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Biomedical subjects

A Ribera

Publications and source records attributed to A Ribera.

At least 37 records · Page 2Linked to original sources

Essential monoclonal gammopathy with an IgM paraprotein that is a cryoglobulin with cold agglutinin and EDTA-dependent platelet antibody properties.

A patient with apparent anaemia and thrombocytopenia caused by a monoclonal paraprotein is described. The patient's serum contained a monoclonal IgM kappa, a cryoglobulin and a cold agglutinin. The cryoglobulin, similar to the serum paraprotein, was a monoclonal IgM kappa. Serum was studied to determine the relationship of the cryoglobulin with the cold agglutinin. The cryoglobulin and cold agglutinin were found to be the same paraprotein. Moreover, with absorption and elution techniques the reactivity of the autoantibody with both erythrocytes and platelets was demonstrated. Reports of cryoprecipitable cold agglutinins are rare and therefore this case is exceptional given that the IgM kappa paraprotein was found to be a cold agglutinin which was also reactive with platelets.

Aged↗

POEMS syndrome, steroid-dependent diabetes mellitus, erythema elevatum diutinum, and rheumatoid arthritis as extramedullary manifestations of plasma cell dyscrasia.

POEMS syndrome is a rare synopsis of different multisystemic disorders (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammapathy, and skin lesions) associated with plasma cell dyscrasia. We herein report the atypical case of a 44-year-old white man presenting with glomerulopathy, POEMS syndrome, and erythema elevatum diutinum with a few-year history of non-insulin-dependent diabetes mellitus (NIDDM) and seronegative rheumatoid arthritis (RA) as early manifestations of IgAlambda multiple myeloma. The prescription of 1 mg/kg/day prednisone improved the patient's features dramatically. Skin lesions improved by the association of glucocorticoids and plasma exchange, recurred when plasmapheresis ceased, and remitted when plasma exchange was reintroduced. NIDDM requiring insulinotherapy recurred when corticoids were discontinued and remitted when prednisone was reintroduced. However, prednisone and plasmapheresis had no effect on polyneuropathy, M-paraprotein, and plasma cell dyscrasia in our patient, who developed indolent multiple myeloma a few years later. We thus concluded that POEMS syndrome, steroid-dependent diabetes mellitus, rheumatoid arthritis, RA, and skin vasculitis in our patient were triggered by plasma cell dyscrasia.

Adult↗

Is prophylactic treatment after myocardial infarction evidence-based?

OBJECTIVE: We aimed to evaluate the use of evidence-based prophylactic treatment after myocardial infarction on hospital discharge and in primary care after 1 year of hospitalization. METHODS AND RESULTS: We conducted a 1-year prospective study of all the patients discharged from a tertiary hospital who had been treated for myocardial infarction from January 1 to December 31 1995. Three hundred and eighty surviving patients were consecutively discharged from the hospital. Seventy per cent of patients were treated with aspirin, 45% with beta-blockers, 27% with calcium channel blockers, 26% with ACE inhibitors, 40% with nitrates and 8% with cholesterol-lowering drugs after discharge from the hospital. In primary care, prescription of lipid-lowering drugs increased to 17%, while prescription of beta-blockers decreased to 34%. ACE inhibitor prescriptions at discharge were clearly more common in patients with impaired ventricular function or heart failure (57%). CONCLUSION: According to the evidence, there is still potential for reducing the risk of a further ischaemic event or death in patients with MI, especially by increasing the use of beta-blockers and lipid-lowering drugs.

Adrenergic beta-Antagonists↗

[Patients with acute coronary syndrome: therapeutic approach (management patterns) and 1-year prognosis in a tertiary general hospital].

BACKGROUND: To assess the determinants of short-term and one-year prognosis of all patients with suspected acute coronary syndrome seen by the cardiologist on duty in the Emergency Service of a tertiary hospital during a six month period. PATIENTS AND METHODS: 153 consecutive patients with a diagnosis of acute myocardial infarction, 225 with a diagnosis of unstable angina and 89 with a diagnosis of atypical chest pain were identified and their in-hospital characteristics and one-year prognosis were prospectively assessed. RESULTS: Age was higher than 65 years in 53% of acute myocardial infarction and in 54% of unstable angina patients. Only 3 patients were lost to follow-up. 35% of acute myocardial infarction patients had died or had reinfarction after one year and 16% of unstable angina patients had died or had suffered acute myocardial infarction. Baseline features, management patterns and prognosis of patients admitted with acute myocardial infarction to the Cardiology Service, to other hospital areas or to other hospitals were markedly different, and admission in areas other than the Cardiology Service was an independent mortality predictor. In unstable angina, complications happened in patients older than 75 years, those with previous revascularization procedures, those undergoing revascularization or those with lesions not deemed revascularizeable. CONCLUSIONS: a) In the study population there was a predominance of elderly patients; the proportion of patients with poor prognosis was considerably high; b) a sizeable proportion of patients with severe complications was scarcely represented in the major clinical trials; c) the possibility arises of a distribution of care resources tending to concentrate the greater therapeutic efforts in the patients with good prognosis.

Acute Disease↗

High prevalence of platelet autoantibodies in patients with systemic lupus erythematosus.

The aim of this study was to determine the prevalence of platelet autoantibodies (PAA) in patients with systemic lupus erythematosus (SLE) and its correlation with clinical and other laboratory manifestations of the disease, as well as to evaluate the influence of platelet count and disease activity on the result of the test for PAA. Ninety SLE patients, 29 with thrombocytopenia, were evaluated. The presence of PAA was determined using the direct and indirect platelet suspension immunofluorescence test. A total of 166 PAA determinations were performed in the 90 patients upon entry into the study. Fifty-six of the 90 patients (62%) with SLE were positive for PAA. There were no statistically significant correlations between the presence of PAA and the different disease manifestations except for thrombocytopenia (P = 0.0005). The presence of PAA in the same patient was significantly associated with current thrombocytopenia and disease activity. It was concluded that the prevalence of PAA in SLE patients is high. All SLE patients with thrombocytopenia had PAA, although some patients with PAA had a normal platelet count. The presence of PAA in SLE patients is not synonymous with thrombocytopenia but should instead be regarded as one of the multiple serological abnormalities which may develop in these patients.

Adolescent↗

The human platelet alloantigens, HPA-5(a+, b-) and HPA-5(a-, b+), are associated with a Glu505/Lys505 polymorphism of glycoprotein Ia (the alpha 2 subunit of VLA-2).

GP Ia/IIa (also called VLA-2 or alpha 2 beta 1) is the primary receptor for collagen on platelets. The human platelet alloantigens HPA-5a(Brb) and HPA-5b(Bra) have been found to reside on the platelet GP Ia/IIa complex. In order to establish the molecular basis of the HPA-5 system, platelet RNA was isolated form HPA-5 (a+, b-) and HPA-5(a-, b+) individuals. After reverse transcription, cDNA coding for glycoprotein Ia (GP Ia) was amplified by the polymerase chain reaction (PCR). Nucleotide sequence analysis of the PCR products revealed an A-->G polymorphism at base pair 1648 of the coding region of the mature protein, resulting in a substitution of lysine (AAG) in HPA-5b(Bra) by glutamic acid (GAG) in HPA-5a(Brb) at amino acid 505. Subsequent PCR-ASRA (allele-specific restriction enzyme analysis) with Mnl I using cDNA derived from three HPA-5 (a+, b-), one HPA-5 (a+, b+) individuals demonstrated that HPA-5a and -5b alleles are distinguishable by DNA typing. In addition to the A-->G substitution at base pair 1648, three silent mutations were identified, G-->C (195 bp), C-->T (837 bp), G-->A (1041 bp).

Antigens, Human Platelet↗

Cys209 Ser mutation in the platelet membrane glycoprotein Ib alpha gene is associated with Bernard-Soulier syndrome.

Molecular genetic analysis has been performed on a patient with Bernard-Soulier syndrome (BSS). The patient had characteristically giant platelets and was deficient in the glycoprotein (GP) Ib/IX/V complex, the von Willebrand factor (vWf) receptor on platelets. Previous studies with monoclonal antibodies directed against GP Ib alpha (CD 42b) and GP IX (CD 42a) demonstrated the absence of GP Ib alpha and presence of small amounts of GP IX on the surface of the patient's platelets. In this study the presence of GP V (CD 42d) is also demonstrated. This indicates a defect in the alpha-subunit of glycoprotein Ib. Therefore polymerase chain reaction (PCR)-amplification of the genomic DNA coding for GP Ib alpha was performed. Nucleotide sequence analysis of the entire coding region of GP Ib alpha revealed a homozygous single base pair mutation T-->A, leading to a single amino acid substitution cysteine-->serine at position 209 of the mature protein. We took advantage of the Mse I target site in the mutant allele, created by the T-->A mutation, to analyse all available family members. PCR-ASRA (allele-specific restriction enzyme analysis) using the restriction enzyme Mse I, revealed the heterozygosity of the mother and the two children of the patient, whereas homozygosity of the patient for the Cys209Ser mutation was confirmed. The sister of the patient was not found to be a carrier of the mutant allele. The mutation identified in the family studied, responsible for the deficiency of the GP Ib/IX/V complex, suggests that the cysteine at amino acid position 209 may be involved in disulphide bonding.

Adult↗

[Serologic and genomic markers of the hepatitis C virus in transfusion recipients with non-A, non-B hepatitis].

BACKGROUND: The exclusion of donors with antibodies against the c-100 protein of the hepatitis C virus (HCV) has permitted a considerable reduction in post transfusional (PTH) non A non B hepatitis in those receiving transfusions. However, the risk has not been completely eliminated and there is evidence that some HCV carriers do not present detectable antibodies against protein c-100. In these cases a new diagnostic methodology, named polymerase chain reaction (PCR), permits the detection of the viral genome. METHODS: One hundred fifty eight prospective transfusion recipients were studied. Nineteen (12%) developed non A non B PTH criteria. The presence of anti c-100 and the viral genome were investigated by PCR in 17 of the patients. RESULTS: Nine of the 17 receptors (53%) with non A non B PTH presented antibodies against the HCV. In all patients and in two seronegative patients genomic sequences of HCV were detected by PCR. CONCLUSIONS: By a combination of genomic (PCR) and serological technique, 11 of the 17 transfusion recipients (64%) with non A non B PTH demonstrated evidence of HCV infection. In two cases the infection was seronegative. Although the HCV appeared to be the principal agent of the non A non B PTH in this study it could not be recognized in a third of the recipients who developed non A non B PTH.

Adult↗

Sexual transmission of hepatitis C virus and its relation with hepatitis B virus and HIV.

OBJECTIVE: To determine the extent of transmission of hepatitis C virus in sexual partners of intravenous drug misusers and to examine the relation between the prevalences of HIV, hepatitis B virus, and hepatitis C virus infections in homosexual men and intravenous drug misusers and their sexual partners. DESIGN: Serum samples collected between 1984 and 1988 were tested for hepatitis B virus markers and antibodies against hepatitis C virus by enzyme linked immunosorbent assay (ELISA) and for HIV antibody by enzyme immune analysis and western blotting. SETTING: Large referral university hospital with an external AIDS clinic in the metropolitan area of Barcelona, Spain. SUBJECTS: 243 Intravenous drug misusers, 143 of their regular heterosexual partners, and 105 homosexual men. MAIN OUTCOME MEASURES: Prevalences of hepatitis C virus, hepatitis B virus, and HIV infections. RESULTS: In all, 178 of the 243 (73%) intravenous drug misusers, 16 out of 143 (11%) of their partners, and 17 of the 105 (16%) homosexual men had antibodies against hepatitis C virus. The presence of hepatitis C virus infection was unrelated to sex, age, the presence of HIV or hepatitis B virus infections, or the Centers for Disease Control stage of HIV. In sexual partners of intravenous drug misusers there were strong correlations between the presence of hepatitis C virus infection and that of HIV (p = 0.001) and hepatitis B virus (p = 0.013) infections. CONCLUSIONS: Intravenous drug misusers have a high risk of acquiring hepatitis C virus, hepatitis B virus, and HIV infections, but the presence of hepatitis C virus infection seems to be unrelated to the presence of the other two viruses. Homosexual men have a high prevalence of HIV and hepatitis B virus infections with a low prevalence of hepatitis C virus infection, the presence of which is not related to that of the other two infections. Conversely, heterosexual partners of intravenous drug misusers have low prevalences of the three virus infections, but the presence of hepatitis C virus infection correlates significantly with the presence of HIV and hepatitis B infections. The rate of sexual transmission of hepatitis C virus seems to be low, even in partners of people known to be seropositive for this virus.

Adolescent↗

[Primary infection caused by human immunodeficiency virus: clinical and serologic aspects of 7 cases].

We report 7 individuals in whom HIV seroconversion developed, in 6 associated with symptoms and in one without any symptom at all. The most florid and sustained symptoms appeared in the oldest patients: a female and her heterosexual partner, who was a promiscuous male with recent seroconversion. A heroin abuser had oral muguet, and another had esophageal candidiasis. Rash was absent in all cases. Antigenemia was demonstrated in 6 of the 7 patients. In one case, a third generation ELISA was more sensitive than Western blot for the identification of seroconversion.

Adult↗

[Evidence of HTLV-1 infection in different groups at risk in Barcelona].

HTLV-1 was the first human retrovirus to be isolated. It has been shown that it is the causative agent of T cell leukemia in the adult and some types of subacute myelopathies. The virus is transmitted by similar routes as the AIDS virus. HTLV-1 infection is endemic in South Japan, the Caribbean countries and some African areas. The prevalence of HTLV-1 infection in our country is unknown in the general population and in the groups at high risk. We report the preliminary results of a study of 1279 serum samples from high risk individuals from the Barcelona area. ELISA and Western blotting were used to detect antibodies. We found evidence of anti-HTLV-1 only in 4 of 905 (0.44%) parenteral drug abusers and in 1 of 102 western Africans. Anti-HTLV-1 were not found in the remaining sera from 62 homosexuals, 53 patients with leukemia and/or lymphoma, 3 with idiopathic spastic paraparesis and 154 with multiple blood transfusions. These results suggest that HTLV-1 has a very low diffusion in our area. The 4 positive heroin addicts are the first reported in our country with evidence of HIV and HTLV-1 coinfection.

Africa, Western↗