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Biomedical subjects

J C Raphael

Publications and source records attributed to J C Raphael.

At least 19 recordsLinked to original sources
PubMed2005

[Long term domiciliary mechanical ventilation in patients with neuromuscular disease (indications, establishment and follow up)].

UNLABELLED: Neuromuscular diseases represent a heterogeneous group of pathologies which common feature is the development of a restrictive ventilatory failure. BACKGROUND: Respiratory insufficiency of neuromuscular origin manifests itself by functional symptoms that must be carefully searched for in the history, such as headaches, sleep disorders, or dyspnoea of effort, sometimes very mild, or in severe cases associated with orthopnoea. Follow up should be multi-disciplinary. On the respiratory level regular measurement of blood gases, vital capacity, maximum inspiratory and expiratory pressures as well as sleep studies, will detect the criteria for mechanical ventilation (hypercarbia > 45 mm Hg, nocturnal desaturation < 88%, vital capacity < 60%, PImax < 60 cm H2O). STATE OF THE ART: The establishment of mechanical ventilation is a major decision for patients with neuromuscular disease because of the important physical, psychological, social and sometimes financial consequences. The patients and their family must be instructed precisely in order to obtain the best possible observation and compliance. The establishment requires a stay in hospital of several days to optimise the choice of ventilator, its settings, and connections. The link with the organisation managing the domiciliary ventilation is fundamental in ensuring follow up after discharge from hospital. Techniques of cough assistance must be taught to each neuromuscular patient requiring mechanical ventilation. CONCLUSION: Ventilation of neuromuscular patients requires careful evaluation of the indications and rigorous follow up by a multidisciplinary team with wide experience of this type of disease.

Cough↗
PubMed2002

Critical incidents related to invasive mechanical ventilation in the ICU: preliminary descriptive study.

OBJECTIVES: To establish a preliminary list of critical incidents (CIs) associated with mechanical ventilation and to describe a CI reporting method. DESIGN: A list of CIs was established based on a consensus among ICU caregivers. The list was compared to CIs collected prospectively during a predefined study period. SETTING: The clinical observations were conducted in two intensive care units. PATIENTS: All patients receiving mechanical ventilation were included. MEASUREMENTS AND RESULTS: The list of CIs included death and 62 other CI types categorized as immediately life-threatening, secondarily life-threatening, or non-life-threatening. The observational study identified 527 CIs in 137 patients. Virtually all non-life-threatening CIs were ascribed to failure to comply with safety rules or to equipment failure and 40% of life-threatening CIs to the course of the disease or to patient-related factors. The match between CI types on the list and CI types observed in the ICUs was excellent. CONCLUSIONS: Use of our reporting method to create a CI database in a multicenter study including ICUs with varying recruitment patterns may help to identify markers suitable for routine continuous use as part of a quality-assurance program.

Humans↗
PubMed2001

Intravenous immune globulins in patients with Guillain-Barré syndrome and contraindications to plasma exchange: 3 days versus 6 days.

Plasma exchange is contraindicated in 10 to 20% of patients with Guillain-Barré syndrome (GBS). The optimal schedule for intravenous immune globulin (IVIg) therapy has not yet been established in these patients. The objective was to compare the efficacy and safety of two IVIg treatment durations in patients with GBS with contraindications for plasma exchange. In this randomised, double blind, multicentre phase II trial conducted in seven French centres, patients with GBS with severe haemostasis, unstable haemodynamics, or uncontrolled sepsis were randomly assigned to 0.4 g/kg/day IVIg for 3 or 6 days. The primary outcome measure was the time needed to regain the ability to walk with assistance. Thirty nine patients were included from March 1994 to May 1997, 21 in the 3 day group and 18 in the 6 day group. Time to walking with assistance was non-significantly shorter in the 6 day group (84 (23-121) v 131 days (51-210), p=0.08); the difference was significant in ventilated patients (86 days (13-151) in the 6 day group v 152 days (54-332) in the 3 day group; p=0.04). The prevalence and severity of IVIg related adverse effects were comparable between the two groups. In conclusion, in patients with GBS and contraindications for plasma exchange, especially those who need ventilatory assistance, IVIg (0.4 g/kg/day) may be more beneficial when given for 6 days rather than 3 days.

Aged↗
PubMed2001

[Electrophysiologic study of 10 cases of Miller Fisher syndrome].

The association of ophthalmoplegia, ataxia and areflexia was described by Miller Fisher in 1956. It is postulated as a variant of the Guillain Barré syndrome. We report 10 Miller Fisher syndrome patients admitted in an intensive care unit between June 1990 and February 1999 who were selected according to clinical criteria of Ropper and Wijdicks. All patients had motor and sensory nerve conduction studies and electromyography, nine had visual and brainstem auditory evoked potentials and two had short latency somatosensory evoked potentials. Peripheral neuropathy was found in all patients. All had sensory nerve changes and some were severe. Motor nerve conduction abnormalities were observed in 7 only cases with moderate increase of F latency in 3 cases and compound muscle action potential reduction in 3 other cases. In the last case, motor conduction abnormalities was more severe, characterized by conduction velocity slowing in both distal and proximal sites and by temporal dispersion of action potentials. All brainstem auditory evoked studies were normal. In 4 patients, MRI studies were normal. These data support that brainstem is preserved in MFS. Only one patient had visual evoked potential abnormalities. Optic neuropathy is debated in Miller Fisher and in Guillain Barré syndrome. As a conclusion, in MFS peripheral neuropathy is always present with severe sensitive changes and moderate motor changes (This is different as compared to Guillain Barré syndrome according to electrophysiological data). We did not find involvement of brainstem in our patients with Miller Fisher syndrome.

Adolescent↗
PubMed2000

Nocturnal mechanical ventilation for chronic hypoventilation in patients with neuromuscular and chest wall disorders.

BACKGROUND: Chronic alveolar hypoventilation is a common complication of many neuromuscular and chest wall disorders. Long term nocturnal mechanical ventilation is used to treat an increasing number of patients. OBJECTIVES: To examine the efficacy of nocturnal mechanical ventilation in relieving hypoventilation related symptoms in patients with neuromuscular or chest wall disorders. SEARCH STRATEGY: Search of the Cochrane Neuromuscular Disease Group register for randomized trials and enquiry from authors of trials and other experts in the field. SELECTION CRITERIA: Types of studies: quasi-randomized or randomized controlled trials TYPES OF PARTICIPANTS: patients with neuromuscular or chest wall disorder-related stable chronic hypoventilation of all ages and all degrees of severity. Types of interventions: any type and any mode of nocturnal mechanical ventilation. Types of outcome measures: Primary: short term and long term reversal of hypoventilation related clinical symptoms Secondary: unplanned hospital admission rate, one year mortality, short term and long term reversal of day time hypercapnia, improvement of lung function and improvement of sleep breathing disorders. DATA COLLECTION AND ANALYSIS: We identified four randomized trials. One author extracted the data and another checked them. Individual data were available from the authors of the largest study. MAIN RESULTS: The four eligible trials included a total of 51 patients. The risk difference (proportion of patients) of no improvement of hypoventilation related clinical symptoms in the short term following nocturnal mechanical ventilation was significant and favoured treatment, -0.417 (95% CI -0.639 to -0.194). However, there was significant heterogeneity across the studies (p<0.001). Similarly, the risk difference of no reversal of day time hypercapnia in the short term following nocturnal ventilation was significant and favoured treatment, -0.635 (95% CI -0.874 to -0.396). The weighted mean difference of nocturnal mean oxygen saturation percent was 5.5 (95% CI 1.5 to 9.4) more improvement in patients treated with nocturnal mechanical ventilation. For the primary and most of the secondary outcome measures there was no significant difference between nocturnal mechanical ventilation and no ventilation in the long term, except for one-year mortality. Indeed, the risk difference of death one year following implementation of nocturnal mechanical ventilation was significant and favoured treatment, -0.259 (95% CI -0.478 to -0. 041). However, there was significant heterogeneity across the studies (p<0.001). Most of the secondary outcomes were not assessed in the eligible trials. No data could be summarised for the comparisons between invasive and non-invasive mechanical ventilation, between intermittent positive pressure and negative pressure ventilation, and between volume-cycled and pressure-cycled ventilation. REVIEWER'S CONCLUSIONS: Current evidence about the therapeutic benefit of mechanical ventilation is weak, but consistent, suggesting alleviation of the symptoms of chronic hypoventilation in the short term, and in two small studies survival was prolonged. Mechanical ventilation should be offered as a therapeutic option to patients with chronic hypoventilation due to neuromuscular diseases. Further larger randomized trials are needed to confirm long term beneficial effects of nocturnal mechanical ventilation on quality of life, morbidity and mortality, to assess its cost-benefit ratio, and to compare the different types and modes of ventilation.

Chronic Disease↗
PubMed2000

[Corticotherapy in severe infectious states].

Septic shock is one of the leading cause of death in modern countries. Scientists have made huge improvement in the understanding of mechanisms of inflammation, and the sequence of activation of the various pro and anti-inflammatory markers is now well known. By contrary, physicians have failed to improve survival from septic shock, in spite of the development of specific targets of the various points of the cytokine cascade sought to have a key role in host survival to sepsis. Corticosteroids were among the first anti-inflammatory drugs, which have been tested in high quality randomised controlled trials. These trials clearly showed that patients with septic shock are unlikely to benefit from a short course of a large dose of an anti-inflammatory steroid. More recent findings highlighting the role of the integrity of the hypothalamic-pituitary -adrenal axis to appropriately respond to a septic insult, have led to a reappraisal of the use of steroids in septic shock. Several high quality randomised controlled trials have evaluated the efficacy and safety of a prolonged treatment with low dose hydrocortisone in severe sepsis. These trials strongly suggested that this strategy of corticotherapy reduced the morbidity of septic shock and may favourably affect survival from septic shock.

Adrenal Cortex Hormones↗
PubMed1999

Treatment of the adult Guillain-Barré syndrome: indications for plasma exchange.

The Guillain-Barré syndrome is the most common cause of acute flaccid paralysis. Currently, 5% of patients die and 10% are left with severe motor sequelae at one year. Multidisciplinary teams, trained to specific treatments, are required to manage these patients. Oral and intravenous steroid treatment of GBS has been disappointing. Two large randomized clinical trials comparing plasma exchange (PE) to standard supportive treatment have shown a short-term and a one-year benefit of PE. Appropriate number of exchanges and indications of PE are now more precisely known. Patients with mild forms of the disease (able to walk) should receive two PEs, while a further two exchanges should be done in case of deterioration or in advanced forms (loss of walking ability, mechanical ventilation). A greater number of exchanges does not appeared beneficial. More recently, two randomized trials produced some evidence that intravenous immune globulin (IVIg, 0.4 g/kg daily for five days) and PE had equivalent efficiency in advanced forms. The combination of PE with IVIg did not yield a significant advantage, but did increase cost and risk. In advanced forms, the choice between PE and IVIg depends on the contraindications of each treatment.

Guillain-Barre Syndrome↗
PubMed1999

Inappropriate sympathetic activation at onset of septic shock: a spectral analysis approach.

The autonomic cardiovascular control was investigated in 10 patients with septic shock, 10 patients with sepsis syndrome, and six tilted healthy subjects. Overall variability, high- and low-frequency components (AUC, HF, and LF, beats/min(2)/Hz or mm Hg(2)/Hz) from heart rate (HR), systolic (SBP) and diastolic (DBP) blood pressures spectra were obtained from 5-min recordings. LF(HR)/HF(HR) and the square root of LF(SBP)/LF(HR) (alpha) were used as indices of sympathovagal interaction and baroreflex control of the heart, respectively. Compared with tilted control subjects and patients with sepsis syndrome, septic shock is characterized by reduction in: (1) HR variability, i.e., decreased AUC(HR) (p = 0.007), LF(HR) (p = 0.002), and LF(HR)/HF(HR) (p = 0.0002); (2) DBP variability, i.e., decreased AUC(DBP) (p = 0.003) and LF(DBP) (p = 0.001), (3) alpha (p = 0.003). In septic shock, LF(HR)/HF(HR), alpha, and LF(DBP) correlated with mean blood pressure (r = 0.67, p = 0.04, r = 0.64, p = 0.03, and r = 0.88, p = 0.0008, respectively), and with plasma norepinephrine levels (r = -0.65, p = 0.03, r = -0.79, p = 0.006, and r = -0.69, p = 0.03, respectively). In conclusion, onset of septic shock is characterized by high concentrations of circulating catecholamines but impaired sympathetic modulation on heart and vessels, suggesting that central autonomic regulatory impairment contributes to circulatory failure.

Adult↗
PubMed1999

Mechanisms underlying effects of nocturnal ventilation on daytime blood gases in neuromuscular diseases.

The hypothesis that, in neuromuscular and chest wall diseases, improvement in central respiratory drive explains the effects of night-time ventilation on diurnal gas exchanges was tested. The effects at 6 months, 1, 2 and 3 yrs of intermittent positive pressure ventilation (IPPV) on arterial blood gas tension, pulmonary function, muscle strength, sleep parameters, respiratory parameters during sleep and ventilatory response to CO2 were evaluated in 16 consecutive patients with neuromuscular or chest wall disorders. As compared with baseline, after IPPV daytime arterial oxygen tension (Pa,O2) increased (+2.3 kPa at peak effect) and arterial carbon dioxide tension (Pa,CO2) and total bicarbonate decreased (-1.8 kPa and -5 mmol x L(-1), respectively) significantly; vital capacity, total lung capacity, maximal inspiratory and expiratory pressures and alveolar-arterial oxygen gradient did not change; the apnoea-hypo-opnoea index and the time spent with an arterial oxygen saturation (Sa,O2) value <90% decreased (-24 and -101 min, respectively), sleep efficiency and mean Sa,O2 increased (+16% and +5%, respectively); and ventilatory response to CO2 increased (+4.56 L x min(-1) x kPa(-1)) significantly. The reduction in Pa,CO2 observed after IPPV correlated solely with the increase in the slope of ventilatory response to the CO2 curve (r=-0.68, p=0.008). In neuromuscular or chest wall diseases, improvement of daytime hypoventilation with nocturnal intermittent positive pressure ventilation may represent an adaptation of the central chemoreceptors to the reduction of profound hypercapnia during sleep or reflect change in the quality of sleep.

Adult↗
PubMed1999

[Guillain-Barré syndrome: from the original description to the modern concepts].

Since the original report, acute paralysis and albuminocytologic dissociation have remained hallmarks of the Guillain-Barré syndrome but the initially described favorable outcome with complete motor recovery is not always observed. Guillain-Barré syndrome can be complicated by early respiratory distress, dysautonomia and late functional impairment. The conduction block induced by acute demyelination accounts for the spontaneous neurological improvement. On the other hand, early axonal damage or sustained demyelination can be responsible for residual deficit. The cause and mechanism of the disease still remain unclear. For these patients, general care is essential and should be provided in appropriate hospital units. Today, specific treatment consists of either plasma exchange or high-dose intravenous immunoglobulins; the choice depends on their respective contraindications.

Electrophysiology↗
PubMed1998

[Guillain-Barré syndrome in the adult: therapeutic aspects].

The Guillain-Barré syndrome is the most common cause of acute flaccid paralysis. The current mortality is 5%, while severe motor sequelae persist after one year in 10%. Multidisciplinary teams, trained in all specific treatments, are required to treat these patients. Oral and intravenous steroids have proved ineffective. Two large randomized clinical trials comparing plasma exchange (PE) with no treatment have shown a short-term and a 1-year benefit. The appropriate number of exchanges and the indications are now more precisely known. In the mild form (walking possible) patients should receive two PEs; a further two exchanges should be done in the event of deterioration or in advanced forms (loss of walking ability, mechanical ventilation). More exchanges are not beneficial. Recently two new randomized trials have produced evidence that intravenous immunoglobulin (IVIg) (0.4 g/kg/day for 5 days) was as effective as 5 PEs in advanced forms. The combination of PE with IVIg did not confer a significant advantage but increased the costs and risks. In advanced forms the choice between PE and IVIg depends on the contraindications for each treatment.

Adult↗
PubMed1998

[Treatment of Guillain-Barré syndrome in adults: role of plasma exchange].

PURPOSE: To review recent data on treatment of Guillain-Barré syndrome, especially indications of plasma exchange. DATA SYNTHESIS: Guillain-Barré syndrome is the most common cause of acute flaccid paralysis. The current mortality is 5%, sever motor sequelae persist after 1 year in 10% of cases. Multidisciplinary teams are required to treat these patients, trained to all specific treatments. Oral and intravenous steroids have proven ineffective. Two large randomized clinical trials comparing plasma exchange (PE) with no treatment have shown a short-term and a 1-year benefit. Appropriate number of exchanges and indications are now more precisely known. In mild form (walking possible), patients should receive two PEs. A further two exchanges should be done in case of deterioration or in advanced forms (loss of walking ability, mechanical ventilation). More exchanges are not beneficial. Recently two new randomized trials have produced evidence that intravenous immune globulin (IVIg) (0.4 g/kg/d for 5 days) were as effective as five PEs in advanced forms. The combination of PE with IVIg did not confer a significant advantage, while increasing cost and risks. CONCLUSION: The combination of PE with IVIg did not confer, in advanced forms, the choice between PE and IVIg depends of the contra-indications of each treatment.

Adrenal Cortex Hormones↗
PubMed1998

Impaired pressor sensitivity to noradrenaline in septic shock patients with and without impaired adrenal function reserve.

AIMS: To investigate the relationship between adrenal gland function and pressor response to noradrenaline in septic shock. METHODS: Basal cortisol level, noradrenaline--mean arterial pressure dose-response curve and cortisol response to intravenous corticotrophin bolus were obtained in nine patients fulfilling usual criteria for septic shock and in six normal volunteers. In patients with septic shock, dose-response curve to noradrenaline was determined a second time 60 min after a 50 mg intravenous hydrocortisone bolus. RESULTS: As compared with controls, patients with septic shock had increased basal cortisol levels (mean+/-s.d.: 1564+/-818 vs 378+/-104 nmol l(-1) , P=0.002, 95% confidence interval for difference in means: [452, 1920]) and a blunted cortisol response to corticotrophin (403+/-461 vs 1132+/-195 nmol l(-1), P=0.008, [-1163, -2951). Five patients had impaired adrenal function reserve. As compared with controls, septic patients displayed a moderate and non significant decrease in pressor sensitivity to noradrenaline (P=0.112). As compared with patients with adequate adrenal response, patients with impaired adrenal function reserve showed a significant decrease in pressor sensitivity to noradrenaline (P=0.038). In septic patients, hydrocortisone improved pressor response to noradrenaline (P=0.032). This effect was more marked in patients with impaired adrenal function reserve so that, as compared with patients with adequate response, the difference was no longer significant (P=0.123). CONCLUSIONS: In septic shock, impaired adrenal function reserve may partly be accounted for by the depressed pressor sensitivity to noradrenaline. The latter may be substantially improved by physiological doses of hydrocortisone.

Adrenal Gland Diseases↗
PubMed1996

Immunoglobulin A levels in bronchial samples during mechanical ventilation and onset of nosocomial pneumonia in critically ill patients.

Local immunoglobulins play a key role in host defense against lung infection. We investigated the pattern of evolution of bronchial albumin, IgA, and IgG levels in ventilated ICU patients in relation to nosocomial pneumonia. Immunocompetent, critically ill patients underwent serial blood and bronchial protein determinations on Day 1 (intubation day), and on Days 3, 7, 10, and 14. The variations in proteins levels were compared with corresponding Day 1 values in the whole population, and between patients who developed lung infections (Group A) and the remaining population (Group B). Forty-four patients were included into the study. In the whole population, when compared with the baseline value, bronchial IgA/albumin ratio increased significantly (Day 3, +58%, p = 0.04); Day 14, +171%, p < 0.01), but serum IgA/albumin and serum and bronchial IgG/albumin ratios did not change significantly. In Group A, the increase in the IgA/albumin ratio was less than in Group B (Day 3, +15% versus +87%, p = 0.04; Day 14, +29% versus +210%, p < 0.01). No significant differences were observed between the two groups for bronchial and plasma albumin and IgG levels and for bronchial polymorphonuclear elastase levels. Bronchial IgA production was enhanced in ventilated patients. A reduction in this enhanced bronchial IgA production might account for the development of nosocomial pneumonia.

Adult↗