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  1. Acute respiratory failure mimicking acute respiratory distress syndrome due to parenchymal infiltration by metastatic melanoma

    PubMed Central

    2013-01-01

    Abstract Malignant melanoma is the most aggressive form of skin cancer and carries a predisposition for metastasis to many different organs. Pulmonary dissemination is common, most often presenting as multiple discrete pulmonary nodules. While a variety of other intrathoracic patterns can occur, diffuse parenchymal infiltration causing acute respiratory failure is an extremely rare manifestation of metastatic disease. We present a case of an otherwise healthy man who developed rapidly progressive respiratory failure mimicking acute respiratory distress syndrome due to melanomatous infiltration of the lung parenchyma and airways. PMID:25006412

  2. Fatal respiratory distress syndrome due to coronavirus infection in a child with severe combined immunodeficiency.

    PubMed

    Szczawinska-Poplonyk, Aleksandra; Jonczyk-Potoczna, Katarzyna; Breborowicz, Anna; Bartkowska-Sniatkowska, Alicja; Figlerowicz, Magdalena

    2013-09-01

    Coronaviruses have been demonstrated to contribute substantially to respiratory tract infections among the child population. Though infected children commonly present mild upper airway symptoms, in high-risk patients with underlying conditions, particularly in immunocompromised children these pathogens may lead to severe lung infection and extrapulmonary disorders. In this paper, we provide the first report of the case of a 15-month-old child with severe combined immunodeficiency and coronavirus HKU1-related pneumonia with fatal respiratory distress syndrome.

  3. [Acute respiratory distress syndrome].

    PubMed

    Hecker, M; Weigand, M A; Mayer, K

    2012-05-01

    Acute respiratory distress syndrome (ARDS) is the clinical manifestation of an acute lung injury caused by a variety of direct and indirect injuries to the lung. The cardinal clinical feature of ARDS, refractory arterial hypoxemia, is the result of protein-rich alveolar edema with impaired surfactant function, due to vascular leakage and dysfunction with consequently impaired matching of ventilation to perfusion. Better understanding of the pathophysiology of ARDS has led to the development of novel therapies, pharmacological strategies, and advances in mechanical ventilation. However, protective ventilation is the only confirmed option in ARDS management improving survival, and few other therapies have translated into improved oxygenation or reduced ventilation time. The development of innovative therapy options, such as extracorporeal membrane oxygenation, have the potential to further improve survival of this devastating disease.

  4. Acute respiratory distress syndrome.

    PubMed

    Gibbons, Cynthia

    2015-01-01

    Acute respiratory distress syndrome (ARDS) is a life-threatening condition with multiple causes and a high mortality rate. Approximately 150,000 cases are reported in the United States annually, making ARDS a public health concern. Management of the condition is complex because of its severity, and medical imaging is essential for both the diagnosis and management of ARDS. This article introduces common signs, symptoms, risk factors, and causes of ARDS. Diagnostic criteria, histopathology, treatment strategies, and prognostic information also are discussed. The article explains the value of medical imaging studies of ARDS, especially radiography, computed tomography, and ultrasonography.

  5. Adult respiratory distress syndrome.

    PubMed

    Cutts, S; Talboys, R; Paspula, C; Prempeh, E M; Fanous, R; Ail, D

    2017-01-01

    Adult respiratory distress syndrome (ARDS) has now been described as a sequela to such diverse conditions as burns, amniotic fluid embolism, acute pancreatitis, trauma, sepsis and damage as a result of elective surgery in general. Patients with ARDS require immediate intubation, with the average patient now being ventilated for between 8 and 11 days. While the acute management of ARDS is conducted by the critical care team, almost any surgical patient can be affected by the condition and we believe that it is important that a broader spectrum of hospital doctors gain an understanding of the nature of the pathology and its current treatment.

  6. [Acute respiratory distress syndrome].

    PubMed

    Matĕjovic, M; Novák, I; Srámek, V; Rokyta, R; Hora, P; Nalos, M

    1999-04-26

    Acute respiratory distress syndrome (ARDS) is the general term used for severe acute respiratory failure of diverse aetiology. It is associated with a high morbidity, mortality (50-70%), and financial costs. Regardless of aetiology, the basic pathogenesis of ARDS is a systemic inflammatory response leading to a diffuse inflammatory process that involves both lungs, thus causing diffuse alveolar and endothelial damage with increased pulmonary capillary permeability and excessive extravascular lung water accumulation. ARDS is commonly associated with sepsis and multiple organ failure. The clinical picture involves progressive hypoxaemia, radiographic evidence of pulmonary oedema, decreased lung compliance and pulmonary hypertension. Despite the scientific and technological progress in critical care medicine, there is no specific ARDS therapy available at the moment and its management remains supportive. Therapeutic goals include resolution of underlying conditions, maintenance of acceptable gas exchange and tissue oxygenation and prevention of iatrogenic lung injury. Many new specific therapeutic strategies have been developed, however, most of them require further scientific evaluation. The paper reviews definition, basic pathogenesis and pathophysiology of ARDS and discusses current concepts of therapeutic possibilities of ARDS.

  7. Time-dependent changes in pulmonary surfactant function and composition in acute respiratory distress syndrome due to pneumonia or aspiration

    PubMed Central

    Schmidt, Reinhold; Markart, Philipp; Ruppert, Clemens; Wygrecka, Malgorzata; Kuchenbuch, Tim; Walmrath, Dieter; Seeger, Werner; Guenther, Andreas

    2007-01-01

    Background Alterations to pulmonary surfactant composition have been encountered in the Acute Respiratory Distress Syndrome (ARDS). However, only few data are available regarding the time-course and duration of surfactant changes in ARDS patients, although this information may largely influence the optimum design of clinical trials addressing surfactant replacement therapy. We therefore examined the time-course of surfactant changes in 15 patients with direct ARDS (pneumonia, aspiration) over the first 8 days after onset of mechanical ventilation. Methods Three consecutive bronchoalveolar lavages (BAL) were performed shortly after intubation (T0), and four days (T1) and eight days (T2) after intubation. Fifteen healthy volunteers served as controls. Phospholipid-to-protein ratio in BAL fluids, phospholipid class profiles, phosphatidylcholine (PC) molecular species, surfactant proteins (SP)-A, -B, -C, -D, and relative content and surface tension properties of large surfactant aggregates (LA) were assessed. Results At T0, a severe and highly significant reduction in SP-A, SP-B and SP-C, the LA fraction, PC and phosphatidylglycerol (PG) percentages, and dipalmitoylation of PC (DPPC) was encountered. Surface activity of the LA fraction was greatly impaired. Over time, significant improvements were encountered especially in view of LA content, DPPC, PG and SP-A, but minimum surface tension of LA was not fully restored (15 mN/m at T2). A highly significant correlation was observed between PaO2/FiO2 and minimum surface tension (r = -0.83; p < 0.001), SP-C (r = 0.64; p < 0.001), and DPPC (r = 0.59; p = 0.003). Outcome analysis revealed that non-survivors had even more unfavourable surfactant properties as compared to survivors. Conclusion We concluded that a profound impairment of pulmonary surfactant composition and function occurs in the very early stage of the disease and only gradually resolves over time. These observations may explain why former surfactant replacement

  8. Neonatal respiratory distress syndrome

    MedlinePlus

    ... term complications may develop due to: Too much oxygen. High pressure delivered to the lungs. More severe disease or immaturity. RDS can be associated with inflammation that causes lung ... when the brain or other organs did not get enough oxygen.

  9. Pharmacotherapy for acute respiratory distress syndrome.

    PubMed

    Shafeeq, Hira; Lat, Ishaq

    2012-10-01

    Acute lung injury (ALI) and acute respiratory distress syndrome (ARDS) represent a continuum of a clinical syndrome of respiratory failure due to refractory hypoxia. Acute respiratory distress syndrome is differentiated from ALI by a greater degree of hypoxemia and is associated with higher morbidity and mortality. The mortality for ARDS ranges from 22-41%, with survivors usually requiring long-term rehabilitation to regain normal physiologic function. Numerous pharmacologic therapies have been studied for prevention and treatment of ARDS; however, studies demonstrating clear clinical benefit for ARDS-related mortality and morbidity are limited. In this focused review, controversial pharmacologic therapies that have demonstrated, at minimum, a modest clinical benefit are discussed. Three pharmacologic treatment strategies are reviewed in detail: corticosteroids, fluid management, and neuromuscular blocking agents. Use of corticosteroids to attenuate inflammation remains controversial. Available evidence does not support early administration of corticosteroids. Additionally, administration after 14 days of disease onset is strongly discouraged. A liberal fluid strategy during the early phase of comorbid septic shock, balanced with a conservative fluid strategy in patients with ALI or ARDS during the postresuscitation phase, is the optimum approach for fluid management. Available evidence supports an early, short course of continuous-infusion cisatracurium in patients presenting with severe ARDS. Evidence of safe and effective pharmacologic therapies for ARDS is limited, and clinicians must be knowledgeable about the areas of controversies to determine application to patient care.

  10. Pathobiology of acute respiratory distress syndrome.

    PubMed

    Sapru, Anil; Flori, Heidi; Quasney, Michael W; Dahmer, Mary K

    2015-06-01

    The unique characteristics of pulmonary circulation and alveolar-epithelial capillary-endothelial barrier allow for maintenance of the air-filled, fluid-free status of the alveoli essential for facilitating gas exchange, maintaining alveolar stability, and defending the lung against inhaled pathogens. The hallmark of pathophysiology in acute respiratory distress syndrome is the loss of the alveolar capillary permeability barrier and the presence of protein-rich edema fluid in the alveoli. This alteration in permeability and accumulation of fluid in the alveoli accompanies damage to the lung epithelium and vascular endothelium along with dysregulated inflammation and inappropriate activity of leukocytes and platelets. In addition, there is uncontrolled activation of coagulation along with suppression of fibrinolysis and loss of surfactant. These pathophysiological changes result in the clinical manifestations of acute respiratory distress syndrome, which include hypoxemia, radiographic opacities, decreased functional residual capacity, increased physiologic deadspace, and decreased lung compliance. Resolution of acute respiratory distress syndrome involves the migration of cells to the site of injury and re-establishment of the epithelium and endothelium with or without the development of fibrosis. Most of the data related to acute respiratory distress syndrome, however, originate from studies in adults or in mature animals with very few studies performed in children or juvenile animals. The lack of studies in children is particularly problematic because the lungs and immune system are still developing during childhood and consequently the pathophysiology of pediatric acute respiratory distress syndrome may differ in significant ways from that seen in acute respiratory distress syndrome in adults. This article describes what is known of the pathophysiologic processes of pediatric acute respiratory distress syndrome as we know it today while also presenting the much

  11. Miliary tuberculosis and adult respiratory distress syndrome.

    PubMed

    Piqueras, A R; Marruecos, L; Artigas, A; Rodriguez, C

    1987-01-01

    Although, miliary tuberculosis is an unusual cause of severe acute respiratory failure, we describe nine patients with miliary tuberculosis who developed adult respiratory distress syndrome. This complication occurred in seven patients despite treatment with antituberculous drugs. In two patients who developed the syndrome, miliary tuberculosis was diagnosed only at postmortem. The presence of pulmonary hypertension in all cases and disseminated intravascular coagulation in seven cases suggests a possible pathophysiologic relationship with severe pulmonary vascular damage. The high mortality rate (88.8%) was associated with nonpulmonary organ system failure. Miliary tuberculosis should be considered in patients with adult respiratory distress syndrome of unknown etiology, and simple diagnostic procedures such as sputum, bronchial brushing, and gastric examination should be followed by invasive diagnostic procedures to confirm this etiology. Since untreated miliary tuberculosis is usually fatal, early recognition of this disease is of great importance, and specific therapy may play a lifesaving role.

  12. [Acute respiratory distress syndrome in children].

    PubMed

    Stucki, P; Scalfaro, P; Parret, L; Wassenberg, J; Krähenbühl, J D; Curchod, P; Di Bernardo, S; Llor, J; Cotting, J

    2001-03-01

    The acute respiratory distress syndrome (ARDS) encountered in a child may be either due to a primary lung infection or may be secondary to a systemic inflammatory response of varying origin. Therapy is based on: 1) the mechanical ventilation strategy aimed at maintaining the functional residual capacity by alveolar recruitment using positive end expiratory pressure and to limit secondary pulmonary lesions by using small tidal volumes, 2) prone positioning as soon as sufficient stability is achieved; 3) optimizing tissue oxygen delivery by cardiac support; 4) correction of any other organ dysfunction. If this conventional approach is not sufficient experimental therapies may be tempted given the vital risk. For instance inhaled nitric oxide and high frequency oscillation ventilation may be a valuable support. Newer techniques, such as partial liquid ventilation, are being developed and could become useful therapeutic options. After the acute phase a close medical follow-up is mandatory. Because of the possibility of a chronic respiratory insufficiency with negative consequences on the right ventricular function, these patients may need long term oxygen therapy and diuretics. Cardiac echography helps orientation in maintaining or discontinuing this long term therapy by estimating the arterial pulmonary pressure.

  13. Prone position in patients with acute respiratory distress syndrome

    PubMed Central

    Setten, Mariano; Plotnikow, Gustavo Adrián; Accoce, Matías

    2016-01-01

    Acute respiratory distress syndrome occupies a great deal of attention in intensive care units. Despite ample knowledge of the physiopathology of this syndrome, the focus in intensive care units consists mostly of life-supporting treatment and avoidance of the side effects of invasive treatments. Although great advances in mechanical ventilation have occurred in the past 20 years, with a significant impact on mortality, the incidence continues to be high. Patients with acute respiratory distress syndrome, especially the most severe cases, often present with refractory hypoxemia due to shunt, which can require additional treatments beyond mechanical ventilation, among which is mechanical ventilation in the prone position. This method, first recommended to improve oxygenation in 1974, can be easily implemented in any intensive care unit with trained personnel. Prone position has extremely robust bibliographic support. Various randomized clinical studies have demonstrated the effect of prone decubitus on the oxygenation of patients with acute respiratory distress syndrome measured in terms of the PaO2/FiO2 ratio, including its effects on increasing patient survival. The members of the Respiratory Therapists Committee of the Sociedad Argentina de Terapia Intensiva performed a narrative review with the objective of discovering the available evidence related to the implementation of prone position, changes produced in the respiratory system due to the application of this maneuver, and its impact on mortality. Finally, guidelines are suggested for decision-making. PMID:27925054

  14. Prone position in patients with acute respiratory distress syndrome.

    PubMed

    Setten, Mariano; Plotnikow, Gustavo Adrián; Accoce, Matías

    2016-01-01

    Acute respiratory distress syndrome occupies a great deal of attention in intensive care units. Despite ample knowledge of the physiopathology of this syndrome, the focus in intensive care units consists mostly of life-supporting treatment and avoidance of the side effects of invasive treatments. Although great advances in mechanical ventilation have occurred in the past 20 years, with a significant impact on mortality, the incidence continues to be high. Patients with acute respiratory distress syndrome, especially the most severe cases, often present with refractory hypoxemia due to shunt, which can require additional treatments beyond mechanical ventilation, among which is mechanical ventilation in the prone position. This method, first recommended to improve oxygenation in 1974, can be easily implemented in any intensive care unit with trained personnel. Prone position has extremely robust bibliographic support. Various randomized clinical studies have demonstrated the effect of prone decubitus on the oxygenation of patients with acute respiratory distress syndrome measured in terms of the PaO2/FiO2 ratio, including its effects on increasing patient survival. The members of the Respiratory Therapists Committee of the Sociedad Argentina de Terapia Intensiva performed a narrative review with the objective of discovering the available evidence related to the implementation of prone position, changes produced in the respiratory system due to the application of this maneuver, and its impact on mortality. Finally, guidelines are suggested for decision-making.

  15. The Lung Surfactant System in Adult Respiratory Distress Syndrome.

    DTIC Science & Technology

    1979-12-01

    TITLE (and Subtitle) S. TYPE OF REPORT & PERIOD COVERo THE LUNG SURFACTANT SYSTEM IN ADULT RESPIRATORY Annual DISTRESS SYNDROME 6. PERFORMING ORO. REPORT...SURFACTANT SYSTEM IN ADULT RESPIRATORY DISTRESS SYNDROME - Annual Progress Report John U. Balis December 1979 Sponsored by: US ARMY MEDICAL RESEARCH AND...112-116, 1979. 6. Hallman, M., Feldman, B.H., Kirkpatrick, E. and Gluck, L.: Absence of phosphatidylglycerol (PG) in respiratory distress syndrome in

  16. Prone positioning in acute respiratory distress syndrome.

    PubMed

    Gibson, Kristy; Dufault, Marlene; Bergeron, Kathy

    2015-08-12

    Acute respiratory distress syndrome (ARDS) is a condition with a high morbidity and mortality rate, and treatment is often long and costly. Prone positioning is a rarely used intervention for patients with this syndrome, although research suggests it may be effective. A literature search was undertaken to examine the effects of prone positioning on oxygenation, morbidity and mortality in patients with ARDS. It revealed that prone positioning, when used with low tidal volume ventilation over an extended period, may reduce mortality rates in selected patients with severe ARDS. The selection of patients with severe ARDS for prone positioning should be done on a case-by-case basis to maximise benefits and minimise complications. Further research is required on the use of prone positioning in patients with severe ARDS to support or disclaim the therapy's use in practice, and to compare confounding variables such as ideal prone duration and mechanical versus manual pronation.

  17. Acute respiratory distress syndrome after cardiac surgery

    PubMed Central

    Rong, Lisa Q.; Di Franco, Antonino

    2016-01-01

    Acute respiratory distress syndrome (ARDS) is a leading cause of postoperative respiratory failure, with a mortality rate approaching 40% in the general population and 80% in the subset of patients undergoing cardiac surgery. The increased risk of ARDS in these patients has traditionally been associated with the use of cardiopulmonary bypass (CPB), the need for blood product transfusions, large volume shifts, mechanical ventilation and direct surgical insult. Indeed, the impact of ARDS in the cardiac population is substantial, affecting not only survival but also in-hospital length of stay and long-term physical and psychological morbidity. No patient undergoing cardiac surgery can be considered ARDS risk-free. Early identification of those at higher risk is crucial to warrant the adoption of both surgical and non-surgical specific preventative strategies. The present review focuses on epidemiology, risk assessment, pathophysiology, prevention and management of ARDS in the specific setting of patients undergoing cardiac surgery. PMID:27867583

  18. Drug-induced pulmonary edema and acute respiratory distress syndrome.

    PubMed

    Lee-Chiong, Teofilo; Matthay, Richard A

    2004-03-01

    Noncardiogenic pulmonary edema, and, to a lesser extent, acute respiratory distress syndrome (ARDS), are common clinical manifestations of drug-induced lung diseases. Clinical features and radiographic appearances are generally indistinguishable from other causes of pulmonary edema and ARDS. Typical manifestations include dyspnea, chest discomfort, tachypnea, and hypoxemia. Chest radiographs commonly reveal interstitial and alveolar filling infiltrates. Unlike pulmonary edema that is due to congestive heart failure, cardiomegaly and pulmonary vascular redistribution are generally absent in cases that are drug-related. Rare cases of drug-induced myocarditis with heart failure and pulmonary edema have been described. Results from laboratory evaluation and respiratory function tests are nonspecific.

  19. Acute Respiratory Distress: from syndrome to disease.

    PubMed

    Cardinal-Fernández, P; Correger, E; Villanueva, J; Rios, F

    2016-04-01

    The acute respiratory distress syndrome (ARDS) is currently one of the most important critical entities given its high incidence, rate of mortality, long-term sequelae and non-specific pharmacological treatment. The histological hallmark of ARDS is diffuse alveolar damage (DAD). Approximately 50% of ARDS patients present DAD, the rest is made up of a heterogeneous group of histological patterns, many of which correspond to a well-recognized disease. For that reason, if these patterns could be diagnosed, patients could benefit from a treatment. Recently, the effect of DAD in clinical and analytical evolution of ARDS has been demonstrated, so the classical approach to ARDS as an entity defined solely by clinical, radiological and gasometrical variables should be reconsidered. This narrative review aims to examine the need to evolve from the concept of ARDS as a syndrome to ARDS as a specific disease. So we have raised 4 critical questions: a) What is a disease?; b) what is DAD?; c) how is DAD considered according to ARDS definition?, and d) what is the relationship between ARDS and DAD?

  20. Progress and perspectives in pediatric acute respiratory distress syndrome.

    PubMed

    Rotta, Alexandre Tellechea; Piva, Jefferson Pedro; Andreolio, Cinara; de Carvalho, Werther Brunow; Garcia, Pedro Celiny Ramos

    2015-01-01

    Acute respiratory distress syndrome is a disease of acute onset characterized by hypoxemia and infiltrates on chest radiographs that affects both adults and children of all ages. It is an important cause of respiratory failure in pediatric intensive care units and is associated with significant morbidity and mortality. Nevertheless, until recently, the definitions and diagnostic criteria for acute respiratory distress syndrome have focused on the adult population. In this article, we review the evolution of the definition of acute respiratory distress syndrome over nearly five decades, with a special focus on the new pediatric definition. We also discuss recommendations for the implementation of mechanical ventilation strategies in the treatment of acute respiratory distress syndrome in children and the use of adjuvant therapies.

  1. Acute respiratory distress syndrome: 30 years later.

    PubMed

    Lesur, O; Berthiaume, Y; Blaise, G; Damas, P; Deland, E; Guimond, J G; Michel, R P

    1999-01-01

    Acute respiratory distress syndrome (ARDS) was first described about 30 years ago. Modern definitions and statements have recently been proposed to describe ARDS accurately, but none is perfect. Diffuse alveolar damage is the basic pathological pattern most commonly observed in ARDS, and the term includes permeability edema. The alveolar epithelium of the alveolar-capillary barrier is clearly a key component requiring repair, given its multipotent functional activity. Lung inflammation and neutrophil accumulation are essential markers of disease in ARDS, and a wide variety of pro- and anti-inflammatory cytokines have been described in the alveolar fluid and blood of patients. These molecules still have to prove their value as diagnostic or prognostic biomarkers of ARDS. Supportive therapy in ARDS improved in the past decade; mechanical ventilation with lung protective strategies and patient positioning are gaining interest, but the indications for corticosteroids for ARDS are still debated. Nitric oxide may have a place in the treatment of one-third of patients. Novel approaches, such as surfactant replacement and liquid ventilation, may further improve supportive therapy. Innovative interventions may be on the horizon in treatments that help to resolve or modulate common pathways of ARDS, such as inflammation (eg, granulocyte-colony stimulating factor) or epithelial repair (eg, keratinocyte growth factor).

  2. Adult respiratory distress syndrome in children.

    PubMed

    Pfenninger, J; Gerber, A; Tschäppeler, H; Zimmermann, A

    1982-09-01

    Twenty patients (age 2 weeks to 15 years) who fulfilled strict selection criteria for adult respiratory distress syndrome were identified during a 3 1/2-year period. The underlying disease was intra-abdominal infection/septicemia in seven, hypovolemic shock, near drowning, closed space burn, or cardiogenic shock caused by nupercaine intoxication in two each, and miscellaneous in five. The mean time of artificial ventilation with PEEP was 18 days (range 5 to 92), and the mean time of FIO2 greater than or equal to 0.5 while on the ventilatory 139 hours (range 12 to 648). PEEP levels were most often between 8 and 15 cm H2O. Eight patients had a pulmonary air leak. Eight patients died (40% mortality). Death was nearly always related to unresolved basic medical or surgical problems and multiple organ failure. Treatment of ARDS includes elimination of the cause of ARDS, early institution of mechanical ventilation with PEEP, prompt recognition and treatment of superimposed infections, and careful management of additional organ failures.

  3. [Kinetic therapy for acute respiratory distress syndrome].

    PubMed

    Chechenin, M G; Voevodin, S V; Pronichev, E Iu; Shuliveĭstrov, Iu V

    2004-01-01

    The authors evaluated the clinical and physiological effects of kinetic therapy (KT) in the treatment of acute respiratory distress syndrome (ARDS). Forty-six patients with ARDS underwent successive postural positioning in accordance with two regimens: 1) lateral, prone, contralateral, supine positions; 2) prone, lateral, contralateral, supine positions. The criterion for changing each position was the change in monitoring indices: SpO2, PaO2, and thoracopulmonary compliance (C). KT was performed until a respirator was withdrawn from the patient. In 25 patients, each maneuver of positioning was made during 30-minute propofol sedation. The control group included 24 patients with ARDS who received neither KT nor propofol sedation. KT caused a decrease in Vd/Vt, Qs/Qt and an increase in PaO2/FiO2 and C was more intensive, as compared with the control group. The duration of the patient's prone position was 3.2-0.7 hours and that of the supine position was 3.4-0.8 hours. The right and left lateral positions lasted 1.1-0.2 and 1.3-0.2 hours, respectively. KT regimen 1 was found to be more effective than KT regimen 2. Propofol sedation enhanced the efficiency of KT. The latter reduced death rates in patients with ARDS.

  4. The Lung Surfactant System in Adult Respiratory Distress Syndrome.

    DTIC Science & Technology

    1980-08-01

    STANDAROS- 193 A AD_ THE LUNG SURFACTANT SYSTEM IN ADULT RESPIRATORY DISTRESS SYNDROME FINAL PROGRESS REPORT John U. Balls August 1980 Sponsored by: US...D-A12l 434 THE LUNG SURFACTANT SYvTKl-OJL E~~rP DISTRESS SYNDROME (U) UNIVERSITY OF SOUTH FLORIDA TAMPA COLL OF MEDICINE J U BALIS RUG 8S DRNDi7-78-C...SURFACTANT SYSTEM IN ADULT Final 1 November 1978 - RESPIRATORY DISTU~SS SYNDROME - 30 April 1980 6. PERFORMING ORG. REPORT NUMBER * 7. AUTHOR(e) G. CONTRACT

  5. Acute respiratory distress syndrome: the Berlin Definition.

    PubMed

    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

  6. 'The Right Ventricle in Acute Respiratory Distress Syndrome'.

    PubMed

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

    2017-03-03

    Acute respiratory distress syndrome is associated with poor clinical outcomes with a pooled mortality rate of approximately 40% despite best standards of care. Current therapeutic strategies are based upon 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, is commonly encountered in moderate to severe acute respiratory distress syndrome and is reported to be one of the major determinants of mortality. The prevalence rate of echocardiographically evident right ventricular dysfunction in acute respiratory distress syndrome varies across studies ranging from 22% to 50%. Although there is no definitive causal relationship between right ventricular dysfunction and mortality, severe right ventricular dysfunction is associated with increased mortality. Factors that can adversely affect right ventricular function include hypoxic pulmonary vasoconstriction, hypercapnia, and invasive ventilation with high driving pressure. It might be expected that early diagnosis of right ventricular dysfunction would be of benefit however, echocardiography markers (qualitative and quantitative) used to prospectively evaluate the right ventricle in acute respiratory distress syndrome have not been tested in adequately powered studies. In this review we examine the prognostic implications and pathophysiology of right ventricular dysfunction in acute respiratory distress syndrome 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.

  7. [Acute respiratory distress syndrome: a review of the Berlin definition].

    PubMed

    de Luis Cabezón, N; Sánchez Castro, I; Bengoetxea Uriarte, U X; Rodrigo Casanova, M P; García Peña, J M; Aguilera Celorrio, L

    2014-01-01

    Acute Respiratory Distress Syndrome (ARDS) is due to many causes. The absence of a universal definition up until now has led to a series of practical problems for a definitive diagnosis. The incidences of ARDS and Acute Lung Injury (ALI) vary widely in the current literature. The American-European Consensus Conference definition has been applied since its publication in 1994 and has helped to improve knowledge about ARDS. However, 18 years later, in 2011, the European Intensive Medicine Society, requested a team of international experts to meet in Berlin to review the ARDS definition. The purpose of the Berlin definition is not to use it as a prognostic tool, but to improve coherence between research and clinical practice.

  8. Neonatal thyroid function: prematurity, prenatal steroids, and respiratory distress syndrome.

    PubMed Central

    Franklin, R C; Purdie, G L; O'Grady, C M

    1986-01-01

    Indices of thyroid function were measured in 97 preterm infants at birth and at 5, 10, and 15 days of age. Triiodothyronine uptake, free thyroxine index, thyroxine, free thyroxine, triiodothyronine, reverse triiodothyronine, and thyroxine binding globulin values at birth correlated with gestational age, whereas thyroid stimulating hormone values did not. Treatment with steroids prenatally had no apparent effect on thyroid function at birth or postnatally. Infants developing respiratory distress syndrome had normal values for all indices at birth. These infants had significantly lower thyroxine, free thyroxine index, free thyroxine, and triiodothyronine values at 5 days of age, while thyroid stimulating hormone values remained normal. This alteration in thyroid function was interpreted as being secondary to respiratory distress syndrome. Gestational maturity and respiratory distress syndrome, if present, must be taken into account when evaluating thyroxine variables in preterm infants, whereas measurement of thyroid stimulating hormone as the screen for congenital hypothyroidism circumvents these considerations. PMID:3729529

  9. Blastomyces gilchristii as Cause of Fatal Acute Respiratory Distress Syndrome.

    PubMed

    Dalcin, Daniel; Rothstein, Aaron; Spinato, Joanna; Escott, Nicholas; Kus, Julianne V

    2016-02-01

    Since the 2013 description of Blastomyces gilchristii, research describing the virulence or clinical outcome of B. gilchristii infection has been lacking. We report molecular evidence of B. gilchristii as an etiologic agent of fatal acute respiratory distress syndrome. B. gilchristii infection was confirmed by PCR and sequence analysis.

  10. Blastomyces gilchristii as Cause of Fatal Acute Respiratory Distress Syndrome

    PubMed Central

    Rothstein, Aaron; Spinato, Joanna; Escott, Nicholas; Kus, Julianne V.

    2016-01-01

    Since the 2013 description of Blastomyces gilchristii, research describing the virulence or clinical outcome of B. gilchristii infection has been lacking. We report molecular evidence of B. gilchristii as an etiologic agent of fatal acute respiratory distress syndrome. B. gilchristii infection was confirmed by PCR and sequence analysis. PMID:26812599

  11. An approach to ventilation in acute respiratory distress syndrome

    PubMed Central

    Houston, Patricia

    2000-01-01

    Appropriate management of patients with acute respiratory distress syndrome (ARDS) represents a challenge for physicians working in the critical care environment. Significant advances have been made in understanding the pathophysiology of ARDS. There is also an increasing appreciation of the role of ventilator-induced lung injury (VILI). VILI is most likely related to several different aspects of ventilator management: barotrauma due to high peak airway pressures, lung overdistension or volutrauma due to high transpulmonary pressures, alveolar membrane damage due to insufficient positive end-expiratory pressure levels and oxygen-related cell toxicity. Various lung protective strategies have been suggested to minimize the damage caused by conventional modes of ventilation. These include the use of pressure- and volume-limited ventilation, the use of the prone position in the management of ARDS, and extracorporeal methods of oxygen delivery and carbon dioxide removal. Although the death rate resulting from ARDS has been declining over the past 10 years, there is no evidence that any specific treatment or change in approach to ventilation is the cause of this improved survival. PMID:10948686

  12. [Non-cardiogenic pulmonary edema, acute respiratory distress syndrome].

    PubMed

    Skalická, Hana; Bělohlávek, Jan

    2015-01-01

    Non-cardiogenic pulmonary edema is a clinical syndrome manifested by rapidly progressive respiratory distress leading, without therapy, to severe respiratory insufficiency and subsequent multiorgan failure. The pathophysiological causes are: the change in the pressure gradients in the pulmonary capillaries, the impaired membrane permeability of the alveolocapillary in the lungs, and impaired lymphatic drainage. Unlike in cardiogenic pulmonary edema, cardiac disease is not a cause, and there is no increase in wedge pressure (< 18 mm Hg). The aetiological base is diverse and includes more clinical pathological factors. The diagnosis and evaluation are usually very difficult due to the rapidly deteriorating clinical condition of the patients. A decisive, quick and comprehensive approach, using all available invasive and non-invasive methods is necessary. The basic steps of treatment are: the use of different types of ventilatory support in order to achieve adequate oxygenation, dealing with possible hemodynamic instability, and, when needed, other specific procedures. It is always important to keep in mind that this is a very serious condition with a high mortality rate. And there is a need for fast and efficient access to the best specialized clinic.

  13. Emerging Therapies for the Prevention of Acute Respiratory Distress Syndrome

    PubMed Central

    Ruthman, Carl A.; Festic, Emir

    2015-01-01

    The development of the acute respiratory distress syndrome (ARDS) carries significant risk of morbidity and mortality. To date, pharmacologic therapy has been largely ineffective for patients with ARDS. We present our personal review aimed at outlining current and future directions for the pharmacologic prevention of ARDS. Several available risk-stratification or prediction scores strategies for identification of patients at risk of ARDS have been reported. Although not ready for the clinical everyday use, they are and will be instrumental in the ongoing and future trials of pharmacoprevention of ARDS. Several systemic medications established the potential role in ARDS prevention based on the preclinical studies and observational data. Due to potential for systemic adverse effects to neutralize any pharmacologic benefits of systemic therapy, inhaled medications appear particularly attractive candidates for ARDS prevention. This is because of their direct delivery to the site of the proposed action (lungs), while pulmonary epithelial surface is still functional. We postulate that overall morbidity and mortality rates from ARDS in the future will be contingent upon decreasing the overall incidence of ARDS through effective identification of those at risk and early application of proven supportive care and pharmacologic interventions. PMID:26002528

  14. Management of the acute respiratory distress syndrome.

    PubMed

    Conrad, Steven A; Bidani, Akhil

    2002-05-01

    Significant advances have occurred in the knowledge of the pathogenesis of ARDS. It is now recognized that ARDS is a manifestation of a diffuse process that results from a complicated cascade of events following an initial insult or injury. Mechanical ventilation and PEEP are still important components of supportive therapy. To avoid ventilator-associated lung injury there is emphasis on targeting ventilator management based on measurement of pulmonary mechanics. For those with resistant hypoxia and severe pulmonary hypertension adjunctive modalities, such as prone positioning and low-dose iNO, may provide important benefit. Alternative modes of supporting gas exchange, such as with partial liquid ventilation and extracorporeal gas-exchange, may serve as rescue therapies. Advances in cell and molecular biology have contributed to a better understanding of the role of inflammatory cells and mediators that contribute to the acute lung injury and the pathophysiology of the syndrome that manifests as ARDS. Based on this new understanding, the potential targets for intervention to ameliorate the systemic inflammatory response have proliferated. Examples include the cytokine network and its receptors, antioxidants, and endothelins. Apart from the challenge of testing these agents in experimental models, it seems likely that determination of the optimum combination of agents will become an equally important endeavor. A particular challenge is to develop better methods of predicting which of the many at-risk patients will go on to full-blown ARDS and MODS, thereby targeting subgroups of patients most likely to benefit from anti-inflammatory therapies. Similarly, the adverse effects of immunosuppressive therapy may be diminished by improved, perhaps molecular, techniques to detect microbial pathogens and permit differentiation between Systemic inflammatory response syndrome and sepsis.

  15. Coping and Psychological Distress of Chinese Parents of Children with Down Syndrome.

    ERIC Educational Resources Information Center

    Cheng, Paul; Tang, Catherine So-Kum

    1995-01-01

    Coping and correlates of psychological distress of 174 Chinese parents of children with Down's syndrome, language delays, or no disabilities were compared. Down's syndrome parents more frequently used avoidance coping style. No differences were observed between Down's syndrome and language delay parents on psychological distress, optimism,…

  16. Acute Respiratory Distress Syndrome in Wartime Military Burns: Application of the Berlin Criteria

    DTIC Science & Technology

    2014-01-01

    Acute respiratory distress syndrome in wartime military burns: Application of the Berlin criteria Slava M. Belenkiy, MD, Allison R. Buel, DO, Jeremy...Andriy I. Batchinsky, MD, Leopoldo C. Cancio, MD, and Kevin K. Chung, MD, San Antonio, Texas BACKGROUND: Acute respiratory distress syndrome (ARDS...EVIDENCE: Epidemiologic/prognostic study, level III. KEY WORDS: Mechanical ventilation; adult respiratory distress syndrome ; the Berlin definition; combat

  17. A Comparison of Acute Respiratory Distress Syndrome Outcomes Between Military and Civilian Burn Patients

    DTIC Science & Technology

    2015-03-01

    MILITARY MEDICINE, 180, 3:56, 2015 A Comparison of Acute Respiratory Distress Syndrome Outcomes Between Military and Civilian Burn Patients J Alan...Chung, MC USA*‡ ABSTRACT Background: The objective of this report was to compare the prevalence of acute respiratory distress syndrome (ARDS) and...Development of acute respiratory distress syndrome (ARDS) is a common complication of burn injury and is associated with poor outcomes. Previous reports using

  18. Brief report: Emotional distress and recent stressful life events in long QT syndrome mutation carriers.

    PubMed

    Määttänen, Ilmari; Jokela, Markus; Pulkki-Råback, Laura; Keltikangas-Järvinen, Liisa; Swan, Heikki; Toivonen, Lauri; Merjonen, Päivi; Hintsa, Taina

    2015-11-01

    To study emotional distress in symptomatic and asymptomatic long QT syndrome mutation carriers who had experienced a recent stressful life event. The participants were 209 symptomatic and 279 asymptomatic long QT syndrome mutation carriers. Emotional distress was assessed with the Cope questionnaire and stressful life events with the Social Readjustment Rating Scale. Symptomatic long QT syndrome mutation carriers with burdening recent stressful life events reported a higher emotional distress (β = 0.35, p < 0.001), while the asymptomatic did not show such difference (β = 0.13, p = 0.393). Symptomatic long QT syndrome mutation carriers who have experienced stressful life events recently report an increased emotional distress.

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

    PubMed Central

    Wilson, Bryan; Typpo, Katri

    2016-01-01

    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 pediatric intensive care unit (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 and advancement and to maintain delivery of EN are needed to improve morbidity and mortality from pediatric ARDS. Both, over and underfeeding, prolong duration of mechanical ventilation in children and worsen 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

  20. Early Treatment of Severe Acute Respiratory Distress Syndrome.

    PubMed

    Przybysz, Thomas M; Heffner, Alan C

    2016-02-01

    Acute respiratory distress syndrome (ARDS) is defined by acute diffuse inflammatory lung injury invoked by a variety of systemic or pulmonary insults. Despite medical progress in management, mortality remains 27% to 45%. Patients with ARDS should be managed with low tidal volume ventilation. Permissive hypercapnea is well tolerated. Conservative fluid strategy can reduce ventilator and hospital days in patients without shock. Prone positioning and neuromuscular blockers reduce mortality in some patients. Early management of ARDS is relevant to emergency medicine. Identifying ARDS patients who should be transferred to an extracorporeal membrane oxygenation center is an important task for emergency providers.

  1. Overview of current lung imaging in acute respiratory distress syndrome.

    PubMed

    Zompatori, Maurizio; Ciccarese, Federica; Fasano, Luca

    2014-12-01

    Imaging plays a key role in the diagnosis and follow-up of acute respiratory distress syndrome (ARDS). Chest radiography, bedside lung ultrasonography and computed tomography scans can provide useful information for the management of patients and detection of prognostic factors. However, imaging findings are not specific and several possible differential diagnoses should be taken into account. Herein we will review the role of radiological techniques in ARDS, highlight the plain radiological and computed tomography findings according to the pathological stage of the disease (exudative, inflammatory and fibroproliferative), and summarise the main points for the differential diagnosis with cardiogenic oedema, which is still challenging in the acute stage.

  2. [Alcohol and acute respiratory distress syndrome: casuality or causality?].

    PubMed

    Sarmiento, Xavier; Guardiola, Juan J; Soler, Manuel

    2013-06-18

    Alcohol has been considered an important risk factor for the development of pneumonia since the last century. Nevertheless, it was not thought that it had relevant effects on lung structure and functions until recently. Recent studies have shown that the risk for acute respiratory distress syndrome (ARDS) is 2-4 times higher among alcoholic patients with sepsis or trauma, and that alcoholism can play a roll in more than 50% of cases in the pathogenesis of this syndrome. Although alcoholism per se does not cause acute lung injury it predisposes to pulmonary dysfunction after inflammatory stress, that is present in clinical situations that cause ARDS leading to its development and complicating its outcome. Recent investigations in animals and humans with alcohol abuse have uncovered several alterations currently known as the "alcoholic lung". This revision discusses the association between alcohol abuse and lung injury/ARDS and tries to explain the physiopathology along with possible treatments.

  3. Therapy of Adult Respiratory Distress Syndrome with Alpha-1- Antiproteinase or Lung Surfactant.

    DTIC Science & Technology

    1991-03-15

    I AD-A239 010I it II II 11 11 1 11 lull 11 III ________________ CONTRACT NO: DAMD17-88-C-8020 TITLE : THERAPY OF ADULT RESPIRATORY DISTRESS SYNDROME ...multiple names including the adult respiratory distress syndrome ( ARDS ), wet lung, shock lung, capillary leak syndrome , DaNang lung, post-perfusion lung...of Adult Respiratory Distress Syndrome with Alpha-l-Antiproteinase or Lung Surfactant 1?. PERSONAL AUTHOR(S) Roger G. Spragg, M.D. 13a. TYPE OF

  4. Acute respiratory distress syndrome: prevention and early recognition.

    PubMed

    de Haro, Candelaria; Martin-Loeches, Ignacio; Torrents, Eva; Artigas, Antonio

    2013-04-24

    Acute respiratory distress syndrome (ARDS) is common in critically ill patients admitted to intensive care units (ICU). ARDS results in increased use of critical care resources and healthcare costs, yet the overall mortality associated with these conditions remains high. Research focusing on preventing ARDS and identifying patients at risk of developing ARDS is necessary to develop strategies to alter the clinical course and progression of the disease. To date, few strategies have shown clear benefits. One of the most important obstacles to preventive interventions is the difficulty of identifying patients likely to develop ARDS. Identifying patients at risk and implementing prevention strategies in this group are key factors in preventing ARDS. This review will discuss early identification of at-risk patients and the current prevention strategies.

  5. Lung parenchyma remodeling in acute respiratory distress syndrome.

    PubMed

    Rocco, P R M; Dos Santos, C; Pelosi, P

    2009-12-01

    Acute respiratory distress syndrome (ARDS), the most severe manifestation of acute lung injury (ALI), is described as a stereotyped response to lung injury with a transition from alveolar capillary damage to a fibroproliferative phase. Most ARDS patients survive the acute initial phase of lung injury and progress to either reparation of the lesion or evolution of the syndrome. Despite advances in the management of ARDS, mortality remains high (40%) and autopsies show extended pulmonary fibrosis in 55% of patients, suggesting the importance of deregulated repair in the morbidity and mortality of these patients. Factors influencing progression to fibroproliferative ARDS versus resolution and reconstitution of the normal pulmonary parenchymal architecture are poorly understood. Abnormal repair and remodeling may be profoundly affected by both environmental and genetic factors. In this line, mechanical ventilation may affect the macromolecules that constitute the extracellular matrix (collagen, elastin, fibronectin, laminin, proteoglycan and glycosaminoglycans), suffer changes and impact the biomechanical behavior of lung parenchyma. Furthermore, evidence suggests that acute inflammation and fibrosis may be partially independent and/or interacting processes that are autonomously regulated, and thus amenable to individual and specific therapies. In this review, we explore recent advances in the field of fibroproliferative ARDS/ALI, with special emphasis on 1) the physiological properties of the extracellular matrix, 2) the mechanisms of remodeling, 3) the impact of mechanical ventilation on lung fibrotic response, and (4) therapeutic interventions in the remodeling process.

  6. Acute Respiratory Distress Syndrome Associated with Tumor Lysis Syndrome in a Child with Acute Lymphoblastic Leukemia

    PubMed Central

    Macaluso, Alessandra; Genova, Selene; Maringhini, Silvio; Coffaro, Giancarlo; Ziino, Ottavio; D’Angelo, Paolo

    2015-01-01

    Tumor lysis syndrome is a serious and dangerous complication usually associated with antiblastic treatment in some malignancies characterized by high cell turn-over. Mild or severe electrolyte abnormalities including high serum levels of uric acid, potassium, phosphorus, creatinine, bun and reduction of calcium can be responsible for multi-organ failure, involving mostly kidneys, heart and central nervous system. Renal damage can be followed by acute renal failure, weight gain, progressive liver impairment, overproduction of cytokines, and subsequent maintenance of multi-organ damage. Life-threatening acute respiratory failure associated with tumor lysis syndrome is rare. We describe a child with T-cell acute lymphoblastic leukemia, who developed an unusually dramatic tumor lysis syndrome, after administration of the first low doses of steroid, that was rapidly associated with severe acute respiratory distress syndrome. Subsequent clinical course and treatment modalities that resulted in the gradual and full recovery of the child are also described. PMID:25918625

  7. A Pathophysiologic Approach to Biomarkers in Acute Respiratory Distress Syndrome.

    PubMed

    Blondonnet, Raiko; Constantin, Jean-Michel; Sapin, Vincent; Jabaudon, Matthieu

    2016-01-01

    Acute respiratory distress syndrome (ARDS) is an acute-onset hypoxic condition with radiographic bilateral lung infiltration. It is characterized by an acute exudative phase combining diffuse alveolar damage and lung edema followed by a later fibroproliferative phase. Despite an improved understanding of ARDS pathobiology, our ability to predict the development of ARDS and risk-stratify patients with the disease remains limited. Biomarkers may help to identify patients at the highest risk of developing ARDS, assess response to therapy, predict outcome, and optimize enrollment in clinical trials. After a short description of ARDS pathobiology, here, we review the scientific evidence that supports the value of various ARDS biomarkers with regard to their major biological roles in ARDS-associated lung injury and/or repair. Ongoing research aims at identifying and characterizing novel biomarkers, in order to highlight relevant mechanistic explorations of lung injury and repair, and to ultimately develop innovative therapeutic approaches for ARDS patients. This review will focus on the pathophysiologic, diagnostic, and therapeutic implications of biomarkers in ARDS and on their utility to ultimately improve patient care.

  8. Clinical Practice Guideline of Acute Respiratory Distress Syndrome

    PubMed Central

    Cho, Young-Jae; Moon, Jae Young; Shin, Ein-Soon; Kim, Je Hyeong; Jung, Hoon; Park, So Young; Kim, Ho Cheol; Sim, Yun Su; Rhee, Chin Kook; Lim, Jaemin; Lee, Seok Jeong; Lee, Won-Yeon; Lee, Hyun Jeong; Kwak, Sang Hyun; Kang, Eun Kyeong; Chung, Kyung Soo

    2016-01-01

    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. PMID:27790273

  9. A Pathophysiologic Approach to Biomarkers in Acute Respiratory Distress Syndrome

    PubMed Central

    Blondonnet, Raiko; Constantin, Jean-Michel; Sapin, Vincent; Jabaudon, Matthieu

    2016-01-01

    Acute respiratory distress syndrome (ARDS) is an acute-onset hypoxic condition with radiographic bilateral lung infiltration. It is characterized by an acute exudative phase combining diffuse alveolar damage and lung edema followed by a later fibroproliferative phase. Despite an improved understanding of ARDS pathobiology, our ability to predict the development of ARDS and risk-stratify patients with the disease remains limited. Biomarkers may help to identify patients at the highest risk of developing ARDS, assess response to therapy, predict outcome, and optimize enrollment in clinical trials. After a short description of ARDS pathobiology, here, we review the scientific evidence that supports the value of various ARDS biomarkers with regard to their major biological roles in ARDS-associated lung injury and/or repair. Ongoing research aims at identifying and characterizing novel biomarkers, in order to highlight relevant mechanistic explorations of lung injury and repair, and to ultimately develop innovative therapeutic approaches for ARDS patients. This review will focus on the pathophysiologic, diagnostic, and therapeutic implications of biomarkers in ARDS and on their utility to ultimately improve patient care. PMID:26980924

  10. The pulmonary vascular lesions of the adult respiratory distress syndrome.

    PubMed Central

    Tomashefski, J. F.; Davies, P.; Boggis, C.; Greene, R.; Zapol, W. M.; Reid, L. M.

    1983-01-01

    Specimen arteriography, morphometry, and light and electron microscopy were used for examination of the pulmonary vasculature of 22 patients who died with the adult respiratory distress syndrome (ARDS), for the purpose of defining the lesions that contribute to pulmonary hypertension in this setting. The different lesions correlated with the duration rather than the cause of ARDS. Thromboemboli occurred in 21 patients, and macrothrombi found at autopsy correlated with the number of filling defects on antemortem angiography. Acute endothelial injury was documented ultrastructurally even in intermediate and late-stage patients. Fibrocellular intimal obliteration of arteries, veins, and lymphatics and infective vasculitis were prominent in those surviving beyond 10 days. In long-term survivors, tortuous arteries and irregularly dilated capillaries were striking features. Peripheral extension of vascular smooth muscle and a significant increase in the percentage of medial thickness of muscular arteries with duration of ARDS were noted. The pathogenesis and clinical significance of these lesions is discussed. Images Figure 1 Figure 2 Figure 5 Figure 6 Figure 7 Figure 8 Figure 9 Figure 10 Figure 11 Figure 12 Figure 13 PMID:6859225

  11. Noninvasive ventilation on mortality of acute respiratory distress syndrome

    PubMed Central

    Ye, Ling; Wang, Jian; Xu, Xiaobo; Song, Yuanlin; Jiang, Jinjun

    2016-01-01

    [Purpose] The aim of this study was to assess the efficacy of noninvasive ventilation (NIV) in acute respiratory distress syndrome (ARDS). [Subjects and Methods] The clinical data of 58 patients with ARDS that required mechanical ventilation in two intensive care units (ICU) was reviewed. [Results] Endotracheal intubation was performed in 55.17% of the total patients and in 39.53% of the patients who received NIV treatment. The APACHE II score for patients who only received IV was significantly higher than those who only underwent NIV (25.67 ± 5.30 vs. 18.12 ± 7.20). However, there were no significant differences in 28-day/90-day survival rates, duration of mechanical ventilation, and length of ICU stay between these two groups. For patients from a NIV-to-IV group, the APACHE II scores before endotracheal intubation were higher than the scores from IV patients (26.12 ± 4.08 vs. 21.94 ± 6.10). The 90-day survival rate in the NIV-to-IV group was significantly lower than that of the IV-only group (23.5% vs. 73.3%), although there was no difference in the 28-day survival rate between the two groups. [Conclusion] The application of NIV reduces the percentage of patients requiring endotracheal intubation. PMID:27630415

  12. Severe acute respiratory distress syndrome caused by unintentional sewing machine lubricant ingestion: A case report

    PubMed Central

    Kishore, Sunil; Chandelia, Sudha; Patharia, Neha; Swarnim

    2016-01-01

    Sewing machine oil ingestion is rare but is possible due to its availability at home. Chemically, it belongs to hydrocarbon family which is toxic if aspirated, owing to their physical properties such as high volatility and low viscosity. On the contrary, sewing machine lubricant has high viscosity and low volatility which makes it aspiration less likely. The main danger of hydrocarbon ingestion is chemical pneumonitis which may be as severe as acute respiratory distress syndrome (ARDS). We report a case of a 5-year-old girl with accidental ingestion of sewing machine lubricant oil, who subsequently developed ARDS refractory to mechanical ventilation. There was much improvement with airway pressure release ventilation mode of ventilation, but the child succumbed to death due to pulmonary hemorrhage. PMID:27994384

  13. Acute Respiratory Distress Syndrome and Outcomes after Near-hanging

    PubMed Central

    Mansoor, Sahar; Afshar, Majid; Barrett, Matthew; Smith, Gordon S.; Barr, Erik A.; Lissauer, Matthew E.; McCurdy, Michael T.; Murthi, Sarah B.; Netzer, Giora

    2015-01-01

    Purpose Assess the case rate of Acute Respiratory Distress Syndrome (ARDS) after near-hanging, and the secondary outcomes of traumatic and/or anoxic brain injury, and death. Risk factors for the outcomes were assessed. Method Single-center, state-wide retrospective cohort study of consecutive patients admitted between August, 2002, and September, 2011, with a primary diagnosis of non-judicial "hanging injury". Results Of 56 patients, 73% were male. The median age was 31 (IQR: 16–56). Upon arrival, 9% (5/56) did not have a pulse, and 23% (13/56) patients were intubated. The median Glasgow Coma Scale (GCS) was 13 (IQR: 3–15); 14% (8/56) had a GCS=3. ARDS developed in 9% (5/56) of patients. Traumatic anoxic brain injury resulted in 9% (5/56) of patients. The in-hospital case fatality was 5% (3/56). Lower median GCS [3 (IQR: 3–7) vs. 14 (IQR: 3–15), p=0.0003] and intubation in field or in trauma resuscitation unit [100% (5/5) vs. 16% (8/51), p=0.0003] were associated with ARDS development. Risk factors of death were GCS=3 [100% (3/3) vs. 9% (5/53), p=0.002]; pulselessness upon arrival of emergency medical services [100% (3/3) vs. 4% (2/53), p<0.001]; and abnormal neurologic imaging [50% (1/2) vs. zero, p=0.04]. Conclusions The ARDS case rate after near-hanging is similar to the general trauma population. Low GCS and intubation are associated with increased risk of ARDS development. The rate of traumatic and/or anoxic brain injury in this population is low. PMID:25596627

  14. Natural vs synthetic surfactants in neonatal respiratory distress syndrome.

    PubMed

    Halliday, H L

    1996-02-01

    This review examines the 11 randomised clinical trials that have compared different surfactant preparations. Seven trials, enrolling 2488 infants with respiratory distress syndrome (RDS), compared the natural surfactant beractant (Survanta) with the synthetic surfactant colfosceril palmitate (Exosurf Neonatal). Infants treated with beractant had lower oxygen requirements for at least 3 days than those treated with colfosceril palmitate. The infants treated with beractant also had lower risks of neonatal mortality [odds ratio (OR) 0.81; 95% confidence interval (CI) 0.65 to 1.01], retinopathy of prematurity (OR 0.81; 95% CI 0.66 to 0.99), and the combined endpoint of death or bronchopulmonary dysplasia (OR 0.86; 95% CI 0.75 to 0.99), compared with those treated with colfosceril palmitate. Calf lung surfactant extract (CLSE; Infasurf), another natural surfactant, has been compared with colfosceril palmitate in 2 studies: in one as prophylaxis and in the other as rescue therapy. Similar, although nonsignificant, advantages were found for the natural surfactant compared with the synthetic surfactant. In 6 of these 9 trials there was a significant reduction in the odds of pulmonary air leaks (OR 0.53; 95% CI 0.41 to 0.64) for infants treated with natural compared with synthetic surfactants. In 7 trials (3554 infants) comparing natural and synthetic surfactants to treat RDS (6 comparing beractant and colfosceril palmitate, and one CLSE and colfosceril palmitate), there was a significantly reduced risk of neonatal mortality (OR 0.80; 95% CI 0.66 to 0.97) with natural compared with synthetic surfactant treatment. In 2 further trials, different natural surfactant preparations have been compared. Reduced oxygen needs for 24 hours after treatment were found for CLSE and Curosurf (porcine-derived lung surfactant, PLS) when each was compared with beractant. Apparent longer term benefits from these surfactants were not statistically proven. Further trials are needed to be certain

  15. Cushing syndrome due to adrenal tumor

    MedlinePlus

    ... syndrome. It occurs when a tumor of the adrenal gland releases excess amounts of the hormone cortisol. Causes ... hormone cortisol. This hormone is made in the adrenal glands . Too much cortisol can be due to various ...

  16. Mycoplasmal Pneumonia and Adult Respiratory Distress Syndrome: A Complication to be Recognized

    PubMed Central

    Dixon, Calvin

    1981-01-01

    Mycoplasma pneumoniae pneumonia is usually a mild, self-limited disease. Complications are rare. A patient is presented with adult respiratory distress syndrome. Recognition of this unusual complication, presenting as a common disease syndrome, is emphasized. ImagesFigure 1Figure 2 PMID:7241615

  17. Staphylococcal toxic shock syndrome presenting as acute respiratory distress and cor pulmonale.

    PubMed

    Zaki, S A; Shanbag, P; Chavan, V; Shenoy, P

    2010-01-01

    We describe a 7-year-old boy with staphylococcal toxic shock syndrome who presented with acute respiratory distress and cor pulmonale. We wish to highlight this unusual presentation as the diagnosis of toxic shock syndrome depends chiefly on a high degree of clinical suspicion. Early diagnosis and prompt institution of appropriate therapy will significantly reduce morbidity and mortality.

  18. Maladaptive Behavior Differences in Prader-Willi Syndrome Due to Paternal Deletion versus Maternal Uniparental Disomy.

    ERIC Educational Resources Information Center

    Dykens, Elisabeth M.; King, Bryan H.; Cassidy, Suzanne B.

    1999-01-01

    This study compared maladaptive behavior in 23 people with Prader-Willi syndrome due to paternal deletion and in 23 age- and gender-matched subjects with maternal uniparental disomy. Controlling for IQs, the deletion cases showed significantly higher maladaptive ratings, more symptom-related distress, and more behavior problems. Findings suggest a…

  19. A Case of Idiopathic Hypereosinophilic Syndrome Presenting With Acute Respiratory Distress Syndrome

    PubMed Central

    Lim, Kyung-Suk; Ko, Jaehoon; Lee, Seong Soo; Shin, Beomsu; Choi, Dong-Chull

    2014-01-01

    Although idiopathic hypereosinophilic syndrome(IHES) commonly involves the lung, it is rarely associated with acute respiratory distress syndrome (ARDS). Here we describe a case of IHES presented in conjunction with ARDS. A 37-year-old male visited the emergency department at Samsung Medical Center, Seoul, Korea, with a chief complaint of dyspnea. Blood tests showed profound peripheral eosinophilia and thrombocytopenia. Patchy areas of consolidation with ground-glass opacity were noticed in both lower lung zones on chest radiography. Rapid progression of dyspnea and hypoxia despite supplement of oxygen necessitated the use of mechanical ventilation. Eosinophilic airway inflammation was subsequently confirmed by bronchoalveolar lavage, leading to a diagnosis of IHES. High-dose corticosteroids were administered, resulting in a dramatic clinical response. PMID:24404401

  20. Treatment of Adenoviral Acute Respiratory Distress Syndrome Using Cidofovir With Extracorporeal Membrane Oxygenation.

    PubMed

    Lee, Minhyeok; Kim, Seulgi; Kwon, Oh Jung; Kim, Ji Hye; Jeong, Inbeom; Son, Ji Woong; Na, Moon Jun; Yoon, Yoo Sang; Park, Hyun Woong; Kwon, Sun Jung

    2017-03-01

    Adenovirus infections are associated with respiratory (especially upper respiratory) infection and gastrointestinal disease and occur primarily in infants and children. Although rare in adults, severe lower respiratory adenovirus infections including pneumonia are reported in specific populations, such as military recruits and immunocompromised patients. Antiviral treatment is challenging due to limited clinical experience and lack of well-controlled randomized trials. Several previously reported cases of adenoviral pneumonia showed promising efficacy of cidofovir. However, few reports discussed the efficacy of cidofovir in acute respiratory distress syndrome (ARDS). We experienced 3 cases of adenoviral pneumonia associated with ARDS and treated with cidofovir and respiratory support, including extracorporeal membrane oxygenation (ECMO). All 3 patients showed a positive clinical response to cidofovir and survival at 28 days. Cidofovir with early ECMO therapy may be a therapeutic option in adenoviral ARDS. A literature review identified 15 cases of adenovirus pneumonia associated with ARDS.

  1. Estimation of dead space fraction can be simplified in the acute respiratory distress syndrome

    PubMed Central

    2010-01-01

    Acute lung injury and acute respiratory distress syndrome are characterized by a non-cardiogenic pulmonary edema responsible for a significant impairment of gas exchange. The pulmonary dead space increase, which is due primarily to an alteration in pulmonary blood flow distribution, is largely responsible for carbon dioxide retention. Previous studies, computing the pulmonary dead space by measuring the expired carbon dioxide and the Enghoff equation, found that the dead space fraction was significantly higher in the non-survivors; it was even an independent risk of death. The computation of the dead space not by measuring the expired carbon dioxide but by applying a rearranged alveolar gas equation that takes into account only the weight, age, height, and temperature of the patient could lead to widespread clinical diffusion of this measurement at the bedside. PMID:20840798

  2. Acute respiratory distress syndrome after verapamil intoxication: case report and literature review.

    PubMed

    Izdes, S; Altintas, N D; Soykut, C

    2014-04-01

    Verapamil intoxication is a life-threatening condition that often presents with severe hemodynamic instability and requires vasopressor support. There are also documented case reports of the development of non-cardiogenic pulmonary oedema after verapamil overdose. However, the exact mechanisms responsible for pulmonary oedema remain unclear. Here, we describe a 36-year-old woman who was admitted to the intensive care unit after ingesting high-dose verapamil and subsequently developed acute respiratory distress syndrome soon after hemodynamic stabilization. Possible mechanisms are presented after taking into account findings in the current literature. Acute respiratory distress syndrome should be considered early during the evaluation of patients with verapamil intoxication.

  3. Potential Application of Viral Empty Capsids for the Treatment of Acute Lung Injury/Acute Respiratory Distress Syndrome

    DTIC Science & Technology

    2016-07-01

    Acute Respiratory Distress Syndrome PRINCIPAL INVESTIGATOR: Prof. Ariella Oppenheim CONTRACTING ORGANIZATION: Hebrew University of Jerusalem...Lung / 5a. CONTRACT NUMBER Injury/Acute Respiratory Distress Syndrome 5b. GRANT NUMBER 5c. PROGRAM ELEMENT NUMBER 6. AUTHOR(S) Prof. Ariella...mechanism elicited by VLPs that attenuate 2CLP-induced sepsis, to be performed as the project continues. 15. SUBJECT TERMS Acute Respiratory Distress

  4. Prone position in acute respiratory distress syndrome. Rationale, indications, and limits.

    PubMed

    Gattinoni, Luciano; Taccone, Paolo; Carlesso, Eleonora; Marini, John J

    2013-12-01

    In the prone position, computed tomography scan densities redistribute from dorsal to ventral as the dorsal region tends to reexpand while the ventral zone tends to collapse. Although gravitational influence is similar in both positions, dorsal recruitment usually prevails over ventral derecruitment, because of the need for the lung and its confining chest wall to conform to the same volume. The final result of proning is that the overall lung inflation is more homogeneous from dorsal to ventral than in the supine position, with more homogeneously distributed stress and strain. As the distribution of perfusion remains nearly constant in both postures, proning usually improves oxygenation. Animal experiments clearly show that prone positioning delays or prevents ventilation-induced lung injury, likely due in large part to more homogeneously distributed stress and strain. Over the last 15 years, five major trials have been conducted to compare the prone and supine positions in acute respiratory distress syndrome, regarding survival advantage. The sequence of trials enrolled patients who were progressively more hypoxemic; exposure to the prone position was extended from 8 to 17 hours/day, and lung-protective ventilation was more rigorously applied. Single-patient and meta-analyses drawing from the four major trials showed significant survival benefit in patients with PaO2/FiO2 lower than 100. The latest PROSEVA (Proning Severe ARDS Patients) trial confirmed these benefits in a formal randomized study. The bulk of data indicates that in severe acute respiratory distress syndrome, carefully performed prone positioning offers an absolute survival advantage of 10-17%, making this intervention highly recommended in this specific population subset.

  5. The acute respiratory distress syndrome in catastrophic antiphospholipid syndrome: analysis of a series of 47 patients

    PubMed Central

    Bucciarelli, S; Espinosa, G; Asherson, R A; Cervera, R; Claver, G; Gómez‐Puerta, J A; Ramos‐Casals, M; Ingelmo, M

    2006-01-01

    Background The acute respiratory distress syndrome (ARDS) is a non‐cardiogenic form of pulmonary oedema characterised by severe hypoxaemia refractory to oxygen therapy, with diffuse pulmonary infiltrates on chest radiographs. It can be precipitated by various serious medical and surgical conditions, including systemic autoimmune diseases. The “catastrophic” variant of the antiphospholipid syndrome (APS) is an accelerated form of this systemic autoimmune condition which results in multiorgan failure because of multiple small vessel occlusions. Objective To analyse the clinical and laboratory characteristics of patients with catastrophic APS who develop ARDS. Methods Cases with ARDS were selected from the web site based international registry of patients with catastrophic APS (CAPS registry) (http://www.med.ub.es/MIMMUN/FORUM/CAPS.HTM) and their characteristics examined. Results Pulmonary involvement was reported in 150 of 220 patients with catastrophic APS (68%) and 47 patients (21%) were diagnosed as having ARDS. Nineteen (40%) of these patients died. Pathological studies were undertaken in 10 patients and thrombotic microangiopathy was present in seven. There were no differences in age, sex, precipitating factors, clinical manifestations, or mortality between catastrophic APS patients with and without ARDS. Conclusions ARDS is the dominant pulmonary manifestation of catastrophic APS. Thus the existence of ARDS in the context of an APS makes it necessary to rule out the presence of the catastrophic variant of this syndrome. PMID:15919677

  6. Severe Acute Respiratory Distress Syndrome during Infliximab Therapy in a Patient with Crohn Disease

    PubMed Central

    Schoehl, Johanna; Mechie, Nicolae-Catalin; Schwoerer, Harald; Moerer, Onnen; Quintel, Michael; Buck, Cordula; Ellenrieder, Volker; Neesse, Albrecht; Amanzada, Ahmad

    2016-01-01

    The occurrence of a noninfectious interstitial lung disease is a rare but life-threatening side effect of infliximab, an antitumor necrosis factor alpha antibody. The following case report of a patient with Crohn disease shows an extremely dramatic progression to a severe acute respiratory distress syndrome. PMID:27920644

  7. A case of Clostridium difficile infection complicated by acute respiratory distress syndrome treated with fecal microbiota transplantation.

    PubMed

    Kim, Ji Eun; Gweon, Tae-Geun; Yeo, Chang Dong; Cho, Young-Seok; Kim, Gi Jun; Kim, Jae Young; Kim, Jong Wook; Kim, Hyunho; Lee, Hye Won; Lim, Taeseok; Ham, Hyoju; Oh, Hyun Jin; Lee, Yeongbok; Byeon, Jaeho; Park, Sung Soo

    2014-09-21

    Acute respiratory distress syndrome is a life-threatening disorder caused mainly by pneumonia. Clostridium difficile infection (CDI) is a common nosocomial diarrheal disease. Disruption of normal intestinal flora by antibiotics is the main risk factor for CDI. The use of broad-spectrum antibiotics for serious medical conditions can make it difficult to treat CDI complicated by acute respiratory distress syndrome. Fecal microbiota transplantation is a highly effective treatment in patients with refractory CDI. Here we report on a patient with refractory CDI and acute respiratory distress syndrome caused by pneumonia who was treated with fecal microbiota transplantation.

  8. The role of inhaled prostacyclin in treating acute respiratory distress syndrome.

    PubMed

    Searcy, Randi J; Morales, James R; Ferreira, Jason A; Johnson, Donald W

    2015-12-01

    Acute respiratory distress syndrome (ARDS) is a syndrome of acute lung injury that is characterized by noncardiogenic pulmonary edema and severe hypoxemia second to a pathogenic impairment of gas exchange. Despite significant advances in the area, mortality remains high among ARDS patients. High mortality and a limited spectrum of therapeutic options have left clinicians searching for alternatives, spiking interest in selective pulmonary vasodilators (SPVs). Despite the lack of robust evidence, SPVs are commonly employed for their therapeutic role in improving oxygenation in patients who have developed refractory hypoxemia in ARDS. While inhaled epoprostenol (iEPO) also impacts arterial oxygenation by decreasing ventilation-perfusion (V/Q) mismatching and pulmonary shunt flow, this effect is not different from inhaled nitric oxide (iNO). The most effective and safest dose for yielding a clinically significant increase in PaO2 and reduction in pulmonary artery pressure (PAP) appears to be 20-30 ng/kg/min in adults and 30 ng/kg/min in pediatric patients. iEPO appears to have a ceiling effect above these doses in which no additional benefit may be derived. iNO and iEPO have shown similar efficacy profiles; however, they differ with respect to cost and ease of therapeutic administration. The most beneficial effects of iEPO have been seen in adult patients with secondary ARDS as compared with primary ARDS, most likely due to the difference in etiology of the two disease states, and in patients suffering from baseline right ventricular heart failure. Although iEPO has demonstrated improvements in hemodynamic parameters and oxygenation in ARDS patients, due to the limited number of randomized clinical trials and the lack of studies investigating mortality, the use of iEPO cannot be recommended as standard of care in ARDS. iEPO should be reserved for those refractory to traditional therapies.

  9. A Healthy Young Woman with Acute Respiratory Distress Syndrome: an unfamiliar face of a familiar disease

    PubMed Central

    Sheybani, Fereshte; Naderi, Hamid Reza; Moghaddam, Ahmad Bagheri; Amiri, Bezat

    2016-01-01

    The presented case features a rare manifestation of pulmonary tuberculosis in a previously healthy young woman who had acute presentation of tuberculous pneumonia complicated by acute respiratory distress syndrome. In developing countries, mycobacterium tuberculosis is an important cause of community-acquired pneumonia (CAP). TB can present as an acute process and should be included in the differential diagnosis of CAP. This case is special in its manifestation from several clinical perspectives, including the lack of an underlying medical condition or immune defect and the development of acute respiratory distress syndrome (ARDS) in non-miliary and non-disseminated tuberculosis. In conclusion, the diagnosis of TB should be considered in all patients who present with CAP in endemic regions. PMID:27957312

  10. Individualized positive end-expiratory pressure application in patients with acute respiratory distress syndrome.

    PubMed

    Pintado, M C; de Pablo, R

    2014-11-01

    Current treatment of acute respiratory distress syndrome is based on ventilatory support with a lung protective strategy, avoiding the development of iatrogenic injury, including ventilator-induced lung injury. One of the mechanisms underlying such injury is atelectrauma, and positive end-expiratory pressure (PEEP) is advocated in order to avoid it. The indicated PEEP level has not been defined, and in many cases is based on the patient oxygen requirements for maintaining adequate oxygenation. However, this strategy does not consider the mechanics of the respiratory system, which varies in each patient and depends on many factors-including particularly the duration of acute respiratory distress syndrome. A review is therefore made of the different methods for adjusting PEEP, focusing on the benefits of individualized application.

  11. Acute respiratory distress syndrome in burn patients: incidence and risk factor analysis.

    PubMed

    Silva, L; Garcia, L; Oliveira, B; Tanita, M; Festti, J; Cardoso, L; Lavado, L; Grion, C

    2016-09-30

    After a burn lesion, Acute Respiratory Distress Syndrome (ARDS) may occur via direct lung injury due to inhaled smoke and fumes or mediated by the inflammatory response associated with the burn or its infectious complications. The aim of the present study is to assess the epidemiologic profile of ARDS in adult burn patients admitted to intensive care in a burn unit at a university hospital. A prospective cohort study was performed from January to December 2012. Demographic and diagnostic data, prognostic scores, etiology and data on the extent and depth of burns were collected. Data related to risk factors for ARDS and death were also recorded. A total of 85 patients were included in the study. Patients were aged 41.7 (SD = 15.7) years old; 71.8% were male and the mean total body surface area burned was 28.3% (SD = 19.1%); 35.3% presented inhalation injuries. Invasive ventilatory support was required in 44 ICU inpatients (51.8%). ARDS was diagnosed in 38.6% of patients under invasive mechanical ventilation. In multivariate analysis, the presence of inhalation injuries was a risk factor for ARDS (OR = 9.75; CI 95% 2.79 - 33.95; P < 0.001). ARDS is a common complication in burn patients admitted to specialized intensive care units. Inhalation injuries were an independent risk factor for ARDS. Mortality rate observed in the study patients was high and associated with ARDS diagnosis.

  12. Acute respiratory distress syndrome in burn patients: incidence and risk factor analysis

    PubMed Central

    Silva, L.; Garcia, L.; Oliveira, B.; Tanita, M.; Festti, J.; Cardoso, L.; Lavado, L.; Grion, C.

    2016-01-01

    Summary After a burn lesion, Acute Respiratory Distress Syndrome (ARDS) may occur via direct lung injury due to inhaled smoke and fumes or mediated by the inflammatory response associated with the burn or its infectious complications. The aim of the present study is to assess the epidemiologic profile of ARDS in adult burn patients admitted to intensive care in a burn unit at a university hospital. A prospective cohort study was performed from January to December 2012. Demographic and diagnostic data, prognostic scores, etiology and data on the extent and depth of burns were collected. Data related to risk factors for ARDS and death were also recorded. A total of 85 patients were included in the study. Patients were aged 41.7 (SD = 15.7) years old; 71.8% were male and the mean total body surface area burned was 28.3% (SD = 19.1%); 35.3% presented inhalation injuries. Invasive ventilatory support was required in 44 ICU inpatients (51.8%). ARDS was diagnosed in 38.6% of patients under invasive mechanical ventilation. In multivariate analysis, the presence of inhalation injuries was a risk factor for ARDS (OR = 9.75; CI 95% 2.79 – 33.95; P < 0.001). ARDS is a common complication in burn patients admitted to specialized intensive care units. Inhalation injuries were an independent risk factor for ARDS. Mortality rate observed in the study patients was high and associated with ARDS diagnosis. PMID:28149245

  13. Prevention of adult respiratory distress syndrome with plasminogen activator in pigs.

    PubMed

    Hardaway, R M; Williams, C H; Marvasti, M; Farias, M; Tseng, A; Pinon, I; Yanez, D; Martinez, M; Navar, J

    1990-12-01

    Death from traumatic shock has been associated with loss of blood externally or internally. However, many patients die after trauma, even though blood volume restoration is adequate. Death is often due to pulmonary failure (adult respiratory distress syndrome [ARDS]). Death and ARDS have been associated with disseminated intravascular coagulation (DIC) and microclots in the lungs. Dissolution of the microclots after trauma can be achieved by activation of endogenous plasmin. Nine pigs were anesthetized for 48 h. Trauma was administered by 60 standard blows to each thigh resulting in a bruise of muscle but no skin, bone, or major vessel injury. Nutrition and respiration were maintained at normal levels. All nine pigs died with severe lung pathology and low PaO2. Ten other traumatized pigs were treated with a plasminogen activator iv 4 h after trauma. Five of these were treated with tissue plasminogen activator (tPA) and five with urokinase. All treated pigs survived 48 h and maintained a normal PaO2. Autopsy showed minimal lung pathology.

  14. Should Immune-Enhancing Formulations Be Used for Patients With Acute Respiratory Distress Syndrome?

    PubMed

    Roosevelt, Hannah

    2016-08-01

    The potential for regulating immune function in acute respiratory distress syndrome (ARDS) through enteral-administered anti-inflammatory lipids has generated much interest over the past 20 years. Yet recommendations remain inconclusive regarding the utilization of ω-3 fatty acids in patients with ARDS and acute lung injury (ALI). Studies are limited in number, with differing methods, small sample sizes, and conflicting results, making recommendations difficult to interpret.

  15. Non lineal respiratory systems mechanics simulation of acute respiratory distress syndrome during mechanical ventilation.

    PubMed

    Madorno, Matias; Rodriguez, Pablo O

    2010-01-01

    Model and simulation of biological systems help to better understand these systems. In ICUs patients often reach a complex situation where supportive maneuvers require special expertise. Among them, mechanical ventilation in patients suffering from acuter respiratory distress syndrome (ARDS) is specially challenging. This work presents a model which can be simulated and use to help in training of physicians and respiratory therapists to analyze the respiratory mechanics in this kind of patients. We validated the model in 2 ARDS patients.

  16. Severe acute respiratory distress syndrome in a child with malaria: favorable response to prone positioning.

    PubMed

    Flores, Jose C; Imaz, Ana; López-Herce, Jesús; Seriñá, Carlota

    2004-03-01

    We present the case of a 4-year-old boy with malaria who developed acute respiratory distress syndrome with severe hypoxemia refractory to mechanical ventilation and inhaled nitric oxide. Placing the patient in prone position immediately and persistently improved oxygenation: the ratio of P(aO(2)) to fraction of inspired oxygen rose from 47 to 180 mm Hg and the oxygenation index decreased from 40 to 11. The patient survived, with no respiratory sequelae.

  17. Interleukin-10 polymorphism in position -1082 and acute respiratory distress syndrome

    PubMed Central

    Gong, M.N.; Thompson, B.T.; Williams, P.L.; Zhou, W.; Wang, M.Z.; Pothier, L.; Christiani, D.C.

    2009-01-01

    The GG genotype of the interleukin (IL)-10 promoter polymorphism in position -1082 (-1082GG) has been associated with increased IL-10 production. The current authors hypothesised that the -1082GG genotype is associated with the development of, and outcomes in, acute respiratory distress syndrome (ARDS). A nested case-control study was conducted in 211 Caucasian cases of ARDS and 429 controls who were admitted to an intensive care unit with sepsis, trauma, aspiration or massive transfusions. Cases were followed for organ failure and 60-day mortality. The -1082GG genotype was associated with the development of ARDS, but only in the presence of a significant interaction between the -1082GG genotype and age. Among patients with ARDS, the -1082GG genotype was associated with decreased severity of illness on admission, lower daily organ dysfunction scores and lower 60-day mortality. In conclusion, the high interleukin-10-producing -1082GG genotype may be associated with variable odds for acute respiratory distress syndrome development depending on age. Among those with acute respiratory distress syndrome, the -1082GG genotype is associated with lower mortality and organ failure. Further studies are needed to confirm these findings. PMID:16585075

  18. A new view of pulmonary edema and acute respiratory distress syndrome.

    PubMed

    Ketai, L H; Godwin, J D

    1998-07-01

    The old division of lung edema into two categories--cardiogenic (hydrostatic) and noncardiogenic (increased permeability)--is no longer adequate. For instance, it fails to distinguish between the capillary leak caused by acute respiratory distress syndrome from that caused by interleukin-2 treatment. Further, it fails to account for the capillary leak ('stress-failure') that may accompany edema. A modern view of edema must recognize the natural barriers to the formation and spread of edema. These barriers are the capillary endothelium and the alveolar epithelium. Varying degrees of damage to them can account for the varying radiographic and clinical manifestations of lung edema. Thus, interleukin-2 administration causes increased endothelial permeability without causing alveolar epithelial damage. The result is lung edema that is largely confined to the interstitium, causing little hypoxia and clearing rapidly. However, acute respiratory distress syndrome, which is characterized by extensive alveolar damage, causes air-space consolidation, severe hypoxia, and slow resolution. Thus, a reasonable classification of lung edema requires at least four categories: 1) hydrostatic edema; 2) acute respiratory distress syndrome (permeability edema caused by diffuse alveolar damage); 3) permeability edema without alveolar damage; and (4) mixed hydrostatic and permeability edema. The authors emphasize the importance of the barriers provided by the capillary endothelium and the alveolar epithelium in determining the clinical and radiographic manifestations of edema. In general, when the alveolar epithelium is intact, the radiographic manifestations are those of interstitial (not air-space) edema; this radiographic pattern predicts a mild clinical course and prompt resolution.

  19. Chest associated to motor physiotherapy improves cardiovascular variables in newborns with respiratory distress syndrome

    PubMed Central

    2011-01-01

    Background We aimed to evaluate the effects of chest and motor physiotherapy treatment on hemodynamic variables in preterm newborns with respiratory distress syndrome. Methods We evaluated heart rate (HR), respiratory rate (RR), systolic (SAP), mean (MAP) and diastolic arterial pressure (DAP), temperature and oxygen saturation (SO2%) in 44 newborns with respiratory distress syndrome. We compared all variables between before physiotherapy treatment vs. after the last physiotherapy treatment. Newborns were treated during 11 days. Variables were measured 2 minutes before and 5 minutes after each physiotherapy treatment. We applied paired Student t test to compare variables between the two periods. Results HR (148.5 ± 8.5 bpm vs. 137.1 ± 6.8 bpm - p < 0.001), SAP (72.3 ± 11.3 mmHg vs. 63.6 ± 6.7 mmHg - p = 0.001) and MAP (57.5 ± 12 mmHg vs. 47.7 ± 5.8 mmHg - p = 0.001) were significantly reduced after 11 days of physiotherapy treatment compared to before the first session. There were no significant changes regarding RR, temperature, DAP and SO2%. Conclusions Chest and motor physiotherapy improved cardiovascular parameters in respiratory distress syndrome newborns. PMID:22029840

  20. On the complexity of scoring acute respiratory distress syndrome: do not forget hemodynamics!

    PubMed Central

    Repessé, Xavier; Aubry, Alix

    2016-01-01

    Acute respiratory distress syndrome (ARDS) remains associated with a poor outcome despite recent major therapeutic advances. Forecasting the outcome of patients suffering from such a syndrome is of a crucial interest and many scores have been proposed, all suffering from limits responsible for important discrepancies. Authors try to elaborate simple, routine and reliable scores but most of them do not consider hemodynamics yet acknowledged as a major determinant of outcome. This article aims at reminding the approach of scoring in ARDS and at deeply describing the most recently published one in order to highlight their main pitfall, which is to forget the hemodynamics. PMID:27618840

  1. Restrictive transfusion practice during extracorporeal membrane oxygenation therapy for severe acute respiratory distress syndrome.

    PubMed

    Voelker, Maria T; Busch, Thilo; Bercker, Sven; Fichtner, Falk; Kaisers, Udo X; Laudi, Sven

    2015-04-01

    Recommendations concerning the management of hemoglobin levels and hematocrit in patients on extracorporeal membrane oxygenation (ECMO) still advise maintenance of a normal hematocrit. In contrast, current transfusion guidelines for critically ill patients support restrictive transfusion practice. We report on a series of patients receiving venovenous ECMO (vvECMO) for acute respiratory distress syndrome (ARDS) treated according to the restrictive transfusion regimen recommended for critically ill patients. We retrospectively analyzed 18 patients receiving vvECMO due to severe ARDS. Hemoglobin concentrations were kept between 7 and 9 g/dL with a transfusion trigger at 7 g/dL or when physiological transfusion triggers were apparent. We assessed baseline data, hospital mortality, time on ECMO, hemoglobin levels, hematocrit, quantities of packed red blood cells received, and lactate concentrations and compared survivors and nonsurvivors. The overall mortality of all patients on vvECMO was 38.9%. Mean hemoglobin concentration over all patients and ECMO days was 8.30 ± 0.51 g/dL, and hematocrit was 0.25 ± 0.01, with no difference between survivors and nonsurvivors. Mean numbers of given PRBCs showed a trend towards higher quantities in the group of nonsurvivors, but the difference was not significant (1.97 ± 1.47 vs. 0.96 ± 0.76 units; P = 0.07). Mean lactate clearance from the first to the third day was 45.4 ± 28.3%, with no significant difference between survivors and nonsurvivors (P = 0.19). In our cohort of patients treated with ECMO due to severe ARDS, the application of a restrictive transfusion protocol did not result in an increased mortality. Safety and feasibility of the application of a restrictive transfusion protocol in patients on ECMO must further be evaluated in randomized controlled trials.

  2. Tension Among Women in North India: An Idiom of Distress and a Cultural Syndrome.

    PubMed

    Weaver, Lesley Jo

    2017-03-01

    The existing literature on Indian ethnopsychology has long asserted that somatization is a key aspect of experiences of distress. The study of idioms of distress arose out of work done in India (Nichter in Cult Med Psychiatry 5(4):379-408, 1981), but ironically, little subsequent work has systematically explored idioms of distress in this part of the world. This ethnographic study focused on the term tension (tenśan) and its relation to a cultural syndrome among women in urban North India. This syndrome appears to involve rapid-onset anger, irritation, rumination, and sleeplessness as key symptoms. It is often linked to specific circumstances such as domestic conflict and is associated with the stresses of modern urban life. People who report more symptoms of tension had consistently higher scores on the Hopkins Symptoms Checklist-25 for depression and anxiety. In this cultural context where psychiatric care is highly stigmatized, the language of tension can aid providers of mental healthcare (many of whom, in India, are not psychiatrists or psychologists) to identify and communicate effectively with potential patients whose mental healthcare needs might otherwise go unaddressed.

  3. Spinal muscular atrophy with respiratory distress syndrome (SMARD1): Case report and review of literature

    PubMed Central

    Lingappa, Lokesh; Shah, Nikit; Motepalli, Ananth Sagar; Shaik, Farhan

    2016-01-01

    Spinal muscular atrophy with respiratory distress syndrome (SMARD1) is a rare cause of early infantile respiratory failure and death. No cases have been currently described from India. Two low-birth-weight infants presented prior to 6 months of age with recurrent apnea and respiratory distress. Both required prolonged ventilation, and had distal arthrogryposis and diaphragmatic eventration. Nerve conduction study revealed motor sensory axonopathy. Genetic testing confirmed mutations in immunoglobulin mu binding protein (IGHMBP2). These two cases establish presence of SMARD1 in our population. Both infants died on discontinuation of ventilation. Antenatal diagnoses done in one pregnancy. Though rare, high index of suspicion is essential in view of poor outcome and aid antenatal counseling. PMID:27570397

  4. Improvement in Functioning and Psychological Distress in Adolescents With Postural Orthostatic Tachycardia Syndrome Following Interdisciplinary Treatment.

    PubMed

    Bruce, Barbara K; Harrison, Tracy E; Bee, Susan M; Luedtke, Connie A; Porter, Co-Burn J; Fischer, Philip R; Hayes, Sarah E; Allman, Daniel A; Ale, Chelsea M; Weiss, Karen E

    2016-03-15

    Significant functional impairment and psychological distress have been observed in adolescent patients with postural orthostatic tachycardia syndrome (POTS). Interdisciplinary rehabilitation programs have been shown to be beneficial in the treatment of chronic pain in adults and adolescents. Only preliminary data have examined interdisciplinary rehabilitation efforts in patients with POTS. This study evaluated the impact of an interdisciplinary rehabilitation program on the functional impairment and psychological distress in 33 adolescents diagnosed with POTS. Patients included in the study were adolescents ages 11 to 18 diagnosed with POTS. Measures completed at admission and discharge from the program included the Functional Disability Index, Center for Epidemiological Studies-Depression-Child scale, and the Pain Catastrophizing Scale for Children. After participation in the 3-week program, adolescents with POTS demonstrated a significant increase in overall functional ability and significant reductions in depression and catastrophizing.

  5. [Mitochondrial and microcirculatory distress syndrome in the critical patient. Therapeutic implications].

    PubMed

    Navarrete, M L; Cerdeño, M C; Serra, M C; Conejero, R

    2013-10-01

    Mitochondrial and microcirculatory distress syndrome (MMDS) can occur during systemic inflammatory response syndrome (SIRS), and is characterized by cytopathic tissue hypoxia uncorrected by oxygen transport optimization, and associated with an acquired defect in the use of oxygen and energy production in mitochondria, leading to multiple organ dysfunction (MOD). We examine the pathogenesis of MMDS, new diagnostic methods, and recent therapeutic approaches adapted to each of the three phases in the evolution of the syndrome. In the initial phase, the aim is prevention and early reversal of mitochondrial dysfunction. Once the latter is established, the aim is to restore flow of the electron chain, mitochondrial respiration, and to avoid cellular energy collapse. Finally, in the third (resolution) stage, treatment should focus on stimulating mitochondrial biogenesis and the repair or replacement of damaged mitochondria.

  6. Use of a dual lumen cannula for venovenous extra corporeal membrane oxygenation in a patient with acute respiratory distress syndrome and a previously inserted inferior vena cava filter: a case report

    PubMed Central

    Palizas Jr., Fernando; García, Christian Casabella; Norese, Mariano

    2016-01-01

    Extracorporeal membrane oxygenation is used in refractory hypoxemia in many clinical settings. Thoracic trauma patients usually develop acute respiratory distress syndrome. Due to high risk of bleeding, thrombotic complications present in this context are particularly difficult to manage and usually require insertion of an inferior vena cava filter to prevent embolism from the distal veins to the pulmonary circulation. Here, we present a case of a thoracic trauma patient with severe acute respiratory distress syndrome requiring venovenous extracorporeal membrane oxygenation via a right internal jugular double lumen cannula due to a previously inserted inferior vena cava filter caused by distal bilateral calf muscle vein deep vein thrombosis. PMID:27096680

  7. Anxiety, depression and distress among irritable bowel syndrome and their subtypes: An epidemiological population based study

    PubMed Central

    Roohafza, Hamidreza; Bidaki, Ehsan Zare; Hasanzadeh-Keshteli, Ammar; Daghaghzade, Hamed; Afshar, Hamid; Adibi, Peyman

    2016-01-01

    Background: Psychiatric disorders are common in irritable bowel syndrome (IBS). We conducted this study to investigate the relationship of IBS and their subtypes with some of psychological factors. Materials and Methods: A cross-sectional study was performed among 4763 staff of Isfahan University of Medical Sciences in 2011. Modified ROME III questionnaire and Talley Bowel Disease Questionnaire were used to evaluate IBS symptoms. Hospital Anxiety and Depression Scale and 12-item General Health Questionnaire were utilized to assess anxiety, depression and psychological distress. Logistic regression analysis was used to determine the association of psychological states and IBS in the total subject and both genders. Results: About, 4763 participants with mean age 36/58 ± 8/09 were included the 2106 males and 2657 females. Three thousand and seven hundred and seventy-six (81.2%) and 2650 (57.2%) participants were married and graduated respectively. Subtype analysis of IBS and its relationship with anxiety, depression and distress comparing the two genders can be observed that: IBS and clinically-significant IBS have higher anxiety, depression symptoms, and distress than the subject without IBS (P < 0.001). Women with IBS, have higher scores than men (P < 0.001). Compared to other subtypes, mixed IBS subtype has a higher anxiety, depression, and distress score. Conclusion: A high prevalence of anxiety, depression symptoms and distress in our subjects emphasize the importance of the psychological evaluation of the patients with IBS, in order to better management of the patients and may also help to reduce the burden of health care costs. PMID:28028523

  8. AB022. A case of severe leptospirosis with acute respiratory distress syndrome

    PubMed Central

    Akritidou, Sofia; Panagiotidou, Evangelia; Sourla, Evdokia; Konstanta, Soultana; Kotoulas, Serafim-Xrisovalantis; Bikos, Vasilios; Bagalas, Vasilios; Katalin, Fekete; Pitsiou, Georgia; Ioannis, Stanopoulos; Athanasia, Pataka

    2016-01-01

    Leptospirosis is a microbial infection which occurs in humans and animals and is caused by Leptospira (Leptospira spp.). In Greece, it occurs at a frequency of 0.22:100,000, and 24 severe cases have been reported in 2013. It manifests in three forms: anicteric (90%), icteric (5–10%) and severe. Severe leptospirosis can be a rare cause of acute respiratory distress syndrome (ARDS), leading to intubation, jaundice, alveolar bleeding and multi-organ failure. A 71-year-old male patient presented at the emergency department with dyspnea on exertion, fever, gastrointestinal disorders, muscle aches and fatigue, that started four days ago. Occupation with pigeons was also reported. Blood gas analysis revealed severe type I respiratory failure, and a chest CT was performed, revealing ARDS. The patient was intubated. Acute renal failure (urea: 238, creatinine: 4.81) that required renal replacement therapy developed along with increased bilirubin (max value of total: 8.2 with direct: 7.42), and positive direct and indirect Coombs test. Moreover, hemorrhage through the tracheal tube complicated the clinical condition, resulting to obstruction of tracheal tube. Furthermore, anemia, thrombocytopenia and severe leycocytosis were also observed (white blood cells: 42,620). Diffuse alveolar hemorrhage due to vasculitis and connective tissue disease was suspected, and a complete immunological control was ordered. Infectious pathogens, such as Str. Pneumoniae, Legionella, Mycoplasma, Leptospira spp., Chlamidia, HBV, HCV, influenza were also suspected and all the necessary samples were tested. He received levofloxacin, piperacillin/tazobactam, vibramycin, and oseltamivir, but after receiving the positive results for leptospira, antibiotic treatment was revised with the final choice of levofloxacin and piperacillin/tazobactam. Despite improvement of renal and liver function, leycocytosis, CRP and PCT values, high fever begun that was attributed at first to the Jarisch

  9. Foster Kennedy Syndrome Due to Meningioma Growth during Pregnancy

    PubMed Central

    Rodríguez-Porcel, Federico; Hughes, Ian; Anderson, Douglas; Lee, John; Biller, José

    2013-01-01

    Tumors of the olfactory groove may cause unilateral optic atrophy with contralateral papilledema and anosmia (Foster Kennedy syndrome). We describe a case of a young pregnant woman with Foster Kennedy syndrome due to an olfactory groove meningioma. PMID:24273529

  10. Substance P and neutral endopeptidase in development of acute respiratory distress syndrome following fire smoke inhalation.

    PubMed

    Wong, Simon S; Sun, Nina N; Lantz, R Clark; Witten, Mark L

    2004-10-01

    To characterize the tachykininergic effects in fire smoke (FS)-induced acute respiratory distress syndrome (ARDS), we designed a series of studies in rats. Initially, 20 min of FS inhalation induced a significant increase of substance P (SP) in bronchoalveolar lavage fluid (BALF) at 1 h and persisted for 24 h after insult. Conversely, FS disrupted 51.4, 55.6, 46.3, and 43.0% enzymatic activity of neutral endopeptidase (NEP, a primary hydrolyzing enzyme for SP) 1, 6, 12, and 24 h after insult, respectively. Immunolabeling density of NEP in the airway epithelium largely disappeared 1 h after insult due to acute cell damage and shedding. These changes were also accompanied by extensive influx of albumin and granulocytes/lymphocytes in BALF. Furthermore, levels of BALF SP and tissue NEP activity dose dependently increased and decreased, respectively, following 0, low (10 min), and high (20 min) levels of FS inhalation. However, neither the time-course nor the dose-response study observed a significant change in the highest affinity neurokinin-1 receptor (NK-1R) for SP. Finally, treatment (10 mg/kg im) with SR-140333B, an NK-1R antagonist, significantly prevented 20-min FS-induced hypoxemia and pulmonary edema 24 h after insult. Further examination indicated that SR-140333B (1.0 or 10.0 mg/kg im) fully abolished early (1 h) plasma extravasation following FS. Collectively, these findings suggest that a combination of sustained SP and NEP inactivity induces an exaggerated neurogenic inflammation mediated by NK-1R, which may lead to an uncontrolled influx of protein-rich edema fluid and cells into the alveoli as a consequence of increased vascular permeability.

  11. Acute respiratory distress syndrome and acute renal failure from Plasmodium ovale infection with fatal outcome

    PubMed Central

    2013-01-01

    Background Plasmodium ovale is one of the causative agents of human malaria. Plasmodium ovale infection has long been thought to be non-fatal. Due to its lower morbidity, P. ovale receives little attention in malaria research. Methods Two Malaysians went to Nigeria for two weeks. After returning to Malaysia, they fell sick and were admitted to different hospitals. Plasmodium ovale parasites were identified from blood smears of these patients. The species identification was further confirmed with nested PCR. One of them was successfully treated with no incident of relapse within 12-month medical follow-up. The other patient came down with malaria-induced respiratory complication during the course of treatment. Although parasites were cleared off the circulation, the patient’s condition worsened. He succumbed to multiple complications including acute respiratory distress syndrome and acute renal failure. Results Sequencing of the malaria parasite DNA from both cases, followed by multiple sequence alignment and phylogenetic tree construction suggested that the causative agent for both malaria cases was P. ovale curtisi. Discussion In this report, the differences between both cases were discussed, and the potential capability of P. ovale in causing severe complications and death as seen in this case report was highlighted. Conclusion Plasmodium ovale is potentially capable of causing severe complications, if not death. Complete travel and clinical history of malaria patient are vital for successful diagnoses and treatment. Monitoring of respiratory and renal function of malaria patients, regardless of the species of malaria parasites involved is crucial during the course of hospital admission. PMID:24180319

  12. Use of lung ultrasound in detection of complications of respiratory distress syndrome.

    PubMed

    Sawires, Happy K; Abdel Ghany, Eman A; Hussein, Nouran F; Seif, Hadeel M

    2015-09-01

    Repeated chest radiography is required for the diagnosis and follow-up of neonates with respiratory distress syndrome (RDS) and carries the risk of radiation hazards. Lung ultrasound (LUS) is a non-invasive bedside diagnostic tool that has proven to be effective in the diagnosis of RDS. Our aim was to assess the role of LUS with respect to the standard chest X-ray (CXR) in the detection of complications of RDS in neonates. Ninety premature newborns of both genders with RDS (mean gestational age = 29.91 ± 1.33 wk) and 40 premature babies as a control group were involved in this study. All patients underwent initial clinical assessment as well as CXR and LUS. Those who presented with respiratory distress and/or exhibited deterioration of oxygenation parameters were followed by CXR and, within 4 h, by LUS. Alveolo-interstitial syndrome and pleural line abnormalities were detected in all cases (100%) in the initial assessment, patchy consolidation was detected in 34 cases and white lung was detected in 80 cases. Alveolo-interstitial syndrome was detected in 19 controls. In follow-up of the patients, LUS was superior to CXR in detection of consolidation and sub-pleural atelectasis, but not in detection of pneumothorax. We concluded that bedside LUS is a good non-hazardous alternative tool in the early detection and follow-up of RDS in the neonatal intensive care unit; it could be of value in reducing exposure to unnecessary radiation.

  13. Severity of Acute Respiratory Distress Syndrome in haematology patients: long-term impact and early predictive factors.

    PubMed

    Lagier, D; Platon, L; Chow-Chine, L; Sannini, A; Bisbal, M; Brun, J-P; Blache, J-L; Faucher, M; Mokart, D

    2016-09-01

    Severe forms of acute respiratory distress syndrome in patients with haematological diseases expose clinicians to specific medical and ethical considerations. We prospectively followed 143 patients with haematological malignancies, and whose lungs were mechanically ventilated for more than 24 h, over a 5-y period. We sought to identify prognostic factors of long-term outcome, and in particular to evaluate the impact of the severity of acute respiratory distress syndrome in these patients. A secondary objective was to identify the early (first 48 h from ICU admission) predictive factors for acute respiratory distress syndrome severity. An evolutive haematological disease (HR 1.71; 95% CI 1.13-2.58), moderate to severe acute respiratory distress syndrome (HR 1.81; 95% CI 1.13-2.69) and need for renal replacement therapy (HR 2.24; 95% CI 1.52-3.31) were associated with long-term mortality. Resolution of neutropaenia during ICU stay (HR 0.63; 95% CI 0.42-0.94) and early microbiological documentation (HR 0.62; 95% CI 0.42-0.91) were associated with survival. The extent of pulmonary infiltration observed on the first chest X-ray and the diagnosis of invasive fungal infection were the most relevant early predictive factors of the severity of acute respiratory distress syndrome.

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

    PubMed

    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.

  15. [Acute respiratory distress syndrome in childhood: Changing definition and news from the Pediatric Consensus Conference].

    PubMed

    Dauger, S; Le Bourgeois, F; Guichoux, J; Brissaud, O

    2017-03-23

    Acute respiratory distress syndrome (ARDS) is a rapidly progressive hypoxemic respiratory insufficiency induced by alveolar filling mainly caused by alveolocapillary wall disruption, following direct or indirect pulmonary injury. Much less frequent in children than in adults, pediatric intensivists had long applied adult guidelines to their daily practice. In 2015, experts from the Pediatric Acute Lung Injury Consensus Conference (PALICC) published the first international guidelines specifically dedicated to pediatric ARDS. After a short summary of the history of the ARDS definition since its first report in 1967, we describe the main diagnostic and therapeutic guidelines for PALICC.

  16. Pneumothorax in patients with acute respiratory distress syndrome: pathophysiology, detection, and treatment.

    PubMed

    Woodside, Kenneth J; vanSonnenberg, Eric; Chon, Kenneth S; Loran, David B; Tocino, Irena M; Zwischenberger, Joseph B

    2003-01-01

    Pneumothorax is a frequent and potentially fatal complication of mechanical ventilation in patients with acute respiratory distress syndrome (ARDS). Prompt recognition and treatment of pneumothoraces is necessary to minimize morbidity and mortality. The radiologic and clinical signs of pneumothoraces in ARDS patients may have unusual and subtle features. Furthermore, small pneumothoraces in these patients can cause severe hemodynamic or pulmonary compromise. Sparse clinical literature exists on when or how to treat pneumothoraces once they develop in patients with ARDS. In this article, the authors review the pathogenesis, radiologic signs, clinical significance, and treatment of pneumothoraces in ARDS patients. Treatment options include traditional tube thoracostomy, open thoracotomy, and image-guided percutaneous catheters.

  17. Acute Respiratory Distress Syndrome: Role of Oleic Acid-Triggered Lung Injury and Inflammation

    PubMed Central

    Gonçalves-de-Albuquerque, Cassiano Felippe; Silva, Adriana Ribeiro; Burth, Patrícia; Castro-Faria, Mauro Velho; Castro-Faria-Neto, Hugo Caire

    2015-01-01

    Lung injury especially acute respiratory distress syndrome (ARDS) can be triggered by diverse stimuli, including fatty acids and microbes. ARDS affects thousands of people worldwide each year, presenting high mortality rate and having an economic impact. One of the hallmarks of lung injury is edema formation with alveoli flooding. Animal models are used to study lung injury. Oleic acid-induced lung injury is a widely used model resembling the human disease. The oleic acid has been linked to metabolic and inflammatory diseases; here we focus on lung injury. Firstly, we briefly discuss ARDS and secondly we address the mechanisms by which oleic acid triggers lung injury and inflammation. PMID:26640323

  18. Fluid Management With a Simplified Conservative Protocol for the Acute Respiratory Distress Syndrome*

    PubMed Central

    Grissom, Colin K.; Hirshberg, Eliotte L.; Dickerson, Justin B.; Brown, Samuel M.; Lanspa, Michael J.; Liu, Kathleen D.; Schoenfeld, David; Tidswell, Mark; Hite, R. Duncan; Rock, Peter; Miller, Russell R.; Morris, Alan H.

    2015-01-01

    Objectives In the Fluid and Catheter Treatment Trial (FACTT) of the National Institutes of Health Acute Respiratory Distress Syndrome Network, a conservative fluid protocol (FACTT Conservative) resulted in a lower cumulative fluid balance and better outcomes than a liberal fluid protocol (FACTT Liberal). Subsequent Acute Respiratory Distress Syndrome Network studies used a simplified conservative fluid protocol (FACTT Lite). The objective of this study was to compare the performance of FACTT Lite, FACTT Conservative, and FACTT Liberal protocols. Design Retrospective comparison of FACTT Lite, FACTT Conservative, and FACTT Liberal. Primary outcome was cumulative fluid balance over 7 days. Secondary outcomes were 60-day adjusted mortality and ventilator-free days through day 28. Safety outcomes were prevalence of acute kidney injury and new shock. Setting ICUs of Acute Respiratory Distress Syndrome Network participating hospitals. Patients Five hundred three subjects managed with FACTT Conservative, 497 subjects managed with FACTT Liberal, and 1,124 subjects managed with FACTT Lite. Interventions Fluid management by protocol. Measurements and Main Results Cumulative fluid balance was 1,918 ± 323 mL in FACTT Lite, −136 ±491 mL in FACTT Conservative, and 6,992 ± 502 mL in FACTT Liberal (p < 0.001). Mortality was not different between groups (24% in FACTT Lite, 25% in FACTT Conservative and Liberal, p = 0.84). Ventilator-free days in FACTT Lite (14.9 ±0.3) were equivalent to FACTT Conservative (14.6±0.5) (p = 0.61) and greater than in FACTT Liberal (12.1 ±0.5, p < 0.001 vs Lite). Acute kidney injury prevalence was 58% in FACTT Lite and 57% in FACTT Conservative (p = 0.72). Prevalence of new shock in FACTT Lite (9%) was lower than in FACTT Conservative (13%) (p = 0.007 vs Lite) and similar to FACTT Liberal (11%) (p = 0.18 vs Lite). Conclusions FACTT Lite had a greater cumulative fluid balance than FACTT Conservative but had equivalent clinical and safety outcomes

  19. Acute Respiratory Distress Syndrome after Viscum album Pleurodesis for Primary Spontaneous Pneumothorax

    PubMed Central

    Noh, Dongsub; Park, Joon Suk; Lee, Doo Yun

    2017-01-01

    A 52-year-old male patient who underwent multiple wedge resections experienced postoperative acute respiratory distress syndrome in both lungs after Viscum album pleurodesis. Despite initial rapid deterioration in clinical condition and rapid progression of bilateral lung infiltration, he exhibited a relatively smooth clinical recovery with marked response to glucocorticoid treatment. Our case report suggests that care must be taken to guard against the development of acute respiratory complications in the use of Viscum album for pleurodesis. However, in view of the clinically benign course, initial aggressive management of complications can prevent suffering and sequelae. PMID:28180108

  20. Acute respiratory distress syndrome and lung fibrosis after ingestion of a high dose of ortho-phenylphenol.

    PubMed

    Cheng, Shih-Lung; Wang, Hao-Chien; Yang, Pan-Chyr

    2005-08-01

    Ortho-phenylphenol (OPP) and its sodium salt are used as fungicides and antibacterial agents, ingestion of which has been found to cause liver toxicity, renal toxicity and carcinomas in the urinary tract of rats. Lung damage due to OPP ingestion has not been reported in humans. We report a suicidal 39-year-old woman with stage II cervical cancer who drank a potentially lethal dose of OPP in the form of a commercial antiseptic, which led to the complication of liver and renal function impairment, severe lung damage with acute respiratory distress syndrome and subsequent severe lung fibrosis. Open lung biopsy showed diffuse alveolar damage. She was discharged after 34 days of hospitalization with continuing domiciliary oxygen therapy.

  1. Acute respiratory distress syndrome (ARDS) treated successfully by veno-venous extracorporeal membrane oxygenation (ECMO) in a nearly drowned patient.

    PubMed

    Sonoo, Tomohiro; Ohshima, Kazuma; Kobayashi, Hiroaki; Asada, Toshifumi; Hiruma, Takahiro; Doi, Kento; Gunshin, Masataka; Murakawa, Tomohiro; Anraku, Masaki; Nakajima, Susumu; Nakajima, Jun; Yahagi, Naoki

    2014-09-01

    This report highlights about one acute respiratory distress syndrome (ARDS) case after near-drowning resuscitated using extracorporeal membrane oxygenation (ECMO). Few cases have been reported about ECMO use for near-drowning and in most of these cases, ECMO was initiated within the first week. However, in our report, we would like to emphasize that seemingly irreversible secondary worsening of ARDS after nearly drowned patient was successfully treated by ECMO use more than 1 week after near-drowning followed by discharge without home oxygen therapy, social support, or any complication. This is probably due to sufficient lung rest for ventilator-associated lung injury during ECMO use. Based on our case's clinical course, intensive care unit physicians must consider ECMO even in the late phase of worsened ARDS after near-drowning.

  2. Psychological distress in newly diagnosed colorectal cancer patients following microsatellite instability testing for Lynch syndrome on the pathologist's initiative.

    PubMed

    Landsbergen, K M; Prins, J B; Brunner, H G; van Duijvendijk, P; Nagengast, F M; van Krieken, J H; Ligtenberg, M; Hoogerbrugge, N

    2012-06-01

    According to the Dutch Guideline on Hereditary Colorectal Cancer published in 2008, patients with recently diagnosed colorectal cancer (CRC) should undergo microsatellite instability (MSI) testing by a pathologist immediately after tumour resection if they are younger than 50 years, or if a second CRC has been diagnosed before the age of 70 years, owing to the high risk of Lynch syndrome (MIPA). The aim of the present MIPAPS study was to investigate general distress and cancer-specific distress following MSI testing. From March 2007 to September 2009, 400 patients who had been tested for MSI after newly diagnosed CRC were recruited from 30 Dutch hospitals. Levels of general distress (SCL-90) and cancer-specific distress (IES) were assessed immediately after MSI result disclosure (T1) and 6 months later (T2). Response rates were 23/77 (30%) in the MSI-positive patients and 58/323 (18%) in the MSI-negative patients. Levels of general distress and cancer-specific distress were moderate. In the MSI-positive group, 27% of the patients had high general distress at T1 versus 18% at T2 (p = 0.5), whereas in the MSI-negative group, these percentage were 14 and 18% (p = 0.6), respectively. At T1 and T2, cancer-specific distress rates in the MSI-positive group and MSI-negative group were 39 versus 27% (p = 0.3) and 38 versus 36% (p = 1.0), respectively. High levels of general distress were correlated with female gender, low social support and high perceived cancer risk. Moderate levels of distress were observed after MSI testing, similar to those found in other patients diagnosed with CRC. Immediately after result disclosure, high cancer-specific distress was observed in 40% of the MSI-positive patients.

  3. Biomarkers in acute respiratory distress syndrome: from pathobiology to improving patient care.

    PubMed

    Walter, James M; Wilson, Jennifer; Ware, Lorraine B

    2014-10-01

    Acute respiratory distress syndrome (ARDS) is a clinical syndrome characterized by alveolar flooding with protein-rich pulmonary edema fluid. Despite an improved understanding of ARDS pathogenesis, our ability to predict the development of ARDS and risk-stratify patients with the disease remains limited. Biomarkers may help identify patients at highest risk of developing ARDS, assess response to therapy, predict outcome, and optimize enrollment in clinical trials. This review begins with a general description of biomarker use in clinical medicine. We then review evidence that supports the value of various ARDS biomarkers organized by the cellular injury processes central to ARDS development: endothelial injury, epithelial injury, disordered inflammation and coagulation, fibrosis, and apoptosis. Finally, we summarize the growing contributions of genomic and proteomic research and suggest ways in which the field may evolve in the coming years.

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

    PubMed

    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.

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

    PubMed

    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).

  6. Epidemiological profile of acute respiratory distress syndrome patients: A tertiary care experience

    PubMed Central

    Magazine, Rahul; Rao, Shobitha; Chogtu, Bharti; Venkateswaran, Ramkumar; Shahul, Hameed Aboobackar; Goneppanavar, Umesh

    2017-01-01

    Background: Acute respiratory distress syndrome (ARDS) is seen in critically ill patients. Its etiological spectrum in India is expected to be different from that seen in western countries due to the high prevalence of tropical infections. Aim: To study the epidemiological profile of ARDS patients. Setting: A tertiary care hospital in Karnataka, India. Materials and Methods: Retrospective analysis of 150 out of the 169 ARDS patients diagnosed during 2010–2012. Data collected included the clinical features and severity scoring parameters. Results: The mean age of the study population was 42.92 ± 13.91 years. The causes of ARDS included pneumonia (n = 35, 23.3%), scrub typhus (n = 33, 22%), leptospirosis (n = 11, 7.3%), malaria (n = 6, 4%), influenza (H1N1) (n = 10, 6.7%), pulmonary tuberculosis (n = 2, 1.3%), dengue (n = 1, 0.7%), abdominal sepsis (n = 16, 10.7%), skin infection (n = 3, 2%), unknown cause of sepsis (n = 18, 12%), and nonseptic causes (n = 15, 10%). A total of 77 (51.3%) patients survived, 66 (44%) expired, and 7 (4.7%) were discharged against medical advice (AMA). Preexisting comorbidities (46) were present in 13 survivors, 19 nonsurvivors, and four discharged AMA. History of surgery prior to the onset of ARDS was present in one survivor, 13 nonsurvivors, and one discharge AMA. Mean Acute Physiology and Chronic Health Evaluation (APACHE) II, APACHE III, and Sequential Organ Failure Assessment scores in survivors were 9.06 ± 4.3, 49.22 ± 14, and 6.43 ± 2.5 and in nonsurvivors 21.11 ± 7, 86.45 ± 23.5, and 10.6 ± 10, respectively. Conclusion: The most common cause of ARDS in our study was pneumonia, but a large percentage of cases were due to the tropical infections. Preexisting comorbidity, surgery prior to the onset of ARDS, higher severity scores, and organ failure scores were more frequently observed among nonsurvivors than survivors. PMID:28144059

  7. Compartment Syndrome of the Calf Due to Nicolau Syndrome

    PubMed Central

    Enshaei, Ali; Afshar, Ahmadreza

    2016-01-01

    We report a case of Nicolau syndrome in a 15 months old girl following an intramuscular injection of penicillin 6.3.3 in her left buttock. This case is unique because she developed compartment syndrome in her left calf far from her injection site. Her toe’s tips gangrened in the course of her ailment. We hypothesized that the compartment syndrome might be produced by a probable intra-arterial injection that had produced embolic obstruction of the small and medium size arteries in her leg or a probable perineural or periarteial injection had produced secondary sympathetic stimulation, extensive vasospasm, compromised microcirculation and the development of compartment syndrome. PMID:26894227

  8. Compartment Syndrome of the Calf Due to Nicolau Syndrome.

    PubMed

    Enshaei, Ali; Afshar, Ahmadreza

    2016-01-01

    We report a case of Nicolau syndrome in a 15 months old girl following an intramuscular injection of penicillin 6.3.3 in her left buttock. This case is unique because she developed compartment syndrome in her left calf far from her injection site. Her toe's tips gangrened in the course of her ailment. We hypothesized that the compartment syndrome might be produced by a probable intra-arterial injection that had produced embolic obstruction of the small and medium size arteries in her leg or a probable perineural or periarteial injection had produced secondary sympathetic stimulation, extensive vasospasm, compromised microcirculation and the development of compartment syndrome.

  9. Hemiplegia due to Churg Strauss syndrome in a young boy.

    PubMed

    Kumar, Nitin; Vaish, A K

    2011-03-01

    We report here a case of a 13-year-old boy with Churg Strauss Syndrome who presented with acute right sided hemiplegia with slurred speech due to cerebral infarction most likely due to cerebral vasculitis. The boy was treated with steroids and aspirin and improved. This case in interesting as Churg Strauss Syndrome is not so common in pediatric age group.

  10. Sudden Death Due to Undiagnosed Wilkie Syndrome.

    PubMed

    Baber, Yeliena Fay; OʼDonnell, Chris

    2016-06-01

    A 56-year-old transgender woman with mitochondrial encephalomyopathy, lactic acidosis, and strokelike episodes syndrome and diabetes presented to hospital with headaches and experiencing with malnutrition. She was agitated and refused medical and physical assistance. Soon after admission, she started to vomit and developed abdominal pain, becoming rapidly unresponsive on the ward after attending the radiology department, and was pronounced deceased. Autopsy revealed a cachectic transgender woman with a grossly distended stomach and proximal duodenum containing 2 L of liquid. The postmortem computed tomography scan showed compression of the duodenum by the superior mesenteric artery, diagnostic of Wilkie syndrome. Superior mesenteric artery syndrome, or Wilkie syndrome, was first described in 1861 by Von Rokitansky. It is an uncommon but well-recognized clinical entity characterized by compression of the third, or transverse, portion of the duodenum between the aorta and the superior mesenteric artery. This results in chronic, intermittent, or acute complete or partial duodenal obstruction. It is a well-recognized complication of anorexia.

  11. Predicting School Readiness from Neurodevelopmental Assessments at Age 2 Years after Respiratory Distress Syndrome in Infants Born Preterm

    ERIC Educational Resources Information Center

    Patrianakos-Hoobler, Athena I.; Msall, Michael E.; Huo, Dezheng; Marks, Jeremy D.; Plesha-Troyke, Susan; Schreiber, Michael D.

    2010-01-01

    Aim: To determine whether neurodevelopmental outcomes at the age of 2 years accurately predict school readiness in children who survived respiratory distress syndrome after preterm birth. Method: Our cohort included 121 preterm infants who received surfactant and ventilation and were enrolled in a randomized controlled study of inhaled nitric…

  12. Surgical lung biopsy to diagnose Behcet's vasculitis with adult respiratory distress syndrome

    PubMed Central

    Vydyula, Ravikanth; Allred, Charles; Huartado, Mariana; Mina, Bushra

    2014-01-01

    A 34-year-old female presented with fever and abdominal pain. Past medical history includes Crohn's and Behcet's disease. Examination revealed multiple skin ulcerations, oral aphthae, and bilateral coarse rales. She developed respiratory distress with diffuse bilateral alveolar infiltrates on chest radiograph requiring intubation. PaO2/FiO2 ratio was 132. The chest computed tomography revealed extensive nodular and patchy ground-glass opacities. Bronchoalveolar lavage demonstrated a predominance of neutrophils. Methylprednisolone 60 mg every 6 h and broad-spectrum antimicrobials were initiated. No infectious etiologies were identified. Surgical lung biopsy demonstrated diffuse alveolar damage (DAD) mixed with lymphocytic and necrotizing vasculitis with multiple small infarcts and thrombi consistent with Behcet's vasculitis. As she improved, steroids were tapered and discharged home on oral cyclophosphamide. Pulmonary involvement in Behcet's is unusual and commonly manifests as pulmonary artery aneurysms, thrombosis, infarction, and hemorrhage. Lung biopsy findings demonstrating DAD are consistent with the clinical diagnosis of adult respiratory distress syndrome. The additional findings of necrotizing vasculitis and infarcts may have led to DAD. PMID:25378849

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

    PubMed

    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.

  14. Role of inflammatory mediators in the pathophysiology of acute respiratory distress syndrome.

    PubMed

    Bhatia, Madhav; Moochhala, Shabbir

    2004-02-01

    Inflammatory response leading to organ dysfunction and failure continues to be the major problem after injury in many clinical conditions such as sepsis, severe burns, acute pancreatitis, haemorrhagic shock, and trauma. In general terms, systemic inflammatory response syndrome (SIRS) is an entirely normal response to injury. Systemic leukocyte activation, however, is a direct consequence of a SIRS and if excessive, can lead to distant organ damage and multiple organ dysfunction syndrome (MODS). When SIRS leads to MODS and organ failure, the mortality becomes high and can be more than 50%. Acute lung injury that clinically manifests as acute respiratory distress syndrome (ARDS) is a major component of MODS of various aetiologies. Inflammatory mediators play a key role in the pathogenesis of ARDS, which is the primary cause of death in these conditions. This review summarizes recent studies that demonstrate the critical role played by inflammatory mediators such as tumour necrosis factor (TNF)-alpha, interleukin (IL)-1beta, IL-6, platelet activating factor (PAF), IL-10, granulocyte macrophage-colony stimulating factor (GM-CSF), C5a, intercellular adhesion molecule (ICAM)-1, substance P, chemokines, VEGF, IGF-I, KGF, reactive oxygen species (ROS), and reactive nitrogen species (RNS) in the pathogenesis of ARDS. It is reasonable to speculate that elucidation of the key mediators in ARDS coupled with the discovery of specific inhibitors would make it possible to develop clinically effective anti-inflammatory therapy.

  15. CXCL10/IP-10 Neutralization Can Ameliorate Lipopolysaccharide-Induced Acute Respiratory Distress Syndrome in Rats

    PubMed Central

    Lang, Shan; Li, Libing; Wang, Xuning; Sun, Junping; Xue, Xinying; Xiao, Yongjiu; Zhang, Mingyue; Ao, Ting; Wang, Jianxin

    2017-01-01

    The role of C-X-C motif chemokine 10 (CXCL10), a pro-inflammatory factor, in the development of acute respiratory distress syndrome (ARDS) remains unclear. In this study, we explored the role of CXCL10 and the effect of CXCL10 neutralization in lipopolysaccharide (LPS)-induced ARDS in rats. The expression of CXCL10 and its receptor chemokine receptor 3(CXCR3) increased after LPS induction. Moreover, neutralization of CXCL10 ameliorated the severity of ARDS by reducing pulmonary edema, inhibiting the release of inflammatory mediators (IFN-γ, IL-6 and ICAM-1) and limiting inflammatory cells (neutrophils, macrophages, CD8+ T cells) influx into the lung, with a reduction in CXCR3 expression in neutrophils and macrophages. Therefore, CXCL10 could be a potential therapeutic target in LPS-induced ARDS. PMID:28046003

  16. Argument against the Routine Use of Steroids for Pediatric Acute Respiratory Distress Syndrome

    PubMed Central

    Hartmann, Silvia M.; Hough, Catherine L.

    2016-01-01

    Steroids have a plausible mechanism of action of reducing severity of lung disease in acute respiratory distress syndrome (ARDS) but have failed to show consistent benefits in patient-centered outcomes. Many studies have confounding from the likely presence of ventilator-induced lung injury and steroids may have shown benefit because administration minimized ongoing inflammation incited by injurious ventilator settings. If steroids have benefit, it is likely for specific populations that fall within the heterogeneous diagnosis of ARDS. Those pediatric patients with concurrent active asthma or reactive airway disease of prematurity, in addition to ARDS, are the most common group likely to derive benefit from steroids, but are poorly studied. With the information currently available, it does not appear that the typical adult or pediatric patient with ARDS derives benefit from steroids and steroids should not be given on a routine basis. PMID:27517035

  17. Lung Postmortem Autopsy Revealing Extramedullary Involvement in Multiple Myeloma Causing Acute Respiratory Distress Syndrome

    PubMed Central

    Ravinet, Aurélie; Perbet, Sébastien; Guièze, Romain; Guérin, Renaud; Gayraud, Guillaume; Aliane, Jugurtha; Tremblay, Aymeric; Pascal, Julien; Ledoux, Albane; Chaleteix, Carine; Dechelotte, Pierre; Bay, Jacques-Olivier; Bazin, Jean-Etienne; Constantin, Jean-Michel

    2014-01-01

    Pulmonary involvement with multiple myeloma is rare. We report the case of a 61-year-old man with past medical history of chronic respiratory failure with emphysema, and a known multiple myeloma (Durie and Salmon stage III B and t(4;14) translocation). Six months after diagnosis and first line of treatment, he presented acute dyspnea with interstitial lung disease. Computed tomography showed severe bullous emphysema and diffuse, patchy, multifocal infiltrations bilaterally with nodular character, small bilateral pleural effusions, mediastinal lymphadenopathy, and a known lytic lesion of the 12th vertebra. He was treated with piperacillin-tazobactam, amikacin, oseltamivir, and methylprednisolone. Finally, outcome was unfavourable. Postmortem analysis revealed diffuse and nodular infracentimetric infiltration of the lung parenchyma by neoplastic plasma cells. Physicians should be aware that acute respiratory distress syndrome not responding to treatment of common causes could be a manifestation of the disease, even with negative BAL or biopsy and could be promptly treated with salvage therapy. PMID:25165587

  18. The role of ventilation-induced surfactant dysfunction and atelectasis in causing acute respiratory distress syndrome.

    PubMed

    Albert, Richard K

    2012-04-01

    This Pulmonary Perspective describes a new pathophysiologic scenario by which the acute respiratory distress syndrome (ARDS) might develop, summarizes the literature on which this new scenario is based, and discusses the resulting implications with respect to patient management. Rather than ARDS occurring as a result of the inflammatory response associated with predisposing risk factors, the proposed scenario theorizes that the initiating problem is atelectasis that develops as a result of a surfactant abnormality that is caused by spontaneous or mechanical ventilation, together with our current approaches to patient positioning and sedation. The proposed pathophysiology implies that ventilation-induced lung injury occurs before, and causes, ARDS (rather than developing after the fact and only serving to magnify the existing injury) and that some instances of ARDS are iatrogenic. If the proposed scenario is correct, it also implies that at least some instances of ARDS might be prevented by implementing a number of simple, safe modifications in patient care.

  19. Mechanical ventilation in acute respiratory distress syndrome: The open lung revisited.

    PubMed

    Amado-Rodríguez, L; Del Busto, C; García-Prieto, E; Albaiceta, G M

    2017-02-23

    Acute respiratory distress syndrome (ARDS) is still related to high mortality and morbidity rates. Most patients with ARDS will require ventilatory support. This treatment has a direct impact upon patient outcome and is associated to major side effects. In this regard, ventilator-associated lung injury (VALI) is the main concern when this technique is used. The ultimate mechanisms of VALI and its management are under constant evolution. The present review describes the classical mechanisms of VALI and how they have evolved with recent findings from physiopathological and clinical studies, with the aim of analyzing the clinical implications derived from them. Lastly, a series of knowledge-based recommendations are proposed that can be helpful for the ventilator assisted management of ARDS at the patient bedside.

  20. Viral Infection in the Development and Progression of Pediatric Acute Respiratory Distress Syndrome

    PubMed Central

    Nye, Steven; Whitley, Richard J.; Kong, Michele

    2016-01-01

    Viral infections are an important cause of pediatric acute respiratory distress syndrome (ARDS). Numerous viruses, including respiratory syncytial virus (RSV) and influenza A (H1N1) virus, have been implicated in the progression of pneumonia to ARDS; yet the incidence of progression is unknown. Despite acute and chronic morbidity associated with respiratory viral infections, particularly in “at risk” populations, treatment options are limited. Thus, with few exceptions, care is symptomatic. In addition, mortality rates for viral-related ARDS have yet to be determined. This review outlines what is known about ARDS secondary to viral infections including the epidemiology, the pathophysiology, and diagnosis. In addition, emerging treatment options to prevent infection, and to decrease disease burden will be outlined. We focused on RSV and influenza A (H1N1) viral-induced ARDS, as these are the most common viruses leading to pediatric ARDS, and have specific prophylactic and definitive treatment options. PMID:27933286

  1. Clinical review: Acute respiratory distress syndrome - clinical ventilator management and adjunct therapy.

    PubMed

    Silversides, Jonathan A; Ferguson, Niall D

    2013-04-29

    Acute respiratory distress syndrome (ARDS) is a potentially devastating form of acute inflammatory lung injury with a high short-term mortality rate and significant long-term consequences among survivors. Supportive care, principally with mechanical ventilation, remains the cornerstone of therapy - although the goals of this support have changed in recent years - from maintaining normal physiological parameters to avoiding ventilator-induced lung injury while providing adequate gas exchange. In this article we discuss the current evidence base for ventilatory support and adjunctive therapies in patients with ARDS. Key components of such a strategy include avoiding lung overdistension by limiting tidal volumes and airway pressures, and the use of positive end-expiratory pressure with or without lung recruitment manoeuvres in patients with severe ARDS. Adjunctive therapies discussed include pharmacologic techniques (for example, vasodilators, diuretics, neuromuscular blockade) and nonpharmacologic techniques (for example, prone position, alternative modes of ventilation).

  2. Viral Infection in the Development and Progression of Pediatric Acute Respiratory Distress Syndrome.

    PubMed

    Nye, Steven; Whitley, Richard J; Kong, Michele

    2016-01-01

    Viral infections are an important cause of pediatric acute respiratory distress syndrome (ARDS). Numerous viruses, including respiratory syncytial virus (RSV) and influenza A (H1N1) virus, have been implicated in the progression of pneumonia to ARDS; yet the incidence of progression is unknown. Despite acute and chronic morbidity associated with respiratory viral infections, particularly in "at risk" populations, treatment options are limited. Thus, with few exceptions, care is symptomatic. In addition, mortality rates for viral-related ARDS have yet to be determined. This review outlines what is known about ARDS secondary to viral infections including the epidemiology, the pathophysiology, and diagnosis. In addition, emerging treatment options to prevent infection, and to decrease disease burden will be outlined. We focused on RSV and influenza A (H1N1) viral-induced ARDS, as these are the most common viruses leading to pediatric ARDS, and have specific prophylactic and definitive treatment options.

  3. Extracorporeal life support for patients with acute respiratory distress syndrome: report of a Consensus Conference.

    PubMed

    Richard, Christian; Argaud, Laurent; Blet, Alice; Boulain, Thierry; Contentin, Laetitia; Dechartres, Agnès; Dejode, Jean-Marc; Donetti, Laurence; Fartoukh, Muriel; Fletcher, Dominique; Kuteifan, Khaldoun; Lasocki, Sigismond; Liet, Jean-Michel; Lukaszewicz, Anne-Claire; Mal, Hervé; Maury, Eric; Osman, David; Outin, Hervé; Richard, Jean-Christophe; Schneider, Francis; Tamion, Fabienne

    2014-01-01

    The influenza H1N1 epidemics in 2009 led a substantial number of people to develop severe acute respiratory distress syndrome and refractory hypoxemia. In these patients, extracorporeal membrane oxygenation was used as rescue oxygenation therapy. Several randomized clinical trials and observational studies suggested that extracorporeal membrane oxygenation associated with protective mechanical ventilation could improve outcome, but its efficacy remains uncertain. Organized by the Société de Réanimation de Langue Française (SRLF) in conjunction with the Société Française d'Anesthésie et de Réanimation (SFAR), the Société de Pneumologie de Langue Française (SPLF), the Groupe Francophone de Réanimation et d'Urgences Pédiatriques (GFRUP), the Société Française de Perfusion (SOFRAPERF), the Société Française de Chirurgie Thoracique et Cardiovasculaire (SFCTV) et the Sociedad Española de Medecina Intensiva Critica y Unidades Coronarias (SEMICYUC), a Consensus Conference was held in December 2013 and a jury of 13 members wrote 65 recommendations to answer the five following questions regarding the place of extracorporeal life support for patients with acute respiratory distress syndrome: 1) What are the available techniques?; 2) Which patients could benefit from extracorporeal life support?; 3) How to perform extracorporeal life support?; 4) How and when to stop extracorporeal life support?; 5) Which organization should be recommended? To write the recommendations, evidence-based medicine (GRADE method), expert panel opinions, and shared decisions taken by all the thirteen members of the jury of the Consensus Conference were taken into account.

  4. Extracorporeal life support for patients with acute respiratory distress syndrome: report of a Consensus Conference

    PubMed Central

    2014-01-01

    The influenza H1N1 epidemics in 2009 led a substantial number of people to develop severe acute respiratory distress syndrome and refractory hypoxemia. In these patients, extracorporeal membrane oxygenation was used as rescue oxygenation therapy. Several randomized clinical trials and observational studies suggested that extracorporeal membrane oxygenation associated with protective mechanical ventilation could improve outcome, but its efficacy remains uncertain. Organized by the Société de Réanimation de Langue Française (SRLF) in conjunction with the Société Française d’Anesthésie et de Réanimation (SFAR), the Société de Pneumologie de Langue Française (SPLF), the Groupe Francophone de Réanimation et d’Urgences Pédiatriques (GFRUP), the Société Française de Perfusion (SOFRAPERF), the Société Française de Chirurgie Thoracique et Cardiovasculaire (SFCTV) et the Sociedad Española de Medecina Intensiva Critica y Unidades Coronarias (SEMICYUC), a Consensus Conference was held in December 2013 and a jury of 13 members wrote 65 recommendations to answer the five following questions regarding the place of extracorporeal life support for patients with acute respiratory distress syndrome: 1) What are the available techniques?; 2) Which patients could benefit from extracorporeal life support?; 3) How to perform extracorporeal life support?; 4) How and when to stop extracorporeal life support?; 5) Which organization should be recommended? To write the recommendations, evidence-based medicine (GRADE method), expert panel opinions, and shared decisions taken by all the thirteen members of the jury of the Consensus Conference were taken into account. PMID:24936342

  5. Obstructive Sleep Apnea, Obesity, and the Development of Acute Respiratory Distress Syndrome

    PubMed Central

    Karnatovskaia, Lioudmila V.; Lee, Augustine S.; Bender, S. Patrick; Talmor, Daniel; Festic, Emir

    2014-01-01

    Background: Obstructive sleep apnea (OSA) may increase the risk of respiratory complications and acute respiratory distress syndrome (ARDS) among surgical patients. OSA is more prevalent among obese individuals; obesity can predispose to ARDS. Hypothesis: It is unclear whether OSA independently contributes towards the risk of ARDS among hospitalized patients. Methods: This is a pre-planned retrospective subgroup analysis of the prospectively identified cohort of 5,584 patients across 22 hospitals with at least one risk factor for ARDS at the time of hospitalization from a trial by the US Critical Illness and Injury Trials Group designed to validate the Lung Injury Prediction Score. A total of 252 patients (4.5%) had a diagnosis of OSA at the time of hospitalization; of those, 66% were obese. Following multivariate adjustment in the logistic regression model, there was no significant relationship between OSA and development of ARDS (OR = 0.65, 95%CI = 0.32-1.22). However, body mass index (BMI) was associated with subsequent ARDS development (OR = 1.02, 95%CI = 1.00-1.04, p = 0.03). Neither OSA nor BMI affected mechanical ventilation requirement or mortality. Conclusions: Prior diagnosis of OSA did not independently affect development of ARDS among patients with at least one predisposing condition, nor the need for mechanical ventilation or hospital mortality. Obesity appeared to independently increase the risk of ARDS. Citation: Karnatovskaia LV, Lee AS, Bender SP, Talmor D, Festic E. Obstructive sleep apnea, obesity, and the development of acute respiratory distress syndrome. J Clin Sleep Med 2014;10(6):657-662. PMID:24932146

  6. ANLN truncation causes a familial fatal acute respiratory distress syndrome in Dalmatian dogs

    PubMed Central

    Syrjä, Pernilla; Arumilli, Meharji; Järvinen, Anna-Kaisa; Rajamäki, Minna

    2017-01-01

    Acute respiratory distress syndrome (ARDS) is the leading cause of death in critical care medicine. The syndrome is typified by an exaggerated inflammatory response within the lungs. ARDS has been reported in many species, including dogs. We have previously reported a fatal familial juvenile respiratory disease accompanied by occasional unilateral renal aplasia and hydrocephalus, in Dalmatian dogs. The condition with a suggested recessive mode of inheritance resembles acute exacerbation of usual interstitial pneumonia in man. We combined SNP-based homozygosity mapping of two ARDS-affected Dalmatian dogs and whole genome sequencing of one affected dog to identify a case-specific homozygous nonsense variant, c.31C>T; p.R11* in the ANLN gene. Subsequent analysis of the variant in a total cohort of 188 Dalmatians, including seven cases, indicated complete segregation of the variant with the disease and confirmed an autosomal recessive mode of inheritance. Low carrier frequency of 1.7% was observed in a population cohort. The early nonsense variant results in a nearly complete truncation of the ANLN protein and immunohistochemical analysis of the affected lung tissue demonstrated the lack of the membranous and cytoplasmic staining of ANLN protein in the metaplastic bronchial epithelium. The ANLN gene encodes an anillin actin binding protein with a suggested regulatory role in the integrity of intercellular junctions. Our study suggests that defective ANLN results in abnormal cellular organization of the bronchiolar epithelium, which in turn predisposes to acute respiratory distress. ANLN has been previously linked to a dominant focal segmental glomerulosclerosis in human without pulmonary defects. However, the lack of similar renal manifestations in the affected Dalmatians suggest a novel ANLN-related pulmonary function and disease association. PMID:28222102

  7. Prevalence and Impact of Active and Passive Cigarette Smoking in Acute Respiratory Distress Syndrome

    PubMed Central

    Hsieh, S. Jean; Zhuo, Hanjing; Benowitz, Neal L.; Thompson, B. Taylor; Liu, Kathleen D.; Matthay, Michael A.; Calfee, Carolyn S.

    2014-01-01

    Objective Cigarette smoke exposure has recently been found to be associated with increased susceptibility to trauma- and transfusion-associated acute respiratory distress syndrome (ARDS). We sought to determine 1) the prevalence of cigarette smoke exposure in a diverse multi-center sample of ARDS patients, and 2) whether cigarette smoke exposure is associated with severity of lung injury and mortality in ARDS. Design Analysis of the Albuterol for the Treatment of ALI (ALTA) and Omega ARDS Network studies. Setting Acute Respiratory Distress Syndrome Network hospitals. Patients Three hundred eighty one patients with ARDS. Interventions None. Measurements NNAL (4-(methylnitrosamino)-1-(3-pyridyl)-1-butanol), a validated tobacco-specific marker, was measured in urine samples from subjects enrolled in two NHLBI ARDS Network randomized controlled trials. Main Results Urine NNAL levels were consistent with active smoking in 36% of ARDS patients and with passive smoking in 41% of nonsmokers (vs 20% and 40% in general population, respectively). Patients with NNAL levels in the active smoking range were younger and had a higher prevalence of alcohol misuse, fewer comorbidities, lower severity of illness, and less septic shock at enrollment compared to patients with undetectable NNAL levels. Despite this lower severity of illness, the severity of lung injury did not significantly differ based on biomarker-determined smoking status. Cigarette smoke exposure was not significantly associated with death after adjusting for differences in age, alcohol use, comorbidities, and severity of illness. Conclusions In this first multicenter study of biomarker-determined cigarette smoke exposure in ARDS patients, we found that active cigarette smoke exposure was significantly more prevalent among ARDS patients compared to population averages. Despite their younger age, better overall health, and lower severity of illness, smokers by NNAL had similar severity of lung injury as patients with

  8. Steven-Johnson syndrome due to unknown drugs [corrected].

    PubMed

    Shivamurthy, Raghu Prasada M; Kallappa, Ravindra; Reddy, Shashikala G H; Rangappa, Druva Kumar B

    2012-01-01

    Steven-Johnson syndrome may be considered as a cytotoxic immune reaction to drugs, infections etc. This is a case report of Steven-Johnson syndrome due to an ayurvedic preparation which was used in the treatment of mental retardation in a young girl.

  9. Steven-Johnson syndrome due to ayurvedic drugs

    PubMed Central

    Shivamurthy, Raghu Prasada M.; Kallappa, Ravindra; Reddy, Shashikala G. H.; Rangappa, Druva Kumar B.

    2012-01-01

    Steven–Johnson syndrome may be considered as a cytotoxic immune reaction to drugs, infections etc. This is a case report of Steven–Johnson syndrome due to an ayurvedic preparation which was used in the treatment of mental retardation in a young girl. PMID:22345890

  10. Recovery and outcomes after the acute respiratory distress syndrome (ARDS) in patients and their family caregivers.

    PubMed

    Herridge, Margaret S; Moss, Marc; Hough, Catherine L; Hopkins, Ramona O; Rice, Todd W; Bienvenu, O Joseph; Azoulay, Elie

    2016-05-01

    Outcomes after acute respiratory distress syndrome (ARDS) are similar to those of other survivors of critical illness and largely affect the nerve, muscle, and central nervous system but also include a constellation of varied physical devastations ranging from contractures and frozen joints to tooth loss and cosmesis. Compromised quality of life is related to a spectrum of impairment of physical, social, emotional, and neurocognitive function and to a much lesser extent discrete pulmonary disability. Intensive care unit-acquired weakness (ICUAW) is ubiquitous and includes contributions from both critical illness polyneuropathy and myopathy, and recovery from these lesions may be incomplete at 5 years after ICU discharge. Cognitive impairment in ARDS survivors ranges from 70 to 100 % at hospital discharge, 46 to 80 % at 1 year, and 20 % at 5 years, and mood disorders including depression and post-traumatic stress disorder (PTSD) are also sustained and prevalent. Robust multidisciplinary and longitudinal interventions that improve these outcomes are still uncertain and data in our literature are conflicting. Studies are needed in family members of ARDS survivors to better understand long-term outcomes of the post-ICU family syndrome and to evaluate how it affects patient recovery.

  11. [Genetic predisposition and Pediatric Acute Respiratory Distress Syndrome: New tools for genetic study].

    PubMed

    Erranz, M Benjamín; Wilhelm, B Jan; Riquelme, V Raquel; Cruces, R Pablo

    2015-01-01

    Acute respiratory distress syndrome (ARDS) is the most severe form of respiratory failure. Theoretically, any acute lung condition can lead to ARDS, but only a small percentage of individuals actually develop the disease. On this basis, genetic factors have been implicated in the risk of developing ARDS. Based on the pathophysiology of this disease, many candidate genes have been evaluated as potential modifiers in patient, as well as in animal models, of ARDS. Recent experimental data and clinical studies suggest that variations of genes involved in key processes of tissue, cellular and molecular lung damage may influence susceptibility and prognosis of ARDS. However, the pathogenesis of pediatric ARDS is complex, and therefore, it can be expected that many genes might contribute. Genetic variations such as single nucleotide polymorphisms and copy-number variations are likely associated with susceptibility to ARDS in children with primary lung injury. Genome-wide association (GWA) studies can objectively examine these variations, and help identify important new genes and pathogenetic pathways for future analysis. This approach might also have diagnostic and therapeutic implications, such as predicting patient risk or developing a personalized therapeutic approach to this serious syndrome.

  12. Efficacy of prone position in acute respiratory distress syndrome patients: A pathophysiology-based review

    PubMed Central

    Koulouras, Vasilios; Papathanakos, Georgios; Papathanasiou, Athanasios; Nakos, Georgios

    2016-01-01

    Acute respiratory distress syndrome (ARDS) is a syndrome with heterogeneous underlying pathological processes. It represents a common clinical problem in intensive care unit patients and it is characterized by high mortality. The mainstay of treatment for ARDS is lung protective ventilation with low tidal volumes and positive end-expiratory pressure sufficient for alveolar recruitment. Prone positioning is a supplementary strategy available in managing patients with ARDS. It was first described 40 years ago and it proves to be in alignment with two major ARDS pathophysiological lung models; the “sponge lung” - and the “shape matching” -model. Current evidence strongly supports that prone positioning has beneficial effects on gas exchange, respiratory mechanics, lung protection and hemodynamics as it redistributes transpulmonary pressure, stress and strain throughout the lung and unloads the right ventricle. The factors that individually influence the time course of alveolar recruitment and the improvement in oxygenation during prone positioning have not been well characterized. Although patients’ response to prone positioning is quite variable and hard to predict, large randomized trials and recent meta-analyses show that prone position in conjunction with a lung-protective strategy, when performed early and in sufficient duration, may improve survival in patients with ARDS. This pathophysiology-based review and recent clinical evidence strongly support the use of prone positioning in the early management of severe ARDS systematically and not as a rescue maneuver or a last-ditch effort. PMID:27152255

  13. Intravenous Vitamin C Administered as Adjunctive Therapy for Recurrent Acute Respiratory Distress Syndrome

    PubMed Central

    Bharara, Amit; Grossman, Catherine; Syed, Aamer; DeWilde, Christine

    2016-01-01

    This case report summarizes the first use of intravenous vitamin C employed as an adjunctive interventional agent in the therapy of recurrent acute respiratory distress syndrome (ARDS). The two episodes of ARDS occurred in a young female patient with Cronkhite-Canada syndrome, a rare, sporadically occurring, noninherited disorder that is characterized by extensive gastrointestinal polyposis and malabsorption. Prior to the episodes of sepsis, the patient was receiving nutrition via chronic hyperalimentation administered through a long-standing central venous catheter. The patient became recurrently septic with Gram positive cocci which led to two instances of ARDS. This report describes the broad-based general critical care of a septic patient with acute respiratory failure that includes fluid resuscitation, broad-spectrum antibiotics, and vasopressor support. Intravenous vitamin C infused at 50 mg per kilogram body weight every 6 hours for 96 hours was incorporated as an adjunctive agent in the care of this patient. Vitamin C when used as a parenteral agent in high doses acts “pleiotropically” to attenuate proinflammatory mediator expression, to improve alveolar fluid clearance, and to act as an antioxidant. PMID:27891260

  14. Local abnormalities in coagulation and fibrinolytic pathways predispose to alveolar fibrin deposition in the adult respiratory distress syndrome.

    PubMed Central

    Idell, S; James, K K; Levin, E G; Schwartz, B S; Manchanda, N; Maunder, R J; Martin, T R; McLarty, J; Fair, D S

    1989-01-01

    To determine the possible mechanism(s) promoting alveolar fibrin deposition in the adult respiratory distress syndrome (ARDS), we investigated the initiation and regulation of both fibrinolysis and coagulation from patients with ARDS (n = 14), at risk for ARDS (n = 5), and with interstitial lung diseases (ILD) (n = 8), and normal healthy individuals (n = 13). Bronchoalveolar lavage (BAL) extrinsic pathway inhibitor activity was increased in ARDS BAL compared with patients at risk for ARDS (P = 0.0146) or normal controls (P = 0.0013) but tissue factor-factor VII procoagulant activity was significantly increased in ARDS BAL compared with all other groups (P less than 0.001). Fibrinolytic activity was not detectable in BAL of 10 of the 14 patients with ARDS and low levels of activity were found in BAL of the other four ARDS patients. Depressed fibrinolysis in ARDS BAL was not due to local insufficiency of plasminogen; rather, there was inhibition of both plasmin and plasminogen activator. Plasminogen activator inhibitor 1 was variably detected and low levels of plasminogen activator inhibitor 2 were found in two ARDS BAL samples, but plasminogen activator inhibitor 2 was otherwise undetectable. ARDS BAL antiplasmin activity was, in part, due to alpha 2-antiplasmin. We conclude that abnormalities that result in enhanced coagulation and depressed fibrinolysis, thereby predisposing to alveolar fibrin deposition, occur in the alveolar lining fluids from patients with ARDS. Images PMID:2788176

  15. Psychological distress of patients suffering from restless legs syndrome: a cross-sectional study

    PubMed Central

    2011-01-01

    Background Restless legs syndrome (RLS) is a chronic disorder with substantial impact on quality of life similar to that seen in diabetes mellitus or osteoarthritis. Little is known about the psychological characteristics of RLS patients although psychological factors may contribute to unfavourable treatment outcome. Methods In an observational cross-sectional design, we evaluated the psychological features of 166 consecutive RLS patients from three outpatient clinics, by means of the Symptom Checklist 90-R (SCL-90-R) questionnaire. Additionally, the Beck Depression Inventory-II (BDI-II) and the International RLS Severity Scale (IRLS) were measured. Both treated and untreated patients were included, all patients sought treatment. Results Untreated patients (n = 69) had elevated but normal scores on the SCL-90-R Global Severity Index (GSI; p = 0.002) and on the sub-scales somatisation (p < 0.001), compulsivity (p = 0.003), depression (p = 0.02), and anxiety (p = 0.004) compared with a German representative sample. In the treated group, particularly in those patients who were dissatisfied with their actual treatment (n = 62), psychological distress was higher than in the untreated group with elevated scores for the GSI (p = 0.03) and the sub-scales compulsivity (p = 0.006), depression (p = 0.012), anxiety (p = 0.031), hostility (p = 0.013), phobic anxiety (p = 0.024), and paranoid ideation (p = 0.012). Augmentation, the most serious side effect of dopaminergic, i.e. first-line treatment of RLS, and loss of efficacy were accompanied with the highest psychological distress, as seen particularly in the normative values of the sub-scales compulsivity and anxiety. Generally, higher RLS severity was correlated with higher psychological impairment (p < 0.001). Conclusion Severely affected RLS patients show psychological impairment in multiple psychological domains which has to be taken into account in the treatment regimen. PMID:21933380

  16. Different ventilation modes combined with ambroxol in the treatment of respiratory distress syndrome in premature infants

    PubMed Central

    Zhou, Bin; Zhai, Jing-Fang; Wu, Jie-Bin; Jin, Bao; Zhang, Yan-Yan

    2017-01-01

    The aim of the present study was to compare the effectiveness of different modes of mechanical ventilation in combination with secretolytic therapy with ambroxol in premature infants with respiratory distress syndrome. Seventy-three premature infants with hyaline membrane disease (HMD) (stage III–IV), also known as respiratory distress syndrome, who were supported by mechanical ventilation in the neonatal intensive care unit (NICU) of Xuzhou Central Hospital, were involved in the present study, between January 2013 and February 2015. Forty cases were randomly selected and treated with high frequency oscillatory ventilation (HFOV), forming the HFOV group, whereas 33 cases were selected and treated with conventional mechanical ventilation (CMV), forming the CMV group. Patients in the two groups were administered ambroxol intravenously at a dosage rate of 30 mg/kg body weight at the beginning of the study. The present study involved monitoring the blood gas index as well as changes in the respiratory function index in the two groups. Additionally, the incidence of complications in the premature infants in the two groups was observed prior to and following the ventilation. Pulmonary arterial oxygen tension (PaO2), the PaO2/fraction of inspired oxygen (FiO2) ratio, the oxygenation index [OI = 100 × mean airway pressure (MAP) × FiO2/PaO2], as well as the arterial/alveolar oxygen partial pressure ratio (a/APO2) = PaO2/(713 × FiO2 partial pressure of carbon dioxide (PaCO2)/0.8) of the patients in the HFOV group after 1, 12 and 24 h of treatment were significantly improved as compared to the patients of the CMV group. However, there was no significant difference between patients in the two groups with regard to the number of mortalities, complications such as pneumothorax, bronchopulmonary dysplasia (BPD), retinopathy of prematurity (ROP), intraventricular hemorrhage (IVH), periventricular leukomalacia (PVL), and the time of ventilation. In conclusion, combining HFOV

  17. Negative-pressure pulmonary edema complicated by acute respiratory distress syndrome in an orangutan (Pongo pygmaeus abelii).

    PubMed

    Kenny, David E; Knightly, Felicia; Haas, Bradley; Hergott, Lawrence; Kutinsky, Ilana; Eller, Jimmie L

    2003-12-01

    A 22-yr-old, 86-kg, morbidly obese female orangutan (Pongo pygmaeus abelii) was immobilized and transported to the Denver Zoological Gardens hospital for a routine physical examination. Immediately after arriving at the hospital, cyanosis and apparent inadequate ventilatory efforts were noted. Clinically significant hypoxia occurred despite attempts to ventilate the orangutan through face mask, and attempts to place an endotracheal tube began. A large volume of pink-tinged frothy fluid flowed from the trachea when the laryngoscope was inserted into the oropharynx. Severe pulmonary edema due to negative-pressure pulmonary edema, precipitating life-threatening hypoxia was suspected. The orangutan was maintained on a mechanical ventilator using the neuromuscular blocking agent cisatracurium besylate and sedation with periodic doses of isoflurane and midazolam for 48 hr. Positive end-expiratory pressure was used while the orangutan was ventilated mechanically to improve respiratory function. The edema and hypoxia improved, but respiratory arrest ensued 30 min after extubation, when the orangutan was removed from mechanical ventilation. Necropsy and histopathology demonstrated that serious lung injury had led to acute respiratory distress syndrome.

  18. Sepsis-related acute respiratory distress syndrome in children with cancer: the respiratory dynamics of a devastating condition

    PubMed Central

    Arduini, Rodrigo Genaro; de Araujo, Orlei Ribeiro; da Silva, Dafne Cardoso Bourguignon; Senerchia, Andreza Almeida; Petrilli, Antonio Sergio

    2016-01-01

    Objective To evaluate the clinical course and respiratory parameters of mechanically ventilated children with cancer suffering from sepsis-related acute respiratory distress syndrome. Methods This 2-year prospective, longitudinal, observational cohort study enrolled 29 children and adolescents. Clinical data, measurements of blood gases and ventilation parameters were collected at four different time points. Fluctuations between measurements as well as differences in estimated means were analyzed by linear mixed models in which death within 28 days from the onset of acute respiratory distress syndrome was the primary endpoint. Results There were 17 deaths within 28 days of acute respiratory distress syndrome onset and another 7 between 29 - 60 days. Only 5 patients survived for more than 60 days. Nine (31%) patients died as a direct consequence of refractory hypoxemia, and the others died of multiple organ failure and catecholamine-refractory shock. In 66% of the measurements, the tidal volume required to obtain oxygen saturation equal to or above 90% was greater than 7mL/kg. The estimated means of dynamic compliance were low and were similar for survivors and non-survivors but with a negative slope between the first and final measurements, accompanied by a negative slope of the tidal volume for non-survivors. Non-survivors were significantly more hypoxemic, with PaO2/FiO2 ratios showing lower estimated means and a negative slope along the four measurements. Peak, expiratory and mean airway pressures showed positive slopes in the non-survivors, who also had more metabolic acidosis. Conclusions In most of our children with cancer, sepsis and acute respiratory distress syndrome progressed with deteriorating ventilation indexes and escalating organic dysfunction, making this triad nearly fatal in children. PMID:28099641

  19. Interdisciplinary Peripartum Management of Acute Respiratory Distress Syndrome with Extracorporeal Membrane Oxygenation – a Case Report and Literature Review

    PubMed Central

    Weyrich, J.; Bogdanski, R.; Ortiz, J. U.; Kuschel, B.; Schneider, K. T. M.; Lobmaier, S. M.

    2016-01-01

    Extracorporeal membrane oxygenation (ECMO) is increasingly used for the management of acute severe cardiac and respiratory failure. One of the indications is acute respiratory distress syndrome (ARDS) for which, in some severe cases, ECMO represents the only possibility to save lives. We report on the successful long-term use of ECMO in a postpartum patient with recurrent pulmonary decompensation after peripartum uterine rupture with extensive blood loss. PMID:27065489

  20. Mechanical ventilation-associated lung fibrosis in acute respiratory distress syndrome: a significant contributor to poor outcome.

    PubMed

    Cabrera-Benitez, Nuria E; Laffey, John G; Parotto, Matteo; Spieth, Peter M; Villar, Jesús; Zhang, Haibo; Slutsky, Arthur S

    2014-07-01

    One of the most challenging problems in critical care medicine is the management of patients with the acute respiratory distress syndrome. Increasing evidence from experimental and clinical studies suggests that mechanical ventilation, which is necessary for life support in patients with acute respiratory distress syndrome, can cause lung fibrosis, which may significantly contribute to morbidity and mortality. The role of mechanical stress as an inciting factor for lung fibrosis versus its role in lung homeostasis and the restoration of normal pulmonary parenchymal architecture is poorly understood. In this review, the authors explore recent advances in the field of pulmonary fibrosis in the context of acute respiratory distress syndrome, concentrating on its relevance to the practice of mechanical ventilation, as commonly applied by anesthetists and intensivists. The authors focus the discussion on the thesis that mechanical ventilation-or more specifically, that ventilator-induced lung injury-may be a major contributor to lung fibrosis. The authors critically appraise possible mechanisms underlying the mechanical stress-induced lung fibrosis and highlight potential therapeutic strategies to mitigate this fibrosis.

  1. Clinical and pathological features of fat embolism with acute respiratory distress syndrome.

    PubMed

    Kao, Shang Jyh; Yeh, Diana Yu-Wung; Chen, Hsing I

    2007-09-01

    FES (fat embolism syndrome) is a clinical problem, and, although ARDS (acute respiratory distress syndrome) has been considered as a serious complication of FES, the pathogenesis of ARDS associated with FES remains unclear. In the present study, we investigated the clinical manifestations, and biochemical and pathophysiological changes, in subjects associated with FES and ARDS, to elucidate the possible mechanisms involved in this disorder. A total of eight patients with FES were studied, and arterial blood pH, PaO(2) (arterial partial pressure of O(2)), PaCO(2) (arterial partial pressure of CO(2)), biochemical and pathophysiological data were obtained. These subjects suffered from crash injuries and developed FES associated with ARDS, and each died within 2 h after admission. In the subjects, chest radiography revealed that the lungs were clear on admission, and pulmonary infiltration was observed within 2 h of admission. Arterial blood pH and PaO(2) declined, whereas PaCO(2) increased. Plasma PLA(2) (phospholipase A(2)), nitrate/nitrite, methylguanidine, TNF-alpha (tumour necrosis factor-alpha), IL-1beta (interleukin-1beta) and IL-10 (interleukin-10) were significantly elevated. Pathological examinations revealed alveolar oedema and haemorrhage with multiple fat droplet depositions and fibrin thrombi. Fat droplets were also found in the arterioles and/or capillaries in the lung, kidney and brain. Immunohistochemical staining identified iNOS (inducible nitric oxide synthase) in alveolar macrophages. In conclusion, our clinical analysis suggests that PLA(2), NO, free radicals and pro-inflammatory cytokines are involved in the pathogenesis of ARDS associated with FES. The major source of NO is the alveolar macrophages.

  2. Inhaled nitric oxide in adult patients with acute respiratory distress syndrome.

    PubMed

    Monsalve-Naharro, José Ángel; Domingo-Chiva, Esther; García Castillo, Sergio; Cuesta-Montero, Pablo; Jiménez-Vizuete, José María

    2017-03-01

    In some patients, acute respiratory distress syndrome (ARDS) leads to life-threatening refractory hypoxemia developing. Physicians may consider hypoxemic rescue therapies in an attempt to improve oxygenation in these patients while on conventional mechanical ventilation support. Use of inhaled nitric oxide (iNO) in ARDS is one of the most widely-studied pharmacological interventions over the past two decades. Its efficacy was examined in several randomized clinical trials and has undergone meta-analyses. Although iNO treatment was associated with improved oxygenation, researchers unfortunately never demonstrated a concomitant decrease in mortality or any improved outcome. Hence the current evidence suggests that iNO should not be routinely used in patients with ARDS however may be considered as adjunct therapy to tentatively improve oxygenation while other therapies are being considered in patients with severely hypoxemic ARDS.This review focuses on the therapeutic use of iNO in adult ARDS patients. We set out some recommendations for its use as rescue therapy against refractory hypoxemia.

  3. Enrichment of the Lung Microbiome with Gut Bacteria in Sepsis and the Acute Respiratory Distress Syndrome

    PubMed Central

    Dickson, Robert P.; Singer, Benjamin H.; Newstead, Michael W.; Falkowski, Nicole R.; Erb-Downward, John R.; Standiford, Theodore J.; Huffnagle, Gary B.

    2016-01-01

    SUMMARY Sepsis and the acute respiratory distress syndrome (ARDS) are major causes of mortality without targeted therapies. Although many experimental and clinical observations have implicated gut microbiota in the pathogenesis of these diseases, culture-based studies have failed to demonstrate translocation of bacteria to the lungs in critically ill patients. Here we report culture-independent evidence that the lung microbiome is enriched with gut bacteria both in a murine model of sepsis and in humans with established ARDS. Following experimental sepsis, lung communities were dominated by viable gut-associated bacteria. Ecologic analysis identified the lower gastrointestinal tract, rather than the upper respiratory tract, as the likely source community of post-sepsis lung bacteria. In bronchoalveolar lavage fluid from humans with ARDS, gut-specific bacteria (Bacteroides spp.) were common and abundant, undetected by culture, and correlated with the intensity of systemic inflammation. Alveolar TNF-α, a key mediator of alveolar inflammation in ARDS, was significantly correlated with altered lung microbiota. Our results demonstrate that the lung microbiome is enriched with gut-associated bacteria in sepsis and ARDS, potentially representing a shared mechanism of pathogenesis in these common and lethal diseases. PMID:27670109

  4. Plasminogen activator inhibitor I 4G/5G polymorphism in neonatal respiratory distress syndrome.

    PubMed

    Armangil, Didem; Yurdakök, Murat; Okur, Hamza; Gürgey, Aytemiz

    2011-08-01

    Fibrin monomers inhibit surfactant function. 4G/5G insertion/deletion polymorphism plays an important role in the regulation of plasminogen activator inhibitor 1 (PAI-1) gene expression. To examine the genotype distribution of PAI-1 polymorphism in 60 infants with respiratory distress syndrome (RDS) and 53 controls, an allele-specific polymerase chain reaction (PCR) was used. The proportion of 4G/4G, 4G/5G, and 5G/5G genotypes did not differ statistically between the RDS and control groups (P > .05). Having PAI-1 4G/4G genotype polymorphism appears to increase the risk of RDS (odds ratio [OR] =1.5; 95% confidence interval [CI], 0.5-4.3), although it was not statistically significant. No relation was found between the PAI-1 4G/5G polymorphisms and RDS, but there was an increased risk associated with the 4G variant of the PAI-1 gene. We believe that our findings of increased 4G allele of the PAI-1 gene in infants with RDS would also help to clarify the pathogenesis of RDS.

  5. [A case of miliary tuberculosis presenting with acute resiratory distress syndrome].

    PubMed

    Nojima, Daisuke; Ozaki, Shinji; Fujii, Yasuhiro; Wada, Sae; Ono, Katsuichiro; Fujimoto, Nobukazu; Gemba, Kenichi; Kishimoto, Takumi

    2009-03-01

    An 87-year-old woman was admitted because of high fever, progressive dyspnea and abnormal shadows on chest roentgenogram. Laboratory investigation on admission demonstrated a normal white cell count with neutrophilia (4000/microl, 90.5% neutrophils), an erythrocyte sedimentation rate of 10 mm/h and C-reactive protein value of 9.0mg/dl. Roentgenogram and computed tomographic scan of the chest shows bilateral infiltration and diffuse ground glass opacity, indicating acute respiratory distress syndrome (ARDS), but disseminated nodules, indicating miliary tuberculosis, were not found. Blood gas analysis demonstrated severe hypoxemia (PaO2 43.2Torr with 6L/ min oxygen). Based on the diagnosis of acute pneumonia and ARDS, intravenous administration of sivelestat sodium hydrate (100 mg/day), and continuous infusion of hydrocortisone (200 mg/day) were started. PaO2/FiO2 ratio improved but X-ray findings showed no improvement and a blood test revealed pancytopenia. Bone marrow biopsy revealed necrotizing epithelioid granuloma and acid-fast bacilli. A polymerase chain reaction (PCR) test detected Mycobacterium tuberculosis. Mycobacterium tuberculosis was also detected in sputum and urine. Therefore, we diagnosed miliary tuberculosis and transferred the patient to an infectious disease hospital. Miliary tuberculosis complicated with ARDS is relatively rare and the prognosis is extremely poor. Miliary tuberculosis should be kept in mind as a cause of ARDS.

  6. Enterobacter cloacae Sacroiliitis with Acute Respiratory Distress Syndrome in an Adolescent.

    PubMed

    Kim, Jin Soo; Ko, Jeong Hee; Lee, Seunghun; Jeon, Seok Chol; Oh, Sung Hee

    2015-06-01

    Enterobacter cloacae has emerged as an important nosocomial pathogen, but is rarely a cause of sacroiliitis. Herein, we present the first reported case of Enterobacter cloacae sacroiliitis associated with sepsis and acute respiratory distress syndrome (ARDS). A previously healthy 14-year-old boy presented with low-grade fever and pain in the left side of the hip that was aggravated by walking. Pelvic computed tomography (CT) showed normal findings, and the patient received supportive care for transient synovitis with no antibiotics. However, there was no clinical improvement. On the third day of hospitalization, magnetic resonance imaging of the hip revealed findings compatible with sacroiliitis, for which vancomycin and ceftriaxone were administered. The patient suddenly developed high fever with dyspnea. Chest radiography and CT findings and a PaO2/FiO2 ratio <200 mmHg were suggestive of ARDS; the patient subsequently received ventilatory support and low-dose methylprednisolone infusions. Within one week, defervescence occurred, and the patient was able to breathe on his own. Following the timely recognition of, and therapeutic challenge to, ARDS, and after 6 weeks of parenteral antimicrobial therapy, the patient was discharged in good health with no complications.

  7. Enterobacter cloacae Sacroiliitis with Acute Respiratory Distress Syndrome in an Adolescent

    PubMed Central

    Kim, Jin Soo; Ko, Jeong Hee; Lee, Seunghun; Jeon, Seok Chol

    2015-01-01

    Enterobacter cloacae has emerged as an important nosocomial pathogen, but is rarely a cause of sacroiliitis. Herein, we present the first reported case of Enterobacter cloacae sacroiliitis associated with sepsis and acute respiratory distress syndrome (ARDS). A previously healthy 14-year-old boy presented with low-grade fever and pain in the left side of the hip that was aggravated by walking. Pelvic computed tomography (CT) showed normal findings, and the patient received supportive care for transient synovitis with no antibiotics. However, there was no clinical improvement. On the third day of hospitalization, magnetic resonance imaging of the hip revealed findings compatible with sacroiliitis, for which vancomycin and ceftriaxone were administered. The patient suddenly developed high fever with dyspnea. Chest radiography and CT findings and a PaO2/FiO2 ratio <200 mmHg were suggestive of ARDS; the patient subsequently received ventilatory support and low-dose methylprednisolone infusions. Within one week, defervescence occurred, and the patient was able to breathe on his own. Following the timely recognition of, and therapeutic challenge to, ARDS, and after 6 weeks of parenteral antimicrobial therapy, the patient was discharged in good health with no complications. PMID:26157593

  8. Cost implications of different approaches to the prevention of respiratory distress syndrome.

    PubMed Central

    Mugford, M; Piercy, J; Chalmers, I

    1991-01-01

    Because the incidence of both neonatal respiratory distress syndrome and neonatal mortality can be reduced by giving corticosteroids to women expected to deliver preterm and by giving surfactant to babies at high risk of developing hyaline membrane disease, we have considered what effects the adoption of one or both of these preventive policies would have on the costs of neonatal care. We have estimated the effects of treatment from overviews of the relevant controlled trials, and estimated costs from observations of care at one neonatal unit. Our results suggest that if either of these policies is adopted for all babies under 35 weeks' gestation at a drug cost of 150 pounds or less/baby, the overall costs of care would be reduced by between 1 and 10%. The cost per survivor would be reduced by up to 16% even if the drug cost were to be as high as pounds 550/baby. If the policies were to be adopted only for babies under 31 weeks' gestation, both policies would result in a reduction in cost of between 5 and 16%/survivor, although the increased survival resulting from the policies would lead to an increase in overall costs for babies of less then 31 weeks' gestation of between 7 and 32%. PMID:1863120

  9. Exhaled breath metabolomics as a noninvasive diagnostic tool for acute respiratory distress syndrome.

    PubMed

    Bos, Lieuwe D J; Weda, Hans; Wang, Yuanyue; Knobel, Hugo H; Nijsen, Tamara M E; Vink, Teunis J; Zwinderman, Aeilko H; Sterk, Peter J; Schultz, Marcus J

    2014-07-01

    There is a need for biological markers of the acute respiratory distress syndrome (ARDS). Exhaled breath contains hundreds of metabolites in the gas phase, some of which reflect (patho)physiological processes. We aimed to determine the diagnostic accuracy of metabolites in exhaled breath as biomarkers of ARDS. Breath from ventilated intensive care unit patients (n=101) was analysed using gas chromatography and mass spectrometry during the first day of admission. ARDS was defined by the Berlin definition. Training and temporal validation cohorts were used. 23 patients in the training cohort (n=53) had ARDS. Three breath metabolites, octane, acetaldehyde and 3-methylheptane, could discriminate between ARDS and controls with an area under the receiver operating characteristic curve (AUC) of 0.80. Temporal external validation (19 ARDS cases in a cohort of 48) resulted in an AUC of 0.78. Discrimination was insensitive to adjustment for severity of disease, a direct or indirect cause of ARDS, comorbidities, or ventilator settings. Combination with the lung injury prediction score increased the AUC to 0.91 and improved net reclassification by 1.17. Exhaled breath analysis showed good diagnostic accuracy for ARDS, which was externally validated. These data suggest that exhaled breath analysis could be used for the diagnostic assessment of ARDS.

  10. Acute Respiratory Distress Syndrome after the Use of Gadolinium Contrast Media.

    PubMed

    Park, Jihye; Byun, Il Hwan; Park, Kyung Hee; Lee, Jae-Hyun; Nam, Eun Ji; Park, Jung-Won

    2015-07-01

    Acute respiratory distress syndrome (ARDS) is a medical emergency that threatens life. To this day, ARDS is very rarely reported by iodine contrast media, and there is no reported case of ARDS induced by gadolinium contrast media. Here, we present a case with ARDS after the use of gadobutrol (Gadovist) as a magnetic resonance imaging (MRI) contrast medium. A 26 years old female without any medical history, including allergic diseases and without current use of drugs, visited the emergency room for abdominal pain. Her abdominopelvic computed tomography with iodine contrast media showed a right ovarian cyst and possible infective colitis. Eighty-three hours later, she underwent pelvis MRI after injection of 7.5 mL (0.1 mL/kg body weight) of gadobutrol (Gadovist) to evaluate the ovarian cyst. She soon presented respiratory difficulty, edema of the lips, nausea, and vomiting, and we could hear wheezing upon auscultation. She was treated with dexamethasone, epinephrine, and norepinephrine. Her chest X-ray showed bilateral central bat-wing consolidative appearance. Managed with mechanical ventilation, she was extubated 3 days later and discharged without complications.

  11. The Current Status of the Respiratory Distress Syndrome of the Newly Born

    PubMed Central

    Swyer, P. R.; Levison, H.

    1965-01-01

    The respiratory distress syndrome (RDS) occurs in 14% of premature infants and is twice as common and twice as lethal in males as in females. Recent work suggests that, during the intrauterine period a disturbance in nutrition of the lung resulting from fetal pulmonary vascular constriction results in alveolar-cell damage and a decrease in pulmonary surface activity with resultant atelectasis. Data on respiratory work levels, oxygen consumption and arterial oxygen tension suggest that there is an oxygen debt in the acute stage of the disease. Such data have further clarified the pathogenesis of the metabolic and respiratory components of the acidosis and the secondary effects thereof. In prevention, prophylaxis of prematurity is of major importance. A program of treatment designed to combat the various aspects of the pathophysiological disturbances is described in the form of a case profile. Modern methods of observation, biochemical control and treatment, as well as the necessity for critical evaluation, suggest that infants with RDS are best cared for in special centres. ImagesFig. 11 PMID:14339292

  12. [A case of Legionella pneumophila pneumonia accompanied by acute respiratory distress syndrome and epilepsy].

    PubMed

    Saito, Nayuta; Shimizu, Kenichiro; Yoshii, Yutaka; Kojima, Jun; Ishikawa, Takeo; Saito, Keisuke; Kuwano, Kazuyoshi

    2013-05-01

    A 32-year-old female with epilepsy presented at our hospital with high-grade fever, seizures, and unconsciousness. She was initially treated for aspiration pneumonia with ampicillin/sulbactam. Despite antibiotic therapy, her chest X-ray findings dramatically worsened, showing extension to the bilateral lung field. Her PaO2/FiO2 ratio decreased to 70.6. Rapid progression of hypoxia, unconsciousness, and hyponatremia led to the suspicion of Legionella pneumonia; however, it was difficult to make a definitive diagnosis because she had denied using a whirlpool spa and the initial urinary Legionella antigen test results were negative. Therefore, we repeated the Legionella urinary antigen test, which was positive. On the basis of these results, sputum polymerase chain reaction findings, and the four-fold elevation of paired antibodies, the patient was diagnosed as having Legionella pneumonia accompanied by acute respiratory distress syndrome. We considered administering fluoroquinolone antibiotics, that are recommended for severe Legionella pneumonia, although quinolones have a potential risk for causing convulsions. In this case, we carefully administered ciprofloxacin. The patient recovered consciousness after treatment without any relapse of epileptic seizures. We also administered a corticosteroid for severe pneumonia with the expectation of clinical improvement and to avoid intubation. We emphasize the importance of aggressive workup and empirical therapy for patients with Legionella pneumonia with rapidly worsening symptoms and clinical features such as unconsciousness, epilepsy, and hyponatremia and in whom fluoroquinolone and corticosteroid therapy are effective despite the presence of epilepsy.

  13. Pathophysiological Approaches of Acute Respiratory Distress syndrome: Novel Bases for Study of Lung Injury

    PubMed Central

    Castillo, R.L; Carrasco Loza, R; Romero-Dapueto, C

    2015-01-01

    Experimental approaches have been implemented to research the lung damage related-mechanism. These models show in animals pathophysiological events for acute respiratory distress syndrome (ARDS), such as neutrophil activation, reactive oxygen species burst, pulmonary vascular hypertension, exudative edema, and other events associated with organ dysfunction. Moreover, these approaches have not reproduced the clinical features of lung damage. Lung inflammation is a relevant event in the develop of ARDS as component of the host immune response to various stimuli, such as cytokines, antigens and endotoxins. In patients surviving at the local inflammatory states, transition from injury to resolution is an active mechanism regulated by the immuno-inflammatory signaling pathways. Indeed, inflammatory process is regulated by the dynamics of cell populations that migrate to the lung, such as neutrophils and on the other hand, the role of the modulation of transcription factors and reactive oxygen species (ROS) sources, such as nuclear factor kappaB and NADPH oxidase. These experimental animal models reproduce key components of the injury and resolution phases of human ALI/ARDS and provide a methodology to explore mechanisms and potential new therapies. PMID:26312099

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

    PubMed Central

    Lee, Kyung-Yil

    2017-01-01

    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. PMID:28208675

  15. Changes in intestinal microflora in rats with acute respiratory distress syndrome

    PubMed Central

    Li, Yan; Liu, Xiang-Yong; Ma, Ming-Ming; Qi, Zhi-Jiang; Zhang, Xiao-Qiang; Li, Zhi; Cao, Guo-Hong; Li, Jun; Zhu, Wei-Wei; Wang, Xiao-Zhi

    2014-01-01

    AIM: To implement high-throughput 16S rDNA sequencing to study microbial diversity in the fecal matter of rats with acute lung injury/acute respiratory distress syndrome (ALI/ARDS). METHODS: Intratracheal instillation of lipopolysaccharide was used to induce ALI, and the pathological changes in the lungs and intestines were observed. D-lactate levels and diamine oxidase (DAO) activities were determined by enzymatic spectrophotometry. The fragments encompassing V4 16S rDNA hypervariable regions were PCR amplified from fecal samples, and the PCR products of V4 were sequenced by Illumina MiSeq. RESULTS: Increased D-lactate levels and DAO activities were observed in the model group (P < 0.01). Sequencing results revealed the presence of 3780 and 4142 species in the control and model groups, respectively. The percentage of shared species was 18.8419%. Compared with the control group, the model group had a higher diversity index and a lower number of species of Fusobacteria (at the phylum level), Helicobacter and Roseburia (at the genus level) (P < 0.01). Differences in species diversity, structure, distribution and composition were found between the control group and early ARDS group. CONCLUSION: The detection of specific bacteria allows early detection and diagnosis of ALI/ARDS. PMID:24914345

  16. Aspirin as a potential treatment in sepsis or acute respiratory distress syndrome.

    PubMed

    Toner, Philip; McAuley, Danny Francis; Shyamsundar, Murali

    2015-10-23

    Sepsis is a common condition that is associated with significant morbidity, mortality and health-care cost. Pulmonary and non-pulmonary sepsis are common causes of the acute respiratory distress syndrome (ARDS). The mortality from ARDS remains high despite protective lung ventilation, and currently there are no specific pharmacotherapies to treat sepsis or ARDS. Sepsis and ARDS are characterised by activation of the inflammatory cascade. Although there is much focus on the study of the dysregulated inflammation and its suppression, the associated activation of the haemostatic system has been largely ignored until recently. There has been extensive interest in the role that platelet activation can have in the inflammatory response through induction, aggregation and activation of leucocytes and other platelets. Aspirin can modulate multiple pathogenic mechanisms implicated in the development of multiple organ dysfunction in sepsis and ARDS. This review will discuss the role of the platelet, the mechanisms of action of aspirin in sepsis and ARDS, and aspirin as a potential therapy in treating sepsis and ARDS.

  17. Acute respiratory distress syndrome following cardiovascular surgery: current concepts and novel therapeutic approaches

    PubMed Central

    Hoegl, Sandra; Zwissler, Bernhard; Eltzschig, Holger K.; Vohwinkel, Christine

    2015-01-01

    Purpose of review This review gives an update on current treatment options and novel concepts on the prevention and treatment of the acute respiratory distress syndrome (ARDS) in cardiovascular surgery patients. Recent findings The only proven beneficial therapeutic options in ARDS are those that help to prevent further ventilator-induced lung injury, such as prone position, use of lung-protective ventilation strategies, and extracorporeal membrane oxygenation. In the future also new approaches like mesenchymal cell therapy, activation of hypoxia-elicited transcription factors or targeting of purinergic signaling may be successful outside the experimental setting. Owing to the so far limited treatment options, it is of great importance to determine patients at risk for developing ARDS already perioperatively. In this context, serum biomarkers and lung injury prediction scores could be useful. Summary Preventing ARDS as a severe complication in the cardiovascular surgery setting may help to reduce morbidity and mortality. As cardiovascular surgery patients are of greater risk to develop ARDS, preventive interventions should be implemented early on. Especially, use of low tidal volumes, avoiding of fluid overload and restrictive blood transfusion regimes may help to prevent ARDS. PMID:26598954

  18. Continuous blood purification treatment for endotoxin-induced acute respiratory distress syndrome

    PubMed Central

    Jiang, Y.; Lin, R.; Xu, Y.; Zhang, S.; Cui, K.; Zhu, M.; Li, A.; Chen, C.; Yang, J.; Yang, W.

    2017-01-01

    This study aimed to explore the effects of continuous blood purification (CBP) treatment in pigs affected with acute respiratory distress syndrome (ARDS). A total of 12 healthy male pigs, weighing 12±1.8 kg, were randomly and equally assigned to the control and experimental groups. The ARDS pig model was prepared by intravenous injections of endotoxin (20 µg/kg). The control group was given conventional supportive therapy, while the experimental group was given continuous veno-venous hemofiltration therapy. During the treatment process, the variations in dynamic lung compliance, oxygenation index, hemodynamics, and urine volume per hour at different times (Baseline, 0, 2, 4, and 6 h) were recorded. The levels of tumor necrosis factor (TNF-α), interleukin 6 (IL-6), and IL-10 in serum and bronchoalveolar lavage fluid (BALF) were measured using the enzyme-linked immunosorbent assay. The histomorphological changes of the lung, heart, and kidney were visualized using a light microscope. The nuclear factor κB p65 protein content of the heart, lung, and kidney tissues was also detected using western blot. The experimental group outperformed the control group in both respiratory and hemodynamic events. CBP treatment cleared TNF-α, IL-6, and IL-10 partially from serum and BALF. The pathological examination of the heart, lung, and kidney tissues revealed that the injury was less severe in the experimental group. CBP treatment can improve the organ functions of pigs affected with endotoxin-induced ARDS and protect these organs to some extent. PMID:28225865

  19. Heterochromia iridis and Horner's syndrome due to paravertebral neurilemmoma.

    PubMed

    Sayed, A K; Miller, B A; Lack, E E; Sallan, S E; Levey, R H

    1983-01-01

    A case of heterochromia iridis and Horner's syndrome is reported in a 7-year old girl with paravertebral neurilemmoma. These clinical findings can be useful in the early diagnosis of mediastinal tumors in the paravertebral axis. While typically associated with neuroblastoma, these findings can be due to tumors which are inately benign--in this case neurilemmoma. The mechanism for heterochromia is briefly discussed.

  20. Hybrid management of Paget-Schroetter syndrome due to thoracic outlet syndrome.

    PubMed

    De Caridi, Giovanni; Massara, Mafalda; Greco, Michele; Villari, Simona; Squillaci, Domenico; Spinelli, Francesco

    2016-02-01

    Paget-Schroetter syndrome due to thoracic outlet syndrome is a rare but potentially disabling condition that generally affects young patients otherwise healthy. The prompt diagnosis and treatment of Paget-Schroetter syndrome is necessary to avoid major morbidity and long-term disability. The more modern treatment paradigm reported in the current literature consists of hybrid procedures with surgical decompression of the thoracic outlet and endovascular techniques to potentially improve long-term vein patency. However, there seems to be no consensus in the literature with regard to the timing and precise nature of active management, and there is presently no agreed protocol for the optimum management of Paget-Schroetter syndrome. Controversy exists partly because no randomised controlled studies are present in literature. We present a case of Paget-Schroetter syndrome due to thoracic outlet syndrome in a young male patient submitted to a multimodal procedure.

  1. Effects of Pharmacologic Intervention on Oxygenation, Lung Water and Protein Leak in the Pseudomonas ARDS Porcine Model. Subtitle: Effects of Pharmacologic and Immunologic Intervention on the Porcine Pseudomonas Model of Adult Respiratory Distress Syndrome (ARDS).

    DTIC Science & Technology

    1992-10-01

    Respiratory Distress Syndrome ( ARDS ) PRINCIPAL INVESTIGATOR: Harvey J. Sugerman, M.D. AUTHORS: Patrick G. Mullen, M.B., F.R.C.S.I., Alastair C.J...Pseudomonas Model of Adult Respiratory Distress Syndrome ( ARDS ) Approved for public release; distribution unlimited 92-29868 Almost twenty-five years after...explosive form of lung injury in critically ill patients, which they termed adult respiratory distress syndrome ( ARDS ), the mortality and

  2. Open lung biopsy in early-stage acute respiratory distress syndrome

    PubMed Central

    Kao, Kuo-Chin; Tsai, Ying-Huang; Wu, Yao-Kuang; Chen, Ning-Hung; Hsieh, Meng-Jer; Huang, Shiu-Feng; Huang, Chung-Chi

    2006-01-01

    Introduction Acute respiratory distress syndrome (ARDS) has heterogeneous etiologies, rapid progressive change and a high mortality rate. To improve the outcome of ARDS, accurate diagnosis is essential to the application of effective early treatment. The present study investigated the clinical effects and safety of open lung biopsy (OLB) in patients with early-stage ARDS of suspected non-infectious origin. Methods We undertook a retrospective study of 41 patients with early-stage ARDS (defined as one week or less after intubation) who underwent OLB in two medical intensive care units of a tertiary care hospital from 1999 to 2005. Data analyzed included baseline characteristics, complication rate, pathological diagnoses, treatment alterations, and hospital survival. Results The age of patients was 55 ± 17 years (mean ± SD). The average ratio of arterial partial pressure of oxygen (PaO2) to fraction of inspired oxygen (FiO2) was 116 ± 43 mmHg (mean ± SD) at biopsy. Seventeen patients (41%) were immunocompromised. Postoperative complications occurred in 20% of patients (8/41). All biopsies provided a pathological diagnosis with a diagnostic yield of 100%. Specific pathological diagnoses were made for 44% of patients (18/41). Biopsy findings led to an alteration of treatment modality in 73% of patients (30/41). The treatment alteration rate was higher in patients with nonspecific diagnoses than in patients with specific diagnoses (p = 0.0024). Overall mortality was 50% (21/41) and was not influenced by age, gender, pre-OLB oxygenation, complication rate, pathological results, and alteration of treatment. There was no surgery-related mortality. The survival rate for immunocompromised patients was better than that for immunocompetent patients (71% versus 33%; p = 0.0187) in this study. Conclusion Our retrospective study suggests that OLB was a useful and acceptably safe diagnostic procedure in some selected patients with early-stage ARDS. PMID:16859510

  3. Lung ventilation strategies for acute respiratory distress syndrome: a systematic review and network meta-analysis.

    PubMed

    Wang, Changsong; Wang, Xiaoyang; Chi, Chunjie; Guo, Libo; Guo, Lei; Zhao, Nana; Wang, Weiwei; Pi, Xin; Sun, Bo; Lian, Ailing; Shi, Jinghui; Li, Enyou

    2016-03-09

    To identify the best lung ventilation strategy for acute respiratory distress syndrome (ARDS), we performed a network meta-analysis. The Cochrane Central Register of Controlled Trials, EMBASE, MEDLINE, CINAHL, and the Web of Science were searched, and 36 eligible articles were included. Compared with higher tidal volumes with FiO2-guided lower positive end-expiratory pressure [PEEP], the hazard ratios (HRs) for mortality were 0.624 (95% confidence interval (CI) 0.419-0.98) for lower tidal volumes with FiO2-guided lower PEEP and prone positioning and 0.572 (0.34-0.968) for pressure-controlled ventilation with FiO2-guided lower PEEP. Lower tidal volumes with FiO2-guided higher PEEP and prone positioning had the greatest potential to reduce mortality, and the possibility of receiving the first ranking was 61.6%. Permissive hypercapnia, recruitment maneuver, and low airway pressures were most likely to be the worst in terms of all-cause mortality. Compared with higher tidal volumes with FiO2-guided lower PEEP, pressure-controlled ventilation with FiO2-guided lower PEEP and lower tidal volumes with FiO2-guided lower PEEP and prone positioning ventilation are associated with lower mortality in ARDS patients. Lower tidal volumes with FiO2-guided higher PEEP and prone positioning ventilation and lower tidal volumes with pressure-volume (P-V) static curve-guided individual PEEP are potential optimal strategies for ARDS patients.

  4. [Antenatal corticosteroids in respiratory distress syndrome prevention: efficacy in relation to treatment--delivery interval].

    PubMed

    Heljić, Suada; Maksić, Hajrija; Misanović, Verica; Dizdarević, Jadranka

    2009-01-01

    Antenatal corticosteroids given to women, who are 24 to 34 weeks pregnant and may deliver within the next 24 hours to 7 days, are associated with significant reduction in rates of respiratory distress syndrome, intraventricular hemorrhage and mortality of pre-term babies. The aim of this study is assessment of antenatal corticosteroid effectiveness in reduction of RDS incidence in optimal delivery-treatment interval, in comparison to babies delivered before and after the optimal treatment interval has elapsed. This investigation included 80 pre-term babies between 26 and 34 gestational weeks whose mothers received corticosteroids before delivery. Control group consisted of 92 children of the same gestational age, whose mothers did not received corticosteroids antenatally. Babies of diabetic mothers, babies with IUGR and babies with congenital abnormalities were excluded. RDS was significantly less frequent in babies antenatally treated by corticosteroids (x2 31,473 p < 0.0001 coefficient contingency 0.366) then in babies whose mothers did not received corticosteroids before delivery. The majority of babies, 54.67% (p < 0.01) were born in optimal interval, 24 hours to 7 days from the beginning of the treatment, 32.0% (24/75) children were born within 24 hours and 13.3% (10/75) were born more then 7 days after the start of treatment. Comparing the incidence of RDS between groups of children born in optimal treatment-delivery interval (1 -7 days) and in the group of children born within 24 hours or after 7 days from the beginning of the treatment, no significant difference was found. The effect was clinically comparable, which suggests the possibility of reduction treatment-delivery interval in acute clinical conditions.

  5. Electrical Impedance Tomography: a new study method for neonatal Respiratory Distress Syndrome?

    PubMed

    Chatziioannidis, I; Samaras, T; Nikolaidis, N

    2011-07-01

    Treatment of cardiorespiratory system diseases is a procedure that usually demands data collection on terms of the anatomy and the operation of the organs that are under study. Electrical Impedance Tomography (EIT) is an alternative approach, in comparison to existing techniques. With EIT electrodes are placed in the perimeter of the human body and images of the estimated organ are reconstructed, using the measurement of its impendence (or resistance) distribution and determining its alteration through time, while at the same time the patient is not exposed to ionizing radiation. Its clinical use presupposes the correct placement of the electrodes over the perimeter of the human body, the rapid data collection and electrical safety. It is a low cost technique and it is implemented near the patient. It is able to determine the distribution of ventilation, blood supply, diffused or localized lung defects, but it can also estimate therapeutic interventions or alteration to assisted ventilation of the neonate. EIT was developed at the beginning of the 1980s, but it has only recently begun to be implemented on neonates, and especially in the study of their respiratory system function. The low rate of image analysis is considered to be a drawback, but it is offset by the potential offered for the estimation of lungs' function (both under normal and pathological conditions), since ventilation and resistance are two quite similar concepts. In this review the most important studies about EIT are mentioned as a method of estimating respiratory distress syndrome in neonates. In terms of the above mentioned development, it is supposed that this technique will offer a great amount of help to the doctor in his / her estimations of the cardiorespiratory system and to his / her selection of the best intervening strategies.

  6. A novel swine model of ricin-induced acute respiratory distress syndrome

    PubMed Central

    Katalan, Shahaf; Falach, Reut; Rosner, Amir; Goldvaser, Michael; Brosh-Nissimov, Tal; Dvir, Ayana; Mizrachi, Avi; Goren, Orr; Cohen, Barak; Gal, Yoav; Sapoznikov, Anita; Ehrlich, Sharon; Kronman, Chanoch

    2017-01-01

    ABSTRACT Pulmonary exposure to the plant toxin ricin leads to respiratory insufficiency and death. To date, in-depth study of acute respiratory distress syndrome (ARDS) following pulmonary exposure to toxins is hampered by the lack of an appropriate animal model. To this end, we established the pig as a large animal model for the comprehensive study of the multifarious clinical manifestations of pulmonary ricinosis. Here, we report for the first time, the monitoring of barometric whole body plethysmography for pulmonary function tests in non-anesthetized ricin-treated pigs. Up to 30 h post-exposure, as a result of progressing hypoxemia and to prevent carbon dioxide retention, animals exhibited a compensatory response of elevation in minute volume, attributed mainly to a large elevation in respiratory rate with minimal response in tidal volume. This response was followed by decompensation, manifested by a decrease in minute volume and severe hypoxemia, refractory to oxygen treatment. Radiological evaluation revealed evidence of early diffuse bilateral pulmonary infiltrates while hemodynamic parameters remained unchanged, excluding cardiac failure as an explanation for respiratory insufficiency. Ricin-intoxicated pigs suffered from increased lung permeability accompanied by cytokine storming. Histological studies revealed lung tissue insults that accumulated over time and led to diffuse alveolar damage. Charting the decline in PaO2/FiO2 ratio in a mechanically ventilated pig confirmed that ricin-induced respiratory damage complies with the accepted diagnostic criteria for ARDS. The establishment of this animal model of pulmonary ricinosis should help in the pursuit of efficient medical countermeasures specifically tailored to deal with the respiratory deficiencies stemming from ricin-induced ARDS. PMID:28067630

  7. Intravenous vitamin C as adjunctive therapy for enterovirus/rhinovirus induced acute respiratory distress syndrome.

    PubMed

    Fowler Iii, Alpha A; Kim, Christin; Lepler, Lawrence; Malhotra, Rajiv; Debesa, Orlando; Natarajan, Ramesh; Fisher, Bernard J; Syed, Aamer; DeWilde, Christine; Priday, Anna; Kasirajan, Vigneshwar

    2017-02-04

    We report a case of virus-induced acute respiratory distress syndrome (ARDS) treated with parenteral vitamin C in a patient testing positive for enterovirus/rhinovirus on viral screening. This report outlines the first use of high dose intravenous vitamin C as an interventional therapy for ARDS, resulting from enterovirus/rhinovirus respiratory infection. From very significant preclinical research performed at Virginia Commonwealth University with vitamin C and with the very positive results of a previously performed phase I safety trial infusing high dose vitamin C intravenously into patients with severe sepsis, we reasoned that infusing identical dosing to a patient with ARDS from viral infection would be therapeutic. We report here the case of a 20-year-old, previously healthy, female who contracted respiratory enterovirus/rhinovirus infection that led to acute lung injury and rapidly to ARDS. She contracted the infection in central Italy while on an 8-d spring break from college. During a return flight to the United States, she developed increasing dyspnea and hypoxemia that rapidly developed into acute lung injury that led to ARDS. When support with mechanical ventilation failed, extracorporeal membrane oxygenation (ECMO) was initiated. Twelve hours following ECMO initiation, high dose intravenous vitamin C was begun. The patient's recovery was rapid. ECMO and mechanical ventilation were discontinued by day-7 and the patient recovered with no long-term ARDS sequelae. Infusing high dose intravenous vitamin C into this patient with virus-induced ARDS was associated with rapid resolution of lung injury with no evidence of post-ARDS fibroproliferative sequelae. Intravenous vitamin C as a treatment for ARDS may open a new era of therapy for ARDS from many causes.

  8. Echocardiographic parameters of right ventricular function predict mortality in acute respiratory distress syndrome: a pilot study

    PubMed Central

    Wadia, Subeer K.; Kovach, Julie; Fogg, Louis; Tandon, Rajive

    2016-01-01

    Abstract Right ventricular (RV) dysfunction in acute respiratory distress syndrome (ARDS) contributes to increased mortality. Our aim is to identify reproducible transthoracic echocardiography (TTE) parameters of RV dysfunction that can be used to predict outcomes in ARDS. We performed a retrospective single-center cohort pilot study measuring tricuspid annular plane systolic excursion (TAPSE), Tei index, RV-fractional area change (RV-FAC), pulmonary artery systolic pressure (PASP), and septal shift, reevaluated by an independent blinded cardiologist (JK). Thirty-eight patients were included. Patients were divided on the basis of 30-day survival. Thirty-day mortality was 47%. Survivors were younger than nonsurvivors. Survivors had a higher pH, PaO2∶FiO2 ratio, and TAPSE. Acute Physiology and Chronic Health Evaluation II (APACHE II), Simplified Acute Physiology Score II (SAPS II), and Sequential Organ Failure Assessment (SOFA) scores were lower in survivors. TAPSE has the strongest association with increased 30-day mortality from date of TTE. Accordingly, TAPSE has a strong positive correlation with PaO2∶FiO2 ratios, and Tei index has a strong negative correlation with PaO2∶FiO2 ratios. Septal shift was associated with lower PaO2∶FiO2 ratios. Decrease in TAPSE, increase in Tei index, and septal shift were seen in the severe ARDS group. In multivariate logistic regression models, TAPSE maintained a significant association with mortality independent of age, pH, PaO2∶FiO2 ratios, positive end expiratory pressure, PCO2, serum bicarbonate, plateau pressures, driving pressures, APACHE II, SAPS II, and SOFA scores. In conclusion, TAPSE and other TTE parameters should be used as novel predictive indicators for RV dysfunction in ARDS. These parameters can be used as surrogate noninvasive RV hemodynamic measurements to be manipulated to improve mortality in patients with ARDS and contributory RV dysfunction. PMID:27252840

  9. Activation of necroptosis in a rat model of acute respiratory distress syndrome induced by oleic acid.

    PubMed

    Pan, Long; Yao, Dun-Chen; Yu, Yu-Zhong; Chen, Bing-Jun; Li, Sheng-Jie; Hu, Gui-He; Xi, Chang; Wang, Zi-Hui; Li, Jian-Hua; Long, Jie; Tu, Yong-Sheng

    2016-10-25

    The present study was aimed to investigate the role of necroptosis in the pathogenesis of acute respiratory distress syndrome (ARDS). The rat model of ARDS was induced by intravenous injection of oleic acid (OA), and observed for 4 h. The lung injury was evaluated by arterial blood gas, lung wet-dry weight ratio (W/D) and histological analyses. Simultaneously, bronchoalveolar lavage fluid (BALF) was collected for total and differential cell analysis and total protein determination. Tumor necrosis factor alpha (TNF-α) level in BALF was determined with a rat TNF-α ELISA kit. Expressions of receptor interacting protein kinase 1 (RIPK1), RIPK3 and mixed lineage kinase domain-like protein (MLKL) in lung tissue were determined by Western blot and immunohistochemical staining. The interaction between RIPK1 and RIPK3 was explored by immunoprecipitation. The results showed that, compared with those in control group, total white blood cells count (WBC), polymorphonuclear percentage (PMN%), total protein concentration, TNF-α level in BALF, W/D, and the alveolar-arterial oxygen tension difference (P(A-a)O2) in OA group were significantly increased at 4 h after OA injection. Western blot and immunostaining further showed remarkably increased expressions of RIPK1, RIPK3 and MLKL in lung tissue from OA group. Additionally, immunoprecipitation results indicated an enforced interaction between RIPK1 and RIPK3 in OA group. Collectively, the TNF-α level in BALF and the RIPK1-RIPK3-MLKL signaling pathway in lung tissue were found to be upregulated and activated with the process of ARDS. These findings implicate that RIPK1/RIPK3-mediated necroptosis plays a possible role in the pathogenesis of ARDS, which may provide a new idea to develop novel drugs for the therapy of ARDS.

  10. Distinct and replicable genetic risk factors for acute respiratory distress syndrome of pulmonary or extrapulmonary origin

    PubMed Central

    Tejera, Paula; Meyer, Nuala; Chen, Feng; Feng, Rui; Zhao, Yang; O’Mahony, D. Shane; Li, Lin; Sheu, Chau-Chyun; Zhai, Rihong; Wang, Zhaoxi; Su, Li; Bajwa, Ed; Ahasic, Amy M.; Clardy, Peter; Gong, Michelle N.; Frank, Angela J.; Lanken, Paul N.; Thompson, B. Taylor; Christie, Jason D.; Wurfel, Mark; O’Keefe, Grant; Christiani, David C.

    2013-01-01

    Background The role of genetics in the development of acute lung injury (ALI)/acute respiratory distress syndrome (ARDS) from direct or indirect lung injury has not been investigated specifically. The aim of this study was to identify genetic variants contributing to ARDS from pulmonary or extrapulmonary causes. Methods We conducted a multi-stage genetic association study. We first performed a large-scale genotyping (50K IBC Chip) in 1,717 Caucasian critically ill patients with either pulmonary or extrapulmonary injury, to identify single nucleotide polymorphisms (SNPs) associated with the development of ARDS from direct or indirect insults to the lung. Identified SNPs (p ≤ 0.0005) were validated in two separated populations (Stage II), with trauma (Population I; n = 765) and pneumonia/pulmonary sepsis (Population II; n = 838), as causes for ARDS/ALI. Genetic variants replicating their association with trauma related-ALI in Stage II were validated in a second trauma-associated ALI population (n = 224, Stage III). Results In Stage I, non-overlapping SNPs were significantly associated with ARDS from direct/indirect lung injury, respectively. The association between rs1190286 (POPDC3) and reduced risk of ARDS from pulmonary injury was validated in Stage II (p < 0.003). SNP rs324420 (FAAH) was consistently associated with increased risk of ARDS from extrapulmonary causes in two independent ALI-trauma populations (p < 0.007, Stage II; p < 0.05, Stage III). Meta-analysis confirmed these associations. Conclusions Different genetic variants may influence ARDS susceptibility depending on direct vs indirect insults. Functional SNPs in POPDC3 and FAAH genes may be driving the association with direct and indirect ALI, respectively. PMID:23048207

  11. Management and Outcomes of Acute Respiratory Distress Syndrome Caused by Blastomycosis

    PubMed Central

    Schwartz, Ilan S.; Embil, John M.; Sharma, Atul; Goulet, Stephen; Light, R. Bruce

    2016-01-01

    Abstract Acute respiratory distress syndrome (ARDS) is an uncommon, highly fatal, and poorly understood manifestation of blastomycosis. Optimal management remains unknown, including the roles of adjunctive corticosteroids and extracorporeal membrane oxygenation (ECMO). We conducted a retrospective chart review of patients with ARDS caused by blastomycosis, managed in intensive care units in Manitoba, Canada, from 1992 to 2014. ARDS was defined using the Berlin definition. Corticosteroid therapy was defined as ≥150 mg cortisol equivalent in 24 hours. Logistic regression was used to identify determinants of a fatal outcome, and bootstrap resampling was used to assess sample size requirements. Forty-three patients with ARDS caused by blastomycosis were identified. ARDS was mild, moderate, and severe in 2 (5%), 12 (28%), and 29 (67%) patients, respectively. Management included amphotericin B (n = 42, 98%), vasopressors (n = 36, 84%), corticosteroids (n = 22, 51%), renal replacement (n = 13, 30%), and ECMO (n = 4, 11%). Seventeen patients (40%) died. All patients treated with ECMO survived (P = 0.14). Corticosteroids were not associated with survival benefit in univariate (P = 0.43) or multivariate analyses (odds ratio 0.52, 95% confidence interval 0.11–2.34). Bootstrap studies indicated that almost 500 patients would be needed to confirm a significant reduction in mortality from corticosteroids (type I error = 0.05, power = 80%). Blastomycosis is an uncommon, albeit important, cause of ARDS in this geographic area. Given the rarity of disease and the large cohort needed to demonstrate mortality benefit, the role of adjunctive therapies, including corticosteroids and ECMO, may remain unconfirmed, and clinical judgment should guide management decisions. PMID:27149459

  12. Management and Outcomes of Acute Respiratory Distress Syndrome Caused by Blastomycosis: A Retrospective Case Series.

    PubMed

    Schwartz, Ilan S; Embil, John M; Sharma, Atul; Goulet, Stephen; Light, R Bruce

    2016-05-01

    Acute respiratory distress syndrome (ARDS) is an uncommon, highly fatal, and poorly understood manifestation of blastomycosis. Optimal management remains unknown, including the roles of adjunctive corticosteroids and extracorporeal membrane oxygenation (ECMO).We conducted a retrospective chart review of patients with ARDS caused by blastomycosis, managed in intensive care units in Manitoba, Canada, from 1992 to 2014. ARDS was defined using the Berlin definition. Corticosteroid therapy was defined as ≥150 mg cortisol equivalent in 24 hours. Logistic regression was used to identify determinants of a fatal outcome, and bootstrap resampling was used to assess sample size requirements.Forty-three patients with ARDS caused by blastomycosis were identified. ARDS was mild, moderate, and severe in 2 (5%), 12 (28%), and 29 (67%) patients, respectively. Management included amphotericin B (n = 42, 98%), vasopressors (n = 36, 84%), corticosteroids (n = 22, 51%), renal replacement (n = 13, 30%), and ECMO (n = 4, 11%). Seventeen patients (40%) died. All patients treated with ECMO survived (P = 0.14). Corticosteroids were not associated with survival benefit in univariate (P = 0.43) or multivariate analyses (odds ratio 0.52, 95% confidence interval 0.11-2.34). Bootstrap studies indicated that almost 500 patients would be needed to confirm a significant reduction in mortality from corticosteroids (type I error = 0.05, power = 80%).Blastomycosis is an uncommon, albeit important, cause of ARDS in this geographic area. Given the rarity of disease and the large cohort needed to demonstrate mortality benefit, the role of adjunctive therapies, including corticosteroids and ECMO, may remain unconfirmed, and clinical judgment should guide management decisions.

  13. Hyperpolarized Gas Diffusion MRI for the Study of Atelectasis and Acute Respiratory Distress Syndrome

    PubMed Central

    Cereda, Maurizio; Xin, Yi; Kadlecek, Stephen; Hamedani, Hooman; Rajaei, Jennia; Clapp, Justin; Rizi, Rahim R.

    2014-01-01

    Considerable uncertainty remains about the best ventilator strategies for the mitigation of atelectasis and associated airspace stretch in patients with acute respiratory distress syndrome (ARDS). In addition to several immediate physiological effects, atelectasis increases the risk of ventilator-associated lung injury (VALI), which has been shown to significantly worsen ARDS outcomes. A number of lung imaging techniques have made substantial headway in clarifying the mechanisms of atelectasis. This paper reviews the contributions of CT, PET, and conventional MRI to understanding this phenomenon. In doing so, it also reveals several important shortcomings inherent to each of these approaches. Once these shortcomings have been made apparent, we describe how hyperpolarized gas magnetic resonance imaging (HP MRI)—a technique that is uniquely able to assess responses to mechanical ventilation and lung injury in peripheral airspaces—is poised to fill several of these knowledge gaps. The HP-MRI-derived apparent diffusion coefficient (ADC) quantifies the restriction of 3He diffusion by peripheral airspaces, thereby obtaining pulmonary structural information at an extremely small scale. Lastly, this paper reports the results of a series of experiments that measured ADC in mechanically ventilated rats in order to investigate (i) the effect of atelectasis on ventilated airspaces; (ii) the relationship between positive end-expiratory pressure (PEEP), hysteresis, and the dimensions of peripheral airspaces; and (iii) the ability of PEEP and surfactant to reduce airspace dimensions after lung injury. An increase in ADC was found to be a marker of atelectasis-induced overdistension. With recruitment, higher airway pressures were shown to reduce stretch rather than worsen it. Moving forward, HP MRI has significant potential to shed further light on the atelectatic processes that occur during mechanical ventilation. PMID:24920074

  14. Polymorphisms in key pulmonary inflammatory pathways and the development of acute respiratory distress syndrome

    PubMed Central

    Brown, Samuel M.; Grissom, Colin K.; Rondina, Matthew T.; Hoidal, John R.; Scholand, Mary Beth; Wolff, Roger K.; Morris, Alan H.; Paine, Robert

    2015-01-01

    Purpose/Aim Acute Respiratory Distress Syndrome (ARDS) is an important clinical and public health problem. Why some at-risk individuals develop ARDS and others do not is unclear but may be related to differences in inflammatory and cell signaling systems. The Receptor for Advanced Glycation Endproducts (RAGE) and Granulocyte-Monocyte Stimulating Factor (GM-CSF) pathways have recently been implicated in pulmonary pathophysiology; whether genetic variation within these pathways contributes to ARDS risk or outcome is unknown. Materials and Methods We studied 842 patients from three centers in Utah and 14 non-Utah ARDS Network centers. We studied patients at risk for ARDS and patients with ARDS to determine whether Single Nucleotide Polymorphisms (SNPs) in the RAGE and GM-CSF pathways were associated with development of ARDS. We studied 29 SNPs in 5 genes within the two pathways and controlled for age, sepsis as ARDS risk factor, and severity of illness, while targeting a false discovery rate of ≤5%. In a secondary analysis we evaluated associations with mortality. Results Of 842 patients, 690 had ARDS, and 152 were at-risk. Sepsis was the risk factor for ARDS in 250 (30%) patients. When controlling for age, APACHE III score, sepsis as risk factor, and multiple comparisons, no SNPs were significantly associated with ARDS. In a secondary analysis, only rs743564 in CSF2 approached significance with regard to mortality (OR 2.17, unadjusted p = 0.005, adjusted p = 0.15). Conclusions Candidate SNPs within 5 genes in the RAGE and GM-CSF pathways were not significantly associated with development of ARDS in this multi-centric cohort. PMID:25513711

  15. Body Temperature and Mortality in Patients with Acute Respiratory Distress Syndrome

    PubMed Central

    Schell-Chaple, Hildy M.; Puntillo, Kathleen A.; Matthay, Michael A.; Liu, Kathleen D.

    2015-01-01

    Background Little is known about the relationship between body temperature and outcomes in patients with acute respiratory distress syndrome (ARDS). A better understanding of this relationship may provide evidence for fever suppression or warming interventions, which are commonly applied in practice. Objective To examine the relationship between body temperature and mortality in patients with ARDS. Methods Secondary analysis of body temperature and mortality using data from the ARDS Network Fluid and Catheter Treatment Trial (n =969). Body temperature at baseline and on study day 2, primary cause of ARDS, severity of illness, and 90-day mortality were analyzed by using multiple logistic regression. Results Mean baseline temperature was 37.5°C (SD, 1.1°C; range, 27.2°C-40.7°C). At baseline, fever (≥ 38.3°C) was present in 23% and hypothermia (< 36°C) in 5% of the patients. Body temperature was a significant predictor of 90-day mortality after primary cause of ARDS and score on the Acute Physiology and Chronic Health Evaluation III were adjusted for. Higher temperature was associated with decreased mortality: for every 1°C increase in baseline temperature, the odds of death decreased by 15% (odds ratio, 0.85; 95% CI, 0.73-0.98, P = .03). When patients were divided into 5 temperature groups, mortality was lower with higher temperature (P for trend=.02). Conclusions Early in ARDS, fever is associated with improved survival rates. Fever in the acute phase response to lung injury and its relationship to recovery may be an important factor in determining patients' outcome and warrants further study. PMID:25554550

  16. MISCLASSIFICATION OF ACUTE RESPIRATORY DISTRESS SYNDROME AFTER TRAUMATIC INJURY: THE COST OF LESS RIGOROUS APPROACHES

    PubMed Central

    Hendrickson, Carolyn M; Dobbins, Sarah; Redick, Brittney J; Greenberg, Molly D; Calfee, Carolyn S; Cohen, Mitchell Jay

    2015-01-01

    BACKGROUND Adherence to rigorous research protocols for identifying acute respiratory distress syndrome (ARDS) after trauma is variable. To examine how misclassification of ARDS may bias observational studies in trauma populations, we evaluated the agreement of two methods for adjudicating ARDS after trauma: the gold standard, direct review of chest radiographs and review of dictated radiology reports, a commonly used alternative. METHODS This nested cohort study included 123 mechanically ventilated patients between 2005–2008, with at least one PaO2:FiO2 <300 within the first 8 days of admission. Two blinded physician investigators adjudicated ARDS by two methods. The investigators directly reviewed all chest radiographs to evaluate for bilateral infiltrates. Several months later, blinded to their previous assessments, they adjudicated ARDS using a standardized rubric to classify radiology reports. A kappa statistics was calculated. Regression analyses quantified the association between established risk factors as well as important clinical outcomes and ARDS determined by the aforementioned methods as well as hypoxemia as a surrogate marker. RESULTS The kappa was 0.47 for the observed agreement between ARDS adjudicated by direct review of chest radiographs and ARDS adjudicated by review of radiology reports. Both the magnitude and direction of bias on the estimates of association between ARDS and established risk factors as well as clinical outcomes varied by method of adjudication. CONCLUSION Classification of ARDS by review of dictated radiology reports had only moderate agreement with the gold standard, ARDS adjudicated by direct review of chest radiographs. While the misclassification of ARDS had varied effects on the estimates of associations with established risk factors, it tended to weaken the association of ARDS with important clinical outcomes. A standardized approach to ARDS adjudication after trauma by direct review of chest radiographs will minimize

  17. Intravenous vitamin C as adjunctive therapy for enterovirus/rhinovirus induced acute respiratory distress syndrome

    PubMed Central

    Fowler III, Alpha A; Kim, Christin; Lepler, Lawrence; Malhotra, Rajiv; Debesa, Orlando; Natarajan, Ramesh; Fisher, Bernard J; Syed, Aamer; DeWilde, Christine; Priday, Anna; Kasirajan, Vigneshwar

    2017-01-01

    We report a case of virus-induced acute respiratory distress syndrome (ARDS) treated with parenteral vitamin C in a patient testing positive for enterovirus/rhinovirus on viral screening. This report outlines the first use of high dose intravenous vitamin C as an interventional therapy for ARDS, resulting from enterovirus/rhinovirus respiratory infection. From very significant preclinical research performed at Virginia Commonwealth University with vitamin C and with the very positive results of a previously performed phase I safety trial infusing high dose vitamin C intravenously into patients with severe sepsis, we reasoned that infusing identical dosing to a patient with ARDS from viral infection would be therapeutic. We report here the case of a 20-year-old, previously healthy, female who contracted respiratory enterovirus/rhinovirus infection that led to acute lung injury and rapidly to ARDS. She contracted the infection in central Italy while on an 8-d spring break from college. During a return flight to the United States, she developed increasing dyspnea and hypoxemia that rapidly developed into acute lung injury that led to ARDS. When support with mechanical ventilation failed, extracorporeal membrane oxygenation (ECMO) was initiated. Twelve hours following ECMO initiation, high dose intravenous vitamin C was begun. The patient’s recovery was rapid. ECMO and mechanical ventilation were discontinued by day-7 and the patient recovered with no long-term ARDS sequelae. Infusing high dose intravenous vitamin C into this patient with virus-induced ARDS was associated with rapid resolution of lung injury with no evidence of post-ARDS fibroproliferative sequelae. Intravenous vitamin C as a treatment for ARDS may open a new era of therapy for ARDS from many causes. PMID:28224112

  18. Pulmonary pathology in surfactant-treated preterm infants with respiratory distress syndrome: an autopsy study.

    PubMed

    Toti, P; Buonocore, G; Rinaldi, G; Catella, A M; Bracci, R

    1996-01-01

    The present study examines the histological features of the lungs of neonates who died of respiratory distress syndrome or related complications after surfactant therapy. Our aim was to determine whether these lungs showed any unusual histological findings. Complete autopsies were performed 6-12 h after death in 10 surfactant-treated preterm infants and in 30 infants who died before surfactant therapy was available. Representative paraffin sections of all pulmonary lobes, stained with haematoxylin and eosin, were examined microscopically. A few selected slides were also stained with periodic acid-Schiff, Vierhoff-van Gieson, and Mallory trichrome. Hyaline membrane disease and bronchopulmonary dysplasia were present in each group, although there was an increased incidence of intra-alveolar haemorrhage in surfactant-treated babies (in 8 of 10 surfactant-treated as compared with 7 of 30 untreated babies). Amongst those treated with surfactant, we observed the persistence of acute alveolar damage with unresolved hyaline membrane disease in 5 infants who died at the ages of 5, 6, 10, 12, and 13 days, respectively, and histological evidence of pneumocyte type 2 hyperplasia and dysplasia in 2 infants who died at 22 and 41 days of age, respectively. These observations reveal that surfactant-treated infants who fail to respond to therapy have continuing alveolar injury and an increased incidence of intra-alveolar haemorrhage. Since oxygen radicals can induce pneumocyte damage and necrosis and since free radicals provoke alveolar haemorrhage in animal models, we propose that the lesions we observed may stem from a lack, in some preterm babies, of specific mechanisms that detoxify oxygen radicals.

  19. Computer simulation allows goal-oriented mechanical ventilation in acute respiratory distress syndrome

    PubMed Central

    Uttman, Leif; Ögren, Helena; Niklason, Lisbet; Drefeldt, Björn; Jonson, Björn

    2007-01-01

    Introduction To prevent further lung damage in patients with acute respiratory distress syndrome (ARDS), it is important to avoid overdistension and cyclic opening and closing of atelectatic alveoli. Previous studies have demonstrated protective effects of using low tidal volume (VT), moderate positive end-expiratory pressure and low airway pressure. Aspiration of dead space (ASPIDS) allows a reduction in VT by eliminating dead space in the tracheal tube and tubing. We hypothesized that, by applying goal-orientated ventilation based on iterative computer simulation, VT can be reduced at high respiratory rate and much further reduced during ASPIDS without compromising gas exchange or causing high airway pressure. Methods ARDS was induced in eight pigs by surfactant perturbation and ventilator-induced lung injury. Ventilator resetting guided by computer simulation was then performed, aiming at minimal VT, plateau pressure 30 cmH2O and isocapnia, first by only increasing respiratory rate and then by using ASPIDS as well. Results VT decreased from 7.2 ± 0.5 ml/kg to 6.6 ± 0.5 ml/kg as respiratory rate increased from 40 to 64 ± 6 breaths/min, and to 4.0 ± 0.4 ml/kg when ASPIDS was used at 80 ± 6 breaths/min. Measured values of arterial carbon dioxide tension were close to predicted values. Without ASPIDS, total positive end-expiratory pressure and plateau pressure were slightly higher than predicted, and with ASPIDS they were lower than predicted. Conclusion In principle, computer simulation may be used in goal-oriented ventilation in ARDS. Further studies are needed to investigate potential benefits and limitations over extended study periods. PMID:17352801

  20. The potential role and limitations of echocardiography in acute respiratory distress syndrome.

    PubMed

    Lazzeri, Chiara; Cianchi, Giovanni; Bonizzoli, Manuela; Batacchi, Stefano; Peris, Adriano; Gensini, Gian Franco

    2016-04-01

    Bedside use of Doppler echocardiography is being featured as a promising, clinically useful tool in assessing the pulmonary circulation in patients with acute respiratory distress syndrome (ARDS). The present review is aimed at summarizing the available evidence obtained with echocardiography on right ventricle (RV) function and pulmonary circulation in ARDS and to highlight the potential of this technique in clinical practice (only articles in English language were considered). According to the available evidence on echocardiographic findings, the following conclusions can be drawn: (a) echocardiography (transthoracic and transesophageal) has a growing role in the management ARDS patients mainly because of the strict interactions between the lung (and ventilation) and the RV and pulmonary circulation; (b) there may be a continuum of alterations in RV size and function and pulmonary circulation which may end in the development of acute cor pulmonale, probably paralleling ARDS disease severity; and (c) the detection of acute cor pulmonale should prompt intensivists to tailor their ventilatory strategy to the individual patient depending on the echocardiography findings. Bearing in mind the clinical role and growing importance of echocardiography in ARDS and the available evidence on this topic, we present a flow chart including the parameters to be measured and the timing of echo exams in ARDS patients. Despite the important progress that echocardiography has gained in the evaluation of patients with ARDS, several open questions remain and echocardiography still appears to be underused in these patients. A more systematic use of echocardiography (mainly through shared protocols) in ARDS could help intensivists to tailor the optimal treatment in individual patients as well as highlighting the limits and potential of this methodology in patients with ALI.

  1. Proteomic Profiles in Acute Respiratory Distress Syndrome Differentiates Survivors from Non-Survivors

    PubMed Central

    Bhargava, Maneesh; Becker, Trisha L.; Viken, Kevin J.; Jagtap, Pratik D.; Dey, Sanjoy; Steinbach, Michael S.; Wu, Baolin; Kumar, Vipin; Bitterman, Peter B.; Ingbar, David H.; Wendt, Christine H.

    2014-01-01

    Acute Respiratory Distress Syndrome (ARDS) continues to have a high mortality. Currently, there are no biomarkers that provide reliable prognostic information to guide clinical management or stratify risk among clinical trial participants. The objective of this study was to probe the bronchoalveolar lavage fluid (BALF) proteome to identify proteins that differentiate survivors from non-survivors of ARDS. Patients were divided into early-phase (1 to 7 days) and late-phase (8 to 35 days) groups based on time after initiation of mechanical ventilation for ARDS (Day 1). Isobaric tags for absolute and relative quantitation (iTRAQ) with LC MS/MS was performed on pooled BALF enriched for medium and low abundance proteins from early-phase survivors (n = 7), early-phase non-survivors (n = 8), and late-phase survivors (n = 7). Of the 724 proteins identified at a global false discovery rate of 1%, quantitative information was available for 499. In early-phase ARDS, proteins more abundant in survivors mapped to ontologies indicating a coordinated compensatory response to injury and stress. These included coagulation and fibrinolysis; immune system activation; and cation and iron homeostasis. Proteins more abundant in early-phase non-survivors participate in carbohydrate catabolism and collagen synthesis, with no activation of compensatory responses. The compensatory immune activation and ion homeostatic response seen in early-phase survivors transitioned to cell migration and actin filament based processes in late-phase survivors, revealing dynamic changes in the BALF proteome as the lung heals. Early phase proteins differentiating survivors from non-survivors are candidate biomarkers for predicting survival in ARDS. PMID:25290099

  2. NF-kappaB regulatory mechanisms in alveolar macrophages from patients with acute respiratory distress syndrome.

    PubMed

    Moine, P; McIntyre, R; Schwartz, M D; Kaneko, D; Shenkar, R; Le Tulzo, Y; Moore, E E; Abraham, E

    2000-02-01

    Activation of the nuclear regulatory factor NF-kappaB occurs in the lungs of patients with the acute respiratory distress syndrome (ARDS) and may contribute to the increased expression of immunoregulatory cytokines and other proinflammatory mediators in this setting. Because of the important role that NF-kappaB activation appears to play in the development of acute lung injury, we examined cytoplasmic and nuclear NF-kapppaB counterregulatory mechanisms, involving IkappaB proteins, in alveolar macrophages obtained from 7 control patients without lung injury and 11 patients with established ARDS. Cytoplasmic levels of the NF-kappaB subunits p50, p65, and c-Rel were significantly decreased in alveolar macrophages from patients with ARDS, consistent with enhanced migration of liberated NF-kappaB dimers from the cytoplasm to the nucleus. Cytoplasmic and nuclear levels of IkappaBalpha were not significantly altered in alveolar macrophages from patients with established ARDS, compared with controls. In contrast, nuclear levels of Bcl-3 were significantly decreased in patients with ARDS compared with controls (P = 0.02). No IkappaBgamma, IkappaBbeta, or p105 proteins were detected in the cytoplasm of alveolar macrophages from control patients or patients with ARDS. The presence of activated NF-kappaB in alveolar macrophages from patients with established ARDS implies the presence of an ongoing stimulus for NF-kappaB activation. In this setting, appropriate counterregulatory mechanisms to normalize nuclear levels of NF-kappaB and to suppress NF-kappaB-mediated transcription, such as increased cytoplasmic and nuclear IkappaBalpha levels or decreased Bcl-3 levels, appeared to be induced. Nevertheless, even though counterregulatory mechanisms to NF-kappaB activation are activated in lung macrophages of patients with ARDS, NF-kappaB remains activated. These results suggest that fundamental abnormalities in transcriptional mechanisms involving NF-kappaB and important in the

  3. Temporal patterns of radiographic infiltration in severely traumatized patients with and without adult respiratory distress syndrome.

    PubMed

    Johnson, K S; Bishop, M H; Stephen, C M; Jorgens, J; Shoemaker, W C; Shori, S K; Ordog, G; Thadepalli, H; Appel, P L; Kram, H B

    1994-05-01

    We prospectively evaluated the patterns of pulmonary structural and functional changes in 100 consecutive surgical intensive care unit trauma patients who had (1) emergent major surgery, (2) a pelvic fracture, or (3) two or more major long bone fractures. For each patient, arterial blood gas measurements (ABGs), central venous pressure (CVP), pulmonary capillary occlusion pressure (PAOP), thoracic compliance, arterial oxygen tension/fraction of inspired oxygen (PAO2/FIO2), pulmonary venous admixture (Qs/Qt), and portable chest roentgenograms were sequentially tracked. The senior staff radiologist interpreted all chest roentgenograms. Pulmonary infiltration was quantitated in each of six fields using a scale ranging from 0 to 4, with 0 being no infiltration and 4 being the maximum. Adult respiratory distress syndrome (ARDS) was defined as follows: Qs/Qt > or = 20%, PAO2/FIO2 < 250 or both; dependence on mechanical ventilation for life support for > or = 24 hours; PAOP or CVP or both < 20 mm Hg; and thoracic compliance < 50 mL/cm H2O. Time zero (T0) the time of onset of ARDS, was defined as the time these criteria were met. Eighty-three of 100 study group patients had penetrating injuries, and 17 were admitted with blunt trauma. Fifty-one of 100 patients developed ARDS: 36 of 51 died. Only 4 of 49 (8%) patients without ARDS died. The injured lungs of patients with and without ARDS had similar amounts of infiltration over most measured time intervals. The noninjured lungs of the ARDS patients, however, had significantly greater infiltration than those without ARDS at T0 and over subsequent time intervals.(ABSTRACT TRUNCATED AT 250 WORDS)

  4. Safety and Efficacy of Combined Extracorporeal Co2 Removal and Renal Replacement Therapy in Patients With Acute Respiratory Distress Syndrome and Acute Kidney Injury: The Pulmonary and Renal Support in Acute Respiratory Distress Syndrome Study*

    PubMed Central

    Castanier, Matthias; Signouret, Thomas; Soundaravelou, Rettinavelou; Lepidi, Anne; Seghboyan, Jean-Marie

    2015-01-01

    Objective: To assess the safety and efficacy of combining extracorporeal Co2 removal with continuous renal replacement therapy in patients presenting with acute respiratory distress syndrome and acute kidney injury. Design: Prospective human observational study. Settings: Patients received volume-controlled mechanical ventilation according to the acute respiratory distress syndrome net protocol. Continuous venovenous hemofiltration therapy was titrated to maintain maximum blood flow and an effluent flow of 45 mL/kg/h with 33% predilution. Patients: Eleven patients presenting with both acute respiratory distress syndrome and acute kidney injury required renal replacement therapy. Interventions: A membrane oxygenator (0.65 m2) was inserted within the hemofiltration circuit, either upstream (n = 7) or downstream (n = 5) of the hemofilter. Baseline corresponded to tidal volume 6 mL/kg of predicted body weight without extracorporeal Co2 removal. The primary endpoint was 20% reduction in Paco2 at 20 minutes after extracorporeal Co2 removal initiation. Tidal volume was subsequently reduced to 4 mL/kg for the remaining 72 hours. Measurements and Main Results: Twelve combined therapies were conducted in the 11 patients. Age was 70 ± 9 years, Simplified Acute Physiology Score II was 69 ± 13, Sequential Organ Failure Assessment score was 14 ± 4, lung injury score was 3 ± 0.5, and Pao2/Fio2 was 135 ± 41. Adding extracorporeal Co2 removal at tidal volume 6 mL/kg decreased Paco2 by 21% (95% CI, 17–25%), from 47 ± 11 to 37 ± 8 Torr (p < 0.001). Lowering tidal volume to 4 mL/kg reduced minute ventilation from 7.8 ± 1.5 to 5.2 ± 1.1 L/min and plateau pressure from 25 ± 4 to 21 ± 3 cm H2O and raised Paco2 from 37 ± 8 to 48 ± 10 Torr (all p < 0.001). On an average of both positions, the oxygenator’s blood flow was 410 ± 30 mL/min and the Co2 removal rate was 83 ± 20 mL/min. The oxygenator blood flow (p <0.001) and the Co2 removal rate (p = 0.083) were higher when

  5. A multicenter, randomized, double-blind trial of a new porcine surfactant in premature infants with respiratory distress syndrome

    PubMed Central

    Rebello, Celso Moura; Precioso, Alexander Roberto; Mascaretti, Renata Suman

    2014-01-01

    Objective To compare the efficacy and safety of a new porcine-derived pulmonary surfactant developed by Instituto Butantan with those of animal-derived surfactants commercially available in Brazil, regarding neonatal mortality and the major complications of prematurity in preterm newborns with birth weight up to 1500g and diagnosed with respiratory distress syndrome. Methods Neonates diagnosed with respiratory distress syndrome were randomized to receive either Butantan surfactant (Butantan group) or one of the following surfactants: Survanta® or Curosurf®. Newborns receiving Survanta® or Curosurf® comprised the control group. The main outcome measures were mortality rates at 72 hours and at 28 days of life; the typical complications of prematurity as evaluated on the 28th day of life were defined as secundary outcomes. Results No differences were observed between the Butantan (n=154) and control (n=173) groups in relation to birth weight, gestational age, sex, and prenatal use of corticosteroids, or in mortality rates both at 72 hours (14.19% versus 14.12%; p=0.98) and at 28 days (39.86% versus 33.33%; p=0.24) of life. Higher 1- and 5-minute Apgar scores were observed among control group newborns. No differences were observed as regards the secondary outcomes, except for greater need for supplemental oxygen and a higher incidence of interstitial pulmonary emphysema in the Butantan group. Conclusion The mortality rates at 72 hours and 28 days of life and the incidence of major complications of prematurity were comparable to those found with the animal-derived surfactants commercially available in Brazil, showing the efficacy and safety of the new surfactant in the treatment of respiratory distress syndrome in newborns. PMID:25628188

  6. Three-view bedside ultrasound for the differentiation of acute respiratory distress syndrome from cardiogenic pulmonary edema.

    PubMed

    Mantuani, Daniel; Nagdev, Arun; Stone, Michael

    2012-09-01

    Bedside ultrasound is being increasingly used by emergency physicians (EPs) for the differentiation of acute dyspnea in critically ill patients. Lung ultrasound is emerging as a highly sensitive tool in diagnosing alveolar interstitial edema with the presence of diffuse “B-lines” arising from the pleural line. However, when used independently, lung ultrasound is unable to differentiate between cardiogenic and noncardiogenic causes of pulmonary edema. This case report describes a rapid 3-view or “triple scan” sonographic examination to differentiate acute respiratory distress syndrome (ARDS) from cardiogenic pulmonary edema.

  7. Acute respiratory distress syndrome and chemical burns after exposure to chlorine-containing bleach: a case report.

    PubMed

    Shin, Hong-Joon; Chang, Jin-Sun; Ahn, Seong; Kim, Tae-Ok; Park, Cheol-Kyu; Lim, Jung-Hwan; Oh, In-Jae; Kim, Yu-Il; Lim, Sung-Chul; Kim, Young-Chul; Kwon, Yong-Soo

    2017-01-01

    Chlorine-containing bleach can cause acute respiratory distress syndrome (ARDS) and chemical burns. However, simultaneous occurrence of the two conditions caused by this agent is very rare. We describe the case of a 74-year-old female who presented with shortness of breath and hemoptysis following accidental exposure to chlorine-containing bleach. She had second- to third-degree chemical burns on both buttocks and thighs, and received mechanical ventilation because of the development of ARDS. Mechanical ventilation was discontinued on day 6 of hospitalization because of the rapid improvement of hypoxemia, and the patient was transferred to another hospital for further management of the chemical burns on day 18.

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

    PubMed

    García de Acilu, M; Leal, S; Caralt, B; Roca, O; Sabater, J; Masclans, J R

    2015-01-01

    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.

  9. Immunoglobulin M-enriched intravenous polyclonal immunoglobulins reduce bacteremia following Klebsiella pneumoniae infection in an acute respiratory distress syndrome rat model.

    PubMed

    Lachmann, R A; van Kaam, A H L C; Haitsma, J J; Verbrugge, S J C; Delreu, F; Lachmann, B

    2004-06-01

    Mechanical ventilation is known to induce bacterial translocation from the lung into the systemic circulation. This study determined the effect of immunoglobulin M (IgM)-enriched polyclonal immunoglobulins on bacteremia due to ventilation-induced translocation in an acute respiratory distress syndrome (ARDS) rat model with Klebsiella-induced pneumonia. After whole lung lavage, Sprague-Dawley rats intravenously received either a high dose or a low dose of an immunoglobulin preparation, or an albumin solution as control, followed by an intratracheal injection of a Klebsiella pneumoniae solution. Blood colony-forming units (CFUs) in the treatment groups were significantly lower during the 3-hour ventilation period compared to the control group. The authors conclude that IgM-enriched polyclonal immunoglobulins lead to a reduction of bacteria in blood of surfactant-deficient, ventilated rats infected with Klebsiella pneumoniae.

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

    PubMed Central

    García de Acilu, M.; Leal, S.; Caralt, B.; Roca, O.; Sabater, J.; Masclans, J. R.

    2015-01-01

    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. PMID:26339627

  11. Comparison of inhaled milrinone, nitric oxide and prostacyclin in acute respiratory distress syndrome

    PubMed Central

    Albert, Martin; Corsilli, Daniel; Williamson, David R; Brosseau, Marc; Bellemare, Patrick; Delisle, Stéphane; Nguyen, Anne QN; Varin, France

    2017-01-01

    AIM To evaluate the safety and efficacy of inhaled milrinone in acute respiratory distress syndrome (ARDS). METHODS Open-label prospective cross-over pilot study where fifteen adult patients with hypoxemic failure meeting standard ARDS criteria and monitored with a pulmonary artery catheter were recruited in an academic 24-bed medico-surgical intensive care unit. Random sequential administration of iNO (20 ppm) or nebulized epoprostenol (10 μg/mL) was done in all patients. Thereafter, inhaled milrinone (1 mg/mL) alone followed by inhaled milrinone in association with inhaled nitric oxide (iNO) was administered. A jet nebulization device synchronized with the mechanical ventilation was use to administrate the epoprostenol and the milrinone. Hemodynamic measurements and partial pressure of arterial oxygen (PaO2) were recorded before and after each inhaled therapy administration. RESULTS The majority of ARDS were of pulmonary cause (n = 13) and pneumonia (n = 7) was the leading underlying initial disease. Other pulmonary causes of ARDS were: Post cardiopulmonary bypass (n = 2), smoke inhalation injury (n = 1), thoracic trauma and pulmonary contusions (n = 2) and aspiration (n = 1). Two patients had an extra pulmonary cause of ARDS: A polytrauma patient and an intra-abdominal abscess Inhaled nitric oxide, epoprostenol, inhaled milrinone and the combination of inhaled milrinone and iNO had no impact on systemic hemodynamics. No significant adverse events related to study medications were observed. The median increase of PaO2 from baseline was 8.8 mmHg [interquartile range (IQR) = 16.3], 6.0 mmHg (IQR = 18.4), 6 mmHg (IQR = 15.8) and 9.2 mmHg (IQR = 20.2) respectively with iNO, epoprostenol, inhaled milrinone, and iNO added to milrinone. Only iNO and the combination of inhaled milrinone and iNO had a statistically significant effect on PaO2. CONCLUSION When comparing the effects of inhaled NO, milrinone and epoprostenol, only NO significantly improved oxygenation

  12. Negative- versus positive-pressure ventilation in intubated patients with acute respiratory distress syndrome

    PubMed Central

    2012-01-01

    Introduction Recent experimental data suggest that continuous external negative-pressure ventilation (CENPV) results in better oxygenation and less lung injury than continuous positive-pressure ventilation (CPPV). The effects of CENPV on patients with acute respiratory distress syndrome (ARDS) remain unknown. Methods We compared 2 h CENPV in a tankrespirator ("iron lung") with 2 h CPPV. The six intubated patients developed ARDS after pulmonary thrombectomy (n = 1), aspiration (n = 3), sepsis (n = 1) or both (n = 1). We used a tidal volume of 6 ml/kg predicted body weight and matched lung volumes at end expiration. Haemodynamics were assessed using the pulse contour cardiac output (PiCCO) system, and pressure measurements were referenced to atmospheric pressure. Results CENPV resulted in better oxygenation compared to CPPV (median ratio of arterial oxygen pressure to fraction of inspired oxygen of 345 mmHg (minimum-maximum 183 to 438 mmHg) vs 256 mmHg (minimum-maximum 123 to 419 mmHg) (P < 0.05). Tank pressures were -32.5 cmH2O (minimum-maximum -30 to -43) at end inspiration and -15 cmH2O (minimum-maximum -15 to -19 cmH2O) at end expiration. NO Inspiratory transpulmonary pressures decreased (P = 0.04) and airway pressures were considerably lower at inspiration (-1.5 cmH2O (minimum-maximum -3 to 0 cmH2O) vs 34.5 cmH2O (minimum-maximum 30 to 47 cmH2O), P = 0.03) and expiration (4.5 cmH2O (minimum-maximum 2 to 5) vs 16 cmH2O (minimum-maximum 16 to 23), P =0.03). During CENPV, intraabdominal pressures decreased from 20.5 mmHg (12 to 30 mmHg) to 1 mmHg (minimum-maximum -7 to 5 mmHg) (P = 0.03). Arterial pressures decreased by approximately 10 mmHg and central venous pressures by 18 mmHg. Intrathoracic blood volume indices and cardiac indices increased at the initiation of CENPV by 15% and 20% (P < 0.05), respectively. Heart rate and extravascular lung water indices remained unchanged. Conclusions CENPV with a tank respirator improved gas exchange in patients with ARDS at

  13. Prognostic and diagnostic value of plasma soluble ST2 concentrations in Acute Respiratory Distress Syndrome

    PubMed Central

    Bajwa, Ednan K.; Volk, Jessica A.; Christiani, David C.; Harris, R. Scott; Matthay, Michael A.; Thompson, B. Taylor; Januzzi, James L.

    2013-01-01

    Objective Soluble ST2 (sST2) is a biomarker of myocardial strain and inflammation. The characteristics of acute respiratory distress syndrome (ARDS) include inflammation and cardiovascular dysfunction. We sought to determine whether plasma sST2 concentration is associated with outcome and response to conservative fluid management, and whether sST2 concentration discriminates ARDS from decompensated heart failure (HF). Design, Setting, and Patients We assayed plasma sST2 concentrations in 826 patients in the Fluid and Catheter Treatment Trial (FACTT), a multi-center randomized controlled trial of conservative fluid management in ARDS, as well as a cohort of patients with decompensated HF. We tested whether sST2 was associated with outcome, response to therapy, and diagnostic utility for ARDS vs. HF. Measurements and Main Results Non-survivors had higher day 0 (P<.0001) and day 3 (P<.0001) sST2 concentrations. After adjustment for severity of illness, higher sST2 concentration was associated with mortality, with odds ratio (ORadj) 1.47 (95% confidence interval [CI] 0.99 – 2.20, P=.06) at day 0, 2.94 (95% CI 2.00 – 4.33, P<.0001) at day 3, and 3.63 (95% CI 2.38 – 5.53, P<.0001) if sST2 increased between days. Cumulative fluid balance was more positive among patients with higher day 0 (median 5212 mL, interquartile range [IQR] 200 – 12284 vs. 2020 mL, −2034 – 7091; P<0.0001), and day 3 sST2 (median 7678 mL, IQR 2217 – 14278 vs. 1492 mL, −2384 – 6239; P<0.0001). sST2 showed excellent discriminative ability between the FACTT and HF populations (Area under ROC curve=0.98, P<0.0001). Conclusions Higher sST2 concentrations are associated with worse outcome in ARDS and may have value for discriminating ARDS from heart failure. PMID:23939353

  14. Systemic combined melatonin-mitochondria treatment improves acute respiratory distress syndrome in the rat.

    PubMed

    Sun, Cheuk-Kwan; Lee, Fan-Yen; Kao, Ying-Hsien; Chiang, Hsin-Ju; Sung, Pei-Hsun; Tsai, Tzu-Hsien; Lin, Yu-Chun; Leu, Steve; Wu, Ying-Chung; Lu, Hung-I; Chen, Yung-Lung; Chung, Sheng-Ying; Su, Hong-Lin; Yip, Hon-Kan

    2015-03-01

    Despite high in-hospital mortality associated with acute respiratory distress syndrome (ARDS), there is no effective therapeutic strategy. We tested the hypothesis that combined melatonin-mitochondria treatment ameliorates 100% oxygen-induced ARDS in rats. Adult male Sprague-Dawley rats (n = 40) were equally categorized into normal controls, ARDS, ARDS-melatonin, ARDS with intravenous liver-derived mitochondria (1500 μg per rat 6 hr after ARDS induction), and ARDS receiving combined melatonin-mitochondria. The results showed that 22 hr after ARDS induction, oxygen saturation (saO2 ) was lowest in the ARDS group and highest in normal controls, significantly lower in ARDS-melatonin and ARDS-mitochondria than in combined melatonin-mitochondria group, and significantly lower in ARDS-mitochondria than in ARDS-melatonin group. Conversely, right ventricular systolic blood pressure and lung weight showed an opposite pattern compared with saO2 among all groups (all P < 0.001). Histological integrity of alveolar sacs showed a pattern identical to saO2 , whereas lung crowding score exhibited an opposite pattern (all P < 0.001). Albumin level and inflammatory cells (MPO+, CD40+, CD11b/c+) from bronchoalveolar lavage fluid showed a pattern opposite to saO2 (all P < 0.001). Protein expression of indices of inflammation (MMP-9, TNF-α, NF-κB), oxidative stress (oxidized protein, NO-1, NOX-2, NOX-4), apoptosis (mitochondrial Bax, cleaved caspase-3, and PARP), fibrosis (Smad3, TGF-β), mitochondrial damage (cytochrome C), and DNA damage (γ-H2AX+) exhibited an opposite pattern compared to saO2 in all groups, whereas protein (HO-1, NQO-1, GR, GPx) and cellular (HO-1+) expressions of antioxidants exhibited a progressively increased pattern from normal controls to ARDS combined melatonin-mitochondria group (all P < 0.001). In conclusion, combined melatonin-mitochondrial was superior to either treatment alone in attenuating ARDS in this rat model.

  15. Lung recruitment maneuver during volume guarantee ventilation of preterm infants with acute respiratory distress syndrome.

    PubMed

    Castoldi, Francesca; Daniele, Irene; Fontana, Paola; Cavigioli, Francesco; Lupo, Enrica; Lista, Gianluca

    2011-08-01

    Preterm infants need the achievement of adequate lung volume. Lung recruitment maneuver (LRM) is applied during high-frequency oscillatory ventilation. We investigated the effect of an LRM with positive end-expiratory pressure (PEEP) on oxygenation and outcomes in infants conventionally ventilated for respiratory distress syndrome (RDS). Preterm infants in assisted controlled ventilation+volume guarantee for RDS after surfactant randomly received an LRM (group A) or did not (group B). LRM entailed increments of 0.2 cm H (2)O PEEP every 5 minutes, until fraction of inspired oxygen (Fi O(2))=0.25. Then PEEP was reduced and the lung volume was set on the deflation limb of the pressure/volume curve. When saturation of peripheral oxygen fell and Fi O(2) rose, we reincremented PEEP until Sp O(2) became stable. Group A ( N=10) and group B ( N=10) infants were similar: gestational age 25 ± 2 versus 25 ± 2 weeks; body weight 747 ± 233 versus 737 ± 219 g; clinical risk index for babies 9.8 versus 8.1; initial Fi O(2) 56 ± 24 versus 52 ± 21, respectively. LRM began at 86 ± 69 minutes of age and lasted for 61 ± 18 minutes. Groups A and B showed different max PEEP during the first 12 hours of life (6.1 ± 0.3 versus 5.3 ± 0.3 cm H (2)O, P=0.00), time to lowest Fi O(2) (94 ± 24 versus 435 ± 221 minutes; P=0.000) and O(2) dependency (29 ± 12 versus 45 ± 17 days; P=0.04). No adverse events and no differences in the outcomes were observed. LRM led to the earlier lowest Fi O(2) of the first 12 hours of life and a shorter O (2) dependency.

  16. Clinical and biological role of secretory phospholipase A2 in acute respiratory distress syndrome infants

    PubMed Central

    2013-01-01

    Introduction Secretory phospholipase A2 is supposed to play a role in acute lung injury but no data are available for pediatric acute respiratory distress syndrome (ARDS). It is not clear which enzyme subtypes are secreted and what the relationships are between enzyme activity, biophysical and biochemical parameters, and clinical outcomes. We aimed to measure the enzyme and identify its subtypes and to study its biochemical and biophysical effect. The secondary aim was to correlate enzyme activity with clinical outcome. Methods Bronchoalveolar lavage was performed in 24 infants with ARDS and 14 controls with no lung disease. Samples were assayed for secretory phospholipase A2 and molecules related to its activity and expression. Western blotting and captive bubble surfactometry were also performed. Clinical data were real time downloaded. Results Tumor necrosis factor-α (814 (506-2,499) vs. 287 (111-1,315) pg/mL; P = 0.04), enzyme activity (430 (253-600) vs. 149 (61-387) IU/mL; P = 0.01), free fatty acids (4.3 (2.8-8.6) vs. 2 (0.8-4.6) mM; P = 0.026), and minimum surface tension (25.6 ± 6.1 vs. 18 ± 1.8 mN/m; P = 0.006) were higher in ARDS than in controls. Phospholipids are lower in ARDS than in controls (76.5 (54-100) vs. 1,094 (536-2,907) μg/mL; P = 0.0001). Three enzyme subtypes were identified (-IIA, -V, -X), although in lower quantities in controls; another subtype (-IB) was mainly detected in ARDS. Significant correlations exist between enzyme activity, free fatty acids (ρ = 0.823; P < 0.001), and surface tension (ρ = 0.55; P < 0.028). Correlations also exist with intensive care stay (ρ = 0.54; P = 0.001), PRISM-III24 (ρ = 0.79; P< 0.001), duration of ventilation (ρ = 0.53; P = 0.002), and oxygen therapy (ρ = 0.54; P = 0.001). Conclusions Secretory phospholipase A2 activity is raised in pediatric ARDS and constituted of four subtypes. Enzyme correlates with some inflammatory mediators, surface tension, and major clinical outcomes. Secretory

  17. Role of Inhaled Nitric Oxide in the Management of Severe Acute Respiratory Distress Syndrome

    PubMed Central

    Hunt, Juliette Lucinda; Bronicki, Ronald A.; Anas, Nick

    2016-01-01

    To date, there have been several systematic reviews with meta-analysis that have shown no reduction in mortality with the use of inhaled nitric oxide (iNO) in patients with acute respiratory distress syndrome (ARDS). Importantly, these reports fail to make a distinction between the pediatric and adult patient. The number of adult patients in these reviews are far greater than the number of pediatric patients, which makes it difficult to interpret the data regarding the role of iNO on the pediatric population. Extrapolating data from the adult population to the pediatric population is complicated as we know that physiology and the body’s response to disease can be different between adult and pediatric patients. iNO has been demonstrated to improve outcomes in term and near-term infants with hypoxic respiratory failure associated with pulmonary hypertension. Recently, Bronicki et al. published a prospective randomized control trial investigating the impact of iNO on the pediatric patient population with acute respiratory failure. In this study, a benefit of decreased duration of mechanical ventilation and an increased rate of ECMO-free survival was demonstrated in patients who were randomized to receiving iNO, suggesting that there may be benefit to the use of iNO in pediatric ARDS (PARDS) that has not been demonstrated in adults. iNO has repeatedly been shown to transiently improve oxygenation in all age groups, and yet neonates and pediatric patients have shown improvement in other outcomes that have not been seen in adults. The mechanism that explains improvement with the use of iNO in these patient populations are not well understood but does not appear to be solely a result of sustained improvement in oxygenation. There are physiologic studies that suggest alternative mechanisms for explaining the positive effects of iNO, such as platelet aggregation inhibition and reduction in systemic inflammation. Hence, the role of iNO by various mechanisms and in various

  18. Evaluation of the Role of Postnatal Ambroxol in the Prevention and Treatment of Respiratory Distress Syndrome in Preterm Neonates

    PubMed Central

    Elsayed, Hesham F; Elkhaiouby, Muhammed I; Elsharkawey, Sunia M; Elnemr, Muna A

    2006-01-01

    Objective: to study the effect of the postnatal administration of Ambroxol in the prevention of respiratory distress syndrome in preterm neonates at risk and on the severity of the disease in those neonates already suffering from it. Methods: the study was a randomized clinical trial performed on 120 preterm neonates admitted to the neonatal unit of the Suez Canal University Hospital, Egypt, with gestational age of 28 to 34 weeks. It was performed in the period from September 2001 through March 2003. Half of the enrolled neonates received intravenous ambroxol (20 mg/kg/d), while the control group received the routine management of prematurity and a placebo. Results: Ambroxol decreased the incidence of Respiratory Distress Syndrome (RDS), improved the gas exchange, and decreased Continious Positive Airway pressure (CPAP) pressure, the length of mechanical ventilation and also the mortality rate. Conclusion: the study concluded that Ambroxol reduced the incidence of this disease in preterm neonates at risk of developing it, and improved the clinical course of RDS. PMID:21748134

  19. [Moebius syndrome due to the use of misoprostol. Case report].

    PubMed

    Sánchez, Otto; Guerra, Dania

    2003-06-01

    We report a patient affected with Moebius Syndrome (OMIM 157900) due to the use of misoprostol during the first trimester of the pregnancy, when abortion was intended twice using this drug, vias vaginal (600 mg) and oral (900 mg), with failure to induce abortion on both occasions. Since the use of misoprostol for abortion, without any medical indication or supervision, appears to be rather frequent in our population and since there are reports of severe malformations in children born after failed intents of abortion with this medication, it is necessary to alert the medical community and the population in general about the teratogenic risks of this drug.

  20. hypertensive intracranial bleed due to mid aortic syndrome.

    PubMed

    Poovazhagi, Varadarajan; Pauline, Leema; Balakrishnan, N

    2014-03-01

    The authors describe an 11-y-old child with intracranial bleed due to malignant hypertension. Child presented with hypertension, right hemiparesis, feeble femoral pulses and lower limb blood pressure less than the upper limb. CT angiogram revealed narrowing of the abdominal aorta with thinned out left renal artery and hypoplasia of the left kidney. A diagnosis of Mid aortic syndrome was arrived at. CT brain revealed left ganglio capsular bleed. Child was treated with antihypertensives and steriods in view of suspected Takayasu arteritis. Child recovered with minimal hemiparesis and is being followed up.

  1. Flushing syndrome due to mahimahi (scombroid fish) poisoning.

    PubMed

    Kim, R

    1979-08-01

    Scombroid fish poisoning, one of the most common adverse reactions to fish, is also probably one of the most common causes of a flushing syndrome. The reaction usually involves fishes of the Scombridae family but, in Hawaii, the reaction is most often due to mahimahi (Coryphaena hippurus). Onset of the reaction is usually abrupt and commonly associated with a prominent flush resembling a sunburn. Headache, tachye to a toxin with histamine-like properties, which is formed because improper refrigeration enables endogenous bacteria to decarboxylate histidine normally present in dark-meat fishes. Symptoms are usually promptly relieved by parenteral antihistamine therapy.

  2. Hoarseness of voice, respiratory distress and dysphagia due to giant primary posterior mediastinal ectopic goitre: a rare clinical entity.

    PubMed

    Chaudhry, Ikram Ulhaq; Cheema, Ahsan Iqbal; AlShamasi, Zahra; Mutairi, Hadi

    2016-04-25

    Primary posterior mediastinal ectopic goitre is an extremely rare entity; we report a case of a 28-year-old man who presented with dysphagia, respiratory distress and hoarseness of voice, gradually worsening over a period of 3 months. CT scan of the thorax revealed a giant posterior mediastinal ectopic goitre. The mass was removed through a right posterolateral thoracotomy. The patient's symptoms, respiratory distress and dysphagia disappeared immediately after surgery while his voice gradually returned to normal after 6 weeks.

  3. High-frequency oscillatory ventilation is an effective treatment for severe pediatric acute respiratory distress syndrome with refractory hypoxemia

    PubMed Central

    Guo, Yu-Xiong; Wang, Zhao-Ni; Li, Ya-Ting; Pan, Li; Yang, Li-Fen; Hu, Yan; Sun, Yue-Yu; Cai, Liang-Ming; Chen, Zhuang-Gui

    2016-01-01

    Background and purpose Early or primary application of high-frequency oscillatory ventilation (HFOV) has been recently suggested not to offer benefit to patients with acute respiratory distress syndrome (ARDS). However, the rescue effects of HFOV on severe pediatric acute respiratory distress syndrome (PARDS) with hypoxemia refractory to conventional mechanical ventilation (CMV) remain unclear. This study aimed to determine whether severe PARDS children would benefit from HFOV when oxygenation deteriorated on CMV and to identify any potential risk factors related to mortality. Patients and methods In a retrospective and observational study, 48 children with severe PARDS between January 2009 and July 2015 were divided into two groups: 26 in HFOV group and 22 in CMV group. Data regarding demographic, underlying conditions, arterial blood gases and clinical outcomes were collected and analyzed. Results The arterial partial pressure of oxygen (PaO2)/fraction of inspiration oxygen (FiO2) ratio and PaO2 improved significantly during HFOV, whereas arterial partial pressure of carbon dioxide (PaCO2) and oxygenation index decreased. There was no statistical difference in the in-hospital mortality between the groups (P=0.367). The odds ratio of survival in HFOV group was 2.74 (95% confidence interval 0.52 to 14.58, P=0.237). The pediatric intensive care unit length of stay and total ventilation duration were longer in HFOV group (P=0.048 and P=0.000, respectively). Vasoactive agents were used more frequently in HFOV group (P=0.007). The incidence of new air leak was similar between the two groups (P=0.674). The presence of multiple organ dysfunction syndrome and heavier body weight were identified as predictors of mortality in the HFOV group (P=0.006 and P=0.020, respectively). Conclusion HFOV as an efficient alternative therapy could significantly improve hypoxemia and promote CO2 removal in severe PARDS children when oxygenation progressively worsens on CMV. PMID:27799777

  4. Mass Spectrometry-based Proteomics in Acute Respiratory Distress Syndrome: A Powerful Modality for Pulmonary Precision Medicine

    PubMed Central

    Xu, Xue-Feng; Dai, Hua-Ping; Li, Yan-Ming; Xiao, Fei; Wang, Chen

    2016-01-01

    Objective: Acute respiratory distress syndrome (ARDS) is an acute and lethal clinical syndrome that is characterized by hypoxemic respiratory failure and diffuse alveolar inflammatory damage. This review aimed to search and discuss the mass spectrometry (MS)-based proteomic studies on different subsets of ARDS patients. Data Sources: Original research articles were collected from the PubMed database published in English up to December 2015. Study Selection: The literature search was done using the term “(acute lung injury OR acute respiratory distress syndrome) AND (proteomics OR proteome OR mass spectrum OR differential in-gel electrophoresis OR two-dimensional polyacrylamide gel electrophoresis)”. Related original research articles were included and were carefully analyzed. Results: Eight original proteomic researches on ARDS patients were found. The common proteomic modalities were two-dimensional (2D) high-performance liquid chromatography-based electronic spray ion-MS/MS and 2D-polyacrylamide gel electrophoresis/differential in-gel electrophoresis-based matrix-assisted laser desorption ionization-time of flight/MS. They compared the proteome between ARDS patients and normal controls and analyzed the dynamic changes of proteome at different ARDS stages or severity. The disturbed proteome in ARDS patients includes plasma acute-phase proteins, inflammatory/immune-associated proteins, and coagulation proteins. Conclusions: Although several previous studies have provided some useful information about the lung proteome in ARDS patients and gained several interesting disease-associated biomarkers, clinical proteomic studies in ARDS patients are still in the initial stage. An increased cooperation is still needed to establish a global and faithful database containing disease-specific proteome from the largest ARDS subsets. PMID:27647196

  5. Effects of Xuanbai Chengqi decoction on lung compliance for patients with exogenous pulmonary acute respiratory distress syndrome

    PubMed Central

    Mao, Zhengrong; Wang, Haifeng

    2016-01-01

    Objective To observe the effects of Xuanbai Chengqi decoction on lung compliance for patients with exogenous pulmonary acute respiratory distress syndrome. Subjects and methods A total of 53 patients with exogenous pulmonary acute respiratory distress syndrome, who were admitted to the intensive care unit of the First Affiliated Hospital of Henan University of Traditional Chinese Medicine from March 2009 to February 2013, were selected. They were randomly divided into the treatment group (25 cases) and the control group (28 cases). Both the groups were treated with conventional treatment and lung-protective ventilation strategy; apart from these, enema therapy with Xuanbai Chengqi decoction was given to the treatment group. Meanwhile, static lung compliance, dynamic lung compliance, peak airway pressure, plateau pressure, and positive end-expiratory pressure (PEEP) for patients in both the groups were observed and recorded at 24, 48, and 72 hours after the drug was used. Moreover, variations in the duration of parenteral nutrition, incidence rate of complications, and case fatality rate in patients after treatment were recorded. Results For patients in the treatment group, at 48 and 72 hours after treatment, the static lung compliance and dynamic lung compliance were significantly higher than those in the control group, while plateau pressure, peak airway pressure, and PEEP were significantly lower than those before treatment. At the same time, PEEP for patients in the treatment group at 72 hours after treatment was remarkably lower than that in the control group, showing significant difference (P<0.05). The duration of parenteral nutrition in the treatment group was significantly shorter than that in the control group (P<0.05). Both the incidence rate and the fatality rate of complications, such as abdominal distension and ventilator-associated pneumonia, for patients in the treatment group were distinctly smaller than those in the control group (P<0

  6. A rat model of acute respiratory distress syndrome (ARDS): Part 1. Time dependency of histological and pathological changes.

    PubMed

    Germann, P G; Häfner, D

    1998-08-01

    The time course of histopathological changes in a rat lung lavage model of the acute respiratory distress syndrome (ARDS) was analyzed by sacrificing animals 10, 30, 60, 180, and 210 min after the last lung parenchyma lavage which was performed with physiological saline solution. This lavage depleted the lung from its natural surfactant resources leading into a pathophysiological cascade similar to that of the acute respiratory distress syndrome. Tracheotomized rats (12 animals per time point) were pressure-controlled ventilated (Siemens Servo Ventilator 900C) with 100% oxygen at a respiratory rate of 30 breaths/min, inspiration-expiration ratio of 1:2, peak inspiratory pressure of 28 cm H2O at positive end-expiratory pressure (PEEP) of 8 cm H2O. During the whole experimental period, the ventilation was not changed. Blood gases (partial arterial oxygen pressures [PaO2, mmHg] and partial arterial carbon dioxide pressures [PaCO2, mmHg]) were estimated before, directly after, and 10, 30, 60, 90, 120, 150, 180, and 210 min after the last lavage. For grading lung lavage-induced histopathological changes associated with the time-dependent development of ARDS, slides were coded and evaluated without any knowledge of the sacrifice time. A semiquantitative grading was performed with respect to the severity of the following parameters: hyaline membrane formation (HM), interstitial and intraalveolar edema edema (E), and margination and infiltration of polymorphonuclear neutrophil leukocytes (PMNL) into the lung alveoli. The severity of these parameters showed a time-dependent increase after the last lavage. This was accompanied by a time-dependent decrease in partial arterial oxygen pressure (PaO2) values during the early postlavage period (up to 30 min). Thereafter, PaO2 levels remained fairly stable. The severity of intraalveolar and/or perivascular hemorrhages within the lung was not time dependent. The rat lavage model shows similarities to the pathophysiological sequelae

  7. Prader-Willi Syndrome: Obesity due to Genomic Imprinting

    PubMed Central

    Butler, Merlin G

    2011-01-01

    Prader-Willi syndrome (PWS) is a complex neurodevelopmental disorder due to errors in genomic imprinting with loss of imprinted genes that are paternally expressed from the chromosome 15q11-q13 region. Approximately 70% of individuals with PWS have a de novo deletion of the paternally derived 15q11-q13 region in which there are two subtypes (i.e., larger Type I or smaller Type II), maternal disomy 15 (both 15s from the mother) in about 25% of cases, and the remaining subjects have either defects in the imprinting center controlling the activity of imprinted genes or due to other chromosome 15 rearrangements. PWS is characterized by a particular facial appearance, infantile hypotonia, a poor suck and feeding difficulties, hypogonadism and hypogenitalism in both sexes, short stature and small hands and feet due to growth hormone deficiency, mild learning and behavioral problems (e.g., skin picking, temper tantrums) and hyperphagia leading to early childhood obesity. Obesity is a significant health problem, if uncontrolled. PWS is considered the most common known genetic cause of morbid obesity in children. The chromosome 15q11-q13 region contains approximately 100 genes and transcripts in which about 10 are imprinted and paternally expressed. This region can be divided into four groups: 1) a proximal non-imprinted region; 2) a PWS paternal-only expressed region containing protein-coding and non-coding genes; 3) an Angelman syndrome region containing maternally expressed genes and 4) a distal non-imprinted region. This review summarizes the current understanding of the genetic causes, the natural history and clinical presentation of individuals with PWS. PMID:22043168

  8. Poorly Controlled Congenital Hypothyroidism due to an Underlying Allgrove Syndrome

    PubMed Central

    van Tellingen, V.; Finken, M.J.J.; Israëls, J.; Hendriks, Y.M.C.; Kamp, G.A.; van Santen, H.M.

    2016-01-01

    Background Congenital hypothyroidism of thyroidal origin (CHT) is a common disorder in pediatric endocrinology practices, which can be difficult to manage. Elevated thyrotropin (TSH) concentrations are in the great majority of cases explained by poor compliance to levothyroxine therapy. Methods Case description. Results We present a boy with CHT, with 2 heterozygous mutations in the TSH receptor gene, who showed persistently elevated TSH concentrations and psychomotor retardation, initially misinterpreted as malcompliance. At the age of 4 years, he was diagnosed with adrenal insufficiency, wherefore a broad diagnostic search was initiated. After the start of glucocorticoid replacement therapy, his TSH normalized and the levothyroxine could be lowered. At the age of 6 years, his TSH increased again, this time caused by malabsorption of levothyroxine due to esophageal achalasia. In retrospect, alacrima was also present and the diagnosis of Allgrove syndrome was genetically confirmed. The CHT was considered a separate disease entity. Conclusions In case of persistently elevated TSH levels in children with CHT, causes other than noncompliance must be considered. Second, in establishing the cause of adrenal insufficiency, specific symptoms, such as alacrima, are easily overlooked. Third, Allgrove syndrome is a rare disorder, in which diagnostic delay can lead to potentially life-threatening complications. PMID:27255745

  9. [Intermittent focal cerebral ischemia in hypotension due to pacemaker syndrome].

    PubMed

    Hagendorff, A; Pizzulli, L; Dettmers, C; Block, A; Omran, H; Hartmann, A; Manz, M; Lüderitz, B

    1994-12-01

    A pacemaker syndrome manifested as transient sensoric aphasia in a 68-year-old woman with a VVI-pace-maker implanted after SA-block. The attack occurred during long-term blood pressure recording and Holter monitoring. Borderline hypotension was documented during ventricular pacing which induced a retrograde excitation of the atrium. Clinical investigations excluded any intracranial abnormality, any source of embolism or stenosis of extra- and intracranial cerebral arteries. Cerebral blood flow measurements revealed a significant increase during pacing at elevated heart rate. Therefore, a device for AV-sequential pacing was implanted and basic pacing rate was elevated. The present case report indicates that focal and not only global cerebral ischemia can be produced by an impairment of systemic hemodynamics due to hypotension and a pacemaker syndrome. Improvement of cerebral blood flow during pacing is an unexpected finding contrasting with the concept of autoregulation. In addition, pacemaker implantation should be discussed in patients with transient cerebral perfusion deficits if an improvement of cerebral blood flow is documented along with rising heart rate.

  10. The cyclin-dependent kinase inhibitor AT7519 accelerates neutrophil apoptosis in sepsis-related acute respiratory distress syndrome

    PubMed Central

    Felton, Jennifer M; Robb, Calum T; Craven, Thomas; Kipari, Tiina; Walsh, Timothy S; Haslett, Christopher; Kefala, Kallirroi; Rossi, Adriano G; Lucas, Christopher D

    2017-01-01

    Acute respiratory distress syndrome (ARDS) is a neutrophil-dominant disorder with no effective pharmacological therapies. While the cyclin-dependent kinase inhibitor AT7519 induces neutrophil apoptosis to promote inflammation resolution in preclinical models of lung inflammation, its potential efficacy in ARDS has not been examined. Untreated peripheral blood sepsis-related ARDS neutrophils demonstrated prolonged survival after 20 hours in vitro culture. AT7519 was able to override this phenotype to induce apoptosis in ARDS neutrophils with reduced expression of the pro-survival protein Mcl-1. We demonstrate the first pharmacological compound to induce neutrophil apoptosis in sepsis-related ARDS, highlighting cyclin-dependent kinase inhibitors as potential novel therapeutic agents. PMID:27965411

  11. Acute respiratory distress syndrome and chemical burns after exposure to chlorine-containing bleach: a case report

    PubMed Central

    Shin, Hong-Joon; Chang, Jin-Sun; Ahn, Seong; Kim, Tae-Ok; Park, Cheol-Kyu; Lim, Jung-Hwan; Oh, In-Jae; Kim, Yu-Il; Lim, Sung-Chul; Kim, Young-Chul

    2017-01-01

    Chlorine-containing bleach can cause acute respiratory distress syndrome (ARDS) and chemical burns. However, simultaneous occurrence of the two conditions caused by this agent is very rare. We describe the case of a 74-year-old female who presented with shortness of breath and hemoptysis following accidental exposure to chlorine-containing bleach. She had second- to third-degree chemical burns on both buttocks and thighs, and received mechanical ventilation because of the development of ARDS. Mechanical ventilation was discontinued on day 6 of hospitalization because of the rapid improvement of hypoxemia, and the patient was transferred to another hospital for further management of the chemical burns on day 18. PMID:28203432

  12. Extracorporeal Membrane Oxygenation (ECMO) for Lung Injury in Severe Acute Respiratory Distress Syndrome (ARDS): Review of the Literature.

    PubMed

    Paolone, Summer

    2016-11-10

    Despite advances in mechanical ventilation, severe acute respiratory distress syndrome (ARDS) is associated with high morbidity and mortality rates ranging from 26% to 58%. Extracorporeal membrane oxygenation (ECMO) is a modified cardiopulmonary bypass circuit that serves as an artificial membrane lung and blood pump to provide gas exchange and systemic perfusion for patients when their own heart and lungs are unable to function adequately. ECMO is a complex network that provides oxygenation and ventilation and allows the lungs to rest and recover from respiratory failure while minimizing iatrogenic ventilator-induced lung injury. In critical care settings, ECMO is proven to improve survival rates and outcomes in patients with severe ARDS. This review defines severe ARDS; describes the ECMO circuit; and discusses recent research, optimal use of the ECMO circuit, limitations of therapy including potential complications, economic impact, and logistical factors; and discusses future research considerations.

  13. Bronchopleural Fistula Resolution with Endobronchial Valve Placement and Liberation from Mechanical Ventilation in Acute Respiratory Distress Syndrome: A Case Series

    PubMed Central

    2017-01-01

    Patients who have acute respiratory distress syndrome (ARDS) with persistent air leaks have worse outcomes. Endobronchial valves (EBV) are frequently deployed after pulmonary resection in noncritically ill patients to reduce and eliminate bronchopleural fistulas (BPFs) with persistent air leak (PAL). Information regarding EBV placement in mechanically ventilated patients with ARDS and high volume persistent air leaks is rare and limited to case reports. We describe three cases where EBV placement facilitated endotracheal extubation in patients with severe respiratory failure on prolonged mechanical ventilation with BPFs. In each case, EBV placement led to immediate resolution of PAL. We believe endobronchial valve placement is a safe method treating persistent air leak with severe respiratory failure and may reduce days on mechanical ventilation. PMID:28367339

  14. Hitch-hiker taken for a ride: an unusual cause of myocarditis, septic shock and adult respiratory distress syndrome

    PubMed Central

    Kushawaha, Anurag; Brown, Mark; Martin, Ismael; Evenhuis, Walther

    2013-01-01

    Rocky Mountain spotted fever (RMSF) is a serious tick-borne illness caused by Rickettsia rickettsii that is endemic in southeastern USA. Although RMSF has been described as causing the classic clinical triad of fever, headache and a characteristic rash, serious and potentially life-threatening manifestations can occur. Cardiopulmonary involvement, although infrequent, may occur with severe cases of RMSF. Rickettsial myocarditis is an uncommon occurrence. We present a case of a previously healthy 26-year-old man, who was hitch-hiking across the southeastern USA, with serologically proven RMSF causing adult respiratory distress syndrome, septic shock and myocarditis manifested by elevated cardiac enzymes and decrease in myocardial function. After treatment with antibiotics, the myocarditis resolved. Therefore, although unusual, clinicians should be aware of possible myocardial involvement in patients with appropriate tick-exposure histories or other clinical signs of RMSF. PMID:23314875

  15. Lugrandoside attenuates LPS-induced acute respiratory distress syndrome by anti-inflammation and anti-apoptosis in mice

    PubMed Central

    Li, Chengbao; Huang, Ying; Yao, Xueya; Hu, Baoji; Wu, Suzhen; Chen, Guannan; Lv, Xin; Tian, Fubo

    2016-01-01

    This study aimed to investigate the protective effects and specific mechanisms of lugrandoside (LG) on lipopolysaccharides (LPS)-induced acute respiratory distress syndrome (ARDS). LG is a novel phenylpropanoid glycoside with many biological properties, isolated from the culinary leaves of Digitalis lutea L. and Digitalis grandiflora Miller. The primary indicators to assess the lung injury were infiltration of inflammatory cells; pulmonary edema; expression of proinflammatory cytokines, cyclo-oxygenase 2, and intracellular adhesion molecule 1; activation of nuclear factor-κB pathways; and cellular apoptosis. The results showed that LG evidently alleviated the inflammatory response, decreased the apoptosis of alveolar macrophages, and improved the lung injury in mice with LPS-induced ARDS. In conclusion, LG improved LPS-induced ARDS by anti-inflammation and anti-apoptosis and might be a promising pharmacological therapy for ARDS. PMID:28078026

  16. Airway pressure release ventilation in morbidly obese surgical patients with acute lung injury and acute respiratory distress syndrome.

    PubMed

    Testerman, George M; Breitman, Igal; Hensley, Sarah

    2013-03-01

    Morbidly obese patients with body mass index greater than 40 kg/m(2) and respiratory failure requiring critical care services are increasingly seen in trauma and acute care surgical centers. Baseline respiratory pathophysiology including decreased pulmonary compliance with dependent atelectasis and abnormal ventilation-perfusion relationships predisposes these patients to acute lung injury (ALI) and adult respiratory distress syndrome (ARDS) as well as prolonged stays in the intensive care unit. Airway pressure release ventilation (APRV) is an increasingly used alternative mode for salvage therapy in patients with hypoxemic respiratory failure that also provides lung protection from ventilator-induced lung injury. APRV provides the conceptual advantage of an "open lung" approach to ventilation that may be extended to the morbidly obese patient population with ALI and ARDS. We discuss the theoretical benefits and a recent clinical experience of APRV ventilation in the morbidly obese patient with respiratory failure at a Level I trauma, surgical critical care, and acute care surgery center.

  17. Coracoid impingement syndrome due to intensive rock climbing training.

    PubMed

    Schöffl, Volker; Schneider, Hans; Küpper, Thomas

    2011-06-01

    Overuse and acute injuries to the upper body are common in rock climbing. Such injuries primarily affect the fingers; but shoulder problems are increasingly common, especially among more experienced and older climbers who climb at a high ability level. Such shoulder problems are often due to subacromial impingement, shoulder dislocations with bankart lesions, hyperlaxity, SLAP lesions or irritations of the long biceps tendon. In contrast to these known conditions, we describe a case of an ambitious female rock climber who trained intensively and developed a coracoid impingement caused by hypertrophied subscapularis tendon and muscle following sport-specific training. Diagnosis was made through clinical evaluation and confirmed by magnetic resonance tomography. Coracoid impingement syndrome is a less common cause of shoulder pain and occurs when the subscapularis tendon impinges between the coracoid and the lesser tuberosity of the humerus. The patient was treated successfully with a conservative therapy and returned to full activity within 6 weeks.

  18. Cavernous sinus syndrome due to osteochondromatosis in a cat.

    PubMed

    Perazzi, Anna; Bernardini, Marco; Mandara, Maria T; De Benedictis, Giulia M; De Strobel, Francesca; Zotti, Alessandro

    2013-12-01

    A 1-year-old sexually intact male Korat cat was referred for ophthalmological consultation due to anisocoria. Mydriasis with external ophthalmoplegia and absence of pupillary light responses in the right eye and nasofacial hypalgesia were seen. Cavernous sinus syndrome (CSS) was suspected. Bilateral deformities of the jaw and phalangeal bones, severe spinal pain and abnormal conformation of the lumbar spine were also present. Radiographic examination revealed several mineralised masses in the appendicular and axial skeleton, indicative of multiple cartilaginous exostoses. For further investigation of the CSS-related neurological deficits, the cat underwent computed tomography (CT) examination of the skull. CT images revealed a non-vascularised, calcified, amorphous mass originating from the right lateral skull base and superimposing on the sella turcica. Based on the severity of diffuse lesions and owing to the clinical signs of extreme pain, the cat was euthanased. A diffuse skeletal and intracranial osteochondromatosis was diagnosed histologically.

  19. Drug-induced hypersensitivity syndrome due to carbapenem antibiotics.

    PubMed

    Goto, Mizuki; Shimizu, Fumiaki; Takeo, Naoko; Okamoto, Osamu; Katagiri, Kazumoto; Ikewaki, Junji; Ogata, Masao; Kadota, Jun-ichi; Fujiwara, Sakuhei

    2010-04-01

    Drug-induced hypersensitivity syndrome (DIHS) is characterized by a serious adverse systemic reaction that usually appears after a 3-6-week exposure to certain drugs, for example, anticonvulsants. Many different precipitating factors have been reported, but the pathophysiology of DIHS remains unknown. However, reactivation of members of the human herpesvirus (HHV) family, and of HHV-6 in particular, has been reported in patients with DIHS. We report the case of a 64-year-old man who developed a generalized erythematous rash, fever, hepatic failure, lymphadenopathy and an increased number of atypical lymphocytes. In addition, reactivation of HHV-6 and cytomegalovirus (CMV) was demonstrated by real-time quantitative amplification by polymerase chain reaction. The patient was given a diagnosis of DIHS due to carbapenem antibiotics based on his clinical course, laboratory data, and results of lymphocyte-stimulation tests with various drugs. This is the first report, to our knowledge, of DIHS induced by carbapenem antibiotics.

  20. Functional Characterization of ATP-Binding Cassette Transporter A3 Mutations from Infants with Respiratory Distress Syndrome.

    PubMed

    Wambach, Jennifer A; Yang, Ping; Wegner, Daniel J; Heins, Hillary B; Kaliberova, Lyudmila N; Kaliberov, Sergey A; Curiel, David T; White, Frances V; Hamvas, Aaron; Hackett, Brian P; Cole, F Sessions

    2016-11-01

    Mutations in the ATP-binding cassette transporter A3 gene (ABCA3) result in severe neonatal respiratory distress syndrome and childhood interstitial lung disease. As most ABCA3 mutations are rare or private, determination of mutation pathogenicity is often based on results from in silico prediction tools, identification in unrelated diseased individuals, statistical association studies, or expert opinion. Functional biologic studies of ABCA3 mutations are needed to confirm mutation pathogenicity and inform clinical decision making. Our objective was to functionally characterize two ABCA3 mutations (p.R288K and p.R1474W) identified among term and late-preterm infants with respiratory distress syndrome with unclear pathogenicity in a genetically versatile model system. We performed transient transfection of HEK293T cells with wild-type or mutant ABCA3 alleles to assess protein processing with immunoblotting. We used transduction of A549 cells with adenoviral vectors, which concurrently silenced endogenous ABCA3 and expressed either wild-type or mutant ABCA3 alleles (p.R288K and p.R1474W) to assess immunofluorescent localization, ATPase activity, and organelle ultrastructure. Both ABCA3 mutations (p.R288K and p.R1474W) encoded proteins with reduced ATPase activity but with normal intracellular localization and protein processing. Ultrastructural phenotypes of lamellar body-like vesicles in A549 cells transduced with mutant alleles were similar to wild type. Mutant proteins encoded by ABCA3 mutations p.R288K and p.R1474W had reduced ATPase activity, a biologically plausible explanation for disruption of surfactant metabolism by impaired phospholipid transport into the lamellar body. These results also demonstrate the usefulness of a genetically versatile, human model system for functional characterization of ABCA3 mutations with unclear pathogenicity.

  1. Twelve-Month Follow-Up of Postmature and Respiratory Distress Syndrome Infants.

    ERIC Educational Resources Information Center

    Field, Tiffany

    A 12-month followup study of 151 infants (46 preterm respiratory disease syndrome--RDS--Ss, 46 postterm postmaturity syndrome Ss, and 59 normal term Ss) was conducted to ascertain any early intervention needs and early predictors on 1-year performance, and to formulate a cumulative risk index which would identify those infants who were continuing…

  2. Living with the unexplained: coping, distress, and depression among women with chronic fatigue syndrome and/or fibromyalgia compared to an autoimmune disorder.

    PubMed

    McInnis, Opal A; Matheson, Kimberly; Anisman, Hymie

    2014-01-01

    Chronic fatigue syndrome (CFS) and fibromyalgia are disabling conditions without objective diagnostic tests, clear-cut treatments, or established etiologies. Those with the disorders are viewed suspiciously, and claims of malingering are common, thus promoting further distress. It was hypothesized in the current study that levels of unsupportive social interactions and the coping styles used among those with CFS/fibromyalgia would be associated with perceived distress and depressive symptoms. Women with CFS/fibromyalgia (n=39), in fact, reported higher depression scores, greater perceived distress and more frequent unsupportive relationships than healthy women (n=55), whereas those with a chronic, but medically accepted illness comprising an autoimmune disorder (lupus erythematosus, multiple sclerosis, rheumatoid arthritis; n=28), displayed intermediate scores. High problem-focused coping was associated with low levels of depression and perceived distress in those with an autoimmune condition. In contrast, although CFS/fibromyalgia was also accompanied by higher depression scores and higher perceived distress, this occurred irrespective of problem-focused coping. It is suggested that because the veracity of ambiguous illnesses is often questioned, this might represent a potent stressor in women with such illnesses, and even coping methods typically thought to be useful in other conditions, are not associated with diminished distress among those with CFS/fibromyalgia.

  3. [Using non-invasive mask lung ventilation in cardiosurgical patients with acute respiratory distress syndrome].

    PubMed

    Eremenko, A A; Levikov, D I; Egorov, V M; Zorin, D E; Kolomiets, V Ia

    2004-01-01

    Twenty patients aged 33 to 71 (54 +/- 6) years (male - 13, female - 7) operated on the heart and main vessels were included in the case study. I.e. those patient were investigated, whose immediate postoperative results were complicated by the syndrome of multiple organ failure (SMOF) that developed due to different-etiology shock, huge blood loss and hemotransfusion or to the syndrome of acute postperfusion lung damage. NIMLV was made at the resolution stage of SMOF and ARDS after artificial pulmonary ventilation (APL) for as long as 5-7 days. The indications for extubation of patients were as follows: PaO2/FiO2 of 200 and more mm Hg, respiratory rate (RR) of less than 30 per min, respiratory volume of more than 6 ml/kg with pressure support at inspiration of less than 5 cm H2O and with the total pressure at the exhalation end of no more than 3 cm H2O. Mask ventilation sessions were started in a growing dyspnea of more than 26 per min, a decreased content of oxyhemoglobin in arterial blood (below 95% at oxygen inhalation of 10-15 l/min), involvement of auxiliary muscles in breathing and at subjective complaints of patients related with complicated breathing and with being short of air. The mask SIMV ventilation with a preset apparatus-aided rate of inhales of 2-6/min, with Bi-PAP and PSV inhale pressure of 15 cm/ H2O and with PEEP of 3-5 cm/ H2O was made by 40-120 min sessions; the number of IFMLV sessions ranged from 6 to 22/patient, mean - 11 +/- 1.1 h. The total IFMLV duration was 10.7 +/- 1.1 h. The need for respiratory support persisted for 4-6 days after extubation. In 18 (90%) of 20 patients, the mask pulmonary ventilation resolved the respiratory insufficiency. Two (10%) patients were reintubated because of progressing multiorgan failure and because of obturation of the left main bronchus. A questioning of patients on the comfort degree of mask ventilation denoted the Flow-by triggering to be by far better tolerated by patients versus the pressure

  4. Hyperimmunoglobulin E syndrome with persistent vertebral osteomyelitis due to methicillin-resistant Staphylococcus aureus

    PubMed Central

    Wong, San S.; Smith, Peter R.; Ayaz, Asim; Sepkowitz, Douglas

    2014-01-01

    We report a case of vertebral osteomyelitis due to methicillin-resistant Staphylococcus aureus (MRSA) with a slowly progressive, relatively asymptomatic course in a young woman with suspected hyperimmunoglobulin E syndrome (Job's syndrome). PMID:26839768

  5. Genetics and Gene Expression Involving Stress and Distress Pathways in Fibromyalgia with and without Comorbid Chronic Fatigue Syndrome

    PubMed Central

    Light, Kathleen C.; White, Andrea T.; Tadler, Scott; Iacob, Eli; Light, Alan R.

    2012-01-01

    In complex multisymptom disorders like fibromyalgia syndrome (FMS) and chronic fatigue syndrome (CFS) that are defined primarily by subjective symptoms, genetic and gene expression profiles can provide very useful objective information. This paper summarizes research on genes that may be linked to increased susceptibility in developing and maintaining these disorders, and research on resting and stressor-evoked changes in leukocyte gene expression, highlighting physiological pathways linked to stress and distress. These include the adrenergic nervous system, the hypothalamic-pituitary-adrenal axis and serotonergic pathways, and exercise responsive metabolite-detecting ion channels. The findings to date provide some support for both inherited susceptibility and/or physiological dysregulation in all three systems, particularly for catechol-O-methyl transferase (COMT) genes, the glucocorticoid and the related mineralocorticoid receptors (NR3C1, NR3C2), and the purinergic 2X4 (P2X4) ion channel involved as a sensory receptor for muscle pain and fatigue and also in upregulation of spinal microglia in chronic pain models. Methodological concerns for future research, including potential influences of comorbid clinical depression and antidepressants and other medications, on gene expression are also addressed. PMID:22110941

  6. Targeting Neutrophils to Prevent Malaria-Associated Acute Lung Injury/Acute Respiratory Distress Syndrome in Mice

    PubMed Central

    Soeiro-Pereira, Paulo V.; Gomes, Eliane; Neto, Antonio Condino; D' Império Lima, Maria R.; Alvarez, José M.; Portugal, Silvia; Epiphanio, Sabrina

    2016-01-01

    Malaria remains one of the greatest burdens to global health, causing nearly 500,000 deaths in 2014. When manifesting in the lungs, severe malaria causes acute lung injury/acute respiratory distress syndrome (ALI/ARDS). We have previously shown that a proportion of DBA/2 mice infected with Plasmodium berghei ANKA (PbA) develop ALI/ARDS and that these mice recapitulate various aspects of the human syndrome, such as pulmonary edema, hemorrhaging, pleural effusion and hypoxemia. Herein, we investigated the role of neutrophils in the pathogenesis of malaria-associated ALI/ARDS. Mice developing ALI/ARDS showed greater neutrophil accumulation in the lungs compared with mice that did not develop pulmonary complications. In addition, mice with ALI/ARDS produced more neutrophil-attracting chemokines, myeloperoxidase and reactive oxygen species. We also observed that the parasites Plasmodium falciparum and PbA induced the formation of neutrophil extracellular traps (NETs) ex vivo, which were associated with inflammation and tissue injury. The depletion of neutrophils, treatment with AMD3100 (a CXCR4 antagonist), Pulmozyme (human recombinant DNase) or Sivelestat (inhibitor of neutrophil elastase) decreased the development of malaria-associated ALI/ARDS and significantly increased mouse survival. This study implicates neutrophils and NETs in the genesis of experimentally induced malaria-associated ALI/ARDS and proposes a new therapeutic approach to improve the prognosis of severe malaria. PMID:27926944

  7. Psychopathological manifestations of joint hypermobility and joint hypermobility syndrome/ Ehlers-Danlos syndrome, hypermobility type: The link between connective tissue and psychological distress revised.

    PubMed

    Sinibaldi, Lorenzo; Ursini, Gianluca; Castori, Marco

    2015-03-01

    Psychological distress is a known feature of generalized joint hypermobility (gJHM), as well as of its most common syndromic presentation, namely Ehlers-Danlos syndrome, hypermobility type (a.k.a. joint hypermobility syndrome - JHS/EDS-HT), and significantly contributes to the quality of life of affected individuals. Most published articles dealt with the link between gJHM (or JHS/EDS-HT) and anxiety-related conditions, and a novel generation of studies is emerging aimed at investigating the psychopathologic background of such an association. In this paper, literature review was carried out with a semi-systematic approach spanning the entire spectrum of psychopathological findings in gJHM and JHS/EDS-HT. Interestingly, in addition to the confirmation of a tight link between anxiety and gJHM, preliminary connections with depression, attention deficit (and hyperactivity) disorder, autism spectrum disorders, and obsessive-compulsive personality disorder were also found. Few papers investigated the relationship with schizophrenia with contrasting results. The mind-body connections hypothesized on the basis of available data were discussed with focus on somatotype, presumed psychopathology, and involvement of the extracellular matrix in the central nervous system. The hypothesis of positive Beighton score and alteration of interoceptive/proprioceptive/body awareness as possible endophenotypes in families with symptomatic gJHM or JHS/EDS-HT is also suggested. Concluding remarks addressed the implications of the psychopathological features of gJHM and JHS/EDS-HT in clinical practice.

  8. Burning-Feet Syndrome: Case Due to Malabsorption and Responding to Riboflavine

    PubMed Central

    Lai, C. S.; Ransome, G. A.

    1970-01-01

    A woman with the burning-feet syndrome was found on investigation to have malabsorption. The syndrome responded rapidly to intramuscular injections of 6 mg. of riboflavine daily. It is suggested that deficiency of this substance, due to malabsorption and aggravated by a defective diet and repeated pregnancies, was responsible for the syndrome in this case. PMID:5440597

  9. Renal dysfunction due to hydronephrosis by SAPHO syndrome: a case report

    PubMed Central

    Kakoki, Katsura; Miyata, Yasuyoshi; Enokizono, Mikako; Uetani, Masataka; Sakai, Hideki

    2015-01-01

    Key Clinical Message Synovitis, Acne, Pustulosis, Hyperostosis, Osteitis (SAPHO) syndrome shows varied pathological symptoms. This is the first report of hydronephrosis due to the mechanical compression of bilateral ureters as a result of SAPHO syndrome. From our experience, MRI is the most useful imaging examination to check the upper urinary tract in SAPHO syndrome. PMID:26331013

  10. Renal dysfunction due to hydronephrosis by SAPHO syndrome: a case report.

    PubMed

    Kakoki, Katsura; Miyata, Yasuyoshi; Enokizono, Mikako; Uetani, Masataka; Sakai, Hideki

    2015-08-01

    Synovitis, Acne, Pustulosis, Hyperostosis, Osteitis (SAPHO) syndrome shows varied pathological symptoms. This is the first report of hydronephrosis due to the mechanical compression of bilateral ureters as a result of SAPHO syndrome. From our experience, MRI is the most useful imaging examination to check the upper urinary tract in SAPHO syndrome.

  11. Diagnostic delay of pulmonary tuberculosis in patients with acute respiratory distress syndrome associated with aspiration pneumonia: Two case reports and a mini-review from Japan.

    PubMed

    Nakao, Makoto; Sone, Kazuki; Kagawa, Yusuke; Kurokawa, Ryota; Sato, Hidefumi; Kunieda, Takefumi; Muramatsu, Hideki

    2016-08-01

    Diagnosing active tuberculosis in elderly patients presents problems due to nonspecific symptoms and complications such as aspiration pneumonia. The current study presents two cases of pulmonary tuberculosis with bilateral pulmonary infiltrates associated with aspiration pneumonia. The two elderly patients developed acute respiratory distress syndrome as a result of aspiration pneumonia. The diagnoses of pulmonary tuberculosis were delayed in both cases, as the patients were diagnosed with active tuberculosis following discharge from hospital. The sputum test for acid-fast bacillus at the time of administration was smear-negative/culture-positive in these patients. They were treated with isoniazid, rifampicin and ethambutol, and nosocomial transmission of tuberculosis from these patients was not reported. The number of elderly patients with aspiration pneumonia is predicted to increase rapidly, and aspiration pneumonia combined with pulmonary tuberculosis is a major medical and healthcare concern in Japan. The present study concludes that physicians should always consider the complication of pulmonary tuberculosis when treating pneumonia patients, in particular in treating elderly patients with pulmonary infiltrates.

  12. Diagnostic delay of pulmonary tuberculosis in patients with acute respiratory distress syndrome associated with aspiration pneumonia: Two case reports and a mini-review from Japan

    PubMed Central

    Nakao, Makoto; Sone, Kazuki; Kagawa, Yusuke; Kurokawa, Ryota; Sato, Hidefumi; Kunieda, Takefumi; Muramatsu, Hideki

    2016-01-01

    Diagnosing active tuberculosis in elderly patients presents problems due to nonspecific symptoms and complications such as aspiration pneumonia. The current study presents two cases of pulmonary tuberculosis with bilateral pulmonary infiltrates associated with aspiration pneumonia. The two elderly patients developed acute respiratory distress syndrome as a result of aspiration pneumonia. The diagnoses of pulmonary tuberculosis were delayed in both cases, as the patients were diagnosed with active tuberculosis following discharge from hospital. The sputum test for acid-fast bacillus at the time of administration was smear-negative/culture-positive in these patients. They were treated with isoniazid, rifampicin and ethambutol, and nosocomial transmission of tuberculosis from these patients was not reported. The number of elderly patients with aspiration pneumonia is predicted to increase rapidly, and aspiration pneumonia combined with pulmonary tuberculosis is a major medical and healthcare concern in Japan. The present study concludes that physicians should always consider the complication of pulmonary tuberculosis when treating pneumonia patients, in particular in treating elderly patients with pulmonary infiltrates. PMID:27446284

  13. Creutzfeldt-Jakob-Like Syndrome due to Hypercalcemic Encephalopathy.

    PubMed

    Rösche, Johannes; Sieveking, Catharina; Kampf, Christina; Benecke, Reiner

    2015-10-01

    Hypercalcemia can cause a subacute syndrome of progressive dementia and marked changes in the electroencephalogram (EEG). We report a case of iatrogenic hypercalcemia with a close correlation between the clinical course and the EEG changes. A 73-year-old woman presented with a subacute syndrome of progressive dementia and bursts of 1.5 to 2 Hz intermittent rhythmic delta activity superimposed on a low-voltage background activity in the EEG. Clinical and EEG abnormalities rapidly resolved after normalization of serum calcium levels. As part of the diagnostic workup of a subacute progressive dementia, a serum calcium level and an EEG should be obtained to detect a Creutzfeldt-Jakob like syndrome in hypercalcemia. Unlike in Creutzfeldt-Jakob disease, and Creutzfeldt-Jakob-like syndrome induced by lithium intoxication, there are rarely myoclonic jerks and periodic discharges in hypercalcemic encephalopathy.

  14. [Syncope due to parapharyngeal space lesions syncope-syndrome].

    PubMed

    Córdoba López, A; Torrico Román, P; Inmaculada Bueno Alvarez-Arenas, M; Monterrubio Villar, J; Corcho Sánchez, G

    2001-05-01

    The clinic association between tumors in the neck and syncopal attacks has been well documented but uncommon. The pathogenesis of this type of syncope has been attributed to an specific sinus node disfunction and several diseases that affect pacemaker activity, cardiac output and blood supply to the brain are able to produce it. The most common reflex cardiovascular sindromes linked to the IX nerve are carotid sinus syndrome and glossopharyngeal neuralgia-asystole syndrome. Some patients with a pathological involvement of the parapharyngeal space also suffers from syncope but the clinical picture of these patients differs so greatly from the other two syndromes, which suggests specific aetiological, clinical and therapeutical features so the term "parapharyngeal space lesiones syncope-syndrome" has been proposed for this entity. We report a case of a patient with a neck tumor in the parapharyngeal space with syncope as the major symptom.

  15. Burning mouth syndrome due to herpes simplex virus type 1.

    PubMed

    Nagel, Maria A; Choe, Alexander; Traktinskiy, Igor; Gilden, Don

    2015-04-01

    Burning mouth syndrome is characterised by chronic orofacial burning pain. No dental or medical cause has been found. We present a case of burning mouth syndrome of 6 months duration in a healthy 65-year-old woman, which was associated with high copy numbers of herpes simplex virus type 1 (HSV-1) DNA in the saliva. Her pain resolved completely after antiviral treatment with a corresponding absence of salivary HSV-1 DNA 4 weeks and 6 months later.

  16. Sleep after critical illness: Study of survivors of acute respiratory distress syndrome and systematic review of literature

    PubMed Central

    Dhooria, Sahajal; Sehgal, Inderpaul Singh; Agrawal, Anshu Kumar; Agarwal, Ritesh; Aggarwal, Ashutosh Nath; Behera, Digambar

    2016-01-01

    Background and Aims: This study aims to evaluate the sleep quality, architecture, sleep-related quality of life, and sleep-disordered breathing (SDB) in acute respiratory distress syndrome (ARDS) survivors early after discharge. Materials and Methods: In this prospective, observational study, consecutive patients with ARDS discharged from the Intensive Care Unit (ICU) underwent evaluation with Epworth sleepiness scale (ESS), Pittsburgh Sleep Quality Index (PSQI), Functional Outcomes of Sleep Questionnaire (FOSQ), and overnight polysomnography. Patients having one or more of the following characteristics were classified as having abnormal sleep: ESS>10, PSQI>5, FOSQ <17.9, apnea–hypopnea index (AHI) ≥5, or AHI during rapid eye movement (REM) sleep ≥5. Results: Twenty patients (median interquartile range [IQR] age of 24 [22–28] years, 11 [55%] females) were included in the study. Acute febrile illness of unknown etiology with multi-organ dysfunction syndrome was the most common underlying etiology for ARDS. The median (IQR) PaO2/FiO2 ratio and APACHE II scores on admission were 176 (151–191.5) and 14 (14–16), respectively. The median (IQR) duration of stay in the ICU was 10 days (7.3–19.5). The overall sleep efficiency (median [IQR], 54% [32.3–65.4%]) was poor. None of the patients had ESS>10, seven (35%) had global PSQI>5 and one had FOSQ <17.9. Ten (50%) patients had at least one characteristic that suggested abnormal sleep (4 insomnia, 2 central sleep apnea, 1 obstructive sleep apnea, 1 REM-SDB, and 2 with a high PSQI, but no specific sleep abnormality). Conclusions: Sleep disturbances are common in ARDS survivors early after discharge from the ICU. PMID:27390455

  17. Extremes of Interferon-Stimulated Gene Expression Associate with Worse Outcomes in the Acute Respiratory Distress Syndrome

    PubMed Central

    Nick, Jerry A.; Caceres, Silvia M.; Kret, Jennifer E.; Poch, Katie R.; Strand, Matthew; Faino, Anna V.; Nichols, David P.; Saavedra, Milene T.; Taylor-Cousar, Jennifer L.; Geraci, Mark W.; Burnham, Ellen L.; Fessler, Michael B.; Suratt, Benjamin T.; Abraham, Edward; Moss, Marc; Malcolm, Kenneth C.

    2016-01-01

    Acute Respiratory Distress Syndrome (ARDS) severity may be influenced by heterogeneity of neutrophil activation. Interferon-stimulated genes (ISG) are a broad gene family induced by Type I interferons, often as a response to viral infections, which evokes extensive immunomodulation. We tested the hypothesis that over- or under-expression of immunomodulatory ISG by neutrophils is associated with worse clinical outcomes in patients with ARDS. Genome-wide transcriptional profiles of circulating neutrophils isolated from patients with sepsis-induced ARDS (n = 31) and healthy controls (n = 19) were used to characterize ISG expression. Hierarchical clustering of expression identified 3 distinct subject groups with Low, Mid and High ISG expression. ISG accounting for the greatest variability in expression were identified (MX1, IFIT1, and ISG15) and used to analyze a prospective cohort at the Colorado ARDS Network site. One hundred twenty ARDS patients from four urban hospitals were enrolled within 72 hours of initiation of mechanical ventilation. Circulating neutrophils were isolated from patients and expression of ISG determined by PCR. Samples were stratified by standard deviation from the mean into High (n = 21), Mid, (n = 82) or Low (n = 17) ISG expression. Clinical outcomes were compared between patients with High or Low ISG expression to those with Mid-range expression. At enrollment, there were no differences in age, gender, co-existing medical conditions, or type of physiologic injury between cohorts. After adjusting for age, race, gender and BMI, patients with either High or Low ISG expression had significantly worse clinical outcomes than those in the Mid for number of 28-day ventilator- and ICU-free days (P = 0.0006 and 0.0004), as well as 90-day mortality and 90-day home with unassisted breathing (P = 0.02 and 0.004). These findings suggest extremes of ISG expression by circulating neutrophils from ARDS patients recovered early in the syndrome are associated

  18. Extracorporeal membrane oxygenation improves survival in a novel 24-hour pig model of severe acute respiratory distress syndrome

    PubMed Central

    Araos, Joaquín; Alegría, Leyla; García, Patricio; Damiani, Felipe; Tapia, Pablo; Soto, Dagoberto; Salomon, Tatiana; Rodriguez, Felipe; Amthauer, Macarena; Erranz, Benjamín; Castro, Gabriel; Carreño, Pamela; Medina, Tania; Retamal, Jaime; Cruces, Pablo; Bugedo, Guillermo; Bruhn, Alejandro

    2016-01-01

    Extracorporeal membrane oxygenation (ECMO) is increasingly being used to treat severe acute respiratory distress syndrome (ARDS). However, there is limited clinical evidence about how to optimize the technique. Experimental research can provide an alternative to fill the actual knowledge gap. The purpose of the present study was to develop and validate an animal model of acute lung injury (ALI) which resembled severe ARDS, and which could be successfully supported with ECMO. Eighteen pigs were randomly allocated into three groups: sham, ALI, and ALI + ECMO. ALI was induced by a double-hit consisting in repeated saline lavage followed by a 2-hour period of injurious ventilation. All animals were followed up to 24 hours while being ventilated with conventional ventilation (tidal volume 10 ml/kg). The lung injury model resulted in severe hypoxemia, increased airway pressures, pulmonary hypertension, and altered alveolar membrane barrier function, as indicated by an increased protein concentration in bronchoalveolar fluid, and increased wet/dry lung weight ratio. Histologic examination revealed severe diffuse alveolar damage, characteristic of ARDS. Veno-venous ECMO was started at the end of lung injury induction with a flow > 60 ml/kg/min resulting in rapid reversal of hypoxemia and pulmonary hypertension. Mortality was 0, 66.6 and 16.6% in the SHAM, ALI and ALI + ECMO groups, respectively (p < 0.05). This is a novel clinically relevant animal model that can be used to optimize the approach to ECMO and foster translational research in extracorporeal lung support. PMID:27398166

  19. A tree-matching algorithm: Application to airways in CT images of subjects with the acute respiratory distress syndrome.

    PubMed

    Morales Pinzón, Alfredo; Hernández Hoyos, Marcela; Richard, Jean-Christophe; Flórez-Valencia, Leonardo; Orkisz, Maciej

    2017-01-01

    To match anatomical trees such as airways, we propose a graph-based strategy combined with an appropriate distance function. The strategy was devised to cope with topological and geometrical differences that may arise between trees corresponding to the same subject, but extracted from images acquired in different conditions. The proposed distance function, called father/family distance, combines topological and geometrical information in a single measure, by calculating a sum of path-to-path distances between sub-trees of limited extent. To use it successfully, the branches of these sub-trees need to be brought closer, which is obtained by successively translating the roots of these sub-trees prior to their actual matching. The work herein presented contributes to a study of the acute respiratory distress syndrome, where a series of pulmonary CT images from the same subject is acquired at varying settings (pressure and volume) of the mechanical ventilation. The method was evaluated on 45 combinations of synthetic trees, as well as on 15 pairs of real airway trees: nine corresponding to end-expiration and end-inspiration with the same pressure, and six corresponding to end-inspiration with significantly different pressures. It achieved a high rate of successful matches with respect to a hand-made reference containing a total of 2391 matches in real data: sensitivity of 94.3% and precision of 92.8%, when using the basic parameter settings of the algorithm.

  20. Deleted in malignant brain tumors 1 protein is a potential biomarker of acute respiratory distress syndrome induced by pneumonia.

    PubMed

    Ren, Shan; Chen, Xia; Jiang, Li; Zhu, Bo; Jiang, Qi; Xi, Xiuming

    2016-09-23

    Acute respiratory distress syndrome (ARDS) is associated with high mortality and morbidity. Early diagnosis and risk stratification in patients with ARDS should improve prognosis. Unfortunately, no clinical biomarkers are available for use in early diagnosis. Quantitative proteomics is a powerful tool for biomarker discovery in cancer, autoimmune diseases, and ARDS. Here, we employed isobaric tags for relative and absolute quantitation (iTRAQ) technology to identify potential biomarkers for early ARDS diagnosis and predict the risk for increased disease severity induced by pneumonia. We collected the bronchoalveolar lavage fluid (BALF) and plasma from ARDS patients with differing degrees of ARDS severity. We identified 338 proteins dysregulated in ARDS through iTRAQ, 18 of which showed significant differences with at least 1.5-fold differential expression in patients with mild or severe ARDS. Differential plasma expression of pulmonary surfactant associated protein A, apolipoprotein A1, and deleted in malignant brain tumors 1 protein (DMBT1) was verified in plasma samples. Our results indicate that DMBT1 can potentially serve as a biomarker for early ARDS diagnosis and disease severity assessment.

  1. Quantitative proteomic analysis by iTRAQ for identification of candidate biomarkers in plasma from acute respiratory distress syndrome patients.

    PubMed

    Chen, Xia; Shan, Qiang; Jiang, Li; Zhu, Bo; Xi, Xiuming

    2013-11-08

    Acute respiratory distress syndrome (ARDS) is a major cause of morbidity and mortality in critical patients. Proteomic analysis of plasma from individuals with ARDS could elucidate new biomarkers for diagnosis and pathophysiology and identify potential ARDS treatment targets. In this study, we recruited 26 patients (15 controls, 11 ARDS). The ARDS group was subdivided into two groups depending on the type of injury: (1) direct lung injury (AD) and (2) indirect lung injury (AI). Using iTRAQ (isobaric tags for relative and absolute quantitation) analysis, we identified 2429 peptides representing 132 plasma proteins. Among these, 16 were differentially expressed in ARDS patients, including 11 overlapping proteins between the AI and AD group and 5 AI-specific proteins. Protein annotation revealed that lipid transport and complement activation were significantly enriched in the biological process category, and lipid transporter, transporter, and serine-type peptidase activities were significantly enriched in the molecular function category. IPA (Ingenuity Pathway Analysis) signaling pathways revealed that the overlapping proteins were involved in a variety of signaling pathways, including those underlying acute phase response; liver X receptor/retinoid X receptor (LXR/RXR) and farnesoid X (FXR)/RXR activation; clathrin-mediated endocytosis; atherosclerosis; interleukin (IL)-12; complement system; and cytokine, nitric oxide, and reactive oxygen species production in macrophages. We present the first proteomic analysis of ARDS plasma using the iTRAQ approach. Our data provide new biomarker candidates and shed light on potential pathological mechanisms underlying ARDS.

  2. Extracorporeal membrane oxygenation improves survival in a novel 24-hour pig model of severe acute respiratory distress syndrome.

    PubMed

    Araos, Joaquín; Alegría, Leyla; García, Patricio; Damiani, Felipe; Tapia, Pablo; Soto, Dagoberto; Salomon, Tatiana; Rodriguez, Felipe; Amthauer, Macarena; Erranz, Benjamín; Castro, Gabriel; Carreño, Pamela; Medina, Tania; Retamal, Jaime; Cruces, Pablo; Bugedo, Guillermo; Bruhn, Alejandro

    2016-01-01

    Extracorporeal membrane oxygenation (ECMO) is increasingly being used to treat severe acute respiratory distress syndrome (ARDS). However, there is limited clinical evidence about how to optimize the technique. Experimental research can provide an alternative to fill the actual knowledge gap. The purpose of the present study was to develop and validate an animal model of acute lung injury (ALI) which resembled severe ARDS, and which could be successfully supported with ECMO. Eighteen pigs were randomly allocated into three groups: sham, ALI, and ALI + ECMO. ALI was induced by a double-hit consisting in repeated saline lavage followed by a 2-hour period of injurious ventilation. All animals were followed up to 24 hours while being ventilated with conventional ventilation (tidal volume 10 ml/kg). The lung injury model resulted in severe hypoxemia, increased airway pressures, pulmonary hypertension, and altered alveolar membrane barrier function, as indicated by an increased protein concentration in bronchoalveolar fluid, and increased wet/dry lung weight ratio. Histologic examination revealed severe diffuse alveolar damage, characteristic of ARDS. Veno-venous ECMO was started at the end of lung injury induction with a flow > 60 ml/kg/min resulting in rapid reversal of hypoxemia and pulmonary hypertension. Mortality was 0, 66.6 and 16.6% in the SHAM, ALI and ALI + ECMO groups, respectively (p < 0.05). This is a novel clinically relevant animal model that can be used to optimize the approach to ECMO and foster translational research in extracorporeal lung support.

  3. The relationship between positive end expiratory pressure and cardiac index in patients with acute respiratory distress syndrome

    PubMed Central

    Fares, Wassim H; Carson, Shannon S

    2013-01-01

    Purpose To evaluate the association between positive end-expiratory pressure (PEEP) and cardiac index in patients with acute respiratory distress syndrome (ARDS). Methods This is a secondary cross-sectional analysis of the FACTT multi-center randomized controlled trial enrolling adult patients within 48 hours of ARDS onset. Patients randomized to the pulmonary artery catheter arm, who had PEEP and cardiac index measurements performed within a short period of each other during the first 3 days of the FACTT study enrollment were included in this study. Since FACTT had a 2×2 factorial design, half of the patients were in a ‘liberal fluids’ study arm, and the other half were in a ‘conservative fluids’ study arm. Results The final study population (833 measurements or observations, in 367 patients) was comparable to the original overall FACTT study population. The mean PEEP level used was 8.2 ± 3.4 cm H2O, and the mean cardiac index was 4.2 ± 1.2 liters/minute/m2. There was no association between PEEP and cardiac index in patients with ARDS, even when adjusted for APACHE score, age, fluid study arm in FACTT, and sepsis. Conclusion In patients with ARDS who are managed with liberal or conservative fluid management protocols, PEEP is not associated with lower cardiac index. PMID:23993772

  4. Setting ventilation parameters guided by electrical impedance tomography in an animal trial of acute respiratory distress syndrome

    NASA Astrophysics Data System (ADS)

    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.

  5. Validity of Outcome Prediction Scoring Systems in Korean Patients with Severe Adult Respiratory Distress Syndrome Receiving Extracorporeal Membrane Oxygenation Therapy.

    PubMed

    Lee, Seunghyun; Yeo, Hye Ju; Yoon, Seong Hoon; Lee, Seung Eun; Cho, Woo Hyun; Jeon, Doo Soo; Kim, Yun Seong; Son, Bong Soo; Kim, Do Hyung

    2016-06-01

    Recently, several prognostic scoring systems for patients with severe acute respiratory distress syndrome (ARDS) requiring extracorporeal membrane oxygenation (ECMO) have been published. The aim of this study was to validate the established scoring systems for outcome prediction in Korean patients. We retrospectively reviewed the data of 50 patients on ECMO therapy in our center from 2012 to 2014. A calculation of outcome prediction scoring tools was performed and the comparison across various models was conducted. In our study, the overall hospital survival was 46% and successful weaning rate was 58%. The Predicting Death for Severe ARDS on V-V ECMO (PRESERVE) score showed good discrimination of mortality prediction for patients on ECMO with AUC of 0.80 (95% CI 0.66-0.90). The respiratory extracorporeal membrane oxygenation survival prediction (RESP) score and simplified acute physiology score (SAPS) II score also showed fair prediction ability with AUC of 0.79 (95% CI 0.65-0.89) and AUC of 0.78 (95% CI 0.64-0.88), respectively. However, the ECMOnet score failed to predict mortality with AUC of 0.51 (95% CI 0.37-0.66). When evaluating the predictive accuracy according to optimal cut-off point of each scoring system, RESP score had a best specificity of 91.3% and 66.7% of sensitivity, respectively. This study supports the clinical usefulness of the prognostic scoring tools for severe ARDS with ECMO therapy when applying to the Korean patients receiving ECMO.

  6. Colfosceril palmitate. A pharmacoeconomic evaluation of a synthetic surfactant preparation (Exosurf Neonatal) in infants with respiratory distress syndrome.

    PubMed

    Bryson, H M; Whittington, R

    1994-12-01

    Comprehensive clinical data provide strong evidence of the efficacy of the synthetic lung surfactant colfosceril palmitate (Exosurf Neonatal) administered as prophylaxis or rescue therapy in infants with respiratory distress syndrome (RDS). The use of rescue therapy with colfosceril palmitate is further supported by cost-effectiveness analyses which report a 9 to 48% reduction in the cost per survivor compared with placebo or historical controls, despite divergent study methodology and location. Importantly, the savings were evident in both larger (> or = 1250g) and smaller (700 to 1350g) infants. All studies considered costs or charges accrued during initial hospitalisation through to 1 year; measurement of long term resource use data and all resulting costs are required for a more complete pharmacoeconomic evaluation. The optimal timing of surfactant administration is likely to be an important economic issue given that efficacy data from a large international trial support earlier administration of colfosceril palmitate versus delayed therapy in high risk patients. Further economic benefits may be realised by the sequential use of antenatal corticosteroids and surfactant therapy, although this has yet to be prospectively investigated. In conclusion, clinical and pharmacoeconomic data strongly support the use of rescue therapy with colfosceril palmitate. Additionally, recent clinical data indicating that even better results may be achieved with earlier administration and/or combined use with antenatal corticosteroids should be assessed from an economic standpoint to determine the optimal prescribing strategy for this agent.

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

    PubMed Central

    2016-01-01

    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. PMID:27761452

  8. Cardiac arrest due to a missed diagnosis of Boerhaave's syndrome.

    PubMed

    Davies, Jennifer; Spitzer, David; Phylactou, Maria; Glasser, Martin

    2016-05-06

    A 91-year-old presented with a rare cause of cardiac arrest. He was initially admitted with severe back pain following vomiting and diagnosed with probable aspiration pneumonia. On day 3 of admission, he was discovered in cardiac arrest and cardiopulmonary resuscitation was started. On intubation, a left-sided pneumothorax and subcutaneous emphysema were noted. Needle decompression showed gastric fluid leaking from the cannula. The patient regained a cardiac output, and a subsequent CT scan confirmed a large pneumomediastinum with air tracking to the neck and chest, and bilateral pneumothoraces. A diagnosis of Boerhaave's syndrome was made. The patient was transferred to the intensive care unit but did not survive. This case demonstrates the importance of looking for and treating the rarer reversible causes of cardiac arrest, and of maintaining a high index of suspicion for Boerhaave's syndrome. Despite its rarity, Boerhaave's syndrome is often misdiagnosed on initial presentation, leading to delayed treatment and poor outcomes.

  9. Role of Infection Due to Campylobacter jejuni in the Initiation of Guillain-Barre Syndrome

    DTIC Science & Technology

    1993-01-01

    Initiation of Guillain - Barre Syndrome 90PP0820 Ban Mishu and Martin J. Blaser Veterans Administration Medical Center 93-26165 Research Service 1310 24th...Unlimited Best Available Copy 104 Role of Infection Due to Campylobacterjejuni in the Initiation of Guillain - Barre Syndrome Ban Mishu and Martin J. Blaser...may cause Guillain -Barrk syndrome (GBS) by triggering demyelination of peripheral nerves. GBS is preceded by an acute infectious illness (due to a

  10. Placenta accreta: adherent placenta due to Asherman syndrome

    PubMed Central

    Engelbrechtsen, Line; Langhoff-Roos, Jens; Kjer, Jens Joergen; Istre, Olav

    2015-01-01

    Key Clinical Message It is important to be aware of the risk of abnormally invasive placenta in patients with a history of Asherman syndrome and uterine scarring. A prenatal diagnosis by ultrasonography is useful when planning of mode of delivery. PMID:25838908

  11. A case of intravascular lymphoma complicated with Fournier's syndrome due to multidrug-resistant Pseudomonas aeruginosa.

    PubMed

    Kaya, Hiroyasu; Yoshida, Takashi

    2011-01-01

    Fournier's syndrome is the fulminant necrotizing fasciitis of the external genitalia. The occurrence of Fournier's syndrome in patients with hematologic malignancies has been reported. Here we report a case of an intravascular lymphoma complicated with Fournier's syndrome due to multidrug-resistant Pseudomonas aeruginosa (MDRP). A 71-year-old Japanese man received intensive chemotherapy for recurring intravascular lymphoma. Blood culture revealed MDRP, and physical examination led to the diagnosis of Fournier's syndrome. Aggressive treatment that comprised granulocyte transfusion, granulocyte stimulating factor, endotoxin filtration, appropriate antibiotic coverage, and aggressive surgical therapy was administered, and this lead to the successful recovery from sepsis and Fournier's syndrome.

  12. West syndrome due to vitamin B12 deficiency.

    PubMed

    Serin, Hepsen Mine; Kara, Aslıhan Oruçoğlu; Oğuz, Baran

    2015-12-01

    Vitamin B12 is one of the essential vitamins affecting various systems of the body. Vitamin B12 deficiency in infants often produces haematological and neurological deficits including macrocyticanaemia, neurodevelopmental delay or regression, irritability, weakness, hypotonia, ataxia, apathy, tremor andseizures. In this article, we report the case of a six-month-old male patient diagnosed with West syndrome associated with vitamin B12 deficiency. Although the patient had no evidence of macrocytic anemia in complete blood count, we measured the level of vitamin B12 because the patient had hypotonicity and found it to be low. No other problem was found in the other investigations directed to the etiology of West syndrome. He was being exclusively breast-fed and vitamin B12 deficiency was related with nutritional inadequacy of his mother. Vitamin B12 deficiency should be considered in the differential diagnosis of patients presenting with different neurological findings. In addition, vitamin B12 deficiency should be considered as a rare cause in West syndrome which has a heterogeneous etiology.

  13. The prognostic value of N-terminal proB-type natriuretic peptide in patients with acute respiratory distress syndrome

    PubMed Central

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

    2017-01-01

    We investigated whether N-terminal proB-type natriuretic peptide (NT-proBNP) predicts the prognosis of patients with acute respiratory distress syndrome (ARDS). Between December 1, 2012, and May 31, 2015, this observational study recruited patients admitted to our tertiary medical center who met the Berlin criteria for ARDS and who had their NT-proBNP measured. The main outcome was 28-day mortality. We enrolled 61 patients who met the Berlin criteria for ARDS: 7 were classified as mild, 29 as moderate, and 25 as severe. The median APACHE II scores were 23 (interquartile range [IQR], 18–28), and SOFA scores were 11 (IQR, 8–13). The median lung injury score was 3.0 (IQR, 2.50–3.25), and the median level of NT-proBNP was 2011 pg/ml (IQR, 579–7216). Thirty-four patients died during this study, and the 28-day mortality rate was 55.7%. Patients who die were older and had significantly (all p < 0.05) higher APACHE II scores and NT-proBNP levels than did patients who survived. Multivariate analysis identified age (HR: 1.546, 95% CI: 1.174–2.035, p = 0.0019) and NT-proBNP (HR: 1.009, 95% CI: 1.004–1.013, p = 0.0001) as significant risk factors of death. NT-proBNP was associated with poor outcomes for patients with ARDS, and its level predicted mortality. PMID:28322314

  14. Global and regional assessment of sustained inflation pressure-volume curves in patients with acute respiratory distress syndrome.

    PubMed

    Becher, Tobias; Rostalski, Philipp; Kott, Matthias; Adler, Andy; Schadler, Dirk; Weiler, Norbert; Frerichs, Inez

    2017-03-24

    Static or quasi-static pressure-volume (P-V) curves can be used to determine the lung mechanical properties of patients suffering from acute respiratory distress syndrome (ARDS). According to the traditional interpretation, lung recruitment occurs mainly below the lower point of maximum curvature (LPMC) of the inflation P-V curve. Although some studies have questioned this assumption, setting of positive end-expiratory pressure 2 cmH2O above the LPMC was part of a "lung-protective" ventilation strategy successfully applied in several clinical trials. The aim of our study was to quantify the amount of unrecruited lung at different clinically relevant points of the P-V curve. P-V curves and electrical impedance tomography (EIT) data from 30 ARDS patients were analysed. We determined the regional opening pressures for every EIT image pixel and fitted the global P-V curves to five sigmoid model equations to determine the LPMC, inflection point (IP) and upper point of maximal curvature (UPMC). Points of maximal curvature and IP were compared between the models by one-way analysis of variance (ANOVA). The percentages of lung pixels remaining closed ("unrecruited lung") at LPMC, IP and UPMC were calculated from the number of lung pixels exhibiting regional opening pressures higher than LPMC, IP and UPMC and were also compared by one-way ANOVA. As results, we found a high variability of LPMC values among the models, a smaller variability of IP and UPMC values. We found a high percentage of unrecruited lung at LPMC, a small percentage of unrecruited lung at IP and no unrecruited lung at UPMC. Our results confirm the notion of ongoing lung recruitment at pressure levels above LPMC for all investigated model equations and highlight the importance of a regional assessment of lung recruitment in patients with ARDS.

  15. “Do Not Resuscitate” Decisions in Acute Respiratory Distress Syndrome. A Secondary Analysis of Clinical Trial Data

    PubMed Central

    Wiener, Renda Soylemez; Walkey, Allan J.

    2014-01-01

    Rationale: Factors and outcomes associated with end-of-life decision-making among patients during clinical trials in the intensive care unit are unclear. Objectives: We sought to determine patterns and outcomes of Do Not Resuscitate (DNR) decisions among critically ill patients with acute respiratory distress syndrome (ARDS) enrolled in a clinical trial. Methods: We performed a secondary analysis of data from the ARDS Network Fluid and Catheter Treatment Trial (FACTT), collected between 2000 and 2005. We calculated mortality outcomes stratified by code status, and compared baseline characteristics of patients who became DNR during the trial with participants who remained full code. Measurements and Main Results: Among 809 FACTT participants with a code status recorded, 232 (28.7%) elected DNR status. Specifically, 37 (15.9%) chose to withhold cardiopulmonary resuscitation alone, 44 (19.0%) elected to withhold some life support measures in addition to cardiopulmonary resuscitation, and 151 (65.1%) had life support withdrawn. Admission severity of illness as measured by APACHE III score was strongly associated with election of DNR status (odds ratio, 2.2; 95% confidence interval, 1.85–2.62; P < 0.0001). Almost all (97.0%; 225 of 232) patients who selected DNR status died, and 79% (225 of 284) of patients who died during the trial were DNR. Among patients who chose DNR status but did not elect withdrawal of life support, 91% (74 of 81) died. Conclusions: The vast majority of deaths among clinical trial patients with ARDS were preceded by a DNR order. Unlike other studies of end-of-life decision-making in the intensive care unit, nearly all patients who became DNR died. The impact of variation of practice in end-of-life decision-making during clinical trials warrants further study. PMID:25386717

  16. A study on the role of noninvasive ventilation in mild-to-moderate acute respiratory distress syndrome

    PubMed Central

    Sehgal, Inderpaul Singh; Chaudhuri, Soumik; Dhooria, Sahajal; Agarwal, Ritesh; Chaudhry, Dhruva

    2015-01-01

    Aim: There is sparse data on the role of noninvasive ventilation (NIV) in acute respiratory distress syndrome (ARDS) from India. Herein, we report our experience with the use of NIV in mild to moderate ARDS. Materials and Methods: This was a prospective observational study involving consecutive subjects of ARDS treated with NIV using an oronasal mask. Patients were monitored clinically with serial arterial blood gas analysis. The success of NIV, duration of NIV use, Intensive Care Unit stay, hospital mortality, and improvement in clinical and blood gas parameters were assessed. The success of NIV was defined as prevention of endotracheal intubation. Results: A total of 41 subjects (27 women, mean age: 30.9 years) were included in the study. Tropical infections followed by abdominal sepsis were the most common causes of ARDS. The use of NIV was successful in 18 (44%) subjects, while 23 subjects required intubation. The median time to intubation was 3 h. Overall, 19 (46.3%) deaths were encountered, all in those requiring invasive ventilation. The mean duration of ventilation was significantly higher in the intubated patients (7.1 vs. 2.6 days, P = 0.004). Univariate analysis revealed a lack of improvement in PaO2/FiO2 at 1 h and high baseline Acute Physiology and Chronic Health Evaluation II (APACHE II) as predictors of NIV failure. Conclusions: Use of NIV in mild to moderate ARDS helped in avoiding intubation in about 44% of the subjects. A baseline APACHE II score of >17 and a PaO2/FiO2 ratio <150 at 1 h predicts NIV failure. PMID:26628824

  17. Kinetics and Role of Plasma Matrix Metalloproteinase-9 Expression in Acute Lung Injury and the Acute Respiratory Distress Syndrome

    PubMed Central

    Hsu, Albert T.; Barrett, Christopher D.; DeBusk, M. George; Ellson, Christian D.; Gautam, Shiva; Talmor, Daniel S.; Gallagher, Diana C.; Yaffe, Michael B.

    2016-01-01

    Primed neutrophils that are capable of releasing matrix metalloproteinases (MMPs) into the circulation are thought to play a significant role in the pathophysiology of acute respiratory distress syndrome (ARDS). We hypothesized that direct measurement of plasma MMP-9 activity may be a predictor of incipient tissue damage and subsequent lung injury, which was investigated in both an animal model of ARDS and a small cohort of 38 critically ill human patients. In a mouse model of ARDS involving instillation of intratracheal LPS to induce lung inflammation, we measured neutrophil-mediated inflammation, along with MMP-9 activity in the airways and lung tissue and MMP-9 expression in the plasma. Neutrophil recruitment, inflammation, and MMP-9 activity in the airways and lung tissue increased throughout the 72 hours after LPS instillation, while plasma MMP-9 expression was greatest at 12–24 hours after LPS instillation. The results suggest that the peak in plasma MMP-9 activity may precede the peak of neutrophil inflammation in the airways and lung tissue in the setting of ARDS. Based on this animal study, a retrospective observational cohort study involving 38 patients admitted to a surgical intensive care unit (SICU) at a tertiary care university hospital with acute respiratory failure requiring intubation and mechanical ventilation was conducted. Plasma samples were collected daily, and MMP-9 activity was compared with lung function as determined by the PaO2/FiO2 ratio. In patients that developed ARDS, a notable increase in plasma MMP-9 activity on a particular day correlated with a decrease in the PaO2/FiO2 ratio on the following day (r = −0.503, p < 0.006). Taken together, these results suggest that plasma MMP-9 activity changes as a surrogate for primed neutrophils may have predictive value for the development of ARDS in a selected subset of critically ill patients. PMID:26009816

  18. The Hemagglutinin Stem-Binding Monoclonal Antibody VIS410 Controls Influenza Virus-Induced Acute Respiratory Distress Syndrome

    PubMed Central

    Baranovich, Tatiana; Jones, Jeremy C.; Russier, Marion; Vogel, Peter; Szretter, Kristy J.; Seiler, Patrick; Trevejo, Jose M.; Webby, Richard J.

    2016-01-01

    Most cases of severe influenza are associated with pulmonary complications, such as acute respiratory distress syndrome (ARDS), and no antiviral drugs of proven value for treating such complications are currently available. The use of monoclonal antibodies targeting the stem of the influenza virus surface hemagglutinin (HA) is a rapidly developing strategy for the control of viruses of multiple HA subtypes. However, the mechanisms of action of these antibodies are not fully understood, and their ability to mitigate severe complications of influenza has been poorly studied. We evaluated the effect of treatment with VIS410, a human monoclonal antibody targeting the HA stem region, on the development of ARDS in BALB/c mice after infection with influenza A(H7N9) viruses. Prophylactic administration of VIS410 resulted in the complete protection of mice against lethal A(H7N9) virus challenge. A single therapeutic dose of VIS410 given 24 h after virus inoculation resulted in dose-dependent protection of up to 100% of mice inoculated with neuraminidase inhibitor-susceptible or -resistant A(H7N9) viruses. Compared to the outcomes in mock-treated controls, a single administration of VIS410 improved viral clearance from the lungs, reduced virus spread in lungs in a dose-dependent manner, resulting in a lower lung injury score, reduced the extent of the alteration in lung vascular permeability and protein accumulation in bronchoalveolar lavage fluid, and improved lung physiologic function. Thus, antibodies targeting the HA stem can reduce the severity of ARDS and show promise as agents for controlling pulmonary complications in influenza. PMID:26787699

  19. The prognostic value of N-terminal proB-type natriuretic peptide in patients with acute respiratory distress syndrome.

    PubMed

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

    2017-03-21

    We investigated whether N-terminal proB-type natriuretic peptide (NT-proBNP) predicts the prognosis of patients with acute respiratory distress syndrome (ARDS). Between December 1, 2012, and May 31, 2015, this observational study recruited patients admitted to our tertiary medical center who met the Berlin criteria for ARDS and who had their NT-proBNP measured. The main outcome was 28-day mortality. We enrolled 61 patients who met the Berlin criteria for ARDS: 7 were classified as mild, 29 as moderate, and 25 as severe. The median APACHE II scores were 23 (interquartile range [IQR], 18-28), and SOFA scores were 11 (IQR, 8-13). The median lung injury score was 3.0 (IQR, 2.50-3.25), and the median level of NT-proBNP was 2011 pg/ml (IQR, 579-7216). Thirty-four patients died during this study, and the 28-day mortality rate was 55.7%. Patients who die were older and had significantly (all p < 0.05) higher APACHE II scores and NT-proBNP levels than did patients who survived. Multivariate analysis identified age (HR: 1.546, 95% CI: 1.174-2.035, p = 0.0019) and NT-proBNP (HR: 1.009, 95% CI: 1.004-1.013, p = 0.0001) as significant risk factors of death. NT-proBNP was associated with poor outcomes for patients with ARDS, and its level predicted mortality.

  20. Impact of Initial Central Venous Pressure on Outcomes of Conservative versus Liberal Fluid Management in Acute Respiratory Distress Syndrome

    PubMed Central

    Semler, Matthew W.; Wheeler, Arthur P.; Thompson, B. Taylor; Bernard, Gordon R.; Wiedemann, Herbert P.; Rice, Todd W.

    2016-01-01

    Objective In acute respiratory distress syndrome (ARDS), conservative fluid management increases ventilator-free days without affecting mortality. Response to fluid management may differ based on patients’ initial central venous pressure (CVP). We hypothesized initial CVP would modify the effect of fluid management on outcomes. Design Retrospective analysis of the Fluid and Catheter Treatment Trial, a multicenter randomized trial comparing conservative to liberal fluid management in ARDS. We examined the relationship between initial CVP, fluid strategy, and 60-day mortality in univariate and multivariable analysis. Setting Twenty acute care hospitals. Patients Nine hundred and thirty-four ventilated ARDS patients with a CVP available at enrollment, 609 without baseline shock (for whom fluid balance was managed by study protocol). Interventions None. Measurements and Main Results Among patients without baseline shock, those with initial CVP > 8 mmHg experienced similar mortality with conservative and liberal fluid management (18% versus 18%, p=0.928), whereas those with CVP ≤8 mmHg experienced lower mortality with a conservative strategy (17% versus 36%, p=0.005). Multivariable analysis demonstrated an interaction between initial CVP and the effect of fluid strategy on mortality (p=0.031). At higher initial CVPs, the difference in treatment between arms was predominantly furosemide administration, which was not associated with mortality (p=0.122). At lower initial CVPs, the difference between arms was predominantly fluid administration, with additional fluid associated with increased mortality (p=0.013). Conclusions Conservative fluid management decreases mortality for ARDS patients with a low initial central venous pressure. In this population, the administration of intravenous fluids appears to increase mortality. PMID:26741580

  1. Effect of surfactant on regional lung function in an experimental model of respiratory distress syndrome in rabbit.

    PubMed

    Bayat, Sam; Porra, Liisa; Broche, Ludovic; Albu, Gergely; Malaspinas, Iliona; Doras, Camille; Strengell, Satu; Peták, Ferenc; Habre, Walid

    2015-08-01

    We assessed the changes in regional lung function following instillation of surfactant in a model of respiratory distress syndrome (RDS) induced by whole lung lavage and mechanical ventilation in eight anaesthetized, paralyzed, and mechanically ventilated New Zealand White rabbits. Regional specific ventilation (sV̇) was measured by K-edge subtraction synchrotron computed tomography during xenon washin. Lung regions were classified as poorly aerated (PA), normally aerated (NA), or hyperinflated (HI) based on regional density. A functional category was defined within each class based on sV̇ distribution (High, Normal, and Low). Airway resistance (Raw), respiratory tissue damping (G), and elastance (H) were measured by forced oscillation technique at low frequencies before and after whole lung saline lavage-induced (100 ml/kg) RDS, and 5 and 45 min after intratracheal instillation of beractant (75 mg/kg). Surfactant instillation improved Raw, G, and H (P < 0.05 each), and gas exchange and decreased atelectasis (P < 0.001). It also significantly improved lung aeration and ventilation in atelectatic lung regions. However, in regions that had remained normally aerated after lavage, it decreased regional aeration and increased sV̇ (P < 0.001) and sV̇ heterogeneity. Although surfactant treatment improved both central airway and tissue mechanics and improved regional lung function of initially poorly aerated and atelectatic lung, it deteriorated regional lung function when local aeration was normal prior to administration. Local mechanical and functional heterogeneity can potentially contribute to the worsening of RDS and gas exchange. These data underscore the need for reassessing the benefits of routine prophylactic vs. continuous positive airway pressure and early "rescue" surfactant therapy in very immature infants.

  2. Kinetics and Role of Plasma Matrix Metalloproteinase-9 Expression in Acute Lung Injury and the Acute Respiratory Distress Syndrome.

    PubMed

    Hsu, Albert T; Barrett, Christopher D; DeBusk, George M; Ellson, Christian D; Gautam, Shiva; Talmor, Daniel S; Gallagher, Diana C; Yaffe, Michael B

    2015-08-01

    Primed neutrophils that are capable of releasing matrix metalloproteinases (MMPs) into the circulation are thought to play a significant role in the pathophysiology of acute respiratory distress syndrome (ARDS). We hypothesized that direct measurement of plasma MMP-9 activity may be a predictor of incipient tissue damage and subsequent lung injury, which was investigated in both an animal model of ARDS and a small cohort of 38 critically ill human patients. In a mouse model of ARDS involving instillation of intratracheal lipopolysaccharide (LPS) to induce lung inflammation, we measured neutrophil-mediated inflammation, along with MMP-9 activity in the airways and lung tissue and MMP-9 expression in the plasma. Neutrophil recruitment, inflammation, and MMP-9 activity in the airways and lung tissue increased throughout the 72 h after LPS instillation, whereas plasma MMP-9 expression was greatest at 12 to 24 h after LPS instillation. The results suggest that the peak in plasma MMP-9 activity may precede the peak of neutrophil inflammation in the airways and lung tissue in the setting of ARDS. Based on this animal study, a retrospective observational cohort study involving 38 patients admitted to a surgical intensive care unit at a tertiary care university hospital with acute respiratory failure requiring intubation and mechanical ventilation was conducted. Plasma samples were collected daily, and MMP-9 activity was compared with lung function as determined by the PaO2/FiO2 ratio. In patients who developed ARDS, a notable increase in plasma MMP-9 activity on a particular day correlated with a decrease in the PaO2/FiO2 ratio on the following day (r = -0.503, P < 0.006). Taken together, these results suggest that plasma MMP-9 activity changes, as a surrogate for primed neutrophils may have predictive value for the development of ARDS in a selected subset of critically ill patients.

  3. Outcome of veno-venous extracorporeal membrane oxygenation use in acute respiratory distress syndrome after cardiac surgery with cardiopulmonary bypass

    PubMed Central

    Song, Joo Han; Woo, Won Ki; Song, Seung Hwan; Kim, Hyo Hyun; Kim, Bong Joon; Kim, Ha Eun; Kim, Do Jung; Suh, Jee Won; Shin, Yu Rim; Park, Han Ki; Lee, Seung Hyun; Joo, Hyun Chel; Lee, Sak; Chang, Byung Chul; Yoo, Kyung Jong; Kim, Young Sam

    2016-01-01

    Background Cardiac surgery with cardiopulmonary bypass (CPB) is a known risk factor for acute respiratory distress syndrome (ARDS). We aimed to analyze the treatment outcome in patients who required veno-venous extracorporeal membrane oxygenation (VV-ECMO) for postcardiotomy ARDS despite other rescue modalities. Methods We retrospectively reviewed the outcomes in 13 patients (mean age, 54.7±5.9 years) who received VV-ECMO support for refractory ARDS after cardiac surgery between March 2013 and February 2016 at Severance Hospital, Yonsei University (Seoul, Korea). Results At the start of VV-ECMO, the average lung injury score was 3.0±0.2, and the Respiratory Extracorporeal Membrane Oxygenation Survival Prediction (RESP) score was −4±1.1. Although 7 patients initiated VV-ECMO support within 24 h from operation, the remaining 6 started at a median of 8.5 days (range, 5−16 days). Nine (69.3%) patients were successfully weaned from VV-ECMO. After a median follow-up duration of 14.5 months (range, 1.0−33.0 months) for survivors, the 1-year overall survival was 58.6%±14.4%. The differences in the overall survival from VV-ECMO according to the RESP score risk classes were borderline significant (100% in class III, 50%±25% in class IV, and 20%±17.9% in class V; P=0.088). Conclusions VV-ECMO support can be a feasible rescue strategy for adult patients who develop refractory ARDS after a cardiac surgery. Additionally, the RESP score seems a valuable prognostic tool for post-ECMO survival outcome in this patient population as well. PMID:27499972

  4. Deaths Due to Choking in Prader-Willli Syndrome

    PubMed Central

    Stevenson, David A.; Heinemann, Janalee; Angulo, Moris; Butler, Merlin G.; Loker, Jim; Rupe, Norma; Kendell, Patrick; Clericuzio, Carol L.; Scheimann, Ann O.

    2011-01-01

    Prader-Willi syndrome (PWS) is the most common known syndromic cause of life threatening obesity, yet few studies have examined the causes of death in PWS. The objective of this study was to examine the contribution of choking leading to mortality in PWS. In 1999, a brief survey was made available from the Prader-Willi Syndrome Association (USA) bereavement program, which documented demographic data and causes of death. Families were subsequently offered the opportunity to fill out a detailed questionnaire and additional forms to release medical records. Demographic information was available on 178 deceased individuals with PWS, and cause of death available on 152 individuals. Fifty-four families completed questionnaires. Of the deceased individuals with completed questionnaires, 34% reported a history of choking. Choking was listed by familial report as the cause of death in 12 (7.9%) of 152 subjects with an average age of 24 years (range 3–52y; median 22.5y) at death from choking. Only two of these individuals were less than eight years of age. The data suggest that risks associated with choking are different in the PWS population compared with normal. Potential causes of increased choking in PWS include poor oral/motor coordination, poor gag reflex, hypotonia, hyperphagia, decreased mastication and voracious feeding habits. We recommend implementation of preventive measures and education for families and group home care providers for all individuals with PWS including the Heimlich maneuver, supervised meals, better food preparation and diet modification to avoid high risk choking items. PMID:17036318

  5. GUYON'S CANAL SYNDROME DUE TO A SYNOVIAL CYST

    PubMed Central

    Ruas, Evandro Silva; Castilho, Rodrigo Simões; Maia, Philipe Eduardo Carvalho; Melo, Gustavus Lemos Ribeiro

    2015-01-01

    We present a case of Guyon's canal syndrome caused by a synovial cyst within the left wrist of a 48-year-old female patient. The patient presented pain and paresthesia in the region of the ulnar nerve, with loss of muscle strength and left-hand deformity. Electroneuromyography showed a compression of the ulnar nerve at the wrist level. Surgical decompression of the nerve at Guyon's canal with resection of the cyst was performed. After the surgery, the patient presented an improvement in the pain and paresthesia, as well as an increase in muscle trophism and correction of the deformity. PMID:27026975

  6. [Locomotive syndrome and frailty. Locomotive syndrome due to the underlying disease of degenerative arthritis].

    PubMed

    Chosa, Etsuo

    2012-04-01

    Japan became a superaging society. We have been putting a new focus on locomotive syndrome and frailty. The prevention and treatment of locomotive syndromes, such as osteoarthritis, degenerative spondylosis, lumbar canal stenosis, osteoporosis, upper extremity diseases, rheumatoid arthritis, and many other disorders of the locomotive organs are important. Because, the locomotive syndrome results in deterioration of the exercise function and loss of mental and physical health. The aim of locomotive syndrome exercises are: to reduce pain, to restore and improve joint function. We need to take a comprehensive approach to locomotive syndrome, including lifestyle modification, muscle exercise, stretching and therapeutic exercise.

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

    PubMed Central

    Valizadeh, Sousan; Hosseini, Mohammad Bagher; Asghari Jafarabadi, Mohammad; Ajoodanian, Najmeh

    2012-01-01

    Introduction: Nowadays particular emphasis is placed on the developmental aspects of premature infants care. Massage therapy is one of the best-known methods of caring. Due to the minimal touch policy in neonatal intensive care units (NICUs), massaging is not usually performed on premature infants. However, there is not sufficient evidence to support the claim that newborn infants with complex medical conditions should not be massaged. This study aimed to determine the effects of massage with coconut and sunflower oils on oxygen saturation of infants with respiratory distress syndrome (RDS) treated with nasal continuous positive airway pressure (NCPAP). Methods: This was a randomized controlled trial on 90 newborns who were admitted to Alzahra Hospital (Tabriz, Iran). The infants were divided into control and massage therapy groups (massage with coconut and sunflower oils). Data was collected using a hospital documentation form. A 15-minute daily massage was performed for 3 days. Respiratory rate (RR), fraction of inspired oxygen (FiO2) and oxygen saturation were measured 5 minutes before the massage, 3 times during the massage, and 5 minutes after the massage. The collected data was analyzed using a mixed model. Results: In comparison to coconut oil and control groups, mean oxygen saturation of sunflower oil group was improved. In addition, the coconut massage group showed lower oxygen saturation than the control group but was all values were within the normal range. Although massage decreased oxygen saturation, there was no need to increase FiO2. Conclusion: Massage therapy can provide developmental care for infants treated with NCPAP. PMID:25276695

  8. Permanent bilateral cortical blindness due to reversible posterior leukoencephalopathy syndrome.

    PubMed

    Iwama, Mayumi; Takahashi, Hiroshi; Takagi, Ryo; Hiraoka, Miki

    2011-01-01

    Reversible posterior leukoencephalopathy syndrome (RPLS) is induced by acute cerebral edema. Its symptoms include seizures, headache, altered mental status, and visual disturbances. The clinical and radiological findings are usually transient. This report describes a case of RPLS resulting in bilateral total blindness. A 40-year-old man presented with lethargy and bilateral visual loss. He had a 20-year history of hypertension, but had never been treated. On presentation, the left eye was able to perceive light, but the right eye was not. Radiological examination showed diffuse edema in the brain, and ocular fundus examination revealed severe bilateral hypertensive retinopathy. Antihypertensive therapy improved the patient's general condition, including blood pressure. Radiological findings 5 months later showed resolution of most of the abnormal signal areas. However, total blindness had developed in both eyes by day 15, and two courses of pulsed corticosteroid therapy failed to restore the visual loss.

  9. [Aspiration syndrome due to laryngeal cleft in an infant].

    PubMed

    Cuestas, Giselle; Demarchi, Victoria; Zanetta, Adrián; Urquizo, Mauricio; Lobos, Pablo; Razetti, Juan

    2014-02-01

    Aspiration is the passage of food content and endogenous secretions into the airway. Anatomical, neuromuscular or functional anomalies are among the major causes. The laryngeal cleft is a rare congenital anomaly that should be considered in the differential diagnosis of aspiration syndrome in neonates and infants. The main symptoms are stridor, recurrent respiratory infections and cyanotic crisis, cough and choking during feeding. The diagnosis is confirmed by endoscopic examination. The therapeutic behaviour will depend on the extent of the cleft, among other factors. We describe the clinical manifestations, diagnostic methods and treatment of an infant with this disease, and we emphasize the need for recognition of swallowing disorders in children in order to establish an accurate diagnosis and appropriate treatment to prevent and avoid malnutrition as well as a severe and potentially irreversible lung compromise.

  10. Distress syndromes, illness behavior, access to care and medical utilization in a defined population.

    PubMed

    Mechanic, D; Cleary, P D; Greenley, J R

    1982-04-01

    This article examines the use of general medical services in a representative sample from a defined geographic area and in a sample of persons seeking psychiatric care from the same area. Psychiatric patients made 100 per cent more general medical care visits in the retrospective period and 83 per cent more in the prospective period than persons who did not seek mental health care. The analysis focuses on the determinants in general medical care use between those who sought mental health care and those who did not. The first hypothesis is that physical symptoms and dysfunction concomitant with psychologic disorder explain the difference. The second argues that the association is a product of help-seeking orientations and illness behavior. The third focuses on variations due to differences in access. The first two types of factors are the most important. Using sex, physical symptoms and illness behavior measures, we explain 50 per cent of the differences in retrospective utilization and 40 per cent of the differences in prospective data.

  11. Pigmentary retinopathy due to Bardet-Biedl syndrome: case report and literature review.

    PubMed

    Andrade, Luis Jesuino de Oliveira; Andrade, Rafael; França, Caroline Santos; Bittencourt, Alcina Vinhaes

    2009-01-01

    Bardet-Biedl syndrome (BBS) is a rare autosomal recessive disorder with clinical and genetic heterogeneity. This syndrome was first described by Laurence and Moon in 1866 and additional cases were described by Bardet and Biedl between 1920 and 1922. The main features are obesity, polydactyly, pigmentary retinopathy, learning disabilities, various degrees of intellectual impairment, hypogonadism, and renal abnormalities. Bardet-Biedl syndrome is both phenotypically and genetically heterogeneous. Clinical diagnosis is based on the presence of 4 of the 5 cardinal features. The authors present a typical case of pigmentary retinopathy due to Bardet-Biedl syndrome and made a brief commentary about the disease's cardinal manifestations.

  12. Treatment with acarbose in severe hypoglycaemia due to late dumping syndrome.

    PubMed

    Wang, Congcong; Pang, Shuguang; Jiang, Qiang; Duan, Guanglan; Sun, Yongmei; Li, Mei

    2013-12-01

    Dumping syndrome is a serious complication that may occur after gastric surgery in approximately 10% of patients in the 1990s. With the increasing number of patients undergoing bariatric surgery, the incidence of dumping syndrome is likely to increase in recent years. It is necessary for clinicians to recognize the syndrome and master its management. We present a case of recurrent loss of consciousness, which was finally accurately diagnosed as late dumping syndrome twelve years after subtotal gastrectomy and successfully treated with acarbose. A 66-year old lean male was found unconscious repeatedly within one year, oral glucose tolerance tests performed before and after acarbose treatment verified the diagnosis of late dumping syndrome. Hypoglycaemia can damage the body in acute and chronic form. Acarbose can be used as a successful treatment modality for reactive hypoglycaemia due to late dumping syndrome by influencing the release of hormone.

  13. Bayesian inference of the lung alveolar spatial model for the identification of alveolar mechanics associated with acute respiratory distress syndrome

    NASA Astrophysics Data System (ADS)

    Christley, Scott; Emr, Bryanna; Ghosh, Auyon; Satalin, Josh; Gatto, Louis; Vodovotz, Yoram; Nieman, Gary F.; An, Gary

    2013-06-01

    Acute respiratory distress syndrome (ARDS) is acute lung failure secondary to severe systemic inflammation, resulting in a derangement of alveolar mechanics (i.e. the dynamic change in alveolar size and shape during tidal ventilation), leading to alveolar instability that can cause further damage to the pulmonary parenchyma. Mechanical ventilation is a mainstay in the treatment of ARDS, but may induce mechano-physical stresses on unstable alveoli, which can paradoxically propagate the cellular and molecular processes exacerbating ARDS pathology. This phenomenon is called ventilator induced lung injury (VILI), and plays a significant role in morbidity and mortality associated with ARDS. In order to identify optimal ventilation strategies to limit VILI and treat ARDS, it is necessary to understand the complex interplay between biological and physical mechanisms of VILI, first at the alveolar level, and then in aggregate at the whole-lung level. Since there is no current consensus about the underlying dynamics of alveolar mechanics, as an initial step we investigate the ventilatory dynamics of an alveolar sac (AS) with the lung alveolar spatial model (LASM), a 3D spatial biomechanical representation of the AS and its interaction with airflow pressure and the surface tension effects of pulmonary surfactant. We use the LASM to identify the mechanical ramifications of alveolar dynamics associated with ARDS. Using graphical processing unit parallel algorithms, we perform Bayesian inference on the model parameters using experimental data from rat lung under control and Tween-induced ARDS conditions. Our results provide two plausible models that recapitulate two fundamental hypotheses about volume change at the alveolar level: (1) increase in alveolar size through isotropic volume change, or (2) minimal change in AS radius with primary expansion of the mouth of the AS, with the implication that the majority of change in lung volume during the respiratory cycle occurs in the

  14. Surfactant Protein A and B Gene Polymorphisms and Risk of Respiratory Distress Syndrome in Late-Preterm Neonates

    PubMed Central

    Tsitoura, Maria-Eleni I.; Stavrou, Eleana F.; Maraziotis, Ioannis A.; Sarafidis, Kosmas; Athanassiadou, Aglaia; Dimitriou, Gabriel

    2016-01-01

    Background and Objectives Newborns delivered late-preterm (between 340/7 and 366/7 weeks of gestation) are at increased risk of respiratory distress syndrome (RDS). Polymorphisms within the surfactant protein (SP) A and B gene have been shown to predispose to RDS in preterm neonates. The aim of this study was to investigate whether specific SP-A and/or SP-B genetic variants are also associated with RDS in infants born late-preterm. Methods This prospective cross-sectional study included 56 late-preterm infants with and 60 without RDS. Specific SP-A1/SP-A2 haplotypes and SP-B Ile131Thr polymorphic alleles were determined in blood specimens using polymerase-chain-reaction and DNA sequencing. Results The SP-A1 6A4 and the SP-A2 1A5 haplotypes were significantly overrepresented in newborns with RDS compared to controls (OR 2.86, 95%CI 1.20–6.83 and OR 4.68, 95%CI 1.28–17.1, respectively). The distribution of the SP-B Ile131Thr genotypes was similar between the two late-preterm groups. Overall, the SP-A1 6A4 or/and SP-A2 1A5 haplotype was present in 20 newborns with RDS (35.7%), resulting in a 4.2-fold (1.60–11.0) higher probability of RDS in carriers. Multivariable regression analysis revealed that the effect of SP-A1 6A4 and SP-A2 1A5 haplotypes was preserved when adjusting for known risk or protective factors, such as male gender, smaller gestational age, smaller weight, complications of pregnancy, and administration of antenatal corticosteroids. Conclusions Specific SP-A genetic variants may influence the susceptibility to RDS in late-preterm infants, independently of the effect of other perinatal factors. PMID:27835691

  15. Bayesian inference of the lung alveolar spatial model for the identification of alveolar mechanics associated with acute respiratory distress syndrome.

    PubMed

    Christley, Scott; Emr, Bryanna; Ghosh, Auyon; Satalin, Josh; Gatto, Louis; Vodovotz, Yoram; Nieman, Gary F; An, Gary

    2013-06-01

    Acute respiratory distress syndrome (ARDS) is acute lung failure secondary to severe systemic inflammation, resulting in a derangement of alveolar mechanics (i.e. the dynamic change in alveolar size and shape during tidal ventilation), leading to alveolar instability that can cause further damage to the pulmonary parenchyma. Mechanical ventilation is a mainstay in the treatment of ARDS, but may induce mechano-physical stresses on unstable alveoli, which can paradoxically propagate the cellular and molecular processes exacerbating ARDS pathology. This phenomenon is called ventilator induced lung injury (VILI), and plays a significant role in morbidity and mortality associated with ARDS. In order to identify optimal ventilation strategies to limit VILI and treat ARDS, it is necessary to understand the complex interplay between biological and physical mechanisms of VILI, first at the alveolar level, and then in aggregate at the whole-lung level. Since there is no current consensus about the underlying dynamics of alveolar mechanics, as an initial step we investigate the ventilatory dynamics of an alveolar sac (AS) with the lung alveolar spatial model (LASM), a 3D spatial biomechanical representation of the AS and its interaction with airflow pressure and the surface tension effects of pulmonary surfactant. We use the LASM to identify the mechanical ramifications of alveolar dynamics associated with ARDS. Using graphical processing unit parallel algorithms, we perform Bayesian inference on the model parameters using experimental data from rat lung under control and Tween-induced ARDS conditions. Our results provide two plausible models that recapitulate two fundamental hypotheses about volume change at the alveolar level: (1) increase in alveolar size through isotropic volume change, or (2) minimal change in AS radius with primary expansion of the mouth of the AS, with the implication that the majority of change in lung volume during the respiratory cycle occurs in the

  16. The effect of demographics and patient location on the outcome of patients with acute respiratory distress syndrome

    PubMed Central

    El-Haddad, Haitham; Jang, Hyejeong; Chen, Wei; Haider, Samran; Soubani, Ayman O.

    2017-01-01

    OBJECTIVE: Outcome of acute respiratory distress syndrome (ARDS) in relation to age, gender, race, pre-Intensive Care Unit (ICU) location, and type of ICU. METHODS: Retrospective cohort study of patients enrolled in the ARDS network randomized controlled trials. RESULTS: A total of 2914 patients were included in these trials. Outcomes were adjusted to baseline covariates including APACHE III score, vasopressor use, cause of lung injury, lung injury score, diabetes, cancer status, body mass index, and study ID. Older patients had significantly higher mortality at both 28- and 60-day (odds ratio [OR] 2.59 [95% confidence interval [CI]: 2.12–3.18] P < 0.001 and 2.79, 95% CI: 2.29–3.39, P < 0.001, respectively); less ICU and ventilator free days (relative risk [RR] 0.92, 95% CI: 0.87–0.96, P < 0.001 and 0.92, 95% CI: 0.88–0.96, P < 0.001, respectively). For preadmission location, the 28- and 60-day mortality were lower if the patient was admitted from the operating room (OR)/recovery room (OR 0.65, 95% CI: 0.44–0.95, P = 0.026; and OR = 0.66, 95% CI: 0.46–0.95, P = 0.025, respectively) or emergency department (OR = 0.78, 95% CI: 0.61–0.99, P = 0.039; and OR = 0.71, 95% CI: 0.56–0.89, P = 0.004, respectively), but no statistical differences in ICU and ventilator free days between different preadmission locations. Races other than white and black had a statistically higher mortality (28- and 60-day mortality: OR = 1.47, 95% CI: 1.09–1.98, P = 0.011; and OR 1.53, 95% CI: 1.15–2.04, P = 0.004, respectively). Between whites and blacks, females and males there were no statistically significant differences in all outcomes. CONCLUSION: Older patients and races other than blacks and whites have higher mortality associated with ARDS. Mortality is affected by patients preadmission location. There are no differences in outcome in relation to the type of ICU, gender, or between blacks and whites. PMID:28197217

  17. Staphylococcal scalded skin syndrome due to burn wound infection.

    PubMed

    Farroha, A; Frew, Q; Jabir, S; Dziewulski, P

    2012-09-30

    Introduction. Le syndrome de la peau ébouillantée staphylococcique (sigle anglais conventionnel, SSSS) est une exfoliation aiguë de la peau causée par des toxines A et B. Bien que le Staphylococcus aureus soit une cause fréquente d’infection des brûlures, la SSSS suite à une infection brûlure est rare. Méthode. Les Auteurs ont effectué une revue rétrospective de tous les cas de patients atteints de SSSS hospitalises admis dans un service régional des brûlures entre janvier 2008 et janvier 2012. Résultats. Deux cas de SSSS ont été signalés au cours de cette période qui se sont produits suite à une brûlure. Le premier cas était un garçon de 17 mois qui avait été hospitalisé pour un traitement conservateur pour ébouillantement dans 6% de la surface corporelle totale de profondeur variable. Le quatrième jour, il a développé une exfoliation dans 85% de la surface corporelle. Quant au deuxième cas, il s’agissait d’un garçon de dix mois qui a subi une brûlure de 1% de la surface corporelle et qui a été traité en manière conservatrice dans la communauté par son médecin généraliste. Le cinquième jour, il a développé une exfoliation dans 80% de la surface corporelle. Le Staphylococcus aureus a été isolé qui provenait des brûlures dans les deux cas. Conclusion. Ces deux cas montrent qu’il est essentiel que les brûlologues et les spécialistes des soins intensifs soient au courant de la possibilité de la présence de SSSS chez des patients souffrant de brûlures, avec tous ses potentiels effets dévastateurs.

  18. Acute respiratory distress syndrome

    MedlinePlus

    ... be signs of fluid in the lungs. Often, blood pressure is low. Cyanosis (blue skin, lips, and nails caused by lack of oxygen to the tissues) is often seen. Tests used to diagnose ARDS include: Arterial blood gas Blood tests, including CBC and blood chemistries ...

  19. The Effect of the Treatment with Heated Humidified High-Flow Nasal Cannula on Neonatal Respiratory Distress Syndrome in China: A Single-Center Experience

    PubMed Central

    Zheng, Ge; Huang, Xiao-qiu; Zhao, Hui-hui; Jin, Guo-Xing

    2017-01-01

    Background. Noninvasive respiratory support is considered the optimal method of providing assistance to preterm babies with breathing problems, including nasal continuous positive airway pressure (NCPAP) and humidified high flow nasal cannula (HHHFNC). The evidence of the efficacy and safety of HHHFNC used as the primary respiratory support for respiratory distress syndrome (RDS) is insufficient in low- and middle-income countries. Objective. To investigate the effect of heated humidified high flow nasal cannula on neonatal respiratory distress syndrome compared with nasal continuous positive airway pressure. Methods. An observational cross-sectional study was performed at a tertiary neonatal intensive care unit in suburban Wenzhou, China, in the period between January 2014 and December 2015. Results. A total of 128 infants were enrolled in the study: 65 in the HHHFNC group and 63 in the NCPAP group. The respiratory support with HHHFNC was similar to that with NCPAP with regard to the primary outcome. There is no significant difference between two groups in secondary outcomes. Comparing with NCPAP group, the incidence of nasal damage was lower in HHHFNC group. Conclusions. HHHFNC is an effective and well-tolerated strategy as the primary treatment of mild to moderate RDS in preterm infants older than 28 weeks of GA. PMID:28167860

  20. Respiratory controversies in the critical care setting. Does high-frequency ventilation offer benefits over conventional ventilation in adult patients with acute respiratory distress syndrome?

    PubMed

    Fessler, Henry E; Hess, Dean R

    2007-05-01

    High-frequency ventilation is the application of mechanical ventilation with a respiratory rate > 100 breaths/min. High-frequency oscillatory ventilation (HFOV) is the form of high-frequency ventilation most widely used in adult critical care. The principles of lung-protective ventilation have matured in parallel with the technology for HFOV. The 2 basic principles of lung-protective ventilation are the use of small tidal volume and maintenance of adequate alveolar recruitment. Research in animal models and humans demonstrate that HFOV can support gas exchange with much smaller tidal volume than can be achieved with conventional ventilation. HFOV also provides more effective lung recruitment than conventional mechanical ventilation. However, at present, evidence is lacking that survival in adults with acute respiratory distress syndrome is improved by HFOV. Although HFOV may improve P(aO(2)) in some patients, this improvement is often transitory. Available evidence does not support that pulmonary inflammation is reduced with HFOV in adult acute respiratory distress syndrome. Heavy sedation and often paralysis are necessary. The promise of HFOV as a lung-protective ventilation strategy remains attractive, but additional clinical trials are needed to determine whether this approach is superior to lung-protective ventilation with conventional mechanical ventilation.

  1. Comparative trial of artificial and natural surfactants in the treatment of respiratory distress syndrome of prematurity: experiences in a developing country.

    PubMed

    da Costa, D E; Pai, M G; Al Khusaiby, S M

    1999-05-01

    We conducted a randomized clinical trial to compare the effects of a synthetic (Exosurf) and natural (Survanta) surfactant in infants with neonatal respiratory distress syndrome. Eighty-nine patients were randomly allocated to receive one of the two surfactants. Primary outcome variables were the acute and long-term effects of the surfactant preparations, i.e., ventilatory requirements at 24 h of age as judged by the oxygenation index (OI), and the combined incidence of chronic lung disease or death at 28 days. The OIs in the Exosurf and Survanta groups at 24 h were the same (10.1 and 7, respectively; P > 0.05). The magnitude and rapidity of response, however, were greater for Survanta than for Exosurf. When arterial/alveolar oxygen tension ratios (a/A) were compared, the Exosurf group had a significantly worse a/A ratio at 24 h than the Survanta group (0.21 Exosurf vs. 0.37 Survanta; P < 0.05). The long-term outcome as judged by the combined incidence of death or chronic lung disease was not different in the two groups (18.6% Exosurf vs. 15.2% Survanta; P > 0.05). When the complications of prematurity were compared, there were no statistically significant differences between the two groups. We conclude that both preparations are reasonable choices for the treatment of respiratory distress syndrome of prematurity.

  2. Neuropathies of Stüve-Wiedemann Syndrome due to mutations in leukemia inhibitory factor receptor (LIFR) gene.

    PubMed

    Oxford, Alexandra E; Jorcyk, Cheryl L; Oxford, Julia Thom

    2016-01-01

    Stüve-Wiedemann syndrome (STWS; OMIM #610559) is a rare disease that results in dysfunction of the autonomic nervous system, which controls involuntary processes such as breathing rate and body temperature. In infants, this can result in respiratory distress, feeding and swallowing difficulties, and hyperthermic episodes. Individuals may sweat excessively when body temperature is not elevated. Additionally, individuals have reduced ability to feel pain and may lose reflexes such as the corneal reflex that normally causes one to blink, and the patellar reflex resulting in the knee-jerk. STWS usually results in infant mortality, yet some STWS patients survive into early adulthood. STWS is caused by a mutation in the leukemia inhibitory factor receptor (LIFR) gene, which is inherited in an autosomal-recessive pattern. Most LIFR mutations resulting in STWS cause instability of the mRNA due to frameshift mutations leading to premature stop codons, which prevent the formation of LIFR protein. STWS is managed on a symptomatic basis as no treatment is currently available.

  3. Superior Vena Cava Syndrome due to Thrombosis: A Rare Paraneoplastic Presentation of Bronchogenic Carcinoma.

    PubMed

    Santra, Avradip; Nandi, Saumen; Mondal, Saibal; Chakraborty, Subhankar

    2016-07-01

    Superior vena cava (SVC) syndrome is not an uncommon occurrence in patients with malignancy and it is often described as a medical emergency. In majority of the cases, SVC syndrome occurs due to mechanical obstruction of the SVC by extraluminal compression with primary intrathoracic malignancies. However, intraluminal obstruction due to thrombosis can also produce symptoms and signs of SVC syndrome. Clot-related SVC obstruction is mostly associated with indwelling central venous catheter and pacemaker leads, although such thrombosis can occur spontaneously in a background of a hypercoagulable state, e.g., malignancy. Here, an unusual case of sudden onset SVC syndrome has been reported, which on initial radiologic evaluation was found to have a lung nodule without any significant mediastinal mass or adenopathy compressing SVC. Subsequent investigation with Doppler ultrasonography of the neck showed thrombosis in the right internal jugular, right subclavian and right brachiocephalic vein, which was responsible for SVC syndrome. Histopathological evaluation of lung nodule confirmed presence of an adenocarcinoma. Therefore, venous thromboembolism as a paraneoplastic syndrome should be kept in mind while evaluating a case of SVC obstruction in a cancer patient. Management of the underlying disease is of prime importance in such cases and anticoagulation is the mainstay of therapy. Ability to identify paraneoplastic syndrome may have a significant effect on clinical outcome, ranging from early diagnosis to improved quality of life of the patient.

  4. Hoyeraal-Hreidarsson Syndrome Due to PARN Mutations: Fourteen Years of Follow-up

    PubMed Central

    Burris, Ashley M.; Ballew, Bari J.; Kentosh, Joshua B.; Turner, Clesson E.; Norton, Scott A.; Giri, Neelam; Alter, Blanche P.; Nellan, Anandani; Gamper, Christopher; Hartman, Kip R.; Savage, Sharon A.

    2015-01-01

    Introduction Hoyeraal-Hreidarsson syndrome is a dyskeratosis congenita-related telomere biology disorder that presents in infancy with intrauterine growth retardation immunodeficiency, and cerebellar hypoplasia in addition to the triad of nail dysplasia, skin pigmentation, and oral leukoplakia. Patients with Hoyeraal-Hreidarsson syndrome often develop bone marrow failure in early childhood. Germline mutations in DKC1, TERT, TINF2, RTEL1, ACD, and PARN cause about 60% of Hoyeraal-Hreidarsson syndrome cases. Case Report We report 14 years of follow-up for a patient with Hoyeraal-Hreidarsson syndrome who initially presented as an infant with intrauterine growth retardation, microcephaly, and central nervous system calcifications. He was diagnosed with Hoyeraal-Hreidarsson syndrome at age six and had a complicated medical history including severe developmental delay, cerebellar hypoplasia, esophageal and urethral stenosis, hip avascular necrosis, immunodeficiency, and bone marrow failure evolving to myelodysplastic syndrome requiring hematopoietic cell transplantation at age 14. He had progressive skin pigmentation changes, oral leukoplakia, and nail dysplasia leading to anonychia. Whole exome sequencing identified novel biallelic variants in PARN. Conclusions This case illustrates that the constellation of IUGR, central nervous system calcifications, and cerebellar hypoplasia, esophageal or urethral stenosis, and cytopenias, in the absence of congenital infection, may be due to Hoyeraal-Hreidarsson syndrome. Early diagnosis of Hoyeraal-Hreidarsson syndrome is important to optimize medical management and provide genetic counseling. PMID:26810774

  5. Steven Johnson syndrome due to I.V Ceftriaxone--a case report.

    PubMed

    Narayanan, Veena S; Mamatha, G P; Ashok, L; Rajashekar, Nadig

    2003-01-01

    Steven-Johnson syndrome (SJS) is a rare vesiculobullous disease characterized by an acute cutaneous eruption that ivolves the skin and mucous membranes including those of the oral cavity. A rare case of Steven-Johnson syndrome, an unexpected treatment response, in a 25-year-old female patient due to administration of intravenous Cefriaxone (1 gm), a third generation cephalosporin has been reported and literature reviewed.

  6. Lethal Keratitis, Ichthyosis, and Deafness Syndrome Due to the A88V Connexin 26 Mutation.

    PubMed

    Esmer, Carmen; Salas-Alanis, Julio C; Fajardo-Ramirez, Oscar R; Ramírez, Brenda; Hua, Rong; Choate, Keith

    2016-01-01

    Keratitis-ichthyosis-deafness syndrome is a well-characterized disease that has been related to mutations in the GJB6 gene. Clinical features such as erythrokeratoderma, palmoplantar keratoderma, alopecia, and progressive vascularizing keratitis, among others, are well known in this entity. In this report we describe a newborn female patient diagnosed with keratitis-ichthyosis-deafness syndrome with a lethal outcome due to sepsis. The patient harbored the mutation A88V that has been previously reported in lethal cases.

  7. A case of squamous cell carcinoma of the skin due to the molecularly confirmed Lynch Syndrome.

    PubMed

    Sorscher, Steven

    2015-01-01

    Patients with Lynch Syndrome are at high risk for developing a variety of cancers including cancers of the colon or rectum, small bowel, stomach, uterus, renal pelvis, ureter, biliary tract, ovaries, brain and pancreas (N Engl J Med 348: 919-32, 2003; Gut 57:1097-101, 2008; NCCN, Inc Guideline. Ft. Washington, PA. Online Version 2.2014). Lack of MLH-1 and MSH-2 expression commonly result from germline mutations in this inherited cancer syndrome. Here, we report the case of a patient with a molecularly confirmed germline mutation in MLH-1 along with a colon cancer showing lack of expression of MLH-1 as well as a squamous cell cancer of the skin from the abdominal wall also demonstrating lack of expression of MLH-1. This case appears to represent the second case report of a squamous cell skin cancer apparently due to the Lynch Syndrome and further supports a proposed relationship between Lynch Syndrome and these tumors.

  8. Formation of a chronic pain syndrome due to mesh shrinkage after laparoscopic intraperitoneal onlay mesh (IPOM).

    PubMed

    Klein, Fritz; Ospina, Carlos; Rudolph, Birgit; Wüstefeld, Joost; Denecke, Timm; Neuhaus, Peter; Schmidt, Sven-Christian

    2012-10-01

    The case of a 58-year-old male patient who developed a chronic pain syndrome after laparoscopic intraperitoneal onlay mesh for treatment of a large symptomatic umbilical hernia combined with rectus diastasis is reported. Twelve months after an uncomplicated initial surgery, the patient presented with progressive signs of a foreign body sensation and pain in the anterior abdominal wall. Computed tomography examination revealed no pathologic findings but a marked shrinkage of the mesh implant. Because of further progressive symptoms, explorative laparotomy was performed. Mesh shrinkage and adhesions with a surrounding chronic tissue reaction were found as the cause of the pain syndrome. This case demonstrates a case of a chronic pain syndrome due to mesh shrinkage 12 months after initial ventral hernia repair. Mesh shrinkage should therefore be taken into consideration in patients with progressive pain chronic syndromes after laparoscopic ventral hernia repair.

  9. A case of orbital apex syndrome due to Pseudomonas aeruginosa infection.

    PubMed

    Kusunoki, Takeshi; Kase, Kaori; Ikeda, Katsuhisa

    2011-09-28

    Orbital apex syndrome is commonly been thought to have a poor prognosis. Many cases of this syndrome have been reported to be caused by paranasal sinus mycosis. We encountered a very rare case (60-year-old woman) of sinusitis with orbital apex syndrome due to Pseudomonas aeruginosa infection. She had received insulin and dialysis for diabtes and diabetic nephropathy, moreover anticoagulants after heart by-pass surgery. She underwent endoscopic sinus operation and was treated with antibiotics, but her loss of left vision did not improve. Recently, sinusitis cases due to Pseudomonas aeruginosa were reported to be a increasing. Therefore, we should consider the possibility of Pseudomonas aeruginosa as well as mycosis as infections of the sinus, especially inpatients who are immunocompromised body.

  10. A case of orbital apex syndrome due to Pseudomonas aeruginosa infection

    PubMed Central

    Kusunoki, Takeshi; Kase, Kaori; Ikeda, Katsuhisa

    2011-01-01

    Orbital apex syndrome is commonly been thought to have a poor prognosis. Many cases of this syndrome have been reported to be caused by paranasal sinus mycosis. We encountered a very rare case (60-year-old woman) of sinusitis with orbital apex syndrome due to Pseudomonas aeruginosa infection. She had received insulin and dialysis for diabtes and diabetic nephropathy, moreover anticoagulants after heart by-pass surgery. She underwent endoscopic sinus operation and was treated with antibiotics, but her loss of left vision did not improve. Recently, sinusitis cases due to Pseudomonas aeruginosa were reported to be a increasing. Therefore, we should consider the possibility of Pseudomonas aeruginosa as well as mycosis as infections of the sinus, especially inpatients who are immunocompromised body. PMID:24765368

  11. Unusual Cushing's Syndrome and Hypercalcitoninaemia due to a Small Cell Prostate Carcinoma

    PubMed Central

    Magnani, Elena; Nuzzo, Fiorella

    2016-01-01

    A 75-year-old man was hospitalized because of severe hypokalaemia due to ACTH dependent Cushing's syndrome. Total body computed tomography (TBCT) and 68 Gallium DOTATATE PET/CT localized a voluminous prostate tumour. A subsequent transurethral prostate biopsy documented a small cell carcinoma positive for ACTH and calcitonin and negative for prostatic specific antigen (PSA) at immunocytochemical study; serum prostatic specific antigen (PSA) was normal. Despite medical treatments, Cushing's syndrome was not controlled and the patient's clinical condition progressively worsened. Surgical resection was excluded; the patient underwent a cycle of chemotherapy followed by febrile neutropenia and fatal intestinal perforation. This case report describes a rare case of Cushing's syndrome and hypercalcitoninaemia due to a small cell carcinoma of the prostate, a rare tumour with very few therapeutic options and negative prognosis. PMID:28044110

  12. Use of an oscillatory PEP device to enhance bronchial hygiene in a patient of post-H1NI pneumonia and acute respiratory distress syndrome with pneumothorax.

    PubMed

    Narula, Deepali; Nangia, Vivek

    2014-03-07

    A 26-year-old, 14 week pregnant woman was admitted to our hospital with pneumonia with acute respiratory distress syndrome in an intubated and mechanically ventilated state. She was diagnosed to have polymicrobial infection and left-sided pneumothorax and was put on a ventilator for 2 weeks. Postextubation, she found it difficult to clear her respiratory secretions despite aggressive routine chest physiotherapy. She was planned to undergo a mini-tracheostomy for tracheobronchial toileting. However, before that, she was given a trial of Acapella, a hand-held oscillatory positive expiratory pressure (OPEP) therapy device, for facilitating airway clearance, with the aim to speed up the recovery. The patient found it easy to use and clear the secretions optimally, thus averting a mini-tracheostomy. This case report highlights the advantages of the OPEP therapy device in effective management of bronchial hygiene in patients with poor respiratory effort.

  13. Use of an oscillatory PEP device to enhance bronchial hygiene in a patient of post-H1NI pneumonia and acute respiratory distress syndrome with pneumothorax

    PubMed Central

    Narula, Deepali; Nangia, Vivek

    2014-01-01

    A 26-year-old, 14 week pregnant woman was admitted to our hospital with pneumonia with acute respiratory distress syndrome in an intubated and mechanically ventilated state. She was diagnosed to have polymicrobial infection and left-sided pneumothorax and was put on a ventilator for 2 weeks. Postextubation, she found it difficult to clear her respiratory secretions despite aggressive routine chest physiotherapy. She was planned to undergo a mini-tracheostomy for tracheobronchial toileting. However, before that, she was given a trial of Acapella, a hand-held oscillatory positive expiratory pressure (OPEP) therapy device, for facilitating airway clearance, with the aim to speed up the recovery. The patient found it easy to use and clear the secretions optimally, thus averting a mini-tracheostomy. This case report highlights the advantages of the OPEP therapy device in effective management of bronchial hygiene in patients with poor respiratory effort. PMID:24717858

  14. Right heart failure in acute respiratory distress syndrome: An unappreciated albeit a potential target for intervention in the management of the disease.

    PubMed

    Biswas, Abhishek

    2015-10-01

    Mortality from acute respiratory distress syndrome (ARDS) has gone down recently. In spite of this trend, the absolute numbers continue to be high even with improvements in ventilator strategies and a better understanding of fluid management with this disease. A possible reason for this could be an under-recognized involvement of the pulmonary vasculature and the right side of the heart in ARDS. The right heart is not designed to function under situations leading to acute elevations in afterload as seen in ARDS, and hence it decompensates. This brief review focuses on the magnitude of the problem, its detection in the intensive care unit, and recognizes the beneficial effect of prone-positioning on the pulmonary vasculature and right heart.

  15. High-volume hemofiltration and prone ventilation in subarachnoid hemorrhage complicated by severe acute respiratory distress syndrome and refractory septic shock

    PubMed Central

    Cornejo, Rodrigo; Romero, Carlos; Ugalde, Diego; Bustos, Patricio; Diaz, Gonzalo; Galvez, Ricardo; Llanos, Osvaldo; Tobar, Eduardo

    2014-01-01

    We report the successful treatment of two patients with aneurismal subarachnoid hemorrhage complicated by severe respiratory failure and refractory septic shock using simultaneous prone position ventilation and high-volume hemofiltration. These rescue therapies allowed the patients to overcome the critical situation without associated complications and with no detrimental effects on the intracranial and cerebral perfusion pressures. Prone position ventilation is now an accepted therapy for severe acute respiratory distress syndrome, and high-volume hemofiltration is a non-conventional hemodynamic support that has several potential mechanisms for improving septic shock. In this manuscript, we briefly review these therapies and the related evidence. When other conventional treatments are insufficient for providing safe limits of oxygenation and perfusion as part of basic neuroprotective care in subarachnoid hemorrhage patients, these rescue therapies should be considered on a case-by-case basis by an experienced critical care team. PMID:25028955

  16. "Dropped-head" syndrome due to isolated myositis of neck extensor muscles: MRI findings.

    PubMed

    Gaeta, Michele; Mazziotti, Silvio; Toscano, Antonio; Rodolico, Carmelo; Mazzeo, Anna; Blandino, Alfredo

    2006-02-01

    MRI findings of a patient with dropped-head syndrome due to focal myositis of the neck extensor muscles are presented. MRI showed oedematous changes and marked enhancement of the neck extensor muscles. After therapy MRI demonstrated disappearance of the abnormal findings.

  17. Chronic Pelvic Pain due to Pelvic Congestion Syndrome: The Role of Diagnostic and Interventional Radiology

    SciTech Connect

    Ganeshan, Arul; Upponi, Sara; Hon, Lye-Quen; Uthappa, M. C.; Warakaulle, Dinuke R.; Uberoi, Raman

    2007-11-15

    Chronic pelvic pain (CPP) is a common cause of gynecologic referral. Pelvic congestion syndrome, which is said to occurs due to ovarian vein incompetence, is a recognized cause of CPP. The aim of this paper is to briefly describe the clinical manifestations, and to review the role of diagnostic and interventional radiology in the management of this probably under-diagnosed condition.

  18. Opsoclonus myoclonus ataxia syndrome due to falciparum malaria in two Indian children

    PubMed Central

    Bose, Kallol; Saha, Sudip; Islam, Md. Rahiul; Chakraborty, Chayan; Laskar, Mustakim

    2016-01-01

    Opsoclonus-myoclonus ataxia (OMA) syndrome is rare in children, mostly caused by neuroblastoma. Here, we present two very rare cases presenting with OMA due to falciparum malaria. Both of them responded to a high dose of adrenocorticotrophin hormone and intravenous immunoglobulin without recurrence and complication. PMID:27958213

  19. Opsoclonus myoclonus ataxia syndrome due to falciparum malaria in two Indian children.

    PubMed

    Bose, Kallol; Saha, Sudip; Islam, Md Rahiul; Chakraborty, Chayan; Laskar, Mustakim

    2016-11-01

    Opsoclonus-myoclonus ataxia (OMA) syndrome is rare in children, mostly caused by neuroblastoma. Here, we present two very rare cases presenting with OMA due to falciparum malaria. Both of them responded to a high dose of adrenocorticotrophin hormone and intravenous immunoglobulin without recurrence and complication.

  20. Central nervous system infection due to Mycobacterium haemophilum in a patient with acquired immunodeficiency syndrome.

    PubMed

    Buppajarntham, Aubonphan; Apisarnthanarak, Anucha; Rutjanawech, Sasinuj; Khawcharoenporn, Thana

    2015-03-01

    Mycobacterium haemophilum is an environmental organism that rarely causes infections in humans. We report a patient with acquired immunodeficiency syndrome who had central nervous system infection due to M. haemophilum. The diagnosis required brain tissue procurement and molecular identification method while the treatment outcome was unfavourable.

  1. Respiratory distress of the term newborn infant.

    PubMed

    Edwards, Martin O; Kotecha, Sarah J; Kotecha, Sailesh

    2013-03-01

    Respiratory distress is recognised as any signs of breathing difficulties in neonates. In the early neonatal period respiratory distress is common, occurring in up to 7% of newborn infants, resulting in significant numbers of term-born infants being admitted to neonatal units. Many risk factors are involved; the increasing number of term infants delivered by elective caesarean section has also increased the incidence. Additionally the risk decreases with each advancing week of gestation. At 37 weeks, the chances are three times greater than at 39-40 weeks gestation. Multiple conditions can present with features of respiratory distress. Common causes in term newborn infants include transient tachypnoea of the newborn, respiratory distress syndrome, pneumonia, meconium aspiration syndrome, persistent pulmonary hypertension of the neonate and pneumothorax. Early recognition of respiratory distress and initiation of appropriate treatment is important to ensure optimal outcomes. This review will discuss these common causes of respiratory distress in term-born infants.

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

    PubMed Central

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

    2013-01-01

    Takotsubo syndrome is characterized by predominantly medial-apical transient left ventricular dysfunction, which is typically triggered by physical or emotional stress. The present article reports the case of a 61-year-old female patient presenting with dizziness, excessive sweating, and sudden state of ill feeling following an episode involving intense emotional stress. The physical examination and electrocardiogram were normal upon admission, but the troponin I and creatine kinase-MB concentrations were increased. Acute myocardial infarction without ST segment elevation was suspected, and coronary angiography was immediately performed, which showed severe diffuse left ventricular hypokinesia, medial-apical systolic ballooning, and a lack of significant coronary injury. The patient was referred to the intensive care unit and was successfully treated with supportive therapy. As this case shows, Takotsubo syndrome might simulate the clinical manifestations of acute myocardial infarction, and coronary angiography is necessary to distinguish between both myocardial infarction and myocardial infarction in the acute stage. The present patient progressed with spontaneous resolution of the ventricular dysfunction without any sequelae. PMID:23887762

  3. Magnetic resonance diagnosis of tarsal tunnel syndrome due to flexor digitorum accessorius longus and peroneocalcaneus internus muscles.

    PubMed

    Duran-Stanton, Amelia M; Bui-Mansfield, Liem T

    2010-01-01

    Anomalous muscles of the ankle are common. Although they are often asymptomatic, they can sometimes cause tarsal tunnel syndrome. We report a case of tarsal tunnel syndrome due to flexor digitorum accessorius longus and peroneocalcaneus internus muscles diagnosed on magnetic resonance imaging. Recognition of the most common accessory muscles of the ankle on magnetic resonance imaging and tarsal tunnel syndrome are also reviewed.

  4. Characteristics of microRNAs and their potential relevance for the diagnosis and therapy of the acute respiratory distress syndrome: from bench to bedside.

    PubMed

    Cardinal-Fernández, Pablo; Ferruelo, Antonio; Esteban, Andrés; Lorente, José A

    2016-03-01

    Acute respiratory distress syndrome (ARDS) is a complex disease associated with high morbidity and mortality. Biomarkers and specific pharmacologic treatment of the syndrome are lacking. MicroRNAs (miRNAs) are small (∼ 19-22 nucleotides) noncoding RNA molecules whose function is the regulation of gene expression. Their uncommon biochemical characteristics (eg, their resistance to degradation because of extreme temperature and pH fluctuations, freeze-thaw cycles, long storage times in frozen conditions, and RNAse digestion) and their presence in a wide range of different biological fluids and the relatively low number of individual miRNAs make these molecules good biomarkers in different clinical conditions. In addition, miRNAs are suitable therapeutic targets as their expression can be modulated by different available strategies. The aim of the present review is to offer clinicians a global perspective of miRNA, covering their structure and nomenclature, biogenesis, effects on gene expression, regulation of expression, and features as disease biomarkers and therapeutic targets, with special attention to ARDS. Because of the early stage of research on miRNAs applied to ARDS, attention has been focused on how knowledge sourced from basic and translational research could inspire future clinical studies.

  5. Acute carotid occlusion and stroke due to antiphospholipid antibody syndrome: case report and literature review.

    PubMed

    Marques, M A; Murad, F F; Ristow, A V; Silveira, P R M; Pinto, J E Ss; Gress, M H T; Massière, B; Cury, J M; Vescovi, A

    2010-08-01

    Neurologic events associated to antiphospholipoid syndrome (APLS) are not uncommon, but ischemic stroke due to acute carotid thrombosis, is a rare presentation of this syndrome. We report a case of a 48 years old female patient, without evidence of atherothrombosis or other vascular pathology, who presented an ischemic stroke due to acute thrombosis of the left internal carotid artery. The occlusion was diagnosed by Duplex scan and magnetic resonance angiography (Ds+MRA). The patient was anticoagulated and experienced total regression of her neurologic symptoms after a week. Ds+MRA were performed again and confirmed re-establishment of normal flow of internal carotid artery. A thorough clinical investigation confirmed the diagnosis of APLS (the association of a major thombotic event and high anticardiolipoid IgG antibody titers in three blood samples). The patient has been submitted to oral anticoagulation for three years and has not experienced new neurologic or thrombotic events.

  6. Physiological, Biochemical, and Biophysical Characterization of the Lung-Lavaged Spontaneously-Breathing Rabbit as a Model for Respiratory Distress Syndrome

    PubMed Central

    Ricci, Francesca; Catozzi, Chiara; Murgia, Xabier; Rosa, Brenda; Amidani, Davide; Lorenzini, Luca; Bianco, Federico; Rivetti, Claudio; Catinella, Silvia; Villetti, Gino; Civelli, Maurizio; Pioselli, Barbara; Dani, Carlo; Salomone, Fabrizio

    2017-01-01

    Nasal continuous positive airway pressure (nCPAP) is a widely accepted technique of non-invasive respiratory support in spontaneously-breathing premature infants with respiratory distress syndrome (RDS). Surfactant administration techniques compatible with nCPAP ventilation strategy are actively investigated. Our aim is to set up and validate a respiratory distress animal model that can be managed on nCPAP suitable for surfactant administration techniques studies. Surfactant depletion was induced by bronchoalveolar lavages (BALs) on 18 adult rabbits. Full depletion was assessed by surfactant component analysis on the BALs samples. Animals were randomized into two groups: Control group (nCPAP only) and InSurE group, consisting of a bolus of surfactant (Poractant alfa, 200 mg/kg) followed by nCPAP. Arterial blood gases were monitored until animal sacrifice, 3 hours post treatment. Lung mechanics were evaluated just before and after BALs, at the time of treatment, and at the end of the procedure. Surfactant phospholipids and protein analysis as well as surface tension measurements on sequential BALs confirmed the efficacy of the surfactant depletion procedure. The InSurE group showed a significant improvement of blood oxygenation and lung mechanics. On the contrary, no signs of recovery were appreciated in animals treated with just nCPAP. The surfactant-depleted adult rabbit RDS model proved to be a valuable and efficient preclinical tool for mimicking the clinical scenario of preterm infants affected by mild/moderate RDS who spontaneously breathe and do not require mechanical ventilation. This population is of particular interest as potential target for the non-invasive administration of surfactant. PMID:28060859

  7. Acute lung injury and acute respiratory distress syndrome requiring tracheal intubation and mechanical ventilation in the intensive care unit: impact on managing uncertainty for patient-centered communication.

    PubMed

    Johnson, Robert F; Gustin, Jillian

    2013-09-01

    A case of acute lung injury (ALI) progressing to acute respiratory distress syndrome (ARDS) requiring tracheal intubation and mechanical ventilation (ETMV) is presented. The palliative medicine service was asked to address concerns expressed by the patient's spouse reflecting uncertainty regarding outcome expectations. Acknowledging and confronting the uncertainties of a critical illness is an essential component of patient-centered communication. Addressing and managing uncertainty for the case scenario requires consideration of both short- and long-term outcomes including mortality, ventilator independence, and adverse effects on quality of life for survivors. In this paper, ALI/ARDS requiring ETMV in the ICU was used as a focal point for preparing a prognostic assessment incorporating these issues. This assessment was based on a review of recently published literature regarding mortality and ventilator independence of survivors for adult patients receiving ETMV for ALI/ARDS in the ICU. In the studies reviewed, long-term survival reported at 60 days to 1 year was 50-73% with greater than 84% of the survivors in each study breathing independently. Selected articles discussing outcomes other than mortality or recovery of respiratory function, particularly quality of life implications for ALI/ARDS survivors, were also reviewed. A case of of ALI/ARDS requiring ETMV in the ICU is used to illustrate the situation of an incapacitated critically ill patient where the outcome is uncertain. Patient-centered communication should acknowledge and address this uncertainty. Managing uncertainty consists of effectively expressing a carefully formulated prognostic assessment and using sound communication principles to alleviate the distress associated with the uncertain outcome probabilities.

  8. Respiratory Distress

    NASA Technical Reports Server (NTRS)

    1976-01-01

    The University of Miami School of Medicine asked the Research Triangle Institute for assistance in improvising the negative pressure technique to relieve respiratory distress in infants. Marshall Space Flight Center and Johnson Space Center engineers adapted this idea to the lower-body negative-pressure system seals used during the Skylab missions. Some 20,000 babies succumb to respiratory distress in the U.S. each year, a condition in which lungs progressively lose their ability to oxygenate blood. Both positive and negative pressure techniques have been used - the first to force air into lungs, the second to keep infant's lungs expanded. Negative pressure around chest helps the baby expand his lungs and maintain proper volume of air. If doctors can keep the infant alive for four days, the missing substance in the lungs will usually form in sufficient quantity to permit normal breathing. The Skylab chamber and its leakproof seals were adapted for medical use.

  9. Compartment syndrome due to extravasation of peripheral parenteral nutrition: extravasation injury of parenteral nutrition

    PubMed Central

    Park, Huee Jin; Kim, Kyung Hoon; Lee, Hyuk Jin; Jeong, Eui Cheol; Kim, Kee Won

    2015-01-01

    Compartment syndrome is a rare but devastating condition that can result in permanent neuromuscular or soft tissue injuries. Extravasation injuries, among the iatrogenic causes of compartment syndrome, occur under a wide variety of circumstances in the inpatient setting. Total parenteral nutrition via a peripheral route is an effective alternative for the management of critically ill children who do not obtain adequate nutrition via the oral route. However, there is an inherent risk of extravasation, which can cause compartment syndrome, especially when detected at a later stage. Herein, we report a rare case of compartment syndrome and skin necrosis due to extravasation, requiring emergency fasciotomy and skin graft in a 7-month-old boy who was treated with peripheral parenteral nutrition via a pressurized infusion pump. Although we cannot estimate the exact time at which extravasation occurred, the extent and degree of the wound suggest that the ischemic insult was prolonged, lasting for several hours. Pediatric clinicians and medical teams should carefully examine the site of insertion of the intravenous catheter, especially in patients receiving parenteral nutrition via a peripheral intravenous catheter with a pressurized infusion pump. PMID:26692883

  10. Budd-chiari syndrome and renal arterial neurysms due to Behcet disease: a rare association.

    PubMed

    Batur, Abdussamet; Dorum, Meltem; Yüksekkaya, Hasan Ali; Koc, Osman

    2015-01-01

    Behcet's disease is a multisystemic vasculitis of unknown etiology with a chronic relapsing course. Vasculitis in Behcet's disease with predominant vascular involvement is the only vasculitis that affects both arteries and veins of any size. Involvement of the renal artery and inferior vena cava is rare among the arteries and veins, respectively. When disease affect the veins, it is in the form of thrombosis. Arterial complications include aneurysms, stenosis and occlusions. Both rupture of arterial aneurysm and occlusion of suprahepatic veins, causing Budd-Chiari syndrome, are associated with a high mortality rate. Vascular involvement is more common in male patients than in female patients. Men and patients with a younger age of onset present with a more severe prognosis. In this case report, we describe a very rare cause of intrarenal arterial aneurysm's rupture with previous Budd-Chiari syndrome due to Behcet's disease and successful angiographic embolization of actively bleeding aneurysm.

  11. Dropped head syndrome due to myogenic atrophy - a case report of surgical treatment.

    PubMed

    Zenmyo, Michihisa; Abematsu, Masahiko; Yamamoto, Takuya; Ishidou, Yasuhiro; Komiya, Setsuro; Ijiri, Kosei

    2011-01-19

    We report a case of a 69-year-old man with dropped head syndrome associated with isolated neck extensor myopathy (INEM). Over a period of 2 years, he exhibited progressive inability to lift his chin off his chest, resulting in the dropped head position that impaired his activities of daily living. He had a disturbed gait with severe imbalance of spinal alignment. Computed tomography revealed osseous contracture of cervical vertebral bodies in flexed position. Anterior combined posterior reconstruction surgery yielded a successful outcome in his activities of daily living, including his walking balance of spinal alignment. Pathologic study confirmed myogenic atrophy in the cervical extensor muscles. We suggest that consideration for surgical management should be given to dropped head syndrome especially due to INEM.

  12. Multiple osteoblastomas in a child with Cushing syndrome due to bilateral adrenal micronodular hyperplasias

    PubMed Central

    Yu, Hyeoh Won; Cho, Won Im; Choi, Keun Hee; Yun, Sumi; Cho, Hwan Seong; Shin, Choong Ho; Yang, Sei Won

    2016-01-01

    Adrenocorticotropin-independent adrenal hyperplasias are rare diseases, which are classified into macronodular (>1 cm) and micronodular (≤1 cm) hyperplasia. Micronodular adrenal hyperplasia is subdivided into primary pigmented adrenocortical disease and a limited or nonpigmented form 'micronodular adrenocortical disease (MAD)', although considerable morphological and genetic overlap is observed between the 2 groups. We present an unusual case of a 44-month-old girl who was diagnosed with Cushing syndrome due to MAD. She had presented with spotty pigmentation on her oral mucosa, lips and conjunctivae and was diagnosed with multiple bone tumors in her femur, pelvis and skull base at the age of 8 years. Her bone tumor biopsies were compatible with osteoblastoma. This case highlights the importance of verifying the clinicopathologic correlation in Cushing syndrome and careful follow-up and screening for associated diseases. PMID:27104180

  13. Pathologic Femoral Neck Fracture Due to Fanconi Syndrome Induced by Adefovir Dipivoxil Therapy for Hepatitis B

    PubMed Central

    Lee, Yoon-Suk; Kim, Byung-Kook; Lee, Ho-Jae

    2016-01-01

    In Fanconi syndrome, hypophosphatemic osteomalacia is caused by proximal renal tubule dysfunction which leads to impaired reabsorption of amino acids, glucose, urate, and phosphate. We present a rare case of a 43-year-old Korean male who was found to have insufficiency stress fracture of the femoral neck secondary to osteomalacia due to Fanconi syndrome. He had been receiving low-dose adefovir dipivoxil (ADV, 10 mg/day) for the treatment of chronic hepatitis B virus infection for 7 years and he subsequently developed severe hypophosphatemia and proximal renal tubule dysfunction. The incomplete femoral neck fracture was fixed with multiple cannulated screws to prevent further displacement of the initial fracture. After cessation of ADV and correction of hypophosphatemia with oral phosphorus supplementation, the patient's clinical symptoms, such as bone pain, muscle weakness, and laboratory findings improved. PMID:27247753

  14. [Case report of recurrent hydrocephalus due to uveo-meningeal syndrome].

    PubMed

    Yamamoto, Yayoi; Suzuki, Kensuke; Yamazaki, Tomosato; Takano, Shingo; Matsumura, Akira

    2007-10-01

    A 43-year-old woman suffered from bilateral uveitis and was suspected of sarcoidosis. General fatigue slowly progressed, and urinary incontinence and disturbance of consciousness also developed. When she was admitted to our hospital by ambulance, her conciousness was disturbed, GCS 3-4-6. Enlargement of all ventricles was shown and cell count was moderately elevated. She underwent VP shunt and fully recovered. However, her activity become disturbed again and, after five months, only her left lateral ventricle dilated. We presumed it was caused by the occlusion of the foramen of Monro. Neuroendoscopic inspection of the right lateral ventricle via a left precoronal burrhole revealed a thick, yellish membrane that occluded the foramen of Monro. She received a left VP shunt and cured. She was diagnosed as having recurrent hydrocepalus due to the uveo-meningeal syndrome. We present this first case and discuss this syndrome associated hydrocephalus.

  15. Analysis of risk factors for and the prognosis of postoperative acute respiratory distress syndrome in patients with Stanford type A aortic dissection

    PubMed Central

    Chen, Mei-Fang; Cao, Hua; Lin, Yong

    2016-01-01

    Background To explore the risk factors for and the prognosis of postoperative acute respiratory distress syndrome (ARDS) in patients with Stanford type A aortic dissection (AD). Methods This retrospective nested case-control study included 527 Stanford type A AD patients who were divided into ARDS groups and non-ARDS groups. The clinical features of the groups were examined. Results The fifty-nine patients in the ARDS group exhibited extended durations of cardiopulmonary bypass (CPB) (P=0.004), deep hypothermic circulatory arrest (DHCA) (P=0.000), ventilator support (P=0.013) and intensive care unit (ICU) stay (P=0.045), higher hospital costs (P=0.000), larger perioperative transfusions volumes [red blood cells (RBC): P=0.002, platelets (PLT): P=0.040, fresh frozen plasma (FFP): P=0.001], more frequent pulmonary infection (P=0.018) and multiple organ dysfunction syndrome (MODS) (P=0.040) and a higher rate of in-hospital mortality (P=0.020). The ARDS group exhibited worse statuses in terms of oxygenation index (OI) values (P=0.000) and Apache II scores (P=0.000). DHCA [P=0.000, odds ratio (OR) =2.589] and perioperative transfusion (RBC: P=0.000, OR =2.573; PLT: P=0.027, OR =1.571; FFP: P=0.002, OR =1.929) were independent risk factors for postoperative ARDS. The survival rates and median survival times after discharge were similar between the two groups (P=0.843). Conclusions DHCA duration and perioperative transfusion volume were independent risk factors for postoperative ARDS which warrants greater attention by the cardiac surgeons. PMID:27867562

  16. ADMINISTRATION OF ANTENATAL GLUCOCORTICOIDS AND POSTNATAL SURFACTANT AMELIORATES RESPIRATORY DISTRESS SYNDROME-ASSOCIATED NEONATAL LETHALITY IN ERK3−/− PUPS

    PubMed Central

    Guaman, Milenka Cuevas; Sbrana, Elena; Shope, Cynthia; Showalter, Lori; Hu, Min; Meloche, Sylvain; Aagaard, Kjersti

    2014-01-01

    BACKGROUND Respiratory distress syndrome (RDS) persists as a prevalent cause of infant morbidity and mortality. We have previously demonstrated that deletion of Erk3 results in pulmonary immaturity and neonatal lethality. Using RNA-Seq, we identified corticotrophin releasing hormone (CRH) and surfactant protein B (SFTPB) as potential molecular mediators of Erk3-dependent lung maturation. In this study, we characterized the impact of antenatal glucocorticoids and postnatal surfactant on neonatal survival of Erk3 null mice. METHODS In a double crossover design, we administered dexamethasone (dex) or saline to pregnant dams during the saccular stage of lung development, followed by postnatal surfactant or saline via inhalation intubation. Survival was recorded, detailed lung histological analysis and staining for CRH and SFTPB protein expression was performed. RESULTS Without treatment, Erk3 null pups die within 6 hours of birth with reduced aerated space, impaired thinning of the alveolar septa, and abundant PAS-positive glycogen stores; as described in human RDS. The administration of dex and surfactant improved RDS-associated lethality of Erk3−/− pups, and partially restored functional fetal lung maturation by accelerating the down-regulation of pulmonary CRH and partially rescuing production of SFTPB. CONCLUSION These findings emphasize that Erk3 is integral to terminal differentiation of type II cells, SFTPB production, and fetal pulmonary maturity. PMID:24732107

  17. Improved arterial oxygenation with biologically variable or fractal ventilation using low tidal volumes in a porcine model of acute respiratory distress syndrome.

    PubMed

    Boker, Abdulaziz; Graham, M Ruth; Walley, Keith R; McManus, Bruce M; Girling, Linda G; Walker, Elizabeth; Lefevre, Gerald R; Mutch, W Alan C

    2002-02-15

    We compared biologically variable ventilation (V (bv); n = 9) with control mode ventilation (V (c); n = 8) at low tidal volume (VT)--initial 6 ml/kg--in a porcine model of acute respiratory distress syndrome (ARDS). Hemodynamics, respiratory gases, airway pressures, and VT data were measured. Static P-V curves were generated at 5 h. Interleukin (IL)-8 and IL-10 were measured in serum and tracheal aspirate. By 5 h, higher Pa(O(2)) (173 +/- 30 mm Hg versus 119 +/- 23 mm Hg; mean +/- SD; p < 0.0001 group x time interaction [G x T]), lower shunt fraction (6 +/- 1% versus 9 +/- 3%; p = 0.0026, G x T) at lower peak airway pressure (21 +/- 2 versus 24 +/- 1 cm H(2)O; p = 0.0342; G x T) occurred with V (bv). IL-8 concentrations in tracheal aspirate and wet:dry weight ratios were inversely related; p = 0.011. With V (c), IL-8 concentrations were 3.75-fold greater at wet:dry weight ratio of 10. IL-10 concentrations did not differ between groups. In both groups, ventilation was on the linear portion of the P-V curve. With V (bv), VT variability demonstrated an inverse power law indicating fractal behavior. In this model of ARDS, V (bv) improved Pa(O(2)) at lower peak airway pressure and IL-8 levels compared with V (c).

  18. Optimization of Positive End-Expiratory Pressure targeting the best arterial oxygen transport in the Acute Respiratory Distress Syndrome: the OPTIPEP study.

    PubMed

    Chimot, Loïc; Fedun, Yannick; Gacouin, Arnaud; Campillo, Boris; Marqué, Sophie; Gros, Antoine; Delour, Pierre; Bedon-Carte, Sandrine; Le Tulzo, Yves

    2016-12-13

    The optimal setting for positive end-expiratory pressure (PEEP) in mechanical ventilation remains controversial in the treatment of acute respiratory distress syndrome (ARDS). The aim of this study was to determine the optimum PEEP level in ARDS, which we defined as the level that allowed the best arterial oxygen delivery (DO2).We conducted a physiological multicenter prospective study on patients who suffering from ARDS according to standard definition and persistent after 6 hours of ventilation. The PEEP was set to 6 cmH2O at the beginning of the test and then was increased by 2 cmH2O after at least 15 min of being stabilized until the plateau pressure achieved 30 cmH2O. At each step, the cardiac output was measured by trans-esophageal echocardiography and gas blood was sampled.We were able to determine the optimal PEEP for twelve patients. The ratio of PaO2/FiO2 at inclusion was 131±40 with a mean FiO2 of 71±3%. The optimal PEEP level was lower than the higher PEEP despite a constant increase in SaO2. The optimal PEEP levels varied between 8 and 18 cmH2O.Our results show that in ARDS patients the optimal PEEP differs between each patient and require being determined with monitoring.

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

    PubMed Central

    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

    2014-01-01

    Background: 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. Methods: 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. Results: 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 (I2 < 50%) for most of the clinical and physiologic outcomes. Interpretation: Our analysis of high-quality evidence showed that use of the prone position during mechanical ventilation improved survival among patients with ARDS who received protective lung ventilation. PMID:24863923

  20. The Protective Effects of HJB-1, a Derivative of 17-Hydroxy-Jolkinolide B, on LPS-Induced Acute Distress Respiratory Syndrome Mice.

    PubMed

    Xu, Xiaohan; Liu, Ning; Zhang, Yu-Xin; Cao, Jinjin; Wu, Donglin; Peng, Qisheng; Wang, Hong-Bing; Sun, Wan-Chun

    2016-01-11

    Acute respiratory distress syndrome (ARDS),which is inflammatory disorder of the lung, which is caused by pneumonia, aspiration of gastric contents, trauma and sepsis, results in widespread lung inflammation and increased pulmonary vascular permeability. Its pathogenesis is complicated and the mortality is high. Thus, there is a tremendous need for new therapies. We have reported that HJB-1, a 17-hydroxy-jolkinolide B derivative, exhibited strong anti-inflammatory effects in vitro. In this study, we investigated its impacts on LPS-induced ARDS mice. We found that HJB-1 significantly alleviated LPS-induced pulmonary histological alterations, inflammatory cells infiltration, lung edema, as well as the generation of inflammatory cytokines TNF-α, IL-1β and IL-6 in BALF. In addition, HJB-1 markedly suppressed LPS-induced IκB-α degradation, nuclear accumulation of NF-κB p65 subunit and MAPK phosphorylation. These results suggested that HJB-1 improved LPS-induced ARDS by suppressing LPS-induced NF-κB and MAPK activation.

  1. The Neuraminidase Inhibitor Oseltamivir Is Effective Against A/Anhui/1/2013 (H7N9) Influenza Virus in a Mouse Model of Acute Respiratory Distress Syndrome

    PubMed Central

    Baranovich, Tatiana; Burnham, Andrew J.; Marathe, Bindumadhav M.; Armstrong, Jianling; Guan, Yi; Shu, Yuelong; Peiris, Joseph Malik Sriyal; Webby, Richard J.; Webster, Robert G.; Govorkova, Elena A.

    2014-01-01

    Background. High mortality and uncertainty about the effectiveness of neuraminidase inhibitors (NAIs) in humans infected with influenza A(H7N9) viruses are public health concerns. Methods. Susceptibility of N9 viruses to NAIs was determined in a fluorescence-based assay. The NAI oseltamivir (5, 20, or 80 mg/kg/day) was administered to BALB/c mice twice daily starting 24, 48, or 72 hours after A/Anhui/1/2013 (H7N9) virus challenge. Results. All 12 avian N9 and 3 human H7N9 influenza viruses tested were susceptible to NAIs. Without prior adaptation, A/Anhui/1/2013 (H7N9) caused lethal infection in mice that was restricted to the respiratory tract and resulted in pulmonary edema and acute lung injury with hyaline membrane formation, leading to decreased oxygenation, all characteristics of human acute respiratory distress syndrome. Oseltamivir at 20 and 80 mg/kg protected 80% and 88% of mice when initiated after 24 hours, and the efficacy decreased to 70% and 60%, respectively, when treatment was delayed by 48 hours. Emergence of oseltamivir-resistant variants was not detected. Conclusions. H7N9 viruses are comparable to currently circulating influenza A viruses in susceptibility to NAIs. Based on these animal studies, early treatment is associated with improved outcomes. PMID:24133191

  2. Boussignac CPAP system for brain death confirmation with apneic test in case of acute lung injury/adult respiratory distress syndrome – series of cases

    PubMed Central

    Wieczorek, Andrzej; Gaszynski, Tomasz

    2015-01-01

    Introduction There are some patients with severe respiratory disturbances like adult respiratory distress syndrome (ARDS) and suspicion of brain death, for whom typical performance of the apneic test is difficult to complete because of quick desaturation and rapid deterioration without effective ventilation. To avoid failure of brain death confirmation and possible loss of organ donation another approach to apneic test is needed. We present two cases of patients with clinical symptoms of brain death, with lung pathology (acute lung injury, ARDS, lung embolism and lung infection), in whom apneic tests for recognizing brain death were difficult to perform. During typical performance of apneic test involving the use of oxygen catheter for apneic oxygenation we observed severe desaturation with growing hypotension and hemodynamic destabilization. But with the use of Boussignac CPAP system all necessary tests were successfully completed, confirming the patient’s brain death, which gave us the opportunity to perform procedures for organ donation. The main reason of apneic test difficulties was severe gas exchange disturbances secondary to ARDS. Thus lack of positive end expiratory pressure during classical performance of apneic test leads to quick desaturation and rapid hemodynamic deterioration, limiting the observation period below dedicated at least 10-minute interval. Conclusion The Boussignac CPAP system may be an effective tool for performing transparent apneic test in case of serious respiratory disturbances, especially in the form of acute lung injury or ARDS. PMID:26124664

  3. Loss of extracellular superoxide dismutase leads to acute lung damage in the presence of ambient air: a potential mechanism underlying adult respiratory distress syndrome.

    PubMed

    Gongora, Maria Carolina; Lob, Heinrich E; Landmesser, Ulf; Guzik, Tomasz J; Martin, W David; Ozumi, Kiyoski; Wall, Susan M; Wilson, David Scott; Murthy, Niren; Gravanis, Michael; Fukai, Tohru; Harrison, David G

    2008-10-01

    The extracellular superoxide dismutase 3 (SOD3) is highly expressed in both blood vessels and lungs. In different models of pulmonary injury, SOD3 is reduced; however, it is unclear whether this contributes to lung injury. To study the role of acute SOD3 reduction in lung injury, the SOD3 gene was deleted in adult mice by using the Cre-Lox technology. Acute reduction of SOD3 led to a fivefold increase in lung superoxide, marked inflammatory cell infiltration, a threefold increase in the arterial-alveolar gradient, respiratory acidosis, histological changes similar to those observed in adult respiratory distress syndrome, and 85% mortality. Treatment with the SOD mimetic MnTBAP and intranasal administration of SOD-containing polyketal microparticles reduced mortality, prevented the histological alterations, and reduced lung superoxide levels. To understand how mice with the SOD3 embryonic deletion survived without lung injury, gene array analysis was performed. These data demonstrated the up-regulation of 37 genes and down-regulation of nine genes, including those involved in cell signaling, inflammation, and gene transcription in SOD3-/- mice compared with either mice with acute SOD3 reduction or wild-type controls. These studies show that SOD3 is essential for survival in the presence of ambient oxygen and that acute loss of this enzyme can lead to severe lung damage. Strategies either to prevent SOD3 inactivation or to augment its levels might prove useful in the treatment of acute lung injury.

  4. Electrical impedance tomography-guided prone positioning in a patient with acute cor pulmonale associated with severe acute respiratory distress syndrome.

    PubMed

    Kotani, Toru; Tanabe, Hitoshi; Yusa, Hiroaki; Saito, Satoshi; Yamazaki, Kenji; Ozaki, Makoto

    2016-02-01

    Electrical impedance tomography (EIT) is a noninvasive technique used to assess regional gas distribution in the lung. We experienced a patient with acute cor pulmonale during high positive-pressure ventilation for the treatment of severe acute respiratory distress syndrome. Prone positioning was beneficial for unloading the right ventricle for treatment of acute cor pulmonale. EIT played a role in detecting lung derecruitment at the patient's bedside. Impedance distribution in ventral, mid-ventral, mid-dorsal, and dorsal layers before and 20 min after the start of prone positioning was 9, 48, 44, and 0 %, and 10, 25, 48, and 16 %, respectively. Lung recruitment monitored by EIT paralleled the improvement of PaO2/FIO2 from 123 to 239 mmHg. Timing of termination of prone positioning and ventilator settings such as lowering positive end-expiration pressure was determined to maintain dorsal recruitment as seen by EIT. The patient was weaned from mechanical ventilation on day 32 and discharged on day 200. EIT assessed the effects of prone positioning with real-time dynamic imaging and guided less injurious mechanical ventilation in a patient with acute cor pulmonale with dorsal lung derecruitment.

  5. Effects of L-carnitine supplementation on respiratory distress syndrome development and prognosis in premature infants: A single blind randomized controlled trial.

    PubMed

    Ozturk, Mehmet Adnan; Kardas, Zehra; Kardas, Fatih; Gunes, Tamer; Kurtoglu, Selim

    2016-03-01

    The aim of the present study was to investigate the efficacy of L-carnitine therapy on the occurrence and prognosis of respiratory distress syndrome (RDS). A single blind, randomized controlled trial study was conducted on 130 infants with gestational ages of 28-36 weeks. Infants were assigned to experimental groups (groups 1 and 2) and control groups (groups 3 and 4). Groups 1 and 3 consisted of infants with RDS, and groups 2 and 4 groups were composed of infants without RDS. The experimental groups were treated with carnitine. No statistically significant differences in serum carnitine levels were detected between the study and the control groups on day 1 of treatment (P=0.06). However, on day 7 of treatment, serum carnitine levels in the experimental groups were significantly increased (P=0.02), as compared with the control groups. The surfactant requirement value, which is how many rounds of surfactant therapy were required, was 1.56±0.97 in group 1, and 2.12±0.99 in group 3 (P<0.001). The mean duration of mechanical ventilation required was 3.04±3.60 days in group 1, and 4.73±5.63 days in group 3 (P<0.001). The present results indicate that carnitine supplementation in premature infants with RDS may help to increase carnitine levels, thus decreasing the duration of mechanical ventilation and surfactant requirement.

  6. Effects of increased positive end-expiratory pressure on intracranial pressure in acute respiratory distress syndrome: a protocol of a prospective physiological study

    PubMed Central

    Chen, Han; Xu, Ming; Yang, Yan-Lin; Chen, Kai; Xu, Jing-Qing; Zhang, Ying-Rui; Yu, Rong-Guo

    2016-01-01

    Introduction There are concerns that the use of positive end-expiratory pressure (PEEP) in patients with brain injury may potentially elevate intracranial pressure (ICP). However, the transmission of PEEP into the thoracic cavity depends on the properties of the lungs and the chest wall. When chest wall elastance is high, PEEP can significantly increase pleural pressure. In the present study, we investigate the different effects of PEEP on the pleural pressure and ICP in different respiratory mechanics. Methods and analysis This study is a prospective, single-centre, physiological study in patients with severe brain injury. Patients with acute respiratory distress syndrome with ventricular drainage will be enrolled. An oesophageal balloon catheter will be inserted to measure oesophageal pressure. Patients will be sedated and paralysed; airway pressure and oesophageal pressure will be measured during end-inspiratory occlusion and end-expiratory occlusion. Elastance of the chest wall, the lungs and the respiratory system will be calculated at PEEP levels of 5, 10 and 15 cm H2O. We will classify each patient based on the maximal ΔICP/ΔPEEP being above or below the median for the study population. 2 groups will thus be compared. Ethics and dissemination The study protocol and consent forms were approved by the Institutional Review Board of Fujian Provincial Hospital. Study findings will be disseminated through peer-reviewed publications and conference presentations. Trial registration number NCT02670733; pre-results. PMID:27852713

  7. Sylvian aqueduct syndrome with slit ventricles in shunted hydrocephalus due to adult aqueduct stenosis.

    PubMed

    Maroulis, Helen; Halmagyi, G Michael; Heard, Robert; Cook, Raymond J

    2008-11-01

    The authors report on 3 patients who developed sylvian aqueduct syndrome (SAS) in the context of shunt dysfunction and slit ventricles. All 3 patients had received shunts for adult onset hydrocephalus due to aqueduct stenosis and were stable for years before presenting with loss of upward gaze, convergence-retraction nystagmus, and slit ventricles, all due to shunt overdrainage. All 3 improved after either shunt revision or a third ventriculostomy procedure. Although it is well known that SAS can be caused by shunt blockage producing a transtentorial pressure gradient, these cases emphasize that an identical clinical pattern can occur with a reverse transtentorial pressure gradient and slit ventricles due to shunt overdrainage. The authors propose a simple management plan for patients with shunted hydrocephalus who develop SAS.

  8. Irreversible Horner’s syndrome diagnosed by aproclonidine test due to benign thyroid nodule

    PubMed Central

    M, Coskun; A, Aydogan; C, Gokce; O, Ilhan; OV, Ozkan; H, Gokce; H, Oksuz

    2013-01-01

    We are reporting an irreversible Horner Syndrome (HS) in a patient with benign thyroid gland nodule in which thyroidectomy was performed for treatment. A 37-year-old female was admitted to our clinic with a swelling in the left lobe of the thyroid gland and ptosis at the left eyelid. The clinical diagnosis of HS was confirmed pharmacologically by aproclonidine. Histopathologic examination of thyroidectomy specimen was reported as benign nodule. To the best of our knowledge, this is a very rare report in terms of thyroid benign nodule associated with irreversible HS due to cervical sympathetic chain compression. PMID:24353546

  9. [Claude Bernard-Horner's syndrome due to malignant pleural mesothelioma (author's transl)].

    PubMed

    Baris, Y I

    1980-01-01

    Seven cases of Claude Bernard-Horner's syndrome due to malignant pleural mesothelioma are described. All the patients were middle aged farmers and lived in the rural part of Central Anatolia. Two of them lived in Karain and had been exposed to inhale erionite type zeolite fibres. Hence the other five subjects lived in asbestos deposit areas. Pleural effusion, pleural thickening and nodular pleural lesions were the radiological findings. Rib erosion was found in one case. All the patients had tissue diagnosis by pleural punch biopsy, thoracoscopy or thoracotomy.

  10. Salbutamol benefits children with congenital myasthenic syndrome due to DOK7 mutations.

    PubMed

    Burke, Georgina; Hiscock, Andrew; Klein, Andrea; Niks, Erik H; Main, Marion; Manzur, Adnan Y; Ng, Joanne; de Vile, Catherine; Muntoni, Francesco; Beeson, David; Robb, Stephanie

    2013-02-01

    Congenital myasthenic syndromes due to DOK7 mutations cause fatigable limb girdle weakness. Treatment with ephedrine improves muscle strength. Salbutamol, a β(2)-adrenergic receptor agonist with fewer side effects and more readily available, has been effective in adult and anecdotal childhood cases. This study reports the effects of salbutamol on motor function in childhood DOK7 congenital myasthenic syndrome. Nine children (age range 5.9-15.1years) were treated with oral salbutamol, 2-4mg TDS. The effect on timed tests of motor function, pre- and up to 28months post-treatment, was audited retrospectively. All 9 reported functional benefit within 1month, with progressive improvement to a plateau at 12-18months. Within the first month, all 3 non-ambulant children resumed walking with assistance. Although improvements were seen in some timed tests (timed 10m, arm raise time, 6min walk time) this did not fully reflect the observed functional benefits in daily living activities. No major side effects were reported. We conclude that oral salbutamol treatment significantly improves strength in children with DOK7 congenital myasthenic syndrome and is well tolerated. Outcome measures need to be refined further, both to accurately reflect functional abilities in children and to document progress and treatment response.

  11. Hypophosphataemic osteomalacia due to de Toni-Debre-Fanconi syndrome in a 19-year old girl.

    PubMed

    Tsilchorozidou, Tasoula; Yovos, John G

    2005-01-01

    Osteomalacia associated with adult onset Fanconi syndrome is thought to result from hypophosphataemia due to renal phosphate loss and relative 1,25-dihydroxyvitamin D3 deficiency. In this disorder, the impaired renal phosphate uptake occurs as part of a generalized tubular defect in association with other features such as bicarbonuria, glycosuria and aminoaciduria. Fanconi syndrome is either hereditary--juvenile form--or is associated with various acquired or heritable diseases. In adults, the disease is similar to the juvenile form, but osteomalacia is a prominent feature. We report a sporadic, adult onset, hypophosphataemia in a 19-year old female patient who presented after puberty complaining of bone and joint pain and difficulty in walking following a minor fall. Radiological examination revealed numerous bilateral fractures of the ribs and pelvis while biochemical investigations showed combination of high phosphate clearance, low serum bicarbonate, glycosuria and glycinuria. Known causes of acquired renal tubular dysfunction were ruled out. The patient was diagnosed as having idiopathic Fanconi syndrome and started on vitamin D3 (Alfacalcidol 1 mg/day) and oral phosphorus (Joulie Solution, 1.5 g/day), which led to resolution of symptoms and an increase in serum phosphate (from 0,54 to 0,71 mmol/l) within few months following the initiation of therapy. However, radiological re-examination showed no signs of fracture healing.

  12. Skin vasomotor hemiparesis followed by overactivity: characteristic thermography findings in a patient with Horner syndrome due to spinal cord infarction.

    PubMed

    Kobayashi, Makoto

    2016-04-01

    We present a 21-year-old female with Horner syndrome due to spinal cord infarction. In this patient, infrared thermography revealed a hemibody skin temperature increase followed by excessive focal decreases, indicating skin vasomotor hemiparesis and overactivity.

  13. [Oral allergy syndrome due to cashew nuts in the patient without pollinosis].

    PubMed

    Inomata, Naoko; Osuna, Hiroyuki; Ikezawa, Zenro

    2006-01-01

    A 26-year-old woman felt tingling on her tongue and itching both in the throat and on the face immediately after she put a cashew nut on her tongue. She had a history of atopic dermatitis and bronchial asthma, but not of pollinosis. CAP-FEIA and skin prick test (SPT) were positive for cashew nuts. The results showed negative for peanuts and other tree nuts than cashew nuts. Consequently, she was diagnosed with oral allergy syndrome due to cashew nuts. In addition, the result of skin prick test with cashew nuts normalized one year after she began avoiding cashew nuts, indicating that cashew nuts allergy would be due to sensitization by itself rather than to cross-reactivity between cashew nuts and pollens in this case.

  14. Acute abdomen due to torsion of the wandering spleen in a patient with Marfan Syndrome.

    PubMed

    Leci-Tahiri, Laura; Tahiri, Afrim; Bajrami, Rifat; Maxhuni, Mehmet

    2013-08-05

    Wandering spleen is a very rare defect characterized by the absence or weakness of one or more of the ligaments that hold the spleen in its normal position in the upper left abdomen. Patient symptomatology is variable and ranges from mere feeling of an abdominal lump to sudden abdominal pain due to infarction. Patients may have subacute to chronic abdominal or gastrointestinal complaints. Because of nonspecific symptoms, clinical diagnosis can be difficult; hence, imaging plays an important role. A major complication is splenic torsion, which is the cause of acute abdomen. We present a case of acute abdominal pain due to torsion of wandering spleen in a patient with Marfan Syndrome, valvular heart disease, and vertebral anomalies. Preoperative diagnosis was made on the basis of ultrasonography and computed tomography, which was later confirmed on surgery, and treated successfully.

  15. Locked-in Syndrome due to Central Pontine Myelinolysis: Case Report.

    PubMed

    Sohn, Min Kyun; Nam, Jin Hee

    2014-10-01

    Central pontine myelinolysis (CPM) classically occurs in alcoholics, malnourished individuals, chronic liver diseases, and rapid correction of hyponatremia. This report presents locked-in syndrome due to CPM following rapid correction of hyponatremia. A 44-year-old male came to the hospital due to a short period of loss of consciousness. He was alert and had no focal neurological abnormalities at admission. The serum sodium concentration was 118 mEq/L and was corrected to 134 mEq/L in the first 18 hours. One week later, progressive weakness in limbs developed and he progressed to a complete quadriplegic state and bulbar palsy, with only eye blinking preserved. Brain magnetic resonance imaging revealed a characteristic hyperintense signal abnormality in both pons, so he was diagnosed to locked-in syndrome caused by CPM. The patient gradually improved following continuous intensive rehabilitation for more than 2 years. He was able to move all joint muscles against gravity in generally and he could gait under supervision.

  16. Rapid community identification, pain and distress associated with lymphoedema and adenolymphangitis due to lymphatic filariasis in resource-limited communities of North-eastern Nigeria.

    PubMed

    Akogun, O B; Akogun, M K; Apake, E; Kale, O O

    2011-09-01

    Identification of communities with people that could benefit from adenolymphangitis (ADL) and lymphoedema morbidity management within Lymphatic Filariasis Elimination Programmes (NLFEP) in many African countries is a major challenge to programme managers. Another challenge is advocating for proportionate allocation of funds to alleviating the suffering that afflicted people bear. In this study we developed a rapid qualitative technique of identifying communities where morbidity management programme could be situated and documenting the pain and distress that afflicted persons endure. Estimates given by health personnel and by community resource persons were compared with systematic household surveys for the number of persons with lymphoedema of the lower limb. Communities in Northeastern Nigeria, with the largest number of lymphoedema cases were selected and a study of local knowledge, physical, psychosocial burden and intervention-seeking activities associated with the disease documented using an array of techniques (including household surveys, key informant interviews, group discussions and informal conversations). Health personnel gave a more accurate estimate of the number of lymphoedema patients in their communities than either the community leader or the community directed ivermectin distributor (CDD). Community members with lymphoedema preferred to confide in health personnel from other communities. The people had a well developed local vocabulary for lymphoedema and are well aware of the indigenous transmission theories. Although the people associated the episodic ADL attacks with the rains which were more frequent at that period they did not associate the episodes with gross lymphoedema. There were diverse theories about lymphoedema causation with heredity, accidental stepping on charmed objects and organisms, breaking taboos. The most popular belief about causation, however, is witchcraft (60.9%). The episodic attacks are dreaded by the afflicted, since

  17. Association of serum interleukin-6, interleukin-8, and Acute Physiology and Chronic Health Evaluation II score with clinical outcome in patients with acute respiratory distress syndrome

    PubMed Central

    Swaroopa, Deme; Bhaskar, Kakarla; Mahathi, T.; Katkam, Shivakrishna; Raju, Y. Satyanarayana; Chandra, Naval; Kutala, Vijay Kumar

    2016-01-01

    Background and Aim: Studies on potential biomarkers in experimental models of acute lung injury (ALI) and clinical samples from patients with ALI have provided evidence to the pathophysiology of the mechanisms of lung injury and predictor of clinical outcome. Because of the high mortality and substantial variability in outcomes in patients with acute respiratory distress syndrome (ARDS), identification of biomarkers such as cytokines is important to determine prognosis and guide clinical decision-making. Materials and Methods: In this study, we have included thirty patients admitted to Intensive Care Unit diagnosed with ARDS, and serum samples were collected on day 1 and 7 and were analyzed for serum interleukin-6 (IL-6) and IL-8 by ELISA method, and Acute Physiology and Chronic Health Evaluation II (APACHE II) scoring was done on day 1. Results: The mortality in the patients observed with ARDS was 34%. APACHE II score was significantly higher in nonsurvivors as compared to survivors. There were no significant differences in gender and biochemical and hematological parameters among the survivors and nonsurvivors. Serum IL-6 and IL-8 levels on day 1 were significantly higher in all the ARDS patients as compared to healthy controls and these levels were returned to near-normal basal levels on day 7. The serum IL-6 and IL-8 levels measured on day 7 were of survivors. As compared to survivors, the IL-6 and IL-8 levels were significantly higher in nonsurvivors measured on day 1. Spearman's rank correlation analysis indicated a significant positive correlation of APACHE II with IL-8. By using APACHE II score, IL-6, and IL-8, the receiver operating characteristic curve was plotted and the provided predictable accuracy of mortality (outcome) was 94%. Conclusion: The present study highlighted the importance of measuring the cytokines such as IL-6 and IL-8 in patients with ARDS in predicting the clinical outcome. PMID:27688627

  18. microRNA-23a-5p acts as a potential biomarker for sepsis-induced acute respiratory distress syndrome in early stage.

    PubMed

    Liu, S; Liu, C; Wang, Z; Huang, J; Zeng, Q

    2016-02-04

    Sepsis is a significant cause of morbidity and mortality worldwide. Acute respiratory distress syndrome (ARDS) is the most common and serious complication of sepsis, which presents with rapid and progressive acute onset respiratory failure. The microRNA-23a-5p, as a kind of circulating microRNA (miRNA), is considered to be a candidate biomarker for cardiovascular diseases. However, correlation between ARDS and miR-23a-5p is also elusive. This study aims to investigate the role of miR-23a-5p as the biomarkers for ARDS. In this study, ARDS was induced by intraperitoneally injected with LPS of Sprague-Dawley rats and serum and lung tissues were collected. The NR8383 macrophages were stimulated with LPS. TNF-α, IL-1β, and miR-23a-5p levels in serum, lung tissues and NR8383 were determined using SYBR-based miRNA quantitative real-time polymerase chain reactions (qRT-PCRs). The results indicated that serum miR-23a-5p was increased by 7 fold, 4 fold and 2 fold at 3 h, 6h, and 12h after injection of LPS, respectively. While the miR-23a-5p in NR8383 was elevated by 3.5 fold, 3 fold, 2.5 fold and 5 fold, at 3 h, 6h, 12h and 24h after stimulated with LPS, respectively. In conclusion, the miR-23a-5p might be employed as the potential biomarkers for ARDS in early stage.

  19. Feasibility of (68)Ga-labeled Siglec-9 peptide for the imaging of acute lung inflammation: a pilot study in a porcine model of acute respiratory distress syndrome.

    PubMed

    Retamal, Jaime; Sörensen, Jens; Lubberink, Mark; Suarez-Sipmann, Fernando; Borges, João Batista; Feinstein, Ricardo; Jalkanen, Sirpa; Antoni, Gunnar; Hedenstierna, Göran; Roivainen, Anne; Larsson, Anders; Velikyan, Irina

    2016-01-01

    There is an unmet need for noninvasive, specific and quantitative imaging of inherent inflammatory activity. Vascular adhesion protein-1 (VAP-1) translocates to the luminal surface of endothelial cells upon inflammatory challenge. We hypothesized that in a porcine model of acute respiratory distress syndrome (ARDS), positron emission tomography (PET) with sialic acid-binding immunoglobulin-like lectin 9 (Siglec-9) based imaging agent targeting VAP-1 would allow quantification of regional pulmonary inflammation. ARDS was induced by lung lavages and injurious mechanical ventilation. Hemodynamics, respiratory system compliance (Crs) and blood gases were monitored. Dynamic examination using [(15)O]water PET-CT (10 min) was followed by dynamic (90 min) and whole-body examination using VAP-1 targeting (68)Ga-labeled 1,4,7,10-tetraaza cyclododecane-1,4,7-tris-acetic acid-10-ethylene glycol-conjugated Siglec-9 motif peptide ([(68)Ga]Ga-DOTA-Siglec-9). The animals received an anti-VAP-1 antibody for post-mortem immunohistochemistry assay of VAP-1 receptors. Tissue samples were collected post-mortem for the radioactivity uptake, histology and immunohistochemistry assessment. Marked reduction of oxygenation and Crs, and higher degree of inflammation were observed in ARDS animals. [(68)Ga]Ga-DOTA-Siglec-9 PET showed significant uptake in lungs, kidneys and urinary bladder. Normalization of the net uptake rate (Ki) for the tissue perfusion resulted in 4-fold higher uptake rate of [(68)Ga]Ga-DOTA-Siglec-9 in the ARDS lungs. Immunohistochemistry showed positive VAP-1 signal in the injured lungs. Detection of pulmonary inflammation associated with a porcine model of ARDS was possible with [(68)Ga]Ga-DOTA-Siglec-9 PET when using kinetic modeling and normalization for tissue perfusion.

  20. Effect of intravenous β-2 agonist treatment on clinical outcomes in acute respiratory distress syndrome (BALTI-2): a multicentre, randomised controlled trial

    PubMed Central

    Smith, Fang Gao; Perkins, Gavin D; Gates, Simon; Young, Duncan; McAuley, Daniel F; Tunnicliffe, William; Khan, Zahid; Lamb, Sarah E

    2012-01-01

    Summary Background In a previous randomised controlled phase 2 trial, intravenous infusion of salbutamol for up to 7 days in patients with acute respiratory distress syndrome (ARDS) reduced extravascular lung water and plateau airway pressure. We assessed the effects of this intervention on mortality in patients with ARDS. Methods We did a multicentre, placebo-controlled, parallel-group, randomised trial at 46 UK intensive-care units between December, 2006, and March, 2010. Intubated and mechanically ventilated patients (aged ≥16 years) within 72 h of ARDS onset were randomly assigned to receive either salbutamol (15 μg/kg ideal bodyweight per h) or placebo for up to 7 days. Randomisation was done by a central telephone or web-based randomisation service with minmisation by centre, pressure of arterial oxygen to fractional inspired oxygen concentration (PaO2/FIO2) ratio, and age. All participants, caregivers, and investigators were masked to group allocation. The primary outcome was death within 28 days of randomisation. Analysis was by intention-to-treat. This trial is registered, ISRCTN38366450 and EudraCT number 2006-002647-86. Findings We randomly assigned 162 patients to the salbutamol group and 164 to the placebo group. One patient in each group withdrew consent. Recruitment was stopped after the second interim analysis because of safety concerns. Salbutamol increased 28-day mortality (55 [34%] of 161 patients died in the salbutamol group vs 38 (23%) of 163 in the placebo group; risk ratio [RR] 1·47, 95% CI 1·03–2·08). Interpretation Treatment with intravenous salbutamol early in the course of ARDS was poorly tolerated. Treatment is unlikely to be beneficial, and could worsen outcomes. Routine use of β-2 agonist treatment in ventilated patients with this disorder cannot be recommended. Funding UK Medical Research Council, UK Department of Health, UK Intensive Care Foundation. PMID:22166903

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

    PubMed Central

    Hoffman, Olivia; Burns, Nana; Vadász, István; Eltzschig, Holger K.; Edwards, Michael G.

    2017-01-01

    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. PMID:28196122

  2. NOX1 is responsible for cell death through STAT3 activation in hyperoxia and is associated with the pathogenesis of Acute Respiratory Distress Syndrome

    PubMed Central

    Carnesecchi, Stephanie; Dunand-Sauthier, Isabelle; Zanetti, Filippo; Singovski, Grigory; Deffert, Christine; Donati, Yves; Cagarelli, Thomas; Pache, Jean-Claude; Krause, Karl-Heinz; Reith, Walter; Barazzone-Argiroffo, Constance

    2014-01-01

    Reactive oxygen species (ROS) contribute to alveolar cell death in Acute Respiratory Distress Syndrome (ARDS) and we previously demonstrated that NOX1-derived ROS contributed to hyperoxia-induced alveolar cell death in mice. The study investigates whether NOX1 expression is modulated in epithelial cells concomitantly to cell death and associated to STAT3 signaling in the exudative phase of ARDS. In addition, the role of STAT3 activation in NOX1-dependent epithelial cell death was confirmed by using a lung epithelial cell line and in mice exposed to hyperoxia. NOX1 expression, cell death and STAT3 staining were evaluated in the lungs of control and ARDS patients by immunohistochemistry. In parallel, a stable NOX1-silenced murine epithelial cell line (MLE12) and NOX1-deficient mice were used to characterize signalling pathways. In the present study, we show that NOX1 is detected in alveolar epithelial cells of ARDS patients in the exudative stage. In addition, increased alveolar epithelial cell death and phosphorylated STAT3 are observed in ARDS patients and associated with NOX1 expression. Phosphorylated STAT3 is also correlated with TUNEL staining. We also confirmed that NOX1-dependent STAT3 activation participates to alveolar epithelial cell death. Silencing and acute inhibition of NOX1 in MLE12 led to decreased cell death and cleaved-caspase 3 induced by hyperoxia. Additionally, hyperoxia-induced STAT3 phosphorylation is dependent on NOX1 expression and associated with cell death in MLE12 and mice. This study demonstrates that NOX1 is involved in human ARDS pathophysiology and is responsible for the damage occurring in alveolar epithelial cells at least in part via STAT3 signalling pathways. PMID:24551274

  3. Genetic Polymorphisms of SP-A, SP-B, and SP-D and Risk of Respiratory Distress Syndrome in Preterm Neonates

    PubMed Central

    Chang, Hong-Yu; Li, Fang; Li, Feng-Sheng; Zheng, Cheng-Zhong; Lei, Yan-Zhe; Wang, Jing

    2016-01-01

    Background We examined selected polymorphisms in 3 pulmonary surfactant-associated proteins (SP) for their influence on serum SP levels and risk of respiratory distress syndrome (RDS) in preterm neonates. Material/Methods Premature infants from a Han population were enrolled, including 100 premature infants with RDS (case group) and 120 premature infants without RDS (control group). SNP genotyping for SP-A (+186A/G and +655C/T), SP-B (−18A/C and 1580C/T), and SP-D (Met11ThrT/C and Ala160ThrG/A) used polymerase chain reaction-restriction fragment length polymorphism. Haplotypes were calculated with Shesis software and serum SP-A/B/D levels were quantified by ELISA. Results Case and control groups exhibited significant differences in genotype and allele frequencies of SP-A (+186A/G, +655C/T) and SP-B (1580C/T). However, no statistically significant differences were observed in the allele and genotype frequencies of SP-B −18A/C, SP-D Met11ThrT/C, and SP-D Ala160ThrG/A. Importantly, serum SP-A and SP-B levels were reduced in RDS patients carrying SP-A (+186A/G, +655C/T) and SP-B (1580C/T) polymorphisms. AA genotype of +186A/G, SP-A level, and CC genotype of 1580C/T were independently correlated with increased RDS risk. Conclusions SP-A (+186A/G) and SP-B (1580C/T) polymorphisms are strongly associated with the risk of RDS in preterm infants. Notably, reduced serum SP-A levels were correlated with a high risk of RDS and may serve as novel biomarkers for RDS detection and monitoring. PMID:28011976

  4. Meta-analysis of high doses of ambroxol treatment for acute lung injury/acute respiratory distress syndrome based on randomized controlled trials.

    PubMed

    Wu, Xiangdong; Li, Suwei; Zhang, Jiuzhi; Zhang, Yongli; Han, Lili; Deng, Qiuming; Wan, Xianyao

    2014-11-01

    This study seeks to evaluate the potential benefits of high doses of ambroxol treatment for acute lung injury (ALI)/acute respiratory distress syndrome (ARDS) by conducting a meta-analysis based on randomized controlled trials (RCTs). We searched the Pubmed, Embase, China National Knowledge Infrastructure, and Wanfang databases through December 2013. Only RCTs evaluating high doses of ambroxol (≥15 mg/kg or 1000 mg/day) treatment for patients with ALI/ARDS were selected. We included 10 RCTs involving 508 patients. Adjuvant treatment with high doses of ambroxol increased PaO(2)/FiO(2) (weight mean differences [WMD] = 69.18, 95% confidence intervals [CI]: 41.71-96.65), PO(2) (WMD = 11.74, 95% CI: 8.50-14.99), and SaO(2) (WMD = 2.15, 95% CI: 1.60-2.71) compared with usual treatment. Treatment with high doses of ambroxol appeared to reduce serum tumor necrosis factor-α level (WMD -7.92 µg/L; 95% CI, -10.94 to -4.9) and interleukin-6 level (WMD = -20.65 µg/L, 95% CI: -24.74 to -16.55) and to increase serum superoxide dismutase level (WMD = 19.07 NU/mL, 95% CI: 6.16-31.97). The findings suggest that treatment with high doses of ambroxol appears to improve PaO(2)/FiO(2), PO(2), and SaO(2), and the benefits might be related to ambroxol's anti-oxidant and anti-inflammatory properties.

  5. Early Hepatic Dysfunction Is Associated with a Worse Outcome in Patients Presenting with Acute Respiratory Distress Syndrome: A Post-Hoc Analysis of the ACURASYS and PROSEVA Studies

    PubMed Central

    Dizier, Stéphanie; Forel, Jean-Marie; Ayzac, Louis; Richard, Jean-Christophe; Hraiech, Sami; Lehingue, Samuel; Loundou, Anderson; Roch, Antoine; Guerin, Claude; Papazian, Laurent

    2015-01-01

    Introduction Bilirubin is well-recognized marker of hepatic dysfunction in intensive care unit (ICU) patients. Multiple organ failure often complicates acute respiratory distress syndrome (ARDS) evolution and is associated with high mortality. The effect of early hepatic dysfunction on ARDS mortality has been poorly investigated. We evaluated the incidence and the prognostic significance of increased serum bilirubin levels in the initial phase of ARDS. Methods The data of 805 patients with ARDS were retrospectively analysed. This population was extracted from two recent multicenter, prospective and randomised trials. Patients presenting with ARDS with a ratio of the partial pressure of arterial oxygen to the fraction of inspired oxygen < 150 mmHg measured with a PEEP ≥ 5 cm of water were included. The total serum bilirubin was measured at inclusion and at days 2, 4, 7 and 14. The primary objective was to analyse the bilirubin at inclusion according to the 90-day mortality rate. Results The 90-day mortality rate was 33.8% (n = 272). The non-survivors were older, had higher Sepsis-related Organ Failure Assessment (SOFA) score and were more likely to have a medical diagnosis on admission than the survivors. At inclusion, the SOFA score without the liver score (10.3±2.9 vs. 9.0±3.0, p<0.0001) and the serum bilirubin levels (36.1±57.0 vs. 20.5±31.5 μmol/L, p<0.0001) were significantly higher in the non-survivors than in the survivors. Age, the hepatic SOFA score, the coagulation SOFA score, the arterial pH level, and the plateau pressure were independently associated with 90-day mortality in patients with ARDS. Conclusion Bilirubin used as a surrogate marker of hepatic dysfunction and measured early in the course of ARDS was associated with the 90-day mortality rate. PMID:26636318

  6. Can non-invasive positive pressure ventilation prevent endotracheal intubation in acute lung injury/acute respiratory distress syndrome? A meta-analysis.

    PubMed

    Luo, Jian; Wang, Mao-yun; Zhu, Hui; Liang, Bin-miao; Liu, Dan; Peng, Xia-ying; Wang, Rong-chun; Li, Chun-tao; He, Chen-yun; Liang, Zong-an

    2014-11-01

    The role of non-invasive positive pressure ventilation (NIPPV) in acute lung injury (ALI)/acute respiratory distress syndrome (ARDS) is controversial. The aim of this study was to investigate whether NIPPV could prevent endotracheal intubation and decrease mortality rate in patients with ALI/ARDS. Randomized controlled trials (RCT) which reported endotracheal intubation and mortality rate in patients with ALI/ARDS treated by NIPPV were identified in Pubmed, Medline, Embase, Central Cochrane Controlled Trials Register, Chinese National Knowledge Infrastructure, reference lists and by manual searches. Fixed- and random-effects models were used to calculate pooled relative risks. This meta-analysis included six RCT involving 227 patients. The results showed that endotracheal intubation rate was lower in NIPPV (95% confidence interval (CI): 0.44-0.80, z = 3.44, P = 0.0006), but no significant difference was found either in intensive care unit (ICU) mortality (95% CI: 0.45-1.07, z = 1.65, P = 0.10) or in hospital mortality (95% CI: 0.17-1.58, z = 1.16, P = 0.25). Only two studies discussed the aetiology of ALI/ARDS as pulmonary or extra-pulmonary, and neither showed statistical heterogeneity (I(2)  = 0%, χ(2)  = 0.31, P = 0.58), nor a significant difference in endotracheal intubation rate (95% CI: 0.35-9.08, z = 0.69, P = 0.49). In conclusion, the early use of NIPPV can decrease the endotracheal intubation rate in patients with ALI/ARDS, but does not change the mortality of these patients.

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

    PubMed

    Hoffman, Olivia; Burns, Nana; Vadász, István; Eltzschig, Holger K; Edwards, Michael G; Vohwinkel, Christine U

    2017-01-01

    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.

  8. Effect of nebulized budesonide on respiratory mechanics and oxygenation in acute lung injury/acute respiratory distress syndrome: Randomized controlled study

    PubMed Central

    Mohamed, Hatem Saber; Meguid, Mona Mohamed Abdel

    2017-01-01

    Background: We tested the hypothesis that nebulized budesonide would improve lung mechanics and oxygenation in patients with early acute lung injury (ALI) and/or acute respiratory distress syndrome (ARDS) during protective mechanical ventilation strategy without adversely affecting systemic hemodynamics. Methods: Patients with ALI/ARDS were included and assigned into two groups; budesonide group (30 cases) in whom 1 mg–2 ml budesonide suspension was nebulized through the endotracheal tube and control group (30 cases) in whom 2 ml saline (placebo) were nebulized instead of budesonide. This regimen was repeated every 12 h for three successive days alongside with constant ventilator settings in both groups. Hemodynamics, airway pressures, and PaO2/FiO2 were measured throughout the study period (72 h) with either nebulized budesonide or saline. Furthermore, tumor necrosis factor-alpha (TNF-α), interleukin-1 beta (IL-1β), and interleukin-6 (IL-6) were analyzed serologically as markers of inflammation at pre- and post-nebulization sessions. Results: We found a significant difference between the two groups regarding PaO2/FiO2 (P = 0.023), peak (P = 0.021), and plateau (P = 0.032) airway pressures. Furthermore, TNF-α, IL-1β, and IL-6 were significantly reduced after budesonide nebulizations. No significant difference was found between the two groups regarding hemodynamic variables. Conclusion: Nebulized budesonide improved oxygenation, peak, and plateau airway pressures and significantly reduced inflammatory markers (TNF-α, IL-1β and IL-6) without affecting hemodynamics. Trial Registry: Australian New Zealand Clinical Trial Registry (ANZCTR) at the number: ACTRN12615000373572. PMID:28217046

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

    PubMed Central

    2016-01-01

    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. PMID:28044135

  10. Feasibility of 68Ga-labeled Siglec-9 peptide for the imaging of acute lung inflammation: a pilot study in a porcine model of acute respiratory distress syndrome

    PubMed Central

    Retamal, Jaime; Sörensen, Jens; Lubberink, Mark; Suarez-Sipmann, Fernando; Borges, João Batista; Feinstein, Ricardo; Jalkanen, Sirpa; Antoni, Gunnar; Hedenstierna, Göran; Roivainen, Anne; Larsson, Anders; Velikyan, Irina

    2016-01-01

    There is an unmet need for noninvasive, specific and quantitative imaging of inherent inflammatory activity. Vascular adhesion protein-1 (VAP-1) translocates to the luminal surface of endothelial cells upon inflammatory challenge. We hypothesized that in a porcine model of acute respiratory distress syndrome (ARDS), positron emission tomography (PET) with sialic acid-binding immunoglobulin-like lectin 9 (Siglec-9) based imaging agent targeting VAP-1 would allow quantification of regional pulmonary inflammation. ARDS was induced by lung lavages and injurious mechanical ventilation. Hemodynamics, respiratory system compliance (Crs) and blood gases were monitored. Dynamic examination using [15O]water PET-CT (10 min) was followed by dynamic (90 min) and whole-body examination using VAP-1 targeting 68Ga-labeled 1,4,7,10-tetraaza cyclododecane-1,4,7-tris-acetic acid-10-ethylene glycol-conjugated Siglec-9 motif peptide ([68Ga]Ga-DOTA-Siglec-9). The animals received an anti-VAP-1 antibody for post-mortem immunohistochemistry assay of VAP-1 receptors. Tissue samples were collected post-mortem for the radioactivity uptake, histology and immunohistochemistry assessment. Marked reduction of oxygenation and Crs, and higher degree of inflammation were observed in ARDS animals. [68Ga]Ga-DOTA-Siglec-9 PET showed significant uptake in lungs, kidneys and urinary bladder. Normalization of the net uptake rate (Ki) for the tissue perfusion resulted in 4-fold higher uptake rate of [68Ga]Ga-DOTA-Siglec-9 in the ARDS lungs. Immunohistochemistry showed positive VAP-1 signal in the injured lungs. Detection of pulmonary inflammation associated with a porcine model of ARDS was possible with [68Ga]Ga-DOTA-Siglec-9 PET when using kinetic modeling and normalization for tissue perfusion. PMID:27069763

  11. Respiratory distress syndrome in moderately late and late preterm infants and risk of cerebral palsy: a population-based cohort study

    PubMed Central

    Thygesen, Sandra Kruchov; Olsen, Morten; Østergaard, John R; Sørensen, Henrik Toft

    2016-01-01

    Objectives Infant respiratory distress syndrome (IRDS) is a known risk factor for intracerebral haemorrhage/intraventricular haemorrhage (ICH/IVH) and periventricular leucomalacia. These lesions are known to increase the risk of cerebral palsy (CP). Thus, we wanted to examine the long-term risk of CP following IRDS in moderately late and late preterm infants. Design Population-based cohort study. Setting All hospitals in Denmark. Participants We used nationwide medical registries to identify a cohort of all moderately and late preterm infants (defined as birth during 32–36 full gestational weeks) born in Denmark in 1997–2007 with and without hospital diagnosed IRDS. Main outcomes measures We followed study participants from birth until first diagnosis of CP, emigration, death or end of follow-up in 2014. We computed the cumulative incidence of CP before age 8 years and used Cox's regression analysis to compute HRs of IRDS, comparing children with IRDS to those without IRDS. HRs were adjusted for multiple covariates. Results We identified 39 420 moderately late and late preterm infants, of whom 2255 (5.7%) had IRDS. The cumulative incidence of CP was 1.9% in infants with IRDS and 0.5% in the comparison cohort. The adjusted HR of CP was 2.0 (95% CI 1.4 to 2.9). The adjusted HR of CP was 12 (95% CI 4.5 to 34) in children with IRDS accompanied by a diagnosis of ICH/IVH. After restriction to children without diagnoses of perinatal breathing disorders other than IRDS, congenital heart disease and viral or bacterial infections occurring within 4 days of birth, the overall adjusted HR was 2.1 (95% CI 1.4 to 3.1). Conclusions The risk of CP was increased in moderately late and late preterm infants with IRDS compared with infants without IRDS born during the same gestational weeks. PMID:27729347

  12. [Respiratory distress].

    PubMed

    Galili, D; Garfunkel, A; Elad, S; Zusman, S P; Malamed, S F; Findler, M; Kaufman, E

    2002-01-01

    Dental treatment is usually conducted in the oral cavity and in very close proximity to the upper respiratory airway. The possibility of unintentionally compromising this airway is high in the dental environment. The accumulation of fluid (water or blood) near to the upper respiratory airway or the loosening of teeth fragmentations and fallen dental instruments can occur. Also, some of the drugs prescribed in the dental practice are central nervous system depressants and some are direct respiratory drive depressors. For this reason, awareness of the respiratory status of the dental patient is of paramount importance. This article focuses on several of the more common causes of respiratory distress, including airway obstruction, hyperventilation, asthma, bronchospasm, pulmonary edema, pulmonary embolism and cardiac insufficiency. The common denominator to all these conditions described here is that in most instances the patient is conscious. Therefore, on the one hand, valuable information can be retrieved from the patient making diagnosis easier than when the patient is unconscious. On the other hand, the conscious patient is under extreme apprehension and stress under such situations. Respiratory depression which occurs during conscious sedation or following narcotic analgesic medication will not be dealt with in this article. Advanced pain and anxiety control techniques such as conscious sedation and general anesthesia should be confined only to operators who undergo special extended training.

  13. Angelman syndrome due to paternal uniparental disomy of chromosome 15: A milder phenotype?

    SciTech Connect

    Bottani, A.; Robinson, W.P.; DeLoizer-Blanchet, C.D.; Engel, E.; Morris, M.A.; Schmitt, Thun-Hohenstein, L.; Schinzel, A.

    1994-05-15

    The Angelman syndrome (AS) is a neurological disorder characterized by severe mental retardation, absent speech, seizures, gait disturbances, and a typical age-dependent facial phenotype. Most cases are due to an interstitial deletion on the maternally inherited chromosome 15, in the critical region q11-q13. Rare cases also result from paternal uniparental disomy of chromosome 15. In a group of 14 patients with sporadic AS diagnosed in Switzerland, we found 2 unrelated females with paternal isodisomy for the entire chromosome 15. Their phenotypes were milder than usually seen in this syndrome: one girl did not show the typical AS facial changes; both patients had late-onset mild seizures; as they grow older, they had largely undisturbed gross motor functions, in particular no severe ataxia. Both girls were born to older fathers (45 and 43 years old, respectively). The apparent association of a relatively milder phenotype in AS with paternal uniparental disomy will have to be confirmed by detailed clinical descriptions of further patients. 25 refs., 2 figs., 1 tab.

  14. Time-dependency of improvements in arterial oxygenation during partial liquid ventilation in experimental acute respiratory distress syndrome

    PubMed Central

    Max, Martin; Kuhlen, Ralf; Dembinski, Rolf; Rossaint, Rolf

    2000-01-01

    Background: The mechanisms by which partial liquid ventilation (PLV) can improve gas exchange in acute lung injury are still unclear. Therefore, we examined the time- and dose-dependency of the improvements in arterial oxygen tension (PaO2) due to PLV in eight pigs with experimental lung injury, in order to discriminate increases due to oxygen dissolved in perfluorocarbon before its intrapulmonary instillation from a persistent diffusion of the respiratory gas through the liquid column. Results: Application of four sequential doses of perfluorocarbon resulted in a dose-dependent increase in PaO2. Comparison of measurements 5 and 30 min after instillation of each dose revealed a time-dependent decrease in PaO2 for doses that approximated the functional residual capacity of the animals. Conclusion: Although oxygen dissolved in perfluorocarbon at the onset of PLV can cause a short-term improvement in arterial oxygenation, diffusion of oxygen through the liquid may not be sufficient to maintain the initially observed increase in PaO2. PMID:11056747

  15. Mild Angelman syndrome phenotype due to a mosaic methylation imprinting defect.

    PubMed

    Fairbrother, Laura C; Cytrynbaum, Cheryl; Boutis, Paula; Buiting, Karin; Weksberg, Rosanna; Williams, Charles

    2015-07-01

    Angelman syndrome (AS) is a neurogenetic disorder causing severe to profound intellectual disability, absent or very limited speech and a high risk for seizures. AS is caused by a loss of function of the maternally-derived UBE3A allele due to one of several mechanisms including imprinting defects (ImpDs). We present a girl with AS due to a mosaic ImpD who has relatively high developmental function (VABS-II composite score of 76) and communication skills (as demonstrated in supplemental video links). Given the patient's relatively mild developmental impairment, without clinical evidence of seizures, gait disturbance or inappropriate laughter, the diagnosis of AS was not initially suspected. Initial laboratory testing for AS was inconclusive but additional studies suggested mosaic ImpD and characteristic EEG findings provided further support for the clinical diagnosis. Our patient, along with other case reports of children with AS and relatively mild phenotypes, raises the question as to whether there exists an undiagnosed group of individuals with mild intellectual disability and expressive speech delays due to mosaic methylation defects of the chromosome 15q11.2-13 region. Population studies may be needed to determine if such an undiagnosed group exists.

  16. First rib fracture and Horner’s syndrome due to a motor vehicle collision: a case report

    PubMed Central

    2013-01-01

    A case of a first rib fracture and Horner's syndrome due to a motor vehicle collision is reported. Initial evaluation in a hospital emergency department and follow-up by a medical primary care physician failed to provide identification of the Horner’s syndrome. Careful assessment and review of the patient’s symptoms, signs and images revealed this uncommon and important neurologic case presentation. A brief discussion related to traumatic first rib fracture, Horner’s syndrome and arterial dissections of the neck is provided. PMID:23830309

  17. Improved Oxygenation 24 Hours After Transition to Airway Pressure Release Ventilation or High-Frequency Oscillatory Ventilation Accurately Discriminates Survival in Immunocompromised Pediatric Patients With Acute Respiratory Distress Syndrome*

    PubMed Central

    Yehya, Nadir; Topjian, Alexis A.; Thomas, Neal J.; Friess, Stuart H.

    2014-01-01

    Objectives Children with an immunocompromised condition and requiring invasive mechanical ventilation have high risk of death. Such patients are commonly transitioned to rescue modes of non-conventional ventilation, including airway pressure release ventilation and high-frequency oscillatory ventilation, for acute respiratory distress syndrome refractory to conventional ventilation. Our aim was to describe our experience with airway pressure release ventilation and high-frequency oscillatory ventilation in children with an immunocompromised condition and acute respiratory distress syndrome refractory to conventional ventilation and to identify factors associated with survival. Design Retrospective cohort study. Setting Tertiary care, university-affiliated PICU. Patients Sixty pediatric patients with an immunocompromised condition and acute respiratory distress syndrome refractory to conventional ventilation transitioned to either airway pressure release ventilation or high-frequency oscillatory ventilation. Interventions None. Measurements and Main Results Demographic data, ventilator settings, arterial blood gases, oxygenation index, and Pao2/Fio2 were recorded before transition to either mode of nonconventional ventilation and at predetermined intervals after transition for up to 5 days. Mortality in the entire cohort was 63% and did not differ between patients transitioned to airway pressure release ventilation and high-frequency oscillatory ventilation. For both airway pressure release ventilation and high-frequency oscillatory ventilation, improvements in oxygenation index and Pao2/Fio2 at 24 hours expressed as a fraction of pretransition values (oxygenation index24/oxygenation indexpre and Pao2/Fio224/Pao2/FIO2pre) reliably discriminated nonsurvivors from survivors, with receiver operating characteristic areas under the curves between 0.89 and 0.95 (p for all curves < 0.001). Sensitivity-specificity analysis suggested that less than 15% reduction in

  18. Hypopituitarism in a patient with Beckwith-Wiedemann syndrome due to hypomethylation of KvDMR1.

    PubMed

    Baiocchi, Michela; Yousuf, Fatimah Sireen; Hussain, Khalid

    2014-04-01

    Beckwith-Wiedemann syndrome (BWS) is caused by dysregulation of imprinted genes on chromosome 11.p15.5. The syndrome includes overgrowth, macroglossia, organomegaly, abdominal wall defects, hypoglycemia, and long-term malignancy risk. No patient who has BWS has been reported with hypopituitarism. We describe a patient who presented at birth with macrosomia, macroglossia, respiratory distress, jaundice, and hypoglycemia, and who was followed for 4.5 years. Genetic test for BWS was performed, which detected loss of maternal methylation on region KvDMR1 (11p15.5). The hypoglycemia was attributable to hyperinsulinism and was treated with diazoxide and chlorothiazide. She responded well, but the hypoglycemia returned after reducing the diazoxide. It was possible to stop the diazoxide after 2.5 years. On routine follow-up she was noted to be developing short stature. Baseline pituitary and growth hormone (GH) stimulation tests detected GH deficiency and secondary hypothyroidism. A brain MRI showed a small anterior pituitary gland. Thereafter, thyroxine and replacement therapy with GH were started, which resulted in a remarkable improvement in growth velocity. This is the first patient to be reported as having hypopituitarism and BWS. It is unclear if the BWS and the hypopituitarism are somehow connected; however, further investigations are necessary. Hypopituitarism explains the protracted hypoglycemia and the short stature. In our patient, GH therapy seems to be safe, but strict follow-up is required given the increased cancer risk related to BWS.

  19. Greater trochanteric pain syndrome due to tumoral calcinosis in a patient with chronic kidney disease.

    PubMed

    Baek, Dongjin; Lee, Sang Eun; Kim, Woo-Jin; Jeon, Sanghoon; Lee, Kihwa; Jung, Jaewook; Joo, Hyunchul; Park, Jaehong; Kim, Yonghan; Choi, Young-gyun

    2014-01-01

    Tumoral calcinosis is a rare syndrome characterized by massive subcutaneous soft tissue deposits of calcium phosphate near the large joints. It is more prevalent in patients with chronic kidney disease undergoing dialysis. A 57-year-old woman was referred to our pain clinic with the complaint of severe pain in the left buttock and lateral hip. The patient had been suffering from chronic kidney disease for 10 years and had been undergoing peritoneal dialysis over the past 5 years. The patient's symptom was initially suspected to be of lumbar origin at the L5 level and a left L5 transforaminal epidural block was performed, but without success. Re-evaluation of the physical examination revealed severe tenderness over the left greater trochanter and piriformis muscle. On ultrasonographic evaluation, multiple mass-like lesions in the left buttock were observed. About 30 mL of fluid was aspirated from the cystic lesions, followed by 30 mL mixture of 0.08% levobupivacaine and triamcinolone 40 mg injected into the bursa under ultrasound guidance, which brought pain relief. Trochanteric bursitis was thought of as the cause of the symptoms. The patient was diagnosed with tumoral calcinosis based on the past medical history, simple plain radiographs, and hip magnetic resonance imaging (MRI). We diagnosed a case of greater trochanteric pain syndrome due to tumoral calcinosis related to chronic kidney disease in a patient whose symptoms had initially been considered to be radiating leg pain caused by lumbar spinal disease. We report our experience of symptomatic improvement following the repeated ultrasound-guided aspiration of calcific fluid and the injection of a mixture of local anesthetic and steroid.

  20. Inhaled nitric oxide in acute respiratory distress syndrome with and without septic shock requiring norepinephrine administration: a dose–response study

    PubMed Central

    Mourgeon, Eric; Puybasset, Louis; Law-Koune, Jean-Dominique; Lu, Qin; Abdennour, Lamine; Gallart, Lluis; Malassine, Patrick; Rao, GS Umamaheswara; Cluzel, Philippe; Bennani, Abdelhai; Coriat, Pierre; Rouby, Jean-Jacques

    1997-01-01

    Background: The aim of this prospective study was to assess whether the presence of septic shock could influence the dose response to inhaled nitric oxide (NO) in NO-responding patients with adult respiratory distress syndrome (ARDS). Results: Eight patients with ARDS and without septic shock (PaO2 = 95 ± 16 mmHg, PEEP = 0, FiO2 = 1.0), and eight patients with ARDS and septic shock (PaO2 = 88 ± 11 mmHg, PEEP = 0, FiO2 = 1.0) receiving exclusively norepinephrine were studied. All responded to 15 ppm inhaled NO with an increase in PaO2 of at least 40 mmHg, at FiO2 1.0 and PEEP 10 cmH2O. Inspiratory intratracheal NO concentrations were recorded continuously using a fast response time chemiluminescence apparatus. Seven inspiratory NO concentrations were randomly administered: 0.15, 0.45, 1.5, 4.5, 15, 45 and 150 ppm. In both groups, NO induced a dose-dependent decrease in mean pulmonary artery pressure (MPAP), pulmonary vascular resistance index (PVRI), and venous admixture (QVA/QT), and a dose-dependent increase in PaO2/FiO2 (P ≤ 0.012). Dose-response of MPAP and PVRI were similar in both groups with a plateau effect at 4.5 ppm. Dose-response of PaO2/FiO2 was influenced by the presence of septic shock. No plateau effect was observed in patients with septic shock and PaO2/FiO2 increased by 173 ± 37% at 150 ppm. In patients without septic shock, an 82 ± 26% increase in PaO2/FiO2 was observed with a plateau effect obtained at 15 ppm. In both groups, dose-response curves demonstrated a marked interindividual variability and in five patients pulmonary vascular effect and improvement in arterial oxygenation were dissociated. Conclusion: For similar NOinduced decreases in MPAP and PVRI in both groups, the increase in arterial oxygenation was more marked in patients with septic shock. PMID:11056694

  1. Efficacy and safety of surfactant replacement therapy for preterm neonates with respiratory distress syndrome in low- and middle-income countries: a systematic review

    PubMed Central

    Sankar, M J; Gupta, N; Jain, K; Agarwal, R; Paul, V K

    2016-01-01

    Surfactant replacement therapy (SRT) has been shown to reduce mortality and air leaks in preterm neonates from high-income countries (HICs). The safety and efficacy of SRT in low- and middle- income countries (LMICs) have not been systematically evaluated. The major objectives of this review were to assess the (1) efficacy and safety, and (2) feasibility and cost effectiveness of SRT in LMIC settings. We searched the following databases—MEDLINE, CENTRAL, CINAHL, EMBASE and WHOLIS using the search terms 'surfactant' OR 'pulmonary surfactant'. Both experimental and observational studies that enrolled preterm neonates with or at-risk of respiratory distress syndrome (RDS) and required surfactant (animal-derived or synthetic) were included. A total of 38 relevant studies were found; almost all were from level-3 neonatal units. Pooled analysis of two randomized controlled trials (RCTs) and 22 observational studies showed a significant reduction in mortality at the last available time point in neonates who received SRT (relative risk (RR) 0.67; 95% confidence interval (CI) 0.57 to 0.79). There was also a significant reduction in the risk of air leaks (five studies; RR 0.51; 0.29 to 0.90). One RCT and twelve observational studies reported the risk of bronchopulmonary dysplasia (BPD) with contrasting results; while the RCT and most before-after/cohort studies showed a significant reduction or no effect, the majority of the case-control studies demonstrated significantly higher odds of receiving SRT in neonates who developed BPD. Two studies—one RCT and one observational—found no difference in the proportion of neonates developing pulmonary hemorrhage, while another observational study reported a higher incidence in those receiving SRT. The failure rate of the intubate-surfactant-extubate (InSurE) technique requiring mechanical ventilation or referral varied from 34 to 45% in four case-series. No study reported on the cost effectiveness of SRT. Available evidence

  2. Assessment of PaO2/FiO2 for stratification of patients with moderate and severe acute respiratory distress syndrome

    PubMed Central

    Villar, Jesús; Blanco, Jesús; del Campo, Rafael; Andaluz-Ojeda, David; Díaz-Domínguez, Francisco J; Muriel, Arturo; Córcoles, Virgilio; Suárez-Sipmann, Fernando; Tarancón, Concepción; González-Higueras, Elena; López, Julia; Blanch, Lluis; Pérez-Méndez, Lina; Fernández, Rosa Lidia; Kacmarek, Robert M

    2015-01-01

    Objectives A recent update of the definition of acute respiratory distress syndrome (ARDS) proposed an empirical classification based on ratio of arterial partial pressure of oxygen to fraction of inspired oxygen (PaO2/FiO2) at ARDS onset. Since the proposal did not mandate PaO2/FiO2 calculation under standardised ventilator settings (SVS), we hypothesised that a stratification based on baseline PaO2/FiO2 would not provide accurate assessment of lung injury severity. Design A prospective, multicentre, observational study. Setting A network of teaching hospitals. Participants 478 patients with eligible criteria for moderate (100300). Primary and secondary outcomes Group severity and hospital mortality. Results At ARDS onset, 173 patients had a PaO2/FiO2≤100 but only 38.7% met criteria for severe ARDS at 24 h under SVS. When assessed under SVS, 61.3% of patients with severe ARDS were reclassified as moderate, mild and non-ARDS, while lung severity and hospital mortality changed markedly with every PaO2/FiO2 category (p<0.000001). Our model of risk stratification outperformed the stratification using baseline PaO2/FiO2 and non-standardised PaO2/FiO2 at 24 h, when analysed by the predictive receiver operating characteristic (ROC) curve: area under the ROC curve for stratification at baseline was 0.583 (95% CI 0.525 to 0.636), 0.605 (95% CI 0.552 to 0.658) at 24 h without SVS and 0.693 (95% CI 0.645 to 0.742) at 24 h under SVS (p<0.000001). Conclusions Our findings support the need for patient assessment under SVS at 24 h after ARDS onset to assess disease severity, and have implications for the

  3. Dose-response comparisons of five lung surfactant factor (LSF) preparations in an animal model of adult respiratory distress syndrome (ARDS).

    PubMed

    Häfner, D; Beume, R; Kilian, U; Krasznai, G; Lachmann, B

    1995-06-01

    1. We have examined the effects of five different lung surfactant factor (LSF) preparations in the rat lung lavage model. In this model repetitive lung lavage leads to lung injury with some similarities to adult respiratory distress syndrome with poor gas exchange and protein leakage into the alveolar spaces. These pathological sequelae can be reversed by LSF instillation soon after lavage. 2. The tested LSF preparations were: two bovine: Survanta and Alveofact: two synthetic: Exosurf and a protein-free phospholipid based LSF (PL-LSF) and one Recombinant LSF at doses of 25, 50 and 100 mg kg-1 body weight and an untreated control group. 3. Tracheotomized rats (10-12 per dose) were pressure-controlled ventilated (Siemens Servo Ventilator 900C) with 100% oxygen at a respiratory rate of 30 breaths min-1, inspiration expiration ratio of 1:2, peak inspiratory pressure (PIP) of 28 cmH2O at positive end-expiratory pressure (PEEP) of 8 cmH2O. Two hours after LSF administration, PEEP and in parallel PIP was reduced from 8 to 6 (1st reduction), from 6 to 3 (2nd reduction) and from 3 to 0 cmH2O (3rd reduction). 4. Partial arterial oxygen pressure (PaO2, mmHg) at 5 min and 120 min after LSF administration and during the 2nd PEEP reduction (PaO2(PEEP23/3)) were used for statistical comparison. All LSF preparations caused a dose-dependent increase for the PaO2(120'), whereas during the 2nd PEEP reduction only bovine and recombinant LSF exhibited dose-dependency. Exosurf did not increase PaO2 after administration of the highest dose. At the highest dose Exosurf exerted no further improvement but rather a tendency to relapse. The bovine and the Recombinant LSF are superior to both synthetic LSFpreparations.5. In this animal model and under the described specific ventilatory settings, even between bovine LSFpreparations there are detectable differences that are pronounced when compared to synthetic LSFwithout any surfactant proteins. We conclude that the difference between bovine

  4. Short communication: Camel milk ameliorates inflammatory responses and oxidative stress and downregulates mitogen-activated protein kinase signaling pathways in lipopolysaccharide-induced acute respiratory distress syndrome in rats.

    PubMed

    Zhu, Wei-Wei; Kong, Gui-Qing; Ma, Ming-Ming; Li, Yan; Huang, Xiao; Wang, Li-Peng; Peng, Zhen-Yi; Zhang, Xiao-Hua; Liu, Xiang-Yong; Wang, Xiao-Zhi

    2016-01-01

    Acute respiratory distress syndrome (ARDS) is a complex syndrome disorder with high mortality rate. Camel milk (CM) contains antiinflammatory and antioxidant properties and protects against numerous diseases. This study aimed to demonstrate the function of CM in lipopolysaccharide (LPS)-induced ARDS in rats. Camel milk reduced the lung wet:dry weight ratio and significantly reduced LPS-induced increases in neutrophil infiltration, interstitial and intra-alveolar edema, thickness of the alveolar wall, and lung injury scores of lung tissues. It also had antiinflammatory and antioxidant effects on LPS-induced ARDS. After LPS stimulation, the levels of proinflammatory cytokines (tumor necrosis factor-α, IL-10, and IL-1β) in serum and oxidative stress markers (malondialdehyde, myeloperoxidase, and total antioxidant capacity) in lung tissue were notably attenuated by CM. Camel milk also downregulated mitogen-activated protein kinase signaling pathways. Given these results, CM is a potential complementary food for ARDS treatment.

  5. ARDS (Acute Respiratory Distress Syndrome)

    MedlinePlus

    ... Technology Transfer Clinical Trials What Are Clinical Trials? Children & Clinical Studies NHLBI Trials Clinical Trial Websites News & ... Therapy Pleurisy and Other Pleural ... underlying cause of ARDS, associated illnesses, and other factors. Some people who survive ...

  6. Acute Respiratory Distress Syndrome (ARDS)

    MedlinePlus

    ... Health & Wellness > Health Professionals Lung Health & Wellness > Healthy Air (Indoor & Outdoor) Lung Health & Wellness > LUNG FORCE Updates Lung Health & Wellness > Quit Smoking Lung Health & ...

  7. What Is Respiratory Distress Syndrome?

    MedlinePlus

    ... Clinical Trials Links Related Topics Atelectasis Bronchopulmonary Dysplasia Patent Ductus Arteriosus Pleurisy and Other Pleural Disorders Ventilator/ ... this page with Gmail. Bookmark this page with Google. Share this page from the NHLBI on LinkedIn. ...

  8. Clinical variability in neurohepatic syndrome due to combined mitochondrial DNA depletion and Gaucher disease.

    PubMed

    Harvengt, Julie; Wanty, Catherine; De Paepe, Boel; Sempoux, Christine; Revencu, Nicole; Smet, Joél; Van Coster, Rudy; Lissens, Willy; Seneca, Sara; Weekers, Laurent; Sokal, Etienne; Debray, François-Guillaume

    2014-01-01

    A 1-year-old girl born to consanguineous parents presented with unexplained liver failure, leading to transplantation at 19 months. Subsequent partial splenectomy for persistent cytopenia showed the presence of foamy cells, and Gaucher disease was confirmed by homozygosity for the p.Leu483Pro mutation in the GBA gene. She was treated by enzyme replacement therapy (ERT). Clinical follow-up showed mild developmental delay, strabismus, nystagmus and oculomotor apraxia. Biochemical studies revealed multiple respiratory chain deficiencies and a mosaic pattern of deficient complex IV immunostaining in liver and fibroblast. Molecular analysis identified a mtDNA depletion syndrome due to the homozygous p.Pro98Leu mutation in MPV17. A younger sister unaffected by mtDNA depletion, presented with pancytopenia and hepatosplenomegaly. ERT for Gaucher disease resulted in visceral normalization without any neurological symptom. A third sister, affected by both conditions, had marked developmental delay, strabismus and ophthalmoplegia but no liver cirrhosis. In conclusion, intrafamilal variability occurs in MPV17-related disease. The combined pathological effect of Gaucher and mitochondrial diseases can negatively impact neurological and liver functions and influence the outcome in consanguineous families. The immunocytochemical staining of OXPHOS protein in tissues and cultured cells is a powerful tool revealing mosaic pattern of deficiency pointing to mtDNA-related mitochondrial disorders.

  9. Heart failure due to ‘stress cardiomyopathy’: a severe manifestation of the opioid withdrawal syndrome

    PubMed Central

    Spadotto, Veronica; Zorzi, Alessandro; ElMaghawry, Mohamed; Pittoni, Giovanni Maria

    2013-01-01

    Takotsubo cardiomyopathy (TTC) is a transient left ventricular (LV) dysfunction due to akinesia of the LV mid-apical segments (‘apical ballooning’) in the absence of critical coronary stenoses which can be complicated in the acute phase by heart failure, mitral regurgitation, life-threatening ventricular arrhythmias, or apical LV thrombosis. The syndrome is typically precipitated by intense emotional or physical stress; however, other causes of sympathetic overstimulation including administration of exogenous sympathomimetics or withdrawal of sympathetic antagonists can trigger TTC. We report the case of a patient who unexpectedly developed an ‘apical ballooning’ with severe reduction in the LV systolic function and heart failure after the withdrawal of methadone. The case supports the concept that increased sympathetic activity secondary to opioids withdrawal can trigger a stress-induced severe LV dysfunction. Physicians should be aware that the abrupt discontinuation of a long-term therapy with opioids may lead to serious cardiac complications. The administration of clonidine may be considered to prevent early clinical manifestations of addictive withdrawal, including TTC. PMID:24062938

  10. A case of contact urticaria syndrome due to di(2-ethylhexyl) phthalate (DOP) in work clothes.

    PubMed

    Sugiura, Keiji; Sugiura, Mariko; Hayakawa, Ritsuko; Shamoto, Mikihiro; Sasaki, Kazumi

    2002-01-01

    We previously reported a case of contact urticaria syndrome (CUS) due to di(2-ethylhexyl) phthalate (DOP) in a polyvinyl chloride (PVC) grip on cotton gloves. The patient reported in this previous paper was careful not to have any contact with PVC products in his daily life or in his working environment. He discontinued the use of protective gloves with a PVC grip that was the cause of CUS. When working, he used cotton gloves without a PVC grip. We prescribed antihistamines which slightly improved his condition. However, when he wore work clothes while on duty, CUS relapsed. This condition was severe and made him feel anxious. When we advised him to wear a cotton shirt under his work clothes, the contact urticaria did not develop. We suspected that some component of the work clothes was the cause of his symptoms. A prick test with the extract solution of his work clothes showed a wheal and flare at the 15 min reading. The common component of the grip and the work clothes was found by analysis to be DOP.

  11. [Immune reconstitution syndrome due to BCG in HIV-treated children].

    PubMed

    Miranda-Choque, Edwin; Candela-Herrera, Jorge; R Segura, Eddy; Farfán-Ramos, Sonia; Barriga, Aldo

    2012-01-01

    The objective of this study is to describe the clinical profile of the immune reconstitution syndrome due to Mycobacterium bovis Bacillus Calmette-Guérin (IRS-BCG) in children with HIV infection who receive highly active antiretroviral treatment (HAART) at Instituto Nacional de Salud del Niño de Lima (National Children's Health Institute of Lima), Peru. A case study was conducted, including 8 children with IRS-BCG, defined as the presence of regional lymphadenopathy or inflammation on the BCG vaccination site with at least one less logarithm in the viral load or immune improvement. All patients had AIDS (C3). The starting median age in HAART was 7.2 months and the event occurred 3 to 11 weeks after the treatment was started. 7 cases showed axillary adenitis. When compared with the Non IRS-BCG group, a significant association between the age at which HAART was started at one year, severe immunodepression, and increased viral load was found. It is concluded that IRS-BCG was related to a rapid clinical progression of the mother-to-child transmitted HIV/AIDS infection, severe immunosuppression and high viral load when the HAART began.

  12. Trauma due to Self-aggression in Patient with Waardenburg Syndrome associated with Congenital Anomalies.

    PubMed

    Marta, Sara Nader; Kawakami, Roberto Yoshio; Sgavioli, Claudia Almeida Prado Piccino; Correa, Ana Eliza; D'Árk de Oliveira El Kadre, Guaniara; Carvalho, Ricardo Sandri

    2016-08-01

    Waardenburg syndrome (WS) is an inherited autosomal dominant genetic disorder presenting variable penetrance and expressivity, with an estimated prevalence of 1:42,000. Clinical characteristics of WS include lateral displacement of the internal eye canthus, hyperplasia of the medial portion of the eyebrows, prominent and broad nasal base, congenital deafness, pigmentation of the iris and skin, and white forelock. A 24-year-old male patient, previously diagnosed with WS, was referred to the Special Needs Dental Clinic of Sacred Heart University, Bauru, Brazil. Parents reported that the patient was experiencing self-mutilation, particularly in the oral region. He presented multiple congenital anomalies, including anophthalmia, mental retardation, low-set ears, and leg deformities. Clinical oral examination revealed hypodontia, abnormalities in dental morphology, extensive dental caries, periodontal disease, and fistulae. Extensive scars on the tongue, lips, and hands caused by self-mutilation were also observed. In accordance with his family and neurologist, full-mouth extraction under general anesthesia was performed, especially considering his severe self-aggressive behavior and the necessity to be fed with soft-food diet due to his inability to chew. After the surgical procedure, a significant reduction in the patient's irritability and gain of weight were reported in the follow-ups of 30, 60, and 180 days.

  13. [Pulmonary and cardiovascular syndrome due to hantavirus: clinical aspects of an emerging disease in southeastern Brazil].

    PubMed

    Campos, Gelse Mazzoni; Borges, Alessandra Abel; Badra, Soraya Jabur; Figueiredo, Glauciane Garcia; Souza, Ricardo Luiz Moro de; Moreli, Marcos Lázaro; Figueiredo, Luiz Tadeu Moraes

    2009-01-01

    Pulmonary and cardiovascular syndrome due to hantavirus is a disease caused by inhalation of aerosols from the excreta of wild rodents contaminated by viruses of the Bunyaviridae family. We studied the clinical and laboratory manifestations of 70 cases that occurred in the region of Ribeirão Preto, SP, Brazil, between 1998 and 2007. The frequency of symptoms was as follows: dyspnea (87%), fever (81%), coughing (44%), headache (34%), tachycardia (81%), low arterial blood pressure (56%), metabolic acidosis (57%), lymphocytopenia (51%), hematocrit > 45% (70%), leukocytosis with left deviation (67%), creatinine (51%) and urea (42%). Mortality (54.3%) occurred mainly on the fourth day. Respiratory insufficiency, low arterial blood pressure and shock occurred after 24 to 48 hours. High hematocrit and decreased platelet levels were signs strongly suggestive of the disease. The diagnostic hypothesis of atypical pneumonia was associated with a good prognosis (p = 0.0136). Fluid infusion greater than 2,000 ml and arterial hypotension were associated with a poor prognosis (p = 0.0286 and p = 0.0453).

  14. Acute Budd-Chiari syndrome due to a simple liver cyst.

    PubMed

    Long, J; Vaughan-Williams, H; Moorhouse, J; Sethi, H; Kumar, N

    2014-01-01

    Simple liver cysts are common, rarely causing significant morbidity or mortality. Budd-Chiari syndrome (BCS) is caused by obstruction of hepatic venous outflow and is the leading cause of postsinusoidal liver failure. We present a rare case of BCS caused by a simple hepatic cyst. A 16 cm × 16 cm liver cyst was found on computed tomography of a 66-year-old woman presenting with abdominal pain. The cyst had become infected, thus enlarged, exerting mass effect with almost complete compression of the inferior vena cava. Shortly after admission, the patient developed acute liver failure, with deranged clotting and hepatic encephalopathy requiring full organ support on the intensive care unit. Cardiac output studies showed a low cardiac index of 1.4 l/min/m(2). An emergency laparotomy with fenestration of the cyst and drainage of 2l of purulent material led to a full recovery. Intraoperative cystic fluid aspirates later confirmed no evidence of Echinococcus. Histology confirmed a simple cyst. Liver biopsies showed severe, confluent, bridging necrosis, without background parenchymal liver disease. Acute BCS due to rapid compression of all major hepatic veins leading to fulminant hepatic failure is rare. Our case highlights a clinically significant complication of a simple liver cyst of which clinicians should be aware when managing these 'innocent' lesions.

  15. Financial Distress in Cancer Patients

    PubMed Central

    de Souza, Jonas A.; Wong, Yu-Ning

    2013-01-01

    Novel diagnostic and therapeutic options offer hope to cancer patients with both localized and advanced disease. However, many of these treatments are often costly and even well-insured patients can face high out-of-pocket costs. Families may also be at risk of financial distress due to lost wages and other treatment-related expenses. Research is needed to measure and characterize financial distress in cancer patients and understand how it affects their quality of life. In addition, health care providers need to be trained to counsel patients and their families so they can make patient-centered treatment decisions that reflect their preferences and values. PMID:24349677

  16. Mental health status of people isolated due to Middle East Respiratory Syndrome

    PubMed Central

    2016-01-01

    OBJECTIVES Isolation due to the management of infectious diseases is thought to affect mental health, but the effects are still unknown. We examined the prevalence of anxiety symptoms and anger in persons isolated during the Middle East Respiratory Syndrome (MERS) epidemic both at isolation period and at four to six months after release from isolation. We also determined risk factors associated with these symptoms at four to six months. METHODS Of 14,992 individuals isolated for 2-week due to having contact with MERS patients in 2015, when MERS was introduced to Korea, 1,692 individuals were included in this study. Anxiety symptoms were evaluated with the Generalized Anxiety Disorder 7-item scale and anger was assessed with the State-Trait Anger Expression Inventory at four to six months after release from isolation for MERS. RESULTS Of 1,692 who came in contact with MERS patients, 1,656 were not diagnosed with MERS. Among 1,656, anxiety symptoms showed 7.6% (95% confidence interval [CI], 6.3 to 8.9%) and feelings of anger were present in 16.6% (95% CI, 14.8 to 18.4%) during the isolation period. At four to six months after release from isolation, anxiety symptoms were observed in 3.0% (95%CI, 2.2 to 3.9%). Feelings of anger were present in 6.4% (95% CI, 5.2 to 7.6%). Risk factors for experiencing anxiety symptoms and anger at four to six months after release included symptoms related to MERS during isolation, inadequate supplies (food, clothes, accommodation), social networking activities (email, text, Internet), history of psychiatric illnesses, and financial loss. CONCLUSIONS Mental health problems at four to six month after release from isolation might be prevented by providing mental health support to individuals with vulnerable mental health, and providing accurate information as well as appropriate supplies, including food, clothes, and accommodation. PMID:28196409

  17. Reactive airways dysfunction syndrome in housewives due to a bleach-hydrochloric acid mixture.

    PubMed

    Gorguner, Metin; Aslan, Sahin; Inandi, Tacettin; Cakir, Zeynep

    2004-02-01

    The sudden onset of asthmalike symptoms and persistence of airway reactivity following an acute exposure to an irritant gas or vapor has been termed reactive airways dysfunction syndrome (RADS). A mixture of sodium hypochlorite (bleach, 40%) and hydrochloric acid (18%) is commonly used as a household cleaning solution in our region. From this mixture, chlorine gas is produced, which can cause airway damage and ensuing RADS. Here we describe findings of patients with RADS due to this cleaning mixture, and determine factors associated with a favorable outcome. Data were collected retrospectively on 55 symptomatic patients presenting to our emergency department after inhalation exposure to a mixture of bleach and hydrochloric acid. Symptoms, past medical and smoking history, details of the exposure, initial peak expiratory flow rate (PEFR) and oxygenation, and acute reversibility of airways obstruction were documented. All patients met previously defined criteria for the diagnosis of RADS, but did not undergo methacholine challenge testing and bronchoalveolar lavage or histopathologic study. Fifty patients were followed over the course of 3 mo. The majority of exposures (64%) occurred in the bathroom or kitchen. Only 21 of 55 (38%) patients showed an improvement in PEFR of 15% or greater following two beta(2)-agonist inhalation treatments. In follow-up, 48 patients (87%) improved clinically and functionally (FEV(1)). Seven patients (13%) deteriorated, with ARDS developing in two, one of whom died from respiratory failure. Advanced age, initial low PEFR, exposure in a small enclosed area, use immediately after mixing, and prolonged short- and long-term exposures were associated with a poorer prognosis. This descriptive study is the largest case series in the literature of RADS developing after exposure to a bleach-hydrochloric acid mixture. The optimum acute treatment and long-term outcomes for patients with RADS due to this combination still need to be determined.

  18. [Severe type A insulin resistance syndrome due to a mutation in the insulin receptor gene].

    PubMed

    Ros, P; Colino-Alcol, E; Grasso, V; Barbetti, F; Argente, J

    2015-01-01

    Insulin resistance syndromes without lipodystrophy are an infrequent and heterogeneous group of disorders with variable clinical phenotypes, associated with hyperglycemia and hyperinsulinemia. The three conditions related to mutations in the insulin receptor gene are leprechaunism or Donohue syndrome, Rabson-Mendenhall syndrome, and Type A syndrome. A case is presented on a patient diagnosed with type A insulin resistance, defined by the triad of extreme insulin resistance, acanthosis nigricans, and hyperandrogenism, carrying a heterozygous mutation in exon 19 of the insulin receptor gene coding for its tyrosine kinase domain that is crucial for the catalytic activity of the receptor. The molecular basis of the syndrome is reviewed, focusing on the structure-function relationships of the insulin receptor, knowing that the criteria for survival are linked to residual insulin receptor function. It is also pointed out that, although type A insulin resistance appears to represent a somewhat less severe condition, these patients have a high morbidity and their treatment is still unsatisfactory.

  19. Ulnar nerve entrapment in Guyon's canal due to recurrent carpal tunnel syndrome: case report.

    PubMed

    Ozdemir, Ozgur; Calisaneller, Tarkan; Gulsen, Salih; Caner, Hakan

    2011-01-01

    Guyon's canal syndrome is a compression neuropathy of the ulnar nerve entrapment at the wrist. Compression of the ulnar nerve at the wrist by a ganglion, lipomas, diseases of the ulnar artery, fractures of the hamate and trauma are common etiologcal factors. Unlike Guyon's canal syndrome, carpal tunnel syndrome (CTS) is the most common nerve entrapment of the upper extremity. Although, open (OCTR) or endoscopic carpal tunnel release (ECTR) is highly effective in relieving pain, failure with carpal tunnel release is seldom seen. In this paper, we presented a patient with ulnar nerve entrapment associated with recurrent CTS and discussed the possible pathomechanism with a review of current literature.

  20. [Visual impairment in juvenile diabetes mellitus due to optic atrophy: Wolfram's syndrome].

    PubMed

    Immink, Annelies; Reeser, H Maarten; Brus, Frank

    2010-01-01

    Wolfram's syndrome is a rare neurodegenerative disorder, which usually first manifests itself around the age of 6 years. The diagnosis can be made based on the characteristics incorporated in the 'DIDMOAD' acronym: diabetes insipidus, diabetes mellitus, optic atrophy and deafness. We present 2 boys, diagnosed with diabetes mellitus at the age of 5 and 4 years respectively. Both children developed optic atrophy over the years. These 2 cases illustrate that alongside diabetic retinopathy, possible syndromes, such as Wolfram's syndrome, should also be considered in children with diabetes mellitus and visual impairment.

  1. The Phenotype of the Musculocontractural Type of Ehlers-Danlos Syndrome due to CHST14 Mutations

    PubMed Central

    Janecke, Andreas R.; Li, Ben; Boehm, Manfred; Krabichler, Birgit; Rohrbach, Marianne; Müller, Thomas; Fuchs, Irene; Golas, Gretchen; Katagiri, Yasuhiro; Ziegler, Shira G.; Gahl, William A.; Wilnai, Yael; Zoppi, Nicoletta; Geller, Herbert M.; Giunta, Cecilia; Slavotinek, Anne; Steinmann, Beat

    2016-01-01

    The musculocontractural type of Ehlers-Danlos syndrome (MC-EDS) has been recently recognized as a clinical entity. MC-EDS represents a differential diagnosis within the congenital neuromuscular and connective tissue disorders spectrum. Thirty-one and three patients have been reported with MC-EDS so far with biallelic mutations identified in CHST14 and DSE, respectively, encoding two enzymes necessary for dermatan sulfate (DS) biosynthesis. We report seven additional patients with MC-EDS from four unrelated families, including the follow-up of a sib-pair originally reported with the kyphoscoliotic type of EDS in 1975. Brachycephaly, a characteristic facial appearance, an asthenic build, hyperextensible and bruisable skin, tapering fingers, instability of large joints, and recurrent formation of large subcutaneous hematomas are always present. Three of seven patients hadmildly elevated serum creatine kinase. The oldest patient was blind due to retinal detachment at 45 years and died at 59 years from intracranial bleeding; her affected brother died at 28 years from fulminant endocarditis. All patients in this series harbored homozygous, predicted loss-of-function CHST14 mutations. Indeed, DS was not detectable in fibroblasts from two unrelated patients with homozygous mutations. Patient fibroblasts produced higher amounts of chondroitin sulfate, showed intracellular retention of collagen types I and III, and lacked decorin and thrombospondin fibrils compared with control. A great proportion of collagen fibrils were not integrated into fibers, and fiber bundles were dispersed into the ground substance in one patient, all of which is likely to contribute to the clinical phenotype. This report should increase awareness for MC-EDS. PMID:26373698

  2. Lemierre’s syndrome due to intratumoral abscess of the uvula

    PubMed Central

    de Campos, Fernando Peixoto Ferraz; Ferreira, Cristiane Rubia; Felipe-Silva, Aloisio

    2015-01-01

    Lemierre’s syndrome (LS), described in detail in 1936, used to be a life-threatening entity until the advent of antibiotics. Tonsillitis or pharyngitis are the main primary infections and oropharyngeal anaerobic flora is the predominant etiology. However, other primary site infections, as well as other microbiological agents have been reported since the first description. Inflammatory symptoms in the neck and marked findings on physical examination predominate the majority of cases. Nonetheless, the authors report the case of a 54-year-old man with a history of dysphagia followed by cough, purulent expectoration, and fever. The bad condition of his dentition was noteworthy. During the diagnostic work-up, an ulcerated lesion in the uvula and a middle lobe pneumonia were disclosed. Streptococcus viridans was isolated from blood culture. On the fifth day of hospitalization, the patient died after a copious episode of hemoptysis. The autopsy findings depicted an abscess within a squamous cell carcinoma of the uvula, pharyngitis with carotid sheath spreading accompanied by pylephlebitis and thrombosis of the internal jugular vein up to the innominate vein, surrounded by an abscess in the mediastinum. Alveolar hemorrhage and pneumonia were also present. We conclude that the ulcerated carcinoma of the uvula housed an abscess, facilitated by the poor oral hygiene, which triggered LS and the descending mediastinitis. Pulmonary involvement was due to the septic embolism from the internal jugular vein. We would like to highlight the uvula abscess as the primary site of infection in this case of LS with S. viridans as the causative agent. PMID:26558242

  3. Specific MRI Abnormalities Reveal Severe Perrault Syndrome due to CLPP Defects

    PubMed Central

    Theunissen, Tom E. J.; Szklarczyk, Radek; Gerards, Mike; Hellebrekers, Debby M. E. I.; Mulder-Den Hartog, Elvira N. M.; Vanoevelen, Jo; Kamps, Rick; de Koning, Bart; Rutledge, S. Lane; Schmitt-Mechelke, Thomas; van Berkel, Carola G. M.; van der Knaap, Marjo S.; de Coo, Irenaeus F. M.; Smeets, Hubert J. M.

    2016-01-01

    In establishing a genetic diagnosis in heterogeneous neurological disease, clinical characterization and whole exome sequencing (WES) go hand-in-hand. Clinical data are essential, not only to guide WES variant selection and define the clinical severity of a genetic defect but also to identify other patients with defects in the same gene. In an infant patient with sensorineural hearing loss, psychomotor retardation, and epilepsy, WES resulted in identification of a novel homozygous CLPP frameshift mutation (c.21delA). Based on the gene defect and clinical symptoms, the diagnosis Perrault syndrome type 3 (PRLTS3) was established. The patient’s brain-MRI revealed specific abnormalities of the subcortical and deep cerebral white matter and the middle blade of the corpus callosum, which was used to identify similar patients in the Amsterdam brain-MRI database, containing over 3000 unclassified leukoencephalopathy cases. In three unrelated patients with similar MRI abnormalities the CLPP gene was sequenced, and in two of them novel missense mutations were identified together with a large deletion that covered part of the CLPP gene on the other allele. The severe neurological and MRI abnormalities in these young patients were due to the drastic impact of the CLPP mutations, correlating with the variation in clinical manifestations among previously reported patients. Our data show that similarity in brain-MRI patterns can be used to identify novel PRLTS3 patients, especially during early disease stages, when only part of the disease manifestations are present. This seems especially applicable to the severely affected cases in which CLPP function is drastically affected and MRI abnormalities are pronounced. PMID:27899912

  4. The phenotype of the musculocontractural type of Ehlers-Danlos syndrome due to CHST14 mutations.

    PubMed

    Janecke, Andreas R; Li, Ben; Boehm, Manfred; Krabichler, Birgit; Rohrbach, Marianne; Müller, Thomas; Fuchs, Irene; Golas, Gretchen; Katagiri, Yasuhiro; Ziegler, Shira G; Gahl, William A; Wilnai, Yael; Zoppi, Nicoletta; Geller, Herbert M; Giunta, Cecilia; Slavotinek, Anne; Steinmann, Beat

    2016-01-01

    The musculocontractural type of Ehlers-Danlos syndrome (MC-EDS) has been recently recognized as a clinical entity. MC-EDS represents a differential diagnosis within the congenital neuromuscular and connective tissue disorders spectrum. Thirty-one and three patients have been reported with MC-EDS so far with bi-allelic mutations identified in CHST14 and DSE, respectively, encoding two enzymes necessary for dermatan sulfate (DS) biosynthesis. We report seven additional patients with MC-EDS from four unrelated families, including the follow-up of a sib-pair originally reported with the kyphoscoliotic type of EDS in 1975. Brachycephaly, a characteristic facial appearance, an asthenic build, hyperextensible and bruisable skin, tapering fingers, instability of large joints, and recurrent formation of large subcutaneous hematomas are always present. Three of seven patients had mildly elevated serum creatine kinase. The oldest patient was blind due to retinal detachment at 45 years and died at 59 years from intracranial bleeding; her affected brother died at 28 years from fulminant endocarditis. All patients in this series harbored homozygous, predicted loss-of-function CHST14 mutations. Indeed, DS was not detectable in fibroblasts from two unrelated patients with homozygous mutations. Patient fibroblasts produced higher amounts of chondroitin sulfate, showed intracellular retention of collagen types I and III, and lacked decorin and thrombospondin fibrils compared with control. A great proportion of collagen fibrils were not integrated into fibers, and fiber bundles were dispersed into the ground substance in one patient, all of which is likely to contribute to the clinical phenotype. This report should increase awareness for MC-EDS.

  5. The effects of salbutamol on epithelial ion channels depend on the etiology of acute respiratory distress syndrome but not the route of administration

    PubMed Central

    2014-01-01

    Introduction We investigated the effects of intravenous and intratracheal administration of salbutamol on lung morphology and function, expression of ion channels, aquaporin, and markers of inflammation, apoptosis, and alveolar epithelial/endothelial cell damage in experimental pulmonary (p) and extrapulmonary (exp) mild acute respiratory distress syndrome (ARDS). Methods In this prospective randomized controlled experimental study, 56 male Wistar rats were randomly assigned to mild ARDS induced by either intratracheal (n = 28, ARDSp) or intraperitoneal (n = 28, ARDSexp) administration of E. coli lipopolysaccharide. Four animals with no lung injury served as controls (NI). After 24 hours, animals were anesthetized, mechanically ventilated in pressure-controlled mode with low tidal volume (6 mL/kg), and randomly assigned to receive salbutamol (SALB) or saline 0.9% (CTRL), intravenously (i.v., 10 μg/kg/h) or intratracheally (bolus, 25 μg). Salbutamol doses were targeted at an increase of ≈ 20% in heart rate. Hemodynamics, lung mechanics, and arterial blood gases were measured before and after (at 30 and 60 min) salbutamol administration. At the end of the experiment, lungs were extracted for analysis of lung histology and molecular biology analysis. Values are expressed as mean ± standard deviation, and fold changes relative to NI, CTRL vs. SALB. Results The gene expression of ion channels and aquaporin was increased in mild ARDSp, but not ARDSexp. In ARDSp, intravenous salbutamol resulted in higher gene expression of alveolar epithelial sodium channel (0.20 ± 0.07 vs. 0.68 ± 0.24, p < 0.001), aquaporin-1 (0.44 ± 0.09 vs. 0.96 ± 0.12, p < 0.001) aquaporin-3 (0.31 ± 0.12 vs. 0.93 ± 0.20, p < 0.001), and Na-K-ATPase-α (0.39 ± 0.08 vs. 0.92 ± 0.12, p < 0.001), whereas intratracheal salbutamol increased the gene expression of aquaporin-1 (0.46 ± 0.11 vs. 0.92 ± 0.06, p < 0

  6. Comparison of rSP-C surfactant with natural and synthetic surfactants after late treatment in a rat model of the acute respiratory distress syndrome.

    PubMed

    Häfner, D; Germann, P G; Hauschke, D

    1998-07-01

    1. In a previous paper we showed that an SP-C containing surfactant preparation has similar activity as bovine-derived surfactants in a rat lung lavage model of the adult respiratory distress syndrome. In this study surfactant was given ten minutes after the last lavage (early treatment). In the present investigation we were interested how different surfactant preparations behave when they are administered 1 h after the last lavage (late treatment). 2. Four protein containing surfactants (rSP-C surfactant, bLES, Infasurf and Survanta) were compared with three protein-free surfactants (ALEC, Exosurf and the phospholipid (PL) mixture of the rSP-C surfactant termed PL surfactant) with respect to their ability to improve gas exchange in this more stringent model when surfactant is given one hour after the last lavage. For better comparison of the surfactants the doses were related to phospholipids. The surfactants were given at doses of 25, 50 and 100 mg kg(-1) body weight. The surfactants were compared to an untreated control group that was only ventilated for the whole experimental period. 3. Tracheotomized rats (8-12 per dose and surfactant) were pressure-controlled ventilated (Siemens Servo Ventilator 900C) with 100% oxygen at a respiratory rate of 30 breaths min(-1), inspiration expiration ratio of 1:2, peak inspiratory pressure of 28 cmH2O at positive endexpiratory pressure (PEEP) of 8 cmH2O. Animals were ventilated for one hour after the last lavage and thereafter the surfactants were intratracheally instilled. During the whole experimental period the ventilation was not changed. 4. Partial arterial oxygen pressures (PaO2, mmHg) at 30 min and 120 min after treatment were used for statistical comparison. All protein containing surfactants caused a dose-dependent increase of the reduced PaO2 values at 30 min after treatment. The protein-free surfactants showed only weak dose-dependent increase in PaO2 values at this time. This difference between the protein

  7. Effect of setting high APRV guided by expiratory inflection point of pressure-volume curve on oxygen delivery in canine models of severe acute respiratory distress syndrome

    PubMed Central

    Li, Jia-Qiong; Xu, Hong-Yang; Li, Mao-Qin; Chen, Jing-Yu

    2016-01-01

    In the present study, the effect of setting high airway pressure release ventilation (APRV) pressure guided by an expiratory inflection point of pressure-volume (PV) curve following lung recruitment maneuver (RM) on oxygen delivery (DO2) in canine models of severe acute respiratory distress syndrome (ARDS) was examined. Canine models of severe ARDS were established by intravenous injection of oleic acid. After injection of sedative muscle relaxants, a PV curve plotted using the super-syringe technique, and the pressure at lower inflection point (LIP) at the inhale branch and the pressure at the point of maximum curvature (PMC) at the exhale branch were measured. The ventilation mode was biphasic positive airway pressure (BiPAP), an inspiration to expiration ratio of 1:2, and Phigh 40 cm H2O, Plow 25 cm H2O. Phigh was decreased to 30 cm H2O after 90 sec. The dogs were randomized into 3 groups after RM, i.e., Blip group, BiPAP Plow = LIP+2 cm H2O; Bpmc group, BiPAP Plow = PMC; and Apmc group. In the APRV group, Phigh was set as PMC, with an inspiratory duration of 4 sec and expiratory duration of 0.4 sec. PMC was 18±1.4 cm H2O, and LIP was 11±1.3 cm H2O. Thirty seconds after RM was stabilized, it was set as 0 h. Hemodynamics, oxygenation and DO2 were measured at 0, 1, 2 and 4 h after RM in ARDS dogs. The results demonstrated: i) cardiac index (CI) in the 3 groups, where CI was significantly decreased in the Bpmc group at 0, 1, 2 and 4 h after RM compared to prior to RM (P<0.05) as well as in the Blip and Apmc groups (P<0.05). CI in the Blip and Apmc groups was not significantly altered prior to and after RM. ii) Oxygenation at 0, 1, 2 and 4 h in the 3 groups was improved after RM and the oxygenation indices for the 3 groups at 1 and 2 h were not significantly different (P>0.05). However, the oxygenation index in the Blip group at 4 h was significantly lower than those at 0 h for the Apmc and Bpmc groups (P<0.05). Oxygenation for the Apmc group at 4 h was higher

  8. Acute Liver Failure Due to Budd-Chiari Syndrome in the Setting of Cardiac Synovial Sarcoma.

    PubMed

    Stine, Jonathan G; Newton, Kelly; Vinayak, Ajeet G

    2015-04-01

    Primary malignant tumors of the heart, specifically cardiac sarcomas, are rare and mainly diagnosed at autopsy. Acute Budd-Chiari syndrome is a recognized cause of acute liver failure and has been associated with several rare cardiac tumors: atrial myxoma, caval rhabdomyosarcoma, and primary cardiac adenocarcinoma. We present the first case of a fatal, highly differentiated cardiac synovial sarcoma that presented as acute liver failure from Budd-Chiari syndrome.

  9. [Acute respiratory distress revealing severe pulmonary leptospirosis].

    PubMed

    Sekkach, Y; Qaçif, H; Jira, M; El Qatni, M; El omri, N; Ghafir, D

    2007-01-01

    We return a clinical case of leptospirose revelated by a complicated febrile harp pneumopathie of a sharp respiratory distress syndrome having required a transfer in resuscitation. The goal of our article is to recall that it is necessary to think systematically about a pulmonary shape of leptospirose facing an atypical pneumopahie.

  10. Acute respiratory distress caused by Neosartorya udagawae

    Technology Transfer Automated Retrieval System (TEKTRAN)

    We describe the first reported case of acute respiratory distress syndrome (ARDS) attributed to Neosartorya infection. The mold grew rapidly in culture of both sputum and bronchoalveolar lavage (BAL) fluid from a previously healthy 43-year-old woman with ARDS, which developed as the culmination of a...

  11. Abdominal compartment syndrome and acute kidney injury due to excessive auto-positive end-expiratory pressure.

    PubMed

    Matthew, Dwight; Oxman, David; Djekidel, Karim; Ahmed, Ziauddin; Sherman, Michael

    2013-02-01

    Abdominal compartment syndrome is an under-recognized cause of acute kidney injury in critically ill patients. We report a case of a patient with severe obstructive lung disease who, while intubated for respiratory failure, developed abdominal compartment syndrome and oliguric acute kidney injury due to air-trapping and excessive auto-positive end-expiratory pressure (auto-PEEP; also known as intrinsic PEEP). When chemical paralysis was initiated and the auto-PEEP resolved, the patient's intra-abdominal hypertension rapidly improved and kidney function recovered immediately. Abdominal compartment syndrome secondary to excessive auto-PEEP appears to be unreported in the literature; however, any process that significantly increases intrathoracic pressure conceivably could cause increased pressure to be transmitted to the abdominal compartment, resulting in organ failure. Patients undergoing mechanical ventilation, which puts them at risk of airflow obstruction and the development of intra-abdominal hypertension, should be evaluated for air-trapping and excessive auto-PEEP.

  12. Pisa-Like Syndrome Under Baclofen in a Patient With Spastic Hemiparesis due to Ischemic Stroke.

    PubMed

    Cannas, Antonino; Solla, Paolo; Mascia, Marcello; Muroni, Antonella; Floris, Gian Luca; Borghero, Giuseppe; Orofino, Gianni; Meloni, Mario; Marrosu, Francesco

    2015-01-01

    In its original description, Pisa syndrome was reported as an iatrogenic dystonia of the trunk caused by neuroleptic drugs. However, sometimes, not dystonic lateral flexion of the trunk is described as Pisa syndrome. These observations support the possibility of a drug-induced lateral flexion of the trunk with clinical presentation similar to Pisa syndrome, although with a different etiology and pathophysiology. Here, we describe the case of a male patient, with a previous ischemic stroke and residual spastic hemiparesis to the right side, who subacutely developed a dramatic lateral flexion of trunk (approximately 45° to the right) a few days after the introduction of Baclofen (5 mg 3 times per day). After the discontinuation of baclofen, a full recovery of the correct posture was obtained. In this respect, our case is paradigmatic: it is drug-induced but not clearly dystonic in its manifestation. Baclofen reduces the spasticity depressing the monosinaptic and polisinaptic reflex in the spinal cord by stimulating Gamma-aminobutyric acid B (GABA-B) receptors, which inhibit the release of excitatory amino acids, glutamate and aspartate. We believe that the definition of Pisa syndrome for these forms, not clearly dystonic, might be not completely appropriate, but they should be defined more correctly as Pisa-like syndromes.

  13. Is the frontal dysexecutive syndrome due to a working memory deficit? Evidence from patients with stroke.

    PubMed

    Roussel, Martine; Dujardin, Kathy; Hénon, Hilde; Godefroy, Olivier

    2012-07-01

    Although frontal dysexecutive disorders are frequently considered to be due to working memory deficit, this has not been systematically examined and very little evidence is available for impairment of working memory in frontal damage. The objective of this study was to examine the components of working memory, their anatomy and the relations with executive functions in patients with stroke involving the frontal or posterior cortex. The study population consisted of 29 patients (frontal: n=17; posterior: n=12) and 29 matched controls. Phonological loop (letter and word spans, phonological store; rehearsal process), visuospatial sketchpad (visuospatial span) and the central executive (working memory span, dual task and updating process) were examined. The group comparison analysis showed impairment in the frontal group of: (i) verbal spans (P<0.03); (ii) with a deficit of the rehearsal process (P=0.006); (iii) visuospatial span (P=0.04); (iv) working memory span (P=0.001) that disappeared after controlling for verbal span and (v) running memory (P=0.05) unrelated to updating conditions. The clinical anatomical correlation study showed that impairment of the central executive depended on frontal and posterior lesion. Cognitive dysexecutive disorders were observed in 11/20 patients with central executive deficit and an inverse dissociation was observed in two patients. Receiver operating characteristic curve analysis indicated that cognitive dysexecutive disorders had the highest ability to discriminate frontal lesions (area under curve=0.844, 95% confidence interval: 0.74-0.95; P=0.0001; central executive impairment: area under curve=0.732, 95% confidence interval: 0.57-0.82; P=0.006). This study reveals that frontal lesions induce mild impairment of short-term memory associated with a deficit of the rehearsal process supporting the role of the frontal lobe in this process; the central executive depends on lesions in the frontal lobe and posterior regions accounting

  14. A new overgrowth syndrome is due to mutations in RNF125.

    PubMed

    Tenorio, Jair; Mansilla, Alicia; Valencia, María; Martínez-Glez, Víctor; Romanelli, Valeria; Arias, Pedro; Castrejón, Nerea; Poletta, Fernando; Guillén-Navarro, Encarna; Gordo, Gema; Mansilla, Elena; García-Santiago, Fé; González-Casado, Isabel; Vallespín, Elena; Palomares, María; Mori, María A; Santos-Simarro, Fernando; García-Miñaur, Sixto; Fernández, Luis; Mena, Rocío; Benito-Sanz, Sara; del Pozo, Ángela; Silla, Juan Carlos; Ibañez, Kristina; López-Granados, Eduardo; Martín-Trujillo, Alex; Montaner, David; Heath, Karen E; Campos-Barros, Ángel; Dopazo, Joaquín; Nevado, Julián; Monk, David; Ruiz-Pérez, Víctor L; Lapunzina, Pablo

    2014-12-01

    Overgrowth syndromes (OGS) are a group of disorders in which all parameters of growth and physical development are above the mean for age and sex. We evaluated a series of 270 families from the Spanish Overgrowth Syndrome Registry with no known OGS. We identified one de novo deletion and three missense mutations in RNF125 in six patients from four families with overgrowth, macrocephaly, intellectual disability, mild hydrocephaly, hypoglycemia, and inflammatory diseases resembling Sjögren syndrome. RNF125 encodes an E3 ubiquitin ligase and is a novel gene of OGS. Our studies of the RNF125 pathway point to upregulation of RIG-I-IPS1-MDA5 and/or disruption of the PI3K-AKT and interferon signaling pathways as the putative final effectors.

  15. Two cases of partial trisomy 10q syndrome due to a familial 10;20 translocation.

    PubMed

    Tüysüz, B; Hacihanefioglu, S; Silahtaroglu, A; Yilmaz, S; Deviren, A; Cenani, A

    2000-01-01

    We describe an eleven day-old boy and his first degree double cousin who both have distal trisomy 10q syndrome. Their cytogenetic analysis using GTG-banding showed an unbalanced translocation 46, XY, -20, +der(20), t(10;20)(q22.3, p11) mat and 46, XX, -20, +der(20), t(10;20)(q22.3, p11) mat. The translocation was confirmed by FISH. We have found balanced translocation t(10;20)(q22.3; p11) with cytogenetic and FISH studies in the mothers and maternal grandfather of these children. Our cases had typical craniofacial and visceral anomalies of this syndrome. However case 1 had an agenesia of corpus callosum which was not previously described and case 2 had hypertrophied cardiomyopathy and cliteromegaly which were previously described as rare anomalies for this syndrome.

  16. Mindfulness and bodily distress.

    PubMed

    Fjorback, Lone Overby

    2012-11-01

    We have created a mindfulness approach to treat patients who experience multiple, persistent, and disabling physical symptoms that cannot be explained by a well-defined medical or surgical condition. Randomized controlled trials in this area are few, and research is hampered by the lack of clear definitions. Bodily distress syndrome (BDS) or bodily stress is an empirically defined definition unifying various conditions such as fibromyalgia, chronic fatigue syndrome, and somatization disorder. In the present PhD, we explored whether patients suffering from BDS may be committed to mental training in the form of mindfulness therapy, which is a mindfulness program specifically targeted patients suffering from BDS. The theoretical model for including mindfulness training in the treatment of BDS is based on identified neurobiological impairments in these patients and the neurobiological improvements that mindfulness training may offer. BDS is a major public health issue possibly associated with the pathology of the immuno-endocrine and autonomic nervous system. BDS patients are often stigmatized, and effective treatment is rarely delivered, which leaves these patients isolated, left by themselves, vulnerable to potentially harming medical and/or alternative treatments. Accordingly, there is a need for non-harming practical tools that patients can learn to master so that they can improve the ability to take responsibility for their own health and wellbeing. Mindfulness-Based Stress Reduction (MBSR) is a group program that employs mindfulness practice to alleviate suffering associated with physical, psychosomatic, and psychiatric disorders. Mindfulness-Based Cognitive Therapy (MBCT) is designed to prevent depressive relapse. Paper I and II present systematic literature reviews only of randomized controlled trials on MBSR and MBCT. The effect of MBSR has been explored on fibromyalgia in three studies, none of them showed convincing results, but gave some indications as to

  17. Two patients with 'Dropped head syndrome' due to mutations in LMNA or SEPN1 genes.

    PubMed

    D'Amico, A; Haliloglu, G; Richard, P; Talim, B; Maugenre, S; Ferreiro, A; Guicheney, P; Menditto, I; Benedetti, S; Bertini, E; Bonne, G; Topaloglu, H

    2005-08-01

    Dropped head syndrome is characterized by severe weakness of neck extensor muscles with sparing of the flexors. It is a prominent sign in several neuromuscular conditions, but it may also be an isolated feature with uncertain aetiology. We report two children in whom prominent weakness of neck extensor muscles is associated with mutations in lamin A/C (LMNA) and selenoprotein N1 (SEPN1) genes, respectively. This report expands the underlying causes of the dropped head syndrome which may be the presenting feature of a congenital muscular dystrophy.

  18. Recurrent gastrointestinal perforation in a patient with Ehlers-Danlos syndrome due to tenascin-X deficiency.

    PubMed

    Sakiyama, Tomo; Kubo, Akiharu; Sasaki, Takashi; Yamada, Taketo; Yabe, Nobushige; Matsumoto, Ken-ichi; Futei, Yuko

    2015-05-01

    Ehlers-Danlos syndrome (EDS) is a clinically and genetically heterogeneous disorder. Using a customized targeted exome-sequencing system we identified nonsense mutations in TNXB in a patient who had recurrent gastrointestinal perforation due to tissue fragility. This case highlights the utility of targeted exome sequencing for the diagnosis of congenital diseases showing genetic heterogeneity, and the importance of attention to gastrointestinal perforation in patients with tenascin-X deficient type EDS.

  19. Unusual Presentation of Gianotti-Crosti Syndrome due to Epstein-Barr Virus Infection.

    PubMed

    Al Dhaheri, Hind Saif; Al Kaabi, Amani; Kara Hamo, Yasmin; Al Kaabi, Aysha; Al Kaabi, Salwa; Al Tatari, Hossam

    2016-01-01

    Gianotti-Crosti syndrome (GCS) is viral exanthema of childhood. It typically presents with a symmetric erythematous papular and papulovesicular eruption. It has been classically associated with hepatitis B virus, as well as rarely with Epstein-Barr virus (EBV). We report a case of GCS related to EBV infection without the classical systemic symptoms in a five-year-old male patient.

  20. Delayed puberty due to a novel mutation in CHD7 causing CHARGE syndrome.

    PubMed

    Dauber, Andrew; Hirschhorn, Joel N; Picker, Jonathan; Maher, Thomas A; Milunsky, Aubrey

    2010-12-01

    We report the case of a 15-year-old girl who presented to a pediatric endocrinology clinic for delayed puberty with no signs of secondary sexual development. Her past medical history was significant for bilateral colobomas, inner-ear anomalies, hearing loss, and anosmia. Genetic testing revealed a novel de novo mutation in the CHD7 gene, one of the causative genes in CHARGE syndrome (coloboma, heart disease, choanal atresia, retarded growth and development and/or central nervous system anomalies, genital anomalies and/or hypogonadism, and ear anomalies and/or deafness). We review the distinction between hypogonadotrophic hypogonadism and hypergonadotrophic hypogonadism and discuss the availability of molecular genetic testing for idiopathic hypogonadotrophic hypogonadism. CHD7 mutations have also been found in some patients with Kallmann syndrome, hypogonadotrophic hypogonadism, and anosmia, and we discuss the overlap between this syndrome and CHARGE syndrome. With the increased availability of genetic testing for a variety of disorders, it is important for pediatricians to become familiar with interpreting genetic test results. Finally, we illustrate that Bayes' theorem is a useful statistical tool for interpreting novel missense mutations of unknown significance.

  1. Surgical Treatment of Snapping Scapula Syndrome Due to Malunion of Rib Fractures.

    PubMed

    Ten Duis, Kaj; IJpma, Frank F A

    2017-02-01

    This report describes a case of snapping scapula syndrome (SSS) caused by malunited rib fractures. Abrasion of the deformed ribs was performed with good results. SSS as a cause of shoulder pain after thoracic trauma has to be considered and can be treated by a surgical abrasion technique.

  2. Are Amygdalar Volume Alterations in Children with Tourette Syndrome Due to ADHD Comorbidity?

    ERIC Educational Resources Information Center

    Ludolph, Andrea G.; Pinkhardt, Elmar H.; van Elst, Ludger Tebartz; Libal, Gerhard; Ludolph, Albert C.; Fegert, Jorg M.; Kassubek, Jan

    2008-01-01

    Recent studies have shown that changes in the basal ganglia circuitry and limbic loops may play an important role both in Tourette syndrome (TS) and attention-deficit-hyperactivity disorder (ADHD). This study aimed to investigate in vivo possible morphological alterations of the amygdala as a key component of the limbic system. Amygdalar and total…

  3. Vesicourethral reflux-induced renal failure in a patient with ICF syndrome due to a novel DNMT3B mutation.

    PubMed

    Kutluğ, Seyhan; Ogur, Gönül; Yilmaz, Aysegül; Thijssen, Peter E; Abur, Ummet; Yildiran, Alisan

    2016-12-01

    ICF syndrome is a primary immunodeficiency disease characterized by hypo- or agammaglobulinemia, centromeric instability mainly on chromosomes 1, 9, and 16 and facial anomalies. ICF syndrome presents with frequent respiratory tract infections in infancy. A 20-month-old female patient was referred to our clinic due to frequent lower respiratory tract infections. ICF syndrome was considered because of comorbidity of hypogammaglobulinemia, facial anomalies, and neuromotor growth retardation. Metaphase chromosome analysis revealed centromeric instability on chromosomes 1, 9, and 16 and through Sanger a previously unreported homozygous missense mutation (c.1805T>C; [p.V602A]) was identified in the DNMT3B, confirming ICF1. The patient was found to have a breakdown in renal function 1 year later; the urinary system was examined and bilateral vesicoureteral reflux was found, warranting the need for dialysis in time. This report expands the mutation spectrum of ICF1 and is the first to describe bilateral vesicoureteral reflux accompanying ICF syndrome. © 2016 Wiley Periodicals, Inc.

  4. Horner's Syndrome due to a Spontaneous Internal Carotid Artery Dissection after Deep Sea Scuba Diving

    PubMed Central

    Fernández Reyes, Jose Luis; Envid Lázaro, Blanca Mar; Fernández Letamendi, Teresa; Yeste Martín, Ryth; Jódar Morente, Francisco José

    2016-01-01

    Internal carotid artery dissection (ICAD) is a rare entity that either results from traumatic injury or can be spontaneously preceded or not by a minor trauma such as sporting activities. It represents a major cause of stroke in young patients. The diagnosis should be suspected with the combination of Horner's syndrome, headache or neck pain, and retinal or cerebral ischaemia. The confirmation is frequently made with a magnetic resonance angiography (MRA). Although anticoagulation with heparin followed by vitamin-K-antagonists is the most common treatment, there is no difference in efficacy of antiplatelet and anticoagulant drugs at preventing stroke and death in patients with symptomatic carotid dissection. We describe a patient with ICAD following deep sea scuba diving, who presented with Horner's syndrome and neck pain and was successfully treated with anticoagulants. PMID:27525139

  5. Cerebral salt wasting syndrome due to tuberculous meningitis; a case report.

    PubMed

    Ahmad, Syed; Majid, Zain; Mehdi, Mehwish; Mubarak, Muhammed

    2016-01-01

    A 58-year-old male presented with fever, nausea, and vomiting since 15 days along with irritability and confusion since 5 days. His laboratory reports showed low serum sodium, serum osmolality and uric acid. Computerized tomography (CT) scan of brain revealed age-related changes. While on lumbar puncture (LP) and cerebrospinal fluid (CSF) examination, CSF protein, lactate dehydrogenase (LDH) and total leukocyte count (predominant lymphocytes) were all increased. On his 14th day of admission, his serum sodium was 116 mEq/l and he had a high urine output. Fluid restriction was tried in order to rule out syndrome of inappropriate antidiuretic hormone secretion (SIADH) but the patient did not respond to it. Keeping in view the above findings, a final diagnosis of tuberculous meningitis leading to cerebral salt wasting syndrome was made. The patient was started on 3% hypertonic saline, mineralocorticoids and anti-tuberculous therapy (ATT), to which he responded favorably and was later discharged.

  6. Horner's Syndrome due to a Spontaneous Internal Carotid Artery Dissection after Deep Sea Scuba Diving.

    PubMed

    Alonso Formento, Jose Enrique; Fernández Reyes, Jose Luis; Envid Lázaro, Blanca Mar; Fernández Letamendi, Teresa; Yeste Martín, Ryth; Jódar Morente, Francisco José

    2016-01-01

    Internal carotid artery dissection (ICAD) is a rare entity that either results from traumatic injury or can be spontaneously preceded or not by a minor trauma such as sporting activities. It represents a major cause of stroke in young patients. The diagnosis should be suspected with the combination of Horner's syndrome, headache or neck pain, and retinal or cerebral ischaemia. The confirmation is frequently made with a magnetic resonance angiography (MRA). Although anticoagulation with heparin followed by vitamin-K-antagonists is the most common treatment, there is no difference in efficacy of antiplatelet and anticoagulant drugs at preventing stroke and death in patients with symptomatic carotid dissection. We describe a patient with ICAD following deep sea scuba diving, who presented with Horner's syndrome and neck pain and was successfully treated with anticoagulants.

  7. [Case report: respiratory infection due to Alcaligenes xylosoxidans in a patient with Mounier-Kuhn syndrome].

    PubMed

    Arroyo-Cózar, Marta; Ruiz-García, Montserrat; Merlos, Eva M; Vielba, David; Macías, Enrique

    2012-10-01

    Mounier-Kuhn syndrome is a rare entity characterized by abnormal dilatation of the trachea and main bronchi (tracheobronchomegaly). Alcaligenes xylosoxidans is a non fermenting gram-negative pathogen common in extra-and intra-hospital environment, which may be related to immunosuppression states. We describe the case of a 75 years old male, ex-smoker with moderate functional obstruction, chronic respiratory failure and chronic colonization by Pseudomonas aeuriginosa. He had an infectious exacerbation of his disease, reason that previously required several hospital admissions. The patient was treated with antibiotics and his evolution was favourable with negativization in cultures of the pathogen. This is the first description of the isolation of Alcaligenes xylosoxidans as a cause of respiratory infection in a patient with Mounier-Kuhn syndrome.

  8. Deafness due to bilateral endolymphatic sac tumours in a case of von Hippel-Lindau syndrome.

    PubMed Central

    Kempermann, G; Neumann, H P; Scheremet, R; Volk, B; Mann, W; Gilsbach, J; Laszig, R

    1996-01-01

    A case of bilateral endolymphatic sac tumours is reported. In a patient with von Hippel-Lindau syndrome, tumour growth in the right cerebellopontine angle caused deafness. The tumour was removed and classified as a metastasis from a thyroid carcinoma. However, on thyroidectomy no primary neoplasm could be found. Eight years later a similar tumour was operated on in the left petrosal bone. Histological appearance, immunocytochemical findings, and the clinical context gave evidence that the tumours had to be reclassified as endolymphatic sac tumours--extremely rare entities. The report supports the hypothesis, suggested by the few earlier case reports, that endolymphatic sac tumours could be one of the inherent tumour manifestations in von Hippel-Lindau syndrome. Images PMID:8795608

  9. Unusual Presentation of Gianotti-Crosti Syndrome due to Epstein-Barr Virus Infection

    PubMed Central

    Al Dhaheri, Hind Saif; Al Kaabi, Amani; Kara Hamo, Yasmin

    2016-01-01

    Gianotti-Crosti syndrome (GCS) is viral exanthema of childhood. It typically presents with a symmetric erythematous papular and papulovesicular eruption. It has been classically associated with hepatitis B virus, as well as rarely with Epstein-Barr virus (EBV). We report a case of GCS related to EBV infection without the classical systemic symptoms in a five-year-old male patient. PMID:28050291

  10. Steven's Johnson syndrome with toxic epidermal necrolysis due to thalidomide in a case of multiple myeloma.

    PubMed

    Das, Anupam; Sil, Amrita; Mishra, Vivek; Das, Nilay Kanti

    2014-01-01

    Thalidomide developed in 1954 for morning sickness had proven to be a teratogen and hence was withdrawn from market. Resurgence of thalidomide began as an immunomodulator when it was shown to be effective in the management of multiple myeloma and many conditions like erythema nodosum leprosum, graft versus host disease, recurrent aphthous ulcers etc. We report a case of Stevens Johnson syndrome-toxic epidermal necrolysis developing in an elderly male who was prescribed thalidomide after being diagnosed with multiple myeloma.

  11. Phenotypic Analysis of Individuals With Costello Syndrome due to HRAS p.G13C

    PubMed Central

    Gripp, Karen W.; Hopkins, Elizabeth; Sol-Church, Katia; Stabley, Deborah L.; Axelrad, Marni E.; Doyle, Daniel; Dobyns, William B.; Hudson, Cindy; Johnson, John; Tenconi, Romano; Graham, Gail E.; Sousa, Ana Berta; Heller, Raoul; Piccione, Maria; Corsello, Giovanni; Herman, Gail E.; Tartaglia, Marco; Lin, Angela E.

    2014-01-01

    Costello syndrome is characterized by severe failure-to-thrive, short stature, cardiac abnormalities (heart defects, tachyarrhythmia, and hypertrophic cardiomyopathy (HCM)), distinctive facial features, a predisposition to papillomata and malignant tumors, postnatal cerebellar overgrowth resulting in Chiari 1 malformation, and cognitive disabilities. De novo germline mutations in the proto-oncogene HRAS cause Costello syndrome. Most mutations affect the glycine residues in position 12 or 13, and more than 80% of patients share p.G12S. To test the hypothesis that subtle genotype–phenotype differences exist, we report the first cohortcomparison between 12 Costello syndrome individuals with p.G13C and individuals with p.G12S. The individuals with p.G13C had many typical findings including polyhydramnios, failure-to-thrive, HCM, macrocephaly with posterior fossa crowding, and developmental delay. Subjectively, their facial features were less coarse. Statistically significant differences included the absence of multifocal atrial tachycardia (P-value =0.033), ulnar deviation of the wrist (P <0.001) and papillomata (P =0.003), and fewer neurosurgical procedures (P =0.024). Fewer individuals with p.G13C had short stature (height below −2 SD) without use of growth hormone (P <0.001). The noteworthy absence of malignant tumors did not reach statistical significance. Novel ectodermal findings were noted in individuals with p.G13C, including loose anagen hair resulting in easily pluckable hair with a matted appearance, different from the tight curls typical for most Costello syndrome individuals. Unusually long eye lashes requiring trimming are a novel finding we termed dolichocilia. These distinctive ectodermal findings suggest a cell type specific effect of this particular mutation. Additional patients are needed to validate these findings. PMID:21438134

  12. Phenotypic analysis of individuals with Costello syndrome due to HRAS p.G13C.

    PubMed

    Gripp, Karen W; Hopkins, Elizabeth; Sol-Church, Katia; Stabley, Deborah L; Axelrad, Marni E; Doyle, Daniel; Dobyns, William B; Hudson, Cindy; Johnson, John; Tenconi, Romano; Graham, Gail E; Sousa, Ana Berta; Heller, Raoul; Piccione, Maria; Corsello, Giovanni; Herman, Gail E; Tartaglia, Marco; Lin, Angela E

    2011-04-01

    Costello syndrome is characterized by severe failure-to-thrive, short stature, cardiac abnormalities (heart defects, tachyarrhythmia, and hypertrophic cardiomyopathy (HCM)), distinctive facial features, a predisposition to papillomata and malignant tumors, postnatal cerebellar overgrowth resulting in Chiari 1 malformation, and cognitive disabilities. De novo germline mutations in the proto-oncogene HRAS cause Costello syndrome. Most mutations affect the glycine residues in position 12 or 13, and more than 80% of patients share p.G12S. To test the hypothesis that subtle genotype-phenotype differences exist, we report the first cohort comparison between 12 Costello syndrome individuals with p.G13C and individuals with p.G12S. The individuals with p.G13C had many typical findings including polyhydramnios, failure-to-thrive, HCM, macrocephaly with posterior fossa crowding, and developmental delay. Subjectively, their facial features were less coarse. Statistically significant differences included the absence of multifocal atrial tachycardia (P-value = 0.033), ulnar deviation of the wrist (P < 0.001) and papillomata (P = 0.003), and fewer neurosurgical procedures (P = 0.024). Fewer individuals with p.G13C had short stature (height below -2 SD) without use of growth hormone (P < 0.001). The noteworthy absence of malignant tumors did not reach statistical significance. Novel ectodermal findings were noted in individuals with p.G13C, including loose anagen hair resulting in easily pluckable hair with a matted appearance, different from the tight curls typical for most Costello syndrome individuals. Unusually long eye lashes requiring trimming are a novel finding we termed dolichocilia. These distinctive ectodermal findings suggest a cell type specific effect of this particular mutation. Additional patients are needed to validate these findings.

  13. Exogenous Cushing's syndrome due to topical corticosteroid application: case report and review literature.

    PubMed

    Tempark, Therdpong; Phatarakijnirund, Voraluk; Chatproedprai, Susheera; Watcharasindhu, Suttipong; Supornsilchai, Vichit; Wananukul, Siriwan

    2010-12-01

    Prolonged use of topical corticosteroids causes systemic adverse effects including Cushing's syndrome and hypothalamic-pituitary-adrenal (HPA) axis suppression, which is less common than that of the oral or parenteral route. At least 43 cases with iatrogenic Cushing syndrome from very potent topical steroid usage (Clobetasol) in children and adult have been published over the last 35 years particularly in developing countries. In children group (n = 22), most are infants with diaper dermatitis and two cases who had started topical application at a very early age and died from severe disseminated CMV infection. For the adult group (n = 21), the most common purpose of steroid use was for treatment of Psoriasis. The recovery period of HPA axis suppression was 3.49 ± 2.92 and 3.84 ± 2.51 months in children and adult, respectively. We report on an 8-month-old female infant who developed Cushing's syndrome and adrenal insufficiency after diaper dermatitis treatment through the misuse of Clobetasol without doctor's prescription. Physiologic dose of hydrocortisone was prescribed to prevent an adrenal crisis for 3 months and discontinued when HPA axis recovery was confirmed by normal morning cortisol and ACTH levels.

  14. Common Carotid Artery Stump Syndrome Due to Mobile Thrombus Detected by Carotid Duplex Ultrasonography.

    PubMed

    Omoto, Shusaku; Hasegawa, Yuki; Sakai, Kenichiro; Matsuno, Hiromasa; Arai, Ayumi; Terasawa, Yuka; Mitsumura, Hidetaka; Iguchi, Yasuyuki

    2016-10-01

    Carotid stump syndrome is a cause of recurrent embolic stroke following occlusion of the ipsilateral internal carotid artery. The present report describes a case of recurrent cerebral embolism ipsilateral to a chronically occluded left common carotid artery (CCA), i.e., "CCA stump syndrome." Doppler color flow imaging showed anterograde flow in the left internal and external carotid arteries, which were supplied by collateral flow from the superior thyroid artery inflowing just proximal to the left carotid bifurcation. According to carotid duplex ultrasonography (CDU), a low-echoic mobile thrombus was noted at the distal stump of the occluded CCA, which presumably caused distal embolism. The low-echoic mobile thrombus dramatically changed to a homogenously high-echoic thrombus, and there was no recurrence of stroke after antiplatelet and anticoagulant therapy. This is the first report to demonstrate a CDU-verified temporal change in the thrombus at the stump in CCA stump syndrome. CDU is a noninvasive and useful technique to characterize hemodynamics, thrombus morphology, and the response to therapy.

  15. DRESS syndrome due to antibiotic therapy of osteoarticular infections in children: two case reports.

    PubMed

    Ramírez, A; Abril, J C; Cano, J

    2015-01-01

    Osteoarticular infection in children frequently occurs before 10 years of age. Surgical drainage is sometimes required, whereas acute osteomyelitis can be treated with antibiotic therapy alone. The duration of antibiotic therapy varies, 2 weeks is sufficient for septic arthritis, whereas 6 weeks is often required for complicated cases. Some of these antibiotic drugs present direct complications with low clinical impact in certain individuals. Hypersensitivity to these drugs causes different reactions in children. DRESS syndrome (Drug Reaction with Eosinophilia and Systemic Symptoms) is a severe and potentially life-threatening drug reaction. It is characterised by high fever, malaise, lymphadenopathy and skin rash. From a clinical perspective, these symptoms can lead to an exacerbation of the initial infectious process for which treatment was commenced. The liver is the organ most often affected in DRESS syndrome associated with haematological changes, potentially similar to sepsis. We present two cases of children with osteoarticular infections who developed DRESS syndrome after antibiotic therapy. Both patients made a complete recovery after cessation of the antibiotic drugs used.

  16. Progressive anterior operculum syndrome due to FTLD-TDP: a clinico-pathological investigation.

    PubMed

    Otsuki, Mika; Nakagawa, Yoshitsugu; Mori, Fumiaki; Tobioka, Hirotoshi; Yoshida, Hideaki; Tatezawa, Yoshiharu; Tanigawa, Toshio; Takahashi, Ikuko; Yabe, Ichiro; Sasaki, Hidenao; Wakabayashi, Koichi

    2010-07-01

    Pathological investigation of progressive anterior operculum syndrome has rarely been reported. We describe clinico-pathological findings in a patient with progressive anterior operculum syndrome. A 74-year-old right-handed man had noticed speech and swallowing difficulties 1 year previously. Neurological examinations showed no abnormality other than a slight limitation of upward gaze and slow tongue movement without fibrillation. We investigated the patient using neuroimaging and neuropsychological examinations and observed him for 2 years until his death, at which point we obtained pathological findings. The patient's facial and masseteric muscles seemed hypotonic with drooling, but he could laugh and yawn normally, showing automatic voluntary dissociation. Palatal and pharyngeal reflexes were normal. Magnetic resonance imaging showed cortical atrophy in the temporal lobes bilaterally. (123)IMP single photon emission computed tomography and positron emission tomography showed decreased blood flow and activity in the frontotemporal lobes, predominantly on the left side. Neuropsychological examinations showed no aphasia, dementia or other neuropsychological abnormality. Intubation fiberscopy, laryngoscopy and video fluorography showed no abnormality. After 6 months his anarthria and dysphagia became aggravated. He died of aspiration pneumonia 2 years after onset. Postmortem examination revealed neuronal degeneration with TDP-43-positive inclusions in the frontal, temporal and insular cortices, consistent with frontotemporal lobar degeneration with TDP inclusions (FTLD-TDP). However, neuronal loss with gliosis was more prominent in the inferior part of the motor cortices, bilaterally. Progressive anterior operculum syndrome could be classified as a variant of FTLD-TDP.

  17. Comparison of facial features of DiGeorge syndrome (DGS) due to deletion 10p13-10pter with DGS due to 22q11 deletion

    SciTech Connect

    Goodship, J.; Lynch, S.; Brown, J.

    1994-09-01

    DiGeorge syndrome (DGS) is a congenital anomaly consisting of cardiac defects, aplasia or hypoplasia of the thymus and parathroid glands, and dysmorphic facial features. The majority of DGS cases have a submicroscopic deletion within chromosome 22q11. However there have been a number of reports of DGS in association with other chromosomal abnormalities including four cases with chromosome 10p deletions. We describe a further 10p deletion case and suggest that the facial features in children with DGS due to deletions of 10p are different from those associated with chromosome 22 deletions. The propositus was born at 39 weeks gestation to unrelated caucasian parents, birth weight 2580g (10th centile) and was noted to be dysmorphic and cyanosed shortly after birth. The main dysmorphic facial features were a broad nasal bridge with very short palpebral fissures. Echocardiography revealed a large subsortic VSD and overriding aorta. She had a low ionised calcium and low parathroid hormone level. T cell subsets and PHA response were normal. Abdominal ultrasound showed duplex kidneys and on further investigation she was found to have reflux and raised plasma creatinine. She had an anteriorly placed anus. Her karyotype was 46,XX,-10,+der(10)t(3;10)(p23;p13)mat. The dysmorphic facial features in this baby are strikingly similar to those noted by Bridgeman and Butler in child with DGS as the result of a 10p deletion and distinct from the face seen in children with DiGeorge syndrome resulting from interstitial chromosome 22 deletions.

  18. [Mononeuritis multiplex due to thrombotic ischemia of primary antiphospholipid antibody syndrome without vasculitis: an autopsy case report].

    PubMed

    Takahashi, Masatoshi; Katada, Fumiaki; Sato, Susumu; Shibayama, Hidehiro; Fukutake, Toshio; Murayama, Shigeo

    2015-01-01

    The patient was a 78-year-old man. Three years before admission, he developed transient peripheral neuropathy and purpura, and at admission, he presented with livedo reticularis of both his lower extremities and with mononeuritis multiplex. Vasculitis was not observed, and antiphospholipid antibodies were detected. The nerve and skin biopsies revealed no inflammation; axonal degeneration accompanied by thrombi was found in his arterioles and venules. Based on these findings, he was diagnosed with ischemic peripheral neuropathy due to primary antiphospholipid syndrome. Administration of anticoagulant therapy resulted in an improvement in symptoms; however, two months later, a relapse occurred, and the patient contracted an infection while undergoing immunosuppressive therapy. The infection became fulminant, and the patient succumbed to multiple organ failure. The autopsy revealed a systemic arterial and venous embolism; however, no vasculitis was observed. Antiphospholipid syndrome, which is responsive to antithrombotic treatment, should be considered as a differential diagnosis of mononeuritis multiplex.

  19. Status spasticus and psoas muscle edema due to anti-GAD antibody associated stiff-man syndrome.

    PubMed

    Maramattom, Boby Varkey

    2015-08-01

    Severe muscle rigidity and spasms are uncommon causes of Intensive Care Unit (ICU) admissions. Stiff-man syndrome (SMS) is a rare disorder characterized by continuous muscle spasms, axial muscle rigidity, "tin soldier gait," and continuous motor unit activity on electromyography. There are three clinical variants of SMS; stiff-limb syndrome, classical SMS, and paraneoplastic encephalomyelitis with rigidity and myoclonus. Three types of antibodies have been associated with SMS; however, anti-glutamic acid decarboxylase (GAD) antibodies are the most frequent and are seen in the idiopathic type of SMS. The spasms of SMS can be very disabling and severe enough to cause muscle ruptures and skeletal fractures. We present a case of anti-GAD positive SMS with "status spasticus" causing bilateral psoas myoedema and rhabdomyolysis due to repeated axial muscle jerking in a 64-year-old man and discuss the differential diagnosis of a "jerking patient in the ICU."

  20. Ectopic ACTH syndrome due to pheochromocytoma: case report and review of the literature.

    PubMed Central

    Forman, B. H.; Marban, E.; Kayne, R. D.; Passarelli, N. M.; Bobrow, S. N.; Livolsi, V. A.; Merino, M.; Minor, M.; Farber, L. R.

    1979-01-01

    A 51-year-old female was diagnosed preoperatively to have a pheochromocytoma producing ACTH. This diagnosis was based upon her paroxysmal hypertension, hyperpigmentation, and hypokalemia. Elevated levels of serum and urine corticosteroids, plasma ACTH, urinary VMA, and catecholamines fell after a right adrenal pheochromocytoma was removed. Subsequently this tumor was found to have a high content of ACTH. Review of the literature indicates a mortality rate of 57% for this syndrome. Proper preoperative recognition and management can result in total cure. Images FIG. 1 FIG. 2 FIG. 3 FIG. 4 FIG. 5 FIG. 6 PMID:222080

  1. Atypical presentation of autoimmune lymphoproliferative syndrome due to CASP10 mutation.

    PubMed

    Tripodi, Serena Ilaria; Mazza, Cinzia; Moratto, Daniele; Ramenghi, Ugo; Caorsi, Roberta; Gattorno, Marco; Badolato, Raffaele

    2016-09-01

    Herein we describe the case of a 8-years-old boy with diagnosis of atypical autoimmune lymphoproliferative syndrome (ALPS), carrying heterozygous mutation of CASP10 gene (I406L). He presented with multiple non-invasive infections of the skin, that were associated to chronic non-malignant non-infectious lymphadenopathy, failure to thrive, weakness, arthralgia, relapsing oral aftosis, and expansion of TCRαβ(+) CD4(-)/CD8(-) T cells. This observation suggests that cutaneous infections can be observed in ALPS patients carrying CASP10 mutations.

  2. Ectopic Cushing’s syndrome due to retroperitoneal ACTH-producing paragangliomas

    PubMed Central

    Chen, Fan; Wang, Xiangyu; Wang, Yang; Meng, Hui; Hou, Xinguo; Zhu, Yaofeng; Gao, Wei; Jiang, Xuewen; Chen, Shouzhen; Zhang, Zhaocun; Zou, Zhichuan; He, Tianyi; Yang, Yue; Zhu, Kejia; Wang, Yong; Liu, Yaxiao; Cui, Jianfeng; Shi, Benkang; Yin, Gang

    2016-01-01

    Extra-adrenal pheochromocytomas, or paragangliomas, are rare tumours that derive from extra-adrenal chromaffin cells. Cushing’s syndrome (CS) caused by paragangliomas is extremely rare. We report a 53-year-old man with hypertension, diabetes, and symptoms of hypokalemia. Computer tomography (CT) revealed two retroperitoneal masses and bilateral adrenal hyperplasia. Together with the laboratory examinations, ectopic CS caused by multiple paragangliomas was highly suspected. The patient underwent resections of retroperitoneal tumours, left kidney, and left adrenal; postoperative histopathology confirmed two paragangliomas that were both positively stained for adrenocorticotropic hormone (ACTH). He got clinical and biochemical recoveries without any recurrent evidence at the nine-month followup. PMID:27695589

  3. Adult-onset opsoclonus-myoclonus syndrome due to West Nile Virus treated with intravenous immunoglobulin.

    PubMed

    Hébert, Julien; Armstrong, David; Daneman, Nick; Jain, Jennifer Deborah; Perry, James

    2017-02-01

    A 63-year-old female with no significant past medical history was presented with a 5-day history of progressive opsoclonus-myoclonus, headaches, and fevers. Her workup was significant only for positive West-Nile Virus serum serologies. She received a 2-day course of intravenous immunoglobulin (IvIG). At an 8-week follow up, she had a complete neurological remission. Adult-onset opsoclonus-myoclonus syndrome is a rare condition for which paraneoplastic and infectious causes have been attributed. To our knowledge, this is the first case reported of opsoclonus-myoclonus secondary to West-Nile Virus treated with intravenous immunoglobulin monotherapy.

  4. Nicolau Syndrome due to Penicillin Injection: A Report of 3 Cases without Long-Term Complication

    PubMed Central

    Memarian, Sara; Gharib, Behdad; Gharagozlou, Mohammd; Alimadadi, Hosein; Ahmadinejad, Zahra

    2016-01-01

    Nicolau syndrome (NS) or livedo-like dermatitis is a rare complication of injection of various medications such as penicillin. The pathophysiology of this events is not clear, but some hypotheses are suggested, such as sympathetic nerve stimulation, embolic occlusion, inflammation, or mechanical injury. In this paper we report 3 cases of NS following benzathine penicillin. Clinical symptoms improved in 2 cases during 2-month follow-up, but one of them had a residual necrosis in the distal phalanges of the toes. PMID:27882254

  5. Thoracic Outlet Syndrome in a Volleyball Player Due to Nonunion of the First Rib Fracture.

    PubMed

    Puttmann, Kathleen T; Satiani, Bhagwan; Vaccaro, Patrick

    2016-11-01

    Fracture of the first rib with ensuing callus formation is a rare cause of thoracic outlet syndrome. We report a case of a 17-year-old female volleyball player who presented with months of chronic arm pain. Radiographic imaging demonstrated nonunion fracture of the first rib. Physical therapy had been unsuccessful in relieving the pain, and surgical management was performed with resection of the first rib through a transaxillary approach with complete resolution of symptoms. Inflammation surrounding such fractures may destroy tissue planes, making dissection more technically difficult.

  6. Patterns of Psychological Distress in Mothers of Preterm Infants

    PubMed Central

    Santos, Hudson; Levy, Janet; White-Traut, Rosemary; O’Shea, T. Michael; Geraldo, Victoria; David, Richard

    2015-01-01

    Mothers of preterm infants experience significant psychological distress, with elevated levels of inter-correlated depressive symptoms, stress, anxiety and post-traumatic stress symptoms. In a sample of racially and ethnically diverse mothers of preterm infants, we identified differing patterns of psychological distress during infant hospitalization and examined the effect of these psychological distress patterns on longitudinal trajectories of each psychological distress measure and on maternal perceptions of the child over the first year of the infant’s life. Mothers of preterm infants (N = 232) completed five questionnaires assessing depressive symptoms, anxiety, post-traumatic stress symptoms, stress due to infant appearance, and stress due to parental role alteration during enrollment in the neonatal hospitalization, discharge, and at 2, 6, and 12 months of age adjusted for prematurity. Latent class analysis on the enrollment psychological distress variables allowed us to identify five sub-groups of mothers exhibiting similar patterns of psychological distress, differing primarily in degree and type: low distress, moderate distress, high NICU-related distress, high depressive and anxiety symptoms, and extreme distress. These classes continued to show different longitudinal trajectories for the psychological distress measures through 12 months corrected age. Mothers in the extreme distress class and, to a lesser degree, mothers in the high depressive and anxiety symptom class remained at risk of significant psychological distress one year after discharge and had less positive perceptions of their child (greater worry and higher perceptions of child vulnerability). In conclusion, distinctive subgroups of mothers during hospitalization had different patterns of psychological distress throughout the 12-month period and may require different interventions in the NICU. PMID:26495909

  7. Neuropathic ocular pain due to dry eye is associated with multiple comorbid chronic pain syndromes

    PubMed Central

    Galor, Anat; Covington, Derek; Levitt, Alexandra E.; McManus, Katherine T.; Seiden, Benjamin; Felix, Elizabeth R.; Kalangara, Jerry; Feuer, William; Patin, Dennis J.; Martin, Eden R.; Sarantopoulos, Konstantinos D.; Levitt, Roy C.

    2015-01-01

    Recent data demonstrate that dry eye (DE) susceptibility and other chronic pain syndromes (CPS) such as chronic widespread pain, irritable bowel syndrome and pelvic pain, may share common heritable factors. Previously, we showed that DE patients describing more severe symptoms tended to report features of neuropathic ocular pain (NOP). We hypothesize that patients with a greater number of CPS would have a different DE phenotype compared to those with fewer CPS. We recruited a cohort of 154 DE patients from the Miami Veterans Affairs Hospital and defined high and low CPS groups by cluster analysis. In addition to worse non-ocular pain complaints and higher PTSD and depression scores (P<0.01), we found that the high CPS group reported more severe neuropathic-type DE symptoms compared to the low CPS group, including worse ocular pain assessed via 3 different pain scales (P<0.05), with similar objective corneal DE signs. This is the first study to demonstrate DE patients who manifest a greater number of comorbid CPS report more severe DE symptoms and features of NOP. These findings provide further evidence that NOP may represent a central pain disorder, and that shared mechanistic factors may underlie vulnerability to some forms of DE and other comorbid CPS. PMID:26606863

  8. Atypical Angelman syndrome due to a mosaic imprinting defect: Case reports and review of the literature.

    PubMed

    Le Fevre, Anna; Beygo, Jasmin; Silveira, Cheryl; Kamien, Benjamin; Clayton-Smith, Jill; Colley, Alison; Buiting, Karin; Dudding-Byth, Tracy

    2017-03-01

    Angelman syndrome (AS) is characterized by severe intellectual disability, limited, or absent speech and a generally happy demeanor. The four known etiological mechanisms; deletions, uniparental disomy, imprinting defects, and UBE3A mutation all affect expression of the UBE3A gene at 15q11-q13. An atypical phenotype is seen in individuals who are mosaic for a chromosome 15q11-q13 imprinting defect on the maternal allele. These patients present with a milder phenotype, often with hyperphagia and obesity or non-specific intellectual disability. Unlike typical AS syndrome, they can have a vocabulary up to 100 words and speak in sentences. Ataxia and seizures may not be present, and the majority of individuals do not have microcephaly. Here we review the current literature and present three individuals with atypical AS caused by a mosaic imprinting defect to demonstrate why DNA methylation analysis at the SNRPN locus needs to be considered in a broader clinical context. © 2017 Wiley Periodicals, Inc.

  9. Burning Mouth Syndrome due to Television Moans, an Enigma for Oral Physician: Treatment with Counseling.

    PubMed

    Gupta, Deepak; Sheikh, Soheyl; Pallagatti, Shambulingappa; Kasariya, Kartikaya; Buttan, Amit; Gupta, Maqul

    2014-01-01

    Burning mouth syndrome (BMS) is a relatively common disease that can severely affect the quality of life of the patient. It causes chronic orofacial pain or oral burning sensation even in the absence of any detectable organic cause. The etiology of BMS is complex and multifactorial. It has been associated with menopause, trigger events and even genetic polymorphisms. Although its etiology remains unclear, there is still much evidence that psychological elements like stress, anxiety or depression do play a significant role. There are several studies in the literature which only report the association of BMS with psychological factors. But to the best of our knowledge, there is no such case reported in the literature which has actually highlighted the management of such a case with psychogenic elements involved. In this case report, apart from discussing the role of psychological factors, the treatment of BMS with emphasis on counseling is also emphasized. Further, it is of interest to know that such patients with psychologically induced burning mouth syndrome have to be evaluated to their deepest details. Even their commonly overlooked gestures and habits like watching a particular television soap opera may be involved in their disease process. It can be concluded that psychological counseling in general dental practice can provide an effective cure for chronic oral burning sensation with psychological factors involved.

  10. [Case of drug-induced hypersensitivity syndrome due to lamotrigine: demonstration of sequential reactivation of herpesviruses].

    PubMed

    Sato, Tatsuharu; Kuniba, Hideo; Matsuo, Mitsuhiro; Matsuzaka, Tetsuo; Moriuchi, Hiroyuki

    2012-01-01

    Drug-induced hypersensitivity syndrome (DIHS) is a rare but severe multiorgan disorder. The reactivation of human herpesvirus-6 (HHV-6) and other human herpesviruses has been reported to be associated with its pathogenesis. We herein report a case of 14-year-old female who developed DIHS during the treatment with lamotrigine, a novel antiepileptic drug. She initially presented with fever, skin rash, cervical lymphadenopathy, leukocytosis with eosinophilia and atypical lymphocytosis, liver dysfunction and hypogammaglobulinemia. Discontinuation of the drug and administration of prednisolone led to improvement;however, tapering of prednisolone and administration of midazolam and ketamine thereafter triggered clinical deterioration. She subsequently developed hyperthyroidism followed by hypothyroidism. Herpesviral loads were determined in her peripheral blood by real-time PCR during the course of the treatment, and sequential reactivation of Epstein-Barr virus (EBV), HHV-6 and cytomegalovirus was demonstrated. EBV viremia was detected throughout the course, except for a short period when HHV-6 viremia was at the peak. HHV-6 viremia developed after the secondary deterioration. Cytomegalovirus viremia appeared transiently before the hyperthyroidic state reversed and became hypothyroidic. Although this syndrome should be regarded as a systemic reaction induced by a complex interplay among herpesviruses and the immune responses against viral infections and drugs, it remains unknown how such a sequential reactivation is related to the pathogenesis of the condition.

  11. A rare case of Cushing's syndrome due to bilateral adrenocortical adenomas.

    PubMed

    Yasuda, Atsushi; Seki, Toshiro; Ito, Kazuko; Takagi, Atsushi; Watanabe, Daisuke; Nakamura, Naoya; Hanai, Kazuya; Terachi, Toshiro; Maekawa, Takashi; Sasano, Hironobu; Fukagawa, Masafumi

    2014-12-20

    We report a rare case of Cushing's syndrome caused by bilateral cortisol-secreting adenomas in a 63-year-old man. Our preoperative diagnosis was based on endocrinological results and imaging findings. Laparoscopic adrenalectomy has become a standard technique for adrenal tumors; however, bilateral adrenalectomy results in postoperative adrenal insufficiency, necessitating lifelong steroid replacement. To preserve adrenal function, the left adrenal gland was completely resected, whereas the right adrenal gland was partially resected laparoscopically. Hydrocortisone supplementation was initiated at a dose of 30 mg/day and was slowly tapered. However, symptoms of adrenal insufficiency developed, and adrenal steroid secretion did not respond to exogenous adrenocorticotropic hormone. Bilateral cortisol-secreting tumors rarely cause Cushing's syndrome. The present study comprised few patients, and the utilized surgical procedures (i.e., total/partial adrenalectomy or bilateral total adrenalectomy) were not uniform. Few cases of bilateral adrenal-preserving surgery have been reported. However, our patient developed adrenal insufficiency after the oral cortisone supplementation was tapered. This report demonstrates that partial adrenalectomy does not necessarily preserve normal adrenocortical function. Therefore, careful postoperative observation is necessary for patients undergoing a partial adrenalectomy.

  12. Minimal Change Nephrotic Syndrome Sequentially Complicated by Acute Kidney Injury and Painful Skin Ulcers due to Calciphylaxis

    PubMed Central

    Sato, Ryuta; Akimoto, Tetsu; Imai, Toshimi; Nakagawa, Saki; Okada, Mari; Miki, Atsushi; Takeda, Shinichi; Yamamoto, Hisashi; Saito, Osamu; Muto, Shigeaki; Kusano, Eiji; Nagata, Daisuke

    2016-01-01

    Calciphylaxis is rare cutaneous manifestation associated with painful skin ulceration and necrosis. It primarily occurs in patients with end-stage chronic kidney disease. In this report, we would like to show our experience with a male patient presenting with minimal change nephrotic syndrome that was sequentially complicated by acute kidney injury and painful ulcerative cutaneous lesions due to calciphylaxis. There seemed to be several contributing factors, including a disturbance of the patient's mineral metabolism and the systemic use of glucocorticoids and warfarin. Various concerns regarding the diagnostic and therapeutic conundrums that were encountered in the present case are also discussed. PMID:27853075

  13. Respiratory syndrome very similar to extrinsic allergic alveolitis due to Penicillium verrucosum in workers in a cheese factory.

    PubMed

    Guglielminetti, M; Valoti, E; Cassini, P; Taino, G; Caretta, G

    2001-01-01

    A respiratory syndrome very similar to extrinsic allergic alveolitis due to Penicillium verrucosum was recognized in 4 workers employed in a Gorgonzola cheese factory. A mycogen allergy to P. verrucosum, used as starter in the production, was demonstrated by positive sputum culture and detection of specific antibodies in the blood. Intense and prolonged exposure to inhalation of fungal spores could have lead to the development of this allergic response. The fact that 2 of the subjects are siblings seems to indicate host susceptibility or immunological constitution in the pathogenesis of the respiratory allergy.

  14. [Case report: Löffler's syndrome due to Ascaris lumbricoides mimicking acute bacterial community--acquired pneumonia].

    PubMed

    Acar, Ali; Oncül, Oral; Cavuşlu, Saban; Okutan, Oğuzhan; Kartaloğlu, Zafer

    2009-01-01

    In this study we present a patient with Loeffler's syndrome caused by Ascaris lumbricoides who presented with the clinical findings of community-acquired pneumonia (CAP). Our patient, who was twenty-five years old, and who had had symptoms such as coughing, expectorating, dyspnea and fever for approximately ten days, was hospitalized. We auscultated polyphonic rhonchuses at the both hemithoraxes. A chest X-ray revealed bilateral lower zone patch consolidation. Acute bacterial community acquired pneumonia (CAP) was diagnosed due to these findings and empirical antibiotic treatment was begun. Repeated sputum Gram stains were negative, and both sputum and blood cultures were sterile. A sputum smear was negative for acid-fast bacilli. The patient's fever and respiratory complaint did not respond to the empirical antibiotics therapy. During the course of advanced investigations, we measured peripheric eosinophilia, and high levels of total Eo and total IgE, and observed Ascaris lumbricoides eggs during stool examination. The patient was given a diagnosis of Loeffler's syndrome. Thereupon the patient was treated successfully with one dose of albendazol 400 mg. In conclusion, we suggest that Loeffler's syndrome must be considered early in the differential diagnosis for CAP when peripheric eosinophilia is seen in patients if they live in an endemic area for parasitic disease.

  15. Transient total mesencephalic locked-in syndrome after bilateral ptosis due to basilar artery thrombosis.

    PubMed

    Fujimoto, Yasunori; Ohnishi, Yu-ichiro; Wakayama, Akatsuki; Yoshimine, Toshiki

    2012-11-01

    Locked-in syndrome (LIS) usually occurs as a result of pontine lesions and has been classified into various categories on the basis of neurologic conditions, of which transient total mesencephalic LIS is extremely rare. A 53-year-old man presented with bilateral ptosis followed by a total locked-in state. In the clinical course, the patient successfully recovered with only left slight hemiparesis and skew deviation remaining. Magnetic resonance imaging revealed multiple ischemic lesions caused by thrombosis at the top of basilar artery, including the bilateral cerebral peduncles, tegmentum of the midbrain, and the right cerebellar hemisphere. Antecedent bilateral ptosis before the locked-in state may be related to ischemia in the central caudal nucleus of the oculomotor nuclei. We should pay attention to this easily missed condition during the treatment of ischemic stroke involving the basilar artery.

  16. Red baby syndrome a new disease due to parvovirus B-19 observed in Kerala.

    PubMed

    Sasidharan, C K; Sugathan, P; Mampilly, Neena; Agarwal, Ramesh; Khare, Shashi; Lal, Shiv; Jayaram Paniker, C K

    2009-03-01

    Red Baby Syndrome is a new disease seen in infants and young children. Dramatic onset of clinical symptoms with high intensity, short duration and lack of similarity with other cutaneous lesions makes it distinct. Of 50 such patients studied over a period of 5 years, half were below one year of age. Abrupt onset of high fever and generalized erythema involving the entire skin, which is swollen and tender is characteristic. These children were highly irritable and had paradoxical cry when cuddled. Rapid resolution of symptoms occurred in 7-10 days with extensive desquamation. Routine investigations were normal, C-reactive protein was raised only in 10 patients. Human Parvo virus B-19 IgM antibodies were positive in 15 out of 24 patients. Real time polymerase chain reaction was positive for human parvovirus B 19 DNA in one. Histopathological changes in the skin biopsy showed post infectious vascular injury pattern.

  17. [Anterior spinal artery syndrome due to cervical spondylosis presenting as cervical angina].

    PubMed

    Odaka, Masaaki; Hirata, Koichi

    2004-11-01

    A 54-year-old woman developed acute progressive paraparesis after repeated precordial pain. Neurological examination revealed bilateral four-limb weakness predominant in the distal part of the upper limbs, upper limbs brisk tendon reflexes, superficial sensory impairment below the C8 level, and atonic bladder. T2-weighted cervical MRI disclosed hyperintense lesion with disc herniation in gray matter of spinal cord between C5 and C7. No vertebral artery abnormalities were detected. We hypothesized that she developed anterior spinal artery syndrome after cervical angina caused by cervical spondylosis. We conclude that physicians need to be aware of patients who experience chest pain without evidence of cardiac disease and that they take into consideration spinal cord infarction.

  18. Pudendal Nerve Entrapment Syndrome due to a Ganglion Cyst: A Case Report

    PubMed Central

    2016-01-01

    Pudendal nerve entrapment syndrome is an unusual cause of chronic pelvic pain. We experienced a case of pudendal neuralgia associated with a ganglion cyst. A 60-year-old male patient with a tingling sensation and burning pain in the right buttock and perineal area visited our outpatient rehabilitation center. Pelvis magnetic resonance imaging showed the presence of multiple ganglion cysts around the right ischial spine and sacrospinous ligament, and the pudendal nerve and vessel bundle were located between the ischial spine and ganglion cyst at the entrance of Alcock's canal. We aspirated the lesions under ultrasound guidance, and consequently his symptoms subsided during a 6-month follow-up. This is the first report of pudendal neuralgia caused by compression from a ganglion cyst around the sacrospinous ligament. PMID:27606282

  19. Opsoclonus myoclonus syndrome due to squamous cell carcinoma of the oesophagus.

    PubMed

    Rossor, Alexander M; Perry, Fiona; Botha, Abrie; Norwood, Fiona

    2014-03-03

    We present the case of a 47-year-old woman with encephalopathy, ataxia and oscillopsia diagnosed with opsoclonus myoclonus syndrome (OMS). In adults, OMS in the context of encephalopathy is commonly paraneoplastic. A CT of the chest, abdomen and pelvis and a gastroscopy were performed and were normal. A fluorodeoxyglucose positron emission tomography CT scan demonstrated increased uptake in the mid-oesophagus and an endoscopic ultrasound examination demonstrated intramural thickening of 5 mm with no epithelial lesion. The patient was treated with three courses of intravenous immunoglobulin (IvIg) with improvement in her symptoms. A year following the initial presentation a repeat endoscopic examination demonstrated an ulcerated oesophageal carcinoma with regional lymph node involvement. The patient was treated with neo adjuvant chemotherapy prior to oesophagectomy. The patient's symptoms resolved within 6 months of tumour resection without the need for further IvIg. Unfortunately, after a further 6 months the patient developed liver metastases and died.

  20. A repeated carpal tunnel syndrome due to tophaceous gout in flexor tendon

    PubMed Central

    Lu, Hui; Chen, Qiang; Shen, Hui

    2017-01-01

    Abstract Rationale: Gouty tophi is a rare cause of CTS. We first report a unique case of repeated CTS with gouty tophi in flexor tendon. In the previous literature, the symptoms cases of CTS were gradually increased. Patient concerns: We report a 44-year-old male porter presented with mass on his left distal forearm combined a repeated carpal tunnel syndrome for 5 years. He felt numbness in fingers and his left palmar. The CTS symptoms had been eased through rest and dugs medication. It recurred twice. Diagnoses: Monosodium urate crystal deposits were found in surgery. Histologic findings confirmed the diagnosis of gout. Interventions: We removed partial of gouty tophus and retained the integrity of the tendon. Outcomes: Two years after the surgery, the patient had not experienced any symptom recurrence. Lessons: Early diagnosis and control of gout are necessary to avoid irreversible complications. The surgery combined with decreasing trioxypurine treatment can improve the treatment outcome of gouty tophus. PMID:28248892

  1. [Association of Moebius syndrome and hypopituitarism due to a midline anomaly. A case report].

    PubMed

    Petrus, M; Rhabbour, M; Clouzeau, J; Bat, P; Bildstein, G; Ibanez, M H; Netter, J C

    1993-06-01

    A 14 year old patient had both Moebius anomalad with arthrogryposis and growth hormone deficiency due to a midline defect. This combination suggests an abnormality in the organization of the neural crest and homologous neural tube segment.

  2. A case of difficult airway due to lingual tonsillar hypertrophy in a patient with Down's syndrome.

    PubMed

    Nakazawa, Koichi; Ikeda, Daisuke; Ishikawa, Seiji; Makita, Koshi

    2003-09-01

    In this report, we describe airway management of symptomatic lingual tonsillar hypertrophy in a pediatric patient with Down's syndrome. Besides obstructive sleep apnea, the history included a small atrial septal defect with mild aortic regurgitation and Moyamoya disease. Anesthesia was induced with IV administration of 1 mg/kg of propofol, followed by inhalation of sevoflurane in 100% oxygen. Muscle relaxants were not used on induction. Rigid laryngoscopy could not visualize the epiglottis because of hypertrophied tonsillar tissue, and mask ventilation became difficult when spontaneous breathing stopped. We avoided using a laryngeal mask airway because of a slight bleeding tendency presumably caused by preoperative antiplatelet therapy. Fiberoptic bronchoscopy through the nasal cavity in combination with jet ventilation successfully identified the glottis and allowed nasotracheal intubation to be accomplished. After lingual tonsillectomy, the patient was extubated on the seventh postoperative day, after supraglottic edema had resolved. Fiberoptic nasotracheal intubation under inhaled anesthesia may therefore be preferable in pediatric or uncooperative patients with symptomatic lingual tonsillar hypertrophy.

  3. Stevens-Johnson Syndrome and toxic epidermal necrolysis overlap due to oral temozolomide and cranial radiotherapy.

    PubMed

    Sarma, Nilendu

    2009-01-01

    A 46-year-old man developed Stevens-Johnson syndrome and toxic epidermal necrolysis overlap, with severe localized denudation of the skin on the head and neck, following radiotherapy and oral temozolomide therapy for cranial glioblastoma multiforme. He also had a primary malignant fibrous histiocytoma of the thigh that was amputated 5 years earlier. A rash developed after 7 days of radio- and chemotherapy. It was an extensive maculopapular rash that started over the temporal area of the head and rapidly spread, sparing only the distal limbs. Radiotherapy and temozolomide were stopped on the tenth day but the rash rapidly progressed for the next 4-6 days. Following this, the spread halted and complete recovery was observed within the next 2 weeks. The peculiarity of the presentation in this case was that the brunt of the disease with severe skin denudation was localized to the surrounding areas of cranial radiotherapy. The patient was also receiving oral phenytoin, diclofenac, and parenteral dexamethasone before chemotherapy was started. These medications were continued, even after development of the skin rash, until well after full recovery from the skin lesions. After critical evaluation of disease onset, progression, and recovery, and their relationship to the introduction and withdrawal of different medicines, it appeared that either temozolomide alone or in combination with radiotherapy most probably triggered the condition.

  4. Nephrotic syndrome due to minimal change disease secondary to spider bite: clinico-pathological case of a non-described complication of latrodectism

    PubMed Central

    Enos, Daniel; Moreira, José Luis; Alvaredo, Fátima; Oddó, David

    2017-01-01

    Abstract The patient was an 18-year-old man who developed nephrotic syndrome after a ‘wheat spider’ bite (Latrodectus mactans). Due to this atypical manifestation of latrodectism, a renal biopsy was performed showing minimal change disease. The nephrotic syndrome subsided after 1 week without specific treatment. This self-limited evolution suggests that the mechanism of podocyte damage was temporary and potentially mediated by a secondary mechanism of hypersensitivity or direct effect of the α-latrotoxin. The patient did not show signs of relapse in subsequent checkup. This is the first reported case of nephrotic syndrome due to a minimal change lesion secondary to latrodectism.

  5. Nephrotic syndrome due to minimal change disease secondary to spider bite: clinico-pathological case of a non-described complication of latrodectism.

    PubMed

    Méndez, Gonzalo P; Enos, Daniel; Moreira, José Luis; Alvaredo, Fátima; Oddó, David

    2017-04-01

    The patient was an 18-year-old man who developed nephrotic syndrome after a 'wheat spider' bite (Latrodectus mactans). Due to this atypical manifestation of latrodectism, a renal biopsy was performed showing minimal change disease. The nephrotic syndrome subsided after 1 week without specific treatment. This self-limited evolution suggests that the mechanism of podocyte damage was temporary and potentially mediated by a secondary mechanism of hypersensitivity or direct effect of the α-latrotoxin. The patient did not show signs of relapse in subsequent checkup. This is the first reported case of nephrotic syndrome due to a minimal change lesion secondary to latrodectism.

  6. Streptococcus pneumoniae bacteraemia due to parotitis in a patient with systemic sclerosis and secondary Sjögren’s syndrome

    PubMed Central

    Tan, Jamie Bee Xian; Fong, Warren Weng Seng

    2016-01-01

    Introduction: Invasive pneumococcal disease is an uncommon and notifiable disease in Singapore. It is often associated with significant morbidity and mortality. We report a rare case of invasive pneumococcal bacteraemia due to parotitis in a patient with systemic sclerosis and secondary Sjögren’s syndrome. We also present a retrospective review of Streptococcus pneumoniae bacteraemia cases in Singapore General Hospital from January 2011 to April 2016. Case presentation: A 59-year-old Malay lady with a history of systemic sclerosis with secondary Sjögren’s syndrome presented with fever and left parotid gland swelling. Clinical examination revealed poor salivary pooling and left parotid swelling without fluctuance. Ultrasound of the left parotid gland confirmed acute parotitis without evidence of abscess or sialolithiasis. Blood cultures were positive for S. pneumoniae. She was diagnosed to have invasive pneumococcal bacteraemia secondary to acute parotitis, and treated with intravenous benzylpenicillin with clearance of bacteraemia after 3 days. Upon discharge, her antibiotics were changed to intravenous ceftriaxone to facilitate outpatient parenteral antibiotic therapy for another 2 weeks. She responded favourably to antibiotics at follow-up, with no complications from the bacteraemia. A review of the microbiological records of the Singapore General Hospital revealed 116 cases of pneumococcal bacteraemia, most (80.3 %) of which were due to pneumonia. None were due to parotitis. Conclusion: S. pneumoniae parotitis and subsequent bacteraemia is rare. Prompt recognition of the disease and appropriate use of antibiotics are important. This case highlights that close communication between healthcare workers (microbiologist, rheumatologist and infectious disease specialist) is essential in ensuring good clinical outcomes in patients with a potentially fatal disease. PMID:28348790

  7. Adolescent Polycystic Ovary Syndrome Due to Functional Ovarian Hyperandrogenism Persists Into Adulthood

    PubMed Central

    Ehrmann, David A.; Littlejohn, Elizabeth E.

    2015-01-01

    Background: Menstrual irregularity and above-average testosterone levels in adolescence may presage polycystic ovary syndrome (PCOS) in adulthood but persist in only a minority. Prolonged anovulatory cycles in normal adolescents are associated with increased testosterone levels. Thus, questions have been raised about the accuracy of PCOS diagnosed in adolescents. Objective: The purpose of this study was to follow-up hyperandrogenic adolescents with features of PCOS to test the hypothesis that adolescent functional ovarian hyperandrogenism (FOH) persists into adulthood. Study Subjects: A series of adults previously reported to have adolescent PCOS, with most documented to have FOH by GnRH agonist or dexamethasone androgen-suppression test criteria, were recalled. Methods: Recall occurred >3 years after the initial diagnosis and at the age of >18.0 years. Respondents underwent examination, baseline androgen evaluation, and an oral glucose tolerance test after discontinuing oral contraceptive therapy. Results: Of the adolescent hyperandrogenic patients, 68% (15 of 22) were traceable, and 60% of those traced returned for follow-up, including half (n = 8) of the original FOH group. The baseline characteristics of respondents and nonrespondents were not significantly different. Patients with FOH were reevaluated when their mean age was 23.0 years (range, 18.4–29.4 years), gynecologic age was 10.7 years (range, 5.5–18.4 years), and body mass index was 42.3 kg/m2 (range, 28.3–52.1 kg/m2; P = .02 vs adolescence). Serum free testosterone was 24 pg/mL (range, 10–38 pg/mL, normal, 3–9 pg/mL; not significant vs adolescence); all were oligomenorrheic. Whereas 3 of 8 had impaired glucose tolerance as adolescents, at follow-up 6 of 8 had developed abnormal glucose tolerance (2 with type 2 diabetes mellitus). Conclusions: Adolescents with FOH, which underlies most PCOS, uniformly have persistent hyperandrogenism, and glucose tolerance tends to deteriorate. Testing ovarian

  8. Two children with "dropped head" syndrome due to lamin A/C mutations.

    PubMed

    Chemla, Jeremy C; Kanter, Ronald J; Carboni, Michael P; Smith, Edward C

    2010-11-01

    LMNA-related congenital muscular dystrophy (L-CMD) is a recently described disorder characterized by infantile-onset myopathy due to mutations in the lamin A/C (LMNA) gene. We report the genetic and clinical characteristics of two unrelated L-CMD patients. Patient 1 harbored a novel, L35P mutation and patient 2 a previously reported R249W mutation. The striking phenotype associated with L-CMD is important to recognize, as molecular diagnostic testing can spare patients unnecessary procedures and prompt the physician to monitor for associated cardiac arrhythmias.

  9. Evidence Report: Risk of Acute Radiation Syndromes Due to Solar Particle Events

    NASA Technical Reports Server (NTRS)

    Carnell, Lisa; Blattnig, Steve; Hu, Shaowen; Huff, Janice; Kim, Myung-Hee; Norman, Ryan; Patel, Zarana; Simonsen, Lisa; Wu, Honglu

    2016-01-01

    Crew health and performance may be impacted by a major solar particle event (SPE), multiple SPEs, or the cumulative effect of galactic cosmic rays (GCR) and SPEs. Beyond low-Earth orbit, the protection of the Earth's magnetosphere is no longer available, such that increased shielding and protective mechanisms are necessary in order to prevent acute radiation sickness and impacts to mission success or crew survival. While operational monitoring and shielding are expected to minimize radiation exposures, there are EVA scenarios outside of low-Earth orbit where the risk of prodromal effects, including nausea, vomiting, anorexia, and fatigue, as well as skin injury and depletion of the blood-forming organs (BFO), may occur. There is a reasonable concern that a compromised immune system due to high skin doses from a SPE or due to synergistic space flight factors (e.g., microgravity) may lead to increased risk to the BFO. The primary data available at present are derived from analyses of medical patients and persons accidentally exposed to acute, high doses of low-linear energy transfer (LET) (or terrestrial) radiation. Data more specific to the space flight environment must be compiled to quantify the magnitude of increase of this risk and to develop appropriate protection strategies. In particular, information addressing the distinct differences between solar proton exposures and terrestrial exposure scenarios, including radiation quality, dose-rate effects, and non-uniform dose distributions, is required for accurate risk estimation.

  10. Bilateral symmetrical adrenal hypermetabolism on FDG PET/CT due to Cushing syndrome in well differentiated neuroendocrine carcinoma.

    PubMed

    Aktas, G E; Soyluoglu Demir, S; Sarikaya, A

    2016-01-01

    The (18)F-FDG PET/CT scan has been suggested for whole-body imaging to identify ectopic adrenocorticotrophic hormone secreting tumours, but there are some challenges involved. The case of a patient is presented, who was admitted with the pre-diagnosis of ectopic ACTH syndrome. On the CT, a nodular lesion was detected in the medial segment of the right lung. The FDG uptake of the lesion seemed to be increased visually, but was not pathological quantitatively (SUVmax: 1.8) on the PET/CT. There was also diffuse increased uptake (SUVmax: 14.2) in the enlarged adrenal glands. The lesion was reported as a possible malignant lesion with low FDG affinity, such as a low grade neuroendocrine tumour, while the diffuse enlarged adrenal glands with high uptake were interpreted as diffusely hyperplasic, due to Cushing's syndrome. The patient was treated with a surgical wedge resection. The histopathological diagnosis confirmed that the tumour was a grade 1 well-differentiated neuroendocrine carcinoma.

  11. Cardiac tamponade due to low-volume effusive constrictive pericarditis in a patient with uncontrolled type II autoimmune polyglandular syndrome.

    PubMed

    Palmer, William C; Kurklinsky, Andrew; Lane, Gary; Ussavarungsi, Kamonpun; Blackshear, Joseph L

    2014-03-01

    Type II autoimmune polyglandular syndrome (APS), a relatively common endocrine disorder, includes primary adrenal insufficiency coupled with type 1 diabetes mellitus and/or autoimmune primary hypothyroidism. Autoimmune serositis, an associated disease, may present as symptomatic pericardial effusion. We present a case of a 54-year old male with APS who developed pericarditis leading to cardiac tamponade with a subacute loculated effusion. After urgent pericardiocentesis intrapericardial pressure dropped to 0, while central venous pressures remain elevated, consistent with acute effusive constrictive pericarditis. Contrast computerized tomography confirmed increased pericardial contrast enhancement. The patient recovered after prolonged inotropic support and glucocorticoid administration. He re-accumulated the effusion 16 days later, requiring repeat pericardiocentesis. Effusive-constrictive pericarditis, an uncommon pericardial syndrome, is characterized by simultaneous pericardial inflammation and tamponade. Prior cases of APS associated with cardiac tamponade despite low volumes of effusion have been reported, albeit without good demonstration of hemodynamic findings. We report a case of APS with recurrent pericardial effusion due to pericarditis and marked hypotension with comprehensive clinical and hemodynamic assessment. These patients may require aggressive support with pericardiocentesis, inotropes, and hormone replacement therapy. They should be followed closely for recurrent tamponade.

  12. Tarsal Tunnel Syndrome Due To Three Different Types of Ganglion During a 12-Year Period: A Case Report.

    PubMed

    Kawakatsu, Motohisa; Ishiko, Toshihiro; Sumiya, Masafumi

    A 52-year-old male complained of numbness and radiating pain affecting the plantar region of his left foot. He was found to have recurrent tarsal tunnel syndrome due to posterior tibial nerve compression by 3 different types of ganglion during a 12-year period. To the best of our knowledge, a similar case has not been documented. At the first operation, flexor retinaculum release and simple excision of an epineural ganglion were performed without injuring the nerve fascicles; however, an intrafascicular ganglion developed approximately 2 years later. At the second operation, the ganglion cyst was resected completely to prevent recurrence, despite the risk of nerve fiber injury. The cyst originated from the subtalar joint; thus, the joint was closed, and a free fat graft was placed to prevent adhesion formation. However, an extraneural ganglion occurred about 3 years later. At the third operation, the cyst was resected completely, and a free periosteal graft was used to close the joint more effectively. No recurrence had developed at 6 years after the third operation. The findings of the present case show the need for long-term monitoring of patients with tarsal tunnel syndrome caused by a ganglion owing to the possibility of recurrence related to different ganglion types.

  13. Autosomal recessive Stickler syndrome due to a loss of function mutation in the COL9A3 gene.

    PubMed

    Faletra, Flavio; D'Adamo, Adamo P; Bruno, Irene; Athanasakis, Emmanouil; Biskup, Saskia; Esposito, Laura; Gasparini, Paolo

    2014-01-01

    Stickler syndrome (STL) is a clinically variable and genetically heterogeneous syndrome characterized by ophthalmic, articular, orofacial, and auditory manifestations. STL has been described with both autosomal dominant and recessive inheritance. The dominant form is caused by mutations of COL2A1 (STL 1, OMIM 108300), COL11A1 (STL 2, OMIM 604841), and COL11A2 (STL 3, OMIM 184840) genes, while recessive forms have been associated with mutations of COL9A1 (OMIM 120210) and COL9A2 (OMIM 120260) genes. Type IX collagen is a heterotrimeric molecule formed by three genetically distinct chains: α1, α2, and α3 encoded by the COL9A1, COL9A2, and COL9A3 genes. Up to this time, only heterozygous mutations of COL9A3 gene have been reported in human and related to: (1) multiple epiphyseal dysplasia type 3, (2) susceptibility to an intervertebral disc disease, and (3) hearing loss. Here, we describe the first autosomal recessive Stickler family due to loss of function mutations (c.1176_1198del, p.Gln393Cysfs*25) of COL9A3 gene. These findings extend further the role of collagen genes family in the disease pathogenesis.

  14. The Role of Inspiratory Muscle Training in Sickle Cell Anemia Related Pulmonary Damage due to Recurrent Acute Chest Syndrome Attacks

    PubMed Central

    Camcıoğlu, Burcu; Boşnak-Güçlü, Meral; Karadallı, Müşerrefe Nur; Akı, Şahika Zeynep; Türköz-Sucak, Gülsan

    2015-01-01

    Background. The sickling of red blood cells causes a constellation of musculoskeletal, cardiovascular, and pulmonary manifestations. A 32-year-old gentleman with sickle cell anemia (SCA) had been suffering from recurrent acute chest syndrome (ACS). Aim. To examine the effects of inspiratory muscle training (IMT) on pulmonary functions, respiratory and peripheral muscle strength, functional exercise capacity, and quality of life in this patient with SCA. Methods. Functional exercise capacity was evaluated using six-minute walk test, respiratory muscle strength using mouth pressure device, hand grip strength using hand-held dynamometer, pain using Visual Analogue Scale, fatigue using Fatigue Severity Scale, dyspnea using Modified Medical Research Council Scale, and health related quality of life using European Organization for Research and Treatment of Cancer QOL measurement. Results. A significant improvement has been demonstrated in respiratory muscle strength, functional exercise capacity, pain, fatigue, dyspnea, and quality of life. There was no admission to emergency department due to acute chest syndrome in the following 12 months after commencing regular erythrocytapheresis. Conclusion. This is the first report demonstrating the beneficial effects of inspiratory muscle training on functional exercise capacity, respiratory muscle strength, pain, fatigue, dyspnea, and quality of life in a patient with recurrent ACS. PMID:26060589

  15. Lipopolysaccharide-induced endotoxemia in corn oil-preloaded mice causes an extended course of lung injury and repair and pulmonary fibrosis: A translational mouse model of acute respiratory distress syndrome

    PubMed Central

    Wu, Chaomin; Evans, Colin E.; Dai, Zhiyu; Huang, Xiaojia; Zhang, Xianming; Jin, Hua; Hu, Guochang; Song, Yuanlin; Zhao, You-Yang

    2017-01-01

    Acute respiratory distress syndrome (ARDS) is characterized by acute hypoxemia respiratory failure, bilateral pulmonary infiltrates, and pulmonary edema of non-cardiac origin. Effective treatments for ARDS patients may arise from experimental studies with translational mouse models of this disease that aim to delineate the mechanisms underlying the disease pathogenesis. Mouse models of ARDS, however, can be limited by their rapid progression from injured to recovery state, which is in contrast to the course of ARDS in humans. Furthermore, current mouse models of ARDS do not recapitulate certain prominent aspects of the pathogenesis of ARDS in humans. In this study, we developed an improved endotoxemic mouse model of ARDS resembling many features of clinical ARDS including extended courses of injury and recovery as well as development of fibrosis following i.p. injection of lipopolysaccharide (LPS) to corn oil-preloaded mice. Compared with mice receiving LPS alone, those receiving corn oil and LPS exhibited extended course of lung injury and repair that occurred over a period of >2 weeks instead of 3–5days. Importantly, LPS challenge of corn oil-preloaded mice resulted in pulmonary fibrosis during the repair phase as often seen in ARDS patients. In summary, this simple novel mouse model of ARDS could represent a valuable experimental tool to elucidate mechanisms that regulate lung injury and repair in ARDS patients. PMID:28333981

  16. Design and implementation of the START (STem cells for ARDS Treatment) trial, a phase 1/2 trial of human mesenchymal stem/stromal cells for the treatment of moderate-severe acute respiratory distress syndrome

    PubMed Central

    2014-01-01

    Background Despite advances in supportive care, moderate-severe acute respiratory distress syndrome (ARDS) is associated with high mortality rates, and novel therapies to treat this condition are needed. Compelling pre-clinical data from mouse, rat, sheep and ex vivo perfused human lung models support the use of human mesenchymal stem (stromal) cells (MSCs) as a novel intravenous therapy for the early treatment of ARDS. Methods This article describes the study design and challenges encountered during the implementation and phase 1 component of the START (STem cells for ARDS Treatment) trial, a phase 1/2 trial of bone marrow-derived human MSCs for moderate-severe ARDS. A trial enrolling 69 subjects is planned (9 subjects in phase 1, 60 subjects in phase 2 treated with MSCs or placebo in a 2:1 ratio). Results This report describes study design features that are unique to a phase 1 trial in critically ill subjects and the specific challenges of implementation of a cell-based therapy trial in the ICU. Conclusions Experience gained during the design and implementation of the START study will be useful to investigators planning future phase 1 clinical trials based in the ICU, as well as trials of cell-based therapy for other acute illnesses. Trial registration Clinical Trials Registration: NCT01775774 and NCT02097641. PMID:25593740

  17. Effects of Oral Stimulus Frequency Spectra on the Development of Non-nutritive Suck in Preterm Infants with Respiratory Distress Syndrome or Chronic Lung Disease, and Preterm Infants of Diabetic Mothers

    PubMed Central

    Barlow, SM; Lee, Jaehoon; Wang, Jingyan; Oder, Austin; Oh, Hyuntaek; Hall, Sue; Knox, Kendi; Weatherstone, Kathleen; Thompson, Diane

    2013-01-01

    The precocial nature of orofacial sensorimotor control underscores the biological importance of establishing ororythmic activity in human infants. The purpose of this study was to assess the effects of comparable doses of three forms of orosensory experience, including a low-velocity spectrally reduced orocutaneous stimulus (NT1), a high-velocity broad spectrum orocutaneous stimulus (NT2), and a SHAM stimulus consisting of a blind pacifier. Each orosensory experience condition was paired with gavage feedings 3x/day for 10 days in the neonatal intensive care unit (NICU). Four groups of preterm infants (N=214), including those with respiratory distress syndrome (RDS), chronic lung disease (CLD), infants of diabetic mothers (IDM), and healthy controls (HI) were randomized to the type of orosensory condition. Mixed modeling, adjusted for gender, gestational age, postmenstrual age, and birth weight, demonstrated the most significant gains in non-nutritive suck (NNS) development among CLD infants who were treated with the NT2 stimulus, with smaller gains realized among RDS and IDM infants. The broader spectrum of the NT2 stimulus maps closely to known response properties of mechanoreceptors in lip, tongue, and oral mucosa and is more effective in promoting NNS development among preterm infants with impaired oromotor function compared to the low-velocity, spectrally reduced NT1 orosensory stimulus. PMID:25018662

  18. Cultural and Social Predictors of Psychological Distress in Mexican Americans.

    ERIC Educational Resources Information Center

    Golding, Jacqueline M.; Burnam, M. Audrey

    Studies of relative levels of psychological distress among Mexican Americans and non-Hispanic Whites have found mixed results, possibly due to cultural differences within Mexican American samples which may confound potential ethnic differences. The hypothesis that differences in psychological distress between Mexican Americans and non-Hispanic…

  19. Psychological distress and patient satisfaction.

    PubMed

    Greenley, J R; Young, T B; Schoenherr, R A

    1982-04-01

    Psychologically distressed patients and clients of health care and social service organizations are found to report somewhat more dissatisfaction with services than do the nondistressed. Four explanations for this relationship are examined: 1) the psychologically distressed are generally dissatisfied; 2) service providers react negatively to the psychologically distressed; 3) psychologically distressed patients are dissatisfied when service providers do not respond to their psychological needs; and 4) patients who deny their psychological distress tend to be dissatisfied. The results show that the psychologically distressed report more dissatisfaction because of the very high levels of dissatisfaction found among patients who deny having personal problems.

  20. Loneliness and psychological distress.

    PubMed

    Jackson, J; Cochran, S D

    1991-05-01

    Research on relationships between loneliness and psychological symptoms has generally shown significant positive associations across a wide spectrum of psychopathologies. However, such results may be artificial, to some extent, given the high intercorrelations of typical psychopathology measures. In the current study, we examined associations between psychological symptoms, assessed by the Symptom Check List-90 (SCL-90; Derogatis, Lipman, & Covi, 1973) and loneliness, as measured by the UCLA-R Loneliness Scale (Russell, Peplau, & Cutrona, 1980), in college students. Using partial correlations to control for the confounding influence of generalized distress, relationships between loneliness and individual dimensions of distress were examined. Results indicate a significant association between loneliness and interpersonal sensitivity (low self-esteem) and depression. Other dimensions of distress were not significantly related to loneliness. In addition, no sex differences in patterns of association were observed. Results support the notion that self-blame and self-devaluation are strong correlates of loneliness.

  1. Hepatoencephalopathy syndrome due to Cassia occidentalis (Leguminosae, Caesalpinioideae) seed ingestion in horses.

    PubMed

    Oliveira-Filho, J P; Cagnini, D Q; Badial, P R; Pessoa, M A; Del Piero, F; Borges, A S

    2013-03-01

    Cassia occidentalis is a bush from the Leguminosae family, subfamily Caesalpinoideae, and is a toxic plant of veterinary interest due to the occasional contamination of animal rations. This report describes the clinical and histopathological findings of an outbreak of C. occidentalis poisoning in horses. Twenty mares were poisoned after consuming ground corn contaminated with 8% of C. occidentalis seeds. Of the 20 animals affected, 12 died: 8 mares were found dead, 2 died 6 h after the onset of clinical signs compatible with hepatic encephalopathy and the 2 other animals were subjected to euthanasia 12 h after the onset of the clinical signs. The remaining 8 mares presented with mild depression and decreased appetite, but improved with treatment and no clinical sequelae were observed. In 6 animals that underwent a necropsy, an enhanced hepatic lobular pattern was noted and within the large intestine, a large number of seeds were consistently observed. Hepatocellular pericentrolobular necrosis and cerebral oedema were the main histological findings. In one mare, there was mild multifocal semimembranosus rhabdomyocytic necrosis and haemorrhage. Seeds collected from intestinal contents and sifted from the culpable feedstuff were planted. Examination of the leaves, flowers, fruits and seeds of the resultant plants identified C. occidentalis. Horses poisoned by C. occidentalis seeds demonstrate clinical signs associated with hepatoencephalopathy and frequently die suddenly. Lesions primarily involve the liver and secondarily, the central nervous system. Cassia occidentalis poisoning should be considered a differential diagnosis in horses with hepatoencephalopathy and special caution should be taken with horse rations to avoid contamination with seeds of this toxic plant.

  2. Autosomal dominant {open_quotes}Opitz{close_quotes} GBBB syndrome due to a 22q11.2 deletion

    SciTech Connect

    McDonald-McGinn, D.M.; Emanuel, B.S.; Zackai, E.H.

    1996-08-23

    The classification of Opitz GBBB syndrome has been associated with the deletion of the DiGeorge chromosome region on human chromosome 22q11.2. The broad phenotype involved in this deletion syndrome has been referred to as the DiGeorge/velocardiofacial syndrome. The clinical description of the patient will influence the diagnosis of the syndrome. More cooperation between the clinicians and the molecular researchers is necessary in order to locate the gene(s) for these disorders. 11 refs.

  3. Is high-frequency oscillatory ventilation more effective and safer than conventional protective ventilation in adult acute respiratory distress syndrome patients? A meta-analysis of randomized controlled trials

    PubMed Central

    2014-01-01

    Introduction Comprehensively evaluating the efficacy and safety of high-frequency oscillatory ventilation (HFOV) is important to allow clinicians who are using or considering this intervention to make appropriate decisions. Methods To find randomized controlled trials (RCTs) comparing HFOV with conventional mechanical ventilation (CMV) as an initial treatment for adult ARDS patients, we searched electronic databases (including PubMed, MedLine, Springer Link, Elsevier Science Direct, ISI web of knowledge, and EMBASE) with the following terms: “acute respiratory distress syndrome”, “acute lung injury”, and “high frequency oscillation ventilation”. Additional sources included reference lists from the identified primary studies and relevant meta-analyses. Two investigators independently screened articles and extracted data. Meta-analysis was conducted using random-effects models. Results We included 6 RCTs with a total of 1,608 patients in this meta-analysis. Compared with CMV, HFOV did not significantly reduce the mortality at 30 or 28 days. The pooled relative risk (RR) was 1.051 (95% confidence interval (CI) 0.813 to 1.358). ICU mortality was also not significantly reduced in HFOV group, with a pooled RR of 1.218 (95% CI 0.925 to 1.604). The pooled effect sizes of HFOV for oxygenation failure, ventilation failure and duration of mechanical ventilation were 0.557 (95% CI 0.351 to 0.884), 0.892 (95% CI 0.435 to 1.829) and 0.079 (95% CI −0.045 to 0.203), respectively. The risk of barotrauma and hypotension were similar between the CMV group and HFOV group, with a RR of 1.205 (95% CI 0.834 to 1.742) and a RR of 1.326 (95% CI 0.271 to 6.476), respectively. Conclusions Although HFOV seems not to increase the risk of barotrauma or hypotension, and reduces the risk of oxygenation failure, it does not improve survival in adult acute respiratory distress syndrome patients. PMID:24887179

  4. Fluctuating Kluver-Bucy syndrome in a child with epilepsy due to bilateral anterior temporal congenital malformations.

    PubMed

    Pestana, Elia M; Gupta, Ajay

    2007-03-01

    An 11-year-old boy with epilepsy due to congenital bilateral anterior temporal lobe malformations presented with fluctuating Kluver-Bucy syndrome (KBS). Since the age of 2, he had experienced clusters of three or four daily complex partial seizures over 2-3 days in a month, followed by a seizure-free interval of 3-4 weeks. During the seizure-free period, the patient exhibited hyperorality, sniffing, irritability alternating with placidity, anxiety, unsolicited sexual gestures, and unusual calmness after eating. KBS features escalated up to the onset of the seizure cluster, and remitted after the seizures. Brain MRI revealed bilateral anterior temporal cortical dysplasia with enlarged and dysmorphic amygdalar-hippocampal complex. Brain [(18)F]fluorodeoxyglucose positron emission tomography PET showed bilateral anterior and mesial temporal hypometabolism. Video/EEG monitoring revealed independent right and left temporal lobe seizures. This is the first reported case of KBS due to congenital bitemporal malformations. Also, KBS behavior phenotype in this patient fluctuated, with escalation during the seizure-free period and remission induced by the monthly seizure cluster. This fluctuating pattern could represent forced normalization.

  5. Tester for Distress Beacons

    NASA Technical Reports Server (NTRS)

    Wade, W. R.

    1986-01-01

    Distress beacons on aircraft and boats checked for proper operation with aid of onboard monitor. Monitor mounted in aircraft cockpit or at wheel of boat. Connected to beacon electronics by cable. Monitor used with interface circuitry in beacon, which acts as buffer so that operation of beacon is not adversely affected if monitor is removed or if connecting cable is accidentally short circuited.

  6. Fulminant neurological deterioration in a neonate with Leigh syndrome due to a maternally transmitted missense mutation in the mitochondrial ND3 gene

    SciTech Connect

    Leshinsky-Silver, E.; E-mail: leshinsky@wolfson.health.gov.il; Lev, D.; Tzofi-Berman, Z.; Cohen, S.; Saada, A.; Yanoov-Sharav, M.; Gilad, E.; Lerman-Sagie, T.

    2005-08-26

    Leigh syndrome can result from both nuclear and mitochondrial DNA defects. Mutations in complex V genes of the respiratory chain were considered until recently as the most frequent cause for mitochondrial inherited Leigh syndrome, while gene defects in complex I were related to recessive Leigh syndrome. Recently few reports of mutations in the mitochondrial-encoded complex I subunit genes causing Leigh syndrome have been reported. We describe a 1-month-old baby who acutely deteriorated, with abrupt onset of brainstem dysfunction, due to basal ganglia lesions extending to the brainstem. A muscle biopsy demonstrated complex I deficiency. Subsequent analysis of the mitochondrial genome revealed a homoplastic T10191C mutation in the ND3 gene (in blood and muscle), resulting in a substitution of serine to proline. Hair root analysis revealed a 50% mutant load, reflecting heteroplasmy in early embryonic stages. The mutation was also detected in his mother (5%). Western blot analysis revealed a decrease of the 20 kDa subunit (likely ND6) and of the 30 kDa subunit (NDUFA9), which is probably due to instability attributed to the inability to form subcomplexes with ND3. This is the first description of infantile Leigh syndrome due to a maternally transmitted T10191C substitution in ND3 and not due to a de novo mutation. This mutation is age and tissue dependent and therefore may not be amenable to prenatal testing.

  7. Guyon's canal syndrome due to tortuous ulnar artery with DeQuervain stenosing tenosynovitis, ligamentous injuries and dorsal intercalated segmental instability syndrome, a rare presentation: a case report

    PubMed Central

    2009-01-01

    The Guyon's canal syndrome is a well known clinical entity and may have significant impact on patient's quality of life. We report a case of 43-year-old male who presented with complaints of pain and numbness in right hand and difficulty in writing for past one month. On imaging diagnosis of Guyon's canal syndrome because of tortuous ulnar artery was made with additional findings of DeQuervain's stenosing tenosynovitis and dorsal intercalated segmental instability syndrome with ligamentous injury and subsequently these were confirmed on surgery. Although it is a rare syndrome, early diagnosis and treatment prevents permanent neurological deficits and improve patient's quality of life. PMID:20076781

  8. Association between Interleukin-10-1082 G/A and Tumor Necrosis Factor-α 308 G/A Gene Polymorphisms and Respiratory Distress Syndrome in Iranian Preterm Infants

    PubMed Central

    Omidvari, Peyman; Moradi, Fahimeh; Hamidi, Majid; Teimori, Hossein

    2017-01-01

    Cytokine polymorphisms may contribute to the prevalence of respiratory distress syndrome. The present study was done to investigate the frequency of interleukin- (IL-) 10 and tumor necrosis factor- (TNF-) α gene polymorphisms and their association with the risk of RDS in preterm infants. One-hundred and nineteen patients with RDS and 119 healthy preterm infants were enrolled. PCR restriction fragment length polymorphism was used to determine the frequency of IL-10 and TNF-α genotypes at -1082 A and -308 A, respectively. One-hundred and nineteen out of 238 infants had RDS (50%). The age of the mothers and gestational age ranged 17–45 (mean: 28.6 ± 5.3) years and 24–34 (mean: 34.3 ± 2.38) weeks, respectively. Totally, 23 deaths were recorded in the RDS group. Incidence of TNF-α-308 A/A and TNF-α-308 G/A was 84% and 16%, respectively. TNF-a-308 G/G was not found in both groups. Prevalence of IL-10-1082 G/G and IL-10-1082 G/A variants was 65.5% and 34.5%, respectively. IL-10-1082 A/A was not found in both groups. The incidence of the allele G in the IL-10-1082 polymorphism was lower in RDS group (P < 0.05). We found that the risk of RDS was correlated to sex, gestational age, and IL-10-1082. PMID:28298812

  9. 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.

    PubMed

    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

    1990-02-01

    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.(ABSTRACT TRUNCATED AT 250 WORDS)

  10. Superior vena cava syndrome due to a leiomyosarcoma of the anterior mediastinum: A case report and literature overview

    PubMed Central

    Labarca, E.; Zapico, A.; Ríos, B.; Martinez, F.; Santamarina, M.

    2014-01-01

    INTRODUCTION Leiomyosarcomas are an infrequent cause of malignant superior vena cava syndrome (VCS). PRESENTATION OF CASE A 51-year old male patient was admitted for a three-day history of dyspnoea, dysphagia and erythema of the head and neck. Computed tomography and magnetic resonance imaging showed a lesion arising on the anterior mediastinum, which was in close proximity with a thrombus in the superior vena cava. Surgical excision was performed, including open resection of the primary tumour and an atrio-innominate vein bypass with 8-mm polytetrafluoroethylene (PTFE). Histology confirmed a leiomyosarcoma and postoperative radiotherapy sessions were performed. Due to evidence of enlargement of the thrombus, a second intervention was undertaken. In this procedure, a remainder of the primary tumour was resected and the superior vena cava reconstructed with an autologous pericardium patch. The patient recovered satisfactorily and was discharged on the seventh postoperative day, with no evidence for relapse after 10 months of follow-up. DISCUSSION Leiomyosarcomas comprise less than 2% of the tumours of the mediastinum and are a rare cause of paraneoplastic VCS. Male patients in their sixties are most commonly affected. Relapses seem to be common, and thus a careful follow-up is often recommended. CONCLUSION In spite of the limited data on the management of thoracic leiomyosarcomas, surgery is currently considered the mainstay of treatment. PMID:25460453

  11. Prader-Willi syndrome with an unusually large 15q deletion due to an unbalanced translocation t(4;15).

    PubMed

    Varela, Monica C; Lopes, Graziela M P; Koiffmann, Celia P

    2004-01-01

    Prader-Willi syndrome (PWS) is a neurobehavioral disorder caused by deletions in the 15q11-q13 region, by maternal uniparental disomy of chromosome 15 or by imprinting defects. Structural rearrangements of chromosome 15 have been described in about 5% of the patients with typical or atypical PWS phenotype. An 8-year-old boy with a clinical diagnosis of PWS, severe neurodevelopmental delay, absence of speech and mental retardation was studied by cytogenetic and molecular techniques, and an unbalanced de novo karyotype 45,XY,der(4)t(4;15)(q35;q14),-15 was detected after GTG-banding. The patient was diagnosed by SNURF-SNRPN exon 1 methylation assay, and the extent of the deletions on chromosomes 4 and 15 was investigated by microsatellite analysis of markers located in 4qter and 15q13-q14 regions. The deletion of chromosome 4q was distal to D4S1652, and that of chromosome 15 was located between D15S1043 and D15S1010. Our patient's severely affected phenotype could be due to the extent of the deletion, larger than usually seen in PWS patients, although the unbalance of the derivative chromosome 4 cannot be ruled out as another possible cause. The breakpoint was located in the subtelomeric region, very close to the telomere, a region that has been described as having the lowest gene concentrations in the human genome.

  12. Sevelamer is an Effective Drug in Treating Hyperphosphatemia Due to Tumor Lysis Syndrome in Children: A Developing World Experience.

    PubMed

    Kahlon, Dilraj Kaur; Dinand, Veronique; Yadav, Satya Prakash; Sachdeva, Anupam

    2016-03-01

    We report here a study on efficacy of sevelamer hydrochloride in treating hyperphosphatemia due to tumor lysis syndrome (TLS) in a developing world setting. Twenty one children with hyperphosphatemia due to TLS were included. All received hyper-hydration, allopurinol and sevelamer. Efficacy was assessed by decrease in serum phosphate level, calcium-phosphate product and TLS score as per Cairo Bishop definition. Four children who underwent dialysis were excluded from analysis. Among the remaining 17 patients with hyperphosphatemia, laboratory TLS was recorded in 15 patients and clinical TLS in five. Sevelamer was given according to weight, most often 400 mg twice to thrice daily. Mean phosphatemia decreased from 8.3 ± 3.0 to 6.7 ± 2.1 mg/dl within 24 h of starting sevelamer (p = 0.02), 6.0 ± 2.1 mg/dl at 48 h, 4.9 ± 1.5 mg/dl at 72 h and 4.39 ± 1.7 mg/dl at 96 h. TLS was corrected in 72 h in 14 patients, 96 h in 1 and 120 h in another patient. Mean calcium-phosphate product decreased from 63.0 ± 14.0 to 49.2 ± 9.7 mg/dl (p = 0.002) at 24 h, 46.1 ± 17.0 mg/dl at 48 h and 39.7 ± 13.5 mg/dl at 72 h. There was no mortality due to hyperphosphatemia. Sevelamer is efficacious in children with malignancy-associated hyperphosphatemia in the developing world.

  13. Moral Distress among Iranian Nurses

    PubMed Central

    Merghati-Khoei, Effat; Tabatabaei, Shahnaz

    2015-01-01

    Objective: The purpose of this study was to describe the moral distress among Iranian registered nurses. Methods: This was a descriptive –analytic study, in which 264 out of 1000 nurses were randomly selected as a sample group and completed the questionnaire. The nurses’ moral distress was assessed using Corley’s 30-item Moral Distress Scale adapted for use in an Iranian population. The collected data were analyzed by SPSS version 19. Results: In this study, no correlation was found between the level of moral distress and any of the demographic data. The mean moral distress score ranged from 3.56 to 5.83, indicating moderate to high levels of moral distress. The item with the highest mean score was “working with unsafe levels of nurse staffing”. The item with the lowest mean score was “giving medication intravenously to a patient who has refused to take it”. Nurses working in EMS and NICU units had the highest levels of moral distress. Conclusion: A higher degree of moral distress is observed among nurses who work in health care systems. The results of this study highly recommend practical and research-oriented evaluation of moral distress in the medical society in Iran. Our findings suggest that Iranian version of MDS is a reliable instrument to measure moral distress in nurses. PMID:26005478

  14. Ethical Aspects of Participating in Psychology Experiments: Effects of Anonymity on Evaluation, and Complaints of Distressed Subjects.

    ERIC Educational Resources Information Center

    Britton, Bruce K.; And Others

    1983-01-01

    Distressed research participants were identified and contacted to investigate their experiences. Anxiety due to physical discomfort was the primary complaint. However, the experiments had very mild distress effects on the subjects. Anonymity did not influence responses. (CS)

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

    SciTech Connect

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

    2015-10-15

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

  16. Delayed diagnosis in a house of correction: Smith-Magenis syndrome due to a de novo nonsense RAI1 variant.

    PubMed

    Yeetong, Patra; Vilboux, Thierry; Ciccone, Carla; Boulier, Kristin; Schnur, Rhonda E; Gahl, William A; Huizing, Marjan; Laje, Gonzalo; Smith, Ann C M

    2016-09-01

    We report a 25-year-old female confirmed to have Smith-Magenis syndrome (SMS) due to a de novo RAI1 variant. Her past history is significant for developmental and intellectual delay, early and escalating maladaptive behaviors, and features consistent with significant sleep disturbance, the etiology of which was not confirmed for over two decades. The diagnosis of SMS was initially suspected in 1998 (at age 12 years), but that was 5 years before the initial report of RAI1 variants as causative of the SMS phenotype; cytogenetic fluorescence in situ hybridization studies failed to confirm an interstitial deletion of 17p11.2. Re-evaluation for suspected SMS was pursued with RAI1 sequencing analysis in response to urgent parental concerns of escalating behaviors and aggression with subsequent incarceration of the subject for assault of a health professional. Genetic analysis revealed a de novo RAI1 (NM_030665.3) nonsense variant, c.5536C>T; p.Q1846X. This case illustrates the importance of confirming the SMS diagnosis, which is associated with cognitive and functional impairment, as well as significant psychiatric co-morbidities and behavioral problems. The diagnosis was particularly relevant to the legal discussion and determination of her competence to stand trial. As other similar cases may exist, this report will help to increase awareness of the possibility of a very late diagnosis of SMS, with the need for re-evaluation of individuals suspected to have SMS who were initially evaluated prior to the identification of the RAI1 gene. © 2016 Wiley Periodicals, Inc.

  17. Beneficial effect of pyruvate therapy on Leigh syndrome due to a novel mutation in PDH E1α gene.

    PubMed

    Koga, Yasutoshi; Povalko, Nataliya; Katayama, Koujyu; Kakimoto, Noriko; Matsuishi, Toyojiro; Naito, Etsuo; Tanaka, Masashi

    2012-02-01

    Leigh syndrome (LS) is a progressive untreatable degenerating mitochondrial disorder caused by either mitochondrial or nuclear DNA mutations. A patient was a second child of unconsanguineous parents. On the third day of birth, he was transferred to neonatal intensive care units because of severe lactic acidosis. Since he was showing continuous lactic acidosis, the oral supplementation of dichloroacetate (DCA) was introduced on 31st day of birth at initial dose of 50 mg/kg, followed by maintenance dose of 25 mg/kg/every 12 h. The patient was diagnosed with LS due to a point mutation of an A-C at nucleotide 599 in exon 6 in the pyruvate dehydrogenase E1α gene, resulting in the substitution of aspartate for threonine at position 200 (N200T). Although the concentrations of lactate and pyruvate in blood were slightly decreased, his clinical conditions were deteriorating progressively. In order to overcome the mitochondrial or cytosolic energy crisis indicated by lactic acidosis as well as clinical symptoms, we terminated the DCA and administered 0.5 g/kg/day TID of sodium pyruvate orally. We analyzed the therapeutic effects of DCA or sodium pyruvate in the patient, and found that pyruvate therapy significantly decreased lactate, pyruvate and alanine levels, showed no adverse effects such as severe neuropathy seen in DCA, and had better clinical response on development and epilepsy. Though the efficacy of pyruvate on LS will be evaluated by randomized double-blind placebo-controlled study design in future, pyruvate therapy is a possible candidate for therapeutic choice for currently incurable mitochondrial disorders such as LS.

  18. Psychological Distress in Twins with Urological Symptoms

    PubMed Central

    Wright, Lisa Johnson; Noonan, Carolyn; Ahumada, Sandra; Rodríguez, María Ángeles Bullones; Buchwald, Dedra; Afari, Niloofar

    2010-01-01

    Objective Interstitial cystitis/painful bladder syndrome (IC/PBS) is a chronic pain condition with unclear underlying etiology. Our objectives were to determine if psychological distress was higher in twins with urological symptoms commonly found in IC/PBS than twins without, and if so, did familial influences contribute to this association. Method Data from 1,165 female twins in a community-based sample were used. Urological symptoms, symptoms of posttraumatic stress disorder (PTSD), depression, anxiety, and perceived stress were assessed with standardized questionnaires. Generalized estimating equation regression models were used to examine the relationship between psychological distress and urological symptoms. Results Compared to unaffected twins, twins with urological symptoms were more likely to report PTSD symptoms (OR = 3.9; CI = 2.6-5.8), depression (OR = 3.1; CI = 2.0-5.0), anxiety (OR = 3.4; CI = 2.3-5.2), and perceived stress (OR = 3.2; CI = 2.1-4.9). After adjusting for familial influences, the within-pair effects remained significant for PTSD symptoms (OR = 2.2; CI = 1.2-3.8) and perceived stress (OR = 2.2; CI = 1.2-3.8). Conclusion Familial influences partially explained the relationship between indicators of psychological distress and urological symptoms. Future research should examine shared environmental and genetic mechanisms that may further explain this relationship and improve diagnosis and treatment of this unexplained clinical condition. PMID:20430229

  19. Models of the Joint Structure of Domain-Related and Global Distress: Implications for the Reconciliation of Quality of Life and Mental Health Perspectives

    ERIC Educational Resources Information Center

    Magee, William; St-Arnaud, Sebastien

    2012-01-01

    Research on subjective wellbeing includes studies of both domain-related and global distress. The mental health literature, though, focuses almost exclusively on global distress. This seems to be partly due to a common belief that psychological distress, and the moods that comprise distress, necessarily lack referential content. However, if that…

  20. Intravitreal dexamethasone implant for recurrent cystoid macular edema due to Irvine-Gass syndrome: a prospective case series.

    PubMed

    Sudhalkar, A; Chhablani, J; Vasavada, A; Bhojwani, D; Vasavada, V; Vasavada, S

    2016-12-01

    PurposeTo determine the preliminary efficacy and safety of off-label dexamethasone implant for treatment of recurrent cystoid macular edema (CME) secondary to Irvine-Gass syndrome (IGS).Patients and methodsThis study was set in Raghudeep Eye Clinic, Ahmedabad and LV Prasad Eye Institute, Hyderabad (India). It is a Prospective Case Series. Prospective case series comprising of patients with uncomplicated pseudophakia and CME due to IGS who recurred after one course of topical steroids with NSAIDS and a sub-Tenon corticosteroid injection. A complete ocular and systemic exam, fluorescein angiography, and central subfield thickness (CST) on optical coherence tomography scans were performed. Follow-up visits were on days 1, 15, and 30 and then monthly for a year. Appropriate statistical analysis was done. The primary outcome measure was the change in CDVA at months 1, 6, and 12. Secondary outcome measures were recurrence of CME and complications if any as noted at months 1, 2, 6, and 12.ResultsAbout 27 patients (27 eyes) with 16 males were included. Median age: 63.24±5.62 years. At 1 month, the CDVA improved to 0.04±0.02 (20/25) logMAR from 0.52±0.12 logMAR (20/70) (P=0.001) with a reduction in CST from 454.2±45.3 to 218.32±38.15 microns(P=0.013). The CDVA was 0.04±0.03 logMAR(P<0.001) at month 6 and 0.05±0.02 logMAR(P<0.001) at month 12. The CST was 221±35.2 microns (P=0.013) at month 6 and 214±43.34 microns (P=0.0124) at month 12. All improvements were maintained for a year. Only one patient required a second injection. No complications were noted.ConclusionThe implant is safe and effective for the treatment of recurrent CME due to IGS.

  1. Hysterectomy due to Abnormal Uterine Bleeding in a 15-year Old Girl with Rubinstein-Taybi Syndrome

    PubMed Central

    Lee, Kwang Ho; Park, Eun Young; Jung, Sang Woo; Song, Seung Woo; Lim, Hyun Kyo

    2016-01-01

    Rubinstein-Taybi syndrome is characterized by mental retardation, atypical facial features, broad thumbs and toes, and scoliosis. Polycystic ovaries are associated with chronic anovulation and abnormal uterine bleeding. A 15-year old female patient was diagnosed with Rubinstein-Taybi Syndrome, and had prolonged abnormal uterine bleeding for 2 years, accompanied by a polycystic ovary. As she showed no improvement during hormonal therapy or medical treatment, a hysterectomy was performed to control the bleeding. PMID:27924288

  2. Coexistence of Obstructive Sleep Apnea and Superior Vena Cava Syndromes Due to Substernal Goitre in a Patient With Respiratory Failure: A Case Report

    PubMed Central

    Tunc, Mehtap; Sazak, Hilal; Karlilar, Bulent; Ulus, Fatma; Tastepe, Irfan

    2015-01-01

    Introduction: Substernal goiter may rarely cause superior vena cava syndrome (SVCS) owing to venous compression, and cause acute respiratory failure due to tracheal compression. Obstructive sleep apnea syndrome (OSAS) may rarely occur when there is a narrowing of upper airway by edema and vascular congestion resulting from SVCS. Case Presentation: We presented the clinical course and treatment of acute respiratory failure (ARF) developed in a patient with SVCS and OSAS due to substernal goiter. After treatment of ARF with invasive mechanical ventilation, weaning and total thyroidectomy were successfully performed through collar incision and median sternotomy without complications. Conclusions: Our case showed that if the respiratory failure occurred due to substernal goiter and SVCS, we would need to investigate the coexistence of OSAS and SVCS. PMID:26082848

  3. Patterns of Distress in African American Mothers of Preterm Infants

    PubMed Central

    Holditch-Davis, Diane; Miles, Margaret Shandor; Weaver, Mark A.; Black, Beth; Beeber, Linda; Thoyre, Suzanne; Engelke, Stephen

    2009-01-01

    Objective To examine inter-relationships among stress due to infant appearance and behavior in the NICU, parental role alteration stress in the NICU, depressive symptoms, state anxiety, post-traumatic stress symptoms, and daily hassles exhibited by African American mothers of preterm infants and to determine whether there were sub-groups of mothers based on patterns of psychological distress. Method 177 African American mothers completed questionnaires on their psychological distress at enrollment during infant hospitalization and 2, 6, 12, 18, and 24 months after term. Results Psychological distress measures were inter-correlated. There were four latent classes of mothers: the low distress class with low scores on all measures; the high NICU-related stress class with high infant appearance and parental role stress and moderate scores on other measures; the high depressive symptoms class with high depressive symptoms and state anxiety and moderately elevated scores on NICU-related stress and post-traumatic stress symptoms; the extreme distress class with the highest means on all measures. Infants in the high stress class were sicker than infants in the other classes. The extreme distress class mothers averaged the lowest educational level. The classes differed on distress measures, worry about the child, and parenting stress through 24 months with the extreme distress class having the highest values. Conclusion Although different types of maternal psychological distress were substantially related, there were distinct sub-groups of mothers that were identifiable in the NICU. Moreover, these sub-groups continued to differ on trajectories of distress and on their perceptions of the infants and parenting through 24 months after term. PMID:19412125

  4. Syndromic mental retardation with thrombocytopenia due to 21q22.11q22.12 deletion: Report of three patients.

    PubMed

    Katzaki, Eleni; Morin, Gilles; Pollazzon, Marzia; Papa, Filomena Tiziana; Buoni, Sabrina; Hayek, Joussef; Andrieux, Joris; Lecerf, Laure; Popovici, Cornel; Receveur, Aline; Mathieu-Dramard, Michèle; Renieri, Alessandra; Mari, Francesca; Philip, Nicole

    2010-07-01

    During the last few years, an increasing number of microdeletion/microduplication syndromes have been delineated. This rapid evolution is mainly due to the availability of microarray technology as a routine diagnostic tool. Microdeletions of the 21q22.11q22.12 region encompassing the RUNX1 gene have been reported in nine patients presenting with syndromic thrombocytopenia and mental retardation. RUNX1 gene is responsible for an autosomal dominant platelet disorder with predisposition to acute myelogenous leukemia. We report on three novel patients with an overlapping "de novo" interstitial deletion involving the band 21q22 characterized by array-CGH. All our patients presented with severe developmental delay, dysmorphic features, behavioral problems, and thrombocytopenia. Comparing the clinical features of our patients with the overlapping ones already reported two potential phenotypes related to 21q22 microdeletion including RUNX1 were highlighted: thrombocytopenia with +/- mild dysmorphic features and syndromic thrombocytopenia with growth and developmental delay.

  5. Postperfusion lung syndrome: physiopathology and therapeutic options

    PubMed Central

    Yuan, Shi-Min

    2014-01-01

    Postperfusion lung syndrome is rare but can be lethal. The underlying mechanism remains uncertain but triggering inflammatory cascades have become an accepted etiology. A better understanding of the pathophysiology and the roles of inflammatory mediators in the development of the syndrome is imperative in the determination of therapeutic options and promotion of patients' prognosis and survival. Postperfusion lung syndrome is similar to adult respiratory distress syndrome in clinical features, diagnostic approaches and management strategies. However, the etiologies and predisposing risk factors may differ between each other. The prognosis of the postperfusion lung syndrome can be poorer in comparison to acute respiratory distress syndrome due to the secondary multiple organ failure and triple acid-base imbalance. Current management strategies are focusing on attenuating inflammatory responses and preventing from pulmonary ischemia-reperfusion injury. Choices of cardiopulmonary bypass circuit and apparatus, innovative cardiopulmonary bypass techniques, modified surgical maneuvers and several pharmaceutical agents can be potential preventive strategies for acute lung injury during cardiopulmonary bypass. PMID:25372917

  6. Performance of Multiple Risk Assessment Tools to Predict Mortality for Adult Respiratory Distress Syndrome with Extracorporeal Membrane Oxygenation Therapy: An External Validation Study Based on Chinese Single-center Data

    PubMed Central

    Huang, Lei; Li, Tong; Xu, Lei; Hu, Xiao-Min; Duan, Da-Wei; Li, Zhi-Bo; Gao, Xin-Jing; Li, Jun; Wu, Peng; Liu, Ying-Wu; Wang, Song; Lang, Yu-Heng

    2016-01-01

    Background: There has been no external validation of survival prediction models for severe adult respiratory distress syndrome (ARDS) with extracorporeal membrane oxygenation (ECMO) therapy in China. The aim of study was to compare the performance of multiple models recently developed for patients with ARDS undergoing ECMO based on Chinese single-center data. Methods: A retrospective case study was performed, including twenty-three severe ARDS patients who received ECMO from January 2009 to July 2015. The PRESERVE (Predicting death for severe ARDS on VV-ECMO), ECMOnet, Respiratory Extracorporeal Membrane Oxygenation Survival Prediction (RESP) score, a center-specific model developed for inter-hospital transfers receiving ECMO, and the classical risk-prediction scores of Acute Physiology and Chronic Health Evaluation (APACHE) II and Sequential Organ Failure Assessment (SOFA) were calculated. In-hospital and six-month mortality were regarded as the endpoints and model performance was evaluated by comparing the area under the receiver operating characteristic curve (AUC). Results: The RESP and APACHE II scores showed excellent discriminate performance in predicting survival with AUC of 0.835 (95% confidence interval [CI], 0.659–1.010, P = 0.007) and 0.762 (95% CI, 0.558–0.965, P = 0.035), respectively. The optimal cutoff values were risk class 3.5 for RESP and 35.5 for APACHE II score, and both showed 70.0% sensitivity and 84.6% specificity. The excellent performance of these models was also evident for the pneumonia etiological subgroup, for which the SOFA score was also shown to be predictive, with an AUC of 0.790 (95% CI, 0.571–1.009, P = 0.038). However, the ECMOnet and the score developed for externally retrieved ECMO patients failed to demonstrate significant discriminate power for the overall cohort. The PRESERVE model was unable to be evaluated fully since only one patient died six months postdischarge. Conclusions: The RESP, APCHAE II, and SOFA scorings

  7. Hypochondroplasia due to FGFR3 gene mutation (N540K) and mosaic form of Down syndrome in the same patient.

    PubMed

    Dumic, Katja; Barisic, Ingeborg; Potocki, Kristina; Sansovic, Ivona

    2011-05-01

    The simultaneous presence of Down syndrome and achondroplasia has rarely been reported in the literature, and our search revealed only six patients with such an association. We are reporting the first case of a patient with Down syndrome and hypochondroplasia. In this patient, Down syndrome was clinically recognised and confirmed by the cytogenetic finding of mosaic karyotype (47,XX,+21/46,XX) shortly after birth. She was subsequently diagnosed with hypochondroplasia at the age of 6 years when disproportional short stature, stocky habitus and macrocephaly were observed. These phenotypic findings were later confirmed by the presence of fibroblast growth factor receptor 3 (FGFR3) gene mutation N540K. The overlapping common clinical features of Down syndrome and hypochondroplasia resulted in delayed diagnosis of hypochondroplasia in our patient and the associated deleterious effect on her linear growth. Her final height is 126.5 cm, which is -3.76 standard deviations (SD) lower than the median height in patients with Down syndrome, and is under the lower borderline of the adult height range for women with hypochondroplasia.

  8. Endogenous Cushing's Syndrome with Precocious Puberty in an 8-Year-Old Boy due to a Large Unilateral Adrenal Adenoma

    PubMed Central

    Hossain, Muhammad Rajib; Alam, Md. Mashiul; Nabi, Junaid; Kibria, Mahzabin

    2013-01-01

    Adrenocortical tumors (ACTs) causing Cushing's syndrome are extremely rare in children and adolescents. Bilateral macronodular adrenocortical disease which is a component of the McCune-Albright syndrome is the most common cause of endogenous Cushing's syndrome. We report the case of a boy with Cushing's syndrome who presented with obesity and growth retardation. The child was hypertensive. The biochemical evaluation revealed that his serum cortisol levels were 25.80 μg/dL, with a concomitant plasma ACTH level of 10.0 pg/mL and nonsuppressed serum cortisol on high-dose dexamethasone suppression test (HDDST) to be 20.38 μg/dL. Computed tomography of the abdomen demonstrated a 8 × 6 × 5 cm left adrenal mass with internal calcifications. Following preoperative stabilization, laparotomy was carried out which revealed a lobulated left adrenal mass with intact capsule weighing 120 grams. Histopathological examination revealed a benign cortical neoplastic lesion, suggestive of adrenal adenoma; composed of large polygonal cells with centrally placed nuclei and prominent nucleoli without capsular and vascular invasion. On the seventh postoperative day, cortisol levels were within normal range indicating biochemical remission of Cushing's syndrome. On followup after three months, the patient showed significant clinical improvement and had lost moderate amount of weight and adrenal imaging was found to be normal. PMID:23533838

  9. Hyperparathyroidism-jaw tumor syndrome in Roma families from Portugal is due to a founder mutation of the HRPT2 gene.

    PubMed

    Cavaco, Branca M; Guerra, Laura; Bradley, Karin J; Carvalho, Davide; Harding, Brian; Oliveira, Amélia; Santos, Maria-Amparo; Sobrinho, Luís G; Thakker, Rajesh V; Leite, Valeriano

    2004-04-01

    The hyperparathyroidism-jaw tumor (HPT-JT) syndrome is an autosomal dominant disorder characterized by the occurrence of parathyroid tumors and ossifying jaw fibromas. The gene causing HPT-JT, HRPT2, is located on chromosome 1q31.2 and consists of 17 exons that encode a 531-amino acid protein, designated parafibromin. We recently identified six Roma families in Portugal with 56 members (11 affected and 45 asymptomatic), who had the HPT-JT syndrome. We postulated that they may have a common ancestor and that the HPT-JT syndrome may be due to a mutation of the HRPT2 gene. Haplotype analysis using 14 chromosome 1q24-q32 polymorphic markers showed that the 11 affected individuals shared a common haplotype defined by seven markers that spanned an approximately 12.5-cM region, flanked centromerically by D1S202 and telomerically by D1S306. DNA sequence analysis identified a 2-bp (TG or GT) frameshift deletion in exon 8, which predicts a truncated parafibromin protein, in all 11 affected individuals. This mutation was also found in 19 unaffected individuals (age range, 12-74 yr) who shared the affected haplotype, suggesting a low age-related penetrance for HPT-JT in these families. Thus, the HPT-JT syndrome in six Roma families from Portugal is due to a novel founder mutation in the HRPT2 gene.

  10. Jacobsen Syndrome: Surgical Complications due to Unsuspected Diagnosis, the Importance of Molecular Studies in Patients with Craniosynostosis.

    PubMed

    Linares Chávez, Etzalli P; Toral López, Jaime; Valdés Miranda, Juan M; González Huerta, Luz M; Perez Cabrera, Adrian; Del Refugio Rivera Vega, María; Messina Baas, Olga M; Cuevas-Covarrubias, Sergio A

    2016-02-01

    Jacobsen syndrome (JBS) is an uncommon contiguous gene syndrome. About 85-92% of cases have a de novo origin. Clinical variability and severity probably depend on the size of the affected region. The typical clinical features in JBS include intellectual disability, growth retardation, craniofacial dysmorphism as well as craniosynostosis, congenital heart disease, and platelet abnormalities. The proband was a 1 year/3-month-old Mexican male. Oligonucleotide-SNP array analysis using the GeneChip Human Cytoscan HD was carried out for the patient from genomic DNA. The SNP array showed a 14.2-Mb deletion in chromosome 11q23.3q25 (120,706-134,938 Mb), which involved 163 RefSeq genes in the database of genomic variation. We report a novel deletion in JBS that increases the knowledge of the variability in the mutation sites in this region and expands the spectrum of molecular and clinical defects in this syndrome.

  11. Jacobsen Syndrome: Surgical Complications due to Unsuspected Diagnosis, the Importance of Molecular Studies in Patients with Craniosynostosis

    PubMed Central

    Linares Chávez, Etzalli P.; Toral López, Jaime; Valdés Miranda, Juan M.; González Huerta, Luz M.; Perez Cabrera, Adrian; del Refugio Rivera Vega, María; Messina Baas, Olga M.; Cuevas-Covarrubias, Sergio A.

    2016-01-01

    Jacobsen syndrome (JBS) is an uncommon contiguous gene syndrome. About 85-92% of cases have a de novo origin. Clinical variability and severity probably depend on the size of the affected region. The typical clinical features in JBS include intellectual disability, growth retardation, craniofacial dysmorphism as well as craniosynostosis, congenital heart disease, and platelet abnormalities. The proband was a 1 year/3-month-old Mexican male. Oligonucleotide-SNP array analysis using the GeneChip Human Cytoscan HD was carried out for the patient from genomic DNA. The SNP array showed a 14.2-Mb deletion in chromosome 11q23.3q25 (120,706-134,938 Mb), which involved 163 RefSeq genes in the database of genomic variation. We report a novel deletion in JBS that increases the knowledge of the variability in the mutation sites in this region and expands the spectrum of molecular and clinical defects in this syndrome. PMID:26997943

  12. What Is Acute Respiratory Distress Syndrome?

    MedlinePlus

    ... usually need the help of a mechanical ventilator ( respirator ) to breathe. With medical care, many people with ... sup- port the person’s breathing (usually with a respirator) until the lungs heal. Most people with ARDS ...

  13. Sequelae of the adult respiratory distress syndrome.

    PubMed Central

    Hert, R.; Albert, R. K.

    1994-01-01

    Most survivors of ARDS have persistent mild reductions of TLCO even as long as a year after their episode. The lung volumes and flows return to normal in most instances, although a subset of patients will have persistent impairment. Both obstructive and restrictive deficits may be seen. This group may be predicted by the degree of acute lung injury assessed by the level of FIO2, PEEP, and gas exchange abnormality that exists in the first few days. In the first year after ARDS most physiological abnormalities will improve, but if deficits persist at one year further improvement is unlikely. Although many patients report dyspnoea following ARDS, the symptom does not correlate with abnormalities of pulmonary function. The possibility that conventional management may augment the degree of acute injury and worsen outcome must be considered. The effects of chronic hyperoxia in humans with acute lung injury or those of high levels of PEEP compared with low levels are not known. Exploring new ventilator management strategies while we await more specific treatment directed at the primary problem of acute lung inflammation will hopefully reduce acute mortality as well as acute and chronic morbidity. Images PMID:8153946

  14. How Is Respiratory Distress Syndrome Treated?

    MedlinePlus

    ... Surfactant Replacement Therapy Surfactant is a liquid that coats the inside of the lungs. It helps keep ... during pregnancy. These medicines can speed up surfactant production and lung development in a fetus. Even if ...

  15. Upper gastrointestinal bleeding in a young patient with Budd Chiari syndrome due to a mutation of factor V Leiden: a case report.

    PubMed

    Dina, Ion; Iacobescu, Claudia; Goldis, Adrian

    2013-06-01

    Budd Chiari syndrome or hepatic venous outflow obstruction is a complex entity with multiple etiologies and various clinical manifestations. It is often difficult to establish the diagnosis. The most common cause is a hypercoagulable state due to either genetic disorders of blood coagulation or several acquired conditions such as hematological diseases, tumors, infections, chronic inflammatory diseases, pregnancy. The most common clinical presentation is hepatomegaly, abdominal pain and ascites, but the onset can also be dramatical and lif