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  • Alagille syndrome  (1)
  • Arteriohepatic dysplasia  (1)
  • Chest wall mechanics  (1)
  • Corticosterone methyl oxidase type II  (1)
  • Ductopenia  (1)
  • Endotracheal tubes  (1)
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Keywords
  • 1
    ISSN: 1432-1238
    Keywords: Muscle relaxants ; Sedation ; Respiratory system mechanics ; Chest wall mechanics ; Mechanical ventilation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Objective To evaluate the separate effects of sedation and paralysis on chest wall and respiratory system mechanics of mechanically ventilated, critically ill patients.Setting: ICU of the University “La Sapienza” Hospital, Rome. Patients and participants 13 critically ill patients were enrolled in this study. All were affected by disease involving both lungs and chest wall mechanics (ARDS in 4 patients, closed chest trauma without flail chest in 4 patients, cardiogenic pulmonary oedema with fluidic overload in 5 patients). Measurements and results Respiratory system and chest wall mechanics were evaluated during constant flow controlled mechanical ventilation in basal conditions (i. e. with the patients under apnoic sedation) and after paralysis with pancuronium bromide. In details, we simultaneously recorded airflow, tracheal pressure, esophageal pressure and tidal volume; with the end-inspiratory and end-expiratory airway occlusion technique we could evaluate respiratory system and chest wall elastance and resistances. Lung mechanics was evaluated by subtracting chest wall from respiratory system data. All data obtained in basal conditions (with the patients sedated with thiopental or propofol) and after muscle paralysis were compared using the Student'st test for paired data. The administration of pancuronium bromide to sedated patients induced a complete muscle paralysis without producing significant modification both to the viscoelastic and to the resistive parameters of chest wall and respiratory system. Conclusions This study demonstrates the lack of additive effects of muscle paralysis in mechanically ventilated, sedated patients. Also in view of the possible side effects of muscle paralysis, our results question the usefulness of generalyzed administration of neuromuscular blocking drugs in mechanically ventilated patients.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-1238
    Keywords: Mechanical ventilation ; Endotracheal tubes ; Obstruction ; Acute respiratory failure
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Objective To evaluate the efficiency of a new device developed to remove obstructions from endotracheal tubes (ETT) in mechanically ventilated patients. Design Open study in mechanically ventilated sedated and paralyzed ICU patients. Setting General ICU and Laboratory of Respiratory Mechanics of the University of Rome “La Sapienza”. Patients 8 consecutive unselected mechanically ventilated, critically ill patients in which a partial obstruction of ETT was suspected on the basis of an increase of the peak inspiratory pressure (〉20%) plus the difficult introduction of a standard suction catheter. Interventions Obstructions to ETT were removed with an experimental “obstruction remover” (OR) Measurements “In vivo” ETT airflow resistance (0.25; 0.5; 0.75; 1l/s) was evaluated before and after use of the OR; the work of breathing necessary to overcome ETT resistance (WOBett) was also evaluated before and after OR use. Results The use of OR significantly reduced in all patients the ETT “in vivo” resistance (From 5.5±2.3 to 2.9±0.5 cmH2O/l/s at 0.25l/s,p〈0.05; from 9±2.4 to 3.8±0.8 cmH2O/l/s at 0.51l/s; from 12.2±3.5 to 5.7±1.2 cmH2O/l/s at 0.75l/s; from 16.9±6 to 9.3±3.8 cmH2O/l/s at 1l/s,p〈0.01 respectively). Also the WOBett was significantly reduced after use of the OR (from 0.66±0.19 to 0.34±0.08 J/l;p〈0.05) Conclusion This experimental device can be safely and successfully used to remove obstructions from the ETT lumen, without suspending mechanical ventilation, reducing the need for rapid ETT substitution in emergency and life-threatening situations.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric nephrology 6 (1992), S. 559-561 
    ISSN: 1432-198X
    Keywords: Alagille syndrome ; Arteriohepatic dysplasia ; IgA nephritis ; Liver transplantation ; Cyclosporine A
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Alagille syndrome (arteriohepatic dysplasia) is a major cause of intrahepatic cholestasis in infancy. The present report describes a patient with Alagille syndrome who presented with hematuria and IgA nephritis 7 years after an orthotopic liver transplantation and immunosuppression. This patient suggests that glomerular lipidosis is not an inherent feature of the Alagille syndrome, and that IgA nephritis may develop in spite of ongoing immunosuppressive treatment.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1432-1076
    Keywords: Corticosterone methyl oxidase type II ; Failure to thrive ; Salt wasting
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Corticosterone methyl oxidase type II (CMO II) deficiency is an uncommon cause of salt-wasting in infancy. We describe a boy who presented with recurrent dehydration and severe failure to thrive in the first 3 months of life, associated with mild hyponatraemia (serum Na+ 127–132 mEq/l) and hyperkalaemia (serum K+ 5.3–5.9 mEq/l). The diagnosis was suggested by an elevated plasma renin activity (PRA): serum aldosterone ratio, and subsequently confirmed by an elevated serum 18-hydroxycorticosterone: aldosterone ratio. Treatment with 9α-fluorohydroxycortisone normalized growth parameters and PRA levels. CMO II deficiency should be considered in infants with recurrent dehydration and failure to thrive, even when serum sodium and potassium levels are not strikingly abnormal.
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1432-1076
    Keywords: Neurogenic arthrogryposis ; Parenchymal giant cell transformation ; Pigmentary liver disease ; Ductopenia ; Renal tubulopathy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We report on five patients from three families with neurogenic arthrogryposis, cholestasis and tubular renal dysfunction. Despite a similar clinical picture the liver histology showed a broad pathological spectrum, ranging from pigment storage to parenchymal giant cell transformation and ductopenia. The findings are compared with those of other cases from the literature in search of a correct nosology of the syndrome characterized by arthrogryposis, renal and liver disease. Conclusion We propose to consider the picture of arthrogryposis, renal tubular dysfunction and cholestasis as a single syndrome.
    Type of Medium: Electronic Resource
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