Evaluating acceptance and user experience of a guideline-based clinical decision support system execution platform.

This study aims to determine what the initial disposition of physicians towards the use of Clinical Decision Support Systems (CDSS) based on Computerised Clinical Guidelines and Protocols (CCGP) is; and whether their prolonged utilisation has a positive effect on their intention to adopt them in the future. For a period of 3 months, 8 volunteer paediatricians monitored each up to 10 asthmatic patients using two CCGPs deployed in the-GuidesMed CDSS. A Technology Acceptance Model (TAM) questionnaire was supplied to them before and after using the system. Results from both questionnaires are analysed searching for significant improvements in opinion between them. An additional survey was performed to analyse the usability of the system. It was found that initial disposition of physicians towards e-GuidesMed is good. Improvement between the pre and post iterations of the TAM questionnaire has been found to be statistically significant. Nonetheless, slightly lower values in the Compatibility and Habit variables show that participants perceive possible difficulties to integrate e-GuidesMed into their daily routine. The variable Facilitators shows the highest correlation with the Intention to Use. Usability of the system has also been rated very high and, in this regard, no fundamental flaw has been detected. Initial views towards e-GuidesMed are positive, and become reinforced after continued utilisation of the system. In order to achieve an effective implementation, it becomes essential to facilitate conditions to integrate the system into the physician's daily routine.

Persistence of essential fatty acid deficiency in cystic fibrosis despite nutritional therapy.

To study the evolution of plasma fatty acid composition of patients with cystic fibrosis (CF) in relation to nutritional status, pancreatic function, and development of CF-related liver disease (CFRLD) and diabetes mellitus, 24 CF pediatric patients with stable pulmonary disease were studied before and after an approximate period of 8 y. Nutritional status, pulmonary function, pancreatic function, and presence of CFRLD or diabetes mellitus were recorded. Results were compared with data obtained in 83 healthy children. Patients with CF have significantly lower linoleic acid (LA), docosahexaenoic acid (DHA), lignoceric acid, and LA x DHA product and higher oleic acid, mead acid, dihomo-gamma-linoleic acid, and docosapentaenoic acid (DPA). Comparison of samples taken at first and second studies revealed a significant decrease in LA levels and lignoceric acid associated with a significant increase in dihomo-gamma-linoleic acid levels. Patients with CFRLD showed significantly higher mead acid/arachidonic acid ratio and lower total omega6 polyunsaturated fatty acids content. There was no relation of plasma fatty acids composition with pancreatic function, pulmonary function, or diabetes mellitus. Follow-up of patients with CF shows that essential fatty acids deficiency, particularly in LA and DHA content, persisted unmodified along time despite an adequate nutritional therapy. Future studies after supplementation with omega3 polyunsaturated fatty acids should be undertaken.

Determination of creatine and guanidinoacetate by GC-MS: study of their stability in urine at different temperatures.

OBJECTIVES: Evaluation of a GC-MS method using N-tert-butyldimethylsilyl-N-methyltrifluoroacetamide (MTBSTFA) as the silylating agent for GC-MS. Study of the stability of creatine and guanidinoacetate in urine. DESIGN AND METHODS: 22 urines were kept at RT, 4 degrees C and -30 degrees C for 15 days. RESULTS: MTBSTFA produces a single chromatographic peak in contrast with other derivatizing agents. Creatine concentration increases at room temperature (326% on average), and at 4 degrees C (75%). However, detection decreases after freezing (-37%). Guanidinoacetate is stable, but decreases after freezing (-37%). Sonication before analysis is crucial to obtain repetitive results. CONCLUSIONS: A modified GC-MS method has been validated and the conditions for preservation of the urine have been established.

Fatty acid deficiency profile in children with food allergy managed with elimination diets.

AIM: To evaluate plasma fatty acid (FA) composition of children with food allergy undergoing elimination diets that avoided the offending antigens. METHODS: Twenty-five children (14 male, 11 female) aged 3.8 +/- 1.6 years (range 2-7 years) affected of multiple food allergy and managed with elimination diets participated in a cross-sectional study. Results of plasma fatty acids were compared with data obtained in 61 healthy children. RESULTS: The patients had significantly lower values for plasma content in total polyunsaturated fatty acids, omega3 polyunsaturated fatty acids and long-chain omega3 polyunsaturated fatty acids (p < 0.001) and particularly, in eicosapentaenoic acid (EPA) (20:5omega3) and docosahexaenoic acid (DHA) (22:6omega3) (p < 0.001). Moreover, all established indices (essential fatty acid [EFA] index ((omega3 +omega6)/(omega7 +omega9)) (p < 0.001) and sufficiency of docosahexaenoic acid index (C22:6omega3/C22:5omega6) (p < 0.001)) confirmed the presence of EFA deficiency. CONCLUSION: Children with food allergy managed with restricted intake of foods such as milk, egg, fish and vegetables are at risk of developing a deficiency in EFA and particularly in omega3 long-chain polyunsaturated fatty acids, which are especially necessary for adequate growth, neurological development and cardiovascular health.

The arginine-creatine pathway is disturbed in children and adolescents with renal transplants.

Cardiovascular disease is an important cause of morbidity in recipients of renal transplants. The aim of the present study was to analyze the status of the arginine-creatine pathway in such patients, given the relationship between the arginine metabolism and both renal function and the methionine-homocysteine cycle. Twenty-nine children and adolescents (median age 13, range 6-18 years), who had received a renal allograft 14.5-82.0 months before, were recruited for the study. On immunosuppressive therapy, all patients evidenced an adequate level of renal function. Plasma concentrations of homocysteine and glycine were significantly higher, whereas urinary excretions of guanidinoacetate and creatine were significantly lower than controls. Urinary excretions of guanidinoacetate and creatine correlated positively with creatinine clearance. Urinary excretion of creatine was negatively correlated with plasma concentration of homocysteine. The demonstration of disturbances in the arginine-creatine pathway in patients with well-functioning renal transplants and in absence of chronic renal failure represents a novel finding. We speculate that the low urinary excretion of guanidinoacetate and creatine is probably related to the nephrotoxic effect of immunosuppressive therapy and to defective methylation associated with the presence of hyperhomocysteinemia.

Stability of urinary glycosaminoglycans in patients with mucopolysaccharidoses.

OBJECTIVE: The aim of this work is to investigate the degradation of urinary glycosaminoglycans (GAGs) at different storage temperatures, in order to identify whether frozen transportation to reference laboratories is necessary. An improved method for the determination of total GAGs with 1,9-dimethylmethylene blue (DMB) is presented. DESIGN AND METHODS: Urine samples of 37 patients suffering from mucopolysaccharidoses (MPS) were analyzed in this study (13 Hunter, 6 Maroteaux-Lamy, 6 Morquio, 6 Sanfilippo, 5 Hurler-Scheie, and 1 Sly). Stability was assayed at room temperature, 5 degrees C and -30 degrees C, and analyses were repeated for at least 15 days. Spectrophotometric quantitation of GAGs with DMB was used for all determinations, using a variable wavelength for quantitation. RESULTS: The concentration of urinary GAGs was stable for 10 days at room temperature, but it was found to be stable for more than 15 days at 5 degrees C and -30 degrees C. CONCLUSIONS: The stability of GAGs allows urine samples to be sent for quantitation at a clinical laboratory without the need to freeze samples, as this would not affect results. This issue is important for the rapid detection of MPS at hospitals or primary health care centres, where GAGs determination is not performed.

Arachidonic acid content in adipose tissue is associated with insulin resistance in healthy children.

BACKGROUND: The fatty acid composition of membrane structural lipids, which is partly dependent on dietary intake, is associated with insulin action. AIM: To examine the association between fatty acid composition of adipose tissue and skeletal muscle phospholipids with insulin resistance markers in a healthy pediatric population. METHODS: Using a cross-sectional design, we studied 83 healthy children divided into 3 groups, ages 2 to 5, 6 to 10 and more than 10 years. MEASUREMENTS: Fatty acid composition of adipose tissue triacylglycerols and skeletal muscle phospholipids, plasma lipid profile and fasting plasma levels of glucose and insulin were measured. RESULTS: There was a linear increase of insulinemia, glycemia and homeostasis adipose tissue model assessment (HOMA) index throughout the pediatric age range. Linoleic acid proportion in skeletal muscle and arachidonic acid proportion in adipose tissue also increased significantly with age. An age-independent positive correlation between insulinemia or HOMA index and arachidonic acid content in adipose tissue triacylglycerols (r = 0.47, P < 0.001) was found. An age-dependent negative correlation was present between insulinemia or HOMA index and oleic acid content in skeletal muscle phospholipids (r = -0.30, P = 0.03 and r = -0.28, P < 0.04, respectively). Trans fatty acids content did not correlate with any marker of insulin resistance. CONCLUSION: Healthy children present a prepubertal increase of insulin resistance, which is significantly correlated with arachidonic acid content in adipose tissue.

Essential fatty acid deficiency profile in patients with nephrotic-range proteinuria.

Plasma free fatty acids are bound to albumin, filtered through the glomeruli, and reabsorbed at the proximal nephron. The aim of the present investigation was to determine if urinary loss of fatty acids results in essential fatty acid (EFA) deficiency in patients with nephrotic-range proteinuria. We studied 12 patients aged 9 months to 23 years (eight male, four female) four suffering from congenital nephrotic syndrome (NS) and eight from different renal diseases. Six patients were studied postrenal transplantation. Proteinuria ranged between 41 and 829 mg/m2/h. Results were compared with data obtained in 83 healthy children. The patients had significantly lower values for plasma arachidonic acid content and EFA index (omega3 + omega6/omega7 + omega9). Deficiency in polyunsaturated fatty acids (PUFA) was especially manifest in infants with congenital NS. Plasma content of arachidonic and docosahexaenoic acids related negatively with the degree of proteinuria. In the lineal regression model, the degree of proteinuria explained 60% of the variability of plasma values of those fatty acids. We conclude that plasma fatty acid status should be regularly monitored in patients with nephrotic-range proteinuria, especially in young infants with congenital NS, who represent a population at special risk with regard to neurological development.

Fatty acid composition of skeletal muscle and adipose tissue in Spanish infants and children.

There is a relationship between the fatty acid profile in skeletal muscle phospholipids and peripheral resistance to insulin in adults, but similar data have not been reported in infancy and childhood. The objective of this study was to investigate the fatty acid composition of skeletal muscle and adipose tissue across the paediatric age range. The fatty acid profile of skeletal muscle phospholipids and adipose tissue triacylglycerols was analysed in ninety-three healthy Spanish infants and children distributed into four groups: group 1 (0 to <2 years, n 10); group 2 (2 to <5 years, n 41); group 3 (5 to <10 years, n 24); group 4 (10 to 15 years, n 18). In skeletal muscle phospholipids, oleic acid (18: 1n-9cis) content decreased significantly whereas that of linoleic (18: 2n-6) acid increased significantly with age (P for trend <0.01). In adipose tissue, the contents of triacylglycerol and linoleic acid increased significantly across the paediatric age range (P for trend <0.01), whereas dihomo-gamma-linolenic (20: 3n-6) and arachidonic (20: 4n-6) showed significant differences between groups. The variations in fatty acid composition observed with age indicated an imbalance in dietary n-3/n-6 long-chain PUFA.

Abnormalities in plasma fatty acid composition in human immunodeficiency virus-infected children treated with protease inhibitors.

AIM: To study plasma fatty acid composition in human immunodeficiency virus-infected children treated with protease inhibitors and its relation with other components of the metabolic syndrome observed after this therapy. DESIGN: Cross-sectional study from collected clinical database. SUBJECTS: 17 children with HIV infection treated with protease inhibitors. Nine patients received ritonavir (20-30 mg/kg/d) and the remaining eight received nelfinavir (60-90 mg/kg/d). Duration of protease inhibitors treatment was 711+/-208 d. As controls, we used 112 matched blood samples from apparently healthy children admitted for minor surgical procedures. METHODS: Plasma fatty acids were determined using a Hewlett Packard GC 5890 gas chromatograph. RESULTS: Plasma levels of cholesterol and triglycerides and insulin-like growth factor 1 (IGF-1) tended to be high in protease inhibitor-treated patients. Plasma content of omega6 long-chain polyunsaturated fatty acids and, in particular, of the highly unsaturated 22ratio4omega6 and 22ratio5omega6, was significantly increased. Also, infected children had increased Delta6 and Delta4 desaturase activities and decreased Delta5 desaturase activity. Significant correlations were present between plasma IGF-1 level and plasma triglycerides, plasminogen activator inhibitor-1 activity and Delta6 desaturase activity. CONCLUSION: HIV-infected, protease inhibitor-treated children exhibit a metabolic syndrome which is associated with significant changes in plasma fatty acid composition. These changes are similar to those observed in situations of insulin resistance and are linked to variations in plasma IGF-1 concentration.

Influence of diet on atherogenic risk in children with renal transplants.

Cardiovascular disease is one of the main causes of morbidity and mortality in recipients of renal transplants. Although the risk for cardiovascular disease is in part genetically determined, it may also be influenced by diet. The aim of the present study was to analyze the cross-sectional association of dietary intake of nutrients with biochemical markers of atherogenic risk. The influence of diet on the plasma profile of fatty acids was specifically investigated. Twenty-nine children and adolescents (mean age 14 years, range 6-18 years) with stable renal transplants and on a normal diet recorded their food intake for a period of 3 days. The mean calorie intake was 40.6 kcal/kg per day (protein provided 16% of total calories, carbohydrates 45%, and fat 39%). Plasma levels of total cholesterol and low-density lipoprotein-cholesterol were significantly and positively related to intake of monounsaturated fatty acids ( r=0.66, P =0.007 and r =0.62, P =0.02, respectively) and to plasma levels of elaidic acid, a trans fatty acid ( r=0.43, P =0.02 and r =0.54, P =0.01, respectively). Insulin resistance, estimated from values of plasma glucose ( r=0.70, P =0.03), plasma insulin ( r=0.59, P =0.02), and HOMA index ( r=0.62, P =0.01), was also directly related to the intake of monounsaturated fatty acids. Plasma plasminogen activator inhibitor-1 activity correlated positively with total fat intake ( r=0.59, P =0.04). Plasma levels of homocysteine were negatively related to the intake of carbohydrates ( r=-0.62, P =0.02). We conclude that reasonable dietary recommendations to minimize the atherogenic risk in children with stable renal transplants should include a protein intake adjusted to the requirements for age, a large intake of carbohydrates leading to a low glycemic load, and a fat intake of less than 30% of the total calorie intake. The amount of monounsaturated and trans fatty acids in the diet should be especially limited. A sufficient intake of polyunsaturated fatty acids, with an adequate ratio between omega 6 and omega 3 components, should also be provided.