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Biomedical subjects

J Girona

Publications and source records attributed to J Girona.

At least 19 recordsLinked to original sources

[Total anomalous pulmonary venous return in children: importance of echocardiographic diagnosis and early surgery].

OBJECTIVE: We studied patients who underwent surgical repair for total anomalous pulmonary venous return at our hospital. We report the importance of diagnosis by echocardiographic imaging before surgical treatment. METHODS: Within the period of 1990-1999, fourteen patients underwent surgical repair of this cardiopathy in our hospital. The type of anomalous drainage was supracardiac in 6 patients, infracardiac in 4, to the coronary sinus in 1, and mixed-type in 3 patients. Eleven cases were diagnosed with an echo-Doppler study, the findings being confirmed intraoperatively. RESULTS: There were 2 early deaths: one occurred in the operating room in a patient with a small left ventricle, and the second one was 35 days postoperatively as a result of a septic complication. Early in the postoperative period our primary goal has steadily been the control and treatment of pulmonary hypertension. After a mean follow-up time of 50 months, only 1 patient needed to be reoperated on and the remainder are symptomless. CONCLUSIONS: That sufficient diagnostic data on total anomalous pulmonary venous return can reliably be obtained by ultrasound scanning so that surgery can be promptly undertaken, and that its surgical risk is currently low and mid-term post-repair outcome is fairly good.

Child↗

Simvastatin decreases aldehyde production derived from lipoprotein oxidation.

Treatment with statins are known to lower plasma and low-density lipoprotein (LDL) cholesterol levels with resultant prevention and regression of atherosclerosis. It has been recently suggested that the action of the statins may also have a direct effect on other mechanisms involved in the atherosclerotic plaque formation. Thus, we investigated whether simvastatin could have an antioxidant effect on plasma lipoproteins. The rate of oxidation of LDL and high-density lipoproteins (HDL) was measured by conjugated diene formation with and without the addition of increasing concentrations of simvastatin (in vitro) and in patients with and without treatment with simvastatin (in vivo). A strong correlation was observed between increasing simvastatin concentration and the lag phase, a negative correlation was observed for maximal rate and maximum diene production in LDL samples (r2 = +0.97, p <0.0001; r2 = -0.92, p <0.0001; r2 = -0.98, p <0.0001, respectively). For HDL no clear correlation could be established with the lag phase, but a strong negative correlation was also observed between simvastatin concentration and maximal rate and maximum diene production (r2 = -0.69, p <0.01; r2 = -0.98, p <0.0001, respectively). After 6 hours of oxidation the production of aldehydes in LDL and HDL was lower (30% and 5%, respectively) in samples obtained during simvastatin therapy with respect to those obtained without treatment. The 2,4-decadienal showed a decrease of 37% and 64% (p <0.05) in both oxidized-LDL and oxidized-HDL particles, respectively, with simvastatin treatment. Our findings demonstrate that simvastatin acts as an antioxidant in lipoprotein particles and, together with its lipid-lowering properties, could play an important role in preventing atherosclerosis.

Aldehydes↗

Hepatic production of apolar aldehydes in rats with carbon tetrachloride-induced cirrhosis.

The aim of this study was to identify apolar aldehydes in liver homogenates from rats with CCl4-induced cirrhosis and, as a corollary, the antioxidant effect of zinc administration. The study was performed in five control rats and in ten cirrhotic rats which were further sub-divided into two groups to receive either a standard diet or one supplemented with zinc. The percentage of hepatic fibrosis, plasma malondialdehyde concentration and alanine aminotransferase activity were measured as well as the following aldehydes: hexanal, octanal, decanal, 2-hexenal, 2-octenal, 2-nonenal, 2,4-heptadienal and 2,4-decadienal. Of the 10 cirrhotic rats, 4 had elevated concentrations of the highly toxic 2,4-dialkenals which coincided with a higher percentage of fibrosis and plasma alanine aminotransferase activity. These aldehydes were not observed in the control group. Zinc administration was associated with a reduction of the hepatic malondialdehyde concentration and an amelioration on the degree of hepatic injury. In conclusion, this study demonstrates the presence of the highly toxic 2,4-dialkenals in hepatic tissue of rats whith CCl4-induced cirrhosis. Results obtained would suggest that these particular aldehydes may be related to the severity of the hepatic injury.

Aldehydes↗

Vitamin A is linked to the expression of the AI-CIII-AIV gene cluster in familial combined hyperlipidemia.

There is growing evidence of the capacity of vitamin A to regulate the expression of the genetic region that encodes apolipoproteins (apo) A-I, C-III, and A-IV. This region in turn has been proposed to modulate the expression of hyperlipidemia in the commonest genetic form of dyslipidemia, familial combined hyperlipidemia (FCHL). The hypothesis tested here was whether vitamin A (retinol), by controlling the expression of the AI-CIII-AIV gene cluster, plays a role in modulating the hyperlipidemic phenotype in FCHL. We approached the subject by studying three genetic variants of this region: a C1100-T transition in exon 3 of the apoC-III gene, a G3206-T transversion in exon 4 of the apoC-III gene, and a G-75-A substitution in the promoter region of the apoA-I gene. The association between plasma vitamin A concentrations and differences in the plasma concentrations of apolipoproteins A-I and C-III based on the different genotypes was assessed in 48 FCHL patients and 74 of their normolipidemic relatives. The results indicated that the subjects carrying genetic variants associated with increased concentrations of apoA-I and C-III (C1100-T and G-75-A) also presented increased plasma concentrations of vitamin A. This was only observed among the FCHL patients, which suggested that certain characteristics of these patients contributed to this association. The G3206-T was not associated with changes in either apolipoprotein concentrations or in vitamin A. In summary, we report a relationship between genetically determined elevations of proteins of the AI-CIII-AIV gene cluster and vitamin A in FCHL patients. More studies will be needed to confirm that vitamin A plays a role in FCHL which might also be important for its potential application to therapeutical approaches.

Adult↗

Evidence against alterations in Lecithin:cholesterol acyltransferase (LCAT) activity in familial combined hyperlipidemia.

Elevated concentrations of plasma cholesterol and triglycerides are characteristic of familial combined hyperlipidemia (FCHL) which may also present with reduced high density lipoprotein (HDL) cholesterol concentrations. Lecithin:cholesterol acyltransferase (LCAT) plays a key role in reverse cholesterol transport by converting unesterified cholesterol to cholesterol ester in the process of maturation of HDL in the presence of its activator, apolipoprotein (apo) A-I. We hypothesised that alterations in LCAT activity or plasma concentrations or gene sequence of apo A-I could influence HDL metabolism in these patients. We studied cholesterol concentrations of high density lipoprotein subfractions and LCAT activity in 25 FCHL subjects and 48 controls. Total HDL (p=0.018) and HDL2 (p=0.008) were significantly decreased in the FCHL group compared with controls. After analyses with adjusted data only HDL2 remained significantly decreased in the FCHL group (p=0.050). The LDLc/HDLc and A-I/HDLc ratios were significantly elevated in the FCHL group (p <0.0001), the latter suggesting the existence of compositional differences in the HDL particles of the FCHL individuals. LCAT activity assessed in the FCHL (19.94+/-3.95 nmol/ml per h) and control (20.13+/-6.86 nmol/ml per h) groups showed no statistically significant differences. A significant positive correlation of LCAT activity with total HDL (r=0.42), HDL3 cholesterol (r=0.46) and apolipoprotein A-I (r=0.47) was observed in affected subjects but not in controls. An association between a Ga(-75)-A variation in the promoter region of the apo A-I gene and elevated concentrations of apo A-I (p=0.009) and apo C-III (p=0.041) was observed. This association was strongly influenced by the status of the subject providing further evidence for a regulatory role of this genetic region in the expression of FCHL. Our data suggests that LCAT activity is normal in FCHL and, therefore, does not account for the abnormalities observed in these patients essentially with regard to the HDL2 subfraction.

Adult↗

[Early postoperative complications after different methods of intestinal reconstruction in deep anterior rectum resection--a prospective study].

For the first period after low anterior resection and total mesorectal excision for rectal cancer, the colonic J-pouch is superior to the straight or lateroterminal anastomosis. Despite the simple technique and short stretch of sutures, the LTA is unsuitable due to the high postoperative morbidity and because the reconstruction functions poorly. If the expected anastomis is below 6 cm from the anal verge, i.e. 4 cm above the sphincter muscle, the colonic J-pouch is recommended.

Adult↗

Identification of a genetic locus for familial atrial fibrillation.

BACKGROUND: Atrial fibrillation, the most common sustained cardiac-rhythm disturbance, affects over 2 million Americans and accounts for one third of all strokes in patients over 65 years of age. The molecular basis for atrial fibrillation is unknown, and palliative therapy is used to control the ventricular rate and prevent systemic emboli. We identified a family of 26 members of whom 10 had atrial fibrillation which segregated as an autosomal dominant disease. We subsequently identified two additional families in which the disease was linked to the same locus. METHODS: We screened the human genome with 300 polymorphic dinucleotide-repeat markers using an unconventional strategy of pooling the DNA samples into two groups (affected and unaffected), which reduced the sample size by approximately 90 percent, before performing linkage analysis to map the locus. This made it possible to identify potential loci within a few weeks. RESULTS: The lod scores for markers D10S569 and D10S607, located at 10q22-q24, were 3.60 in Family 1. The disease locus in Families 2 and 3 was also linked to the same markers, with lod scores of 6.02 and 5.35 for markers D10S569 and D10S607, respectively, when data on all three families were combined. Haplotype analysis of the three families showed that the locus was between D10S1694 and D10S1786, an interval of 11.3 centimorgans. CONCLUSIONS: Identification of the gene for familial atrial fibrillation will help to elucidate the molecular basis of the disease and provide insights into acquired forms. The strategy of pooling DNA samples for analysis is more time and cost effective than conventional screening and should accelerate the process of gene mapping in the future.

Adult↗

[Biofeedback].

The overall success rate of biofeedback in ano-rectal incontinence and chronic constipation is reported to be 50-92% and 35-90%, respectively. In patients with neurogenic incontinence, the results are poor. Biofeedback, in case of outlet obstruction, seems only to be useful if there is paradoxical contraction of the external anal sphincter and/or the puborectalis muscle during straining (anismus). Further investigations to select patients who would profit from this therapy are, therefore, necessary.

Anal Canal↗

[Familial auricular fibrillation].

INTRODUCTION AND OBJECTIVE: We present two families with atrial fibrillation in 20 of 50 members, during three generations, with known cardiac rhythms, in order to communicate their infrequent existence and the most relevant clinical facts. METHOD: Clinical situation, evolution, ECG and ECHO findings, treatments and complications related with the disease are investigated. RESULTS: The presence of atrial fibrillation in 20 members is demonstrated, although one of them was on sinus rhythm at the time of the study; 3 patients had left ventricular enlargement on the ECHO study; the clinical situation was good in all patients except two who died because of complications related to the arrythmia and a third patient that had a brain stroke. The patients received different treatments because they where controlled by different physicians; the possible lethal proarrythmic effect in such cases must be taken into account. CONCLUSION: Familiar atrial fibrillation is a very infrequent arrythmia, usually well tolerated, that follows a dominant autosomic hereditary pattern. The use of antiagregants is advised because of the risk of embolism, or the use of anticoagulants in the presence of associated risk factors. Electric cardioversion has been show not be useful. The possible proarrythmic effect of some antiarrythmic agents, used in the control of cardiac frequency, must be taken into account.

Adolescent↗

[Double-outlet left ventricle with subaortic interventricular defect and pulmonary stenosis: surgical correction with cryopreserved aortic homograft].

Double outlet left ventricle with subaortic ventricular septal defect and pulmonary stenosis is a very rare congenital heart disease. We present a case of this cardiopathy which underwent correction consisting of ventricular septal defect closure and right ventricular outflow tract reconstruction using a cryopreserved aortic homograft, with a postsurgical follow-up time of 18 months.

Blood Vessel Prosthesis Implantation↗

Oleic acid rich diet protects against the oxidative modification of high density lipoprotein.

Oxidative modifications of lipoproteins could contribute to the development of atherosclerosis, but the influence of dietary fats on high density lipoprotein (HDL) oxidative modification is unknown. This study was designed to determine whether a diet rich in oleic acid could modulate the oxidative modification of HDL3. Twenty two healthy men were randomly placed on a 32-wk crossover study of an oleic acid rich diet supplied by a variant of sunflower oil vs a linoleic acid rich diet provided by conventional sunflower oil. Plasma HDL3 obtained after the diet rich in oleic acid showed a significantly higher oleic acid content in the phospholipid than lipoprotein isolated after the linoleic acid rich diet. HDL3 isolated after the oleic acid rich diet had lower values of thiobarbituric acid reactive substances (TBARS) than HDL3 obtained after the linoleic acid rich diet both for native (mean +/- SE; 0.24 +/- 0.02 vs 0.42 +/- 0.08 nmol MDA/mg protein; p < 0.01) and copper oxidized HDL3 (0.75 +/- 0.06 vs 0.95 +/- 0.07 nmol MDA/mg protein; p < 0.01). Indeed, TBARS for native HDL3 were negatively correlated with the oleic acid to linoleic acid ratio and positively with the percentage of linoleic acid in their phospholipids. Interestingly, HDL3 after both diets had similar antioxidant vitamins A and E content. HDL3 overall composition and fluidity were similar after the two diets. Moreover, HDL3 obtained after both diets produced identical [3H] free cholesterol efflux from human monocyte-derived macrophages (29%) and fibroblasts (26%). In conclusion, HDL3 rich in oleic acid was less easily oxidized regardless of the content of antioxidants such as vitamins A and E. Therefore, dietary monounsaturated fatty acid prevent the oxidative modification of lipoproteins.

Cholesterol↗

Oxidized lipoproteins including HDL and their lipid peroxidation products inhibit TNF-alpha secretion by THP-1 human macrophages.

It has been established that oxidized LDL (ox-LDL) modifies cytokine secretion by macrophages, for example, by reducing tumor necrosis factor alpha (TNF-(alpha) m-RNA. However, little is known about the effects of oxidized high density lipoprotein (ox-HDL). This study reports the effects of ox-HDL subfractions 2 and 3 (ox-HDL2, ox-HDL3) compared with that of ox-LDL and some products of oxidation (hydroperoxides and aldehydes) on the secretion of TNF-alpha from THP-1 human monocytes derived macrophages in vitro. HDL2, HDL3 and LDL were oxidized with 10 microM Cu++ for 12 h and/or 24 h. Native and oxidized HDL and LDL were incubated for 24 h with macrophages with or without LPS (10 ng/ml) after which TNF-alpha secretion was measured in the culture medium. Lipid hydroperoxides and apolar aldehydes were also incubated with the cells for 2 h following which the medium was replaced and TNF-alpha secretion measured after a further 22 h of incubation. An inhibition of TNF-alpha by ox-HDL2 (p < .05), ox-HDL3 (p < .05) and ox-LDL (p < .05) from THP-1 macrophages was observed in the presence and absence of LPS. This inhibition remained the same after incubation with ox-HDL 12 h and 24 h. Hydroperoxides of linoleic acid did not modify TNF-alpha secretion by cells while five out of eight aldehydes analyzed (2,4-heptadienal, hexanal, 2-nonenal, 2-octenal, 2,4-decadienal) inhibited TNF-alpha secretion (p < .05). These findings demonstrate that ox-HDL, and some of its lipid peroxidation products, plays a role in the modulation of the inflammatory response by macrophages as previously observed for ox-LDL.

Aldehydes↗

Low plasma vitamin A concentrations in familial combined hyperlipidemia.

As many as 20% of the survivors of acute myocardial infarction present with the heritable form of hyperlipidemia, termed familial combined hyperlipidemia (FCHL). Some of the genes reported to be involved in this disorder, such as those for lipoprotein lipase (LPL) and apolipoprotein (apo) C-III, are controlled by a peroxisome proliferator-activated receptor (PPAR)/retinoic acid receptor X (RXR) regulatory system, which is retinoic acid dependent. If, as we hypothesized, the availability of retinoic acid or its precursor retinol (vitamin A) could be altered in FCHL, this could help explain some aspects of the phenotypic expression of the disease. We therefore measured plasma retinol concentrations in 30 FCHL subjects and 56 controls. Plasma retinol concentrations in FCHL subjects were significantly lower than that of control subjects (1.96 +/- 0.83 mumol/L vs 2.91 +/- 1.23 mumol/L, respectively; P < 0.0001). This novel finding of significantly decreased concentrations of plasma retinol in FCHL relative to control subjects gives support to the hypothesis that vitamin A might be involved in the expression of this disorder.

Adult↗

[Fistula, fissure, abscess].

Anal fistulas, anal fissures and anal abscesses are common disorders of the anorectal region that can often only be cured durably by operation. The acute anal fissure can usually be treated successfully by conservative means, whereas the chronical anal fissure can be cured by local fissurectomy as a rule. Primary anal abscesses and anal fistulas are different phenomena resulting from an infection of the proctodeal glands. The acute form, the anal abscess, always requires wide opening and drainage. Caudal intersphincteric and -trans-sphincteric anal fistulas can be cured by the division method. Types of anal fistulas that occur higher can be treated effectively by a special operative technique developed on the basis of the method of Parks.

Abscess↗

[Left aorto-ventricular tunnel. Diagnosis with transthoracic and transesophageal echo-Doppler].

Aortic-left ventricular tunnel is an infrequent congenital heart lesion consisting of an abnormal tubular-shaped communication between the aortic root and the left ventricle that bypasses the aortic valve. It is clinically undistinguishable from aortic insufficiency. Its prompt and precise diagnosis is crucial since early surgical treatment is definitely indicated, in contrast to aortic insufficiency. In our case, two-dimensional and pulsed color-flow Doppler imaging established the diagnosis, and the transesophageal approach was a great aid in detecting the aortic opening with its turbulent diastolic flow, and in tracing its intramyocardial course. This paper may be of interest not just as a new case-report but insofar as it stresses how importantly the transthoracic and transesophageal echocardiographic and Doppler scanning contributes to its diagnosis and therapeutic indication.

Adolescent↗

[The follow-up of transposition of the great arteries corrected by Senning's technic].

INTRODUCTION AND OBJECTIVES: The incidence of late complications after a physiological correction of the patients with transposition of the great arteries (D-TGA) is very significant, due to the alternative operation of the arterial switch. METHODS: We studied 125 patients with D-TGA, treated with Senning surgical correction between december of 1978 and november of 1990. Surgery was performed at a mean age of 11.7 months (from 7 days to 11.2 years), and the postoperative mean follow-up was 7.3 years (from 1.4 to 14.3 years). We analyzed their evolutive clinical condition, ECG, Holter and echocardiogram-Doppler. Four groups were defined: A) Simple, 48.8%. B) Associated with ventricular septal defect, 22.4%. C) With pulmonary stenosis, 15.2%. D) Both anomalies, 13.6%. RESULTS: Sixteen children died (12.8%), 11 of them on the postoperative period. The remaining 5 patients died, at a mean time of 34.3 months after surgery, because they were in cardiac failure. All of patients had enlargement of right ventricle and tricuspid regurgitation was observed in 39 children. There were 3 reoperations. Atrioventricular block was observed in 5.7% of the patients, 33.3% were not in sinus rhythm, 6.6% had atrial flutter-fibrillation, sinus node dysfunction was observed in 24.7%, and five permanent pacemakers were implanted (4.7%). CONCLUSIONS: The later mortality is not high, and the clinical outcome is good, but the frequent rhythm disturbances and enlargement of the right ventricle could let us conclude the hypothesis that anatomical correction is an optimal alternative procedure.

Analysis of Variance↗