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Biomedical subjects

F Gilsanz

Publications and source records attributed to F Gilsanz.

At least 55 records · Page 3Linked to original sources
PubMed1992

Cytohematologic and cytogenetic prognostic factors at diagnosis and in the evolution in 46 primary myelodysplastic syndromes.

The myelodysplastic syndromes (MDS) are a heterogeneous group of diseases with different prognosis and evolution. Most of the studies on prognostic factors performed previously have independently evaluated the clinico-hematologic or cytogenetic data at diagnosis. In the present paper, 46 primary MDS were clinically, hematologically, and cytogenetically investigated at diagnosis, in order to determine the principal factors affecting the survival probability between a great number of characteristics. A univariate regression analysis of all the data allows one to recognize that the main factors are: the complexity of karyotype (p = 0.00001), the percentage of type I and total marrow blast cells (p = 0.001), and the abnormal localized immature myeloid precursors' (ALIP) presence (p = 0.001). Twenty-five patients underwent consecutive studies during their evolution. The karyotype instability gives information both on the likely evolution to acute leukemia and on poor survival.

Adult↗
PubMed1992

Chronic ethanol abuse and membrane fluidity changes in liver disease.

The long-term effect of ethanol on human red cell membrane fluidity was studied, by fluorescence polarization with 1,6-diphenyl-1,3,5-hexatriene as a probe, in 11 healthy subjects, 9 chronic alcoholics without evidence of liver disease, 12 chronic alcoholics with biopsy-proven alcoholic liver disease and 9 abstemious patients with chronic active liver disease, most of them cirrhosis of the liver. Fluorescence polarization values were not significantly different in the two groups without liver disease. Patients with alcoholic and non-alcoholic liver disease showed higher fluorescence polarization values than patients without liver disease. These changes correlated with the severity of liver dysfunction and were not related to alcohol consumption. In conclusion, the decrease in fluidity of the erythrocyte membrane in alcoholic patients with chronic liver disease, is related to liver dysfunction but not to chronic ethanol ingestion. Changes in membrane fluidity in chronic alcoholics are found only in the presence of liver disease.

Adult↗
PubMed1992

Homozygous 4.1(-) hereditary elliptocytosis associated with a point mutation in the downstream initiation codon of protein 4.1 gene.

We studied a 43 yr-old Spanish patient with homozygous 4.1(-) hereditary elliptocytosis. Any form of protein 4.1 was missing in the red cells. Spectrin and actin were slightly, yet significantly, diminished. Alterations appeared at the level of proteins 4.5 and 4.9. Glycophorin C was sharply reduced. The abnormal allele was associated with the -++-- haplotype (Pvu II, Bgl II, Bgl II, Pvu II, Pvu II). mRNA 4.1(-) had an apparently normal size but was diminished by about two-thirds. Because the abnormal phenotype pertained to the red cell, we sequenced the 4.1 cDNA regions that appear critical to this cell type. The ultimate change turned out to be a point mutation of the downstream translation initiation codon (AUG-->AGG). No disorders in other cell types could be related with certainty to the present 4.1(-) HE allele.

Adult↗
PubMed1992

Age and sex matched analysis of Hb Lepore trait in a new population in Spain.

A group of subjects with Hb Lepore trait has been found in the region between Extremadura and Toledo in Spain. Clinical, radiological and hematological studies were carried out on 81 cases from 23 families. Asthenia was the sole complaint in seven of forty cases. Abdominal echography showed no cholelithiasis in 16 children under 16 years. Hb Lepore mean was 10.81 +/- 1.97%, range 6.5 to 16.1%, Hb A2 levels were normal and Hb F values were high. Globin chain synthesis in reticulocytes showed a total alpha/beta ratio of 1.89 +/- 0.3. Hematological values from Hb Lepore trait subjects were analyzed according to age and sex and the data compared to beta thalassemia and delta-beta thalassemia cases of matched age and sex. Hb Lepore trait patients had a milder form of thalassemia minor than beta thalassemia patients, with higher levels of hemoglobin, MCV and MCH for all three groups: children under 13 years, males over 14 years and females over 14 years. Children and females with Hb Lepore had higher hemoglobin levels than those with delta-beta thalassemia minor, while no significant difference was found in males.

Adolescent↗
PubMed1989

[Hemolytic anemia caused by pyrimidine 5'-nucleotidase (P5N) deficiency 15 years later. Apropos of 2 new cases of hereditary deficit and another one of lead poisoning].

Congenital pyrimidin 5'nucleotidase deficiency manifests as hemolytic anemia with basophilic stippling. In lead poisoning, anemia, basophilic stippling and inhibition of erythrocyte pyrimidin 5' deficiency are also observed. In the present work, we report two cases of hemolytic anemia secondary to congenital deficiency of pyrimidin 5' nucleotidase and another case secondary to lead poisoning. Since 1974, when pyrimidin 5' nucleotidase deficiency was isolated, is known that hemolysis is related to the accumulation of pyrimidin nucleotides within the erythrocytes that behave as metabolic inhibitors. However, the precise metabolic process whose inhibition leads to the shortening of erythrocytes half life has not been elucidated yet.

5'-Nucleotidase↗
PubMed1989

Characterization of a new alpha-thalassemia-1 deletion in a Spanish family.

A new type of alpha-thalassemia-1 was characterized in one Spanish patient with Hb H disease and in her mother. The restriction map of this deletion suggests that the deletion of 22 kb has occurred on a chromosome carrying a zeta-globin triplication. The resulting chromosome lacks the alpha 2- and alpha 1-globin genes, the psi alpha 2- and psi alpha 1-globin genes, and one of the three zeta-globin genes, while the other two zeta-globin genes and the theta 1-globin gene have been retained.

Adult↗
PubMed1989

[Usefulness of cytometry based on the diffraction of a laser beam in evaluating spherocytosis].

Thirty four cases of hereditary spherocytosis were studied by means of laser diffraction cytometry. The cases were grouped for study in accordance to previous splenectomy or not, familial involvement or not, and, in patients not subjected to splenectomy, severity of the clinical course. The values used to assess the presence of spherocytosis were those measuring the haemoglobin concentration within red cells, such as CH (directly estimated mean corpuscular haemoglobin), HDW (standard deviation of the distribution according to haemoglobin concentration) and % hyper (percentage of cells with haemoglobin concentration higher than 41 g/dL). The variables attained were statistically analysed by means of non-parametric tests. In patients with spherocytosis, MCV, MCHC, RDW, HDW, % hyper, and CH were significantly different from the normal group. This method points to the presence of spherocytosis by means of CH, HDW and % hyper. A reduction of the limit of haemoglobin concentration used to define % hyper (41 g/dL) could improve the sensitivity of the instrument for the diagnosis of the mild forms, which is often more difficult.

Erythrocyte Indices↗
PubMed1989

[Effects of high-frequency ventilation on the intracranial pressure and cerebral elastance in dogs].

We have evaluated the effects of the high frequency "jet" ventilation (HFJV) in 12 healthy dogs, under normal intracranial pressure (ICP) as well as under progressive Intracranial Hypertension (ICH). With a normal ICP, no significant differences were found in the mean ICP regarding the intermittent positive pressure ventilation (IPPV). However, during ICH the HFJV not only decreases the global cerebral elastance (CE), P/V curve with smaller slope, but also places the brain in an improved dynamic condition in the sense that, at an equal ICH level, the CE is lower, CE mean ICP relation having a significant difference (p less than 0.001).

Animals↗
PubMed1988

Improvement in the erythropoiesis of chronic haemodialysis patients with desferrioxamine.

16 chronic haemodialysis patients (group I), with non-microcytic anaemia (mean haemoglobin 7.2 g/dl, SD 1.0, range 5.8-9.8), moderate aluminium overload (serum aluminium 44 micrograms/l, SD 16, range 21-74), and normal or high iron stores (ferritin 800 micrograms/l SD 464, range 34-2013) were treated with intravenous desferrioxamine 1 g at the end of each dialysis for six months. 8 patients with similar characteristics served as controls (group II). After six months group I showed a rise in haemoglobin to 9.1 (SD 2.5) g/dl and a decrease in blood transfusion requirements, both significant, whereas group II showed no changes. Other significant changes observed in group I, but not group II, were a rise in reticulocytes and in red cell creatine and a fall in red cell protoporphyrin and serum ferritin. Ferritin decreased more in the patients whose anaemia improved. Minor increases in serum aluminium in group I did not differ from those in the control group. Desferrioxamine may benefit the anaemia of chronic haemodialysis patients through improvement of erythropoiesis. The effect seems not to be related to chelation of a heavy aluminium overload.

Adult↗
PubMed1988

Hemodynamic and morphologic alterations after experimental administration of protamine sulfate.

The hemodynamic, hematologic, and morphologic effects induced by protamine sulfate have been studied in 28 dogs divided into 6 groups. All of the groups were given heparin (3 mg/kg body weight) and Groups I, II, III, and IV were given protamine (5 mg/kg body weight). Group I (control group) was not subjected to extracorporeal circulation. The other groups had the following interventions: Group II, cardiopulmonary bypass without aortic clamp, hypothermia, or cardioplegia; Groups III and V, hypothermia of 25 degrees C, aortic clamping for 25 minutes, administration of cardioplegic solution, and slow rewarming; and Groups IV and VI, the same as Groups III and V, but with rapid rewarming. After injection of protamine sulfate, there was a decrease in mean arterial pressure due to peripheral vasodilation and an increase in the mean pulmonary pressure due to increased pulmonary vascular resistance; marked diminution of the number of circulating platelets aside from the extracorporeal circulation; a decrease in the contractility of both ventricles with augmented right ventricular work and decreased cardiac output; and right ventricular edema in Groups I, II, III, and IV. These alterations were most evident in Groups III and IV.

Animals↗
PubMed1987

Effects of high-frequency jet ventilation on intracranial pressure and cerebral elastance in dogs.

In order to assess the effect of high-frequency jet ventilation (HFJV) on intracranial pressure (ICP) and cerebral elastance (CE), a model of progressive intracranial hypertension was developed. Six healthy dogs were ventilated using HFJV and another six with conventional mechanical ventilation. ICP and its pulse amplitude (Amp) were measured by means of an epidural transducer, and CE was assessed by analysis of the relationship between the Amp and the mean ICP. The mean ICP, under basal conditions, did not differ between the two forms of ventilation, the Amp and the slope of the relationship Amp/ICP being lower with HFJV (p less than .001). Since the lower airway pressure created with HFJV was the only difference, it is suggested that the differences found in this study are related to these pressures.

Animals↗
PubMed1981

Acute pulmonary oedema due to lithium intoxication.

We present a case of acute lithium ion intoxication in which the main clinical feature was respiratory failure secondary to pulmonary oedema possibly due to myocardial depression, associated with profound stupor. The initial lithium ion concentrations were 3.15 mmol/l in plasma and 27.6 mmol/l in urine. Under symptomatic treatment with oxygen, digitalis and diuretics, both the pulmonary and the neurological disorders reverted gradually, in parallel with the decreasing plasma lithium ion concentrations.

Acute Disease↗