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C Sierra

Publications and source records attributed to C Sierra.

At least 19 recordsLinked to original sources

Low-high and high-low biphasic injection forms in computed tomography examinations of the upper abdomen.

PURPOSE: To analyze the influence of different biphasic and monophasic injection rate protocols in abdominal computed tomography (CT). MATERIAL AND METHODS: A randomized, consecutive, parallel group study was designed and conducted in 60 patients studied with the same CT helical protocol. Patients were randomly distributed into three groups: (A) monophasic (120 ml at 2.5 ml/s); (B) low-high biphasic (120 ml, first 60 ml at a rate of 2 ml/s, the other 60 ml at 2.5 ml/s); and (C) high-low biphasic (120 ml, first 60 ml at a rate of 2.5 ml/s, the other 60 ml at 2 ml/s). All patients were injected with 300 mg I/ml non-ionic contrast media at a fixed delay time of 55 s. Contrast enhancement efficacy was evaluated by attenuation coefficient measurements. RESULTS: Although non-significant, monophasic protocol enhancements were higher than biphasic protocol enhancements in all measurements except aortic bifurcation (p=0.003). At this level, biphasic protocols obtained an increased mean enhancement from 7.6% to 2.5% compared to monophasic protocols. CONCLUSION: Monophasic contrast agent injection in helical CT of the upper abdomen produces a higher enhancement of parenchymal and venous structures. No significant difference was observed between low-high and high-low biphasic protocols.

Adult↗

Novel antihypertensive agents.

Coronary heart disease and cerebrovascular disease continue to be the leading causes of illness and death among adults from developed countries. Their prevalence is strongly related to the effects of many different risk factors, including high blood pressure, cigarette smoking, dyslipidaemia and diabetes. The management of cardiovascular (CV) risk factors should be viewed as an integrated strategy of intervention aimed at correcting as many of the underlying causes of CV disease as possible. Blocking the renin-angiotensin-aldosterone system with new, well-tolerated antihypertensive drugs, and blood pressure regulation by means of new drugs that also reduce plasma cholesterol levels or improve insulin resistance and glucose tolerance, could lead to a reduction of CV diseases.

Animals↗

Plasma thiols and their determinants in phenylketonuria.

OBJECTIVE: Treatment of phenylketonuria (PKU) patients consists of a phenylalanine-restricted diet supplemented with a tyrosine-, vitamin- and oligoelement-enriched amino-acid mixture. Vitamins and oligoelements may be deficient when compliance with the supplemented special formula is poor. Plasma thiol concentrations (especially homocysteine) depend mainly on B-vitamin intake. Our aim was to evaluate the plasma thiol concentrations (homocysteine, cysteine and glutathione) and their determinants (methionine, cobalamin and folate) in PKU patients under dietary treatment compared with age-matched controls. DESIGN AND SETTING: Cross-sectional study performed in a tertiary care Hospital. SUBJECTS: PKU (42) patients under dietary treatment compared with 42 age-matched controls. INTERVENTIONS: Plasma total homocysteine, cysteine and glutathione were analyzed by HPLC with fluorescence detection. Plasma phenylalanine and methionine were analyzed by ion exchange chromatography. Serum folate and cobalamin were analyzed by radioimmunoassay procedures. RESULTS: Total homocysteine concentrations were significantly lower in the PKU patients compared with the control group (Students t-test; P<0.0001). Serum folate and cobalamin were significantly higher in the PKU group (t-Student; P<0.0001) compared with controls. A significantly negative correlation was observed between total homocysteine and folate (r=-0.378; P=0.016), and between cobalamin and phenylalanine concentrations (r=-0.367; P=0.022) in the PKU group. CONCLUSIONS: Plasma total homocysteine values are lower in the PKU group than in the controls, probably because of high folate values. High phenylalanine values, an indicator of poor dietary compliance, are negatively associated with cobalamin, which might be deficient in some cases.

Adolescent↗

Low serum carnitine in HIV-infected children on antiretroviral treatment.

OBJECTIVE: HIV-infection and antiretroviral therapies are associated with energy dysfunction and lipid metabolism in adults. Our aim was to detect a possible carnitine deficiency in HIV-infected children on antiretroviral treatments. We analysed the relation among serum carnitine, its amino-acid precursors (methionine and lysine), clinical evaluation and antiretroviral therapy. DESIGN AND SETTING: Cross-sectional study performed in a tertiary care hospital. SUBJECTS: A total of 79 HIV-infected children on antiretroviral therapy, monitored prospectively in our hospital. INTERVENTIONS: Antiretroviral therapy included nucleoside analogues plus protease inhibitors and/or non-nucleoside analogues. Carnitine was analysed by an enzymatic-spectrometric procedure, and amino acids by ion exchange chromatography. Reference values of carnitine and amino acids were established in apparently healthy children who underwent presurgical analysis for minor surgery. RESULTS: Serum free and total carnitine, acylcarnitines, methionine and lysine were significantly lower in HIV-infected children compared with our reference values for similar ages (P<0.0001; Student's t-test). Low carnitine values were observed in 37% of our HIV-infected children. A significantly positive correlation was observed between serum total carnitine and methionine or lysine (P<0.0001 and P=0.005, respectively; Pearson test). No relation was observed between serum carnitine and clinical stage of HIV infection, immunological or nutritional status or lipodystrophy. Free and total carnitine were significantly lower (P=0.002 and 0.033, respectively) in HIV-infected patients on protease inhibitors (N=56) compared with those on other treatments (N=23). CONCLUSIONS: Low serum carnitine concentration was observed in 37% of our HIV-infected children on antiretroviral therapy. Malabsorption or defective synthesis may also account for the low serum carnitine values detected in these patients.

Adolescent↗

Intestinal permeability in adult patients with growth hormone deficiency.

Pathological disruption of the intestinal mucosa increases the paracellular pathway, leading to an increase in the penetration of large molecules. Since growth hormone (GH) has a trophic intestinal effect, we used a double marker test to enable examination of intestinal permeability, which reflects the state of integrity of the intestinal mucosa. We recruited 22 adult patients, mean age 54+/-13.3 years, with GH deficiency due to partial or total hypopituitarism. None had received GH treatment at any time, although they were all in optimized replacement therapy. A control group was composed of 19 healthy age-matched relatives. The intestinal permeability test was performed with lactulose (5 g) and mannitol (1 g) after an oral load of 100 ml of aqueous solution. The urinary lactulose/mannitol ratio and the percentages of lactulose and mannitol excreted were determined on a 5-h urine collection. There were no significant differences between the patients and the control group in the lactulose/mannitol ratio (0.087+/-0.059 vs 0.077+/-0.064, respectively) or in the urinary excretion percentages of lactulose (0.067+/-0.048% vs 0.073+/-0.070%, respectively) or mannitol (5.127+/-3.269% vs 5.068+/-2.985%, respectively). In conclusion, no increase in intestinal permeability was detected in patients with GH deficiency, so that in spite of the known trophic effects of GH on the epithelial crypt cells, there was no intestinal hyperpermeability in these patients.

Adult↗

Cerebral white matter lesions in essential hypertension.

The pathogenesis and clinical significance of cerebral white matter lesions (WML) remain controversial. Most studies have shown that age, hypertension, diabetes mellitus, and a history of stroke or heart disease are the most important factors related to the presence of cerebral WML. Moreover, some studies suggest that the presence of WML are closely related to cerebrovascular disease and cognitive impairment in elderly patients with vascular risk factors, particularly hypertension. In this review, different points of view about cerebral WML are discussed, with special focus on the presence of WML in essential hypertension. Some authors suggest that the presence of WML in hypertensive patients could be considered an early marker of brain damage.

Adult↗

Oxidative stress in Rett syndrome.

The investigation of parameters that might influence the neurological evolution of Rett syndrome might also yield new information about its pathogenic mechanisms. Oxidative stress caused by oxygen free radicals is involved in the neuropathology of several neurodegenerative disorders, as well as in stroke and seizures. To evaluate the free radical metabolism in Rett syndrome, we measured red blood cell antioxidant enzyme activities (superoxide dismutase, glutathione peroxidase, glutathione reductase and catalase) and plasma malondialdehyde, as lipid peroxidation marker in a group of patients with Rett syndrome. No significant differences were observed in erythrocyte glutathione peroxidase, glutathione reductase and catalase activities, between the Rett syndrome patients and the control group. Erythrocyte superoxide dismutase activities were significantly decreased in Rett syndrome patients (P<0.001) compared with the control group. Plasma malondialdehyde concentrations were significantly increased in Rett syndrome patients (P<0.001). An unbalanced nutritional status in Rett syndrome might explain the reduced enzyme activity found in these patients. Our results suggest that free radicals generated from oxidation reactions might contribute to the pathogenesis of Rett syndrome. The high levels of malondialdehyde reflect peroxidative damage of biomembranes that may contribute to progressive dementia, impaired motor function, behavioural changes, and seizures, in Rett syndrome. We found a probable relationship between the degree of oxidative stress and the severity of symptoms, which should be further investigated with a larger number of patients in different disease stages.

Adolescent↗

Sialylation is modulated through maturation in hemocytes from Macrobrachium rosenbergii.

In this work we identified in adult and juvenile freshwater prawn, Macrobrachium rosenbergii, three major type of circulating hemocytes: fusiform; rounded; and large ovoid hemocytes. Rounded and large hemocytes represent the first defense line, since this type of cells exerts phagocytic activity as well as lectin synthesis. Considering that glycosylation plays important roles in cell communication and as a target for pathogenic microorganisms, in this report was also described the main glycosidic modifications that occur in the large and rounded hemocytes from the freshwater prawn during maturation as determined with lectins. Neu5Acalpha2,6Gal, was identified homogeneously distributed in the membrane in 90% of hemocytes from juvenile organisms. Maturation of the freshwater prawn induced a decrease or complete loss of Neu5Acalpha2,6Gal residues that were replaced with Neu5Acalpha2,3 molecules in practically all hemocytes from adult organisms. This change was paralleled by a diminution in 9-O-acetyl-neuraminic acid (Neu5,9Ac(2)) expression. T and Tn antigens (Galbetal,3 GalNAcalpha1-0-Ser/Thr or GalNAcalpha1-0-Ser/Thr, respectively), as well as N-glycosidically linked glycans, seem to be highly conserved throughout maturation. Our results show that sialylation of freshwater prawn hemocytes is modulated throughout the maturation process.

Animals↗

Plasma phenylalanine is associated with decreased serum ubiquinone-10 concentrations in phenylketonuria.

Decreased serum ubiquinone-10 concentrations is a common condition in patients with phenylketonuria (PKU) under dietary treatment. Our aim was to investigate the implication of the metabolic abnormalities of PKU (low concentrations of tyrosine and high concentrations of phenylalanine) and the effect of treatment with phenylalanine-restricted diets in decreased ubiquinone-10 concentrations in PKU patients. We studied 30 PKU patients (age range 5 months to 35 years; median age 7 years) under dietary treatment. Correlation between plasma tyrosine or phenylalanine and serum ubiquinone-10 concentrations was investigated. Daily cholesterol intake was calculated from the data obtained through a dietary questionnaire. The index of dietary control (IDC) was calculated as the average of the medians of plasma phenylalanine concentrations obtained every 6 months in the metabolic control of patients. Negative correlations were observed between serum ubiquinone and the IDC (r=-0.46; p<0.01) in PKU patients. No correlation was observed between tyrosine or daily cholesterol intake and serum ubiquinone concentrations. After adjustment for daily cholesterol intake by multiple linear regression analysis, for each 113 units of increase in IDC values serum ubiquinone decreased 0.1 micromol/L. According to our results, the main factor associated with the decreased serum ubiquinone concentrations was high plasma phenylalanine values. Although daily cholesterol intake seems to be associated with ubiquinone concentrations, it may not be relevant if we take into account the low intake of cholesterol in treated PKU patients.

Adolescent↗

PCR-PRINS-FISH analysis of structurally abnormal sex chromosomes in eight patients with Turner phenotype.

According to cytogenetic analysis, about 50% of Turner individuals are 45,X. The remaining cases have a structurally abnormal X chromosome or are mosaics with a second cell line containing a normal or abnormal sex chromosome. In these mosaics, approximately 20% have a sex marker chromosome whose identity cannot usually be determined by classical cytogenetic methods, requiring the use of molecular techniques. Polymerase chain reaction (PCR), primed in situ labeling (PRINS), and fluorescence in situ hybridization (FISH) analyses were performed in 8 patients with Turner syndrome and 45,X mosaic karyotypes to determine the origin and structure of the marker chromosome in the second cell line. Our data showed that markers were Y-derived in 2 patients and X-derived in the remaining 6 patients. We were also able to determine the breakpoints in the two Y chromosomes. The use of cytogenetic and molecular techniques allowed us to establish unequivocally the origin, X or Y, of the marker chromosomes in the 8 patients with Turner phenotype. This study illustrates the power of resolution and utility of combined cytogenetic and molecular approaches in some clinical cases.

Adolescent↗

Hyperhomocysteinaemia and folate deficiency in human immunodeficiency virus-infected children.

BACKGROUND: Our aim was the detection of possible deficiencies of folate and cobalamin by the measurement of plasma total homocysteine (tHcy) in 69 human immunodeficiency virus (HIV) -infected children on antiretroviral treatment. We studied the relationship of these vitamins and methionine with tHcy values. MATERIALS AND METHODS: Plasma tHcy was determined by high-performance liquid chromatography with fluorescence detection, folate and cobalamin by competitive protein-binding chemiluminescence, and methionine by ion exchange chromatography. RESULTS: Significant differences were observed between tHcy concentrations in the HIV-infected patients and the reference values for children of similar ages (P < 0.0001). Folate values were significantly lower in HIV-infected children compared with our reference paediatric population (P < 0.0001), but cobalamin concentrations were similar between patients and reference values. A significantly negative correlation was found between tHcy and folate (r = - 0.596; P < 0.0001), and a significantly positive correlation between folate and the methionine : tHcy ratio (r = 0.501; P < 0.0001). Plasma tHcy was significantly higher (P = 0.008), while folate values and methionine : tHcy ratios were significantly lower (P = 0.007 and P = 0.042), in patients on protease inhibitor treatment than in patients on other antiretroviral therapies. CONCLUSIONS: The hyperhomocysteinaemia and low methionine : tHcy ratios observed in our group of HIV-infected children are probably a consequence of the low folate values, which interfere in the remethylation of homocysteine to methionine. Patients on protease inhibitor treatment showed significantly higher plasma tHcy concentrations, and lower folate values and methionine : tHcy ratios, compared with patients on other antiretroviral therapies. Hyperhomocysteinaemia is associated with the risk of premature stroke, which may have adverse consequences in the evolution of disease.

Adolescent↗

Quantification of lectin in freshwater prawn (Macrobrachium rosenbergii ) hemolymph by ELISA.

An enzyme-linked immunoabsorbent assay was developed to quantify the lectin present in the hemolymph of the freshwater prawn Macrobrachium rosenbergii. This method involves the use of murine monoclonal IgG1 with kappa light chain (designated as 3G1) antibodies raised against the purified lectin, the assay that we developed recognized as little as 30 ng/ml of lectin, and was used to measure the lectin concentration in animals at different maturation stages. The highest concentration of lectin was identified in the hemolymph from post-larval prawns and the lowest in molt stage adult animals. The hemagglutination activity of the lectin was four-fold higher in adult than in juvenile specimens, although in all cases N-acetylated sugar residues, such as N-acetyl-D-glucosamine, N-acetyl-D-galactosamine, and N-acetyl-D-neuraminic acid were inhibitors of the lectin activity, suggesting that lectin plays a role in the transport of N-acetylated sugar in juvenile prawns. Our results indicate that lectin concentration and hemagglutinating activity could be influenced by developmental conditions of the freshwater prawn.

Acetylgalactosamine↗

Chemical characterization of the lectin from the freshwater prawn Macrobrachium rosenbergii (De Man) by MALDI-TOF.

The serum of the freshwater prawn contains a sialic acid specific lectin (MrL) that agglutinates erythrocytes from rat and rabbit, as well as some Gram negative and positive bacterial strains. In this work, we performed the chemical characterization of the MrL purified by affinity chromatography on stroma from rat erythrocytes and by ion exchange chromatography. In its active form, MRL is a dimeric glycoprotein with 9.5 kDa per subunit. The amino acid sequence of the lectin was deduced from peptides obtained after trypsin treatment by matrix-assisted laser desorption ionization mass spectrometry-time of flight analysis (MALDI-TOF). The predicted amino acid sequence of the lectin showed 54% homology with the hyperglycemic hormone from Macrobrachium rosenbergii. It also showed homology with the variable region of the human immunoglobulin kappa (22%) and lambda (27%) light chains. The lectin is a glycoprotein with 11% (w/w) carbohydrate content and is constituted by Gal, Man, GlcNAc, GalNAc and NeuAc in a molar ratio of 4:3:2:1:0.6. The primary structure of the carbohydrate chains of the lectin from the freshwater prawn was determined by affinity chromatography of MrL-glycopeptides on Con A and LCA lectin columns, which indicated that the main carbohydrate chains conforming the lectin are N-glycosidically linked. Man3 GlcNAc2.1 oligosaccharides were the most abundant structures with 57%) followed by Gal1.3 Man3 GlcNAc2.8 with 24%. Our results suggest that the freshwater prawn possess a lectin in the hemolymph plasma, related to those from the immunoglobulin superfamily.

Animals↗

Lack of association between ACE gene polymorphism and left ventricular hypertrophy in essential hypertension.

The possible association between the insertion/deletion (I/D) polymorphism of the angiotensin I converting enzyme (ACE) gene and left ventricular hypertrophy (LVH) was investigated in a group of essential hypertensive patients. Seventy-one essential hypertensive patients (35 men and 36 women), aged 51 +/- 1 years, were genotyped by PCR for the I/D polymorphism of the ACE gene. Cardiac morphology and function were assessed by means of M-mode echocardiography. The relative frequencies of the three genotypes, DD, DI, and II, were respectively: 24%, 55%, and 21%. Mean values of left ventricular mass index were 145, 144, and 150 g/m2 for DD, DI, and II genotypes, without significant differences among them (P = 0.82). Likewise, the prevalence of LVH (76%, 64%, and 87%) was not significantly different among the three genotypes (P = 0.23). We conclude that the ACE gene I/D polymorphism is not associated with LVH in essential hypertension. Journal of Human Hypertension (2000) 14, 47-49.

Adolescent↗

Extra-laryngeal head and neck tuberculosis.

OBJECTIVE: Extra-laryngeal head and neck tuberculosis is exceptional. Therefore, a retrospective multicenter study in patients with head and neck tuberculosis, excluding solitary lymphadenitis and laryngeal locations was carried out. METHODS: We reviewed the patients with these features and tuberculosis confirmation by culture and/or histologic granuloma with presence of acid-fast bacilli (AFB). RESULTS: We found 16 patients with the following locations: eight in oral cavity and/or pharynx, four in ear, two in salivary glands, one in nose and one in frontal sinuses. The average duration of symptoms was 11.5 months. Purified protein derivative (PPD) was positive (> 10 mm) in all but one patient in whom it was performed (six of seven). Except tuberculous otitis, which occured without reactive lymphadenitis, this was present in 50% of the rest (six of the 12). In all cases a biopsy was required for diagnosis. Only in four patients, all with pharyngeal locations, was coincident pulmonary tuberculosis confirmed. One patient with tuberculous otitis developed meningitis and died; three additional patients (two with otitis) were cured but with sequelae; the evolution of the remaining patients was satisfactory with medical therapy. CONCLUSIONS: Extra-laryngeal head and neck tuberculosis has a slow course. The diagnosis is difficult due to the common absence of lung involvement and the usual requirements for biopsy procedures. The outcome is usually favorable with antituberculous drugs alone although in tuberculous otitis there are possibilities of complications.

Adolescent↗

Microalbuminuria in essential hypertension: clinical and biochemical profile.

This study aims to evaluate the clinical and biochemical profile associated with the presence of microalbuminuria in a group of essential hypertensive patients referred to a hypertension clinic. A total of 188 non-diabetic, untreated essential hypertensive patients (100 men, 88 women) aged 55.8 +/- 11.7 years are studied. Urinary albumin excretion was determined in two 24-h urine collections. Clinical and biochemical evaluations and 24-h ambulatory blood pressure (BP) monitoring were performed at baseline. Forty-two patients (22.3%) showed an increased urinary albumin excretion rate (20-200 micrograms/min). These patients showed significantly higher values (P < 0.01) for 24-h, daytime and night-time systolic and diastolic BP, compared with essential hypertensives with normal urinary albumin excretion. However, nocturnal reduction in BP did not differ between the groups. Furthermore, patients with microalbuminuria showed significantly higher (P < 0.01) creatinine, serum uric acid and triglycerides, as well as lower high-density lipoprotein (HDL)-cholesterol. In a multiple logistic regression analysis, a 24-h systolic BP > 140 mmHg (odds ratio: 3.19; 95% confidence interval [CI 95%]: 1.44-7.06) and a serum creatinine > 88 mumol/L (odds ratio: 3.08; CI 95%: 1.39-6.84) were the two factors associated independently with increased urinary albumin excretion. We conclude that, in essential hypertensive patients, the presence of microalbuminuria is associated with elevated BP, but not with its circadian pattern. Likewise, microalbuminuria is associated with the degree of renal impairment, and with increased uric acid and triglycerides and decreased HDL-cholesterol.

Adult↗