Human immunodeficiency virus-associated thrombotic microangiopathies: clinical characteristics and outcome according to ADAMTS13 activity.

Human immunodeficiency virus (HIV) infection is a risk factor for thrombotic microangiopathy (TMA). We sought whether a severe deficiency in ADAMTS13, the enzyme specifically involved in the cleavage of von Willebrand factor, was associated with specific presenting features and outcome in HIV-associated TMA. In this prospective, multicentre, case-control study, 29 patients of 236 in the French Network on TMA had an HIV-associated TMA. Seventeen patients with severe ADAMTS13 deficiency (ADAMTS13 <5% HIV(+) group) were compared to 12 patients with a detectable ADAMTS13 activity (ADAMTS13 >or=5% HIV(+) group). HIV(+) patients were also compared to 62 patients with idiopathic TMA, either with (45 patients, ADAMTS13 <5% idiopathic group) or without (17 patients, ADAMTS13 >or=5% idiopathic group) severe ADAMTS13 deficiency. ADAMTS13 <5% HIV(+) patients had less AIDS-related complications than ADAMTS13 >or=5% HIV(+) patients (23.5% versus 91.6%, respectively, P = 0.0005) and their median CD4(+) T cell count was higher (P = 0.05). TMA-associated death rate was higher in ADAMTS13 >or=5% HIV(+) patients than in ADAMTS13 <5% HIV(+) patients (50% versus 11.7%, respectively, P = 0.04). In ADAMTS13 <5% patients, TMA-associated death rate was comparable between HIV(+) and idiopathic patients (15.5% in idiopathic patients, P-value was non-significant). By contrast, TMA-associated death rate in ADAMTS13 >or=5% HIV(+) patients was higher than in idiopathic patients (11.7% in idiopathic patients, P = 0.04). In conclusion, HIV-associated TMA with severe ADAMTS13 deficiency have less AIDS-related complications and a higher CD4(+) T cell count. TMA prognosis is better and comparable to this of idiopathic forms.

What are the indications for plasma exchanges in autoimmune diseases?: The registry of the Société Française d'Hémaphérèse.

The use of plasma exchange (PE) in the autoimmune diseases is encouraged in France. Since 1990, the national registry of PE allows an analysis of the evolution of the coverage of these pathologies. The variation of the number of patients treated by PE is correlated in respect to the results of the therapeutic studies. After a decrease of PE activity in these indications during the 90s, one observes a new increase of the patients treated because of the validation of new indications. Autoimmune diseases represent the third cause of morbidity in developed countries, with a global prevalence of 5%, and concerns four groups of pathologies of organs or systems (neurology, haematology, nephrology and vasculitis). In 1976, Lockwood demonstrated the place of plasma exchanges (PE) in Goodpasture's syndrome [Lockwood CM, Rees AJ, Pearson TA, Evans DJ, Peters DK, Wilson CB. Immunosuppression and plasma exchange in the treatment of Goopasture's syndrome. Lancet 1976;1(7962):723-6. [1]], with a significant decrease of antibodies during large volume exchanges. In the 80s, several prospective studies began to estimate the efficiency of PE in other autoimmune diseases. The national registry of the Société Française d' Hémaphérèse, has collected the epidemiological and technical data of PE since 1985. This work analyses the evolution of the validated indications, between 1990 and 2005 in France.

World apheresis association--world apheresis registry.

In 2002 WAA decided to start a world-wide apheresis registry to gain insight into the extent of treatment, adverse events, and to facilitate contacts among centers when treatment indications are rare and experience limited. Stem cell and other blood products collections intended for therapeutic application can also be entered. The WAA planned to use the French Registry. Its translation into English has not been accomplished and the fiscal obligations for that registry has not, as yet, been determined or considered and approved by the WAA Board. From Dec 2002 the proposed registry (a merged version of the French, Canadian and Swedish registries) can be immediately implemented. We now cordially invite all centers to join that registry. Please, also inform colleagues at other centers in your country to join. E-mail and address lists of colleagues in your country who have not registered will be welcomed. The site is at: Go to World Apheresis Registry; Login code to test the Registry is: al61tms. Then apply for a specific login code for your center. We welcome you to this registry for your input of data. You will not be charged any registration fee. The registry includes a randomization system that can be used for local or multi center studies (randomization by in-center basis allows you to make your own studies). It includes a formula that increases the chance to get a more even distribution between groups also for smaller sample sizes.

Role of replacement fluids in the immediate complications of plasma exchange. French Registry Cooperative Group.

OBJECTIVE: Comparison of side effects (fever shivering and/or allergic reactions, collapse, symptomatic hypocalcaemia) of the different substitution fluids used during therapeutical plasmapheresis. DESIGN: Multicentric retrospective comparative study designed to explore the side effects due to the replacements fluids (albumin alone or associated with dextran 40, modified fluid gelatin, hydroxyethyl starch), and to determine the best combination in patients undergoing plasmapheresis. PATIENTS: 46,895 procedures were performed on 5,167 patients between 1990 and 1994. The analysis concerns 33,962 sessions, divided into two groups (group 1: 21,079 sessions from 1990 to 1992 comparing albumin alone versus combined albumin-macromolecules and the different combinations among these, group 2: 12,883 sessions in 1993-1994 comparing albumin alone versus albumin-hydroxyethyl starch). Because of the diversity of the products used, 13,029 sessions were not studied because groups of small sample sizes were formed. MEASUREMENTS AND RESULTS: In group 1, the comparison of albumin + macromolecules with albumin alone, shows the best haemodynamic tolerance, lower frequency of fever shivering and/or allergic reactions in the albumin alone subgroup. On the other hand, hypocalcaemia is significantly higher in this subgroup. The combinations albumin + dextran 40 (but after hapten inhibition) and albumin + hydroxyethyl starch are equivalent. Albumin + modified fluid gelatin is the combination presenting the highest incidence in terms of allergic manifestations. In group 2, albumin + hydroxyethyl starch versus albumin alone, the former is preferable in terms of the three side effects studied. CONCLUSION: The results of this study show a combination of albumin 4% hydroxyethyl starch to replace plasma during plasma exchange to be the method presenting the lowest morbidity and the best cost/effectiveness ratio.

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.

Plasma exchange in France: Epidemiology 2001.

The French plasma exchange registry created in 1985 lists the indications, techniques and complications of the French therapeutic haemapheresis. In 2001 it contains the data of more than 16,700 patients for a total of 153,641 sessions. The indications concern five groups of pathologies (neurology, haematology, nephrology, vasculitis, and endocrinology). Until 2000, the neurology represented the most important group but the use of the high dose IgIV for Guillain-Barre and myasthenia gravis decreased the indications. The haematology became most important group treated because of the increase of the TTP and HUS number treated. The endocrinology (familial hypercholesterolemia) represents at present 10% of the patients treated for 18.7% of the sessions. The vascular access little changed since 1985, the peripheral venous access being the most used. The plasma substitution initially based on the albumin alone was gradually replaced by an association albumin macromolecules, in particular hydroxyethylstarch since 1990. After the observation of the side effects due to starches we observed an increase of the albumin alone use. The immediate complications decreased in half in 15 years. The French plasma exchange registry is the largest world database of haemapheresis with the cooperation of about 80 centres, allowing numerous scientific studies.

14 years of therapeutic plasma exchange in France.

The French Registry for plasma exchange (PE) was set up in 1985. For 14 years it has allowed analysis of the techniques used along with the indications and complications. Recent analysis shows a slight fall in activity as some studies have ended, while the neurological disorders remain the most frequent indications for PE. The important changes observed over the years are the increased use of the centrifugation technique, the development of plasma and whole blood treatment and plasma substitution using a mixture of albumin and pentastarch. The French Registry for PE is the largest such database which, along with the Canadian Registry for therapeutic hemapheresis, allows both retrospective and prospective studies.

Digoxin poisoning and anuric acute renal failure: efficiency of the treatment associating digoxin-specific antibodies (Fab) and plasma exchanges.

Digoxin-specific antibodies (Fab) are currently the treatment of choice for digoxin intoxication. These fragments bind to digoxin, leading to Fab-digoxin complexes, and promote the release of receptor-bound digoxin. These complexes are renally excreted. In the case of anuria, they could be dissociated and lead to renewed intoxication. In this case plasma exchanges are proposed. We report the case of an anuric patient with digoxin intoxication, treated with a Fab injection, followed by a plasma exchange 16 hours later, a second Fab injection was given followed by two plasma exchanges, 38 and 86 hours later. The disappearance of cardiac abnormalities showed the efficiency of the Fab, the drop in serum digoxin concentration and the high digoxin concentration in the exchanged plasma indicate effective elimination. The association of Fab and plasma exchanges could be proposed in the case of digoxin intoxication in the anuric patient.

[Campylobacter fetus subspecies fetus endoaortitis on a Bentall tube prosthesis. Apropos of a case]. / Endoaortite à Campylobacter fetus sous-espèce fetus sur tube prothétique de Bentall. A propos d'un cas.

Campylobacter fetus is a rare cause of endocarditis and endoaortitis: the authors believe this to be the second reported case of infection of an intracardiac prosthesis. The patient was a man who had already undergone replacement of the aortic valve and ascending aorta, and a gastrectomy, which were predisposing factors. The portal of entry was not found. The diagnosis was confirmed by positive blood cultures and transoesophageal echocardiography. The outcome was rapidly fatal despite antibiotic therapy and surgery, because of the seriousness of the lesions (pseudo-aneurysm of the aorta ruptured into the right atrium), the precarity of the terrain and surgical difficulties.

Incidence and causes of carbon monoxide intoxication: results of an epidemiologic survey in a French department.

The purpose of this study was to estimate the incidence, mortality, and causes of carbon monoxide intoxications in France. A survey was conducted in the department of the Hauts-de-Seine, which is representative of the French population. Data were collected through a headquarters that had direct contact with all department emergency organizations and to a technical laboratory. During the 3-y study, 735 cases that were related to 291 events were reported. Thirty-six patients died. The average incidence of carbon monoxide intoxications was 17.5 per 100,000 inhabitants. Poisoning was caused by fires in 36 events and by car exhausts in 12. For the remaining events, causes were determined as follows for 196: water heaters (57%), boiler (20.5%), coal stove (9%), brazier (4%), cooker (2%), heating device (1.5%), and miscellaneous (6%). The main mechanisms of intoxication were a defective device, poor ventilation, or poor evacuation of combustion gases. Carbon monoxide intoxication occurs frequently in France, and preventive actions are warranted.

[Severe methotrexate poisoning]. / Intoxication grave au méthotrexate.

Methotrexate toxicity is rare but extremely severe. When complete, it consists of ulcerations of the gastrointestinal mucosae responsible for necrotizing enteritis, erythroderma, bone marrow aplasia, interstitial pneumonia, hepatitis and organic renal failure with diuresis. Toxicity is facilitated by pre-existing renal impairment, third sector and abstention or underdosage of foliculinic acid prescribed as antagonist. The diagnosis rests on serum assays, the results of which must be interpreted taking into account the assay method and the time elapsed between the injection of methotrexate and its assay in serum. The multivisceral pathology observed may totally regress, as in the case reported here. Treatment is based on symptomatic measures, starting with maintenance of an abundant and alkaline diuresis, and on the parenteral administration of folinic acid in doses that vary with the authors.