WorldWideScience

Sample records for airways dysfunction syndrome

  1. Acute expanded perlite exposure with persistent reactive airway dysfunction syndrome.

    Science.gov (United States)

    Du, Chung-Li; Wang, Jung-Der; Chu, Po-Chin; Guo, Yue-Liang Leon

    2010-01-01

    Expanded perlite has been assumed as simple nuisance, however during an accidental spill out in Taiwan, among 24 exposed workers followed for more than 6 months, three developed persisted respiratory symptoms and positive provocation tests were compatible with reactive airway dysfunction syndrome. During simulation experiment expanded perlite is shown to be very dusty and greatly exceed current exposure permission level. Review of literature and evidence, though exposure of expanded perlite below permission level may be generally safe, precautionary protection of short term heavy exposure is warranted.

  2. Reactive airways dysfunction syndrome (RADS): fact or fantasy?

    Science.gov (United States)

    Bardana, E J

    1999-01-01

    Most evidence suggests that RADS is a distinct syndrome and not simply an exacerbation of pre-existing hyperresponsiveness. There is no doubt that much remains to be explained regarding pathogenesis. On the other hand, I urge caution about the claims of "low-level RADS". Here the data are much more anecdotal and leave much to be desired. PMID:10735647

  3. Inhalational exposure to dimethyl sulfate vapor followed by reactive airway dysfunction syndrome

    Directory of Open Access Journals (Sweden)

    Aghabiklooei Abbas

    2010-01-01

    Full Text Available Dimethyl sulfate (DMS is an oily liquid used as a solvent, stabilizer, sulfonation agent, and catalyst. Exposure to DMS primarily happens in the workplace via inhalational contact and damages the upper and lower airways. Our manuscript reports a case of DMS-related reactive airway dysfunction syndrome ( RADS. The patient was a healthy 29-year-old man who was referred to our ER after accidental exposure to the vapor of DMS with the complaint of dyspnea, dry cough, photophobia, and hoarseness. His vital signs were normal except for a low-grade fever. Redness of the pharynx, conjunctivitis, and cholinergic signs and symptoms were present. Conservative management with O 2 and fluid therapy was initiated. Twenty hours later, the patient became drowsy and his respiratory symptoms exacerbated; chest X-ray revealed haziness in the base of the right lung and prominence of the vessels of the lung hillum. After 1 week, the liver transaminases rose and C-reactive protein elevated (2+. The patient got better with conservative treatment and was discharged after 9 days; however, exertional dyspnea, wheezing, and thick white sputum persisted and therefore, reactive airway dysfunction syndrome (RADS related to DMS vapor was confirmed which was treated by prednisolone. Exertional dyspnea continued up to 10 months. Hoarseness lasted for 6 months. This case shows that DMS vapor inhalation can cause RADS especially in the chemical workers who continue working in the contaminated place despite the relatively good air conditioning.

  4. First Reported Case of Reactive Airway Dysfunction Syndrome in a Laborer Due to Porcelain Tile Dust

    Directory of Open Access Journals (Sweden)

    Tasleem Arif

    2013-06-01

    Full Text Available Reactive airway dysfunction syndrome (RADS is a type of non-immunologically mediated asthma-like disease. It usually occurs after a massive exposure to an irritating substance in the atmosphere in the form of smoke, fumes, gases, and vapor. Unlike bronchial asthma, there is no latency to the symptoms seen in RADS. A number of agents are known to cause RADS, but tile dust, as an etiological agent, has not been previously reported. We report a 45-year-old male laborer, who presented with an acute onset of cough, chest tightness, breathlessness, and audible wheeze after his first time exposure to porcelain tile dust within 5 hours of exposure. Lab tests, including, chest X-ray, electrocardiogram, air blood gas analysis, and serum IgE, were unremarkable. Spirometry showed a mild obstruction [forced expiratory volume in 1 second (FEV1=72% of predicted], while the bronchodilator reversibility test was significant(14% increase in FEV1 above the baseline.Bronchial biopsy revealed a chronic inflammatory reaction with lymphocytic and plasma cell infiltration and more importantly a striking absence of eosinophils. To the best of our knowledge, this is the first reported case of RADS as a result of exposure to tile dust (porcelain ceramics.

  5. Reactive airways dysfunction syndrome (RADS): guidelines for diagnosis and treatment and insight into likely prognosis.

    Science.gov (United States)

    Bardana, E J

    1999-12-01

    Reactive airways dysfunction syndrome (RADS) is defined as the sudden onset of asthma following a high level exposure to a corrosive gas, vapor, or fume. This variant of occupational asthma continues to generate controversy regarding the criteria for its diagnosis. There is also some disagreement as to the likely prognosis with this disorder. Currently, the diagnosis requires the assumption of normal premorbid pulmonary physiology and absence of bronchial hyperreactivity. Criteria for the diagnosis of RADS are discussed with a proposal for both major and minor criteria to increase the confidence of an accurate diagnosis. The pathology of RADS involves a primarily lymphocytic inflammatory response with some evidence of subepithelial thickening and fibrosis. Most patients with this condition who survive the short-term exposure to a toxicant recover completely without significant clinical or physiologic sequelae. The issue of low-level RADs remains controversial and problematic as a tenable diagnosis, and will require further careful investigation to evaluate the premise that chronic, low-level toxicants are capable of leading to such a condition. More likely, most of the cases which have been reported represent preexisting asthma and/or expressions of an atopic predisposition. PMID:10619325

  6. Upper airway resistance syndrome.

    Science.gov (United States)

    Hasan, N; Fletcher, E C

    1998-07-01

    Many clinicians are familiar with the clinical symptoms and signs of obstructive sleep apnea (OSA). In its most blatant form, OSA is complete airway obstruction with repetitive, prolonged pauses in breathing, arterial oxyhemoglobin desaturation; followed by arousal with resumption of breathing. Daytime symptoms of this disorder include excessive daytime somnolence, intellectual dysfunction, and cardiovascular effects such as systemic hypertension, angina, myocardial infarction, and stroke. It has been recently recognized that increased pharyngeal resistance with incomplete obstruction can lead to a constellation of symptoms identical to OSA called "upper airway resistance syndrome" (UARS). The typical findings of UARS on sleep study are: (1) repetitive arousals from EEG sleep coinciding with a (2) waxing and waning of the respiratory airflow pattern and (3) increased respiratory effort as measured by esophageal pressure monitoring. There may be few, if any, obvious apneas or hypopneas with desaturation, but snoring may be a very prominent finding. Treatment with nasal positive airway pressure (NCPAP) eliminates the symptoms and confirms the diagnosis. Herein we describe two typical cases of UARS. PMID:9676067

  7. Small Airway Dysfunction and Abnormal Exercise Responses

    Science.gov (United States)

    Petsonk, Edward L.; Stansbury, Robert C.; Beeckman-Wagner, Lu-Ann; Long, Joshua L.; Wang, Mei Lin

    2016-01-01

    Rationale Coal mine dust exposure can cause symptoms and loss of lung function from multiple mechanisms, but the roles of each disease process are not fully understood. Objectives We investigated the implications of small airway dysfunction for exercise physiology among a group of workers exposed to coal mine dust. Methods Twenty coal miners performed spirometry, first breathing air and then helium-oxygen, single-breath diffusing capacity, and computerized chest tomography, and then completed cardiopulmonary exercise testing. Measurements and Main Results Six participants meeting criteria for small airway dysfunction were compared with 14 coal miners who did not. At submaximal workload, miners with small airway dysfunction used a higher proportion of their maximum voluntary ventilation and had higher ventilatory equivalents for both O2 and CO2. Regression modeling indicated that inefficient ventilation was significantly related to small airway dysfunction but not to FEV1 or diffusing capacity. At the end of exercise, miners with small airway dysfunction had 27% lower O2 consumption. Conclusions Small airway abnormalities may be associated with important inefficiency of exercise ventilation. In dust-exposed individuals with only mild abnormalities on resting lung function tests or chest radiographs, cardiopulmonary exercise testing may be important in defining causes of exercise intolerance. PMID:27073987

  8. MOEBIUS SYNDROME: CHALLENGES OF AIRWAY MANAGEMENT.

    Science.gov (United States)

    Budić, Ivana; Šurdilović, Dušan; Slavković, Anđelka; Marjanović, Vesna; Stević, Marija; Simić, Dušica

    2016-03-01

    Moebius syndrome is a rare nonprogressive congenital neurological disorder with a wide range of severity and variability of symptoms. This diversity is a consequence of dysfunction of different cranial nerves (most often facial and abducens nerves), accompanying orofacial abnormalities, musculoskeletal malformations, congenital cardiac diseases, as well as specific associations of Moebius and other syndromes. The authors present anesthesia and airway management during the multiple tooth extraction surgery in a 10-year-old girl with Moebius syndrome associated with Poland and trigeminal trophic syndromes. PMID:27276780

  9. Síndrome de disfunción reactiva de las vías aéreas Reactive airways dysfunction syndrome

    Directory of Open Access Journals (Sweden)

    R. Costa

    2005-01-01

    Full Text Available El síndrome de disfunción reactiva de las vías aéreas, más conocido como RADS (Reactive Airways Dysfunction Syndrome, fue descrito como una entidad clínica consistente en la aparición de asma bronquial a partir de una inhalación tóxica masiva. El término fue acuñado y reconocido por primera vez en el año 1985. Desde entonces se recogen diversas publicaciones donde se han constatado nuevos casos así como diferentes agentes causales. Suele constituir un accidente en el lugar de trabajo y en lugares cerrados o mal ventilados, donde se inhalan productos irritantes a altas concentraciones en forma de gases, humos o vapores. De manera aguda aparecen síntomas de obstrucción bronquial en los siguientes minutos u horas persistiendo hiperreactividad bronquial durante meses o años. A dosis no tóxicas los pacientes afectos no muestran recurrencia de síntomas tras la exposición al mismo agente que inició los síntomas, por lo que el diagnóstico se basa en criterios clínicos al no ser reproducible por una prueba de provocación.Reactive airways dysfunction syndrome, better known as RADS, was described as a clinical entity consisting in the appearance of bronchial asthma due to massive toxic inhalation. The term was coined and recognised for the first time in 1985. Since then different publications have verified new cases as well as different causal agents. It usually arises from an accident at the work place and in closed or poorly ventilated spaces, where high concentrations of irritant products are inhaled in the form of gas, smoke or vapour. In the following minutes or hours symptoms of bronchial obstruction appear in an acute form, with bronchial hyperresponsiveness persisting for months or years. The affected patients do not show a recurrence of symptoms following exposure to non-toxic doses of the same agent that started the symptoms. This is why diagnosis is based on clinical manifestations as it is not reproducible through a

  10. Airway statuses and nasopharyngeal airway use for airway obstruction in syndromic craniosynostosis.

    Science.gov (United States)

    Kouga, Takeshi; Tanoue, Koji; Matsui, Kiyoshi

    2014-05-01

    Syndromic craniosynostosis is associated with a high rate of respiratory difficulty, due mainly to midfacial hypoplasia. Nasopharyngeal airway establishment has been reported as the first-line approach to airway obstruction and may obviate the need for a highly invasive tracheotomy. No previous studies have compared airway obstruction status in syndromic craniosynostosis between cases requiring and not requiring airway managements. We focus on nasopharyngeal airway use and airway status outcomes to assess respiratory difficulty in patients with syndromic craniosynostosis. A retrospective data analysis of 51 cases with syndromic craniosynostosis was carried out. We divided 30 of the 51 cases with lateral pharyngeal x-rays taken before operations affecting airway diameters into 2 groups, one with neither nasopharyngeal airway insertion nor tracheotomy and the other with one or both of these interventions, and the mean diameters for 8 indices related to the pharyngeal space were compared. Cases with respiratory difficulty due to nasopharyngeal stenosis and requiring airway managements comprised a significantly higher proportion of those with Pfeiffer syndrome than patients with Crouzon or Apert syndrome. Comparative examination of lateral x-ray cephalometry between cases with neither nasopharyngeal airway insertion nor tracheotomy and cases with one or both revealed oropharyngeal diameters tended to be smaller in those with interventions. Cases requiring nasopharyngeal airway insertion were able to continue nasopharyngeal airway use for more than 1 year and a considerable number avoided tracheotomy. It may be worth considering an oropharyngeal-bypass nasopharyngeal airway before performing a tracheotomy. PMID:24820706

  11. 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

  12. Mitochondrial Dysfunction in Metabolic Syndrome and Asthma

    Directory of Open Access Journals (Sweden)

    Ulaganathan Mabalirajan

    2013-01-01

    Full Text Available 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.

  13. Clinical characteristics of adult asthma associated with small airway dysfunction.

    Science.gov (United States)

    Kjellberg, S; Houltz, B K; Zetterström, O; Robinson, P D; Gustafsson, Per M

    2016-08-01

    Suboptimal asthma control is common despite modern asthma therapy. The degree of peripheral airway involvement remains unclear and poor medication delivery to these regions might be a contributing reason for this failure in obtaining adequate symptom control. A cohort of 196 adults (median (range) age 44 (18-61) years, 109 females, 54 ex-smokers, six current smokers) with physician-diagnosed asthma were recruited from primary care. Subjects were characterized clinically by interviews, questionnaires, skin prick tests (SPT) and blood eosinophil counts. Lung function was assessed by spirometry, impulse oscillometry (IOS) and nitrogen multiple breath washout (N2 MBW). IOS assessed peripheral airway resistance (FDR, frequency dependence of resistance). N2 MBW assessed global ventilation inhomogeneity (LCI, lung clearance index), specific indices of peripheral airway function (Scond × VT and Sacin × VT; VT, tidal volume), and inter-regional inhomogeneity (specific ventilation ratio). Never-smoking healthy cohorts of 158 and 400 adult subjects provided local reference values for IOS and N2 MBW variables, respectively. Peripheral airway dysfunction was detected in 31% (FDR or specific ventilation ratio) to 47% (Scond x VT) of subjects. Risk factors for peripheral airway dysfunction were identified. Among subjects with low FEV1 and either positive smoking history and/or blood eosinophilia (>4.0%), 63% had abnormality across all peripheral airway outcomes, whilst only one subject was completely normal. Abnormal peripheral airway function was present in a large proportion of adult asthmatics at baseline. Reduced FEV1, a positive smoking history, and/or blood eosinophilia identified "a small airway asthma subtype" that might benefit from peripheral airway targeted therapy. PMID:27492518

  14. Olfactory dysfunction in Down's Syndrome.

    Science.gov (United States)

    Murphy, C; Jinich, S

    1996-01-01

    Down's Syndrome subjects over 40 years old show neuropathology similar to that of Alzheimer's disease. The olfactory system is particularly vulnerable in Alzheimer's disease, both anatomically and functionally. Several measures of sensory and cognitive functioning were studied in the older Down's Syndrome patient, with the hypothesis of significant olfactory dysfunction. Participants were 23 Down's subjects, and 23 controls. The Dementia Rating Scale showed mean scores of 103 for Down's subjects and 141 for controls. Down's subjects showed significant deficits in odor detection threshold, odor identification, and odor recognition memory. Normal performance in a taste threshold task, similar to the olfactory threshold task in subject demands, suggested that the Down's syndrome subjects' poor performance was not due to task demands. Deficits in olfaction may provide a sensitive and early indicator of the deterioration and progression of the brain in older subjects with Down's Syndrome.

  15. Myofascial Pain Dysfunction Syndrome (MPDS

    Directory of Open Access Journals (Sweden)

    Hamed Mortazavi

    2010-10-01

    Full Text Available Introduction: Myofascial Pain Dysfunction Syndrome (MPDS is one of the most important causes of the orofacial pain. The main purpose of this study was to evaluate 40 related variables in this regard. Materials and Methods: Thirty nine patients with MPDS were evaluated in this study. Different factors including age, gender, occupation, marital status, sensitivity of masticatory muscles, maximum opening of the mouth, deviation, deflection, involvement of temporomandibular joint, habit, parafunction, malocclusion, neck pain, headache, earache and history of jaw involvement, etc were analyzed in this  evaluation. Results: In our study, 39 patients (32 females and 7 males, 20-40 years old, with the average age of 35 ± 13.32 years were studied. 51% were housewives and 74.4% were married. The most common involvements were Clicking (74.4%, pain in temporomandibular joint (54%, headache (46.2%, earache (41%, neck-pain (35.9%, trouble in the mouth opening (71.8%, malocclusion Class I (74.4%, cross bite and deep bite (25%, clenching (64.1% and involvement of masseter and lateral pterygoid muscle (84%. Conclusion: Since MPDS consists of variable symptoms, it might be very difficult to provide any definite diagnosis and treatment. Therefore the more the specialists extend their knowledge and information about this disorder, the more they will make the best decision in this regard.

  16. CONGENITAL HIGH AIRWAY OBSTRUCTION (CHAOS SYNDROME: A RARE CASE PRESENTATION

    Directory of Open Access Journals (Sweden)

    Dinakara

    2014-04-01

    Full Text Available Congenital high airway obstruction syndrome (CHAOS results in a predictable constellation of findings: large echogenic lungs flattened or inverted diaphragms, dilated airways distal to the obstruction, and fetal ascites and/or hydrops.1 The finding of CHAOS on prenatal ultrasound examination is diagnostic of complete or near-complete obstruction of the fetal upper airway, most likely caused by laryngeal atresia. A greater understanding of the natural history of CHAOS may permit improved prenatal and perinatal management

  17. Dysfunctional lung anatomy and small airways degeneration in COPD

    Directory of Open Access Journals (Sweden)

    Burgel PR

    2013-01-01

    Full Text Available Clémence Martin, Justine Frija, Pierre-Régis BurgelDepartment of Respiratory Medicine, Cochin Hospital, AP-HP and Université Paris Descartes, Sorbonne Paris Cité, Paris, FranceAbstract: Chronic obstructive pulmonary disease (COPD is characterized by incompletely reversible airflow obstruction. Direct measurement of airways resistance using invasive techniques has revealed that the site of obstruction is located in the small conducting airways, ie, bronchioles with a diameter < 2 mm. Anatomical changes in these airways include structural abnormalities of the conducting airways (eg, peribronchiolar fibrosis, mucus plugging and loss of alveolar attachments due to emphysema, which result in destabilization of these airways related to reduced elastic recoil. The relative contribution of structural abnormalities in small conducting airways and emphysema has been a matter of much debate. The present article reviews anatomical changes and inflammatory mechanisms in small conducting airways and in the adjacent lung parenchyma, with a special focus on recent anatomical and imaging data suggesting that the initial event takes place in the small conducting airways and results in a dramatic reduction in the number of airways, together with a reduction in the cross-sectional area of remaining airways. Implications of these findings for the development of novel therapies are briefly discussed.Keywords: emphysema, small airways disease, airway mucus, innate immunity, adaptive immunity

  18. Airway Management in a Patient with Wolf-Hirschhorn Syndrome

    Science.gov (United States)

    Udani, Andrea G.

    2016-01-01

    We present a case of a 3-month-old female with Wolf-Hirschhorn syndrome (WHS) undergoing general anesthesia for laparoscopic gastrostomy tube placement with a focus on airway management. WHS is a rare 4p microdeletion syndrome resulting in multiple congenital abnormalities, including craniofacial deformities. Microcephaly, micrognathia, and glossoptosis are common features in WHS patients and risk factors for a pediatric airway that is potentially difficult to intubate. We discuss anesthesia strategies for airway preparation and management in a WHS patient requiring general anesthesia with endotracheal intubation. PMID:27752382

  19. Difficult airway in a patient with Marshall-Smith syndrome.

    Science.gov (United States)

    Antila, H; Laitio, T; Aantaa, R; Silvoniemi, P; Pakkanen, A

    1998-01-01

    Marshall-Smith syndrome is a rare clinical disorder characterized by accelerated bone maturation, dysmorphic facial features, airway abnormalities and death in early infancy because of respiratory complications. Although patients with Marshall-Smith syndrome have several features with potential anaesthetic problems, previous reports about anaesthetic management of these patients do not exist. We present a case, in which severe hypoxia developed rapidly after routine anaesthesia induction in an eight-month-old male infant with this syndrome. After several unsuccessful attempts the airway was finally secured by blind oral intubation. After 2 weeks, laryngeal anatomy was examined with fibreoptic laryngoscopy which revealed significant laryngomalacia. Laryngoscopy was performed without problems with ketamine anaesthesia and spontaneous breathing. The possibility of a compromised airway should always be borne in mind when anaesthetizing patients with Marshall-Smith syndrome. Anaesthesia maintaining spontaneous breathing is safest for children with this syndrome. If tracheal intubation or muscle relaxation is required, precautions are needed to maintain a patent airway. Muscle relaxants should possibly be avoided before intubation.

  20. Immune Dysfunction in Tourette Syndrome

    Directory of Open Access Journals (Sweden)

    Ishraga Elamin

    2013-01-01

    Full Text Available The association between immunity and neurodevelopmental disorders has been extensively investigated in autism, suggesting a potential involvement of both cellular and humoral immunity in the establishment of synaptic connectivity modulation during development. A similar link has been proposed also for Tourette syndrome (TS, a complex, multifactorial disorder, in which the interplay between genetic, environmental, hormonal and immunological factors might be relevant. Lymphocyte subpopulation analysis in TS suggests a possible systemic activation of several T- and B-cell subtypes, whereas the observed decreased numbers of T regulatory lymphocytes might predispose to autoimmunity. Genes related to both cell- and antibody-mediated immune responses may be over-expressed at specific ages in youngsters with TS. Data from cytokine measurements and transcriptomics profiles in TS patients are coherent with the systemic immune activation detected by studies on lymphocyte subpopulations. Moreover, TS patients have exhibited IgG3 and IgA dysgammaglobulinemia, which might predispose to recurrent infections and autoimmunity. To date, the association between TS and autoantibodies has not been demonstrated. Interestingly, however, there is a higher degree of maternal family history of autoimmune diseases among TS patients. Finally, TS patients could be prone to allergic illnesses (asthma, atopic dermatitis, rhinitis, conjunctivitis, but more work is needed in this area.

  1. Apraxia and motor dysfunction in corticobasal syndrome.

    Directory of Open Access Journals (Sweden)

    James R Burrell

    Full Text Available BACKGROUND: Corticobasal syndrome (CBS is characterized by multifaceted motor system dysfunction and cognitive disturbance; distinctive clinical features include limb apraxia and visuospatial dysfunction. Transcranial magnetic stimulation (TMS has been used to study motor system dysfunction in CBS, but the relationship of TMS parameters to clinical features has not been studied. The present study explored several hypotheses; firstly, that limb apraxia may be partly due to visuospatial impairment in CBS. Secondly, that motor system dysfunction can be demonstrated in CBS, using threshold-tracking TMS, and is linked to limb apraxia. Finally, that atrophy of the primary motor cortex, studied using voxel-based morphometry analysis (VBM, is associated with motor system dysfunction and limb apraxia in CBS. METHODS: Imitation of meaningful and meaningless hand gestures was graded to assess limb apraxia, while cognitive performance was assessed using the Addenbrooke's Cognitive Examination - Revised (ACE-R, with particular emphasis placed on the visuospatial subtask. Patients underwent TMS, to assess cortical function, and VBM. RESULTS: In total, 17 patients with CBS (7 male, 10 female; mean age 64.4+/- 6.6 years were studied and compared to 17 matched control subjects. Of the CBS patients, 23.5% had a relatively inexcitable motor cortex, with evidence of cortical dysfunction in the remaining 76.5% patients. Reduced resting motor threshold, and visuospatial performance, correlated with limb apraxia. Patients with a resting motor threshold <50% performed significantly worse on the visuospatial sub-task of the ACE-R than other CBS patients. Cortical function correlated with atrophy of the primary and pre-motor cortices, and the thalamus, while apraxia correlated with atrophy of the pre-motor and parietal cortices. CONCLUSIONS: Cortical dysfunction appears to underlie the core clinical features of CBS, and is associated with atrophy of the primary motor and

  2. Endothelin receptor antagonist and airway dysfunction in pulmonary arterial hypertension

    Directory of Open Access Journals (Sweden)

    Borst Mathias M

    2009-12-01

    Full Text Available Abstract Background In idiopathic pulmonary arterial hypertension (IPAH, peripheral airway obstruction is frequent. This is partially attributed to the mediator dysbalance, particularly an excess of endothelin-1 (ET-1, to increased pulmonary vascular and airway tonus and to local inflammation. Bosentan (ET-1 receptor antagonist improves pulmonary hemodynamics, exercise limitation, and disease severity in IPAH. We hypothesized that bosentan might affect airway obstruction. Methods In 32 IPAH-patients (19 female, WHO functional class II (n = 10, III (n = 22; (data presented as mean ± standard deviation pulmonary vascular resistance (11 ± 5 Wood units, lung function, 6 minute walk test (6-MWT; 364 ± 363.7 (range 179.0-627.0 m, systolic pulmonary artery pressure, sPAP, 79 ± 19 mmHg, and NT-proBNP serum levels (1427 ± 2162.7 (range 59.3-10342.0 ng/L were measured at baseline, after 3 and 12 months of oral bosentan (125 mg twice per day. Results and Discussion At baseline, maximal expiratory flow at 50 and 25% vital capacity were reduced to 65 ± 25 and 45 ± 24% predicted. Total lung capacity was 95.6 ± 12.5% predicted and residual volume was 109 ± 21.4% predicted. During 3 and 12 months of treatment, 6-MWT increased by 32 ± 19 and 53 ± 69 m, respectively; p Conclusion This study gives first evidence in IPAH, that during long-term bosentan, improvement of hemodynamics, functional parameters or serum biomarker occur independently from persisting peripheral airway obstruction.

  3. Contribution of air pollution to COPD and small airway dysfunction.

    Science.gov (United States)

    Berend, Norbert

    2016-02-01

    Although in many Western countries levels of ambient air pollution have been improving with the setting of upper limits and better urban planning, air pollution in developing countries and particularly those with rapid industrialization has become a major global problem. Together with increased motor vehicle ownership and traffic congestion, there is a growing issue with airborne particles of respirable size. These particles are thought responsible for respiratory and cardiovascular effects and have also been implicated in cancer pathogenesis. The pathologic effects in the lung are mediated via inflammatory pathways and involve oxidative stress similar to cigarette smoking. These effects are seen in the peripheral airways where the smaller particle fractions are deposited and lead to airway remodelling. However, emphysema and loss of bronchioles seen with cigarette smoking have not been described with ambient air pollution, and there are few studies specifically looking at peripheral airway function. Definitive evidence of air pollution causing COPD is lacking and a different study design is required to link air pollution and COPD. PMID:26412571

  4. Contribution of air pollution to COPD and small airway dysfunction.

    Science.gov (United States)

    Berend, Norbert

    2016-02-01

    Although in many Western countries levels of ambient air pollution have been improving with the setting of upper limits and better urban planning, air pollution in developing countries and particularly those with rapid industrialization has become a major global problem. Together with increased motor vehicle ownership and traffic congestion, there is a growing issue with airborne particles of respirable size. These particles are thought responsible for respiratory and cardiovascular effects and have also been implicated in cancer pathogenesis. The pathologic effects in the lung are mediated via inflammatory pathways and involve oxidative stress similar to cigarette smoking. These effects are seen in the peripheral airways where the smaller particle fractions are deposited and lead to airway remodelling. However, emphysema and loss of bronchioles seen with cigarette smoking have not been described with ambient air pollution, and there are few studies specifically looking at peripheral airway function. Definitive evidence of air pollution causing COPD is lacking and a different study design is required to link air pollution and COPD.

  5. Airway management in Escobar syndrome: A formidable challenge

    Directory of Open Access Journals (Sweden)

    Shaji Mathew

    2013-01-01

    Full Text Available Escobar syndrome is a rare autosomal recessive disorder characterized by flexion joint and digit contractures, skin webbing, cleft palate, deformity of spine and cervical spine fusion. Associated difficult airway is mainly due to micrognathia, retrognathia, webbing of neck and limitation of the mouth opening and neck extension. We report a case of a 1 year old child with Escobar syndrome posted for bilateral hamstrings to quadriceps transfer. The child had adequate mouth opening with no evidence of cervical spine fusion, yet we faced difficulty in intubation which was ultimately overcome by securing a proseal laryngeal mask airway (PLMA and then by intubating with an endotracheal tube railroaded over a paediatric fibreoptic bronchoscope passed through the lumen of a PLMA.

  6. Understanding Muscle Dysfunction in Chronic Fatigue Syndrome

    Directory of Open Access Journals (Sweden)

    Gina Rutherford

    2016-01-01

    Full Text Available Introduction. Chronic fatigue syndrome/myalgic encephalomyelitis (CFS/ME is a debilitating disorder of unknown aetiology, characterised by severe disabling fatigue in the absence of alternative diagnosis. Historically, there has been a tendency to draw psychological explanations for the origin of fatigue; however, this model is at odds with findings that fatigue and accompanying symptoms may be explained by central and peripheral pathophysiological mechanisms, including effects of the immune, oxidative, mitochondrial, and neuronal pathways. For example, patient descriptions of their fatigue regularly cite difficulty in maintaining muscle activity due to perceived lack of energy. This narrative review examined the literature for evidence of biochemical dysfunction in CFS/ME at the skeletal muscle level. Methods. Literature was examined following searches of PUB MED, MEDLINE, and Google Scholar, using key words such as CFS/ME, immune, autoimmune, mitochondria, muscle, and acidosis. Results. Studies show evidence for skeletal muscle biochemical abnormality in CFS/ME patients, particularly in relation to bioenergetic dysfunction. Discussion. Bioenergetic muscle dysfunction is evident in CFS/ME, with a tendency towards an overutilisation of the lactate dehydrogenase pathway following low-level exercise, in addition to slowed acid clearance after exercise. Potentially, these abnormalities may lead to the perception of severe fatigue in CFS/ME.

  7. Understanding Muscle Dysfunction in Chronic Fatigue Syndrome.

    Science.gov (United States)

    Rutherford, Gina; Manning, Philip; Newton, Julia L

    2016-01-01

    Introduction. Chronic fatigue syndrome/myalgic encephalomyelitis (CFS/ME) is a debilitating disorder of unknown aetiology, characterised by severe disabling fatigue in the absence of alternative diagnosis. Historically, there has been a tendency to draw psychological explanations for the origin of fatigue; however, this model is at odds with findings that fatigue and accompanying symptoms may be explained by central and peripheral pathophysiological mechanisms, including effects of the immune, oxidative, mitochondrial, and neuronal pathways. For example, patient descriptions of their fatigue regularly cite difficulty in maintaining muscle activity due to perceived lack of energy. This narrative review examined the literature for evidence of biochemical dysfunction in CFS/ME at the skeletal muscle level. Methods. Literature was examined following searches of PUB MED, MEDLINE, and Google Scholar, using key words such as CFS/ME, immune, autoimmune, mitochondria, muscle, and acidosis. Results. Studies show evidence for skeletal muscle biochemical abnormality in CFS/ME patients, particularly in relation to bioenergetic dysfunction. Discussion. Bioenergetic muscle dysfunction is evident in CFS/ME, with a tendency towards an overutilisation of the lactate dehydrogenase pathway following low-level exercise, in addition to slowed acid clearance after exercise. Potentially, these abnormalities may lead to the perception of severe fatigue in CFS/ME.

  8. Long-term clearance from small airways in subjects with ciliary dysfunction

    Directory of Open Access Journals (Sweden)

    Hjelte Lena

    2006-05-01

    Full Text Available Abstract The objective of this study was to investigate if long-term clearance from small airways is dependent on normal ciliary function. Six young adults with primary ciliary dyskinesia (PCD inhaled 111 Indium labelled Teflon particles of 4.2 μm geometric and 6.2 μm aerodynamic diameter with an extremely slow inhalation flow, 0.05 L/s. The inhalation method deposits particles mainly in the small conducting airways. Lung retention was measured immediately after inhalation and at four occasions up to 21 days after inhalation. Results were compared with data from ten healthy controls. For additional comparison three of the PCD subjects also inhaled the test particles with normal inhalation flow, 0.5 L/s, providing a more central deposition. The lung retention at 24 h in % of lung deposition (Ret24 was higher (p 24 with slow inhalation flow was 73.9 ± 1.9 % compared to 68.9 ± 7.5 % with normal inhalation flow in the three PCD subjects exposed twice. During day 7–21 the three PCD subjects exposed twice cleared 9 % with normal flow, probably representing predominantly alveolar clearance, compared to 19 % with slow inhalation flow, probably representing mainly small airway clearance. This study shows that despite ciliary dysfunction, clearance continues in the small airways beyond 24 h. There are apparently additional clearance mechanisms present in the small airways.

  9. 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.

  10. Noninvasive Investigations for the Early Detection of Chronic Airways Dysfunction Following Lung Transplantation

    OpenAIRE

    Cook, Richard C.; Guy Fradet; Nestor L Muller; Daniel F Worsley; David Ostrow; Levy, Robert D

    2003-01-01

    BACKGROUND: The diagnosis of chronic rejection after lung transplantation is limited by the lack of a reliable test to detect airways disease early.OBJECTIVES: To determine whether maximum midexpiratory flow (MMEF), or changes on high resolution computed tomography (HRCT) or ventilation/perfusion lung (V/Q) scans are sensitive and specific for early detection of bronchiolitis obliterans syndrome (BOS; forced expiratory volume in 1 s [FEV1] less than 80% post-transplant baseline) by evaluating...

  11. Postmortem magnetic resonance appearances of congenital high airway obstruction syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Arthurs, Owen J. [Great Ormond Street Hospital for Children NHS Foundation Trust, Department of Radiology, London (United Kingdom); UCL Institute of Child Health, London (United Kingdom); Chitty, Lyn S. [UCL Institute of Child Health, Genetics and Genomic Medicine, London (United Kingdom); Great Ormond Street and UCLH NHS Foundation Trusts, London (United Kingdom); Judge-Kronis, Lydia [Great Ormond Street Hospital for Children NHS Foundation Trust, Department of Histopathology, London (United Kingdom); Sebire, Neil J. [UCL Institute of Child Health, London (United Kingdom); Great Ormond Street Hospital for Children NHS Foundation Trust, Department of Histopathology, London (United Kingdom)

    2015-04-01

    Congenital high airway obstruction syndrome (CHAOS) is a rare life-threatening condition characterised by complete or near-complete developmental obstruction of the foetal airway. Although antenatal imaging findings have been described, the postmortem MRI findings have not been reported. To present postmortem MRI features of CHAOS. We retrospectively reviewed our hospital pathology and imaging databases for cases of CHAOS over a 2-year period. We identified two cases of CHAOS. In both cases, postmortem plain radiographs demonstrated gross abdominal distension with distortion and splaying of the rib cage. Both foetuses had characteristic postmortem MRI findings including large-volume fluid-filled lungs on T2-weighted imaging, diaphragmatic eversion, fluid-filled airway dilatation below the level of obstruction, centrally positioned and compressed heart, and massive ascites. One foetus had an associated limb abnormality. Postmortem MRI in foetuses suspected of having CHAOS allows confirmation of the diagnosis, determination of the anatomical level of the atresia or stenosis, and identification of associated abnormalities without the need for invasive autopsy. (orig.)

  12. Adipose Tissue Dysfunction in Nascent Metabolic Syndrome

    Directory of Open Access Journals (Sweden)

    Andrew A. Bremer

    2013-01-01

    Full Text Available The metabolic syndrome (MetS confers an increased risk for both type 2 diabetes mellitus (T2DM and cardiovascular disease (CVD. Moreover, studies on adipose tissue biology in nascent MetS uncomplicated by T2DM and/or CVD are scanty. Recently, we demonstrated that adipose tissue dysregulation and aberrant adipokine secretion contribute towards the syndrome’s low-grade chronic proinflammatory state and insulin resistance. Specifically, we have made the novel observation that subcutaneous adipose tissue (SAT in subjects with nascent MetS has increased macrophage recruitment with cardinal crown-like structures. We have also shown that subjects with nascent MetS have increased the levels of SAT-secreted adipokines (IL-1, IL-6, IL-8, leptin, RBP-4, CRP, SAA, PAI-1, MCP-1, and chemerin and plasma adipokines (IL-1, IL-6, leptin, RBP-4, CRP, SAA, and chemerin, as well as decreased levels of plasma adiponectin and both plasma and SAT omentin-1. The majority of these abnormalities persisted following correction for increased adiposity. Our data, as well as data from other investigators, thus, highlight the importance of subcutaneous adipose tissue dysfunction in subjects with MetS and its contribution to the proinflammatory state and insulin resistance. This adipokine profile may contribute to increased insulin resistance and low-grade inflammation, promoting the increased risk of T2DM and CVD.

  13. Salivary Gland Dysfunction and Xerostomia in Sjogren's Syndrome

    NARCIS (Netherlands)

    Jensen, Siri Beier; Vissink, Arjan

    2014-01-01

    In this article, salivary gland dysfunction and xerostomia in Sjogren's syndrome (SS) are discussed, with a focus on the pathophysiology of salivary dysfunction in SS, the clinical presentation of dry mouth in SS, how to assess salivary gland hypofunction and xerostomia in SS, and the impact of sali

  14. Relationship between metabolic syndrome and erectile dysfunction

    Institute of Scientific and Technical Information of China (English)

    M.I.Gǘiundǘz; B.H.Gǘmǘs; C.Sekuri

    2004-01-01

    Aim: To determine the relationship between metabolic syndrome (MS) and erectile dysfunction (ED) and to see which risk factors correlated the best with ED. Methods: Seventy-nine cardiology clinic outpatients with coronary artery disease (CAD) and lipid metabolism disorder were recruited. They were categorized as having MS,hypertension (blood pressure greater than 130/85 mmHg) and dyslipidemia. ED was classified based on International Index of Erectile Function scores. Patients were grouped into quartiles based on body mass index (BMI). Chi-square,Pearson's correlation and regression tests were used for statistical analysis. Results: The mean age of the patients was 56.6 years. ED was diagnosed in 59 (74.7 %) of the 79 patients. In the 38 patients with MS, all had ED. ED was not significantly correlated with cholesterol levels (P>0.05), but was found often in patients who had both hypercholesterolemia and HT (P<0.01). Nineteen(76 %) of the 25 patients who had dyslipidemia had ED. However, ED wasnot significantly correlated with dyslipidemia (P>0.05). Tweenty-two of the 23 patients who had BMI greater than30 had ED, which was significantly more prevalent than that in those who had normal BMI (P<0.01). ED was seen in 38 of 53 smoker patients. Although ED was more prevalent in cigarette smokers, it was not significantly different from non-smokers (P>0.5). Conclusion: ED is present in a high percentage of patients with MS. Among multiple risk factors for ED, MS correlates the most highly. The next most important risk group is the patients with hypertension +hypercholestrolemia and obesity (BMI>30). (Asian JAndrol 2004 Dec; 6: 355-358)

  15. Upper airway obstruction in a patient with Ehlers-Danlos syndrome.

    Science.gov (United States)

    Chatzoudis, D; Kelly, T J; Lancaster, J; Jones, T M

    2015-04-01

    We report a case of recurrent airway obstruction episodes resulting from laryngeal hypermobility in a patient with Ehlers-Danlos syndrome. A 44-year-old woman, with known Ehlers-Danlos syndrome, presented with recent onset of episodes of upper airway obstruction due to hypermobility of her larynx. A suitable conservative management strategy proved elusive and the patient finally underwent a thyrohyoidopexy. The patient remains symptom free nine months after the procedure. This is the first report of spontaneous life threatening upper airway obstruction due to hypermobility of the suprahyoid suspensory soft tissues in Ehlers-Danlos syndrome. PMID:26263828

  16. Upper Airway Elasticity Estimation in Pediatric Down Syndrome Sleep Apnea Patients Using Collapsible Tube Theory.

    Science.gov (United States)

    Subramaniam, Dhananjay Radhakrishnan; Mylavarapu, Goutham; McConnell, Keith; Fleck, Robert J; Shott, Sally R; Amin, Raouf S; Gutmark, Ephraim J

    2016-05-01

    Elasticity of the soft tissues surrounding the upper airway lumen is one of the important factors contributing to upper airway disorders such as snoring and obstructive sleep apnea. The objective of this study is to calculate patient specific elasticity of the pharynx from magnetic resonance (MR) images using a 'tube law', i.e., the relationship between airway cross-sectional area and transmural pressure difference. MR imaging was performed under anesthesia in children with Down syndrome (DS) and obstructive sleep apnea (OSA). An airway segmentation algorithm was employed to evaluate changes in airway cross-sectional area dilated by continuous positive airway pressure (CPAP). A pressure-area relation was used to make localized estimates of airway wall stiffness for each patient. Optimized values of patient specific Young's modulus for tissue in the velopharynx and oropharynx, were estimated from finite element simulations of airway collapse. Patient specific deformation of the airway wall under CPAP was found to exhibit either a non-linear 'hardening' or 'softening' behavior. The localized airway and tissue elasticity were found to increase with increasing severity of OSA. Elasticity based patient phenotyping can potentially assist clinicians in decision making on CPAP and airway or tissue elasticity can supplement well-known clinical measures of OSA severity.

  17. Upper airway obstruction in a patient with Ehlers–Danlos syndrome

    OpenAIRE

    Chatzoudis, D; Kelly, TJ; Lancaster, J.; Jones, TM

    2015-01-01

    We report a case of recurrent airway obstruction episodes resulting from laryngeal hypermobility in a patient with Ehlers–Danlos syndrome. A 44-year-old woman, with known Ehlers–Danlos syndrome, presented with recent onset of episodes of upper airway obstruction due to hypermobility of her larynx. A suitable conservative management strategy proved elusive and the patient finally underwent a thyrohyoidopexy. The patient remains symptom free nine months after the procedure. This is the first re...

  18. "DIFFICULT AIRWAY MANAGEMENT IN A PATIENT WITH TREACHER-COLLIN’S SYNDROME WITH INTUBATING LARYNGEAL MASK AIRWAY "

    Directory of Open Access Journals (Sweden)

    M. Gharebaghian

    2006-08-01

    Full Text Available Treacher Collin’s syndrome (TCS is a rare inherited condition characterized by bilateral and symmetric abnormalities of structures within the first and second bronchial arches. Patients with TCS present a serious problem to anesthetists maintaining their airway as upper airway obstruction and difficult tracheal intubation due to severe facial deformity. Because of retrognathia, airway management of these patients is often challenging. We report the case of a 25-yr-old patient with TCS undergoing microtia repair under general anesthesia twice. In the first time he could not be intubated via direct laryngoscopy and was intubated via blind nasal intubation. In the second time, he was intubated through an ILMA using endotracheal tube.

  19. Risk factors for multiple organic dysfunctions syndrome in burnt children.

    Directory of Open Access Journals (Sweden)

    Elvira Maria Martinez Barreto

    2009-05-01

    Full Text Available Background: The creation of intensive care units allows extending the life of patients with serious conditions, including multiple organic dysfunction syndromes. Objective: To determine the clinical variables and laboratory variables that are risk factors for multiple organic dysfunction syndromes in burnt children. Methods: Analytical, retrospective study of case series including burnt patients between 0 and 5 years hospitalized in the university Paediatric Hospital “Paquito González” in Cienfuegos and classified as: serious, very serious, critical, and extremely critical. This study was developed from January 1st, 2000 to December 31st, 2005. The considered systems for dysfunction diagnosis were: respiratory, cardiovascular, gastrointestinal, hepatic, renal, metabolic, central nervous system, hematologic, immunologic, and wounds healing. Data was processed by bivariate analysis of independent variables in relation with the dependent variable, to model a response variable of the syndrome occurrence (or not. The multivariate analysis of logistic regression was used. Results: 34 children developed the syndrome 44, 2 %. Significant variables linked to this syndrome were: seriousness of the injuries, serum potassium, blood creatinine, leukocyte counting, and cardiac rhythm. Conclusions: After five days of research development, a group of factors was identified proving risky for the development of multiple organic dysfunctions in burnt children.

  20. Compliance Measurements of the Upper Airway in Pediatric Down Syndrome Sleep Apnea Patients.

    Science.gov (United States)

    Subramaniam, Dhananjay Radhakrishnan; Mylavarapu, Goutham; McConnell, Keith; Fleck, Robert J; Shott, Sally R; Amin, Raouf S; Gutmark, Ephraim J

    2016-04-01

    Compliance of soft tissue and muscle supporting the upper airway are two of several factors contributing to pharyngeal airway collapse. We present a novel, minimally invasive method of estimating regional variations in pharyngeal elasticity. Magnetic resonance images for pediatric sleep apnea patients with Down syndrome [9.5 ± 4.3 years (mean age ± standard deviation)] were analyzed to segment airways corresponding to baseline (no mask pressure) and two positive pressures. A three dimensional map was created to evaluate axial and circumferential variation in radial displacements of the airway, dilated by the positive pressures. The displacements were then normalized with respect to the appropriate transmural pressure and radius of an equivalent circle to obtain a measure of airway compliance. The resulting elasticity maps indicated the least and most compliant regions of the pharynx. Airway stiffness of the most compliant region [403 ± 204 (mean ± standard deviation) Pa] decreased with severity of obstructive sleep apnea. The non-linear response of the airway wall to continuous positive airway pressure was patient specific and varied between anatomical locations. We identified two distinct elasticity phenotypes. Patient phenotyping based on airway elasticity can potentially assist clinical practitioners in decision making on the treatments needed to improve airway patency.

  1. Small airway dysfunction and flow and volume bronchodilator responsiveness in patients with chronic obstructive pulmonary disease

    Directory of Open Access Journals (Sweden)

    Pisi R

    2015-06-01

    Full Text Available Roberta Pisi,1 Marina Aiello,1 Andrea Zanini,2 Panagiota Tzani,1 Davide Paleari,3 Emilio Marangio,1 Antonio Spanevello,2,4 Gabriele Nicolini,5 Alfredo Chetta1 1Department of Clinical and Experimental Medicine, University of Parma, Parma, 2Division of Pneumology, IRCCS Rehabilitation Institute of Tradate, Salvatore Maugeri Foundation, Tradate, 3Medical Department, Chiesi Farmaceutici SpA, Parma, 4Department of Clinical and Experimental Medicine, University of Insubria, Varese, 5Corporate Clinical Development, Chiesi Farmaceutici SpA, Parma, Italy Background: We investigated whether a relationship between small airways dysfunction and bronchodilator responsiveness exists in patients with chronic obstructive pulmonary disease (COPD.Methods: We studied 100 (20 female; mean age: 68±10 years patients with COPD (forced expiratory volume in 1 second [FEV1]: 55% pred ±21%; FEV1/forced vital capacity [FVC]: 53%±10% by impulse oscillometry system. Resistance at 5 Hz and 20 Hz (R5 and R20, in kPa·s·L-1 and the fall in resistance from 5 Hz to 20 Hz (R5 – R20 were used as indices of total, proximal, and peripheral airway resistance; reactance at 5 Hz (X5, in kPa·s·L-1 was also measured. Significant response to bronchodilator (salbutamol 400 µg was expressed as absolute (≥0.2 L and percentage (≥12% change relative to the prebronchodilator value of FEV1 (flow responders, FRs and FVC (volume responders, VRs.Results: Eighty out of 100 participants had R5 – R20 >0.03 kPa·s·L-1 (> upper normal limit and, compared to patients with R5 – R20 ≤0.030 kPa·s·L-1, showed a poorer health status, lower values of FEV1, FVC, FEV1/FVC, and X5, along with higher values of residual volume/total lung capacity and R5 (P<0.05 for all comparisons. Compared to the 69 nonresponders and the 8 FRs, the 16 VRs had significantly higher R5 and R5 – R20 values (P<0.05, lower X5 values (P<0.05, and greater airflow obstruction and lung

  2. Multiple organ dysfunction syndrome associated with Mycoplasma pneumoniae infection

    Directory of Open Access Journals (Sweden)

    Shu-Bo Zhai

    2012-03-01

    Full Text Available In this study, we report one case of a three-year-old boy infected with Mycoplasma pneumonia (MP and presenting concomitant multiple organ damage of the heart, kidney, lung and liver, among others, together with a brief review for the diagnosis and treatment of MP infection with multiple organ dysfunction syndrome (MODS.

  3. Role of Oxidative Stress in the Neurocognitive Dysfunction of Obstructive Sleep Apnea Syndrome

    Science.gov (United States)

    Chen, Ping

    2016-01-01

    Obstructive sleep apnea syndrome (OSAS) is characterized by chronic nocturnal intermittent hypoxia and sleep fragmentations. Neurocognitive dysfunction, a significant and extraordinary complication of OSAS, influences patients' career, family, and social life and reduces quality of life to some extent. Previous researches revealed that repetitive hypoxia and reoxygenation caused mitochondria and endoplasmic reticulum dysfunction, overactivated NADPH oxidase, xanthine oxidase, and uncoupling nitric oxide synthase, induced an imbalance between prooxidants and antioxidants, and then got rise to a series of oxidative stress (OS) responses, such as protein oxidation, lipid peroxidation, and DNA oxidation along with inflammatory reaction. OS in brain could trigger neuron injury especially in the hippocampus and cerebral cortex regions. Those two regions are fairly susceptible to hypoxia and oxidative stress production which could consequently result in cognitive dysfunction. Apart from continuous positive airway pressure (CPAP), antioxidant may be a promising therapeutic method to improve partially reversible neurocognitive function. Understanding the role that OS played in the cognitive deficits is crucial for future research and therapeutic strategy development. In this paper, recent important literature concerning the relationship between oxidative stress and cognitive impairment in OSAS will be summarized and the results can provide a rewarding overview for future breakthrough in this field.

  4. Epigenetic Dysfunction in Turner Syndrome Immune Cells.

    Science.gov (United States)

    Thrasher, Bradly J; Hong, Lee Kyung; Whitmire, Jason K; Su, Maureen A

    2016-05-01

    Turner syndrome (TS) is a chromosomal condition associated with partial or complete absence of the X chromosome that involves characteristic findings in multiple organ systems. In addition to well-known clinical characteristics such as short stature and gonadal failure, TS is also associated with T cell immune alterations and chronic otitis media, suggestive of a possible immune deficiency. Recently, ubiquitously transcribed tetratricopeptide repeat on the X chromosome (UTX), a histone H3 lysine 27 (H3K27) demethylase, has been identified as a downregulated gene in TS immune cells. Importantly, UTX is an X-linked gene that escapes X-chromosome inactivation and thus is haploinsufficient in TS. Mice with T cell-specific UTX deficiency have impaired clearance of chronic viral infection due to decreased frequencies of T follicular helper (Tfh) cells, which are critical for B cell antibody generation. In parallel, TS patients have decreased Tfh frequencies in peripheral blood. Together, these findings suggest that haploinsufficiency of the X-linked UTX gene in TS T cells underlies an immune deficit, which may manifest as increased predisposition to chronic otitis media.

  5. Epigenetic Dysfunction in Turner Syndrome Immune Cells.

    Science.gov (United States)

    Thrasher, Bradly J; Hong, Lee Kyung; Whitmire, Jason K; Su, Maureen A

    2016-05-01

    Turner syndrome (TS) is a chromosomal condition associated with partial or complete absence of the X chromosome that involves characteristic findings in multiple organ systems. In addition to well-known clinical characteristics such as short stature and gonadal failure, TS is also associated with T cell immune alterations and chronic otitis media, suggestive of a possible immune deficiency. Recently, ubiquitously transcribed tetratricopeptide repeat on the X chromosome (UTX), a histone H3 lysine 27 (H3K27) demethylase, has been identified as a downregulated gene in TS immune cells. Importantly, UTX is an X-linked gene that escapes X-chromosome inactivation and thus is haploinsufficient in TS. Mice with T cell-specific UTX deficiency have impaired clearance of chronic viral infection due to decreased frequencies of T follicular helper (Tfh) cells, which are critical for B cell antibody generation. In parallel, TS patients have decreased Tfh frequencies in peripheral blood. Together, these findings suggest that haploinsufficiency of the X-linked UTX gene in TS T cells underlies an immune deficit, which may manifest as increased predisposition to chronic otitis media. PMID:27039394

  6. Pure Obstructive Sleep Apnea Syndrome and Erectile Dysfunction

    Directory of Open Access Journals (Sweden)

    Cenk Gürbüz

    2011-11-01

    Full Text Available Objective: The aim of this study is to investigate the existence of erectile dysfunction in patients with obstructive sleep apnea syndrome (OSAS in which the other possible causes of erectile dysfunction were eliminated.Material and Methods: The study group consisted of 24 patients diagnosed as OSAS with polysomnographic evaluation, and 15 non-apneic controls (mean age; 41.0±8.8 and 42.3±7.9 year respectively whose comorbidities which might be associated with erectile dysfunction were excluded. Daytime sleepiness was evaluated by Epworth Sleepiness Scale (ESS and measurement of erectile function was performed by International Index of Erectile Function.Results: The rate of erectile dysfunction in OSAS and control groups were 54.2% and 33.3% respectively (p=0.204. The difference between mean erectile function scores of patient and control groups was non-significant (26.1±4.5 and 26.3±4.3 respectively, p=0.900. There was no correlation between erectile function scores and apnea hypnoea index (r=-0.140; p=0.395.Conclusion: Findings obtained from this study suggest that the high incidence of erectile dysfunction reported in OSAS patients seems to be related with concomitant comorbidities such as diabetes, atherosclerosis and neuroendocrine disorders rather than sleep apnea.

  7. Möbius Syndrome: Surgical Treatment for Eyelid Dysfunction

    Directory of Open Access Journals (Sweden)

    Gloria Lopez-Valverde

    2013-11-01

    Full Text Available Introduction: Möbius syndrome is a heterogeneous congenital disorder that is linked to bilateral palsies of the cranial nerves VI and VII, resulting in congenital facial paralysis sometimes associated with impaired ocular abduction. Case Report: We present the case of a 44-year-old woman with Möbius syndrome and inferior recurrent keratitis secondary to scleral show in both eyes. We decided to use a cartilage graft from the ear in the inferior eyelid to avoid eyelid retraction and scleral show. Discussion: Patients with Möbius syndrome have a severe dysfunction of their facial mimic. Their treatment must be individualized, depending on their age, clinical examination and symptoms.

  8. Treatment of upper airway resistance syndrome in adults: Where do we stand?☆

    Science.gov (United States)

    de Godoy, Luciana B.M.; Palombini, Luciana O.; Guilleminault, Christian; Poyares, Dalva; Tufik, Sergio; Togeiro, Sonia M.

    2015-01-01

    Objective: To evaluate the available literature regarding Upper Airway Resistance Syndrome (UARS) treatment. Methods: Keywords “Upper Airway Resistance Syndrome,” “Sleep-related Breathing Disorder treatment,” “Obstructive Sleep Apnea treatment” and “flow limitation and sleep” were used in main databases. Results: We found 27 articles describing UARS treatment. Nasal continuous positive airway pressure (CPAP) has been the mainstay therapy prescribed but with limited effectiveness. Studies about surgical treatments had methodological limitations. Oral appliances seem to be effective but their efficacy is not yet established. Conclusion: Randomized controlled trials with larger numbers of patients and long-term follow-up are important to establish UARS treatment options. PMID:26483942

  9. Small-airways dysfunction associates with respiratory symptoms and clinical features of asthma : A systematic review

    NARCIS (Netherlands)

    van der Wiel, Erica; ten Hacken, Nick H. T.; Postma, Dirkje S.; van den Berge, Maarten

    2013-01-01

    Traditionally, asthma has been considered a disease that predominantly involves the large airways. Today, this concept is being challenged, and increasing evidence has become available showing that abnormalities in the small airways also contribute to the clinical expression of asthma. The small air

  10. Crouzon syndrome with bony upper airway obstruction: case report and review literature.

    Science.gov (United States)

    Gothwal, Sunil; Nayan, Swati; Kumar, Jagdish

    2014-08-01

    Crouzon syndrome is a rare genetic disorder. It is inherited as an autosomal dominant pattern. Mutations in the FGFR2 gene are known to cause Crouzon syndrome. Craniofacial abnormalities are common at birth and may progress with time. The severity of these signs and symptoms varies among affected children. A full term, large for date, male baby was delivered to a gravida 2 mother by cesarean section having facial dimorphism suggestive of Crouzon syndrome. Genetic team confirmed the diagnosis. Baby had severe respiratory distress. On work up, upper bony airway narrowing was found (diameter 3 mm). Later on, baby was operated for the same. Baby is asymptomatic now and doing well up to 2 months of follow-up. Management of Crouzon disease is multidisciplinary and early diagnosis has prime importance. Follow-up must ensure late features like hearing problems, vision problems, dental problems, intelligence, cranial synostosis, and upper airway problems. PMID:24828762

  11. MR imaging appearance of laryngeal atresia (congenital high airway obstruction syndrome): unique course in a fetus

    Energy Technology Data Exchange (ETDEWEB)

    Kuwashima, Shigeko; Kitajima, Kazuhiro; Kaji, Yasushi [Dokkyo Medical University, Department of Radiology, Mibu, Shimotsuga-gun, Tochigi (Japan); Watanabe, Hiroshi [Dokkyo Medical University, Department of Obstetrics and Gynecology, Mibu (Japan); Watabe, Yoshiyuki; Suzumura, Hiroshi [Dokkyo Medical University, Department of Pediatrics, Mibu (Japan)

    2008-03-15

    Congenital high airway obstruction syndrome (CHAOS) is a rare life-threatening syndrome. Most cases are diagnosed prenatally by US. We report a fetus with this syndrome that showed a unique course revealed on MRI. Ultrasonography at 22 weeks demonstrated that the fetus had ascites and bilaterally enlarged hyperechoic lungs. Congenital infection, congenital cystic adenomatoid malformation or CHAOS was suspected. Subsequent MRI performed at 24 weeks demonstrated bilaterally enlarged high-signal lungs, dilated bronchi, massive ascites, subcutaneous oedema and polyhydramnios. MRI confirmed the diagnosis of CHAOS. A second MRI at 35 weeks showed that the bilateral lung enlargement, ascites, oedema and polyhydramnios had resolved, but that the appearance of the airway was unchanged. The infant was delivered by caesarean section at 38 weeks of gestation and immediate tracheostomy was performed. This spontaneous regression was explained by a tracheo-oesophageal fistula that may have decreased the intrathoracic pressure. (orig.)

  12. MR imaging appearance of laryngeal atresia (congenital high airway obstruction syndrome): unique course in a fetus

    International Nuclear Information System (INIS)

    Congenital high airway obstruction syndrome (CHAOS) is a rare life-threatening syndrome. Most cases are diagnosed prenatally by US. We report a fetus with this syndrome that showed a unique course revealed on MRI. Ultrasonography at 22 weeks demonstrated that the fetus had ascites and bilaterally enlarged hyperechoic lungs. Congenital infection, congenital cystic adenomatoid malformation or CHAOS was suspected. Subsequent MRI performed at 24 weeks demonstrated bilaterally enlarged high-signal lungs, dilated bronchi, massive ascites, subcutaneous oedema and polyhydramnios. MRI confirmed the diagnosis of CHAOS. A second MRI at 35 weeks showed that the bilateral lung enlargement, ascites, oedema and polyhydramnios had resolved, but that the appearance of the airway was unchanged. The infant was delivered by caesarean section at 38 weeks of gestation and immediate tracheostomy was performed. This spontaneous regression was explained by a tracheo-oesophageal fistula that may have decreased the intrathoracic pressure. (orig.)

  13. Development of sorbent therapy for multiple organ dysfunction syndrome (MODS)

    International Nuclear Information System (INIS)

    As a syndrome, multiple organ dysfunction (MODS) is defined as an altered organ function in the setting of sepsis, septic shock or systemic inflammatory response syndrome (SIRS) and is the most common cause of death in intensive care units. Endotoxin, a constituent of cell walls of Gram-negative bacteria, plays an important role in the initiation and development of MODS. The cytokines, especially tumor necrosis factor alpha (TNF-alpha), are early regulators of the immune response and can induce the release of secondary cytokines. To remove endotoxin and TNF-alpha from patients with MODS, the adsorption method has proven to be most effective. In this review, we provide various methods of removal of endotoxins and TNF-alpha using different adsorbents. (topical review)

  14. Development of sorbent therapy for multiple organ dysfunction syndrome (MODS)

    Energy Technology Data Exchange (ETDEWEB)

    Li Li; Pan Jilun; Yu Yaoting [Key Laboratory of Bioactive Materials, Ministry of Education, Nankai University, Tianjin 300071 (China)

    2007-06-01

    As a syndrome, multiple organ dysfunction (MODS) is defined as an altered organ function in the setting of sepsis, septic shock or systemic inflammatory response syndrome (SIRS) and is the most common cause of death in intensive care units. Endotoxin, a constituent of cell walls of Gram-negative bacteria, plays an important role in the initiation and development of MODS. The cytokines, especially tumor necrosis factor alpha (TNF-alpha), are early regulators of the immune response and can induce the release of secondary cytokines. To remove endotoxin and TNF-alpha from patients with MODS, the adsorption method has proven to be most effective. In this review, we provide various methods of removal of endotoxins and TNF-alpha using different adsorbents. (topical review)

  15. Breathtaking TRP Channels: TRPA1 and TRPV1 in Airway Chemosensation and Reflex Control

    OpenAIRE

    Bessac, Bret F.; Jordt, Sven-Eric

    2008-01-01

    New studies have revealed an essential role for TRPA1, a sensory neuronal TRP ion channel, in airway chemosensation and inflammation. TRPA1 is activated by chlorine, reactive oxygen species and noxious constituents of smoke and smog, initiating irritation and airway reflex responses. Together with TRPV1, the capsaicin receptor, TRPA1 may contribute to chemical hypersensitivity, chronic cough and airway inflammation in asthma, COPD and reactive airway dysfunction syndrome.

  16. Breathtaking TRP channels: TRPA1 and TRPV1 in airway chemosensation and reflex control.

    Science.gov (United States)

    Bessac, Bret F; Jordt, Sven-Eric

    2008-12-01

    New studies have revealed an essential role for TRPA1, a sensory neuronal TRP ion channel, in airway chemosensation and inflammation. TRPA1 is activated by chlorine, reactive oxygen species, and noxious constituents of smoke and smog, initiating irritation and airway reflex responses. Together with TRPV1, the capsaicin receptor, TRPA1 may contribute to chemical hypersensitivity, chronic cough, and airway inflammation in asthma, COPD, and reactive airway dysfunction syndrome. PMID:19074743

  17. The effect of fatigue and fibromyalgia on sexual dysfunction in women with chronic fatigue syndrome.

    Science.gov (United States)

    Blazquez, Alicia; Ruiz, Eva; Aliste, Luisa; García-Quintana, Ana; Alegre, José

    2015-01-01

    Sexual dysfunction in patients with chronic fatigue syndrome is attracting growing interest but, to date, few studies have analyzed it. For this reason, the authors evaluated sexual dysfunction in women with chronic fatigue syndrome (using the Golombok Rust Inventory of Sexual Satisfaction) and explore correlations with fatigue and other symptoms. Sexual dysfunction was greater in patients with chronic fatigue syndrome (n = 615) with a higher number of cognitive, neurological, and neurovegetative symptoms, concomitant fibromyalgia, Sjögren's syndrome, or myofascial pain syndrome, and more intense fatigue (p <.05).

  18. [Fiberoptic tracheal intubation through a laryngeal mask airway in a pediatric patient with treacher collins syndrome].

    Science.gov (United States)

    Ogata, Tokiko; Saito, Tomoyuki; Tachikawa, Mayumi; Arai, Takero; Okuda, Yasuhisa

    2013-12-01

    A 6-month-old girl with Treacher Collins syndrome was scheduled for tracheotomy because of severe airway obstruction. During slow induction of anesthesia with inhalation of sevoflurane, assisted mask ventilation was successfully performed using oropharyngeal airway. Either direct laryngoscope or GlideScope Video Laryngoscope could not reveal any part of the epiglottis (Cormack and Lehane grade 4). Even fiberoptic bronchoscopic examination assisted by GlideScope Video Laryngoscope gave a poor view of the pharynx on the video monitor. Laryngeal mask airway (LMA) was inserted easily and allowed adequate ventilation, through which fiberoptic intubation was successfully achieved. We cut the LMA short in order to pass the 3 mm tracheal tube until the glottis through it.

  19. Tracheobronchial stent insertions in the management of major airway obstruction in a patient with Hunter syndrome (type-II mucopolysaccharidosis)

    International Nuclear Information System (INIS)

    We report a case of a 22-year-old male with Hunter syndrome who developed progressive major airway obstruction and was treated with insertion of plastic and metallic stents, with dramatic improvement in the patient's symptomatic and functional status. To the best of our knowledge, this is the first reported case of endoluminal stents being used in the management of major airway obstruction in a patient with Hunter syndrome. (orig.)

  20. Tracheobronchial stent insertions in the management of major airway obstruction in a patient with Hunter syndrome (type-II mucopolysaccharidosis)

    Energy Technology Data Exchange (ETDEWEB)

    Davitt, S.M.; Hatrick, A.; Sabharwal, T.; Adam, A. [Department of Radiology, Guy' s and St. Thomas' Hospitals, London (United Kingdom); Pearce, A. [Department of Anaesthetics, Guy' s and St. Thomas' Hospitals, Londoon (United Kingdom); Gleeson, M. [Department of Otolaryngology, Guy' s and St. Thomas' Hospitals, London (United Kingdom)

    2002-02-01

    We report a case of a 22-year-old male with Hunter syndrome who developed progressive major airway obstruction and was treated with insertion of plastic and metallic stents, with dramatic improvement in the patient's symptomatic and functional status. To the best of our knowledge, this is the first reported case of endoluminal stents being used in the management of major airway obstruction in a patient with Hunter syndrome. (orig.)

  1. Laryngeal mask airway guided tracheal intubation in a neonate with the Pierre Robin syndrome

    DEFF Research Database (Denmark)

    Hansen, T G; Joensen, H; Henneberg, S W;

    1995-01-01

    Endotracheal intubation in infants with the Pierre Robin syndrome may sometimes be impossible to accomplish by conventional means. To aid difficult tracheal intubation many different techniques have been described. We present a case, in which we successfully intubated a small-for-date newborn boy...... with the Pierre Robin syndrome by using a modified laryngeal mask airway (no. 1) as a guide for the endotracheal tube. The technique is easy to perform, less traumatic and less time-consuming than multiple attempts at laryngoscopy or blind tracheal intubation....

  2. Small airways dysfunction in long-term survivors of pediatric stem cell transplantation

    DEFF Research Database (Denmark)

    Uhlving, Hilde Hylland; Mathiesen, Sidsel; Buchvald, Frederik;

    2015-01-01

    BACKGROUND: Chronic graft-versus-host disease (cGvHD) in the lungs is a life-threatening complication of allogeneic hematopoietic stem cell transplantation (HSCT). Pulmonary cGvHD is initiated in the peripheral airways, and diagnosis may be delayed by low sensitivity of standard pulmonary functio...

  3. Pseudo-acute myocardial infarction due to transient apical ventricular dysfunction syndrome (Takotsubo syndrome)

    OpenAIRE

    Maciel, Bruno Araújo; Cidrão, Alan Alves de Lima; Sousa, Ítalo Bruno dos Santos; Ferreira, José Adailson da Silva; Messias Neto, Valdevino Pedro

    2013-01-01

    Takotsubo syndrome is characterized by predominantly medial-apical transient left ventricular dysfunction, which is typically triggered by physical or emotional stress. The present article reports the case of a 61-year-old female patient presenting with dizziness, excessive sweating, and sudden state of ill feeling following an episode involving intense emotional stress. The physical examination and electrocardiogram were normal upon admission, but the troponin I and creatine kinase-MB concen...

  4. The effect of severe acute respiratory syndrome (SARS) on emergency airway management.

    Science.gov (United States)

    Wong, Evelyn; Ho, Khoy Kheng

    2006-07-01

    From early March 2003 to late May 2003, severe acute respiratory syndrome (SARS) was detected in Singapore. The increase in workload and new infection control procedures were thought to affect resuscitation and airway management. Our aim was to study the effects of wearing of personal protective equipment (PPE) and powered air-purifying respirator (PAPR) and the restriction in the number of resuscitation personnel on airway management during the SARS crisis. Data was collected prospectively through an ongoing emergency airway registry. The data was divided into three periods: (1) before PPE was instituted from 1 November 2002 to 31 March 2003; (2) during SARS (when PPE use was mandatory) from 1 April to 31 July 2003; (3) post-SARs (when PPE use was non-mandatory but encouraged) from 1 August to 31 March 2004. There was no change in patient demographics during the three periods. There were significant increases in the proportion of resuscitation cases and airway interventions during the SARS period compared to the pre-SARS period. The resident medical officer intubation rate decreased from 45.1% pre-SARS to 35.2% during SARS and 17.7% post-SARS. The complication rates were 10.5%, 9.9% and 9.4% in periods 1-3, respectively. Restriction in the number of healthcare staff attending to each patient may have influenced the department's decision to allow only the most confident or experienced personnel to manage the airway. The exposure of junior medical officers in emergency airway management during SARS and the immediate post-SARS period was decreased. This trend should be monitored further and intervention may be necessary should it continue to decline.

  5. An unanticipated difficult airway in Lesch-Nyhan syndrome

    Directory of Open Access Journals (Sweden)

    Rashmi Salhotra

    2012-01-01

    Full Text Available An 11-year-old boy with Lesch-Nyhan syndrome presented to the emergency for fixation of a fractured femur. During induction of general anesthesia, unexpected difficult intubation was encountered with a 6.5-mm ID endotracheal tube and successively smaller tubes, also failing to pass 1 cm beyond the vocal cords. Intubation was finally achieved with a 4.5-mm ID tube. The surgery was completed uneventfully. A tracheal diverticulum was found in the computerized tomography (CT scan performed postoperatively to account for this unexpected difficult intubation. This case highlights the anesthetic concerns in Lesch-Nyhan syndrome and also reports the rare occurrence of a tracheal diverticulum associated with it.

  6. Significance of upper airway influence among patients of vocal cord dysfunction for its diagnosis: Role of impulse oscillometry

    Directory of Open Access Journals (Sweden)

    Hira H

    2009-01-01

    Full Text Available Background: To identify the patients of bronchial asthma (suspected or proven, not responding to optimal therapy, for the presence of vocal cord dysfunction (VCD and to compare the diagnostic ability of flow volume (FV loop and impulse oscillometry (IOS. Materials and Methods: Fifty one patients of suspected/proven bronchial asthma not responding to optimal therapy were included for the study. Each patient was subjected to both FV loop and IOS studies. Direct visualization of the vocal cords with flexible fiberoptic bronchoscope for the presence of inspiratory vocal cord adduction during quiet respiration, with speech, and while performing provocative maneuvers was carried out. All patients were subjected to simple pulmonary function tests and recording of FV loop. IOS was performed on each patient to look for the site of obstruction and upper airway influence. The observations of both FV loop and IO studies were compared. Results: Among 51 patients participated, 12 (23.53% had bronchoscopical evidence of VCD and were labeled as VCD-positive group and rest 39 were designated VCD negative. No statistically significant difference in pulmonary function test (prereversibility results between the VCD-positive and VCD-negative patients was found. Reversible airway obstruction was observed in 75% of the patients of VCD-positive group and 67.65% of the patients in the VCD-negative group. Only one patient in the VCD-positive and none in VCD-negative group had inspiratory limb flattening of FV loop. Upper airway influence was evident by IOS in 58.3% of patients in the VCD-positive group and in 15.4% of patients in the VCD-negative group. This difference was statistically significant (P < 0.005. Conclusion: VCD was a common finding in patients with symptoms suggestive of asthma and frequently coexists with asthma. IOS was found to be a useful screening test for VCD and was more sensitive than FV loop.

  7. Volumetric MR imaging of the upper airway in obstructive sleep apnea syndrome

    International Nuclear Information System (INIS)

    Structural abnormalities in the upper airway and surrounding soft tissues may contribute to the obstructive sleep apnea syndrome (OSAS). The authors have utilized MR imaging (3-mm contiguous T1-weighted sagittal images obtained with a local coil at 1.5 T) combined with a computer graphics-based analysis of three-dimensional geometry to study the upper airways of 10 awake, supine normal subjects (29--50 years-old), seven patients with OSAS (34--54 years old), and a nonapneic snorer (24 years old). Upper-airway anatomic segments were compared with regard to regional volumes, minimum cross-sectional areas, and pharyngeal wall thickness. Results to date show a smaller retropalatial airway volume in the patients with OSAS (1.8 cm3 ± 0.8 [SEM]) and a smaller minimum cross-sectional retropalatal area in patients with OSAS (0.45 cm2) than in the nonapneic snorer (0.9 cm2) and the normal subjects (2.5 cm2 ± 0.2)

  8. A case of late-onset allgrove syndrome presenting with predominant autonomic dysfunction

    Directory of Open Access Journals (Sweden)

    Debmalya Sanyal

    2013-01-01

    Full Text Available Allgrove Syndrome or triple A syndrome is a rare familial multisystem disorder characterized by achalasia, alacrima and adrenal insufficiency. The objective was to describe a case of 4A syndrome where autonomic dysfunction was the presenting feature. A 22-year-old male presented with erectile dysfunction and loss of spontaneous morning erections for six months. He was having nocturnal diarrhea and recurrent postural dizziness for three months. He was found to have hyperpigmentation at pressure points, postural hypotension and other features of autonomic dysfunction. Laboratory investigations and imaging studies revealed hypoadrenalism, achalasia, alacrima and peripheral neuropathy. Autonomic neuropathy-related features persisted even after correction of hypoadrenalism. Based on clinical features and investigation he was diagnosed as a case of 4A syndrome presenting with autonomic dysfunction. Allgrove or 4A syndrome should be considered as a rare differential diagnosis of someone presenting with features of autonomic neuropathy.

  9. Congenital high airway obstruction syndrome: MR/US findings, effect on management, and outcome

    Energy Technology Data Exchange (ETDEWEB)

    Mong, Andrew; Johnson, Ann M.; Kramer, Sandra S.; Jaramillo, Diego [Children' s Hospital of Philadelphia, Department of Radiology, Philadelphia, PA (United States); Coleman, Beverly G. [Hospital of the University of Pennsylvania, Department of Radiology, Philadelphia, PA (United States); Hedrick, Holly L.; Flake, Alan; Johnson, Mark; Wilson, R.D.; Adzick, N.S. [Children' s Hospital of Philadelphia, The Center for Fetal Diagnosis and Treatment, Philadelphia, PA (United States); Kreiger, Portia [Children' s Hospital of Philadelphia, Department of Pathology and Laboratory Medicine, Philadelphia, PA (United States)

    2008-11-15

    Congenital high airway obstruction syndrome (CHAOS) is a rare disorder defined as any fetal abnormality that obstructs the larynx or trachea. Prompt airway intervention at delivery after accurate prenatal diagnosis may allow survival of this otherwise fatal condition. To identify prenatal MRI findings in CHAOS, to compare these findings with those of fetal US, to determine if imaging alters diagnosis and management decisions, and to correlate prenatal with postnatal imaging findings. Records and MRI scans of ten fetuses with CHAOS were reviewed, and the findings correlated with outside and same-day fetal US and postnatal imaging findings. Fetal lung volumes were measured on MRI scans. Large lung volumes were found in 90% of the fetuses. Increased lung signal intensity, inverted diaphragm, and a dilated, fluid-filled lower airway were identified in all. The obstruction level was identified in 90%. MRI changed screening US diagnosis in 70%, but was concordant with the tertiary care US imaging in 90%. Seven fetuses were terminated or died in utero, and three fetuses survived after ex utero intrapartum tracheostomy placement. Autopsy or bronchoscopy performed in 60% confirmed CHAOS. Postnatal chest radiographs and CT showed hyperinflation, while US and fluoroscopy showed diminished diaphragmatic motion. MRI demonstrates large lung volumes, increased lung signal intensity, inverted diaphragm, and dilated fluid-filled lower airway, and usually identifies the obstruction level. The degree of correlation between MRI and tertiary prenatal US is high, but CHAOS is frequently misdiagnosed on screening US. Correct diagnosis may enable planned airway management. Voluminous lungs and diaphragmatic abnormalities persist on postnatal imaging. (orig.)

  10. Differential diagnostics of different nosological forms of the temporomandibular joint pain dysfunction syndrome

    OpenAIRE

    Kameneva L.A.; Potapov V.P.; Tlustenko V.P.; Potapov I.V.; Maltceva A.V.; Hatuntseva T.N.

    2014-01-01

    Research objective: to carry out differential diagnostics of various nosological forms of a temporal and temporomandibular joint pain dysfunction syndrome. Material and methods. On clinical base of orthopedic odontology chair of SamSMU inspection of 244 patients with temporomandibular joint pain dysfunction syndrome was performed. Diagnostics was carried out according to the special card of inspection which was developed on orthopedic odontology chair of SamSMU and included subjective, object...

  11. Eye features in three Danish patients with multisystemic smooth muscle dysfunction syndrome

    DEFF Research Database (Denmark)

    Moller, Hans Ulrik; Fledelius, Hans C; Milewicz, Dianna M;

    2012-01-01

    A de novo mutation of the ACTA2 gene encoding the smooth muscle cell α-actin has been established in patients with multisystemic smooth muscle dysfunction syndrome associated with patent ductus arteriosus and mydriasis present at birth.......A de novo mutation of the ACTA2 gene encoding the smooth muscle cell α-actin has been established in patients with multisystemic smooth muscle dysfunction syndrome associated with patent ductus arteriosus and mydriasis present at birth....

  12. Salivary gland dysfunction and xerostomia in Sjögren's syndrome

    DEFF Research Database (Denmark)

    Jensen, Siri Beier; Vissink, Arjan

    2014-01-01

    In this article, salivary gland dysfunction and xerostomia in Sjögren's syndrome (SS) are discussed, with a focus on the pathophysiology of salivary dysfunction in SS, the clinical presentation of dry mouth in SS, how to assess salivary gland hypofunction and xerostomia in SS, and the impact of...

  13. Stressful life events and psychological dysfunction in complex regional pain syndrome type I

    NARCIS (Netherlands)

    Geertzen, JHB; de Bruijn-Kofman, AT; de Bruijn, HP; van de Wiel, HBM; Dijkstra, PU

    1998-01-01

    Objective: To determine to what extent stressful life events and psychological dysfunction play a role in the pathogenesis of Complex Regional Pain Syndrome type I (CRPS). Design: A comparative study between a CRPS group and a control group. Stressful life events and psychological dysfunction evalua

  14. Computational Modeling of Airway Obstruction in Sleep Apnea in Down Syndrome: A Feasibility Study.

    Science.gov (United States)

    Mylavarapu, Goutham; Subramaniam, Dhananjay; Jonnagiri, Raghuvir; Gutmark, Ephraim J; Fleck, Robert J; Amin, Raouf S; Mahmoud, Mohamed; Ishman, Stacey L; Shott, Sally R

    2016-07-01

    Current treatment options are successful in 40% to 60% of children with persistent obstructive sleep apnea after adenotonsillectomy. Residual obstruction assessments are largely subjective and do not clearly define multilevel obstruction. We endeavor to use computational fluid dynamics to perform virtual surgery and assess airflow changes in patients with Down syndrome and persistent obstructive sleep apnea. Three-dimensional airway models were reconstructed from respiratory-gated computed tomography and magnetic resonance imaging. Virtual surgeries were performed on 10 patients, mirroring actual surgeries. They demonstrated how surgical changes affect airflow resistance. Airflow and upper airway resistance was calculated from computational fluid dynamics. Virtual and actual surgery outcomes were compared with obstructive apnea-hypopnea index values. Actual surgery successfully treated 6 of 10 patients (postoperative obstructive apnea-hypopnea index <5). In 8 of 10 subjects, both apnea-hypopnea index and the calculated upper airway resistance after virtual surgery decreased as compared with baseline values. This is a feasibility and proof-of-concept study. Further studies are needed before using these techniques in surgical planning. PMID:27048669

  15. Defective Resensitization in Human Airway Smooth Muscle Cells Evokes β-Adrenergic Receptor Dysfunction in Severe Asthma.

    Directory of Open Access Journals (Sweden)

    Manveen K Gupta

    Full Text Available β2-adrenergic receptor (β2AR agonists (β2-agonist are the most commonly used therapy for acute relief in asthma, but chronic use of these bronchodilators paradoxically exacerbates airway hyper-responsiveness. Activation of βARs by β-agonist leads to desensitization (inactivation by phosphorylation through G-protein coupled receptor kinases (GRKs which mediate β-arrestin binding and βAR internalization. Resensitization occurs by dephosphorylation of the endosomal βARs which recycle back to the plasma membrane as agonist-ready receptors. To determine whether the loss in β-agonist response in asthma is due to altered βAR desensitization and/or resensitization, we used primary human airway smooth muscle cells (HASMCs isolated from the lungs of non-asthmatic and fatal-asthmatic subjects. Asthmatic HASMCs have diminished adenylyl cyclase activity and cAMP response to β-agonist as compared to non-asthmatic HASMCs. Confocal microscopy showed significant accumulation of phosphorylated β2ARs in asthmatic HASMCs. Systematic analysis of desensitization components including GRKs and β-arrestin showed no appreciable differences between asthmatic and non-asthmatic HASMCs. However, asthmatic HASMC showed significant increase in PI3Kγ activity and was associated with reduction in PP2A activity. Since reduction in PP2A activity could alter receptor resensitization, endosomal fractions were isolated to assess the agonist ready β2ARs as a measure of resensitization. Despite significant accumulation of β2ARs in the endosomes of asthmatic HASMCs, endosomal β2ARs cannot robustly activate adenylyl cyclase. Furthermore, endosomes from asthmatic HASMCs are associated with significant increase in PI3Kγ and reduced PP2A activity that inhibits β2AR resensitization. Our study shows that resensitization, a process considered to be a homeostasis maintaining passive process is inhibited in asthmatic HASMCs contributing to β2AR dysfunction which may underlie

  16. Impaired Capacity of Fibroblasts to Support Airway Epithelial Progenitors in Bronchiolitis Obliterans Syndrome

    Science.gov (United States)

    Zhang, Su-Bei; Sun, Xin; Wu, Qi; Wu, Jun-Ping; Chen, Huai-Yong

    2016-01-01

    Background: Bronchiolitis obliterans syndrome (BOS) often develops in transplant patients and results in injury to the respiratory and terminal airway epithelium. Owing to its rising incidence, the pathogenesis of BOS is currently an area of intensive research. Studies have shown that injury to the respiratory epithelium results in dysregulation of epithelial repair. Airway epithelial regeneration is supported by stromal cells, including fibroblasts. This study aimed to investigate whether the supportive role of lung fibroblasts is altered in BOS. Methods: Suspensions of lung cells were prepared by enzyme digestion. Lung progenitor cells (LPCs) were separated by fluorescence-activated cell sorting. Lung fibroblasts from patients with BOS or healthy controls were mixed with sorted mouse LPCs to compare the colony-forming efficiency of LPCs by counting the number of colonies with a diameter of ≥50 μm in each culture. Statistical analyses were performed using the SPSS 17.0 software (SPSS Inc., USA). The paired Student's t-test was used to test for statistical significance. Results: LPCs were isolated with the surface phenotype of CD31- CD34- CD45- EpCAM+ Sca-1+. The colony-forming efficiency of LPCs was significantly reduced when co-cultured with fibroblasts isolated from patients with BOS. The addition of SB431542 increased the colony-forming efficiency of LPCs to 1.8%; however, it was still significantly less than that in co-culture with healthy control fibroblasts (P < 0.05). Conclusion: The epithelial-supportive capacity of fibroblasts is impaired in the development of BOS and suggest that inefficient repair of airway epithelium could contribute to persistent airway inflammation in BOS. PMID:27569228

  17. The asthma–COPD overlap syndrome: do we really need another syndrome in the already complex matrix of airway disease?

    Directory of Open Access Journals (Sweden)

    Kostikas K

    2016-06-01

    Full Text Available Konstantinos Kostikas, Andreas Clemens, Francesco Patalano Novartis Pharma AG, Basel, Switzerland Abstract: The term asthma–COPD overlap syndrome (ACOS is one of multiple terms used to describe patients with characteristics of both COPD and asthma, representing ~20% of patients with obstructive airway diseases. The recognition of both sets of morbidities in patients is important to guide practical treatment decisions. It is widely recognized that patients with COPD and coexisting asthma present with a higher disease burden, despite the conceptual expectation that the “reversible” or “treatable” component of asthma would allow for more effective management and better outcomes. However, subcategorization into terms such as ACOS is complicated by the vast spectrum of heterogeneity that is encapsulated by asthma and COPD, resulting in different clinical clusters. In this review, we discuss the possibility that these different clusters are suboptimally described by the umbrella term “ACOS”, as this additional categorization may lead to clinical confusion and potential inappropriate use of resources. We suggest that a more clinically relevant approach would be to recognize the extreme variability and the numerous phenotypes encompassed within obstructive airway diseases, with various degrees of overlapping in individual patients. In addition, we discuss some of the evidence to be considered when making practical decisions on the treatment of patients with overlapping characteristics between COPD and asthma, as well as the potential options for phenotype and biomarker-driven management of airway disease with the aim of providing more personalized treatment for patients. Finally, we highlight the need for more evidence in patients with overlapping disease characteristics and to facilitate better characterization of potential treatment responders. Keywords: emphysema, chronic bronchitis, COPD, asthma, ACOS, overlap syndrome

  18. Endothelial dysfunction in patients with metabolic syndrome: a prospective study in a rural institute in India

    Directory of Open Access Journals (Sweden)

    Khwaja Saifullah Zafar

    2014-08-01

    Conclusion: Estimation of Endothelial Dysfunction in patients at risk of developing full blown Metabolic Syndrome may predict the cardiovascular morbidity and mortality in these individuals even before fulfilling the 3/5 criteria of NCEP/ATP III Guidelines for the diagnosis of metabolic syndrome. [Int J Res Med Sci 2014; 2(4.000: 1612-1619

  19. Management of dysfunctional tear syndrome: a Canadian consensus.

    Science.gov (United States)

    Jackson, W Bruce

    2009-08-01

    Dry eye complaints are common, have a diverse etiology, and result from disruption of the normal tear film; hence, the term "dysfunctional tear syndrome." Recent research has shown that ocular surface disorders have an inflammatory origin, that inflammation of the ocular surface does not always manifest as "red eye," and that a patient does not have to have a systemic autoimmune disease to experience a local, ocular autoimmune event. A panel of Canadian cornea and external disease subspecialists met and developed a questionnaire and treatment algorithm to aid the comprehensive ophthalmologist. Management of ocular surface disorders begins with a review of the patient's medical history, with particular attention to medication use, and a thorough ophthalmological examination. Use of a simple questionnaire can aid in the diagnosis. A variety of treatment modalities are available, the most effective of which are those that target the underlying inflammatory process with the goal of restoring the normal tear film. A treatment algorithm is presented that matches the severity of symptoms with the intensity of treatment. Lifestyle modifications, regular hygiene, and tear supplements may be sufficient in patients with mild symptoms. Anti-inflammatory medications (topical cyclosporin A, short courses of topical steroids, and [or] oral tetracyclines) and physical measures (punctal plugs, moisture-retaining eye wear) are implemented for those with moderate-to-severe symptoms. Autologous serum tears, scleral contact lenses, and surgery are reserved for patients with severe symptoms who have an unsatisfactory response to anti-inflammatory medications. Patients with lid disease or rosacea and those with allergic conditions should be identified during the initial encounter and should receive specific therapy to relieve their symptoms.

  20. Dysaesthesias and dysautonomia: a self-limited syndrome of painful dysaesthesias and autonomic dysfunction in childhood.

    OpenAIRE

    Nass, R.; Chutorian, A

    1982-01-01

    Three children with an acute self-limited syndrome characterised by painful dysaethesias, hypertension, and autonomic dysfunction, in the absence of motor and reflex abnormalities, are presented. They appear to have had a variant of acute polyneuritis involving sensory and autonomic systems. The pathophysiology of hypertension in the Guillain-Barré syndrome and of acute pandysautonomia is discussed. Excessive adrenergic function is considered as a cause of the pain component of the syndrome.

  1. 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.

  2. COMPUTED TOMOGRAPHIC, RADIOGRAPHIC, AND ENDOSCOPIC TRACHEAL DIMENSIONS IN ENGLISH BULLDOGS WITH GRADE 1 CLINICAL SIGNS OF BRACHYCEPHALIC AIRWAY SYNDROME

    NARCIS (Netherlands)

    Kaye, Benjamin M; Boroffka, Susanne A E B; Haagsman, Annika N; Haar, Gert Ter

    2015-01-01

    Tracheal hypoplasia is commonly seen in English Bulldogs affected with brachycephalic airway syndrome. Previously published diagnostic criteria for tracheal hypoplasia in this breed have been a radiographic tracheal diameter:tracheal inlet ratio (TD:TI) < 0.12 or a tracheal diameter:third rib diamet

  3. Differential diagnostics of different nosological forms of the temporomandibular joint pain dysfunction syndrome

    Directory of Open Access Journals (Sweden)

    Kameneva L.A.

    2014-09-01

    Full Text Available Research objective: to carry out differential diagnostics of various nosological forms of a temporal and temporomandibular joint pain dysfunction syndrome. Material and methods. On clinical base of orthopedic odontology chair of SamSMU inspection of 244 patients with temporomandibular joint pain dysfunction syndrome was performed. Diagnostics was carried out according to the special card of inspection which was developed on orthopedic odontology chair of SamSMU and included subjective, objective and special methods of research. As a result of research it is taped: 71 — an occlusal-articulation dysfunctional syndrome, 44 — a neuromuscular dysfunctional syndrome, 76 patients have a hardly set dislocation of intra joint disk and 53 — a habitual dislocation and a subluxation of a temporomandibular joint. We used Yu.A. Petrosov's classification as it displays most precisely the processes happening at pathology of the intra joint relations. Conclusion: On the basis of the obtained data the table of differential diagnostics of various nosological forms of temporomandibular joint pain dysfunction syndrome has been made.

  4. [Automatic positive airway pressure in titration and treatment of the obstructive sleep apnea syndrome].

    Science.gov (United States)

    Randerath, W J

    2007-04-01

    Although continuous airway pressure therapy (CPAP) represents the standard treatment for obstructive sleep apnea syndrome (OSAS) auto-adjusting CPAP (APAP) devices were developed which adapt the treatment pressure to the actual requirement of the patients. The aim of automatic CPAP therapy is to improve the patients' acceptance of positive pressure treatment. The devices react to respiratory flow, flattening of the inspiratory flow contour, snoring, generator speed or the upper airway impedance. In recent years several studies showed that auto CPAP effectively treats respiratory disturbances, improves sleep profile and the self-assessment of the patients equally as good as the gold standard constant CPAP. Moreover, APAP reduces the treatment pressure substantially. Although an improvement of the patient's compliance has not consistently been proven, most patients prefer APAP versus constant CPAP. APAP devices use different algorithms depending on the primary purpose of the application. Therefore, a clear distinction between automatic titration and treatment is of major relevance. While titration devices aim at the finding of one single pressure which is fixed to a constant CPAP device, automatic treatment means the chronic use of APAP at home for optimal adaptation of the treatment pressure to the actual requirements of the patient. A high constant CPAP level, huge pressure variability, insufficient compliance with constant CPAP may be indications for APAP treatment. The main reason for automatic titration is the standardisation of the initiation process. PMID:17455137

  5. Introduction to a new clinical entity:the multiple organ dysfunction syndrome in the elderly

    Institute of Scientific and Technical Information of China (English)

    Shiwen WANG

    2004-01-01

    @@ Multiple organ dysfunction syndrome in the elderly (MODSE) is an important syndrome in the critical care of elderly patients. MODSE is defined as simultaneous or sequential dysfunction or failure of two or more organs on the top of advanced age and chronic multiple organ dysfunction. MODSE is triggered by precipitating factors such as infection (usually pulmonary infection) trauma,surgery, etc. It occurs in two phases. In the early phase, dysfunction of multiple organs (MODE) occurs,and in the later or severe phase, multiple organ failure (MOFE) occurs. MODSE is the most common cause of mortality in the critically iii elderly patient. It is important to understand its clinical characteristics and elucidate its pathogenesis in order to reduce mortality and improve quality of life for these patients.

  6. Maxillary sinusitis as a differential diagnosis in temporomandibular joint pain-dysfunction syndrome.

    Science.gov (United States)

    Rihani, A

    1985-01-01

    Maxillary sinusitis may be diagnosed incorrectly as TMJ pain-dysfunction syndrome because of a similarity of signs and symptoms. Both conditions can manifest with headache, facial pain radiating to the ear and the maxillary teeth, preauricular pain, and pain in the buccal vestibule posterior and superior to the maxillary tuberosity. It can be concluded that (1) more consideration should be given to sinus disturbances as a differential diagnosis in TMJ pain-dysfunction syndrome, (2) it may be preferable to refer some patients with TMJ pain to a medical center where specialists in dentistry, otolaryngology, neurology, rheumatology, and psychiatry can evaluate the patient, and (3) TMJ pain-dysfunction syndrome should be evaluated and treated by a dentist experienced in management of this disorder. PMID:3856028

  7. Acute superior vena caval syndrome with airway obstruction following elective mediastinoscopy.

    LENUS (Irish Health Repository)

    Power, C K

    2012-02-03

    A 47-year-old female patient had a subclinical superior vena caval syndrome which developed into the \\'full blown\\' acute condition when she was placed into the left lateral position after mediastinoscopy. She developed airway obstruction requiring urgent re-intubation and subsequent admission to the intensive care unit. This subclinical condition might have been suspected pre-operatively if closer attention had been paid to the history, physical examination and review of the computerised axial tomography scan: she had a history of intermittent dysponea, wheeze and cough which was worse on waking and improved as the day progressed, she had a positive Pemberton\\'s sign and the computerised axial tomography scan showed that the lesion was encroaching on the superior vena cava.

  8. Multiple organ dysfunction syndrome due to massive wasp stings:an autopsy case report

    Institute of Scientific and Technical Information of China (English)

    ZHANG Ling; TANG Yi; LIU Fang; SHI Yu-ying; CAO Yu; XU Huan; FU Ping

    2012-01-01

    We reported a case of multiple organ dysfunction syndrome (MODS) following about 300 wasp stings.The diagnosis was based on autopsy findings of acute pulmonary edema,acute kidney injury,hepatic and cardiac dysfunction,and cerebral edema.MODS is a life-threatening complication,and should be considered a possibility after multiple wasp stings.Our autopsy helped to establish the cause of unexpected death due to wasp stings and to elucidate a possible mechanism of MODS.

  9. 'TEST-NEGATIVE ANGELMAN SYNDROME' WITH THYROID DYSFUNCTION: A RARITY BUT A REALITY!

    Science.gov (United States)

    Ali, Mohammad Usman; Mehmood, Anjum; Ali, Mahrukh Ayesha

    2015-01-01

    Angelman Syndrome (AS) is believed to be a complex neuro-developmental genetic disorder that is often described clinically by the presence of behavioural uniqueness and movement disorders; in addition to having developmental delay and speech impairment. Genetic factors have been linked to the syndrome's aetiology in 90% of cases, although in 10% cases, an unidentified genetic mechanism accounts for the classic phenotypic features of AS. Angelman Syndrome in general or with associated thyroid dysfunction, have never been reported from Pakistan. This is the first ever case report from Pakistan reporting a rare case of clinically diagnosed AS with associated thyroid dysfunction in the presence of normal molecular genetic testing (DNA methylation test and UBE3A gene sequencing). In future, clinicians should make efforts in documenting similar cases with associated clinical profiles from our part of the world, thereby contributing to the local and regional epidemiology of these syndromes. PMID:27004361

  10. Interactions of Constipation, Dysfunctional Elimination Syndrome, and Vesicoureteral Reflux

    Directory of Open Access Journals (Sweden)

    Walid A. Farhat

    2008-06-01

    Full Text Available Vesicoureteral reflux (VUR is simply described as incompetence of the unidirectional valve at the ureterovesical junction (UVJ, leading to backflow of urine to the kidney. Today, it is clear that VUR is not only related to the UVJ function but also to a combination of processes including immunity, bladder and pelvic floor function, dysfunctional voiding, and constipation. Although our surgical aims directed towards improving the valve coaptation at the UVJ, we understand today the importance of the diagnosis and treatment of constipation and dysfunctional voiding adjunctively.

  11. Evaluation of Autonomic Dysfunction in Obstructive Sleep Apnea Syndrome

    Directory of Open Access Journals (Sweden)

    Ahmet Turan Evlice

    2012-04-01

    Full Text Available The nervous system, which controls the body's internal organs is called Autonomic Nervous System. Parkinson's disease, vascular diseases, diabetes mellitus and Guilllain-Barre syndrome cause to disotonomia. Recent studies has been shown, obstructive sleep apnea syndrome cause to disotonomia too. To investigate disotonomia in in obstructive sleep apnea syndrome , should be preferred the methods like analysis of heart rate variability and sympathetic skin response which have low cost and easy applicability. Thus, it will be possible to prevent morbidity and mortality due to autonomic dysfuncion. [Archives Medical Review Journal 2012; 21(2.000: 109-121

  12. Reward circuitry dysfunction in psychiatric and neurodevelopmental disorders and genetic syndromes: animal models and clinical findings

    Directory of Open Access Journals (Sweden)

    Dichter Gabriel S

    2012-07-01

    Full Text Available Abstract This review summarizes evidence of dysregulated reward circuitry function in a range of neurodevelopmental and psychiatric disorders and genetic syndromes. First, the contribution of identifying a core mechanistic process across disparate disorders to disease classification is discussed, followed by a review of the neurobiology of reward circuitry. We next consider preclinical animal models and clinical evidence of reward-pathway dysfunction in a range of disorders, including psychiatric disorders (i.e., substance-use disorders, affective disorders, eating disorders, and obsessive compulsive disorders, neurodevelopmental disorders (i.e., schizophrenia, attention-deficit/hyperactivity disorder, autism spectrum disorders, Tourette’s syndrome, conduct disorder/oppositional defiant disorder, and genetic syndromes (i.e., Fragile X syndrome, Prader–Willi syndrome, Williams syndrome, Angelman syndrome, and Rett syndrome. We also provide brief overviews of effective psychopharmacologic agents that have an effect on the dopamine system in these disorders. This review concludes with methodological considerations for future research designed to more clearly probe reward-circuitry dysfunction, with the ultimate goal of improved intervention strategies.

  13. Comparison of the effects of continuous positive airway pressure, oral appliance and exercise training in obstructive sleep apnea syndrome

    Directory of Open Access Journals (Sweden)

    Teresa Cristina Barros Schutz

    2013-01-01

    Full Text Available OBJECTIVE: There are several treatments for obstructive sleep apnea syndrome, such as weight loss, use of an oral appliance and continuous positive airway pressure, that can be used to reduce the signs and symptoms of obstructive sleep apnea syndrome. Few studies have evaluated the effectiveness of a physical training program compared with other treatments. The aim of this study was to assess the effects of physical exercise on subjective and objective sleep parameters, quality of life and mood in obstructive sleep apnea patients and to compare these effects with the effects of continuous positive airway pressure and oral appliance treatments. METHODS: Male patients with moderate to severe obstructive sleep apnea and body mass indices less than 30 kg/m2 were randomly assigned to three groups: continuous positive airway pressure (n = 9, oral appliance (n = 9 and physical exercise (n = 7. Polysomnographic recordings, blood samples and daytime sleepiness measurements were obtained prior to and after two months of physical exercise or treatment with continuous positive airway pressure or an oral appliance. Clinicaltrials.gov: NCT01289392 RESULTS: After treatment with continuous positive airway pressure or an oral appliance, the patients presented with a significant reduction in the apnea-hypopnea index. We did not observe changes in the sleep parameters studied in the physical exercise group. However, this group presented reductions in the following parameters: T leukocytes, very-low-density lipoprotein and triglycerides. Two months of exercise training also had a positive impact on subjective daytime sleepiness. CONCLUSIONS: Our results suggest that isolated physical exercise training was able to modify only subjective daytime sleepiness and some blood measures. Continuous positive airway pressure and oral appliances modified the apnea-hypopnea index.

  14. Increased myocardial fibrosis and left ventricular dysfunction in Cushing's syndrome.

    NARCIS (Netherlands)

    Yiu, K.H.; Marsan, N.A.; Delgado, V.; Biermasz, N.R.; Holman, E.R.; Smit, J.W.A.; Feelders, R.A.; Bax, J.J.; Pereira, A.M.

    2012-01-01

    OBJECTIVE: Active Cushing's syndrome (CS) is associated with cardiomyopathy, characterized by myocardial structural, and ultrastructural abnormalities. The extent of myocardial fibrosis in patients with CS has not been previously evaluated. Therefore, the objective of this study was to assess myocar

  15. Vascular risk factors and adipocyte dysfunction in metabolic syndrome

    NARCIS (Netherlands)

    Hajer, G.R.

    2008-01-01

    The cluster of vascular risk factors closely associated with obesity, consists of fasting and postprandial dyslipidemia, hypertension, and insulin resistance, also known as metabolic syndrome, is associated with an increased cardiovascular morbidity and mortality. In addition, adipose tissue in conc

  16. Can Individualized Health Care Plans Help Increase Continence in Children with Dysfunctional Elimination Syndrome?

    Science.gov (United States)

    Boisclair-Fahey, Anne

    2009-01-01

    School-age children with dysfunctional elimination syndrome (DES) do not always have school support for their treatment plans, including an every 2-hr voiding schedule. The objective of this study was to increase school support of treatment plans by allowing access to bathrooms, thereby improving continence. An eight-question survey about bathroom…

  17. Daytime sleepiness, cognitive performance and mood after continuous positive airway pressure for the sleep apnoea/hypopnoea syndrome.

    OpenAIRE

    Engleman, H. M.; Cheshire, K. E.; Deary, I.J.; Douglas, N.J.

    1993-01-01

    BACKGROUND--Patients with the sleep apnoea/hypopnoea syndrome often receive continuous positive airway pressure to improve their symptoms and daytime performance, yet objective evidence of the effect of this treatment on cognitive performance is lacking. METHODS--A prospective parallel group study was performed comparing the change in objective daytime sleepiness as assessed by multiple sleep latency, cognitive function, and mood in 21 patients (mean (SE) number of apnoeas and hypopnoeas/hour...

  18. Cognitive Dysfunction in Chronic Fatigue Syndrome: a Review of Recent Evidence.

    Science.gov (United States)

    Cvejic, Erin; Birch, Rachael C; Vollmer-Conna, Uté

    2016-05-01

    Cognitive difficulties represent a common and debilitating feature of the enigmatic chronic fatigue syndrome (CFS). These difficulties manifest as self-reported problems with attention, memory, and concentration and present objectively as slowed information processing speed particularly on complex tasks requiring sustained attention. The mechanisms underlying cognitive dysfunction remain to be established; however, alterations in autonomic nervous system activity and cerebral blood flow have been proposed as possibilities. Heterogeneity in the experience of cognitive impairment, as well as differences in the methods utilised to quantify dysfunction, may contribute to the difficulties in establishing plausible biological underpinnings. The development of a brief neurocognitive battery specifically tailored to CFS and adoption by the international research community would be beneficial in establishing a profile of cognitive dysfunction. This could also provide better insights into the underlying biological mechanisms of cognitive dysfunction in CFS and enhance the development of targeted treatments. PMID:27032787

  19. Roentgenographic observation of the myofascial pain dysfunction syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Ahn, Hyung Kyu [Department of Radiology, College of Dentistry, Seoul National University, Seoul (Korea, Republic of)

    1975-11-15

    The author has observed 64 films from M.P.D. syndrome cases taken in Dept. of Oral Radiology, the Infirmary of Den tal College, Seoul National University in 1974, and obtained the following results: 1. In M.P.D. syndrome, the ratio between the female and the male were 2:1. 2. The prevalent age was aged 20,30,10, and 40 in decreasing order. 3. The incidence was 21.8% in the left side, 29% in the right, and 21% was bilaterally, which show the right most frequent. 4. The roentgenograms revealed variable findings in each case, but there were not recognized any significant differences.

  20. Obstructive airway in Morquio A syndrome, the past, the present and the future.

    Science.gov (United States)

    Tomatsu, Shunji; Averill, Lauren W; Sawamoto, Kazuki; Mackenzie, William G; Bober, Michael B; Pizarro, Christian; Goff, Christopher J; Xie, Li; Orii, Tadao; Theroux, Mary

    2016-02-01

    Patients with severe tracheal obstruction in Morquio A syndrome are at risk of dying of sleep apnea and related complications. Tracheal obstruction also leads to life-threatening complications during anesthesia as a result of the difficulty in managing the upper airway due to factors inherent to the Morquio A syndrome, compounded by the difficulty in intubating the trachea. A detailed description of the obstructive pathology of the trachea is not available in the literature probably due to lack of a homogenous group of Morquio A patients to study at any one particular center. We present a series of cases with significant tracheal obstruction who were unrecognized due to the difficulty in interpreting tracheal narrowing airway symptoms. Our goal is to provide the guidelines in the management of these patients that allow earlier recognition and intervention of tracheal obstruction. Sagittal MRI images of the cervical spine of 28 Morquio A patients (12±8.14years) showed that19/28 (67.9%) patients had at least 25% tracheal narrowing and that narrowing worsened with age (all 8 patients over 15years had greater than 50% narrowing). Eight out of 28 patients were categorized as severe (>75%) tracheal narrowing when images were evaluated in neutral head and neck position. Of the 19 patients with tracheal narrowing, compression by the tortuous brachiocephalic artery was the most common cause (n=15). Evidence of such tracheal narrowing was evident as early as at 2years of age. The etiology of tracheal impingement by the brachiocephalic artery in Morquio A appears to be due to a combination of the narrow thoracic inlet crowding structures and the disproportionate growth of trachea and brachiocephalic artery in relationship to the chest cavity leading to tracheal tortuosity. In conclusion, tracheal narrowing, often due to impression from the crossing tortuous brachiocephalic artery, increases with age in Morquio A patients. Greater attention to the trachea is needed when

  1. The Neuropsychology of Down Syndrome: Evidence for Hippocampal Dysfunction.

    Science.gov (United States)

    Pennington, Bruce F.; Moon, Jennifer; Edgin, Jamie; Stedron, Jennifer; Nadel, Lynn

    2003-01-01

    Tested prefrontal and hippocampal functions in school-aged individuals with Down syndrome (DS) compared functions with those of typically developing children individually matched on mental age. Found that hippocampal and prefrontal composite scores contributed unique variance to the prediction of mental age and adaptive behavior. Noted a…

  2. Timing Deficits Are Implicated in Motor Dysfunction in Asperger's Syndrome

    Science.gov (United States)

    Price, Kelly J.; Edgell, Dorothy; Kerns, Kimberly A.

    2012-01-01

    This study addressed what role movement timing irregularities have in producing the motor deficits documented in Asperger's Syndrome (AS). Participants included males with AS (n = 14) and without (n = 16), matched by age (7-23 years) and with no significant IQ differences. They completed measures of timing perception (comparisons of tempo of…

  3. Cardiac dysfunction is reversed upon successful treatment of Cushing's syndrome

    NARCIS (Netherlands)

    A.M. Pereira (Alberto); V. Delgado (Victoria); J.A. Romijn (Johannes); J.W.A. Smit (Jan); J.J. Bax (Jeroen); R.A. Feelders (Richard)

    2010-01-01

    textabstractObjective: In patients with active Cushing's syndrome (CS), cardiac structural and functional changes have been described in a limited number of patients. It is unknown whether these changes reverse after successful treatment. We therefore evaluated the changes in cardiac structure and d

  4. MRI analysis on soft tissue around upper airway in obese adolescent patients with obstructive sleep apnea syndrome

    International Nuclear Information System (INIS)

    Objective: To investigate the correlation of soft tissue structure of upper airway with the pathogenesis of obstructive sleep apnea syndrome (OSAS) in adolescents age group by analyzing magnetic resonance imaging (MRI) of upper airway. Methods: The subjects were divided into obese OSAS, obese controls and normal weight controls groups according to the results from polysomnography and body mass index measurements; Upper airway was scanned by MRI sagittally and axially; upper airway at all levels and soft tissue was analyzed by Amira Medical image analysis system. Results: Tongue volumes in obese OSAS and obese controls were significantly greater than that in normal weight controls (P<0.05); tonsil and adenoid volumes in obese OSAS were significantly higher than those in two control groups (P<0.05 or P<0.001), but no significant difference was found between two control groups. The volumes of lateral pharyngeal wall in obese OSAS were higher than those in obese controls and normal weight controls (P<0.05 or P<0.001), and they were higher in obese controls compared with normal weight controls (P<0.05). In obese OSAS group, positive correlations were found between volumes of lateral pharyngeal wall and apnea/hypopnea index (AHI) (r=0.879, P<0.01), as well volumes of tonsils and AHI (r=0.824, P<0.01). Conclusion: Obesity can increase the soft tissue volumes around upper airway, there by increase the upper airway obstruction; lateral pharyngeal wall and adenoid volumes play major roles in evaluating the severity of OSAS in adolescents. (authors)

  5. Association and pattern of diastolic dysfunction in patients of metabolic syndrome

    International Nuclear Information System (INIS)

    Diastolic dysfunction is important predictor of morbidity and mortality in patients with metabolic syndrome. This prospective study is to evaluate an association and pattern of diastolic dysfunction in patients of metabolic syndrome in our population. This cross-sectional study was performed at Armed Forces Institute of Cardiology Rawalpindi for a period of 6 months from 20th November 2007 to 20th April 2008. One hundred eligible and consenting patients having metabolic syndrome reporting in the OPD were registered. Inclusion criteria included patients of metabolic syndrome with negative ETT and normal systolic function. Exclusion criteria were patients with age above 60 years and valvular heart disease. Data was collected by a structured clinical interview with a physician, ECG and a transthoracic M-mode, 2D and TDI echocardiogram. The metabolic syndrome was defined according to International Diabetes Federation. There was a positive association between the degree of the metabolic syndrome-assessed as number of concurrently present components-and parameters of cardiac structure and function, with a consistent and statistically significant trend for all cardiac variables considered(p=0.000). There was also a positive association between each parameter and the cardiac diastolic dysfunction grading, e.g., systolic blood pressure (p=0.000), diastolic blood pressure (p=0.005), waist circumference (p=0.004), fasting blood sugar (p=0.008), triglycerides (p=0.006), HDL cholesterol (p=0.001). Several cardiac functional abnormalities regardless of symptoms increased progressively with increasing degree of metabolic syndrome. (author)

  6. OBSTRUCTIVE SLEEP APNEA AND METABOLIC DYSFUNCTION IN POLYCYSTIC OVARY SYNDROME

    OpenAIRE

    Nitsche, Katie; Ehrmann, David A.

    2010-01-01

    Obstructive sleep apnea (OSA) is an underrecognized, yet significant factor in the pathogenesis of metabolic derangements in polycystic ovary syndrome (PCOS). Recent findings suggest that there may be two “subtypes” of PCOS, i.e. PCOS with or without OSA, and these two subtypes may be associated with distinct metabolic and endocrine alterations. PCOS women with OSA may be at much higher risk for diabetes and cardiovascular disease than PCOS women without OSA and may benefit from therapeutic i...

  7. Thyroid dysfunction in Down's syndrome and screening for hypothyroidism in children and adolescents using capillary TSH measurement.

    LENUS (Irish Health Repository)

    Murphy, J

    2008-02-01

    Thyroid dysfunction is more common in individuals with Down\\'s syndrome (DS) than in the general population, whose clinical features can mask the presenting signs and symptoms of hypothyroidism. Biochemical screening is necessary; however, venepuncture may be difficult.

  8. Respiratory and autonomic dysfunction in congenital central hypoventilation syndrome.

    Science.gov (United States)

    Moreira, Thiago S; Takakura, Ana C; Czeisler, Catherine; Otero, Jose J

    2016-08-01

    The developmental lineage of the PHOX2B-expressing neurons in the retrotrapezoid nucleus (RTN) has been extensively studied. These cells are thought to function as central respiratory chemoreceptors, i.e., the mechanism by which brain Pco2 regulates breathing. The molecular and cellular basis of central respiratory chemoreception is based on the detection of CO2 via intrinsic proton receptors (TASK-2, GPR4) as well as synaptic input from peripheral chemoreceptors and other brain regions. Murine models of congenital central hypoventilation syndrome designed with PHOX2B mutations have suggested RTN neuron agenesis. In this review, we examine, through human and experimental animal models, how a restricted number of neurons that express the transcription factor PHOX2B play a crucial role in the control of breathing and autonomic regulation. PMID:27226447

  9. Evaluation of Salivary Cortisol and Anxiety Levels in Myofascial Pain Dysfunction Syndrome

    OpenAIRE

    Nadendla, Lakshmi Kavitha; Meduri, Venkateswarlu; Paramkusam, Geetha; Pachava, Koteswara Rao

    2013-01-01

    Background Myofascial pain dysfunction syndrome (MPDS), otherwise called myofascial pain is one of the most common temporomandibular disorders, which in turn is the most common cause of orofacial pain of non-dental origin. Its etiology is multifactorial and still poorly understood. Psychological factors have been shown to play a role in the etiology. The aim of the study was to evaluate the association between anxiety and salivary cortisol levels in patients with myofascial pain. Methods Twen...

  10. Management of patients with rectocele, multiple pelvic floor dysfunctions and obstructed defecation syndrome

    Directory of Open Access Journals (Sweden)

    Sthela Maria Murad-Regadas

    2012-06-01

    Full Text Available CONTEXT: Management of patients with obstructed defecation syndrome is still controversial. OBJECTIVE: To analyze the efficacy of clinical, clinical treatment followed by biofeedback, and surgical treatment in patients with obstructed defecation, rectocele and multiple dysfunctions evaluated with echodefecography. METHODS: The study included 103 females aged 26-84 years with obstructed defecation, grade-II/III rectocele and multiple dysfunctions on echodefecography. Patients were distributed into three treatment groups and constipation scores were assigned. Group I: 34 (33% patients with significant improvement of symptoms through clinical management only. Group II: 14 (14% with improvement through clinical treatment plus biofeedback. Group III: 55 (53% referred to surgery due to treatment failure. RESULTS: Group I: 20 (59% patients had grade-II rectocele, 14 (41% grade-III. Obstructed defecation syndrome was associated with intussusception (41%, mucosal prolapse (41%, anismus (29%, enterocele (9% or 2 dysfunctions (23%. The average constipation score decreased significantly from 11 to 5. Group II: 11 (79% grade-II rectocele, 3 (21% grade-III, associated with intussusception (7%, mucosal prolapse (43%, anismus (71% or 2 dysfunctions (29%. There was significant decrease in constipation score from 13 to 6. Group III: 8 (15% grade-II rectocele, 47 (85% grade-III, associated with intussusception (42%, mucosal prolapse (40% or 2 dysfunctions (32%. The constipation score remained unchanged despite clinical treatment and biofeedback. Twenty-three underwent surgery had a significantly decrease in constipation score from 12 to 4. The remaining 32 (31% patients which 22 refused surgery, 6 had low anal pressure and 4 had slow transit. CONCLUSIONS: Approximately 50% of patients with obstructed defecation, rectocele and multiple dysfunctions presented a satisfactory response to clinical treatment and/or biofeedback. Surgical repair was mainly required in

  11. CXCL12/CXCR4-Axis Dysfunctions: Markers of the Rare Immunodeficiency Disorder WHIM Syndrome

    Directory of Open Access Journals (Sweden)

    Françoise Bachelerie

    2010-01-01

    Full Text Available The WHIM syndrome features susceptibility to human Papillomavirus infection-induced warts and carcinomas, hypogammaglobulinemia, recurrent bacterial infections, B and T-cell lymphopenia, and neutropenia associated with retention of senescent neutrophils in the bone marrow (i.e. myelokathexis. This rare disorder is mostly linked to inherited heterozygous autosomal dominant mutations in the gene encoding CXCR4, a G protein coupled receptor with a unique ligand, the chemokine CXCL12/SDF-1. Some individuals who have full clinical forms of the syndrome carry a wild type CXCR4 gene. In spite of this genetic heterogeneity, leukocytes from WHIM patients share in common dysfunctions of the CXCR4-mediated signaling pathway upon exposure to CXCL12. Dysfunctions are characterized by impaired desensitization and receptor internalization, which are associated with enhanced responses to the chemokine. Our increasing understanding of the mechanisms that account for the aberrant CXCL12/CXCR4-mediated responses is beginning to provide insight into the pathogenesis of the disorder. As a result we can expect to identify markers of the WHIM syndrome, as well as other disorders with WHIM-like features that are associated with dysfunctions of the CXCL12/CXCR4 axis.

  12. Effect of intestinal function-recovering decoction on treatment of multiple organ dysfunction syndrome in rats

    Institute of Scientific and Technical Information of China (English)

    Shu-Jie Zhao; Dong Zhang; Shi-Ji Wang; Ying Chen; Jin-Feng Han; Yu-Shan Wang

    2013-01-01

    Objective:To analyze the effect of intestinal function-recovering decoction on multiple organ dysfunction syndrome in rats, and to investigate a novel solution to multiple organ dysfunction syndrome.Methods:Multiple organ dysfunction syndrome was induced in60Sprague-Dawley rats by intestinal ischemia-reperfusion combined with cecal ligation and puncture.Then these rats were intragastrically administered physiological saline(groupⅠ,n=20), ampicillin (groupⅡ,n=20) or intestinal function-recovering decoction(groupⅢ,n=20).After treatment, serum malondialdehyde and superoxide dismutase levels were compared among three groups. Simultaneously, bacterial culture of various organ tissues was performed and bacterial and endotoxin translocation were observed.Results:Compared with groupI, serum malondialdehyde, alanine aminotransferase and aspartate aminotransferase levels were significantly decreased(all P0.05). The rate of bacterial translocation in the groupsⅡ andⅢ was significantly lower than in the groupⅠ(P0.05). Conclusions:Intestinal function-recovering decoction can significantly reduce endotoxin and bacterial translocation and stabilize enteral oxidative-antioxidative balance.

  13. Association between the glomerular filtration rate of renal dysfunction and metabolic syndrome: an age-stratified analysis

    Institute of Scientific and Technical Information of China (English)

    宋慧

    2014-01-01

    Objective To explore the relationship between the renal dysfunction rate and metabolic syndrome(MS),stratified by age.Methods People took part in physical check-up in a certain tertiary hospital from March 2010to September 2012,were enrolled in this study.Estimated glomerular filtration rate(e GFR),—a renal dysfunction indicator,was calculated by modified MDRD

  14. Management of the Difficult Paediatric Airway with a Simple Fiberoptic-Assisted Laryngoscope: A Report of Two Cases with Pierre Robin and Patau’s (Trisomy 13) Syndrome

    OpenAIRE

    Kılıçaslan, Alper; Erol, Atilla; Topal, Ahmet; Et, Tayfun; Otelcioğlu, Şeref

    2014-01-01

    Airway management of children with congenital craniofacial anomalies is a challenge for paediatric anaesthesiologists. We do not have any video-assisted airway device in our department for difficult paediatric intubations. We decided to attach a regular fiberoptic (outer diameter; 3.7 mm, Karl Storz, Germany) scope to a conventional Macintosh Laryngoscope (size 1). We describe two cases of Pierre Robin and Patau’s (Trisomy 13) syndrome successfully intubated with a fiberoptic-assisted laryngo...

  15. Characterisation of Brachycephalic Obstructive Airway Syndrome in French Bulldogs Using Whole-Body Barometric Plethysmography.

    Directory of Open Access Journals (Sweden)

    Nai-Chieh Liu

    Full Text Available Brachycephalic obstructive airway syndrome (BOAS is an important health and welfare problem in several popular dog breeds. Whole-body barometric plethysmography (WBBP is a non-invasive method that allows safe and repeated quantitative measurements of respiratory cycles on unsedated dogs. Here respiratory flow traces in French bulldogs from the pet population were characterised using WBBP, and a computational application was developed to recognise affected animals. Eighty-nine French bulldogs and twenty non-brachycephalic controls underwent WBBP testing. A respiratory functional grading system was used on each dog based on respiratory signs (i.e. respiratory noise, effort, etc. before and after exercise. For development of an objective BOAS classifier, functional Grades 0 and I were considered to have insignificant clinical signs (termed here BOAS- and Grades II and III to have significant signs (termed here BOAS+. A comparison between owner-perception of BOAS and functional grading revealed that 60 % of owners failed to recognise BOAS in dogs that graded BOAS+ in this study.WBBP flow traces were found to be significantly different between non-brachycephalic controls and Grade 0 French bulldogs; BOAS- and BOAS+ French bulldogs. A classifier was developed using quadratic discriminant analysis of the respiratory parameters to distinguish BOAS- and BOAS + French bulldogs, and a BOAS Index was calculated for each dog. A cut-off value of the BOAS Index was selected based on a receiver operating characteristic (ROC curve. Sensitivity, specificity, positive predictive value, and negative predictive value of the classifier on the training group (n=69 were 0.97, 0.93, 0.95, and 0.97, respectively. The classifier was validated using a test group of French bulldogs (n=20 with an accuracy of 0.95. WBBP offers objective screening for the diagnosis of BOAS in French Bulldogs. The technique may be applied to other brachycephalic breeds affected by BOAS, and

  16. Upper Airway Resistance Syndrome Patients Have Worse Sleep Quality Compared to Mild Obstructive Sleep Apnea.

    Directory of Open Access Journals (Sweden)

    Luciana Balester Mello de Godoy

    Full Text Available To compare sleep quality and sustained attention of patients with Upper Airway Resistance Syndrome (UARS, mild Obstructive Sleep Apnea (OSA and normal individuals.UARS criteria were presence of excessive daytime sleepiness (Epworth Sleepiness Scale-ESS-≥ 10 and/or fatigue (Modified Fatigue Impact Scale-MFIS-≥ 38 associated to Apnea/hypopnea index (AHI ≤ 5 and Respiratory Disturbance Index (RDI > 5 events/hour of sleep or more than 30% of total sleep time with flow limitation. Mild OSA was considered if the presence of excessive daytime sleepiness (ESS ≥ 10 and/or fatigue (MFIS ≥ 38 associated to AHI ≥ 5 and ≤ 15 events/hour. "Control group" criteria were AHI < 5 events/hour and RDI ≤ 5 events/hour and ESS ≤ 9, without any sleep, clinical, neurological or psychiatric disorder. 115 individuals (34 UARS and 47 mild OSA patients and 34 individuals in "control group", adjusted for age, gender, body mass index (BMI and schooling years, performed sleep questionnaires and sustained attention evaluation. Psychomotor Vigilance Task (PVT was performed five times (each two hours from 8 a.m. to 4 p.m.UARS patients had worse sleep quality (Functional Outcomes of Sleep Questionnaire-FOSQ-and Pittsburgh Sleep Quality Index-PSQI: p < 0.05 and more fatigue than mild OSA patients (p = 0.003 and scored significantly higher in both Beck inventories than "control group" (p < 0.02. UARS patients had more lapses early in the morning (in time 1 compared to the results in the afternoon (time 5 than mild OSA (p = 0.02. Mild OSA patients had more lapses in times 2 than in time 5 compared to "control group" (p = 0.04.UARS patients have a worse sleep quality, more fatigue and a worse early morning sustained attention compared to mild OSA. These last had a worse sustained attention than controls.

  17. Renal Dysfunction, Metabolic Syndrome and Cardiovascular Disease Mortality

    Directory of Open Access Journals (Sweden)

    David Martins

    2010-01-01

    Full Text Available Background. Renal disease is commonly described as a complication of metabolic syndrome (MetS but some recent studies suggest that Chronic Kidney disease (CKD may actually antecede MetS. Few studies have explored the predictive utility of co-clustering CKD with MetS for cardiovascular disease (CVD mortality. Methods. Data from a nationally representative sample of United States adults (NHANES was utilized. A sample of 13115 non-pregnant individuals aged ≥35 years, with available follow-up mortality assessment was selected. Multivariable Cox Proportional hazard regression analysis techniques explored the relationship between co-clustered CKD, MetS and CVD mortality. Bayesian analysis techniques tested the predictive accuracy for CVD Mortality of two models using co-clustered MetS and CKD and MetS alone. Results. Co-clustering early and late CKD respectively resulted in statistically significant higher hazard for CVD mortality (HR = 1.80, CI = 1.45–2.23, and HR = 3.23, CI = 2.56–3.70 when compared with individuals with no MetS and no CKD. A model with early CKD and MetS has a higher predictive accuracy (72.0% versus 67.6%, area under the ROC (0.74 versus 0.66, and Cohen's kappa (0.38 versus 0.21 than that with MetS alone. Conclusion. The study findings suggest that the co-clustering of early CKD with MetS increases the accuracy of risk prediction for CVD mortality.

  18. Clinical application ultrafast MRI to the sleep apnea syndrome, 1; Evaluation of the site of obstruction within upper airway

    Energy Technology Data Exchange (ETDEWEB)

    Suto, Yuji; Nakamura, Kiyoshi; Kato, Terumi (Tottori Univ., Yonago (Japan). School of Medicine) (and others)

    1992-07-01

    To evaluate the site of obstruction within upper airway, we observed the Turbo-fast low angle shot (FLASH) imaging, in 10 patients with sleep apnea syndrome (SAS) during wakefulness and sleep. After intravenous injection of Gd-DTPA (0.1 mmol/kg), sequential images of pharyngeal portion were obtained in midline sagittal section. An imaging protocol was 1.13s per image with a 1s delay between images, for a total of 30s. Then sequential images were displayed in a cine on C. R. T.. In eight patients, upper airway obstructions were present during sleep, while narrowings were present in four cases during awake. The sites of obstruction were located at the velopharynx exclusively in three cases, velopharynx plus glosspharynx in three cases, velopharynx plus glosspharynx in one case. Velopharynx plus hypopharynx in one case, respectively. It was concluded that ultrafast MRI had an important role in evaluating the sites of obstruction within upper airway in patients with SAS. (author).

  19. Percutaneous dilational tracheotomy for airway management in a newborn with Pierre-Robin syndrome and a glossopharyngeal web.

    Science.gov (United States)

    Pirat, Arash; Candan, Selim; Unlükaplan, Aytekin; Kömürcü, Ozgür; Kuşlu, Selim; Arslan, Gülnaz

    2012-04-01

    Pierre-Robin syndrome (PRS) is often associated with difficulty in endotracheal intubation. We present the use of percutaneous dilational tracheotomy (PDT) for airway management of a newborn with PRS and a glossopharyngeal web. A 2-day-old term newborn with PRS and severe obstructive dyspnea was evaluated by the anesthesiology team for airway management. A direct laryngoscopy revealed a glossopharyngeal web extending from the base of the tongue to the posterior pharyngeal wall. The infant was spontaneously breathing through a 2 mm diameter fistula in the center of this web. It was decided that endotracheal intubation was impossible, and a PDT was planned. The trachea of the newborn was cannulated, using a 20 gauge peripheral venous catheter and a 0.71 mm guide wire was introduced through this catheter. Using 5 French, 7 French, 9 French, and 11 French central venous catheter kit dilators, staged tracheotomy stoma dilation was performed. By inserting a size 3.0 tracheotomy cannula, PDT was successfully completed in this newborn. This case describes the successful use of PDT for emergency airway management of a newborn with PRS and glossopharyngeal web.

  20. Dysfunction in the hip joints in children with Charcot-Marie-Tooth syndrome (literature review

    Directory of Open Access Journals (Sweden)

    Иван Юрьевич Поздникин

    2015-09-01

    Full Text Available A review of the literature on the treatment of children with dysfunction in the hip joints in motor-sensory neuropathy Charcot-Marie-Tooth is presented. Peculiarities of disease diagnosis and the approach used in the treatment of patients are described. The Charcot-Marie-Tooth syndrome is a hereditary neuromuscular disease characterized by progressive atrophy of the distal muscle group of the lower limbs. According to international authors, the incidence of hip joint dysfunction in this condition is at least 10%, ranking second only to foot deformities. In the Russian literature, the problem has not been adequately interpreted. Early diagnosis of dysfunction in the hip joints during Charcot-Marie-Tooth syndrome is complicated by the child's age and is characterized by progression. Conflicting clinical signs and trivial symptoms of the disease also confuse diagnosis, until it becomes clearer in adolescence or the second or third decade of life. Surgical reconstructive operations on the hip joint often occur too late, and they are accompanied by a greater frequency of neurological complications. Practitioner awareness coupled with an early diagnosis of hip subluxation and decentration and complex orthopedic and neurological examinations of children with the disease of Charcot-Marie-Tooth should result in more favorable outcomes.

  1. RPGR-containing protein complexes in syndromic and non-syndromic retinal degeneration due to ciliary dysfunction

    Indian Academy of Sciences (India)

    Carlos A. Murga-Zamalloa; Anand Swaroop; Hemant Khanna

    2009-12-01

    Dysfunction of primary cilia due to mutations in cilia-centrosomal proteins is associated with pleiotropic disorders. The primary (or sensory) cilium of photoreceptors mediates polarized trafficking of proteins for efficient phototransduction. Retinitis pigmentosa GTPase regulator (RPGR) is a cilia-centrosomal protein mutated in >70% of X-linked RP cases and 10%–20% of simplex RP males. Accumulating evidence indicates that RPGR may facilitate the orchestration of multiple ciliary protein complexes. Disruption of these complexes due to mutations in component proteins is an underlying cause of associated photoreceptor degeneration. Here, we highlight the recent developments in understanding the mechanism of cilia-dependent photoreceptor degeneration due to mutations in RPGR and RPGR-interacting proteins in severe genetic diseases, including retinitis pigmentosa, Leber congenital amaurosis (LCA), Joubert syndrome, and Senior–Loken syndrome, and explore the physiological relevance of photoreceptor ciliary protein complexes.

  2. Possible association of thymus dysfunction with fading syndromes in puppies and kittens.

    Science.gov (United States)

    Roth, J A

    1987-05-01

    thymique serique, and rabbit thymus acetone powder) and bovine growth hormone are commercially available. Before initiating therapy, one should consider that if the cause of the wasting syndrome is genetic, then successful treatment may perpetuate a genetic defect. More research (both basic and clinical) is needed to determine the role of thymus gland dysfunction in fading syndromes of puppies and kittens and if therapy with one or several of the thymus hormones or with growth hormone could reverse the symptoms of wasting. PMID:3496704

  3. Upper Airway Resistance Syndrome Patients Have Worse Sleep Quality Compared to Mild Obstructive Sleep Apnea

    Science.gov (United States)

    de Godoy, Luciana Balester Mello; Luz, Gabriela Pontes; Palombini, Luciana Oliveira; e Silva, Luciana Oliveira; Hoshino, Wilson; Guimarães, Thaís Moura; Tufik, Sergio; Bittencourt, Lia; Togeiro, Sonia Maria

    2016-01-01

    Purpose To compare sleep quality and sustained attention of patients with Upper Airway Resistance Syndrome (UARS), mild Obstructive Sleep Apnea (OSA) and normal individuals. Methods UARS criteria were presence of excessive daytime sleepiness (Epworth Sleepiness Scale—ESS—≥ 10) and/or fatigue (Modified Fatigue Impact Scale—MFIS—≥ 38) associated to Apnea/hypopnea index (AHI) ≤ 5 and Respiratory Disturbance Index (RDI) > 5 events/hour of sleep or more than 30% of total sleep time with flow limitation. Mild OSA was considered if the presence of excessive daytime sleepiness (ESS ≥ 10) and/or fatigue (MFIS ≥ 38) associated to AHI ≥ 5 and ≤ 15 events/hour. “Control group” criteria were AHI < 5 events/hour and RDI ≤ 5 events/hour and ESS ≤ 9, without any sleep, clinical, neurological or psychiatric disorder. 115 individuals (34 UARS and 47 mild OSA patients and 34 individuals in “control group”), adjusted for age, gender, body mass index (BMI) and schooling years, performed sleep questionnaires and sustained attention evaluation. Psychomotor Vigilance Task (PVT) was performed five times (each two hours) from 8 a.m. to 4 p.m. Results UARS patients had worse sleep quality (Functional Outcomes of Sleep Questionnaire—FOSQ—and Pittsburgh Sleep Quality Index—PSQI: p < 0.05) and more fatigue than mild OSA patients (p = 0.003) and scored significantly higher in both Beck inventories than “control group” (p < 0.02). UARS patients had more lapses early in the morning (in time 1) compared to the results in the afternoon (time 5) than mild OSA (p = 0.02). Mild OSA patients had more lapses in times 2 than in time 5 compared to “control group” (p = 0.04). Conclusions UARS patients have a worse sleep quality, more fatigue and a worse early morning sustained attention compared to mild OSA. These last had a worse sustained attention than controls. PMID:27228081

  4. Meibomian gland dysfunction in a case of ichthyosis follicularis with alopecia and photophobia syndrome

    Directory of Open Access Journals (Sweden)

    Tarannum Fatima

    2014-01-01

    Full Text Available We are reporting an interesting case of ichthyosis follicularis with alopecia and photophobia syndrome in a daughter and father from the Indian subcontinent associated with Meibomian gland dysfunction. A three-year-old female child presented with pain, redness and severe photophobia in both eyes since one month. Cutaneous examination revealed ichthyosis, absence of hair all over the body including eyebrows, scalp and angular cheilosis. Ocular examination revealed bilateral severe meibomianitis, multiple superficial punctate keratitides in right eye and corneal epithelial defect in the left eye. Other systemic features were prominent high forehead and large ears. Her father had similar symptoms. Her symptoms improved after adequate treatment of meibomian gland dysfunction. She is asymptomatic at present.

  5. Interleukin-1α drives the dysfunctional cross-talk of the airway epithelium and lung fibroblasts in COPD.

    Science.gov (United States)

    Osei, Emmanuel T; Noordhoek, Jacobien A; Hackett, Tillie L; Spanjer, Anita I R; Postma, Dirkje S; Timens, Wim; Brandsma, Corry-Anke; Heijink, Irene H

    2016-08-01

    Chronic obstructive pulmonary disease (COPD) has been associated with aberrant epithelial-mesenchymal interactions resulting in inflammatory and remodelling processes. We developed a co-culture model using COPD and control-derived airway epithelial cells (AECs) and lung fibroblasts to understand the mediators that are involved in remodelling and inflammation in COPD.AECs and fibroblasts obtained from COPD and control lung tissue were grown in co-culture with fetal lung fibroblast or human bronchial epithelial cell lines. mRNA and protein expression of inflammatory mediators, pro-fibrotic molecules and extracellular matrix (ECM) proteins were assessed.Co-culture resulted in the release of pro-inflammatory mediators interleukin (IL)-8/CXCL8 and heat shock protein (Hsp70) from lung fibroblasts, and decreased expression of ECM molecules (e.g. collagen, decorin) that was not different between control and COPD-derived primary cells. This pro-inflammatory effect was mediated by epithelial-derived IL-1α and increased upon epithelial exposure to cigarette smoke extract (CSE). When exposed to CSE, COPD-derived AECs elicited a stronger IL-1α response compared with control-derived airway epithelium and this corresponded with a significantly enhanced IL-8 release from lung fibroblasts.We demonstrate that, through IL-1α production, AECs induce a pro-inflammatory lung fibroblast phenotype that is further enhanced with CSE exposure in COPD, suggesting an aberrant epithelial-fibroblast interaction in COPD. PMID:27418555

  6. Dynamic imaging assessment on the upper airway in patients with obstructive sleep apnea hypopnea syndrome%OSAHS的上气道动态影像学评估

    Institute of Scientific and Technical Information of China (English)

    闫智强; 孙建军

    2012-01-01

    Dynamic imaging assessment on the upper airway in patients with obstructive sleep apnea hypopnea syndrome Summary It is vital to make an individual plan for each patient with obstructive sleep apnea-hypopnea syn-drome(OSAHS) according to the obstruction sites. The high resolution anatomical information of upper airway and soft tissue can be obtained, especially by MRI and CT scans. Dynamic and state-dependent imaging techniques are beneficial to study stereo changes of anatomy and morphology of upper airway in quiet breathing, sleeping or airway closure. Although dynamic imaging examination has value in diagnosis and treatment of OSAHS, there has no uniform position diagnosis standard. This article reviews the history of dynamic imaging study on OSAHS, the advantages and disadvantages of various imaging technologies and prospects of imaging position diagnosis.

  7. Overlapping gastroesophageal reflux disease and irritable bowel syndrome:Increased dysfunctional symptoms

    Institute of Scientific and Technical Information of China (English)

    Shadi; Sadeghi; Yarandi; Siavosh; Nasseri-Moghaddam; Pardis; Mostajabi; Reza; Malekzadeh

    2010-01-01

    AIM:To investigate the association of gastroesophageal reflux disease (GERD) and irritable bowel syndrome (IBS) in Iranian patients and examine the prevalence of functional symptoms of the gastrointestinal tract in patients presenting with either IBS, GERD or both.METHODS: Six thousand four hundred and seventy six patients presented to the Gastro-intestinal (GI) clinic with symptoms of functional dysfunction of GI tract, 1419 patients (62.0% women, 38.0% men; mean age: 37.4±11.5 years) met Rome or Rome crit...

  8. Magnetic resonance imaging correlates of bee sting induced multiple organ dysfunction syndrome: A case report

    Institute of Scientific and Technical Information of China (English)

    Sushant; K; Das; Li-Chuan; Zeng; Bing; Li; Xiang-Ke; Niu; Jing-Liang; Wang; Anup; Bhetuwal; Han-Feng; Yang

    2014-01-01

    Occasionally systemic complications with high risk of death,such as multiple organ dysfunction syndrome(MODS),can occur following multiple bee stings.This case study reports a patient who presented with MODS,i.e.,acute kidney injury,hepatic and cardiac dysfunc-tion,after multiple bee stings.The standard clinical findings were then correlated with magnetic resonance imaging(MRI)findings,which demonstrates that MRI may be utilized as a simpler tool to use than other mul-tiple diagnostics.

  9. Cognitive dysfunction syndrome: a disease of canine and feline brain aging.

    Science.gov (United States)

    Landsberg, Gary M; Nichol, Jeff; Araujo, Joseph A

    2012-07-01

    Brain aging is a degenerative process manifest by impairment of cognitive function; although not all pets are affected at the same level, once cognitive decline begins it is generally a progressive disorder. Diagnosis of cognitive dysfunction syndrome (CDS) is based on recognition of behavioral signs and exclusion of other medical causes that might mimic CDS or complicate its diagnosis. Drugs, diets, and supplements are now available that might slow CDS progression by various mechanisms including reducing oxidative stress and inflammation or improving mitochondrial and neuronal function. Moreover, available therapeutics may provide some level of improvement in cognitive and clinical signs of CDS. PMID:22720812

  10. Sensory Dysfunction of Bladder Mucosa and Bladder Oversensitivity in a Rat Model of Metabolic Syndrome

    OpenAIRE

    Wei-Chia Lee; Po-Hui Chiang; You-Lin Tain; Chia-Ching Wu; Yao-Chi Chuang

    2012-01-01

    PURPOSE: To study the role of sensory dysfunction of bladder mucosa in bladder oversensitivity of rats with metabolic syndrome. MATERIALS AND METHODS: Female Wistar rats were fed a fructose-rich diet (60%) or a normal diet for 3 months. Based on cystometry, the fructose-fed rats (FFRs) were divided into a group with normal detrusor function or detrusor overactivity (DO). Acidic adenosine triphosphate (ATP) solution (5mM, pH 3.3) was used to elicit reflex micturition. Cystometric parameters we...

  11. Endothelial dysfunction in normal and prediabetic rats with metabolic syndrome exposed by oral gavage to carbon black nanoparticles

    DEFF Research Database (Denmark)

    Folkmann, Janne Kjærsgaard; Vesterdal, Lise Kristine; Sheykhzade, Majid;

    2012-01-01

    Exposure to nanosized particles may increase the risk of cardiovascular diseases by endothelial dysfunction, particularly in susceptible subjects with metabolic syndrome. We investigated vasomotor dysfunction in aorta from obese and lean Zucker rats after oral exposure to nanosized carbon black (CB......-induced vasorelaxation and phenylephrine-induced vasocontraction was not affected in rats exposed to CB. The endothelial dysfunction was not observed in rats sacrificed 13 weeks after the last CB exposure. There was unaltered expression of Chrm3, Nos3, Nos2, Ccl2, and Hmox1 in aorta tissue of CB-exposed rats. In...... conclusion, repeated oral exposure to CB was associated with endothelial dysfunction in rats, further aggravating the effect of metabolic syndrome....

  12. Posture-dependent aphasia: Focal cortical dysfunction in the sinking scalp flap syndrome

    Directory of Open Access Journals (Sweden)

    Prasad Krishnan

    2015-01-01

    Full Text Available Decompressive craniotomies are being increasingly used in the treatment of raised intracranial pressure due to a variety of reasons like large infarcts, hypertensive hemorrhages and contusions. Though effective in decreasing raised intracranial pressure, they have certain complications like the sinking scalp flap syndrome that is caused by cortical dysfunction of the area below the craniotomy which is exposed to the effects of atmospheric pressure. We describe a 60-year-old patient who underwent decompressive craniotomy for acute subdural hematoma and after an initial uneventful postoperative period developed incontinence, irrelevant verbalization and ataxia. He was found to have hydrocephalus and underwent a ventriculo-peritoneal shunt with resolution of his symptoms. Three weeks later his flap had sunk in deeply and the skin was non-pinchable and he was noted to have headaches, vomiting and retching when he sat up. In addition he became aphasic when seated and the symptoms subsided on lying down. A diagnosis of focal cortical dysfunction due to sinking scalp flap syndrome was made. We highlight the incidence and pathophysiology of this unusual complication of decompressive craniotomy and stress the need to be aware of this entity particularly in patients who do not show an initial improvement after decompressive craniotomy as the cause of their poor neurological status may not be explained by any other mechanism.

  13. Animal model of non-bacterial multiple organ dysfunction syndrome in the elderly

    Institute of Scientific and Technical Information of China (English)

    Qinglei ZHU; Shiwen WANG; Jie YANG; Tong YIN; Xiaoshun QIAN; Qiao XUE; Bin XU

    2004-01-01

    Objective To establish a model of multiple organ dysfunction syndrome in the elderly (MODSE) by intraperitoneal injection of different doses of zymosan, and to compare the multiple organ dysfunction syndrome (MODS) in adult and in the elderly rats. Methods Adult and senile rats, injected with different doses of zymosan intraperitoneally were examined for the changes in the function and morphology of the vital organs, including heart, liver, brain, lungs, and kidneys using blood gas and biochemistry analysis and histopathological examination methods. Results Compared with the normal controls of the adult and the elderly rats, the blood gas and blood biochemistry changed in different degrees in the different dosed zymosan groups. Pathological changes were also found in the vital organs including lungs, heart, liver, brain, kidneys, erc in the experimental groups. Under the same concentrations of zymosan, the reductions in respiratory, cardiac and renal functions in the senile groups were much more severe than those in the corresponding adult group. In the similar degree of model duplication, the senile rats had the tendency to die later than the adult rats. Conclusions Zymosan can be used in both elderly and adult rats to induce MODS model, and the best dosage for MODSE was 0.Sg/kg injected peritoneally. The model would hopefully be used in the study of mechanisms and the therapeutics on MODSE.

  14. [Autonomic dysfunction syndrome and diabetic cardiac autonomic neuropathy in children with diabetes mellitus type I. The correction method].

    Science.gov (United States)

    Manukian, V Iu; Bolotova, N V; Aver'ianov, A P; Filina, N Iu; Raĭgorodskiĭ, Iu M

    2011-01-01

    We assessed the state of the autonomic nervous system in 90 children with diabetes mellitus type I. The autonomic dysfunction syndrome was found in 58,9% and diabetic cardiac autonomic neuropathy in 28,9% of patients. We revealed the high risk of the development of diabetic cardiac autonomic neuropathy in children with diabetes mellitus type I in the presence of the autonomic dysfunction syndrome. It has been shown that the early treatment of functional disturbances of the autonomic nervous system using transcranial magnetic stimulation is necessary to prevent the manifestation of diabetic cardiac autonomic neuropathy.

  15. Extension of the mitochondria dysfunction hypothesis of metabolic syndrome to atherosclerosis with emphasis on the endocrine‐disrupting chemicals and biophysical laws

    OpenAIRE

    Lee, Hong Kyu; Shim, Eun Bo

    2013-01-01

    Abstract Metabolic syndrome and its component phenotypes, hyperglycemia, hypertension, (abdominal) obesity and hypertriglyceridemia, are major risk factors for atherosclerosis. Recently, associations between exposure to endocrine‐disrupting chemicals (EDCs), mitochondrial dysfunction, metabolic syndrome and atherosclerosis have been established, suggesting a possible common mechanism underlying these phenomena. Extending a previously proposed mitochondria dysfunction theory of metabolic syndr...

  16. Effect of continuous positive airway pressure ventilation on prethrombotic state in patients with obstructive sleep apnea-hypopnea syndrome

    International Nuclear Information System (INIS)

    To investigate the prethrombotic state (PTS) in patients with obstructive sleep apnea syndrome (OS-AS) and the effect of continuous positive airway pressure ventilation (CPAP) on their PTS, the blood samples of 49 patients with OSAS were taken before treatment and on day 30 after treatment respectively. The platelet aggregation ( PAG), P-selections, endothdlin-1 (ET-1) and plasma vom willebrand factor (vWF) in 49 patients and 42 health controls were detected by radioimmunoassay and enzyme-immunoassay. The results showed that the PAG, P-selections, ET-1 and vWF in patients with OSAS before treatment were significantly higher than those after treatment and in control group (P0.05). The results indicate that there were PTS in most patients with OSAS before treatment. The activity of platelet could be corrected, and the function of endotheliocyte could be repaired after CPAP treatment. It had certain effect in lightening the clinical symptoms. (authors)

  17. Bezafibrate improves postprandial hypertriglyceridemia and associated endothelial dysfunction in patients with metabolic syndrome: a randomized crossover study

    OpenAIRE

    Ohno, Yuko; Miyoshi, Toru; NODA, YOKO; Oe, Hiroki; Toh, Norihisa; Nakamura, Kazufumi; Kohno, Kunihisa; Morita, Hiroshi; Ito, Hiroshi

    2014-01-01

    Background Postprandial elevation of triglyceride-rich lipoproteins impairs endothelial function, which can initiate atherosclerosis. We investigated the effects of bezafibrate on postprandial endothelial dysfunction and lipid profiles in patients with metabolic syndrome. Methods Ten patients with metabolic syndrome were treated with 400 mg/day bezafibrate or untreated for 4 weeks in a randomized crossover study. Brachial artery flow-mediated dilation (FMD) and lipid profiles were assessed du...

  18. Genetic Rodent Models of Obesity-Associated Ovarian Dysfunction and Subfertility: Insights into Polycystic Ovary Syndrome

    Science.gov (United States)

    Huang-Doran, Isabel; Franks, Stephen

    2016-01-01

    Polycystic ovary syndrome (PCOS) is the most common endocrinopathy affecting women and a leading cause of female infertility worldwide. Defined clinically by the presence of hyperandrogenemia and oligomenorrhoea, PCOS represents a state of hormonal dysregulation, disrupted ovarian follicle dynamics, and subsequent oligo- or anovulation. The syndrome’s prevalence is attributed, at least partly, to a well-established association with obesity and insulin resistance (IR). Indeed, the presence of severe PCOS in human genetic obesity and IR syndromes supports a causal role for IR in the pathogenesis of PCOS. However, the molecular mechanisms underlying this causality, as well as the important role of hyperandrogenemia, remain poorly elucidated. As such, treatment of PCOS is necessarily empirical, focusing on symptom alleviation. The generation of knockout and transgenic rodent models of obesity and IR offers a promising platform in which to address mechanistic questions about reproductive dysfunction in the context of metabolic disease. Similarly, the impact of primary perturbations in rodent gonadotrophin or androgen signaling has been interrogated. However, the insights gained from such models have been limited by the relatively poor fidelity of rodent models to human PCOS. In this mini review, we evaluate the ovarian phenotypes associated with rodent models of obesity and IR, including the extent of endocrine disturbance, ovarian dysmorphology, and subfertility. We compare them to both human PCOS and other animal models of the syndrome (genetic and hormonal), explore reasons for their discordance, and consider the new opportunities that are emerging to better understand and treat this important condition. PMID:27375552

  19. Metabolic Syndrome, Hormone Levels, and Inflammation in Patients with Erectile Dysfunction

    Directory of Open Access Journals (Sweden)

    Miguel Ángel Arrabal-Polo

    2012-01-01

    Full Text Available Background. The end point of this study was to investigate the prevalence of MS in patients with ED in comparison with control subjects and to analyse the association with acute phase reactants (CRP, ESR and hormone levels. Methods. This case-control study included 65 patients, 37 with erectile dysfunction, according to the International Index of Erectile Function (IIEF from the Urology Department of San Cecilio University Hospital, Granada (Spain and 28 healthy controls. The prevalence of metabolic syndrome was calculated according to ATP-III criteria. Hormone levels and acute phase parameters were studied in samples drawn. Results. The ATP-III criteria for MS were met by 64.9% of the patients with ED and only 9.5% of the controls (P<0.0001, OR = 17.53, 95% CI: 3.52–87.37. Binary logistic regression analysis showed a strong association between patients with ED and MS, even after additional adjustment for confounding factors (OR = 20.05, 95% CI: 1.24–32.82, P<0.034. Patients with hypogonadism presented a significantly higher prevalence of metabolic syndrome. Multiple linear regression analysis showed that systolic BP and CRP predicted 0.46 (model R2 of IIEF changes. Conclusion. Chronic inflammation found in patients with ED might explain the association between ED and metabolic syndrome.

  20. Management of the Difficult Paediatric Airway with a Simple Fiberoptic-Assisted Laryngoscope: A Report of Two Cases with Pierre Robin and Patau’s (Trisomy 13) Syndrome

    Science.gov (United States)

    Kılıçaslan, Alper; Erol, Atilla; Topal, Ahmet; Et, Tayfun; Otelcioğlu, Şeref

    2014-01-01

    Airway management of children with congenital craniofacial anomalies is a challenge for paediatric anaesthesiologists. We do not have any video-assisted airway device in our department for difficult paediatric intubations. We decided to attach a regular fiberoptic (outer diameter; 3.7 mm, Karl Storz, Germany) scope to a conventional Macintosh Laryngoscope (size 1). We describe two cases of Pierre Robin and Patau’s (Trisomy 13) syndrome successfully intubated with a fiberoptic-assisted laryngoscope (FOL). A fiberoptic scope and any size of a laryngoscope blade can be easily assembled in the operating room. The FOL may be a useful device in the setting of difficult paediatric intubation. PMID:27366452

  1. Ultrastructural Changes of Airway in Murine Models of Allergy and Diet-Induced Metabolic Syndrome

    OpenAIRE

    Leishangthem, Geeta Devi; Mabalirajan, Ulaganathan; Singh, Vijay Pal; Agrawal, Anurag; Ghosh, Balaram; Dinda, Amit Kumar

    2013-01-01

    Studying ultrastructural changes could reveal novel pathophysiology of obese-asthmatic condition as existing concepts in asthma pathogenesis are based on the histological changes of the diseased airway. While asthma is defined in functional terms, the potential of electron microscopy (EM) in providing cellular and subcellular detail is underutilized. With this view, we have performed transmission EM in the lungs from allergic mice that show key features of asthma and high-fat- or high-fructos...

  2. Nosography of systemic inflammatory response syndrome, sepsis, severe sepsis, septic shock, and multiple organ dysfunction syndrome in internal medicine patients

    Directory of Open Access Journals (Sweden)

    Silvia Spoto

    2015-09-01

    Full Text Available Sepsis is defined by the presence of at least two systemic inflammatory response syndrome criteria associated with an infection microbiologically or clinically evidenced. In Italy sepsis is responsible for 80,000 hospital admissions per year and, in the last decades, severe sepsis and septic shock cases are increasing, in correlation with the increased prevalence of multi-drugresistant microbial strains. The predominant etiologic agents are Gram-positive and Gram-negative bacteria, but sepsis caused by fungi is increasing. The host response with both inflammatory and anti-inflammatory processes is responsible for organic failures, which complicate the syndrome, and for the susceptibility to secondary infections. The impairment of one or more organs or systems may be the onset clinical presentation. The organ dysfunctions complicating sepsis involve mainly cardiorespiratory system, kidneys, hemostatis and central nervous system. Fever or hypothermia, tachycardia, tachypnea, leukocytosis or leukopenia, elevated blood levels of lactate and procalcitonin, hypotension are diagnostically sensitive findings for sepsis. Definitive diagnosis requires isolation of the pathogen from blood sample or from the focus of infection. Therapeutic success against sepsis depends on the appropriate use of antibiotics, on the treatment of hemodynamic and respiratory disorder and on general supportive care. In some cases the use of activated protein C is to take in consideration.

  3. Motor dysfunction in complex regional pain syndrome : the role of sensory processing and sensory-motor integration

    NARCIS (Netherlands)

    Bank, Paulina Johanna Maria

    2014-01-01

    In the chronic stage of Complex Regional Pain Syndrome (CRPS), motor disturbances are common and cause significant disability. The motor dysfunction of CRPS is a poorly understood phenomenon that is characterized predominantly by a decrease or loss of voluntary muscle control. This thesis aims to ob

  4. A Case of Montelukast-Induced Churg-Strauss Syndrome Associated with Liver Dysfunction

    Directory of Open Access Journals (Sweden)

    Keiji Matsui

    2011-01-01

    Full Text Available A 64-year-old woman was admitted to hospital due to protracted diarrhea and liver dysfunction. The patient was diagnosed as Churg-Strauss syndrome (CSS due to asthma, paranasal sinusitis, hypereosinophilia, and polyneuropathy. There was a history of taking montelukast, a leukotriene receptor antagonist (LTRA, which is thought to have some relationship with CSS. The liver biopsy specimen showed eosinophilic infiltration and centrolobular fatty change. In this paper, we review the relationship between LTRA and CSS. Several lines of evidence suggest that leukotriene plays an important role in maintaining neural tissues. We also review the potential relationship between centrolobular fatty change and pivoxil-containing antibiotics, which was prescribed for sinusitis before admission. Carnitine deficiency induced by pivoxil-containing agents may cause impaired fatty acid oxidation in mitochondria.

  5. Physiotherapy in diagnosis and treatment of the myofascial pain dysfunction syndrome.

    Science.gov (United States)

    Trott, P H; Goss, A N

    1978-08-01

    Thirty-four patients with the Myofascial Pain Dysfunction (M.P.D.) syndrome of the masticatory system were investigated and treated using physiotherapy techniques. Resisted static contraction of the temporomandibular joints, passive movement tests of the muscles and electromyography indicated that the joint function was abnormal in all cases with minimal muscle involvement. Cervical spine tests indicated that pain referral from the cervical spine was involved in 19 of 34 patients (56%). Physiotherapy treatment armed at restoring a normal painless range of movement to the temporomandibular joint was successful in six of 10 patients (60%). Generalized relaxation therapy with biofeedback was successful in 19 of 24 patients (80%). It was found that with the five of 24 patients (20%) in whom generalized relaxation failed, there were significant psychiatric factors.

  6. Right hemisphere dysfunction and metaphor comprehension in young adults with Asperger syndrome.

    Science.gov (United States)

    Gold, Rinat; Faust, Miriam

    2010-07-01

    This study examined whether the known difficulties in metaphor comprehension exhibited by persons with Asperger syndrome (AS) can be explained by a dysfunctional right hemisphere (RH). Using the divided visual field paradigm, 27 AS participants and 36 matched controls were presented with word pairs of four types (literal, conventional metaphors, novel metaphors, and unrelated word pairs), and were asked to perform a semantic judgment task. The main hypothesis was that whereas the control group participants will show RH superiority for novel metaphor processing, no RH superiority will be found in the AS group. Results indeed indicate much less RH contribution to novel metaphor comprehension in AS, and are discussed in light of linguistic models and the neurobiology of autism.

  7. Weight loss is associated with improved endothelial dysfunction via NOX2-generated oxidative stress down-regulation in patients with the metabolic syndrome.

    Science.gov (United States)

    Angelico, Francesco; Loffredo, Lorenzo; Pignatelli, Pasquale; Augelletti, Teresa; Carnevale, Roberto; Pacella, Antonio; Albanese, Fabiana; Mancini, Ilaria; Di Santo, Serena; Del Ben, Maria; Violi, Francesco

    2012-06-01

    The aim of this study was to assess whether adherence to a restricted-calorie, Mediterranean-type diet improves endothelial dysfunction and markers of oxidative stress in patients with metabolic syndrome. A moderately low-calorie (600 calories/day negative energy balance), low-fat, high-carbohydrate diet (syndrome. Participants were divided into two groups according to body weight loss > or syndrome. The coexistent decrease of NOX2 activation suggests a role for oxidative stress in eliciting artery dysfunction.

  8. Sensory dysfunction of bladder mucosa and bladder oversensitivity in a rat model of metabolic syndrome.

    Directory of Open Access Journals (Sweden)

    Wei-Chia Lee

    Full Text Available PURPOSE: To study the role of sensory dysfunction of bladder mucosa in bladder oversensitivity of rats with metabolic syndrome. MATERIALS AND METHODS: Female Wistar rats were fed a fructose-rich diet (60% or a normal diet for 3 months. Based on cystometry, the fructose-fed rats (FFRs were divided into a group with normal detrusor function or detrusor overactivity (DO. Acidic adenosine triphosphate (ATP solution (5mM, pH 3.3 was used to elicit reflex micturition. Cystometric parameters were evaluated before and after drug administration. Functional proteins of the bladder mucosa were assessed by western blotting. RESULTS: Compared to the controls, intravesical acidic ATP solution instillation induced a significant increase in provoked phasic contractions in both FFR groups and a significant decrease in the mean functional bladder capacity of group DO. Pretreatment with capsaicin for C-fiber desentization, intravesical liposome for mucosal protection, or intravenous pyridoxal 5-phosphate 6-azophenyl-2',4'-disulfonic acid for antagonized purinergic receptors can interfere with the urodynamic effects of intravesical ATP in FFRs and controls. Over-expression of TRPV1, P2X(3, and iNOS proteins, and down-regulation of eNOS proteins were observed in the bladder mucosa of both fructose-fed groups. CONCLUSIONS: Alterations of sensory receptors and enzymes in the bladder mucosa, including over-expression of TRPV1, P2X(3, and iNOS proteins, can precipitate the emergence of bladder phasic contractions and oversensitivity through the activation of C-afferents during acidic ATP solution stimulation in FFRs. The down-regulation of eNOS protein in the bladder mucosa of FFRs may lead to a failure to suppress bladder oversensitivity and phasic contractions. Sensory dysfunction of bladder mucosa and DO causing by metabolic syndrome are easier to elicit bladder oversensitivity to certain urothelium stimuli.

  9. The FSH-inhibin axis in prader-willi syndrome: heterogeneity of gonadal dysfunction

    Directory of Open Access Journals (Sweden)

    Gross-Tsur Varda

    2012-05-01

    Full Text Available Abstract Background We characterized the spectrum and etiology of hypogonadism in a cohort of Prader-Willi syndrome (PWS adolescents and adults. Methods Reproductive hormonal profiles and physical examination were performed on 19 males and 16 females ages 16–34 years with PWS. Gonadotropins, sex-steroids, inhibin B (INB and anti-Mullerian hormone (AMH were measured. We defined 4 groups according to the relative contribution of central and gonadal dysfunction based on FSH and INB levels: Group A: primary hypogonadism (FSH >15 IU/l and undetectable INB (20 pg/ml; Group D: mild central and severe gonadal dysfunction (FSH 1.5–15 IU/l, INB  Results There were 10, 8, 9 and 8 individuals in Groups A-D respectively; significantly more males in group A (9, 4, 4 and 2; P = 0.04. Significant differences between the groups were found in mean testosterone (P = 0.04, AMH (P = 0.003 and pubic hair (P = 0.04 in males and mean LH (P = 0.003 and breast development (P = 0.04 in females. Mean age, height, weight, BMI and the distribution of genetic subtypes were similar within the groups. Conclusions Analysis of FSH and inhibin B revealed four distinct phenotypes ranging from primary gonadal to central hypogonadism. Primary gonadal dysfunction was common, while severe gonadotropin deficiency was rare. Longitudinal studies are needed to verify whether the individual phenotypes are consistent.

  10. Obesity, polycystic ovarian syndrome and thyroid dysfunction in women with epilepsy

    Directory of Open Access Journals (Sweden)

    Mythili Ayyagari

    2012-01-01

    Full Text Available Introduction: Women with epilepsy (WWE have an increased risk for several endocrine disorders. Obesity and Polycystic Ovarian Syndrome (PCOS are common side-effects of anticonvulsant drugs. Aim: To study the prevalence of Obesity, PCOS, Thyroid dysfunction in WWE on monotherapy with Carbamazepine (CBZ, Sodium Valproate (VAL and Phenytoin (DPH Material and Methods: Sixty WWE in the reproductive age group (13 - 45 yr who are on atleast 6 months of monotherapy with either CBZ (20 or VAL (20 or DPH (20 are subjects of the study. Their Anthropometric data is recorded. They are interviewed and investigated for PCOS and thyroid dysfunction. Twenty healthy women in the reproductive age group served as controls. BMI>25 is taken as cut-off for Obesity. PCOS is defined as menstrual irregularity and/or clinical /biochemical hyperandrogenism with ultrasound evidence of PCO as per the Rotterdam criteria. TSH 4 is taken as evidence of thyroid dysfunction. Women are grouped according to the anticonvulsant drug received and the data analyzed in each group. Results: The mean BMI among VAL and CBZ users is significantly higher than among DPH users (23.3 & 23.4 vs 20.4. There is no significant difference in incidence of PCOS among WWE using either DPH or VAL or CBZ. Elevated TSH>4 is seen more often in WWE on VAL (9/20 compared to CBZ (6/20 and DPH (3/20. WWE on CBZ, VAL and DPH did not differ in mean BMI, Obesity, PCOS compared to healthy controls. As compared to healthy controls, more WWE on drug therapy had significantly elevated TSH (1/20 vs20/60. Conclusions: WWE on VAL and CBZ had significant weight gain compared to DPH users. Despite weight gain, there was no difference in the incidence of PCOS between the users of VAL, CBZ and DPH. As compared to healthy controls, more WWE on drug therapy had significantly elevated TSH, more so in the VAL group.

  11. A Metabolic Signature of Mitochondrial Dysfunction Revealed through a Monogenic Form of Leigh Syndrome

    Directory of Open Access Journals (Sweden)

    Julie Thompson Legault

    2015-11-01

    Full Text Available A decline in mitochondrial respiration represents the root cause of a large number of inborn errors of metabolism. It is also associated with common age-associated diseases and the aging process. To gain insight into the systemic, biochemical consequences of respiratory chain dysfunction, we performed a case-control, prospective metabolic profiling study in a genetically homogenous cohort of patients with Leigh syndrome French Canadian variant, a mitochondrial respiratory chain disease due to loss-of-function mutations in LRPPRC. We discovered 45 plasma and urinary analytes discriminating patients from controls, including classic markers of mitochondrial metabolic dysfunction (lactate and acylcarnitines, as well as unexpected markers of cardiometabolic risk (insulin and adiponectin, amino acid catabolism linked to NADH status (α-hydroxybutyrate, and NAD+ biosynthesis (kynurenine and 3-hydroxyanthranilic acid. Our study identifies systemic, metabolic pathway derangements that can lie downstream of primary mitochondrial lesions, with implications for understanding how the organelle contributes to rare and common diseases.

  12. Does microglial dysfunction play a role in autism and Rett syndrome?

    Science.gov (United States)

    MAEZAWA, IZUMI; CALAFIORE, MARCO; WULFF, HEIKE; JIN, LEE-WAY

    2016-01-01

    Autism spectrum disorders (ASDs) including classic autism is a group of complex developmental disabilities with core deficits of impaired social interactions, communication difficulties and repetitive behaviors. Although the neurobiology of ASDs has attracted much attention in the last two decades, the role of microglia has been ignored. Existing data are focused on their recognized role in neuroinflammation, which only covers a small part of the pathological repertoire of microglia. This review highlights recent findings on the broader roles of microglia, including their active surveillance of brain microenvironments and regulation of synaptic connectivity, maturation of brain circuitry and neurogenesis. Emerging evidence suggests that microglia respond to pre- and postnatal environmental stimuli through epigenetic interface to change gene expression, thus acting as effectors of experience-dependent synaptic plasticity. Impairments of these microglial functions could substantially contribute to several major etiological factors of autism, such as environmental toxins and cortical underconnectivity. Our recent study on Rett syndrome, a syndromic autistic disorder, provides an example that intrinsic microglial dysfunction due to genetic and epigenetic aberrations could detrimentally affect the developmental trajectory without evoking neuroinflammation. We propose that ASDs provide excellent opportunities to study the influence of microglia on neurodevelopment, and this knowledge could lead to novel therapies. PMID:22717189

  13. Exercise training prevents diastolic dysfunction induced by metabolic syndrome in rats

    Directory of Open Access Journals (Sweden)

    Cristiano Mostarda

    2012-07-01

    Full Text Available OBJECTIVE: High fructose consumption contributes to the incidence of metabolic syndrome and, consequently, to cardiovascular outcomes. We investigated whether exercise training prevents high fructose diet-induced metabolic and cardiac morphofunctional alterations. METHODS: Wistar rats receiving fructose overload (F in drinking water (100 g/l were concomitantly trained on a treadmill (FT for 10 weeks or kept sedentary. These rats were compared with a control group (C. Obesity was evaluated by the Lee index, and glycemia and insulin tolerance tests constituted the metabolic evaluation. Blood pressure was measured directly (Windaq, 2 kHz, and echocardiography was performed to determine left ventricular morphology and function. Statistical significance was determined by one-way ANOVA, with significance set at p<0.05. RESULTS: Fructose overload induced a metabolic syndrome state, as confirmed by insulin resistance (F: 3.6 ± 0.2 vs. C: 4.5 ± 0.2 mg/dl/min, hypertension (mean blood pressure, F: 118 ± 3 vs. C: 104 ± 4 mmHg and obesity (F: 0.31±0.001 vs. C: 0.29 ± 0.001 g/mm. Interestingly, fructose overload rats also exhibited diastolic dysfunction. Exercise training performed during the period of high fructose intake eliminated all of these derangements. The improvements in metabolic parameters were correlated with the maintenance of diastolic function. CONCLUSION: The role of exercise training in the prevention of metabolic and hemodynamic parameter alterations is of great importance in decreasing the cardiac morbidity and mortality related to metabolic syndrome.

  14. Value of MRI olfactory bulb evaluation in the assessment of olfactory dysfunction in Bardet-Biedl syndrome.

    Science.gov (United States)

    Braun, J J; Noblet, V; Kremer, S; Molière, S; Dollfus, H; Marion, V; Goetz, N; Muller, J; Riehm, S

    2016-07-01

    Olfactory bulb (OB) volume evaluation by magnetic resonance imaging (MRI) has been demonstrated to be related to olfactory dysfunction in many different diseases. Olfactory dysfunction is often overlooked in Bardet-Biedl syndrome (BBS) patients and is rarely objectively evaluated by MRI. We present a series of 20 BBS patients with olfactory dysfunction. The OB was evaluated separately and blindly by two radiologists (SR and SM) with 3 Tesla MRI imaging comparatively to 12 normal control subjects by global visual evaluation and by quantitative measurement of OB volume. In the 12 control cases OB visual evaluation was considered as normal in all cases for radiologist (SR) and in 10 cases for radiologist (SM). In the 20 BBS patients, OB visual evaluation was considered as abnormal in 18 cases for SR and in all cases for SM. OB volumetric evaluation for SR and SM in BBS patients was able to provide significant correlation between BBS and olfactory dysfunction. This study indicates that OB volume evaluation by MRI imaging like structural MRI scan for gray matter modifications demonstrates that olfactory dysfunction in BBS patients is a constant and cardinal symptom integrated in a genetical syndrome with peripheral and central olfactory structure alterations. PMID:26586152

  15. Nonalcoholic steatohepatitis as a novel player in metabolic syndrome-induced erectile dysfunction: an experimental study in the rabbit.

    OpenAIRE

    Vignozzi L; Filippi S; Comeglio P; Cellai I; Sarchielli E; Morelli A; Rastrelli G; Maneschi E; Galli A; Vannelli GB; Saad F; Mannucci E; Adorini L; Maggi M.

    2014-01-01

    A pathogenic link between erectile dysfunction (ED) and metabolic syndrome (MetS) is now well established. Nonalcoholic steatohepatitis (NASH), the hepatic hallmark of MetS, is regarded as an active player in the pathogenesis of MetS-associated cardiovascular disease (CVD). This study was aimed at valuating the relationship between MetS-induced NASH and penile dysfunction. We used a non-genomic, high fat diet (HFD)-induced, rabbit model of MetS, and treated HFD rabbits with testosterone (T...

  16. Dysfunction of intraflagellar transport-A causes hyperphagia-induced obesity and metabolic syndrome

    Directory of Open Access Journals (Sweden)

    Damon T. Jacobs

    2016-07-01

    Full Text Available Primary cilia extend from the plasma membrane of most vertebrate cells and mediate signaling pathways. Ciliary dysfunction underlies ciliopathies, which are genetic syndromes that manifest multiple clinical features, including renal cystic disease and obesity. THM1 (also termed TTC21B or IFT139 encodes a component of the intraflagellar transport-A complex and mutations in THM1 have been identified in 5% of individuals with ciliopathies. Consistent with this, deletion of murine Thm1 during late embryonic development results in cystic kidney disease. Here, we report that deletion of murine Thm1 during adulthood results in obesity, diabetes, hypertension and fatty liver disease, with gender differences in susceptibility to weight gain and metabolic dysfunction. Pair-feeding of Thm1 conditional knock-out mice relative to control littermates prevented the obesity and related disorders, indicating that hyperphagia caused the obese phenotype. Thm1 ablation resulted in increased localization of adenylyl cyclase III in primary cilia that were shortened, with bulbous distal tips on neurons of the hypothalamic arcuate nucleus, an integrative center for signals that regulate feeding and activity. In pre-obese Thm1 conditional knock-out mice, expression of anorexogenic pro-opiomelanocortin (Pomc was decreased by 50% in the arcuate nucleus, which likely caused the hyperphagia. Fasting of Thm1 conditional knock-out mice did not alter Pomc nor orexogenic agouti-related neuropeptide (Agrp expression, suggesting impaired sensing of changes in peripheral signals. Together, these data indicate that the Thm1-mutant ciliary defect diminishes sensitivity to feeding signals, which alters appetite regulation and leads to hyperphagia, obesity and metabolic disease.

  17. Dysfunction of intraflagellar transport-A causes hyperphagia-induced obesity and metabolic syndrome.

    Science.gov (United States)

    Jacobs, Damon T; Silva, Luciane M; Allard, Bailey A; Schonfeld, Michael P; Chatterjee, Anindita; Talbott, George C; Beier, David R; Tran, Pamela V

    2016-07-01

    Primary cilia extend from the plasma membrane of most vertebrate cells and mediate signaling pathways. Ciliary dysfunction underlies ciliopathies, which are genetic syndromes that manifest multiple clinical features, including renal cystic disease and obesity. THM1 (also termed TTC21B or IFT139) encodes a component of the intraflagellar transport-A complex and mutations in THM1 have been identified in 5% of individuals with ciliopathies. Consistent with this, deletion of murine Thm1 during late embryonic development results in cystic kidney disease. Here, we report that deletion of murine Thm1 during adulthood results in obesity, diabetes, hypertension and fatty liver disease, with gender differences in susceptibility to weight gain and metabolic dysfunction. Pair-feeding of Thm1 conditional knock-out mice relative to control littermates prevented the obesity and related disorders, indicating that hyperphagia caused the obese phenotype. Thm1 ablation resulted in increased localization of adenylyl cyclase III in primary cilia that were shortened, with bulbous distal tips on neurons of the hypothalamic arcuate nucleus, an integrative center for signals that regulate feeding and activity. In pre-obese Thm1 conditional knock-out mice, expression of anorexogenic pro-opiomelanocortin (Pomc) was decreased by 50% in the arcuate nucleus, which likely caused the hyperphagia. Fasting of Thm1 conditional knock-out mice did not alter Pomc nor orexogenic agouti-related neuropeptide (Agrp) expression, suggesting impaired sensing of changes in peripheral signals. Together, these data indicate that the Thm1-mutant ciliary defect diminishes sensitivity to feeding signals, which alters appetite regulation and leads to hyperphagia, obesity and metabolic disease. PMID:27482817

  18. Sarcoidosis of the upper and lower airways.

    Science.gov (United States)

    Morgenthau, Adam S; Teirstein, Alvin S

    2011-12-01

    Sarcoidosis is a systemic granulomatous disease of undetermined etiology characterized by a variable clinical presentation and disease course. Although clinical granulomatous inflammation may occur within any organ system, more than 90% of sarcoidosis patients have lung disease. Sarcoidosis is considered an interstitial lung disease that is frequently characterized by restrictive physiologic dysfunction on pulmonary function tests. However, sarcoidosis also involves the airways (large and small), causing obstructive airways disease. It is one of a few interstitial lung diseases that affects the entire length of the respiratory tract - from the nose to the terminal bronchioles - and causes a broad spectrum of airways dysfunction. This article examines airway dysfunction in sarcoidosis. The anatomical structure of the airways is the organizational framework for our discussion. We discuss sarcoidosis involving the nose, sinuses, nasal passages, larynx, trachea, bronchi and small airways. Common complications of airways disease, such as, atelectasis, fibrosis, bullous leions, bronchiectasis, cavitary lesions and mycetomas, are also reviewed. PMID:22082167

  19. Laryngeal mask airway guided tracheal intubation in a neonate with the Pierre Robin syndrome

    DEFF Research Database (Denmark)

    Hansen, Tom Giedsing; Joensen, Henning; Henneberg, Steen Winther;

    1995-01-01

    Endotracheal intubation in infants with the Pierre Robin syndrome may sometimes be impossible to accomplish by conventional means. To aid difficult tracheal intubation many different techniques have been described. We present a case, in which we successfully intubated a small-for-date newborn boy...

  20. MR Image Analytics to Characterize the Upper Airway Structure in Obese Children with Obstructive Sleep Apnea Syndrome

    Science.gov (United States)

    Tong, Yubing; Udupa, Jayaram K.; Sin, Sanghun; Liu, Zhengbing; Wileyto, E. Paul; Torigian, Drew A.; Arens, Raanan

    2016-01-01

    Purpose Quantitative image analysis in previous research in obstructive sleep apnea syndrome (OSAS) has focused on the upper airway or several objects in its immediate vicinity and measures of object size. In this paper, we take a more general approach of considering all major objects in the upper airway region and measures pertaining to their individual morphological properties, their tissue characteristics revealed by image intensities, and the 3D architecture of the object assembly. We propose a novel methodology to select a small set of salient features from this large collection of measures and demonstrate the ability of these features to discriminate with very high prediction accuracy between obese OSAS and obese non-OSAS groups. Materials and Methods Thirty children were involved in this study with 15 in the obese OSAS group with an apnea-hypopnea index (AHI) = 14.4 ± 10.7) and 15 in the obese non-OSAS group with an AHI = 1.0 ± 1.0 (p<0.001). Subjects were between 8–17 years and underwent T1- and T2-weighted magnetic resonance imaging (MRI) of the upper airway during wakefulness. Fourteen objects in the vicinity of the upper airways were segmented in these images and a total of 159 measurements were derived from each subject image which included object size, surface area, volume, sphericity, standardized T2-weighted image intensity value, and inter-object distances. A small set of discriminating features was identified from this set in several steps. First, a subset of measures that have a low level of correlation among the measures was determined. A heat map visualization technique that allows grouping of parameters based on correlations among them was used for this purpose. Then, through T-tests, another subset of measures which are capable of separating the two groups was identified. The intersection of these subsets yielded the final feature set. The accuracy of these features to perform classification of unseen images into the two patient groups was

  1. Evaluation of C-reactive protein, Haptoglobin and cardiac troponin 1 levels in brachycephalic dogs with upper airway obstructive syndrome

    Directory of Open Access Journals (Sweden)

    Planellas Marta

    2012-08-01

    Full Text Available Abstract Background Brachycephalic dogs have unique upper respiratory anatomy with abnormal breathing patterns similar to those in humans with obstructive sleep apnea syndrome (OSAS. The objective of this study was to evaluate the correlation between anatomical components, clinical signs and several biomarkers, used to determine systemic inflammation and myocardial damage (C-reactive protein, CRP; Haptoglobin, Hp; cardiac troponin I, cTnI, in dogs with brachycephalic upper airway obstructive syndrome (BAOS. Results Fifty brachycephalic dogs were included in the study and the following information was studied: signalment, clinical signs, thoracic radiographs, blood work, ECG, components of BAOS, and CRP, Hp and cTnI levels. A high proportion of dogs with BAOS (88% had gastrointestinal signs. The prevalence of anatomic components of BAOS was: elongated soft palate (100%, stenotic nares (96%, everted laryngeal saccules (32% and tracheal hypoplasia (29.1%. Increased serum levels of biomarkers were found in a variable proportion of dogs: 14% (7/50 had values of CRP > 20 mg/L, 22.9% (11/48 had values of Hp > 3 g/L and 47.8% (22/46 had levels of cTnI > 0.05 ng/dl. Dogs with everted laryngeal saccules had more severe respiratory signs (p Conclusions According to the low percentage of patients with elevated levels of CRP and Hp, BAOS does not seem to cause an evident systemic inflammatory status. Some degree of myocardial damage may occur in dogs with BAOS that can be detected by cTnI concentration.

  2. Association between peripheral airway function and neutrophilic inflammation in asthma

    NARCIS (Netherlands)

    Farah, Claude S.; Keulers, Laurien A. B.; Hardaker, Kate M.; Peters, Matthew J.; Berend, Norbert; Postma, Dirkje S.; Salome, Cheryl M.; King, Gregory G.

    2015-01-01

    Background and objectiveSmall airway dysfunction is associated with asthma severity and control, but its association with airway inflammation is unknown. The aim was to determine the association between sputum inflammatory cells and the site of small airway dysfunction, measured by multiple breath n

  3. Factors associated with major complications in the short-term postoperative period in dogs undergoing surgery for brachycephalic airway syndrome.

    Science.gov (United States)

    Ree, Jennifer J; Milovancev, Milan; MacIntyre, Laura A; Townsend, Katy L

    2016-09-01

    Surgical correction of brachycephalic airway syndrome (BAS) in dogs has been reported to result in low complication rates and good long-term outcomes. Previous reports have not identified risk factors for the development of complications following BAS surgery. This retrospective study evaluated a wide variety of patient- and procedure-related, pre-operative, intra-operative, and post-operative factors for an association with the development of major postoperative complications in the short-term period following BAS surgery. The overall major complication rate, including death or euthanasia, was 4/55 (7%) dogs. Temporary tracheostomy was the only major surgical complication identified (n = 3). Multiple logistic regression identified postoperative radiographic evidence of pneumonia as associated with the development of any major complication overall, requirement of a temporary tracheostomy postoperatively, and death or euthanasia, within the short-term postoperative period. Future prospective studies should evaluate specific risk factors for an association with major complications following BAS surgery in dogs to improve patient outcomes. PMID:27587891

  4. Fermented Red Ginseng Potentiates Improvement of Metabolic Dysfunction in Metabolic Syndrome Rat Models.

    Science.gov (United States)

    Kho, Min Chul; Lee, Yun Jung; Park, Ji Hun; Kim, Hye Yoom; Yoon, Jung Joo; Ahn, You Mee; Tan, Rui; Park, Min Cheol; Cha, Jeong Dan; Choi, Kyung Min; Kang, Dae Gill; Lee, Ho Sub

    2016-01-01

    Metabolic syndrome including obesity, dyslipidemia and hypertension is a cluster of risk factors of cardiovascular disease. Fermentation of medicinal herbs improves their pharmacological efficacy. Red ginseng (RG), a widely used traditional herbal medicine, was reported with anti-inflammatory and anti-oxidant activity. Aim in the present study was to investigate that the effects of fermented red ginseng (FRG) on a high-fructose (HF) diet induced metabolic disorders, and those effects were compared to RG and losartan. Animals were divided into four groups: a control group fed a regular diet and tap water, and fructose groups that were fed a 60% high-fructose (HF) diet with/without RG 250 mg/kg/day or FRG 250 mg/kg/day for eight weeks, respectively. Treatment with FRG significantly suppressed the increments of body weight, liver weight, epididymal fat weight and adipocyte size. Moreover, FRG significantly prevented the development of metabolic disturbances such as hyperlipidemia and hypertension. Staining with Oil-red-o demonstrated a marked increase of hepatic accumulation of triglycerides, and this increase was prevented by FRG. FRG ameliorated endothelial dysfunction by downregulation of endothelin-1 (ET-1) and adhesion molecules in the aorta. In addition, FRG induced markedly upregulation of Insulin receptor substrate 1 (IRS-1) and glucose transporter type 4 (Glut4) in the muscle. These results indicate that FRG ameliorates obesity, dyslipidemia, hypertension and fatty liver in HF diet rats. More favorable pharmacological effects on HF diet induced metabolic disorders were observed with FRG, compared to an equal dose of RG. These results showed that the pharmacological activity of RG was enhanced by fermentation. Taken together, fermentated red ginseng might be a beneficial therapeutic approach for metabolic syndrome. PMID:27322312

  5. Fermented Red Ginseng Potentiates Improvement of Metabolic Dysfunction in Metabolic Syndrome Rat Models

    Directory of Open Access Journals (Sweden)

    Min Chul Kho

    2016-06-01

    Full Text Available Metabolic syndrome including obesity, dyslipidemia and hypertension is a cluster of risk factors of cardiovascular disease. Fermentation of medicinal herbs improves their pharmacological efficacy. Red ginseng (RG, a widely used traditional herbal medicine, was reported with anti-inflammatory and anti-oxidant activity. Aim in the present study was to investigate that the effects of fermented red ginseng (FRG on a high-fructose (HF diet induced metabolic disorders, and those effects were compared to RG and losartan. Animals were divided into four groups: a control group fed a regular diet and tap water, and fructose groups that were fed a 60% high-fructose (HF diet with/without RG 250 mg/kg/day or FRG 250 mg/kg/day for eight weeks, respectively. Treatment with FRG significantly suppressed the increments of body weight, liver weight, epididymal fat weight and adipocyte size. Moreover, FRG significantly prevented the development of metabolic disturbances such as hyperlipidemia and hypertension. Staining with Oil-red-o demonstrated a marked increase of hepatic accumulation of triglycerides, and this increase was prevented by FRG. FRG ameliorated endothelial dysfunction by downregulation of endothelin-1 (ET-1 and adhesion molecules in the aorta. In addition, FRG induced markedly upregulation of Insulin receptor substrate 1 (IRS-1 and glucose transporter type 4 (Glut4 in the muscle. These results indicate that FRG ameliorates obesity, dyslipidemia, hypertension and fatty liver in HF diet rats. More favorable pharmacological effects on HF diet induced metabolic disorders were observed with FRG, compared to an equal dose of RG. These results showed that the pharmacological activity of RG was enhanced by fermentation. Taken together, fermentated red ginseng might be a beneficial therapeutic approach for metabolic syndrome.

  6. Role of adrenocortical dysfunction in the pathogenesis of poisoning syndromes due to some industrial toxins (aromatic nitro compounds, lead)

    Energy Technology Data Exchange (ETDEWEB)

    Makotcenko, V.M.

    1974-10-01

    Comparative study is presented of adrenocortical dysfunction in workers chronically exposed to aromatic nitro compounds and to lead. The chronic intoxications produced by aromatic nitro compounds and by lead are characterized by a slight reduction in adrenocortical activity, which plays an important part in the pathogenesis of certain syndromes such as asthenia, gastric secretion disorders, lead anemia and lead polyneuritis. It is desirable to take measures to normalize corticosteroid formation when chronic occupational poisoning is being treated. (CIS Abstr. Vol. 2)

  7. Developmental androgen excess programs sympathetic tone and adipose tissue dysfunction and predisposes to a cardiometabolic syndrome in female mice.

    Science.gov (United States)

    Nohara, Kazunari; Waraich, Rizwana S; Liu, Suhuan; Ferron, Mathieu; Waget, Aurélie; Meyers, Matthew S; Karsenty, Gérard; Burcelin, Rémy; Mauvais-Jarvis, Franck

    2013-06-15

    Among women, the polycystic ovarian syndrome (PCOS) is considered a form of metabolic syndrome with reproductive abnormalities. Women with PCOS show increased sympathetic tone, visceral adiposity with enlarged adipocytes, hypoadiponectinemia, insulin resistance, glucose intolerance, increased inactive osteocalcin, and hypertension. Excess fetal exposure to androgens has been hypothesized to play a role in the pathogenesis of PCOS. Previously, we showed that neonatal exposure to the androgen testosterone (NT) programs leptin resistance in adult female mice. Here, we studied the impact of NT on lean and adipose tissues, sympathetic tone in cardiometabolic tissues, and the development of metabolic dysfunction in mice. Neonatally androgenized adult female mice (NTF) displayed masculinization of lean tissues with increased cardiac and skeletal muscle as well as kidney masses. NTF mice showed increased and dysfunctional white adipose tissue with increased sympathetic tone in both visceral and subcutaneous fat as well as increased number of enlarged and insulin-resistant adipocytes that displayed altered expression of developmental genes and hypoadiponectinemia. NTF exhibited dysfunctional brown adipose tissue with increased mass and decreased energy expenditure. They also displayed decreased undercarboxylated and active osteocalcin and were predisposed to obesity during chronic androgen excess. NTF showed increased renal sympathetic tone associated with increased blood pressure, and they developed glucose intolerance and insulin resistance. Thus, developmental exposure to testosterone in female mice programs features of cardiometabolic dysfunction, as can be observed in women with PCOS, including increased sympathetic tone, visceral adiposity, insulin resistance, prediabetes, and hypertension.

  8. Effect of continuous positive airway pressure treatment on vascular endothelial function in patients with obstructive sleep apnea hypopnea syndrome and coronary artery disease

    Institute of Scientific and Technical Information of China (English)

    张希龙; 殷凯生; 毛辉; 王虹; 杨玉

    2004-01-01

    Background Continuous positive airway pressure (CPAP) treatment has been proven to be effective in improving the symptoms of coexisting coronary heart disease (CHD) in patients with obstructive sleep apnea hypopnea syndrome (OSAHS). However, it is still unclear whether such improvements are linked to changes in vascular endothelial function. This research was carried out to investigate the effects of CPAP treatment on vascular endothelial function in patients with OSAHS and CHD.Methods Thirty-six patients with moderate or severe OSAHS and CHD undergoing three months of CPAP treatment were recruited for this study. The changes in their morning plasma nitric oxide (NO) and endothelin (ET) levels, NO/ET ratio, total ischemic burden (TIB) of the myocardium, apnea hypopnea index (AHI), and minimal and mean pulse oxygen saturation (SpO2) were compared and analyzed before and during CPAP treatment. Results Compared with the plasma levels of ET [(51.39±11.69) ng/L] and NO [(36.67±11.86) μmol/L], NO/ET (0.71±0.14), AHI (32.4±7.9), minimal SpO2 [(68.9±11.4)%], and myocardial TIB [(66.29±16.37) mm*min] before treatment, there were significant decreases in ET [(33.41±10.03) ng/L] (P<0.05), increases in NO [(59.89±10.26) μmol/L] and NO/ET (1.79±0.38) (P<0.01), decreases in AHI (1.9±0.5), and increases in minimal SpO2 [(90.6±1.8) %] (all P<0.01) and myocardial TIB [(36.42±10.87) mm*min] (P<0.05) after three months of CPAP treatment.Conclusion CPAP treatment may play an important role in the improvement and protection of vascular endothelial dysfunction and myocardial ischemia in OSAHS patients with CHD.

  9. Pathophysiology of primary burning mouth syndrome with special focus on taste dysfunction: a review.

    Science.gov (United States)

    Kolkka-Palomaa, M; Jääskeläinen, S K; Laine, M A; Teerijoki-Oksa, T; Sandell, M; Forssell, H

    2015-11-01

    Primary burning mouth syndrome (BMS) is a chronic oral condition characterized by burning pain often accompanied with taste dysfunction and xerostomia. The most compelling evidence concerning BMS pathophysiology comes from studies on the somatosensory system using neurophysiologic or psychophysical methods such as blink reflex, thermal quantitative sensory testing, as well as functional brain imaging. They have provided convincing evidence for neuropathic involvement at several levels of the somatosensory system in BMS pain pathophysiology. The number of taste function studies trying to substantiate the subjective taste disturbances or studies on salivary factors in BMS is much more limited, and most of them suffer from definitional and methodological problems. This review aims to critically evaluate the existing literature on the pathophysiology of BMS, paying special attention to the correctness of case selection and the methodology used in published studies, and to summarize the current state of knowledge. Based on the recognition of several gaps in the current understanding of the pathophysiology of BMS especially as regards taste and pain system interactions, the review ends with future scenarios for research in this area. PMID:25962669

  10. Dysfunctional hippocampal inhibition in the Ts65Dn mouse model of Down syndrome.

    Science.gov (United States)

    Best, Tyler K; Cramer, Nathan P; Chakrabarti, Lina; Haydar, Tarik F; Galdzicki, Zygmunt

    2012-02-01

    GABAergic dysfunction is implicated in hippocampal deficits of the Ts65Dn mouse model of Down syndrome (DS). Since Ts65Dn mice overexpress G-protein coupled inward-rectifying potassium (GIRK2) containing channels, we sought to evaluate whether increased GABAergic function disrupts the functioning of hippocampal circuitry. After confirming that GABA(B)/GIRK current density is significantly elevated in Ts65Dn CA1 pyramidal neurons, we compared monosynaptic inhibitory inputs in CA1 pyramidal neurons in response to proximal (stratum radiatum; SR) and distal (stratum lacunosum moleculare; SLM) stimulation of diploid and Ts65Dn acute hippocampal slices. Synaptic GABA(B) and GABA(A) mediated currents evoked by SR stimulation were generally unaffected in Ts65Dn CA1 neurons. However, the GABA(B)/GABA(A) ratios evoked by stimulation within the SLM of Ts65Dn hippocampus were significantly larger in magnitude, consistent with increased GABA(B)/GIRK currents after SLM stimulation. These results indicate that GIRK overexpression in Ts65Dn has functional consequences which affect the balance between GABA(B) and GABA(A) inhibition of CA1 pyramidal neurons, most likely in a pathway specific manner, and may contribute to cognitive deficits reported in these mice.

  11. Endothelial Progenitor Cell Dysfunction in Myelodysplastic Syndromes: Possible Contribution of a Defective Vascular Niche to Myelodysplasia

    Directory of Open Access Journals (Sweden)

    Luciana Teofili

    2015-05-01

    Full Text Available We set a model to replicate the vascular bone marrow niche by using endothelial colony forming cells (ECFCs, and we used it to explore the vascular niche function in patients with low-risk myelodysplastic syndromes (MDS. Overall, we investigated 56 patients and we observed higher levels of ECFCs in MDS than in healthy controls; moreover, MDS ECFCs were found variably hypermethylated for p15INK4b DAPK1, CDH1, or SOCS1. MDS ECFCs exhibited a marked adhesive capacity to normal mononuclear cells. When normal CD34+ cells were co-cultured with MDS ECFCs, they generated significant lower amounts of CD11b+ and CD41+ cells than in co-culture with normal ECFCs. At gene expression profile, several genes involved in cell adhesion were upregulated in MDS ECFCs, while several members of the Wingless and int (Wnt pathways were underexpressed. Furthermore, at miRNA expression profile, MDS ECFCs hypo-expressed various miRNAs involved in Wnt pathway regulation. The addition of Wnt3A reduced the expression of intercellular cell adhesion molecule-1 on MDS ECFCs and restored the defective expression of markers of differentiation. Overall, our data demonstrate that in low-risk MDS, ECFCs exhibit various primary abnormalities, including putative MDS signatures, and suggest the possible contribution of the vascular niche dysfunction to myelodysplasia.

  12. Metabolic Syndrome after Hematopoietic Cell Transplantation: At the Intersection of Treatment Toxicity and Immune Dysfunction.

    Science.gov (United States)

    Turcotte, Lucie M; Yingst, Ashley; Verneris, Michael R

    2016-07-01

    Hematopoietic cell transplantation (HCT) survivors face a multitude of short- and long-term health complications in the years after treatment. One important health complication that is associated with significant morbidity is metabolic syndrome (MetSyn). This constellation of findings, which includes obesity, glucose and lipid dysmetabolism, and hypertension, places affected individuals at increased risk for type 2 diabetes mellitus, cardiovascular complications, and stroke. Previous studies have linked MetSyn in HCT survivors to prior treatment; however, few studies have addressed the potential roles of systemic inflammation and immune system dysfunction after HCT. Within this review, we address the recent advances in the understanding of adipose tissue biology, immune, and inflammatory mechanisms involved in MetSyn in non-HCT patients, and lastly, we discuss potential novel mechanisms that may play a role in MetSyn development after HCT, such as hematopoietic stem cell source, inflammatory status of the stem cell donor, and microbiome composition, all of which represent potential new directions for post-HCT MetSyn research. PMID:27013015

  13. Hormonal Modulation in Aging Patients with Erectile Dysfunction and Metabolic Syndrome

    Directory of Open Access Journals (Sweden)

    Inês Campos Costa

    2013-01-01

    Full Text Available Erectile dysfunction (ED, metabolic syndrome (MetS, and hypogonadism are closely related, often coexisting in the aging male. Obesity was shown to raise the risk of ED and hypogonadism, as well as other endocrinological disturbances with impact on erectile function. We selected 179 patients referred for ED to our andrology unit, aiming to evaluate gonadotropins and estradiol interplay in context of ED, MetS, and hypogonadism. Patients were stratified into groups in accordance with the presence (or not of MetS and/or hypogonadism. Noticeable differences in total testosterone (TT and free testosterone (FT levels were found between patients with and without MetS. Men with MetS evidenced lower TT circulating levels with an increasing number of MetS parameters, for which hypertriglyceridemia and waist circumference strongly contributed. Regarding the hypothalamic-pituitary-gonadal axis, patients with hypogonadism did not exhibit raised LH levels. Interestingly, among those with higher LH levels, estradiol values were also increased. Possible explanations for this unexpected profile of estradiol may be the age-related adiposity, other estrogen-raising pathways, or even unknown mechanisms. Estradiol is possibly a molecule with further interactions beyond the currently described. Our results further enlighten this still unclear multidisciplinary and complex subject, raising new investigational opportunities.

  14. Calcitriol attenuates cardiac remodeling and dysfunction in a murine model of polycystic ovary syndrome.

    Science.gov (United States)

    Gao, Ling; Cao, Jia-Tian; Liang, Yan; Zhao, Yi-Chao; Lin, Xian-Hua; Li, Xiao-Cui; Tan, Ya-Jing; Li, Jing-Yi; Zhou, Cheng-Liang; Xu, Hai-Yan; Sheng, Jian-Zhong; Huang, He-Feng

    2016-05-01

    Polycystic ovary syndrome (PCOS) is a complex reproductive and metabolic disorder affecting 10 % of reproductive-aged women, and is well associated with an increased prevalence of cardiovascular risk factors. However, there are few data concerning the direct association of PCOS with cardiac pathologies. The present study aims to investigate the changes in cardiac structure, function, and cardiomyocyte survival in a PCOS model, and explore the possible effect of calcitriol administration on these changes. PCOS was induced in C57BL/6J female mice by chronic dihydrotestosterone administration, as evidenced by irregular estrous cycles, obesity and dyslipidemia. PCOS mice progressively developed cardiac abnormalities including cardiac hypertrophy, interstitial fibrosis, myocardial apoptosis, and cardiac dysfunction. Conversely, concomitant administration of calcitriol significantly attenuated cardiac remodeling and cardiomyocyte apoptosis, and improved cardiac function. Molecular analysis revealed that the beneficial effect of calcitriol was associated with normalized autophagy function by increasing phosphorylation levels of AMP-activated protein kinase and inhibiting phosphorylation levels of mammalian target of rapamycin complex. Our findings provide the first evidence for the presence of cardiac remodeling in a PCOS model, and vitamin D supplementation may be a potential therapeutic strategy for the prevention and treatment of PCOS-related cardiac remodeling.

  15. Sympathetic dysfunction in vasovagal syncope and the postural orthostatic tachycardia syndrome.

    Science.gov (United States)

    Lambert, Elisabeth; Lambert, Gavin W

    2014-01-01

    Orthostatic intolerance is the inability to tolerate the upright posture and is relieved by recumbence. It most commonly affects young women and has a major impact on quality of life and psychosocial well-being. Several forms of orthostatic intolerance have been described. The most common one is the recurrent vasovagal syncope (VVS) phenotype which presents as a transient and abrupt loss of consciousness and postural tone that is followed by rapid recovery. Another common type of orthostatic intolerance is the postural orthostatic tachycardia syndrome (POTS) which is characterized by an excessive rise in heart rate upon standing and is associated with symptoms of presyncope such as light-headedness, fatigue, palpitations, and nausea. Maintenance of arterial pressure under condition of reduced central blood volume during the orthostasis is accomplished in large part through sympathetic efferent nerve traffic to the peripheral vasculature. Therefore sympathetic nervous system (SNS) dysfunction is high on the list of possible contributors to the pathophysiology of orthostatic intolerance. Investigations into the role of the SNS in orthostatic intolerance have yielded mixed results. This review outlines the current knowledge of the function of the SNS in both VVS and POTS.

  16. Sympathetic dysfunction in vasovagal syncope and the postural orthostatic tachycardia syndrome

    Directory of Open Access Journals (Sweden)

    Elisabeth eLambert

    2014-07-01

    Full Text Available Orthostatic intolerance is the inability to tolerate the upright posture and is relieved by recumbence. It most commonly affects young women and has a major impact on quality of life and psychosocial well being. Several forms of orthostatic intolerance have been described. The most common one is the recurrent vasovagal syncope (VVS phenotype which presents as a transient and abrupt loss of consciousness and postural tone that is followed by rapid recovery. Another common type of orthostatic intolerance is the postural orthostatic tachycardia syndrome (POTS which is characterized by an excessive rise in heart rate upon standing and is associated with symptoms of presyncope such as light-headedness, fatigue, palpitations and nausea. Maintenance of arterial pressure under condition of reduced central blood volume during the orthostasis is accomplished in large part through sympathetic efferent nerve traffic to the peripheral vasculature. Therefore sympathetic nervous system (SNS dysfunction is high on the list of possible contributors to the pathophysiology of orthostatic intolerance. Investigations into the role of the SNS in orthostatic intolerance have yielded mixed results. This review outlines the current knowledge of the function of the SNS in both VVS and POTS.

  17. Mental Fatigue and Executive Dysfunction in Patients with Cushing’s Syndrome in Remission

    Directory of Open Access Journals (Sweden)

    Eleni Papakokkinou

    2015-01-01

    Full Text Available Patients with Cushing’s syndrome (CS in remission often suffer from impaired quality of life and cognitive dysfunction. The primary aim was to investigate the occurrence of mental fatigue, characterized by mental exhaustion and long recovery time following mentally strenuous tasks, in patients with CS in remission. The secondary aim was to examine whether the newly developed parts C and D of the trail making test (TMT are more sensitive, compared to the conventional parts A and B, to evaluate attention and executive function. This was a cross-sectional study including 51 patients with CS in remission and 51 controls. All subjects completed the self-administrated mental fatigue scale (MFS and performed all four parts of the TMT. The patients had worse outcome on all components of the MFS except for sensitivity to noise. After adjustment for mental fatigue, depression, and anxiety, the patients performed worse only on part D of the TMT (P<0.05. Mental fatigue is common in patients with CS in remission and can be captured by using the MFS. The most demanding part of the TMT, part D, is more useful to capture cognitive deficits in patients with CS in remission compared to the conventional parts A and B.

  18. Obstructive sleep apnea syndrome, continuous positive airway pressure and treatment of hypertension.

    Science.gov (United States)

    Floras, John S

    2015-09-15

    Obstructive sleep apnea (OSA), present in ~15% of the general population, increases the risks of stroke, heart failure, and premature death. Importantly, individuals with cardiovascular disease have a higher prevalence yet they often have few symptoms to alert clinicians to its presence. OSA with an apnea-hypopnea index (AHI) ≥15 events/hour is present in ≥30% of patients with primary hypertension and in up to 80% of those with drug resistant hypertension, suggesting that the neural, hormonal, inflammatory and vascular cascades triggered by OSA may elevate blood pressure chronically. The purpose of this review is to summarize: (1) the epidemiology of OSA and its relation to cardiovascular risk; (2) potential mechanisms by which OSA could promote conditions known to increase the risk of hypertension or contribute to its development and progression; (3) evidence for and against a pro-hypertensive effect of OSA; and, (4) the impact of treatment with continuous positive airway pressure (CPAP) on blood pressure and blood pressure-related morbidities. The prevailing view that the effect of treatment on blood pressure is modest arises from the inability of most contemporary technology to measure accurately the true impact of CPAP on OSA-entrained surges in nocturnal blood pressure. Moreover the exclusive focus on blood pressure, as if this is the principal determinant of cardiovascular event rates in this population, is naïve. The capacity to reduce cardiovascular risk by treating OSA with CPAP likely transcends a simple blood pressure effect; formal testing of this hypothesis will require adequately powered randomized clinical trials.

  19. X-ray upper airway changes in individuals suffering from obstructive respiratory disorders during sleep

    International Nuclear Information System (INIS)

    Obstructive respiratory disorders during sleep present an important medical and social problem. Serious dysfunctions of cardiovascular, nervous, endocrine and other vital systems of the body reduce longevity and life quality. On the other hand, load nocturnal snore and abnormal during sleepiness cause great damage to family life, reduce working capacity and induce accidents. X-ray visualization of the upper airways is essential in diagnosing obstructive upper airway states and selecting patients for surgical treatment. The paper presents the author's own experience in using various X-ray diagnostic methods in patients with chronic snore and obstructive sleep apnoea-hypopnoea syndrome

  20. Hearing Dysfunction in Heterozygous MitfMi-wh/+ Mice, a Model for Waardenburg Syndrome Type 2 and Tietz Syndrome

    OpenAIRE

    Ni, Christina; Zhang, Deming; Beyer, Lisa A.; Halsey, Karin E.; Raphael, Yehoash; Dolan, David F.; Hornyak, Thomas J.

    2012-01-01

    The human deafness-pigmentation syndromes Waardenburg syndrome type 2a and Tietz syndrome are characterized by profound deafness but only partial cutaneous pigmentary abnormalities. Both syndromes are caused by mutations in MITF. To illuminate differences between cutaneous and otic melanocytes in these syndromes, their development and survival in heterozygous Microphthalmia-White (MitfMi‐wh/+) mice were studied and hearing function of these mice characterized. MitfMi-wh/+ mice have a profound...

  1. Chronic functional bowel syndrome enhances gut-brain axis dysfunction, neuroinflammation, cognitive impairment, and vulnerability to dementia.

    Science.gov (United States)

    Daulatzai, Mak Adam

    2014-04-01

    The irritable bowel syndrome (IBS) is a common chronic functional gastrointestinal disorder world wide that lasts for decades. The human gut harbors a diverse population of microbial organisms which is symbiotic and important for well being. However, studies on conventional, germ-free, and obese animals have shown that alteration in normal commensal gut microbiota and an increase in pathogenic microbiota-termed "dysbiosis", impact gut function, homeostasis, and health. Diarrhea, constipation, visceral hypersensitivity, and abdominal pain arise in IBS from the gut-induced dysfunctional metabolic, immune, and neuro-immune communication. Dysbiosis in IBS is associated with gut inflammation. Gut-related inflammation is pivotal in promoting endotoxemia, systemic inflammation, and neuroinflammation. A significant proportion of IBS patients chronically consume alcohol, non-steroidal anti-inflammatories, and fatty diet; they may also suffer from co-morbid respiratory, neuromuscular, psychological, sleep, and neurological disorders. The above pathophysiological substrate is underpinned by dysbiosis, and dysfunctional bidirectional "Gut-Brain Axis" pathways. Pathogenic gut microbiota-related systemic inflammation (due to increased lipopolysaccharide and pro-inflammatory cytokines, and barrier dysfunction), may trigger neuroinflammation enhancing dysfunctional brain regions including hippocampus and cerebellum. These as well as dysfunctional vago-vagal gut-brain axis may promote cognitive impairment. Indeed, inflammation is characteristic of a broad spectrum of neurodegenerative diseases that manifest demntia. It is argued that an awareness of pathophysiological impact of IBS and implementation of appropriate therapeutic measures may prevent cognitive impairment and minimize vulnerability to dementia. PMID:24590859

  2. Autonomic Dysfunction Presenting as Postural Orthostatic Tachycardia Syndrome in Patients with Multiple Sclerosis

    Directory of Open Access Journals (Sweden)

    Khalil Kanjwal, Beverly Karabin, Yousuf Kanjwal, Blair P Grubb

    2010-01-01

    Full Text Available Background: Autonomic dysfunction is common in patients suffering from multiple sclerosis (MS and orthostatic dizziness occurs in almost 50% of these patients. However, there have been no reports on postural orthostatic tachycardia syndrome (POTS in patients suffering from MS. Methods: The patients were included for analysis in this study if they had POTS with either a prior history of MS or having developed MS while being followed for POTS. Postural orthostatic tachycardia (POTS is defined as symptoms of orthostatic intolerance(>6months accompanied by a heart rate increase of at least 30 beats/min (or a rate that exceeds 120 beats/min that occurs in the first 10 minutes of upright posture or head up tilt test (HUTT occurring in the absence of other chronic debilitating disorders. We identified nine patients with POTS who were suffering from MS as well. Each of these patients had been referred from various other centers for second opinions. Results: The mean age at the time of diagnosis of POTS was 49±9 years and eight of the 9 patients were women. Five patients (55% had hyperlipidemia, 3 (33% migraine and 2 (22% patients had coronary artery disease and diabetes each. Fatigue and palpitations (on assuming upright posture were the most common finding in our patients (9/9. All patients also had orthostatic dizziness. Syncope was seen in 5/9(55% of patients. Four patients (44%, who did not have clear syncope, were having episodes of near syncope. The presence of POTS in our study population resulted in substantial limitation of daily activities. Following recognition and treatment of POTS, 6/9(66%, patients were able to resume daily activities of living. Their symptoms (especially fatigue and orthostatic intolerance improved. The frequency and severity of syncope also improved. Three (33% patients failed to show a good response to treatment. Conclusion: Patients suffering from MS may manifest autonomic dysfunction by developing POTS. Early

  3. Mitochondrial dysfunction as a central actor in intellectual disability-related diseases: an overview of Down syndrome, autism, Fragile X and Rett syndrome.

    Science.gov (United States)

    Valenti, Daniela; de Bari, Lidia; De Filippis, Bianca; Henrion-Caude, Alexandra; Vacca, Rosa Anna

    2014-10-01

    Clinical manifestations typical of mitochondrial diseases are often present in various genetic syndromes associated with intellectual disability, a condition leading to deficit in cognitive functions and adaptive behaviors. Until now, the causative mechanism leading to intellectual disability is unknown and the progression of the condition is poorly understood. We first report latest advances on genetic and environmental regulation of mitochondrial function and its role in brain development. Starting from the structure, function and regulation of the oxidative phosphorylation apparatus, we review how mitochondrial biogenesis and dynamics play a central role in neurogenesis and neuroplasticity. We then discuss how dysfunctional mitochondria and alterations in reactive oxygen species homeostasis are potentially involved in the pathogenesis of various neurodevelopmental syndromes with a special focus on Down, Rett, Fragile X syndromes and autism spectrum disorders. Finally, we review and suggest novel therapeutic approaches aimed at improving intellectual disability by activating mitochondrial function and reducing oxidative stress to amiliorate the quality of life in the subjects affected.

  4. X-linked Aarskog syndrome: report on a novel FGD1 gene mutation. Executive dysfunction as part of the behavioural phenotype

    NARCIS (Netherlands)

    Verhoeven, W.M.A.; Egger, J.I.M.; Hoogeboom, A.J.M.

    2012-01-01

    X-linked Aarskog syndrome: report on a novel FGDI gene mutation. Executive dysfunction as part of the behavioural phenotype: Aarskog-Scott syndrome [OMIM 1000501 is a predominantly X-linked disorder that is phenotypically characterized by short stature, craniofacial dysmorphisms, brachydactyly and u

  5. 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.

  6. 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)

    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, P2 before operations to 275.0 mm2 after operations, where t=-5.011, P2 before operations to 128.0 mm2 after operations, where t=3.087, P2 before operation to 10.9 mm, 76.0 mm2 after operation, where t=-3.413, -2.216, respectively and P2 before operations to 76.0 mm2 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 OSAS

  7. Effects of Low-level Laser in the Treatment of Myofascial Pain Dysfunction Syndrome

    Science.gov (United States)

    Azizi, Arash; Sahebjamee, Mahnaz; Lawaf, Shirin; Jamalee, Fereydoon; Maroofi, Nader

    2007-01-01

    Background and aims Muscular pain in the facial region is the most common cause of facial pains. Myofascial pain dysfunction syndrome (MPDS) is one of the most important facial muscle disorders comprising of signs and symptoms including pain during function, tenderness in the muscles of mastication and restricted jaw movement. Due to the lack of an accepted therapeutic approach, the purpose of this paper was to find an effective treatment to decrease the pain of such patients. Considering the analgesic and anti-inflammatory action of laser therapy, the effects of low level laser (Ga-Al-As) in the treatment of MPDS patients referred to Tehran University of Medical Sciences Faculty of Dentistry, were investigated in the present study. Materials and methods This study was a quasi-experimental research. Twenty-two MPDS patients were selected from those referred to Department of Oral Medicine, Tehran University of Medical Sciences Faculty of Dentistry. Clinical examination was performed at six stages in the following order: prior to the treatment, at 2 and 4 weeks after treatment, and monthly thereafter for a 3-months follow-up. All patients underwent low-level laser therapy for 4 weeks (12 sessions). Variables such as pain severity, pain of cheek region, pain frequency, tenderness of masticatory muscles, click, and mouth opening were evaluated at each stage. Numerical variables were investigated using Analysis of Variance test for repeated measures whereas ranking variables were studied by non-parametric Kruskal-Wallis test. Results At the end of treatment period, pain severity, pain of cheek region, pain frequency, tenderness of masseter, temporalis, medial pterygoid, and lateral pterygoid muscles showed significant improvement as compared with the commencement of any treatment which continued during the 3-months post-treatment (ppain and decrease the tenderness of masticatory muscles among MPDS patients through a continuous and regular therapeutic program. PMID

  8. Diminished telomeric 3' overhangs are associated with telomere dysfunction in Hoyeraal-Hreidarsson syndrome.

    Directory of Open Access Journals (Sweden)

    Noa Lamm

    Full Text Available BACKGROUND: Eukaryotic chromosomes end with telomeres, which in most organisms are composed of tandem DNA repeats associated with telomeric proteins. These DNA repeats are synthesized by the enzyme telomerase, whose activity in most human tissues is tightly regulated, leading to gradual telomere shortening with cell divisions. Shortening beyond a critical length causes telomere uncapping, manifested by the activation of a DNA damage response (DDR and consequently cell cycle arrest. Thus, telomere length limits the number of cell divisions and provides a tumor-suppressing mechanism. However, not only telomere shortening, but also damaged telomere structure, can cause telomere uncapping. Dyskeratosis Congenita (DC and its severe form Hoyeraal-Hreidarsson Syndrome (HHS are genetic disorders mainly characterized by telomerase deficiency, accelerated telomere shortening, impaired cell proliferation, bone marrow failure, and immunodeficiency. METHODOLOGY/PRINCIPAL FINDINGS: We studied the telomere phenotypes in a family affected with HHS, in which the genes implicated in other cases of DC and HHS have been excluded, and telomerase expression and activity appears to be normal. Telomeres in blood leukocytes derived from the patients were severely short, but in primary fibroblasts they were normal in length. Nevertheless, a significant fraction of telomeres in these fibroblasts activated DDR, an indication of their uncapped state. In addition, the telomeric 3' overhangs are diminished in blood cells and fibroblasts derived from the patients, consistent with a defect in telomere structure common to both cell types. CONCLUSIONS/SIGNIFICANCE: Altogether, these results suggest that the primary defect in these patients lies in the telomere structure, rather than length. We postulate that this defect hinders the access of telomerase to telomeres, thus causing accelerated telomere shortening in blood cells that rely on telomerase to replenish their telomeres

  9. Study on acid- base disturbance in patients with posttraumatic multiple organ dysfunction syndrome

    Institute of Scientific and Technical Information of China (English)

    2000-01-01

    Objective: To investigate the classification and incidence of acid-base disturbance (ABD) in the patients with post-traumatic multiple organ dysfunction syndrome (MODS). Methods: A total of 119 patients with MODS were examined with arterial blood gas analysis and serum electrolytes detection for 675 times in this study. Results: Different types of ABD existed in 647 times out of 675 times (95.9%) of blood-gas analyses. There were 270 times (41.7%) of simple ABD, 271 times (41.9%) of double ABD and 106 times (16.4%) of triple ABD. Among which, 404 times (62.4%) were in respiratory alkalosis (RAL), 332 times (51.3%) in metabolic acidosis (MA), 227 times (35.1% ) in metabolic alkalosis (MAL) and 167 times (25.8%) in respiratory acidosis (RA). In this study, 79 cases (66.4%) out of 119cases with MODS died from these kinds of ABD. Conclusions: It suggests that in the early stage of MODS, RAL with or without hypoxemia may exist, and later on, MA or even triple ABD may occur. In order to detect and correct the primary disorders as early as possible, it is important to keep the balance of hydrolyte. The treatment of primary diseases is also important.Disorders of acid-base balance were corrected according to pH standard values, anion gap (AG) and the potential [HCO3- ] were also calculated simultaneously. When pH was more than 7.50 or lower than 7.20, it is necessary to give drugs of acidity or alkalinity to the patients with ABD to maintain pH value within a normal range.

  10. Prognosis and weaning of elderly multiple organ dysfunction syndrome patients with invasive mechanical ventilation

    Institute of Scientific and Technical Information of China (English)

    Xiao Kun; Su Longxiang; Han Bingchao; Guo Chao; Feng Lin; Jiang Zhaoxu; Wang Huijuan

    2014-01-01

    Background Elderly multiple organ dysfunction syndrome (MODS) patients receiving invasive mechanical ventilation have poor prognosis in intensive care units (ICUs).We studied the usefulness of four commonly used severity scores and extrapulmonary factors that affected weaning to predict outcome of such patients.Methods Clinical data of 197 patients on admission to ICUs (from January 2009 to June 2012) were used retrospectively.The Acute Physiology and Chronic Health Evaluation (APACHE) Ⅱ,APACHE Ⅲ,Sample Acute Physiological Score (SAPS) Ⅱ and MODS scores were calculated.All the patients were grouped into survivors and nonsurvivors according to the prognosis.Patients,who weaned from ventilator (n=154),were subdivided into a successful weaning group and a failed weaning group.The receiver operating characteristic (ROC) curves and Logistic regression was used for prognostic and weaning assessment.Results Based on the outcomes,the areas under the ROC of APACHE Ⅱ,APACHE Ⅲ,SAPS Ⅱ,and MODS were 0.837,0.833,0.824,and 0.837,respectively.The Logistic regression analysis revealed that the odds ratio (OR) of underlying lung diseases,serum albumin and creatinine,and the number of organ failures was 2.374,0.920,1.003,and 1.547.APACHE Ⅱ scores on admission performed excellent (ROC:0.921) on the weaning assessments.Conclusions APACHE Ⅱ and MODS systems were marginally better for evaluating the prognosis of elderly MODS patients who received invasive mechanical ventilation.Underlying lung diseases,serum albumin,serum creatinine and the number of organ failures were independent prognostic factors.Using the APACHE Ⅱ scores on admission before weaning may increase the likelihood of successful weaning.(ClinicalTrial.gov identifier NCT01802983).

  11. Obesity-related metabolic dysfunction in dogs: a comparison with human metabolic syndrome

    Directory of Open Access Journals (Sweden)

    Tvarijonaviciute Asta

    2012-08-01

    Full Text Available Abstract Background Recently, metabolic syndrome (MS has gained attention in human metabolic medicine given its associations with development of type 2 diabetes mellitus and cardiovascular disease. Canine obesity is associated with the development of insulin resistance, dyslipidaemia, and mild hypertension, but the authors are not aware of any existing studies examining the existence or prevalence of MS in obese dogs. Thirty-five obese dogs were assessed before and after weight loss (median percentage loss 29%, range 10-44%. The diagnostic criteria of the International Diabetes Federation were modified in order to define canine obesity-related metabolic dysfunction (ORMD, which included a measure of adiposity (using a 9-point body condition score [BCS], systolic blood pressure, fasting plasma cholesterol, plasma triglyceride, and fasting plasma glucose. By way of comparison, total body fat mass was measured by dual-energy X-ray absorptiometry, whilst total adiponectin, fasting insulin, and high-sensitivity C-reactive protein (hsCRP were measured using validated assays. Results Systolic blood pressure (P = 0.008, cholesterol (P = 0.003, triglyceride (P = 0.018, and fasting insulin (P P = 0.001. However, hsCRP did not change with weight loss. Prior to weight loss, 7 dogs were defined as having ORMD, and there was no difference in total fat mass between these dogs and those who did not meet the criteria for ORMD. However, plasma adiponectin concentration was less (P = 0.031, and plasma insulin concentration was greater (P = 0.030 in ORMD dogs. Conclusions In this study, approximately 20% of obese dogs suffer from ORMD, and this is characterized by hypoadiponectinaemia and hyperinsulinaemia. These studies can form the basis of further investigations to determine path genetic mechanisms and the health significance for dogs, in terms of disease associations and outcomes of weight loss.

  12. Managing bronchiolitis obliterans syndrome (BOS) and chronic lung allograft dysfunction (CLAD) in children: what does the future hold?

    Science.gov (United States)

    Snell, Gregory I; Paraskeva, Miranda; Westall, Glen P

    2013-08-01

    The success of pediatric lung transplantation continues to be limited by long-term graft dysfunction. Historically this has been characterized as an obstructive spirometric defect in the form of the bronchiolitis obliterans syndrome (BOS). It is recognized, however, that this does not reflect many of the other acknowledged etiologies of chronic lung dysfunction-noting it is the sum of the parts that contribute to respiratory morbidity and mortality after transplant. The term chronic lung allograft dysfunction (CLAD) has been coined to reflect these other entities and, in particular, a group of relatively recently described lung disorders called the restrictive allograft syndrome (RAS). RAS is characterized by a restrictive spirometric defect. Although these entities have not yet been studied in a pediatric setting their association with poor compliance, antibody-mediated rejection (AMR), and post-infectious lung damage (particularly viral) warrants attention by pediatric lung transplant teams. Current therapy for the BOS subset of CLAD is otherwise limited to changing immunosuppressants and avoiding excessive infectious risk by avoiding over-immunosuppression. Long-term macrolide therapy in lung transplantation is not of proven efficacy. Reviewing previous BOS studies to explore restrictive spirometric cases and joint projects via groups like the International Pediatric Lung Transplant Collaborative will be the way forward to solve this pressing problem.

  13. 儿童上气道咳嗽综合征48例临床分析%Clinical study of upper airway cough syndrome in 48 cases

    Institute of Scientific and Technical Information of China (English)

    董传莉; 谢怀珍; 张兰

    2011-01-01

    Objective: To summarize the clinical characteristics of chronic cough in children caused by upper airway cough syndrome ( UACS) . Methods: Forty-eight children diagnosed with UACS were given systematic treatment such as anti-inflammation, anti-allergy, anti-infection decongestants and so on, and the clinical data were collected and analyzed. Results: There were 26 misdiagnosed cases among the 48 cases. The misdiagnositic rate was 54. 17%. After two-week reasonable and targeted treatment, the symptoms and signs in 42 cases were obviously relieved or disappeared. The effective rate was 87. 50%. Conclusions: Chronic cough is the chief complaint of UACS associated with other complex symptoms, so it is easy to be misdiagnosed and mistreated. All the children with upper airway cough syndrome can be diagnosed through its detail history of disease, reasonable physical examination and specialist consultation. The targeted therapy can give satisfied effect to upper airway cough syndrome.%目的:总结上气道咳嗽综合征(upper airway cough syndrome,UACS)引起儿童慢性咳嗽的临床特点,提高对该病的认识.方法:确诊后采用抗炎、抗变态反应、局部使用减充血剂、抗感染等系统治疗.结果:48例UACS患儿中误诊26例,误诊率54.17%.42例患儿在2周内症状明显减轻或消失,治疗有效率87.50%.结论:UACS均以慢性咳嗽为主诉,伴随症状复杂,易被误诊误治.详细询问病史、合理检查、专科会诊有助于明确诊断.给予针对性治疗,绝大部分疗效好.

  14. Continuous plasma filtration adsorption in treatment of severe infection-induced multiple organ dysfunction syndrome.

    Science.gov (United States)

    Yin, S L; Lan, C; Pei, H; Zu, Z Q

    2016-01-01

    Multiple organ dysfunction syndrome (MODS), a high-risk disease, has a fatality rate of 70%. To improve treatment of this disease, in recent years many scholars have explored the pathological and physiological changes of MODS. To observe the curative effect of continuous plasma filtration adsorption (CPFA) in the treatment of MODS, we selected 96 patients who were diagnosed with severe infection-induced MODS and were treated in the First Affiliated Hospital of Zhengzhou University between February 2012 and October 2014 and divided them into an observation group and a control group. Besides conventional treatment, the observation group was also given CFPA in combination with high volume hemofiltration (HVHF), while the control group only received HVHF. Changes of blood routine index, balance of electrolyte and acid-base as well as vital signs were observed before and after treatment. Also, blood, kidney and blood gas were examined. For all patients, tumor necrosis factor-α (TNF-α), interleukin-6 (IL-6) and C-reactive protein (CRP) were recorded at the start of treatment (0 h), and 5 h and 10 h after treatment. It was found that both therapies could lower blood urea nitrogen (BUN) and creatinine levels and maintain balance of electrolyte and acid-base, but had no obvious influence on leukocyte, blood platelet and hematocrit. In the observation group, PaO(2)/FiO(2) and mean arterial pressure (MAP) were significantly improved after surgery (P less than 0.05), while Acute Physiology and Chronic Health Evaluation (APACHE) II score had an obvious decrease (P less than 0.05). In contrast, the control group was observed with insignificantly changed PaO(2)/FiO(2), MAP and APACHE II score (P>0.05). TNF-α, IL-6 and CRP levels of the two groups had no statistically significant difference at the start of treatment (P>0.05), but TNF-α, IL-6 and CRP levels of the observation group became remarkably lower than those of the control group 5 h and 10 h after treatment (P less than

  15. Continuous plasma filtration adsorption in treatment of severe infection-induced multiple organ dysfunction syndrome.

    Science.gov (United States)

    Yin, S L; Lan, C; Pei, H; Zu, Z Q

    2016-01-01

    Multiple organ dysfunction syndrome (MODS), a high-risk disease, has a fatality rate of 70%. To improve treatment of this disease, in recent years many scholars have explored the pathological and physiological changes of MODS. To observe the curative effect of continuous plasma filtration adsorption (CPFA) in the treatment of MODS, we selected 96 patients who were diagnosed with severe infection-induced MODS and were treated in the First Affiliated Hospital of Zhengzhou University between February 2012 and October 2014 and divided them into an observation group and a control group. Besides conventional treatment, the observation group was also given CFPA in combination with high volume hemofiltration (HVHF), while the control group only received HVHF. Changes of blood routine index, balance of electrolyte and acid-base as well as vital signs were observed before and after treatment. Also, blood, kidney and blood gas were examined. For all patients, tumor necrosis factor-α (TNF-α), interleukin-6 (IL-6) and C-reactive protein (CRP) were recorded at the start of treatment (0 h), and 5 h and 10 h after treatment. It was found that both therapies could lower blood urea nitrogen (BUN) and creatinine levels and maintain balance of electrolyte and acid-base, but had no obvious influence on leukocyte, blood platelet and hematocrit. In the observation group, PaO(2)/FiO(2) and mean arterial pressure (MAP) were significantly improved after surgery (P less than 0.05), while Acute Physiology and Chronic Health Evaluation (APACHE) II score had an obvious decrease (P less than 0.05). In contrast, the control group was observed with insignificantly changed PaO(2)/FiO(2), MAP and APACHE II score (P>0.05). TNF-α, IL-6 and CRP levels of the two groups had no statistically significant difference at the start of treatment (P>0.05), but TNF-α, IL-6 and CRP levels of the observation group became remarkably lower than those of the control group 5 h and 10 h after treatment (P less than

  16. The importance of administration of early surfactant and nasal continuous positive airway pressure in newborns with respiratory distress syndrome

    Science.gov (United States)

    Ceylan, Abdullah; Gezer, Suat; Demir, Nihat; Tuncer, Oğuz; Peker, Erdal; Kırımi, Ercan

    2014-01-01

    Aim: Mechanical ventilation is an invasive method and causes to important problems in the respiratory tract and lung parenchyma. The objective of our study was to investigate if administration of early surfactant and nasal continuous positive airway pressure (nCPAP) was superior to delayed surfactant administration and mechanical ventilation. Material and Methods: The study was conducted in the Van 100th Year University, Medical Faculty Hospital, Neonatal Intensive Care Unit. One hundred and nine infants with respiratory distrss syndrome (RDS) with a gestational age of 32 weeks and/or below were included in the study. Surfactant was given to 61 infants in the delivery room or intensive care unit and subsequently nCPAP was administered. Surfactant was administered in 48 infants in the control group and mechanical ventilation was inititated subsequently. Informed consent was obtained from the relatives of all patients and ethics committee approval was also obtained (Approval number: 03.02.2011/15). Results: There was no statistically significant difference between the two groups in terms of gestational age, birth weight, gender, height and head circumference measurements (p>0.05). The mean hospitalization time in the patients in the study group was 24.4±17.8 days, whereas the mean time of nCPAP was 28.4 (4–120) hours. In the study group, intracranial hemorrhage was found with a rate of 27.85%, bronchopulmonary dysplasia was found with a rate of 4.91%, pneumothorax was found with a rate of 3.27%, necrotizing enterocolitis was found with a rate of 3.27%, patent ductus arteriosus was found with a rate of 16.39, sepsis was found with a rate of 22.95% and retinopathy of prematurity was found with a rate of 1.63%. No statistically significant difference was found between the study and control groups in terms of the rates of complications. During the follow-up period, 17 (27.86%) patients were lost. The length of stay on mechanical ventilation in the study group was

  17. Chinese Medicinal Herbs in the Treatment of Upper Airway Cough Syndrome: A Systematic Review of Randomized, Controlled Trials.

    Science.gov (United States)

    Jiang, Hongli; Liu, Wei; Li, Guanhong; Fan, Tao; Mao, Bing

    2016-03-01

    Context • Upper airway cough syndrome (UACS), previously called postnasal drip syndrome (PNDS), has been considered universally to be one of the most common causes of chronic cough. As an important part of complementary and alternative therapy, traditional Chinese medicine (TCM) has found an exact curative therapy for chronic cough through clinical practice for thousands of years. Objective • The aim of the current review was to investigate systematically the beneficial and adverse effects of Chinese medicinal herbs (CMH) in the treatment of UACS. Design • The research team performed searches in 11 main databases from respective inception to October 31, 2015, supplemented with manual retrieval of other data. Only randomized, controlled trials (RCTs) reporting on the effectiveness of CMH in patients with UACS were included. Descriptive and quantitative data on the studies' designs, population demographics, interventions, outcomes, and methodological quality were extracted and tabulated. Methodological quality was assessed using the Cochrane risk-of-bias system and the quality of the evidence was evaluated using the Grades of Recommendation Assessment, Development, and Evaluation (GRADE) system. Participants • The reviewed studies included 1355 participants-720 in the CMH groups and 635 in the control groups-of both genders, from various professional and ethnic groups, and with a wide range of ages. They all had a duration of cough symptoms of longer than 8 wk and a clinical diagnosis of chronic cough induced by UACS that was supported by appropriate physical findings. Outcome Measures • The primary outcomes included (1) TCM recovery rate and (2) TCM cough symptom score. TCM's curative effect was calculated as the cumulative percentage of the symptom-score reduction (PSSR), estimated between baseline and postintervention. The cough symptom scores were graded according to the Chinese Criteria Guiding Principle of Clinical Research on New Drugs of TCM, with

  18. Extension of the mitochondria dysfunction hypothesis of metabolic syndrome to atherosclerosis with emphasis on the endocrine-disrupting chemicals and biophysical laws.

    Science.gov (United States)

    Lee, Hong Kyu; Shim, Eun Bo

    2013-01-29

    Metabolic syndrome and its component phenotypes, hyperglycemia, hypertension, (abdominal) obesity and hypertriglyceridemia, are major risk factors for atherosclerosis. Recently, associations between exposure to endocrine-disrupting chemicals (EDCs), mitochondrial dysfunction, metabolic syndrome and atherosclerosis have been established, suggesting a possible common mechanism underlying these phenomena. Extending a previously proposed mitochondria dysfunction theory of metabolic syndrome and using biophysical laws, such as metabolic scaling, Murray's law and fractal geometry of the vascular branching system, we propose that atherosclerosis could be explained as an ill-adaptive change occurring in nutrient-supplying arteries in response to the decreasing tissue energy demand caused by tissue mitochondrial dysfunction. Various aspects of this new hypothesis are discussed. PMID:24843625

  19. Hearing dysfunction in heterozygous Mitf(Mi-wh) /+ mice, a model for Waardenburg syndrome type 2 and Tietz syndrome.

    Science.gov (United States)

    Ni, Christina; Zhang, Deming; Beyer, Lisa A; Halsey, Karin E; Fukui, Hideto; Raphael, Yehoash; Dolan, David F; Hornyak, Thomas J

    2013-01-01

    The human deafness-pigmentation syndromes, Waardenburg syndrome (WS) type 2a, and Tietz syndrome are characterized by profound deafness but only partial cutaneous pigmentary abnormalities. Both syndromes are caused by mutations in MITF. To illuminate differences between cutaneous and otic melanocytes in these syndromes, their development and survival in heterozygous Microphthalmia-White (Mitf(Mi-wh) /+) mice were studied and hearing function of these mice characterized. Mitf(Mi-wh) /+ mice have a profound hearing deficit, characterized by elevated auditory brainstem response thresholds, reduced distortion product otoacoustic emissions, absent endocochlear potential, loss of outer hair cells, and stria vascularis abnormalities. Mitf(Mi-wh) /+ embryos have fewer melanoblasts during embryonic development than their wild-type littermates. Although cochlear melanocytes are present at birth, they disappear from the Mitf(Mi-wh) /+ cochlea between P1 and P7. These findings may provide insight into the mechanism of melanocyte and hearing loss in human deafness-pigmentation syndromes such as WS and Tietz syndrome and illustrate differences between otic and follicular melanocytes. PMID:23020089

  20. Electro-mechanical dysfunction in long QT syndrome: Role for arrhythmogenic risk prediction and modulation by sex and sex hormones.

    Science.gov (United States)

    Lang, C N; Menza, M; Jochem, S; Franke, G; Perez Feliz, S; Brunner, M; Koren, G; Zehender, M; Bugger, H; Jung, B A; Foell, D; Bode, C; Odening, K E

    2016-01-01

    Long QT syndrome (LQTS) is a congenital arrhythmogenic channelopathy characterized by impaired cardiac repolarization. Increasing evidence supports the notion that LQTS is not purely an "electrical" disease but rather an "electro-mechanical" disease with regionally heterogeneously impaired electrical and mechanical cardiac function. In the first part, this article reviews current knowledge on electro-mechanical (dys)function in LQTS, clinical consequences of the observed electro-mechanical dysfunction, and potential underlying mechanisms. Since several novel imaging techniques - Strain Echocardiography (SE) and Magnetic Resonance Tissue Phase Mapping (TPM) - are applied in clinical and experimental settings to assess the (regional) mechanical function, advantages of these non-invasive techniques and their feasibility in the clinical routine are particularly highlighted. The second part provides novel insights into sex differences and sex hormone effects on electro-mechanical cardiac function in a transgenic LQT2 rabbit model. Here we demonstrate that female LQT2 rabbits exhibit a prolonged time to diastolic peak - as marker for contraction duration and early relaxation - compared to males. Chronic estradiol-treatment enhances these differences in time to diastolic peak even more and additionally increases the risk for ventricular arrhythmia. Importantly, time to diastolic peak is particularly prolonged in rabbits exhibiting ventricular arrhythmia - regardless of hormone treatment - contrasting with a lack of differences in QT duration between symptomatic and asymptomatic LQT2 rabbits. This indicates the potential added value of the assessment of mechanical dysfunction in future risk stratification of LQTS patients. PMID:26718598

  1. Affective dysfunction in a mouse model of Rett syndrome: Therapeutic effects of environmental stimulation and physical activity.

    Science.gov (United States)

    Kondo, Mari A; Gray, Laura J; Pelka, Gregory J; Leang, Sook-Kwan; Christodoulou, John; Tam, Patrick P L; Hannan, Anthony J

    2016-02-01

    Rett syndrome (RTT) is a neurodevelopmental disorder associated with mutations in the X-linked gene encoding methyl-CpG-binding protein 2 (MeCP2) and consequent dysregulation of brain maturation. Patients suffer from a range of debilitating physical symptoms, however, behavioral and emotional symptoms also severely affect their quality of life. Here, we present previously unreported and clinically relevant affective dysfunction in the female heterozygous Mecp2(tm1Tam) mouse model of RTT (129sv and C57BL6 mixed background). The affective dysfunction and aberrant anxiety-related behavior of the Mecp2(+/-) mice were found to be reversible with environmental enrichment (EE) from 4 weeks of age. The effect of exercise alone (via wheel running) was also explored, providing the first evidence that increased voluntary physical activity in an animal model of RTT is beneficial for some phenotypes. Mecp2(+/-) mutants displayed elevated corticosterone despite decreased Crh expression, demonstrating hypothalamic-pituitary-adrenal axis dysregulation. EE of Mecp2(+/-) mice normalized basal serum corticosterone and hippocampal BDNF protein levels. The enrichment-induced rescue appears independent of the transcriptional regulation of the MeCP2 targets Bdnf exon 4 and Crh. These findings provide new insight into the neurodevelopmental role of MeCP2 and pathogenesis of RTT, in particular the affective dysfunction. The positive outcomes of environmental stimulation and physical exercise have implications for the development of therapies targeting the affective symptoms, as well as behavioral and cognitive dimensions, of this devastating neurodevelopmental disorder.

  2. Asymmetry of parental origin in long QT syndrome: preferential maternal transmission of KCNQ1 variants linked to channel dysfunction.

    Science.gov (United States)

    Itoh, Hideki; Berthet, Myriam; Fressart, Véronique; Denjoy, Isabelle; Maugenre, Svetlana; Klug, Didier; Mizusawa, Yuka; Makiyama, Takeru; Hofman, Nynke; Stallmeyer, Birgit; Zumhagen, Sven; Shimizu, Wataru; Wilde, Arthur A M; Schulze-Bahr, Eric; Horie, Minoru; Tezenas du Montcel, Sophie; Guicheney, Pascale

    2016-08-01

    Transmission distortion of disease-causing alleles in long QT syndrome (LQTS) has been reported, suggesting a potential role of KCNQ1 and KCNH2 in reproduction. This study sought to investigate parental transmission in LQTS families according to ethnicity, gene loci (LQT1-3: KCNQ1, KCNH2, and SCN5A) or severity of channel dysfunction. We studied 3782 genotyped members from 679 European and Japanese LQTS families (2748 carriers). We determined grandparental and parental origins of variant alleles in 1903 children and 624 grandchildren, and the grandparental origin of normal alleles in healthy children from 44 three-generation control families. LQTS alleles were more of maternal than paternal origin (61 vs 39%, Pgrandmother alleles. For LQT1, maternal transmission differs according to the variant level of dysfunction with 68% of maternal transmission for dominant negative or unknown functional consequence variants vs 58% for non-dominant negative and variants leading to haploinsufficiency, P<0.01; however, for LQT2 or LQT3 this association was not significant. An excess of disease-causing alleles of maternal origin, most pronounced in LQT1, was consistently found across ethnic groups. This observation does not seem to be linked to an imbalance in transmission of the LQTS subtype-specific grandparental allele, but to the potential degree of potassium channel dysfunction. PMID:26669661

  3. Endothelial dysfunction in hyperandrogenic polycystic ovary syndrome is not explained by either obesity or ectopic fat deposition.

    Science.gov (United States)

    Sprung, Victoria S; Jones, Helen; Pugh, Christopher J A; Aziz, Nabil F; Daousi, Christina; Kemp, Graham J; Green, Daniel J; Cable, N Timothy; Cuthbertson, Daniel J

    2014-01-01

    PCOS (polycystic ovary syndrome) is associated with IR (insulin resistance), increased visceral fat and NAFLD (non-alcoholic fatty liver disease) all of which may contribute to endothelial dysfunction, an early marker of CVD (cardiovascular disease) risk. Our objective was to examine the relationships between endothelial dysfunction in PCOS, the volume of AT (adipose tissue) compartments and the size of intracellular TAG (triacylglycerol) pools in liver and skeletal muscle. A total of 19 women with PCOS (means±S.D.; 26±6 years, 36±5 kg/m2) and 16 control women (31±8 years, 30±6 kg/m2) were recruited. Endothelial function was assessed in the brachial artery using FMD (flow-mediated dilation). VAT (visceral AT) and abdominal SAT (subcutaneous AT) volume were determined by whole body MRI, and liver and skeletal muscle TAG by 1H-MRS (proton magnetic resonance spectroscopy). Cardiorespiratory fitness and HOMA-IR (homoeostasis model assessment of IR) were also determined. Differences between groups were analysed using independent Student's t tests and ANCOVA (analysis of co-variance). FMD was impaired in PCOS by 4.6% [95% CI (confidence interval), 3.0-7.7; Plean and obese PCOS women. The results suggest that endothelial dysfunction in PCOS is not explained by body fat distribution or volume. FMD might be a useful independent prognostic tool to assess CVD risk in this population. PMID:23826984

  4. Electro-mechanical dysfunction in long QT syndrome: Role for arrhythmogenic risk prediction and modulation by sex and sex hormones.

    Science.gov (United States)

    Lang, C N; Menza, M; Jochem, S; Franke, G; Perez Feliz, S; Brunner, M; Koren, G; Zehender, M; Bugger, H; Jung, B A; Foell, D; Bode, C; Odening, K E

    2016-01-01

    Long QT syndrome (LQTS) is a congenital arrhythmogenic channelopathy characterized by impaired cardiac repolarization. Increasing evidence supports the notion that LQTS is not purely an "electrical" disease but rather an "electro-mechanical" disease with regionally heterogeneously impaired electrical and mechanical cardiac function. In the first part, this article reviews current knowledge on electro-mechanical (dys)function in LQTS, clinical consequences of the observed electro-mechanical dysfunction, and potential underlying mechanisms. Since several novel imaging techniques - Strain Echocardiography (SE) and Magnetic Resonance Tissue Phase Mapping (TPM) - are applied in clinical and experimental settings to assess the (regional) mechanical function, advantages of these non-invasive techniques and their feasibility in the clinical routine are particularly highlighted. The second part provides novel insights into sex differences and sex hormone effects on electro-mechanical cardiac function in a transgenic LQT2 rabbit model. Here we demonstrate that female LQT2 rabbits exhibit a prolonged time to diastolic peak - as marker for contraction duration and early relaxation - compared to males. Chronic estradiol-treatment enhances these differences in time to diastolic peak even more and additionally increases the risk for ventricular arrhythmia. Importantly, time to diastolic peak is particularly prolonged in rabbits exhibiting ventricular arrhythmia - regardless of hormone treatment - contrasting with a lack of differences in QT duration between symptomatic and asymptomatic LQT2 rabbits. This indicates the potential added value of the assessment of mechanical dysfunction in future risk stratification of LQTS patients.

  5. Viral Bronchiolitis in Young Rats Causes Small Airway Lesions that Correlate with Reduced Lung Function

    OpenAIRE

    Sorkness, Ronald L.; Renee J Szakaly; Louis A Rosenthal; Sullivan, Ruth; James E Gern; Lemanske, Robert F.; Sun, Xin

    2013-01-01

    Viral illness with wheezing during infancy is associated with the inception of childhood asthma. Small airway dysfunction is a component of childhood asthma, but little is known about how viral illness at an early age may affect the structure and function of small airways. We used a well-characterized rat model of postbronchiolitis chronic airway dysfunction to address how postinfectious small airway lesions affect airway physiological function and if the structure/function correlates persist...

  6. Executive dysfunction in children affected by obstructive sleep apnea syndrome: an observational study

    Directory of Open Access Journals (Sweden)

    Esposito M

    2013-08-01

    Full Text Available Maria Esposito,1 Lorenzo Antinolfi,1 Beatrice Gallai,2 Lucia Parisi,3 Michele Roccella,3 Rosa Marotta,4 Serena Marianna Lavano,4 Giovanni Mazzotta,5 Francesco Precenzano,1 Marco Carotenuto1 1Sleep Clinic for Developmental Age, Clinic of Child and Adolescent Neuropsychiatry, Department of Mental and Physical Health and Preventive Medicine, Second University of Naples, Naples, Italy; 2Unit of Child and Adolescent Neuropsychiatry, University of Perugia, Perugia, Italy; 3Child Neuropsychiatry, Department of Psychology, University of Palermo, Palermo, Italy; 4Department of Psychiatry, Magna Graecia University of Catanzaro, Catanzaro, Italy; 5Unit of Child and Adolescent Neuropsychiatry, AUSL Umbria 2, Terni, Italy Introduction: The role of sleep in cognitive processes can be considered clear and well established. Different reports have disclosed the association between sleep and cognition in adults and in children, as well as the impact of disturbed sleep on various aspects of neuropsychological functioning and behavior in children and adolescents. Behavioral and cognitive dysfunctions can also be considered as related to alterations in the executive functions (EF system. In particular, the EF concept refers to self-regulatory cognitive processes that are associated with monitoring and controlling both thought and goal directed behaviors. The aim of the present study is to assess the impact of the obstructive sleep apnea syndrome (OSAS on EF in a large sample of school aged children. Materials and methods: The study population comprised 79 children (51 males and 28 females aged 7–12 years (mean 9.14 ± 2.36 years with OSAS and 92 healthy children (63 males and 29 females, mean age 9.08 ± 2.44 years. To identify the severity of OSAS, an overnight respiratory evaluation was performed. All subjects filled out the Italian version of the Modified Card Sorting Test to screen EFs. Moreover, to check the degree of subjective perceived daytime sleepiness

  7. Improved Spatial Ability Correlated with Left Hemisphere Dysfunction in Turner's Syndrome. Implications for Mechanism.

    Science.gov (United States)

    Rovet, Joanne F.

    This study contrasts the performance of a 17-year-old female subject with Turner's syndrome before and after developing left temporal lobe seizures, as a means of identifying the mechanism responsible for the Turner's syndrome spatial impairment. The results revealed a deficit in spatial processing before onset of the seizure disorder. Results…

  8. Aspiration-related pulmonary syndromes.

    Science.gov (United States)

    Hu, Xiaowen; Lee, Joyce S; Pianosi, Paolo T; Ryu, Jay H

    2015-03-01

    Aspiration of foreign matter into the airways and lungs can cause a wide spectrum of pulmonary disorders with various presentations. The type of syndrome resulting from aspiration depends on the quantity and nature of the aspirated material, the chronicity, and the host responses. Aspiration is most likely to occur in subjects with a decreased level of consciousness, compromised airway defense mechanisms, dysphagia, gastroesophageal reflux, and recurrent vomiting. These aspiration-related syndromes can be categorized into airway disorders, including vocal cord dysfunction, large airway obstruction with a foreign body, bronchiectasis, bronchoconstriction, and diffuse aspiration bronchiolitis, or parenchymal disorders, including aspiration pneumonitis, aspiration pneumonia, and exogenous lipoid pneumonia. In idiopathic pulmonary fibrosis, aspiration has been implicated in disease progression and acute exacerbation. Aspiration may increase the risk of bronchiolitis obliterans syndrome in patients who have undergone a lung transplant. Accumulating evidence suggests that a causative role for aspiration is often unsuspected in patients presenting with aspiration-related pulmonary diseases; thus, many cases go undiagnosed. Herein, we discuss the broadening spectrum of these pulmonary syndromes with a focus on presenting features and diagnostic aspects. PMID:25732447

  9. Airway hyperresponsiveness in chronic obstructive pulmonary disease : A marker of asthma-chronic obstructive pulmonary disease overlap syndrome?

    NARCIS (Netherlands)

    Tkacova, Ruzena; Dai, Darlene L Y; Vonk, Judith M; Leung, Janice M; Hiemstra, Pieter S; van den Berge, Maarten; Kunz, Lisette; Hollander, Zsuzsanna; Tashkin, Donald; Wise, Robert; Connett, John; Ng, Raymond; McManus, Bruce; Paul Man, S F; Postma, Dirkje S; Sin, Don D

    2016-01-01

    BACKGROUND: The impact of airway hyperreactivity (AHR) on respiratory mortality and systemic inflammation among patients with chronic obstructive pulmonary disease (COPD) is largely unknown. We used data from 2 large studies to determine the relationship between AHR and FEV1 decline, respiratory mor

  10. [Orthostatic hypotension due to autonomous nervous system dysfunction. Review of different syndromes and their treatment].

    Science.gov (United States)

    Grubb, B P; Blanc, J J

    1999-01-01

    Dysfunction of the autonomous nervous system was poorly known to cardiologists until recently. Advances in the diagnosis of vasovagal syncope have initiated intense cardiological research into this subject. This type of syncope does not cover by far all dysfunctions of this system, all or nearly all of which have vasoplegic or bradycardic components. The aim of this article is to review the disorders of the autonomous nervous system which may interest every day practice of the cardiologist. The diagnostic advances and new treatments will require a further update of this review in the months or years to come. PMID:10065281

  11. Cholinergic Dysfunction in Fragile X Syndrome and Potential Intervention: A Preliminary 1H MRS Study

    OpenAIRE

    Kesler, Shelli R.; Lightbody, Amy A.; Reiss, Allan L.

    2009-01-01

    Males with fragile X syndrome are at risk for significant cognitive and behavioral deficits, particularly those involving executive prefrontal systems. Disruption of the cholinergic system secondary to fragile X mental retardation protein deficiency may contribute to the cognitive-behavioral impairments associated with fragile X. We measured choline in the dorsolateral prefrontal cortex of 9 males with fragile X syndrome and 9 age-matched typically developing controls using 1H magnetic resona...

  12. Cerebral hemodynamics in patients with obstructive sleep apnea syndrome monitored with near-infrared spectroscopy (NIRS) during positive airways pressure (CPAP) therapy: a pilot study

    Science.gov (United States)

    Zhang, Zhongxing; Schneider, Maja; Laures, Marco; Fritschi, Ursula; Lehner, Isabella; Qi, Ming; Khatami, Ramin

    2014-03-01

    In obstructive sleep apnea syndrome (OSA) the periodic reduction or cessation of breathing due to narrowing or occlusion of the upper airway during sleep leads to daytime symptoms and increased cardiovascular risk, including stroke. The higher risk of stroke is related to the impairment in cerebral vascular autoregulation. Continuous positive airways pressure (CPAP) therapy at night is the most effective treatment for OSA. However, there is no suitable bedside monitoring method evaluating the treatment efficacy of CPAP therapy, especially to monitor the recovery of cerebral hemodynamics. NIRS is ideally suited for non-invasive monitoring the cerebral hemodynamics during sleep. In this study, we will for first time assess dynamic changes of cerebral hemodynamics during nocturnal CPAP therapy in 3 patients with OSA using NIRS. We found periodic oscillations in HbO2, HHb, tissue oxygenation index (TOI) and blood volume associated with periodic apnea events without CPAP in all OSA patients. These oscillations were gradually attenuated and finally eliminated with the stepwise increments of CPAP pressures. The oscillations were totally eliminated in blood volume earlier than in other hemodynamic parameters. These results suggested that 1) the cerebral hemodynamic oscillations induced by OSA events can effectively be attenuated by CPAP therapy, and 2) blood flow and blood volume recovered first during CPAP therapy, followed by the recovery of oxygen consumption. Our study suggested that NIRS is a useful tool to evaluate the efficacy of CPAP therapy in patients with OSA bedside and in real time.

  13. Interventional effects of da-cheng-qi decoction on enteric nerve system in a rat model of multiple organ dysfunction syndrome

    OpenAIRE

    Xie, Ming-Zheng; Luo, Peng; Ma, Bin; Li, Lu; Wang, De-Hua; Qi, Qing-Hui

    2015-01-01

    In this study, we investigate the morphologic changes of enteric nerve system (ENS) and the expression of neurotransmitters, acetylcholine (ACh), substance P (SP), vasoactive intestinal peptide (VIP) and nitric oxide synthase (NOS), in small bowel of rats undergoing multiple organ dysfunction syndrome (MODS). Undergoing MODS, fluorescence integral optical density (IOD) value of enteric nerve fibers were significantly decreased (P

  14. Retroviral sequences related to human T-lymphotropic virus type II in patients with chronic fatigue immune dysfunction syndrome

    International Nuclear Information System (INIS)

    Chronic fatigue immune dysfunction syndrome (CFIDS) is a recently recognized illness characterized by debilitating fatigue as well as immunological and neurological abnormalities. Once thought to be caused by Epstein-Barr virus, it is now thought to have a different but unknown etiology. The authors evaluted 30 adult and pediatric CFIDS patients from six eastern states for the presence of human T-lymphotropic virus (HTLV) types I and II by Western immunoblotting, polymerase chain reaction, and in situ hybridization of blood samples. The majority of patients were positive for HTLV antibodies by Western blotting and for HTLV-II gag sequences by polymerase chain reaction and in situ hybridization. Twenty nonexposure healthy controls were negative in all assays. These data support an association between an HTLV-II-like virus and CFIDS

  15. Inflammatory cytokines regulate secretion of VEGF and chemokines by human conjunctival fibroblasts: Role in dysfunctional tear syndrome.

    Science.gov (United States)

    Nagineni, Chandrasekharam N; William, Abitha; Cherukuri, Aswini; Samuel, William; Hooks, John J; Detrick, Barbara

    2016-02-01

    Ocular surface inflammation is one of the primary mechanisms associated with dysfunctional tear syndrome (DTS), also known as dry eye disease. DTS, more prevalent in older populations, causes ocular discomfort and visual disturbance due to dryness on the surface layer in the eye. We used human conjunctival fibroblast cultures (HCJVF) to investigate the effects of inflammatory cytokines IFN-γ, TNF-α and IL-1β (ITI) on the secretions of VEGF and chemokines. Our results demonstrate the elevated secretion of angiogenic VEGF molecules by ITI without affecting anti-angiogenic molecules, PEDF, endostatin, thrombospondin and sVEGF-R1. The secretion of interferon-γ inducible chemokines, CXCL9, -10, -11 by HCJVF were significantly enhanced by ITI. Our in vitro study supports previously reported observations of elevated VEGF and chemokines in tear fluids of DTS patients, reiterating the role of inflammatory reactions in DTS. PMID:26615568

  16. Soft occlusal splint therapy in the management of myofascial pain dysfunction syndrome: A follow-up study

    Directory of Open Access Journals (Sweden)

    Naikmasur Venkatesh

    2008-01-01

    Full Text Available Background and Objectives: Myofascial Pain Dysfunction Syndrome (MPDS has been recognized as the most common, nontooth-related chronic orofacial pain condition that confronts dentists. A variety of therapies has been described in literature for its management. The present study is a prospective study carried out to evaluate the efficacy of occlusal splint therapy and compare it with pharmacotherapy (using analgesics and muscle relaxants in the management of Myofascial Pain Dysfunction Syndrome. Materials and Methods: Forty patients in the age range of 17-55 years were included in the study and randomly assigned to one of two equally sized groups, A and B. Group A patients received a combination of muscle relaxants and analgesics while Group B patients received soft occlusal splint therapy. All the patients were evaluated for GPI, VAS, maximum comfortable mouth opening, TMJ clicking and tenderness during rest and movement as well as for the number of tender muscles at the time of diagnosis, after the 1 st week of initiation of therapy and every month for three months of follow-up. Results: There was a progressive decrease in GPI scores, number of tender muscles, TMJ clicking and tenderness with various jaw movements and significant improvement in mouth opening in patients on occlusal splint therapy during the follow-up period as compared to the pharmacotherapy group. Conclusion: Occlusal splint therapy has better long-term results in reducing the symptoms of MPDS. It has better patient compliance, fewer side effects, and is more cost-effective than pharmacotherapy; hence, it can be chosen for the treatment of patients with MPDS.

  17. Role Of Adhesion Molecules Vcam-1 And Ve-Cadherin In Endothelium Dysfunction Development At Hemorrhagic Fever With Renal Syndrome

    Directory of Open Access Journals (Sweden)

    А.А. Baygildina

    2009-12-01

    Full Text Available The research goal is to determine the changes in concentration of both sVCAM-1 and VE-cadherin in blood serum of patients suffered from hemorrhagic fever with renal syndrome (HFRS. 87 patients aged 15-65 were examined. Concentrations of both sVCAM-1 and VE- cadherin in blood serum by means of "Bender MedSystems" (Austria ELISA test were determined. It was shown that in both medium severe and severe forms of HFRS statistically the significant rise of sVCAM-1 concentration in blood with high indices in oliguric period took place. Complicated form was characterized by high indices of sVCAM-1 level in fever period, extremely decreasing in concentration in oliguric period and tendency to normalizing in clinical convalescence period. VE-cadherin level in blood was predominantly lower than control in all the observed groups with the exception of fever period in group with medium severe disease form. Negative correlation of normal intensity between adhesion molecules levels in blood was revealed. In conclusion it is necessary to point out that high VCAM-1 expression by endotheliocytes evidences the development of an adhesion form of endothelial dysfunction, low VE-cadherin production in a base for development of angiogenic form of endothelial dysfunction and changes in expression of these adhesion molecules that have adaptive metabolic response to macroorganism of HFRS pathogenic action

  18. Nutrient-Induced Inflammation in Polycystic Ovary Syndrome: Role in the Development of Metabolic Aberration and Ovarian Dysfunction.

    Science.gov (United States)

    González, Frank

    2015-07-01

    A pathophysiology paradigm shift has emerged with the discovery that polycystic ovary syndrome (PCOS) is a proinflammatory state. Despite the dogma that the compensatory hyperinsulinemia of insulin resistance is the promoter of hyperandrogenism, physiological insulin infusion has no effect on androgen levels in PCOS. The dogma also does not explain the cause of hyperandrogenism and ovarian dysfunction in the 30 to 50% of women with PCOS who are of normal weight and lack insulin resistance. Inflammation is the underpinning of insulin resistance in obesity and type 2 diabetes, and may also be the cause of insulin resistance when present in PCOS. The origin of inflammation in PCOS has been ascribed to excess abdominal adiposity or frank obesity. However, nutrients such as glucose and saturated fat can incite inflammation from circulating mononuclear cells (MNC) of women with PCOS independent of excess adiposity and insulin resistance, and can also promote atherogenesis. Hyperandrogenism activates MNC in the fasting state to increase MNC sensitivity to nutrients, and is a potential mechanism for initiating inflammation in PCOS. However, chronic ovarian androgen suppression does not reduce inflammation in normal-weight women with PCOS. Direct exposure of ovarian theca cells to proinflammatory stimuli in vitro increases androgen production. These findings may be corroborated in vivo with anti-inflammatory therapy to normal-weight insulin-sensitive women with PCOS without abdominal adiposity to observe for amelioration of ovarian dysfunction.

  19. Additive Effect of Non-Alcoholic Fatty Liver Disease on Metabolic Syndrome-Related Endothelial Dysfunction in Hypertensive Patients

    Directory of Open Access Journals (Sweden)

    Maria Perticone

    2016-03-01

    Full Text Available Metabolic syndrome (MS is characterized by an increased risk of incident diabetes and cardiovascular (CV events, identifying insulin resistance (IR and endothelial dysfunction as key elements. Moreover, non-alcoholic fatty liver disease (NAFLD is bidirectionally linked with MS as a consequence of metabolic and inflammatory abnormalities. We addressed the question if the evolution in NAFLD might worsen endothelium-dependent vasodilating response in MS hypertensives. We recruited 272 Caucasian newly-diagnosed never-treated hypertensive outpatients divided into three groups according to the presence/absence of MS alone or in combination with NAFLD. MS and NAFLD were defined according to the National Cholesterol Education Program-Adult Treatment Panel III (NCEP-ATPIII and non-invasive fatty liver index, respectively. We determined IR by using the homeostasis model assessment (HOMA index. Vascular function, as forearm blood flow (FBF, was determined through strain-gauge plethysmography after intra-arterial infusion of acetylcholine (ACh and sodium nitroprusside. MS+NAFLD+ group showed worse metabolic, inflammatory and vascular profiles compared with MS−NAFLD− and MS+NAFLD−. HOMA resulted in being the strongest predictor of FBF both in the MS+NAFLD− and in the MS+NAFLD+ groups, accounting for 20.5% and 33.2% of its variation, respectively. In conclusion, we demonstrated that MS+NAFLD+ hypertensives show a worse endothelium-dependent vasodilation compared with MS+NAFLD−, allowing for consideration of NAFLD as an early marker of endothelial dysfunction in hypertensives.

  20. Mucus hypersecretion in the airway

    Institute of Scientific and Technical Information of China (English)

    WANG Ke; WEN Fu-qiang; XU Dan

    2008-01-01

    @@ Mucus hypersecretion is a distinguishing feature of Chronic intlammation diseases,such as asthma,1chronic bronchitis.2 bronchiectasis3 and cystic fibrosis.4Mucus hypersecretion leads to impairment of mucociliary clearance,abnormal bacterial plantation,mucus plug in the airway,and dysfunction of gas exchange.5

  1. Procalcitonin and C-reactive protein during systemic inflammatory response syndrome, sepsis and organ dysfunction

    OpenAIRE

    Castelli, Gian Paolo; Pognani, Claudio; Meisner, Michael; Stuani, Antonio; Bellomi, Daniela; Sgarbi, Laura

    2004-01-01

    Introduction Both C-reactive protein (CRP) and procalcitonin (PCT) are accepted sepsis markers. However, there is still some debate concerning the correlation between their serum concentrations and sepsis severity. We hypothesised that PCT and CRP concentrations are different in patients with infection or with no infection at a similar severity of organ dysfunction or of systemic inflammatory response. Patients and methods One hundred and fifty adult intensive care unit patients were observed...

  2. Prevention of multiple organ dysfunction syndrome in patients with extensive deep burns

    Institute of Scientific and Technical Information of China (English)

    盛志勇

    2002-01-01

    @@ Multiple organ dysfunction (or failure) syndrone (MODS or MOFS) remains a hurdle for us to overcome before further improvement in the survival rate can be achieved in the patients with extensive deep bums. It is, however, generally recognized that MODS is the final result of the liberation and interplay of multiple inflammatory mediators or cytokines, and there is a two-hit phenomenon in its pathogenesis.

  3. The treatment of myofascial pain-dysfunction syndrome using the biofeedback principle.

    Science.gov (United States)

    Clarke, N G; Kardachi, B J

    1977-10-01

    Facial pain is a relatively common sequel to bruxism and the biofeedback principle was used on seven subjects experiencing this syndrome. The results obtained were satisfactory and support the concept that the etiology of the M.P.D. syndrome is psychophysiological. This study showed that biofeedback is both a logical and appropriate form of treatment. However, the result with subject 7 indicates that not all patients are willing to wear the equipment but conselling and empathy probably form an equally satisfactory form of treatment. PMID:269245

  4. tudy on Diagnosis and Treatment of Infectious Multiple Organ Dysfunction Syndrome by Integrated Traditional Chinese and Western Medicine

    Institute of Scientific and Technical Information of China (English)

    2001-01-01

    Objective: To explore the diagnostic and therapeutic approach of integrated traditional Chinese and western medicine (TCM-WM) on infectious multiple organs dysfunction syndrome/multiple system and organ failure (MODS/MSOF) for elevating the successful rate of rescuing the patients. Methods: Diagnosis with western medicine and Syndrome Differentiation of TCM in 225 in-patients of acute infectious disease complicated with MODS/MSOF were conducted, and TCM treatment, based on western medical comprehensive treatment, was given to observe the effect and explore the mechanism of the TCM-WM therapy. Results: Up to the end of 1998, 161 cases of the 225 cases were successfully cured and 64 died, the mortality being 28.4%. Among them, 58 out of 140 cases of MSOF died, the mortality was accounted for 41.4%. In 106 cases conformed to the diagnostic criteria of MSOF proposed by Professor Knaus WA, USA, 52 cases were cured successfully and 54 died, the mortality being 50.94%. Conclusion:TCM-WM treatment could elevate the therapeutic effect in treating MODS, the mechanism might be through improving the hemodynamic and hemorrheologic condition of patients to relieve nail-fold microcirculation disorder; influencing the levels of cytokine and inflammatory mediator, so as to alleviate the systemic inflammatory reaction, it might also abate the inhibited condition of gastro-intestinal motility, alleviate the intestinal flora imbalance, prevent intestinal bacteria and endotoxin malposition, and protect cells from peroxidation.

  5. Respiratory and gastrointestinal dysfunctions associated with auriculo-condylar syndrome and a homozygous PLCB4 loss-of-function mutation.

    Science.gov (United States)

    Leoni, Chiara; Gordon, Christopher T; Della Marca, Giacomo; Giorgio, Valentina; Onesimo, Roberta; Perrino, Francesca; Cianfoni, Alessandro; Cerchiari, Antonella; Amiel, Jeanne; Zampino, Giuseppe

    2016-06-01

    Auriculo-Condylar Syndrome (ACS) is a craniofacial malformation syndrome characterized by external ear anomalies, hypoplasia of the mandibular condyle, temporomandibular joint abnormalities, micrognathia, and microstomia. Glossoptosis, masticatory abnormalities, orthodontic problems, and malocclusion occur in a majority of affected subjects. The clinical diagnosis is usually suggested by the pathognomonic ear appearance ("question mark ear"), consisting of a variable degree of clefting between the helix and earlobe. The genetic mechanisms underlying ACS have recently been identified. Both autosomal dominant and recessive inheritance of mutations in phospholipase C, beta 4 (PLCB4) and endothelin 1 (EDN1) have been reported along with autosomal dominant mutations in guanine nucleotide-binding protein (G protein) α inhibiting activity polypeptide 3 (GNAI3). We report 6 years of follow-up of a child with a clinical phenotype consistent with ACS due to a homozygous frameshift mutation in PLCB4. The baby presented feeding difficulties associated with failure to thrive and a complex sleep-related respiratory disorder, characterized by central and obstructive apnoeas. Our observations of this case further delineate the phenotype of ACS associated with autosomal recessive PLCB4 loss-of-function mutations, underscoring gastrointestinal dysfunction and severe sleep-related breathing abnormalities as additional features when compared to patients with heterozygous mutations with a presumed dominant negative effect. © 2016 Wiley Periodicals, Inc. PMID:27007857

  6. Cognitive dysfunction syndrome: updated behavioral and clinical evaluations as a tool to evaluate the well-being of aging dogs

    Directory of Open Access Journals (Sweden)

    S Pineda

    2014-01-01

    Full Text Available Improvements in veterinary medicine, nutrition and the enrichment of the domestic environment have helped more dogs reach to their senile phase. However, greater longevity leads to a higher prevalence of cognitive problems in our pets. Throughout their geriatric lives, dogs are more vulnerable to suffering progressive neurodegenerative dementia syndromes. Due to its high prevalence, cognitive dysfunction syndrome is one of the most studied neurodegenerative diseases. This disease is a neurobehavioral entity that presents itself in senile dogs, and it is characterized by a deficit in learning, memory and the dog's spatial awareness. It is a clinical disorder that has a significant impact on older dogs and their owners. There is a lack of reliable diagnostic tests that can guarantee the presence of the disease, provide early identification of the clinical signs of this disease, or establish a prognosis in terms of the recovery and expansion of the quality of life of the affected patient. Treatment should be directed at slowing down the progression of cognitive loss through the establishment of techniques and programs that improve physical activity, nutrition and the animal's environment. Therefore, future research on the knowledge, control and prevention of this pathology are necessary to lower the impact generated by its effects on the quality of life of the animal.

  7. Ocular Motor Indicators of Executive Dysfunction in Fragile X and Turner Syndromes

    Science.gov (United States)

    Lasker, Adrian G.; Mazzocco, Michele M. M.; Zee, David S.

    2007-01-01

    Fragile X and Turner syndromes are two X-chromosome-related disorders associated with executive function and visual spatial deficits. In the present study, we used ocular motor paradigms to examine evidence that disruption to different neurological pathways underlies these deficits. We tested 17 females with fragile X, 19 females with Turner…

  8. Frontal lobe dysfunctions in Korsakoff's syndrome and chronic alcoholism: Continuity or discontinuity?

    NARCIS (Netherlands)

    Brokate, B.; Hildebrandt, H.; Eling, P.A.T.M.; Fichtner, H.; Runge, K.; Timm, C.

    2003-01-01

    The effect of long-term heavy alcohol consumption on brain functions is still under debate. The authors investigated a sample of 17 Korsakoff amnesics, 23 alcoholics without Korsakoff's syndrome, and 21 controls with peripheral nerve diseases, matched for intelligence and education. Executive functi

  9. Is theory of mind related to social dysfunction and emotional problems in 22q11.2 deletion syndrome (velo-cardio-facial syndrome)?

    Science.gov (United States)

    Campbell, Linda E; Stevens, Angela F; McCabe, Kathryn; Cruickshank, Lynne; Morris, Robin G; Murphy, Declan G M; Murphy, Kieran C

    2011-06-01

    Social dysfunction is intrinsically involved in severe psychiatric disorders such as depression and psychosis and linked with poor theory of mind. Children with 22q11.2 deletion syndrome (22q11DS, or velo-cardio-facial syndrome) have poor social competence and are also at a particularly high risk of developing mood (40%) and psychotic (up to 30%) disorders in adolescence and young adulthood. However, it is unknown if these problems are associated with theory of mind skills, including underlying social-cognitive and social-perceptual mechanisms. The present cross-sectional study included classic social-cognitive false-belief and mentalising tasks and social-perceptual face processing tasks. The performance of 50 children with 22q11DS was compared with 31 age-matched typically developing sibling controls. Key findings indicated that, while younger children with 22q11DS showed impaired acquisition of social-cognitive skills, older children with 22q11DS were not significantly impaired compared with sibling controls. However, children with 22q11DS were found to have social-perceptual deficits, as demonstrated by difficulties in matching faces on the basis of identity, emotion, facial speech and gaze compared with sibling controls. Furthermore, performance on the tasks was associated with age, language ability and parentally rated social competence and emotional problems. These results are discussed in relation to the importance of a better delineation of social competence in this population.

  10. Novel therapeutic strategies for lung disorders associated with airway remodelling and fibrosis.

    Science.gov (United States)

    Royce, Simon G; Moodley, Yuben; Samuel, Chrishan S

    2014-03-01

    Inflammatory cell infiltration, cytokine release, epithelial damage, airway/lung remodelling and fibrosis are central features of inflammatory lung disorders, which include asthma, chronic obstructive pulmonary disease, acute respiratory distress syndrome and idiopathic pulmonary fibrosis. Although the lung has some ability to repair itself from acute injury, in the presence of ongoing pathological stimuli and/or insults that lead to chronic disease, it no longer retains the capacity to heal, resulting in fibrosis, the final common pathway that causes an irreversible loss of lung function. Despite inflammation, genetic predisposition/factors, epithelial-mesenchymal transition and mechanotransduction being able to independently contribute to airway remodelling and fibrosis, current therapies for inflammatory lung diseases are limited by their ability to only target the inflammatory component of the disease without having any marked effects on remodelling (epithelial damage and fibrosis) that can cause lung dysfunction independently of inflammation. Furthermore, as subsets of patients suffering from these diseases are resistant to currently available therapies (such as corticosteroids), novel therapeutic approaches are required to combat all aspects of disease pathology. This review discusses emerging therapeutic approaches, such as trefoil factors, relaxin, histone deacetylase inhibitors and stem cells, amongst others that have been able to target airway inflammation and airway remodelling while improving related lung dysfunction. A better understanding of the mode of action of these therapies and their possible combined effects may lead to the identification of their clinical potential in the setting of lung disease, either as adjunct or alternative therapies to currently available treatments. PMID:24513131

  11. Time Domain Measures of Heart Rate Variability to Assess Autonomic Dysfunction In Irritable Bowel Syndrome

    Directory of Open Access Journals (Sweden)

    Mohammad Nayem, Noorzahan Begum, Sultana Ferdousi

    2012-12-01

    Full Text Available AbstractBackground: Autonomic nerve function impairment is related to development of Irritable Bowel Syndrome (IBS. Time domain measures of Heart rate variability (HRV is a useful tool to measure autonomic nerve function activity.Objective: To assess autonomic nerve function activity by time domain measures of heart rate variability in patients with Irritable Bowel Syndrome.Methods: This cross sectional study was conducted in the Department of Physiology, Bangabandhu Sheikh Mujib Medical University (BSMMU, Dhaka in 2011. Ninety patients aged 20-50 years of both sex with Irritable bowel syndrome were included in the study group. They were collected from the OPD of Gastroenterology in BSMMU. Age and sex matched 30 apparently healthy subjects served as control. For assessing HRV by time domain method, Mean heart rate Mean R-R interval, Max/Min R-R interval, SDNN, RMSSD, PNN50%, NN50% were recorded by a digital Polyrite. ANOVA, independent sample t-test and Pearson’s correlation coefficient tests were performed as applicable.Results: Mean heart rate were significantly higher and Mean R-R interval, Max/Min R-R interval, SDNN, RMSSD, PNN50%, NN50% were significantly lower in IBS groups compared to those of control. Correlation analysis showed negative correlations of SDNN, RMSSD, PNN50%, NN50% with duration of disease.Conclusion: This study concludes that parasympathetic activity was reduced in patients of IBS. In addition, decreased vagal modulation is inversely related to the duration IBS.

  12. Safe performance of spinal anesthesia in a critical patient with neurofibromatosis, pectus carinatum, and temporomandibular joint dysfunction: A case report

    OpenAIRE

    Zencirci Beyazit

    2010-01-01

    Abstract Background Neurofibromatosis is a syndrome caused by the abnormal deposition of neural tissues of the nervous system, endocrine system, visceral structures, and skin. On the other hand, pectus carinatum and temporomandibular joint dysfunction are illnesses that adversly affect the respiratory system and cause additional problems in airway management. Case Presentation Fifty-eight-year-old Turkish male patient had neurofibromatosis, pectus carinatum and temporomandibular joint dysfunc...

  13. Maternal Metabolic Syndrome Programs Mitochondrial Dysfunction via Germline Changes across Three Generations

    Directory of Open Access Journals (Sweden)

    Jessica L. Saben

    2016-06-01

    Full Text Available Maternal obesity impairs offspring health, but the responsible mechanisms are not fully established. To address this question, we fed female mice a high-fat/high-sugar diet from before conception until weaning and then followed the outcomes in the next three generations of offspring, all fed a control diet. We observed that female offspring born to obese mothers had impaired peripheral insulin signaling that was associated with mitochondrial dysfunction and altered mitochondrial dynamic and complex proteins in skeletal muscle. This mitochondrial phenotype persisted through the female germline and was passed down to the second and third generations. Our results indicate that maternal programming of metabolic disease can be passed through the female germline and that the transfer of aberrant oocyte mitochondria to subsequent generations may contribute to the increased risk for developing insulin resistance.

  14. Maternal Metabolic Syndrome Programs Mitochondrial Dysfunction via Germline Changes across Three Generations.

    Science.gov (United States)

    Saben, Jessica L; Boudoures, Anna L; Asghar, Zeenat; Thompson, Alysha; Drury, Andrea; Zhang, Wendy; Chi, Maggie; Cusumano, Andrew; Scheaffer, Suzanne; Moley, Kelle H

    2016-06-28

    Maternal obesity impairs offspring health, but the responsible mechanisms are not fully established. To address this question, we fed female mice a high-fat/high-sugar diet from before conception until weaning and then followed the outcomes in the next three generations of offspring, all fed a control diet. We observed that female offspring born to obese mothers had impaired peripheral insulin signaling that was associated with mitochondrial dysfunction and altered mitochondrial dynamic and complex proteins in skeletal muscle. This mitochondrial phenotype persisted through the female germline and was passed down to the second and third generations. Our results indicate that maternal programming of metabolic disease can be passed through the female germline and that the transfer of aberrant oocyte mitochondria to subsequent generations may contribute to the increased risk for developing insulin resistance. PMID:27320925

  15. Comparison of 3 titration methods of positive airway pressure for obstructive sleep apnea syndrome: a random, single-blind and self-control clinical study

    Directory of Open Access Journals (Sweden)

    Yan LI

    2013-05-01

    Full Text Available Objective  To evaluate the efficacy and safety of polysomnography-manual continuous positive airway pressure titration (PSG-CPAP, polysomnography-automatic positive airway pressure titration (PSG-APAP, or automatic positive airway pressure titration (APAP in patients with moderate or severe simple obstructive sleep apnea syndrome (OSAS. Methods  Twenty patients with moderate or severe OSAS sequentially underwent PSG-CPAP, PSG-APAP and APAP titration 3 days apart, and then 3 primary efficacy indicators (titration pressure, remaining respiratory event and state of sleep, and safety indicators (compression injury of face skin, the subjective evaluation on degree of comfort or any complaint during titration were compared. Results  The results of efficacy indicators revealed that all PSG-APAP, APAP and PSG-CPAP were effective. Compared with the optimal pressure of PSG-CPAP titration, PSG-APAP and APAP pressures were 3.05 and 2.55cmH2O higher, respectively, in 90% of occasion (P0.05. There was no statistically significant difference between the optimal pressure of PSG-CPAP and the mean pressures of PSG-APAP and APAP (P>0.05. There was no statistically significant difference between the 3 titration methods on residue apnea/hypopnea index (AHI, P>0.05. The oxygen desaturation index (ODI decreased significantly after titration treatment (P0.05. Both PSG-CPAP and PSG-APAP titrations showed the same effects in improving ODI. Compared with basic PSG, no obvious improvement was found in sleep efficiency (SE after PSG-CPAP and PSG-APAP titration (P>0.05, however, the arousal index (ArI decreased obviously (P0.05. The results of safety indicators showed that no face skin compression injury or severe adverse event related to the titration was found in all the patients during the study. The main complaints of the patients were sleep disturbance, dryness of eyes or mouth, headache, breath holding and abdominal distention. Conclusion  Both PSG-APAP and

  16. Sustained inflation and incremental mean airway pressure trial during conventional and high-frequency oscillatory ventilation in a large porcine model of acute respiratory distress syndrome

    Directory of Open Access Journals (Sweden)

    Wunder Christian

    2006-06-01

    Full Text Available Abstract Background To compare the effect of a sustained inflation followed by an incremental mean airway pressure trial during conventional and high-frequency oscillatory ventilation on oxygenation and hemodynamics in a large porcine model of early acute respiratory distress syndrome. Methods Severe lung injury (Ali was induced in 18 healthy pigs (55.3 ± 3.9 kg, mean ± SD by repeated saline lung lavage until PaO2 decreased to less than 60 mmHg. After a stabilisation period of 60 minutes, the animals were randomly assigned to two groups: Group 1 (Pressure controlled ventilation; PCV: FIO2 = 1.0, PEEP = 5 cmH2O, VT = 6 ml/kg, respiratory rate = 30/min, I:E = 1:1; group 2 (High-frequency oscillatory ventilation; HFOV: FIO2 = 1.0, Bias flow = 30 l/min, Amplitude = 60 cmH2O, Frequency = 6 Hz, I:E = 1:1. A sustained inflation (SI; 50 cmH2O for 60s followed by an incremental mean airway pressure (mPaw trial (steps of 3 cmH2O every 15 minutes were performed in both groups until PaO2 no longer increased. This was regarded as full lung inflation. The mPaw was decreased by 3 cmH2O and the animals reached the end of the study protocol. Gas exchange and hemodynamic data were collected at each step. Results The SI led to a significant improvement of the PaO2/FiO2-Index (HFOV: 200 ± 100 vs. PCV: 58 ± 15 and TAli: 57 ± 12; p 2-reduction (HFOV: 42 ± 5 vs. PCV: 62 ± 13 and TAli: 55 ± 9; p Ali: 6.1 ± 1 vs. T75: 3.4 ± 0.4; PCV: TAli: 6.7 ± 2.4 vs. T75: 4 ± 0.5; p Conclusion A sustained inflation followed by an incremental mean airway pressure trial in HFOV improved oxygenation at a lower mPaw than during conventional lung protective ventilation. HFOV but not PCV resulted in normocapnia, suggesting that during HFOV there are alternatives to tidal ventilation to achieve CO2-elimination in an "open lung" approach.

  17. Nonalcoholic steatohepatitis as a novel player in metabolic syndrome-induced erectile dysfunction: an experimental study in the rabbit.

    Science.gov (United States)

    Vignozzi, Linda; Filippi, Sandra; Comeglio, Paolo; Cellai, Ilaria; Sarchielli, Erica; Morelli, Annamaria; Rastrelli, Giulia; Maneschi, Elena; Galli, Andrea; Vannelli, Gabriella Barbara; Saad, Farid; Mannucci, Edoardo; Adorini, Luciano; Maggi, Mario

    2014-03-25

    A pathogenic link between erectile dysfunction (ED) and metabolic syndrome (MetS) is now well established. Nonalcoholic steatohepatitis (NASH), the hepatic hallmark of MetS, is regarded as an active player in the pathogenesis of MetS-associated cardiovascular disease (CVD). This study was aimed at evaluating the relationship between MetS-induced NASH and penile dysfunction. We used a non-genomic, high fat diet (HFD)-induced, rabbit model of MetS, and treated HFD rabbits with testosterone (T), with the selective farnesoid X receptor (FXR) agonist obeticholic acid (OCA), or with the anti-TNFα mAb infliximab. Rabbits fed a regular diet were used as controls. Liver histomorphological and gene expression analysis demonstrated NASH in HFD rabbits. Several genes related to inflammation (including TNFα), activation of stellate cells, fibrosis, and lipid metabolism parameters were negatively associated to maximal acetylcholine (Ach)-induced relaxation in penis. When all these putative liver determinants of penile Ach responsiveness were tested as covariates in a multivariate model, only the association between hepatic TNFα expression and Ach response was confirmed. Accordingly, circulating levels of TNFα were increased 15-fold in HFD rabbits. T and OCA dosing in HFD rabbits both reduced TNFα liver expression and plasma levels, with a parallel increase of penile eNOS expression and responsiveness to Ach. Also neutralization of TNFα with infliximab treatment fully normalized HFD-induced hypo-responsiveness to Ach, as well as responsiveness to vardenafil, a phosphodiesterase type 5 inhibitor. Thus, MetS-induced NASH in HFD rabbits plays an active role in the pathogenesis of ED, likely through TNFα, as indicated by treatments reducing liver and circulating TNFα levels (T or OCA), or neutralizing TNFα action (infliximab), which significantly improve penile responsiveness to Ach in HFD rabbits. PMID:24486698

  18. An adult case of polysplenia syndrome associated with sinus node dysfunction, dextrocardia, and systemic venous anomalies.

    Science.gov (United States)

    Fukuda, Kentaro; Onda, Toshihito; Kimura, Yuki; Miura, Seiji; Matsumori, Rie; Masaki, Yoshiyuki; Nishino, Akihisa; Inoue, Kenji; Fujiwara, Yasumasa; Sumiyoshi, Masataka

    2015-01-01

    A 54-year-old woman was referred to our hospital for symptomatic sinus bradyarrhythmia with a sinus pause of 8 seconds. She was diagnosed with dextrocardia during childhood and discovered to have heterotaxy syndrome when she had an appendectomy during her teenager years. Chest and abdominal examinations by computed tomography showed multiple spleens located on the right side and abnormal drainages of the superior and inferior vena cava. Left isomerism was diagnosed by bilaterally bilobed lungs. Because of a patent bilateral superior vena cava, pacemaker leads were implanted using the right cephalic vein approach. Her fainting symptoms disappeared after pacemaker implantation.

  19. Barth Syndrome:From mitochondrial dysfunctions associated with aberrant production of reactive oxygen species to pluripotent stem cell studies

    Directory of Open Access Journals (Sweden)

    Ana eSaric

    2016-01-01

    Full Text Available Mutations in the gene encoding the enzyme tafazzin, TAZ, cause Barth syndrome (BTHS. Individuals with this X-linked multisystem disorder present cardiomyopathy (often dilated, skeletal muscle weakness, neutropenia, growth retardation and 3-methylglutaconic aciduria. Biopsies of the heart, liver and skeletal muscle of patients have revealed mitochondrial malformations and dysfunctions. It is the purpose of this review to summarize recent results of studies on various animal or cell models of Barth syndrome, which have characterized biochemically the strong cellular defects associated with TAZ mutations. Tafazzin is a mitochondrial phospholipid-lysophospholipid transacylase that shuttles acyl groups between phospholipids and regulates the remodeling of cardiolipin (CL, a unique inner mitochondrial membrane phospholipid dimer consisting of two phosphatidyl residues linked by a glycerol bridge. After their biosynthesis, the acyl chains of CLs may be modified in remodeling processes involving up to three different enzymes. Their characteristic acyl chain composition depends on the function of tafazzin, although the enzyme itself surprisingly lacks acyl specificity. CLs are crucial for correct mitochondrial structure and function. In addition to their function in the basic mitochondrial function of ATP production, CLs play essential roles in cardiac function, apoptosis, autophagy, cell cycle regulation and Fe-S cluster biosynthesis. Recent developments in tafazzin research have provided strong insights into the link between mitochondrial dysfunction and the production of reactive oxygen species (ROS. An important tool has been the generation of BTHS-specific induced pluripotent stem cells (iPSCs from BTHS patients. In a complementary approach, disease-specific mutations have been introduced into wild-type iPSC lines enabling direct comparison with isogenic controls. iPSC-derived cardiomyocytes were then characterized using biochemical and classical

  20. Successful treatment of a case of extensive radiation burns with multiple organ dysfunction syndrome.

    Science.gov (United States)

    Li, Yeyang; Wang, Jinlun; Li, Gang; Lin, Weihua; Li, Xiaojian; Tong, Renlian

    2013-01-01

    A patient sustained acute third-degree radiation burns over 41% of his body surface. The burns were due to occupational injury caused by an electron accelerator. Most of his wounds appeared and spread gradually during the 10th week after the radiation burn. Subsequently, severe wound infection with methicillin-resistant Staphylococcus aureus, severe pneumonia, respiratory failure, systemic inflammatory response syndrome, nephropathy, and hypoproteinemia had developed 3 months after the radiation injury. Most of the skin grafts could neither survive nor spread on the fresh wound after removing the necrotic tissue. This phenomenon resulted in many more wounds after operations, increasing the risk of wound infection. Parenteral nutrition, respiratory support with a ventilator, antibiotics for methicillin-resistant Staphylococcus aureus, steroid therapeutics for nephropathy, deeper debridement for wounds, and skin grafting were applied for treatment of this patient. The patient recovered gradually and was discharged from the hospital in good condition after 18 months. The authors suggest that deeper excision of necrotic tissue and skin grafting as well as appropriate antibiotics are principal measures to counteract systemic inflammatory response syndrome. Sufficient albumen by vein and steroid should be administered for treatment against nephropathy and for control of infection. Functions of organs should be carefully monitored to fine-tune the therapeutic programs and to minimize complications of organs.

  1. Anaesthetic Management of Children with Rubinstein-Taybi Syndrome.

    Science.gov (United States)

    Karahan, Mahmut Alp; Sert, Hüseyin; Ayhan, Zeliha; Ayhan, Bülend

    2016-06-01

    Rubinstein-Taybi syndrome (RTS) is a rare, autosomal dominant syndrome presenting with mental retardation and physical abnormalities, including broad thumbs, big and broad toes, short stature and craniofacial anomalies. Special attention was paid to the possibilities of difficult airway, aspiration pneumonia and cardiovascular dysfunction during anaesthesia. Micrognathia, retrognathia, broad nasal bridge, abnormally large or 'beak-shaped' nose, hypoplastic maxilla and small mouth-typical dysmorphic facial features are one of the biggest causes of the difficult airway in this syndrome. Approximately one-third of the affected individuals have a variety of congenital heart diseases. Recurrent respiratory infections are likely to be the result of microaspiration or gastro-oesophageal reflux in this syndrome. In this case report, we discussed the anaesthesia management of a child with RTS who underwent right endoscopic dacryocystorhinostomy. PMID:27366579

  2. A Clinical Study on Characteristics of TCM Syndrome Elements in Upper Airway Cough Syndrome%上气道咳嗽综合征中医证素特点的临床研究

    Institute of Scientific and Technical Information of China (English)

    陈文喜; 洪敏俐; 刘朝辉; 杨朝阳; 郑伟斌; 黄小华; 李灿东

    2012-01-01

    Objective: To investigate the characteristics of syndrome elements in patients with upper airway cough Syndrome (UACS). Methods: Patients with UACS who met the inclusion and exclusion criteria of UACS were observed by syndrome elements differentiation methods. The syndrome elements of the disease location and the disease nature were extracted by syndrome elements differentiation. The features of TCM pathology of UACS were analyzed with mathematical statistics methods. Results: The syndrome element of the disease location lung appeared more frequently than exterior, liver and stomach. The syndrome element of the disease nature phlegm appeared more frequently than heat, qi stagnation, dampness, cold, retention of fluid, yin asthenia, yang asthenia, qi asthenia and blood asthenia. And only the average integrals of syndrome elements lung and phlegm were above 150,which were regarded as severe lesion. Conclusion: The core disease location of UACS is lung, which could company with exterior, liver and stomach. The primary disease nature of UACS was phlegm which could be concurrence of cold and heat, deficiency and excess. The production of phlegm due to lung qi depression is the key pathological mechanism of UACS.%目的 探讨上气道咳嗽综合征(UACS)患者中医证素特征.方法 采用“证素辨证”方法,提取患者病位、病性证素,对其进行统计分析,探讨本病的中医病理特征.结果 UACS病位证素肺频数显著高于表、肝、胃;病性证素痰频数显著高于热、气滞、湿、寒、饮、阴虚、阳虚、气虚、血虚;并且只有肺和痰两个证素平均积分超过150,为重度病变.结论 UACS核心病位在肺,可兼表、肝、胃;病性以痰为主,寒热虚实互见,肺气郁而生痰是UACS的核心病理机制.

  3. Multi-organic dysfunction syndrome caused by dengue 3 in children of Neiva Huila, Colombia

    International Nuclear Information System (INIS)

    Dengue is the main arthropod -transmitted viral disease in the world with a growing number of cases in Neiva, Colombia. Clinical presentation of dengue hemorrhagic fever (DHF) is showing compromise of organs different from endothelium which could be associated to the circulating serotype and/or host immunological factors. Objective. To alert about the association of multiple organic dysfunction (MOD) and DHF. Results. MOD associated to DHF is described in three girls with an average of 16 months of age, two 7-month sold and 1 three year old. The evolution of fever at the beginning was 4 days; they had shock resistant to usual treatment with crystalloid and colloids with tachycardia,ventricular arrhythmia, increased CPK MB, AST and ALT,coagulopathy with prolonged PTT and PT but without severe thrombocytopenia, metabolic alteration with acidaemia and hypoglycemia in the three girls. Score for MOD was applied with an average of 23 and evidence of myocardial, hepatic and hematological major compromise. dengue 3 was showed by RT-PCR. Discussion. Similar reports are compared with these cases and probable pathophysiologic mechanisms are discussed. Conclusion. It has to be stressed that DHF might affect different organs, because of this definition of severity in dengue has to be reconsidered. An early thinking in different organs affected might help to introduce an opportune intervention or treatment.

  4. The effects of low level laser therapy (Ga- Al- As on myofascial pain dysfunction syndrome

    Directory of Open Access Journals (Sweden)

    Sahebjamei M

    2002-06-01

    Full Text Available Muscular pain is one of the most common causes for facial pain. Myofacial pain dysfunction"nsyndrome (MPDS is one of the most important disorders of facial area that affected patients suffer from"npain, tenderness of one or more masticatory muscles and limitation of movements. Lack of specific and"neffective therapeutic method, makes it necessary to find a treatment to decrease pain. Considering the"nanalgesic and anti- inflammatory effects of low-level laser, some investigators have recommended it for"nMPDS patient's treatment. In this study, the effects of low level laser (Ga-Al-As on MPDS patients"nreferred to dental faculty of Tehran University of Medical Sciences, were investigated. Twenty- two"nMPDS patents, with mean age 33.32 years, were treated for 4 weeks (12 visits. Clinical examinations"nwere performed in 6 stages. The results of this study showed that variables such as pain severity, cheek"npain, pain frequency and tenderness of masseter, temporal, internal and external pterygoid muscles, had a"nstatistically significant improvement at the end of treatment with low level laser (GA-A1-AS which"nlasted for three months, (follow- up period. Results of this study showed that, low-level laser therapy"nhas a sedative effect on the pain and tenderness of masticatory muscles in MPDS. Patients and having an"naccurate and regular program to complete treatment period, has an important role in MPDS"nimprovement.

  5. Hyperandrogenism and Insulin Resistance, Not Changes in Body Weight, Mediate the Development of Endothelial Dysfunction in a Female Rat Model of Polycystic Ovary Syndrome (PCOS).

    Science.gov (United States)

    Hurliman, Amanda; Keller Brown, Jennifer; Maille, Nicole; Mandala, Maurizio; Casson, Peter; Osol, George

    2015-11-01

    This study was designed to differentiate the contributions of hyperandrogenism, insulin resistance (IR), and body weight to the development of endothelial dysfunction in polycystic ovary syndrome and determine the effectiveness of insulin sensitization and antiandrogenic therapy after the establishment of vascular and metabolic dysfunction using a rat model of polycystic ovary syndrome. We hypothesized that the observed endothelial dysfunction was a direct steroidal effect, as opposed to changes in insulin sensitivity or body weight. Prepubertal female rats were randomized to the implantation of a pellet containing DHT or sham procedure. In phase 1, DHT-exposed animals were randomized to pair feeding to prevent weight gain or metformin, an insulin-sensitizing agent, from 5 to 14 weeks. In phase 2, DHT-exposed animals were randomized to treatment with metformin or flutamide, a nonsteroidal androgen receptor blocker from 12 to 16 weeks. Endothelial function was assessed by the vasodilatory response of preconstricted arteries to acetylcholine. Serum steroid levels were analyzed in phase 1 animals. Fasting blood glucose and plasma insulin were analyzed and homeostasis model assessment index calculated in all animals. Our data confirm the presence of endothelial dysfunction as well as increased body weight, hypertension, hyperinsulinemia, and greater IR among DHT-treated animals. Even when normal weight was maintained through pair feeding, endothelial dysfunction, hyperinsulinemia, and IR still developed. Furthermore, despite weight gain, treatment with metformin and flutamide improved insulin sensitivity and blood pressure and restored normal endothelial function. Therefore, the observed endothelial dysfunction is most likely a direct result of hyperandrogenism-induced reductions in insulin sensitivity, as opposed to weight gain.

  6. Variations in dysfunction of sister chromatid cohesion in esco2 mutant zebrafish reflect the phenotypic diversity of Roberts syndrome

    Directory of Open Access Journals (Sweden)

    Stefanie M. Percival

    2015-08-01

    Full Text Available Mutations in ESCO2, one of two establishment of cohesion factors necessary for proper sister chromatid cohesion (SCC, cause a spectrum of developmental defects in the autosomal-recessive disorder Roberts syndrome (RBS, warranting in vivo analysis of the consequence of cohesion dysfunction. Through a genetic screen in zebrafish targeting embryonic-lethal mutants that have increased genomic instability, we have identified an esco2 mutant zebrafish. Utilizing the natural transparency of zebrafish embryos, we have developed a novel technique to observe chromosome dynamics within a single cell during mitosis in a live vertebrate embryo. Within esco2 mutant embryos, we observed premature chromatid separation, a unique chromosome scattering, prolonged mitotic delay, and genomic instability in the form of anaphase bridges and micronuclei formation. Cytogenetic studies indicated complete chromatid separation and high levels of aneuploidy within mutant embryos. Amongst aneuploid spreads, we predominantly observed decreases in chromosome number, suggesting that either cells with micronuclei or micronuclei themselves are eliminated. We also demonstrated that the genomic instability leads to p53-dependent neural tube apoptosis. Surprisingly, although many cells required Esco2 to establish cohesion, 10-20% of cells had only weakened cohesion in the absence of Esco2, suggesting that compensatory cohesion mechanisms exist in these cells that undergo a normal mitotic division. These studies provide a unique in vivo vertebrate view of the mitotic defects and consequences of cohesion establishment loss, and they provide a compensation-based model to explain the RBS phenotypes.

  7. Comparative Value of Simple Inflammatory Markers in the Prediction of Left Ventricular Systolic Dysfunction in Postacute Coronary Syndrome Patients

    Directory of Open Access Journals (Sweden)

    Panagiotis Aggelopoulos

    2009-01-01

    Full Text Available Objectives. We sought to assess the comparative value of inflammatory markers on the occurrence of left ventricular systolic dysfunction (LVSD after an acute coronary syndrome (ACS. Methods. During 2006–2008, 760 patients with an ACS were enrolled. C-reactive protein (CRP and white blood cell (WBC count were measured during the first 12 hours of hospital admission. Results. CRP levels and WBC count were significantly higher in those who developed LVSD compared to those who did not. The analysis revealed that a 10 mg/dL increase of CRP levels and a 1000/L increase in WBC are associated with a 6% and a 7% increase in the likelihood of developing LVSD, respectively. Furthermore, WBC count at entry and CRP have almost the same predictive value for development of LVSD after an ACS (2=0.109 versus 2=0.093. Conclusions. Serum CRP levels and WBC count at entry are almost equally powerful independent predictors of LVSD, after an ACS.

  8. Variations in dysfunction of sister chromatid cohesion in esco2 mutant zebrafish reflect the phenotypic diversity of Roberts syndrome.

    Science.gov (United States)

    Percival, Stefanie M; Thomas, Holly R; Amsterdam, Adam; Carroll, Andrew J; Lees, Jacqueline A; Yost, H Joseph; Parant, John M

    2015-08-01

    Mutations in ESCO2, one of two establishment of cohesion factors necessary for proper sister chromatid cohesion (SCC), cause a spectrum of developmental defects in the autosomal-recessive disorder Roberts syndrome (RBS), warranting in vivo analysis of the consequence of cohesion dysfunction. Through a genetic screen in zebrafish targeting embryonic-lethal mutants that have increased genomic instability, we have identified an esco2 mutant zebrafish. Utilizing the natural transparency of zebrafish embryos, we have developed a novel technique to observe chromosome dynamics within a single cell during mitosis in a live vertebrate embryo. Within esco2 mutant embryos, we observed premature chromatid separation, a unique chromosome scattering, prolonged mitotic delay, and genomic instability in the form of anaphase bridges and micronuclei formation. Cytogenetic studies indicated complete chromatid separation and high levels of aneuploidy within mutant embryos. Amongst aneuploid spreads, we predominantly observed decreases in chromosome number, suggesting that either cells with micronuclei or micronuclei themselves are eliminated. We also demonstrated that the genomic instability leads to p53-dependent neural tube apoptosis. Surprisingly, although many cells required Esco2 to establish cohesion, 10-20% of cells had only weakened cohesion in the absence of Esco2, suggesting that compensatory cohesion mechanisms exist in these cells that undergo a normal mitotic division. These studies provide a unique in vivo vertebrate view of the mitotic defects and consequences of cohesion establishment loss, and they provide a compensation-based model to explain the RBS phenotypes.

  9. Elevated CaMKIIα and Hyperphosphorylation of Homer Mediate Circuit Dysfunction in a Fragile X Syndrome Mouse Model

    Directory of Open Access Journals (Sweden)

    Weirui Guo

    2015-12-01

    Full Text Available Abnormal metabotropic glutamate receptor 5 (mGluR5 function, as a result of disrupted scaffolding with its binding partner Homer, contributes to the pathophysiology of fragile X syndrome, a common inherited form of intellectual disability and autism caused by mutations in Fmr1. How loss of Fmr1 disrupts mGluR5-Homer scaffolds is unknown, and little is known about the dynamic regulation of mGluR5-Homer scaffolds in wild-type neurons. Here, we demonstrate that brief (minutes-long elevations in neural activity cause CaMKIIα-mediated phosphorylation of long Homer proteins and dissociation from mGluR5 at synapses. In Fmr1 knockout (KO cortex, Homers are hyperphosphorylated as a result of elevated CaMKIIα protein. Genetic or pharmacological inhibition of CaMKIIα or replacement of Homers with dephosphomimetics restores mGluR5-Homer scaffolds and multiple Fmr1 KO phenotypes, including circuit hyperexcitability and/or seizures. This work links translational control of an FMRP target mRNA, CaMKIIα, to the molecular-, cellular-, and circuit-level brain dysfunction in a complex neurodevelopmental disorder.

  10. The functional state of neutrophils correlates with the severity of renal dysfunction in children with hemolytic uremic syndrome.

    Science.gov (United States)

    Fernandez, Gabriela C; Gomez, Sonia A; Ramos, Maria V; Bentancor, Leticia V; Fernandez-Brando, Romina J; Landoni, Veronica I; Lopez, Laura; Ramirez, Flavia; Diaz, Mario; Alduncin, Marta; Grimoldi, Irene; Exeni, Ramon; Isturiz, Martin A; Palermo, Marina S

    2007-01-01

    Hemolytic Uremic Syndrome (HUS) is the main cause of acute renal failure in children. The high percentage of patients who develop long-term sequelae constitutes an important medical concern. The identification of parameters that correlate with the degree of renal failure may be useful to plan the best treatment soon after hospitalization. Here, we investigated the functional state of neutrophils (PMN) from HUS patients on admission, before dialysis and/or transfusion, in relation to the severity of renal impairment reached during the acute period (AP). We found that all PMN activation parameters measured in severe cases of HUS (HUS AP3) were statistically lower comparing to children with mild cases of HUS (HUS AP1). As HUS PMN phenotype and dysfunction is compatible with that of cells undergoing cell death, we also studied spontaneous apoptosis. Not only were HUS PMN not apoptotic, but HUS AP3 PMN showed an increased survival. Almost all phenotypic and functional parameters measured on PMN correlated with severity. Our results revealed a marked deactivation of PMN in severe cases of HUS, and suggest that studying the functional state of PMN could be of prognostic value.

  11. Obesity, metabolic syndrome, impaired fasting glucose, and microvascular dysfunction: a principal component analysis approach

    Directory of Open Access Journals (Sweden)

    Panazzolo Diogo G

    2012-11-01

    Full Text Available Abstract Background We aimed to evaluate the multivariate association between functional microvascular variables and clinical-laboratorial-anthropometrical measurements. Methods Data from 189 female subjects (34.0±15.5 years, 30.5±7.1 kg/m2, who were non-smokers, non-regular drug users, without a history of diabetes and/or hypertension, were analyzed by principal component analysis (PCA. PCA is a classical multivariate exploratory tool because it highlights common variation between variables allowing inferences about possible biological meaning of associations between them, without pre-establishing cause-effect relationships. In total, 15 variables were used for PCA: body mass index (BMI, waist circumference, systolic and diastolic blood pressure (BP, fasting plasma glucose, levels of total cholesterol, high-density lipoprotein cholesterol (HDL-c, low-density lipoprotein cholesterol (LDL-c, triglycerides (TG, insulin, C-reactive protein (CRP, and functional microvascular variables measured by nailfold videocapillaroscopy. Nailfold videocapillaroscopy was used for direct visualization of nutritive capillaries, assessing functional capillary density, red blood cell velocity (RBCV at rest and peak after 1 min of arterial occlusion (RBCVmax, and the time taken to reach RBCVmax (TRBCVmax. Results A total of 35% of subjects had metabolic syndrome, 77% were overweight/obese, and 9.5% had impaired fasting glucose. PCA was able to recognize that functional microvascular variables and clinical-laboratorial-anthropometrical measurements had a similar variation. The first five principal components explained most of the intrinsic variation of the data. For example, principal component 1 was associated with BMI, waist circumference, systolic BP, diastolic BP, insulin, TG, CRP, and TRBCVmax varying in the same way. Principal component 1 also showed a strong association among HDL-c, RBCV, and RBCVmax, but in the opposite way. Principal component 3 was

  12. Vascular dysfunction in a mouse model of Rett syndrome and effects of curcumin treatment.

    Directory of Open Access Journals (Sweden)

    Anna Panighini

    Full Text Available Mutations in the coding sequence of the X-linked gene MeCP2 (Methyl CpG-binding protein are present in around 80% of patients with Rett Syndrome, a common cause of intellectual disability in female and to date without any effective pharmacological treatment. A relevant, and so far unexplored feature of RTT patients, is a marked reduction in peripheral circulation. To investigate the relationship between loss of MeCP2 and this clinical aspect, we used the MeCP2 null mouse model B6.129SF1-MeCP2tm1Jae for functional and pharmacological studies. Functional experiments were performed on isolated resistance mesenteric vessels, mounted on a pressurized myograph. Vessels from female MeCP2(+/- mice show a reduced endothelium-dependent relaxation, due to a reduced Nitric Oxide (NO availability secondary to an increased Reactive Oxygen Species (ROS generation. Such functional aspects are associated with an intravascular increase in superoxide anion production, and a decreased vascular eNOS expression. These alterations are reversed by curcumin administration (5% (w/w dietary curcumin for 21 days, which restores endothelial NO availability, decreases intravascular ROS production and normalizes vascular eNOS gene expression. In conclusion our findings highlight alterations in the vascular/endothelial system in the absence of a correct function of MeCP2, and uncover related cellular/molecular mechanisms that are rescued by an anti-oxidant treatment.

  13. Fragile X-Associated Tremor/Ataxia Syndrome (FXTAS) Motor Dysfunction Modeled in Mice.

    Science.gov (United States)

    Foote, Molly; Arque, Gloria; Berman, Robert F; Santos, Mónica

    2016-10-01

    Fragile X-associated tremor/ataxia syndrome (FXTAS) is a late-onset neurodegenerative disorder that affects some carriers of the fragile X premutation (PM). In PM carriers, there is a moderate expansion of a CGG trinucleotide sequence (55-200 repeats) in the fragile X gene (FMR1) leading to increased FMR1 mRNA and small to moderate decreases in the fragile X mental retardation protein (FMRP) expression. The key symptoms of FXTAS include cerebellar gait ataxia, kinetic tremor, sensorimotor deficits, neuropsychiatric changes, and dementia. While the specific trigger(s) that causes PM carriers to progress to FXTAS pathogenesis remains elusive, the use of animal models has shed light on the underlying neurobiology of the altered pathways involved in disease development. In this review, we examine the current use of mouse models to study PM and FXTAS, focusing on recent advances in the field. Specifically, we will discuss the construct, face, and predictive validities of these PM mouse models, the insights into the underlying disease mechanisms, and potential treatments.

  14. Sleep disturbances and autonomic dysfunction in patients with postural orthostatic tachycardia syndrome

    Directory of Open Access Journals (Sweden)

    Julia eMallien

    2014-07-01

    Full Text Available Many patients with Postural Tachycardia Syndrome (PoTS suffer from fatigue, daytime sleepiness and sleeping disturbances. The objective of this study was to compare subjective and objective sleep quality of PoTS patients with a group of healthy controls. All Patients completed a Pittsburgh Sleep Quality Index questionnaire and the Epworth Sleepiness Scale. The patients sleep architecture, heart rate and heart rate variability measurements were taken during one night at the sleep laboratorium. All Data was collected at the Sleep Unit, at Helios Klinikum Wuppertal. 38 patients diagnosed with PoTS were compared to 31 healthy controls, matched in age and gender. Patients with PoTS reached significantly higher scores in sleep questionnaires, which means that they were more sleepy and had a lower sleep qualitiy. Polysomnography showed a significantly higher proportion of stage 2 sleep. The results of heart rate variability analysis in different sleep stages confirmed changes in autonomic activity in both groups. PoTS patients, however, showed a diminished variability of the LF band, HF band and LF/HF ratio in different sleep stages. It can therefore be gathererd that PoTS could be considered as potential differential diagnosis for sleep disturbances since PoTS patients had a subjective diminished sleep quality, reached higher levels of daytime sleepiness and showed a higher proportion of stage 2 sleep. PoTS patients showed furthermore a reduction of LF/HF ratio variability in different sleep stages.

  15. Neuroendorine and Epigentic Mechanisms Subserving Autonomic Imbalance and HPA Dysfunction in the Metabolic Syndrome

    Science.gov (United States)

    Lemche, Erwin; Chaban, Oleg S.; Lemche, Alexandra V.

    2016-01-01

    Impact of environmental stress upon pathophysiology of the metabolic syndrome (MetS) has been substantiated by epidemiological, psychophysiological, and endocrinological studies. This review discusses recent advances in the understanding of causative roles of nutritional factors, sympathomedullo-adrenal (SMA) and hypothalamic-pituitary adrenocortical (HPA) axes, and adipose tissue chronic low-grade inflammation processes in MetS. Disturbances in the neuroendocrine systems for leptin, melanocortin, and neuropeptide Y (NPY)/agouti-related protein systems have been found resulting directly in MetS-like conditions. The review identifies candidate risk genes from factors shown critical for the functioning of each of these neuroendocrine signaling cascades. In its meta-analytic part, recent studies in epigenetic modification (histone methylation, acetylation, phosphorylation, ubiquitination) and posttranscriptional gene regulation by microRNAs are evaluated. Several studies suggest modification mechanisms of early life stress (ELS) and diet-induced obesity (DIO) programming in the hypothalamic regions with populations of POMC-expressing neurons. Epigenetic modifications were found in cortisol (here HSD11B1 expression), melanocortin, leptin, NPY, and adiponectin genes. With respect to adiposity genes, epigenetic modifications were documented for fat mass gene cluster APOA1/C3/A4/A5, and the lipolysis gene LIPE. With regard to inflammatory, immune and subcellular metabolism, PPARG, NKBF1, TNFA, TCF7C2, and those genes expressing cytochrome P450 family enzymes involved in steroidogenesis and in hepatic lipoproteins were documented for epigenetic modifications. PMID:27147943

  16. Fragile X-Associated Tremor/Ataxia Syndrome (FXTAS) Motor Dysfunction Modeled in Mice.

    Science.gov (United States)

    Foote, Molly; Arque, Gloria; Berman, Robert F; Santos, Mónica

    2016-10-01

    Fragile X-associated tremor/ataxia syndrome (FXTAS) is a late-onset neurodegenerative disorder that affects some carriers of the fragile X premutation (PM). In PM carriers, there is a moderate expansion of a CGG trinucleotide sequence (55-200 repeats) in the fragile X gene (FMR1) leading to increased FMR1 mRNA and small to moderate decreases in the fragile X mental retardation protein (FMRP) expression. The key symptoms of FXTAS include cerebellar gait ataxia, kinetic tremor, sensorimotor deficits, neuropsychiatric changes, and dementia. While the specific trigger(s) that causes PM carriers to progress to FXTAS pathogenesis remains elusive, the use of animal models has shed light on the underlying neurobiology of the altered pathways involved in disease development. In this review, we examine the current use of mouse models to study PM and FXTAS, focusing on recent advances in the field. Specifically, we will discuss the construct, face, and predictive validities of these PM mouse models, the insights into the underlying disease mechanisms, and potential treatments. PMID:27255703

  17. Effects of Continuous Positive Airway Pressure on Cognitive Deficits in Middle-aged Patients with Obstructive Sleep Apnea Syndrome: A Meta-analysis of Randomized Controlled Trials

    Institute of Scientific and Technical Information of China (English)

    Yue-Ying Pan; Yan Deng; Xiu Xu; Ya-Ping Liu; Hui-Guo Liu

    2015-01-01

    Background:Current views on continuous positive airway pressure (CPAP) treatment to improve the cognitive deficits of patients with obstructive sleep apnea syndrome (OSAS) are controversial,so we performed a meta-analysis.Methods:A comprehensive literature search was tmdertaken in PubMed,CINAHL,Medline,PsycInfo,EMBASE,Cochrane Library,CNKI,WanFang,VIP,and CBMdisc for studies published from June 1971 to July 2014.The outcome measures included neuropsychological tests of the 7 cognitive domains detailed below.Results:After screening the titles and abstracts and thoroughly reading the full text,we obtained 13 studies with little risk of bias that incorporated 1744 middle-aged obese participants with mild to severe OSAS.The studies were published from 1994 to 2012.Treatment durations varied from 1 to 24 weeks.The effect sizes of attention,vigilance,processing speed,working memory,memory,verbal fluency,and visuoconstructive skills domains were-0.10 (P =0.24),-0.12 (P =0.04),-0.08 (P =0.16),0.00 (P =0.95),-0.04 (P =0.30),-0.06 (P =0.34),and-0.01 (P =0.92),respectively.Conclusions:Cognition partially improved in patients with OSAS after CPAP treatment.The only domain with significant improvement was vigilance.Rigorous randomized controlled trials need to be performed to obtain clear results.

  18. Effect of continuous positive airway pressure treatment on serum adiponectin level and mean arterial pressure in male patients with obstructive sleep apnea syndrome

    Institute of Scientific and Technical Information of China (English)

    ZHANG Xi-long; YIN Kai-sheng; LI Chong; JIA En-zhi; LI Yan-qun; GAO Zhao-fang

    2007-01-01

    Background Recent research suggested that obstructive sleep apnea syndrome (OSAS) might be independently associated with hypoadiponectinemia, which was linked to some complications of OSAS, such as hypertension, diabetes,etc. This study was conducted to investigate the effect of continuous positive airway pressure (CPAP) treatment on changes of both serum adiponectin levels and mean arterial pressure and their possible links in male OSAS patients.Methods Twenty-three adult male patients with moderate-to-severe OSAS but without obesity, coronary heart disease and diabetes were recruited. Their blood sampleswere collected and moming mean arterial pressure (MAP) was measured before CPAP treatment and on day 3, 7, 14 of CPAP treatment respectively. The serum adiponectin concentration was tested with radioimmunoassay.Results Compared with the serum adiponectin level before CPAP treatment, no significant change was found in OSAS patients on day 3 and day 7 of CPAP treatment (P>0.05). It was not until day 14 of CPAP treatment did a significant elevation in serum adiponectin level occur (P<0.01). Meanwhile, the MAP showed no statistically significant difference among its levels before CPAP, on day 3 and day 7 of CPAP treatment (P>0.05). However, on day 14 of CPAP treatment,a significantly lower MAP than that obtained before treatment was observed (P<0.05).Conclusions CPAP treatment can gradually reverse hypoadiponectinemia and reduce MAP in OSAS patients.Hypoadiponectinemia might be involved in the pathogenesis of OSAS-mediated hypertension.

  19. Measurement of the square measure of the pharynx and the positional diagnosis of airway obstruction during obstructive sleep apnea syndrome by dynamic MRI

    International Nuclear Information System (INIS)

    The purpose of this study was to apply dynamic MRI for the positional diagnosis of airway obstruction during snoring and sleep apnea and to compare the apnea hypopnea index (AHI) and the square measure of the pharynx obtained before and after laser-assisted uvula-palate-pharyngoplasty (LAUP). From December 1997 to October 1998, dynamic MRI and overnight monitoring were performed at the hospital of Tokyo Medical University on 42 patients who complained of snoring and symptoms related to sleep apnea syndrome (SAS). Of the 42 patients, four exhibited collapse at the position of the soft palate (soft palate type) as diagnosed by dynamic MRI, and four exhibited collapse at the position of the soft palate as well as the tongue (complex type). LAUP was performed on these eight patients with obstructive SAS (OSAS). After LAUP, the AHI of these eight patients with OSAS decreased significantly (p<0.05). The square measure of the pharynx of these eight patients was increased (p<0.01). The AHI of all four patients with soft-palate obstruction decreased, and the square measure of the pharynx of three of these four patients increased. The AHI of three of four patients with the complex type decreased, while the square measure of the pharynx of two of these four patients increased. (author)

  20. Measurement of the square measure of the pharynx and the positional diagnosis of airway obstruction during obstructive sleep apnea syndrome by dynamic MRI

    Energy Technology Data Exchange (ETDEWEB)

    Ozuki, Taizo; Ohkubo, Yasuo; Abe, Kimihiko [Tokyo Medical Coll. (Japan)

    2000-11-01

    The purpose of this study was to apply dynamic MRI for the positional diagnosis of airway obstruction during snoring and sleep apnea and to compare the apnea hypopnea index (AHI) and the square measure of the pharynx obtained before and after laser-assisted uvula-palate-pharyngoplasty (LAUP). From December 1997 to October 1998, dynamic MRI and overnight monitoring were performed at the hospital of Tokyo Medical University on 42 patients who complained of snoring and symptoms related to sleep apnea syndrome (SAS). Of the 42 patients, four exhibited collapse at the position of the soft palate (soft palate type) as diagnosed by dynamic MRI, and four exhibited collapse at the position of the soft palate as well as the tongue (complex type). LAUP was performed on these eight patients with obstructive SAS (OSAS). After LAUP, the AHI of these eight patients with OSAS decreased significantly (p<0.05). The square measure of the pharynx of these eight patients was increased (p<0.01). The AHI of all four patients with soft-palate obstruction decreased, and the square measure of the pharynx of three of these four patients increased. The AHI of three of four patients with the complex type decreased, while the square measure of the pharynx of two of these four patients increased. (author)

  1. Effects of a Physical Activity Program on Markers of Endothelial Dysfunction, Oxidative Stress, and Metabolic Status in Adolescents with Metabolic Syndrome

    Science.gov (United States)

    Camarillo-Romero, Eneida; Dominguez-Garcia, Ma Victoria; Amaya-Chavez, Araceli; Camarillo-Romero, Maria del Socorro; Talavera-Piña, Juan; Huitron-Bravo, Gerardo; Majluf-Cruz, Abraham

    2012-01-01

    The metabolic syndrome (MetS) is a precursor of diabetes. Physical activity (PA) improves endothelial dysfunction and may benefit patients with MetS. Aims. To evaluate the effect of a physical activity (PA) program on markers of endothelial dysfunction and oxidative stress in adolescents with (MetS). Methods. We carried out a cohort study of 38 adolescents with and without MetS (18 females and 20 males). All participants completed a 3-month PA program. All variables of the MetS as well as markers of endothelial dysfunction and oxidative stress tests were evaluated. Results. Females with and without MetS showed significant differences for almost all components of the MetS, whereas males were significantly different in half of the components. After the PA program, components of the MetS were not different from baseline values except for HDL-C levels. Some baseline endothelial dysfunction markers were significantly different among adolescents with and without MetS; however, after the PA program, most of these markers significantly improved in subjects with and without MetS. Conclusion. PA improves the markers of endothelial dysfunction in adolescents with MetS although other changes in the components of the MetS were not observed. Perhaps the benefits of PA on all components of MetS would appear after a PA program with a longer duration. PMID:22888450

  2. Advances in the Management of Meconium Aspiration Syndrome

    OpenAIRE

    Sindhu Sivanandan; Soraisham, Amuchou S.; Kamala Swarnam

    2011-01-01

    Meconium aspiration syndrome (MAS) is a common cause of severe respiratory distress in term infants, with an associated highly variable morbidity and mortality. MAS results from aspiration of meconium during intrauterine gasping or during the first few breaths. The pathophysiology of MAS is multifactorial and includes acute airway obstruction, surfactant dysfunction or inactivation, chemical pneumonitis with release of vasoconstrictive and inflammatory mediators, and persistent pulmonary hype...

  3. Developmental SHP2 dysfunction underlies cardiac hypertrophy in Noonan syndrome with multiple lentigines.

    Science.gov (United States)

    Lauriol, Jessica; Cabrera, Janel R; Roy, Ashbeel; Keith, Kimberly; Hough, Sara M; Damilano, Federico; Wang, Bonnie; Segarra, Gabriel C; Flessa, Meaghan E; Miller, Lauren E; Das, Saumya; Bronson, Roderick; Lee, Kyu-Ho; Kontaridis, Maria I

    2016-08-01

    Hypertrophic cardiomyopathy is a common cause of mortality in congenital heart disease (CHD). Many gene abnormalities are associated with cardiac hypertrophy, but their function in cardiac development is not well understood. Loss-of-function mutations in PTPN11, which encodes the protein tyrosine phosphatase (PTP) SHP2, are implicated in CHD and cause Noonan syndrome with multiple lentigines (NSML), a condition that often presents with cardiac hypertrophic defects. Here, we found that NSML-associated hypertrophy stems from aberrant signaling mechanisms originating in developing endocardium. Trabeculation and valvular hyperplasia were diminished in hearts of embryonic mice expressing a human NSML-associated variant of SHP2, and these defects were recapitulated in mice expressing NSML-associated SHP2 specifically in endothelial, but not myocardial or neural crest, cells. In contrast, mice with myocardial- but not endothelial-specific NSML SHP2 expression developed ventricular septal defects, suggesting that NSML-associated mutations have both cell-autonomous and nonautonomous functions in cardiac development. However, only endothelial-specific expression of NSML-associated SHP2 induced adult-onset cardiac hypertrophy. Further, embryos expressing the NSML-associated SHP2 mutation exhibited aberrant AKT activity and decreased downstream forkhead box P1 (FOXP1)/FGF and NOTCH1/EPHB2 signaling, indicating that SHP2 is required for regulating reciprocal crosstalk between developing endocardium and myocardium. Together, our data provide functional and disease-based evidence that aberrant SHP2 signaling during cardiac development leads to CHD and adult-onset heart hypertrophy. PMID:27348588

  4. Association of inflammation and endothelial dysfunction with metabolic syndrome, prediabetes and diabetes in adults from Inner Mongolia, China

    Directory of Open Access Journals (Sweden)

    Kelly Tanika N

    2011-10-01

    Full Text Available Abstract Background We examined the association of biomarkers of inflammation and endothelial dysfunction with diabetes and metabolic syndrome (MetS in persons from Inner Mongolia. Methods A cross-sectional study was conducted among 2,536 people aged 20 years and older from Inner Mongolia, China. Overnight fasting blood samples were obtained to measure plasma concentrations of high sensitivity C-reactive protein (hsCRP, soluble inter-cellular adhesion molecule-1 (sICAM-1, sE-selectin, angiotensin II, high density lipoprotein cholesterol, triglycerides, and blood glucose. Waist circumference and blood pressure were measured by trained staff. MetS was defined according to the modified ATP III definition for Asians. Elevated level of the biomarker was defined as values in the upper tertile of the distribution. Participants were categorized into one of four groups based on the presence or absence of metabolic and glycemic abnormalities: 1 free of prediabetes, diabetes and MetS (reference group, 2 prediabetes or diabetes only, 3 MetS without prediabetes or diabetes, and 4 MetS plus prediabetes or diabetes. The multivariable models are adjusted for age, gender, smoking, drinking, family history of hypertension, and body mass index. Results Among study participants, 18.5% had prediabetes, 3.6% had diabetes, and 27.4% of the entire study population had 3 or more components of the MetS. Elevated hsCRP was associated with an increased odds of prediabetes or diabetes only, MetS without prediabetes or diabetes, and MetS plus prediabetes or diabetes with multivariable adjusted odds ratios (95% confidence intervals of 2.3 (1.7-3.1, 3.0 (2.4-3.8, and 5.8 (4.5-7.5, respectively. Elevated sICAM-1 was associated with increased odds (95% CI of prediabetes or diabetes only (2.1, 1.6-2.9 and MetS plus prediabetes or diabetes (4.2, 3.2-5.3 but was not associated with MetS alone. Elevated sE-selectin was associated with a modestly increased risk of MetS (OR 1.7, 95

  5. Comparison of the Pharyngeal Airway Volume between Non-Syndromic Unilateral Cleft Palate and Normal Individuals Using Cone Beam Computed Tomography

    Directory of Open Access Journals (Sweden)

    Shoaleh Shahidi

    2016-09-01

    Full Text Available Statement of the Problem: Individuals with cleft lip and cleft palate mostly have airway problems. Introduction of cone beam computed tomography (CBCT and imaging software has provided the opportunity for a more precisely evaluating 3D volume of the airway. Purpose: The purpose of this study was to analyze and compare 3D the pharyngeal airway volumes of cleft palate patients with normal individuals using CBCT. Materials and Method: 30 complete cleft palate patients were selected from the Department of Orthodontics; Dental University (Shiraz, Iran who had CBCT scans of the head. The control group included 30 individuals with Class I angle occlusion who were matched for age and gender with the experimental group. ITK-SNAP 2.4.0 PC software was used to build 3D models of the airways for the subjects and measuring airway volumes. The statistical analyses were performed using SPSS software (version 19. Mann-Whitney test was adopted with p< 0.05 as statistical significance. Results: The average volume of the pharyngeal airway of cleft group was 18.6 cm3, with mean volumes of 6.8 cm3 for the superior component and 11.3 cm3 for the inferior component. The total and superior airway volume of cleft group were significantly lower than non-cleft groups (p= 0.008, p= 0.00, respectively but the inferior airway volumes were not significantly different between the cleft and non-cleft groups. There was a significant and positive correlation between superior airway volume and inferior airway volume in cleft palate patients (r=+0.786, p< 0.001 and control group (r=+0.575, p= 0.001. Conclusion: 3D analysis showed that the nasal and total airway was restricted in individuals with cleft palate but the inferior airway was not compromised in these individuals. This would be a crucial data to be considered for surgeons during surgical planning.

  6. Natural killer cell dysfunction is a distinguishing feature of systemic onset juvenile rheumatoid arthritis and macrophage activation syndrome.

    Science.gov (United States)

    Villanueva, Joyce; Lee, Susan; Giannini, Edward H; Graham, Thomas B; Passo, Murray H; Filipovich, Alexandra; Grom, Alexei A

    2005-01-01

    Macrophage activation syndrome (MAS) has been reported in association with many rheumatic diseases, most commonly in systemic juvenile rheumatoid arthritis (sJRA). Clinically, MAS is similar to hemophagocytic lymphohistiocytosis (HLH), a genetic disorder with absent or depressed natural killer (NK) function. We have previously reported that, as in HLH, patients with MAS have profoundly decreased NK activity, suggesting that this abnormality might be relevant to the pathogenesis of the syndrome. Here we examined the extent of NK dysfunction across the spectrum of diseases that comprise juvenile rheumatoid arthritis (JRA). Peripheral blood mononuclear cells (PBMC) were collected from patients with pauciarticular (n = 4), polyarticular (n = 16), and systemic (n = 20) forms of JRA. NK cytolytic activity was measured after co-incubation of PBMC with the NK-sensitive K562 cell line. NK cells (CD56+/T cell receptor [TCR]-alphabeta-), NK T cells (CD56+/TCR-alphabeta+), and CD8+ T cells were also assessed for perforin and granzyme B expression by flow cytometry. Overall, NK cytolytic activity was significantly lower in patients with sJRA than in other JRA patients and controls. In a subgroup of patients with predominantly sJRA, NK cell activity was profoundly decreased: in 10 of 20 patients with sJRA and in only 1 of 20 patients with other JRA, levels of NK activity were below two standard deviations of pediatric controls (P = 0.002). Some decrease in perforin expression in NK cells and cytotoxic T lymphocytes was seen in patients within each of the JRA groups with no statistically significant differences. There was a profound decrease in the proportion of circulating CD56bright NK cells in three sJRA patients, a pattern similar to that previously observed in MAS and HLH. In conclusion, a subgroup of patients with JRA who have not yet had an episode of MAS showed decreased NK function and an absence of circulating CD56bright population, similar to the abnormalities observed

  7. Dimethyl sulfoxide inhibits zymosan-induced intestinal inflammation and barrier dysfunction

    OpenAIRE

    Li, Yu-Meng; Wang, Hai-Bin; Zheng, Jin-Guang; Bai, Xiao-Dong; Zhao, Zeng-Kai; Li, Jing-yuan; Hu, Sen

    2015-01-01

    AIM: To investigate whether dimethyl sulfoxide (DMSO) inhibits gut inflammation and barrier dysfunction following zymosan-induced systemic inflammatory response syndrome and multiple organ dysfunction syndrome.

  8. Syndrome of iron pill inhalation in four patients with accidental tablet aspiration: Severe airway complications are described

    Directory of Open Access Journals (Sweden)

    U. Caterino

    2015-01-01

    Full Text Available Iron pill inhalation represents a uncommon cause of syntomatic endobronchial foreign bodies. Unlike foreign body, the direct contact of iron tablet onto the bronchial mucosa results in severe bronchial damage in addition to obstruction and local irritation. Four patients with Iron Pill Inhalation Syndrome are described. All but one patient developed irreversible bronchial stenosis as late post inflammatory complication. Bronchoscopic features and clinical evolution are described in order to reduce the risk of severe side-effects in patients highly suspected for iron pill aspiration.

  9. Roles of calcium and IP3 in impaired colon contractility of rats following multiple organ dysfunction syndrome

    Directory of Open Access Journals (Sweden)

    C. Zheyu

    2007-10-01

    Full Text Available The purpose of the present study was to explore changes in rat colon motility, and determine the roles of calcium and inositol (1,4,5-triphosphate (IP3 in colon dysmotility induced by multiple organ dysfunction syndrome (MODS caused by bacteria peritonitis. The number of stools, the contractility of the muscle strips and the length of smooth muscle cells (SMC in the colon, the concentration of calcium and IP3 in SMC, and serum nitric oxide were measured. Number of stools, fecal weight, IP3 concentration in SMC and serum nitric oxide concentration were 0.77 ± 0.52 pellets, 2.51 ± 0.39 g, 4.14 ± 2.07 pmol/tube, and 113.95 ± 37.89 µmol/L, respectively, for the MODS group (N = 11 vs 1.54 ± 0.64 pellets, 4.32 ± 0.57 g, 8.19 ± 3.11 pmol/tube, and 37.42 ± 19.56 µmol/L for the control group (N = 20; P < 0.05. After treatment with 0.1 mM acetylcholine and 0.1 M potassium chloride, the maximum contraction stress of smooth muscle strips, the length of SMC and the changes of calcium concentration were 593 ± 81 and 458 ± 69 g/cm³, 48.1 ± 11.8 and 69.2 ± 15.7 µM, 250 ± 70 and 167 ± 48%, respectively, for the control group vs 321 ± 53 and 284 ± 56 g/cm³, 65.1 ± 18.5 and 87.2 ± 23.7 µM, 127 ± 35 and 112 ± 35% for the MODS group (P < 0.05. Thus, colon contractility was decreased in MODS, a result possibly related to reduced calcium concentration and IP3 in SMC.

  10. Temporo-parietal dysfunction in Tourette syndrome: Insights from an fMRI study of Theory of Mind.

    Science.gov (United States)

    Eddy, Clare M; Cavanna, Andrea E; Rickards, Hugh E; Hansen, Peter C

    2016-10-01

    Tourette syndrome (TS) is a neurodevelopmental disorder characterized by tics, repetitive movements and vocalizations which are prompted by a sensory-cognitive premonitory urge. Complex tics include environmentally dependent social behaviors such as echoing of other people's speech and actions. Recent studies have suggested that adults with TS can show differences to controls in Theory of Mind (ToM): reasoning about mental states (e.g. beliefs, emotions). In this study, twenty-five adults with uncomplicated TS (no co-morbid disorders, moderate tic severity), and twenty-five healthy age and gender matched controls were scanned with fMRI during an established ToM task. Neural activity was contrasted across ToM trials involving reasoning about false-belief, and matched trials requiring judgments about physical states rather than mental states. Contrasting task conditions uncovered differential fMRI activation in TS during ToM involving the right temporo-parietal junction (TPJ), right amygdala and posterior cingulate. Further analysis revealed that activity within the right TPJ as localised by this task covaried with the severity of symptoms including echophenomena, impulse control problems and premonitory urges in TS. Amygdala activation was also linked to premonitory urges, while activity in the left TPJ during ToM was linked to ratings of non-obscene socially inappropriate symptoms. These findings indicate that patients with TS exhibit atypical functional activation within key neural substrates involved in ToM. More generally, our data could highlight an important role for TPJ dysfunction in driving compulsive behaviors. PMID:27424063

  11. Lipids in airway secretions

    International Nuclear Information System (INIS)

    Lipids form a significant portion of airway mucus yet they have not received the same attention that epithelial glycoproteins have. We have analysed, by thin layer chromatography, lipids present in airway mucus under 'normal' and hypersecretory (pathological) conditions.The 'normals' included (1) bronchial lavage obtained from healthy human volunteers and from dogs and (2) secretions produced ''in vitro'' by human (bronchial) and canine (tracheal) explants. Hypersecretory mucus samples included (1) lavage from dogs made bronchitic by exposure to SO2, (2) bronchial aspirates from acute and chronic tracheostomy patients, (3) sputum from patients with cystic fibrosis and chronic bronchitis and (4) postmortem secretions from patients who died from sudden infant death syndrome (SIDS) or from status asthmaticus. Cholesterol was found to be the predominant lipid in 'normal' mucus with lesser amounts of phospholipids. No glycolipids were detected. In the hypersecretory mucus, in addition to neutral and phospholipids, glycolipids were present in appreciable amounts, often the predominant species, suggesting that these may be useful as markers of disease. Radioactive precursors 14C acetate and 14C palmitate were incorporated into lipids secreted ''in vitro'' by canine tracheal explants indicating that they are synthesised by the airway. (author)

  12. Lipids in airway secretions

    Energy Technology Data Exchange (ETDEWEB)

    Bhaskar, K.R.; DeFeudis O' Sullivan, D.; Opaskar-Hincman, H.; Reid, L.M.

    1987-01-01

    Lipids form a significant portion of airway mucus yet they have not received the same attention that epithelial glycoproteins have. We have analysed, by thin layer chromatography, lipids present in airway mucus under 'normal' and hypersecretory (pathological) conditions.The 'normals' included (1) bronchial lavage obtained from healthy human volunteers and from dogs and (2) secretions produced ''in vitro'' by human (bronchial) and canine (tracheal) explants. Hypersecretory mucus samples included (1) lavage from dogs made bronchitic by exposure to SO/sub 2/, (2) bronchial aspirates from acute and chronic tracheostomy patients, (3) sputum from patients with cystic fibrosis and chronic bronchitis and (4) postmortem secretions from patients who died from sudden infant death syndrome (SIDS) or from status asthmaticus. Cholesterol was found to be the predominant lipid in 'normal' mucus with lesser amounts of phospholipids. No glycolipids were detected. In the hypersecretory mucus, in addition to neutral and phospholipids, glycolipids were present in appreciable amounts, often the predominant species, suggesting that these may be useful as markers of disease. Radioactive precursors /sup 14/C acetate and /sup 14/C palmitate were incorporated into lipids secreted ''in vitro'' by canine tracheal explants indicating that they are synthesised by the airway.

  13. Relationship between aldosterone and the metabolic syndrome in patients with obstructive sleep apnea hypopnea syndrome: effect of continuous positive airway pressure treatment.

    Directory of Open Access Journals (Sweden)

    Antonia Barceló

    Full Text Available BACKGROUND: Metabolic syndrome (MS occurs frequently in patients with obstructive sleep apnea-hypopnea syndrome (OSAHS. We hypothesized that aldosterone levels are elevated in OSAHS and associated with the presence of MS. METHODS: We studied 66 patients with OSAHS (33 with MS and 33 without MS and 35 controls. The occurrence of the MS was analyzed according to the National Cholesterol Education Program Adult Treatment Panel III (NCEP ATP III clinical criteria. Measurements of plasma renin activity (PRA, aldosterone, aldosterone:PRA ratio, creatinine, glucose, triglycerides, cholesterol and HDL cholesterol were obtained at baseline and after CPAP treatment. RESULTS: Aldosterone levels were associated with the severity of OSAHS and higher than controls (p = 0.046. Significant differences in aldosterone levels were detected between OSAHS patients with and without MS (p = 0.041. A significant reduction was observed in the aldosterone levels in patients under CPAP treatment (p = 0.012. CONCLUSION: This study shows that aldosterone levels are elevated in OSAHS in comparison to controls, and that CPAP therapy reduces aldosterone levels. It also shows that aldosterone levels are associated with the presence of metabolic syndrome, suggesting that aldosterone excess might predispose or aggravate the metabolic and cardiovascular complications of OSAHS. TRIAL REGISTRATION: The study is not a randomized controlled trial and was not registered.

  14. Loss and Gain of MeCP2 Cause Similar Hippocampal Circuit Dysfunction that Is Rescued by Deep Brain Stimulation in a Rett Syndrome Mouse Model.

    Science.gov (United States)

    Lu, Hui; Ash, Ryan T; He, Lingjie; Kee, Sara E; Wang, Wei; Yu, Dinghui; Hao, Shuang; Meng, Xiangling; Ure, Kerstin; Ito-Ishida, Aya; Tang, Bin; Sun, Yaling; Ji, Daoyun; Tang, Jianrong; Arenkiel, Benjamin R; Smirnakis, Stelios M; Zoghbi, Huda Y

    2016-08-17

    Loss- and gain-of-function mutations in methyl-CpG-binding protein 2 (MECP2) underlie two distinct neurological syndromes with strikingly similar features, but the synaptic and circuit-level changes mediating these shared features are undefined. Here we report three novel signs of neural circuit dysfunction in three mouse models of MECP2 disorders (constitutive Mecp2 null, mosaic Mecp2(+/-), and MECP2 duplication): abnormally elevated synchrony in the firing activity of hippocampal CA1 pyramidal neurons, an impaired homeostatic response to perturbations of excitatory-inhibitory balance, and decreased excitatory synaptic response in inhibitory neurons. Conditional mutagenesis studies revealed that MeCP2 dysfunction in excitatory neurons mediated elevated synchrony at baseline, while MeCP2 dysfunction in inhibitory neurons increased susceptibility to hypersynchronization in response to perturbations. Chronic forniceal deep brain stimulation (DBS), recently shown to rescue hippocampus-dependent learning and memory in Mecp2(+/-) (Rett) mice, also rescued all three features of hippocampal circuit dysfunction in these mice. PMID:27499081

  15. The Effects of Massage with Coconut and Sunflower Oils on Oxygen Saturation of Premature Infants with Respiratory Distress Syndrome Treated With Nasal Continuous Positive Airway Pressure

    Directory of Open Access Journals (Sweden)

    Sousan Valizadeh

    2012-11-01

    Full Text Available Introduction: Nowadays particular emphasis is placed on the developmental aspects of premature infants care. Massage therapy is one of the best-known methods of caring. Due to the minimal touch policy in neonatal intensive care units (NICUs, massaging is not usually performed on premature infants. However, there is not sufficient evidence to support the claim that newborn infants with complex medical conditions should not be massaged. This study aimed to determine the effects of massage with coconut and sunflower oils on oxygen saturation of infants with respiratory distress syndrome (RDS treated with nasal continuous positive airway pressure (NCPAP. Methods: This was a randomized controlled trial on 90 newborns who were admitted to Alzahra Hospital (Tabriz, Iran. The infants were divided into control and massage therapy groups (massage with coconut and sunflower oils. Data was collected using a hospital documentation form. A 15-minute daily massage was performed for 3 days. Respiratory rate (RR, fraction of inspired oxygen (FiO2 and oxygen saturation were measured 5 minutes before the massage, 3 times during the massage, and 5 minutes after the massage. The collected data was analyzed using a mixed model. Results: In comparison to coconut oil and control groups, mean oxygen saturation of sunflower oil group was improved. In addition, the coconut massage group showed lower oxygen saturation than the control group but was all values were within the normal range. Although massage decreased oxygen saturation, there was no need to increase FiO2. Conclusion: Massage therapy can provide developmental care for infants treated with NCPAP.

  16. Effects of heated humidification and topical steroids on compliance, nasal symptoms, and quality of life in patients with obstructive sleep apnea syndrome using nasal continuous positive airway pressure.

    LENUS (Irish Health Repository)

    Ryan, Silke

    2012-02-01

    BACKGROUND: Nasal side effects are common in patients with obstructive sleep apnea syndrome (OSAS) starting on nasal continuous positive airway pressure (CPAP) therapy. We tested the hypothesis that heated humidification or nasal topical steroids improve compliance, nasal side effects and quality of life in this patient group. METHODS: 125 patients with the established diagnosis of OSAS (apnea\\/hypopnea index > or = 10\\/h), who tolerated CPAP via a nasal mask, and who had a successful CPAP titration were randomized to 4 weeks of dry CPAP, humidified CPAP or CPAP with additional topical nasal steroid application (fluticasone, GlaxoWellcome). Groups were similar in all demographic variables and in frequency of nasal symptoms at baseline. Outcome measures were objective compliance, quality of life (short form 36), subjective sleepiness (Epworth Sleepiness Scale score) and nasal symptoms such as runny, dry or blocked nose, sneezing and headaches; all variables assessed using a validated questionnaire and by direct interview. RESULTS: There was no difference in compliance between groups after 4 weeks (dry: 5.21 +\\/- 1.66 h\\/night, fluticasone: 5.66 +\\/- 1.68, humidifier: 5.21 +\\/- 1.84; p = 0.444). Quality of life and subjective sleepiness improved in all groups, but there were no differences in the extent of improvement. Nasal Symptoms were less frequently reported in the humidifier group (28%) than in the remaining groups (dry: 70%, fluticasone: 53%, p = 0.002). However, the addition of fluticasone resulted in increased frequency of sneezing. CONCLUSION: The addition of a humidifier, but not nasal steroids decreases the frequency of nasal symptoms in unselected OSAS patients initiating CPAP therapy; however compliance and quality of life remain unaltered.

  17. THREE-DIMENSIONAL ASSESSMENT OF THE PHARYNGEAL AIRWAY AND MAXILLARY SINUS VOLUMES IN INDIVIDUALS WITH NON-SYNDROMIC CLEFT LIP AND PALATE

    Directory of Open Access Journals (Sweden)

    Ana NEMȚOI

    2015-09-01

    Full Text Available Introduction: Children with cleft lip and palate (CLP are known to have airway problems. Introduction of ConeBeam CT (CBCT and imaging software has facilitated generation of 3D images for assessing the volume of maxillary sinuses and pharyngeal airway. Consequently, the present study aimed at evaluating and comparing the maxillary sinus and pharyngeal airway volume of patients with cleft lip and palate in healthy patients, using cone beam computed tomography (CBCT images. Materials and method: The sample group included 27 individuals (15 with cleft lip and palate subjects and 12 healthy subjects. The pharyngeal airway and each maxillary sinus were three-dimensionally assessed, segmented and their volume was calculated. A comparison between the right and left sinus was performed by Student t-test, and the differences between the control and cleft groups were calculated using ANOVA. Results: No statistically significant differences were found when the maxillary sinuses volumes from each side were compared (p >0.05. The unilateral CLP patients presented the lowest sinus volume. Individuals with CLP did not exhibit a total airway volume smaller than the nonCLP controls. Conclusions: 3D imaging using CBCT and Romexis software is reliable for assessing maxillary sinus and pharyngeal airway volume. The present study showed that the pharyngeal airway is not compromised in CLP individuals. The unilateral CLP individuals present maxillary sinuses with smaller volumes, no differences being recorded between the cleft and non-cleft side.

  18. Zebrafish Models for the Mechanosensory Hair Cell Dysfunction in Usher Syndrome 3 Reveal That Clarin-1 Is an Essential Hair Bundle Protein

    OpenAIRE

    Gopal, Suhasini R.; Chen, Daniel H.-C.; Chou, Shih-Wei; Zang, Jingjing; Neuhauss, Stephan C. F.; Stepanyan, Ruben; McDermott, Brian M., Jr.; Alagramam, Kumar N.

    2015-01-01

    Usher syndrome type III (USH3) is characterized by progressive loss of hearing and vision, and varying degrees of vestibular dysfunction. It is caused by mutations that affect the human clarin-1 protein (hCLRN1), a member of the tetraspanin protein family. The missense mutation CLRN1N48K, which affects a conserved N-glycosylation site in hCLRN1, is a common causative USH3 mutation among Ashkenazi Jews. The affected individuals hear at birth but lose that function over time. Here, we developed...

  19. Ethinylestradiol30μg-drospirenone and metformin: could this combination improve endothelial dysfunction in polycystic ovary syndrome?

    Directory of Open Access Journals (Sweden)

    Ilie Ioana

    2012-06-01

    Full Text Available Abstract Background We are hereby investigating for the first time the effect of the association ethinylestradiol30μg-drospirenone 3mg (DRP/EE30μg plus metformin and weight loss on endothelial status and C-reactive protein (hsCRP levels in polycystic ovary syndrome (PCOS. Methods 25 young women with PCOS (mean age 22.76 ± 0.83 years, body mass index (BMI: 28.44 ± 6.23 who completed the study were prospectively evaluated. The oral contraceptive- DRP/EE30μg (21 days/month and metformin (1700 mg daily were administered for 6 months to the PCOS group. Additionally, the 15 overweight and obese patients (BMI > 25 kg/m2 were instructed in a diet of no more than 1500 cal daily. Primary outcome measures were surrogate markers of cardiovascular disease and included endothelial function, i.e. flow-mediated dilatation (FMD on the brachial artery and endothelin-1 levels, as well as hsCRP concentrations, body composition (measured by whole-body dual-energy X-ray-absorptiometry and insulin resistance. Variables were assessed at baseline, as well as after our medical intervention. Results The combination between DRP/EE30μg plus metformin combined with weight loss triggered a significant improvement in the FMD values (FMD-PCOSbasal 3.48 ± 1.00 vs FMD-PCOS6 months7.43 ± 1.04, p = 0.033, as well as body composition and insulin insensitivity (p 6months – PCOSbasal difference. Conclusion A 6-month course of metformin- DRP/EE30μg (associated with weight loss improves the endothelial dysfunction in PCOS and shows neutral effects on hsCRP concentrations as an inflammation marker. These data demand for reevaluation of the medical therapy in PCOS, particularly in women with additional metabolic and cardiovascular risk factors (ClinicalTrials.gov Identifier: NCT01459445.

  20. Prevalence of pituitary hormone dysfunction, metabolic syndrome, and impaired quality of life in retired professional football players: a prospective study.

    Science.gov (United States)

    Kelly, Daniel F; Chaloner, Charlene; Evans, Diana; Mathews, Amy; Cohan, Pejman; Wang, Christina; Swerdloff, Ronald; Sim, Myung-Shin; Lee, Jihey; Wright, Mathew J; Kernan, Claudia; Barkhoudarian, Garni; Yuen, Kevin C J; Guskiewicz, Kevin

    2014-07-01

    Hypopituitarism is common after moderate and severe traumatic brain injury (TBI). Herein, we address the association between mild TBI (mTBI) and pituitary and metabolic function in retired football players. Retirees 30-65 years of age, with one or more years of National Football League (NFL) play and poor quality of life (QoL) based on Short Form 36 (SF-36) Mental Component Score (MCS) were prospectively enrolled. Pituitary hormonal and metabolic syndrome (MetS) testing was performed. Using a glucagon stimulation test, growth hormone deficiency (GHD) was defined with a standard cut point of 3 ng/mL and with a more stringent body mass index (BMI)-adjusted cut point. Subjects with and without hormonal deficiency (HD) were compared in terms of QoL, International Index of Erectile Function (IIEF) scores, metabolic parameters, and football career data. Of 74 subjects, 6 were excluded because of significant non-football-related TBIs. Of the remaining 68 subjects (mean age, 47.3±10.2 years; median NFL years, 5; median NFL concussions, 3; mean BMI, 33.8±6.0), 28 (41.2%) were GHD using a peak GH cutoff of <3 ng/mL. However, with a BMI-adjusted definition of GHD, 13 of 68 (19.1%) were GHD. Using this BMI-adjusted definition, overall HD was found in 16 (23.5%) subjects: 10 (14.7%) with isolated GHD; 3 (4.4%) with isolated hypogonadism; and 3 (4.4%) with both GHD and hypogonadism. Subjects with HD had lower mean scores on the IIEF survey (p=0.016) and trended toward lower scores on the SF-36 MCS (p=0.113). MetS was present in 50% of subjects, including 5 of 6 (83%) with hypogonadism, and 29 of 62 (46.8%) without hypogonadism (p=0.087). Age, BMI, median years in NFL, games played, number of concussions, and acknowledged use of performance-enhancing steroids were similar between HD and non-HD groups. In summary, in this cohort of retired NFL players with poor QoL, 23.5% had HD, including 19% with GHD (using a BMI-adjusted definition), 9% with hypogonadism, and 50% had Met

  1. Clinical effects of continuous high volume hemofiltration on severe acute pancreatitis complicated with multiple organ dysfunction syndrome

    Institute of Scientific and Technical Information of China (English)

    Hao Wang; Wei-Qin Li; Wei Zhou; Ning Li; Jie-Shou Li

    2003-01-01

    AIM: To investigate the efficiency of continuous high volume hemofiltration (HVHF) in the treatment of severe acute pancreatitis (SAP) complicated with multiple organ dysfunction syndrome (MODS).METHODS: A total of 28 SAP patients with an average of 14.36±3.96 APACHE Ⅱ score were involved. Diagnostic criteria for SAP standardized by the Chinese Medical Association and diagnostic criteria for MODS standardized by American College of Chest Physicians (ACCP) and Society of Critical Care Medicine (SCCM) were applied for inclusion. HVHF was started 6.0±6.1 (1-30) days after onset of the disease and sustained for at least 72 hours, AN69 hemofilter (1.2 m2)was changed every 24 hours. The ultrafiltration rate during HVHF was 4 000 mi/h, blood flow rate was 250-300 mi/min,and the substitute fluid was infused with pre-dilution. Low molecular weight heparin was used for anticoagulation.RESULTS: HVHF was well tolerated in all the patients, and lasted for 4.04±3.99 (3-24) days. 20 of the patients survived,6 patients died and 2 of the patients quited for financial reason.The ICU mortality was 21.4%. Body temperature, heart rate and breath rate decreased significantly after HVHF.APACHE Ⅱ score was 14.4±3.9 before HVHF, and 9.9±4.3after HVHF, which decreased significantly (P<0.01). Partial pressure of oxygen in arterial blood before HVHF was 68.5±19.5 mmHg, and increased significantly after HVHF,which was 91.9±25 mmHg (P<0.01). During HVHF the hemodynamics was stable, and serum potassium, sodium,chlorine, glucose and pH were at normal level.CONCLUSION: HVHF is technically possible in SAP patients complicated with MODS. It does not appear to have detrimental effects and may have beneficial effects.Continuous HVHF, which seldom disturbs the hemodynamics and causes few side-effects, is expected to become a beneficial adjunct therapy for SAP complicated with MODS.

  2. Dysfunctional voiding.

    Science.gov (United States)

    Chiozza, M L

    2002-01-01

    Wetting may be considered the Cinderella of paediatric medicine. Before discussing dysfunctional voiding, the milestones of the normal development of continence in the child and the definitions used to describe this topic are presented. Bladder storage requires (1): accommodation of increasing volumes of urine at low intravesical pressure and with appropriate sensation; (2): a bladder outlet that is closed and not modified during increase in intra-abdominal pressure; (3): absence of involuntary bladder contractions. Development of continence in the child involves three independent factors maturing concomitantly: (1) development of normal bladder capacity; (2) maturation of urethral sphincter function; (3) development of neural control over bladder-sphincter function. All these processes are discussed. Abnormalities of any of these maturational sequences, which run parallel and overlapping, may result in clinically evident abnormalities of bladder sphincter control. Although dysfunctional voiding (DV) in children is very common its prevalence has not been well studied and, to date, and its origin is not well known. In a correct evaluation of functional voiding we must take into account different elements: the bladder capacity (that increases during the first 8 years of life roughly 30 ml per year), the micturition frequency, post-void residual volumes, bladder dynamics, urinary flow rates. Thus the correct assessment of children with lower urinary tract dysfunction should include a detailed history. Signs of DV range from urge syndrome to complex incontinence patterns during the day and the night. In addition to incontinence problems, children may have frequency, urgency, straining to void, weak or interrupted urinary stream, urinary tract infections (UTIs) and chronic constipation with or without encopresis. DV are also referred in enuretic children who wet the bed more than one time per night and have a functional bladder capacity lower than attended for age

  3. Effect of Continuous Positive Airway Pressure Therapy on Glycemic Excursions and Insulin Sensitivity in Patients with Obstructive Sleep Apnea-hypopnea Syndrome and Type 2 Diabetes

    Institute of Scientific and Technical Information of China (English)

    Li-Xin Guo; Xin Zhao; Qi Pan; Xue Sun; Hui Li; Xiao-Xia Wang; Li-Na Zhang

    2015-01-01

    Background:For patients with obstructive sleep apnea-hypopnea syndrome (OSAHS) and type 2 diabetes mellitus (T2DM),the night sleep interruption and intermittent hypoxia due to apnea or hypopnea may induce glycemic excursions and reduce insulin sensitivity.This study aimed to investigate the effect of continuous positive airway pressure (CPAP) therapy in patients with OSAHS and T2DM.Methods:Continuous glucose monitoring system (CGMS) was used in 40 patients with T2DM and newly diagnosed OSAHS.The measurements were repeated after 30 days of CPAP treatment.Subsequently,insulin sensitivity and glycohemoglobin (HbA1c) were measured and compared to the pretreatment data.Results:After CPAP therapy,the CGMS indicators showed that the 24-h mean blood glucose (MBG) and the night time MBG were significantly reduced (P < 0.05 and P =0.03,respectively).The mean ambulatory glucose excursions (MAGEs) and the mean of daily differences were also significantly reduced (P < 0.05 and P =0.002,respectively) compared to pretreatment levels.During the night,MAGE also significantly decreased (P =0.049).The differences between the highest and lowest levels of blood glucose over 24 h and during the night were significantly lower than prior to CPAP treatment (P < 0.05 and P =0.024,respectively).The 24 h and night time durations of high blood glucose (>7.8 mmol/L and > 11.1 mmol/L) decreased (P < 0.05 and P < 0.05,respectively) after the treatment.Inaddition,HbA1c levels were also lower than those before treatment (P < 0.05),and the homeostasis model assessment index of insulin resistance was also significantly lower than before CPAP treatment (P =0.034).Conclusions:CPAP therapy may have a beneficial effect on improving not only blood glucose but also upon insulin sensitivity in T2DM patients with OSAHS.This suggests that CPAP may be an effective treatment for T2DM in addition to intensive diabetes management.

  4. Initial treatment of respiratory distress syndrome with nasal intermittent mandatory ventilation versus nasal continuous positive airway pressure: A randomized controlled trial

    Directory of Open Access Journals (Sweden)

    Amir-Mohammad Armanian

    2014-01-01

    Full Text Available Background: Neonatal respiratory distress syndrome (RDS in premature infants who survived and its complications are a common problem. Due to high morbidity and mechanical ventilation (MV nowadays researchers in interested minimizing MV. To determine, in very low birth weight (BW preterm neonates with RDS, if initial treatment with nasal intermittent mandatory ventilation (early NIMV compared with early nasal continuous positive airway pressure (early NCPAP obtains more favorable outcomes in terms of the duration of treatment, and the need for endotracheal tube ventilation. Methods: In this single-center randomized control trial study, infants (BW ≤ 1500 g and/or gestational age ≤ 34 weeks with respiratory distress were considered eligible. Forty-four infants were randomly assigned to receive early-NIMV and 54 comparable infants to early-NCPAP. Surfactants were given, when FIO 2 requirement was of >30%. Primary outcomes were failure of noninvasive respiratory support, that is, the need for MV in the first 48 h of life and for the duration of noninvasive respiratory support in each group. Results: 98 infants were enrolled (44 in the NIMV and 54 in the NCPAP group. The Preventive power of MV of NIMV usage (95.5% was not lower than the NCPAP (98.1% strength (hazard ratio: 0.21 (95% confidence interval: 0.02-2.66; P: 0.23. The duration of noninvasive respiratory support in the NIMV group was significantly shorter than NCPAP (the median (range was 24 (18.00-48.00 h versus 48.00 (22.00-120.00 h in NIMV versus NCPAP groups; P < 0.001. Similarly, the duration of dependency on oxygen was less, for NIMV (the median (range was 96.00 (41.00-504.00 h versus144.00 (70.00-1130.00 h in NIMV versus NCPAP groups; P: 0.009. Interestingly, time to full enteral feeds and length of hospital stay were more favorable in the NIMV versus the NCPAP group. Conclusions: Initial treatment of RDS with NIMV was safe, and well tolerated. Furthermore, NIMV had excellent

  5. Airway management in trauma

    Directory of Open Access Journals (Sweden)

    Rashid M Khan

    2011-01-01

    Full Text Available Trauma has assumed epidemic proportion. 10% of global road accident deaths occur in India. Hypoxia and airway mismanagement are known to contribute up to 34% of pre-hospital deaths in these patients. A high degree of suspicion for actual or impending airway obstruction should be assumed in all trauma patients. Objective signs of airway compromise include agitation, obtundation, cyanosis, abnormal breath sound and deviated trachea. If time permits, one should carry out a brief airway assessment prior to undertaking definitive airway management in these patients. Simple techniques for establishing and maintaining airway patency include jaw thrust maneuver and/or use of oro- and nas-opharyngeal airways. All attempts must be made to perform definitive airway management whenever airway is compromised that is not amenable to simple strategies. The selection of airway device and route- oral or -nasal, for tracheal intubation should be based on nature of patient injury, experience and skill level.

  6. Rare Upper Airway Anomalies.

    Science.gov (United States)

    Windsor, Alanna; Clemmens, Clarice; Jacobs, Ian N

    2016-01-01

    A broad spectrum of congenital upper airway anomalies can occur as a result of errors during embryologic development. In this review, we will describe the clinical presentation, diagnosis, and management strategies for a few select, rare congenital malformations of this system. The diagnostic tools used in workup of these disorders range from prenatal tests to radiological imaging, swallowing evaluations, indirect or direct laryngoscopy, and rigid bronchoscopy. While these congenital defects can occur in isolation, they are often associated with disorders of other organ systems or may present as part of a syndrome. Therefore workup and treatment planning for patients with these disorders often involves a team of multiple specialists, including paediatricians, otolaryngologists, pulmonologists, speech pathologists, gastroenterologists, and geneticists. PMID:26277452

  7. Immune dysfunction in cirrhosis

    OpenAIRE

    Sipeki Nóra; Antal-Szalmás Péter (1968-) (laboratóriumi szakorvos, laboratóriumi hematológus és immunológus, klinikai farmakológus szakorvos); Lakatos Péter László; Papp Mária (1975-) (belgyógyász, gasztroenterológus)

    2014-01-01

    Innate and adaptive immune dysfunction, also referred to as cirrhosis-associated immune dysfunction syndrome, is a major component of cirrhosis, and plays a pivotal role in the pathogenesis of both the acute and chronic worsening of liver function. During the evolution of the disease, acute decompensation events associated with organ failure(s), so-called acute-on chronic liver failure, and chronic decompensation with progression of liver fibrosis and also development of disease specific comp...

  8. Management of systemic inflammatory response syndrome and prevention and treatment of multiple organ dysfunction syndromes%全身炎症反应综合征调控与多器官功能不全综合征防治

    Institute of Scientific and Technical Information of China (English)

    陈敏英

    2010-01-01

    @@ 1991年美国胸科医师和危重病医学联席会议明确全身炎症反应综合征(systemic inflammatory response syndrome,SIRS) 和多器官功能不全综合征(multiple organ dysfunction syndrome,MODS)的概念[1],炎症反应学说成为MODS的基石.机体炎症反应的转归取决于促炎一抗炎反应平衡,任何一方的过度优势均可以损害机体,成为MODS的基础.早期干预SIRS,积极防治MODS,是降低危重病患者病死率的重要手段.

  9. Inflammation markers as mediators of vasculo-endothelial dysfunction and atherosclerosis in the metabolic syndrome and type 2 diabetes

    Institute of Scientific and Technical Information of China (English)

    Ali A. Rizvi

    2007-01-01

    @@ The prevalence of both type 2 diabetes and metabolic syndrome is increasing exponentially all over the world because of global changes in obesity, sedentary lifestyle, and the aging of the population.1

  10. Developmental androgen excess programs sympathetic tone and adipose tissue dysfunction and predisposes to a cardiometabolic syndrome in female mice

    OpenAIRE

    Nohara, Kazunari; Waraich, Rizwana S.; Liu, Suhuan; Ferron, Mathieu; Waget, Aurélie; Meyers, Matthew S.; Karsenty, Gérard; Burcelin, Rémy,; Mauvais-Jarvis, Franck

    2013-01-01

    Among women, the polycystic ovarian syndrome (PCOS) is considered a form of metabolic syndrome with reproductive abnormalities. Women with PCOS show increased sympathetic tone, visceral adiposity with enlarged adipocytes, hypoadiponectinemia, insulin resistance, glucose intolerance, increased inactive osteocalcin, and hypertension. Excess fetal exposure to androgens has been hypothesized to play a role in the pathogenesis of PCOS. Previously, we showed that neonatal exposure to the androgen t...

  11. Duration of Type 2 Diabetes and Very Low Density Lipoprotein Levels Are Associated with Cognitive Dysfunction in Metabolic Syndrome

    OpenAIRE

    Divya Yogi-Morren; Rachel Galioto; Sarah Elizabeth Strandjord; KENNEDY, L; Pooja Manroa; Kirwan, John P.; Sangeeta Kashyap; John Gunstad

    2014-01-01

    Type 2 diabetes (T2D) is now recognized as an independent risk factor for accelerated cognitive decline and neurological conditions like Alzheimer's disease. Less is known about the neurocognitive function of T2D patients with comorbid metabolic syndrome, despite their elevated risk for impairment. Computerized testing in 47 adults with T2D that met criteria for NCEP metabolic syndrome revealed that cognitive impairment was prevalent, including 13% in tests of memory, 50% in attention, and 35...

  12. Triggers of airway inflammation.

    Science.gov (United States)

    Kerrebijn, K F

    1986-01-01

    Most asthmatics have hyperresponsive airways. This makes them more sensitive than non-asthmatics to bronchoconstricting environmental exposures which, in their turn, may enhance responsiveness. Airway inflammation is considered to be a key determinant of airway hyperresponsiveness: the fact that chronic airway inflammation in cystic fibrosis does not lead to airway hyperresponsiveness of any importance indicates, however, that the role of airway inflammation is complex and incompletely elucidated. The main inducers of airway inflammation are viral infections, antigens, occupational stimuli and pollutants. Although exercise, airway cooling and hyper- or hypotonic aerosols are potent stimuli of bronchoconstriction, it is questionable if airway inflammation is involved in their mode of action. Each of the above-mentioned stimuli is discussed, with emphasis laid on the relation of symptoms to mechanisms. PMID:3533597

  13. Phenotyping airways disease: an A to E approach.

    Science.gov (United States)

    Gonem, S; Raj, V; Wardlaw, A J; Pavord, I D; Green, R; Siddiqui, S

    2012-12-01

    The airway diseases asthma and chronic obstructive pulmonary disease (COPD) are heterogeneous conditions with overlapping pathophysiological and clinical features. It has previously been proposed that this heterogeneity may be characterized in terms of five relatively independent domains labelled from A to E, namely airway hyperresponsiveness (AHR), bronchitis, cough reflex hypersensitivity, damage to the airways and surrounding lung parenchyma, and extrapulmonary factors. Airway hyperresponsiveness occurs in both asthma and COPD, accounting for variable day to day symptoms, although the mechanisms most likely differ between the two conditions. Bronchitis, or airway inflammation, may be predominantly eosinophilic or neutrophilic, with different treatments required for each. Cough reflex hypersensitivity is thought to underlie the chronic dry cough out of proportion to other symptoms that can occur in association with airways disease. Structural changes associated with airway disease (damage) include bronchial wall thickening, airway smooth muscle hypertrophy, bronchiectasis and emphysema. Finally, a variety of extrapulmonary factors may impact upon airway disease, including rhinosinusitis, gastroesophageal reflux disease, obesity and dysfunctional breathing. This article discusses the A to E concept in detail and describes how this framework may be used to assess and treat patients with airway diseases in the clinic. PMID:23181785

  14. Changes and implications of cough sensitivity in upper airway cough syndrome%上气道咳嗽综合征的咳嗽敏感性改变及其意义

    Institute of Scientific and Technical Information of China (English)

    王岚; 余莉; 邱志宏; 吕寒静; 邱忠民

    2012-01-01

    目的 探讨上气道咳嗽综合征咳嗽敏感性的改变和临床意义.方法 用辣椒素咳嗽敏感性试验检测94例上气道咳嗽综合征的咳嗽阈值C5,分析治疗后咳嗽阈值C5的改变.结果 94例上气道咳嗽综合征的辣椒素咳嗽阚值C5为3.9 (0.98,7.8)μmol/L,位于正常值范围,仅36例(36/94,38.30%)患者存在咳嗽高敏感性.经治疗咳嗽症状消失后30例患者接受了随访复查,治疗前辣椒素咳嗽阈值C5为5.85 (1.95,31.2) μmol/L,与治疗后的7.8 (0.98,7.8) μmol/L相比,差别无统计学意义(Z=-0.72,P=0.47).其中12例治疗前咳嗽敏感性增高者中有6例降至正常.结论 上气道咳嗽综合征的咳嗽敏感性存在不均一性,与正常值存在较大的重叠和交叉,大部分不符合咳嗽高敏感性综合征的概念.%Objective To investigate the changes and clinical implications of cough sensitivity in upper airway cough syndrome ( UACS ). Methods Cough threshold C5 was measured by capsaicin cough sensitivity test in 94 patients with upper airway cough syndrome; it was also measured in 30 patients after cough completely resolved with specific therapy. Results The mean cough threshold C5 was 5. 85 (1. 95, 31. 2) μmol/L and within the normal range; however, 36 patients (38.30% ) presented cough hypersensitivity. Thirty patients received re-examination of cough sensitivity to capsaicin after cough disappeared, there was no significant difference in cough threshold C5 between pre-and post-treatment [ 5.85 (1.95,31.2) μmol/L vs7.8 (0.98, 7.8)μmol/L, Z= -0.72,P = 0.47 ]. The effective treatment resulted in the recover of cough threshold C5 to normal level in 6 of 12 patients with increased cough sensitivity to capsaicin. Conclusion Cough sensitivity in upper airway cough syndrome is heterogeneous and overlapped with normal value. Therefore, upper airway cough syndrome is not always consistent with the concept of cough hypersensitivity syndrome.

  15. Rimonabant-mediated changes in intestinal lipid metabolism and improved renal vascular dysfunction in the JCR:LA-cp rat model of prediabetic metabolic syndrome.

    Science.gov (United States)

    Russell, James C; Kelly, Sandra E; Diane, Abdoulaye; Wang, Ye; Mangat, Rabban; Novak, Susan; Vine, Donna F; Proctor, Spencer D

    2010-08-01

    Rimonabant (SR141716) is a specific antagonist of the cannabinoid-1 receptor. Activation of the receptor initiates multiple effects on central nervous system function, metabolism, and body weight. The hypothesis that rimonabant has protective effects against vascular disease associated with the metabolic syndrome was tested using JCR:LA-cp rats. JCR:LA-cp rats are obese if they are cp/cp, insulin resistant, and exhibit associated micro- and macrovascular disease with end-stage myocardial and renal disease. Treatment of obese rats with rimonabant (10 mg.kg(-1).day(-1), 12-24 wk of age) caused transient reduction in food intake for 2 wk, without reduction in body weight. However, by 4 wk, there was a modest, sustained reduction in weight gain. Glycemic control improved marginally compared with controls, but at the expense of increased insulin concentration. In contrast, rimonabant normalized fasting plasma triglyceride and reduced plasma plasminogen activator inhibitor-1 and acute phase protein haptoglobin in cp/cp rats. Furthermore, these changes were accompanied by reduced postprandial intestinal lymphatic secretion of apolipoprotein B48, cholesterol, and haptoglobin. While macrovascular dysfunction and ischemic myocardial lesion frequency were unaffected by rimonabant treatment, both microalbuminuria and glomerular sclerosis were substantially reduced. In summary, rimonabant has a modest effect on body weight in freely eating obese rats and markedly reduces plasma triglyceride levels and microvascular disease, in part due to changes in intestinal metabolism, including lymphatic secretion of apolipoprotein B48 and haptoglobin. We conclude that rimonabant improves renal disease and intestinal lipid oversecretion associated with an animal model of the metabolic syndrome that appears to be independent of hyperinsulinemia or macrovascular dysfunction.

  16. How does serum brain natriuretic peptide level change under nasal continuous positive airway pressure in obstructive sleep apnea-hypopnea syndrome?

    Science.gov (United States)

    Msaad, Sameh; Marrakchi, Rim; Grati, Malek; Gargouri, Rahma; Kammoun, Samy; Jammoussi, Kamel; Yangui, Ilhem

    2016-01-01

    Background Obstructive sleep apnea-hypopnea syndrome (OSAHS) is associated with cardiovascular morbidity and mortality, which can be improved by using continuous positive airway pressure (CPAP) therapy. However, the pathophysiological links between the two kinds of disease and the mechanism of the CPAP effect remain incompletely understood. We aimed to inquire into the myocardial involvement in this relationship. We suggested that serum brain natriuretic peptide (BNP) is sensitive enough to detect myocardial stress caused by OSAHS. Design and methods Sixty-four subjects without cardiovascular disease (21 controls, 24 normotensive OSAHS patients, and 19 hypertensive OSAHS patients) were analyzed for serum BNP at baseline and serially over 6 months. CPAP was applied to 23 patients with severe OSAHS. Results At baseline, the serum BNP levels were significantly higher (p=0.0001) in the OSAHS group (22.3±14.79 pg/ml) than in the control group (9.2±6.75 pg/ml). Increased serum BNP levels were significantly associated with mean transcutaneous oxygen saturation (SpO2) (psleep time spent with SpO2 lower than 90% (p=0.002). All patients with elevated BNP levels (≥37 pg/ml) had moderate or severe OSAHS (11/43 OSAHS patients). The more severe the OSAHS, the higher the BNP levels were. However, only the difference between severe and mild OSAHS was statistically significant (p=0.029). Hypertensive OSAHS patients had the highest baseline BNP levels (27.7±16.74 pg/ml). They were significantly higher (p=0.001) than in normotensive OSAHS patients (18±11.72 pg/ml) (p=0.039) and the controls (9.2±6.75 pg/ml). As compared with baseline, treatment with CPAP significantly decreased BNP levels in both hypertensive and normotensive OSAHS patients (respectively, from 36±16.10 to 29.7±14.29 pg/ml, p<0.001, and from 20±10.09 to 16±8.98 pg/ml, p<0.001). In contrast, the BNP levels slightly increased in the controls (from 9.2±6.75 to 9.5±7.02 pg/ml, p=0.029), but there was no

  17. Evaluation of Salivary Gland Dysfunction Using Salivary Gland Scintigraphy in Sjoegren's Syndrome Patients and in Thyroid Cancer Patients after Radioactive Iodine Therapy

    Energy Technology Data Exchange (ETDEWEB)

    Kang, Ji Yeon; Jang, Su Jin; Lee, Won Woo; Jang, Sung June; Lee, Yun Jong; Kim, Sang Eun [Seoul National Univ., Seoul (Korea, Republic of)

    2011-09-15

    Salivary gland scintigraphy (SGS) provides an objective means of diagnosing salivary gland dysfunction in Sjogren's syndrome (SS) patients and in thyroid cancer patients after radioactive iodine (RAI) therapy. In the present study, SGS was performed in SS patients and in thyroid cancer patients post RAI, and scintigraphic parameters were compared. Twenty eight SS patients (males:females=1:27, age 53.3{+-}11.9 years), 28 controls (males:females=3.25, age 54.1{+-}10.1 years), and 92 thyroid cancer patients (males:females=28:64, age 46.2{+-}12.9) who had undergone a session of high dose RAI therapy (mean dose, 5.2{+-}1.5 GBq) were included. SGS was performed using Tc 99m pertechnetate (925MBq). Scintigraphic parameters (parotid uptake ratio PU, submandibular uptake ratio SU, percent age parotid excretion %PE, and percentage submandibular excretion %SE) were measured and compared for SS, thyroid cancer post RAI, and control patients. PU, SU, %SE, and %PE were all significantly lower in SS than in post RAI thyroid cancer of control patients (p<0.05), whereas only %PE was significantly lower in post RAI thyroid cancer patients than in controls (P<0.05). SU and %SE were found to be correlated with the unstimulated whole salivary flow rate. Scintigraphic parameters derived from SGS can play a crucial role in the detection of salivary gland dysfunction in SS patients and in post RAI thyroid cancer patients.

  18. Potentially diagnostic electron paramagnetic resonance spectra elucidate the underlying mechanism of mitochondrial dysfunction in the deoxyguanosine kinase deficient rat model of a genetic mitochondrial DNA depletion syndrome.

    Science.gov (United States)

    Bennett, Brian; Helbling, Daniel; Meng, Hui; Jarzembowski, Jason; Geurts, Aron M; Friederich, Marisa W; Van Hove, Johan L K; Lawlor, Michael W; Dimmock, David P

    2016-03-01

    A novel rat model for a well-characterized human mitochondrial disease, mitochondrial DNA depletion syndrome with associated deoxyguanosine kinase (DGUOK) deficiency, is described. The rat model recapitulates the pathologic and biochemical signatures of the human disease. The application of electron paramagnetic (spin) resonance (EPR) spectroscopy to the identification and characterization of respiratory chain abnormalities in the mitochondria from freshly frozen tissue of the mitochondrial disease model rat is introduced. EPR is shown to be a sensitive technique for detecting mitochondrial functional abnormalities in situ and, here, is particularly useful in characterizing the redox state changes and oxidative stress that can result from depressed expression and/or diminished specific activity of the distinct respiratory chain complexes. As EPR requires no sample preparation or non-physiological reagents, it provides information on the status of the mitochondrion as it was in the functioning state. On its own, this information is of use in identifying respiratory chain dysfunction; in conjunction with other techniques, the information from EPR shows how the respiratory chain is affected at the molecular level by the dysfunction. It is proposed that EPR has a role in mechanistic pathophysiological studies of mitochondrial disease and could be used to study the impact of new treatment modalities or as an additional diagnostic tool. PMID:26773591

  19. Full Airway Drainage by Fiber Bronchoscopy Through Artificial Airway in the Treatment of Occult Traumatic Atelectasis.

    Science.gov (United States)

    Zhao, Xue Hong; Zhang, Yun; Liang, Zhong Yan; Zhang, Shao Yang; Yu, Wen Qiao; Huang, Fang-Fang

    2015-12-01

    The objective of this study is to investigate the effects of full airway drainage by fiber bronchoscopy through artificial airway in the treatment of traumatic atelectasis with occult manifestations. From May 2006 to May 2011, 40 cases of occult traumatic atelectasis were enrolled into our prospective study. Group A (n = 18) received drainage by nasal bronchoscope; group B underwent airway drainage by fiber bronchoscopy through artificial airway (n = 22). The effects of treatment were evaluated by the incidence of adult respiratory distress syndrome (ARDS), lung abscess, and the average length of hospital stay. Compared with nasal fiber-optic treatment, airway drainage by fiber bronchoscopy through artificial airway reduced the incidence of ARDS (p = 0.013) and lung abscess (p = 0.062) and shortened the mean length of stay (p = 0.018). Making the decision to create an artificial airway timely and carry out lung lavage by fiber bronchoscopy through artificial airway played a significant role in the treatment of occult traumatic atelectasis.

  20. Study of a human immunodeficiency virus-associated multiple-tissue dysfunction syndrome in one boy: Analysis of multiple tissues using X-ray, computed tomography and magnetic resonance imaging

    Directory of Open Access Journals (Sweden)

    Xichao Xia

    2016-03-01

    Full Text Available In the previous study, reports of human immunodeficiency virus (HIV-associated multiple tissue dysfunction syndromes are limit. Now, multiple tissue lesions of one boy with AIDS were analyzed using X-ray, computed tomography (CT and magnetic resonance imaging (MRI. Results showed that obvious brain atrophy and several focus were detected by CT and MRI. Lung lymphoid inflammation and pulmonary lymphoplasia were observed by CT. A swelling with a slight low density in hepatic anterior region of right lobe and a large soft tissue neoplasm in right abdomen were obtained by CT. An obvious damage of intestinal duct and intestinal lymphomas were respectively observed by molybdenum contrast image and CT. X-ray showed a low density of thoracic bone and iliac bone, and irregular shapes of bone joint and epiphysis. Characterizations of HIV-associated multiple organ dysfunction syndromes in the case are benefit to understand features of children with acquired immune deficiency syndrome.

  1. Ornithine and Homocitrulline Impair Mitochondrial Function, Decrease Antioxidant Defenses and Induce Cell Death in Menadione-Stressed Rat Cortical Astrocytes: Potential Mechanisms of Neurological Dysfunction in HHH Syndrome.

    Science.gov (United States)

    Zanatta, Ângela; Rodrigues, Marília Danyelle Nunes; Amaral, Alexandre Umpierrez; Souza, Débora Guerini; Quincozes-Santos, André; Wajner, Moacir

    2016-09-01

    Hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome is caused by deficiency of ornithine translocase leading to predominant tissue accumulation and high urinary excretion of ornithine (Orn), homocitrulline (Hcit) and ammonia. Although affected patients commonly present neurological dysfunction manifested by cognitive deficit, spastic paraplegia, pyramidal and extrapyramidal signs, stroke-like episodes, hypotonia and ataxia, its pathogenesis is still poorly known. Although astrocytes are necessary for neuronal protection. Therefore, in the present study we investigated the effects of Orn and Hcit on cell viability (propidium iodide incorporation), mitochondrial function (thiazolyl blue tetrazolium bromide-MTT-reduction and mitochondrial membrane potential-ΔΨm), antioxidant defenses (GSH) and pro-inflammatory response (NFkB, IL-1β, IL-6 and TNF-α) in unstimulated and menadione-stressed cortical astrocytes that were previously shown to be susceptible to damage by neurotoxins. We first observed that Orn decreased MTT reduction, whereas both amino acids decreased GSH levels, without altering cell viability and the pro-inflammatory factors in unstimulated astrocytes. Furthermore, Orn and Hcit decreased cell viability and ΔΨm in menadione-treated astrocytes. The present data indicate that the major compounds accumulating in HHH syndrome impair mitochondrial function and reduce cell viability and the antioxidant defenses in cultured astrocytes especially when stressed by menadione. It is presumed that these mechanisms may be involved in the neuropathology of this disease. PMID:27161368

  2. Ornithine and Homocitrulline Impair Mitochondrial Function, Decrease Antioxidant Defenses and Induce Cell Death in Menadione-Stressed Rat Cortical Astrocytes: Potential Mechanisms of Neurological Dysfunction in HHH Syndrome.

    Science.gov (United States)

    Zanatta, Ângela; Rodrigues, Marília Danyelle Nunes; Amaral, Alexandre Umpierrez; Souza, Débora Guerini; Quincozes-Santos, André; Wajner, Moacir

    2016-09-01

    Hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome is caused by deficiency of ornithine translocase leading to predominant tissue accumulation and high urinary excretion of ornithine (Orn), homocitrulline (Hcit) and ammonia. Although affected patients commonly present neurological dysfunction manifested by cognitive deficit, spastic paraplegia, pyramidal and extrapyramidal signs, stroke-like episodes, hypotonia and ataxia, its pathogenesis is still poorly known. Although astrocytes are necessary for neuronal protection. Therefore, in the present study we investigated the effects of Orn and Hcit on cell viability (propidium iodide incorporation), mitochondrial function (thiazolyl blue tetrazolium bromide-MTT-reduction and mitochondrial membrane potential-ΔΨm), antioxidant defenses (GSH) and pro-inflammatory response (NFkB, IL-1β, IL-6 and TNF-α) in unstimulated and menadione-stressed cortical astrocytes that were previously shown to be susceptible to damage by neurotoxins. We first observed that Orn decreased MTT reduction, whereas both amino acids decreased GSH levels, without altering cell viability and the pro-inflammatory factors in unstimulated astrocytes. Furthermore, Orn and Hcit decreased cell viability and ΔΨm in menadione-treated astrocytes. The present data indicate that the major compounds accumulating in HHH syndrome impair mitochondrial function and reduce cell viability and the antioxidant defenses in cultured astrocytes especially when stressed by menadione. It is presumed that these mechanisms may be involved in the neuropathology of this disease.

  3. Evaluation of short-term use of nocturnal nasal continuous positive airway pressure for a clinical profile and exercise capacity in adult patients with obstructive sleep apnea-hypopnea syndrome

    Directory of Open Access Journals (Sweden)

    Amrit K Goel

    2015-01-01

    Full Text Available Background and Aim: The obstructive sleep apnea-hypopnea syndrome (OSAHS is a common chronic respiratory disease, characterized by repetitive complete or partial collapse of the upper airway during sleep. The clinical spectrum extends between stoppage of breathing, snoring, daytime somnolence, and fatigue, to serious cardiovascular disease, stroke, metabolic syndrome, increased morbidity, and mortality. We aim to evaluate the short-term use of nasal continuous positive airway pressure (nCPAP therapy for the clinical profile and exercise capacity of patients with OSAHS. Patient Selection: Twenty patients diagnosed with moderate-to-severe OSAHS were enrolled in the study (study group - 15; clinically and PSG-matched control group - 5. Materials and Methods: Each patient was clinically evaluated for sleep-related symptoms, and also assessed with spirometry, the six-minute walk test (6MWT, and a symptom-limited incremental cardiopulmonary exercise test (CPET. The study group patients were administered nCPAP therapy for eight hours each night for four weeks, while the control group patients were just observed. They were re-assessed after four weeks and the data were statistically analyzed between the two groups. Results: The study group patients showed a significant (P- < 0.05 improvement in the OSAHS symptoms-the Epworth sleepiness score, six-minute walk distance; duration of exercise, power output, peak oxygen uptake, anaerobic threshold, diastolic blood pressure, dyspnea, and fatigue-in comparison with the control group patients. The improvement in exercise capacity following nCPAP therapy was attributed to the relief of disabling the OSAHS symptoms and improved cardiovascular, ventilator, and musculoskeletal functions. Conclusion: All OSAHS patients must be treated with nCPAP.

  4. Propranolol Treatment for Erectile Dysfunction Syndrome%勃起功能障碍证治心得

    Institute of Scientific and Technical Information of China (English)

    蒋平; 戴宁; 王祖龙

    2014-01-01

    Objective:To explore the basic pathogenesis of erectile dysfunction .Method:There were 120 cases with typical erectile dysfunction ,and use the prescription treatment based on pathogenesis of blood stasis and stagnation of liver and deficiency of kidney .Result:72 cases in clinic were 1~3 cured after months treatment , 18 cases markedly effective , 16 cases effective , 9 cases invalid , 5 cases with interruption of treatment , and the total effective rate was 88 .3%.Conclusion:With the development of the time , erectile dysfunction gradu-ally shifts to emotional disorders for the etiology with pathogenesis of stagnation of liver and deficiency of kid -ney and blood stasis .Self-made prescription has achieved a satisfactory clinical efficacy by tonifying kidney , activating blood and relieving the depressed liver .%目的:探讨当今勃起功能障碍的基本病机。方法:选取典型勃起功能障碍患者120例,运用根据肝郁肾虚血瘀病机制定的方药治疗。结果:1~3个月治疗后临床治愈72例,显效18例,有效16例,无效9例,5例中断治疗,总有效率为88.3%。结论:勃起功能障碍逐渐转变为以情志失调为病因,以肝郁肾虚血瘀为病机。通过自拟中药方滋阴补肾、活血补血、疏肝解郁,临床取得满意疗效。

  5. Early versus delayed initiation of nasal continuous positive airway pressure for treatment of respiratory distress syndrome in premature newborns: A randomized clinical trial

    OpenAIRE

    Zohreh Badiee; Fatemeh Naseri; Alireza Sadeghnia

    2013-01-01

    Background: This prospective study was performed to identify whether the early use of nasal continuous positive airway pressure (n CPAP) would reduce the rate of endotracheal intubation, mechanical ventilation and surfactant administration. Materials and Methods: This study was conducted from June 2009 to September 2010 in the Shahid Beheshti University Hospital, Isfahan-Iran. A total of 72 preterm infants with 25-30 weeks gestation who needed respiratory support at 5 min after birth enter...

  6. Effect of progressive muscle relaxation therapy on improving signs and symptoms of patients with myofacial pain dysfunction syndrome

    Directory of Open Access Journals (Sweden)

    Momen Beitollahi J.

    2009-03-01

    Full Text Available "nBackground and Aim: One of the most common causes of facial pain, is muscular pain. MPDS is one of the most important disorders of facial area that many of patients suffer from pain, tenderness of one or more masticatory muscles and limitation of movement. Psychological factors play an important role in MPDS. Anxiety and depression by increasing muscle tension, can cause myofacial pain syndrome; They can also accompany the syndrome as comorbidities or develop as sequelae of chronic pain and disability. Many approaches can be used to treat myofacial pain syndrome. The least invasive and least traumatic approach should be se.lected. Often, treatment for myofacial pain syndrome fails, because underlying problems go untreated. Progressive Muscle Relaxation (PMR is mainly used for treating anxiety and depression. The aim of this study was to evaluate the effect of PMR in improving signs and symptoms of patients with MPDS. "nMaterials and Methods: In this before and after clinical trial study, 33 patients with MPDS that had anxiety and or mild depression(were assessed by Beck Inventorywere treated by PMR in a period of a month (3 visitsand were followed up until 2 months. Analysis was done using paired t-test/ wilcoxon-sign-rank test. "nResults: The results of this study showed that variants like intensity of pain, tenderness of masticatory muscles, maximum opening of mouth with and without pain, anxiety (p<0.001 and depression (p=0.001 improved significant 16 after treatment in comparison with before treatment. "nConclusion: In conclusion, PMR is effective in improving signs and symptoms in patients with MPDS. Therefore psychological status should be considered in treatment plan of these patients.

  7. Duration of Type 2 Diabetes and Very Low Density Lipoprotein Levels Are Associated with Cognitive Dysfunction in Metabolic Syndrome

    Directory of Open Access Journals (Sweden)

    Divya Yogi-Morren

    2014-01-01

    Full Text Available Type 2 diabetes (T2D is now recognized as an independent risk factor for accelerated cognitive decline and neurological conditions like Alzheimer’s disease. Less is known about the neurocognitive function of T2D patients with comorbid metabolic syndrome, despite their elevated risk for impairment. Computerized testing in 47 adults with T2D that met criteria for NCEP metabolic syndrome revealed that cognitive impairment was prevalent, including 13% in tests of memory, 50% in attention, and 35% in executive function. Partial correlations showed that longer duration of diabetes was associated with poorer performance on tests of basic attention (r=-0.43, working memory (r=0.43, and executive function (r=0.42. Strong associations between very low density lipoprotein and poor cognitive function also emerged, including tests of set shifting (r=0.47 and cognitive inhibition (r=-0.51. Findings suggest that patients with T2D that meet criteria for metabolic syndrome are at high risk for cognitive impairment. Prospective studies should look to replicate these findings and examine the possible neuroprotective effects of lipid-lowering medication in this population.

  8. Impaired coronary metabolic dilation in the metabolic syndrome is linked to mitochondrial dysfunction and mitochondrial DNA damage.

    Science.gov (United States)

    Guarini, Giacinta; Kiyooka, Takahiko; Ohanyan, Vahagn; Pung, Yuh Fen; Marzilli, Mario; Chen, Yeong Renn; Chen, Chwen Lih; Kang, Patrick T; Hardwick, James P; Kolz, Christopher L; Yin, Liya; Wilson, Glenn L; Shokolenko, Inna; Dobson, James G; Fenton, Richard; Chilian, William M

    2016-05-01

    Mitochondrial dysfunction in obesity and diabetes can be caused by excessive production of free radicals, which can damage mitochondrial DNA. Because mitochondrial DNA plays a key role in the production of ATP necessary for cardiac work, we hypothesized that mitochondrial dysfunction, induced by mitochondrial DNA damage, uncouples coronary blood flow from cardiac work. Myocardial blood flow (contrast echocardiography) was measured in Zucker lean (ZLN) and obese fatty (ZOF) rats during increased cardiac metabolism (product of heart rate and arterial pressure, i.v. norepinephrine). In ZLN increased metabolism augmented coronary blood flow, but in ZOF metabolic hyperemia was attenuated. Mitochondrial respiration was impaired and ROS production was greater in ZOF than ZLN. These were associated with mitochondrial DNA (mtDNA) damage in ZOF. To determine if coronary metabolic dilation, the hyperemic response induced by heightened cardiac metabolism, is linked to mitochondrial function we introduced recombinant proteins (intravenously or intraperitoneally) in ZLN and ZOF to fragment or repair mtDNA, respectively. Repair of mtDNA damage restored mitochondrial function and metabolic dilation, and reduced ROS production in ZOF; whereas induction of mtDNA damage in ZLN reduced mitochondrial function, increased ROS production, and attenuated metabolic dilation. Adequate metabolic dilation was also associated with the extracellular release of ADP, ATP, and H2O2 by cardiac myocytes; whereas myocytes from rats with impaired dilation released only H2O2. In conclusion, our results suggest that mitochondrial function plays a seminal role in connecting myocardial blood flow to metabolism, and integrity of mtDNA is central to this process. PMID:27040114

  9. CLINICAL IMPORTANCE OF ENDOTHELIAL DYSFUNCTION AND INSULIN RESISTANCE SYNDROME IN PATIENTS WITH GOUT ASSOCIATED WITH ARTERIAL HYPERTENSION

    Directory of Open Access Journals (Sweden)

    N. N. Kushnarenko

    2015-09-01

    Full Text Available Aim. To study the endothelium status and determine the correlation between endothelial dysfunction and glucose metabolism in men with gout associated with arterial hypertension (HT.Material and methods. Patients (n=175, all are males with gout were enrolled into the study. Ambulatory blood pressure monitoring (ABPM was performed in all patients. Endothelial function was studied in tests with reactive hyperemia (endothelium-dependent reaction and nitroglycerin (endothelium independent reaction in brachial artery by ultrasonic Doppler examination. The level of nitrite-nitrate and endothelin-1 in blood serum was determined by ELISA technique. Fasting blood glucose and oral glucose tolerance tests were performed as well as fasting insulin blood level was determined by immunoenzyme method. Insulin-resistance index (HOMA-IR was calculated. Patients with HOMA- IR>2.77 were considered as insulin-resistant.Results. Patients with gout demonstrated endothelial deterioration associated with activation of nitroxid producing function, elevation in endothelin-1 serum level (1.36 fmol/ml [0.91; 2.32 fmol/ml] vs 0.19 fmol/ml [0.16; 0.27 fmol/ml] in controls, p<0.05 and impairments of endothelium-dependent vasodilation (6.4% [3.3; 7.3%] vs 17.8% [12.7; 23.9%] in controls, p<0.05. The revealed changes were the most marked in patients with gout associated with HT. The correlation between some endothelial dysfunction in- dices and glucose metabolism was observed.Conclusion. ABPM, brachial artery endothelium-dependent vasodilation and glucose metabolism status should be studied in patients with gout. Complex treatment of cardiovascular diseases in patients with gout should include ω-3 polyunsaturated fatty acids, angiotensin receptor antagonists should be used for antihypertensive therapy.

  10. CLINICAL IMPORTANCE OF ENDOTHELIAL DYSFUNCTION AND INSULIN RESISTANCE SYNDROME IN PATIENTS WITH GOUT ASSOCIATED WITH ARTERIAL HYPERTENSION

    Directory of Open Access Journals (Sweden)

    N. N. Kushnarenko

    2013-01-01

    Full Text Available Aim. To study the endothelium status and determine the correlation between endothelial dysfunction and glucose metabolism in men with gout associated with arterial hypertension (HT.Material and methods. Patients (n=175, all are males with gout were enrolled into the study. Ambulatory blood pressure monitoring (ABPM was performed in all patients. Endothelial function was studied in tests with reactive hyperemia (endothelium-dependent reaction and nitroglycerin (endothelium independent reaction in brachial artery by ultrasonic Doppler examination. The level of nitrite-nitrate and endothelin-1 in blood serum was determined by ELISA technique. Fasting blood glucose and oral glucose tolerance tests were performed as well as fasting insulin blood level was determined by immunoenzyme method. Insulin-resistance index (HOMA-IR was calculated. Patients with HOMA- IR>2.77 were considered as insulin-resistant.Results. Patients with gout demonstrated endothelial deterioration associated with activation of nitroxid producing function, elevation in endothelin-1 serum level (1.36 fmol/ml [0.91; 2.32 fmol/ml] vs 0.19 fmol/ml [0.16; 0.27 fmol/ml] in controls, p<0.05 and impairments of endothelium-dependent vasodilation (6.4% [3.3; 7.3%] vs 17.8% [12.7; 23.9%] in controls, p<0.05. The revealed changes were the most marked in patients with gout associated with HT. The correlation between some endothelial dysfunction in- dices and glucose metabolism was observed.Conclusion. ABPM, brachial artery endothelium-dependent vasodilation and glucose metabolism status should be studied in patients with gout. Complex treatment of cardiovascular diseases in patients with gout should include ω-3 polyunsaturated fatty acids, angiotensin receptor antagonists should be used for antihypertensive therapy.

  11. Some Issues in the Diagnosis of Upper Airway Cough Syndrome and Chronic Pharyngitis%上气道咳嗽综合征与慢性咽喉炎的诊断问题

    Institute of Scientific and Technical Information of China (English)

    赖克方

    2010-01-01

    @@ 2006年美国胸科医师学会(ACCP)咳嗽指南委员会修订的第二版美国咳嗽诊治指南建议用上气道咳嗽综合征(upper airway cough syndrome,UACS)替代鼻后滴流综合征(post nasal drip syndrome,PNDS)[1].PNDS最早由美国提出,指鼻炎或鼻窦炎引起分泌物倒流鼻后和咽喉等部位,导致以咳嗽为主要表现的综合征[2].欧美国家和国内研究表明,UACs或PNDS是慢性咳嗽的重要原因,国内的数据为14%~26%[3-6].总体而言,欧美国家PNDs的患病率更高,占慢性咳嗽病因的12%~41%[7-9].

  12. Viral bronchiolitis in young rats causes small airway lesions that correlate with reduced lung function.

    Science.gov (United States)

    Sorkness, Ronald L; Szakaly, Renee J; Rosenthal, Louis A; Sullivan, Ruth; Gern, James E; Lemanske, Robert F; Sun, Xin

    2013-11-01

    Viral illness with wheezing during infancy is associated with the inception of childhood asthma. Small airway dysfunction is a component of childhood asthma, but little is known about how viral illness at an early age may affect the structure and function of small airways. We used a well-characterized rat model of postbronchiolitis chronic airway dysfunction to address how postinfectious small airway lesions affect airway physiological function and if the structure/function correlates persist into maturity. Brown Norway rats were sham- or virus inoculated at 3 to 4 weeks of age and allowed to recover from the acute illness. At 3 to 14 months of age, physiology (respiratory system resistance, Newtonian resistance, tissue damping, and static lung volumes) was assessed in anesthetized, intubated rats. Serial lung sections revealed lesions in the terminal bronchioles that reduced luminal area and interrupted further branching, affecting 26% (range, 13-39%) of the small airways at 3 months of age and 22% (range, 6-40%) at 12 to 14 months of age. At 3 months of age (n = 29 virus; n = 7 sham), small airway lesions correlated with tissue damping (rs = 0.69) but not with Newtonian resistance (rs = 0.23), and Newtonian resistance was not elevated compared with control rats, indicating that distal airways were primarily responsible for the airflow obstruction. Older rats (n = 7 virus; n = 6 sham) had persistent small airway dysfunction and significantly increased Newtonian resistance in the postbronchiolitis group. We conclude that viral airway injury at an early age may induce small airway lesions that are associated quantitatively with small airway physiological dysfunction early on and that these defects persist into maturity.

  13. Linoleic acid metabolite drives severe asthma by causing airway epithelial injury

    OpenAIRE

    Mabalirajan, Ulaganathan; Rehman, Rakhshinda; Ahmad, Tanveer; Kumar, Sarvesh; Singh, Suchita; Leishangthem, Geeta D.; Aich, Jyotirmoi; Kumar, Manish; Khanna, Kritika; Singh, Vijay P.; Dinda, Amit K; Biswal, Shyam; Agrawal, Anurag; Ghosh, Balaram

    2013-01-01

    Airway epithelial injury is the hallmark of various respiratory diseases, but its mechanisms remain poorly understood. While 13-S-hydroxyoctadecadienoic acid (13-S-HODE) is produced in high concentration during mitochondrial degradation in reticulocytes little is known about its role in asthma pathogenesis. Here, we show that extracellular 13-S-HODE induces mitochondrial dysfunction and airway epithelial apoptosis. This is associated with features of severe airway obstruction, lung remodeling...

  14. Study on Effect of Kangyanling(抗炎灵) on Cytokine and C-Reactive Protein inPatients of Systemic Inflammatory Reaction Syndrome and Multi-Organ Dysfunction Syndrome after Abdominal Surgery

    Institute of Scientific and Technical Information of China (English)

    陈哲宇; 齐清会

    2001-01-01

    Objective: To observe the clinical efficacy and mechanism of Kangyanling (KYL) in treating patients with systemic inflammatory reaction syndrome and multi-organ dysfunction syndrome (SIRS/MODS) after abdominal surgery. Methods: Eighty-two patients of SIRS/MODS after abdominal surgery were divided into two groups according to admission time, the KYL treated group (n=35) and the control group (n=47). The levels of serum C-reactive protein (CRP), plasma tumor necrosis factor α (TNFα) and interleukin-6 (IL-6) were measured at the 1st, 3rd and 7th days post-operationally. Results: The levels of CRP, TNFα and IL-6 decreased gradually after surgical operation in both groups, but the reducing velocity was shorter in the KYL group than that in the control group, so the comparison of the levels in the two groups showed significant difference on the 3rd day after operation. Conclusion:KYL could inhibit the release of inflammatory mediator and relieve the inflammatory response so as to treat post-operational SIRS/MODS effectively.

  15. Airway distensibility in Chronic Obstructive Airway Disease

    DEFF Research Database (Denmark)

    Winkler Wille, Mathilde Marie; Pedersen, Jesper Holst; Dirksen, Asger;

    2013-01-01

    -dose CT for a period of 5 years (table 1). Images were reconstructed both with high contrast resolution (3 mm, kernel C) for emphysema analysis and with high spatial resolution (1 mm, kernel D) for airway analysis. Images were analysed by in-house developed software designed to segment lungs and localize...... the interior and exterior airway wall surface in three dimensions, and branches were matched in consecutive scans by image registration. Emphysema was defined as attenuation limits were set at

  16. Noninvasive clearance of airway secretions.

    Science.gov (United States)

    Hardy, K A; Anderson, B D

    1996-06-01

    Airway clearance techniques are indicated for specific diseases that have known clearance abnormalities (Table 2). Murray and others have commented that such techniques are required only for patients with a daily sputum production of greater than 30 mL. The authors have observed that patients with diseases known to cause clearance abnormalities can have sputum clearance with some techniques, such as positive expiratory pressure, autogenic drainage, and active cycle of breathing techniques, when PDPV has not been effective. Hasani et al has shown that use of the forced exhalatory technique in patients with nonproductive cough still resulted in movement of secretions proximally from all regions of the lung in patients with airway obstruction. It is therefore reasonable to consider airway clearance techniques for any patient who has a disease known to alter mucous clearance, including CF, dyskinetic cilia syndromes, and bronchiectasis from any cause. Patients with atelectasis from mucous plugs and hypersecretory states, such as asthma and chronic bronchitis, patients with pain secondary to surgical procedures, and patients with neuromuscular disease, weak cough, and abnormal patency of the airway may also benefit from the application of airway clearance techniques. Infants and children up to 3 years of age with airway clearance problems need to be treated with PDPV. Manual percussion with hands alone or a flexible face mask or cup and small mechanical vibrator/percussors, such as the ultrasonic devices, can be used. The intrapulmonary percussive ventilator shows growing promise in this area. The high-frequency oscillator is not supplied with vests of appropriate sizes for tiny babies and has not been studied in this group. Young patients with neuromuscular disease may require assisted ventilation and airway oscillations can be applied. CPAP alone has been shown to improve achievable flow rates that will increase air-liquid interactions for patients with these diseases

  17. Nasal continuous positive airway pressure combined with surfactant and NO for treatment of respiratory distress syndrome, prevention of bronchopulmonary dysplasia, and brain protection

    Institute of Scientific and Technical Information of China (English)

    Henrik Verder

    2010-01-01

    @@ Respiratory distress syndrome (RDS) is the single most important cause of mortality and morbidity in preterm infants and bronchopulmonary dysplasia (BPD)is a leading cause of neuro-muscular disablement and decreased lung function in the most preterm infants.

  18. Early versus delayed initiation of nasal continuous positive airway pressure for treatment of respiratory distress syndrome in premature newborns: A randomized clinical trial

    Directory of Open Access Journals (Sweden)

    Zohreh Badiee

    2013-01-01

    Conclusions: Early n CPAP is more effective than late n CPAP for the treatment of respiratory distress syndrome. In addition, the early use of n CPAP would reduce the need for some invasive procedures such as intubation and mechanical ventilation.

  19. Study of a human immunodeficiency virus-associated multiple-tissue dysfunction syndrome in one boy: Analysis of multiple tissues using X-ray, computed tomography and magnetic resonance imaging

    OpenAIRE

    Xichao Xia; Qingfu Hu; Shifeng Zhang; Juan Cui; Bingqin Shi; Hongwen Li; Hongjun Li; Xizhong Jia; Lei Jiang; Rongzhi Liu

    2016-01-01

    In the previous study, reports of human immunodeficiency virus (HIV)-associated multiple tissue dysfunction syndromes are limit. Now, multiple tissue lesions of one boy with AIDS were analyzed using X-ray, computed tomography (CT) and magnetic resonance imaging (MRI). Results showed that obvious brain atrophy and several focus were detected by CT and MRI. Lung lymphoid inflammation and pulmonary lymphoplasia were observed by CT. A swelling with a slight low density in hepatic anterior region ...

  20. Engineering Airway Epithelium

    Directory of Open Access Journals (Sweden)

    John P. Soleas

    2012-01-01

    Full Text Available Airway epithelium is constantly presented with injurious signals, yet under healthy circumstances, the epithelium maintains its innate immune barrier and mucociliary elevator function. This suggests that airway epithelium has regenerative potential (I. R. Telford and C. F. Bridgman, 1990. In practice, however, airway regeneration is problematic because of slow turnover and dedifferentiation of epithelium thereby hindering regeneration and increasing time necessary for full maturation and function. Based on the anatomy and biology of the airway epithelium, a variety of tissue engineering tools available could be utilized to overcome the barriers currently seen in airway epithelial generation. This paper describes the structure, function, and repair mechanisms in native epithelium and highlights specific and manipulatable tissue engineering signals that could be of great use in the creation of artificial airway epithelium.

  1. Short-term effects of self-massage combined with home exercise on pain, daily activity, and autonomic function in patients with myofascial pain dysfunction syndrome.

    Science.gov (United States)

    Chan, Yuan-Chi; Wang, Tzyy-Jiuan; Chang, Cheng-Chiang; Chen, Liang-Cheng; Chu, Heng-Yi; Lin, Shiou-Ping; Chang, Shin-Tsu

    2015-01-01

    [Purpose] The aim of the present was to investigate the short-term effects of a program combining self-massage and home exercise for patients with myofascial pain dysfunction syndrome (MPDS). [Subjects and Methods] In this retrospective study, 63 patients were allocated to the experimental (n = 32) and control (n = 31) groups. Both groups received 6 sessions of treatment with physical modalities over the course of two weeks. The experimental group completed an additional program with a combination of self-massage and home exercise. The outcome measurements included a pain scale, pressure pain threshold (PPT), neck disability index (NDI), patient-specific functional scales (PSFS), and heart rate variability (HRV). The interactions between the groups and over time were analyzed using two-way repeated measures ANOVA. [Results] Only the experimental group demonstrated significant improvements in the pain scale with varying conditions. The PPTs of the trigger points increased significantly in the experimental group, and significant functional improvements in NDI and PSFS were observed in the same group. There were significant increases in high-frequency HRV and high-frequency % in the experimental group. [Conclusion] Treatment with physical modalities plus combination of self-massage and home exercise is more effective than the physical modalities treatment alone.

  2. Engineering Airway Epithelium

    OpenAIRE

    John P. Soleas; Paz, Ana; Marcus, Paula; McGuigan, Alison; Waddell, Thomas K.

    2012-01-01

    Airway epithelium is constantly presented with injurious signals, yet under healthy circumstances, the epithelium maintains its innate immune barrier and mucociliary elevator function. This suggests that airway epithelium has regenerative potential (I. R. Telford and C. F. Bridgman, 1990). In practice, however, airway regeneration is problematic because of slow turnover and dedifferentiation of epithelium thereby hindering regeneration and increasing time necessary for full maturation and fun...

  3. Barth syndrome: cellular compensation of mitochondrial dysfunction and apoptosis inhibition due to changes in cardiolipin remodeling linked to tafazzin (TAZ) gene mutation.

    Science.gov (United States)

    Gonzalvez, François; D'Aurelio, Marilena; Boutant, Marie; Moustapha, Aoula; Puech, Jean-Philippe; Landes, Thomas; Arnauné-Pelloquin, Laeticia; Vial, Guillaume; Taleux, Nellie; Slomianny, Christian; Wanders, Ronald J; Houtkooper, Riekelt H; Bellenguer, Pascale; Møller, Ian Max; Gottlieb, Eyal; Vaz, Frederic M; Manfredi, Giovanni; Petit, Patrice X

    2013-08-01

    Cardiolipin is a mitochondrion-specific phospholipid that stabilizes the assembly of respiratory chain complexes, favoring full-yield operation. It also mediates key steps in apoptosis. In Barth syndrome, an X chromosome-linked cardiomyopathy caused by tafazzin mutations, cardiolipins display acyl chain modifications and are present at abnormally low concentrations, whereas monolysocardiolipin accumulates. Using immortalized lymphoblasts from Barth syndrome patients, we showed that the production of abnormal cardiolipin led to mitochondrial alterations. Indeed, the lack of normal cardiolipin led to changes in electron transport chain stability, resulting in cellular defects. We found a destabilization of the supercomplex (respirasome) I+III2+IVn but also decreased amounts of individual complexes I and IV and supercomplexes I+III and III+IV. No changes were observed in the amounts of individual complex III and complex II. We also found decreased levels of complex V. This complex is not part of the supercomplex suggesting that cardiolipin is required not only for the association/stabilization of the complexes into supercomplexes but also for the modulation of the amount of individual respiratory chain complexes. However, these alterations were compensated by an increase in mitochondrial mass, as demonstrated by electron microscopy and measurements of citrate synthase activity. We suggest that this compensatory increase in mitochondrial content prevents a decrease in mitochondrial respiration and ATP synthesis in the cells. We also show, by extensive flow cytometry analysis, that the type II apoptosis pathway was blocked at the mitochondrial level and that the mitochondria of patients with Barth syndrome cannot bind active caspase-8. Signal transduction is thus blocked before any mitochondrial event can occur. Remarkably, basal levels of superoxide anion production were slightly higher in patients' cells than in control cells as previously evidenced via an increased

  4. Conquering the difficult airway.

    Science.gov (United States)

    Gandy, William E

    2008-01-01

    Every medic should practice regularly for the inevitable difficult airway case. Practice should include review of the causes of difficult airways, as well as skill practice. Having a preassembled airway kit can make your response to an unexpected difficult situation easier. Of all the devices mentioned, the bougie is the airway practitioner's best friend. Using the BURP technique, if not contraindicated, together with the bougie will enable you to intubate many difficult patients with confidence. Remember, "If your patient cannot breathe, nothing else matters. PMID:18251307

  5. Adipocyte dysfunction in a mouse model of polycystic ovary syndrome (PCOS: evidence of adipocyte hypertrophy and tissue-specific inflammation.

    Directory of Open Access Journals (Sweden)

    Joseph S Marino

    Full Text Available Clinical research shows an association between polycystic ovary syndrome (PCOS and chronic inflammation, a pathological state thought to contribute to insulin resistance. The underlying pathways, however, have not been defined. The purpose of this study was to characterize the inflammatory state of a novel mouse model of PCOS. Female mice lacking leptin and insulin receptors in pro-opiomelanocortin neurons (IR/LepR(POMC mice and littermate controls were evaluated for estrous cyclicity, ovarian and adipose tissue morphology, and body composition by QMR and CT scan. Tissue-specific macrophage infiltration and cytokine mRNA expression were measured, as well as circulating cytokine levels. Finally, glucose regulation during pregnancy was evaluated as a measure of risk for diabetes development. Forty-five percent of IR/LepR(POMC mice showed reduced or absent ovulation. IR/LepR(POMC mice also had increased fat mass and adipocyte hypertrophy. These traits accompanied elevations in macrophage accumulation and inflammatory cytokine production in perigonadal adipose tissue, liver, and ovary. These mice also exhibited gestational hyperglycemia as predicted. This report is the first to show the presence of inflammation in IR/LepR(POMC mice, which develop a PCOS-like phenotype. Thus, IR/LepR(POMC mice may serve as a new mouse model to clarify the involvement of adipose and liver tissue in the pathogenesis and etiology of PCOS, allowing more targeted research on the development of PCOS and potential therapeutic interventions.

  6. 双水平正压通气在早产儿呼吸窘迫综合征治疗中的应用%A clinical trial of duo positive airway pressure ventilation versus nasal continuous positive airway pressure in treatment of preterm infant with neonatal respiratory distress syndrome after INSURE

    Institute of Scientific and Technical Information of China (English)

    戴立英; 张健; 王琍琍

    2014-01-01

    Objective To explore the efficacy of duo positive airway pressure ventilation in treating preterm infant with neonatal respiratory distress syndrome (RDS). Methods 65 preterm infants admitted to the neonatal intensive care unit from December 2012 to December 2013 were randomly divided into DuoPAP group (34 cases) or NCPAP (31 cases) group. Blood gas analysis(pH value, PaO2, PaCO2 and OI) at 1h, 12h, 24h, and 72h, and incidence of apnea, pulmonary air leak, repeated application of pulmonary surfactant and non-invasive ventilation failure at 72h in the two groups were measured and compared after using intubate surfactant extubate (INSURE). Results OI after non-invasive ventilation at 1h, 12h, and 24h was signiifcantly higherin DuoPAP group than that in NCPAP group P0.05)。结论 DuoPAP治疗NRDS与nCPAP相比能更快改善氧合,减少CO2潴留,减少有创机械通气比例。

  7. Acute and long term effects of grape and pomegranate juice consumption on endothelial dysfunction in pediatric metabolic syndrome

    Directory of Open Access Journals (Sweden)

    Roya Kelishadi

    2011-01-01

    Full Text Available Background: This study aimed to determine the short- and long-term effects of consumption of grape and pomegranate juices on markers of endothelial function and inflammation in adolescents with metabolic syndrome (MetS. Methods: In a non-pharmacologic randomized controlled trial, 30 individuals were randomly assigned to two groups of drinking natural grape or pomegranate juice for 1 month. Measurements of inflammatory factors [Hs-CRP, sE-selectin, sICAM-1, sVCAM, and interleukin 6 (IL-6] and flow-mediated dilation (FMD were made at baseline, 4 hours after first juice consumption and after one month of juice consumption. Results: The percent changes of FMD were significant in both groups in the short- and long-term. Hs-CRP had a nonsignificant decrease. sE selectin had a significant decrease after 4 hours in total and in the pomegranate juice group, followed by a significant decrease after 1 month in both groups. After 4 hours, sICAM-1 significantly decreased in the pomegranate juice group, and after 1 month it decreased in total and pomegranate juice group. Interleulkin-6 (IL-6 had a significant constant decrease at 4-hour and 1-month measurements after drinking pomegranate juice, and in both groups after 1 month. Significant negative correlations of changes in sICAM-1 and sE-selectin with changes in FMD were found in both periods of follow-up; and at 1 month for IL-6. Conclusions: Decline in inflammation was associated with improvement in FMD without changes in conventional risk factors. Daily consumption of natural antioxidants may improve endothelial function in adolescents with MetS.

  8. Left ventricular systolic dysfunction in asymptomatic Marfan syndrome patients is related to the severity of gene mutation: insights from the novel three dimensional speckle tracking echocardiography.

    Directory of Open Access Journals (Sweden)

    Mohamed Abd El Rahman

    Full Text Available In asymptomatic Marfan syndrome (MFS patients we evaluated the relationship between the types of fibrillin-1 (FBN1 gene mutation and possible altered left ventricular (LV function as assessed by three-dimensional speckle tracking echocardiography (3D-STE.Forty-five MFS patients (mean age 24 ± 15 years and 40 age-matched healthy controls were studied. Genetic evaluation for the FBN1 gene was carried on 32 MFS patients. Gene mutation (n = 15, 47% was classified as mild when the mutation resulted in nearly normally functioning protein, while mutations resulting in abnormally function protein were considered to be severe (n = 17, 53%. All patients and controls underwent 3D-STE for evaluation of LV function by an echocardiographer blinded to the results of the genetic testing. Compared to controls, MFS patients had significantly lower 3D-STE derived LV ejection fraction (EF, 57.43 ± 7.51 vs. 62.69 ± 4.76%, p = 0.0001, global LV longitudinal strain (LS, 14.85 ± 2.89 vs. 17.90 ± 2.01%, p = 0.0001, global LV circumferential strain (CS, 13.93 ± 2.81 vs. 16.82 ± 2.17%, p = 0.0001 and global LV area strain (AS, 25.76 ± 4.43 vs. 30.51 ± 2.61%, p = 0.0001. Apart from the global LV LS all these parameters were significantly lower in patients with severe gene mutation than in those with mild mutation (p < 0.05. In the multivariate linear regression analysis only the type of mutation had a significant influence on the 3D-STE derived LVEF (p = 0.017, global CS (p = 0.005 and global AS (p = 0.03.In asymptomatic MFS patients latent LV dysfunction can be detected using 3D STE. The LV dysfunction is mainly related to the severity of gene mutation, suggesting possible primary cardiomyopathy in MFS patients.

  9. Role of endothelin in microvascular dysfunction following percutaneous coronary intervention for non-ST elevation acute coronary syndromes: a single-centre randomised controlled trial

    Science.gov (United States)

    Guddeti, Raviteja R; Prasad, Abhiram; Matsuzawa, Yasushi; Aoki, Tatsuo; Rihal, Charanjit; Holmes, David; Best, Patricia; Lennon, Ryan J; Lerman, Lilach O; Lerman, Amir

    2016-01-01

    Objectives Percutaneous coronary intervention (PCI) for acute coronary syndromes frequently fails to restore myocardial perfusion despite establishing epicardial vessel patency. Endothelin-1 (ET-1) is a potent vasoconstrictor, and its expression is increased in atherosclerosis and after PCI. In this study, we aim to define the role of endothelin in regulating coronary microvascular blood flow and myocardial perfusion following PCI in patients with non-ST elevation acute coronary syndromes (NSTACS), by assessing whether adjunctive therapy with a selective endothelin A (ETA) receptor antagonist acutely improves postprocedural coronary microvascular blood flow. Methods In a randomised, double-blinded, placebo-controlled trial, 23 NSTACS patients were enrolled to receive an intracoronary infusion of placebo (n=11) or BQ-123 (n=12) immediately before PCI. Post-PCI coronary microvascular blood flow and myocardial perfusion were assessed by measuring Doppler-derived average peak velocity (APV), and cardiac biomarker levels were quantified. Results Compared with the placebo group, APV was significantly higher in the drug group immediately after PCI (30 (20, 37) vs 19 (9, 26) cm/s; p=0.03). Hyperaemic APV, measured post-adenosine administration, was higher in the BQ-123 group, but the difference did not achieve statistical significance (56 (48, 72) vs 46 (34, 64) cm/s; p=0.090). Maximum coronary flow reserve postprocedure was not different between the two groups (2.1 (1.6, 2.3) vs 2.5 (1.8, 3.0)). Per cent change in creatine kinase isoenzyme MB from the time of PCI to 8 and 16 hours post-PCI was significantly lower in the drug group compared with the placebo group (−17 (−26, −10) vs 26 (−15, 134); p=0.02 and −17 (−38, 14) vs 107 (2, 446); p=0.007, respectively). Conclusions Endothelin is a mediator of microvascular dysfunction during PCI in NSTACS, and adjunctive selective ETA antagonist may augment myocardial perfusion during PCI. Trial registration number

  10. Pattern of cytokine (IL-6 and IL-10) level as inflammation and anti-inflammation mediator of multiple organ dysfunction syndrome (MODS) in polytrauma.

    Science.gov (United States)

    Sapan, Heber Bombang; Paturusi, Idrus; Jusuf, Irawan; Patellongi, Ilhamjaya; Massi, Muh Nasrum; Pusponegoro, Aryono Djuned; Arief, Syafrie Kamsul; Labeda, Ibrahim; Islam, Andi Asadul; Rendy, Leo; Hatta, Mochammad

    2016-01-01

    Massive injury remains the most common cause of death for productive age group globally. The current immune, inflammatory paradigm, based on an incomplete understanding of the functional integration of the complex host response, remains a major impediment to the development of effective innovative diagnostic and therapeutic effort. This study attempt to investigate the pattern of inflammatory and anti-inflammatory cytokines such as interleukin-6 and 10 (IL-6 and IL-10) and their interaction in severe injury condition with its major complication as multiple organ dysfunction syndrome (MODS) and failure (MOF) after polytrauma. This is multicenter study held at 4 academic Level-1 Trauma center included 54 polytrauma participants. Inclusion criteria were age between 16-60 years old, had new acute episode of polytrauma which defined as injury in ≥2 body region with Injury Severity Score (ISS) ≥16, and the presence of Systemic Inflammation Response Syndrome (SIRS). Serum level of IL-6 and IL-10 were taken on day 2, 3, and 5 after trauma. During hospitalization, samples were observed for the occurrence of MODS or MOF using Sequential Organ Failure Assessment (SOFA) and mortality rate were also noted. Participant were mostly male with mean of age of 35, 9 years old, endured polytrauma caused by traffic accident. Elevation of cytokines (IL-6, IL-10, and IL-6/IL-10 ratio) had directly proportional with MODS and mortality. Threshold level of compensation for severe trauma is IL-6 of 50 pg/mL and trauma load of ISS ≥30. Inflammation reaction greater than this threshold level would result in downhill level of IL-6, IL-10, or IL-6/IL-10 ratio which associated with poor outcome (MODS and death). The elevation of these cytokines level were represent as compensation/adaptive immune system and its fall represent decompensating/failure of immune system after severe trauma. The pattern of IL-6 and IL-10 after polytrauma represent immune system effort to restore homeostasis

  11. USEFULNESS OF SEQUENTIAL ORGAN FAILURE ASSESSMENT (SOFA AND ACUTE PHYSIOLOGY AND CHRONIC HEALTH EVALUATION II (APACHE II SCORE IN ANALYSING PATIENTS WITH MULTIPLE ORGAN DYSFUNCTION SYNDROME IN SEPSIS

    Directory of Open Access Journals (Sweden)

    Abhinandan

    2013-12-01

    Full Text Available Sepsis with multiple organ dysfunction syndrome (MODS is a common cause of Intensive Care Unit (ICU mortality and morbidity. Early initiation of appro priate effective antimicrobial therapy is essential for a favorable outcome in the patient with sepsis. Cultures and serology are available only after 24 to 48 hours. In the crucial hours which determine the prognosis of the patient the physician has to de pend on clinical symptoms and demographic data to aid in diagnosis and management. Using scores like APACHE II at the admission and SOFA on admission and also in their due course may help in predicting outcome. Though there are some studies on sepsis in In dia but use of APACHE II and SOFA scores in India have been rare. OBJECTIVES: To assess morbidity and mortality of patients with multi - organ dysfunction syndrome in sepsis. To prognosticate the patients by using defined scores like SOFA and APACHE II score s. MATERIALS AND METHODS: The study was carried out in the period of November 2010 to September 2012 and 50 patients were included in the study. The detailed history, clinical examination and all the relevant laboratory investigations were done including b lood culture. In the present study the conditions were defined according to standard practice and based on relevant literature. All the patients of sepsis admitted to ICU/ emergency ward were prognosticated on the basis of APACHE II score and SOFA score. W e have analysed various profiles between two groups; survivor group which include the patients who are successfully discharged after recovery and non - survivor group which include the patients who died. RESULTS: The clinical profile of 50 patients with seps is with MODS was studied. There were 28 males and 22 females in this cohort. In this study, 18 patients died and 32 patients survived with mortality rate of 36%. In this study, though APACHE II score was high among non survivors than survivors (23.28 v/s 1 8.75, it was of just

  12. Does Pre-Treatment with High Dose Atorvastatin Prevent Microvascular Dysfunction after Percutaneous Coronary Intervention in Patients with Acute Coronary Syndrome?

    Science.gov (United States)

    Lee, Bong-Ki; Nam, Chang-Wook; Doh, Joon-Hyung; Chung, Woo-Young; Cho, Byung-Ryul; Fearon, William F.

    2016-01-01

    Background and Objectives There is controversy surrounding whether or not high dose statin administration before percutaneous coronary intervention (PCI) decreases peri-procedural microvascular injury. We performed a prospective randomized study to investigate the mechanisms and effects of pre-treatment high dose atorvastatin on myocardial damage in patients with non-ST-segment elevation acute coronary syndrome (NSTE-ACS) undergoing PCI. Subjects and Methods Seventy seven patients with NSTE-ACS were randomly assigned to either the high dose group (atorvastatin 80 mg loading 12 to 24 h before PCI with a further 40 mg loading 2 h before PCI, n=39) or low dose group (atorvastatin 10 mg administration 12 to 24 h before PCI, n=38). Index of microcirculatory resistance (IMR) was measured after stent implantation. Creatine kinase-myocardial band (CK-MB) and high sensitivity C-reactive protein (CRP) levels were measured before and after PCI. Results The baseline characteristics were not different between the two patient groups. Compared to the low dose group, the high dose group had lower post PCI IMR (14.1±5.0 vs. 19.2±9.3 U, p=0.003). Post PCI CK-MB was also lower in the high dose group (median: 1.40 ng/mL (interquartile range [IQR: 0.75 to 3.45] vs. 4.00 [IQR: 1.70 to 7.37], p=0.002) as was the post-PCI CRP level (0.09 mg/dL [IQR: 0.04 to 0.16] vs. 0.22 [IQR: 0.08 to 0.60], p=0.001). Conclusion Pre-treatment with high dose atorvastatin reduces peri-PCI microvascular dysfunction verified by post-PCI IMR and exerts an immediate anti-inflammatory effect in patients with NSTE-ACS. PMID:27482255

  13. Erectile Dysfunction in Chronic Prostatitis/Chronic Pelvic Pain Syndrome: Outcomes from a Multi-Center Study and Risk Factor Analysis in a Single Center.

    Directory of Open Access Journals (Sweden)

    Yadong Zhang

    Full Text Available The aim of this study was to investigate the prevalence of erectile dysfunction (ED in patients with chronic prostatitis/chronic pelvic pain syndrome (CP/CPPS and explore the influence of UPOINT domains, National Institutes of Health-CP symptom index (NIH-CPSI and other factors on ED prevalence. This was a prospective study of consecutive patients with CP/CPPS seen at 11 tertiary hospitals during January-July 2014. ED was diagnosed as a score of<21 on the International Index of Erectile Function (IIEF-5. Patients from one center were evaluated by the UPOINT system and NIH-CPSI. Each patient was assessed using clinical examination, asocio-demographic questionnaire, the Patient Health Questionnaire (PHQ, the Pain Catastrophizing Scale (PCS, NIH-CPSI and IIEF-5.1406 patients from 11 centers (mean age, 32.18 years; range 18-60 years were enrolled. ED was found in 638/1406 patients (45.4%, and was categorized as mild in 291(45.6%, moderate in 297(46.6% and severe in50(7.7%. 192 patients from one center(mean age,31.3 years; range 18-57 years were further studied.IIEF-5 score correlated negatively with NIH-CPSI(r = 0.251, PHQ (r = 0.355 and PCS (r = 0.322scores (P<0.001.PHQ score correlated positively with NIH-CPSI (r = 0.586 and PCS(r = 0.662 scores (P<0.001.NIH-CPSI, PHQ, PCS and IIEF-5 scores did not differ significantly between class IIIA and IIIB CP/CPPS. Multivariate logistic regression showed that UPOINT psychological (P domain and NIH-CPSI symptom severity were independent risk factors for ED in CP/CPPS. It is concluded that psychological factors and symptom severity are independent risk factors for ED in CP/CPPS.

  14. Airway Clearance Devices for Cystic Fibrosis

    Science.gov (United States)

    2009-01-01

    Executive Summary Objective The purpose of this evidence-based analysis is to examine the safety and efficacy of airway clearance devices (ACDs) for cystic fibrosis and attempt to differentiate between devices, where possible, on grounds of clinical efficacy, quality of life, safety and/or patient preference. Background Cystic fibrosis (CF) is a common, inherited, life-limiting disease that affects multiple systems of the human body. Respiratory dysfunction is the primary complication and leading cause of death due to CF. CF causes abnormal mucus secretion in the airways, leading to airway obstruction and mucus plugging, which in turn can lead to bacterial infection and further mucous production. Over time, this almost cyclical process contributes to severe airway damage and loss of respiratory function. Removal of airway secretions, termed airway clearance, is thus an integral component of the management of CF. A variety of methods are available for airway clearance, some requiring mechanical devices, others physical manipulation of the body (e.g. physiotherapy). Conventional chest physiotherapy (CCPT), through the assistance of a caregiver, is the current standard of care for achieving airway clearance, particularly in young patients up to the ages of six or seven. CF patients are, however, living much longer now than in decades past. The median age of survival in Canada has risen to 37.0 years for the period of 1998-2002 (5-year window), up from 22.8 years for the 5-year window ending in 1977. The prevalence has also risen accordingly, last recorded as 3,453 in Canada in 2002, up from 1,630 in 1977. With individuals living longer, there is a greater need for independent methods of airway clearance. Airway Clearance Devices There are at least three classes of airway clearance devices: positive expiratory pressure devices (PEP), airway oscillating devices (AOD; either handheld or stationary) and high frequency chest compression (HFCC)/mechanical percussion (MP

  15. Blockage of upper airway

    Science.gov (United States)

    ... is made through the neck into the airway ( tracheostomy or cricothyrotomy). If the obstruction is due to ... team. Related MedlinePlus Health Topics Choking Throat Disorders Tracheal Disorders Browse the Encyclopedia A.D.A.M., Inc. ...

  16. Effects of a mandibular advancement device on the upper airway morphology : a cephalometric analysis

    NARCIS (Netherlands)

    Doff, M. H. J.; Hoekema, A.; Pruim, G. J.; van der Hoeven, J. H.; de Bont, L. G. M.; Stegenga, B.

    2009-01-01

    The aims of this study were to assess changes in the upper airway morphology associated with an oral appliance in situ in patients suffering from the obstructive sleep apnoea-hypopnoea syndrome and to relate these changes to treatment response. Changes in upper airway morphology as a result of an or

  17. Equine recurrent airway obstruction

    OpenAIRE

    Artur Niedźwiedź

    2014-01-01

    Equine Recurrent Airway Obstruction (RAO), also known as heaves or broken wind, is one of the most common disease in middle-aged horses. Inflammation of the airway is inducted by organic dust exposure. This disease is characterized by neutrophilic inflammation, bronchospasm, excessive mucus production and pathologic changes in the bronchiolar walls. Clinical signs are resolved in 3-4 weeks after environmental changes. Horses suffering from RAO are susceptible to allergens throughout their liv...

  18. 肝脾肾功能失调与代谢综合征的关系探讨%Discussion of the Relation between Dysfunction of Liver Spleen and Kidney and Metabolic Syndrome

    Institute of Scientific and Technical Information of China (English)

    王昌儒; 向楠

    2013-01-01

    目的:用中医理论,从肝脾肾功能失调入手,探讨代谢综合征的病理基础,为中医药治疗代谢综合征提供理论支撑。方法:运用中医药理论,对代谢综合征的形成机理进行论证。结果:能量代谢紊乱是代谢综合征形成的根源,脾肾阳虚会使人体的气机运行失常,引起能量代谢失衡从而发生代谢综合征。结论:代谢综合征发生的中医病机当为脾肾阳虚、肝气郁滞、痰浊内阻,对于代谢综合征的治疗,应以疏肝温肾健脾化痰为主。%Objective:To study the pathological basis of metabolic syndrome and provide theoretic support for TCM in treating metabolic syndrome based on the connotations of the dysfunction of liver spleen and kidney .Methods:Discuss on the formation of metabolic syn-drome with TCM theories .Results: The disturbance of energy metabolism is the source of metabolic syndrome .Yang deficiency of spleen and kidney can cause Qi movement disorders that lead to metabolic syndrome .Conclusion:Yang deficiency of spleen and kidney and phlegm resistance is the pathogenesis of metabolic syndrome , thus warming the kidney and strengthening the spleen is the right scheme for the treatment of metabolic syndrome .

  19. Bladder Dysfunction and Vesicoureteral Reflux

    Directory of Open Access Journals (Sweden)

    Ulla Sillén

    2008-01-01

    Full Text Available In this overview the influence of functional bladder disturbances and of its treatment on the resolution of vesicoureteral reflux (VUR in children is discussed. Historically both bladder dysfunction entities, the overactive bladder (OAB and the dysfunctional voiding (DV, have been described in conjunction with VUR. Treatment of the dysfunction was also considered to influence spontaneous resolution in a positive way. During the last decades, however, papers have been published which could not support these results. Regarding the OAB, a prospective study with treatment of the bladder overactivity with anticholinergics, did not influence spontaneous resolution rate in children with a dysfunction including also the voiding phase, DV and DES (dysfunctional elimination syndrome, most studies indicate a negative influence on the resolution rate of VUR in children, both before and after the age for bladder control, both with and without treatment. However, a couple of uncontrolled studies indicate that there is a high short-term resolution rate after treatment with flow biofeedback. It should be emphasized that the voiding phase dysfunctions (DV and DES are more severe than the genuine filling phase dysfunction (OAB, with an increased frequency of UTI and renal damage in the former groups. To be able to answer the question if treatment of bladder dysfunction influence the resolution rate of VUR in children, randomized controlled studies must be performed.

  20. Salivary changes and dental caries as potential oral markers of autoimmune salivary gland dysfunction in primary Sjögren's syndrome

    Directory of Open Access Journals (Sweden)

    Nauntofte Birgitte

    2005-03-01

    Full Text Available Abstract Background the classification criteria for primary Sjögren's syndrome (pSS include a number of oral components. In this study we evaluated if salivary flow and composition as well as dental caries are oral markers of disease severity in pSS. Methods in 20 patients fulfilling the American-European Consensus criteria for pSS and 20 age-matched healthy controls whole and parotid saliva flow rates and composition, measures of oral dryness, scores of decayed, missing and filled tooth surfaces (DMFS, periodontal indices, oral hygiene, and dietary habits were examined. Results in pSS, salivary flow rates, pH, and buffer capacities were lower, and DMFS, salivary sodium and chloride concentrations higher than in the healthy controls. DMFS also correlated inversely to salivary flow rates and positively to oral dryness. Apart from slightly increased gingival index, and more frequent dental visits in pSS, the periodontal condition, oral hygiene or sugar intake did not differ between these two groups. In pSS, findings were correlated to labial salivary gland focus score (FS and presence of serum-autoantibodies to SSA/SSB (AB. The patients having both presence of AB and the highest FS (>2 also had the highest salivary sodium and chloride concentrations, the lowest salivary phosphate concentrations, lowest salivary flow rates, and highest DMFS compared to those with normal salivary concentrations of sodium and chloride at a given flow rate. Conclusion the salivary changes observed in some pSS patients reflect impaired ductal salt reabsorption, but unaffected acinar transport mechanisms, despite low salivary secretion. Our results suggest that changes in salivary flow and composition as well as dental caries may serve as potential markers of the extent of autoimmune-mediated salivary gland dysfunction in pSS. The study also indicates that the ductal epithelium is functionally affected in some pSS patients, which calls for future pathophysiological

  1. Pulmonary Surfactant Function in Alveoli and Conducting Airways

    Directory of Open Access Journals (Sweden)

    Goran Enhorning

    1996-01-01

    Full Text Available Surface tension plays a very important role in aeration of the neonate's lungs. Pulmonary surfactant, which is inadequate in the premature infant, modifies surface tension during the act of breathing and is necessary for maintenance of alveolar stability. These facts led to the development of the concept that it might be possible to treat the premature infant by supplementing the infant's inadequate surfactant supply. In addition to maintaining alveolar stability, pulmonary surfactant might also be of vital importance for maintenance of small airway patency. Various conditions, most importantly asthma, might be the reason for a surfactant dysfunction to develop. This in turn might cause airway resistance to increase.

  2. 脓毒症胃肠功能障碍中医证候分布规律探讨%Discussion on differentiation laws of traditional Chinese medicine syndromes of patients with sepsis and gastrointestinal dysfunction

    Institute of Scientific and Technical Information of China (English)

    孔立; 郭琪钰; 赵浩

    2013-01-01

      目的观察脓毒症胃肠功能障碍中医证候分布规律。方法选择2009年11月至2011年2月山东中医药大学附属医院收治的脓毒症胃肠功能障碍患者80例,观察患者的临床症状和体征,采集中医四诊信息,对患者症状和体征分布采用频次描述方法,对证候分布运用聚类分析的方法。结果80例患者中症状分布特点前5位依次为腹胀63例(78.7%)、纳呆43例(53.7%)、便秘40例(50.0%)、泄泻37例(46.2%)、脘痞35例(43.7%);体征分布特点舌象分布特点前5位依次为黄苔52例(65.0%)、舌淡红30例(37.5%)、薄苔29例(36.2%)、舌红24例(30.0%)、厚苔24例(30.0%);脉象分布特点前5位依次为脉数40例(50.0%)、脉沉21例(26.2%)、脉弱17例(21.2%)、脉滑14例(17.5%)、脉弦12例(15.0%)。结合聚类分析结果及患者的临床表现,脓毒症胃肠功能障碍患者的主要证型分布为实证63例(占78.8%),分别为肠热腑实证、胃热气滞证、痰湿蕴脾证、瘀滞胃肠证;虚证主要为脾胃气虚证17例(占21.3%)。结论脓毒症胃肠功能障碍的中医证候特点是实证与虚证并见,以实证为主。实证以肠热腑实证、胃热气滞证、痰湿蕴脾证、瘀滞胃肠证多见;虚证以脾胃气虚证多见。%  Objective To observe the differentiation laws of traditional Chinese medicine(TCM)syndromes of patients with sepsis and gastrointestinal dysfunction. Methods Eighty cases with sepsis and gastrointestinal dysfunction paid visits to the Affiliated Hospital of Shandong University of TCM in a period from November 2009 to February 2011. According to the four diagnostic methods of TCM,we gathered all patients' information about their clinical symptoms and signs,frequency describing methods were used to explore the distribution of patients' symptoms and signs and the cluster analysis was applied to determine the

  3. Relationship between airway pathophysiology and airway inflammation in older asthmatics

    DEFF Research Database (Denmark)

    Porsbjerg, Celeste M; Gibson, Peter G; Pretto, Jeffrey J;

    2013-01-01

    BACKGROUND AND OBJECTIVE: Asthma-related morbidity is greater in older compared with younger asthmatics. Airway closure is also greater in older asthmatics, an observation that may be explained by differences in airway inflammation. We hypothesized that in older adult patients with asthma......, neutrophil airway inflammation increases airway closure during bronchoconstriction, while eosinophil airway inflammation increases airway hyperresponsiveness (AHR). METHODS: Asthmatic subjects (n = 26), aged ≥55 years (68% female), were studied, and AHR to 4.5% saline challenge was measured by the response......-dose ratio (%fall in forced expiratory volume in 1 s (FEV1 )/mg saline). Airway closure was assessed during bronchoconstriction percent change in forced vital capacity (FVC)/percent change in FEV1 (i.e. Closing Index). Airway inflammation was assessed by induced sputum and exhaled nitric oxide (eNO). RESULTS...

  4. Role of upper airway ultrasound in airway management.

    Science.gov (United States)

    Osman, Adi; Sum, Kok Meng

    2016-01-01

    Upper airway ultrasound is a valuable, non-invasive, simple, and portable point of care ultrasound (POCUS) for evaluation of airway management even in anatomy distorted by pathology or trauma. Ultrasound enables us to identify important sonoanatomy of the upper airway such as thyroid cartilage, epiglottis, cricoid cartilage, cricothyroid membrane, tracheal cartilages, and esophagus. Understanding this applied sonoanatomy facilitates clinician to use ultrasound in assessment of airway anatomy for difficult intubation, ETT and LMA placement and depth, assessment of airway size, ultrasound-guided invasive procedures such as percutaneous needle cricothyroidotomy and tracheostomy, prediction of postextubation stridor and left double-lumen bronchial tube size, and detecting upper airway pathologies. Widespread POCUS awareness, better technological advancements, portability, and availability of ultrasound in most critical areas facilitate upper airway ultrasound to become the potential first-line non-invasive airway assessment tool in the future. PMID:27529028

  5. 双水平正压通气与持续呼吸道正压通气在早产儿呼吸窘迫综合征中的比较研究%Comparison of Bi-level positive airway pressure and continuous positive airway pressure in preterm infants with respiratory distress syndrome

    Institute of Scientific and Technical Information of China (English)

    白丽亚; 赵莉; 马玲彦; 王丹

    2015-01-01

    Objective:To compare the therapeutic effects for nasal bi-level positive airway pressure (BiPAP) and nasal continuous positive airway pressure (NCPAP) combined with pulmonary surfactant in preterm infants with respiratory distress syndrome (RDS) and to determine whether can reduce the need for intubation and mechanical ventilation.Methods: In this single-center, randomized controlled trial, preterm infants (gestational ages were 28-34+6weeks) with RDS were randomly assigned to BiPAP group (24 cases) and NCPAP group(25 cases). If the 2 noninvasive ventilation were not effective, intubation was administered as rescue therapy. The primary outcome was the need for mechanical ventilation within the first 24 hours, 48 hours, 78 hours of life. The secondary outcomes were incidence of pneumothorax, incidence of necrotizing enterocolitis, incidence of intraventricular hemorrhage(grade 3 and grade 4).Results: Rates of intubation in the first 24 hours did not differ significantly between the BiPAP group and NCPAP group, however, significantly more infants in the BiPAP group remained extubated compared with those in the NCPAP group within 48 hours, 72 hours (x2=4.056,x2=4.325;P<0.05). No significant differences were noted between the 2 treatment groups for the secondary outcomes.Conclusion: BiPAP can decrease the need for mechanical ventilation compared with NCPAP after 24 hours.%目的:比较双水平正压通气(BiPAP)和持续呼吸道正压通气(NCPAP)联合肺表面活性物质(PS)在早产儿呼吸窘迫综合征(RDS)中的应用效果,探讨是否可以降低气管插管有创呼吸支持率。方法:将胎龄在28~34周(+6 d)的RDS早产儿随机分为BiPAP组(24例)和NCPAP组(25例),常规使用PS进行替代治疗,若这两种方式不能维持患儿生命体征则使用气管内插管,接呼吸机辅助呼吸。主要观察指标为出生24 h、48 h及72 h插管有创呼吸支持率,次要观察指标为气胸发病率、坏死性小肠结肠炎

  6. Integrated care pathways for airway diseases (AIRWAYS-ICPs)

    NARCIS (Netherlands)

    Bousquet, J.; Addis, A.; Adcock, I.; Agache, I.; Agusti, A.; Alonso, A.; Annesi-Maesano, I.; Anto, J. M.; Bachert, C.; Baena-Cagnani, C. E.; Bai, C.; Baigenzhin, A.; Barbara, C.; Barnes, P. J.; Bateman, E. D.; Beck, L.; Bedbrook, A.; Bel, E. H.; Benezet, O.; Bennoor, K. S.; Benson, M.; Bernabeu-Wittel, M.; Bewick, M.; Bindslev-Jensen, C.; Blain, H.; Blasi, F.; Bonini, M.; Bonini, S.; Boulet, L. P.; Bourdin, A.; Bourret, R.; Bousquet, P. J.; Brightling, C. E.; Briggs, A.; Brozek, J.; Buh, R.; Bush, A.; Caimmi, D.; Calderon, M.; Calverley, P.; Camargos, P. A.; Camuzat, T.; Canonica, G. W.; Carlsen, K. H.; Casale, T. B.; Cazzola, M.; Sarabia, A. M. Cepeda; Cesario, A.; Chen, Y. Z.; Chkhartishvili, E.; Chavannes, N. H.; Chiron, R.; Chuchalin, A.; Chung, K. F.; Cox, L.; Crooks, G.; Crooks, M. G.; Cruz, A. A.; Custovic, A.; Dahl, R.; Dahlen, S. E.; De Blay, F.; Dedeu, T.; Deleanu, D.; Demoly, P.; Devillier, P.; Didier, A.; Dinh-Xuan, A. T.; Djukanovic, R.; Dokic, D.; Douagui, H.; Dubakiene, R.; Eglin, S.; Elliot, F.; Emuzyte, R.; Fabbri, L.; Wagner, A. Fink; Fletcher, M.; Fokkens, W. J.; Fonseca, J.; Franco, A.; Frith, P.; Furber, A.; Gaga, M.; Garces, J.; Garcia-Aymerich, J.; Gamkrelidze, A.; Gonzales-Diaz, S.; Gouzi, F.; Guzman, M. A.; Haahtela, T.; Harrison, D.; Hayot, M.; Heaney, L. G.; Heinrich, J.; Hellings, P. W.; Hooper, J.; Humbert, M.; Hyland, M.; Iaccarino, G.; Jakovenko, D.; Jardim, J. R.; Jeandel, C.; Jenkins, C.; Johnston, S. L.; Jonquet, O.; Joos, G.; Jung, K. S.; Kalayci, O.; Karunanithi, S.; Keil, T.; Khaltaev, N.; Kolek, V.; Kowalski, M. L.; Kull, I.; Kuna, P.; Kvedariene, V.; Le, L. T.; Carlsen, K. C. Lodrup; Louis, R.; MacNee, W.; Mair, A.; Majer, I.; Manning, P.; Keenoy, E. de Manuel; Masjedi, M. R.; Meten, E.; Melo-Gomes, E.; Menzies-Gow, A.; Mercier, G.; Mercier, J.; Michel, J. P.; Miculinic, N.; Mihaltan, F.; Milenkovic, B.; Molimard, M.; Mamas, I.; Montilla-Santana, A.; Morais-Almeida, M.; Morgan, M.; N'Diaye, M.; Nafti, S.; Nekam, K.; Neou, A.; Nicod, L.; O'Hehir, R.; Ohta, K.; Paggiaro, P.; Palkonen, S.; Palmer, S.; Papadopoulos, N. G.; Papi, A.; Passalacqua, G.; Pavord, I.; Pigearias, B.; Plavec, D.; Postma, D. S.; Price, D.; Rabe, K. F.; Pontal, F. Radier; Redon, J.; Rennard, S.; Roberts, J.; Robine, J. M.; Roca, J.; Roche, N.; Rodenas, F.; Roggeri, A.; Rolland, C.; Rosado-Pinto, J.; Ryan, D.; Samolinski, B.; Sanchez-Borges, M.; Schunemann, H. J.; Sheikh, A.; Shields, M.; Siafakas, N.; Sibille, Y.; Similowski, T.; Small, I.; Sola-Morales, O.; Sooronbaev, T.; Stelmach, R.; Sterk, P. J.; Stiris, T.; Sud, P.; Tellier, V.; To, T.; Todo-Bom, A.; Triggiani, M.; Valenta, R.; Valero, A. L.; Valiulis, A.; Valovirta, E.; Van Ganse, E.; Vandenplas, O.; Vasankari, T.; Vestbo, J.; Vezzani, G.; Viegi, G.; Visier, L.; Vogelmeier, C.; Vontetsianos, T.; Wagstaff, R.; Wahn, U.; Wallaert, B.; Whalley, B.; Wickman, M.; Williams, D. M.; Wilson, N.; Yawn, B. P.; Yiallouros, P. K.; Yorgancioglu, A.; Yusuf, O. M.; Zar, H. J.; Zhong, N.; Zidarn, M.; Zuberbier, T.

    2014-01-01

    The objective of Integrated Care Pathways for Airway Diseases (AIRWAYS-ICPs) is to launch a collaboration to develop multi-sectoral care pathways for chronic respiratory diseases in European countries and regions. AIRWAYS-ICPs has strategic relevance to the European Union Health Strategy and will ad

  7. Integrated care pathways for airway diseases (AIRWAYS-ICPs)

    DEFF Research Database (Denmark)

    Bousquet, J; Addis, A; Adcock, I;

    2014-01-01

    The objective of Integrated Care Pathways for Airway Diseases (AIRWAYS-ICPs) is to launch a collaboration to develop multi-sectoral care pathways for chronic respiratory diseases in European countries and regions. AIRWAYS-ICPs has strategic relevance to the European Union Health Strategy and will...

  8. Exercise and airway injury in athletes.

    Science.gov (United States)

    Couto, Mariana; Silva, Diana; Delgado, Luis; Moreira, André

    2013-01-01

    Olympic level athletes present an increased risk for asthma and allergy, especially those who take part in endurance sports, such as swimming or running, and in winter sports. Classical postulated mechanisms behind EIA include the osmotic, or airway-drying, hypothesis. Hyperventilation leads to evaporation of water and the airway surface liquid becomes hyperosmolar, providing a stimulus for water to move from any cell nearby, which results in the shrinkage of cells and the consequent release of inflammatory mediators that cause airway smooth muscle contraction. But the exercise-induced asthma/bronchoconstriction explanatory model in athletes probably comprises the interaction between environmental training factors, including allergens and ambient conditions such as temperature, humidity and air quality; and athlete's personal risk factors, such as genetic and neuroimmuneendocrine determinants. After the stress of training and competitions athletes experience higher rate of upper respiratory tract infections (URTI), compared with lesser active individuals. Increasing physical activity in non-athletes is associated with a decreased risk of URTI. Heavy exercise induces marked immunodepression which is multifactorial in origin. Prolonged, high intensity exercise temporarily impairs the immune competence while moderate activity may enhance immune function. The relationship between URTI and exercise is affected by poorly known individual determinants such genetic susceptibility, neurogenic mediated immune inflammation and epithelial barrier dysfunction. Further studies should better define the aetiologic factors and mechanisms involved in the development of asthma in athletes, and propose relevant preventive and therapeutic measures. PMID:23697359

  9. Exercise and airway injury in athletes.

    Science.gov (United States)

    Couto, Mariana; Silva, Diana; Delgado, Luis; Moreira, André

    2013-01-01

    Olympic level athletes present an increased risk for asthma and allergy, especially those who take part in endurance sports, such as swimming or running, and in winter sports. Classical postulated mechanisms behind EIA include the osmotic, or airway-drying, hypothesis. Hyperventilation leads to evaporation of water and the airway surface liquid becomes hyperosmolar, providing a stimulus for water to move from any cell nearby, which results in the shrinkage of cells and the consequent release of inflammatory mediators that cause airway smooth muscle contraction. But the exercise-induced asthma/bronchoconstriction explanatory model in athletes probably comprises the interaction between environmental training factors, including allergens and ambient conditions such as temperature, humidity and air quality; and athlete's personal risk factors, such as genetic and neuroimmuneendocrine determinants. After the stress of training and competitions athletes experience higher rate of upper respiratory tract infections (URTI), compared with lesser active individuals. Increasing physical activity in non-athletes is associated with a decreased risk of URTI. Heavy exercise induces marked immunodepression which is multifactorial in origin. Prolonged, high intensity exercise temporarily impairs the immune competence while moderate activity may enhance immune function. The relationship between URTI and exercise is affected by poorly known individual determinants such genetic susceptibility, neurogenic mediated immune inflammation and epithelial barrier dysfunction. Further studies should better define the aetiologic factors and mechanisms involved in the development of asthma in athletes, and propose relevant preventive and therapeutic measures.

  10. Airway reconstruction in children

    Directory of Open Access Journals (Sweden)

    Rao Sanjay

    2009-01-01

    Full Text Available Aim/Background : Airway anomalies are infrequent but potentially life threatening in children. A program to care for these difficult children was set up at our institution, and this paper summarizes our experience. Methods: A total of 34 children were enrolled in the program over a period of three years. These children were evaluated as per the standard protocols. Treatment was individualized. Results: Of these 34 children, 28 had their airways restored and are doing well. Four children continue to remain on tracheostomy and two will require long term tracheostomy. There were two deaths. All children are under surveillance as there is a risk of recurrence. Conclusions: Airway anomalies are complex problems with significant morbidity and mortality. Current therapeutic modalities allow for good results. Most children were successfully decannulated and did well.

  11. A research on TCM syndromes and related factors of patients with ovarian dysfunction, irregular menstruation%卵巢功能低下月经不调患者的中医证型及相关因素研究

    Institute of Scientific and Technical Information of China (English)

    朱俊芬

    2012-01-01

      目的:分析卵巢功能低下月经不调的中医证型和相关因素,以供今后的临床工作参考。方法:选择我院2010年10月~2011年9月收治的卵巢功能低下月经不调患者60例,对其临床资料进行回顾性分析,探讨其中医证型和相关因素。结果:卵巢功能低下月经不调的中医证型为肾阴亏虚证、肝郁气滞证,与患者年龄、初潮年龄、孕产次等因素有较大的关系。结论:卵巢功能低下月经不调与肝肾关系密切,更年期、初潮年龄较小、孕、产次较多的女性易发。%  Objective: To analyze the TCM syndromes and related factors of patients with ovarian dysfunction, irregular menstruation, for the future clinical work of reference. Methods: 60 patients with irregular menstruation were treated in our hospital from October 2010 to September 2011, and the clinical data of them were retrospectively analyzed, to explore its TCM syndromes and related factors. Results: TCM type of ovarian dysfunction, irregular menstruation was kidney yin vacuity, qi stagnation, that related to patient age, age at menarche and maternal inferior factors. Conclusion: The ovarian dysfunction, irregular menstruation and liver and kidney are closely related to menopause, age at menarche is less prone to pregnancy, birth more women.

  12. Overexpression of mclca3 in airway epithelium of asthmatic murine models with airway inflammation

    Institute of Scientific and Technical Information of China (English)

    ZHANG Hui-lan; HE Li

    2010-01-01

    Asthma is a worldwide prevalent disease that is a considerable health burden in many countries.1 In recent years, the airway epithelium is increasingly recognized as a central contributor to the pathogenesis of asthma.2 One of the most highly induced genes in epithelial cells in experimental allergic airway disease is the third murine calcium-activated chloride channel homologue (mclca3, alias gob-5). Its human homology protein is hCLCA1,3,4 which has been identified as clinically relevant molecules in diseases with secretory dysfunctions including asthma and cystic fibrosis. In initial studies, mclca3 was thought to be a member of calcium-activated chloride channel (CaCCs) family,whereas some new interesting reports suggest that the two mclca3 cleavage products cannot form an anion channel on their own but may instead act as extracellular signaling molecules with as yet unknown functions and interacting partners.5

  13. 经鼻呼气末气道正压治疗阻塞性睡眠呼吸暂停低通气综合征的研究进展%Research progress of a novel nasal expiratory positive airway pressure device for the treatment of obstructive sleep apnea hypopnea syndrome

    Institute of Scientific and Technical Information of China (English)

    魏月; 李庆云; 李宁

    2014-01-01

    OSAHS是一种常见的睡眠呼吸疾病,其特征是睡眠过程中上气道塌陷所致的呼吸暂停或低通气.经鼻呼气末气道正压(nasal expiratory positive airway pressure,nEPAP)治疗为OSAHS患者提供了一种新的治疗策略.多个研究证实nEPAP治疗可有效降低呼吸暂停低通气指数,改善夜间睡眠质量,且患者对该治疗的依从性良好.本文对近年来nEPAP治疗OSAHS的相关研究进行综述.%Obstructive sleep apnea hypopnea syndrome (OSAHS) is a prevalent breathing sleepdisorder,characterized by the recurrent collapse of the upper airway which induces apnea or hypopnea.Nasal expiratory positive airway pressure (nEPAP) provides a new treatment option for OSAHS.Several studies indicated that nEPAP contributed to obvious reduction of apnea hypopnea index and improvement of sleep quality,with good compliance.This review is intended to focus on recent clinical studies about nEPAP for the treatment of OSAHS.

  14. Distinct PKA and Epac compartmentalization in airway function and plasticity

    NARCIS (Netherlands)

    Dekkers, Bart G. J.; Racke, Kurt; Schmidt, Martina

    2013-01-01

    Asthma and chronic obstructive pulmonary disease (COPD) are obstructive lung diseases characterized by airway obstruction, airway inflammation and airway remodelling. Next to inflammatory cells and airway epithelial cells, airway mesenchymal cells, including airway smooth muscle cells and (myo)fibro

  15. Issues of critical airway management (Which anesthesia; which surgical airway?

    Directory of Open Access Journals (Sweden)

    Fabrizio Giuseppe Bonanno

    2012-01-01

    Full Text Available Which anesthesia for patients with critical airway? Safe and effective analgesia and anesthesia in critical airway is a skilled task especially after severe maxillofacial injury combined with head injury and hemorrhagic shock. If on one side sedation is wanted, on the other hand it may worsen the airway and hemodynamic situation to a point where hypoventilation and decrease of blood pressure, common side-effect of many opioids, may prejudice the patient′s level of consciousness and hemodynamic compensation, compounding an already critical situation. What to do when endotracheal intubation fails and blood is trickling down the airways in an unconscious patient or when a conscious patient has to sit up to breathe? Which surgical airway in critical airway? Comparative studies among the various methods of emergency surgical airway would be unethical; furthermore, operator′s training and experience is relevant for indications and performance.

  16. Issues of critical airway management (Which anesthesia; which surgical airway?).

    Science.gov (United States)

    Bonanno, Fabrizio Giuseppe

    2012-10-01

    Which anesthesia for patients with critical airway? Safe and effective analgesia and anesthesia in critical airway is a skilled task especially after severe maxillofacial injury combined with head injury and hemorrhagic shock. If on one side sedation is wanted, on the other hand it may worsen the airway and hemodynamic situation to a point where hypoventilation and decrease of blood pressure, common side-effect of many opioids, may prejudice the patient's level of consciousness and hemodynamic compensation, compounding an already critical situation. What to do when endotracheal intubation fails and blood is trickling down the airways in an unconscious patient or when a conscious patient has to sit up to breathe? Which surgical airway in critical airway? Comparative studies among the various methods of emergency surgical airway would be unethical; furthermore, operator's training and experience is relevant for indications and performance. PMID:23248494

  17. Role of Inhaled Steroids in Vascular Airway Remodelling in Asthma and COPD

    OpenAIRE

    Alfredo Chetta; Dario Olivieri

    2012-01-01

    In chronic obstructive airway diseases, such as asthma and chronic obstructive pulmonary disease (COPD), changes in bronchial microvasculature are present in response to inflammatory stimuli. Vascular changes may significantly contribute to airway wall remodelling. Angiogenesis and vascular leakage are prevalent in asthma, while vasodilation and vascular leakage dominate in COPD. An endothelial dysfunction may be present both in asthma and in COPD. Vascular changes may occur simultaneously wi...

  18. Airway management and morbid obesity

    DEFF Research Database (Denmark)

    Kristensen, Michael S

    2010-01-01

    airway and the function of the lungs (decreased residual capacity and aggravated ventilation perfusion mismatch) worse than in lean patients. Proper planning and preparation of airway management is essential, including elevation of the patient's upper body, head and neck. Preoxygenation is mandatory...... solely on whether morbid obesity is present or not. It is important to ensure sufficient depth of anaesthesia before initiating manipulation of the airway because inadequate anaesthesia depth predisposes to aspiration if airway management becomes difficult. The intubating laryngeal mask airway is more...

  19. Methacholine-Induced Variations in Airway Volume and the Slope of the Alveolar Capnogram Are Distinctly Associated with Airflow Limitation and Airway Closure.

    Science.gov (United States)

    Plantier, Laurent; Marchand-Adam, Sylvain; Boyer, Laurent; Taillé, Camille; Delclaux, Christophe

    2015-01-01

    Mechanisms driving alteration of lung function in response to inhalation of a methacholine aerosol are incompletely understood. To explore to what extent large and small airways contribute to airflow limitation and airway closure in this context, volumetric capnography was performed before (n = 93) and after (n = 78) methacholine provocation in subjects with an intermediate clinical probability of asthma. Anatomical dead space (VDaw), reflecting large airway volume, and the slope of the alveolar capnogram (slope3), an index of ventilation heterogeneity linked to small airway dysfunction, were determined. At baseline, VDaw was positively correlated with lung volumes, FEV1 and peak expiratory flow, while slope3 was not correlated with any lung function index. Variations in VDaw and slope3 following methacholine stimulation were correlated to a small degree (R2 = -0.20). Multivariate regression analysis identified independent associations between variation in FEV1 and variations in both VDaw (Standardized Coefficient-SC = 0.66) and Slope3 (SC = 0.35). By contrast, variation in FVC was strongly associated with variations in VDaw (SC = 0.8) but not Slope3. Thus, alterations in the geometry and/or function of large and small airways were weakly correlated and contributed distinctly to airflow limitation. While both large and small airways contributed to airflow limitation as assessed by FEV1, airway closure as assessed by FVC reduction mostly involved the large airways. PMID:26599006

  20. Methacholine-Induced Variations in Airway Volume and the Slope of the Alveolar Capnogram Are Distinctly Associated with Airflow Limitation and Airway Closure.

    Directory of Open Access Journals (Sweden)

    Laurent Plantier

    Full Text Available Mechanisms driving alteration of lung function in response to inhalation of a methacholine aerosol are incompletely understood. To explore to what extent large and small airways contribute to airflow limitation and airway closure in this context, volumetric capnography was performed before (n = 93 and after (n = 78 methacholine provocation in subjects with an intermediate clinical probability of asthma. Anatomical dead space (VDaw, reflecting large airway volume, and the slope of the alveolar capnogram (slope3, an index of ventilation heterogeneity linked to small airway dysfunction, were determined. At baseline, VDaw was positively correlated with lung volumes, FEV1 and peak expiratory flow, while slope3 was not correlated with any lung function index. Variations in VDaw and slope3 following methacholine stimulation were correlated to a small degree (R2 = -0.20. Multivariate regression analysis identified independent associations between variation in FEV1 and variations in both VDaw (Standardized Coefficient-SC = 0.66 and Slope3 (SC = 0.35. By contrast, variation in FVC was strongly associated with variations in VDaw (SC = 0.8 but not Slope3. Thus, alterations in the geometry and/or function of large and small airways were weakly correlated and contributed distinctly to airflow limitation. While both large and small airways contributed to airflow limitation as assessed by FEV1, airway closure as assessed by FVC reduction mostly involved the large airways.

  1. Risk factors for multiple organic dysfunctions syndrome in burnt children. Factores de riesgo de síndrome de disfunción orgánica múltiple en niños quemados

    Directory of Open Access Journals (Sweden)

    Jorge González Mendoza

    2009-05-01

    Full Text Available Background: The creation of intensive care units allows extending the life of patients with serious conditions, including multiple organic dysfunction syndromes. Objective: To determine the clinical variables and laboratory variables that are risk factors for multiple organic dysfunction syndromes in burnt children. Methods: Analytical, retrospective study of case series including burnt patients between 0 and 5 years hospitalized in the university Paediatric Hospital “Paquito González” in Cienfuegos and classified as: serious, very serious, critical, and extremely critical. This study was developed from January 1st, 2000 to December 31st, 2005. The considered systems for dysfunction diagnosis were: respiratory, cardiovascular, gastrointestinal, hepatic, renal, metabolic, central nervous system, hematologic, immunologic, and wounds healing. Data was processed by bivariate analysis of independent variables in relation with the dependent variable, to model a response variable of the syndrome occurrence (or not. The multivariate analysis of logistic regression was used. Results: 34 children developed the syndrome 44, 2 %. Significant variables linked to this syndrome were: seriousness of the injuries, serum potassium, blood creatinine, leukocyte counting, and cardiac rhythm. Conclusions: After five days of research development, a group of factors was identified proving risky for the development of multiple organic dysfunctions in burnt children.Fundamento: El surgimiento de los cuidados intensivos ha permitido que se prolongue la vida de pacientes antes considerados como insalvables y con ello que aparezca, en este tipo de enfermos, el síndrome de disfunción orgánica múltiple. Objetivo: Determinar las variables clínicas y de laboratorio que constituyan factores de riesgo de síndrome de disfunción orgánica múltiple en

  2. SÍNDROME DE DISFUNCIÓN COGNITIVA DEL PERRO COMO MODELO DE INVESTIGACIÓN DE LAS ENFERMEDADES NEURODEGENERATIVAS DEL HUMANO Cognitive dysfunction syndrome in dog senior: a suitable model for human neurodegenerative diseases?

    Directory of Open Access Journals (Sweden)

    Diana Y. Gallego1

    2010-01-01

    envejecimiento de los humanos.In humans and canines, the aging process increases the risk of neurodegenerative diseases such as Alzheimer’s disease and cognitive dysfunction syndrome in senior dogs. These pathological changes in different brain areas cause dementia syndromes, generating an apparent cognitive deficit characterized by behavioral changes such as alterations in memory and learning processes. The deleterious effects on quality of life in human patient affected by AD, promote the need to find similar pathologies affecting other species, making these, experimental models useful for the investigation of human suffering. Recently it has been suggested a close similarity between several of the clinical, anatomical and physiological characteristic of the Alzheimer’s disease and the cognitive dysfunction syndrome senior dogs, which includes the formation and accumulation of amyloid plaques, apoptosis of cholinergic neurons with a consequent reduction of the neurotransmitter acetyl - hill, progressive type cognitive deficits and alterations in the sleep-wake cycle, among others. Thus, progress in understanding the processes involved in the pathophysiology of cognitive dysfunction syndrome senior dogs, and recognizing their similarities to those that occurred during the Alzheimer’s disease, has facilitated studies aimed at understanding some aspects that have not been well detailed neurodegenrativas disease in humans. Moreover, considering the high probability of getting the cognitive dysfunction syndrome senior dogs, which is identified in several canine populations, allows for the possibility of proposing the dog changes, as an optimal model for research experimentate neurodegenerative processes associated with aging in humans.

  3. Supraglottic airway devices in children

    Science.gov (United States)

    Ramesh, S; Jayanthi, R

    2011-01-01

    Modern anaesthesia practice in children was made possible by the invention of the endotracheal tube (ET), which made lengthy and complex surgical procedures feasible without the disastrous complications of airway obstruction, aspiration of gastric contents or asphyxia. For decades, endotracheal intubation or bag-and-mask ventilation were the mainstays of airway management. In 1983, this changed with the invention of the laryngeal mask airway (LMA), the first supraglottic airway device that blended features of the facemask with those of the ET, providing ease of placement and hands-free maintenance along with a relatively secure airway. The invention and development of the LMA by Dr. Archie Brain has had a significant impact on the practice of anaesthesia, management of the difficult airway and cardiopulmonary resuscitation in children and neonates. This review article will be a brief about the clinical applications of supraglottic airways in children. PMID:22174464

  4. 蜂蜇伤致多器官功能障碍综合征一例并文献复习%Treatment of multiple organs dysfunction syndrome from bee stings:a case report and literature review

    Institute of Scientific and Technical Information of China (English)

    段享建

    2014-01-01

    Objective To summer the treatment experience of multiple organs dysfunction syndrome from bee stings and improve diagnosis and treatment. Methods Pathogenesis, clinical manifestations, treatment options, and the reasons cause death could be found by analyzing a case report of multiple organs dysfunction syndrome from bee stings and reviewing the literatures. Results Bee venom could cause serious consequences anaphylactic shock, renal failure or multiple organ failure and so on. Mortality rate was related to the failure organs. The treatment included topical treatment, systemic medication, blood purification treatment such as integrated. Conclusion Continuous renal replacement therapy(CPRT)combining with Hemoperfusion (HP)is the first choice to treat multiple organs dysfunction syndrome from bee stings in the early time.%目的:总结蜂蜇伤致多器官功能障碍综合征(MODS)的治疗经验,提高蜂蜇伤的诊断和治疗水平。方法回顾性分析蜂蜇伤致MODS患者1例,总结救治经验并复习相关文献,探讨蜂蜇伤的发病机制、临床表现、治疗方案、疗效及死因等。结果蜂蜇伤以蜂毒致病,可致过敏性休克、肾衰竭或多器官功能衰竭等严重后果,病死率与衰竭器官数目有关。其治疗包括局部处理、全身用药、血液净化等综合性治疗。结论在基础治疗的早期连续性肾脏替代治疗联合血液灌流(CRRT+HP)是蜂蜇伤致MODS有效的治疗手段。

  5. Mast Cell Tryptase Reduces Junctional Adhesion Molecule-A (JAM-A) Expression in Intestinal Epithelial Cells: Implications for the Mechanisms of Barrier Dysfunction in Irritable Bowel Syndrome.

    LENUS (Irish Health Repository)

    Wilcz-Villega, Ewa M

    2013-07-01

    The objective of this study was to investigate how mast cell tryptase may influence intestinal permeability and tight junction (TJ) proteins in vitro and explore translation to irritable bowel syndrome (IBS).

  6. The clinical analysis of central neurological dysfunctions in primary Sjogren's syndrome: 4 cases report%原发性干燥综合征中枢神经系统损害4例分析

    Institute of Scientific and Technical Information of China (English)

    姚晓萍; 李智文; 林仕芳

    2012-01-01

    目的 探讨原发性干燥综合征中枢神经系统损害的临床特点,以提高其早期诊断、治疗效果.方法 回顾性分析4例原发性干燥综合征中枢神经系统损害患者的临床特征、诊疗情况,并结合国内外相关文献进行分析.结果 4例患者均为女性,年龄30~52岁.3例患者中枢神经系统损害表现先于干燥综合征诊断,1例患者先诊断干燥综合征后出现中枢神经系统损害.1例患者出现脊髓损害表现,2例患者为脑实质损害表现,1例具有脊髓、脑、视神经损害表现.4例患者MR均表现为点状或小片状长T1长T2信号影.治疗上1例患者单用糖皮质激素,其余3例患者均合用免疫抑制剂,治疗后病情均好转,随访无再新发中枢神经系统损害表现.结论 对不能用其他原因解释的中枢神经系统损害,尤其是中老年女性,应警惕原发性干燥综合征的可能.重视干燥综合征症状的询问,加深对干燥综合征中枢神经系统损害临床表现的认识,进行相关检查,以提高早期诊断、治疗效果,减少患者的致残率.%Objective To explore the clinical features of central neurological dysfunctions in primary Sjogren' s syndrome , and to improve its early diagnosis and treatment. Methods Retrospective review the clinical features, diagnosis and treatment in four patients of central neurological dysfunctions in primary Sjogren s syndrome, and analyze the relative literatures. Results The four patients were female, aged from 30 years old to 52 years old. Three patients of them presented the manifestation of central nervous system damage ahead of the diagnosis of Sjogren' s syndrome. One patient with spinal cord injury , two patients with brain parenchymal damage, and one patient with spinal cord injury, brain parenchyma and optic nerve damage. All of the patients showed punctate or small pieces of long Tl, T2 signal intensity in the MR examination. In terms of treatment, one patient was treated

  7. Clinical analysis of systemic inflammatory response syndrome and multiple organ dysfunction syndrome after severe trauma%重度创伤后全身炎性反应综合征和多器官功能障碍综合征的临床分析

    Institute of Scientific and Technical Information of China (English)

    刀敏; 高静波; 赵弼武

    2015-01-01

    目的:提高对重度创伤后全身炎性反应综合征(SIRS)和多器官功能障碍综合征(MODS)的认识。方法:2011年5月-2014年5月收治SIRS患者300例,其中损伤严重度评分>16分MODS患者120例,对其临床资料进行回顾性分析。结果:300例患者中符合SIRS 2项标准110例(36.7%),符合3项标准132例,符合4项标准58例;MODS并发2个器官功能障碍44例,3个器官功能障碍35例,4个功能障碍30例,≥5个功能障碍11例;患者符合SIRS标准的项目数愈多,器官功能障碍越多,其病死率越高(P<0.05),总体死亡率80.0%;MODS患者器官功能障碍发生频度及病死率由高到低分别为呼吸系统、循环系统、中枢神经系统、代谢系统、血液系统、肾脏、胃肠道、肝脏。结论:重度创伤后的休克、感染以及免疫功能障碍均可引发SIRS,病情严重时可导致MODS,代偿性抗炎反应综合征和混合型对抗反应综合征可有效维持机体的内环境平衡。%Objective:To improve the understanding of the systemic inflammatory response syndrome(SIRS) and multiple organ dysfunction syndrome(MODS) after severe trauma.Methods:300 patients with SIRS were selected from May 2011 to May 2014.The injury severity score of 120 MODS patients>16.We retrospectively analyzed the clinical datas of them.Results:In 300 patients, 110 cases were complianced with SIRS 2 standards(36.7%),meet the 3 criteria in 132 cases,accord with 4 criteria in 58 cases;MODS concurrent 2 organs dysfunction in 44 cases,35 cases of 3 organs dysfunction,30 cases of dysfunction in 4,more than 5 dysfunction in 11 cases;patients who met the SIRS criteria for the project number,the more the organ dysfunction,its mortality was higher(P<0.05),the overall mortality rate was 80%;the frequency of occurrence and mortality rate of organ dysfunction in MODS patients from high to low were respiratory system,circulatory system,nervous system,metabolic system

  8. Maxillomandibular Advancement Surgery as Alternative to Continuous Positive Airway Pressure in Morbidly Severe Obstructive Sleep Apnea : A Case Report

    NARCIS (Netherlands)

    Doff, Michiel H. J.; Jansma, Johan; Schepers, Rutger H.; Hoekema, Aamoud

    2013-01-01

    Obstructive sleep apnea syndrome (OSAS) is a sleep-related breathing disorder, characterized by disrupted snoring and repetitive upper airway obstructions. Oral appliance therapy is an effective alternative to continuous positive airway pressure (CPAP) and is especially effective in mild and moderat

  9. 糖尿病多器官功能失常综合征的诊断与治疗%Diagnosis and therapy of polyorganic dysfunction syndrome caused by diabetes meuitus

    Institute of Scientific and Technical Information of China (English)

    阎德文; 金之欣

    1999-01-01

    @@ 多器官功能失常综合征(multiple organ dysfunction syndrome,MODS)是机体遭受严重感染、严重创伤(包括重型胰腺炎、休克)和病理产科等损害24小时后,同时或序贯性地出现2个或2个以上器官功能失常、以至衰竭的临床综合征.其发病机制还不十分明确,可能是由失控的全身炎症反应和组织氧代谢障碍引起[1].

  10. Airways Disease: Phenotyping Heterogeneity Using Measures of Airway Inflammation

    OpenAIRE

    Siddiqui Salman; Brightling Christopher E

    2007-01-01

    Despite asthma and chronic obstructive pulmonary disease being widely regarded as heterogeneous diseases, a consensus for an accurate system of classification has not been agreed. Recent studies have suggested that the recognition of subphenotypes of airway disease based on the pattern of airway inflammation may be particularly useful in increasing our understanding of the disease. The use of non-invasive markers of airway inflammation has suggested the presence of four distinct phenotypes: ...

  11. Issues of critical airway management (Which anesthesia; which surgical airway?)

    OpenAIRE

    Fabrizio Giuseppe Bonanno

    2012-01-01

    Which anesthesia for patients with critical airway? Safe and effective analgesia and anesthesia in critical airway is a skilled task especially after severe maxillofacial injury combined with head injury and hemorrhagic shock. If on one side sedation is wanted, on the other hand it may worsen the airway and hemodynamic situation to a point where hypoventilation and decrease of blood pressure, common side-effect of many opioids, may prejudice the patient′s level of consciousness and hemodynami...

  12. Biomarkers in Airway Diseases

    Directory of Open Access Journals (Sweden)

    Janice M Leung

    2013-01-01

    Full Text Available The inherent limitations of spirometry and clinical history have prompted clinicians and scientists to search for surrogate markers of airway diseases. Although few biomarkers have been widely accepted into the clinical armamentarium, the authors explore three sources of biomarkers that have shown promise as indicators of disease severity and treatment response. In asthma, exhaled nitric oxide measurements can predict steroid responsiveness and sputum eosinophil counts have been used to titrate anti-inflammatory therapies. In chronic obstructive pulmonary disease, inflammatory plasma biomarkers, such as fibrinogen, club cell secretory protein-16 and surfactant protein D, can denote greater severity and predict the risk of exacerbations. While the multitude of disease phenotypes in respiratory medicine make biomarker development especially challenging, these three may soon play key roles in the diagnosis and management of airway diseases.

  13. Managing upper airway obstruction.

    Science.gov (United States)

    Innes, M H

    A complete respiratory obstruction can lead to death in 3 minutes. The first and constant duty of the nurse aider is to check that the person is breathing by looking, listening and feeling. Partial obstruction is no less serious than complete obstruction. The nurse aider, in any situation, should assess the problem and attempt to overcome the airway obstruction using the measures described. PMID:1490067

  14. Hemodynamics of Acute Right Heart Failure in Mechanically Ventilated Patients with Acute Respiratory Distress Syndrome.

    Science.gov (United States)

    McLean, Barbara

    2015-12-01

    In critically ill patients with circulatory shock, the role of the left ventricle has long been appreciated and the object of measurement and therapeutic targeting. The right ventricle is often under appreciated and dysfunction may be overlooked. Generally, the right ventricle operates passively to support the ejection of the left ventricular diastolic volume. A loss of right ventricular wall compliance secondary to pulmonary pressures may result in an alteration in the normal pressure-volume relationship, ultimately affecting the stroke volume and cardiac output. Traditional right heart filling indices may increase because of decreasing compliance, further complicating the picture. The pathophysiology of pulmonary vascular dysfunction in acute respiratory distress syndrome combined with the effects of a mean airway pressure strategy may create an acute cor pulmonale. PMID:26567491

  15. 双水平呼吸道正压通气治疗重叠综合征的临床效果观察%Clinical observation of Bi-level positive airway pressure ventilation therapy treating overlap syndrome

    Institute of Scientific and Technical Information of China (English)

    刘晓丽; 刘芳勋; 张华; 刘芳; 牛志红; 黄秀清; 蒋延文

    2016-01-01

    Objective To discuss the therapeutic effect of Bi-Level positive airway pressure (Bi-PAP) ventilation therapy treating overlap syndrome.Methods Fifty patients with chronic obstructive pulmonary disease (COPD) and obstructive sleep apnea hypopnea syndrome(OSAHS) from August 2013 to December 2014 in Beijing Shijitan Hospital,Capital Medical University were collected;according to the random number table method,all patients were divided into observation group and control group(25 cases in each group).The observation group was treated by Bi-PAP for 72 h and the control group was treated by continuous positive airway pressure (CPAP) for 72 h.pH value,arterial partial pressure of oxygen (PaO2),arterial partial pressure of carbon dioxide (PaCO2),arterial oxygen saturation (SaO2),serum C reactive protein (CRP) and tumor necrosis factor-α (TNF-α) levels before and after treatment in two groups were compared.After treatment,the apnea hypopnea index (AHI),the longest apnea duration,forced expiratory volume in one second (FEV1) and forced vital capacity(FVC) in two groups were compared.Results The pH value,PaO2,PaCO2,SaO2 had no statistical difference in two groups before treatment(P >0.05).After treatment,the pH value,PaO2 and SaO2 were significantly higher in observation group than those in control group;PaCO2 was significantly lower[(7.41 ±0.05) vs (7.31 ±0.04),(90 ±5)mmHg vs (76 ± 7) mmHg,(98.3 ± 1.2) % vs (95.7 ± 1.6) %,(45 ± 6) mmHg vs (57 ± 8) mmHg,P < 0.05].Serum CRP and TNF-α levels did not show significant difference before treatment in 2 groups(P >0.05);after treatment,those in the observation group were significantly lower than those of the control group[(4.9 ± 1.2) mg/L vs (7.6 ±1.1) mg/L,(44 ± 8) ng/L vs (53 ± 6) ng/L,P < 0.05].Mter treatment,the AHI and the longest apnea duration in observation group were significantly lower than those of the control group[(7.2 ±2.9) vs (15.6 ±3.2),(10 ±4)s vs (16 ±5)s,P <0.05].After treatment

  16. Eosinophilic airway inflammation in COPD

    OpenAIRE

    Saha, Shironjit; Brightling, Christopher E.

    2006-01-01

    Chronic obstructive pulmonary disease is a common condition and a major cause of mortality. COPD is characterized by irreversible airflow obstruction. The physiological abnormalities observed in COPD are due to a combination of emphysema and obliteration of the small airways in association with airway inflammation. The predominant cells involved in this inflammatory response are CD8+ lymphocytes, neutrophils, and macrophages. Although eosinophilic airway inflammation is usually considered a f...

  17. Anticholinergic treatment in airways diseases.

    LENUS (Irish Health Repository)

    Flynn, Robert A

    2009-10-01

    The prevalence of chronic airways diseases such as chronic obstructive pulmonary disease and asthma is increasing. They lead to symptoms such as a cough and shortness of breath, partially through bronchoconstriction. Inhaled anticholinergics are one of a number of treatments designed to treat bronchoconstriction in airways disease. Both short-acting and long-acting agents are now available and this review highlights their efficacy and adverse event profile in chronic airways diseases.

  18. Pharmacology of airway smooth muscle proliferation

    NARCIS (Netherlands)

    Gosens, Reinoud; Roscioni, Sara S.; Dekkers, Bart G. J.; Pera, Tonio; Schmidt, Martina; Schaafsma, Dedmer; Zaagsma, Johan; Meurs, Herman

    2008-01-01

    Airway smooth muscle thickening is a pathological feature that contributes significantly to airflow limitation and airway hyperresponsiveness in asthma. Ongoing research efforts aimed at identifying the mechanisms responsible for the increased airway smooth muscle mass have indicated that hyperplasi

  19. Predictors of Airway Hyperresponsiveness in Elite Athletes

    DEFF Research Database (Denmark)

    Toennesen, Louise L; Porsbjerg, Celeste; Pedersen, Lars;

    2015-01-01

    INTRODUCTION: Elite athletes frequently experience asthma and airway hyperresponsiveness (AHR). We aimed to investigate predictors of airway pathophysiology in a group of unselected elite summer-sport athletes, training for the summer 2008 Olympic Games, including markers of airway inflammation...

  20. Cholinergic regulation of airway inflammation and remodelling

    NARCIS (Netherlands)

    Kolahian, Saeed; Gosens, Reinoud

    2012-01-01

    Acetylcholine is the predominant parasympathetic neurotransmitter in the airways that regulates bronchoconstriction and mucus secretion. Recent findings suggest that acetylcholine regulates additional functions in the airways, including inflammation and remodelling during inflammatory airway disease

  1. Safe performance of spinal anesthesia in a critical patient with neurofibromatosis, pectus carinatum, and temporomandibular joint dysfunction: A case report

    Directory of Open Access Journals (Sweden)

    Zencirci Beyazit

    2010-05-01

    Full Text Available Abstract Background Neurofibromatosis is a syndrome caused by the abnormal deposition of neural tissues of the nervous system, endocrine system, visceral structures, and skin. On the other hand, pectus carinatum and temporomandibular joint dysfunction are illnesses that adversly affect the respiratory system and cause additional problems in airway management. Case Presentation Fifty-eight-year-old Turkish male patient had neurofibromatosis, pectus carinatum and temporomandibular joint dysfunction. The case was due to be operated on with the diagnosis of incarcerated umbilical hernia. Spinal anesthesia was successfully performed and the duration of the surgery was 1 hour. No postoperative complications were observed and he was discharged from the hospital on the 3rd post-operative day. Conclusion The anesthetic management of patients with neurofibromatosis requires attention to all possible abnormalities and associated disturbances. Furthermore, the presence of pectus carinatum and temporomandibular joint dysfunction also increase the potential risks. The operation was successfully completed with spinal anesthesia that was carefully applied upon taking the required measures and considering all pathologies that may accompany the case and complications that may occur.

  2. High-resolution CT findings of idiopathic pneumonia syndrome after haematopoietic stem cell transplantation: based on the updated concept of idiopathic pneumonia syndrome by the American Thoracic Society in 2011.

    Science.gov (United States)

    Tanaka, N; Kunihiro, Y; Kobayashi, T; Yujiri, T; Kido, S; Ueda, K; Matsunaga, N

    2016-10-01

    Idiopathic pneumonia syndrome (IPS) is an acute lung dysfunction of non-infectious aetiology and a severe complication following haematopoietic stem cell transplantation (HSCT). Recently, the American Thoracic Society (ATS) updated the concept of IPS and extended the concept to a wider range; it defined IPS as "an idiopathic syndrome of pneumopathy after HSCT, with evidence of widespread alveolar injury and in which infectious aetiologies and cardiac dysfunction, acute renal failure, or iatrogenic fluid overload have been excluded." The ATS also categorised the presumed site of primary tissue injury into three patterns (pulmonary parenchyma, vascular endothelium, and airway epithelium), each of which has several entities. Since the therapeutic strategies for IPS are clearly different from those of infectious diseases, and therapeutic delay causes a poor prognosis, radiologists should be aware of some characteristic HRCT findings of IPS, which includes a wide spectrum of entities. In this article, the characteristic HRCT findings of these entities, including acute interstitial pneumonia/acute respiratory distress syndrome, eosinophilic pneumonia, non-cardiogenic capillary leak syndrome, diffuse alveolar haemorrhage, transfusion-related acute lung injury, organising pneumonia, and bronchiolitis obliterans syndrome, are shown. PMID:27421574

  3. O uso da neuromodulação no tratamento das disfunções de eliminações Neuromodulation in the treatment of eliminations dysfunction syndrome

    Directory of Open Access Journals (Sweden)

    Lisieux Eyer de Jesus

    2007-12-01

    Full Text Available OBJETIVO: Apresentar a experiência clínica com o uso de neuromodulação através de eletroestimulação transcutânea no tratamento das disfunções de eliminação. MÉTODOS: Foram analisados retrospectivamente seis pacientes com disfunções de eliminação submetidos à neuromodulação através de eletroestimulação percutânea peri-sacral. Todos os pacientes incluídos eram refratários ao tratamento clínico convencional e a eletroestimulação foi utilizada como terapia de resgate. As sessões terapêuticas eram semanais e o seguimento mínimo foi de seis meses. RESULTADOS: Todos os pacientes obtiveram melhora clínica. Quatro pacientes não necessitaram de mais medicação. Todos aceitaram bem o tratamento e referiram melhora da qualidade de vida. CONCLUSÃO: A eletroestimulação percutânea mostrou-se útil no tratamento das disfunções do assoalho pélvico e da musculatura vesical.OBJECTIVE: To present a clinical trial with neuromodulation through percutaneous electrical stimulation in the treatment of elimination dysfunctions. METHODS: Six patients with dysfunctional elimination diagnosis were included and their data were analyzed retrospectively, regarding percutaneous sacral electrical stimulation. All the patients included, have had unsuccessful conventional treatments and the electrical stimulation was used as rescue therapy. All the sessions were applied in weekly fashion for six months. RESULTS: All the patients have had a positive clinical outcome, with four of them interrupting their medication. All the patients accepted well the therapy and referred an improvement in their quality of life. CONCLUSION: The electrical percutaneous stimulation, showed to be effective on vesical muscle and pelvic floor dysfunctions.

  4. Airways Disease: Phenotyping Heterogeneity Using Measures of Airway Inflammation

    Directory of Open Access Journals (Sweden)

    Siddiqui Salman

    2007-06-01

    Full Text Available Despite asthma and chronic obstructive pulmonary disease being widely regarded as heterogeneous diseases, a consensus for an accurate system of classification has not been agreed. Recent studies have suggested that the recognition of subphenotypes of airway disease based on the pattern of airway inflammation may be particularly useful in increasing our understanding of the disease. The use of non-invasive markers of airway inflammation has suggested the presence of four distinct phenotypes: eosinophilic, neutrophilic, mixed inflammatory and paucigranulocytic asthma. Recent studies suggest that these subgroups may differ in their etiology, immunopathology and response to treatment. Importantly, novel treatment approaches targeted at specific patterns of airway inflammation are emerging, making an appreciation of subphenotypes particularly relevant. New developments in phenotyping inflammation and other facets of airway disease mean that we are entering an era where careful phenotyping will lead to targeted therapy.

  5. Airway emergencies in cancer

    Directory of Open Access Journals (Sweden)

    Patil Vijaya

    2007-01-01

    Full Text Available Management of airway obstruction is always challenging but more so in cancer setting, as obstruction can lie at any level right from pyriform fossa to low down in medistinum. Morbidity is significant but if not managed properly leads to frightful death by suffocation. These cases need to be evaluated, diagnosed and managed with care, skill, speed and appropriate intervention. With the advent of technology, it has become much easier to manage such situations with a team of specialists involving anesthetist, thoracic surgeon and intensivist.

  6. From "bacterial-toxin treated simutaneously" to "four syndromes and four methods"——improvement and integration of the syndrome-differentating thinking for treating multiple organ dysfunction syndrome with integrated traditional Chinese and western medicine%从"菌毒并治"到"四证四法"——关于中西医结合治疗多器官功能障碍综合征辨证思路的深入与完善

    Institute of Scientific and Technical Information of China (English)

    曹书华; 王今达; 李银平

    2005-01-01

    @@ 多器官功能障碍综合征(multiple organ dysfunction syndrome,MODS)发病急,病情进展迅速,病死率极高,是危重病医学领域内的尖端课题.天津市急救医学研究所自20世纪70年代起就开始了以中西医结合方法治疗MODS的探索.

  7. Paediatric airway management: basic aspects

    DEFF Research Database (Denmark)

    Holm-Knudsen, R J; Rasmussen, L S

    2009-01-01

    children. This paper aims at providing the non-paediatric anaesthesiologist with a set of safe and simple principles for basic paediatric airway management. In contrast to adults, most children with difficult airways are recognised before induction of anaesthesia but problems may arise in all children...

  8. Prenatal MRI Findings of Fetuses with Congenital High Airway Obstruction Sequence

    Energy Technology Data Exchange (ETDEWEB)

    Guimaraes, Carolina V. A.; Linam, Leann E.; Kline-Fath, Beth M. [Cincinnati Children' s Hospital Medical Center, Cincinnati (United States)] (and others)

    2009-04-15

    To define the MRI findings of congenital high airway obstruction sequence (CHAOS) in a series of fetuses. Prenatal fetal MR images were reviewed in seven fetuses with CHAOS at 21 to 27 weeks of gestation. The MRI findings were reviewed. The MRI parameters evaluated included the appearance of the lungs and diaphragm, presence or absence of hydrops, amount of amniotic fluid, airway appearance, predicted level of airway obstruction, and any additional findings or suspected genetic syndromes. All the fetuses viewed (7 of 7) demonstrated the following MRI findings: dilated airway below the level of obstruction, increased lung signal, markedly increased lung volumes with flattened or inverted hemidiaphragms, massive ascites, centrally positioned and compressed heart, as well as placentomegaly. Other frequent findings were anasarca (6 of 7) and polyhydramnios (3 of 7). MRI identified the level of obstruction as laryngeal in five cases and tracheal in two cases. In four of the patients, surgery or autopsy confirmed the MRI predicted level of obstruction. Associated abnormalities were found in 4 of 7 (genetic syndromes in 2). Postnatal radiography (n = 3) showed markedly hyperinflated lungs with inverted or flattened hemidiaphragms, strandy perihilar opacities, pneumothoraces and tracheotomy. Two fetuses were terminated and one fetus demised in utero. Four fetuses were delivered via ex utero intrapartum treatment procedure. MRI shows a consistent pattern of abnormalities in fetuses with CHAOS, accurately identifies the level of airway obstruction, and helps differentiate from other lung abnormalities such as bilateral congenital pulmonary airway malformation by demonstrating an abnormally dilated airway distal to the obstruction.

  9. Prenatal MRI Findings of Fetuses with Congenital High Airway Obstruction Sequence

    International Nuclear Information System (INIS)

    To define the MRI findings of congenital high airway obstruction sequence (CHAOS) in a series of fetuses. Prenatal fetal MR images were reviewed in seven fetuses with CHAOS at 21 to 27 weeks of gestation. The MRI findings were reviewed. The MRI parameters evaluated included the appearance of the lungs and diaphragm, presence or absence of hydrops, amount of amniotic fluid, airway appearance, predicted level of airway obstruction, and any additional findings or suspected genetic syndromes. All the fetuses viewed (7 of 7) demonstrated the following MRI findings: dilated airway below the level of obstruction, increased lung signal, markedly increased lung volumes with flattened or inverted hemidiaphragms, massive ascites, centrally positioned and compressed heart, as well as placentomegaly. Other frequent findings were anasarca (6 of 7) and polyhydramnios (3 of 7). MRI identified the level of obstruction as laryngeal in five cases and tracheal in two cases. In four of the patients, surgery or autopsy confirmed the MRI predicted level of obstruction. Associated abnormalities were found in 4 of 7 (genetic syndromes in 2). Postnatal radiography (n = 3) showed markedly hyperinflated lungs with inverted or flattened hemidiaphragms, strandy perihilar opacities, pneumothoraces and tracheotomy. Two fetuses were terminated and one fetus demised in utero. Four fetuses were delivered via ex utero intrapartum treatment procedure. MRI shows a consistent pattern of abnormalities in fetuses with CHAOS, accurately identifies the level of airway obstruction, and helps differentiate from other lung abnormalities such as bilateral congenital pulmonary airway malformation by demonstrating an abnormally dilated airway distal to the obstruction

  10. Sildenafil versus continuous positive airway pressure for erectile dysfunction in men with obstructive sleep apnea: a comparative study of their efficacy and safety and the patient's satisfaction with treatment%比较西地那非与连续气道正压通气治疗阻塞性睡眠呼吸暂停患者的勃起功能障碍:疗效、安全性和患者满意度

    Institute of Scientific and Technical Information of China (English)

    Petros Perimenis; Kyriakos Karkoulias; Angelis Konstantinopoulos; Paraskevi P. Perimeni; George Katsenis; Anastasios Athanasopoulos; Konstantinos Spyropoulos

    2007-01-01

    Aim: To assess the efficacy of sildenafil and continuous positive airway pressure (CPAP) in the treatment of concurrent erectile dysfunction (ED) with obstructive sleep apnea (OSA), and to gauge the level of treatment satisfaction in patients and their partners. Methods: Forty men were treated for 12 weeks with sildenafil 100 mg (20 men) or CPAP during nighttime sleep (20 men). Treatment efficacy was assessed by the rate of successful intercourse attempts,and satisfaction with treatment was assessed by patients' and partners' answers to question 1 of the Erectile Dysfunction Inventory of Treatment Satisfaction. Results: Under sildenafil, 128 of 249 (51.4%) intercourse attempts were successful; under CPAP, 51 of 193 (26.9%) attempts were successful (Cp < 0.001). Erectile function was improved in both groups. After sildenafil and CPAP treatment, the mean International Index for Erectile Function domain scores were 14.3 and 10.8, respectively (Bp = 0.025), compared to 7.8 and 7 at baseline, respectively. CPAP and sildenafil were well tolerated. Sporadic episodes of nasal dryness under CPAP and transient headache and flushing under sildenafil were not significant. Fifty percent of patients treated with sildenafil and 25% with CPAP were satisfied with the treatment, and their partners were equally satisfied. The satisfaction scores for both patients and partners under sildenafil were superior to those under CPAP (Cp < 0.002). Conclusion: Both sildenafil 100 mg and CPAP, used separately, had positive therapeutic impact but sildenafil was superior. Patients and their partners were more satisfied with sildenafil for the treatment of ED. However, because of the high proportion of dissatisfied men and partners, new therapeutic agents or a combination of the two methods must be studied further.

  11. Síndrome pulmonar por hantavírus com disfunção de múltiplos órgãos: relato de caso Hantavirus pulmonary syndrome with multiple organ dysfunctions: case report

    Directory of Open Access Journals (Sweden)

    Marcelo Spegiorin Moreno

    2007-12-01

    rates. The aim of this study is to report a case of HPS with multiple organ failure, managed with early goal-directed therapy guided by flow and tissue perfusion parameters. CASE REPORT: A 36 year-old male had fever with progressive dispnea, severe hypoxia and acute respiratory failure. Diffuse interstitial alveolar infiltrates were seen in the chest X-Ray. He developed multiple organ dysfunction syndromes (pulmonary, renal, coagulation, cardiovascular and metabolic. Treatment and invasive hemodynamic monitoring with pulmonary artery catheter was early instituted. The most important laboratory findings were thrombocytopenia, elevated hematocrit and hemoglobin concentrations, elevated liver enzymes, elevated lactate dehydrogenase and a positive sorology for Hantavirus (ELISA IgM positive. Organ dysfunctions reverted to normal and he was discharged after 21 days in hospital. CONCLUSIONS: An early and adequate resuscitation with goal-directed therapy enabled the reversion of the multiple organ failure syndromes and a favorable outcome, despite the severity of the disease.

  12. Fetal tracheolaryngeal airway obstruction: prenatal evaluation by sonography and MRI

    OpenAIRE

    Courtier, Jesse; Poder, Liina; Wang, Zhen J.; Westphalen, Antonio C.; Yeh, Benjamin M.; Coakley, Fergus V.

    2010-01-01

    We reviewed the sonographic and MRI findings of tracheolaryngeal obstruction in the fetus. Conditions that can cause tracheolaryngeal obstruction include extrinsic causes such as lymphatic malformation, cervical teratoma and vascular rings and intrinsic causes such as congenital high airway obstruction syndrome (CHAOS). Accurate distinction of these conditions by sonography or MRI can help facilitate parental counseling and management, including the decision to utilize the ex utero intrapartu...

  13. Fetal tracheolaryngeal airway obstruction: prenatal evaluation by sonography and MRI

    Energy Technology Data Exchange (ETDEWEB)

    Courtier, Jesse; Poder, Liina; Wang, Zhen J.; Westphalen, Antonio C.; Yeh, Benjamin M.; Coakley, Fergus V. [University of California San Francisco, Department of Radiology, San Francisco, CA (United States)

    2010-11-15

    We reviewed the sonographic and MRI findings of tracheolaryngeal obstruction in the fetus. Conditions that can cause tracheolaryngeal obstruction include extrinsic causes such as lymphatic malformation, cervical teratoma and vascular rings and intrinsic causes such as congenital high airway obstruction syndrome (CHAOS). Accurate distinction of these conditions by sonography or MRI can help facilitate parental counseling and management, including the decision to utilize the ex utero intrapartum treatment (EXIT) procedure. (orig.)

  14. Resuscitation and auto resuscitation by airway reflexes in animals

    OpenAIRE

    Tomori, Zoltan; Donic, Viliam; Benacka, Roman; Jakus, Jan; Gresova, Sona

    2013-01-01

    Various diseases often result in decompensation requiring resuscitation. In infants moderate hypoxia evokes a compensatory augmented breath – sigh and more severe hypoxia results in a solitary gasp. Progressive asphyxia provokes gasping respiration saving the healthy infant – autoresuscitation by gasping. A neonate with sudden infant death syndrome, however, usually will not survive. Our systematic research in animals indicated that airway reflexes have similar resuscitation potential as gasp...

  15. Advances in the Management of Meconium Aspiration Syndrome

    Directory of Open Access Journals (Sweden)

    Kamala Swarnam

    2012-01-01

    Full Text Available Meconium aspiration syndrome (MAS is a common cause of severe respiratory distress in term infants, with an associated highly variable morbidity and mortality. MAS results from aspiration of meconium during intrauterine gasping or during the first few breaths. The pathophysiology of MAS is multifactorial and includes acute airway obstruction, surfactant dysfunction or inactivation, chemical pneumonitis with release of vasoconstrictive and inflammatory mediators, and persistent pulmonary hypertension of newborn (PPHN. This disorder can be life threatening, often complicated by respiratory failure, pulmonary air leaks, and PPHN. Approaches to the prevention of MAS have changed over time with collaboration between obstetricians and pediatricians forming the foundations for care. The use of surfactant and inhaled nitric oxide (iNO has led to the decreased mortality and the need for extracorporeal membrane oxygenation (ECMO use. In this paper, we review the current understanding of the pathophysiology and management of MAS.

  16. Lack of awareness of erectile dysfunction in many men with risk factors for erectile dysfunction

    Directory of Open Access Journals (Sweden)

    Magee Michelle

    2010-11-01

    Full Text Available Abstract Background Men with erectile dysfunction often have concurrent medical conditions. Conversely, men with these conditions may also have underlying erectile dysfunction. The prevalence of unrecognized erectile dysfunction in men with comorbidities commonly associated with erectile dysfunction was determined in men invited to participate in a double-blind, randomized, placebo-controlled trial of sildenafil citrate. Methods Men ≥30 years old presenting with ≥1 erectile dysfunction risk factor (controlled hypertension, hypercholesterolemia, smoking, metabolic syndrome, stable coronary artery disease, diabetes, depression, lower urinary tract symptoms, obesity [body mass index ≥30 kg/m2] or waist circumference ≥40 inches, and not previously diagnosed with erectile dysfunction were evaluated. The screening question, "Do you have erectile dysfunction?," with responses of "no," "yes," and "unsure," and the Erectile Function domain of the International Index of Erectile Function (IIEF-EF were administered. Results Of 1084 men screened, 1053 answered the screening question and also had IIEF-EF scores. IIEF-EF scores indicating erectile dysfunction occurred in 71% (744/1053, of whom 54% (399/744 had moderate or severe erectile dysfunction. Of 139 answering "yes," 526 answering "unsure," and 388 answering "no," 96%, 90%, and 36%, respectively, had some degree of erectile dysfunction. The mean±SD (range number of risk factors was 2.9 ± 1.7 (3-8 in the "yes" group, 3.2 ± 1.7 (3-9 in the "unsure" group, and 2.6 ± 1.5 (2-8 in the "no" group. Conclusion Although awareness of having erectile dysfunction was low, most men with risk factors had IIEF-EF scores indicating erectile dysfunction. Erectile dysfunction should be suspected and assessed in men with risk factors, regardless of their apparent level of awareness of erectile dysfunction. Trial registration ClinicalTrials.gov Identifier NCT00343200.

  17. Dysfunction of inflammation-resolving pathways is associated with exaggerated postoperative cognitive decline in a rat model of the metabolic syndrome.

    Science.gov (United States)

    Su, Xiao; Feng, Xiaomei; Terrando, Niccolo; Yan, Yan; Chawla, Ajay; Koch, Lauren G; Britton, Steven L; Matthay, Michael A; Maze, Mervyn

    2013-02-08

    The cholinergic antiinflammatory pathway (CAP), which terminates in the spleen, attenuates postoperative cognitive decline (PCD) in rodents. Surgical patients with metabolic syndrome exhibit exaggerated and persistent PCD that is reproduced in postoperative rats selectively bred for easy fatigability and that contain all features of metabolic syndrome (low-capacity runners [LCRs]). We compared the CAP and lipoxin A(4) (LXA(4)), another inflammation-resolving pathway in LCR, with its counterpart high-capacity runner (HCR) rats. Isoflurane-anesthetized LCR and HCR rats either underwent aseptic trauma involving tibial fracture (surgery) or not (sham). At postoperative d 3 (POD3), compared with HCR, LCR rats exhibited significantly exaggerated PCD (trace fear conditioning freezing time 43% versus 57%). Separate cohorts were killed at POD3 to collect plasma for LXA4 and to isolate splenic mononuclear cells (MNCs) to analyze CAP signaling, regulatory T cells (Tregs) and M2 macrophages (M2 Mφ). Under lipopolysaccharide (LPS) stimulation, tumor necrosis factor (TNF)-α produced by splenic MNCs was 117% higher in LCR sham and 52% higher in LCR surgery compared with HCR sham and surgery rats; LPS-stimulated TNF-α production could not be inhibited by an α7 nicotinic acetylcholine receptor agonist, whereas inhibition by the β(2) adrenergic agonist, salmeterol, was significantly less (-35%) than that obtained in HCR rats. Compared to HCR, sham and surgery LCR rats had reduced β(2) adrenergic receptor-expressing T lymphocytes (59%, 44%), Tregs (47%, 54%) and M2 Mφ (45%, 39%); surgical LCR rats' hippocampal M2 Mφ was 66% reduced, and plasma LXA4 was decreased by 120%. Rats with the metabolic syndrome have ineffective inflammation-resolving mechanisms that represent plausible reasons for the exaggerated and persistent PCD.

  18. Comparison of different continuous positive airway pressure titration methods for obstructive sleep apnea hypopnea syndrome%正压通气治疗中手动及自动压力滴定模式比较

    Institute of Scientific and Technical Information of China (English)

    李京京; 叶京英; 张鹏; 亢丹; 曹鑫; 张玉焕; 丁秀; 郑莉; 李鸿光

    2014-01-01

    Objective To explore whether there were differences between the results of automatic titration and the results of manual titration for positive airway pressure treatment in patients with obstructive sleep apnea hypopnea syndrome (OSAHS) and its influencing factors,the results might provide a theoretical basis for the rational use of two pressure titration methods.Methods Sixty one patients with OSAHS were included in this study.All patients underwent a manual titration and an automatic titration within one week.The clinical informations,polysomnography data,and the results of both two titration of all patients were obtained for analysis.Results The overall apnea/hypopnea index was (63.1 ± 17.7)/h,with a range of 14.9/h to l10.4/h.The treatment pressure of manual titration was (8.4 ± 2.1)cmH2O,which was significantly lower than the treatment pressure of automatic titration,(11.5 ± 2.7) cmH2O (t =-9.797,P<0.001).After using a △P of 3 cmH2O for the cutoff value (△p was defined as the difference of automatic titration and manual titration),it was found that the pressure of automatic titration was significantly higher in patients with a △P > 3 cmH2O than in patients with a △P≤3 cmH2O,which was (13.3±2.3)cmH2O vs (10.0 ±2.0)cmH2O (t =-6.159,P<0.001).However,there were no differences for the pressure of manual titration between these two groups,which was (8.6 ± 2.4) cmH2O vs (8.3 ± 2.0) cmH2O (P > 0.05).There was no significant difference in age,body mass index,neck circumference,abdomen circumference,apnea hypopnea index,and arterial oxygen saturation between these two groups.Conclusions The treatment pressure of automatic titration is usually higher than that of manual titration.For patients with a high treatment pressure which is derived from automatic titration,a suggestion about manual titration could be given to decrease the potential treatment pressure of continuous positive airway pressure,which may be helpful in improving the

  19. Loss of Thrombomodulin in Placental Dysfunction in Preeclampsia

    NARCIS (Netherlands)

    Turner, Rosanne J; Bloemenkamp, Kitty W M; Bruijn, Jan A; Baelde, Hans J

    2016-01-01

    OBJECTIVE: Preeclampsia is a pregnancy-specific syndrome characterized by placental dysfunction and an angiogenic imbalance. Systemically, levels of thrombomodulin, an endothelium- and syncytiotrophoblast-bound protein that regulates coagulation, inflammation, apoptosis, and tissue remodeling, are i

  20. 原发性肾病综合征继发甲状腺功能异常的机制及治疗%Mechanism and treatment of thyroid dysfunction secondary to primary nephrotic syndrome

    Institute of Scientific and Technical Information of China (English)

    周维燕; 潘斌斌; 杜新

    2012-01-01

    临床上原发性肾病综合征(PNS)继发甲状腺功能异常的病例并不少见.近年来多项研究对于该现象进行了深入的探讨,提出了各种可能的病理生理机制,目前尚无统一定论.早期行甲状腺激素补充及抗氧化应激等治疗,可以帮助患者维持甲状腺功能正常,保护肾小球结构、改善内皮功能障碍、利于原发疾病的缓解等.儿童PNS患者外源摄入甲状腺激素联合依那普利还可促进其自身机体发育、骨骼生长及智力成熟.动态观察患者血清甲状腺激素水平可作为观察病情、判断疗效以及预后的敏感指标.%It's not uncommon that thyroid dysfunction is secondary to primary nephrotic syndrome.Recently a number of studies have explored this phenomenon and several possible pathophysiological mechanisms have been put forward.However,at present there is no unified conclusion.If thyroid hormone supplement and anti-oxidative stress treatment are given at early stages,patients may benefit a lot such as maintaining normal thyroid function,protecting glomerular structure,improving endothelial dysfunction and easing the primary diseases,etc.Intaking exogenous thyroid hormone as well as enalaprilat also can help children with primary nephrotic syndrome to promote their own body development,bone growth and intellectual maturity.Dynamic monitor of serum thyroid hormone level can served as a sensitive index for illness evaluation,response judgment and prognosis.

  1. Role of Inhaled Steroids in Vascular Airway Remodelling in Asthma and COPD

    Directory of Open Access Journals (Sweden)

    Alfredo Chetta

    2012-01-01

    Full Text Available In chronic obstructive airway diseases, such as asthma and chronic obstructive pulmonary disease (COPD, changes in bronchial microvasculature are present in response to inflammatory stimuli. Vascular changes may significantly contribute to airway wall remodelling. Angiogenesis and vascular leakage are prevalent in asthma, while vasodilation and vascular leakage dominate in COPD. An endothelial dysfunction may be present both in asthma and in COPD. Vascular changes may occur simultaneously with the thickening of the airway wall and the narrowing of the bronchial lumen. Consequently, pharmacological control of bronchial vascular remodelling may be crucial for symptom control in asthma and COPD. In asthmatic airways, inhaled steroids can downregulate vascular remodelling by acting on proangiogenic factors. Additionally, studies on combination therapy with long-acting β2-agonists and inhaled steroids have provided evidence of a possible synergistic action on components of vascular remodelling in asthma. In COPD, there is less experimental evidence on the effect of inhaled steroids on airway microvascular changes. Importantly, vascular endothelial growth factor (VEGF, the most specific growth factor for vascular endothelium, is crucially involved in the pathophysiology of airway vascular remodelling, both in asthma and COPD. The inhibition of VEGF and its receptor may be useful in the treatment of the vascular changes in the airway wall.

  2. Pulmonary surfactant in the airway physiology: a direct relaxing effect on the smooth muscle.

    Science.gov (United States)

    Calkovska, A; Uhliarova, B; Joskova, M; Franova, S; Kolomaznik, M; Calkovsky, V; Smolarova, S

    2015-04-01

    Beside alveoli, surface active material plays an important role in the airway physiology. In the upper airways it primarily serves in local defense. Lower airway surfactant stabilizes peripheral airways, provides the transport and defense, has barrier and anti-edematous functions, and possesses direct relaxant effect on the smooth muscle. We tested in vitro the effect of two surfactant preparations Curosurf® and Alveofact® on the precontracted smooth muscle of intra- and extra-pulmonary airways. Relaxation was more pronounced for lung tissue strip containing bronchial smooth muscle as the primary site of surfactant effect. The study does not confirm the participation of ATP-dependent potassium channels and cAMP-regulated epithelial chloride channels known as CFTR chloride channels, or nitric oxide involvement in contractile response of smooth muscle to surfactant.By controlling wall thickness and airway diameter, pulmonary surfactant is an important component of airway physiology. Thus, surfactant dysfunction may be included in pathophysiology of asthma, COPD, or other diseases with bronchial obstruction. PMID:25583659

  3. Vessel-guided Airway Tree Segmentation

    DEFF Research Database (Denmark)

    Lo, P.; Sporring, J.; Ashraf, H.;

    2010-01-01

    This paper presents a method for airway tree segmentation that uses a combination of a trained airway appearance model, vessel and airway orientation information, and region growing. We propose a voxel classification approach for the appearance model, which uses a classifier that is trained...... to differentiate between airway and non-airway voxels. This is in contrast to previous works that use either intensity alone or hand crafted models of airway appearance. We show that the appearance model can be trained with a set of easily acquired, incomplete, airway tree segmentations. A vessel orientation...

  4. Membrane Currents in Airway Smooth Muscle: Mechanisms and Therapeutic Implications

    Directory of Open Access Journals (Sweden)

    Luke J Janssen

    1997-01-01

    Full Text Available Electrophysiological and pharmacological techniques were used to characterize the membrane conductance changes underlying spasmogen-evoked depolarization in airway smooth muscle (ASM. Changes included a transient activation of chloride ion channels and prolonged suppression of potassium ion channels; both changes are triggered by release of internally sequestered calcium ion and in turn cause opening of voltage-dependent calcium channels. The resultant influx of calcium ions contributes to contraction as well as to refilling of the internal calcium ion pool. Bronchodilators, on the other hand, act in part through activation of potassium channels, with consequent closure of calcium channels. The tools used to study ion channels in ASM are described, and the investigations of the roles of ion channels in ASM physiology (autacoid-evoked depolarization and hyperpolarization and pathophysiology (airway hyperresponsiveness are summarized. Finally, how the relationship between ion channels and ASM function/dysfunction may relate to the treatment of asthma and related breathing disorders is discussed.

  5. Continuous positive airway pressure therapy: new generations.

    Science.gov (United States)

    Garvey, John F; McNicholas, Walter T

    2010-02-01

    Continuous positive airway pressure (CPAP) is the treatment of choice for obstructive sleep apnoea syndrome (OSAS). However, CPAP is not tolerated by all patients with OSAS and alternative modes of pressure delivery have been developed to overcome pressure intolerance, thereby improving patient comfort and adherence. Auto-adjustable positive airway pressure (APAP) devices may be utilised for the long-term management of OSAS and may also assist in the initial diagnosis of OSAS and titration of conventional CPAP therapy. Newer modalities such as C-Flex and A-Flex also show promise as treatment options in the future. However, the evidence supporting the use of these alternative modalities remains scant, in particular with regard to long-term cardiovascular outcomes. In addition, not all APAP devices use the same technological algorithms and data supporting individual APAP devices cannot be extrapolated to support all. Further studies are required to validate the roles of APAP, C-Flex and A-Flex. In the interim, standard CPAP therapy should continue as the mainstay of OSAS management. PMID:20308751

  6. Continuous positive airway pressure therapy: new generations.

    LENUS (Irish Health Repository)

    Garvey, John F

    2012-02-01

    Continuous positive airway pressure (CPAP) is the treatment of choice for obstructive sleep apnoea syndrome (OSAS). However, CPAP is not tolerated by all patients with OSAS and alternative modes of pressure delivery have been developed to overcome pressure intolerance, thereby improving patient comfort and adherence. Auto-adjustable positive airway pressure (APAP) devices may be utilised for the long-term management of OSAS and may also assist in the initial diagnosis of OSAS and titration of conventional CPAP therapy. Newer modalities such as C-Flex and A-Flex also show promise as treatment options in the future. However, the evidence supporting the use of these alternative modalities remains scant, in particular with regard to long-term cardiovascular outcomes. In addition, not all APAP devices use the same technological algorithms and data supporting individual APAP devices cannot be extrapolated to support all. Further studies are required to validate the roles of APAP, C-Flex and A-Flex. In the interim, standard CPAP therapy should continue as the mainstay of OSAS management.

  7. Continuous positive airway pressure therapy: new generations.

    LENUS (Irish Health Repository)

    Garvey, John F

    2010-02-01

    Continuous positive airway pressure (CPAP) is the treatment of choice for obstructive sleep apnoea syndrome (OSAS). However, CPAP is not tolerated by all patients with OSAS and alternative modes of pressure delivery have been developed to overcome pressure intolerance, thereby improving patient comfort and adherence. Auto-adjustable positive airway pressure (APAP) devices may be utilised for the long-term management of OSAS and may also assist in the initial diagnosis of OSAS and titration of conventional CPAP therapy. Newer modalities such as C-Flex and A-Flex also show promise as treatment options in the future. However, the evidence supporting the use of these alternative modalities remains scant, in particular with regard to long-term cardiovascular outcomes. In addition, not all APAP devices use the same technological algorithms and data supporting individual APAP devices cannot be extrapolated to support all. Further studies are required to validate the roles of APAP, C-Flex and A-Flex. In the interim, standard CPAP therapy should continue as the mainstay of OSAS management.

  8. Otologic Problems in Turner Syndrome

    OpenAIRE

    Ahmadreza Okhovat; Dr. Mahin Hashemipour; Dr. Alireza Majlesi; Mehdi Salek; Masoumeh Raufi; Dr. Saied Hanif Okhovat

    2011-01-01

    Background and Aim: Turner syndrome is the most common sex chromosome abnormality in females, affecting an estimated 3% of all conceiving females. Otologic disease is a common problem in Turner syndrome patients that is due to a combination of small dysfunction Eustachian tube, palatal dysfunction and cochlear malformation.Methods: This study assessed the otologic and audiologic characteristics of a group of Turner syndrome patients. We studied 40 Turner patients aged 10 to 20 years (mean age...

  9. [Pharmacologic treatment of Asperger syndrome].

    Science.gov (United States)

    Yamada, Satoru

    2007-03-01

    Asperger syndrome is associated with various dysfunctional and problematic behaviors, in addition to the core features of communication and social skills dysfunction that define these conditions. Although there is currently no pharmacologic cure for the core features of Asperger syndrome. This article discusses the various medications for the behavioral symptoms of Asperger syndrome, which include hyperactivity, aggression, tantrums, self-injury, depression, obsession and so on. Methylphenidate, SSRIs, atypical antipsychotics and mood stabilizer were introduced.

  10. Multiscale Vessel-guided Airway Tree Segmentation

    DEFF Research Database (Denmark)

    Lo, Pechin Chien Pau; Sporring, Jon; de Bruijne, Marleen

    2009-01-01

    This paper presents a method for airway tree segmentation that uses a combination of a trained airway appearance model, vessel and airway orientation information, and region growing. The method uses a voxel classification based appearance model, which involves the use of a classifier that is trai......This paper presents a method for airway tree segmentation that uses a combination of a trained airway appearance model, vessel and airway orientation information, and region growing. The method uses a voxel classification based appearance model, which involves the use of a classifier...... that is trained to differentiate between airway and non-airway voxels. Vessel and airway orientation information are used in the form of a vessel orientation similarity measure, which indicates how similar the orientation of the an airway candidate is to the orientation of the neighboring vessel. The method...

  11. Airway vascular reactivity and vascularisation in human chronic airway disease

    NARCIS (Netherlands)

    Bailey, Simon R; Boustany, Sarah; Burgess, Janette K; Hirst, Stuart J; Sharma, Hari S; Simcock, David E; Suravaram, Padmini R; Weckmann, Markus

    2009-01-01

    Altered bronchial vascular reactivity and remodelling including angiogenesis are documented features of asthma and other chronic inflammatory airway diseases. Expansion of the bronchial vasculature under these conditions involves both functional (vasodilation, hyperperfusion, increased microvascular

  12. Analysis of 36 cases of chronic pulmonary heart disease complicated with multiple organ dysfunction syndrome%慢性肺源性心脏病伴多器官功能障碍综合症36例分析

    Institute of Scientific and Technical Information of China (English)

    许丙俊; 刘志刚

    2012-01-01

    目的 探讨慢性肺源性心脏病伴发多器官功能障碍综合症的临床治疗.方法 对本院ICU诊治的36例慢性肺心病伴发多器官功能障碍综合症进行总结分析.结果 除肺、心以外,器官功能障碍发生比例由高到低依次为脑、肾、肝、胃肠.36例患者中17例死亡,死亡率为47%,其余患者好转出院或转其他相关科室继续治疗.结论 心肺功能越差,受累的脏器越多,则死亡率越高.%Objective To investigate the clinical treatment of chronir pulmonary heart disease romplirated with multiple organ dysiunction syndrome. Methods Clinical features of 36 cases of chronic pulmonary heart disease complicated with multiple organ dysfunr-tion syndrome in our hospital ICU from the 2007 ~ 2010 were retrospectively analyzed. Results Incidence of organ dysfunction from high to low was the brain, kidney, liver, gastrointestinal in addition to the lung and heart. In 36 patients, 17 patients died and the mortality rate was 47% . The other patients got better or transferred to other related departments to continue the treatment. Conclusion The Worser of heart and lung function, the number of organ involvement and the mortality is higher.

  13. T cell subsets in human airways prior to and following endobronchial administration of endotoxin

    DEFF Research Database (Denmark)

    Ronit, Andreas; Plovsing, Ronni R; Gaardbo, Julie C;

    2015-01-01

    BACKGROUND AND OBJECTIVES: Bronchial instillation of lipopolysaccharide (LPS) provides a reversible model of lung inflammation that may resemble early stages of acute respiratory distress syndrome (ARDS). We investigated the distributions of T-cell subsets in the human airways and sought to deter......BACKGROUND AND OBJECTIVES: Bronchial instillation of lipopolysaccharide (LPS) provides a reversible model of lung inflammation that may resemble early stages of acute respiratory distress syndrome (ARDS). We investigated the distributions of T-cell subsets in the human airways and sought...

  14. Diabetes and sexual dysfunction: current perspectives

    Directory of Open Access Journals (Sweden)

    Maiorino MI

    2014-03-01

    Full Text Available Maria Ida Maiorino,1 Giuseppe Bellastella,1 Katherine Esposito2 1Department of Medical, Surgical, Neurological, Metabolic and Geriatric Sciences, Second University of Naples, Naples, Italy; 2Department of Clinical and Experimental Medicine, Second University of Naples, Naples, Italy Abstract: Diabetes mellitus is one of the most common chronic diseases in nearly all countries. It has been associated with sexual dysfunction, both in males and in females. Diabetes is an established risk factor for sexual dysfunction in men, as a threefold increased risk of erectile dysfunction was documented in diabetic men, as compared with nondiabetic men. Among women, evidence regarding the association between diabetes and sexual dysfunction are less conclusive, although most studies have reported a higher prevalence of female sexual dysfunction in diabetic women as compared with nondiabetic women. Female sexual function appears to be more related to social and psychological components than to the physiological consequence of diabetes. Hyperglycemia, which is a main determinant of vascular and microvascular diabetic complications, may participate in the pathogenetic mechanisms of sexual dysfunction in diabetes. Moreover, diabetic people may present several clinical conditions, including hypertension, overweight and obesity, metabolic syndrome, cigarette smoking, and atherogenic dyslipidemia, which are themselves risk factors for sexual dysfunction, both in men and in women. The adoption of healthy lifestyles may reduce insulin resistance, endothelial dysfunction, and oxidative stress – all of which are desirable achievements in diabetic patients. Improved well-being may further contribute to reduce and prevent sexual dysfunction in both sexes. Keywords: diabetes mellitus, diabetes complications, erectile dysfunction, female sexual dysfunction, lifestyle changes

  15. Risk factors for sexual dysfunction in male patients with obstructive sleep apnea and hypoventilation syndrome%阻塞性睡眠呼吸暂停低通气综合征引起男性性功能障碍危险因素分析

    Institute of Scientific and Technical Information of China (English)

    刘松; 陈颖; 郝钢跃; 贺正一

    2013-01-01

    目的 探讨男性阻塞性睡眠呼吸暂停低通气综合征(OSAHS)患者性功能障碍的危险因素.方法 采用国际勃起功能指数量表(IIEF-5)评估121例男性22~50岁OSAHS患者性功能,分为性功能正常组(65例)和性功能障碍组(56例).比较两组患者临床资料和多导睡眠图监测结果.部分性功能障碍患者进行持续气道正压(CPAP)治疗后再次以IIEF-5评价性功能.结果 性功能障碍组患者体质量指数(BMI)显著高于性功能正常组[(31.5±3.9) kg/m2与(30.1±3.5) kg/m2,t=6.897,P<0.01],有糖尿病病史者显著多于性功能正常组(18例与10例,x2=4.750,P<0.05),晨间血压升高者显著多于性功能正常组(38例与27例,x2 =8.381,P<0.01);性功能障碍组患者多导睡眠图监测显示,呼吸紊乱指数(RDI)、最长呼吸暂停时间(Tmax)、最低脉搏血氧饱和度(LSp02)、脉搏血氧饱和度低于90%时间占总睡眠(TST)时间比(T90%)、浅睡眠(S1+S2)占ST比、深睡眠(S3+S4)占TST比、快动眼睡眠(REM)占TST比等指标与性功能正常组之间差异有统计学意义(t值分别为5.290、2.253、3.770、7.536、6.599、3.935和5.536,P均<0.05).多因素Logistic回归分析显示,糖尿病病史、多导睡眠图监测中S1 +S2占TST比、RDI和LSp02对男性OSAHS患者出现性功能障碍有统计学意义,其OR值(95%可信区间)分别为2.10(1.17 ~5.01)、3.52(1.33 ~ 5.97)、1.45(1.08 ~2.95)和0.86(0.67~0.93),P均<0.05.20例性功能障碍患者持续气道正压治疗2个月后14例经IIEF-5评分(>21分)正常.结论 具有糖尿病病史,睡眠结构紊乱S1 +S2占TST比增多、RDI较高和LSpO2较低是男性OSAHS患者发生性功能障碍的独立危险因素.持续气道正压治疗可以显著改善男性OSAHS患者性功能.%Objective To investigate the risk factors of sexual dysfunction in male patients with obstructive sleep apnea and hypoventilation syndrome (OSAHS).Methods One hundred and twenty-one OSAHS male

  16. Medical management of metabolic dysfunction in PCOS

    OpenAIRE

    Duleba, Antoni J.

    2011-01-01

    Polycystic ovary syndrome (PCOS) is associated with metabolic derangements including insulin resistance, dyslipidemia, systemic inflammation and endothelial dysfunction. There is a growing need to develop pharmacologic interventions to improve metabolic function in women with PCOS. Medications that have been tested in patients with PCOS include metformin, thiazolidinediones, acarbose, naltrexone, orlistat, vitamin D and statins.

  17. Short-Term Memory Impairment and Left Dorsolateral Prefrontal Cortex Dysfunction in the Orthostatic Position: A Single Case Study of Sinking Skin Flap Syndrome

    Directory of Open Access Journals (Sweden)

    Luca Sebastianelli

    2015-01-01

    Full Text Available We describe the case of a patient who underwent craniectomy for hemorrhage of the left parietal lobe. Three weeks later, orthostatic memory impairment was detected as initial symptom of sinking skin flap syndrome (SSFS. This deficit was examined by neuropsychological testing and associated with a posture-dependent increase in the delta/alpha ratio at the F3 electrode, an electroencephalographic (EEG index related to brain hypoperfusion. This EEG spectral alteration was detected in a brain region that includes the left dorsolateral prefrontal cortex, an area known to be involved in memory processing; therefore we hypothesize that SSFS induced reversible hypoperfusion of this otherwise undamaged cortical region. Neither of these findings was present after cranioplasty. This case suggests that SSFS may induce neuropsychological deficits potentially influencing outcome in the postacute phase and is further evidence supporting the clinical benefits of early cranioplasty.

  18. AB271. Sexual dysfunction in chronic prostatitis

    Science.gov (United States)

    Cho, In-Rae

    2016-01-01

    Chronic prostatitis/ chronic pelvic pain syndrome (CP/CPPS), or NIH category III prostatitis, is a clinical syndrome characterized by genital/ pelvic pain and lower urinary tract symptoms in the absence of urinary tract infection. CPPS is the most common prostatic disease in men younger than 50 years of age and the third most common in men older than 50 years of age. CP/CPPS is a complex entity with unclear etiology. Many articles reported that the high percentage of patients with CP/CPPS had sexual dysfunction. The most common symptoms of sexual dysfunction in chronic prostatitis patients are erectile dysfunction (ED), painful ejaculation and premature ejaculation. So we will discuss about ED and ejaculation problems in CP/CPPS patients.

  19. NTI-tss 咬合板治疗咀嚼肌筋膜痛的临床疗效观察%Effects of NTI-tss splint in the treatment of myofascial pain dysfunction syndrome

    Institute of Scientific and Technical Information of China (English)

    孙梅; 刘红

    2014-01-01

    20 patients with myofascial pain dysfunction syndrome(MPDS)were were treated by NTI-tss splint only during night for 2 months.After treatment,VAS decreased(P <0.01)and active maximum mouth opening degree increased(P <0.01).NTI-tss splint is ef-fective in the treatment of MPDS.Its side effect needs to be further observed.%咀嚼肌筋膜痛患者20名,夜间戴用 NTI-tss 咬合板治疗2个月。治疗后,患者咀嚼时咀嚼肌疼痛的 VAS 值较治疗前明显减小(P <0.01),主动最大张口度明显增加(P <0.01),疗效良好,可能存在的副作用有待进一步观察。

  20. Small airway obstruction in patients with rheumatoid arthritis.

    Science.gov (United States)

    Mori, Shunsuke; Koga, Yukinori; Sugimoto, Mineharu

    2011-04-01

    This work was intended to evaluate the prevalence of obstructive small-airway disease in patients with rheumatoid arthritis (RA) and its association with clinical characteristics. Pulmonary function testing (PFT) and high-resolution computed tomography (HRCT) were performed on 189 consecutive RA patients. Each case was diagnosed based on abnormal HRCT findings. We defined obstructive dysfunction of small airways as a forced expiratory flow from 25% to 75% of vital capacity (FEF(25-75)) value >1.96 residual standard deviation (RSD) below predicted values. We found 19 patients (10.1%) with an interstitial pneumonia (IP) pattern and 15 (7.9%) with a bronchiolitis pattern; the other 155 (82.0%) had no abnormal HRCT patterns. In patients with neither abnormal pattern, median values of percentage predicted for carbon monoxide diffusing capacity (DL(CO)) and ratio of DL(CO) to alveolar ventilation (DLco/VA) were within the normal range, but median FEF(25-75), forced expiratory flow at 25% of vital capacity (V(25)), and V(25)/height were dysfunction. Multivariate logistic regression analysis indicated that this type of abnormality is strongly associated with respiratory symptoms [odds ratio (OR) 5.18; 95% confidence interval (CI) 1.70-15.75; p = 0.012), smoking history (OR 2.78; 95% CI 1.10-6.99; p = 0.03), and disease duration >10 years (OR 2.86; 95% CI 1.27-6.48; p = 0.012). Parenchymal micronodules, bronchial-wall thickening, and bronchial dilatation on HRCT scans were also predictive factors for abnormal FEF(25-75), although these morphological changes were too limited for us to diagnose these patients with the bronchiolitis pattern. Obstructive dysfunction of small airways is apparently common among RA patients, even among those with neither the IP nor the bronchiolitis pattern on HRCT scans. Factors significantly associated with abnormal FEF(25-75) are respiratory symptoms, smoking history, and RA duration.

  1. A multicenter study on Leigh syndrome

    DEFF Research Database (Denmark)

    Sofou, Kalliopi; De Coo, Irenaeus F M; Isohanni, Pirjo;

    2014-01-01

    BACKGROUND: Leigh syndrome is a progressive neurodegenerative disorder, associated with primary or secondary dysfunction of the mitochondrial oxidative phosphorylation. Despite the fact that Leigh syndrome is the most common phenotype of mitochondrial disorders in children, longitudinal natural h...

  2. Progress in treatment of systemic inflammatory response syndrome and multiple organ dysfunction syndrome%全身炎症反应综合征和多器官功能障碍综合征的治疗进展

    Institute of Scientific and Technical Information of China (English)

    徐盛明; 刘明媛; 李保春

    2004-01-01

    全身炎症反应综合征(systemic inflammatory response syndrome,SIRS)和多器官功能障碍综合征(multiple organdysfunction syndrome,MODS)是重症监护病房(ICU)中治疗的难题.除传统的治疗方法外,近年来人们尝试通过免疫治疗来调节机体的炎症反应系统,纠正机体异常自身应答,从而达到治疗SIRS和MODS的目的.本文从阻断炎症反应的激活、调节炎症反应的活性、基因治疗以及中西医结合治疗几个方面综述了SIRS和MODS的治疗进展.

  3. Surfactant and allergic airway inflammation.

    Science.gov (United States)

    Winkler, Carla; Hohlfeld, Jens M

    2013-01-01

    Pulmonary surfactant is a complex mixture of unique proteins and lipids that covers the airway lumen. Surfactant prevents alveolar collapse and maintains airway patency by reducing surface tension at the air-liquid interface. Furthermore, it provides a defence against antigen uptake by binding foreign particles and enhancing cellular immune responses. Allergic asthma is associated with chronic airway inflammation and presents with episodes of airway narrowing. The pulmonary inflammation and bronchoconstriction can be triggered by exposure to allergens or pathogens present in the inhaled air. Pulmonary surfactant has the potential to interact with various immune cells which orchestrate allergen- or pathogen-driven episodes of airway inflammation. The complex nature of surfactant allows multiple sites of interaction, but also makes it susceptible to external alterations, which potentially impair its function. This duality of modulating airway physiology and immunology during inflammatory conditions, while at the same time being prone to alterations accompanied by restricted function, has stimulated numerous studies in recent decades, which are reviewed in this article. PMID:23896983

  4. 甲状腺功能异常与代谢综合征组分关系的流行病学研究%Relationship between the prevalence of thyroid dysfunction and components of metabolic syndrome: an epidemiological survey

    Institute of Scientific and Technical Information of China (English)

    毛玉山; 麦一峰; 洪中立; 杜娟; 朱忠伟

    2012-01-01

    Objective To study the relationship between the prevalence of thyroid dysfunction and components of metabolic syndrome.Methods A total of 10 461 working and retired employees aged 20 to 90 years in a Petrochemical Corporation in Ningbo were included.Body mass index ( BMI),waist circumference,blood pressure,fasting blood glucose,blood lipid profile,serum thyroid-stimulating hormone (TSH),free thyroxine ( FT4 ),and free triiodothyronine (FT3 ) were measured in all subjects. Metabolic syndrome was diagnosed according to the International Diabetes Federation(IDF) criteria.Results ( 1 ) The prevalences of metabolic syndrome and overall thyroid dysfunction were 10.2% and 4.6%,respectively. ( 2 ) 18.1% petrochemical employees had abdominal obesity with at least one component of metabolic syndrome.( 3 ) There was no significant difference in the prevalence of metabolic syndrome among the groups with lowered,normal,and elevated TSH. (4) Logistic regression analysis revealed that lowered high density lipoprotein cholesterol ( HDL-C ) was associated with lowered TSH ( OR =0.313,95% CI 0.184-0.530 ),and raised triglyceride was associated with elevated TSH ( OR =0.767,95% CI 0.595-0.991 ). ( 5 ) There were significant associations between serum TSH levels and lipid parameters such as total cholesterol in males,triglyceride and low density lipoprotein cholesterol in females,and HDL-C in both genders.Conclusion The prevalence of thyroid dysfunction was not associated with central obesity,hypertension,and hyperglycemia.Lipid disorder was correlated with serum TSH levels.%目的 探讨甲状腺功能异常与代谢综合征及其各组分的关系.方法 调查10 461名年龄20~90岁的宁波市某石化企业在职和退休员工人群,测定其体重指数、腰围、血压、空腹血糖、血脂谱和甲状腺功能.采用2005年国际糖尿病联盟(IDF)标准诊断代谢综合征.结果 (1)该企业员工甲状腺功能异常和代谢综

  5. Incidence of unanticipated difficult airway using an objective airway score versus a standard clinical airway assessment

    DEFF Research Database (Denmark)

    Nørskov, Anders Kehlet; Rosenstock, Charlotte Valentin; Wetterslev, Jørn;

    2013-01-01

    the examination and registration of predictors for difficult mask ventilation with a non-specified clinical airway assessment on prediction of difficult mask ventilation.Method/Design: We cluster-randomized 28 Danish departments of anaesthesia to airway assessment either by the SARI or by usual non......-specific assessment. Data from patients' pre-operative airway assessment are registered in the Danish Anaesthesia Database. Objective scores for intubation and mask ventilation grade the severity of airway managements. The accuracy of predicting difficult intubation and mask ventilation is measured for each group...... reduction equalling a number needed to treat of 180. Sample size estimation is adjusted for the study design and based on standards for randomization on cluster-level. With an average cluster size of 2,500 patients, 70,000 patients will be enrolled over a 1-year trial period. The database is programmed so...

  6. Immune Dysfunction in Rett Syndrome Patients Revealed by High Levels of Serum Anti-N(Glc IgM Antibody Fraction

    Directory of Open Access Journals (Sweden)

    Anna Maria Papini

    2014-01-01

    Full Text Available Rett syndrome (RTT, a neurodevelopmental disorder affecting exclusively (99% female infants, is associated with loss-of-function mutations in the gene encoding methyl-CpG binding protein 2 (MECP2 and, more rarely, cyclin-dependent kinase-like 5 (CDKL5 and forkhead box protein G1 (FOXG1. In this study, we aimed to evaluate the function of the immune system by measuring serum immunoglobulins (IgG and IgM in RTT patients (n=53 and, by comparison, in age-matched children affected by non-RTT pervasive developmental disorders (non-RTT PDD (n=82 and healthy age-matched controls (n=29. To determine immunoglobulins we used both a conventional agglutination assay and a novel ELISA based on antibody recognition by a surrogate antigen probe, CSF114(Glc, a synthetic N-glucosylated peptide. Both assays provided evidence for an increase in IgM titer, but not in IgG, in RTT patients relative to both healthy controls and non-RTT PDD patients. The significant difference in IgM titers between RTT patients and healthy subjects in the CSF114(Glc assay (P=0.001 suggests that this procedure specifically detects a fraction of IgM antibodies likely to be relevant for the RTT disease. These findings offer a new insight into the mechanism underlying the Rett disease as they unveil the possible involvement of the immune system in this pathology.

  7. Origins of and implementation concepts for upper airway stimulation therapy for obstructive sleep apnea.

    Science.gov (United States)

    Strohl M D, Kingman P; Baskin M D, Jonathan; Lance M D, Colleen; Ponsky M D, Diana; Weidenbecher M D, Mark; Strohl B A, Madeleine; Yamauchi M D, Motoo

    2016-07-01

    Upper airway stimulation, specifically hypoglossal (CN XII) nerve stimulation, is a new, alternative therapy for patients with obstructive sleep apnea hypopnea syndrome who cannot tolerate positive airway pressure, the first-line therapy for symptomatic patients. Stimulation therapy addresses the cause of inadequate upper airway muscle activation for nasopharyngeal and oropharyngeal airway collapse during sleep. The purpose of this report is to outline the development of this first-in-class therapy and its clinical implementation. Another practical theme is assessment of the features for considering a surgically implanted device and the insight as to how both clinical and endoscopic criteria increase the likelihood of safe and durable outcomes for an implant and how to more generally plan for management of CPAP-intolerant patients. A third theme is the team building required among sleep medicine and surgical specialties in the provision of individualized neurostimulation therapy. PMID:27424823

  8. Rett syndrome and dopaminergic system dysfunction%雷特综合征与多巴胺系统功能障碍

    Institute of Scientific and Technical Information of China (English)

    陆斌; 杨灿; 熊志奇

    2014-01-01

    雷特综合征(Rett syndrome)属于神经发育障碍类疾病,主要由×性染色体上mecp2基因突变所致,患者多数为女孩.临床症状于出生后6~18个月逐渐显现,主要表现为头部发育缓慢,已获得的语言及手部目的性运动技能消退,智力障碍,呼吸功能障碍及自闭倾向等.多巴胺系统的功能包括运动调节、奖赏学习、情感、内分泌调控以及药物成瘾等多个方面.由于多巴胺系统在运动和精神方面与雷特综合征部分临床症状存在表面相关性,早期有学者根据临床特征提出雷特综合征患者可能存在多巴胺系统功能障碍,但两者之间是否具有实质性的内在联系以及mecp2基因是否会通过影响多巴胺系统导致相关临床症状是目前雷特综合征研究的一个热点.本文将针对雷特综合征与多巴胺系统功能障碍的相关研究进展作一综述.

  9. Inter-dependent mechanisms behind cognitive dysfunction, vascular biology and Alzheimer’s dementia in Down syndrome: multi-faceted roles of APP

    Directory of Open Access Journals (Sweden)

    Dean eNizetic

    2015-12-01

    Full Text Available People with Down syndrome (DS virtually all develop intellectual disability (ID of varying degree of severity, and also have a high risk of early Alzheimer’s disease (AD. Intellectual disability prior to the onset of dementia, and its relationship to the onset of dementia in DS is a complex phenomenon influenced by many factors, and scarcely understood. Unravelling the causative factors and modulators of these processes remains a challenge, with potential to be informative for both ID and AD, for the development of early biomarkers and/or therapeutic approaches. We review the potential relative and inter-connected roles of the chromosome 21 gene for amyloid precursor protein (APP, in both pathological conditions. Rare non-DS people with duplication of APP (dupAPP get familial early onset AD (FEOAD with virtually 100% penetrance and prominent cerebrovascular pathology, but don’t suffer from ID before dementia onset. All of these features appear to be radically different in DS. On the other hand, rare individuals with partial trisomy 21 (T21 (with APP, but not DS-critical region in trisomy have been described having ID. Likewise, partial T21 DS (without APP trisomy show a range of ID, but no AD pathology. We review the multi-faceted roles of APP that might affect cognitive functioning. Given the fact that both Aβ secretion and synaptic maturation/plasticity are dependent on neuronal activity, we explore how this conflicting inter-dependency might affect cognitive pathogenesis in a dynamic way in DS, throughout the lifespan of an individual.

  10. Inter-Dependent Mechanisms Behind Cognitive Dysfunction, Vascular Biology and Alzheimer's Dementia in Down Syndrome: Multi-Faceted Roles of APP.

    Science.gov (United States)

    Nizetic, Dean; Chen, Christopher L; Hong, Wanjin; Koo, Edward H

    2015-01-01

    People with Down syndrome (DS) virtually all develop intellectual disability (ID) of varying degree of severity, and also have a high risk of early Alzheimer's disease (AD). ID prior to the onset of dementia, and its relationship to the onset of dementia in DS is a complex phenomenon influenced by many factors, and scarcely understood. Unraveling the causative factors and modulators of these processes remains a challenge, with potential to be informative for both ID and AD, for the development of early biomarkers and/or therapeutic approaches. We review the potential relative and inter-connected roles of the chromosome 21 gene for amyloid precursor protein (APP), in both pathological conditions. Rare non-DS people with duplication of APP (dupAPP) get familial early onset AD (FEOAD) with virtually 100% penetrance and prominent cerebrovascular pathology, but don't suffer from ID before dementia onset. All of these features appear to be radically different in DS. On the other hand, rare individuals with partial trisomy 21 (T21) (with APP, but not DS-critical region in trisomy) have been described having ID. Likewise, partial T21 DS (without APP trisomy) show a range of ID, but no AD pathology. We review the multi-faceted roles of APP that might affect cognitive functioning. Given the fact that both Aβ secretion and synaptic maturation/plasticity are dependent on neuronal activity, we explore how this conflicting inter-dependency might affect cognitive pathogenesis in a dynamic way in DS, throughout the lifespan of an individual. PMID:26648852

  11. Analysis of airways in computed tomography

    DEFF Research Database (Denmark)

    Petersen, Jens

    have become the standard with which to assess emphysema extent but airway abnormalities have so far been more challenging to quantify. Automated methods for analysis are indispensable as the visible airway tree in a CT scan can include several hundreds of individual branches. However, automation...... of scan on airway dimensions in subjects with and without COPD. The results show measured airway dimensions to be affected by differences in the level of inspiration and this dependency is again influenced by COPD. Inspiration level should therefore be accounted for when measuring airways, and airway...

  12. Role of Small Airways in Asthma.

    Science.gov (United States)

    Finkas, Lindsay K; Martin, Richard

    2016-08-01

    Asthma is an inflammatory condition of both the small and large airways. Recently the small airways have gained attention as studies have shown significant inflammation in the small airways in all severities of asthma. This inflammation has correlated with peripheral airway resistance and as a result, noninvasive methods to reliably measure small airways have been pursued. In addition, recent changes in asthma inhalers have led to alterations in drug formulations and the development of extrafine particle inhalers that improve delivery to the distal airways. PMID:27401620

  13. Asthma: vocal cord dysfunction (VCD) and other dysfunctional breathing disorders.

    Science.gov (United States)

    Balkissoon, Ron; Kenn, Klaus

    2012-12-01

    Vocal cord dysfunction (VCD) and dysfunctional breathing (DB) disorders may mimic or coexist with asthma, leading to overtreatment with corticosteroids with consequent morbidity. Iatrogenic complications can be averted by early and correct diagnosis. VCD, also termed paradoxical vocal fold motion disorder (PVFMD), is characterized by intermittent paradoxical adduction of the vocal cords, mainly during inspiration, leading to airflow obstruction and dyspnea. Patients with VCD may have repetitive emergency room visits due to acute dyspnea (mimicking exacerbations of asthma). In the seminal descriptions of VCD, young women (often with psychiatric issues) predominated; however, other groups at increased risk for developing VCD include elite athletes, military recruits, and individuals exposed to irritants (inhaled or aspirated). Chronic postnasal drip, laryngopharyngeal reflux (LPR), and gastroesophageal reflux (GER) may lead to laryngeal hyperresponsiveness. The diagnosis of VCD may be difficult because physical exam and spirometry may be normal between episodes. During symptomatic episodes, spirometry typically reveals variable extrathoracic airway obstruction (truncated inspiratory flow volume loop). The gold standard for identifying VCD is flexible fiberoptic rhinolaryngoscopy. Management of VCD includes identification and treatment of underlying disorders (eg, chronic postnasal drip, LPR, GER, anxiety, depression) and a multidisciplinary approach (including highly trained speech therapists). Speech therapy and biofeedback play a critical role in teaching techniques to override various dysfunctional breathing habits. When postnasal drip, LPR, or GER coexist, these disorders should be aggressively treated. With successful therapy, corticosteroids can often be discontinued. During severe, acute episodes of VCD, therapeutic strategies include heliox (80% helium/20% oxygen), topical lidocaine, anxiolytics, and superior laryngeal blocks with Clostridium botulinum toxin

  14. Sexual Dysfunction and Infertility

    Science.gov (United States)

    ... Sexual dysfunction is a problem in a person’s sexual desire, arousal, or orgasm. Sexual dysfunction is common. It ... find they have times when they have less sexual desire and satisfaction because of emotional distress or the ...

  15. Erectile Dysfunction (ED)

    Science.gov (United States)

    ... age. Is erectile dysfunction just a part of old age? Erectile dysfunction doesn't have to be a ... episode of impotence Feeling stressed, including stress from work or family situations Being troubled by problems in ...

  16. The App-Runx1 region is critical for birth defects and electrocardiographic dysfunctions observed in a Down syndrome mouse model.

    Directory of Open Access Journals (Sweden)

    Matthieu Raveau

    2012-05-01

    Full Text Available Down syndrome (DS leads to complex phenotypes and is the main genetic cause of birth defects and heart diseases. The Ts65Dn DS mouse model is trisomic for the distal part of mouse chromosome 16 and displays similar features with post-natal lethality and cardiovascular defects. In order to better understand these defects, we defined electrocardiogram (ECG with a precordial set-up, and we found conduction defects and modifications in wave shape, amplitudes, and durations in Ts65Dn mice. By using a genetic approach consisting of crossing Ts65Dn mice with Ms5Yah mice monosomic for the App-Runx1 genetic interval, we showed that the Ts65Dn viability and ECG were improved by this reduction of gene copy number. Whole-genome expression studies confirmed gene dosage effect in Ts65Dn, Ms5Yah, and Ts65Dn/Ms5Yah hearts and showed an overall perturbation of pathways connected to post-natal lethality (Coq7, Dyrk1a, F5, Gabpa, Hmgn1, Pde10a, Morc3, Slc5a3, and Vwf and heart function (Tfb1m, Adam19, Slc8a1/Ncx1, and Rcan1. In addition cardiac connexins (Cx40, Cx43 and sodium channel sub-units (Scn5a, Scn1b, Scn10a were found down-regulated in Ts65Dn atria with additional down-regulation of Cx40 in Ts65Dn ventricles and were likely contributing to conduction defects. All these data pinpoint new cardiac phenotypes in the Ts65Dn, mimicking aspects of human DS features and pathways altered in the mouse model. In addition they highlight the role of the App-Runx1 interval, including Sod1 and Tiam1, in the induction of post-natal lethality and of the cardiac conduction defects in Ts65Dn. These results might lead to new therapeutic strategies to improve the care of DS people.

  17. The Recent Curative Effect Observation of Adjuvanttherapy by Tongqiao Biyan Granules for the Upper Airway Cough Syndrome (UACS) in Children%通窍鼻炎颗粒辅助治疗儿童上气道咳嗽综合征的近期疗效观察

    Institute of Scientific and Technical Information of China (English)

    吴梦晖; 张竹君

    2012-01-01

      目的观察通窍鼻炎颗粒辅助治疗上气道咳嗽综合征的效果.方法84例6个月~14岁符合诊断标准的患儿均给予孟鲁司特钠咀嚼片、复方福尔可定溶液口服,伴脓涕者加抗生素治疗,脓涕量多者清洗鼻腔,观察组48例同时口服通窍鼻炎颗粒7~10d,对照组36例不再加用其他药物.比较两组治疗3d、7d后咳嗽及鼻部症状缓解情况.结果治疗3d后观察组、对照组咳嗽缓解率分别为65.58%、41.67%,(P <0.05),治疗7d 后咳嗽缓解率分别为81.25%、61.11%,P <0.05;治疗3d 后,观察组、对照组鼻部症状缓解率分别为58.33%、36.11%,P <0.05,治疗7d 后鼻部症状缓解率分别为75%、52.78%,P <0.05.结论上气道咳嗽综合征患儿在常规综合治疗的基础上联用通窍鼻炎颗粒可更有效缓解咳嗽和鼻部症状,优于常规综合治疗,值得临床推广使用.%  Objective To evaluate the recent curative effects of adjuvant therapy by Tongqiao Biyan granules for the Upper Airway Cough Syndrome(UACS) in children. Methods 84 cases of 6 to 14-year-old children with the Upper Airway Cough Syndrome were all given montelukast sodium chewable and the compound Pholcodine solution oral, with purulent nasal discharge by antibiotic ,with Nasal wash purulent amount increased by nasal lavage;48 cases of observation group were taken orally Tongqiao Biyan granules at the same time for 7 to 10 days;36 cases of control group didn't take any other drugs.The cough and nasal symptoms were to compare in two groups treated for 3 days and 7 days. Results After 3 days of treatment,the remission rate of cough were 65.58%、41.67% respectively in observation group and control group(P<0.05);After 7 days of treatment,the remission rate of cough were 81.25%、61.11% respectively (P<0.05) in two groups; After 3 days of treatment,the remission rate of nasal symptoms were 58.33%、36.11% respectively in observation group and control group(P<0.05);After 7 days of treatment

  18. The Airway Microbiome at Birth.

    Science.gov (United States)

    Lal, Charitharth Vivek; Travers, Colm; Aghai, Zubair H; Eipers, Peter; Jilling, Tamas; Halloran, Brian; Carlo, Waldemar A; Keeley, Jordan; Rezonzew, Gabriel; Kumar, Ranjit; Morrow, Casey; Bhandari, Vineet; Ambalavanan, Namasivayam

    2016-01-01

    Alterations of pulmonary microbiome have been recognized in multiple respiratory disorders. It is critically important to ascertain if an airway microbiome exists at birth and if so, whether it is associated with subsequent lung disease. We found an established diverse and similar airway microbiome at birth in both preterm and term infants, which was more diverse and different from that of older preterm infants with established chronic lung disease (bronchopulmonary dysplasia). Consistent temporal dysbiotic changes in the airway microbiome were seen from birth to the development of bronchopulmonary dysplasia in extremely preterm infants. Genus Lactobacillus was decreased at birth in infants with chorioamnionitis and in preterm infants who subsequently went on to develop lung disease. Our results, taken together with previous literature indicating a placental and amniotic fluid microbiome, suggest fetal acquisition of an airway microbiome. We speculate that the early airway microbiome may prime the developing pulmonary immune system, and dysbiosis in its development may set the stage for subsequent lung disease. PMID:27488092

  19. The Airway Microbiome at Birth

    Science.gov (United States)

    Lal, Charitharth Vivek; Travers, Colm; Aghai, Zubair H.; Eipers, Peter; Jilling, Tamas; Halloran, Brian; Carlo, Waldemar A.; Keeley, Jordan; Rezonzew, Gabriel; Kumar, Ranjit; Morrow, Casey; Bhandari, Vineet; Ambalavanan, Namasivayam

    2016-01-01

    Alterations of pulmonary microbiome have been recognized in multiple respiratory disorders. It is critically important to ascertain if an airway microbiome exists at birth and if so, whether it is associated with subsequent lung disease. We found an established diverse and similar airway microbiome at birth in both preterm and term infants, which was more diverse and different from that of older preterm infants with established chronic lung disease (bronchopulmonary dysplasia). Consistent temporal dysbiotic changes in the airway microbiome were seen from birth to the development of bronchopulmonary dysplasia in extremely preterm infants. Genus Lactobacillus was decreased at birth in infants with chorioamnionitis and in preterm infants who subsequently went on to develop lung disease. Our results, taken together with previous literature indicating a placental and amniotic fluid microbiome, suggest fetal acquisition of an airway microbiome. We speculate that the early airway microbiome may prime the developing pulmonary immune system, and dysbiosis in its development may set the stage for subsequent lung disease. PMID:27488092

  20. Extracorporeal Lung Support as a Bridge to Airway Stenting and Radiotherapy for Airway-Obstructing Pancoast Tumor.

    Science.gov (United States)

    McLenon, Melissa; Bittle, Gregory J; Jones, Kevin; Menaker, Jay; Pham, Si M; Iacono, Aldo T; Sachdeva, Ashutosh; Rajagopal, Keshava

    2016-07-01

    Venovenous (V-V) extracorporeal membrane oxygenation (ECMO) is used for respiratory failure that is suspected to be reversible (bridge to recovery), or as a bridge to lung transplantation. Patients with proximal airway obstruction due to endobronchial malignancy can develop acute respiratory failure, and may benefit from V-V ECMO as a bridge to airway intervention, further treatment, and eventual recovery. We describe a case of a superior sulcus tumor with tracheobronchial and superior vena cava invasion causing both respiratory failure and superior vena cava syndrome. This was treated successfully with V-V ECMO, bronchial stenting, and radiotherapy. PMID:27343540

  1. A two-hit dog model for the study of multiple organ dysfunction syndrome%两次打击致犬多器官功能衰竭的实验研究

    Institute of Scientific and Technical Information of China (English)

    陈继红; 张振宇; 李玉芳; 刘健

    2010-01-01

    Objective Beagle dogs were subjected to hemorrhagic shock plus resuscitation and endotoxiemia (two-hit) to set up multiple organ dysfunction syndrome (MODS) model. In the study, this model can be used to monitor the responses of organ compensation and in compensation. Method Seven male Beagle dogs, weight ( 15 ± 2) kg, were provided by animal experiment centre of Xinjiang Medical University. MODS model was set up in 7 Beagle dogs. Hemorrhagic shock was produced according to the method of Wigger. After the resuscitation,Escherichia coli endotoxin was given via the femoral vein at a dose of 1.5 mg/kg. The criteria of MODS were defined as the presence of two or more of organ dysfunction. Clinical biochemical values were examined before making model and 0 h,3 h,6 h,9 h, 12 h after the establishment of model. The pathological change of the liver and the kidney were observed under the light microscopy. Results Significant differences of WBC,PaO2,LP,ALT, AST,Cr and BUN were observed after the establishment of model compared with those before the establishment of model ( P < 0.05). Severe pathological lesions were observed in tissues of the liver and kidney. Conclusions Hemorrhagic shock and endotoxemia,a two-hit method, can be used to set up a delayed animal model for MODS to study the responses of organ dysfunction caused by ischemic and infectious diseases.%目的 通过失血性休克加内毒素血症两次打击建立犬多器官功能障碍综合征(MODS)的动物模型,研究动物各器官代偿与失代偿的反应过程.方法 选择由新疆医科大学实验动物中心提供的体质量(15±2)kg雄性Beagle犬7只,采用Wiggers法造成失血性休克,复苏12 h后由静脉持续12 h滴入1.5 mg/kg内毒素,建立MODS模型.MODS诊断标准为出现两个或两个以上器官功能障碍者.在建模前、建模后0,3,6,9,12 h观察实验犬各脏器功能,及肝、肾病理组织学变化.采用SPSS12.0软件包,于各时间点行重复

  2. Nap-titration : An effective alternative for continuous positive airway pressure titration

    NARCIS (Netherlands)

    Hoekema, A; Stegenga, B; Meinesz, AF; van der Hoeven, JH; Wijkstra, PJ

    2006-01-01

    When treating Obstructive Steep Apnea-Hypopnea Syndrome (OSAHS) several alternatives for standard (manual) continuous positive airway pressure (CPAP) titration are feasible. A practical alternative is titration without polysomnography during an afternoon nap (Nap-titration). The aim of the present s

  3. Treating asthma means treating airway smooth muscle cells

    NARCIS (Netherlands)

    Zuyderduyn, S; Sukkar, M B; Fust, A; Dhaliwal, S; Burgess, J K

    2008-01-01

    Asthma is characterised by airway hyperresponsiveness, airway inflammation and airway remodelling. Airway smooth muscle cells are known to be the main effector cells of airway narrowing. In the present paper, studies will be discussed that have led to a novel view of the role of airway smooth muscle

  4. Sphincter of Oddi Dysfunction

    Science.gov (United States)

    ... Intolerance Malabsorption Narcotic Bowel Syndrome Radiation Therapy Injury Short Bowel Syndrome Symptoms & Causes Treatments Nutrition and Diet Managing Secondary Effects Medications Surgery Daily Living with SBS Resources SMA Syndrome Volvulus ...

  5. Recent advances in airway management in children

    OpenAIRE

    Veyckemans, Francis

    2009-01-01

    Recent anatomic findings, technological progress, and both in vitro and in vivo studies of the pressure generated in the cuff of endotracheal tubes and supraglottic airways should lead to modification of the way we control the pediatric upper airway.

  6. The role of the small airways in the pathophysiology of asthma and chronic obstructive pulmonary disease.

    Science.gov (United States)

    Bonini, Matteo; Usmani, Omar S

    2015-12-01

    Chronic respiratory diseases, such as asthma and chronic obstructive pulmonary disease (COPD), represent a major social and economic burden for worldwide health systems. During recent years, increasing attention has been directed to the role of small airways in respiratory diseases, and their exact contribution to the pathophysiology of asthma and COPD continues to be clarified. Indeed, it has been suggested that small airways play a distinct role in specific disease phenotypes. Besides providing information on small airways structure and diagnostic procedures, this review therefore aims to present updated and evidence-based findings on the role of small airways in the pathophysiology of asthma and COPD. Most of the available information derives from either pathological studies or review articles and there are few data on the natural history of small airways disease in the onset or progression of asthma and COPD. Comparisons between studies on the role of small airways are hard to draw because both asthma and COPD are highly heterogeneous conditions. Most studies have been performed in small population samples, and different techniques to characterize aspects of small airways function have been employed in order to assess inflammation and remodelling. Most methods of assessing small airways dysfunction have been largely confined to research purposes, but some data are encouraging, supporting the utilization of certain techniques into daily clinical practice, particularly for early-stage diseases, when subjects are often asymptomatic and routine pulmonary function tests may be within normal ranges. In this context further clinical trials and real-life feedback on large populations are desirable.

  7. Pharmacogenetics, pharmacogenomics and airway disease

    Directory of Open Access Journals (Sweden)

    Hall Ian P

    2001-11-01

    Full Text Available Abstract The availability of a draft sequence for the human genome will revolutionise research into airway disease. This review deals with two of the most important areas impinging on the treatment of patients: pharmacogenetics and pharmacogenomics. Considerable inter-individual variation exists at the DNA level in targets for medication, and variability in response to treatment may, in part, be determined by this genetic variation. Increased knowledge about the human genome might also permit the identification of novel therapeutic targets by expression profiling at the RNA (genomics or protein (proteomics level. This review describes recent advances in pharmacogenetics and pharmacogenomics with regard to airway disease.

  8. Airway Tree Extraction with Locally Optimal Paths

    DEFF Research Database (Denmark)

    Lo, Pechin Chien Pau; Sporring, Jon; Pedersen, Jesper Johannes Holst;

    2009-01-01

    for tree extraction that can overcome local occlusions. The cost function for obtaining the optimal paths takes into account of an airway probability map as well as measures of airway shape and orientation derived from multi-scale Hessian eigen analysis on the airway probability. Significant improvements...

  9. Nrf2 protects against airway disorders

    International Nuclear Information System (INIS)

    Nuclear factor-erythroid 2 related factor 2 (Nrf2) is a ubiquitous master transcription factor that regulates antioxidant response elements (AREs)-mediated expression of antioxidant enzyme and cytoprotective proteins. In the unstressed condition, Kelch-like ECH-associated protein 1 (Keap1) suppresses cellular Nrf2 in cytoplasm and drives its proteasomal degradation. Nrf2 can be activated by diverse stimuli including oxidants, pro-oxidants, antioxidants, and chemopreventive agents. Nrf2 induces cellular rescue pathways against oxidative injury, abnormal inflammatory and immune responses, apoptosis, and carcinogenesis. Application of Nrf2 germ-line mutant mice has identified an extensive range of protective roles for Nrf2 in experimental models of human disorders in the liver, gastrointestinal tract, airway, kidney, brain, circulation, and immune or nerve system. In the lung, lack of Nrf2 exacerbated toxicity caused by multiple oxidative insults including supplemental respiratory therapy (e.g., hyperoxia, mechanical ventilation), cigarette smoke, allergen, virus, bacterial endotoxin and other inflammatory agents (e.g., carrageenin), environmental pollution (e.g., particles), and a fibrotic agent bleomycin. Microarray analyses and bioinformatic studies elucidated functional AREs and Nrf2-directed genes that are critical components of signaling mechanisms in pulmonary protection by Nrf2. Association of loss of function with promoter polymorphisms in NRF2 or somatic and epigenetic mutations in KEAP1 and NRF2 has been found in cohorts of patients with acute lung injury/acute respiratory distress syndrome or lung cancer, which further supports the role for NRF2 in these lung diseases. In the current review, we address the role of Nrf2 in airways based on emerging evidence from experimental oxidative disease models and human studies.

  10. Functional phenotype of airway myocytes from asthmatic airways

    NARCIS (Netherlands)

    Wright, David B.; Trian, Thomas; Siddiqui, Sana; Pascoe, Chris D.; Ojo, Oluwaseun O.; Johnson, Jill R.; Dekkers, Bart G. J.; Dakshinamurti, Shyamala; Bagchi, Rushita; Burgess, Janette K.; Kanabar, Varsha

    2013-01-01

    In asthma, the airway smooth muscle (ASM) cell plays a central role in disease pathogenesis through cellular changes which may impact on its microenvironment and alter ASM response and function. The answer to the long debated question of what makes a 'healthy' ASM cell become 'asthmatic' still remai

  11. Potassium permanganate toxicity: A rare case with difficult airway management and hepatic damage

    Directory of Open Access Journals (Sweden)

    Vijay Kumar Agrawal

    2014-01-01

    Full Text Available Potassium permanganate (KMnO 4 is rarely used for suicidal attempt. Its ingestion can lead to local as well as systemic toxicities due to coagulation necrosis and damage, caused by free radicals of permanganate. We recently managed a case of suicidal ingestion of KMnO 4 in a lethal dose. She had significant narrowing of upper airway leading to difficult intubation as well as hepatic dysfunction and coagulopathy as systemic manifestation. We suggest to keep ourselves ready to handle difficult airway with the aid of fiber optic bronchoscope or surgical airway management in such patients. Upper gastrointestinal (GI endoscopy should be done at the earliest to determine the extent of upper GI injury and further nutrition planning.

  12. Numerical simulation of soft palate movement and airflow in human upper airway by fluid-structure interaction method

    Institute of Scientific and Technical Information of China (English)

    Xiuzhen Sun; Chi Yu; Yuefang Wang; Yingxi Liu

    2007-01-01

    In this paper, the authors present airflow field characteristics of human upper airway and soft palate move-ment attitude during breathing. On the basis of the data taken from the spiral computerized tomography images of a healthy person and a patient with Obstructive Sleep Apnea-Hypopnea Syndrome (OSAHS), three-dimensional models of upper air-way cavity and soft palate are reconstructed by the method of surface rendering. Numerical simulation is performed for airflow in the upper airway and displacement of soft palate by fluid-structure interaction analysis. The reconstructed three-dimensional models precisely preserve the original config-uration of upper airways and soft palate. The results of the pressure and velocity distributions in the airflow field are quantitatively determined, and the displacement of soft palate is presented. Pressure gradients of airway are lower for the healthy person and the airflow distribution is quite uniform in the case of free breathing. However, the OSAHS patient remarkably escalates both the pressure and velocity in the upper airway, and causes higher displacement of the soft palate. The present study is useful in revealing pathogenesis and quantitative mutual relationship between configuration and function of the upper airway as well as in diagnosing diseases related to anatomical structure and function of the upper airway.

  13. Numerical simulation of soft palate movement and airflow in human upper airway by fluid-structure interaction method

    Science.gov (United States)

    Sun, Xiuzhen; Yu, Chi; Wang, Yuefang; Liu, Yingxi

    2007-08-01

    In this paper, the authors present airflow field characteristics of human upper airway and soft palate movement attitude during breathing. On the basis of the data taken from the spiral computerized tomography images of a healthy person and a patient with Obstructive Sleep Apnea-Hypopnea Syndrome (OSAHS), three-dimensional models of upper airway cavity and soft palate are reconstructed by the method of surface rendering. Numerical simulation is performed for airflow in the upper airway and displacement of soft palate by fluid-structure interaction analysis. The reconstructed three-dimensional models precisely preserve the original configuration of upper airways and soft palate. The results of the pressure and velocity distributions in the airflow field are quantitatively determined, and the displacement of soft palate is presented. Pressure gradients of airway are lower for the healthy person and the airflow distribution is quite uniform in the case of free breathing. However, the OSAHS patient remarkably escalates both the pressure and velocity in the upper airway, and causes higher displacement of the soft palate. The present study is useful in revealing pathogenesis and quantitative mutual relationship between configuration and function of the upper airway as well as in diagnosing diseases related to anatomical structure and function of the upper airway.

  14. Prolonged ozone exposure in an allergic airway disease model: Adaptation of airway responsiveness and airway remodeling

    Directory of Open Access Journals (Sweden)

    Park Chang-Soo

    2006-02-01

    Full Text Available Abstract Background Short-term exposure to high concentrations of ozone has been shown to increase airway hyper-responsiveness (AHR. Because the changes in AHR and airway inflammation and structure after chronic ozone exposure need to be determined, the goal of this study was to investigate these effects in a murine model of allergic airway disease. Methods We exposed BALB/c mice to 2 ppm ozone for 4, 8, and 12 weeks. We measured the enhanced pause (Penh to methacholine and performed cell differentials in bronchoalveolar lavage fluid. We quantified the levels of IL-4 and IFN-γ in the supernatants of the bronchoalveolar lavage fluids using enzyme immunoassays, and examined the airway architecture under light and electron microscopy. Results The groups exposed to ozone for 4, 8, and 12 weeks demonstrated decreased Penh at methacholine concentrations of 12.5, 25, and 50 mg/ml, with a dose-response curve to the right of that for the filtered-air group. Neutrophils and eosinophils increased in the group exposed to ozone for 4 weeks compared to those in the filtered-air group. The ratio of IL-4 to INF-γ increased significantly after exposure to ozone for 8 and 12 weeks compared to the ratio for the filtered-air group. The numbers of goblet cells, myofibroblasts, and smooth muscle cells showed time-dependent increases in lung tissue sections from the groups exposed to ozone for 4, 8, and 12 weeks. Conclusion These findings demonstrate that the increase in AHR associated with the allergic airway does not persist during chronic ozone exposure, indicating that airway remodeling and adaptation following repeated exposure to air pollutants can provide protection against AHR.

  15. Cigarette Smoke and Estrogen Signaling in Human Airway Smooth Muscle

    Directory of Open Access Journals (Sweden)

    Venkatachalem Sathish

    2015-06-01

    Full Text Available Aims: Cigarette smoke (CS in active smokers and second-hand smoke exposure exacerbate respiratory disorders such as asthma and chronic bronchitis. While women are known to experience a more asthmatic response to CS than emphysema in men, there is limited information on the mechanisms of CS-induced airway dysfunction. We hypothesize that CS interferes with a normal (protective bronchodilatory role of estrogens, thus worsening airway contractility. Methods: We tested effects of cigarette smoke extract (CSE on 17β-estradiol (E2 signaling in enzymatically-dissociated bronchial airway smooth muscle (ASM obtained from lung samples of non-smoking female patients undergoing thoracic surgery. Results: In fura-2 loaded ASM cells, CSE increased intracellular calcium ([Ca2+]i responses to 10µM histamine. Acute exposure to physiological concentrations of E2 decreased [Ca2+]i responses. However, in 24h exposed CSE cells, although expression of estrogen receptors was increased, the effect of E2 on [Ca2+]i was blunted. Acute E2 exposure also decreased store-operated Ca2+ entry and inhibited stromal interaction molecule 1 (STIM1 phosphorylation: effects blunted by CSE. Acute exposure to E2 increased cAMP, but less so in 24h CSE-exposed cells. 24h CSE exposure increased S-nitrosylation of ERα. Furthermore, 24h CSE-exposed bronchial rings showed increased bronchoconstrictor agonist responses that were not reduced as effectively by E2 compared to non-CSE controls. Conclusion: These data suggest that CS induces dysregulation of estrogen signaling in ASM, which could contribute to increased airway contractility in women exposed to CS.

  16. Motor Impairments in Angelman Syndrome

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2004-04-01

    Full Text Available Of 33 children and adolescents (median age 6 years investigated for learning disability, epilepsy, and motor dysfunction to detect suspected Angelman syndrome (AS, in a study at Goteborg University, Sweden, 23 fulfilled criteria for AS.

  17. [Airway equipment and its maintenance for a non difficult adult airway management (endotracheal intubation and its alternative: face mask, laryngeal mask airway, laryngeal tube)].

    Science.gov (United States)

    Francon, D; Estèbe, J P; Ecoffey, C

    2003-08-01

    The airway equipment for a non difficult adult airway management are described: endotracheal tubes with a specific discussion on how to inflate the balloon, laryngoscopes and blades, stylets and intubation guides, oral airways, face masks, laryngeal mask airways and laryngeal tubes. Cleaning and disinfections with the maintenance are also discussed for each type of airway management. PMID:12943860

  18. Pharyngeal airway changes following mandibular setback surgery

    Directory of Open Access Journals (Sweden)

    Babu Ramesh

    2005-01-01

    Full Text Available Treatment of dentofacial deformities with jaw osteotomies has an effect on airway anatomy and therefore mandibular setback surgery has the potential to diminish airway size. The purpose of this study was to evaluate the effect of mandibular setback surgery on airway size. 8 consecutive patients were examined prospectively. All patients underwent mandibular setback surgery. Cephalometric analysis was performed preoperatively and 3 months post operatively with particular attention to pharyngeal airway changes. Pharyngeal airway size decreased considerably in all, patients thus predisposing to development of obstructive sleep apnea. Therefore, large anteroposterior discrepancies should be corrected by combined maxillary and mandibular osteotomies.

  19. Inflammatory bowel disease and airway diseases

    Science.gov (United States)

    Vutcovici, Maria; Brassard, Paul; Bitton, Alain

    2016-01-01

    Airway diseases are the most commonly described lung manifestations of inflammatory bowel disease (IBD). However, the similarities in disease pathogenesis and the sharing of important environmental risk factors and genetic susceptibility suggest that there is a complex interplay between IBD and airway diseases. Recent evidence of IBD occurrence among patients with airway diseases and the higher than estimated prevalence of subclinical airway injuries among IBD patients support the hypothesis of a two-way association. Future research efforts should be directed toward further exploration of this association, as airway diseases are highly prevalent conditions with a substantial public health impact. PMID:27678355

  20. Determinants of sexual dysfunction and interventions for patients with obstructive sleep apnoea

    DEFF Research Database (Denmark)

    Steinke, E; Palm Johansen, P; Fridlund, B;

    2016-01-01

    dysfunction, while for men factors included BMI, hormonal status and inflammatory markers. Continuous positive airway pressure (CPAP) not only improved clinical measures such as excessive daytime sleepiness but also the erectile and orgasmic function. Nevertheless, sildenafil was superior CPAP with regard...

  1. Risk assessment of sleeping disorder breathing based on upper airway centerline evaluation

    Science.gov (United States)

    Alsufyani, Noura; Shen, Rui; Cheng, Irene; Major, Paul

    2013-02-01

    One of the most important breathing disorders in childhood is obstructive sleep apnea syndrome which affects 2-3% of children, and the reported failure rate of surgical treatment was as high as 54%. A possible reason in respiratory complications is having reduced dimensions of the upper airway which are further compressed when muscle tone is decreased during sleep. In this study, we use Cone-beam computed tomography (CBCT) to assess the location or cause of the airway obstruction. To date, all studies analyzing the upper airway in subjects with Sleeping Disorder Breathing were based on linear, area, or volumetric measurements, which are global computations and can easily ignore local significance. Skeletonization was initially introduced as a 3D modeling technique by which representative medial points of a model are extracted to generate centerlines for evaluations. Although centerlines have been commonly used in guiding surgical procedures, our novelty lies in comparing its geometric properties before and after surgeries. We apply 3D data refinement, registration and projection steps to quantify and localize the geometric deviation in target airway regions. Through cross validation with corresponding subjects' therapy data, we expect to quantify the tolerance threshold beyond which reduced dimensions of the upper airway are not clinically significant. The ultimate goal is to utilize this threshold to identify patients at risk of complications. Outcome from this research will also help establish a predictive model for training and to estimate treatment success based on airway measurements prior to intervention. Preliminary results demonstrate the feasibility of our approach.

  2. Mast cell-derived neurotrophin 4 mediates allergen-induced airway hyperinnervation in early life

    Science.gov (United States)

    Patel, Kruti R.; Aven, Linh; Shao, Fengzhi; Krishnamoorthy, Nandini; Duvall, Melody G.; Levy, Bruce D.; Ai, Xingbin

    2016-01-01

    Asthma often progresses from early episodes of insults. How early life events connect to long-term airway dysfunction remains poorly understood. We demonstrated previously that increased neurotrophin 4 (NT4) levels following early life allergen exposure cause persistent changes in airway smooth muscle (ASM) innervation and airway hyper-reactivity (AHR) in mice. Herein, we identify pulmonary mast cells as a key source of aberrant NT4 expression following early insults. NT4 is selectively expressed by ASM and mast cells in mice, nonhuman primates and humans. We show in mice that mast cell-derived NT4 is dispensable for ASM innervation during development. However, upon insults, mast cells expand in number and degranulate to release NT4 and thus become the major source of NT4 under pathological condition. Adoptive transfer of wild type mast cells, but not NT4−/− mast cells restores ASM hyperinnervation and AHR in KitW-sh/W-sh mice following early life insults. Notably, an infant nonhuman primate model of asthma also exhibits ASM hyperinnervation associated with the expansion and degranulation of mast cells. Together, these findings identify an essential role of mast cells in mediating ASM hyperinnervation following early life insults by producing NT4. This role may be evolutionarily conserved in linking early insults to long-term airway dysfunction. PMID:26860818

  3. Systems-level airway models of bronchoconstriction.

    Science.gov (United States)

    Donovan, Graham M

    2016-09-01

    Understanding lung and airway behavior presents a number of challenges, both experimental and theoretical, but the potential rewards are great in terms of both potential treatments for disease and interesting biophysical phenomena. This presents an opportunity for modeling to contribute to greater understanding, and here, we focus on modeling efforts that work toward understanding the behavior of airways in vivo, with an emphasis on asthma. We look particularly at those models that address not just isolated airways but many of the important ways in which airways are coupled both with each other and with other structures. This includes both interesting phenomena involving the airways and the layer of airway smooth muscle that surrounds them, and also the emergence of spatial ventilation patterns via dynamic airway interaction. WIREs Syst Biol Med 2016, 8:459-467. doi: 10.1002/wsbm.1349 For further resources related to this article, please visit the WIREs website. PMID:27348217

  4. State-dependent and reflex drives to the upper airway: Basic physiology with clinical implications

    OpenAIRE

    Horner, RL; Hughes, SW; Malhotra, A

    2014-01-01

    The root cause of the most common and serious of the sleep disorders is impairment of breathing, and a number of factors predispose a particular individual to hypoventilation during sleep. In turn, obstructive hypopneas and apneas are the most common of the sleep-related respiratory problems and are caused by dysfunction of the upper airway as a conduit for airflow. The overarching principle that underpins the full spectrum of clinical sleep-related breathing disorders is that the sleeping br...

  5. State-dependent and reflex drives to the upper airway: basic physiology with clinical implications

    OpenAIRE

    Horner, Richard L.; Hughes, Stuart W.; Malhotra, Atul

    2013-01-01

    The root cause of the most common and serious of the sleep disorders is impairment of breathing, and a number of factors predispose a particular individual to hypoventilation during sleep. In turn, obstructive hypopneas and apneas are the most common of the sleep-related respiratory problems and are caused by dysfunction of the upper airway as a conduit for airflow. The overarching principle that underpins the full spectrum of clinical sleep-related breathing disorders is that the sleeping br...

  6. Potassium permanganate toxicity: A rare case with difficult airway management and hepatic damage

    OpenAIRE

    Vijay Kumar Agrawal; Abhishek Bansal; Ranjeet Kumar; Bhanwar Lal Kumawat; Parul Mahajan

    2014-01-01

    Potassium permanganate (KMnO 4 ) is rarely used for suicidal attempt. Its ingestion can lead to local as well as systemic toxicities due to coagulation necrosis and damage, caused by free radicals of permanganate. We recently managed a case of suicidal ingestion of KMnO 4 in a lethal dose. She had significant narrowing of upper airway leading to difficult intubation as well as hepatic dysfunction and coagulopathy as systemic manifestation. We suggest to keep ourselves ready to handle difficul...

  7. The therapeutic effect of nutrition support in patients with multiple organ dysfunction syndrome (MODS)%营养支持对多器官功能不全综合征的治疗作用

    Institute of Scientific and Technical Information of China (English)

    徐鹏远; 许世才; 谭晶; 陈加勇; 甘平; 孙敏

    2000-01-01

    目的为了观察营养支持对多器官功能不全综合征(MODS)治疗的临床作用.方法对4例多器官功能不全综合征病人进行肠内与肠外营养治疗,其中男性2例,女性2例.4个重要脏器受损2例,3个器官受损2例.原发病因中2例为腹腔内严重感染,2例为重症坏死性胰腺炎.在治疗原发病及必要器械支持的同时给予病人肠内与肠外营养,早期以肠外营养支持为主,其中非蛋白热卡30Kcal·hg-1·d-1,脂肪占40%~50%,氮量0.25g·k8-1.d-1,如伴肾功能衰竭则氮热比降至1g:400Kcal,氮源主要给予必需氨基酸.所有病人均辅以少量肠内营养(口服谷氨酰胺Gln),并视肠功能情况逐渐向肠内营养过渡.结果3例病人痊愈出院,1例死亡.营养支持时间最长47天,最短23天.除死亡病例外,其余3例病人随营养支持全身情况和营养指标改善,治愈出院.讨论①要正确理解和接受MODS这一新的命名,它能更准确地反应此综合征进行性和可逆性的特点,从而指导早期诊断和治疗.②MODS病情凶险,机体处于严重应激,蛋白质分解,机体器官功能和免疫力下降,营养支持能为病人提供足够而合理的营养代谢底物,纠正机体负氮平衡,为MODS的抢救成功奠定基础.③严重应激时肠屏障功能受损,细菌和内毒素移位必将加重MODS,补充Gln和早期少量进食是预防和阻断加重MODS的重要环节,同时早期少量进食还能改善危重病人内脏微循环障碍,加速病人恢复.④保持内环境稳定对MODS的治疗很重要,对呼衰病人要限制糖量,提高脂肪乳比例.氮量的补充除肾衰外应提高支链氨基酸含量,肾衰则给予小量必需氨基酸.结论合理的营养支持能使MODS渡过危险而漫长的病理过程,改善预后.%Objective To estimate the clinical therapeutic effect of nutrition support in patients with Multiple Organ Dysfunction Syndrome (MODS). Methods We studied 4 patients (two male and two female

  8. Takotsubo Syndrome: Insights from Japan.

    Science.gov (United States)

    Akashi, Yoshihiro J; Ishihara, Masaharu

    2016-10-01

    We report the history and new insights of takotsubo syndrome based on the achievements that Japanese researchers have contributed and summarize the evidence originally presented from Japan. Takotsubo syndrome is a newly described heart failure characterized by transient left ventricular dysfunction. We should be aware of this entity as a syndrome, not actual cardiomyopathy. Japanese researchers focus on the experimental approaches for clinical diagnosis and treatment of takotsubo syndrome. As representatives from a country originally naming this syndrome takotsubo, a global registry for takotsubo syndrome including Japan should be established.

  9. 多囊卵巢综合征患者血管内皮功能损伤的研究%Selected markers of endothelial dysfunction in polycystic ovary syndrome

    Institute of Scientific and Technical Information of China (English)

    任涛; 熊小英; 栾峰; 蔡春芳

    2014-01-01

    目的 探讨多囊卵巢综合征(PCOS)患者血管内皮功能损伤的情况,以及其与代谢性指标相关性的研究.方法 30例PCOS患者和20例的正常妇女(对照组)为研究对象;于月经期第3天抽取空腹静脉血去血清,检测血清选择素E (E-selectin)、内皮素-1(ET-1)和血管内皮vWF因子水平及总胆固醇(TC)、三酰甘油(TG)、低密度脂蛋白胆固醇(LDL-C)和高密度脂蛋白胆固醇(HDL-C)的水平变化;测定空腹胰岛素及血糖(FBG)水平.结果 PCOS组血浆E-selectin和vWF、TG和LDL-C、血浆空腹血糖和胰岛素水平明显高于正常对照组(P均<0.05).PCOS患者血浆E-selectin与血糖、胰岛素及血三酰甘油呈正相关,r值分别为0.44、0.56和0.61,P均<0.05;尚不能认为与血胆固醇相关.PCOS患者血浆vWF与血糖、胰岛素及血三酰甘油呈正相关,r值分别为0.48,0.52和0.63,P均<0.05;尚不能认为与血胆固醇相关.PCOS组患者血ET-1仅与总胆固醇呈正相关性,r值为0.49,P<0.05.结论 PCOS患者中E-selectin和vWF的升高提示了其血管内皮功能受损,血糖、高胰岛素和血脂升高是PCOS中影响血管内皮损伤的重要因子.%Objective To investigate the endothelial dysfunction in patients with polycystic ovary syndrome(PCOS) and its correlation with metabolic status.Methods Thirty patients with PCOS and 20 normal women(NGT group) were enrolled in the study.Fasting blood samples were drawn from the vein on the third day of the menstrual cycle.Plasma levels of ET-1,vWF and soluble E-selectin were detected by ELISA.Lipid metabolism (total cholesterol,HDL-cholesterol,LDL-cholesterol,triglycerides) and glucose were also detected.Results The study showed higher levels of vWF,E-selectin,insulin and glucose and TG,LDL in PCOS patients versus healthy women(all P <0.05).A positive correlation was demonstrated between E-selectin and glucose,triglycerides and insulin (all P < 0.05).vWF levels showed a positive correlation with

  10. Patterns of airway involvement in inflammatory bowel diseases

    Institute of Scientific and Technical Information of China (English)

    Ilias; Papanikolaou; Konstantinos; Kagouridis; Spyros; A; Papiris

    2014-01-01

    Extraintestinal manifestations occur commonly in inflammatory bowel diseases(IBD). Pulmonary manifestations(PM) of IBD may be divided in airway disorders, interstitial lung disorders, serositis, pulmonary vasculitis, necrobiotic nodules, drug-induced lung disease, thromboembolic lung disease and enteropulmonary fistulas. Pulmonary involvement may often be asymptomatic and detected solely on the basis of abnormal screening tests. The common embryonic origin of the intestine and the lungs from the primitive foregut, the co-existence of mucosa associated lymphoid tissue in both organs, autoimmunity, smoking and bacterial translocation from the colon to the lungs may all be involved in the pathogenesis of PM in IBD. PM are mainly detected by pulmonary function tests and highresolution computed tomography. This review will focus on the involvement of the airways in the context of IBD, especially stenoses of the large airways, tracheo-bronchitis, bronchiectasis, bronchitis, mucoid impaction, bronchial granulomas, bronchiolitis, bronchiolitis obliterans syndrome and the co-existence of IBD with asthma, chronic obstructive pulmonary disease, sarcoidosis and a1-antitrypsin deficiency.

  11. Clinical Analysis of10Cases withMultipleOrganDysfunction SyndromeDuetoBeeStinginChildren%儿童蜂蜇伤致多脏器功能障碍综合征10例治疗体会

    Institute of Scientific and Technical Information of China (English)

    陈雯

    2013-01-01

    目的:探讨早期床旁连续性血液净化(CRRT)及综合治疗对蜂蜇伤导致多脏器功能障碍综合征(MODS)患儿的疗效。方法:回顾性分析武汉市儿童医院急救科2011年9月~11月收治的10例蜂蜇伤导致MODS重症患儿的诊治过程。结果:经早期床旁CRRT及综合治疗措施,7例痊愈,2例好转,1例死亡,总治愈好转率为90%,治愈率70%。结论:早期床旁CRRT治疗是抢救治疗蜂蜇伤导致MODS的关键,早期大剂量激素治疗对防止病情进一步加重起到重要作用,综合治疗,促进多器官功能恢复是抢救治疗该病切实有效的措施。%Objective:To investigate the clinical effects of early bedside continuous renal replace treatment (CRRT) and comprehensive treatment on children with multiple organ dysfunction syndrome (MODS) due to bee sting. Methods:Clinical data of 10 children with MODS caused by bee sting was analyzed retrospectively in the emergency department of Wuhan City Children's Hospital from September to November in 2011. Results:Through early bedside CRRT and comprehensive treatment, 7 patients were cured, 2 cases were improved, and one died. The improvement rate was 90%, and the cure rate was 70%. Conclusions:Early bedside CRRT、high-dose hormone and comprehensive treatment were effective measurements in control ing the conditions and promoting the multi-organ recovery.

  12. 有创动脉血压监测在多器官功能障碍患者中的应用研究%Application of Invasive Arterial Blood Pressure Monitoring in Patients with Multiple Organ Dysfunction Syndrome

    Institute of Scientific and Technical Information of China (English)

    凌双; 杨春万; 李展鹏

    2013-01-01

    Objective To explore the clinical value of invasive blood pressure monitoring in patients with multiple organ dysfunction syndrome (MODS). Methods From Jan. 2007 to Feb. 2012,90 patients with MODS were enrolled in this study,whose peripheral artery was punctured to measure the invasive blood pressure continuously. The invasive blood pressure values were recorded and simultaneously compared with non-invasive blood pressure values. Results The invasive blood pressure monitoring monitored the pressure of patients continuously and timely, the invasive blood pressure values were 5 - 20 mm Hg higher than the non-invasive blood pressure values, but their change were synchronistical. Conclusion Invasive blood pressure monitoring can guide the clinical diagnosis and therapy in the MODS patients because it can continuously and timely show the blood pressure and the artery pulse wave form.%目的 探讨有创动脉血压监测在多器官功能障碍患者中的临床应用价值.方法 选择2007年1月-2012年2月90例多器官功能障碍患者,采用动脉留置针对多器官功能障碍患者的外周动脉进行穿刺,将留置针导管通过压力传感器测压管道与压力监测仪相连,监测动脉压力和波形,将数据与患者的同肢体无创动脉血压监测数据相对比,观察两组在血压数据的差异性.结果 有创动脉血压值比无创动脉血压值高5 ~20 mm Hg(1 mm Hg=0.133 kPa),与无创动脉血压的变化同步,而且可实时连续监测血压.结论 有创动脉血压监测较无创动脉血压监测可以准确、实时、连续监测血压,有创动脉血压监测使医生正确、及时地评估病人的病情和治疗反应,及时指导和调整治疗计划,提高患者抢救成功率和降低死亡率,值得推广应用.

  13. Primary sleep apnoea syndrome.

    OpenAIRE

    Chokroverty, S.; Sharp, J T

    1981-01-01

    Polygraphic study in 18 men with the sleep apnoea syndrome showed central, upper airway obstructive, and mixed apnoeas. Fifty per cent of the total apnoea time was central, 33% was obstructive, and 17% was mixed. Apnoeic episodes were accompanied by oxygen desaturation, relative bradycardia and hypotonia of orofacial muscles innervated by ponto-medullary neurons. During regular breathing these muscles revealed tonic and phasic inspiratory EMG activities. The data suggest that the primary slee...

  14. High prevalence of obstructive sleep apnea in Marfan's syndrome

    Institute of Scientific and Technical Information of China (English)

    Mo Li; He Quanying; Wang Yinna; Dong Birong; He Jinhan

    2014-01-01

    Objective To review the current evidence about the prevalence of obstructive sleep apnea in patients with Marfan's syndrome,and discuss some proposed potential mechanisms for this relationship.Data sources The data in this review were mainly from Medline and PubMed articles published in English from 1990 to 2013.The search term was "Marfan's syndrome and sleep apnea".Study selection Clinical evidence about the epidemiology of obstructive sleep apnea in patients with Marfan's syndrome; the mechanism that causes obstructive sleep apnea; interventional therapy for patients with Marfan's syndrome,and coexisting obstructive sleep apnea.Results A high prevalence of obstructive sleep apnea exists in patients with Marfan's syndrome.The potential reasons are craniofacial abnormalities and lax upper airway muscles,which lead to high nasal airway resistance and upper airway collapse.Obstructive sleep apnea mechanically deteriorates aortic dilatation and accelerates progression of aortic aneurysms.The condition is reversible and rapid maxillary expansion and adequate continuous positive airway pressure therapy are possible effective therapies to delay the expansion of aortic diameter in patients with Marfan's syndrome.Conclusions Obstructive sleep apnea is strongly associated with Marfan's syndrome.Craniofacial abnormalities and lax upper airway are the main mechanisms.Untreated obstructive sleep apnea accelerates progression of aortic dissection and rupture.Effective therapies for obstructive sleep apnea could postpone the aortic dilatation in patients with Marfan's syndrome.

  15. Metabolic Syndrome: Polycystic Ovary Syndrome.

    Science.gov (United States)

    Mortada, Rami; Williams, Tracy

    2015-08-01

    Polycystic ovary syndrome (PCOS) is a heterogeneous condition characterized by androgen excess, ovulatory dysfunction, and polycystic ovaries. It is the most common endocrinopathy among women of reproductive age, affecting between 6.5% and 8% of women, and is the most common cause of infertility. Insulin resistance is almost always present in women with PCOS, regardless of weight, and they often develop diabetes and metabolic syndrome. The Rotterdam criteria are widely used for diagnosis. These criteria require that patients have at least two of the following conditions: hyperandrogenism, ovulatory dysfunction, and polycystic ovaries. The diagnosis of PCOS also requires exclusion of other potential etiologies of hyperandrogenism and ovulatory dysfunction. The approach to PCOS management differs according to the presenting symptoms and treatment goals, particularly the patient's desire for pregnancy. Weight loss through dietary modifications and exercise is recommended for patients with PCOS who are overweight. Oral contraceptives are the first-line treatment for regulating menstrual cycles and reducing manifestations of hyperandrogenism, such as acne and hirsutism. Clomiphene is the first-line drug for management of anovulatory infertility. Metformin is recommended for metabolic abnormalities such as prediabetes, and a statin should be prescribed for cardioprotection if the patient meets standard criteria for statin therapy. PMID:26280343

  16. Airway epithelial homeostasis and planar cell polarity signaling depend on multiciliated cell differentiation

    Science.gov (United States)

    Vladar, Eszter K.; Nayak, Jayakar V.; Milla, Carlos E.; Axelrod, Jeffrey D.

    2016-01-01

    Motile airway cilia that propel contaminants out of the lung are oriented in a common direction by planar cell polarity (PCP) signaling, which localizes PCP protein complexes to opposite cell sides throughout the epithelium to orient cytoskeletal remodeling. In airway epithelia, PCP is determined in a 2-phase process. First, cell-cell communication via PCP complexes polarizes all cells with respect to the proximal-distal tissue axis. Second, during ciliogenesis, multiciliated cells (MCCs) undergo cytoskeletal remodeling to orient their cilia in the proximal direction. The second phase not only directs cilium polarization, but also consolidates polarization across the epithelium. Here, we demonstrate that in airway epithelia, PCP depends on MCC differentiation. PCP mutant epithelia have misaligned cilia, and also display defective barrier function and regeneration, indicating that PCP regulates multiple aspects of airway epithelial homeostasis. In humans, MCCs are often sparse in chronic inflammatory diseases, and these airways exhibit PCP dysfunction. The presence of insufficient MCCs impairs mucociliary clearance in part by disrupting PCP-driven polarization of the epithelium. Consistent with defective PCP, barrier function and regeneration are also disrupted. Pharmacological stimulation of MCC differentiation restores PCP and reverses these defects, suggesting its potential for broad therapeutic benefit in chronic inflammatory disease. PMID:27570836

  17. Airway epithelial homeostasis and planar cell polarity signaling depend on multiciliated cell differentiation

    Science.gov (United States)

    Vladar, Eszter K.; Nayak, Jayakar V.; Milla, Carlos E.; Axelrod, Jeffrey D.

    2016-01-01

    Motile airway cilia that propel contaminants out of the lung are oriented in a common direction by planar cell polarity (PCP) signaling, which localizes PCP protein complexes to opposite cell sides throughout the epithelium to orient cytoskeletal remodeling. In airway epithelia, PCP is determined in a 2-phase process. First, cell-cell communication via PCP complexes polarizes all cells with respect to the proximal-distal tissue axis. Second, during ciliogenesis, multiciliated cells (MCCs) undergo cytoskeletal remodeling to orient their cilia in the proximal direction. The second phase not only directs cilium polarization, but also consolidates polarization across the epithelium. Here, we demonstrate that in airway epithelia, PCP depends on MCC differentiation. PCP mutant epithelia have misaligned cilia, and also display defective barrier function and regeneration, indicating that PCP regulates multiple aspects of airway epithelial homeostasis. In humans, MCCs are often sparse in chronic inflammatory diseases, and these airways exhibit PCP dysfunction. The presence of insufficient MCCs impairs mucociliary clearance in part by disrupting PCP-driven polarization of the epithelium. Consistent with defective PCP, barrier function and regeneration are also disrupted. Pharmacological stimulation of MCC differentiation restores PCP and reverses these defects, suggesting its potential for broad therapeutic benefit in chronic inflammatory disease.

  18. Somatosensory evaluation in Dysfunctional Syndrome patients.

    Science.gov (United States)

    Hilgenberg-Sydney, P B; Kowacs, P A; Conti, P C R

    2016-02-01

    Many chronic pain patients are refractory to treatment, which leads to the suspicion that somehow they are not fully effective and probably some mechanism of pain generation and/or maintenance is still unknown. The aim of this cross-sectional study was to provide evidence-based data on pain mechanisms in different types of chronic pain conditions. Eighty women, with 18-65 years old, were included, divided into four groups: myofascial pain of the masticatory muscles (n = 20), fibromyalgia (n = 20), chronic daily headache and healthy volunteers (n = 20). All patients were submitted to quantitative sensory tests: pressure pain threshold, mechanical detection threshold, mechanical pain threshold, ischaemic pain tolerance, cold pain sensitivity, aftersensation, wind-up ratio and conditioned pain modulation. Current perception threshold was also determined (Neurometer CPT/C - Neurotron). Three different zones were evaluated: trigeminal (masseter muscle), cervical and extratrigeminal (thenar eminence). Data were recorded and subjected to statistical analysis (anova, Tukey and Student's t-tests). Masticatory myofascial pain, fibromyalgia and chronic daily headache individuals presented lower pressure pain thresholds than healthy volunteers (P = 0.00). Chronic daily headache individuals had a significantly higher mechanical detection threshold than healthy volunteers (P = 0.01). Individuals of the symptomatic groups showed lower values for mechanical pain threshold and for ischaemic pain tolerance (P = 0.00) than healthy volunteers. The ability to activate the mechanism of endogenous modulation is impaired in women with fibromyalgia and myofascial pain (P = 0.00). These results reinforce evidence of central sensitisation and impaired endogenous modulation system in individuals with myofascial pain, fibromyalgia and chronic daily headache.

  19. Integrated care pathways for airway diseases (AIRWAYS-ICPs).

    Science.gov (United States)

    Bousquet, J; Addis, A; Adcock, I; Agache, I; Agusti, A; Alonso, A; Annesi-Maesano, I; Anto, J M; Bachert, C; Baena-Cagnani, C E; Bai, C; Baigenzhin, A; Barbara, C; Barnes, P J; Bateman, E D; Beck, L; Bedbrook, A; Bel, E H; Benezet, O; Bennoor, K S; Benson, M; Bernabeu-Wittel, M; Bewick, M; Bindslev-Jensen, C; Blain, H; Blasi, F; Bonini, M; Bonini, S; Boulet, L P; Bourdin, A; Bourret, R; Bousquet, P J; Brightling, C E; Briggs, A; Brozek, J; Buhl, R; Bush, A; Caimmi, D; Calderon, M; Calverley, P; Camargos, P A; Camuzat, T; Canonica, G W; Carlsen, K H; Casale, T B; Cazzola, M; Cepeda Sarabia, A M; Cesario, A; Chen, Y Z; Chkhartishvili, E; Chavannes, N H; Chiron, R; Chuchalin, A; Chung, K F; Cox, L; Crooks, G; Crooks, M G; Cruz, A A; Custovic, A; Dahl, R; Dahlen, S E; De Blay, F; Dedeu, T; Deleanu, D; Demoly, P; Devillier, P; Didier, A; Dinh-Xuan, A T; Djukanovic, R; Dokic, D; Douagui, H; Dubakiene, R; Eglin, S; Elliot, F; Emuzyte, R; Fabbri, L; Fink Wagner, A; Fletcher, M; Fokkens, W J; Fonseca, J; Franco, A; Frith, P; Furber, A; Gaga, M; Garcés, J; Garcia-Aymerich, J; Gamkrelidze, A; Gonzales-Diaz, S; Gouzi, F; Guzmán, M A; Haahtela, T; Harrison, D; Hayot, M; Heaney, L G; Heinrich, J; Hellings, P W; Hooper, J; Humbert, M; Hyland, M; Iaccarino, G; Jakovenko, D; Jardim, J R; Jeandel, C; Jenkins, C; Johnston, S L; Jonquet, O; Joos, G; Jung, K S; Kalayci, O; Karunanithi, S; Keil, T; Khaltaev, N; Kolek, V; Kowalski, M L; Kull, I; Kuna, P; Kvedariene, V; Le, L T; Lodrup Carlsen, K C; Louis, R; MacNee, W; Mair, A; Majer, I; Manning, P; de Manuel Keenoy, E; Masjedi, M R; Melen, E; Melo-Gomes, E; Menzies-Gow, A; Mercier, G; Mercier, J; Michel, J P; Miculinic, N; Mihaltan, F; Milenkovic, B; Molimard, M; Momas, I; Montilla-Santana, A; Morais-Almeida, M; Morgan, M; N'Diaye, M; Nafti, S; Nekam, K; Neou, A; Nicod, L; O'Hehir, R; Ohta, K; Paggiaro, P; Palkonen, S; Palmer, S; Papadopoulos, N G; Papi, A; Passalacqua, G; Pavord, I; Pigearias, B; Plavec, D; Postma, D S; Price, D; Rabe, K F; Radier Pontal, F; Redon, J; Rennard, S; Roberts, J; Robine, J M; Roca, J; Roche, N; Rodenas, F; Roggeri, A; Rolland, C; Rosado-Pinto, J; Ryan, D; Samolinski, B; Sanchez-Borges, M; Schünemann, H J; Sheikh, A; Shields, M; Siafakas, N; Sibille, Y; Similowski, T; Small, I; Sola-Morales, O; Sooronbaev, T; Stelmach, R; Sterk, P J; Stiris, T; Sud, P; Tellier, V; To, T; Todo-Bom, A; Triggiani, M; Valenta, R; Valero, A L; Valiulis, A; Valovirta, E; Van Ganse, E; Vandenplas, O; Vasankari, T; Vestbo, J; Vezzani, G; Viegi, G; Visier, L; Vogelmeier, C; Vontetsianos, T; Wagstaff, R; Wahn, U; Wallaert, B; Whalley, B; Wickman, M; Williams, D M; Wilson, N; Yawn, B P; Yiallouros, P K; Yorgancioglu, A; Yusuf, O M; Zar, H J; Zhong, N; Zidarn, M; Zuberbier, T

    2014-08-01

    The objective of Integrated Care Pathways for Airway Diseases (AIRWAYS-ICPs) is to launch a collaboration to develop multi-sectoral care pathways for chronic respiratory diseases in European countries and regions. AIRWAYS-ICPs has strategic relevance to the European Union Health Strategy and will add value to existing public health knowledge by: 1) proposing a common framework of care pathways for chronic respiratory diseases, which will facilitate comparability and trans-national initiatives; 2) informing cost-effective policy development, strengthening in particular those on smoking and environmental exposure; 3) aiding risk stratification in chronic disease patients, using a common strategy; 4) having a significant impact on the health of citizens in the short term (reduction of morbidity, improvement of education in children and of work in adults) and in the long-term (healthy ageing); 5) proposing a common simulation tool to assist physicians; and 6) ultimately reducing the healthcare burden (emergency visits, avoidable hospitalisations, disability and costs) while improving quality of life. In the longer term, the incidence of disease may be reduced by innovative prevention strategies. AIRWAYSICPs was initiated by Area 5 of the Action Plan B3 of the European Innovation Partnership on Active and Healthy Ageing. All stakeholders are involved (health and social care, patients, and policy makers). PMID:24925919

  20. MUCOCILIARY DYSFUNCTION IN HIV AND SMOKED SUBSTANCE ABUSE

    Directory of Open Access Journals (Sweden)

    Srinivasan eChinnapaiyan

    2015-10-01

    Full Text Available Impaired mucociliary clearance (MCC is a hallmark of acquired chronic airway diseases like chronic bronchitis associated with chronic obstructive pulmonary disease (COPD and asthma. This manifests as microbial colonization of the lung consequently leading to recurrent respiratory infections. People living with HIV demonstrate increased incidence of these chronic airway diseases. Bacterial pneumonia continues to be an important comorbidity in people living with HIV even though anti-retroviral therapy has succeeded in restoring CD4+ cell counts. People living with HIV demonstrate increased microbial colonization of the lower airways. The microbial flora is similar to that observed in diseases like cystic fibrosis and COPD suggesting that mucociliary dysfunction could be a contributing factor to the increased incidence of chronic airway diseases in people living with HIV. The three principal components of the MCC apparatus are, a mucus layer, ciliary beating and a periciliary airway surface liquid (ASL layer that facilitates ciliary beating. Cystic fibrosis transmembrane conductance regulator (CFTR plays a pivotal role in regulating the periciliary airway surface liquid. HIV proteins can suppress all the components of the MCC apparatus by increasing mucus secretion and suppressing CFTR function. This can decrease ASL height leading to suppressed ciliary beating. The effects of HIV on MCC are exacerbated when combined with other aggravating factors like smoking or inhaled substance abuse, which by themselves can suppress one or more components of the MCC system. This review discusses the pathophysiological mechanisms that lead to MCC suppression in people living with HIV who also smoke tobacco or abuse illicit drugs.

  1. Paediatric airway management: What is new?

    Directory of Open Access Journals (Sweden)

    S Ramesh

    2012-01-01

    Full Text Available Airway management plays a pivotal role in Paediatric Anaesthesia. Over the last two decades many improvements in this area have helped us to overcome this final frontier. From an era where intubation with a conventional laryngoscope or blind nasal intubation was the only tool for airway management, we have come a long way. Today supraglottic airway devices have pride of place in the Operating Room and are becoming important airway devices used in routine procedures. Direct and indirect fibreoptic laryngoscopes and transtracheal devices help us overcome difficult and previously impossible airway situations. These developments mean that we need to update our knowledge on these devices. Also much of our basic understanding of the physiology and anatomy of the paediatric airway has changed. This article attempts to shed light on some of the most important advances/opinions in paediatric airway management like, cuffed endotracheal tubes, supraglottic airway devices, video laryngoscopes, rapid sequence intubation, the newly proposed algorithm for difficult airway management and the role of Ex Utero Intrapartum Treatment (EXIT procedure in the management of the neonatal airway.

  2. Paediatric airway management: What is new?

    Science.gov (United States)

    Ramesh, S; Jayanthi, R; Archana, SR

    2012-01-01

    Airway management plays a pivotal role in Paediatric Anaesthesia. Over the last two decades many improvements in this area have helped us to overcome this final frontier. From an era where intubation with a conventional laryngoscope or blind nasal intubation was the only tool for airway management, we have come a long way. Today supraglottic airway devices have pride of place in the Operating Room and are becoming important airway devices used in routine procedures. Direct and indirect fibreoptic laryngoscopes and transtracheal devices help us overcome difficult and previously impossible airway situations. These developments mean that we need to update our knowledge on these devices. Also much of our basic understanding of the physiology and anatomy of the paediatric airway has changed. This article attempts to shed light on some of the most important advances/opinions in paediatric airway management like, cuffed endotracheal tubes, supraglottic airway devices, video laryngoscopes, rapid sequence intubation, the newly proposed algorithm for difficult airway management and the role of Ex Utero Intrapartum Treatment (EXIT) procedure in the management of the neonatal airway. PMID:23293383

  3. Temporomandibular joint dysfunction in children

    Directory of Open Access Journals (Sweden)

    Fernanda Mara de Paiva BERTOLI

    2009-03-01

    Full Text Available Introduction and objective: The aim of this study was to review aspects related to the temporomandibular dysfunctions (TMD in children,like etiology, diagnosis and treatment, emphasizing the importance of the correct diagnosis, since these patients are in their growth and development of the face period. Literature review: The TMDs include many clinical problems that involve the mastigatory muscles, the temporomandibular joint and near structures. In children the symptoms of this syndrome are present although with mild intensitywhen compared to adults. In relation to the prevalence, there is a great amount of discordance between the authors. The TMDs in childrenhave multifatorial etiology, and the most cited in literature areparafunctional habits, traumas, occlusal, systemic and psychologicalfactors. Conclusion: The signs and symptoms of the TMDs in pediatricpopulations are generally mild and increase with age, being veryimportant the early diagnosis and the inclusion of this kind of exam in routine examinations.

  4. Liquid Therapy Delivery Models Using Microfluidic Airways

    Science.gov (United States)

    Mulligan, Molly K.; Grotberg, James B.; Waisman, Dan; Filoche, Marcel; Sznitman, Josué

    2013-11-01

    The propagation and break-up of viscous and surfactant-laden liquid plugs in the lungs is an active area of research in view of liquid plug installation in the lungs to treat a host of different pulmonary conditions. This includes Infant Respiratory Distress Syndrome (IRDS) the primary cause of neonatal death and disability. Until present, experimental studies of liquid plugs have generally been restricted to low-viscosity Newtonian fluids along a single bifurcation. However, these fluids reflect poorly the actual liquid medication therapies used to treat pulmonary conditions. The present work attempts to uncover the propagation, rupture and break-up of liquid plugs in the airway tree using microfluidic models spanning three or more generations of the bronchiole tree. Our approach allows the dynamics of plug propagation and break-up to be studied in real-time, in a one-to-one scale in vitro model, as a function of fluid rheology, trailing film dynamics and bronchial tree geometry. Understanding these dynamics are a first and necessary step to deliver more effectively boluses of liquid medication to the lungs while minimizing the injury caused to epithelial cells lining the lungs from the rupture of such liquid plugs.

  5. Advances in sepsis-associated liver dysfunction

    Directory of Open Access Journals (Sweden)

    Dawei Wang

    2014-07-01

    Full Text Available Recent studies have revealed liver dysfunction as an early event in sepsis. Sepsis-associated liver dysfunction is mainly resulted from systemic or microcirculatory disturbances, spillovers of bacteria and endotoxin (lipopolysaccharide, LPS, and subsequent activation of inflammatory cytokines as well as mediators. Three main cell types of the liver which contribute to the hepatic response in sepsis are Kupffer cells (KCs, hepatocytes and liver sinusoidal endothelial cells (LSECs. In addition, activated neutrophils, which are also recruited to the liver and produce potentially destructive enzymes and oxygen-free radicals, may further enhance acute liver injury. The clinical manifestations of sepsis-associated liver dysfunction can roughly be divided into two categories: Hypoxic hepatitis and jaundice. The latter is much more frequent in the context of sepsis. Hepatic failure is traditionally considered as a late manifestation of sepsis-induced multiple organ dysfunction syndrome. To date, no specific therapeutics for sepsis-associated liver dysfunction are available. Treatment measure is mainly focused on eradication of the underlying infection and management for severe sepsis. A better understanding of the pathophysiology of liver response in sepsis may lead to further increase in survival rates.

  6. Compound Pseudoephedrine Hydrochloride Oral Solution Treatment of Upper Airway Cough Syndrome 93 Cases of Clinical Observation%美敏伪麻溶液治疗上气道咳嗽综合征93例临床观察

    Institute of Scientific and Technical Information of China (English)

    李晓荣

    2012-01-01

    目的:观察美敏伪麻溶液治疗上呼吸道咳嗽综合征(UACS)的临床疗效、安全性.方法:93例UACS患者随机分为治疗组48例,给予美敏伪麻溶液,对照组45例,给予复方磷酸可待因溶液.观察两组患者治疗前后咳嗽症状积分、卡他症状群积分.结果:美敏伪麻溶液组临床总有效率为85.42%,复方磷酸可待因溶液组临床总有效率为77.78%,治疗效果无显著差异(P>0.05);在改善咳嗽症状方面,第7d与第14d比较差异有统计学意义(P0.05); In improving the symptoms of cough, 7 days and 14 days the difference was statistically significant (PO.05). In improving the symptoms of catarrh, 1 day, 2 days and 3 days the difference was statistically significant (P<0.05).Conclusion:Compound pseudoephedrine hydrochloride oral solution for the control card syndrome and cough syndrome was better than the compound codeine phosphate solution.

  7. United airway disease: current perspectives

    OpenAIRE

    Giavina-Bianchi P; Aun MV; Takejima P; Kalil J; Agondi RC

    2016-01-01

    Pedro Giavina-Bianchi,* Marcelo Vivolo Aun,* Priscila Takejima, Jorge Kalil, Rosana Câmara Agondi Clinical Immunology and Allergy Division, Faculty of Medicine, University of São Paulo, São Paulo, Brazil*These authors contributed equally to this work. Abstract: Upper and lower airways are considered a unified morphological and functional unit, and the connection existing between them has been observed for many years, both in health and in disease. There is str...

  8. Mitochondrial dysfunction in heart failure.

    Science.gov (United States)

    Rosca, Mariana G; Hoppel, Charles L

    2013-09-01

    Heart failure (HF) is a complex chronic clinical syndrome. Energy deficit is considered to be a key contributor to the development of both cardiac and skeletal myopathy. In HF, several components of cardiac and skeletal muscle bioenergetics are altered, such as oxygen availability, substrate oxidation, mitochondrial ATP production, and ATP transfer to the contractile apparatus via the creatine kinase shuttle. This review focuses on alterations in mitochondrial biogenesis and respirasome organization, substrate oxidation coupled with ATP synthesis in the context of their contribution to the chronic energy deficit, and mechanical dysfunction of the cardiac and skeletal muscle in HF. We conclude that HF is associated with decreased mitochondrial biogenesis and function in both heart and skeletal muscle, supporting the concept of a systemic mitochondrial cytopathy. The sites of mitochondrial defects are located within the electron transport and phosphorylation apparatus and differ with the etiology and progression of HF in the two mitochondrial populations (subsarcolemmal and interfibrillar) of cardiac and skeletal muscle. The roles of adrenergic stimulation, the renin-angiotensin system, and cytokines are evaluated as factors responsible for the systemic energy deficit. We propose a cyclic AMP-mediated mechanism by which increased adrenergic stimulation contributes to the mitochondrial dysfunction.

  9. Cardiorenal Syndromes and Sepsis

    Directory of Open Access Journals (Sweden)

    C. Chelazzi

    2011-01-01

    Full Text Available The cardiorenal syndrome is a clinical and pathophysiological entity defined as the concomitant presence of renal and cardiovascular dysfunction. In patients with severe sepsis and septic shock, acute cardiovascular, and renal derangements are common, that is, the septic cardiorenal syndrome. The aim of this paper is to describe the pathophysiology and clinical features of septic cardiorenal syndrome in light of the actual clinical and experimental evidence. In particular, the importance of systemic and intrarenal endothelial dysfunction, alterations of kidney perfusion, and myocardial function, organ “crosstalk” and ubiquitous inflammatory injury have been extensively reviewed in light of their role in cardiorenal syndrome etiology. Treatment includes early and targeted optimization of hemodynamics to reverse systemic hypotension and restore urinary output. In case of persistent renal impairment, renal replacement therapy may be used to remove cytokines and restore renal function.

  10. Cardio Renal Syndrome

    Directory of Open Access Journals (Sweden)

    KV Sahasranam

    2014-10-01

    Full Text Available For a long time, physicians have recognized that the kidney and the heart are related especially when there is severe dysfunction of either of them. Dysfunction of one of these organs seldom occurs in isolation. Of late the cardio renal syndrome is assuming significance because of its increasing incidence, awareness and complications. There is no definite definition of the cardio renal syndrome. However, an attempted definition states that it is a "decline in renal function in the setting of advanced heart failure". This definition does not cover the whole gamut of the cardio renal syndrome. Cardiac diseases are associated independently with a decrease in renal function and progression of existing renal disease. Chronic Kidney disease (CKD is an independent risk factor for cardiovascular events and outcome. This bidirectional nature of cardiac and renal interaction is called Cardio Renal Syndrome (CRS.

  11. Airway injury during emergency transcutaneous airway access: a comparison at cricothyroid and tracheal sites.

    LENUS (Irish Health Repository)

    Salah, Nazar

    2009-12-01

    Oxygenation via the cricothyroid membrane (CTM) may be required in emergencies, but inadvertent tracheal cannulation may occur. In this study, we compared airway injury between the tracheal and CTM sites using different techniques for airway access.

  12. [Diagnostic dyspraxia and frontal syndrome].

    Science.gov (United States)

    Donnet, A; Schmitt, A; Poncet, M

    1998-05-01

    A 27-year-old ambidexter woman experienced a clinical and psychometric frontal syndrome associated with a partial callosal syndrome following transcallosal surgery for an intraventricular neurocytoma. She also complained of difficulties with her left hand which realized a particular form of diagnostic dyspraxia: there were specific features of an isolated dysfunction of the control of the realization of a program. PMID:9773062

  13. The refeeding syndrome and hypophosphatemia.

    Science.gov (United States)

    Marinella, Mark A

    2003-09-01

    The refeeding syndrome is an underappreciated entity characterized by acute electrolyte derangements--notably hypophosphatemia--that occur during nutritional repletion of patients with significant suboptimal caloric intake. Adverse effects of hypophosphatemia include cardiac failure, muscle weakness, immune dysfunction, and death. Hypokalemia and hypomagnesemia commonly complicate refeeding syndrome as well; however, this report briefly reviews the clinical manifestations of refeeding-induced hypophosphatemia.

  14. Nasal and bronchial airway reactivity in allergic and non allergic airway inflammation

    OpenAIRE

    Kölbeck, Karl-Gustav

    2003-01-01

    In allergic or asthmatic airways disease, upper and lower airways show a uniform eosinophilic inflammation of the mucosa, and bronchial hyperreactivity is a common finding. To study the co- variation of mucosal reactivity in upper and lower airways, histamine challenges of both sites were performed in a group of patients with allergic rhinitis during non-season. Upper airways were monitored during challenge by the use of rhinostereometry, an optical technique that non-invasi...

  15. The Three A’s in Asthma – Airway Smooth Muscle, Airway Remodeling & Angiogenesis

    OpenAIRE

    Keglowich, L F; Borger, P

    2015-01-01

    Asthma affects more than 300 million people worldwide and its prevalence is still rising. Acute asthma attacks are characterized by severe symptoms such as breathlessness, wheezing, tightness of the chest, and coughing, which may lead to hospitalization or death. Besides the acute symptoms, asthma is characterized by persistent airway inflammation and airway wall remodeling. The term airway wall remodeling summarizes the structural changes in the airway wall: epithelial cell shedding, goblet ...

  16. The three A's in asthma - airway smooth muscle, airway remodeling & angiogenesis

    OpenAIRE

    Keglowich, L F; Borger, P

    2015-01-01

    Asthma affects more than 300 million people worldwide and its prevalence is still rising. Acute asthma attacks are characterized by severe symptoms such as breathlessness, wheezing, tightness of the chest, and coughing, which may lead to hospitalization or death. Besides the acute symptoms, asthma is characterized by persistent airway inflammation and airway wall remodeling. The term airway wall remodeling summarizes the structural changes in the airway wall: epithelial cell shedding, goblet ...

  17. Rett Syndrome

    OpenAIRE

    Sitholey, Prabhat; Agarwal, Vivek; Srivastava, Rohit

    2012-01-01

    Rett syndrome is one of the most common causes of complex disability in girls. It is characterized by early neurological regression that severely affects motor, cognitive and communication skills, by autonomic dysfunction and often a seizure disorder. It is a monogenic X-linked dominant neurodevelopmental disorder related to mutation in MECP2, which encodes the methyl-CpG-binding protein MeCP2. There are several mouse models either based on conditional knocking out of the Mecp2 gene or on a t...

  18. Griscelli syndrome.

    Science.gov (United States)

    Ariffin, H; Geikowski, A; Chin, T F; Chau, D; Arshad, A; Abu Bakar, K; Krishnan, S

    2014-08-01

    We report a case of Griscelli Syndrome (GS). Our patient initially presented with a diagnosis of haemophagocytic lymphistiocytosis (HLH). Subsequent microscopic analysis of the patient's hair follicle revealed abnormal distribution of melanosomes in the shaft, which is a hallmark for GS. Analysis of RAB27A gene in this patient revealed a homozygous mutation in exon 6, c.550C>T, p.R184X . This nonsense mutation causes premature truncation of the protein resulting in a dysfunctional RAB27A. Recognition of GS allows appropriate institution of therapy namely chemotherapy for HLH and curative haemotopoeitic stem cell transplantation. PMID:25500851

  19. Myofascial syndrome

    Directory of Open Access Journals (Sweden)

    Giancarlo Carli

    2008-12-01

    Full Text Available Myofascial pain syndrome is common cause one of musculoskeletal pain and it is characterized by trigger points (TP, limited range of motion in joints and local twitch response (LTR during mechanical stimulation of the TP. Trigger point is a hyperirritable spot in skeletal muscle that is associated with a hypersensitive palpable nodule in a taut band. The spot is tender when pressed and can give rise to characteristic referred pain, motor dysfunction and autonomic phenomena. Palpation is reliable diagnostic criterion for locating TP in patients. Treatment is based on anesthetise TP, stretch and spray, local pression and physical activity.

  20. The airway microvasculature and exercise induced asthma.

    OpenAIRE

    Anderson, S. D.; Daviskas, E

    1992-01-01

    It has been proposed that exercise induced asthma is a result of "rapid expansion of the blood volume of peribronchial plexi" (McFadden ER, Lancet 1990;335:880-3). This hypothesis proposes that the development of exercise induced asthma depends on the thermal gradient in the airways at the end of hyperpnoea. The events that result in exercise induced asthma are vasoconstriction and airway cooling followed by reactive hyperaemia. We agree that the airway microcirculation has the potential for ...