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  • 1
    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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  • 2
    ISSN: 1432-1238
    Keywords: Key words Kyphoscoliosis ; Mechanical ventilation ; Respiratory mechanics
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Objective: To evaluate respiratory mechanics in the early phase of decompensation in a group of seven patients with severe kyphoscoliosis (KS) (Cobb angle 〉 90 °) requiring mechanical ventilatory support. Design: Prospective clinical study with a control group. Setting: General intensive care unit at University of Rome “La Sapienza”. Patients: Seven consecutive patients affected by severe KS in the early phase of acute decompensation and a control group of six ASA (American Society of Anesthesiology) 1 subjects who were mechanically ventilated during minor surgery. Measurements and results: Respiratory mechanics were evaluated during constant flow-controlled mechanical ventilation at zero end-expiratory pressure with the end-inspiratory and end-expiratory occlusion technique. In five patients who showed increased ohmic resistance (RRSmin), we evaluated the possibility of reversing this increase with a charge dose of 6 mg/kg doxophylline i. v. In four KS patients, in whom a reliable esophageal pressure was confirmed by a positive occlusion test, we separated respiratory system data into lung and chest wall component. All KS patients showed reduced values of respiratory compliance (CRS) and increased respiratory resistance (RRS). The average basal values of CRS were 36 ± 10 vs 58 ± 8.5 cmH2O in control patients; RRSmax was 20 ± 3.1 vs. 4.5 ± 1.2 cmH2O/l per s; RRSmin 6.2 ± 1.2 vs. 2 ± 0.5 cmH2O/l per s: ΔRRS 14 ± 2.6 cmH2O vs 2.4 ± 0.7 cmH2O/l per s. All KS patients showed low values of intrinsic positive end-expiratory pressure (PEEPi) (1.8 ± 1.5 cmH2O). Separation of lung and chest-wall mechanics, performed only in four patients, showed a reduction in both lung (66.7 ± 7.2 ml/cmH2O) and chest wall values (84 ± 8.2 ml/cmH2O), while both RmaxL and RmaxCW were increased (16.6 ± 2 and 2.8 ± 0.4 cmH2O/l per s, respectively). Infusion of doxophylline did not significantly change respiratory mechanics when evaluated 15, 30, and 45 min after the infusion. Conclusions: During acute decompensation, both lung and chest-wall compliance are severely reduced in KS patients: conversely, and, contrary to that in patients with chronic obstructive pulmonary disease, increases in airway resistance and PEEPi seem to play only a secondary role.
    Type of Medium: Electronic Resource
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  • 3
    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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  • 4
    ISSN: 1432-1076
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1432-1076
    Keywords: Glycogen storage disease ; Glucose-6-phosphatase ; Glucose-6-phosphate translocase ; Debranching enzyme ; Phosphorylase-6-kinase
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A workshop was held on “Aspects of treatment of patients with glycogen storage disease” within the framework of the Concerted Action “Inborn errors of metabolism” of the European Communities. Consensus was reached on the main issues of treatment of patients with deficiency of glucose-6-phosphatase, glucose-6-phosphate translocase, debranching enzyme, liver phosphorylase and phosphorylase-b-kinase. The resulting recommendations are reported.
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 150 (1991), S. 599-600 
    ISSN: 1432-1076
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 7
    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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  • 8
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 156 (1997), S. 737-738 
    ISSN: 1432-1076
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 9
    ISSN: 1432-1017
    Keywords: Key words Translational diffusion ; Rotational diffusion ; Relaxation times ; Intrinsic viscosity ; Protein structure and dynamics
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Physics
    Notes: Abstract Spheres, cylinders or ellipsoids, whose hydrodynamic properties can be computed from analytical or semi-analytical expressions, have been traditionally used as low-resolution approximate descriptors of macromolecular size and shape. However, these simple geometrical bodies can seldom faithfully reproduce any detail of a macromolecular surface. A more sophisticated procedure utilizes instead ensembles of spheres (``beads'') of various diameters in an appropriate spatial arrangement to model the macromolecule. This method has not yet gained widespread application, partially because of the difficulties involved both in the generation and in the handling of the models, and because of the rather complicated mathematics involved in the computation of the hydrodynamic parameters, requiring non-trivial dedicated computer software virtually unavailable in the public-domain. A notable exception was the ``TRV'' program and its predecessors developed by the Garcι′a de la Torre's group, which have been recently updated and made available as the ``HYDRO'' package (Garcι′a de la Torre et al. 1994). To make accessible as many aspects as possible of this powerful modelling procedure, we have assembled a set of computer programs written in C language called BEAMS (BEAds Modelling System), which are described in this paper. The main BEAMS programs provide the user with a choice of four different methods for the computation of the hydrodynamic and structural parameters of ensembles of beads, with the option of automatically generating many random conformations of linear, branched and/or looped strings-of-beads. Selected models can be visualized from any desired point of view and manipulated interactively on a high-resolution colour monitor, and plotted as two-dimensional projections on an eight colour plotter. A further option permits the calculation of the parameters for segmentally flexible models composed of two subunits. Two ancillary programs, PROMOLP (written in VisualBasic for WindowsTM) and GRUMB (written in C for general PC use) help the user in the definition of the number and radius of the beads to be used for the models, and in the interactive construction of spatially pre-defined models. BEAMS should be especially useful in the generation of low-resolution models of large-sized proteins which are difficult or impossible to solve with high-resolution techniques such as X-ray crystallography or NMR, and, in multidomain/modular proteins, in defining the overall spatial arrangement of the various domains/modules from their known 3D-structures.
    Type of Medium: Electronic Resource
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  • 10
    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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