mainly because of the availability of low-cost Chinese bauxite and also because many furnaces were designed by international companies that cannot easily access high-quality products. Currently, the availability of low-cost bauxite is under threat as a result of high export duties and tariffs as well as...

  6. Adaptive servo ventilation for central sleep apnoea in heart failure : SERVE-HF on-treatment analysis

    NARCIS (Netherlands)

    Woehrle, Holger; Cowie, Martin R.; Eulenburg, Christine; Suling, Anna; Angermann, Christiane; d'Ortho, Marie-Pia; Erdmann, Erland; Levy, Patrick; Simonds, Anita K.; Somers, Virend K.; Zannad, Faiez; Teschler, Helmut; Wegscheider, Karl

    2017-01-01

    This on-treatment analysis was conducted to facilitate understanding of mechanisms underlying the increased risk of all-cause and cardiovascular mortality in heart failure patients with reduced ejection fraction and predominant central sleep apnoea randomised to adaptive servo ventilation versus the

  7. Chemotherapy refractory testicular germ cell tumor is associated with a variant in Armadillo Repeat gene deleted in Velco-Cardio-Facial syndrome (ARVCF

    Directory of Open Access Journals (Sweden)

    Chunkit eFung

    2012-12-01

    Full Text Available Introduction: There is evidence that inherited genetic variation affects both testicular germ cell tumor (TGCT treatment outcome and risks of late-complications arising from cisplatin-based chemotherapy. Using a candidate gene approach, we examined associations of three genes involved in the cisplatin metabolism pathway, GSTP1, COMT, and TPMT, with TGCT outcome and cisplatin-induced neurotoxicity. Material and Methods: Our study population includes a subset of patients (n=137 from a genome-wide association study at the University of Pennsylvania that evaluates inherited genetic susceptibility to TGCT. All patients in our study had at least one course of cisplatin-based chemotherapy with at least one year of follow up. A total of 90 markers in GSTP1, COMT and TPMT and their adjacent genomic regions (± 20 kb were analyzed for associations with refractory TGCT after first course of chemotherapy, progression-free survival (PFS, overall survival (OS, peripheral neuropathy, and ototoxicity. Results: After adjustment for multiple comparisons, one SNP, rs2073743, in the flanking region (± 20 kb of COMT was associated with refractory TGCT after initial chemotherapy. This SNP lies within the intron region of the Armadillo Repeat gene deleted in Velco-Cardio-Facial syndrome (ARVCF. The G allele of rs2073743 predisposed patients to refractory disease with a relative risk of 2.6 (95% CI 1.1, 6.3; P=0.03. Assuming recessive inheritance, patients with the GG genotype had 22.7 times higher risk (95% CI 3.3, 155.8; P=0.04 of developing refractory disease when compared to those with the GC or CC genotypes. We found no association of our candidate genes with peripheral neuropathy, ototoxicity, PFS and OS. Discussion: This is the first study to suggest that germline genetic variants of ARVCF may affect TGCT outcome. The result of this study is hypothesis generating and should be validated in future studies.

  8. Long-term effects of hypnotherapy in patients with refractory irritable bowel syndrome.

    Science.gov (United States)

    Lindfors, Perjohan; Unge, Peter; Nyhlin, Henry; Ljótsson, Brjánn; Björnsson, Einar S; Abrahamsson, Hasse; Simrén, Magnus

    2012-04-01

    Gut-directed hypnotherapy is considered to be an effective treatment in irritable bowel syndrome (IBS) but few studies report the long-term effects. This retrospective study aims to evaluate the long-term perceived efficacy of gut-directed hypnotherapy given outside highly specialized hypnotherapy centers. 208 patients, who all had received gut-directed hypnotherapy, were retrospectively evaluated. The Subjective Assessment Questionnaire (SAQ) was used to measure changes in IBS symptoms, and patients were classified as responders and non-responders. Patients were also asked to report changes in health-care seeking, use of drugs for IBS symptoms, use of alternative non-pharmacological treatments, and if they still actively used hypnotherapy. Immediately after hypnotherapy, 103 of 208 patients (49%) were responders and 75 of these (73%) had improved further at the follow-up 2-7 years after hypnotherapy (mean 4 years). A majority of the responders still used hypnotherapy on a regular basis at follow-up (73%), and the responders reported a greater reduction in health-care seeking than non-responders. A total of 87% of all patients reported that they considered gut-directed hypnotherapy to be worthwhile, and this differed between responders and non-responders (100% vs. 74%; p hypnotherapy in refractory IBS is an effective treatment option with long-lasting effects, also when given outside highly specialized hypnotherapy centers. Apart from the clinical benefits, the reduction in health-care utilization has the potential to reduce the health-care costs.

  9. Effectiveness of transnasal humidified rapid-insufflation ventilatory exchange versus traditional preoxygenation followed by apnoeic oxygenation in delaying desaturation during apnoea: A preliminary study

    Directory of Open Access Journals (Sweden)

    Sunil Rajan

    2018-01-01

    Full Text Available Background and Aims: Transnasal humidified rapid-insufflation ventilatory exchange (THRIVE during apnoea has shown to delay desaturation. The primary objective was to compare time to desaturate to 200 mmHg even at 12 min of apnoea with a significant rise in PaCO2along with fall in pH after 6 min. Conclusion: During apnoeic periods time to desaturate to <90% was significantly prolonged with use of THRIVE.

  10. The neuropharmacology of upper airway motor control in the awake and asleep states: implications for obstructive sleep apnoea

    Directory of Open Access Journals (Sweden)

    Horner Richard L

    2001-08-01

    Full Text Available Abstract Obstructive sleep apnoea is a common and serious breathing problem that is caused by effects of sleep on pharyngeal muscle tone in individuals with narrow upper airways. There has been increasing focus on delineating the brain mechanisms that modulate pharyngeal muscle activity in the awake and asleep states in order to understand the pathogenesis of obstructive apnoeas and to develop novel neurochemical treatments. Although initial clinical studies have met with only limited success, it is proposed that more rational and realistic approaches may be devised for neurochemical modulation of pharyngeal muscle tone as the relevant neurotransmitters and receptors that are involved in sleep-dependent modulation are identified following basic experiments.

  11. Impact and Timing of Bilateral Adrenalectomy for Refractory ACTH-Dependent Cushing’s Syndrome

    Science.gov (United States)

    Morris, Lilah F.; Harris, Rachel S.; Milton, Denái R.; Waguespack, Steven G.; Habra, Mouhammed A.; Jimenez, Camilo; Vassilopoulou-Sellin, Rena; Lee, Jeffrey E.; Perrier, Nancy D.; Grubbs, Elizabeth G.

    2014-01-01

    Introduction In patients with refractory ACTH-dependent Cushing’s syndrome (CS), we evaluated steroidogenesis inhibition (SI) and bilateral adrenalectomy (BA) to predict which patients might benefit most from each treatment modality. Methods Clinical data from patients treated 1970-2012 were retrospectively reviewed by treatment group (SI or SI+BA). Validated severity scales were used to calculate metabolic (M) score (hypokalemia, hyperglycemia, hypertension, proximal muscle weakness) and adverse events (AE) score (thrombosis, fracture, infection). Results 65 patients (16 pituitary, 49 ectopic) were treated with SI+BA (n=21,32%) or SI alone (n=44,68%). Presenting M scores and source of ACTH excess (ectopic vs. pituitary) were similar. Both groups improved metabolically after treatment. 39% of AEs in the SI+BA group occurred within 12 months of presentation. 24(55%) SI patients died (median survival 24.0 months); steroid excess contributed to 71%. Six SI+BA patients died (29%), including all 3 patients with recurrent CS after BA. Minor perioperative complications occurred in 7 patients (33%). Conclusions Post-treatment M and AE scores improved for all patients and 70% of AEs occurred in SI+BA patients within 12 months of presentation, emphasizing the importance of early surgical intervention. These data argue for the safety and efficacy of early BA in selected patients with uncontrollable CS. PMID:24383115

  12. [A 14-day-old boy with jaundice and apnoea].

    Science.gov (United States)

    Smerud, Ole-Jørgen Olsøy; Solevåg, Anne Lee; Hansen, Thor Willy Ruud; Grønn, Morten

    2015-12-15

    We describe an infant who was readmitted from home at 14 days of age with jaundice and a history of apnoea and episodes of retrocollis/opisthotonos. He had been only mildly jaundiced on discharge from the maternity clinic at 2 days of age. The total serum bilirubin (TSB) on admission was 542 µmol/L, and the infant was treated intensively with triple phototherapy and exchange transfusion. In contrast to what is recommended in Norwegian national guidelines for management of neonatal jaundice, the parents had apparently neither received oral nor written information about jaundice and its follow-up at the time of discharge from maternity. They therefore contacted their child healthcare centre when they had questions about jaundice, though the national guidelines specifically state that follow-up for neonatal jaundice during the first 2 weeks of life is the responsibility of the birth hospital. Inappropriate advice resulted in delayed referral, and the child has been diagnosed with chronic kernicterus, probably the first such case in Norway since national guidelines were formalised in 2006. Genetic work-up disclosed compound heterozygosity for Crigler-Najjar syndrome type I, to the best of our knowledge the first instance of this disorder ever to have been diagnosed in Norway. The incidence of kernicterus is Norway is much lower than in other industrialised countries. This is most likely due to national guidelines for management of neonatal jaundice, which place the responsibility for management and follow-up of jaundice with the birth hospital during the crucial first 2 weeks of life. This case report reminds us that tragedies may occur when guidelines are disregarded.

  13. Changes in sexual function of women with refractory interstitial cystitis/bladder pain syndrome after intravesical therapy with a hyaluronic acid solution.

    Science.gov (United States)

    Hung, Man-Jung; Su, Tsung-Hsien; Lin, Yi-Hao; Huang, Wen-Chu; Lin, Tzu-Yin; Hsu, Chun-Shuo; Chuang, Fei-Chi; Tsai, Ching-Pei; Shen, Pao-Sheng; Chen, Gin-Den

    2014-09-01

    Intravesical instillation with a hyaluronic acid (HA) solution is an effective treatment for interstitial cystitis/bladder pain syndrome (IC/BPS), but its impact on sexual functioning of patients is not known. The aim of this study was to evaluate the changes in sexual function of women with refractory IC/BPS who underwent a second-line intravesical HA therapy. A total of 103 women diagnosed with refractory IC/BPS were enrolled in this prospective, multicenter study. Sexual function was evaluated using the short form of the Pelvic Organ Prolapse/Urinary Incontinence Sexual Function Questionnaire (PISQ-9). Bladder-related symptoms and bother were assessed by the Interstitial Cystitis Symptom Index (ICSI) and Interstitial Cystitis Problem Index (ICPI), and a pain visual analog scale (VAS), respectively. Data were analyzed with univariate methods or multivariate logistic regression analysis accordingly. Changes in PISQ-9, ICSI, ICPI, and pain VAS scores after treatment were assessed. Mean age and duration of symptoms was 43.6 ± 11.8 and 5.1 ± 5.0 years, respectively. ICSI, ICPI, and pain VAS scores were significantly (P sexually active women evaluated, PISQ-9 total scores improved significantly (P sexual intercourse, and "intensity" (P sexual orgasms. After a logistic regression analysis, we found that a baseline PISQ-9 score was negatively correlated with the duration of IC/BPS symptoms (P = 0.022). Meanwhile, the changes in PISQ-9 scores were positively correlated with the reduction in ICSI scores after treatment (P = 0.045). Intravesical HA is an effective treatment for refractory IC/BPS. A longer duration of IC/BPS symptoms may be a predictor of poor sexual function. However, intravesical HA may improve sexual function along with the reduction of IC/BPS symptoms. © 2014 International Society for Sexual Medicine.

  14. Quantifying the ventilatory control contribution to sleep apnoea using polysomnography.

    Science.gov (United States)

    Terrill, Philip I; Edwards, Bradley A; Nemati, Shamim; Butler, James P; Owens, Robert L; Eckert, Danny J; White, David P; Malhotra, Atul; Wellman, Andrew; Sands, Scott A

    2015-02-01

    Elevated loop gain, consequent to hypersensitive ventilatory control, is a primary nonanatomical cause of obstructive sleep apnoea (OSA) but it is not possible to quantify this in the clinic. Here we provide a novel method to estimate loop gain in OSA patients using routine clinical polysomnography alone. We use the concept that spontaneous ventilatory fluctuations due to apnoeas/hypopnoeas (disturbance) result in opposing changes in ventilatory drive (response) as determined by loop gain (response/disturbance). Fitting a simple ventilatory control model (including chemical and arousal contributions to ventilatory drive) to the ventilatory pattern of OSA reveals the underlying loop gain. Following mathematical-model validation, we critically tested our method in patients with OSA by comparison with a standard (continuous positive airway pressure (CPAP) drop method), and by assessing its ability to detect the known reduction in loop gain with oxygen and acetazolamide. Our method quantified loop gain from baseline polysomnography (correlation versus CPAP-estimated loop gain: n=28; r=0.63, p<0.001), detected the known reduction in loop gain with oxygen (n=11; mean±sem change in loop gain (ΔLG) -0.23±0.08, p=0.02) and acetazolamide (n=11; ΔLG -0.20±0.06, p=0.005), and predicted the OSA response to loop gain-lowering therapy. We validated a means to quantify the ventilatory control contribution to OSA pathogenesis using clinical polysomnography, enabling identification of likely responders to therapies targeting ventilatory control. Copyright ©ERS 2015.

  15. [Clinical implications of polycystic ovary syndrome].

    Science.gov (United States)

    Dravecká, Ingrid

    Polycystic ovary syndrome (PCOS) is a heterogeneous and complex endocrine disease which among the female population belongs to the most widespread endocrinopathies and it is the most frequent cause of hyperthyroidism, anticoagulation and infertility. Insulin resistance is one of the important diabetology factors impacting hyperglycaemia in a majority of women with PCOS (60-80 %). Clinical expressions of PCOS include reproduction disorders, metabolic characteristics and psychological implications. Reproduction disorders include hyperthyroidism, menstruation cycle disorders, infertility and pregnancy complications as well as early abortions, gestational diabetes and pregnancy induced hypertension. Long-term metabolic risks of PCOS include type 2 diabetes mellitus, dyslipidemia, arterial hypertension and endothelial dysfunction. The available data confirms higher incidence of cardiovascular diseases in women with PCOS. In particular among obese women PCOS is more frequently associated with non-alcoholic hepatic steatosis, sleep apnoea syndrome and endometrial cancer. The literature includes some controversial data about the relationship between PCOS and autoimmunity. Women with PCOS are more prone to suffer from insufficient confidence with higher incidence of anxiety, depression, bipolar disorder and eating disorders. autoimmunity - diabetes mellitus - pregnancy - insulin resistance - metabolic syndrome - menstrual disorders - polycystic ovary syndrome.

  16. Intermittent hypoxia, cardiovascular disease and obstructive sleep apnoea.

    Science.gov (United States)

    Turnbull, Chris D

    2018-01-01

    Obstructive sleep apnoea (OSA) is a common disorder and is associated with cardiovascular disease. Continuous positive airway pressure (CPAP), whilst reducing blood pressure, has not been shown to reduce cardiovascular events when used as a treatment solely for this purpose in patients with previous cardiovascular disease. Developing a better understanding of the mechanisms underlying cardiovascular disease in OSA is important to develop new treatments. Potential causative mechanisms for cardiovascular disease in OSA include arousal induced sympathetic activation, large intrathoracic pressure swings leading to shear stress on the heart and great vessels, and intermittent hypoxia (IH). This review discusses the role of IH, as a major physiological consequence of OSA, in the development of cardiovascular disease.

  17. Flextube reflectometry and pressure recordings for level diagnosis in obstructive sleep apnoea

    DEFF Research Database (Denmark)

    Faber, C E; Grymer, L; Hilberg, O

    2002-01-01

    The objective of this study was to compare sound reflections in a flexible tube (flextube reflectometry) with pressure-catheter recordings (ApneaGraph) for identifying the predominant obstructive level of the upper airway during sleep. Seventeen males with suspected obstructive sleep apnoea...... results were found in flextube reflectometry studies and pressure-recordings performed on different nights regarding the level distribution of obstructions during sleep. Possible explanations of this discrepancy are discussed....

  18. Refractory Materials for Flame Deflector Protection System Corrosion Control: Refractory Ceramics Literature Survey

    Science.gov (United States)

    Calle, Luz Marina; Hintze, Paul E.; Parlier, Christopher R.; Curran, Jerome P.; Kolody, Mark; Perusich, Stephen; Whitten, Mary C.; Trejo, David; Zidek, Jason; Sampson, Jeffrey W.; hide

    2009-01-01

    Ceramics can be defmed as a material consisting of hard brittle properties produced from inorganic and nonmetallic minerals made by firing at high temperatures. These materials are compounds between metallic and nonmetallic elements and are either totally ionic, or predominately ionic but having some covalent character. This definition allows for a large range of materials, not all applicable to refractory applications. As this report is focused on potential ceramic materials for high temperature, aggressive exposure applications, the ceramics reviewed as part of this report will focus on refractory ceramics specifically designed and used for these applications. Ceramic materials consist of a wide variety of products. Callister (2000) 1 characterized ceramic materials into six classifications: glasses, clay products, refractories, cements, abrasives, and advanced ceramics. Figure 1 shows this classification system. This review will focus mainly on refractory ceramics and cements as in general, the other classifications are neither applicable nor economical for use in large structures such as the flame trench. Although much work has been done in advanced ceramics over the past decade or so, these materials are likely cost prohibitive and would have to be fabricated off-site, transported to the NASA facilities, and installed, which make these even less feasible. Although the authors reviewed the literature on advanced ceramic refractories 2 center dot 3 center dot 4 center dot 5 center dot 6 center dot 7 center dot 8 center dot 9 center dot 10 center dot 11 center dot 12 after the review it was concluded that these materials should not be ' the focus of this report. A review is in progress on materials and systems for prefabricated refractory ceramic panels, but this review is focusing more on typical refractory materials for prefabricated systems, which could make the system more economically feasible. Refractory ceramics are used for a wide variety of applications

  19. Novel method for detection of Sleep Apnoea using respiration signals

    DEFF Research Database (Denmark)

    Nielsen, Kristine Carmes; Kempfner, Lykke; Sørensen, Helge Bjarup Dissing

    2014-01-01

    desaturations > 3%, extracted from the thorax and abdomen respiration effort belts, and the oxyhemoglobin saturation (SaO2), fed to an Elastic Net classifier and validated according to American Academy of Sleep Medicine (AASM) using the patients' AHI value. The method was applied to 109 patient recordings......Polysomnography (PSG) studies are considered the “gold standard” for the diagnosis of Sleep Apnoea (SA). Identifying cessations of breathing from long-lasting PSG recordings manually is a labour-intensive and time-consuming task for sleep specialist, associated with inter-scorer variability...

  20. Refractory vasculitis

    NARCIS (Netherlands)

    Rutgers, Bram; Kallenberg, Cees G. M.

    Refractory vasculitis occurs in 4-5% of patients with anti-neutrophil cytoplasmic antibody associated vasculitis (AAV). Differences between therapies used for refractory disease are mostly reflected in the percentages of complete and partial remissions, but also in the number of serious side

  1. Effects of CPAP therapy on visceral fat thickness, carotid intima-media thickness and adipokines in patients with obstructive sleep apnoea.

    Science.gov (United States)

    Ng, Susanna S S; Liu, Eric K H; Ma, Ronald C W; Chan, Tat-On; To, Kin-Wang; Chan, Ken K P; Ngai, Jenny; Yip, Wing-Ho; Ko, Fanny W S; Wong, Chun-Kwok; Hui, David S C

    2017-05-01

    Obstructive sleep apnoea (OSA) is associated with an increased prevalence of metabolic syndrome. This study explores the effects of continuous positive airway pressure (CPAP) for patients with OSA on visceral and mesenteric fat thickness, carotid intima-media thickness (IMT) and adipokines. A randomized controlled study was conducted at a teaching hospital on 90 patients newly diagnosed with OSA to receive either therapeutic CPAP or subtherapeutic CPAP for 3 months. Visceral fat thickness and carotid IMT were measured with B-mode ultrasound; adipokine levels were assessed at baseline and 3 months. Altogether, 45 patients received therapeutic CPAP and 45 received subtherapeutic CPAP without significant differences in age 50.3 (10.1) versus 48.7 (9.0) years, BMI 28.2 (3.9) versus 28.2 (4.5) kg/m 2 , Epworth Sleepiness Scale (ESS) 12.4 (5.9) versus 11.3 (4.7), apnoea-hypopnoea index (AHI) 30.6 (21.4) versus 35.2 (25.5) /h, minimum SaO 2 79.6 (10.8) versus 76.7 (12.4) % and existing co-morbidities. CPAP usage was therapeutic 4.2 (2.1) versus subtherapeutic 4.1 (2.0) h/night over 3 months. Adiponectin and irisin levels changed significantly following therapeutic CPAP for 3 months versus subtherapeutic CPAP (-1.6 vs 7.3, P = 0.042; 0.1 vs -0.1, P = 0.028 respectively) while only serum level of monocyte chemotactic protein 1 (MCP-1) at baseline was positively correlated with AHI (r = 0.278). No significant changes were observed in other adipokines, visceral fat thickness and IMT. Short-term therapeutic CPAP versus subtherapeutic CPAP does not significantly reduce visceral fat thickness and IMT, although it reduces adiponectin and increases irisin. © 2016 Asian Pacific Society of Respirology.

  2. Interictal brain SPECT in patients with medically refractory temporal lobe epilepsy

    International Nuclear Information System (INIS)

    Andraus, Maria Emilia Cosenza

    2000-06-01

    The brain single photon emission computed tomography (SPECT) is s functional neuroimaging method that can detect localized changes in cerebral blood flow. The temporal lobe epilepsy (TLE) is the most common epileptic syndrome in adults, and more than 50% are medically refractory. The SPECT can contribute to investigation of epileptogenic focus and is one of the methods of pre-surgical evaluation of these patients. (author)

  3. Refractory obstetrical antiphospholipid syndrome: Features, treatment and outcome in a European multicenter retrospective study.

    Science.gov (United States)

    Mekinian, Arsène; Alijotas-Reig, Jaume; Carrat, Fabrice; Costedoat-Chalumeau, Nathalie; Ruffatti, Amelia; Lazzaroni, Maria Grazia; Tabacco, Sara; Maina, Aldo; Masseau, Agathe; Morel, Nathalie; Esteve-Valverde, Enrique Esteve; Ferrer-Oliveras, Raquel; Andreoli, Laura; De Carolis, Sara; Josselin-Mahr, Laurence; Abisror, Noémie; Nicaise-Roland, Pascale; Tincani, Angela; Fain, Olivier

    2017-07-01

    To describe the consecutive pregnancy outcome and treatment in refractory obstetrical antiphospholipid syndrome (APS). Retrospective multicenter open-labelled study from December 2015 to June 2016. We analyzed the outcome of pregnancies in patients with obstetrical APS (Sydney criteria) and previous adverse obstetrical event despite low-dose aspirin and low-molecular weight heparin LMWH (LMWH) conventional treatment who experienced at least one subsequent pregnancy. Forty nine patients with median age 27years (23-32) were included from 8 European centers. Obstetrical APS was present in 71%, while 26% had obstetrical and thrombotic APS. Lupus anticoagulant was present in 76% and triple antiphospholipid antibody (APL) positivity in 45% of patients. Pregnancy loss was noted in 71% with a median age of gestation of 11 (8-21) weeks. The presence of APS non-criteria features (35% vs 17% in pregnancies without adverse obstetrical event; p=0.09), previous intrauterine death (65% vs 38%; p=0.06), of LA (90% vs 65%; p=0.05) were more frequent in pregnancies with adverse pregnancy outcome, whereas isolated recurrent miscarriage profile was more frequent in pregnancies without any adverse pregnancy outcome (15% vs 41%; p=0.04). In univariate analysis considering all pregnancies (index and subsequent ones), an history of previous intrauterine death was associated with pregnancy loss (odds-ratio 2.51 (95% CI 1.274.96); p=0.008), whereas previous history of prematurity related to APS (odds-ratio 0.13 95%CI 0.04 0.41, P=0.006), steroids use during the pregnancy (odds-ratio 0.30 95% CI 0.11-0.82, p=0.019) and anticardiolipids isolated profile (odds-ratio 0.51 95% CI 0.26-1.03, p=0.0588) were associated with favorable outcome. In multivariate analysis, only previous history of prematurity, steroids use and anticardiolipids isolated profiles were associated with live-birth pregnancy. The main features of refractory obstetrical APS were the high rates of LA and triple APL positivity

  4. Real Time Apnoea Monitoring of Children Using the Microsoft Kinect Sensor: A Pilot Study

    Directory of Open Access Journals (Sweden)

    Ali Al-Naji

    2017-02-01

    Full Text Available The objective of this study was to design a non-invasive system for the observation of respiratory rates and detection of apnoea using analysis of real time image sequences captured in any given sleep position and under any light conditions (even in dark environments. A Microsoft Kinect sensor was used to visualize the variations in the thorax and abdomen from the respiratory rhythm. These variations were magnified, analyzed and detected at a distance of 2.5 m from the subject. A modified motion magnification system and frame subtraction technique were used to identify breathing movements by detecting rapid motion areas in the magnified frame sequences. The experimental results on a set of video data from five subjects (3 h for each subject showed that our monitoring system can accurately measure respiratory rate and therefore detect apnoea in infants and young children. The proposed system is feasible, accurate, safe and low computational complexity, making it an efficient alternative for non-contact home sleep monitoring systems and advancing health care applications.

  5. Diagnostic approaches to respiratory sleep disorders

    OpenAIRE

    Riha, Renata L.

    2015-01-01

    Sleep disordered breathing (SDB) comprises a number of breathing disturbances occurring during sleep including snoring, the obstructive sleep apnoea/hypopnea syndrome (OSAHS), central sleep apnoea (CSA) and hypoventilation syndromes. This review focuses on sleep disordered breathing and diagnostic approaches in adults, in particular clinical assessment and overnight assessment during sleep. Although diagnostic approaches to respiratory sleep disorders are reasonably straightforward, they do r...

  6. Pharmacotherapy for Refractory and Super-Refractory Status Epilepticus in Adults.

    Science.gov (United States)

    Holtkamp, Martin

    2018-03-01

    Patients with prolonged seizures that do not respond to intravenous benzodiazepines and a second-line anticonvulsant suffer from refractory status epilepticus and those with seizures that do not respond to continuous intravenous anesthetic anticonvulsants suffer from super-refractory status epilepticus. Both conditions are associated with significant morbidity and mortality. A strict pharmacological treatment regimen is urgently required, but the level of evidence for the available drugs is very low. Refractory complex focal status epilepticus generally does not require anesthetics, but all intravenous non-anesthetizing anticonvulsants may be used. Most descriptive data are available for levetiracetam, phenytoin and valproate. Refractory generalized convulsive status epilepticus is a life-threatening emergency, and long-term clinical consequences are eminent. Administration of intravenous anesthetics is mandatory, and drugs acting at the inhibitory gamma-aminobutyric acid (GABA) A receptor such as midazolam, propofol and thiopental/pentobarbital are recommended without preference for one of those. One in five patients with anesthetic treatment does not respond and has super-refractory status epilepticus. With sustained seizure activity, excitatory N-methyl-d-aspartate (NMDA) receptors are increasingly expressed post-synaptically. Ketamine is an antagonist at this receptor and may prove efficient in some patients at later stages. Neurosteroids such as allopregnanolone increase sensitivity at GABA A receptors; a Phase 1/2 trial demonstrated safety and tolerability, but randomized controlled data failed to demonstrate efficacy. Adjunct ketogenic diet may contribute to termination of difficult-to-treat status epilepticus. Randomized controlled trials are needed to increase evidence for treatment of refractory and super-refractory status epilepticus, but there are multiple obstacles for realization. Hitherto, prospective multicenter registries for pharmacological

  7. Maximal exercise capacity in patients with obstructive sleep apnoea syndrome: A systematic review and meta-analysis.

    Science.gov (United States)

    Mendelson, Monique; Marillier, Mathieu; Bailly, Sébastien; Flore, Patrice; Borel, Jean-Christian; Vivodtzev, Isabelle; Doutreleau, Stéphane; Tamisier, Renaud; Pépin, Jean-Louis; Verges, Samuel

    2018-04-26

    Maximal aerobic capacity is a strong health predictor and peak oxygen consumption (VO 2peak ) is considered a reflection of total body health. No systematic reviews or meta-analysis' to date have synthesised the existing data regarding VO 2peak in patients with obstructive sleep apnoea (OSA).A systematic review of English and French articles using Pubmed/Medline and Embase included studies assessing VO 2peak of OSA patients in mL·kg -1 ·min -1 compared with controls or in % predicted. Two independent reviewers analysed the studies, extracted the data and assessed the quality of evidence.Mean VO 2peak expressed in mL·kg -1 ·min -1 was significantly lower in patients with OSA when compared with controls (mean difference=-2.7 mL·kg -1 ·min -1 ; p<0.001; n=850). This reduction in VO 2peak was found to be larger in non-obese patients (BMI<30 kg·m -2 ). Mean VO 2peak in % predicted was 90.7±21.0% in OSA patients (n=643).OSA patients present reduced maximal aerobic capacity, which can be associated with increased cardiovascular risks and reduced survival in certain patient subgroups. Maximal exercise testing can be useful to characterise functional limitation and to evaluate health status in OSA patients. Registration # CRD42017057319. Copyright ©ERS 2018.

  8. Síndroma de apneia obstrutiva do sono como causa de acidentes de viação Obstructive sleep apnoea syndrome as a cause of road traffic accidents

    Directory of Open Access Journals (Sweden)

    M Aguiar

    2009-05-01

    Full Text Available Vários estudos demonstram que os doentes com síndroma de apneia obstrutiva do sono (SAOS têm um risco aumentado de acidentes de viação. O objectivo do presente trabalho consistiu em analisar, nestes doentes, se há diferenças nos que referem acidentes e/ /ou quase acidentes e aqueles que o não fazem. Material e métodos: Estudaram-se prospectivamente 163 doentes com SAOS (índice apneia/hipopneia (IAH>10/h diagnosticados por polissonografia nocturna (PSG, todos condutores de veículos, 18,4% do quais profissionais. Na altura da entrevista clínica foi inquirido se tinham tido, nos três anos antes acidentes e/ou quase acidentes devido a hipersonia diurna (Grupo II = 74 ou não (Grupo I = 89. Estes dois grupos foram comparados quanto a: idade, índice de massa corporal (IMC, escala de sonolência de Epworth (ESE, PaO2 e PaCO2 diurnas, avaliação da qualidade de vida pelo inquérito Functional Outcomes of Sleep Questionnaire (FOSQ teste e dados da polissonografia - tempo total de sono (TTS, eficiência do sono, estádios do sono, índice de microdespertares (IMD, índice de apneia/hipopneia (IAH, SaO2 mínima e média, % tempo SaO2Several studies have demonstrated that obstructive sleep apnoea syndrome (OSAS patients have a higher rate of road traffic accidents. Our study aimed to analyse any differences in OSAS patients between those who reported having had road traffic accidents and/or near misses and those who did not. Methods: We studied 163 patients with OSAS (apnoea-hypopnoea index (AHI>10/h diagnosed using nocturnal polysomnography (NPSG, all drivers, 18.4% of whom drove for a living. Patients were asked at their first clinical interview to self-report road traffic accidents and/or near misses over the past 3 years which had been caused by abnormal daytime drowsiness. This allowed patients to be divided into two groups, those who had had road traffic accidents and/or near misses and those who had not. Both were compared as to age

  9. Three-dimensional photography for the evaluation of facial profiles in obstructive sleep apnoea.

    Science.gov (United States)

    Lin, Shih-Wei; Sutherland, Kate; Liao, Yu-Fang; Cistulli, Peter A; Chuang, Li-Pang; Chou, Yu-Ting; Chang, Chih-Hao; Lee, Chung-Shu; Li, Li-Fu; Chen, Ning-Hung

    2018-06-01

    Craniofacial structure is an important determinant of obstructive sleep apnoea (OSA) syndrome risk. Three-dimensional stereo-photogrammetry (3dMD) is a novel technique which allows quantification of the craniofacial profile. This study compares the facial images of OSA patients captured by 3dMD to three-dimensional computed tomography (3-D CT) and two-dimensional (2-D) digital photogrammetry. Measurements were correlated with indices of OSA severity. Thirty-eight patients diagnosed with OSA were included, and digital photogrammetry, 3dMD and 3-D CT were performed. Distances, areas, angles and volumes from the images captured by three methods were analysed. Almost all measurements captured by 3dMD showed strong agreement with 3-D CT measurements. Results from 2-D digital photogrammetry showed poor agreement with 3-D CT. Mandibular width, neck perimeter size and maxillary volume measurements correlated well with the severity of OSA using all three imaging methods. Mandibular length, facial width, binocular width, neck width, cranial base triangle area, cranial base area 1 and middle cranial fossa volume correlated well with OSA severity using 3dMD and 3-D CT, but not with 2-D digital photogrammetry. 3dMD provided accurate craniofacial measurements of OSA patients, which were highly concordant with those obtained by CT, while avoiding the radiation associated with CT. © 2018 Asian Pacific Society of Respirology.

  10. Current demand of paediatric otolaryngology input for children with Down's syndrome in a tertiary referral centre.

    Science.gov (United States)

    Khalid-Raja, M; Tzifa, K

    2016-11-01

    This study aimed to evaluate the activity of paediatric otolaryngology services required for children with Down's syndrome in a tertiary referral centre. A review of the paediatric otolaryngology input for children with Down's syndrome was performed; data were obtained from the coding department for a two-year period and compared with other surgical specialties. Between June 2011 and May 2013, 106 otolaryngology procedures were performed on children with Down's syndrome. This compared to 87 cardiac and 81 general paediatrics cases. The most common pathologies in children with Down's syndrome were obstructive sleep apnoea, otitis media, hearing loss and cardiac disease. The most common otolaryngology procedures performed were adenoidectomy, tonsillectomy, grommet insertion and bone-anchored hearing aid implant surgery. ENT manifestations of Down's syndrome are common. Greater provisions need to be made to streamline the otolaryngology services for children and improve transition of care to adult services.

  11. Iodine I 131 Monoclonal Antibody BC8, Fludarabine Phosphate, Cyclophosphamide, Total-Body Irradiation and Donor Bone Marrow Transplant in Treating Patients With Advanced Acute Myeloid Leukemia, Acute Lymphoblastic Leukemia, or High-Risk Myelodysplastic Syndrome

    Science.gov (United States)

    2018-05-14

    Acute Myeloid Leukemia Arising From Previous Myelodysplastic Syndrome; Adult Acute Lymphoblastic Leukemia in Remission; Adult Acute Myeloid Leukemia in Remission; CD45-Positive Neoplastic Cells Present; Chronic Myelomonocytic Leukemia; Previously Treated Myelodysplastic Syndrome; Refractory Anemia With Excess Blasts; Refractory Anemia With Ring Sideroblasts; Refractory Cytopenia With Multilineage Dysplasia; Refractory Cytopenia With Multilineage Dysplasia and Ring Sideroblasts

  12. Autoradiographic study of corrosion of refractories

    International Nuclear Information System (INIS)

    Lisenenkova, S.B.; Kucheryavyi, M.N.; Bursteva, T.A.

    1988-01-01

    A comparative study was made of the character of the interaction between a container-glass melt consisting of sodium calcium silicate and refractories in various furnace sections using an autoradiographic method. Static tests were conducted on specimens of the following refractories: chrome-aluminum-zircon, Bakor 41, corundum, a high alumina refractory, and a refractory based on tin dioxide. The specimens were activated by calcium 45. Autoradiography and photomicrography indicated that an intrinsic feature of all refractories was that calcium from the melt penetrated the refractories along the weak link; for fused-cast refractories, the glass phase; and for sintered refractories, through the binder and cracks

  13. Reduced Inspiratory Muscle Strength in Patients with Type 2 Diabetes Mellitus and Obstructive Sleep Apnoea

    DEFF Research Database (Denmark)

    Rehling, Thomas; Banghoj, Anne Margareta; Kristiansen, Marie Hvelplund

    2017-01-01

    Background: Obstructive sleep apnoea (OSA) is related to type 2 diabetes (T2DM), and it may be associated with reduced inspiratory muscle strength (IMS). The aim of this study was to investigate the IMS in patients with T2DM, with or without OSA. Methods: Patients with T2DM with OSA (n = 33...

  14. Craniofacial morphology, head posture, and nasal respiratory resistance in obstructive sleep apnoea : An inter-ethnic comparison

    NARCIS (Netherlands)

    Wong, M.L.; Sandham, John; Ang, PK; Wong, DC; Tan, WC; Huggare, J

    The aim of this study was to measure craniofacial morphology and nasal respiratory resistance (NRR) in Malay, Indian and Chinese subjects with obstructive sleep apnoea (OSA). The sample consisted of 34 male subjects, 27-52 years of age (Malay n = 11, which included five mild and six moderate-severe

  15. Erythroleukemia shares biological features and outcome with myelodysplastic syndromes with excess blasts: a rationale for its inclusion into future classifications of myelodysplastic syndromes.

    Science.gov (United States)

    Calvo, Xavier; Arenillas, Leonor; Luño, Elisa; Senent, Leonor; Arnan, Montserrat; Ramos, Fernando; Ardanaz, María Teresa; Pedro, Carme; Tormo, Mar; Montoro, Julia; Díez-Campelo, María; Arrizabalaga, Beatriz; Xicoy, Blanca; Bonanad, Santiago; Jerez, Andrés; Nomdedeu, Benet; Ferrer, Ana; Sanz, Guillermo F; Florensa, Lourdes

    2016-12-01

    Erythroleukemia was considered an acute myeloid leukemia in the 2008 World Health Organization (WHO) classification and is defined by the presence of ≥50% bone marrow erythroblasts, having <20% bone marrow blasts from total nucleated cells but ≥20% bone marrow myeloblasts from nonerythroid cells. Erythroleukemia shares clinicopathologic features with myelodysplastic syndromes, especially with erythroid-predominant myelodysplastic syndromes (≥50% bone marrow erythroblasts). The upcoming WHO revision proposes to eliminate the nonerythroid blast cell count rule and to move erythroleukemia patients into the appropriate myelodysplastic syndrome category on the basis of the absolute blast cell count. We conducted a retrospective study of patients with de novo erythroleukemia and compared their clinico-biological features and outcome with those of de novo myelodysplastic syndromes, focusing on erythroid-predominant myelodysplastic syndromes. Median overall survival of 405 erythroid-predominant myelodysplastic syndromes without excess blasts was significantly longer than that observed in 57 erythroid-predominant refractory anemias with excess blasts-1 and in 59 erythroleukemias, but no significant difference was observed between erythroid-predominant refractory anemias with excess blasts-1 and erythroleukemias. In this subset of patients with ≥50% bone marrow erythroblasts and excess blasts, the presence of a high-risk karyotype defined by the International Prognostic Scoring System or by the Revised International Prognostic Scoring System was the main prognostic factor. In the same way, the survival of 459 refractory anemias with excess blasts-2, independently of having ≥20% bone marrow blasts from nonerythroid cells or not, was almost identical to the observed in 59 erythroleukemias. Interestingly, 11 low-blast count erythroleukemias with 5 to <10% bone marrow blasts from total nucleated cells showed similar survival than the rest of erythroleukemias. Our data

  16. Assessment of obstructive sleep apnoea treatment success or failure after maxillomandibular advancement.

    Science.gov (United States)

    de Ruiter, M H T; Apperloo, R C; Milstein, D M J; de Lange, J

    2017-11-01

    Maxillomandibular advancement (MMA) is an alternative therapeutic option that is highly effective for treating obstructive sleep apnoea (OSA). MMA provides a solution for OSA patients that have difficulty accepting lifelong treatments with continuous positive airway pressure or mandibular advancement devices. The goal of this study was to investigate the different characteristics that determine OSA treatment success/failure after MMA. The apnoea-hypopnoea index (AHI) was used to determine the success or failure of OSA treatment after MMA. Sixty-two patients underwent MMA for moderate and severe OSA. A 71% success rate was observed with a mean AHI reduction of 69%. A statistically significant larger neck circumference was measured in patients with failed OSA treatments following MMA (P=0.008), and older patients had failed OSA treatments with MMA: 58 vs. 53 years respectively (P=0.037). Cephalometric analysis revealed no differences between successful and failed OSA treatment outcomes. There was no difference in maxillary and mandibular advancements between success and failed MMA-treated OSA patients. The complications most frequently reported following MMA were sensory disturbances in the inferior alveolar nerve (60%) and malocclusion (24%). The results suggest that age and neck girth may be important factors that could predict susceptibility to OSA treatment failures by MMA. Copyright © 2017 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.

  17. Sibutramine versus continuous positive airway pressure in obese obstructive sleep apnoea patients.

    Science.gov (United States)

    Ferland, A; Poirier, P; Sériès, F

    2009-09-01

    The aim of the present study was to compare the efficacy of 1 yr of sibutramine-induced weight loss versus continuous positive airway pressure (CPAP) treatment on sleep-disordered breathing, cardiac autonomic function and systemic blood pressure in obese patients with obstructive sleep apnoea. Subjects with a body mass index of > or =30 kg.m(-2) without previous treatment for obstructive sleep apnoea underwent either sibutramine (n = 22) or CPAP (n = 18) treatment for 1 yr. Sibutramine induced a 5.4+/-1.4 kg decrease in body weight compared to the CPAP group, in which no changes in anthropometric variables were observed. The CPAP treatment improved all sleep and respiratory variables, whereas sibutramine-induced weight loss improved only nocturnal arterial oxygen saturation profile. Only CPAP treatment improved night-time systolic and diastolic blood pressure and 24-h and daytime ambulatory diastolic blood pressure. Sibutramine-induced weight loss had no impact on indices of heart rate variability, whereas CPAP treatment increased daytime time domain indices. CPAP treatment for 1 yr had beneficial impacts on nocturnal breathing disturbances, and improved nocturnal oxygenation, night-time systolic and diastolic blood pressure, and daytime cardiac parasympathetic modulation. Sibutramine did not improve sleep-disordered breathing, systemic blood pressure or heart rate variability. There were no adverse effects, such as increment in blood pressure or arrhythmias, associated with this treatment regimen.

  18. Office management of obstructive sleep apnoea: appliances.

    Science.gov (United States)

    Johnson, Matthew D

    2015-08-01

    Oral appliances are becoming increasingly common in the treatment of obstructive sleep apnoea (OSA). They work by advancing the mandible and opening the pharynx. There are several types of devices available for use. Many patients intolerant to continuous positive airway pressure (CPAP) are using oral appliances for OSA. Oral appliances have been shown to improve polysomnography, quality of life and health measures associated with OSA. There is current work to better identify patients who are ideal candidates. Development of titratable devices and monitoring are optimizing usage. They have been compared with CPAP, with both showing improvement in OSA; however, CPAP remains superior overall. Oral appliances are becoming first-line therapy for mild and moderate OSA. They provide a meaningful alternative in severe OSA for patients unable to use CPAP. Device titration and usage monitoring are beginning to hone oral appliances as a therapeutic option.

  19. Single-Session CT-Guided Percutaneous Microwave Ablation of Bilateral Adrenal Gland Hyperplasia Due to Ectopic ACTH Syndrome

    International Nuclear Information System (INIS)

    Sarma, Asha; Shyn, Paul B.; Vivian, Mark A.; Ng, Ju-Mei; Tuncali, Kemal; Lorch, Jorchen H.; Zaheer, Sarah N.; Gordon, Michael S.; Silverman, Stuart G.

    2015-01-01

    Bilateral adrenalectomy is currently the only available treatment for adrenocorticotropic hormone (ACTH)-dependent Cushing’s syndrome (ectopic ACTH syndrome) that is refractory to pharmacologic therapy. We describe two patients with refractory ectopic ACTH syndrome who were treated with CT-guided percutaneous microwave ablation of both hyperplastic adrenal glands in a single session: One was not a surgical candidate, and the other had undergone unsuccessful surgery. Following the procedure, both patients achieved substantial decreases in serum cortisol, symptomatic improvement, and decreased anti-hypertensive medication requirements

  20. Single-Session CT-Guided Percutaneous Microwave Ablation of Bilateral Adrenal Gland Hyperplasia Due to Ectopic ACTH Syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Sarma, Asha, E-mail: ashasarma@gmail.com; Shyn, Paul B., E-mail: pshyn@partners.org [Brigham and Women’s Hospital, Department of Radiology (United States); Vivian, Mark A. [University of Manitoba, Department of Radiology (Canada); Ng, Ju-Mei [Brigham and Women’s Hospital, Department of Anesthesiology (United States); Tuncali, Kemal [Brigham and Women’s Hospital, Department of Radiology (United States); Lorch, Jorchen H. [Dana Farber Cancer Institute, Department of Medicine (United States); Zaheer, Sarah N.; Gordon, Michael S. [Brigham and Women’s Hospital, Department of Endocrinology (United States); Silverman, Stuart G. [Brigham and Women’s Hospital, Department of Radiology (United States)

    2015-10-15

    Bilateral adrenalectomy is currently the only available treatment for adrenocorticotropic hormone (ACTH)-dependent Cushing’s syndrome (ectopic ACTH syndrome) that is refractory to pharmacologic therapy. We describe two patients with refractory ectopic ACTH syndrome who were treated with CT-guided percutaneous microwave ablation of both hyperplastic adrenal glands in a single session: One was not a surgical candidate, and the other had undergone unsuccessful surgery. Following the procedure, both patients achieved substantial decreases in serum cortisol, symptomatic improvement, and decreased anti-hypertensive medication requirements.

  1. Behavioural hyperventilation as a novel clinical condition associated with central sleep apnoea: A report of three cases

    NARCIS (Netherlands)

    Pevernagie, D.; Mariman, A.; Vandenbussche, N.; Tobback, E.; Overeem, S.; Delesie, L.; Janssen, H.; Vogelaers, D.

    2012-01-01

    Central sleep apnoea (CSA) is a disorder characterised by repetitive episodes of decreased ventilation due to complete or partial reduction in the central neural outflow to the respiratory muscles. Hyperventilation plays a prime role in the pathogenesis of CSA. Chronic heart failure and dwelling at

  2. Long-term screening for sleep apnoea in paced patients: preliminary assessment of a novel patient management flowchart by using automatic pacemaker indexes and sleep lab polygraphy.

    Science.gov (United States)

    Aimé, Ezio; Rovida, Marina; Contardi, Danilo; Ricci, Cristian; Gaeta, Maddalena; Innocenti, Ester; Cabral Tantchou-Tchoumi, Jacques

    2014-10-01

    The primary aim of this pilot study was to prospectively assess a flowchart to screen and diagnose paced patients (pts) affected by sleep apnoeas, by crosschecking indexes derived from pacemakers (minute ventilation sensor on-board) with Sleep-Lab Polygraphy (PG) outcomes. Secondarily, "smoothed" long-term pacemaker indexes (all the information between two consecutive follow-up visits) have been retrospectively compared vs. standard short-term pacemaker indexes (last 24h) at each follow-up (FU) visit, to test their correlation and diagnostic concordance. Data from long-term FU of 61 paced pts were collected. At each visit, the standard short-term apnoea+hypopnoea (PM_AHI) index was retrieved from the pacemaker memory. Patients showing PM_AHI ≥ 30 at least once during FU were proposed to undergo a PG for diagnostic confirmation. Smoothed pacemaker (PM_SAHI) indexes were calculated by averaging the overall number of apnoeas/hypopnoeas over the period between two FU visits, and retrospectively compared with standard PM_AHI. Data were available from 609 consecutive visits (overall 4.64 ± 1.78 years FU). PM_AHI indexes were positive during FU in 40/61 pts (65.6%); 26/40 pts (65%) accepted to undergo a PG recording; Sleep-Lab confirmed positivity in 22/26 pts (84.6% positive predictive value for PM_AHI). A strong correlation (r=0.73) and a high level of concordance were found between smoothed and standard indexes (multivariate analysis, Cohen's-k and Z-score tests). Pacemaker-derived indexes may help in screening paced pts potentially affected by sleep apnoeas. Long-term "smoothed" apnoea indexes could improve the accuracy of pacemaker screening capability, even though this hypothesis must be prospectively confirmed by larger studies. Copyright © 2014 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier B.V. All rights reserved.

  3. Joubert syndrome: large clinical variability and a unique neuroimaging aspect

    International Nuclear Information System (INIS)

    Leao, Emilia Katiane Embirucu; Lima, Marcilia Martyn; Kok, Fernando; Parizotto, Juliana; Maia Junior, Otacilio de Oliveira

    2010-01-01

    Joubert syndrome (JS) is an autosomal recessive inherited disorder characterized by hypotonia, cerebellar vermis hypoplasia, ocular abnormalities (e.g. pigmentary retinopathy, oculomotor apraxia and nystagmus), renal cysts and hepatic fibrosis. Respiratory abnormalities, as apnoea and hyperpnoea, may be present, as well as mental retardation. At least seven JS loci have been determined and five genes identified. Herein, we report five children, belonging to independent families, with JS: they shared the same typical MRI abnormality, known as molar tooth sign, but had an otherwise quite variable phenotype, regarding mostly their cognitive performance, visual abilities and extra-neurological compromise. (author)

  4. Joubert syndrome: large clinical variability and a unique neuroimaging aspect

    Energy Technology Data Exchange (ETDEWEB)

    Leao, Emilia Katiane Embirucu; Lima, Marcilia Martyn; Kok, Fernando; Parizotto, Juliana [University of Sao Paulo (USP), Sao Paulo, SP (Brazil). Clinical Hospital. Dept. of Child Neurology; Maia Junior, Otacilio de Oliveira [University of Sao Paulo (USP), Sao Paulo, SP (Brazil). Clinical Hospital. Dept. of Child Ophthalmology

    2010-04-15

    Joubert syndrome (JS) is an autosomal recessive inherited disorder characterized by hypotonia, cerebellar vermis hypoplasia, ocular abnormalities (e.g. pigmentary retinopathy, oculomotor apraxia and nystagmus), renal cysts and hepatic fibrosis. Respiratory abnormalities, as apnoea and hyperpnoea, may be present, as well as mental retardation. At least seven JS loci have been determined and five genes identified. Herein, we report five children, belonging to independent families, with JS: they shared the same typical MRI abnormality, known as molar tooth sign, but had an otherwise quite variable phenotype, regarding mostly their cognitive performance, visual abilities and extra-neurological compromise. (author)

  5. Sleeping over a sleep disorder - Awareness of obstructive sleep apnoea as a modifiable risk factor for hypertension and stroke: A survey among health care professionals and medical students.

    Science.gov (United States)

    Sharma, Sushma; Srijithesh, P R

    2013-04-01

    Obstructive sleep apnoea (OSA) syndrome is an established and modifiable but under recognized risk factor for common disorders like stroke and hypertension. To assess awareness level of health care practitioners and medical students about OSA as a risk factor for stroke and hypertension. Questionnaire based survey with multiple response type and fill in the blanks type questions. The data was compiled and analyzed using SPSS version 19. 180 participants completed the survey questionnaire. Only 24 (13.3%) identified OSA as a reversible risk factor for ischemic stroke. 11 (6%) participants only could answer OSA as an identified risk factor for hypertension as per Seventh Joint National Committee report. This study reveals dismal level of awareness, among health professionals and medical students, about OSA being an established and modifiable risk factor for hypertension and ischemic stroke.

  6. Oxidative stress mediated arterial dysfunction in patients with obstructive sleep apnoea and the effect of continuous positive airway pressure treatment

    Directory of Open Access Journals (Sweden)

    Del Ben Maria

    2012-07-01

    Full Text Available Abstract Background Several studies suggest an increase of oxidative stress and a reduction of endothelial function in obstructive sleep apnoea syndrome (OSAS. We assessed the association between OSAS, endothelial dysfunction and oxidative stress. Further aim was to evaluate the effect of nasal continuous positive airway pressure (nCPAP on oxidative stress and arterial dysfunction. Methods We studied 138 consecutive patients with heavy snoring and possible OSAS. Patients underwent unattended overnight home polysomnography. Ten patients with severe OSAS were revaluated after 6 months of nCPAP therapy. To assess oxidative stress in vivo, we measured urinary 8-iso-PGF2α and serum levels of soluble NOX2-derived peptide (sNOX2-dp. Serum levels of nitrite/nitrate (NOx were also determined. Flow-mediated brachial artery dilation (FMD was measured to asses endothelial function. Results Patients with severe OSAS had higher urinary 8-iso-PGF2α (p Conclusions The results of our study indicate that patients with OSAS and cardiometabolic comorbidities have increased oxidative stress and arterial dysfunction that are partially reversed by nCPAP treatment.

  7. Long-term side effects on the temporomandibular joints and oro-facial function in patients with obstructive sleep apnoea treated with a mandibular advancement device

    DEFF Research Database (Denmark)

    Knappe, S W; Bakke, M; Svanholt, P

    2017-01-01

    Patients with obstructive sleep apnoea (OSA) in long-term treatment with a mandibular advancement device (MAD) to increase the upper airway space may develop changes in the temporomandibular joint (TMJ) and the oro-facial function due to the protruded jaw position during sleep. The aim was to inv......Patients with obstructive sleep apnoea (OSA) in long-term treatment with a mandibular advancement device (MAD) to increase the upper airway space may develop changes in the temporomandibular joint (TMJ) and the oro-facial function due to the protruded jaw position during sleep. The aim...... was to investigate the influence of long-term MAD treatment on the TMJs, oro-facial function and occlusion. This prospective study included 30 men and 13 women (median age 54) with OSA [Apnoea-Hypopnoea Index (AHI): 7-57]. They were examined with the Nordic Orofacial Test Screening (NOT-S), the Research Diagnostic...... changes in the TMJs, the oro-facial function and the occlusion. However, these changes seemed to be less harmful than previously reported with careful adaptation, control and follow-ups....

  8. Deep brain stimulation for Tourette syndrome.

    Science.gov (United States)

    Kim, Won; Pouratian, Nader

    2014-01-01

    Gilles de la Tourette syndrome is a movement disorder characterized by repetitive stereotyped motor and phonic movements with varying degrees of psychiatric comorbidity. Deep brain stimulation (DBS) has emerged as a novel therapeutic intervention for patients with refractory Tourette syndrome. Since 1999, more than 100 patients have undergone DBS at various targets within the corticostriatothalamocortical network thought to be implicated in the underlying pathophysiology of Tourette syndrome. Future multicenter clinical trials and the use of a centralized online database to compare the results are necessary to determine the efficacy of DBS for Tourette syndrome. Copyright © 2014 Elsevier Inc. All rights reserved.

  9. Arterial Hypertension in a Child with Williams-Beuren Syndrome (7q11.23 Chromosomal Deletion

    Directory of Open Access Journals (Sweden)

    Cristina de Sylos

    2002-08-01

    Full Text Available We report the case of a 7-year-old male child diagnosed with Williams-Beuren syndrome and arterial hypertension refractory to clinical treatment. The diagnosis was confirmed by genetic study. Narrowing of the descending aorta and stenosis of the renal arteries were also diagnosed. Systemic vascular alterations caused by deletion of the elastin gene may occur early in individuals with Williams-Beuren syndrome, leading to the clinical manifestation of systemic arterial hypertension refractory to drug treatment.

  10. Investigation of magnetic resonance imaging texture analysis as an aid tool for characterization of refractory epilepsies

    Directory of Open Access Journals (Sweden)

    Mauricio Martins Baldissin

    2013-12-01

    Full Text Available Refractory epilepsies are syndromes for which therapies that employ two or more antiepileptic drugs, separately or in association, do not result in control of crisis. Patients may present focal cortical dysplasia or diffuse dysplasia and/or hippocampal atrophic alterations that may not be detectable by a simple visual analysis in magnetic resonance imaging. The aim of this study was to evaluate MRI texture in regions of interest located in the hippocampi, limbic association cortex and prefrontal cortex of 20 patients with refractory epilepsy and to compare them with the same areas in 20 healthy individuals, in order to find out if the texture parameters could be related to the presence of the disease. Of the 11 texture parameters calculated, three indicated the existence of statistically significant differences between the studied groups. Such findings suggest the possibility of this technique contributing to studies of refractory epilepsies.

  11. Hypophosphatemia in a Malnourished Child: When Renal Fanconi Syndrome Does Not Stand for Refeeding Syndrome.

    Science.gov (United States)

    Runde, Joseph; Rivera-Rivera, Edgardo; Pompeii-Wolfe, Cecelia; Clardy, Christopher; Sentongo, Timothy

    2018-05-10

    Refeeding syndrome is diagnosed based on the onset of multiple laboratory abnormalities (most commonly hypophosphatemia) and clinical signs in the setting of nutrition rehabilitation of malnourished patients. Because definitions are not uniform, a broad differential diagnosis should always include renal tubular dysfunction. Our report details a 3 year-old child with undiagnosed renal tubular dysfunction who presented with the clinical picture of refeeding syndrome with refractory electrolyte abnormalities. A diagnosis of renal Fanconi syndrome was made after urinalysis that revealed glucosuria and urine electrolyte losses. Thus, urinalysis can aid in making a positive diagnosis of refeeding syndrome. © 2018 American Society for Parenteral and Enteral Nutrition.

  12. Report from a Survey of Parents Regarding the Use of Cannabidiol (Medicinal cannabis in Mexican Children with Refractory Epilepsy

    Directory of Open Access Journals (Sweden)

    Carlos G. Aguirre-Velázquez

    2017-01-01

    Full Text Available Structured online surveys were used to explore the experiences of the parents of children with refractory epilepsy using medicinal cannabis in Mexico during September 2016. The surveys, which were completed in full, were reviewed, and 53 cases of children aged between 9 months and 18 years were identified. Of these, 43 cases (82% were from Mexico and 10 (18% were from Latin American countries. Of the 43 Mexican cases, the diagnoses were as follows: 20 cases (47% had Lennox-Gastaut syndrome (LGS; 13 cases (30% had unspecified refractory epilepsy (URE; 8 cases (19% had West syndrome (WS; 1 case (2% had Doose syndrome (DS; and 1 case (2% had Ohtahara syndrome (OS. In total, 47.1% of cases had previously been treated with 9 or more anticonvulsant therapies. The parents reported a decrease in convulsions when cannabidiol was used in 81.3% of the cases; a moderate to significant decrease occurred in 51% of cases, and 16% of cases were free from seizure. The number of antiepileptic drugs being used was reduced in 9/43 (20.9% cases. No serious adverse effects were reported, with only some mild adverse effects, such as increased appetite or changes in sleep patterns, reported in 42% of cases.

  13. Refractory burning mouth syndrome: clinical and paraclinical evaluation, comorbiities, treatment and outcome.

    Science.gov (United States)

    Mitsikostas, Dimos D; Ljubisavljevic, Srdjan; Deligianni, Christina I

    2017-12-01

    Burning Mouth Syndrome (BMS) is a chronic pain condition characterized by persistent intraoral burning without related objective findings and unknown etiology that affects elderly females mostly. There is no satisfactory treatment for BMS. We aimed to observe the long-term efficacy of high velanfaxine doses combined with systemic and topical administered clonazepam in a particular subgroup of BMS patients who do not respond to current clinical management. Eight (66.1 ± 6.2 years old females) out of 14 BMS patients fulfilled the inclusion criteria and were treated with venlafaxine (300 mg/d) and clonazepam (5 mg/d) for 35.4 ± 12.1 (mean ± SD) months. The average duration of the symptoms at baseline was 4.3 ± 1.4 years and the overall mean daily pain intensity score was 8.6 ± 1.3 (VAS); pain was in tongue and within the oral mucosa, accompanying by oral and facial dysesthesia. In five patients tasting was abnormal. All patients had positive history of concomitant primary headache. The average score of Hamilton Rating scale for Anxiety and Depression was 21 ± 4.2, and 26.1 ± 2.9, respectively. Previous ineffective treatments include anticonvulsants and anti-depressants. All patients responded (more than 50% decrease in VAS) after three months treatment (mean VAS 3.2 ± 2.2) with no remarkable adverse events. BMS deserves bottomless psychiatric evaluation and management when current available treatments fail. Treatment with venlafaxine combined with topical and systemic clonazepam may be effective in refractory BMS cases but further investigation in a large-scale controlled study is needed to confirm these results.

  14. Proportional positive airway pressure: a new concept to treat obstructive sleep apnoea.

    Science.gov (United States)

    Juhász, J; Becker, H; Cassel, W; Rostig, S; Peter, J H

    2001-03-01

    Proportional positive airway pressure (PPAP) was designed to optimize airway pressure for the therapy of obstructive sleep apnoea (OSA). In a randomized crossover prospective study, the clinical feasibility of PPAP and its immediate effects on the breathing disorder and sleep in comparison with continuous positive airway pressure (CPAP) was evaluated. Twelve patients requiring CPAP therapy underwent CPAP and PPAP titration in a random order. Obstructive and mixed respiratory events could be completely abolished with both forms of treatment. This efficacy could be achieved at a significantly lower mean mask pressure during PPAP titration (8.45+/-2.42 cmH2O) compared to CPAP (9.96+/-2.7 cmH2O) (p=0.002). The mean minimal arterial oxygen saturation (Sa,O2) (82.8+/-6.5%) on the diagnostic night increased significantly (pPPAP titration. Total sleep time, slow wave sleep and rapid eye movement (REM) sleep increased significantly by the same amount during both CPAP and PPAP titration (pPPAP titration night, four patients did not have a preference, and two patients preferred CPAP. The present data show that proportional positive airway pressure is as effective as continuous positive airway pressure in eliminating obstructive events and has the same immediate effect on sleep. The lower average mask pressure during proportional positive airway pressure implies potential advantages compared to continuous positive airway pressure. Proportional positive airway pressure presents a new effective therapeutic approach to obstructive sleep apnoea.

  15. Metabolic Syndrome Predicts Refractoriness to Intravenous Thrombolysis in Acute Ischemic Stroke.

    Science.gov (United States)

    Dorado, Laura; Arenillas, Juan F; López-Cancio, Elena; Hernández-Pérez, María; Pérez de la Ossa, Natalia; Gomis, Meritxell; Millán, Mònica; Granada, María Luisa; Galán, Amparo; Palomeras, Ernest; Dávalos, Antoni

    2015-11-01

    Metabolic syndrome (MetS) has been associated with higher resistance to clot lysis at 24 hours after tissue plasminogen activator (tPA) administration in patients with acute ischemic stroke. We aimed to test this hypothesis at earlier time points, when neurointerventional rescue procedures may still be indicated to achieve arterial recanalization. This is a prospective and observational study in consecutive stroke patients with MCA occlusion treated with IV tPA. MetS was diagnosed following the unified criteria of the last Joint Interim Statement 2009 participating several major organizations. The primary outcome variable was resistance to thrombolysis, defined as the absence of complete middle cerebral artery recanalization 2 hours after tPA bolus assessed by transcranial color-coded duplex or when rescue mechanical thrombectomy after IV tPA was required. Secondary outcome variables were dramatic neurological improvement (decrease in ≥10 points, or a National Institutes of Health Stroke Scale [NIHSS] score of 0-1 at 24 hours), symptomatic intracerebral hemorrhage following European-Australasian Acute Stroke Study II criteria, infarct volume at 24 hours (calculated by using the formula for irregular volumes, ABC/2), and good outcome (modified Rankin Scale score < 3) at 3 months. A total of 234 patients (median baseline NIHSS score 16 [10-20]) were included and 146 (62.4%) fulfilled MetS criteria. After multivariate analysis, MetS emerged as an independent predictor of resistance to thrombolysis (odds ratio = 2.2 [1.3-4.2], P = .01) and absence of dramatic neurological improvement (odds ratio = .5 [.28-.97], P = .04). In addition, MetS conferred poorer functional outcome, higher symptomatic intracerebral hemorrhage rate, and increased infarct volume, although these associations disappeared after adjustment for covariates. MetS predicts patients with middle cerebral artery occlusion refractory to early clot dissolution after IV tPA. This

  16. Refractoriness in human atria

    DEFF Research Database (Denmark)

    Skibsbye, Lasse; Jespersen, Thomas; Christ, Torsten

    2016-01-01

    BACKGROUND: Refractoriness of cardiac cells limits maximum frequency of electrical activity and protects the heart from tonic contractions. Short refractory periods support major arrhythmogenic substrates and augmentation of refractoriness is therefore seen as a main mechanism of antiarrhythmic...... drugs. Cardiomyocyte excitability depends on availability of sodium channels, which involves both time- and voltage-dependent recovery from inactivation. This study therefore aims to characterise how sodium channel inactivation affects refractoriness in human atria. METHODS AND RESULTS: Steady......-state activation and inactivation parameters of sodium channels measured in vitro in isolated human atrial cardiomyocytes were used to parameterise a mathematical human atrial cell model. Action potential data were acquired from human atrial trabeculae of patients in either sinus rhythm or chronic atrial...

  17. Evaluation of the upper airway measurements by multi-slice CT before and after operations in obstructive sleep apnoea syndrome patients

    International Nuclear Information System (INIS)

    Tao Ping; Dang Yuqing; Chang Bei; Wang Xiao; Jin Zhengyu; Li Wuyi; Huo Hong

    2009-01-01

    Objective: To evaluate the changes of the upper airway of the patients with obstructive sleep apnoea syndrome (OSAS) before and after operations and to know the effects of operations by MSCT. Methods: The upper airway dimensions of 26 patients with OSAS were measured on multiplanar reformatted (MPR), curved-planar reformatted (CPR), volume rendering (VR) images of 16-slice spiral CT. The measurements include the anteroposterior calibres and the areas on the reformatted axial images on the pharyngeal cavity levels, the calibres and the minimum areas in retropalatal and retroglossal regions, the areas of the soft palate and uvula on the reformatted sagittal view with maximum thickness, the maximum wall thickness of the right and left the upper airway on the coronary images, the volume of the upper airway before and after the operations. The measurements were correlated with the polysomnography (PSG) records. The data were analyzed paired-samples t-test and Pearson correlations. Results: By comparison, the anteroposterior calibres and the cross-sectional areas on the reformatted axial view of the lower retropalatal region (slice 4) of the upper airway increased significantly after operations. The anteroposterior diameter increased from 5.9 mm before operations to 12.8 mm after operations, where t=-5.506, P 2 before operations to 275.0 mm 2 after operations, where t=-5.011, P 2 before operations to 128.0 mm 2 after operations, where t=3.087, P 2 before operation to 10.9 mm, 76.0 mm 2 after operation, where t=-3.413, -2.216, respectively and P 2 before operations to 76.0 mm 2 after operations, were t=-4.932, P<0.05. The anteroposterior calibres increased from 4.6 mm before operations to 6.6 mm after operations, where t=-7.308, P<0.05. The L-R calibres increased from 8.3 mm before operations to 13.6 mm after operations, where t=-4.320, P<0.05. Conclusions: MPR, CPR, VR of MSCT can evaluate the not only the morphology but the function changes of the upper airways on the

  18. Nasal CPAP and surfactant for treatment of respiratory distress syndrome and prevention of bronchopulmonary dysplasia

    DEFF Research Database (Denmark)

    Verder, Henrik; Bohlin, Kajsa; Kamper, Jens

    2009-01-01

    The Scandinavian approach is an effective combined treatment for respiratory distress syndrome (RDS) and prevention of bronchopulmonary dysplasia (BPD). It is composed of many individual parts. Of significant importance is the early treatment with nasal continuous positive airway pressure (n......CPAP) and surfactant treatment. The approach may be supplemented with caffeine citrate and non-invasive positive pressure ventilation for apnoea. The low incidence of BPD seen as a consequence of the treatment strategy is mainly due to a reduced need for mechanical ventilation (MV). Conclusion: Early...

  19. Comparison of long-term outcomes between children with aplastic anemia and refractory cytopenia of childhood who received immunosuppressive therapy with antithymocyte globulin and cyclosporine.

    Science.gov (United States)

    Hama, Asahito; Takahashi, Yoshiyuki; Muramatsu, Hideki; Ito, Masafumi; Narita, Atsushi; Kosaka, Yoshiyuki; Tsuchida, Masahiro; Kobayashi, Ryoji; Ito, Etsuro; Yabe, Hiromasa; Ohga, Shouichi; Ohara, Akira; Kojima, Seiji

    2015-11-01

    The 2008 World Health Organization classification proposed a new entity in childhood myelodysplastic syndrome, refractory cytopenia of childhood. However, it is unclear whether this morphological classification reflects clinical outcomes. We retrospectively reviewed bone marrow morphology in 186 children (median age 8 years; range 1-16 years) who were enrolled in the prospective study and received horse antithymocyte globulin and cyclosporine between July 1999 and November 2008. The median follow-up period was 87 months (range 1-146 months). Out of 186 patients, 62 (33%) were classified with aplastic anemia, 94 (49%) with refractory cytopenia of childhood, and 34 (18%) with refractory cytopenia with multilineage dysplasia. Aplastic anemia patients received granulocyte colony-stimulating factor more frequently and for longer durations than other patients (Paplastic anemia, 4 patients with refractory cytopenia of childhood, and 3 patients with refractory cytopenia with multilineage dysplasia. Although the cumulative incidence of total clonal evolution at ten years was not significantly different among the 3 groups, the cumulative incidence of monosomy 7 development was significantly higher in aplastic anemia than in the other groups (P=0.02). Multivariate analysis revealed that only granulocyte colony-stimulating factor administration duration of 40 days or more was a significant risk factor for monosomy 7 development (P=0.02). These findings suggest that even the introduction of a strict morphological distinction from hypoplastic myelodysplastic syndrome cannot eradicate clonal evolution in children with aplastic anemia. Copyright© Ferrata Storti Foundation.

  20. Eosinophilic granulomatosis with polyangiitis (formerly known as Churg-Strauss syndrome as a differential diagnosis of hypereosinophilic syndromes

    Directory of Open Access Journals (Sweden)

    Yuri Albuquerque Pessoa Santos

    2017-01-01

    Full Text Available Eosinophilic granulomatosis with polyangiitis (EGPA, formerly known as Churg-Strauss syndrome, is a rare systemic disease situated between primary small vessel vasculitides associated with antineutrophil cytoplasmic antibodies (ANCAs and hypereosinophilic syndromes (HES. Here, we present a case of EGPA in a 38-year-old male, with a previous diagnosis of asthma, who presented with fever, migratory lung infiltrates and systemic eosinophilia that was refractory to previous courses of antibiotics. This case highlights the importance of the primary care physician understanding the differential diagnosis of pulmonary eosinophilic syndromes.

  1. Plasma spraying of refractory metals and refractory hard materials. State of the art

    International Nuclear Information System (INIS)

    Eschnauer, H.; Lugscheider, E.; Jaeger, D.

    1989-01-01

    Suitable spraying processes for manufacturing refractory metals, refractory hard materials as well as spray materials with refractory components are the VPS- and IPS-spraying techniques. The advantages of these special spraying process variations are described. The reactive spraying materials are systematically organized. The characteristical properties used in purpose of improving the substrate surfaces are explained. Finally some examples of the latest results of research concerning plasma spraying of reactive materials are shown. 16 refs., 10 figs. (Author)

  2. Acquired Bartter syndrome following gentamicin therapy.

    Science.gov (United States)

    Singh, J; Patel, M L; Gupta, K K; Pandey, S; Dinkar, A

    2016-01-01

    Aminoglycoside nephrotoxicity may manifest as nonoliguric renal failure or tubular dysfunction, such as Fanconi-like syndrome, Bartter-like syndrome (BS), or distal renal tubular acidosis. We report a case who developed severe renal tubular dysfunction on the the 7 th day of gentamicin therapy, resulting in metabolic alkalosis, refractory hypokalemia, hypocalcemia, hypomagnesemia, and polyuria. The patient was diagnosed as a case of transient BS associated with gentamicin exposure. The patient recovered with conservative management.

  3. Screening and managing obstructive sleep apnoea in nocturnal heart block patients: an observational study.

    Science.gov (United States)

    Wu, Xu; Liu, Zilong; Chang, Su Chi; Fu, Cuiping; Li, Wenjing; Jiang, Hong; Jiang, Liyan; Li, Shanqun

    2016-02-16

    Nocturnal heart block often occurs in patients with obstructive sleep apnoea (OSA). It is more likely to be undiagnosed in heart block patients who are ignorant of the symptoms of sleep disorder. Berlin Questionnaire (BQ) is a highly reliable way to discover the risk factors of OSA, whereas the validity in sleep-related heart block patients is uncertain. We performed an observational study to address these issues and confirmed the potential protective effect of continuous positive airway pressure (CPAP). Patients who were previously diagnosed with nocturnal heart block with R-R pauses exceeding 2 seconds were retrospective screened from the ECG centre of Zhongshan hospital. These recruited participants completed Berlin Questionnaire and underwent polysomnography synchronously with 24-hour Holter monitoring. A cross-sectional analysis was performed to confirm the association between nocturnal arrhythmia and OSA, as well as to assess the diagnostic accuracy of the BQ. Subsequently, subjects diagnosed with OSA (apnoea-hypopnoea index > 5) underwent 3 consecutive days of CPAP therapy. On the third day, patients repeated 24-hour Holter monitoring within the institution of CPAP. The symptoms of disruptive snoring and hypersomnolence in 72 enrolled patients were more related to the occurrence of nocturnal heart block (r = 0.306, 0.226, respectively, p = 0.015, 0.019) than syncope (r = 0.134, p = 0.282) and palpitations (r = 0.106, p = 0.119), which were prominent trait of our study population. The sensitivity, specificity, positive and negative predictive value of the BQ at a cut-off point of 5 of AHI for detecting OSA in heart block patients was 81.0 %, 44.4 %, 91.07 % and 25 %. Nocturnal heart block does not appear to occur exclusively in severe sleep apnoea. The frequent occurrence of arrhythmias in prominent oxygen desaturation supports the correlation between them. CPAP therapy resulted in significant decrease in the average number of

  4. Theoretical and practical aspects about corrosion of refractories used in steel metallurgy: part 3: characterization of commercial refractories

    International Nuclear Information System (INIS)

    Braganca, S.R.

    2012-01-01

    In this study, it was reviewed the main aspects found in the literature about refractories corrosion, evaluating the feasibility of certain tests and relating them with experimental results. The physical properties and microstructure of commercial refractories were analyzed, considering the differences between them and the quality implications and probable life of the refractory. Thus, it was studied the various types of refractories used as lining on steel ladle. Magnesia-carbon and doloma-carbon refractories were analyzed, highlighting the differences between them. The examined refractory showed characteristics favoring high resistance to corrosion process, presenting a series of properties to be selected in accordance with industry practice. (author)

  5. Are We overestimating the prevalence of depression in chronic illness using questionnaires? Meta-analytic evidence in obstructive sleep apnoea

    DEFF Research Database (Denmark)

    Nanthakumar, Shenooka; Bucks, Romola S.; Skinner, Timothy C.

    2016-01-01

    Depression is common in chronic illness, albeit prevalence can be highly variable. This variability may be a function of symptom overlap between depression and chronic illness. Using Obstructive Sleep Apnoea (OSA) as an exemplar, this meta-analysis explored whether the proportion of overlapping s...

  6. Sweet's Syndrome Successfully Treated with Granulocyte and Monocyte Adsorption Apheresis

    OpenAIRE

    Fujii, Asami; Mizutani, Yoko; Hattori, Yuki; Takahashi, Tomoko; Ohnishi, Hidenori; Yoshida, Shozo; Seishima, Mariko

    2017-01-01

    Sweet’s syndrome is a neutrophilic dermatosis characterized by an abrupt onset of painful erythematous lesions showing neutrophilic infiltrates in the dermis. Fever and an elevated neutrophil level are generally observed. Sweet’s syndrome may be idiopathic, malignancy-associated, or drug-induced (mainly involving granulocyte colony-stimulating factor (G-CSF) administration). Although systemic corticosteroids are usually effective, the symptoms of Sweet’s syndrome recur in some refractory case...

  7. Intravenous levetiracetam terminates refractory status epilepticus in two patients with migrating partial seizures in infancy.

    Science.gov (United States)

    Cilio, Maria Roberta; Bianchi, Roberto; Balestri, Martina; Onofri, Alfredo; Giovannini, Simona; Di Capua, Matteo; Vigevano, Federico

    2009-09-01

    To evaluate the efficacy and tolerability of intravenous (IV) levetiracetam in refractory status epilepticus of migrating partial seizures in infancy (MPSI). IV levetiracetam was infused in two infants, first as a loading dose of 60mg/kg in 30min, then at 30mg/kg twice a day. Both infants were continuously monitored with video-EEG before, during and after the drug trial. Blood count, liver enzymes, serum creatinine, ammonia and lactate blood levels were performed repeatedly before and after the IV levetiracetam administration. Follow-up was of 16 and 10 months. EEG monitoring allowed the diagnosis of MPSI, showing the typical seizures pattern in both patients. IV levetiracetam was effective in stopping status epilepticus in both infants. Levetiracetam also prevented the recurrence of status epilepticus during follow-up. No adverse reactions were observed during the infusion phase or during follow-up. MPSI is a newly recognized epileptic syndrome characterized by early onset of intractable partial seizures arisingly independently and sequentially from both hemispheres, migrating from one region of the brain to another and from one hemisphere to another. We report the efficacy of intravenous levetiracetam in resolving refractory status epilepticus in two infants with this new epilepsy syndrome.

  8. Acquired Bartter syndrome following gentamicin therapy

    Directory of Open Access Journals (Sweden)

    J Singh

    2016-01-01

    Full Text Available Aminoglycoside nephrotoxicity may manifest as nonoliguric renal failure or tubular dysfunction, such as Fanconi-like syndrome, Bartter-like syndrome (BS, or distal renal tubular acidosis. We report a case who developed severe renal tubular dysfunction on the the 7 th day of gentamicin therapy, resulting in metabolic alkalosis, refractory hypokalemia, hypocalcemia, hypomagnesemia, and polyuria. The patient was diagnosed as a case of transient BS associated with gentamicin exposure. The patient recovered with conservative management.

  9. Effect of CPAP on arterial stiffness in severely obese patients with obstructive sleep apnoea.

    Science.gov (United States)

    Seetho, Ian W; Asher, Rebecca; Parker, Robert J; Craig, Sonya; Duffy, Nick; Hardy, Kevin J; Wilding, John P H

    2015-12-01

    Obstructive sleep apnoea (OSA) may independently increase cardiovascular risk in obesity. Although there is evidence that arterial stiffness is altered in OSA, knowledge of these effects with continuous positive airway pressure (CPAP) in severe obesity (body mass index (BMI) ≥ 35 kg/m(2)) is limited. This study aimed to explore how arterial stiffness, as measured by the augmentation index (Aix), changed in severely obese patients with OSA who were treated with CPAP and in patients without OSA. Forty-two patients with severe obesity-22 with OSA, 20 without OSA-were recruited at baseline and followed-up after a median of 13.5 months. Pulse wave analysis (PWA) was performed using applanation tonometry at the radial artery to measure augmentation index (Aix), augmentation pressure (AP) and subendocardial viability ratio (SEVR). Cardiovascular parameters and body composition were also measured. There were significant improvements in Aix, AP (both P CPAP compared with subjects without OSA. Epworth scores (P CPAP. Regression showed that CPAP was significantly associated with change in arterial stiffness from baseline. However, patients with OSA on CPAP continued to have increased arterial stiffness (Aix) (P CPAP in severe obesity, CPAP alone is not sufficient to modify PWA measures to levels comparable with non-OSA patients. This supports a need for a multifaceted approach when managing cardiovascular risk in patients with severe obesity and obstructive sleep apnoea receiving CPAP therapy.

  10. Nocturnal motor activity in fibromyalgia patients with poor sleep quality.

    Science.gov (United States)

    Hyyppä, M T; Kronholm, E

    1995-01-01

    Nocturnal motor activity was examined in long-term rehabilitation patients complaining of poor sleep and having fibromyalgia syndrome (N = 24) or other musculoskeletal disorders (N = 60) and compared with that in 91 healthy controls drawn from a random community sample. Self-reports on sleep complaints and habits were collected. The frequency of nocturnal body movements, the "apnoea" index and ratio of "quiet sleep" to total time in bed were measured using the Static Charge Sensitive Bed (SCSB) (BioMatt). As a group, patients with fibromyalgia syndrome did not differ from patients with other musculoskeletal disorders or from healthy controls in their nocturnal motor activity. The "apnoea" index was a little higher in the fibromyalgia group than in the healthy control group but did not differ from that of the group of other musculoskeletal patients. Further multivariate analyses adjusted for age, BMI, medication and "apnoea" index did not support the assumption that an increased nocturnal motor activity characterizes patients with fibromyalgia syndrome.

  11. Evaluation of the reusing of MGO-C refractory brick in refractory mixes

    International Nuclear Information System (INIS)

    Silva, R.D.S. da; Braganca, S.R.

    2012-01-01

    The residue from the use of MgO-C refractories in electric arc furnace presents, mostly, magnesium oxide in its composition and some slag contamination and impurities from the process of electrofusion scrap iron/steel. In this study, it was studied the characteristics of this residue and reused through its introduction into a commercial refractory mix, employed as material of coating and repair. This refractory mix was tested by thermogravimetric analysis, compressive strength, evaluation of plasticity and porosity as well as aspects of its installation, such as adhesion in situ. Results showed that there potential for reuse with the introduction of the waste in commercial mix was 30%, with little loss of compressive strength and plasticity. (author)

  12. Transnasal Humidified Rapid-Insufflation Ventilatory Exchange (THRIVE): a physiological method of increasing apnoea time in patients with difficult airways.

    Science.gov (United States)

    Patel, A; Nouraei, S A R

    2015-03-01

    Emergency and difficult tracheal intubations are hazardous undertakings where successive laryngoscopy-hypoxaemia-re-oxygenation cycles can escalate to airway loss and the 'can't intubate, can't ventilate' scenario. Between 2013 and 2014, we extended the apnoea times of 25 patients with difficult airways who were undergoing general anaesthesia for hypopharyngeal or laryngotracheal surgery. This was achieved through continuous delivery of transnasal high-flow humidified oxygen, initially to provide pre-oxygenation, and continuing as post-oxygenation during intravenous induction of anaesthesia and neuromuscular blockade until a definitive airway was secured. Apnoea time commenced at administration of neuromuscular blockade and ended with commencement of jet ventilation, positive-pressure ventilation or recommencement of spontaneous ventilation. During this time, upper airway patency was maintained with jaw-thrust. Transnasal Humidified Rapid-Insufflation Ventilatory Exchange (THRIVE) was used in 15 males and 10 females. Mean (SD [range]) age at treatment was 49 (15 [25-81]) years. The median (IQR [range]) Mallampati grade was 3 (2-3 [2-4]) and direct laryngoscopy grade was 3 (3-3 [2-4]). There were 12 obese patients and nine patients were stridulous. The median (IQR [range]) apnoea time was 14 (9-19 [5-65]) min. No patient experienced arterial desaturation gaseous exchange through flow-dependent deadspace flushing. It has the potential to transform the practice of anaesthesia by changing the nature of securing a definitive airway in emergency and difficult intubations from a pressured stop-start process to a smooth and unhurried undertaking. © 2014 The Authors Anaesthesia published by John Wiley & Sons Ltd on behalf of Association of Anaesthetists of Great Britain and Ireland.

  13. Febrile Infection-Related Epilepsy Syndrome (FIRES): An Overview of Treatment and Recent Patents.

    Science.gov (United States)

    Hon, Kam Lun E Lun; Leung, Alexander K C; Torres, Alcy R

    2018-05-08

    New-onset refractory status epilepticus (NORSE) refers to a clinical presentation in a patient without active epilepsy or other existing relevant neurological disorder, with new onset of refractory status epilepticus in the absence of a clear acute or active structural, metabolic, or toxic cause. Febrile infection-related epilepsy syndrome (FIRES) is a subset of NORSE that requires a febrile infection between 24 hours and 2 weeks prior to the onset of refractory status epilepticus, with or without fever at the onset of status epilepticus, and with no restriction to the age of the patient. The literature on FIRES is scarce. This article reviews the pathophysiology, clinical features, and various treatment modalities in the treatment of FIRES. A Medline/Pubmed search was conducted using Clinical Queries with the key terms "febrile infection-related epilepsy syndrome", "FIRES", "new-onset refractory status epilepticus" and "NORSE". The search strategy included meta-analyses, randomized controlled trials, clinical trials, reviews and pertinent references. Patents were searched using the key term "FIRES", "NORSE" and "febrile epilepsy syndrome" from www.google.com/patents, www.uspto.gov, and www.freepatentsonline.com. FIRES almost invariably begins with a mild nonspecific febrile illness in an otherwise healthy individual. Twenty four hours to two weeks later, seizures begin and quickly become very frequent and worsen, becoming status epilepticus. Seizures can be simple motor, complex partial or secondary generalized. The exact etiology is no known. It is possible that the syndrome is caused by an inflammatory or autoimmune mechanism. Seizures in FIRES are notoriously very difficult to treat. Treatment modalities include, among others, various antiepileptic drugs, ketogenic diet, intravenous corticosteroids, intravenous immunoglobulin, and burst-suppression coma. Outcome is poor; most children are left with significant cognitive disability and refractory epilepsy

  14. The effect of sleep onset on upper airway muscle activity in patients with sleep apnoea versus controls

    Science.gov (United States)

    Fogel, Robert B; Trinder, John; White, David P; Malhotra, Atul; Raneri, Jill; Schory, Karen; Kleverlaan, Darci; Pierce, Robert J

    2005-01-01

    Pharyngeal dilator muscles are important in the pathophysiology of obstructive sleep apnoea syndrome (OSA). We have previously shown that during wakefulness, the activity of both the genioglossus (GGEMG) and tensor palatini (TPEMG) is greater in patients with OSA compared with controls. Further, EMG activity decreases at sleep onset, and the decrement is greater in apnoea patients than in healthy controls. In addition, it is known that the prevalence of OSA is greater in middle-aged compared with younger men. Thus, we had two goals in this study. First we compared upper airway muscle activity between young and middle-aged healthy men compared with men with OSA. We also explored the mechanisms responsible for the decrement in muscle activity at sleep onset in these groups. We investigated muscle activity, ventilation , and upper airway resistance (UAR) during wakefulness and sleep onset (transition from α to θ EEG activity) in all three groups. Measurements were obtained during basal breathing (BB) and nasal continuous positive airway pressure (CPAP) was applied to reduce negative pressure-mediated muscle activation). We found that during wakefulness there was a gradation of GGEMG and UAR (younger < older < OSA) and that muscle activity was reduced by the application of nasal CPAP (to a greater degree in the OSA patients). Although CPAP eliminated differences in UAR during wakefulness and sleep, GGEMG remained greater in the OSA patients. During sleep onset, a greater initial fall in GGEMG was seen in the OSA patients followed by subsequent muscle recruitment in the third to fifth breaths following the α to θ transition. On the CPAP night, and GGEMG still fell further in the OSA patients compared with control subjects. CPAP prevented the rise in UAR at sleep onset along with the associated recruitment in GGEMG. Differences in TPEMG among the groups were not significant. These data suggest that the middle-aged men had upper airway function midway between that of

  15. Severe atypical herpes zoster as an initial symptom of fatal myelodysplastic syndrome with refractory anemia and blast excess (RAEB II

    Directory of Open Access Journals (Sweden)

    Wollina U

    2017-05-01

    Full Text Available Uwe Wollina,1 Gesina Hansel,1 Anja Baunacke,1 Georgi Tchernev2 1Department of Dermatology and Allergology, Academic Teaching Hospital Dresden-Friedrichstadt, Dresden, Germany; 2Department of Dermatology and Dermatologic Surgery, Medical Institute of Ministry of Interior (MVR, Sofia, Bulgaria Abstract: Herpes zoster is a common disease caused due to varicella zoster virus (VZV infection with increasing incidence by age. If the patient has a severe, extended, or treatment-recalcitrant course of herpes zoster, this must be a red flag to search for underlying pathologies. Here, we report about a 64-year-old male patient with diabetes, who came to our emergency department because of general malaise, fever, chills, and a pronounced nuchal and facial swelling on the left side. Based on herpetiform-grouped vesicles and yellowish crusts, an impetiginized facial herpes zoster was diagnosed, and combined antiviral and antibiotic treatment was initiated. He was HIV negative. Despite intensified treatment, his situation worsened. We observed blasts in peripheral blood, but bone marrow biopsy was initially denied. Some days later after deterioration of his disease, he accepted further diagnostics. A myelodysplastic syndrome with blast excess (refractory anemia and blast excess II, RAEB II could be confirmed. The following translocations were detected: t(2;12(p13; q13 and t(6;9(p22;q34. REAB II has an unfortunate prognosis. Cytoreductive treatment was initiated by the hemato-oncologist. Unfortunately, the patient deceased due to septic shock. Keywords: herpes zoster, varicella zoster virus, myelodysplastic syndrome, sepsis, emergency

  16. Predicting uptake of continuous positive airway pressure (CPAP) therapy in obstructive sleep apnoea (OSA)

    DEFF Research Database (Denmark)

    Skinner, Timothy; McNeil, Lindsay; Olaithe, Michelle

    2013-01-01

    diagnosed with OSA. Epworth sleepiness scale (ESS), Fatigue Severity Scale, Depression Anxiety Stress Scale and Illness Perception Questionnaire-Revised (IPQ-R) were administered at time of sleep study. These, patient demographics and sleep study variables were used to determine factors predicting patient......Purpose: Obstructive sleep apnoea (OSA) is a common disorder, for which continuous positive airway pressure (CPAP) therapy is a standard treatment. Despite its well-established efficacy, many patients choose not to initiate CPAP treatment. The present study investigated the degree to which...

  17. Obesity-hypoventilation syndrome and associated factors.

    Science.gov (United States)

    Espínola Rodríguez, Ana; Lores Obradors, Luis; Parellada Esquius, Neus; Rubio Muñoz, Felisa; Espinosa Gonzalez, Neus; Arellano Marcuello, Elisabet

    2018-02-23

    Obesity causes important alterations in the respiratory physiology like sleep obstructive apnoea (SOA) and obesity-hypoventilation syndrome (OHS), both associated with high morbidity and mortality. Also, these entities are clearly infradiagnosed and in the case of OHS the prevalence is unknown in the general obese population. To determine the prevalence of OHS in the population of patients with morbid obesity and to know the comorbidity related with OHS, the associated respiratory symptoms and the pulse oximetry alterations. Descriptive study. Selection of 136 adult patients with morbid obesity (BMI >40). Collected were, anthropometric data, toxic habits, concomitant disease, symptom data, analytic data, dyspnoea grade, sleepiness scale (Epworth Test), electrocardiogram, chest X-ray, spirometry, nocturne ambulatory pulse oximetry and arterial gasometry. 136 were studied, mean age 60 years old (SD 12.9 years), 73% (98) were women; 6.6% of patients presented diurnal hypercapnia indicative of OHS; 72% presented high blood pressure, 44% dyslipidaemia, 18% presented cardiovascular disease, 83% snored and 46% had apnoea; 30% presented stageII dyspnoea and 10% stageIII. The desaturation/hour index was above 3% ≥30 of occasions in 28.6% of patients and the percentage of patients with saturations <90% more than 30% of the time was 23.5%. The results were worse in patients with OHS. The prevalence of OHS was lower than expected. Noteworthy was the high comorbidity of cardiovascular disease and the high frequency of respiratory symptoms associated with important alterations of pulse oximetry. Copyright © 2017 Elsevier España, S.L.U. All rights reserved.

  18. Compatibility of refractory materials with boiling sodium

    International Nuclear Information System (INIS)

    Meacham, S.A.

    1976-01-01

    The program employed to determine the compatibility of commercially available refractories with boiling sodium is described. The effects of impurities contained within the refractory material, and their relations with the refractory's physical stability are discussed. Also, since consideration of refractories for use as an insulating material within Liquid Metal Fast Breeder Reactor Plants (LMFBR's) is currently under investigation; recommendations, based upon this program, are presented

  19. REFRACTORY THROMBOCYTOPENIA AND NEUTROPENIA: A DIAGNOSTIC CHALLENGE

    OpenAIRE

    Emmanuel Gyan; François Dreyfus; Pierre Fenaux

    2015-01-01

    Background. The 2008 WHO classification identified refractory cytopenia with unilineage dysplasia (RCUD) as a composite entity encompassing refractory anemia, refractory thrombocytopenia (RT), and refractory neutropenia (RN), characterized by 10% or more dysplastic cells in the bone marrow respective lineage. The diagnosis of RT and RN is complicated by several factors.  Diagnosing RT first requires exclusion of familial thrombocytopenia, chronic auto-immune thrombocytopenia, concomitant medi...

  20. Ocular Features of Cerebro-Costo-Mandibular Syndrome.

    Science.gov (United States)

    Hameed, Zoya; Taylor, Simon; Lindfield, Dan

    2018-01-01

    Cerebro-costo-mandibular syndrome (CCMS) is a rare hereditary disorder characterized by micrognathia, posterior rib gaps, and secondary developmental delay. Patients often require ventilation and feeding support throughout life. We describe the first reported ophthalmic findings of CCMS and propose that defects in choroidal permeability lead to chronic macular edema and refractory aqueous misdirection syndrome. Here we discuss the medical and surgical management concerns of recurrent angle closure and raised intraocular pressure in a CCMS patient.

  1. Refractory bin for burning

    Energy Technology Data Exchange (ETDEWEB)

    McPherson, D.L.; McPherson, T.L.

    1989-12-26

    This patent describes a refractory bin. It has a generally rectangular horizontal cross sectional configuration. It has wall structures each comprising an upper and a lower pair of elongated horizontal vertically spaced generally parallel support beams each having a vertical flange defining a support edge along its upper surface, a first generally rectangular refractory panel arranged with its lower edge at the bottom of the bin and with its outer surface in flat face contacting relation with the vertical flanges of the lower pair of support beams, a plurality of brackets each having a horizontal part and a vertical part and being secured to the outer surface of the first refractory panel.

  2. Systematic review and meta-analysis of complications and mortality of veno-venous extracorporeal membrane oxygenation for refractory acute respiratory distress syndrome.

    Science.gov (United States)

    Vaquer, Sergi; de Haro, Candelaria; Peruga, Paula; Oliva, Joan Carles; Artigas, Antonio

    2017-12-01

    Veno-venous extracorporeal membrane oxygenation (ECMO) for refractory acute respiratory distress syndrome (ARDS) is a rapidly expanding technique. We performed a systematic review and meta-analysis of the most recent literature to analyse complications and hospital mortality associated with this technique. Using the PRISMA guidelines for systematic reviews and meta-analysis, MEDLINE and EMBASE were systematically searched for studies reporting complications and hospital mortality of adult patients receiving veno-venous ECMO for severe and refractory ARDS. Studies were screened for low bias risk and assessed for study size effect. Meta-analytic pooled estimation of study variables was performed using a weighted random effects model for study size. Models with potential moderators were explored using random effects meta-regression. Twelve studies fulfilled inclusion criteria, representing a population of 1042 patients with refractory ARDS. Pooled mortality at hospital discharge was 37.7% (CI 95% = 31.8-44.1; I 2  = 74.2%). Adjusted mortality including one imputable missing study was 39.3% (CI 95% = 33.1-45.9). Meta-regression model combining patient age, year of study realization, mechanical ventilation (MV) days and prone positioning before veno-venous ECMO was associated with hospital mortality (p < 0.001; R 2  = 0.80). Patient age (b = 0.053; p = 0.01) and maximum cannula size during treatment (b = -0.075; p = 0.008) were also independently associated with mortality. Studies reporting H1N1 patients presented inferior hospital mortality (24.8 vs 40.6%; p = 0.027). Complication rate was 40.2% (CI 95% = 25.8-56.5), being bleeding the most frequent 29.3% (CI 95% = 20.8-39.6). Mortality due to complications was 6.9% (CI 95% = 4.1-11.2). Mechanical complications were present in 10.9% of cases (CI 95% = 4.7-23.5), being oxygenator failure the most prevalent (12.8%; CI 95% = 7.1-21.7). Despite initial severity, significant portion of patients

  3. Autologous transplantation of the internal limiting membrane for refractory macular holes.

    Science.gov (United States)

    Morizane, Yuki; Shiraga, Fumio; Kimura, Shuhei; Hosokawa, Mio; Shiode, Yusuke; Kawata, Tetsuhiro; Hosogi, Mika; Shirakata, Yukari; Okanouchi, Toshio

    2014-04-01

    To determine the effectiveness of autologous transplantation of the internal limiting membrane (ILM) for refractory macular holes. Prospective, interventional case series. Ten eyes of 10 consecutive patients who underwent autologous transplantation of the ILM for the treatment of refractory macular holes were studied. The primary diseases in these patients were large idiopathic macular holes that had existed for more than 1 year (4 eyes), a traumatic macular hole (1 eye), myopic foveoschisis (2 eyes), foveoschisis resulting from pit-macular syndrome (2 eyes), and proliferative diabetic retinopathy (1 eye). Apart from the 5 eyes with idiopathic or traumatic macular holes, macular holes developed in the other 5 eyes after initial vitrectomies with ILM removal. In all eyes, regular macular hole surgery failed to achieve closure. The main outcome measures used in this study were macular hole closure and best-corrected visual acuity (BCVA). Macular holes were closed successfully in 9 eyes (90%) after autologous transplantation of the ILM. The postoperative BCVAs were significantly better than the preoperative BCVAs (P = .007, paired t test). Postoperative BCVAs improved by more than 0.2 logarithm of the minimal angle of resolution units in 8 eyes (80%) and were unchanged in 2 eyes (20%). Although this is a pilot study, the results suggest that autologous transplantation of the ILM may contribute to improved anatomic and visual outcomes in the treatment of refractory macular holes and may warrant further investigation. Copyright © 2014 Elsevier Inc. All rights reserved.

  4. Morbidly obese patient with obstructive sleep apnoea for major spine surgery: An anaesthetic challenge

    Directory of Open Access Journals (Sweden)

    Shruti Redhu

    2016-01-01

    Full Text Available Morbidly obese patients with clinical features of obstructive sleep apnoea can present a myriad of challenges to the anaesthesiologists which must be addressed to minimise the perioperative risks. Initiation of continuous positive airway pressure (CPAP therapy early in the pre- and post-operative period along with appropriate anaesthetic planning is of paramount importance in such patients. This case report emphasises the usefulness of CPAP therapy, even for a short duration, to minimise morbidity, improve recovery and hasten early discharge from the hospital after major surgery.

  5. Polymorphism of the ACE gene and the risk of obstructive sleep apnoea.

    Science.gov (United States)

    Chmielewska, Izabela; Mlak, Radosław; Krawczyk, Paweł; Czukiewska, Ewa; Milanowski, Janusz

    2013-01-01

    Obstructive sleep apnoea/hypopnea syndrome (OSA) is characterized by obstruction of the upper airway during sleep, resulting in repetitive breathing pauses accompanied by oxygen desaturation and arousal from sleep. Among the candidate genes affecting the risk of OSA, genes whose polymorphisms influence the development of diseases with similar pathogenesis such as OSA could be listed: APOE, genes for leptin and leptin receptor, TNFA1, ADRB2 and ACE (gene for angiotensin-converting enzyme). Until now there has been a confirmed relationship between ACE gene polymorphism and cardiovascular diseases, but its effect on the incidence of OSA is debatable. The aim of this study was to investigate the effect of ACE gene insertion/deletion (I/D) polymorphism on the risk of OSA. Fifty-five patients with confirmed diagnose of OSA and qualified to CPAP therapy entered the study. The control group included 50 subjects who did not complain of any sleep related symptoms. Diagnose of OSA was set on the basis of full overnight polysomnography together with Epworth Sleepiness Scale according to American Academy of Sleep Medicine guidelines. DNA was isolated from peripheral blood leukocytes with Qiagen DNA mini Kit. ACE gene polymorphism was determined in genomic DNA using allele specific polymerase chain reaction. Different sizes of PCR products were observed on agarose gel electrophoresis. There were non-significant differences in the frequency of ACE genotypes. However, allele D had significantly lower prevalence in the study group than in the control group. (χ(2) = 4.25 p = 0.04). Moreover, I allele carriers had a threefold greater risk of developing OSA (HR = 2.748, 95% CI = 1.029-7.340, p gene polymorphism might be useful to determine the risk of developing OSA in clinically predisposed patients.

  6. Retrospective analysis of rituximab therapy and splenectomy in childhood chronic and refractory immune thrombocytopenic purpura.

    Science.gov (United States)

    Ay, Yilmaz; Karapinar, Tuba H; Oymak, Yesim; Toret, Ersin; Demirag, Bengu; Ince, Dilek; Ozcan, Esin; Moueminoglou, Nergial; Koker, Sultan A; Vergin, Canan

    2016-06-01

    Immune thrombocytopenic purpura (ITP) results from accelerated platelet destruction mediated by autoantibodies to platelet glycoproteins. Some patients with chronic ITP are refractory to all therapies [steroids, intravenous immunoglobulin (IVIG), anti-D and immunosuppresive drugs] and have chronic low platelet counts and episodic bleeding. We retrospectively evaluated the efficacy and safety of rituximab treatment and splenectomy in paediatric patients diagnosed with chronic and refractory ITP who were unresponsive to steroids, IVIG, cyclosporine and mycophenolate mofetil. Records of patients with chronic and refractory ITP in 459 patients with primary ITP who were followed up in our hospital from January 2005 to December 2014 were reviewed. Fifteen of patients received rituximab and/or applied splenectomy. Fifteen chronic ITP patients (10 boys, five girls) with a mean age of 10 years were enrolled in the study. Two of these patients were suffering from Evans syndrome. The median time since diagnosis of ITP was 10 years. The median follow-up duration after starting Rituximab and splenectomy were 13 and 9.5 months, respectively.None of the seven patients who were treated with rituximab achieved a response. A splenectomy was performed in six of the seven patients who had been treated with rituximab. Complete and partial responses were achieved in 67 and 33% of the patients, respectively. We evaluated the clinical characteristics and responses of chronic ITP patients who did not receive rituximab therapy and underwent a splenectomy. The success rate was 100% in the eight patients with chronic and refractory ITP. Rituximab therapy might not be beneficial for some children with severe chronic ITP who are refractory to standard agents. A splenectomy might be useful and preferable to rituximab.

  7. Emergency Surgery for Refractory Status Epilepticus.

    Science.gov (United States)

    Botre, Abhijeet; Udani, Vrajesh; Desai, Neelu; Jagadish, Spoorthy; Sankhe, Milind

    2017-08-15

    Management of refractory status epilepticus in children is extremely challenging. Two children with medically refractory status epilepticus, both of whom had lesional pathology on MRI and concordant data on EEG and PET scan. Emergency hemispherotomy performed in both patients. A complete, sustained seizure freedom obtained postoperatively. Emergency surgery is a treatment option in selected cases of drug refractory status epilepticus with lesional pathology and concordant data.

  8. Updates in Refractory Status Epilepticus

    Science.gov (United States)

    Mahulikar, Advait; Suchdev, Kushak; Shah, Aashit

    2018-01-01

    Refractory status epilepticus is defined as persistent seizures despite appropriate use of two intravenous medications, one of which is a benzodiazepine. It can be seen in up to 40% of cases of status epilepticus with an acute symptomatic etiology as the most likely cause. New-onset refractory status epilepticus (NORSE) is a recently coined term for refractory status epilepticus where no apparent cause is found after initial testing. A large proportion of NORSE cases are eventually found to have an autoimmune etiology needing immunomodulatory treatment. Management of refractory status epilepticus involves treatment of an underlying etiology in addition to intravenous anesthetics and antiepileptic drugs. Alternative treatment options including diet therapies, electroconvulsive therapy, and surgical resection in case of a focal lesion should be considered. Short-term and long-term outcomes tend to be poor with significant morbidity and mortality with only one-third of patients reaching baseline neurological status. PMID:29854452

  9. Sleeping over a sleep disorder - Awareness of obstructive sleep apnoea as a modifiable risk factor for hypertension and stroke: A survey among health care professionals and medical students

    Directory of Open Access Journals (Sweden)

    Sushma Sharma

    2013-01-01

    Full Text Available Background: Obstructive sleep apnoea (OSA syndrome is an established and modifiable but under recognized risk factor for common disorders like stroke and hypertension. Objective: To assess awareness level of health care practitioners and medical students about OSA as a risk factor for stroke and hypertension. Methods: Questionnaire based survey with multiple response type and fill in the blanks type questions. The data was compiled and analyzed using SPSS version 19. Results: 180 participants completed the survey questionnaire. Only 24 (13.3% identified OSA as a reversible risk factor for ischemic stroke. 11 (6% participants only could answer OSA as an identified risk factor for hypertension as per Seventh Joint National Committee report. Poor awareness extended over all categories of participants (medical students, trained doctors and nursing staff . Conclusion: This study reveals dismal level of awareness, among health professionals and medical students, about OSA being an established and modifiable risk factor for hypertension and ischemic stroke.

  10. Cogan's syndrome: present and future directions.

    LENUS (Irish Health Repository)

    Murphy, Grainne

    2009-08-01

    Cogan\\'s syndrome, typified by the combination of interstitial keratitis and immune-mediated sensorineural hearing loss, is a rare condition, and commonly associated with a diagnostic delay. Using a standard search protocol, we review the literature to date, focusing on a number of key areas pertaining to diagnosis, presentation and treatment. Using a case illustration of atypical disease which led to fulminant aortic regurgitation, we highlight the need for continued and collaborative research in order to identify negative prognostic factors and thus tailor therapeutic regimens. Atypical Cogan\\'s syndrome is more commonly associated with systemic manifestations than typical disease, and may be refractory to immunosuppressive treatment. We discuss the application of laboratory (e.g antibodies targeting inner ear antigens) and radiological (PET-CT) aids to disease confirmation and detection of sub-clinical vascular inflammation. As illustrated by the included case description, some patients remain refractory to intense immunosuppression and delineation of adverse prognostic factors which may direct treatment, perhaps including the use of PET-CT, will contribute in the future to improving patient outcomes.

  11. Progressive anticonvulsant hypersensitivity syndrome associated with change of drug product

    DEFF Research Database (Denmark)

    Sabroe, T.P.; Sabers, A.

    2008-01-01

    This report describes the laboratory and physical manifestations of lamotrigine-like toxicity in a young man with refractory epilepsy receiving lamotrigine presenting as anticonvulsant hypersensitivity syndrome (AHS) associated with an abrupt change of drug product Udgivelsesdato: 2008/6...

  12. Refractory status epilepticus

    Directory of Open Access Journals (Sweden)

    Sanjay P Singh

    2014-01-01

    Full Text Available Refractory status epilepticus is a potentially life-threatening medical emergency. It requires early diagnosis and treatment. There is a lack of consensus upon its semantic definition of whether it is status epilepticus that continues despite treatment with benzodiazepine and one antiepileptic medication (AED, i.e., Lorazepam + phenytoin. Others regard refractory status epilepticus as failure of benzodiazepine and 2 antiepileptic medications, i.e., Lorazepam + phenytoin + phenobarb. Up to 30% patients in SE fail to respond to two antiepileptic drugs (AEDs and 15% continue to have seizure activity despite use of three drugs. Mechanisms that have made the treatment even more challenging are GABA-R that is internalized during status epilepticus and upregulation of multidrug transporter proteins. All patients of refractory status epilepticus require continuous EEG monitoring. There are three main agents used in the treatment of RSE. These include pentobarbital or thiopental, midazolam and propofol. RSE was shown to result in mortality in 35% cases, 39.13% of patients were left with severe neurological deficits, while another 13% had mild neurological deficits.

  13. Refractory status epilepticus

    Science.gov (United States)

    Singh, Sanjay P; Agarwal, Shubhi; Faulkner, M

    2014-01-01

    Refractory status epilepticus is a potentially life-threatening medical emergency. It requires early diagnosis and treatment. There is a lack of consensus upon its semantic definition of whether it is status epilepticus that continues despite treatment with benzodiazepine and one antiepileptic medication (AED), i.e., Lorazepam + phenytoin. Others regard refractory status epilepticus as failure of benzodiazepine and 2 antiepileptic medications, i.e., Lorazepam + phenytoin + phenobarb. Up to 30% patients in SE fail to respond to two antiepileptic drugs (AEDs) and 15% continue to have seizure activity despite use of three drugs. Mechanisms that have made the treatment even more challenging are GABA-R that is internalized during status epilepticus and upregulation of multidrug transporter proteins. All patients of refractory status epilepticus require continuous EEG monitoring. There are three main agents used in the treatment of RSE. These include pentobarbital or thiopental, midazolam and propofol. RSE was shown to result in mortality in 35% cases, 39.13% of patients were left with severe neurological deficits, while another 13% had mild neurological deficits. PMID:24791086

  14. Development of refractory concrete for extreme conditions

    International Nuclear Information System (INIS)

    Pundiene, I; Antonovich, V; Stonys, R; Demidova-Buiziniene, I

    2011-01-01

    Comparative analysis is provided for the properties of medium-cement refractory concrete with microsilica based on mullite filler in relation to different type of deflocculant. The effect of different deflocculants on refractory concrete structure formation, hydration, rheology, strength and heat resistance is discussed. Corrosion resistance test, determined that samples with hybrid deflocculant showed better resistance for slag penetration than samples with only the sodium tripolyphosphate or polycarboxylate ether deflocculant. Moreover, a composition of hybrid deflocculant let to control the rate of the hydration process and to get features of refractory refractory concrete.

  15. Cholesterol Metabolism and Weight Reduction in Subjects with Mild Obstructive Sleep Apnoea: A Randomised, Controlled Study

    Directory of Open Access Journals (Sweden)

    Maarit Hallikainen

    2013-01-01

    Full Text Available To evaluate whether parameters of obstructive sleep apnoea (OSA associate with cholesterol metabolism before and after weight reduction, 42 middle-aged overweight subjects with mild OSA were randomised to intensive lifestyle intervention (N=23 or to control group (N=18 with routine lifestyle counselling only. Cholesterol metabolism was evaluated with serum noncholesterol sterol ratios to cholesterol, surrogate markers of cholesterol absorption (cholestanol and plant sterols and synthesis (cholestenol, desmosterol, and lathosterol at baseline and after 1-year intervention. At baseline, arterial oxygen saturation (SaO2 was associated with serum campesterol (P<0.05 and inversely with desmosterol ratios (P<0.001 independently of gender, BMI, and homeostasis model assessment index of insulin resistance (HOMA-IR. Apnoea-hypopnoea index (AHI was not associated with cholesterol metabolism. Weight reduction significantly increased SaO2and serum cholestanol and decreased AHI and serum cholestenol ratios. In the groups combined, the changes in AHI were inversely associated with changes of cholestanol and positively with cholestenol ratios independent of gender and the changes of BMI and HOMA-IR (P<0.05. In conclusion, mild OSA seemed to be associated with cholesterol metabolism independent of BMI and HOMA-IR. Weight reduction increased the markers of cholesterol absorption and decreased those of cholesterol synthesis in the overweight subjects with mild OSA.

  16. High-Density Infrared Surface Treatments of Refractories

    Energy Technology Data Exchange (ETDEWEB)

    Tiegs, T.N.

    2005-03-31

    Refractory materials play a crucial role in all energy-intensive industries and are truly a crosscutting technology for the Industries of the Future (IOF). One of the major mechanisms for the degradation of refractories and a general decrease in their performance has been the penetration and corrosion by molten metals or glass. Methods and materials that would reduce the penetration, wetting, and corrosive chemistry would significantly improve refractory performance and also maintain the quality of the processed liquid, be it metal or glass. This report presents the results of an R&D project aimed at investigating the use of high-density infrared (HDI) heating to surface treat refractories to improve their performance. The project was a joint effort between Oak Ridge National Laboratory (ORNL) and the University of Missouri-Rolla (UMR). HDI is capable of heating the near-surface region of materials to very high temperatures where sintering, diffusion, and melting can occur. The intended benefits of HDI processing of refractories were to (1) reduce surface porosity (by essentially sealing the surface to prevent liquid penetration), (2) allow surface chemistry changes to be performed by bonding an adherent coating onto the underlying refractory (in order to inhibit wetting and/or improve corrosion resistance), and (3) produce noncontact refractories with high-emissivity surface coatings.

  17. Hepatorenal Syndrome

    Directory of Open Access Journals (Sweden)

    Ebru Yilmaz

    2014-06-01

    Full Text Available Hepatorenal syndrome (HRS is functional renal failure that occurs with advanced liver failure. HRS is considered the most severe complication of cirrhosis. Type 1 HRS develops due to severe reduction of effective circulating volume results in hemodynamic dysfunction. Type 1 HRS is characterized by acute renal failure and rapid deterioration in the function of other organs. It can ocur spontaneously or in the setting of a precipitating event. Type 2 hepatorenal syndrome (HRS, which is characterized by slowly progressive renal failure and refractory ascites. Liver transplantation is the only definitive treatment for both type. The most suitable and ldquo;bridge treatments and rdquo; or treatment for patients ineligible for a liver transplant include terlipressin plus albumin. [J Contemp Med 2014; 4(2.000: 106-113

  18. Adaptive servo ventilation for central sleep apnoea in heart failure: SERVE-HF on-treatment analysis.

    Science.gov (United States)

    Woehrle, Holger; Cowie, Martin R; Eulenburg, Christine; Suling, Anna; Angermann, Christiane; d'Ortho, Marie-Pia; Erdmann, Erland; Levy, Patrick; Simonds, Anita K; Somers, Virend K; Zannad, Faiez; Teschler, Helmut; Wegscheider, Karl

    2017-08-01

    This on-treatment analysis was conducted to facilitate understanding of mechanisms underlying the increased risk of all-cause and cardiovascular mortality in heart failure patients with reduced ejection fraction and predominant central sleep apnoea randomised to adaptive servo ventilation versus the control group in the SERVE-HF trial.Time-dependent on-treatment analyses were conducted (unadjusted and adjusted for predictive covariates). A comprehensive, time-dependent model was developed to correct for asymmetric selection effects (to minimise bias).The comprehensive model showed increased cardiovascular death hazard ratios during adaptive servo ventilation usage periods, slightly lower than those in the SERVE-HF intention-to-treat analysis. Self-selection bias was evident. Patients randomised to adaptive servo ventilation who crossed over to the control group were at higher risk of cardiovascular death than controls, while control patients with crossover to adaptive servo ventilation showed a trend towards lower risk of cardiovascular death than patients randomised to adaptive servo ventilation. Cardiovascular risk did not increase as nightly adaptive servo ventilation usage increased.On-treatment analysis showed similar results to the SERVE-HF intention-to-treat analysis, with an increased risk of cardiovascular death in heart failure with reduced ejection fraction patients with predominant central sleep apnoea treated with adaptive servo ventilation. Bias is inevitable and needs to be taken into account in any kind of on-treatment analysis in positive airway pressure studies. Copyright ©ERS 2017.

  19. Nelson Syndrome: Update on Therapeutic Approaches.

    Science.gov (United States)

    Azad, Tej D; Veeravagu, Anand; Kumar, Sunny; Katznelson, Laurence

    2015-06-01

    To review the pathophysiology and therapeutic modalities availble for Nelson syndrome. We reviewed the current literature including managment for Nelson syndrome. For patients with NS, surgical intervention is often the first-line therapy. With refractory NS or tumors with extrasellar involvement, radiosurgery offers an important alternative or adjuvant option. Pharmacologic interventions have demonstrated limited usefulness, although recent evidence supports the feasibility of a novel somatostatin analog for patients with NS. Modern neuroimaging, improved surgical techniques, and the advent of stereotactic radiotherapy have transformed the management of NS. An up-to-date understanding of the pathophysiology underlying Nelson Syndrome and evidence-based management is imperative. Early detection may allow for more successful therapy in patients with Nelson Syndrome. Improved radiotherapeutic interventions and rapidly evolving pharmacologic therapies offer an opportunity to create targeted, multifocal treatment regiments for patients with Nelson Syndrome. Copyright © 2015 Elsevier Inc. All rights reserved.

  20. Genetics Home Reference: iron-refractory iron deficiency anemia

    Science.gov (United States)

    ... refractory iron deficiency anemia Iron-refractory iron deficiency anemia Printable PDF Open All Close All Enable Javascript ... expand/collapse boxes. Description Iron-refractory iron deficiency anemia is one of many types of anemia , which ...

  1. Robust classification of neonatal apnoea-related desaturations

    International Nuclear Information System (INIS)

    Monasterio, Violeta; Burgess, Fred; Clifford, Gari D

    2012-01-01

    Respiratory signals monitored in the neonatal intensive care units are usually ignored due to the high prevalence of noise and false alarms (FA). Apneic events are generally therefore indicated by a pulse oximeter alarm reacting to the subsequent desaturation. However, the high FA rate in the photoplethysmogram may desensitize staff, reducing the reaction speed. The main reason for the high FA rates of critical care monitors is the unimodal analysis behaviour. In this work, we propose a multimodal analysis framework to reduce the FA rate in neonatal apnoea monitoring. Information about oxygen saturation, heart rate, respiratory rate and signal quality was extracted from electrocardiogram, impedance pneumogram and photoplethysmographic signals for a total of 20 features in the 5 min interval before a desaturation event. 1616 desaturation events from 27 neonatal admissions were annotated by two independent reviewers as true (physiologically relevant) or false (noise-related). Patients were divided into two independent groups for training and validation, and a support vector machine was trained to classify the events as true or false. The best classification performance was achieved on a combination of 13 features with sensitivity, specificity and accuracy of 100% in the training set, and a sensitivity of 86%, a specificity of 91% and an accuracy of 90% in the validation set. (paper)

  2. Refractory hypertension: definition, prevalence, and patient characteristics.

    Science.gov (United States)

    Acelajado, Maria Czarina; Pisoni, Roberto; Dudenbostel, Tanja; Dell'Italia, Louis J; Cartmill, Falynn; Zhang, Bin; Cofield, Stacey S; Oparil, Suzanne; Calhoun, David A

    2012-01-01

    Among patients with resistant hypertension (RHTN), there are those whose blood pressure (BP) remains uncontrolled in spite of maximal medical therapy. This retrospective analysis aims to characterize these patients with refractory hypertension. Refractory hypertension was defined as BP that remained uncontrolled after ≥3 visits to a hypertension clinic within a minimum 6-month follow-up period. Of the 304 patients referred for RHTN, 29 (9.5%) remained refractory to treatment. Patients with refractory hypertension and those with controlled RHTN had similar aldosterone levels and plasma renin activity (PRA). Patients with refractory hypertension had higher baseline BP (175±23/97±15 mm Hg vs 158±25/89±15 mm Hg; P=.001/.005) and heart rate, and higher rates of prior stroke and congestive heart failure. During follow-up, the BP of patients with refractory hypertension remained uncontrolled (168.4±14.8/93.8±17.7 mm Hg) in spite of use of an average of 6 antihypertensive medications, while those of patients with controlled RHTN decreased to 129.3±11.2/77.6±10.8 mm Hg. Spironolactone reduced the BP by 12.9±17.8/6.6±13.7 mm Hg in patients with refractory hypertension and by 24.1±16.7/9.2±12.0 mm Hg in patients with controlled RHTN. In patients with RHTN, approximately 10% remain refractory to treatment. Similar aldosterone and PRA levels and a diminished response to spironolactone suggest that aldosterone excess does not explain the treatment failure. © 2011 Wiley Periodicals, Inc.

  3. Comparing electromagnetic stimulation with electrostimulation plus biofeedback in treating male refractory chronic pelvic pain syndrome

    Directory of Open Access Journals (Sweden)

    Min-Hsin Yang

    2017-09-01

    Conclusion: Both EMS and ESB physical therapy of the pelvic floor muscle effectively reduce pain, increase the QoL, and improve urinary tract symptoms in male CPPS patients who are refractory to medical treatments. The combination therapy of ES plus biofeedback demonstrates additional benefits in pain and QoL when compared with EMS alone.

  4. Correlation of obstructive sleep apnoea and laryngopharyngeal reflux: phmetry study.

    Science.gov (United States)

    Elhennawi, D M; Ahmed, M R; Abou-Halawa, A S

    2016-12-01

    To study the correlation of obstructive sleep apnoea (OSA) and laryngopharyngeal reflux (LPR). A descriptive study. Suez Canal University Hospital, Ismailia, Egypt. 62 patients with polysomnography confirmed OSA. Patients were evaluated with ambulatory 24-h double channel pH monitoring. Mean reflux symptom index in the study group was 9 ± 5.5, and it was > 13 in all patients with severe OSA. Signs of LPR reflux were present in 34 (55%) patients. Abnormal reflux was detected in the distal oesophagus in 41 patients (66%) and in the proximal oesophagus in 21 patients (34%). Patients with severe OSA had significantly higher nocturnal LPR reflux episodes compared to patients with mild disease (P .05). LPR is common in patients with OSA. Patients with severe OSA have significantly higher nocturnal LPR. This should be considered when treating this group of patients. © 2016 John Wiley & Sons Ltd.

  5. Scleral lens use in dry eye syndrome.

    Science.gov (United States)

    Bavinger, J Clay; DeLoss, Karen; Mian, Shahzad I

    2015-07-01

    Dry eye syndrome can be difficult to manage in severe or refractory cases. In patients in whom traditional treatments have limited efficacy, alternative treatments may be considered for dry eye syndrome, including scleral lenses. The present review summarizes the evidence regarding scleral lens use in dry eye syndrome. Scleral lenses have become a viable option for severe dry eye syndrome, and have been shown to be efficacious and well tolerated, with most reports citing improved visual acuity and relief of symptoms. Currently, there are 18 manufacturers of scleral lenses, although published reports on scleral lenses primarily focus on the BostonSight PROSE and the Jupiter Lens. Scleral lenses are efficacious and well tolerated for use in severe dry eye syndrome. Further research is needed to compare different sizes and types of lenses, and to standardize outcome measures.

  6. Phase I/Phase II Study of Blinatumomab in Pediatric Patients With Relapsed/Refractory Acute Lymphoblastic Leukemia.

    Science.gov (United States)

    von Stackelberg, Arend; Locatelli, Franco; Zugmaier, Gerhard; Handgretinger, Rupert; Trippett, Tanya M; Rizzari, Carmelo; Bader, Peter; O'Brien, Maureen M; Brethon, Benoît; Bhojwani, Deepa; Schlegel, Paul Gerhardt; Borkhardt, Arndt; Rheingold, Susan R; Cooper, Todd Michael; Zwaan, Christian M; Barnette, Phillip; Messina, Chiara; Michel, Gérard; DuBois, Steven G; Hu, Kuolung; Zhu, Min; Whitlock, James A; Gore, Lia

    2016-12-20

    Purpose Blinatumomab is a bispecific T-cell engager antibody construct targeting CD19 on B-cell lymphoblasts. We evaluated the safety, pharmacokinetics, recommended dosage, and potential for efficacy of blinatumomab in children with relapsed/refractory B-cell precursor acute lymphoblastic leukemia (BCP-ALL). Methods This open-label study enrolled children treatment cycles. Primary end points were maximum-tolerated dosage (phase I) and complete remission rate within the first two cycles (phase II). Results We treated 49 patients in phase I and 44 patients in phase II. Four patients had dose-limiting toxicities in cycle 1 (phase I). Three experienced grade 4 cytokine-release syndrome (one attributed to grade 5 cardiac failure); one had fatal respiratory failure. The maximum-tolerated dosage was 15 µg/m 2 /d. Blinatumomab pharmacokinetics was linear across dosage levels and consistent among age groups. On the basis of the phase I data, the recommended blinatumomab dosage for children with relapsed/refractory ALL was 5 µg/m 2 /d for the first 7 days, followed by 15 µg/m 2 /d thereafter. Among the 70 patients who received the recommended dosage, 27 (39%; 95% CI, 27% to 51%) achieved complete remission within the first two cycles, 14 (52%) of whom achieved complete minimal residual disease response. The most frequent grade ≥ 3 adverse events were anemia (36%), thrombocytopenia (21%), and hypokalemia (17%). Three patients (4%) and one patient (1%) had cytokine-release syndrome of grade 3 and 4, respectively. Two patients (3%) interrupted treatment after grade 2 seizures. Conclusion This trial, which to the best of our knowledge was the first such trial in pediatrics, demonstrated antileukemic activity of single-agent blinatumomab with complete minimal residual disease response in children with relapsed/refractory BCP-ALL. Blinatumomab may represent an important new treatment option in this setting, requiring further investigation in curative indications.

  7. Refractories for Industrial Processing. Opportunities for Improved Energy Efficiency

    Energy Technology Data Exchange (ETDEWEB)

    Hemrick, James G. [Oak Ridge National Lab. (ORNL), Oak Ridge, TN (United States); Hayden, H. Wayne [Metals Manufacture Process and Controls Technology, Inc., Oak Ridge, TN (United States); Angelini, Peter [Oak Ridge National Lab. (ORNL), Oak Ridge, TN (United States); Moore, Robert E. [R.E. Moore Associates, Maricopa, AZ (United States); Headrick, William L. [R.E. Moore Associates, Maricopa, AZ (United States)

    2005-01-01

    Refractories are a class of materials of critical importance to manufacturing industries with high-temperature unit processes. This study describes industrial refractory applications and identifies refractory performance barriers to energy efficiency for processing. The report provides recommendations for R&D pathways leading to improved refractories for energy-efficient manufacturing and processing.

  8. Neurobehavioural correlates in older children and adolescents with obesity and obstructive sleep apnoea.

    Science.gov (United States)

    Tan, Evan; Healey, Dione; Schaughency, Elizabeth; Dawes, Patrick; Galland, Barbara

    2014-01-01

    The relationship between obstructive sleep apnoea (OSA) and poorer neurobehavioural outcomes in school-age children is well established, but the relationship in obese children and adolescents, in whom OSA is more common, is not so well established. We aimed to investigate this relationship in 10-18-year-olds. Thirty-one participants with a mean body mass index (BMI) of 32.3 ± 4.9 enrolled. BMI-for-age cut-offs were used to define obesity. Participants underwent polysomnography and were classified into OSA (apnoea-hypopnoea index (AHI) > 2 per hour) and non-OSA (AHI ≤ 2) groups. Intelligence, memory and learning, academic achievement, behaviour and executive functioning were assessed using the Wechsler Abbreviated Scale of Intelligence, Wide Range Assessment of Memory and Learning 2, Wechsler Individual Achievement Test II (WIAT-II), Behavioural Assessment System for Children 2 and Behaviour Rating Inventory of Executive Function, respectively. Forty-eight per cent (15/31) were classified as having OSA, and 52% (16/31) as non-OSA. The obese cohort performed below the average of normative data on several neurobehavioural measures. WIAT-II maths scores were significantly lower (P = 0.034) in the OSA group than in the non-OSA group (means 84.5 vs. 94.6, respectively), losing significance after adjustment for IQ, age and gender. Self-reported school problems were significantly worse in the OSA group before and after multivariate adjustment (P = 0.010, Cohen's d = 1.02). No other significant differences were found. Results suggest that OSA may increase risk for some poorer educational and behavioural outcomes. The findings are reasonably consistent with and add to the evidence base of the few studies that have explored this relationship. © 2013 The Authors. Journal of Paediatrics and Child Health © 2013 Paediatrics and Child Health Division (Royal Australasian College of Physicians).

  9. Comparison of Standard and Novel Signal Analysis Approaches to Obstructive Sleep Apnoea Classification

    Directory of Open Access Journals (Sweden)

    Aoife eRoebuck

    2015-08-01

    Full Text Available Obstructive sleep apnoea (OSA is a disorder characterised by repeated pauses in breathing during sleep, which leads to deoxygenation and voiced chokes at the end of each episode. OSA is associated by daytime sleepiness and an increased risk of serious conditions such as cardiovascular disease, diabetes and stroke. Between 2-7% of the adult population globally has OSA, but it is estimated that up to 90% of those are undiagnosed and untreated. Diagnosis of OSA requires expensive and cumbersome screening. Audio offers a potential non-contact alternative, particularly with the ubiquity of excellent signal processing on every phone.Previous studies have focused on the classification of snoring and apnoeic chokes. However, such approaches require accurate identification of events. This leads to limited accuracy and small study populations. In this work we propose an alternative approach which uses multiscale entropy (MSE coefficients presented to a classifier to identify disorder in vocal patterns indicative of sleep apnoea. A database of 858 patients was used, the largest reported in this domain. Apnoeic choke, snore, and noise events encoded with speech analysis features were input into a linear classifier. Coefficients of MSE derived from the first 4 hours of each recording were used to train and test a random forest to classify patients as apnoeic or not.Standard speech analysis approaches for event classification achieved an out of sample accuracy (Ac of 76.9% with a sensitivity (Se of 29.2% and a specificity (Sp of 88.7% but high variance. For OSA severity classification, MSE provided an out of sample Ac of 79.9%, Se of 66.0% and Sp = 88.8%. Including demographic information improved the MSE-based classification performance to Ac = 80.5%, Se = 69.2%, Sp = 87.9%. These results indicate that audio recordings could be used in screening for OSA, but are generally under-sensitive.

  10. Globus Pallidus Interna Deep Brain Stimulation in a Patient with Medically Intractable Meige Syndrome

    Directory of Open Access Journals (Sweden)

    Dae-Woong Bae

    2014-10-01

    Full Text Available Medical therapies in patients with Meige syndrome, including botulinum toxin injection, have been limited because of incomplete response or adverse side effects. We evaluated a patient with Meige syndrome who was successfully treated with deep brain stimulation (DBS in the globus pallidus interna (GPi. This case report and other previous reports suggest that bilateral GPi DBS may be an effective treatment for medically refractory Meige syndrome, without significant adverse effects.

  11. Hirschsprung's Disease-Related Giant Sigmoid Volvulus Complicated by Refractory Hypertension in an Elderly Man.

    Science.gov (United States)

    Wu, Shaohan; Sun, Xiaofang; Yu, Yawei; Shen, Yiyu

    2018-04-20

    BACKGROUND Sigmoid volvulus (SV) is a life-threatening condition occasionally seen in adults. Adult Hirschsprung's disease (HD)-related SV is rarely complicated by difficult-to-control hypertension. In this report we present the case of an elderly man with a rare constellation of HD, SV, and refractory hypertension. CASE REPORT An 82-year-old man had long-term constipation, moderate abdominal pain, and progressive abdominal distension. A CT scan revealed the typical "coffee bean sign". Blood pressure was abnormal high. Subsequently, the patient's condition deteriorated. Therefore, he underwent a Hartmann's procedure. A giant and redundant sigmoid colon (length more than 60 cm, maximal diameter about 15 cm) was demonstrated to be the cause of SV during the process of surgery. Moreover, abdominal compartment syndrome caused by SV resulted in his high and refractory blood pressure (BP). Postoperative pathological results revealed HD in his sigmoid colon. CONCLUSIONS SV is rarely combined with conditions like refractory hypertension or HD among the elderly. Clinical features of SV typically present with long-term constipation, severe abdominal pain, and progressive abdominal distension. The "coffee bean sign" could be observed in imaging examinations. It is important to note that the management of SV is to relieve the obstruction and prevent recurrence, no matter which therapy is used in elderly patients with Hirschsprung's disease.

  12. Successful treatment of myelodysplastic syndrome-induced pyoderma gangrenosum.

    Science.gov (United States)

    Koca, E; Duman, A E; Cetiner, D; Buyukasik, Y; Haznedaroglu, I C; Uner, A; Demirhan, B; Kerimoglu, U; Barista, I; Calguneri, M; Ozcebe, O I

    2006-12-01

    We report successful treatment of a refractory myelodysplastic syndrome-associated pyoderma gangrenosum with the combination of thalidomide and interferon-alpha2a in a single patient. A non-healing wound developed on a 40-year-old woman's left thumb after minor trauma. Massive ulcerovegetative lesions developed after reconstruction surgery. Histopathological examination of the bone marrow and cytogenetic studies revealed an atypical myeloproliferative/myelodysplastic syndrome. The skin lesions resolved dramatically after two months of thalidomide and interferon-alpha2a combination therapy and the haematological status improved.

  13. Complex Regional Pain Syndrome and Treatment Approaches

    Directory of Open Access Journals (Sweden)

    Neslihan Gokcen

    2013-08-01

    Full Text Available Complex Regional Pain Syndrome is a symptom complex including severe pain which is disproportioned by the initiating event. Formerly, it was known as reflex sympathetic dystropy, Sudeck’s atrophy and algoneurodystrophy. There are two types of complex regional pain syndrome (CPRS. CRPS type 1 (Reflex sympathetic dystropy occurs after a minor trauma of the extremities, CRPS type 2 (Causalgia occurs following peripheral nevre injury. Diagnosis is made according to the history, symptoms and physical findings of the patients. Patient education, physical therapy and medical treatment are the most common treatment approaches of complex regional pain syndrome. The aim of this review is to revise the treatment options ofcomplex regional pain syndrome, as well as to overview the new treatment approaches and options for the refractory complex regional pain syndrome cases. [Archives Medical Review Journal 2013; 22(4.000: 514-531

  14. Novel Surgical Treatment for Refractory Heel Ulcers in Werner’s Syndrome

    Directory of Open Access Journals (Sweden)

    Keisuke Oe

    2013-01-01

    Full Text Available Patients with Werner’s syndrome frequently develop chronic leg ulcers that heal poorly. We present a patient who suffered from this rare syndrome and developed typical heel ulcers. Treatment of the ulcer is challenging, as flap options are limited over the lower third of the leg and skin grafting is not easy as there is a lack of healthy granulations. We successfully treated the ulcer with osteomyelitis by drilling the bone and applying an ultrathin split thickness skin graft with the thigh skin as the donor site.

  15. Social consequences of sleep disordered breathing on patients and their partners

    DEFF Research Database (Denmark)

    Jennum, Poul; Ibsen, Rikke Falkner; Kjellberg, Jakob

    2014-01-01

    We aimed to evaluate the total costs to patients and their partners of sleep apnoea and obesity hypoventilation syndrome (OHS) and their treatment, as this is poorly described in families. Using data from the Danish National Patient Registry and other public databases, all patients and their part......, and increased as the disease advanced. Sleep-disordered breathing has major socioeconomic consequences for patients and their spouses years before and after diagnosis.......We aimed to evaluate the total costs to patients and their partners of sleep apnoea and obesity hypoventilation syndrome (OHS) and their treatment, as this is poorly described in families. Using data from the Danish National Patient Registry and other public databases, all patients...... and their partners with a diagnosis of sleep apnoea (n=30,278) or OHS (n=1562) were included. They were compared with age-, sex- and community location-matched citizens at a ratio 1:4 (120,506 and 6241 control subjects, respectively). Direct and indirect costs were evaluated for patients and their partners. Sleep...

  16. Tungsten and refractory metals 3, proceedings

    International Nuclear Information System (INIS)

    Bose, A.; Dowding, R.J.

    1996-01-01

    The Third International Conference on Tungsten and Refractory Metals was held in Greater Washington DC at the McLean Hilton, McLean Virginia, on November 15--16, 1995. This meeting was the third in a series of conferences held in the Washington DC area. The first meeting was in 1992 and was entitled ''International Conference on Tungsten and Tungsten Alloys.'' In 1994, the scope of the meeting was expanded to include other refractory metals such as molybdenum, iridium, rhenium, tantalum and niobium. The tremendous success of that meeting was the primary motivation for this Conference. The broader scope (the inclusion of other refractory metals and alloys) of the Conference was kept intact for this meeting. In fact, it was felt that the developments in the technology of these materials required a common forum for the interchange of current research information. The papers presented in this meeting examined the rapid advancements in the technology of refractory metals, with special emphasis on the processing, structure, and properties. Among the properties there was emphasis on both quasi-static and dynamic rates. Another topic that received considerable interest was the area of refractory carbides and tungsten-copper composites. One day of concurrent session was necessary to accommodate all of the presentations

  17. Potential refractory alloy requirements for space nuclear power applications

    International Nuclear Information System (INIS)

    Cooper, R.H. Jr.

    1984-01-01

    In reviewing design requirements for refractory alloys for space nuclear applications, several key points are identified. First, the successful utilization of refractory alloys is considered an enabling requirement for the successful deployment of high efficiency, lightweight, and small space nuclear systems. Second, the recapture of refractory alloy nuclear technology developed in the 1960s and early 1970s appears to be a pacing activity in the successful utilization of refractory alloys. Third, the successful application of refractory alloys for space nuclear applications will present a significant challenge to both the materials and the systems design communities

  18. Refractories for steel-works

    International Nuclear Information System (INIS)

    Villanova, R.A.; Galant, C.L.; Haas, C.; Rosenbaum, V.

    The routine procedures utilized for quality control of refractory materials used by PIRATINI's steel-works, are presented ' under an objetive and practical maner. The attention of the paper is concentrated upon the following' refractory types with higher consume: silicon-aluminous; aluminous; basic magnesia; basic chrom-magnesia. All steps of utilization are described, including specification, supplies programation, storage; sampling; physical tests, and also aplication procedures. Results from routine analysis during a six month period, by ' means of X-Ray Quantometry, using the fusion pearls procedure, are presented compared with Atomic Absorption [pt

  19. Development of AZS refractories for the glass industry

    International Nuclear Information System (INIS)

    Guzman, A.M.; Rodriguez, P.

    2004-01-01

    Refractory materials can support high temperatures, thermal strength and the contact with aggressive environments, for this reason they are widely used in the cement, glass and steel industry. Commercial AZS (alumina-zirconia-silica) refractories are a good alternative in refractory materials for the glass industry' because they can support the aggressive conditions during liquid processing of glass. However, another problem encountered in glass industry is contamination by refractory' material that fall into the molten glass, which can produce a series of defects in the final product. This research was conducted to develop new formulations of AZS refractories with different amounts of ZrO 2 with the purpose of improving the characteristics, properties and the work conditions in the glass melting furnaces and, at the same time, lower the costs this type of refractories. The results obtained indicate that the composition with low content of ZrO 2 can provide better properties than the commercial product, with some modifications in the particle size distribution. Copyright (2004) AD-TECH - International Foundation for the Advancement of Technology Ltd

  20. Total artificial heart implantation in a young Marfan syndrome patient.

    Science.gov (United States)

    Rao, Prashant; Keenan, Jack B; Rajab, Taufiek K; Kim, Samuel; Smith, Richard; Amabile, Orazio; Khalpey, Zain

    2018-03-01

    Cardiovascular complications represent the leading cause of morbidity and mortality in patients with Marfan syndrome. Here, we describe a unique case where a total artificial heart was implanted in a young Marfan syndrome woman. A 22-year-old postpartum African American female with Marfan syndrome developed multiple severe valve dysfunction and biventricular failure that was refractory to medical management. She previously had a Bentall procedure for Type A aortic dissection and repair of a Type B dissection. We implanted a total artificial heart with a good outcome. Total artificial heart is a durable option for severe biventricular failure and multiple valvular dysfunction as a bridge to transplant in a young patient with Marfan syndrome.

  1. Treatment outcomes of obstructive sleep apnoea in obese community-dwelling children: the NANOS study.

    Science.gov (United States)

    Alonso-Álvarez, María Luz; Terán-Santos, Joaquin; Navazo-Egüia, Ana Isabel; Martinez, Mónica Gonzalez; Jurado-Luque, María José; Corral-Peñafiel, Jaime; Duran-Cantolla, Joaquin; Cordero-Guevara, José Aurelio; Kheirandish-Gozal, Leila; Gozal, David

    2015-09-01

    The first line of treatment of obstructive sleep apnoea syndrome (OSAS) in children consists of adenotonsillectomy (T&A). The aim of the present study was to evaluate treatment outcomes of OSAS among obese children recruited from the community.A cross-sectional, prospective, multicentre study of Spanish obese children aged 3-14 years, with four groups available for follow-up: group 1: non-OSAS with no treatment; group 2: dietary treatment; group 3: surgical treatment; and group 4: continuous positive airway pressure treatment.117 obese children (60 boys, 57 girls) with a mean age of 11.3±2.9 years completed the initial (T0) and follow-up (T1) assessments. Their mean body mass index (BMI) at T1 was 27.6±4.7 kg·m(-2), corresponding to a BMI Z-score of 1.34±0.59. Mean respiratory disturbance index (RDI) at follow-up was 3.3±3.9 events·h(-1). Among group 1 children, 21.2% had an RDI ≥3 events·h(-1) at T1, the latter being present in 50% of group 2, and 43.5% in group 3. In the binary logistic regression model, age emerged as a significant risk factor for residual OSAS (odds ratio 1.49, 95% confidence interval 1.01-2.23; p<0.05) in obese children surgically treated, and RDI at T0 as well as an increase in BMI emerged as significant risk factors for persistent OSAS in obese children with dietary treatment (OR 1.82, 95% CI 1.09-3.02 (p<0.03) and OR 8.71, 95% CI 1.24-61.17 (p=0.03)).Age, RDI at diagnosis and obesity are risk factors for relatively unfavourable OSAS treatment outcomes at follow-up. Copyright ©ERS 2015.

  2. Clinical phenotype of South-East Asian temporomandibular disorder patients with upper airway resistance syndrome.

    Science.gov (United States)

    Tay, D K L; Pang, K P

    2018-01-01

    Clinical and radiographic characteristics of a subset of South East Asian temporomandibular disorder (TMD) patients with comorbid upper airway resistance syndrome (UARS) were documented in a multi-center prospective series of 86 patients (26 men and 60 women / mean age 35.7 years). All had excessive daytime sleepiness, high arousal index and Apnoea-Hypopnoea Index (AHI) temporomandibular joint (TMJ) arthralgia while 90·7% reported sleep bruxism (SB). Unlike patients with obstructive sleep apnoea (OSA), hypertension was uncommon (4·7%) while depression was prevalent at 68·6% with short REM latency of 25% documented in 79·6% and 57·6% of these depressed patients, respectively. 65·1% displayed a posteriorly displaced condyle at maximum intercuspation with or without TMJ clicking. Most exhibited a forward head posture (FHP) characterised by loss of normal cervical lordosis (80·2%), C0-C1 narrowing (38·4%) or an elevated hyoid position (50%), and 91·9% had nasal congestion. We postulate the TMD-UARS phenotype may have originally developed as an adaptive response to 'awake' disordered breathing during growth. Patients with persistent TMD and/or reporting SB should be screened for UARS and chronic nasal obstruction, especially when they also present with FHP. The lateral cephalogram is a useful tool in the differentiation of UARS from other OSA phenotypes. © 2017 John Wiley & Sons Ltd.

  3. Granulomatous slack skin syndrome: Report of a unique case.

    Science.gov (United States)

    Maheswari, S Uma; Sampath, V; Ramesh, A

    2018-01-01

    Granulomatous slack skin syndrome is a rare variant of cutaneous T-cell lymphoma (mycosis fungoides). It is characterized clinically by redundant skin folds, which show a predilection towards flexural areas such as the axilla and the groin. Histologically, it shows a granulomatous T-cell infiltrate and loss of elastic tissue. It has an indolent but progressive course; and is usually refractory to treatment. We report a unique case of slack skin syndrome, sparing the classical sites with rapid and unusual involvement of non-intertriginous areas.

  4. Refractory migraine in a headache clinic population

    Directory of Open Access Journals (Sweden)

    Fernandez-Torron Roberto

    2011-08-01

    Full Text Available Abstract Background Many migraineurs who seek care in headache clinics are refractory to treatment, despite advances in headache therapies. Epidemiology is poorly characterized, because diagnostic criteria for refractory migraine were not available until recently. We aimed to determine the frequency of refractory migraine in patients attended in the Headache Unit in a tertiary care center, according to recently proposed criteria. Methods The study population consisted of a consecutive sample of 370 patients (60.8% females with a mean age of 43 years (range 14-86 evaluated for the first time in our headache unit over a one-year period (between October 2008 and October 2009. We recorded information on clinical features, previous treatments, Migraine Disability Assessment Score (MIDAS, and final diagnosis. Results Overall migraine and tension-type headache were found in 46.4% and 20.5% of patients, respectively. Refractory migraine was found in 5.1% of patients. In refractory migraineurs, the mean MIDAS score was 96, and 36.8% were medication-overusers. Conclusions Refractory migraine is a relatively common and very disabling condition between the patients attended in a headache unit. The proposed operational criteria may be useful in identifying those patients who require care in headache units, the selection of candidates for combinations of prophylactic drugs or invasive treatments such as neurostimulation, but also to facilitate clinical studies in this patient group.

  5. Surgical fasciectomy of the trapezius muscle combined with neurolysis of the Spinal accessory nerve; results and long-term follow-up in 30 consecutive cases of refractory chronic whiplash syndrome

    Directory of Open Access Journals (Sweden)

    Freeman Michael

    2010-04-01

    Full Text Available Abstract Background Chronic problems from whiplash trauma generally include headache, pain and neck stiffness that may prove refractory to conservative treatment modalities. As has previously been reported, such afflicted patients may experience significant temporary relief with injections of local anesthetic to painful trigger points in muscles of the shoulder and neck, or lasting symptomatic improvement through surgical excision of myofascial trigger points. In a subset of patients who present with chronic whiplash syndrome, the clinical findings suggest an affliction of the spinal accessory nerve (CN XI, SAN by entrapment under the fascia of the trapezius muscle. The present study was undertaken to assess the effectiveness of SAN neurolysis in chronic whiplash syndrome. Methods A standardized questionnaire and a linear visual-analogue scale graded 0-10 was used to assess disability related to five symptoms (pain, headache, insomnia, weakness, and stiffness before, and one year after surgery in a series of thirty consecutive patients. Results The preoperative duration of symptoms ranged from seven months to 13 years. The following changes in disability scores were documented one year after surgery: Overall pain decreased from 9.5 +/- 0.9 to 3.2 +/- 2.6 (p Conclusions Entrapment of the spinal accessory nerve and/or chronic compartment syndrome of the trapezius muscle may cause chronic debilitating pain after whiplash trauma, without radiological or electrodiagnostic evidence of injury. In such cases, surgical treatment may provide lasting relief.

  6. NOvel Refractory Materials for High Alkali, High Temperature Environments

    Energy Technology Data Exchange (ETDEWEB)

    Hemrick, J.G.; Griffin, R. (MINTEQ International, Inc.)

    2011-08-30

    Refractory materials can be limited in their application by many factors including chemical reactions between the service environment and the refractory material, mechanical degradation of the refractory material by the service environment, temperature limitations on the use of a particular refractory material, and the inability to install or repair the refractory material in a cost effective manner or while the vessel was in service. The objective of this project was to address the need for new innovative refractory compositions by developing a family of novel MgO-Al2O3 spinel or other similar magnesia/alumina containing unshaped refractory composition (castables, gunnables, shotcretes, etc) utilizing new aggregate materials, bond systems, protective coatings, and phase formation techniques (in-situ phase formation, altered conversion temperatures, accelerated reactions, etc). This family of refractory compositions would then be tailored for use in high-temperature, highalkaline industrial environments like those found in the aluminum, chemical, forest products, glass, and steel industries. A research team was formed to carry out the proposed work led by Oak Ridge National Laboratory (ORNL) and was comprised of the academic institution Missouri University of Science and Technology (MS&T), and the industrial company MINTEQ International, Inc. (MINTEQ), along with representatives from the aluminum, chemical, glass, and forest products industries. The two goals of this project were to produce novel refractory compositions which will allow for improved energy efficiency and to develop new refractory application techniques which would improve the speed of installation. Also methods of hot installation were sought which would allow for hot repairs and on-line maintenance leading to reduced process downtimes and eliminating the need to cool and reheat process vessels.

  7. SINTERED REFRACTORY TUNGSTEN ALLOYS. Gesinterte hochschmelzende wolframlegierungen

    Energy Technology Data Exchange (ETDEWEB)

    Kieffer, R.; Sedlatschek, K.; Braun, H.

    1971-12-15

    Dependence of the melting point of the refractory metals on their positions in the periodic system - alloys of tungsten with other refractory metals - sintering of the alloys - processing of the alloys - technological properties.

  8. Stochastic histories of refractory interstellar dust

    International Nuclear Information System (INIS)

    Liffman, K.; Chayton, D.D.

    1988-01-01

    The authors calculate histories for refractory dust particles in the interstellar medium. The double purposes are to learn something of the properties of interstellar dust as a system and to evaluate with specific assumptions the cosmic chemical memory interpretation of a specific class of isotopic anomalies. They assemble the profile of a particle population from a large number of stochastic, or Monte Carlo, histories of single particles, which are necessarily taken to be independent with this approach. They specify probabilities for each of the events that may befall a given particle and unfold its history by a sequence of random numbers. They assume that refractory particles are created only by thermal condensation within stellar material during its ejection from stars, and that these refractory particles can be destroyed only by being sputtered to a size too small for stability or by being incorporated into the formation of new stars. In order to record chemical detail, the authors take each new refractory particle to consist of a superrefractory core plus a more massive refractory mantle. They demonstrate that these superrefractory cores have effective lifetimes much longer than the turnover time of dust mass against sputtering. As examples of cosmic chemical memory they evaluate the 16 O-richness of interstellar aluminum and mechanisms for the 48 Ca/ 50 Ti correlation. Several related consequences of this approach are discussed

  9. Progressive transfusion and growth factor independence with adjuvant sertraline in low risk myelodysplastic syndrome treated with an erythropoiesis stimulating agent and granulocyte-colony stimulating factor

    Directory of Open Access Journals (Sweden)

    Kirtan Nautiyal

    2015-01-01

    Full Text Available Refractoriness to growth factor therapy is commonly associated with inferior outcome in patients with low-risk myelodysplastic syndrome (LR-MDS who require treatment for cytopenias. However, the mechanisms leading to refractoriness are unknown. Here we describe a clinically depressed 74-year-old male with refractory cytopenia with multilineage dysplasia (RCMD and documented growth factor refractory anemia after erythropoeisis stimulating agent (ESA therapy, who attained transfusion and growth factor independence after the addition of sertraline to his medication regimen. Our case demonstrates hematological improvement-erythroid (HI-E in growth factor refractory, low risk MDS and highlights a potential mechanistic link between common inflammatory diseases and LR-MDS.

  10. Interaction of obstructive sleep apnoea and cognitive impairment with slow gait speed in middle-aged and older adults.

    Science.gov (United States)

    Lee, Sunghee; Shin, Chol

    2017-07-01

    to investigate whether slow gait speed is associated with cognitive impairment and further whether the association is modified by obstructive sleep apnoea (OSA). in total, 2,222 adults aged 49-80 years, free from dementia, stroke and head injury were asked to walk a 4-m course at fast and usual gait speeds. The time taken to walk was measured. All participants completed the Korean Mini-Mental State Examination, which was validated in the Korean language, to assess cognitive function. Additionally, the participants completed a polysomnography test to ascertain OSA (defined as an apnoea-hypopnoea index ≥15). Multivariable linear regression models were utilised to test the associations. time taken to walk 4 m showed significant inverse associations with cognitive scores (P value = 0.001 at fast gait speed and P = 0.002 at usual gait speed). Furthermore, a significant interaction according to OSA on the association between time to walk and cognitive impairment was found (P value for interaction = 0.003 at fast gait speed and P value for interaction = 0.007 at usual gait speed). we found that the inverse association between the time taken to walk 4 m and a cognitive score became significantly stronger, if an individual had OSA. © The Author 2017. Published by Oxford University Press on behalf of the British Geriatrics Society. All rights reserved. For Permissions, please email: journals.permissions@oup.com

  11. Response properties of the refractory auditory nerve fiber.

    Science.gov (United States)

    Miller, C A; Abbas, P J; Robinson, B K

    2001-09-01

    The refractory characteristics of auditory nerve fibers limit their ability to accurately encode temporal information. Therefore, they are relevant to the design of cochlear prostheses. It is also possible that the refractory property could be exploited by prosthetic devices to improve information transfer, as refractoriness may enhance the nerve's stochastic properties. Furthermore, refractory data are needed for the development of accurate computational models of auditory nerve fibers. We applied a two-pulse forward-masking paradigm to a feline model of the human auditory nerve to assess refractory properties of single fibers. Each fiber was driven to refractoriness by a single (masker) current pulse delivered intracochlearly. Properties of firing efficiency, latency, jitter, spike amplitude, and relative spread (a measure of dynamic range and stochasticity) were examined by exciting fibers with a second (probe) pulse and systematically varying the masker-probe interval (MPI). Responses to monophasic cathodic current pulses were analyzed. We estimated the mean absolute refractory period to be about 330 micros and the mean recovery time constant to be about 410 micros. A significant proportion of fibers (13 of 34) responded to the probe pulse with MPIs as short as 500 micros. Spike amplitude decreased with decreasing MPI, a finding relevant to the development of computational nerve-fiber models, interpretation of gross evoked potentials, and models of more central neural processing. A small mean decrement in spike jitter was noted at small MPI values. Some trends (such as spike latency-vs-MPI) varied across fibers, suggesting that sites of excitation varied across fibers. Relative spread was found to increase with decreasing MPI values, providing direct evidence that stochastic properties of fibers are altered under conditions of refractoriness.

  12. Stimulation of the bilateral anterior nuclei of the thalamus in the treatment of refractory epilepsy: two cases of subcortical band heterotopia.

    Science.gov (United States)

    Franco, Ana; Pimentel, José; Campos, Alexandre Rainha; Morgado, Carlos; Pinelo, Sara; Ferreira, António Gonçalves; Bentes, Carla

    2016-12-01

    Subcortical band heterotopia is a neuronal migration disorder that may cause refractory epilepsy. In these patients, resective surgery has yielded inadequate results. Deep brain stimulation of the anterior nuclei of the thalamus has been used for the treatment of refractory epilepsy with good results. We describe the first two patients with subcortical band heterotopia who were submitted to deep brain stimulation of the anterior nuclei of the thalamus, with evaluation of seizure outcome after 12 and 18 months of follow-up. At these times, both showed a >50% decrease in seizure frequency and an increase in seizure freedom. Both patients had a depressive syndrome after surgery that responded fully to anti-depressive medication in one patient and partly in the other. In both, deep brain stimulation of the anterior nuclei of the thalamus was associated with good seizure outcome. This procedure can therefore be considered in the treatment of patients with subcortical band heterotopia and refractory epilepsy. Depression may be a transient adverse event of the surgery or stimulation, however, its aetiology is probably multifactorial.

  13. Refractory alloy technology for space nuclear power applications

    International Nuclear Information System (INIS)

    Cooper, R.H. Jr.; Hoffman, E.E.

    1984-01-01

    Purpose of this symposium is twofold: (1) to review and document the status of refractory alloy technology for structural and fuel-cladding applications in space nuclear power systems, and (2) to identify and document the refractory alloy research and development needs for the SP-100 Program in both the short and the long term. In this symposium, an effort was made to recapture the space reactor refractory alloy technology that was cut off in midstream around 1973 when the national space nuclear reactor program began in the early 1960s, was terminated. The six technical areas covered in the program are compatibility, processing and production, welding and component fabrication, mechanical and physical properties, effects of irradiation, and machinability. The refractory alloys considered are niobium, molybdenum, tantalum, and tungsten. Thirteen of the 14 pages have been abstracted separately. The remaining paper summarizes key needs for further R and D on refractory alloys

  14. [Norrie-Wardburg syndrome].

    Science.gov (United States)

    Skevas, A; Kastanioudakis, I; Daniilidis, B; Exarchakos, G

    1992-10-01

    We describe a case of a 25-year old patient with typical Norrie-Warburg Syndrome. From the first year of his life he was found to be blind, with bilateral sensorineural loss of hearing. Audiological examination showed symmetrical moderate bilateral sensorineural hearing loss. His hearing loss was refractory to treatment for the last eight years. Because of timely diagnosis of hearing loss and timely fitting of a hearing aid, the patient could study at school and graduate from university education. Disease carriers who are clinically healthy can be identified only via chromosome analysis.

  15. Continuous positive airway pressure improves gait control in severe obstructive sleep apnoea: A prospective study.

    Directory of Open Access Journals (Sweden)

    Sébastien Baillieul

    Full Text Available Severe obstructive sleep apnoea (OSA can lead to neurocognitive alterations, including gait impairments. The beneficial effects of continuous positive airway pressure (CPAP on improving excessive daytime sleepiness and daily functioning have been documented. However, a demonstration of CPAP treatment efficacy on gait control is still lacking. This study aims to test the hypothesis that CPAP improves gait control in severe OSA patients.In this prospective controlled study, twelve severe OSA patients (age = 57.2±8.9 years, body mass index = 27.4±3.1 kg·m-2, apnoea-hypopnoea index = 46.3±11.7 events·h-1 and 10 healthy matched subjects were included. Overground gait parameters were recorded at spontaneous speed and stride time variability, a clinical marker of gait control, was calculated. To assess the role of executive functions in gait and postural control, a dual-task paradigm was applied using a Stroop test as secondary cognitive task. All assessments were performed before and after 8 weeks of CPAP treatment.Before CPAP treatment, OSA patients had significantly larger stride time variability (3.1±1.1% vs 2.1±0.5% and lower cognitive performances under dual task compared to controls. After CPAP treatment, stride time variability was significantly improved and no longer different compared to controls. Cognitive performance under dual task also improved after CPAP treatment.Eight weeks of CPAP treatment improves gait control of severe OSA patients, suggesting morphological and functional cerebral improvements. Our data provide a rationale for further mechanistic studies and the use of gait as a biomarker of OSA brain consequences.

  16. An investigation into mineral processing of north Semnan refractory earth

    International Nuclear Information System (INIS)

    Aslani, S.; Samin-Bani-Hashemi, H.R.; Taghi-Zadeh, O.

    2002-01-01

    This paper is dealing with refractory earth of North Semnan. Having an area of 2000 square kilometers, Semnan province is mainly formed by sedimentary rocks with a verity of refractory earth, red earth and kaolin containing heavy minerals. The refractory earth of this area contains a considerable rate of aluminum oxide in shape of dia spore minerals, behemoth and gybsite along with heavy minerals of iron and titanium. To improve the quality of refractory earth, in order to be used in related industries, these minerals have to be separated. To assess the quality of refractory earth of North Semnan as the raw materials of refractory industries, their genesis and mineralogy properties have been precisely studied. Based on the rate of aluminium oxide of the refractory earth of North Semnan mines, a suitable mineral deposit has been selected for more investigation. Using XRD and X RF methods along with electronic and photo microscopes, the refractory earth and heavy minerals of them have been assessed. The elementary laboratory experiments of fragmentation and magnetic separation have been performed. It has been proved that the iron minerals can be separated and, therefore, the quality of the refractory earth can be improved. The separation of titanium minerals has to be investigated with other methods

  17. REFRACTORY THROMBOCYTOPENIA AND NEUTROPENIA: A DIAGNOSTIC CHALLENGE

    Directory of Open Access Journals (Sweden)

    Emmanuel Gyan

    2015-02-01

    Full Text Available Background. The 2008 WHO classification identified refractory cytopenia with unilineage dysplasia (RCUD as a composite entity encompassing refractory anemia, refractory thrombocytopenia (RT, and refractory neutropenia (RN, characterized by 10% or more dysplastic cells in the bone marrow respective lineage. The diagnosis of RT and RN is complicated by several factors.  Diagnosing RT first requires exclusion of familial thrombocytopenia, chronic auto-immune thrombocytopenia, concomitant medications, viral infections, or hypersplenism. Diagnosis of RN should also be made after ruling out differential diagnoses such as ethnic or familial neutropenia, as well as acquired, drug-induced, infection-related or malignancy-related neutropenia. An accurate quantification of dysplasia should be performed in order to distinguish RT or RN from the provisional entity named idiopathic cytopenia of unknown significance (ICUS. Cytogenetic analysis, and possibly in the future somatic mutation analysis (of genes most frequently mutated in MDS, and flow cytometry analysis aberrant antigen expression on myeloid cells may help in this differential diagnosis. Importantly, we and others found that, while isolated neutropenia and thrombocytopenia are not rare in MDS, those patients can generally be classified (according to WHO 2008 classification as refractory cytopenia with multilineage dysplasia or refractory anemia with excess blasts, while RT and RN (according to WHO 2008 are quite rare.These results suggest in particular that identification of RT and RN as distinct entities could be reconsidered in future WHO classification updates.

  18. Extracorporeal life support for cardiac arrest in a 13-year-old girl caused by Wolff-Parkinson-White syndrome.

    Science.gov (United States)

    Song, Kyoung Hwan; Lee, Byung Kook; Jeung, Kyung Woon; Lee, Dong Hun

    2015-10-01

    Generally, Wolff-Parkinson-White (WPW) syndrome presents good prognosis. However, several case reports demonstrated malignant arrhythmia or sudden cardiac death as WPW syndrome's first presentation. Cardiopulmonary resuscitation using extracorporeal life support is a therapeutic option in refractory cardiac arrest. We present a WPW syndrome patient who had sudden cardiac arrest as the first presentation of the disease and treated it using extracorporeal life support with good neurologic outcome.

  19. Obstetric antiphospholipid syndrome.

    Science.gov (United States)

    Esteve-Valverde, E; Ferrer-Oliveras, R; Alijotas-Reig, J

    2016-04-01

    Obstetric antiphospholipid syndrome is an acquired autoimmune disorder that is associated with various obstetric complications and, in the absence of prior history of thrombosis, with the presence of antiphospholipid antibodies directed against other phospholipids, proteins called cofactors or PL-cofactor complexes. Although the obstetric complications have been related to the procoagulant properties of antiphospholipid antibodies, pathological studies of human placenta have shown the proinflammatory capacity of antiphospholipid antibodies via the complement system and proinflammatory cytokines. There is no general agreement on which antiphospholipid antibodies profile (laboratory) confers the greatest obstetric risk, but the best candidates are categories I and IIa. Combined treatment with low doses of aspirin and heparin achieves good obstetric and maternal outcomes. In this study, we also review the therapeutic possibilities in refractory cases, although the likelihood of progressing to other autoimmune diseases is low. We briefly comment on incomplete obstetric antiphospholipid syndrome, also known as antiphospholipid antibody-mediated pregnancy morbidity syndrome. Copyright © 2015 Elsevier España, S.L.U. y Sociedad Española de Medicina Interna (SEMI). All rights reserved.

  20. Refractory alloy technology for space nuclear power applications

    Energy Technology Data Exchange (ETDEWEB)

    Cooper, R.H. Jr.; Hoffman, E.E. (eds.)

    1984-01-01

    Purpose of this symposium is twofold: (1) to review and document the status of refractory alloy technology for structural and fuel-cladding applications in space nuclear power systems, and (2) to identify and document the refractory alloy research and development needs for the SP-100 Program in both the short and the long term. In this symposium, an effort was made to recapture the space reactor refractory alloy technology that was cut off in midstream around 1973 when the national space nuclear reactor program began in the early 1960s, was terminated. The six technical areas covered in the program are compatibility, processing and production, welding and component fabrication, mechanical and physical properties, effects of irradiation, and machinability. The refractory alloys considered are niobium, molybdenum, tantalum, and tungsten. Thirteen of the 14 pages have been abstracted separately. The remaining paper summarizes key needs for further R and D on refractory alloys. (DLC)

  1. Temperature and Thermal Stress Analysis of Refractory Products

    Directory of Open Access Journals (Sweden)

    Shaoyang Shi

    2013-05-01

    Full Text Available Firstly current status of temperature and thermal stress research of refractory product at home and aboard are analyzed. Finite element model of two classical refractory products is building by using APDL language. Distribution law of temperature and thermal stress of two typical refractory products-ladles and tundish are analyzed and their structures are optimized. Stress of optimal structure is dropped obviously, and operation life is increased effectively.

  2. Obstructive sleep apnoea is frequent in patients with type 1 diabetes

    DEFF Research Database (Denmark)

    Banghoej, Anne Margareta; Nerild, Henriette Holst; Kristensen, Peter Lommer

    2017-01-01

    mild OSA (60 patients (69%)). OSA was present in 32% of the patients with normal BMI, in 60% of overweight patients, and in 61% of obese patients. Patients with type 1 diabetes and OSA were largely asymptomatic and did not report more sleepiness than patients without OSA. At multivariate analysis, age......AIM: Obstructive Sleep Apnoea (OSA) is frequent in patients with type 2 diabetes. The aim of this study is to evaluate prevalence of OSA in patients with type 1 diabetes. METHODS: In a cross-sectional design, all patients with type 1 diabetes attending the outpatient clinic were offered screening...... of 200 of 518 eligible patients with type 1 diabetes (39%) participated (68% men; age 52±15years (mean±SD), diabetes duration 24±14years and BMI 25.3±3.3kg/m(2)). OSA was diagnosed in 92 patients (46% (95% CI: 40-53)). Five patients had known OSA, and OSA was newly diagnosed in 87 patients, predominantly...

  3. Comparative evaluation of the locally manufactured low-alumina fireclay refractories

    International Nuclear Information System (INIS)

    Mohammad, D.; Ahmed, W.; Khan, M.I.

    2007-01-01

    Properties of domestically produced low-alumina refractories commonly known as medium duty fireclay refractories were studied and a comparative evaluation was performed. Refractory properties such as density, porosity, cold strength, reheat change and deformation at high-temperature were determined. Significant differences were discovered between these products sold as medium duty fireclay refractories. One brand marketed as medium duty refractory was found to be too inferior to be designated as medium duty firebrick. Absence of any standards and lack of awareness on the part of users of the various test methods appear to be the cause of this situation. (author)

  4. [3-hydroxy-3-methylglutaric aciduria and recurrent Reye-like syndrome].

    Science.gov (United States)

    Eirís, J; Ribes, A; Fernández-Prieto, R; Rodríguez-García, J; Rodríguez-Segade, S; Castro-Gago, M

    1998-06-01

    3-hydroxy-3-methylglutaryl-coenzyme A lyase deficiency (HMG-CoA lyase) is an inborn error of ketogenesis and Leucine catabolism. HMG-CoA lyase catalyses the final step in leucine degradation, converting HMG-CoA to acetyl-CoA and acetoacetic acid. Clinical manifestations include hepatomegaly, lethargy or coma and apnoea. Biochemically there is a characteristic absence of ketosis with hypoglycemia, acidosis, hipertransaminasemia and variable hyperammoniemia. The urinary organic acid profile includes elevated concentrations of 3-hydroxy-3-isovaleric, 3-hydroxy-3-methylglutaric, 3-methylglutaconic and 3-methylglutaric acids. Here, we report the case of a 17-year-old girl who presented in both ten months and five years of age a clinical picture characterized by lethargy leading to apnea and coma, hepatomegaly, hypoglycemia, metabolic acidosis, hyperammoniemia, elevated serum transaminases and absence of ketonuria. Diagnostic of Reye syndrome was suggested by hystopathologic finding of hepatic steatosis and clinical and biochemical data. As of 11 years old, laboratory investigations revealed carnitine deficiency and characteristic aciduria. Confirmatory enzyme diagnosis revealing deficiency of HMG-CoA lyase was made in cultured fibroblasts. Our report constitutes an example of the presentation of HMG-CoA lyase deficiency as recurrent Reye-like syndrome.

  5. Directed light fabrication of refractory metals and alloys

    International Nuclear Information System (INIS)

    Fonseca, J.C.; Lewis, G.K.; Dickerson, P.G.; Nemec, R.B.

    1999-01-01

    This report covers deposition of refractory pure metals and alloys using the Directed Light Fabrication (DLF) process and represents progress in depositing these materials through September 1998. In extending the DLF process technology to refractory metals for producing fully dense, structurally sound deposits, several problems have become evident. (1) Control of porosity in DLF-deposited refractory metal is difficult because of gases, apparently present in commercially purchased refractory metal powder starting materials. (2) The radiant heat from the molten pool during deposition melts the DLF powder feed nozzle. (3) The high reflectivity of molten refractory metals, at the Nd-YAG laser wavelength (1.06microm), produces damaging back reflections to the optical train and fiber optic delivery system that can terminate DLF processing. (4) The current limits on the maximum available laser power to prevent back reflection damage limit the parameter range available for densification of refractory metals. The work to date concentrated on niobium, W-25Re, and spherodized tungsten. Niobium samples, made from hydride-dehydride powder, had minimal gas porosity and the deposition parameters were optimized; however, test plates were not made at this time. W-25Re samples, containing sodium and potassium from a precipitation process, were made and porosity was a problem for all samples although minimized with some process parameters. Deposits made from potassium reduced tungsten that was plasma spherodized were made with minimized porosity. Results of this work indicate that further gas analysis of starting powders and de-gassing of starting powders and/or gas removal during deposition of refractory metals is required

  6. Vasoplegic syndrome: A challenge to anaesthetic management

    Directory of Open Access Journals (Sweden)

    Amarjyoti Hazarika

    2015-01-01

    Full Text Available Perioperative hypotension is a well-recognized and relatively common problem during surgery. Vasoplegic syndrome is one such condition which is characterized by severe persistent hypotension with normal to high cardiac output and low systemic resistance. It is commonly seen in patients undergoing cardiac surgery on cardiopulmonary bypass. However, this syndrome has also been reported in off pump surgeries. Management of intraoperative hypotension may be challenging for an anaesthesiologist, if it does not respond or poorly respond to conventional therapy. We report the management of a hypertensive patient posted for spine surgery in prone position, who developed severe hypotension under anaesthesia refractory to treatment.

  7. Alcohol percutaneous neurolysis of the sphenopalatine ganglion in the management of refractory cranio-facial pain

    International Nuclear Information System (INIS)

    Kastler, Adrian; Cadel, Gilles; Gory, Guillaume; Comte, Alexandre; Piccand, Veronique; Tavernier, Laurent; Kastler, Bruno

    2014-01-01

    The sphenopalatine ganglion (SPN) has been proven to be involved in various types of facial pain syndromes. Management of these cranio-facial pain syndromes can be challenging, and existing specific treatments are sometimes inefficient and may fail. The purpose of this study is to describe and evaluate alcohol SPN in the management of cranio-facial pain. Forty-two patients suffering from refractory facial pain who underwent 58 consecutive SPN were included in this study between 2000 and 2013. Patients were divided into three groups: group ''cluster headache'' (CH), group ''persistent idiopathic facial pain'' (PFIP), and group ''Other''. Pain was assessed using Visual Analogue Scale scores (measured immediately before and after procedure and at regular intervals following the procedure). Alcohol SPN was considered to be effective when pain relief was equal to or greater than 50 % and lasting for at least 1 month. All procedures were realized ambulatory under CT guidance and consisted of an injection of 1 ml of absolute alcohol. Overall efficacy rate of alcohol SPN was 67.2 %, with mean pain relief duration of 10.3 months. Procedure was graded either not painful or tolerable by patients in 64.2 %. Analysis showed a higher efficacy rate in the groups CH (76.5 %) and PFIP (85.7 %) compared to the group Other (40 %). No difference was found between groups regarding the recurrence rate. Alcohol SPN under CT guidance appears as a safe and effective treatment of refractory facial pain, especially in cases of cluster headache and persistent idiopathic facial pain. (orig.)

  8. Efficacy and Safety of Deep Brain Stimulation in Tourette Syndrome: The International Tourette Syndrome Deep Brain Stimulation Public Database and Registry.

    Science.gov (United States)

    Martinez-Ramirez, Daniel; Jimenez-Shahed, Joohi; Leckman, James Frederick; Porta, Mauro; Servello, Domenico; Meng, Fan-Gang; Kuhn, Jens; Huys, Daniel; Baldermann, Juan Carlos; Foltynie, Thomas; Hariz, Marwan I; Joyce, Eileen M; Zrinzo, Ludvic; Kefalopoulou, Zinovia; Silburn, Peter; Coyne, Terry; Mogilner, Alon Y; Pourfar, Michael H; Khandhar, Suketu M; Auyeung, Man; Ostrem, Jill Louise; Visser-Vandewalle, Veerle; Welter, Marie-Laure; Mallet, Luc; Karachi, Carine; Houeto, Jean Luc; Klassen, Bryan Timothy; Ackermans, Linda; Kaido, Takanobu; Temel, Yasin; Gross, Robert E; Walker, Harrison C; Lozano, Andres M; Walter, Benjamin L; Mari, Zoltan; Anderson, William S; Changizi, Barbara Kelly; Moro, Elena; Zauber, Sarah Elizabeth; Schrock, Lauren E; Zhang, Jian-Guo; Hu, Wei; Rizer, Kyle; Monari, Erin H; Foote, Kelly D; Malaty, Irene A; Deeb, Wissam; Gunduz, Aysegul; Okun, Michael S

    2018-03-01

    Collective evidence has strongly suggested that deep brain stimulation (DBS) is a promising therapy for Tourette syndrome. To assess the efficacy and safety of DBS in a multinational cohort of patients with Tourette syndrome. The prospective International Deep Brain Stimulation Database and Registry included 185 patients with medically refractory Tourette syndrome who underwent DBS implantation from January 1, 2012, to December 31, 2016, at 31 institutions in 10 countries worldwide. Patients with medically refractory symptoms received DBS implantation in the centromedian thalamic region (93 of 163 [57.1%]), the anterior globus pallidus internus (41 of 163 [25.2%]), the posterior globus pallidus internus (25 of 163 [15.3%]), and the anterior limb of the internal capsule (4 of 163 [2.5%]). Scores on the Yale Global Tic Severity Scale and adverse events. The International Deep Brain Stimulation Database and Registry enrolled 185 patients (of 171 with available data, 37 females and 134 males; mean [SD] age at surgery, 29.1 [10.8] years [range, 13-58 years]). Symptoms of obsessive-compulsive disorder were present in 97 of 151 patients (64.2%) and 32 of 148 (21.6%) had a history of self-injurious behavior. The mean (SD) total Yale Global Tic Severity Scale score improved from 75.01 (18.36) at baseline to 41.19 (20.00) at 1 year after DBS implantation (P tic subscore improved from 21.00 (3.72) at baseline to 12.91 (5.78) after 1 year (P tic subscore improved from 16.82 (6.56) at baseline to 9.63 (6.99) at 1 year (P Tourette syndrome but also with important adverse events. A publicly available website on outcomes of DBS in patients with Tourette syndrome has been provided.

  9. Machining refractory alloys: an overview

    International Nuclear Information System (INIS)

    Christopher, J.D.

    1984-01-01

    Nontraditional machining is a generic term for those material removal processes that differ drastically from the historic operations such as turning, milling, drilling, tapping, and grinding. The use of primary energy modes other than mechanical, such as thermal, electrical, and chemical, sets these operations apart and reinforces their nontraditional label. Several of these newer processes have been very successful in machining close tolerance parts from refractory materials. This paper provides a general overview of both traditional and nontraditional aspects of machining refractory materials. 11 figures, 7 tables

  10. Transvenous stimulation of the phrenic nerve for the treatment of central sleep apnoea: 12 months' experience with the remedē® System.

    Science.gov (United States)

    Jagielski, Dariusz; Ponikowski, Piotr; Augostini, Ralph; Kolodziej, Adam; Khayat, Rami; Abraham, William T

    2016-11-01

    Patients with central sleep apnoea (CSA) often have poor quality of life and are at increased risk of morbidity and mortality. This study sought to evaluate the 12-month clinical outcomes of patients with CSA treated with unilateral transvenous phrenic nerve stimulation in the prospective, multicentre, non-randomized remedē ® System pilot study. Forty-seven patients with CSA were treated with the remedē ® System (Respicardia Inc., Minnetonka, MN, USA) for a minimum of 3 months. Sleep-disordered breathing parameters were evaluated by polysomnography (PSG) at 3, 6, and 12-month follow-up. Sleep symptoms and quality of life were also evaluated. Forty-one patients completed all follow-up PSGs and were included in the analysis. At 12 months, there was sustained improvement compared with baseline in the apnoea-hypopnoea index (49.9 ± 15.1 vs. 27.5 ± 18.3 events/h, P phrenic nerve stimulation is associated with sustained improvement in key sleep parameters, sleep symptoms, and quality of life over 12 months of follow-up. © 2016 The Authors. European Journal of Heart Failure © 2016 European Society of Cardiology.

  11. Production of lightweight refractory material by hydrothermal process

    International Nuclear Information System (INIS)

    Sulejmani, Ramiz B.

    2002-01-01

    Many different processes of production of lightweight refractories are well known over the World. Traditional production of lightweight refractories is by addition of combustibles or by a special frothing process. This work is concerned with hydrothermal of lightweight refractories from rice husk ash. The rice husk ash, used in present investigations were from Kocani region, R. Macedonia. The chemical analysis of the rice husk ash shows that it contains 91,8 - 93,7% SiO 2 and some alkaline and alkaline earth oxides. Microscopic and X - ray diffraction examinations of the rice husk ash have shown that it is composed of cristobalite, tridimite and amorphous silica. The composition of the mixture for lightweight refractory brick production is 93,4% rice husk ash and 6,6% Ca(OH) 2 . The mixtures were well mixed, moistened and pressed at 5 - 10 MPa. The hydrothermal reactions between calcium hydroxide and rice husk ash over the temperature range 80 - 160 o C were investigated. The period of autoclave treatment was from 2 to 72 h. After the hydrothermal treatment of the samples, the mineralogical composition, bulk density, density, cold crushing strength, porosity, refractoriness and thermal expansion were examined. Analysing the properties of the obtained samples it can be concluded that from rice husk ash and calcium hydroxide under hydrothermal condition it is possible to obtain lightweight acid refractory material with high quality.(Author)

  12. Temporal associations between arousal and body/limb movement in children with suspected obstructed sleep apnoea.

    Science.gov (United States)

    Lamprecht, Marnie L; Bradley, Andrew P; Williams, Gordon; Terrill, Philip I

    2016-01-01

    The inter-relationship between arousal events and body and/or limb movements during sleep may significantly impact the performance and clinical interpretation of actigraphy. As such, the objective of this study was to quantify the temporal association between arousals and body/limb movement. From this, we aim to determine whether actigraphy can predict arousal events in children, and identify the impact of arousal-related movements on estimates of sleep/wake periods. Thirty otherwise healthy children (5-16 years, median 9 years, 21 male) with suspected sleep apnoea were studied using full polysomnography and customised raw tri-axial accelerometry measured at the left fingertip, left wrist, upper thorax, left ankle and left great toe. Raw data were synchronised to within 0.1 s of the polysomnogram. Movements were then identified using a custom algorithm. On average 67.5% of arousals were associated with wrist movement. Arousals associated with movement were longer than those without movement (mean duration: 12.2 s versus 7.9 s respectively, p  <  0.01); movements during wake and arousal were longer than other sleep movements (wrist duration: 6.26 s and 9.89 s versus 2.35 s respectively, p  <  0.01); and the movement index (movements/h) did not predict apnoea-hypopnoea index (ρ  =  -0.11). Movements associated with arousals are likely to unavoidably contribute to actigraphy's poor sensitivity for wake. However, as sleep-related movements tend to be shorter than those during wake or arousal, incorporating movement duration into the actigraphy scoring algorithm may improve sleep staging performance. Although actigraphy-based measurements cannot reliably predict all arousal events, actigraphy can likely identify longer events that may have the greatest impact on sleep quality.

  13. Improvement in symptoms of the syndrome of mitochondrial encephalopathy, lactic acidosis, and stroke-like symptoms (MELAS) following treatment with sympathomimetic amines--possible implications for improving fecundity in women of advanced reproductive age.

    Science.gov (United States)

    Potestio, C P; Check, J H; Mitchell-Williams, J

    2014-01-01

    To evaluate the efficacy of sympathomimetic amine therapy on a mitochondrial abnormality known as the mitochondrial encephalopathy lactic acidosis and stroke-like symptoms syndrome (MELAS syndrome). Dextroamphetamine sulfate 15 mg extended release capsule was prescribed to a woman with a 25 year history of MELAS syndrome refractory to most other therapies. Within one month of therapy the woman noticed considerable improvement in her chronic fatigue, pain, and edema. The MELAS syndrome is thus another condition to add to the list of various chronic refractory disorders that improve considerably after dextroamphetamine therapy. This is the first mitochondrial disorder shown to improve with sympathomimetic amines which could suggest that dextroamphetamine could prove useful in decreasing the risk of aneuploidy in women of advanced reproductive age.

  14. SB-715992 in Treating Patients With Acute Leukemia, Chronic Myelogenous Leukemia, or Advanced Myelodysplastic Syndromes

    Science.gov (United States)

    2013-01-10

    Acute Undifferentiated Leukemia; Adult Acute Megakaryoblastic Leukemia (M7); Adult Acute Minimally Differentiated Myeloid Leukemia (M0); Adult Acute Monoblastic Leukemia (M5a); Adult Acute Monocytic Leukemia (M5b); Adult Acute Myeloblastic Leukemia With Maturation (M2); Adult Acute Myeloblastic Leukemia Without Maturation (M1); Adult Acute Myeloid Leukemia With 11q23 (MLL) Abnormalities; Adult Acute Myeloid Leukemia With Inv(16)(p13;q22); Adult Acute Myeloid Leukemia With t(15;17)(q22;q12); Adult Acute Myeloid Leukemia With t(16;16)(p13;q22); Adult Acute Myeloid Leukemia With t(8;21)(q22;q22); Adult Acute Myelomonocytic Leukemia (M4); Adult Acute Promyelocytic Leukemia (M3); Adult Erythroleukemia (M6a); Adult Pure Erythroid Leukemia (M6b); Blastic Phase Chronic Myelogenous Leukemia; de Novo Myelodysplastic Syndromes; Previously Treated Myelodysplastic Syndromes; Recurrent Adult Acute Lymphoblastic Leukemia; Recurrent Adult Acute Myeloid Leukemia; Refractory Anemia With Excess Blasts; Refractory Anemia With Excess Blasts in Transformation; Relapsing Chronic Myelogenous Leukemia; Secondary Acute Myeloid Leukemia; Secondary Myelodysplastic Syndromes; Untreated Adult Acute Myeloid Leukemia

  15. Directed light fabrication of refractory metals and alloys

    International Nuclear Information System (INIS)

    Fonseca, J.C.; Lewis, G.K.; Dickerson, P.G.; Nemec, R.B.

    1999-01-01

    This report covers work performed under Order No. FA0000020 AN Contract DE-AC12-76SN00052 for deposition of refractory pure metals and alloys using the Directed Light Fabrication (DLF) process and represents the progress in depositing these materials through September 1998. In extending the DLF process technology to refractory metals for producing fully dense, structurally sound deposits, several problems have become evident. 1. Control of porosity in DLF-deposited refractory metal is difficult because of gases, apparently present in commercially purchased refractory metal powder starting materials. 2. The radiant heat from the molten pool during deposition melts the DLF powder feed nozzle. 3. The high reflectivity of molten refractory metals, at the Nd-YAG laser wavelength (1.06microm), produces damaging back reflections to the optical train and fiber optic delivery system that can terminate DLF processing. 4. The current limits on the maximum available laser power to prevent back reflection damage limit the parameter range available for densification of refractory metals. The work to date concentrated on niobium, W-25Re, and spherodized tungsten. Niobium samples, made from hydride-dehydride powder, had minimal gas porosity and the deposition parameters were optimized; however, test plates were not made at this time. W-25Re samples, containing sodium and potassium from a precipitation process, were made and porosity was a problem for all samples although minimized with some process parameters. Deposits made from potassium reduced tungsten that was plasma spherodized were made with minimized porosity. Results of this work indicate that further gas analysis of starting powders and de-gassing of starting powders and/or gas removal during deposition of refractory metals is required

  16. Neurobehavioral aspects, pathophysiology, and management of Tourette syndrome.

    Science.gov (United States)

    Shprecher, David R; Schrock, Lauren; Himle, Michael

    2014-08-01

    This update summarizes progress in understanding Tourette syndrome clinical characteristics, etiology, and treatment over the past year. Premonitory sensory phenomena were found to have important impacts on Tourette syndrome quality of life. A rare genetic form of Tourette syndrome due to L-histidine-decarboxylase mutation, with similar features in human and rodent, has inspired new research on functional anatomy of Tourette syndrome. In response to new data, treatment guidelines have been revised to include behavioral therapy as first-line treatment. Novel dopamine receptor antagonists aripiprazole and ecopipam have shown potential efficacy - as well as tolerability concerns. Recent work has suggested efficacy and tolerability of topiramate and fluphenazine, but more rigorous studies are needed to further understand their role in Tourette syndrome management. Recent consensus guidelines explain when deep brain stimulation can be considered for severe refractory cases under a multidisciplinary team. More research is needed to identify better tolerated treatments for, to understand pathophysiology or functional anatomy of, and to predict or influence longitudinal outcome of Tourette syndrome.

  17. How to assess, diagnose, refer and treat adult obstructive sleep apnoea: a commentary on the choices.

    Science.gov (United States)

    Mansfield, Darren R; Antic, Nicholas A; McEvoy, R Doug

    2013-10-21

    Obstructive sleep apnoea (OSA) determined by polysomnography is highly prevalent, affecting about 25% of men and 10% of women in the United States, although most have few or no symptoms. Symptomatic moderate to severe OSA has major health implications related to daytime sleepiness, such as increased accidents, altered mood and loss of productivity in the workplace. Severe OSA may increase the risk of cardiovascular disease independent of daytime sleepiness. A major challenge is to correctly identify, from the large community pool of disease, people with symptoms and those at risk of long-term complications. For treatment plans to achieve quality patient outcomes, clinicians must have a clear understanding of patients' symptoms and their motivations for presentation, and be knowledgeable about the evidence surrounding the health risks of OSA and the relative merits of the various diagnostic and treatment options available. The diagnosis of OSA represents a teachable moment to target adverse lifestyle factors such as excessive weight, excessive alcohol consumption and smoking, which may be contributing to OSA and long-term cardiometabolic risk. OSA assessment and management has traditionally involved specialist referral and in-laboratory polysomnography. However, these services may not always be easy to access. Controlled studies have shown that patients with a high pretest probability of symptomatic, moderate to severe OSA can be managed well in primary care, or by skilled nurses with appropriate medical backup, using simplified ambulatory models of care. The future of sleep apnoea assessment and management will likely include models of care that involve early referral to specialists of patients with complex or atypical presentations, and an upskilled and supported primary care workforce to manage symptomatic, uncomplicated, high pretest probability disease.

  18. Maxillomandibular advancement as the initial treatment of obstructive sleep apnoea: Is the mandibular occlusal plane the key?

    Science.gov (United States)

    Rubio-Bueno, P; Landete, P; Ardanza, B; Vázquez, L; Soriano, J B; Wix, R; Capote, A; Zamora, E; Ancochea, J; Naval-Gías, L

    2017-11-01

    Maxillomandibular advancement (MMA) can be effective for managing obstructive sleep apnoea (OSA); however, limited information is available on the predictor surgical variables. This study investigated whether normalization of the mandibular occlusal plane (MOP) was a determinant factor in curing OSA. Patients with moderate or severe OSA who underwent MMA were evaluated by preoperative and postoperative three-dimensional (3D) scans and polysomnograms. The postoperative value of MOP and the magnitude of skeletal advancement were the predictor variables; change in the apnoea-hypopnoea index (AHI) was the main outcome variable. Thirty-four subjects with a mean age of 41±14years and 58,8% female were analysed. The Epworth Sleepiness Scale (ESS) was 17.4±5.4 and AHI was 38.3±10.7 per hour before surgery. Postoperative AHI was 6.5±4.3 per hour (P<0.001) with 52.94% of the patients considered as cured, and 47.06% suffering from a mild residual OSA with ESS 0.8±1.4 (P<0.001). 3D changes revealed a volume increase of 106.3±38.8%. The mandible was advanced 10.4±3.9mm and maxilla 4.9±3.2mm. MOP postoperative value was concluded to be the best predictor variable. Treatment planning should include MOP normalization and a mandibular advancement between 6 and 10mm. The maxillary advancement would depend on the desired aesthetic changes and final occlusion. Copyright © 2017 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.

  19. Spinning of refractory metals

    International Nuclear Information System (INIS)

    Chang Wenkua; Zheng Han

    1989-01-01

    The effects of spinning process parameters including max. pass percentage reduction, spinning temperature, feed rate, lubricant and annealing technology on the quality of shaped components are summarized and discussed in the present paper. The above mentioned parameters are adopted in the process of spinning of barrel-shaped and specially shaped components of refractory metals and their alloys W, Mo, Nb, Zr, TZM molybdenum alloy, C-103, C-752 niobium alloy etc. The cause of leading to usual defects of spun products of refractory metals such as lamellar as 'scaling', crack, swelling, wrinkle, etc. have been analysed and the ways to eliminate the defects have been put forward. 8 figs., 5 tabs. (Author)

  20. Migration of TRU-containing slag into candidate refractories for waste management

    International Nuclear Information System (INIS)

    Seymour, W.C.

    1978-11-01

    Results of initial tests on transuranic migration into commercial refractories are reported. Five castable refractories and a plastic-bonded refractory were tested using the cup test method. Initial results indicate that castable materials will not survive conditions expected in the slagging pyrolysis unit; plastic refractories appear to have better performance. The major mechanism of migration into the tested refractories is pore penetration. Al 2 O 3 and Al 2 O 3 -Cr 2 O 3 phases appear resistant to chemical diffusion of transuranics. 2 figures, 16 tables

  1. Myelodysplastic syndromes in Chernobyl clean-up workers.

    Science.gov (United States)

    Gluzman, Daniil F; Sklyarenko, Lilia M; Koval, Stella V; Rodionova, Nataliia K; Zavelevich, Michael P; Ivanivskaya, Tetiana S; Poludnenko, Liudmyla Yu; Ukrainskaya, Nataliia I

    2015-10-01

    The studies of the recent decades posed the question of the association between radiation exposure and myelodysplastic syndromes (MDS). This association has been proved in secondary MDS originating upon exposure to chemotherapeutics and/or radiation therapy. The long-term study in Japanese atomic (A)-bomb survivors demonstrated the significant linear dose-response for MDS confirming the link between radiation exposure and this form of hematopoietic malignancies. All these findings provide the strong basis for studying MDS in the persons exposed to radiation following the Chernobyl disaster, especially those in the cohort of Chernobyl clean-up workers of 1986-1987. The data on MDS among Chernobyl clean-up workers (1986-1987) diagnosed in 1996-2012 at the reference laboratory of RE Kavetsky Institute of Experimental Pathology, Oncology and Radiobiology are summarized. MDS cases were diagnosed in 23 persons (21 males and 2 females) having been exposed to radiation as clean-up workers of 1986-1987. Refractory anemia (RA) has been detected in 13, refractory anemia with ring sideroblasts (RARS)-in 2, and refractory anemia with excess blasts (RAEB)-in 8 patients. The median age of those MDS patients was 62.0 years. In addition, 5 cases of chronic myelomonocytic leukemia (CMML) were recorded in the group of Chernobyl clean-up workers with the median time of 14.8 years from 1986-1987 to diagnosis. The association between radiation exposure and MDS is discussed. The suggested life-long risk for myelodysplastic syndromes among A-bomb survivors in Japan highlights the importance of the continuing follow-up studies in the affected populations in the post-Chernobyl period.

  2. The health-related quality of life of children with refractory epilepsy: a comparison of those with and without intellectual disability.

    Science.gov (United States)

    Sabaz, M; Cairns, D R; Lawson, J A; Bleasel, A F; Bye, A M

    2001-05-01

    To determine whether refractory epilepsy affects the health-related quality of life (HRQOL) of children with or without intellectual disability (ID), and if the presence of ID independently compromises HRQOL in children with refractory epilepsy. Subjects were parents of children with refractory epilepsy, whose syndrome had been defined using ILAE (International League Against Epilepsy) criteria and video-EEG monitoring. Children had the presence or absence of ID determined by formal neuropsychological or educational assessment. The relative effect of epilepsy on the two intellectual ability groups was determined using relevant clinical variables. Parents completed a valid epilepsy-specific HRQOL questionnaire for children, the Quality of Life in Childhood Epilepsy Questionnaire (QOLCE), and, depending on intellectual ability level, the Child Behaviour Checklist or Developmental Behaviour Checklist. Both intellectually normal children with epilepsy and children with epilepsy and ID were more likely to have psychosocial problems compared with their respective intellectual ability reference populations. The results also revealed that children with ID had reduced HRQOL compared with intellectually normal children; a result independent of epilepsy. Analysis of the relationship between epilepsy variables and HRQOL revealed that the QOLCE was the most sensitive in detecting variation in age at onset, seizure frequency, and medications taken. The HRQOL of children with refractory epilepsy is greatly affected, regardless of intellectual ability level. The presence of ID in children with epilepsy independently depresses HRQOL outcomes. Compared with two generic HRQOL measures, the QOLCE was the most sensitive measure to variation in epilepsy variables.

  3. Refractory of Furnaces to Reduce Environmental Impact

    International Nuclear Information System (INIS)

    Hanzawa, Shigeru

    2011-01-01

    The energy load of furnaces used in the manufacturing process of ceramics is quite large. Most of the environmental impact of ceramics manufacturing is due to the CO 2 produced from this high energy load. To improve this situation, R and D has focused on furnace systems and techniques of control in order to reduce energy load. Since furnaces are comprised of refractory, consideration of their mechanical and thermal characteristics is important. Herein are described several refractory types which were chosen through comparison of the characteristics which contribute to heat capacity reduction, heat insulating reinforcement and high emissivity, thereby improving thermal radiation heat transfer efficiency to the ceramic articles. One selected refractory material which will reduce the environmental impact of a furnace, chosen considering low heat capacity and high emissivity characteristics, is SiC. In this study, thermal radiation heat transfer efficiency improvement and its effect on ceramic articles in the furnace and oxidation behaviour were investigated at 1700K. A high density SiC refractory, built into the furnace at construction, has relatively high oxidation durability and has the ability to reduce environmental impact-CO 2 by 10 percent by decreasing the furnace's energy load. However, new oxidation prevention techniques for SiC will be necessary for long-term use in industrial furnaces, because passive to active oxidation transition behaviour of commercial SiC refractory is coming to close ideal.

  4. Refractory of Furnaces to Reduce Environmental Impact

    Science.gov (United States)

    Hanzawa, Shigeru

    2011-10-01

    The energy load of furnaces used in the manufacturing process of ceramics is quite large. Most of the environmental impact of ceramics manufacturing is due to the CO2 produced from this high energy load. To improve this situation, R&D has focused on furnace systems and techniques of control in order to reduce energy load. Since furnaces are comprised of refractory, consideration of their mechanical and thermal characteristics is important. Herein are described several refractory types which were chosen through comparison of the characteristics which contribute to heat capacity reduction, heat insulating reinforcement and high emissivity, thereby improving thermal radiation heat transfer efficiency to the ceramic articles. One selected refractory material which will reduce the environmental impact of a furnace, chosen considering low heat capacity and high emissivity characteristics, is SiC. In this study, thermal radiation heat transfer efficiency improvement and its effect on ceramic articles in the furnace and oxidation behaviour were investigated at 1700K. A high density SiC refractory, built into the furnace at construction, has relatively high oxidation durability and has the ability to reduce environmental impact-CO2 by 10 percent by decreasing the furnace's energy load. However, new oxidation prevention techniques for SiC will be necessary for long-term use in industrial furnaces, because passive to active oxidation transition behaviour of commercial SiC refractory is coming to close ideal.

  5. Development of alumino-silicate refractories in Ghana

    International Nuclear Information System (INIS)

    Kisiedu, A. K.; Tetteh, D.M.B.; Obiri, H. A.; Brenya, E. F.; Ayensu, A.

    2008-01-01

    Alumino-silicate (bauxite), andalusite, kaolin and clay were investigated for suitability in production of alumina, mullite and fireclay brick refractories. The raw materials were characterized by X-ray diffraction, differential thermal and silicate analyses. The x-ray diffraction analysis of alumina and mullite refractories fired at 1450 0 C, and fireclay bricks fired at 1350 0 C, indicated presence of corundum and alpha-alumina crystals. The values of thermal (fired) shrinkage, crushing, strength, porosity, water absorption and bulk density determined were 31.1%, 2.3 x 10 3 kg/m 3 , 4.86 x 10 6 N/m 2 and 13.2 % for mullite; 30.2%, 2.4 x 10 3 kg/m 3 , 3.20 x 10 6 N/m 2 and W = 12.8 % for alumina; and 25.2 %, 2.1 x 10 3 kg/m 3 , 2.61 x 10 6 N/m 2 and W = 11.8% for fireclay, respectively. Bauxite, andalusite and special kaolin were identified as potential raw materials for developing alumina and mullite refractories for construction of high temperature kilns and furnaces operating at 1350 0 C. The clay and kaolin minerals could be used to produce fireclay refractories for construction of incinerators operating at maximum temperatures of about 1000 0 C. The performance of the refractories was demonstrated by producing bricks to construct kilns and incinerators for the ceramic industry and hospitals. (au)

  6. Refractory disease in antineutrophil cytoplasmic antibodies associated vasculitis

    NARCIS (Netherlands)

    Rutgers, Abraham; Kallenberg, Cornelis

    Purpose of review Induction treatment of antineutrophil cytoplasmic antibodies (ANCA) associated vasculitis (AAV) is not always successful and nonresponding patients are considered refractory. Recent findings Refractory disease should be subdefined to the treatment that was received.

  7. Diagnosis and management of refractory celiac disease: a systematic review.

    Science.gov (United States)

    Labidi, Asma; Serghini, Meriem; Karoui, Sami; Boubaker, Jalel; Filali, Azza

    2013-01-01

    Refractory celiac disease is defined by persisting malabsorptive symptoms in spite of a strict gluten free diet for at least 6 to 12 months. Alternatives to gluten free diet seem to be still controversial. To describe the clinical and epidemiologic aspects of refractory celiac disease, and to identify therapeutic options in this condition. Systematic review and critical analysis of observational studies, clinical trials and case reports that focused on diagnosis and management of refractory celiac disease. Refractory celiac disease can be classified as type 1 or type 2 according to the phenotype of intraepithelial lymphocytes. Great complications such as enteropathy-associated T-cell lymphoma may occur in a subgroup of these patients mainly in refractory celiac disease type 2. Curative therapies are still lacking. Refractory celiac disease remains a diagnosis of exclusion. Its prognosis remains still dismal by the absence yet of curative therapies. However, some new treatments seem to hold promise during few cohort-studies.

  8. Modern materials based on refractory compounds

    International Nuclear Information System (INIS)

    Kosolapova, T.Ya.

    1979-01-01

    Discussed are the existing methods for synthesizing powders of binary refractory compounds and high-productivity techniques which hold promise as regards the manufacture of highly disperse and pure powders. Plasmochemical synthesis is shown to be an effective method for obtaining practically all carbides, nitrides and borides. A description is given of three main methods for obtaining single crystals of refractory compounds (TiN, TiC, ZrC, ZrB 2 , NbC) fairly perfect in structure and composition. These processes include deposition from vapour-gas phase, melting in arc plasma and crystallization from solutions in metallic melts. The advantages have been shown of the self-propagating high-temperature synthesis of refractory compounds, ensuring the manufacture of products, close in composition to stoichiometric ones simultaneously with forming of items. Mechanical, thermal, abrasive, and resistive characteristics of the above materials are presented

  9. Longstanding refractory pseudophakic cystoid macular edema resolved using intravitreal 0.7 mg dexamethasone implants

    Directory of Open Access Journals (Sweden)

    Brynskov T

    2013-06-01

    Full Text Available Troels Brynskov,1,2 Caroline Schmidt Laugesen,1 Jakob Halborg,1 Henrik Kemp,1 Torben Lykke Sørensen1,21Department of Ophthalmology, Copenhagen University Hospital Roskilde, Roskilde, Denmark; 2Faculty of Health Sciences, University of Copenhagen, Copenhagen, DenmarkBackground: Refractory pseudophakic cystoid macular edema (PCME following cataract surgery has long posed a challenge to clinicians, but intravitreal injections with a sustained delivery 0.7 mg dexamethasone implant has emerged as a promising therapy for this condition.Objective: To present a case of longstanding and refractory PCME with complete remission through 189 days of follow-up after two successive injections with intravitreal dexamethasone implants.Case report: A 59-year-old male had experienced metamorphopsia for approximately 4 years and had been diagnosed with PCME 15 months earlier. Since the time of the diagnosis, the condition had been refractory to both subtenon triamcinolone acetonide and a total of five injections with intravitreal ranibizumab. After the last injection with ranibizumab, central subfield mean thickness was 640 µm, and the best corrected visual acuity was 78 Early Treatment Diabetic Retinopathy Study letters. Following an intravitreal injection with a dexamethasone implant, the macular edema resolved at the next follow-up. The macular edema returned 187 days after the first injection and was treated with another intravitreal dexamethasone implant. Again, the macular edema subsided completely, and best corrected visual acuity improved to 84 Early Treatment Diabetic Retinopathy Study letters, a condition which was maintained through an additional 189 days of follow-up.Conclusion: Chronic PCME is traditionally a difficult condition to treat, but we are encouraged by the optimal response experienced with intravitreal sustained release dexamethasone implants in our patient whose longstanding PCME had been refractory to previous treatments with both

  10. Structure, preparation and properties of refractory compounds and systems

    International Nuclear Information System (INIS)

    Holleck, H.; Thuemmler, F.

    1977-01-01

    At the beginning of this report the possibilities of hardness optimization of refractory carbides are generally discussed. Three papers deal with TaC-basis refractories and hard metals. In particular, carbides with very low nonmetal/metal ratios and composites with hard phases formed by decomposition of tantalum carbonitrides are discussed. Another contribution reports investigations concerning the influence of the microstructure on the hardness of polycristaline mixed carbides. In a series of four papers, results are presented on the work of optimization conventional WC hard metals by introduction of a Fe,Co,Ni-binder: The influence of composition, carbon content and sintering conditions, as well as the wetting behaviour between carbides and binder metals are discussed. Phase relations in the refractory nitride and refractory nitride-binder metal systems as well as phase stabilities of ordered transition metal phases are reported in three papers, fundamental in character. Finally, the work concerning chemical analysis of refractory systems is described. (orig.) [de

  11. CLINICAL AND LABORATORY ASPECTS OF CHRONIC HEPATITIS B ON THE BACKGROUND OF REFRACTORY ANEMIA OF INFLAMMATION IN CHILDREN OF UZBEKISTAN

    Directory of Open Access Journals (Sweden)

    F. I. Inoyatova

    2017-01-01

    Full Text Available A total of 75 children with chronic hepatitis B (ChHB with a refractory variant of anemia of inflammation (AV course were examined, the pathogenetic manifestation of which was the development of iron overload syndrome (IOS. It was revealed that against the background of an increase in the severity of the IOS, the incidence of progressive forms of the disease with persistent prevalence of asthenovegetative, hemorrhagic syndromes and severe hepatosplenomegaly increased. At the same time, the leading biochemical syndromes were the presence of cytolysis with prolonged hyperfermentemia, endotoxemia and mesenchymal inflammatory syndrome. A directly proportional dependence of the hepcidin-25 peptide level on the degree of expression of the IOS, the higher the presentation of the IOS, the higher the level of suppression of peptide expression in hepatocytes. Diagnostically significant tests of severe forms of IOS in ChHB in children are the presence of hemosiderin in the urine and an increase in the level of sIL-6R in the serum.  

  12. Tourette Syndrome: Update.

    Science.gov (United States)

    Hallett, Mark

    2015-08-01

    Tourette Syndrome is a disorder characterized by tics. It typically begins in childhood and often improves in adult life. Tics are best described as voluntary movements made automatically so that volition is not ordinarily appreciated. There is frequently an urge, sometimes in the form of a specific sensory feeling (sensory tic), that precedes the tic. Patients say that they make the tic in order to reduce the urge, although shortly after the tic, the urge recurs. The sensory feeling may arise due to defective sensory habituation. Since tics relieve the urge, this can be considered rewarding, and repetition of this behavior may perpetuate the tic as a habit. Tourette Syndrome affects boys more than girls and is associated with attention deficit hyperactivity disorder and obsessive compulsive disorder. Although Tourette Syndrome often appears to be autosomal recessive in inheritance, it has been difficult to find any abnormal genes. There is a loss of inhibition in these patients and recent studies show abnormalities in brain GABA. Certainly there is also an abnormality in dopamine function and dopamine blocking agents are effective therapy. In severe drug-refractory patients, deep brain stimulation can be effective. Published by Elsevier B.V.

  13. Refractory versus resistant hypertension: Novel distinctive phenotypes

    Science.gov (United States)

    Dudenbostel, Tanja; Siddiqui, Mohammed; Gharpure, Nitin; Calhoun, David A.

    2017-01-01

    Resistant hypertension (RHTN) is relatively common with an estimated prevalence of 10-20% of treated hypertensive patients. It is defined as blood pressure (BP) >140/90 mmHg treated with ≥3 antihypertensive medications, including a diuretic, if tolerated. Refractory hypertension is a novel phenotype of severe antihypertensive treatment failure. The proposed definition for refractory hypertension, i.e. BP >140/90 mmHg with use of ≥5 different antihypertensive medications, including a diuretic and a mineralocorticoid receptor antagonist (MRA) has been applied inconsistently. In comparison to RHTN, refractory hypertension seems to be less prevalent than RHTN. This review focuses on current knowledge about this novel phenotype compared with RHTN including definition, prevalence, mechanisms, characteristics and comorbidities, including cardiovascular risk. In patients with RHTN excess fluid retention is thought to be a common mechanism for the development of RHTN. Recently, evidence has emerged suggesting that refractory hypertension may be more of neurogenic etiology due to increased sympathetic activity as opposed to excess fluid retention. Treatment recommendations for RHTN are generally based on use and intensification of diuretic therapy, especially with the combination of a long-acting thiazide-like diuretic and an MRA. Based on findings from available studies, such an approach does not seem to be a successful strategy to control BP in patients with refractory hypertension and effective sympathetic inhibition in such patients, either with medications and/or device based approaches may be needed. PMID:29034321

  14. Forming Refractory Insulation On Copper Wire

    Science.gov (United States)

    Setlock, J.; Roberts, G.

    1995-01-01

    Alternative insulating process forms flexible coat of uncured refractory insulating material on copper wire. Coated wire formed into coil or other complex shape. Wire-coating apparatus forms "green" coat on copper wire. After wire coiled, heating converts "green" coat to refractory electrical insulator. When cured to final brittle form, insulating material withstands temperatures above melting temperature of wire. Process used to make coils for motors, solenoids, and other electrical devices to be operated at high temperatures.

  15. Leg ulcers associated with Klinefelter’s syndrome: a case report and review of the literature

    OpenAIRE

    Shanmugam, Victoria K; Tsagaris, Katina C; Attinger, Christopher E

    2011-01-01

    We present the case of a young man with type II diabetes, stage III chronic kidney disease, hypertension, obstructive sleep apnea and diabetes who presented to the Georgetown University Hospital Center for Wound Healing with refractory lower extremity ulcers. Autoimmune work-up was negative. However, chromosome analysis showed a genetic variant of Klinefelter’s syndrome (48 XXYY). Lower extremity ulceration is a recognised complication of Klinefelter’s syndrome. The pathogenesis of ulcers in ...

  16. High-dose chemotherapy and auto-SCT for relapsed and refractory Hodgkin's lymphoma patients refractory to first-line salvage chemotherapy but responsive to second-line salvage chemotherapy.

    Science.gov (United States)

    Rauf, Muhammad Shahzad; Maghfoor, Irfan; Elhassan, Tusneem Ahmed M; Akhtar, Saad

    2015-01-01

    Relapsed or primary refractory Hodgkin's lymphoma (HL) patients refractory to first-line salvage chemotherapy (first salvage) and unable to undergo high-dose chemotherapy (HDC) and autologous stem cell transplant (auto-SCT) have very poor outcome. Some patients are offered second-line salvage chemotherapy (second salvage), if they are responsive and may receive HDC auto-SCT. We identified 31 patients (18 males, 13 females) from 1996-2012 who received second salvage prior to auto-SCT. Median age at auto-SCT is 22 years. Patients were grouped as (1) relapsed-refractory (Rel:Ref): patients with prior complete response (CR) and on relapse found refractory to first salvage and received second salvage and (2) refractory-refractory (Ref:Ref): patients refractory to both primary treatment and first salvage and received second salvage. Median follow-up is 63 months (18-170). Disease status after second salvage prior to HDC was CR 16 %, partial response (PR) 71 % and stable disease 13 %. After HDC auto-SCT, CR:PR: progressive disease was observed in 18 (58 %): four (12 %): nine (29 %) patients, respectively. Five-year overall survival (OS) for whole group is 57 % (Rel:Ref vs. Ref:Ref, 73 % vs. 48 %, p = 0.48). Progression-free survival (PFS) for whole group is 52 % (Rel:Ref vs. Ref:Ref, 73 % vs. 40 % respectively, p = 0.11). Second-line salvage is a valid approach with no long-term side effects for those HL patients who do not respond to first-line salvage chemotherapy and they can be candidate of HDC and stem cell transplant with a high ORR, the PFS and OS in relapse-refractory and refractory-refractory group of patients.

  17. Interictal brain SPECT in patients with medically refractory temporal lobe epilepsy; SPECT cerebral interictal em pacientes com epilepsia do lobo temporal de dificil controle

    Energy Technology Data Exchange (ETDEWEB)

    Andraus, Maria Emilia Cosenza

    2000-06-01

    The brain single photon emission computed tomography (SPECT) is s functional neuroimaging method that can detect localized changes in cerebral blood flow. The temporal lobe epilepsy (TLE) is the most common epileptic syndrome in adults, and more than 50% are medically refractory. The SPECT can contribute to investigation of epileptogenic focus and is one of the methods of pre-surgical evaluation of these patients. (author)

  18. Clinical characterization and proposed mechanism of juvenile glaucoma--a patient with a chromosome 4p deletion, Wolf-Hirschhorn Syndrome.

    Science.gov (United States)

    Curtin, Jeremy; Moloney, Greg; Grigg, John; Sharota Franzco, Dorian

    2010-09-01

    The case presented is that of a 22-year-old male with Wolf-Hirschhorn syndrome who was referred with glaucoma refractory to medical treatment. Six other patients have been described with Wolf-Hirschhorn syndrome (WHS) and glaucoma, most being congenital glaucoma with diagnosis in infancy. We describe the first case of juvenile onset glaucoma in this syndrome. Our patient had narrow angles on gonioscopy, with ultrasound biomicroscopy revealing ciliary body cysts. We alert others to the possibility of this mechanism of secondary narrow angle glaucoma associated with this chromosomal deletion syndrome.

  19. Alcohol percutaneous neurolysis of the sphenopalatine ganglion in the management of refractory cranio-facial pain

    Energy Technology Data Exchange (ETDEWEB)

    Kastler, Adrian [Grenoble University Hospital, Neuroradiology Department, Grenoble (France); Franche Comte University, I4S Laboratory, EA4268, IFR133, Besancon (France); Cadel, Gilles; Gory, Guillaume [Franche Comte University, I4S Laboratory, EA4268, IFR133, Besancon (France); Comte, Alexandre [University Hospital Besancon, Functional Imaging Research Department, Besancon (France); Piccand, Veronique [University Hospital Jean Minjoz, Pain Evaluation and Treatment Unit, Besancon (France); Tavernier, Laurent [Franche Comte University, I4S Laboratory, EA4268, IFR133, Besancon (France); University Hospital Jean Minjoz, Head and Neck Surgery-Otolaryngology Unit, Besancon (France); Kastler, Bruno [Franche Comte University, I4S Laboratory, EA4268, IFR133, Besancon (France); University Hospital Jean Minjoz, Interventional Pain Management Unit, Besancon (France)

    2014-07-15

    The sphenopalatine ganglion (SPN) has been proven to be involved in various types of facial pain syndromes. Management of these cranio-facial pain syndromes can be challenging, and existing specific treatments are sometimes inefficient and may fail. The purpose of this study is to describe and evaluate alcohol SPN in the management of cranio-facial pain. Forty-two patients suffering from refractory facial pain who underwent 58 consecutive SPN were included in this study between 2000 and 2013. Patients were divided into three groups: group ''cluster headache'' (CH), group ''persistent idiopathic facial pain'' (PFIP), and group ''Other''. Pain was assessed using Visual Analogue Scale scores (measured immediately before and after procedure and at regular intervals following the procedure). Alcohol SPN was considered to be effective when pain relief was equal to or greater than 50 % and lasting for at least 1 month. All procedures were realized ambulatory under CT guidance and consisted of an injection of 1 ml of absolute alcohol. Overall efficacy rate of alcohol SPN was 67.2 %, with mean pain relief duration of 10.3 months. Procedure was graded either not painful or tolerable by patients in 64.2 %. Analysis showed a higher efficacy rate in the groups CH (76.5 %) and PFIP (85.7 %) compared to the group Other (40 %). No difference was found between groups regarding the recurrence rate. Alcohol SPN under CT guidance appears as a safe and effective treatment of refractory facial pain, especially in cases of cluster headache and persistent idiopathic facial pain. (orig.)

  20. Refractory myasthenia gravis - clinical profile, comorbidities and response to rituximab.

    Science.gov (United States)

    Sudulagunta, Sreenivasa Rao; Sepehrar, Mona; Sodalagunta, Mahesh Babu; Settikere Nataraju, Aravinda; Bangalore Raja, Shiva Kumar; Sathyanarayana, Deepak; Gummadi, Siddharth; Burra, Hemanth Kumar

    2016-01-01

    Introduction: Myasthenia gravis (MG) is an antibody mediated autoimmune neuromuscular disorder characterized by fatigable muscle weakness. A proportion of myasthenia gravis patients are classified as refractory due to non responsiveness to conventional treatment. This retrospective study was done to evaluate clinical profile, epidemiological, laboratory, and features of patients with MG and mode of management using rituximab and complications. Methods: Data of myasthenia gravis patients admitted or presented to outpatient department (previous medical records) with MG between January 2008 and January 2016 were included. A total of 512 patients fulfilled the clinical and diagnostic criteria of myasthenia gravis of which 76 patients met the diagnostic certainty for refractory myasthenia gravis and were evaluated. Results: Out of 76 refractory MG patients, 53 (69.73%) patients fulfilled all the three defined criteria. The median age of onset of the refractory MG group was 36 years with a range of 27-53 years. In our study 25 patients (32.89%) belonged to the age group of 21-30 years. Anti-MuSK antibodies were positive in 8 non-refractory MG patients (2.06%) and 36 refractory MG patients (47.36%). Mean HbA 1C was found to be 8.6±2.33. The dose of administered prednisone decreased by a mean of 59.7% ( p =3.3x10 -8 ) to 94.6% ( p =2.2x10 -14 ) after the third cycle of rituximab treatment. Conclusion: The refractory MG patients are most commonly female with an early age of onset, anti-MuSK antibodies, and thymomas. Refractory MG patients have higher prevalence and poor control (HbA 1C >8%) of diabetes mellitus and dyslipidemia probably due to increased steroid usage. Rituximab is very efficient in treatment of refractory MG with adverse effects being low.

  1. RESULTS OF OUTPATIENT PROGRAM ON EFFECTIVE THERAPY OF REFRACTORY ARTERIAL HYPERTENSION

    Directory of Open Access Journals (Sweden)

    M. M. Batyushin

    2015-12-01

    Full Text Available Aim. To increase in efficacy of antihypertensive therapy in patients with refractory arterial hypertension (HT.Material and methods. Patients with refractory HT were revealed during first month of program. The causes of refractory HT were analyzed. Combined antihypertensive therapy was prescribed to reach target level of blood pressure (BP. This therapy lasted 24 weeks and included angiotensin converting enzyme (ACE inhibitor, thiazid diuretic (indapamide and dihydropyridine calcium antagonist (nifedipine XL.Results. 200 patients with refractory HT were revealed. True refractory HT took place in 59,9% of patients and pseudo refractory HT – in 40,1% of patients. Lack of diuretics or combined antihypertensive therapy were the main reason of insufficient BP control. Proposed 3-drugs therapy resulted in reduction of systolic BP from 190 to 132 Hg mm and diastolic BP from 104 to 81 Hg mm. Target level of BP was reached in 94% patients. There were no side effects which demanded to stop therapy.Conclusion. High incidence of pseudorefractory HT (40,1% is revealed. Significant prevalence of renal disturbances especially chronic interstitial inflammatory could be responsible for refractory HT development. Use of 3-drugs therapy (ACE inhibitor, indapamide and nifedipine XL provides effective control of BP in refractory and pseudorefractory HT.

  2. RESULTS OF OUTPATIENT PROGRAM ON EFFECTIVE THERAPY OF REFRACTORY ARTERIAL HYPERTENSION

    Directory of Open Access Journals (Sweden)

    M. M. Batyushin

    2007-01-01

    Full Text Available Aim. To increase in efficacy of antihypertensive therapy in patients with refractory arterial hypertension (HT.Material and methods. Patients with refractory HT were revealed during first month of program. The causes of refractory HT were analyzed. Combined antihypertensive therapy was prescribed to reach target level of blood pressure (BP. This therapy lasted 24 weeks and included angiotensin converting enzyme (ACE inhibitor, thiazid diuretic (indapamide and dihydropyridine calcium antagonist (nifedipine XL.Results. 200 patients with refractory HT were revealed. True refractory HT took place in 59,9% of patients and pseudo refractory HT – in 40,1% of patients. Lack of diuretics or combined antihypertensive therapy were the main reason of insufficient BP control. Proposed 3-drugs therapy resulted in reduction of systolic BP from 190 to 132 Hg mm and diastolic BP from 104 to 81 Hg mm. Target level of BP was reached in 94% patients. There were no side effects which demanded to stop therapy.Conclusion. High incidence of pseudorefractory HT (40,1% is revealed. Significant prevalence of renal disturbances especially chronic interstitial inflammatory could be responsible for refractory HT development. Use of 3-drugs therapy (ACE inhibitor, indapamide and nifedipine XL provides effective control of BP in refractory and pseudorefractory HT.

  3. Refractory Pigmentation Associated with Laugier-Hunziker Syndrome following Er:YAG Laser Treatment.

    Science.gov (United States)

    Ergun, Sertan; Saruhanoğlu, Alp; Migliari, Dante-Antonio; Maden, Ilay; Tanyeri, Hakkı

    2013-01-01

    The present report describes a case of Laugier-Hunziker syndrome (LHS), a rare benign condition. A patient with LHS develops acquired melanotic pigmentation of the lips and buccal mucosa, often with pigmentation of the nails occurring. No systemic symptoms are associated with this syndrome. Normally, no treatment is required for this condition, unless for aesthetic reason, mainly due to pigmentation on the lip mucosa. We present a case of LHS, 37-year-old female, whose pigmentations on her lip and in the oral cavity were treated with an Er:YAG laser. At the postoperative 12th month followup, the lesions recurred. The effects of any surgical attempt to treat pigmentations associated with LHS were discussed.

  4. Per-oral endoscopic myotomy for esophageal achalasia in a case of Allgrove syndrome.

    Science.gov (United States)

    Nakamura, Jun; Hikichi, Takuto; Inoue, Haruhiro; Watanabe, Ko; Kikuchi, Hitomi; Takagi, Tadayuki; Suzuki, Rei; Sugimoto, Mitsuru; Konno, Naoki; Waragai, Yuichi; Asama, Hiroyuki; Takasumi, Mika; Sato, Yuki; Irie, Hiroki; Obara, Katsutoshi; Ohira, Hiromasa

    2018-01-30

    Allgrove syndrome, also known as Triple A syndrome, is a rare autosomal recessive genetic disease characterized by three signs: esophageal achalasia, adrenocorticotropic hormone refractoriness, and alacrima. A 31-year-old male presented to our hospital for treatment of difficulty swallowing caused by esophageal achalasia. Because he had complicating alacrima, a neurologic disease, and a family history of consanguineous marriage, a genetic neurologic disease was suspected. Then, a mutation in the achalasia-addisonianism-alacrima syndrome gene was identified. With the diagnosis of Allgrove syndrome, a per-oral endoscopic myotomy (POEM) was performed for esophageal achalasia. After the POEM, the symptoms and the esophageal pressure findings ameliorated quickly, with no recurrence noted throughout a follow-up of more than 1 year. Here, we report the first case of POEM performed for esophageal achalasia in Allgrove syndrome.

  5. Oxidative DNA damage in bone marrow cells of patients with low-risk myelodysplastic syndrome

    Czech Academy of Sciences Publication Activity Database

    Novotná, Božena; Bagryantseva, Yana; Šišková, M.; Neuwirtová, R.

    2009-01-01

    Roč. 33, č. 2 (2009), s. 340-343 ISSN 0145-2126 R&D Projects: GA MZd NR8265 Institutional research plan: CEZ:AV0Z50390512 Keywords : Myelodysplastic syndrome * Refractory anemia * Oxidative DNA damage Subject RIV: EB - Genetics ; Molecular Biology Impact factor: 2.358, year: 2009

  6. Obstructive sleep apnoea-hypopnoea and arrhythmias: new updates.

    Science.gov (United States)

    Vizzardi, Enrico; Sciatti, Edoardo; Bonadei, Ivano; D'Aloia, Antonio; Curnis, Antonio; Metra, Marco

    2017-07-01

    Obstructive sleep apnoea-hypopnoea (OSAH) is a prevalent condition characterized by repetitive pharyngeal collapse during sleep, leading to hypoxemia, hypercapnia, and persistent inspiratory efforts against an occluded airway until arousal. Several studies demonstrated that OSAH exerts acute and chronic effects on the cardiovascular system. Thus, although being a respiratory problem, the most important consequences of OSAH are cardiovascular, among which there are arrhythmias. The purpose of this review is to systematically analyse what has been recently published about the relationship between OSAH and every cardiac arrhythmia separately. We searched Pubmed, Scopus, Web of Science and Cochrane Collaboration databases for 'OSAHS arrhythmias', 'OSAH arrhythmias' and 'OSA arrhythmias'. We analyse 1298 articles and meta-analyses, excluding already edited reviews. Arrhythmias, especially of ventricular origin, are frequent in OSAH. Ventricular premature beats, couplets and ventricular tachycardia runs are even more frequent in patients suffering from heart failure. They may be due to left heart remodelling, overwork and ischaemia and can explain at least some sudden deaths occurring between midnight and 6 a.m. Sinus pauses and atrioventricular blocks are increased according to the severity of the disturbance and may be reduced by continuous positive airway pressure therapy, preventing pace-maker implantation. Finally, atrial fibrillation, resistance against antiarrhythmic drugs and recurrences after surgical procedures are strongly related to OSAH. Arrhythmias are frequent in OSAH. Treatment of OSAH may reduce some of them. An implantable cardioverter-defibrillator and continuous positive airway pressure should be considered in some patients.

  7. Flextube reflectometry for level diagnosis in patients with obstructive sleep apnoea and snoring

    DEFF Research Database (Denmark)

    Faber, C E; Hilberg, O; Grymer, L

    2002-01-01

    The aim of this study was to use sound reflections in a flexible tube (flextube reflectometry) for identifying the predominant obstructive level of the upper airway in a series of patients referred to a sleep clinic. We also wished to study the relationship between the number of flextube narrowings...... per hour recording and the RDI (respiratory disturbance index = apnoeas and hypopneas per hour recording) by ResMed AutoSet (AS), which is a device based on nasal pressure variations. We performed sleep studies on 54 patients referred for snoring or OSA; 1) at home with AS; 2) in hospital using...... flextube reflectometry and AS simultaneously. The predominant obstructive level of the upper airway was retropalatal in 15 of the patients and retrolingual in 25 of the patients determined by flextube reflectometry. In 14 there was no predominant level of narrowing. We found a statistically significant...

  8. Wolff-Parkinson-white syndrome mimics a conduction disease.

    Science.gov (United States)

    Marrakchi, S; Kammoun, I; Kachboura, S

    2014-01-01

    Background. It is important to recognise Wolff-Parkinson-White (WPW) syndrome in electrocardiograms (ECG), as it may mimic ischaemic heart disease, ventricular hypertrophy, and bundle branch block. Recognising WPW syndrome allows for risk stratification, the identification of associated conditions, and the institution of appropriate management. Objective. The present case showed that electrophysiological study is indicated in patients with abnormal ECG and syncope. Case Report. A 40-year-old man with Wolff-Parkinson-White syndrome was presented to emergency with syncope. A baseline ECG was a complete right branch block and posterior left hemiblock. He was admitted to the cardiac care unit for pacemaker implantation. The atypical figure of complete right branch block and posterior left hemiblock was thought to be a "false positive" of conduction abnormality. But the long anterograde refractory period of the both accessory pathway and atrioventricular conduction may cause difficulty in diagnosing Wolff-Parkinson-White syndrome, Conclusion. A Wolff-Parkinson-White Syndrome may mimic a conduction disease. No reliable algorithm exists for making an ECG diagnosis of a preexcitation syndrome with conduction disorders. This can lead to diagnostic and therapeutic dilemmas in the context of syncope.

  9. Wolff-Parkinson-White Syndrome Mimics a Conduction Disease

    Directory of Open Access Journals (Sweden)

    S. Marrakchi

    2014-01-01

    Full Text Available Background. It is important to recognise Wolff-Parkinson-White (WPW syndrome in electrocardiograms (ECG, as it may mimic ischaemic heart disease, ventricular hypertrophy, and bundle branch block. Recognising WPW syndrome allows for risk stratification, the identification of associated conditions, and the institution of appropriate management. Objective. The present case showed that electrophysiological study is indicated in patients with abnormal ECG and syncope. Case Report. A 40-year-old man with Wolff-Parkinson-White syndrome was presented to emergency with syncope. A baseline ECG was a complete right branch block and posterior left hemiblock. He was admitted to the cardiac care unit for pacemaker implantation. The atypical figure of complete right branch block and posterior left hemiblock was thought to be a “false positive” of conduction abnormality. But the long anterograde refractory period of the both accessory pathway and atrioventricular conduction may cause difficulty in diagnosing Wolff-Parkinson-White syndrome, Conclusion. A Wolff-Parkinson-White Syndrome may mimic a conduction disease. No reliable algorithm exists for making an ECG diagnosis of a preexcitation syndrome with conduction disorders. This can lead to diagnostic and therapeutic dilemmas in the context of syncope.

  10. Colectomy for refractory constipation

    DEFF Research Database (Denmark)

    Raahave, Dennis; Loud, Franck Bjørn; Christensen, Elsebeth

    2010-01-01

    OBJECTIVE: This study evaluated the type of colectomy, postoperative complications, functional results, and satisfaction in patients with constipation refractory to conservative therapy. Further, colonic transit time (CTT), faecal load (coprostasis), and colon length (redundancies) were compared ...

  11. Statistical experimental design for refractory coatings

    International Nuclear Information System (INIS)

    McKinnon, J.A.; Standard, O.C.

    2000-01-01

    The production of refractory coatings on metal casting moulds is critically dependent on the development of suitable rheological characteristics, such as viscosity and thixotropy, in the initial coating slurry. In this paper, the basic concepts of mixture design and analysis are applied to the formulation of a refractory coating, with illustration by a worked example. Experimental data of coating viscosity versus composition are fitted to a statistical model to obtain a reliable method of predicting the optimal formulation of the coating. Copyright (2000) The Australian Ceramic Society

  12. Typical corrosion of alumina refractory in aluminum reflow oven

    International Nuclear Information System (INIS)

    Baldo, Jaoa B.

    2014-01-01

    The refractory linings of furnaces for secondary melting of aluminum, are exposed to intense attack by the molten metal. This occurs, because molten aluminum has a strong reducing power over the refractory oxide components, particularly Fe 2 O 3 , SiO 2 and TiO 2 . In this work, based on X-ray diffraction and scanning electron microscopy, it is presented a post mortem study of the mechanisms that lead to a premature wear of a 80% Al2O3 chemically bonded refractory bricks, used in the metal line of an aluminum re-melting furnace. The SEM analysis demonstrated that the oxides SiO 2 and TiO 2 contained in the refractory were reduced and transformed into their metallic elements causing an intense structural spalling. (author)

  13. Beta-blockers in cirrhosis and refractory ascites

    DEFF Research Database (Denmark)

    Kimer, Nina; Feineis, Martin; Møller, Søren

    2015-01-01

    OBJECTIVE: It is currently discussed if beta-blockers exert harmful effects and increase mortality in patients with cirrhosis and refractory ascites. In this study, we provide an overview of the available literature in this field in combination with a retrospective analysis of 61 patients...... trials (9 trials on propranolol, 1 case-control study and 4 retrospective analyses) were identified. One trial suggested an increased mortality in patients treated with beta-blockers and refractory ascites. The results of the remaining trials were inconclusive. No increase in mortality among beta-blocker......-treated patients was found in the present retrospective analysis. CONCLUSIONS: Treatment with beta-blockers may increase mortality in patients with cirrhosis and refractory ascites. However, the current evidence is sparse and high-quality studies are warranted to clarify the matter....

  14. Results of the Treatment of Chronic, Refractory CRPS with Ketamine Infusions: a Preliminary Report.

    Science.gov (United States)

    Puchalski, P; Zyluk, A

    2016-06-01

    Chronic, refractory complex regional pain syndrome remains very difficult to treat. A sub-anaesthetic low-dose ketamine has shown promise in advanced CRPS. We investigated the efficacy of ketamine in anaesthetic dosage in chronic, refractory CRPS patients that had failed available standard therapies. 5 female patients, aged a mean of 34 years with long-standing, a mean of 8 years', CRPS received ketamine in anaesthetic dosage over 10 days. The patients received 1-5 ketamine courses. The effect of gradual pain reduction was observed beginning on the 4(th)-5(th) day of treatment, associated with a decrease in the intensity of the allodynia (pain at light touch). No improvement in function (finger range of motion, grip strength) of the affected hands was noted in any patient. This beneficial analgesic effect was confined to 1.5-2.5 months after treatment and then pain relapsed to the baseline level. The results of this study show a short-term analgesic effect for this therapy, with no effect on movement and function of the affected limbs. Nevertheless, this method brings hope to the most severely ill patients who cannot be offered any other reasonable treatment option. © Georg Thieme Verlag KG Stuttgart · New York.

  15. Fire and explosion hazards of refractory compound powders

    International Nuclear Information System (INIS)

    Krivtsov, V.A.; Kostina, E.S.

    1978-01-01

    Data on fire and explosion hazards of refractory compound powders (HfC, ZrC,LaB 6 , ZrN, etc.) are presented. It is shown that refractory compounds can be fire- and exposion hazardous in various degrees. Qualitative and quantitative estimations of one of fire-hazard characteristics - smoldering temperature - are presented

  16. Reduction of central neuropathic pain with ketamine infusion in a patient with Ehlers-Danlos syndrome: a case report.

    Science.gov (United States)

    Lo, Tony Chung Tung; Yeung, Stephen Tung; Lee, Sujin; Skavinski, Kira; Liao, Solomon

    2016-01-01

    Ehlers-Danlos syndrome frequently causes acute and chronic pain because of joint subluxations and dislocations secondary to hypermobility. Current treatments for pain related to Ehlers-Danlos syndrome and central pain syndrome are inadequate. This case report discusses the therapeutic use of ketamine intravenous infusion as an alternative. A 27-year-old Caucasian female with a history of Ehlers-Danlos syndrome and spinal cord ischemic myelopathy resulting in central pain syndrome, presented with severe generalized body pain refractory to multiple pharmacological interventions. After a 7-day course of ketamine intravenous infusion under controlled generalized sedation in the intensive care unit, the patient reported a dramatic reduction in pain levels from 7-8 out of 10 to 0-3 out of 10 on a numeric rating scale and had a significant functional improvement. The patient tolerated a reduction in her pain medication regimen, which originally included opioids, gabapentin, pregabalin, tricyclic antidepressants, and nonsteroidal anti-inflammatory drugs. Ketamine infusion treatment has been used in various pain syndromes, including central neuropathic pain, ischemic pain, and regional pain syndrome. Reports have suggested that ketamine modulates pain by the regression of N-methyl-D-aspartate receptor to a resting state. As such, propagation of nociceptive signal to brain is interrupted allowing for the restoration of physiological balance between pain inhibition and facilitation. The present report shows that this treatment option can be used in patients with refractory central pain syndrome in the setting of spinal cord myelopathy secondary to Ehlers-Danlos syndrome. In addition, as seen in this case, this protocol can potentially decrease the chronic use of pain medication, such as opioids.

  17. Role of atrial fibrillation and atrioventricular conduction (including Wolff-Parkinson-White Syndrome) in sudden death

    NARCIS (Netherlands)

    Meijler, F.L.; Tweel, I. van der; Herbschleb, J.N.; Hauer, R.N.W.; Robles de Medina, E.O.

    A short refractory period of the accessory pathway is considered a major threat for sudden death in patients with Wolff-Parkinson-White syndrome and atrial fibrillation. RR interval and QRS signal analysis together with signal analysis of a bipolar high right atrial electrogram were obtained in six

  18. Hepatorenal syndrome: diagnosis, treatment and prevention

    DEFF Research Database (Denmark)

    Israelsen, Mads Egerod; Gluud, Lise Lotte; Bendtsen, Flemming

    2014-01-01

    Cirrhosis, ascites and renal impairment are associated with high morbidity and mortality. The hepatorenal syndrome (HRS) is a type of renal failure that affects patients with cirrhosis and ascites. This paper provides an update on evidence-based interventions in HRS. A number of factors can...... precipitate HRS. The monitoring, prevention, early detection, and correct treatment of these are essential. Terlipressin combined with albumin is the first-line treatment of type 1 HRS. In type 2 HRS with refractory ascites, liver transplantation and TIPS should be considered....

  19. Hepatorenalt syndrom: diagnose, behandling og forebyggelse

    DEFF Research Database (Denmark)

    Egerod Israelsen, Mads; Gluud, Lise Lotte; Bendtsen, Flemming

    2013-01-01

    Cirrhosis, ascites and renal impairment are associated with high morbidity and mortality. The hepatorenal syndrome (HRS) is a type of renal failure that affects patients with cirrhosis and ascites. This paper provides an update on evidence-based interventions in HRS. A number of factors can...... precipitate HRS. The monitoring, prevention, early detection, and correct treatment of these are essential. Terlipressin combined with albumin is the first-line treatment of type 1 HRS. In type 2 HRS with refractory ascites, liver transplantation and TIPS should be considered....

  20. Immunomodulatory therapy in refractory/recurrent ovarian cancer.

    Science.gov (United States)

    Chen, Chao-Yu; Lai, Chyong-Huey; Yang, Lan-Yan; Tang, Yun-Hsin; Chou, Hung-Hsueh; Chang, Chee-Jen; Lin, Cheng-Tao

    2015-04-01

    To investigate the efficacy and toxicity of immunomodulatory therapy (IMT) alone or as an add-on to palliative/salvage chemotherapy in patients with refractory/recurrent epithelial ovarian cancer (EOC). We retrospectively analyzed the efficacy and toxicity of IMT in 15 patients with refractory/recurrent EOC who had previously received multiple chemotherapy regimens. The median age of the patients was 56 years (range, 41-75 years). Three patients were platinum-sensitive, two were platinum-resistant, and the remaining 10 patients were refractory to platinum-based front-line chemotherapy. IMT consisted of picibanil (OK-432) on Day 1, interleukin-2 and/or interferon-α on Day 2 administered by subcutaneous injection (every week or 2-weekly). Five patients never received metronomic oral cyclophosphamide. After IMT, three patients achieved partial remission (PR, lasting for 11 months, ≥ 12 months, and 16 months), and six patients had stable disease (SD). The disease stabilizing rate (PR+SD) was 60% (3/3 in platinum-sensitive and 6/12 in platinum-resistant/refractory patients). The absolute lymphocyte count (ALC) at 1 month after IMT was significantly higher in the PR+SD group (median 1242.0/μL) than in the progression group (median 325.0/μL) (p = 0.012). No ≥ Grade 3 toxicities were observed. The median post-IMT survival time was 12 months (range, 2-39 months). IMT alone or add-on to palliative/salvage chemotherapy for refractory/recurrent EOC achieves a substantial disease stabilizing rate without severe toxicity, which might be a potential option in selected patients. The ALC 1 month after IMT could be an early indicator to disease stabilization. Copyright © 2015. Published by Elsevier B.V.

  1. A 20-year multicentre outcome analysis of salvage mechanical circulatory support for refractory cardiogenic shock after cardiac surgery

    Directory of Open Access Journals (Sweden)

    Maziar Khorsandi

    2016-11-01

    Full Text Available Abstract Background Refractory post-cardiotomy cardiogenic shock (PCCS is a relatively rare phenomenon that can lead to rapid multi-organ dysfunction syndrome and is almost invariably fatal without advanced mechanical circulatory support (AMCS, namely extra-corporeal membrane oxygenation (ECMO or ventricular assist devices (VAD. In this multicentre observational study we retrospectively analyzed the outcomes of salvage venoarterial ECMO (VA ECMO and VAD for refractory PCCS in the 3 adult cardiothoracic surgery centres in Scotland over a 20-year period. Methods The data was obtained through the Edinburgh, Glasgow and Aberdeen cardiac surgery databases. Our inclusion criteria included any adult patient from April 1995 to April 2015 who had received salvage VA ECMO or VAD for PCCS refractory to intra-aortic balloon pump (IABP and maximal inotropic support following adult cardiac surgery. Results A total of 27 patients met the inclusion criteria. Age range was 34–83 years (median 51 years. There was a large male predominance (n = 23, 85 %. Overall 23 patients (85 % received VA ECMO of which 14 (61 % had central ECMO and 9 (39 % had peripheral ECMO. Four patients (15 % were treated with short-term VAD (BiVAD = 1, RVAD = 1 and LVAD = 2. The most common procedure-related complication was major haemorrhage (n = 10. Renal failure requiring renal replacement therapy (n = 7, fatal stroke (n = 5, septic shock (n = 2, and a pseudo-aneurysm at the femoral artery cannulation site (n = 1 were also observed. Overall survival to hospital discharge was 40.7 %. All survivors were NYHA class I-II at 12 months’ follow-up. Conclusion AMCS for refractory PCCS carries a survival benefit and achieves acceptable functional recovery despite a significant complication rate.

  2. [Therapy-resistant and therapy-refractory arterial hypertension].

    Science.gov (United States)

    Wallbach, M; Koziolek, M J

    2018-05-02

    Therapy-resistant and therapy-refractory arterial hypertension differ in prevalence, pathogenesis, prognosis and therapy. In both cases, a structured approach is required, with the exclusion of pseudoresistance and, subsequently, secondary hypertension. Resistant hypertension has been reported to be more responsive to intensified diuretic therapy, whereas refractory hypertension is presumed to require sympathoinhibitory therapy. Once the general measures and the drug-based step-up therapy have been exhausted, interventional procedures are available.

  3. Refractory myasthenia gravis – clinical profile, comorbidities and response to rituximab

    Science.gov (United States)

    Sudulagunta, Sreenivasa Rao; Sepehrar, Mona; Sodalagunta, Mahesh Babu; Settikere Nataraju, Aravinda; Bangalore Raja, Shiva Kumar; Sathyanarayana, Deepak; Gummadi, Siddharth; Burra, Hemanth Kumar

    2016-01-01

    Introduction: Myasthenia gravis (MG) is an antibody mediated autoimmune neuromuscular disorder characterized by fatigable muscle weakness. A proportion of myasthenia gravis patients are classified as refractory due to non responsiveness to conventional treatment. This retrospective study was done to evaluate clinical profile, epidemiological, laboratory, and features of patients with MG and mode of management using rituximab and complications. Methods: Data of myasthenia gravis patients admitted or presented to outpatient department (previous medical records) with MG between January 2008 and January 2016 were included. A total of 512 patients fulfilled the clinical and diagnostic criteria of myasthenia gravis of which 76 patients met the diagnostic certainty for refractory myasthenia gravis and were evaluated. Results: Out of 76 refractory MG patients, 53 (69.73%) patients fulfilled all the three defined criteria. The median age of onset of the refractory MG group was 36 years with a range of 27–53 years. In our study 25 patients (32.89%) belonged to the age group of 21–30 years. Anti-MuSK antibodies were positive in 8 non-refractory MG patients (2.06%) and 36 refractory MG patients (47.36%). Mean HbA1C was found to be 8.6±2.33. The dose of administered prednisone decreased by a mean of 59.7% (p=3.3x10–8) to 94.6% (p=2.2x10–14) after the third cycle of rituximab treatment. Conclusion: The refractory MG patients are most commonly female with an early age of onset, anti-MuSK antibodies, and thymomas. Refractory MG patients have higher prevalence and poor control (HbA1C >8%) of diabetes mellitus and dyslipidemia probably due to increased steroid usage. Rituximab is very efficient in treatment of refractory MG with adverse effects being low. PMID:27790079

  4. IMPROVED CORROSION RESISTANCE OF ALUMINA REFRACTORIES

    Energy Technology Data Exchange (ETDEWEB)

    John P. Hurley; Patty L. Kleven

    2001-09-30

    The initial objective of this project was to do a literature search to define the problems of refractory selection in the metals and glass industries. The problems fall into three categories: Economic--What do the major problems cost the industries financially? Operational--How do the major problems affect production efficiency and impact the environment? and Scientific--What are the chemical and physical mechanisms that cause the problems to occur? This report presents a summary of these problems. It was used to determine the areas in which the EERC can provide the most assistance through bench-scale and laboratory testing. The final objective of this project was to design and build a bench-scale high-temperature controlled atmosphere dynamic corrosion application furnace (CADCAF). The furnace will be used to evaluate refractory test samples in the presence of flowing corrodents for extended periods, to temperatures of 1600 C under controlled atmospheres. Corrodents will include molten slag, steel, and glass. This test should prove useful for the glass and steel industries when faced with the decision of choosing the best refractory for flowing corrodent conditions.

  5. Clinical and microbiological features of refractory periodontitis subjects.

    Science.gov (United States)

    Colombo, A P; Haffajee, A D; Dewhirst, F E; Paster, B J; Smith, C M; Cugini, M A; Socransky, S S

    1998-02-01

    The purpose of this investigation was to compare the clinical parameters and the site prevalence and levels of 40 subgingival species in successfully treated and refractory periodontitis subjects. 94 subjects received scaling and root planing and if needed, periodontal surgery and systemically administered tetracycline. 28 refractory subjects showed mean full mouth attachment loss and/or > 3 sites showing attachment loss > 2.5 mm within 1 year post-therapy. 66 successfully treated subjects showed mean attachment level gain and no sites with attachment loss > 2.5 mm. Baseline subgingival plaque samples were taken from the mesial aspect of each tooth and the presence and levels of 40 subgingival taxa were determined using whole genomic DNA probes and checkerboard DNA-DNA hybridization. The mean levels and % of sites colonized by each species (prevalence) was computed for each subject and differences between groups sought using the Mann-Whitney test. Most of the 40 species tested, including Actinobacillus actinomycetemcomitans, Porphyromonas gingivalis, Treponema denticola and Bacteroides forsythus, were equally or less prevalent in the refractory group. Prevotella nigrescens was significantly more prevalent in successfully treated subjects, while refractory subjects harbored a larger proportion of Streptococcus species, particularly Streptococcus constellatus. The odds of a subject being refractory was 8.6 (p or = 3.5% of the total DNA probe count. Since few microbiological differences existed between treatment outcome groups using DNA probes to known species, the predominant cultivable microbiota of 33 subgingival samples from 14 refractory subjects was examined. 85% of the 1649 isolates were identified using probes to 69 recognized subgingival species. The remaining unidentified strains were classified by analyzing 16S rRNA gene sequences. Many sequenced isolates were of taxa not considered a common part of the oral microbiota such as Acinetobacter baumanni

  6. Fæcestransplantation som behandling af Clostridium difficile-infektion, colitis ulcerosa og metabolisk syndrom

    DEFF Research Database (Denmark)

    Carstensen, Jeppe West; Hansen, Axel Kornerup

    2014-01-01

    Faecal transplantation as a treatment for Clostridium difficile infection, ulcerative colitis and the metabolic syndrome Faecal transplantation as a therapeutic tool is increasingly reported in the scientific literature. Faecal transplantation is currently becoming a treatment for nosocomial......, refractory infections with C. difficile. Furthermore, faecal transplantation has been suggested as a treatment for ulcerative colitis as well as for the metabolic syndrome. In the accumulated literature faecal transplantations appear to be safe, effective and superior to current treatments. Faecal...... transplantation remains a sparsely investigated treatment, however, especially for other diagnoses than C. difficile infection....

  7. The sleepy teenager - diagnostic challenges

    OpenAIRE

    Anne-Marie eLandtblom; Anne-Marie eLandtblom; Anne-Marie eLandtblom; Anne-Marie eLandtblom; Maria eEngström

    2014-01-01

    The sleepy teenager is a diagnostic challenge because the problems may be physiological or pathological, with behavioural, social and pychological expressions. It is of great importance that health staff that encounter young people with sleep disturbance have good knowledge about the diseases that must be excluded. Narcolepsy, periodic hypersomnia like Kleine Levin syndrome, delayed sleep phase syndrome and obstructive sleep apnoea syndrome, depression and substance use as well as fatigue f...

  8. Surgery for adult patients with obstructive sleep apnoea: A review for general practitioners.

    Science.gov (United States)

    Phan, Nga T; Wallwork, Benjamin; Panizza, Benedict

    2016-08-01

    Obstructive sleep apnoea (OSA) is a complex disease process that involves collapse of the upper airway during sleep and subsequent reduction or cessation of airflow. Continuous positive airway pressure (CPAP) is the primary treatment for OSA and is the recommended first-line treatment for patients with moderate-to-severe forms of the disease. However, some patients are unable to tolerate CPAP or are unwilling to accept it as a form of permanent management. In these cases, surgical management aimed at addressing anatomical obstruction may be useful and warranted. This article presents an overview of the surgical options available for OSA. The review also describes a useful approach for selecting appropriate patients for surgery. On the basis of an OSA model that accounts for observed increased risk of stroke, cardiovascular disease and motor vehicle accidents, there is evidence to support that surgery is beneficial and cost-effective for patients with severe OSA who are intolerant of CPAP. There are many surgical options available for OSA.

  9. [Respiratory manifestations of yellow nail syndrome: report of two cases and literature review].

    Science.gov (United States)

    Li, S; Huang, H; Xu, K; Xu, Z J

    2018-03-12

    Objective: To describe the clinical characteristics of respiratory manifestations of yellow nail syndrome. Methods: We conducted a retrospective analysis of 2 patients with respiratory diseases associated with yellow nail syndrome. Their clinical and chest radiological data were collected. We searched PubMed, Wanfang and CNKI databases with the keywords "yellow nail syndrome, yellow nail and lung" in Chinese and English. And the relevant literatures, including 6 articles in Chinese and 81 articles in English, were reviewed. Results: Our 2 patients were male, one 60 years old and the other 76. Typical yellow nails were present in their fingers, and one of them also showed toe yellow nails. One patient was admitted for refractory respiratory infection and he was diagnosed with diffuse bronchiectasis. The respiratory symptoms could be relieved with antibiotics according to the results of sputum microbiological analysis. The other patient was admitted for cough and exertional dyspnea, and refractory pleural effusions were revealed bilaterally. He received repeated effusion drainage by thoracentesis, and Octreotide was tried recently. A total of 373 cases were reviewed in Chinese and English literatures. Pleural effusions (152 cases) and diffuse bronchiectasis (121 cases) were the most common reported respiratory manifestations. Lymphoedema was present in almost all cases with pleural effusion associated with yellow nail syndrome, and the effusion was usually exudative and lymphocyte predominant. Pleurodesis and decortication were effective for them. But, somatostatin analogues had been tried effectively for these patients recently. On the other hand, literatures showed that diffuse bronchiectasis in yellow nail syndrome was less severe than idiopathic diffuse bronchiectasis, and might benefit from long-term macrolide antibiotics. Conclusions: Yellow nail syndrome is a very rare disorder. Besides yellow nail, respiratory manifestations are the main clinical

  10. Congenital Short QT Syndrome

    Directory of Open Access Journals (Sweden)

    Lia Crotti

    2010-02-01

    Full Text Available The Short QT Syndrome is a recently described new genetic disorder, characterized by abnormally short QT interval, paroxysmal atrial fibrillation and life threatening ventricular arrhythmias. This autosomal dominant syndrome can afflict infants, children, or young adults; often a remarkable family background of cardiac sudden death is elucidated. At electrophysiological study, short atrial and ventricular refractory periods are found, with atrial fibrillation and polymorphic ventricular tachycardia easily induced by programmed electrical stimulation. Gain of function mutations in three genes encoding K+ channels have been identified, explaining the abbreviated repolarization seen in this condition: KCNH2 for Ikr (SQT1, KCNQ1 for Iks (SQT2 and KCNJ2 for Ik1 (SQT3. The currently suggested therapeutic strategy is an ICD implantation, although many concerns exist for asymptomatic patients, especially in pediatric age. Pharmacological treatment is still under evaluation; quinidine has shown to prolong QT and reduce the inducibility of ventricular arrhythmias, but awaits additional confirmatory clinical data.

  11. Defining refractory migraine: results of the RHSIS Survey of American Headache Society members.

    Science.gov (United States)

    Schulman, Elliott A; Peterlin, B Lee; Lake, Alvin E; Lipton, Richard B; Hanlon, Alexandra; Siegel, Sherry; Levin, Morris; Goadsby, Peter J; Markley, Herbert G

    2009-04-01

    To gauge consensus regarding a proposed definition for refractory migraine proposed by Refractory Headache Special Interest Section, and where its use would be most appropriate. Headache experts have long recognized that a subgroup of headache sufferers remains refractory to treatment. Although different groups have proposed criteria to define refractory migraine, the definition remains controversial. The Refractory Headache Special Interest Section of the American Headache Society developed a definition through a consensus process, assisted by a literature review and initial membership survey. A 12-item questionnaire was distributed at the American Headache Society meeting in 2007 during a platform session and at the Refractory Headache Special Interest Section symposium. The same questionnaire was subsequently sent to all American Headache Society members via e-mail. A total of 151 responses from AHS members form the basis of this report. The survey instrument was designed using Survey Monkey. Frequencies and percentages of the survey were used to describe survey responses. American Headache Society members agreed that a definition for refractory migraine is needed (91%) that it should be added to the International Classification of Headache Disorders-2 (86%), and that refractory forms of non-migraine headache disorders should be defined (87%). Responders believed a refractory migraine definition would be of greatest value in selecting patients for clinical drug trials. The current refractory migraine definition requires a diagnosis of migraine, interference with function or quality of life despite modification of lifestyle factors, and adequate trials of acute and preventive medicines with established efficacy. The proposed criteria for the refractory migraine definition require failing 2 preventive medications to meet the threshold for failure. Although 42% of respondents agreed with the working definition of refractory migraine, 43% favored increasing the

  12. The Role of Refractory Dissolved Organic Matter in Ocean Carbon Sequestration

    DEFF Research Database (Denmark)

    Jørgensen, Linda

    The ocean assimilates a large amount of atmospheric CO2 and is potentially a buffer for climate change. A fraction of the assimilated CO2 is incorporated into algal biomass and further converted into refractory dissolved organic matter (DOM). Carbon bound in refractory DOM has the potential...... studies the prokaryotic production and degradation of oceanic refractory DOM and discusses the reasons for the persistent nature of this large DOM fraction. The first two papers investigate the microbial carbon pump, i.e. prokaryotic transfor-mation of organic carbon into refractory DOM. The results show...... DOM compounds in the ocean are rare—possibly too rare to sustain viable uptake and assimilation. Hence, the dilute concentration of individual compounds is a possible explanation for the apparent refractory nature of most DOM in the ocean. Understanding the mechanisms that control the quality...

  13. Blood pressure response to CPAP treatment in subjects with obstructive sleep apnoea: the predictive value of 24-h ambulatory blood pressure monitoring.

    Science.gov (United States)

    Castro-Grattoni, Anabel L; Torres, Gerard; Martínez-Alonso, Montserrat; Barbé, Ferran; Turino, Cecilia; Sánchez-de-la-Torre, Alicia; Cortijo, Anunciacion; Duran-Cantolla, Joaquin; Egea, Carlos; Cao, Gonzalo; Sánchez-de-la-Torre, Manuel

    2017-10-01

    The reduction in blood pressure (BP) with continuous positive airway pressure (CPAP) is modest and highly variable. In this study, we identified the variables that predict BP response to CPAP.24-h ambulatory BP monitoring (ABPM), C-reactive protein (CRP), leptin, adiponectin and 24-h urinary catecholamine were measured before and after 6 months of CPAP in obstructive sleep apnoea (OSA) patients.Overall, 88 middle-aged, obese male patients with severe OSA (median apnoea-hypopnoea index 42 events·h -1 ) were included; 28.4% had hypertension. 62 patients finished the study, and 60 were analysed. The daytime diastolic BP (-2 mmHg) and norepinephrine (-109.5 nmol·day -1 ) were reduced after CPAP, but no changes in the 24-h BP, night-time BP, dopamine, epinephrine, CRP, leptin or adiponectin were detected. The nocturnal normotension was associated with an increased night-time-BP (+4 mmHg) after CPAP, whereas nocturnal hypertension was associated with a reduction of 24-h BP (-3 mmHg). A multivariate linear regression model showed differential night-time BP changes after CPAP. Specifically, low night-time heart rate (CPAP and support the usefulness of 24-h ABPM for OSA patients before treatment initiation. These results need to be confirmed in further studies. Copyright ©ERS 2017.

  14. Intraepithelial lymphocytes in refractory celiac disease : lost in transition

    NARCIS (Netherlands)

    Schmitz, Frederike

    2014-01-01

    Refractory coeliac disease type II (RCDII) is a severe complication of coeliac disease. Whereas celiac disease can successfully be treated by the strict avoidance of gluten, refractory celiac patients show no remission despite a gluten-free diet. The pathology of RCDII is only partially understood,

  15. Complementary effect of fibrinogen and rFVIIa on clotting ex vivo in Bernard-Soulier syndrome and combined use during three deliveries

    DEFF Research Database (Denmark)

    Palsson, Ragnar; Vidarsson, Brynjar; Gudmundsdottir, Brynja R

    2014-01-01

    Women with Bernard-Soulier syndrome (BSS) are considered to be at high risk of serious bleeding during childbirth. Due to the frequently occurring platelet transfusion refractoriness, alternative prophylactic therapy is required. Using rotational thromboelastometry, we evaluated the whole blood......, the findings were confirmed on blood from a non-pregnant woman and three men suffering from BSS. During delivery, bleeding refractory to platelets occurred and immediately following delivery she received both rFVIIa and fibrinogen intravenously. Immediate cessation of bleeding occurred, and no postpartum...

  16. Bi-level CPAP does not improve gas exchange when compared with conventional CPAP for the treatment of neonates recovering from respiratory distress syndrome.

    Science.gov (United States)

    Lampland, Andrea L; Plumm, Brenda; Worwa, Cathy; Meyers, Patricia; Mammel, Mark C

    2015-01-01

    We hypothesised that short-term application of bi-level nasal continuous positive airway pressure CPAP (SiPAP) compared with conventional nasal CPAP (nCPAP) at the same mean airway pressure in infants with persistent oxygen need recovering from respiratory distress syndrome would improve CO2 removal with no change in oxygen requirement. Non-blinded, randomised, observational four-period crossover study. Level III NICU; low-birthweight infants requiring CPAP and oxygen while recovering from respiratory distress syndrome. Infants requiring nasal CPAP for >24 h prior to study enrolment, and fraction of inspired oxygen requirement (FiO2) of 0.25-0.5, were randomised to either nCPAP or SiPAP. A crossover design with four 1 h treatment periods was used such that each infant received both treatments twice. Oxygen saturations (SaO2), transcutaneous CO2 (tcCO2) and vital signs were monitored continuously. Polysomnographic recordings were analysed for apnoea, bradycardia and oxygen desaturation. Twenty low-birthweight infants receiving 0.3±0.04% supplemental oxygen on CPAP of 6 cm H2O were studied at an average of 33 days of age (±23 days, SD). There were no differences in tcCO2 or other physiological parameters except mean blood pressure, which was lower during nCPAP (52.3±8.3 vs 54.4±9.1 mm Hg; ±SD; p<0.01). No differences in short or prolonged apnoea, bradycardia or significant desaturation events were observed. At similar mean airway pressures, SiPAP does not improve CO2 removal, oxygenation or other studied physiological parameters with the exception of mean blood pressure, which was not clinically significant. NCT01053455. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.

  17. Resistant and Refractory Hypertension: Antihypertensive Treatment Resistance vs Treatment Failure

    Science.gov (United States)

    Siddiqui, Mohammed; Dudenbostel, Tanja; Calhoun, David A.

    2017-01-01

    Resistant or difficult to treat hypertension is defined as high blood pressure that remains uncontrolled with 3 or more different antihypertensive medications, including a diuretic. Recent definitions also include controlled blood pressure with use of 4 or more medications as also being resistant to treatment. Recently, refractory hypertension, an extreme phenotype of antihypertensive treatment failure has been defined as hypertension uncontrolled with use of 5 or more antihypertensive agents, including a long-acting thiazide diuretic and a mineralocorticoid receptor antagonist. Patients with resistant vs refractory hypertension share similar characteristics and comorbidities, including obesity, African American race, female sex, diabetes, coronary heart disease, chronic kidney disease, and obstructive sleep apnea. Patients with refractory vs resistant hypertension tend to be younger and are more likely to have been diagnosed with congestive heart failure. Refractory hypertension might also differ from resistant hypertension in terms of underlying cause. Preliminary evidence suggests that refractory hypertension is more likely to be neurogenic in etiology (ie, heightened sympathetic tone), vs a volume-dependent hypertension that is more characteristic of resistant hypertension in general. PMID:26514749

  18. Performance assessment of refractory samples in the Los Alamos Controlled Air Incinerator

    International Nuclear Information System (INIS)

    Hutchins, D.A.; Borduin, L.C.; Koenig, R.A.; Vavruska, J.S.; Warner, C.L.

    1986-01-01

    A refractory evaluation project was initiated in 1979 to study the performance of six selected refractory materials within the Los Alamos Controlled Air Incinerator (CAI). Determining refractory resistance to thermal shock, chemical attack, and plutonium uptake was of particular interest. The experimental refractories were subjected to a variety of waste materials, including transuranic (TRU) contaminated wastes, highly chlorinated compounds and alkaline metal salts of perchlorate, chlorate, nitrate and oxylate, over the six year period of this study. Results of this study to date indicate that the use of high alumina, and possibly specialty plastic refractories, is advisable for the lining of incinerators used for the thermal destruction of diverse chemical compounds. 12 refs., 4 tabs

  19. Trichloroethylene hypersensitivity syndrome: a disease of fatal outcome.

    Science.gov (United States)

    Jung, Hyun Gul; Kim, Hyung Hun; Song, Bong Gun; Kim, Eun Jin

    2012-01-01

    Trichloroethylene is commonly used as an industrial solvent and degreasing agent. The clinical features of acute and chronic intoxication with trichloroethylene are well-known and have been described in many reports, but hypersensitivity syndrome caused by trichloroethylene is rarely encountered. For managing patients with trichloroethylene hypersensitivity syndrome, avoiding trichloroethylene and initiating glucocorticoid have been generally accepted. Generally, glucocorticoid had been tapered as trichloroethylene hypersensitivity syndrome had ameliorated. However, we encountered a typical case of trichloroethylene hypersensitivity syndrome refractory to high dose glucocorticoid treatment. A 54-year-old Korean man developed jaundice, fever, red sore eyes, and generalized erythematous maculopapular rashes. A detailed history revealed occupational exposure to trichloroethylene. After starting intravenous methylprednisolone, his clinical condition improved remarkably, but we could not reduce prednisolone because his liver enzyme and total bilirubin began to rise within 2 days after reducing prednisolone under 60 mg/day. We recommended an extended admission for complete recovery, but the patient decided to leave the hospital against medical advice. The patient visited the emergency department due to pneumonia and developed asystole, which did not respond to resuscitation.

  20. White-Coat Effect Is Uncommon in Patients With Refractory Hypertension.

    Science.gov (United States)

    Siddiqui, Mohammed; Judd, Eric K; Oparil, Suzanne; Calhoun, David A

    2017-09-01

    Refractory hypertension is a recently described phenotype of antihypertensive treatment failure defined as uncontrolled blood pressure (BP) despite the use of ≥5 different antihypertensive agents, including chlorthalidone and spironolactone. Recent studies indicate that refractory hypertension is uncommon, with a prevalence of ≈5% to 10% of patients referred to a hypertension specialty clinic for uncontrolled hypertension. The prevalence of white-coat effect, that is, uncontrolled automated office BP ≥135/85 mm Hg and controlled out-of-office BP hypertensive patients overall is ≈30% to 40%. The prevalence of white-coat effect among patients with refractory hypertension has not been previously reported. In this prospective evaluation, consecutive patients referred to the University of Alabama at Birmingham Hypertension Clinic for uncontrolled hypertension were enrolled. Refractory hypertension was defined as uncontrolled automated office BP ≥135/85 mm Hg with the use of ≥5 antihypertensive agents, including chlorthalidone and spironolactone. Automated office BP measurements were based on 6 serial readings, done automatically with the use of a BpTRU device unobserved in the clinic. Out-of-office BP measurements were done by 24-hour ambulatory BP monitor. Thirty-four patients were diagnosed with refractory hypertension, of whom 31 had adequate ambulatory BP monitor readings. White-coat effect was present in only 2 patients, or 6.5% of the 31 patients with refractory hypertension, suggesting that white-coat effect is largely absent in patients with refractory hypertension. These findings suggest that white-coat effect is not a common cause of apparent lack of BP control in patients failing maximal antihypertensive treatment. © 2017 American Heart Association, Inc.

  1. Friction measurements of steel on refractory bricks

    International Nuclear Information System (INIS)

    Eiselstein, L.E.

    1981-08-01

    During startup or shutdown of a pool-type LMFBR, substantial shear stresses may arise between the base of the steel reactor vessel and the refractory brick support base. The magnitude of these stresses, which result from differences in thermal expansion, can be estimated if the friction coefficient is known. This report describes experiments to determine friction coefficients between 2 1/4 Cr-1Mo steel and several refractory materials and to examine effects to contact pressure, temperature, sliding velocity, lubricants, and surface condition

  2. Characterization of refractory brick based on local raw material from Lampung Province - Indonesia

    Science.gov (United States)

    Amin, Muhammad; Suryana, Yayat I.; Isnugroho, Kusno; Aji, Bramantyo B.; Birawidha, David C.; Hendronursito, Yusup

    2018-04-01

    Refractories are non-metallic inorganic materials that are difficult to melt at high temperatures and used in high-temperature casting industries. Refractories are classified into their constituent mineral feed stocks, refractories having typical plot properties commonly called fire bricks. In the manufacture of refractory bricks that exist in the market during the use of mangrove materials derived from abroad that is from China. In this research the refractory brick materials used are quartz sand, feldspart, kaolin, bentonite, and ball clay. All materials come from local Lampung Province - Indonesia. The experiment, there are 7 kinds of experimental composition, made of plot shape with size 230 mm, 65 mm in thickness, 114 mm height mould using manual press machine with 10 tons power and burning at 1400°C for 5 hours. Refractory brick product is done by physical test in the form of porosity, specific gravity, compressive strength and XRF and SEM characteristics. The result of XRF characteristic of refractory brick composition of 1 to 5 compared to the refractory brick type SK 34 in the market and the result of composition 1 is a composition close to refractory brick composition type SK 34 namely SiO2 is 54.21 %, Al2O3 is 25.38 % and test Physical of Bulk density is 2.25 g/cm3, porosity is 18.98 % and compressive strength is 325 kg/cm2.

  3. Refractory benign esophageal strictures

    Directory of Open Access Journals (Sweden)

    Gopal Goyal

    2016-01-01

    Full Text Available Refractory benign esophageal stricture (RBES is a frequently encountered problem worldwide. These strictures arise from various causes such as corrosive injury, radiation therapy, peptic origin, ablative therapy, and after surgery. Most strictures can be treated successfully with endoscopic dilatation using bougies or balloons, with only a few complications. Those patients who fail after serial dilatation with bougies or balloons will come to the category of refractory strictures. Dilatation combined with intralesional steroid injections can be considered for peptic strictures, whereas incisional therapy has been demonstrated to be effective for short anastomotic strictures. When these therapeutic options do not resolve the stenosis, stent placement should be considered. Self-bougienage can be proposed to a selected group of patients with a proximal stenosis. Most of the patients of RBES respond to above-mentioned treatment and occasional patient may require surgery as the final treatment option. This review aims to provide a comprehensive approach toward endoscopic management of RBESs based on current literature and personal experience.

  4. Illness identity in young adults with refractory epilepsy.

    Science.gov (United States)

    Luyckx, Koen; Oris, Leen; Raymaekers, Koen; Rassart, Jessica; Moons, Philip; Verdyck, Ludo; Mijnster, Teus; Mark, Ruth E

    2018-03-01

    Refractory epilepsy is an intrusive condition with important implications for daily functioning in emerging and young adulthood. The present study examined the degree to which refractory epilepsy is integrated in one's identity, and examined how such a sense of illness identity was related to health-related quality of life (HRQOL). A total of 121 18- to 40-year-old patients with refractory epilepsy (56.2% women) completed self-report questionnaires assessing the four illness identity states of acceptance, enrichment, engulfment, and rejection (Illness Identity Questionnaire (IIQ)); HRQOL (Quality of Life in Epilepsy Inventory - 31); and seizure frequency and severity (Liverpool Seizure Severity Scale (LSSS)). Illness identity scores were compared with a sample of 191 patients with a nonneurological chronic disease (congenital heart disease). Hierarchical regression analyses were conducted to assess the predictive value of illness identity for HRQOL when simultaneously controlling for demographic and clinical features. Patients with refractory epilepsy scored higher on rejection and engulfment and lower on acceptance when compared with patients with congenital heart disease. Further, seizure severity and number of medication side-effects were positively related to engulfment and negatively to acceptance. Finally, when simultaneously controlling for various demographic and clinical variables, illness identity significantly predicted HRQOL (with engulfment being the strongest and most consistent predictor). The extent to which patients with refractory epilepsy succeed in integrating their illness into their identity may have important implications for HRQOL. Clinicians should be especially attentive for signs that patients feel engulfed by their epilepsy. Copyright © 2018 Elsevier Inc. All rights reserved.

  5. Healthcare transition in persons with intellectual disabilities: general issues, the Maastricht model, and Prader-Willi syndrome.

    Science.gov (United States)

    Schrander-Stumpel, Constance T R M; Sinnema, Margje; van den Hout, Lieke; Maaskant, Marian A; van Schrojenstein Lantman-de Valk, Henny M J; Wagemans, Annemieke; Schrander, Jaap J P; Curfs, Leopold M G

    2007-08-15

    In current healthcare, transitional healthcare is a very important and timely issue. Thanks to the major advances made in medical care and technology, many children with childhood onset diseases and/or genetic syndromes survive to adulthood. These children are at risk of not being provided with adequate healthcare as they reach adulthood. Healthcare transition is an essential part of healthcare provision, referred to as the shift from one type of healthcare to another. In Maastricht, we developed a transition/out clinic led by a medical doctor specialized in persons with intellectual disability (ID), together with a clinical geneticist. We aim to coordinate healthcare issues based on guidelines if available. Also questions concerning living, daily activities, relations, sexuality, and sterilization can be discussed. The aging process of persons with ID has been a topic of interest in recent years. Little is known about the aging process of people with specific syndromes, except for persons with Down syndrome. We present some data of a recent questionnaire study in persons with Prader-Willi syndrome. In only 50% in persons with a clinical diagnosis genetic test results could be reported. The majority of persons were obese. Diabetes mellitus, hypertension, skin problems, sleep apnea, and hormonal problems like osteoporosis and hypothyroidism were common. Psychiatric problems were frequent, especially in the persons with uniparental disomy. Osteoporosis and sleep apnoea seem to be underestimated. Further longitudinal research is necessary for a better understanding of the aging process in PWS. (c) 2007 Wiley-Liss, Inc.

  6. Mitochondrial Dysfunction in Metabolic Syndrome and Asthma

    Science.gov (United States)

    Mabalirajan, Ulaganathan; Ghosh, Balaram

    2013-01-01

    Though severe or refractory asthma merely affects less than 10% of asthma population, it consumes significant health resources and contributes significant morbidity and mortality. Severe asthma does not fell in the routine definition of asthma and requires alternative treatment strategies. It has been observed that asthma severity increases with higher body mass index. The obese-asthmatics, in general, have the features of metabolic syndrome and are progressively causing a significant burden for both developed and developing countries thanks to the westernization of the world. As most of the features of metabolic syndrome seem to be originated from central obesity, the underlying mechanisms for metabolic syndrome could help us to understand the pathobiology of obese-asthma condition. While mitochondrial dysfunction is the common factor for most of the risk factors of metabolic syndrome, such as central obesity, dyslipidemia, hypertension, insulin resistance, and type 2 diabetes, the involvement of mitochondria in obese-asthma pathogenesis seems to be important as mitochondrial dysfunction has recently been shown to be involved in airway epithelial injury and asthma pathogenesis. This review discusses current understanding of the overlapping features between metabolic syndrome and asthma in relation to mitochondrial structural and functional alterations with an aim to uncover mechanisms for obese-asthma. PMID:23840225

  7. A Boy with Relentless Pruritus: Job's Syndrome.

    Science.gov (United States)

    Khan, Kamran; Wozniak, Susan E; Giannone, Anna Lucia; Abdulmassih, Maria Elena

    2016-02-21

    Job's syndrome (hyper IgE syndrome) is a very rare primary immunodeficiency disease that has an annual approximate incidence of less than 1/1,000,000. This manuscript aims to provide education regarding diagnosis and management strategies of this syndrome worldwide. A 6-year-old boy was seen at the clinic secondary to persistent pruritus interfering with sleep. At the age of 2 months, the patient developed diffuse eczematous and desquamating skin lesions. He was subsequently diagnosed with atopic dermatitis and managed conservatively. From 2 months to 7 years of age, intermittent exacerbations of dermatitis persisted despite an aggressive treatment regimen. The serum IgE level increased exponentially over a period of 7 years, with a peak value of 57,400 IU/ml. Molecular genetic testing revealed a dominant negative mutation within the SH2 domain of the Signal Transducer and Activator of Transcription (STAT3) gene. The patient was subsequently diagnosed with Job's syndrome. Management included proper skin care, prophylactic antibiotics, immunomodulating agents, and psychotherapy. Job's syndrome can often go unrecognized and masquerade as atopic dermatitis. Therefore, genetic testing for this condition should be obtained in all patients with treatment-refractory AD. Additionally, psychotherapy can be a successful management strategy for the grating psychological impact that can be imposed on children with excessive pruritus.

  8. Reproducibility of the World Health Organization 2008 criteria for myelodysplastic syndromes.

    Science.gov (United States)

    Senent, Leonor; Arenillas, Leonor; Luño, Elisa; Ruiz, Juan C; Sanz, Guillermo; Florensa, Lourdes

    2013-04-01

    The reproducibility of the World Health Organization 2008 classification for myelodysplastic syndromes is uncertain and its assessment was the major aim of this study. The different peripheral blood and bone marrow variables required for an adequate morphological classification were blindly evaluated by four cytomorphologists in samples from 50 patients with myelodysplastic syndromes. The degree of agreement among observers was calculated using intraclass correlation coefficient and the generalized kappa statistic for multiple raters. The degree of agreement for the percentages of blasts in bone marrow and peripheral blood, ring sideroblasts in bone marrow, and erythroid, granulocytic and megakaryocytic dysplastic cells was strong (P<0.001 in all instances). After stratifying the percentages according to the categories required for the assignment of World Health Organization subtypes, the degree of agreement was not statistically significant for cases with 5-9% blasts in bone marrow (P=0.07), 0.1-1% blasts in peripheral blood (P=0.47), or percentage of erythroid dysplastic cells (P=0.49). Finally, the interobserver concordance for World Health Organization-defined subtypes showed a moderate overall agreement (P<0.001), the reproducibility being lower for cases with refractory anemia with excess of blasts type 1 (P=0.05) and refractory anemia with ring sideroblasts (P=0.09). In conclusion, the reproducibility of the World Health Organization 2008 classification for myelodysplastic syndromes is acceptable but the defining criteria for blast cells and features of erythroid dysplasia need to be refined.

  9. Sexual function in male patients with obstructive sleep apnoea after 1 year of CPAP treatment

    DEFF Research Database (Denmark)

    Petersen, Marian Christin; Kristensen, Ellids; Berg, Søren

    2012-01-01

    OBJECTIVE: Our objective was to investigate what impact 1 year of effective nocturnal continuous positive airway pressure (CPAP) treatment had on general and functional aspects of sexuality in male patients with a confirmed diagnosis of obstructive sleep apnoea (OSA). METHODS: Before and after 1...... year of CPAP treatment, a total of 207 CPAP-compliant male patients (age 26-77) received a survey with questions drawn from two self-administered questionnaires on sexuality - Life Satisfaction 11 (LiSat-11) and brief sexual function inventory (BSFI). For assessment of daytime sleepiness, we used...... of CPAP treatment. ESS score decreased significantly after 1 year of CPAP treatment. CONCLUSION: One year of CPAP treatment improves all aspects of sexual function in male patients with OSA. Our data indirectly suggest that organic factors are the most likely explanation to these improvements....

  10. T-cell receptor Vbeta CDR3 oligoclonality frequently occurs in childhood refractory cytopenia (MDS-RC) and severe aplastic anemia

    DEFF Research Database (Denmark)

    Vries, A.C. de; Langerak, A.W.; Verhaaf, B.

    2008-01-01

    (Very) severe acquired aplastic anemia ((v)SAA) and myelodysplastic syndrome (MDS) are rare diseases in childhood. (V)SAA is a bone marrow (BM) failure syndrome characterized by immune-mediated destruction of hematopoietic progenitors. MDS is a malignant clonal stem cell disorder, of which...... the hypoplastic variant is, in case of absence of a cytogenetic clone, difficult to separate from (v)SAA. Recently, studies provided a molecular signature of autoimmunity in adult (v)SAA, by showing oligoclonality based on the length of the TCR Vbeta CDR3 region. We investigated retrospectively the frequency...... and the discriminative value of TCR Vbeta CDR3 oligoclonality in pediatric (v)SAA and MDS patients. Peripheral blood (PB) and/or BM mononuclear cell samples of pediatric patients with (v)SAA (n=38), refractory cytopenia (MDS-RC) (n=28) and 18 controls were analysed via TCR Vbeta heteroduplex PCR analysis of extracted...

  11. [Hantavirus pulmonary syndrome (Rio Mamore virus) in the Peruvian Amazon region].

    Science.gov (United States)

    Casapía, Martín; Mamani, Enrique; García, María P; Miraval, María L; Valencia, Pedro; Quino, Alberto H; Alvarez, Carlos; Donaires, Luis F

    2012-01-01

    Hantavirus infection is a viral zoonotic infection borne by rodents which most letal form clinical is the Hantavirus Pulmonary Syndrome (SPH, Spanish abbreviation). The Mamore River variant originates in South America and was found in rodents without any association to human diseases. Two cases of SPH were identified in the Peruvian Amazon region in November 2011. In both cases, a molecular diagnostic testing was conducted by the Instituto Nacional de Salud from Peru. A phylogenetic analysis of a viral genome fragment and a histopathological evaluation were conducted. Both patients developed adult respiratory distress syndrome and refractory shock. A patient died and another one recovered 12 days later.

  12. Clinical outcome and risk stratification in Brugada syndrome

    Directory of Open Access Journals (Sweden)

    Tadashi Wada, MD

    2013-04-01

    Full Text Available Since the first report on Brugada syndrome, various risk markers for the prediction of ventricular fibrillation (VF in patients with Brugada syndrome have been reported. Multicenter trials reported spontaneous type 1 electrocardiogram (ECG and disease symptoms as prognostic predictors. VF induction by programmed electrical stimulation is still controversial, and most of the studies have failed to prove its significance for the prediction of spontaneous VF episodes. In Japan, although most multicenter studies have shown that patients with type 1 ECG were at high risk, it is difficult to determine the indication for implantation of an implantable cardioverter defibrillator only based on the ECG type. Recent studies have added new risk markers, such as inferolateral early repolarization, fragmented QRS, and shorter effective refractory periods of the ventricle, in addition to type 1 ECG and symptoms. Here, we review the clinical outcome and indices reported as reliable prognostic factors of Brugada syndrome with a focus on the clinical and ECG markers for risk stratification.

  13. Central Diabetes Insipidus in Refractory Antineutrophil Cytoplasmic Antibody-associated Vasculitis.

    Science.gov (United States)

    Ohashi, Keiji; Morishita, Michiko; Watanabe, Haruki; Sada, Ken-Ei; Katsuyama, Takayuki; Miyawaki, Yoshia; Katsuyama, Eri; Narazaki, Mariko; Tatebe, Noriko; Watanabe, Katsue; Kawabata, Tomoko; Wada, Jun

    2017-11-01

    We herein describe two cases of refractory antineutrophil cytoplasmic antibody-associated vasculitis (AAV) complicated with diabetes insipidus (DI) possibly related to hypertrophic pachymeningitis (HP). One patient had microscopic polyangiitis and HP, which were refractory to cyclophosphamide, azathioprine, rituximab, mycophenolate mofetil (MMF), and mizoribine. Remission was finally achieved with the use of etanercept, but DI occurred 5 years later. The other patient had granulomatosis with polyangiitis, which that was refractory to cyclophosphamide, methotrexate, MMF, and rituximab. DI subsequently developed, but was successfully treated with etanercept. Dura mater hypertrophy was macroscopically observed in the latter case.

  14. A Review on Recent Advances on Magnesia-Doloma Refractories by Nano-Technology.

    Directory of Open Access Journals (Sweden)

    Salman Ghasemi-Kahrizsangi

    2017-07-01

    Full Text Available Due to exhibiting an excellent chemical resistance against basic environments at high temperature, good thermal shock resistance, thermodynamic stability in the presence of carbon, and a suitable abrasion resistance, MgO-CaO refractories are widely used in argon-oxygen decarburization furnaces in the metallurgy industry and cement rotary kilns. Furthermore, MgO-CaO refractories are beneficial to removing inclusions from molten steels; thus, they have been considered to be one of the effective refractory types for processing clean steel products. Also, MgO-CaO materials have become one of the attractive steelmaking refractories because of their low cost and high ore reserves.  However, in spite of these primary advantages, the application of MgO-CaO refractories has not been popular due to its tendency to hydration when exposed to the atmosphere.  In world most of MgO-CaO bricks producers used of organic components such as tar, pitch, and peck for produce MgO-CaO refractories. But during the application of these bricks in steel and cement and industrials, they released CO and CO2 gases to air and pollute the atmosphere. For this reason, recently some researcher investigate the effect of additive nanoparticles on MgO-CaO refractories performance. They reported the used of Nano-additive have acceptable results and additive nanoparticles can replace by aforementioned environment contaminating organic compounds. In this study, we reviewed all effort that done for improving the hydration resistance of MgO-CaO refractories by application of Nano-additives with an emphasis on the beneficial the use of additives nanoparticle for reduction of environmental pollution by various industries which used of MgO-CaO refractory bricks.

  15. Refractory Research Group - U.S. DOE, Albany Research Center [Institution Profile

    Energy Technology Data Exchange (ETDEWEB)

    Bennett, James P.

    2004-09-01

    The refractory research group at the Albany Research Center (ARC) has a long history of conducting materials research within the U.S. Bureau of Mines, and more recently, within the U.S. Dept. of Energy. When under the U.S. Bureau of Mines, research was driven by national needs to develop substitute materials and to conserve raw materials. This mission was accomplished by improving refractory material properties and/or by recycling refractories using critical and strategic materials. Currently, as a U.S. Dept of Energy Fossil Energy field site, research is driven primarily by the need to assist DOE in meeting its vision to develop economically and environmentally viable technologies for the production of electricity from fossil fuels. Research at ARC impacts this vision by: • Providing information on the performance characteristics of materials being specified for the current generation of power systems; • Developing cost-effective, high performance materials for inclusion in the next generation of fossil power systems; and • Solving environmental emission and waste problems related to fossil energy systems. A brief history of past refractory research within the U.S. Bureau of Mines, the current refractory research at ARC, and the equipment and capabilities used to conduct refractory research at ARC will be discussed.

  16. Computed tomographic angiography study of the relationship between the lingual artery and lingual markers in patients with obstructive sleep apnoea

    Energy Technology Data Exchange (ETDEWEB)

    Hou, T.-N., E-mail: dr-htn@hotmail.co [Department of Otolaryngology Head and Neck Surgery, Affiliated SIR RUN RUN SHAW Hospital, Zhejiang University, Hangzhou 310016 (China); Zhou, L.-N.; Hu, H.-J. [Department of Otolaryngology Head and Neck Surgery, Affiliated SIR RUN RUN SHAW Hospital, Zhejiang University, Hangzhou 310016 (China)

    2011-06-15

    Aim: To determine the relationship between the lingual artery and lingual markers for preoperative evaluation of the lingual artery in patients with obstructive sleep apnoea/hypopnoea syndrome (OSAHS). Methods: A 16-section computed tomographic angiography (CTA) of the lingual artery was performed in 87 inpatient cases with OSAHS, from December 2007 to May 2009. The course of the lingual artery and the anatomic relationship between the lingual artery and the lingual markers were analyzed using CTA imaging. Results: The course of the lingual artery with the tongue in a resting position was similar to that of the Big Dipper constellation (Plough) in the sagittal view of CTA imaging. The first segment of the lingual artery declined approximately 19.27 {+-} 5.24 mm, the middle segment of the lingual artery was forward approximately 19.30 {+-} 6.79 mm, and the ascending segment of the lingual artery rose approximately 52.49 {+-} 10.98 mm. The entry point where the lingual artery entered into the tongue was adjacent to the tip of the greater horn of the hyoid bone. The relationship between the second segment of the lingual artery and the greater horn of the hyoid bone was relatively steady with the tongue in whatever position. The interval between the bilateral greater horn of the hyoid bone equalled that between the bilateral lingual arteries. Conclusions: Recognizing some lingual markers in the patients with OSAHS, such as the greater horn of the hyoid bone, foramen cecum, circumvallate papilla, lingual vein and tongue midline, may facilitate the surgeon's ability to define the course of the lingual artery accurately in the treatment of OSAHS.

  17. Twin infant with lymphatic dysplasia diagnosed with Noonan syndrome by molecular genetic testing.

    Science.gov (United States)

    Mathur, Deepan; Somashekar, Santhosh; Navarrete, Cristina; Rodriguez, Maria M

    2014-08-01

    Noonan Syndrome is an autosomal dominant disorder characterized by short stature, congenital heart defects, developmental delay, dysmorphic facial features and occasional lymphatic dysplasias. The features of Noonan Syndrome change with age and have variable expression. The diagnosis has historically been based on clinical grounds. We describe a child that was born with congenital refractory chylothorax and subcutaneous edema suspected to be secondary to pulmonary lymphangiectasis. The infant died of respiratory failure and anasarca at 80 days. The autopsy confirmed lymphatic dysplasia in lungs and mesentery. The baby had no dysmorphic facial features and was diagnosed postmortem with Noonan syndrome by genomic DNA sequence analysis as he had a heterozygous mutation for G503R in the PTPN11 gene.

  18. Testing of the melter lid refractory for the West Valley Demonstration Project (WVDP)

    International Nuclear Information System (INIS)

    Gupta, A.; Jain, V.; Mahoney, J.L.; Holman, T.M.

    1991-01-01

    Monofrax H and Mulfrax 202 refractory were tested for potential application as the melter lid refractory for the WVDP. Resistance to spalling and corrosion by the slurry and offgas salts were primary criteria for selection. Test specimens were subjected to thermal cycling between 450 and 1,100C for five weeks. Visual examination indicated some corrosion but no spalling. SEM/EDS analysis was performed to determine the glass/refractory interface corrosion mechanism. The refractory selection basis will be discussed

  19. Electrophysiological effects of desflurane in children with Wolff-Parkinson-White syndrome: a randomized crossover study.

    Science.gov (United States)

    Hino, H; Oda, Y; Yoshida, Y; Suzuki, T; Shimada, M; Nishikawa, K

    2018-02-01

    We hypothesized that, compared with propofol, desflurane prolongs the antegrade accessory pathway effective refractory period (APERP) in children undergoing radiofrequency catheter ablation for Wolff-Parkinson-White (WPW) syndrome. In this randomized crossover study, children aged 4.1-16.1 years undergoing radiofrequency catheter ablation for WPW syndrome were randomly divided into four groups according to the concentration of desflurane and anesthetics used in the first and the second electrophysiological studies (EPS). After induction of general anesthesia with propofol and tracheal intubation, they received one of the following regimens: 0.5 minimum alveolar concentration (MAC) desflurane (first EPS) and propofol (second EPS) (Des0.5-Prop group, n = 8); propofol (first EPS) and 0.5 MAC desflurane (second EPS) (Prop-Des0.5 group, n = 9); 1 MAC desflurane (first EPS) and propofol (second EPS) (Des1.0-Prop group, n = 10); propofol (first EPS) and 1 MAC desflurane (second EPS) (Prop-Des1.0 group, n = 9). Radiofrequency catheter ablation was performed upon completion of EPS. Sample size was determined to detect a difference in the APERP. Desflurane at 1.0 MAC significantly prolonged the APERP compared with propofol, but did not affect the sinoatrial conduction time, atrio-His interval or atrioventricular node effective refractory period. Supraventricular tachycardia was induced in all children receiving propofol, but not induced in 1 and 4 children receiving 0.5 MAC and 1.0 MAC desflurane, respectively. Desflurane enhances the refractoriness and may block the electrical conduction of the atrioventricular accessory pathway, and is therefore not suitable for use in children undergoing radiofrequency catheter ablation for WPW syndrome. © 2017 The Acta Anaesthesiologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.

  20. Pathophysiology and management of thrombocytopenia in bone marrow failure: possible clinical applications of TPO receptor agonists in aplastic anemia and myelodysplastic syndromes

    Science.gov (United States)

    Desmond, Ronan; Dunbar, Cynthia E.; Young, Neal S.

    2014-01-01

    Aplastic anemia is a bone marrow failure syndrome that causes pancytopenia and can lead to life-threatening complications. Bone marrow transplantation remains the standard of care for younger patients and those with a good performance status but many patients may not have a suitable donor. Immunosuppressive therapy is able to resolve cytopenias in a majority of patients with aplastic anemia but relapses are not uncommon and some patients remain refractory to this approach. Patients may require frequent blood and platelet transfusion support which is expensive and inconvenient. Life-threatening bleeding complications still occur despite prophylactic platelet transfusion. Thrombopoietin (TPO) mimetics, such as romiplostim and eltrombopag, were developed to treat patients with refractory immune thrombocytopenia but are now being investigated for the treatment of bone marrow failure syndromes. TPO is the main regulator for platelet production and its receptor (c-Mpl) is present on megakaryocytes and hematopoietic stem cells. Trilineage hematopoietic responses were observed in a recent clinical trial using eltrombopag in patients with severe aplastic anemia refractory to immunosuppression suggesting that these agents can provide a new therapeutic option for enhancing blood production. In this review, we discuss these recent results and ongoing investigation of TPO mimetics for aplastic anemia and other bone marrow failure states like myelodysplastic syndromes. Clonal evolution or progression to acute myeloid leukemia remains a concern when using these drugs in bone marrow failure and patients should only be treated in the setting of a clinical trial. PMID:23690288

  1. Effectiveness of botulinum toxin A in treatment of refractory erythromelalgia

    Directory of Open Access Journals (Sweden)

    Kuan-Hsiang Lin

    2013-05-01

    Full Text Available Erythromelalgia is characterized by intense burning pain, erythema, and heat in affected areas after precipitating factors such as warm temperature or stress. It is refractory to treatment in some situations. We describe a woman with adenosquamous cell carcinoma of the lung and medically refractory erythromelalgia. The symptoms of erythromelalgia presented as refractory to any medical treatment. Due to the unresponsive nature of her condition, botulinum toxin type A (onabotulinumtoxin A was injected over both of her cheeks, periodically for six cycles. Her symptoms responded dramatically to subcutaneous and intradermal injection of botulinum toxin type A. Repetitive injection demonstrated consistent and reproducible responses, and the efficacy was maintained for approximately 1 month. No adverse effects or complications were noted. Botulinum toxin type A might be safe and effective as an alternative treatment for refractory erythromelalgia, but further large-scale studies are required.

  2. Pamidronate Rescue Therapy for Hypercalcemia in a Child With Williams Syndrome

    Directory of Open Access Journals (Sweden)

    Sami A. Sanjad

    2018-05-01

    Full Text Available A 15-month-old male infant diagnosed with Williams Syndrome (WS was admitted with severe hypercalcemia and nephrocalcinosis. Intravenous hydration and furosemide failed to yield an appreciable and sustainable fall in serum calcium, while the injection of pamidronate achieved a significant decrease in serum calcium in a short period of time. This bisphosphonate could be considered as a second-line treatment for refractory hypercalcemia in WS.

  3. Theoretical and practical aspects about corrosion of refractories used in steel metallurgy: part 3: characterization of commercial refractories; Aspectos teoricos e praticos sobre a corrosao de refratarios utilizados na metalurgia: caracterizacao de refratarios comerciais

    Energy Technology Data Exchange (ETDEWEB)

    Braganca, S.R., E-mail: saulorb@ufrgs.br [Universidade Federal do Rio Grande Sul (DEMAT/UFRGS), RS (Brazil)

    2012-07-01

    In this study, it was reviewed the main aspects found in the literature about refractories corrosion, evaluating the feasibility of certain tests and relating them with experimental results. The physical properties and microstructure of commercial refractories were analyzed, considering the differences between them and the quality implications and probable life of the refractory. Thus, it was studied the various types of refractories used as lining on steel ladle. Magnesia-carbon and doloma-carbon refractories were analyzed, highlighting the differences between them. The examined refractory showed characteristics favoring high resistance to corrosion process, presenting a series of properties to be selected in accordance with industry practice. (author)

  4. [Burning mouth syndrome - a joint biopsychosocial approach].

    Science.gov (United States)

    Arpone, Francesca; Combremont, Florian; Weber, Kerstin; Scolozzi, Paolo

    2016-02-10

    Burning mouth syndrome (BMS) is a medical condition that is often refractory to conventional diagnostic and therapeutic methods. Patients suffering from BMS can benefit from a biopsychosocial approach in a joint, medical-psychological consultation model. Such a consultation exists at Geneva University Hospitals, involving the collaboration of the maxillo-facial and oral surgery division and the division of liaison psychiatry and crisis intervention, in order to take into account the multiple factors involved in BMS onset and persistence. This article will describe BMS clinical presentation, and present an integrate approach to treat these patients.

  5. Interventional embolization for refractory hyperthyroidism

    International Nuclear Information System (INIS)

    Jiang Guomin; Zhang Yunping; Chen Yaxian; Zhao Jinwei; Yang Weihuai; Chen Jinluo

    2001-01-01

    Objective: To evaluate the clinical effects of the thyroid artery embolization for refractory hyperthyroidism. Methods: Super selective catheterization and interventional embolization of thyroid superior and inferior arteries were performed with PVA microspheres and Gelfoam particles. Changes of function and size of thyroid were investigated after embolization. Results: The procedures were succeeded in all 21 patients. Followed up by 3-12 months, serum level of thyroid hormones dropped significantly (median FT 3 from 17.1 pmol/L to 7.44 pmol/L, median FT 4 from 51.1 pmol/L to 23.3 pmol/L, P< 0.01). The size of thyroid glands decreased remarkably. Symptoms of hyperthyroidism were controlled in 4 patients and were effectively controlled through low dose antithyroid medication in 17 patients. No serious complications occurred. Conclusions: Thyroid artery embolization is a new safe and effective method for refractory hyperthyroidism

  6. Efficacy of infliximab in refractory posterior uveitis in Behcet's disease patients

    OpenAIRE

    Ayman K. El Garf; Amira A. Shahin; Sherif A. Shawky; Mohammed A. Azim; Dina A. Effat; Sherry K. Abdelrahman

    2018-01-01

    Aim of the work: Ocular manifestations are the main cause of morbidity in Behcet's disease (BD). Infliximab (IFX), a chimeric monoclonal antibody directed against tumor necrosis factor-alpha, may be efficient in refractory uveitis due to BD. The aim of this study was to assess the efficacy and safety of IFX in the treatment of patients with BD-associated refractory posterior uveitis (PU). Patient and Methods: Twenty patients with refractory Behcet's PU received IFX therapy as intravenous infu...

  7. Treatment for Relapsed/Refractory AML Based on a High Throughput Drug Sensitivity Assay

    Science.gov (United States)

    2018-04-11

    Adult Acute Megakaryoblastic Leukemia (M7); Adult Acute Minimally Differentiated Myeloid Leukemia (M0); Adult Acute Monoblastic Leukemia (M5a); Adult Acute Monocytic Leukemia (M5b); Adult Acute Myeloblastic Leukemia With Maturation (M2); Adult Acute Myeloblastic Leukemia Without Maturation (M1); Adult Acute Myeloid Leukemia With 11q23 (MLL) Abnormalities; Adult Acute Myeloid Leukemia With Del(5q); Adult Acute Myeloid Leukemia With Inv(16)(p13;q22); Adult Acute Myeloid Leukemia With t(16;16)(p13;q22); Adult Acute Myeloid Leukemia With t(8;21)(q22;q22); Adult Acute Myelomonocytic Leukemia (M4); Adult Erythroleukemia (M6a); Adult Pure Erythroid Leukemia (M6b); Chronic Myelomonocytic Leukemia; Myelodysplastic/Myeloproliferative Neoplasm, Unclassifiable; Previously Treated Myelodysplastic Syndromes; Recurrent Adult Acute Myeloid Leukemia; Refractory Anemia With Excess Blasts

  8. Ketogenic diet in 3 cases of childhood refractory status epilepticus

    DEFF Research Database (Denmark)

    Sort, Rune; Born, Alfred P; Pedersen, Karen N.

    2013-01-01

    Refractory status epilepticus (RSE) in children is associated with a significant risk of death or neurological morbidity. Recently attention has been drawn to the ketogenic diet (KD) as an acute treatment, as it has shown promise in controlling seizures in otherwise refractory status epilepticus...

  9. Perspectives on environmental protection of refractory metals

    International Nuclear Information System (INIS)

    Perking, R.A.

    1992-01-01

    Alloys of refractory metals which combine high strength to weight ratios with useful low-temperature toughness and ductility have been designed to meet the requirements for the next generation of high-temperature aerospace structural materials with one exception: long term resistance to oxidation. It is considered unlikely that refractory metal alloys can be modified to possess useful resistance to oxidation as bulk materials or that coating can be designed to provide high reliability fail-safe protection for the structural alloys developed to date. Recent developments indicate that bulk alloys of W and Mo can be designed to possess short term resistance to oxidation, providing a base material which can be coated to extend useful life with fail-safe protection in the event of random coating failures. Current research on silicide coatings indicates that significant improvements in coating reliability and performance also are feasible. The technical basis for these conclusions is presented in this paper and the direction of future work that could lead to environmentally stable refractory metal alloy/coating systems is discussed

  10. Super Refractory Status Epilepticus

    African Journals Online (AJOL)

    user

    et al did retrospective cohort study from 1 January st. 1994 to 31 March 1998 at Presbyterian Medical. Centre in Columbia, to determine the frequency, risk factors and impact on the outcome of RSE. They found out that 69% of seizures recurred after. Key Words: Super refractory status epilepticus, Zambia. Medical Journal of ...

  11. Process technology - rare and refractory metals

    International Nuclear Information System (INIS)

    Gupta, C.K.; Bose, D.K.

    1989-01-01

    India has fairly rich resreves of rare and refractory metals. Abundant sources of ilmenite, rutile, zircon and rare earths are found in the placer deposits of the southern and eastern coasts of the country. Columbite-tantalite occur in mica and the mining belts of Bihar and cassiterite deposits are found in Bastar (Madhya Pradesh). Vanadium as a minor associate occurs in bauxites and in the vast deposits of titaniferrous magnetites. Over the years, research and development and pilot plant works in many research organisations in India have built up a sound technological base in the country for process metallurgy of many refractory and rare earth metals starting from their indigenous sources. The present paper provides a comprehensive view of the developments that have taken place till now on the processing of various refractory and rare earth metals with particular reference to the extensive work carried out at the Department of Atomic Energy. The coverage includes mineral benification separation of individual elements, preparation of pure intermediates, techniques of reduction to metal and final purification. The paper also reviews some of the recent developments that have been taken place in these fields and the potential application of these metals in the foreseeable future. (author). 22 refs., 18 fi g., 7 tabs

  12. Nasal inflammation in sleep apnoea patients using CPAP and effect of heated humidification.

    Science.gov (United States)

    Koutsourelakis, I; Vagiakis, E; Perraki, E; Karatza, M; Magkou, C; Kopaka, M; Roussos, C; Zakynthinos, S

    2011-03-01

    Nasal continuous positive airway pressure (CPAP) can cause undesirable nasal symptoms, such as congestion to obstructive sleep apnoea (OSA) patients, whose symptoms can be attenuated by the addition of heated humidification. However, neither the nature of nasal symptoms nor the effect of heated humidification on nasal pathophysiology and pathology are convincingly known. 20 patients with OSA on nasal CPAP who exhibited symptomatic nasal obstruction were randomised to receive either 3 weeks of CPAP treatment with heated humidification or 3 weeks of CPAP treatment with sham-heated humidification, followed by 3 weeks of the opposite treatment, respectively. Nasal symptom score, nasal resistance, nasal lavage interleukin-6, interleukin-12 and tumour necrosis factor-α and nasal mucosa histopathology were assessed at baseline and after each treatment arm. Heated humidification in comparison with sham-heated humidification was associated with decrease in nasal symptomatology, resistance and lavage cytokines, and attenuation of inflammatory cell infiltration and fibrosis of the nasal mucosa. In conclusion, nasal obstruction of OSA patients on CPAP treatment is inflammatory in origin and the addition of heated humidification decreases nasal resistance and mucosal inflammation.

  13. Reuse of ultrafine mineral wool production waste in the manufacture of refractory concrete.

    Science.gov (United States)

    Stonys, R; Kuznetsov, D; Krasnikovs, A; Škamat, J; Baltakys, K; Antonovič, V; Černašėjus, O

    2016-07-01

    The paper deals with the mineral wool production waste (cupola dust - CD), presents CD characterization and aims to reuse CD in production of refractory concrete with calcium aluminate cement. The study of CD covers its chemical, phase and thermal analyses along with the morphological study and determination of particles size distribution. Zeta-potential, electrical conductivity and pH values of CD suspension are presented in the paper as well. Commercial microsilica additive in refractory concrete has been replaced with cupola dust. Compositions of refractory concrete have been prepared by incorporating 1%, 2% and 3% of CD. The bulk density, ultrasonic wave velocity, cold crushing strength and thermal shock resistance of the created refractory concrete have been determined. Based on experimental results, it has been found that cupola dust may be used for the production of refractory concrete. The environmental impact related to the CD reuse in refractory concrete production has been evaluated as well. Copyright © 2016 Elsevier Ltd. All rights reserved.

  14. When myasthenia gravis is deemed refractory: clinical signposts and treatment strategies

    Science.gov (United States)

    Mantegazza, Renato; Antozzi, Carlo

    2018-01-01

    The prognosis for patients with myasthenia gravis (MG) has improved significantly over the past half century, including substantial reductions in mortality and morbidity. However, approximately 10% of patients fails to respond adequately to current therapies and are considered treatment refractory, or treatment intolerant, and up to 80% have disease that fails to achieve complete stable remission. Although patients with autoantibodies to muscle-specific tyrosine kinase (anti-MuSK positive) are more likely to become treatment refractory than those with autoantibodies to the acetylcholine receptor (anti-AChR positive), each of these serotypes is substantially represented in the refractory MG population. Other risk factors for becoming treatment refractory include history of thymoma or thymectomy and female sex. A modified treatment algorithm for MG is proposed: patients who have disease that fails to respond to the stepwise approach to therapy, are treatment intolerant, or who require chronic rescue measures despite ongoing therapy, should be considered treatment refractory and emerging therapies should be considered. Three emerging monoclonal antibody-based therapies are discussed: the anti-B-cell agent rituximab; the terminal complement activation inhibitor eculizumab; and belimumab, which targets B-cell activating factor. Increased understanding of molecular pathophysiology and accurate antibody subtyping in MG should lead to the use of new therapeutic agents and successful management of treatment-refractory patients. PMID:29403543

  15. Refractory organic substances in the environment

    National Research Council Canada - National Science Library

    Frimmel, F. H

    2002-01-01

    ... and its Quality Control in Fractions of Refractory Organic Substances and the Corresponding Original Water Samples 39 Introduction 39 Description of Analytical Methods 41 Sample Treatment 41 Fl...

  16. Evaluation of Alternative Refractory Materials for the Main Flame Deflectors at KSC Launch Complexes

    Science.gov (United States)

    Calle, Luz Marina; Trejo, David; Rutkowsky, Justin

    2006-01-01

    The deterioration of the refractory materials used to protect the KSC launch complex steel base structures from the high temperatures during launches results in frequent and costly repairs and safety hazards. KSC-SPEC-P-0012, Specification for Refractory Concrete, is ineffective in qualifying refractory materials. This study of the specification and of alternative refractory materials recommends a complete revision of the specification and further investigation of materials that were found to withstand the environment of the Solid Rocket Booster main flame deflector better than the refractory materials in current use in terms of compressive strength, tensile strength, modulus of rupture, shrinkage, and abrasion.

  17. 131I-MIBG followed by consolidation with busulfan, melphalan and autologous stem cell transplantation for refractory neuroblastoma.

    Science.gov (United States)

    French, Sarah; DuBois, Steven G; Horn, Biljana; Granger, Meaghan; Hawkins, Randall; Pass, Amy; Plummer, Ellen; Matthay, Katherine

    2013-05-01

    (131) I-metaiodobenzylguanidine (MIBG) produces a 37% response rate in relapsed/refractory neuroblastoma, and could be used to improve remission status prior to myeloablative chemotherapy with autologous stem cell transplant (ASCT). The purpose of our report was to evaluate safety and response with MIBG therapy followed by myeloablative busulfan and melphalan (BuMel) with ASCT in patients with refractory neuroblastoma. Retrospective chart review was done on patients treated with MIBG (18 mCi/kg) on Day 1 and ASCT on day 14. Six to eight weeks after MIBG, patients without progressive disease received IV busulfan on days -6 to -2 (target Css 700-900), melphalan (140 mg/m2 IV) on day -1, and ASCT on Day 0. Response and toxicity were evaluated after MIBG and again after myeloablative therapy. Eight patients completed MIBG/ASCT followed by BuMel/ASCT. MIBG was well tolerated, with grade 3 or 4 non-hematologic toxicity limited to one patient with sepsis. Grade 3 mucositis occurred in six patients after BuMel/ASCT. One patient developed sinusoidal obstructive syndrome (SOS) and died 50 days post-ASCT following myeloablative conditioning. All patients engrafted neutrophils (median 16.5 days) and platelets (median 32 days) after BuMel, excluding the patient with SOS. After all therapy, there were three complete, two partial, and one minor response in seven evaluable patients. MIBG at doses up to 18 mCi/kg can be safely administered 6 weeks prior to a BuMel consolidative regimen for refractory neuroblastoma. Preceding MIBG did not impair engraftment following BuMel. This regimen is being further evaluated in a Children's Oncology Group (COG) trial. Copyright © 2012 Wiley Periodicals, Inc.

  18. Amikacin-induced type 5 Bartter-like syndrome with severe hypocalcemia

    Directory of Open Access Journals (Sweden)

    Chrispal A

    2009-01-01

    Full Text Available Aminoglycoside-induced renal toxicity is well known and may manifest with nonoliguric renal failure or renal tubular dysfunction. Aminoglycoside-induced renal tubular dysfunction could result in diffuse damage or manifest as a Fanconi-like syndrome, Bartter-like syndrome, or distal renal tubular acidosis. We discuss a patient who developed severe renal tubular dysfunction secondary to short-term therapy with Amikacin, resulting in refractory hypokalemia, hypocalcemia, hypomagnesemia, metabolic alkalosis, and polyuria. This constellation of biochemical abnormalities mimic Type 5 Bartter′s syndrome, where there is activating mutation of the calcium sensing receptor in the thick ascending loop of Henle and the distal tubule. In this case this activation of the calcium sensing receptor was triggered by amikacin. This phenomenon has been described with gentamicin though never with amikacin. Recovery of the tubular dysfunction took 15 days following cessation of the offending drug, Amikacin.

  19. [Large vessel vasculitis with myelodysplastic syndrome: A rare association].

    Science.gov (United States)

    Galland, J; Kawski, H; Guichard, J-F; Maurier, F

    2017-07-01

    The vasculitis can be the consequence of malignancy: most often hematologic rather than solid tumors. The association between large vessels vasculitis and myelodysplastic syndrome is rare. A 55-year-old man experienced asthenia, fever, polyarthritis and inflammatory syndrome. Haematological investigations found a type 2 refractory anemia with excess blasts (RAEB-2) with discovery of severe anemia (Hb: 7,8g/dl) and thrombopenia (platelets: 40,000/mm 3 ). Radiological examinations found thoracic aortitis and carotid vasculitis. Treatment in the form of steroids and azacitidine was instituted. The lack of control of both RAEB-2 and vasculitis was responsible for the death of the patient. Myelodysplastic syndrome and large vessels vasculitis is a rare but serious association disease. The lack of efficiency of corticosteroids seems to be common. Prognosis depends on the haematological treatment effectiveness. Copyright © 2016 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier SAS. All rights reserved.

  20. Neuroleptic malignant syndrome and subsequent clozapine-withdrawal effects in a patient with refractory schizophrenia

    Directory of Open Access Journals (Sweden)

    Cheng MF

    2016-03-01

    Full Text Available Minfeng Cheng,* Huaying Gu,* Liangrong Zheng, Houliang Wang, Zhiyong Zhong, Shenglin Wen Department of Psychiatry, Third Affiliated Hospital of Sun Yat-Sen University, Guangzhou, People’s Republic of China *These authors contributed equally to this work Abstract: Here, we report a female patient developing neuroleptic malignant syndrome following the use of a combination of clozapine and haloperidol. Subsequently, the patient presented withdrawal effects after an abrupt discontinuation of clozapine. Psychiatrists not aware of possible clozapine-withdrawal effects may misdiagnose as a part of the primary mental illness or as the initial symptoms worsening, if unrecognized. Keywords: clozapine, neuroleptic malignant syndrome, withdrawal effect, schizophrenia

  1. Chronic recurrent Gorham-Stout syndrome with cutaneous involvement

    Directory of Open Access Journals (Sweden)

    Nahum Duker

    2010-09-01

    Full Text Available Type IV osteolysis or Gorham-Stout syndrome is a rare condition characterized by recurrent vascular tumors that disrupt normal anatomical architecture. Gorham-Stout syndrome is most commonly associated with the skeletal system with resulting replacement of bone with scar tissue following tumor regression. The loss of entire bones has given Gorham-Stout syndrome the moniker vanishing bone disease. Natural progression of Gorham-Stout syndrome is characterized by spontaneous disease resolution. However, rare variants of recurrent, progressive, and/or systemic disease have been reported. We present a patient with a history of recurrent Gorham-Stout disease refractory to all treatment options considered. In addition to skeletal disease, our patient had soft tissue and cutaneous involvement, thus reflecting the more aggressive disease variant. Previous surgical attempts to control disease had been ineffective and the patient was referred to us for radiation therapy. Treatment with external beam radiation therapy resulted in good local control and symptom palliation, but full disease resolution was never accomplished. In addition to presentation of this patient, a review of the literature on etiological hypotheses and past/future treatment options was conducted and is included.

  2. Therapeutic burst-suppression coma in pediatric febrile refractory status epilepticus.

    Science.gov (United States)

    Lin, Jainn-Jim; Chou, Cheng-Che; Lan, Shih-Yun; Hsiao, Hsiang-Ju; Wang, Yu; Chan, Oi-Wa; Hsia, Shao-Hsuan; Wang, Huei-Shyong; Lin, Kuang-Lin

    2017-09-01

    Evidence for the beneficial effect of therapeutic burst-suppression coma in pediatric patients with febrile refractory status epilepticus is limited, and the clinical outcomes of this treatment strategy are largely unknown. Therefore, the aim of this study was to explore the outcomes of therapeutic burst-suppression coma in a series of children with febrile refractory status epilepticus. We retrospectively reviewed consecutive pediatric patients with febrile refractory status epilepticus admitted to our pediatric intensive care unit between January 2000 and December 2013. The clinical characteristics were analyzed. Thirty-five patients (23 boys; age range: 1-18years) were enrolled, of whom 28 (80%) developed super-refractory status epilepticus. All of the patients received the continuous administration of intravenous antiepileptic drugs for febrile refractory status epilepticus, and 26 (74.3%) achieved therapeutic burst-suppression coma. All of the patients received mechanical ventilatory support, and 26 (74.3%) received inotropic agents. Eight (22.9%) patients died within 1month. The neurologically functional outcomes at 6months were good in six (27.3%) of the 22 survivors, of whom two returned to clinical baseline. The patients with therapeutic burst-suppression coma were significantly associated with hemodynamic support than the patients with electrographic seizures control (p=0.03), and had a trend of higher 1-month mortality rate, worse 6months outcomes, and a longer duration of hospitalization. Our results suggest that therapeutic burst-suppression coma to treat febrile refractory status epilepticus may lead to an increased risk of hemodynamic instability and a trend of worse outcomes. Copyright © 2017 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

  3. Zirconium determination in refractories (gravimetric method)

    International Nuclear Information System (INIS)

    Capiotto, N.; Narahashi, Y.; Perish, P.G.; Souza, J.R. de

    1991-01-01

    A gravimetric method for zirconium determination in refractories is described. X-ray fluorescence analysis is also employed in this experiment and considerations about interfering elements are presented. (M.V.M.)

  4. Association of a Novel Nonsense Mutation in KIAA1279 with Goldberg-Shprintzen Syndrome.

    Science.gov (United States)

    Salehpour, Shadab; Hashemi-Gorji, Feyzollah; Soltani, Ziba; Ghafouri-Fard, Soudeh; Miryounesi, Mohammad

    2017-01-01

    Goldberg-Shprintzen syndrome (OMIM 609460) (GOSHS) is an autosomal recessive multiple congenital anomaly syndrome distinguished by intellectual disability, microcephaly, and dysmorphic facial characteristics. Most affected individuals also have Hirschsprung disease and/or gyral abnormalities of the brain. This syndrome has been associated with KIAA1279 gene mutations at 10q22.1. Here we report a 16 yr old male patient referred to Center for Comprehensive Genetic Services, Tehran, Iran in 2015 with cardinal features of GOSHS in addition to refractory seizures. Whole exome sequencing in the patient revealed a novel nonsense (stop gain) homozygous mutation in KIAA1279 gene (KIAA1279: NM_015634:exon6:c.C976T:p.Q326X). Considering the wide range of phenotypic variations in GOSHS, relying on phenotypic characteristics for discrimination of GOSH from similar syndromes may lead to misdiagnosis. Consequently, molecular diagnostic tools would help in accurate diagnosis of such overlapping phenotypes.

  5. Development of various welding techniques for refractory and reactive metals and alloys

    International Nuclear Information System (INIS)

    Tonpe, Sunil; Saibaba, N.

    2016-01-01

    Nuclear Fuel Complex (NFC), Hyderabad, India with its excellent manufacturing facilities, produces nuclear fuel and structural components for nuclear reactors. NFC has taken up the challenging job of production of various critical components made out of refractory and reactive metals and alloys for nuclear and aerospace applications as an indigenization import substitute program. Refractory metals are prime candidates for many high temperature aerospace components because of refractory metal's high melting points and inherent creep resistance. The use of refractory metals is often limited because of their poor room temperature properties, inadequate oxidation resistance at elevated temperatures, difficulties associated with joining or welding etc. These advanced materials demand stringent requirement with respect to chemistry, dimensional tolerances, mechanical and metallurgical properties. This paper discusses in detail various welding techniques adopted in NFC for refractory and reactive metals and alloys such as Nb, Zr, Ti, Ta, Zircaloy, Titanium-half alloy etc. to manufacture various components and assemblies required for nuclear and aerospace applications

  6. Takayasu arteritis with middle aortic syndrome and mesenteric ischemia treated by aortic stenting

    Directory of Open Access Journals (Sweden)

    Sarah Morrissey, MD, MA, FRCSC

    2017-09-01

    Full Text Available A 48-year-old woman suffering from Takayasu arteritis presented with middle aortic syndrome consisting of abdominal pain, refractory hypertension with pulmonary edema and pleural effusions, and lower limb ischemia. She failed to improve with high-dose steroid therapy and underwent endovascular stenting of two severe stenoses in the supraceliac and infrarenal aorta. Her symptoms resolved and hypertension improved after the procedure.

  7. Applications of radioisotopes for studying refractory wear-out in Bhilai Steel Plant

    International Nuclear Information System (INIS)

    Dubey, R.S.; Bose, U.P.; Shipstone, A.J.

    1979-01-01

    In Bhilai Steel Plant, investigations were carried out to study the refractory wear-out of (i) hearth bottom of blast furnaces, (ii) roof of open hearth furnaces, and (iii) hot metal mixer lining, by using radioisotope tracer techniques with a view to evaluate the life of the refractory lining at various locations and to help in planning its timely hot and cold repairs. The life of the refractory lining has the effective bearing on the overall production and hence on the economy of the plant. The two radiometric methods employed for studying the erosion of the refractory lining, by using isotope inserted bricks at various positions without damaging the lining are (i) based on recording the penetration of gamma rays emitting from the radioactive isotopes inserted at definite points of the brick lining and, (ii) by detecting the radioactivity of the pig iron or steel arising due to washing away of the respective radioactive isotopes previously inserted in the lining. In hot mixers also radioisotope sources were placed in the critical location of refractory lining and the washing out of radioisotope due to refractory brick wear out was detected by radiogauging at site. It has been found that radiotracer technique with periodic radiogauging is very useful method for tracing the radioisotope source if more than one refractory brick with isotope is placed, as in the case of open hearth furnaces. The results of radioanalysis revealed that radioactivity coming alongwith hot metal steel has been far below the permissible limit of concentration i.e. 20 micro-curie per ton of metal. Further, during dismantling of the residual refractory lining of open hearth furnaces or hot metal mixers, bricks containing radioisotopes have been successfully retrieved for safe disposal. (auth.)

  8. Neonatal Marfan Syndrome: Report of a Case with an Inherited Splicing Mutation outside the Neonatal Domain.

    Science.gov (United States)

    Le Gloan, Laurianne; Hauet, Quentin; David, Albert; Hanna, Nadine; Arfeuille, Chloé; Arnaud, Pauline; Boileau, Catherine; Romefort, Bénédicte; Benbrik, Nadir; Gournay, Véronique; Joram, Nicolas; Baron, Olivier; Isidor, Bertrand

    2016-02-01

    We report a child and her mother affected by Marfan syndrome. The child presented with a phenotype of neonatal Marfan syndrome, revealed by acute and refractory heart failure, finally leading to death within the first 4 months of life. Her mother had a common clinical presentation. Genetic analysis revealed an inherited FBN1 mutation. This intronic mutation (c.6163+3_6163+6del), undescribed to date, leads to exon 49 skipping, corresponding to in-frame deletion of 42 amino acids (p.Ile2014_Asp2055del). FBN1 next-generation sequencing did not show any argument for mosaicism. Association in the same family of severe neonatal and classical Marfan syndrome illustrates the intrafamilial phenotype variability.

  9. Fragmentation process of vitrified ceramic waste (VCW) aiming its incorporation in silico-aluminous refractory concrete for production of refractory bricks

    International Nuclear Information System (INIS)

    Gomes, L.B.; Brandalise, R.N.; Santos, V. dos; Bergmann, C.P.

    2012-01-01

    Ceramic industry generates large amounts of waste, usually disposed in landfills. Reuse could minimize their generation and provides sustainable solutions. However, the energy cost of grinding these waste becomes a hindrance to their reuse. This work aims to obtain particle sizes of vitrified ceramic waste (VCW) using a fast, efficient and low cost fragmentation process as well as its use in refractory concrete. The results shows a wide range of particle size of VCW, which can be used as a promising source of raw material for production of refractory concrete. (author)

  10. Depression in patients with refractory temporal lobe epilepsy

    Directory of Open Access Journals (Sweden)

    Eleonora Borges Gonçalves

    2011-10-01

    Full Text Available OBJECTIVE: To evaluate the comorbidity of depressive disorders in patients with refractory temporal lobe epilepsy (TLE. METHOD: We evaluated 25 consecutive patients with refractory TLE (16 women and 9 men, using semi-structured psychiatric interviews, according to the International Classification of Diseases (ICD-10, and the Beck Depression Inventory. RESULTS: Seventeen of 25 patients (68% had depressive disorder: 6 with dysthymia, three with major depressive episodes and 8 with recurrent depressive disorders. Two (8% were diagnosed with mixed anxiety and depression. Only 5 of 17 patients (29.4% were previously diagnosed with depressive disorder and received prior antidepressant treatment. Duration of epilepsy was significantly higher in patients with depressive disorder (p=0.016, but there was no relationship between depression and seizure frequency. CONCLUSION: This study confirmed that depressive disorders are common and underdiagnosed in patients with TLE refractory to AEDs. Patients with longer duration of epilepsy are at higher risk of having depression.

  11. Alkali metal-refractory metal biphase electrode for AMTEC

    Science.gov (United States)

    Williams, Roger M. (Inventor); Bankston, Clyde P. (Inventor); Cole, Terry (Inventor); Khanna, Satish K. (Inventor); Jeffries-Nakamura, Barbara (Inventor); Wheeler, Bob L. (Inventor)

    1989-01-01

    An electrode having increased output with slower degradation is formed of a film applied to a beta-alumina solid electrolyte (BASE). The film comprises a refractory first metal M.sup.1 such as a platinum group metal, suitably platinum or rhodium, capable of forming a liquid or a strong surface adsorption phase with sodium at the operating temperature of an alkali metal thermoelectric converter (AMTEC) and a second refractory metal insoluble in sodium or the NaM.sup.1 liquid phase such as a Group IVB, VB or VIB metal, suitably tungsten, molybdenum, tantalum or niobium. The liquid phase or surface film provides fast transport through the electrode while the insoluble refractory metal provides a structural matrix for the electrode during operation. A trilayer structure that is stable and not subject to deadhesion comprises a first, thin layer of tungsten, an intermediate co-deposited layer of tungsten-platinum and a thin surface layer of platinum.

  12. Convergent validity of actigraphy with polysomnography and parent reports when measuring sleep in children with Down syndrome.

    Science.gov (United States)

    Esbensen, A J; Hoffman, E K; Stansberry, E; Shaffer, R

    2018-04-01

    There is a need for rigorous measures of sleep in children with Down syndrome as sleep is a substantial problem in this population and there are barriers to obtaining the gold standard polysomnography (PSG). PSG is cost-prohibitive when measuring treatment effects in some clinical trials, and children with Down syndrome may not cooperate with undergoing a PSG. Minimal information is available on the validity of alternative methods of assessing sleep in children with Down syndrome, such as actigraphy and parent ratings. Our study examined the concurrent and convergent validity of different measures of sleep, including PSG, actigraphy and parent reports of sleep among children with Down syndrome. A clinic (n = 27) and a community (n = 47) sample of children with Down syndrome were examined. In clinic, children with Down syndrome wore an actigraph watch during a routine PSG. In the community, children with Down syndrome wore an actigraph watch for a week at home at night as part of a larger study on sleep and behaviour. Their parent completed ratings of the child's sleep during that same week. Actigraph watches demonstrated convergent validity with PSG when measuring a child with Down syndrome's total amount of sleep time, total wake time after sleep onset and sleep period efficiency. In contrast, actigraph watches demonstrated poor correlations with parent reports of sleep, and with PSG when measuring the total time in bed and total wake episodes. Actigraphy, PSG and parent ratings of sleep demonstrated poor concurrent validity with clinical diagnosis of obstructive sleep apnoea. Our current data suggest that actigraph watches demonstrate convergent validity and are sensitive to measuring certain sleep constructs (duration, efficiency) in children with Down syndrome. However, parent reports, such as the Children's Sleep Habits Questionnaire, may be measuring other sleep constructs. These findings highlight the importance of selecting measures of sleep related to

  13. Physiological consequences of CPAP therapy withdrawal in patients with obstructive sleep apnoea-an opportunity for an efficient experimental model.

    Science.gov (United States)

    Schwarz, Esther I; Stradling, John R; Kohler, Malcolm

    2018-01-01

    Randomised controlled trials (RCTs) of continuous positive airway pressure (CPAP) in obstructive sleep apnoea (OSA) are time consuming, and their findings often inconclusive or limited due to suboptimal CPAP adherence in CPAP-naïve patients with OSA. Short-term CPAP withdrawal in patients with prior optimal CPAP adherence results in recurrence of OSA and its consequences. Thus, this experimental model serves as an efficient tool to investigate both the consequences of untreated OSA, and potential treatment alternatives to CPAP. The CPAP withdrawal protocol has been thoroughly validated, and applied in several RCTs focusing on cardiovascular and metabolic consequences of untreated OSA, as well as the assessment of treatment alternatives to CPAP.

  14. Plasma deposition of refractories

    International Nuclear Information System (INIS)

    Kudinov, V.V.; Ivanov, V.M.

    1981-01-01

    The problems of deposition, testing and application of plasma coating of refractory metals and oxides are considered. The process fundamentals, various manufacturing procedures and equipment for their realization are described in detail. Coating materials are given (Al, Mg, Al 2 O 3 , ZrO 2 , MgAlO 4 ) which are used in reactor engineering and their designated purposes are shown [ru

  15. Biological Therapy in Treating Patients With Advanced Myelodysplastic Syndrome, Acute or Chronic Myeloid Leukemia, or Acute Lymphoblastic Leukemia Who Are Undergoing Stem Cell Transplantation

    Science.gov (United States)

    2017-03-27

    Adult Acute Myeloid Leukemia With 11q23 (MLL) Abnormalities; Adult Acute Myeloid Leukemia With Inv(16)(p13;q22); Adult Acute Myeloid Leukemia With t(15;17)(q22;q12); Adult Acute Myeloid Leukemia With t(16;16)(p13;q22); Adult Acute Myeloid Leukemia With t(8;21)(q22;q22); B-cell Adult Acute Lymphoblastic Leukemia; B-cell Childhood Acute Lymphoblastic Leukemia; Childhood Chronic Myelogenous Leukemia; Childhood Myelodysplastic Syndromes; Chronic Myelomonocytic Leukemia; Essential Thrombocythemia; Polycythemia Vera; Previously Treated Myelodysplastic Syndromes; Recurrent Adult Acute Lymphoblastic Leukemia; Recurrent Adult Acute Myeloid Leukemia; Recurrent Childhood Acute Lymphoblastic Leukemia; Recurrent Childhood Acute Myeloid Leukemia; Refractory Anemia With Excess Blasts; Refractory Anemia With Excess Blasts in Transformation; Relapsing Chronic Myelogenous Leukemia; Secondary Acute Myeloid Leukemia; T-cell Adult Acute Lymphoblastic Leukemia; T-cell Childhood Acute Lymphoblastic Leukemia

  16. Barrier Coatings for Refractory Metals and Superalloys

    Energy Technology Data Exchange (ETDEWEB)

    SM Sabol; BT Randall; JD Edington; CJ Larkin; BJ Close

    2006-02-23

    In the closed working fluid loop of the proposed Prometheus space nuclear power plant (SNPP), there is the potential for reaction of core and plant structural materials with gas phase impurities and gas phase transport of interstitial elements between superalloy and refractory metal alloy components during service. Primary concerns are surface oxidation, interstitial embrittlement of refractory metals and decarburization of superalloys. In parallel with kinetic investigations, this letter evaluates the ability of potential coatings to prevent or impede communication between reactor and plant components. Key coating requirements are identified and current technology coating materials are reviewed relative to these requirements. Candidate coatings are identified for future evaluation based on current knowledge of design parameters and anticipated environment. Coatings were identified for superalloys and refractory metals to provide diffusion barriers to interstitial transport and act as reactive barriers to potential oxidation. Due to their high stability at low oxygen potential, alumina formers are most promising for oxidation protection given the anticipated coolant gas chemistry. A sublayer of iridium is recommended to provide inherent diffusion resistance to interstitials. Based on specific base metal selection, a thin film substrate--coating interdiffusion barrier layer may be necessary to meet mission life.

  17. Barrier Coatings for Refractory Metals and Superalloys

    International Nuclear Information System (INIS)

    SM Sabol; BT Randall; JD Edington; CJ Larkin; BJ Close

    2006-01-01

    In the closed working fluid loop of the proposed Prometheus space nuclear power plant (SNPP), there is the potential for reaction of core and plant structural materials with gas phase impurities and gas phase transport of interstitial elements between superalloy and refractory metal alloy components during service. Primary concerns are surface oxidation, interstitial embrittlement of refractory metals and decarburization of superalloys. In parallel with kinetic investigations, this letter evaluates the ability of potential coatings to prevent or impede communication between reactor and plant components. Key coating requirements are identified and current technology coating materials are reviewed relative to these requirements. Candidate coatings are identified for future evaluation based on current knowledge of design parameters and anticipated environment. Coatings were identified for superalloys and refractory metals to provide diffusion barriers to interstitial transport and act as reactive barriers to potential oxidation. Due to their high stability at low oxygen potential, alumina formers are most promising for oxidation protection given the anticipated coolant gas chemistry. A sublayer of iridium is recommended to provide inherent diffusion resistance to interstitials. Based on specific base metal selection, a thin film substrate--coating interdiffusion barrier layer may be necessary to meet mission life

  18. Prognostic Factors for Refractory Status Epilepticus

    Directory of Open Access Journals (Sweden)

    J. Gordon Millichap

    2013-03-01

    Full Text Available Researchers at the Mayo Clinic, Rochester, MN studied the outcome and identified prognostic factors for refractory status epilepticus (RSE in 54 adult patients, median age 52 years [range 18-93].

  19. Renal hemangiopericytoma secondary to refractory hypertension in a child: A case report.

    Science.gov (United States)

    Hu, Qingfeng; Fang, Zujun; Zhou, Zhongwen; Zheng, Jie

    2014-12-01

    Hemangiopericytoma is a rare perivascular tumor that often involves the extremities, pelvis, head and neck, and meninges, but rarely occurs in the kidney. The differentiation from renal cancer prior to surgery is extremely challenging; therefore, almost all cases of renal hemangiopericytoma are diagnosed by pathological examination. The majority of cases are identified in patients between the ages of 20 and 50 years of age, and a considerable proportion of patients exhibit hypertension, hypoglycaemia or additional paraneoplastic syndromes. The current study reports a rare case of renal hemangiopericytoma with drug refractory hypertension in a 14-year-old female. Following the complete resection of the tumor, the patient's blood pressure returned to normal. No evidence of recurrence or metastasis was observed during a follow-up of 12 months following surgery. The present case indicated that surgery provides satisfactory outcomes and appears to be the most effective modality of treatment for renal hemangiopericytoma. Furthermore, this case also demonstrated that secondary hypertension may also recover following tumor excision.

  20. Refractory disease in autoimmune diseases

    NARCIS (Netherlands)

    Vasconcelos, Carlos; Kallenberg, Cees; Shoenfeld, Yehuda

    Refractory disease (RD) definition has different meanings but it is dynamic, according to knowledge and the availability of new drugs. It should be differentiated from severe disease and damage definitions and it must take into account duration of adequate therapy and compliance of the patient. It

  1. Chemical analysis of refractories by plasma spectrometry

    International Nuclear Information System (INIS)

    Coutinho, C.A.

    1990-01-01

    X-ray spectrometry has been, since the last two or three decades, the traditional procedure for the chemical analysis of refractories, due to its high degree of accuracy and speed to produce analytical results. An interesting alternative to X-ray fluorescence is provided by the Inductively Coupled Plasma Spectrometry technique, for those laboratories where wet chemistry facilities are already available or process control is not required at high speed, or investiment costs have to be low. This paper presents results obtained by plasma spectroscopy for the analysis of silico - aluminous refractories, showing calibration curves, precion and detection limits. Considerations and comparisons with X-ray fluorescence are also made. (author) [pt

  2. Improvements in or relating to refractory materials

    International Nuclear Information System (INIS)

    Peckett, J.W.A.

    1980-01-01

    A process is described for the production of a refractory material which includes heating an intermediate material containing carbon to cause a thermally induced reaction involving carbon in the intermediate material, wherein the intermediate material has been produced by heating a shaped gel precipitated gel, and the carbon in the intermediate material for participating in the thermally induced reaction has been produced from a gelling agent, or a derivative thereof, incorporated in the gel during gel precipitation. As examples, the refractory material may comprise uranium/plutonium oxide, or uranium/plutonium carbide, or thorium/uranium carbide, or tungsten carbide, or tungsten carbide/cobalt metal. (author)

  3. Development of electro fused aggregates for use in refractories for the burning zone of cement kilns

    International Nuclear Information System (INIS)

    Ferreira, Luis Leonardo Horne Curimbaba

    2006-01-01

    Electro fused aggregates are largely used in refractory production due to the better performance reached when they are employed. In this work electro fused aggregates were designed for application in refractories for the burning zone of cement kilns. Initially reaction evaluation was conducted aiming the identification of the most prone refractory systems when single refractory phases react with Portland cement phases at high temperatures. In the next step, raw materials of the best refractory systems were electro fused to generate different aggregate compositions. The electro fused aggregates properties were evaluated and the classified ones were used to produce refractory bricks for the burning zone of cement kilns. General characteristics of these bricks were measured and compared with a standard magnesia-spinel refractory. Aggregates of the system Mg O - TiO 2 - Ca O, more specifically aggregates belonged to the compatibility triangle Mg O - Mg 2 TiO 4 - CaTiO 3 , showed suitable characteristics for development of refractories for the burning zone cement kilns. (author)

  4. Refractory Seizures in Tramadol Poisoning: A Case Report

    Directory of Open Access Journals (Sweden)

    Mohammad Majidi

    2014-09-01

    Full Text Available Background: Tramadol, an analgesic drug abused by opioid addicts, is also abused accidentally or for suicidal purposes. Tramadol poisoning can induce CNS depression, seizures, coma, and ultimately death. Case: In this report, a 30-year-old male was admitted to the emergency department due to suicidal attempt with ingestion of 14000 mg (140 tablet 100 mg of tramadol. He had history of suicidal attempts in past years as well as depression in his past medical history, but he had not abused tramadol and other drugs in his history. There was no history of epilepsy or head trauma in. He presented with generalized seizures two hours post ingestion, and, then, he was referred to hospital four hours later. Generalized seizures were poorly controlled by multiple medications. Due to respiratory arrest, endotracheal tube was inserted and he was admitted to the ICU immediately. At admission, he experienced hypovolemic shock, hypoglycemia, coma, apnea, refractory seizures, muscle spasms, acute respiratory distress syndrome, coagolative disorder, rhabdomyolysis, and acute renal failure. Despite medical managements, he died 38 days after ingestion. Conclusion: In this report, despite using inhalational anesthetic drugs, seizures continued and were very poorly controlled. Cause of death in this patient can be seen as the side effects of tramadol poisoning.

  5. Acute coronary syndrome caused by coronary vasospasms associated with Churg-Strauss syndrome: effects of betamethasone therapy.

    Science.gov (United States)

    Suzuki, Yuji; Nishiyama, Osamu; Sakai, Toshiaki; Niiyama, Masanobu; Itoh, Tomonori; Nakamura, Motoyuki

    2014-01-01

    A 42-year-old woman with a history of aspirin-induced asthma was admitted with severe chest pain. Emergency coronary angiography revealed coronary artery spasms. The administration of vasodilators did not suppress the anginal symptoms, and the differential white blood cell count continued to show eosinophilia. The patient's symptoms of aspirin-induced asthma, eosinophilia and other allergic states led to the diagnosis of Churg-Strauss syndrome (CSS). After starting betamethasone therapy, the eosinophilia and cardiac symptoms rapidly disappeared. Although coronary vasospasms related to CSS are rare, the present case suggests that a differential white blood cell count should be obtained in patients with refractory coronary vasospasms.

  6. Zirconium determination in refractories (gravimetric method)

    International Nuclear Information System (INIS)

    Capiotto, N.; Narahashi, Y.; Perish, C.G.; Souza, J.R.

    1991-01-01

    The zirconium determination in refractories is described, consisting in two separation methods for eliminating the interferences. The formatted product is calcined at 1100 0 C and determined gravimetrically as Zr P z 07. (author)

  7. Genetics of refractoriness to Plasmodium falciparum in the mosquito Anopheles stephensi

    NARCIS (Netherlands)

    Feldmann, A.M.; Gemert, Geert-Jan van; Vegte-Bolmer, Marga G. van de; Jansen, Ritsert C.

    1998-01-01

    We previously selected a line of the malaria vector mosquito Anopheles stephensi refractory (resistant) to the human malaria parasite Plasmodium falciparum, using in vitro infections with P. falciparum gametocytes. This report presents data on the genetic background of refractoriness. The results of

  8. Productivity Costs in Patients with Refractory Chronic Rhinosinusitis

    Science.gov (United States)

    Rudmik, Luke; Smith, Timothy L.; Schlosser, Rodney J.; Hwang, Peter H.; Mace, Jess C.; Soler, Zachary M.

    2014-01-01

    Objective Disease-specific reductions in patient productivity can lead to substantial economic losses to society. The purpose of this study was to: 1) define the annual productivity cost for a patient with refractory chronic rhinosinusitis (CRS) and 2) evaluate the relationship between degree of productivity cost and CRS-specific characteristics. Study Design Prospective, multi-institutional, observational cohort study. Methods The human capital approach was used to define productivity costs. Annual absenteeism, presenteeism, and lost leisure time was quantified to define annual lost productive time (LPT). LPT was monetized using the annual daily wage rates obtained from the 2012 US National Census and the 2013 US Department of Labor statistics. Results A total of 55 patients with refractory CRS were enrolled. The mean work days lost related to absenteeism and presenteeism was 24.6 and 38.8 days per year, respectively. A total of 21.2 household days were lost per year related to daily sinus care requirements. The overall annual productivity cost was $10,077.07 per patient with refractory CRS. Productivity costs increased with worsening disease-specific QoL (r=0.440; p=0.001). Conclusion Results from this study have demonstrated that the annual productivity cost associated with refractory CRS is $10,077.07 per patient. This substantial cost to society provides a strong incentive to optimize current treatment protocols and continue evaluating novel clinical interventions to reduce this cost. PMID:24619604

  9. Productivity costs in patients with refractory chronic rhinosinusitis.

    Science.gov (United States)

    Rudmik, Luke; Smith, Timothy L; Schlosser, Rodney J; Hwang, Peter H; Mace, Jess C; Soler, Zachary M

    2014-09-01

    Disease-specific reductions in patient productivity can lead to substantial economic losses to society. The purpose of this study was to: 1) define the annual productivity cost for a patient with refractory chronic rhinosinusitis (CRS) and 2) evaluate the relationship between degree of productivity cost and CRS-specific characteristics. Prospective, multi-institutional, observational cohort study. The human capital approach was used to define productivity costs. Annual absenteeism, presenteeism, and lost leisure time was quantified to define annual lost productive time (LPT). LPT was monetized using the annual daily wage rates obtained from the 2012 U.S. National Census and the 2013 U.S. Department of Labor statistics. A total of 55 patients with refractory CRS were enrolled. The mean work days lost related to absenteeism and presenteeism were 24.6 and 38.8 days per year, respectively. A total of 21.2 household days were lost per year related to daily sinus care requirements. The overall annual productivity cost was $10,077.07 per patient with refractory CRS. Productivity costs increased with worsening disease-specific QoL (r = 0.440; p = 0.001). Results from this study have demonstrated that the annual productivity cost associated with refractory CRS is $10,077.07 per patient. This substantial cost to society provides a strong incentive to optimize current treatment protocols and continue evaluating novel clinical interventions to reduce this cost. © 2014 The American Laryngological, Rhinological and Otological Society, Inc.

  10. Refractory metal based superalloys

    International Nuclear Information System (INIS)

    Alonso, Paula R.; Vicente, Eduardo E.; Rubiolo, Gerardo H.

    1999-01-01

    Refractory metals are looked as promising materials for primary circuits in fission reactors and even as fusion reactor components. Indeed, superalloys could be developed which take advantage of their high temperature properties together with the benefits of a two- phase (intermetallic compound-refractory metal matrix) coherent structure. In 1993, researchers of the Office National d'Etudes et de Recherches Aerospatiales of France reported the observation of such a coherent structure in the Ta-Ti-Zr-Al-Nb-Mo system although the exact composition is not reported. The intermetallic compound would be Ti 2 AlMo based. However, the formation of this compound and its possible coexistence with a disordered bcc phase in the ternary system Ti-Al-Mo is a controversial subject in the related literature. In this work we develop a technique to obtain homogeneous alloys samples with 50 Ti-25 Al-25 Mo composition. The resulting specimens were characterized by optical and electronic metallography (SEM), microprobe composition measurements (EPMA) and X-ray diffraction (XRD) analyses. The results show the evidence for a bcc (A2→B2) ordering reaction in the Ti-Al-Mo system in the 50 Ti-25 Al-25 Mo composition. (author)

  11. Tonsillectomy or adenotonsillectomy versus non-surgical management for obstructive sleep-disordered breathing in children

    NARCIS (Netherlands)

    Venekamp, Roderick P; Hearne, Benjamin J; Chandrasekharan, Deepak; Blackshaw, Helen; Lim, Jerome; Schilder, Anne G M

    2015-01-01

    BACKGROUND: Obstructive sleep-disordered breathing (oSDB) is a condition that encompasses breathing problems when asleep, due to an obstruction of the upper airways, ranging in severity from simple snoring to obstructive sleep apnoea syndrome (OSAS). It affects both children and adults. In children,

  12. Felbamate in an adult population with severe refractory epilepsy.

    LENUS (Irish Health Repository)

    Kearney, H

    2011-04-05

    Felbamate (FBM) is efficacious in treating patients with refractory epilepsy but was withdrawn due to cases of aplastic anaemia, hepatic failure and five reported deaths. FBM is currently used in specialist centres and is only being used in one Irish centre to date. This papers aim is to review the efficacy and safety experience of FBM in Irish adult patients with refractory epilepsy. A retrospective chart review was done on patients\\' medical records. Patients were subdivided into responders and non responders based on change in seizure frequency and side effects were recorded for all. Of the 13 patients on FBM nine patients responded to FBM, four patients did not. FBM is a safe and efficacious alternative in an Irish adult population with refractory epilepsy. However close monitoring is still required given the potential fatal side effects that are possible with this anticonvulsant.

  13. MICROWAVE MEASUREMENT OF REFRACTORY MATERIALS AT HIGH-TEMPERATURE

    International Nuclear Information System (INIS)

    Kharkovsky, S.; Zoughi, R.; Smith, J.; Davis, B.; Limmer, R.

    2009-01-01

    Knowledge of the electrical behavior of refractory materials may enable the development and optimization of microwave nondestructive techniques to detect and evaluate changes in their physical properties while the materials are in service. This paper presents the results of a limited and preliminary investigation in which two refractory materials (dense chrome and dense zircon) were subjected to increasing temperature in a furnace and in which a frequency-modulated continuous-wave radar operating in the frequency range of 8-18 GHz radar was used to evaluate their attenuation properties.

  14. Microwave Measurement of Refractory Materials at High-Temperature

    Science.gov (United States)

    Kharkovsky, S.; Zoughi, R.; Smith, J.; Davis, B.; Limmer, R.

    2009-03-01

    Knowledge of the electrical behavior of refractory materials may enable the development and optimization of microwave nondestructive techniques to detect and evaluate changes in their physical properties while the materials are in service. This paper presents the results of a limited and preliminary investigation in which two refractory materials (dense chrome and dense zircon) were subjected to increasing temperature in a furnace and in which a frequency-modulated continuous-wave radar operating in the frequency range of 8-18 GHz radar was used to evaluate their attenuation properties.

  15. Case Report: Sjogren-Larsson Syndrome: Two Cases from One Family

    Directory of Open Access Journals (Sweden)

    Parvaneh Karim-Zadeh

    2006-04-01

    Full Text Available Sjogren–Larsson Syndrome (SLS is an autosomal recessive disorder characterized by generalized Ichthyosis, mental retardation, spastic diplegia or tetraplegia and epilepsy. This is a rare syndrome that caused by mutation in the ALDH3A2 gene, on chromosome 17p11.2. That encodes fatty aldehyde dehydrogenase (FAIDH, an enzyme that catalyzes the oxidation of medium – long chain aldehydes derived from lipid metabolism. Neuroimaging (MRI shows retardation of myelination and a mild myelin deficit. Proton Magnetic Resonance Spectroscopy (MRS shows the peak of lipids that accumulate because of fatty alchohols. We report two cases that they are siblings from relative parents. The Brother is 4 years old and his sister is 3 years old. , The clinical findings are developmental delay, mental retardation, spastic Tetraplegia and refractory seizure. The most important finding in these two siblings was generalized Icthyosis. MRI showed hyper signality in white matter and MRS showed the peak of accumulated lipids that confirmed the diagnosis of "Sjogren-Larsson Syndrome".

  16. CT-guided periradicular therapy in cervical and lumbar radicular syndromes

    International Nuclear Information System (INIS)

    Nian Dingfang; Zhou Jun; Li Wenhua; Cao Qingxuan; Xia Baosu; Apitzsch, D.E.

    2007-01-01

    Objective: To evaluate the effectiveness of CT-guided periradicular therapy (PRT) in cervical and lumber radicular syndromes. Methods: Seventy two cases of cervical and lumbar radicular syndromes were treated by CT-guided PRT. During PRT, 10-20 mg of Triamhexal and 3-4 ml of Carbostesin 0.25%(Bupivacainhydrochlorid) were injected into the space around the corresponding spinal root, once a week, all together six times as a course. Results: Four hundreds and forty six PRT procedures were carried out in 72 patients having a successful puncture and a satisfying spread of Triamhexal and Carbostesin around the spinal nerve root space for every time. After 6 to 24 months follow up, 62 patients showed a significant pain reduction or complete disappearance. Moderate improvement was not noted in 6 and no improvement in 4. The effective rate was 94.4% without any correlative complication. Conclusions: CT-guided PRT is an effective, easy and safe procedure to treat patients with refractory cervical or lumbar radicular pain syndromes. (authors)

  17. Ten patients with refractory status epilepticus in an intensive care department

    NARCIS (Netherlands)

    ter Maaten, JC; van Schijndel, RJM; Heimans, JJ; Schreuder, WO

    1998-01-01

    Status epilepticus (SE) is a serious disease, associated with a high morbidity and mortality, particularly if refractory to initial therapy. We describe the clinical manifestations and outcome in ten cases with refractory SE admitted to our medical intensive care unit. Three of these selected group

  18. Treatment of Refractory Idiopathic Supraorbital Neuralgia Using Percutaneous Pulsed Radiofrequency.

    Science.gov (United States)

    Luo, Fang; Lu, Jingjing; Ji, Nan

    2018-02-26

    No ideal therapeutic method currently exists for refractory idiopathic supraorbital neuralgia patients who do not respond to conservative therapy, including medications and nerve blocks. Pulsed radiofrequency is a neuromodulation technique that does not produce sequelae of nerve damage after treatment. However, the efficacy of percutaneous pulsed radiofrequency for the treatment of refractory idiopathic supraorbital neuralgia is still not clear. The purpose of our study was to evaluate the efficacy and safety of pulsed radiofrequency treatment of the supraorbital nerve for refractory supraorbital neuralgia patients. We prospectively investigated the long-term effects of ultrasound-guided percutaneous pulsed radiofrequency in the treatment of 22 refractory idiopathic supraorbital neuralgia patients. A reduction in the verbal pain numeric rating scale score of more than 50% was used as the standard of effectiveness. The effectiveness rates at different time points within 2 years were calculated. After a single pulsed radiofrequency treatment, the effectiveness rate at 1 and 3 months was 77%, and the rates at 6 months, 1 year, and 2 years were 73%, 64%, and 50%, respectively. Except for a small portion of patients (23%) who experienced mild upper eyelid ecchymosis that gradually disappeared after approximately 2 weeks, no obvious complications were observed. In conclusion, the results of our study demonstrate that for patients with refractory idiopathic supraorbital neuralgia, percutaneous pulsed radiofrequency may be an effective and safe treatment choice. © 2018 World Institute of Pain.

  19. Surgical disconnection of patent paraumbilical vein in refractory hepatic encephalopathy.

    Science.gov (United States)

    Ishikawa, Yoshinori; Yoshida, Hiroshi; Mamada, Yasuhiro; Taniai, Nobuhiko; Bando, Koichi; Mizuguchi, Yoshiaki; Kakinuma, Daisuke; Kanda, Tomohiro; Akimaru, Koho; Tajiri, Takashi

    2008-06-01

    Refractory hepatic encephalopathy (HE) frequently develops in patients with cirrhosis and portal-systemic shunt. Recently, patients with refractory HE associated with portal-systemic shunt have been treated with interventional radiology. We describe a promising new treatment for portal-systemic shunt, ligation of the patent paraumbilical vein (PUV) after partial splenic embolization, in patients with refractory HE. Four patients with cirrhosis (3 women and 1 man; mean age, 56 years) and refractory HE due to a patent PUV were studied. Patency of the PUV had recurred in 1 patient after primary occlusion by interventional radiological procedures. The Child-Pugh class was B in 2 patients and C in 2. Before the present treatment, all patients had been hospitalized at least 3 times because of recurrent HE. Partial splenic embolization was performed in all patients to decrease portal venous pressure before surgery. Surgical ligation of the patent PUV was performed under epidural anesthesia. The patent PUV was carefully skeletonized and doubly ligated. Esophageal varices were evaluated with upper gastrointestinal endoscopy before and after surgery. The mean follow-up duration was 15.8 months. After ligation, there were no clinically significant complications. Esophageal varices were unchanged. The serum ammonia level was higher before surgery (162.3 +/- 56.4 mug/dL, mean +/- SD) than after surgery (41.8 +/- 20.2 mug/dL; p=0.0299). No patient had symptoms of HE. Ligation of the patent PUV is an effective treatment for patients with refractory HE.

  20. Bulk Vitrification Performance Enhancement: Refractory Lining Protection Against Molten Salt Penetration

    Energy Technology Data Exchange (ETDEWEB)

    Hrma, Pavel R.; Bagaasen, Larry M.; Schweiger, Michael J.; Evans, Michael B.; Smith, Benjamin T.; Arrigoni, Benjamin M.; Kim, Dong-Sang; Rodriguez, Carmen P.; Yokuda, Satoru T.; Matyas, Josef; Buchmiller, William C.; Gallegos, Autumn B.; Fluegel, Alexander

    2007-08-06

    Bulk vitrification (BV) is a process that heats a feed material that consists of glass-forming solids and dried low-activity waste (LAW) in a disposable refractory-lined metal box using electrical power supplied through carbon electrodes. The feed is heated to the point that the LAW decomposes and combines with the solids to generate a vitreous waste form. This study supports the BV design and operations by exploring various methods aimed at reducing the quantities of soluble Tc in the castable refractory block portion of the refractory lining, which limits the effectiveness of the final waste form.

  1. Comparison of Refractory Performance in Black Liquor Gasifiers and a Smelt Test System

    International Nuclear Information System (INIS)

    Peascoe, RA

    2001-01-01

    Prior laboratory corrosion studies along with experience at the black liquor gasifier in New Bern, North Carolina, clearly demonstrate that serious material problems exist with the gasifier's refractory lining. Mullite-based and alumina-based refractories used at the New Bern facility suffered significant degradation even though they reportedly performed adequately in smaller scale systems. Oak Ridge National Laboratory's involvement in the failure analysis, and the initial exploration of suitable replacement materials, led to the realization that a simple and reliable, complementary method for refractory screening was needed. The development of a laboratory test system and its suitability for simulating the environment of black liquor gasifiers was undertaken. Identification and characterization of corrosion products were used to evaluate the test system as a rapid screening tool for refractory performance and as a predictor of refractory lifetime. Results from the test systems and pl ants were qualitatively similar

  2. Targeting renin-angiotensin system in malignant hypertension in atypical hemolytic uremic syndrome

    Directory of Open Access Journals (Sweden)

    V Raghunathan

    2017-01-01

    Full Text Available Hypertension is common in hemolytic uremic syndrome (HUS and often difficult to control. Local renin-angiotensin activation is believed to be an important part of thrombotic microangiopathy, leading to a vicious cycle of progressive renal injury and intractable hypertension. This has been demonstrated in vitro via enhanced tissue factor expression on glomerular endothelial cells which is enhanced by angiotensin II. We report two pediatric cases of atypical HUS with severe refractory malignant hypertension, in which we targeted the renin-angiotensin system by using intravenous (IV enalaprilat, oral aliskiren, and oral enalapril with quick and dramatic response of blood pressure. Both drugs, aliskiren and IV enalaprilat, were effective in controlling hypertension refractory to multiple antihypertensive medications. These appear to be promising alternatives in the treatment of severe atypical HUS-induced hypertension and hypertensive emergency.

  3. A near catastrophe from trigeminocardiac reflex

    Directory of Open Access Journals (Sweden)

    Parmod K Bithal

    2017-01-01

    Full Text Available Trigeminocardiac reflex is a brainstem reflex that results from stimulation of any branch of the trigeminal nerve along its course. It produces a constellation of signs and symptoms decrease in blood pressure (BP and heart rate, dysrhythmias, apnoea and increased gastric motility. We present a case of 80-year-old female patient who developed alarming hypotension and bradycardia during craniotomy for meningioma excision resulting from this reflex. In the face of refractory hypotension despite administering ephedrine and phenylephrine, we had to resort to adrenaline to restore her normal BP.

  4. Computed tomographic cephalometric analysis of chinese patients with obstructive sleep apnoea

    International Nuclear Information System (INIS)

    Peh, W.C.G.; Chu, F.S.K.; Ip, M.S.M.; Chung, K.-F.

    2000-01-01

    Variations of craniofacial and upper airway structures are recognized to contribute to the phenomenon of obstructive sleep apnoea (OSA). Most previous cephalometric studies were performed using erect lateral radiographs in Caucasian patients. The present project aims to determine cephalometric measurements, utilizing CT, in normal Chinese subjects and in Chinese patients with OSA. Computed tomography of 25 patients with OSA (proven using overnight polysomnography), and of 25 age-, sex-, height-, bodyweight- and body mass index (BMI)-matched control subjects were prospectively performed. Thirteen standard bony and soft-tissue measurements were obtained from the CT lateral scout view of the head and neck, taken with each subject in the neutral supine position. The cross-sectional area was calculated at two axial levels (velopharynx and hypopharynx). The measurements in the two groups, OSA and control subjects, were compared. The measurements for hyoid position (P=0.00), nasal cavity length (P=0.01), mandibular prognathism (P = 0.05), tongue size (P = 0.02), oropharyngeal airway space (P = 0.02), posterior tongue airway space (P = 0.04) and cross-sectional areas at the level of the velopharynx (P = 0.00) and hypopharynx (P = 0.01) differed significantly between the two groups. In conclusion, CT cephalometric measurements show that certain anatomical variations in the head and neck are likely to contribute to the pathogenesis of OSA in Chinese patients. Copyright (1999) Blackwell Science Pty Ltd

  5. Impact of Facial Conformation on Canine Health: Brachycephalic Obstructive Airway Syndrome

    Science.gov (United States)

    Packer, Rowena M. A.; Hendricks, Anke; Tivers, Michael S.; Burn, Charlotte C.

    2015-01-01

    The domestic dog may be the most morphologically diverse terrestrial mammalian species known to man; pedigree dogs are artificially selected for extreme aesthetics dictated by formal Breed Standards, and breed-related disorders linked to conformation are ubiquitous and diverse. Brachycephaly–foreshortening of the facial skeleton–is a discrete mutation that has been selected for in many popular dog breeds e.g. the Bulldog, Pug, and French Bulldog. A chronic, debilitating respiratory syndrome, whereby soft tissue blocks the airways, predominantly affects dogs with this conformation, and thus is labelled Brachycephalic Obstructive Airway Syndrome (BOAS). Despite the name of the syndrome, scientific evidence quantitatively linking brachycephaly with BOAS is lacking, but it could aid efforts to select for healthier conformations. Here we show, in (1) an exploratory study of 700 dogs of diverse breeds and conformations, and (2) a confirmatory study of 154 brachycephalic dogs, that BOAS risk increases sharply in a non-linear manner as relative muzzle length shortens. BOAS only occurred in dogs whose muzzles comprised less than half their cranial lengths. Thicker neck girths also increased BOAS risk in both populations: a risk factor for human sleep apnoea and not previously realised in dogs; and obesity was found to further increase BOAS risk. This study provides evidence that breeding for brachycephaly leads to an increased risk of BOAS in dogs, with risk increasing as the morphology becomes more exaggerated. As such, dog breeders and buyers should be aware of this risk when selecting dogs, and breeding organisations should actively discourage exaggeration of this high-risk conformation in breed standards and the show ring. PMID:26509577

  6. Impact of Facial Conformation on Canine Health: Brachycephalic Obstructive Airway Syndrome.

    Science.gov (United States)

    Packer, Rowena M A; Hendricks, Anke; Tivers, Michael S; Burn, Charlotte C

    2015-01-01

    The domestic dog may be the most morphologically diverse terrestrial mammalian species known to man; pedigree dogs are artificially selected for extreme aesthetics dictated by formal Breed Standards, and breed-related disorders linked to conformation are ubiquitous and diverse. Brachycephaly--foreshortening of the facial skeleton--is a discrete mutation that has been selected for in many popular dog breeds e.g. the Bulldog, Pug, and French Bulldog. A chronic, debilitating respiratory syndrome, whereby soft tissue blocks the airways, predominantly affects dogs with this conformation, and thus is labelled Brachycephalic Obstructive Airway Syndrome (BOAS). Despite the name of the syndrome, scientific evidence quantitatively linking brachycephaly with BOAS is lacking, but it could aid efforts to select for healthier conformations. Here we show, in (1) an exploratory study of 700 dogs of diverse breeds and conformations, and (2) a confirmatory study of 154 brachycephalic dogs, that BOAS risk increases sharply in a non-linear manner as relative muzzle length shortens. BOAS only occurred in dogs whose muzzles comprised less than half their cranial lengths. Thicker neck girths also increased BOAS risk in both populations: a risk factor for human sleep apnoea and not previously realised in dogs; and obesity was found to further increase BOAS risk. This study provides evidence that breeding for brachycephaly leads to an increased risk of BOAS in dogs, with risk increasing as the morphology becomes more exaggerated. As such, dog breeders and buyers should be aware of this risk when selecting dogs, and breeding organisations should actively discourage exaggeration of this high-risk conformation in breed standards and the show ring.

  7. Impact of Facial Conformation on Canine Health: Brachycephalic Obstructive Airway Syndrome.

    Directory of Open Access Journals (Sweden)

    Rowena M A Packer

    Full Text Available The domestic dog may be the most morphologically diverse terrestrial mammalian species known to man; pedigree dogs are artificially selected for extreme aesthetics dictated by formal Breed Standards, and breed-related disorders linked to conformation are ubiquitous and diverse. Brachycephaly--foreshortening of the facial skeleton--is a discrete mutation that has been selected for in many popular dog breeds e.g. the Bulldog, Pug, and French Bulldog. A chronic, debilitating respiratory syndrome, whereby soft tissue blocks the airways, predominantly affects dogs with this conformation, and thus is labelled Brachycephalic Obstructive Airway Syndrome (BOAS. Despite the name of the syndrome, scientific evidence quantitatively linking brachycephaly with BOAS is lacking, but it could aid efforts to select for healthier conformations. Here we show, in (1 an exploratory study of 700 dogs of diverse breeds and conformations, and (2 a confirmatory study of 154 brachycephalic dogs, that BOAS risk increases sharply in a non-linear manner as relative muzzle length shortens. BOAS only occurred in dogs whose muzzles comprised less than half their cranial lengths. Thicker neck girths also increased BOAS risk in both populations: a risk factor for human sleep apnoea and not previously realised in dogs; and obesity was found to further increase BOAS risk. This study provides evidence that breeding for brachycephaly leads to an increased risk of BOAS in dogs, with risk increasing as the morphology becomes more exaggerated. As such, dog breeders and buyers should be aware of this risk when selecting dogs, and breeding organisations should actively discourage exaggeration of this high-risk conformation in breed standards and the show ring.

  8. Neurocognitive function in obstructive sleep apnoea: a meta-review.

    Science.gov (United States)

    Bucks, Romola S; Olaithe, Michelle; Eastwood, Peter

    2013-01-01

    Adult obstructive sleep apnoea (OSA) is associated with cognitive dysfunction. While many review articles have attempted to summarize the evidence for this association, it remains difficult to determine which domains of cognition are affected by OSA. This is because of marked differences in the nature of these reviews (e.g. many are unsystematic) and the many different tasks and domains assessed. This paper addresses this issue by comparing the results of only systematic reviews or meta-analyses assessing the effects of OSA on cognition, the relationship between OSA severity and cognition, and/or the effects of treatment on cognition in OSA. Electronic databases and hand-searching were undertaken to select reviews that reported on these areas. We found 33 reviews; five reviews met predetermined, stringent selection criteria. The majority of reviews supported deficits in attention/vigilance, delayed long-term visual and verbal memory, visuospatial/constructional abilities, and executive function in individuals with OSA. There is also general agreement that language ability and psychomotor function are unaffected by OSA. Data are equivocal for the effects of OSA on working memory, short-term memory and global cognitive functioning. Attention/vigilance dysfunction appears to be associated with sleep fragmentation and global cognitive function with hypoxaemia. Continuous positive airway pressure treatment of OSA appears to improve executive dysfunction, delayed long-term verbal and visual memory, attention/vigilance and global cognitive functioning. In order to improve our understanding of cognitive dysfunction in OSA, future research should pay particular attention to participant characteristics, measures of disease severity and choice of neuropsychological tests. © 2012 The Authors. Respirology © 2012 Asian Pacific Society of Respirology.

  9. Oral tranexamic acid lightens refractory melasma.

    Science.gov (United States)

    Tan, Aaron Wei Min; Sen, Priya; Chua, Sze Hon; Goh, Boon Kee

    2017-08-01

    Melasma is a common acquired hyperpigmentary disorder, particularly among Asians and Hispanics, but its exact pathomechanism is poorly understood. Tranexamic acid has been found to lighten melasma by interfering with the interaction of melanocytes and keratinocytes by inhibiting the plasminogen/plasmin system. The aim was to evaluate the therapeutic effects of oral tranexamic acid in the treatment of melasma refractory to topical skin-lightening agents. This retrospective study analyses patients with melasma recruited from a tertiary dermatological centre in Singapore between 1 August 2009 and 31 March 2011. The patients chosen had refractory melasma treated with oral tranexamic acid 250 mg twice daily in addition to pre-existing combination topical therapy. Objective assessment using the physician's global assessment and melasma area and severity index (MASI) scores were performed based on a post-hoc analysis of photographic records by three independent physicians. A paired t-test was used to evaluate the changes in the MASI scores pre-therapy and post-treatment. Statistical significance was defined as P tranexamic acid for a mean period of 3.7 ± 0.33 months, in addition to combination topical therapy. Their mean age was 47.2 ± 1.61 years. The mean MASI scores after tranexamic acid treatment (2.7 ± 1.6) were significantly lower (P tranexamic acid can serve as a safe and useful adjunct in the treatment of refractory melasma. © 2016 The Australasian College of Dermatologists.

  10. Driving simulator and neuropsychological [corrected] testing in OSAS before and under CPAP therapy.

    Science.gov (United States)

    Orth, M; Duchna, H-W; Leidag, M; Widdig, W; Rasche, K; Bauer, T T; Walther, J W; de Zeeuw, J; Malin, J-P; Schultze-Werninghaus, G; Kotterba, S

    2005-11-01

    Patients with obstructive sleep apnoea syndrome (OSAS) have an increased car accident rate. Investigations on accident frequency are based on case history, insurance reports and driving simulator studies. The present study combines neuropsychological testing of different attention aspects engaged in driving a car and driving simulation to evaluate a suitable instrument for assessing therapeutic effects of continuous positive airway pressure (CPAP). Driving simulator investigation and neuropsychological testing of alertness, vigilance and divided attention were performed in 31 patients with polysomnographically confirmed OSAS (apnoea-hypopnoea index 24.8+/-21.5.h(-1)) before, and 2 and 42 days after initiation of CPAP. Divided attention and alertness improved significantly during CPAP, whereas vigilance remained unchanged. However, accident frequency (OSAS before therapy: 2.7+/-2.0; 2 days after CPAP: 1.5+/-1.4; 42 days after CPAP: 0.9+/-1.3) and frequency of concentration faults (OSAS before therapy: 12.4+/-5.1; 2 days after CPAP: 6.5+/-3.9; 42 days after CPAP: 4.9+/-3.3) decreased in the simulated driving situation after 2 and 42 days of therapy. There was no relation between accident frequency, concentration faults and daytime sleepiness, as measured by the Epworth Sleepiness Scale, and polysomnographic or neuropsychological findings, respectively. In conclusion, the present results suggest that driving simulation is a possible benchmark parameter of driving performance in obstructive sleep apnoea syndrome patients.

  11. Clinical features and course of refractory anemia with ring sideroblasts associated with marked thrombocytosis

    Science.gov (United States)

    Broseus, Julien; Florensa, Lourdes; Zipperer, Esther; Schnittger, Susanne; Malcovati, Luca; Richebourg, Steven; Lippert, Eric; Cermak, Jaroslav; Evans, Jyoti; Mounier, Morgane; Raya, José Maria; Bailly, François; Gattermann, Norbert; Haferlach, Torsten; Garand, Richard; Allou, Kaoutar; Besses, Carlos; Germing, Ulrich; Haferlach, Claudia; Travaglino, Erica; Luno, Elisa; Pinan, Maria Angeles; Arenillas, Leonor; Rozman, Maria; Perez Sirvent, Maria Luz; Favre, Bernardine; Guy, Julien; Alonso, Esther; Ahwij, Nuhri; Jerez, Andrés; Hermouet, Sylvie; Maynadié, Marc; Cazzola, Mario; Girodon, François

    2012-01-01

    Background Refractory anemia with ring sideroblasts associated with marked thrombocytosis was proposed as a provisional entity in the 2001 World Health Organization classification of myeloid neoplasms and also in the 2008 version, but its existence as a single entity is contested. We wish to define the clinical features of this rare myelodysplastic/myeloproliferative neoplasm and to compare its clinical outcome with that of refractory anemia with ring sideroblasts and essential thrombocythemia. Design and Methods We conducted a collaborative retrospective study across Europe. Our database included 200 patients diagnosed with refractory anemia with ring sideroblasts and marked thrombocytosis. For each of these patients, each patient diagnosed with refractory anemia with ring sideroblasts was matched for age and sex. At the same time, a cohort of 454 patients with essential thrombocythemia was used to compare outcomes of the two diseases. Results In patients with refractory anemia with ring sideroblasts and marked thrombocytosis, depending on the Janus Kinase 2 V617F mutational status (positive or negative) or platelet threshold (over or below 600×109/L), no difference in survival was noted. However, these patients had shorter overall survival and leukemia-free survival with a lower risk of thrombotic complications than did patients with essential thrombocythemia (P<0.001) but better survival (P<0.001) and a higher risk of thrombosis (P=0.039) than patients with refractory anemia with ring sideroblasts. Conclusions The clinical course of refractory anemia with ring sideroblasts and marked thrombocytosis is better than that of refractory anemia with ring sideroblasts and worse than that of essential thrombocythemia. The higher risk of thrombotic events in this disorder suggests that anti-platelet therapy might be considered in this subset of patients. From a clinical point of view, it appears to be important to consider refractory anemia with ring sideroblasts and

  12. Nanostructured refractory thin films for solar applications

    Science.gov (United States)

    Ollier, E.; Dunoyer, N.; Dellea, O.; Szambolics, H.

    2014-08-01

    Selective solar absorbers are key elements of all solar thermal systems. Solar thermal panels and Concentrated Solar Power (CSP) systems aim respectively at producing heat and electricity. In both cases, a surface receives the solar radiation and is designed to have the highest optical absorption (lowest optical reflectivity) of the solar radiation in the visible wavelength range where the solar intensity is the highest. It also has a low emissivity in the infrared (IR) range in order to avoid radiative thermal losses. Current solutions in the state of the art usually consist in deposited interferential thin films or in cermets [1]. Structured surfaces have been proposed and have been simulated because they are supposed to be more efficient when the solar radiation is not normal to the receiving surface and because they could potentially be fabricated with refractory materials able to sustain high operating temperatures. This work presents a new method to fabricate micro/nanostructured surfaces on molybdenum (refractory metal with a melting temperature of 2623°C). This method now allows obtaining a refractory selective surface with an excellent optical selectivity and a very high absorption in the visible range. This high absorption performance was obtained by achieving a double structuration at micro and nano scales thanks to an innovative process flow.

  13. Low-Chrome/Chrome Free Refractories for Slagging Gasifiers

    International Nuclear Information System (INIS)

    Bennett, J.P.; Kwong, K.-S.; Powell, C.P.; Thomas, H.; Petty, A.V. Jr.

    2007-01-01

    Gasifiers are containment vessels used to react carbon-containing materials with oxygen and water, producing syngas (CO and H2) that is used in chemical and power production. It is also a potential source of H2 in a future hydrogen economy. Air cooled slagging gasifiers are one type of gasifier, operating at temperatures from 1275-1575 C and at pressures of 400 psi or higher. They typically use coal or petroleum coke as the carbon source, materials which contain ash impurities that liquefy at the gasification temperatures, producing liquid slag in quantities of 100 or more tons/day, depending on the carbon fed rate and the percent ash present in the feedstock. The molten slag is corrosive to refractory linings, causing chemical dissolution and spalling. The refractory lining is composed of chrome oxide, alumina, and zirconia; and is replaced every 3-24 months. Gasifier users would like greater on-line availability and reliability of gasifier liners, something that has impacted gasifier acceptance by industry. Research is underway at NETL to improve refractory service life and to develop a no-chrome or low-chrome oxide alternative refractory liner. Over 250 samples of no- or low-chrome oxide compositions have been evaluated for slag interactions by cup testing; with potential candidates for further studies including those with ZrO2, Al2O3, and MgO materials. The development of improved liner materials is necessary if technologies such as IGCC and DOE's Near Zero Emissions Advanced Fossil Fuel Power Plant are to be successful and move forward in the marketplace

  14. Long-term evaluation of treatment of chronic, therapeutically refractory tinnitus by neurostimulation

    NARCIS (Netherlands)

    Staal, M. J.; Holm, A. F.; Mooij, J. J. A.; Albers, F. W. J.; Bartels, H.

    2007-01-01

    Objective: Long-term evaluation of treatment of chronic, therapeutically refractory tinnitus by means of chronic electrical stimulation of the vestibulocochlear nerve. Patients: Inclusion criteria were severe, chronic, therapeutically refractory, unilateral tinnitus and severe hearing loss at the

  15. Super-Refractory Status Epilepticus: Report of a Case and Review of the Literature.

    Science.gov (United States)

    Lapenta, Leonardo; Frisullo, Giovanni; Vollono, Catello; Brunetti, Valerio; Giannantoni, Nadia Mariagrazia; Sandroni, Claudio; Di Lella, Giuseppe; Della Marca, Giacomo

    2015-10-01

    Super-refractory status epilepticus (SE; ie, SE continuing or recurring despite 24 hours of general anesthesia) is a severe condition with high percentage of mortality and morbidity. Usually, this condition occurs because of serious brain damage; nevertheless, some patients develop super-refractory SE without identifiable etiology. Although not uncommonly encountered in neurointensive care, scientific data on this condition are still lacking in terms of treatment and prognosis. Herein, we report a case of super-refractory SE with recovery after 50 days, despite electroencephalographic (EEG) and magnetic resonance imaging (MRI) signs traditionally related to poor prognosis. A review of the literature on super-refractory SE is also presented. © EEG and Clinical Neuroscience Society (ECNS) 2014.

  16. Influence of craniofacial and upper spine morphology on mandibular advancement device treatment outcome in patients with obstructive sleep apnoea

    DEFF Research Database (Denmark)

    Svanholt, Palle; Petri, Niels; Wildschiødtz, Gordon

    2015-01-01

    Summary BACKGROUND/OBJECTIVES: The aim of the study was to assess cephalometric predictive markers in terms of craniofacial morphology including posterior cranial fossa and upper spine morphology for mandibular advancement device (MAD) treatment outcome in patients with obstructive sleep apnoea...... patients and the no success treatment group of 19 patients. Before MAD treatment lateral cephalograms were taken and analyses of the craniofacial morphology including the posterior cranial fossa and upper spine morphology were performed. Differences between the groups were analysed by Fisher's exact test......, t-test, and multiple regression analysis. RESULTS: Upper spine morphological deviations occurred non-significantly in 25 per cent in the success treatment group and in 42.1 per cent in the no success treatment group. Body mass index (BMI; P

  17. Tioguanine in the treatment of refractory coeliac disease--a single centre experience.

    Science.gov (United States)

    Tack, G J; van Asseldonk, D P; van Wanrooij, R L J; van Bodegraven, A A; Mulder, C J

    2012-08-01

    Refractory coeliac disease type I is a complicated form of coeliac disease characterised by primary or secondary resistance to a gluten-free diet with persisting or reoccurring intestinal villous atrophy and symptoms of malabsorption. Besides corticosteroids, azathioprine has been advocated for the treatment of refractory coeliac disease type I. However, tioguanine (TG) might be better tolerated and more efficacious owing to a simpler metabolism towards bioactivation. To evaluate tolerability and effectiveness of the nonconventional thiopurine derivative TG in refractory coeliac disease type I. Refractory coeliac disease type I patients treated with TG between June 2001 and November 2010 with a follow-up period of at least 1 year were included. Adverse events, laboratory values, 6-thioguanine nucleotide concentrations and rates of both clinical and histological response were evaluated at baseline and during follow-up. Twelve adult refractory coeliac disease type I patients were included. The median TG treatment duration was 14 months. Ten patients tolerated TG treatment on the long term, whereas two patients withdrew treatment due to adverse events. No nodular regenerative hyperplasia of the liver was observed. During follow-up clinical and histological response was observed in 83% and 78%, respectively. Corticosteroid dependency decreased by 50%. Tioguanine appears to be a convenient drug for the treatment of refractory coeliac disease type I based on higher histological and similar clinical response rates as compared with historical conventional therapies. © 2012 Blackwell Publishing Ltd.

  18. Refractory, Abrasive and Other Industrial Mineral Operations

    Data.gov (United States)

    Department of Homeland Security — This map layer includes refractory, abrasive, and other industrial minerals operations in the United States. The data represent commodities covered by the Minerals...

  19. Evaluation of nutritional status in children with refractory epilepsy

    Directory of Open Access Journals (Sweden)

    Testolin G

    2006-04-01

    Full Text Available Abstract Background children affected by refractory epilepsy could be at risk of malnutrition because of feeding difficulties (anorexia, chewing, swallowing difficulties or vomiting and chronic use of anticonvulsants, which may affect food intake and energy metabolism. Moreover, their energy requirement may be changed as their disabilities would impede normal daily activities. The aim of the present study was to evaluate nutritional status, energy metabolism and food intake in children with refractory epilepsy. Methods 17 children with refractory epilepsy (13 boys and 4 girls; mean age 9 ± 3,2 years; Body Mass Index 15,7 ± 3,6 underwent an anthropometric assessment, body composition evaluation by dual-energy X-ray absorptiometry, detailed dietetic survey and measurement of resting energy expenditure by indirect calorimetry. Weight-for-age, height-for-age (stunting and weight-for-height (wasting were estimated compared to those of a reference population of the same age. Results 40% of children were malnourished and 24% were wasted. The nutritional status was worse in the more disabled children. Dietary intake resulted unbalanced (18%, 39%, 43% of total daily energy intake derived respectively from protein, lipid and carbohydrate. Adequacy index [nutrient daily intake/recommended allowance (RDA × 100] was Conclusion many children with refractory epilepsy would benefit from individual nutritional assessment and management as part of their overall care.

  20. Ogilvie's syndrome (acute colonic pseudo-obstruction) after caesarean section

    International Nuclear Information System (INIS)

    Munzar, Z.; Munir, T. A.; Asad, M.

    2013-01-01

    Ogilvie's syndrome (OS), a massive dilation of the colon in the absence of mechanical obstruction, is a rare postsurgical complication. The high mortality rate (normally 15 - 31% and upto 45% after caecal perforation) explains the seriousness of this clinical situation. Early diagnosis is made by plain abdominal X-ray and CT abdomen. Conservative treatment is usually effective and surgery should be reserved for complicated cases or refractory to conservative treatment. We report a case of 25 years primigravida who developed OS, 24 hours after cesarean section, which was treated by conservative methods. (author)

  1. Reduction of central neuropathic pain with ketamine infusion in a patient with Ehlers–Danlos syndrome: a case report

    Directory of Open Access Journals (Sweden)

    Lo TC

    2016-09-01

    Full Text Available Tony Chung Tung Lo,1,* Stephen Tung Yeung,2,* Sujin Lee,1 Kira Skavinski,3 Solomon Liao,4 1Department of Physical Medicine and Rehabilitation, University of California Irvine, Orange, CA, 2Department of Immunology, University of Connecticut School of Medicine, Farmington, CT, 3Department of Palliative Medicine, University of California San Diego, La Jolla, 4Department of Palliative Medicine, University of California Irvine, Orange, CA, USA *These authors contributed equally to this work Objective: Ehlers–Danlos syndrome frequently causes acute and chronic pain because of joint subluxations and dislocations secondary to hypermobility. Current treatments for pain related to Ehlers–Danlos syndrome and central pain syndrome are inadequate. This case report discusses the therapeutic use of ketamine intravenous infusion as an alternative. Case report: A 27-year-old Caucasian female with a history of Ehlers–Danlos syndrome and spinal cord ischemic myelopathy resulting in central pain syndrome, presented with severe generalized body pain refractory to multiple pharmacological interventions. After a 7-day course of ketamine intravenous infusion under controlled generalized sedation in the intensive care unit, the patient reported a dramatic reduction in pain levels from 7–8 out of 10 to 0–3 out of 10 on a numeric rating scale and had a significant functional improvement. The patient tolerated a reduction in her pain medication regimen, which originally included opioids, gabapentin, pregabalin, tricyclic antidepressants, and nonsteroidal anti-inflammatory drugs. Conclusion: Ketamine infusion treatment has been used in various pain syndromes, including central neuropathic pain, ischemic pain, and regional pain syndrome. Reports have suggested that ketamine modulates pain by the regression of N-methyl-D-aspartate receptor to a resting state. As such, propagation of nociceptive signal to brain is interrupted allowing for the restoration of

  2. Improvement of castable refractories for RH snorkel; RH shinshitsukanyo futeikeizai no kaizen

    Energy Technology Data Exchange (ETDEWEB)

    Nishi, K.; Obana, T.; Fijii, T.; Shimizu, I. [Harima Ceramics Corp., Hyogo (Japan)

    1999-11-01

    Relating to corrosion of RH submerged nozzle, resistance against iron oxide of monolithic refractories was examined. Corrosion and seepage of refractories were measured by rotating corrosion, refractories include alumina-spinel castable, which is applied to RH under tank, and alumina-magnesia castable, which is generally used for RH, submerged nozzle. Alumina-spinel castable is superior in resistance against iron oxide than alumina-magnesia castable, and the resistance decreased with increase of stainless fiber addition to castable. Alumina-spinel castable without stainless fiber was suitable for bottom end of the dipping pipe. (NEDO)

  3. Effectiveness of intravenous levetiracetam as an adjunctive treatment in pediatric refractory status epilepticus.

    Science.gov (United States)

    Kim, Jon Soo; Lee, Jeong Ho; Ryu, Hye Won; Lim, Byung Chan; Hwang, Hee; Chae, Jong-Hee; Choi, Jieun; Kim, Ki Joong; Hwang, Yong Seung; Kim, Hunmin

    2014-08-01

    Intravenous levetiracetam (LEV) has been shown to be effective and safe in treating adults with refractory status epilepticus (SE). We sought to investigate the efficacy and safety of intravenous LEV for pediatric patients with refractory SE. We performed a retrospective medical-record review of pediatric patients who were treated with intravenous LEV for refractory SE. Clinical information regarding age, sex, seizure type, and underlying neurological status was collected. We evaluated other anticonvulsants that were used prior to administration of intravenous LEV and assessed loading dose, response to treatment, and any adverse events from intravenous LEV administration. Fourteen patients (8 boys and 6 girls) received intravenous LEV for the treatment of refractory SE. The mean age of the patients was 4.4 ± 5.5 years (range, 4 days to 14.6 years). Ten of the patients were neurologically healthy prior to the refractory SE, and the other 4 had been previously diagnosed with epilepsy. The mean loading dose of intravenous LEV was 26 ± 4.6 mg/kg (range, 20-30 mg/kg). Seizure termination occurred in 6 (43%) of the 14 patients. In particular, 4 (57%) of the 7 patients younger than 2 years showed seizure termination. No immediate adverse events occurred during or after infusions. The current study demonstrated that the adjunctive use of intravenous LEV was effective and well tolerated in pediatric patients with refractory SE, even in patients younger than 2 years. Intravenous LEV should be considered as an effective and safe treatment option for refractory SE in pediatric patients.

  4. Neuroimaging in refractory epilepsy. Current practice and evolving trends

    Energy Technology Data Exchange (ETDEWEB)

    Ramli, N. [Department of Biomedical Imaging, University Malaya Research Imaging Centre (Malaysia); Rahmat, K., E-mail: katt_xr2000@yahoo.com [Department of Biomedical Imaging, University Malaya Research Imaging Centre (Malaysia); Lim, K.S.; Tan, C.T. [Neurology Unit, Department of Medicine, University Malaya, Kuala Lumpur (Malaysia)

    2015-09-15

    Highlights: • Neuroimaging is imperative in diagnostic work up and therapeutic assessment of refractory epilepsy. • Identification of epileptogenic zone on EEG, MRI and functional imaging improves the success of surgery. • High performance MRI greatly enhanced metabolic information and elucidate brain functions. • Optimisation of epilepsy protocols in structural and functional MRI are presented in this article. - Abstract: Identification of the epileptogenic zone is of paramount importance in refractory epilepsy as the success of surgical treatment depends on complete resection of the epileptogenic zone. Imaging plays an important role in the locating and defining anatomic epileptogenic abnormalities in patients with medically refractory epilepsy. The aim of this article is to present an overview of the current MRI sequences used in epilepsy imaging with special emphasis of lesion seen in our practices. Optimisation of epilepsy imaging protocols are addressed and current trends in functional MRI sequences including MR spectroscopy, diffusion tensor imaging and fusion MR with PET and SPECT are discussed.

  5. Neuroimaging in refractory epilepsy. Current practice and evolving trends

    International Nuclear Information System (INIS)

    Ramli, N.; Rahmat, K.; Lim, K.S.; Tan, C.T.

    2015-01-01

    Highlights: • Neuroimaging is imperative in diagnostic work up and therapeutic assessment of refractory epilepsy. • Identification of epileptogenic zone on EEG, MRI and functional imaging improves the success of surgery. • High performance MRI greatly enhanced metabolic information and elucidate brain functions. • Optimisation of epilepsy protocols in structural and functional MRI are presented in this article. - Abstract: Identification of the epileptogenic zone is of paramount importance in refractory epilepsy as the success of surgical treatment depends on complete resection of the epileptogenic zone. Imaging plays an important role in the locating and defining anatomic epileptogenic abnormalities in patients with medically refractory epilepsy. The aim of this article is to present an overview of the current MRI sequences used in epilepsy imaging with special emphasis of lesion seen in our practices. Optimisation of epilepsy imaging protocols are addressed and current trends in functional MRI sequences including MR spectroscopy, diffusion tensor imaging and fusion MR with PET and SPECT are discussed

  6. Italian real life experience with ibrutinib: results of a large observational study on 77 relapsed/refractory mantle cell lymphoma.

    Science.gov (United States)

    Broccoli, Alessandro; Casadei, Beatrice; Morigi, Alice; Sottotetti, Federico; Gotti, Manuel; Spina, Michele; Volpetti, Stefano; Ferrero, Simone; Spina, Francesco; Pisani, Francesco; Merli, Michele; Visco, Carlo; Paolini, Rossella; Zilioli, Vittorio Ruggero; Baldini, Luca; Di Renzo, Nicola; Tosi, Patrizia; Cascavilla, Nicola; Molica, Stefano; Ilariucci, Fiorella; Rigolin, Gian Matteo; D'Alò, Francesco; Vanazzi, Anna; Santambrogio, Elisa; Marasca, Roberto; Mastrullo, Lucia; Castellino, Claudia; Desabbata, Giovanni; Scortechini, Ilaria; Trentin, Livio; Morello, Lucia; Argnani, Lisa; Zinzani, Pier Luigi

    2018-05-04

    Although sometimes presenting as an indolent lymphoma, mantle cell lymphoma (MCL) is an aggressive disease, hardly curable with standard chemo-immunotherapy. Current approaches have greatly improved patients' outcomes, nevertheless the disease is still characterized by high relapse rates. Before approval by EMA, Italian patients with relapsed/refractory MCL were granted ibrutinib early access through a Named Patient Program (NPP). An observational, retrospective, multicenter study was conducted. Seventy-seven heavily pretreated patients were enrolled. At the end of therapy there were 14 complete responses and 14 partial responses, leading to an overall response rate of 36.4%. At 40 months overall survival was 37.8% and progression free survival was 30%; disease free survival was 78.6% at 4 years: 11/14 patients are in continuous complete response with a median of 36 months of follow up. Hematological toxicities were manageable, and main extra-hematological toxicities were diarrhea (9.4%) and lung infections (9.0%). Overall, 4 (5.2%) atrial fibrillations and 3 (3.9%) hemorrhagic syndromes occurred. In conclusions, thrombocytopenia, diarrhea and lung infections are the relevant adverse events to be clinically focused on; regarding effectiveness, ibrutinib is confirmed to be a valid option for refractory/relapsed MCL also in a clinical setting mimicking the real world.

  7. The effect of various sintering temperature on used refractory towards its physical properties

    Science.gov (United States)

    Sudibyo; Wulandari, Y. R.; Amin, M.; Azhar

    2018-01-01

    The used magnesia refractory from the kiln of cement industry was successfully recycled to new refractory using Kaolin as an adhesive. In this work, the temperatures of sintering were varied from 1000°C to 1500°C. The result shows that the increment temperature effects in sintering process will enhance refractory physical properties such as bulk density, cold crushing strength or pressure strength and thermal conductivity. Meanwhile, the porosity was decreased as the increase of the sintering temperature.

  8. Rapid pacing results in changes in atrial but not in ventricular refractoriness

    NARCIS (Netherlands)

    Schoonderwoerd, BA; Van Gelder, IC; Tieleman, RG; Bel, KJ; Crijns, HJGM

    It is well known that atrial tachycardia causes atrial electrical remodeling, characterized by shortening of atrial effective refractory periods (AERPs) and loss of physiological adaptation of AERP to rate. However, the nature and time course of changes in ventricular effective refractory periods

  9. Cavitatory mesenteric lymph node syndrome: A rare entity

    International Nuclear Information System (INIS)

    Vibhuti; Vishal, Kalia; Gill, Anakhvir

    2010-01-01

    Celiac disease is a gluten sensitive enteropathy that involves an abnormal immunological response to glutens in wheat, rye etc. It predominantly involves the small intestinal mucosa, though, extra luminal manifestations can also occur. One rare extraluminal manifestation is cavitatory mesenteric lymph node syndrome. It occurs in refractory celiac disease and is associated with poor prognosis due to various complications. The diagnosis is often made on imaging when cystic mesenteric lymph nodes with fat-fluid levels are seen and this can then be confirmed by histopathological examination. We recently had a typical case where we were able to make this diagnosis

  10. Transient refractory material dissolution by a volumetrically-heated melt

    Energy Technology Data Exchange (ETDEWEB)

    Seiler, Jean Marie, E-mail: jean-marie.seiler@cea.fr [CEA, DEN, DTN, 17 Rue des Martyrs, 38054 Grenoble Cedex 9 (France); Ratel, Gilles [CEA, DEN, DTN, 17 Rue des Martyrs, 38054 Grenoble Cedex 9 (France); Combeau, Hervé [Institut Jean Lamour, UMR 7198, Lorraine University, Ecole des Mines de Nancy, Parc de Saurupt, 54042 Nancy Cedex (France); Gaus-Liu, Xiaoyang; Kretzschmar, Frank; Miassoedov, Alexei [Karlsruhe Institut of Technology, Hermann-von-Helmholtz-Platz 1, 76344 Eggenstein-Leopoldshafen (Germany)

    2014-12-15

    Highlights: • We describe a test investigating ceramic dissolution by a molten non-eutectic melt. • The evolution of the interface temperature between melt and refractory is measured. • A theoretical model describing dissolution kinetics is proposed. • When dissolution stops, interface temperature is the liquidus temperature of the melt. - Abstract: The present work addresses the question of corium–ceramic interaction in a core catcher during a core-melt accident in a nuclear power plant. It provides an original insight into transient aspects concerning dissolution of refractory material by a volumetrically heated pool. An experiment with simulant material (LIVECERAM) is presented. Test results clearly show that dissolution of solid refractory material can occur in a non-eutectic melt at a temperature which is lower than the melting temperature of the refractory material. During the dissolution transient, the interface temperature rises above the liquidus temperature, corresponding to the instantaneous average composition of the melt pool. With constant power dissipation in the melt and external cooling of the core-catcher, a final steady-state situation is reached. Dissolution stops when the heat flux (delivered by the melt to the refractory) can be removed by conduction through the residual thickness of the ceramic, with T{sub interface} = T{sub liquidus} (calculated for the average composition of the final liquid pool). The final steady state corresponds to a uniform pool composition and uniform interface temperature distribution. Convection in the pool is governed by natural thermal convection and the heat flux distribution is therefore similar to what would be obtained for a single component pool. An interpretation of the experiment with two model-based approaches (0D and 1D) is presented. The mass transfer kinetics between the interface and the bulk is controlled by a diffusion sublayer within the boundary layer. During the dissolution transient

  11. Occipital Nerve Stimulation for the Treatment of Refractory Occipital Neuralgia: A Case Series.

    Science.gov (United States)

    Keifer, Orion P; Diaz, Ashley; Campbell, Melissa; Bezchlibnyk, Yarema B; Boulis, Nicholas M

    2017-09-01

    Occipital neuralgia is a chronic pain syndrome characterized by sharp, shooting pains in the distribution of the occipital nerves. Although relatively rare, it associated with extremely debilitating symptoms that drastically affect a patient's quality of life. Furthermore, it is extremely difficult to treat as the symptoms are refractory to traditional treatments, including pharmacologic and procedural interventions. A few previous case studies have established the use of a neurostimulation of the occipital nerves to treat occipital neuralgia. The following expands on that literature by retrospectively reviewing the results of occipital nerve stimulation in a relatively large patient cohort (29 patients). A retrospective review of 29 patients undergoing occipital nerve stimulation for occipital neuralgia from 2012 to 2017 at a single institution with a single neurosurgeon. Of those 29 patients, 5 were repair or replacement of previous systems, 4 did not have benefit from trial stimulation, and 20 saw benefit to their trial stage of stimulation and went on to full implantation. Of those 20 patients, even with a history of failed procedures and pharmacological therapies, there was an overall success rate of 85%. The average preoperative 10-point pain score dropped from 7.4 ± 1.7 to a postoperative score of 2.9 ± 1.7. However, as with any peripheral nerve stimulation procedure, there were complications (4 patients), including infection, hardware erosion, loss of effect, and lead migration, which required revision or system removal. Despite complications, the results suggest, overall, that occipital nerve stimulation is a safe and effective procedure for refractory occipital neuralgia and should be in the neurosurgical repertoire for occipital neuralgia treatment. Copyright © 2017 Elsevier Inc. All rights reserved.

  12. A randomized controlled trial of the ketogenic diet in refractory childhood epilepsy

    NARCIS (Netherlands)

    Lambrechts, D.A.J.E.; de Kinderen, R.J.A.; Vles, J.S.H.; de Louw, A.J.A.; Aldenkamp, A.P.; Majoie, H.J.M.

    2017-01-01

    Objective: To evaluate the efficacy and tolerability of the ketogenic diet (KD) during the first 4 months of a randomized controlled trial (RCT) in refractory epilepsy patients aged 1–18 years. Methods: Children and adolescents with refractory epilepsy, not eligible for epilepsy surgery, were

  13. Improved cooler design of electric arc furnace refractory in mining industry using thermal analysis modeling and simulation

    International Nuclear Information System (INIS)

    Istadi, I.; Bindar, Y.

    2014-01-01

    Production of steel and nickel using the electric arc furnace should be focused on the intensification of energy. Improvement of energy efficiency of the most consuming facilities was achieved by improving the use of alternative energy minimization such as reducing the heat lost of hot gases, minimizing the heat radiated through refractory linings of metallurgical furnaces, and cooling the highly thermally stressed components. The refractory of electric arc furnace should be modified to achieve the best cooling system of the furnace. In this physical modeling and simulation works, four modification scenarios of wall refractory designs were simulated, i.e. refractory with basic design, refractory with deep plate coolers, refractory with extra plate coolers, and refractory with wall falling film coolers. Finally, the use of deep plate cooler and the existing waffle cooler system was considered to be the best design of efficient electric arc furnace operationally. - Highlights: • Electric arc furnace design should be focused on the intensification of energy. • Refractory of electric arc furnace were modified to achieve the best cooling system. • Four modification scenarios of the wall refractory designs were simulated. • Use of deep plate cooler and existing waffle cooler system is the best cooling

  14. Ixabepilone: a new chemotherapeutic option for refractory metastatic breast cancer

    Directory of Open Access Journals (Sweden)

    Shannon Puhalla

    2008-09-01

    Full Text Available Shannon Puhalla, Adam BrufskyUPMC Magee-Womens Cancer Program, University of Pittsburgh, Pittsburgh, Pennsylvania, USAAbstract: Taxane therapy is commonly used in the treatment of metastatic breast cancer. However, most patients will eventually become refractory to these agents. Ixabepilone is a newly approved chemotherapeutic agent for the treatment of metastatic breast cancer. Although it targets microtubules similarly to docetaxel and paclitaxel, ixabepilone has activity in patients that are refractory to taxanes. This review summarizes the pharmacology of ixapebilone and clinical trials with the drug both as a single agent and in combination. Data were obtained using searches of PubMed and abstracts of the annual meetings of the American Society of Clinical Oncology and the San Antonio Breast Cancer Symposium from 1995 to 2008. Ixapebilone is a semi-synthetic analog of epothilone B that acts to induce apoptosis of cancer cells via the stabilization of microtubules. Phase I clinical trials have employed various dosing schedules ranging from daily to weekly to 3-weekly. Dose-limiting toxicites included neuropathy and neutropenia. Responses were seen in a variety of tumor types. Phase II studies verified activity in taxane-refractory metastatic breast cancer. The FDA has approved ixabepilone for use as monotherapy and in combination with capecitabine for the treatment of metastatic breast cancer. Ixabepilone is an efficacious option for patients with refractory metastatic breast cancer. The safety profile is similar to that of taxanes, with neuropathy and neutropenia being dose-limiting. Studies are ongoing with the use of both iv and oral formulations and in combination with other chemotherapeutic and biologic agents.Keywords: ixabepilone, epothilone, metastatic breast cancer, taxane-refractory

  15. Prednisone and vardenafil hydrochloride for refractory levamisole-induced vasculitis.

    Science.gov (United States)

    Mandrell, Joshua; Kranc, Christina L

    2016-08-01

    Levamisole is an immunomodulatory drug that was previously used to treat various medical conditions, including parasitic infections, nephrotic syndrome, and colorectal cancer. Over the last few years, increasing amounts of levamisole have been used as an adulterant in cocaine. Levamisole-cut cocaine has become a concern because it is known to cause a necrotizing purpuric rash, autoantibody production, and life-threatening leukopenia. Mixed histologic findings of vasculitis and thrombosis are characteristic of levamisole-induced purpura. The recommended management of levamisole-induced vasculitis currently involves withdrawal of the culprit along with supportive treatment. We describe a patient with levamisole-induced vasculitis who continued to develop skin lesions despite self-reported cocaine cessation. Complete resolution of cutaneous disease occurred with the addition of oral prednisone and vardenafil hydrochloride, suggesting the possibility of a new treatment option in patients with refractory disease. In addition, we review the clinical presentation, disease course, diagnostic approach, laboratory findings, histology, and management of levamisole-induced vasculitis. The harmful effects of levamisole-cut cocaine are serious enough that public alerts have been issued to increase awareness. Clinicians should consider the possibility of levamisole exposure in cocaine users presenting with any combination of fever, neutropenia, and necrotic skin lesions, especially in acral areas including the ears.

  16. Regorafenib Versus Trifluridine/Tipiracil for Refractory Metastatic Colorectal Cancer: A Retrospective Comparison.

    Science.gov (United States)

    Masuishi, Toshiki; Taniguchi, Hiroya; Hamauchi, Satoshi; Komori, Azusa; Kito, Yosuke; Narita, Yukiya; Tsushima, Takahiro; Ishihara, Makoto; Todaka, Akiko; Tanaka, Tsutomu; Yokota, Tomoya; Kadowaki, Shigenori; Machida, Nozomu; Ura, Takashi; Fukutomi, Akira; Ando, Masashi; Onozawa, Yusuke; Tajika, Masahiro; Yasui, Hirofumi; Muro, Kei; Mori, Keita; Yamazaki, Kentaro

    2017-06-01

    Regorafenib and trifluridine/tipiracil (TAS-102) both prolong survival for patients with refractory metastatic colorectal cancer. However, it is unclear which drug should be administered first. We retrospectively evaluated the data from patients who had received regorafenib or TAS-102 at 2 institutions from May 2013 to March 2015. The inclusion criteria were disease refractory or intolerant to fluoropyrimidines, oxaliplatin, irinotecan, anti-vascular endothelial growth factor antibodies, and anti-epidermal growth factor receptor (EGFR) antibodies (if KRAS exon 2 wild-type), and no previous treatment with regorafenib or TAS-102. A total of 146 and 54 patients received regorafenib and TAS-102, respectively. The baseline characteristics were similar between the 2 groups, except for a history of irinotecan and anti-EGFR therapy and high alkaline phosphatase levels. The median progression-free survival and overall survival were 2.1 months and 6.7 months, respectively, with regorafenib and 2.1 months and 6.5 months, respectively, with TAS-102 (progression-free survival hazard ratio 1.20, P = .27; overall survival hazard ratio, 1.01, P = .97). The analysis of overall survival for patients after the approval of TAS-102 in Japan was similar to the overall survival for the entire population. The frequency of hand-foot syndrome and increased aspartate aminotransferase, alanine aminotransferase, and bilirubin levels was higher and the frequency of neutropenia, leukopenia, anemia, nausea, and febrile neutropenia was lower with regorafenib than with TAS-102. No remarkable differences were found in the efficacy and safety of TAS-102 between patients with and without previous regorafenib and vice versa. Regorafenib and TAS-102 had similar efficacy but resulted in different toxicities, which could guide the agent choice. Copyright © 2016 Elsevier Inc. All rights reserved.

  17. Clinical outcomes of intravenous immunoglobulin therapy in refractory uveitis.

    Science.gov (United States)

    Garcia-Geremias, M; Carreño, E; Epps, S J; Lee, R W J; Dick, A D

    2015-04-01

    Intravenous immunoglobulin (IVIg) therapy has multiple mechanisms of immunomodulatory action. We wished therefore to assess its efficacy in a spectrum of patients with refractory uveitis. Retrospective review of clinical charts was conducted to document response to IVIg treatment in consecutive patients with treatment-refractory uveitis. Main outcome measures were control of intraocular inflammation, visual acuity, progression of the disease, and complications. Four (two male) patients, with a mean age at the beginning of the treatment of 47 years (range: 39-64), were included in the study. Indication for treatment was patients with active non-infectious uveitis refractory to steroids and immunomodulatory therapy. All patients received a course of 0.5 g/kg per day of IVIg for three consecutive days, repeating this course at a mean of 11 week (range: 2-39 weeks) intervals when indicated clinically. The median duration of the IVIg therapy was 7 months (range: 3-14 months). In three patients treatment resulted in stabilisation and prevention of progression of the disease, and additionally in two patients it facilitated a decrease in prednisolone dose. Treatment failed to induce long-term remission in one patient with recurrence of macular oedema. IVIg was well tolerated with neither immediate nor longer-term adverse events observed. In three out of four cases IVIg was an effective adjunctive therapy and well tolerated for the management of treatment-refractory uveitis.

  18. Use of bibloc and monobloc oral appliances in obstructive sleep apnoea: a multicentre, randomized, blinded, parallel-group equivalence trial.

    Science.gov (United States)

    Isacsson, Göran; Nohlert, Eva; Fransson, Anette M C; Bornefalk-Hermansson, Anna; Wiman Eriksson, Eva; Ortlieb, Eva; Trepp, Livia; Avdelius, Anna; Sturebrand, Magnus; Fodor, Clara; List, Thomas; Schumann, Mohamad; Tegelberg, Åke

    2018-05-16

    The clinical benefit of bibloc over monobloc appliances in treating obstructive sleep apnoea (OSA) has not been evaluated in randomized trials. We hypothesized that the two types of appliances are equally effective in treating OSA. To compare the efficacy of monobloc versus bibloc appliances in a short-term perspective. In this multicentre, randomized, blinded, controlled, parallel-group equivalence trial, patients with OSA were randomly assigned to use either a bibloc or a monobloc appliance. One-night respiratory polygraphy without respiratory support was performed at baseline, and participants were re-examined with the appliance in place at short-term follow-up. The primary outcome was the change in the apnoea-hypopnea index (AHI). An independent person prepared a randomization list and sealed envelopes. Evaluating dentist and the biomedical analysts who evaluated the polygraphy were blinded to the choice of therapy. Of 302 patients, 146 were randomly assigned to use the bibloc and 156 the monobloc device; 123 and 139 patients, respectively, were analysed as per protocol. The mean changes in AHI were -13.8 (95% confidence interval -16.1 to -11.5) in the bibloc group and -12.5 (-14.8 to -10.3) in the monobloc group. The difference of -1.3 (-4.5 to 1.9) was significant within the equivalence interval (P = 0.011; the greater of the two P values) and was confirmed by the intention-to-treat analysis (P = 0.001). The adverse events were of mild character and were experienced by similar percentages of patients in both groups (39 and 40 per cent for the bibloc and monobloc group, respectively). The study shows short-term results with a median time from commencing treatment to the evaluation visit of 56 days and long-term data on efficacy and harm are needed to be fully conclusive. In a short-term perspective, both appliances were equivalent in terms of their positive effects for treating OSA and caused adverse events of similar magnitude. Registered with Clinical

  19. Retreatment with intravenous immunoglobulin (IVIG of refractory Guillain Barre syndrome in children

    Directory of Open Access Journals (Sweden)

    Rivas Larrauri Francisco

    2014-07-01

    Full Text Available Guillain-Barré syndrome (GBS is an acute symmetrical pa- ralyzing disease due to a demyelinating polyrradiculoneuropathy, often induced by a preceding infection 1. The main modalities for the treatment of GBS include plasmapheresis and intrave- nous immune globulin. Reports of the use of IVIG in children with GBS are limited: 1 g/kg for two days or 400 mg/kg for five days. While these studies in children are not definitive because of design limitations, their results are consistent with the larger randomized trials in adults 2,3.

  20. Advances in refractories for the metallurgical industries III: proceedings of the international symposium

    Energy Technology Data Exchange (ETDEWEB)

    Allaire, C.; Rigaud, M. [eds.] [Ecole Polytchnique, Montreal, PQ (Canada)

    1999-07-01

    In the three years since the last symposium key innovations have been made in the penetration of monoliths in all sectors of the metallurgical industries. The symposium was presented within the 38th Annual Conference of Metallurgists of the Canadian Institute of Mining, Metallurgy and Petroleum. Broad international coverage was maintained with papers originating from 12 different countries including: Australia, Canada, China, France, Germany, India, Iran, Japan, Korea, Mexico, New Zealand, Norway, and the United States. The 27 papers presented are divided into seven sessions to reflect the interests of the participants. The sessions included mold fluxes I and II, aluminum refractories, alumina castables, andalusite castables, continuous casting of refractories, aluminum refractories II and copper-nickel refineries. One paper on refractories for exhaust gas scrubbers is abstracted separately.