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  1. Macrolide therapy is associated with reduced mortality in acute respiratory distress syndrome (ARDS) patients

    NARCIS (Netherlands)

    Simonis, Fabienne D.; de Iudicibus, Gianfranco; Cremer, Olaf L.; Ong, David S.Y.; van der Poll, Tom; Bos, Lieuwe D.; Schultz, Marcus J.

    Background: Macrolides have been associated with favorable immunological effects in various inflammatory disease states. We investigated the association between macrolide therapy and mortality in patients with the acute respiratory distress syndrome (ARDS). Methods: This was an unplanned secondary

  2. ARDS (Acute Respiratory Distress Syndrome)

    Science.gov (United States)

    ... Also known as What Is ARDS, or acute respiratory distress syndrome, is a lung condition that leads ... treat ARDS. Other Names Acute lung injury Adult respiratory distress syndrome Increased-permeability pulmonary edema Noncardiac pulmonary ...

  3. The Acute Respiratory Distress Syndrome (ARDS) in mechanically ventilated burn patients: An analysis of risk factors, clinical features, and outcomes using the Berlin ARDS definition.

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    Cartotto, Robert; Li, Zeyu; Hanna, Steven; Spano, Stefania; Wood, Donna; Chung, Karen; Camacho, Fernando

    2016-11-01

    The Berlin definition of Acute Respiratory Distress Syndrome (ARDS) has been applied to military burns resulting from combat-related trauma, but has not been widely studied among civilian burns. This study's purpose was to use the Berlin definition to determine the incidence of ARDS, and its associated respiratory morbidity, and mortality among civilian burn patients. Retrospective study of burn patients mechanically ventilated for ≥48h at an American Burn Association-verified burn center. The Berlin criteria identified patients with mild, moderate, and severe ARDS. Logistic regression was used to identify variables predictive of moderate to severe ARDS, and mortality. The outcome measures of interest were duration of mechanical ventilation and in-hospital mortality. Values are shown as the median (Q1-Q3). We included 162 subjects [24% female, age 48 (35-60), % total body surface area (TBSA) burn 28 (19-40), % body surface area (BSA) full thickness (FT) burn 13 (0-30), and 62% with inhalation injury]. The incidence of ARDS was 43%. Patients with ARDS had larger %TBSA burns [30.5 (23.1-47.0) vs. 24.8 (17.1-35), p=0.007], larger FT burns [20.5(5.4-35.5) vs. 7 (0-22.1), p=0.001], but had no significant difference in the incidence of inhalation injury (p=0.216), compared to those without ARDS. The % FT burn predicted the development of moderate to severe ARDS [OR 1.034, 95%CI (1.013-1.055), p=0.001]. ARDS developed in the 1st week after burn in 86% of cases. Worsening severity of ARDS was associated with increased days of mechanical ventilation in survivors (p=0.001), a reduction in ventilator-free days/1st 30 days in all subjects (p=0.004), and a strong indication of increased mortality (0% in mild ARDS vs. 50% in severe ARDS, unadjusted p=0.02). Neither moderate ARDS nor severe ARDS were significant predictors of death. ARDS is common among mechanically ventilated civilian burn patients, and develops early after burn. The extent of full thickness burn predicted

  4. Macrolide therapy is associated with reduced mortality in acute respiratory distress syndrome (ARDS) patients

    Science.gov (United States)

    de Iudicibus, Gianfranco; Cremer, Olaf L.; Ong, David S. Y.; van der Poll, Tom; Bos, Lieuwe D.; Schultz, Marcus J.

    2018-01-01

    Background Macrolides have been associated with favorable immunological effects in various inflammatory disease states. We investigated the association between macrolide therapy and mortality in patients with the acute respiratory distress syndrome (ARDS). Methods This was an unplanned secondary analysis of patients with ARDS within a large prospective observational study of critically ill patients in the intensive care units (ICUs) of two university-affiliated hospitals in the Netherlands. The exposure of interest was low-dose macrolide use prescribed for another reason than infection; we excluded patients who received high-dose macrolides for an infection. The primary endpoint was 30-day mortality. The association between macrolide therapy and mortality was determined in the whole cohort, as well as in a propensity score matched cohort; the association was compared between pulmonary versus non-pulmonary ARDS, and between two biological phenotypes based on plasma levels of 20 biomarkers. Results In total, 873 patients with ARDS were analyzed, of whom 158 patients (18%) received macrolide therapy during stay in ICU for a median duration of 3 (interquartile range, 1–4) days. Erythromycin was the most frequent prescribed macrolide (97%). Macrolide therapy was associated with reduced 30-day mortality in the whole cohort [22.8% vs. 31.6%; crude odds ratio (OR), 0.64 (interquartile range, 0.43–0.96), P=0.03]. The association in the propensity score matched cohort remained significant [22.8% vs. 32.9%; OR, 0.62 (interquartile range, 0.39–0.96), P=0.03]. Propensity matched associations with mortality were different in patients with non-pulmonary ARDS vs. pulmonary ARDS and also varied by biological phenotype. Conclusions These data together show that low-dose macrolide therapy prescribed for another reason than infection is associated with decreased mortality in patients with ARDS. PMID:29430441

  5. Macrolide therapy is associated with reduced mortality in acute respiratory distress syndrome (ARDS) patients.

    Science.gov (United States)

    Simonis, Fabienne D; de Iudicibus, Gianfranco; Cremer, Olaf L; Ong, David S Y; van der Poll, Tom; Bos, Lieuwe D; Schultz, Marcus J

    2018-01-01

    Macrolides have been associated with favorable immunological effects in various inflammatory disease states. We investigated the association between macrolide therapy and mortality in patients with the acute respiratory distress syndrome (ARDS). This was an unplanned secondary analysis of patients with ARDS within a large prospective observational study of critically ill patients in the intensive care units (ICUs) of two university-affiliated hospitals in the Netherlands. The exposure of interest was low-dose macrolide use prescribed for another reason than infection; we excluded patients who received high-dose macrolides for an infection. The primary endpoint was 30-day mortality. The association between macrolide therapy and mortality was determined in the whole cohort, as well as in a propensity score matched cohort; the association was compared between pulmonary versus non-pulmonary ARDS, and between two biological phenotypes based on plasma levels of 20 biomarkers. In total, 873 patients with ARDS were analyzed, of whom 158 patients (18%) received macrolide therapy during stay in ICU for a median duration of 3 (interquartile range, 1-4) days. Erythromycin was the most frequent prescribed macrolide (97%). Macrolide therapy was associated with reduced 30-day mortality in the whole cohort [22.8% vs. 31.6%; crude odds ratio (OR), 0.64 (interquartile range, 0.43-0.96), P=0.03]. The association in the propensity score matched cohort remained significant [22.8% vs. 32.9%; OR, 0.62 (interquartile range, 0.39-0.96), P=0.03]. Propensity matched associations with mortality were different in patients with non-pulmonary ARDS vs. pulmonary ARDS and also varied by biological phenotype. These data together show that low-dose macrolide therapy prescribed for another reason than infection is associated with decreased mortality in patients with ARDS.

  6. Impact and safety of open lung biopsy in patients with acute respiratory distress syndrome (ARDS).

    Science.gov (United States)

    Ortiz, G; Garay, M; Mendoza, D; Cardinal-Fernández, P

    2018-02-28

    Acute respiratory distress syndrome (ARDS) is an inflammatory lung disorder, and its pathological hallmark is diffuse alveolar damage (DAD). Given that open lung biopsy (OLB) can sometimes result in severe side effects, it is rarely performed in patients with ARDS. The aims of this study were to describe: (a) the rate of treatment change associated with the histological result; and (b) the incidence of side effects induced by OLB. A retrospective, single-center, descriptive observational study was carried out in Hospital Santa Clara (Bogotá, Colombia) from February 2007 to January 2014. Critically ill patients over 18 years of age, undergoing invasive mechanical ventilation, diagnosed with ARDS of unknown etiology, and with OLB performed at the bedside. ARDS was diagnosed according to the Berlin definition. DAD was defined by the presence of a hyaline membrane plus at least one of the following: intra-alveolar edema, alveolar type I cell necrosis, alveolar type II cell (cuboidal cells) proliferation progressively covering the denuded alveolar-capillary membrane, interstitial proliferation of fibroblasts and myofibroblasts, or organizing interstitial fibrosis. The rate of treatment change (RTC) was established according to whether the OLB pathology report resulted in: a) the prescription or discontinuation of an antimicrobial; b) the indication of new procedures; c) medical interconsultation; or d) limitation of therapeutic effort. Patients were followed-up until death or hospital discharge. This study was approved by the Ethics Committee. A total of 32 OLBs were performed during the study period; 17 were ruled out as they did not involve ARDS, and 15 were considered for further analysis. A histological diagnosis was reached in 14 of the 15 patients (12 DAD, one case of bronchiolitis obliterans organizing pneumonia and one case of Wegener's granulomatosis with alveolar hemorrhage). The RTC rate was 0.73. The most frequent intervention was discontinuation of

  7. Hypoxia Inducible Factor-2 Alpha and Prolinhydroxylase 2 Polymorphisms in Patients with Acute Respiratory Distress Syndrome (ARDS

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    Annika Dötsch

    2017-06-01

    Full Text Available Hypoxia-inducible-factor-2α (HIF-2α and HIF-2 degrading prolyl-hydroxylases (PHD are key regulators of adaptive hypoxic responses i.e., in acute respiratory distress syndrome (ARDS. Specifically, functionally active genetic variants of HIF-2α (single nucleotide polymorphism (SNP [ch2:46441523(hg18] and PHD2 (C/T; SNP rs516651 and T/C; SNP rs480902 are associated with improved adaptation to hypoxia i.e., in high-altitude residents. However, little is known about these SNPs’ prevalence in Caucasians and impact on ARDS-outcome. Thus, we tested the hypotheses that in Caucasian ARDS patients SNPs in HIF-2α or PHD2 genes are (1 common, and (2 independent risk factors for 30-day mortality. After ethics-committee approval, 272 ARDS patients were prospectively included, genotyped for PHD2 (Taqman SNP Genotyping Assay and HIF-2α-polymorphism (restriction digest + agarose-gel visualization, and genotype dependent 30-day mortality was analyzed using Kaplan-Meier-plots and multivariate Cox-regression analyses. Frequencies were 99.62% for homozygous HIF-2α CC-carriers (CG: 0.38%; GG: 0%, 2.3% for homozygous PHD2 SNP rs516651 TT-carriers (CT: 18.9%; CC: 78.8%, and 3.7% for homozygous PHD2 SNP rs480902 TT-carriers (CT: 43.9%; CC: 52.4%. PHD2 rs516651 TT-genotype in ARDS was independently associated with a 3.34 times greater mortality risk (OR 3.34, CI 1.09–10.22; p = 0.034 within 30-days, whereas the other SNPs had no significant impact (p = ns. The homozygous HIF-2α GG-genotype was not present in our Caucasian ARDS cohort; however PHD2 SNPs exist in Caucasians, and PHD2 rs516651 TT-genotype was associated with an increased 30-day mortality suggesting a relevance for adaptive responses in ARDS.

  8. Analysis of bronchoalveolar lavage fluid (Balf) from patients with adult respiratory distress syndrome (ARDS)

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    Henderson, R.F.; Baughman, R.P. [Univ. of Cincinnati College of Medicine, Cincinnati, OH (United States); Waide, J.J.

    1995-12-01

    The pathogenesis of ARDS is largely unknown, but many factors are known to predispose one to ARDS: sepsis, aspiration of gastric contents, pneumonia, fracture, multiple transfusions, cardiopulmonary bypass, burn, dissemination intravascular coagulation, pulmonary contusion, near drowning, and pancreatitis. ARDS is characterized by severe hypoxemia, diffuse pulmonary infiltrates, and decreased pulmonary compliance. Current treatment methods still result in 50% mortality. Studies are underway at the University of Cincinnati to determine if treatment with a synthetic pulmonary surfactant, Exosurf{sup {reg_sign}} (contains dipalmitoyl phosphatidyl choline, Burroughs-Wellcome), improves the prognosis of these patients. BALF from these patients, before and after treatment, was analyzed to determine if the treatment resulted in an increase in disaturated phospholipids (surfactant phospholipids) in the epithelial lining fluid and if the treatments reduced the concentration of markers of inflammation and toxicity in the BALF. This study indicates that the method of administering Exosurf{sup {reg_sign}} did not lead to an increase in surfactant lipid or protein in the bronchoalveolar region of the respiratory tract.

  9. Antisynthetase syndrome (ASS) presenting as acute respiratory distress syndrome (ARDS) in a patient without myositis features.

    Science.gov (United States)

    Kanchustambham, Venkat Kiran; Saladi, Swetha; Mahmoudassaf, Sarah; Patolia, Setu

    2016-12-09

    A woman aged 61 years presented to the emergency room with a 1-week history of dyspnoea on exertion and dry cough. X-ray of the chest showed diffuse interstitial opacities and was started on antibiotics and furosemide, and despite these measures, patient's respiratory status worsened, prompting endotracheal intubation. CT of the chest showed diffuse bilateral ground glass opacities and underwent bronchoscope with trans-bronchial biopsy that showed chronic bronchitis. Pt was empirically started on intravenous steroids due to concerns for interstitial lung disease (ILD). Autoimmune work up was sent and underwent video-assisted thoracoscopic surgery-guided biopsy of the lung that showed non-specific interstitial pattern with fibrosis. The patient was diagnosed as having antisynthetase syndrome with pulmonary involvement (ILD) as the cause of her acute respiratory failure. Azathioprine was started as steroid-sparing agent and was weaned off the ventilator to a tracheostomy collar and discharged to long-term rehabilitation centre. 2016 BMJ Publishing Group Ltd.

  10. MANAGEMENT OF A PATIENT WITH ARDS: A CASE REPORT

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    Preethi Thomas

    2014-12-01

    Full Text Available Acute Respiratory Distress Syndrome (ARDS is a permeability pulmonary edema characterized by increased permeability of pulmonary capillary endothelial cells and alveolar epithelial cells, leading to hypoxemia that is refractory to usual oxygen therapy. ARDS is characterized by a brief precipitating event followed by rapidly developing dyspnea. These patients have markedly impaired respiratory system compliance and reduced lung volume. The hypoxemia is refractory to low fraction of oxygen concentration and low positive end expiratory pressure (PEEP. The mortality of ARDS is around 35-40%. Current therapy of ARDS resolves around treatment of underlying cause, lung protective ventilatory strategy and appropriate fluid management. We present a case of ARDS managed in our ICU along with a detailed discussion about the pathophysiology and treatment modalities for the management of a patient with ARDS.

  11. A prospective international observational prevalence study on prone positioning of ARDS patients: the APRONET (ARDS Prone Position Network) study

    NARCIS (Netherlands)

    Guérin, C.; Beuret, P.; Constantin, J. M.; Bellani, G.; Garcia-Olivares, P.; Roca, O.; Meertens, J. H.; Maia, P. Azevedo; Becher, T.; Peterson, J.; Larsson, A.; Gurjar, M.; Hajjej, Z.; Kovari, F.; Assiri, A. H.; Mainas, E.; Hasan, M. S.; Morocho-Tutillo, D. R.; Baboi, L.; Chrétien, J. M.; François, G.; Ayzac, L.; Chen, L.; Brochard, L.; Mercat, A.; Hajjej, Zied; Sellami, Walid; Ferjani, M.; Gurjar, Mohan; Assiri, Amer; Al Bshabshe, Ali; Almekhlafi, Ghaleb; Mandourah, Yasser; Hasan, Mohd Shahnaz; Rai, Vineya; Marzida, M.; Corcoles Gonzalez, Virgilio; Sanchez Iniesta, Rafael; Garcia, Pablo; Garcia-Montesinos de La Peña, Manuel; Garcia Herrera, Adriana; Roca, Oriol; Garcia-de-Acilu, Marina; Masclans Enviz, Joan Ramon; Mancebo, Jordi; Heili, Sarah; Artigas Raventos, Antonio; Blanch Torra, Lluís; Roche-Campo, Ferran; Schultz, Marcus

    2018-01-01

    While prone positioning (PP) has been shown to improve patient survival in moderate to severe acute respiratory distress syndrome (ARDS) patients, the rate of application of PP in clinical practice still appears low. This study aimed to determine the prevalence of use of PP in ARDS patients (primary

  12. Effect of protective lung ventilation strategy combined with lung recruitment maneuver in patients with acute respiratory distress syndrome (ARDS

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    Sheng Yu

    2017-01-01

    Full Text Available Objective: To evaluate the efficacy and safety of protective lung ventilation strategy combined with lung recruitment maneuver (RM in the treatment patients with acute respiratory distress syndrome (ARDS.Methods: Totally 74 patients with ARDS admitted to the Department of Intensive Care Unit, Changshu Second People's Hospital in Jiangsu Province between September 2010 and June 2013 were selected and randomly divided into lung recruitment group and non-lung recruitment group, and the initial ventilation solution for both groups was synchronized intermittent mandatory ventilation (SIMV. For RM, SIMV mode (pressure control and pressure support was adopted. Positive end expiratory pressure (PEEP was increased by 5 cm H2O every time and maintained for 40-50 s before entering the next increasing period, and the peak airway pressure was kept below 45 cm H2O. After PEEP reached the maximum value, it was gradually reduced by 5 cm H2O every time and finally maintained at 15 cm H2O for 10 min.Results: A total of 74 patients with mean age of (49.0±18.6 years old were enrolled, 36 patients were enrolled in lung recruitment maneuver (RM group and 38 patients were enrolled into non-lung recruitment maneuver (non-RM group. 44 were male and accounted for 59.5% of all the patients. For the indicators such as PEEP, pressure support (PS, plateau airway pressure (Pplat, peak airway pressure (Ppeak, vital capacity (VC and fraction of inspired oxygen (FiO2, no statistical differences in the indicators were found between the RM group and non-RM group on D1, D3 and D7 (P>0.05, except that only FiO2 of RM group on D7 was significantly lower than that of non-RM group (47.2±10.0 vs. (52.2±10.5, P0.05. 28-day mortality, ICU mortality and in-hospital mortality were 25% vs. 28.9%, 25% vs. 26.3% and 36.1% vs. 39.5% respectively between RM group and non-RM group (all P>0.05.Conclusion: Protective lung ventilation strategy combined with lung recruitment maneuver can improve

  13. ARDS: challenges in patient care and frontiers in research

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    Lieuwe D. Bos

    2018-01-01

    Full Text Available This review discusses the clinical challenges associated with ventilatory support and pharmacological interventions in patients with acute respiratory distress syndrome (ARDS. In addition, it discusses current scientific challenges facing researchers when planning and performing trials of ventilatory support or pharmacological interventions in these patients. Noninvasive mechanical ventilation is used in some patients with ARDS. When intubated and mechanically ventilated, ARDS patients should be ventilated with low tidal volumes. A plateau pressure <30 cmH2O is recommended in all patients. It is suggested that a plateau pressure <15 cmH2O should be considered safe. Patient with moderate and severe ARDS should receive higher levels of positive end-expiratory pressure (PEEP. Rescue therapies include prone position and neuromuscular blocking agents. Extracorporeal support for decapneisation and oxygenation should only be considered when lung-protective ventilation is no longer possible, or in cases of refractory hypoxaemia, respectively. Tracheotomy is only recommended when prolonged mechanical ventilation is expected. Of all tested pharmacological interventions for ARDS, only treatment with steroids is considered to have benefit. Proper identification of phenotypes, known to respond differently to specific interventions, is increasingly considered important for clinical trials of interventions for ARDS. Such phenotypes could be defined based on clinical parameters, such as the arterial oxygen tension/inspiratory oxygen fraction ratio, but biological marker profiles could be more promising.

  14. Acute respiratory distress syndrome (ARDS): HRCT findings in survivors

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    Jung, Jung Im; Park, Seog Hee; Lee, Jae Mun; Song, Jeong Sup; Lee, Kyo Young

    1999-01-01

    The purpose of this report is to describe the high-resolution computed tomography (HRCT) findings of the lung in survivors of acute respiratory distress syndrome (ARDS). Among eleven patients who survived ARDS for one year, chest radiography and HRCT revealed pulmonary fibrosis in four. Causes of ARDS included pneumonia during pregnancy, near drowning, pneumonia during liver cirrhosis, and postoperative sepsis. Thoracoscopic biopsy and histopathologic correlation were available in one patient. HRCT showed diffuse interlobular septal thickening, ground glass opacity, parenchymal distortion, and traction bronchiectasis. Fuzzy centrilobular nodules were seen in two patients and one patient had multiple, large bullae in the left hemithorax. In all patients, lesions affected the upper and anterior zones of the lung more prominently. The distribution of pulmonary fibrosis was characteristic and reflected the pathogenesis of lung injury; fibrosis was largely due to hyperoxia caused by ventilator care. In one patient, histopathologic correlation showed that imaging findings were accounted for by thickening of the alveolar septum along with infiltration of chronic inflammatory cells and fibrosis. Fuzzy centrilobular nodules corresponded with bronchiolitis

  15. Acute Respiratory Distress Syndrome (ARDS After Nitric Acid Inhalation

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    Gülay Kır

    2014-12-01

    Full Text Available Lung injury resulting from inhalation of chemical products continues to be associated with high morbidity and mortality. Concentrated nitric acids are also extremely corrosive fuming chemical liquids. Fumes of nitric acid (HNO3 and various oxides of nitrogen such as nitric oxide (NO and nitrogen dioxide (NO2 may cause fatal illnesses such as severe pulmonary edema and acute respiratory distress syndrome (ARDS when inhaled. Intensive respiratory management including mechanical ventilation with positive end expiratory pressure (PEEP, inverse ratio ventilation, replacement of surfactant and extracorporeal membrane oxygenation (ECMO, steroids and n-acetylcysteine (NAC may improve survival. In this case report we present the diagnosis and successful treatment of a 57 years old male patient who developed ARDS following pulmonary edema due to nitric acid fumes inhalation.

  16. Fifty Years of Research in ARDS. Gas Exchange in Acute Respiratory Distress Syndrome.

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    Radermacher, Peter; Maggiore, Salvatore Maurizio; Mercat, Alain

    2017-10-15

    Acute respiratory distress syndrome (ARDS) is characterized by severe impairment of gas exchange. Hypoxemia is mainly due to intrapulmonary shunt, whereas increased alveolar dead space explains the alteration of CO 2 clearance. Assessment of the severity of gas exchange impairment is a requisite for the characterization of the syndrome and the evaluation of its severity. Confounding factors linked to hemodynamic status can greatly influence the relationship between the severity of lung injury and the degree of hypoxemia and/or the effects of ventilator settings on gas exchange. Apart from situations of rescue treatment, targeting optimal gas exchange in ARDS has become less of a priority compared with prevention of injury. A complex question for clinicians is to understand when improvement in oxygenation and alveolar ventilation is related to a lower degree or risk of injury for the lungs. In this regard, a full understanding of gas exchange mechanism in ARDS is imperative for individualized symptomatic support of patients with ARDS.

  17. ADULT RESPIRATORY-DISTRESS SYNDROME (ARDS) DUE TO BACTEREMIC PNEUMOCOCCAL PNEUMONIA

    NARCIS (Netherlands)

    MANNES, GPM; BOERSMA, WG; BAUR, CHJM; POSTMUS, PE

    We describe a patient, who had no pre-existing disease, with bacteraemic pneumococcal pneumonia and adult respiratory distress syndrome (ARDS), a rare complication. In spite of the use of antibiotics and intensive treatment the mortality rate of this kind of infection remains high. Streptococcus

  18. Is surfactant a promising additive drug in ALI/ARDS-patients?

    NARCIS (Netherlands)

    Schultz, MJ; Kesecioglu, J

    The rationale for surfactant replacement therapy in patients with acute respiratory distress syndrome (ARDS) is to restore the normal composition of the surfactant system, as well as to overcome ongoing inactivation of present surfactant. Indeed, surfactant replacement therapy call normalize the

  19. Is surfactant a promising additive drug in ALI/ARDS-patients?

    NARCIS (Netherlands)

    Schultz, Marcus J.; Kesecioglu, Jozef

    2004-01-01

    The rationale for surfactant replacement therapy in patients with acute respiratory distress syndrome (ARDS) is to restore the normal composition of the surfactant system, as well as to overcome ongoing inactivation of present surfactant. Indeed, surfactant replacement therapy call normalize the

  20. Aerosolized prostacyclins for acute respiratory distress syndrome (ARDS)

    DEFF Research Database (Denmark)

    Afshari, Arash; Bastholm Bille, Anders; Allingstrup, Mikkel

    2017-01-01

    BACKGROUND: Acute respiratory distress syndrome (ARDS) is a critical condition that is associated with high mortality and morbidity. Aerosolized prostacyclin has been used to improve oxygenation despite the limited evidence available so far.This review was originally published in 2010 and updated...... (very low quality of evidence), and one RCT involved 67 critically ill adults (very low quality evidence).Only one RCT (paediatric trial) provided data on mortality and found no difference between intervention and control. However, this trial was eligible for meta-analysis due to a cross-over design...... low quality evidence).There were no adverse events such as bleeding or organ dysfunction in any of the included trials. Due to the limited number of RCTs, we were unable to perform the prespecified subgroup and sensitivity analyses or trial sequential analysis. AUTHORS' CONCLUSIONS: We are unable...

  1. Human herpesviruses respiratory infections in patients with acute respiratory distress (ARDS).

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    Bonizzoli, Manuela; Arvia, Rosaria; di Valvasone, Simona; Liotta, Francesco; Zakrzewska, Krystyna; Azzi, Alberta; Peris, Adriano

    2016-08-01

    Acute respiratory distress syndrome (ARDS) is today a leading cause of hospitalization in intensive care unit (ICU). ARDS and pneumonia are closely related to critically ill patients; however, the etiologic agent is not always identified. The presence of human herpes simplex virus 1, human cytomegalovirus and Epstein-Barr virus in respiratory samples of critically ill patients is increasingly reported even without canonical immunosuppression. The main aim of this study was to better understand the significance of herpesviruses finding in lower respiratory tract of ARDS patients hospitalized in ICU. The presence of this group of herpesviruses, in addition to the research of influenza viruses and other common respiratory viruses, was investigated in respiratory samples from 54 patients hospitalized in ICU, without a known microbiological causative agent. Moreover, the immunophenotype of each patient was analyzed. Herpesviruses DNA presence in the lower respiratory tract seemed not attributable to an impaired immunophenotype, whereas a significant correlation was observed between herpesviruses positivity and influenza virus infection. A higher ICU mortality was significantly related to the presence of herpesvirus infection in the lower respiratory tract as well as to impaired immunophenotype, as patients with poor outcome showed severe lymphopenia, affecting in particular T (CD3+) cells, since the first days of ICU hospitalization. In conclusion, these results indicate that herpesviruses lower respiratory tract infection, which occurs more frequently following influenza virus infection, can be a negative prognostic marker. An independent risk factor for ICU patients with ARDS is an impaired immunophenotype.

  2. Aerosolized prostacyclin for acute lung injury (ALI) and acute respiratory distress syndrome (ARDS)

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    Afshari, Arash; Brok, Jesper; Møller, Ann

    2010-01-01

    Acute lung injury (ALI) and acute respiratory distress syndrome (ARDS) are critical conditions that are associated with high mortality and morbidity. Aerosolized prostacyclin has been used to improve oxygenation despite the limited evidence available so far.......Acute lung injury (ALI) and acute respiratory distress syndrome (ARDS) are critical conditions that are associated with high mortality and morbidity. Aerosolized prostacyclin has been used to improve oxygenation despite the limited evidence available so far....

  3. Combination therapy with sivelestat and recombinant human soluble thrombomodulin for ARDS and DIC patients

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    Miyoshi S

    2014-09-01

    Full Text Available Seigo Miyoshi,1 Ryoji Ito,1 Hitoshi Katayama,1 Kentaro Dote,2 Mayuki Aibiki,3 Hironobu Hamada,1,4 Takafumi Okura,1 Jitsuo Higaki1 1Department of Cardiology, Pulmonology, Hypertension and Nephrology, Ehime University Graduate School of Medicine, 2Intensive Care Division, Ehime University Hospital, 3Department of Emergency and Critical Care Medicine, School of Medicine, Ehime University, Shitsukawa, Toon, Ehime, 4Department of Physical Analysis and Therapeutic Sciences, Graduate School of Biomedical and Health Sciences, Hiroshima University, Kasumi, Minami-ku, Hiroshima, Japan Background: Neutrophil elastase, alveolar thrombin generation, and fibrin deposition play crucial roles in the development of acute respiratory distress syndrome (ARDS and disseminated intravascular coagulation (DIC. However, the usefulness of combination therapy with a selective neutrophil elastase inhibitor, sivelestat, and recombinant human soluble thrombomodulin (rhTM for patients with ARDS and DIC remains unknown. Methods: We conducted a retrospective data analysis of 142 ARDS patients with DIC to assess the effects of sivelestat combined with rhTM. Patients were divided into four groups: control (no sivelestat or rhTM treatment, sivelestat treatment alone, rhTM treatment alone, and combined treatment with sivelestat and rhTM. A Cox proportional hazard model was used to assess subject mortality rates. The efficacy of these drugs was evaluated based on survival rate, number of ventilator-free days, and change in PaO2/FIO2 (P/F ratios and DIC scores before and at 7 days after a diagnosis of ARDS with DIC. Results: Multivariate analysis showed that patient age, combination therapy, gas exchange, organ failure, cause, associated disease score, and serum C-reactive protein levels were predictors of mortality for patients with ARDS and DIC. As compared with untreated controls, combination therapy significantly improved the 60-day survival rate of patients with ARDS and DIC

  4. Is pulmonary resistance constant, within the range of tidal volume ventilation, in patients with ARDS?

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    Mols, G; Kessler, V; Benzing, A; Lichtwarck-Aschoff, M; Geiger, K; Guttmann, J

    2001-02-01

    When managing patients with acute respiratory distress syndrome (ARDS), respiratory system compliance is usually considered first and changes in resistance, although recognized, are neglected. Resistance can change considerably between minimum and maximum lung volume, but is generally assumed to be constant in the tidal volume range (V(T)). We measured resistance during tidal ventilation in 16 patients with ARDS or acute lung injury by the slice method and multiple linear regression analysis. Resistance was constant within V(T) in only six of 16 patients. In the remaining patients, resistance decreased, increased or showed complex changes. We conclude that resistance within V(T) varies considerably from patient to patient and that constant resistance within V(T) is not always likely.

  5. Fifty Years of Research in ARDS. Cell-based Therapy for Acute Respiratory Distress Syndrome. Biology and Potential Therapeutic Value.

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    Laffey, John G; Matthay, Michael A

    2017-08-01

    On the basis of several preclinical studies, cell-based therapy has emerged as a potential new therapeutic for acute respiratory distress syndrome (ARDS). Of the various cell-based therapy options, mesenchymal stem/stromal cells (MSCs) from bone marrow, adipose tissue, and umbilical cord have the most experimental data to support their potential efficacy for lung injury from both infectious and noninfectious causes. Mechanistically, MSCs exert their beneficial effects by release of paracrine factors, microvesicles, and transfer of mitochondria, all of which have antiinflammatory and pro-resolving effects on injured lung endothelium and alveolar epithelium, including enhancing the resolution of pulmonary edema by up-regulating sodium-dependent alveolar fluid clearance. MSCs also have antimicrobial effects mediated by release of antimicrobial factors and by up-regulating monocyte/macrophage phagocytosis. Phase 2a clinical trials to establish safety in ARDS are in progress, and two phase 1 trials did not report any serious adverse events. Several issues need further study, including: determining the optimal methods for large-scale production, reconstitution of cryopreserved cells for clinical use, defining cell potency assays, and determining the therapeutic potential of conditioned media derived from MSCs. Because ARDS is a heterogeneous syndrome, targeting MSCs to patients with ARDS with a more hyperinflammatory endotype may further enhance their potential for efficacy.

  6. Experts' opinion on management of hemodynamics in ARDS patients: focus on the effects of mechanical ventilation

    NARCIS (Netherlands)

    Vieillard-Baron, A.; Matthay, M.; Teboul, J. L.; Bein, T.; Schultz, M.; Magder, S.; Marini, J. J.

    2016-01-01

    Acute respiratory distress syndrome (ARDS) is frequently associated with hemodynamic instability which appears as the main factor associated with mortality. Shock is driven by pulmonary hypertension, deleterious effects of mechanical ventilation (MV) on right ventricular (RV) function, and

  7. Clinical characteristics and outcomes of patients with acute lung injury and ARDS

    Directory of Open Access Journals (Sweden)

    R R Bhadade

    2011-01-01

    Full Text Available Background : Acute lung injury (ALI and acute respiratory distress syndrome (ARDS are critical illnesses associated with significant morbidity and mortality. Aims : This was designed to assess various etiologies of ALI/ARDS, to determine the correlation between the diagnostic criteria and need of mechanical ventilation, and to correlate biochemical factors with the outcome of patients. Settings and Design : An observational, prospective study was conducted in a medical intensive care unit (MICU of a tertiary care hospital, for a period of 1 year. Materials and Methods : This study encompassed 58 consecutive cases of ALI/ARDS admitted to a MICU as per AECC guidelines. Patients excluded were with cardiac failure, chronic kidney diseases with fluid overload, and age below 12 years. Statistical Analysis : The data were analysed applying χ2 -test, multivariate logistic regression analysis of significance, using computer-based program SPSS. Results : There were more males (74% than females, and presentation was more common in the younger age group, with a total mortality of 57%. Factors attributable for ALI/ARDS were malaria in 16 patients (27.6%, leptospirosis in 12 (20.7%, malaria with dengue in 3 (5.2%, undiagnosed fever in 16 (27.6%, pneumonia in 8 (13.8%, urinary tract infection in 2 (3.4%, and pancreatitis in 1 (1.7% patient. Out of 41 patients with PaO 2 /FiO 2 200, 11 patients though initially managed on noninvasive ventilation (NIV subsequently required invasive ventilation, and remaining six were successfully managed on NIV. Out of 41 patients requiring mechanical ventilation, 36 had LIS >2.5, whereas only 3 out of 17 patients with LIS <2.5 required mechanical ventilation. Conclusion : Malaria, leptospirosis, and undiagnosed fever were the main etiologies followed by pneumonia, urinary tract infections, and pancreatitis. Both the PaO 2 /FiO 2 ratio and lung injury score (LIS at the time of admission were significant predictors of the

  8. Inhaled nitric oxide for acute respiratory distress syndrome (ARDS) in children and adults

    DEFF Research Database (Denmark)

    Gebistorf, Fabienne; Karam, Oliver; Wetterslev, Jørn

    2016-01-01

    BACKGROUND: Acute hypoxaemic respiratory failure (AHRF) and mostly acute respiratory distress syndrome (ARDS) are critical conditions. AHRF results from several systemic conditions and is associated with high mortality and morbidity in individuals of all ages. Inhaled nitric oxide (INO) has been...

  9. Minimization of Ventilator-Induced Lung Injury in ARDS Patients – Part I: Complex Model of Mechanically Ventilated ARDS Lungs

    Directory of Open Access Journals (Sweden)

    Glapiński Jarosław

    2017-12-01

    Full Text Available A complex model of mechanically ventilated ARDS lungs is proposed in the paper. This analogue is based on a combination of four components that describe breathing mechanics: morphology, mechanical properties of surfactant, tissue and chest wall characteristics. Physical-mathematical formulas attained from experimental data have been translated into their electrical equivalents and implemented in MultiSim software. To examine the adequacy of the forward model to the properties and behaviour of mechanically ventilated lungs in patients with ARDS symptoms, several computer simulations have been performed and reported in the paper. Inhomogeneous characteristics observed in the physical properties of ARDS lungs were mapped in a multi-lobe model and the measured outputs were compared with the data from physiological reports. In this way clinicians and scientists can obtain the knowledge on the moment of airway zone reopening/closure expressed as a function of pressure, volume or even time. In the paper, these trends were assessed for inhomogeneous distributions (proper for ARDS of surfactant properties and airway geometry in consecutive lung lobes. The proposed model enables monitoring of temporal alveolar dynamics in successive lobes as well as those occurring at a higher level of lung structure organization, i.e. in a point P0 which can be used for collection of respiratory data during indirect management of recruitment/de-recruitment processes in ARDS lungs. The complex model and synthetic data generated for various parametrization scenarios make possible prospective studies on designing an indirect mode of alveolar zone management, i.e. with

  10. Acute interstitial pneumonia (AIP): relationship to Hamman-Rich syndrome, diffuse alveolar damage (DAD), and acute respiratory distress syndrome (ARDS).

    Science.gov (United States)

    Mukhopadhyay, Sanjay; Parambil, Joseph G

    2012-10-01

    Acute interstitial pneumonia (AIP) is a term used for an idiopathic form of acute lung injury characterized clinically by acute respiratory failure with bilateral lung infiltrates and histologically by diffuse alveolar damage (DAD), a combination of findings previously known as the Hamman-Rich syndrome. This review aims to clarify the diagnostic criteria of AIP, its relationship with DAD and acute respiratory distress syndrome (ARDS), key etiologies that need to be excluded before making the diagnosis, and the salient clinical features. Cases that meet clinical and pathologic criteria for AIP overlap substantially with those that fulfill clinical criteria for ARDS. The main differences between AIP and ARDS are that AIP requires a histologic diagnosis of DAD and exclusion of known etiologies. AIP should also be distinguished from "acute exacerbation of IPF," a condition in which acute lung injury (usually DAD) supervenes on underlying usual interstitial pneumonia (UIP)/idiopathic pulmonary fibrosis (IPF). Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

  11. Preemptive antibiotic treatment based on gram staining reduced the incidence of ARDS in mechanically ventilated patients.

    Science.gov (United States)

    Matsushima, Asako; Tasaki, Osamu; Shimizu, Kentaro; Tomono, Kazunori; Ogura, Hiroshi; Shimazu, Takeshi; Sugimoto, Hisashi

    2008-08-01

    Ventilator-associated pneumonia (VAP) is one of the major complications in the intensive care unit. VAP sometimes results in acute respiratory distress syndrome (ARDS), and the associated mortality is high. We hypothesized that preemptive antibiotic therapy based on results of bedside gram staining would reduce the incidence of VAP. Patients who were endotracheally intubated in our intensive care unit for more than 72 hours were included. Patients younger than 16 years of age or patients died because of brain death were excluded. The study was divided into two periods. During the first period, we used antibiotics according to the American Thoracic Society guidelines. During the second period, antibiotics were given according to the results of bedside gram staining even before radiographic infiltrate appeared. One hundred twenty-eight patients and 133 patients were included in the first and second periods, respectively. The incidence of VAP was significantly decreased in the second period (first period, 22%; second period, 9%, p gram staining significantly reduced the incidences of VAP and ARDS without an increase in the use of antibiotics.

  12. Noninvasive Ventilation of Patients with Acute Respiratory Distress Syndrome Insights from the LUNG SAFE Study

    NARCIS (Netherlands)

    Bellani, Giacomo; Laffey, John G.; Pham, Tài; Madotto, Fabiana; Fan, Eddy; Brochard, Laurent; Esteban, Andres; Gattinoni, Luciano; Bumbasirevic, Vesna; Piquilloud, Lise; van Haren, Frank; Larsson, Anders; McAuley, Daniel F.; Bauer, Philippe R.; Arabi, Yaseen M.; Ranieri, Marco; Antonelli, Massimo; Rubenfeld, Gordon D.; Thompson, B. Taylor; Wrigge, Hermann; Slutsky, Arthur S.; Pesenti, Antonio; Rios, Fernando; Sottiaux, T.; Depuydt, p; Lora, Fredy S.; Azevedo, Luciano Cesar; Bugedo, Guillermo; Qiu, Haibo; Gonzalez, Marcos; Silesky, Juan; Cerny, Vladimir; Nielsen, Jonas; Jibaja, Manuel; Matamis, Dimitrios; Ranero, Jorge Luis; Amin, Pravin; Hashemian, S. M.; Clarkson, Kevin; Kurahashi, Kiyoyasu; Villagomez, Asisclo; Zeggwagh, Amine Ali; Heunks, Leo M.; Laake, Jon Henrik; Palo, Jose Emmanuel; do Vale Fernandes, Antero; Sandesc, Dorel; Arabi, Yaasen; Bumbasierevic, Vesna; Nin, Nicolas; Lorente, Jose A.; Abroug, Fekri; McNamee, Lia; Hurtado, Javier; Bajwa, Ed; Démpaire, Gabriel; Francois, Guy M.; Sula, Hektor; Nunci, Lordian; Cani, Alma; Zazu, Alan; Dellera, Christian; Insaurralde, Carolina S.; Alejandro, Risso V.; Daldin, Julio; Vinzio, Mauricio; Fernandez, Ruben O.; Cardonnet, Luis P.; Bettini, Lisandro R.; Bisso, Mariano Carboni; Osman, Emilio M.; Setten, Mariano G.; Lovazzano, Pablo; Alvarez, Javier; Villar, Veronica; Pozo, Norberto C.; Grubissich, Nicolas; Plotnikow, Gustavo A.; Vasquez, Daniela N.; Ilutovich, Santiago; Tiribelli, Norberto; Chena, Ariel; Pellegrini, Carlos A.; Saenz, María G.; Estenssoro, Elisa; Brizuela, Matias; Gianinetto, Hernan; Gomez, Pablo E.; Cerrato, Valeria I.; Bezzi, Marco G.; Borello, Silvina A.; Loiacono, Flavia A.; Fernandez, Adriana M.; Knowles, Serena; Reynolds, Claire; Inskip, Deborah M.; Miller, Jennene J.; Kong, Jing; Whitehead, Christina; Bihari, Shailesh; Seven, Aylin; Krstevski, Amanda; Rodgers, Helen J.; Millar, Rebecca T.; Mckenna, Toni E.; Bailey, Irene M.; Hanlon, Gabrielle C.; Aneman, Anders; Lynch, Joan M.; Azad, Raman; Neal, John; Woods, Paul W.; Roberts, Brigit L.; Kol, Mark R.; Wong, Helen S.; Riss, Katharina C.; Staudinger, Thomas; Wittebole, Xavier; Berghe, Caroline; Bulpa, Pierre A.; Dive, Alain M.; Verstraete, Rik; Lebbinck, Herve; Depuydt, Pieter; Vermassen, Joris; Meersseman, Philippe; Ceunen, Helga; Rosa, Jonas I.; Beraldo, Daniel O.; Piras, Claudio; Rampinelli, Adenilton M.; Nassar Jr, Antonio P.; Mataloun, Sergio; Moock, Marcelo; Thompson, Marlus M.; Gonçalves, Claudio H.; Antônio, Ana Carolina P.; Ascoli, Aline; Biondi, Rodrigo S.; Fontenele, Danielle C.; Nobrega, Danielle; Sales, Vanessa M.; Shindhe, Suresh; Aiman, Maizatul; Laffey, John; Beloncle, Francois; Davies, Kyle G.; Cirone, Rob; Manoharan, Venika; Ismail, Mehvish; Goligher, Ewan C.; Jassal, Mandeep; Nishikawa, Erin; Javeed, Areej; Curley, Gerard; Rittayamai, Nuttapol; Parotto, Matteo; Ferguson, Niall D.; Mehta, Sangeeta; Knoll, Jenny; Pronovost, Antoine; Canestrini, Sergio; Bruhn, Alejandro R.; Garcia, Patricio H.; Aliaga, Felipe A.; Farías, Pamela A.; Yumha, Jacob S.; Ortiz, Claudia A.; Salas, Javier E.; Saez, Alejandro A.; Vega, Luis D.; Labarca, Eduardo F.; Martinez, Felipe T.; Carreño, Nicolás G.; Lora, Pilar; Liu, Haitao; Liu, Ling; Tang, Rui; Luo, Xiaoming; An, Youzhong; Zhao, Huiying; Gao, Yan; Zhai, Zhe; Ye, Zheng L.; Wang, Wei; Li, Wenwen; Li, Qingdong; Zheng, Ruiqiang; Yu, Wenkui; Shen, Juanhong; Li, Xinyu; Yu, Tao; Wu, Ya Q.; Huang, Xiao B.; He, Zhenyang; Lu, Yuanhua; Han, Hui; Zhang, Fan; Sun, Renhua; Wang, Hua X.; Qin, Shu H.; Zhu, Bao H.; Zhao, Jun; Liu, Jian; Li, Bin; Liu, Jing L.; Zhou, Fa C.; Li, Qiong J.; Zhang, Xing Y.; Li-Xin, Zhou; Xin-Hua, Qiang; Jiang, Liangyan; Gao, Yuan N.; Zhao, Xian Y.; Li, Yuan Y.; Li, Xiao L.; Wang, Chunting; Yao, Qingchun; Yu, Rongguo; Chen, Kai; Shao, Huanzhang; Qin, Bingyu; Huang, Qing Q.; Zhu, Wei H.; Hang, Ai Y.; Hua, Ma X.; Li, Yimin; Xu, Yonghao; Di, Yu D.; Ling, Long L.; Qin, Tie H.; Wang, Shou H.; Qin, Junping; Han, Yi; Zhou, Suming; Vargas, Monica P.; Silesky Jimenez, Juan I.; González Rojas, Manuel A.; Solis-Quesada, Jaime E.; Ramirez-Alfaro, Christian M.; Máca, Jan; Sklienka, Peter; Gjedsted, Jakob; Christiansen, Aage; Villamagua, Boris G.; Llano, Miguel; Burtin, Philippe; Buzancais, Gautier; Beuret, Pascal; Pelletier, Nicolas; Mortaza, Satar; Mercat, Alain; Chelly, Jonathan; Jochmans, Sébastien; Terzi, Nicolas; Daubin, Cédric; Carteaux, Guillaume; de Prost, Nicolas; Chiche, Jean-Daniel; Daviaud, Fabrice; Pham, Tai; Fartoukh, Muriel; Barberet, Guillaume; Biehler, Jerome; Dellamonica, Jean; Doyen, Denis; Arnal, Jean-Michel; Briquet, Anais; Hraiech, Sami; Papazian, Laurent; Follin, Arnaud; Roux, Damien; Messika, Jonathan; Kalaitzis, Evangelos; Dangers, Laurence; Combes, Alain; Au, Siu-Ming; Béduneau, Gaetan; Carpentier, Dorothée; Zogheib, Elie H.; Dupont, Herve; Ricome, Sylvie; Santoli, Francesco L.; Besset, Sebastien L.; Michel, Philippe; Gelée, Bruno; Danin, Pierre-Eric; Goubaux, Bernard; Crova, Philippe J.; Phan, Nga T.; Berkelmans, Frantz; Badie, Julio C.; Tapponnier, Romain; Gally, Josette; Khebbeb, Samy; Herbrecht, Jean-Etienne; Schneider, Francis; Declercq, Pierre-Louis M.; Rigaud, Jean-Philippe; Duranteau, Jacques; Harrois, Anatole; Chabanne, Russell; Marin, Julien; Bigot, Charlene; Thibault, Sandrine; Ghazi, Mohammed; Boukhazna, Messabi; Zein, Salem Ould; Richecoeur, Jack R.; Combaux, Daniele M.; Grelon, Fabien; Le Moal, Charlene; Sauvadet, Elise P.; Robine, Adrien; Lemiale, Virginie; Reuter, Danielle; Dres, Martin; Demoule, Alexandre; Goldgran-Toledano, Dany; Baboi, Loredana; Guérin, Claude; Lohner, Ralph; Kraßler, Jens; Schäfer, Susanne; Zacharowski, Kai D.; Meybohm, Patrick; Reske, Andreas W.; Simon, Philipp; Hopf, Hans-Bernd F.; Schuetz, Michael; Baltus, Thomas; Papanikolaou, Metaxia N.; Papavasilopoulou, Theonymfi G.; Zacharas, Giannis A.; Ourailogloy, Vasilis; Mouloudi, Eleni K.; Massa, Eleni V.; Nagy, Eva O.; Stamou, Electra E.; Kiourtzieva, Ellada V.; Oikonomou, Marina A.; Avila, Luis E.; Cortez, Cesar A.; Citalán, Johanna E.; Jog, Sameer A.; Sable, Safal D.; Shah, Bhagyesh; Gurjar, Mohan; Baronia, Arvind K.; Memon, Mohammedfaruk; Muthuchellappan, Radhakrishnan; Ramesh, Venkatapura J.; Shenoy, Anitha; Unnikrishnan, Ramesh; Dixit, Subhal B.; Rhayakar, Rachana V.; Ramakrishnan, Nagarajan; Bhardwaj, Vallish K.; Mahto, Heera L.; Sagar, Sudha V.; Palaniswamy, Vijayanand; Ganesan, Deeban; Hashemian, Seyed Mohammadreza; Jamaati, Hamidreza; Heidari, Farshad; Meaney, Edel A.; Nichol, Alistair; Knapman, Karl M.; O’Croinin, Donall; Dunne, Eimhin S.; Breen, Dorothy M.; Clarkson, Kevin P.; Jaafar, Rola F.; Dwyer, Rory; Amir, Fahd; Ajetunmobi, Olaitan O.; O’Muircheartaigh, Aogan C.; Black, Colin S.; Treanor, Nuala; Collins, Daniel V.; Altaf, Wahid; Zani, Gianluca; Fusari, Maurizio; Spadaro, Savino; Volta, Carlo A.; Graziani, Romano; Brunettini, Barbara; Palmese, Salvatore; Formenti, Paolo; Umbrello, Michele; Lombardo, Andrea; Pecci, Elisabetta; Botteri, Marco; Savioli, Monica; Protti, Alessandro; Mattei, Alessia; Schiavoni, Lorenzo; Tinnirello, Andrea; Todeschini, Manuel; Giarratano, Antonino; Cortegiani, Andrea; Sher, Sara; Rossi, Anna; Antonelli, Massimo M.; Montini, Luca M.; Casalena, Paolo; Scafetti, Sergio; Panarello, Giovanna; Occhipinti, Giovanna; Patroniti, Nicolò; Pozzi, Matteo; Biscione, Roberto R.; Poli, Michela M.; Raimondi, Ferdinando; Albiero, Daniela; Crapelli, Giulia; Beck, Eduardo; Pota, Vincenzo; Schiavone, Vincenzo; Molin, Alexandre; Tarantino, Fabio; Monti, Giacomo; Frati, Elena; Mirabella, Lucia; Cinnella, Gilda; Fossali, Tommaso; Colombo, Riccardo; Terragni, Pierpaolo; Pattarino, Ilaria; Mojoli, Francesco; Braschi, Antonio; Borotto, Erika E.; Cracchiolo, Andrea N.; Palma, Daniela M.; Raponi, Francesco; Foti, Giuseppe; Vascotto, Ettore R.; Coppadoro, Andrea; Brazzi, Luca; Floris, Leda; Iotti, Giorgio A.; Venti, Aaron; Yamaguchi, Osamu; Takagi, Shunsuke; Maeyama, Hiroki N.; Watanabe, Eizo; Yamaji, Yoshihiro; Shimizu, Kazuyoshi; Shiozaki, Kyoko; Futami, Satoru; Ryosuke, Sekine; Saito, Koji; Kameyama, Yoshinobu; Ueno, Keiko; Izawa, Masayo; Okuda, Nao; Suzuki, Hiroyuki; Harasawa, Tomofumi; Nasu, Michitaka; Takada, Tadaaki; Ito, Fumihito; Nunomiya, Shin; Koyama, Kansuke; Abe, Toshikazu; Andoh, Kohkichi; Kusumoto, Kohei; Hirata, Akira; Takaba, Akihiro; Kimura, Hiroyasu; Matsumoto, Shuhei; Higashijima, Ushio; Honda, Hiroyuki; Aoki, Nobumasa; Imai, Hiroshi; Ogino, Yasuaki; Mizuguchi, Ichiko; Ichikado, Kazuya; Nitta, Kenichi; Mochizuki, Katsunori; Hashida, Tomoaki; Tanaka, Hiroyuki; Nakamura, Tomoyuki; Niimi, Daisuke; Ueda, Takeshi; Kashiwa, Yozo; Uchiyama, Akinori; Sabelnikovs, Olegs; Oss, Peteris; Haddad, Youssef; Liew, Kong Y.; Ñamendys-Silva, Silvio A.; Jarquin-Badiola, Yves D.; Sanchez-Hurtado, Luis A.; Gomez-Flores, Saira S.; Marin, Maria C.; Villagomez, Asisclo J.; Lemus, Jordana S.; Fierro, Jonathan M.; Cervantes, Mavy Ramirez; Flores Mejia, Francisco Javier; Dector, Dulce; Dector, Dulce M.; Gonzalez, Daniel R.; Estrella, Claudia R.; Sanchez-Medina, Jorge R.; Ramirez-Gutierrez, Alvaro; George, Fernando G.; Aguirre, Janet S.; Buensuseso, Juan A.; Poblano, Manuel; Dendane, Tarek; Balkhi, Hicham; Elkhayari, Mina; Samkaoui, Nacer; Ezzouine, Hanane; Benslama, Abdellatif; Amor, Mourad; Maazouzi, Wajdi; Cimic, Nedim; Beck, Oliver; Bruns, Monique M.; Schouten, Jeroen A.; Rinia, Myra; Raaijmakers, Monique; van Wezel, Hellen M.; Heines, Serge J.; Strauch, Ulrich; Buise, Marc P.; Simonis, Fabienne D.; Schultz, Marcus J.; Goodson, Jennifer C.; Browne, Troy S.; Navarra, Leanlove; Hunt, Anna; Hutchison, Robyn A.; Bailey, Mathew B.; Newby, Lynette; Mcarthur, Colin; Kalkoff, Michael; Mcleod, Alex; Casement, Jonathan; Hacking, Danielle J.; Andersen, Finn H.; Dolva, Merete S.; Laake, Jon H.; Barratt-Due, Andreas; Noremark, Kim Andre L.; Søreide, Eldar; Sjøbø, Brit Å; Guttormsen, Anne B.; Yoshido, Hector H. Leon; Aguilar, Ronald Zumaran; Oscanoa, Fredy A. Montes; Alisasis, Alain U.; Robles, Joanne B.; Pasanting-Lim, Rossini Abbie B.; Tan, Beatriz C.; Andruszkiewicz, Pawel; Jakubowska, Karina; Coxo, Cristina M.; Alvarez, António M.; Oliveira, Bruno S.; Montanha, Gustavo M.; Barros, Nelson C.; Pereira, Carlos S.; Messias, António M.; Monteiro, Jorge M.; Araujo, Ana M.; Catorze, Nuno T.; Marum, Susan M.; Bouw, Maria J.; Gomes, Rui M.; Brito, Vania A.; Castro, Silvia; Estilita, Joana M.; Barros, Filipa M.; Serra, Isabel M.; Martinho, Aurelia M.; Tomescu, Dana R.; Marcu, Alexandra; Bedreag, Ovidiu H.; Papurica, Marius; Corneci, Dan E.; Negoita, Silvius Ioan; Grigoriev, Evgeny; Gritsan, Alexey I.; Gazenkampf, Andrey A.; Almekhlafi, Ghaleb; Albarrak, Mohamad M.; Mustafa, Ghanem M.; Maghrabi, Khalid A.; Salahuddin, Nawal; Aisa, Tharwat M.; Al Jabbary, Ahmed S.; Tabhan, Edgardo; Trinidad, Olivia A.; Al Dorzi, Hasan M.; Tabhan, Edgardo E.; Bolon, Stefan; Smith, Oliver; Mancebo, Jordi; Lopez-Delgado, Juan C.; Esteve, Francisco; Rialp, Gemma; Forteza, Catalina; de Haro, Candelaria; Artigas, Antonio; Albaiceta, Guillermo M.; de Cima-Iglesias, Sara; Seoane-Quiroga, Leticia; Ruiz-Aguilar, Antonio L.; Claraco-Vega, Luis M.; Soler, Juan Alfonso; Lorente, Maria del Carmen; Hermosa, Cecilia; Gordo, Federico; Prieto-González, Miryam; López-Messa, Juan B.; Perez, Manuel P.; Perez, Cesar P.; Allue, Raquel Montoiro; Roche-Campo, Ferran; Ibañez-Santacruz, Marcos; Temprano, Susana; Pintado, Maria C.; de Pablo, Raul; Gómez, Pilar Ricart Aroa; Rodriguez Ruiz, Silvia; Iglesias Moles, Silvia; Jurado, Mª Teresa; Arizmendi, Alfons; Piacentini, Enrique A.; Franco, Nieves; Honrubia, Teresa; Perez Cheng, Meisy; Perez Losada, Elena; Blanco, Javier; Yuste, Luis J.; Carbayo-Gorriz, Cecilia; Cazorla-Barranquero, Francisca G.; Alonso, Javier G.; Alda, Rosa S.; Algaba, Ángela; Navarro, Gonzalo; Cereijo, Enrique; Diaz-Rodriguez, Esther; Pastor Marcos, Diego; Alvarez Montero, Laura; Herrera Para, Luis; Jimenez Sanchez, Roberto; Blasco Navalpotro, Miguel Angel; Diaz Abad, Ricardo; Castro, Alejandro G.; Jose D Artiga, Maria; Ceniceros-Barros, Alexandra; Montiel González, Raquel; Parrilla Toribio, Dácil; Penuelas, Oscar; Roser, Tomas P.; Olga, Moreno F.; Gallego Curto, Elena; Manzano Sánchez, Rocío; Imma, Vallverdu P.; Elisabet, Garcia M.; Claverias, Laura; Magret, Monica; Pellicer, Ana M.; Rodriguez, Lucia L.; Sánchez-Ballesteros, Jesús; González-Salamanca, Ángela; Jimenez, Antonio G.; Huerta, Francisco P.; Sotillo Diaz, Juan Carlos J.; Bermejo Lopez, Esther; Llinares Moya, David D.; Tallet Alfonso, Alec A.; Eugenio Luis, Palazon Sanchez; Sanchez Cesar, Palazon; Rafael, Sánchez I.; Virgilio, Corcoles G.; Recio, Noelia N.; Adamsson, Richard O.; Rylander, Christian C.; Holzgraefe, Bernhard; Broman, Lars M.; Wessbergh, Joanna; Persson, Linnea; Schiöler, Fredrik; Kedelv, Hans; Oscarsson Tibblin, Anna; Appelberg, Henrik; Hedlund, Lars; Helleberg, Johan; Eriksson, Karin E.; Glietsch, Rita; Larsson, Niklas; Nygren, Ingela; Nunes, Silvia L.; Morin, Anna-Karin; Kander, Thomas; Adolfsson, Anne; Zender, Hervé O.; Leemann-Refondini, Corinne; Elatrous, Souheil; Bouchoucha, Slaheddine; Chouchene, Imed; Ouanes, Islem; Souissi, Asma Ben; Kamoun, Salma; Demirkiran, Oktay; Aker, Mustafa; Erbabacan, Emre; Ceylan, Ilkay; Girgin, Nermin Kelebek; Ozcelik, Menekse; Ünal, Necmettin; Meco, Basak Ceyda; Akyol, Onat O.; Derman, Suleyman S.; Kennedy, Barry; Parhar, Ken; Srinivasa, Latha; McAuley, Danny; Hopkins, Phil; Mellis, Clare; Kakar, Vivek; Hadfield, Dan; Vercueil, Andre; Bhowmick, Kaushik; Humphreys, Sally K.; Ferguson, Andrew; Mckee, Raymond; Raj, Ashok S.; Fawkes, Danielle A.; Watt, Philip; Twohey, Linda; Jha, Rajeev R.; Thomas, Matthew; Morton, Alex; Kadaba, Varsha; Smith, Mark J.; Hormis, Anil P.; Kannan, Santhana G.; Namih, Miriam; Reschreiter, Henrik; Camsooksai, Julie; Kumar, Alek; Rugonfalvi, Szabolcs; Nutt, Christopher; Oneill, Orla; Seasman, Colette; Dempsey, Ged; Scott, Christopher J.; Ellis, Helen E.; McKechnie, Stuart; Hutton, Paula J.; Di Tomasso, Nora N.; Vitale, Michela N.; Griffin, Ruth O.; Dean, Michael N.; Cranshaw, Julius H.; Willett, Emma L.; Ioannou, Nicholas; Gillis, Sarah; Csabi, Peter; Macfadyen, Rosaleen; Dawson, Heidi; Preez, Pieter D.; Williams, Alexandra J.; Boyd, Owen; Ortiz-Ruiz de Gordoa, Laura; Bramall, Jon; Symmonds, Sophie; Chau, Simon K.; Wenham, Tim; Szakmany, Tamas; Toth-Tarsoly, Piroska; Mccalman, Katie H.; Alexander, Peter; Stephenson, Lorraine; Collyer, Thomas; Chapman, Rhiannon; Cooper, Raphael; Allan, Russell M.; Sim, Malcolm; Wrathall, David W.; Irvine, Donald A.; Zantua, Kim S.; Adams, John C.; Burtenshaw, Andrew J.; Sellors, Gareth P.; Welters, Ingeborg D.; Williams, Karen E.; Hessell, Robert J.; Oldroyd, Matthew G.; Battle, Ceri E.; Pillai, Suresh; Kajtor, Istvan; Sivashanmugavel, Mageswaran; Okane, Sinead C.; Donnelly, Adrian; Frigyik, Aniko D.; Careless, Jon P.; May, Martin M.; Stewart, Richard; Trinder, T. John; Hagan, Samantha J.; Wise, Matt P.; Cole, Jade M.; MacFie, Caroline C.; Dowling, Anna T.; Nin, Nicolás; Nuñez, Edgardo; Pittini, Gustavo; Rodriguez, Ruben; Imperio, María C.; Santos, Cristina; França, Ana G.; Ebeid, Alejandro; Deicas, Alberto; Serra, Carolina; Uppalapati, Aditya; Kamel, Ghassan; Banner-Goodspeed, Valerie M.; Beitler, Jeremy R.; Reddy Mukkera, Satyanarayana; Kulkarni, Shreedhar; Lee, Jarone; Mesar, Tomaz; Shinn Iii, John O.; Gomaa, Dina; Tainter, Christopher; Yeatts, Dale J.; Warren, Jessica; Lanspa, Michael J.; Miller, Russel R.; Grissom, Colin K.; Brown, Samuel M.; Gosselin, Ryan J.; Kitch, Barrett T.; Cohen, Jason E.; Beegle, Scott H.; Gueret, Renaud M.; Tulaimat, Aiman; Choudry, Shazia; Stigler, William; Batra, Hitesh; Huff, Nidhi G.; Lamb, Keith D.; Oetting, Trevor W.; Mohr, Nicholas M.; Judy, Claine; Saito, Shigeki; Kheir, Fayez M.; Kheir, Fayez; Schlichting, Adam B.; Delsing, Angela; Crouch, Daniel R.; Elmasri, Mary; Ismail, Dina; Dreyer, Kyle R.; Blakeman, Thomas C.; Baron, Rebecca M.; Quintana Grijalba, Carolina; Hou, Peter C.; Seethala, Raghu; Aisiku, Imo; Henderson, Galen; Frendl, Gyorgy; Hou, Sen-Kuang; Owens, Robert L.; Schomer, Ashley; Jovanovic, Bojan; Surbatovic, Maja; Veljovic, Milic

    2017-01-01

    Rationale: Noninvasive ventilation (NIV) is increasingly used in patients with acute respiratory distress syndrome (ARDS). The evidence supporting NIV use in patients with ARDS remains relatively sparse. Objectives: To determine whether, during NIV, the categorization of ARDS severity based on the

  13. Associations between positive end-expiratory pressure and outcome of patients without ARDS at onset of ventilation: a systematic review and meta-analysis of randomized controlled trials

    NARCIS (Netherlands)

    Serpa Neto, Ary; Filho, Roberto Rabello; Cherpanath, Thomas; Determann, Rogier; Dongelmans, Dave A.; Paulus, Frederique; Tuinman, Pieter Roel; Pelosi, Paolo; de Abreu, Marcelo Gama; Schultz, Marcus J.

    2016-01-01

    The aim of this investigation was to compare ventilation at different levels of positive end-expiratory pressure (PEEP) with regard to clinical important outcomes of intensive care unit (ICU) patients without acute respiratory distress syndrome (ARDS) at onset of ventilation. Meta-analysis of

  14. Epidemiology, Patterns of Care, and Mortality for Patients With Acute Respiratory Distress Syndrome in Intensive Care Units in 50 Countries

    NARCIS (Netherlands)

    Bellani, Giacomo; Laffey, John G.; Pham, Tài; Fan, Eddy; Brochard, Laurent; Esteban, Andres; Gattinoni, Luciano; van Haren, Frank; Larsson, Anders; McAuley, Daniel F.; Ranieri, Marco; Rubenfeld, Gordon; Thompson, B. Taylor; Wrigge, Hermann; Slutsky, Arthur S.; Pesenti, Antonio; Francois, Guy M.; Rabboni, Francesca; Madotto, Fabiana; Conti, Sara; Sula, Hektor; Nunci, Lordian; Cani, Alma; Zazu, Alan; Dellera, Christian; Insaurralde, Carolina S.; Alejandro, Risso V.; Daldin, Julio; Vinzio, Mauricio; Fernandez, Ruben O.; Cardonnet, Luis P.; Bettini, Lisandro R.; Bisso, Mariano Carboni; Osman, Emilio M.; Setten, Mariano G.; Lovazzano, Pablo; Alvarez, Javier; Villar, Veronica; Pozo, Norberto C.; Grubissich, Nicolas; Plotnikow, Gustavo A.; Vasquez, Daniela N.; Ilutovich, Santiago; Tiribelli, Norberto; Chena, Ariel; Pellegrini, Carlos A.; Saenz, María G.; Estenssoro, Elisa; Simonis, Fabienne D.; Schultz, Marcus J.

    2016-01-01

    IMPORTANCE Limited information exists about the epidemiology, recognition, management, and outcomes of patients with the acute respiratory distress syndrome (ARDS). OBJECTIVES To evaluate intensive care unit (ICU) incidence and outcome of ARDS and to assess clinician recognition, ventilation

  15. Early alveolar and systemic mediator release in patients at different risks for ARDS after multiple trauma.

    Science.gov (United States)

    Raymondos, Konstantinos; Martin, Michael U; Schmudlach, Tanja; Baus, Stefan; Weilbach, Christian; Welte, Tobias; Krettek, Christian; Frink, Michael; Hildebrand, Frank

    2012-02-01

    Alveolar IL-8 has been reported to early identify patients at-risk to develop ARDS. However, it remains unknown how alveolar IL-8 is related to pulmonary and systemic inflammation in patients predisposed for ARDS. We studied 24 patients 2-6h after multiple trauma. Patients with IL-8 >200 pg/ml in bronchoalveolar lavage (BAL) were assigned to the group at high risk for ARDS (H, n = 8) and patients with BAL IL-8 mediators. The enhanced alveolar and systemic inflammation associated with alveolar IL-8 release should be considered to identify high-risk patients for pulmonary complications after multiple trauma to adjust surgical and other treatment strategies to the individual risk profile. Copyright © 2011 Elsevier Ltd. All rights reserved.

  16. Perioperative ARDS and lung injury: for anaesthesia and beyond

    African Journals Online (AJOL)

    Acute respiratory distress syndrome (ARDS), a life- ... failure or fluid overload, and an objective assessment .... Initial experimental work has confirmed that ... positive net fluid balance – independent of ventilator set- .... intravenous KGF in patients with ARDS, and the immunomodulating role of mesenchymal stem cells88.

  17. Inhaled nitric oxide for acute respiratory distress syndrome (ARDS) and acute lung injury in children and adults

    DEFF Research Database (Denmark)

    Afshari, Arash; Brok, Jesper; Møller, Ann

    2010-01-01

    Acute hypoxaemic respiratory failure (AHRF), defined as acute lung injury (ALI) and acute respiratory distress syndrome (ARDS), are critical conditions. AHRF results from a number of systemic conditions and is associated with high mortality and morbidity in all ages. Inhaled nitric oxide (INO) has...

  18. Gardening can induce pulmonary failure: Aspergillus ARDS in an immunocompetent patient, a case report.

    Science.gov (United States)

    Jung, Nina; Mronga, Silke; Schroth, Susanne; Vassiliou, Timon; Sommer, Frank; Walthers, Eduard; Aepinus, Christian; Jerrentrup, Andreas; Vogelmeier, Claus; Holland, Angelique; Koczulla, Rembert

    2014-11-26

    Acute Aspergillus fumigatus infection in immunocompetent patients is rare. This is the first known case of a patient who survived Aspergillus sepsis after being treated early with veno-venous extracorporeal membrane (ECMO) and antifungal therapy. An immunocompetent 54-year-old woman was exposed to plant mulch during gardening and subsequently developed pulmonary failure that progressed to sepsis with multiorgan failure. Owing to her severe clinical condition, she was treated for acute respiratory distress syndrome (ARDS) with veno-venous ECMO. Empiric antifungal therapy comprising voriconazole was also initiated owing to her history and a previous case report of aspergillosis after plant mulch exposure, though there was no microbiological proof at the time. A. fumigatus was later cultured and detected on antibody testing. The patient recovered, and ECMO was discontinued 1 week later. After 7 days of antifungal treatment, Aspergillus antibodies were undetectable. In cases of sepsis that occur after gardening, clinicians should consider Aspergillus inhalation as an aetiology, and early antimycotic therapy is recommended.

  19. The association between fluid balance and mortality in patients with ARDS was modified by serum potassium levels: a retrospective study

    Directory of Open Access Journals (Sweden)

    Zhongheng Zhang

    2015-02-01

    Full Text Available Background and Objective. Acute respiratory distress syndrome (ARDS is characterized by pulmonary edema and may benefit from conservative fluid management. However, conflicting results exist in the literature. The study aimed to investigate the association between mean fluid balance and mortality outcome in ARDS patients who required invasive mechanical ventilation.Methods. The study was a secondary analysis of a prospectively collected dataset obtained from the NHLBI Biologic Specimen and Data Repository Information Coordinating Center. ARDS patients with invasive mechanical ventilation were eligible. Demographic and laboratory data were extracted from the dataset. Multivariable regression model was built by stepwise selection of covariates. A fractional polynomial approach was used to test the linearity of mean fluid balance in the model. The potential interactions of mean fluid balance with other variables were tested.Main Results. A total of 282 patients were eligible for the analysis, including 61 non-survivors with a mortality rate of 21.6%. After stepwise regression analysis, mean fluid balance remained to be an independent predictor of death (OR: 1.00057; 95% CI [1.00034–1.00080]. The two-term model obtained using fractional polynomial analysis was not superior to the linear model. There was significant interaction between mean fluid balance and serum potassium levels (p = 0.011. While the risk of death increased with increasing mean fluid balance at potassium levels of 1.9, 2.9 , 3.9 and 4.9 mmol/l, the risk decreased at potassium level of 5.9 mmol/l.Conclusion. The present study demonstrates that more positive fluid balance in the first 8 days is significantly associated with increased risk of death. However, the relationship between mean fluid balance and mortality can be modified by serum potassium levels. With hyperkalemia, more positive fluid balance is associated with reduced risk of death.

  20. Interaction between peri-operative blood transfusion, tidal volume, airway pressure and postoperative ARDS: an individual patient data meta-analysis.

    Science.gov (United States)

    Serpa Neto, Ary; Juffermans, Nicole P; Hemmes, Sabrine N T; Barbas, Carmen S V; Beiderlinden, Martin; Biehl, Michelle; Fernandez-Bustamante, Ana; Futier, Emmanuel; Gajic, Ognjen; Jaber, Samir; Kozian, Alf; Licker, Marc; Lin, Wen-Qian; Memtsoudis, Stavros G; Miranda, Dinis Reis; Moine, Pierre; Paparella, Domenico; Ranieri, Marco; Scavonetto, Federica; Schilling, Thomas; Selmo, Gabriele; Severgnini, Paolo; Sprung, Juraj; Sundar, Sugantha; Talmor, Daniel; Treschan, Tanja; Unzueta, Carmen; Weingarten, Toby N; Wolthuis, Esther K; Wrigge, Hermann; de Abreu, Marcelo Gama; Pelosi, Paolo; Schultz, Marcus J

    2018-01-01

    Transfusion of blood products and mechanical ventilation with injurious settings are considered risk factors for postoperative lung injury in surgical Patients. A systematic review and individual patient data meta-analysis was done to determine the independent effects of peri-operative transfusion of blood products, intra-operative tidal volume and airway pressure in adult patients undergoing mechanical ventilation for general surgery, as well as their interactions on the occurrence of postoperative acute respiratory distress syndrome (ARDS). Observational studies and randomized trials were identified by a systematic search of MEDLINE, CINAHL, Web of Science, and CENTRAL and screened for inclusion into a meta-analysis. Individual patient data were obtained from the corresponding authors. Patients were stratified according to whether they received transfusion in the peri-operative period [red blood cell concentrates (RBC) and/or fresh frozen plasma (FFP)], tidal volume size [≤7 mL/kg predicted body weight (PBW), 7-10 and >10 mL/kg PBW] and airway pressure level used during surgery (≤15, 15-20 and >20 cmH 2 O). The primary outcome was development of postoperative ARDS. Seventeen investigations were included (3,659 patients). Postoperative ARDS occurred in 40 (7.2%) patients who received at least one blood product compared to 40 patients (2.5%) who did not [adjusted hazard ratio (HR), 2.32; 95% confidence interval (CI), 1.25-4.33; P=0.008]. Incidence of postoperative ARDS was highest in patients ventilated with tidal volumes of >10 mL/kg PBW and having airway pressures of >20 cmH 2 O receiving both RBC and FFP, and lowest in patients ventilated with tidal volume of ≤7 mL/kg PBW and having airway pressures of ≤15 cmH 2 O with no transfusion. There was a significant interaction between transfusion and airway pressure level (P=0.002) on the risk of postoperative ARDS. Peri-operative transfusion of blood products is associated with an increased risk of

  1. Construction and management of ARDS/sepsis registry with REDCap.

    Science.gov (United States)

    Pang, Xiaoqing; Kozlowski, Natascha; Wu, Sulong; Jiang, Mei; Huang, Yongbo; Mao, Pu; Liu, Xiaoqing; He, Weiqun; Huang, Chaoyi; Li, Yimin; Zhang, Haibo

    2014-09-01

    The study aimed to construct and manage an acute respiratory distress syndrome (ARDS)/sepsis registry that can be used for data warehousing and clinical research. The workflow methodology and software solution of research electronic data capture (REDCap) was used to construct the ARDS/sepsis registry. Clinical data from ARDS and sepsis patients registered to the intensive care unit (ICU) of our hospital formed the registry. These data were converted to the electronic case report form (eCRF) format used in REDCap by trained medical staff. Data validation, quality control, and database management were conducted to ensure data integrity. The clinical data of 67 patients registered to the ICU between June 2013 and December 2013 were analyzed. Of the 67 patients, 45 (67.2%) were classified as sepsis, 14 (20.9%) as ARDS, and eight (11.9%) as sepsis-associated ARDS. The patients' information, comprising demographic characteristics, medical history, clinical interventions, daily assessment, clinical outcome, and follow-up data, was properly managed and safely stored in the ARDS/sepsis registry. Data efficiency was guaranteed by performing data collection and data entry twice weekly and every two weeks, respectively. The ARDS/sepsis database that we constructed and manage with REDCap in the ICU can provide a solid foundation for translational research on the clinical data of interest, and a model for development of other medical registries in the future.

  2. Comparison of the Berlin definition with the American European consensus definition for acute respiratory distress syndrome in burn patients.

    Science.gov (United States)

    Bordes, Julien; Lacroix, Guillaume; Esnault, Pierre; Goutorbe, Philippe; Cotte, Jean; Dantzer, Eric; Meaudre, Eric

    2014-06-01

    Acute respiratory distress syndrome (ARDS) is a leading cause of mortality in burn patients. Smoke inhalation, pneumonia and inflammation process are the major causes of ARDS in burn patients. The American European Consensus Conference (AECC) definition proposed in 1994 has recently been revised by the Berlin definition. Our objective was to describe the epidemiology of ARDS comparing the Berlin definition with the AECC definition in a retrospective cohort of burn patients. We reviewed admitted burn adult patients for a two year period, and investigated patient who received mechanical ventilation for more than 48 h and in whom pneumonia was diagnosed. 40 patients were analyzed. According to the AECC definition, 11 patients met criteria for ALI (27.5%), and 29 patients for ARDS (72.5%). According to the Berlin definition, all patients met criteria for ARDS: 4 (10%) for a severe ARDS, 25 (62.5%) for a moderate ARDS, 11 (27.5%) for a mild ARDS. Inhalation injury was diagnosed in 10 patients (25%). Categorizing patients with the Berlin definition showed statistically significative difference of mortality within the three groups, but not with the AECC definition. The Berlin definition seems to be more accurate than the AECC definition to assess the severity of ARDS in term of outcome in burn patients. This definition may facilitate prompt recognition of ARDS in burn patients, and promote protective ventilation strategy to a larger number of patients. Copyright © 2014 Elsevier Ltd and ISBI. All rights reserved.

  3. Use of dynamic CT in acute respiratory distress syndrome (ARDS) with comparison of positive and negative pressure ventilation

    Energy Technology Data Exchange (ETDEWEB)

    Helm, Emma; Babyn, Paul [Hospital for Sick Children, Department of Diagnostic Imaging, Toronto (Canada); Talakoub, Omid; Alirezaie, Javad [Ryerson University, Department of Electrical and Computer Engineering, Toronto, ON (Canada); Grasso, Francesco; Engelberts, Doreen; Kavanagh, Brian P. [Hospital for Sick Children and the University of Toronto, Departments of Anesthesia and Critical Care Medicine and the Program in Pulmonary and Experimental Medicine, Toronto (Canada)

    2009-01-15

    Negative pressure ventilation via an external device ('iron lung') has the potential to provide better oxygenation with reduced barotrauma in patients with ARDS. This study was designed to see if oxygenation differences between positive and negative ventilation could be explained by CT. Six anaesthetized rabbits had ARDS induced by repeated saline lavage. Rabbits were ventilated with positive pressure ventilation (PPV) and negative pressure ventilation (NPV) in turn. Dynamic CT images were acquired over the respiratory cycle. A computer-aided method was used to segment the lung and calculate the range of CT densities within each slice. Volumes of ventilated lung and atelectatic lung were measured over the respiratory cycle. NPV was associated with an increased percentage of ventilated lung and decreased percentage of atelectatic lung. The most significant differences in ventilation and atelectasis were seen at mid-inspiration and mid-expiration (ventilated lung NPV=61%, ventilated lung PPV=47%, p<0.001; atelectatic lung NPV=10%, atelectatic lung PPV 19%, p<0.001). Aeration differences were not significant at end-inspiration. Dynamic CT can show differences in lung aeration between positive and negative ventilation in ARDS. These differences would not be appreciated if only static breath-hold CT was used. (orig.)

  4. The pragmatics of feeding the pediatric patient with acute respiratory distress syndrome.

    Science.gov (United States)

    Verger, Judy T; Bradshaw, Darla J; Henry, Elizabeth; Roberts, Kathryn E

    2004-09-01

    Acute respiratory distress syndrome (ARDS) represents the ultimate pulmonary response to a wide range of injuries, from septicemia to trauma. Optimal nutrition is vital to enhancing oxygen delivery, supporting adequate cardiac contractility and respiratory musculature, eliminating fluid and electrolyte imbalances, and supporting the proinflammatory response. Research is providing a better understanding of nutrients that specifically address the complex physiologic changes in ARDS. This article highlights the pathophysiology of ARDS as it relates to nutrition, relevant nutritional assessment, and important enteral and parenteral considerations for the pediatric patient who has ARDS.

  5. Assisted Ventilation in Patients with Acute Respiratory Distress Syndrome: Lung-distending Pressure and Patient-Ventilator Interaction

    NARCIS (Netherlands)

    Doorduin, J.; Sinderby, C.A.; Beck, J.; Hoeven, J.G. van der; Heunks, L.M.

    2015-01-01

    BACKGROUND: In patients with acute respiratory distress syndrome (ARDS), the use of assisted mechanical ventilation is a subject of debate. Assisted ventilation has benefits over controlled ventilation, such as preserved diaphragm function and improved oxygenation. Therefore, higher level of

  6. Cytomegalovirus reactivation and mortality in patients with acute respiratory distress syndrome

    NARCIS (Netherlands)

    Ong, David S Y; Spitoni, Cristian|info:eu-repo/dai/nl/304625957; Klein Klouwenberg, Peter M C; Verduyn Lunel, Frans M; Frencken, Jos F; Schultz, Marcus J; van der Poll, Tom; Kesecioglu, Jozef; Bonten, Marc J M; Cremer, Olaf L

    2015-01-01

    PURPOSE: Cytomegalovirus (CMV) reactivation occurs frequently in patients with the acute respiratory distress syndrome (ARDS) and has been associated with increased mortality. However, it remains unknown whether this association represents an independent risk for poor outcome. We aimed to estimate

  7. The Omega-3 Fatty Acid Docosahexaenoic Acid Modulates Inflammatory Mediator Release in Human Alveolar Cells Exposed to Bronchoalveolar Lavage Fluid of ARDS Patients

    Directory of Open Access Journals (Sweden)

    Paolo Cotogni

    2015-01-01

    Full Text Available Background. This study investigated whether the 1 : 2 ω-3/ω-6 ratio may reduce proinflammatory response in human alveolar cells (A549 exposed to an ex vivo inflammatory stimulus (bronchoalveolar lavage fluid (BALF of acute respiratory distress syndrome (ARDS patients. Methods. We exposed A549 cells to the BALF collected from 12 ARDS patients. After 18 hours, fatty acids (FA were added as docosahexaenoic acid (DHA, ω-3 and arachidonic acid (AA, ω-6 in two ratios (1 : 2 or 1 : 7. 24 hours later, in culture supernatants were evaluated cytokines (TNF-α, IL-6, IL-8, and IL-10 and prostaglandins (PGE2 and PGE3 release. The FA percentage content in A549 membrane phospholipids, content of COX-2, level of PPARγ, and NF-κB binding activity were determined. Results. The 1 : 2 DHA/AA ratio reversed the baseline predominance of ω-6 over ω-3 in the cell membranes (P < 0.001. The proinflammatory cytokine release was reduced by the 1 : 2 ratio (P < 0.01 to <0.001 but was increased by the 1 : 7 ratio (P < 0.01. The 1 : 2 ratio reduced COX-2 and PGE2 (P < 0.001 as well as NF-κB translocation into the nucleus (P < 0.01, while it increased activation of PPARγ and IL-10 release (P < 0.001. Conclusion. This study demonstrated that shifting the FA supply from ω-6 to ω-3 decreased proinflammatory mediator release in human alveolar cells exposed to BALF of ARDS patients.

  8. High-frequency oscillatory ventilation in ALI/ARDS.

    Science.gov (United States)

    Ali, Sammy; Ferguson, Niall D

    2011-07-01

    In the last 2 decades, our goals for mechanical ventilatory support in patients with acute respiratory distress syndrome (ARDS) or acute lung injury (ALI) have changed dramatically. Several randomized controlled trials have built on a substantial body of preclinical work to demonstrate that the way in which we employ mechanical ventilation has an impact on important patient outcomes. Avoiding ventilator-induced lung injury (VILI) is now a major focus when clinicians are considering which ventilatory strategy to employ in patients with ALI/ARDS. Physicians are searching for methods that may further limit VILI, while still achieving adequate gas exchange. Copyright © 2011 Elsevier Inc. All rights reserved.

  9. The incidence of ARDS and associated mortality in severe TBI using the Berlin definition.

    Science.gov (United States)

    Aisiku, Imoigele P; Yamal, Jose-Miguel; Doshi, Pratik; Rubin, Maria Laura; Benoit, Julia S; Hannay, Julia; Tilley, Barbara C; Gopinath, Shankar; Robertson, Claudia S

    2016-02-01

    The incidence of adult respiratory distress syndrome (ARDS) in severe traumatic brain injury (TBI) is poorly reported. Recently, a new definition for ARDS was proposed, the Berlin definition. The percentage of patients represented by TBI in the Berlin criteria study is limited. This study describes the incidence and associated mortality of ARDS in TBI patients. The study was an analysis of the safety of erythropoietin administration and transfusion threshold on the incidence of ARDS in severe TBI patients. Three reviewers independently assessed all patients enrolled in the study for acute lung injury/ARDS using the Berlin and the American-European Consensus Conference (AECC) definitions. A Cox proportional hazards model was used to assess the relationship between ARDS and mortality and 6-month Glasgow Outcome Scale (GOS) score. Two hundred patients were enrolled in the study. Of the patients, 21% (41 of 200) and 26% (52 of 200) developed ARDS using the AECC and Berlin definitions, respectively, with a median time of 3 days (interquartile range, 3) after injury. ARDS by either definition was associated with increased mortality (p = 0.04) but not with differences in functional outcome as measured by the GOS score at 6 months. Adjusted analysis using the Berlin criteria showed an increased mortality associated with ADS (p = 0.01). Severe TBI is associated with an incidence of ARDS ranging from 20% to 25%. The incidence is comparable between the Berlin and AECC definitions. ARDS is associated with increased mortality in severe TBI patients, but further studies are needed to validate these findings. Epidemiologic study, level II.

  10. Effect of extracorporeal CO2 removal on right ventricular and hemodynamic parameters in a patient with acute respiratory distress syndrome

    NARCIS (Netherlands)

    Cherpanath, Thomas G. V.; Landburg, Pearl P.; Lagrand, Wim K.; Schultz, Marcus J.; Juffermans, Nicole P.

    2016-01-01

    We present a female patient with severe acute respiratory distress syndrome (ARDS) necessitating intubation and mechanical ventilation on the intensive care unit (ICU). High ventilatory pressures were needed because of hypoxia and severe hypercapnia with respiratory acidosis, resulting in right

  11. Effect of extracorporeal CO2 removal on right ventricular and hemodynamic parameters in a patient with acute respiratory distress syndrome

    NARCIS (Netherlands)

    Cherpanath, Thomas G. V.; Landburg, Pearl P.; Lagrand, Wim K.; Schultz, Marcus J.; Juffermans, Nicole P.

    We present a female patient with severe acute respiratory distress syndrome (ARDS) necessitating intubation and mechanical ventilation on the intensive care unit (ICU). High ventilatory pressures were needed because of hypoxia and severe hypercapnia with respiratory acidosis, resulting in right

  12. Acute Respiratory Distress Syndrome in Obstetric Patients

    Directory of Open Access Journals (Sweden)

    S. V. Galushka

    2007-01-01

    Full Text Available Objective: to define the specific features of the course of acute respiratory distress syndrome (ARDS in puer-peras with a complicated postpartum period. Subjects and methods. Sixty-seven puerperas with ARDS were examined. Group 1 included 27 puerperas with postpartum ARDS; Group 2 comprised 10 puerperas who had been treated in an intensive care and died; Group 3 consisted of nonobstetric patients with ARDS of various genesis (a control group. Results. In obstetric patients, the baseline oxygenation index was significantly lower than that in the control group. However, Group 1 patients showed a rapid increase in PaO2/FiO2 on days 3—4 of treatment. In the control group, the changes occurred later — on days 5—6. The baseline alveolar-arterial oxygen difference was significantly higher in the obstetric patients than that in the controls. In Group 1, AaDpO2 drastically decreased on days 3—4, which took place in parallel with an increase in the oxygenation index. At the beginning of the study, pulmonary shunting was high in the group of survivors, deceased, and controls. In Group 1, the shunting decreased on days 3—4 whereas in the control group this index normalized later — only by days 6—7. In Group 1, compliance remained lower throughout the observation, but on day 7 there was a significant difference in this index between the deceased, survivors, and controls. Conclusion. Thus, more severe baseline pulmonary gas exchange abnormalities are observed in obstetric patients than in general surgical and traumatological patients; the oxygenation index, alveolar-arterial oxygen difference, and pulmonary shunting index more rapidly change in patients with severe obstetric disease in its favorable course than in general surgical and traumatological patients; throughout the observation, thoracopulmonary compliance was less in obstetric patients than in the controls. Key words: acute respiratory distress syndrome, puerperium.

  13. Ventilation with lower tidal volumes for critically ill patients without the acute respiratory distress syndrome: a systematic translational review and meta-analysis

    NARCIS (Netherlands)

    Serpa Neto, Ary; Nagtzaam, Liselotte; Schultz, Marcus J.

    2014-01-01

    There is convincing evidence for benefit from lung-protective mechanical ventilation with lower tidal volumes in patients with the acute respiratory distress syndrome (ARDS). It is uncertain whether this strategy benefits critically ill patients without ARDS as well. This manuscript systematically

  14. Association Between Use of Lung-Protective Ventilation With Lower Tidal Volumes and Clinical Outcomes Among Patients Without Acute Respiratory Distress Syndrome A Meta-analysis

    NARCIS (Netherlands)

    Serpa Neto, Ary; Cardoso, Sérgio Oliveira; Manetta, José Antônio; Pereira, Victor Galvão Moura; Espósito, Daniel Crepaldi; Pasqualucci, Manoela de Oliveira Prado; Damasceno, Maria Cecília Toledo; Schultz, Marcus J.

    2012-01-01

    Context Lung-protective mechanical ventilation with the use of lower tidal volumes has been found to improve outcomes of patients with acute respiratory distress syndrome (ARDS). It has been suggested that use of lower tidal volumes also benefits patients who do not have ARDS. Objective To determine

  15. Effect of driving pressure on mortality in ARDS patients during lung protective mechanical ventilation in two randomized controlled trials.

    Science.gov (United States)

    Guérin, Claude; Papazian, Laurent; Reignier, Jean; Ayzac, Louis; Loundou, Anderson; Forel, Jean-Marie

    2016-11-29

    Driving pressure (ΔPrs) across the respiratory system is suggested as the strongest predictor of hospital mortality in patients with acute respiratory distress syndrome (ARDS). We wonder whether this result is related to the range of tidal volume (V T ). Therefore, we investigated ΔPrs in two trials in which strict lung-protective mechanical ventilation was applied in ARDS. Our working hypothesis was that ΔPrs is a risk factor for mortality just like compliance (Crs) or plateau pressure (Pplat,rs) of the respiratory system. We performed secondary analysis of data from 787 ARDS patients enrolled in two independent randomized controlled trials evaluating distinct adjunctive techniques while they were ventilated as in the low V T arm of the ARDSnet trial. For this study, we used V T , positive end-expiratory pressure (PEEP), Pplat,rs, Crs, ΔPrs, and respiratory rate recorded 24 hours after randomization, and compared them between survivors and nonsurvivors at day 90. Patients were followed for 90 days after inclusion. Cox proportional hazard modeling was used for mortality at day 90. If colinearity between ΔPrs, Crs, and Pplat,rs was verified, specific Cox models were used for each of them. Both trials enrolled 805 patients of whom 787 had day-1 data available, and 533 of these survived. In the univariate analysis, ΔPrs averaged 13.7 ± 3.7 and 12.8 ± 3.7 cmH 2 O (P = 0.002) in nonsurvivors and survivors, respectively. Colinearity between ΔPrs, Crs and Pplat,rs, which was expected as these variables are mathematically coupled, was statistically significant. Hazard ratios from the Cox models for day-90 mortality were 1.05 (1.02-1.08) (P = 0.005), 1.05 (1.01-1.08) (P = 0.008) and 0.985 (0.972-0.985) (P = 0.029) for ΔPrs, Pplat,rs and Crs, respectively. PEEP and V T were not associated with death in any model. When ventilating patients with low V T , ΔPrs is a risk factor for death in ARDS patients, as is Pplat,rs or Crs. As our data

  16. Neutrophil extracellular traps contribute to the pathogenesis of acid-aspiration-induced ALI/ARDS.

    Science.gov (United States)

    Li, Haitao; Zhou, Xiaoting; Tan, Hongyi; Hu, Yongbin; Zhang, Lemeng; Liu, Shuai; Dai, Minhui; Li, Yi; Li, Qian; Mao, Zhi; Pan, Pinhua; Su, Xiaoli; Hu, Chengpin

    2018-01-05

    Acute lung injury/acute respiratory distress syndrome (ALI/ARDS) is a manifestation of systemic inflammation in the lungs, but the factors that trigger inflammation in ALI/ARDS are unclear. We hypothesized that neutrophil extracellular traps (NETs) contribute to the pathogenesis of acid aspiration-induced ALI/ARDS. Analysis of bronchial aspirates from ARDS patients showed that NETs were significantly correlated with the degree of ARDS (r = -0.5846, p = 0.0359). NETs in bronchoalveolar lavage fluid of acid-aspiration mice were significantly higher (141.6 ± 23.08) at 3 h after injury than those in the sham group (1234 ± 101.9; p = 0.003, n = 5 per group). Exogenous NETs aggravated lung injury, while alvelestat and DNase markedly attenuated the intensity of ARDS. We investigated whether NETs are involved in the severity of gastric aspiration-induced ARDS. Then, a hydrochloric acid aspiration-induced ALI murine model was used to assess whether NETs are pathogenic and whether targeting NETs is protective. Exogenous NETs were administered to mice. Alvelestat can inhibit neutrophil elastase (NE), which serves an important role in NET formation, so we investigated whether alvelestat could protect against ALI in cell and mouse models. NETs may contribute to ALI/ARDS by promoting tissue damage and systemic inflammation. Targeting NETs by alvelestat may be a potential therapeutic strategy.

  17. Pulmonary extraction of serotonin and propranolol in patients with adult respiratory distress syndrome

    International Nuclear Information System (INIS)

    Morel, D.R.; Dargent, F.; Bachmann, M.; Suter, P.M.; Junod, A.F.

    1985-01-01

    Because injury to the pulmonary vascular endothelium is associated with the development of the adult respiratory distress syndrome (ARDS), the authors assessed the metabolic function of pulmonary endothelial cells by the measurements of the first-pass pulmonary extraction of [ 14 C]serotonin and [ 3 H]propranolol in 15 patients with ARDS and 15 patients at risk for developing ARDS. Serotonin extraction ratio was lower in patients with ARDS (0.85 +/- 0.10, mean +/- SD) than in patients at risk (0.91 +/- 0.04) (p less than 0.025), and both values were significantly reduced (p less than 0.005) when compared with a control group value (0.97 +/- 0.01). The decrease in serotonin extraction was correlated with the severity of ARDS (r = -0.67) (p less than 0.001) and with pulmonary function changes over time. Propranolol extraction ratio was decreased in patients at risk (0.66 +/- 0.11) (p less than 0.005) but not in patients with ARDS (0.75 +/- 0.11), when compared with those in the control group (0.81 +/- 0.03). Low values in patients at risk were restored to normal by continuous positive airway pressure breathing. The authors conclude that pulmonary extraction of serotonin, an index of pulmonary endothelial cell function, correlates with the severity of ARDS

  18. Rapid induction of autoantibodies during ARDS and septic shock

    Directory of Open Access Journals (Sweden)

    Meduri G Umberto

    2010-10-01

    Full Text Available Abstract Background Little is known about the induction of humoral responses directed against human autoantigens during acute inflammation. We utilized a highly sensitive antibody profiling technology to study autoantibodies in patients with acute respiratory distress syndrome (ARDS and severe sepsis, conditions characterized by intensive immune activation leading to multiple organ dysfunction. Methods Using Luciferase Immunoprecipitation Systems (LIPS, a cohort of control, ARDS and sepsis patients were tested for antibodies to a panel of autoantigens. Autoantibody titers greater than the mean plus 3 SD of the 24 control samples were used to identify seropositive samples. Available longitudinal samples from different seropositive ARDS and sepsis patient samples, starting from within the first two days after admission to the intensive care, were then analyzed for changes in autoantibody over time. Results From screening patient plasma, 57% of ARDS and 46% of septic patients without ARDS demonstrated at least one statistically significant elevated autoantibody compared to the controls. Frequent high titer antibodies were detected against a spectrum of autoantigens including potassium channel regulator, gastric ATPase, glutamic decarboxylase-65 and several cytokines. Analysis of serial samples revealed that several seropositive patients had low autoantibodies at early time points that often rose precipitously and peaked between days 7-14. Further, the use of therapeutic doses of corticosteroids did not diminish the rise in autoantibody titers. In some cases, the patient autoantibody titers remained elevated through the last serum sample collected. Conclusion The rapid induction of autoantibodies in ARDS and severe sepsis suggests that ongoing systemic inflammation and associated tissue destruction mediate the break in tolerance against these self proteins.

  19. Feasibility and safety of low-flow extracorporeal CO2 removal managed with a renal replacement platform to enhance lung-protective ventilation of patients with mild-to-moderate ARDS.

    Science.gov (United States)

    Schmidt, Matthieu; Jaber, Samir; Zogheib, Elie; Godet, Thomas; Capellier, Gilles; Combes, Alain

    2018-05-10

    Extracorporeal carbon-dioxide removal (ECCO 2 R) might allow ultraprotective mechanical ventilation with lower tidal volume (VT) (ventilator-induced lung injury. This study was undertaken to assess the feasibility and safety of ECCO 2 R managed with a renal replacement therapy (RRT) platform to enable very low tidal volume ventilation of patients with mild-to-moderate acute respiratory distress syndrome (ARDS). Twenty patients with mild (n = 8) or moderate (n = 12) ARDS were included. VT was gradually lowered from 6 to 5, 4.5, and 4 ml/kg, and PEEP adjusted to reach 23 ≤ P plat  ≤ 25 cmH 2 O. Standalone ECCO 2 R (no hemofilter associated with the RRT platform) was initiated when arterial PaCO 2 increased by > 20% from its initial value. Ventilation parameters (VT, respiratory rate, PEEP), respiratory system compliance, P plat and driving pressure, arterial blood gases, and ECCO 2 R-system operational characteristics were collected during at least 24 h of very low tidal volume ventilation. Complications, day-28 mortality, need for adjuvant therapies, and data on weaning off ECCO 2 R and mechanical ventilation were also recorded. While VT was reduced from 6 to 4 ml/kg and P plat kept ventilation with moderate increase in PaCO 2 in patients with mild-to-moderate ARDS. ClinicalTrials.gov, NCT02606240. Registered on 17 November 2015.

  20. Fifty Years of Research in ARDS. Respiratory Mechanics in Acute Respiratory Distress Syndrome.

    Science.gov (United States)

    Henderson, William R; Chen, Lu; Amato, Marcelo B P; Brochard, Laurent J

    2017-10-01

    Acute respiratory distress syndrome is a multifactorial lung injury that continues to be associated with high levels of morbidity and mortality. Mechanical ventilation, although lifesaving, is associated with new iatrogenic injury. Current best practice involves the use of small Vt, low plateau and driving pressures, and high levels of positive end-expiratory pressure. Collectively, these interventions are termed "lung-protective ventilation." Recent investigations suggest that individualized measurements of pulmonary mechanical variables rather than population-based ventilation prescriptions may be used to set the ventilator with the potential to improve outcomes beyond those achieved with standard lung protective ventilation. This review outlines the measurement and application of clinically applicable pulmonary mechanical concepts, such as plateau pressures, driving pressure, transpulmonary pressures, stress index, and measurement of strain. In addition, the concept of the "baby lung" and the utility of dynamic in addition to static measures of pulmonary mechanical variables are discussed.

  1. Sepsis and ARDS: The Dark Side of Histones

    Science.gov (United States)

    Xu, Zhiheng; Huang, Yongbo; Mao, Pu; Zhang, Jianrong; Li, Yimin

    2015-01-01

    Despite advances in management over the last several decades, sepsis and acute respiratory distress syndrome (ARDS) still remain major clinical challenges and the leading causes of death for patients in intensive care units (ICUs) due to insufficient understanding of the pathophysiological mechanisms of these diseases. However, recent studies have shown that histones, also known as chromatin-basic structure proteins, could be released into the extracellular space during severe stress and physical challenges to the body (e.g., sepsis and ARDS). Due to their cytotoxic and proinflammatory effects, extracellular histones can lead to excessive and overwhelming cell damage and death, thus contributing to the pathogenesis of both sepsis and ARDS. In addition, antihistone-based treatments (e.g., neutralizing antibodies, activated protein C, and heparin) have shown protective effects and have significantly improved the outcomes of mice suffering from sepsis and ARDS. Here, we review researches related to the pathological role of histone in context of sepsis and ARDS and evaluate the potential value of histones as biomarkers and therapeutic targets of these diseases. PMID:26609197

  2. History of mechanical ventilation may affect respiratory mechanics evolution in acute respiratory distress syndrome.

    Science.gov (United States)

    Koutsoukou, Antonia; Perraki, Helen; Orfanos, Stylianos E; Koulouris, Nikolaos G; Tromaropoulos, Andreas; Sotiropoulou, Christina; Roussos, Charis

    2009-12-01

    The aim of this study was to investigate the effect of mechanical ventilation (MV) before acute respiratory distress syndrome (ARDS) on subsequent evolution of respiratory mechanics and blood gases in protectively ventilated patients with ARDS. Nineteen patients with ARDS were stratified into 2 groups according to ARDS onset relative to the onset of MV: In group A (n = 11), MV was applied at the onset of ARDS; in group B (n = 8), MV had been initiated before ARDS. Respiratory mechanics and arterial blood gas were assessed in early (protectively ventilated patients with ARDS, late alteration of respiratory mechanics occurs more commonly in patients who have been ventilated before ARDS onset, suggesting that the history of MV affects the subsequent progress of ARDS even when using protective ventilation.

  3. Mortality in patients with respiratory distress syndrome.

    Science.gov (United States)

    Lopez Saubidet, I; Maskin, L P; Rodríguez, P O; Bonelli, I; Setten, M; Valentini, R

    2016-01-01

    Mortality in Acute Respiratory Distress Syndrome (ARDS) is decreasing, although its prognosis after hospital discharge and the prognostic accuracy of Berlin's new ARDS stratification are uncertain. We did a restrospective analysis of hospital and 6 month mortality of patients with ARDS admitted to the Intensive Care Unit of a Univeristy Hospital in Buenos Aires, between January 2008 and June 2011. ARDS was defined by PaO2/FiO2 lower than 200 mmHg under ventilation with at least 10 cm H2O of PEEP and a FiO2 higher or equal than 0.5. and the presence of bilateral infiltrates in chest radiography, in the absence of cardiogenic acute pulmonary edema, during the first 72 hs of mechanical ventilation. Mortality associated risk factors, the use of rescue therapies and Berlin's stratification for moderate and severe ARDS patients were considered. Ninety eight patients were included; mean age was 59±19 years old, 42,9% had mayor co-morbidities; APACHE II at admission was 22±7; SOFA at day 1 was 8±3. Prone position ventilation was applied in 20,4% and rescue measures in 12,2% (12 patients with nitric oxide and 1 with extracorporeal membrane oxygenation). Hospital and 6 months mortality were 37.7 and 43.8% respectively. After logistic regression analysis, only age, the presence of septic shock at admission, Ppl >30 cmH2O, and major co-morbidities were independently associated with hospital outcome. There was no difference between moderate and severe groups (41,2 and 36,8% respectively; p=0,25). In this cohort, including patients with severe hypoxemia and high percentage of mayor co-morbidities, ARDS associated mortality was lower than some previous studies. There was no increase in mortality after hospital discharge. There was no difference in mortality between moderate and severe groups according to Berlin's definition. Copyright © 2015 Elsevier España, S.L.U. y SEMICYUC. All rights reserved.

  4. Extracorporeal membrane oxygenation (ECMO) as a treatment strategy for severe acute respiratory distress syndrome (ARDS) in the low tidal volume era: A systematic review.

    Science.gov (United States)

    Tillmann, Bourke W; Klingel, Michelle L; Iansavichene, Alla E; Ball, Ian M; Nagpal, A Dave

    2017-10-01

    To evaluate the hospital survival in patients with severe ARDS managed with ECMO and low tidal volume ventilation as compared to patients managed with low tidal volume ventilation alone. Electronic databases were searched for studies of at least 10 adult patients with severe ARDS comparing the use of ECMO with low tidal volume ventilation to mechanical ventilation with a low tidal volume alone. Only studies reporting hospital or ICU survival were included. All identified studies were assessed independently by two reviewers. Of 1782 citations, 27 studies (n=1674) met inclusion criteria. Hospital survival for ECMO patients ranged from 33.3 to 86%, while survival with conventional therapy ranged from 36.3 to 71.2%. Five studies were identified with appropriate control groups allowing comparison, but due to the high degree of variability between studies (I 2 =63%), their results could not be pooled. Two of these studies demonstrated a significant difference, both favouring ECMO over conventional therapy. Given the lack of studies with appropriate control groups, our confidence in a difference in outcome between the two therapies remains weak. Future studies on the use of ECMO for severe ARDS are needed to clarify the role of ECMO in this disease. Copyright © 2017 Elsevier Inc. All rights reserved.

  5. The Montreux definition of neonatal ARDS: biological and clinical background behind the description of a new entity

    NARCIS (Netherlands)

    de Luca, Daniele; van Kaam, Anton H.; Tingay, David G.; Courtney, Sherry E.; Danhaive, Olivier; Carnielli, Virgilio P.; Zimmermann, Luc J.; Kneyber, Martin C. J.; Tissieres, Pierre; Brierley, Joe; Conti, Giorgio; Pillow, Jane J.; Rimensberger, Peter C.

    2017-01-01

    Acute respiratory distress syndrome (ARDS) is undefined in neonates, despite the long-standing existing formal recognition of ARDS syndrome in later life. We describe the Neonatal ARDS Project: an international, collaborative, multicentre, and multidisciplinary project which aimed to produce an ARDS

  6. The Montreux definition of neonatal ARDS : Biological and clinical background behind the description of a new entity

    NARCIS (Netherlands)

    De Luca, Daniele; van Kaam, Anton H.; Tingay, David G.; Courtney, Sherry E.; Danhaive, Olivier; Carnielli, Virgilio P.; Zimmermann, Luc J.; Kneyber, Martin C. J.; Tissieres, Pierre; Brierley, Joe; Conti, Giorgio; Pillow, Jane J.; Rimensberger, Peter C.

    Acute respiratory distress syndrome (ARDS) is undefined in neonates, despite the long-standing existing formal recognition of ARDS syndrome in later life. We describe the Neonatal ARDS Project: an international, collaborative, multicentre, and multidisciplinary project which aimed to produce an ARDS

  7. Serum Uric Acid Level as a Prognostic Marker in Patients With Acute Respiratory Distress Syndrome.

    Science.gov (United States)

    Lee, Hyun Woo; Choi, Sun Mi; Lee, Jinwoo; Park, Young Sik; Lee, Chang-Hoon; Yim, Jae-Joon; Yoo, Chul-Gyu; Kim, Young Whan; Han, Sung Koo; Lee, Sang-Min

    2017-01-01

    Uric acid acts as both a pathogenic inflammatory mediator and an antioxidative agent. Several studies have shown that uric acid level correlates with the incidence, severity, and prognosis of pulmonary diseases. However, the association between uric acid level and acute respiratory distress syndrome (ARDS) has not been studied. This study was conducted to elucidate how serum uric acid level is related with clinical prognosis of ARDS. A retrospective cohort study with propensity score matching was conducted at a medical intensive care unit of a tertiary teaching hospital. The medical records of patients diagnosed with ARDS admitted from 2005 through 2011 were reviewed. Two hundred thirty-seven patients with ARDS met the inclusion criteria. Patients with a serum uric acid level uric acid group, and those with a level ≥3 mg/dL were classified into the normal to high uric acid group. We selected 40 patients in each group using propensity score matching. A higher percentage of patients in the low uric acid group experienced clinical improvement in ARDS. More patients died from sepsis in the normal to high uric acid group. Kaplan-Meier analysis showed that a low serum uric acid level was significantly associated with better survival rate. In patients with ARDS, a low serum uric acid level may be a prognostic marker of a low risk of in-hospital mortality.

  8. Recurrent Spontaneous Pneumothorax during the Recovery Phase of ARDS Due to H1N1 Infection

    Directory of Open Access Journals (Sweden)

    Canan Bor

    2013-03-01

    Full Text Available The pregnant patients are prone to influenza A (H1N1 virus infection, which may rapidly progress to lower respiratory tract infection and subsequent respiratory failure and acute respiratory distress syndrome (ARDS. Pneumothorax might develop in ARDS under mechanical ventilation. But post-ARDS pneumothorax in spontaneously breathing patient has not been reported in the literature. We report a 31-year old pregnant woman infected with influenza A (H1N1 virus and progressed to ARDS. Mechanical ventilation with high PEEP improved patient's gas exchange parameters within 3 weeks. However spontaneous pneumothorax was developed one week after she weaned off the ventilator. After successful drainage therapy, the patient was discharged. However she re-admitted to the hospital because of a recurrent pneumothorax one week later. She was discharged in good health after being treated with negative continuous pleural aspiration for 10 days. Influenza might cause severe pulmonary infection and death. In addition to diffuse alveolar damage, sub-pleural and intrapulmonary air cysts might occur in influenza-related ARDS and may lead to spontaneous pneumothorax. This complication should always be considered during the recovery period of ARDS and a long-term close follow-up is necessary.

  9. Ventilator-associated pneumonia in ARDS patients: the impact of prone positioning. A secondary analysis of the PROSEVA trial.

    Science.gov (United States)

    Ayzac, L; Girard, R; Baboi, L; Beuret, P; Rabilloud, M; Richard, J C; Guérin, C

    2016-05-01

    The goal of this study was to assess the impact of prone positioning on the incidence of ventilator-associated pneumonia (VAP) and the role of VAP in mortality in a recent multicenter trial performed on patients with severe ARDS. An ancillary study of a prospective multicenter randomized controlled trial on early prone positioning in patients with severe ARDS. In suspected cases of VAP the diagnosis was based on positive quantitative cultures of bronchoalveolar lavage fluid or tracheal aspirate at the 10(4) and 10(7) CFU/ml thresholds, respectively. The VAP cases were then subject to central, independent adjudication. The cumulative probabilities of VAP were estimated in each position group using the Aalen-Johansen estimator and compared using Gray's test. A univariate and a multivariate Cox model was performed to assess the impact of VAP, used as a time-dependent covariate for mortality hazard during the ICU stay. In the supine and prone position groups, the incidence rate for VAP was 1.18 (0.86-1.60) and 1.54 (1.15-2.02) per 100 days of invasive mechanical ventilation (p = 0.10), respectively. The cumulative probability of VAP at 90 days was estimated at 46.5 % (27-66) in the prone group and at 33.5 % (23-44) in the supine group. The difference between the two cumulative probability curves was not statistically significant (p = 0.11). In the univariate Cox model, VAP was associated with an increase in the mortality rate during the ICU stay [HR 1.65 (1.05-2.61), p = 0.03]. HR increased to 2.2 (1.39-3.52) (p position group, age, SOFA score, McCabe score, and immunodeficiency. In severe ARDS patients prone positioning did not reduce the incidence of VAP and VAP was associated with higher mortality.

  10. The Right Ventricle in ARDS.

    Science.gov (United States)

    Zochios, Vasileios; Parhar, Ken; Tunnicliffe, William; Roscoe, Andrew; Gao, Fang

    2017-07-01

    ARDS is associated with poor clinical outcomes, with a pooled mortality rate of approximately 40% despite best standards of care. Current therapeutic strategies are based on improving oxygenation and pulmonary compliance while minimizing ventilator-induced lung injury. It has been demonstrated that relative hypoxemia can be well tolerated, and improvements in oxygenation do not necessarily translate into survival benefit. Cardiac failure, in particular right ventricular dysfunction (RVD), is commonly encountered in moderate to severe ARDS and is reported to be one of the major determinants of mortality. The prevalence rate of echocardiographically evident RVD in ARDS varies across studies, ranging from 22% to 50%. Although there is no definitive causal relationship between RVD and mortality, severe RVD is associated with increased mortality. Factors that can adversely affect RV function include hypoxic pulmonary vasoconstriction, hypercapnia, and invasive ventilation with high driving pressure. It might be expected that early diagnosis of RVD would be of benefit; however, echocardiographic markers (qualitative and quantitative) used to prospectively evaluate the right ventricle in ARDS have not been tested in adequately powered studies. In this review, we examine the prognostic implications and pathophysiology of RVD in ARDS and discuss available diagnostic modalities and treatment options. We aim to identify gaps in knowledge and directions for future research that could potentially improve clinical outcomes in this patient population. Copyright © 2017 American College of Chest Physicians. All rights reserved.

  11. Post-ICU psychological morbidity in very long ICU stay patients with ARDS and delirium.

    Science.gov (United States)

    Bashar, Farshid R; Vahedian-Azimi, Amir; Hajiesmaeili, Mohammadreza; Salesi, Mahmood; Farzanegan, Behrooz; Shojaei, Seyedpouzhia; Goharani, Reza; Madani, Seyed J; Moghaddam, Kivan G; Hatamian, Sevak; Moghaddam, Hosseinali J; Mosavinasab, Seyed M M; Elamin, Elamin M; Miller, Andrew C

    2018-02-01

    We investigated the impact of delirium on illness severity, psychological state, and memory in acute respiratory distress syndrome patients with very long ICU stay. Prospective cohort study in the medical-surgical ICUs of 2 teaching hospitals. Very long ICU stay (>75days) and prolonged delirium (≥40days) thresholds were determined by ROC analysis. Subjects were ≥18years, full-code, and provided informed consent. Illness severity was assessed using Acute Physiology and Chronic Health Evaluation IV, Simplified Acute Physiology Score-3, and Sequential Organ Failure Assessment scores. Psychological impact was assessed using the Hospital Anxiety and Depression Scale, Impact of Event Scale-Revised, and the 14-question Post-Traumatic Stress Syndrome (PTSS-14). Memory was assessed using the ICU Memory Tool survey. 181 subjects were included. Illness severity did not correlate with delirium duration. On logistic regression, only PTSS-14<49 correlated with delirium (p=0.001; 95% CI 1.011, 1.041). 49% remembered their ICU stay clearly. 47% had delusional memories, 50% reported intrusive memories, and 44% reported unexplained feelings of panic or apprehension. Delirium was associated with memory impairment and PTSS-14 scores suggestive of PTSD, but not illness severity. Copyright © 2017. Published by Elsevier Inc.

  12. [Analysis of the risk factors of acute respiratory distress syndrome of Berlin new definition in patients with sepsis in emergency department].

    Science.gov (United States)

    Qiao, Liang; Liu, Zhi

    2015-07-01

    To discuss the risk factors of acute respiratory distress syndrome (ARDS) in patients with sepsis in emergency department. 312 patients with sepsis admitted to Department of Emergency of China Medical University Affiliated First Hospital were retrospectively analyzed, and they were divided into two groups according to development of ARDS, which was defined according to the Berlin new definition. The age, gender, vital signs, laboratory results, underlying disease, the mortality in emergency department sepsis (MEDS) score and lung injury prediction score (LIPS) were collected. Univariate analysis was done for each parameter. Statistical significance results were evaluated by multivariate logistic regression analysis. Receiver operating characteristic (ROC) curve was plotted to analyze the predictive value of the parameter for ARDS. The incidence of sepsis-related ARDS was 11.2% (35/312). Within 35 cases of ARDS, there were 10 cases of mild ARDS, 18 cases of moderate ARDS, and 7 cases of severe ARDS. Univariate analysis showed that age (t=-2.134, P=0.035), oxygenation index (t=-4.245, P=0.001), arterial lactate (Lac, t=6.245, Pemergency department, which may be a reference for the early clinical diagnosis of ARDS.

  13. Coinfection and Mortality in Pneumonia-Related Acute Respiratory Distress Syndrome Patients with Bronchoalveolar Lavage: A Prospective Observational Study.

    Science.gov (United States)

    Kao, Kuo-Chin; Chiu, Li-Chung; Hung, Chen-Yiu; Chang, Chih-Hao; Yang, Cheng-Ta; Huang, Chung-Chi; Hu, Han-Chung

    2017-05-01

    Pneumonia is the leading risk factor of acute respiratory distress syndrome (ARDS). It is increasing studies in patients with pneumonia to reveal that coinfection with viral and bacterial infection can lead to poorer outcomes than no coinfection. This study evaluated the role of coinfection identified through bronchoalveolar lavage (BAL) examination on the outcomes of pneumonia-related ARDS. We performed a prospective observational study at Chang Gung Memorial Hospital from October 2012 to May 2015. Adult patients were included if they met the Berlin definition of ARDS. The indications for BAL were clinically suspected pneumonia-related ARDS and no definite microbial sample identified from tracheal aspirate or sputum. The presence of microbial pathogens and clinical outcomes were analyzed. Of the 19,936 patients screened, 902 (4.5%) fulfilled the Berlin definition of ARDS. Of these patients, 255 (22.7%) had pneumonia-related ARDS and were included for analysis. A total of 142 (55.7%) patients were identified to have a microbial pathogen through BAL and were classified into three groups: a virus-only group (n = 41 [28.9%]), no virus group (n = 60 [42.2%]), and coinfection group (n = 41 [28.9%]). ARDS severity did not differ significantly between the groups (P = 0.43). The hospital mortality rates were 53.7% in virus-only identified group, 63.3% in no virus identified group, and 80.5% in coinfection identified group. The coinfection group had significantly higher mortality than virus-only group (80.5% vs. 53.7%; P = 0.01). In patients with pneumonia-related ARDS, the BAL pathogen-positive patients had a trend of higher mortality rate than pathogen-negative patients. Coinfection with a virus and another pathogen was associated with increased hospital mortality in pneumonia-related ARDS patients.

  14. Acute Respiratory Distress Syndrome in Burn Patients: A Comparison of the Berlin and American-European Definitions.

    Science.gov (United States)

    Sine, Christy R; Belenkiy, Slava M; Buel, Allison R; Waters, J Alan; Lundy, Jonathan B; Henderson, Jonathan L; Stewart, Ian J; Aden, James K; Liu, Nehemiah T; Batchinsky, Andriy; Cannon, Jeremy W; Cancio, Leopoldo C; Chung, Kevin K

    2016-01-01

    The purpose of this study was to compare the Berlin definition to the American-European Consensus Conference (AECC) definition in determining the prevalence of acute respiratory distress syndrome (ARDS) and associated mortality in the critically ill burn population. Consecutive patients admitted to our institution with burn injury that required mechanical ventilation for more than 24 hours were included for analysis. Included patients (N = 891) were classified by both definitions. The median age, % TBSA burn, and injury severity score (interquartile ranges) were 35 (24-51), 25 (11-45), and 18 (9-26), respectively. Inhalation injury was present in 35.5%. The prevalence of ARDS was 34% using the Berlin definition and 30.5% using the AECC definition (combined acute lung injury and ARDS), with associated mortality rates of 40.9 and 42.9%, respectively. Under the Berlin definition, mortality rose with increased ARDS severity (14.6% no ARDS; 16.7% mild; 44% moderate; and 59.7% severe, P Berlin definition was not different from patients without ARDS (P = .91). The Berlin definition better stratifies ARDS in terms of severity and correctly excludes those with minimal disease previously captured by the AECC.

  15. Residual high- and low-attenuation lung lesions in survivors of adult respiratory distress syndrome: Etiologies and functional consequences

    International Nuclear Information System (INIS)

    Greene, R.; Kanarek, D.; Lynch, K.; Stark, P.; Zapol, W.

    1986-01-01

    Postrecovery CT and tests of respiratory function were performed in a subset of survivors from among 100 patients who had previously undergone bedide balloon occlusion pulmonary angiography for adult respiratory distress syndrome (ARDS). CT demonstrated multiple poorly marginated, low attenuation lesions, frequently corresponding to areas of vascular obstruction demonstrated on angiography during ARDS. The severity and extent of the lesions correlated with the clinical severity of ARDS, the presence of angiographic filling defects during ARDS, and persistent abnormalities of pulmonary function

  16. Respiratory mechanics to understand ARDS and guide mechanical ventilation.

    Science.gov (United States)

    Mauri, Tommaso; Lazzeri, Marta; Bellani, Giacomo; Zanella, Alberto; Grasselli, Giacomo

    2017-11-30

    As precision medicine is becoming a standard of care in selecting tailored rather than average treatments, physiological measurements might represent the first step in applying personalized therapy in the intensive care unit (ICU). A systematic assessment of respiratory mechanics in patients with the acute respiratory distress syndrome (ARDS) could represent a step in this direction, for two main reasons. Approach and Main results: On the one hand, respiratory mechanics are a powerful physiological method to understand the severity of this syndrome in each single patient. Decreased respiratory system compliance, for example, is associated with low end expiratory lung volume and more severe lung injury. On the other hand, respiratory mechanics might guide protective mechanical ventilation settings. Improved gravitationally dependent regional lung compliance could support the selection of positive end-expiratory pressure and maximize alveolar recruitment. Moreover, the association between driving airway pressure and mortality in ARDS patients potentially underlines the importance of sizing tidal volume on respiratory system compliance rather than on predicted body weight. The present review article aims to describe the main alterations of respiratory mechanics in ARDS as a potent bedside tool to understand severity and guide mechanical ventilation settings, thus representing a readily available clinical resource for ICU physicians.

  17. Gamma scintigraphic analysis of albumin flux in patients with acute respiratory distress syndrome

    International Nuclear Information System (INIS)

    Sugerman, H.J.; Tatum, J.L.; Burke, T.S.; Strash, A.M.; Glauser, F.L.

    1984-01-01

    Computerized gamma-scintigraphy provides a new method for the analysis of albumin flux in patients with pulmonary permeability edema. In this technique, 10 mCi of 99 mTc -tagged human serum albumin is administered and lung:heart radioactivity ratios are determined. This ratio remains constant unless there is a leak of albumin, when a rising ratio with time, called the ''slope index'' (SI), is seen. Thirty-five scintigraphic studies were obtained in 28 patients by means of a portable computerized gamma-camera. Thirteen of these patients had clinical evidence of the acute respiratory distress syndrome (ARDS) and six had or were recovering from left ventricular induced congestive heart failure (CHF). Five of the patients with CHF and pulmonary capillary wedge pressure (PCWP) below 30 mm Hg had normal scintigraphic studies. The patients with ARDS were found to have significantly higher SIs than patients who did not have, or had recovered from, ARDS. Positive SIs were present from 1 to 8 days following the apparent onset of ARDS in seven studies in five patients. Recovery of gas exchange was associated with a return to a normal SI in four patients. In conclusion, computerized gamma-scintigraphy was a sensitive, noninvasive tool for the detection of a pathologic increase in pulmonary protein flux. Positive scintigraphic findings were associated with significantly impaired gas exchange. The method documented that the leak of albumin in patients with ARDS may last for days but resolves with recovery

  18. ARDS User Manual

    Science.gov (United States)

    Fleming, David P.

    2001-01-01

    Personal computers (PCs) are now used extensively for engineering analysis. their capability exceeds that of mainframe computers of only a few years ago. Programs originally written for mainframes have been ported to PCs to make their use easier. One of these programs is ARDS (Analysis of Rotor Dynamic Systems) which was developed at Arizona State University (ASU) by Nelson et al. to quickly and accurately analyze rotor steady state and transient response using the method of component mode synthesis. The original ARDS program was ported to the PC in 1995. Several extensions were made at ASU to increase the capability of mainframe ARDS. These extensions have also been incorporated into the PC version of ARDS. Each mainframe extension had its own user manual generally covering only that extension. Thus to exploit the full capability of ARDS required a large set of user manuals. Moreover, necessary changes and enhancements for PC ARDS were undocumented. The present document is intended to remedy those problems by combining all pertinent information needed for the use of PC ARDS into one volume.

  19. Pulmonary Mechanics and Mortality in Mechanically Ventilated Patients Without Acute Respiratory Distress Syndrome: A Cohort Study.

    Science.gov (United States)

    Fuller, Brian M; Page, David; Stephens, Robert J; Roberts, Brian W; Drewry, Anne M; Ablordeppey, Enyo; Mohr, Nicholas M; Kollef, Marin H

    2018-03-01

    Driving pressure has been proposed as a major determinant of outcome in patients with acute respiratory distress syndrome (ARDS), but there is little data examining the association between pulmonary mechanics, including driving pressure, and outcomes in mechanically ventilated patients without ARDS. Secondary analysis from 1,705 mechanically ventilated patients enrolled in a clinical study that examined outcomes associated with the use of early lung-protective mechanical ventilation. The primary outcome was mortality and the secondary outcome was the incidence of ARDS. Multivariable models were constructed to: define the association between pulmonary mechanics (driving pressure, plateau pressure, and compliance) and mortality; and evaluate if driving pressure contributed information beyond that provided by other pulmonary mechanics. The mortality rate for the entire cohort was 26.0%. Compared with survivors, non-survivors had significantly higher driving pressure [15.9 (5.4) vs. 14.9 (4.4), P = 0.005] and plateau pressure [21.4 (5.7) vs. 20.4 (4.6), P = 0.001]. Driving pressure was independently associated with mortality [adjusted OR, 1.04 (1.01-1.07)]. Models related to plateau pressure also revealed an independent association with mortality, with similar effect size and interval estimates as driving pressure. There were 152 patients who progressed to ARDS (8.9%). Along with driving pressure and plateau pressure, mechanical power [adjusted OR, 1.03 (1.00-1.06)] was also independently associated with ARDS development. In mechanically ventilated patients, driving pressure and plateau pressure are risk factors for mortality and ARDS, and provide similar information. Mechanical power is also a risk factor for ARDS.

  20. The Berlin definition of ARDS: an expanded rationale, justification, and supplementary material.

    Science.gov (United States)

    Ferguson, Niall D; Fan, Eddy; Camporota, Luigi; Antonelli, Massimo; Anzueto, Antonio; Beale, Richard; Brochard, Laurent; Brower, Roy; Esteban, Andrés; Gattinoni, Luciano; Rhodes, Andrew; Slutsky, Arthur S; Vincent, Jean-Louis; Rubenfeld, Gordon D; Thompson, B Taylor; Ranieri, V Marco

    2012-10-01

    Our objective was to revise the definition of acute respiratory distress syndrome (ARDS) using a conceptual model incorporating reliability and validity, and a novel iterative approach with formal evaluation of the definition. The European Society of Intensive Care Medicine identified three chairs with broad expertise in ARDS who selected the participants and created the agenda. After 2 days of consensus discussions a draft definition was developed, which then underwent empiric evaluation followed by consensus revision. The Berlin Definition of ARDS maintains a link to prior definitions with diagnostic criteria of timing, chest imaging, origin of edema, and hypoxemia. Patients may have ARDS if the onset is within 1 week of a known clinical insult or new/worsening respiratory symptoms. For the bilateral opacities on chest radiograph criterion, a reference set of chest radiographs has been developed to enhance inter-observer reliability. The pulmonary artery wedge pressure criterion for hydrostatic edema was removed, and illustrative vignettes were created to guide judgments about the primary cause of respiratory failure. If no risk factor for ARDS is apparent, however, objective evaluation (e.g., echocardiography) is required to help rule out hydrostatic edema. A minimum level of positive end-expiratory pressure and mutually exclusive PaO(2)/FiO(2) thresholds were chosen for the different levels of ARDS severity (mild, moderate, severe) to better categorize patients with different outcomes and potential responses to therapy. This panel addressed some of the limitations of the prior ARDS definition by incorporating current data, physiologic concepts, and clinical trials results to develop the Berlin definition, which should facilitate case recognition and better match treatment options to severity in both research trials and clinical practice.

  1. CT predictors of mortality in pathology confirmed ARDS

    Energy Technology Data Exchange (ETDEWEB)

    Chung, Jonathan H.; Greene, Reginald E.; Shepard, Jo-Anne O.; Digumarthy, Subba R. [Massachusetts General Hospital, Department of Radiology, Boston, MA (United States); Kradin, Richard L. [Massachusetts General Hospital, Department of Pathology and Pulmonary Medicine, Boston, MA (United States)

    2011-04-15

    To identify CT findings that predict mortality in acute respiratory distress syndrome (ARDS) and to identify CT findings that differentiate diffuse alveolar damage (DAD) from DAD with prominent histopathological features of organizing pneumonia (DAD-OP). Twenty-eight patients with ARDS (corroborated by open biopsy) and chest CT within 2 weeks of biopsy were included in our study. Differences in CT findings in patients with survivors versus nonsurvivors as well as for DAD versus DAD-OP were compared using Fisher's exact test. Lung involvement of greater than 80%, RA/LA ratio >1, and varicoid traction bronchiectasis were statistically more common in nonsurvivors than in survivors (respective p values of 0.001, 0.008, and 0.038). PA dilation greater than 3 cm and RV/LV ratio greater than 0.9 were also more common in nonsurvivors than in survivors but these factors did not achieve significance. CT findings did not differentiate DAD from DAD-OP. Our study suggests that >80% of lung involvement, RA/LA ratio >1, and varicoid bronchiectasis predict mortality in patients with ARDS/DAD. Signs of right-sided heart failure (PA dilation greater than 3 cm and RV/LV ratio greater than 0.9) approached significance. CT findings did not differentiate DAD from DAD-OP. (orig.)

  2. Cost Analysis of Noninvasive Helmet Ventilation Compared with Use of Noninvasive Face Mask in ARDS

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    Kwadwo Kyeremanteng

    2018-01-01

    Full Text Available Intensive care unit (ICU costs have doubled since 2000, totalling 108 billion dollars per year. Acute respiratory distress syndrome (ARDS has a prevalence of 10.4% and a 28-day mortality of 34.8%. Noninvasive ventilation (NIV is used in up to 30% of cases. A recent randomized controlled trial by Patel et al. (2016 showed lower intubation rates and 90-day mortality when comparing helmet to face mask NIV in ARDS. The population in the Patel et al. trial was used for cost analysis in this study. Projections of cost savings showed a decrease in ICU costs by $2527 and hospital costs by $3103 per patient, along with a 43.3% absolute reduction in intubation rates. Sensitivity analysis showed consistent cost reductions. Projected annual cost savings, assuming the current prevalence of ARDS, were $237538 in ICU costs and $291682 in hospital costs. At a national level, using yearly incidence of ARDS cases in American ICUs, this represents $449 million in savings. Helmet NIV, compared to face mask NIV, in nonintubated patients with ARDS, reduces ICU and hospital direct-variable costs along with intubation rates, LOS, and mortality. A large-scale cost-effectiveness analysis is needed to validate the findings.

  3. External validation of the APPS, a new and simple outcome prediction score in patients with the acute respiratory distress syndrome

    NARCIS (Netherlands)

    Bos, Lieuwe D.; Schouten, Laura R.; Cremer, Olaf L.; Ong, David S. Y.; Schultz, Marcus J.; Frencken, Jos F.; Bonten, Marc; Klein Klouwenberg, Peter M. C.; Ong, David; van Hooijdonk, Roosmarijn T. M.; Huson, Mischa A.; Schouten, Laura R. A.; Straat, Marleen; van Vught, Lonneke A.; Wiewel, Maryse A.; Witteveen, Esther; Glas, Gerie J.; Wieske, Luuk; van der Poll, Tom

    2016-01-01

    A recently developed prediction score based on age, arterial oxygen partial pressure to fractional inspired oxygen ratio (PaO2/FiO2) and plateau pressure (abbreviated as 'APPS') was shown to accurately predict mortality in patients diagnosed with the acute respiratory distress syndrome (ARDS). After

  4. The Role of Omega-3 Polyunsaturated Fatty Acids in the Treatment of Patients with Acute Respiratory Distress Syndrome: A Clinical Review

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    M. García de Acilu

    2015-01-01

    Full Text Available Acute respiratory distress syndrome (ARDS is defined as the acute onset of noncardiogenic edema and subsequent gas-exchange impairment due to a severe inflammatory process. Recent report on the prognostic value of eicosanoids in patients with ARDS suggests that modulating the inflammatory response through the use of polyunsaturated fatty acids may be a useful strategy for ARDS treatment. The use of enteral diets enriched with eicosapentaenoic acid (EPA and gamma-linolenic acid (GLA has reported promising results, showing an improvement in respiratory variables and haemodynamics. However, the interpretation of the studies is limited by their heterogeneity and methodology and the effect of ω-3 fatty acid-enriched lipid emulsion or enteral diets on patients with ARDS remains unclear. Therefore, the routine use of ω-3 fatty acid-enriched nutrition cannot be recommended and further large, homogeneous, and high-quality clinical trials need to be conducted to clarify the effectiveness of ω-3 polyunsaturated fatty acids.

  5. High initial tidal volumes in emergency department patients at risk for acute respiratory distress syndrome.

    Science.gov (United States)

    Allison, Michael G; Scott, Michael C; Hu, Kami M; Witting, Michael D; Winters, Michael E

    2015-04-01

    Emergency department (ED) patients are at high risk for the acute respiratory distress syndrome (ARDS). Settings only 1 mL/kg above recommended tidal volumes confers harm for these patients. The purpose of this study was to determine whether ED physicians routinely initiate mechanical ventilation with low tidal volumes in patients at risk for ARDS. We retrospectively reviewed the charts of all adult patients who were intubated in an urban, academic ED. The charts were analyzed to identify patients in whom ARDS developed within 48 hours after ED admission. Patients were eligible for inclusion if they had bilateral infiltrates on imaging, had a Pao2/Fio2 ratio less than 300 mm Hg and did not have heart failure contributing to their presentation. The tidal volumes set in the ED were then compared with the recommended tidal volume of 6 mL/kg of predicted body weight. The initial tidal volumes set in the ED were higher than recommended by an average of 80 mL (95% confidence interval, 60-110, P tidal volume ventilation setting. In an academic, tertiary hospital, newly intubated ED patients in whom ARDS developed within 48 hours after intubation were ventilated with tidal volumes that exceeded recommendations by an average of 1.5 mL/kg. Copyright © 2014 Elsevier Inc. All rights reserved.

  6. Evaluation of CT findings in patients with adult respiratory distress syndrome in intensive care unit

    International Nuclear Information System (INIS)

    Honda, Ryoichi; Yoshida, Kazuhiro; Sato, Morihito; Yoshida, Masashi; Ura, Nobuyuki; Kaneko, Masamitsu

    1996-01-01

    Computed tomographic (CT) findings of 19 critically ill patients with adult respiratory distress syndrome (ARDS) in ICU were analyzed retrospectively. ARDS was diagnosed according to Murray's criteria (Lung Injury Score> 2.5). The CT findings obtained were diffuse increased lung opacities [100%] (ground-glass opacity [100%] and air-space consolidation [74%]), lobular distributions of increased opacity [53%], loss of lung volume [53%], bronchiectasis and bronchioloectasis [42%] , fine reticular opacity [26%] and peribronchovascular distributions of increased density [11%]. Among those findings, diffuse increased lung opacities were seen in almost all patients. Especially ground-glass opacity was seen in all patients and distributed in the whole lung. Air-space consolidation was observed in 14 patients and involved mainly lower lung fields. Loss of lung volume and bronchiectasis and bronchioloectasis were likely to exist in the same area of air-space consolidation. Lobular distributions of increased opacity was seen in 10 patients without specific distribution. Those findings were thought to be relatively specific CT findings of ARDS. It is concluded that CT findings provide more information than bedside plain roentgenograms and was useful for clinical management of patients with ARDS. (author)

  7. Clinical features and radiological findings of adenovirus pneumonia associated with progression to acute respiratory distress syndrome: A single center study in 19 adult patients

    Energy Technology Data Exchange (ETDEWEB)

    Cha, Min Jae; Chong, Semin [Dept. of Radiology, Chung-Ang University Hospital, Chung-Ang University College of Medicine, Seoul (Korea, Republic of); Chung, Myung Jin; Lee, Kyung Soo; KIm, Tae Jung; Kim, Tae Sung; Han, Jung Ho [Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul (Korea, Republic of)

    2016-11-15

    To describe radiologic findings of adenovirus pneumonia and to understand clinico-radiological features associated with progression to acute respiratory distress syndrome (ARDS) in patients with adenovirus pneumonia. This study included 19 patients diagnosed with adenovirus pneumonia at a tertiary referral center, in the period between March 2003 and April 2015. Clinical findings were reviewed, and two radiologists assessed imaging findings by consensus. Chi-square, Fisher's exact, and Student's t tests were used for comparing patients with and without subsequent development of ARDS. Of 19 patients, nine were immunocompromised, and 10 were immunocompetent. Twelve patients (63%) progressed to ARDS, six of whom (32%) eventually died from the disease. The average time for progression to ARDS from symptom onset was 9.6 days. Initial chest radiographic findings were normal (n = 2), focal opacity (n = 9), or multifocal or diffuse opacity (n = 8). Computed tomography (CT) findings included bilateral (n = 17) or unilateral (n = 2) ground-glass opacity with consolidation (n = 14) or pleural effusion (n = 11). Patients having subsequent ARDS had a higher probability of pleural effusion and a higher total CT extent compared with the non-ARDS group (p = 0.010 and 0.007, respectively). However, there were no significant differences in clinical variables such as patient age and premorbid condition. Adenovirus pneumonia demonstrates high rates of ARDS and mortality, regardless of patient age and premorbid conditions, in the tertiary care setting. Large disease extent and presence of pleural effusion on CT are factors suggestive of progression to ARDS.

  8. CT-findings in ARDS

    Energy Technology Data Exchange (ETDEWEB)

    Stark, P; Greene, R; Kott, M M; Hall, T; Vanderslice, L

    1987-08-01

    The CT features of 28 patients with ARDS are described. Diffuse lung consolidation, multifocal patchy involvement and lobar or segmental disease were observed. Large lung cysts as well as small cysts producing a 'swiss-cheese' appearance of the parenchyma, were detected. These findings were not regularly appreciated on chest radiographs. The overall mortality of our 28 patients was 72.7% (22 out 28). Patients with lung cysts showed a trend toward higher mortality (87.5% or 13 out 16). Other unexpected findings were basilar lung abscesses and an empyema. In 15 out of 28 patients, CT scans provided additional information, not obvious on bedside chest radiographs and led to a change in management in five patients.

  9. Absence of association between angiotensin converting enzyme polymorphism and development of adult respiratory distress syndrome in patients with severe acute respiratory syndrome: a case control study

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    Chiu Rossa WK

    2005-04-01

    Full Text Available Abstract Background It has been postulated that genetic predisposition may influence the susceptibility to SARS-coronavirus infection and disease outcomes. A recent study has suggested that the deletion allele (D allele of the angiotensin converting enzyme (ACE gene is associated with hypoxemia in SARS patients. Moreover, the ACE D allele has been shown to be more prevalent in patients suffering from adult respiratory distress syndrome (ARDS in a previous study. Thus, we have investigated the association between ACE insertion/deletion (I/D polymorphism and the progression to ARDS or requirement of intensive care in SARS patients. Method One hundred and forty genetically unrelated Chinese SARS patients and 326 healthy volunteers were recruited. The ACE I/D genotypes were determined by polymerase chain reaction and agarose gel electrophoresis. Results There is no significant difference in the genotypic distributions and the allelic frequencies of the ACE I/D polymorphism between the SARS patients and the healthy control subjects. Moreover, there is also no evidence that ACE I/D polymorphism is associated with the progression to ARDS or the requirement of intensive care in the SARS patients. In multivariate logistic analysis, age is the only factor associated with the development of ARDS while age and male sex are independent factors associated with the requirement of intensive care. Conclusion The ACE I/D polymorphism is not directly related to increased susceptibility to SARS-coronavirus infection and is not associated with poor outcomes after SARS-coronavirus infection.

  10. Assessing the quality of studies supporting genetic susceptibility and outcomes of ARDS

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    Marialbert eAcosta-Herrera

    2014-02-01

    Full Text Available The acute respiratory distress syndrome (ARDS is a severe inflammatory disease manifested as a result of pulmonary and systemic responses to several insults. It is now well accepted that genetic variation influences these responses. However, little is known about the genes that are responsible for patient susceptibility and outcome of ARDS. Methodological flaws are still abundant among genetic association studies with ARDS and here, we aimed to highlight the quality criteria where the standards have not been reached, to expose the associated genes to facilitate replication attempts, and to provide quick-reference guidance for future studies. We conducted a PubMed search from January 2008 to September 2012 for original articles. Studies were considered if a statistically significant association was declared with either susceptibility or outcomes of all-cause ARDS. Fourteen criteria were used for evaluation and results were compared to those from a previous quality assessment report. Significant improvements affecting study design and statistical analysis were detected. However, major issues such as adjustments for the underlying population stratification and replication studies remain poorly addressed.

  11. Effect of vitamin D deficiency in Korean patients with acute respiratory distress syndrome.

    Science.gov (United States)

    Park, Sojung; Lee, Min Gi; Hong, Sang-Bum; Lim, Chae-Man; Koh, Younsuck; Huh, Jin Won

    2018-06-20

    Vitamin D modulates innate and adaptive immune responses, and vitamin D deficiency is associated with increased mortality in hospitalized patients with pneumonia. We evaluated the prevalence of vitamin D deficiency in Korean patients with acute respiratory distress syndrome (ARDS) and its effect on the clinical outcomes of ARDS. We retrospectively analyzed the data of 108 patients who had a measured serum level of 25-hydroxy vitamin D3 (25(OH)D3) at the time of diagnosis with ARDS. The clinical outcomes were evaluated based on 25(OH)D3 levels of 20 ng/mL and stratified by quartiles of 25(OH)D3 levels. The mean age of patients was 59.4 years old; 77 (71.3%) were male. Vitamin D deficiency was found in 103 patients (95.4%). The mean 25(OH)D3 level was 8.3 ± 7.0 ng/mL. Neither in-hospital mortality (40.0% vs. 68.0%) nor 6-month mortality (40.0% vs. 71.8%) significantly differed between groups. There were no significant differences in 25(OH)D3 level between survivors (8.1 ± 7.6 ng/mL) and non-survivors (8.5 ± 6.8 ng/mL, p = 0.765). There were no trends toward a difference in mortality among quartiles of 25(OH)D3 levels. However, 25(OH)D3 levels were inversely related with length of hospital stay and intensive care unit stay among in-hospital survivors. Vitamin D deficiency was prevalent in Korean patients with ARDS. However, levels of vitamin D were not associated with mortality. A large, prospective study is needed to evaluate the effects of vitamin D deficiency on clinical outcomes of ARDS.

  12. Recent advances in understanding acute respiratory distress syndrome [version 1; referees: 2 approved

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    Peter Wohlrab

    2018-03-01

    Full Text Available Acute respiratory distress syndrome (ARDS is characterized by acute diffuse lung injury, which results in increased pulmonary vascular permeability and loss of aerated lung tissue. This causes bilateral opacity consistent with pulmonary edema, hypoxemia, increased venous admixture, and decreased lung compliance such that patients with ARDS need supportive care in the intensive care unit to maintain oxygenation and prevent adverse outcomes. Recently, advances in understanding the underlying pathophysiology of ARDS led to new approaches in managing these patients. In this review, we want to focus on recent scientific evidence in the field of ARDS research and discuss promising new developments in the treatment of this disease.

  13. Association of Heme Oxygenase 1 with Lung Protection in Malaria-Associated ALI/ARDS.

    Science.gov (United States)

    Pereira, Marcelo L M; Ortolan, Luana S; Sercundes, Michelle K; Debone, Daniela; Murillo, Oscar; Lima, Flávia A; Marinho, Claudio R F; Epiphanio, Sabrina

    2016-01-01

    Malaria is a serious disease, caused by the parasite of the genus Plasmodium , which was responsible for 440,000 deaths in 2015. Acute lung injury/acute respiratory distress syndrome (ALI/ARDS) is one of the main clinical complications in severe malaria. The murine model DBA/2 reproduces the clinical signs of ALI/ARDS in humans, when infected with Plasmodium berghei ANKA. High levels of HO-1 were reported in cases of severe malaria. Our data indicated that the HO-1 mRNA and protein expression are increased in mice that develop malaria-associated ALI/ARDS (MA-ALI/ARDS). Additionally, the hemin, a HO-1 inducing drug, prevented mice from developing MA-ALI/ARDS when administered prior to the development of MA-ALI/ARDS in this model. Also, hemin treatment showed an amelioration of respiratory parameters in mice, high VEGF levels in the sera, and a decrease in vascular permeability in the lung, which are signs of ALI/ARDS. Therefore, the induction of HO-1 before the development of MA-ALI/ARDS could be protective. However, the increased expression of HO-1 on the onset of MA-ALI/ARDS development may represent an effort to revert the phenotype of this syndrome by the host. We therefore confirm that HO-1 inducing drugs could be used for prevention of MA-ALI/ARDS in humans.

  14. Diagnosis of Acute Respiratory Distress Syndrome in Nosocomial Pneumonia

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    A. N. Kuzovlev

    2009-01-01

    Full Text Available Objective: to define the informative value of the parameters of gas exchange, lung volumetry, and central hemodynamics in the diagnosis of acute respiratory distress syndrome (ARDS in nosocomial pneumonia (NP. Subjects and methods. The study included 38 patients with cancer and severe injury who were divided into 3 groups in accordance with the diagnostic criteria of ARDS and NP: 1 patients with ARDS + NP; 2 those with NP; 3 those with non-ARDS, non-PN. ARDS was diagnosed in 2 steps. At Step 1, the investigators took into account risk factors for ARDS and used the lung injury scale developed by J. Murray et al. and the ARDS diagnostic criteria defined by the American-European Consensus Conference on ARDS. At Step 2, after obtaining the data of lung volume-try (1—2 hours after Step 1, they assessed the compliance of the above criteria for ARDS with those developed by the V. A. Negovsky Research Institute of General Reanimatology, Russian Academy of Medical Sciences, and redistributed the patients between the groups. The stage of ARDS was determined in accordance with the classification of the above Institute. All the patients underwent a comprehensive examination the key element of which was to estimate gas exchange parameters and to monitor lung volumetry and central hemodynamics by the transpulmonary thermodilution methods, by using a Pulsion PiCCO Plus monitor (Pulsion Medical Systems, Germany. The findings were statistically analyzed using a Statistica 7.0 package (arithmetic mean, error of the mean, _ Student’s test, Newman-Keuls test, correlation analysis. The difference was considered to be significant if p-value was Results. The patients with ARDS + NP were observed to have a significantly lower oxygenation index (10 ml/kg and Murray scale scores (>2 than those in patients with NP without ARDS. The reference values of the pulmonary vascular permeability index due to its inadequate informative value call for further investigation. The

  15. Effects on hemodynamics and gas exchange of omega-3 fatty acid-enriched lipid emulsion in acute respiratory distress syndrome (ARDS: a prospective, randomized, double-blind, parallel group study

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    Sacanell Judit

    2008-10-01

    Full Text Available Abstract Introduction We investigated the effects on hemodynamics and gas exchange of a lipid emulsion enriched with omega-3 fatty acids in patients with ARDS. Methods The design was a prospective, randomized, double-blind, parallel group study in our Intensive Medicine Department of Vall d'Hebron University Hospital (Barcelona-Spain. We studied 16 consecutive patients with ARDS and intolerance to enteral nutrition (14 men and 2 women; mean age: 58 ± 13 years; APACHE II score: 17.8 ± 2.3; Lung Injury Score: 3.1 ± 0.5; baseline PaO2/FiO2 ratio: 149 ± 40. Patients were randomized into 2 groups: Group A (n = 8 received the study emulsion Lipoplus® 20%, B.Braun Medical (50% MCT, 40% LCT, 10% ω-3; Group B (n = 8 received the control emulsion Intralipid® Fresenius Kabi (100% LCT. Lipid emulsions were administered during 12 h at a dose of 0.12 g/kg/h. Measurements of the main hemodynamic and gas exchange parameters were made at baseline (immediately before administration of the lipid emulsions, every hour during the lipid infusion, at the end of administration, and six hours after the end of administration lipid infusion. Results No statistically significant changes were observed in the different hemodynamic values analyzed. Likewise, the gas exchange parameters did not show statistically significant differences during the study. No adverse effect attributable to the lipid emulsions was seen in the patients analyzed. Conclusion The lipid emulsion enriched with omega-3 fatty acids was safe and well tolerated in short-term administration to patients with ARDS. It did not cause any significant changes in hemodynamic and gas exchange parameters. Trial registration ISRCTN63673813

  16. Low Tidal Volume Ventilation in Patients without Acute Respiratory Distress Syndrome: A Paradigm Shift in Mechanical Ventilation

    OpenAIRE

    Lipes, Jed; Bojmehrani, Azadeh; Lellouche, Francois

    2012-01-01

    Protective ventilation with low tidal volume has been shown to reduce morbidity and mortality in patients suffering from acute lung injury (ALI) and acute respiratory distress syndrome (ARDS). Low tidal volume ventilation is associated with particular clinical challenges and is therefore often underutilized as a therapeutic option in clinical practice. Despite some potential difficulties, data have been published examining the application of protective ventilation in patients without lung inj...

  17. Hemodynamics and Gas Exchange Effects of Inhaled Nitrous Oxide in Patients with Acute Respiratory Distress Syndrome

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    V. N. Poptsov

    2006-01-01

    Full Text Available Inhaled nitrous oxide (iNO therapy aimed at improving pulmonary oxygenizing function and at decreasing artificial ventilation (AV load has been used in foreign clinical practice in the past decade. The study was undertaken to evaluate the hemodynamic and gas exchange effects of iNO in acute respiratory distress syndrome (ARDS that developed after car-diosurgical operations. Fifty-eight (43 males and 15 females patients aged 21 to 76 (55.2±2.4 years were examined. The study has demonstrated that in 48.3% of cases, the early stage of ARDS is attended by the increased tone pulmonary vessels due to impaired NO-dependent vasodilatation. In these patients, iNO therapy is an effective therapeutic method for correcting hemodynamic disorders and lung oxygenizing function.

  18. Factors among patients receiving prone positioning for the acute respiratory distress syndrome found useful for predicting mortality in the intensive care unit.

    Science.gov (United States)

    Modrykamien, Ariel M; Daoud, Yahya

    2018-01-01

    Optimal mechanical ventilation management in patients with the acute respiratory distress syndrome (ARDS) involves the use of low tidal volumes and limited plateau pressure. Refractory hypoxemia may not respond to this strategy, requiring other interventions. The use of prone positioning in severe ARDS resulted in improvement in 28-day survival. To determine whether mechanical ventilation strategies or other parameters affected survival in patients undergoing prone positioning, a retrospective analysis was conducted of a consecutive series of patients with severe ARDS treated with prone positioning. Demographic and clinical information involving mechanical ventilation strategies, as well as other variables associated with prone positioning, was collected. The rate of in-hospital mortality was obtained, and previously described parameters were compared between survivors and nonsurvivors. Forty-three patients with severe ARDS were treated with prone positioning, and 27 (63%) died in the intensive care unit. Only three parameters were significant predictors of survival: APACHE II score ( P = 0.03), plateau pressure ( P = 0.02), and driving pressure ( P = 0.04). The ability of each of these parameters to predict mortality was assessed with receiver operating characteristic curves. The area under the curve values for APACHE II, plateau pressure, and driving pressure were 0.74, 0.69, and 0.67, respectively. In conclusion, in a group of patients with severe ARDS treated with prone positioning, only APACHE II, plateau pressure, and driving pressure were associated with mortality in the intensive care unit.

  19. A Markov computer simulation model of the economics of neuromuscular blockade in patients with acute respiratory distress syndrome

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    Chow John L

    2006-03-01

    Full Text Available Abstract Background Management of acute respiratory distress syndrome (ARDS in the intensive care unit (ICU is clinically challenging and costly. Neuromuscular blocking agents may facilitate mechanical ventilation and improve oxygenation, but may result in prolonged recovery of neuromuscular function and acute quadriplegic myopathy syndrome (AQMS. The goal of this study was to address a hypothetical question via computer modeling: Would a reduction in intubation time of 6 hours and/or a reduction in the incidence of AQMS from 25% to 21%, provide enough benefit to justify a drug with an additional expenditure of $267 (the difference in acquisition cost between a generic and brand name neuromuscular blocker? Methods The base case was a 55 year-old man in the ICU with ARDS who receives neuromuscular blockade for 3.5 days. A Markov model was designed with hypothetical patients in 1 of 6 mutually exclusive health states: ICU-intubated, ICU-extubated, hospital ward, long-term care, home, or death, over a period of 6 months. The net monetary benefit was computed. Results Our computer simulation modeling predicted the mean cost for ARDS patients receiving standard care for 6 months to be $62,238 (5% – 95% percentiles $42,259 – $83,766, with an overall 6-month mortality of 39%. Assuming a ceiling ratio of $35,000, even if a drug (that cost $267 more hypothetically reduced AQMS from 25% to 21% and decreased intubation time by 6 hours, the net monetary benefit would only equal $137. Conclusion ARDS patients receiving a neuromuscular blocker have a high mortality, and unpredictable outcome, which results in large variability in costs per case. If a patient dies, there is no benefit to any drug that reduces ventilation time or AQMS incidence. A prospective, randomized pharmacoeconomic study of neuromuscular blockers in the ICU to asses AQMS or intubation times is impractical because of the highly variable clinical course of patients with ARDS.

  20. Lung Microbiota Is Related to Smoking Status and to Development of Acute Respiratory Distress Syndrome in Critically Ill Trauma Patients.

    Science.gov (United States)

    Panzer, Ariane R; Lynch, Susan V; Langelier, Chaz; Christie, Jason D; McCauley, Kathryn; Nelson, Mary; Cheung, Christopher K; Benowitz, Neal L; Cohen, Mitchell J; Calfee, Carolyn S

    2018-03-01

    Cigarette smoking is associated with increased risk of acute respiratory distress syndrome (ARDS) in patients after severe trauma; however, the mechanisms underlying this association are unknown. To determine whether cigarette smoking contributes to ARDS development after trauma by altering community composition of the lung microbiota. We studied the lung microbiota of mechanically ventilated patients admitted to the ICU after severe blunt trauma. To do so, we used 16S ribosomal RNA gene amplicon sequencing of endotracheal aspirate samples obtained on ICU admission (n = 74) and at 48 hours after admission (n = 30). Cigarette smoke exposure (quantified using plasma cotinine), ARDS development, and other clinical parameters were correlated with lung microbiota composition. Smoking status was significantly associated with lung bacterial community composition at ICU admission (P = 0.007 by permutational multivariate ANOVA [PERMANOVA]) and at 48 hours (P = 0.03 by PERMANOVA), as well as with significant enrichment of potential pathogens, including Streptococcus, Fusobacterium, Prevotella, Haemophilus, and Treponema. ARDS development was associated with lung community composition at 48 hours (P = 0.04 by PERMANOVA) and was characterized by relative enrichment of Enterobacteriaceae and of specific taxa enriched at baseline in smokers, including Prevotella and Fusobacterium. After severe blunt trauma, a history of smoking is related to lung microbiota composition, both at the time of ICU admission and at 48 hours. ARDS development is also correlated with respiratory microbial community structure at 48 hours and with taxa that are relatively enriched in smokers at ICU admission. The data derived from this pilot study suggest that smoking-related changes in the lung microbiota could be related to ARDS development after severe trauma.

  1. Cachexia Syndrome, anorexia patient

    International Nuclear Information System (INIS)

    Roldán, G.; Musé, I.

    2004-01-01

    Introduction: Two thirds of patients (ptes) cancer present slimming recognized a negative prognostic factor. Anorexia cachexia syndrome (SCA) results from the interaction of multiple factors and causes death of 22% of these patients. Nutritional support produces a moderate recovery weight without affecting the underlying metabolic disorders. Objectives: Conduct a review of current knowledge of the underlying pathophysiology and management the cachexia-anorexia syndrome in cancer patients. Designing indications possible policy interventions in the management of these patients. Method: Performed an a literature review on SCA. Conclusions: We identify patients at risk for early implementation of non-pharmacological measures preventive. The control side effects to treatment oncospecific with particular attention to the need for antiemetics, laxatives / antidiarrheal control dental and proper pain management is fundamental. Keep track enteral is a priority. In those with swallowing disorders or dysphagia, nasogastric feeding tube should be considered early. Indications for gastrostomy / jejunostomy and total parenteral nutrition (TPN) are very limited. The NPT is a complementary treatment maneuver a temporary and reversible complication, in order to prevent deterioration

  2. Plasma Adiponectin, clinical factors, and patient outcomes during the acute respiratory distress syndrome.

    Directory of Open Access Journals (Sweden)

    Allan J Walkey

    Full Text Available Adiponectin (APN is an anti-inflammatory hormone derived from adipose tissue that attenuates acute lung injury in rodents. In this study, we investigated the association between circulating APN and outcomes among patients with acute respiratory distress syndrome (ARDS.We performed a retrospective cohort study using data and plasma samples from participants in the multicenter ARDS Network Fluid and Catheter Treatment Trial.Plasma APN concentrations were measured in 816 (81.6% trial participants at baseline and in 568 (56.8% subjects at both baseline and day 7 after enrollment. Clinical factors associated with baseline APN levels in multivariable-adjusted models included sex, body mass index, past medical history of cirrhosis, and central venous pressure (model R2 = 9.7%. We did not observe an association between baseline APN and either severity of illness (APACHE III or extent of lung injury (Lung Injury Score. Among patients who received right heart catheterization (n = 384, baseline APN was inversely related to mean pulmonary artery pressure (β = -0.015, R2 1.5%, p = 0.02; however, this association did not persist in multivariable models (β = -0.009, R2 0.5%, p = 0.20. Neither baseline APN levels [HR per quartile1.04 (95% CI 0.91-1.18, p = 0.61], nor change in APN level from baseline to day 7 [HR 1.04 (95% CI 0.89-1.23, p = 0.62] were associated with 60 day mortality in Cox proportional hazards regression models. However, subgroup analysis identified an association between APN and mortality among patients who developed ARDS from extra-pulmonary etiologies [HR per quartile 1.31 (95% CI 1.08-1.57]. APN levels did not correlate with mortality among patients developing ARDS in association with direct pulmonary injury [HR 0.96 (95% CI 0.83-1.13], pinteraction = 0.016.Plasma APN levels did not correlate with disease severity or mortality in a large cohort of patients with ARDS. However, higher APN levels were

  3. Personalizing mechanical ventilation for acute respiratory distress syndrome.

    Science.gov (United States)

    Berngard, S Clark; Beitler, Jeremy R; Malhotra, Atul

    2016-03-01

    Lung-protective ventilation with low tidal volumes remains the cornerstone for treating patient with acute respiratory distress syndrome (ARDS). Personalizing such an approach to each patient's unique physiology may improve outcomes further. Many factors should be considered when mechanically ventilating a critically ill patient with ARDS. Estimations of transpulmonary pressures as well as individual's hemodynamics and respiratory mechanics should influence PEEP decisions as well as response to therapy (recruitability). This summary will emphasize the potential role of personalized therapy in mechanical ventilation.

  4. Personalizing mechanical ventilation for acute respiratory distress syndrome

    OpenAIRE

    Berngard, S. Clark; Beitler, Jeremy R.; Malhotra, Atul

    2016-01-01

    Lung-protective ventilation with low tidal volumes remains the cornerstone for treating patient with acute respiratory distress syndrome (ARDS). Personalizing such an approach to each patient's unique physiology may improve outcomes further. Many factors should be considered when mechanically ventilating a critically ill patient with ARDS. Estimations of transpulmonary pressures as well as individual's hemodynamics and respiratory mechanics should influence PEEP decisions as well as response ...

  5. Extravascular lung water and the pulmonary vascular permeability index may improve the definition of ARDS.

    Science.gov (United States)

    Perel, Azriel

    2013-01-24

    The recent Berlin definition has made some improvements in the older definition of acute respiratory distress syndrome (ARDS), although the concepts and components of the definition remained largely unchanged. In an effort to improve both predictive and face validity, the Berlin panel has examined a number of additional measures that may reflect increased pulmonary vascular permeability, including extravascular lung water. The panel concluded that although extravascular lung water has improved face validity and higher values are associated with mortality, it is infeasible to mandate on the basis of availability and the fact that it does not distinguish between hydrostatic and inflammatory pulmonary edema. However, the results of a multi-institutional study that appeared in the previous issue of Critical Care show that this latter reservation may not necessarily be true. By using extravascular lung water and the pulmonary vascular permeability index, both of which are derived from transpulmonary thermodilution, the authors could successfully differentiate between patients with ARDS and other patients in respiratory failure due to either cardiogenic edema or pleural effusion with atelectasis. This commentary discusses the merits and limitations of this study in view of the potential improvement that transpulmonary thermodilution may bring to the definition of ARDS.

  6. Acute Respiratory Distress Syndrome

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    Carmen Sílvia Valente Barbas

    2012-01-01

    Full Text Available This paper, based on relevant literature articles and the authors' clinical experience, presents a goal-oriented respiratory management for critically ill patients with acute respiratory distress syndrome (ARDS that can help improve clinicians' ability to care for these patients. Early recognition of ARDS modified risk factors and avoidance of aggravating factors during hospital stay such as nonprotective mechanical ventilation, multiple blood products transfusions, positive fluid balance, ventilator-associated pneumonia, and gastric aspiration can help decrease its incidence. An early extensive clinical, laboratory, and imaging evaluation of “at risk patients” allows a correct diagnosis of ARDS, assessment of comorbidities, and calculation of prognostic indices, so that a careful treatment can be planned. Rapid administration of antibiotics and resuscitative measures in case of sepsis and septic shock associated with protective ventilatory strategies and early short-term paralysis associated with differential ventilatory techniques (recruitment maneuvers with adequate positive end-expiratory pressure titration, prone position, and new extracorporeal membrane oxygenation techniques in severe ARDS can help improve its prognosis. Revaluation of ARDS patients on the third day of evolution (Sequential Organ Failure Assessment (SOFA, biomarkers and response to infection therapy allows changes in the initial treatment plans and can help decrease ARDS mortality.

  7. [Asthenic syndrome in patients with burnout syndrome].

    Science.gov (United States)

    Chutko, L S; Surushkina, S Iu; Rozhkova, A V; Nikishena, I S; Iakovenko, E A

    2013-01-01

    The authors present the results of a survey of 103 patients aged 25 to 45 years with burnout syndrom. The results showed that most patients with the syndrome of burnout have clinical manifestations of asthenia, varying degrees of severity. According to psychological and psychophysiological examination in this group of patients were found attention and memory dysfunction. This study evaluated the efficacy of memoplant in the treatment of this pathology. The high efficiency of memoplant (improvement in 69.7% of cases) was detected, confirmed by the data of the clinical, psychological and neuropsychological research.

  8. The Ratio of Partial Pressure Arterial Oxygen and Fraction of Inspired Oxygen 1 Day After Acute Respiratory Distress Syndrome Onset Can Predict the Outcomes of Involving Patients.

    Science.gov (United States)

    Lai, Chih-Cheng; Sung, Mei-I; Liu, Hsiao-Hua; Chen, Chin-Ming; Chiang, Shyh-Ren; Liu, Wei-Lun; Chao, Chien-Ming; Ho, Chung-Han; Weng, Shih-Feng; Hsing, Shu-Chen; Cheng, Kuo-Chen

    2016-04-01

    The initial hypoxemic level of acute respiratory distress syndrome (ARDS) defined according to Berlin definition might not be the optimal predictor for prognosis. We aimed to determine the predictive validity of the stabilized ratio of partial pressure arterial oxygen and fraction of inspired oxygen (PaO2/FiO2 ratio) following standard ventilator setting in the prognosis of patients with ARDS.This prospective observational study was conducted in a single tertiary medical center in Taiwan and compared the stabilized PaO2/FiO2 ratio (Day 1) following standard ventilator settings and the PaO2/FiO2 ratio on the day patients met ARDS Berlin criteria (Day 0). Patients admitted to intensive care units and in accordance with the Berlin criteria for ARDS were collected between December 1, 2012 and May 31, 2015. Main outcome was 28-day mortality. Arterial blood gas and ventilator setting on Days 0 and 1 were obtained.A total of 238 patients met the Berlin criteria for ARDS were enrolled, and they were classified as mild (n = 50), moderate (n = 125), and severe (n = 63) ARDS, respectively. Twelve (5%) patients who originally were classified as ARDS did not continually meet the Berlin definition, and a total of 134 (56%) patients had the changes regarding the severity of ARDS from Day 0 to Day 1. The 28-day mortality rate was 49.1%, and multivariate analysis identified age, PaO2/FiO2 on Day 1, number of organ failures, and positive fluid balance within 5 days as significant risk factors of death. Moreover, the area under receiver-operating curve for mortality prediction using PaO2/FiO2 on Day 1 was significant higher than that on Day 0 (P = 0.016).PaO2/FiO2 ratio on Day 1 after applying mechanical ventilator is a better predictor of outcomes in patients with ARDS than those on Day 0.

  9. An NFKB1 promoter insertion/deletion polymorphism influences risk and outcome in acute respiratory distress syndrome among Caucasians.

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    Ednan K Bajwa

    2011-05-01

    Full Text Available Nuclear factor-κB (NF-κB is required for transcription of many pro-inflammatory genes and has been implicated in the pathogenesis of acute respiratory distress syndrome (ARDS. We hypothesized that a known functional polymorphism in the promoter of the NFKB1 gene may affect susceptibility to and outcome from ARDS.A case control study was conducted among a cohort of patients admitted to the intensive care unit (ICU with risk factors for the development of ARDS. 379 patients with ARDS and 793 at-risk controls were studied. Patients were followed for 60 days with development of ARDS as a primary outcome; ARDS-related mortality and organ dysfunction were secondary outcomes.Patients homozygous for the 4 base pair deletion in the promoter of NFKB1 (del/del did not have an increased odds ratio (OR of developing ARDS in unadjusted analysis but were more likely to develop ARDS in the presence of a significant interaction between the del/del genotype and age (OR 5.21, 95% CI 1.35-20.0. In multivariate analysis, patients with ARDS and the del/del genotype also had increased 60 day mortality (HR 1.54, 95% CI 1.01-2.36 and more severe daily organ dysfunction (P<.001 when compared to ARDS patients with other genotypes.The del/del genotype is associated with an age-dependent increase in odds of developing ARDS. Patients with the del/del genotype and ARDS also have increased hazard of 60 day mortality and more organ failure.

  10. The Montreux definition of neonatal ARDS: biological and clinical background behind the description of a new entity.

    Science.gov (United States)

    De Luca, Daniele; van Kaam, Anton H; Tingay, David G; Courtney, Sherry E; Danhaive, Olivier; Carnielli, Virgilio P; Zimmermann, Luc J; Kneyber, Martin C J; Tissieres, Pierre; Brierley, Joe; Conti, Giorgio; Pillow, Jane J; Rimensberger, Peter C

    2017-08-01

    Acute respiratory distress syndrome (ARDS) is undefined in neonates, despite the long-standing existing formal recognition of ARDS syndrome in later life. We describe the Neonatal ARDS Project: an international, collaborative, multicentre, and multidisciplinary project which aimed to produce an ARDS consensus definition for neonates that is applicable from the perinatal period. The definition was created through discussions between five expert members of the European Society for Paediatric and Neonatal Intensive Care; four experts of the European Society for Paediatric Research; two independent experts from the USA and two from Australia. This Position Paper provides the first consensus definition for neonatal ARDS (called the Montreux definition). We also provide expert consensus that mechanisms causing ARDS in adults and older children-namely complex surfactant dysfunction, lung tissue inflammation, loss of lung volume, increased shunt, and diffuse alveolar damage-are also present in several critical neonatal respiratory disorders. Copyright © 2017 Elsevier Ltd. All rights reserved.

  11. Extravascular lung water and pulmonary arterial wedge pressure for fluid management in patients with acute respiratory distress syndrome.

    Science.gov (United States)

    Hu, Wei; Lin, Chang-Wen; Liu, Bing-Wei; Hu, Wei-Hang; Zhu, Ying

    2014-01-16

    Extravascular lung water (EVLW) is a sensitive prognostic indicator of pulmonary edema. Thus, EVLW may be an advantageous method of fluid management. This study aims to evaluate the outcomes of using EVLW and pulmonary artery wedge pressure (PAWP) as strategies for fluid management in patients with acute respiratory distress syndrome (ARDS). Twenty-nine patients were randomly divided into the EVLW and PAWP groups. The survival rate, ICU (Intensive Care Unit) length of stay, duration of mechanical ventilation, acute lung injury scores, and oxygenation index of the EVLW and PAWP groups were compared. No significant difference in the survival rates at 28 and 60 days (d) after treatment was found between the two groups (p = 0.542). The duration of mechanical ventilation and ICU length of stay were significantly lower (p management improved clinical results in patients with ARDS better than PAWP.

  12. Influence of quality of care and individual patient characteristics on quality of life and return to work in survivors of the acute respiratory distress syndrome: protocol for a prospective, observational, multi-centre patient cohort study (DACAPO).

    Science.gov (United States)

    Brandstetter, Susanne; Dodoo-Schittko, Frank; Blecha, Sebastian; Sebök, Philipp; Thomann-Hackner, Kathrin; Quintel, Michael; Weber-Carstens, Steffen; Bein, Thomas; Apfelbacher, Christian

    2015-12-17

    Health-related quality of life (HRQoL) and return to work are important outcomes in critical care medicine, reaching beyond mortality. Little is known on factors predictive of HRQoL and return to work in critical illness, including the acute respiratory distress syndrome (ARDS), and no evidence exists on the role of quality of care (QoC) for outcomes in survivors of ARDS. It is the aim of the DACAPO study ("Surviving ARDS: the influence of QoC and individual patient characteristics on quality of life") to investigate the role of QoC and individual patient characteristics on quality of life and return to work. A prospective, observational, multi-centre patient cohort study will be performed in Germany, using hospitals from the "ARDS Network Germany" as the main recruiting centres. It is envisaged to recruit 2400 patients into the DACAPO study and to analyse a study population of 1500 survivors. They will be followed up until 12 months after discharge from hospital. QoC will be assessed as process quality, structural quality and volume at the institutional level. The main outcomes (HRQoL and return to work) will be assessed by self-report questionnaires. Further data collection includes general medical and ARDS-related characteristics of patients as well as sociodemographic and psycho-social parameters. Multilevel hierarchical modelling will be performed to analyse the effects of QoC and individual patient characteristics on outcomes, taking the cluster structure of the data into account. By obtaining comprehensive data at patient and hospital level using a prospective multi-centre design, the DACAPO-study is the first study investigating the influence of QoC on individual outcomes of ARDS survivors.

  13. Acute respiratory distress syndrome

    Directory of Open Access Journals (Sweden)

    Marco Confalonieri

    2017-04-01

    Full Text Available Since its first description, the acute respiratory distress syndrome (ARDS has been acknowledged to be a major clinical problem in respiratory medicine. From July 2015 to July 2016 almost 300 indexed articles were published on ARDS. This review summarises only eight of them as an arbitrary overview of clinical relevance: definition and epidemiology, risk factors, prevention and treatment. A strict application of definition criteria is crucial, but the diverse resource-setting scenarios foster geographic variability and contrasting outcome data. A large international multicentre prospective cohort study including 50 countries across five continents reported that ARDS is underdiagnosed, and there is potential for improvement in its management. Furthermore, epidemiological data from low-income countries suggest that a revision of the current definition of ARDS is needed in order to improve its recognition and global clinical outcome. In addition to the well-known risk-factors for ARDS, exposure to high ozone levels and low vitamin D plasma concentrations were found to be predisposing circumstances. Drug-based preventive strategies remain a major challenge, since two recent trials on aspirin and statins failed to reduce the incidence in at-risk patients. A new disease-modifying therapy is awaited: some recent studies promised to improve the prognosis of ARDS, but mortality and disabling complications are still high in survivors in intensive care.

  14. How to approach the acute respiratory distress syndrome: Prevention, plan, and prudence.

    Science.gov (United States)

    Koh, Younsuck

    2017-05-01

    The acute respiratory distress syndrome (ARDS) is typically manifested by refractory hypoxemia with high mortality. A correct diagnosis is the first step to achieve better outcomes. An early intervention to manage modifiable risk factors of ARDS development and the avoidance of aggravating factors that increase disease severity and progression should be carefully addressed. A management plan is necessary at an early stage of ARDS to determine the level of intensive care. It should be carefully decided which therapeutic measures should be performed depending on the patient׳s underlying clinical condition. The clinician׳s considerate prudence is required in decisions of when to apply intensive measures for an ARDS treatment. Mechanical ventilator support should be carefully used depending on the patient׳s severity and pathological phase. Decreasing inappropriate alveolar strain through a low tidal volume under optimal positive end-expiratory pressure is key for ventilator support in ARDS. The extracorporeal membrane oxygenation applied in the experienced centers seems to improve the survival of patients with severe ARDS. A constellation of physical and psychological problems can develop or persist for up to 5 years in patients with ARDS. Therefore, an early mobilization with rehabilitation, even during an intensive care unit stay, should be seriously considered whenever feasible. Lastly, prevention of aspiration, stress ulcers, deep vein thrombosis, catheter-related infection, overhydration, and heavy sedation is essential to achieve better outcomes in ARDS. Copyright © 2017 The Japanese Respiratory Society. Published by Elsevier B.V. All rights reserved.

  15. Síndrome da ardência bucal: revisão de cem casos = Burning mouth syndrome: review of one hundred cases

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    Cherubini, Karen

    2005-01-01

    Full Text Available Os prontuários de cem pacientes portadores de síndrome da ardência bucal (SAB foram revisados considerando-se as seguintes variáveis: idade, sexo e cor dos pacientes bem como drogas usadas regularmente. Os resultados evidenciaram que a SAB é mais freqüente em mulheres na faixa etária compreendida entre 51 e 70 anos, a idade média dos pacientes foi de 59,8 anos e as drogas mais freqüentemente utilizadas foram os anti-hipertensivos (40% e os psicotrópicos (30%. A alta freqüência de uso desses fármacos pode ser resultado, respectivamente, da faixa etária dos pacientes acometidos pela SAB e de sua relação com condições como ansiedade e depressão. Embora muitos possíveis fatores etiológicos tenham sido apontados para a síndrome, sua causa específica permanece desconhecida e, conseqüentemente, não há tratamento eficaz para a condição. Novos estudos fazem-se necessários a fim de que se elucide a causa da SAB para então proporcionar-se melhor qualidade de vida aos pacientes

  16. Acute respiratory distress syndrome mimickers lacking common risk factors of the Berlin definition.

    Science.gov (United States)

    Gibelin, Aude; Parrot, Antoine; Maitre, Bernard; Brun-Buisson, Christian; Mekontso Dessap, Armand; Fartoukh, Muriel; de Prost, Nicolas

    2016-02-01

    Some patients presenting with acute respiratory failure and meeting the Berlin criteria for acute respiratory distress syndrome (ARDS) lack exposure to common risk factors (CRF). These so-called ARDS mimickers often lack histological diffuse alveolar damage. We aimed to describe such ARDS mimickers lacking CRF (ARDS CRF-) in comparison with others (ARDS CRF+). Retrospective study including all patients receiving invasive mechanical ventilation for ARDS admitted to the intensive care units (ICUs) of two tertiary care centers from January 2003 to December 2012. The prevalence of ARDS CRF- was 7.5 % (95 % CI [5.5-9.5]; n = 50/665). On the basis of medical history, bronchoalveolar lavage fluid cytology, and chest CT scan patterns, four etiological categories were identified: immune (n = 18; 36 %), drug-induced (n = 13; 26 %), malignant (n = 7; 14 %), and idiopathic (n = 12; 24 %). Although the ARDS CRF- patients had a lower logistic organ dysfunction score (4 [3-8] vs. 10 [6-13]; p logistic regression analysis (adjusted OR = 2.06; 95 % CI [1.02-4.18]; p = 0.044). Among ARDS CRF- patients, the presence of potentially reversible lung lesions with corticosteroids (aOR = 0.14; 95 % CI [0.03-0.62]) was associated with ICU survival. The absence of CRF among patients with ARDS is common and associated with a higher risk of mortality. For such atypical ARDS, a complete diagnostic workup, including bronchoalveolar lavage fluid cytology and chest CT scan patterns, should be performed to identify those patients who might benefit from specific therapies, including corticosteroids.

  17. Mechanical Ventilation and ARDS in the ED: A Multicenter, Observational, Prospective, Cross-sectional Study.

    Science.gov (United States)

    Fuller, Brian M; Mohr, Nicholas M; Miller, Christopher N; Deitchman, Andrew R; Levine, Brian J; Castagno, Nicole; Hassebroek, Elizabeth C; Dhedhi, Adam; Scott-Wittenborn, Nicholas; Grace, Edward; Lehew, Courtney; Kollef, Marin H

    2015-08-01

    There are few data regarding mechanical ventilation and ARDS in the ED. This could be a vital arena for prevention and treatment. This study was a multicenter, observational, prospective, cohort study aimed at analyzing ventilation practices in the ED. The primary outcome was the incidence of ARDS after admission. Multivariable logistic regression was used to determine the predictors of ARDS. We analyzed 219 patients receiving mechanical ventilation to assess ED ventilation practices. Median tidal volume was 7.6 mL/kg predicted body weight (PBW) (interquartile range, 6.9-8.9), with a range of 4.3 to 12.2 mL/kg PBW. Lung-protective ventilation was used in 122 patients (55.7%). The incidence of ARDS after admission from the ED was 14.7%, with a mean onset of 2.3 days. Progression to ARDS was associated with higher illness severity and intubation in the prehospital environment or transferring facility. Of the 15 patients with ARDS in the ED (6.8%), lung-protective ventilation was used in seven (46.7%). Patients who progressed to ARDS experienced greater duration in organ failure and ICU length of stay and higher mortality. Lung-protective ventilation is infrequent in patients receiving mechanical ventilation in the ED, regardless of ARDS status. Progression to ARDS is common after admission, occurs early, and worsens outcome. Patient- and treatment-related factors present in the ED are associated with ARDS. Given the limited treatment options for ARDS, and the early onset after admission from the ED, measures to prevent onset and to mitigate severity should be instituted in the ED. ClinicalTrials.gov; No.: NCT01628523; URL: www.clinicaltrials.gov.

  18. Incidence Proportion of Acute Cor Pulmonale in Patients with Acute Respiratory Distress Syndrome Subjected to Lung Protective Ventilation: A Systematic Review and Meta-analysis.

    Science.gov (United States)

    Das, Saurabh Kumar; Choupoo, Nang Sujali; Saikia, Priyam; Lahkar, Amitabh

    2017-06-01

    Reported incidence of acute cor pulmonale (ACP) in patients with acute respiratory distress syndrome (ARDS) varies from 10% to 84%, despite being subjected to lung protective ventilation according to the current guidelines. The objective of this review is to find pooled cumulative incidence of ACP in patients with ARDS undergoing lung protective ventilation. We searched MEDLINE, EMBASE, Cochrane Library, KoreaMed, LILACS, and WHO Clinical Trial Registry. Cross-sectional or cohort studies were included if they reported or provided data that could be used to calculate the incidence proportion of ACP. Inverse variance heterogeneity (IVhet) and random effect model were used for the main outcome and measures. We included 16 studies encompassing 1661 patients. The cumulative incidence of ACP using IVhet analysis was 23% (95% confidence interval [CI] = 18%-28%) over 3 days of lung protective ventilation. Random effect analysis of 7 studies (1250 patients) revealed pooled odd ratio of mortality of 1.16 (95% CI = 0.80-1.67, P = 0.44) due to ACP. Patients with ARDS have a 23% risk of developing ACP with lung protective ventilation. Findings of this review indicate the need of updating existing guidelines for ventilating ARDS patients to incorporate right ventricle protective strategy.

  19. Identification of novel single nucleotide polymorphisms associated with acute respiratory distress syndrome by exome-seq.

    Directory of Open Access Journals (Sweden)

    Katherine Shortt

    Full Text Available Acute respiratory distress syndrome (ARDS is a lung condition characterized by impaired gas exchange with systemic release of inflammatory mediators, causing pulmonary inflammation, vascular leak and hypoxemia. Existing biomarkers have limited effectiveness as diagnostic and therapeutic targets. To identify disease-associating variants in ARDS patients, whole-exome sequencing was performed on 96 ARDS patients, detecting 1,382,399 SNPs. By comparing these exome data to those of the 1000 Genomes Project, we identified a number of single nucleotide polymorphisms (SNP which are potentially associated with ARDS. 50,190SNPs were found in all case subgroups and controls, of which89 SNPs were associated with susceptibility. We validated three SNPs (rs78142040, rs9605146 and rs3848719 in additional ARDS patients to substantiate their associations with susceptibility, severity and outcome of ARDS. rs78142040 (C>T occurs within a histone mark (intron 6 of the Arylsulfatase D gene. rs9605146 (G>A causes a deleterious coding change (proline to leucine in the XK, Kell blood group complex subunit-related family, member 3 gene. rs3848719 (G>A is a synonymous SNP in the Zinc-Finger/Leucine-Zipper Co-Transducer NIF1 gene. rs78142040, rs9605146, and rs3848719 are associated significantly with susceptibility to ARDS. rs3848719 is associated with APACHE II score quartile. rs78142040 is associated with 60-day mortality in the overall ARDS patient population. Exome-seq is a powerful tool to identify potential new biomarkers for ARDS. We selectively validated three SNPs which have not been previously associated with ARDS and represent potential new genetic biomarkers for ARDS. Additional validation in larger patient populations and further exploration of underlying molecular mechanisms are warranted.

  20. Lynch syndrome: the patients' perspective

    NARCIS (Netherlands)

    Seppen, Jurgen; Bruzzone, Linda

    2013-01-01

    People with Lynch syndrome have a high lifetime risk for the development of colorectal, endometrial and several other types of cancer. Lynch syndrome is caused by germline mutations in genes encoding DNA mismatch repair proteins. In this review, issues that concern Lynch patients are highlighted

  1. Lower tidal volume strategy (?3?ml/kg) combined with extracorporeal CO2 removal versus ?conventional? protective ventilation (6?ml/kg) in severe ARDS

    OpenAIRE

    Bein, Thomas; Weber-Carstens, Steffen; Goldmann, Anton; M?ller, Thomas; Staudinger, Thomas; Brederlau, J?rg; Muellenbach, Ralf; Dembinski, Rolf; Graf, Bernhard M.; Wewalka, Marlene; Philipp, Alois; Wernecke, Klaus-Dieter; Lubnow, Matthias; Slutsky, Arthur S.

    2013-01-01

    Background Acute respiratory distress syndrome is characterized by damage to the lung caused by various insults, including ventilation itself, and tidal hyperinflation can lead to ventilator induced lung injury (VILI). We investigated the effects of a low tidal volume (V T) strategy (V T???3?ml/kg/predicted body weight [PBW]) using pumpless extracorporeal lung assist in established ARDS. Methods Seventy-nine patients were enrolled after a ?stabilization period? (24?h with optimized therapy an...

  2. Effects of short-term pressure-controlled ventilation on gas exchange, airway pressures, and gas distribution in patients with acute lung injury/ARDS: comparison with volume-controlled ventilation.

    Science.gov (United States)

    Prella, Maura; Feihl, François; Domenighetti, Guido

    2002-10-01

    The potential clinical benefits of pressure-controlled ventilation (PCV) over volume-controlled ventilation (VCV) in patients with acute lung injury (ALI) or ARDS still remain debated. We compared PCV with VCV in patients with ALI/ARDS with respect to the following physiologic end points: (1) gas exchange and airway pressures, and (2) CT scan intrapulmonary gas distribution at end-expiration. Prospective, observational study. A multidisciplinary ICU in a nonuniversity, acute-care hospital. Ten patients with ALI or ARDS (9 men and 1 woman; age range, 17 to 80 years). Sequential ventilation in PCV and VCV with a constant inspiratory/expiratory ratio, tidal volume, respiratory rate, and total positive end-expiratory pressure; measurement of gas exchange and airway pressures; and achievement of CT sections at lung base, hilum, and apex for the quantitative analysis of lung densities and of aerated vs nonaerated zones. PaO(2), PaCO(2), and PaO(2)/fraction of inspired oxygen ratio levels did not differ between PCV and VCV. Peak airway pressure (Ppeak) was significantly lower in PCV compared with VCV (26 +/- 2 cm H(2)O vs 31 +/- 2 cm H(2)O; p mean +/- SEM). The surface areas of the nonaerated zones as well as the total areas at each section level were unchanged in PCV compared with VCV, except at the apex level, where there was a significantly greater nonaerated area in VCV (11 +/- 2 cm(2) vs 9 +/- 2 cm(2); p mean CT number of each lung (20 lungs from 10 patients) was similar in the two modes, as were the density values at the basal and apical levels; the hilum mean CT number was - 442 +/- 28 Hounsfield units (HU) in VCV and - 430 +/- 26 HU in PCV (p lower Ppeaks through the precise titration of the lung distending pressure, and might be applied to avoid regional overdistension by means of a more homogeneous gas distribution.

  3. Ex vivo effect of varespladib on secretory phospholipase A2 alveolar activity in infants with ARDS.

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    Daniele De Luca

    Full Text Available Secretory phospholipase A2 (sPLA2 plays a pivotal role in acute respiratory distress syndrome (ARDS. This enzyme seems an interesting target to reduce surfactant catabolism and lung tissue inflammation. Varespladib is a specifically designed indolic sPLA2 inhibitor, which has shown promising results in animals and adults. No specific data in pediatric ARDS patients are yet available.We studied varespladib in broncho-alveolar lavage (BAL fluids obtained ex vivo from pediatric ARDS patients. Clinical data and worst gas exchange values during the ARDS course were recorded. Samples were treated with saline or 10-40-100 µM varespladib and incubated at 37°C. Total sPLA2 activity was measured by non-radioactive method. BAL samples were subjected to western blotting to identify the main sPLA isotypes with different sensitivity to varespladib. Results was corrected for lavage dilution using the serum-to-BAL urea ratio and for varespladib absorbance.Varespladib reduces sPLA2 activity (p<0.0001 at 10,40 and 100 µM; both sPLA2 activity reduction and its ratio to total proteins significantly raise with increasing varespladib concentrations (p<0.001. IC(50 was 80 µM. Western blotting revealed the presence of sPLA2-IIA and -IB isotypes in BAL samples. Significant correlations exist between the sPLA2 activity reduction/proteins ratio and PaO(2 (rho = 0.63;p<0.001, PaO(2/FiO(2 (rho = 0.7; p<0.001, oxygenation (rho = -0.6; p<0.001 and ventilation (rho = -0.4;p = 0.038 indexes.Varespladib significantly inhibits sPLA2 in BAL of infants affected by post-neonatal ARDS. Inhibition seems to be inversely related to the severity of gas exchange impairment.

  4. Effect of prone positioning during mechanical ventilation on mortality among patients with acute respiratory distress syndrome: a systematic review and meta-analysis.

    Science.gov (United States)

    Sud, Sachin; Friedrich, Jan O; Adhikari, Neill K J; Taccone, Paolo; Mancebo, Jordi; Polli, Federico; Latini, Roberto; Pesenti, Antonio; Curley, Martha A Q; Fernandez, Rafael; Chan, Ming-Cheng; Beuret, Pascal; Voggenreiter, Gregor; Sud, Maneesh; Tognoni, Gianni; Gattinoni, Luciano; Guérin, Claude

    2014-07-08

    Mechanical ventilation in the prone position is used to improve oxygenation and to mitigate the harmful effects of mechanical ventilation in patients with acute respiratory distress syndrome (ARDS). We sought to determine the effect of prone positioning on mortality among patients with ARDS receiving protective lung ventilation. We searched electronic databases and conference proceedings to identify relevant randomized controlled trials (RCTs) published through August 2013. We included RCTs that compared prone and supine positioning during mechanical ventilation in patients with ARDS. We assessed risk of bias and obtained data on all-cause mortality (determined at hospital discharge or, if unavailable, after longest follow-up period). We used random-effects models for the pooled analyses. We identified 11 RCTs (n=2341) that met our inclusion criteria. In the 6 trials (n=1016) that used a protective ventilation strategy with reduced tidal volumes, prone positioning significantly reduced mortality (risk ratio 0.74, 95% confidence interval 0.59-0.95; I2=29%) compared with supine positioning. The mortality benefit remained in several sensitivity analyses. The overall quality of evidence was high. The risk of bias was low in all of the trials except one, which was small. Statistical heterogeneity was low (I2mechanical ventilation improved survival among patients with ARDS who received protective lung ventilation. © 2014 Canadian Medical Association or its licensors.

  5. [A patient with Noonan syndrome].

    Science.gov (United States)

    Bins, A; Gortzak, R A Th

    2013-12-01

    Noonan syndrome is a relatively common autosomal dominant genetic disorder which is characterised by typical facial features, congenital heart diseases and small stature. In 50% of the cases the syndrome is caused by a mutation on the PTPN11-gen. The expression of symptoms associated with Noonan syndrome can be very mild in nature and facial features usually become less pronounced with age, which can sometimes make a correct diagnosis more difficult. Despite a wide range of associated symptoms most adults with Noonan syndrome can be self-sustaining, with a good quality of life. It is important that the dentist is well-informed about this syndrome due to the heart diseases and bleeding disorders which can be present with these patients and may influence a dentist's choice of therapy when invasive treatment is indicated.

  6. Effects of an omega-3 fatty acid-enriched lipid emulsion on eicosanoid synthesis in acute respiratory distress syndrome (ARDS: A prospective, randomized, double-blind, parallel group study

    Directory of Open Access Journals (Sweden)

    Chacon Pilar

    2011-04-01

    Full Text Available Abstract Background The use of lipid emulsions has been associated with changes in lung function and gas exchange which may be mediated by biologically active metabolites derived from arachidonic acid. The type and quantity of the lipid emulsions used could modulate this response, which is mediated by the eicosanoids. This study investigates the use of omega-3 fatty acid-enriched lipid emulsions in ARDS patients and their effects on eicosanoid values. Methods Prospective, randomized, double-blind, parallel group study carried out at the Intensive Medicine Department of Vall d'Hebron University Hospital (Barcelona-Spain. We studied 16 consecutive patients with ARDS and intolerance to enteral nutrition (14 men; age: 58 ± 13 years; APACHE II score 17.8 ± 2.3; Lung Injury Score: 3.1 ± 0.5; baseline PaO2/FiO2 ratio: 149 ± 40. Patients were randomized into two groups: Group A (n = 8 received the study emulsion Lipoplus® 20%, B. Braun Medical (50% MCT, 40% LCT, 10% fish oil (FO; Group B (n = 8 received the control emulsion Intralipid® Fresenius Kabi (100% LCT. Lipid emulsions were administered for 12 h at a dose of 0.12 g/kg/h. We measured LTB4, TXB2, and 6-keto prostaglandin F1α values at baseline [immediately before the administration of the lipid emulsions (T-0], at the end of the administration (T-12 and 24 hours after the beginning of the infusion (T 24 in arterial and mixed venous blood samples. Results In group A (FO LTB4, TXB2, 6-keto prostaglandin F1α levels fell during omega-3 administration (T12. After discontinuation (T24, levels of inflammatory markers (both systemic and pulmonary behaved erratically. In group B (LCT all systemic and pulmonary mediators increased during lipid administration and returned to baseline levels after discontinuation, but the differences did not reach statistical significance. There was a clear interaction between the treatment in group A (fish oil and changes in LTB4 over time. Conclusions Infusion of

  7. Determinants of early adult respiratory distress syndrome with special reference to chest radiography

    International Nuclear Information System (INIS)

    Wegenius, G.; Modig, J.; Akademiska Sjukhuset, Uppsala

    1985-01-01

    The medical records of 220 consecutive patients with traumatic injuries admitted to the intensive care unit in the years 1974-1982 were scrutinised in an attempt to find radiographic signs and clinical determinants of early adult respiratory distress syndrome (ARDS). All patients included in this study were considered to run a risk of developing ARDS and had 'pure' major fractures in the sense that there were no accompanying severe brain, chest or abdominal injuries. There were no deaths in this series of patients. ARDS developed in 27 patients (12.3%), on an average on the second day of trauma. Chest radiographs of 21 of these patients showed features indicative of ARDS, whereas those of the other 6 patients were normal despite hypoxaemia. In most of the 27 patients the only radiographic manifestation of ARDS was interstitial oedema; only a minority presented with alveolar oedema in addition. In 6 patients no radiographic changes ever occurred. Ventilator treatment with positive end expiratory pressure may have prevented the pulmonary insufficiency from becoming radiographically manifest. The clinical determinants of post-traumatic ARDS were a high fracture index, based on the number and severity of fractures, implying severe trauma, and shock on admission. Fluid overload was not found to be the cause of ARDS. (orig.)

  8. Association between insertion/deletion polymorphism in angiotensin-converting enzyme gene and acute lung injury/acute respiratory distress syndrome: a meta-analysis

    Directory of Open Access Journals (Sweden)

    Matsuda Akihisa

    2012-08-01

    Full Text Available Abstract Background A previous meta-analysis reported a positive association between an insertion/deletion (I/D polymorphism in the angiotensin-converting enzyme gene (ACE and the risk of acute lung injury (ALI/acute respiratory distress syndrome (ARDS. Here, we updated this meta-analysis and additionally assessed the association of this polymorphism with ALI/ARDS mortality. Methods We searched electronic databases through October 2011 for the terms “angiotensin-converting enzyme gene”, “acute lung injury”, and “acute respiratory distress syndrome,” and reviewed all studies that reported the relationship of the I/D polymorphism in ACE with ALI/ARDS in humans. Seven studies met the inclusion criteria, comprising 532 ALI/ARDS patients, 3032 healthy controls, and 1432 patients without ALI/ARDS. We used three genetic models: the allele, dominant, and recessive models. Results The ACE I/D polymorphism was not associated with susceptibility to ALI/ARDS for any genetic model. However, the ACE I/D polymorphism was associated with the mortality risk of ALI/ARDS in Asian subjects ( Pallele Pdominant = 0.001, Precessive = 0.002. This finding remained significant after correction for multiple comparisons. Conclusions There is a possible association between the ACE I/D polymorphism genotype and the mortality risk of ALI/ARDS in Asians.

  9. Acute Respiratory Distress Syndrome in Severe Brain Injury

    Directory of Open Access Journals (Sweden)

    Yu. A. Churlyaev

    2009-01-01

    Full Text Available Objective: to study the development of acute respiratory distress syndrome (ARDS in victims with isolated severe brain injury (SBI. Subject and methods. 171 studies were performed in 16 victims with SBI. Their general condition was rated as very critical. The patients were divided into three groups: 1 non-ARDS; 2 Stage 1 ARDS; and 3 Stage 2 ARDS. The indicators of Stages 1 and 2 were assessed in accordance with the classification proposed by V. V. Moroz and A. M. Golubev. Intracranial pressure (ICP, extravascular lung water index, pulmonary vascular permeability, central hemodynamics, oxygenation index, lung anastomosis, the X-ray pattern of the lung and brain (computed tomography, and its function were monitored. Results. The hemispheric cortical level of injury of the brain with function compensation of its stem was predominantly determined in the controls; subcompensation and decompensation were ascertained in the ARDS groups. According to the proposed classification, these patients developed Stages 1 and 2 ARDS. When ARDS developed, there were rises in the level of extravascular lung fluid and pulmonary vascular permeability, a reduction in the oxygenation index (it was 6—12 hours later as compared with them, increases in a lung shunt and ICP; X-ray study revealed bilateral infiltrates in the absence of heart failure in Stage 2 ARDS. The correlation was positive between ICP and extravascular lung water index, and lung vascular permeability index (r>0.4;p<0.05. Conclusion. The studies have indicated that the classification proposed by V. V. Moroz and A. M. Golubev enables an early diagnosis of ARDS. One of its causes is severe brainstem injury that results in increased extravascular fluid in the lung due to its enhanced vascular permeability. The ICP value is a determinant in the diagnosis of secondary brain injuries. Key words: acute respiratory distress syndrome, extravascu-lar lung fluid, pulmonary vascular permeability, brain injury

  10. Outcome prediction in pneumonia induced ALI/ARDS by clinical features and peptide patterns of BALF determined by mass spectrometry.

    Science.gov (United States)

    Frenzel, Jochen; Gessner, Christian; Sandvoss, Torsten; Hammerschmidt, Stefan; Schellenberger, Wolfgang; Sack, Ulrich; Eschrich, Klaus; Wirtz, Hubert

    2011-01-01

    Peptide patterns of bronchoalveolar lavage fluid (BALF) were assumed to reflect the complex pathology of acute lung injury (ALI)/acute respiratory distress syndrome (ARDS) better than clinical and inflammatory parameters and may be superior for outcome prediction. A training group of patients suffering from ALI/ARDS was compiled from equal numbers of survivors and nonsurvivors. Clinical history, ventilation parameters, Murray's lung injury severity score (Murray's LISS) and interleukins in BALF were gathered. In addition, samples of bronchoalveolar lavage fluid were analyzed by means of hydrophobic chromatography and MALDI-ToF mass spectrometry (MALDI-ToF MS). Receiver operating characteristic (ROC) analysis for each clinical and cytokine parameter revealed interleukin-6>interleukin-8>diabetes mellitus>Murray's LISS as the best outcome predictors. Outcome predicted on the basis of BALF levels of interleukin-6 resulted in 79.4% accuracy, 82.7% sensitivity and 76.1% specificity (area under the ROC curve, AUC, 0.853). Both clinical parameters and cytokines as well as peptide patterns determined by MALDI-ToF MS were analyzed by classification and regression tree (CART) analysis and support vector machine (SVM) algorithms. CART analysis including Murray's LISS, interleukin-6 and interleukin-8 in combination was correct in 78.0%. MALDI-ToF MS of BALF peptides did not reveal a single identifiable biomarker for ARDS. However, classification of patients was successfully achieved based on the entire peptide pattern analyzed using SVM. This method resulted in 90% accuracy, 93.3% sensitivity and 86.7% specificity following a 10-fold cross validation (AUC = 0.953). Subsequent validation of the optimized SVM algorithm with a test group of patients with unknown prognosis yielded 87.5% accuracy, 83.3% sensitivity and 90.0% specificity. MALDI-ToF MS peptide patterns of BALF, evaluated by appropriate mathematical methods can be of value in predicting outcome in pneumonia induced

  11. Hearing impairment in genotyped Wolfram syndrome patients.

    NARCIS (Netherlands)

    Plantinga, R.F.; Pennings, R.J.E.; Huygen, P.L.M.; Bruno, R.; Eller, P.; Barrett, T.G.; Vialettes, B.; Paquis-Fluklinger, V.; Lombardo, F.; Cremers, C.W.R.J.

    2008-01-01

    OBJECTIVES: Wolfram syndrome is a progressive neurodegenerative syndrome characterized by the features "DIDMOAD" (diabetes insipidus, diabetes mellitus, optic atrophy, and deafness). We sought to study the audiometric data of genotyped Wolfram syndrome patients with sensorineural hearing impairment.

  12. Clinical practice of acute respiratory distress syndrome in Japan: A nationwide survey and scientific evidences.

    Science.gov (United States)

    Tasaka, Sadatomo; Tatsumi, Koichiro

    2017-07-01

    There has been limited information about epidemiology and clinical practice of acute respiratory distress syndrome (ARDS) in Japan. An invitation letter to the web-based survey was mailed to all 871 board certified hospitals of the Japanese Respiratory Society. The questionnaires were designed to collect data on epidemiology and clinical practice of ARDS, including diagnostic measures and therapeutics. Within 4 months of the survey period, valid responses were obtained from 296 (34%) hospitals. The incidence of ARDS was estimated to be 3.13 cases/100 hospital beds or 1.91 cases/ICU bed per year. The most frequent underlying disease was pneumonia (34%), followed by sepsis (29%). In hospitals with fewer ICU beds, pulmonologists tended to be in charge of management of ARDS patients. Routine diagnostic measures included computed tomography of the chest (69.6% of the hospitals) and Swan-Ganz catheterization was rarely performed for diagnosis. In 87.4% of the hospitals, non-invasive ventilation was applied to management of ARDS patients, especially those with mild disease. Prone positioning and extracorporeal membrane oxygenation (ECMO) for ARDS patients was more widely adopted in hospitals with larger numbers of ICU beds and intensivists. In 58.2% of the responding hospitals, corticosteroid was considered as a treatment option for ARDS, among which pulse therapy was routinely introduced to ARDS patients in 35.4%. The incidence of ARDS in Japan was estimated to be lower than that in the recent international study. The scale and equipment of hospitals and the number of intensivists might influence clinical practice of ARDS. Copyright © 2017 The Japanese Respiratory Society. Published by Elsevier B.V. All rights reserved.

  13. June 2015 Phoenix critical care journal club: interventions in ARDS

    Directory of Open Access Journals (Sweden)

    Robbins RA

    2015-06-01

    Full Text Available No abstract available. Article truncated at 150 words. Mortality has been declining in the adult respiratory distress syndrome (ARDS (1. However, the cause of the decline in mortality is unclear. The only intervention shown to improve survival has been low tidal volume ventilation but the mortality was improving before this intervention was widely used (2. Nevertheless, it was suggested that we look at system performance regarding ARDS management from a critical appraisal standpoint. This journal club was hoped to help as a starting point in that regard. Four potential beneficial interventions were discussed: 1. Conservative fluid management; 2. Optimal PEEP as determined by esophageal pressure; 3. Prone positioning; and 4. Mechanical ventilation driving pressure. National Heart, Lung, and Blood Institute Acute Respiratory Distress Syndrome (ARDS Clinical Trials Network, Wiedemann HP, Wheeler AP, Bernard GR, Thompson BT, Hayden D, deBoisblanc B, Connors AF Jr, Hite RD, Harabin AL. Comparison of two fluid-management strategies in acute lung injury. N Engl ...

  14. Optimal support techniques when providing mechanical ventilation to patients with acute respiratory distress syndrome.

    Science.gov (United States)

    Parissopoulos, Stelios; Mpouzika, Meropi DA; Timmins, Fiona

    2017-01-01

    Adult respiratory distress syndrome (ARDS) is a type of acute diffuse lung injury characterized by severe inflammation, increased pulmonary vascular permeability and a loss of aerated lung tissue. The effects of high fraction of inspired oxygen (FiO 2 ) include oxygen toxicity manifested by damage to the lung parenchyma in the acute phase of lung injury. There is still a high mortality rate among this group of patients, so clinically sensitive evidence-based interventions are paramount to maximize survival chances during critical care. The aim of this article is to explore the current opinion concerning optimal mechanical ventilation support techniques for patients with acute respiratory distress syndrome. A literature search of clinical trials and observation studies, reviews, discussion papers, meta-analyses and clinical guidelines written in English up to 2015, derived from the databases of Scopus, CINAHL, Cochrane Library databases and PubMed was conducted. Low tidal volume, pressure limitation and prone positioning in severe ARDS patients appear to be of some benefit. More research is required and further development and use of standardized protocols is an important strategy for reducing practice variations across disciplines, as well as giving clear guidelines to nurses practising in critical care. There is also evidence that this syndrome is under-diagnosed and the utilization of lung protective ventilation is still variable. It is important that nurses have underlying knowledge of both aetiology of ARDS and ventilation management, and that they monitor patients very closely. The adoption of a low tidal ventilation protocol, which is based on quality evidence guidelines, the value of rescue therapies and patient observation practices in the overall patient management, and the need to place emphasis on long-term patient outcomes, all these emerge as key factors for consideration and future research. However, there is also a need for more research that would

  15. External Validity of Electronic Sniffers for Automated Recognition of Acute Respiratory Distress Syndrome.

    Science.gov (United States)

    McKown, Andrew C; Brown, Ryan M; Ware, Lorraine B; Wanderer, Jonathan P

    2017-01-01

    Automated electronic sniffers may be useful for early detection of acute respiratory distress syndrome (ARDS) for institution of treatment or clinical trial screening. In a prospective cohort of 2929 critically ill patients, we retrospectively applied published sniffer algorithms for automated detection of acute lung injury to assess their utility in diagnosis of ARDS in the first 4 ICU days. Radiographic full-text reports were searched for "edema" OR ("bilateral" AND "infiltrate") and a more detailed algorithm for descriptions consistent with ARDS. Patients were flagged as possible ARDS if a radiograph met search criteria and had a PaO 2 /FiO 2 or SpO 2 /FiO 2 of 300 or 315, respectively. Test characteristics of the electronic sniffers and clinical suspicion of ARDS were compared to a gold standard of 2-physician adjudicated ARDS. Thirty percent of 2841 patients included in the analysis had gold standard diagnosis of ARDS. The simpler algorithm had sensitivity for ARDS of 78.9%, specificity of 52%, positive predictive value (PPV) of 41%, and negative predictive value (NPV) of 85.3% over the 4-day study period. The more detailed algorithm had sensitivity of 88.2%, specificity of 55.4%, PPV of 45.6%, and NPV of 91.7%. Both algorithms were more sensitive but less specific than clinician suspicion, which had sensitivity of 40.7%, specificity of 94.8%, PPV of 78.2%, and NPV of 77.7%. Published electronic sniffer algorithms for ARDS may be useful automated screening tools for ARDS and improve on clinical recognition, but they are limited to screening rather than diagnosis because their specificity is poor.

  16. Male patients with partial androgen insensitivity syndrome

    DEFF Research Database (Denmark)

    Hellmann, Philip; Christiansen, Peter; Johannsen, Trine Holm

    2012-01-01

    To describe the natural history of phenotype, growth and gonadal function in patients with partial androgen insensitivity syndrome.......To describe the natural history of phenotype, growth and gonadal function in patients with partial androgen insensitivity syndrome....

  17. Initial ventilator settings for critically ill patients

    OpenAIRE

    Kilickaya, Oguz; Gajic, Ognjen

    2013-01-01

    The lung-protective mechanical ventilation strategy has been standard practice for management of acute respiratory distress syndrome (ARDS) for more than a decade. Observational data, small randomized studies and two recent systematic reviews suggest that lung protective ventilation is both safe and potentially beneficial in patients who do not have ARDS at the onset of mechanical ventilation. Principles of lung-protective ventilation include: a) prevention of volutrauma (tidal volume 4 to 8 ...

  18. Extracellular histones play an inflammatory role in acid aspiration-induced acute respiratory distress syndrome.

    Science.gov (United States)

    Zhang, Yanlin; Wen, Zongmei; Guan, Li; Jiang, Ping; Gu, Tao; Zhao, Jinyuan; Lv, Xin; Wen, Tao

    2015-01-01

    Systemic inflammation is a key feature in acid aspiration-induced acute respiratory distress syndrome (ARDS), but the factors that trigger inflammation are unclear. The authors hypothesize that extracellular histones, a newly identified inflammatory mediator, play important roles in the pathogenesis of ARDS. The authors used a hydrochloric acid aspiration-induced ARDS model to investigate whether extracellular histones are pathogenic and whether targeting histones are protective. Exogenous histones and antihistone antibody were administered to mice. Heparin can bind to histones, so the authors studied whether heparin could protect from ARDS using cell and mouse models. Furthermore, the authors analyzed whether extracellular histones are clinically involved in ARDS patients caused by gastric aspiration. Extracellular histones in bronchoalveolar lavage fluid of acid-treated mice were significantly higher (1.832 ± 0.698) at 3 h after injury than in sham-treated group (0.63 ± 0.153; P = 0.0252, n = 5 per group). Elevated histones may originate from damaged lung cells and neutrophil infiltration. Exogenous histones aggravated lung injury, whereas antihistone antibody markedly attenuated the intensity of ARDS. Notably, heparin provided a similar protective effect against ARDS. Analysis of plasma from ARDS patients (n = 21) showed elevated histones were significantly correlated with the degree of ARDS and were higher in nonsurvivors (2.723 ± 0.2933, n = 7) than in survivors (1.725 ± 0.1787, P = 0.006, n = 14). Extracellular histones may play a contributory role toward ARDS by promoting tissue damage and systemic inflammation and may become a novel marker reflecting disease activity. Targeting histones by neutralizing antibody or heparin shows potent protective effects, suggesting a potentially therapeutic strategy.

  19. Effects of tidal volume on work of breathing during lung-protective ventilation in patients with acute lung injury and acute respiratory distress syndrome.

    Science.gov (United States)

    Kallet, Richard H; Campbell, Andre R; Dicker, Rochelle A; Katz, Jeffrey A; Mackersie, Robert C

    2006-01-01

    To assess the effects of step-changes in tidal volume on work of breathing during lung-protective ventilation in patients with acute lung injury (ALI) or the acute respiratory distress syndrome (ARDS). Prospective, nonconsecutive patients with ALI/ARDS. Adult surgical, trauma, and medical intensive care units at a major inner-city, university-affiliated hospital. Ten patients with ALI/ARDS managed clinically with lung-protective ventilation. Five patients were ventilated at a progressively smaller tidal volume in 1 mL/kg steps between 8 and 5 mL/kg; five other patients were ventilated at a progressively larger tidal volume from 5 to 8 mL/kg. The volume mode was used with a flow rate of 75 L/min. Minute ventilation was maintained constant at each tidal volume setting. Afterward, patients were placed on continuous positive airway pressure for 1-2 mins to measure their spontaneous tidal volume. Work of breathing and other variables were measured with a pulmonary mechanics monitor (Bicore CP-100). Work of breathing progressively increased (0.86 +/- 0.32, 1.05 +/- 0.40, 1.22 +/- 0.36, and 1.57 +/- 0.43 J/L) at a tidal volume of 8, 7, 6, and 5 mL/kg, respectively. In nine of ten patients there was a strong negative correlation between work of breathing and the ventilator-to-patient tidal volume difference (R = -.75 to -.998). : The ventilator-delivered tidal volume exerts an independent influence on work of breathing during lung-protective ventilation in patients with ALI/ARDS. Patient work of breathing is inversely related to the difference between the ventilator-delivered tidal volume and patient-generated tidal volume during a brief trial of unassisted breathing.

  20. High tidal volume decreases adult respiratory distress syndrome, atelectasis, and ventilator days compared with low tidal volume in pediatric burned patients with inhalation injury.

    Science.gov (United States)

    Sousse, Linda E; Herndon, David N; Andersen, Clark R; Ali, Arham; Benjamin, Nicole C; Granchi, Thomas; Suman, Oscar E; Mlcak, Ronald P

    2015-04-01

    Inhalation injury, which is among the causes of acute lung injury and acute respiratory distress syndrome (ARDS), continues to represent a significant source of mortality in burned patients. Inhalation injury often requires mechanical ventilation, but the ideal tidal volume strategy is not clearly defined in burned pediatric patients. The aim of this study was to determine the effects of low and high tidal volume on the number of ventilator days, ventilation pressures, and incidence of atelectasis, pneumonia, and ARDS in pediatric burned patients with inhalation injury within 1 year post burn injury. From 1986 to 2014, inhalation injury was diagnosed by bronchoscopy in pediatric burned patients (n = 932). Patients were divided into 3 groups: unventilated (n = 241), high tidal volume (HTV, 15 ± 3 mL/kg, n = 190), and low tidal volume (LTV, 9 ± 3 mL/kg, n = 501). High tidal volume was associated with significantly decreased ventilator days (p tidal volume significantly decreases ventilator days and the incidence of both atelectasis and ARDS compared with low tidal volume in pediatric burned patients with inhalation injury. Therefore, the use of HTV may interrupt sequences leading to lung injury in our patient population. Copyright © 2015 American College of Surgeons. Published by Elsevier Inc. All rights reserved.

  1. Activation of Coagulation and Fibrinolysis in Acute Respiratory Distress Syndrome: a Prospective Pilot Study

    Directory of Open Access Journals (Sweden)

    Agnese Ozolina

    2016-11-01

    Full Text Available Introduction: Coagulation and fibrinolysis remain sparsely addressed with regards to acute respiratory distress syndrome (ARDS. We hypothesized that ARDS development might be associated with changes in plasma coagulation and fibrinolysis. Our aim was to investigate the relationships between ARDS diagnosis and plasma concentrations of tissue factor (TF, tissue plasminogen activator (t-PA and plasminogen activator inhibitor-1 (PAI-1 in mechanically ventilated patients at increased risk of developing ARDS. Materials and Methods: We performed an ethically approved prospective observational pilot study. Inclusion criteria: patients with PaO2/FiO2 < 300 mmHg admitted to the intensive care unit (ICU for mechanical ventilation for 24 hours, or more, because of one or more disease conditions associated with increased risk of developing ARDS. Exclusion criteria: age below 18 years; cardiac disease. We sampled plasma prospectively and compared patients who developed ARDS with those who did not using descriptive statistics and chi-square analysis of baseline demographical and clinical data. We also analyzed plasma concentrations of TF, t-PA and PAI-1 at inclusion (T0 and on third (T3 and seventh day (T7 of the ICU stay with non-parametric statistics inclusive their sensitivity and specificity associated with the development of ARDS using receiver operating characteristic (ROC curve analysis. Statistical significance: p < 0.05.Results: Of 24 patients at risk, six developed mild ARDS and four of each moderate or severe ARDS, respectively, 3 ± 2 (Mean ± SD days after inclusion. Median plasma concentrations of TF and PAI-1 were significantly higher at T7 in patients with ARDS, as compared to non-ARDS. Simultaneously, we found moderate correlations between plasma concentrations of TF and PAI-1, TF and PaO2/FiO2 and PEEP and TF. TF plasma concentration was associated with ARDS with 71% sensitivity and 100% specificity, a cut off level of 145 pg/ml and AUC 0

  2. Mortality Trends of Acute Respiratory Distress Syndrome in the United States from 1999 to 2013.

    Science.gov (United States)

    Cochi, Shea E; Kempker, Jordan A; Annangi, Srinadh; Kramer, Michael R; Martin, Greg S

    2016-10-01

    Acute respiratory distress syndrome (ARDS) is an acute hypoxemic respiratory failure seen in critically ill patients after an inciting injury. The burden of ARDS mortality in the United States in recent years is not well characterized. In this study, we aimed to describe trends in the annual incidence of ARDS mortality in the United States from 1999 to 2013. We also describe demographic characteristics, geographic and seasonal trends, and other associated underlying causes of death in this population. Data on all deceased U.S. residents are available through the Multiple Cause of Death (MCOD) database of the National Center for Health Statistics. ARDS-related deaths were identified in the MCOD database using International Classification of Diseases, 10th Revision. Aggregate annual crude and age-adjusted mortality rates and mortality rate ratios were used to compare various demographic subpopulations. Over the 15-year period, the national ARDS-related age-adjusted mortality rate demonstrated an annual seasonal variation, peaking in winter. The annual rate decreased in a nonlinear fashion, with a plateau from 2010 to 2013. The ARDS-related age-adjusted mortality rate was 5.01 per 100,000 persons (95% confidence interval, 4.92-5.09) in 1999 and 2.82 per 100,000 persons (95% confidence interval, 2.76-2.88) in 2013. Males had a higher average ARDS-related mortality rate than did females. Asian/Pacific Islanders had the lowest average age-adjusted ARDS-related mortality rate, and black/African-American individuals, the highest. National age-adjusted ARDS-related mortality rates decreased between 1999 and 2013 in the United States, yet still show relative racial and sex disparities. However, death certificates largely underestimate the overall mortality burden from ARDS when compared with studies of clinically ascertained cases.

  3. A new definition for the acute respiratory distress syndrome.

    Science.gov (United States)

    Thompson, B Taylor; Moss, Marc

    2013-08-01

    Similar to other syndromes, patients are defined as having acute respiratory distress syndrome (ARDS) when they meet prespecified diagnostic criteria. These criteria have evolved over time, having gained and lost complexity, but the core principles have remained remarkably similar over the past 45 years. The specific diagnostic criteria allow clinicians and investigators to reliably identify patients with the syndrome of ARDS. ARDS is a form of acute diffuse lung injury occurring in patients with a predisposing risk factor. Lung injury is characterized by inflammation leading to increased endothelial and epithelial permeability and loss of aerated lung tissue resulting in hypoxemia and bilateral radiographic opacities on chest radiography. Additional physiological derangements include increased venous admixture and physiological dead space along with decreased respiratory system compliance. The corresponding pathological findings are lung edema, inflammation, hyaline membranes, and alveolar hemorrhage (i.e., diffuse alveolar damage). This article discusses the evolution of the definition of ARDS to the new Berlin definition of ARDS proposed in 2012 and its novel iterative refinement. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

  4. Acute Respiratory Distress Syndrome after Early Successful Primary Percutaneous Coronary Intervention Therapy in Acute Myocardial Infarction: A Case Report

    Directory of Open Access Journals (Sweden)

    Ho-Ming Su

    2005-02-01

    Full Text Available Acute respiratory distress syndrome (ARDS is characterized by acute-onset dyspnea, diffuse bilateral pulmonary infiltration, low pulmonary capillary wedge pressure (PCWP, and an arterial oxygen tension/ inspired oxygen fraction (PaO2/FiO2 ratio of less than 200 mmHg. Acute myocardial infarction (AMI, whether complicated by circulatory arrest, cardiogenic shock, and hypotension or not, was reported as an etiologic factor in the development of ARDS in the prethrombolytic era. In the thrombolytic era, two cases of AMI complicated with ARDS have been reported. ARDS in these two patients resulted from anaphylactic reaction to the thrombolytic agent and not from the hemodynamic consequences of AMI. Development of ARDS during the AMI period has not been reported after early successful primary percutaneous coronary intervention (PCI. Herein, we report a 61-year-old male patient with persistent chest pain who was diagnosed with Killip II anterior ST-segment elevation AMI. He was treated successfully with primary PCI 2.5 hours after the onset of chest pain. Unfortunately, on the third hospital day, acuteonset dyspnea (respiratory rate, 33 beats/min, fever (38.5°C, leukocytosis (white blood cell count, 18,360/μL, and diffuse bilateral pulmonary infiltration were noted. ARDS was diagnosed from the low PCWP (8 mmHg and a PaO2/FiO2 of less than 200 mmHg (160 mmHg. No usual causes of ARDS such as infection, aspiration, trauma, shock, or drug reactions were noted. We assumed that, in this particular patient, the systemic inflammatory response syndrome frequently induced by AMI might have caused this episode of ARDS. This may imply that AMI itself is a possible etiology of ARDS.

  5. A new horizon for the use of non-invasive ventilation in patients with acute respiratory distress syndrome.

    Science.gov (United States)

    Carron, Michele

    2016-09-01

    Non-invasive ventilation (NIV) has assumed an important role in the management of acute respiratory failure (ARF). NIV, compared with standard medical therapy, improves survival and reduces complications in selected patients with ARF. NIV represents the first-line intervention for some forms of ARF, such as chronic obstructive pulmonary disease (COPD) exacerbations and acute cardiogenic pulmonary edema. The use of NIV is also well supported for immunocompromised patients who are at high risk for infectious complications from endotracheal intubation. Selection of appropriate patients is crucial for optimizing NIV success rates. Appropriate ventilator settings, a well-fitting and comfortable interface, and a team skilled and experienced in managing NIV are key components to its success. In a recent issue of the Journal of the American Medical Association , Patel et al . reported the results of their single-center trial of 83 patients with acute respiratory distress syndrome (ARDS) who were randomly assigned to NIV delivered via a helmet or face mask. Patients assigned to the helmet group exhibited a significantly lower intubation rate and were more likely to survive through 90 days. This perspective reviews the findings of this trial in the context of current clinical practice and in light of data from the literature focused on the potential reasons for success of NIV delivered through a helmet compared to face mask. The implications for early management of patients with ARDS are likewise discussed.

  6. Acute respiratory distress syndrome: evaluation and management.

    Science.gov (United States)

    Cortés, I; Peñuelas, O; Esteban, A

    2012-03-01

    Acute respiratory distress syndrome (ARDS) is a life-threatening condition that affects patients admitted in the Intensive Care Units (ICUs) under mechanical ventilation. ARDS is a process of non-hydrostatic pulmonary edema and hypoxemia associated with a variety of conditions, resulting in a direct (e.g., pneumonia) or indirect (e.g., sepsis) lung injury and is associated with a significant morbidity and mortality. A large body of clinical and basic research has focused in ventilatory strategies and novel pharmacological therapies but, nowadays, treatment is mainly supportive. Mechanical ventilation is the hallmark of the management of these patients. In the last decades, the recognition that mechanical ventilation can contribute to harming the lung has changed the goals of this therapy and has driven research to focus in ventilatory strategies that mitigate lung injury. This review emphasizes clinical aspects in the evaluation and management of ARDS in the ICUs and updates the latest advances in these therapies.

  7. Acute Respiratory Distress Syndrome: Insights Gained from Clinical and Translational Research

    Directory of Open Access Journals (Sweden)

    Marija Kojicic

    2009-10-01

    Full Text Available Acute lung injury and its more severe form acute respiratory distress syndrome (ARDS are characterized by diffuse impairment of alveolocapillary membrane in the settings of different predisposing conditions such as sepsis, trauma and shock. Many intrahospital exposures, including aspiration, delayed resuscitation, high tidal volume mechanical ventilation and non critical use of transfusions may contribute or worsen ARDS. Therapy is targeted to treatment of predisposing condition, life supportive measures and prevention of nosocomial complications. Rigorous adherence to lung-protective mechanical ventilation is critical to prevent ventilator induced lung injury and decrease mortality. Although survival of ARDS patients has improved in the last decades ARDS mortality rates are still high and survivors encounter significant physical and psychological impairments

  8. Acute respiratory distress syndrome assessment after traumatic brain injury

    Directory of Open Access Journals (Sweden)

    Shahrooz Kazemi

    2016-01-01

    Full Text Available Background: Acute respiratory distress syndrome (ARDS is one of the most important complications associated with traumatic brain injury (TBI. ARDS is caused by inflammation of the lungs and hypoxic damage with lung physiology abnormalities associated with acute respiratory distress syndrome. Aim of this study is to determine the epidemiology of ARDS and the prevalence of risk factors. Methods: This prospective study performed on patients with acute traumatic head injury hospitalization in the intensive care unit of the Shohaday-e Haftom-e-Tir Hospital (September 2012 to September 2013 done. About 12 months, the data were evaluated. Information including age, sex, education, employment, drug and alcohol addiction, were collected and analyzed. The inclusion criteria were head traumatic patients and exclusion was the patients with chest trauma. Questionnaire was designed with doctors supervision of neurosurgery. Then the collected data were analysis. Results: In this study, the incidence of ARDS was 23.8% and prevalence of metabolic acidosis was 31.4%. Most injury with metabolic acidosis was Subarachnoid hemorrhage (SAH 48 (60% and Subdural hemorrhage (SDH was Next Level with 39 (48% Correlation between Glasgow Coma Scale (GCS and Respiratory Distress Syndrome (ARDS were significantly decreased (P< 0.0001. The level of consciousness in patients with skull fractures significantly lower than those without fractures (P= 0.009 [(2.3±4.6 vs (4.02±7.07]. Prevalence of metabolic acidosis during hospitalization was 80 patients (31.4%. Conclusion: Acute respiratory distress syndrome is a common complication of traumatic brain injury. Management and treatment is essential to reduce the mortality. In this study it was found the age of patients with ARDS was higher than patients without complications. ARDS risk factor for high blood pressure was higher in men. Most victims were pedestrians. The most common injury associated with ARDS was SDH. Our analysis

  9. Management of moyamoya syndrome in patients with Noonan syndrome.

    Science.gov (United States)

    Gupta, Mihir; Choudhri, Omar A; Feroze, Abdullah H; Do, Huy M; Grant, Gerald A; Steinberg, Gary K

    2016-06-01

    A few isolated reports have described an association between Noonan syndrome and cerebrovascular abnormalities, including moyamoya syndrome. These reports have been limited to pediatric patients presenting with recurrent transient ischemic attacks (TIA) or headaches. Management has primarily been pharmacologic, with only one prior report of surgical revascularization to our knowledge. We report four cases of Noonan syndrome patients presenting with headaches and/or sensorimotor strokes in childhood that caused unilateral sensorimotor impairment. Cerebral angiography and MRI revealed bilateral moyamoya syndrome. All patients underwent successful bilateral extracranial-to-intracranial revascularization. The first patient was a 10-year-old girl who presented following a hemorrhagic stroke and recovered well after indirect bypass. The second patient was an adult with a history of childhood stroke whose symptoms progressed in adulthood. She underwent a direct bypass and improved, but continued to experience TIA at her 4 year follow-up. The third patient was a 7-year-old girl with headaches and a new onset TIA who failed pharmacological therapy and subsequently underwent bilateral indirect bypass. The fourth patient was a 24-year-old woman with worsening headaches and an occluded left middle cerebral artery from unilateral moyamoya syndrome. A left sided direct bypass was completed given delayed MRI perfusion with poor augmentation. To our knowledge these are the first reported surgical cases of combined Noonan and moyamoya syndrome. These cases highlight the need to recognize moyamoya syndrome in patients with Noonan syndrome. Early surgical revascularization should be pursued in order to prevent symptom progression. Copyright © 2015 Elsevier Ltd. All rights reserved.

  10. Pulmonary hypertension due to acute respiratory distress syndrome

    Directory of Open Access Journals (Sweden)

    S.A. Ñamendys-Silva

    2014-10-01

    Full Text Available Our aims were to describe the prevalence of pulmonary hypertension in patients with acute respiratory distress syndrome (ARDS, to characterize their hemodynamic cardiopulmonary profiles, and to correlate these parameters with outcome. All consecutive patients over 16 years of age who were in the intensive care unit with a diagnosis of ARDS and an in situ pulmonary artery catheter for hemodynamic monitoring were studied. Pulmonary hypertension was diagnosed when the mean pulmonary artery pressure was >25 mmHg at rest with a pulmonary artery occlusion pressure or left atrial pressure <15 mmHg. During the study period, 30 of 402 critically ill patients (7.46% who were admitted to the ICU fulfilled the criteria for ARDS. Of the 30 patients with ARDS, 14 met the criteria for pulmonary hypertension, a prevalence of 46.6% (95% CI; 28-66%. The most common cause of ARDS was pneumonia (56.3%. The overall mortality was 36.6% and was similar in patients with and without pulmonary hypertension. Differences in patients' hemodynamic profiles were influenced by the presence of pulmonary hypertension. The levels of positive end-expiratory pressure and peak pressure were higher in patients with pulmonary hypertension, and the PaCO2 was higher in those who died. The level of airway pressure seemed to influence the onset of pulmonary hypertension. Survival was determined by the severity of organ failure at admission to the intensive care unit.

  11. Clinical effect of alprostadil in patients with septic shock associated with acute respiratory distress syndrome

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    Li-ping LIU

    2017-10-01

    Full Text Available Objective To evaluate the clinical efficacy of alprostadil in patients with septic shock associated with acute respiratory distress syndrome (ARDS, and to explore its possible mechanism. Methods From January 2015 to June 2016, patients with septic shock associated with ARDS and meeting the inclusion criteria were involved in the study in department of critical care medicine in First Hospital of Lanzhou University and randomly divided into the control group and alprostadil group. The standard treatment was given in control group, alprostadil 10μg 2/d was given in alprostadil group on base of standard treatment. Monitoring indexes were recorded in 1, 3 and 6 days after enrollment. General condition of patients, APACHE Ⅱ score, ventilator conditions (PO2, PCO2, RR, PEEP, FiO2, oxygenation index, airway resistance, lung compliance, mechanical ventilation time, ICU stay time, hospital follow-up, 28-day follow-up, immune index (CD4+/CD8+, inflammatory markers (CRP, PCT, IL-6 were monitored. Results Sixty-five patients were included in this study, 32 in control group and 33 in alprostadil group. At 3 and 6 days after the treatment, APACHE Ⅱ score, respiratory rate (RR, the inspired oxygen concentration (FiO2, airway resistance, and C reactive protein (CRP, procalcitonin (PCT -6 and interleukin (IL-6 levels significantly decreased, compared with pretreatment and 1 day posttreatment, in the two groups and lower in alprostadil group than in the control group on the 6th day (P<0.05; at the same time, these indexes such as arterial partial pressure of oxygen (PaO2, lung compliance, oxygenation index, CD4+/CD8+ significantly increased 3 and 6 days after the treatment compared with pretreatment and 1 day posttreatment in the two groups, and on the 6th day, significantly higher in the alprostadil group than in the control group (P<0.05. Time of mechanical ventilation, ICU stay and hospital stay in the alprostadil group was respectively lower than that in

  12. Clinical Practice Guideline of Acute Respiratory Distress Syndrome

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    Young-Jae Cho

    2016-05-01

    Full Text Available There is no well-stated practical guideline for mechanically ventilated patients with or without acute respiratory distress syndrome (ARDS. We generate strong (1 and weak (2 grade of recommendations based on high (A, moderate (B and low (C grade in the quality of evidence. In patients with ARDS, we recommend low tidal volume ventilation (1A and prone position if it is not contraindicated (1B to reduce their mortality. However, we did not support high-frequency oscillatory ventilation (1B and inhaled nitric oxide (1A as a standard treatment. We also suggest high positive end-expiratory pressure (2B, extracorporeal membrane oxygenation as a rescue therapy (2C, and neuromuscular blockage for 48 hours after starting mechanical ventilation (2B. The application of recruitment maneuver may reduce mortality (2B, however, the use of systemic steroids cannot reduce mortality (2B. In mechanically ventilated patients, we recommend light sedation (1B and low tidal volume even without ARDS (1B and suggest lung protective ventilation strategy during the operation to lower the incidence of lung complications including ARDS (2B. Early tracheostomy in mechanically ventilated patients can be performed only in limited patients (2A. In conclusion, of 12 recommendations, nine were in the management of ARDS, and three for mechanically ventilated patients.

  13. Antioxidant treatment with N-acetylcysteine during adult respiratory distress syndrome

    DEFF Research Database (Denmark)

    Jepsen, S; Herlevsen, P; Knudsen, P

    1992-01-01

    OBJECTIVE: To examine whether the antioxidant N-acetylcysteine could ameliorate the course of the adult respiratory distress syndrome (ARDS) in man. DESIGN: Randomized, double-blind, placebo-controlled study. SETTING: Medical and surgical ICU in a regional hospital. PATIENTS: Sixty-six ICU patients...

  14. Identification and validation of distinct biological phenotypes in patients with acute respiratory distress syndrome by cluster analysis.

    Science.gov (United States)

    Bos, L D; Schouten, L R; van Vught, L A; Wiewel, M A; Ong, D S Y; Cremer, O; Artigas, A; Martin-Loeches, I; Hoogendijk, A J; van der Poll, T; Horn, J; Juffermans, N; Calfee, C S; Schultz, M J

    2017-10-01

    We hypothesised that patients with acute respiratory distress syndrome (ARDS) can be clustered based on concentrations of plasma biomarkers and that the thereby identified biological phenotypes are associated with mortality. Consecutive patients with ARDS were included in this prospective observational cohort study. Cluster analysis of 20 biomarkers of inflammation, coagulation and endothelial activation provided the phenotypes in a training cohort, not taking any outcome data into account. Logistic regression with backward selection was used to select the most predictive biomarkers, and these predicted phenotypes were validated in a separate cohort. Multivariable logistic regression was used to quantify the independent association with mortality. Two phenotypes were identified in 454 patients, which we named 'uninflamed' (N=218) and 'reactive' (N=236). A selection of four biomarkers (interleukin-6, interferon gamma, angiopoietin 1/2 and plasminogen activator inhibitor-1) could be used to accurately predict the phenotype in the training cohort (area under the receiver operating characteristics curve: 0.98, 95% CI 0.97 to 0.99). Mortality rates were 15.6% and 36.4% (p<0.001) in the training cohort and 13.6% and 37.5% (p<0.001) in the validation cohort (N=207). The 'reactive phenotype' was independent from confounders associated with intensive care unit mortality (training cohort: OR 1.13, 95% CI 1.04 to 1.23; validation cohort: OR 1.18, 95% CI 1.06 to 1.31). Patients with ARDS can be clustered into two biological phenotypes, with different mortality rates. Four biomarkers can be used to predict the phenotype with high accuracy. The phenotypes were very similar to those found in cohorts derived from randomised controlled trials, and these results may improve patient selection for future clinical trials targeting host response in patients with ARDS. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please

  15. Outcome prediction in pneumonia induced ALI/ARDS by clinical features and peptide patterns of BALF determined by mass spectrometry.

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    Jochen Frenzel

    Full Text Available BACKGROUND: Peptide patterns of bronchoalveolar lavage fluid (BALF were assumed to reflect the complex pathology of acute lung injury (ALI/acute respiratory distress syndrome (ARDS better than clinical and inflammatory parameters and may be superior for outcome prediction. METHODOLOGY/PRINCIPAL FINDINGS: A training group of patients suffering from ALI/ARDS was compiled from equal numbers of survivors and nonsurvivors. Clinical history, ventilation parameters, Murray's lung injury severity score (Murray's LISS and interleukins in BALF were gathered. In addition, samples of bronchoalveolar lavage fluid were analyzed by means of hydrophobic chromatography and MALDI-ToF mass spectrometry (MALDI-ToF MS. Receiver operating characteristic (ROC analysis for each clinical and cytokine parameter revealed interleukin-6>interleukin-8>diabetes mellitus>Murray's LISS as the best outcome predictors. Outcome predicted on the basis of BALF levels of interleukin-6 resulted in 79.4% accuracy, 82.7% sensitivity and 76.1% specificity (area under the ROC curve, AUC, 0.853. Both clinical parameters and cytokines as well as peptide patterns determined by MALDI-ToF MS were analyzed by classification and regression tree (CART analysis and support vector machine (SVM algorithms. CART analysis including Murray's LISS, interleukin-6 and interleukin-8 in combination was correct in 78.0%. MALDI-ToF MS of BALF peptides did not reveal a single identifiable biomarker for ARDS. However, classification of patients was successfully achieved based on the entire peptide pattern analyzed using SVM. This method resulted in 90% accuracy, 93.3% sensitivity and 86.7% specificity following a 10-fold cross validation (AUC = 0.953. Subsequent validation of the optimized SVM algorithm with a test group of patients with unknown prognosis yielded 87.5% accuracy, 83.3% sensitivity and 90.0% specificity. CONCLUSIONS/SIGNIFICANCE: MALDI-ToF MS peptide patterns of BALF, evaluated by appropriate

  16. Acute respiratory distress syndrome

    Science.gov (United States)

    ... page: //medlineplus.gov/ency/article/000103.htm Acute respiratory distress syndrome To use the sharing features on this page, please enable JavaScript. Acute respiratory distress syndrome (ARDS) is a life-threatening lung ...

  17. Lung recruitment maneuver effects on respiratory mechanics and extravascular lung water index in patients with acute respiratory distress syndrome.

    Science.gov (United States)

    Zhang, Jian-Guo; Chen, Xiao-Juan; Liu, Fen; Zeng, Zhen-Guo; Qian, Ke-Jian

    2011-01-01

    Animal experiments showed that recruitment maneuver (RM) and protective ventilation strategy of the lung could improve oxygenation and reduce extravascular lung water. This study was to investigate the effects of RM on respiratory mechanics and extravascular lung water index (EVLWI) in patients with acute respiratory distress syndrome (ARDS). Thirty patients with ARDS were randomized into a RM group and a non-RM group. In the RM group, after basic mechanical ventilation stabilized for 30 minutes, RM was performed and repeated once every 12 hours for 3 days. In the non-RM group, lung protective strategy was conducted without RM. Oxygenation index (PaO2/FiO2), peak inspiratory pressure (PIP), Plateau pressure (Pplat), static pulmonary compliance (Cst) and EVLWI of patients before treatment and at 12, 24, 48, 72 hours after the treatment were measured and compared between the groups. Hemodynamic changes were observed before and after RM. One-way ANOVA, Student's t test and Fisher's exact test were used to process the data. The levels of PaO2/FiO2 and Cst increased after treatment in the two groups, but they were higher in the RM group than in the non-RM group (P0.05). RM could reduce EVLWI, increase oxygenation and lung compliance. The effect of RM on hemodynamics was transient.

  18. Mechanical ventilation strategies for intensive care unit patients without acute lung injury or acute respiratory distress syndrome: a systematic review and network meta-analysis.

    Science.gov (United States)

    Guo, Lei; Wang, Weiwei; Zhao, Nana; Guo, Libo; Chi, Chunjie; Hou, Wei; Wu, Anqi; Tong, Hongshuang; Wang, Yue; Wang, Changsong; Li, Enyou

    2016-07-22

    It has been shown that the application of a lung-protective mechanical ventilation strategy can improve the prognosis of patients with acute lung injury (ALI) or acute respiratory distress syndrome (ARDS). However, the optimal mechanical ventilation strategy for intensive care unit (ICU) patients without ALI or ARDS is uncertain. Therefore, we performed a network meta-analysis to identify the optimal mechanical ventilation strategy for these patients. We searched the Cochrane Central Register of Controlled Trials (CENTRAL) in the Cochrane Library, EMBASE, MEDLINE, CINAHL, and Web of Science for studies published up to July 2015 in which pulmonary compliance or the partial pressure of arterial oxygen/fraction of inspired oxygen (PaO2/FIO2) ratio was assessed in ICU patients without ALI or ARDS, who received mechanical ventilation via different strategies. The data for study characteristics, methods, and outcomes were extracted. We assessed the studies for eligibility, extracted the data, pooled the data, and used a Bayesian fixed-effects model to combine direct comparisons with indirect evidence. Seventeen randomized controlled trials including a total of 575 patients who received one of six ventilation strategies were included for network meta-analysis. Among ICU patients without ALI or ARDS, strategy C (lower tidal volume (VT) + higher positive end-expiratory pressure (PEEP)) resulted in the highest PaO2/FIO2 ratio; strategy B (higher VT + lower PEEP) was associated with the highest pulmonary compliance; strategy A (lower VT + lower PEEP) was associated with a shorter length of ICU stay; and strategy D (lower VT + zero end-expiratory pressure (ZEEP)) was associated with the lowest PaO2/FiO2 ratio and pulmonary compliance. For ICU patients without ALI or ARDS, strategy C (lower VT + higher PEEP) was associated with the highest PaO2/FiO2 ratio. Strategy B (higher VT + lower PEEP) was superior to the other strategies in improving pulmonary

  19. Recent advances in mechanical ventilation in patients with acute respiratory distress syndrome

    Directory of Open Access Journals (Sweden)

    Nuttapol Rittayamai

    2015-03-01

    Full Text Available Acute respiratory distress syndrome (ARDS is characterised by different degrees of severity and different stages. Understanding these differences can help to better adapt the ventilatory settings to protect the lung from ventilator-induced lung injury by reducing hyperinflation or keeping the lung open when it is possible. The same therapies may be useful and beneficial in certain forms of ARDS, and risky or harmful at other stages: this includes high positive end-expiratory pressure, allowance of spontaneous breathing activity or use of noninvasive ventilation. The severity of the disease is the primary indicator to individualise treatment. Monitoring tools such as oesophageal pressure or lung volume measurements may also help to set the ventilator. At an earlier stage, an adequate lung protective strategy may also help to prevent the development of ARDS.

  20. Screening for Cushing's syndrome in obese patients

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    Ozay Tiryakioglu

    2010-01-01

    Full Text Available OBJECTIVES: The aim of this study was to examine the frequency of Cushing's syndrome (CS in obese patients devoid of specific clinical symptoms of Cushing's syndrome. METHODS: A total of 150 obese patients (129 female, 21 male; mean age 44.41 ± 13.34 yr; mean BMI 35.76 ± 7.13 were included in the study. As a first screening step, we measured 24-h urinary free cortisol (UFC. An overnight 1-mg dexamethasone suppression test was also performed on all patients. Urinary free cortisol levels above 100 μg/24 h were considered to be abnormal. Suppression of serum cortisol 100 μg/24 h were recorded in 37 patients (24%. Cushing's syndrome was diagnosed in 14 of the 150 patients (9.33%. Etiologic reasons for Cushing's syndrome were pituitary microadenoma (9 patients, adrenocortical adenoma (3 patients, and adrenocortical carcinoma (1 patient. CONCLUSION: A significant proportion (9.33% of patients with simple obesity were found to have Cushing's syndrome. These findings argue that obese patients should be routinely screened for Cushing's syndrome.

  1. Low Tidal Volume Ventilation in Patients without Acute Respiratory Distress Syndrome: A Paradigm Shift in Mechanical Ventilation

    Directory of Open Access Journals (Sweden)

    Jed Lipes

    2012-01-01

    Full Text Available Protective ventilation with low tidal volume has been shown to reduce morbidity and mortality in patients suffering from acute lung injury (ALI and acute respiratory distress syndrome (ARDS. Low tidal volume ventilation is associated with particular clinical challenges and is therefore often underutilized as a therapeutic option in clinical practice. Despite some potential difficulties, data have been published examining the application of protective ventilation in patients without lung injury. We will briefly review the physiologic rationale for low tidal volume ventilation and explore the current evidence for protective ventilation in patients without lung injury. In addition, we will explore some of the potential reasons for its underuse and provide strategies to overcome some of the associated clinical challenges.

  2. Low Tidal Volume Ventilation in Patients without Acute Respiratory Distress Syndrome: A Paradigm Shift in Mechanical Ventilation

    Science.gov (United States)

    Lipes, Jed; Bojmehrani, Azadeh; Lellouche, Francois

    2012-01-01

    Protective ventilation with low tidal volume has been shown to reduce morbidity and mortality in patients suffering from acute lung injury (ALI) and acute respiratory distress syndrome (ARDS). Low tidal volume ventilation is associated with particular clinical challenges and is therefore often underutilized as a therapeutic option in clinical practice. Despite some potential difficulties, data have been published examining the application of protective ventilation in patients without lung injury. We will briefly review the physiologic rationale for low tidal volume ventilation and explore the current evidence for protective ventilation in patients without lung injury. In addition, we will explore some of the potential reasons for its underuse and provide strategies to overcome some of the associated clinical challenges. PMID:22536499

  3. Waardenburg syndrome in four Mexican patients.

    Science.gov (United States)

    Aguilar Caso, Sandra I; Ortiz Nieva, Gabriela

    2009-01-01

    Waardenburg syndrome is a hereditary auditory-pigmentary syndrome. The major features include pigmentary disturbances and congenital deafness. Clinical findings are extremely variable, not only at the authors' institution but also in the literature. The authors describe four patients who presented with various clinical features and different genetic pedigree penetration.

  4. Vaginal rhabdomyosarcoma in a patient with Noonan syndrome.

    OpenAIRE

    Khan, S; McDowell, H; Upadhyaya, M; Fryer, A

    1995-01-01

    This is the first report of a Noonan syndrome patient who has had a vaginal rhabdomyosarcoma. Recent reports of Noonan syndrome patients with leukaemia have prompted speculation that there may be a slightly increased malignancy risk associated with this syndrome.

  5. Plasma biomarker analysis in pediatric ARDS: generating future framework from a pilot randomized control trial of methylprednisoloneA framework for identifying plasma biomarkers related to clinical outcomes in pediatric ARDS

    Directory of Open Access Journals (Sweden)

    Dai eKimura

    2016-03-01

    Full Text Available Objective: Lung injury activates multiple pro-inflammatory pathways, including neutrophils, epithelial and endothelial injury, and coagulation factors leading to acute respiratory distress syndrome (ARDS. Low-dose methylprednisolone therapy (MPT improved oxygenation and ventilation in early pediatric ARDS without altering duration of mechanical ventilation or mortality. We evaluated the effects of MPT on biomarkers of endothelial (Ang-2, sICAM-1 or epithelial (sRAGE injury, neutrophil activation (MMP-8, and coagulation (PAI-1. Design: Double-blind, placebo-controlled randomized trialSetting: Tertiary-care Pediatric Intensive Care Unit Patients: Mechanically ventilated children (0-18 years with early ARDS.Interventions: Blood samples were collected on Days 0 (before MPT, 7, and 14 during low-dose MPT (n=17 vs. placebo (n=18 therapy. The MPT group received a 2mg/kg loading dose followed by 1mg/kg/day continuous infusions from days 1-7, tapered off over 7 days; placebo group received equivalent amounts of 0.9% saline. We analyzed plasma samples using a multiplex assay for 5 biomarkers of ARDS. Multiple regression models were constructed to predict associations between changes in biomarkers and the clinical outcomes reported earlier including: P/F ratio on days 8&9, plateau pressure on days 1&2, PaCO2 on days 2&3, racemic epinephrine following extubation, and supplemental oxygen at ICU discharge.Results: No differences occurred in biomarker concentrations between the groups on Day 0. On Day 7, reduction in MMP-8 levels (p=0.0016 occurred in the MPT group, whereas increases in sICAM-1 levels (p=0.0005 occurred in the placebo group (no increases in sICAM-1 in the MPT group. sRAGE levels decreased in both MPT and placebo groups (p<0.0001 from Day 0 to Day 7. On Day 7, sRAGE levels were positively correlated with MPT group PaO2/FiO2 ratios on Day 8 (r=0.93, p=0.024. O2 requirements at ICU transfer positively correlated with Day 7 MMP-8 (r=0.85, p=0

  6. RESISTANT HYPERTENSION IN A PATIENT WITH METABOLIC SYNDROME

    OpenAIRE

    O. M. Drapkina; J. S. Sibgatullina

    2016-01-01

    Clinical case of resistant hypertension in a patient with metabolic syndrome is presented. Features of hypertension in metabolic syndrome and features of metabolic syndrome in women of pre- and postmenopausal age are also considered. Understanding the features of metabolic syndrome in women, as well as features of hypertension and metabolic syndrome will improve the results of treatment in patients with resistant hypertension.

  7. Twenty-two patients with Sotos' syndrome

    International Nuclear Information System (INIS)

    Miyake, Shota; Tada, Hiroshi; Hayashi, Michiko; Iwamoto, Hiroko; Fukushima, Yoshimitsu; Kuroki, Yoshikazu

    1985-01-01

    A retrospective study of 22 patients with Sotos' syndrome (17 boys and 5 girls) seen in the past 14 years was made respecting the age of the patients at the first visit, the age of the patients' parents at birth, neonatal abnormalities, neonatal physical characteristics, facial expression characteristics of this syndrome, growth index, and CT findings. CT showed ventricular dilation in all patients (moderate or extreme dilation in 87%, and the association of structure abnormality in the median part in many patients), subdural edema in 4 and arachnoid cyst in 2. (Namekawa, K.)

  8. Acute respiratory distress syndrome: the Berlin Definition.

    Science.gov (United States)

    Ranieri, V Marco; Rubenfeld, Gordon D; Thompson, B Taylor; Ferguson, Niall D; Caldwell, Ellen; Fan, Eddy; Camporota, Luigi; Slutsky, Arthur S

    2012-06-20

    The acute respiratory distress syndrome (ARDS) was defined in 1994 by the American-European Consensus Conference (AECC); since then, issues regarding the reliability and validity of this definition have emerged. Using a consensus process, a panel of experts convened in 2011 (an initiative of the European Society of Intensive Care Medicine endorsed by the American Thoracic Society and the Society of Critical Care Medicine) developed the Berlin Definition, focusing on feasibility, reliability, validity, and objective evaluation of its performance. A draft definition proposed 3 mutually exclusive categories of ARDS based on degree of hypoxemia: mild (200 mm Hg < PaO2/FIO2 ≤ 300 mm Hg), moderate (100 mm Hg < PaO2/FIO2 ≤ 200 mm Hg), and severe (PaO2/FIO2 ≤ 100 mm Hg) and 4 ancillary variables for severe ARDS: radiographic severity, respiratory system compliance (≤40 mL/cm H2O), positive end-expiratory pressure (≥10 cm H2O), and corrected expired volume per minute (≥10 L/min). The draft Berlin Definition was empirically evaluated using patient-level meta-analysis of 4188 patients with ARDS from 4 multicenter clinical data sets and 269 patients with ARDS from 3 single-center data sets containing physiologic information. The 4 ancillary variables did not contribute to the predictive validity of severe ARDS for mortality and were removed from the definition. Using the Berlin Definition, stages of mild, moderate, and severe ARDS were associated with increased mortality (27%; 95% CI, 24%-30%; 32%; 95% CI, 29%-34%; and 45%; 95% CI, 42%-48%, respectively; P < .001) and increased median duration of mechanical ventilation in survivors (5 days; interquartile [IQR], 2-11; 7 days; IQR, 4-14; and 9 days; IQR, 5-17, respectively; P < .001). Compared with the AECC definition, the final Berlin Definition had better predictive validity for mortality, with an area under the receiver operating curve of 0.577 (95% CI, 0.561-0.593) vs 0.536 (95% CI, 0.520-0.553; P

  9. Renal tumor leading to acute respiratory distress syndrome – a rare ...

    African Journals Online (AJOL)

    Arun Kumar Agnihotri

    renal cell carcinoma (RCC). KEY WORDS: ARDS; Renal tumor; Adult respiratory distress syndrome. INTRODUCTIONᴪ. ARDS due to ... unable to maintain saturation in spite of high flow ... Blood investigations showed mild leukocytosis.

  10. Surfactant disaturated-phosphatidylcholine kinetics in acute respiratory distress syndrome by stable isotopes and a two compartment model

    Directory of Open Access Journals (Sweden)

    Cogo Paola E

    2007-02-01

    Full Text Available Abstract Background In patients with acute respiratory distress syndrome (ARDS, it is well known that only part of the lungs is aerated and surfactant function is impaired, but the extent of lung damage and changes in surfactant turnover remain unclear. The objective of the study was to evaluate surfactant disaturated-phosphatidylcholine turnover in patients with ARDS using stable isotopes. Methods We studied 12 patients with ARDS and 7 subjects with normal lungs. After the tracheal instillation of a trace dose of 13C-dipalmitoyl-phosphatidylcholine, we measured the 13C enrichment over time of palmitate residues of disaturated-phosphatidylcholine isolated from tracheal aspirates. Data were interpreted using a model with two compartments, alveoli and lung tissue, and kinetic parameters were derived assuming that, in controls, alveolar macrophages may degrade between 5 and 50% of disaturated-phosphatidylcholine, the rest being lost from tissue. In ARDS we assumed that 5–100% of disaturated-phosphatidylcholine is degraded in the alveolar space, due to release of hydrolytic enzymes. Some of the kinetic parameters were uniquely determined, while others were identified as lower and upper bounds. Results In ARDS, the alveolar pool of disaturated-phosphatidylcholine was significantly lower than in controls (0.16 ± 0.04 vs. 1.31 ± 0.40 mg/kg, p de novo synthesis of disaturated-phosphatidylcholine were also significantly lower, while mean resident time in lung tissue was significantly higher in ARDS than in controls. Recycling was 16.2 ± 3.5 in ARDS and 31.9 ± 7.3 in controls (p = 0.08. Conclusion In ARDS the alveolar pool of surfactant is reduced and disaturated-phosphatidylcholine turnover is altered.

  11. Extravascular lung water and the pulmonary vascular permeability index may improve the definition of ARDS

    OpenAIRE

    Perel, Azriel

    2013-01-01

    The recent Berlin definition has made some improvements in the older definition of acute respiratory distress syndrome (ARDS), although the concepts and components of the definition remained largely unchanged. In an effort to improve both predictive and face validity, the Berlin panel has examined a number of additional measures that may reflect increased pulmonary vascular permeability, including extravascular lung water. The panel concluded that although extravascular lung water has improve...

  12. A Recruiting Maneuver Algorithm in Patients with Early Acute Respiratory Distress Syndrome

    Directory of Open Access Journals (Sweden)

    D. I. Levikov

    2011-01-01

    Full Text Available Objective: to evaluate the efficiency of a recruiting maneuver (RM and adjustment of positive end-expiratory pressure (PEEP in patients with early acute respiratory distress syndrome (ARDS. Subjects and methods. The study enrolled 16 patients (14 men and 2 women aged 46 to 78 years (range 62±5.6 years with ARDS of various genesis. RM was made, by stepwisely increasing PEEP and inspiratory pressure under the control of dynamic lung compliance and hemodynamic parameters. The values of blood gas composition and hemodynamics were determined during the study. Results. RM caused an increase in oxygenation index (OI from 153.5±48.3 to 348.5±53.2 mm Hg. Oxygenation values returned to the baseline levels 30—40 min after the PEEP was set at the closure point of +2 cm H2O. If the set PEEP was 8—10 cm H2O higher than the objective, the effect of RM was retained for as long as 24 hours. When RM was performed using the maximum pressure of 50—60 cm H2O, the cardiac index (CI was lower in all the patients and 30—50% of the baseline values were achieved in all cases, which required the optimization of cardiotonic therapy. The time of this pronounced reduction in cardiac output with RM was not longer than 5 min. After RM, during mechanical ventilation with 18—26 cm H2O PEEP, the CI did not practically differ from the baseline values (3.31±0.41 and 3.37±0.36 l/min/m2, respectively, though the dopamine dose required to maintain normal hemodynamics was somewhat higher (7.5±2.3 and 6.3±2.6 ^g/kg/min. Conclusion. Analysis of the given cases suggests that RM is highly effective in patients at the early stages of acute lung injury. The duration of RM effects may depend on the set PEEP level in individual cases. Setting PEEP at a level of +2—4 cm H2O fails to prevent repeated alveolar derecruitment in a number of patients. In these cases, it is expedient to individually adjust PEEP levels, by taking into account the long-term changes in OI and Cdyn. In

  13. Alternative and Natural Therapies for Acute Lung Injury and Acute Respiratory Distress Syndrome

    Directory of Open Access Journals (Sweden)

    Vipul J. Patel

    2018-01-01

    Full Text Available Introduction. Acute respiratory distress syndrome (ARDS is a complex clinical syndrome characterized by acute inflammation, microvascular damage, and increased pulmonary vascular and epithelial permeability, frequently resulting in acute respiratory failure and death. Current best practice for ARDS involves “lung-protective ventilation,” which entails low tidal volumes and limiting the plateau pressures in mechanically ventilated patients. Although considerable progress has been made in understanding the pathogenesis of ARDS, little progress has been made in the development of specific therapies to combat injury and inflammation. Areas Covered. In recent years, several natural products have been studied in experimental models and have been shown to inhibit multiple inflammatory pathways associated with acute lung injury and ARDS at a molecular level. Because of the pleiotropic effects of these agents, many of them also activate antioxidant pathways through nuclear factor erythroid-related factor 2, thereby targeting multiple pathways. Several of these agents are prescribed for treatment of inflammatory conditions in the Asian subcontinent and have shown to be relatively safe. Expert Commentary. Here we review natural remedies shown to attenuate lung injury and inflammation in experimental models. Translational human studies in patients with ARDS may facilitate treatment of this devastating disease.

  14. Supernumerary teeth in non-syndromic patients

    Energy Technology Data Exchange (ETDEWEB)

    Mali, Santosh; Karjodkar, Freny Rashmiraj; Sontakke, Subodh; Sansare, Kaustubh [Nair Hospital Dental College, Maharashtra (India)

    2012-03-15

    Hyperdontia or supernumerary teeth without associated syndrome is a rare phenomenon, as supernumerary teeth are usually associated with cleft lip and palate or other syndromes such as Gardner's syndrome, cleidocranial dysplasia, and so on. Five patients with supernumerary teeth visited our department. They had no familial history or other pathology, certain treatment protocols was modified due to the presence of supernumerary teeth. Non-syndromic supernumerary teeth, if asymptomatic, need to have periodical radiographic observation. If they showed no variation as they impacted in the jaw, careful examination is necessary because they may develop into pathological status such as dentigerous cysts. The importance of a precise clinical history and radiographic examination for patients with multiple supernumerary teeth should be emphasized.

  15. Supernumerary teeth in non-syndromic patients

    International Nuclear Information System (INIS)

    Mali, Santosh; Karjodkar, Freny Rashmiraj; Sontakke, Subodh; Sansare, Kaustubh

    2012-01-01

    Hyperdontia or supernumerary teeth without associated syndrome is a rare phenomenon, as supernumerary teeth are usually associated with cleft lip and palate or other syndromes such as Gardner's syndrome, cleidocranial dysplasia, and so on. Five patients with supernumerary teeth visited our department. They had no familial history or other pathology, certain treatment protocols was modified due to the presence of supernumerary teeth. Non-syndromic supernumerary teeth, if asymptomatic, need to have periodical radiographic observation. If they showed no variation as they impacted in the jaw, careful examination is necessary because they may develop into pathological status such as dentigerous cysts. The importance of a precise clinical history and radiographic examination for patients with multiple supernumerary teeth should be emphasized.

  16. Investigating hyperventilation syndrome in patients suffering from empty nose syndrome.

    Science.gov (United States)

    Mangin, David; Bequignon, Emilie; Zerah-Lancner, Francoise; Isabey, Daniel; Louis, Bruno; Adnot, Serge; Papon, Jean-François; Coste, André; Boyer, Laurent; Devars du Mayne, Marie

    2017-09-01

    Patients with empty nose syndrome (ENS) following turbinate surgery often complain about breathing difficulties. We set out to determine if dyspnea in patients with ENS was associated with hyperventilation syndrome (HVS). We hypothesized that lower airway symptoms in ENS could be explained by HVS. Observational prospective study. All consecutive patients referred to our center for ENS over 1 year were invited to participate. Patients completed the Nijmegen score and underwent a hyperventilation provocation test (HVPT) and arterial blood gas and cardiopulmonary tests. HVS was defined by a delayed return of the end-tidal partial pressure of carbon dioxide in the expired gas to baseline during HVPT. Patients with HVS were asked to complete the Sinonasal Outcome Test (SNOT)-16 questionnaire before and after a specific eight-session respiratory rehabilitation program. Twenty-two of the 29 patients referred for ENS during the study period were eligible for inclusion and underwent a complete workup. HVS was diagnosed in 17 of these patients (77.3%). In the five patients who completed the SNOT-16, the score was significantly lower after rehabilitation. This study suggests that HVS is frequent in patients with ENS, and that symptoms can be improved by respiratory rehabilitation. Pathophysiological links between ENS and HVS deserve to be further explored. 2b Laryngoscope, 127:1983-1988, 2017. © 2017 The American Laryngological, Rhinological and Otological Society, Inc.

  17. Glucocorticoid treatment in patients with acute respiratory distress syndrome. A systematic review and meta-analysis = El tratamiento con glucocorticoides en pacientes con síndrome de dificultad respiratoria aguda. Una revisión sistemática de la literatura y metaanálisis

    Directory of Open Access Journals (Sweden)

    Giraldo Ramírez, Nelson Darío

    2013-04-01

    Full Text Available Background: The Acute Respiratory Distress Syndrome (ARDS is a lung inflammation secondary to primary or extrinsic pulmonary pathology. It is a common disease in the intensive care unit and its mortality rate is high. Objectives: To determine the efficacy and safety of corticosteroids in patients with ARDS older than 18 years, in terms of mortality, mechanical ventilationfree days, and safety in regard to nosocomial infections, health-care related pneumonia, neuromiopathy, and gastrointestinal bleeding. Search methods: A systematic search of electronic and manual literature was done, without restriction of language, of controlled clinical trials involving adults with ARDS, randomized to placebo vs. steroids, and that measured the outcomes described. Results: Seven clinical trials were found showing a decrease in hospital mortality (OR 0.56 [0.38-0.81], 3.5 more days free from mechanical ventilation, a decrease in nosocomial infections and in hospitalacquired pneumonia. There were no differences in the presentation of steroid-associated neuromiopathy. There was a non-significant tendency to increase in bleeding from the digestive tract. Conclusion: Low-dose steroids seem to have a beneficial effect on mortality and ventilator-free days in adult patients with ARDS with no increase in adverse effects

  18. THE METABOLIC SYNDROME AMONG PATIENTS WITH ...

    African Journals Online (AJOL)

    Objectives: To determine the frequency of occurrence of the Metabolic Syndrome among patients presenting with cardiovascular disease at the Korle Bu Teaching Hospital, Ghana. Methods: This was a case-control study of 100 con-secutive cardiovascular disease patients and 100 age- and sex- matched controls who ...

  19. Hearing impairment in genotyped Wolfram syndrome patients.

    Science.gov (United States)

    Plantinga, Rutger F; Pennings, Ronald J E; Huygen, Patrick L M; Bruno, Rocco; Eller, Philipp; Barrett, Timothy G; Vialettes, Bernard; Paquis-Fluklinger, Veronique; Lombardo, Fortunato; Cremers, Cor W R J

    2008-07-01

    Wolfram syndrome is a progressive neurodegenerative syndrome characterized by the features "DIDMOAD" (diabetes insipidus, diabetes mellitus, optic atrophy, and deafness). We sought to study the audiometric data of genotyped Wolfram syndrome patients with sensorineural hearing impairment. Pure tone threshold data of 23 Wolfram syndrome patients were used for cross-sectional analysis in subgroups (age less than 16 years or between 19 and 25 years, gender, and origin). All subgroups, with 1 exception, showed a fairly similar type of hearing impairment with, on average, thresholds of about 25 dB (range, 0 to 65 dB) at 0.25 to 1 kHz, gently sloping downward to about 60 dB (range, 25 to 95 dB) at 8 kHz. The subgroup of Dutch women, which was excluded from the calculations of the average hearing thresholds, showed a higher degree of hearing impairment. Only the latter subgroup showed progression; however, contrary to the previous longitudinal analysis, progression was not significant in the present cross-sectional analysis, presumably because of the high degree of cross-subject variability. This unique collection of audiometric data from genotyped Wolfram syndrome patients shows no substantial progression in sensorineural hearing impairment with advancing age, no relation to the types of WFS1 mutations identified, and, with exclusion of the subgroup of Dutch female patients, no significant sex-related differences.

  20. Early application of airway pressure release ventilation may reduce the duration of mechanical ventilation in acute respiratory distress syndrome.

    Science.gov (United States)

    Zhou, Yongfang; Jin, Xiaodong; Lv, Yinxia; Wang, Peng; Yang, Yunqing; Liang, Guopeng; Wang, Bo; Kang, Yan

    2017-11-01

    Experimental animal models of acute respiratory distress syndrome (ARDS) have shown that the updated airway pressure release ventilation (APRV) methodologies may significantly improve oxygenation, maximize lung recruitment, and attenuate lung injury, without circulatory depression. This led us to hypothesize that early application of APRV in patients with ARDS would allow pulmonary function to recover faster and would reduce the duration of mechanical ventilation as compared with low tidal volume lung protective ventilation (LTV). A total of 138 patients with ARDS who received mechanical ventilation for mechanical ventilation from enrollment to day 28. The secondary endpoints included oxygenation, P plat , respiratory system compliance, and patient outcomes. Compared with the LTV group, patients in the APRV group had a higher median number of ventilator-free days {19 [interquartile range (IQR) 8-22] vs. 2 (IQR 0-15); P mechanical ventilation and ICU stay.

  1. Assessment of a volume-dependent dynamic respiratory system compliance in ALI/ARDS by pooling breathing cycles

    International Nuclear Information System (INIS)

    Zhao, Zhanqi; Möller, Knut; Guttmann, Josef

    2012-01-01

    New methods were developed to calculate the volume-dependent dynamic respiratory system compliance (C rs ) in mechanically ventilated patients. Due to noise in respiratory signals and different characteristics of the methods, their results can considerably differ. The aim of the study was to establish a practical procedure to validate the estimation of intratidal dynamic C rs . A total of 28 patients from intensive care units of eight German university hospitals with acute lung injury (ALI) and acute respiratory distress syndrome (ARDS) were studied retrospectively. Dynamic volume-dependent C rs was determined during ongoing mechanical ventilation with the SLICE method, dynostatic algorithm and adaptive slice method. Conventional two-point compliance C 2P was calculated for comparison. A number of consecutive breathing cycles were pooled to reduce noise in the respiratory signals. C rs -volume curves produced with different methods converged when the number of pooling cycles increased (n ≥ 7). The mean volume-dependent C rs of 20 breaths was highly correlated with mean C 2P (C 2P,mean = 0.945 × C rs,mean − 0.053, r 2 = 0.968, p < 0.0001). The Bland–Altman analysis indicated that C 2P,mean was lower than C rs,mean (−2.4 ± 6.4 ml cm −1 H 2 O, mean bias ± 2 SD), but not significant according to the paired t-test (p > 0.05). Methods for analyzing dynamic respiratory mechanics are sensitive to noise and will converge to a unique solution when the number of pooled cycles increases. Under steady-state conditions, assessment of the volume-dependent C rs in ALI/ARDS patients can be validated by pooling respiratory data of consecutive breaths regardless of which method is applied. Confidence in dynamic C rs determination may be increased with the proposed pooling. (note)

  2. [Fournier syndrome: the perception of the patient].

    Science.gov (United States)

    Cavalini, Fernanda; Moriya, Tokico Murakawa; Pelá, Nilza Teresa Rotter

    2002-06-01

    The purpose of the present investigation was to identify the perception of the Fournier syndrome's patients about their disease and caregivers. Data was collected by means of interviews with a structured routine and the analysis was based on the André's analysis of prose. According to the patients, the syndrome causes intense pain, edema, fever and wounds, demanding surgical treatment, dressings and bringing physical, economical and familiar problems. The caregivers' attitudes and behaviors were described as having more negative aspects than good ones. The necessity of a better training of the caregivers in the cognitive, psychomotor and affective sense is evident.

  3. Lung microvascular transport properties measured by multiple indicator dilution methods in patients with adult respiratory distress syndrome. A comparison between patients reversing respiratory failure and those failing to reverse

    International Nuclear Information System (INIS)

    Harris, T.R.; Bernard, G.R.; Brigham, K.L.; Higgins, S.B.; Rinaldo, J.E.; Borovetz, H.S.; Sibbald, W.J.; Kariman, K.; Sprung, C.L.

    1990-01-01

    We conducted indicator dilution studies on the lungs of patients in the early phases of adult respiratory distress syndrome (ARDS) to test the hypothesis that capillary permeability was increased in patients with respiratory failure. Indicator dilution studies were performed using 51Cr-erythrocytes, 125I-albumin, 14C-urea, and 3H-water as tracers. The injectate was infused as a bolus into a central venous line. Peripheral arterial blood was collected and counted for radioactivity. Mathematical analysis of the indicator curves yielded cardiac output, measures of the product of capillary permeability and surface area for urea (PS and D1/2S), the intravascular lung volume (Vv), and the extravascular lung water volume (Ve). Permeability was separated from surface area by normalizing PS and D1/2S to Vv. Patients could be divided into 16 in whom blood gas determinations and radiologic criteria for ARDS were reversed and 23 in whom they were not. We examined indicator dilution and other measures of lung function in the two groups to determine whether significant differences in microvascular function existed. PS and PS/Vv were significantly higher in the nonreversal patients. Ve was above normal, but not different between groups. Linear regression analysis showed significant correlations for all of the following in the nonreversal group: Ve and all measures of permeability, pulmonary vascular resistance (PVR), and the inverse of permeability-surface area measures and AaDO2 and PVR. Only measures of Ve and PS correlated in the reversal group. These results support the hypothesis that capillary permeability is increased in patients with early ARDS and continuing respiratory failure

  4. Development of ARDS after Excessive Kath Consumption: A Case Report

    Directory of Open Access Journals (Sweden)

    Marlene Wewalka

    2011-01-01

    Full Text Available Khat is a drug widely used in the Horn of Africa and the Arabian Peninsula. Khat leaves contain, among other substances, the psychoactive alkaloid cathinone, which induce central nervous system stimulation leading to euphoria, hyperactivity, restlessness, and insomnia. However, it also could cause psychological adverse effects such as lethargy, sleepiness, psychoses, and depression necessitating pharmacologic treatment. Here we report the case of a 35-year-old man from Somalia who became unconscious and developed aspiration pneumonia and subsequent ARDS after excessive consumption of khat leaves. His unconsciousness was possibly caused by the sleepiness developed after khat consumption and a benzodiazepine intake by the patient himself. Thus, khat-induced adverse effects should not primarily be treated pharmacologically, but patients should be urged to quit khat consumption in order to eliminate or, at least, reduce the severity of present psychological adverse effects.

  5. BURNOUT SYNDROME IN PATIENTS WITH WORKPLACE HYPERTENSION

    Directory of Open Access Journals (Sweden)

    Yu. P. Zinchenko

    2016-01-01

    Full Text Available Aim. To study the presence, severity and qualitative characteristics of «burnout syndrome» in patients with «workplace hypertension» (WPH, in comparison with patients with essential hypertension and healthy people.Material and methods. Untreated patients with hypertension stage II, degree 1-2 (n=170; age 32-52 years; mean age 46.7Ѓ}4.1 years were examined. Group 1 included 85 patients with WPH (mean age 44.7Ѓ}4.3 years and Group 2 included 85 patients without WPH (mean age 47.4Ѓ}4.5 years. The duration of hypertension on average was 7.2Ѓ}2.6 years and was comparable in both groups. The control group included 82 healthy subjects (mean age 44.9Ѓ}3.1 years. The Russian version of the Maslach Burnout Inventory (MBI was used to diagnose «burnout syndrome».Results. Signs of «burnout syndrome» were found in the hypertensive patients of both groups. The high and medium levels of the «burnout syndrome» severity according to all three analyzed factors (emotional exhaustion, dehumanization/depersonification, and personal achievements were found in 59% of WPH patients, in 36% of hypertensive patients without WPH, and in 9% of healthy individuals. Most of WPH patients had high emotional exhaustion compared with other groups (27.5Ѓ}3.67 points vs 24.6Ѓ}4.3 and 20.1Ѓ}5.7 points in group 2, and group of healthy, respectively; p<0.05. Hypertensive men rated themselves as less successful professionally in comparison with women. Hypertensive women were more prone to emotional exhaustion and dehumanization/depersonification in comparison with men. Conclusion. Signs of «burnout syndrome» were found significantly more often in hypertensive patients in both groups than in healthy people. Medium and high intensity of all forms of «burnout syndrome» occurred in patients with WPH in comparison with healthy people and hypertensive patients without WPH.

  6. BURNOUT SYNDROME IN PATIENTS WITH WORKPLACE HYPERTENSION

    OpenAIRE

    Yu. P. Zinchenko; E. I. Pervichko; O. D. Ostroumova

    2016-01-01

    Aim. To study the presence, severity and qualitative characteristics of «burnout syndrome» in patients with «workplace hypertension» (WPH), in comparison with patients with essential hypertension and healthy people.Material and methods. Untreated patients with hypertension stage II, degree 1-2 (n=170; age 32-52 years; mean age 46.7Ѓ}4.1 years) were examined. Group 1 included 85 patients with WPH (mean age 44.7Ѓ}4.3 years) and Group 2 included 85 patients without WPH (mean age 47.4Ѓ}4.5 years)...

  7. Clinicopathological comparison of colorectal and endometrial carcinomas in patients with Lynch-like syndrome versus patients with Lynch syndrome.

    Science.gov (United States)

    Mas-Moya, Jenny; Dudley, Beth; Brand, Randall E; Thull, Darcy; Bahary, Nathan; Nikiforova, Marina N; Pai, Reetesh K

    2015-11-01

    Screening for DNA mismatch repair (MMR) deficiency in colorectal and endometrial carcinomas identifies patients at risk for Lynch syndrome. Some patients with MMR-deficient tumors have no evidence of a germline mutation and have been described as having Lynch-like syndrome. We compared the clinicopathological features of colorectal and endometrial carcinomas in patients with Lynch-like syndrome and Lynch syndrome. Universal screening identified 356 (10.6%) of 3352 patients with colorectal carcinoma and 72 (33%) of 215 patients with endometrial carcinoma with deficient DNA MMR. Sixty-six patients underwent germline mutation analysis with 45 patients (68%) having evidence of a germline MMR gene mutation confirming Lynch syndrome and 21 patients (32%) having Lynch-like syndrome with no evidence of a germline mutation. Most patients with Lynch-like syndrome had carcinoma involving the right colon compared to patients with Lynch syndrome (93% versus 45%; P Lynch syndrome confirmed by germline mutation analysis. Synchronous or metachronous Lynch syndrome-associated carcinoma was more frequently identified in patients with Lynch syndrome compared to Lynch-like syndrome (38% versus 7%; P = .04). There were no significant differences in clinicopathological variables between patients with Lynch-like syndrome and Lynch syndrome with endometrial carcinoma. In summary, 32% of patients with MMR deficiency concerning Lynch syndrome will have Lynch-like syndrome. Our results demonstrate that patients with Lynch-like syndrome are more likely to have right-sided colorectal carcinoma, less likely to have synchronous or metachronous Lynch syndrome-associated carcinoma, and less likely to demonstrate isolated loss of MSH6 expression within their tumor. Copyright © 2015 Elsevier Inc. All rights reserved.

  8. Parent-of-origin effects in Turner Syndrome patients

    OpenAIRE

    Wang, Jada; Styers, Marshall; Sayres, Melissa Wilson

    2015-01-01

    Turner Syndrome patients have a single X chromosome, without a partner, X or Y. It has been suggested that the inheritance of the maternal X or paternal X may affect the severity of Turner Syndrome, as well as the incidence of mental disorders in Turner Syndrome individuals. Parental imprinting on the X chromosome may lead to different phenotypic variations in Turner Syndrome patients. In this project, we conduct an analysis of the current state of research on Turner Syndrome, and review the ...

  9. Mortality in Patients with Endogenous Cushing's Syndrome.

    Science.gov (United States)

    Javanmard, Pedram; Duan, Daisy; Geer, Eliza B

    2018-06-01

    Cushing's syndrome is associated with increased morbidity and mortality. Cardiovascular events, sepsis, and thromboembolism are the leading causes of mortality. Patient's with Cushing's due to a pituitary adenoma and those with Cushing's due to benign adrenal adenoma have relatively good survival outcomes often mirroring that of the general population. Persistent or recurrent disease is associated with high mortality risk. Ectopic Cushing's syndrome and Cushing's due to adrenocortical carcinoma confer the highest mortality risk among Cushing's etiologies. Prompt diagnosis and treatment, and specific monitoring for and treatment of associated comorbidities are essential to decrease the burden of mortality from Cushing's. Copyright © 2018 Elsevier Inc. All rights reserved.

  10. Physiotherapy improves patient reported shoulder function and health status in patients with subacromial impingement syndrome

    DEFF Research Database (Denmark)

    Storgaard, Filip Holst; Pedersen, Christina Gravgaard; Jensen, Majbritt Lykke

    Physiotherapy improves patient reported shoulder function and health status in patients with subacromial impingement syndrome.......Physiotherapy improves patient reported shoulder function and health status in patients with subacromial impingement syndrome....

  11. Combined Immunodeficiency in Patients With Trichohepatoenteric Syndrome

    Directory of Open Access Journals (Sweden)

    Frédéric Vély

    2018-05-01

    Full Text Available The syndromic diarrhea/trichohepatoenteric syndrome (SD/THE is a rare and multi-system genetic disorder caused by mutation in SKIV2L or in TTC37, two genes encoding subunits of the putative human SKI complex involved in RNA degradation. The main features are intractable diarrhea of infancy, hair abnormalities, facial dysmorphism, and intrauterine growth restriction. Immunologically this syndrome is associated with a hypogammaglobulinemia leading to an immunoglobulin supplementation. Our immune evaluation of a large French cohort of SD/THE patient revealed several immunological defects. First, switched memory B lymphocytes count is very low. Second, IFN-γ production by T and NK cells is impaired and associated with a reduced degranulation of NK cells. Third, T cell proliferation was abnormal in 3/6 TTC37-mutated patients. These three patients present with severe EBV infection and a transient hemophagocytosis which may be related to these immunological defects. Moreover, an immunological screening of patients with clinical features of SD/THE could facilitate both diagnosis and therapeutic management of these patients.

  12. Respiratory reovirus 1/L induction of diffuse alveolar damage: pulmonary fibrosis is not modulated by corticosteroids in acute respiratory distress syndrome in mice.

    Science.gov (United States)

    London, Lucille; Majeski, Elizabeth I; Altman-Hamamdzic, Sanja; Enockson, Candace; Paintlia, Manjeet K; Harley, Russell A; London, Steven D

    2002-06-01

    Acute respiratory distress syndrome (ARDS) is a clinical syndrome characterized by diffuse alveolar damage (DAD) secondary to an intense host inflammatory response of the lung to a pulmonary or extrapulmonary infectious or noninfectious insult. We have previously described a unique animal model in which CBA/J mice infected with reovirus 1/L develop ARDS. This model recapitulates the histopathological changes observed in human ARDS, which consist of the overlapping phases of exudation, including the formation of hyaline membranes, regeneration, and healing via repair with fibrosis. In this report, we show that the development of DAD in the acute phase of the disease and intraalveolar fibrosis in the late phase of the disease was not modulated by treatment with methylprednisolone (MPS). In the presence or absence of MPS, the majority of cells infiltrating the lungs after reovirus 1/L infection were polymorphonuclear leukocytes and macrophages. A number of key proinflammatory and anti-inflammatory cytokines/chemokines that are observed in the BAL fluid of ARDS patients were also found in the lungs of mice after reovirus 1/L infection and were not modulated by MPS. These include interferon-gamma, interleukin-10, and monocyte chemoattractant protein. The histopathology, cytokine/chemokine expression, and response to corticosteroids in reovirus 1/L-induced ARDS are similar to what is observed in human patients, making this a clinically relevant model.

  13. A Chinese patient with pusher syndrome and unilateral spatial neglect syndrome.

    Science.gov (United States)

    Chen, Xiao-Wei; Lin, Cheng-He; Zheng, Hua; Lin, Zhen-Lan

    2014-07-01

    To observe clinical manifestations, behavioral characteristics, and effects of rehabilitation on a patient with pusher syndrome and unilateral spatial neglect caused by right thalamic hemorrhage. Assessment of pusher syndrome was made by the Scale for Contraversive pushing (SCP), and unilateral spatial neglect syndrome was diagnosed using line cancellation, letter and star cancellation, line bisection tests and copy and continuation of graphic sequence test. Behavioral therapy, occupational therapy, reading training and traditional Chinese medicine methods were adopted for treatment of pusher syndrome and unilateral spatial neglect. The patient showed typical pusher syndrome and unilateral spatial neglect symptoms. The pusher syndrome and unilateral spatial neglect symptoms were significantly improved following rehabilitation treatments. Pusher syndrome and unilateral spatial neglect syndrome occurred simultaneously after right thalamic hemorrhage. Early rehabilitation therapy can reduce the symptoms of pusher syndrome and unilateral spatial neglect syndrome and improve motor function.

  14. Pediatric acute respiratory distress syndrome: Host factors in Down syndrome and the general population

    NARCIS (Netherlands)

    Bruijn, M.

    2013-01-01

    We find that Down syndrome is an important risk factor for developing acute respiratory distress syndrome (ARDS) in children, but the reason why remains to be elucidated. In addition, we find several differences between adult and pediatric ARDS. The association between C-reactive protein (CRP)

  15. Nutrition: A Primary Therapy in Pediatric Acute Respiratory Distress Syndrome

    Directory of Open Access Journals (Sweden)

    Bryan Wilson

    2016-10-01

    Full Text Available Appropriate nutrition is an essential component of intensive care management of children with Acute Respiratory Distress Syndrome (ARDS and is linked to patient outcomes. One out of every two children in the PICU will develop malnutrition or have worsening of baseline malnutrition, and present with specific micronutrient deficiencies. Early and adequate enteral nutrition (EN is associated with improved 60-day survival after pediatric critical illness and yet, despite early EN guidelines, critically ill children receive on average only 55% of goal calories by PICU day 10. Inadequate delivery of EN is due to perceived feeding intolerance, reluctance to enterally feed children with hemodynamic instability, and fluid restriction. Underlying each of these factors is large practice variation between providers and across institutions for initiation, advancement and maintenance of EN. Strategies to improve early initiation, advancement, and to maintain delivery of EN are needed to improve morbidity and mortality from pediatric ARDS. Both over and underfeeding prolongs duration of mechanical ventilation in children and worsens other organ function such that precise calorie goals are needed. The gut is thought to act as a ‘motor’ of organ dysfunction and emerging data regarding the role of intestinal barrier functions and the intestinal microbiome on organ dysfunction and outcomes of critical illness present exciting opportunities to improve patient outcomes. Nutrition should be considered a primary rather than supportive therapy for pediatric ARDS. Precise nutritional therapies, which are titrated and targeted to preservation of intestinal barrier function, prevention of intestinal dysbiosis, preservation of lean body mass, and blunting of the systemic inflammatory response, offer great potential for improving outcomes of pediatric ARDS. In this review we examine the current evidence regarding dose, route, and timing of nutrition, current

  16. The EIT-based global inhomogeneity index is highly correlated with regional lung opening in patients with acute respiratory distress syndrome.

    Science.gov (United States)

    Zhao, Zhanqi; Pulletz, Sven; Frerichs, Inéz; Müller-Lisse, Ullrich; Möller, Knut

    2014-02-06

    The electrical impedance tomography (EIT)-based global inhomogeneity (GI) index was introduced to quantify tidal volume distribution within the lung. Up to now, the GI index was evaluated for plausibility but the analysis of how it is influenced by various physiological factors is still missing. The aim of our study was to evaluate the influence of proportion of open lung regions measured by EIT on the GI index. A constant low-flow inflation maneuver was performed in 18 acute respiratory distress syndrome (ARDS) patients (58 ± 14 years, mean age ± SD) and 8 lung-healthy patients (41 ± 12 years) under controlled mechanical ventilation. EIT raw data were acquired at 25 scans/s and reconstructed offline. Recruited lung regions were identified as those image pixels of the lung regions within the EIT scans where local impedance amplitudes exceeded 10% of the maximum amplitude during the maneuver. A series of GI indices was calculated during mechanical lung inflation, based on the differential images obtained between different time points. Respiratory system elastance (Ers) values were calculated at 10 lung volume levels during low-flow maneuver. The GI index decreased during low-flow inflation, while the percentage of open lung regions increased. The values correlated highly in both ARDS (r2 = 0.88 ± 0.08, p EIT. The GI index may prove to be a useful EIT-based index to guide ventilation therapy.

  17. Restless legs syndrome in patients on hemodialysis

    Directory of Open Access Journals (Sweden)

    Saleh Mohammad Yaser Salman

    2011-01-01

    Full Text Available Restless legs syndrome (RLS is common among dialysis patients, with a reported prevalence of 6-60%. The prevalence of RLS in Syrian patients on hemodialysis (HD is not known. The purpose of this study is to determine the prevalence of RLS in patients on regular HD, and to find the possible correlation between the presence of RLS and demographic, clinical, and biochemical factors. One hundred and twenty-three patients (male/female = 70/53, mean age = 41.95 ± 15.11 years on HD therapy at the Aleppo University Hospital were enrolled into the study. RLS was diagnosed based on criteria established by the International Restless Legs Syn-drome Study Group (IRLSSG. Data procured were compared between patients with and without RLS. Applying the IRLSSG criteria for the diagnosis, RLS was seen in 20.3% of the study pa-tients. No significant difference in age, gender, and intake of nicotine and caffeine was found between patients with and without the RLS. Similarly, there was no difference between the two groups in the duration of end-stage renal disease (ESRD, the period of dialysis dependence, dialysis adequacy, urea and creatinine levels, and the presence of anemia. The co-morbidities and the use of drugs also did not differ in the two groups. Our study suggests that the high prevalence of RLS among patients on HD requires careful attention and correct diagnosis can lead to better therapy and better quality of life. The pathogenesis of RLS is not clear and further studies are required to identify any possible cause as well as to discover the impact of this syndrome on sleep, quality of life, and possibly other complications such as cardiovasculare disease.

  18. Cloning, characterization, and expression analysis of the novel acetyltransferase retrogene Ard1b in the mouse.

    Science.gov (United States)

    Pang, Alan Lap-Yin; Peacock, Stephanie; Johnson, Warren; Bear, Deborah H; Rennert, Owen M; Chan, Wai-Yee

    2009-08-01

    N-alpha-terminal acetylation is a modification process that occurs cotranslationally on most eukaryotic proteins. The major enzyme responsible for this process, N-alpha-terminal acetyltransferase, is composed of the catalytic subunit ARD1A and the auxiliary subunit NAT1. We cloned, characterized, and studied the expression pattern of Ard1b (also known as Ard2), a novel homolog of the mouse Ard1a. Comparison of the genomic structures suggests that the autosomal Ard1b is a retroposed copy of the X-linked Ard1a. Expression analyses demonstrated a testis predominance of Ard1b. A reciprocal expression pattern between Ard1a and Ard1b is also observed during spermatogenesis, suggesting that Ard1b is expressed to compensate for the loss of Ard1a starting from meiosis. Both ARD1A and ARD1B can interact with NAT1 to constitute a functional N-alpha-terminal acetyltransferase in vitro. The expression of ARD1B protein can be detected in mouse testes but is delayed until the first appearance of round spermatids. In a cell culture model, the inclusion of the long 3' untranslated region of Ard1b leads to reduction of luciferase reporter activity, which implicates its role in translational repression of Ard1b during spermatogenesis. Our results suggest that ARD1B may have an important role in the later course of the spermatogenic process.

  19. Metabolic syndrome in Tunisian bipolar I patients | Ezzaher | African ...

    African Journals Online (AJOL)

    Gender, age, illness episode and treatment were not significantly associated with metabolic syndrome, while patients under lithium had higher prevalence of metabolic syndrome than those under valproic acid, carbamazepine or antipsychotics. Patients with metabolic syndrome had significant higher levels of HOMA-IR and ...

  20. Diagnostic value of static and dynamic scintigraphy in diagnosis of acute respiratory distress syndrome in the course of sepsis. Part 1. Lung perfusion scintigraphy

    International Nuclear Information System (INIS)

    Jurgilewicz, D.; Rogowski, F.; Malinowska, L.

    1997-01-01

    One of the most important complication of sepsis is acute respiratory distress syndrome (ARDS). Diagnosis of the illness is mainly based on chest radiography and gasometric parameters of the blood. The aim of the study was to estimate the diagnostic usefulness of lung perfusion scintigraphy in early detection of blood flow and gas-exchange abnormalities in patients with ARDS in the course of sepsis. Scintigraphic studies of 12 patients in critical condition were performed, using Hungarian planar gamma camera type MB9200 and human albumin microspheres labelled with 99m Tc. Perfusion scans of patients with ARDS demonstrated blurring outlines and abundant diffuse foci of lack of radioactivity in both lungs and quantitative analysis indicated relative increase of Tc99m-MSA accumulation in upper zones of both lungs. Scans of suffering from sepsis were similar to control one. The course of the studies showed that scintigraphic methods could be safely use in patients with sepsis and ARDS and may be helpful in the early diagnosis of ARDS in the septic patients. (author)

  1. Polycystic Ovary Syndrome in Patients with Epilepsy

    OpenAIRE

    Goltz, Christoph Freiherr von der

    2010-01-01

    Epilepsy has been associated with an increased frequency of reproductive endocrine disorders including polycystic ovary syndrome (PCOS). Some study groups claim that epilepsy itself plays a pathogenic role, whereas others propose that PCOS may be attributable to the use of antiepileptic drugs (AEDs), particularly sodium valproate (VPA). Estimates regarding the prevalence of PCOS in this patient group vary, among other reasons, because of different definitions of PCOS. The aim of the present c...

  2. Prolonged Extracorporeal Membrane Oxygenation Support for Acute Respiratory Distress Syndrome

    Directory of Open Access Journals (Sweden)

    Wen-Je Ko

    2006-01-01

    Full Text Available When all conventional treatments for respiratory failure in patients with acute respiratory distress syndrome (ARDS have failed, extracorporeal membrane oxygenation (ECMO can provide a chance of survival in these desperately ill patients. A 49-year-old male patient developed septic shock and progressive ARDS after liver abscess drainage. Venovenous ECMO was given due to refractory respiratory failure on postoperative day 6. Initially, two heparin-binding hollow-fiber microporous membrane oxygenators in parallel were used in the ECMO circuit. Twenty-two oxygenators were changed in the first 22 days of ECMO support because of plasma leak in the oxygenators. Each oxygenator had an average life of 48 hours. Thereafter, a single silicone membrane oxygenator was used in the ECMO circuit, which did not require change during the remaining 596 hours of ECMO. The patient's tidal volume was only 90 mL in the nadir and less than 300 mL for 26 days during the ECMO course. The patient required ECMO support for 48 days and survived despite complications, including septic shock, ARDS, acute renal failure, drug-induced leukopenia, and multiple internal bleeding. This patient received an unusually long duration of ECMO support. However, he survived, recovered well, and was in New York Heart Association functional class I-II, with a forced expiratory volume in 1 second of 81% of the predicted level 18 months later. In conclusion, ECMO can provide a chance of survival for patients with refractory ARDS. The reversibility of lung function is possible in ARDS patients regardless of the severity of lung dysfunction at the time of treatment.

  3. Subclinical nephritic syndrome in children cohabiting with pediatric patients, Presenting acute nephritic syndrome

    OpenAIRE

    Guerrero-Tinoco Gustavo Adolfo; Julio-Barrios Emil

    2012-01-01

    Introduction: subclinical nephritic syndrome is the presence of hematuria, hypocomplementemiaand/or proteinuria without the presence of signs and/or symptoms.Objective: to determine the incidence of subclinical nephritic syndrome in childrenliving with pediatric patients diagnosed with acute nephritic syndrome.Methods: family visit to identify children living together in the two previous months, with pediatric patients hospitalized with acute nephritic syndrome, at Hospital InfantilNapoleon F...

  4. A mouse model for MERS coronavirus-induced acute respiratory distress syndrome.

    Science.gov (United States)

    Cockrell, Adam S; Yount, Boyd L; Scobey, Trevor; Jensen, Kara; Douglas, Madeline; Beall, Anne; Tang, Xian-Chun; Marasco, Wayne A; Heise, Mark T; Baric, Ralph S

    2016-11-28

    Middle East respiratory syndrome coronavirus (MERS-CoV) is a novel virus that emerged in 2012, causing acute respiratory distress syndrome (ARDS), severe pneumonia-like symptoms and multi-organ failure, with a case fatality rate of ∼36%. Limited clinical studies indicate that humans infected with MERS-CoV exhibit pathology consistent with the late stages of ARDS, which is reminiscent of the disease observed in patients infected with severe acute respiratory syndrome coronavirus. Models of MERS-CoV-induced severe respiratory disease have been difficult to achieve, and small-animal models traditionally used to investigate viral pathogenesis (mouse, hamster, guinea-pig and ferret) are naturally resistant to MERS-CoV. Therefore, we used CRISPR-Cas9 gene editing to modify the mouse genome to encode two amino acids (positions 288 and 330) that match the human sequence in the dipeptidyl peptidase 4 receptor, making mice susceptible to MERS-CoV infection and replication. Serial MERS-CoV passage in these engineered mice was then used to generate a mouse-adapted virus that replicated efficiently within the lungs and evoked symptoms indicative of severe ARDS, including decreased survival, extreme weight loss, decreased pulmonary function, pulmonary haemorrhage and pathological signs indicative of end-stage lung disease. Importantly, therapeutic countermeasures comprising MERS-CoV neutralizing antibody treatment or a MERS-CoV spike protein vaccine protected the engineered mice against MERS-CoV-induced ARDS.

  5. Pneumonia, Acute Respiratory Distress Syndrome, and Early Immune-Modulator Therapy

    Directory of Open Access Journals (Sweden)

    Kyung-Yil Lee

    2017-02-01

    Full Text Available Acute respiratory distress syndrome (ARDS is caused by infectious insults, such as pneumonia from various pathogens or related to other noninfectious events. Clinical and histopathologic characteristics are similar across severely affected patients, suggesting that a common mode of immune reaction may be involved in the immunopathogenesis of ARDS. There may be etiologic substances that have an affinity for respiratory cells and induce lung cell injury in cases of ARDS. These substances originate not only from pathogens, but also from injured host cells. At the molecular level, these substances have various sizes and biochemical characteristics, classifying them as protein substances and non-protein substances. Immune cells and immune proteins may recognize and act on these substances, including pathogenic proteins and peptides, depending upon the size and biochemical properties of the substances (this theory is known as the protein-homeostasis-system hypothesis. The severity or chronicity of ARDS depends on the amount of etiologic substances with corresponding immune reactions, the duration of the appearance of specific immune cells, or the repertoire of specific immune cells that control the substances. Therefore, treatment with early systemic immune modulators (corticosteroids and/or intravenous immunoglobulin as soon as possible may reduce aberrant immune responses in the potential stage of ARDS.

  6. Lung tissue remodeling in the acute respiratory distress syndrome

    Directory of Open Access Journals (Sweden)

    Souza Alba Barros de

    2003-01-01

    Full Text Available Acute respiratory distress syndrome (ARDS is characterized by diffuse alveolar damage, and evolves progressively with three phases: exsudative, fibroproliferative, and fibrotic. In the exudative phase, there are interstitial and alveolar edemas with hyaline membrane. The fibropro­liferative phase is characterized by exudate organization and fibroelastogenesis. There is proliferation of type II pneumocytes to cover the damaged epithelial surface, followed by differentiation into type I pneumocytes. The fibroproliferative phase starts early, and its severity is related to the patient?s prognosis. The alterations observed in the phenotype of the pulmonary parenchyma cells steer the tissue remodeling towards either progressive fibrosis or the restoration of normal alveolar architecture. The fibrotic phase is characterized by abnormal and excessive deposition of extracellular matrix proteins, mainly collagen. The dynamic control of collagen deposition and degradation is regulated by metalloproteinases and their tissular regulators. The deposition of proteoglycans in the extracellular matrix of ARDS patients needs better study. The regulation of extracellular matrix remodeling, in normal conditions or in several pulmonary diseases, such as ARDS, results from a complex mechanism that integrate the transcription of elements that destroy the matrix protein and produce activation/inhibition of several cellular types of lung tissue. This review article will analyze the ECM organization in ARDS, the different pulmonary parenchyma remodeling mechanisms, and the role of cytokines in the regulation of the different matrix components during the remodeling process.

  7. Malignant vasovagal syndrome in two patients with Wolff-Parkinson-White syndrome

    Science.gov (United States)

    Gandhi, N M; Bennett, D H

    2004-01-01

    The presence of Wolff-Parkinson-White (WPW) syndrome in patients presenting with syncope suggests that tachyarrhythmia may be the cause. However, the symptoms require careful evaluation. Two young patients presented with syncope and were found to have WPW syndrome on their ECG. In both patients symptoms were suggestive of vasovagal syncope. During tilt testing, both the patients developed their typical symptoms with a fall in blood pressure and heart rate confirming the diagnosis of malignant vasovagal syndrome. PMID:15020537

  8. Incidence of refeeding syndrome in internal medicine patients.

    Science.gov (United States)

    Kraaijenbrink, B V C; Lambers, W M; Mathus-Vliegen, E M H; Siegert, C E H

    2016-03-01

    Refeeding syndrome is a potentially fatal shift of fluids and electrolytes that may occur after reintroducing nutrition in a malnourished patient. Its incidence in internal medicine patients is not known. We aimed at determining the incidence in a heterogeneous group of patients acutely admitted to a department of internal medicine. All patients acutely admitted to the department of internal medicine of a teaching community hospital in Amsterdam, the Netherlands, between 22 February 2011 and 29 April 2011, were included. We applied the National Institute for Health and Care Excellence (NICE) criteria for determining people at risk of refeeding syndrome and took hypophosphataemia as the main indicator for the presence of this syndrome. Of 178 patients included in the study, 97 (54%) were considered to be at risk of developing refeeding syndrome and 14 patients actually developed the syndrome (14% of patients at risk and 8% of study population). Patients with a malignancy or previous malignancy were at increased risk of developing refeeding syndrome (p refeeding syndrome. The Short Nutritional Assessment Questionnaire score had a positive and negative predictive value of 13% and 95% respectively. The incidence of refeeding syndrome was relatively high in patients acutely admitted to the department of internal medicine. Oncology patients are at increased risk of developing refeeding syndrome. When taking the occurrence of hypophosphataemia as a hallmark, no other single clinical or composite parameter could be identified that accurately predicts the development of refeeding syndrome.

  9. Craniofacial abnormalities among patients with Edwards Syndrome

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    Rafael Fabiano M. Rosa

    2013-09-01

    Full Text Available OBJECTIVE To determine the frequency and types of craniofacial abnormalities observed in patients with trisomy 18 or Edwards syndrome (ES. METHODS This descriptive and retrospective study of a case series included all patients diagnosed with ES in a Clinical Genetics Service of a reference hospital in Southern Brazil from 1975 to 2008. The results of the karyotypic analysis, along with clinical data, were collected from medical records. RESULTS: The sample consisted of 50 patients, of which 66% were female. The median age at first evaluation was 14 days. Regarding the karyotypes, full trisomy of chromosome 18 was the main alteration (90%. Mosaicism was observed in 10%. The main craniofacial abnormalities were: microretrognathia (76%, abnormalities of the ear helix/dysplastic ears (70%, prominent occiput (52%, posteriorly rotated (46% and low set ears (44%, and short palpebral fissures/blepharophimosis (46%. Other uncommon - but relevant - abnormalities included: microtia (18%, orofacial clefts (12%, preauricular tags (10%, facial palsy (4%, encephalocele (4%, absence of external auditory canal (2% and asymmetric face (2%. One patient had an initial suspicion of oculo-auriculo-vertebral spectrum (OAVS or Goldenhar syndrome. CONCLUSIONS: Despite the literature description of a characteristic clinical presentation for ES, craniofacial alterations may be variable among these patients. The OAVS findings in this sample are noteworthy. The association of ES with OAVS has been reported once in the literature.

  10. West syndrome in a patient with Schinzel-Giedion syndrome.

    Science.gov (United States)

    Miyake, Fuyu; Kuroda, Yukiko; Naruto, Takuya; Ohashi, Ikuko; Takano, Kyoko; Kurosawa, Kenji

    2015-06-01

    Schinzel-Giedion syndrome is a rare recognizable malformation syndrome defined by characteristic facial features, profound developmental delay, severe growth failure, and multiple congenital anomalies. The causative gene of Schinzel-Giedion syndrome, SETBP1, has been identified, but limited cases have been confirmed by molecular analysis. We present a 9-month-old girl affected by West syndrome with Schinzel-Giedion syndrome. Congenital severe hydronephrosis, typical facial features, and multiple anomalies suggested a clinical diagnosis of Schinzel-Giedion syndrome. Hypsarrhythmia occurred at 7 months of age and was temporarily controlled by adrenocorticotropic hormone (ACTH) therapy during 5 weeks. SETBP1 mutational analysis showed the presence of a recurrent mutation, p.Ile871Thr. The implications in management of Schinzel-Giedion syndrome are discussed. © The Author(s) 2014.

  11. Orthodontic treatment considerations in Down syndrome patients

    Directory of Open Access Journals (Sweden)

    Sianiwati Goenharto

    2012-03-01

    Full Text Available Background: Down syndrome is an easily recognized congenital disease anomaly, a common autosomal chromosomal anomaly with high prevalence of malocclusion. Orthodontic treatment demand should be high but it seems difficult to be done because of specific condition of disability. Purpose: The purpose of this literature review was to discribe the orthodontic problems found in Down syndrome patients and several consideration that shoud be done to treat them. Reviews: Many studies report the high prevalence of malocclusion among people with Down syndrome. There is a greater frequency of clas III relationship, crossbite, crowding and also open bite. Several problems might appear in the treatment because of dental, medical, mental, and behavioural factor. Conclusion: It is concluded that orthodonic treatment can be performed in Down syndrome patient, although several difficulties may appear. Good consideration in mental, behavior, medical and also dental condition will influence whether the treatment will success or not. Special care and facilities will support the orthodontic treatment.Latar belakang: Sindroma Down adalah suatu kelainan congenital yang mudah dikenali, merupakan kelaian kromosom autosomal yang cukup banyak terjadi, dengan prevalensi maloklusi cukup tinggi. Seharusnya permintaan akan perawatan ortodonti juga tinggi meskipun tampaknya sulit dilakukan karena adanya kondisi ketidakmampuan/cacat yang spesifik. Tujuan: Tujuan studi pustaka ini adalah untuk menggambarkan problem perawatan ortodonti pada penderita sindroma Down dan pertimbangan apa yang sebaiknya diambil untuk mengatasi masalah tersebut. Tinjauan pustaka: Banyak penelitian melaporkan tentang prevalensi maloklusi yang tinggi pada penderita sindroma Down. Maloklusi yang sering dijumpai adalah relasi klas III, gigitan silang, berdesakan dan juga gigitan terbuka. Problem dapat terjadi saat perawatan ortodonti karena adanya faktor dental, medis, mental dan tingkah laku penderita

  12. Noonan syndrome and Turner syndrome patients respond similarly to 4 years' growth-hormone therapy

    DEFF Research Database (Denmark)

    Lee, Peter A; Ross, Judith L; Pedersen, Birgitte Tønnes

    2015-01-01

    BACKGROUND: Turner syndrome (TS) and Noonan syndrome (NS) are distinct syndromes associated with short stature and other similar phenotypic features. We compared the responses to growth hormone (GH) therapy of TS and NS patients enrolled in the NordiNet® International Outcome Study (IOS...

  13. An attempt to validate the modification of the American-European consensus definition of acute lung injury/acute respiratory distress syndrome by the Berlin definition in a university hospital.

    Science.gov (United States)

    Hernu, R; Wallet, F; Thiollière, F; Martin, O; Richard, J C; Schmitt, Z; Wallon, G; Delannoy, B; Rimmelé, T; Démaret, C; Magnin, C; Vallin, H; Lepape, A; Baboi, L; Argaud, L; Piriou, V; Allaouchiche, B; Aubrun, F; Bastien, O; Lehot, J J; Ayzac, L; Guérin, C

    2013-12-01

    The Berlin definition for acute respiratory distress syndrome (ARDS) is a new proposal for changing the American-European consensus definition but has not been assessed prospectively as yet. In the present study, we aimed to determine (1) the prevalence and incidence of ARDS with both definitions, and (2) the initial characteristics of patients with ARDS and 28-day mortality with the Berlin definition. We performed a 6-month prospective observational study in the ten adult ICUs affiliated to the Public University Hospital in Lyon, France, from March to September 2012. Patients under invasive or noninvasive mechanical ventilation, with PaO2/FiO2 Conference and the Berlin definition criteria. The complete data set was measured at the time of inclusion. Patient outcome was measured at day 28 after inclusion. During the study period 3,504 patients were admitted and 278 fulfilled the American-European Consensus Conference criteria. Among them, 18 (6.5 %) did not comply with the Berlin criterion PEEP ≥ 5 cmH2O and 20 (7.2 %) had PaO2/FiO2 ratio ≤200 while on noninvasive ventilation. By using the Berlin definition in the remaining 240 patients (n = 42 mild, n = 123 moderate, n = 75 severe), the overall prevalence was 6.85 % and it was 1.20, 3.51, and 2.14 % for mild, moderate, and severe ARDS, respectively (P > 0.05 between the three groups). The incidence of ARDS amounted to 32 per 100,000 population per year, with values for mild, moderate, and severe ARDS of 5.6, 16.3, and 10 per 100,000 population per year, respectively (P Berlin definition of ARDS. Neither the stratification by severity nor the PaO2/FiO2 at study entry was independently associated with mortality.

  14. Serial Manifestation of Acute Kidney Injury and Nephrotic Syndrome in a Patient with TAFRO syndrome.

    Science.gov (United States)

    Ito, Seigo; Uchida, Takahiro; Itai, Hiroki; Yamashiro, Aoi; Yamagata, Akira; Matsubara, Hidehito; Imakiire, Toshihiko; Shimazaki, Hideyuki; Kumagai, Hiroo; Oshima, Naoki

    2018-06-06

    A 76-year-old woman suddenly developed anasarca and a fever, and an examination revealed thrombocytopenia, reticulin fibrosis, and acute kidney injury, yielding the diagnosis of TAFRO syndrome. Renal replacement therapy and steroid treatment were soon started. Her proteinuria was minor at first; however, once the kidney function improved, nephrotic syndrome occurred. A kidney biopsy showed membranoproliferative glomerulonephritis-like glomerulopathy with massive macrophage infiltration. Although kidney dysfunction is often observed in TAFRO syndrome patients, its detailed mechanism is unclear. This case suggests that TAFRO syndrome involves both acute kidney injury with minor proteinuria and nephrotic syndrome, and these disorders can develop serially in the same patient.

  15. Rationale and Description of Right Ventricle-Protective Ventilation in ARDS.

    Science.gov (United States)

    Paternot, Alexis; Repessé, Xavier; Vieillard-Baron, Antoine

    2016-10-01

    Pulmonary vascular dysfunction is associated with ARDS and leads to increased right-ventricular afterload and eventually right-ventricular failure, also called acute cor pulmonale. Interest in acute cor pulmonale and its negative impact on outcome in patients with ARDS has grown in recent years. Right-ventricular function in these patients should be closely monitored, and this is helped by the widespread use of echocardiography in intensive care units. Because mechanical ventilation may worsen right-ventricular failure, the interaction between the lungs and the right ventricle appears to be a key factor in the ventilation strategy. In this review, a rationale for a right ventricle-protective ventilation approach is provided, and such a strategy is described, including the reduction of lung stress (ie, the limitation of plateau pressure and driving pressure), the reduction of PaCO2 , and the improvement of oxygenation. Prone positioning seems to be a crucial part of this strategy by protecting both the lungs and the right ventricle, resulting in increased survival of patients with ARDS. Further studies are required to validate the positive impact on prognosis of right ventricle-protective mechanical ventilation. Copyright © 2016 by Daedalus Enterprises.

  16. Viridans streptococcal shock syndrome during bone marrow transplantation.

    Science.gov (United States)

    Martino, R; Manteiga, R; Sánchez, I; Brunet, S; Sureda, A; Badell, I; Argilés, B; Subirá, M; Bordes, R; Domingo-Albós, A

    1995-01-01

    Of 320 patients receiving a marrow transplant at the Hospital de Sant Pau between 1986 and 1992, 12% developed viridans streptococcal bacteremia during severe neutropenia. Five of these patients (13%) developed a rapidly progressive fatal shock syndrome characterized by bilateral pulmonary infiltrates, acute respiratory failure (ARDS) and septic shock early in the transplantation course (6 or 7 days posttransplantation). All patients were transplanted for acute leukemia in remission, and 2 received an allogeneic and 3 an autologous transplant. Four of these subjects were younger than 15 years of age and all had received cyclophosphamide and total body irradiation as conditioning regimen for marrow transplantation. All 5 patients died, and postmortem examinations revealed diffuse pulmonary lesions characteristic of the ARDS. These observations contribute to defining the clinical and pathologic characteristics of this serious complication of intensive anticancer treatment.

  17. Cotard Syndrome without Depressive Symptoms in a Schizophrenic Patient.

    OpenAIRE

    Morgado, P; Ribeiro, R; Cerqueira, JJ

    2015-01-01

    Cotard Syndrome without Depressive Symptoms in a Schizophrenic Patient Introduction . Cotard syndrome is a rare condition characterized by nihilistic delusions concerning body or life that can be found in several neuropsychiatry conditions. It is typically associated with depressive symptoms. Method . We present a case of Cotard syndrome without depressive symptoms in the context of known paranoid schizophrenia. A literature review of Cotard syndrome in schizophrenia was performed. Res...

  18. Down patients with Eisenmenger syndrome: Is bosentan treatment an option?

    NARCIS (Netherlands)

    Duffels, Mariëlle G. J.; Vis, Jeroen C.; van Loon, Rosa L. E.; Berger, Rolf M. F.; Hoendermis, Elke S.; van Dijk, Arie P. J.; Bouma, Berto J.; Mulder, Barbara J. M.

    2009-01-01

    Background: Favorable results of treatment with bosentan in patients with Eisenmenger syndrome are available. However, data in Down patients are lacking. In this study, we evaluate the therapeutic role of bosentan treatment in Down patients with Eisenmenger syndrome. Methods: In this open-label

  19. Down patients with Eisenmenger syndrome : Is bosentan treatment an option?

    NARCIS (Netherlands)

    Duffels, Marielle G. J.; Vis, Jeroen C.; van Loon, Rosa L. E.; Berger, Rolf M. F.; Hoendermis, Elke S.; van Dijk, Arie P. J.; Bouma, Berto J.; Mulder, Barbara J. M.

    2009-01-01

    Background: Favorable results of treatment with bosentan in patients with Eisenmenger syndrome are available. However, data in Down patients are lacking. In this study, we evaluate the therapeutic role of bosentan treatment in Down patients with Eisenmenger syndrome. Methods: In this open-label

  20. Down patients with Eisenmenger syndrome: Is bosentan treatment an option?

    NARCIS (Netherlands)

    Duffels, M.G.; Vis, J.C.; Loon, R.L. van; Berger, R.M.; Hoendermis, E.S.; Dijk, A.P.J. van; Bouma, B.J.; Mulder, B.J.

    2009-01-01

    BACKGROUND: Favorable results of treatment with bosentan in patients with Eisenmenger syndrome are available. However, data in Down patients are lacking. In this study, we evaluate the therapeutic role of bosentan treatment in Down patients with Eisenmenger syndrome. METHODS: In this open-label

  1. Clinical Characteristics of Dry Eye Patients With Chronic Pain Syndromes

    NARCIS (Netherlands)

    Vehof, Jelle; Smitt-Kamminga, Nicole Sillevis; Kozareva, Diana; Nibourg, Simone A.; Hammond, Christopher J.

    PURPOSE: To investigate clinical characteristics of dry eye disease (DED) patients with a chronic pain syndrome. DESIGN: Cross-sectional. study. METHODS: Four hundred twenty-five patients of a tertiary care DED patient cohort in the Netherlands were included. Chronic pain syndromes irritable bowel

  2. Metabolic Syndrome in Patients with Polycystic Ovary Syndrome in Iran

    Directory of Open Access Journals (Sweden)

    Ziba Zahiri

    2016-12-01

    Full Text Available Background: The prevalence of metabolic syndrome (MetS in polycystic ovary syndrome (PCOS has been studied in different populations, but their results were so controversial regarding Iranian women. These controversial data indicated the need for more investigation of MetS characteristics in PCOS patients in our population. So this study aimed to evaluate the clinical and laboratory characteristics and metabolic features of patients with PCOS in Rasht. Materials and Methods: This prospective cross sectional study was conducted on 215 PCOS women who lived in Rasht, north of Iran, from March 2010 to July 2012. The participants were then divided into two groups of women with MetS (n=62 and women without MetS (n=153. The diagnosis of PCOS and MetS were based on the Rotterdam 2003 criteria and the Adult Treatment Panel III (ATP III criteria, respectively. Demographic characteristics, fertility characteristics, family history and laboratory findings were assessed. Results: The prevalence of MetS in women with PCOS was 28.8%. In PCOS women of both groups, the waist circumference (WC exceeded 88cm in 72.6%, hypertension [systolic blood pressure (SBP and/or diastolic blood pressure (DBP ≥130/85mm Hg] was prevalent in 9.3%, fasting blood sugar (FBS level was ≥110 mg/dl in 6%, triglycerides (Tg level were ≥150 mg/dl in 47%, and high-density lipoprotein (HDL level was <50 mg/dl in 86%. The values of WC, SBP, DBP, body mass index (BMI, ovarian size, Tg, cholesterol, FBS, 2-hour blood sugar, aspartate aminotransferase (AST, and alanine aminotransferase (ALT were significantly greater in PCOS women with MetS than women without MetS. Also HDL and luteinizing hormone (LH levels in women with MetS were significantly lower than women without MetS. Conclusion: Prevalence of MetS in PCOS women was 28.8%, indicating that this value is higher than other studies conducted on PCOS women in Iran and other studies conducted on general population in Iran. PCOS women

  3. [Characteristics of traditional Chinese medicine syndromes in patients with acute ischemic stroke of yin or yang syndrome: a multicenter trial].

    Science.gov (United States)

    You, Jin-song; Huang, Yan; Cai, Ye-feng; Guo, Jian-wen; Liang, Wei-xiong; Huang, Pei-xin; Liu, Mao-cai

    2008-04-01

    To explore the composition characteristics of traditional Chinese medicine (TCM) syndromes in patients with acute ischemic stroke of yin or yang syndrome by investigating the characteristics of TCM syndromes at different periods after onset. One thousand two hundred and forty-six patients with acute ischemic stroke were admitted in twenty hospitals. According to the "diagnostic criteria of syndrome differentiation of stroke", the characteristics of syndromes in the patients were investigated at the periods of 1-3 days, 4-10 days and 11-30 days after they had ischemic stroke. General distribution of six basic syndromes was compared between the patients with yin syndrome and the patients with yang syndrome at the three periods. The six basic syndromes were wind syndrome, pathogenic fire syndrome, phlegm syndrome, blood stasis syndrome, qi deficiency syndrome, and syndrome of yin deficiency and yang hyperactivity. The percentages of wind, pathogenic fire, and phlegm syndromes in the patients were decreased at the period of 11-30 days as compared with the period of 1-3 days (87.1% vs 79.3%, 52.1% vs 38.7% and 67.1% vs 57.4% respectively, P0.05). There were no differences in the distribution of yin and yang syndromes among the three periods (P>0.05). The percentages of syndromes of wind, pathogenic fire, phlegm, and yin deficiency and yang hyperactivity were higher (Pfour or five syndromes were higher, and the percentages of single-syndromes and complex syndromes of two syndromes were lower in patients with yang syndrome than in patients with yin syndrome (P<0.05, P<0.01). The most frequent complex syndromes in patients with yin syndrome were complex syndrome of wind, phlegm, blood stasis and qi deficiency, and complex syndrome of wind, phlegm and qi deficiency; while the most frequent complex syndromes in patients with yang syndrome were complex syndrome of wind, pathogenic fire, phlegm and qi deficiency, and complex syndrome of wind, pathogenic fire and phlegm. The

  4. Comparative evaluation of acute respiratory distress syndrome in patients with and without H1N1 infection at a tertiary care referral center

    Directory of Open Access Journals (Sweden)

    Tanvir Samra

    2011-01-01

    Full Text Available H1N1 subtype of influenza A virus has clinical presentation ranging from mild flu like illness to severe lung injury and acute respiratory distress syndrome (ARDS. The aim of our study was to compare the demographic characteristics, clinical presentation, and mortality of critically ill patients with (H1N1+ and without H1N1 infection (H1N1-. We retrospectively analyzed medical charts of patients admitted in "Swine Flu ICU" with ARDS from August 2009 to May 2010. Real-time reverse transcriptase polymerase chain reaction (RT-PCR assay was used for detection of H1N1 virus in the respiratory specimens. Clinical data from 106 (H1N1 , 45; H1N1+, 61 patients was collected and compared. Mean delay in presentation to our hospital was 5.7 ± 3.1 days and co-morbidities were present in two-fifth of the total admissions. Sequential Organ Failure Assessment (SOFA score of patients with and without H1N1 infection was comparable; 7.8 ± 3.5 and 6.6 ± 3.1 on day 1 and 7.2 ± 4.5 and 6.5 ± 3.1 on day 3, respectively. H1N1+ patients were relatively younger in age (34.2 ± 12.9 years vs. 42.8 ± 18.1, P = 0.005 but presented with significantly lower PaO 2 :FiO 2 ratio (87.3 ± 48.7 vs. 114 ± 51.7 in comparison to those who subsequently tested as H1N1 . The total leucocyte counts were significantly lower in H1N1+ patients during the first four days of illness but incidence of renal failure (P = 0.02 was higher in H1N1+ patients. The mortality in both the groups was high (H1N1+, 77%; H1N1, 68% but comparable. There was a mean delay of 5.7 ± 3.1 days in initiation of antivirals. Patients with H1N1 infection were relatively younger in age and with a significantly higher incidence of refractory hypoxia and acute renal failure. Mortality from ARDS reported in our study in both the groups was high but comparable.

  5. Irritable Bowel Syndrome in a Population of African Patients

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    Sylvester Chuks Nwokediuko

    2012-01-01

    Full Text Available Background. Functional dyspepsia is the prototype functional gastrointestinal disorder. This study was designed to determine its prevalence, subtypes, and risk factors associated with the subtypes. Method. Patients with upper gastrointestinal symptoms who presented for endoscopy were administered a questionnaire containing the functional dyspepsia and irritable bowel syndrome modules of the Rome III diagnostic criteria. Results. Of 192 patients who had functional dyspepsia, epigastric pain syndrome, postprandial distress syndrome, and combination of the two subtypes accounted for 79.2%, 62.5%, and 50%, respectively. Multivariate analysis of the risk factors showed that independent predictors of postprandial distress syndrome were alcohol and irritable bowel syndrome while irritable bowel syndrome was independent predictor of epigastric pain syndrome. Alcohol, smoking, and use of nonsteroidal anti-inflammatory drugs were independent predictors of cooccurrence of postprandial distress syndrome and epigastric pain syndrome. Conclusion. Functional dyspepsia accounts for 62.5% of dyspepsia in a population of black African patients. Regarding symptomatology, epigastric pain syndrome, postprandial distress syndrome, and combination of the two subtypes account for 79.2%, 62.5%, and 50%, respectively. Risk factors for functional dyspepsia are irritable bowel syndrome, alcohol, smoking, and use of nonsteroidal anti-inflammatory drugs.

  6. Prone positioning ventilation for treatment of acute lung injury and acute respiratory distress syndrome.

    Science.gov (United States)

    Lan, Mei-juan; He, Xiao-di

    2009-08-01

    Patients who are diagnosed with acute lung injury/acute respiratory distress syndrome (ALI/ARDS) usually have ventilation-perfusion mismatch, severe decrease in lung capacity, and gas exchange abnormalities. Health care workers have implemented various strategies in an attempt to compensate for these pathological alterations. By rotating patients with ALI/ARDS between the supine and prone position, it is possible to achieve a significant improvement in PaO2/FiO2, decrease shunting and therefore improve oxygenation without use of expensive, invasive and experimental procedures. Prone positioning is a safe and effective way to improve ventilation when conventional strategies fail to initiate a patient response. Because a specific cure for ARDS is not available, the goal is to support the patients with therapies that cause the least amount of injury while the lungs have an opportunity to heal. Based on current data, a trial of prone positioning ventilation should be offered to the patients who have ALI/ARDS in the early course of the disease. Published studies exhibit substantial heterogeneity in clinical results, suggesting that an adequately sized study optimizing the duration of proning ventilation strategy is warranted to enable definitive conclusions to be drawn.

  7. A Rare Case of Acute Coronary Syndrome in a Patient With Turner Syndrome.

    Science.gov (United States)

    Kemaloglu, Tugba; Ozer, Nihat; Fikri Yapici, Mehmet

    2016-05-01

    In Turner syndrome, cardiovascular complications are the most important causes of early mortality. Congenital cardiovascular abnormalities are found in approximately one third of Turner syndrome patients. Developments in diagnosis and treatment have decreased the rate of mortality related to these abnormalities. In recent years, many papers have mentioned that coronary artery disease developing at early ages in patients with Turner syndrome causes sudden deaths. The patient, a 27-year-old female was admitted to the emergency room with chest pain at rest. She was diagnosed with Turner Syndrome in her teenage years due to amenorrhea. Patients with ECG changes and cardiac enzyme elevations were treated with acute coronary syndrome. The young woman with Turner Syndrome have several risk factors for early Coronary Artery Disease development. In such cases, dramatic results like sudden death or heart attack at an early age may occur in cases of insufficient follow-up and treatment.

  8. Association between use of lung-protective ventilation with lower tidal volumes and clinical outcomes among patients without acute respiratory distress syndrome: a meta-analysis.

    Science.gov (United States)

    Serpa Neto, Ary; Cardoso, Sérgio Oliveira; Manetta, José Antônio; Pereira, Victor Galvão Moura; Espósito, Daniel Crepaldi; Pasqualucci, Manoela de Oliveira Prado; Damasceno, Maria Cecília Toledo; Schultz, Marcus J

    2012-10-24

    Lung-protective mechanical ventilation with the use of lower tidal volumes has been found to improve outcomes of patients with acute respiratory distress syndrome (ARDS). It has been suggested that use of lower tidal volumes also benefits patients who do not have ARDS. To determine whether use of lower tidal volumes is associated with improved outcomes of patients receiving ventilation who do not have ARDS. MEDLINE, CINAHL, Web of Science, and Cochrane Central Register of Controlled Trials up to August 2012. Eligible studies evaluated use of lower vs higher tidal volumes in patients without ARDS at onset of mechanical ventilation and reported lung injury development, overall mortality, pulmonary infection, atelectasis, and biochemical alterations. Three reviewers extracted data on study characteristics, methods, and outcomes. Disagreement was resolved by consensus. Twenty articles (2822 participants) were included. Meta-analysis using a fixed-effects model showed a decrease in lung injury development (risk ratio [RR], 0.33; 95% CI, 0.23 to 0.47; I2, 0%; number needed to treat [NNT], 11), and mortality (RR, 0.64; 95% CI, 0.46 to 0.89; I2, 0%; NNT, 23) in patients receiving ventilation with lower tidal volumes. The results of lung injury development were similar when stratified by the type of study (randomized vs nonrandomized) and were significant only in randomized trials for pulmonary infection and only in nonrandomized trials for mortality. Meta-analysis using a random-effects model showed, in protective ventilation groups, a lower incidence of pulmonary infection (RR, 0.45; 95% CI, 0.22 to 0.92; I2, 32%; NNT, 26), lower mean (SD) hospital length of stay (6.91 [2.36] vs 8.87 [2.93] days, respectively; standardized mean difference [SMD], 0.51; 95% CI, 0.20 to 0.82; I2, 75%), higher mean (SD) PaCO2 levels (41.05 [3.79] vs 37.90 [4.19] mm Hg, respectively; SMD, -0.51; 95% CI, -0.70 to -0.32; I2, 54%), and lower mean (SD) pH values (7.37 [0.03] vs 7.40 [0

  9. Acute respiratory distress syndrome

    OpenAIRE

    Confalonieri, Marco; Salton, Francesco; Fabiano, Francesco

    2017-01-01

    Since its first description, the acute respiratory distress syndrome (ARDS) has been acknowledged to be a major clinical problem in respiratory medicine. From July 2015 to July 2016 almost 300 indexed articles were published on ARDS. This review summarises only eight of them as an arbitrary overview of clinical relevance: definition and epidemiology, risk factors, prevention and treatment. A strict application of definition criteria is crucial, but the diverse resource-setting scenarios foste...

  10. Restless legs syndrome in hemodialysis patients

    Directory of Open Access Journals (Sweden)

    Shahram Rafie

    2016-01-01

    Full Text Available Restless legs syndrome (RLS is a neurological disorder characterized by uncomfortable sensation of paresthesia in legs that subsequently causes involuntary and continuous movement of the lower limbs, especially at rest. Its prevalence in hemodialysis is more than that in the general population. Different risk factors have been suggested for RLS. We studied the prevalence and risk factors of RLS in 137 hemodialysis patients followed up at our center. The patients completed at least three months on dialysis and fulfilled four criteria for the diagnosis of RLS. We compared the patients with and without RLS, and the odds ratios (ORs were estimated by the logistic regression models. The prevalence of RLS was 36.5% in the study patients. Among the variables, diabetes was the only predicting factor for the development of RLS. The diabetic patients may be afflicted with RLS 2.25 times more than the non-diabetics. Women developed severe RLS 5.23 times more than men. Neurodegeneration, decrease in dopamine level, higher total oxidant status, and neuropathy in diabetic patients may explain the RLS symptoms.

  11. Superior vena cava syndrome in hemodialysis patient

    Directory of Open Access Journals (Sweden)

    Azeb Molhem

    2011-01-01

    Full Text Available Obstruction of blood flow in the superior vena cava (SVC results in symptoms and signs of SVC syndrome. SVC obstruction can be caused either by invasion or external compression of the SVC by contagious pathologic processes involving the right lung, lymph nodes, and other mediastinal structures, or by thrombosis of blood within the SVC. Occasionally, both mechanisms co-exist. We hereby report a case of a 28-year-old male, Saudi patient who was diagnosed with end-stage renal disease and was maintained on regular hemodiaysis via right jugular vein dual lumen catheter for ten months. Three years later, the patient presented with signs and symptoms suggestive of SVC obstruction that was successfully managed with SVC stenting.

  12. Metabolic syndrome in patients with severe mental illness in Gorgan

    Science.gov (United States)

    Kamkar, Mohammad Zaman; Sanagoo, Akram; Zargarani, Fatemeh; Jouybari, Leila; Marjani, Abdoljalal

    2016-01-01

    Background: Metabolic syndrome is commonly associated with cardiovascular diseases and psychiatric mental illness. Hence, we aimed to assess the metabolic syndrome among severe mental illness (SMI). Materials and Methods: The study included 267 patients who were referred to the psychiatric unit at 5th Azar Education Hospital of Golestan University of Medical Sciences in Gorgan, Iran. Results: The mean waist circumference, systolic and diastolic blood pressure, triglyceride and fasting blood glucose levels were significantly higher in the SMI with metabolic syndrome, but the high density lipoprotein (HDL)-cholesterol was significantly lower. The prevalence of metabolic syndrome in SMI patients was 20.60%. There were significant differences in the mean of waist circumference, systolic (except for women) and diastolic blood pressure, triglyceride, HDL-cholesterol and fasting blood glucose in men and women with metabolic syndrome when compared with subjects without metabolic syndrome. The prevalence of metabolic syndrome in SMI women was higher than men. The most age distribution was in range of 30-39 years old. The most prevalence of metabolic syndrome was in age groups 50-59 years old. The prevalence of metabolic syndrome was increased from 30 to 59 years old. Conclusion: The prevalence of metabolic syndrome in patients with SMI in Gorgan is almost similar to those observed in Asian countries. The prevalence of metabolic syndrome was lower than western countries. These observations may be due to cultural differences in the region. It should be mention that the families of mental illness subjects in our country believe that their patients must be cared better than people without mental illness. These findings of this study suggest that mental illness patients are at risk of metabolic syndrome. According to our results, risk factors such as age and gender differences may play an important role in the presence of metabolic syndrome. In our country, women do less

  13. Cotard Syndrome without Depressive Symptoms in a Schizophrenic Patient.

    Science.gov (United States)

    Morgado, Pedro; Ribeiro, Ricardo; Cerqueira, João J

    2015-01-01

    Introduction. Cotard syndrome is a rare condition characterized by nihilistic delusions concerning body or life that can be found in several neuropsychiatry conditions. It is typically associated with depressive symptoms. Method. We present a case of Cotard syndrome without depressive symptoms in the context of known paranoid schizophrenia. A literature review of Cotard syndrome in schizophrenia was performed. Results. Although there are few descriptions of this syndrome in schizophrenia, patients usually present depressive mood and psychomotor retardation, features not seen in our patient. Loss of the sense of the inner self, present in schizophrenia, could explain patient's symptomatology but neurobiological bases of this syndrome remain unclear. Conclusion. Despite not being considered in actual classifications, Cotard syndrome is still relevant and psychiatric evaluation is critical to diagnosing and treating this condition in psychiatric patients.

  14. Epilepsy in patients with Angelman syndrome

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    Fiumara Agata

    2010-04-01

    Full Text Available Abstract Angelman syndrome (AS is a neuro-behavioural, genetically determined condition, characterized by ataxic jerky movements, happy sociable disposition and unprovoked bouts of laughter in association with seizures, learning disabilities and language impairment. Most of the cases are hardly diagnosed during infancy as jerky movements, the cardinal sign, appear later in childhood. AS is caused by a variety of genetic mechanisms involving the 15q 11-13 chromosome. About 70% of cases are due to a "de novo" interstitial deletion in the long arm region, arising on the maternally inherited chromosome. The diagnosis is confirmed by methylation test or by mutation analysis of UBE3A gene. The deletion phenotype is generally linked to a more severe clinical picture in that 95% of patients manifest more severe seizures, severe mental and motor retardation, dysmorphic features and microcephaly. The pathogenesis of epilepsy in AS is still not fully understood. The presence in the commonly deleted region of a cluster of genes coding for 3 subunits of the GABAa receptor complex has lead to the hypothesis that GABA neurotransmission is involved. Epilepsy, often severe and hard to control, is present in 85% of patients within the first three years of life, although less than 25% develop seizures during the first year. It was observed that febrile seizures often precede the diagnosis. Most frequent types are atypical absences, generalized tonic-clonic, atonic or myoclonic seizures, with multiple seizure types occurring in 50% of deleted patients. There is still some doubt about the association with West syndrome. The EEG abnormalities are not themselves pathognomonic of AS and both background activity and epileptic discharges vary even in the same patient with time. Nevertheless, the existence of some suggestive patterns should facilitate the early diagnosis allowing the correct genetic counselling for the family. Some drugs seems to act better than others

  15. Unprovoked Pulmonary Embolism in a Young Patient with Marfan Syndrome.

    Science.gov (United States)

    Pak, Stella; Kilgore, Andrew; Thornhill, Rosanne; Rako, Kyle; Meier, Ali; Pora, Gavriella; Costello, Jillian M; Dee, Christine

    2017-09-05

    Marfan syndrome is a rare connective tissue disorder with a prevalence of approximately 2 to 3 per 10,000 individuals. There have been some reports of young patients with Marfan syndrome developing arteriovenous thromboembolism. These events were unprovoked and recurrent. Owing to its rarity, hypercoagulopathy and other metabolic derangement in patients with Marfan syndrome remains largely unknown. Herein, we report a case of a young man with Marfan syndrome who had myocardial infarction and pulmonary embolism. We hope that this case adds to the scant body of knowledge about this patient population.

  16. Acute Respiratory Distress Syndrome diagnosis after coronary artery bypass: comparison between diagnostic criteria and clinical picture.

    Directory of Open Access Journals (Sweden)

    Manzar Vakili

    2015-01-01

    Full Text Available Acute Respiratory Distress Syndrome (ARDS is a potential complication of cardiac surgery, given that patients undergoing CABG frequently have hypoxemia and pulmonary dysfunction during initial hours after surgery. Thus, ARDS criteria in these patients are more likely to be positive while these criteria may not match the patient`s clinical picture. We aimed to investigate frequency of rapid onset hypoxemia in Pressure of Arterial Oxygen to Fractional Inspired Oxygen Concentration (PaO2/FiO2 less than 200 and diffuse pulmonary infiltrates as two diagnostic criteria forwards and compared these criteria with the clinical picture of the patients after Coronary Artery Bypass Graft (CABG in this study. The study was prospective case series which carried out in about six months. All patients admitted to intensive care unit of Tehran Heart Center, who had undergone CABG on cardiopulmonary pump (CPB recruited in the study. After considering inclusion criteria, age, sex, duration of intubation, arterial blood gas and chest radiography, on 24 hours and 48 hours after admission to the ICU were recorded. Then, patients with rapid onset of hypoxemia (PaO2/FiO2≤200mmHg and diffuse pulmonary infiltrates and without sign or symptoms of obvious heart failure (probable positive ARDS cases criteria were recorded and comparison between these probable positive cases with clinician`s clinical diagnosis (blinded to the study was performed. In this study, a total of 300 patients after on-pump coronary artery bypass surgery were included. Postoperatively, 2 (0.66 % in the 24 hours and 4 (1.33% patients in 48 hours after surgery were positive for the two ARDS criteria according to the checklists, but; nobody had saved persistently ARDS criteria persistently during 48 hours after surgery. At the same time, clinician did not report any case of ARDS among 300 patients. In this study patients with ARDS criteria had no significant differences in age (P.value=0.937 and sex (P

  17. COCHLEAR IMPLANTATION IN A PATIENT WITH USHER'S SYNDROME

    OpenAIRE

    Derinsu, Ufuk; Ciprut, Ayca

    2016-01-01

    Usher's Syndrome is an autosomal recessive disorder characterized by congenital hearing loss and retinitis pigmentosa. Usher’s Syndrome patients with severe to profound sensorineural hearing loss can be considered as candidates for cochlear implantation.This case study reports a deaf-blind with Usher's Syndrome who received a cochlear implant, the audiological evaluation is presented and the therapy sessions are discussed. The patient demonstrated good performance overtime after the...

  18. Cochlear hearing loss in patients with Laron syndrome.

    Science.gov (United States)

    Attias, Joseph; Zarchi, Omer; Nageris, Ben I; Laron, Zvi

    2012-02-01

    The aim of this prospective clinical study was to test auditory function in patients with Laron syndrome, either untreated or treated with insulin-like growth factor I (IGF-I). The study group consisted of 11 patients with Laron syndrome: 5 untreated adults, 5 children and young adults treated with replacement IGF-I starting at bone age Laron syndrome and may be prevented by starting treatment with IGF-I at an early developmental age.

  19. Catastrophizing in Patients with Burning Mouth Syndrome

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    Ana ANDABAK ROGULJ

    2014-01-01

    Full Text Available Background: Burning mouth syndrome (BMS is an idiopathic painful condition which manifests with burning sensations in the oral cavity in patients with clinically normal oral mucosa and without any local and/or systemic causative factor. Catastrophizing is defined as an exaggerated negative orientation toward pain stimuli and pain experience. The aim of this study was to examine the association between catastrophizing and clinical parameters of BMS, and to examine the association between catastrophizing and the quality of life in patients with BMS. Materials and methods: Anonymous questionnaire consisting of 3 parts (demographic and clinical data with 100 mm visual analogue scale (VAS, Croatian version of the Oral Health Impact Profile (OHIP-14 scale and Croatian version of the Pain Catastrophizing scale (PC, was distributed to 30 patients diagnosed with BMS. Results: A higher level of catastrophizing was clinically significant in 30% of the patients. Total catastrophizing score and all three subcomponents of catastrophizing significantly correlated with the intensity of symptoms, but did not correlate with the duration of symptoms. Gender and previous treatment did not affect the catastrophizing. Conclusion: Obtaining the information about catastrophizing could help a clinician to identify patients with negative behavioural patterns. Additional psychological intervention in these individuals could reduce/eliminate negative cognitive factors and improve coping with chronic painful condition such as BMS.

  20. Baraitser–Winter syndrome: An additional Egyptian patient with ...

    African Journals Online (AJOL)

    We report a 3.5 year old male child, second in order of birth of non consanguineous Egyptian parents with Baraitser–Winter syndrome (BRWS). The patient had bilateral colobomas of the iris and choroid. Our patient had also retinal hypoplasia, which was not reported previously in this syndrome, bilateral congenital ptosis, ...

  1. [Anesthetic management of four patients with Fournier syndrome].

    Science.gov (United States)

    Sato, Rui; Tomioka, Toshiya; Orii, Ryo; Yamada, Yoshitsugu

    2008-03-01

    We experienced anesthetic managements of four patients with Fournier syndrome. In the anesthetic management of the patients with Fournier syndrome the following three points should be kept in mind; (a) the necessity of careful preoperative examination, (b) the better anesthesia, and (c) the careful postoperative care.

  2. Peters' plus syndrome in an Egyptian patient with some unusual ...

    African Journals Online (AJOL)

    Some of the features reported in Kabuki make-up syndrome were also present in our patient including mainly hepatomegaly and craniosynostosis. However in our patient some features were present not reported before in both syndromes including thick tounge, thick everted lower lip, anteverted naris, broad thumb and big ...

  3. Cotard Syndrome without Depressive Symptoms in a Schizophrenic Patient

    Directory of Open Access Journals (Sweden)

    Pedro Morgado

    2015-01-01

    Full Text Available Introduction. Cotard syndrome is a rare condition characterized by nihilistic delusions concerning body or life that can be found in several neuropsychiatry conditions. It is typically associated with depressive symptoms. Method. We present a case of Cotard syndrome without depressive symptoms in the context of known paranoid schizophrenia. A literature review of Cotard syndrome in schizophrenia was performed. Results. Although there are few descriptions of this syndrome in schizophrenia, patients usually present depressive mood and psychomotor retardation, features not seen in our patient. Loss of the sense of the inner self, present in schizophrenia, could explain patient’s symptomatology but neurobiological bases of this syndrome remain unclear. Conclusion. Despite not being considered in actual classifications, Cotard syndrome is still relevant and psychiatric evaluation is critical to diagnosing and treating this condition in psychiatric patients.

  4. Low Tidal Volume versus Non-Volume-Limited Strategies for Patients with Acute Respiratory Distress Syndrome. A Systematic Review and Meta-Analysis.

    Science.gov (United States)

    Walkey, Allan J; Goligher, Ewan C; Del Sorbo, Lorenzo; Hodgson, Carol L; Adhikari, Neill K J; Wunsch, Hannah; Meade, Maureen O; Uleryk, Elizabeth; Hess, Dean; Talmor, Daniel S; Thompson, B Taylor; Brower, Roy G; Fan, Eddy

    2017-10-01

    Trials investigating use of lower tidal volumes and inspiratory pressures for patients with acute respiratory distress syndrome (ARDS) have shown mixed results. To compare clinical outcomes of mechanical ventilation strategies that limit tidal volumes and inspiratory pressures (LTV) to strategies with tidal volumes of 10 to 15 ml/kg among patients with ARDS. This is a systematic review and meta-analysis of clinical trials investigating LTV mechanical ventilation strategies. We used random effects models to evaluate the effect of LTV on 28-day mortality, organ failure, ventilator-free days, barotrauma, oxygenation, and ventilation. Our primary analysis excluded trials for which the LTV strategy was combined with the additional strategy of higher positive end-expiratory pressure (PEEP), but these trials were included in a stratified sensitivity analysis. We performed metaregression of tidal volume gradient achieved between intervention and control groups on mortality effect estimates. We used Grading of Recommendations Assessment, Development, and Evaluation methodology to determine the quality of evidence. Seven randomized trials involving 1,481 patients met eligibility criteria for this review. Mortality was not significantly lower for patients receiving an LTV strategy (33.6%) as compared with control strategies (40.4%) (relative risk [RR], 0.87; 95% confidence interval [CI], 0.70-1.08; heterogeneity statistic I 2  = 46%), nor did an LTV strategy significantly decrease barotrauma or ventilator-free days when compared with a lower PEEP strategy. Quality of evidence for clinical outcomes was downgraded for imprecision. Metaregression showed a significant inverse association between larger tidal volume gradient between LTV and control groups and log odds ratios for mortality (β, -0.1587; P = 0.0022). Sensitivity analysis including trials that protocolized an LTV/high PEEP cointervention showed lower mortality associated with LTV (nine trials and 1

  5. Geriatric syndromes in patients with chronic kidney disease

    Directory of Open Access Journals (Sweden)

    Tomasz Gołębiowski

    2016-06-01

    Full Text Available The recent epidemiologic data pointed out, that the general number of patients on hemodialysis is steadily increasing, especially in group of elderly patients over 75 years old. The geriatric syndromes are a multietiological disorder related to physiological aging and partly associated with comorbid conditions. Frailty, falls, functional decline and disability, cognitive impairment and depression are main geriatric syndromes and occurs in patients with impaired renal function more often than among general population. The causes of higher prevalence of those syndromes are not well known, but uremic environment and overall renal replacement therapy may have an important impact on its progress. The patient with geriatric syndrome require comprehensive treatment as well as physical rehabilitation, psychiatric cure and support in everyday activities.Herein below we would like to review recent literature regarding to particular features of main geriatric syndromes in a group of nephrological patients.

  6. Brugada Syndrome in a Patient with Vascular Ehlers-Danlos Syndrome: Sudden Death Risk Amplified.

    Science.gov (United States)

    D'Souza, Jason; Malhotra, Divyanshu; Goud, Aditya; Dahagam, Chanukya; Everett, George

    2017-04-19

    The vast majority of sudden cardiac arrests occur in patients with structural heart disease and in approximately 10% of the cases, it can occur in those with structurally normal hearts. Brugada syndrome is an autosomal dominant sodium channelopathy that has been implicated in sudden deaths. Given their low prevalence, our knowledge about Brugada syndrome is still evolving. Apart from schizophrenia, there have been no reports of associated medical conditions. We recently encountered a patient with vascular Ehlers-Danlos syndrome who was also found to have Brugada syndrome. Both these conditions share some common clinical presentations including a propensity for sudden death.

  7. Lupus anticoagulant-hypoprothrombinemia syndrome and catastrophic antiphospholipid syndrome in a patient with antidomain I antibodies.

    Science.gov (United States)

    Galland, Joris; Mohamed, Shirine; Revuz, Sabine; de Maistre, Emmanuel; de Laat, Bas; Marie, Pierre-Yves; Zuily, Stéphane; Lévy, Bruno; Regnault, Véronique; Wahl, Denis

    2016-07-01

    Lupus anticoagulant-hypoprothrombinemia syndrome is a rare condition characterized by the association of acquired factor II deficiency and lupus anticoagulant. Contrary to classical antiphospholipid syndrome, it may cause severe life-threatening bleeding (89% of published cases). We report a patient, positive for antidomain I antibodies, with initially primary lupus anticoagulant-hypoprothrombinemia syndrome without previous clinical manifestation or underlying systemic disease. Five years later, he experienced the first systemic lupus erythematous flare. Within a few days, catastrophic antiphospholipid syndrome was diagnosed with heart, liver and kidney involvement. The patient recovered under pulse steroids, intravenous heparin and intravenous immunoglobulins.

  8. Patients with carcinoid syndrome exhibit symptoms of aggressive impulse dysregulation

    NARCIS (Netherlands)

    Russo, S; Boon, JC; Kema, IP; Willemse, PHB; den Boer, JA; Korf, J; de Vries, EGE

    2004-01-01

    Objective: Carcinoid tumors can produce excessive amounts of biogenic amines, notably serotonin. We assessed psychiatric symptoms in carcinoid patients and peripheral metabolism of tryptophan, the precursor of serotonin. Methods: Twenty consecutive patients with carcinoid syndrome underwent a

  9. Adropin Levels in Polycystic Ovary Syndrome Patients

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    Hacer Sen

    2017-01-01

    Full Text Available Aim: Polycystic ovary syndrome (PCOS is one of the most commonly observed endocrinopathies in women of reproductive age. Women with PCOS are said to have increased classic risk factors for cardiovascular disease, hypertension, dyslipidemia, diabetes, and obesity, in addition to non-classic risk factors such as an increase in C-reactive protein (CRP, homocysteine, and tumor necrosis factor-%u03B1. Adropin is a protein thought to play a role in maintaining energy homeostasis and insulin response. The aim of our study is to investigate the relationship between levels of adropin and insulin resistance in PCOS patients with insulin resistance and an increased risk of diabetes.Material and Method: Fifty-seven female patients (30 patients with PCOS and 27 healthy control subjects were enrolled in this study. All patient%u2019s body mass index and insulin resistance were calculated. The adropin levels were measured using commercial kits based on a competitive plasma EIA (enzyme immunoassay method. Results: The adropin levels in the patient group were 10.79 ng/L, while the value was 13.02 ng/L in the control group, and the difference was statistically significant (p=0.04. There was a significant negative correlation between the adropin levels and the insulin, aspartate aminotransferase (AST, triglyseride (TG, and homeostasis model assessment of insulin resistance (HOMA-IR levels (p=0.03, p=0.03, p=0.04, and p=0.02, respectively. Discussion: In our study, the adropin level which is associated with insulin resistance, was found to be decreased in patients with PCOS. We think that it would be valuable to conduct new studies for the evaluation of adropin related clinical conditions leading to insulin resistance in patients with PCOS.

  10. Metalloproteinase inhibition prevents acute respiratory distress syndrome.

    Science.gov (United States)

    Carney, D E; McCann, U G; Schiller, H J; Gatto, L A; Steinberg, J; Picone, A L; Nieman, G F

    2001-08-01

    The acute respiratory distress syndrome (ARDS) occurs in patients with clearly identifiable risk factors, and its treatment remains merely supportive. We postulated that patients at risk for ARDS can be protected against lung injury by a prophylactic treatment strategy that targets neutrophil-derived proteases. We hypothesized that a chemically modified tetracycline 3 (COL-3), a potent inhibitor of neutrophil matrix metalloproteinases (MMPs) and neutrophil elastase (NE) with minimal toxicity, would prevent ARDS in our porcine endotoxin-induced ARDS model. Yorkshire pigs were anesthetized, intubated, surgically instrumented for hemodynamic monitoring, and randomized into three groups: (1) control (n = 4), surgical instrumentation only; (2) lipopolysaccharide (LPS) (n = 4), infusion of Escherichia coli lipopolysaccharide at 100 microg/kg; and (3) COL-3 + LPS (n = 5), ingestion of COL-3 (100 mg/kg) 12 h before LPS infusion. All animals were monitored for 6 h following LPS or sham LPS infusion. Serial bronchoalveolar lavage (BAL) samples were analyzed for MMP concentration by gelatin zymography. Lung tissue was fixed for morphometric assessment at necropsy. LPS infusion was marked by significant (P decrement in arterial oxygen partial pressure (P(a)O(2)) (LPS = 66 +/- 15 mm Hg, Control = 263 +/- 25 mm Hg) 6 h following LPS or sham LPS infusion, respectively. Pretreatment with COL-3 reduced the above pathophysiological changes 6 h following LPS infusion (P(plat) = 18.5 +/- 1.7 mm Hg, P(a)O(2) = 199 +/- 35 mm Hg; P = NS vs control). MMP-9 and MMP-2 concentration in BAL fluid was significantly increased between 2 and 4 h post-LPS infusion; COL-3 reduced the increase in MMP-9 and MMP-2 concentration at all time periods. Morphometrically LPS caused a significant sequestration of neutrophils and monocytes into pulmonary tissue. Pretreatment with COL-3 ameliorated this response. The wet/dry lung weight ratio was significantly greater (P single prophylactic treatment with COL

  11. Soluble Receptor for Advanced Glycation End-Products Predicts Impaired Alveolar Fluid Clearance in Acute Respiratory Distress Syndrome.

    Science.gov (United States)

    Jabaudon, Matthieu; Blondonnet, Raiko; Roszyk, Laurence; Bouvier, Damien; Audard, Jules; Clairefond, Gael; Fournier, Mathilde; Marceau, Geoffroy; Déchelotte, Pierre; Pereira, Bruno; Sapin, Vincent; Constantin, Jean-Michel

    2015-07-15

    Levels of the soluble form of the receptor for advanced glycation end-products (sRAGE) are elevated during acute respiratory distress syndrome (ARDS) and correlate with severity and prognosis. Alveolar fluid clearance (AFC) is necessary for the resolution of lung edema but is impaired in most patients with ARDS. No reliable marker of this process has been investigated to date. To verify whether sRAGE could predict AFC during ARDS. Anesthetized CD-1 mice underwent orotracheal instillation of hydrochloric acid. At specified time points, lung injury was assessed by analysis of blood gases, alveolar permeability, lung histology, AFC, and plasma/bronchoalveolar fluid measurements of proinflammatory cytokines and sRAGE. Plasma sRAGE and AFC rates were also prospectively assessed in 30 patients with ARDS. The rate of AFC was inversely correlated with sRAGE levels in the plasma and the bronchoalveolar fluid of acid-injured mice (Spearman's ρ = -0.73 and -0.69, respectively; P < 10(-3)), and plasma sRAGE correlated with AFC in patients with ARDS (Spearman's ρ = -0.59; P < 10(-3)). Similarly, sRAGE levels were significantly associated with lung injury severity, and decreased over time in mice, whereas AFC was restored and lung injury resolved. Our results indicate that sRAGE levels could be a reliable predictor of impaired AFC during ARDS, and should stimulate further studies on the pathophysiologic implications of RAGE axis in the mechanisms leading to edema resolution. Clinical trial registered with www.clinicaltrials.gov (NCT 00811629).

  12. Catastrophic primary antiphospholipid syndrome

    International Nuclear Information System (INIS)

    Kim, Dong Hun; Byun, Joo Nam; Ryu, Sang Wan

    2006-01-01

    Catastrophic antiphospholipid syndrome (CAPLS) was diagnosed in a 64-year-old male who was admitted to our hospital with dyspnea. The clinical and radiological examinations showed pulmonary thromboembolism, and so thromboembolectomy was performed. Abdominal distension rapidly developed several days later, and the abdominal computed tomography (CT) abdominal scan revealed thrombus within the superior mesenteric artery with small bowel and gall bladder distension. Cholecystectomy and jejunoileostomy were performed, and gall bladder necrosis and small bowel infarction were confirmed. The anticardiolipin antibody was positive. Anticoagulant agents and steroids were administered, but the patient expired 4 weeks after surgery due to acute respiratory distress syndrome (ARDS). We report here on a case of catastrophic APLS with manifestations of pulmonary thromboembolism, rapidly progressing GB necrosis and bowel infarction

  13. Catastrophic primary antiphospholipid syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Dong Hun; Byun, Joo Nam [Chosun University Hospital, Gwangju (Korea, Republic of); Ryu, Sang Wan [Miraero21 Medical Center, Gwangju (Korea, Republic of)

    2006-09-15

    Catastrophic antiphospholipid syndrome (CAPLS) was diagnosed in a 64-year-old male who was admitted to our hospital with dyspnea. The clinical and radiological examinations showed pulmonary thromboembolism, and so thromboembolectomy was performed. Abdominal distension rapidly developed several days later, and the abdominal computed tomography (CT) abdominal scan revealed thrombus within the superior mesenteric artery with small bowel and gall bladder distension. Cholecystectomy and jejunoileostomy were performed, and gall bladder necrosis and small bowel infarction were confirmed. The anticardiolipin antibody was positive. Anticoagulant agents and steroids were administered, but the patient expired 4 weeks after surgery due to acute respiratory distress syndrome (ARDS). We report here on a case of catastrophic APLS with manifestations of pulmonary thromboembolism, rapidly progressing GB necrosis and bowel infarction.

  14. Effects of Early Continuous Venovenous Hemofiltration on E-Selectin, Hemodynamic Stability, and Ventilatory Function in Patients with Septic-Shock-Induced Acute Respiratory Distress Syndrome

    Directory of Open Access Journals (Sweden)

    Jian-biao Meng

    2016-01-01

    Full Text Available Objective. To investigate the effects of 72-hour early-initiated continuous venovenous hemofiltration (ECVVH treatment in patients with septic-shock-induced acute respiratory distress syndrome (ARDS (not acute kidney injury, AKI with regard to serum E-selectin and measurements of lung function and hemodynamic stability. Methods. This prospective nonblinded single institutional randomized study involved 51 patients who were randomly assigned to receive or not receive ECVVH, an ECVVH group (n=24 and a non-ECVVH group (n=27. Besides standard therapies, patients in ECVVH group underwent CVVH for 72 h. Results. At 0 and 24 h after initiation of treatment, arterial partial pressure of oxygen/fraction of inspired oxygen (PaO2/FiO2 ratio, extravascular lung water index (EVLWI, and E-selectin level were not significantly different between groups (all P>0.05. Compared to non-ECVVH group, PaO2/FiO2 is significantly higher and EVLWI and E-selectin level are significantly lower in ECVVH group (all P<0.05 at 48 h and 72 h after initiation of treatment. The lengths of mechanical ventilation and stay in intensive care unit (ICU were shorter in ECVVH group (all P<0.05, but there was no difference in 28-day mortality between two groups. Conclusions. In patients with septic-shock-induced ARDS (not AKI, treatment with ECVVH in addition to standard therapies improves endothelial function, lung function, and hemodynamic stability and reduces the lengths of mechanical ventilation and stay in ICU.

  15. Prevalence of Burnout Syndrome in patients admitted with acute coronary syndrome.

    Science.gov (United States)

    Prosdócimo, Ana Cláudia Giaxa; Lucina, Luciane Boreki; Marcia, Olandoski; Jobs, Priscila Megda João; Schio, Nicolle Amboni; Baldanzi, Fernanda Fachin; Costantini, Costantino Ortiz; Benevides-Pereira, Ana Maria Teresa; Guarita-Souza, Luiz Cesar; Faria-Neto, José Rocha

    2015-03-01

    Burnout Syndrome is the extreme emotional response to chronic occupational stress, manifesting as physical and mental exhaustion. Although associated with higher prevalence of cardiovascular risk factors, no study so far has evaluated whether the Burnout Syndrome could be a prevalent factor in non-elderly individuals active in the labor market, admitted for acute coronary syndrome (ACS). To evaluate the prevalence of the Burnout Syndrome in non-elderly, economically active patients, hospitalized with ACS. Cross-sectional study conducted in a tertiary and private cardiology center, with economically active patients aged Burnout Syndrome was evaluated with the Burnout Syndrome Inventory (BSI), which assesses workplace conditions and four dimensions that characterize the syndrome: emotional exhaustion (EE), emotional distancing (EmD), dehumanization (De) and professional fulfillment (PF). The Lipp's Stress Symptoms Inventory for Adults (LSSI) was applied to evaluate global stress. Of 830 patients evaluated with suspected ACS, 170 met the study criteria, 90% of which were men, overall average age was 52 years, and 40.5% had an average income above 11 minimum wages. The prevalence of the Burnout Syndrome was 4.1%. When we evaluated each dimension individually, we found high EE in 34.7%, high De in 52.4%, high EDi in 30.6%, and low PF in 5.9%. The overall prevalence of stress was 87.5%. We found a low prevalence of Burnout Syndrome in an economically active, non-elderly population among patients admitted for ACS in a tertiary and private hospital.

  16. [Refeeding syndrome in geriatric patients : A frequently overlooked complication].

    Science.gov (United States)

    Wirth, Rainer; Diekmann, Rebecca; Fleiter, Olga; Fricke, Leonhardt; Kreilkamp, Annika; Modreker, Mirja Katrin; Marburger, Christian; Nels, Stefan; Schaefer, Rolf; Willschrei, Heinz-Peter; Volkert, Dorothee

    2018-01-01

    The refeeding syndrome is a life-threatening complication that can occur after initiation of a nutrition therapy in malnourished patients. If the risk factors and pathophysiology are known, the refeeding syndrome can effectively be prevented and treated, if recognized early. A slow increase of food intake and the close monitoring of serum electrolyte levels play an important role. Because the refeeding syndrome is not well known and the symptoms may vary extremely, this complication is poorly recognized, especially against the background of geriatric multimorbidity. This overview is intended to increase the awareness of the refeeding syndrome in the risk group of geriatric patients.

  17. Restless legs syndrome in patients on dialysis

    International Nuclear Information System (INIS)

    AlJahdali, Hamdan H; AlQadhi, Waleed A; Khogeer, Haithm A; AlHejaili, Fayez F; Al Sayyari, Abdullah A; AlGhamdi, Saeed M

    2009-01-01

    Restless legs syndrome (RLS) is an extremely distressing problem experienced by patients on dialysis; the prevalence appears to be greater than in the general population, with a wide variation from 6.6% to 80%. The diagnosis of RLS is a clinical one, and its definition has been clarified and standardized by internationally recognized diagnostic criteria, published in 1995 by the International Restless Legs Syndrome Study Group (IRLSSG). This study was designed to find out the prevalence of RLS in Saudi patients with end-stage renal disease (ESRD) on maintenance dialysis. This is a cross sectional study carried out between May and Sept 2007 at two centers, King Abdulaziz Medical City-King Fahad National Guard Hospital (KAMC-KFNGH), Riyadh and King Faisal Specialist Hospital and Research Centre (KFHRC), Jeddah, Saudi Arabia. Data were gathered on 227 Saudi patients on chronic maintenance hemodialysis or chronic peritoneal dialysis. The prevalence of RLS was measured using IRLSSG's RLS Questionnaire (RLSQ). Potential risk factors for RLS including other sleep disorders, underlying cause of chronic renal failure, duration on dialysis, dialysis shift, biochemical tests and demographic data were also evaluated. The overall prevalence of RLS was 50.22% including 53.7% males and 46.3% females. Their mean age was 55.7 + - 17.2 years and mean duration on dialysis 40.4 + - 37.8 months. Significant predictors of RLS were history of diabetes mellitus (DM), coffee intake, afternoon dialysis, gender and type of dialysis (P= 0.03, 0.01, < 0.001, 0.05 and 0.009 respectively). Patients with RLS were found to be at increased risk of having insomnia and excessive daytime sleepiness (EDS) (P= < 0.001 and 0.001, respectively). Our study suggests that RLS is a very common problem in dialysis population and was significantly associated with other sleep disorders, particularly insomnia, and EDS. Optimal care of dialysis patient should include particular attention to the diagnosis and

  18. Restless legs syndrome in patients on dialysis

    Directory of Open Access Journals (Sweden)

    Al-Jahdali Hamdan

    2009-01-01

    Full Text Available Restless legs syndrome (RLS is an extremely distressing problem experienced by patients on dialysis; the prevalence appears to be greater than in the general population, with a wide variation from 6.6% to 80%. The diagnosis of RLS is a clinical one, and its definition has been clarified and standardized by internationally recognized diagnostic criteria, published in 1995 by the International Restless Legs Syndrome Study Group (IRLSSG. This study was designed to find out the prevalence of RLS in Saudi patients with end-stage renal disease (ESRD on maintenance dialysis. This is a cross sectional study carried out between May and Sept 2007 at two centers, King Abdulaziz Medical City-King Fahad National Guard Hospital (KAMC-KFNGH, Riyadh and King Faisal Specialist Hospital and Research Centre (KFHRC, Jeddah, Saudi Arabia. Data were gathered on 227 Saudi patients on chronic maintenance hemodialysis or chronic peritoneal dialysis. The prevalence of RLS was measured using IRLSSG′s RLS Questionnaire (RLSQ. Potential risk factors for RLS including other sleep disorders, underlying cause of chronic renal failure, duration on dialysis, dialysis shift, biochemical tests and demographic data were also evaluated. The overall prevalence of RLS was 50.22% including 53.7% males and 46.3% females. Their mean age was 55.7 ± 17.2 years and mean duration on dialysis 40.4 ± 37.8 months. Significant predictors of RLS were history of diabetes mellitus (DM, coffee intake, afternoon dialysis, gender and type of dialysis (P= 0.03, 0.01, < 0.001, 0.05 and 0.009 respectively. Patients with RLS were found to be at increased risk of having insomnia and excessive daytime sleepiness (EDS (P= < 0.001 and 0.001, respectively. Our study suggests that RLS is a very common problem in dialysis population and was significantly associated with other sleep disorders, particularly insomnia, and EDS. Optimal care of dialysis patient should include particular attention to the diagnosis

  19. Nitrofurantoin-induced acute respiratory distress syndrome during pregnancy: A case report

    Directory of Open Access Journals (Sweden)

    Sherif S. Wahba

    2014-01-01

    Full Text Available Acute respiratory distress syndrome (ARDS is a rarely seen complication with nitrfurantoin. We report improvement of a parturient who was admitted to our hospital’s obstetrical unit with life threatening nitrofurantoin-induced acute respiratory failure. She had been taking nitrofurantoin for one week for urinary tract infection (UTI. Her chest radiography showed bilateral parenchymal infiltrates of the lung. The patient responded well to nitrofurantoin discontinuation and methylprednisolone infusion 1 mg/kg/day.

  20. Trauma indices for prediction of acute respiratory distress syndrome.

    Science.gov (United States)

    Afshar, Majid; Smith, Gordon S; Cooper, Richard S; Murthi, Sarah; Netzer, Giora

    2016-04-01

    A myriad of trauma indices has been validated to predict probability of trauma survival. We aimed to compare the performance of commonly used indices for the development of the acute respiratory distress syndrome (ARDS). Historic, observational cohort study of 27,385 consecutive patients admitted to a statewide referral trauma center between July 11, 2003 and October 31, 2011. A validated algorithm was adapted to identify patients with ARDS. Each trauma index was evaluated in logistic regression using the area under the receiver operating characteristic curve. The case rate for ARDS development was 5.8% (1594). The receiver operating characteristics for injury severity score (ISS) had the best discrimination and had an area under the curve of 0.88 (95% confidence interval [CI] = 0.87-0.89). Glasgow coma score (0.71, 95% CI = 0.70-0.73), A Severity Characterization of Trauma (0.86, 95% CI = 0.85-0.87), Revised Trauma Score (0.71, 95% CI = 0.70-0.72) and thorax Abbreviated Injury Score (0.73, 95% CI = 0.72-0.74) performed worse (P < 0.001) and Trauma and Injury Severity Score (0.88, 95% CI = 0.87-0.88) performed equivocally (P = 0.51) in comparison to ISS. Using a cutoff point ISS ≥16, sensitivity and specificity were 84.9% (95% CI = 83.0%-86.6%) and 75.6% (95% CI = 75.1%-76.2%), respectively. Among commonly used trauma indices, ISS has superior or equivocal discriminative ability for development of ARDS. A cutoff point of ISS ≥16 provided good sensitivity and specificity. The use of ISS ≥16 is a simple method to evaluate ARDS in trauma epidemiology and outcomes research. Copyright © 2016 Elsevier Inc. All rights reserved.

  1. Acute respiratory distress syndrome and acute lung injury.

    Science.gov (United States)

    Dushianthan, A; Grocott, M P W; Postle, A D; Cusack, R

    2011-09-01

    Acute respiratory distress syndrome (ARDS) is a life threatening respiratory failure due to lung injury from a variety of precipitants. Pathologically ARDS is characterised by diffuse alveolar damage, alveolar capillary leakage, and protein rich pulmonary oedema leading to the clinical manifestation of poor lung compliance, severe hypoxaemia, and bilateral infiltrates on chest radiograph. Several aetiological factors associated with the development of ARDS are identified with sepsis, pneumonia, and trauma with multiple transfusions accounting for most cases. Despite the absence of a robust diagnostic definition, extensive epidemiological investigations suggest ARDS remains a significant health burden with substantial morbidity and mortality. Improvements in outcome following ARDS over the past decade are in part due to improved strategies of mechanical ventilation and advanced support of other failing organs. Optimal treatment involves judicious fluid management, protective lung ventilation with low tidal volumes and moderate positive end expiratory pressure, multi-organ support, and treatment where possible of the underlying cause. Moreover, advances in general supportive measures such as appropriate antimicrobial therapy, early enteral nutrition, prophylaxis against venous thromboembolism and gastrointestinal ulceration are likely contributory reasons for the improved outcomes. Although therapies such as corticosteroids, nitric oxide, prostacyclins, exogenous surfactants, ketoconazole and antioxidants have shown promising clinical effects in animal models, these have failed to translate positively in human studies. Most recently, clinical trials with β2 agonists aiding alveolar fluid clearance and immunonutrition with omega-3 fatty acids have also provided disappointing results. Despite these negative studies, mortality seems to be in decline due to advances in overall patient care. Future directions of research are likely to concentrate on identifying potential

  2. Research advances in traditional Chinese medicine syndromes in cancer patients.

    Science.gov (United States)

    Ji, Qing; Luo, Yun-quan; Wang, Wen-hai; Liu, Xuan; Li, Qi; Su, Shi-bing

    2016-01-01

    Traditional Chinese medicine (TCM) syndrome, also known as TCM ZHENG or TCM pattern, is an integral and essential part of TCM theory that helps to guide the design of individualized treatments. A TCM syndrome, in essence, is a characteristic profile of all clinical manifestations in one patient that can be readily identified by a TCM practitioner. In this article, the authors reviewed the presentations of TCM syndromes in seven common malignancies (liver, lung, gastric, breast, colorectal, pancreatic and esophageal cancers), the objectivity and the standardization of TCM syndrome differentiation, the evaluation of TCM syndrome modeling in cancer research, and syndrome differentiation-guided TCM treatment of cancers. A better understanding of TCM syndrome theory, as well as its potential biological basis, may contribute greatly to the clinical TCM diagnosis and the treatment of cancer.

  3. Gender differences in patients with obesity hypoventilation syndrome.

    Science.gov (United States)

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

    2016-08-01

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

  4. Cryoglobulinemic vasculitis in a patient with CREST syndrome.

    Science.gov (United States)

    Hurst, Rebecca L; Berianu, Florentina; Ginsburg, William W; Klein, Christopher J; Englestad, Janean K; Kennelly, Kathleen D

    2014-10-01

    Cryoglobulinemic vasculitis is a rare entity. Although it has been reported in diffuse systemic sclerosis, it has not been reported in calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly and telangiectasia (CREST) syndrome. We report a patient with cryoglobulinemic vasculitis with CREST syndrome who did not have typical clinical features of vasculitis. This 58-year-old woman presented with mild generalized weakness and a diagnosis of CREST syndrome, which included Raynaud's syndrome, dysphagia and telangiectasias. She was positive for serum cryoglobulins, which led to a sural nerve biopsy. The biopsy results were consistent with cryoglobulinemic vasculitis. Cryoglobulinemic vasculitis has not been previously reported in CREST syndrome to our knowledge. Additionally, the patient also had limited clinical symptoms. Our patient displays the importance of checking for cryoglobulins and obtaining a nerve biopsy when the serum is positive. Both of these diagnostic tests were integral for directing appropriate treatment for this patient. Copyright © 2014 Elsevier Ltd. All rights reserved.

  5. Femur Neck Fracture in a Young Marfan Syndrome Patient.

    Science.gov (United States)

    Kwon, Yong-Uk; Kong, Gyu-Min; Park, Jun-Ho

    2016-12-01

    Marfan syndrome is an autosomal dominant and could decrease bone mineral density. So patients with Marfan syndrome could vulnerable to trauma in old ages. We present the first report, to the best of our knowledge, of a rare fracture of the femoral neck with a minor traumatic history in a juvenile Marfan syndrome patient whose physis is still open. Although the patient is young, her bone mineral density was low and the geometry of femur is changed like old ages. The femur neck fracture in children is very rare and only caused by high energy trauma, we concluded that the Marfan syndrome makes the bone weaker in young age and preventative medications to avoid fractures in younger Marfan syndrome patients are necessary in early ages.

  6. Cardiac findings in 31 patients with Noonan's syndrome

    Directory of Open Access Journals (Sweden)

    Bertola Débora Romeo

    2000-01-01

    Full Text Available OBJECTIVE: To evaluate cardiac findings in 31 Noonan syndrome patients. METHODS: Thirty-one (18 males and 13 femalespatients from 26 families affected with Noonan's syndrome were evaluated from the cardiac point of view with electrocardiography and echodopplercardiography. RESULTS: Twenty patients had some type of cardiac abnormality. The most frequent was pulmonary valve stenosis followed by hypertrophic myocardiopathy, commonly associated with valve defects. Upper deviation of the QRS axis was observed in 80% of these patients. CONCLUSION: In view of the high frequency and diversity of cardiac abnormalities present in Noonan syndrome, cardiac evaluation with electrocardiography and echocardiography should be performed in all patients diagnostically suspected of having this disease.

  7. Physiologically based indices of volumetric capnography in patients receiving mechanical ventilation.

    Science.gov (United States)

    Romero, P V; Lucangelo, U; Lopez Aguilar, J; Fernandez, R; Blanch, L

    1997-06-01

    Several indices of ventilatory heterogeneity can be identified from the expiratory CO2 partial pressure or CO2 elimination versus volume curves. The aims of this study were: 1) to analyse several computerizable indices of volumetric capnography in order to detect ventilatory disturbances; and 2) to establish the relationship between those indices and respiratory system mechanics in subjects with normal lungs and in patients with acute respiratory distress syndrome (ARDS), both receiving mechanical ventilation. We studied six normal subjects and five patients with early ARDS mechanically ventilated at three levels of tidal volume (VT). Respiratory system mechanics were assessed by end-expiratory and end-inspiratory occlusion methods, respectively. We determined Phase III slopes, Fletcher's efficiency index, Bohr's dead space (VD,Bohr/VT), and the ratio of alveolar ejection volume to tidal volume (VAE/VT) from expiratory capnograms, as a function of expired volume. Differences between normal subjects and ARDS patients were significant both for capnographic and mechanical parameters. Changes in VT significantly altered capnographic indices in normal subjects, but failed to change ventilatory mechanics and VAE/VT in ARDS patients. After adjusting for breathing pattern, VAE/VT exhibited the best correlation with the mechanical parameters. In conclusion, volumetric capnography, and, specifically, the ratio of alveolar ejection volume to tidal volume allows evaluation and monitoring of ventilatory disturbances in patients with adult respiratory distress syndrome.

  8. Adult respiratory distress syndrome

    International Nuclear Information System (INIS)

    Murphy, C.H.; Colvin, R.S.

    1987-01-01

    Due to improved emergency resuscitation procedures, and with advancing medical technology in the field of critical care, an increasing number of patients survive the acute phase of shock and catastrophic trauma. Patients who previously died of massive sepsis, hypovolemic or hypotensive shock, multiple fractures, aspiration, toxic inhalation, and massive embolism are now surviving long enough to develop previously unsuspected and unrecognized secondary effects. With increasing frequency, clinicians are recognizing the clinical and radiographic manifestations of pathologic changes in the lungs occurring secondary to various types of massive insult. This paper gives a list of diseases that have been shown to precipitate or predispose to diffuse lung damage. Various terms have been used to describe the lung damage and respiratory failure secondary to these conditions. The term adult respiratory distress syndrome (ARDS) is applied to several cases of sudden respiratory failure in patients with previously healthy lungs following various types of trauma or shock. Numerous investigations and experiments have studied the pathologic changes in ARDS, and, while there is still no clear indication of why it develops, there is now some correlation of the sequential pathologic developments with the clinical and radiographic changes

  9. Mutiple Spontaneous Rib Fractures in Patient with Cushing's Syndrome.

    Science.gov (United States)

    Lee, Hyun Jung; Je, Ji Hye; Seo, Ji Hye; Na, Young Ju; Yoo, Hye Jin

    2014-11-01

    Glucocorticoid (GC) excess, including Cushing's syndrome, is a common cause of secondary osteoporosis. Thirty to fifty percent of Cushing's syndrome patients experience non-traumatic fractures, which is often the presenting manifestation of Cushing's syndrome. However, there have been rare cases of Cushing's syndrome diagnosed only based upon bone manifestations. We describe a case of Cushing's syndrome that was diagnosed in a 44-year-old woman who initially visited our hospital due to multiple non-traumatic rib fractures. She did not exhibit any other manifestations of Cushing's syndrome such as moon face, buffalo hump or abdominal striae. Initially, we evaluated her for bone metastases from a cancer of unknown origin, but there was no evidence of metastatic cancer. Instead, we found a left adrenal incidentaloma. As a result of the hormone study, she was diagnosed as having Cushing's syndrome. Interestingly, her bony manifestation of Cushing's syndrome, which was evident in the bone scan and bone mineral densitometry, completely recovered after a left adrenalectomy. Therefore, the possibility of Cushing's syndrome as a cause of secondary osteoporosis should be considered in young patients with non-traumatic multiple fractures, with or without any other typical features of Cushing's syndrome.

  10. Continuous distending pressure effects on variables contributing to oxygenation in healthy and ARDS model pigs during HFOV

    Science.gov (United States)

    Laviola, Marianna; Hajny, Ondrej; Roubik, Karel

    2014-10-01

    High frequency oscillatory ventilation (HFOV) is an alternative mode of mechanical ventilation. HFOV has been shown to provide adequate ventilation and oxygenation in acute respiratory distress syndrome (ARDS) patients and may represent an effective lung-protective ventilation in patients where conventional ventilation is failing. The aim of this study is to evaluate effects of continuous distending pressure (CDP) on variables that contribute to the oxygenation in healthy and ARDS lung model pigs. Methods. In order to simulate a lung disease, lung injury was induced by lavage with normal saline with detergent in three pigs. HFOV ventilation was applied before and after the lung lavage. CDP was stepwise increased by 2 cmH2O, until the maximum CDP (before the lung lavage 32 cmH2O and after the lung lavage 42 cmH2O) and then it was stepwise decreased by 2 cmH2O to the initial value. In this paper we analyzed the following parameters acquired during our experiments: partial pressure of oxygen in arterial blood (PaO2), cardiac output (CO) and mixed venous blood oxygen saturation (SvO2). In order to find how both PaO2 and CO affected SvO2 during the increase of CDP before and after lavage, a nonlinear regression fitting of the response in SvO2 on the predictors (PaO2 and CO) was implemented. Results. Before the lavage, with increasing of CDP, PaO2 remained constant, CO strongly decreased and SvO2 slightly decreased. After the lavage, with increasing of CDP, PaO2 strongly increased, CO decreased and SvO2 increased. So, development of SvO2 followed the PaO2 and CO trends. Changes in PaO2 and CO occur at decisive CDP step and it was much higher after the lung lavage compared to the healthy lungs. The implemented nonlinear model gives a good goodness of fitting in all three pigs. The values of PaO2 and CO estimated coefficients changed at the same decisive step of CDP identified by the trends. Also the algorithm identified a CDP step much higher after the lung lavage

  11. The Bentall procedure in patients with Marfan's syndrome.

    Science.gov (United States)

    Nguyen, K H; Ergin, M A; Galla, J D; Lansman, S L; McCullough, J N; Griepp, R B

    1997-01-01

    From 1985 to 1996, 18 patients with Marfan's syndrome underwent the Bentall procedure at Mount Sinai Hospital. They are compared with 38 patients aged Marfan's syndrome who also underwent composite valve-graft replacement of the ascending aorta. The mean age of the non-Marfan group was 33 while that of the Marfan group was 29. Nearly three-quarters of each group were male. Aortic dissection was present in 33% of the Marfan group but only in 18% of the other group. Seventy-eight percent of the Marfan patients and 89% of the non-Marfan patients had aortic regurgitation. Twenty-one percent of the patients in the control group nevertheless had gross aortic pathology suggestive of Marfan's syndrome and may have had variants of the syndrome; 26% of the non-Marfan group had a bicuspid aortic valve. A modification of the Bentall procedure with implantation of coronary artery buttons was performed in the majority of the patients in both groups. Three patients, all in the group with Marfan's syndrome, required a concomitant mitral valve procedure. There was one death in each group. Two non-Marfan patients required reoperation; neither had dissection. Four patients with Marfan's syndrome underwent reoperation for distal disease in the aorta; they comprised one third of the Marfan patients who had aortic dissection. Three late complications occurred in the group with Marfan's syndrome: progressive cardiomyopathy; myocardial infarction; and late tamponade. There were also two late sudden deaths in the group with Marfan's syndrome, which may have been the consequence of aortic rupture. No difference in immediate operative mortality following the Bentall procedure was noted between patients with and without Marfan's syndrome, but young patients without Marfan's syndrome seem to have better event-free and long-term survival. In patients with Marfan's syndrome, the presence of acute dissection makes reoperation more likely, and sudden death from rupture still occurs despite careful

  12. Morphological changes of carotid bodies in acute respiratory distress syndrome: a morphometric study in humans

    Directory of Open Access Journals (Sweden)

    Vinhaes E.N.G.

    2002-01-01

    Full Text Available Carotid bodies are chemoreceptors sensitive to a fall of partial oxygen pressure in blood (hypoxia. The morphological alterations of these organs in patients with chronic obstructive pulmonary disease (COPD and in people living at high altitude are well known. However, it is not known whether the histological profile of human carotid bodies is changed in acute clinical conditions such as acute respiratory distress syndrome (ARDS. The objective of the present study was to perform a quantitative analysis of the histology of carotid bodies collected from patients who died of ARDS. A morphometric study of carotid bodies collected during routine autopsies was carried out on three groups: patients that died of non-respiratory diseases (controls, N = 8, patients that presented COPD and died of its complications or associated diseases (N = 7, and patients that died of ARDS (N = 7. Morphometric measurements of the volume fraction of clusters of chief cells were performed in five fields on each slide at 40X magnification. The numerical proportion of the four main histological cell types (light, dark, progenitor and sustentacular cells was determined analyzing 10 fields on each slide at 400X magnification. The proportion of dark cells was 0.22 in ARDS patients, 0.12 in controls (P<0.001, and 0.08 in the COPD group. The proportion of light cells was 0.33 (ARDS, 0.44 (controls (P<0.001, and 0.36 (COPD. These findings suggest that chronic and acute hypoxia have different effects on the histology of glomic tissue.

  13. Phosphorylation of ARD1 by IKKβ contributes to its destabilization and degradation

    International Nuclear Information System (INIS)

    Kuo, Hsu-Ping; Lee, Dung-Fang; Xia, Weiya; Lai, Chien-Chen; Li, Long-Yuan; Hung, Mien-Chie

    2009-01-01

    IκB kinase β (IKKβ), a major kinase downstream of various proinflammatory signals, mediates multiple cellular functions through phosphorylation and regulation of its substrates. On the basis of protein sequence analysis, we identified arrest-defective protein 1 (ARD1), a protein involved in apoptosis and cell proliferation processes in many human cancer cells, as a new IKKβ substrate. We provided evidence showing that ARD1 is indeed a bona fide substrate of IKKβ. IKKβ physically associated with ARD1 and phosphorylated it at Ser209. Phosphorylation by IKKβ destabilized ARD1 and induced its proteasome-mediated degradation. Impaired growth suppression was observed in ARD1 phosphorylation-mimic mutant (S209E)-transfected cells as compared with ARD1 non-phosphorylatable mutant (S209A)-transfected cells. Our findings of molecular interactions between ARD1 and IKKβ may enable further understanding of the upstream regulation mechanisms of ARD1 and of the diverse functions of IKKβ.

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

    Science.gov (United States)

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

    2001-08-01

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

  15. Decreased sweating in seven patients with Laron syndrome

    DEFF Research Database (Denmark)

    Main, K M; Price, D A; Savage, M O

    1993-01-01

    patients with Laron syndrome, measured by pilocarpine iontophoresis. The patients had significantly lower SSRs than healthy children matched for sex and pubertal stage (P ...). These observations further supported the hypothesis that sweat gland function in humans is under the influence of the GH-insulin-like growth factor-I axis. It remains to be seen whether the decrease in SSR also leads to altered thermoregulation in patients with Laron syndrome....

  16. Geriatric syndromes in patients with chronic kidney disease

    OpenAIRE

    Tomasz Gołębiowski; Hanna Augustyniak-Bartosik; Wacław Weyde; Marian Klinger

    2016-01-01

    The recent epidemiologic data pointed out, that the general number of patients on hemodialysis is steadily increasing, especially in group of elderly patients over 75 years old. The geriatric syndromes are a multietiological disorder related to physiological aging and partly associated with comorbid conditions. Frailty, falls, functional decline and disability, cognitive impairment and depression are main geriatric syndromes and occurs in patients with impaired renal function more often than ...

  17. Primary headaches in restless legs syndrome patients

    Directory of Open Access Journals (Sweden)

    Ravi Gupta

    2012-01-01

    Full Text Available Earlier studies conducted among migraineurs have shown an association between migraine and restless legs syndrome (RLS. We chose RLS patients and looked for migraine to exclude sample bias. Materials and Methods: 99 consecutive subjects of idiopathic RLS were recruited from the sleep clinic during four months period. Physician diagnosis of headache and depressive disorder was made with the help of ICHD-2 and DSM-IV-TR criteria, respectively. Sleep history was gathered. Severity of RLS and insomnia was measured using IRLS (Hindi version and insomnia severity index Hindi version, respectively. Chi-square test, one way ANOVA and t-test were applied to find out the significance. Results: Primary headache was seen in 51.5% cases of RLS. Migraine was reported by 44.4% subjects and other types of ′primary headaches′ were reported by 7.1% subjects. Subjects were divided into- RLS; RLS with migraine and RLS with other headache. Females outnumbered in migraine subgroup (χ2 =16.46, P<0.001. Prevalence of depression (χ2 =3.12, P=0.21 and family history of RLS (χ2 =2.65, P=0.26 were not different among groups. Severity of RLS (P=0.22 or insomnia (P=0.43 were also similar. Conclusion: Migraine is frequently found in RLS patients in clinic based samples. Females with RLS are prone to develop migraine. Depression and severity of RLS or insomnia do not affect development of headache.

  18. [Psychiatric disturbances in five patients with MELAS syndrome].

    Science.gov (United States)

    Magner, Martin; Honzik, Tomas; Tesarova, Marketa; Dvorakova, Veronika; Hansiková, Hana; Raboch, Jiři; Zeman, Jiři

    2014-01-01

    Mitochondrial disorders of energetic metabolism (MD) represent a heterogeneous group of diseases manifesting at any age with a broad spectrum of clinical symptoms, including psychiatric disorders. The aim of the study was to characterize psychiatric symptoms and diagnoses in five patients with MELAS syndrome between the ages of 17 and 53 years. Four of MELAS patients them harbored the prevalent mitochondrial DNA (mtDNA) mutation 3243A>G, and one patient had the mtDNA mutation 12706T>C. Three patients had positive family histories for MELAS syndrome. In one patient, depression was diagnosedas the first symptom ofMELAS syndrome. Depression also preceded a stroke-like episode in one patient. Four patients had disturbed cognitive functions, confusional states occurred in three patients. One patient manifested psychotic (schizophrenia-like) symptoms. Mitochondrial disorders deserve consideration as part of the differential diagnosis, especially, if there is suspected involvement of other organ groups or positive family history of MD.

  19. Lenz microphthalmic syndrome in an Indian patient

    Directory of Open Access Journals (Sweden)

    Gupta Arvind

    2007-01-01

    Full Text Available A case of Lenz microphthalmia syndrome in a seven-month-old male child having features of unilateral anophthalmia, microcephaly, external ear and finger abnormalities, hydrocele and hypospadias is being reported. The unilateral involvement and anophthalmia is rare in Lenz syndrome. The manifestation of hydrocele in association with this syndrome has not been seen in earlier cases. This is the first documented case from India.

  20. Low tendon stiffness and abnormal ultrastructure distinguish classic Ehlers-Danlos syndrome from benign joint hypermobility syndrome in patients

    DEFF Research Database (Denmark)

    Nielsen, Rie Harboe; Couppé, Christian; Jensen, Jacob Kildevang

    2014-01-01

    There is a clinical overlap between classic Ehlers-Danlos syndrome (cEDS) and benign joint hypermobility syndrome (BJHS), with hypermobility as the main symptom. The purpose of this study was to investigate the role of type V collagen mutations and tendon pathology in these 2 syndromes. In patients...... and abnormal ultrastructure distinguish classic Ehlers-Danlos syndrome from benign joint hypermobility syndrome in patients....

  1. Myxedema coma in a patient with Down's syndrome.

    Science.gov (United States)

    Bansal, Darpan; Nanda, Ashish; Gupta, Ekta; Croker, Mary; Williams, Misty L; Bacchus, Amy; Simmons, Debra; Erbland, Marcia

    2006-11-01

    hyroid dysfunction is common in Down's syndrome, most common being hypothyroidism. Longstanding, untreated hypothyroidism can lead to myxedema coma. Here we report a patient with Down's syndrome who presented with myxedema coma. The three essential elements for the diagnosis of myxedema coma include altered mental status, defective thermoregulation and a precipitating event or illness; all of these were present in our patient. Also, very high TSH, low T3 and T4, and the rapid response to the treatment with levothyroxine confirmed the diagnosis. Patients with Down's syndrome should have regular screening for thyroid dysfunction.

  2. Moyamoya syndrome in a patient with Noonan-like syndrome with loose anagen hair.

    Science.gov (United States)

    Choi, Jin-Ho; Oh, Moon-Yeon; Yum, Mi-Sun; Lee, Beom Hee; Kim, Gu-Hwan; Yoo, Han-Wook

    2015-03-01

    Noonan-like syndrome with loose anagen hair is one of the RASopathies characterized by Noonan syndrome-like features with unique ectodermal abnormalities. This syndrome is caused by mutations in the SHOC2 gene. We encountered a patient with moyamoya syndrome associated with Noonan-like syndrome with loose anagen hair presenting with transient ischemic attacks. A 6-year-old girl was diagnosed with Noonan-like syndrome with loose anagen hair because of profound short stature and ectodermal anomalies such as sparse and easily pluckable hair. A heterozygous mutation of c.4A>G (p.S2G) in the SHOC2 gene was identified, and recombinant human growth hormone therapy was initiated at 8 years of age. At age 10, she manifested recurrent left hemiplegia. Moreover, cerebrovascular imaging revealed occlusion or narrowing of both internal carotid arteries and both middle cerebral arteries with distal moyamoya-like vessels. She is treated with aspirin and calcium channel blocker. We describe the first case of Noonan-like syndrome with loose anagen hair associated with moyamoya syndrome, although it has been reported to be associated with a few cases of other RASopathies, including Noonan, cardiofaciocutaneous, and Costello syndromes. This report emphasizes the associations between cerebrovascular anomalies and Noonan-like syndrome with loose anagen hair. Copyright © 2015 Elsevier Inc. All rights reserved.

  3. SICK SINUS SYNDROME IN PATIENTS WITH ACUTE CEREBROVASCULAR ACCIDENTS

    Directory of Open Access Journals (Sweden)

    E. K. Kazakova

    2015-01-01

    Full Text Available The article presents a clinical case of 2 patients with heart arrhythmias of the sick sinus syndrome type, who were implanted electriccardiac pacemakers in the acute period of cerebrovascular accidents. There were no cardiac complaints in the clinical manifestation, however, a comprehensive assessment confirmed the diagnosis of sick sinus syndrome.

  4. Progression of salivary gland dysfunction in patients with Sjogren's syndrome

    NARCIS (Netherlands)

    Pijpe, J.; Kalk, W. W. I.; Bootsma, H.; Spijkervet, F. K. L.; Kallenberg, C. G. M.; Vissink, A.

    Background: Salivary gland dysfunction is one of the key manifestations of Sjogren's syndrome. Objectives: (1) To assess prospectively loss of function of individual salivary glands in patients with primary and secondary Sjogren's syndrome in relation to disease duration and use of immunomodulatory

  5. Metabolic Syndrome among Type-2 Diabetic Patients in Benghazi ...

    African Journals Online (AJOL)

    Background: Metabolic syndrome is a cluster of three out of five conditions that are due to hyperinsulinemia: abdominal obesity, atherogenic dyslipidemia (high triglycerides and/or low HDL), elevated blood pressure, and elevated plasma glucose. The syndrome is highly prevalent in patients with type-2 diabetes mellitus ...

  6. Rivaroxaban in patients with a recent acute coronary syndrome

    DEFF Research Database (Denmark)

    Mega, Jessica L; Braunwald, Eugene; Wiviott, Stephen D

    2012-01-01

    Acute coronary syndromes arise from coronary atherosclerosis with superimposed thrombosis. Since factor Xa plays a central role in thrombosis, the inhibition of factor Xa with low-dose rivaroxaban might improve cardiovascular outcomes in patients with a recent acute coronary syndrome....

  7. Electrodiagnostic findings in a patient with Waardenburg syndrome.

    Science.gov (United States)

    Ahmed, Aiesha; Simmons, Zachary

    2009-09-01

    Waardenburg syndrome is associated with a variety of clinical features, which may include polyneuropathy. We describe a patient with Waardenburg syndrome type 2 and provide detailed electrodiagnostic findings, thus complementing the biopsy data and brief descriptions of nerve conduction studies found in the literature.

  8. Celiac disease in patients with Williams-Beuren syndrome.

    Science.gov (United States)

    Mıhçı, Ercan; Nur, Banu Güzel; Berker-Karaüzüm, Sibel; Yılmaz, Aygen; Artan, Reha

    2015-01-01

    Celiac disease is an autoimmune, gastrointestinal disorder characterized by intolerance to the dietary grain protein gluten. An increased prevalence of celiac disease has been reported in Down syndrome and Turner syndrome, but there has been only few previous reports with respect to the association of celiac disease in Williams-Beuren syndrome. The aim of this study was to evaluate the frequency of celiac disease in our 24 Williams-Beuren syndrome patients. Gastrointestinal problems and celiac disease symptoms of patients were noted. All patients were analyzed by the titer of tissue transglutaminases IgA and IgG. HLA genotyping and intestinal biopsy was performed to the patients with positive serology. We also performed gluten free diet in the presence of compatible symptoms, serology, HLA genotyping and intestinal biopsy. In our study, two patients had positive tTG antibodies, but only one had positive biopsy finding for celiac disease. The frequency of celiac disease in patients with Williams-Beuren syndrome was estimated as 1/24 (4.1%). Though the number of participants in this study was limited, the results show that the frequency of celiac disease is higher in Williams-Beuren syndrome compared to the general population. We suggest that a high suspicion and testing for celiac disease should be recommended at certain intervals in all cases with Williams-Beuren syndrome to detect the cause of growth retardation and gastrointestinal problems.

  9. Aspirin reduces lipopolysaccharide-induced pulmonary inflammation in human models of ARDS.

    Science.gov (United States)

    Hamid, U; Krasnodembskaya, A; Fitzgerald, M; Shyamsundar, M; Kissenpfennig, A; Scott, C; Lefrancais, E; Looney, M R; Verghis, R; Scott, J; Simpson, A J; McNamee, J; McAuley, D F; O'Kane, C M

    2017-11-01

    Platelets play an active role in the pathogenesis of acute respiratory distress syndrome (ARDS). Animal and observational studies have shown aspirin's antiplatelet and immunomodulatory effects may be beneficial in ARDS. To test the hypothesis that aspirin reduces inflammation in clinically relevant human models that recapitulate pathophysiological mechanisms implicated in the development of ARDS. Healthy volunteers were randomised to receive placebo or aspirin 75  or 1200 mg (1:1:1) for seven days prior to lipopolysaccharide (LPS) inhalation, in a double-blind, placebo-controlled, allocation-concealed study. Bronchoalveolar lavage (BAL) was performed 6 hours after inhaling 50 µg of LPS. The primary outcome measure was BAL IL-8. Secondary outcome measures included markers of alveolar inflammation (BAL neutrophils, cytokines, neutrophil proteases), alveolar epithelial cell injury, systemic inflammation (neutrophils and plasma C-reactive protein (CRP)) and platelet activation (thromboxane B2, TXB2). Human lungs, perfused and ventilated ex vivo (EVLP) were randomised to placebo or 24 mg aspirin and injured with LPS. BAL was carried out 4 hours later. Inflammation was assessed by BAL differential cell counts and histological changes. In the healthy volunteer (n=33) model, data for the aspirin groups were combined. Aspirin did not reduce BAL IL-8. However, aspirin reduced pulmonary neutrophilia and tissue damaging neutrophil proteases (Matrix Metalloproteinase (MMP)-8/-9), reduced BAL concentrations of tumour necrosis factor α and reduced systemic and pulmonary TXB2. There was no difference between high-dose and low-dose aspirin. In the EVLP model, aspirin reduced BAL neutrophilia and alveolar injury as measured by histological damage. These are the first prospective human data indicating that aspirin inhibits pulmonary neutrophilic inflammation, at both low and high doses. Further clinical studies are indicated to assess the role of aspirin in the

  10. [metabonomics research on coronary heart disease patients of phlegm turbidity syndrome and qi deficiency syndrome].

    Science.gov (United States)

    Cheng, Peng; Chen, Ze-qi; Wang, Dong-sheng

    2015-02-01

    To study the correlation between Chinese medical types of coronary heart disease (CHD) [i.e., phlegm turbidity syndrome (PTS) and qi deficiency syndrome (QDS)] and their metabolites. Recruited were 65 CHD patients including 37 cases of PTS and 28 cases of QDS. Serum endogenous metabolites in the two syndrome types were determined by gas chromatograph-mass spectrometer-computer (GC/MS), and their differences between their metabolic profiles analyzed. More than 100 chromatographic peaks were totally scanned. Chromatograms obtained was matched with mass spectrum bank, and finally we got the category contribution value of 46 kinds of substances. Results of MCTree analysis showed patients of PTS and patients of QDS could be effectively distinguished. Compounds contributing to identify the two syndromes were sequenced as serine, valine, 2 hydroxy propionic acid. Comparison of metabolites showed contents of serine and 2 hydroxy propionic acid were higher in patients of PTS than in patients of QDS (Pmetabonomics of CHD TCM syndrome types could provide material bases for TCM syndrome differentiation of CHD, indicating that metabonomics technologies might become a new research method for TCM syndrome typing.

  11. Epilepsy in fragile-X-syndrome mimicking panayiotopoulos syndrome: Description of three patients.

    Science.gov (United States)

    Bonanni, Paolo; Casellato, Susanna; Fabbro, Franco; Negrin, Susanna

    2017-10-01

    Fragile-X-syndrome is the most common cause of inherited intellectual disability. Epilepsy is reported to occur in 10-20% of individuals with Fragile-X-syndrome. A frequent seizure/electroencephalogram (EEG) pattern resembles that of benign rolandic epilepsy. We describe the clinical features, EEG findings and evolution in three patients affected by Fragile-X-syndrome and epilepsy mimicking Panayiotopoulos syndrome. Age at seizure onset was between 4 and about 7 years. Seizures pattern comprised a constellation of autonomic symptoms with unilateral deviation of the eyes and ictal syncope. Duration of the seizures could be brief or lengthy. Interictal EEGs revealed functional multifocal abnormalities. The evolution was benign in all patients with seizures remission before the age of 14. This observation expands the spectrum of benign epileptic phenotypes present in Fragile-X-syndrome and may be quite helpful in guiding anticonvulsant management and counseling families as to expectations regarding seizure remission. © 2017 Wiley Periodicals, Inc.

  12. Study of Winter Wheat Yield Quality Analysis at ARDS Turda

    Directory of Open Access Journals (Sweden)

    Ovidiu Adrian Ceclan

    2016-11-01

    Full Text Available The purpose of this research is to study the potential for yield and quality indicators for winter wheat genotypes in terms of pedological and climate condition and applied technology, at ARDS Turda during 2014 – 2015. Depending on the climatic conditions that are associated with applied technology is a decisive factor in successful wheat crop for all genotypes that were studied at Ards Turda during the 2014 – 2016. That’s wy each genotype responded differently to the conditions of the ARDS Turda also through the two levels of fertilisations applied in the winter with fertilizers 20:20:0, 250 kg/ha assuring 50 kg/ha N and P active substance and second level of fertilisations with 150 kg/ha ammonium nitrate assuring 50 kg/ha N active substance. All genotype that were studied in terms of yield and quality indicators were influenced by the fertilization level. The influence of pedo-climatic conditions, applied technologies and fertilizers level at ARDS Turda showed that all genotypes with small yield had higher protein and gluten content respectively Zeleny index.

  13. Cotard syndrome in neurological and psychiatric patients.

    Science.gov (United States)

    Ramirez-Bermudez, Jesus; Aguilar-Venegas, Luis C; Crail-Melendez, Daniel; Espinola-Nadurille, Mariana; Nente, Francisco; Mendez, Mario F

    2010-01-01

    The authors describe the frequency and characteristics of Cotard syndrome among neurological and psychiatric inpatients at a tertiary referral center. All inpatients from the National Institute of Neurology of Mexico (March 2007-May 2009) requiring neuropsychiatric consultation were reviewed. Among 1,321 inpatient consultations, 63.7% had neurological disease and one (0.11%) had viral encephalitis and Cotard syndrome. Of inpatients, 36.2% had pure psychiatric disorders and three (0.62%) had Cotard syndrome, associated with psychotic depression, depersonalization, and penile retraction (koro syndrome). This review discusses potential mechanisms for Cotard syndrome, including the role of a perceptual-emotional dissociation in self-misattribution in the deliré des negations.

  14. Extracorporeal carbon dioxide removal for patients with acute respiratory failure secondary to the acute respiratory distress syndrome: a systematic review

    Science.gov (United States)

    2014-01-01

    Acute respiratory distress syndrome (ARDS) continues to have significant mortality and morbidity. The only intervention proven to reduce mortality is the use of lung-protective mechanical ventilation strategies, although such a strategy may lead to problematic hypercapnia. Extracorporeal carbon dioxide removal (ECCO2R) devices allow uncoupling of ventilation from oxygenation, thereby removing carbon dioxide and facilitating lower tidal volume ventilation. We performed a systematic review to assess efficacy, complication rates, and utility of ECCO2R devices. We included randomised controlled trials (RCTs), case–control studies and case series with 10 or more patients. We searched MEDLINE, Embase, LILACS (Literatura Latino Americana em Ciências da Saúde), and ISI Web of Science, in addition to grey literature and clinical trials registries. Data were independently extracted by two reviewers against predefined criteria and agreement was reached by consensus. Outcomes of interest included mortality, intensive care and hospital lengths of stay, respiratory parameters and complications. The review included 14 studies with 495 patients (two RCTs and 12 observational studies). Arteriovenous ECCO2R was used in seven studies, and venovenous ECCO2R in seven studies. Available evidence suggests no mortality benefit to ECCO2R, although post hoc analysis of data from the most recent RCT showed an improvement in ventilator-free days in more severe ARDS. Organ failure-free days or ICU stay have not been shown to decrease with ECCO2R. Carbon dioxide removal was widely demonstrated as feasible, facilitating the use of lower tidal volume ventilation. Complication rates varied greatly across the included studies, representing technological advances. There was a general paucity of high-quality data and significant variation in both practice and technology used among studies, which confounded analysis. ECCO2R is a rapidly evolving technology and is an efficacious treatment to enable

  15. Cloning, Characterization, and Expression Analysis of the Novel Acetyltransferase Retrogene Ard1b in the Mouse1

    Science.gov (United States)

    Pang, Alan Lap-Yin; Peacock, Stephanie; Johnson, Warren; Bear, Deborah H.; Rennert, Owen M.; Chan, Wai-Yee

    2009-01-01

    N-alpha-terminal acetylation is a modification process that occurs cotranslationally on most eukaryotic proteins. The major enzyme responsible for this process, N-alpha-terminal acetyltransferase, is composed of the catalytic subunit ARD1A and the auxiliary subunit NAT1. We cloned, characterized, and studied the expression pattern of Ard1b (also known as Ard2), a novel homolog of the mouse Ard1a. Comparison of the genomic structures suggests that the autosomal Ard1b is a retroposed copy of the X-linked Ard1a. Expression analyses demonstrated a testis predominance of Ard1b. A reciprocal expression pattern between Ard1a and Ard1b is also observed during spermatogenesis, suggesting that Ard1b is expressed to compensate for the loss of Ard1a starting from meiosis. Both ARD1A and ARD1B can interact with NAT1 to constitute a functional N-alpha-terminal acetyltransferase in vitro. The expression of ARD1B protein can be detected in mouse testes but is delayed until the first appearance of round spermatids. In a cell culture model, the inclusion of the long 3′ untranslated region of Ard1b leads to reduction of luciferase reporter activity, which implicates its role in translational repression of Ard1b during spermatogenesis. Our results suggest that ARD1B may have an important role in the later course of the spermatogenic process. PMID:19246321

  16. Altered gastric emptying in patients with irritable bowel syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Caballero-Plasencia, A.M.; Valenzuela-Barranco, M. [Department of Medicine, School of Medicine, University of Granada (Spain); Herrerias-Gutierrez, J.M. [Division of Gastroenterology, University Hospital ``Virgen de la Macarena``, Sevilla (Spain); Esteban-Carretero, J.M. [Central Service of Investigation in Health Sciences, University of Cadiz, Cadiz (Spain)

    1999-04-29

    Irritable bowel syndrome is the most frequent functional disorder of the digestive system. Patients with irritable bowel syndrome have motor disorders not only in the colon, but also in other parts of the digestive tract such as the oesophagus and small intestine; however, it is not known whether the stomach is also involved. We used a radiolabelled mixed solid-liquid meal (technetium-99m for the solid component, indium-111 for the liquid component) to study gastric emptying of solids (GES), liquids (GEL) and indigestible solids (GER) in 50 patients diagnosed as having irritable bowel syndrome (30 with predominant constipation and 20 with predominant diarrhoea). GER was measured by counting the number of indigestible solids remaining in the stomach 4 h after they were swallowed. In patients with irritable bowel syndrome, GES and GEL were slower than in control subjects (P<0.05). GER was normal in all patients except for two women. Thirty-two patients (64%) showed delayed GES, 29 (58%) delayed GEL, and 2 (4%) delayed GER. Among patients with irritable bowel syndrome, GES was slower in those with predominant constipation than in those with predominant diarrhoea (P<0.05); GEL and GER were similar in both groups. Gastroparesis was found in a large proportion of patients with irritable bowel syndrome, suggesting the presence of a more generalised motor disorder of the gut. (orig.) With 1 fig., 3 tabs., 48 refs.

  17. Altered gastric emptying in patients with irritable bowel syndrome

    International Nuclear Information System (INIS)

    Caballero-Plasencia, A.M.; Valenzuela-Barranco, M.; Herrerias-Gutierrez, J.M.; Esteban-Carretero, J.M.

    1999-01-01

    Irritable bowel syndrome is the most frequent functional disorder of the digestive system. Patients with irritable bowel syndrome have motor disorders not only in the colon, but also in other parts of the digestive tract such as the oesophagus and small intestine; however, it is not known whether the stomach is also involved. We used a radiolabelled mixed solid-liquid meal (technetium-99m for the solid component, indium-111 for the liquid component) to study gastric emptying of solids (GES), liquids (GEL) and indigestible solids (GER) in 50 patients diagnosed as having irritable bowel syndrome (30 with predominant constipation and 20 with predominant diarrhoea). GER was measured by counting the number of indigestible solids remaining in the stomach 4 h after they were swallowed. In patients with irritable bowel syndrome, GES and GEL were slower than in control subjects (P<0.05). GER was normal in all patients except for two women. Thirty-two patients (64%) showed delayed GES, 29 (58%) delayed GEL, and 2 (4%) delayed GER. Among patients with irritable bowel syndrome, GES was slower in those with predominant constipation than in those with predominant diarrhoea (P<0.05); GEL and GER were similar in both groups. Gastroparesis was found in a large proportion of patients with irritable bowel syndrome, suggesting the presence of a more generalised motor disorder of the gut. (orig.)

  18. The clinical usefulness of extravascular lung water and pulmonary vascular permeability index to diagnose and characterize pulmonary edema: a prospective multicenter study on the quantitative differential diagnostic definition for acute lung injury/acute respiratory distress syndrome

    Science.gov (United States)

    2012-01-01

    Introduction Acute lung injury (ALI)/acute respiratory distress syndrome (ARDS) is characterized by features other than increased pulmonary vascular permeability. Pulmonary vascular permeability combined with increased extravascular lung water content has been considered a quantitative diagnostic criterion of ALI/ARDS. This prospective, multi-institutional, observational study aimed to clarify the clinical pathophysiological features of ALI/ARDS and establish its quantitative diagnostic criteria. Methods The extravascular lung water index (EVLWI) and the pulmonary vascular permeability index (PVPI) were measured using the transpulmonary thermodilution method in 266 patients with PaO2/FiO2 ratio ≤ 300 mmHg and bilateral infiltration on chest radiography, in 23 ICUs of academic tertiary referral hospitals. Pulmonary edema was defined as EVLWI ≥ 10 ml/kg. Three experts retrospectively determined the pathophysiological features of respiratory insufficiency by considering the patients' history, clinical presentation, chest computed tomography and radiography, echocardiography, EVLWI and brain natriuretic peptide level, and the time course of all preceding findings under systemic and respiratory therapy. Results Patients were divided into the following three categories on the basis of the pathophysiological diagnostic differentiation of respiratory insufficiency: ALI/ARDS, cardiogenic edema, and pleural effusion with atelectasis, which were noted in 207 patients, 26 patients, and 33 patients, respectively. EVLWI was greater in ALI/ARDS and cardiogenic edema patients than in patients with pleural effusion with atelectasis (18.5 ± 6.8, 14.4 ± 4.0, and 8.3 ± 2.1, respectively; P edema or pleural effusion with atelectasis patients (3.2 ± 1.4, 2.0 ± 0.8, and 1.6 ± 0.5; P edema patients. A PVPI value of 2.6 to 2.85 provided a definitive diagnosis of ALI/ARDS (specificity, 0.90 to 0.95), and a value < 1.7 ruled out an ALI/ARDS diagnosis (specificity, 0.95). Conclusion

  19. Overcoming barriers to work participation for patients with postpoliomyelitis syndrome

    NARCIS (Netherlands)

    ten Katen, Kim; Beelen, Anita; Nollet, Frans; Frings-Dresen, Monique H. W.; Sluiter, Judith K.

    2011-01-01

    Purpose. This study aimed to explore the perceived work ability of patients with postpoliomyelitis syndrome (PPS), to identify barriers and factors that are impeding or conducive, respectively, to work participation, and to identify possible interventions. Methods. Qualitative cross-sectional

  20. Meier-Gorlin syndrome: Report of an additional patient with ...

    African Journals Online (AJOL)

    Rabah M. Shawky

    2014-05-16

    May 16, 2014 ... which functions during replication can cause Seckel syndrome, a clinically related ... brain, and growth hormone provocation test were normal. Skeletal survey ... atrial defect with left–right shunt [17]. Also our patient suf-.

  1. Diagnostic value of static and dynamic scintigraphy in diagnosis of acute respiratory distress syndrome in the course of sepsis. Part 2. Lung aerosol scintigraphy

    International Nuclear Information System (INIS)

    Jurgilewicz, D.; Rogowski, F.; Malinowska, L.

    1997-01-01

    Rapid increase in permeability of functionally and/or structurally damaged alveolar-capillary barrier is the pivotal factor in non-cardiogenic pulmonary edema development. Rate of exudative changes progress in the lungs decides about prognosis in patients with this serious, over 50% death rate, syndrome. The aim of the study was to estimate the usefulness of dynamic lung scintigraphy in diagnosis of gas exchange abnormalities in sepsis and ARDS. Aerosol scintigraphy with pulmonary clearance of DTPA in 6 septic and in 6 ARDS patients were performed. Studies were done using planar gamma camera, type MB9200, in A-P projection. They were analyzed with the modified NMS Warsaw Polytechnic computer program. Aerosol scintiscans demonstrated very irregular borderlines of both lungs, features of marked impairment in ventilation (with foci of increased radioactivity in the view of main bronchi) and about 2.5-fold faster pulmonary clearance of DTPA in ARDS patients comparing to sepsis and control groups. The results showed that dynamic scintigraphy can be safely performed in patients in critical condition. Changes in scintiscans and rapid pulmonary clearance of DTPA might be an early indicator of ARDS development in septic patients. (author)

  2. [Cardiofaciocutaneous syndrome, a Noonan syndrome related disorder: clinical and molecular findings in 11 patients].

    Science.gov (United States)

    Carcavilla, Atilano; García-Miñaúr, Sixto; Pérez-Aytés, Antonio; Vendrell, Teresa; Pinto, Isabel; Guillén-Navarro, Encarna; González-Meneses, Antonio; Aoki, Yoko; Grinberg, Daniel; Ezquieta, Begoña

    2015-01-20

    To describe 11 patients with cardiofaciocutaneous syndrome (CFC) and compare them with 130 patients with other RAS-MAPK syndromes (111 Noonan syndrome patients [NS] and 19 patients with LEOPARD syndrome). Clinical data from patients submitted for genetic analysis were collected. Bidirectional sequencing analysis of PTPN11, SOS1, RAF1, BRAF, and MAP2K1 focused on exons carrying recurrent mutations, and of all KRAS exons were performed. Six different mutations in BRAF were identified in 9 patients, as well as 2 MAP2K1 mutations. Short stature, developmental delay, language difficulties and ectodermal anomalies were more frequent in CFC patients when compared with other neuro-cardio-faciocutaneous syndromes (P<.05). In at least 2 cases molecular testing helped reconsider the diagnosis. CFC patients showed a rather severe phenotype but at least one patient with BRAF mutation showed no developmental delay, which illustrates the variability of the phenotypic spectrum caused by BRAF mutations. Molecular genetic testing is a valuable tool for differential diagnosis of CFC and NS related disorders. Copyright © 2014 Elsevier España, S.L.U. All rights reserved.

  3. Gilles de la Tourette's syndrome in a patient with 47(XXX) syndrome: a case report.

    Science.gov (United States)

    Chiappedi, Matteo; de Vincenzi, Silvia; Dolci, Roberta; De Luca, Sara; Bejor, Maurizio

    2011-11-05

    To the best of our knowledge, this is the first report of a comorbidity between Gilles de la Tourette's syndrome and 47 (XXX) syndrome. The clinical picture of Gilles de la Tourette's Syndrome is well described, while 47 (XXX) syndrome is much more rare and has a broader spectrum of possible phenotypic presentations. An Italian Caucasian girl was referred at the age of 11 to our Rehabilitation Center for anxiety and learning difficulties. The girl had already been diagnosed as having 47(XXX) syndrome; she had some rather typical features of the chromosomal abnormality, but she also showed a high level of anxiety and the presence of motor and vocal tics. When an accurate history was taken, a diagnosis of Gilles de la Tourette's Syndrome emerged. The possible interaction between peculiar features of these two syndromes in terms of neuropsychological and affective functioning is both interesting for the specific case and to hypothesize models of rehabilitation for patients with one or both syndromes. Executive functions are specifically reduced in both syndromes, therefore it might be hard to discriminate the contribution of each one to the general impairment; the same applies to anxiety. Moreover, mental retardation (with a significantly lower verbal cognitive functioning) poses relevant problems when suggesting cognitive behavioral or psychoeducational rehabilitative approaches.

  4. Gilles de la Tourette's syndrome in a patient with 47(XXX syndrome: a case report

    Directory of Open Access Journals (Sweden)

    Chiappedi Matteo

    2011-11-01

    Full Text Available Abstract Introduction To the best of our knowledge, this is the first report of a comorbidity between Gilles de la Tourette's syndrome and 47 (XXX syndrome. The clinical picture of Gilles de la Tourette's Syndrome is well described, while 47 (XXX syndrome is much more rare and has a broader spectrum of possible phenotypic presentations. Case presentation An Italian Caucasian girl was referred at the age of 11 to our Rehabilitation Center for anxiety and learning difficulties. The girl had already been diagnosed as having 47(XXX syndrome; she had some rather typical features of the chromosomal abnormality, but she also showed a high level of anxiety and the presence of motor and vocal tics. When an accurate history was taken, a diagnosis of Gilles de la Tourette's Syndrome emerged. Conclusions The possible interaction between peculiar features of these two syndromes in terms of neuropsychological and affective functioning is both interesting for the specific case and to hypothesize models of rehabilitation for patients with one or both syndromes. Executive functions are specifically reduced in both syndromes, therefore it might be hard to discriminate the contribution of each one to the general impairment; the same applies to anxiety. Moreover, mental retardation (with a significantly lower verbal cognitive functioning poses relevant problems when suggesting cognitive behavioral or psychoeducational rehabilitative approaches.

  5. Acute Respiratory Distress Syndrome: Challenge for Diagnosis and Therapy

    Directory of Open Access Journals (Sweden)

    Chun Pan

    2018-01-01

    Conclusions: ARDS is a devastating clinical syndrome whose incidence and mortality has remained high over the past 50 years. Its definition and treatments are still confronted with challenges, and early recognition and intervention are crucial for improving the outcomes of ARDS. More clinical studies are needed to improve early diagnosis and appropriate therapy.

  6. Digital subtraction angiography in patients with Marfan's syndrome

    International Nuclear Information System (INIS)

    Rauber, K.; Riemann, H.

    1987-01-01

    Marfan's syndrome is a rare inborn error of metabolism. Marfan patients are prone to aneurysms of the ascending aorta and run a high risk of rupture of the aortic arch. The diameter of the aneurysm is the most important predictor of the risk and therefore the leading point for surgical interventions. IV and IA-DSA according to our experiences are simple and effective methods in pre- and postoperative evaluation of patients with the syndrome. (orig.) [de

  7. Digital subtraction angiography in patients with Marfan's syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Rauber, K; Riemann, H

    1987-06-01

    Marfan's syndrome is a rare inborn error of metabolism. Marfan patients are prone to aneurysms of the ascending aorta and run a high risk of rupture of the aortic arch. The diameter of the aneurysm is the most important predictor of the risk and therefore the leading point for surgical interventions. IV and IA-DSA according to our experiences are simple and effective methods in pre- and postoperative evaluation of patients with the syndrome.

  8. Frequency of Metabolic Syndrome and Its Components in Patients with Carpal Tunnel Syndrome

    International Nuclear Information System (INIS)

    Iftikhar, S.; Javed, M. A.; Kasuri, M. N.

    2016-01-01

    Objective: To determine the frequency of metabolic syndrome and its components in patients with carpal tunnel syndrome. Study Design: Case-series. Place and Duration of Study: Department of Neurology, Mayo Hospital, Lahore, from January to June 2012. Methodology: Seventy-five (64 females and 11 males) patients with clinically diagnosed and electrodiagnostically confirmed carpal tunnel syndrome were inducted. Their waist circumference, blood pressure, fasting blood glucose, fasting triglycerides and high density lipoprotein cholesterol levels were recorded. Patients were categorized having metabolic syndrome according to Adult Treatment Panel III criteria, if any 3 were present out of hypertension, elevated fasting triglycerides, reduced high density lipoprotein cholesterol, elevated fasting blood glucose, and elevated waist circumference. Result: Mean age of the patients was 42.04±9.31 years, mean waist circumference was 95.32±9.03 cm, mean systolic blood pressure was 134.13±13.72 mmHg, mean diastolic blood pressure was 89.13±8.83 mmHg, mean fasting blood glucose was 94.35±21.81 mg/dl, mean fasting triglycerides was 177.48±48.69 mg/dl, and mean high density lipoprotein cholesterol was 41.95±11.17 mg/dl. Metabolic syndrome was found in 54 (72 percentage) patients including 9 (16.7 percentage) males and 45 (83.3 percentage) females. Out of 75 patients, 54 (72 percentage) had elevated waist circumference, 52 (69.3 percentage) had elevated blood pressure, 19 (25.3 percentage) had elevated fasting blood glucose, 53 (70.6 percentage) had elevated fasting triglycerides and 54 (72 percentage) had reduced high density lipoprotein cholesterol. Highest frequency of metabolic syndrome was found in age range of 40 - 49 years in both genders. Conclusion: Metabolic syndrome is frequently found in the patients with carpal tunnel syndrome. (author)

  9. Brown-McLean Syndrome in a Pediatric Patient

    Science.gov (United States)

    Tourkmani, Abdo Karim; Martinez, Jaime D.; Berrones, David; Juárez-Domínguez, Brenda Y.; Beltrán, Francisco; Galor, Anat

    2015-01-01

    The purpose of this manuscript is to report the case of a 12-year-old patient who presented for routine ophthalmic examination after congenital cataract surgery performed at 2 months of age. The patient was diagnosed with bilateral Brown-McLean syndrome by slit lamp examination. No treatment was required because the patient was asymptomatic and had a clear central cornea. This is the first described case of Brown-McLean syndrome in a pediatric patient, representing the importance of clinical examination in the pediatric age group after cataract surgery because of the risk for patients of developing peripheral edema. PMID:26034485

  10. Combination of Extracorporeal Life Support and Mesenchymal Stem Cell Therapy for Treatment of ARDS in Combat Casualties and Evacuation of Service Members with ARDS

    Science.gov (United States)

    2017-10-01

    AWARD NUMBER: W81XWH-15-2-0072 TITLE: Combination of Extracorporeal Life Support and Mesenchymal Stem Cell Therapy for Treatment of ARDS in...Mesenchymal Stem Cell Therapy for Treatment of ARDS in Combat Casualties and Evacuation of Service Members with ARDS 5b. GRANT NUMBER W81XWH-15...Figure 4. Mitochondrial activity is mostly preserved on the animals that were supported with ventilator devices and mesenchymal stem cells . Using a

  11. Prevention of infectious diseases in patients with Good syndrome.

    Science.gov (United States)

    Multani, Ashrit; Gomez, Carlos A; Montoya, José G

    2018-08-01

    Good syndrome is a profoundly immunocompromising condition with heterogeneous immune deficits characterized by the presence of thymoma, low-to-absent B-lymphocyte counts, hypogammaglobulinemia, and impaired cell-mediated immunity. Opportunistic infectious diseases associated with Good syndrome represent a diagnostic and therapeutic challenge, given their protean clinical manifestations. Although these infectious complications have been reviewed in prior publications, recommendations regarding their prevention have been lacking. Good syndrome usually occurs in adult patients between the ages of 40 and 70 years. Immunologically, it is characterized by low or absent peripheral blood B lymphocytes, hypogammaglobulinemia, and variable defects in cell-mediated immunity including low CD4 T counts, inverted CD4:CD8 T-lymphocyte ratio, and reduced T-lymphocyte mitogen proliferative responses. Patients with Good syndrome are susceptible to a variety of infectious diseases, of which the most common are recurrent bacterial sinopulmonary infections, mucocutaneous candidiasis, and CMV tissue-invasive disease. Preventive guidelines including targeted antimicrobial prophylaxis and vaccination strategies can mitigate infectious complications in patients with Good syndrome. Immunological deficits and infectious complications in Good syndrome have been described for over 60 years. Further research is needed to elucidate its exact pathogenesis and define the mechanistic relationship between thymoma and hypogammaglobulinemia. However, tailored prophylactic strategies can be recommended for patients with Good syndrome.

  12. PMS2 involvement in patients suspected of Lynch syndrome.

    Science.gov (United States)

    Niessen, Renée C; Kleibeuker, Jan H; Westers, Helga; Jager, Paul O J; Rozeveld, Dennie; Bos, Krista K; Boersma-van Ek, Wytske; Hollema, Harry; Sijmons, Rolf H; Hofstra, Robert M W

    2009-04-01

    It is well-established that germline mutations in the mismatch repair genes MLH1, MSH2, and MSH6 cause Lynch syndrome. However, mutations in these three genes do not account for all Lynch syndrome (suspected) families. Recently, it was shown that germline mutations in another mismatch repair gene, PMS2, play a far more important role in Lynch syndrome than initially thought. To explore this further, we determined the prevalence of pathogenic germline PMS2 mutations in a series of Lynch syndrome-suspected patients. Ninety-seven patients who had early-onset microsatellite instable colorectal or endometrial cancer, or multiple Lynch syndrome-associated tumors and/or were from an Amsterdam Criteria II-positive family were selected for this study. These patients carried no pathogenic germline mutation in MLH1, MSH2, or MSH6. When available, tumors were investigated for immunohistochemical staining (IHC) for PMS2. PMS2 was screened in all patients by exon-by-exon sequencing. We identified four patients with a pathogenic PMS2 mutation (4%) among the 97 patients we selected. IHC of PMS2 was informative in one of the mutation carriers, and in this case, the tumor showed loss of PMS2 expression. In conclusion, our study confirms the finding of previous studies that PMS2 is more frequently involved in Lynch syndrome than originally expected.

  13. ATP catabolism by tissue nonspecific alkaline phosphatase contributes to development of ARDS in influenza-infected mice.

    Science.gov (United States)

    Woods, Parker S; Doolittle, Lauren M; Hickman-Davis, Judy M; Davis, Ian C

    2018-01-01

    Influenza A viruses are highly contagious respiratory pathogens that are responsible for significant morbidity and mortality worldwide on an annual basis. We have shown previously that influenza infection of mice leads to increased ATP and adenosine accumulation in the airway lumen. Moreover, we demonstrated that A 1 -adenosine receptor activation contributes significantly to influenza-induced acute respiratory distress syndrome (ARDS). However, we found that development of ARDS in influenza-infected mice does not require catabolism of ATP to adenosine by ecto-5'-nucleotidase (CD73). Hence, we hypothesized that increased adenosine generation in response to infection is mediated by tissue nonspecific alkaline phosphatase (TNAP), which is a low-affinity, high-capacity enzyme that catabolizes nucleotides in a nonspecific manner. In the current study, we found that whole lung and BALF TNAP expression and alkaline phosphatase enzymatic activity increased as early as 2 days postinfection (dpi) of C57BL/6 mice with 10,000 pfu/mouse of influenza A/WSN/33 (H1N1). Treatment at 2 and 4 dpi with a highly specific quinolinyl-benzenesulfonamide TNAP inhibitor (TNAPi) significantly reduced whole lung alkaline phosphatase activity at 6 dpi but did not alter TNAP gene or protein expression. TNAPi treatment attenuated hypoxemia, lung dysfunction, histopathology, and pulmonary edema at 6 dpi without impacting viral replication or BALF adenosine. Treatment also improved epithelial barrier function and attenuated cellular and humoral immune responses to influenza infection. These data indicate that TNAP inhibition can attenuate influenza-induced ARDS by reducing inflammation and fluid accumulation within the lung. They also further emphasize the importance of adenosine generation for development of ARDS in influenza-infected mice.

  14. Abort Region Determinator (ARD) module feasibility report. Mission planning, mission analysis and software formulation

    Science.gov (United States)

    Draeger, B. G.; Joyner, J. A.

    1976-01-01

    A detailed performance evaluation of the Abort Region Determinator (ARD) module design was provided in support of OFT-1 ascent and OFT-1 intact launch aborts. The evaluation method used compared ARD results against results obtained using the full-up Space Vehicle Dynamic Simulations program under the same conditions. Results were presented for each of the three major ARD math models: (1) the ascent numerical integrator; (2) the mass model, and (3) the second stage predictor as well as the total ARD module. These results demonstrate that the baselined ARD module meets all design objectives for mission control center orbital flight test launch/abort support.

  15. Epidemiological and clinical characteristics of Reiter's syndrome in Jordanian patients

    International Nuclear Information System (INIS)

    Al-Mrayat, Z.; Abdallat, S.; Marabha, T.

    2004-01-01

    Objective: To study the epidemiological and clinical features of Reiter's syndrome in patients who visited the rheumatology clinic in King Hussein Medical Centre (KHMC), Jordan. Methods: A prospective study, including 43 patients with the diagnosis of Reiter's syndrome was done. Patients were assessed by taking complete history, physical examination and appropriate investigations including urinalysis and culture, stool examination and culture, synovial fluid analysis, complement fixation test for Chlamydia trachomatis, complete blood count, erythrocyte sedimentation rate, rheumatoid factor, antinuclear antibodies. HLA-B27 and radiological study. Results: All patients in this study were white men, with mean age of 26.3 years. HLA-B27 was positive in 37 patients (86%). Most cases were post venereal (32 patients, 74%) while the rest were dysenteric. The clinical manifestations were arthritis in all patients (100%), urethritis in 21 patients (48%), ocular involvement in 20 patients (46%), diarrhea in 12 patients (28%), painless oral ulcers in 11 patients (26%), skin lesions in 5 patients (12%) and constitutional symptoms in 7 patients (16%). Arthritis was mostly oligoarticular (25 patients, 58%) with asymmetrical pattern in 34 patients (78%). Large joints of lower extremity were most involved (29 patients, 68%). Rheumatoid factor and antinuclear antibodies were negative in all patients. Relapses occurred in 7 patients (16%) after a mean period of 6.2 months. Conclusion: It is concluded that the epidemiological and clinical features of Reiter's syndrome in Jordan are not different from those in the literature. (author)

  16. Prevalence of Burnout Syndrome in Patients Admitted with Acute Coronary Syndrome

    Directory of Open Access Journals (Sweden)

    Ana Cláudia Giaxa Prosdócimo

    2015-03-01

    Full Text Available Background: Burnout Syndrome is the extreme emotional response to chronic occupational stress, manifesting as physical and mental exhaustion. Although associated with higher prevalence of cardiovascular risk factors, no study so far has evaluated whether the Burnout Syndrome could be a prevalent factor in non-elderly individuals active in the labor market, admitted for acute coronary syndrome (ACS. Objective: To evaluate the prevalence of the Burnout Syndrome in non-elderly, economically active patients, hospitalized with ACS. Methods: Cross-sectional study conducted in a tertiary and private cardiology center, with economically active patients aged <65 years, hospitalized with diagnosis of ACS. The Burnout Syndrome was evaluated with the Burnout Syndrome Inventory (BSI, which assesses workplace conditions and four dimensions that characterize the syndrome: emotional exhaustion (EE, emotional distancing (EmD, dehumanization (De and professional fulfillment (PF. The Lipp’s Stress Symptoms Inventory for Adults (LSSI was applied to evaluate global stress. Results: Of 830 patients evaluated with suspected ACS, 170 met the study criteria, 90% of which were men, overall average age was 52 years, and 40.5% had an average income above 11 minimum wages. The prevalence of the Burnout Syndrome was 4.1%. When we evaluated each dimension individually, we found high EE in 34.7%, high De in 52.4%, high EDi in 30.6%, and low PF in 5.9%. The overall prevalence of stress was 87.5%. Conclusion: We found a low prevalence of Burnout Syndrome in an economically active, non-elderly population among patients admitted for ACS in a tertiary and private hospital.

  17. Abdominal Compartment Syndrome in Surgical Patients

    African Journals Online (AJOL)

    abdominal hypertension and abdominal compartment syndrome, affect ... timely surgical intervention is crucial. Key words: .... On the second postoperative day, he was noted to be restless ... Although surgery is very effective in managing ACS.

  18. Incidence of refeeding syndrome in internal medicine patients

    NARCIS (Netherlands)

    Kraaijenbrink, B. V. C.; Lambers, W. M.; Mathus-Vliegen, E. M. H.; Siegert, C. E. H.

    2016-01-01

    Refeeding syndrome is a potentially fatal shift of fluids and electrolytes that may occur after reintroducing nutrition in a malnourished patient. Its incidence in internal medicine patients is not known. We aimed at determining the incidence in a heterogeneous group of patients acutely admitted to

  19. Toxicity of oral radiotherapy in patients with acquired immunodeficiency syndrome

    International Nuclear Information System (INIS)

    Cooper, J.S.; Fried, P.R.

    1987-01-01

    Although radiotherapy is a standard form of management of head and neck tumors, treatment of the oral cavity in patients who have the acquired immunodeficiency syndrome has produced unacceptable toxicity. Five such patients are described as a warning of enhanced toxicity of oral radiotherapy in this patient population

  20. Dental management of patient with Williams Syndrome - A case report.

    Science.gov (United States)

    Wong, Daniel; Ramachandra, Srinivas Sulugodu; Singh, Ashish Kumar

    2015-01-01

    Williams syndrome is a multisystemic rare genetic disorder caused by deletion of 26-28 genes in the long arm of chromosome 7. It is characterized by developmental and physical abnormalities including congenital cardiovascular abnormalities, mental retardation, neurological features, growth deficiency, genitourinary manifestations, gastrointestinal problems, musculoskeletal problems, unique behavioral characteristics, and dental problems. Dental abnormalities include malocclusion, hypodontia, malformed teeth, taurodontism, pulp stones, increased space between teeth, enamel hypoplasia, and high prevalence of dental caries. Authors report a 17-year-old female patient with underlying Williams syndrome. Oral features and problems seen in the patient are listed. Malocclusion and screwdriver shaped teeth were noticed. Generalized widening of the periodontal ligament space with vital teeth was seen. This finding has not been reported in cases of Williams syndrome earlier. Precautions taken during dental treatment in patients with Williams syndrome are also discussed.

  1. Patient with confirmed LEOPARD syndrome developing multiple melanoma.

    Science.gov (United States)

    Colmant, Caroline; Franck, Deborah; Marot, Liliane; Matthijs, Gert; Sznajer, Yves; Blomme, Sandrine; Tromme, Isabelle

    2018-01-01

    LEOPARD syndrome, also known as Gorlin syndrome II, cardiocutaneous syndrome, lentiginosis profusa syndrome, Moynahan syndrome, was more recently coined as Noonan syndrome with multiple lentigines (NSML), inside the RASopathies. Historically, the acronym LEOPARD refers to the presence of distinctive clinical features such as: lentigines (L), electrocardiographic/conduction abnormalities (E), ocular hypertelorism (O), pulmonary stenosis (P), genital abnormalities (A), retardation of growth (R), and sensorineural deafness (D). This condition is identified in 85% of patients with phenotype hallmarks caused by presence a germline point mutation in PTPN11 gene. Association of melanoma to NSML seems to be rare: to our knowledge, two patients so far were reported in the literature. We herein present a patient diagnosed with LEOPARD syndrome, in whom molecular investigation confirmed the presence of the c.1403C>T mutation in exon 12 of the PTPN11 gene, who developed four superficial spreading melanomas and three atypical lentiginous hyperplasias. Three of the melanomas were achromic or hypochromic, three were in situ, and one had a Breslow index under 0.5 mm. Dermoscopic examination showed some characteristic white structures in most of the lesions, which were a signature pattern and a key for the diagnosis.

  2. Optimal right heart filling pressure in acute respiratory distress syndrome determined by strain echocardiography.

    Science.gov (United States)

    Garcia-Montilla, Romel; Imam, Faryal; Miao, Mi; Stinson, Kathryn; Khan, Akram; Heitner, Stephen

    2017-06-01

    Right ventricular (RV) systolic dysfunction is common in acute respiratory distress syndrome (ARDS). While preload optimization is crucial in its management, dynamic fluid responsiveness indices lack reliability, and there is no consensus on target central venous pressure (CVP). We analyzed the utility of RV free wall longitudinal strain (RVFWS) in the estimation of optimal RV filling pressure in ARDS. A retrospective cross-sectional analysis of clinical data and echocardiograms of patients with ARDS was performed. Tricuspid annular plane systolic excursion (TAPSE), tricuspid peak systolic velocity (S'), RV fractional area change (RVFAC), RVFWS, CVP, systolic pulmonary artery pressure (SPAP), and left ventricular ejection fraction (LVEF) were measured. Fifty-one patients with moderate-severe ARDS were included. There were inverse correlations between CVP and TAPSE, S', RVFAC, RVFWS, and LVEF. The most significant was with RVFWS (r:.74, R 2 :.55, P:.00001). Direct correlations with creatinine and lactate were noted. Receiver operating characteristic analysis showed that RVFWS -21% (normal reference value) was associated with CVP: 13 mm Hg (AUC: 0.92, 95% CI: 0.83-1.00). Regression model analysis of CVP, and RVFWS interactions established an RVFWS range from -18% to -24%. RVFWS -24% corresponded to CVP: 11 mm Hg and RVFWS -18% to CVP: 15 mm Hg. Beyond a CVP of 15 mm Hg, biventricular systolic dysfunction rapidly ensues. Our data are the first to show that an RV filling pressure of 13±2 mm Hg-as by CVP-correlates with optimal RV mechanics as evaluated by strain echocardiography in patients with moderate-severe ARDS. © 2017, Wiley Periodicals, Inc.

  3. [The influence of positive end-expiratory pressure on cerebral blood flow and cerebrovascular autoregulation in patients with acute respiratory distress syndrome].

    Science.gov (United States)

    Yang, Chunli; Chen, Zhi; Lu, Yuanhua; He, Huiwei; Zeng, Weihua

    2014-05-01

    To explore the influence of different positive end-expiratory pressure (PEEP) levels on cerebral blood flow (CBF) and cerebrovascular autoregulation in patients with acute respiratory distress syndrome(ARDS). A prospective study was conducted. Moderate or severe ARDS patients admitted to Department of Critical Care Medicine of Jiangxi Provincial People's Hospital from January 1st, 2013 to October 1st, 2013 were enrolled. The changes in hemodynamics, respiratory mechanics and gas exchange under different levels of PEEP were observed. CBF velocity of middle cerebral artery (MCA) was measured using transcranial Doppler (TCD), and breath-holding index (BHI) was also calculated. 35 patients with ARDS were included. The oxygenation index (OI), peak inspiratory pressure (PIP), plat pressure (Pplat) and central venous pressure (CVP) were markedly elevated (OI: 324.7±117.2 mmHg vs. 173.4±95.8 mmHg, t=5.913, P=0.000; PIP: 34.7±9.1 cmH2O vs. 26.1±7.9 cmH2O,t=4.222, P=0.000; Pplat: 30.5±8.4 cmH2O vs. 22.2±7.1 cmH2O, t=4.465, P=0.000; CVP: 12.1±3.5 mmHg vs. 8.8±2.2 mmHg, t=4.723, P=0.000) when PEEP was increased from (6.4±1.0) cmH2O to (14.5±2.0) cmH2O (1 cmH2O=0.098 kPa). But no significant difference in the heart rate (85.5±19.1 beats/min vs. 82.7±17.3 beats/min, t=0.643, P=0.523), mean arterial pressure (73.5±12.4 mmHg vs. 76.4±15.1 mmHg, t=0.878, P=0.383) and CBF velocity of MCA [peak systolic flow velocity (Vmax): 91.26±17.57 cm/s vs. 96.64±18.71 cm/s, t=1.240, P=0.219; diastolic flow velocity (Vmin): 31.54±7.71 cm/s vs. 33.87±8.53 cm/s, t=1.199, P=0.235; mean velocity (Vmean): 51.19±12.05 cm/s vs. 54.27±13.36 cm/s, t=1.013, P=0.315] was found. 18 patients with BHI<0.1 at baseline demonstrated that cerebral vasomotor reactivity was poor. BHI was slightly decreased with increase in PEEP (0.78±0.16 vs. 0.86±0.19, t=1.905, P=0.061). Some of moderate or severe ARDS patients without central nervous system disease have independent of preexisting cerebral

  4. ARD June07 New.pmd

    African Journals Online (AJOL)

    personal

    treatment. These complications may range from preseptal cellulitis, orbital cellulitis ... patients were seen during the dry season (November to February). The duration ... occurred in 14 patients (58.3%); right eye (4), left eye (10). Both eyes were ...

  5. [Features of the periodontal pathology at patients with metabolic syndrome].

    Science.gov (United States)

    Ermolaeva, L A; Shishkin, A N; Sheveleva, N A; Penkovoi, E A; Sheveleva, M A; Sokolovich, N A; Khabarova, O V; Mihailova, E S

    2016-01-01

    The purpose of this article is to familiarize readers on the relationship between metabolic syndrome and periodontitis, as well as common pathogenetic processes underlying these diseases. The data of modern researches, devoted to the correlation of lesions of periodontal and systemic diseases associated with metabolic syndrome. In the article analyzed also the data of the original study of the interaction of periodontitis and metabolic syndrome, which also used special methods of examination like Doppler ultrasound microcirculatory vasculature of the periodontal tissues and ultrasound densitometry. The possible methods of diagnostics of a condition of periodontal tissues in patients with metabolic syndrome are considered. Conclusions about the relationship of each component of metabolic syndrome with periodontitis are made.

  6. Efficiency of Combined Use of a Surfactant and the «Lung Opening» Maneuver in the Treatment of Acute Respiratory Distress Syndrome

    Directory of Open Access Journals (Sweden)

    A. V. Vlasenko

    2007-01-01

    Full Text Available The paper discusses whether the «lung opening» maneuver in combination with the endobronchial administration of a pulmonary surfactant can be used in the treatment of patients with acute respiratory distress syndrome (ARDS of various genesis. The authors outline data of their studies of the separate use of both methods and present the results of successful treatment in a patient with severe concomitant injury and posttraumatic ARDS in the combined use of the «lung opening» maneuver and Surfactant-BL. With intensive care, the combined use of these methods is a more effective way of improving gas exchange as compared with their use alone. Key words: acute respiratory distress syndrome, surfactant-BL, «lung opening» maneuver, combined use of both methods.

  7. Increased prevalence of metabolic syndrome in patients with acne inversa.

    Directory of Open Access Journals (Sweden)

    Robert Sabat

    Full Text Available BACKGROUND: Acne inversa (AI; also designated as Hidradenitis suppurativa is a common chronic inflammatory skin disease, localized in the axillary, inguinal and perianal skin areas that causes painful, fistulating sinuses with malodorous purulence and scars. Several chronic inflammatory diseases are associated with the metabolic syndrome and its consequences including arteriosclerosis, coronary heart disease, myocardial infraction, and stroke. So far, the association of AI with systemic metabolic alterations is largely unexplored. METHODS AND FINDINGS: A hospital-based case-control study in 80 AI patients and 100 age- and sex-matched control participants was carried out. The prevalence of central obesity (odds ratio 5.88, hypertriglyceridemia (odds ratio 2.24, hypo-HDL-cholesterolemia (odds ratio 4.56, and hyperglycemia (odds ratio 4.09 in AI patients was significantly higher than in controls. Furthermore, the metabolic syndrome, previously defined as the presence of at least three of the five alterations listed above, was more common in those patients compared to controls (40.0% versus 13.0%; odds ratio 4.46, 95% confidence interval 2.02 to 9.96; P<0.001. AI patients with metabolic syndrome also had more pronounced metabolic alterations than controls with metabolic syndrome. Interestingly, there was no correlation between the severity or duration of the disease and the levels of respective parameters or the number of criteria defining the metabolic syndrome. Rather, the metabolic syndrome was observed in a disproportionately high percentage of young AI patients. CONCLUSIONS: This study shows for the first time that AI patients have a high prevalence of the metabolic syndrome and all of its criteria. It further suggests that the inflammation present in AI patients does not have a major impact on the development of metabolic alterations. Instead, evidence is given for a role of metabolic alterations in the development of AI. We recommend

  8. Disease pattern in Danish patients with Peutz-Jeghers syndrome

    DEFF Research Database (Denmark)

    Jelsig, A M; Qvist, N; Sunde, L

    2016-01-01

    PURPOSE: In this paper, we aimed to collect genetic and medical information on all Danish patients with Peutz-Jeghers syndrome (PJS), in order to contribute to the knowledge of phenotype and genotype. Peutz-Jeghers syndrome is a hereditary syndrome characterized by multiple hamartomatous polyps...... in the GI tract, mucocutaneous pigmentations, and an increased risk of cancer in the GI tract and at extraintestinal sites. Over 90 % of patients harbour a pathogenic mutation in STK11. METHODS: Based on the Danish Pathology Data Bank, the Danish National Patient Register, as well as information from...... of the small bowel as the most frequent presenting symptom. We noted 18 occurrences of cancer at various anatomical sites, including a case of thyroid cancer and penile cancer. Eight of the deceased patients had died of cancer. Eighteen different mutations in STK11 had been detected in 28 patients. CONCLUSION...

  9. Rare successful pregnancy in a patient with Swyer Syndrome

    Directory of Open Access Journals (Sweden)

    Jyoti Taneja

    2016-10-01

    Full Text Available Objective: To report a rare successful pregnancy after fertility treatment in a patient with Swyer syndrome. Design: Case report. Setting: Herts & Essex Fertility Centre, Cheshunt, UK. Patient(s: A 36-year-old patient with 46, XY gonadal dysgenesis. 31 year old husband with normal sperm analysis. Intervention(s: Chromosomal analysis, Saline infusion sonography, Pipelle endometrial scratch, ICSI using donor eggs, Embryo Transfer, and Caesarean delivery. Main Outcome Measure(s: Successful pregnancy and live birth. Result(s: Successful treatment with donor eggs, pregnancy, and delivery. Conclusion(s: A patient with 46, XY gonadal dysgenesis in a specially tailored fertility program, can maintain a normal pregnancy and delivery. Keywords: Swyer syndrome, XY female, Gonadal dysgenesis, Primary amenorrhoea and HRT, Pregnancy in Swyer syndrome

  10. Enhanced MRI in patients with Ramsay-Hunt's syndrome

    International Nuclear Information System (INIS)

    Yanagida, Masahiro; Ushiro, Koichi; Yamashita, Toshio; Kumazawa, Tadami; Katoh, Tsutomu

    1993-01-01

    Enhanced MRI was performed in 14 patients with Ramsay-Hunt,s syndrome to investigate the pathogenesis of this syndrome. All MRI studies were performed on a 0.5T superconductivity MRI system using a head coil with Gd-DTPA. Enhancement was observed in the areas of the distal internal auditory canal and labyrinthine segment in many patients, and was especially prominent in patients suffering from vertigo, tinnitus, and hearing loss. In some patients it involved not only the facial nerve of the internal auditory canal but also the cochlear nerve and vestibular nerves. Since histological changes of the facial nerve in patients with Ramsay-Hunt's syndrome are assumed to occur in the distal internal auditory canal and labyrinthine segment, which is more proximal than the geniculate ganglion, and the possibility is suggested that inflammation may be spread to the vestibular and cochlear nerve via the internal auditory canal. (14 refs., 2 figs.)

  11. Enhanced MRI in patients with Ramsay-Hunt's syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Yanagida, Masahiro; Ushiro, Koichi; Yamashita, Toshio; Kumazawa, Tadami [Kansai Medical Univ., Osaka (Japan). Dept. of Otolaryngology; Katoh, Tsutomu [Kansai Medical Univ., Osaka (Japan). Dept. of Radiology

    1993-01-01

    Enhanced MRI was performed in 14 patients with Ramsay-Hunt,s syndrome to investigate the pathogenesis of this syndrome. All MRI studies were performed on a 0.5T superconductivity MRI system using a head coil with Gd-DTPA. Enhancement was observed in the areas of the distal internal auditory canal and labyrinthine segment in many patients, and was especially prominent in patients suffering from vertigo, tinnitus, and hearing loss. In some patients it involved not only the facial nerve of the internal auditory canal but also the cochlear nerve and vestibular nerves. Since histological changes of the facial nerve in patients with Ramsay-Hunt's syndrome are assumed to occur in the distal internal auditory canal and labyrinthine segment, which is more proximal than the geniculate ganglion, and the possibility is suggested that inflammation may be spread to the vestibular and cochlear nerve via the internal auditory canal. (14 refs., 2 figs.).

  12. [Pulmonary reperfusion syndrome after pulmonary stent implants in a patient with vascular tortuosity syndrome].

    Science.gov (United States)

    Berenguer Potenciano, M; Piris Borregas, S; Mendoza Soto, A; Velasco Bayon, J M; Caro Barri, A

    2015-01-01

    Vascular tortuosity syndrome is a rare genetic disorder that causes tortuosity and stenosis of the pulmonary, systemic and / or coronary circulations. As a result of treatment of pulmonary stenosis, symptoms of pulmonary edema, known as lung reperfusion syndrome, may occur. The case is presented of an adolescent patient with vascular tortuosity syndrome who presented with a pulmonary reperfusion syndrome after multiple stent implants in the left pulmonary artery. After the procedure, the patient immediately developed an acute pulmonary edema with severe clinical deterioration, which required assistance with extracorporeal membrane oxygenation for recovery. Copyright © 2014 Asociación Española de Pediatría. Published by Elsevier Espana. All rights reserved.

  13. Fluid in the management of the acute respiratory distress syndrome

    Directory of Open Access Journals (Sweden)

    Karki S

    2013-06-01

    Full Text Available Introduction Non-cardiogenic pulmonary edema is the hallmark of the acute respiratory distress syndrome (ARDS. The amount of fluid and which fluid should be used in these patients is controversial. Methods 43 patients with ARDS treated in the intensive care unit (ICU of the Second Hospital, Jilin University between November 1, 2011-November 1, 2012 were prospectively analyzed and was observational. Volume and the type of fluid administered were compared to 90 day mortality and the 24 and 72 hour sequential organ failure assessment (SOFA score, lactate level, oxygenation index (PaO2/FiO2, duration of ICU stay, total ventilator days, and need for continuous renal replacement therapy (CRRT. Results Mortality was increased when hydroxylethyl starch (HES was used in the first day or plasma substitutes were used during the first 3 days (P3000 ml during the first 24 hours or >8000 ml during the first 72 hours were associated with higher SOFA scores at 24 and 72 hours (P<0.05, both comparisons. Colloid, especially higher volume colloid use was also associated with increased SOFA scores at either 24 or 72 hours. Conclusions Limiting the use of colloids and the total amount of fluid administered to patients with ARDS is associated with improved mortality and SOFA scores.

  14. Musculo-Skeletal Abnormalities in Patients with Marfan Syndrome

    Directory of Open Access Journals (Sweden)

    Ali Al Kaissi

    2013-01-01

    Full Text Available Background A leptosomic body type is tall and thin with long hands. Marfanoid features may be familial in nature or pathological, as occurs in congenital contractual arachnodactyly (Beal's syndrome and Shprintzen-Goldberg syndrome mimicking some of the changes of Marfan syndrome, although not accompanied by luxation of lens and dissecting aneurysm of aorta. Methods In this article we collected eight patients who were consistent with the diagnosis of Marfan syndrome via phenotypic and genotypic characterization. Results Our patients manifested a constellation of variable presentations of musculo-skeletal abnormalities ranging from developmental dysplasia of the hip, protrusio acetabuli, leg length inequality, patellar instability, scoliosis, to early onset osteoarthritis. Each abnormality has been treated accordingly. Conclusion This is the first paper which includes the diagnosis and the management of the associated musculo-skeletal abnormalities in patients with Marfan syndrome, stressing that patients with Marfan syndrome are exhibiting great variability in the natural history and the severity of musculo-skeletal abnormalities.

  15. Metabolic syndrome in asthmatic patients of hazara division

    International Nuclear Information System (INIS)

    Ahmed, N.; Kazim, S.M.; Gillani, S.Y.

    2017-01-01

    Bronchial asthma is a common disease and most asthmatics are obese. Both asthma and obesity are showing parallel trends in their increasing prevalence. Obesity is also the main component of metabolic syndrome and several studies have shown metabolic syndrome to be associated with bronchial asthma. The present study was, therefore, designed to determine the frequency of metabolic syndrome among patients with chronic asthma in our setup. Methods: This cross-sectional study was conducted in Department of Medicine, Ayub Teaching Hospital, Abbottabad from May to November, 2014. One hundred and fifty-four asthmatic patients were enrolled in this study. Samples for blood glucose, triglycerides and HDL Cholesterol were taken after an overnight fast. Sitting blood pressure was measured with mercury sphygmomanometer after 10 minutes of rest. Waist circumference was measured at the level of the midpoint between the high point of the iliac crest and the last rib. Results: Out of 154 patients, 80 were males and 74 were females. Metabolic syndrome was diagnosed in 46 (29.87 percent) patients. When metabolic syndrome was stratified according to age, sex and duration of asthma, the results were found to be insignificant (p-0.89, 0.30 and 0.85). Conclusion: This study showed that metabolic syndrome was present in almost one third of study population. (author)

  16. Optic nerve pilomyxoid astrocytoma in a patient with Noonan syndrome.

    Science.gov (United States)

    Nair, Sushmita; Fort, John A; Yachnis, Anthony T; Williams, Charles A

    2015-06-01

    Noonan syndrome (NS; MIM 163950) is an autosomal dominant syndrome which is clinically diagnosed by the distinct facial features, short stature, cardiac anomalies and developmental delay. About 50% of cases are associated with gain of function mutations in PTPN11 gene which leads to activation of the RAS/mitogen-activated protein kinase signaling pathway. This is known to have a role in tumorigenesis. Despite this, only limited reports of solid tumors (Fryssira H, Leventopoulos G, Psoni S, et al. Tumor development in three patients with Noonan syndrome. Eur J Pediatr 2008;167:1025-1031; Schuettpelz LG, McDonald S, Whitesell K et al. Pilocytic astrocytoma in a child with Noonan syndrome. Pediatr Blood Cancer 2009;53:1147-1149; Sherman CB, Ali-Nazir A, Gonzales-Gomez I, et al. Primary mixed glioneuronal tumor of the central nervous system in a patient with Noonan syndrome. J Pediatr Hematol Oncol 2009;31:61-64; Sanford RA, Bowman R, Tomita T, et al. A 16 year old male with Noonan's syndrome develops progressive scoliosis and deteriorating gait. Pediatr Neurosurg 1999;30:47-52) and no prior reports of optic gliomas have been described in patients with NS. We present here a patient with NS with a PTPN11 mutation and an optic pathway pilomyxoid astrocytoma. © 2015 Wiley Periodicals, Inc.

  17. Cases of Churg-Strauss syndrome in patients receiving montelukast

    Directory of Open Access Journals (Sweden)

    Petrović Jelena

    2012-01-01

    Full Text Available Churg-Strauss syndrome is a rare disorder, but in patients with asthma it may develop as an adverse effect of the administered drugs. The aim of this study was to investigate possible causal relationship between montelukast and the occurrence of Churg-Strauss syndrome. Medical literature was reviewed by searching the databases 'Medline' and 'Googlescholar', in order to detect published cases of Churg-Strauss syndrome associated with use of montelukast. In this article is included 13 publications which contain the following keywords: montelukast, Churg-Strauss syndrome and side effects. Relationship between use of montelukast and development of Churg-Strauss syndrome was not clearly causal, although montelukast was associated with development and relapse of the syndrome. This fact supports the hypothesis that leukotriene antagonists are involved in the pathogenesis of this serious disease. Special attention should be paid to appearance of new symptoms in an asthmatic patient, already treated with corticosteroids, who start receiving leukotriene antagonists, especially if the dose of corticosteroids is reduced. Definitive confirmation or rejection of the hypothesis that leukotriene antagonists are directly involved in the development of this syndrome require further investigations.

  18. Fluctuating Cotard syndrome in a patient with advanced Parkinson disease.

    Science.gov (United States)

    Solla, Paolo; Cannas, Antonino; Orofino, Gianni; Marrosu, Francesco

    2015-02-01

    Nonmotor fluctuations of psychiatric symptoms in patients suffering from Parkinson disease (PD) represent a very disabling condition, which may seriously interfere with the quality of life of patients and caregivers. In this regard, these disturbances are present with a higher frequency in advanced PD patients with associated motor complications and can appear both in "on" and in "off" period. Here we report on a case of fluctuating Cotard syndrome clearly related to "wearing-off" deterioration and responsive to levodopa treatment in a patient affected by advanced PD. A 76-year-old woman presented with a 13-year history of PD. Her caregivers reported that, in the last 2 months, she has developed a sudden onset of nihilistic delusion (Cotard syndrome), mainly during the "wearing-off" condition and associated with end of dose dyskinesias and akathisia.As Cotard syndrome clearly improved with the administration of levodopa, the patient was successfully treated changing the levodopa schedule with the shortening of intervals between levodopa intakes in small doses. Both the appearance of the Cotard syndrome in this patient during the "off" state and the subsequent improvement of psychotic symptoms after levodopa administration strongly suggest an important correlation with the dopaminergic dysregulation.This finding suggests that dopaminergic deficit might play a key factor in the development of Cotard syndrome.

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

    Science.gov (United States)

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

    2018-03-01

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

  20. Cyclic Bicytopenia in a Patient with Shapiro Syndrome

    Directory of Open Access Journals (Sweden)

    Lindsey E. Roeker

    2013-01-01

    Full Text Available Shapiro syndrome and periodic hypothermia have been reported approximately fifty times in the literature. Shapiro syndrome is defined as the constellation of periodic hypothermia and hyperhidrosis along with agenesis of the corpus callosum by Shapiro et al. in 1969. Periodic hypothermia is a more broad diagnosis with a number of proposed mechanisms; it occurs in patients without structural brain abnormalities. Hematologic abnormalities beyond iron-deficiency anemia have not been documented in any of the reported cases of Shapiro syndrome or periodic hypothermia. Though accidental and therapeutic hypothermia have been associated with thrombocytopenia, this is, to our knowledge, the first reported case of periodic intrinsic hypothermia causing bicytopenia. In this report, we present the case of a patient with Shapiro syndrome who experienced cyclic bicytopenia mirroring hypothermic episodes. We address the differential diagnosis of bicytopenia, review the mechanisms proposed for cytopenias related to hypothermia, and propose possible mechanisms for the finding in this case.

  1. Noonan syndrome: a clinical and genetic study of 31 patients

    Directory of Open Access Journals (Sweden)

    Bertola Débora Romeo

    1999-01-01

    Full Text Available Noonan syndrome is a multiple congenital anomaly syndrome, inherited in an autosomal dominant pattern. We studied 31 patients (18 males and 13 females affected by this disorder regarding their clinical and genetic characteristics. The most frequent clinical findings were short stature (71%; craniofacial dysmorphisms, especially hypertelorism, ptosis, downslanting of the palpebral fissures; short or webbed neck (87%; cardiac anomalies (65%, and fetal pads in fingers and toes (70%. After studying the probands' first-degree relatives, we made the diagnosis of Noonan syndrome in more than one family member in three families. Therefore, the majority of our cases were sporadic.

  2. Craniofacial morphology in Turner syndrome patients treated with growth hormone

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    Jovana Julsoki

    2015-05-01

    Full Text Available ABSTRACT Introduction: In addition to well-established physical characteristics, Turner syndrome patients have distinct craniofacial morphology. Since short stature is the most typical characteristic, Turner syndrome patients are commonly treated with growth hormone in order to increase final height. At the same time, growth hormone treatment was found to influence craniofacial growth and morphology in various groups of treated patients. Whereas craniofacial characteristics of Turner syndrome patients are well documented, comparatively little is known of craniofacial morphology of those who are treated with growth hormone. Aim: The aim of this study was to investigate craniofacial morphology in Turner syndrome patients treated with growth hormone in comparison to healthy females. Materials and methods: The cephalometric evaluation was conducted on twenty lateral cephalograms of Turner syndrome patients (13.53 ± 4.04 years treated with growth hormone for at least one year (4.94 ± 1.92 years in average. As a control group, forty lateral cephalograms of healthy female controls, who matched Turner syndrome patients by chronological (11.80 ± 2.37 years and skeletal age, were used. Eleven angular, seven linear measurements and six dimensional ratios were measured to describe craniofacial morphology. Results: The results obtained for angular measurements, in cephalometric analyses for Turner syndrome patients treated with growth hormone, revealed bimaxillary retrognathism. The linear measurements indicated longer mandibular ramus, anterior cranial base and both anterior and posterior facial heights. However, posterior cranial base and maxilla were in proportion to the anterior cranial base, when comparing dimensional ratios. Anterior cranial base, maxilla and mandibular ramus were larger in proportion to mandibular body; as well as posterior facial height was when compared to anterior facial height. Turner syndrome patients treated with growth

  3. Patients with uninjured lungs may also benefit from lung-protective ventilator settings [version 1; referees: 2 approved

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    Roger Alencar

    2017-11-01

    Full Text Available Although mechanical ventilation is a life-saving strategy in critically ill patients and an indispensable tool in patients under general anesthesia for surgery, it also acts as a double-edged sword. Indeed, ventilation is increasingly recognized as a potentially dangerous intrusion that has the potential to harm lungs, in a condition known as ‘ventilator-induced lung injury’ (VILI. So-called ‘lung-protective’ ventilator settings aiming at prevention of VILI have been shown to improve outcomes in patients with acute respiratory distress syndrome (ARDS, and, over the last few years, there has been increasing interest in possible benefit of lung-protective ventilation in patients under ventilation for reasons other than ARDS. Patients without ARDS could benefit from tidal volume reduction during mechanical ventilation. However, it is uncertain whether higher levels of positive end-expiratory pressure could benefit these patients as well. Finally, recent evidence suggests that patients without ARDS should receive low driving pressures during ventilation.

  4. Culture-bound syndromes in Hispanic primary care patients.

    Science.gov (United States)

    Bayles, Bryan P; Katerndahl, David A

    2009-01-01

    We sought to document Hispanic primary care patients' knowledge and experience of five culture-bound syndromes (CBS), as well as the basic socio-cultural correlates of these disorders. A convenience sample of 100 adult Hispanic patients presenting in an urban South Texas primary care clinic was recruited to complete a brief cross-sectional survey, presented in an oral format. Interviews sought information concerning five culture-bound syndromes--susto, empacho, nervios, mal de ojo, and ataques de nervios. Additional demographic, socio-economic, and acculturation data was collected. Descriptive and bivariate statistics (chi square, Fisher's) were used to assess relationships among variables and experience with each CBS. A multivariate logistic analysis was conducted to determine the possible contributions of age, gender, acculturation, and education to the personal experience of a culture-bound syndrome. Results indicate that 77% of respondents had knowledge of all five syndromes, with 42% reporting having personally experienced at least one CBS. Nervios was the most commonly suffered disorder, being reported by 30 respondents. This was followed, in declining order ofprevalence, by susto, mal de ojo, empacho, and ataques de nervios. Multivariate logistic regression analysis found that higher education beyond high school was associated with a slightly decreased likelihood of reporting having suffered from any culture-bound syndrome. While co-occurrence among these disorders occurred, the patterns of predictors suggest that the co-occurrence is not a reflection of mislabeling of one common syndrome. Knowledge of and experience with culture-bound syndromes is common among Hispanic primary care patients in South Texas. Healthcare providers ought to consider discussing these illnesses in a non-judgmental manner with patients who present with symptoms that are consistent with these syndromes. Future studies, with larger sample sizes, are warranted to elucidate the nature

  5. Detrimental ELAVL-1/HuR-dependent GSK3β mRNA stabilization impairs resolution in acute respiratory distress syndrome.

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    Olivia Hoffman

    Full Text Available A hallmark of acute respiratory distress syndrome (ARDS is accumulation of protein-rich edema in the distal airspaces and its removal is critical for patient survival. Previous studies have shown a detrimental role of Glycogen Synthase Kinase (GSK 3β during ARDS via inhibition of alveolar epithelial protein transport. We hypothesized that post-transcriptional regulation of GSK3β could play a functional role in ARDS resolution. To address this hypothesis, we performed an in silico analysis to identify regulatory genes whose expression correlation to GSK3β messenger RNA utilizing two lung cancer cell line array datasets. Among potential regulatory partners of GSK3β, these studies identified the RNA-binding protein ELAVL-1/HuR (Embryonic Lethal, Abnormal Vision, Drosophila-Like as a central component in a likely GSK3β signaling network. ELAVL-1/HuR is a RNA-binding protein that selectively binds to AU-rich elements of mRNA and enhances its stability thereby increasing target gene expression. Subsequent studies with siRNA suppression of ELAVL-1/HuR demonstrated deceased GSK3β mRNA and protein expression and improved clearance of FITC-albumin in A549 cells. Conversely, stabilization of ELAVL-1/HuR with the proteasome inhibitor MG-132 resulted in induction of GSK3β at mRNA and protein level and attenuated FITC-albumin clearance. Utilizing ventilator-induced lung injury or intra-tracheal installation of hydrochloric acid to induce ARDS in mice, we observed increased mRNA and protein expression of ELAVL-1/HuR and GSK3β. Together, our findings indicate a previously unknown interaction between GSK3β and ELAV-1 during ARDS, and suggest the inhibition of the ELAV-1- GSK3β pathways as a novel ARDS treatment approach.

  6. CE: Nursing Management of Patients with Ehlers-Danlos Syndrome.

    Science.gov (United States)

    Anderson, Linda K

    2015-07-01

    Ehlers-Danlos syndrome (EDS), a hereditary connective tissue disorder, has historically been misunderstood and underdiagnosed by health care providers. Because of the high degree of phenotypic variability, patients are often correctly diagnosed only after years of seemingly unrelated but debilitating injuries and illnesses. Specific genetic mutations have been identified for some, but not all, EDS types; patients presenting with a high index of suspicion should be referred to a geneticist. As awareness and recognition of the syndrome improve, nurses are increasingly likely to care for patients with EDS. This article gives a brief overview of the syndrome and provides guidance on ways to manage symptoms, recognize and prevent serious complications, and improve patients' quality of life.

  7. Report of seven neurological patients with misidentification syndrome

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    Edson José Amâncio

    2004-12-01

    Full Text Available Objective: To present clinical, neuropsychological and laboratorydata on 7 patients with misidentification syndrome and to discussits possible etiologies and pathophysiology. Methods: Sevenpatients presenting misidentification syndrome, 6 female and 1male, aged 64-78 years were studied. All had a brain diseasediagnosed by clinical and laboratory data. All patients weresubmitted to general clinical examination, neurological andneuropsychological examinations, and brain magnetic resonanceimaging. Results: All patients were capable to recognizephotographs of relatives or famous persons. They presented goodvisual acuity that allowed them reading texts with small print andpreserved visual field. The etiologies of brain lesions were ischemicstroke, left temporal lobe tumor, idiopathic hydrocephalus in elderlypatients, Parkinson’s disease and probable Alzheimer’s disease.None presented enough cognitive disorders to characterize seniledementia. Conclusion: Misidentification syndromes are notnecessarily related to one single psychogenic etiology; on thecontrary, many organic causes may be related with the clinicalpicture. Most patients improved when submitted to treatmentwith typical or atypical neuroleptic drugs.

  8. Acute retroviral syndrome in Slovenian patients infected with HIV

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    Mateja Pirš

    2005-06-01

    Full Text Available Background: Two to six weeks after primary infection with HIV 50 to 90 percent of patients develop an acute retroviral syndrome which usually presents with mononucleosis or flu-like illness. Due to nonspecific symptoms ARS is frequently misdiagnosed.Patients and methods: Data of Slovenian patients with acute retroviral syndrome is shown, as well as their symptoms, approaches to management and diagnostic particularities of primary HIV infection.Conclusions: The combination of particular symptoms and epidemiological data should lead us to consider the possibility of an early HIV infection.

  9. Longitudinal Neuropsychological Profile in a Patient with Triple A Syndrome

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    Luigi Mazzone

    2013-01-01

    Full Text Available Triple A syndrome is an autosomal recessive disorder characterized by the triad of adrenocorticotropic hormone resistant adrenal insufficiency, achalasia, and alacrima. Our aim was to describe the neuropsychological characteristics and the cooccurring psychopathological and neurological disorders in an Italian male child suffering from Triple A syndrome at the time of admission (T0 and after one year of follow-up (T1. Many difficulties were observed in the motor domain, as well as in manual dexterity and static/dynamic balance domains of the motor task over time. In sharp contrast with previous literature reports on frequent mild cognitive dysfunction in patients with Triple A syndrome, our child did not show any mental retardation. By contrast, he showed an average IQ at T0 with a slight improvement at T1. To our knowledge, this report is the first describing neuropsychological profile and co-occurring psychopathological problems in a child with Triple A syndrome. Considering that the Triple A syndrome is a progressive disorder which can take years to develop the full-blown clinical picture, these patients require periodical medical controls. Moreover, assessment of neuropsychological and psychopathological features should be performed in patients with this disease, in order to underline the variability of this syndrome.

  10. Specifics of mental disorders of patients with metabolic syndrome

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    K. I. Kleban

    2017-09-01

    Full Text Available In the general-somatic network there is a steady increase in the number of patients with psychosomatic disorders. Problems of providing adequate psychiatric and psychotherapeutic assistance to this category of patients are related to the motivation of patients to participate in psychological measures and the readiness of the medical system to provide comprehensive care on the basis of the biopsychosocial approach. Mental factors are involved both in the occurrence and course of a metabolic syndrome in the form of a patient's lifestyle and behavior patterns of healthy functioning, and is a consequence of somatic pathology. Mental factors are involved both in the occurrence and course of a metabolic syndrome in the form of a patient's lifestyle and behavior patterns of healthy functioning, and is a consequence of somatic pathology. So mental disorders of metabolic syndrome are manifested in the form of psychosocial maladaptation, neurotic, affective, personality, and organic disorders. Desynchronosis which is a factor of the development of a metabolic syndrome and characterizes the complex chronobiological component of the regulation of psychophysiological functions in norm and under the influence of stress, deserves special attention. Addressing the diagnosis of mental disorders associated with metabolic syndrome is precisely aimed at determining chronobiological disorders of psychosomatic integrated areas and is supposed to improve diagnostic and treatment process and to shorten the treatment of these disorders.

  11. Cogan's Syndrome in a Jordanian patient: A case report | Al ...

    African Journals Online (AJOL)

    We reported a Jordanian case of Cogan's Syndrome (CS). A 22-year old male patient presented with interstitial keratitis. The patient was treated successfully with topical steroids but over the following months, he developed vertigo, sensorineural hearing loss (SNHL) and generalized vasculitis. This is the first reported case ...

  12. Association of metabolic syndrome in patients with osteoarthritis

    International Nuclear Information System (INIS)

    Malik, S.; Salim, B.; Khalil, Z.; Nasim, A.

    2015-01-01

    Objective: To determine association of osteoarthritis (OA) with metabolic syndrome in a tertiary care hospital of Pakistan. Methodology: A cross-sectional study was conducted at Fauji Foundation Hospital, Rawalpindi, Pakistan. Patients were randomly interviewed in the Female Rheumatology department and a total of 240 patients with single rheumatologic disease and age >35 years were selected. Informed consent was taken and patients were interviewed using a self-made questionnaire to evaluate their medical history, physical and laboratory examination. SPSS version 17 was used to analyze the data. Results: Out of 240 subjects, 81 patients had OA and another 81 patients were randomly selected from the age and gender matched control (non-OA) group. The mean age of patients in OA and non-OA group was 56.68 ± 09.76 and 53.57 ± 11.01 years, respectively. In OA group, 48.1% and in non-OA group 22.2% of patients were falling in category of being obese/morbidly obese. According to AHA criteria for Metabolic Syndrome, percentage of OA patients labeled to have metabolic syndrome was 58.8% as compared to 19.5% in non-OA group. Conclusion: There was a strong association of metabolic syndrome with OA and would surely make a foreground for future studies to be conducted on developing preventive strategies and ultimately reducing the morbidities and mortalities associated with Metabolic Osteoarthritis. (author)

  13. Value of urine cytology in screening patients with prostatitis syndromes

    NARCIS (Netherlands)

    de la Rosette, J. J.; Hubregtse, M. R.; Wiersma, A. M.; Debruyne, F. M.

    1993-01-01

    We reviewed the results of urine cytology examination of 206 patients with a diagnosis of prostatitis syndromes in the period 1985-1991. The urine samples showed an incidence of 20.4% for slight to moderate atypia and 6.3% for severe atypia. In these patients, cystoscopy, bladder biopsies and

  14. A further patient with Pai syndrome with autosomal dominant inheritance?

    OpenAIRE

    Rudnik-Schöneborn, S; Zerres, K

    1994-01-01

    We report a patient with median cleft of the upper lip, cutaneous facial polyps, and lipoma of the corpus callosum who represents a further case of Pai syndrome. The father of the patient showed coloboma of the right iris and shared some facial dysmorphism with his son, thus raising the question of autosomal dominant inheritance.

  15. Excess mortality in mothers of patients with polycystic ovary syndrome

    NARCIS (Netherlands)

    Louwers, Y. V.; Roest-Schalken, M. E.; Kleefstra, N.; van Lennep, J. Roeters; van den Berg, M.; Fauser, B. C. J. M.; Bilo, H. J. G.; Sijbrands, E. J. G.; Laven, J. S. E.

    STUDY QUESTION: Do diabetic parents of patients with polycystic ovary syndrome (PCOS) encounter excess mortality compared with the mortality of men and women with type 2 diabetes, recruited without selection for PCOS? SUMMARY ANSWER: Type 2 diabetes among mothers of PCOS patients results in excess

  16. Protein Carbonylation in Patients with Myelodysplastic Syndromes

    Czech Academy of Sciences Publication Activity Database

    Hlaváčková, A.; Štikarová, J.; Kotlín, R.; Chrastinová, L.; Šácha, Pavel; Májek, P.; Čermák, J.; Suttnar, J.; Dyr, J. E.

    2015-01-01

    Roč. 126, č. 23 (2015), s. 5232 ISSN 0006-4971. [Annual Meeting and Exposition of the American Society of Hematology /55./. 07.12.2013-10.12.2013, New Orleans] Institutional support: RVO:61388963 Keywords : protein carbonylation * myelodysplastic syndromes Subject RIV: CE - Biochemistry

  17. Posterior reversible encephalopathy syndrome in patient

    African Journals Online (AJOL)

    abp

    2015-05-26

    May 26, 2015 ... reversible Posterior leukoencephalopathy syndrome [1, 2]. The. PRES is a clinical and radiological entity associating varying degrees, headaches, impaired consciousness, seizures and visual disturbances to neurological and radiological abnormalities of the parietal-occipital white matter [3]. PRES has a ...

  18. Setting ventilation parameters guided by electrical impedance tomography in an animal trial of acute respiratory distress syndrome

    Science.gov (United States)

    Czaplik, Michael; Biener, Ingeborg; Leonhardt, Steffen; Rossaint, Rolf

    2014-03-01

    Since mechanical ventilation can cause harm to lung tissue it should be as protective as possible. Whereas numerous options exist to set ventilator parameters, an adequate monitoring is lacking up to date. The Electrical Impedance Tomography (EIT) provides a non-invasive visualization of ventilation which is relatively easy to apply and commercially available. Although there are a number of published measures and parameters derived from EIT, it is not clear how to use EIT to improve clinical outcome of e.g. patients suffering from acute respiratory distress syndrome (ARDS), a severe disease with a high mortality rate. On the one hand, parameters should be easy to obtain, on the other hand clinical algorithms should consider them to optimize ventilator settings. The so called Global inhomogeneity (GI) index bases on the fact that ARDS is characterized by an inhomogeneous injury pattern. By applying positive endexpiratory pressures (PEEP), homogeneity should be attained. In this study, ARDS was induced by a double hit procedure in six pigs. They were randomly assigned to either the EIT or the control group. Whereas in the control group the ARDS network table was used to set the PEEP according to the current inspiratory oxygen fraction, in the EIT group the GI index was calculated during a decremental PEEP trial. PEEP was kept when GI index was lowest. Interestingly, PEEP was significantly higher in the EIT group. Additionally, two of these animals died ahead of the schedule. Obviously, not only homogeneity of ventilation distribution matters but also limitation of over-distension.

  19. Lung inhomogeneities, inflation and [18F]2-fluoro-2-deoxy-D-glucose uptake rate in acute respiratory distress syndrome.

    Science.gov (United States)

    Cressoni, Massimo; Chiumello, Davide; Chiurazzi, Chiara; Brioni, Matteo; Algieri, Ilaria; Gotti, Miriam; Nikolla, Klodiana; Massari, Dario; Cammaroto, Antonio; Colombo, Andrea; Cadringher, Paolo; Carlesso, Eleonora; Benti, Riccardo; Casati, Rosangela; Zito, Felicia; Gattinoni, Luciano

    2016-01-01

    The aim of the study was to determine the size and location of homogeneous inflamed/noninflamed and inhomogeneous inflamed/noninflamed lung compartments and their association with acute respiratory distress syndrome (ARDS) severity.In total, 20 ARDS patients underwent 5 and 45 cmH2O computed tomography (CT) scans to measure lung recruitability. [(18)F]2-fluoro-2-deoxy-d-glucose ([(18)F]FDG) uptake and lung inhomogeneities were quantified with a positron emission tomography-CT scan at 10 cmH2O. We defined four compartments with normal/abnormal [(18)F]FDG uptake and lung homogeneity.The homogeneous compartment with normal [(18)F]FDG uptake was primarily composed of well-inflated tissue (80±16%), double-sized in nondependent lung (32±27% versus 16±17%, pinflation and [(18)F]FDG uptake decreases with ARDS severity, while the inhomogeneous poorly/not inflated compartment increases. Most of the lung inhomogeneities are inflamed. A minor fraction of healthy tissue remains in severe ARDS. Copyright ©ERS 2016.

  20. Rhabdomyosarcoma in patients with constitutional mismatch-repair-deficiency syndrome.

    Science.gov (United States)

    Kratz, C P; Holter, S; Etzler, J; Lauten, M; Pollett, A; Niemeyer, C M; Gallinger, S; Wimmer, K

    2009-06-01

    Biallelic germline mutations in the mismatch repair genes MLH1, MSH2, MSH6 or PMS2 cause a recessive childhood cancer syndrome characterised by early-onset malignancies and signs reminiscent of neurofibromatosis type 1 (NF1). Alluding to the underlying genetic defect, we refer to this syndrome as constitutional mismatch repair-deficiency (CMMR-D) syndrome. The tumour spectrum of CMMR-D syndrome includes haematological neoplasias, brain tumours and Lynch syndrome-associated tumours. Other tumours, such as neuroblastoma, Wilm tumour, ovarian neuroectodermal tumour or infantile myofibromatosis, have so far been found only in individual cases. We analysed two consanguineous families that had members with suspected CMMR-D syndrome who developed rhabdomyosarcoma among other neoplasias. In the first family, we identified a pathogenic PMS2 mutation for which the affected patient was homozygous. In family 2, immunohistochemistry analysis showed isolated loss of PMS2 expression in all tumours in the affected patients, including rhabdomyosarcoma itself and the surrounding normal tissue. Together with the family history and microsatellite instability observed in one tumour this strongly suggests an underlying PMS2 alteration in family 2 also. Together, these two new cases show that rhabdomyosarcoma and possibly other embryonic tumours, such as neuroblastoma and Wilm tumour, belong to the tumour spectrum of CMMR-D syndrome. Given the clinical overlap of CMMR-D syndrome with NF1, we suggest careful examination of the family history in patients with embryonic tumours and signs of NF1 as well as analysis of the tumours for loss of one of the mismatch repair genes and microsatellite instability. Subsequent mutation analysis will lead to a definitive diagnosis of the underlying disorder.

  1. Pediatric Acute Respiratory Distress Syndrome: Fluid Management in the PICU.

    Science.gov (United States)

    Ingelse, Sarah A; Wösten-van Asperen, Roelie M; Lemson, Joris; Daams, Joost G; Bem, Reinout A; van Woensel, Job B

    2016-01-01

    The administration of an appropriate volume of intravenous fluids, while avoiding fluid overload, is a major challenge in the pediatric intensive care unit. Despite our efforts, fluid overload is a very common clinical observation in critically ill children, in particular in those with pediatric acute respiratory distress syndrome (PARDS). Patients with ARDS have widespread damage of the alveolar-capillary barrier, potentially making them vulnerable to fluid overload with the development of pulmonary edema leading to prolonged course of disease. Indeed, studies in adults with ARDS have shown that an increased cumulative fluid balance is associated with adverse outcome. However, age-related differences in the development and consequences of fluid overload in ARDS may exist due to disparities in immunologic response and body water distribution. This systematic review summarizes the current literature on fluid imbalance and management in PARDS, with special emphasis on potential differences with adult patients. It discusses the adverse effects associated with fluid overload and the corresponding possible pathophysiological mechanisms of its development. Our intent is to provide an incentive to develop age-specific fluid management protocols to improve PARDS outcomes.

  2. Pediatric acute respiratory distress syndrome: fluid management in the PICU

    Directory of Open Access Journals (Sweden)

    Sarah A Ingelse

    2016-03-01

    Full Text Available The administration of an appropriate volume of intravenous fluids, while avoiding fluid overload, is a major challenge in the pediatric intensive care unit. Despite our efforts, fluid overload is a very common clinical observation in critically ill children, in particular in those with pediatric acute respiratory distress syndrome (PARDS. Patients with ARDS have widespread damage of the alveolar capillary barrier, potentially making them vulnerable to fluid overload with the development of pulmonary edema leading to prolonged course of disease. Indeed, studies in adults with ARDS have shown that an increased cumulative fluid balance is associated with adverse outcome. However, age-related differences in the development and consequences of fluid overload in ARDS may exist due to disparities in immunologic response and body water distribution. This systematic review summarizes the current literature on fluid imbalance and management in PARDS, with special emphasis on potential differences with adult patients. It discusses the adverse effects associated with fluid overload and the corresponding possible pathophysiological mechanisms of its development. Our intent is to provide an incentive to develop age-specific fluid management protocols to improve PARDS outcomes.

  3. H3N2 Virus as Causative Agent of ARDS Requiring Extracorporeal Membrane Oxygenation Support

    Directory of Open Access Journals (Sweden)

    Adriano Peris

    2014-01-01

    Full Text Available Pandemic influenza virus A(H1N1 2009 was associated with a higher risk of viral pneumonia in comparison with seasonal influenza viruses. The influenza season 2011-2012 was characterized by the prevalent circulation of influenza A(H3N2 viruses. Whereas most H3N2 patients experienced mild, self-limited influenza-like illness, some patients were at increased risk for influenza complications because of age or underlying medical conditions. Cases presented were patients admitted to the Intensive Care Unit (ICU of ECMO referral center (Careggi Teaching Hospital, Florence, Italy. Despite extracorporeal membrane oxygenation treatment (ECMO, one patient with H3N2-induced ARDS did not survive. Our experience suggests that viral aetiology is becoming more important and hospitals should be able to perform a fast differential diagnosis between bacterial and viral aetiology.

  4. Bronchoalveolar lavage with diluted porcine surfactant in mechanically ventilated term infants with meconium aspiration syndrome.

    Science.gov (United States)

    Lista, Gianluca; Bianchi, Silvia; Castoldi, Francesca; Fontana, Paola; Cavigioli, Francesco

    2006-01-01

    To evaluate the efficacy and safety of bronchoalveolar lavage (BAL) with diluted porcine surfactant in mechanically ventilated term infants with severe acute respiratory distress syndrome (ARDS) due to meconium aspiration syndrome (MAS). Eight consecutive mechanically ventilated term infants with severe ARDS due to MAS underwent BAL with 15 mL/kg of diluted (5.3mg phospholipid/mL) surfactant saline suspension (porcine surfactant [Curosurf]). Treatment was administered slowly in aliquots of 2.5 mL. The mean age of neonates at treatment was 3.5 (range 1-8) hours. Heart rate, systemic blood pressure and oxygen saturation were monitored continuously. Arterial blood gases were measured immediately before treatment, and again at 3 and 6 hours post-treatment. Chest x-rays were taken 6 and 24 hours after treatment. Radiological improvement was evident in all eight patients 6 hours post-treatment. Compared with pre-BAL values, significant improvements (p fluids that had been meconium-stained prior to BAL were clear of meconium after BAL. Only one patient required nitric oxide therapy for transient pulmonary hypertension. No adverse sequelae of treatment occurred during the study. BAL with dilute porcine surfactant administered slowly in 2.5 mL aliquots improved oxygenation and chest x-ray findings, without causing major adverse effects, in mechanically ventilated term infants with ARDS due to MAS.

  5. Does altered aortic flow in marfan syndrome relate to aortic root dilatation?

    Science.gov (United States)

    Wang, Hung-Hsuan; Chiu, Hsin-Hui; Tseng, Wen-Yih Isaac; Peng, Hsu-Hsia

    2016-08-01

    To examine possible hemodynamic alterations in adolescent to adult Marfan syndrome (MFS) patients with aortic root dilatation. Four-dimensional flow MRI was performed in 20 MFS patients and 12 age-matched normal subjects with a 3T system. The cross-sectional areas of 10 planes along the aorta were segmented for calculating the axial and circumferential wall shear stress (WSSaxial , WSScirc ), oscillatory shear index (OSIaxial , OSIcirc ), and the nonroundness (NR), presenting the asymmetry of segmental WSS. Pearson's correlation analysis was performed to present the correlations between the quantified indices and the body surface area (BSA), aortic root diameter (ARD), and Z score of the ARD. P < 0.05 indicated statistical significance. Patients exhibited lower WSSaxial in the aortic root and the WSScirc in the arch (P < 0.05-0.001). MFS patients exhibited higher OSIaxial and OSIcirc in the sinotubular junction and arch, but lower OSIcirc in the descending aorta (all P < 0.05). The NR values were lower in patients (P < 0.05). The WSSaxial or WSScirc exhibited moderate to strong correlations with BSA, ARD, or Z score (R(2)  = 0.50-0.72) in MFS patients. The significant differences in the quantified indices, which were associated with BSA, ARD, or Z score, in MFS were opposite to previous reports for younger MFS patients, indicating that altered flows in MFS patients may depend on the disease progress. The possible time dependency of hemodynamic alterations in MFS patients strongly suggests that longitudinal follow-up of 4D Flow is needed to comprehend disease progress. J. Magn. Reson. Imaging 2016;44:500-508. © 2016 Wiley Periodicals, Inc.

  6. Treatment of orofacial pain in patients with stylomandibular ligament syndrome (Ernest Syndrome).

    Science.gov (United States)

    Peñarrocha-Oltra, D; Ata-Ali, J; Ata-Ali, F; Peñarrocha-Diago, M A; Peñarrocha, M

    2013-06-01

    Ernest syndrome involves the stylomandibular ligament. It is characterised by pain in the preauricular area and mandibular angle, radiating to the neck, shoulder, and eye on the same side, and associated with pain during palpation of that ligament. The purpose of this study is to describe the clinical characteristics, treatment, and course of the disease in a series of patients with Ernest syndrome. Retrospective observational study covering the period from 1998 to 2008. We recorded patients' age, sex, duration of the disorder, and pain characteristics. All patients were injected with 40mg triamcinolone acetonide at the mandibular insertion of the stylomandibular ligament. The study included a total of 6 patients. Mean age was 40.3 years (range, 35-51). All of the subjects were women. Four patients had undergone lengthy dental treatments in the month prior to onset of the pain. The mean time between pain onset and first consultation was 23 months. The syndrome resolved completely in all cases after treatment, with a minimum follow-up period of 12 months. We analysed the clinical characteristics, treatment, and course of disease in 6 patients with Ernest syndrome. Correct diagnosis is the key to being able to provide proper treatment. This disorder is sometimes confused with other types of orofacial pain, and may therefore be more prevalent than the literature would indicate. Copyright © 2012 Sociedad Española de Neurología. Published by Elsevier Espana. All rights reserved.

  7. Budd-Chiari Syndrome in a Patient with Hepatitis C

    Directory of Open Access Journals (Sweden)

    Joseph Frankl

    2016-01-01

    Full Text Available Chronic Budd-Chiari syndrome can present with cirrhosis and signs and symptoms similar to those of other chronic liver diseases. We present a case of Budd-Chiari syndrome discovered during attempted transjugular intrahepatic portosystemic shunting in a patient with decompensated cirrhosis believed to be secondary to hepatitis C. Although the patient had hepatocellular carcinoma, the Budd-Chiari syndrome was a primary disease due to hepatic venous webs. Angioplasty was performed in this case, which resolved the patient’s symptoms related to portal hypertension. Follow-up venography 5 months after angioplasty demonstrated continued patency of the hepatic veins. A biopsy was obtained in the same setting, which showed centrilobular fibrosis indicating that venous occlusion was indeed the cause of cirrhosis. It is important to consider a second disease when treating a patient with difficult to manage portal hypertension.

  8. What's in a Gene? Pseudoexfoliation Syndrome and Pigment Dispersion Syndrome in the Same Patient.

    Science.gov (United States)

    Pokrovskaya, Olya; O'Brien, Colm

    2016-01-01

    Pseudoexfoliation syndrome (PXS) and pigment dispersion syndrome (PDS) are two of the commonest disorders to produce secondary open-angle glaucoma through trabecular meshwork blockage. Each is a defined clinical entity, and while genetics likely play a significant role in the pathogenesis of both, the specific genes involved appear to be distinct. There is surprisingly little published in the literature regarding the coexistence of PDS and PXS in the same patient. We present the intriguing case of a patient who developed PDS in one eye and PXS in the other. This unusual case acts as a platform for an interesting discussion of the genomics of PXS and PDS.

  9. What's in a Gene Pseudoexfoliation Syndrome and Pigment Dispersion Syndrome in the Same Patient

    Directory of Open Access Journals (Sweden)

    Olya Pokrovskaya

    2016-01-01

    Full Text Available Pseudoexfoliation syndrome (PXS and pigment dispersion syndrome (PDS are two of the commonest disorders to produce secondary open-angle glaucoma through trabecular meshwork blockage. Each is a defined clinical entity, and while genetics likely play a significant role in the pathogenesis of both, the specific genes involved appear to be distinct. There is surprisingly little published in the literature regarding the coexistence of PDS and PXS in the same patient. We present the intriguing case of a patient who developed PDS in one eye and PXS in the other. This unusual case acts as a platform for an interesting discussion of the genomics of PXS and PDS.

  10. Lower tidal volume strategy (≈3 ml/kg) combined with extracorporeal CO2 removal versus 'conventional' protective ventilation (6 ml/kg) in severe ARDS: the prospective randomized Xtravent-study.

    Science.gov (United States)

    Bein, Thomas; Weber-Carstens, Steffen; Goldmann, Anton; Müller, Thomas; Staudinger, Thomas; Brederlau, Jörg; Muellenbach, Ralf; Dembinski, Rolf; Graf, Bernhard M; Wewalka, Marlene; Philipp, Alois; Wernecke, Klaus-Dieter; Lubnow, Matthias; Slutsky, Arthur S

    2013-05-01

    Acute respiratory distress syndrome is characterized by damage to the lung caused by various insults, including ventilation itself, and tidal hyperinflation can lead to ventilator induced lung injury (VILI). We investigated the effects of a low tidal volume (V(T)) strategy (V(T) ≈ 3 ml/kg/predicted body weight [PBW]) using pumpless extracorporeal lung assist in established ARDS. Seventy-nine patients were enrolled after a 'stabilization period' (24 h with optimized therapy and high PEEP). They were randomly assigned to receive a low V(T) ventilation (≈3 ml/kg) combined with extracorporeal CO2 elimination, or to a ARDSNet strategy (≈6 ml/kg) without the extracorporeal device. The primary outcome was the 28-days and 60-days ventilator-free days (VFD). Secondary outcome parameters were respiratory mechanics, gas exchange, analgesic/sedation use, complications and hospital mortality. Ventilation with very low V(T)'s was easy to implement with extracorporeal CO2-removal. VFD's within 60 days were not different between the study group (33.2 ± 20) and the control group (29.2 ± 21, p = 0.469), but in more hypoxemic patients (PaO2/FIO2 ≤150) a post hoc analysis demonstrated significant improved VFD-60 in study patients (40.9 ± 12.8) compared to control (28.2 ± 16.4, p = 0.033). The mortality rate was low (16.5%) and did not differ between groups. The use of very low V(T) combined with extracorporeal CO2 removal has the potential to further reduce VILI compared with a 'normal' lung protective management. Whether this strategy will improve survival in ARDS patients remains to be determined (Clinical trials NCT 00538928).

  11. Frequency of metabolic syndrome in patients with type-2 diabetes

    International Nuclear Information System (INIS)

    Ahmed, N.; Ahmad, T.; Hussain, S.J.; Javed, M.

    2010-01-01

    Background: Diabetes, Hypertension, Obesity and Ischaemic Heart Disease have become a problem of public health magnitude with substantial economic burden both in the developed as well as the developing countries. Obesity is quite frequent in Type 2 diabetics and also plays a central role in causing Metabolic Syndrome (MetS). Metabolic Syndrome significantly increases the incidence of cardiovascular complications. This study was done to determine the frequency of MetS in our Type 2 diabetic patients as most of the components of MetS can be modified and identifying/managing these at an early stage might be of considerable help in reducing cardiovascular complications. Methods: This cross-sectional study was done in Medical B and Medical A wards of Ayub Teaching Hospital, Abbottabad from Nov, 08 to April, 09. Type 2 Diabetic patients aged above 40 years who gave informed consent were included in the study. Data was collected through a structured proforma. Frequency of Metabolic Syndrome was estimated according to the IDF consensus worldwide definition of the MetS. Results: Of the 100 patients enrolled in this study 56 were females and 44 were males with a mean age of 59.9 years. Out of these 100 participants seventy six (76%) were diagnosed to have metabolic syndrome. Of the 56 females, forty eight (85.71%) were having metabolic syndrome while twenty eight (63.63%) of the 44 male participants were having the syndrome. The difference was statistically significant (p<0.05). Conclusion: Frequency of MetS was found to be significantly high in this study with female preponderance. All the components, except Hypertension were more frequent in females. Diabetic patients with metabolic syndrome need more aggressive approach in management so as to decrease the incidence of cardiovascular complications. (author)

  12. Turner Syndrome: Care of the Patient: Birth to Late Adolescence.

    Science.gov (United States)

    Paolucci, Denise Gruccio; Bamba, Vaneeta

    2017-06-01

    Turner syndrome (TS) is a genetic condition occurring in females resulting from the loss of part or all of one of the X chromosomes. The two hallmark features of Turner syndrome include short stature and primary ovarian insufficiency. In addition, Turner syndrome can involve multiple healthcare issues including cardiac and renal anomalies, autoimmune disorders, hearing loss, ophthalmologic issues, bone anomalies, dermatologic issues and psychosocial and educational concerns. The presenting signs of Turner syndrome can vary markedly, leading to delayed or even missed diagnosis. Early identification of TS allows for appropriate screening and surveillance evaluations and more timely treatment intervention. This article will provide an overview of the healthcare issues common to patients with TS, treatments available and the screening and surveillance testing that is recommended. Copyright© of YS Medical Media ltd.

  13. Assessment of Sexual Dysfunction in Patients with Fibromyalgia Syndrome

    Directory of Open Access Journals (Sweden)

    Rahime Nur Ülker

    2013-12-01

    Full Text Available Objectives: The aim of our study is to determine the presence, quality of sexual dysfunction in patients with fibromyalgia syndrome and to compare with normal population. Material and Methods: A total of 55 sexually active women who were admitted to Department of Physical Therapy and Rehabilitation of Antalya Research and Training Hospital and diagnosed with fibromyalgia syndrome according to 1990 and 2010 American College of Rheumatology (ACR criteria. A control group composed of 50 sexually active women who were admitted to our clinic with various musculoskeletal system complaints were also included in the study in order to compare the parameters used for clinical assessment of patients and to determine whether the patients differ from normal population. Patients and controls who met inclusion criteria were applied Female Sexual Function Index (FSFI for assessment of sexual function. This test was developed by Rosen and colleagues in 2000, it is composed of 19 questions and inquires six different dimensions including desire, arousal, lubrication, orgasm, sexual satisfaction and pain. Turkish validation test was done by Turkish Society Of Andrology in 2003, answers are multiplied with a coefficient and each section is evaluated on six scores. Minimum score is 2.4 and maximum is 36 and standardly used for assessment of female sexual dysfunction in Turkey. Results: Subscale and total score of Female Sexual Function Index of Fibromyalgia syndrome patients were found statistically significantly lower than those of control group (p<0.05. Conclusion: Disorders of sexual function or its quality are one of the problems seen in fibromyalgia syndrome patients. It should be noticed that sexual function assessment must be a part of treatment of fibromyalgia syndrome. It is quite difficult to determine the mechanism between sexual dysfunction and fibromyalgia syndrome and new and larger studies are needed to determine this mechanism. (Turkish Journal of

  14. Adropin Levels in Polycystic Ovary Syndrome Patients

    OpenAIRE

    Hacer Sen

    2017-01-01

    Aim: Polycystic ovary syndrome (PCOS) is one of the most commonly observed endocrinopathies in women of reproductive age. Women with PCOS are said to have increased classic risk factors for cardiovascular disease, hypertension, dyslipidemia, diabetes, and obesity, in addition to non-classic risk factors such as an increase in C-reactive protein (CRP), homocysteine, and tumor necrosis factor-%u03B1. Adropin is a protein thought to play a role in maintaining energy homeostasis and insulin respo...

  15. Multiple giant cell lesions in patients with Noonan syndrome and cardio-facio-cutaneous syndrome

    Science.gov (United States)

    Neumann, Thomas E; Allanson, Judith; Kavamura, Ines; Kerr, Bronwyn; Neri, Giovanni; Noonan, Jacqueline; Cordeddu, Viviana; Gibson, Kate; Tzschach, Andreas; Krüger, Gabriele; Hoeltzenbein, Maria; Goecke, Timm O; Kehl, Hans Gerd; Albrecht, Beate; Luczak, Klaudiusz; Sasiadek, Maria M; Musante, Luciana; Laurie, Rohan; Peters, Hartmut; Tartaglia, Marco; Zenker, Martin; Kalscheuer, Vera

    2009-01-01

    Noonan syndrome (NS) and cardio-facio-cutaneous syndrome (CFCS) are related developmental disorders caused by mutations in genes encoding various components of the RAS-MAPK signaling cascade. NS is associated with mutations in the genes PTPN11, SOS1, RAF1, or KRAS, whereas CFCS can be caused by mutations in BRAF, MEK1, MEK2, or KRAS. The NS phenotype is rarely accompanied by multiple giant cell lesions (MGCL) of the jaw (Noonan-like/MGCL syndrome (NL/MGCLS)). PTPN11 mutations are the only genetic abnormalities reported so far in some patients with NL/MGCLS and in one individual with LEOPARD syndrome and MGCL. In a cohort of 75 NS patients previously tested negative for mutations in PTPN11 and KRAS, we detected SOS1 mutations in 11 individuals, four of whom had MGCL. To explore further the relevance of aberrant RAS-MAPK signaling in syndromic MGCL, we analyzed the established genes causing CFCS in three subjects with MGCL associated with a phenotype fitting CFCS. Mutations in BRAF or MEK1 were identified in these patients. All mutations detected in these seven patients with syndromic MGCL had previously been described in NS or CFCS without apparent MGCL. This study demonstrates that MGCL may occur in NS and CFCS with various underlying genetic alterations and no obvious genotype–phenotype correlation. This suggests that dysregulation of the RAS-MAPK pathway represents the common and basic molecular event predisposing to giant cell lesion formation in patients with NS and CFCS rather than specific mutation effects. PMID:18854871

  16. Multiple giant cell lesions in patients with Noonan syndrome and cardio-facio-cutaneous syndrome.

    Science.gov (United States)

    Neumann, Thomas E; Allanson, Judith; Kavamura, Ines; Kerr, Bronwyn; Neri, Giovanni; Noonan, Jacqueline; Cordeddu, Viviana; Gibson, Kate; Tzschach, Andreas; Krüger, Gabriele; Hoeltzenbein, Maria; Goecke, Timm O; Kehl, Hans Gerd; Albrecht, Beate; Luczak, Klaudiusz; Sasiadek, Maria M; Musante, Luciana; Laurie, Rohan; Peters, Hartmut; Tartaglia, Marco; Zenker, Martin; Kalscheuer, Vera

    2009-04-01

    Noonan syndrome (NS) and cardio-facio-cutaneous syndrome (CFCS) are related developmental disorders caused by mutations in genes encoding various components of the RAS-MAPK signaling cascade. NS is associated with mutations in the genes PTPN11, SOS1, RAF1, or KRAS, whereas CFCS can be caused by mutations in BRAF, MEK1, MEK2, or KRAS. The NS phenotype is rarely accompanied by multiple giant cell lesions (MGCL) of the jaw (Noonan-like/MGCL syndrome (NL/MGCLS)). PTPN11 mutations are the only genetic abnormalities reported so far in some patients with NL/MGCLS and in one individual with LEOPARD syndrome and MGCL. In a cohort of 75 NS patients previously tested negative for mutations in PTPN11 and KRAS, we detected SOS1 mutations in 11 individuals, four of whom had MGCL. To explore further the relevance of aberrant RAS-MAPK signaling in syndromic MGCL, we analyzed the established genes causing CFCS in three subjects with MGCL associated with a phenotype fitting CFCS. Mutations in BRAF or MEK1 were identified in these patients. All mutations detected in these seven patients with syndromic MGCL had previously been described in NS or CFCS without apparent MGCL. This study demonstrates that MGCL may occur in NS and CFCS with various underlying genetic alterations and no obvious genotype-phenotype correlation. This suggests that dysregulation of the RAS-MAPK pathway represents the common and basic molecular event predisposing to giant cell lesion formation in patients with NS and CFCS rather than specific mutation effects.

  17. Frailty syndrome in patients with heart rhythm disorders.

    Science.gov (United States)

    Mlynarska, Agnieszka; Mlynarski, Rafal; Golba, Krzysztof S

    2017-09-01

    To assess the prevalence of frailty syndrome in patients with heart rhythm disorders that qualified for pacemaker implantation. The study included 171 patients (83 women, aged 73.9 ± 6.7 years) who qualified for pacemaker implantation as a result of sinus node dysfunction (81 patients) or atrio-ventricular blocks (AVB; 90 patients). A total of 60 patients (25 women, aged 72.40 ± 7.09 years) without heart rhythm disorders were included in the control group. Frailty syndrome was diagnosed using the Canadian Study of Health and Aging Clinical Frailty Scale test. Frailty syndrome was diagnosed in 25.15% of the patients, and pre-frailty in 36.84% of the patients. Frailty syndrome was diagnosed in 10% of the control group, and the average value of frailty was 3.35 ± 0.92. Frailty occurred significantly more often among patients with AVB (33.34%) compared with patients who were diagnosed with sinus node dysfunction (16.05%); P = 0.0081. The average score of frailty for sinus node dysfunction was 3.71 ± 0.89, and for AVB it was 4.14 ± 0.93; P = 0.0152. In the case of AVB, the women had a statistically more intense level of frailty of 4.54 ± 0.90 as compared with the men 3.87 ± 0.85; P = 0.0294. In the multiple logistic analysis, the presence of any arrhythmia was strongly associated with frailty syndrome (OR 2.1286, 95% CI 1.4594 - 3.1049; P = 0.0001). Frailty syndrome was diagnosed in one-quarter of patients with cardiac arrhythmias, whereas a further 40% were at a higher risk of frailty syndrome, and its occurrence was significantly higher if compared with the control group. Frailty occurred significantly more often among patients with atrio-ventricular blocks, especially in women. The results of the present research showed that there is a statistical association between frailty and arrhythmias. Geriatr Gerontol Int 2017; 17: 1313-1318. © 2016 Japan Geriatrics Society.

  18. Cardiac computed tomography in patients with acute coronary syndrome

    International Nuclear Information System (INIS)

    Schlett, C.L.; Bamberg, F.

    2014-01-01

    Currently, cardiac computed tomography (CT) is increasingly being implemented into clinical algorithms, primarily due to substantial technical advances over the last decade. Its use in the setting of suspected acute coronary syndrome is of particular relevance, given the high degree of accumulating scientific evidence of improving patient outcomes. Performing cardiac CT requires specific knowledge on the available scan acquisitions and patient preparation. Also, expertise is required in order to interpret the coronary and extra-coronary findings adequately. The present article provides an overview of the different aspects on the use of cardiac CT in the setting of acute coronary syndrome.

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

    Science.gov (United States)

    Gamble, John F; Kurian, Dinesh J; Udani, Andrea G; Greene, Nathaniel H

    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.

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

    Directory of Open Access Journals (Sweden)

    John F. Gamble

    2016-01-01

    Full Text Available 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.

  1. Chickenpox ARDS in a health care worker following occupational exposure.

    LENUS (Irish Health Repository)

    Knaggs, A

    2012-02-03

    A case is described of chickenpox acute respiratory distress syndrome in an ambulance driver after the inter-hospital transfer of a patient known to have chickenpox pneumonia. Following this exposure, he neither avoided patient contact nor received varicella zoster immune globulin. He subsequently required 13 days of ventilatory support before making a full recovery. The case described supports the contention that health care workers should be screened by serology for immunity to chickenpox before patient contact occurs, with subsequent vaccination of those who are non-immune, when the vaccine becomes available.

  2. Prevalence of dry eye syndrome and Sjogren's syndrome in patients with rheumatoid arthritis.

    Science.gov (United States)

    Kosrirukvongs, Panida; Ngowyutagon, Panotsom; Pusuwan, Pawana; Koolvisoot, Ajchara; Nilganuwong, Surasak

    2012-04-01

    Rheumatoid arthritis has manifestations in various organs including ophthalmic involvement. The present study evaluates prevalence of dry eye and secondary Sjogren's syndrome using salivary scintigraphy which has not been used in previous reports. To evaluate the prevalence of secondary Sjogren's syndrome in patients with rheumatoid arthritis, including clinical characteristics and dry eye, compared with non-Sjogren's syndrome. Descriptive cross sectional study Sixty-one patients with rheumatoid arthritis were recruited at Siriraj Hospital during March 2009-September 2010 and filled in the questionnaires about dry eye for Ocular Surface Disease Index (OSDI) with a history taking of associated diseases, medications, duration of symptoms of dry eyes and dry mouth. The Schirmer I test without anesthesia, tear break-up time, rose bengal staining score, severity of keratitis and salivary scintigraphy were measured and analyzed. Prevalence of secondary Sjogren's syndrome and dry eye were 22.2% (95% CI 15.4 to 30.9) and 46.7% (95% CI 38.0 to 55.6), respectively. Dry eye interpreted from OSDI, Schirmer 1 test, tear break-up time and rose bengal staining was 16.4%, 46.7%, 82% and 3.3% respectively. Fifty-two percent of patients had a history of dry eye and dry mouth with mean duration 27.4 and 29.8 months, respectively. Superficial punctate keratitis and abnormal salivary scintigraphy were found in 58.2% and 77.8%. Duration of rheumatoid arthritis, erythrocyte sedimentation rate were not correlated with secondary Sjogren's syndrome. Dry eye from OSDI with secondary Sjogren's syndrome (33.3%) compared with non-Sjogren's syndrome (9.5%) was significant difference (p = 0.008). Adjusted odds ratio for secondary Sjogren's syndrome in OSDIL score > 25 was 13.8 (95% CI 2.6 to 73.8, p = 0.002) compared to OSDI score dry eye syndrome and secondary Sjogren's syndrome in rheumatoid arthritis was crucial for evaluation of their severity and proper management.

  3. Sensory Testing in Patients With Postthoracotomy Pain Syndrome

    DEFF Research Database (Denmark)

    Werner, Mads Utke; Ringsted, Thomas K; Kehlet, Henrik

    2013-01-01

    pain syndrome [PTPS (n=14)]. The primary outcome was investigation of the areas of sensory dysfunction, evaluated twice by dynamic sensory mapping with metal rollers and a brush. RESULTS:: In PTPS patients, sensory dysfunction was present on the surgical side, and in 12 of 14 patients MISD......OBJECTIVES:: Mirror-image sensory dysfunction (MISD) has not been systematically characterized in persistent postoperative pain. METHODS:: The presence of MISD was evaluated with standardized stimuli, in preoperative patients scheduled for a thoracotomy (n=14) and in patients with postthoracotomy...... of the PTPS patients experienced mirror pain. DISCUSSION:: MISD is a common finding in PTPS patients and deserves further study involving mechanism and clinical implications....

  4. Computed tomography in patients with Ehlers-Danlos syndrome

    International Nuclear Information System (INIS)

    Hagino, Hiroshi; Sugitani, Akitoshi; Eda, Isematsu; Takashima, Sachio; Takeshita, Kenzo

    1985-01-01

    Three patients with Ehlers-Danlos syndrome were reported. Unusual findings on computed tomography were seen in two of the three patients. One case showed peculiar and marked dilatation of the 4th ventricle, supracerebellar cistern and lateral ventricle. The other case presented disproportionate enlargement of the anterior horn of the lateral ventricle. These CT findings in the two patients suggest that developmental abnormalities may constitute a structural defect. (orig.)

  5. Computed tomography in patients with Ehlers-Danlos syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Hagino, Hiroshi; Sugitani, Akitoshi; Eda, Isematsu; Takashima, Sachio; Takeshita, Kenzo

    1985-09-01

    Three patients with Ehlers-Danlos syndrome were reported. Unusual findings on computed tomography were seen in two of the three patients. One case showed peculiar and marked dilatation of the 4th ventricle, supracerebellar cistern and lateral ventricle. The other case presented disproportionate enlargement of the anterior horn of the lateral ventricle. These CT findings in the two patients suggest that developmental abnormalities may constitute a structural defect.

  6. Congenital heart defects in molecularly proven Kabuki syndrome patients.

    Science.gov (United States)

    Digilio, Maria Cristina; Gnazzo, Maria; Lepri, Francesca; Dentici, Maria Lisa; Pisaneschi, Elisa; Baban, Anwar; Passarelli, Chiara; Capolino, Rossella; Angioni, Adriano; Novelli, Antonio; Marino, Bruno; Dallapiccola, Bruno

    2017-11-01

    The prevalence of congenital heart defects (CHD) in Kabuki syndrome ranges from 28% to 80%. Between January 2012 and December 2015, 28 patients had a molecularly proven diagnosis of Kabuki syndrome. Pathogenic variants in KMT2D (MLL2) were detected in 27 patients, and in KDM6A gene in one. CHD was diagnosed in 19/27 (70%) patients with KMT2D (MLL2) variant, while the single patient with KDM6A change had a normal heart. The anatomic types among patients with CHD included aortic coarctation (4/19 = 21%) alone or associated with an additional CHD, bicuspid aortic valve (4/19 = 21%) alone or associated with an additional CHD, perimembranous subaortic ventricular septal defect (3/19 = 16%), atrial septal defect ostium secundum type (3/19 = 16%), conotruncal heart defects (3/19 = 16%). Additional CHDs diagnosed in single patients included aortic dilatation with mitral anomaly and hypoplastic left heart syndrome. We also reviewed CHDs in patients with a molecular diagnosis of Kabuki syndrome reported in the literature. In conclusion, a CHD is detected in 70% of patients with KMT2D (MLL2) pathogenic variants, most commonly left-sided obstructive lesions, including multiple left-sided obstructions similar to those observed in the spectrum of the Shone complex, and septal defects. Clinical management of Kabuki syndrome should include echocardiogram at the time of diagnosis, with particular attention to left-sided obstructive lesions and mitral anomalies, and annual monitoring for aortic arch dilatation. © 2017 Wiley Periodicals, Inc.

  7. Mortality in Pediatric Acute Respiratory Distress Syndrome: A Systematic Review and Meta-Analysis.

    Science.gov (United States)

    Wong, Judith Ju-Ming; Jit, Mark; Sultana, Rehena; Mok, Yee Hui; Yeo, Joo Guan; Koh, Jia Wen Janine Cynthia; Loh, Tsee Foong; Lee, Jan Hau

    2017-01-01

    Sparse and conflicting evidence exists regarding mortality risk from pediatric acute respiratory distress syndrome (ARDS). We aimed to determine the pooled mortality in pediatric ARDS and to describe its trend over time. MEDLINE, EMBASE, and Web of Science were searched from 1960 to August 2015. Keywords or medical subject headings (MESH) terms used included "respiratory distress syndrome, adult," "acute lung injury," "acute respiratory insufficiency," "acute hypoxemic respiratory failure," "pediatrics," and "child." Study inclusion criteria were (1) pediatric patients aged 0 days to 18 years, (2) sufficient baseline data described in the pediatric ARDS group, and (3) mortality data. Randomized controlled trials (RCTs) and prospective observational studies were eligible. Data on study characteristics, patient demographics, measures of oxygenation, and mortality were extracted using a standard data extraction form. Independent authors conducted the search, applied the selection criteria, and extracted the data. Methodological quality of studies was assessed. Meta-analysis using a random-effects model was performed to obtain pooled estimates of mortality. Meta-regression was performed to analyze variables contributing to change in mortality over time. Eight RCTs and 21 observational studies (n = 2274 patients) were included. Pooled mortality rate was 24% (95% confidence interval [CI]: 19-31). There was a decrease in mortality rates over 3 epochs (≤2000, 2001-2009, and ≥2010: 40% [95% CI: 24-59], 35% [95% CI: 21-51], and 18% [95% CI: 12-26], respectively, P < .001). Observational studies reported a higher mortality rate than RCTs (27% [95% CI: 24-29] versus 16% [95% CI: 12-20], P < .001). Earlier year of publication was an independent factor associated with mortality. Overall mortality rate in pediatric ARDS is approximately 24%. Studies conducted and published later were associated with better survival.

  8. Reduced apolipoprotein glycosylation in patients with the metabolic syndrome.

    Directory of Open Access Journals (Sweden)

    Olga V Savinova

    Full Text Available The purpose of this study was to compare the apolipoprotein composition of the three major lipoprotein classes in patients with metabolic syndrome to healthy controls.Very low density (VLDL, intermediate/low density (IDL/LDL, hereafter LDL, and high density lipoproteins (HDL fractions were isolated from plasma of 56 metabolic syndrome subjects and from 14 age-sex matched healthy volunteers. The apolipoprotein content of fractions was analyzed by one-dimensional (1D gel electrophoresis with confirmation by a combination of mass spectrometry and biochemical assays.Metabolic syndrome patients differed from healthy controls in the following ways: (1 total plasma--apoA1 was lower, whereas apoB, apoC2, apoC3, and apoE were higher; (2 VLDL--apoB, apoC3, and apoE were increased; (3 LDL--apoC3 was increased, (4 HDL--associated constitutive serum amyloid A protein (SAA4 was reduced (p<0.05 vs. controls for all. In patients with metabolic syndrome, the most extensively glycosylated (di-sialylated isoform of apoC3 was reduced in VLDL, LDL, and HDL fractions by 17%, 30%, and 25%, respectively (p<0.01 vs. controls for all. Similarly, the glycosylated isoform of apoE was reduced in VLDL, LDL, and HDL fractions by 15%, 26%, and 37% (p<0.01 vs. controls for all. Finally, glycosylated isoform of SAA4 in HDL fraction was 42% lower in patients with metabolic syndrome compared with controls (p<0.001.Patients with metabolic syndrome displayed several changes in plasma apolipoprotein composition consistent with hypertriglyceridemia and low HDL cholesterol levels. Reduced glycosylation of apoC3, apoE and SAA4 are novel findings, the pathophysiological consequences of which remain to be determined.

  9. Features of Turner syndrome among a group of Cameroonian patients.

    Science.gov (United States)

    Wonkam, Ambroise; Veigne, Sandra W; Abass, Ali; Ngo Um, Suzanne; Noubiap, Jean Jacques N; Mbanya, Jean-Claude; Sobngwi, Eugene

    2015-06-01

    To describe the features of Turner syndrome among a group of Cameroonian patients. A descriptive cross-sectional study was conducted among patients with amenorrhea and/or short stature who attended the genetic unit of Yaoundé Gynecology, Obstetrics and Pediatric Hospital (Yaoundé, Cameroon) for a specialist consultation between July 1, 2007, and December 31, 2008. Sociodemographic, clinical, and cytogenetic data were collected. Turner syndrome was confirmed among 11 of the 14 participants (seven had monosomy of the X chromosome; four had mosaicism involving a structural abnormality of the second X chromosome). The mean age at diagnosis was 18.4±2.8years. The reasons for consultation were delayed puberty (n=10) and short stature (n=1). Nine patients had a short neck, nine had a forearm carrying-angle deformity, eight had a low hairline, and two had a webbed neck. Abdominal ultrasonography identified a horseshoe kidney in two patients and a rudimentary uterus in nine patients. None of the patients displayed cardiac abnormalities. Hypergonadotropic hypogonadism was reported among five patients. Eight patients did not receive hormonal treatment owing to advanced bone age or economic reasons. Late diagnosis and variable phenotypic expression were key features of Cameroonian patients with Turner syndrome. Copyright © 2015 International Federation of Gynecology and Obstetrics. Published by Elsevier Ireland Ltd. All rights reserved.

  10. A patient with Werner syndrome and adiponectin gene mutation.

    Science.gov (United States)

    Hashimoto, Naotake; Hatanaka, Sachiko; Yokote, Koutaro; Kurosawa, Hiroko; Yoshida, Tomohiko; Iwai, Rie; Takahashi, Hidenori; Yoshida, Katsuya; Horie, Atsuya; Sakurai, Kenichi; Yagui, Kazuo; Saito, Yasushi; Yoshida, Shouji

    2007-01-01

    Werner syndrome is a premature aging disease characterized by genomic instability and increased cancer risk. Here, we report a 45-year-old diabetic man as the first Werner syndrome patient found to have an adiponectin gene mutation. Showing graying and loss of hair, skin atrophy, and juvenile cataract, he was diagnosed with Werner syndrome type 4 by molecular analysis. His serum adiponectin concentration was low. In the globular domain of the adiponectin gene, I164T in exon 3 was detected. When we examined effects of pioglitazone (15 mg/day) on serum adiponectin multimer and monomer concentrations using selective assays, the patient's relative percentage increased in adiponectin concentration was almost same as that in the 18 diabetic patients without an adiponectin mutation, but the absolute adiponectin concentration was half of those seen in diabetic patients treated with the same pioglitazone dose who had no adiponectin mutation. The response suggested that pioglitazone treatment might help to prevent future Werner syndrome-related acceleration of atherosclerosis. Present and further clinical relevant to atherosclerosis in this patient should be imformative concerning the pathogenesis and treatment of atherosclerosis in the presence of hypoadiponectinemia and insulin resistance.

  11. Total Endovascular Aortic Repair in a Patient with Marfan Syndrome.

    Science.gov (United States)

    Amako, Mau; Spear, Rafaëlle; Clough, Rachel E; Hertault, Adrien; Azzaoui, Richard; Martin-Gonzalez, Teresa; Sobocinski, Jonathan; Haulon, Stéphan

    2017-02-01

    The aim of this study is to describe a total endovascular aortic repair with branched and fenestrated endografts in a young patient with Marfan syndrome and a chronic aortic dissection. Open surgery is the gold standard to treat aortic dissections in patients with aortic disease and Marfan syndrome. In 2000, a 38-year-old man with Marfan syndrome underwent open ascending aorta repair for an acute type A aortic dissection. One year later, a redo sternotomy was performed for aortic valve replacement. In 2013, the patient presented with endocarditis and pulmonary infection, which necessitated tracheostomy and temporary dialysis. In 2014, the first stage of the endovascular repair was performed using an inner branched endograft to exclude a 77-mm distal arch and descending thoracic aortic aneurysm. In 2015, a 63-mm thoracoabdominal aortic aneurysm was excluded by implantation of a 4-fenestrated endograft. Follow-up after both endovascular repairs was uneventful. Total aortic endovascular repair was successfully performed to treat a patient with arch and thoraco-abdominal aortic aneurysm associated with chronic aortic dissection and Marfan syndrome. The postoperative images confirmed patency of the endograft and its branches, and complete exclusion of the aortic false lumen. Endovascular repair is a treatment option in patients with connective tissue disease who are not candidates for open surgery. Long-term follow-up is required to confirm these favorable early outcomes. Copyright © 2016 Elsevier Inc. All rights reserved.

  12. Characterization of thrombosis in patients with Proteus syndrome.

    Science.gov (United States)

    Keppler-Noreuil, Kim M; Lozier, Jay N; Sapp, Julie C; Biesecker, Leslie G

    2017-09-01

    Patients with overgrowth and complex vascular malformation syndromes, including Proteus syndrome have an increased risk of thromboembolism. Proteus syndrome is a mosaic, progressive overgrowth disorder involving vasculature, skin, and skeleton, and caused by a somatic activating mutation in AKT1. We conducted a comprehensive review of the medical histories and hematologic evaluations of 57 patients with Proteus syndrome to identify potential risk factors for thrombosis. We found that six of ten patients, who were deceased, died secondary to deep venous thrombosis and/or pulmonary embolism. Of the remaining 47 living patients, six had thromboembolic events that all occurred postoperatively and in an affected limb. Eleven of 21 patients had an abnormal hypercoagulable panel including Factor V Leiden heterozygotes, antithrombin III deficiency, positive lupus anticoagulant, or Protein C or S deficiencies. We observed that eight of 17 patients had an abnormal D-dimer level >0.5 mcg/dl, but deep venous thromboses occurred in only four of those with D-dimer >1.0 mcg/dl. We conclude that the predisposition to thrombosis is likely to be multifaceted with risk factors including vascular malformations, immobility, surgery, additional prothrombotic factors, and possible pathophysiologic effects of the somatic AKT1 mutation on platelet function or the vascular endothelium. The D-dimer test is useful as a screen for thromboembolism, although the screening threshold may need to be adjusted for patients with this disorder. We propose developing a registry to collect D-dimer and outcome data to facilitate adjustment of the D-dimer threshold for Proteus syndrome and related disorders, including PIK3CA-Related Overgrowth Spectrum. © 2017 Wiley Periodicals, Inc.

  13. Quantitative Sensory Testing in Patients With Postthoracotomy Pain Syndrome

    DEFF Research Database (Denmark)

    Wildgaard, Kim; Ringsted, Thomas K; Kehlet, Henrik

    2013-01-01

    -retest variability of thermal thresholds in patients (n=14) with the postthoracotomy pain syndrome. METHODS:: Sensory mapping with a metal roller (25°C) on the surgical side delineated an area with cool sensory dysfunction. In this area and in a contralateral area, 4 prespecified sites (2.6 cm) were outlined...... was demonstrated, the much lower within-patient than between-patient variances facilitated estimations of highly statistical significant, within-patient differences in thermal thresholds. DISCUSSION:: In patients with postthoracotomy pain syndrome, several statistical methods indicated an excessively high......, in addition to the maximum pain site on the surgical side. In these total 9 sites, warmth detection threshold, cool detection threshold, and heat pain threshold were assessed. RESULTS:: Comparisons of thermal test-retest assessments did not demonstrate any significant intraside differences. The SDs...

  14. A Brazilian cohort of patients with Tourette's syndrome.

    Science.gov (United States)

    Cardoso, F; Veado, C C; de Oliveira, J T

    1996-01-01

    The clinical features of 32 patients (24 males) with Tourette's syndrome in Brazil were studied. The mean age at onset was 7.1 years, tics being the first symptom in 71% and hyperactivity in 29%. Blinking, grimacing, and shoulder elevation were the most common motor tics and sniffing, throat clearing, and grunting noises, the most frequent vocal tics. Coprolalia was present in 28%, echolalia in 16%, palilalia in 9%, and copropraxia in 25% of patients. Attention deficit and hyperactivity disorder was diagnosed in 63%, and obsessive compulsive behaviour in 44% of patients. In 84% of patients there was a family history of tics whereas attention deficit and hyperactivity disorder and obsessive compulsive behaviour were respectively present in relatives of 19% and 53% of the patients studied. These data suggest that Tourette's syndrome in Brazil is not clinically different from other countries, supporting the notion that genetic factors play the most important part in its aetiology. PMID:8708658

  15. Pathophysiology and Management of Acute Respiratory Distress Syndrome in Children.

    Science.gov (United States)

    Heidemann, Sabrina M; Nair, Alison; Bulut, Yonca; Sapru, Anil

    2017-10-01

    Acute respiratory distress syndrome (ARDS) is a syndrome of noncardiogenic pulmonary edema and hypoxia that accompanies up to 30% of deaths in pediatric intensive care units. Pediatric ARDS (PARDS) is diagnosed by the presence of hypoxia, defined by oxygenation index or Pao 2 /Fio 2 ratio cutoffs, and new chest infiltrate occurring within 7 days of a known insult. Hallmarks of ARDS include hypoxemia and decreased lung compliance, increased work of breathing, and impaired gas exchange. Mortality is often accompanied by multiple organ failure. Although many modalities to treat PARDS have been investigated, supportive therapies and lung protective ventilator support remain the mainstay. Copyright © 2017 Elsevier Inc. All rights reserved.

  16. CHOROIDAL MELANOMA IN A PATIENT WITH WAARDENBURG SYNDROME.

    Science.gov (United States)

    Itty, Sujit; Richter, Elizabeth R; McCannel, Tara A

    2015-01-01

    To report a case of choroidal malignant melanoma in a patient with Waardenburg syndrome and bilateral choroidal pigmentary abnormalities. Clinical examination and multimodal imaging of the case. A 45-year-old woman presented with asymptomatic flat choroidal pigmentation abnormalities in both eyes. A choroidal lesion was identified in the inferotemporal periphery of the left eye arising from an area of hyperpigmentation; ultrasonography findings were consistent with a choroidal melanoma. The patient endorsed a personal and family history of premature graying of hair and was identified to have dystopia canthorum consistent with the diagnosis of Waardenburg syndrome. The authors present the first reported case of concurrent Waardenburg syndrome and choroidal malignant melanoma. This cooccurrence may suggest that the relative hyperpigmented regions in affected fundi may be abnormal and should be monitored closely for the development of choroidal melanoma.

  17. Respiratory problems in patients with ectodermal dysplasia syndromes.

    Science.gov (United States)

    Fete, Timothy

    2014-10-01

    The ectodermal dysplasias (EDs) are a heterogeneous group of disorders characterized by a deficiency of ectoderm- and mesoderm-derived tissues and appendages, particularly hair, skin, teeth, and nails. Many of these disorders are associated with a greater risk of respiratory disease than found in the general population. There are no published papers that comprehensively describe these findings and the possible etiologies. Patients have been reported with dramatic decrease in mucous glands in the respiratory tract. Anatomic defects, including cleft palate, that predispose to respiratory infection, are associated with several of the ED syndromes. Atopy and immune deficiencies have been shown to have a higher prevalence in ED syndromes. Clinicians who care for patients affected by ED syndromes should be aware of the potential respiratory complications, and consider evaluation for structural anomalies, atopy and immunodeficiency in individuals with recurrent or chronic respiratory symptoms. © 2014 Wiley Periodicals, Inc.

  18. Prognosis of patients treated for Cushing syndrome.

    Science.gov (United States)

    Aulinas, Anna; Valassi, Elena; Webb, Susan M

    2014-01-01

    Cushing syndrome (CS), due to an ACTH-secreting pituitary adenoma, adrenal tumors, or ectopic ACTH secretion, causes hypercortisolism. CS is associated with major morbidity, especially metabolic and cardiovascular complications, osteoporosis, psychiatric changes, and cognitive impairment. Despite biochemical "cure" of hypercortisolism and clinical improvement after effective treatment, these complications are only partially reversible. Exacerbation of prior autoimmune diseases is also seen. All of these lead to quality of life impairment and increased mortality. This review addresses the main comorbidities and long-term consequences of CS despite clinical and biochemical "cure". Copyright © 2013 SEEN. Published by Elsevier Espana. All rights reserved.

  19. Primary myelodysplastic syndrome with complex chromosomal rearrangements in a patient with Klinefelter's syndrome.

    OpenAIRE

    Abidi, S M; Griffiths, M; Oscier, D G; Mufti, G J; Hamblin, T J

    1986-01-01

    A patient with Klinefelter's syndrome and diabetes mellitus was diagnosed as having myelodysplasia. Cytogenetic analysis of the peripheral blood and the bone marrow cells confirmed the presence of a constitutional 47,XXY chromosome complement. In addition, complex karyotypic abnormalities were present.

  20. Acute coronary syndrome in a patient with Marfan syndrome following emergent surgical repair of aortic dissection.

    Science.gov (United States)

    Bovolato, Francesca Elisa; Isabella, Giambattista; Rampazzo, Debora; Guglielmi, Cosimo; Gerosa, Gino; Iliceto, Sabino; Bilato, Claudio

    2008-06-01

    We report a case of acute coronary syndrome in a patient with suspect Marfan syndrome, 25 days after emergent modified Bentall-De Bono intervention for acute type I peripartum aortic dissection. She was admitted to our intensive care unit because of unstable angina, caused by critical blood flow reduction in a large portion of the myocardium, according to the severity of the symptoms and the electrocardiographic alterations. Coronary angiography showed a sub-occlusive stenosis of the left main coronary artery as a result of the dissection extension to the coronary ostium. Because of the high risk related to heart surgery, the patient was successfully treated by unprotected angioplasty and drug-eluting stent positioning. Short- and mid-term outcomes were favourable. Subsequent tests confirmed the diagnosis of Marfan syndrome. After 2 years of follow-up, the patient remains asymptomatic and in good health. To our knowledge, this is the first report of a successful percutaneous intervention of the left main coronary artery in a patient with Marfan syndrome who had already undergone ascending aortic root and valve replacement by the Bentall-De Bono procedure for acute dissection.

  1. Managing a patient with burning mouth syndrome

    Science.gov (United States)

    Cheung, Danny; Trudgill, Nigel

    2015-01-01

    A 64-year-old woman presented with an increasing frequency of symptoms of heartburn and retrosternal pain over the last few months, and a constant and intense burning pain affecting her tongue tip, mouth and lips for the past 5 years. She found consuming hot drinks exacerbated the burning oral pain and chewing gum seemed to alleviate some of her symptoms. She thought these oral sensations were caused by frequently licking her finger tips to separate prints in her work in publishing. She had been previously diagnosed with gastro-oesophageal reflux disease (GORD), and her heartburn symptoms had been controlled until recently with lansoprazole 15 mg daily. Her past medical history included irritable bowel syndrome and depression, for which she had been treated with mebeverine and paroxetine for a number of years. She was a non-smoker and did not consume alcohol. Clinical examination was unremarkable with no oral lesions on examination. Her routine laboratory tests, including autoimmune serology, haematinics and thyroid function tests were all within normal limits. She underwent a gastroscopy, which revealed moderate reflux oesophagitis, and following commencing omeprazole 20 mg twice daily, her heartburn resolved. However, her oral burning symptoms were not affected and a diagnosis of burning mouth syndrome (BMS) was made. Following explanation and reassurance concerning the cause of her BMS symptoms, she chose not to receive treatment for this but to access cognitive behavioural therapy in the future if her symptoms worsened. PMID:28839812

  2. Studies of microparticles in patients with the antiphospholipid syndrome (APS).

    Science.gov (United States)

    Vikerfors, A; Mobarrez, F; Bremme, K; Holmström, M; Ågren, A; Eelde, A; Bruzelius, M; Antovic, A; Wallén, H; Svenungsson, E

    2012-06-01

    To study circulating platelet, monocyte and endothelial microparticles (PMPs, MMPs and EMPs) in patients with antiphospholipid syndrome (APS) in comparison with healthy controls. Fifty-two patients with APS and 52 healthy controls were investigated. MPs were measured on a flow cytometer (Beckman Gallios) and defined as particles sized APS patients versus controls (p APS patients. We observed a high number of EMPs expressing TF in APS patients. The numbers of MMPs and total EMPs were also higher as compared with healthy controls but in contrast to previous reports, the number of PMPs did not differ between groups.

  3. Increased Risk of Metabolic Syndrome in Patients with Vitiligo.

    Science.gov (United States)

    Ataş, Hatice; Gönül, Müzeyyen

    2017-05-05

    Inflammatory and immune processes can be triggered in vitiligo due to a decreased number of melanocytes and their anti-inflammatory effects. Because of the systemic nature of vitiligo, metabolic abnormalities such as insulin resistance and lipid profile disturbances as well as skin involvement may be observed in vitiligo. To investigate the association between metabolic syndrome and vitiligo. Case-control study. The demographic, clinical and laboratory features in the subjects were compared according to presence of vitiligo and metabolic syndrome [patients (n=63) vs. gender-age matched controls (n=65) and metabolic syndrome positive (n=38) vs. negative (n=90)]. A logistic regression analysis was also used. We identified metabolic syndrome in 24 (38.1%) subjects with vitiligo and 14 (21.5%) subjects without vitiligo (p=0.04). Active vitiligo, segmental vitiligo, an increased duration of vitiligo and an increased percentage in the affected body surface area were determined to be independent predictors of metabolic syndrome [activity of vitiligo: p=0.012, OR (95% CI)=64.4 (2.5-1672); type of vitiligo: p=0.007, OR (95% CI)=215.1 (4.3-10725.8); duration of vitiligo: p=0.03, OR (95% CI)=1.4 (1.1-2.0); percentage of affected body surface area: p=0.07, OR (95% CI)=1.2 (0.98-1.5)]. The risk of developing metabolic syndrome is increased in patients with vitiligo. The poor clinical features of vitiligo, such as active, extended and segmental vitiligo with an increased duration of time, are independent predictors for developing metabolic syndrome.

  4. Metabolic syndrome in patients with ischemic heart disease

    International Nuclear Information System (INIS)

    Yasmin, S.; Naveed, T.; Shakoor, T.

    2008-01-01

    To determine the frequency of metabolic syndrome in patients with Ischemic Heart Disease (IHD). Cross-sectional, descriptive study. A total of 100 subjects with ischemic heart disease, fulfilling the inclusion criteria, were enrolled in the study. Demographic data (age and gender) and the 5 component conditions of the metabolic syndrome were noted. Subjects were physically assessed for the abdominal obesity, based on waist circumference. Fasting blood samples for glucose and lipid profile in first 24 hours after acute coronary insult were drawn and tested in central laboratory. Variables were processed for descriptive statistics. In this study population, 68% were male and 32% were female with mean age of 52 +-13.6 years in men and 56 +- 12.5 years in women. Frequency of metabolic syndrome was 32% in men and 28% in women. It increased with age. The highest rate of metabolic syndrome was in men diagnosed as STEMI (odds ratio: 3.39, 95% CI=1.36-8.41). Frequency of metabolic syndrome was high among the patients with IHD. It supports the potential for preventive efforts in persons with high-risk of IHD. (author)

  5. What are patients with Rett syndrome interested in?

    Science.gov (United States)

    Hirano, Daisuke; Taniguchi, Takamichi

    2018-02-01

    [Purpose] Rett syndrome is a severe neurodevelopmental disease; individuals typically have no verbal skills or purposeful hand movements. In clinical settings, knowledge of their interests would be helpful for therapy. Therefore, we investigated the interests of Rett syndrome patients. [Subjects and Methods] In 2016, we sent a questionnaire regarding the interests of individuals with Rett syndrome to 1,016 directors of schools for special needs education and 204 directors of rehabilitation departments (130 facilities for persons with severe motor and intellectual disabilities, 73 wards for patients with severe motor and intellectual disabilities, and the National Hospital Organization and National Center Hospital, and the National Center of Neurology and Psychiatry) in Japan. We used descriptive statistics and content analysis to examine the answers to the questionnaires. [Results] Information was acquired from 216 individuals (3-53 years old) with Rett syndrome. 92.9% of the individuals were reported to have some interests (e.g., in people, music, things to see, animation, or books). [Conclusion] Individuals with Rett syndrome were observed to be interested in various things despite their having severe motor and intellectual disabilities. These findings suggest that family members and care staff might facilitate various changes or developments of these individuals and discover their hidden strengths by focusing on their interests.

  6. Diagnosis and treatment of 409 patients with prostatitis syndromes

    NARCIS (Netherlands)

    de la Rosette, J. J.; Hubregtse, M. R.; Meuleman, E. J.; Stolk-Engelaar, M. V.; Debruyne, F. M.

    1993-01-01

    We reviewed 409 patients who had prostatitis syndromes during the period 1985-1991. Urine analysis, x-ray film of abdomen, and sonograms of the kidneys did not contribute to the diagnosis of prostatitis. In 22 percent of the urine samples, slight-to-moderate atypia was seen in urine cytology but no

  7. Prader-Willi syndrome in South African patients clinical and ...

    African Journals Online (AJOL)

    Prader-Willi syndrome in. South African patients clinical and molecular diagnosis. A L Christianson, 0 L Viljoen, W S Winship,. M de la Rey, E J van Rensburg. Study objective. ... This probe detects a parent-of-origin-specific methylation imprint at locus ... These studies were undertaken in the Molecular Genetic. Laboratory of ...

  8. Metabolic Syndrome in Patients attending the Staff Clinic of a ...

    African Journals Online (AJOL)

    Background/objective: Metabolic syndrome (MetS) is characterised by a clustering of cardiometabolic risk factors. It contributes to morbidity and mortality in adults. The objective of the study was to identify new cases and associated factors of MetS in patients attending a tertiary hospital staff clinic. Materials and methods: The ...

  9. Pulsatile thyrotropin secretion in patients with Cushing's syndrome

    NARCIS (Netherlands)

    Adriaanse, R.; Brabant, G.; Endert, E.; Wiersinga, W. M.

    1994-01-01

    Pulsatile and circadian thyrotropin (TSH) secretion were studied in 16 healthy controls and in three patients with Cushing's syndrome who were studied twice (before and after treatment). Blood was sampled every 10 minutes over 24 hours for TSH (immunoradiometric assay [IRMA]). Mean 24-hour TSH in

  10. Testicular cancer in a patient with Primrose syndrome

    NARCIS (Netherlands)

    Mathijssen, Inge B.; van Hasselt-van der Velde, Jos; Hennekam, Raoul C. M.

    2006-01-01

    A mentally retarded, adult man was found to have joint contractures, sparse body hair, hearing loss, dysmorphic facial features, large calcified pinnae and a huge torus palatinus. All features are similar to those earlier described in patients with Primrose syndrome. In addition he developed a germ

  11. Atrial fibrillation in patients with sick sinus syndrome

    DEFF Research Database (Denmark)

    Nielsen, Jens Cosedis; Thomsen, Poul Erik B; Højberg, Søren

    2012-01-01

    between minimal-paced programmed AVI = 100 and >100 ms (median value), respectively (P= 0.60).ConclusionsThe present study indicates that a longer baseline PQ-interval is associated with an increased risk of AF in patients with sick sinus syndrome. Atrial fibrillation burden is not associated...

  12. Hemorrhagic abscess in a patient with the acquired immunodeficiency syndrome

    International Nuclear Information System (INIS)

    Casado-Naranjo, I.; Lopez-Trigo, J.; Ferrandiz, A.; Cervello, A.; Navarro, V.

    1989-01-01

    Cerebral toxoplasmosis is frequent among patients with the acquired immunodeficiency syndrome. The common computed tomography appearance of this complication is multiple low density area with ring enhancement. We describe a very rare picture of cerebral toxoplasmosis, this is multiple hemorrhagic toxoplasmic abscess. (orig.)

  13. Difficult airway in a patient with Coffin-Siris syndrome.

    Science.gov (United States)

    Dimaculangan, D; Lokhandwala, B; Wlody, D; Gross, R

    2001-02-01

    We report on a patient with Coffin-Siris syndrome and consider a potential association between this condition and difficult intubation. Although this inherited condition is extremely rare, anesthesiologists should be aware of its existence and prepare for potential airway management problems whenever it is encountered.

  14. The identification of Lynch syndrome in Congolese colorectal cancer patients.

    Science.gov (United States)

    Poaty, Henriette; Aba Gandzion, Chandra; Soubeyran, Isabelle; Gassaye, Déby; Peko, Jean Félix; Nkoua Bon, Jean Bernard; Gombé Mbalawa, Charles

    2017-10-01

    We aimed to investigate the prevalence of Lynch syndrome as one of hereditary causes of colorectal cancer (CRC) among young Congolese individuals affected by the CRC, and to define methods for diagnosis in Congo Brazzaville. We conducted a transversal cohort study of 34 patients having a CRC with a family history for a period of eight years. They were selected among 89 CRCs of any type from the Bethesda guidelines criteria combined with pedigrees. Mismatch repair (MMR) genes alterations were researched by immunohistochemistry (IHC). We identified with the Bethesda criteria a total of 38.2% (34/89) patients having familial CRC with a confidence interval (CI) of 95%=[0.34-0.41]. Only 14.7% (5/34) 95% CI=[0.34-2.32] patients showed MMR immunodeficiency involving firstly MLH1 protein then MSH2 protein. These data account for 5.6% (5/89) 95% CI=[0.15-0.33] of patients affected by Lynch syndrome with an earlier median age of 35 years (range 20 to 47 years). The prevalence of Lynch syndrome found in Brazzaville is comparable to that is found in northern countries. The combined Bethesda guidelines, pedigree and IHC is an accessible and good alternative method for the positive diagnosis of Lynch syndrome in current practice in Congo. Copyright © 2017 Société Française du Cancer. Published by Elsevier Masson SAS. All rights reserved.

  15. Effects of vertical positioning on gas exchange and lung volumes in acute respiratory distress syndrome.

    Science.gov (United States)

    Richard, Jean-Christophe M; Maggiore, Salvatore Maurizio; Mancebo, Jordi; Lemaire, François; Jonson, Bjorn; Brochard, Laurent

    2006-10-01

    Supine position may contribute to the loss of aerated lung volume in patients with acute respiratory distress syndrome (ARDS). We hypothesized that verticalization increases lung volume and improves gas exchange by reducing the pressure surrounding lung bases. Prospective observational physiological study in a medical ICU. In 16 patients with ARDS we measured arterial blood gases, pressure-volume curves of the respiratory system recorded from positive-end expiratory pressure (PEEP), and changes in lung volume in supine and vertical positions (trunk elevated at 45 degrees and legs down at 45 degrees ). Vertical positioning increased PaO(2) significantly from 94+/-33 to 142+/-49 mmHg, with an increase higher than 40% in 11 responders. The volume at 20 cmH(2)O measured on the PV curve from PEEP increased using the vertical position only in responders (233+/-146 vs. -8+/-9 1ml in nonresponders); this change was correlated to oxygenation change (rho=0.55). End-expiratory lung volume variation from supine to vertical and 1 h later back to supine, measured in 12 patients showed a significant increase during the 1-h upright period in responders (n=7) but not in nonresponders (n=5; 215+/-220 vs. 10+/-22 ml), suggesting a time-dependent recruitment. Vertical positioning is a simple technique that may improve oxygenation and lung recruitment in ARDS patients.

  16. [Treatment of acute respiratory distress syndrome using pressure and volume controlled ventilation with lung protective strategy].

    Science.gov (United States)

    Ge, Ying; Wan, Yong; Wang, Da-qing; Su, Xiao-lin; Li, Jun-ying; Chen, Jing

    2004-07-01

    To investigate the significance and effect of pressure controlled ventilation (PCV) as well as volume controlled ventilation (VCV) by lung protective strategy on respiratory mechanics, blood gas analysis and hemodynamics in patients with acute respiratory distress syndrome (ARDS). Fifty patients with ARDS were randomly divided into PCV and VCV groups with permissive hypercapnia and open lung strategy. Changes in respiratory mechanics, blood gas analysis and hemodynamics were compared between two groups. Peak inspiration pressure (PIP) in PCV group was significantly lower than that in VCV group, while mean pressure of airway (MPaw) was significantly higher than that in VCV after 24 hours mechanical ventilation. After 24 hours mechanical ventilation, there were higher central venous pressure (CVP) and slower heart rate (HR) in two groups, CVP was significantly higher in VCV compared with PCV, and PCV group had slower HR than VCV group, the two groups had no differences in mean blood pressure (MBP) at various intervals. All patients showed no ventilator-induced lung injury. Arterial blood oxygenations were obviously improved in two groups after 24 hours mechanical ventilation, PCV group had better partial pressure of oxygen in artery (PaO2) than VCV group. Both PCV and VCV can improve arterial blood oxygenations, prevent ventilator-induced lung injury, and have less disturbance in hemodynamic parameters. PCV with lung protective ventilatory strategy should be early use for patients with ARDS.

  17. Pulmonar recruitment in acute respiratory distress syndrome. What is the best strategy?

    Directory of Open Access Journals (Sweden)

    Cíntia Lourenço Santos

    Full Text Available Supporting patients with acute respiratory distress syndrome (ARDS, using a protective mechanical ventilation strategy characterized by low tidal volume and limitation of positive end-expiratory pressure (PEEP is a standard practice in the intensive care unit. However, these strategies can promote lung de-recruitment, leading to the cyclic closing and reopening of collapsed alveoli and small airways. Recruitment maneuvers (RM can be used to augment other methods, like positive end-expiratory pressure and positioning, to improve aerated lung volume. Clinical practice varies widely, and the optimal method and patient selection for recruitment maneuvers have not been determined, considerable uncertainty remaining regarding the appropriateness of RM. This review aims to discuss recent findings about the available types of RM, and compare the effectiveness, indications and adverse effects among them, as well as their impact on morbidity and mortality in ARDS patients. Recent developments include experimental and clinical evidence that a stepwise extended recruitment maneuver may cause an improvement in aerated lung volume and decrease the biological impact seen with the traditionally used sustained inflation, with less adverse effects. Prone positioning can reduce mortality in severe ARDS patients and may be an useful adjunct to recruitment maneuvers and advanced ventilatory strategies, such noisy ventilation and BIVENT, which have been useful in providing lung recruitment.

  18. Edaravone attenuates lipopolysaccharide-induced acute respiratory distress syndrome associated early pulmonary fibrosis via amelioration of oxidative stress and transforming growth factor-β1/Smad3 signaling.

    Science.gov (United States)

    Wang, Xida; Lai, Rongde; Su, Xiangfen; Chen, Guibin; Liang, Zijing

    2018-01-01

    Pulmonary fibrosis is responsible for the both short-term and long-term outcomes in patients with acute respiratory distress syndrome (ARDS). There is still no effective cure to improve prognosis. The purpose of this study was to investigate whether edaravone, a free radical scavenger, have anti-fibrosis effects in the rat model of ARDS associated early pulmonary fibrosis by lipopolysaccharide (LPS) administration. Rats were subjected to intravenous injection of LPS, and edaravone was given intraperitoneally after LPS administration daily for 7 consecutive days. LPS treatment rapidly increased lung histopathology abnormalities, coefficient of lung, hydroxyproline and collagen I levels, stimulated myofibroblast differentiation and induced expression of TGF-β1 and activation of TGF-β1/Smad3 signaling as early as day 7 after LPS injection. Moreover, LPS intoxication significantly increased the contents of malondialdehyde (MDA), interleukin-6 (IL-6) and tumor necrosis factor-α (TNF-α), whereas it dramatically decreased superoxide dismutase (SOD) and glutathione peroxidase (GSH-PX) activities from day 1 after LPS treatment. On the contrary, edaravone treatment ameliorated LPS-induced myofibroblast differentiation and pulmonary fibrosis, simultaneously, and attenuated LPS-stimulated oxidative stress and activation of TGF-β1/Smad3 signaling. Collectively, edaravone may attenuate ARDS associated early pulmonary fibrosis through amelioration of oxidative stress and TGF-β1/Smad3 signaling pathway. Edaravone may be a promising drug candidate for the treatment of ARDS-related pulmonary fibrosis in early period. Copyright © 2017 Elsevier Inc. All rights reserved.

  19. Individualized Plastic Reconstruction Strategy for Patients With Ectodermal Dysplasia Syndrome.

    Science.gov (United States)

    Hou, Yikang; Jin, Yunbo; Lin, Xiaoxi; Chai, Gang; Zhang, Yan; Qi, Zuoliang

    2017-06-01

    Ectodermal dysplasia syndrome is a hereditary disease of ectodermal origin. Appearances of nail dystrophy, alopecia or hypotrichosis, saddle nose deformity, and palmoplantar hyperkeratosis are usually associated with a lack of sweat glands as well as partial or complete absence of teeth. These manifestations are usually corrected only with oral rehabilitation by mounting dentures. In this study, plastic rehabilitation was developed to correct the special features of patients with ectodermal dysplasia. Four men and 1 woman with ectodermal dysplasia syndrome were treated. Four patients showed dysostosis of the midface, and rhinoplasty with costal bone was performed, whereas cosmetic operation aiming to repair soft tissue defects was adopted for the last patient. After plastic corrections, all 5 patients were satisfied with the results and had no social embarrassment.

  20. Predictors of Death in Contemporary Adult Patients With Eisenmenger Syndrome

    DEFF Research Database (Denmark)

    Kempny, Aleksander; Hjortshøj, Cristel Sørensen; Gu, Hong

    2017-01-01

    : In a multicenter approach, we identified adults with Eisenmenger syndrome under follow-up between 2000 and 2015. We examined survival and its association with clinical, electrocardiographic, echocardiographic, and laboratory parameters. RESULTS: We studied 1098 patients (median age, 34.4 years; range, 16.......1-84.4 years; 65.1% female; 31.9% with Down syndrome). The majority had a posttricuspid defect (n=643, 58.6%), followed by patients with a complex (n=315, 28.7%) and pretricuspid lesion (n=140, 12.7%). Over a median follow-up of 3.1 years (interquartile range, 1.4-5.9), allowing for 4361.6 patient......-years observation, 278 patients died and 6 underwent transplantation. Twelve parameters emerged as significant predictors of death on univariable analysis. On multivariable Cox regression analysis, only age (hazard ratio [HR], 1.41/10 years; 95% confidence interval [CI], 1.24-1.59; P

  1. Ultrasound assessment of lung consolidation and reaeration after pleural effusion drainage in patients with Acute Respiratory Distress Syndrome: a pilot study.

    Science.gov (United States)

    Chinardet, B; Brisson, H; Arbelot, C; Langeron, O; Rouby, J J; Lu, Q

    2016-01-01

    The aim of the pilot study was to assess by ultrasound changes in dimensions of lung consolidation and reaeration after drainage of large pleural effusion in patients with acute respiratory distress syndrome (ARDS). Lung ultrasound and blood gas were performed before, 2 hours (H2) and 24 hours (H24) after drainage of pleural effusion. Lung ultrasound aeration score was calculated. Cephalocaudal dimension and diaphragmatic transversal area of lung consolidation were measured. Ten patients were studied. Median volume of drained effusion was 675 ml at H2 and 895 at H24. Two hours after drainage, dimension of cephalocaudal consolidation and diaphragmatic transversal area decreased significantly. Lung reaeration after drainage occurred mainly in latero-inferior and postero-superior regions. PaO2/FiO2 increased significantly at H24. Ultrasound is a useful method to assess lung consolidation after pleural effusion drainage. Drainage of pleural effusion may lead to a decrease of lung consolidation and improvement of lung reaeration.

  2. KERATODERMA BLENORRHAGICUM IN A PATIENT WITH REITER SYNDROME

    Directory of Open Access Journals (Sweden)

    Valentina Dimitrova

    2008-10-01

    Full Text Available Reiter syndrome is a systemic disorder, originally defined as a triad of arthritis, urethritis and conjunctivitis. This symptoms complex usually follows an episode of either urethritis or dysentery. Skin and mucosal involvement is observed in about 10% of the cases. We present a case of Reiter’s syndrome in a 55-year-old man who developed the typical skin lesions - kerathoderma blenorrhagicum. The disease started with a severe asymmetric oligoarthritis a month after the patient had urethritis. Two weeks after the onset of the arthritis red patches on the palms and plants appeared, which transformed quickly in harder and elevated plaques.

  3. [Anesthesia for a pediatric patient with Coffin-Siris syndrome].

    Science.gov (United States)

    Shirakami, Gotaro; Tazuke-Nishimura, Misako; Hirakata, Hideo; Fukuda, Kazuhiko

    2005-01-01

    A 3-year-old boy with Coffin-Siris syndrome was scheduled to undergo diagnostic laparoscopy, inguinal herniorrhaphy and orchiopexy at an ambulatory setting and same-day admission. Following anesthesia induction with inhalational sevoflurane, upper airway obstruction and hypoxemia developed. Hypoxemia was resolved immediately by manual positive pressure ventilation, although the stomach became bulged. Operation was finished uneventfully. However, he had massive bronchial secretion during anesthesia. He was admitted as planned and discharged on postoperative day 2. Since patients with Coffin-Siris syndrome have potential airway and pulmonary dysfunctions, careful perianesthesia airway and respiratory managements are essential.

  4. Spontaneous intracranial hypotension syndrome: magnetic resonance findings in two patients

    International Nuclear Information System (INIS)

    Ortega, R.; Pastor, J.; Escamilla, F.; Romero, M. I.

    1999-01-01

    The postural headache syndrome associated with a decrease in the cerebrospinal fluid (CSF) pressure is generally secondary to the CSF leakage that usually occurs after diagnostic lumbar puncture. Spontaneous intracranial hypotension can not be attributed to any known cause or previous diagnostic or therapeutic intervention. The syndrome is characterized by severe headache that is relieved by lying supine. During lumbar puncture, the CSF pressure is normally low. We present two patients in whom gadolinium-enhanced magnetic resonance imaging showed widespread thickening and enhancement of the dura mater and subdural fluid collections. (Author) 13 refs

  5. Phase analysis in patients with Wolff-Parkinson-White syndrome

    International Nuclear Information System (INIS)

    Nakajima, Kenichi; Bunko, Hisashi; Tada, Akira; Taki, Junichi; Tonami, Norihisa

    1983-01-01

    Twenty-five patients with Wolff-Parkinson-White (WPW) syndrome who underwent surgical division of the accessory conduction pathway (ACP) were studied by gated blood pool studies and phase analyses. All of 11 patients with right cardiac type (R-type) had abnormal initial phase in the right ventricle (RV), while 10 out of 14 patients with left cardiac type (L-type) had initial phase in the left ventricle (LV). However, in 4 L-type patients, there were no significant differences in the initiation of both ventricular contractions. In 10 patients who had radionuclide studies before and after surgical division of the ACP, the ventricular contraction patterns were apparently changed and the abnormal wall motions induced by the presence of ACPs disappeared. These observations indicate that the abnormal initial contraction is associated with pre-excitation of WPW syndrome. Sensitivities to identify the side of preexcitation were 100% (11/11) for R-type and 71% (10/14) for L-type. However, regarding the detection of the precise site of ACP, the agreement was 48% (12/25). Therefore, as a method of preoperative study, it seemed difficult to identify the precise localization of the ACP by phase analysis alone. Phase analysis provided interesting informations and was useful for evaluating patients with WPW syndrome before and after surgery. (author)

  6. Anaplerosis in Complex Treatment of Patients with Diabetic Foot Syndrome

    Directory of Open Access Journals (Sweden)

    B.G. Bezrodny

    2015-05-01

    Full Text Available The article describes the improvement of anaplerosis in patients with diabetic foot syndrome using skin flaps on vascular pedicle of the perforating vessels. The study involved patients with type 2 diabetes mellitus complicated with diabetic foot syndrome of neuroischemic form and chronic wounds of the lower extremities that do not heal for more than 21 days from the date of occurrence. The wounds were cleaned with ultrasonic cavitation. There was applied bandage with sorption antibacterial remedy base on nanodispersed silicon dioxide. There was applied a drainage vacuum bandage on a wound on the third day (VAC therapy. The flap is forming fitting to the size and configuration of a wound on a foot. Fourteen patients (93 % in the basic group were found to have survived flaps. Long-term follow up in 6 months demonstrated full maintenance of supporting function and good survived skin graft, absence of foot ulcers. In a control group 7 patients had recurrent foot ulcer. Improved techniques of autodermoplasty in patients with diabetic foot syndrome include glycemia control, preparation of a wound using vacuum apparatus bandage. Usage of split-skin graft combined with vacuum apparatus bandage allows close acute and chronic wounds effectively, maintain supporting function of an extremity, decrease in-hospital staying, and improve quality of patient’s life. Adequate foot wound closure prevents high-level amputation of low extremities in diabetic patients.

  7. Relationship between two blood stasis syndromes and inflammatory factors in patients with acute coronary syndrome.

    Science.gov (United States)

    Ma, Cai-Yun; Liu, Jing-Hua; Liu, Jian-Xun; Shi, Da-Zhuo; Xu, Zhen-Ye; Wang, Shao-Ping; Jia, Min; Zhao, Fu-Hai; Jiang, Yue-Rong; Ma, Qin; Peng, Hong-Yu; Lu, Yuan; Zheng, Ze; Ren, Feng-Xue

    2017-11-01

    To investigate the relationship between inflammatory factors and two Chinese medicine (CM) syndrome types of qi stagnation and blood stasis (QSBS) and qi deficiency and blood stasis (QDBS) in patients with acute coronary syndrome (ACS). Sixty subjects with ACS, whose pathogenesis changes belongs to qi disturbance blood stasis syndrome, were divided into 2 groups: 30 in the QSBS group and 30 in the QDBS group. The comparative analysis on them was carried out through comparing general information, coronary angiography and inflammatory factors including intracellular adhesion molecule-1 (ICAM-1), chitinase-3-like protein 1 (YKL-40) and lipoprotein-associated phospholipase A2 (Lp-PLA2). Compared with the QSBS group, Lp-PLA2 and YKL-40 levels in the QDBS group showed no-significant difference (P>0.05); ICAM-1 was significantly higher in the QDBS group than in the QSBS group in the pathological processes of qi disturbance and blood stasis syndrome of ACS (Psyndrome typing of QSBS and QDBS, which provides a research direction for standardization research of CM syndrome types.

  8. Application of dead space fraction to titrate optimal positive end-expiratory pressure in an ARDS swine model.

    Science.gov (United States)

    Bian, Weishuai; Chen, Wei; Chao, Yangong; Wang, Lan; Li, Liming; Guan, Jian; Zang, Xuefeng; Zhen, Jie; Sheng, Bo; Zhu, Xi

    2017-04-01

    This study aimed to apply the dead space fraction [ratio of dead space to tidal volume (VD/VT)] to titrate the optimal positive end-expiratory pressure (PEEP) in a swine model of acute respiratory distress syndrome (ARDS). Twelve swine models of ARDS were constructed. A lung recruitment maneuver was then conducted and the PEEP was set at 20 cm H 2 O. The PEEP was reduced by 2 cm H 2 O every 10 min until 0 cm H 2 O was reached, and VD/VT was measured after each decrement step. VD/VT was measured using single-breath analysis of CO 2 , and calculated from arterial CO 2 partial pressure (PaCO 2 ) and mixed expired CO 2 (PeCO 2 ) using the following formula: VD/VT = (PaCO 2 - PeCO 2 )/PaCO 2 . The optimal PEEP was identified by the lowest VD/VT method. Respiration and hemodynamic parameters were recorded during the periods of pre-injury and injury, and at 4 and 2 cm H 2 O below and above the optimal PEEP (Po). The optimal PEEP in this study was found to be 13.25±1.36 cm H 2 O. During the Po period, VD/VT decreased to a lower value (0.44±0.08) compared with that during the injury period (0.68±0.10) (P<0.05), while the intrapulmonary shunt fraction reached its lowest value. In addition, a significant change of dynamic tidal respiratory compliance and oxygenation index was induced by PEEP titration. These results indicate that minimal VD/VT can be used for PEEP titration in ARDS.

  9. Ultrastructural pathology of aortic dissections in patients with Marfan syndrome: Comparison with dissections in patients without Marfan syndrome

    NARCIS (Netherlands)

    Dingemans, Koert P.; Teeling, Peter; van der Wal, Allard C.; Becker, Anton E.

    2006-01-01

    Despite the discovery in 1990 that mutations in the fibrillin-1 gene cause the Marfan syndrome, the pathogenesis of the life-threatening dissections associated with this disease is far from elucidated. Both the massive number of known fibrillin-1 mutations that result in a heterogeneous patient

  10. Taxane-induced morphea in a patient with CREST syndrome

    Directory of Open Access Journals (Sweden)

    Susan Michele Bouchard

    2010-07-01

    Full Text Available The taxanes, docetaxel and paclitaxel, are microtubule stabilizing chemotherapeutic agents that have demonstrated antineoplastic effects in a variety of solid tumors. They have been linked to the development of localized cutaneous sclerosis in some patients. We present a case of docetaxel-induced cutaneous sclerosis of the lower extremities in a patient with pre-existing CREST syndrome. We propose that patients with a history of limited or diffuse systemic sclerosis should be given taxane chemotherapy with caution, as these patients may have an immunological predisposition for the development of drug-induced morphea.

  11. Liver transplantation in a patient with primary antiphospholipid syndrome and Budd-Chiari syndrome

    Science.gov (United States)

    Reshetnyak, Tatiana M; Seredavkina, Natalia V; Satybaldyeva, Maria A; Nasonov, Evgeniy L; Reshetnyak, Vasiliy I

    2015-01-01

    The antiphospholipid syndrome (APS) is an acquired thrombophilic disorder in which autoantibodies are produced to a variety of phospholipids determinants of cell membranes or phospholipid binding proteins. There are few reports about association between antiphospholipid antibodies and development of Budd-Chiari syndrome (BCS). We report the case of BCS development in young Russian male with primary APS. The patient underwent orthotopic liver transplantation on August 26, 2012. At present time his state is good, the blood flow in the liver restored and its function is not impaired. We report about the first time the successful use of dabigatran etexilate for prolonged anticoagulation therapy in APS patient with BCS. In addition patient is managed with immunosuppressive drugs. PMID:26380049

  12. Patient safety and nursing: interface with stress and Burnout Syndrome

    Directory of Open Access Journals (Sweden)

    Cláudia Cristiane Filgueira Martins Rodrigues

    Full Text Available ABSTRACT Objective: To analyze studies on stress, Burnout Syndrome, and patient safety in the scope of nursing care in the hospital environment. Method: This was an integrative literature review. Data collection was performed in February 2016 in the following databases: Medical Literature Analysis and Retrieval System Online - PubMed/MEDLINE, Latin American and Caribbean Literature in Health Sciences - LILACS. Results: Ten scientific productions were selected, which listed that factors contributing to stress and Burnout Syndrome of nursing professionals are the work environment as a source of stress, and excessive workload as a source of failures. Conclusion: The analysis found that the stress and Burnout Syndrome experienced by these professionals lead to greater vulnerability and development of unsafe care, and factors such as lack of organizational support can contribute to prevent these failures.

  13. BILATERAL SEROUS MACULAR DETACHMENT IN A PATIENT WITH NEPHROTIC SYNDROME.

    Science.gov (United States)

    Bilge, Ayse D; Yaylali, Sevil A; Yavuz, Sara; Simsek, İlke B

    2018-01-01

    The purpose of this study was to report a case of a woman with nephrotic syndrome who presented with blurred vision because of bilateral serous macular detachment. Case report and literature review. A 55-year-old woman with a history of essential hypertension, diabetes, and nephrotic syndrome was presented with blurred vision in both eyes. Her fluorescein angiography revealed dye leakage in the early and subretinal pooling in the late phases, and optical coherence tomography scans confirmed the presence of subretinal fluid in the subfovel area. In nephrotic syndrome cases especially with accompaniment of high blood pressure, fluid accumulation in the retina layer may occur. Serous macular detachment must be kept in mind when treating these patients.

  14. Cellulitis as complication of nephrotic syndrome in a pediatric patient

    Science.gov (United States)

    Siregar, R. S.; Daulay, K. R.; Siregar, B.; Ramayani, O. R.; Eyanoer, P. C.

    2018-03-01

    Nephrotic syndrome is a chronic disease that may act as a risk for other major infection in skin, respiratory and urinary tract, while also increasingthe chance for other diseases, like peritonitis, meningitis, and cellulitis. Cellulitis is often caused by Streptococcus β-hemolytic, Staphylococcus aureus, and Escherichia coli. The clinical features of cellulitis marked with redness rash and well-defined borders, pain pressure and swelling. Hypoalbuminemia which occurs due to proteinuria occurred in this patient acts as a risk factor for cellulitis. It has been reported the case of cellulitis as one of the complications of the nephrotic syndrome in the pediatric patient. The treatment has been given to the patient such as antibiotics and supportive therapy and also planned albumin substitution.

  15. Safety and efficacy of angioplasty with intracoronary stenting in patients with unstable coronary syndromes. Comparison with stable coronary syndromes

    Directory of Open Access Journals (Sweden)

    Luís C. L. Correia

    2000-06-01

    Full Text Available OBJECTIVE: To assess safety and efficacy of coronary angioplasty with stent implantation in unstable coronary syndromes. METHODS: Retrospective analysis of in-hospital and late evolution of 74 patients with unstable coronary syndromes (unstable angina or infarction without elevation of the ST segment undergoing coronary angioplasty with stent placement. These 74 patients were compared with 31 patients with stable coronary syndromes (stable angina or stable silent ischemia undergoing the same procedure. RESULTS: No death and no need for revascularization of the culprit artery occurred in the in-hospital phase. The incidences of acute non-Q-wave myocardial infarction were 1.4% and 3.2% (p=0.6 in the unstable and stable coronary syndrome groups, respectively. In the late follow-up (11.2±7.5 months, the incidences of these events combined were 5.7% in the unstable coronary syndrome group and 6.9% (p=0.8 in the stable coronary syndrome group. In the multivariate analysis, the only variable with a tendency to significance as an event predictor was diabetes mellitus (p=0.07; OR=5.2; 95% CI=0.9-29.9. CONCLUSION: The in-hospital and late evolutions of patients with unstable coronary syndrome undergoing angioplasty with intracoronary stent implantation are similar to those of the stable coronary syndrome group, suggesting that this procedure is safe and efficacious when performed in unstable coronary syndrome patients.

  16. Posterior reversible encephalopathy syndrome in patient

    African Journals Online (AJOL)

    abp

    2015-05-26

    neuroradiologic condition, not commonly reported in the literature. PRES is an uncommon complication of severe preeclampsia/eclampsia. We report the management of one patient with postpartum preeclampsia as an.

  17. Implantation of cultured thymic fragments in patients with acquired immunodeficiency syndrome

    NARCIS (Netherlands)

    Danner, S. A.; Schuurman, H. J.; Lange, J. M.; Gmelig Meyling, F. H.; Schellekens, P. T.; Huber, J.; Kater, L.

    1986-01-01

    Cultured thymic fragments were implanted in one patient with acquired immunodeficiency syndrome (AIDS)-related complex (ARC) and in eight AIDS patients with opportunistic infections (OIs, four patients), Kaposi's sarcoma (KS, two patients), or both (two patients). Thereafter, objective clinical

  18. Cochlear implantation in patient with Dandy-walker syndrome.

    Science.gov (United States)

    de Oliveira, Adriana Kosma Pires; Hamerschmidt, Rogerio; Mocelin, Marcos; Rezende, Rodrigo K

    2012-07-01

     Dandy Walker Syndrome is a congenital abnormality in the central nervous system, characterized by a deficiency in the development of middle cerebelar structures, cystic dilatation of the posterior pit communicating with the fourth ventricle and upward shift of the transverse sinuses, tentorium and dyes. Among the clinical signs are occipital protuberances, a progressive increase of the skull, bowing before the fontanels, papilledema, ataxia, gait disturbances, nystagmus, and intellectual impairment.  To describe a case of female patient, 13 years old with a diagnosis of this syndrome and bilateral hearing loss underwent cochlear implant surgery under local anesthesia and sedation.  CGS, 13 years old female was referred to the Otolaryngological Department of Otolaryngology Institute of Parana with a diagnosis of "Dandy-Walker syndrome" for Otolaryngological evaluation for bilateral hearing loss with no response to the use of hearing aids. Final Comments: The field of cochlear implants is growing rapidly. We believe that the presence of Dandy-Walker syndrome cannot be considered a contraindication to the performance of cochlear implant surgery, and there were no surgical complications due to neurological disorders with very favorable results for the patient who exhibits excellent discrimination. It has less need for lip reading with improvement in speech quality.

  19. Cochlear implantation in patient with Dandy-walker syndrome

    Directory of Open Access Journals (Sweden)

    Oliveira, Adriana Kosma Pires de

    2012-01-01

    Full Text Available Introduction: Dandy Walker Syndrome is a congenital abnormality in the central nervous system, characterized by a deficiency in the development of middle cerebelar structures, cystic dilatation of the posterior pit communicating with the fourth ventricle and upward shift of the transverse sinuses, tentorium and dyes. Among the clinical signs are occipital protuberances, a progressive increase of the skull, bowing before the fontanels, papilledema, ataxia, gait disturbances, nystagmus, and intellectual impairment. Objectives: To describe a case of female patient, 13 years old with a diagnosis of this syndrome and bilateral hearing loss underwent cochlear implant surgery under local anesthesia and sedation. Case Report: CGS, 13 years old female was referred to the Otolaryngological Department of Otolaryngology Institute of Parana with a diagnosis of "Dandy-Walker syndrome" for Otolaryngological evaluation for bilateral hearing loss with no response to the use of hearing aids. Final Comments: The field of cochlear implants is growing rapidly. We believe that the presence of Dandy-Walker syndrome cannot be considered a contraindication to the performance of cochlear implant surgery, and there were no surgical complications due to neurological disorders with very favorable results for the patient who exhibits excellent discrimination. It has less need for lip reading with improvement in speech quality.

  20. Vitamin K status in patients with short bowel syndrome.

    Science.gov (United States)

    Krzyżanowska, Patrycja; Książyk, Janusz; Kocielińska-Kłos, Małgorzata; Banaś, Elżbieta; Kaleta, Małgorzata; Popińska, Katarzyna; Szczapa, Tomasz; Walkowiak, Jarosław

    2012-12-01

    Available evidence suggests that patients with short bowel syndrome (SBS) might be at risk of vitamins A, D, E and B(1) deficiency. However, there is little clinical data describing the vitamin K status. Therefore, in the present study we aimed to assess the body resources of vitamin K in a subset of SBS patients. The study comprised 33 patients aged 1 month to 16 years. PIVKA-II concentrations were determined in all subjects. In all studied subjects, coagulation parameters were normal. PIVKA-II levels indicative of vitamin K deficiency was found in 3 (9.1%) SBS patients. One patient had been receiving an additional intravenous vitamin K dose of 5 mg/week. In all SBS patients with cirrhosis and cholestasis, PIVKA-II concentrations were low (vitamin K several times a month. Vitamin K deficiency may appear in SBS patients. Copyright © 2012. Published by Elsevier Ltd.

  1. Improvement of Oxygenation in Severe Acute Respiratory Distress Syndrome With High-Volume Continuous Veno-venous Hemofiltration.

    Science.gov (United States)

    Yang, Wenmin; Hong, Jie; Zeng, Qiyi; Tao, Jianping; Chen, Feiyan; Dang, Run; Liang, Yufeng; Wu, Zhiyuan; Yang, Yiyu

    2016-01-01

    The efficacy and therapeutic mechanisms of continuous renal replacement therapy (CRRT) for improvement of oxygenation in acute respiratory distress syndrome (ARDS) remain controversial. These questions were addressed by retrospective analysis of severe ARDS patients admitted to the pediatric intensive care unit of our hospital from 2009 to 2015 who received high-volume continuous veno-venous hemofiltration during mechanical ventilation. There was a significant improvement in partial oxygen pressure/fraction of inspired oxygen (PaO2/FiO2) 24 hours after CRRT onset compared with baseline (median change = 51.5; range = -19 to 450.5; P Improvement in oxygenation is likely related to both restoration of fluid balance and clearance of inflammatory mediators.

  2. Marfan syndrome. Part 2: treatment and management of patients.

    Science.gov (United States)

    Cañadas, Victoria; Vilacosta, Isidre; Bruna, Isidoro; Fuster, Valentin

    2010-05-01

    Aortic disease is the main cause of death among patients with Marfan syndrome. Before the development of open surgery, most patients died in the fourth decade of life. Improvements in surgical techniques have facilitated prophylactic surgery and have dramatically changed the life expectancy of patients with Marfan syndrome. Valve-sparing techniques are becoming the standard surgical treatment for these patients, since the operative and long-term results are comparable with those obtained with the Bentall and De Bono procedure and their theoretical advantages over the Bentall and De Bono procedure are attractive for young patients. Distal aortic complications still cause substantial morbidity in patients who have undergone surgery. On the other hand, several medical approaches have appeared as alternatives or adjuncts to the standard treatment with beta-blockers. Mouse models of the disease have shown that the angiotensin II receptor blocker losartan can rescue the phenotype. Among female patients, pregnancy deserves special consideration. Aortic dissection occurs mainly in the third trimester of gestation and in patients with dilated aortas. As aortic dissection carries a high risk of maternal mortality and fetal demise, prophylactic aortic surgery is recommended before attempting pregnancy for those women with an aortic diameter exceeding 40 mm.

  3. Joubert syndrome: Clinical and radiological characteristics of nine patients

    Directory of Open Access Journals (Sweden)

    Ahmed Farag Elhassanien

    2013-01-01

    Full Text Available Background: Joubert Syndrome (JS is a rare genetic developmental disorder, first identified in 1969. In patients with JS, certain regions of the brain (mainly cerebellar vermis and brainstem are underdeveloped or malformed. This can lead to impaired attention, visual, spatial, motor, language and social functional skills. JS is characterized by a host of features, many of which do not occur in every patient. Aim of the Study: To spotlight and increase awareness of clinical profile and neuroimaging findings of children with Joubert syndrome. Methods: This is a retrospective case series study of patients with JS who attended the Pediatric Neurology Clinic in Aladan and Alfarawanya Hospitals in Kuwait, from September 2007 to September 2012. Clinical and radiological data were obtained from the patient medical records. Results: Cerebellar vermis hypoplasia/aplasia and apnea were present in all patients, polydactly in 3 of 16, renal problems with cysts in 5 patients and 11 of 16 had abnormal electroretinograms (ERGs. Blood investigations of organic acids, amino acids and very-long-chain fatty acid, were normal in the all the nine patients. Conclusion: JS is a rare genetic brain malformation with association of retinal dystrophy and renal abnormalities. The retinal dystrophy may be progressive. The prognosis of patients depends mainly on the degree of brain malformation.

  4. Acute flaccid paraparesis (cauda equina syndrome) in a patient with Bardet–Biedl syndrome

    Science.gov (United States)

    Viswanathan, Vibhu Krishnan; Kanna, Rishi Mugesh; Shetty, Ajoy Prasad; Rajasekaran, S

    2017-01-01

    Bardet–Biedl syndrome (BBS) is a rare, autosomal-recessive, debilitating genetic disorder, which can present with multitudinous systemic clinical features including rod-cone dystrophy, polydactyly, Frohlich-like central obesity, mental retardation, hypogonadism, and renal anomalies. Diverse neuromuscular manifestations in patients afflicted by this heterogeneous disorder include ataxia, cervical, and thoracic canal stenoses, presenting as spastic quadriparesis and other gait disturbances. We report a young patient with BBS, who had presented with acute flaccid paraparesis due to severe primary lumbar canal stenosis. She underwent immediate lumbar decompression and discectomy following which she recovered significantly. Acute cauda equina syndrome due to primary lumbar canal stenosis has not been reported as a clinical feature of BBS previously. PMID:28566787

  5. Chest wall syndrome among primary care patients: a cohort study

    Directory of Open Access Journals (Sweden)

    Verdon François

    2007-09-01

    Full Text Available Abstract Background The epidemiology of chest pain differs strongly between outpatient and emergency settings. In general practice, the most frequent cause is the chest wall pain. However, there is a lack of information about the characteristics of this syndrome. The aims of the study are to describe the clinical aspects of chest wall syndrome (CWS. Methods Prospective, observational, cohort study of patients attending 58 private practices over a five-week period from March to May 2001 with undifferentiated chest pain. During a one-year follow-up, questionnaires including detailed history and physical exam, were filled out at initial consultation, 3 and 12 months. The outcomes were: clinical characteristics associated with the CWS diagnosis and clinical evolution of the syndrome. Results Among 24 620 consultations, we observed 672 cases of chest pain and 300 (44.6% patients had a diagnosis of chest wall syndrome. It affected all ages with a sex ratio of 1:1. History and sensibility to palpation were the keys for diagnosis. Pain was generally moderate, well localised, continuous or intermittent over a number of hours to days or weeks, and amplified by position or movement. The pain however, may be acute. Eighty-eight patients were affected at several painful sites, and 210 patients at a single site, most frequently in the midline or a left-sided site. Pain was a cause of anxiety and cardiac concern, especially when acute. CWS coexisted with coronary disease in 19 and neoplasm in 6. Outcome at one year was favourable even though CWS recurred in half of patients. Conclusion CWS is common and benign, but leads to anxiety and recurred frequently. Because the majority of chest wall pain is left-sided, the possibility of coexistence with coronary disease needs careful consideration.

  6. Chest wall syndrome among primary care patients: a cohort study.

    Science.gov (United States)

    Verdon, François; Burnand, Bernard; Herzig, Lilli; Junod, Michel; Pécoud, Alain; Favrat, Bernard

    2007-09-12

    The epidemiology of chest pain differs strongly between outpatient and emergency settings. In general practice, the most frequent cause is the chest wall pain. However, there is a lack of information about the characteristics of this syndrome. The aims of the study are to describe the clinical aspects of chest wall syndrome (CWS). Prospective, observational, cohort study of patients attending 58 private practices over a five-week period from March to May 2001 with undifferentiated chest pain. During a one-year follow-up, questionnaires including detailed history and physical exam, were filled out at initial consultation, 3 and 12 months. The outcomes were: clinical characteristics associated with the CWS diagnosis and clinical evolution of the syndrome. Among 24 620 consultations, we observed 672 cases of chest pain and 300 (44.6%) patients had a diagnosis of chest wall syndrome. It affected all ages with a sex ratio of 1:1. History and sensibility to palpation were the keys for diagnosis. Pain was generally moderate, well localised, continuous or intermittent over a number of hours to days or weeks, and amplified by position or movement. The pain however, may be acute. Eighty-eight patients were affected at several painful sites, and 210 patients at a single site, most frequently in the midline or a left-sided site. Pain was a cause of anxiety and cardiac concern, especially when acute. CWS coexisted with coronary disease in 19 and neoplasm in 6. Outcome at one year was favourable even though CWS recurred in half of patients. CWS is common and benign, but leads to anxiety and recurred frequently. Because the majority of chest wall pain is left-sided, the possibility of coexistence with coronary disease needs careful consideration.

  7. Chest wall syndrome among primary care patients: a cohort study

    Science.gov (United States)

    Verdon, François; Burnand, Bernard; Herzig, Lilli; Junod, Michel; Pécoud, Alain; Favrat, Bernard

    2007-01-01

    Background The epidemiology of chest pain differs strongly between outpatient and emergency settings. In general practice, the most frequent cause is the chest wall pain. However, there is a lack of information about the characteristics of this syndrome. The aims of the study are to describe the clinical aspects of chest wall syndrome (CWS). Methods Prospective, observational, cohort study of patients attending 58 private practices over a five-week period from March to May 2001 with undifferentiated chest pain. During a one-year follow-up, questionnaires including detailed history and physical exam, were filled out at initial consultation, 3 and 12 months. The outcomes were: clinical characteristics associated with the CWS diagnosis and clinical evolution of the syndrome. Results Among 24 620 consultations, we observed 672 cases of chest pain and 300 (44.6%) patients had a diagnosis of chest wall syndrome. It affected all ages with a sex ratio of 1:1. History and sensibility to palpation were the keys for diagnosis. Pain was generally moderate, well localised, continuous or intermittent over a number of hours to days or weeks, and amplified by position or movement. The pain however, may be acute. Eighty-eight patients were affected at several painful sites, and 210 patients at a single site, most frequently in the midline or a left-sided site. Pain was a cause of anxiety and cardiac concern, especially when acute. CWS coexisted with coronary disease in 19 and neoplasm in 6. Outcome at one year was favourable even though CWS recurred in half of patients. Conclusion CWS is common and benign, but leads to anxiety and recurred frequently. Because the majority of chest wall pain is left-sided, the possibility of coexistence with coronary disease needs careful consideration. PMID:17850647

  8. Rare successful pregnancy in a patient with Swyer Syndrome.

    Science.gov (United States)

    Taneja, Jyoti; Ogutu, David; Ah-Moye, Michael

    2016-10-01

    To report a rare successful pregnancy after fertility treatment in a patient with Swyer syndrome. Case report. Herts & Essex Fertility Centre, Cheshunt, UK. A 36-year-old patient with 46, XY gonadal dysgenesis. 31 year old husband with normal sperm analysis. Chromosomal analysis, Saline infusion sonography, Pipelle endometrial scratch, ICSI using donor eggs, Embryo Transfer, and Caesarean delivery. Successful pregnancy and live birth. Successful treatment with donor eggs, pregnancy, and delivery. A patient with 46, XY gonadal dysgenesis in a specially tailored fertility program, can maintain a normal pregnancy and delivery.

  9. A patient with WPW syndrome and coronary artery disease

    International Nuclear Information System (INIS)

    Zarebinski, M.; Krupienicz, A.; Marciniak, W.; Ostrowski, M.

    1993-01-01

    A 61-year-old patient with Wolff-Parkinson-White's syndrome, and hypertension was admitted to the CCU, because of the first episode of substernal chest pain. ECG was deformed by Wolff-Parkinson-White's syndrome, type B, with accessory pathway located on the right side, without evolution. Serum enzymes remained low. Echocardiography showed akinesis of the posterior wall and hypokinesis of the lateral wall (the same contraction disorders were described in previous echocardiographical examination 5 years ago), it was observed that the first portion of myocardium to contract was the base of the right ventricle. To elucidate the etiology of the contraction disorders, scintigraphy of the heart, using thallium 201, was performed, showing normal perfusion of the myocardium. To illustrate the dependence of the contraction disorders and abnormal depolarization pattern of the heart, echocardiographical examination was repeated, confirming the previous results, then 100 mg of Ajmaline was given to the patient intravenously, and echocardiographical examination was continued. Administration of the drug caused antidromic atrioventricular re-entrant tachycardia during which the lateral wall of the heart had been contracting properly. This case shows contraction disorders of the heart caused by the abnormal depolarization pattern, resulting from the presence of accessory pathway. It also illustrates the diagnostic difficulties in patients with Wolff-Parkinson-White's syndrome and suspected myocardial infarction, at the same time showing that scintigraphy of the heart might be very helpful in such patients. This case confirms the usefulness of echocardiography for localization of the accessory pathway. (author)

  10. Cataract surgery in patients with pseudoexfoliation syndrome: current updates

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    Fontana L

    2017-07-01

    Full Text Available Luigi Fontana, Marco Coassin, Alfonso Iovieno, Antonio Moramarco, Luca Cimino Ophthalmology Unit, Arcispedale Santa Maria Nuova – IRCCS, Reggio Emilia, Italy Abstract: Pseudoexfoliation is a ubiquitous syndrome of multifactorial origin affecting elderly people by increasing the risk of cataract and secondary glaucoma development. Despite modern techniques and technologies for cataract surgery, pseudoexfoliation syndrome represents a challenge for surgeons because of the increased weakness of the zonular apparatus and limited pupil dilation. Due to the inherent difficulties during surgery, the risk of vitreous loss in these patients is several times higher than in cataract patients without pseudoexfoliation. Using currently available surgical devices (ophthalmic viscosurgical device, iris retractors and ring dilators, capsular tension ring, etc., the risk of intraoperative complications may be much reduced, allowing the surgeon to handle difficult cases with greater confidence and safety. This review analyzes the methodologic approach to the patient with zonular laxity with the aim of providing useful advices to limit the risks of intraoperative and postoperative complications. From the preoperative planning, to the intraoperative management of the small pupil and phacodonesis, and to the postoperative correction of capsule phimosis and intraocular lens dislocation, a step approach to the surgical management of pseudoexfoliation patients is illustrated. Keywords: pseudoexfoliation syndrome, cataract surgery, zonular laxity, intraocular lens implant, complications

  11. Pituitary size in patients with Laron syndrome (primary GH insensitivity).

    Science.gov (United States)

    Kornreich, Liora; Horev, Gadi; Schwarz, Michael; Karmazyn, Boaz; Laron, Zvi

    2003-03-01

    The purpose of the present study was to investigate whether lifelong secretion of high levels of GH, characteristic of Laron syndrome, leads to an increase in the size of the pituitary gland. Eleven patients (six females, five males) with Laron syndrome underwent magnetic resonance imaging of the pituitary region with a system operating at 0.5 T. There were nine adults aged 36-68 Years and two children, a 4-Year-old boy and a 9-Year-old girl. The latter patient had been treated with IGF-I (150-180 mg/kg per day) since the age of 3 Years; all the other patients were untreated. The height of the adenohypophysis was measured on the sagittal images and compared with reference values for age and sex. The height of the adenohypophysis was within the normal range for age and gender in all patients, except for one male, who had a small gland. No congenital anomalies of the pituitary-hypothalamic region were detected. Despite the lifelong high levels of GH, no pituitary hypertrophy was detected. The anatomy of the pituitary-hypothalamic region in Laron syndrome is normal.

  12. [Anemic syndrome frequency in complicated obstetrical patients].

    Science.gov (United States)

    Martínez, Maria Guadalupe Veloz; Erasto, Luis Cruz; Maxines, Claudia García; Rodríguez, María Antonia Basavilvazo; Valencia, Marcelino Hernández

    2008-09-01

    The prevalence of anemia varies from country to country and there is not a trustworthy record. To determine the frequency of anemia in obstetric patients and the association among healthy pregnancy and aggregate complications. Was carried out as transversal, observational and comparative study. Obstetrical patients entered and responded in the period of a year, were formed a group with normal pregnancy and another with complicated pregnancy, with a total sample of 194 patients. In the statistical analysis was employed Student t test for independent groups, with value if p anemia was found in 22.4%. Hematological stage from group with normal pregnancy was mild anemia in 16.9% and anemia moderated in 4.1% of the cases. The anemia degrees in the group with associated illness and pregnancy were mild anemia in 19.2% and moderated anemia in 4.2%. Not any case was found with severe anemia. The statistical analysis showed difference significant among both groups p preeclampsia severe (22.6%), type 2 diabetes (13.9%), gestational diabetes (12.2%) and the remainder with other complications that include to the hypertiroidism, rheumatoid arthritis, lupus, asthma and vein deep thrombosis. Frequency of anemia in this study was greater upon informing in the international literature. The obstetrical complication more frequently relates to diverse anemia degrees were the hypertensive stage during pregnancy. The anemia is presented with greater frequency in pregnancy patients with others associated illness.

  13. Retrospective evaluation of the prevalence, risk factors, management, outcome, and necropsy findings of acute lung injury and acute respiratory distress syndrome in dogs and cats: 29 cases (2011-2013).

    Science.gov (United States)

    Balakrishnan, Anusha; Drobatz, Kenneth J; Silverstein, Deborah C

    2017-11-01

    To determine the prevalence and risk factors for veterinary acute lung injury (VetALI) and veterinary acute respiratory distress syndrome (VetARDS), assess mechanical ventilation settings and patient outcomes, and to evaluate the relationship of clinical diagnoses with necropsy findings. Retrospective study. University teaching hospital. Twenty-four dogs and 5 cats with a clinical diagnosis of VetALI or VetARDS. Control population includes 24 dogs and 5 cats with a clinical diagnosis of respiratory disease other than VetALI or VetARDS. None. VetALI and VetARDS were diagnosed in 3.2% of dogs and 1.3% of cats presenting to the ICU. Systemic inflammatory response syndrome was the most common inciting condition (16/24 dogs, 2/5 cats), followed by vomiting and subsequent aspiration of gastric contents (9/24 dogs), sepsis (5/24 dogs, 3/5 cats), multiple transfusions (4/24 dogs), trauma (3/24 dogs), and adverse drug reactions (1/24 dogs, 1/5 cats).  None of these conditions were found to be significantly associated with a risk of development of VetALI or VetARDS when compared to controls. Twelve dogs (50%) and 4 cats (80%) underwent mechanical ventilation for a median duration of 18 hours in dogs (range: 6-174 h) and 15.5 hours in cats (range: 6-91 h). Overall, 3/29 patients survived to discharge including 2/24 dogs and 1/5 cats. Necropsy results were available for 8/22 dogs and 3/4 cats. A total of 6/8 dogs (75%) dogs and 3/3 (100%) cats met the histopathologic criteria for diagnosis of VetALI or VetARDS. VetALI and VetARDS can cause life-threatening respiratory distress in dogs and cats necessitating mechanical ventilation in 50% of dogs and 80% of cats in this study. These diseases are associated with a poor clinical outcome and a high rate of humane euthanasia. © Veterinary Emergency and Critical Care Society 2017.

  14. Thoracoabdominal aortic aneurysm repair in patients with marfan syndrome.

    Science.gov (United States)

    Mommertz, G; Sigala, F; Langer, S; Koeppel, T A; Mess, W H; Schurink, G W H; Jacobs, M J

    2008-02-01

    We assessed the surgical outcome of descending thoracic aortic aneurysm repair (DTAA) and thoracoabdominal aortic aneurym (TAAA) repair in patients with Marfan syndrome. During a six year period, 206 patients underwent DTAA and TAAA repair. In 22 patients, Marfan syndrome was confirmed. The median age was 40 years with a range between 18 and 57 years. The extend of the aneurysms included 6 DTAA (1 with total arch, 2 with distal hemi-arch), 11 type II TAAA (2 with total arch, 3 with distal hemi-arch), 4 type III and one type IV TAAA. All patients suffered from previous type A (n=6) or type B (n=16) aortic dissection and 15 already underwent aortic procedures like Bentall (n=7) and ascending aortic replacement (n=8). All patients were operated on according to the standard protocol with cerebrospinal fluid drainage, distal aortic and selective organ perfusion and monitoring motor evoked potentials. In patients undergoing simultaneous arch replacement (via left thoracotomy), transcranial Doppler and EEG assessed cerebral physiology during antegrade brain perfusion. In four patients circulatory arrest under moderate hypothermia was required. In-hospital mortality did not occur. Major postoperative complications like paraplegia, renal failure, stroke and myocardial infarction were not encountered. Mean pre-operative creatinine level was 125mmol/L, which peaked to a mean maximal level of 130 and returned to 92mmol/L at discharge. Median intubation time was 1.5 days (range 0.33-30 days). Other complications included bleeding requiring surgical intervention (n=1), arrhythmia (n=2), pneumonia (n=2) and respiratory distress syndrome (n=1). At a median follow-up of 38 months all patients were alive. Using CT surveillance, new or false aneurysms were not detected, except in one patient who developed a visceral patch aneurysm six years after open type II repair. Surgical repair of descending and thoracoabdominal aortic aneurysms provides excellent short- and mid-term results in

  15. [Pseudomeigs syndrome in a patient with Krukenberg's tumor].

    Science.gov (United States)

    Bayod, M J Herráiz; Carlón, M Elorz; Idoate, M A

    2007-01-01

    We report the case of a fiftyone-year-old woman with a past medical history of Linfoma no Hodking and a gastric adenocarcinoma with signet ring cells. She came to our institution with a twenty month history of dysnea secondary to pleural effussion, bilateral lower extremity edema and probably had ascitis. On CT and US two bilateral pelvic masses were found and biopsied. The anatomopathological analysis showed bilateral ovarian implants from signet ring cell adenocarcinoma (Krukenberg tumor). This patient developed a PseudoMeigs syndrome consisting on malignant ovarian tumor asociated with ascitis and pleural effusion without malignant cells. Oncological patients who present with ascitis and benign pleural effusion, the diagnosis of PseudoMeigs syndrome should be considered.

  16. Patient with confirmed LEOPARD syndrome developing multiple melanoma

    OpenAIRE

    Colmant, Caroline; Franck, Deborah; Marot, Liliane; Matthijs, Gert; Sznajer, Yves; Blomme, Sandrine; Tromme, Isabelle

    2018-01-01

    LEOPARD syndrome, also known as Gorlin syndrome II, cardiocutaneous syndrome, lentiginosis profusa syndrome, Moynahan syndrome, was more recently coined as Noonan syndrome with multiple lentigines (NSML), inside the RASopathies. Historically, the acronym LEOPARD refers to the presence of distinctive clinical features such as: lentigines (L), electrocardiographic/conduction abnormalities (E), ocular hypertelorism (O), pulmonary stenosis (P), genital abnormalities (A), retardation of growth (R)...

  17. X-derived marker chromosome in patient with mosaic Turner syndrome and Dandy-Walker syndrome: a case report

    OpenAIRE

    Telepova, Alena S.; Romanenko, Svetlana A.; Lemskaya, Natalya A.; Maksimova, Yulia V.; Shorina, Asia R.; Yudkin, Dmitry V.

    2017-01-01

    Background Small supernumerary marker chromosomes can be derived from autosomes and sex chromosomes and can accompany chromosome pathologies, such as Turner syndrome. Case presentation Here, we present a case report of a patient with mosaic Turner syndrome and Dandy-Walker syndrome carrying a marker chromosome. We showed the presence of the marker chromosome in 33.8% of blood cells. FISH of the probe derived from the marker chromosome by microdissection revealed that it originated from the ce...

  18. Traditional Chinese Medicine Syndromes for Essential Hypertension: A Literature Analysis of 13,272 Patients

    Directory of Open Access Journals (Sweden)

    Jie Wang

    2014-01-01

    Full Text Available Background. To simplify traditional Chinese medicine syndrome differentiation and allow researchers to master syndrome differentiation for hypertension, this paper retrospectively studied the literature and analyzed syndrome elements corresponding to hypertension syndromes. Methods. Six databases including PubMed, EMBASE, Chinese Bio-Medical Literature Database, Chinese National Knowledge Infrastructure, Chinese Scientific Journal Database, and Wan-fang Data were searched from 1/January/2003 to 30/October/2013. We included all clinical literature testing hypertension syndromes and retrospectively studied the hypertension literature published from 2003 to 2013. Descriptive statistics calculated frequencies and percentages. Results. 13,272 patients with essential hypertension were included. Clinical features of hypertension could be attributed to 11 kinds of syndrome factors. Among them, seven syndrome factors were excess, while four syndrome factors were deficient. Syndrome targets were mainly in the liver and related to the kidney and spleen. There were 33 combination syndromes. Frequency of single-factor syndromes was 31.77% and frequency of two-factor syndromes was 62.26%. Conclusions. Excess syndrome factors of hypertension patients include yang hyperactivity, blood stasis, phlegm turbidity, internal dampness, and internal fire. Deficient syndrome factors of hypertension patients are yin deficiency and yang deficiency. Yin deficiency with yang hyperactivity, phlegm-dampness retention, and deficiency of both yin and yang were the three most common syndromes in clinical combination.

  19. Is refeeding syndrome relevant for critically ill patients?

    Science.gov (United States)

    Koekkoek, Wilhelmina A C; Van Zanten, Arthur R H

    2018-03-01

    To summarize recent relevant studies regarding refeeding syndrome (RFS) in critically ill patients and provide recommendations for clinical practice. Recent knowledge regarding epidemiology of refeeding syndrome among critically ill patients, how to identify ICU patients at risk, and strategies to reduce the potential negative impact on outcome are discussed. RFS is a potentially fatal acute metabolic derangement that ultimately can result in marked morbidity and even mortality. These metabolic derangements in ICU patients differ from otherwise healthy patients with RFS, as there is lack of anabolism. This is because of external stressors inducing a hypercatabolic response among other reasons also reflected by persistent high glucagon despite initiation of feeding. Lack of a proper uniform definition complicates diagnosis and research of RFS. However, refeeding hypophosphatemia is commonly encountered during critical illness. The correlations between risk factors proposed by international guidelines and the occurrence of RFS in ICU patients remains unclear. Therefore, regular phosphate monitoring is recommended. Based on recent trials among critically ill patients, only treatment with supplementation of electrolytes and vitamins seems not sufficient. In addition, caloric restriction for several days and gradual increase of caloric intake over days is recommendable.

  20. Radial tunnel syndrome. Findings and treatment in 17 patients

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    Gustavo Alberto Breglia

    2015-05-01

    Full Text Available Backround Radial tunnel syndrome is a condition secondary to the intermittent entrapment of the posterior interosseous nerve between superficial and deep mass of short supinator adjacent structures, such as vessels and fascias. The purpose of this study was to identify the anatomical structures that produce the eventual compression, to establish and communicate the differences in the subjective pain perception before and after the release of the posterior interosseous nerve in the radial tunnel. Method Between 2009 and 2014, 17 patients underwent surgical treatment by posterior interosseous nerve release. We used the approach between the first external radial and brachioradialis. Patients were assessed by visual analogue scale for pain intensity before surgery and at week 6, and according to the Roles and Maudsley functional criteria. Results The causes of posterior interosseous nerve compression were fibrous band of short supinator (arcade of Frohse (7 cases, recurrent vessels (4 cases, compression by the mass of the superficial portion of the short supinator muscle (2 cases and secondary compression by extensor carpi radialis brevis tendon (4 cases. Results were excellent (4 patients, good (10 patients and fair (3 patients. Patients treated through the Labor Risk Insurance had worse outcomes than those who were not covered by this system. Conclusions Radial tunnel syndrome is a condition that must be taken into account when there is refractory lateral epicondylalgia. This disease has a marked effect in patients with labor conflict, which may bias the outcome of treatment.

  1. Cardiometabolic risk in patients with polycystic ovary syndrome.

    Science.gov (United States)

    Ozegowska, Katarzyna; Pawelczyk, Leszek

    2015-11-01

    Polycystic ovary syndrome (PCOS) is a common endocrinopathy in premenopausal women, associated with risk of metabolic syndrome and cardiovascular disease (CVD). CVD risk evaluation is recommended for PCOS patients. This study aimed to evaluate the risk of CVD in PCOS patients and to identify the best predictors for metabolic and cardiovascular disturbances. The study included 169 PCOS patients and 110 healthy women in reproductive age. We estimated cardiovascular risk according to American Heart Association and Androgen Excess-PCOS Society criteria that classified patients as metabolically unhealthy (MU) or metabolically healthy (MH). The PCOS group had significantly higher body mass index (BMI), waist circumference, and waist-to-hip ratio (P PCOS patients (8.9%). No obesity was observed in the control group. Waist circumference ≥ 80 cm was presented in 44% of PCOS patients in comparison to 14.5% of control participants (P PCOS population (P PCOS-MH group had the highest high-density lipoprotein (HDL) levels. ROC curves were used to indicate parameters diagnosing metabolically unhealthy women and revealed that WC, BMI and HC seem to be the strongest predictors of metabolic disturbances in PCOS but in the healthy population in reproductive age biochemical findings such as low HDL or increased fasting glycemia presented stronger predictive value than patients' anthropometric features. Physicians need to remember to adopt different diagnostic approach while seeking metabolic complications in these different groups of women.

  2. Remote clinical prognosis in patients with coronary X syndrome

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    Sebov D.M.

    2015-09-01

    Full Text Available The article analyzes data of 3234 coronary angiographies with established coronary X syndrome (CXS in 217 cases, herewith expressed tortuosity of coronary arteries (ETCA was found out in 148 (more than 2/3 of cases. A 5-years’ analysis of cardio-vascular events (CVE in patients with CXS in comparison with the group of IHD patients and initial atherosclerosis of coronary arteries was made. Absence of reliable difference of developing severe cardio-vascular events (SCVE bet¬ween patients with initial atherosclerosis and CXS was proved. Risk of CVE development was significantey higher in patients with ETCA, OR=4,93; 95% (0,62; 3929. Patients with CXS had higher risk of severe arrhythmias development as compared with IHD patients with initial atherosclerosis: OR=2,36 (1,01; 5,56. There was no reliable difference between lethality of any causes and number of coronary interventions in all groups.

  3. Definition and epidemiology of acute respiratory distress syndrome.

    Science.gov (United States)

    Rezoagli, Emanuele; Fumagalli, Roberto; Bellani, Giacomo

    2017-07-01

    Fifty years ago, Ashbaugh and colleagues defined for the first time the acute respiratory distress syndrome (ARDS), one among the most challenging clinical condition of the critical care medicine. The scientific community worked over the years to generate a unified definition of ARDS, which saw its revisited version in the Berlin definition, in 2014. Epidemiologic information about ARDS is limited in the era of the new Berlin definition, and wide differences are reported among countries all over the world. Despite decades of study in the field of lung injury, ARDS is still so far under-recognized, with 2 out of 5 cases missed by clinicians. Furthermore, although advances of ventilator strategies in the management of ARDS associated with outcome improvements-such as protective mechanical ventilation, lower driving pressure, higher PEEP levels and prone positioning-ARDS appears to be undertreated and mortality remains elevated up to 40%. In this review, we cover the history that led to the current worldwide accepted Berlin definition of ARDS and we summarize the recent data regarding ARDS epidemiology.

  4. Clinical profile of patients with fibromyalgia syndrome

    Directory of Open Access Journals (Sweden)

    Andrei Pereira Pernambuco

    Full Text Available Abstract Introduction: The new diagnostic criteria for fibromyalgia (FM include the presence of chronic, widespread pain associated with other symptoms such as fatigue, sleep disturbance, anxiety and depression. All these symptoms should be considered when thinking and clinical decision making of physiotherapists dealing with FM. However, it is clear that the other symptoms that accompany the pain are often neglected. Objective: To measure the levels of fatigue, sleep disturbances, anxiety and depression in patients with FM and compare them to levels found in healthy controls. Methods: Forty-six women diagnosed with FM and 30 healthy controls participated in the study. The levels of each of the symptoms were assessed by four validated questionnaires in Brazil (Piper Fatigue Scale - Revised, Pittsburgh Sleep Quality Index, Beck Anxiety Inventory and the Beck Depression Inventory. Statistical analysis was performed using GraphPad Prism software and all tests used a significance level of 5% (α = 0.05. Results: FM patients had significantly elevated levels of fatigue (p = 0.0005, sleep disturbances (p = 0.003, anxiety (p = 0.0012 and depression (p = 0.0003 compared to healthy controls. Symptoms fatigue and depression correlated strongly and positively with one another and with other symptoms evaluated. Conclusion: The other symptoms that comprise the clinical picture of FM need be considered not only in order to recover the health of patients, but above all in an attempt to preserve it and promote it.

  5. [Cochlear implantation in patients with Waardenburg syndrome type II].

    Science.gov (United States)

    Wan, Liangcai; Guo, Menghe; Chen, Shuaijun; Liu, Shuangriu; Chen, Hao; Gong, Jian

    2010-05-01

    To describe the multi-channel cochlear implantation in patients with Waardenburg syndrome including surgeries, pre and postoperative hearing assessments as well as outcomes of speech recognition. Multi-channel cochlear implantation surgeries have been performed in 12 cases with Waardenburg syndrome type II in our department from 2000 to 2008. All the patients received multi-channel cochlear implantation through transmastoid facial recess approach. The postoperative outcomes of 12 cases were compared with 12 cases with no inner ear malformation as a control group. The electrodes were totally inserted into the cochlear successfully, there was no facial paralysis and cerebrospinal fluid leakage occurred after operation. The hearing threshold in this series were similar to that of the normal cochlear implantation. After more than half a year of speech rehabilitation, the abilities of speech discrimination and spoken language of all the patients were improved compared with that of preoperation. Multi-channel cochlear implantation could be performed in the cases with Waardenburg syndrome, preoperative hearing and images assessments should be done.

  6. Effects of canrenone in patients with metabolic syndrome.

    Science.gov (United States)

    Derosa, Giuseppe; Bonaventura, Aldo; Bianchi, Lucio; Romano, Davide; D'Angelo, Angela; Fogari, Elena; Maffioli, Pamela

    2013-11-01

    Metabolic syndrome is becoming a common disease due to a rise in obesity rates among adults. The aim was to evaluate the effects of canrenone compared to placebo on metabolic and inflammatory parameters in patients affected by metabolic syndrome. A total of 145 patients were treated with placebo or canrenone, 50 mg/day, for 3 months and then 50 mg b.i.d. till the end of the study. Blood pressure, body weight, body mass index, fasting plasma glucose (FPG), fasting plasma insulin, HOMA-IR, lipid profile, plasma aldosterone, brain natriuretic peptide, high-sensitivity C-reactive protein (Hs-CRP), tumor necrosis factor-α (TNF-α) and M value were evaluated. A decrease of blood pressure was observed in canrenone group compared to baseline; moreover, systolic blood pressure value recorded after 6 months of canrenone therapy was lower than the one recorded with placebo. Canrenone gave a significant decrease of FPI and HOMA index, and an increase of M value both compared to baseline and to placebo. Canrenone also decreased triglycerides and FPG was not observed with placebo. Canrenone also decreased plasma aldosterone, Hs-CRP and TNF-α compared to baseline and to placebo. Canrenone seems to be effective in reducing some factors involved in metabolic syndrome and in improving insulin-resistance and the inflammatory state observed in these patients.

  7. The characteristics of the patients with Rett syndrome: Case series

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    Dutina Aleksandra

    2016-01-01

    Full Text Available Rett syndrome is a neurodevelopmental disorder caused by mutation in the gene MECP2 on the X chromosome. The main clinical characteristics are breathing disorders, epileptic seizures, scoliosis, walking disability and constipation with frequent growth failure and malnutrition. Our transversal study included 29 female patients with Rett syndrome treated in the Institute for Mother and Child Health Care ''Dr Vukan Čupić'' in Belgrade. The patients were anthropometrically measured by their height and weight and caluculating body mass index. The degree of scolisios, the frequency of epileptic seizures, walking ability and grade of respiratory dysfunction were assessed based on the clinical scales for disease severity estimation. The statistical data analysis was done in the software package PASW Statistics 18. The average age of the patients was 145.5±90.1 months. The average height was 125.2±22.1cm, the average weight 27.6±12.3kg, and the average body mass index was 16.6±3.5kg/m2. The average z scores for height, weight and BMI-for-age were statistically significantly lower in comparison to general population, which is given with negative z scores. There was the statistically significant negative coorelation between the age of patients and the average z scores for height-for-age (r=-0.464 p=0.011, weight-for-age (r=-0.433 p=0.019 and BMI-for-age (r=-0.433 p=0.019. The statistically significant negative coorelation was found between the degree of scoliosis and the average z scores for height and weight-for-age, but not with the average z score for BMI-for-age. The growth failure and malnutrition represent significant problems which are found in Rett syndrome. The detection of factors affecting the growth and nutrition in patients with Rett syndrome could greatly contribute to the improvement of the quality of life in the patients with Rett syndrome and perhaps lead to the correction of the approach of symptomatic and supportive therapy.

  8. Colorectal choriocarcinoma in a patient with probable Lynch syndrome

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    Viktor Hendrik Koelzer

    2016-11-01

    Full Text Available Background: Personalized therapy of colorectal cancer (CRC is influenced by morphological, molecular and host-related factors. Here we report the comprehensive clinicopathological and molecular analysis of a pure extra-gestational colorectal choriocarcinoma in a patient with probable Lynch syndrome.Case presentation: A 61 year old female with history of gastric cancer at age 36 presented with a transmurally invasive tumor of the right hemicolon and liver metastasis. A right hemicolectomy was performed. Histopathological analysis showed a mixed trophoblastic and syncytiotrophoblastic differentiation, consistent with choriocarcinoma. Disease progression was rapid under oxaliplatin, capecitabine, irinotecan (XELOXIRI and bevacizumab. Molecular phenotyping identified loss of the mismatch-repair (MMR protein PMS2, microsatellite instability, a lack of MLH1 promoter methylation and lack of of BRAF mutation suggestive of Lynch-Syndrome. Targeted next generation sequencing revealed an Ataxia Telangiectasia Mutated (ATM p.P604S missense mutation. A bleomycin, etoposide and cisplatin (BEP treatment protocol targeting germ-cell neoplasia lead to disease remission and prolonged survival of 34 months.Conclusions: Comprehensive immunohistochemical and genetic testing is essential to identify uncommon cancers possibly related to Lynch syndrome. For rare tumors, personalized therapeutic approaches should take both molecular and morphological information into account.Key words: Colorectal cancer, choriocarcinoma, histopathology, prognostic factors, Lynch syndrome, microsatellite instability, ataxia telangiectasia mutated, molecular pathology, next generation sequencing, personalized medicine

  9. Clinical, radiological and imunogenectical study in patients with Reiter's Syndrome

    International Nuclear Information System (INIS)

    Souza Meirelles, E. de.

    1987-01-01

    This study puspose was to investigate the clinical, radiological and immunogenetical parameters from a brazilian Reiter's Syndrome population. Twenty Reiter's Syndrome patients from ''Hospital das Clinicas da Faculdade de Medicina da Universidade de Sao Paulo'' were prospectivelly studied in their demographical (sex, race, age at study, age at disease onset and disease duration), epidemiological (family history), clinical (general, articular, mucocutaneous, genitourinary, ocular and intestinal manifestations besides functional capacity at study), radiological (sacro-iliitis, spondylitis and calcaneal spur) and immunogenetical (HLA loci A, B and DR typing). The technique employed in the 61 aloantigens from loci HLA A (17), B (34) and DR (10) typing was Terasaki microlymphocitotoxicity modified by Danilovs, had being performed in the ''Laboratorio de Imunogenetica da Faculdade de Medicina da Universidade de Sao Paulo''. We concluded that the clinical, radiological and immunogenetical Reiter's Syndrome expression in the brazilian population is similar to the others north american or european already studied populations and that the probability of one brazilian HLA B27 positie individual to develop Reiter's Syndrome is 19 fold larger when compared to one brazilian HLA B27 negative individual. (author) [pt

  10. Colorectal Cancer Risk in Patients With Lynch Syndrome and Inflammatory Bowel Disease.

    Science.gov (United States)

    Derikx, Lauranne A A P; Smits, Lisa J T; van Vliet, Shannon; Dekker, Evelien; Aalfs, Cora M; van Kouwen, Mariëtte C A; Nagengast, Fokko M; Nagtegaal, Iris D; Hoogerbrugge, Nicoline; Hoentjen, Frank

    2017-03-01

    Lynch syndrome and inflammatory bowel diseases (IBD) are associated with an increased risk of colorectal cancer (CRC). However, it is not clear whether the risk of CRC is even higher for patients with a combination of Lynch syndrome and IBD. We investigated the risk for CRC in this subgroup by establishing a Lynch syndrome cohort from the Radboud University Medical Center (Nijmegen, The Netherlands) and the Academic Medical Center (Amsterdam, The Netherlands). Patients with heterozygous germline mutations in MLH1, MSH2 (and EPCAM deletion-mediated MSH2 methylation), MSH6, or PMS2 who were tested and/or treated from 1998 through 2014 were included. Patients who developed IBD were identified by linkage of this cohort to the Dutch nationwide Pathology Registry (PALGA). Subsequently, we compared the risk of CRC between Lynch syndrome patients with IBD and without IBD. Of 1046 patients with Lynch syndrome, 15 developed IBD (1.4%). Patients with Lynch syndrome and IBD were significantly younger (median age, 38.0 y) than patients with Lynch syndrome without IBD (median age, 52.0 y; P = .001). Nevertheless, a similar proportion of patients in each group developed CRC: 4 of the 15 patients (26.7%) with Lynch syndrome and IBD compared with 311 of the 1031 patients (30.2%) with Lynch syndrome without IBD. Patients with Lynch syndrome and IBD developed CRC at a younger age (median age, 36.0 y) than patients with Lynch syndrome without IBD (median age, 46.0 y; P = .045). However, the cumulative incidence of CRC was similar between groups (P = .121). All patients with Lynch syndrome and IBD who developed CRC had ulcerative colitis, producing a higher cumulative incidence of CRC for this IBD subgroup (P Lynch syndrome and IBD develop CRC risk at a younger age than patients without IBD; patients with ulcerative colitis are at especially high risk. Copyright © 2017 AGA Institute. Published by Elsevier Inc. All rights reserved.

  11. Hematological abnormalities in adult patients with Down's syndrome.

    LENUS (Irish Health Repository)

    McLean, S

    2012-02-01

    BACKGROUND: There is a paucity of data regarding hematological abnormalities in adults with Down\\'s syndrome (DS). AIMS: We aimed to characterize hematological abnormalities in adult patients with DS and determine their long-term significance. METHODS: We retrospectively studied a cohort of nine DS patients referred to the adult hematology service in our institution between May 2001 and April 2008. Data collected were: full blood count (FBC), comorbidities, investigations performed, duration of follow-up and outcome to most recent follow-up. RESULTS: Median follow-up was 26 months (9-71). Of the nine patients, two had myelodysplastic syndrome (MDS) at presentation. Of these, one progressed, with increasing marrow failure, and requiring support with transfusions and gCSF. The remaining eight patients, with a variety of hematological abnormalities including leukopenia, macrocytosis, and thrombocytopenia, had persistently abnormal FBCs. However there was no evidence of progression, and no patient has evolved to acute myeloid leukemia (AML). CONCLUSIONS: MDS is a complication of DS and may require supportive therapy. However, minor hematological abnormalities are common in adult DS patients, and may not signify underlying marrow disease.

  12. [Attachment Representation and Emotion Regulation in Patients with Burnout Syndrome].

    Science.gov (United States)

    Söllner, Wolfgang; Behringer, Johanna; Böhme, Stephanie; Stein, Barbara; Reiner, Iris; Spangler, Gottfried

    2016-06-01

    Burnout describes a syndrome of exhaustion resulting from insufficient coping with work-related distress. We investigated if patients that are being clinically treated for burnout show insecure and unresolved attachment representation more often compared with healthy controls. 50 out of 60 consecutive burnout patients participated in the study. Mental representation of attachment was measured by using the Adult Attachment Interview. Additionally, we administered the Self Report Questionnaire to Assess Emotional Experience and Emotion Regulation and several burnout specific questionnaires. A population sample was used as control group. Burnout patients were classified as insecurely attached significantly more often than controls. Unresolved attachment status concerning loss or trauma was found significantly more often within the burnout sample. Patients with insecure attachment representation reported a lower subjective significance of work. Patients with avoidant insecure attachment showed more depersonalisation. Patients with unresolved loss/trauma reported less social support. They showed more passive-negative emotion experience and emotion regulation characterized by externalization. The results of the study suggest that an insecure or unresolved attachment representation might constitute an intrapersonal risk factor for the development of burnout syndrome. © Georg Thieme Verlag KG Stuttgart · New York.

  13. Restless legs syndrome in patients with sequelae of poliomyelitis.

    Science.gov (United States)

    Kumru, Hatice; Portell, Enric; Barrio, Manuela; Santamaria, Joan

    2014-10-01

    No studies have examined the association between RLS and the sequelae of poliomyelitis (PM). We studied the frequency and severity of RLS in a group of consecutive patients with the sequelae of poliomyelitis (PM) and the effect of treatment with dopaminergic drugs. A diagnosis of RLS was made according to the criteria of the International RLS Study Group, and severity was assessed by the RLS rating scale. Information on sex, age, age at onset, site affected by PM, disease duration of PM, and history of post-polio syndrome (pPS) was obtained in a cohort of 52 PM patients. The mean age was 55.9 ± 6.5 years; 39 patients had post-polio syndrome (75%). RLS was diagnosed in 21 (40.4%) patients. Sixteen of the 21 patients (76.2%) with RLS had pPS, which was similar to the non-RLS group (74.2% patients wit