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Biomedical subjects

R Girot

Publications and source records attributed to R Girot.

At least 55 records · Page 3Linked to original sources
PubMed1997

Full or partial seroreversion in patients infected by hepatitis C virus.

Cases of partial seroreversion have been reported in hemodialyzed or immunodepressed patients, but spontaneous clearance of viremia associated with a disappearance of specific antibodies or clearance while receiving therapy has not been precisely documented in immunocompetent hepatitis C virus (HCV)-infected persons. A longitudinal study of markers of HCV infection in a cohort of 178 multitransfused patients followed over an 8-year period was done to establish well-documented cases of partial or full seroreversion. Thirty (16.8%) of 178 patients were HCV-infected; among them, 5 had partial or full seroreversion. Seroreversion to an anti-HCV-negative state is characterized by a quantitative decrease in antibody. A seroreversion may be observed in three circumstances: spontaneously, induced by therapy, and in conjunction with human immunodeficiency virus infection. Long-term follow-up of seroreverters will establish whether they have definitively eradicated HCV from their systems.

Alanine Transaminase↗
PubMed1997

Chronic lymphocytic leukaemia with binucleated lymphocytes.

Chronic lymphocytic leukaemia (CLL) is a monoclonal proliferation usually involving B cells and composed of mature lymphoid cells. Distinct morphologic subtypes have been recognized according to lymphocyte size, nuclear:cytoplastic ratio and nucleolus. However the presence of characteristically binucleated lymphocytes in patients fulfilling criteria for CLL diagnosis has never been described. We here report immunological and cytogenetic studies of four patients with CLL but with binucleated lymphocytes. Moreover, trisomy 12, known to be associated with atypical morphology in CLL, was detected in two of these four patients. We suggest that this be considered as a possible new entity.

Aged↗
PubMed1996

[Sickle cell anemia in the south of Zaire. Study of two series of 251 and 340 patients followed-up 1988-1992].

BACKGROUND: This work concerns 591 sickle cell disease patients followed by the same paediatrics team in south Zaire. POPULATION: Two series of homozygous sickle cell patients were studied: 1) 251 in-patients hospitalized in Kolwezi during the period 1988-1989, and 2) 340 out-patients examined in Lubumbashi during the period 1990-1992. RESULTS: In the first series, a high proportion of children aged 3-5 years (30.7%) and 6-12 years (54%) was observed in comparison with a control group of patients hospitalized for anemia at the same time. The symptoms of the disease occurred during the first year of life in 80% of the children: hand-foot syndrome and/or anemia. Tooth decay was observed as soon as the age of 3 years with a high frequency (62%) as compared with the control group. Epistaxis, sometimes very important, was observed in 39.5% and 52% of the cases in children respectively aged 6-12 years and up to 13. A splenomegaly was noted during a longer period than in the control group, suggesting associated causing factors different from malaria, perhaps alpha thalassemia. The high frequency of viral contaminations due to transfusion is illustrated by the seropositivity prevalences of HIV (11.5%), HBV (10%) and in the children in Lubumbashi. CONCLUSION: The main interest of these series of children is to point out the clinical specificities of a cohort genetically homogenous, offering the opportunity of defining basis of inter-individual variability of sickle cell disease.

Adolescent↗
PubMed1996

Implications of prenatal diagnosis of sickle cell disease.

Prenatal diagnosis (PND) of sickle cell disease (SCD) has been feasible since about 15 years. The number of PND performed for SCD has constantly increased during these years, but its availability raises difficult ethical questions for parents and counsellors. Concerning at-risk parents, only 50% (data in the literature) to 70% (personal data) ask for PND. Our study shows that mainly cultural reasons, then religious ones, educational level and the number of children in the family weigh on the parents' decision to request this diagnosis. The counsellors' position is difficult since clinical severity of the disease is highly variable, there is no early prognostic factor, and the median life expectancy of patients in industrialized countries exceeds 40 years. We need to define a counselling which would consider the image of the illness in the populations involved, in order to help parents understand the implications of the choice they are asked to make.

Abortion, Eugenic↗
PubMed1996

Increased procoagulant activity of red blood cells from patients with homozygous sickle cell disease and beta-thalassemia.

It has recently been proved that, in vitro, red blood cells (RBCs) from patients with homozygous beta-thalassemia behave as procoagulant cells. The procoagulant activity of beta-thalassemia RBCs might be the result of an increased exposure of procoagulant phospholipids (i.e. phosphatidylserine) in the outer leaflet of the membrane. In order to test this hypothesis, we compared the catalytic properties of RBCs of patients with beta-thalassemia and homozygous sickle cell disease (SS-RBCs) with that of controls. The catalytic parameters (Km, kcat) of prothrombin activation by factor Xa were determined both in the absence and in the presence of RBCs. The turn-over number (kcat) of the reaction was not modified by normal, SS- or beta-thalassemia RBCs. The Km was lower in the presence of normal RBCs (mean value: 9.1 microM) than in the absence of cells (26 microM). The Km measured in the presence of either SS-RBCs (mean value: 1.6 microM) or beta-thalassemia RBCs (mean value: 1.5 microM) was significantly lower compared to normal RBCs (p < 0.001). No significant difference was observed between SS-RBCs and beta-thalassemia RBCs. Annexin V, a protein with high affinity and specificity for anionic phospholipids, inhibited the procoagulant activity of both SS-RBCs and beta-thalassemia RBCs, in a dose-dependent manner. More than 95% inhibition was achieved at nanomolar concentrations of annexin V. These results indicate that the procoagulant activity of both beta-thalassemia RBCs and SS-RBCs may be fully ascribed to an abnormal exposure of phosphatidylserine at the outer surface of the red cells.

Adolescent↗
PubMed1995

Acral dysostosis dyserythropoiesis syndrome.

UNLABELLED: The term congenital dyserythropoietic anaemia (CDA) designates a group of rare but well defined erythrocytic disorders. Type I is defined by macrocytosis and megaloblastic changes of the bone marrow cells. Two unrelated children with CDA are described with associated defects: absence of nails and short or absent phalanges, polysyndactyly of the fourth metacarpal. One of them had also areas of depigmentation. CONCLUSION: The association of congenital dyserythropoietic anaemia with morphological defects of hands and feet is suggested to constitute a new syndrome caused by a single morphogenetic gene.

Abnormalities, Multiple↗
PubMed1994

Gadolinium-DOTA enhanced MRI of painful osseous crises in children with sickle cell anemia.

In order to evaluate the role of gadolinium-DOTA enhanced MRI in the management of painful osseous crises in children with sickle cell anemia (SCA), nine children with SCA underwent MRI, bone scans and ultrasonographic studies during 11 osseous crises. Imaging findings were compared with the final diagnosis: three acute osteomyelitis (AO) and 16 acute infarcts (AI). MRI could not differentiate AO from AI. The appearance of severe AI was very misleading and was similar to the usual appearance of AO, including soft tissue changes, periosteal reaction and patterns of enhancement. Gadolinium-DOTA enhanced MRI was useful for determining the anatomic site and extent of AO or AI and for distinguishing between necrotic material, fluid collection and vascularized inflammatory tissue. It can also help to guide the aspiration of intraosseous, subperiosteal and soft tissue fluid collections.

Acute Disease↗
PubMed1994

The use of Staphylococcus V8 protease in the structural determination of human hemoglobin variants: HB Valparaiso [alpha 88(F9)Ala-->Gly] example.

In human hemoglobin, enzymic cleavage with the protease from Staphylococcus aureus strain V8 (protease V8) is a convenient method for the study of variants that involve large peptides such as alpha T-9 and alpha T-12b. We report here on Hb Valparaiso [alpha 88(F9)Ala-->Gly], a new neutral variant with a slight increase in oxygen affinity, that was identified by use of this strategy.

Female↗
PubMed1994

[Separation of hemoglobins F, Fac, S, C, A1c and determination of hemoglobin F using high performance liquid chromatography].

Separation of hemoglobins F, Fac, S, C and A1c was performed using high performance liquid chromatography with a cation exchange Polycat A column in a 15-minute assay. HbF titration results were well correlated with those of the reference alkali denaturation technique for values below 12% (r = 0.95; P < 0.001). This technique may be used as a confirmation test for neonatal screening of sickle cell disease.

Anemia, Sickle Cell↗