Synthesis of diaminosuberic acid derivatives via ring-closing alkyne metathesis.
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Alkynes↗
Biomedical subjects
B Aguilera
Publications and source records attributed to B Aguilera.
[Incidence and causes of sudden death in persons less than 36 years of age].
BACKGROUND: Epidemiological, clinical and pathological study of sudden death in children and young adults under 36 years old. SUBJECTS, MATERIAL AND METHOD: Sudden deaths between 1 and 35 years that occurred in Bizkaia (North of Spain) from 1991 to 1998 were analyzed. In all cases a complete forensic autopsy including histopathological, toxicological and occasionally microbiological studies were performed. Also, pathological antecedents were investigated. RESULTS: 107 sudden death cases between 1 and 35 years occurred, being the mortality rate of 2.4/100,000/year. Males were threefold at risk of sudden death than females. Mortality rate was higher in youngs than children. 4.5% of natural deaths were sudden deaths.43% were sudden cardiac deaths, and atheromatous coronary disease was the most frequent over 30 years. Myocardial diseases and conduction system abnormalities were common between 15 and 29 years. 39% were extracardiac sudden deaths. Infections were frequent in children, and epilepsy and asthma between 15 and 29 years. Intracraneal hemorrhage was also frequent. In 19 cases (18%), specially in children, the cause of death was unexplained. Cause of death was different according to gender and age. CONCLUSIONS: Sudden death is infrequent in children and youngs, and causes are numerous. This study supports the importance of complete forensic autopsies in order to encourage epidemiological and preventive studies on sudden death.
Adolescent↗
[Sudden death caused by atheromatous coronary disease in the young].
INTRODUCTION AND OBJECTIVES: Atheromatous coronary disease (ACD) is the most frequent cause of sudden death (SD) in adults. Few studies have focused in SD due to ACD in those patients under 35 years old. The aim of this study is to analyze the incidence, and clinical and pathological characteristics of ACD as a cause of death in young people. MATERIAL AND METHODS: We reviewed all SD cases in people from 1-35 years old which occurred in Bizkaia and in which there was a legal-medical autopsy from 1991 to 1998. A complete autopsy was performed in each case. SD due to ACD were analyzed, including pathological antecedents. RESULTS: 19 out of 107 SD (18%) occurred by ACD. All of them were males from 27 to 35 years of age. According to the male population from 30 to 35 years, the incidence was 3.7/100,000/year. In two patients ACD was diagnosed before death. Coronary risk factors were obtained in 10 cases and prodromal symptoms were described in 5 (chest pain in 4). In 79% death occurred during routine activity. None of the 19 patients arrived alive to hospital. In 6 cases multiple coronary disease was observed; coronary thrombosis in 8; recent acute ischemic myocardial necrosis in 4 and old ischemic damage in 7. 18 cases showed cardiac hypertrophy. CONCLUSIONS: ACD is an important cause of SD in young males, frequently being the first manifestation of the disease. As identification of groups at risk is so difficult and death occurs so quickly, primary prevention of ACD, rapid intervention of emergency services and educational programs in cardiopulmonar resuscitation for normal population are fundamental in reducing the mortality.
Adolescent↗
[Sudden death due to an anomalous origin of the left anterior descending coronary artery from the pulmonary trunk in a 9-year-old boy].
A 9-year-old healthy boy is presented, who complained of dizziness and to being exhausted after climbing numerous stairs, collapsing immediately after. Advanced resuscitation efforts were unsuccessful. At autopsy an isolated congenital anomaly in the origin of the left anterior descending coronary artery from the pulmonary trunk was found, with the origin of the right and left circumflex coronary arteries from the aorta, accompanied by with cardiac hypertrophy and chronic ischemic changes in the myocardium irrigated by the anterior descending artery. This represents a very infrequent variant that allows a longer survival and less functional repercussion if compared with those cases in which the main left coronary is originated from the pulmonary trunk.
Child↗
Sudden unexpected non-violent death between 1 and 19 years in north Spain.
AIMS: To study the epidemiological, clinical, and pathological characteristics of sudden unexpected non-violent deaths between 1 and 19 years. METHODS: Population based observational study of all sudden unexpected non-violent deaths between 1 and 19 years, from 1990 to 1997, in a north Spain county (Bizkaia). In each case, clinical information and circumstances of death were obtained and a complete forensic autopsy was performed. RESULTS: There were 34 sudden unexpected non-violent deaths. The mortality rate was 1.7 per 100 000 persons per year (representing 9% of the mortality rate of all non-violent deaths). In 10 cases the cause of death was cardiac, in 13 cases extracardiac, and 11 cases were unexplained. In 17 cases there were no pathological antecedents and in nine cases death occurred in patients with known disorders. Syncopes prior to death were present in five cases. Exercise related deaths occurred in seven cases (mainly associated with cardiac causes). Eight cases were "death in bed".
Adolescent↗
Epstein-Barr virus-latent membrane protein 1 expression has a favorable influence in the outcome of patients with Hodgkin's Disease treated with chemotherapy.
The effect of molecular factors in the outcome of Hodgkin's Disease (HD) is being currently studied. In a previous series of HD, including patients treated only with radiotherapy and patients treated with chemotherapy (with or without radiotherapy), we found that a high proliferation index had an adverse influence in overall survival (OS) and in the achievement of a complete remission (CR). Loss of Rb expression also had an adverse prognostic influence in achievement of CR. On the other hand LMP1-EBV expression had a favorable influence for OS. The expression of other molecular factors, p53, bcl2 and CD15 did not show prognostic influence. In the present paper we have studied the effect of these molecular variables in 110 patients, of the previous series who had been treated with chemotherapy. A retrospective study was performed in these 110 patients with HD treated with chemotherapy (ABVD or variants, 62%, or regimes not containing adriamycin, 38%) with or without adjutant radiotherapy, collected at the 11 centers belonging to the Spanish Collaborative Group for the Study of Hodgkin's Disease. The prognostic value of clinical variables and the expression of p53, bcl2, CD15, Rb, LMP 1-EBV and proliferative fraction demonstrated with sensitive immunohistochemical methods were studied. Cox's multivariate analysis was performed to assess their influence in failure-free survival (FFS) and OS. A multivariate logistic regression analysis was performed for studying the effect of the variables in the achievement of a CR. Of the clinical variables, only advanced stage (III/IV) had a significant independent adverse influence in FFS, in OS and in the achievement of CR and advanced age in OS. Of the molecular variables, LMP1-EBV had an independent and strong favorable influence in FFS, in OS and in the achievement of CR. Rb expression had a modest favorable influence in CR. The rest of the molecular variables had no independent influence on the outcome of the disease. In conclusion these results confirm the favorable prognostic value of LMP1-EBV expression in the subset of patients with HD treated with chemotherapy.
Analysis of Variance↗
[Arrhythmogenic cardiomyopathy as cause of sudden death in Spain. Report of 21 cases].
INTRODUCTION AND OBJECTIVES: Arrhythmogenic cardiomyopathy is a myocardial disease of unknown origin characterized by progressive replacement of right and/or left ventricular myocardium by fibrofatty tissue. Young adult people are more frequently affected and symptoms include palpitations, syncopes or sudden death. The objective of this study is to assess the frequency and characteristics of arrhythmogenic cardiomyopathy as a cause of sudden death in a series of cases in Spain. MATERIAL AND METHODS: Among the sudden death cases studied at the Toxicology Institute of Madrid between 1991 and 1997, those with arrhythmogenic cardiomyopathy diagnosis were analysed. RESULTS: 21 cases were diagnosed (0.62% of all sudden deaths and 6.8% of sudden deaths under 35 years). There were 20 males and one female (mean age 24.5 +/- 9.37 years); eleven (52.3%) died during sport activity and 8 (38%) had previous exercise related symptoms. In only two cases other members of the family had died suddenly and only one had been diagnosed in life. Biventricular involvement was observed in 13 cases; isolated involvement of the left ventricle in 4, and isolated involvement of the right ventricle in 4. CONCLUSIONS: Arrhythmogenic cardiomyopathy is a relatively frequent cause of sudden death in the young population in Spain. It is very often the first manifestation of the disease. The myocardial involvement is more frequently biventricular than isolated in the right ventricle. The diagnosis could be difficult for cardiologists, pathologists and forensic doctors.
Adolescent↗
Novel disaccharide inhibitors of human glioma cell division.
Several alpha-L-Fuc-(1-->3)-alpha-D-GlcNAcOC8H17 disaccharide derivatives bearing different hydroxylated alkyl chains, with or without sulfate groups at C-4 and/or C-6 positions of the GlcNAc unit, have been synthesized and tested as inhibitors of human astrocytoma lines U-373 and U-118. The antimitotic activity was dependent on the structure and position of the hydroxylated chain linked to the disaccharide. The compounds with a pentaerythritol or L-glyceryl chain at the C-6 position showed the best inhibitory properties, with an ID50 value of ca. 200 microM. On the contrary, sulfated disaccharide derivatives were inactive. The antimitotic activities of the compounds tested were essentially independent of the mitogen used to stimulate cell division.
Antineoplastic Agents↗
Histopathology of the conduction system in sudden infant death.
Because ventricular fibrillation or other arrhythmia have been suggested as a cause of unexplained sudden infant death, many authors focused their studies on the conduction system especially in the 1970s. These studies were not conclusive and many pathologists have abandoned the study of the conduction system. We have reviewed the conduction system of 87 sudden infant death cases between 15 days and 1 year old: 55 were SIDS cases; 17 had mild lesions insufficient to explain death and 15 cases were sudden explained deaths considered as controls. Fetal dispersion of the AV node and/or His bundle and left-sided His bundle, the most frequent findings in the three groups, can be considered normal variations. Nodoventricular tracts were observed in five cases from the three groups whereas fasciculoventricular tracts were present in seven cases of the SIDS group, one case of the second group and in no controls. We consider the possible pathologic significance of this accessory pathway in some SIDS cases. Other infrequent lesions were fibromuscular hyperplasia of AV node artery and His bundle hemorrhage in one SIDS case each; lymphocytic infiltration of the conduction system in one SIDS case and one case with myocarditis and left bundle branch fibrosis in a female infant with endocardial fibroelastosis.
Female↗
Conformational differences between Fuc(alpha 1-3) GlcNAc and its thioglycoside analogue.
NOE measurements and molecular mechanics calculations have been performed to study the conformational behaviour of Fuc(alpha 1-3)GlcNAc and its thioglycoside analogue in solution. Experimental data show that, in contrast with the natural O-disaccharide, which is basically monoconformational, the S-analogue shows two conformational families, namely syn and anti.
Carbohydrate Conformation↗
Adverse clinical outcome in Hodgkin's disease is associated with loss of retinoblastoma protein expression, high Ki67 proliferation index, and absence of Epstein-Barr virus-latent membrane protein 1 expression.
Previous studies have shown that in non-Hodgkin's lymphomas and others neoplasms, tumoral progression, treatment response, and outcome are related to the expression of different oncogenic and tumor suppressor proteins. This study aimed to determine the prognostic significance of the expression of p53, bcl2, retinoblastoma protein (Rb), Ki67, CD15, and latent membrane protein 1-Epstein-Barr Virus (LMP1-EBV) proteins in Hodgkin's disease. A retrospective study was performed on 140 patients collected at the 11 participating centers belonging to the Spanish Collaborative Group for the Study of Hodgkin's Disease. A highly sensitive immunohistochemical method with previous microwave-induced antigen retrieval technique was used for the demonstration of the above-mentioned proteins. A Cox's multivariate analysis was performed to evaluate the impact of the variables in the overall survival, together with a logistic regression model for the achievement of complete remission. Univariate statistical analysis confirmed the prognostic significance of the alredy known clinical parameters: stage, age over 60 years, and B symptoms. High proliferation index (Ki67) and loss of Rb expression were also found to be adverse prognostic factors influencing respectively lower overall survival and failure to achieve complete remission. Multivariate analysis confirmed the independent significance of these two parameters and additionally identifies LMP1-EBV expression as a favorable prognostic marker, in relation with overall survival. Histopathological type, p53, bcl2, and CD15 expression lack significant influence on the outcome of this series. The progression of the disease or the response to treatment in HD patients is the consequence of the interrelationship of different factors, among which LMP1 expression, loss of Rb, and high growth fraction seems to play a more relevant role.
Adolescent↗
Cytokine mRNA expression in lung tissue from toxic oil syndrome patients: a TH2 immunological mechanism.
In 1981, an epidemic occurred in Spain, toxic oil syndrome (TOS), in people who consumed rapeseed oil denatured with 2% aniline, and it was one of the largest intoxication epidemics ever recorded. In 1989, a similar disease, eosinophilia-myalgia syndrome (EMS) was reported in the USA and was associated with the ingestion of L-tryptophan. The pathologic findings in TOS showed primary endothelial injury, with cell proliferation and perivascular inflammatory infiltrates. Immunologic mechanisms have presumably been operative in the pathogenesis and perpetuation of TOS. Our previous findings pointed to a T-cell activation during acute phase of the disease. In order to analyze which T-cell subset is involved on TOS, we have developed an mRNA extraction procedure from paraffin-embedded lung tissues in patients with pulmonary involvement. We analyzed mRNA expression from different cytokines (IL-1, IL-2, IL-4, IL-5, IFN-gamma, GM-CSF) and CD25 (interleukin 2 receptor) and CD23 (low affinity IgE receptor), using RT-PCR technique. In lung tissues from these patients a T-cell activation was observed. We found a significant increase in Th1 (P = 0.006) and Th2 (P = 0.003) cytokine profile in TOS patients with respect to controls. The increment in TH2 response with respect to TH1 is significant (P = 0.03) in TOS lung specimens. Non-significant differences were obtained in other cytokines and receptors studied as IL-1, CD25, CD23 and GM-CSF. Data presented in this paper are the first clear evidence that an immunological mechanism is directly implicated in this illness.
Adolescent↗
Molecular analysis of the IgH gene in 212 cases of Hodgkin's disease: correlation of IgH clonality with the histologic and the immunocytochemical features.
The aims of this study are to evaluate the frequency of clonal immunoglobulin heavy chain gene rearrangements in paraffin-embedded samples of Hodgkin's disease (HD) with use of the polymerase chain reaction method and to correlate the molecular findings with the histologic and immunocytochemical features. DNA extracts from paraffin-embedded sections from 212 HD samples were used for amplification of the IgH gene by use of framework 2 and framework 3 region primers. Immunohistochemical studies were performed on paraffin sections by use of monoclonal antibodies for CD20 and latent membrane protein-1 and polyclonal antibody for CD3. With use of both primer combinations, monoclonality was detected in 18.7% of lymphocyte-predominant HD cases and in 32.2% of classical HD cases. These results suggest that immunoglobulin heavy chain gene clonal rearrangements are relatively frequent in classical HD. In addition, the statistical analyses of the genotypic and immunocytochemical data revealed that the detection of B-cell populations is significantly associated with the expression of CD20 on HRS cells. There was, however, no correlation between the histologic subtype, the percentage of HRS cells, the presence of latent membrane protein-1 expression, and the molecular analysis results.
Antigens, CD20↗
Liver disease patterns in hemodialysis patients with antibodies to hepatitis C virus.
The present study correlated histopathology and diagnostic tests in hemodialysis patients with serologic markers for hepatitis C virus (HCV). Hepatitis C virus infection was found in 65 of 163 patients, as assessed by anti-c100-3 (ELISA 1), anti-c22-3, c33C (ELISA 2), and RIBA 2. Several histopathologic patterns were found in 33 liver samples from HCV-positive individuals: cirrhosis (n = 3), chronic active hepatitis (n = 14), chronic persistent hepatitis (n = 2), isolated hemosiderosis (n = 5), reactive hepatitis (n = 6), and others (n = 3). There was a positive correlation between time from the first aminotransferase peak and histologic damage (P = 0.015). However, the severity of liver disease did not correlate with the intensity of RIBA 2 positivity, mean levels or pattern of aminotransferases elevation, or markers of past hepatitis B virus infection. Moreover, aminotransferases were persistently normal in three patients with severe liver disease and were elevated in 10 patients with only mild changes. In 19 biopsied patients, the presence of plasma HCV RNA was examined by the polymerase chain reaction (PCR), which was positive in 15 of the 19 biopsy specimens. The ability of PCR positivity to predict the histologic severity of the disease was insufficient: four patients with minor liver damage had positive PCR and two patients with significant liver damage had negative PCR. No further correlations of PCR positivity were found with the other biochemical or immunologic markers of HCV infection.(ABSTRACT TRUNCATED AT 250 WORDS)
Female↗
[The immunological characterization of 63 cases of Hodgkin's disease with a panel of 8 antibodies].
Sixty three cases of Hodgkin's disease are studied (two with lymphonodular predominance, 15 with diffuse lymphocyte predominance, 26 nodular sclerosis, 15 mixed cell and 5 lymphocyte depletion) with a panel of 8 monoclonal antibodies, material routinely used and included in paraffin: Ber H2 (CD30), Leu M1 (CD15), Common Leukocyte Antigen (CD45), L26 (CD20), MB2, UCHL1 (CD45 RO), MTI (CD43) and Epithelial Membrane Antigen. Ber H2 turned out to be the most usefull marker, positive in 100% of cases, independently of the histologic type. Positiveness with Leu M1 ranged from 100% (2/2 cases) of lymphonodular predominance, to 53.3% (8/15 cases) of diffuse lymphocyte predominance. The reactivity of the rest was variable, 1 though it is note worthy the positiveness of B markers (L26, MB2) in the two cases with lymphonodular predominance. In the other subtypes, reactivity with L26 was greater than that with MB2. T cell marker expression was minimal, except for the positiveness in 40% of cases (2/5) of the lymphocyte depletion type. In addition, the results of other series are revised and as a result the possible histogenesis of Hodgkin's disease is discussed.
Antibodies, Monoclonal↗
Ischaemic hepatic necrosis and intestinal infarction in a haemodialysis patient.
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Humans↗
Intraneural haemangioma in a patient with toxic syndrome caused by adulterated rapeseed oil.
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Fatty Acids, Monounsaturated↗
[The harmful biopsies of Kaiser Frederick III].
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Biopsy↗