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Biomedical subjects

R Girot

Publications and source records attributed to R Girot.

At least 127 records · Page 7Linked to original sources
PubMed1986

Hemoglobin J Iran alpha 2 beta 2 77 (EF1) his----Asp in a Russian-Armenian family.

A third case of Hb J Iran is reported. The propositus is of Russian-Armenian origin and was investigated for hematuria. The electrophoretic behavior and the characterization of primary structure are described. Hb J Iran is stable and has normal functional properties. High resolution Nuclear Magnetic Resonance spectra suggest the presence of structural perturbations in the heme pocket of the variant. Solubility studies of Hb S/Hb J Iran mixture indicated that His beta 77 belongs to a contact region of deoxy Hb S polymers.

Adolescent↗
PubMed1986

Heterogeneity of sickle cell disease as shown by density profiles: effects of fetal hemoglobin and alpha thalassemia.

Factors that modify the intraerythrocytic concentration of hemoglobin S may influence the clinical expression of the disease. Using the phthalate ester method, the red blood cell density has been studied as a function of the mean corpuscular hemoglobin concentration. Four parameters have been used to compare the density distribution of the erythrocytes: D50 (median cell density), R60 (density range in which the middle 60% of the cells are found), F4 and F5 (proportion of cells with density greater than 1.110 and 1.120 g/ml). Compared to normal controls the density distribution of sickle red cells is heterogeneous, reproducible for the same patient (except in case of crisis), while different from one to another. The R60 is correlated with the percentage of dense cells, and the highest values for both R60 and dense cells are found when hemoglobin F is less than 10%. The highest values for the median cell density and dense red cells, but not for R60 which is normal, are observed in S/C patients. In sickle cell anemia patients, the median cell density values are not very different from the normal ones. The highest levels of hemoglobin F are found in this median subpopulation of red cells, while they are very low in the densest cells. R60 and the percentage of dense cells are not affected by the association of sickle cell disease with the deletion of one alpha gene. Their values are very near the normal ones in the case of an association with beta thalassemia or homozygous alpha thalassemia.(ABSTRACT TRUNCATED AT 250 WORDS)

Anemia, Sickle Cell↗
PubMed1986

[Transient erythroblastopenia in young children].

A case of transient erythroblastopenia of childhood is reported. This syndrome occurred in a 13 month-old girl. Initial signs were a 6.1 g/dl hemoglobin level and severe erythroblastopenia. Spontaneous recovery occurred after a few weeks. No etiology could be found.

Anemia, Aplastic↗
PubMed1986

[Autoimmune hemolytic anemia complicating homozygotic beta- thalassemia].

Four cases of auto-immune hemolytic anemia complicating the course of intermediate or major beta-thalassemia syndromes are reported in children. Ages ranged from 2 to 13 years. Two children had undergone a splenectomy. The direct Coombs test was positive of the IgG +/- complement or complement alone type. Corticosteroid therapy was beneficial in the 3 children who were given this treatment. Coombs test became negative after intervals ranging from 15 days to 8 months.

Anemia, Hemolytic, Autoimmune↗
PubMed1986

Hémoglobinopathies in France.

A first approximation of the incidences of abnormal hemoglobin traits in France is given. The data include all the large ethnic minorities living in France, and also take into account the second and third generations. We estimate 1.3 X 10(5) and 1.8 X 10(5) individuals with HbS trait and beta-thalassemia trait respectively.

Anemia, Sickle Cell↗
PubMed1986

[Transfusion requirements and mean annual hemoglobin level in thalassemia major].

The blood requirements using packed red cells (C) and the mean annual haemoglobin level (Hb ma), corresponding to the mean of all haemoglobin levels before (Hb pre T) and after (Hb post T) transfusion over 1 year, have been measured in 14 splenectomized and regularly transfused thalassaemia major patients. In these non-hypersplenic patients a linear relation was determined between C and Hb ma. The blood consumption C has also been measured in 5 patients with hypersplenism. The comparison of C between these two groups of patients offered the possibility of defining the limit of C, for a given value of Hb ma, above which hypersplenism is indicated. The measurement of Hb ma requires the value of Hb post T, a parameter which is sometimes difficult to obtain in practice. For this reason, a method of calculating Hb post T using Hb pre T, C and the number of transfusions over 1 year (N) is described. The relation between these parameters is: Hb post T = Hb pre T + C/N X 0.23. To use this formula, the conditions must be as follows: the haematocrit of blood units must be between 75% and 85%, and the blood volume of each transfusion in a given patient must be identical throughout the year.

Adolescent↗
PubMed1985

[Homozygous beta-thalassemia in Algeria. Study of 50 cases].

The clinical and laboratory data recorded at first presentation in 50 homozygous beta-thalassaemic untransfused children seen at the National Transfusion Centre, Algiers, are reported. These children came from 38 families, including 25 with consanguinous parents. Pallor was observed in all cases but jaundice and asthenia were present in only 11 and 10 children respectively. Splenomegaly was frequent (45 cases), as were skeletal changes mainly in the skull and face (35 cases). Haemoglobin levels ranged from 2.4 to 9.6 g/dl and MCV from 71 to 89 fl. Among these 50 patients, 34 had beta + thalassaemia and 16 beta 0 thalassaemia. Levels of foetal haemoglobin (Hb F) were similar in both groups but clinical symptoms appeared earlier in beta 0 thalassaemia patients. Thirty-seven cases were diagnosed as thalassaemia major and 6 as thalassaemia intermedia. Comparison of various parameters between siblings (20 children belonging to 9 families) showed no differences between Hb F and Hb A2 levels and clinical courses. These findings should be taken into consideration for the prenatal diagnosis of beta-thalassaemia.

Algeria↗
PubMed1985

[Acute erythroblastopenia disclosing homozygous beta-thalassemia. Role of parvovirus infection].

An acute, transient aplastic crisis in a 15-month old boy revealed the presence of homozygous beta-thalassaemia. The crisis was very likely due to a parvovirus infection, in view of the presence of specific IgM at the onset and of seroconversion to total antibodies. Later, requirements for transfusions were in favour of an intermediate type thalassaemia. The responsibility of the parvovirus is discussed in the light of recent data concerning the inhibitory action of this virus on bone marrow erythropoiesis.

Acute Disease↗
PubMed1985

[Partial disarterialization of the spleen and partial splenectomy in children].

Splenic hyperactivity can be reduced by partial vascular disconnection of the spleen or by partial splenectomy. The first method, which preserves only one superior polar artery and the whole venous system, was used in 6 patients, and the second method, which preserves a small portion of the spleen, usually fed by an inferior polar artery, was used in 11 patients. Ultrasonographic and scintigraphic measurements of the spleen, platelet counts, transfusion quotient and 51 Cr-labelled red cell survival were used to evaluate the effects of these two methods. No immediate post-operative complication was observed.

Arteries↗
PubMed1985

Neurological findings in triosephosphate isomerase deficiency.

Two siblings with hemolytic anemia caused by triosephosphate isomerase deficiency developed a progressive neurological syndrome featuring dystonic movements, tremor, pyramidal tract signs, and evidence of spinal motor neuron involvement. Intelligence was unaffected. The findings in these patients and in 14 previously published cases indicate that neurological manifestations are an integral part of the disorder and suggest that specific structures in the basal ganglia, brainstem, and spinal cord bear the brunt of the pathological process, which does not affect the cerebral cortex.

Anemia, Hemolytic↗
PubMed1985

Mössbauer spectroscopy study of iron overloaded livers.

Absorption 57Fe Mössbauer spectra have been carried out directly on fresh or lyophylized tissues of liver with either normal iron depot or iron overload. Two types of overloading have been studied: primary iron overload due to an excessive intestinal iron absorption and secondary iron overload (hemosiderosis) produced in beta-thalassemia patients by hypertransfusional therapeutics. The Mössbauer spectra, at room temperature, 77 and 4.2 K, on normal liver samples, are typical for the ferritin-hemosiderin compounds. In the spectra, performed on hemosiderosis liver samples, there appears, in addition to ferritin and hemosiderin, a new iron molecular environment, typical of high spin ferric iron and characterized by a superparamagnetic behaviour which begins at high temperature (above 77 K). This new component does not show up in the primary iron overload cases and seems characteristic of the physiological process which induces the iron overload.

Animals↗