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Biomedical subjects

M C García Rodríguez

Publications and source records attributed to M C García Rodríguez.

At least 19 recordsLinked to original sources
PubMed1999

[Neutropenia as early manifestation of X-linked agammaglobulinemia. Report on 4 patients].

OBJECTIVE: The aim of this study was to determine the frequency of neutropenia associated to X-linked agammaglobulinemia (XLA) and to describe the clinical characteristics of the children diagnosed in our unit. PATIENTS AND METHODS: A revision of the medical records registered in our unit during a 28 year period (1970-1998) according to the diagnostic criteria of XLA was performed. We included in the study group those patients that expressed a neutropenia. Immunological studies by standard techniques were performed. RESULTS: Of the 37 patients fulfilling the diagnostic criteria of XLA, 4 cases had experienced episodes of neutropenia (10.81%). The frequency of neutropenia within the group without familiar antecedents was 15% and within the group with familiar antecedents 5.88%. In all cases, the neutropenia was present during a serious acute infectious disease. The neutropenia was transient and resolved promptly after the onset of antibiotic therapy in all patients. None of the patients experienced neutropenia while under therapy with intravenous gammaglobulin. CONCLUSIONS: The association of XLA and neutropenia seems to be sufficiently frequent as to include it in the differential diagnosis of neutropenia during infancy. It is important to consider a primary immunodeficiency diagnosis when a child presents neutropenia and a serious acute infectious disease. Quantification of serum immunoglobulin levels and enumeration of lymphocyte subpopulations can lead to an early diagnosis.

Agammaglobulinemia↗
PubMed1992

[Azidothymidine in the treatment of patients with human immunodeficiency virus infection and persistent generalized adenopathies].

The effectiveness and security of azidothymidine (AZT) in the treatment of patients with infection by the human immunodeficiency virus (HIV) and persistent generalized adenopathies (PGA), were assessed. Thirty six patients with HIV infection and PGA participate in the study. Eighteen were treated with AZT and the other 18 were included in the control group, since they did not accept the treatment. Both groups were homogeneous with respect to their clinical, immunological and virological characteristics. A common study protocol was used and the clinical, immunological and virological effectiveness was assessed. Lymphocyte subpopulations were quantified by flow cytometry, viral antigens were determined by sandwich-type ELISA and antibodies against viral proteins (anti-gp120, anti-gp160, anti-gp41, anti-gp24 and anti-p18) were detected by Western blot. Naranjo and Busto's algorithm was used for the causality of adverse effects. We did not observe any significant differences regarding the presence of infection and the evolution of AIDS in both groups. A positive response to thrombocytopenia was observed, more evident in patients under low doses of AZT. The small initial transitory improvement of the immunological parameters was not statistically significant. The viral antigen was not modified by the treatment. With respect to the behaviour of the several antibodies studied, no differences were observed. The initial doses of AZT had to be modified in 44% of patients due to their hematological toxicity, more frequent in the first stages of the treatment. In two patients, the treatment had to be finally discontinued due to severe neutropenia. 25% of patients showed mild to moderate gastrointestinal manifestations.(ABSTRACT TRUNCATED AT 250 WORDS)

AIDS-Related Complex↗
PubMed1992

[Long-term follow-up study of 35 children with ADS treated with zidovudine (AZT)].

Thirty-five children diagnosed of AIDS were studied in order to evaluate toxicity and efficacy of oral Zidovudine treatment (AZT), as well as to analyze the clinical, biochemical, immunological and virological evolution of HIV infection throughout the treatment. Patients (19 males and 16 females) were studied from April 1988 to May 1990 with a mean follow-up time of 13.5 months (SD = 6.7 months). The mean age of the group was 4.68 years. The means of acquisition of this disease was 71.45 vertical and 28.6% via hemo-derivatives. Tolerance has been good with the main toxicity being hematological (28.5% anemia and/or neutropenia), 23% of which required blood supplements. The presence of neurological involvement and thrombopenia were observed in the incidence of greater toxicity. No influence on weight during AXT treatment was observed and hepatosplenomegalia and adenopathies were not modified. Bacterial and opportunistic infections were observed in 97.1% and 20% of patients, respectively. Neurological evolution was irregular and the improvement observed in some patients was mild and transitory. Three patients died during the follow-up from intercurrent infectious process. A progressive increase in MCV and a tendency towards leucopenia and lymphopenia (mainly in hemo-derivative infected patients) was observed. Neither significant immunological nor virological changes were observed during the treatment (except the tendency to diminish basal hypergammaglobulinemia). The results of this study were compared to other pediatric series treated with AZT.

Acquired Immunodeficiency Syndrome↗
PubMed1991

Autoantibodies in patients with IgA and IgG2 deficiencies.

Patients with primary immunodeficiencies have a high incidence of autoantibodies, mainly of no clinical significance. It has recently been suggested that patients with a combined IgA-IgG2 deficiency have more autoantibodies than those patients with isolated deficiencies. We have studied 42 patients with selective IgA deficiency, nine with isolated IgG2 deficiency and 13 with combined IgA-IgG2 deficiency, and have found that the combined IgA-IgG2 deficiency has no influence on autoantibody prevalence, except for anti-IgA antibodies. The presence of chronic respiratory infections (a clinical feature commonly associated with both selective IgA and IgG2 deficiencies) is unrelated to the prevalence of autoantibodies. The most frequent autoantibodies found are anti-IgA and anti-cardiolipin. Most of the autoantibodies have been found to be devoid of actual clinical significance. Only three patients had overt autoimmune disease.

Adolescent↗
PubMed1991

Soluble class I histocompatibility antigens (s-HLA) and beta 2-microglobulin at delivery.

In this work we have quantified soluble class I histocompatibility antigens (s-HLA) and beta 2-microglobulin (beta 2 m) in sera of HIV+ or HIV- mothers at delivery and in cord blood sera of their newborn children. The results obtained for beta 2 m show that cord blood sera of newborn children have higher concentrations than their mothers, implying that most of the beta 2 m in the newborn is self-produced as described previously. s-HLA serum concentrations in the newborn children are significantly lower than in their mothers or in age-matched controls. Moreover, HIV+ mothers have significantly higher serum concentrations than HIV- mothers or an age-matched control group. These results suggest that s-HLA does not cross the placental barrier.

Female↗
PubMed1990

[Prospective immunologic study of mothers of HIV positive children].

From a cohort of 162 children born to 161 mothers belonging to risk groups for human immunodeficiency virus (HIV) infection, we have studied 32 asymptomatic HIV seropositive and 19 HIV seronegative mothers and their offspring seropositive mothers when compared with the seronegative group had lower counts of leukocytes, lymphocytes, CD4+ cells and CD4+/CD8+ ratio as well as higher IgG and IgM serum levels. The offspring from HIV seropositive mothers differed from children born to HIV seronegative mothers in having higher lymphocyte counts, serum IgG level and spontaneous in vitro IgG production. The number of lymphocytes and the IgG serum level correlated in the child HIV seropositive mother pairs. Two children born to HIV seropositive mothers had a CD4+/CD8+ ratio below 0.8. The significance of these abnormalities and its possible relationship with active HIV infection in children is at present unknown.

Adult↗
PubMed1988

Anticardiolipin antibodies in patients with primary immunodeficiency diseases.

The presence of antibodies to cardiolipin was determined (by an ELISA) in 143 patients with primary immunodeficiency diseases. Thirty (21%) had raised anticardiolipin antibody levels compared with only three in 98 age matched controls. The highest prevalence of this autoantibody was found in the Wiskott-Aldrich syndrome. Patients with selective IgA deficiency also showed a high prevalence of this autoantibody (32%), while patients with severe defects in antibody production showed a low prevalence or did not have such autoantibodies. This study provides further evidence of the association between autoimmune phenomena and primary immunodeficiency diseases.

Adolescent↗
PubMed1982

[Fractionated bone marrow transplantation in a case of severe combined immunodeficiency].

Authors present a male infant affected by severe combined immunodeficiency with a high percentage of circulating B lymphocytes. Pathogenetic mechanism underlying our patient's disease seems to be an intrinsic defect in the lymphoid stem cell. As no histocompatible donor could be found, an hemiallogeneic bone marrow transplantation of maternal origin, fractionated with soybean agglutinin, was performed. The patient died 27 days after transplantation. At this time the percentage of T cells in his peripheral blood rose to near normal values, and the percentage of B cells normalized, karyotype analysis demonstrated circulating cells of female origin. Necropsy showed a spleen with lymphoid follicles and germinal centres but no signs of graft-versus-host could be found. Fractionated bone marrow could be of paramount importance in the treatment of these patients, since it could circumvent graft-versus-host disease.

Adult↗