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Biomedical subjects

M García-Fuentes

Publications and source records attributed to M García-Fuentes.

12 recordsLinked to original sources
PubMed1998

Cutaneous vasculitis in children and adults. Associated diseases and etiologic factors in 303 patients.

Cutaneous vasculitis (CV), a condition characterized by palpable purpura and nonspecific histopathologic findings, presents a diagnostic and therapeutic challenge because it may be a primary disorder or it may be a cutaneous manifestation of another entity, such as systemic necrotizing vasculitis, connective tissue disease, systemic bacterial infection, or malignancy. We studied 303 unselected patients (172 adults and 131 children) with CV to assess the disease associations and etiologic factors, to identify the frequency of primary and secondary CV in different age-groups, and to characterize features that help to distinguish between primary and secondary CV. Of the 131 children, 130 had primary CV: Henoch-Schönlein purpura (HSP) in 116 and hypersensitivity vasculitis (HV) in 14. In contrast, of the 172 adults, only 120 had primary CV: HSP in 39, HV in 70, and essential mixed cryoglobulinemia in 11. CV was a manifestation of systemic necrotizing vasculitis in 23 adults (polyarteritis nodosa in 17, Wegener granulomatosis in 4, and Churg-Strauss syndrome in 2). CV was secondary to other processes in 29 adults: in 20 patients CV was associated with connective tissue disease or another autoimmune or rheumatic disease, in 5 patients CV was a manifestation of severe bacterial infection, especially bacterial endocarditis (4 cases), and in the other 4 patients CV was the presenting symptom of an underlying malignancy. The patients for whom CV was a manifestation of systemic necrotizing vasculitis or secondary to a connective tissue disease, severe bacterial infection, or malignancy had clinical and laboratory data suggestive of the associated disorder. The clinical picture and outcome of primary CV in both children and adults were benign. By contrast, the prognosis of patients with CV in the context of systemic necrotizing vasculitis or secondary to other entities depended on the primary process. Given the different disease association in children and adults, we propose a simple diagnostic workup in children with CV. By contrast the diagnostic approach in adults with CV should be more cautious and the workup more extensive. The early differentiation between primary CV, secondary CV, and CV presenting as a symptom of systemic necrotizing vasculitis, especially in adults, is of paramount importance for an adequate diagnosis and appropriate treatment.

Adolescent↗
PubMed1997

Henoch-Schönlein purpura in adulthood and childhood: two different expressions of the same syndrome.

OBJECTIVE: To assess the possible differences between children (< or = 20 years) and adults (> 20 years) with Henoch-Schönlein purpura (HSP). METHODS: A retrospective study of an unselected population of patients with HSP who presented to our teaching hospital between 1975 and 1994. Patients were classified as having HSP according to the criteria proposed by Michel et al. RESULTS: Following the above-mentioned criteria, 162 white patients (113 male and 49 female) were classified as having HSP; 46 of the patients were adults (mean +/- SD age 53.2 +/- 16.9 years) and 116 were children (6.9 +/- 3.1 years). We were unable to identify any precipitating event in 72% of the adults and 66% of the children. The frequency of previous drug treatment, primarily antibiotics or analgesics, was similar in both groups, whereas previous upper respiratory tract infection was more frequent among the children (P < 0.02). At symptom onset, cutaneous lesions were the main clinical manifestation in both groups. However, adults had a lower frequency of abdominal pain (P < 0.008) and fever (P < 0.01), and a higher frequency of joint symptoms (P < 0.001). During the clinical course, adults had more frequent (P < 0.001) and severe renal involvement. An increased erythrocyte sedimentation rate was also more frequent in the adults (P < 0.001). Adults required more aggressive therapy, consisting of steroids (P < 0.002) and/or cytotoxic agents (P < 0.001). The outcome was relatively good in both age groups, with complete recovery in 107 children (93.9%) and in 33 adults (89.2%) after a mean +/- SD followup of 19.4 +/- 27.7 (median 12) and 21.8 +/- 33.5 (median 15) months, respectively. CONCLUSION: In adulthood, HSP, as defined by the criteria proposed by Michel et al, represents a more severe clinical syndrome, with a higher frequency of renal involvement. However, the final outcome of HSP is equally good in patients of both age groups.

Abdominal Pain↗
PubMed1997

[Blood coagulation, genetics and post-angioplasty restenosis].

Throughout the last few decades, different factors have been related to coronary stenosis which is clinically evidenced by coronary heart disease, the leading cause of death in developed countries. Different experimental models have contributed towards defining some of these factors, and to an understanding of the physiopathology of the atherosclerotic lesion. The genetic basis related to individual responses to the same event is currently being established. As endothelial injury reparative mechanisms are fundamental in atherosclerosis pathogeny, patients who experiment restenosis after undergoing revascularization procedures are useful human models in the study of these processes. We review from the literature the genetic factors related to thrombus formation, which may be associated with restenosis after percutaneous transluminal coronary angioplasty, in order to define the most suitable anticoagulant therapy for each patient. We refer to the recently characterized gene for the platelet receptors and its relationship with fibrinogenous, factor Xa, PAI-I, and the involvement of apolipoprotein (a) in the coagulation process.

Angioplasty, Balloon, Coronary↗
PubMed1997

PI SZ phenotype in chronic obstructive pulmonary disease.

BACKGROUND: A study was undertaken to clarify whether the PI SZ phenotype of the protease inhibitor system predisposes to chronic obstructive pulmonary disease (COPD). METHODS: The prevalence of PI Z and PI SZ deficient phenotypes was investigated in a population of 702 patients with COPD followed up at the Chest Unit of a tertiary hospital and in 15400 newborn infants from the same geographical area. Individuals with deficiency were detected by screening of dried blood spots on filter paper using a comparative electro-immunodiffusion technique for alpha 1-antitrypsin and transferrin. The serum phenotype was confirmed by means of isoelectrofocusing on polyacrylamide gel. RESULTS: Of the 702 blood samples from patients with COPD, six PI Z subjects (0.85%) and one PI SZ (0.14%) were detected. Of the 15400 samples from neonates, the number of PI Z subjects was eight (0.052%) and that of PI SZ was 24 (0.156%). The difference between the two groups was significant for PI Z but not for PI SZ. CONCLUSIONS: The data do not indicate an increased risk for development of COPD associated with the PI SZ phenotype but confirm the predisposition of PI Z individuals for the development of COPD.

Adult↗
PubMed1987

Hypomagnesaemia of hereditary renal origin.

Cases of hypomagnesaemia of hereditary renal origin represent at least three different congenital disorders of tubular reabsorption of magnesium (Mg). Isolated familial hypomagnesaemia has been reported in a heterogeneous group of patients and an autosomal dominant pattern of inheritance has often been found to be present. Familial hypokalaemia-hypomagnesaemia, inherited as an autosomal recessive trait, has been reported in 17 patients and we now describe 3 additional cases. Hypomagnesaemia is accompanied by hypokalaemia, metabolic alkalosis, hypocalciuria and moderate sodium chloride wasting. Titration of renal Mg reabsorption indicates the presence of a low threshold but a normal Tm. The inherited defect is probably situated at the level of the distal convoluted tubule and mimics the therapeutic effect of thiazides. This condition is frequently confused with Bartter's syndrome. Familial hypomagnesaemia-hypercalciuria, also inherited as an autosomal recessive trait, has been reported in at least 15 patients and we now add 3 new cases. Hypomagnesaemia is always accompanied by hypercalciuria and nephrocalcinosis. Ocular abnormalities such as myopia and horizontal nystagmus are often present. Hypermagnesiuria is of a greater degree than that observed in the previous entity and reflects a low Tm of Mg reabsorption. The defect must be situated at the level of the ascending limb of the loop of Henle and affects the transport of both calcium and Mg but not of sodium and chloride. This condition has not been clearly separated from hereditary distal renal tubular acidosis in the literature.

Humans↗
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↗
PubMed1981

[Poststreptococcal glomerulonephritis].

Acute poststreptococcal glomerulonephritis is one of the best defined renal diseases and it is the commonest form of acute glomerulonephritis in children. Antigen-antibody complexes formed in the circulation or "in situ" seem to play an important role in the pathogenesis and even though antiglobulins have recently been incriminated, there is still controversy over the nature of the antigen/s involved. Usually the clinical picture is very characteristic, but in those cases of atypical presentation mainly in those without urinary abnormalities, the diagnosis could be resolved by demonstration of well defined histological lesions. Although it is generally agreed upon, on the basis of clinical observations, that recovery from acute glomerulonephritis generally occurs in children, confusion still exists concerning the precise frequency with which chronicity may happen in this disease. There is no specific treatment for the immunological processes but symptomatic therapy has considerably reduced early mortality.

Antibodies, Anti-Idiotypic↗
PubMed1980

Serum levels of alpha 1-antitrypsin and Pi types in children with bronchiolitis.

Alpha 1-antitrypsin levels were determined at the beginning of the disease and after clinical recovery in a group of 51 infants with bronchiolitis, and the results were compared to those obtained in 24 normal infants and in 15 infants with viral bronchopneumonia. Distribution of Pi types in the patients with bronchiolitis was also compared to that observed in a control population of 170 blood donors of the same ethnic background. Both serum levels of alpha 1-antitrypsin and prevalence of non-M phenotypes were not statistically different from the values found in the control groups, thus not supporting the hypothesis that a deficiency of alpha 1-antitrypsin plays a role in the pathogenesis of bronchiolitis. A non explained finding was the lack of elevation of serum level of alpha 1-antitrypsin during the acute phase of the bronchiolitis process, a fact present in the group of patients with bronchopneumonia.

Bronchiolitis, Viral↗