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V Rodriguez-Valverde

Publications and source records attributed to V Rodriguez-Valverde.

At least 37 records · Page 2Linked to original sources
PubMed1994

Malignant ventricular arrhythmia in systemic sclerosis controlled with an implantable cardioverter defibrillator.

The prognosis of systemic sclerosis (SSc) is poor, and a significant number of patients suffer sudden death, probably related to malignant ventricular arrhythmias for which there is no reliable drug treatment. We describe a patient with SSc who had 2 documented episodes of ventricular fibrillation, that reverted after electrical defibrillation. An electrophysiological study performed after 3 weeks of oral loading with amiodarone 1200 mg/day, demonstrated the induction of unstable sustained syncopal ventricular tachycardia. She has been successfully controlled with the implant of a 3rd generation implantable cardioverter defibrillator. We suggest that this treatment should be seriously considered in those patients with SSc and malignant ventricular arrhythmias unresponsive to drug therapy.

Adult↗
PubMed1992

Self-limited autoimmune disease related to transient donor B cell activation in mice neonatally injected with semi-allogeneic F1 cells.

BALB/c mice injected at birth with 10(8) semi-allogeneic (C57BL/6 x BALB.IgHb)F1 spleen cells develop a lupus-like syndrome in which autoantibodies bear exclusively the donor allotype. We have analyzed the evolution of donor B cell chimerism and the autoimmune manifestations during the first year of life in these mice. Anti-DNA, -histone, and -cardiolipin IgG antibodies as well as circulating immune complexes appeared in the second week of life, reached the highest values around the sixth week, and then progressively dropped to normal values after the sixth month in most mice. The kinetics of the evolution of the autoimmune manifestations, as well as the kinetics of serum donor Ig allotype, were parallel to the kinetics of donor B cell chimerism, which was particularly prominent in the spleens in early weeks of life, and progressively decreased after remission of the autoimmune syndrome. Membrane-proliferative glomerulonephritis, which was followed as the more representative histological abnormality in this model, was particularly evident after 10 weeks of life, but disappeared by the end of the follow-up. Interestingly, when mice with a self-limited disease were re-injected with 10(8) F1 spleen cells i.v., a flare in the serological manifestations was observed. In these re-injected mice a predominance of anti-DNA, IgG1 antibodies bearing exclusively the donor allotype was also observed, as in the early weeks of life.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗
PubMed1991

Contribution of monocytes to the decreased lymphoproliferative response to phytohemagglutinin in patients with lung cancer.

Patients with lung cancer (LC) have a reduced T-cell proliferative response to phytohemagglutinin (PHA) compared with that of healthy individuals. This decreased response is a result of an inhibitory effect exerted by the monocytes as evidenced by: (1) a restoration to normal levels of the response to PHA when the peripheral blood mononuclear cells were depleted of adherent cells (AD) and (2) a dose-dependent inhibition of the response to PHA when the nonadherent cell population was co-cultured with increasing numbers of autologous AD cells. The addition of indomethacin to the cultures resulted in only a partial restoration of the response to PHA. Monocyte production of interleukin-1 from patients with LC in response to lipopolysaccharide was normal. These findings support the hypothesis that the AD cell population plays a major role in the low T-cell proliferative response to PHA in patients with LC. This suppressor effect is partially mediated by the prostaglandins released by the monocytes.

Adenocarcinoma↗
PubMed1990

Autologous mixed lymphocyte reaction and T-cell suppressor activity in patients with Henoch-Schönlein purpura and IgA nephropathy.

To assess the existence of persistent abnormalities in the cellular mechanisms regulating the immunoglobulin (Ig) synthesis in Henoch-Schönlein purpura (HSP) and IgA nephropathy, we studied through a hemolytic plaque assay (PFC) the response to the autologous mixed lymphocyte reaction (AMLR) and the T-cell suppressor activity in 24 patients with IgA nephropathy, in 20 individuals with inactive HSP (IHSP) and in 18 normal controls. In the group with IgA nephropathy there was a significant increase in the number of IgA-secreting cells after AMLR (p less than 0.01), and 9 of the 15 patients tested had an impaired generation of T-cell suppressor activity. No such abnormalities were found in individuals with IHSP. These findings support the existence of persistent defect in the mechanisms regulating the Ig synthesis, limited only to the patients with IgA nephropathy.

Adolescent↗
PubMed1990

Identification of the heterozygotes for deficiency of the beta-subunit of the eighth component of complement by reduced levels of C8 beta and increased amounts of free C8 alpha-gamma.

Sera from obligate heterozygotes for deficiency of the C8 beta subunit of the eighth component of human complement (C8) were analyzed for the molecular composition of C8. The C8 alpha-gamma and C8 beta subunits were separated by SDS-PAGE, visualized by immunoblotting, and the resulting bands were quantitated by laser densitometry. The laser densitometric absorption data were set to 100 arbitrary units (AU) for both subunits of pooled normal human sera. The AU values of individual normal sera ranged from 45 to 150 AU for C8 alpha-gamma (median 99 AU) and from 45 to 140 AU for C8 beta (median 101), whereas the C8 alpha-gamma/C8 beta-ratio varied from 0.7 to 1.4. Sera from C8 beta-deficient heterozygotes differed, as expected, from the normal sera for the markedly reduced levels of C8 beta (20 to 90 AU, median 55 AU) and for the higher C8 alpha-gamma/C8 beta-ratio (1.3 to 3.5). High voltage agarose gel electrophoresis was used to separate free and C8 beta-bound C8 alpha-gamma. The migration of free and C8 beta-bound C8 alpha-gamma subunit was checked by hemolytic overlay gels and by second dimension SDS-PAGE and immunoblotting. Immunochemical evaluation of C8 alpha-gamma using this system revealed about 5-14% free C8 alpha-gamma in sera with normal C8 and higher levels, from 33-71%, in the C8 beta D heterozygous sera. Functional analysis confirmed the substantial increase of free C8 alpha-gamma in the heterozygous group. We conclude that the C8 in C8 beta D heterozygous sera is characterized by increased amounts of free C8 alpha-gamma due to reduced concentrations of the C8 beta subunit. This finding may help to identify individuals heterozygous for C8 beta deficiency.

Complement C8↗
PubMed1989

Deficiency of the eighth component of complement. Evidence for linkage of C8 alpha-gamma pattern with C8 beta deficiency in sera of twelve patients.

The C8 alpha-gamma subunit of the eighth component of complement was analysed by sodium dodecyl sulphate-polyacrylamide gel electrophoresis and immunoblotting in sera from 68 normal individuals, 12 C8 beta-deficient patients (from seven unrelated families), and 10 of the parents of the latter. Three different forms of the C8 alpha-gamma subunit were observed: 34/68 normal individuals were found to have a C8 alpha-gamma triple band (termed C8 alpha-gamma 1, C8 alpha-gamma 2, C8 alpha-gamma 3 variants), 23/68 the C8 alpha-gamma 2 and C8 alpha-gamma 3 variants, and 11/68 the C8 alpha-gamma 1 and C8 alpha-gamma 3 variants. In contrast, all C8 beta-deficient patients had detectable C8 alpha-gamma 2 and C8 alpha-gamma 3 variants but lacked the C8 alpha-gamma 1 variant in addition to the C8 beta subunit. Three out of ten parents of the C8 beta-deficient patients were found to have the C8 alpha-gamma triple band, whereas 7/10, like their children, had the C8 alpha-gamma 2 and C8 alpha-gamma 3 variants only. We conclude that there is a linkage between the C8 alpha-gamma pattern and C8 beta deficiency. These data may support earlier findings that in humans the genes encoding for C8 alpha-gamma and C8 beta are closely linked on chromosome 1.

Adult↗
PubMed1988

Hereditary articular chondrocalcinosis. Clinical and genetic features in 13 pedigrees.

Thirteen pedigrees with familial articular chondrocalcinosis were identified through a systematic radiologic survey of the first-degree blood relatives of 76 patients with chondrocalcinosis. Forty-one persons, 30 women and 11 men, distributed in 25 sibships were affected. Their mean age at the time of study was 65.09 +/- 11.36 years. The disease was of early onset only in four pedigrees. The clinical manifestations in these four pedigrees were similar to those found in the kindred with a late onset. In 15 persons, the process was asymptomatic. In the 26 symptomatic patients, the arthropathy was mild, with clinical and radiologic features similar to those observed in sporadic chondrocalcinosis. There was no linkage of chondrocalcinosis to the HLA-A and HLA-B antigens in the 11 pedigrees in which tissue typing was performed. The pattern of involvement in these 13 pedigrees supports an autosomal dominant mode of inheritance. These data suggest that hereditary chondrocalcinosis is not infrequent and very often is clinically indistinguishable from the sporadic form of the disease.

Aged↗
PubMed1988

Circulating IgA producing cells in the differential diagnosis of Henoch-Schönlein purpura.

The number of immunoglobulin producing cells was determined by a plaque forming cell assay in 23 patients with definite Henoch-Schönlein purpura, in 5 children with probable Henoch-Schönlein purpura, in 11 patients with leukocytoclastic vasculitis not associated with a collagen vascular disease, and in 2 age matched control groups of healthy individuals. In the group with leukocytoclastic vasculitis, the numbers of circulating Ig producing cells were within normal limits, and lower than those found in definite Henoch-Schönlein purpura for IgA secreting cells (t = 8.26, p less than 0.001) and IgM secreting cells (t = 2.78, p less than 0.01). The number of circulating IgA secreting cells discriminated between definite Henoch-Schönlein purpura and leukocytoclastic vasculitis with a diagnostic sensitivity of 95.7% and specificity of 90.9%. Four of the 5 patients with probable Henoch-Schönlein purpura also had an increased number of IgA secreting cells. Therefore, an increased number of circulating IgA secreting cells in a patient with hypersensitivity vasculitis without a collagen vascular disease supports the diagnosis of Henoch-Schönlein purpura.

Adolescent↗
PubMed1987

Role of monocytes in the inhibitory effect of calcitriol on PHA-stimulated lymphocytes.

The possible role played by monocytes in the inhibitory effect of calcitriol on phytohemagglutinin (PHA)-stimulated lymphocyte proliferation was assessed by testing the effect of this sterol under different cell culture conditions. Calcitriol had a dose-dependent inhibitory effect on lymphocyte proliferation in concentrations ranging from 10(-10) up to 10(-8) M. The effect of 10(-9) M calcitriol was almost completely abolished by: a) monocyte depletion, b) inhibition of prostaglandin (PG) synthesis by indomethacin, and c) addition of exogenous interleukin-2 (IL-2). These results suggested that the inhibitory effect of calcitriol was mediated through monocytes. This possibility was substantiated by the following observations: a) the calcitriol inhibitory effect was restored when autologous adherent cells were added to monocyte-depleted PBM cells; b) the supernatant of adherent cells cultured for 24 hours in the presence of calcitriol exerted a marked inhibitory effect on lymphocyte proliferation; and c) this effect was not longer evident when adherent cells were cultured in the presence of calcitriol plus indomethacin. These data support the hypothesis that calcitriol acts, at least partially, through the monocytes, inducing an increased release of PG, with subsequent inhibition of IL-2 synthesis, then resulting in a decreased lymphocyte proliferation.

Calcitriol↗
PubMed1986

Immunoglobulin-producing cells in IgA nephropathy.

The number of peripheral blood mononuclear cells (PBMC) producing IgA, IgG and IgM spontaneously, after in vitro polyclonal stimulation with pokeweek mitogen (PWM) and in response to autologous mixed lymphocyte reaction (AMLR), were determined by a protein A hemolytic plaque assay in 23 patients with IgA nephropathy confirmed by renal biopsies and in 24 normal controls. The geometric mean of circulating IgA-producing cells in Berger's disease (689 +/- 1.73 cells/10(6) PBMC) was increased when compared with the normal controls (332 X divided by 1.52 cells/10(6) PBMC; p less than 0.001). To a lesser degree, there was also an increase in the number of IgG-secreting cells (98 +/- 3.97 cells/10(6) PBMC vs. 38 +/- 2.90 cells/10(6) PBMC; p less than 0.05). After PWM stimulation, although the number of IgA-producing cells was increased in patients with IgA nephropathy, no significant differences were observed between the 2 groups. In response to AMLR, the number of IgA-secreting cells was significantly higher in the cases with Berger's disease (1,979 +/- 1.76 cells/10(6) non-T cells vs. 783 +/- 1.95 cells/10(6) non-T cells; p less than 0.001). Although it did not reach statistical significance, the patient group had also an increase in the number of IgG-producing cells (884 +/- 2.64 cells/10(6) non-T cells vs. 317 +/- 5.05 cells/10(6) non-T cells). These data support the existence of some abnormalities in the mechanisms regulating the synthesis of IgA in Berger's disease which might contribute to its pathogenesis.

Adolescent↗
PubMed1984

Clinical profiles of patients with antibodies to nuclear ribonucleoprotein.

Currently there are no widely accepted criteria for the diagnosis of MCTD. In this work we attempted to define the clinical profile of a group of 68 patients with anti nRNP antibodies, detected by immunoprecipitation in 0.6% agarose. The diagnosis of each collagen vascular disease was established in every patient, who met with the strict diagnostic criteria either at clinical presentation or during the follow-up period. Twenty-eight patients had SLE, 9 had classical erosive RA, three had PSS and one had PM. The only distinctive features in the group of SLE with anti nRNP was an increased incidence of anti Sm antibodies (p less than 0.05). In the RA group there was a trend towards a high frequency of Raynaud's phenomenon and swollen hands. At clinical presentation twenty-seven patients did not fulfil enough criteria to be diagnosed of any of the well-defined collagen vascular disease. They presented an undifferentiated syndrome, characterized clinically by Raynaud's phenomenon (100%), swollen hands (88.9%) and joint symptoms (88.9%), with scarce tendency of developing severe systemic manifestations. The main laboratory abnormalities in this group were hypergammaglobulinemia, mildly increased ESR, abnormal levels of CIC, negative anti nDNA and anti Sm antibodies, and the virtual absence of hypocomplementemia. During a clinical course of 96 +/- 72.5 months only one patient evolved into another collagen disease (SLE). The clinical course in the remaining cases, was stable improving with low doses of prednisone and/or NSAID. We suggest considering this undifferentiated syndrome as a distinct entity, for which the already classical term of MCTD could be reserved.

Antibodies, Antinuclear↗
PubMed1983

Increased IgA-producing cells in the blood of patients with active Henoch-Schönlein purpura.

Peripheral blood lymphocytes (PBL) producing IgA, IgM, and IgG, both spontaneously and after pokeweed mitogen stimulation in cell culture, were determined by a protein A hemolytic plaque assay in 20 children with active Henoch-Schönlein purpura (HSP), 42 with inactive disease, 22 normal controls of the same ages, and 18 children with upper respiratory tract infections (URTI). The geometric mean of circulating IgA-producing cells in active HSP (1,016 X divided by 1.55 cells/10(6) PBL) was increased when compared with the group with inactive disease (P less than 0.001), normal controls (P less than 0.001), and children with URTI (542 X divided by 2.03 cells/10(6) PBL, P less than 0.05). The number of circulating IgM-producing cells was slightly increased in active HSP (260 X divided by 2.65 cells/10(6) PBL) and URTI (256 X divided by 3.16 cells/10(6) PBL). Both values were higher than those found in the group with inactive disease (113 X divided by 2.26 cells/10(6) PBL, P less than 0.01). There were no significant differences among the 4 groups in the number of circulating IgG-producing cells. The number of cells producing each type of immunoglobulin after in vitro stimulation with pokeweed mitogen was similar in patients with active HSP, normal controls, and the group with inactive disease. These data demonstrate a selective increase in the number of circulating IgA-producing cells only during disease activity, and add further support to a possible pathogenic role of this immunoglobulin in HSP.

Adolescent↗
PubMed1982

Hemiplegia and peripheral gangrene secondary to large and medium size vessels involvement in C.R.E.S.T. syndrome.

A woman with CREST syndrome since the age of 35, had 11 and 13 years respectively after her disease onset, two episodes of CVA with residual right side hemiplegia. The angiography revealed segmented stenosis in the left common carotid, right subclavian and left renal arteries. At the age of 49 she developed gangrene of the right foot, requiring below the knee amputation. Pathological examination of the surgical specimen, showed extensive intimal fibrosis of the vessel walls in large and medium size arteries. Involvement of large and medium size arteries is infrequent in scleroderma. The case described illustrates this severe and unusual complication.

Arteries↗
PubMed1980

Familial chondrocalcinosis. Prevalence in Northern Spain and clinical features in five pedigrees.

The first-degree consanguineous relatives of 46 patients with calcium pyrophosphate dihydrate (CPPD) crystal deposition disease were examined for the presence of articular chondrocalcinosis. In 5 cases the process was familial, with 17 persons in the oldest living generation (mean age 69 +/- 7.4) showing radiographic evidence of calcified cartilage. The clinical syndrome was characterized by a female predominance, late onset of symptoms with mild arthritic manifestations, and oligoarticular chondrocalcinosis. These data suggest that the familial type of CPPD crystal deposition disease is more frequent than formerly thought.

Adolescent↗
PubMedDate not supplied

Familial aggregation of polymyalgia rheumatica and giant cell arteritis: genetic and T cell repertoire analysis.

OBJECTIVE: Several reports of familial aggregation of giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) have been described although detailed genetic and immunological studies are scarce. Our aims were to investigate the influence of HLA-DRB1 alleles and to analyze the phenotype and T cell receptor (TCR) usage of circulating T lymphocytes in a familial case of GCA and PMR. METHODS: HLA-DRB1 typing was carried out using polymerase chain reaction amplification with specific primers. The study of the circulating T cell repertoire was performed by staining with specific monoclonal antibodies and flow cytometry analysis. RESULTS: Patient 1 developed GCA at the age of 71, four years prior to the diagnosis of PMR in her older brother. The HLA-DRB1 typing of Patient 1 was DRB1*04 (DRB1*0401)/DRB1*12 and in Patient 2 was DRB1*07/DRB1*12. In our patient population, GCA was associated with an increased frequency of HLA-DRB1*04 compared with PMR patients. Regarding T cell phenotype, the brother with active PMR had a higher expression of surface markers indicating activation in both T cell subsets (CD25 and HLA-DR). The sister with GCA showed a pronounced decrease of CD4+/CD45RA+ T cells with respect to her brother with PMR. Both patients carried a significant depletion of CD28 in both subsets, specially within the CD8+ T cell compartment. The BV gene usage differed from one patient to the other. T cell expansions were identified in both patients but the specificities were different. CONCLUSION: We describe an association of GCA and PMR between two first degree relatives with significant genetic and immunologic differences. Our results suggest that the pathogenic mechanisms leading to the development of GCA and PMR are probably multifactorial, and both genetic and environmental factors may contribute to the development of these diseases.

Aged↗
PubMedDate not supplied

Radiographic features of hereditary articular chondrocalcinosis. A comparative study with the sporadic type.

To assess the radiological features of hereditary articular chondrocalcinosis, we performed a blind comparative study between 21 randomly selected patients with hereditary disease and 21 cases of sporadic pseudogout matched for age and sex. Each individual had AP projections of the hands, pelvis and knees. The films were evaluated for the presence of articular chondrocalcinosis and for the severity of the associated degenerative arthropathy. A grade of 0 to 3+ was assigned to each of the 4 variables of osteoarthritis: joint space narrowing, sclerosis, osteophytosis and subchondral cysts. The mean number of joints with chondrocalcinosis and its distribution was similar in both groups. In addition, no differences were found in the overall severity of the associated degenerative arthropathy. In both groups the disease was characterized by oligoarticular calcification and a mild degenerative arthropathy. These data along with data from other reported pedigrees, show that the radiological appearance in the hereditary type is frequently indistinguishable from that commonly observed in sporadic articular chondrocalcinosis.

Aged↗