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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

[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↗

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↗

[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↗

[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↗

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↗

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↗

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↗

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↗

Hereditary hemolytic anemia with increased red cell adenosine deaminase (45- to 70-fold) and decreased adenosine triphosphate.

Hereditary hemolytic anemia, a dominantly transmitted disorder, has affected 12 family members spanning three generations. The concentration of adenosine triphosphate in the red cells was about half that of comparably reticulocyte-rich blood. Since adenosine deaminase and adenosine kinase compete for a common substrate, the greatly increased activity of the former may interfere with nucleotide salvage via the latter.

Adenine Nucleotides↗