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  1. Neonatal respiratory distress syndrome

    MedlinePlus

    Hyaline membrane disease (HMD); Infant respiratory distress syndrome; Respiratory distress syndrome in infants; RDS - infants ... Neonatal RDS occurs in infants whose lungs have not yet fully ... disease is mainly caused by a lack of a slippery substance in ...

  2. [Nitric oxide inhalation as an effective therapy for acute respiratory distress syndrome due to near-drowning: a case report].

    PubMed

    Takano, Y; Hirosako, S; Yamaguchi, T; Saita, N; Suga, M; Kukita, I; Okamoto, K; Ando, M

    1999-12-01

    A 16-year-old boy with acute respiratory distress syndrome (ARDS) due to near-drowning was admitted to our hospital. ARDS was treated with low-level nitric oxide (NO) inhalation (ranging from 4 ppm to 1 ppm) for 24 days. Oxygenation was improved and pulmonary hypertension was reduced after NO inhalation, but systemic blood pressure, heart rate, and cardiac output were not affected. PaO2 improved from 153 Torr to 354 Torr under identical ventilating conditions (F1O2 1.0), and mean pulmonary arterial pressure fell from 40 mm Hg to 27 mmHg. It has been reported that NO inhalation alleviates ventilation-flow mismatch and pulmonary hypertension. It is unclear, however, whether this therapy improves the prognosis for ARDS. In our patient, NO inhalation was effective in alleviating the oxygenation impairment and pulmonary hypertension associated with ARDS.

  3. [Acute respiratory distress syndrome].

    PubMed

    Estenssoro, Elisa; Dubin, Arnaldo

    2016-01-01

    Acute respiratory distress syndrome (ARDS) is an acute respiratory failure produced by an inflammatory edema secondary to increased lung capillary permeability. This causes alveolar flooding and subsequently deep hypoxemia, with intrapulmonary shunt as its most important underlying mechanism. Characteristically, this alteration is unresponsive to high FIO2 and only reverses with end-expiratory positive pressure (PEEP). Pulmonary infiltrates on CXR and CT are the hallmark, together with decreased lung compliance. ARDS always occurs within a week of exposition to a precipitating factor; most frequently pneumonia, shock, aspiration of gastric contents, sepsis, and trauma. In CT scan, the disease is frequently inhomogeneous, with gravitational infiltrates coexisting with normal-density areas and also with hyperaerated parenchyma. Mortality is high (30-60%) especially in ARDS associated with septic shock and neurocritical diseases. The cornerstone of therapy lies in the treatment of the underlying cause and in the use mechanical ventilation which, if inappropriately administered, can lead to ventilator-induced lung injury. Tidal volume = 6 ml/kg of ideal body weight to maintain an end-inspiratory (plateau) pressure = 30 cm H2O ("protective ventilation") is the only variable consistently associated with decreased mortality. Moderate-to-high PEEP levels are frequently required to treat hypoxemia, yet no specific level or titration strategy has improved outcomes. Recently, the use of early prone positioning in patients with PaO2/FIO2 = 150 was associated with increased survival. In severely hypoxemic patients, it may be necessary to use adjuvants of mechanical ventilation as recruitment maneuvers, pressure-controlled modes, neuromuscular blocking agents, and extracorporeal-membrane oxygenation. Fluid restriction appears beneficial. PMID:27576283

  4. Repeated lung lavage with extracorporeal membrane oxygenation treating severe acute respiratory distress syndrome due to nasogastric tube malposition for enternal nutrition: a case report.

    PubMed

    Kao, Xiaoming; Yu, Wenkui; Zhu, Weiming; Li, Ning; Li, Jieshou

    2012-01-01

    Enternal nutritional support, a frequently applied technique for providing nutrition and energy, played a pivotal role in the treatment of high risk patients. However, severe complications induced by malposition of nasogastric tube caused great danger and even death to the patients. In this case report, we present a patient with severe acute respiratory distress syndrome (ARDS) induced by bronchopleural fistula (BPF) due to malposition of nasogastric tube. Repeated lung lavage combined with extracorporeal membrane oxygenation (ECMO) was performed after transferring to the ICU of our hospital. Finally, the patient recovered and discharged 7 days after admission.

  5. [Survival by a young woman with malnutrition due to alcoholism and eating disorders and with acute respiratory distress syndrome due to severe pneumonia who showed increased serum neutrophil elastase activity].

    PubMed

    Nakajima, Hirokazu; Sawaguchi, Hirochiyo; Nakajima, Shigenori

    2006-11-01

    A 30-year-old woman with malnutrition due to alcoholism and eating disorders was found to have acute respiratory distress syndrome (ARDS) and sepsis due to severe Streptococcus pneumoniae pneumonia. S. pneumoniae was detected by an in vitro rapid immunochromatographic assay for S. pneumoniae antigen in urine on the day of admission and by blood culture 2 days after admission. Symptoms and laboratory findings improved after treatment with sivelestat sodium hydrate, antibiotics, and mechanical ventilation. Treatment with sivelestat sodium hydrate also decreased serum neutrophil elastase activity. This case demonstrates the usefulness of early treatment with sivelestat sodium hydrate in ARDS due to severe pneumonia.

  6. Maternal smoking and respiratory distress syndrome.

    PubMed

    Curet, L B; Rao, A V; Zachman, R D; Morrison, J; Burkett, G; Poole, W K

    1983-10-15

    Infants of 603 patients on whom information about smoking habits during pregnancy was available were studied for incidence respiratory distress syndrome. Among the 360 patients who did not smoke, the incidence of respiratory distress syndrome in the neonate was 15.1%, whereas among patients who smoked, the incidence was 9.1%. We speculate that smoking produces a condition of chronic stress in the fetus which brings about an acceleration of fetal pulmonary maturation. PMID:6624813

  7. Gas exchange in the respiratory distress syndromes.

    PubMed

    Albert, Richard K; Jobe, Alan

    2012-07-01

    This article describes the gas exchange abnormalities occurring in the acute respiratory distress syndrome seen in adults and children and in the respiratory distress syndrome that occurs in neonates. Evidence is presented indicating that the major gas exchange abnormality accounting for the hypoxemia in both conditions is shunt, and that approximately 50% of patients also have lungs regions in which low ventilation-to-perfusion ratios contribute to the venous admixture. The various mechanisms by which hypercarbia may develop and by which positive end-expiratory pressure improves gas exchange are reviewed, as are the effects of vascular tone and airway narrowing. The mechanisms by which surfactant abnormalities occur in the two conditions are described, as are the histological findings that have been associated with shunt and low ventilation-to-perfusion. PMID:23723019

  8. Surfactant treatment for acute respiratory distress syndrome

    PubMed Central

    Lopez-Herce, J.; de Lucas, N.; Carrillo, A.; Bustinza, A.; Moral, R.

    1999-01-01

    OBJECTIVE—To determine prospectively the efficacy of surfactant in acute respiratory distress syndrome.
STUDY DESIGN—Twenty patients, 1 month to 16 years of age, diagnosed with an acute pulmonary disease with severe hypoxaemia (PaO2/FiO2 < 100) (13 with systemic or pulmonary disease and seven with cardiac disease) were treated with one to six doses of 50-200 mg/kg of porcine surfactant administered directly into the trachea. The surfactant was considered to be effective when the PaO2/FiO2 improved by > 20%.
RESULTS—After initial surfactant administration the PaO2/FiO2 increased significantly in patients with systemic or pulmonary disease from 68 to 111, and the oxygenation index (OI) diminished significantly from 36.9 to 27.1. The PaO2/FiO2 and OI did not improve in children with cardiac disease. The improvement of the patients who survived was greater than that of those who died.
CONCLUSIONS—Surfactant moderately improves oxygenation in some children with severe acute respiratory distress syndrome secondary to pulmonary or systemic disease.

 PMID:10325705

  9. Controversies involving hypercapnic acidosis in acute respiratory distress syndrome.

    PubMed

    Nardelli, Liliane; Rocco, Patricia Rieken Macedo; Garcia, Cristiane Sousa Nascimento Baez

    2009-12-01

    Acute respiratory distress syndrome is characterized by a diffuse inflammatory reaction of lung parenchyma induced by a direct insult to the alveolar epithelium (pulmonary acute respiratory distress syndrome) or an indirect lesion through the vascular endothelium (extrapulmonary acute respiratory distress syndrome). The main therapeutic strategy for acute respiratory distress syndrome is the ventilatory support. However, mechanical ventilation can worsen lung injury. In this context, a protective ventilatory strategy with low tidal volume has been proposed. The use of low tidal volume reduced the mortality rate of acute respiratory distress syndrome patients, but result in hypercapnic acidosis. The current article presents a review of literature on the effects of permissive hypercapnia in acute respiratory distress syndrome. To that end, we carried out a systematic review of scientific literature based on established criteria for documental analysis including clinical and experimental articles, using as data bases MedLine, LILACS, SciELO, PubMed, Cochrane. Hypercapnic acidosis has been considered by some authors as a modulator of the inflammatory process of acute respiratory distress syndrome. However, clinical and experimental studies on the effects of hypercapnic acidosis have shown controversial results. Therefore it is important to better elucidate the role of hypercapnic acidosis in acute respiratory distress syndrome.

  10. Dress syndrome with sepsis, acute respiratory distress syndrome and pneumomediastinum.

    PubMed

    Giri, Prabhas Prasun; Roy, Swapan; Bhattyacharya, Sukanta; Pal, Priyankar; Dhar, Sandipan

    2011-11-01

    Drug rash with eosinophilia and systemic symptoms (DRESS) syndrome reflects a serious hypersensitivity reaction to drugs, and is characterized by skin rash, fever, lymph node enlargement, and internal organ involvement. So far, numerous drugs such as sulfonamides, phenobarbital, sulfasalazine, carbamazepine, and phenytoin have been reported to cause DRESS syndrome. We report a case of a 10-year-old girl who developed clinical manifestations of fever, rash, lymphadenopathy, hypereosinophilia, and visceral involvement (hepatitis and pneumonitis) after taking phenobarbital for seizures, with subsequent development of sepsis, acute respiratory distress syndrome (ARDS) and spontaneous air leak syndrome (pnemothorax and pneumomediastinum). She was put on steroids and various antibiotics and was ventilated, but ultimately succumbed to sepsis and pulmonary complications. PMID:22345792

  11. 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?

  12. Association between ruptured membranes, tocolytic therapy, and respiratory distress syndrome.

    PubMed

    Curet, L B; Rao, A V; Zachman, R D; Morrison, J C; Burkett, G; Poole, W K; Bauer, C

    1984-02-01

    Two hundred ninety-seven patients from the placebo group of the National Institutes of Health Collaborative Study on Antenatal Steroid Therapy for prevention of respiratory distress syndrome were selected for analysis to investigate a possible association between premature rupture of the membranes, tocolytic therapy, and respiratory distress syndrome. Both premature rupture of the membranes and tocolytic therapy with isoxsuprine were individually associated with a lowered incidence of respiratory distress syndrome. However, when present together, their protective effect was not additive and resulted in a higher incidence of respiratory distress syndrome. It is suggested that the use of tocolytic therapy with beta-adrenergic agents be restricted to patients with intact membranes. PMID:6695972

  13. 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

  14. Acute respiratory distress syndrome in the global context.

    PubMed

    Buregeya, Egide; Fowler, Robert A; Talmor, Daniel S; Twagirumugabe, Theogene; Kiviri, Willy; Riviello, Elisabeth D

    2014-09-01

    Acute respiratory distress syndrome (ARDS) is a clinically defined syndrome of hypoxia and bilateral pulmonary infiltrates due to inflammatory pathways triggered by pulmonary and nonpulmonary insults, and ARDS is pathologically correlated with diffuse alveolar damage. Estimates of ARDS's impact in the developed world vary widely, with some of the discrepancies attributed to marked differences in the availability of intensive care beds and mechanical ventilation. Almost nothing is known about the epidemiology of ARDS in the developing world, in part due to a clinical definition requiring positive pressure ventilation, arterial blood gases, and chest radiography. Current frameworks for comparing the epidemiology of death and disability across the world including the GBD (Global Burden of Disease Study) 2010 are ill-suited to quantifying critical illness syndromes including ARDS. Modifications to the definition of ARDS to allow a provision for environments without the capacity for positive pressure ventilation, and to allow for alternate diagnostic techniques including pulse oximetry and ultrasound, may make it possible to quantify and describe the impact of ARDS in the global context. PMID:25667180

  15. [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.

  16. Genetic risk factors associated with respiratory distress syndrome.

    PubMed

    Jo, Heui Seung

    2014-04-01

    Respiratory distress syndrome (RDS) among preterm infants is typically due to a quantitative deficiency of pulmonary surfactant. Aside from the degree of prematurity, diverse environmental and genetic factors can affect the development of RDS. The variance of the risk of RDS in various races/ethnicities or monozygotic/dizygotic twins has suggested genetic influences on this disorder. So far, several specific mutations in genes encoding surfactant-associated molecules have confirmed this. Specific genetic variants contributing to the regulation of pulmonary development, its structure and function, or the inflammatory response could be candidate risk factors for the development of RDS. This review summarizes the background that suggests the genetic predisposition of RDS, the identified mutations, and candidate genetic polymorphisms of pulmonary surfactant proteins associated with RDS.

  17. 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

  18. A Brief Review of the Adult Respiratory Distress Syndrome

    PubMed Central

    Hassan, Sheik N.; Hackney, Robert L.

    1982-01-01

    The adult respiratory distress syndrome (ARDS) is seen with increasing frequency. It is associated with a variety of conditions even in the absence of preexisting pulmonary diseases. The treatment often includes intubation, using positive-end expiratory pressure (PEEP), and other ancillary support. The mortality rate is high, and physicians caring for such patients should be on guard for disseminated intravascular coagulation, gastrointestinal hemorrhage, and infection. PMID:7120452

  19. Acute respiratory distress syndrome (ARDS) in lambs. Hematology.

    PubMed

    Ulvund, M J; Grønstøl, H

    1984-01-01

    Lambs suffering from Acute Respiratory Distress Syndrome (ARDS) showed elevated PCV, neutrophilia, a tendency towards lymphopenia, eosinopenia, hyperphosphatemia, hypoglycemia and extremely low serum Ca values during the first couple of days after the outbreak of symptoms. During the very early phase, plasma potassium values were mostly lowered (Figs. 1-3, Table I). The possible involvement of histamine is shortly discussed: either 1) through an atopic reaction, 2) because of acute ruminal acidosis and sudden histamine formation, or 3) involvement of endotoxins.

  20. Pediatric Acute Respiratory Distress Syndrome: Fluid Management in the PICU

    PubMed Central

    Ingelse, Sarah A.; Wösten-van Asperen, Roelie M.; Lemson, Joris; Daams, Joost G.; Bem, Reinout A.; van Woensel, Job B.

    2016-01-01

    The administration of an appropriate volume of intravenous fluids, while avoiding fluid overload, is a major challenge in the pediatric intensive care unit. Despite our efforts, fluid overload is a very common clinical observation in critically ill children, in particular in those with pediatric acute respiratory distress syndrome (PARDS). Patients with ARDS have widespread damage of the alveolar–capillary barrier, potentially making them vulnerable to fluid overload with the development of pulmonary edema leading to prolonged course of disease. Indeed, studies in adults with ARDS have shown that an increased cumulative fluid balance is associated with adverse outcome. However, age-related differences in the development and consequences of fluid overload in ARDS may exist due to disparities in immunologic response and body water distribution. This systematic review summarizes the current literature on fluid imbalance and management in PARDS, with special emphasis on potential differences with adult patients. It discusses the adverse effects associated with fluid overload and the corresponding possible pathophysiological mechanisms of its development. Our intent is to provide an incentive to develop age-specific fluid management protocols to improve PARDS outcomes. PMID:27047904

  1. Acute respiratory distress syndrome and acute lung injury.

    PubMed

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

    2011-09-01

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

  2. Fatal measles presenting as acute respiratory distress syndrome in an immunocompetent adult

    PubMed Central

    Karanth, Suman S; Marupudi, Krishna Chaitanya; Gupta, Anurag; Rau, Nileshwar Radhakrishna

    2014-01-01

    Fatal measles is known to occur among immunocompromised adults. We report a rare case of an immunocompetent non-pregnant young lady who suffered from fatal acute respiratory distress syndrome due to measles. Physicians must be vigilant to this deadly presentation of measles even in immunocompetent individuals. We emphasise the inadequacies of vaccination programmes in India reflected not only by the existing high measles-related childhood mortalities, but also an emerging rise in deaths among adults. PMID:25139919

  3. Fatal measles presenting as acute respiratory distress syndrome in an immunocompetent adult.

    PubMed

    Karanth, Suman S; Marupudi, Krishna Chaitanya; Gupta, Anurag; Rau, Nileshwar Radhakrishna

    2014-08-19

    Fatal measles is known to occur among immunocompromised adults. We report a rare case of an immunocompetent non-pregnant young lady who suffered from fatal acute respiratory distress syndrome due to measles. Physicians must be vigilant to this deadly presentation of measles even in immunocompetent individuals. We emphasise the inadequacies of vaccination programmes in India reflected not only by the existing high measles-related childhood mortalities, but also an emerging rise in deaths among adults.

  4. 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

  5. Sepsis and Acute Respiratory Distress Syndrome: Recent Update.

    PubMed

    Kim, Won-Young; Hong, Sang-Bum

    2016-04-01

    Severe sepsis or septic shock is characterized by an excessive inflammatory response to infectious pathogens. Acute respiratory distress syndrome (ARDS) is a devastating complication of severe sepsis, from which patients have high mortality. Advances in treatment modalities including lung protective ventilation, prone positioning, use of neuromuscular blockade, and extracorporeal membrane oxygenation, have improved the outcome over recent decades, nevertheless, the mortality rate still remains high. Timely treatment of underlying sepsis and early identification of patients at risk of ARDS can help to decrease its development. In addition, further studies are needed regarding pathogenesis and novel therapies in order to show promising future treatments of sepsis-induced ARDS. PMID:27066082

  6. The acute respiratory distress syndrome: from mechanism to translation.

    PubMed

    Han, SeungHye; Mallampalli, Rama K

    2015-02-01

    The acute respiratory distress syndrome (ARDS) is a form of severe hypoxemic respiratory failure that is characterized by inflammatory injury to the alveolar capillary barrier, with extravasation of protein-rich edema fluid into the airspace. Although many modalities to treat ARDS have been investigated over the past several decades, supportive therapies remain the mainstay of treatment. In this article, we briefly review the definition, epidemiology, and pathophysiology of ARDS and present emerging aspects of ARDS pathophysiology that encompass modulators of the innate immune response, damage signals, and aberrant proteolysis that may serve as a foundation for future therapeutic targets.

  7. [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.

  8. Serial Determinations of Blood Lactate in Respiratory Distress Syndrome

    PubMed Central

    Beca, J. P.; Scopes, J. W.

    1972-01-01

    Blood lactate was measured 4-hourly in 21 newborn babies with respiratory distress syndrome, of whom 13 survived and 8 died. In general, lactate levels were higher in babies who died than in survivors, but there were inconsistencies which were uninterpretable if only a single estimation were made in a baby. Analysis of serial determinations showed that all patients in whom the lactate level never exceeded 35 mg/100 ml survived, and babies with high but falling values also did well. Only those who had rising lactate values, even if initially normal, died. In most cases a high or normal Pao2 was associated with normal or decreasing lactate level; but babies with Pao2 below 60 mmHg had often also normal or decreasing lactate levels. Some babies had high and increasing lactate levels despite having normal Pao2. In order to use lactate levels for prognosis in respiratory distress syndrome (RDS) serial determinations are required. It is concluded that there may be a wide range of hypoxaemia without oxygen deficit in body tissues, so that it is not possible to define a `lower acceptable Pao2' which will define adequate tissue oxygenation. PMID:5046772

  9. Relevant Outcomes in Pediatric Acute Respiratory Distress Syndrome Studies

    PubMed Central

    Yehya, Nadir; Thomas, Neal J.

    2016-01-01

    Despite distinct epidemiology and outcomes, pediatric acute respiratory distress syndrome (PARDS) is often managed based on evidence extrapolated from treatment of adults. The impact of non-pulmonary processes on mortality as well as the lower mortality rate compared to adults with acute respiratory distress syndrome (ARDS) renders the utilization of short-term mortality as a primary outcome measure for interventional studies problematic. However, data regarding alternatives to mortality are profoundly understudied, and proposed alternatives, such as ventilator-free days, may be themselves subject to hidden biases. Given the neuropsychiatric and functional impairment in adult survivors of ARDS, characterization of these morbidities in children with PARDS is of paramount importance. The purpose of this review is to frame these challenges in the context of the existing pediatric literature, and using adult ARDS as a guide, suggest potential clinically relevant outcomes that deserve further investigation. The goal is to identify important areas of study in order to better define clinical practice and facilitate future interventional trials in PARDS. PMID:27242980

  10. [ACUTE RESPIRATORY DISTRESS SYNDROME CAUSED BY BETA-LACTAM ANTIBIOTICS].

    PubMed

    Skipskiy, I M; Efimov, N W; Remizov, A S; Miroshnikov, B I

    2015-01-01

    We report a case of iatrogenic lesion of the lungs in a 61 year old patient following simultaneous subtotal resection and plastic surgery of esophagus using a gastric stem with the removed cardial portion along with subtotal mediastinal lympho-dissection for the treatment of 2 x 1 cm T2NoMO tumour in the middle third of esophagus. The patient's medical history contained no evidence of previous pulmonary pathology, preoperative chest X-ray study revealed neither focal nor infiltrative changes in the lungs. On day 5 after extensive surgical intervention with the use of a parenteral beta-lactam antibiotic, the patient developed acute respiratory distress the symptoms of which increased wavelike during the next 4.5 weeks in association with subfebrility, leukocytosis, enhanced ESR and large shaded areas in the lungs. These conditions were regarded as signs of pneumonia that required the application of increasingly more powerful beta-lactam antibiotics. All these symptoms became less apparent and completely disappeared within 2 days and 1 week respectively after withdrawal of the antibiotics and prescription of parenteral prednisolone. It confirmed the iatrogenic origin of lung lesions regarded by the authors as recurring acute respiratory distress syndrome caused by beta-lactam antibiotics.

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

    PubMed

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

    2013-01-01

    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. PMID:23617961

  12. Haemodynamic changes during high frequency oscillation for respiratory distress syndrome.

    PubMed Central

    Laubscher, B.; van Melle, G.; Fawer, C. L.; Sekarski, N.; Calame, A.

    1996-01-01

    In a crossover trial left ventricular output (LVO), cerebral blood flow velocity (CBFV), and resistance index (RI) of the anterior cerebral artery were compared using Doppler ultrasonography, in eight preterm infants with respiratory distress syndrome (RDS) during conventional mechanical ventilation and high frequency oscillation. LVO was 14% to 18% lower with high frequency oscillation. There were no significant changes in CBFV. On the first day of life there was a trend towards lower RI on high frequency oscillation; the fall in LVO on high frequency oscillation was not related to lung hyperinflation. Changes in ventilation type (from conventional mechanical ventilation to high frequency oscillation, or vice versa) can induce significant LVO changes in preterm infants with RDS. PMID:8777679

  13. [Tracheal phospholipid composition and respiratory distress syndrome of the newborn].

    PubMed

    Obladen, M

    1979-03-01

    Tracheal or pharyngeal aspirates were collected in 50 newborn infants with and without respiratory distress syndrome (RDS). After lipid extraction the phospholipids were analyzed with 2-dimensional thin layer chromatography. Surface-active are lecithin (PC), phosphatidylglycerol (PG), and phosphatidylinositol (PI). Newborn infants with RDS always have a complete lack of PG, which makes up to 11% of phospholipid-phosphors in mature newborns. In all infants with and without RDS, a sharp increase of PC occurs in the lung effluent after birth. The recovery from RDS is characterized by marked changes of PI: this phospholipid rises up to twice its initial value if the infants survive. The PI-increase parallels the clinical improvement and reaches its maximum usually on the 5th day of life. At the time of the PI-peak, the infants' surfactant function is sufficient to maintain alveolar stability with spontaneous breathing. In infants dying from RDS the PI-increase was not observed.

  14. PEEP titration during prone positioning for acute respiratory distress syndrome.

    PubMed

    Beitler, Jeremy R; Guérin, Claude; Ayzac, Louis; Mancebo, Jordi; Bates, Dina M; Malhotra, Atul; Talmor, Daniel

    2015-01-01

    No major trial evaluating prone positioning for acute respiratory distress syndrome (ARDS) has incorporated a high-positive end-expiratory pressure (high-PEEP) strategy despite complementary physiological rationales. We evaluated generalizability of three recent proning trials to patients receiving a high-PEEP strategy. All trials employed a relatively low-PEEP strategy. After protocol ventilator settings were initiated and the patient was positioned per treatment assignment, post-intervention PEEP was not more than 5 cm H2O in 16.7 % and not more than 10 cm H2O in 66.0 % of patients. Post-intervention PEEP would have been nearly twice the set PEEP had a high-PEEP strategy been employed. Use of either proning or high-PEEP likely improves survival in moderate-severe ARDS; the role for both concomitantly remains unknown.

  15. Bronchoalveolar hemostasis in lung injury and acute respiratory distress syndrome.

    PubMed

    Glas, G J; Van Der Sluijs, K F; Schultz, M J; Hofstra, J-J H; Van Der Poll, T; Levi, M

    2013-01-01

    Enhanced intrapulmonary fibrin deposition as a result of abnormal broncho-alveolar fibrin turnover is a hallmark of acute respiratory distress syndrome (ARDS), pneumonia and ventilator-induced lung injury (VILI), and is important to the pathogenesis of these conditions. The mechanisms that contribute to alveolar coagulopathy are localized tissue factor-mediated thrombin generation, impaired activity of natural coagulation inhibitors and depression of bronchoalveolar urokinase plasminogen activator-mediated fibrinolysis, caused by the increase of plasminogen activator inhibitors. There is an intense and bidirectional interaction between coagulation and inflammatory pathways in the bronchoalveolar compartment. Systemic or local administration of anticoagulant agents (including activated protein C, antithrombin and heparin) and profibrinolytic agents (such as plasminogen activators) attenuate pulmonary coagulopathy. Several preclinical studies show additional anti-inflammatory effects of these therapies in ARDS and pneumonia. PMID:23114008

  16. Pharmacotherapy of Acute Lung Injury and Acute Respiratory Distress Syndrome

    PubMed Central

    Raghavendran, Krishnan; Pryhuber, Gloria S.; Chess, Patricia R.; Davidson, Bruce A.; Knight, Paul R.; Notter, Robert H.

    2009-01-01

    Acute lung injury (ALI) and the acute respiratory distress syndrome (ARDS) are characterized by rapid-onset respiratory failure following a variety of direct and indirect insults to the parenchyma or vasculature of the lungs. Mortality from ALI/ARDS is substantial, and current therapy primarily emphasizes mechanical ventilation and judicial fluid management plus standard treatment of the initiating insult and any known underlying disease. Current pharmacotherapy for ALI/ARDS is not optimal, and there is a significant need for more effective medicinal chemical agents for use in these severe and lethal lung injury syndromes. To facilitate future chemical-based drug discovery research on new agent development, this paper reviews present pharmacotherapy for ALI/ARDS in the context of biological and biochemical drug activities. The complex lung injury pathophysiology of ALI/ARDS offers an array of possible targets for drug therapy, including inflammation, cell and tissue injury, vascular dysfunction, surfactant dysfunction, and oxidant injury. Added targets for pharmacotherapy outside the lungs may also be present, since multiorgan or systemic pathology is common in ALI/ARDS. The biological and physiological complexity of ALI/ARDS requires the consideration of combined-agent treatments in addition to single-agent therapies. A number of pharmacologic agents have been studied individually in ALI/ARDS, with limited or minimal success in improving survival. However, many of these agents have complementary biological/biochemical activities with the potential for synergy or additivity in combination therapy as discussed in this article. PMID:18691048

  17. Acute respiratory distress syndrome associated with tumor lysis syndrome in a child with acute lymphoblastic leukemia.

    PubMed

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

    2015-02-24

    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

  18. 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

  19. 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.

  20. Adenovirus Pneumonia Complicated With Acute Respiratory Distress Syndrome

    PubMed Central

    Hung, Ka-Ho; Lin, Lung-Huang

    2015-01-01

    Abstract Severe adenovirus infection in children can manifest with acute respiratory distress syndrome (ARDS) and respiratory failure, leading to the need for prolonged mechanical support in the form of either mechanical ventilation or extracorporeal life support. Early extracorporeal membrane oxygenation (ECMO) intervention for children with ARDS should be considered if selection criteria fulfill. We report on a 9-month-old boy who had adenovirus pneumonia with rapid progression to ARDS. Real-time polymerase chain reaction tests of sputum and pleural effusion samples confirmed adenovirus serotype 7. Chest x-rays showed progressively increasing infiltrations and pleural effusions in both lung fields within 11 days. Because conventional ARDS therapies failed, we initiated ECMO with high-frequency oscillatory ventilation (HFOV) for 9 days. Chest x-rays showed gradual improvements in lung expansion. This patient was subsequently discharged after a hospital stay of 38 days. Post-ECMO and adenovirus sequelae were followed in our outpatient department. Adenovirus pneumonia in children can manifest with severe pulmonary morbidity and respiratory failure. The unique lung recruitment by HFOV can be a useful therapeutic option for severe ARDS patients when combined with sufficient lung rest provided by ECMO. PMID:25997046

  1. 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

  2. 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

  3. Noninvasive ventilation on mortality of acute respiratory distress syndrome.

    PubMed

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

    2016-08-01

    [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

  4. Extracorporeal lung assist for sepsis and acute respiratory distress syndrome.

    PubMed

    Iwashita, Yoshiaki; Imai, Hiroshi

    2015-01-01

    Acute respiratory distress syndrome (ARDS) is one of the major causes of ICU deaths. Extracorporeal lung assist (ECLA) has been used as a rescue therapy for most severe form of ARDS. However, its survival benefit had not been shown until CESAR trial in 2009. This has been because the concept of lung protective ventilation strategy had not yet known. Since CESAR trial, the clinical application of ECLA for ARDS as a method to achieve lung rest has wide spread. The effectiveness is further appreciated during the 2009 H1N1 influenza pandemic. The succeeded countries achieved building the transportation systems to collect ECLA patients. With the accumulating evidences of survival benefit, the long-term outcome such as pulmonary function and quality of life are in concern. PumplessECLA which is a newly developed form of ECLA is also reviewed. In this essay we will firstly review the basics of ARDS and ECLA. Then the historical development of ECLA evidences for ARDS are reviewed. PMID:25567336

  5. Acute Respiratory Distress Syndrome as the Initial Clinical Manifestation of an Antisynthetase Syndrome.

    PubMed

    Kim, Seo-Hyun; Park, I-Nae

    2016-07-01

    Antisynthetase syndrome has been recognized as an important cause of autoimmune inflammatory myopathy in a subset of patients with polymyositis and dermatomyositis. It is associated with serum antibody to aminoacyl-transfer RNA synthetases and is characterized by a constellation of manifestations, including fever, myositis, interstitial lung disease, mechanic's hand-like cutaneous involvement, Raynaud phenomenon, and polyarthritis. Lung disease is the presenting feature in 50% of the cases. We report a case of a 60-year-old female with acute respiratory distress syndrome (ARDS), which later proved to be an unexpected and initial manifestation of anti-Jo-1 antibody-positive antisynthetase syndrome. The present case showed resolution of ARDS after treatment with high-dose corticosteroids. Given that steroids are not greatly beneficial in the treatment of ARDS, it is likely that the improvement of the respiratory symptoms in this patient also resulted from the prompt suppression of the inflammatory systemic response by corticosteroids. PMID:27433180

  6. Acute Respiratory Distress Syndrome as the Initial Clinical Manifestation of an Antisynthetase Syndrome

    PubMed Central

    Kim, Seo-Hyun

    2016-01-01

    Antisynthetase syndrome has been recognized as an important cause of autoimmune inflammatory myopathy in a subset of patients with polymyositis and dermatomyositis. It is associated with serum antibody to aminoacyl-transfer RNA synthetases and is characterized by a constellation of manifestations, including fever, myositis, interstitial lung disease, mechanic's hand-like cutaneous involvement, Raynaud phenomenon, and polyarthritis. Lung disease is the presenting feature in 50% of the cases. We report a case of a 60-year-old female with acute respiratory distress syndrome (ARDS), which later proved to be an unexpected and initial manifestation of anti-Jo-1 antibody–positive antisynthetase syndrome. The present case showed resolution of ARDS after treatment with high-dose corticosteroids. Given that steroids are not greatly beneficial in the treatment of ARDS, it is likely that the improvement of the respiratory symptoms in this patient also resulted from the prompt suppression of the inflammatory systemic response by corticosteroids. PMID:27433180

  7. [Acute respiratory distress syndrome caused by tropical eosinophilic lung disease: a case in Gabon].

    PubMed

    Chani, M; Iken, M; Eljahiri, Y; Nzenze, J R; Mion, G

    2011-04-01

    The purpose of this report is to describe the case of a 28-year-old woman in whom acute respiratory distress syndrome (ARDS) following cholecystectomy led to the discovery of eosinophilic lung disease. Outcome was favorable after oxygenotherapy and medical treatment using ivermectin and corticosteroids. The case shows that hypereosinophilic syndrome can be the underlying cause of ARDS. PMID:21695880

  8. Pediatric Acute Respiratory Distress Syndrome: Fibrosis versus Repair

    PubMed Central

    Im, Daniel; Shi, Wei; Driscoll, Barbara

    2016-01-01

    Clinical and basic experimental approaches to pediatric acute lung injury (ALI), including acute respiratory distress syndrome (ARDS), have historically focused on acute care and management of the patient. Additional efforts have focused on the etiology of pediatric ALI and ARDS, clinically defined as diffuse, bilateral diseases of the lung that compromise function leading to severe hypoxemia within 7 days of defined insult. Insults can include ancillary events related to prematurity, can follow trauma and/or transfusion, or can present as sequelae of pulmonary infections and cardiovascular disease and/or injury. Pediatric ALI/ARDS remains one of the leading causes of infant and childhood morbidity and mortality, particularly in the developing world. Though incidence is relatively low, ranging from 2.9 to 9.5 cases/100,000 patients/year, mortality remains high, approaching 35% in some studies. However, this is a significant decrease from the historical mortality rate of over 50%. Several decades of advances in acute management and treatment, as well as better understanding of approaches to ventilation, oxygenation, and surfactant regulation have contributed to improvements in patient recovery. As such, there is a burgeoning interest in the long-term impact of pediatric ALI/ARDS. Chronic pulmonary deficiencies in survivors appear to be caused by inappropriate injury repair, with fibrosis and predisposition to emphysema arising as irreversible secondary events that can severely compromise pulmonary development and function, as well as the overall health of the patient. In this chapter, the long-term effectiveness of current treatments will be examined, as will the potential efficacy of novel, acute, and long-term therapies that support repair and delay or even impede the onset of secondary events, including fibrosis. PMID:27066462

  9. The Acute Respiratory Distress Syndrome: Mechanisms and Perspective Therapeutic Approaches

    PubMed Central

    Gonzales, JN; Lucas, R; Verin, AD

    2015-01-01

    Acute Respiratory Distress Syndrome (ARDS) is a severe lung inflammatory disorder with a 30–50% mortality. Sepsis and pneumonia are the leading causes of ARDS. On the cellular level there is pulmonary capillary endothelial cell permeability and fluid leakage into the pulmonary parenchyma, followed by neutrophils, cytokines and an acute inflammatory response. When fluid increases in the interstitium then the outward movement continues and protein rich fluid floods the alveolar spaces through the tight junctions of the epithelial cells. Neutrophils play an important role in the development of pulmonary edema associated with acute lung injury or ARDS. Animal studies have shown that endothelial injury appears within minutes to hours after Acute Lung Injury (ALI) initiation with resulting intercellular gaps of the endothelial cells. The Endothelial Cell (EC) gaps allow for permeability of fluid, neutrophils and cytokines into the pulmonary parenchymal space. The neutrophils that infiltrate the lungs and migrate into the airways express pro-inflammatory cytokines such as tumor necrosis factor-alpha (TNF-α), interleukin-1 beta (IL-1β), and contribute to both the endothelial and epithelial integrity disruption of the barriers. Pharmacological treatments have been ineffective. The ARDS Network trial identified low tidal volume mechanical ventilation, positive end expiratory pressure and fluid management guidelines that have improved outcomes for patients with ARDS. Extracorporeal membrane oxygenation is used in specialized centers for severe cases. Prone positioning has recently proven to have significantly decreased ventilator days and days in the intensive care unit. Current investigation includes administration of mesenchymal stem cell therapy, partial fluid ventilation, TIP peptide nebulized administration and the continued examination of pharmacologic drugs. PMID:26973981

  10. 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

  11. Incidence and Outcomes of Acute Respiratory Distress Syndrome

    PubMed Central

    Chen, Wei; Chen, Yih-Yuan; Tsai, Ching-Fang; Chen, Solomon Chih-Cheng; Lin, Ming-Shian; Ware, Lorraine B.; Chen, Chuan-Mu

    2015-01-01

    Abstract Most epidemiological studies of acute respiratory distress syndrome (ARDS) have been conducted in western countries, and studies in Asia are limited. The aim of our study was to evaluate the incidence, in-hospital mortality, and 1-year mortality of ARDS in Taiwan. We conducted a nationwide inpatient cohort study based on the Taiwan National Health Insurance Research Database between 1997 and 2011. A total of 40,876 ARDS patients (68% male; mean age 66 years) were identified by International Classification of Diseases, 9th edition coding and further analyzed for clinical characteristics, medical costs, and mortality. The overall crude incidence of ARDS was 15.74 per 100,000 person-years, and increased from 2.53 to 19.26 per 100,000 person-years during the study period. The age-adjusted incidence of ARDS was 15.19 per 100,000 person-years. The overall in-hospital mortality was 57.8%. In-hospital mortality decreased from 59.7% in 1997 to 47.5% in 2011 (P < 0.001). The in-hospital mortality rate was lowest (33.5%) in the youngest patients (age 18–29 years) and highest (68.2%) in the oldest patients (>80 years, P < 0.001). The overall 1-year mortality rate was 72.1%, and decreased from 75.8% to 54.7% during the study period. Patients who died during hospitalization were older (69 ± 17 versus 62 ± 19, P < 0.001) and predominantly male (69.8% versus 65.3%, P < 0.001). In addition, patients who died during hospitalization had significantly higher medical costs (6421 versus 5825 US Dollars, P < 0.001) and shorter lengths of stay (13 versus 19 days, P < 0.001) than patients who survived. We provide the first large-scale epidemiological analysis of ARDS incidence and outcomes in Asia. Although the overall incidence was lower than has been reported in a prospective US study, this may reflect underdiagnosis by International Classification of Diseases, 9th edition code and identification of only patients with more severe ARDS in this

  12. Severe Acute Infection Due to Serratia marcescens Causing Respiratory Distress in An Immunocompetent Adult.

    PubMed

    Ruiz-Sada, Pablo; Escalante, Mikel; Lizarralde, Eva

    2016-01-01

    The role of Serratia marcescens changed from a harmless saprophytic microorganism to an important opportunistic human pathogen. It often causes nosocomial device-associated outbreaks and rarely serious invasive community acquired infections. We present a case of a community-acquired Serratia marcescens bacteremia leading to Respiratory Distress Syndrome in a previously healthy 51-year-old man without identifiable risk factors. Full recovery was achieved with solely medical treatment and observation in ICU during three days. To our knowledge it is an extremely uncommon presentation and just few cases have been previously reported in the literature.

  13. Vibrio vulnificus infection complicated by acute respiratory distress syndrome in a child with nephrotic syndrome.

    PubMed

    Wang, S M; Liu, C C; Chiou, Y Y; Yang, H B; Chen, C T

    2000-05-01

    A 9-year-old girl with nephrotic syndrome visited a local hospital after developing fever, chills, and edematous changes and multiple hemorrhagic bullae on both legs over 2 days. Cultures of blood and an aspirate from the bullae yielded Vibrio vulnificus. The patient was transferred to our hospital because of persistent fever, generalized edema, acute renal failure, and disseminated intravascular coagulopathy. We treated this patient as a V. vulnificus infection complicated with necrotizing fasciitis. With minocycline and ceftazidime combination therapy was instituted. Emergency fasciotomy and continuous peritoneal dialysis were performed. The patient developed acute respiratory distress syndrome (ARDS) during the hospitalization, requiring intubation and mechanical ventilation. She eventually died. The histopathological findings showed diffuse alveolar damage with lobular pneumonitis. Hyaline membranes, composed of proteinaceous exudate and cellular debris, covered the alveolar surfaces. Microscopic examinations of lung could not distinguish the effects of cytolysin from other insults to lungs that occur in ARDS. This report highlights the postmortem pathological findings in V. vulnificus infection in a child with nephrotic syndrome complicated by ARDS.

  14. 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

  15. High or conventional positive end-expiratory pressure in adult respiratory distress syndrome.

    PubMed

    Díaz-Alersi, R; Navarro-Ramírez, C

    2014-01-01

    Patients with acute respiratory distress syndrome may require high positive end-expiratory pressure (PEEP) levels, though the optimum level remains to be established. Several clinical trials have compared high PEEP levels versus conventional PEEP. Overall, although high PEEP levels improve oxygenation and are safe, they do not result in a significant reduction of the mortality rates. Nevertheless, some metaanalyses have revealed 2 situations in which high PEEP may decrease mortality: When used in severe distress and when PEEP is set following the characteristics of lung mechanics. Five studies have explored this latter scenario. Unfortunately, all of them have small sample sizes and have used different means to determine optimum PEEP. It is therefore necessary to conduct studies of sufficient sample size to compare the treatment of patients with severe acute respiratory distress syndrome, using a protective ventilation strategy with high PEEP guided by the characteristics of lung mechanics and ventilation with the protocol proposed by the ARDS Network.

  16. Organophosphate poisoning complicated by a tachyarrhythmia and acute respiratory distress syndrome in a child.

    PubMed

    Nel, L; Hatherill, M; Davies, J; Andronikou, S; Stirling, J; Reynolds, L; Argent, A

    2002-10-01

    A 9-year-old child presented with documented organophosphate insecticide poisoning. His course was initially complicated by a tachyarrhythmia with QT-interval prolongation that responded promptly to intravenous magnesium. However, following partial recovery, he developed progressive acute respiratory distress syndrome characterized by irreversible fibrosis and obliteration of the lung parenchyma. PMID:12354276

  17. [Acute respiratory distress syndrome after near-drowning (author's transl)].

    PubMed

    Tempel, G; Jelen, S; Forster, B; Gullotta, U; Daum, S

    1977-08-01

    After successful rescue from drowning there may develop a situation which is called secondary drowning, resulting in acute respiratory distress characterized by interstitial pulmonary oedema, hypoxaemia, hypercapnia and acidosis during drowning, direct alteration of the alveolar membrane by aspirated water and particulate matters and a volume overloading by adsorption and--not seldom--inept therapy. This situation requires mechanical ventilation and forced diuresis, combined with high doses of steroids, antibiotics and digitalis. We present the case of an eleven year old patient whose clinical course demonstrate the necessity of exact clinical observation after rescue from drowning. After development of acute respiratory distress only the immediate utilization of the therapeutic modalities of an intensive care may result in a satisfactory outcome. Four months later our patient had normal pulmonary function except for a moderate reduction of compliance.

  18. Emerging therapies for treatment of acute lung injury and acute respiratory distress syndrome.

    PubMed

    Bosma, Karen J; Lewis, James F

    2007-09-01

    Acute lung injury/acute respiratory distress syndrome (ALI/ARDS) is a life-threatening form of respiratory failure that affects a heterogeneous population of critically ill patients. Although overall mortality appears to be decreasing in recent years due to improvements in supportive care, there are presently no proven, effective pharmacological therapies to treat ARDS and prevent its associated complications. The most common cause of death in ARDS is not hypoxemia or pulmonary failure, but rather multiple organ dysfunction syndrome (MODS), suggesting that improving survival in patients with ARDS may be linked to decreasing the incidence or severity of MODS. The key to developing novel treatments depends, in part, on identifying and understanding the mechanisms by which ARDS leads to MODS, although the heterogeneity and complexity of this disorder certainly poses a challenge to investigators. Novel therapies in development for treatment of ALI/ARDS include exogenous surfactant, therapies aimed at modulating neutrophil activity, such as prostaglandin and complement inhibitors, and treatments targeting earlier resolution of ARDS, such as beta-agonists and granulocyte macrophage colony-stimulating factor. From a clinical perspective, identifying subpopulations of patients most likely to benefit from a particular therapy and recognising the appropriate stage of illness in which to initiate treatment could potentially lead to better outcomes in the short term.

  19. Exogenous surfactant therapy in a patient with adult respiratory distress syndrome after near drowning.

    PubMed

    Staudinger, T; Bankier, A; Strohmaier, W; Weiss, K; Locker, G J; Knapp, S; Röggla, M; Laczika, K; Frass, M

    1997-10-01

    A 24-year-old woman developed adult respiratory distress syndrome (ARDS) after near-drowning due to attempted suicide. Conventional mechanical ventilation together with prone positioning and inhaled nitric oxide could not provide sufficient oxygenation. Surface tension data (gamma min = 27 dyn/cm, stability index = 0.341) from a lavage sample supported the hypothesis that the surfactant function of this patient was drastically reduced due to a washout effect by aspiration of fresh water. Porcine surfactant (Curosurf, 50 mg/kg for each lung) was instilled via fibreoptic bronchoscope. The partial arterial carbon dioxide pressure (paCO2) and fraction of inspired oxygen (FiO2) ratio as well as shunt fraction (Qs/Qt) improved impressively. When respiratory situation deteriorated again, surfactant application was repeated. Altogether, six bolus instillations of surfactant (total dose 300 mg/kg = 18,000 mg) were administered until the respiratory situation had stabilized and oxygenation could be maintained by conventional mechanical ventilation. The radiological findings did not show substantial amelioration. The patient developed septic shock and died 12 days after admission. Surfactant application apparently led to a significant improvement of the respiratory function. However, the outcome could not be influenced positively. The high cost of surfactant therapy prevents the more widespread early administration in patients at risk.

  20. Coping and psychological distress of Chinese parents of children with Down syndrome.

    PubMed

    Cheng, P; Tang, C S

    1995-02-01

    Coping and correlates of psychological distress of Chinese parents of children with Down syndrome were examined and compared to parents of children with language delays or no disabilities. Individual parent scores were used for analyses, with groups and gender of child and parent as independent factors. Down syndrome group parents reported the most frequent use of avoidance coping style, followed by parents of the language delay and no disabilities groups. Compared to parents in the no disabilities group, the other parents reported a higher level of psychological distress, were less optimistic, felt less self-efficacious, and engaged in more frequent use of self-reliance coping style. Main effects for parent gender revealed that mothers engaged in more frequent use of self-reliance, avoidance, and seeking social support coping styles. Mothers also reported a higher level of distress but lower levels of optimism and self-mastery. Intercorrelations among variables showed that avoidance coping style and self-mastery emerged as the two robust correlates of parental distress.

  1. Recruitment manoeuvres in acute respiratory distress syndrome: Little evidence for routine use.

    PubMed

    Poole, Oliver

    2013-01-01

    The use of alveolar recruitment manoeuvres for the treatment of acute respiratory distress syndrome is a topic of uncertainty in current critical care practice. Acute respiratory distress syndrome leads to inflammatory atelectasis, which challenges the gas exchange properties of the lung. Recruitment of atelectatic lung tissue requires elevation of transpulmonary pressure. Transpulmonary pressure can be suppressed at a given airway pressure when pleural pressures are elevated. The present review discusses recruitment of lung tissue in detail, highlighting the key research in the field. Differing techniques for recruiting lung tissue, as well as various outcome measures to determine efficacy, are analyzed and critiqued. The commonly used sustained inflation manoeuvre is perhaps regarded as the only strategy to recruit the lung, explaining its prevalence. Staircase recruitment with positive end-expiratory pressure titration is shown to be an equally - if not more - effective therapy that devotes attention to the maintenance of lung recruitment.

  2. 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.

  3. [A case of respiratory distress syndrome complicated by the development of interstitial emphysema and pneumomediastinum].

    PubMed

    Zhidkov, K P; Bogatyr', M N; Chezhin, A N; Leĭman, V A; Smirnova, O R

    2000-01-01

    A 15-year-old patient has been admitted to the intensive care unit for severe respiratory distress syndrome that developed as a result of pneumonia. Interstitial lung edema was confirmed by computer-aided tomography. It was successfully treated by positive pressure ventilation (PPV). Although PEEP did not exceed 7 cm H2O, PPV was complicated by interstitial emphysema, pneumomediastinum, and bilateral pneumothorax as a result of barotrauma. Pulmonary artery pressure (PAP) and pulmonary capillary wedge pressure (PCWP) were monitored. High PCWP values were inconsistent with the diagnosis of acute respiratory distress syndrome. The authors suggest that high PCWP was caused by high intraalveolar pressure, pneumomediastinum, and venule constriction in the hypoxic sites of the lung.

  4. 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. PMID:24485531

  5. Pulmonary Alveolar Type II Epithelial Cells and Adult Respiratory Distress Syndrome

    PubMed Central

    Mason, Robert J.

    1985-01-01

    During the past ten years, functions of alveolar type II cells have been well characterized with isolated cells in vitro. Some of the functions were well known from studies in vivo, but others such as transepithelial sodium transport were unsuspected. A better understanding of this important pulmonary cell type improves our knowledge of the pathophysiology of adult respiratory distress syndrome and may in time lead to new therapeutic strategies. ImagesFigure 1.Figure 2.Figure 3.Figure 4. PMID:3909639

  6. Acute respiratory distress syndrome: use of specialized nutrients in pediatric patients and infants.

    PubMed

    Hamilton, Leslie A; Trobaugh, Kimberly A

    2011-02-01

    With a high rate of mortality, acute respiratory distress syndrome (ARDS) has limited treatments options. Immune-enhanced formulas, containing eicosapentaenoic acid, borage oil, and antioxidants, have shown to be beneficial in adults patients with ARDS, decreasing mortality, length of mechanical ventilation, and new organ dysfunction. There is promising research in pediatric patients with improvement in oxygenation status found, but further trials are needed to realize these benefits in pediatric and infant populations. PMID:21266694

  7. [Pneumomediastinum: an aspect of pulmonary barotrauma during mechanical ventilation of acute respiratory distress syndrome].

    PubMed

    Aissaoui, Y; En-Nafaa, I; Chkoura, K; Boughalem, M; Kamili, N Drissi

    2014-06-01

    Mechanical ventilation is a fundamental treatment of acute respiratory distress syndrome (ARDS). Despite compliance with the recommendations of protective mechanical ventilation, it can results in serious complications including the pulmonary barotrauma. This is often manifested by a pneumothorax. This observation describes an unusual aspect of barotrauma which is pneumomediastinum. The authors also point out the role of chest imaging in the management of mechanical ventilation during ARDS.

  8. Venovenous Extracorporeal Life Support in Single-Ventricle Patients with Acute Respiratory Distress Syndrome

    PubMed Central

    Nair, Alison B.; Oishi, Peter

    2016-01-01

    There is new and growing experience with venovenous extracorporeal life support (VV ECLS) for neonatal and pediatric patients with single-ventricle physiology and acute respiratory distress syndrome (ARDS). Outcomes in this population have been defined but could be improved; survival rates in single-ventricle patients on VV ECLS for respiratory failure are slightly higher than those in single-ventricle patients on venoarterial ECLS for cardiac failure (48 vs. 32–43%), but are lower than in patients with biventricular anatomy (58–74%). To that end, special consideration is necessary for patients with single-ventricle physiology who require VV ECLS for ARDS. Specifically, ARDS disrupts the balance between pulmonary and systemic blood flow through dynamic alterations in cardiopulmonary mechanics. This complexity impacts how to run the VV ECLS circuit and the transition back to conventional support. Furthermore, these patients have a complicated coagulation profile. Both venous and arterial thrombi carry marked risk in single-ventricle patients due to the vulnerability of the pulmonary, coronary, and cerebral circulations. Finally, single-ventricle palliation requires the preservation of low resistance across the pulmonary circulation, unobstructed venous return, and optimal cardiac performance including valve function. As such, the proper timing as well as the particular conduct of ECLS might differ between this population and patients without single-ventricle physiology. The goal of this review is to summarize the current state of knowledge of VV ECLS in the single-ventricle population in the context of these special considerations. PMID:27446889

  9. Submersion and early-onset acute respiratory distress syndrome: a case report.

    PubMed

    Diamond, Wayde; MacDonald, Russell D

    2011-01-01

    Drowning is a common cause of accidental death, particularly in younger people, and acute respiratory failure is common in these patients. This case report describes a healthy 18-year-old man who suffered a cardiorespiratory arrest due to submersion while swimming in a freshwater lake. First-responder cardiopulmonary resuscitation and defibrillation using an automated external defibrillator resulted in a return of spontaneous circulation. The patient was evacuated to a tertiary care center by a rotor-wing air medical crew. The crew experienced difficulties in oxygenating and ventilating the patient because of early-onset acute respiratory distress syndrome (ARDS). This case report describes the pathophysiology and prehospital management of a patient with suspected early-onset ARDS secondary to drowning. This case report is unique because it describes the oxygenation and ventilation difficulties encountered in managing this patient in the transport setting, and possible strategies to deal with these difficulties. Finally, this case report highlights the prehospital bypass decision-making process for patients requiring specialized medical care.

  10. Surfactants in the Management of Respiratory Distress Syndrome in Extremely Premature Infants

    PubMed Central

    Ramanathan, Rangasamy

    2006-01-01

    Respiratory distress syndrome (RDS) is primarily due to decreased production of pulmonary surfactant, and it is associated with significant neonatal morbidity and mortality. Exogenous pulmonary surfactant therapy is currently the treatment of choice for RDS, as it demonstrates the best clinical and economic outcomes. Studies confirm the benefits of surfactant therapy to include reductions in mortality, pneumothorax, and pulmonary interstitial emphysema, as well as improvements in oxygenation and an increased rate of survival without bronchopulmonary dysplasia. Phospholipids (PL) and surfactant-associated proteins (SP) play key roles in the physiological activity of surfactant. Different types of natural and synthetic surfactant preparations are currently available. To date, natural surfactants demonstrate superior outcomes compared to the synthetic surfactants, at least during the acute phase of RDS. This disparity is often attributed to biochemical differences including the presence of surfactant-associated proteins in natural products that are not found in the currently available synthetic surfactants. Comparative trials of the natural surfactants strive to establish the precise differences in clinical outcomes among the different preparations. As new surfactants become available, it is important to evaluate them relative to the known benefits of the previously existing surfactants. In order to elucidate the role of surfactant therapy in the management of RDS, it is important to review surfactant biochemistry, pharmacology, and outcomes from randomized clinical trials. PMID:23118650

  11. Recruitment maneuvers in acute respiratory distress syndrome and during general anesthesia.

    PubMed

    Chiumello, Davide; Algieri, Ilaria; Grasso, Salvatore; Terragni, Pierpaolo; Pelosi, Paolo

    2016-02-01

    The use of low tidal volume ventilation and low to moderate positive end-expiratory pressure (PEEP) levels is a widespread strategy to ventilate patients with non-injured lungs during general anesthesia and in intensive care as well with mild to moderate acute respiratory distress syndrome (ARDS). Higher PEEP levels have been recommended in severe ARDS. Due to the presence of alveolar collapse, recruitment maneuvers (RMs) by causing a transient elevation in airway pressure (i.e. transpulmonary pressure) have been suggested to improve lung inflation in non-inflated and poorly-inflated lung regions. Various types of RMs such as sustained inflation at high pressure, intermittent sighs and stepwise increases of PEEP and/or airway plateau inspiratory pressure have been proposed. The use of RMs has been associated with mixed results in terms of physiological and clinical outcomes. The optimal method for RMs has not yet been identified. The use of RMs is not standardized and left to the individual physician based on his/her experience. Based on the same grounds, RMs have been proposed to improve lung aeration during general anesthesia. The aim of this review was to present the clinical evidence supporting the use of RMs in patients with ARDS and during general anesthesia and as well their potential biological effects in experimental models of acute lung injury.

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

    PubMed

    Repessé, Xavier; Aubry, Alix; Vieillard-Baron, Antoine

    2016-08-01

    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

  13. 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

  14. 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.

  15. [Cushing syndrome due to ectopic ACTH secretion].

    PubMed

    Mendonça, B B; Madureira, G; Bloise, W; Albergaria, A; Halpern, A; Liberman, B; Villares, S M; Batista, M C; Avancini, V F; Nitterdorfi, C T

    1989-01-01

    The authors studied 8 patients (4 males and 4 females) with Cushing's syndrome due to ectopic ACTH secretion. Chronological age ranged from 15 to 45 years and duration of the disease ranged from 3 to 48 months. All patients presented typical signs of Cushing's syndrome, blood hypertension, and four of them had hyperpigmentation of the skin. Five patients had fasting hyperglycemia and all patients but one had serum hypokalemia (serum K = 2.2 to 3.9mEq/l). The circadian rhythm of cortisol was absent in all patients and basal cortisol levels were elevated in all patients but one. Basal ACTH levels evaluated in 7 patients were elevated in 6 (29 to 1050 pg/ml-MRC). One patient presented normal depression of urinary 17-OH after two days of dexamethasone and normal increase of urinary 17-OH and serum 11-dexycortisol after methyrapone. Four patients had carcinoid tumor (3 thymic and 1 bronchial), two had pancreatic islets cell tumors, one had bilateral pheochromocytoma and medular carcinoma of the thyroid, and one had oat cell carcinoma of the lung and medular carcinoma of the thyroid. Thoracic X-rays identified the ectopic ACTH secretion tumor in four cases, all confirmed by CT scan. Abdominal CT showed a difuse enlargement of the adrenals in seven cases and bilateral nodules in one case (pheochromocytomas). Six patients died within 3 years of the diagnosis. The authors concluded that clinical and hormonal findings could mislead the findings of ACTH ectopic secretion and Cushing's disease, and suggest that thoracic X-rays and CT scans of the skull, thorax, and abdome should be done in all cases of Cushing's syndrome. PMID:2559451

  16. Cushing syndrome due to adrenal tumor

    MedlinePlus

    Adrenal tumor - Cushing syndrome ... Cushing syndrome is a disorder that occurs when your body has a higher than normal level of the ... or cancerous (malignant). Noncancerous tumors that can cause ... Adrenal adenomas Micronodular hyperplasia Cancerous tumors that ...

  17. Approach to the Patient with the Acute Respiratory Distress Syndrome

    PubMed Central

    Janz, David R; Ware, Lorraine B

    2014-01-01

    Given the high incidence and mortality of ARDS in critically ill patients, every practitioner needs a bedside approach both for early identification of patients at risk for ARDS and for the appropriate evaluation of patients who meet the diagnostic criteria of ARDS. Recent advances such as the Lung Injury Prediction Score, the Early Acute Lung Injury score, and validation of the SpO2/FiO2 ratio for assessing the degree of hypoxemia are all practical tools to aid the practitioner in caring for patients at risk of ARDS and will likely become more important in the future as more preventative therapies for ARDS are investigated. For patients who meet the diagnostic criteria for ARDS, the practitioner should focus on a thorough search for an underlying cause as well as the concurrent possibility of an underlying disease process that mimics the clinical syndrome of ARDS. PMID:25453418

  18. Cushing's syndrome due to ectopic ACTH secretion.

    PubMed

    Cieszyński, Łukasz; Berendt-Obołończyk, Monika; Szulc, Michał; Sworczak, Krzysztof

    2016-01-01

    Cushing's syndrome (CS) is defined as a constellation of clinical signs and symptoms occurring due to hypercortisolism. Cortisol excess may be endogenous or exogenous. The most common cause of CS is glucocorticoid therapy with supraphysiological (higher than in the case of substitution) doses used in various diseases (e.g. autoimmune). One possible CS cause is ectopic (extra-pituitary) ACTH secretion (EAS) by benign or malignant tumours. Since its first description in 1963, EAS aetiology has changed, i.e. as well as small cell lung cancer (SCLC), higher incidence in other malignancies has been reported. Ectopic ACTH secretion symptoms are usually similar to hypercortisolism symptoms due to other causes. A clinical suspicion of CS requires laboratory investigations. There is no single and specific laboratory test for making a CS diagnosis, and therefore multiple dynamic tests should be ordered. A combination of multiple laboratory noninvasive and invasive tests gives 100% sensitivity and 98% specificity for EAS diagnosis. If the EAS is caused by localised malignancy, surgery is the optimal treatment choice. Radical tumour excision may be performed in 40% of patients, and 80% of them are cured of the disease. The authors present an interesting clinical case of EAS, which is always a huge diagnostic challenge for clinicians. (Endokrynol Pol 2016; 67 (4): 458-464). PMID:27387249

  19. 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

  20. Disseminated Cryptococcal Infection Resulting in Acute Respiratory Distress Syndrome (ARDS) as the Initial Clinical Presentation of AIDS.

    PubMed

    Orsini, Jose; Blaak, Christa; Tam, Eric; Rajayer, Salil; Morante, Joaquin

    2016-01-01

    Cryptococcosis is a cosmopolitan but rare opportunistic mycosis which is usually caused by Cryptococcus neoformans. Although the most common and worrisome disease manifestation is meningoencephalitis, pulmonary cryptococcosis has the potential to be lethal. The diagnosis of cryptococcal pneumonia is challenging, given its non-specific clinical and radiographic features. Respiratory failure leading to acute respiratory distress syndrome as a consequence of cryptococcal disease has been infrequently addressed in the literature. We herein present a case of disseminated cryptococcal infection leading to acute respiratory distress syndrome, refractory shock, and multiorgan dysfunction as the initial clinical manifestation in a patient who was newly diagnosed with acquired immunodeficiency syndrome. PMID:27086819

  1. [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.

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

    PubMed

    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

  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. Can We Optimize Long-Term Outcomes in Acute Respiratory Distress Syndrome by Targeting Normoxemia?

    PubMed Central

    Anderson, Brian; Christie, Jason D.; Hopkins, Ramona O.; Lanken, Paul N.

    2014-01-01

    Since its original description in 1967, acute respiratory distress syndrome (ARDS) has been recognized as a devastating condition associated with significant morbidity and mortality. Advances in critical care medicine and ARDS management have led to a substantial increase in the number of ARDS survivors. Long-term cognitive impairment after critical illness is a significant public health concern. ARDS survivors frequently experience long-term cognitive impairment, as well as physical impairment, psychiatric morbidity, and reduced health-related quality of life. At present, no intensive care unit–based intervention has been proven to reduce the risk of long-term cognitive impairment after ARDS. To address the urgent need to identify strategies to preserve long-term health, investigators have advocated the measurement of short- and long-term outcomes in clinical trials. Maintaining adequate oxygen delivery to preserve organ function is of vital importance in ARDS management. The optimal target range for arterial oxygenation in ARDS remains unknown, due in part to the challenge to maintain adequate tissue oxygenation and to minimize harm, such as oxygen toxicity. An approach targeted to subnormal oxygenation values (partial pressure of arterial oxygen, 55–80 mm Hg) has emerged as a means to accomplish these aims. In this perspective, we critically evaluate this strategy from short- and long-term perspectives, with a focus on the potential long-term cognitive effects of the strategy. We conclude with a proposal to consider resetting the target range for arterial oxygenation higher (85–110 mm Hg) as a potential strategy to improve the long-term outcomes of ARDS survivors. PMID:24621125

  5. 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

  6. 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. PMID:15194566

  7. 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.

  8. 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. PMID:27418297

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

    PubMed

    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

  10. 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

  11. Acute respiratory distress syndrome caused by Mycoplasma pneumoniae without elevated pulmonary vascular permeability: a case report

    PubMed Central

    Takahashi, Naoki; Oi, Rie; Ota, Muneyuki; Toriumi, Shinichi; Ogushi, Fumitaka

    2016-01-01

    Sporadic patients with acute respiratory distress syndrome (ARDS) caused by Mycoplasma pneumoniae have been reported. However, knowledge about the pathophysiology and pharmacological treatment of this condition is insufficient. Moreover, the pulmonary vascular permeability in ARDS related to M. pneumoniae infection has not been reported. We report a case of ARDS caused by Mycoplasma pneumoniae without elevated pulmonary vascular permeability, which was successfully treated using low-dose short-term hydrocortisone, suggesting that pulmonary infiltration in ARDS caused by Mycoplasma pneumoniae does not match the criteria of permeability edema observed in typical ARDS. PMID:27162691

  12. Clinical trial design in prevention and treatment of acute respiratory distress syndrome.

    PubMed

    Curley, Gerard F; McAuley, Daniel F

    2014-12-01

    Our ability to define appropriate molecular targets for preclinical development and develop better methods needs to be improved, to determine the clinical value of novel acute respiratory distress syndrome (ARDS) agents. Clinical trials must have realistic sample sizes and meaningful end points and use the available observation and meta-analytical data to inform design. Biomarker-driven studies or defined ARDS subsets should be considered to categorize specific at-risk populations most likely to benefit from a new treatment. Innovations in clinical trial design should be pursued to improve the outlook for future interventional trials in ARDS.

  13. [Liver monooxygenase system inducers in the treatment of respiratory distress syndrome in newborn].

    PubMed

    Kabulov, Sh M

    2002-03-01

    Physical and biochemical parameters of pulmonary surfactant (PS) were studied in 6-day-old rabbits with the respiratory distress syndrome (RDS) treated by benzonal and zixorin inductors. Surface-active characteristics of PS were impaired under conditions of RDS at the expense of deficiency of total phospholipids, specifically phosphatidylcholine (PC) and phosphatidylethanolamine (PEA). Treatment with benzonal and zixorin improved the surface-active characteristics of PS and increased the content of total phospholipids mainly at the expense of PC and PEA. PMID:11980138

  14. 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.

  15. [Magnetopuncture therapy in the combined corrective treatment of clinical manifestations of non-specific distress syndrome].

    PubMed

    El'chininov, N V

    2009-01-01

    The efficiency of a combined approach to the correction of clinical manifestations of non-specific distress syndrome was evaluated in patients with psychovegetative syndrome by comparing effects of phytoaeroionotherapy, graduated physical exercises, and soft tissue manual therapy in different combinations with simultaneous magnetopuncture therapy and without it. It was shown that above therapeutic modalities combined with magnetotherapy decreased the degree of asymmetry of both right and left heart meridians (by 60.5%) and interhemisphere asymmetry of blood flow in the system of internal carotid arteries (by 74.19%), reduced the tone of cerebral arterioles and veins (by 40.7% and 8.6% respectively), improved symptomes of depression and asthenia (by 23.2% and 63.9% respectively), increased mental performance quotient and activity indices (by 34.7% and 28.7% respectively). These changes were far less significant in the absence of by magnetopuncture therapy. PMID:19514296

  16. The Influence of Prehospital Systemic Corticosteroid Use on Development of Acute Respiratory Distress Syndrome and Hospital Outcomes

    PubMed Central

    Karnatovskaia, Lioudmila V.; Lee, Augustine S.; Gajic, Ognjen; Festic, Emir

    2015-01-01

    Objective The role of systemic corticosteroids in pathophysiology and treatment of acute respiratory distress syndrome is controversial. Use of prehospital systemic corticosteroid therapy may prevent the development of acute respiratory distress syndrome and improve hospital outcomes. Design This is a preplanned retrospective subgroup analysis of the prospectively identified cohort from a trial by the U.S. Critical Illness and Injury Trials Group designed to validate the Lung Injury Prediction Score. Setting Twenty-two acute care hospitals. Patients Five thousand eighty-nine patients with at least one risk factor for acute respiratory distress syndrome at the time of hospitalization. Intervention Propensity-based analysis of previously recorded data. Measurements and Main Results Three hundred sixty-four patients were on systemic corticosteroids. Prevalence of acute respiratory distress syndrome was 7.7% and 6.9% (odds ratio, 1.1 [95% CI, 0.8–1.7]; p = 0.54) for patients on systemic corticosteroid and not on systemic corticosteroids, respectively. A propensity for being on systemic corticosteroids was derived through logistic regression by using all available covariates. Subsequently, 354 patients (97%) on systemic corticosteroids were matched to 1,093 not on systemic corticosteroids by their propensity score for a total of 1,447 patients in the matched set. Adjusted risk for acute respiratory distress syndrome (odds ratio, 0.96 [95% CI, 0.54–1.38]), invasive ventilation (odds ratio, 0.84 [95% CI, 0.62–1.12]), and inhospital mortality (odds ratio, 0.97 [95% CI, 0.63–1.49]) was then calculated from the propensity-matched sample using conditional logistic regression model. No significant associations were present. Conclusions Prehospital use of systemic corticosteroids neither decreased the development of acute respiratory distress syndrome among patients hospitalized with at one least risk factor, nor affected the need for mechanical ventilation or hospital

  17. Pumpless extracorporeal removal of carbon dioxide combined with ventilation using low tidal volume and high positive end-expiratory pressure in a patient with severe acute respiratory distress syndrome.

    PubMed

    Bein, T; Zimmermann, M; Hergeth, K; Ramming, M; Rupprecht, L; Schlitt, H J; Slutsky, A S

    2009-02-01

    The effects of the combination of a 'lowest' lung ventilation with extracorporeal elimination of carbon dioxide by interventional lung assist are described in a patient presenting with severe acute respiratory distress syndrome due to fulminant pneumonia. Reducing tidal volume to 3 ml.kg(-1) together with interventional lung assist resulted in a decrease in severe hypercapnia without alveolar collapse or hypoxaemia but with a decrease in serum levels of interleukin-6. This approach was applied for 12 days with recovery of the patient, without complications. Extracorporeal removal of carbon dioxide by interventional lung assist may be a useful tool to enable 'ultraprotective' ventilation in severe acute respiratory distress syndrome.

  18. 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).

  19. Noninvasive ventilation for acute respiratory distress syndrome: the importance of ventilator settings

    PubMed Central

    Costa, Eduardo L. V.; Nakamura, Maria A. M.; Morais, Caio C. A.

    2016-01-01

    Noninvasive ventilation (NIV) is commonly used to prevent endotracheal intubation in patients with acute respiratory distress syndrome (ARDS). Patients with hypoxemic acute respiratory failure who fail an NIV trial carry a worse prognosis as compared to those who succeed. Additional factors are also knowingly associated with worse outcomes: higher values of ICU severity score, presence of severe sepsis, and lower ratio of arterial oxygen tension to fraction of inspired oxygen. However, it is still unclear whether NIV failure is responsible for the worse prognosis or if it is merely a marker of the underlying disease severity. There is therefore an ongoing debate as to whether and which ARDS patients are good candidates to an NIV trial. In a recent paper published in JAMA, “Effect of Noninvasive Ventilation Delivered by Helmet vs. Face Mask on the Rate of Endotracheal Intubation in Patients with Acute Respiratory Distress Syndrome: A Randomized Clinical Trial”, Patel et al. evaluated ARDS patients submitted to NIV and drew attention to the importance of the NIV interface. We discussed their interesting findings focusing also on the ventilator settings and on the current barriers to lung protective ventilation in ARDS patients during NIV. PMID:27747041

  20. Evaluation of In-111 neutrophils in a model of the adult respiratory distress syndrome

    SciTech Connect

    Cooper, J.A.; Solano, S.J.; Bizios, R.; Line, B.R.; Malik, A.B.

    1984-01-01

    Neutrophils (PMNs) have been implicated in the pathogenesis of the adult respiratory distress syndrome. To further define their role, the authors studied the kinetics of In-111 labeled PMNs in a sheep model of acute pulmonary vascular injury. PMNs isolated by Percoll-plasma gradient centrifugation, and labeled with 500 uCi of In-111-oxine. Following i.v. reinfusion of the labeled PMNs, lung activity was monitored with the labeled PMNs, lung activity was monitored with a gamma camera. After a two hour baseline, pulmonary vascular injury secondary to intravascular coagulation was induced by the i.v. infusion of 100 units/kg of thrombin (n=5). Pulmonary time activity curves demonstrated increases in pulmonary PMN activity averaging 14% over baseline following thrombin infusion. A portion of the uptake was transient, lasting about 20 to 30 min., but PMN activity remained above baseline for the remainder of the study. Following the infusion of gamma thrombin, a form of thrombin unable to cleave fibrinogen, increased PMN uptake was not observed. Inhibition of fibrinolysis with tranaxemic acid, reduced the PMN response to thrombin to less than a 3% increase over baseline (n=2). The findings demonstrate that PMNs are involved in acute pulmonary vascular injury, and suggest a potential role for labeled PMNs in the clinical investigation of the adult respiratory distress syndrome.

  1. Morbidity and survival in neonates ventilated for the respiratory distress syndrome.

    PubMed Central

    Greenough, A; Roberton, N R

    1985-01-01

    In a retrospective analysis the records of all (210) infants ventilated to treat the respiratory distress syndrome over three years were reviewed. A mortality of 19% was found. Intraventricular haemorrhage was associated than a significant increase in mortality in infants of less with 30 weeks' gestation (p less than 0.001) and was the commonest cause of death. Pneumothoraces developed in one third of babies regardless of gestational age but were significantly associated with an increase in mortality only in infants of 27-29 weeks' gestation. Patent ductus arteriosus was present in 31 infants and was commoner in babies of very low birth weight. The presence of a patent ductus arteriosus was not associated with decreased survival but was significantly related to an increased need for prolonged respiratory support (p less than 0.001). Thirty six infants developed chronic lung disease, three of whom died. Comparison with data from earlier studies indicated a steady improvement over the past decade in outcome for infants ventilated for the respiratory distress syndrome. PMID:3918687

  2. 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. PMID:26963629

  3. Clinical review: Exogenous surfactant therapy for acute lung injury/acute respiratory distress syndrome - where do we go from here?

    PubMed Central

    2012-01-01

    Acute lung injury and acute respiratory distress syndrome (ARDS) are characterised by severe hypoxemic respiratory failure and poor lung compliance. Despite advances in clinical management, morbidity and mortality remains high. Supportive measures including protective lung ventilation confer a survival advantage in patients with ARDS, but management is otherwise limited by the lack of effective pharmacological therapies. Surfactant dysfunction with quantitative and qualitative abnormalities of both phospholipids and proteins are characteristic of patients with ARDS. Exogenous surfactant replacement in animal models of ARDS and neonatal respiratory distress syndrome shows consistent improvements in gas exchange and survival. However, whilst some adult studies have shown improved oxygenation, no survival benefit has been demonstrated to date. This lack of clinical efficacy may be related to disease heterogeneity (where treatment responders may be obscured by nonresponders), limited understanding of surfactant biology in patients or an absence of therapeutic effect in this population. Crucially, the mechanism of lung injury in neonates is different from that in ARDS: surfactant inhibition by plasma constituents is a typical feature of ARDS, whereas the primary pathology in neonates is the deficiency of surfactant material due to reduced synthesis. Absence of phenotypic characterisation of patients, the lack of an ideal natural surfactant material with adequate surfactant proteins, coupled with uncertainty about optimal timing, dosing and delivery method are some of the limitations of published surfactant replacement clinical trials. Recent advances in stable isotope labelling of surfactant phospholipids coupled with analytical methods using electrospray ionisation mass spectrometry enable highly specific molecular assessment of phospholipid subclasses and synthetic rates that can be utilised for phenotypic characterisation and individualisation of exogenous surfactant

  4. 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…

  5. Classifying Acute Respiratory Distress Syndrome Severity: Correcting the Arterial Oxygen Partial Pressure to Fractional Inspired Oxygen at Altitude.

    PubMed

    Pérez-Padilla, Rogelio; Hernández-Cárdenas, Carmen Margarita; Lugo-Goytia, Gustavo

    2016-01-01

    In the well-known Berlin definition of acute respiratory distress syndrome (ARDS), there is a recommended adjustment for arterial oxygen partial pressure to fractional inspired oxygen (PaO2/FIO2) at altitude, but without a reference as to how it was derived. PMID:27623033

  6. Multiple organ failure with the adult respiratory distress syndrome in homicidal arsenic poisoning.

    PubMed

    Bolliger, C T; van Zijl, P; Louw, J A

    1992-01-01

    A 30-year-old man and a 39-year-old woman, who was 28 weeks pregnant, were simultaneously poisoned by eating chocolate containing arsenic trioxide. They developed a picture of multiple organ failure peaking around the 8th to 10th day after ingestion, with the development of life-threatening adult respiratory distress syndrome (ARDS) in both patients. This rarely reported complication of arsenic poisoning was managed successfully by intubation and mechanical ventilation with positive end expiratory pressure in both patients. Hemodynamic and laboratory data are presented supporting the clinical course. Arsenic toxicity further resulted in intrauterine fetal death. The effects of severe arsenic poisoning leading to early multiple organ failure with ARDS as well as to protracted, debilitating polyneuropathy are discussed.

  7. Arthritis and adult respiratory distress syndrome: unusual presentations of typhoid fever

    PubMed Central

    Dhakad, Urmila; Das, Siddharth K; Srivastva, Durgesh; Nolkha, Nilesh

    2014-01-01

    A middle-aged woman presented with fever of 1-month duration along with bilateral knee joint pain, swelling and difficulty in walking for 2 weeks. The patient's Typhidot test was positive for IgM antibodies. Her Widal test was negative, and blood culture and synovial fluid culture were sterile. She was started on ceftriaxone, to which her fever initially responded. However, after 4 days of treatment her disease course was complicated by relapse of fever and acute respiratory distress syndrome (ARDS). This settled with respiratory support and addition of azithromycin. Following recovery from ARDS and fever, her persistent knee arthritis responded to intra-articular methyl prednisolone instillation. PMID:25336548

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

    PubMed Central

    2013-01-01

    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). PMID:23672857

  9. Adult onset Still's disease accompanied by acute respiratory distress syndrome: A case report

    PubMed Central

    Xi, Xiao-Tu; Wang, Mao-Jie; Huang, Run-Yue; Ding, Bang-Han

    2016-01-01

    Adult onset Still's disease (AOSD) is a systemic inflammatory disorder characterized by rash, leukocytosis, fever and arthralgia/arthritis. The most common pulmonary manifestations associated with AOSD are pulmonary infiltrates and pleural effusion. The present study describes a 40-year-old male with AOSD who developed fever, sore throat and shortness of breath. Difficulty breathing promptly developed, and the patient was diagnosed with acute respiratory distress syndrome (ARDS). The patient did not respond to antibiotics, including imipenem, vancomycin, fluconazole, moxifloxacin, penicillin, doxycycline and meropenem, but was sensitive to glucocorticoid treatment, including methylprednisolone sodium succinate. ARDS accompanied by AOSD has been rarely reported in the literature. In conclusion, in a patient with ARDS who does not respond to antibiotic treatment, the involvement of AOSD should be considered. PMID:27588099

  10. Effect of mode of delivery on incidence of respiratory distress syndrome.

    PubMed

    Curet, L B; Zachman, R D; Rao, A V; Poole, W K; Morrison, J; Burkett, G

    1988-10-01

    The effect of route of delivery on incidence of respiratory distress syndrome (RDS) has been controversial. While some investigators have reported no difference in RDS rates in infants born by cesarean section as compared to vaginal delivery, others have shown a significant increase in risk for RDS among infants born by cesarean section. Data from the 297 patients comprising the placebo group in the recently completed collaborative study of antenatal steroid therapy in the prevention of neonatal RDS, were analyzed to determine the effect of mode of delivery on RDS. The results indicate that infants born by cesarean section without labor have a higher risk for neonatal RDS than infants born vaginally or by cesarean section after a trial of labor. PMID:2903080

  11. Effect of diabetes mellitus on amniotic fluid lecithin/sphingomyelin ratio and respiratory distress syndrome.

    PubMed

    Curet, L B; Olson, R W; Schneider, J M; Zachman, R D

    1979-09-01

    Ninety-eight insulin-dependent diabetic patients were cared for during 108 pregnancies following an ambulatory program aimed at close control of blood sugar levels. The incidence of respiratory distress syndrome (RDS) in these patients was not different from that in nondiabetic patients. Fetal pulmonary maturity seemed to progress at a normal rate as evidenced by an equal incidence of mature lecithin/sphingomyelin (L/S) ratios at various stages of gestation in diabetic and nondiabetic patients. There was no evidence that Class B and C diabetic patients have delayed fetal lung maturation or that Class E, F, and R patients have accelerated maturation. It is concluded that careful metabolic control of the pregnant diabetic patient results in a normal process of fetal pulmonary maturation without increased risk of RDS. PMID:474639

  12. 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

  13. [Two cases of acute respiratory distress syndrome related to zinc fumes and zinc dust inhalation].

    PubMed

    Ishimoto, Hiroshi; Yatera, Kazuhiro; Oda, Keishi; Kawanami, Toshinori; Soda, Hiroshi; Kohno, Shigeru; Mukae, Hiroshi

    2014-06-01

    Two cases of acute respiratory distress syndrome related to zinc fume inhalation and zinc powder inhalation are presented. Case 1 demonstrated acute respiratory symptoms during the work of distortion correction of iron boards, coated with a rust preventative including rich zinc using an acetylene gas burner. Case 2 occurred after the work of applying a rust preventative that included zinc powder, mainly using an airless compressor. Since both were working in a boathouse, without using protective equipment, the possible causes of Case 1 and 2 were inhalation exposure to zinc fumes and zinc powder, respectively. The two patients showed similar clinical courses, including favorable treatment outcomes, mimicking acute respiratory failure complicated by a metal fume fever. PMID:24930880

  14. A method to determine the kinetics of multiple proteins in human infants with respiratory distress syndrome.

    PubMed

    Bereman, Michael S; Tomazela, Daniela M; Heins, Hillary S; Simonato, Manuela; Cogo, Paola E; Hamvas, Aaron; Patterson, Bruce W; Cole, F Sessions; MacCoss, Michael J

    2012-06-01

    We report a method to measure in vivo turnover of four proteins from sequential tracheal aspirates obtained from human newborn infants with respiratory distress syndrome using targeted proteomics. We detected enrichment for all targeted proteins approximately 3 h from the start of infusion of [5,5,5-(2)H(3)] leucine, secretion times that varied from 1.2 to 2.5 h, and half lives that ranged between 10 and 21 h. Complement factor B, a component of the alternative pathway of complement activation, had an approximately twofold-longer half-life than the other three proteins. In addition, the kinetics of mature and carboxy-terminal tryptic peptides from the same protein (surfactant protein B) were not statistically different (p = 0.49).

  15. [Two cases of acute respiratory distress syndrome related to zinc fumes and zinc dust inhalation].

    PubMed

    Ishimoto, Hiroshi; Yatera, Kazuhiro; Oda, Keishi; Kawanami, Toshinori; Soda, Hiroshi; Kohno, Shigeru; Mukae, Hiroshi

    2014-06-01

    Two cases of acute respiratory distress syndrome related to zinc fume inhalation and zinc powder inhalation are presented. Case 1 demonstrated acute respiratory symptoms during the work of distortion correction of iron boards, coated with a rust preventative including rich zinc using an acetylene gas burner. Case 2 occurred after the work of applying a rust preventative that included zinc powder, mainly using an airless compressor. Since both were working in a boathouse, without using protective equipment, the possible causes of Case 1 and 2 were inhalation exposure to zinc fumes and zinc powder, respectively. The two patients showed similar clinical courses, including favorable treatment outcomes, mimicking acute respiratory failure complicated by a metal fume fever.

  16. Inhaled nitric oxide therapy for acute respiratory distress syndrome in children.

    PubMed

    Medjo, Biljana; Atanaskovic-Markovic, Marina; Nikolic, Dimitrije; Cuturilo, Goran; Djukic, Slobodanka

    2012-07-01

    The aim of this study was to evaluate the effects of inhaled nitric oxide (iNO) therapy on oxygenation and mortality in children with acute respiratory distress syndrome (ARDS). Thirty-three children with ARDS and an arterial SatO2 <88% despite mechanical ventilation were analyzed. Patients in the iNO group were prospectively enrolled and treated with conventional therapy plus iNO. The control group consisted of retrospectively analyzed patients treated only with conventional therapy. A significant increase in PaO2/FiO2 ratio (25.6%) and decrease in oxygenation index (19.5%) was observed after 4 h of iNO treatment, when compared to baseline values. A positive response to iNO was detected in 69% of patients, and there was no difference between pulmonary and extrapulmonary ARDS. There was no difference in mortality and duration of mechanical ventilation between iNO and control group. PMID:22885439

  17. [Ultrastructural changes in the lung in acute adult respiratory distress syndrome].

    PubMed

    Szemenyei, K; Széll, K; Kádas, L

    1980-04-01

    Morphological alterations of the lung in respiratory distress syndrome of adults (ARDS) were analyzed in 10 cases with traumatic-and septic shock, laryngitis subglottica descendens and bronchopneumonia. For the better understanding of the pathomechanism of the disease in addition to the standard methods, first of all ultrastructural alterations were studied. Two phases of the morphologic alterations could be distinguished, the phase of the destruction and the phase of the repair. These two processes are not sharply distinguishable. Genesis of the characteristic histological alterations (damage to the epithelial and endothelial cells, formation of hyaline membranes, microcoagulation, proliferation of the type II pneumocytes and fibroblasts, fibrosis) is discussed, with regard to the data of the literature.

  18. [Acute respiratory distress syndrome: how to optimize oxygen transport and to improve prognosis].

    PubMed

    Shtabnitskiy, V A; Chuchalin, A G

    2014-01-01

    The paper reviews the state-of-the-art of acute respiratory distress syndrome (ARDS) and current approaches to correcting respiratory failure. It highlights the historical and present-day data on the efficiency of extracorporeal membrane hemoxygenation, high-frequency ventilation, surfactant and inhaled nitric oxide therapy, and prone ventilation. The examinations have shown that ventilation in the prone position and extracorporeal membrane hemoxygenation not only improve gas exchange, but have a positive prognostic impact. The use of inhaled nitric oxide and surfactant achieves improved oxygenation for a while, but has no substantial effect on prognosis. The place of high-frequency oscillatory ventilation in the treatment of patients with ARDS has not been fully determined as some examinations have indicated a positive prognostic impact and other examinations have shown none or a negative impact.

  19. [Pumpless extracorporeal pulmonary care: an alternative in the treatment of persistent acute respiratory distress syndrome].

    PubMed

    Tomicic, V; Montalván, C; Espinoza, M; Graf, J; Martínez, E; Umaña, A; Torres, J

    2008-01-01

    A 34-year old woman who developed persistent and severe acute respiratory distress syndrome with underlying myelomonocytic leukemia (M4FAB) is described. After ruling out the most common causes of pulmonary infiltration in this type of patient and one week of broad spectrum antibiotics and steroids therapy, we proposed leukemic pulmonary infiltration as etiological diagnosis. Despite using a protective ventilatory strategy, recruitment maneuvers, prone position and high frequency oscillatory ventilation, her gas exchange became worse. Under this condition we used a Pumpless-Extracorporeal life assist (PELA) and begun chemotherapy. The method, arterial blood gases, hemodynamic parameters and ventilatory mechanics before and after its use are described. The patient remained on P-ELA for nine days; one week later she was extubated and ten days after she was discharged from the Intensive Care Unit the patient left the hospital in good health condition.

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

    PubMed

    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

  1. Computerized scintigraphic technique for the evaluation of adult respiratory distress syndrome: initial clinical trials

    SciTech Connect

    Tatum, J.L.; Burke, T.S.; Sugerman, H.J.; Strash, A.M.; Hirsch, J.I.; Fratkin, M.J.

    1982-04-01

    Eleven patients with suspected adult respiratory distress syndrome (ARDS) and five control patients were studied using a computerized gamma imaging and analysis technique and 99mTc-labeled human serum albumin. The heart and right lung were imaged, lung:heart ratio was plotted vs. time, and a linear regression was fitted to the data points displayed. The slope of this fit was termed the ''slope index.'' An index value of 2 standard deviations greater than the control mean was considered positive. Radiographs from the six positive studies revealed typical diffuse air-space disease. Radiographs from two of the five negative studies demonstrated air-space consolidation. Both of these patients had elevated pulmonary capillary wedge pressure, cardiomegaly, and clinical course consistent with cardiogenic pulmonary edema. These preliminary data demonstrated a good correlation between positive slope index and clinical ARDS.

  2. Increased pulmonary alveolar-capillary permeability in patients at risk for adult respiratory distress syndrome

    SciTech Connect

    Tennenberg, S.D.; Jacobs, M.P.; Solomkin, J.S.; Ehlers, N.A.; Hurst, J.M.

    1987-04-01

    Two methods for predicting adult respiratory distress syndrome (ARDS) were evaluated prospectively in a group of 81 multitrauma and sepsis patients considered at clinical high risk. A popular ARDS risk-scoring method, employing discriminant analysis equations (weighted risk criteria and oxygenation characteristics), yielded a predictive accuracy of 59% and a false-negative rate of 22%. Pulmonary alveolar-capillary permeability (PACP) was determined with a radioaerosol lung-scan technique in 23 of these 81 patients, representing a statistically similar subgroup. Lung scanning achieved a predictive accuracy of 71% (after excluding patients with unilateral pulmonary contusion) and gave no false-negatives. We propose a combination of clinical risk identification and functional determination of PACP to assess a patient's risk of developing ARDS.

  3. Proteomic study of acute respiratory distress syndrome: current knowledge and implications for drug development.

    PubMed

    Levitt, Joseph E; Rogers, Angela J

    2016-05-01

    The acute respiratory distress syndrome (ARDS) is a common cause of acute respiratory failure, and is associated with substantial mortality and morbidity. Dozens of clinical trials targeting ARDS have failed, with no drug specifically targeting lung injury in widespread clinical use. Thus, the need for drug development in ARDS is great. Targeted proteomic studies in ARDS have identified many key pathways in the disease, including inflammation, epithelial injury, endothelial injury or activation, and disordered coagulation and repair. Recent studies reveal the potential for proteomic changes to identify novel subphenotypes of ARDS patients who may be most likely to respond to therapy and could thus be targeted for enrollment in clinical trials. Nontargeted studies of proteomics in ARDS are just beginning and have the potential to identify novel drug targets and key pathways in the disease. Proteomics will play an important role in phenotyping of patients and developing novel therapies for ARDS in the future. PMID:27031735

  4. Clinical review: Lung imaging in acute respiratory distress syndrome patients - an update

    PubMed Central

    2013-01-01

    Over the past 30 years lung imaging has greatly contributed to the current understanding of the pathophysiology and the management of acute respiratory distress syndrome (ARDS). In the past few years, in addition to chest X-ray and lung computed tomography, newer functional lung imaging techniques, such as lung ultrasound, positron emission tomography, electrical impedance tomography and magnetic resonance, have been gaining a role as diagnostic tools to optimize lung assessment and ventilator management in ARDS patients. Here we provide an updated clinical review of lung imaging in ARDS over the past few years to offer an overview of the literature on the available imaging techniques from a clinical perspective. PMID:24238477

  5. [Septic shock Fusobacterium necrophorum from origin gynecological at complicated an acute respiratory distress syndrome: a variant of Lemierre's syndrome].

    PubMed

    Huynh-Moynot, Sophie; Commandeur, Diane; Danguy des Déserts, Marc; Drouillard, Isabelle; Leguen, Patrick; Ould-Ahmed, Mehdi

    2011-01-01

    We report a case of a female patient of 47 years old who presents in a state of septic shock with acute insufficient respiratory complicated with syndrome of acute respiratory distress, together with a list of abdominal pain and polyarthralgia too. In her case of medical history, it is retained that she has had a intra-uterine device since 6 years without medical follow up. The initial thoraco-abdomino-pelvic scan shows a left ovarian vein thrombosis, as well as the opaqueness alveolus diffused interstitiel bilaterally and an aspect of ileitis. The IUD is taken off because of sudden occuring of purulent leucorrhoea. This results in a clinical and paraclinical improvement, whereas aminopenicillin was administered to the patient since 1 week. The microbiological blood test allows to put in evidence Fusobacterium necrophorum found in a blood culture and is sensitive to the amoxicilline-acide clavulanique and metronidazole. Isolation of this bacteria, classically found in Lemierre's syndrome, allowed to explain the multilfocalization of the symtoms and the list of pain. The whole concerns about a variant of Lemierre's syndrom: a state of septic shock secondary then caused by the anaerobic Gram negative bacilli, which is a commensal bacteria of the female genital tractus, complicated of septic emboli typical.

  6. Lethal neuroleptic malignant syndrome due to amisulpride.

    PubMed

    Musshoff, Frank; Doberentz, Elke; Madea, Burkhard

    2013-06-01

    A 42-year old-man was found lying in his bed having seizures. Later he became unconscious and hypotonic developing mydriasis as well as rigidity. The body core temperature (rectal temperature) was above 42 °C. Blood pH was decreased during treatment, and his general condition deteriorated. The patient developed gasping respiration, ventricular fibrillation, and died. During autopsy and histological investigation cerebral and pulmonary edema were noted together with general congestion of the internal organs. Further observations included contraction bands of myocytes, a contracted spleen, fibrosis of the liver, and gall stones. Toxicological analyses of peripheral blood revealed the following results: amisulpride 4.65 mg/l, biperiden 0.12 mg/l, imipramine 0.33 mg/l, and desipramine 0.68 mg/l. An amisulpride-induced neuroleptic malignant syndrome was therefore diagnosed as the patho-physiological mechanism leading to death.

  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. 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

  9. Clinical predictors of lacunar syndrome not due to lacunar infarction

    PubMed Central

    2010-01-01

    Background Lacunar syndrome not due to lacunar infarct is poorly characterised. This single centre, retrospective study was conducted to describe the clinical characteristics of patients with lacunar syndrome not due to lacunar infarct and to identify clinical predictors of this variant of lacunar stroke. Methods A total of 146 patients with lacunar syndrome not due to lacunar infarction were included in the "Sagrat Cor Hospital of Barcelona Stroke Registry" during a period of 19 years (1986-2004). Data from stroke patients are entered in the stroke registry following a standardized protocol with 161 items regarding demographics, risk factors, clinical features, laboratory and neuroimaging data, complications and outcome. The characteristics of these 146 patients with lacunar syndrome not due to lacunar infarct were compared with those of the 733 patients with lacunar infarction. Results Lacunar syndrome not due to lacunar infarct accounted for 16.6% (146/879) of all cases of lacunar stroke. Subtypes of lacunar syndromes included pure motor stroke in 63 patients, sensorimotor stroke in 51, pure sensory stroke in 14, atypical lacunar syndrome in 9, ataxic hemiparesis in 5 and dysarthria-clumsy hand in 4. Valvular heart disease, atrial fibrillation, sudden onset, limb weakness and sensory symptoms were significantly more frequent among patients with lacunar syndrome not due to lacunar infarct than in those with lacunar infarction, whereas diabetes was less frequent. In the multivariate analysis, atrial fibrillation (OR = 4.62), sensorimotor stroke (OR = 4.05), limb weakness (OR = 2.09), sudden onset (OR = 2.06) and age (OR = 0.96) were independent predictors of lacunar syndrome not due to lacunar infarct. Conclusions Although lacunar syndromes are highly suggestive of small deep cerebral infarctions, lacunar syndromes not due to lacunar infarcts are found in 16.6% of cases. The presence of sensorimotor stroke, limb weakness and sudden onset in a patient with atrial

  10. Cardiac arrest due to baclofen withdrawal syndrome

    PubMed Central

    Cardoso, Ana Luísa; Quintaneiro, Claudio; Seabra, Helena; Teixeira, Carla

    2014-01-01

    A 41-year-old man presented with postcervical traumatic complete quadriparesis under intrathecal baclofen therapy (ITB) for refractory spasticity. Less than 24 h after having his baclofen pump substituted, he develops hyperthermia, seizures, cognitive depression, acute hypoxaemic respiratory failure and cardiovascular instability leading to mechanical ventilation and vasopressor support. He was transferred to an intensive care unit with diagnosis of community-acquired pneumonia leading to septic shock. He evolved with progressive clinical worsening and multisystem organ failure and cardiac arrest in non-shockable rhythm (pulseless electrical activity)—4 min resuscitation with return of spontaneous circulation. Considering the possible diagnosis of baclofen withdrawal syndrome and, in suspicion of ITB delivery disruption, the catheter system was surgically explored and a leaking tubule attachment was found. Despite aggressive cardiovascular, respiratory and renal support therapy, clinical improvement occurred only after restoration of intrathecal drug delivery. He was discharged from the hospital after 56 days, having returned to baseline status. PMID:24827663

  11. Systemic coagulation and fibrinolysis in patients with or at risk for the adult respiratory distress syndrome.

    PubMed

    Groeneveld, A B; Kindt, I; Raijmakers, P G; Hack, C E; Thijs, L G

    1997-12-01

    The authors sought to evaluate the pathogenetic and prognostic role of a procoagulant and hypofibrinolytic state in the adult respiratory distress syndrome (ARDS). Twenty-two consecutive patients admitted to the intensive care unit (ICU) for respiratory monitoring (n = 2) or mechanical ventilation (n = 20) were studied, of whom 13 had ARDS and 9 were at risk for the syndrome. Plasma levels of thrombin-antithrombin III complexes (TAT), the plasmin-alpha2-antiplasmin complexes (PAP), tissue-type plasminogen activator (tPA) and plasminogen activator inhibitor type 1 (PAI-1) were measured within 48 h after admission, together with respiratory variables allowing computation of the lung injury score (LIS), and pulmonary microvascular permeability [67Gallium-transferrin pulmonary leak index (PLI)], as measures of pulmonary dysfunction. Blood was also sampled 6-hourly until 2 days after admission. The LIS and PLI were higher in ARDS than at risk patients, in the presence of similar systemic morbidity and mortality. TAT complexes were elevated in a minority of patients of both groups, whereas the PAP, tPA and PAI levels were elevated above normal in the majority of ARDS and at risk patients, but groups did not differ. Neither circulating coagulation nor fibrinolysis variables correlated to either LIS or PLI. Furthermore, the course of haemostatic variables did not relate to outcome. These data indicate that systemic activation of coagulation and impaired fibrinolysis do not play a major role in ARDS development and outcome in patients with acute lung injury.

  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. Noninvasive ventilation for patients with acute lung injury or acute respiratory distress syndrome.

    PubMed

    Nava, Stefano; Schreiber, Ania; Domenighetti, Guido

    2011-10-01

    Few studies have been performed on noninvasive ventilation (NIV) to treat hypoxic acute respiratory failure in patients with acute lung injury (ALI) or acute respiratory distress syndrome (ARDS). The outcomes of these patients, for whom endotracheal intubation is not mandatory, depend on the degree of hypoxia, the presence of comorbidities and complications, and their illness severity. The use of NIV as an alternative to invasive ventilation in severely hypoxemic patients with ARDS (ie, P(aO(2))/F(IO(2)) < 200) is not generally advisable and should be limited to hemodynamically stable patients who can be closely monitored in an intensive care unit by highly skilled staff. Early NIV application may be extremely helpful in immunocompromised patients with pulmonary infiltrates, in whom intubation dramatically increases the risk of infection, pneumonia, and death. The use of NIV in patients with severe acute respiratory syndrome and other airborne diseases has generated debate, despite encouraging clinical results, mainly because of safety issues. Overall, the high rate of NIV failure suggests a cautious approach to NIV use in patients with ALI/ARDS, including early initiation, intensive monitoring, and prompt intubation if signs of NIV failure emerge. PMID:22008399

  14. [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.

  15. Neuroleptic malignant syndrome due to risperidone treatment in a child with Joubert syndrome.

    PubMed

    Vurucu, Sebahattin; Congologlu, Ayhan; Altun, Demet; Unay, Bulent; Akin, Ridvan

    2009-03-01

    Joubert syndrome is a rare autosomal recessive disease characterized by hypotonia, ataxia, episodic hyperpnea, psychomotor retardation, abnormal ocular movements, cerebellar vermian hypoplasia, and molar tooth sign on magnetic resonance imaging. Neuroleptic malignant syndrome is an uncommon and potentially fatal idiosynchratic reaction of antipsychotic drugs, in which the clinical scenario encompass muscular rigidity, hyperthermia, autonomic dysfunction, altered consciousness, high creatinine phosphokinase levels, and leukocytosis. This report describes a case of neuroleptic malignant syndrome due to risperidone in a child with Joubert syndrome.

  16. 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

  17. Mugwort-Mustard Allergy Syndrome due to Broccoli Consumption.

    PubMed

    Sugita, Yuri; Makino, Teruhiko; Mizawa, Megumi; Shimizu, Tadamichi

    2016-01-01

    Pollen-food allergy syndrome (PFAS) is a relatively rare form of food allergy which develops in individuals who are sensitized to pollen. Tree pollens, especially birch pollen, frequently induce PFAS; however, the incidence of PFAS due to grass or weed pollens such as ragweed or mugwort is relatively rare. Mugwort-mustard allergy syndrome (MMAS) is an example of a PFAS in which individuals sensitized to mugwort may develop an allergy to mustard and experience severe reactions. We herein describe a case of MMAS due to broccoli consumption. PMID:27478657

  18. Mugwort-Mustard Allergy Syndrome due to Broccoli Consumption

    PubMed Central

    2016-01-01

    Pollen-food allergy syndrome (PFAS) is a relatively rare form of food allergy which develops in individuals who are sensitized to pollen. Tree pollens, especially birch pollen, frequently induce PFAS; however, the incidence of PFAS due to grass or weed pollens such as ragweed or mugwort is relatively rare. Mugwort-mustard allergy syndrome (MMAS) is an example of a PFAS in which individuals sensitized to mugwort may develop an allergy to mustard and experience severe reactions. We herein describe a case of MMAS due to broccoli consumption. PMID:27478657

  19. Isolated deafness following recovery from neurologic injury and adult respiratory distress syndrome. A sequela of intercurrent aminoglycoside and diuretic use.

    PubMed

    Lynn, A M; Redding, G J; Morray, J P; Tyler, D C

    1985-05-01

    We report two children who survived neurologic injury (near-drowning and Reye's syndrome) and adult respiratory distress syndrome and who required prolonged ventilatory support. Follow-up examination in both children showed steady neurologic recovery, but five months following discharge from their acute illness, profound hearing loss was diagnosed in both children. A review of the literature is reported and the hypothesis that combined aminoglycoside antibiotic and loop diuretic therapy caused the hearing loss is presented. Recommendation is made for audiologic assessment within six months of recovery from critical illness of pediatric patients in whom therapy has included loop diuretic and aminoglycoside antibiotic therapy.

  20. Intrapleural steroid instillation for multiple organ failure with acute respiratory distress syndrome.

    PubMed

    Huang, Pei-Ming; Lin, Tzu-Hsin; Tsai, Pi-Ru; Ko, Wen-Je

    2013-11-01

    Acute respiratory distress syndrome (ARDS) increases mortality in patients with multiorgan dysfunction syndrome (MODS). This study evaluates the feasibility of intrapleural steroid instillation (IPSI) in patients with ARDS and MODS unresponsive to conventional extracorporeal membrane oxygenation (ECMO). Ninety-two of 467 patients who underwent ECMO between 2005 and 2009 had ARDS, and 30 consecutive adult patients of these 92 patients with severe ARDS and MODS were retrospectively analyzed in this study. Nine of these 30 patients, who did not respond to therapy and whose condition deteriorated, were managed with IPSI. All patients met the inclusion criteria of hemodynamic instability with high catecholamine infusion requirement and 100% oxygen demand in ventilation and ECMO flow. On initial diagnosis of ARDS, no differences in prognostic scorings were observed in patients who underwent conventional treatment (n = 21) and those who underwent IPSI (n = 9). Blood oxygenation, tidal volume, changing in chest radiographic findings, and survival rates were analyzed. The primary outcome was survival until discharge from the hospital. Pulmonary radiographic appearance improved after 3 days of IPSI treatment (P = 0.008); the ratio of arterial partial pressure of oxygen to fraction of inspired oxygen also increased significantly after 5 days of IPSI treatment (P = 0.028). Moreover, the 28-day mortality rate (P = 0.017), 60-day mortality rate (P = 0.003), and survival rate (78% vs. 19%; P = 0.003) significantly improved in patients undergoing IPSI, which therefore appears to be an easily implemented and highly effective treatment for patients with severe ARDS in combination with MODS, particularly in patients who fail to respond to conventional treatment.

  1. Acute respiratory distress syndrome in a welder exposed to metal fumes.

    PubMed

    Barbee, J Y; Prince, T S

    1999-05-01

    A 43-year-old man began having malaise, chills, and fever 12 hours after cutting a galvanized steel grating with an acetylene torch at work. Over the next 72 hours, his symptoms persisted and became worse with progressive shortness of breath. He was admitted to the hospital and begun on antibiotics and steroids. The next day his condition had deteriorated to the point that he had to be intubated. Chest x-ray film and computed tomography showed patchy and interstitial infiltration bilaterally, consistent with acute respiratory distress syndrome. Open lung biopsy showed focal mild interstitial pneumonia. Multiple laboratory studies were negative for an infectious or an immune process. The patient remained on mechanical ventilation for 10 days and was discharged from the hospital 2 days after extubation. He continued to improve, with minimal symptoms and a return to normal activity levels several months after the incident with no continued treatment. Re-creation of his exposure was done under controlled circumstances, with air sampling revealing elevated air levels for cadmium and zinc and borderline levels of arsenic, manganese, lead, and iron.

  2. Recruitment maneuvers in acute respiratory distress syndrome: The safe way is the best way.

    PubMed

    Santos, Raquel S; Silva, Pedro L; Pelosi, Paolo; Rocco, Patricia Rm

    2015-11-01

    Acute respiratory distress syndrome (ARDS) represents a serious problem in critically ill patients and is associated with in-hospital mortality rates of 33%-52%. Recruitment maneuvers (RMs) are a simple, low-cost, feasible intervention that can be performed at the bedside in patients with ARDS. RMs are characterized by the application of airway pressure to increase transpulmonary pressure transiently. Once non-aerated lung units are reopened, improvements are observed in respiratory system mechanics, alveolar reaeration on computed tomography, and improvements in gas exchange (functional recruitment). However, the reopening process could lead to vascular compression, which can be associated with overinflation, and gas exchange may not improve as expected (anatomical recruitment). The purpose of this review was to discuss the effects of different RM strategies - sustained inflation, intermittent sighs, and stepwise increases of positive end-expiratory pressure (PEEP) and/or airway inspiratory pressure - on the following parameters: hemodynamics, oxygenation, barotrauma episodes, and lung recruitability through physiological variables and imaging techniques. RMs and PEEP titration are interdependent events for the success of ventilatory management. PEEP should be adjusted on the basis of respiratory system mechanics and oxygenation. Recent systematic reviews and meta-analyses suggest that RMs are associated with lower mortality in patients with ARDS. However, the optimal RM method (i.e., that providing the best balance of benefit and harm) and the effects of RMs on clinical outcome are still under discussion, and further evidence is needed.

  3. Pulmonar recruitment in acute respiratory distress syndrome. What is the best strategy?

    PubMed

    Santos, Cíntia Lourenço; Samary, Cynthia dos Santos; Fiorio Júnior, Pedro Laurindo; Santos, Bruna Lourenço; Schanaider, Alberto

    2015-01-01

    Supporting patients with acute respiratory distress syndrome (ARDS), using a protective mechanical ventilation strategy characterized by low tidal volume and limitation of positive end-expiratory pressure (PEEP) is a standard practice in the intensive care unit. However, these strategies can promote lung de-recruitment, leading to the cyclic closing and reopening of collapsed alveoli and small airways. Recruitment maneuvers (RM) can be used to augment other methods, like positive end-expiratory pressure and positioning, to improve aerated lung volume. Clinical practice varies widely, and the optimal method and patient selection for recruitment maneuvers have not been determined, considerable uncertainty remaining regarding the appropriateness of RM. This review aims to discuss recent findings about the available types of RM, and compare the effectiveness, indications and adverse effects among them, as well as their impact on morbidity and mortality in ARDS patients. Recent developments include experimental and clinical evidence that a stepwise extended recruitment maneuver may cause an improvement in aerated lung volume and decrease the biological impact seen with the traditionally used sustained inflation, with less adverse effects. Prone positioning can reduce mortality in severe ARDS patients and may be an useful adjunct to recruitment maneuvers and advanced ventilatory strategies, such noisy ventilation and BIVENT, which have been useful in providing lung recruitment.

  4. Total ginsenosides synergize with ulinastatin against septic acute lung injury and acute respir atory distress syndrome

    PubMed Central

    Sun, Rongju; Li, Yana; Chen, Wei; Zhang, Fei; Li, Tanshi

    2015-01-01

    Total ginsenosides synergize with ulinastatin (UTI) against septic acute lung injury (ALI) and acute respiratory distress syndrome (ARDS). We randomly divided 80 cases of severe sepsis-induced ALI and ARDS into a UTI group and a ginsenosides (GS)+UTI group. Continuous electrocardiac monitoring of pulse, respiratory rate, blood pressure, and heart rate; invasive hemodynamic monitoring; ventilator-assisted breathing and circulation support; and anti-infection as well as UTI treatment were given in the UTI group with GS treatment added for 7 consecutive days in the GS+UTI group. The indicators of pulmonary vascular permeability, pulmonary circulation, blood gases, and hemodynamics as well as APACHE II and ALI scores were detected on days 1, 3, and 7. The ALI score in the GS+UTI group was significantly decreased (P < 0.05) compared with that of the UTI group, and the indicators of pulmonary capillary permeability such as pulmonary vascular permeability index, extravascular lung water index, and oxygenation index, in the GS+UTI group improved significantly more than that of the UTI group. The indicators of hemodynamics and pulmonary circulation such as cardiac index, intrathoracic blood volume index, and central venous pressure improved significantly (P < 0.05), and the APACHE II score in the GS+UTI group was lower than that of the UTI group. GS can effectively collaborate with UTI against ALI and/or ARDS. PMID:26261640

  5. Total ginsenosides synergize with ulinastatin against septic acute lung injury and acute respiratory distress syndrome.

    PubMed

    Sun, Rongju; Li, Yana; Chen, Wei; Zhang, Fei; Li, Tanshi

    2015-01-01

    Total ginsenosides synergize with ulinastatin (UTI) against septic acute lung injury (ALI) and acute respiratory distress syndrome (ARDS). We randomly divided 80 cases of severe sepsis-induced ALI and ARDS into a UTI group and a ginsenosides (GS)+UTI group. Continuous electrocardiac monitoring of pulse, respiratory rate, blood pressure, and heart rate; invasive hemodynamic monitoring; ventilator-assisted breathing and circulation support; and anti-infection as well as UTI treatment were given in the UTI group with GS treatment added for 7 consecutive days in the GS+UTI group. The indicators of pulmonary vascular permeability, pulmonary circulation, blood gases, and hemodynamics as well as APACHE II and ALI scores were detected on days 1, 3, and 7. The ALI score in the GS+UTI group was significantly decreased (P < 0.05) compared with that of the UTI group, and the indicators of pulmonary capillary permeability such as pulmonary vascular permeability index, extravascular lung water index, and oxygenation index, in the GS+UTI group improved significantly more than that of the UTI group. The indicators of hemodynamics and pulmonary circulation such as cardiac index, intrathoracic blood volume index, and central venous pressure improved significantly (P < 0.05), and the APACHE II score in the GS+UTI group was lower than that of the UTI group. GS can effectively collaborate with UTI against ALI and/or ARDS.

  6. The acute respiratory distress syndrome: role of nutritional modulation of inflammation through dietary lipids.

    PubMed

    Mizock, Barry A; DeMichele, Stephen J

    2004-12-01

    The acute respiratory distress syndrome (ARDS) is the most serious form of acute hypoxic respiratory failure. ARDS represents the expression of an acute, diffuse, inflammatory process in the lungs consequent to a variety of infectious and noninfectious conditions. It is characterized pathologically by damage to pulmonary epithelial and endothelial cells, with subsequent alveolar-capillary leak and exudative pulmonary edema. The main clinical features of ARDS include rapid onset of dyspnea, severe defects in gas exchange, and imaging studies demonstrating diffuse pulmonary infiltrates. The role of nutrition in the management of ARDS has traditionally been supportive. Recent research has demonstrated the potential of certain dietary oils (eg, fish oil, borage oil) to modulate pulmonary inflammation, thereby improving lung compliance and oxygenation, and reducing time on mechanical ventilation. This article reviews the alterations in the immune response that underlie ARDS, discusses the physiology of dietary oils as immunonutrients, summarizes animal and human studies that explore the therapeutic effects of dietary oils, and provides clinical recommendations for their use. PMID:16215155

  7. Enrichment of the lung microbiome with gut bacteria in sepsis and the acute respiratory distress syndrome.

    PubMed

    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

    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. Ecological 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

  8. Phosphatidylglycerol, lecithin/sphingomyelin ratio and respiratory distress syndrome in diabetic and non-diabetic pregnancies.

    PubMed

    Curet, L B; Tsao, F H; Zachman, R D; Olson, R W; Henderson, P A

    1989-10-01

    Samples of amniotic fluid from 514 non-diabetic and 69 diabetic patients were analyzed for phospholipid content. Results were correlated with incidence of respiratory distress syndrome (RDS) in the neonate. The incidence of RDS was 4.5% among diabetics and 5.3% among non-diabetics. In the presence of phosphatidylglycerol (PG), no infant developed RDS while in the absence of PG the incidence of RDS was 16.7% and 14.4%, respectively. In the presence of a mature lecithin/sphingomyelin (L/S) ratio the respective incidence of RDS was 1.6 and 1.8, while with an immature L/S ratio the incidence of RDS was 28.6% and 29%. The incidence of RDS after 37 weeks gestation was 0% among diabetics and 0.6% among non-diabetics. We conclude that amniotic fluid phospholipids are equally predictive of risk for RDS in diabetics as among non-diabetic patients. We suggest that in patients with accurate gestational dating, amniotic fluid analysis for phospholipids might not be necessary to establish fetal lung maturity. PMID:2572479

  9. Prescribing Patterns of Drugs in Acute Respiratory Distress Syndrome (ARDS): An Observational Study

    PubMed Central

    Rao, Shobitha; Chogtu, Bharti

    2015-01-01

    Introduction: Acute respiratory distress syndrome (ARDS) is characterized by acute respiratory failure and is associated with wide range of clinical disorders. Controversy prevails over the pharmacological intervention in this disease. The aim of the study was to observe the prescribing pattern of drugs in patients with ARDS managed at a tertiary care hospital. Materials and Methods: This observational study was conducted at tertiary care hospital in India. Data of patients admitted from January 2010 to December 2012 was collected. Patients aged more than 18 years admitted in ICU, who were diagnosed to have ARDS during the study period, were included. A total of 150 patients of ARDS were selected. Data was collected as per the pre designed proforma and it included patients’ age, gender, clinical disorders precipitating ARDS, prescribing pattern of drugs and outcome. The data of the subjects was collected till discharge from hospital or death. Results: Infection was the cause of ARDS in 81.3% (n=122) of subjects. Antibiotics were prescribed in all the subjects and beta-lactams were prescribed in 97.3% (n=146). 41.3% (n=62) were prescribed corticosteroids, 39.3% (n=59) diuretics and 89.3% (n=134) intravenous fluids. Conclusion: The outcome of patients on different pharmacological treatment did not show any statistically significant difference. PMID:25859465

  10. THE CURRENT STATUS OF THE RESPIRATORY DISTRESS SYNDROME OF THE NEWLY BORN.

    PubMed

    SWYER, P R; LEVISON, H

    1965-08-21

    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. PMID:14339292

  11. Measurement of pulmonary status and surfactant protein levels during dexamethasone treatment of neonatal respiratory distress syndrome.

    PubMed Central

    Wang, J. Y.; Yeh, T. F.; Lin, Y. C.; Miyamura, K.; Holmskov, U.; Reid, K. B.

    1996-01-01

    BACKGROUND: Early postnatal use of dexamethasone in infants with respiratory distress syndrome (RDS) has been shown effectively to improve pulmonary status and to allow early weaning off mechanical ventilation. However, the mechanisms to explain the beneficial effects of dexamethasone in ventilatory dependent preterm infants remain unclear. METHODS: A double blind, placebo controlled study was performed to determine the change in pulmonary ventilation of premature infants with RDS as a result of dexamethasone treatment, and to evaluate the effect of dexamethasone on the levels of surfactant-associated proteins A (SP-A) and D (SP-D) in the tracheal fluid from 34 premature infants with RDS and 29 control subjects. RESULTS: Dexamethasone treatment decreased fractional inspired oxygen concentration (FIO2), arterial carbon dioxide tension (PCO2), mean airway pressure (MAP), and facilitated successful weaning from mechanical ventilation. SP-A concentrations in the tracheal aspirates were increased at days 7 and 14, and SP-D concentrations were increased during the period from days 3 to 14 in the dexamethasone treated group compared with the control group. However, albumin levels in the tracheal aspirate samples were decreased after dexamethasone treatment over the period from days 3 to 14. There was an inverse correlation between PCO2 values and SP-A concentrations. CONCLUSIONS: These results suggest that early use of dexamethasone can improve pulmonary status and also increase SP-A and SP-D levels in the tracheal fluid in premature infants with RDS. PMID:8984701

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

    SciTech Connect

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

    1984-06-01

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

  13. Untargeted LC-MS Metabolomics of Bronchoalveolar Lavage Fluid Differentiates Acute Respiratory Distress Syndrome from Health

    PubMed Central

    Evans, Charles R.; Karnovsky, Alla; Kovach, Melissa A.; Standiford, Theodore J.; Burant, Charles F.; Stringer, Kathleen A.

    2014-01-01

    Acute respiratory distress syndrome (ARDS) remains a significant hazard to human health and is clinically challenging because there are no prognostic biomarkers and no effective pharmacotherapy. The lung compartment metabolome may detail the status of the local environment that could be useful in ARDS biomarker discovery and the identification of drug target opportunities. However, neither the utility of bronchoalveolar lavage fluid (BALF) as a biofluid for metabolomics nor the optimal analytical platform for metabolite identification are established. To address this, we undertook a study to compare metabolites in BALF samples from patients with ARDS and healthy controls using a newly developed liquid chromatography (LC)-mass spectroscopy (MS) platform for untargeted metabolomics. Following initial testing of three different high performance liquid chromatography (HPLC) columns, we determined that reversed phase (RP)-LC and hydrophilic interaction chromatography (HILIC), were the most informative chromatographic methods because they yielded the most and highest quality data. Following confirmation of metabolite identification, statistical analysis resulted in 37 differentiating metabolites in the BALF of ARDS compared with health across both analytical platforms. Pathway analysis revealed networks associated with amino acid metabolism, glycolysis and gluconeogenesis, fatty acid biosynthesis, phospholipids and purine metabolism in the ARDS BALF. The complementary analytical platforms of RPLC and HILIC-LC generated informative, insightful metabolomics data of the ARDS lung environment. PMID:24289193

  14. Pulmonary histopathology in dalmatians with familial acute respiratory distress syndrome (ARDS).

    PubMed

    Syrjä, P; Saari, S; Rajamäki, M; Saario, E; Järvinen, A-K

    2009-11-01

    The histopathological changes in the lungs of 12 related Dalmatians with idiopathic acute respiratory distress syndrome (ARDS) are described. Affected dogs had multiple foci of marked atypical hyperplasia and squamous metaplasia of the bronchiolar epithelium, patchy ongoing fibrosis with myofibroblastic metaplasia, smooth muscle hyperplasia and occasional honeycombing of alveolar walls, and hyperplasia of atypical type II pneumocytes. There was an abrupt transition between these proliferative lesions and areas of acute alveolar oedema with hyaline membranes in partially normal lung. Diseased areas were associated with moderate lymphohistiocytic interstitial inflammation. Immunohistochemical labelling for cytokeratin expression indicated that the metaplastic epithelium was of bronchiolar origin and that it extended into peribronchiolar alveolar spaces. Some of the bronchiolar lesions were pre-neoplastic and one adult dog suffered from bronchoalveolar carcinoma. These lesions are compared with the two forms of idiopathic interstitial pneumonia reported as causes of ARDS in man: acute interstitial pneumonia (AIP) and acute exacerbation of idiopathic pulmonary fibrosis (IPF). The observed lesions in the Dalmatians are distinct from the diffuse alveolar damage that characterizes AIP, but show some histological similarities to the usual interstitial pneumonia (UIP) that occurs in IPF with acute exacerbation in man. UIP has not previously been described in the dog.

  15. Necrostatin-1 protects against oleic acid-induced acute respiratory distress syndrome in rats.

    PubMed

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

    2016-09-30

    Necroptosis is a recently discovered necrotic cell death which is regulated by receptor interacting protein kinase 1 (RIPK1) and RIPK3 under the stimulus of death signal and can be inhibited by necrostatin-1 (Nec-1) specifically. Therefore, the aim was to investigate the role of necroptosis in a rat model of acute respiratory distress syndrome (ARDS) induced by oleic acid (OA) and assess the effect of Nec-1 on lung injury in ARDS. Our results found that RIPK1, RIPK3 and mixed lineage kinase domain-like protein (MLKL) were abundantly expressed in rat lung tissues of OA-induced ARDS. Nec-1 pretreatment improved pulmonary function and attenuated lung edema dramatically in OA-induced ARDS rats. Furthermore, Nec-1 reduced RIPK1-RIPK3 interaction and down-regulated RIPK1-RIPK3-MLKL signal pathway, and inhibited inflammatory response by reducing neutrophil infiltration and protein leakage into lung tissue in OA-induced ARDS. Collectively, our study proves the intervention of necroptosis in OA-induced ARDS. Moreover, our findings imply that Nec-1 plays an important role in the treatment of ARDS via inhibiting necroptosis and inflammation. PMID:27586277

  16. [The basics on mechanical ventilation support in acute respiratory distress syndrome].

    PubMed

    Tomicic, V; Fuentealba, A; Martínez, E; Graf, J; Batista Borges, J

    2010-01-01

    Acute Respiratory Distress Syndrome (ARDS) is understood as an inflammation-induced disruption of the alveolar endothelial-epithelial barrier that results in increased permeability and surfactant dysfunction followed by alveolar flooding and collapse. ARDS management relies on mechanical ventilation. The current challenge is to determine the optimal ventilatory strategies that minimize ventilator-induced lung injury (VILI) while providing a reasonable gas exchange. The data support that a tidal volume between 6-8 ml/kg of predicted body weight providing a plateau pressure < 30 cmH₂O should be used. High positive end expiratory pressure (PEEP) has not reduced mortality, nevertheless secondary endpoints are improved. The rationale used for high PEEP argues that it prevents cyclic opening and closing of airspaces, probably the major culprit of development of VILI. Chest computed tomography has contributed to our understanding of anatomic-functional distribution patterns in ARDS. Electric impedance tomography is a technique that is radiation-free, but still under development, that allows dynamic monitoring of ventilation distribution at bedside.

  17. 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

  18. Noninvasive Ventilation for Preterm Twin Neonates with Respiratory Distress Syndrome: A Randomized Controlled Trial

    PubMed Central

    Chen, Long; Wang, Li; Li, Jie; Wang, Nan; Shi, Yuan

    2015-01-01

    Noninvasive ventilation has been proven to be effective strategies for reducing the need for endotracheal ventilation in preterm infant with respiratory distress syndrome (RDS), however the best option needs to be further determined. A single center, paired design, randomized, controlled trial was conducted between Jan 2011 and July 2014. Preterm twins with RDS were included. One of a pair was randomized to NIPPV, while another to NCPAP. Surfactant was administrated as rescue treatment. The primary outcome was the need for endotracheal ventilation. The secondary outcomes were the complications. 143 pairs were randomized and 129 pairs finished the trial. The rates of endotracheal ventilation did not differ significantly between NIPPV and NCPAP groups (11.9% vs 19.6%, P = 0.080). This difference was not observed in the subgroup of infants who received surfactant therapy (11.1% vs 19.7%, P = 0.087). No secondary outcomes also differed significantly between the two groups. NIPPV did not result in a significantly lower incidence of intubation as compared with NCPAP in preterm twins with RDS. PMID:26399752

  19. 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.

  20. MicroRNA Regulation of Acute Lung Injury and Acute Respiratory Distress Syndrome.

    PubMed

    Rajasekaran, Subbiah; Pattarayan, Dhamotharan; Rajaguru, P; Sudhakar Gandhi, P S; Thimmulappa, Rajesh K

    2016-10-01

    The acute respiratory distress syndrome (ARDS), a severe form of acute lung injury (ALI), is a very common condition associated with critically ill patients, which causes substantial morbidity and mortality worldwide. Despite decades of research, effective therapeutic strategies for clinical ALI/ARDS are not available. In recent years, microRNAs (miRNAs), small non-coding molecules have emerged as a major area of biomedical research as they post-transcriptionally regulate gene expression in diverse biological and pathological processes, including ALI/ARDS. In this context, this present review summarizes a large body of evidence implicating miRNAs and their target molecules in ALI/ARDS originating largely from studies using animal and cell culture model systems of ALI/ARDS. We have also focused on the involvement of miRNAs in macrophage polarization, which play a critical role in regulating the pathogenesis of ALI/ARDS. Finally, the possible future directions that might lead to novel therapeutic strategies for the treatment of ALI/ARDS are also reviewed. J. Cell. Physiol. 231: 2097-2106, 2016. © 2016 Wiley Periodicals, Inc.

  1. 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

  2. Therapeutic modulation of coagulation and fibrinolysis in acute lung injury and the acute respiratory distress syndrome.

    PubMed

    Sebag, Sara C; Bastarache, Julie A; Ware, Lorraine B

    2011-09-01

    Acute respiratory distress syndrome (ARDS) and acute lung injury (ALI) are characterized by excessive intraalveolar fibrin deposition, driven, at least in part by inflammation. The imbalance between activation of coagulation and inhibition of fibrinolysis in patients with ALI/ARDS favors fibrin formation and appears to occur both systemically and in the lung and airspace. Tissue factor (TF), a key mediator of the activation of coagulation in the lung, has been implicated in the pathogenesis of ALI/ARDS. As such, there have been numerous investigations modulating TF activity in a variety of experimental systems in order to develop new therapeutic strategies for ALI/ARDS. This review will summarize current understanding of the role of TF and other proteins of the coagulation cascade as well the fibrinolysis pathway in the development of ALI/ARDS with an emphasis on the pathways that are potential therapeutic targets. These include the TF inhibitor pathway, the protein C pathway, antithrombin, heparin, and modulation of fibrinolysis through plasminogen activator- 1 (PAI-1) or plasminogen activators (PA). Although experimental studies show promising results, clinical trials to date have proven unsuccessful in improving patient outcomes. Modulation of coagulation and fibrinolysis has complex effects on both hemostasis and inflammatory pathways and further studies are needed to develop new treatment strategies for patients with ALI/ARDS. PMID:21401517

  3. 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

  4. Glucocorticoid levels in maternal and cord serum after prenatal betamethasone therapy to prevent respiratory distress syndrome.

    PubMed Central

    Ballard, P L; Granberg, P; Ballard, R A

    1975-01-01

    Serum glucocorticoid levels were determined in 20 mothers and 43 premature infants who received prenatal betamethasone therapy for prevention of respiratory distress syndrome (RDS). Maternal betamethasone peaked at 75 microg cortisol equivalents per 100 ml 1 h after injection of 12 mg steroid and declined to half by 6 h. Betamethasone in cord blood was 14.3 microg cortisol equivalents per 100 ml at 1 h, decreased to a level of 4.7 at 20 h, and was not detected 2 days after a second dose at 24 h. After the second dose, the mean level of cortisol in cord blood was 5.9 microg per 100 ml compared with 13.05 microg per 100 ml (p less than 0.001) in untreated premature infants. The unbound glucocorticoid activity in treated infants delivered 1-10 h after the second dose (mean, 8.4 microg per 100 ml) is similar to the unbound cortisol level after birth in untreated premature infants who develop RDS. These findings indicate that (a) serum glucocorticoid levels in the physiologic stress range can induce lung maturation in the human and (b) antenatal treatment with this dose of betamethasone does not expose the human fetus to potentially harmful pharmacologic levels of steroid. PMID:1202085

  5. [Cauda equina syndrome due to giant disc herniation].

    PubMed

    Barriga, A; Villas, C

    2002-01-01

    In cases of acute or progressive development in a few hours of bilateral sciatica, severe foot and occasional quadriceps weakness and/or retention or incontinence of urine with perineal hypalgesia or anesthesia, acute compression of the cauda equina should be suspected, which is usually due to a lumbar disc herniation. Cauda equina syndrome requires emergency spinal surgery. To identify and confirm this syndrome by MR, Ismanoatory. Early surgical decompression must be achieved. Decompression within 24-48 hours significantly improves the neurological and urological outcome. We present the case of a patient who had previously been treated for low back pain who developed a cauda equina syndrome a few days later. PMID:12685115

  6. [Skin manifestations of toxic syndrome due to denatured rapeseed oil].

    PubMed

    Fonseca, E

    2009-12-01

    This article offered an extensive description of the clinical and pathological features and time-course of the skin manifestations of toxic syndrome caused by denatured rapeseed oil, also known as toxic oil syndrome. This new condition occurred in Spain in 1981 and was due to the ingestion of rapeseed oil intended for industrial use that had been denatured with anilines and subsequently refined and sold fraudulently as olive oil. In total, 20,000 cases and 400 deaths were reported. The disease affected mainly women, particularly in the late stages. In the acute phase, the predominant skin manifestations were toxic-allergic rashes reminiscent of allergic urticaria in the dermatopathologic study. In approximately 25% of cases, the patients' skin subsequently took on an edematous appearance, with pigmentary abnormalities shown to be related to cutaneous mucinosis. Finally, a characteristic sclerodermatous condition would develop that tended to improve spontaneously. The constant presence of mast cells in all biopsies and the development of mastocytosis in several patients pointed to an important role for these cells in the pathogenesis of the condition. This was subsequently confirmed in other sclerodermatous processes. In 1989, eosinophilia-myalgia syndrome caused by toxins present in tryptophan food supplements was reported in the United States. This syndrome resembled toxic oil syndrome in many ways and demonstrated that mucinosis and toxic sclerodermatous processes do exist.

  7. Interleukin 8-related neutrophil elastase and the severity of the adult respiratory distress syndrome.

    PubMed

    Groeneveld, A B; Raijmakers, P G; Hack, C E; Thijs, L G

    1995-10-01

    The interaction between activated neutrophils and pulmonary endothelium is thought to contribute to the pathogenesis of the adult respiratory distress syndrome (ARDS), but its relation to ARDS severity, which may support a pathogenetic role, is unclear. Therefore, circulating inflammatory mediators, including the neutrophil chemoattractant and activator interleukin 8 (IL-8), the acute phase cytokine IL-6, and the neutrophil product elastase complexed to alpha 1-antitrypsin (alpha 1-AT), were measured prospectively, together with gas exchange, ventilatory and radiographic variables, in 13 mechanically ventilated patients with ARDS, mostly owing to sepsis, at admission into the intensive care unit. Measurements were repeated in the eight improving patients at the time that positive end-expiratory pressure could be reduced to 0 cm H2O. From the gas exchange, ventilatory and radiographic abnormalities, a lung injury score (LIS) was calculated. For pooled data, the LIS and the arterial PO2/inspiratory O2 fraction, the oxygenation ratio, correlated with plasma levels of IL-8 (rs = 0.60, P < 0.01 and rs = -0.65, P < 0.005, respectively), with levels of IL-6 (rs = 0.60, P < 0.01, and rs = -0.68, P < 0.005, respectively), and the oxygenation ratio related to elastase-alpha 1-AT (rs = -0.70, P < 0.005). Levels of IL-8 and IL-6 interrelated (rs = 0.61, P < 0.01) and related to the elastase complexes (rs = 0.45, P < 0.05). Hence, our data support a role of cytokine-induced activation of neutrophils in the clinical severity of ARDS.

  8. [Pathophysiologic and therapeutic aspects of the adult respiratory distress syndrome (ARDS)].

    PubMed

    Thiel, M; Forst, H; Peter, K

    1991-02-01

    Since the first characterization of the adult respiratory distress syndrome (ARDS), knowledge of its aetiology and pathogenesis has grown considerably. In spite of this, mortality remains up to 50 to 90%, particularly if multiple organ failure is present. Because no causative clinical therapy is available up to now, significant attention is given to preventive measures like early operative stabilisation of long bone fractures, or prophylaxis of nosocomial infections. After clinical manifestation of ARDS, treatment focuses on functional disturbances of the cardiopulmonary system and on the underlying disease. The aim of this symptomatic therapy is to ensure oxygen supply according to the organisms demand. It is still unknown, however, whether the mortality of patients with ARDS can be reduced by optimising the oxygen supply. In general, oxygen supply can be enhanced by improving pulmonary gas exchange, cardiac output and blood oxygen transport capacity. For practical use the therapy often ends up with a therapeutical dilemma: On one hand, the improvement of the pulmonary gas exchange by application of PEEP can be associated with a critical decline in cardiac output, particularly if the afterload of the right ventricle is elevated. On the other hand, to increase cardiac output, both volume replacement and vasodilators can severely affect pulmonary gas exchange if the alveolo-capillary permeability is increased and pulmonary hypoxic vasoconstriction is disturbed. Thus, oxygen supply can be optimised only via invasive monitoring of the cardiorespiratory system. Although still experimental, the most promising approaches seem to be pharmacological interventions directed at suppressing the formation and effects of various humoral and cellular mediators. An improved understanding of the inflammatory processes might provide new insights in the pathophysiology of ARDS and the related therapeutic interventions. PMID:1863681

  9. Effect of intrauterine cocaine exposure on respiratory distress syndrome in very low birthweight infants.

    PubMed Central

    Beeram, M. R.; Abedin, M.; Young, M.; Leftridge, C.; Dhanireddy, R.

    1994-01-01

    To evaluate the effect of intrauterine cocaine exposure on lung maturity of very low birthweight infants, the medical records of all infants with birthweight < 1500 g born between January 1989 and December 1990 at DC General Hospital were reviewed. Infants with conditions known to cause lung maturity, severe congenital anomalies, proven early sepsis, and birthweight > or = 500 g were excluded. A total of 69 infants were included in the study. Chest roentgenograms of these infants were evaluated by a pediatric radiologist, who was unaware of the infant's medical course, for evidence of respiratory distress syndrome (RDS), and radiological findings were correlated with clinical signs. Forty infants were exposed to cocaine in utero (cocaine group) and 29 were not exposed (noncocaine group). African-American ethnicity, pregnancy-induced hypertension, prolonged rupture of membranes, and alcohol use were similar in both groups. Tobacco use among cocaine group mothers was higher (42.5% versus 13.8%; P = .01). Gestational age (28.3 +/- 2.8 versus 28.3 +/- 3 weeks), birthweight (966 +/- 282 versus 1059 +/- 295 g), male gender, and Apgar scores were similar in both groups. Thirty (75%) infants in the cocaine group developed RDS compared with 19 (66%) in the noncocaine group (P > .05). Using multiple logistic regression analysis and controlling for smoking, alcohol use, and prolonged rupture of membranes (24 to 72 hours), the incidence of RDS between the groups remained statistically insignificant. We conclude that intrauterine cocaine exposure does not alter the incidence of RDS in very low birthweight infants. PMID:8046765

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

    PubMed Central

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

    2016-01-01

    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. PMID:26955891

  11. Titration of tidal volume and induced hypercapnia in acute respiratory distress syndrome.

    PubMed

    Roupie, E; Dambrosio, M; Servillo, G; Mentec, H; el Atrous, S; Beydon, L; Brun-Buisson, C; Lemaire, F; Brochard, L

    1995-07-01

    Mechanical ventilation may promote overdistension-induced pulmonary lesions in patients with acute respiratory distress syndrome (ARDS). The static pressure-volume (P-V) curve of the respiratory system can be used to determine the lung volume and corresponding static airway pressure at which lung compliance begins to diminish (the upper inflection point, or UIP). This fall in compliance may indicate overdistension of lung units. We prospectively studied 42 patients receiving mechanical ventilation with an FIO2 of 0.5 or more for at least 24 h. According to the Lung Injury Score (LIS), 25 patients were classified as having ARDS (LIS > 2.5), while 17 patients constituted a non-ARDS control group. The P-V curve was obtained every 2 d. Mechanical ventilation initially used standard settings (volume-control mode, a positive end-expiratory pressure [PEEP] adjusted to the lower inflection point on the P-V curve, and a tidal volume [VT] of 10 ml/kg). The end-inspiratory plateau pressure (Pplat) was compared to the UIP, and VT was lowered when the Pplat was above the UIP. In the range of lung volume studied on the P-V curves (up to 1600 ml), a UIP could be shown in only one control patient (at 23 cm H2O). By contrast, a UIP was present on the P-V curve obtained from all patients with ARDS, corresponding to a mean airway pressure of 26 +/- 6 cm H2O, a lung volume of 850 +/- 200 ml above functional residual capacity and 610 +/- 235 ml above PEEP.(ABSTRACT TRUNCATED AT 250 WORDS) PMID:7599810

  12. Surfactant chemical composition and biophysical activity in acute respiratory distress syndrome.

    PubMed Central

    Gregory, T J; Longmore, W J; Moxley, M A; Whitsett, J A; Reed, C R; Fowler, A A; Hudson, L D; Maunder, R J; Crim, C; Hyers, T M

    1991-01-01

    Acute Respiratory Distress Syndrome (ARDS) is characterized by lung injury and damage to the alveolar type II cells. This study sought to determine if endogenous surfactant is altered in ARDS. Bronchoalveolar lavage was performed in patients at-risk to develop ARDS (AR, n = 20), with ARDS (A, n = 66) and in normal subjects (N, n = 29). The crude surfactant pellet was analyzed for total phospholipids (PL), individual phospholipids, SP-A, SP-B, and minimum surface tension (STmin). PL was decreased in both AR and A (3.48 +/- 0.61 and 2.47 +/- 0.40 mumol/ml, respectively) compared to N (7.99 +/- 0.60 mumol/ml). Phosphatidylcholine was decreased in A (62.64 +/- 2.20% PL) compared to N (76.27 +/- 2.05% PL). Phosphatidylglycerol was 11.58 +/- 1.21% PL in N and was decreased to 6.48 +/- 1.43% PL in A. SP-A was 123.64 +/- 20.66 micrograms/ml in N and was decreased to 49.28 +/- 21.68 micrograms/ml in AR and to 29.88 +/- 8.49 micrograms/ml in A. SP-B was 1.28 +/- 0.33 micrograms/ml in N and was decreased to 0.57 +/- 0.24 micrograms/ml in A. STmin was increased in AR (15.1 +/- 2.53 dyn/cm) and A (29.04 +/- 2.05 dyn/cm) compared to N (7.44 +/- 1.61 dyn/cm). These data demonstrate that the chemical composition and functional activity of surfactant is altered in ARDS. Several of these alterations also occur in AR, suggesting that these abnormalities occur early in the disease process. PMID:1752956

  13. 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. PMID:26660667

  14. 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)

  15. Aspiration-Related Acute Respiratory Distress Syndrome in Acute Stroke Patient

    PubMed Central

    Zhao, Jiang-nan; Liu, Yao; Li, Huai-chen

    2015-01-01

    Background Aspiration of oral or gastric contents into the larynx and lower respiratory tract is a common problem in acute stroke patients, which significantly increases the incidence of acute respiratory distress syndrome (ARDS). However, little is known about the clinical characteristics of aspiration-related ARDS in acute stroke patients. Methods Over 17-month period a retrospective cohort study was done on 1495 consecutive patients with acute stroke. The data including demographic characteristics, clinical manifestations, laboratory examinations, chest imaging, and hospital discharge status were collected to analysis. Results Aspiration-related ARDS was diagnosed in 54 patients (3.6%). The most common presenting symptom was tachypnea (respiratory rate ≥25 breaths/min) in 50 cases. Computed tomography (CT) images usually demonstrated diffuse ground-glass opacities (GGOs) and inhomogeneous patchy consolidations involving the low lobes. Age, NIHSS score, GCS score, dysphagia, dysarthria, hemoglobin concentration, serum aspertate aminotransferase (AST), serum albumin, serum sodium, and admission glucose level were independently associated with aspiration-related ARDS (odds ratio (OR) 1.05, 95% confidence interval (CI) (1.04–1.07); OR 2.87, (2.68–3.63); OR 4.21, (3.57–5.09); OR 2.18, (1.23–3.86); OR 1.67, (1.31–2.14); OR 2.31, (1.11–4.84); OR 1.68, (1.01–2.80); OR 2.15, (1.19–3.90); OR 1.92, (1.10–3.36) and OR 1.14, (1.06–1.21) respectively). Conclusions Aspiration-related ARDS frequently occurs in acute stroke patient with impairment consciousness. It is advisable that performing chest CT timely may identify disease early and prompt treatment to rescue patients. PMID:25790377

  16. A preterm pig model of lung immaturity and spontaneous infant respiratory distress syndrome.

    PubMed

    Caminita, Frank; van der Merwe, Marie; Hance, Brittany; Krishnan, Ramesh; Miller, Sarah; Buddington, Karyl; Buddington, Randal K

    2015-01-15

    Respiratory distress syndrome (RDS) and bronchopulmonary dysplasia remain the leading causes of preterm infant morbidity, mortality, and lifelong disability. Research to improve outcomes requires translational large animal models for RDS. Preterm pigs delivered by caesarian section at gestation days (GD) 98, 100, 102, and 104 were provided 24 h of neonatal intensive care, monitoring (pulse oximetry, blood gases, serum biomarkers, radiography), and nutritional support, with or without intubation and mechanical ventilation (MV; pressure control ventilation with volume guarantee). Spontaneous development of RDS and mortality without MV are inversely related with GD at delivery and correspond with inadequacy of tidal volume and gas exchange. GD 98 and 100 pigs have consolidated lungs, immature alveolar architecture, and minimal surfactant protein-B expression, and MV is essential at GD 98. Although GD 102 pigs had some alveoli lined by pneumocytes and surfactant was released in response to MV, blood gases and radiography revealed limited recruitment 1-2 h after delivery, and mortality at 24 h was 66% (35/53) with supplemental oxygen provided by a mask and 69% (9/13) with bubble continuous positive airway pressure (8-9 cmH2O). The lungs at GD 104 had higher densities of thin-walled alveoli that secreted surfactant, and MV was not essential. Between GD 98 and 102, preterm pigs have ventilation inadequacies and risks of RDS that mimic those of preterm infants born during the saccular phase of lung development, are compatible with standards of neonatal intensive care, and are alternative to fetal nonhuman primates and lambs.

  17. 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

  18. Positive predictive value of the infant respiratory distress syndrome diagnosis in the Danish National Patient Registry

    PubMed Central

    Thygesen, Sandra Kruchov; Olsen, Morten; Christian, Fynbo Christiansen

    2013-01-01

    Background Infant respiratory distress syndrome (IRDS) is the most common respiratory disease in preterm infants, and is associated with considerable morbidity and mortality. Valid data on IRDS are important in clinical epidemiological research. Objectives The objective of this study was to estimate the positive predictive value (PPV) of the IRDS diagnosis registered in the population-based Danish National Patient Registry according to the International Classification of Diseases, 8th and 10th revisions. Methods Between January 1, 1977 and December 31, 2008, we randomly selected three patients per year, 96 in total, who were registered with an IRDS diagnosis in the Danish National Patient Registry and living in the northern part of Denmark. Data on the infants included information on the presence of predefined clinical symptoms. We defined IRDS as the presence of at least two of four clinical symptoms (tachypnea, retractions or nasal flaring, grunting, and central cyanosis), which had to be present for more than 30 minutes. Using medical record review as the reference standard, we computed the positive predictive value of the registered IRDS diagnosis including 95% confidence intervals (CIs). Results We located the medical record for 90 of the 96 patients (94%), and found an overall PPV of the IRDS diagnosis of 81% (95% CI 72%–88%). This did not vary substantially between primary and secondary diagnoses. The PPV was higher, at 89% (95% CI 80%–95%), for preterm infants born before 37 weeks of gestation. Conclusion The PPV of the IRDS diagnosis in the Danish National Patient Registry is reasonable when compared with symptoms described in the corresponding medical records. The Danish National Patient Registry is a useful data source for studies of IRDS, particularly if restricted to preterm infants. Nonetheless, the potential impact of misclassification of the IRDS diagnosis must be considered. PMID:23976865

  19. 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

  20. Characterization of oleic acid-induced acute respiratory distress syndrome model in rat.

    PubMed

    Akella, Aparna; Sharma, Parul; Pandey, Ratna; Deshpande, Shripad B

    2014-07-01

    Animal studies using oleic acid (OA) model to produce acute respiratory distress syndrome (ARDS) have been inconsistent. Therefore, the present study was undertaken to establish an acute model of ARDS in rats using OA and to characterize its effect on cardio-respiratory parameters and lethality. The trachea, jugular vein and femoral artery of anesthetized adult rats were cannulated. A dose of OA (30-90 microL; iv) was injected in each animal and changes in respiratory frequency (RF), heart rate (HR) and mean arterial pressure (MAP) were recorded. Minute ventilation and PaO2/FiO2 (P/F) ratio were also determined. At the end, lungs were excised for determination of pulmonary water content and histological examination. At all doses of OA, there was immediate decrease followed by increase in RF, however at 75 and 90 microL of OA, RF decreased abruptly and the animals died by 63 +/- 8.2 min and 19 +/- 6.3 min; respectively. In all the groups, HR and MAP changes followed the respiratory changes. The minute ventilation increased in a dose-dependent manner while the values of P/F ratio decreased correspondingly. Pulmonary edema was induced at all doses. Histological examination of the lung showed alveolar damage, microvascular congestion, microvascular injury, infiltration of inflammatory cells, pulmonary edema and necrosis in a dose-dependent manner. With these results, OA can be used to induce different grades of ARDS in rats and OA doses of 50, 60 and 75 microL resemble mild, moderate and severe forms of ARDS respectively. Hence, OA model serves as a useful tool to study the pathophysiology of ARDS.

  1. Unexpected Role for Adaptive αβTH17 Cells in Acute Respiratory Distress Syndrome1

    PubMed Central

    Li, John T.; Melton, Andrew C.; Su, George; Hamm, David E.; LaFemina, Michael; Howard, James; Fang, Xiaohui; Bhat, Sudarshan; Huynh, Kieu-My; O’Kane, Cecilia M.; Ingram, Rebecca J.; Muir, Roshell R.; McAuley, Daniel F.; Matthay, Michael A.; Sheppard, Dean

    2015-01-01

    Acute respiratory distress syndrome (ARDS) is a devastating disorder characterized by increased alveolar permeability with no effective treatment beyond supportive care. Current mechanisms underlying ARDS focus on alveolar endothelial and epithelial injury caused by products of innate immune cells and platelets. However, the role of adaptive immune cells in ARDS remains largely unknown. Here we report that expansion of antigen-specific αβT helper 17 (αβTH17) cells contribute to ARDS by local secretion of IL-17A, which in turn directly increases alveolar epithelial permeability. Mice with a highly restrictive defect in antigen-specific αβTH17 cells were protected from experimental ARDS induced by a single dose of endotracheal lipopolysaccharide (LPS). Loss of IL-17 receptor C or antibody blockade of IL-17A was similarly protective, further suggesting that IL-17A released by these cells was responsible for this effect. LPS induced a rapid and specific clonal expansion of αβTH17 cells in the lung, as determined by deep sequencing of the hypervariable CD3RβVJ region of the T cell receptor. Our findings could be relevant to ARDS in humans, since we found significant elevation of IL-17A in bronchoalveolar lavage (BAL) fluid from patients with ARDS and recombinant IL-17A directly increased permeability across cultured human alveolar epithelial monolayers. These results reveal a previously unexpected role for adaptive immune responses that increase alveolar permeability in ARDS and suggest that αβTH17 cells and IL-17A could be novel therapeutic targets for this currently untreatable disease. PMID:26002979

  2. 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

  3. Circulating nucleosomes are associated with mortality in pediatric acute respiratory distress syndrome.

    PubMed

    Yehya, Nadir; Thomas, Neal J; Margulies, Susan S

    2016-06-01

    Mechanisms underlying pediatric acute respiratory distress syndrome (PARDS) are poorly understood. The recent implication of circulating nucleosomes as pathogenic in sepsis and trauma-associated ARDS in adults led us to investigate the significance of nucleosomes in PARDS. We conducted a prospective, observational study on children with PARDS at the Children's Hospital of Philadelphia between July 2014 and September 2015. Plasma was collected within 48 h of PARDS onset and nucleosomes quantified by enzyme-linked immunosorbent assay. Samples from 76 children with PARDS (11 deaths, 14%) were collected early [median 15 (IQR 7, 21) h] after PARDS onset. Nucleosome levels were higher in nonsurvivors [0.59 AU (IQR 0.46, 0.84)] relative to survivors [0.21 AU (IQR 0.08, 0.33), rank sum P < 0.001]. Nucleosome levels were not associated with either Berlin (P = 0.845) or PALICC (P = 0.886) oxygenation categories, nor with etiology of PARDS (P = 0.527). Nucleosomes were correlated with increasing numbers of nonpulmonary organ failures (P = 0.009 for trend), and were higher in patients whose PaO2 /FiO2 worsened (P = 0.012) over the first 72 h of PARDS. In regression analysis, nucleosome levels were independently associated with mortality after adjusting for either age, severity of illness score, number of nonpulmonary organ failures, vasopressor score, or PaO2 /FiO2 (all P < 0.05). In conclusion, plasma nucleosome levels in early PARDS were associated with increased mortality, correlated with number of nonpulmonary organ failures, and preceded worsening oxygenation. The potential utility of this biomarker for prognostication, risk stratification, and mechanistic insight should be investigated further. PMID:27130528

  4. Patient characteristics and frequency of bodily distress syndrome in primary care: a cross-sectional study

    PubMed Central

    Budtz-Lilly, Anna; Vestergaard, Mogens; Fink, Per; Carlsen, Anders Helles; Rosendal, Marianne

    2015-01-01

    Background Bodily distress syndrome (BDS) is a newly proposed diagnosis of medically unexplained symptoms, which is based on empirical research in primary care. Aim To estimate the frequency of BDS in primary care and describe the characteristics of patients with BDS. Design and setting A cross-sectional study of primary care patients in urban and rural areas of Central Denmark Region. Method Data were obtained from GP one-page registration forms, patient questionnaires (including a checklist for BDS), and national registers. Results A total of 1356 primary care patients were included, of whom 230 patients (17.0%, 95% confidence intervals [CI] = 15.0 to 19.1) fulfilled the BDS criteria. BDS was more common among primary care patients aged 41–65 years (odds ratio [OR] = 1.9, 95% CI = 1.3 to 3.0) and was equally frequent among males and females (female sex, OR 0.9, 95% CI = 0.6 to 1.3). Patients with BDS were characterised by poor health-related quality of life (HRQOL) on the 12-item Short-Form Health Survey, that is, physical component summary scores <40 (OR 20.5, 95% CI = 12.9 to 32.4) and mental component summary scores <40 (OR 3.5, 95% CI = 2.2 to 5.6). Furthermore, patients with BDS were more likely to have high scores on the Symptom Checklist for anxiety (OR 2.2, 95% CI = 1.4 to 3.4) and depression (OR 5.1, 95% CI = 3.3 to 7.9), but regression analyses showed that mental morbidity did not account for the poor HRQOL. Conclusion BDS is common among primary care patients, and patients with BDS have a higher probability of poor HRQOL and mental health problems. PMID:26324499

  5. Persistent pulmonary hypertension in premature neonates with severe respiratory distress syndrome.

    PubMed

    Walther, F J; Benders, M J; Leighton, J O

    1992-12-01

    Cardiac catheterization studies have demonstrated that Doppler-derived flow velocities in the ductal flow jet and the left pulmonary artery accurately predict the aortopulmonary pressure difference and left-to-right shunt size in newborns. To assess the presence of persistent pulmonary hypertension in premature newborns with various degrees of respiratory distress syndrome (RDS) severity, we estimated pulmonary artery pressure from the aortopulmonary pressure difference and pulmonary blood flow from the left pulmonary artery flow velocity with color-flow-directed, pulsed Doppler echocardiography. Seventy-nine premature neonates were divided into three groups--no or mild RDS (n = 27), severe RDS (n = 38), and fatal RDS (n = 14)--and compared with a group of healthy term neonates (n = 34). In premature and term neonates with no/mild RDS the mean +/- SEM aortopulmonary pressure difference increased from 7.3 +/- 0.4 and 6.6 +/- 0.5 mm Hg to 22.8 +/- 1.4 and 21.4 +/- 1.1 mm Hg over the first 24 hours (P < .001). The mean aortopulmonary pressure difference was 0.9 +/- 0.3 mm Hg during the first 72 hours in neonates with fatal RDS, but increased from 1.5 +/- 0.3 mm Hg at 4 hours to 7.4 +/- 0.6 at 24 hours and 21.5 +/- 0.7 mm Hg at 72 hours of age in neonates with severe RDS. Left pulmonary artery velocity time integrals were 18.3 +/- 0.5 cm in premature and 18.8 +/- 0.5 cm in term neonates with no/mild RDS at 12 hours vs 11.2 +/- 0.4 cm in neonates with severe and 9.9 +/- 0.5 cm in neonates with fatal RDS (P < .001).(ABSTRACT TRUNCATED AT 250 WORDS)

  6. 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

  7. 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

  8. Moral distress and Burnout syndrome: are there relationships between these phenomena in nursing workers?1

    PubMed Central

    Dalmolin, Graziele de Lima; Lunardi, Valéria Lerch; Lunardi, Guilherme Lerch; Barlem, Edison Luiz Devos; da Silveira, Rosemary Silva

    2014-01-01

    Objective to identify relationships between moral distress and Burnout in the professional performance from the perceptions of the experiences of nursing workers. Methods this is a survey type study with 375 nursing workers working in three different hospitals of southern Rio Grande do Sul, with the application of adaptations of the Moral Distress Scale and the Maslach Burnout Inventory, validated and standardized for use in Brazil. Data validation occurred through factor analysis and Cronbach's alpha. For the data analysis bivariate analysis using Pearson's correlation and multivariate analysis using multiple regression were performed. Results the existence of a weak correlation between moral distress and Burnout was verified. A possible positive correlation between Burnout and therapeutic obstinacy, and a negative correlation between professional fulfillment and moral distress were identified. Conclusion the need was identified for further studies that include mediating and moderating variables that may explain more clearly the models studied. PMID:24553701

  9. A Case of Scrub Typhus Complicated by Adult Respiratory Distress Syndrome and Successful Management with Extracorporeal Membrane Oxygenation.

    PubMed

    Choi, Woo Young; Lee, Seung Yun; Kwon, Hea Yoon; Im, Jae Hyoung; Durey, Areum; Baek, Ji Hyeon; Kim, Young Sam; Kang, Jae-Seung; Lee, Jin-Soo

    2016-09-01

    A 67-year-old woman was diagnosed as having scrub typhus with pneumonitis. On admission, she was started on a combination therapy with levofloxacin and doxycycline. However, the patient developed severe acute respiratory distress syndrome (ARDS) on the 2nd day, and as a result, she underwent extracorporeal membrane oxygenation (ECMO). She was weaned from ECMO on the 10th day, as her respiratory status gradually improved. She was discharged without sequelae on the 23rd day. The outcome suggests that the use of ECMO should be considered for patients with ARDS induced from scrub typhus. PMID:27458040

  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. Acute respiratory failure due to refeeding syndrome and hypophosphatemia induced by hypocaloric enteral nutrition.

    PubMed

    Patel, Utpal; Sriram, Krishnan

    2009-03-01

    We report a case of acute respiratory failure due to refeeding syndrome caused by hypocaloric enteral tube feeds. A 60-y-old obese man, with a diagnosis of esophageal carcinoma with local metastases, underwent feeding jejunostomy tube insertion. Enteral tube feeding was initiated at small volumes providing 4.4 kcal x kg(-1) x d(-1) and gradually increased over 48 h to 29 kcal x kg(-1) x d(-1) (based on adjusted body weight). The patient then developed acute respiratory distress requiring intubation and ventilatory support. Serum phosphorus (P) level was extremely low at <0.7 mg/dL. Serum potassium (K) and magnesium (Mg) levels were also low. It took >4 d to adequately correct the electrolyte derangements. Successful liberation from mechanical ventilation was then possible. In chronically malnourished patients undergoing nutritional support, even hypocaloric feeding should be considered a risk factor for developing refeeding syndrome leading to severe and acute electrolyte fluid-balance and metabolic abnormalities.

  12. The relationship between clinico-biochemical characteristics and psychiatric distress in young women with polycystic ovary syndrome.

    PubMed

    Adali, E; Yildizhan, R; Kurdoglu, M; Kolusari, A; Edirne, T; Sahin, H G; Yildizhan, B; Kamaci, M

    2008-01-01

    The relationship between clinico-biochemical characteristics and self reported psychological parameters in 42 women with polycystic ovary syndrome (PCOS) and 42 age-matched healthy controls was examined. The General Health Questionnaire was used (GHQ-12) to ascertain emotional distress and the Beck Depression Inventory (BDI) to determine depressive symptoms. Emotional distress, depressive symptoms, hirsutism score, body mass index (BMI), waist-to-hip ratio (WHR), luteinizing hormone/follicle-stimulating hormone ratio, serum total testosterone, dehydroepiandrosterone sulphate levels and the insulin resistance index were significantly greater in women with PCOS than in healthy women. The BDI and GHQ-12 scores of the women with PCOS were significantly higher than those of the control group (BDI, 11.69 +/- 9.49 vs 5.80 +/- 4.58; GHQ-12, 3.38 +/- 3.38 vs 1.54 +/- 1.97, respectively), and BMI and WHR were positively correlated with the BDI and GHQ-12 scores. Clinicians should be aware of the increased risk of emotional distress and depression in women with PCOS, especially those who are obese, and of the need to screen these patients for such symptoms.

  13. 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.

  14. 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

  15. 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

  16. “Obesity Paradox” in Acute Respiratory Distress Syndrome: Asystematic Review and Meta-Analysis

    PubMed Central

    Zhi, Guo; Xin, Wang; Ying, Wang; Guohong, Xing; Shuying, Liu

    2016-01-01

    Background It is unclear whether an “obesity paradox” exists in the respiratory system, especially in acute respiratory distress syndrome (ARDS) and acute lung injury (ALI). Previous studies have postulated a causal relation between obesity and ARDS/ALI but have lacked power to form a definitive conclusion. Objective To investigate the relationships between obesity, ARDS/ALIrisk, and mortality. Methods A systematic search current to April 2016 was performed in Pubmed, EMBASE, Medline, Cochrane databases to find relevant studies. All studies that estimate the effect of obesity in the morbidity and mortality of ARDS/ALI were included. Results A total of 24 studies including 9,187,248 subjects were analyzed. The combined results from 16 studies that examined the effect of obesity in morbidity of ARDS/ALI showed an89% increase in odds ratio(pooled odds ratios (OR) 1.89, 95% confidence intervals (CI) 1.45 to 2.47). In subgroup analysis, compared to normal weight, obesity was associated with an increased risk of ARDS/ALI (OR1.57, 95% CI 1.30–1.90 for obese (BMI30-39.9kg/m2); OR1.75, 95% CI 1.42–2.15 for obese(BMI≥30kg/m2); OR1.67, 95% CI 1.04–2.68 for morbid obese(BMI≥40kg/m2)). The combined results from 9 studies that examined the effect of obesity in mortality of ARDS/ALI had a pooled odds ratio(pooled OR 0.63, 95% CI 0.41 to 0.98). Inversely, obesity was significantly associated with reduced risk of ARDS/ALI mortality(OR0.88, 95% CI 0.78–1.00 for overweight(BMI≤18.5m2); OR0.74, 95% CI 0.64–0.84 for obese (BMI30-39.9kg/m2);OR0.84, 95% CI 0.75–0.94 for 60days mortality; OR0.38, 95% CI 0.22–0.66 for 90days mortality). Conclusions Our data identify obesity as an important risk factor for the development of ARDS/ALI; however, ARDS/ALI outcomes are improved in this population when compared to individuals with a normal body mass index. This meta-analysis results supported ‘‘obesity paradox” in ARDS/ALI. PMID:27684705

  17. Low-dose CT for quantitative analysis in acute respiratory distress syndrome

    PubMed Central

    2013-01-01

    Introduction The clinical use of serial quantitative computed tomography (CT) to characterize lung disease and guide the optimization of mechanical ventilation in patients with acute respiratory distress syndrome (ARDS) is limited by the risk of cumulative radiation exposure and by the difficulties and risks related to transferring patients to the CT room. We evaluated the effects of tube current-time product (mAs) variations on quantitative results in healthy lungs and in experimental ARDS in order to support the use of low-dose CT for quantitative analysis. Methods In 14 sheep chest CT was performed at baseline and after the induction of ARDS via intravenous oleic acid injection. For each CT session, two consecutive scans were obtained applying two different mAs: 60 mAs was paired with 140, 15 or 7.5 mAs. All other CT parameters were kept unaltered (tube voltage 120 kVp, collimation 32 × 0.5 mm, pitch 0.85, matrix 512 × 512, pixel size 0.625 × 0.625 mm). Quantitative results obtained at different mAs were compared via Bland-Altman analysis. Results Good agreement was observed between 60 mAs and 140 mAs and between 60 mAs and 15 mAs (all biases less than 1%). A further reduction of mAs to 7.5 mAs caused an increase in the bias of poorly aerated and nonaerated tissue (-2.9% and 2.4%, respectively) and determined a significant widening of the limits of agreement for the same compartments (-10.5% to 4.8% for poorly aerated tissue and -5.9% to 10.8% for nonaerated tissue). Estimated mean effective dose at 140, 60, 15 and 7.5 mAs corresponded to 17.8, 7.4, 2.0 and 0.9 mSv, respectively. Image noise of scans performed at 140, 60, 15 and 7.5 mAs corresponded to 10, 16, 38 and 74 Hounsfield units, respectively. Conclusions A reduction of effective dose up to 70% has been achieved with minimal effects on lung quantitative results. Low-dose computed tomography provides accurate quantitative results and could be used to characterize lung compartment distribution and

  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. Evaluation of a practice guideline for the management of respiratory distress syndrome in preterm infants: A quality improvement initiative

    PubMed Central

    Read, Brooke; Lee, David SC; Fraser, Debbie

    2016-01-01

    BACKGROUND: The use of mechanical ventilation to treat respiratory distress syndrome in preterm infants has been associated with the development of bronchopulmonary dysplasia. As part of a quality improvement initiative to reduce the incidence of bronchopulmonary dysplasia in preterm infants, a new practice guideline for the management of respiratory distress syndrome was developed and adopted into practice in a neonatal intensive care unit in February 2012. OBJECTIVE: To evaluate the effects of implementing the new guideline in regard to the use of mechanical ventilation and surfactant, and the incidence of bronchopulmonary dypslasia. METHODS: An historical cohort of very preterm infants (gestational age 260 to 326 weeks) born one year before guideline implementation was compared with a similar cohort of infants born one year following guideline implementation. Data were collected retrospectively from the local neonatal intensive care unit database. RESULTS: A total of 272 preterm infants were included in the study: 129 in the preguideline cohort and 143 in the postguideline cohort. Following the implementation of the guideline, the proportion of infants treated with ongoing mechanical ventilation was reduced from 49% to 26% (P<0.001) and there was a trend toward a reduction in bronchopulmonary dysplasia (27% versus 18%; P=0.07). There was no difference in the proportion of infants treated with surfactant (54% versus 50%). CONCLUSION: The implementation of the practice guideline helped to minimize the use of ongoing mechanical ventilation in preterm infants. PMID:26941562

  20. 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. PMID:572660

  1. Chronic shoulder pain in the community: a syndrome of disability or distress?

    PubMed Central

    Badcock, L; Lewis, M; Hay, E; McCarney, R; Croft, P

    2002-01-01

    Objectives: To investigate two questions in a community based population of people with chronic shoulder pain. Firstly, does chronic pain lead to impaired psychological health over time? Secondly, how does restriction of daily activity influence pain perception and psychological health? Methods: Two postal surveys, two years apart, were carried out to identify a group of subjects with chronic shoulder pain. The first survey was sent to a random sample of adults (n=40026) registered with a primary care practice, and included a pain manikin, demographic information, and the Hospital Anxiety and Depression scale (HAD). The second survey was sent to those subjects who reported unilateral shoulder region pain in the first survey and it included a shoulder-specific disability scale, pain severity score, and the HAD. Results: 2606 (65.1%) people responded to the initial survey. Of these, 304 (11.7%) reported unilateral shoulder region pain at baseline. In the subsequent survey, there were 234 responders (83.3% adjusted response): 142 of these reported shoulder pain and formed our study group of "subjects with chronic shoulder pain". Within this group there was no significant change in psychological distress scores between baseline and follow up. Both the disability score and psychological distress scores correlated significantly with pain severity (disability v pain r=0.536, p<0.001; psychological distress v pain r=0.269, p=0.002). When the correlation between disability and pain severity was corrected for possible confounders, it remained significant (r=0.490, p<0.001). This was not the case for the correlation between psychological distress and pain (p>0.05). Disability was significantly correlated with psychological distress on univariate (r=0.445, p<0.001) and multivariate analysis (r=0.341, p=0.002). Conclusion: In those with chronic shoulder pain the relation between pain and psychological health seems to be linked to disability. Psychological distress was not

  2. Clinical review: Exogenous surfactant therapy for acute lung injury/acute respiratory distress syndrome--where do we go from here?

    PubMed

    Dushianthan, Ahilanandan; Cusack, Rebecca; Goss, Victoria; Postle, Anthony D; Grocott, Mike P W

    2012-01-01

    Acute lung injury and acute respiratory distress syndrome (ARDS) are characterised by severe hypoxemic respiratory failure and poor lung compliance. Despite advances in clinical management, morbidity and mortality remains high. Supportive measures including protective lung ventilation confer a survival advantage in patients with ARDS, but management is otherwise limited by the lack of effective pharmacological therapies. Surfactant dysfunction with quantitative and qualitative abnormalities of both phospholipids and proteins are characteristic of patients with ARDS. Exogenous surfactant replacement in animal models of ARDS and neonatal respiratory distress syndrome shows consistent improvements in gas exchange and survival. However, whilst some adult studies have shown improved oxygenation, no survival benefit has been demonstrated to date. This lack of clinical efficacy may be related to disease heterogeneity (where treatment responders may be obscured by nonresponders), limited understanding of surfactant biology in patients or an absence of therapeutic effect in this population. Crucially, the mechanism of lung injury in neonates is different from that in ARDS: surfactant inhibition by plasma constituents is a typical feature of ARDS, whereas the primary pathology in neonates is the deficiency of surfactant material due to reduced synthesis. Absence of phenotypic characterisation of patients, the lack of an ideal natural surfactant material with adequate surfactant proteins, coupled with uncertainty about optimal timing, dosing and delivery method are some of the limitations of published surfactant replacement clinical trials. Recent advances in stable isotope labelling of surfactant phospholipids coupled with analytical methods using electrospray ionisation mass spectrometry enable highly specific molecular assessment of phospholipid subclasses and synthetic rates that can be utilised for phenotypic characterisation and individualisation of exogenous surfactant

  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. 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

  6. Surfactant therapy in preterm infants with respiratory distress syndrome and in near-term or term newborns with acute RDS.

    PubMed

    Ramanathan, R

    2006-05-01

    Many different surfactant preparations derived from animal sources, as well as synthetic surfactants, are available for the treatment of preterm infants with respiratory distress syndrome (RDS). Natural, modified surfactants containing surfactant-associated proteins appear to be more effective than non-protein-containing synthetic surfactants. Comparative trials with poractant alfa at a higher initial dose of 200 mg/kg appear to be associated with rapid weaning of FiO2, less need for additional doses, and decreased mortality in infants <32 weeks gestation when compared with beractant. Early rescue (<30 min of age) surfactant therapy is an effective method to minimize over treatment of some preterm infants who may not develop RDS. Surfactant therapy followed by rapid extubation to nasal ventilation appears to be more beneficial than continued mechanical ventilation. In near-term or term newborns with acute RDS, surfactant therapy has been shown to be 70% effective in improving respiratory failure.

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

    PubMed

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

    2016-09-01

    We present a female patient with severe acute respiratory distress syndrome (ARDS) necessitating intubation and mechanical ventilation on the intensive care unit (ICU). High ventilatory pressures were needed because of hypoxia and severe hypercapnia with respiratory acidosis, resulting in right ventricular dysfunction with impaired haemodynamic stability. A veno-venous extracorporeal CO2 removal (ECCO2R) circuit was initiated, effectively eliminating carbon dioxide while improving oxygenation and enabling a reduction in applied ventilatory pressures. We noted a marked improvement of right ventricular function with restoration of haemodynamic stability. Within one week, the patient was weaned from both ECCO2R and mechanical ventilation. Besides providing adequate gas exchange, extracorporeal assist devices may be helpful in ameliorating right ventricular dysfunction during ARDS.

  8. Lavage-induced Surfactant Depletion in Pigs As a Model of the Acute Respiratory Distress Syndrome (ARDS).

    PubMed

    Russ, Martin; Kronfeldt, Sebastian; Boemke, Willehad; Busch, Thilo; Francis, Roland C E; Pickerodt, Philipp A

    2016-01-01

    Various animal models of lung injury exist to study the complex pathomechanisms of human acute respiratory distress syndrome (ARDS) and evaluate future therapies. Severe lung injury with a reproducible deterioration of pulmonary gas exchange and hemodynamics can be induced in anesthetized pigs using repeated lung lavages with warmed 0.9% saline (50 ml/kg body weight). Including standard respiratory and hemodynamic monitoring with clinically applied devices in this model allows the evaluation of novel therapeutic strategies (drugs, modern ventilators, extracorporeal membrane oxygenators, ECMO), and bridges the gap between bench and bedside. Furthermore, induction of lung injury with lung lavages does not require the injection of pathogens/endotoxins that impact on measurements of pro- and anti-inflammatory cytokines. A disadvantage of the model is the high recruitability of atelectatic lung tissue. Standardization of the model helps to avoid pitfalls, to ensure comparability between experiments, and to reduce the number of animals needed. PMID:27684585

  9. Atypical Progeroid Syndrome due to Heterozygous Missense LMNA Mutations

    PubMed Central

    Garg, Abhimanyu; Subramanyam, Lalitha; Agarwal, Anil K.; Simha, Vinaya; Levine, Benjamin; D'Apice, Maria Rosaria; Novelli, Giuseppe; Crow, Yanick

    2009-01-01

    Context: Hutchinson-Gilford progeria syndrome (HGPS) and mandibuloacral dysplasia are well-recognized allelic autosomal dominant and recessive progeroid disorders, respectively, due to mutations in lamin A/C (LMNA) gene. Heterozygous LMNA mutations have also been reported in a small number of patients with a less well-characterized atypical progeroid syndrome (APS). Objective: The objective of the study was to investigate the underlying genetic and molecular basis of the phenotype of patients presenting with APS. Results: We report 11 patients with APS from nine families, many with novel heterozygous missense LMNA mutations, such as, P4R, E111K, D136H, E159K, and C588R. These and previously reported patients now reveal a spectrum of clinical features including progeroid manifestations such as short stature, beaked nose, premature graying, partial alopecia, high-pitched voice, skin atrophy over the hands and feet, partial and generalized lipodystrophy with metabolic complications, and skeletal anomalies such as mandibular hypoplasia and mild acroosteolysis. Skin fibroblasts from these patients when assessed for lamin A/C expression using epifluorescence microscopy revealed variable nuclear morphological abnormalities similar to those observed in patients with HGPS. However, these nuclear abnormalities in APS patients could not be rescued with 48 h treatment with farnesyl transferase inhibitors, geranylgeranyl transferase inhibitors or trichostatin-A, a histone deacetylase inhibitor. Immunoblots of cell lysates from fibroblasts did not reveal prelamin A accumulation in any of these patients. Conclusions: APS patients have a few overlapping but some distinct clinical features as compared with HGPS and mandibuloacral dysplasia. The pathogenesis of clinical manifestations in APS patients seems not to be related to accumulation of mutant farnesylated prelamin A. PMID:19875478

  10. Anton's Syndrome due to Bilateral Ischemic Occipital Lobe Strokes.

    PubMed

    Zukić, Sanela; Sinanović, Osman; Zonić, Lejla; Hodžić, Renata; Mujagić, Svjetlana; Smajlović, Edina

    2014-01-01

    We present a case of a patient with Anton's syndrome (i.e., visual anosognosia with confabulations), who developed bilateral occipital lobe infarct. Bilateral occipital brain damage results in blindness, and patients start to confabulate to fill in the missing sensory input. In addition, the patient occasionally becomes agitated and talks to himself, which indicates that, besides Anton's syndrome, he might have had Charles Bonnet syndrome, characterized by both visual loss and hallucinations. Anton syndrome, is not so frequent condition and is most commonly caused by ischemic stroke. In this particular case, the patient had successive bilateral occipital ischemia as a result of massive stenoses of head and neck arteries.

  11. 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

  12. 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.

  13. 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. PMID:25821094

  14. Turner syndrome with spinal hemorrhage due to vascular malformation.

    PubMed

    Yu, Min Kyung; Jung, Mo Kyung; Kim, Ki Eun; Kwon, Ah Reum; Chae, Hyun Wook; Kim, Duk Hee; Kim, Ho-Seong

    2015-12-01

    Turner syndrome (TS) is a relatively common chromosomal disorder and is associated with a range of comorbidities involving the cardiovascular system. Vascular abnormalities, in particular, are a common finding in cases of TS. However, dissection involving the vertebral arteries is rare. Here, we report the case of a 9-year-old girl with TS who had been treated with growth hormone replacement therapy for the past 3 years. She presented with weakness of both lower legs, and was ultimately diagnosed with spinal hemorrhage due to vascular malformation. We treated her with intravenous high dose dexamethasone (0.6 mg/kg) and she could walk without assistance after 6 days of treatment. In conclusion, when a patient with TS shows sudden weakness of the lower limbs, we should consider the possibility of spinal vessel rupture and try to take spine magnetic resonance imaging as soon as possible. We suggest a direction how to make a proper diagnosis and management of sudden vertebral artery hemorrhage in patients with TS.

  15. Turner syndrome with spinal hemorrhage due to vascular malformation

    PubMed Central

    Yu, Min Kyung; Jung, Mo Kyung; Kim, Ki Eun; Kwon, Ah Reum; Kim, Duk Hee; Kim, Ho-Seong

    2015-01-01

    Turner syndrome (TS) is a relatively common chromosomal disorder and is associated with a range of comorbidities involving the cardiovascular system. Vascular abnormalities, in particular, are a common finding in cases of TS. However, dissection involving the vertebral arteries is rare. Here, we report the case of a 9-year-old girl with TS who had been treated with growth hormone replacement therapy for the past 3 years. She presented with weakness of both lower legs, and was ultimately diagnosed with spinal hemorrhage due to vascular malformation. We treated her with intravenous high dose dexamethasone (0.6 mg/kg) and she could walk without assistance after 6 days of treatment. In conclusion, when a patient with TS shows sudden weakness of the lower limbs, we should consider the possibility of spinal vessel rupture and try to take spine magnetic resonance imaging as soon as possible. We suggest a direction how to make a proper diagnosis and management of sudden vertebral artery hemorrhage in patients with TS. PMID:26817012

  16. Controversies in the pathophysiology and fluid management of postoperative adult respiratory distress syndrome.

    PubMed

    Shoemaker, W C

    1985-08-01

    Physiologic changes that lead to the development of ARDS begin with the precipitating shock syndrome. Hypovolemia, pulmonary vasoconstriction, reduced myocardial performance, and diminished O2 transport typically precede the development of clinical ARDS after hemorrhage, trauma, postoperative conditions, and sepsis. Since shock lung is a complication of shock, it is not surprising that the antecedent clinical and physiologic events that characterize the shock state may be determinants of both the genesis and the outcome of ARDS. Postoperative ARDS follows unrecognized or inadequately treated hypovolemia and hypoxia during an antecedent period of preoperative or intraoperative shock. Hypovolemia and hypoxia increase cardiac and ventilatory drive and stimulate neurohumoral mechanisms to increase pulmonary vasoconstriction. The last-named, when extensive and uneven, produces maldistribution of flow and reduces DO2 and VO2. Subsequently, mediator-induced pulmonary vasoconstriction increases the problem. When sufficiently extensive, these antecedent physiologic alterations culminate in ARDS. With impaired flow and O2 transport, pathogenic mechanisms of ARDS and acute renal failure may be set in motion; further, the naturally occurring immune mechanisms may be impaired and may lead to associated infection. There are at least six redistributions that are major pathophysiologic influences in ARDS. They are uneven ventilation throughout the lung; redistribution of regional pulmonary blood flow between zones due to gravity; nonuniform pulmonary blood flow between individual metarteriolar-capillary networks because of local vasoconstriction; uneven systemic blood flow between organs; irregular systemic blood flow at the microcirculatory level, producing inadequate nutritional flow to the tissues; and redistribution of body water, leading particularly to fluid accumulation in the extracellular compartment, with expanded interstitial space and contracted plasma volume

  17. 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

  18. Lemierre's syndrome due to intratumoral abscess of the uvula.

    PubMed

    Rahhal, Hassan; 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

  19. 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.

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

    PubMed

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

    2015-01-01

    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. PMID:25833911

  1. 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

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

    PubMed

    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

  3. Comparison of INSURE method with conventional mechanical ventilation after surfactant administration in preterm infants with respiratory distress syndrome: therapeutic challenge.

    PubMed

    Nayeri, Fatemeh Sadat; Esmaeilnia Shirvani, Tahereh; Aminnezhad, Majid; Amini, Elaheh; Dalili, Hossein; Moghimpour Bijani, Faezeh

    2014-01-01

    Administration of endotracheal surfactant is potentially the main treatment for neonates suffering from RDS (Respiratory Distress Syndrome), which is followed by mechanical ventilation. Late and severe complications may develop as a consequence of using mechanical ventilation. In this study, conventional methods for treatment of RDS are compared with surfactant administration, use of mechanical ventilation for a brief period and NCPAP (Nasal Continuous Positive Airway Pressure), (INSURE method ((Intubation, Surfactant administration and extubation)). A randomized clinical trial study was performed, including all newborn infants with diagnosed RDS and a gestational age of 35 weeks or less, who were admitted in NICU of Valiasr hospital. The patients were then divided randomly into two CMV (Conventional Mechanical Ventilation) and INSURE groups. Surfactant administration and consequent long-term mechanical ventilation were done in the first group (CMV group). In the second group (INSURE group), surfactant was administered followed by a short-term period of mechanical ventilation. The infants were then extubated, and NCPAP was embedded. The comparison included crucial duration of mechanical ventilation and oxygen therapy, IVH (Intraventricular Hemorrhage), PDA (Patent Ductus Arteriosus), air-leak syndromes, BPD (Broncho-Pulmonary Dysplasia) and mortality rate. The need for mechanical ventilation in 5th day of admission was 43% decreased (P=0.005) in INSURE group in comparison to CMV group. A decline (P=0.01) in the incidence of IVH and PDA was also achieved. Pneumothorax, chronic pulmonary disease and mortality rates, were not significantly different among two groups. (P=0.25, P=0.14, P=0.25, respectively). This study indicated that INSURE method in the treatment of RDS decreases the need for mechanical ventilation and oxygen-therapy in preterm neonates. Moreover, relevant complications as IVH and PDA were observed to be reduced. Thus, it seems rationale to perform

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

    PubMed Central

    Macario, Alex; Chow, John L; Dexter, Franklin

    2006-01-01

    Background Management of acute respiratory distress syndrome (ARDS) in the intensive care unit (ICU) is clinically challenging and costly. Neuromuscular blocking agents may facilitate mechanical ventilation and improve oxygenation, but may result in prolonged recovery of neuromuscular function and acute quadriplegic myopathy syndrome (AQMS). The goal of this study was to address a hypothetical question via computer modeling: Would a reduction in intubation time of 6 hours and/or a reduction in the incidence of AQMS from 25% to 21%, provide enough benefit to justify a drug with an additional expenditure of $267 (the difference in acquisition cost between a generic and brand name neuromuscular blocker)? Methods The base case was a 55 year-old man in the ICU with ARDS who receives neuromuscular blockade for 3.5 days. A Markov model was designed with hypothetical patients in 1 of 6 mutually exclusive health states: ICU-intubated, ICU-extubated, hospital ward, long-term care, home, or death, over a period of 6 months. The net monetary benefit was computed. Results Our computer simulation modeling predicted the mean cost for ARDS patients receiving standard care for 6 months to be $62,238 (5% – 95% percentiles $42,259 – $83,766), with an overall 6-month mortality of 39%. Assuming a ceiling ratio of $35,000, even if a drug (that cost $267 more) hypothetically reduced AQMS from 25% to 21% and decreased intubation time by 6 hours, the net monetary benefit would only equal $137. Conclusion ARDS patients receiving a neuromuscular blocker have a high mortality, and unpredictable outcome, which results in large variability in costs per case. If a patient dies, there is no benefit to any drug that reduces ventilation time or AQMS incidence. A prospective, randomized pharmacoeconomic study of neuromuscular blockers in the ICU to asses AQMS or intubation times is impractical because of the highly variable clinical course of patients with ARDS. PMID:16539706

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

    PubMed

    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

  6. Genetic susceptibility to nosocomial pneumonia, acute respiratory distress syndrome and poor outcome in patients at risk of critical illness.

    PubMed

    Salnikova, Lyubov E; Smelaya, Tamara V; Vesnina, Irina N; Golubev, Arkadiy M; Moroz, Viktor V

    2014-04-01

    Genetic susceptibility may partially explain the clinical variability observed during the course of similar infections. To establish the contribution of genetic host factors in the susceptibility to critical illness, we genotyped 750 subjects (419 at high risk of critical illness) for 14 single nucleotide polymorphisms (SNPs) in the xenobiotics detoxification/oxidative stress and vascular homeostasis metabolic pathways. In the group of nosocomial pneumonia (NP; 268 patients) the risk of acute respiratory distress syndrome (ARDS) is significantly higher for the carriers of CYP1A1 rs2606345 T/T genotypes and AhR rs2066853 G/A-A/A genotypes. AGTR1 rs5186 allele C is more common among NP non-survivors. The duration of stay in intensive care units (ICU) is higher for NP patients with ABCB1 rs1045642-T allele. The cumulative effect of the risk alleles in the genes comprising two sets of genes partners (xenobiotics detoxification: CYP1A1, AhR and RAS family: ACE, AGT, AGTR1) is associated with the development of both NP and ARDS.

  7. 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.

  8. Effect of Antiplatelet Therapy on Acute Respiratory Distress Syndrome and Mortality in Critically Ill Patients: A Meta-Analysis

    PubMed Central

    Wang, Lijun; Li, Heng; Gu, Xiaofei; Wang, Zhen; Liu, Su; Chen, Liyong

    2016-01-01

    Background Antiplatelet agents are commonly used for cardiovascular diseases, but their pleiotropic effects in critically ill patients are controversial. We therefore performed a meta-analysis of cohort studies to investigate the effect of antiplatelet therapy in the critically ill. Methods Nine cohort studies, retrieved from PubMed and Embase before November 2015, involving 14,612 critically ill patients and 4765 cases of antiplatelet users, were meta-analysed. The main outcome was hospital or 30-day mortality. Secondary outcome was acute respiratory distress syndrome (ARDS) or acute lung injury (ALI). Random- or fixed-effect models were taken for quantitative synthesis of the data. Results Antiplatelet therapy was associated with decreased mortality (odds ratio (OR) 0.61; 95% confidence interval (CI), 0.52–0.71; I2 = 0%; P <0. 001) and ARDS/ALI (OR 0.64; 95% CI, 0.50–0.82; I2 = 0%; P <0. 001). In every stratum of subgroups, similar findings on mortality reduction were consistently observed in critically ill patients. Conclusions Antiplatelet therapy is associated with reduced mortality and lower incidence of ARDS/ALI in critically ill patients, particularly those with predisposing conditions such as high-risk surgery, trauma, pneumonia, and sepsis. However, it remains unclear whether similar findings can be observed in the unselected and broad population with critical illness. PMID:27182704

  9. 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

  10. Customization of an open-lung ventilation strategy to treat a case of life-threatening acute respiratory distress syndrome.

    PubMed

    Grooms, David A; Sibole, Stephen H; Tomlinson, James R; Marik, Paul E; Chatburn, Robert L

    2011-04-01

    The ARDS Network low-tidal-volume protocol is considered the standard of care for patients with acute lung injury (ALI) or acute respiratory distress syndrome (ARDS). The protocol is built on the foundation of low-tidal-volume ventilation, use of a combined PEEP and F(IO(2)) table, and managing alveolar end-inspiratory pressure by limiting the plateau airway pressure to ≤ 30 cm H(2)O. Although this strategy, to date, is the only method that significantly improves ALI/ARDS survival, alternative methods of improving hypoxemia and minimizing ventilator-induced lung injury, in conjunction with low-tidal-volume ventilation, can be used for life-threatening ARDS. We present a case in which we customized the use of alveolar recruitment maneuvers by analyzing the hysteresis of the pressure-volume curve to assess lung recruitability, decremental PEEP to sustain lung recruitment, and careful use of plateau pressure ≥ 30 cm H(2)O, which improved our patient's life-threatening hypoxemia within the first 36 min of arrival to our ICU.

  11. 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. PMID:27398166

  12. [Vegetable oil-induced acute respiratory distress syndrome (ARDS) in near drowning: evaluation based on extravascular lung water index].

    PubMed

    Yoshida, Takeshi; Rinka, Hiroshi; Kaji, Arito

    2008-06-01

    Lipoid pneumonia usually presents after chronic recurrent ingestion of oily substances or accidental aspiration during "fire-eating" demonstrations. Massive exposure by near drowning extremely rare and potentially fatal. We present here a case of survival after total immersion in oil in her workplace. A 66-year-old woman who nearly drowned in a vat of vegetable oil was admitted as an emergency case with severe hypoxia after rescue. Chest computed tomography (CT) findings showed bilateral ground-glass opacity, consolidation, and the case fulfilled the criteria for acute respiratory distress syndrome (ARDS). Bronchoscopy and bronchoalveolar lavage performed on admission indicated oil droplets and marked neutrophilia (67%), which made us diagnose ARDS induced by lipoid pneumonia. We commenced treatment with pulsed steroids and strictly managed fluid balance under mechanical ventilation. Despite immediate improvement in oxygenation, the value of extravascular lung water index (EVLWI) measured by the PiCCO system consistently remained over 30 ml/Kg through her clinical course. We concluded that lipoid pneumonia is characterized by prolonged elevatation of pulmonary vascular permeability.

  13. Mouse Models of Acute Respiratory Distress Syndrome: A Review of Analytical Approaches, Pathologic Features, and Common Measurements.

    PubMed

    Aeffner, Famke; Bolon, Brad; Davis, Ian C

    2015-12-01

    Acute respiratory distress syndrome (ARDS) is a severe pulmonary reaction requiring hospitalization, which is incited by many causes, including bacterial and viral pneumonia as well as near drowning, aspiration of gastric contents, pancreatitis, intravenous drug use, and abdominal trauma. In humans, ARDS is very well defined by a list of clinical parameters. However, until recently no consensus was available regarding the criteria of ARDS that should be evident in an experimental animal model. This lack was rectified by a 2011 workshop report by the American Thoracic Society, which defined the main features proposed to delineate the presence of ARDS in laboratory animals. These should include histological changes in parenchymal tissue, altered integrity of the alveolar capillary barrier, inflammation, and abnormal pulmonary function. Murine ARDS models typically are defined by such features as pulmonary edema and leukocyte infiltration in cytological preparations of bronchoalveolar lavage fluid and/or lung sections. Common pathophysiological indicators of ARDS in mice include impaired pulmonary gas exchange and histological evidence of inflammatory infiltrates into the lung. Thus, morphological endpoints remain a vital component of data sets assembled from animal ARDS models.

  14. Equipoise in Research and the Development of Neonatal Interventions for the Management of Respiratory Distress Syndrome: A Historical Perspective.

    PubMed

    Rojas, Mario Augusto

    2015-08-01

    The historical review of how evidence was developed for the management of respiratory distress syndrome in premature infants has not been clearly characterized. Knowledge of this process is essential to understand the role of equipoise and its influence on the decision to evaluate interventions as they were implemented in the practice of medicine. We suspect that errant approaches to clinical equipoise secondary to states of false certainty and false uncertainty have been important barriers to the timely acquisition and implementation of evidence-based knowledge necessary to improve outcomes in this fragile population of infants. When confronted with the decision to test an intervention, physicians should question whether they have lost clinical equipoise based on opinion, expertise, or observational data rather than evidence obtained from methodological inquiry; doing so facilitates reaching clinical equipoise and promotes the application of scientific methodology to answer relevant clinical questions. Timely acquisition of evidence-based knowledge can be viewed as an ethical imperative when the status quo may have negative consequences on outcomes for generations.

  15. Mesobuthus tamulus venom induces acute respiratory distress syndrome in rats involving additional mechanisms as compared to oleic acid model.

    PubMed

    Akella, Aparna; Tiwari, Anil K; Patne, Shashikant C U; Deshpande, Shripad B

    2015-04-01

    The present study was undertaken to determine whether acute respiratory distress syndrome (ARDS) is produced after Mesobuthus tamulus (MBT) envenomation and compared it with oleic acid (OA)-induced ARDS. The trachea, jugular vein and femoral artery were cannulated in anesthetized adult rats. Lethal dose of MBT venom (5 mg/kg) or OA (75 μL) was administered intravenously and the time-dependent changes in respiratory frequency (RF), heart rate (HR) and mean arterial pressure (MAP) were recorded. Minute ventilation (MV) and the PaO2/FiO2 (P/F) ratio were also determined. At the end lungs were excised, one lung was used for histopathological examination and the other was used for determination of pulmonary water content physically. MBT venom or OA produced hypoxemia, pulmonary pathology (alveolar damage, infiltration of inflammatory cells, capillary damage and exudation) and pulmonary edema implicating for ARDS. However, the hypoxemia in MBT venom group was associated with decreased MV, apnea/bradypnea, and bradycardia whereas, in OA group it was seen with increased MV, tachypnea, and tachycardia. Lack of effect of hypoxemic drive on RF/MV or HR in MBT venom group unlike OA group, suggests the involvement of medullary centers. The present results demonstrate that MBT venom produces ARDS. However MBT venom-induced ARDS involves pulmonary as well as extrapulmonary mechanisms.

  16. Toward Smarter Lumping and Smarter Splitting: Rethinking Strategies for Sepsis and Acute Respiratory Distress Syndrome Clinical Trial Design.

    PubMed

    Prescott, Hallie C; Calfee, Carolyn S; Thompson, B Taylor; Angus, Derek C; Liu, Vincent X

    2016-07-15

    Both quality improvement and clinical research efforts over the past few decades have focused on consensus definition of sepsis and acute respiratory distress syndrome (ARDS). Although clinical definitions based on readily available clinical data have advanced recognition and timely use of broad supportive treatments, they likely hinder the identification of more targeted therapies that manipulate select biological mechanisms underlying critical illness. Sepsis and ARDS are by definition heterogeneous, and patients vary in both their underlying biology and their severity of illness. We have long been able to identify subtypes of sepsis and ARDS that confer different prognoses. The key is that we are now on the verge of identifying subtypes that may confer different response to therapy. In this perspective, inspired by a 2015 American Thoracic Society International Conference Symposium entitled "Lumpers and Splitters: Phenotyping in Critical Illness," we highlight promising approaches to uncovering patient subtypes that may predict treatment responsiveness and not just differences in prognosis. We then discuss how this information can be leveraged to improve the success and translatability of clinical trials by using predictive enrichment and other design strategies. Last, we discuss the challenges and limitations to identifying biomarkers and endotypes and incorporating them into routine clinical practice. PMID:27244481

  17. 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

  18. 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.

  19. Immunohistochemical expression of MPO, CD163 and VEGF in inflammatory cells in acute respiratory distress syndrome: a case report

    PubMed Central

    Maretta, Milan; Toth, Stefan; Jonecova, Zuzana; Kruzliak, Peter; Kubatka, Peter; Pingorova, Stanislava; Vesela, Jarmila

    2014-01-01

    Acute respiratory distress syndrome (ARDS) is a serious medical condition occurring in patients with polytrauma, pulmonary or non-pulmonary sepsis, pneumonia and many other circumstances. It causes inflammation of the lung parenchyma leading to impaired gas exchange with a systemic release of inflammatory mediators, causing consequential lung tissue injury, hypoxemia and frequently multiple organ failure. The aim of current study was to describe expression of inflammatory markers (myeloperoxidase, CD163 and vascular endothelial growth factor) by the cells in acute phase of ARDS. The lung samples of a 20-year-old man who had suffered a serious motorbike accident were obtained for histological examination. He died on the seventh day as a consequence of respiratory failure. Our results imply that expression of CD163 was restricted to activated alveolar macrophages and monocytes. Immunopositivityof MPO was observed in neutrophil granulocytes within lung alveoli and lung blood vessels. Myeloperoxidase positivity was observed in alveolar macrophages, too. Vascular endothelial growth factor was expressed in cytoplasm of neutrophil granulocytes, monocytes, small-sized alveolar macrophages and type II pneumocytes localized mostly inside lung alveoli. On the contrary, no positivity was observed in lung endothelial cells of blood vessels. PMID:25120850

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

    PubMed

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

    2016-01-01

    The purpose of this study was to compare the Berlin definition to the American-European Consensus Conference (AECC) definition in determining the prevalence of acute respiratory distress syndrome (ARDS) and associated mortality in the critically ill burn population. Consecutive patients admitted to our institution with burn injury that required mechanical ventilation for more than 24 hours were included for analysis. Included patients (N = 891) were classified by both definitions. The median age, % TBSA burn, and injury severity score (interquartile ranges) were 35 (24-51), 25 (11-45), and 18 (9-26), respectively. Inhalation injury was present in 35.5%. The prevalence of ARDS was 34% using the Berlin definition and 30.5% using the AECC definition (combined acute lung injury and ARDS), with associated mortality rates of 40.9 and 42.9%, respectively. Under the Berlin definition, mortality rose with increased ARDS severity (14.6% no ARDS; 16.7% mild; 44% moderate; and 59.7% severe, P < 0.001). By contrast, under the AECC definition increased mortality was seen only for ARDS category (14.7% no ARDS; 15.1% acute lung injury; and 46.0% ARDS, P < 0.001). The mortality of the 22 subjects meeting the AECC, but not the Berlin definition was not different from patients without ARDS (P = .91). The Berlin definition better stratifies ARDS in terms of severity and correctly excludes those with minimal disease previously captured by the AECC.

  1. Brown-Sequard syndrome due to isolated blunt trauma.

    PubMed

    Henderson, S O; Hoffner, R J

    1998-01-01

    Blunt trauma without associated fracture or ligamentous injury is a rare cause of Brown-Sequard syndrome. We report a case of Brown-Sequard syndrome after a direct blow to the cervical spine that did not cause injury to adjacent bone or ligaments. Characteristic neurologic findings, including a unilateral hemiparesis with associated contralateral sensory findings, were noted at the time of presentation. High-dose steroids were instituted after recognition of the patient's injury, and magnetic resonance imaging of the cervical spine revealed a unilateral cord contusion with no associated fractures. After 1 month, the patient had recovered much of his function and was able to ambulate unassisted.

  2. 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

  3. Sporadic Corticobasal Syndrome due to FTLD-TDP

    PubMed Central

    Tartaglia, Maria Carmela; Sidhu, Manu; Laluz, Victor; Racine, Caroline; Rabinovici, Gil D; Creighton, Kelly; Karydas, Anna; Rademakers, Rosa; Huang, Eric J; Miller, Bruce L; DeArmond, Stephen J; Seeley, William W

    2010-01-01

    Sporadic corticobasal syndrome (CBS) has been associated with diverse pathological substrates, but frontotemporal lobar degeneration with TDP-43 immunoreactive inclusions (FTLD-TDP) has only been linked to CBS among progranulin mutation carriers. We report the clinical, neuropsychological, imaging, genetic, and neuropathological features of GS, a patient with sporadic corticobasal syndrome. Genetic testing revealed no mutations in the microtubule associated protein tau (MAPT) or progranulin (PGRN) genes, but GS proved homozygous for the T allele of the rs5848 PGRN variant. Autopsy showed ubiquitin and TDP-43 pathology most similar to a pattern previously associated with PGRN mutation carriers. These findings confirm that FTLD-TDP should be included in the pathological differential diagnosis for sporadic CBS. PMID:19876635

  4. [Bilateral diaphragmatic paralysis due to Parsonage-Turner syndrome].

    PubMed

    Tissier-Ducamp, D; Martinez, S; Alagha, K; Charpin, D; Chanez, P; Palot, A

    2015-09-01

    We report the case of a 49-years-old patient who presented to the accident and emergency department with sudden onset dyspnea associated with acute shoulder pain. He was breathless at rest with supine hypoxemia. He had an amyotrophic left shoulder with localized paresis of the shoulder. Both hemi-diaphragms were elevated on chest X-rays. Pulmonary function tests showed a restrictive pattern and both phrenic nerve conduction velocities were decreased. At night, alveolar hypoventilation was evidenced by elevated mean capnography (PtcCO2: 57mmHg). Neuralgic amyotrophy, Parsonage-Turner syndrome was the final diagnosis. This syndrome is a brachial plexus neuritis with a predilection for the suprascapular and axillary nerves. Phrenic nerve involvement is rare but where present can be the most prominent clinical feature as in our case report.

  5. 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. PMID:26884697

  6. 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.

  7. Initial Treatment of Respiratory Distress Syndrome with Nasal Intermittent Mandatory Ventilation versus Nasal Continuous Positive Airway Pressure: A Randomized Controlled Trial

    PubMed Central

    Armanian, Amir-Mohammad; Badiee, Zohreh; Heidari, Ghobad; Feizi, Awat; Salehimehr, Nima

    2014-01-01

    Background: Neonatal respiratory distress syndrome (RDS) in premature infants who survived and its complications are a common problem. Due to high morbidity and mechanical ventilation (MV) nowadays researchers in interested minimizing MV. To determine, in very low birth weight (BW) preterm neonates with RDS, if initial treatment with nasal intermittent mandatory ventilation (early NIMV) compared with early nasal continuous positive airway pressure (early NCPAP) obtains more favorable outcomes in terms of the duration of treatment, and the need for endotracheal tube ventilation. Methods: In this single-center randomized control trial study, infants (BW ≤ 1500 g and/or gestational age ≤ 34 weeks) with respiratory distress were considered eligible. Forty-four infants were randomly assigned to receive early-NIMV and 54 comparable infants to early-NCPAP. Surfactants were given, when FIO2 requirement was of >30%. Primary outcomes were failure of noninvasive respiratory support, that is, the need for MV in the first 48 h of life and for the duration of noninvasive respiratory support in each group. Results: 98 infants were enrolled (44 in the NIMV and 54 in the NCPAP group). The Preventive power of MV of NIMV usage (95.5%) was not lower than the NCPAP (98.1%) strength (hazard ratio: 0.21 (95% confidence interval: 0.02-2.66); P: 0.23). The duration of noninvasive respiratory support in the NIMV group was significantly shorter than NCPAP (the median (range) was 24 (18.00-48.00) h versus 48.00 (22.00-120.00) h in NIMV versus NCPAP groups; P < 0.001). Similarly, the duration of dependency on oxygen was less, for NIMV (the median (range) was 96.00 (41.00-504.00) h versus144.00 (70.00-1130.00) h in NIMV versus NCPAP groups; P: 0.009). Interestingly, time to full enteral feeds and length of hospital stay were more favorable in the NIMV versus the NCPAP group. Conclusions: Initial treatment of RDS with NIMV was safe, and well tolerated. Furthermore, NIMV had excellent

  8. Psychological distress and stressful life events in pediatric complex regional pain syndrome

    PubMed Central

    Wager, Julia; Brehmer, Hannah; Hirschfeld, Gerrit; Zernikow, Boris

    2015-01-01

    BACKGROUND: There is little knowledge regarding the association between psychological factors and complex regional pain syndrome (CRPS) in children. Specifically, it is not known which factors precipitate CRPS and which result from the ongoing painful disease. OBJECTIVES: To examine symptoms of depression and anxiety as well as the experience of stressful life events in children with CRPS compared with children with chronic primary headaches and functional abdominal pain. METHODS: A retrospective chart study examined children with CRPS (n=37) who received intensive inpatient pain treatment between 2004 and 2010. They were compared with two control groups (chronic primary headaches and functional abdominal pain; each n=37), who also received intensive inpatient pain treatment. Control groups were matched with the CRPS group with regard to admission date, age and sex. Groups were compared on symptoms of depression and anxiety as well as stressful life events. RESULTS: Children with CRPS reported lower anxiety and depression scores compared with children with abdominal pain. A higher number of stressful life events before and after the onset of the pain condition was observed for children with CRPS. CONCLUSIONS: Children with CRPS are not particularly prone to symptoms of anxiety or depression. Importantly, children with CRPS experienced more stressful life events than children with chronic headaches or abdominal pain. Prospective long-term studies are needed to further explore the potential role of stressful life events in the etiology of CRPS. PMID:26035287

  9. 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

  10. Chronic abdominal pain due to periostitis pubis. A new syndrome.

    PubMed

    Rubenstein, N H

    1992-01-01

    Periostitis pubis is a clinical syndrome previously undescribed in the literature. It is characterized by lower abdominal pain that may have persisted for several weeks to several years. Physical findings are limited to tenderness in one of the lower abdominal quadrants and over the os pubis on the affected side. The diagnosis can be confirmed by injecting lidocaine hydrochloride into the area of point tenderness over the os pubis, which should relieve tenderness in both sites. An elaborate laboratory workup is not necessary. The condition can be cured with an injection of prednisolone tebutate at the site of tenderness over the os pubis.

  11. Altered Lipid Composition of Surfactant and Lung Tissue in Murine Experimental Malaria-Associated Acute Respiratory Distress Syndrome.

    PubMed

    Scaccabarozzi, Diletta; Deroost, Katrien; Lays, Natacha; Omodeo Salè, Fausta; Van den Steen, Philippe E; Taramelli, Donatella

    2015-01-01

    Malaria-associated acute lung injury (MA-ALI) and its more severe form malaria-associated acute respiratory distress syndrome (MA-ARDS) are common, often fatal complications of severe malaria infections. However, little is known about their pathogenesis. In this study, biochemical alterations of the lipid composition of the lungs were investigated as possible contributing factors to the severity of murine MA-ALI/ARDS. C57BL/6J mice were infected with Plasmodium berghei NK65 to induce lethal MA-ARDS, or with Plasmodium chabaudi AS, a parasite strain that does not induce lung pathology. The lipid profile of the lung tissue from mice infected with Plasmodium berghei NK65 developing MA-ALI/ARDS, but not that from mice without lung pathology or controls, was characterized by high levels of phospholipids -mainly phosphatidylcholine- and esterified cholesterol. The high levels of polyunsaturated fatty acids and the linoleic/oleic fatty acid ratio of the latter reflect the fatty acid composition of plasma cholesterol esters. In spite of the increased total polyunsaturated fatty acid pool, which augments the relative oxidability of the lung membranes, and the presence of hemozoin, a known pro-oxidant, no excess oxidative stress was detected in the lungs of Plasmodium berghei NK65 infected mice. The bronchoalveolar lavage (BAL) fluid of Plasmodium berghei NK65 infected mice was characterized by high levels of plasma proteins. The phospholipid profile of BAL large and small aggregate fractions was also different from uninfected controls, with a significant increase in the amounts of sphingomyelin and lysophosphatidylcholine and the decrease in phosphatidylglycerol. Both the increase of proteins and lysophosphatidylcholine are known to decrease the intrinsic surface activity of surfactant. Together, these data indicate that an altered lipid composition of lung tissue and BAL fluid, partially ascribed to oedema and lipoprotein infiltration, is a characteristic feature of murine

  12. Exploring the heterogeneity of effects of corticosteroids on acute respiratory distress syndrome: a systematic review and meta-analysis

    PubMed Central

    2014-01-01

    Introduction The effectiveness of corticosteroid therapy on the mortality of acute respiratory distress syndrome (ARDS) remains under debate. We aimed to explore the grounds for the inconsistent results in previous studies and update the evidence. Methods We searched MEDLINE, Cochrane Central Register of Controlled Trials and Web of Science up to December 2013. Eligible studies included randomized clinical trials (RCTs) and cohort studies that reported mortality and that had corticosteroid nonusers for comparison. The effect of corticosteroids on ARDS mortality was assessed by relative risk (RR) and risk difference (RD) for ICU, hospital, and 60-day mortality using a random-effects model. Results Eight RCTs and 10 cohort studies were included for analysis. In RCTs, corticosteroids had a possible but statistically insignificant effect on ICU mortality (RD, −0.28; 95% confidence interval (CI), −0.53 to −0.03 and RR, 0.55; 95% CI, 0.24 to 1.25) but no effect on 60-day mortality (RD, −0.01; 95% CI, −0.12 to 0.10 and RR, 0.97; 95% CI, 0.75 to 1.26). In cohort studies, corticosteroids had no effect on ICU mortality (RR, 1.05; 95% CI, 0.74 to 1.49) but non-significantly increased 60-day mortality (RR, 1.30; 95% CI, 0.96 to 1.78). In the subgroup analysis by ARDS etiology, corticosteroids significantly increased mortality in influenza-related ARDS (three cohort studies, RR, 2.45, 95% CI, 1.40 to 4.27). Conclusions The effects of corticosteroids on the mortality of ARDS differed by duration of outcome measures and etiologies. Corticosteroids did not improve longer-term outcomes and may cause harm in certain subgroups. Current data do not support routine use of corticosteroids in ARDS. More clinical trials are needed to specify the favorable and unfavorable subgroups for corticosteroid therapy. PMID:24708846

  13. 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

  14. High-frequency percussive ventilation as a salvage modality in adult respiratory distress syndrome: a preliminary study.

    PubMed

    Paulsen, Stephen M; Killyon, Garry W; Barillo, David J

    2002-10-01

    Despite multiple advances in critical care patients with severe adult respiratory distress syndrome (ARDS) can exhaust the capability of conventional ventilation; this results in respiratory failure and death. High-frequency percussive ventilation (HFPV), which was initially utilized for salvage of burn patients with smoke inhalation injury refractory to conventional ventilation, has evolved as a standard of burn care. Based on our experience with HFPV in burn patients the burn team was consulted to provide salvage ventilation for non-burn surgical intensive care unit patients with refractory respiratory failure. Over a 14-month period ten patients with refractory ARDS from multiple causes were treated. Retrospective chart review was performed. Respiratory parameters were assessed before and 24 hours after initiation of HFPV. Mean values of fraction of inspired oxygen (FiO2), pH, partial pressure of O2 in arterial blood (PaO2), partial pressure of CO2 in arterial blood (PaCO2), HCO3, oxygen saturation in arterial blood (SaO2), PaO2/FiO2, and peak inspiratory pressure were compared. Significant improvement in oxygenation was reflected by increases in SaO2, PaO2, and the PaO2/FiO2 ratio in the first 24 hours of HFPV. No significant increase in peak inspiratory pressure was documented by conversion from conventional ventilation to HFPV. No hemodynamic changes directly associated with HFPV were noted. Seven of ten patients failing conventional ventilation survived to hospital discharge after salvage therapy with HFPV. We advocate further studies of HFPV in non-burn patients with ARDS both as salvage therapy and as replacement for conventional ventilation for the initial treatment for ARDS.

  15. Does smoking in pregnancy modify the impact of antenatal steroids on neonatal respiratory distress syndrome? Results of the Epipage study

    PubMed Central

    Burguet, A; Kaminski, M; Truffert, P; Menget, A; Marpeau, L; Voyer, M; Roze, J; Escande, B; Cambonie, G; Hascoet, J; Grandjean, H; Breart, G; Larroque, B; on, b

    2005-01-01

    Objectives: To assess the relation between cigarette smoking during pregnancy and neonatal respiratory distress syndrome (RDS) in very preterm birth, and to analyse the differential effect of antenatal steroids on RDS among smokers and non-smokers. Design: A population based cohort study (the French Epipage study). Setting: Regionally defined births in France. Methods: A total of 858 very preterm liveborn singletons (27–32 completed weeks of gestation) of the French Epipage study were included in this analysis. The odds ratio for RDS in relation to smoking in pregnancy was estimated using a logistic regression to control for gestational age. The odds ratio for RDS in relation to antenatal steroids was estimated taking into account an interaction between antenatal steroids and cigarette smoking, using multiple logistic regression to control for gestational age, birthweight ratio, main causes of preterm birth, mode of delivery, and sex. Results: The odds ratio for RDS in relation to smoking in pregnancy adjusted for gestational age (aOR) was 0.59 (95% confidence interval (CI) 0.44 to 0.79). The aOR for RDS in relation to antenatal steroids was 0.31 (95% CI 0.19 to 0.49) in babies born to non-smokers and 0.63 (95% CI 0.38 to 1.05) in those born to smokers; the difference was significant (p = 0.04). Conclusions: Cigarette smoking during pregnancy is associated with a decrease in the risk of RDS in very preterm babies. Although antenatal steroids reduce the risk of RDS in babies born to both smokers and non-smokers, the reduction is smaller in those born to smokers. PMID:15613572

  16. The role of stretch-activated ion channels in acute respiratory distress syndrome: finally a new target?

    PubMed

    Schwingshackl, Andreas

    2016-09-01

    Mechanical ventilation (MV) and oxygen therapy (hyperoxia; HO) comprise the cornerstones of life-saving interventions for patients with acute respiratory distress syndrome (ARDS). Unfortunately, the side effects of MV and HO include exacerbation of lung injury by barotrauma, volutrauma, and propagation of lung inflammation. Despite significant improvements in ventilator technologies and a heightened awareness of oxygen toxicity, besides low tidal volume ventilation few if any medical interventions have improved ARDS outcomes over the past two decades. We are lacking a comprehensive understanding of mechanotransduction processes in the healthy lung and know little about the interactions between simultaneously activated stretch-, HO-, and cytokine-induced signaling cascades in ARDS. Nevertheless, as we are unraveling these mechanisms we are gathering increasing evidence for the importance of stretch-activated ion channels (SACs) in the activation of lung-resident and inflammatory cells. In addition to the discovery of new SAC families in the lung, e.g., two-pore domain potassium channels, we are increasingly assigning mechanosensing properties to already known Na(+), Ca(2+), K(+), and Cl(-) channels. Better insights into the mechanotransduction mechanisms of SACs will improve our understanding of the pathways leading to ventilator-induced lung injury and lead to much needed novel therapeutic approaches against ARDS by specifically targeting SACs. This review 1) summarizes the reasons why the time has come to seriously consider SACs as new therapeutic targets against ARDS, 2) critically analyzes the physiological and experimental factors that currently limit our knowledge about SACs, and 3) outlines the most important questions future research studies need to address. PMID:27521425

  17. Early Respiratory Management of Respiratory Distress Syndrome in Very Preterm Infants and Bronchopulmonary Dysplasia: A Case-Control Study

    PubMed Central

    te Pas, Arjan B.; Lopriore, Enrico; Engbers, Marissa J.; Walther, Frans J.

    2007-01-01

    Background In the period immediately after birth, preterm infants are highly susceptible to lung injury. Early nasal continuous positive airway pressure (ENCPAP) is an attempt to avoid intubation and may minimize lung injury. In contrast, ENCPAP can fail, and at that time surfactant rescue can be less effective. Objective To compare the pulmonary clinical course and outcome of very preterm infants (gestational age 25–32 weeks) with respiratory distress syndrome (RDS) who started with ENCPAP and failed (ECF group), with a control group of infants matched for gestational age, who were directly intubated in the delivery room (DRI group). Primary outcome consisted of death during admission or bronchopulmonary dysplasia (BPD). Results 25 infants were included in the ECF group and 50 control infants matched for gestational age were included in the DRI group. Mean gestational age and birth weight in the ECF group were 29.7 weeks and 1,393 g and in the DRI group 29.1 weeks and 1,261 g (p = NS). The incidence of BPD was significantly lower in the ECF group than in the DRI group (4% vs. 35%; P<0.004; OR 12.6 (95% CI 1.6–101)). Neonatal mortality was similar in both groups (4%). The incidence of neonatal morbidities such as severe cerebral injury, patent ductus arteriosus, necrotizing enterocolitis and retinopathy of prematurity, was not significantly different between the two groups. Conclusion A trial of ENCPAP at birth may reduce the incidence of BPD and does not seem to be detrimental in very preterm infants. Randomized controlled trials are needed to test whether early respiratory management of preterm infants with RDS plays an important role in the development of BPD. PMID:17285145

  18. High PEEP in acute respiratory distress syndrome: quantitative evaluation between improved arterial oxygenation and decreased oxygen delivery

    PubMed Central

    Das, A.; Haque, M.; Wang, W.; Bates, D. G.; Hardman, J. G.

    2016-01-01

    Background. Positive end-expiratory pressure (PEEP) is widely used to improve oxygenation and prevent alveolar collapse in mechanically ventilated patients with the acute respiratory distress syndrome (ARDS). Although PEEP improves arterial oxygenation predictably, high-PEEP strategies have demonstrated equivocal improvements in ARDS-related mortality. The effect of PEEP on tissue oxygen delivery is poorly understood and is difficult to quantify or investigate in the clinical environment. Methods. We investigated the effects of PEEP on tissue oxygen delivery in ARDS using a new, high-fidelity, computational model with highly integrated respiratory and cardiovascular systems. The model was configured to replicate published clinical trial data on the responses of 12 individual ARDS patients to changes in PEEP. These virtual patients were subjected to increasing PEEP levels during a lung-protective ventilation strategy (0–20 cm H2O). Measured variables included arterial oxygenation, cardiac output, peripheral oxygen delivery, and alveolar strain. Results. As PEEP increased, tissue oxygen delivery decreased in all subjects (mean reduction of 25% at 20 cm H2O PEEP), despite an increase in arterial oxygen tension (mean increase 6.7 kPa at 20 cm H2O PEEP). Changes in arterial oxygenation and tissue oxygen delivery differed between subjects but showed a consistent pattern. Static and dynamic alveolar strain decreased in all patients as PEEP increased. Conclusions. Incremental PEEP in ARDS appears to protect alveoli and improve arterial oxygenation, but also appears to impair tissue oxygen delivery significantly because of reduced cardiac output. We propose that this trade-off may explain the poor improvements in mortality associated with high-PEEP ventilation strategies. PMID:27799180

  19. Low-Dose Heparin Anticoagulation During Extracorporeal Life Support for Acute Respiratory Distress Syndrome in Conscious Sheep

    PubMed Central

    Prat, Nicolas J.; Meyer, Andrew D.; Langer, Thomas; Montgomery, Robbie K.; Parida, Bijaya K.; Batchinsky, Andriy I.; Cap, Andrew P.

    2015-01-01

    ABSTRACT Background: Over 32% of burned battlefield causalities develop trauma-induced hypoxic respiratory failure, also known as acute respiratory distress syndrome (ARDS). Recently, 9 out of 10 US combat soldiers’ survived life-threatening trauma-induced ARDS supported with extracorporeal membrane oxygenation (ECMO), a portable form of cardiopulmonary bypass. Unfortunately, the size, incidence of coagulation complications, and the need for systematic anticoagulation for traditional ECMO devices have prevented widespread use of this lifesaving technology. Therefore, a compact, mobile, ECMO system using minimal anticoagulation may be the solution to reduce ARDS in critically ill military and civilian patients. Methods: We conducted a prospective cohort laboratory investigation to evaluate the coagulation function in an ovine model of oleic acid induced ARDS supported with veno-venous ECMO. The experimental design approximated the time needed to transport from a battlefield setting to an advanced facility and compared bolus versus standard heparin anticoagulation therapy. Results: Comprehensive coagulation and hemostasis assays did not show any difference because of ECMO support over 10 h between the two groups but did show changes because of injury. Platelet count and function did decrease with support on ECMO, but there was no significant bleeding or clot formation during the entire experiment. Conclusions: A bolus heparin injection is sufficient to maintain ECMO support for up to 10 h in an ovine model of ARDS. With a reduced need for systematic anticoagulation, ECMO use for battlefield trauma could reduce significant morbidity and mortality from ventilator-induced lung injury and ARDS. Future studies will investigate the mechanisms and therapies to support patients for longer periods on ECMO without coagulation complications. Level of Evidence: V—therapeutic animal experiment. PMID:26263439

  20. Altered Lipid Composition of Surfactant and Lung Tissue in Murine Experimental Malaria-Associated Acute Respiratory Distress Syndrome

    PubMed Central

    Scaccabarozzi, Diletta; Deroost, Katrien; Lays, Natacha; Taramelli, Donatella

    2015-01-01

    Malaria-associated acute lung injury (MA-ALI) and its more severe form malaria-associated acute respiratory distress syndrome (MA-ARDS) are common, often fatal complications of severe malaria infections. However, little is known about their pathogenesis. In this study, biochemical alterations of the lipid composition of the lungs were investigated as possible contributing factors to the severity of murine MA-ALI/ARDS. C57BL/6J mice were infected with Plasmodium berghei NK65 to induce lethal MA-ARDS, or with Plasmodium chabaudi AS, a parasite strain that does not induce lung pathology. The lipid profile of the lung tissue from mice infected with Plasmodium berghei NK65 developing MA-ALI/ARDS, but not that from mice without lung pathology or controls, was characterized by high levels of phospholipids -mainly phosphatidylcholine- and esterified cholesterol. The high levels of polyunsaturated fatty acids and the linoleic/oleic fatty acid ratio of the latter reflect the fatty acid composition of plasma cholesterol esters. In spite of the increased total polyunsaturated fatty acid pool, which augments the relative oxidability of the lung membranes, and the presence of hemozoin, a known pro-oxidant, no excess oxidative stress was detected in the lungs of Plasmodium berghei NK65 infected mice. The bronchoalveolar lavage (BAL) fluid of Plasmodium berghei NK65 infected mice was characterized by high levels of plasma proteins. The phospholipid profile of BAL large and small aggregate fractions was also different from uninfected controls, with a significant increase in the amounts of sphingomyelin and lysophosphatidylcholine and the decrease in phosphatidylglycerol. Both the increase of proteins and lysophosphatidylcholine are known to decrease the intrinsic surface activity of surfactant. Together, these data indicate that an altered lipid composition of lung tissue and BAL fluid, partially ascribed to oedema and lipoprotein infiltration, is a characteristic feature of murine

  1. 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

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

    PubMed

    Baranovich, Tatiana; Jones, Jeremy C; Russier, Marion; Vogel, Peter; Szretter, Kristy J; Sloan, Susan E; Seiler, Patrick; Trevejo, Jose M; Webby, Richard J; Govorkova, Elena A

    2016-04-01

    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

  3. 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. PMID:25997942

  4. Role of antioxidant nutrients and lipid peroxidation in premature infants with respiratory distress syndrome and bronchopulmonary dysplasia.

    PubMed

    Falciglia, Horacio S; Johnson, J Robert; Sullivan, JoAnn; Hall, Charles F; Miller, Jeffery D; Riechmann, George C; Falciglia, Grace A

    2003-02-01

    The objective of this study was to determine if newborn premature infants with severe respiratory distress syndrome (RDS) who developed bronchopulmonary dysplasia (BPD) demonstrate, within the first 3 days of life, lower blood levels of antioxidants and higher urine levels of lipid peroxidation products than premature infants who recovered from RDS. Perinatal variables (gestational age, birth weight, and Apgar scores) and antioxidant indices in cord and in third day of life plasma and red blood cell (RBC) samples from healthy premature infants (n = 35), infants with RDS (n = 23) and infants with BPD (n = 23) were examined. Antioxidant indices included selenium, alpha-tocopherol, total and oxidized glutathione, glutathione peroxidase, superoxide dismutase, and urinary malondialdehyde. By inferential statistics, only the perinatal variables and cord plasma selenium distinguished healthy premature infants from premature infants with RDS or BPD. From perinatal variables and antioxidant indices we calculated: (1) cord to third-day-of-life variable differences, (2) variable-to-variable ratios, and (3) ratios of a difference for one variable to a difference for any second variable. Subset regression analysis yielded an equation (adjusted R2 = 0.8839) that correctly predicted infants who developed BPD 100% of the time. Predictor variables for BPD were gestational age, Apgar at 1 min, cord and third-day-of-life RBC selenium, cord total glutathione, cord and third-day-of-life glutathione peroxidase and nine different ratios involving Apgar scores, RBC selenium, total and oxidized glutathione, alpha-tocopherol, glutathione peroxidase, and superoxide dismutase. In this study, there was no relationship between lipid peroxidation and BPD. There was a higher rate of patent ductus arteriosus, congestive heart failure, and retinopathy of prematurity in infants with BPD. This study confirms that low plasma selenium and alpha-tocopherol levels in premature infants (< or = 30 weeks

  5. Association between the surfactant protein A (SP-A) gene locus and respiratory-distress syndrome in the Finnish population.

    PubMed Central

    Rämet, M; Haataja, R; Marttila, R; Floros, J; Hallman, M

    2000-01-01

    Respiratory-distress syndrome (RDS) in the newborn is a major cause of neonatal mortality and morbidity. Although prematurity is the most-important risk factor for RDS, the syndrome does not develop in many premature infants. The main cause of RDS is a deficiency of pulmonary surfactant, which consists of phospholipids and specific proteins. The genes underlying susceptibility to RDS are insufficiently known. The candidate-gene approach was used to study the association between the surfactant protein A (SP-A) gene locus and RDS in the genetically homogeneous Finnish population. In the present study, 88 infants with RDS and 88 control infants that were matched for degree of prematurity, prenatal glucocorticoid therapy, and sex were analyzed for SP-A genotypes. We show that certain SP-A1 alleles (6A2 and 6A3) and an SP-A1/SP-A2 haplotype (6A2/1A0) were associated with RDS. The 6A2 allele was overrepresented and the 6A3 allele was underrepresented in infants with RDS. These associations were particularly strong among small premature infants born at gestational age <32 wk. In infants protected from RDS (those that had no RDS, despite extreme prematurity and lack of glucocorticoid therapy), compared with infants that had RDS develop despite having received glucocorticoid therapy, the frequencies of 6A2 (.22 vs.71), 6A3 (.72 vs.17), 6A2/1A0 (.17 vs.68), 6A3/1A1 (.39 vs.10), and 6A3/1A2 (.28 vs.06) in the two groups, respectively, were strikingly different. According to the results of conditional logistic-regression analysis, diseases associated with premature birth did not explain the association between the odds of a particular homozygous SP-A1 genotype (6A2/6A2 and 6A3/6A3) and RDS. In the population evaluated in the present study, SP-B intron 4 variant frequencies were low and had no detectable association with RDS. We conclude that the SP-A gene locus is an important determinant for predisposition to RDS in premature infants. PMID:10762543

  6. Newborn Respiratory Distress.

    PubMed

    Hermansen, Christian L; Mahajan, Anand

    2015-12-01

    Newborn respiratory distress presents a diagnostic and management challenge. Newborns with respiratory distress commonly exhibit tachypnea with a respiratory rate of more than 60 respirations per minute. They may present with grunting, retractions, nasal flaring, and cyanosis. Common causes include transient tachypnea of the newborn, respiratory distress syndrome, meconium aspiration syndrome, pneumonia, sepsis, pneumothorax, persistent pulmonary hypertension of the newborn, and delayed transition. Congenital heart defects, airway malformations, and inborn errors of metabolism are less common etiologies. Clinicians should be familiar with updated neonatal resuscitation guidelines. Initial evaluation includes a detailed history and physical examination. The clinician should monitor vital signs and measure oxygen saturation with pulse oximetry, and blood gas measurement may be considered. Chest radiography is helpful in the diagnosis. Blood cultures, serial complete blood counts, and C-reactive protein measurement are useful for the evaluation of sepsis. Most neonates with respiratory distress can be treated with respiratory support and noninvasive methods. Oxygen can be provided via bag/mask, nasal cannula, oxygen hood, and nasal continuous positive airway pressure. Ventilator support may be used in more severe cases. Surfactant is increasingly used for respiratory distress syndrome. Using the INSURE technique, the newborn is intubated, given surfactant, and quickly extubated to nasal continuous positive airway pressure. Newborns should be screened for critical congenital heart defects via pulse oximetry after 24 hours but before hospital discharge. Neonatology consultation is recommended if the illness exceeds the clinician's expertise and comfort level or when the diagnosis is unclear in a critically ill newborn. PMID:26760414

  7. [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.

  8. [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. PMID:24566793

  9. Dysenteric syndrome due to Balantidium coli: a case report.

    PubMed

    Bellanger, Anne-Pauline; Scherer, Emeline; Cazorla, Arnault; Grenouillet, Frederic

    2013-04-01

    A 28-year-old man was hospitalized for a dysenteric syndrome that had developed during the previous days. Physical examination revealed abdominal pains, fever, vomiting and more than ten liquid stools per day. Fresh stool examination showed numerous mobile ciliated trophozoites of Balantidium coli. The patient reported having been on a hike the previous weekend during which he had drunk water through a hydration pouch bladder. Complete resolution was observed after intravenous rehydration and ten days of oral treatment with metronidazole (Flagyl®). Balantidium coli is the largest ciliate protozoan able to infect humans. This parasite is common in pigs and has a worldwide distribution. Human infections, a rare event in industrialised countries, are usually acquired by ingestion of food or water contaminated by mammal faeces. Human B. coli infections are easily treated but may be severe and even fatal if neglected. PMID:23686128

  10. Sudden death of a patient with pandemic influenza (A/H1N1pdm) virus infection by acute respiratory distress syndrome.

    PubMed

    Takiyama, Akihiro; Wang, Lei; Tanino, Mishie; Kimura, Taichi; Kawagishi, Naoki; Kunieda, Yasuyuki; Katano, Harutaka; Nakajima, Noriko; Hasegawa, Hideki; Takagi, Tomoyuki; Nishihara, Hiroshi; Sata, Tetsutaro; Tanaka, Shinya

    2010-01-01

    We describe an autopsy case of a patient with pandemic influenza (A/H1N1pdm) virus infection in Japan, who developed rapidly progressive viral pneumonia exhibiting diffuse alveolar damage. A 41-year-old female visited our hospital with a fever of 38.7C. She was a public health nurse with no underlying disease and had had contact with a group of elementary school students who had been infected with the influenza (A/H1N1pdm) virus 1 week earlier. She was prescribed oseltamivir and returned to the hotel where she was staying alone. The next day, she was found dead in her hotel room. At autopsy, both lungs were voluminous and microscopic examination revealed acute-stage, severe diffuse alveolar damage with remarkable mononuclear cell infiltration and hyaline membrane formation in the lungs. CD8-positive T lymphocytes were dominantly observed. Immunohistochemically, influenza A viral protein was confirmed in the damaged type II pneumocytes and also in the infiltrated macrophages. Real-time RT-PCR analysis of both pre- and post-mortem pharyngeal swabs confirmed a novel influenza (A/H1N1pdm) virus infection. This is the second autopsy case of influenza (A/H1N1pdm) virus infection in Japan, and the findings indicated that the patient died due to an exceptionally rapid progression of viral pneumonia. This case indicates that patients with influenza (A/H1N1pdm) virus infection should be carefully monitor for acute respiratory distress syndrome. PMID:20093769

  11. 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

  12. Coupled plasma filtration adsorption for the treatment of a patient with acute respiratory distress syndrome and acute kidney injury: a case report.

    PubMed

    Lucisano, Gaetano; Capria, Maria; Matera, Giovanni; Presta, Pierangela; Comi, Nicolino; Talarico, Roberta; Rametti, Linda; Quirino, Angela; Giancotti, Aida; Fuiano, Giorgio

    2011-10-01

    Coupled plasma filtration adsorption (CPFA) is an extracorporeal blood purification therapy based on non-specific pro- and anti-inflammatory mediator adsorption on a special resin cartridge coupled with continuous veno-venous haemofiltration or continuous veno-venous haemodiafiltration and is one of the emerging treatments for septic patients. However, in the literature, there are limited data about its efficacy in treating patients with acute diseases but without the traditional criteria for sepsis. We describe the case of a 43-year-old male who developed acute respiratory distress syndrome secondary to pneumonia and acute kidney injury, whose clinical conditions rapidly improved after early CPFA therapy.

  13. Coupled plasma filtration adsorption for the treatment of a patient with acute respiratory distress syndrome and acute kidney injury: a case report

    PubMed Central

    Lucisano, Gaetano; Capria, Maria; Matera, Giovanni; Presta, Pierangela; Comi, Nicolino; Talarico, Roberta; Rametti, Linda; Quirino, Angela; Giancotti, Aida

    2011-01-01

    Coupled plasma filtration adsorption (CPFA) is an extracorporeal blood purification therapy based on non-specific pro- and anti-inflammatory mediator adsorption on a special resin cartridge coupled with continuous veno-venous haemofiltration or continuous veno-venous haemodiafiltration and is one of the emerging treatments for septic patients. However, in the literature, there are limited data about its efficacy in treating patients with acute diseases but without the traditional criteria for sepsis. We describe the case of a 43-year-old male who developed acute respiratory distress syndrome secondary to pneumonia and acute kidney injury, whose clinical conditions rapidly improved after early CPFA therapy. PMID:25984170

  14. Incidence and Outcomes of Acute Respiratory Distress Syndrome: A Nationwide Registry-Based Study in Taiwan, 1997 to 2011.

    PubMed

    Chen, Wei; Chen, Yih-Yuan; Tsai, Ching-Fang; Chen, Solomon Chih-Cheng; Lin, Ming-Shian; Ware, Lorraine B; Chen, Chuan-Mu

    2015-10-01

    Most epidemiological studies of acute respiratory distress syndrome (ARDS) have been conducted in western countries, and studies in Asia are limited. The aim of our study was to evaluate the incidence, in-hospital mortality, and 1-year mortality of ARDS in Taiwan.We conducted a nationwide inpatient cohort study based on the Taiwan National Health Insurance Research Database between 1997 and 2011. A total of 40,876 ARDS patients (68% male; mean age 66 years) were identified by International Classification of Diseases, 9th edition coding and further analyzed for clinical characteristics, medical costs, and mortality.The overall crude incidence of ARDS was 15.74 per 100,000 person-years, and increased from 2.53 to 19.26 per 100,000 person-years during the study period. The age-adjusted incidence of ARDS was 15.19 per 100,000 person-years. The overall in-hospital mortality was 57.8%. In-hospital mortality decreased from 59.7% in 1997 to 47.5% in 2011 (P < 0.001). The in-hospital mortality rate was lowest (33.5%) in the youngest patients (age 18-29 years) and highest (68.2%) in the oldest patients (>80 years, P < 0.001). The overall 1-year mortality rate was 72.1%, and decreased from 75.8% to 54.7% during the study period. Patients who died during hospitalization were older (69 ± 17 versus 62 ± 19, P < 0.001) and predominantly male (69.8% versus 65.3%, P < 0.001). In addition, patients who died during hospitalization had significantly higher medical costs (6421 versus 5825 US Dollars, P < 0.001) and shorter lengths of stay (13 versus 19 days, P < 0.001) than patients who survived.We provide the first large-scale epidemiological analysis of ARDS incidence and outcomes in Asia. Although the overall incidence was lower than has been reported in a prospective US study, this may reflect underdiagnosis by International Classification of Diseases, 9th edition code and identification of only patients with more severe ARDS in this analysis

  15. Dual hit lipopolysaccharide & oleic acid combination induced rat model of acute lung injury/acute respiratory distress syndrome

    PubMed Central

    Hagawane, T.N.; Gaikwad, R.V.; Kshirsagar, N.A.

    2016-01-01

    Background & objectives: Despite advances in therapy and overall medical care, acute lung injury (ALI)/acute respiratory distress syndrome (ARDS) management remains a problem. Hence the objective of this study was to develop a rat model that mimics human ALI/ARDS. Methods: Four groups of Wistar rats, 48 per group were treated with (i) intratracheal (IT) lipopolysaccharide (LPS) (5 mg/kg) dissolved in normal saline (NS), (ii) intravenous (iv) oleic acid (OA) (250 μl/kg) suspension in bovine serum albumin (BSA), (iii) dual hit: IT LPS (2 mg/kg) dissolved in NS and iv OA (100 μl/kg) and (iv) control group: IT NS and iv BSA. From each group at set periods of time various investigations like chest X-rays, respiratory rate (RR), tidal volume (TV), total cell count, differential cell count, total protein count and cytokine levels in bronchoalveolar lavage fluid (BALF), lung wet/dry weight ratio and histopathological examination were done. Results: It was noted that the respiratory rate, and tumour necrosis factor-α (TNF-α) levels were significantly higher at 4 h in the dual hit group as compared to LPS, OA and control groups. Interleukin-6 (IL-6) levels were significantly higher in the dual hit group as compared to LPS at 8 and 24 h, OA at 8 h and control (at all time intervals) group. IL-1β levels were significantly higher in LPS and dual hit groups at all time intervals, but not in OA and control groups. The injury induced in dual hit group was earlier and more sustained as compared to LPS and OA alone. Interpretation & conclusions: The lung pathology and changes in respiration functions produced by the dual hit model were closer to the diagnostic criteria of ALI/ARDS in terms of clinical manifestations and pulmonary injury and the injury persisted longer as compared to LPS and OA single hit model. Therefore, the ARDS model produced by the dual hit method was closer to the diagnostic criteria of ARDS in terms of clinical manifestations and pulmonary injury. PMID

  16. Neutrophil apoptosis: a marker of disease severity in sepsis and sepsis-induced acute respiratory distress syndrome

    PubMed Central

    Fialkow, Léa; Fochesatto Filho, Luciano; Bozzetti, Mary C; Milani, Adriana R; Rodrigues Filho, Edison M; Ladniuk, Roberta M; Pierozan, Paula; de Moura, Rafaela M; Prolla, João C; Vachon, Eric; Downey, Gregory P

    2006-01-01

    Introduction Apoptosis of neutrophils (polymorphonuclear neutrophils [PMNs]) may limit inflammatory injury in sepsis and acute respiratory distress syndrome (ARDS), but the relationship between the severity of sepsis and extent of PMN apoptosis and the effect of superimposed ARDS is unknown. The objective of this study was to correlate neutrophil apoptosis with the severity of sepsis and sepsis-induced ARDS. Methods A prospective cohort study was conducted in intensive care units of three tertiary hospitals in Porto Alegre, southern Brazil. Fifty-seven patients with sepsis (uncomplicated sepsis, septic shock, and sepsis-induced ARDS) and 64 controls were enrolled. Venous peripheral blood was collected from patients with sepsis within 24 hours of diagnosis. All surgical groups, including controls, had their blood drawn 24 hours after surgery. Control patients on mechanical ventilation had blood collected within 24 hours of initiation of mechanical ventilation. Healthy controls were blood donors. Neutrophils were isolated, and incubated ex vivo, and apoptosis was determined by light microscopy on cytospun preparations. The differences among groups were assessed by analysis of variance with Tukeys. Results In medical patients, the mean percentage of neutrophil apoptosis (± standard error of the mean [SEM]) was lower in sepsis-induced ARDS (28% ± 3.3%; n = 9) when compared with uncomplicated sepsis (57% ± 3.2%; n = 8; p < 0.001), mechanical ventilation without infection, sepsis, or ARDS (53% ± 3.0%; n = 11; p < 0.001) and healthy controls (69% ± 1.1%; n = 33; p < 0.001) but did not differ from septic shock (38% ± 3.7%; n = 12; p = 0.13). In surgical patients with sepsis, the percentage of neutrophil apoptosis was lower for all groups when compared with surgical controls (52% ± 3.6%; n = 11; p < 0.001). Conclusion In medical patients with sepsis, neutrophil apoptosis is inversely proportional to the severity of sepsis and thus may be a marker of the severity of

  17. Receptor Interacting Protein 3-Mediated Necroptosis Promotes Lipopolysaccharide-Induced Inflammation and Acute Respiratory Distress Syndrome in Mice

    PubMed Central

    Li, Haobo; Liu, Qing; Zhang, Zhongjun; Xie, Wanli; Feng, Yinglu; Socorburam, Tumenjavkhlan; Wu, Gui; Xia, Zhengyuan; Wu, Qingping

    2016-01-01

    Necrosis amplifies inflammation and plays important roles in acute respiratory distress syndrome (ARDS). Necroptosis is a newly identified programmed necrosis that is mediated by receptor interacting protein 3 (RIP3). However, the potential involvement and impact of necroptosis in lipopolysaccharide (LPS)-induced ARDS remains unknown. We therefore explored the role and mechanism of RIP3-mediated necroptosis in LPS-induced ARDS. Mice were instilled with increasing doses of LPS intratracheally to induce different degrees of ARDS. Lung tissues were harvested for histological and TUNEL staining and western blot for RIP3, p-RIP3, X-linked inhibitor of apoptosis protein (XIAP), mixed lineage kinase domain-like protein (MLKL), total and cleaved caspases-3/8. Then, wild-type and RIP3 knock-out mice were induced ARDS with 30 mg/kg LPS. Pulmonary cellular necrosis was labeled by the propidium Iodide (PI) staining. Levels of TNF-a, Interleukin (IL)-1β, IL-6, IL-1α, IL-10 and HMGB1, tissue myeloperoxidase (MPO) activity, neutrophil counts and total protein concentration were measured. Results showed that in high dose LPS (30mg/kg and 40mg/kg) -induced severe ARDS, RIP3 protein was increased significantly, accompanied by increases of p-RIP3 and MLKL, while in low dose LPS (10mg/kg and 20mg/kg) -induced mild ARDS, apoptosis was remarkably increased. In LPS-induced severe ARDS, RIP3 knock-out alleviated the hypothermia symptom, increased survival rate and ameliorated the lung tissue injury RIP3 depletion also attenuated LPS-induced increase in IL-1α/β, IL-6 and HMGB1 release, decreased tissue MPO activity, and reduced neutrophil influx and total protein concentration in BALF in severe ARDS. Further, RIP3 depletion reduced the necrotic cells in the lung and decreased the expression of MLKL, but had no impact on cleaved caspase-3 in LPS-induced ARDS. It is concluded that RIP3-mediated necroptosis is a major mechanism of enhanced inflammation and lung tissue injury in high dose

  18. Expiratory washout versus optimization of mechanical ventilation during permissive hypercapnia in patients with severe acute respiratory distress syndrome.

    PubMed

    Richecoeur, J; Lu, Q; Vieira, S R; Puybasset, L; Kalfon, P; Coriat, P; Rouby, J J

    1999-07-01

    The aim of this study was to compare three ventilatory techniques for reducing PaCO2 in patients with severe acute respiratory distress syndrome treated with permissive hypercapnia: (1) expiratory washout alone at a flow of 15 L/min, (2) optimized mechanical ventilation defined as an increase in the respiratory frequency to the maximal rate possible without development of intrinsic positive end- expiratory pressure (PEEP) combined with a reduction of the instrumental dead space, and (3) the combination of both methods. Tidal volume was set according to the pressure-volume curve in order to obtain an inspiratory plateau airway pressure equal to the upper inflection point minus 2 cm H2O after setting the PEEP at 2 cm H2O above the lower inflection point and was kept constant throughout the study. The three modalities were compared at the same inspiratory plateau airway pressure through an adjustment of the extrinsic PEEP. During conventional mechanical ventilation using a respiratory frequency of 18 breaths/min, respiratory acidosis (PaCO2 = 84 +/- 24 mm Hg and pH = 7.21 +/- 0.12) was observed. Expiratory washout and optimized mechanical ventilation (respiratory frequency of 30 +/- 4 breaths/min) had similar effects on CO2 elimination (DeltaPaCO2 = -28 +/- 11% versus -27 +/- 12%). A further decrease in PaCO2 was observed when both methods were combined (DeltaPaCO2 = -46 +/- 7%). Extrinsic PEEP had to be reduced by 5.3 +/- 2.1 cm H2O during expiratory washout and by 7.3 +/- 1.3 cm H2O during the combination of the two modes, whereas it remained unchanged during optimized mechanical ventilation alone. In conclusion, increasing respiratory rate and reducing instrumental dead space during conventional mechanical ventilation is as efficient as expiratory washout to reduce PaCO2 in patients with severe ARDS and permissive hypercapnia. When used in combination, both techniques have additive effects and result in PaCO2 levels close to normal values. PMID:10390383

  19. Albumin versus crystalloid solutions in patients with the acute respiratory distress syndrome: a systematic review and meta-analysis

    PubMed Central

    2014-01-01

    Introduction In patients with acute respiratory distress syndrome (ARDS) fluid therapy might be necessary. The aim of this systematic review and meta-analysis is to determine the effects of colloid therapy compared to crystalloids on mortality and oxygenation in adults with ARDS. Methods Randomized controlled trials (RCTs) were identified through a systematic literature search of MEDLINE, EMBASE, CENTRAL and LILACS. Articles published up to 15th February 2013 were independently screened, abstracted, and assessed (Cochrane Risk of Bias Tool) to provide evidence-based therapy recommendations. RCTs were eligible if they compared colloid versus crystalloid therapy on lung function, inflammation, damage or mortality in adults with ARDS. Primary outcome parameters were respiratory mechanics, gas exchange lung inflammation and damage as well as hospital mortality. Kidney function, need for renal replacement therapy, hemodynamic stabilization and intensive care unit (ICU) length of stay served as secondary outcomes. Results A total of 3 RCTs out of 4130 potential trials found in the databases were selected for qualitative and quantitative analysis totaling 206 patients who received either albumin or saline. Overall risk of bias was unclear to high in the identified trials. Calculated pooled risk of death was not statistically significant (albumin 34 of 100 (34.0%) versus 40 of 104 (38.5%), relative risk (RR) = 0.89, 95% confidence interval (CI) 0.62 to 1.28, P = 0.539). Weighted mean difference (WMD) in PaO2/FiO2 (mmHg) improved in the first 48 hours (WMD = 62, 95% CI 47 to 77, P <0.001, I2 = 0%) after therapy start and remained stable after 7 days (WMD = 20, 95% CI 4 to 36, P = 0.017, I2 = 0%). Conclusions There is a high need for RCTs investigating the effects of colloids in ARDS patients. Based on the findings of this review, colloid therapy with albumin improved oxygenation but did not affect mortality. PMID:24405693

  20. Incidence and Outcomes of Acute Respiratory Distress Syndrome: A Nationwide Registry-Based Study in Taiwan, 1997 to 2011.

    PubMed

    Chen, Wei; Chen, Yih-Yuan; Tsai, Ching-Fang; Chen, Solomon Chih-Cheng; Lin, Ming-Shian; Ware, Lorraine B; Chen, Chuan-Mu

    2015-10-01

    Most epidemiological studies of acute respiratory distress syndrome (ARDS) have been conducted in western countries, and studies in Asia are limited. The aim of our study was to evaluate the incidence, in-hospital mortality, and 1-year mortality of ARDS in Taiwan.We conducted a nationwide inpatient cohort study based on the Taiwan National Health Insurance Research Database between 1997 and 2011. A total of 40,876 ARDS patients (68% male; mean age 66 years) were identified by International Classification of Diseases, 9th edition coding and further analyzed for clinical characteristics, medical costs, and mortality.The overall crude incidence of ARDS was 15.74 per 100,000 person-years, and increased from 2.53 to 19.26 per 100,000 person-years during the study period. The age-adjusted incidence of ARDS was 15.19 per 100,000 person-years. The overall in-hospital mortality was 57.8%. In-hospital mortality decreased from 59.7% in 1997 to 47.5% in 2011 (P < 0.001). The in-hospital mortality rate was lowest (33.5%) in the youngest patients (age 18-29 years) and highest (68.2%) in the oldest patients (>80 years, P < 0.001). The overall 1-year mortality rate was 72.1%, and decreased from 75.8% to 54.7% during the study period. Patients who died during hospitalization were older (69 ± 17 versus 62 ± 19, P < 0.001) and predominantly male (69.8% versus 65.3%, P < 0.001). In addition, patients who died during hospitalization had significantly higher medical costs (6421 versus 5825 US Dollars, P < 0.001) and shorter lengths of stay (13 versus 19 days, P < 0.001) than patients who survived.We provide the first large-scale epidemiological analysis of ARDS incidence and outcomes in Asia. Although the overall incidence was lower than has been reported in a prospective US study, this may reflect underdiagnosis by International Classification of Diseases, 9th edition code and identification of only patients with more severe ARDS in this analysis

  1. Early administration of surfactant via a thin intratracheal catheter in preterm infants with respiratory distress syndrome: Feasibility and outcome

    PubMed Central

    Mohammadizadeh, Majid; Ardestani, Azam Ghehsareh; Sadeghnia, Ali Reza

    2015-01-01

    Objective: Currently, the method of early nasal continuous positive airway pressure (nCPAP) and selective administration of surfactant via an endotracheal tube is widely used in the treatment of respiratory distress syndrome (RDS) in premature infants. To prevent complications related to endotracheal intubation and even a brief period of mechanical ventilation, in this study, we compared the effectiveness of surfactant administration via a thin intratracheal catheter versus the current method using an endotracheal tube. Methods: Thirty eight preterm infants ≤34 weeks' gestation with birth weight of 1000–1800 g who were putted on nCPAP for RDS within the first hour of life, were randomly assigned to receive surfactant either via endotracheal tube (ET group) or via thin intratracheal catheter (CATH group). The primary outcomes were the need for mechanical ventilation and duration of oxygen therapy. Data were analyzed by independent t-test, Mann-Whitney U-test, and Chi-square test, using SPSS v. 21. Findings: There was no significant difference between groups regarding to need for mechanical ventilation during the first 72 h of birth (3 [15.8%] in ET group vs. 2 [10.5%] in CATH group; P = 0.99). Duration of oxygen therapy in CATH group was significantly lower than ET group (243.7 ± 74.3 h vs. 476.8 ± 106.8 h, respectively; P = 0.018). The incidence of adverse events during all times of surfactant administration was not statistically significant between groups (P = 0.14), but the number of infants who experienced adverse events during surfactant administration was significantly lower in CATH group than ET group (6 [31.6%] vs. 12 [63.2%], respectively; P = 0.049). All other outcomes, including duration of treatment with CPAP and mechanical ventilation, times of surfactant administration and the need for more than one dose of the drug, the rate of intraventricular hemorrhage, mortality and combined outcome of chronic lung disease or mortality were statistically

  2. 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

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

    PubMed

    Fitzgerald, Marianne; Millar, Jonathan; Blackwood, Bronagh; Davies, Andrew; Brett, Stephen J; McAuley, Daniel F; McNamee, James J

    2014-05-15

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

  4. 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

  5. Gluteal compartment syndrome due to prolonged immobilization after alcohol intoxication: a case report.

    PubMed

    Iizuka, Shinichi; Miura, Naoyuki; Fukushima, Tomokazu; Seki, Tomoko; Sugimoto, Katuhiko; Inokuchi, Sadaki

    2011-07-01

    Gluteal compartment syndrome is a relatively rare condition that mostly result from atraumatic causes such as prolonged immobilization due to drug abuse or alcoholic intoxication and incorrect positioning during surgical procedures rather than traumatic causes. Early diagnosis is difficult and sometimes delayed or overlooked because of poor physical signs resulting from altered mental status and inappropriate diagnosis by clinicians. It has been reported that more than half of the cases of gluteal compartment syndrome are associated with crush syndrome and sciatic nerve palsy. Early diagnosis and immediate fasciotomy are necessary to improve the functional prognosis. Here, we report the case of a patient with gluteal compartment syndrome caused by prolonged immobilization after acute alcoholic intoxication. After disease onset, the patient developed complications of crush syndrome and sciatic nerve palsy, but immediate fasciotomy improved his condition.

  6. Acute respiratory distress syndrome

    MedlinePlus

    ... and stiff, which decreases the lungs' ability to expand. The level of oxygen in the blood can ... Tests used to diagnose ARDS include: Arterial blood gas Blood tests, including CBC and blood chemistries Blood ...

  7. Respiratory Distress Syndrome

    MedlinePlus

    ... their lungs aren't able to make enough surfactant (sur-FAK-tant). Surfactant is a liquid that coats the inside of ... in air once they're born. Without enough surfactant, the lungs collapse and the infant has to ...

  8. 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. PMID:27365560

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

    PubMed Central

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

    2016-01-01

    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. PMID:27365560

  10. 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.

  11. Extra-Pontine Myelinolysis in a Case of Pan-hypopituitarism Due to Empty Sella Syndrome.

    PubMed

    Gupta, Vishal Anand; Karnik, Niteen; Itolikar, Manish; Vekariya, Ketan

    2015-10-01

    Rapid correction of hyponatremia is known to cause central pontine myelinolysis (CPM). It may concurrently involve other areas of brain as well, referred as extra-pontine myelinolysis (EPM). Isolated EPM however is a very rare occurrence. We present a case of EPM where the hyponatremia was secondary to hypothyroidism due to empty sella syndrome. Chronic hyponatremia should always be corrected slowly to avoid such osmotic myelinolysis syndromes (OMS). PMID:27608705

  12. 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. PMID:27409001

  13. Abnormal cerebellar development and axonal decussation due to mutations in AHI1 in Joubert syndrome.

    PubMed

    Ferland, Russell J; Eyaid, Wafaa; Collura, Randall V; Tully, Laura D; Hill, R Sean; Al-Nouri, Doha; Al-Rumayyan, Ahmed; Topcu, Meral; Gascon, Generoso; Bodell, Adria; Shugart, Yin Yao; Ruvolo, Maryellen; Walsh, Christopher A

    2004-09-01

    Joubert syndrome is a congenital brain malformation of the cerebellar vermis and brainstem with abnormalities of axonal decussation (crossing in the brain) affecting the corticospinal tract and superior cerebellar peduncles. Individuals with Joubert syndrome have motor and behavioral abnormalities, including an inability to walk due to severe clumsiness and 'mirror' movements, and cognitive and behavioral disturbances. Here we identified a locus associated with Joubert syndrome, JBTS3, on chromosome 6q23.2-q23.3 and found three deleterious mutations in AHI1, the first gene to be associated with Joubert syndrome. AHI1 is most highly expressed in brain, particularly in neurons that give rise to the crossing axons of the corticospinal tract and superior cerebellar peduncles. Comparative genetic analysis of AHI1 indicates that it has undergone positive evolutionary selection along the human lineage. Therefore, changes in AHI1 may have been important in the evolution of human-specific motor behaviors.

  14. Anomalous responses to stimulation and suppression tests in Cushing's syndrome due to a calcified adrenal adenoma

    PubMed Central

    Epstein, S.; McLaren, E. H.; Goldin, A. R.

    1973-01-01

    A case of Cushing's syndrome, due to an adrenal adenoma, which responded to dexamethasone with a rise in plasma urinary steroids is described. Further unusual features were radiologically visible calcification and a response to ACTH stimulation. ImagesFig. 1Fig. 2 PMID:4377169

  15. 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.

  16. The Zollinger-Ellison syndrome due to an infiltrating tumour of the stomach

    PubMed Central

    Royston, C. M. S.; Brew, D. St. J.; Garnham, J. R.; Stagg, B. H.; Polak, Julia

    1972-01-01

    This is the first case report of the Zollinger-Ellison syndrome due to an infiltrating tumour of the stomach. Plasma gastrin levels were high and gastrin was demonstrated in argyrophil tumour cells by an immunofluorescent technique. Evidence is presented that the tumour arose from the G cells. ImagesFig. 1Fig. 2Fig. 3Fig. 4Fig. 5Fig. 6 PMID:4562021

  17. 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. PMID:26628826

  18. Can we Replace Arterial Blood Gas Analysis by Pulse Oximetry in Neonates with Respiratory Distress Syndrome, who are Treated According to INSURE Protocol?

    PubMed Central

    Niknafs, Pedram; Norouzi, Elahe; Bahman Bijari, Bahareh; Baneshi, Mohammad Reza

    2015-01-01

    Neonates with respiratory distress syndrome (RDS), who are treated according to INSURE protocol; require arterial blood gas (ABG) analysis to decide on appropriate management. We conducted this study to investigate the validity of pulse oximetry instead of frequent ABG analysis in the evaluation of these patients. From a total of 193 blood samples obtained from 30 neonates <1500 grams with RDS, 7.2% were found to have one or more of the followings: acidosis, hypercapnia, or hypoxemia. We found that pulse oximetry in the detection of hyperoxemia had a good validity to appropriately manage patients without blood gas analysis. However, the validity of pulse oximetry was not good enough to detect acidosis, hypercapnia, and hypoxemia. PMID:25999627

  19. Act fast and ventilate soft: the Düsseldorf hands-on translation of the acute respiratory distress syndrome Berlin definition.

    PubMed

    Luedike, Peter; Totzeck, Matthias; Meyer, Christian; Westenfeld, Ralf; Kindgen-Milles, Detlef; Kelm, Malte; Rassaf, Tienush

    2014-10-01

    Early identification of acute respiratory distress syndrome (ARDS) and forceful implementation of standardized therapy algorithms are the mandatory basis of an effective therapy to improve patient outcome. Recently, a new definition of ARDS was implemented, which simplified the diagnostic criteria for ARDS. Evidence-based therapies are rare, but some cornerstone interventions can be recommended. Lung-protective ventilation with high positive end-expiratory pressure and low tidal volume and early prone positioning in severe cases improve survival rate. We here present an integrated "Düsseldorf hands-on translation" in the form of a "one-page" standard operating procedure in order to fasten and standardize both diagnosis and therapeutic algorithms on an intensive care unit.

  20. 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

  1. 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

  2. Acute left-arm compartment syndrome due to cephalic arch stenosis in a dialysis patient.

    PubMed

    Lee, Wei-Yang; Hsu, Hsiang-Hao; Yen, Tzung-Hai; Wang, Li-Jen; Lee, Shen-Yang

    2013-01-01

    Acute compartment syndrome of the upper limb due to dialysis access-related bleeding is a rare and severe complication of hemodialysis. In most reported cases, this complication is caused by an enlarging hematoma after puncture or perforation of a fistula in combination with the use of heparin. In this case report, we describe a 52-year-old woman presenting with venous hypertension and left-arm swelling that progressed suddenly on the fifth day of presentation to neurological deficits, cyanotic skin changes, and typical clinical symptoms of acute arm compartment syndrome. An angiographic scan confirmed a critical stenosis at the proximal cephalic-axillary venous junction, and balloon angioplasty successfully dilated the lesion. The arm swelling and other symptoms subsided dramatically within 3 days. This case represents a potentially different mechanism for the development of acute arm compartment syndrome in dialysis patients as well as a treatment strategy different from standard fasciotomy to reduce intracompartmental pressure.

  3. Case report: acute forearm compartment syndrome due to sudden, forceful supination of the hand.

    PubMed

    Pantle, Hardin A; Chanmugam, Arjun

    2010-10-01

    Acute compartment syndrome is a limb-threatening condition if not recognized and treated promptly. Appropriate management includes early fasciotomy, which often results in better functional outcomes. Although there are many causes of compartment syndrome, the common findings are significant pain, swelling, and limited range of motion. Diagnosis is usually based on physical findings in the setting of a compelling history. Before surgical intervention, the diagnosis is usually confirmed by measuring elevated compartment pressures. The patient described in this case report developed acute compartment syndrome of the forearm after his hand became trapped in machinery that applied sudden supination to the hand, and avulsed the distal portion of the left index finger. There was no direct trauma to the forearm. In this case, acute compartment syndrome was likely due to a combination of contained hemorrhage into the muscle sheath, closed muscle strain causing edema, and possibly axial traction applied to the tendons of the index finger. Acute compartment syndrome should be considered in the differential diagnosis for any patient complaining of severe pain in an extremity, even in the absence of commonly recognized mechanisms of injury.

  4. Management of Neonatal Respiratory Distress Syndrome Employing ACoRN Respiratory Sequence Protocol versus Early Nasal Continuous Positive Airway Pressure Protocol

    PubMed Central

    Niknafs, Pedram; Faghani, Asadallah; Afjeh, Seyed-Abolfazl; Moradinazer, Mehdi; Bahman-Bijari, Bahareh

    2014-01-01

    Objective: Respiratory distress syndrome (RDS) is a common cause of respiratory distress in premature infants. This study was designed to evaluate two different RDS treatment protocols by comparing the outcomes. Methods: This study was a double center cross sectional study performed from June to December 2012. During that period, 386 neonates with RDS were hospitalized and treated according to two different therapeutic protocols so-called Acute Care of at-Risk Newborns (ACoRN) respiratory sequence protocol (group I) and Early Nasal Continuous Positive Airway Pressure (E-NCPAP) protocol (group II). The variables and main outcomes of this study were gestational age, birth weight, bronchopulmonary dysplasia (BPD), pulmonary hemorrhage (PH), intraventricular hemorrhage (IVH), air leak and mortality rate (MR). Findings : Out of 386 infants, 202 infants were in group I (male 60.4%, female 39.6%, mean gestational age 316/7 weeks, mean birth weight=1688 grams) and group II included 184 infants (male 61.4%, female 38.6%, mean gestational age 32 weeks, mean birth weight 1787 grams), P= 0.07. The ratios of BPD of group I to group II and PH of group I to group two were not significant (P=0.63 and P=0.84, respectively). Air leak ratio in group I was higher than in group II (P=0.001). Although IVH ratio in group II was higher than in group I (P=0.01), grade III and IV IVH was higher in group I (30% vs. 4.6%). In case of MR, it was higher in group I than in group II (P=0.001). Conclusion: According to the findings the incidence of air leak, grade III and IV IVH and MR was less common in E-NCPAP protocol, so it may show the effectiveness of this protocol. The authors suggest that more researches are needed for more accurate results. PMID:25793046

  5. Rapunzel syndrome due to ingested hair extensions: Surgical and psychiatric considerations

    PubMed Central

    Flaherty, Devin C.; Aguilar, Francis; Pradhan, Basant; Grewal, Harsh

    2015-01-01

    Introduction Rapunzel syndrome is a unique clinical manifestation of trichotillomania and trichophagia. The resulting gastric trichobezoar can be massive and necessitate surgical extraction. Presentation of case We present a case involving a 15 year-old female with a known history of trichotillomania. The patient possessed symptoms of nausea and early satiety, and admitted that she had recently consumed a large number of hair extensions. Computed tomography imaging revealed a massive gastric bezoar not amenable to endoscopic extraction. The patient underwent surgical laparotomy, and a large gastric trichobezoar was removed through an anterior gastrotomy. The trichobezoar extended past the pylorus into the duodenum, thus confirming a diagnosis of Rapunzel syndrome. Discussion Trichobezoars formed from the consumption of hair extensions is a rare cause of Rapunzel syndrome. Surgical extraction is often necessitated due to the sheer size of the gastric bezoar that results from consuming hair extensions. If not previously established, psychiatric consultation should be pursued in the immediate post-operative course as these patients may require pharmacologic management along with behavioral therapy to avoid further episodes of trichotillomania. Conclusion This case presents a unique and modern manifestation of Rapunzel syndrome. Surgical treatment most often is required when a patient presents with a massive gastric trichobezoar. Regular post-operative psychiatric follow-up is necessary to prevent recurrent episodes. PMID:26629852

  6. 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

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

    PubMed Central

    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. PMID:26491527

  8. Pathologic Femoral Neck Fracture Due to Fanconi Syndrome Induced by Adefovir Dipivoxil Therapy for Hepatitis B.

    PubMed

    Lee, Yoon-Suk; Kim, Byung-Kook; Lee, Ho-Jae; Dan, Jinmyoung

    2016-06-01

    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

  9. 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.

  10. Carcinoid syndrome, acromegaly, and hypoglycemia due to an insulin-secreting neuroendocrine tumor of the liver.

    PubMed

    Furrer, J; Hättenschwiler, A; Komminoth, P; Pfammatter, T; Wiesli, P

    2001-05-01

    We report a patient with a hepatic neuroendocrine tumor showing an extraordinary change of the tumor's humoral manifestations from a clinically documented extrapituitary acromegaly and a typical carcinoid syndrome toward a hyperinsulinemic hypoglycemia syndrome. At the primary manifestation of the tumor, an increased serum level of insulin-like growth factor I due to overproduction of GHRH and an increased urinary excretion of 5-hydroxyindoleacetic acid were found. The clinical manifestation of the GHRH excess was an arthralgia, which resolved completely after operative tumor debulking and normalization of insulin-like growth factor I and GHRH serum levels. The secretion of serotonin from the tumor resulted in a typical carcinoid syndrome including right-sided valvular heart disease. On the later course of the disease, the humoral manifestations of the tumor were supplemented by the secretion of insulin, leading to recurrent severe hyperinsulinemic hypoglycemia. The hepatic origin of hyperinsulinism was demonstrated by selective arterial calcium stimulation. Moreover, tumor cells revealed insulin and C-peptide immunoreactivity in the immunohistochemical analysis. The patient died 8 yr after the initial diagnosis of the tumor, and a carefully performed autopsy procedure confirmed the absence of any extrahepatic tumor manifestation. PMID:11344231

  11. Carcinoid syndrome, acromegaly, and hypoglycemia due to an insulin-secreting neuroendocrine tumor of the liver.

    PubMed

    Furrer, J; Hättenschwiler, A; Komminoth, P; Pfammatter, T; Wiesli, P

    2001-05-01

    We report a patient with a hepatic neuroendocrine tumor showing an extraordinary change of the tumor's humoral manifestations from a clinically documented extrapituitary acromegaly and a typical carcinoid syndrome toward a hyperinsulinemic hypoglycemia syndrome. At the primary manifestation of the tumor, an increased serum level of insulin-like growth factor I due to overproduction of GHRH and an increased urinary excretion of 5-hydroxyindoleacetic acid were found. The clinical manifestation of the GHRH excess was an arthralgia, which resolved completely after operative tumor debulking and normalization of insulin-like growth factor I and GHRH serum levels. The secretion of serotonin from the tumor resulted in a typical carcinoid syndrome including right-sided valvular heart disease. On the later course of the disease, the humoral manifestations of the tumor were supplemented by the secretion of insulin, leading to recurrent severe hyperinsulinemic hypoglycemia. The hepatic origin of hyperinsulinism was demonstrated by selective arterial calcium stimulation. Moreover, tumor cells revealed insulin and C-peptide immunoreactivity in the immunohistochemical analysis. The patient died 8 yr after the initial diagnosis of the tumor, and a carefully performed autopsy procedure confirmed the absence of any extrahepatic tumor manifestation.

  12. Use of ECMO in the Management of Severe Acute Respiratory Distress Syndrome: A Survey of Academic Medical Centers in the United States.

    PubMed

    Sharma, Nirmal S; Wille, Keith M; Zhi, Degui; Thannickal, Victor J; Brodie, Daniel M; Hoopes, Charles W; Diaz-Guzman, Enrique

    2015-01-01

    Mortality of severe acute respiratory distress syndrome (ARDS) remains high. Once conventional mechanical ventilation fails, alternative modes of therapy are used; most of which have limited evidence to support their use. No definitive guidelines exist for the management of these patients with alternate modalities of treatment. We conducted a cross-sectional national survey of 302 adult critical care training programs in the United States to understand the current preferences of intensivists regarding the use of different therapies for severe ARDS, including the use of extracorporeal membrane oxygenation (ECMO). A total of 381 responses were received: 203 critical care faculty and 174 critical care trainees. Airway pressure release ventilation was the initial choice of treatment reported by most when conventional mechanical ventilation strategy failed followed by inhaled nitric oxide and prone positioning. Extracorporeal membrane oxygenation availability was reported by 80% of the respondents at their institutions. Most respondents (83%) would consider ECMO in patients who fail optimal mechanical ventilation strategies, and the majority (60%) believed that ECMO use can facilitate lung protective ventilation, but few favored its use as a first-line modality. The majority of respondents reported limited knowledge of ECMO and desired specific ECMO education during training. PMID:25914957

  13. 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

  14. 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

  15. Strong correlation between lung ultrasound and chest computerized tomography imaging for the detection of acute lung injury/acute respiratory distress syndrome in rats

    PubMed Central

    Ma, Huan; Huang, Daozheng; Guo, Liheng; Chen, Quanfu; Zhong, Wenzhao

    2016-01-01

    Background Lung ultrasound (LUS) is a clinical imaging technique for diagnosing acute lung injury (ALI) and acute respiratory distress syndrome (ARDS). In humans and several large animals, LUS demonstrates similar specificity and sensitivity to computerized tomography (CT) scanning. Current study evaluated the degree of agreement between LUS and CT imaging in characterizing ALI/ARDS in rats. Methods Thirty male Sprague-Dawley rats were imaged by LUS before randomization into three groups to receive intratracheal saline, 3 or 6 mg/kg LPS respectively (n=10). LUS and CT imaging was conducted 2 hours after instillation. Cross table analyses and kappa statistics were used to determine agreement levels between LUS and CT assessments of lung condition. Results Before instillation, rats presented with a largely A-pattern in LUS images, however, a significantly increase B-lines were observed in all groups after instillation and showed dose response to LPS or to saline. One rat treated with 6 mg/kg lipopolysaccharide (LPS) presented with lung consolidation. The agreement between the LUS and the CT in detecting the main characteristics of ALI/ARDS in rat was strong (r=0.758, P<0.01, k=0.737). Conclusions In conclusion, LUS detects ALI/ARDS with high agreement with micro PET/CT scanning in a rat model, suggesting that LUS represents a positive refinement in rat ALI/ARDS disease models. PMID:27499930

  16. 3,5,4′-Tri-O-acetylresveratrol Attenuates Lipopolysaccharide-Induced Acute Respiratory Distress Syndrome via MAPK/SIRT1 Pathway

    PubMed Central

    Ma, Lijie; Zhao, Yilin; Wang, Ruixuan; Chen, Tingting; Li, Wangping; Nan, Yandong; Liu, Xueying; Jin, Faguang

    2015-01-01

    The aim of the present research was to investigate the protecting effects of 3,5,4′-tri-O-acetylresveratrol (AC-Rsv) on LPS-induced acute respiratory distress syndrome (ARDS). Lung injuries have been evaluated by histological examination, wet-to-dry weight ratios, and cell count and protein content in bronchoalveolar lavage fluid. Inflammation was assessed by MPO activities and cytokine secretion in lungs and cells. The results showed that AC-Rsv significantly reduced the mortality of mice stimulated with LPS. Pretreatment of AC-Rsv attenuated LPS-induced histological changes, alleviated pulmonary edema, reduced blood vascular leakage, and inhibited the MPO activities in lungs. What was more, AC-Rsv and Rsv treatment reduced the secretion of TNF-α, IL-6, and IL-1β in lungs and NR8383 cells, respectively. Further exploration revealed that AC-Rsv and Rsv treatment relieved LPS-induced inhibition on SIRT1 expression and restrained the activation effects of LPS on MAPKs and NF-κB activation both in vitro and in vivo. More importantly, in vivo results have also demonstrated that the protecting effects of Rsv on LPS-induced inflammation would be neutralized when SIRT1 was in-hibited by EX527. Taken together, these results indicated that AC-Rsv protected lung tissue against LPS-induced ARDS by attenuating inflammation via p38 MAPK/SIRT1 pathway. PMID:26648661

  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. Salmonella Typhi-Induced Septic Shock and Acute Respiratory Distress Syndrome in a Previously Healthy Teenage Patient Treated With High-Dose Dexamethasone.

    PubMed

    Ugas, Melissa Brosset; Carroll, Timothy; Kovar, Lacey; Chavez-Bueno, Susana

    2016-01-01

    Typhoid fever is commonly characterized by fever and abdominal pain. Rare complications include intestinal hemorrhage, bowel perforation, delirium, obtundation, and septic shock. Herein we describe the case of a previously healthy 16-year-old male without history of travel, diagnosed with typhoid fever complicated by septic shock and acute respiratory distress syndrome treated with high-dose dexamethasone. This case details severe complications of typhoid fever that are uncommonly seen in developed countries, and the successful response to high-dose dexamethasone as adjunct therapy. High-dose dexamethasone treatment has reportedly decreased Salmonella Typhi mortality, but controlled studies specifically performed in children are lacking, and most reports of its use are over 30 years old and all have originated in developing countries. Providers should include Salmonella Typhi in the differential diagnosis of the pediatric patient with fever, severe abdominal pain, and enteritis, and be aware of its potentially severe complications and the limited data on safety and efficacy of adjunctive therapies that can be considered in addition to antibiotics. PMID:27294165

  19. Sustained lung inflation in the delivery room in preterm infants at high risk of respiratory distress syndrome (SLI STUDY): study protocol for a randomized controlled trial

    PubMed Central

    2013-01-01

    Background Some studies have suggested that the early sustained lung inflation (SLI) procedure is effective in decreasing the need for mechanical ventilation (MV) and improving respiratory outcome in preterm infants. We planned the present randomized controlled trial to confirm or refute these findings. Methods/Design In this study, 276 infants born at 25+0 to 28+6 weeks’ gestation at high risk of respiratory distress syndrome (RDS) will be randomized to receive the SLI maneuver (25 cmH2O for 15 seconds) followed by nasal continuous positive airway pressure (NCPAP) or NCPAP alone in the delivery room. SLI and NCPAP will be delivered using a neonatal mask and a T-piece ventilator. The primary endpoint is the need for MV in the first 72 hours of life. The secondary endpoints include the need and duration of respiratory support (NCPAP, MV and surfactant), and the occurrence of bronchopulmonary dysplasia (BPD). Trial registration Trial registration number: NCT01440868 PMID:23497495

  20. Novel Mutation in the ATP-Binding Cassette Transporter A3 (ABCA3) Encoding Gene Causes Respiratory Distress Syndrome in A Term Newborn in Southwest Iran

    PubMed Central

    Rezaei, Farideh; Shafiei, Mohammad; Shariati, Gholamreza; Dehdashtian, Ali; Mohebbi, Maryam; Galehdari, Hamid

    2016-01-01

    Introduction ABCA3 glycoprotein belongs to the ATP-binding cassette (ABC) superfamily of transporters, which utilize the energy derived from hydrolysis of ATP for the translocation of a wide variety of substrates across the plasma membrane. Mutations in the ABCA3 gene are knowingly causative for fatal surfactant deficiency, particularly respiratory distress syndrome (RDS) in term babies. Case Presentation In this study, Sanger sequencing of the whole ABCA3 gene (NCBI NM_001089) was performed in a neonatal boy with severe RDS. A homozygous mutation has been identified in the patient. Parents were heterozygous for the same missense mutation GGA > AGA at position 202 in exon 6 of the ABCA3 gene (c.604G > A; p.G202R). Furthermore, 70 normal individuals have been analyzed for the mentioned change with negative results. Conclusions Regarding Human Genome Mutation Database (HGMD) and other literature recherche, the detected change is a novel mutation and has not been reported before. Bioinformatics mutation predicting tools prefer it as pathogenic. PMID:27437095

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

    PubMed Central

    Badiee, Zohreh; Naseri, Fatemeh; Sadeghnia, Alireza

    2013-01-01

    Background: This prospective study was performed to identify whether the early use of nasal continuous positive airway pressure (n CPAP) would reduce the rate of endotracheal intubation, mechanical ventilation and surfactant administration. Materials and Methods: This study was conducted from June 2009 to September 2010 in the Shahid Beheshti University Hospital, Isfahan-Iran. A total of 72 preterm infants with 25-30 weeks gestation who needed respiratory support at 5 min after birth entered the study. Infants were randomly assigned to the very early CPAP (initiated 5 min after birth) or to the late CPAP (initiated 30 min after birth) treatment groups. The primary outcomes were need for intubation and mechanical ventilation during the first 48 h after birth and secondary outcomes were death, pneumothorax, intraventricular hemorrhage, duration of mechanical ventilation and bronchopulmonary dysplasia. Results: There were no significant differences between the two groups with regard to mortality rate, bronchopulmonary dysplasia and patent ductus arteriosus. The need for surfactant administration was significantly reduced in the early CPAP group (P = 0.04). Infants in the early CPAP group less frequently required intubation and mechanical ventilation. Conclusions: Early n CPAP is more effective than late n CPAP for the treatment of respiratory distress syndrome. In addition, the early use of n CPAP would reduce the need for some invasive procedures such as intubation and mechanical ventilation. PMID:23930249

  2. Early Prophylactic versus Late Selective Use of Surfactant for Respiratory Distress Syndrome in Very Preterm Infants: A Collaborative Study of 53 Multi-Center Trials in Korea

    PubMed Central

    2014-01-01

    Pulmonary surfactant (PS) therapy was proven to be highly successful for the treatment of respiratory distress syndrome in premature infants. As a results, early prophylactic (EP) PS therapy has been introduced recently in Europe, the US and Korea. However, no multi-center study was compared EP and late selective (LS) PS therapies in Korea. We performed a retrospective multi-center study to compare the outcomes of EP and LS PS therapies in very preterm infants. We analyzed clinical morbidity and mortality for 1,291 infants in 2010 (LS group) and 1,249 infants in 2011 (EP group); the infants were born <30 weeks of gestation and had birth weight ≤1,250 g, and were chosen from 53 neonatal intensive care units in Korea. Compared to the LS group (22.5%), the overall mortality was better in the EP group (19.9%) and there was no increased need for retreatment.There were additional benefits in the EP group such as fewer associated complications. To the best of knowledge, our study is the first nationwide Korean study to compare the outcomes of EP and LS therapies, and it provides evidences that EP PS therapy is important in very preterm infants to improve for survival and reduce morbidities. Graphical Abstract PMID:25120324

  3. Acute respiratory distress syndrome complicating community-acquired pneumonia secondary to mycobacterium tuberculosis in a tertiary care center in Saudi Arabia

    PubMed Central

    Mahmoud, Ebrahim S.; Baharoon, Salim A.; Alsafi, Eiman; Al-Jahdali, Hamdan

    2016-01-01

    Objectives: To discuss our center’s experience with acute respiratory distress syndrome (ARDS) secondary to pulmonary tuberculosis (TB) in a major tertiary referral hospital in the Kingdom of Saudi Arabia. Methods: A retrospective review of medical records of all patients with community-acquired pneumonia secondary to mycobacterium TB infection who were admitted for critical care in a single center of King Abdulaziz Medical City, Riyadh, Kingdom of Saudi Arabia from 2004 to 2013. Results: In our review of 350 patients with community-acquired pneumonia admitted to Intensive Care Unit, 11 cases of TB complicated with ARDS were identified. The mean age of patients was 51.9 years. The median time from hospital admission to pulmonary TB diagnosis and start of therapy was 5 days, while the median time from onset of symptoms to initiation of treatment was 18 days. The mortality rate was 64%, and the median length of hospital stay before death was 21.4 days. Delayed treatment, as well as high acute physiology and chronic health evaluation II and CURB-65 scores at presentation, were independent risk factors for death. Conclusion: Patients with pulmonary TB infrequently present to intensive care with acute symptoms that meet all criteria for ARDS. Such a presentation of TB carries a high mortality risk. PMID:27570853

  4. Pseudo-guillain-barre syndrome due to "whippet"-induced myeloneuropathy.

    PubMed

    Tatum, William O; Bui, Daniel D; Grant, Edmund G; Murtagh, Ryan

    2010-10-01

    Guillain-Barre syndrome (GBS) is the rubric encompassing highly variable phenotypic subgroups of acute, postinfectious, immune-mediated peripheral neuropathy. The hallmark of GBS phenomenology is a rapidly progressive ascending lower extremity weakness. GBS taxonomy includes a motor and sensory axonal neuropathy (AMSAN). Nitrous oxide (NO) abuse may create a pattern of neurological dysfunction almost identical to subacute combined degeneration. We report an adult with myeloneuropathy due to NO abuse that mimicked the presenting features of the GBS-subtype AMSAN. PMID:19490402

  5. Drug-induced hypersensitivity syndrome due to anticonvulsants in a two-year-old boy.

    PubMed

    Criado, Paulo Ricardo; Criado, Roberta F J; Vasconcellos, Cidia; Pegas, Jose Roberto P; Cera, Patrícia Calil

    2004-12-01

    Drug-induced hypersensitivity syndrome (DIHS) usually refers to severe cutaneous drug eruption associated with systemic involvement and potentially fatal outcome. We report a 2-year-old Caucasian boy who developed DIHS due to phenytoin and phenobarbital and who showed extensive internal organ involvement. We are alerting that failure to recognize this drug eruption and discontinue the culprit drug may result in increased severity, greater extent of internal organ involvement, and fatal outcome. The recent research about the influence of human herpesvirus 6 co-infection on the pathogenesis of DIHS is also discussed by the authors in this paper. PMID:15801266

  6. [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.

  7. 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.

  8. Colon perforation with peritonitis in an acquired immunodeficiency syndrome patient due to cytomegalovirus and amoebic colitis.

    PubMed

    Tsai, Hung-Chin; Lee, Susan Shin-Jung; Wann, Shue-Ren; Chen, Yao-Shen; Chen, Eng-Rin; Yen, Chuan-Min; Liu, Yung-Ching

    2005-11-01

    Invasive amoebiasis is rarely seen in human immunodeficiency virus (HIV)-infected individuals, even in endemic areas. By contrast, cytomegalovirus (CMV) disease is recognized as a major clinical problem in acquired immunodeficiency syndrome patients. A 34-year-old HIV-infected man with amoeba colitis, disseminated Mycobacterium avian complex and CMV infection with cecum perforation, presented with the initial symptoms of fever, shortness of breath and painful sensation when swallowing. He was treated with fluconazole, trimethoprim-sulfamethoxazole and hydrocortisone under the impression of esophageal candidiasis and Pneumocystis jiroveci pneumonia. However, diarrhea and abdominal pain developed on day 6 of hospitalization. Invasive amoebiasis and CMV colitis was diagnosed after examination of colon pathological specimens. Emergent laparotomy was performed. Right hemicolectomy with double barrel ileostomy and colostomy was done due to perforation of the cecum. Iodoquinol was given, followed by metronidazole 14 days afterwards. He underwent closure of double barrel ileostomy and colostomy 5 months later. This case illustrates the diagnostic challenge of caring for acquired immunodeficiency syndrome persons with multiple illnesses and medication use. CMV infection, amoebic colitis and possibly corticosteroid may have played a role in colon perforation in our patient.

  9. Refeeding syndrome in a patient with advanced kidney failure due to nephronophthisis.

    PubMed

    El-Reshaid, Kamel

    2013-11-01

    Refeeding syndrome (RS) is a serious and potentially fatal disorder. It is caused by a shift of fluids, sodium, potassium, magnesium and phosphorus as well changes in the metabolism of glucose, protein, fat and vitamins following the refeeding of malnourished patients, whether enterally or parenterally. RS has rarely been reported in patients with advanced kidney disease probably due to the pre-existing hyperphosphatemia, hypermagnesemia and hyperkalemia in these patients. In the following report, we present a patient with nephronophthisis type 1 deletion syndrome in whom her main previous nutrition was limited to simply rehydration to avoid renal replacement therapy. On presentation, she was cachectic and dehydrated with advanced kidney failure. She was treated with medical nephrectomy using non-steroidal anti-inflammatory drugs and then placed on maintenance hemodialysis. Percutaneous endoscopic gastrostomy was used for her initial feeding. Care was exercised during her early refeeding with regard to correction of fluids and essential electrolytes, viz. potassium, phosphorus and magnesium, as well as multivitamins to avoid the cardiovascular and neurological complications of RS. However, the changes in the gut, pancreas and liver as well as her hyperlipidemia were a clear obstacle. Fortunately, the ileus and pancreatitis she developed on refeeding improved dramatically with a decrease of the feeding dose to half; however, the liver abnormalities and hyperlipidemia were severe and slow to recover. These improved after addition of ursodeoxycholic acid and permitted successful increase of the dose of feeding subsequently.

  10. A Stromal Cell–Derived Factor 1α Analogue Improves Endothelial Cell Function in Lipopolysaccharide-Induced Acute Respiratory Distress Syndrome

    PubMed Central

    Guo, Changrun; Goodwin, Andrew; Buie, Joy N Jones; Cook, James; Halushka, Perry; Argraves, Kelley; Zingarelli, Basilia; Zhang, Xian; Wang, Liping; Fan, Hongkuan

    2016-01-01

    Endothelial cell (EC) dysfunction is a critical mediator of the acute respiratory distress syndrome (ARDS). Recent studies have demonstrated that stromal cell–derived factor 1α (SDF-1α) promotes EC barrier integrity. Our previous studies used a SDF-1α analogue CTCE-0214 (CTCE) in experimental sepsis and demonstrated that it attenuated vascular leak and modulated microRNA (miR) levels. We examined the hypothesis that CTCE improves EC function in lipopolysaccharide (LPS)-induced ARDS through increasing miR-126 expression. Human microvascular endothelial cells (HMVECs) were treated with thrombin to disrupt the EC integrity followed by incubation with CTCE or SDF-1α. Barrier function was determined by trans-endothelial electrical resistance assay. CTCE-induced alterations in miRNA expression and signaling pathways involved in barrier function were determined. Thrombin-induced vascular leak was abrogated by both CTCE and SDF-1α. CTCE also prevented thrombin-induced decreases of vascular endothelial (VE)-cadherin cell surface expression and expansion of the intercellular space. CTCE increased miR-126 levels and induced activation of AKT/Rac 1 signaling. Cotreatment with a miR-126 inhibitor blocked the protective effects of CTCE on AKT activation and endothelial permeability. In subsequent in vivo studies, ARDS was induced by intratracheal instillation of LPS. Intravenous injection of CTCE diminished the injury severity as evidenced by significant reductions in protein, immune cells, inflammatory cytokines and chemokines in the bronchoalveolar lavage fluid, increased miR-126 expression and decreased pulmonary vascular leak and alveolar edema. Taken together, our data show that CTCE improves endothelial barrier integrity through increased expression of miR-126 and activation of Rac 1 signaling and represents an important potential therapeutic strategy in ARDS. PMID:27031787

  11. 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

  12. 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

  13. 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. PMID:27069763

  14. Comparison of quantitative computed tomography analysis and single-indicator thermodilution to measure pulmonary edema in patients with acute respiratory distress syndrome

    PubMed Central

    2014-01-01

    Objective To compare quantitative computed tomography (CT) analysis and single-indicator thermodilution to measure pulmonary edema in patients with acute respiratory distress syndrome (ARDS). Method Ten patients with ARDS were included. All underwent spiral CT of the thorax for estimating gas content of lung (GVCT), tissue volume of lung (TVCT), tissue volume index (TVI), mean radiographic attenuation (CTmean) for the whole lung and gas-to-tissue ratio (g/t). Pulmonary thermal volume (PTV) and extravascular lung water index (ELWI) were determined by the PiCCO plus system. CT or single-indicator thermodilution variables were correlated with respiratory system compliance (Crs), PaO2/FiO2, and Acute Physiology And Chronic Health EvaluationII (APACHE II) and Sequential Organ Failure Assessment (SOFA) scores. Results 1) TVCT and PTV were positively correlated (r =0.8878; P = 0.0006; equation of regression line: PTV = 1.0793 × TVCT + 179.8) as were TVI and ELWI (r =0.9459; P < 0.0001; equation of regression line: ELWI = 1.4506 × TVI-8.7792). The bias between TVCT and PTV as well as TVI and ELWI was -277 ± 217 and 0.62 ± 4.56, respectively. 2) ELWI and CT distribution of lung-tissue compartments were not correlated. 3) CT or single-indicator thermodilution variables were not correlated with Crs, PaO2/FiO2 or APACHE II or SOFA score. Conclusion Quantitative CT analysis and single-indicator thermodilution showed good agreement in measuring pulmonary edema. PMID:24625023

  15. Multicentre randomised trial comparing high and low dose surfactant regimens for the treatment of respiratory distress syndrome (the Curosurf 4 trial).

    PubMed Central

    Halliday, H L; Tarnow-Mordi, W O; Corcoran, J D; Patterson, C C

    1993-01-01

    A randomised trial was conducted in 82 centres using the porcine surfactant extract, Curosurf, to compare two regimens of multiple doses to treat infants with respiratory distress syndrome and arterial to alveolar oxygen tension ratio < 0.22. Infants were randomly allocated to a low dosage group (100 mg/kg initially, with two further doses at 12 and 24 hours to a maximum cumulative total of 300 mg/kg; n = 1069) or a high dosage group (200 mg/kg initially with up to four further doses of 100 mg/kg to a maximum cumulative total of 600 mg/kg; n = 1099). There was no difference between those allocated low and high dosage in the rates of death or oxygen dependency at 28 days (51.1% v 50.8%; difference -0.3%, 95% confidence interval (CI) -4.6% to 3.9%), death before discharge (25.0% v 23.5%; difference -1.5%, 95% CI -5.1% to 2.2%), and death or oxygen dependency at the expected date of delivery (32.2% v 31.0%; difference -1.2%, 95% CI -5.2% to 2.7%). For 14 predefined secondary measures of clinical outcome there were no significant differences between the groups but the comparison of duration of supplemental oxygen > 40% did attain significance; 48.4% of babies in the low dose group needed > 40% oxygen after three days compared with 42.6% of those in the high dose group. The total amount of surfactant administered in the low dose regimen (mean 242 mg phospholipid/kg) was probably enough to replace the entire pulmonary surfactant pool. Adopting the low dose regimen would lead to considerable cost savings, with no clinically significant loss in efficacy. PMID:8215564

  16. 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.

  17. Administration of microparticles from blood of the lipopolysaccharide-treated rats serves to induce pathologic changes of acute respiratory distress syndrome

    PubMed Central

    Li, Hongxia; Meng, Xiangyu; Liang, Xiaoyan; Gao, Yue

    2015-01-01

    This study was conducted to investigate the effect of intratracheal and intravenous administration of microparticles (MPs) on developing acute respiratory distress syndrome (ARDS). The blood MPs from lipopolysaccharide-treated rats were collected and examined by transmission electron microscopy (TEM). Cellular source of the MPs was identified by fluorescent-labeled antibodies after the circulating MPs were delivered to naïve rats. Levels of myeloperoxidase (MPO), tumor necrosis factor (TNF)-α, interleukin (IL)-1β, and IL-10 productions in bronchoalveolar lavage fluid (BALF) and plasma were determined 24 h after the rats received intratracheal and intravenous administration of the MPs. Histopathologic examination of lungs was performed by light microscope. A TEM image of MPs showed spherical particles at a variable diameter from 0.1 to 0.5 µm. Endothelial- and leukocyte-derived vesicles were abundant in the investigated samples. Treatment with MPs may lead to significant increases in MPO, TNF-α, IL-1β, and IL-10 productions in BALF and plasma of the rats (all P < 0.001). Morphological observation indicated that alveolar structures were destroyed with a large amount of neutrophil infiltration in the lungs of the MP-treated rats. Perivascular and/or intra-alveolar hemorrhage were serious and hyaline membrane formed in the alveoli. Intratracheal and intravenous approaches to delivery of the circulating MPs to naïve recipient rats may induce ARDS. This presents an inducer of the onset of ARDS and provides potential therapeutic targets for attenuating lung injury. PMID:26088862

  18. A comparison of surfactant administration through i-gel and ET-tube in the treatment of respiratory distress syndrome in newborns weighing more than 2000 grams

    PubMed Central

    Sadeghnia, Alireza; Tanhaei, Mozhgan; Mohammadizadeh, Majid; Nemati, Mohammad

    2014-01-01

    Background: Surfactant administration together with nasal Continuous Positive Airway Pressure (nCPAP) administration is considered to be the basis for Newborn's Respiratory Distress Syndrome (RDS) management. This study evaluated the method of directing the surfactant to the lungs in newborns affiliated with RDS through i-gel (i-gel surfactant administration/i-gelSA) compared to the standard care INSURE method, in a clinical trial. Materials and Methods: This randomized control trial (RCT) was done on newborns weighing ≥2000 g, with RDS, while being supported with Bubble-CPAP. Newborns, which required FiO2 ≥0.3 under Continuous Distending Pressure (CDP) ≥5 cm H2O for more than 30 minutes to maintain SpO2 in the range of 89 - 95%, were given 100 mg/kg of Survanta. In the interventional group or the i-gelSA (i-gel Surfactant Administration) group, 35 newborns experienced surfactant administration with i-gel and 35 newborns in the control or INSURE group. The average a/APO2 before and after surfactant administration, repeated need for surfactant administration, average nCPAP duration, need for invasive mechanical ventilation, pneumothorax, and the average duration of hospitalization in the Neonatal Intensive Care Unit (NICU) were compared. Results: Although the average a/APO2 showed no significant difference before the procedure; in the i-gelSA group, this average was meaningfully higher after the administration of the surfactant (P = 0.001). The other factors showed no significant difference. Conclusion: According to the results of this study, the surfactant administration using i-gel was more successful in oxygenation improvement than the INSURE method, and the i-gel method could even be promoted to the standard care position. However, more research is needed in this area. PMID:25221763

  19. Early Surfactant Therapy With Nasal Continuous Positive Airway Pressure or Continued Mechanical Ventilation in Very Low Birth Weight Neonates With Respiratory Distress Syndrome

    PubMed Central

    Najafian, Bita; Fakhraie, Seyed Hasan; Afjeh, Seyed Abulfazl; Kazemian, Mohammad; Shohrati, Majid; Saburi, Amin

    2014-01-01

    Background: Various strategies have been suggested for the treatment of respiratory distress syndrome (RDS). Objectives: The aim of this study was to compare the efficacies of two common methods of RDS management among neonates with low birth weight. Patients and Methods: A cohort study was conducted on 98 neonates with definite diagnosis of RDS during 2008-2009. The neonates were divided into two groups by a blinded supervisor using simple randomization (odd and even numbers). Forty-five cases in the first group were treated with intubation, surfactant therapy, extubation (INSURE method) followed by nasal continuous positive airway pressure (N.CPAP) and 53 cases in the second group underwent intubation, surfactant therapy followed by mechanical ventilation (MV). Results: Five (11.1%) cases in the first group and 23 (43%) cases in the second group expired during the study. The rates of MV dependency among cases with INSURE failure and cases in the MV group were 37% and 83%, respectively (P < 0.001). Birth weight (BW) (P = 0.017), presence of retinopathy of prematurity (P = 0.022), C/S delivery (P = 0.029) and presence of lung bleeding (P = 0.010) could significantly predict mortality in the second group, although only BW (P = 0.029) had a significant impact on the mortality rate in the first group. Moreover, BW was significantly related to the success rate in the first group (P = 0.001). Conclusions: Our findings demonstrated that INSURE plus NCPAP was more effective than the routine method (permanent intubation after surfactant prescription). In addition, the lower rates of mortality, MV dependency, duration of hospitalization, and complications were observed in cases treated with the INSURE method compared to the routine one. PMID:24910785

  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. 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.

  2. Soluble Forms and Ligands of the Receptor for Advanced Glycation End-Products in Patients with Acute Respiratory Distress Syndrome: An Observational Prospective Study

    PubMed Central

    Jabaudon, Matthieu; Blondonnet, Raiko; Roszyk, Laurence; Pereira, Bruno; Guérin, Renaud; Perbet, Sébastien; Cayot, Sophie; Bouvier, Damien; Blanchon, Loic; Sapin, Vincent; Constantin, Jean-Michel

    2015-01-01

    Background The main soluble form of the receptor for advanced glycation end-products (sRAGE) is elevated during acute respiratory distress syndrome (ARDS). However other RAGE isoforms and multiple ligands have been poorly reported in the clinical setting, and their respective contribution to RAGE activation during ARDS remains unclear. Our goal was therefore to describe main RAGE isoforms and ligands levels during ARDS. Methods 30 ARDS patients and 30 mechanically ventilated controls were prospectively included in this monocenter observational study. Arterial, superior vena cava and alveolar fluid levels of sRAGE, endogenous-secretory RAGE (esRAGE), high mobility group box-1 protein (HMGB1), S100A12 and advanced glycation end-products (AGEs) were measured in duplicate ELISA on day 0, day 3 and day 6. In patients with ARDS, baseline lung morphology was assessed with computed tomography. Results ARDS patients had higher arterial, central venous and alveolar levels of sRAGE, HMGB1 and S100A12, but lower levels of esRAGE and AGEs, than controls. Baseline arterial sRAGE, HMGB1 and S100A12 were correlated with nonfocal ARDS (AUC 0.79, 0.65 and 0.63, respectively). Baseline arterial sRAGE, esRAGE, S100A12 and AGEs were associated with severity as assessed by PaO2/FiO2. Conclusions This is the first kinetics study of levels of RAGE main isoforms and ligands during ARDS. Elevated sRAGE, HMGB1 and S100A12, with decreased esRAGE and AGEs, were found to distinguish patients with ARDS from those without. Our findings should prompt future studies aimed at elucidating RAGE/HMGB1/S100A12 axis involvement in ARDS. Trial Registration clinicaltrials.gov Identifier: NCT01270295. PMID:26274928

  3. 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. PMID:25174313

  4. Efficacy of low tidal volume ventilation in patients with different clinical risk factors for acute lung injury and the acute respiratory distress syndrome.

    PubMed

    Eisner, M D; Thompson, T; Hudson, L D; Luce, J M; Hayden, D; Schoenfeld, D; Matthay, M A

    2001-07-15

    In patients with acute lung injury (ALI) and acute respiratory distress syndrome (ARDS), a recent ARDS Network randomized controlled trial demonstrated that a low tidal volume (VT) mechanical ventilation strategy (6 ml/kg) reduced mortality by 22% compared with traditional mechanical ventilation (12 ml/kg). In this study, we examined the relative efficacy of low VT mechanical ventilation among 902 patients with different clinical risk factors for ALI/ARDS who participated in ARDS Network randomized controlled trials. The clinical risk factor for ALI/ARDS was associated with substantial variation in mortality. The risk of death (before discharge home with unassisted breathing) was highest in patients with sepsis (43%); intermediate in subjects with pneumonia (36%), aspiration (37%), and other risk factors (35%); and lowest in those with trauma (11%) (p < 0.0001). Despite these differences in mortality, there was no evidence that the efficacy of the low VT strategy varied by clinical risk factor (p = 0.76, for interaction between ventilator group and risk factor). There was also no evidence of differential efficacy of low VT ventilation in the other study outcomes: proportion of patients achieving unassisted breathing (p = 0.59), ventilator-free days (p = 0.58), or development of nonpulmonary organ failure (p = 0.44). Controlling for demographic and clinical covariates did not appreciably affect these results. After reclassifying the clinical risk factors as pulmonary versus nonpulmonary predisposing conditions and infection-related versus non-infection-related conditions, there was still no evidence that the efficacy of low VT ventilation differed among clinical risk factor subgroups. In conclusion, we found no evidence that the efficacy of the low VT ventilation strategy differed among clinical risk factor subgroups for ALI/ARDS.

  5. A Pregnant Woman Who Underwent Laparoscopic Adrenalectomy due to Cushing's Syndrome

    PubMed Central

    Bayram, Fahri; Simsek, Yasin; Ozkan, Yusuf; Akcan, Alper; Karahan, Ibrahim; Ileri, Ibrahim; Aribas, Sulbiye; Koc, Mehmet Sait

    2014-01-01

    Cushing's syndrome (CS) may lead to severe maternal and fetal morbidities and even mortalities in pregnancy. However, pregnancy complicates the diagnosis and treatment of CS. This study describes a 26-year-old pregnant woman admitted with hypertension-induced headache. Hormonal analyses performed due to her cushingoid phenotype revealed a diagnosis of adrenocorticotropic hormone- (ACTH-) independent CS. MRI showed a 3.5 cm adenoma in her right adrenal gland. After preoperative metyrapone therapy, she underwent a successful unilateral laparoscopic adrenalectomy at 14-week gestation. Although she had a temporary postoperative adrenal insufficiency, hormonal analyses showed that she has been in remission since delivery. Findings in this patient, as well as those in previous patients, indicate that pregnancy is not an absolute contraindication for laparoscopic adrenalectomy. Rather, such surgery should be considered a safe and efficient treatment method for pregnant women with cortisol-secreting adrenal adenomas. PMID:25544906

  6. 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. PMID:25899869

  7. Response of Smooth Bronchial Musculature in Bronchoconstrictor Substances in Newborn with Lung Atelectasis at the Respiratory Distress Syndrome (RDS)

    PubMed Central

    Mustafa, Lirim; Islami, Pëllumb; Shabani, Nora; Jashanica, Adelina; Islami, Hilmi

    2014-01-01

    ABSTRACT Objective: Role of the atelectasis (hypoxia) in the respiratory system of the live and exited newborn (250 up to 3000 g. of body weight), which has died due to different causes was studied in this work. Methods: Response of tracheal rings to dopamine, serotonine and ethanol in the different molar concentrations (dopamine: 0,05 mg/ml, 0,5 mg/ml, 5 mg/ml; serotonine (5-HT): 10-4, 10-3, 10-2, 10-1 mol/dm3; ethanol: 0,2 ml, 0,5 ml, 1,0 ml; 96%) was followed up. Study of the smooth tracheal musculature tone (STM) was elaborated in 16 tracheal preparations taken following the newborn death due to different causes. Results: Based on functional researches of tracheal isolated preparations, it was ascertained as follows: atelectasis (cases born with lung hypoxia) has changed the response of STM to dopamine, serotonine and ethanol in a significant manner (p<0,01) in comparison to cases of controlling group, which has died due to lung inflammatory processes (e.g. pneumonia, bronchopneumonia, cerebral hemorrhage), which have also caused significant response (p<0,05). Conclusion: Results suggest that exited cases from lung atelectasis and cases of controlling group reacts to above mentioned substances by causing significant constrictor action of tracheobronchial system. PMID:24783902

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

    PubMed Central

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

    2014-01-01

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

  9. 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

  10. Overlap of Juvenile polyposis syndrome and Cowden syndrome due to de novo chromosome 10 deletion involving BMPR1A and PTEN: implications for treatment and surveillance.

    PubMed

    Alimi, Adebisi; Weeth-Feinstein, Lauren A; Stettner, Amy; Caldera, Freddy; Weiss, Jennifer M

    2015-06-01

    We describe a patient with a severe juvenile polyposis phenotype, due to a de novo deletion of chromosome 10q22.3-q24.1. He was initially diagnosed with Juvenile polyposis syndrome (JPS) at age four after presenting with hematochezia due to multiple colonic juvenile polyps. He then re-presented at 23 years with recurrent hematochezia from juvenile polyps in his ileoanal pouch. He is one of the earliest reported cases of JPS associated with a large deletion of chromosome 10. Since his initial diagnosis of JPS further studies have confirmed an association between JPS and mutations in BMPR1A in chromosome band 10q23.2, which is in close proximity to PTEN. Mutations in PTEN cause Cowden syndrome (CS) and other PTEN hamartoma tumor syndromes. Due to the chromosome 10 deletion involving contiguous portions of BMPR1A and PTEN in our patient, he may be at risk for CS associated cancers and features, in addition to the polyps associated with JPS. This case presents new challenges in developing appropriate surveillance algorithms to account for the risks associated with each syndrome and highlights the importance of longitudinal follow-up and transitional care between pediatric and adult gastroenterology for patients with hereditary polyposis syndromes.

  11. Nonthyroidal illness syndrome in patients with subarachnoid hemorrhage due to intracranial aneurysm.

    PubMed

    Casulari, Luiz Augusto; Mangieri, Paola; Naves, Luciana A; Suzuki, Kunio; Ferreira, Moema; Domingues, Lucilia

    2004-03-01

    We have previously reported that subarachnoid hemorrhage due to ruptured intracranial aneurysm (SH) is associated with changes in the hormonal profile in the first 24 hours after the event. We proposed that the hormonal changes observed are due to the intense stress to which the patients are exposed. However, the thyroidal hormonal profile is indicative of the presence of a nonthyroidal illness syndrome (NTIS). In this paper, we examined whether the change in the thyroid hormone profile is compatible with a NTIS. Two groups of patients were included in the study: A) 30 patients with SH (21 females and 9 males; 41.7+/-11.4 years) and B) a control group including 25 patients with benign diseases of the spine (BDS) (lumbar disc hernia or stable spinal trauma) (8 females and 17 males; 41.3+/-14.2 years). In a subgroup of eight patients of each group serum triiodothyronine (T3) and reverse T3 levels were measured. The blood samples were obtained between 8:00 and 9:00 AM. The following results were obtained: The SH group had smaller serum T3 and free T4 levels than the BDS group (p<0.05): T3 (ng/mL): SH = 58.7+/-1.1 and BDS = 74.5+/-13.9; free T4 (ng/dL): SH = 0.9+/-0.2 and BDS = 1.1+/-0.3. There was no significant difference in the serum levels of total thyroxine (T4) and thyroid-stimulating hormone (TSH) between the two groups: T4 ( microg/dL): SH = 6.9+/-1.1 and BDS = 7.4+/-2.1; TSH ( microUI/mL): SH = 1.5+/-0.8 and BDS = 1.8+/-1,0. In the sample of eight patients of each group we had the following results: T3 (ng/mL): SH = 66.8+/-3.8 and BDS = 77.2+/-1.1 (p <0.05); reverse T3 (ng/dL): SH = 32.8+/-8 and BDS = 24.7+/-2.2 (NS); T3/ reverse T3 ratio: SH = 2.6+/-0.3 and BDS = 3.3+/-0.4 (NS). Thyreoglobulin and microsomal antibodies were not detectable, except in one patient in the SH group. In conclusion, the SH patients present serum levels of T3 and free T4 significantly lower than that of BDS patients; the thyroidal hormone profile suggests that SH patients have

  12. Cerebral salt-wasting syndrome due to hemorrhagic brain infarction: a case report

    PubMed Central

    2014-01-01

    Introduction Cerebral salt-wasting syndrome is a condition featuring hyponatremia and dehydration caused by head injury, operation on the brain, subarachnoid hemorrhage, brain tumor and so on. However, there are a few reports of cerebral salt-wasting syndrome caused by cerebral infarction. We describe a patient with cerebral infarction who developed cerebral salt-wasting syndrome in the course of hemorrhagic transformation. Case presentation A 79-year-old Japanese woman with hypertension and arrhythmia was admitted to our hospital for mild consciousness disturbance, conjugate deviation to right, left unilateral spatial neglect and left hemiparesis. Magnetic resonance imaging revealed a broad ischemic change in right middle cerebral arterial territory. She was diagnosed as cardiogenic cerebral embolism because atrial fibrillation was detected on electrocardiogram on admission. She showed hyponatremia accompanied by polyuria complicated at the same time with the development of hemorrhagic transformation on day 14 after admission. Based on her hypovolemic hyponatremia, she was evaluated as not having syndrome of inappropriate secretion of antidiuretic hormone but cerebral salt-wasting syndrome. She fortunately recovered with proper fluid replacement and electrolyte management. Conclusions This is a rare case of cerebral infarction and cerebral salt-wasting syndrome in the course of hemorrhagic transformation. It may be difficult to distinguish cerebral salt-wasting syndrome from syndrome of inappropriate antidiuretic hormone, however, an accurate assessment is needed to reveal the diagnosis of cerebral salt-wasting syndrome because the recommended fluid management is opposite in the two conditions. PMID:25055823

  13. 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

  14. 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

  15. Inhibition of c-Jun N-terminal Kinase Signaling Pathway Alleviates Lipopolysaccharide-induced Acute Respiratory Distress Syndrome in Rats

    PubMed Central

    Lai, Jian-Bo; Qiu, Chun-Fang; Chen, Chuan-Xi; Chen, Min-Ying; Chen, Juan; Guan, Xiang-Dong; Ouyang, Bin

    2016-01-01

    Background: An acute respiratory distress syndrome (ARDS) is still one of the major challenges in critically ill patients. This study aimed to investigate the effect of inhibiting c-Jun N-terminal kinase (JNK) on ARDS in a lipopolysaccharide (LPS)-induced ARDS rat model. Methods: Thirty-six rats were randomized into three groups: control, LPS, and LPS + JNK inhibitor. Rats were sacrificed 8 h after LPS treatment. The lung edema was observed by measuring the wet-to-dry weight (W/D) ratio of the lung. The severity of pulmonary inflammation was observed by measuring myeloperoxidase (MPO) activity of lung tissue. Moreover, the neutrophils in bronchoalveolar lavage fluid (BALF) were counted to observe the airway inflammation. In addition, lung collagen accumulation was quantified by Sircol Collagen Assay. At the same time, the pulmonary histologic examination was performed, and lung injury score was achieved in all three groups. Results: MPO activity in lung tissue was found increased in rats treated with LPS comparing with that in control (1.26 ± 0.15 U in LPS vs. 0.77 ± 0.27 U in control, P < 0.05). Inhibiting JNK attenuated LPS-induced MPO activity upregulation (0.52 ± 0.12 U in LPS + JNK inhibitor vs. 1.26 ± 0.15 U in LPS, P < 0.05). Neutrophils in BALF were also found to be increased with LPS treatment, and inhibiting JNK attenuated LPS-induced neutrophils increase in BALF (255.0 ± 164.4 in LPS vs. 53 (44.5-103) in control vs. 127.0 ± 44.3 in LPS + JNK inhibitor, P < 0.05). At the same time, the lung injury score showed a reduction in LPS + JNK inhibitor group comparing with that in LPS group (13.42 ± 4.82 vs. 7.00 ± 1.83, P = 0.001). However, the lung W/D ratio and the collagen in BALF did not show any differences between LPS and LPS + JNK inhibitor group. Conclusions: Inhibiting JNK alleviated LPS-induced acute lung inflammation and had no effects on pulmonary edema and fibrosis. JNK inhibitor might be a potential therapeutic medication in ARDS, in the

  16. Effect of Noninvasive Ventilation Delivered by Helmet vs Face Mask on the Rate of Endotracheal Intubation in Patients With Acute Respiratory Distress Syndrome

    PubMed Central

    Patel, Bhakti K.; Wolfe, Krysta S.; Pohlman, Anne S.; Hall, Jesse B.; Kress, John P.

    2016-01-01

    IMPORTANCE Noninvasive ventilation (NIV) with a face mask is relatively ineffective at preventing endotracheal intubation in patients with acute respiratory distress syndrome (ARDS). Delivery of NIV with a helmet may be a superior strategy for these patients. OBJECTIVE To determine whether NIV delivered by helmet improves intubation rate among patients with ARDS. DESIGN, SETTING, AND PARTICIPANTS Single-center randomized clinical trial of 83 patients with ARDS requiring NIV delivered by face mask for at least 8 hours while in the medical intensive care unit at the University of Chicago between October 3, 2012, through September 21, 2015. INTERVENTIONS Patients were randomly assigned to continue face mask NIV or switch to a helmet for NIV support for a planned enrollment of 206 patients (103 patients per group). The helmet is a transparent hood that covers the entire head of the patient and has a rubber collar neck seal. Early trial termination resulted in 44 patients randomized to the helmet group and 39 to the face mask group. MAIN OUTCOMES AND MEASURES The primary outcome was the proportion of patients who required endotracheal intubation. Secondary outcomes included 28-day invasive ventilator–free days (ie, days alive without mechanical ventilation), duration of ICU and hospital length of stay, and hospital and 90-day mortality. RESULTS Eighty-three patients (45% women; median age, 59 years; median Acute Physiology and Chronic Health Evaluation [APACHE] II score, 26) were included in the analysis after the trial was stopped early based on predefined criteria for efficacy. The intubation rate was 61.5% (n = 24) for the face mask group and 18.2% (n = 8) for the helmet group (absolute difference, −43.3%; 95% CI, −62.4%to −24.3%; P < .001). The number of ventilator-free days was significantly higher in the helmet group (28 vs 12.5, P < .001). At 90 days, 15 patients (34.1%) in the helmet group died compared with 22 patients (56.4%) in the face mask group

  17. 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

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

    PubMed

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

    2016-04-01

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

  19. Drug-induced hypersensitivity syndrome due to minocycline complicated by severe myocarditis.

    PubMed

    Kanno, Kyoko; Sakai, Hiroyuki; Yamada, Yutaka; Iizuka, Hajime

    2014-02-01

    A 60-year-old woman presented with a 13-day history of a generalized erythematous rash accompanied by fever, periorbital edema and axillary lymphadenopathy. Prior to the appearance of the rash, the patient had been treated with intermittent courses of oral minocycline for cystitis. The patient was diagnosed with drug-induced hypersensitivity syndrome (DIHS) due to minocycline. During the admission, infectious endocarditis was suspected and the patient was treated with i.v. gammaglobulin (0.4 g/kg per day). The following day, the patient suffered from systemic deterioration and symptomatic low blood pressure that prompted repeat echocardiography which revealed an ejection fraction of 10%. DIHS-associated myocarditis was suspected and management with circulation assistance devices and steroid pulse therapy were started, resulting in satisfactory resolution. A rise in titer of human herpesvirus-6, cytomegalovirus and Herpes simplex virus-1 antibodies was detected. Although minocycline-induced myocarditis is rare, this severe drug reaction should be considered with DIHS.

  20. Iatrogenic Cushing's Syndrome Due to Intranasal Usage of Ophthalmic Dexamethasone: A Case Report.

    PubMed

    Orton, Sarah; Censani, Marisa

    2016-05-01

    Iatrogenic Cushing's syndrome (ICS) is caused by exogenous corticosteroid administration with suppression of the hypothalamic-pituitary-adrenal axis. It has been commonly described with oral and topical steroid use, but scarce reports have documented intranasal steroid usage as the etiology in infancy. In this article, we describe a case of a 4-month-old infant who developed ICS after 6 weeks of intranasal dexamethasone ophthalmic solution administration for nasal obstruction. To our knowledge, this is the youngest patient reported with ICS due to intranasal use of a prescribed dose of an ophthalmic steroid. His hypothalamic-pituitary-adrenal axis recovered fully 4.5 months after steroid discontinuation. Because of the small body surface area and supine position during administration, infants are particularly susceptible to ICS. Given that intranasal steroids are commonly prescribed to infants and children for a variety of diagnoses, this case highlights the risks inherent in the use of intranasal steroid drops, particularly in young infants, for both adrenal suppression and linear growth deceleration, even with short-term use. Close monitoring of these patients' height and weight should occur while on steroid treatment, with every effort made to decrease or discontinue steroid use when possible. PMID:27244810

  1. 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.

  2. 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

    2016-01-01

    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. PMID:27659091

  3. Autoimmune manifestations in SCID due to IL7R mutations: Omenn syndrome and cytopenias.

    PubMed

    Zago, Claudia Augusta; Jacob, Cristina Miuki Abe; de Albuquerque Diniz, Edna Maria; Lovisolo, Silvana Maria; Zerbini, Maria Claudia Nogueira; Dorna, Mayra; Watanabe, Letícia; Fernandes, Juliana Folloni; Rocha, Vanderson; Oliveira, João Bosco; Carneiro-Sampaio, Magda

    2014-07-01

    B+NK+SCID (severe combined immunodeficiency) due to IL7Rα deficiency represents approximately 10% of American SCID cases. To better understand the spectrum of autoimmune disorders associated with IL7Rα deficiency, we describe two unrelated IL7Rα-deficient female SCID infants whose clinical picture was dominated by autoimmune manifestations: one with intrauterine Omenn syndrome (OS) and another with persistent thrombocytopenic purpura since 4months of age. The OS baby harbored a homozygous p.C118Y mutation in IL7R. She presented dense eosinophilic infiltrates in several organs, including pancarditis, which may have contributed to her death (on the 2nd day of life). B cells were observed in lymph nodes, spleen, bone marrow and thymus. The second patient harbored compound heterozygous p.C118Y and p.I121NfsX8 mutations. She underwent a successful unrelated cord blood transplant. In conclusion, early OS can be observed in patients with IL7R mutations, and autoimmune cytopenias could also complicate the clinical course of SCID babies with this type of defect.

  4. 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

    2016-01-01

    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.

  5. [A case of occupational contact urticaria and oral allergy syndrome due to seafood].

    PubMed

    Yamaguchi, Junko; Inomata, Naoko; Hirokado, Michiko; Shimakura, Kuniyoshi; Shiomi, Kazuo; Ikezawa, Zenro

    2007-01-01

    A 20-year-old woman was referred for evaluation after about 2 years of recurrent episodes of localized urticaria during handling of several kinds of raw fish in a sushi shop, where she had worked part-time for 2 years. She had also experienced allergic symptoms such as itching and swelling of her lips, generalized urticaria, laryngeal tightness, stridor and dyspnea immediately after ingestion of raw and cooked seafood, including sole, horse mackerel, sea eel and shellfish, over the previous 1 year before referral. Skin prick tests and blood test for specific IgE antibodies were positive for many kinds of seafood, including sole, horse mackerel, sea eel, eel, crab, and abalone, which belonged to different taxonomic phyla, including Chordata, Arthropoda, and Mollusca. A challenge with a piece of broiled sole induced swelling of the lips, obstruction of the larynx, difficulty with deglutition, and abdominal pain. In addition, serum-specific IgE antibodies to two major fish allergens, parvalbumin and collagen, were detected by ELISA, suggesting that allergic symptoms could be induced by many kinds of seafood in the present patient. She was therefore diagnosed with occupational contact urticaria and oral allergy syndrome due to seafood. At the time of this report, the present patient had been followed for one year and no reactions have occurred since she started to avoid the causative types of seafood.

  6. [Microphthalmos in Pierre Robin syndrome. Clinical and x-ray computed tomographic study].

    PubMed

    Girard, B; Topouzis, F; Saraux, H

    1989-12-01

    The Pierre Robin Syndrome is characterized by three defects (8,9): micrognathia, cleft palate and glossoptosis responsible for respiratory failure. The new definition of this syndrome associates retrognathia, cleft palate and respiratory distress. This respiratory distress is mixed: obstructive due to glossoptosis, and central, secondary to brainstem immaturity (1,2). The main ocular manifestations associated with the syndrome are congenital glaucoma, high congenital myopia and retinal detachment. Microphtalmia has already been reported, but is infrequent. We present a clinical case of a major microphthalmia in a Pierre Robin Syndrome, confirmed by CT scan exploration.

  7. Nephrotic syndrome due to immunoglobulin M mesangial glomerulonephritis preceding juvenile idiopathic arthritis.

    PubMed

    Voyer, Luis E; Alvarado, Caupolican; Cuttica, Rubén J; Balestracci, Alejandro; Zardini, Marta; Lago, Néstor

    2013-05-21

    The association between nephrotic syndrome and juvenile idiopathic arthritis have rarely been described in pediatric patients. We report a child with steroid-responsive nephrotic syndrome, with frequent relapses, who presented with a new relapse of nephrotic syndrome associated with arthritis and uveitis at 21 months in remission after treatment with chlorambucil. Juvenile idiopathic arthritis was diagnosed and kidney biopsy examination showed mesangial glomerulonephritis with immunoglobulin M deposits. To our knowledge, only 2 cases of nephrotic syndrome preceding juvenile idiopathic arthritis have been reported, one without histopathology assessment and the other with minimal change disease. Although mesangial glomerulonephritis with nephrotic syndrome and juvenile idiopathic arthritis could have been coincidental, the immune pathogenic mechanism accepted for both diseases suggests they could be related.

  8. [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.

  9. Nephrotic Syndrome without Hematuria due to Infection-Related Glomerulonephritis Mimicking Minimal-Change Disease in a Child.

    PubMed

    Iwafuchi, Yoichi; Morioka, Tetsuo; Morita, Takashi; Watanabe, Kanako; Oyama, Yuko; Narita, Ichiei

    2016-01-01

    Nephrotic syndrome without hematuria due to infection-related glomerulonephritis is uncommon. The present report describes a case of nephrotic syndrome due to infection-related glomerulonephritis without hematuria and hypertension in an older child. A 14-year-old boy was referred to our hospital because of a 5-day history of fever, nausea, weight gain and recent leg edema without hypertension. Laboratory data showed nephrotic-range proteinuria, hypoalbuminemia, mild hypocomplementemia and acute renal injury without hematuria. Although, due to the clinical presentation, minimal-change nephrotic syndrome was mostly suspected, a renal biopsy showed endocapillary hypercellularity mainly of mononuclear cells with segmental mesangiolytic changes. Fine granular IgG and C3 deposits were noted by an immunofluorescent study; many relatively small electron-dense deposits were observed electron-microscopically. These findings led to the diagnosis of nephrotic syndrome due to infection-related endocapillary proliferative glomerulonephritis, although the causative organism of his nephritis was not detected. He recovered with rest and dietary cure. When we examine an acute nephrotic child, infection-related glomerulonephritis should be considered as the differential diagnosis to avoid unnecessary use of corticosteroids.

  10. Nephrotic Syndrome without Hematuria due to Infection-Related Glomerulonephritis Mimicking Minimal-Change Disease in a Child

    PubMed Central

    Iwafuchi, Yoichi; Morioka, Tetsuo; Morita, Takashi; Watanabe, Kanako; Oyama, Yuko; Narita, Ichiei

    2016-01-01

    Nephrotic syndrome without hematuria due to infection-related glomerulonephritis is uncommon. The present report describes a case of nephrotic syndrome due to infection-related glomerulonephritis without hematuria and hypertension in an older child. A 14-year-old boy was referred to our hospital because of a 5-day history of fever, nausea, weight gain and recent leg edema without hypertension. Laboratory data showed nephrotic-range proteinuria, hypoalbuminemia, mild hypocomplementemia and acute renal injury without hematuria. Although, due to the clinical presentation, minimal-change nephrotic syndrome was mostly suspected, a renal biopsy showed endocapillary hypercellularity mainly of mononuclear cells with segmental mesangiolytic changes. Fine granular IgG and C3 deposits were noted by an immunofluorescent study; many relatively small electron-dense deposits were observed electron-microscopically. These findings led to the diagnosis of nephrotic syndrome due to infection-related endocapillary proliferative glomerulonephritis, although the causative organism of his nephritis was not detected. He recovered with rest and dietary cure. When we examine an acute nephrotic child, infection-related glomerulonephritis should be considered as the differential diagnosis to avoid unnecessary use of corticosteroids. PMID:26889476

  11. Nephrotic Syndrome without Hematuria due to Infection-Related Glomerulonephritis Mimicking Minimal-Change Disease in a Child.

    PubMed

    Iwafuchi, Yoichi; Morioka, Tetsuo; Morita, Takashi; Watanabe, Kanako; Oyama, Yuko; Narita, Ichiei

    2016-01-01

    Nephrotic syndrome without hematuria due to infection-related glomerulonephritis is uncommon. The present report describes a case of nephrotic syndrome due to infection-related glomerulonephritis without hematuria and hypertension in an older child. A 14-year-old boy was referred to our hospital because of a 5-day history of fever, nausea, weight gain and recent leg edema without hypertension. Laboratory data showed nephrotic-range proteinuria, hypoalbuminemia, mild hypocomplementemia and acute renal injury without hematuria. Although, due to the clinical presentation, minimal-change nephrotic syndrome was mostly suspected, a renal biopsy showed endocapillary hypercellularity mainly of mononuclear cells with segmental mesangiolytic changes. Fine granular IgG and C3 deposits were noted by an immunofluorescent study; many relatively small electron-dense deposits were observed electron-microscopically. These findings led to the diagnosis of nephrotic syndrome due to infection-related endocapillary proliferative glomerulonephritis, although the causative organism of his nephritis was not detected. He recovered with rest and dietary cure. When we examine an acute nephrotic child, infection-related glomerulonephritis should be considered as the differential diagnosis to avoid unnecessary use of corticosteroids. PMID:26889476

  12. Fatal Case of Toxic Shock-Like Syndrome Due to Group C Streptococcus Associated with Superantigen Exotoxin

    PubMed Central

    Korman, Tony M.; Boers, Anthony; Gooding, Travis M.; Curtis, Nigel; Visvanathan, Kumar

    2004-01-01

    Group C streptococci have been reported to cause invasive disease similar to that classically associated with group A streptococcus (GAS). We describe a fatal case of toxic shock-like syndrome due to Streptococcus equi subsp. zooepidemicus. The causative organism did not possess any known GAS superantigen exotoxin genes but did show evidence of superantigen production. PMID:15184494

  13. Anesthetic management of superior vena cava syndrome due to anterior mediastinal mass

    PubMed Central

    Chaudhary, Kapil; Gupta, Anshu; Wadhawan, Sonia; Jain, Divya; Bhadoria, Poonam

    2012-01-01

    Anesthetic management of superior vena cava syndrome carries a possible risk of life-threatening complications such as cardiovascular collapse and complete airway obstruction during anesthesia. Superior vena cava syndrome results from the enlargement of a mediastinal mass and consequent compression of mediastinal structures resulting in impaired blood flow from superior vena cava to the right atrium and venous congestion of face and upper extremity. We report the successful anesthetic management of a 42-year-old man with superior vena cava syndrome posted for cervical lymph node biopsy. PMID:22557753

  14. [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.

  15. A surge of flu-associated adult respiratory distress syndrome in an Austrian tertiary care hospital during the 2009/2010 Influenza A H1N1v pandemic.

    PubMed

    Schellongowski, Peter; Ullrich, Roman; Hieber, Cornelia; Hetz, Hubert; Losert, Heidrun; Hermann, Maria; Hermann, Alexander; Gattringer, Klaus-Bernhard; Siersch, Viktoria; Rabitsch, Werner; Fuhrmann, Valentin; Bojic, Andja; Robak, Oliver; Sperr, Wolfgang R; Laczika, Klaus; Locker, Gottfried J; Staudinger, Thomas

    2011-04-01

    We report on 17 patients with influenza A H1N1v-associated Adult Respiratory Distress Syndrome who were admitted to the intensive care unit (ICU) between June 11th 2009 and August 10th 2010 (f/m: 8/9; age: median 39 (IQR 29-54) years; SAPS II: 35 (29-48)). Body mass index was 26 (24-35), 24% were overweight and 29% obese. The Charlson Comorbidity Index was 1 (0-2) and all but one patient had comorbid conditions. The median time between onset of the first symptom and admission to the ICU was 5 days (range 0-14). None of the patients had received vaccination against H1N1v. Nine patients received oseltamivir, only two of them within 48 hours of symptom onset. All patients developed severe ARDS (PaO(2)/FiO(2)-Ratio 60 (55-92); lung injury score 3.8 (3.3-4.0)), were mechanically ventilated and on vasopressor support. Fourteen patients received corticosteroids, 7 patients underwent hemofiltration, and 10 patients needed extracorporeal membrane-oxygenation (ECMO; 8 patients veno-venous, 2 patients veno-arterial), three patients Interventional Lung Assist (ILA) and two patients pump driven extracorporeal low-flow CO(2)-elimination (ECCO(2)-R). Seven of 17 patients (41%) died in the ICU (4 patients due to bleeding, 3 patients due to multi-organ failure), while all other patients survived the hospital (59%). ECMO mortality was 50%. The median ICU length-of-stay was 26 (19-44) vs. 21 (17-25) days (survivors vs. nonsurvivors), days on the ventilator were 18 (14-35) vs. 20 (17-24), and ECMO duration was 10 (8-25) vs. 13 (11-16) days, respectively (all p = n.s.). Compared to a control group of 241 adult intensive care unit patients without H1N1v, length of stay in the ICU, rate of mechanical ventilation, days on the ventilator, and TISS 28 scores were significantly higher in patients with H1N1v. The ICU survival tended to be higher in control patients (79 vs. 59%; p = 0.06). Patients with H1N1v admitted to either of our ICUs were young, overproportionally obese and almost all

  16. Perry syndrome due to the DCTN1 G71R mutation: a distinctive levodopa responsive disorder with behavioral syndrome, vertical gaze palsy, and respiratory failure.

    PubMed

    Newsway, Victoria; Fish, Mark; Rohrer, Jonathan D; Majounie, Elisa; Williams, Nigel; Hack, Melissa; Warren, Jason D; Morris, Huw R

    2010-04-30

    Perry syndrome is a rare form of autosomal dominant Parkinsonism with respiratory failure recently defined as being due to mutations in the DCTN1 gene. We describe a new family carrying a G71R mutation in the DCTN1 gene. The proband displayed a series of distinctive features not previously described in Perry syndrome: a disorder of vertical downward saccades accompanied by progressive midbrain atrophy, predominant nonmotor symptoms responsive to levodopa, distinctive craniocervical levodopa induced dyskinesias, and a good response to high-dose levodopa therapy and respiratory support. The family was initially thought to have autosomal dominant behavioral variant frontotemporal dementia with Parkinsonism. This report expands the clinical definition of this distinctive syndrome.

  17. Organ failures due to low cardiac output syndrome following open heart surgery.

    PubMed

    Kumon, K; Tanaka, K; Hirata, T; Naito, Y; Fujita, T

    1986-04-01

    During the period from August, 1977 to December, 1984, a total of 3003 patients who received open heart surgery were treated postoperatively at the ICU of National Cardiovascular Center. Low cardiac output syndrome (LOS) developed in 669 (22.3%) patients. Organ failures due to LOS were studied in these patients. Although the overall mortality of postoperative patients was 5.6% and improved to around 4% in the later years, death rate of patients with LOS was persistently high (22.8%) and showed no tendency to improve even in the latest years. Moreover, the clinical results of those LOS patients who developed organ failure were extremely poor; the mortality of patients with respiratory failure (RF) accounted for 36.8% and that of patients with other organ failure exceeded 50%. The incidence of impaired organs in LOS patients was 49.9% in RF, 29.9% in acute renal failure (ARF), 18.4% in hepatic failure (HF), 16.4% in disseminated intravascular coagulation (DIC), 15.5% in central nervous system failure (CNSF), and 11.1% in gastrointestinal bleeding (GIB). Pathophysiological mechanisms as well as the management of these major complications caused by LOS are also discussed. Some patients developed multiple organ failure (MOF). Plasma exchange (PE) was performed on 16 patients who developed MOF. Improvement of various organ functions was obtained and consequently three patients were successfully treated by means of PE. Removal of various substances toxic to organs, supplement of deficient substances and cessation of the vicious cycle produced by the interaction of impaired organs in patients with MOF are major roles of PE in the treatment of MOF.

  18. 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.

  19. 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

  20. 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. PMID:26373698

  1. Delayed diagnosis in patients with Prader-Willi syndrome due to maternal uniparental disomy 15.

    PubMed

    Gunay-Aygun, M; Heeger, S; Schwartz, S; Cassidy, S B

    1997-07-11

    Prader-Willi syndrome (PWS) results from absence of the normally active paternally inherited genes on proximal 15q, due to del(15)(q11q13) or by maternal uniparental disomy (UPD) 15 in most cases. In addition to a higher frequency of hypopigmentation among deletion patients, minor phenotypic differences between deletion and UPD patients have recently been reported, including lower birth weight in the deletion group, shorter birth length in males with UPD, and shorter course of gavage feeding and later onset of hyperphagia in females with UPD. We previously reported that those with UPD had a less "typical" facial appearance, and they less often had skin picking, skill with puzzles, and high pain threshold. There were no children younger than 3.5 years of age in the UPD group, in contrast to several of them in the deletion group, suggesting a possible diagnostic delay in the UPD group. To assess this possibility and seek reasons for it, we reviewed the charts of 60 PWS patients with complete molecular testing. Mean age at diagnosis of patients with UPD was significantly higher than in the deletion group. Mean percentiles of birth weights and lengths of patients with UPD were significantly lower than in those with deletion. Mean duration of gestation, mean duration of gavage feeding, and mean age at onset of hyperphagia did not differ significantly between groups. Delay in the diagnosis of patients with UPD, which may influence the management and impact of the disorder, might be explained by a lower frequency of typical facial anomalies in this group. PMID:9215778

  2. A Rare Combination: Congenital Adrenal Hyperplasia Due To 21 Hydroxylase Deficiency and Turner Syndrome

    PubMed Central

    Peltek Kendirci, Havva Nur; Aycan, Zehra; Çetinkaya, Semra; Baş, Veysel Nijat; Ağladıoğlu, Sebahat Yılmaz; Önder, Aşan

    2012-01-01

    A combination of Turner syndrome (TS) and classical congenital adrenal hyperplasia (CAH) is rare. A one-day-old newborn was referred to our hospital with ambiguous genitalia. The parents were third-degree relatives. The infant’s weight was 3350g (50-75p), and the head circumference was 34.5cm (50p). The gonads were nonpalpable. Presence of a 3 cm phallus, one urogenital opening into the perineum, and incomplete labial fusion were identified. Laboratory tests revealed a classical type of CAH due to 21-hydroxylase deficiency. Karyotyping revealed a 45X0(35)/46XX(22) pattern with negative sex-determining region Y (SRY) on gene analysis. At the most recent follow-up visit, the patient appeared to be in good health - her height was 70.4 cm [-1.5 standard deviation (SD)] and her weight was 9.8 kg (0.3 SD). She was receiving hydrocortisone in a dose of 10 mg/m2/day, fludrocortisone acetate in a dose of 0.075 mg/day, and oral salt of 1 g/day. System examinations were normal. The patient’s electrolyte levels were found to be normal and she was in good metabolic control. The findings of this patient demonstrate that routine karyotyping during investigation of patients with sexual differentiation disorders can reveal TS. Additionally, signs of virilism should always be investigated at diagnosis or during physical examinations for follow-up of TS cases. [i][/i]SRY analysis should be performed primarily when signs of virilism are observed. CAH should also be considered in patients with negative [i]SRY[/i]. Conflict of interest:None declared. PMID:23261864

  3. Two rare conditions in an Eisenmenger patient: left main coronary artery compression and Ortner's syndrome due to pulmonary artery dilatation.

    PubMed

    Andjelkovic, Kristina; Kalimanovska-Ostric, Dimitra; Djukic, Milan; Vukcevic, Vladan; Menkovic, Nemanja; Mehmedbegovic, Zlatko; Topalovic, Mirko; Tesic, Milorad

    2013-01-01

    The left-main coronary artery extrinsic compression due to enlarged pulmonary artery has been described in several case series. Ortner's syndrome is also a rare condition in some cardiovascular disorders. There have been no reports about these two rare conditions in the same patient. Hence, we report a very rare case of an Eisenmenger patient with severe pulmonary hypertension and dilated pulmonary artery which has compressed the left main coronary artery, severely narrowing it, and the left laryngeal recurrent nerve with subsequent Ortner's syndrome and brief literature review. PMID:23831302

  4. Lethal Progressive Thoracic Insufficiency in a Neonate Due to Jarcho Levin Syndrome

    PubMed Central

    Bhutia, Euden; Maria, Arti; Verma, Arushi; Sethi, Sidharth Kumar

    2014-01-01

    A rare case of Jarcho Levin syndrome (JLS) presenting as a lethal progressive respiratory insufficiency in early neonatal period is reported. The neonate had classical features of this syndrome including vertebral segmentation defects, typical costo-vertebral fusion defects and scoliosis resulting in small thoracic volume and limited chest expansion; all consistent with a clinical diagnosis of JLS with thoracic insufficiency. In addition, our case had a rare association of dextrocardia and acyanotic congenital heart disease. PMID:24741543

  5. Lethal progressive thoracic insufficiency in a neonate due to jarcho levin syndrome.

    PubMed

    Bhutia, Euden; Maria, Arti; Verma, Arushi; Sethi, Sidharth Kumar

    2014-01-01

    A rare case of Jarcho Levin syndrome (JLS) presenting as a lethal progressive respiratory insufficiency in early neonatal period is reported. The neonate had classical features of this syndrome including vertebral segmentation defects, typical costo-vertebral fusion defects and scoliosis resulting in small thoracic volume and limited chest expansion; all consistent with a clinical diagnosis of JLS with thoracic insufficiency. In addition, our case had a rare association of dextrocardia and acyanotic congenital heart disease.

  6. Fraternal twins with Aarskog-Scott syndrome due to maternal germline mosaicism.

    PubMed

    Pilozzi-Edmonds, Laura; Maher, Thomas A; Basran, Raveen K; Milunsky, Aubrey; Al-Thihli, Khalid; Braverman, Nancy E; Alfares, Ahmed

    2011-08-01

    Aarskog-Scott syndrome is a rare X-linked recessive disorder with characteristic facial, skeletal, and genital abnormalities. We report on Aarskog-Scott syndrome in male dizygotic twins with an identical de novo mutation in FGD1 that resulted from germline mosaicism in the phenotypically normal mother. This is the first report of inheritance by germline mosaicism for the FGD1 gene. PMID:21739585

  7. Case of inappropriate ADH syndrome: hyponatremia due to polyethylene glycol bowel preparation.

    PubMed

    Ko, Sun-Hye; Lim, Chul-Hyun; Kim, Jae-Young; Kang, Seung Hun; Baeg, Myong Ki; Oh, Hyun Jin

    2014-09-14

    Colonoscopic screening has been reported to reduce deaths from colorectal cancer. Adequate bowel preparation is essential for this and safety is an important issue in choosing the methods. Polyethylene glycol (PEG) is regarded as a safe method for cleansing, especially compared with oral sodium phosphate. Here, we present a case of hyponatremia caused by the syndrome of inappropriate antidiuretic hormone (ADH) syndrome after PEG precolonoscopic cleansing resulting in generalized tonic-clonic seizures. A 62-year-old women had ingested PEG for precolonoscopic bowel cleansing. While waiting for the colonoscopy, she developed a stuporous mentality and generalized tonic-clonic seizures, which did not correlate with brain magnetic resonance imaging. Her serum sodium level was 113 mEq per liter and laboratory analyses were consistent with inappropriate ADH syndrome. Her thyroid and adrenal functions were normal. There were no malignancies, infections, respiratory disorders or central nervous disorders and she had no history of taking either diuretics or other medications, which might have caused inappropriate ADH syndrome. She was treated with 3% hypertonic saline and showed a complete neurological recovery as her sodium levels recovered. Follow-up visits showed the patient to have a normal sodium level without neurologic deficits. This case shows that inappropriate ADH syndrome can be caused by PEG preparation, which implies that physicians have to be aware of the possible side effects of this colonic cleansing approach and mindful of the possible ensuing symptoms. PMID:25232272

  8. Case of inappropriate ADH syndrome: Hyponatremia due to polyethylene glycol bowel preparation

    PubMed Central

    Ko, Sun-Hye; Lim, Chul-Hyun; Kim, Jae-Young; Kang, Seung Hun; Baeg, Myong Ki; Oh, Hyun Jin

    2014-01-01

    Colonoscopic screening has been reported to reduce deaths from colorectal cancer. Adequate bowel preparation is essential for this and safety is an important issue in choosing the methods. Polyethylene glycol (PEG) is regarded as a safe method for cleansing, especially compared with oral sodium phosphate. Here, we present a case of hyponatremia caused by the syndrome of inappropriate antidiuretic hormone (ADH) syndrome after PEG precolonoscopic cleansing resulting in generalized tonic-clonic seizures. A 62-year-old women had ingested PEG for precolonoscopic bowel cleansing. While waiting for the colonoscopy, she developed a stuporous mentality and generalized tonic-clonic seizures, which did not correlate with brain magnetic resonance imaging. Her serum sodium level was 113 mEq per liter and laboratory analyses were consistent with inappropriate ADH syndrome. Her thyroid and adrenal functions were normal. There were no malignancies, infections, respiratory disorders or central nervous disorders and she had no history of taking either diuretics or other medications, which might have caused inappropriate ADH syndrome. She was treated with 3% hypertonic saline and showed a complete neurological recovery as her sodium levels recovered. Follow-up visits showed the patient to have a normal sodium level without neurologic deficits. This case shows that inappropriate ADH syndrome can be caused by PEG preparation, which implies that physicians have to be aware of the possible side effects of this colonic cleansing approach and mindful of the possible ensuing symptoms. PMID:25232272

  9. Case of inappropriate ADH syndrome: hyponatremia due to polyethylene glycol bowel preparation.

    PubMed

    Ko, Sun-Hye; Lim, Chul-Hyun; Kim, Jae-Young; Kang, Seung Hun; Baeg, Myong Ki; Oh, Hyun Jin

    2014-09-14

    Colonoscopic screening has been reported to reduce deaths from colorectal cancer. Adequate bowel preparation is essential for this and safety is an important issue in choosing the methods. Polyethylene glycol (PEG) is regarded as a safe method for cleansing, especially compared with oral sodium phosphate. Here, we present a case of hyponatremia caused by the syndrome of inappropriate antidiuretic hormone (ADH) syndrome after PEG precolonoscopic cleansing resulting in generalized tonic-clonic seizures. A 62-year-old women had ingested PEG for precolonoscopic bowel cleansing. While waiting for the colonoscopy, she developed a stuporous mentality and generalized tonic-clonic seizures, which did not correlate with brain magnetic resonance imaging. Her serum sodium level was 113 mEq per liter and laboratory analyses were consistent with inappropriate ADH syndrome. Her thyroid and adrenal functions were normal. There were no malignancies, infections, respiratory disorders or central nervous disorders and she had no history of taking either diuretics or other medications, which might have caused inappropriate ADH syndrome. She was treated with 3% hypertonic saline and showed a complete neurological recovery as her sodium levels recovered. Follow-up visits showed the patient to have a normal sodium level without neurologic deficits. This case shows that inappropriate ADH syndrome can be caused by PEG preparation, which implies that physicians have to be aware of the possible side effects of this colonic cleansing approach and mindful of the possible ensuing symptoms.

  10. Systemic inflammatory response syndrome in adult patients with nosocomial bloodstream infections due to enterococci

    PubMed Central

    Bar, Katharine; Wisplinghoff, Hilmar; Wenzel, Richard P; Bearman, Gonzalo ML; Edmond, Michael B

    2006-01-01

    Background Enterococci are the third leading cause of nosocomial bloodstream infection (BSI). Vancomycin resistant enterococci are common and provide treatment challenges; however questions remain about VRE's pathogenicity and its direct clinical impact. This study analyzed the inflammatory response of Enterococcal BSI, contrasting infections from vancomycin-resistant and vancomycin-susceptible isolates. Methods We performed a historical cohort study on 50 adults with enterococcal BSI to evaluate the associated systemic inflammatory response syndrome (SIRS) and mortality. We examined SIRS scores 2 days prior through 14 days after the first positive blood culture. Vancomycin resistant (n = 17) and susceptible infections (n = 33) were compared. Variables significant in univariate analysis were entered into a logistic regression model to determine the affect on mortality. Results 60% of BSI were caused by E. faecalis and 34% by E. faecium. 34% of the isolates were vancomycin resistant. Mean APACHE II (A2) score on the day of BSI was 16. Appropriate antimicrobials were begun within 24 hours in 52%. Septic shock occurred in 62% and severe sepsis in an additional 18%. Incidence of organ failure was as follows: respiratory 42%, renal 48%, hematologic 44%, hepatic 26%. Crude mortality was 48%. Progression to septic shock was associated with death (OR 14.9, p < .001). There was no difference in A2 scores on days -2, -1 and 0 between the VRE and VSE groups. Maximal SIR (severe sepsis, septic shock or death) was seen on day 2 for VSE BSI vs. day 8 for VRE. No significant difference was noted in the incidence of organ failure, 7-day or overall mortality between the two groups. Univariate analysis revealed that AP2>18 at BSI onset, and respiratory, cardiovascular, renal, hematologic and hepatic failure were associated with death, but time to appropriate therapy >24 hours, age, and infection due to VRE were not. Multivariate analysis revealed that hematologic (OR 8.4, p = .025

  11. Severe gastric dilatation due to superior mesenteric artery syndrome in anorexia nervosa.

    PubMed

    Mascolo, Margherita; Dee, Elizabeth; Townsend, Ronald; Brinton, John T; Mehler, Philip S

    2015-07-01

    Forty-seven year old female, with a history of anorexia nervosa, was admitted to a medical stabilization unit (ACUTE) complaining of abdominal pain exacerbated by oral intake, associated with nausea, and relieved by emesis. Admission body mass index was 10.6. Labs were notable for hepatitis and hypoglycemia. On her progressive oral refeeding plan, she suddenly developed severe abdominal pain. Computed tomography (CT) revealed gastric dilatation and superior mesenteric artery (SMA) syndrome. SMA syndrome is a rare complication of severe malnutrition resulting from compression of the duodenum between the aorta and the SMA. It is diagnosed by an upper gastrointestinal series or an abdominal CT. Gastric dilatation, in turn, is a rare complication of SMA syndrome to be included in the differential diagnoses of abdominal pain in severely malnourished patients as it is potentially life-threatening. The patient was switched to an oral liquid diet, began weight restoring, and had resolution of symptoms.

  12. 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.

  13. Painful Horner syndrome due to arteritis of the internal carotid artery.

    PubMed

    Bollen, A E; Krikke, A P; de Jager, A E

    1998-11-01

    A 44-year-old man presented with painful Horner syndrome: severe periorbital pain, ptosis, and miosis of his right eye, with intact facial sweating. Lymphadenitis at the right side of his neck preceded the symptoms. MRI and magnetic resonance angiography showed thickening of the right internal carotid artery, extending from the bifurcation to the cavernous sinus, without evidence for dissection. The patient was treated with corticosteroids with immediate improvement. Control MRI scanning was normal after 6 weeks. We conclude that the painful Horner syndrome was caused by a reactive arteritis of the right internal carotid artery. PMID:9818886

  14. 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...

  15. Dejerine-Sottas syndrome and vestibular loss due to a point mutation in the PMP22 gene.

    PubMed

    Jen, Joanna; Baloh, Robert H; Ishiyama, Akira; Baloh, Robert W

    2005-10-15

    We describe a father and daughter with Dejerine-Sottas syndrome and bilateral vestibular loss due to an L71P missense mutation in the peripheral myelin protein 22 (PMP22). The combination of vestibular loss and peripheral neuropathy led to profound imbalance at a young age. It is important to recognize this combination of peripheral nerve and vestibular deficits since rehabilitation strategies and prognosis are different. PMID:15992829

  16. 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. PMID:25772043

  17. Myocardial ischemia due to compression of an unruptured thoracic aortic aneurysm in a patient with Marfan syndrome.

    PubMed

    Minami, Hiroya; Asada, Tatsuro; Gan, Kunio; Abe, Koichiro; Izumi, Satoshi

    2007-06-01

    We report a 33-year-old woman who had a 60-mm thoracic aneurysm of the ascending aorta with Marfan syndrome and effort angina due to compression of the right coronary artery (RCA) by the aneurysm. Surgery was performed using the Bentall procedure and a coronary artery bypass graft to the RCA. Postoperatively, coronary angiography showed that the coronary flow of the RCA was restored by removing the aneurysmal compression. The patient was discharged without angina on postoperative day 21. PMID:17642279

  18. Late compartment syndrome of the hand due to wasp sting in a child.

    PubMed

    Petratos, Dimitrios V; Galanakos, Spyridon P; Stavropoulos, Nikolaos A; Anastasopoulos, John N

    2011-01-01

    We report a 6-year-old girl, with no history of previous anaphylactic reaction, who sustained a wasp sting to the volar aspect of her left hand. The child did not present any symptoms at the beginning. She was first examined at the emergency department with developed compartment syndrome, after more than 24 hours later and she was urgently taken to the operating theatre. The midpalmar, thenar, and hypothenar spaces were decompressed, and the transverse carpal ligament was released. At the 11-month followup, she presented with normal function of the hand and normal 2-point discrimination in all fingers. Although the accurate mechanism of the development of compartment syndrome after a wasp sting in children is not thoroughly clear, the treatment seems to be the same as in all other cases of compartment syndrome; urgent fasciotomy. It is very important to keep in mind the possibility, even if it is extremely low, of compartment syndrome after a wasp sting in children; even of those with no history of anaphylactic reaction. PMID:22214147

  19. 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…

  20. Physical and Psychological Health in Persons with Deafblindness that Is due to Usher Syndrome Type II

    ERIC Educational Resources Information Center

    Wahlqvist, Moa; Moller, Claes; Moller, Kerstin; Danermark, Berth

    2013-01-01

    Introduction: The objectives of the study reported here were to describe the physical and psychological health of persons with Usher syndrome Type II (USH2) and to explore any differences in terms of gender. Methods: The participants were recruited from the Swedish Usher database. In the first step, 122 persons received the questionnaire by mail,…

  1. Laparoscopic treatment of an upper gastrointestinal obstruction due to Bouveret’s syndrome

    PubMed Central

    Yang, Dong; Wang, Zhen; Duan, Zhi-Jun; Jin, Shi

    2013-01-01

    Bouveret’s syndrome is an extremely rare type of gallstone-induced ileus with atypical clinical manifestations, such as abdominal distension and pain, nausea and vomiting, fever or even gastrointestinal bleeding, which may easily be misdiagnosed. In the present case, a 55-year-old male was admitted to the hospital with upper gastrointestinal obstructive symptoms but without pain, fever, jaundice or melena. At first, gastrolithiasis and peptic ulcer combined with pyloric obstruction were suspected after gastroscopy revealed a large, hard stone in the duodenal bulb. A revised diagnosis of Bouveret’s syndrome was made following abdominal computed tomography. Subsequently, the patient exhibited a good postoperative recovery after laparoscopic duodenotomy for gallstone removal and subtotal cholecystectomy. The condition of the patient remained stable after being followed up for 6 mo. The successful application of laparoscopic therapy to treat Bouveret’s syndrome has seldom been reported. Laparoscopic enterolithotomy is safe and effective, with good patient tolerability, rapid postoperative recovery and few wound-related complications. The laparoscopic treatment of Bouveret’s syndrome is worth exploring. PMID:24187475

  2. Severe manifestation of Leber's hereditary optic neuropathy due to 11778G>A mtDNA mutation in a female with hypoestrogenism due to Perrault syndrome.

    PubMed

    Badura-Stronka, Magdalena; Wawrocka, Anna; Zawieja, Krzysztof; Silska, Sylwia; Krawczyński, Maciej Robert

    2013-11-01

    Perrault syndrome (PS) is a rare autosomal recessive condition with ovarian dysgenesis, hearing deficit and neurological abnormalities in female patients. The molecular basis of the syndrome is heterogeneous, mutations in the HSD17B4 gene have been identified in one family and mutations in the HARS2 gene have been found in another one. We have excluded pathogenic changes in the HSD17B4 gene and in the HARS2 gene by a direct sequencing of all coding exons in a female with clinical hallmarks of PS, ataxia and mild mental retardation. In addition, the patient suffers from severe Leber's hereditary optic neuropathy (LHON) due to 11778G>A mtDNA mutation. This case is the first reported patient with PS and LHON. Possible influence of hypoestrogenism on the manifestation of optic neuropathy in this patient is discussed in the context of recent findings concerning the crucial role of estrogens in supporting the vision capacity in LHON-related mtDNA mutation carriers.

  3. Congenital myasthenic syndromes due to mutations in ALG2 and ALG14.

    PubMed

    Cossins, Judith; Belaya, Katsiaryna; Hicks, Debbie; Salih, Mustafa A; Finlayson, Sarah; Carboni, Nicola; Liu, Wei Wei; Maxwell, Susan; Zoltowska, Katarzyna; Farsani, Golara Torabi; Laval, Steven; Seidhamed, Mohammed Zain; Donnelly, Peter; Bentley, David; McGowan, Simon J; Müller, Juliane; Palace, Jacqueline; Lochmüller, Hanns; Beeson, David

    2013-03-01

    Congenital myasthenic syndromes are a heterogeneous group of inherited disorders that arise from impaired signal transmission at the neuromuscular synapse. They are characterized by fatigable muscle weakness. We performed linkage analysis, whole-exome and whole-genome sequencing to determine the underlying defect in patients with an inherited limb-girdle pattern of myasthenic weakness. We identify ALG14 and ALG2 as novel genes in which mutations cause a congenital myasthenic syndrome. Through analogy with yeast, ALG14 is thought to form a multiglycosyltransferase complex with ALG13 and DPAGT1 that catalyses the first two committed steps of asparagine-linked protein glycosylation. We show that ALG14 is concentrated at the muscle motor endplates and small interfering RNA silencing of ALG14 results in reduced cell-surface expression of muscle acetylcholine receptor expressed in human embryonic kidney 293 cells. ALG2 is an alpha-1,3-mannosyltransferase that also catalyses early steps in the asparagine-linked glycosylation pathway. Mutations were identified in two kinships, with mutation ALG2p.Val68Gly found to severely reduce ALG2 expression both in patient muscle, and in cell cultures. Identification of DPAGT1, ALG14 and ALG2 mutations as a cause of congenital myasthenic syndrome underscores the importance of asparagine-linked protein glycosylation for proper functioning of the neuromuscular junction. These syndromes form part of the wider spectrum of congenital disorders of glycosylation caused by impaired asparagine-linked glycosylation. It is likely that further genes encoding components of this pathway will be associated with congenital myasthenic syndromes or impaired neuromuscular transmission as part of a more severe multisystem disorder. Our findings suggest that treatment with cholinesterase inhibitors may improve muscle function in many of the congenital disorders of glycosylation. PMID:23404334

  4. Sudden cardiac arrest due to a single sodium channel mutation producing a mixed phenotype of Brugada and Long QT3 syndromes.

    PubMed

    Lakshmanadoss, U; Mertens, A; Gallagher, M; Kutinsky, I; Williamson, B

    2016-01-01

    Inherited arrhythmia syndromes are a known, albeit rare, cause of sudden cardiac arrest which may present with characteristic electrocardiogram changes in patients with structurally normal heart. There are a variety of distinct arrhythmogenic syndromes that arise from mutations in voltage gated sodium channels, resulting in either gain or loss of function. We describe a patient with a primary inherited arrhythmia syndrome which presented as sudden cardiac arrest. Further workup revealed that her arrest was due to a combination of Brugada syndrome and Long QT3 syndrome secondary to a deleterious mutation of voltage-gated, sodium channel, type V alpha subunit (SCN5A Thr1709Met). PMID:27676163

  5. Moral distress: the state of the science.

    PubMed

    Hanna, Debra R

    2004-01-01

    Moral distress, a complex human experience, has lacked a clear, complete definition. Intuitively, clinicians know that moral distress might be occurring for patients with increasing frequency due to technological advances that alter the natural order of life and death. Yet clinicians have not been able to evaluate the presence or extent of moral distress. To date, moral distress has been investigated mainly as an occupational issue using Jameton's (1984) definition, which has been problematic for several reasons. Without an adequate definition, moral distress can be unrecognized, yet have a silent, clinically significant impact on health. The literature is discussed from several perspectives to show the current state of the science in this topical area, and its potential future.

  6. 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

  7. 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. PMID:27525139

  8. Cerebral salt wasting syndrome due to tuberculous meningitis; a case report

    PubMed Central

    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. PMID:27069970

  9. 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.

  10. 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.

  11. Immune reconstitution syndrome in a human immunodeficiency virus infected child due to giardiasis leading to shock.

    PubMed

    Nandy, Sneha; Shah, Ira

    2015-01-01

    Human immunodeficiency virus (HIV)-associated immune reconstitution inflammatory syndrome has been reported in association with tuberculosis, herpes zoster (shingles), Cryptococcus neoformans, Kaposi's sarcoma, Pneumocystis pneumonia, hepatitis B virus, hepatitis C virus, herpes simplex virus, Histoplasma capsulatum, human papillomavirus, and Cytomegalovirus. However, it has never been documented with giardiasis. We present a 7-year-old HIV infected girl who developed diarrhea and shock following the initiation of antiretroviral therapy, and her stool showed the presence of giardiasis. PMID:26985424

  12. Death due to fulminant neuroleptic malignant syndrome induced by low doses of haloperidol: a rare case.

    PubMed

    Zou, Donghua; Shao, Yu; Qin, Zhiqiang; Zhang, Jianhua; Liu, Ningguo; Li, Zhengdong; Huang, Ping; Chen, Yijiu

    2014-05-01

    The paper reports on a rare case of fulminant neuroleptic malignant syndrome (NMS) with several risk factors, typical manifestation and rapid death induced by low doses of haloperidol. The pathological findings, pathogenesis, clinical manifestations, diagnostic criteria, risk factors and other features of NMS are discussed. The importance of forensic pathologists being aware of the possibility of NMS as the cause of death in people taking antipsychotic drugs is stressed. PMID:24794843

  13. The Forgotten One: Lemierre’s Syndrome Due to Gram-Negative Rods Prevotella Bacteremia

    PubMed Central

    Wani, Priyanka; Antony, Nishaal; Wardi, Miraie; Rodriguez-Castro, Carlos E.; Teleb, Mohamed

    2016-01-01

    Patient: Male, 22 Final Diagnosis: Lemierre’s syndrome Symptoms: Dyspnea • chest pain • swelling Medication: — Clinical Procedure: Thoracentesis Specialty: Infectious Diseases Objective: Rare co-existance of disease or pathology Background: Lemierre’s syndrome (LS) is a rare syndrome caused by an acute oropharyngeal infection with metastatic spreading. It was described in 1939 as jugular vein septic thrombophlebitis associated with retropharyngeal infection. Different organisms can cause LS, such as Fusobacterium species, Peptostreptococcus, group B and C, Streptococcus, Staphylococcus, and Enterococcus species, but the most commonly isolated pathogen is Fusobacterium necrophorum, a common oral flora. Management depends on the initial presentation, type of pathogen isolated, and proper selection of antibiotics. Case Report: We report a case of a 22-year-old man with no past medical history, who presented with left jaw pain and progressive left facial area swelling associated with dyspnea. A final diagnosis of LS was made based on criteria of computed tomography (CT) of the neck and the clinical symptoms. The patient was started on broad-spectrum antibiotics. Subsequent imaging of the chest showed pleural effusion with septic emboli. He underwent thoracentesis and chest tube placement. Final blood cultures were remarkable for gram-negative rods – Prevotella anaerobes – which supported the diagnosis of LS. His condition improved, including the dyspnea, and he was discharged on the proper antibiotics coverage with outpatient follow-up. Conclusions: LS is a rare condition associated with metastatic infection spreading. This syndrome can be associated with further complications, such as pleural effusions and/or empyemas. Early recognition is important to prevent fatal complications and provide adequate antibiotics coverage. We report only the third case in the medical literature of Prevotella-induced LS with a secondary complication of pleural effusion

  14. 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.

  15. Acute intraparenchymal spinal cord injury in a cat due to high-rise syndrome

    PubMed Central

    Cruz–Arámbulo, Robert; Nykamp, Stephanie

    2012-01-01

    A 9-year-old spayed female Bengal Red cat was evaluated for high-rise syndrome. The cat had paraplegia of the hind limbs, intact reflexes and pain perception, and hyperesthesia in the caudal thoracic area. Mentation, cranial nerve function, forelimb proprioceptive responses, and spinal reflexes were normal. There were no abnormalities on radiographs or computed tomography scan, but magnetic resonance imaging revealed a hyperintense intraparenchymal spinal cord lesion on T2-weighted and T2 fat saturation images. PMID:22942443

  16. [Alice in Wonderland syndrome due to Epstein-Barr virus infection].

    PubMed

    Pérez Méndez, C; Martín Mardomingo, M; Otero Martínez, B; Lagunilla Herrero, L; Fernández Zurita, C

    2001-06-01

    The Alice in Wonderland syndrome refers to distortions in body image and in the apparent sizes, shapes, and spatial relations of objects seen. The syndrome is usually associated with migraine headaches and has also been reported in several viral infections. We report a 6-year-old boy who presented to the emergency department complaining of several episodes in which the ceiling, the objects and the people around him seemed very small and far away. The child presented no alteration in the level of consciousness. The episodes provoked great fear in the child. Physical examination revealed no abnormalities except pharyngoamygdalitis. Serologic studies (IgM antibodies to viral capsid antigen) confirmed Epstein-Barr virus infection. The child's symptoms resolved spontaneously within 48 hours and he continued to be asymptomatic after a 4 -month follow-up. We consider that all children presenting a clinical picture consistent with the Alice in Wonderland syndrome should undergo serological testing for Epstein Barr virus infection. Diagnosis would enable physicians to reassure the family of the temporary and benign nature of this alarming condition. PMID:11412412

  17. Abnormal visual evoked potentials in children with "Alice in Wonderland" syndrome due to infectious mononucleosis.

    PubMed

    Lahat, E; Berkovitch, M; Barr, J; Paret, G; Barzilai, A

    1999-11-01

    Visual illusions characterized by distortion of form, size, reciprocal position of objects, movement, or color, labeled as "Alice in Wonderland" syndrome, were discussed in children with infectious mononucleosis, as well as in other clinical conditions, such as migraine, epilepsy, use of certain hallucinogenic drugs, etc. The purpose of our study was to investigate for the first time visual evoked potential results in children with "Alice in Wonderland" syndrome associated with infectious mononucleosis. Five children with "Alice in Wonderland" syndrome associated with infectious mononucleosis underwent visual evoked potential studies during and after their clinical symptoms. Visual evoked potential results during the disease demonstrated statistically significant high amplitudes of P100-N145 in all children compared to the control group. A few weeks later, repeated studies after the resolution of the complaints were normal. Since the same findings can be observed in patients with migraine, we postulate that a common pathophysiologic underlying abnormality, which can cause transient focal decreased cerebral perfusion, could be involved in the disease process of these two conditions. PMID:10593551

  18. A case of severe hyperkalaemia and compartment syndrome due to rhabdomyolysis after drugs abuse.

    PubMed

    Ochoa-Gómez, J; Villar-Arias, A; Aresti, Irune; Marco-Aguilar, Pedro

    2002-07-01

    Severe hyperkalaemia is one of the complications of the non-traumatic rhabdomyolysis, which have been related to drug abuse, alcohol, etc. We report on a case of bilateral tibial compartment syndrome, severe hyperkalaemia and rhabdomyolysis after drug abuse. A 35-year-old male intravenous drug user was admitted to the emergency department after being found unconscious in his cell of the prison. Physical examination at emergency department revealed no abnormalities except constricted pupils. Two hours after admission a wide QRS was observed in the electrocardiography and he developed asystole. Cardiopulmonary resuscitation was performed and transcutaneous pacing was applied at the beginning of cardiac arrest (150 mAmp and stimulation frequency 80 beats/min). At the moment of cardiac arrest, laboratory test showed potassium 9.2 mmol/l. Return of spontaneous circulation occurred in 21 min, and he was admitted to the Intensive Care Unit. Thirty-six hours after admission a compartment syndrome of both lower legs was suspected because of oedema with peripheral pulses. Rhabdomyolysis has been reported after drug abuse. There is severe hyperkalaemia which should be identified and treated. A more rare complication of rhabdomyolysis is the compartment syndrome, a surgical emergency, which requires immediate fasciotomy to prevent serious complications.

  19. 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. PMID:27567297

  20. 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

  1. 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.

  2. Intracranial hemorrhage due to intracranial hypertension caused by the superior vena cava syndrome.

    PubMed

    Bartek, Jiri; Abedi-Valugerdi, Golbarg; Liska, Jan; Nyström, Harriet; Andresen, Morten; Mathiesen, Tiit

    2013-07-01

    We report a patient with intracranial hemorrhage secondary to venous hypertension as a result of a giant aortic pseudoaneurysm that compressed the superior vena cava and caused obstruction of the venous return from the brain. To our knowledge, this is the first patient reported to have an intracranial hemorrhage secondary to a superior vena cava syndrome. The condition appears to be caused by a reversible transient rise in intracranial pressure, as a result of compression of the venous return from the brain. Treatment consisted of surgery for the aortic pseudoaneurysm, which led to normalization of the intracranial pressure and resorption of the intracranial hemorrhage.

  3. 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.

  4. 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

  5. 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.

  6. Hyponatremia due to cerebral salt-wasting syndrome. Combined cerebral and distal tubular lesion.

    PubMed

    Al-Mufti, H; Arieff, A I

    1984-10-01

    A 76-year-old white man was evaluated for a syndrome of hyponatremia, hypotension, and high urinary sodium excretion. There was evidence of inappropriate secretion of antidiuretic hormone and renal salt wasting in the presence of a normal glomerular filtration rate. He had a distal tubular acidification defect and unresponsiveness to standard doses of mineralocorticoids. The renin aldosterone axis was normal, as were thyroid and adrenal function. The patient could not dilute the urine, nor excrete a standard water load. Renal concentrating ability was normal, but there was no additional response to exogenous vasopressin. With modest salt restitution, the patient continued to lose large quantities of sodium in the urine, resulting in severe postural hypotension. Renal biopsy showed normal glomeruli with distinct degeneration of the distal tubules. There was no evidence of an acute inflammatory interstitial nephritis. The patient did not respond to therapeutic doses of mineralocorticoid (fludrocortisone), but treatment with water restriction, increased salt intake, and large doses of mineralocorticoids resulted in a normal serum sodium level and blood pressure. This case falls in the category of "cerebral salt wasting" syndrome. The cause was a combination of idiopathic secretion of antidiuretic hormone and distal tubular degeneration resulting in pseudohypoaldosteronism.

  7. Neonatal liver failure and Leigh syndrome possibly due to CoQ-responsive OXPHOS deficiency.

    PubMed

    Leshinsky-Silver, E; Levine, A; Nissenkorn, A; Barash, V; Perach, M; Buzhaker, E; Shahmurov, M; Polak-Charcon, S; Lev, D; Lerman-Sagie, T

    2003-08-01

    CoQ transfers electrons from complexes I and II of the mitochondrial respiratory chain to complex III. There are very few reports on human CoQ deficiency. The clinical presentation is usually characterized by: epilepsy, muscle weakness, ataxia, cerebellar atrophy, migraine, myogloblinuria and developmental delay. We describe a patient who presented with neonatal liver and pancreatic insufficiency, tyrosinemia and hyperammonemia and later developed sensorineural hearing loss and Leigh syndrome. Liver biopsy revealed markedly reduced complex I+III and II+III. Addition of CoQ to the liver homogenate restored the activities, suggesting CoQ depletion. Histological staining showed prominent bridging; septal fibrosis and widening of portal spaces with prominent mixed inflammatory infiltrate, associated with interface hepatitis, bile duct proliferation with numerous bile plugs. Electron microscopy revealed a large number of mitochondria, which were altered in shape and size, widened and disordered intercristal spaces. This may be the first case of Leigh syndrome with liver and pancreas insufficiency, possibly caused by CoQ responsive oxphos deficiency. PMID:12948744

  8. Myoclonic status and central fever in Angelman syndrome due to paternal uniparental disomy.

    PubMed

    Nicita, Francesco; Garone, Giacomo; Papetti, Laura; Consoli, Federica; Magliozzi, Monia; De Luca, Alessandro; Spalice, Alberto

    2015-01-01

    Myoclonic status in nonprogressive encephalopathy (MSNE) is an early-onset, drug-resistant epileptic syndrome characterized by occurrence of continuous diffuse epileptiform abnormalities, associated with positive and/or negative phenomena and accompanied by transient and recurring motor, cognitive, and behavioral impairment. MSNE has been reported in Angelman syndrome (AS) secondary to 15q11-13 deletions or UBE3A mutations but not to paternal uniparental disomy (UPD). We describe the case of a male patient with AS caused by UPD who developed a myoclonic status (MS) associated with long-lasting fever of central origin, both promptly regressed with introduction of levetiracetam. Only three descriptions of thermal dysregulation in AS exist, and none of the previously reported cases were associated with MS or with UPD. Association of MS and central fever expands the spectrum of epileptic and non-epileptic features in UPD-related AS and provides a further evidence of hypothalamus involvement in the pathogenesis of this neurodevelopmental disorder. PMID:26559560

  9. Turner syndrome presented with tall stature due to overdosage of the SHOX gene

    PubMed Central

    Seo, Go Hun; Kang, Eungu; Cho, Ja Hyang; Lee, Beom Hee; Choi, Jin-Ho; Kim, Gu-Hwan; Seo, Eul-Ju

    2015-01-01

    Turner syndrome is one of the most common chromosomal disorders. It is caused by numerical or structural abnormalities of the X chromosome and results in short stature and gonadal dysgenesis. The short stature arises from haploinsufficiency of the SHOX gene, whereas overdosage contributes to tall stature. This report describes the first Korean case of Turner syndrome with tall stature caused by SHOX overdosage. The patient presented with primary amenorrhea and hypergonadotropic hypogonadism at the age of 17 years. Estrogen replacement therapy was initiated at that time. She displayed tall stature from childhood, with normal growth velocity, and reached a final height of 190 cm (standard deviation score, 4.3) at the age of 30 years. Her karyotype was 46,X, psu idic(X)(q21.2), representing partial monosomy of Xq and partial trisomy of Xp. Analysis by multiplex ligation-dependent probe amplification detected a duplication at Xp22.3-Xp22.2, encompassing the PPP2R3 gene near the 5'-end of the SHOX gene through the FANCD gene at Xp22.2. PMID:26191517

  10. Successful Medical Therapy for Hypophosphatemic Rickets due to Mitochondrial Complex I Deficiency Induced de Toni-Debré-Fanconi Syndrome

    PubMed Central

    Bowden, Sasigarn A.; Patel, Hiren P.; Beebe, Allan; McBride, Kim L.

    2013-01-01

    Primary de Toni-Debré-Fanconi syndrome is a non-FGF23-mediated hypophosphatemic disorder due to a primary defect in renal proximal tubule cell function resulting in hyperphosphaturia, renal tubular acidosis, glycosuria, and generalized aminoaciduria. The orthopaedic sequela and response to treatment of this rare disorder are limited in the literature. Herein we report a long term followup of a 10-year-old female presenting at 1 year of age with rickets initially misdiagnosed as vitamin D deficiency rickets. She was referred to the metabolic bone and genetics clinics at 5 years of age with severe genu valgum deformities of 24 degrees and worsening rickets. She had polyuria, polydipsia, enuresis, and bone pain. Diagnosis of hypophosphatemic rickets due to de Toni-Debré-Fanconi syndrome was subsequently made. Respiratory chain enzyme analysis identified a complex I mitochondrial deficiency as the underlying cause. She was treated with phosphate (50–70 mg/kg/day), calcitriol (30 ng/kg/day), and sodium citrate with resolution of bone pain and normal growth. By 10 years of age, her genu valgus deformities were 4 degrees with healing of rickets. Her excellent orthopaedic outcome despite late proper medical therapy is likely due to the intrinsic renal tubular defect that is more responsive to combined alkali, phosphate, and calcitriol therapy. PMID:24386581

  11. Anticholinergic syndrome due to 'Devil's herb': when risks come from the ancient time.

    PubMed

    Piccillo, G A; Miele, L; Mondati, E; Moro, P A; Musco, A; Forgione, A; Gasbarrini, G; Grieco, A

    2006-04-01

    We describe a case of Mandragora autumnalis poisoning which occurred in a 72-year-old female patient who had eaten the venenous M. Autumnalis, picked near her home, mistaking it for the edible Borago Officinalis. M. Autumnalis is a solanaceous plant, common in the Sicilian countryside, which contains a variable concentration of solanum alkaloids, causing gastrointestinal irritation, and tropane alkaloids, with anticholinergic properties. Unluckily, M. Autumnalis is often mistaken for the edible B. Officinalis, likewise widespread in Sicilian countryside. The diagnosis of Mandragora poisoning was made on the basis of clinical symptoms and signs of anticholinergic syndrome associated with a history of vegetable meal of uncontrolled origin, moreover analysing the vegetable obtained from gastric lavage. Decontamination and symptomatic treatment were useful in our patient to control acute poisoning. PMID:16620365

  12. Hemolytic uremic syndrome due to gemcitabine in a young woman with cholangiocarcinoma.

    PubMed

    Nieto-Ríos, John Fredy; Zuluaga-Quintero, Mónica; Higuita, Lina Maria Serna; Rincón, Cristian Ivan García; Galvez-Cárdenas, Kenny Mauricio; Ocampo-Kohn, Catalina; Aristizabal-Alzate, Arbey; Florez-Vargas, Adriana Alejandra; Zuluaga-Valencia, Gustavo Adolfo

    2016-06-01

    Gemcitabine is a medication used to treat various types of malignant neoplasms. Its association with hemolytic uremic syndrome (HUS) has been described in few cases, although these cases have resulted in mortality rates of at least 50%. We report on the case of a 25-year-old patient with cholangiocarcinoma in remission who developed microangiopathic hemolytic anemia with acute anuric renal failure after receiving 5 cycles of gemcitabine chemotherapy; this condition was consistent with HUS caused by the side effects of this drug. The administration of gemcitabine was stopped, and hemodialysis, blood transfusions, plasma exchanges, steroids, doxycycline, and rituximab were used to treat the patient. A favorable outcome was achieved; in particular, hemolysis was controlled, and renal function was completely recovered. PMID:27438981

  13. Subarachnoid hemorrhage due to ruptured intracranial aneurysm following posterior reversible encephalopathy syndrome

    PubMed Central

    Nanba, Takamasa; Kashimura, Hiroshi; Saura, Hiroaki; Takeda, Masaru

    2016-01-01

    Although posterior reversible encephalopathy syndrome (PRES) is rarely associated with subarachnoid hemorrhage, to our knowledge, rupture of a concomitant cerebral aneurysm following PRES has not been reported. We describe a patient with atypical PRES involving the brainstem, thalamus, and periventricular white matter without cortical or subcortical edema of the parietooccipital lobe on magnetic resonance imaging, with rupture of a concomitant cerebral aneurysm. Preexisting extremely high blood pressure may trigger atypical PRES, and failure to lower blood pressure may lead to a concomitant aneurysm rupture. In the future treatment of hypertensive urgency with a recurrence of symptoms and mean arterial blood pressure >150 mmHg, it is advisable to immediately hospitalize the patient for aggressive blood pressure management, especially if PRES is suspected based on clinical and radiological features. PMID:27365964

  14. Pudendal Nerve Entrapment Syndrome due to a Ganglion Cyst: A Case Report.

    PubMed

    Lee, Jae Wook; Lee, Sung-Moon; Lee, Dong Gyu

    2016-08-01

    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

  15. CLINICAL VARIABILITY IN TWO SISTERS WITH KEUTEL SYNDROME DUE TO A HOMOZYGOUS MUTATION IN MGP GENE.

    PubMed

    Tüysüz, B; Cinar, B; Laçiner, S; Onay, H; Mittaz-Crettol, L

    2015-01-01

    Keutel syndrome (KS) is an autosomal recessive disease characterised by abnormal cartilage calcification, brachytelephalangism, peripheral pulmonary artery stenosis, hearing loss and midface retrusion. KS is caused by homozygous mutations in MGP, a gene encoding Matrix Gla protein which acts as a calcification inhibitor in extracellular matrix. We present two Turkish sisters (22 and 13 years old) who had abnormal cartilage calcification, brachytelephalangism, congenital heart defect and chronic asthmatic bronchitis. The patients were homozygous for c.62-2A>G (IVS1-2 A>G) mutation in MGP gene. Abnormal cartilage calcification, brachytelephalangism and midfacial retrusion are the hallmarks of KS. It was observed that the younger sister had striking cartilaginous calcifications, midfacial retrusion and severe brachytelephalangism while her older sister had mild costal cartilaginous calcifications and brachytelephalangism without any midfacial retrusion. Intrafamiliar clinical variability for KS has not been described previously. PMID:26349188

  16. 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

  17. Complete androgen insensitivity syndrome due to X chromosome inversion: a clinical report.

    PubMed

    Xu, Weizhen; Robert, Claudia; Thornton, Paul S; Spinner, Nancy B

    2003-07-30

    We have studied a patient with complete androgen insensitivity syndrome (CAIS) and a 46, inv(X),Y karyotype. The patient's mother and maternal aunt also carry the inverted X, and the mother is phenotypically normal, with a 46, inv(X),X karyotype, while a maternal aunt has CAIS with a 46,inv(X),Y karyotype. Molecular cytogenetic studies demonstrate that one of the X inversion breakpoints occurs within Xq11.2-12, which is the locus for the androgen receptor. FISH analysis demonstrated that a BAC clone containing the androgen receptor gene was disrupted by the inversion. We therefore hypothesize that disruption of the androgen receptor gene causes CAIS in this patient. This is the first report of CAIS caused by a chromosome inversion.

  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.

  19. Frequency of Births Due to Assisted Reproductive Technology (ART) in Prader-Willi Syndrome

    PubMed Central

    Gold, June-Anne; Ruth, Chelsey; Osann, Kathryn; Flodman, Pamela; McManus, Barbara; Lee, Hye-Seung; Donkervoort, Sandra; Khare, Manaswitha; Roof, Elizabeth; Dykens, Elizabeth; Driscoll, Daniel J.; Butler, Merlin G.; Heinemann, Janalee; Cassidy, Suzanne; Kimonis, Virginia

    2014-01-01

    Purpose Prader-Willi syndrome (PWS) is an imprinting disorder characterized by typical facial, physical and cognitive/behavioral features, resulting from lack of paternally-expressed genes on chromosome 15q11.2-q13. Studies have suggested an increased risk of other imprinting disorders in children conceived by assisted reproductive techniques (ART). This study was designed to determine the association between ART and PWS. Methods Data on individuals with PWS were collected from three distinct sources and the proportion of ART-births analyzed. Results The proportion of ART-births in the Prader-Willi Syndrome Association [PWSA (USA)], Rare Diseases Clinical Research Network (RDCRN), and University of California, Irvine Medical Center (UCIMC) populations was 1.0% (18/1,736), 1.0% (1/98), and 2.0% (1/50), respectively (overall 1.1%; population frequency for the U.S was 1.0%). Interestingly, 2.4% (45/1,898) of participants were co-twins (eleven born after ART procedures); U.S. twin frequency is 1.6% (p=0.007). The proportion of individuals with maternal disomy 15/imprinting defects born after ART was higher than in the total sample, 55.6% (10/18) and 34.5% (431/1,250), respectively. Conclusion This study found no association between ART and PWS. There was an increased frequency of twinning. The number of individuals with maternal disomy 15/imprinting defect was nearly double in the ART group compared to the total PWS participants. PMID:23928912

  20. MERRF/MELAS overlap syndrome due to the m.3291T>C mutation.

    PubMed

    Liu, Kaiming; Zhao, Hui; Ji, Kunqian; Yan, Chuanzhu

    2014-03-01

    We report the case of a 19-year-old Chinese female harboring the m.3291T>C mutation in the MT-TL1 gene encoding the mitochondrial transfer RNA for leucine. She presented with a complex phenotype characterized by progressive cerebellar ataxia, frequent myoclonus seizures, recurrent stroke-like episodes, migraine-like headaches with nausea and vomiting, and elevated resting lactate blood level. It is known that the myoclonus epilepsy with ragged-red fibers (MERRF) is characterized by cerebellar ataxia and myoclonus epilepsy, while that the mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) is characterized by recurrent stroke-like episodes, migraine-like headaches, and elevated resting lactate blood level. So the patient's clinical manifestations suggest the presence of a MERRF/MELAS overlap syndrome. Muscle biopsy of the patient showed the presence of numerous scattered ragged-red fibers, some cytochrome c oxidase-deficient fibers, and several strongly succinate dehygrogenase-reactive vessels, suggestive of a mitochondrial disorder. Direct sequencing of the complete mitochondrial genome of the proband revealed no mutations other than the T-to-C transition at nucleotide position 3291. Restriction fragment length polymorphism analysis of the proband and her family revealed maternal inheritance of the mutation in a heteroplasmic manner. The analysis of aerobic respiration and glycolysis demonstrated that the fibroblasts from the patient had mitochondrial dysfunction. Our results suggest that the m.3291T>C is pathogenic. This study is the first to describe the m.3291T>C mutation in association with the MERRF/MELAS overlap syndrome.

  1. Low-molecular-weight dextran infusion is more effective for the treatment of hemoconcentration due to severe ovarian hyperstimulation syndrome than human albumin infusion.

    PubMed

    Endo, Toshiaki; Kitajima, Yoshimitsu; Hayashi, Takuhiro; Fujii, Miho; Hata, Hiroshi; Azumaguchi, Atsushi

    2004-11-01

    The most severe complication of ovarian hyperstimulation syndrome (OHSS) is thromboembolism, which is related to hemoconcentration. Dextran 40 infusion has greater effectiveness for the treatment of hemoconcentration due to OHSS than does human albumin infusion. PMID:15533378

  2. Probable case of drug reaction with eosinophilia and systemic symptom syndrome due to combination therapy with daclatasvir and asunaprevir

    PubMed Central

    Suga, Takayoshi; Sato, Ken; Yamazaki, Yuichi; Ohyama, Tatsuya; Horiguchi, Norio; Kakizaki, Satoru; Kusano, Motoyasu; Yamada, Masanobu

    2015-01-01

    A 66-year-old, interferon-ineligible, treatment-naive man who was diagnosed with chronic hepatitis C due to hepatitis C virus genotype 1b began combination therapy with daclatasvir and asunaprevir. On day 14 of treatment, hepatic reserve and renal function deterioration was observed, while his transaminase levels were normal. Both daclatasvir and asunaprevir were discontinued on day 18 of treatment, because the patient complained of dark urine and a rash on his trunk and four limbs. After discontinuing antiviral therapy, the abnormal laboratory finding and clinical manifestations gradually improved, without recurrence. Our case fulfilled the diagnostic criteria of probable drug reaction with eosinophilia and systemic symptom (DRESS) syndrome. Despite the 18-d treatment, sustained virological response 12 was achieved. Based on the clinical course, we concluded that there was a clear cause-and-effect relationship between the treatment and adverse events. To our knowledge, this patient represents the first case of probable DRESS syndrome that includes concomitant deterioration of hepatic reserve and renal function due to combination therapy with daclatasvir and asunaprevir, regardless of normalization of transaminase levels. Our case suggests that we should pay attention not only to the transaminase levels but also to allergic symptoms associated with organ involvement during combination therapy with daclatasvir and asunaprevir. PMID:26677451

  3. Hyperphosphatasia-mental retardation syndrome due to PIGV mutations: expanded clinical spectrum.

    PubMed

    Horn, Denise; Krawitz, Peter; Mannhardt, Anca; Korenke, Georg Christoph; Meinecke, Peter

    2011-08-01

    Hyperphosphatasia-mental retardation syndrome is a recently delineated disorder associated with a recognizable facial phenotype and brachytelephalangy. This autosomal recessive condition is caused by homozygous and compound heterozygous missense mutations of PIGV, encoding a member of the GPI-anchor biosynthesis pathway. Here, we report on two further, unrelated patients with developmental delay, elevated serum levels of AP, distinctive facial features, hypoplastic terminal phalanges, anal atresia in one and Hirschsprung disease in the other patient. By sequencing PIGV we detected compound heterozygous mutations c.467G>A and c.1022C>A in Patient 1 and a homozygous mutation c.1022C>A in Patient 2. We reviewed the eight reported cases with proven PIGV mutations and re-defined the phenotypic spectrum associated with PIGV mutations: intellectual disability, the distinct facial gestalt, brachytelephalangy, and hyperphosphatasia are constant features but also anorectal malformations and Hirschsprung disease as well as cleft lip/palate and hearing impairment should be considered as part of the clinical spectrum. Moreover, seizures and muscular hypotonia are frequently associated with PIGV mutations. PMID:21739589

  4. Evidence for decreased growth hormone in patients with hypothalamic hamartoma due to Pallister-Hall syndrome.

    PubMed

    Feuillan, P; Peters, K F; Cutler, G B; Biesecker, L G

    2001-02-01

    Pallister-Hall syndrome (PHS) is characterized by hypothalamic hamartoma, bifid epiglottis, and central or postaxial polydactyly. Familial transmission is autosomal dominant; isolated cases also occur. To screen for hypothalamic-pituitary dysfunction in PHS, we studied a 12 year-old boy (patient #1), and 14 additional patients (patients #2-14: 7M, 7F; ages 4-72 yr). We performed serial sampling of GH, LH/FSH, TSH, and cortisol from 20.00-08 00 h. At 08.00 h, we measured IGF-I, peak responses of LH and FSH after GnRH, and cortisol after ACTH. We found that 6/7 children, including patient #1, and 6/8 adults had low or absent spontaneous GH secretion and/or low levels of IGF-I. Patient #1 also had accelerated pubertal development, but no other patient had abnormalities of the pituitary-gonadal axis, and none of the 14 patients had an abnormal thyroid or adrenal axis. We conclude that decreased pituitary GH secretion is common in PHS, and may exist in the absence of other forms of endocrine dysfunction.

  5. [A case with both infectious cavernous sinus thrombosis and Lemierre syndrome due to intraoral resident flora].

    PubMed

    Nishida, Akihiro; Ogata, Toshiyasu; Kudo, Masataka; Fukuhara, Kousuke; Fukae, Jiro; Tsuboi, Yoshio

    2015-01-01

    The present report describes a 54-year-old woman with cavernous sinus thrombosis (CST) presenting with fever, and marked periorbital swelling. There is a history of untreated periodontal disease. On initial examination, periorbital pain associated with bilateral blephaloptosis, chemosis, and disturbed eye movement was present. The laboratory evaluation showed significant elevations in inflammatory and fibrinolytic markers. Diffusion-weighted MRI revealed high signal intensities in the bilateral superior ophthalmic veins (SOV). Contrast-enhanced computed tomography (CT) of the cranium showed an enlarged right SOV and a non-enhancing lesion within the right SOV and bilateral cavernous sinus, indicating cavernous sinus thrombosis with diffuse SOV thrombosis. Blood culture performed on admission showed bacterial infection by intraoral resident flora; therefore, the CST was attributed to untreated periodontal disease. Contrast-enhanced CT of the case also revealed the presence of thrombosis in the jugular vein associated with micropulmonary embolus, indicating co-occurrence of Lemierre's syndrome. Antibiotic and anticoagulant treatment were initiated, and the tooth decay was treated; all clinical symptoms and signs subsequently improved. Additional neuroimaging showed that the thrombus was absent from both SOV and the cavernous sinus. Infectious CST is life threatening; therefore, laboratory and imaging examination should be performed quickly, and antibiotic and anticoagulant therapy administrated immediately. PMID:26041393

  6. Patterns of psychological distress in mothers of preterm infants.

    PubMed

    Holditch-Davis, Diane; Santos, Hudson; Levy, Janet; White-Traut, Rosemary; O'Shea, T Michael; Geraldo, Victoria; David, Richard

    2015-11-01

    Mothers of preterm infants experience significant psychological distress, with elevated levels of inter-correlated depressive, 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 during 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 sub-groups 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. Haemophilia A and cardiovascular morbidity in a female SHAM syndrome carrier due to skewed X chromosome inactivation.

    PubMed

    Janczar, Szymon; Kosinska, Joanna; Ploski, Rafal; Pastorczak, Agata; Wegner, Olga; Zalewska-Szewczyk, Beata; Paige, Adam J W; Borowiec, Maciej; Mlynarski, Wojciech

    2016-01-01

    We have recently described a severe haemophilia A and moyamoya (SHAM) syndrome caused by Xq28 deletions encompassing F8 and the BRCC3 familial moyamoya gene. The phenotype includes haemophilia A, moyamoya angiopathy, dysmorphia and hypertension. The genetic analysis of the family of our SHAM patient demonstrated carrier state in proband's mother and sister. The patient's mother is apparently well, whereas his currently 18-years-old sister presents with mild haemophilia A, coarctation of the aorta, hypertension, and ventricular arrhythmia. We performed X chromosome inactivation assay based on HpaII methylation analysis of a polymorphic short tandem repeat (STR) in the X linked AR (androgen receptor) gene and used quantitative real-time RT PCR to measure the expression of genes from the deleted region in proband's family members. We found an extremely skewed X chromosome inactivation pattern in the female members of the family leading to preferential inactivation of the X chromosome without Xq28 deletion in patient's sister. We demonstrated differential expression of the genes from the deleted region in four members of the family, that tightly correlates with the clinical features. In conclusion, we show that the haematologic and cardiovascular morbidity and the discrepancy between patient's sister and mother despite the same genetic lesion are due to skewed X chromosome inactivation leading to clinically relevant differential expression of SHAM syndrome genes. This report highlights the role for BRCC3 in cardiovascular physiology and disease, and demonstrates that in some complex hereditary syndromes full diagnostics may require the examination of both genetic and epigenetic events. PMID:26691666

  8. [A case of acute chronic respiratory failure due to fat embolism syndrome after the left femoral neck fracture].

    PubMed

    Oda, Keishi; Kawanami, Toshinori; Yatera, Kazuhiro; Ogoshi, Takaaki; Kozaki, Minako; Nagata, Shuya; Nishida, Chinatsu; Yamasaki, Kei; Ishimoto, Hiroshi; Mukae, Hiroshi

    2011-09-01

    A 78 year old Japanese woman was transferred to our hospital for the treatment of a fracture of the left femoral neck in April, 2010. She had been taking oral corticosteroid (prednisolone 5 mg/day) for the treatment of idiopathic interstitial pneumonia since 2003, and had been treated by home oxygen therapy since 2007. She fell in the restroom at home and hurt herself, and was transferred to our hospital for treatment of a left femoral neck fracture in April, 2010. Her respiratory status was stable just after the transfer; however, she was transferred to the intensive care unit and started to receive mechanical ventilation due to rapidly progressive respiratory failure on the fourth day after admission. Chest X-ray and computed tomography revealed rapid progression of bilateral ground-glass attenuations, and acute exacerbation of interstitial pneumonia was clinically suspected. However, the elevation of D-dimer over time and characteristic findings of petechial hemorrhagic lesions on her palpebral conjunctivae and neck with microscopic findings of phagocytized lipid in alveolar macrophages in her endobronchial secretion led to the diagnosis of fat embolism syndrome. She was successfully treated with high-dose corticosteroid and sivelestat sodium, and she was discharged on the 21st day after admission. Although a differential diagnosis of acute exacerbation of interstitial pneumonia and fat embolism syndrome was necessary and difficult in the present case, characteristic findings of petechial hemorrhagic lesions of skin, palpebral conjunctiva and lipid-laden alveolar macrophages in endotracheal aspirate were useful for the accurate and prompt diagnosis of fat embolism syndrome.

  9. 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

  10. 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

  11. The Role of Inspiratory Muscle Training in Sickle Cell Anemia Related Pulmonary Damage due to Recurrent Acute Chest Syndrome Attacks.

    PubMed

    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

  12. 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.

  13. Hemolytic uremic syndrome with mild renal involvement due to Shiga toxin-producing Escherichia coli (STEC) O145 strain.

    PubMed

    Pérez, Lucía; Apezteguía, Lucía; Piñeyrúa, Cecilia; Dabezies, Agustín; Bianco, María N; Schelotto, Felipe; Varela, Gustavo

    2014-01-01

    Hemolytic uremic syndrome (HUS) is a disorder characterized by the presence of the classic triad: microangiopathic hemolytic anemia, thrombocytopenia and acute renal injury. HUS without acute renal failure can be confused with other hematologic diseases. An infantile HUS caused by a Shiga-toxin-producing Escherichia coli (STEC) O145 strain carrying genotype stx2, ehxA, eae subtype β1 is herein reported. The infant did not require dialysis during the acute stage of HUS, evolved favorably, maintained normal blood pressure and normal renal function and had no recurrence until the last control. This could be due to several factors, such as the characteristics of infecting STEC strain and a reduction in host susceptibility to renal injury. This report highlights the regional participation of non-O157 STEC in childhood diseases and the importance of performing active surveillance for all forms of HUS.

  14. 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

  15. ARCN1 Mutations Cause a Recognizable Craniofacial Syndrome Due to COPI-Mediated Transport Defects.

    PubMed

    Izumi, Kosuke; Brett, Maggie; Nishi, Eriko; Drunat, Séverine; Tan, Ee-Shien; Fujiki, Katsunori; Lebon, Sophie; Cham, Breana; Masuda, Koji; Arakawa, Michiko; Jacquinet, Adeline; Yamazumi, Yusuke; Chen, Shu-Ting; Verloes, Alain; Okada, Yuki; Katou, Yuki; Nakamura, Tomohiko; Akiyama, Tetsu; Gressens, Pierre; Foo, Roger; Passemard, Sandrine; Tan, Ene-Choo; El Ghouzzi, Vincent; Shirahige, Katsuhiko

    2016-08-01

    Cellular homeostasis is maintained by the highly organized cooperation of intracellular trafficking systems, including COPI, COPII, and clathrin complexes. COPI is a coatomer protein complex responsible for intracellular protein transport between the endoplasmic reticulum and the Golgi apparatus. The importance of such intracellular transport mechanisms is underscored by the various disorders, including skeletal disorders such as cranio-lenticulo-sutural dysplasia and osteogenesis imperfect, caused by mutations in the COPII coatomer complex. In this article, we report a clinically recognizable craniofacial disorder characterized by facial dysmorphisms, severe micrognathia, rhizomelic shortening, microcephalic dwarfism, and mild developmental delay due to loss-of-function heterozygous mutations in ARCN1, which encodes the coatomer subunit delta of COPI. ARCN1 mutant cell lines were revealed to have endoplasmic reticulum stress, suggesting the involvement of ER stress response in the pathogenesis of this disorder. Given that ARCN1 deficiency causes defective type I collagen transport, reduction of collagen secretion represents the likely mechanism underlying the skeletal phenotype that characterizes this condition. Our findings demonstrate the importance of COPI-mediated transport in human development, including skeletogenesis and brain growth. PMID:27476655

  16. Intermittent Fever, Progressive Weight Gain, and Personality Changes in a Five-Year-Old Girl: Unusual Paraneoplastic Syndrome due to Presacral Ganglioneuroma

    PubMed Central

    Yang, Chao; Li, Chang-chun; Zhang, Jun; Kong, Xiang-ru; Zhao, Zhenzhen; Deng, Xiao-bin; Peng, Liang; Wang, Shan

    2016-01-01

    Ganglioneuromas are rare tumors in the neuroblastoma group. Paraneoplastic syndrome (PNS) due to presacral ganglioneuromas was hardly reported in previous literature. Here, we reported that a case of a 5-year-old girl with a presacral ganglioneuroma presented with PNS, who presented with intermittent fever, progressive weight gain, and personality changes. Our report revealed intermittent fever, progressive weight gain, and personality changes may represent rare paraneoplastic syndromes in ganglioneuromas. PMID:27413558

  17. Japanese case of Budd-Chiari syndrome due to hepatic vein thrombosis successfully treated with liver transplantation.

    PubMed

    Iwasaki, Tomohiro; Kawai, Hirokazu; Oseki, Koushi; Togashi, Tadayuki; Shioji, Kazuhiko; Yamamoto, Satoshi; Sato, Yoshinobu; Suzuki, Kenji; Toba, Ken; Nomoto, Minoru; Hatakeyama, Katsuyoshi; Aoyagi, Yutaka

    2012-02-01

    A 22-year-old Japanese woman was found to have severe esophageal varices and then suffered from hepatic encephalopathy. She was diagnosed with Budd-Chiari syndrome (BCS) due to hepatic vein (HV) thrombosis accompanied by portal vein thrombosis without inferior vena cava (IVC) obstruction. Latent myeloproliferative neoplasm (MPN) lacking the JAK2-V617F mutation was considered to be the underlying disease. Liver transplantation was strikingly effective for treating the clinical symptoms attributable to portal hypertension. Although thrombosis of the internal jugular vein occurred due to thrombocythemia, which manifested after transplantation despite anticoagulation therapy with warfarin, the thrombus immediately disappeared with the addition of aspirin. Neither thrombosis nor BCS has recurred in more than 4 years since the amelioration of the last thrombotic event, and post-transplant immunosuppression with tacrolimus has not accelerated the progression of MPN. In Japan, IVC obstruction, which was a predominant type of BCS, is suggested to have decreased in incidence with recent improvements in hygiene. The precise diagnosis of BCS and causative underlying diseases should be made with attention to the current trend of the disease spectrum, which fluctuates with environmental sanitation levels. Because the stepwise strategy, including liver transplantation, has been proven effective for patients with pure HV obstruction in Western countries, this strategy should also be validated for utilization in Japan and in developing countries where HV obstruction potentially predominates.

  18. 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.

  19. 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

    Zeeshan, Muhammad; Ahmed, Farhan; Kanwal, Darakhshan; Khalid, Qazi Saad Bin; Ahmed, Muhammad Nadeem

    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

  20. Recombinant Human Erythropoietin Therapy for a Jehovah's Witness Child With Severe Anemia due to Hemolytic-Uremic Syndrome.

    PubMed

    Woo, Da Eun; Lee, Jae Min; Kim, Yu Kyung; Park, Yong Hoon

    2016-02-01

    Patients with hemolytic-uremic syndrome (HUS) can rapidly develop profound anemia as the disease progresses, as a consequence of red blood cell (RBC) hemolysis and inadequate erythropoietin synthesis. Therefore, RBC transfusion should be considered in HUS patients with severe anemia to avoid cardiac or pulmonary complications. Most patients who are Jehovah's Witnesses refuse blood transfusion, even in the face of life-threatening medical conditions due to their religious convictions. These patients require management alternatives to blood transfusions. Erythropoietin is a glycopeptide that enhances endogenous erythropoiesis in the bone marrow. With the availability of recombinant human erythropoietin (rHuEPO), several authors have reported its successful use in patients refusing blood transfusion. However, the optimal dose and duration of treatment with rHuEPO are not established. We report a case of a 2-year-old boy with diarrhea-associated HUS whose family members are Jehovah's Witnesses. He had severe anemia with acute kidney injury. His lowest hemoglobin level was 3.6 g/dL, but his parents refused treatment with packed RBC transfusion due to their religious beliefs. Therefore, we treated him with high-dose rHuEPO (300 IU/kg/day) as well as folic acid, vitamin B12, and intravenous iron. The hemoglobin level increased steadily to 7.4 g/dL after 10 days of treatment and his renal function improved without any complications. To our knowledge, this is the first case of successful rHuEPO treatment in a Jehovah's Witness child with severe anemia due to HUS. PMID:26958070

  1. Inherited 5p deletion syndrome due to paternal balanced translocation: Phenotypic heterogeneity due to duplication of 8q and 12p.

    PubMed

    Sharma, Pankaj; Gupta, Neerja; Chowdhury, Madhumita R; Sapra, Savita; Shukla, Rashmi; Lall, Meena; Kabra, Madhulika

    2013-09-01

    5p deletion syndrome or Cri du Chat syndrome is a autosomal deletion syndrome, caused by the de novo deletion of chromosome 5p in the majority of the cases. Clinical features include developmental delay, microcephaly, subtle facial dysmorphism and high-pitched cry. With the advent of newer techniques such as multiplex ligation-dependent probe amplification, rapid diagnosis is possible and chromosomal microarray helps in accurate delineation of the breakpoints. In this study, we characterized probands from two Indian families who had duplication of another chromosome in addition to deletion of 5p region. In the first family, two females of 3 and 5 yr of age had deletion of 5p15.33p15.2 (14.7 Mb) and duplication of 8q24.21q24.3 (15.4 Mb). Proband in the second family was a 2-year-old female and had deletion of 5p15.33p14.3 (22.55 Mb) along with duplication of 12p13.33p13.31 (7.7 Mb). In both the families, father was balanced translocation carrier of the chromosomes involved. Patients in family 1 had overwhelming features of 5p deletion while patient in family 2, besides having features of 5p deletion, showed many features of 12p duplications. Prenatal diagnosis was possible in both the families. To the best of our knowledge, this is the first detailed molecular cytogenetic analysis and prenatal diagnosis report of 5p deletion syndrome from India. PMID:27625854

  2. Inherited 5p deletion syndrome due to paternal balanced translocation: Phenotypic heterogeneity due to duplication of 8q and 12p

    PubMed Central

    Sharma, Pankaj; Gupta, Neerja; Chowdhury, Madhumita R.; Sapra, Savita; Shukla, Rashmi; Lall, Meena; Kabra, Madhulika

    2013-01-01

    5p deletion syndrome or Cri du Chat syndrome is a autosomal deletion syndrome, caused by the de novo deletion of chromosome 5p in the majority of the cases. Clinical features include developmental delay, microcephaly, subtle facial dysmorphism and high-pitched cry. With the advent of newer techniques such as multiplex ligation-dependent probe amplification, rapid diagnosis is possible and chromosomal microarray helps in accurate delineation of the breakpoints. In this study, we characterized probands from two Indian families who had duplication of another chromosome in addition to deletion of 5p region. In the first family, two females of 3 and 5 yr of age had deletion of 5p15.33p15.2 (14.7 Mb) and duplication of 8q24.21q24.3 (15.4 Mb). Proband in the second family was a 2-year-old female and had deletion of 5p15.33p14.3 (22.55 Mb) along with duplication of 12p13.33p13.31 (7.7 Mb). In both the families, father was balanced translocation carrier of the chromosomes involved. Patients in family 1 had overwhelming features of 5p deletion while patient in family 2, besides having features of 5p deletion, showed many features of 12p duplications. Prenatal diagnosis was possible in both the families. To the best of our knowledge, this is the first detailed molecular cytogenetic analysis and prenatal diagnosis report of 5p deletion syndrome from India. PMID:27625854

  3. 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.

  4. Abnormal neutrophil-pulmonary interaction in the adult respiratory distress syndrome. Qualitative and quantitative assessment of pulmonary neutrophil kinetics in humans with in vivo /sup 111/indium neutrophil scintigraphy

    SciTech Connect

    Warshawski, F.J.; Sibbald, W.J.; Driedger, A.A.; Cheung, H.

    1986-05-01

    In the absence of direct toxins, the majority of evidence from animal models suggests that neutrophils (PMN) are necessary for the full expression of the abnormal pulmonary permeability accompanying acute microvascular lung injury. We therefore studied the role of the PMN in the human correlate of this disease, the adult respiratory distress syndrome (ARDS), by assessing the pulmonary retention of infused autologous /sup 111/Indium-labeled PMN (PMN-In). We evaluated 79 patients, prospectively categorized as: active ARDS (Aa; n = 30), active ARDS and concurrent corticosteroid therapy (As; n = 11), resolving ARDS (Ar; n = 13), sepsis without pulmonary edema (S; n = 7), and cardiac pulmonary edema (C; n = 18). This clinical separation was confirmed by retrospective analysis of associated measures of hemodynamic and respiratory dysfunction. We found that both analog scintigrams (positive/negative for diffuse pulmonary PMN-In sequestration) and computer-assisted quantitative analysis in 46 patients (T 1/2 of first hour demargination and percentage of peak activity/pixel/second remaining at 17 to 20 h) showed a significant rank order decrease in the pulmonary retention of labeled PMN-In through the Groups Aa----As----S----Ar----C. Our findings recognized aspects of in vivo PMN-In behavior that implied pathophysiologic differences between groups of critically ill patients in either the PMN themselves or in PMN-pulmonary endothelial interaction. This demonstrates the possibility of abnormal in vivo PMN-endothelial interaction in ARDS by virtue of the greater pulmonary localization of PMN in active ARDS versus resolving disease, septic non-ARDS states, and cardiac pulmonary edema.

  5. Analysis and comparison of the effects of N-BiPAP and Bubble-CPAP in treatment of preterm newborns with the weight of below 1500 grams affiliated with respiratory distress syndrome: A randomised clinical trial

    PubMed Central

    Sadeghnia, Alireza; Barekateyn, Behzad; Badiei, Zohre; Hosseini, Seyyed Mohsen

    2016-01-01

    Background: Nowadays, establishment of nCPAP and surfactant administration is considered to be the first level of intervention for newborns engaged in the process of Respiratory Distress Syndrome (RDS). In order to decrease the side effects of the nCPAP management placed in noninvasive-non-cycled respiratory support. Noninvasive-cycled respiratory support mechanism have been developed such as N-BiPAP. Therefore, we compared N-BiPAP with Bubble-CPAP in a clinical trial. Materials and Methods: This research was done as an on newborns weighing less than 1500 grams affiliated with RDS. A3 The total number of newborns was 70. Newborns were divided into two groups with the sample size of 35 patients in each, according to odd and even document numbers. One group was treated with N-BiPAP and the other with Bubble-CPAP. Patients were compared according to the length of treatment with noninvasive respiratory support, length of oxygen intake, number of surfactant doses administered, need for invasive mechanical ventilation, apnea, patent ductus arteriosus (PDA), chronic lung disease, intraventricular hemorrhage, pneumothorax, and death. Data was recorded and compared. Results: The average duration for noninvasive respiratory support and the average time of need to complementary oxygen was not significantly different in both groups (P value > 0.05). Need for invasive ventilation, also chronic lung disease, intraventricular hemorrhage (IVH), pneumothorax, need for the next dose of surfactant, and the death rate did also have no meaningful difference. (P value > 0.05). Conclusion: In this research N-BiPAP did not show any obvious clinical preference over the Bubble-CPAP in treatment of newborns weighing less than 1500 grams and affiliated with RDS. PMID:26955624

  6. 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.

  7. 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.

  8. 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. PMID:26855510

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

    PubMed

    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

    2015-10-01

    Bilateral adrenalectomy is currently the only available treatment for adrenocorticotropic hormone (ACTH)-dependent Cushing's syndrome (ectopic ACTH syndrome) that is refractory to pharmacologic therapy. We describe two patients with refractory ectopic ACTH syndrome who were treated with CT-guided percutaneous microwave ablation of both hyperplastic adrenal glands in a single session: O ne 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.

  10. 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.

  11. 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.

  12. 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. PMID:27311559

  13. 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

  14. A girl with permanent neonatal diabetes due to KCNJ11 mutation presented with Mauriac syndrome after improper adjustment in sulfonylurea dosage over 6 years.

    PubMed

    Chai-Udom, Rapeepun; Sahakitrungruang, Taninee; Wacharasindhu, Suttipong; Supornsilchai, Vichit

    2016-09-01

    Mauriac syndrome is characterized by growth impairment, Cushingoid features, and hepatomegaly in patients with poorly controlled type 1 diabetes mellitus (T1DM). We report a novel presentation of Mauriac syndrome in a 9-year-old girl who was diagnosed with neonatal diabetes at 3 months of age due to the p.R201C mutation in KCNJ11. She was initially treated successfully with glipizide at a dose of 0.85 mg/kg/day but after being lost to follow-up and having improper adjustment in dose over many years, the recent dose of 0.6 mg/kg/day appears to have been insufficient for glycemic control but enough to maintain a low level of C-peptide and prevent diabetic ketoacidosis. With proper insulin administration, all presenting clinical characteristics were resolved within 1 month. A review of the literature relating to clinical manifestations of Mauriac syndrome in children with diabetes was performed and included in this report for comparison with our patient. While Mauriac syndrome has been traditionally associated with T1DM, the presence of Mauriac syndrome should not be excluded in other types of diabetes mellitus.

  15. Assessing Racial Microaggression Distress in a Diverse Sample.

    PubMed

    Torres-Harding, Susan; Turner, Tasha

    2015-12-01

    Racial microaggressions are everyday subtle or ambiguous racially related insults, slights, mistreatment, or invalidations. Racial microaggressions are a type of perceived racism that may negatively impact the health and well-being of people of color in the United States. This study examined the reliability and validity of the Racial Microaggression Scale distress subscales, which measure the perceived stressfulness of six types of microaggression experiences in a racially and ethnically diverse sample. These subscales exhibited acceptable to good internal consistency. The distress subscales also evidenced good convergent validity; the distress subscales were positively correlated with additional measures of stressfulness due to experiencing microaggressions or everyday discrimination. When controlling for the frequency of one's exposure to microaggression incidents, some racial/ethnic group differences were found. Asian Americans reported comparatively lower distress and Latinos reporting comparatively higher distress in response to Foreigner, Low-Achieving, Invisibility, and Environmental microaggressions. African Americans reported higher distress than the other groups in response to Environmental microaggressions. Results suggest that the Racial Microaggressions Scale distress subscales may aid health professionals in assessing the distress elicited by different types of microaggressions. In turn, this may facilitate diagnosis and treatment planning in order to provide multiculturally competent care for African American, Latino, and Asian American clients.

  16. Assessing Racial Microaggression Distress in a Diverse Sample.

    PubMed

    Torres-Harding, Susan; Turner, Tasha

    2015-12-01

    Racial microaggressions are everyday subtle or ambiguous racially related insults, slights, mistreatment, or invalidations. Racial microaggressions are a type of perceived racism that may negatively impact the health and well-being of people of color in the United States. This study examined the reliability and validity of the Racial Microaggression Scale distress subscales, which measure the perceived stressfulness of six types of microaggression experiences in a racially and ethnically diverse sample. These subscales exhibited acceptable to good internal consistency. The distress subscales also evidenced good convergent validity; the distress subscales were positively correlated with additional measures of stressfulness due to experiencing microaggressions or everyday discrimination. When controlling for the frequency of one's exposure to microaggression incidents, some racial/ethnic group differences were found. Asian Americans reported comparatively lower distress and Latinos reporting comparatively higher distress in response to Foreigner, Low-Achieving, Invisibility, and Environmental microaggressions. African Americans reported higher distress than the other groups in response to Environmental microaggressions. Results suggest that the Racial Microaggressions Scale distress subscales may aid health professionals in assessing the distress elicited by different types of microaggressions. In turn, this may facilitate diagnosis and treatment planning in order to provide multiculturally competent care for African American, Latino, and Asian American clients. PMID:25237154

  17. NAA10 mutation causing a novel intellectual disability syndrome with Long QT due to N-terminal acetyltransferase impairment

    PubMed Central

    Casey, Jillian P.; Støve, Svein I.; McGorrian, Catherine; Galvin, Joseph; Blenski, Marina; Dunne, Aimee; Ennis, Sean; Brett, Francesca; King, Mary D.; Arnesen, Thomas; Lynch, Sally Ann

    2015-01-01

    We report two brothers from a non-consanguineous Irish family presenting with a novel syndrome characterised by intellectual disability, facial dysmorphism, scoliosis and long QT. Their mother has a milder phenotype including long QT. X-linked inheritance was suspected. Whole exome sequencing identified a novel missense variant (c.128 A > C; p.Tyr43Ser) in NAA10 (X chromosome) as the cause of the family’s disorder. Sanger sequencing confirmed that the mutation arose de novo in the carrier mother. NAA10 encodes the catalytic subunit of the major human N-terminal acetylation complex NatA. In vitro assays for the p.Tyr43Ser mutant enzyme showed a significant decrease in catalytic activity and reduced stability compared to wild-type Naa10 protein. NAA10 has previously been associated with Ogden syndrome, Lenz microphthalmia syndrome and non-syndromic developmental delay. Our findings expand the clinical spectrum of NAA10 and suggest that the proposed correlation between mutant Naa10 enzyme activity and phenotype severity is more complex than anticipated; the p.Tyr43Ser mutant enzyme has less catalytic activity than the p.Ser37Pro mutant associated with lethal Ogden syndrome but results in a milder phenotype. Importantly, we highlight the need for cardiac assessment in males and females with NAA10 variants as both patients and carriers can have long QT. PMID:26522270

  18. NAA10 mutation causing a novel intellectual disability syndrome with Long QT due to N-terminal acetyltransferase impairment.

    PubMed

    Casey, Jillian P; Støve, Svein I; McGorrian, Catherine; Galvin, Joseph; Blenski, Marina; Dunne, Aimee; Ennis, Sean; Brett, Francesca; King, Mary D; Arnesen, Thomas; Lynch, Sally Ann

    2015-01-01

    We report two brothers from a non-consanguineous Irish family presenting with a novel syndrome characterised by intellectual disability, facial dysmorphism, scoliosis and long QT. Their mother has a milder phenotype including long QT. X-linked inheritance was suspected. Whole exome sequencing identified a novel missense variant (c.128 A > C; p.Tyr43Ser) in NAA10 (X chromosome) as the cause of the family's disorder. Sanger sequencing confirmed that the mutation arose de novo in the carrier mother. NAA10 encodes the catalytic subunit of the major human N-terminal acetylation complex NatA. In vitro assays for the p.Tyr43Ser mutant enzyme showed a significant decrease in catalytic activity and reduced stability compared to wild-type Naa10 protein. NAA10 has previously been associated with Ogden syndrome, Lenz microphthalmia syndrome and non-syndromic developmental delay. Our findings expand the clinical spectrum of NAA10 and suggest that the proposed correlation between mutant Naa10 enzyme activity and phenotype severity is more complex than anticipated; the p.Tyr43Ser mutant enzyme has less catalytic activity than the p.Ser37Pro mutant associated with lethal Ogden syndrome but results in a milder phenotype. Importantly, we highlight the need for cardiac assessment in males and females with NAA10 variants as both patients and carriers can have long QT. PMID:26522270

  19. NAA10 mutation causing a novel intellectual disability syndrome with Long QT due to N-terminal acetyltransferase impairment.

    PubMed

    Casey, Jillian P; Støve, Svein I; McGorrian, Catherine; Galvin, Joseph; Blenski, Marina; Dunne, Aimee; Ennis, Sean; Brett, Francesca; King, Mary D; Arnesen, Thomas; Lynch, Sally Ann

    2015-11-02

    We report two brothers from a non-consanguineous Irish family presenting with a novel syndrome characterised by intellectual disability, facial dysmorphism, scoliosis and long QT. Their mother has a milder phenotype including long QT. X-linked inheritance was suspected. Whole exome sequencing identified a novel missense variant (c.128 A > C; p.Tyr43Ser) in NAA10 (X chromosome) as the cause of the family's disorder. Sanger sequencing confirmed that the mutation arose de novo in the carrier mother. NAA10 encodes the catalytic subunit of the major human N-terminal acetylation complex NatA. In vitro assays for the p.Tyr43Ser mutant enzyme showed a significant decrease in catalytic activity and reduced stability compared to wild-type Naa10 protein. NAA10 has previously been associated with Ogden syndrome, Lenz microphthalmia syndrome and non-syndromic developmental delay. Our findings expand the clinical spectrum of NAA10 and suggest that the proposed correlation between mutant Naa10 enzyme activity and phenotype severity is more complex than anticipated; the p.Tyr43Ser mutant enzyme has less catalytic activity than the p.Ser37Pro mutant associated with lethal Ogden syndrome but results in a milder phenotype. Importantly, we highlight the need for cardiac assessment in males and females with NAA10 variants as both patients and carriers can have long QT.

  20. 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

  1. 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

  2. Stickler-like syndrome due to a dominant negative mutation in the COL2A1 gene.

    PubMed

    Ballo, R; Beighton, P H; Ramesar, R S

    1998-10-30

    The type II collagenopathies include a wide spectrum of phenotypes ranging from mild spondylo epiphyseal dysplasia (SED) to severe achondrogenesis/hypochondrogenesis. Several attempts have been made at providing phenotype-genotype correlations in this group of disorders. In this report we discuss a South African family in which four members have a phenotype resembling Stickler syndrome type 1. Ocular problems and conductive deafness predominate, while skeletal changes resemble those of a mild form of multiple epiphyseal dysplasia (MED). In distinction to the classical form of Stickler syndrome, the affected persons have stubby digits. DNA analysis of the exons of the COL2A1 gene documented a C-T transversion in exon 39, resulting in an Arg704Cys substitution in the triple helical domain of the type II collagen peptide; this nontermination mutation may be indicative of further heterogeneity in the Stickler group of disorders or of a new syndrome amongst the type II collagenopathies. PMID:9800905

  3. 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

  4. 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

  5. Regulatory effect of cytokine-induced neutrophil chemoattractant, epithelial neutrophil-activating peptide 78 and pyrrolidine dithiocarbamate on pulmonary neutrophil aggregation mediated by nuclear factor-κB in lipopolysaccharide-induced acute respiratory distress syndrome mice

    PubMed Central

    Wang, Hongman; Zhao, Jiping; Xue, Guansheng; Wang, Junfei; Wu, Jinxiang; Wang, Donghui; Dong, Liang

    2016-01-01

    In the present study, the regulatory effect of cytokine-induced neutrophil chemoattractant (CINC) and epithelial neutrophil-activating peptide 78 (ENA-78) on pulmonary neutrophil (PMN) accumulation in lipopolysaccharide (LPS)-induced acute respiratory distress syndrome (ARDS) mice, and the therapeutic effect of pyrrolidine dithiocarbamate (PDTC), was investigated. BALB/c mice were divided into control, LPS and PDTC + LPS groups using a random number table. The phosphorylation of nuclear factor-κB (NF-κB) was detected using a western blot, and the mRNA expression levels of CINC were evaluated using reverse transcription-quantitative polymerase chain reaction. The expression of NF-κB, CINC and ENA-78 was detected using immunohistochemistry. The production of interleukin (IL)-8 and IL-10 in serum and broncho-alveolar lavage fluid (BALF) was analyzed using an enzyme-linked immunosorbent assay. The total number of leukocytes and proportion of PMNs in BALF was also determined. Following injection with LPS (20 mg/kg), the expression levels of p-NF-κB, CINC and ENA-78 were increased in lung tissue, and the expression levels of IL-8, IL-10 and the number of PMNs increased in serum and BALF. However, in comparison with the LPS group, the degree of lung injury was reduced in ARDS mice that were treated with PDTC. In addition, the expression level of p-NF-κB and the production of chemokines in lung tissue decreased in ARDS mice that were treated with PDTC, and the number of PMNs in BALF also decreased. In conclusion, the results of the present study suggest that the LPS-induced phosphorylation of NF-κB may result in the synthesis and release of CINC and ENA-78, which induce the accumulation of PMNs in the lung. Therefore, PDTC may be used to reduce the production of chemokines and cytokines, thereby decreasing the activation of PMNs in lung tissue and reducing the damage of lung tissue in ARDS. PMID:27602092

  6. Regulatory effect of cytokine-induced neutrophil chemoattractant, epithelial neutrophil-activating peptide 78 and pyrrolidine dithiocarbamate on pulmonary neutrophil aggregation mediated by nuclear factor-κB in lipopolysaccharide-induced acute respiratory distress syndrome mice

    PubMed Central

    Wang, Hongman; Zhao, Jiping; Xue, Guansheng; Wang, Junfei; Wu, Jinxiang; Wang, Donghui; Dong, Liang

    2016-01-01

    In the present study, the regulatory effect of cytokine-induced neutrophil chemoattractant (CINC) and epithelial neutrophil-activating peptide 78 (ENA-78) on pulmonary neutrophil (PMN) accumulation in lipopolysaccharide (LPS)-induced acute respiratory distress syndrome (ARDS) mice, and the therapeutic effect of pyrrolidine dithiocarbamate (PDTC), was investigated. BALB/c mice were divided into control, LPS and PDTC + LPS groups using a random number table. The phosphorylation of nuclear factor-κB (NF-κB) was detected using a western blot, and the mRNA expression levels of CINC were evaluated using reverse transcription-quantitative polymerase chain reaction. The expression of NF-κB, CINC and ENA-78 was detected using immunohistochemistry. The production of interleukin (IL)-8 and IL-10 in serum and broncho-alveolar lavage fluid (BALF) was analyzed using an enzyme-linked immunosorbent assay. The total number of leukocytes and proportion of PMNs in BALF was also determined. Following injection with LPS (20 mg/kg), the expression levels of p-NF-κB, CINC and ENA-78 were increased in lung tissue, and the expression levels of IL-8, IL-10 and the number of PMNs increased in serum and BALF. However, in comparison with the LPS group, the degree of lung injury was reduced in ARDS mice that were treated with PDTC. In addition, the expression level of p-NF-κB and the production of chemokines in lung tissue decreased in ARDS mice that were treated with PDTC, and the number of PMNs in BALF also decreased. In conclusion, the results of the present study suggest that the LPS-induced phosphorylation of NF-κB may result in the synthesis and release of CINC and ENA-78, which induce the accumulation of PMNs in the lung. Therefore, PDTC may be used to reduce the production of chemokines and cytokines, thereby decreasing the activation of PMNs in lung tissue and reducing the damage of lung tissue in ARDS.

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

    PubMed Central

    2012-01-01

    Introduction Acute lung injury (ALI)/acute respiratory distress syndrome (ARDS) is characterized by features other than increased pulmonary vascular permeability. Pulmonary vascular permeability combined with increased extravascular lung water content has been considered a quantitative diagnostic criterion of ALI/ARDS. This prospective, multi-institutional, observational study aimed to clarify the clinical pathophysiological features of ALI/ARDS and establish its quantitative diagnostic criteria. Methods The extravascular lung water index (EVLWI) and the pulmonary vascular permeability index (PVPI) were measured using the transpulmonary thermodilution method in 266 patients with PaO2/FiO2 ratio ≤ 300 mmHg and bilateral infiltration on chest radiography, in 23 ICUs of academic tertiary referral hospitals. Pulmonary edema was defined as EVLWI ≥ 10 ml/kg. Three experts retrospectively determined the pathophysiological features of respiratory insufficiency by considering the patients' history, clinical presentation, chest computed tomography and radiography, echocardiography, EVLWI and brain natriuretic peptide level, and the time course of all preceding findings under systemic and respiratory therapy. Results Patients were divided into the following three categories on the basis of the pathophysiological diagnostic differentiation of respiratory insufficiency: ALI/ARDS, cardiogenic edema, and pleural effusion with atelectasis, which were noted in 207 patients, 26 patients, and 33 patients, respectively. EVLWI was greater in ALI/ARDS and cardiogenic edema patients than in patients with pleural effusion with atelectasis (18.5 ± 6.8, 14.4 ± 4.0, and 8.3 ± 2.1, respectively; P < 0.01). PVPI was higher in ALI/ARDS patients than in cardiogenic edema or pleural effusion with atelectasis patients (3.2 ± 1.4, 2.0 ± 0.8, and 1.6 ± 0.5; P < 0.01). In ALI/ARDS patients, EVLWI increased with increasing pulmonary vascular permeability (r = 0.729, P < 0.01) and was weakly

  8. The effectiveness and applicability of different lifestyle interventions for enhancing wellbeing: the study design for a randomized controlled trial for persons with metabolic syndrome risk factors and psychological distress

    PubMed Central

    2014-01-01

    Background Obesity and stress are among the most common lifestyle-related health problems. Most of the current disease prevention and management models are not satisfactorily cost-effective and hardly reach those who need them the most. Therefore, novel evidence-based controlled interventions are necessary to evaluate models for prevention and treatment based on self-management. This randomized controlled trial examines the effectiveness, applicability, and acceptability of different lifestyle interventions with individuals having symptoms of metabolic syndrome and psychological distress. The offered interventions are based on cognitive behavioral approaches, and are designed for enhancing general well-being and supporting personalized lifestyle changes. Methods/Design 339 obese individuals reporting stress symptoms were recruited and randomized to either (1) a minimal contact web-guided Cognitive Behavioral Therapy-based (CBT) intervention including an approach of health assessment and coaching methods, (2) a mobile-guided intervention comprising of mindfulness, acceptance and value-based exercises, (3) a face-to-face group intervention using mindfulness, acceptance and value-based approach, or (4) a control group. The participants were measured three times during the study (pre = week 0, post = week 10, and follow-up = week 36). Psychological well-being, lifestyles and habits, eating behaviors, and user experiences were measured using online surveys. Laboratory measurements for physical well-being and general health were performed including e.g. liver function, thyroid glands, kidney function, blood lipids and glucose levels and body composition analysis. In addition, a 3-day ambulatory heart rate and 7-day movement data were collected for analyzing stress, recovery, physical activity, and sleep patterns. Food intake data were collected with a 48 -hour diet recall interview via telephone. Differences in the effects of the interventions would be

  9. [Congenital ectropion of the upper eyelids due to an anomaly of the eyelids in down's syndrome (author's transl)].

    PubMed

    Hennighausen, U; Schmidt-Martens, F W; Reim, M

    1978-05-01

    A 5-months-old female baby with Down's Syndrome developed an intermittent spastic ectropion of the upper eyelids. The reasons for this are thought to be the flaccidity of the connective tissue, which is typical in Down's Syndrome, and a little anomaly of the eyelids, the tarsus was too short horizontally and very weak and the upper eyelids were somewhat larger than normal and elongated. Suturing Bangerter's lid-sheets on the upper eyelids for 15 days resulted in a scarring of the tarsus with the lax connective tissue of the upper eyelids. The ectropion disappeared and did not recur.

  10. 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…

  11. Non-syndromic hereditary gingival fibromatosis in three Chinese families is not due to SOS1 gene mutations.

    PubMed

    Ma, Yanyan; Sun, Zheng; Hu, Ying; Liu, Yi; Jin, Lingling; Zhang, Fenqiu

    2014-12-01

    Non-syndromic hereditary gingival fibromatosis (HGF) is a rare condition, characterized by a progressive gingival hyperplasia that occurs as an isolated disease. Hitherto, only one insertion (g.126,142-126,143insC) in son-of-sevenless-1 (SOS1) gene has been associated with non-syndromic HGF in a Brazilian family. The aim of the present study was to determine if SOS1 is the causative gene of non-syndromic HGF in the Chinese population. Peripheral blood samples were collected from six affected and seven unaffected individuals from three Chinese families with history of non-syndromic HGF. Genomic DNA was extracted and SOS1 gene exons were sequenced. Neither g.126,142-126,143insC nor any other novel mutation was detected in SOS1 gene. Our results suggest that the SOS1 may not be the gene responsible for HGF in these three Chinese families and, therefore, it is possible that other genes are involved in the manifestation of HGF in these Chinese HGF families.

  12. How Is Respiratory Distress Syndrome Treated?

    MedlinePlus

    ... build up in the babies' lungs. Treatment for Patent Ductus Arteriosus PDA is a possible complication of ... For more information, go to the Health Topics Patent Ductus Arteriosus article. Rate This Content: NEXT >> Updated: ...

  13. What Is Acute Respiratory Distress Syndrome?

    MedlinePlus

    ... treated in the intensive care unit (ICU) or critical care unit (CCU) of a hospital. Therapies commonly used ... may be done. (See ATS fact sheet on Tracheostomy). What is an ICU and what can I ...

  14. Respiratory distress of the newborn: congenital laryngeal atresia.

    PubMed

    Ambrosio, Art; Magit, Anthony

    2012-11-01

    Congenital laryngeal atresia is a rare cause of respiratory distress of the newborn. The defect may be isolated or occur in association with other congenital abnormalities, notably the presence of a tracheoesophageal fistula, esophageal atresia, encephalocele, or Congenital High Airway Obstructive Syndrome (CHAOS). We present the case of a newborn with no identified intrapartum abnormalities with respiratory distress at birth secondary to near-complete laryngeal atresia. Management included tracheostomy, repeated endoscopic incisions, and serial balloon dilatations employing the topical use of Mitomycin C. Seven year follow-up was significant for mobilization of the true vocal cords bilaterally, as well as successful decannulation.

  15. Early-Lethal Costello syndrome due to rare HRAS tandem base substitution (c.35_36GC>AA; p.G12E) associated pulmonary vascular disease

    PubMed Central

    Nicole Weaver, K.; Wang, Dehua; Cnota, James; Gardner, Nicholas; Stabley, Deborah; Sol-Church, Katia; Gripp, Karen W.; Witte, David P.; Bove, Kevin E.; Hopkin, Robert J.

    2014-01-01

    Costello syndrome is a rare, autosomal dominant syndrome caused by activating missense mutations in the Harvey rat sarcoma viral oncogene homolog (HRAS), most often p.G12S. Several rare mutations have consistently been associated with a more severe phenotype which is often lethal in infancy. Cause of death is most often respiratory failure with hypertrophic cardiomyopathy playing a significant role in morbidity. Impaired fibroblast elastogenesis is thought to contribute to the Costello phenotype, but reports of histologic evidence of disordered elastogenesis at autopsy are limited. We report a patient with Costello syndrome due to a rare tandem base substitution (c.35_36GC>AA) resulting in the p.G12E missense change. The proband died at age 3 months from respiratory failure, with minimal evidence for cardiomyopathy. Autopsy disclosed pulmonary vascular dysplasia affecting small arteries and veins associated with abnormal elastin distribution in tortuous dilated arteries and veins, with non-uniform wall thickness and semi-obstructive lesions at artery branch points typical of early pulmonary hypertensive vascular disease. Elastic fibers in the dermis were abnormally short and fragmented. This case suggests that disordered elastogenesis in the pulmonary vasculature and undiagnosed (or underdiagnosed) pulmonary hypertension may contribute to morbidity in patients with Costello syndrome. PMID:25133308

  16. Burning Mouth Syndrome and "Burning Mouth Syndrome".

    PubMed

    Rifkind, Jacob Bernard

    2016-03-01

    Burning mouth syndrome is distressing to both the patient and practitioner unable to determine the cause of the patient's symptoms. Burning mouth syndrome is a diagnosis of exclusion, which is used only after nutritional deficiencies, mucosal disease, fungal infections, hormonal disturbances and contact stomatitis have been ruled out. This article will explore the many causes and treatment of patients who present with a chief complaint of "my mouth burns," including symptomatic treatment for those with burning mouth syndrome. PMID:27209717

  17. Burning Mouth Syndrome and "Burning Mouth Syndrome".

    PubMed

    Rifkind, Jacob Bernard

    2016-03-01

    Burning mouth syndrome is distressing to both the patient and practitioner unable to determine the cause of the patient's symptoms. Burning mouth syndrome is a diagnosis of exclusion, which is used only after nutritional deficiencies, mucosal disease, fungal infections, hormonal disturbances and contact stomatitis have been ruled out. This article will explore the many causes and treatment of patients who present with a chief complaint of "my mouth burns," including symptomatic treatment for those with burning mouth syndrome.

  18. Organic arsenic-induced Guillain-Barré-like syndrome due to melarsoprol: a clinical, electrophysiological, and pathological study.

    PubMed

    Gherardi, R K; Chariot, P; Vanderstigel, M; Malapert, D; Verroust, J; Astier, A; Brun-Buisson, C; Schaeffer, A

    1990-07-01

    A young woman suffering from sleeping sickness was treated with melarsoprol. Thirty-eight days after the first administration of this organo-arsenic compound, myalgias, distal paresthesias and rapidly progressive weakness developed in all four limbs. Electrophysiological studies were misleading for Guillain-Barré syndrome. Neuropathological data included massive distal wallerian degeneration in peripheral nerves and abnormalities in dorsal ganglia and spinal cord where vacuolation of anterior horn cells and axonal neurofilamentous masses were observed. Very high concentrations of arsenic were found in the spinal cord, contrasting with undetectable levels in peripheral nerves. Our findings are consistent with an arsenic neuronopathy manifested by initial proximal demyelination and delayed distal axonal damage. Renal and hepatic dysfunctions, which were implicated in the toxic arsenic accumulation, should be systematically detected before administration of melarsoprol. The diagnosis of Guillain-Barré syndrome must be considered with caution in patients treated with this compound.

  19. Börjeson-Forssman-Lehmann Syndrome due to a novel plant homeodomain zinc finger mutation in the PHF6 gene.

    PubMed

    Mangelsdorf, Marie; Chevrier, Evelyne; Mustonen, Aki; Picketts, David J

    2009-05-01

    The Börjeson-Forssman-Lehmann syndrome is an X-linked mental retardation disorder caused by mutations in the PHF6 gene. The PHF6 gene contains 2 plant homeodomain zinc fingers, suggesting a role for the protein in chromatin remodeling. In this study, the authors report on a Finnish family with a classical Börjeson-Forssman-Lehmann syndrome phenotype caused by a G to T nucleotide substitution at position 266 within exon 4 within the PHF6 gene (c.266G>T). The resulting glycine to valine (p.G89V) change corresponds to a highly conserved residue within the first plant homeodomain zinc finger domain. This is a novel change that adds to the number of plant homeodomain zinc finger mutations identified, such that 23% of all Börjeson-Forssman-Lehmann syndrome mutations lie within this motif. Moreover, it highlights the functional importance of plant homeodomain zinc finger motifs to human disease and more specifically to PHF6 function.

  20. Ichthyosis in Sjögren-Larsson syndrome reflects defective barrier function due to abnormal lamellar body structure and secretion.

    PubMed

    Rizzo, William B; S'Aulis, Dana; Jennings, M Anitia; Crumrine, Debra A; Williams, Mary L; Elias, Peter M

    2010-08-01

    Sjögren-Larsson syndrome is a genetic disease characterized by ichthyosis, mental retardation, spasticity and mutations in the ALDH3A2 gene coding for fatty aldehyde dehydrogenase, an enzyme necessary for oxidation of fatty aldehydes and fatty alcohols. We investigated the cutaneous abnormalities in 9 patients with Sjögren-Larsson syndrome to better understand how the enzymatic deficiency results in epidermal dysfunction. Histochemical staining for aldehyde oxidizing activity was profoundly reduced in the epidermis. Colloidal lanthanum perfusion studies showed abnormal movement of tracer into the extracellular spaces of the stratum corneum consistent with a leaky water barrier. The barrier defect could be attributed to the presence of abnormal lamellar bodies, many with disrupted limiting membranes or lacking lamellar contents. Entombed lamellar bodies were present in the cytoplasm of corneocytes suggesting blockade of lamellar body secretion. At the stratum granulosum-stratum corneum interface, non-lamellar material displaced or replaced secreted lamellar membranes, and in the stratum corneum, the number of lamellar bilayers declined and lamellar membrane organization was disrupted by foci of lamellar/non-lamellar phase separation. These studies demonstrate the presence of a permeability barrier abnormality in Sjögren-Larsson syndrome, which localizes to the stratum corneum interstices and can be attributed to abnormalities in lamellar body formation and secretion.