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Biomedical subjects

E Andrès

Publications and source records attributed to E Andrès.

At least 19 recordsLinked to original sources
PubMed2006

[Update of clinical findings in cobalamin deficiency: personal data and review of the literature].

PURPOSE: During last decades, several progresses have been made in the diagnosis of cobalamin (vitamin B12) deficiency. Routine used of cobalamin standardized assays have potentially modified the frequency and the type of hematologic abnormalities. CURRENT KNOWLEDGE AND KEY POINTS: Current studies on cobalamin deficiency, including more precise definitions and the description of new etiologies of cobalamin deficiency in adults, as the food-cobalamin malabsorption syndrome, show that hematological abnormalities are generally incomplete compared to historical descriptions of megaloblastic anemia. Nevertheless, they include severe manifestations in 10% of the patients: pancytopenia, severe anemia (hemoglobin < 6 g/dl) or hemolytic anemia and pseudo thrombotic microangiopathy related to cobalamin deficiency. These studies also show the efficacy of new treatment modalities including oral cobalamin administration. PROSPECTS AND PROJECTS: Future studies will confirm these data with the routine use of the new cobalamin assay: holotranscobalamin and validate the usefulness of oral cobalamin therapy.

Administration, Oral↗
PubMed2006

Pharmacological properties of rhamnose-rich polysaccharides, potential interest in age-dependent alterations of connectives tissues.

Rhamnose-rich oligo- and polysaccharides (RROPs) were tested for their potential pharmacological properties using human skin fibroblasts in serial cultures. The substances tested were shown to stimulate cell proliferation, decrease elastase-type activity, stimulate collagen biosynthesis, and protect hyaluronan against free radical mediated degradation. These reactions appear to be triggered by the mediation of a specific alpha-L-rhamnose recognizing lectin-site acting as a receptor, transmitting signals to the cell-interior. The rapid increase of intracellular free calcium after addition of RROP-1 and preliminary data using micro arrays appear also to confirm this contention.

Adult↗
PubMed2006

Current hematological findings in cobalamin deficiency. A study of 201 consecutive patients with documented cobalamin deficiency.

With the introduction of automated assays for measuring serum cobalamin levels over the last decades, the hematological manifestations related to cobalamin deficiency have been changed from the description reported in 'old' studies or textbooks. We studied the hematological manifestations or abnormalities in 201 patients (median age: 67 +/- 6 years) with well-documented cobalamin deficiency (mean serum vitamin B12 levels 125 +/- 47 pg/ml) extracted from an observational cohort study (1995-2003). Assessment included clinical features, blood count and morphological review. Hematological abnormalities were reported in at least two-third of the patients: anemia (37%), leukopenia (13.9%), thrombopenia (9.9%), macrocytosis (54%) and hypegmented neutrophils (32%). The mean hemoglobin level was 10.3 +/- 0.4 g/dl and the mean erythrocyte cell volume 98.9 +/- 25.6 fl. Approximately 10% of the patients have life-threatening hematological manifestations with documented symptomatic pancytopenia (5%), 'pseudo' thrombotic microangiopathy (Moschkowitz; 2.5%), severe anemia (defined as Hb levels <6 g/dl; 2.5%) and hemolytic anemia (1.5%). Correction of the hematological abnormalities was achieved in at least two-thirds of the patients, equally well in patients treated with either intramuscular or oral crystalline cyanocobalamin. This study, based on real data from a single institution with a large number of consecutive patients with well-documented cobalamin deficiency, confirms several 'older' findings that were previously reported before the 1990s in several studies and in textbooks.

Aged↗
PubMed2005

Clinical and immunological aspects of HLA class I deficiency.

Human leukocyte antigen (HLA) class I deficiency is a rare disease with remarkable clinical and biological heterogeneity. The spectrum of possible manifestations extends from the complete absence of symptoms to life-threatening disease conditions. It is usually diagnosed when HLA class I serological typing is unsuccessful; flow cytometric studies then reveal a severe reduction in the cell surface expression of HLA class I molecules (90-99% reduction compared to normal cells). In most cases to date, this low expression is due to a homozygous inactivating mutation in one of the two subunits of the transporter associated with antigen processing (TAP), critically involved in the peptide loading of HLA class I molecules. Although asymptomatic cases have been described, TAP deficiencies are usually characterized by chronic bacterial infections of the upper and lower airways, evolving to bronchiectasis, and in half of the cases, also skin ulcers with features of a chronic granulomatous inflammation. Despite the defect in HLA class-I-mediated presentation of viral antigens to cytotoxic T cells, the patients do not suffer from severe viral infections, presumably because of other efficient antiviral defence mechanisms such as antibodies, non-HLA-class-I-restricted cytotoxic effector cells and CD8+ T-cell responses to TAP-independent antigens. Treatment is at present exclusively symptomatic, and should particularly focus on the prevention of bronchiectasis, which requires early detection.

ATP-Binding Cassette Transporters↗
PubMed2005

[Idiosyncratic drug-induced agranulocytosis].

BACKGROUND: Agranulocytosis is a life-threatening disorder that frequently occurs as an adverse reaction to drugs. CURRENT DATA: Idiosyncratic drug-induced agranulocytosis is characterized by a neutrophil count <0.5x10(9)/l, in serious forms <0,1x10(9)/l that currently occurs especially in association with antibiotics, antithyroid drugs ant ticlopidine (>60% of the incriminated drugs). The overall incidence of idiosyncratic agranulocytosis ranges from 2.4 to 15.4 cases per million patients exposed to drugs per year. Although patients experiencing idiosyncratic agranulocytosis may be asymptomatic (50%), the severity of the neutropenia usually leads to severe sepsis: fever of unknown origin, septicemia, septic shock or localized documented infections such as sore throat, various cutaneous infections or pneumonia. Nevertheless, the mortality rate of idiosyncratic agranulocytosis is now around 5% with appropriate management. PERSPECTIVES: In the future, management of drug-induced agranulocytosis may include pre-established procedures using in critically situations, broad-spectrum antibiotic therapy and hematopoietic growth factors (G-CSF).

Agranulocytosis↗
PubMed2005

[A urachus cyst revealing a torpid Crohn's disease in a young adult with chronic fever].

INTRODUCTION: This case report describes a rare situation in which a superinfected cyst of the urachus complicated initially unknown and inactive Crohn's disease. CASE: A 21-year-old man presented a chronic fever finally attributed to a superinfected urachal cyst. Six months after ablation of the cyst, progressive Crohn's disease was diagnosed. DISCUSSION: The association of Crohn's disease and a superinfected urachal cyst is extremely rare. The case reported here is original in two aspects: the slowly progressive Crohn's disease was diagnosed after its complication; the superinfection developed through local bacterial translocation (ileal loop adjacent to the urachal cyst).

Adult↗
PubMed2005

[Low vitamin B12 levels in elderly patients cured within one week by oral cobalamin therapy].

OBJECTIVE: Non-dissociation of vitamin B12 from its carrying proteins is the most frequent cause of vitamin B12 deficiency in the elderly. The aim of this study was to determine the initial dose of oral cyanocobalamin that would correct the B12 vitamin deficiency within one week. METHODS: This was an open, prospective, study on 30 elderly patients suffering from vitamin deficiency (B12 < 0.20 microg/L) induced by food-cobalamin malabsorption. Ten patients (group I) were treated with a daily dose of 1000 microg of oral cyanocobalamin (from D1 to D8), 10 (group II) with 1000 microg every other day (D1, D3, D5 and D7), 5 (group III) with 1000 microg every 4 days (D1 and D5) and 5 (group IV) with 1000 microg only on D1. The biological response was assessed by control measurement of vitamin B12 serum levels on the 8th day. RESULTS: Mean vitamin B12 serum levels had significantly increased (p < 0.01) in groups I, II and III, but not in group IV. The dose-effect, assessed by the mean increase in vitamin B12 serum levels, was significantly greater (p < 0.05) in groups I (0.25 microg/L) and II (0.18 microg/L), than in groups III and IV (0.09 microg/L). CONCLUSION: This prospective study shows that an oral dose of 1000 microg of cyanocobalamin every 4 days, which corresponds to 250 microg per day, was sufficient to correct B12 vitamin deficiency induced by food-cobalamin malabsorption within one week. However, initial doses of 1000 microg per day or every other day would be preferable because of the greater dose-effect with daily doses higher than 500 microg. A randomised study is warranted to validate these preliminary results.

Administration, Oral↗
PubMed2005

[Cobalamin deficiencies in adults: update of etiologies, clinical manifestations and treatment].

PURPOSE: Update of the adult cobalamin deficiencies. CURRENT KNOWLEDGE AND KEY POINTS: More precise definitions establish the true cobalamin deficiencies and determine their real epidemiology. The current clinical data suggest the concept of food-cobalamin malabsorption as the leading etiology of cobalamin deficiency. The new features of cobalamin deficiency include neurological, gynecological and vascular manifestations. Current treatment modalities include nasal, sublingual and oral cobalamin administration. PROSPECTS AND PROJECTS: Studies are in the way to establish the relevance of the new clinical manifestations and to validate the usefulness of oral cobalamin therapy.

Diagnosis, Differential↗
PubMed2004

[Cationic anti-microbial peptides: from innate immunity study to drug development].

OBJECTIVE: Host defense, cationic anti-microbial peptides are recognized as an important component of innate immune response in most multicellular organisms. NEW FEATURES: They are cationic amphipathic peptides, comprising 20-50 amino acids. Several hundreds of peptides have been characterized. They have broad spectrum of activity against bacterial, fungal and viral pathogens. The mode of action is best known for cecropins and magainins, which act upon the cytoplasmic membrane of microorganisms, causing its disruption by a detergent like activity and pore formation. Several of these peptides or analogs (from magainin, protegrin, indolicidin and histatin) are in advanced clinical development, especially for localized infections. PROJECTS AND PERSPECTIVES: Several other molecules (rBPI, heliomicin and thanatine) are currently under development for various systemic infections. They may represent important drugs of the further anti-infectious therapeutic arsenal.

Animals↗
PubMed2004

[Update of pernicious anemia. A retrospective study of 49 cases].

PURPOSE: The aim of this study was to describe the present clinical characteristics of the pernicious anemia (PA). METHOD: It is a retrospective (1996-2002) multicenter (five departments of internal medicine) study of 49 patients presenting an established cobalamin deficiency related to PA. RESULTS: The median age of the patients was 74 years (25-93), the female/male ratio 2:9. Several autoimmune disorders were noted in 35% of the patients. Various clinical manifestations, mainly neurological, cutaneous and thrombotic, were found in 65.4% of the patients, at least one hematological abnormalities in 100%. Average serum vitamin B12 and homocystein levels were with 73 pg/ml (20-1960) and 42.9 micromol/l (7, 8-124). Anti-intrinsic factor or anti-parietal gastric cells antibodies were found in 87.5% and 62% of the patients (at least one antibody, in 96%) abnormal Schilling's test results in 86%. All the followed patients were successful treated with intramuscular (n = 27) or oral crystalline cyanocobalamin (n = 5). CONCLUSIONS: PA was associated with several autoimmune disorders; PA may be responsible of various clinical manifestations or biological abnormalities; and oral crystalline cyanocobalamin treatment may be successful.

Adult↗
PubMed2004

[Is there a link between the food-cobalamin malabsorption and the pernicious anemia?].

BACKGROUND: A relation between food-cobalamin malabsorption and pernicious anemia has been suggested, particularly in the event of food-cobalamin malabsorption related to hypochlorhydric atrophic gastritis. STUDY DESIGN: This work describes three cases of well-documented cobalamin deficiency related to food-cobalamin malabsorption in three women aged 56, 82 and 68 Years who had atrophic gastritis (not associated with Helicobacter pylori infection) and later developed authentic pernicious anemia. CONCLUSIONS: This work illustrates the potential relation between these two disorders responsible for cobalamin deficiency.

Adult↗
PubMed2004

Current management of adult idiopathic thrombocytopenic purpura in practice: a cohort study of 201 patients from a single center.

To define usefulness and response to therapy and outcome in adults with idiopathic thrombocytopenic purpura (ITP) in clinical practice. We retrospectively reviewed a cohort of 201 consecutive patients with ITP, diagnosed between 1985 and 1994. In particular, we analyzed the therapies used, their response rates, prognostic indicators of response and outcome. In 62 patients, with minor bleeding episodes and a mean (+/- SD) platelet count of 88 +/- 23 x 10(9)/l, no treatment was used and chronic ITP was diagnosed in 59%. A total of 139 patients, with bleeding episodes in 71.2% cases and a mean platelet count of 20 +/- 13 x 10(9)/l, received at least one treatment. Three patients died (1.5% of the series). Corticosteroids were used in 118 patients, with an initial response rate of 82.2% and a long-term complete response (CR) of only 22.9%. Intravenous immunoglobulin was used in 26 patients, with an initial transient response in more than 60%. A splenectomy was performed in 55 patients, with an initial response rate of 92.5% and a long-term CR in 60%. Young age and prior response to corticosteroids were significant predictors of a durable response to splenectomy. Danazol was given in 37 patients, with a favorable response in 73% of cases. Our results illustrate the guidelines of the American Society of Hematology. Patients with moderate thrombocytopenia do not require treatment. In severe cases, splenectomy is the only treatment giving durable cures in a significant proportion of patients. Despite frequent chronicity, ITP is life-threatening only in a minor subset of patients.

Adrenal Cortex Hormones↗