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Biomedical subjects

E Muñiz-Díaz

Publications and source records attributed to E Muñiz-Díaz.

At least 19 recordsLinked to original sources

A new successful therapy for fetal chylothorax by intrapleural injection of maternal blood.

We present two cases of fetal chylothorax and hydrops diagnosed at 20 weeks' gestation, both of which underwent successful intrauterine treatment. In Case 1, a transient, near total resolution began 2 weeks after an iatrogenic hemothorax following a second thoracocentesis performed at 24 + 6 weeks. Because of pleural fluid reaccumulation, a Cesarean section was performed at 36 weeks. The 3805-g female neonate was admitted to neonatal intensive care but was discharged 50 days later in a healthy condition. In Case 2, resolution occurred after a third thoracocentesis and a second pleural injection of maternal blood, performed at 26 weeks. A 2660-g female neonate was delivered vaginally at 38 weeks. The infant remained asymptomatic and was discharged aged 4 days. Our experience suggests a possible useful role of intrapleural blood injection for the treatment of fetal chylothorax.

Adult↗

[Fetal-neonatal alloimmune thrombocytopenia].

Fetal-neonatal alloimmune thrombocytopenia is the commonest cause of severe thrombocytopenia in the newborn. This disorder is due to the destruction of fetal platelets by a maternal platelet-specific antibody caused by fetal-maternal incompatibility. The most serious complication is intracranial hemorrhage (10-30 % of newborns), which may cause death (10 % of the reported cases) or irreversible neurological sequelae (20 %). The diagnosis is usually made after birth when most affected neonates have petechiae, purpura or overt bleeding. The degree of severity varies according to platelet count. Current methods allow detection of maternal platelet alloantibodies (usually HPA-1a). Clinical grounds and the exclusion of other causes of neonatal thrombocytopenia are required to establish an accurate diagnosis. Recurrence of this disease is very high and has prompted clinicians to develop antenatal prophylactic programs in subsequent pregnancies. However, the optimal treatment of at-risk pregnancies remains controversial. The early diagnosis of this process allows effective therapy based on the infusion of compatible platelets and IgG immunoglobulins when hemorrhage is not obvious. Antenatal management of subsequent pregnancies can prevent recurrence of thrombocytopenia and intracranial hemorrhage. The aim of this review is to draw pediatricians' attention to the importance of this probably under-diagnosed disease in which early diagnosis can prevent potentially severe complications.

Antigens, Human Platelet↗

Chimerism analysis following allogeneic peripheral blood stem cell transplantation with reduced-intensity conditioning.

We have performed a prospective study to evaluate early chimerism and its kinetics after allogeneic peripheral blood stem cell transplantation among 68 patients who received a reduced-intensity conditioning (RIC) regimen with fludarabine plus melphalan (n=40) or busulphan (n=28). Chimerism was analyzed by polymerase chain reaction amplification of short tandem repeats in unfractionated (UF) and/or fractionated nucleated cells from bone marrow and peripheral blood (PB). All of the patients showed initial donor engraftment and no patient presented primary or secondary graft failure. In UF samples, the probability of achieving stable complete donor chimerism (CDC) in PB within the first 6 months was 70% on day +30, 85% on day +100 and 95% on day +180. CDC in granulocytes was observed in nearly all cases from day +30 onwards. CDC in T cells, however, differed among melphalan and busulphan recipients during the first 3 months (100 vs 0% on day +30 and 93 vs 20% on day +90, respectively). In multivariate analysis, the only significant variable associated with the achievement of early CDC was having received more than two lines of chemotherapy pretransplant (P<0.02). No correlation was found between the rate of achieving early CDC and the occurrence of acute graft-versus-host disease (GVHD) or disease progression post-transplant. In multivariate analysis, the only variable that influenced the incidence of disease progression post-transplant was the development of chronic extensive GVHD (P<0.05). In conclusion, a state of CDC is readily obtained within the first 6 months after our RIC protocols. Donor myeloid engraftment occurs rapidly in all cases, while early T-cell CDC is more common in more immunosuppressed hosts and, perhaps, in melphalan recipients.

Adult↗

Successful treatment of pure red cell aplasia after major ABO-incompatible T cell-depleted bone marrow transplantation with erythropoietin.

A 40-year-old woman with acute myeloid leukemia in first remission developed pure red cell aplasia after a T cell-depleted ABO-incompatible bone marrow transplant from her HLA-identical sister. She remained transfusion-dependent for 11 months despite conversion of the ABO blood group to donor type, and titers of anti-donor isohemagglutinin being undetectable. Treatment with erythropoietin resulted in rapid improvement of the anemia with no further need for transfusions up to 21 months post-transplant. This case suggests that erythropoietin may provide effective therapy for pure red cell aplasia after ABO-incompatible bone marrow transplantation without the additional risks of further immunosuppression.

ABO Blood-Group System↗

[Isolated thrombocytopenia in pregnancy. Etiopathogenic study and therapeutic approach in 60 patients].

BACKGROUND: We analyze the etiopathogenesis and clinical and immunohematological characteristics of 60 pregnant women with isolated thrombocytopenia (TP) (platelet count < 150 x 10(9)/l); and the frequency of TP and hemorrhagic complications in their newborn. We suggest the therapeutic approach for each maternal TP type. PATIENTS AND METHODS: We performed: clinical history, platelet count (EDTA K3, sodium citrate, microscopic exam) and investigation of antiplatelet antibodies (immunofluorescence) in all pregnant women. A familial history and ultrastructure of platelets were studied when hereditary macrothrombocytopenia (HM) was suspected. A Levine's test of homogenicity of variances was applied to compare the mean platelet count in each diagnostic group. A linear regression between maternal and newborn platelet counts was performed. RESULTS: In 37 thrombocytopenic women (62%) no antiplatelet antibodies were found, and the clinical history was negative for previous TP or abnormal bleeding. Four patients (7%) were diagnosed as pseudothrombocytopenia EDTA-mediated, and eight (13%) of HM. Finally, an autoimmune etiology was suspected in 11 women (18%) and antiplatelet antibodies were detected in 9. Mean platelet counts of mother with immune TP did not show statistically significant differences with other diagnostic groups. Abnormal bleeding was not observed in any patient or newborn. There was no correlation between platelet counts of mothers and newborns. Platelet count obtained by skull bone punction led to unnecessary caesarians in four cases. CONCLUSIONS: The frequency of immune thrombocytopenia in pregnant women is low (18%). There is a high prevalence of benign TP (62%). The pseudothrombocytopenias and HM are frequent findings (20%), and special care is advisable in these cases to avoid unnecessary therapeutic procedures.

Female↗

[Post-transfusion purpura. Description of 2 cases diagnosed at the same hospital in a 6-month period].

Two new cases of postransfusional purpura diagnosed at the same hospital within the space of six months are described. This finding supports the idea that despite being an infrequent disorder, a substantial rise in PTP cases inside and outside our country, has been recorded. This increase has coincided with a greater interest in platelet immunology and, particularly, in complications associated with blood transfusion. Both cases constitute an example of the clinical epidemiological profile which characterizes the patients suffering from this disorder. The patients are two women aged 74 and 60 years who after 8 and 9 days, respectively, of being transfused with red cells developed a severe thrombocytopenia accompanied by generalized haemorrhagic diathesis. The serological studies performed revealed the presence, in both patients, of an HPA-1a platelet specific antibody. The platelet genotyping enabled us to confirm this specificity after detecting an HPA-1 (a-b+) platelet genotype. The treatment with immunoglobulins at high doses proved to be effective in both cases. The adsorption-elution experiments of the antibody versus HPA-1 (a + b) platelets were positive in the patient with the highest antibody titre (1024). This finding support the most recent hypothesis concerning the pathogenic mechanism of PTP. According to this theory, the antibody, which is detected in the acute phase of the PTP, would not yet have acquired the restricted specificity corresponding to it. This could enable it to react with a structure shared by the HPA-1a positive and HPA-1a negative platelets.

Aged↗

Hereditary macrothrombocytopenia and pregnancy.

INTRODUCTION: Hereditary macrothrombocytopenias (HM) are a group of infrequent disorders characterized by hereditary giant platelets. Little has been published about the course of these diseases during pregnancy and delivery. SUBJECTS AND METHODS: Forty consecutive thrombocytopenic pregnant women were studied. Platelet count, mean platelet volume and blood smear examination were performed. Platelet antibodies were studied by immunofluorescence. Familial study, bleeding time, ultrastructural platelet examination, a von Willebrand disease screening and aggregation tests were carried out when HM was suspected. RESULTS: Four cases of HM were diagnosed. Giant platelets were observed in all cases, with the typical ultrastructural pattern. Döhle-like cytoplasmic inclusions in granulocytes were observed in one case. Platelet antibodies were detected in only one case. No prophylactic measures to prevent haemorrhage were adopted, and all patients underwent vaginal deliveries. Haemorrhagic events were absent in both mothers and children. CONCLUSIONS: The prevalence of HM in pregnant trombocytopenic women is higher than assumed. Prophylactic treatment should be avoided in the absence of a history of haemorrhagic complications and obstetrical risk factors.

Adult↗

Aprotinin versus desmopressin for patients undergoing operations with cardiopulmonary bypass. A double-blind placebo-controlled study.

BACKGROUND: Aprotinin reduces blood loss in operations done with cardiopulmonary bypass, whereas the use of desmopressin remains controversial. We compared aprotinin, desmopressin, and placebo in a double-blind, randomized trial to evaluate bleeding and transfusion requirements. METHODS AND RESULTS: One hundred forty-nine patients (48 received aprotinin, 50 desmopressin, 51 placebo) were included. Blood loss and transfusion requirements were recorded and levels of Factor VIII coagulant activity, von Willebrand's factor, thrombin-antithrombin complexes, and D-dimer were measured. Overall blood loss was 195 +/- 146 ml/m2 in the aprotinin group, 400 +/- 192 ml/m2 in the desmopressin group, and 489 +/- 361 ml/m2 in the placebo group (95% confidence intervals: difference between desmopressin and aprotinin 98 to 312 ml/m2, p < 0.001; difference between placebo and aprotinin 190 to 398 ml/m2, p < 0.001). Twenty-six percent of patients treated with aprotinin, 66% of those treated with desmopressin, and 56% of those treated with placebo were given transfusion (95% confidence intervals: difference between aprotinin versus placebo plus desmopressin 51% to 71%, p < 0.001). Fibrinolytic activation throughout cardiopulmonary bypass was markedly higher with placebo or desmopressin administration. D-dimer level correlated with overall blood loss in patients receiving desmopressin or placebo, but not in those receiving aprotinin. CONCLUSION: Aprotinin administration reduces blood loss and transfusion requirements in cardiopulmonary bypass. This benefit may be explained by a lower activation of fibrinolysis.

Antithrombin III↗

[Blood donors seropositive for type 1 human immunodeficiency virus (HIV-1). Epidemiologic characteristics and clinical course].

PURPOSES: To evaluate retrospectively the epidemiologic profile and clinical course of the HIV-positive voluntary blood donors in the Sant Pau's Hospital Blood Bank (Barcelona-Spain) between 1986 and 1993. PATIENTS AND METHODS: A total of 119,345 blood donors were studied, and fifty seven such cases were identified. Risk behavior, causes of failure of the pre-donation procedures and clinical stage were analyzed. A follow-up of their infection was performed by the Infectious Disease Unit. RESULTS: The mean age of 57 seropositive donors was 31 +/- 8 years. Prevalence of HIV-1 infection among donors was 0.48 per 1000. Forty four (77%) were male and 13 (23%) female. Forty three (75%) were first time donors while 14 (25%) were repeat donors. The causes for the failure of the self-deferral questionnaire were: 42 subjects did not consider that they had engaged in "risk" behavior, seven donors lied in order to know if they were HIV carriers and two cases were driven to donate due to social or family pressure. The distribution of these donors according to risk behaviour was: 30 (53%) heterosexual, 11 (20%) homosexual, 11 (20%) intravenous drug users and five (7%) with no identified risk. It was noted that HIV infection progressed more rapidly to AIDS in HIV - positive homosexual donors than in heterosexual subjects (p < 0.05). CONCLUSIONS: Heterosexual donors who maintain sexual contacts with different partners without prophylactic measures for HIV infection currently represent the highest risk group for transfusion-related HIV infection. Clinical evolution of HIV infection was faster and more devastating in the homosexual group than in the heterosexual group.

Adult↗

[Thrombocytopenia associated with human immunodeficiency virus infection. Immunologic study of 60 patients addicted to parenteral drugs].

BACKGROUND: The immunologic study of 60 intravenous drug addict patients who were seropositive for the human immunodeficiency virus (HIV) and who developed thrombocytopenia (TP) is reported with the aim of establishing the relation of possible pathogenic factors which may trigger this complication. METHODS: In all the patients the presence of antiplatelet antibodies was studied by direct and indirect immunofluorescence together with crypto-antibodies, immune complexes, lymphocytotoxic antibodies, antiphospholipid antibodies, immunoglobulins, lymphocytic populations and subpopulations and serology against different infectious agents. RESULTS: Antiplatelet antibodies were detected in 71% of the patients of which 50% corresponded to immunoglobulins of IgG class, 12% to IgM, 21% to IgG plus IgM, 7% IgM plus IgA, 5% to IgG plus IgA and 5% IgG plus IgM plus IgA. In these patients a characteristic membrane fluorescence pattern was observed in which the fluorescein is distributed forming a thick, hard point. In 3 patients EDTA dependent crypto-antibodies were detected which in one case determined pseudothrombocytopenia. The immune complexes were demonstrated in 50% of the cases. Other findings were: hypergammaglobulinemia (86%), decrease in the CD4 population (47%), CD4/CD8 ratio < 1 (71%), lymphocytotoxic antibodies (70%), antiphospholipid antibodies (60%), and seropositivity for cytomegalovirus (62%), Epstein-Barr virus (10%) and hepatitis B virus (anti-HBc 75%, HBsAg 33%). CONCLUSIONS: Thrombocytopenia associated to infection by the human immunodeficiency virus in intravenous drug addict patients is due to the concurrence of multiple factors. The relevance of each may vary according to the risk practice of the collective analysed and even within the same group of some individuals or others. The numerous serologic findings in these patients fundamentally express the existence of a chronic polyclonal stimulation of B cells which may be initiated by the action of the drug itself and which becomes aggravated during the course of the multiple acquired infections among which that due to the human immunodeficiency virus is of note.

Adult↗

[Frequency of platelet alloantigens in the Spanish population].

PURPOSE: The aim of this study is to investigate the distribution and incidence of platelet alloantigens in Spain and to compare this frequency with that found in other populations. MATERIAL AND METHODS: Five hundred blood donors were phenotyped for the HPA-1a (Zwa), HPA-3a (Bak(a)), HPA-4a (Yukb/Pen(a)) y HPA-4b (Yuk(a)). One hundred of these donors were also phenotyped for the HPA-5a (Br(a)) antigen. Furthermore, we also phenotyped a group of 50 black individuals from a community of west African immigrants who live in the province of Barcelona. The typing of all the antigens except those of the HPA-5 system was carried out using the technique of Indirect Immunofluorescence. To type the antigens of the HPA-5 system the MAIPA technique was employed. RESULTS AND CONCLUSIONS: The incidence of the different platelet specific antigens in the Spanish population is, in general, the same as that detected in the other European countries and in the white population of the USA. In Europe and North America, the HPA-1a antigen is the most important of all owing to its involvement in alloimmune clinical disorders. In Asia, this role seems to be played by the HPA-4b antigen. Moreover, polymorphism for the HPA-1a allele does not seem to exist. The incidence of the HPA-5b (Br(a)) antigen in Spain, which is higher than that for France and Germany, suggests a high involvement of this antigen in our country in different alloimmune processes. The preliminary data obtained in a group of black west Africans suggest the absence on polymorphism for the HPA-1a allele and that the incidence of the remaining antigens is comparable to that of the Spanish population.

Africa, Western↗