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Biomedical subjects

Marc Ruivard

Publications and source records attributed to Marc Ruivard.

10 recordsLinked to original sources
PubMed2006

Plasma exchanges do not increase red blood cell transfusion efficiency in severe autoimmune hemolytic anemia: a retrospective case-control study.

Severe autoimmune hemolytic anemia (AIHA) can cause life-threatening hemolysis requiring red blood cell transfusions (RBT). The efficiency of RBT could be improved with the use of plasma exchanges (PEx) before RBT. The objective of this study was to assess the effectiveness of PEx in severe AIHA of adults. All adult patients with severe AIHA requiring RBT were included in this single center retrospective case-control study. The end point was change in hemoglobin (Hb) level after RBT, depending on whether PEx was done (experimental group) or not (control group). Thirty-one sessions of RBT following PEx were performed on the 5 patients of the experimental group and were compared with the 7 sessions of BT without PEx performed on the 4 patients of the control group. Despite a lower mean Hb value before the session of RBT (5.7 g/dl vs. 7.2 g/dl, P = 0.04) in the control group, the mean Hb value 5 (4-7) days following RBT (8.7 g/dl vs. 8.6 g/dl, P = 0.85) was not different in the experimental and in the control group, respectively. In a second analysis in which each patient was his own control (31 sessions of RBT following PEx vs. 51 sessions of RBT alone), the gain in Hb was not different (1.4 g/dl vs. 1.9 g/dl, P = 0.67). When RBT are required in severe AIHA of adults, the use of a single PEx before BT does not increase the efficiency of RBT based on day 5 evaluation.

Adrenal Cortex Hormones↗
PubMed2005

Regressive aortic valve and infundibular tumors during idiopathic hypereosinophilic syndrome.

Fibroblastic endocarditis is a classic complication of prolonged hypereosinophilic syndrome, whatever the cause. In France, it is most frequently encountered in cases of idiopathic hypereosinophilic syndrome. It commonly involves the apex of the ventricles, with a clinical picture of restrictive cardiomyopathy of unfavorable prognosis, and the auriculoventricular valves. We report the case of a 77-year-old man in whom atypical cardiac involvement disclosed idiopathic hypereosinophilic syndrome. In addition to the usual features of obliteration of the apex and restrictive cardiomyopathy, echocardiographic examination showed severe left ventricular dysfunction and intracardiac tumors, one of which was unusually localized to an aortic valve. Treatment, which comprised strict control of the eosinophilic process, standard treatment for cardiac failure, and anticoagulation therapy, produced rapid and long-lasting improvement of his clinical status and left systolic and diastolic ventricular function, and on echocardiography the intracardiac tumors had totally disappeared. The patient suddenly died of septic shock 16 months after first being seen.

Aged↗
PubMed2004

Autoimmune thrombocytopenic purpura and common variable immunodeficiency: analysis of 21 cases and review of the literature.

To describe the main characteristics and outcome of autoimmune thrombocytopenic purpura (AITP) in patients with common variable immunodeficiency (CVID), we analyzed data from 21 patients and reviewed additional cases from the literature. To be included in this study, patients had to have CVID and a previous history of AITP with a platelet count < or = 50 x 10(9)/L at onset. A complete response to treatment was defined by a platelet count > or = 150 x 10(9)/L, and a partial response by a platelet count >>50 x 10(9)/L with an increase of at least twofold the initial level. The median platelet count at AITP diagnosis was 20 x 10(9)/L (range, 2-50 x 10(9)/L). The median age at AITP diagnosis was 23 years (range, 1-51 yr), whereas the median age at CVID diagnosis was 27 years (range, 10-74 yr). CVID was diagnosed before the onset of AITP in only 4 patients (19%), 3 of whom were being treated with intravenous immunoglobulin (i.v.Ig) replacement therapy. CVID was diagnosed more than 6 months after AITP in 13 cases (62%), and the 2 conditions were diagnosed concomitantly in 4 cases. Eleven patients (52%) had at least 1 autoimmune manifestation other than AITP, among which autoimmune hemolytic anemia (7 cases) and autoimmune neutropenia (5 cases) were preeminent. Seventeen of the 21 patients (80%) received at least 1 treatment for AITP; 13 patients received corticosteroids alone and 7 (54%) achieved at least a partial response; 8 patients received i.v.Ig at 1-2 g/kg alone or in combination with steroids, leading to a short-term response rate of 50%. Four patients underwent a splenectomy (2 complete responses, 2 failures); 2 additional splenectomies were performed for associated autoimmune hemolytic anemia. With a mean follow-up of 5.6 years after the surgical procedure, none of the 6 splenectomized patients had a life-threatening infection. With a median follow-up after AITP onset of 12 years, 13/21 patients (62%) were in treatment-free remission (7 complete responses, 6 partial responses), 7 patients (23%) were in remission while on prednisone < or = 20 mg/day with or without azathioprine, and only 1 patient still had a platelet count <50 x 10(9)/L. Five patients had died at the time of the analysis; none of the deaths was related to a hemorrhage. Severe infections including 3 fatal bacterial infections and 2 opportunistic infections occurred in 6 patients during or after treatment of AITP. In conclusion, AITP, alone or in combination with autoimmune hemolytic anemia (Evans syndrome) and/or autoimmune neutropenia, is frequent in patients with CVID, and is not prevented by i.v.Ig substitutive therapy. Since AITP frequently precedes the diagnosis of CVID, testing for immunoglobulin levels should be performed in every patient diagnosed with AITP. Steroids and splenectomy seem to have the same efficacy as in idiopathic AITP, but the increased risk of severe infections must be taken into consideration.

Adolescent↗
PubMed2003

Treatment of polyarteritis nodosa and microscopic polyangiitis with poor prognosis factors: a prospective trial comparing glucocorticoids and six or twelve cyclophosphamide pulses in sixty-five patients.

OBJECTIVE: Because the optimal cyclophosphamide (CY) treatment duration for severe polyarteritis nodosa (PAN) without virus infection and microscopic polyangiitis (MPA) has not been established, we conducted a trial to compare the effectiveness of 6 versus 12 CY pulses given in combination with corticosteroids (CS). METHODS: Sixty-five (18 PAN, 47 MPA) previously untreated patients were randomized to receive 12 (n = 34) or 6 (n = 31) CY pulses combined with CS. PAN and MPA were histologically proven or met ACR criteria. All patients presented >or=1 factor of severity according to the five factor score (FFS). CY pulses were administered every 2 weeks for 1 month, then every 4 weeks. The end point of the study was the number of events (relapses and/or deaths) occurring in each group, analyzed according to an intention-to-treat strategy. The outcome was evaluated by Cox proportional hazards analysis. RESULTS: The baseline characteristics were similar for both groups. The mean (+/- SD) followup was 32 +/- 21 months. Survival analysis showed a significantly lower relapse probability (P = 0.02; hazards ratio [HR] = 0.34) and higher event-free survival (P = 0.02, HR = 0.44) for the 12 CY-pulse group while the mortality rates were not significantly different (P = 0.47). CONCLUSION: These results suggest that 6 CY pulses are less effective than 12 CY pulses to treat severe PAN and MPA, particularly with respect to the risk of relapses.

Adult↗
PubMed2003

Dietary iron regulates hepatic hepcidin 1 and 2 mRNAs in mice.

Recently discovered peptide-hepcidin (Hepc) may be a central player in the communication of iron body stores to the intestinal absorptive cells and thus involved in the maintenance of iron homeostasis. The aim of this study was to determine the effects of the level of dietary iron on Hepc gene expression in the liver. OF1 male mice were fed for 3 weeks either control diet (35 mg iron/kg diet), low-iron diet (1 mg iron/kg diet), or high-iron diet (500 mg iron/kg diet), and Hepc 1 and 2 mRNA abundance in the liver was assessed by reverse transcriptase-polymerase chain reaction (RT-PCR). Results clearly showed that Hepc gene expression is upregulated by high dietary iron and downregulated when the dietary iron level is low. Both Hepc 1 and Hepc 2 expression responds coordinately to dietary iron. This work provides additional evidence of the key role of Hepc in the regulation of iron homeostasis.

Actins↗
PubMed2003

Physician compliance with outpatient oral anticoagulant guidelines in Auvergne, France.

OBJECTIVE: Oral anticoagulants (OA) are commonly used, but they can lead to severe bleeding. We studied the indications and monitoring of OA in patients treated by general practitioners. DESIGN: Retrospective cross-sectional study. SETTING: Primary care. PATIENTS: Four hundred thirty-eight randomly selected patients of a population of 2,452 patients treated with OA were studied. INTERVENTIONS: We compared the indications for OA as reported by general practitioners with indications as defined according to recent guidelines. MAIN RESULTS: Twenty-five percent of patients should not have been treated with OA. Inappropriate indications (13% of patients) were atrial fibrillation without risk factor (3.9%), prior uncomplicated myocardial infarction (2.7%), peripheral arterial disease (2.7%), superficial thrombophlebitis (2.3%), and atherothrombotic ischemic stroke (1.6%). For 12% of patients, the duration of OA was too long (venous thromboembolism without permanent risk factor in 10%). Frequency of International Normalized Ratio (INR) measurement was insufficient for 14% of patients and target INR was not achieved in 31%. CONCLUSIONS: Our study demonstrated that clinicians' adherence to recommendations regarding indications for OA and management of this treatment should be improved. Implementation of anticoagulation clinics is probably needed.

Administration, Oral↗
PubMed2003

[Mesenteric venous thrombosis. A retrospective study of 23 cases].

UNLABELLED: Mesenteric venous thrombosis is uncommon. Diagnosis is difficult and much remains to be learned about clinical aspects, etiology and treatment options. PATIENTS AND METHODS: We conducted a retrospective study over 5 years concerning patients with isolated mesenteric venous thrombosis or with extension to the portal vein. RESULTS: Twenty-three patients (12 women), mean age 55 years, were included. Abdominal pain (96%), bowel obstruction (35%), fever (26%), diarrhea (17%), gastrointestinal bleeding (13%) were the most common symptoms at presentation. In 78% of patients, at least one underlying condition was identified: thrombophilia, oral contraceptive, cancer, paroxysmal nocturnal hemoglobinuria, essential thrombocythemia, intra-abdominal processes. CT scan and ultrasound diagnostic sensitivity were 89% and 57% respectively. Outcome was favorable for the 18 patients treated with anticoagulants (associated with surgery in 4 patients). Three patients with neoplasm died early. CONCLUSION: Mesenteric venous thrombosis must be kept in mind as a possible diagnosis in patients with abdominal pain. Predisposing factors are frequently associated and should be searched for. Early diagnosis, using CT scan or ultrasound, enables immediate anticoagulation and seems to improve outcome.

Abdominal Pain↗
PubMed2002

Severe methylenetetrahydrofolate reductase deficiency revealed by a pulmonary embolism in a young adult.

Deficiency in methylenetetrahydrofolate reductase (MTHFR), the enzyme involved in the remethylation of homocysteine to methionine using methyltetrahydrofolate as cofactor, induces hyperhomocysteinaemia, homocysteinuria, hypomethioninaemia and low methylfolate levels. Diagnosis usually occurs during infancy because of various neurological abnormalities. We report MTHFR deficiency diagnosed in an adult woman after a pulmonary embolism. Her adult sister, intellectually retarded, suffered from the same disease. Molecular analysis of the MTHFR gene exhibited four different mutations (two missense mutations, one exon skipping and C677T). The impact of these mutations was analysed through the biological abnormalities in the parents and children.

Adult↗