[Show abstract][Hide abstract] ABSTRACT: Management of nonviral cryoglobulinemia vasculitis has yet to be defined. Rituximab has emerged as a novel and promising therapeutic alternative, but data are scarce. Our objective was to evaluate the safety and efficacy of rituximab in nonviral cryoglobulinemia vasculitis in off-trial real-life patients.
Prospective data from the French AutoImmunity and Rituximab (AIR) registry, which includes data on patients with autoimmune disorders treated with rituximab in off-label conditions, were analyzed.
Twenty-three patients received treatment with rituximab for cryoglobulinemia vasculitis. Tolerance was marked by the occurrence of side effects in almost half of the patients, including severe infections in 6 (26%) of 23, with a rate of 14.1 per 100 patient-years. These infections occurred in a particular subset of patients ages>70 years, with essential type II mixed cryoglobulinemia and renal failure with a glomerular filtration rate of <60 ml/minute, and receiving high-dose corticosteroids. Three of these patients died. In contrast, clinical and immunologic efficacy was noted in all evaluable patients. Clinical relapses occurred in half of the patients after a median time of 13.5 months following rituximab administration, and were more frequent in patients refractory to previous immunosuppressive therapy than in previously untreated patients.
Data from the AIR registry show a dramatic efficacy and a steroid-sparing effect of rituximab, but also show the occurrence of severe infections in elderly patients with renal failure and high-dose steroids. The role of rituximab in nonviral cryoglobulinemia vasculitis remains to be defined in well-designed randomized controlled trials.
Arthritis care & research. 12/2010; 62(12):1787-95.
[Show abstract][Hide abstract] ABSTRACT: The aim of the medical autopsy is to define the causes of a patient's death. Although its rate has dramatically decreased, the post-mortem examination remains one of the basic tools for the assessment of medical care. The objective of this study was to identify the factors influencing the acceptance of medical autopsies.
A prospective survey was performed using questionnaire that was administered to patients and health care professionals of the Saint-Étienne university hospital. The survey consisted in several questions relating to the autopsy practice (on one's own body) and the knowledge of the procedure.
The population surveyed consisted of 186 individuals and 112 health care professionals, including physicians, residents and nurses. The results of the study overall showed a good acceptance of autopsy. Actually, 86% of the individauls and 94.6% of health professionals would not be opposed to their own autopsy (in order to identify the cause of the death, to help relatives bereavement, to foster medical research). The main reasons of medical autopsy opposition include religion belief, body integrity, dignity and respect. Only 42.5% of patients had previously received some information about autopsies, while 33.9% of health care professionals had received a specific formation.
The rate of refusal of medical autopsy was low in this survey but information relative to this procedure remains insufficient. The current law imposes to look after a nonopposition to medical autopsy of the died individual, including asking his/her relatives that markedly limit the organ donation. Modification of the French bioethical law should be beneficial to increase both medical autopsy organ donation rates.
La Revue de Médecine Interne 10/2010; 32(4):205-11. · 0.90 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Camptocormia refers to an abnormal posture with flexion of the thoraco-lumbar spine which increases during walking and resolves in supine position. This symptom is an increasingly recognized feature of parkinsonian and dystonic disorders, but may also be caused by neuromuscular diseases. There is recent evidence that both central and peripheral mechanisms may be involved in the pathogenesis of camptocormia. We report a case of acute onset of camptocormia, a rare side effect induced by olanzapine, a second-generation atypical anti-psychotic drug with fewer extra-pyramidal side-effects, increasingly used as first line therapy for schizophrenia, delusional disorders and bipolar disorder.
A 73-year-old Caucasian woman with no history of neuromuscular disorder, treated for chronic delusional disorder for the last ten years, received two injections of long-acting haloperidol. She was then referred for fatigue. Physical examination showed a frank parkinsonism without other abnormalities. Routine laboratory tests showed normal results, notably concerning creatine kinase level. Fatigue was attributed to haloperidol which was substituted for olanzapine. Our patient left the hospital after five days without complaint. She was admitted again three days later with acute back pain. Examination showed camptocormia and tenderness in paraspinal muscles. Creatine kinase level was elevated (2986 UI/L). Magnetic resonance imaging showed necrosis and edema in paraspinal muscles. Olanzapine was discontinued. Pain resolved quickly and muscle enzymes were normalized within ten days. Risperidone was later introduced without significant side-effect. The camptocormic posture had disappeared when the patient was seen as an out-patient one year later.
Camptocormia is a heterogeneous syndrome of various causes. We believe that our case illustrates the need to search for paraspinal muscle damage, including drug-induced rhabdomyolysis, in patients presenting with acute-onset bent spine syndrome. Although rare, the occurrence of camptocormia induced by olanzapine must be considered.
[Show abstract][Hide abstract] ABSTRACT: There are no specific studies evaluating the benefit/risk of antithrombotic prophylactic treatment in patients hospitalised in a palliative care unit. So, the aim of this study was to evaluate the clinicians attitudes about antithrombotic prophylaxis for patients in palliative care units and the elements which determined their decisions.
The clinical data of 4 terminally ill patients were extracted from database of a Palliative Care Unit in France. These 4 patients were selected as they represented several different situations according to the presence or not of major thrombotic risk factors, bleeding risk factors, and request of compassionate care. Through an open questionnaire, fourteen clinicians usually in charge of palliative care patients were individually interviewed about antithrombotic prophylactic therapy for each case of patients.
except in the case of a patient with major thrombotic risk factors, no bleeding risk factor and wishing to receive active care, both the attitudes of clinicians to initiate or continue a prophylactic therapy and the elements which lead to their decisions were heterogeneous at least.
the absence of recommendations based on validated clinical trials evaluating the efficacy and safety of thromboembolism prophylactic treatment in palliative care patients lead to uncertain decisions for clinicians. Added to an objective evaluation of thrombotic and hemorrhagic risks factors, the wish of patient to receive or not active care is probably the most important element to consider.
La Presse Médicale 05/2009; 38(9):1235-9. · 0.87 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: "Atypical" antipsychotics tend to replace traditional antipsychotics as first line therapy for psychotic disorders, due to their better side-effect profile with fewer extrapyramidal manifestations, allowing a better observance. Nevertheless, second-generation antipsychotics may also lead to adverse events such as metabolic disorders, agranulocytosis or muscle damage. Cases of rhabdomyolysis (aside neuroleptic malignant syndrome) have been reported in patients receiving olanzapine (Zyprexa).
We reviewed the cases of olanzapine induced rhabdomyolysis reported to the French national database of drug adverse events and retrieved additional cases published in the medical literature.
We collected 13 cases from the French pharmacovigilance database and eight additional cases from the literature. Seventeen patients needed hospitalization. Creatine kinase (CK) rate ranged between 413 and 34,500 UI/L. Outcome was favorable in 85% of the cases (17 out of 20 cases) after discontinuation of olanzapine.
Although rhabdomyolysis is a rare side effect (< 1%) of olanzapine, this adverse event should be evoked when a patient with olanzapine presents with muscle pain, unexplained fatigue or weakness. Prompt dosage of CK should be performed. However, it remains uncertain whether a mild and asymptomatic muscle enzyme increase without any metabolic disorder requires the discontinuation of olanzapine therapy.
La Revue de Médecine Interne 04/2009; 30(6):477-85. · 0.90 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Chronic-granulomatous disease (CGD) is a rare inherited primary immunodeficiency syndrome caused by a defective oxidative metabolism of phagocytic cells. Dysfunction of the membranous NADPH oxidase complex leads to a greatly increased susceptibility to severe fungal and bacterial infections, early in childhood. The most severe and frequent type of GCD is the X-linked transmitted form caused by mutations in the CYBB gene encoding the redox element of the oxidase complex, gp91phox or Nox2. However, very rare autosomal recessive CGD affecting other oxidase components than Nox2 are characterized by mild-clinical manifestations that could appear later at the adult age. Long-term antibiotic prophylaxis is essential to prevent infections associated with CGD, but approaches based on hematopoietic stem-cell transplantation and gene therapy offer valuable hope in a near future.
La Revue de Médecine Interne 03/2009; 30(3):221-32. · 0.90 Impact Factor