Health-related quality of life in patients with spinocerebellar ataxia. / Calidad de vida relacionada con la salud en pacientes con ataxias espinocerebelosas.

INTRODUCTION: The progressive deterioration of patients with spinocerebellar ataxia (SCA) has a major impact on their health-related quality of life (HRQOL). This study evaluates HRQOL in a sample of patients diagnosed with SCA and aims to estimate the predictive ability of a set of sociodemographic variables for the different dimensions of the General Health Questionnaire. METHODS: A total of 80 patients diagnosed with SCA were assessed using a sociodemographic questionnaire and the SF-36 General Health Questionnaire. The sociodemographic variables studied were sex, age, presence of a carer, employment status, and time elapsed from diagnosis of the disease. RESULTS: The 8 subscales of the SF-36 show positive and significant correlations to one another. Mean scores obtained on each SF-36 subscale differ between women and men, although this difference is significant only on the general health subscale, with men scoring higher than women. We found significant age differences on the vitality and social function subscales, with higher scores among younger patients (< 34 years). The variable 'presence of a carer' accounts for most of the total variance of the questionnaire. CONCLUSIONS: The SF-36 is a valid and useful instrument for evaluating HRQOL in patients diagnosed with SCA. Presence of a carer seems to be a determinant of self-perceived quality of life in these patients.

[Health-related quality of life in patients with amyotrophic lateral sclerosis]. / Calidad de vida relacionada con la salud en pacientes con esclerosis lateral amiotrófica.

INTRODUCTION: Progressive deterioration in patients with amyotrophic lateral sclerosis (ALS) has a major impact on their health-related quality of life (HRQOL). The objectives of this study are to evaluate HRQOL in a sample of patients diagnosed with ALS and estimate the predictive capability of a set of sociodemographic variables for the different scales covered by a general health survey. METHODS: A total of 63 patients diagnosed with ALS were assessed using a sociodemographic questionnaire and the SF-36 general health survey. The sociodemographic variables studied were sex, age, presence of a caregiver, employment status, and time from diagnosis of disease. RESULTS: The SF-36 survey shows positive correlations between the different scales composing it, which proves its reliability. The mean scores obtained for each of the SF-36 scales were higher in men than in women, although the only statistically significant difference was for the Physical Role scale. The lowest age range (less than 56 years) presented the highest mean scores for most of these dimensions. Most of the variance in the test is explained by the variable 'presence of caregiver'. CONCLUSIONS: The SF-36 health survey has been confirmed as a valid and useful tool for evaluating HRQOL in ALS patients, and it discriminates between patients in different states of health according to their level of dependency.

Seroepidemiology of Toxoplasma gondii infection in a Mennonite community in Durango State, Mexico.

There is a lack of information concerning the epidemiology of Toxoplasma gondii infection in Mennonites (an ethnic group of Mexican citizens of German descent living in rural communities). The prevalence of anti- T. gondii immunoglobulin (Ig) G and IgM antibodies was examined in 152 Mennonites in Durango State, Mexico, using enzyme-linked immunoassays. In total, 46 (30.3%) of 152 participants (mean age 38.4 ± 15.5 yr) had IgG T. gondii antibodies; 5 (3.3%) also had IgM T. gondii antibodies. Toxoplasma gondii infection was significantly associated with the presence of cats at home (adjusted OR  =  3.93; 95% CI: 1.40-11.05), raising cattle (adjusted OR  =  3.88; 95% CI: 1.24-12.11), consumption of pigeon meat (adjusted OR  =  3.0; 95% CI: 1.36-6.63), and consumption of untreated water (adjusted OR  =  2.42; 95% CI: 1.09-5.40). This is the first report of seroprevalence and contributing factors for T. gondii infection in Mennonites and of an association of the consumption of pigeon meat with T. gondii infection. Results of this study should be useful in the design of optimal preventive measures against T. gondii infection.

Seroepidemiology of Toxoplasma gondii infection in pregnant women in rural Durango, Mexico.

The epidemiology of Toxoplasma gondii infection in pregnant women in rural Mexico is largely unknown. The seroepidemiology of T. gondii infection in 439 pregnant women from 9 communities in rural Durango State, Mexico was investigated. Using commercial enzyme-linked immunoassays, sera were tested for T. gondii IgG, IgM, and avidity antibodies. Prevalences of T. gondii IgG antibodies in the communities varied from 0% to 20%. Overall, 36 (8.2%) of the 439 women had IgG T. gondii antibodies. Ten (2.3%) women had also T. gondii IgM antibodies; IgG avidity was high in all IgM-positive women, suggesting chronic infection. None of the women, however, had delivered a known T. gondii-infected child. The seroprevalence was significantly higher (P < 0.05) in women from low socio-economic conditions (14%) than in those with higher socio-economic status (6.6%). Multivariate analysis showed that T. gondii infection was associated with soil floors at home (adjusted OR = 2.89; 95% CI: 1.12-7.49). This is the first epidemiological study of T. gondii infection in pregnant women in rural Mexico.

The effect of sildenafil on ocular blood flow.

Sildenafil is a potent phosphodiesterase (PDE) 5 inhibitor that is used for patients with erectile dysfunction. Sildenafil induces vasodilation in selected smooth muscle via increased levels of guanosine 3', 5' cyclic monophosphate and increase in nitric oxide. The vasodilatory effects of the PDE 5 inhibitors led us to review its effect on the ocular vasculature. Sildenafil appears to increase blood flow velocity significantly in the retrobulbar and choroidal circulation. Most studies suggest an increase in choroidal blood flow, with a lesser effect on the retinal vasculature.

Characterization of hemoglobin Hotel Dieu in a Puerto Rican adolescent.

PURPOSE: Hemoglobin Hotel Dieu (HbHD) is a high-oxygen affinity variant of HbA never before reported in a Hispanic patient. This Hb variant was first reported in 1981 by Blouquit et al. in a white person with erythrocytosis with a substitution in the beta 99 aspartic acid residue by glycine. METHODS: A 13-year-old Puerto Rican boy had pain in his chest, headaches, easy fatigability, and high Hb (as high as 19.1 g/dl). Protein analysis was performed by cellulose acetate, citrate agar, and isoelectric focusing electrophoresis and high-pressure liquid chromatography (HPLC), polymerase chain reaction (PCR) amplification, and DNA sequencing of the second exon of the beta gene in samples obtained from the mother, father, and the patient, and DNA fingerprinting to determine paternity. RESULTS: The variant found in the patient migrated on cellulose acetate electrophoresis to a cathodic position relative to HbF, and a band cathodal to HbA and close to HbF on isoelectric focusing electrophoresis. The patient showed an abnormal well-resolved peak on HPLC with a retention time slightly shorter than that for HbS. DNA analysis by direct sequencing of the PCR product demonstrated heterozygosity for codon 99 (GAT-->GGT) in the patient but not in either parent. DNA fingerprinting by multiplex PCR amplification of three simple tandem repeat loci showed that the patient shared alleles in all three loci with both parents, ruling out nonpaternity. CONCLUSIONS: The protein and DNA analysis indicate that the erythrocytosis is caused by the presence of HbHD in this Hispanic adolescent.

Differentiation between isolates of Helicobacter pylori by PCR-RFLP analysis of urease A and B genes and comparison with ribosomal RNA gene patterns.

Genetic diversity amongst 21 human gastric isolates of Helicobacter pylori was investigated by polymerase chain reaction amplification and HaeIII digest (restriction fragment length polymorphism) analysis of an internal 2.4-kb segment of the urease A and urease B genes. H. pylori from 11 independent individuals yielded nine distinct restriction fragment patterns but only one pattern was common to H. pylori from two individuals. By contrast, multiple isolate sets of H. pylori from two patients each had common urease gene patterns. Most strains with the same urease gene patterns were distinguishable in their ribosomal RNA gene patterns. The study demonstrated diversity amongst H. pylori and established that PCR analysis of urease genes provided a novel method of identifying isolates. The profiles were reproducible and convenient to obtain and analyse, and were almost as discriminatory as HaeIII ribopatterns.

[Conjugate vaccines against bacterial infections: typhoid fever]. / Vacunas conjugadas contra infecciones bacterianas: fiebre tifoidea.

Capsular polysaccharides have been studied as possible vaccines against infectious diseases. However, they are capable to induce only short-run protection because of their T-independent properties and they would not be protective against infection in high-risk populations. The alternative to face this problem is to develop methods to join covalently the polysaccharide and proteins to both increase the immunogenicity of and to confer the property of T-dependence to this antigen. In order to obtain a conjugate vaccine against typhoid fever, in our laboratory we have tried to synthesize a conjugate immunogen between the Vi antigen and porins from Salmonella typhi.

Mitochondrial acetyl-CoA carboxylase. Time course of mobilization/activation in liver of refed rats.

Fasted (48 h) rats were killed at 0, 2, 4, 6, 8, 12, 16, 20 and 24 h after they were refed on a high-carbohydrate diet. An increase in the maximal activity and quantity of cystolic acetyl-CoA carboxylase was found in liver of refed rats after a lag time of about 8 h. The increased quantity of cytosolic enzyme was attributable primarily to mobilization of mitochondrial storage forms and not to substantial increase in the rate of synthesis of acetyl-CoA carboxylase.

Mitochondrial storage forms of acetyl CoA carboxylase: mobilization/activation accounts for increased activity of the enzyme in liver of genetically obese Zucker rats.

In earlier reports, we have described a previously unrecognized mechanism which regulates the activity of acetyl CoA carboxylase in rat liver by the control of its distribution between relatively inactive mitochondrial and active cytosolic forms. In this study, the activity, total quantity and the subcellular distribution of acetyl CoA carboxylase were determined in liver of fed and fasted (48 h) homozygous obese (fa/fa) zucker rats and homozygous lean (Fa/Fa) littermates. The results indicate that neither diet nor genetic obesity affected the total quantity of acetyl CoA carboxylase per unit weight of liver. Instead, increased activity of this enzyme in the liver of the Zucker rat was primarily due to a shift in the subcellular distribution away from relatively inactive mitochondrial forms toward active cytosolic forms. Thus, the Zucker rat appears to be yet another example illustrating the physiological importance of regulating the activity of acetyl CoA carboxylase by controlling its subcellular distribution.

Bovine milk-fat-globule membrane contains an enzymically inactive form of acetyl-CoA carboxylase.

Enzymically inactive acetyl-CoA carboxylase [acetyl-CoA:carbon-dioxide ligase (ADP-forming), EC 6.4.1.2] was found as a component of bovine milk-fat-globule membrane (MFGM). Acetyl-CoA carboxylase was present in MFGM at a higher concentration than in cytosolic or mitochondrial fractions of bovine mammary tissue, which makes it unlikely that its presence was due to simple contamination by these subcellular constituents.

Enzymatically inactive forms of acetyl-CoA carboxylase in rat liver mitochondria.

Biotinyl proteins were labelled by incubation of SDS-denatured preparations of subcellular fractions of rat liver with [14C]methylavidin before polyacrylamide-gel electrophoresis. Fluorographic analysis showed that mitochondria contained two forms of acetyl-CoA carboxylase [acetyl-CoA:carbon dioxide ligase (ADP-forming) EC 6.4.1.2], both of which were precipitated by antibody to the enzyme. When both forms were considered, almost three-quarters of the total liver acetyl-CoA carboxylase was found in the mitochondrial fraction of liver from fed rats while only 3.5% was associated with the microsomal fraction. The remainder was present in cytosol, either as the intact active enzyme or as a degradation product. The actual specific activity of the cytosolic enzyme was approx. 2 units/mg of acetyl-CoA carboxylase protein while that of the mitochondrial enzyme was about 20-fold lower, indicating that mitochondrial acetyl-CoA carboxylase was relatively inactive. Fractionation of mitochondria with digitonin showed that acetyl-CoA carboxylase was associated with the outer mitochondrial membrane. The available evidence suggests that mitochondrial acetyl-CoA carboxylase represents a reservoir of enzyme which can be released and activated under lipogenic conditions.

Acute alloxan diabetes alters the activity but not the total quantity of acetyl CoA carboxylase in rat liver.

Alloxan diabetes has repeatedly been shown to reduce lipogenesis in rat liver concomitant with decreased activity of acetyl CoA carboxylase. This and other observations led to the deduction that insulin is required for the synthesis of acetyl CoA carboxylase even though the actual amount of enzyme was not measured. We have developed methods to determine the quantity of acetyl CoA carboxylase in crude tissue extracts with which we have reexamined the role of insulin in regulating the amount of the enzyme in liver of acute (3-d) alloxan diabetic rats. The results show that although there was a decrease in the quantity of the active cytoplasmic form of acetyl CoA carboxylase in the liver of alloxan diabetic rats, there was a corresponding increase in the quantity of relatively inactive forms of the enzyme associated with mitochondria. Thus, the total amount of enzyme was minimally affected by the diabetic state. Instead, the results indicate that decreased acetyl CoA carboxylase activity in liver of the diabetic rats was attributable to a shift in the subcellular distribution of the enzyme from the active cytoplasmic to inactive mitochondrial forms. We have shown previously that subcellular distribution of the enzyme is dietary dependent. Results of this study implicate insulin in the mobilization and activation of mitochondrial acetyl CoA carboxylase.

Determination of the quantity of acetyl CoA carboxylase by [14C]methyl avidin binding.

Conditions are described under which monomeric [14C]methyl avidin binds to SDS-denatured biotin enzymes and remains bound through polyacrylamide gel electrophoresis. The location of radioactive proteins on the dried gel was determined by fluorography and their identity was established by subunit molecular weight. The relative quantity of bound radioactive avidin, stoichiometrically equivalent to the molar quantity of biotin protein, can be determined by scanning the fluorograph with a soft laser densitometer. To determine the absolute quantity of biotin protein, the radioactive areas of the dried gel were cut out, resolubilized, and assayed for radioactivity. Since the specific radioactivity of the [14C]methyl avidin was known, the quantity of avidin bound and therefore the quantity of biotin enzyme could be calculated. The method is illustrated by the analysis of purified acetyl CoA carboxylase and is applied to the analysis of biotin enzymes in isolated rat liver mitochondria.

Histometric components of aortic atherosclerosis which vary or remain constant among eight populations.

In the objective examination of aortic histology, each position observed in a sample can be classed as having or not having atheronecrosis, and each aorta can be scored as affected in a measured percentage of the sample. Positions not having necrosis can be measured for intimal thickness and the mean of those measurements is an estimate of the quantity of intimal fibroplasia. Both atheronecrosis and fibroplasia, measured in these ways, increase with age. Within age groups these two variables are correlated with each other. By multivariate statistical methods, independent variation of the two variables among populations was assessed. Populations compared were from Bogota, Durban (Bantu and Indian), Manila, Mexico, New Orleans (Negro and White) and Sao Paulo. Manila ranked highest on the quantity of fibroplasia and Durban Bantu lowest. Sao Paulo ranked highest on the extent of atheronecrosis and New Orleans Negro was lowest. The two variables assorted among the eight populations independently of each other to a large degree, suggesting that fibroplasia and atheronecrosis are at least in part subject to separate and independent causes. The numbers of smooth muscle cells and the extent of foam cell infiltration did not differ significantly between atherosclerosis related and basal cause of death groups. These cellularity measures were, with minor exceptions, not significantly different among populations, suggesting that they are inherent human characteristics largely unaffected by environmental circumstances.

Dietary dependent distribution of acetyl CoA carboxylase between cytoplasm and mitochondria of rat liver.

Biotinyl proteins in cytoplasm and mitochondria of rat liver were examined by fluorography and the quantity of acetyl CoA carboxylase was determined after sodium dodecyl sulfate-denatured proteins were incubated with [14C] methyl avidin and separated by polyacrylamide gel electrophoresis. Results show that one-half of the total acetyl CoA carboxylase in liver of fed rats was associated with mitochondria in a relatively inactive form. Fasting shifted the distribution of the enzyme toward the mitochondrial fraction and refeeding previously fasted rats shifted the distribution towards cytoplasm. Thus, acetyl CoA carboxylase can be added to the list of ambiquitous enzymes whose subcellular distribution varies with physiological conditions.

Spatial dispersion of spindle cells in the human thoracic aorta.

The intima of the human thoracic aorta thickens with age. Those portions of thickened intima that lack foci of atheronecrosis or foam cells are populated by smooth muscle cells, which are usually characterized in sections by spindle-shaped nuclei, and by occasional superficial monocytic macrophagic cells. Populations of these cells were censused by counting nuclear profiles at 40 anatomically defined sites in each of 200 aortas aged 10 to 69 years. The different depths within the intima between the lumenal surface and the media with minor exceptions did not significantly differ in cell numbers. The density of cells decreased with thickness at all ages and with age at all thicknesses after completion of growth and maturation. The total numbers of cells in 100-microns lengths of intima, assessed in longitudinal tissue sections, were virtually constant with aging after 20 to 29 years. The dispersion of cells over the intima in the lateral walls of the thoracic aorta followed a negative binomial distribution, which has an upward skewed unimodal form. Because of the sampling properties of this form of distribution the mean of eight to 12 measurements appears to be adequate to describe the spindle cell numbers in the nonnecrotic parts of an aorta.

Tratamiento conservador en tumores malignos del ovario. / Conservative treatment in malignant ovarian tumors

Se presentan 7 casos de pacientes portadores de tumores malignos del ovario, 4 de ellos asociados a gestacion, en los que se realizo tratamiento quirurgico inicialmente conservador obteniendose una sobrevida promedio de 7 anos. Se analizan los antecedentes bibliograficos existentes sobre tratamiento conservador en esta patologia y los parametros que deben ser tenidos en cuenta para efectuar dicha terapeutica, por ejemplo edad de la paciente, tipo y grado histologico, discutiendose las conductas seguidas en los casos presentados.Se concluye especificando las indicaciones actuales que le caben al tratamiento conservador en los tumores malignos del ovario y la necesidad de realizar hemiparticion y biopsia del ovario contralateral