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Biomedical subjects

J C Raphael

Publications and source records attributed to J C Raphael.

At least 37 records · Page 2Linked to original sources
PubMed1996

[Treatment of Guillain-Barré syndrome by plasma exchange: proposal of a therapeutic strategy].

Although the favorable effect of plasma exchange (PE) has been demonstrated in Guillain-Barré syndrome, the optimal number of PE and management at different degrees of gravity remain to be ascertained. A new controlled multicentric trial is therefore being conducted since 1986. Mid-term results show that 2 PE give better results than 0 in patients who are still able to walk. Inversely, 6 PE do not give better results when ventilatory assistance was initially required. It is therefore logical to propose 2PE as early as possible. Two supplementary PE can be used in case of disease progression. Use of high dose immunoglobulins is not justified except when PE is contraindicated.

Humans↗
PubMed1994

Randomised trial of preventive nasal ventilation in Duchenne muscular dystrophy. French Multicentre Cooperative Group on Home Mechanical Ventilation Assistance in Duchenne de Boulogne Muscular Dystrophy.

Duchenne muscular dystrophy (DMD) is the most common muscular dystrophy in children. Paralysis of respiratory muscles causes a decrease in forced vital capacity (FVC) from age 12 years, and death occurs between 20 and 25 years old and is usually related to respiratory insufficiency. Uncontrolled studies suggest that early home use of nasal intermittent positive-pressure ventilation (NIPPV) in DMD patients free of respiratory failure could limit progression of the restrictive syndrome and therefore improve survival because efficacy of preventive NIPPV has not been demonstrated in a controlled trial, we undertook a randomised multicentre study in which 70 patients with DMD were included. Patients were free of daytime respiratory failure and FVC was between 20 and 50% of predicted values. At least 6 h of nocturnal NIPPV (n = 35) was compared with conventional treatment (n = 35). During a mean follow-up of 52 months, 10 patients died, 8 in the NIPPV group and 2 in the control group (p = 0.05, log-rank test). No differences were observed between the two groups for occurrence of hypercapnia, decrease of FVC below 20% of initial values, or use of necessary mechanical ventilation. Preventive NIPPV did not improve respiratory handicap and reduced survival of DMD patients. Use of NIPPV for preventive purposes should be avoided in patients with FVC between 20 and 50% of predicted values.

Adolescent↗
PubMed1994

Sleep-related respiratory disturbances in patients with Duchenne muscular dystrophy.

Sleep-related respiratory disturbances (SRD) in patients with muscle diseases may have significant clinical implications, because the patients frequently die at night. The aims of the study were to :1) assess the presence and severity of sleep-related respiratory disturbances in patients with Duchenne muscular distrophy (DMD); and 2) investigate the relationship of sleep-related respiratory disturbances to daytime symptoms and pulmonary function. We studied six clinically stable patients with Duchenne muscular dystrophy, mean age (+/- SD) 18 +/- 2 yrs. Vital capacity was 27 +/- 19% of predicted and daytime arterial oxygen tension (PaO2) was 10.9 +/- 1 kPa (range 8.9-12.4 kPa). The presence of daytime somnolence, insomnia, headache, nightmares and/or snoring was recorded. Four patients (67%) showed symptoms that suggest sleep-related respiratory disturbances. At night, the apnoea-hypopnoea index (AHI) was 11 +/- 6. The patients with more symptoms during the daytime had the highest AHI scores. Most of the apnoeas (85%) were central, particularly during rapid eye movement (REM) sleep. Sleep architecture was well-preserved. Arterial desaturation (> 5% below baseline) occurred during 25 +/- 23% of total time. AHI correlated with daytime PaO2, and AHI in REM sleep correlated with age. A stepwise multivariate analysis showed that PaO2 and, to some extent, the degree of airflow obstruction were significantly correlated with AHI. We conclude that sleep-related respiratory disturbance are frequently present in patients with Duchenne muscular dystrophy. Therefore, physicians should look for symptoms related to sleep-related respiratory disturbances in these patients.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1994

[Evaluation of hospital care. An experiment at the Public Assistance Hospitals in Paris].

Since 1990 the Assistance Publique--Hôpitaux de Paris (AP-HP) developed a policy of health care assessment in agreement with the implications of the law of July 31, 1991. The department of Evaluation of health care, in close collaboration with the local Committees which operate in most of the hospitals of AP-HP, performed during the last three years about fifty studies dealing with two main topics: professional procedures and quality of care. Through three specific selected studies, the authors emphasize the requirements of an assessment policy to efficiently contribute to the improvement of quality of care.

Hospitals, Public↗
PubMed1993

Plasma exchange morbidity in Guillain-Barré syndrome: results from the French prospective, randomized, multicenter study. The French Cooperative Group.

OBJECTIVES: To describe all adverse events occurring during plasma exchange sessions in adult patients with the Guillain-Barré syndrome. To analyze these events with regard to the technical modalities and biological changes induced by sessions, and to try to identify a population at high risk for adverse events. DESIGN: Double-blind, randomized, prospective, multicenter trial. SETTING: A total of 28 French and Swiss intensive care units. PATIENTS: The study is based on 220 patients allocated either to plasma exchange (n = 109) or not (n = 111). This study focused on 105 patients who received at least one plasma exchange, with replacement fluid secondly allocated by randomization to albumin, or fresh frozen plasma. A total of 105 patients underwent 390 plasma exchanges. Fifty-five patients received albumin (208 sessions) as replacement fluid, and 50 patients received fresh frozen plasma (182 sessions). INTERVENTIONS: Prospective monitoring of patients for each session including technical modalities, adverse effects, and biological parameters. MEASUREMENTS AND MAIN RESULTS: A total of 253 adverse incidents were recorded. At least one adverse incident occurred in 39% of plasma exchange sessions among 80 (76%) patients. In 15 patients, plasma exchange treatment had to be discontinued because of severe intolerance (six patients, including three patients with severe bradycardias), intercurrent complications, mainly infections (four patients), and technical difficulties. One patient with pneumococcal septicemia and pneumonia died during the second plasma exchange session. Fresh frozen plasma was associated with more adverse incidents than albumin (135 vs. 118, p = .008). The occurrence of adverse events was also related to the preplasma exchange hemoglobin level assessed before the session (p = .04). Otherwise, the frequency of adverse effects did not depend on technical modalities (type of equipment, anticoagulation). Age, sex, previous history, neurologic severity, and the need for mechanical ventilation, as assessed on inclusion in the study, did not modify the risk of adverse effects. Finally, occurrence of bradycardia did not rely on initial neurologic severity. CONCLUSIONS: These results confirm that fresh frozen plasma should be abandoned as replacement fluid in plasma exchanges of Guillain-Barré syndrome patients. They also underline the need for close monitoring of patients during sessions and, especially, the respect of treatment contraindications. Some adverse incidents could be attributed to the underlying disease rather than to the plasma exchange session.

Albumins↗
PubMed1992

Breathing disorders during sleep in myasthenia gravis.

Twenty consecutive patients (16 women and 4 men), with a mean age of 40 years, who were diagnosed and treated for myasthenia gravis were enrolled in a prospective investigation aimed at determining the amount of respiratory disturbance occurring during sleep while they received treatment. Patients were clinically evaluated to determine body mass index, presence of upper airway anatomical abnormalities, level of functional capacity and activity scored from 1 to 5, and presence of sleep-related complaints. They underwent daytime pulmonary function tests, determination of maximal static inspiratory pressure, measurement of transdiaphragmatic pressure, and measurement of arterial blood gas levels. Polygraphic monitoring during sleep, evaluating respiration and oxygen saturation, was also performed. Results indicated that in the studied population, all subjects had evidence of daytime diaphragmatic weakness as demonstrated by transdiaphragmatic pressure measurements, independent of the degree of autonomy and functional capacity and activity level reached. Older patients with moderately increased body mass index, abnormal total lung capacity, and abnormal daytime blood gas concentrations were the primary candidates for development of diaphragmatic sleep apneas and hypopneas, and oxygen desaturation of less than 90% during sleep. However, these clear indicators were not found in all subjects with sleep-related disordered breathing. Rapid-eye-movement sleep was the time of highest breathing vulnerability during sleep. Sleep-related complaints may also help identify subjects at risk for abnormal breathing during sleep, even when daytime functional activity is judged normal.

Adult↗
PubMed1992

[Methods of investigating orthostatic hypotension].

Orthostatic hypotension, which is common mainly in the elderly, is in many cases related to hypovolemia and/or vasodilators intake. However, when an impairment of the autonomic nervous system is suspected, orthostatic hypotension severity and mechanism may be investigated. The most common tests are the head upright tilt-test and the Valsalva manoeuvre. Both of them examine the baroreflex system as a whole, and become non invasive tests with the development of finger arterial blood pressure continuous measurement. Each part of the baroreflex system may be investigated separately. So, cardiac vagal responses to ocular compression, to carotid sinus massage, to respiratory change or to atropine infusion, may be tested. On the other hand, sympathetic efferent pathways may be stimulated in a variety of ways, such as isometric exercise, cutaneous cold, mental arithmetic, norepinephrine infusion, or tiltest. None of these tests should be applied systematically, but according to the clinical features.

Aged↗
PubMed1991

Acute carbon monoxide intoxication and hyperbaric oxygen in pregnancy.

Modalities of oxygen therapy for pregnant women intoxicated with carbon monoxide (CO) are ill defined. Hyperbaric oxygen (HBO) is presumed to be hazardous to the pregnancy. On the other hand CO entails anoxic injuries in the mother and fetus. We have entered 44 pregnant women who sustained an acute carbon monoxide poisoning at home, into a prospective study in order to assess HBO tolerance. They were treated within 5.3 +/- 3.7 h (range: 1-12) of the intoxication with a combination of 2 h of HBO at a pressure of 2 atmospheres absolute (ATA) and 4 h of normobaric oxygen, irrespective of the clinical severity of the intoxication and of the age of pregnancy. Six patients were lost to obstetric follow-up. Only 2 patients sustained a spontaneous abortion: 1 within 12 h and 1 within 15 days of the intoxication. Thirty-four women gave birth to normal newborns. Finally 1 elected to undergo abortion for reasons unrelated to the intoxication and 1 gave birth to a baby with Down's syndrome. There is no evidence that HBO was involved with either abortion of our study. We conclude that HBO may be carried out in pregnant women acutely intoxicated with carbon monoxide.

Acute Disease↗
PubMed1989

Trial of normobaric and hyperbaric oxygen for acute carbon monoxide intoxication.

The value of hyperbaric oxygen in the treatment of acute carbon monoxide intoxication was assessed in 629 adults who had been poisoned at home in the 12 h before admission to hospital. In patients without initial impairment of consciousness (group A) the effect of 6 h of normobaric oxygen (NBO) (group A0, n = 170) was compared with that of 2 h of hyperbaric oxygen (HBO) at 2 atmospheres absolute (ATA) plus 4 h NBO (group A1, n = 173). At the 1 month follow-up 66% of A0 and 68% of A1 patients had recovered. In patients with initial impairment of consciousness the effect of one session of HBO (group B1, n = 145) was compared with that of two sessions (group B2, n = 141); all group B patients also received 4 h of NBO. At 1 month of follow-up 54% group B1 and 52% group B2 patients had recovered. The 7 patients left with neuropsychiatric sequelae (3 B1, 4 B2) and the 4 who died (2 B1, 2 B2) had all presented with coma. HBO was not useful in patients who did not lose consciousness during carbon monoxide intoxication, irrespective of their carboxyhaemoglobin level, nor were two sessions of HBO in patients who sustained only a brief loss of consciousness. The prognosis is poorest for those presenting with coma; the trial needs to be pursued in this group of patients until the power of the study is sufficient to demonstrate the value or otherwise of HBO.

Accidents, Home↗
PubMed1989

[Serum demyelinating activity and Guillain-Barre syndrome: favorable effect of plasma exchange].

The present study was designed to determine whether plasma exchange has a beneficial effect on the activity of the demyelinating factor present in the serum of patients with the Guillain-Barre syndrome. The demyelinating activity was assessed electrophysiologically after subperineurial injection of 20 to 30 microliters of serum into the rat sciatic nerves Serum was obtained from 26 patients from the French Cooperative Trial, among whom 12 had plasma exchange. Sera were drawn at hospital admission (DO) and at D15, D30 and D60. They consistently produced conduction block in a large proportion of motor axons, measured by the ratio of amplitudes of the motor action potential elicited by stimulation at both proximal and distal sites of injection. The serum activity diminished during the time course of the illness earlier in patients receiving plasma exchange and even more in patients with the less severe clinical score at the plateau phase. Serum from patients without motor sequelae after one year evolution did not produce conduction block prior to D30, as compared to sera from patients with sequelae which were still very active. This work add biological data to the positive results of plasma exchange in Guillain-Barre syndrome obtained by the North American and French Cooperative Trials.

Adult↗
PubMed1988

Adult maltase acid deficiency myopathy: treatment with long-term home mechanical ventilation.

We report a study of an adult with a maltase acid deficiency myopathy. A restrictive respiratory syndrome due to respiratory muscle weakness is associated with paralysis of other muscular groups. In 1982 the patient presented with an alveolar hypoventilation, and mechanical ventilation was required after acute respiratory failure. The patient has received nocturnal mechanical ventilation by tracheostomy at home for 5 years. His clinical status gradually improved in parallel to amelioration of his respiratory condition. Functional respiratory tests improved: initial hypoxia-hypercapnia disappeared, vital capacity increased. The possible mechanisms underlying the improvement are discussed. Increase in pulmonary compliance is an argument to explain the functional improvement observed. Ventilatory response to carbon dioxide was abnormal whereas the ventilatory response to exercise and maxima minute ventilation test were normal. Results are consistent with a respiratory control impairment. The role of mechanical ventilation is difficult to assess in the improvement we observed.

Adult↗
PubMed1986

[Surgically treated syphilitic gumma of the brain. Computerized tomography findings].

Syphilitic gumma is now exceptional. Symptomatology is non-specific and frequently, as in the case reported here, the onset is marked by a localized or generalized convulsion. A space-occupying lesion is recognized by angiography and CT. The precise diagnosis is suggested by evidence of arteritis or of a hypodense mass outlined by contrast or slight calcifications and confirmed by positive serology in a patient with a suspected cerebral tumor. In some cases a positive diagnosis can be made only by pathological examination after surgical ablation of the gumma. An area of central necrosis is surrounded by a granulomatous layer rich in plasmocytes (the polyclonal nature of the secretion is shown by immunocytochemistry) and a peripheral fibroblastic zone. The arteries are ensheathed by a dense inflammatory infiltrate constituted almost entirely by plasmocytes and their lumens are either stenosed or filled with endarteritic debris. Surgical treatment should be combined with penicillin administration. For a patient in good general condition with a suspected gumma, medical treatment can be given initially, with follow-up by CT, neurosurgery being indicated only in case of failure of medical therapy. The rarity of gummata in relation to other tertiary syphilitic lesions remains unexplained. The patient reported here had multiple infections including one due to cytomegalovirus after ablation of the gumma, highly suggesting an immunity disorder.

Adult↗
PubMed1986

[The Landry-Guillain-Barré syndrome. Study of prognostic factors in 223 cases].

A retrospective study of 223 patients (111 men, 112 women, mean age 40.6 years) with the Landry-Guillain-Barre syndrome investigated vital or functional prognostic factors. Patients were first seen between 1963 and 1981, when 192 were noted to have cranial nerve disorders. Assisted ventilation was required in 152 cases, and 23 patients died, including 5 from cardiocirculatory dysautonomy. Plasma exchange was not used. Patients were divided into 3 groups as a function of the degree of maximum paralysis: group 1 (n = 63) patients had incomplete quadriplegia without assisted ventilation, group 2 (n = 93) incomplete quadriplegia with assisted ventilation, group 3 (n = 62) quadriplegia with or without assisted ventilation. Vital prognosis was related to the severity of neurologic disorders. Frequency of intercurrent complications, dysautonomic disorders and mortality increased with maximal severity of motor deficit. The 3 groups did not differ as a function of previous history, mode of onset of polyradiculoneuritis, duration of extension phase of paralysis, or CSF protein content at the first examination during the extension phase of the paralyses. It is therefore impossible at an early stage of the disease to predict future motor deficit. The cumulative percentage of patients who recovered a normal muscular force in limbs and cranial territories was 48 p. 100 after one year and 60 p. 100 after two years (actuarial values). The functional prognosis depends upon the degree of motor deficit at maximum paralysis and duration of the plateau phase. Probability of full muscular strength recovery was lower in group 3 and in those patients with a plateau phase duration longer than 2 weeks. Independently of groups, motility recovery rate was markedly higher in patients with a plateau phase duration of less than one week. Motility recuperation was independent of age, sex, duration of extension phase, CSF protein levels during the acute phase, and presence of autonomic nervous system disorders.

Adolescent↗