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Biomedical subjects

A Zubizarreta

Publications and source records attributed to A Zubizarreta.

At least 55 records · Page 3Linked to original sources

[Prospective study on the prevalence of iron deficiency in Cantabria among children 6 to 14 years old].

Iron deficiency is the most common worldwide micronutrient deficiency in developed countries. To analyze its prevalence in children and adolescent population from our region, we studied Hb, Hto, red cell volumes, serum iron, total iron binding capacity, transferrin saturation and serum ferritin in a population of 380 individuals. These also fulfilled an epidemiological questionnaire. Mean values and normal reference ranges are shown according to age sex, which is very important to optimize the identification of individuals with iron deficiency. Overall prevalence of iron deficiency was 15.7% and it was higher in adolescent males from 12-14 years (21.8%) and children from 6-8 year (18.8%). Significant differences related to residence or to socioeconomic status were not found. The health consequences of iron deficiency are to be considered in any public health planning project.

Adolescent↗

Treatment of myelodysplastic syndrome with 1.25-dihydroxy-vitamin D3.

1.25-dihydroxy-vitamin D3 (1.25 (OH)2D3) was tested in seven patients with myelodysplastic syndrome. The study was undertaken because 1.25 (OH)2D3 promotes differentiating myeloid cells in vitro and because of a prior report of potential benefit in a clinical study. The drug was given orally at a dose of 2.5 micrograms/day for a minimum of 8 weeks (range 8-28). After therapy, there were no significant changes in any of the parameters observed in peripheral blood or bone marrow. We did not observe any feature of granulocytic-monocytic differentiation. Treatment was well tolerated. One patient died because of bone marrow failure. Survivors have persisting myelodysplastic syndrome and continue to be transfusion dependent. 1.25 (OH)2D3 has no beneficial effect in patients with myelodysplastic syndrome with this dose regimen.

Aged↗

Hairy-cell leukaemia associated with auto-immune disorders in the form of a 'lupus-type' anticoagulant and a positive direct Coombs' test.

An account is given of a case of hairy-cell leukaemia associated with a 'lupus-type' anticoagulant and a positive direct Coombs' test, both of which were clinically symptom free. This is yet another example of the coexistence of hairy-cell leukaemia and an auto-immune disorder, but the disorder in question has not been described previously.

Autoantibodies↗

Acquired von Willebrand's syndrome associated with hydatid disease of the spleen--disappearance after splenectomy.

A new case of acquired von Willebrand's syndrome (vWS) is described in a 31-year-old woman with a hydatid splenomegaly and with a history of repeated abortions at an advanced stage of pregnancy, a positive serology for syphilis and a mildly elevated titre of antinuclear antibodies, with no family history of bleeding. There is an inhibitory effect on factor VIII: C (antihaemophilic factor) as well as on factor VIIIR: Ag (related antigen) and on factor VIIIR: RCo (ristocetin cofactor), and it is precipitated by rabbit anti-IgG antiserum. This inhibitory effect was demonstrated using the patient's plasma heated to 56 degrees C for one hour so as to dissociate circulating immunocomplexes. All the abnormalities of haemostasis, as well as the positive serology for syphilis, disappeared after splenectomy, and the ANA titre reverted to normal. The clinical and biological peculiarities of the case are discussed, and are interpreted in the light of the findings recorded in patients showing "lupus" anticoagulant.

Adult↗

Complete recovery of hemopoiesis following bone marrow transplant in a patient with unresponsive congenital hypoplastic anemia (Blackfan-Diamond syndrome).

Allogeneic bone marrow transplantation (BMT) was carried out on a 5-year-old boy with congenital hypoplastic anemia (CHA), who did not respond to corticosteroids and who was displaying signs of progressive hemosiderosis. Pretransplant preparation had to be modified because respiratory failure and cerebral edema supervened. This preparatory regimen consisted of busulfan (2 mg/kg for four days), cyclophosphamide (50 mg/kg for one day), and total body irradiation (750 rad). Hemopoiesis was completely restored and is still maintained 650 days after transplantation. This is the second published report on the use of BMT to treat a patient with CHA, and it is the first time it has resulted in long-term survival. BMT should be considered for patients with CHA who do not respond to corticosteroids.

Anemia, Aplastic↗

Transmission and scanning electron microscopy study on congenital dyserythropoietic anemia type I.

An ultrastructural study of the aspirated bone marrow of a patient with congenital dyserythropoietic anemia type I is presented. Both in transmission electron microscopy (TEM) and in scanning electron microscopy (SEM) ultrastructural abnormalities of the bone marrow erythroid precursors were seen. These abnormalities in TEM included uneven condensation of chromatin with spongy appearance and alterations of cellular division. The most striking among these alterations were: firstly, the intercellular bridges which were typified by the presence of chromatin and the absence of the midbody and contractile ring and, secondly, the anomalies of the nuclear membrane. Occasionally, autolytic areas within the cytoplasma and iron-laden mitochondria were to be seen. In the SEM the following features were notable: the length of the intercellular bridges, the absence of the central ridge in the midbody area, as well as the scanty number of cytoplasmic blebs on the surface of the bridges.

Adolescent↗

Unusual ultrastructural findings in neuroblastoma.

A morphologic study was made of the cell population which had infiltrated the bone marrow of a five-year-old boy. These cells showed a tendency to form rosette-like structures. These structures as well as the presence (at ultrastructural level) of neurosecretory granules, cell processes, and microtubules in the neoplastic cells led to a diagnosis of neuroblastoma. Certain characteristics, not previously reported in neuroblastoma, were identified, such as gap junction type intercellular contacts, paracrystalline arrays in mitochondrial atpyical cristae and nucleolus-like bodies (nematosomes). Gap junctions are involved in the intercellular transfer of ions and low molecular weight metabolites and may explain the tendency to form cellular cluster in "rosettes" which are characteristic of this neoplasm. The presence of nematosomes in the tumor cell cytoplasm is one more piece of evidence which substantiates the nervous origin of these cells.

Bone Marrow↗