Quality of life in mucopolysaccharidoses: construction of a specific measure using the focus group technique.

OBJECTIVE: To describe the perceptions of patients, their caregivers, and their healthcare providers to the development of a new specific instrument for assessment of the quality of life (QoL) in patients with mucopolysaccharidoses (MPS) using a qualitative focus group (FG) design. FGs were held in two Brazilian states (Rio Grande do Sul and Rio de Janeiro). RESULTS: Three versions of the new instrument were developed, each for a different age group: children (age 8-12 years), adolescents (age 13-17), and adults (age ≥ 18). The FGs mostly confirmed the relevance of items. All FGs unanimously agreed on the facets: School, Happiness, Life Prospects, Religiosity, Pain, Continuity of Treatment, Trust in Treatment, Relationship with Family, Relationship with Healthcare Providers, Acceptance, and Meaning of Life. The overall concept of QoL (as proposed by the WHO-World Health Organization) and its facets apply to this patient population. However, other specific facets-particularly concerning clinical manifestations and the reality of the disease-were suggested, confirming the need for the development of a specific QoL instrument for MPS.

Oxidative status of the myocardium in response to different intensities of physical training.

The intensity of exercise determines the metabolic pathway and the energetic substrate that is spent. Our study sought to identify the effects of different intensities of swimming on myocardial oxidative status and the blood lipid profile. Eighty Wistar rats (male and female) submitted to different intensities of a swimming regimen (low, LS; moderate, MS; or high, HS) for 16 weeks. Samples of blood and myocardium from the left ventricle were collected to determine lipid profiles and oxidative status. Reactive oxygen species (ROS) and antioxidant capacity against peroxyl radicals (ACAP), lipid profiles and lipid peroxidation was analyzed. ROS levels and ACAP were higher in male rats than in female rats overall (p<0.05). However, ACAP in the myocardium was significantly elevated in LS female rats compared to the MS and HS female rats, which had a significantly lower ACAP compared to all other groups. LS and MS training in both sexes and HS training (in females) led to significant decreases in the heart's lipid peroxidation. Amelioration of the lipid profile and reduction in oxidative damage contributed to a physiological state that benefits cardiovascular function in exercised animals. The results show that low and moderate intensity exercise promotes beneficial adaptations.

Effects of cryotherapy combined with therapeutic ultrasound on oxidative stress and tissue damage after musculoskeletal contusion in rats.

OBJECTIVE: To investigate the combined effects of cryotherapy and pulsed ultrasound therapy (PUT) on oxidative stress parameters, tissue damage markers and systemic inflammation after musculoskeletal injury. DESIGN: Experimental animal study. SETTING: Research laboratory. PARTICIPANTS: Seventy male Wistar rats were divided into five groups: control, lesion, cryotherapy, PUT, and cryotherapy+PUT. INTERVENTIONS: The gastrocnemius muscle was injured by mechanical crushing. Cryotherapy was applied immediately after injury (immersion in water at 10°C for 20minutes). PUT was commenced 24hours after injury (1MHz, 0.4W/cm2SPTA, 20% duty cycle, 5minutes). All animals were treated every 8hours for 3 days. MAIN OUTCOME MEASURES: Oxidative stress in muscle was evaluated by concentration of reactive oxygen species (ROS), lipid peroxidation (LPO), anti-oxidant capacity against peroxyl radicals (ACAP) and catalase. Plasma levels of creatine kinase (CK), lactate dehydrogenase (LDH) and C-reactive protein (CRP) were assessed. RESULTS: When applied individually, cryotherapy and PUT reduced CK, LDH, CRP and LPO caused by muscle damage. Cryotherapy+PUT in combination maintained the previous results, caused a reduction in ROS [P=0.005, mean difference -0.9×10-8 relative area, 95% confidence interval (CI) -0.2 to -1.9], and increased ACAP {P=0.007, mean difference 0.34 1/[relative area with/without 2,2-azobis(2-methylpropionamidine)dihydrochloride], 95% CI 0.07 to 0.61} and catalase (P=0.002, mean difference 0.41units/mg protein, 95% CI 0.09 to 0.73) compared with the lesion group. CONCLUSIONS: Cryotherapy+PUT in combination reduced oxidative stress in muscle, contributing to a reduction in adjacent damage and tissue repair.

Effect of incorporation of antioxidants on the chemical, rheological, and sensory properties of probiotic petit suisse cheese.

This work investigated the effect of the addition of different antioxidants (ascorbic acid, glucose oxidase, cysteine, and jabuticaba extract) on the rheological and sensorial properties of the probiotic petit suisse cheese. Absence of influence of the antioxidants at the physico-chemical characteristics of the petit suisse cheese was observed. Overall, the petit suisse cheeses presented weak gel characteristics and behaved as pseudoplastic material, except for control. All treatments exhibited a thixotropic non-Newtonian behavior; however, higher hysteresis area was obtained for control sample, which indicates that antioxidants incorporated to petit suisse had a protective effect on the typical thixotropic behavior of the Quark gel. The commercial sample presented higher scores for all aspects by consumers, whereas the probiotic petit suisse samples presented opposite behavior. Projective mapping was able to generate a vocabulary where the sample containing jabuticaba skin extract obtained by supercritical extraction was characterized by the panelists as presenting grape flavor and purple color.

Oocyte quality of tambaqui (Colossoma macropomum) during the reproductive season.

The study aimed to analyze the Colossoma macropomum reproductive behavior and quality of the female gametes throughout the reproductive season. The experiment was carried out in Pimenta Bueno - Rondônia State (Northern Brazil) during the reproductive season (2010-2011) using 36 females. Each sampling was performed on a 15 ± 5 days interval. Female gametes were collected by stripping and the following analyses were performed: weight of oocytes released (g); productivity index, fertilization and hatching rate. During the sampling period was verified effect (p < 0.05) of collecting time into the season for oocytes weight, productivity index and fertilization rate. Although the period 3 (December) did not differ significantly from other periods, it showed better parameters for the quality of C. macropomum oocytes.

Development of probiotic dairy beverages: rheological properties and application of mathematical models in sensory evaluation.

Strawberry-flavored probiotic dairy beverages (2% vol/vol Lactobacillus acidophilus) were produced using 0, 20, 35, 50, 65, and 80% (vol/vol) whey in their formulations. Mathematical models (survival analysis, minimal significant difference, and mean global acceptance) were used to identify the optimal (sensorially) whey concentration in probiotic beverages. Fifty-five consumers evaluated acceptance of the beverages using hybrid 9-point hedonic scales. In addition, Lb. acidophilus were enumerated and pH was determined. Rheological behavior is an important characteristic for the processing and sensory acceptance of dairy beverages, varying with the presence of additives, fermentation process (time, bacterial strain), and whey concentrations used. All beverages presented minimal counts of 8 log cfu/mL of Lb. acidophilus, and pH ranged from 4.09 to 4.14. Increasing the whey content increased the fragility of the gel structure, probably because of the replacement of casein by whey proteins, once the concentrations of other ingredients in formulation were fixed. Whey content had a significant effect on acceptance of the probiotic dairy beverages; beverages with whey contents greater than 65% resulted in lower acceptance by consumers. The model of mean global acceptance presented 2 solutions with high sensory scores: beverages with 12 and 65% whey, the latter being of interest because it allows greater use of the whey by-product. The Weibull distribution presented a prediction of whey concentration of 49%, with higher sensory acceptance. The methodologies used in this research were shown to be useful in determining the constituents of food formulations, especially for whey-based probiotic beverages.

Molecular mobility of nematic E7 confined to molecular sieves with a low filling degree.

The nematic liquid crystalline mixture E7 was confined with similar filling degrees to molecular sieves with constant composition but different pore diameters (from 2.8 to 6.8 nm). Fourier transform infrared analysis proved that the E7 molecules interact via the cyanogroup with the pore walls of the molecular sieves. The molecular dynamics of the system was investigated by broadband dielectric spectroscopy (10(-2)-10(9) Hz) covering a wide temperature range of approximately 200 K from temperatures well above the isotropic-nematic transition down to the glass transition of bulk E7. A variety of relaxation processes is observed including two modes that are located close to the bulk behavior in its temperature dependence. For all confined samples, two relaxation processes, at frequencies lower than the processes observed for the bulk, were detected. At lower temperatures, their relaxation rates have different temperature dependencies whereas at higher temperatures, they seem to collapse into one chart. The temperature dependence of the slowest process (S-process) obeys the Vogel-Fulcher-Tammann law indicating a glassy dynamics of the E7 molecules anchored to the pore surface. The pore size dependence of both the Vogel temperature and fragility revealed a steplike transition around 4 nm pore size, which indicates a transition from a strong to a fragile behavior. The process with a relaxation rate in between the bulklike and the S-process (I-process) shows no dependence on the pore size. The agreement of the I-process with the behavior of a 5CB surface layer adsorbed on nonporous silica leads to the assignment of E7 molecules anchored at the outer surface of the microcrystals of the molecular sieves.

[The endocrine pancreas]. / Pâncreas endócrino.

The islets of Langerhans provide energy storage and disposal, and protection from plasma glucose excursions, especially hypoglycemia. Insulin-dependent diabetes mellitus (IDDM) results from autoimmune beta-cell damage. Prevention of IDDM has already been achieved in animal investigation and some centers are now screening and treating individuals at high risk for developing IDDM. Immunosuppressive drugs can induce transient remission of recent-onset IDDM. Intensive insulin treatment of IDDM delays the onset and slows the progression of long-term complications. Non-insulin dependent diabetes mellitus (NIDDM) is the result of beta-cell malfunction and is strongly associated with X syndrome. Diet and exercise are of undoubted importance in NIDDM prevention and treatment. Functional endocrine tumors of the pancreas (FET) are rare hormone and peptide-secreting neoplasms. These peptides may or may not occur naturally in the islets. FETs often occur with multiple endocrine neoplasia 1 (MEN 1) so that MEN-1 screening should always be performed, and extended to family members whenever diagnosed. Drugs--alcohol, insulin and sulfonilureas--are the main cause of hypoglycemia. Insulinoma is the main cause of post-absorptive organic hypoglycemia. Non islet-cell tumors seldom cause hypoglycemia. Insulinoma often is a solitary tumor, but it may be multicentric and may coexist with cell hyperplasia and nesidioblastosis. Symptoms of neuroglycopenia may be mistaken for neuropsychiatric disease. The diagnosis is based on confirmation of post absorptive hypoglycemia and hyperinsulinism. Gastrinoma causes Zollinger-Ellison syndrome (ZES) which is characterized by fulminating peptic ulcer disease. The tumor is often malignant, and it may be multicentric and may occur with cell hyperplasia and nesidioblastosis.(ABSTRACT TRUNCATED AT 250 WORDS)

[Pure autonomic failure]. / Falência autonómica pura.

We present the case of a 64-year-old woman who, in the past 5 years, complained of constipation/diarrhea, hyposudoresis, xerostomia and xerophthalmia, dysuria and orthostatic hypotension. Cardiovascular reflexes analysis revealed sympathetic and parasympathetic failure. Norepinephrine was markedly reduced, both lying and after tilt. Norepinephrine infusion determined a significant rise in blood pressure, allowing the diagnosis of denervation hypersensitivity. The diagnosis of pure autonomic failure was made. Therapy with 9 alpha fludrocortisone and metoclopramide was initiated with marked and sustained symptomatic effect.