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Biomedical subjects

Olivier Fain

Publications and source records attributed to Olivier Fain.

14 recordsLinked to original sources

[Macrophage activation syndrome].

Macrophage activation syndrome is due to macrophage stimulation secondary to excessive cytokine secretion. Infectious (mainly viral) and neoplastic (lymphomas primarily) diseases are the mainly triggering circumstances of this syndrome associated with immunodepression. Signs are fever, hepatosplenomegaly, lymphadenopathy, cytopenia, deranged liver function increases level of lactate dehydrogenase, triglyceridemia and serum ferritin. Diagnosis is established by the presence of cellular phagocytosis by macrophage on medullary or hepatic biopsy. It is a potentially life threatening condition. Treatment of the triggering circumstance associated with corticosteroids, chemotherapy (etoposide) or intravenous immunoglobulins, according to etiology, are necessary.

Histiocytosis, Non-Langerhans-Cell↗

Esophageal function and Sjögren's syndrome.

The frequency and characteristics of esophageal dysmotility in Sjögren's syndrome (SS) are as controversial as their related symptoms. We evaluated esophageal function and gastroesophageal reflux (GER) in 21 SS patients using manometry and 24-hr esophageal pH monitoring. All patients complained of xerostomia, 33% of dysphagia, and 62% of heartburn. Compared to controls, the mean percentage abdominal length of their lower esophageal sphincters (LES) and resting LES pressures were significantly lower, with no difference in primary esophageal peristalsis. Tertiary waves without swallowing were detected in 29% of them and pathological GER in 67%. Symptoms, esophageal motor abnormalities, and reflux features were similar in primary and secondary SS. ANOVA indicated that dysphagia was unrelated to the esophageal impairments and GER analysis results, while heartburn was significantly associated with GER severity. Esophageal acid-exposure time was significantly longer in SS patients with distal tertiary waves, while proximal esophagus wave velocity was significantly lower. While SS patients have nonspecific esophageal motility disorders and frequently GER disease, early and accurate diagnosis of GER is essential to identify SS patients at risk for acidic reflux, especially because the acid-clearance capacity of the esophagus is already diminished by the lack of saliva.

Adult↗

[Epidemiologic, clinical, biological and therapeutic aspects of Gaucher disease].

GENERAL CHARACTERISTICS: Gaucher's disease is a genetic disease of autosomal recessive transmission due to a deficit in a lysosomal enzyme: beta-glucocerebrosidase. The disease is characterised by deposits of glucosylceramide in the cells of the liver, spleen and bone marrow. Acute or chronic neurological forms (type 2 and 3) account for only 5% of patients suffering from Gaucher's disease and are less frequent than the non-neurological forms (type 1). CLINICAL AND BIOCHEMICAL MANIFESTATIONS: Gaucher's disease is associated with spleno- or hepato-megalia, asthenia, bone complications (Erlenmeyer flask deformity, osteopenia and osteonecrosis), as well as with haematological (thrombopenia, anaemia) or biochemical abnormalities (increase in angiotensin-converting enzyme, ferritin, tartrate-resistant acid phosphatase and chitotriosidase). Central nervous system involvement is only found in the type 2 and 3. Diagnosis relies on measurement of beta-glucocerebrosidase activity in the circulating leukocytes. REGARDING TREATMENT: Treatment with enzyme replacement (imiglucerase: recombinant enzyme preparation) improves the haematological abnormalities, hepatosplenomegalia and quality of life in a matter of a few months. Regression of the bone disorders is usually observed only after 3-4 years of treatment. Recently, gene therapy trials have successfully been started.

Chromosome Aberrations↗

Hypovitaminosis C in hospitalized patients.

BACKGROUND: In recent years, cases of scurvy have mainly been described in populations at risk. The prevalence and risk factors for hypovitaminosis C among hospitalized patients in a department of internal medicine are largely unknown. METHODS: We determined serum ascorbic acid level (SAAL) and searched for clinical and biological signs of scurvy in 184 patients hospitalized during a 2-month period. RESULTS: The prevalence of hypovitaminosis C (depletion: SAAL<5 mg/l or deficiency: SAAL<2 mg/l ) was 47.3%. Some 16.9% of the patients had vitamin C deficiency. There was a strong association between hypovitaminosis C and the presence of an acute phase response (p=0.002). Other univariate risk factors for vitamin C depletion were male sex (p=0.02), being retired (p=0.037), and infectious diseases (p=0.002). For vitamin C deficiency, the significant univariate risk factors included the same ones found for vitamin C depletion, plus being unemployed (p=0.003) and concomitant excessive alcohol and tobacco consumption (p<0.0001). Logistic regression showed that being retired (p=0.015) and concomitant excessive alcohol and tobacco consumption (p=0.0003) were significant independent risk factors. Hemorrhagic syndrome and edema were described more often in patients with vitamin C deficiency than in those with vitamin C depletion or without hypovitaminosis. Clinical signs were more frequent for an ascorbic acid level below 2.5 mg/l. CONCLUSION: Hypovitaminosis C is frequent in hospitalized patients but should be interpreted according to the presence or absence of an acute phase response. The main risk factors are living conditions and excessive alcohol and tobacco consumption.

Journal Article↗

Septic shock caused by Ochrobactrum anthropi in an otherwise healthy host.

Reported is a case of life-threatening septic shock that occurred in an otherwise healthy host after administration of a peripheral venous infusion of a solution contaminated with Ochrobactrum anthropi, an unusual human pathogen. The rapid onset of shock may have been due to a large inoculum caused by nonsterile practices at the time of reconstitution.

Adult↗

Utilization of 16S ribosomal DNA sequencing for diagnosis of septicemia due to Neisseria elongata subsp. glycolytica in a neutropenic patient.

Septicemia due to Neisseria elongata subsp. glycolytica occurs infrequently. We report a case of septicemia in a patient undergoing antimitotic chemotherapy. Gram-negative coccobacilli were isolated from blood cultures. The identity of the isolate by phenotypic methods was uncertain. In contrast, identity was confirmed by 16S ribosomal DNA sequencing, which appeared to be very useful for correct identification.

Aged↗

[Extrathoracic tuberculosis].

Incidence of extrathoracic tuberculosis has increased these last years. The average time to diagnosis is 3 months, owing to the poor specificity of some of its clinical signs, but also the failure to evoke these infections. Most common localizations are lymph nodes, bones and joints, genitourinary track and more rarely neuromeningeal or digestive systems. Diagnosis is based on bacteriological data (traditional culture, polymerase chain reaction) and histology. Computed tomography and magnetic resonance imaging are effective to detect the lesions at an early stage and appreciate their extent. Delayed diagnosis is responsible for mortality and sequelae of this affection for which treatment is effective, if it is correctly managed and followed.

Bone and Bones↗

[Evaluation of medical semiology teaching and teachers. Study of student satisfaction in the second cycle first year].

OBJECTIVE: To know the students' opinion concerning semiology teaching and teachers. METHODS: Annual enquiry between 1990-91 and 2000-01, before and after a reform increasing to 400 the hours dedicated to medical clerkships in the second and third years of the courses. RESULTS: Before the reform, at least 62% of the students were in favor of the general organization, while a growing percentage (reaching 79.6%; p < 0.0001) considered the number of hours dedicated to clinical clerkship as insufficient. Concerning the teachers, the assessment concerning the "assistant heads of departments" (AHD) showed great disparities. Out of 100 teachers-year, 4 obtained a score lesser than 10/20 and 59 greater than 15/20. After the reform, 19 AHD out of 33 obtained more than 15/20 and 5 less than 10/20. The reintroduction of lectures was not criticized. The professors who gave lectures were reproached by the students for delaying them or for not having given them at all. They were also reproached for their lack of relationship with the teaching objectives. The scores allocated to professors ranged from 4.5 to 15.9/20. CONCLUSION: Enhancing the value of teaching for an AHD's university career and reducing the administrative burden for all teachers would be useful to improve medical education, especially in semiology.

Attitude of Health Personnel↗

Intravenous immunoglobulin or high-dose methylprednisolone, with or without oral prednisone, for adults with untreated severe autoimmune thrombocytopenic purpura: a randomised, multicentre trial.

BACKGROUND: Treatment of adults with autoimmune thrombocytopenic purpura (AITP) is based more on individual experience than on results of controlled studies. We compared intravenous immunoglobulin with high-dose methylprednisolone in untreated adults with severe AITP and assessed efficacy of subsequent oral steroids compared with placebo. Primary outcome was number of days with platelet count greater than 50 x 10(9)/L within the first 21 days. METHODS: We did a randomised multicentre trial based on a 232 design. 122 adults with severe AITP (platelet count < or =20 x 10(9)/L) were randomly assigned to receive either intravenous immunoglobulin or high-dose methylprednisolone on days 1-3 (randomisation A), and then to receive either oral prednisone or placebo (randomisation B) on days 4-21. Analysis was by intention to treat. FINDINGS: Six patients were excluded from the analysis. The number of days on which platelet counts were above 50 x 10(9)/L was 18 in 56 patients receiving intravenous immunoglobulin and 14 in 60 receiving high-dose methylprednisolone (p=0.02). Percentage of patients who had platelet counts over 50 x 10(9)/L on days 2 and 5 was 7% and 79%, respectively, in the intravenous immunoglobulin group compared with 2% and 60%, respectively, in the high-dose methylprednisolone group (p=0.04). During the second treatment period, prednisone was more effective than placebo for all short-term endpoints. Patients who received intravenous immunoglobulin and prednisone had platelet count greater than 50 x 10(9)/L for 18.5 days (p=0.005), and those treated with high-dose methylprednisolone and prednisone had this count for 17.5 days. INTERPRETATION: Intravenous immunoglobulin and oral prednisone seems to be more effective than high-dose methylprednisolone and oral prednisone in adults with severe AITP, although the latter treatment is effective and well tolerated.

Adult↗

[Neuromeningeal tuberculosis in northeastern suburbs of Paris. Nineteen cases].

SUBJECT: Analyze characteristics of neuromeningeal tuberculosis in the northeastern suburbs of Paris. MATERIAL AND METHOD: Retrospective study of 19 observations between 1988 and 1999. RESULTS: Thirteen cases of meningitis, 3 cases of meningitis associated with a tuberculoma and 3 cases with isolated tuberculoma were described. Young subjects (average age 46.4), foreign born (73.7%) were preferentially affected. Three patients were infected by the human immunodeficiency virus. Weight-loss and weakness (84%), fever (79%), headache (63%) dominate the clinical manifestations. The diagnosis delay was 56.6 days, and the therapeutic time, 8.6 days. The search for acid fast bacilli was positive only in one case on direct examination of the cerebrospinal fluid, whereas the culture was positive in 68.7% of the cases. Cerebral imagery was abnormal in13 patients. Average duration of antituberculous treatment was 13,4 months (range: 9-24). Corticosteroids were associated in 12 patients. Thirteen patients were cured, 3 died and 3 were lost to follow up. CONCLUSION: The characteristics of neuromeningeal tuberculosis are not modified, but the diagnosis delay of the neuromeningeal tuberculosis, which conditions the prognosis, remains significant. The optimal duration of the antituberculous treatment and the conditions of the steroid therapy have to be specified.

Adult↗