Análisis de la validez convergente de un instrumento informatizado para evaluar la atención en deportistas: Rejilla 1.0 / Analysis of the convergent validity of a computerized instrument to evaluate attention in athletes: Reilla 1.0 / Análise da validade convergente de um instrumento informatizado para avaliar a atenção nos atletas: Rejilla 1.0

El objetivo de esta investigación fue analizar la validez convergente de un programa informatizado denominado Rejilla 1.0, que se construyó con el objetivo de evaluar la atención selectiva y para ser utilizado en contextos como el deporte. Un total de 99 participantes colaboraron en el estudio, con edades entre 19 y 36 años (M ± DT = 25.15 ± 3.56). Para valorar la validez convergente se realizaron análisis de correlaciones con otros instrumentos que se han utilizado tradicionalmente para evaluar la atención selectiva, como son el Test de Atención D2 y el Test Toulouse-Pieron. Los resultados encontrados pusieron de manifiesto correlaciones moderadas y bajas entre los instrumentos, lo cual sugiere relaciones entre ellos, aunque se deben tomar con cautela. Las medidas principales del Test de Atención D2 y el Test Toulouse-Pieron mostraron niveles moderados con los aciertos de Rejilla 1.0, pero las medidas secundarias manifestaron asociaciones bajas. Se sugiere que otras funciones cognitivas, además de atención selectiva, podrían participar en la realización de los ejercicios de Rejilla 1.0, lo cual se discute en el trabajo

The objective of this research was to analyze the convergent validity of a computerized program called Rejilla V.1, which was built with the aim of evaluating selective attention and to be used in contexts such as sport. A total of 99 participants collaborated on the study, aged between 19 and 36 years old (M ± SD = 25.15 ± 3.56). To assess convergent validity, correlation analyses were performed with other instruments that have traditionally been used to evaluate selective attention, such as the D2 Attention Test and the Toulouse-Pieron Test. The results found revealed moderate and low correlations between the instruments, suggesting relationships between them, although they should be taken with caution. The main measures of the D2 Test and the Toulouse-Pieron Test showed moderate levels with the hits of Rejilla V.1, but the secondary measures showed low associations. It is suggested that other cognitive functions, in addition to selective attention, could participate in the performance of Rejilla V.1 exercises, which is discussed at work

O objetivo desta pesquisa foi analisar a validade convergente de um programa informatizado chamado Rejilla 1.0, que foi construído com o objetivo de avaliar a atenção seletiva e ser utilizado em contextos como o esporte. Um total de 99 participantes colaboraram no estudo, variando na idade de 19 a 36 anos (M ± DP = 25.15 ± 3.56). Para avaliar a validade convergente, foram realizadas análises de correlação com outros instrumentos que tradicionalmente têm sido usados para avaliar a atenção seletiva, como o Teste de Atenção D2 e o Teste Toulouse-Pieron. Os resultados encontrados revelaram correlações moderadas e baixas entre os instrumentos, sugerindo relações entre eles, embora devam ser tomadas com cautela. As principais medidas do Teste D2 e do Teste toulouse-pieron mostraram níveis moderados com os hits da Rejilla 1.0, mas as medidas secundárias mostraram associações baixas. Sugere-se que outras funções cognitivas, além da atenção seletiva, possam participar do desempenho dos exercícios Rejilla 1.0, que é discutido no trabalho

Testing for genetic associations with the PAX gene family in a spina bifida population.

Neural tube defects (NTDs) are among the most common severely disabling birth defects in the United States, affecting approximately 1-2 of every 1,000 live births. The etiology of NTDs is multifactorial, involving the combined action of both genetic and environmental factors. A nonparametric linkage method, the transmission disequilibrium test (TDT), was utilized to determine if the genes in the PAX family play a role in the formation of NTDs. DNA from 459 spina bifida (SB) patients and their parents (430 mothers and 239 fathers, for a total population of 1,128 subjects) was tested for linkage and association utilizing polymorphic markers from within or very close to the PAX genes of interest. Significant findings were obtained for the following markers: marker locus D20S101 flanking the PAX1 gene (P = 0.019), marker locus D1S228 within the PAX7 gene (P = 0.011), and marker locus D2S110 within the PAX8 gene (P = 0.013). Even though our findings are only mildly significant, given the known expression patterns of the PAX genes in development and the availability of their sequences, we elected to follow up these results by testing these genes directly for mutations utilizing single-strand conformational analysis (SSCA) and direct sequencing. Multiple variations were detected in each of the PAX genes with significant TDT results; however, these variations were not passed from parent to child in phase with the positively transmitted allele. Therefore, it is unlikely that these variations contribute to susceptibility for SB, but rather are previously unreported polymorphisms.

Testing for genetic associations in a spina bifida population: analysis of the HOX gene family and human candidate gene regions implicated by mouse models of neural tube defects.

Neural tube defects (NTDs) are among the most common severely disabling birth defects in the United States, affecting approximately 1-2 of every 1,000 live births. The etiology of NTDs is multifactorial, involving the combined action of both genetic and environmental factors. HOX genes play a central role in establishing the initial body plan by providing positional information along the anterior-posterior body and limb axis and have been implicated in neural tube closure. There are many mouse models that exhibit both naturally occurring NTDs in various mouse strains as well as NTDs that have been created by "knocking out" various genes. A nonparametric linkage method, the transmission disequilibrium test (TDT), was utilized to test the HOX gene family and human equivalents of genes (when known) or the syntenic region in humans to those in mouse models which could play a role in the formation of NTDs. DNA from 459 spina bifida (SB) affected individuals and their parents was tested for linkage and association utilizing polymorphic markers from within or very close to the HOXA, HOXB, HOXC, and HOXD genes as well as from within the genes/gene regions of eight mouse models that exhibit NTDs. No significant findings were obtained for the tested markers.

Osteoarthritis in children associated with a mutation in the type II procollagen gene (COL2A1).

A single-base mutation resulting in an arginine-519-cysteine (R519C) mutation of type II procollagen (COL2A1) has been shown to result in precocious osteoarthritis with mild spinal chondrodysplasia without severe foreshortening (OMIM 604864). The nature of childhood disease among affected individuals has not been described. The recent presentation of four children with this mutation allows us to provide clinical correlation. This form of premature osteoarthritis may present in childhood and should be considered in the differential diagnosis of childhood arthropathy presenting in the context of a positive family history.

Current medication choices in juvenile rheumatoid arthritis II--update of a survey performed in 1993.

The documentation of treatments used for Juvenile Rheumatoid Arthritis (JRA) is important to allow for the evaluation of practice patterns for future outcome studies. A survey of nine pediatric rheumatologists was performed between September 1999 and February 2000. Each of the physicians prospectively recorded demographic and treatment information on consecutively sampled JRA patients (n=395). Pauciarticular onset JRA was present in 46%, polyarticular onset JRA in 35%, and systemic onset JRA in 19% of the children. Naproxen was the most frequently prescribed medication (55% of the patients), followed by methotrexate (MTX), which was used in 39% of the patients. Folic acid supplementation (1 mg/day) was provided to 69% of the patients treated with MTX. Etanercept was used in 11% of the children. Eleven percent of the patients received corticosteroids, and 13% of children on corticosteroids took calcium supplements. Uveitis was present in 8% and had a chronic course in 79% of those cases. Although systemic medications were used in 50% of the children with uveitis to control eye inflammation, severe damage to the eyes developed in 30% of them. Fourteen percent of the patients required gastroprotective medications. Compared with findings of a similar survey performed in 1993, there was no significant change in the frequency of use of naproxen, but nabumetone is now more often prescribed, and COX-2 inhibitors have been introduced in the therapy of JRA. Changes among second-line agents used for JRA have also occurred, although there was no change in the frequency of use of MTX or corticosteroids. JRA continues to be a treatment challenge for the practicing pediatric rheumatologist. Patients often show incomplete response to the currently available medications. Therefore, new therapeutic agents need to be evaluated for their use in JRA, and the treatment of JRA associated uveitis especially needs to be improved.

Treatment of sialorrhea with glycopyrrolate: A double-blind, dose-ranging study.

OBJECTIVE: To determine the safety and efficacy of glycopyrrolate in the treatment of developmentally disabled children with sialorrhea. DESIGN: Placebo-controlled, double-blind, crossover dose-ranging study. SETTING: Outpatient facilities in 2 pediatric hospitals. PATIENTS: Thirty-nine children with both developmental disabilities and excessive and bothersome sialorrhea. MAIN OUTCOME MEASURES: Parent and investigator evaluation of change in sialorrhea and adverse effects. RESULTS: Glycopyrrolate in doses of 0.10 mg/kg per dose is effective at controlling sialorrhea. Even at low doses, 20% of children may exhibit adverse effects severe enough to require discontinuation. CONCLUSIONS: Glycopyrrolate is effective in the control of excessive sialorrhea in children with developmental disabilities. Approximately 20% of children given glycopyrrolate may experience substantial adverse effects, enough to require discontinuation of medication. Arch Pediatr Adolesc Med. 2000;154:1214-1218.

Development of validated disease activity and damage indices for the juvenile idiopathic inflammatory myopathies. II. The Childhood Myositis Assessment Scale (CMAS): a quantitative tool for the evaluation of muscle function. The Juvenile Dermatomyositis Disease Activity Collaborative Study Group.

OBJECTIVE: To develop, validate, and determine the measurement characteristics of a quantitative tool for assessing the severity of muscle involvement in children with idiopathic inflammatory myopathies. METHODS: The Childhood Myositis Assessment Scale (CMAS) was developed from 2 existing observational functional assessment tools to assess muscle function in the areas of strength and endurance across a wide range of ability and ages. The 14 ordinal items included were chosen to assess primarily axial and proximal muscle groups and are ranked with standard performance and scoring methods. Following the development of the CMAS, a training video and written instructions were developed and reviewed by the physicians participating in this study. Subsequently, utilizing a randomized block design, 12 physicians independently scored 10 children (9 with dermatomyositis, 1 with polymyositis; ages 4-15 years) twice in one day (morning and afternoon) on the CMAS. A pediatric physical therapist performed quantitative manual muscle strength testing (MMT) twice on each child (morning and afternoon), including the neck, trunk, and proximal and distal extremity muscle groups. RESULTS: The CMAS has a potential range of 0-51, with higher scores indicating greater muscle strength and endurance. The observed mean for the 10 patients was 36.4 (median 44, SD 14.1, observed range 5-51). The total score for the CMAS correlated with the physician's global assessment (by visual analog scale) of disease activity, the MMT score, serum creatine kinase level, and the Juvenile Arthritis Functional Assessment Report score. The score on the CMAS was not correlated with patient age. Interrater reliability (Kendall's coefficient of concordance) ranged from 0.77 to 1.0 for individual items (all P < 0.001), and overall, it was 0.95 (P < 0.001). Intrarater reliability for the individual physicians was measured by correlation of the CMAS scores for each patient on 2 separate evaluations and ranged from 0.97 to 0.99, with an overall correlation for all physicians of 0.98 (all P < 0.001). CONCLUSION: The CMAS demonstrated an acceptable range of observed scores, excellent convergent validity, and excellent inter- and intrarater reliability. The CMAS is validated to quantitatively assess muscle function in the areas of strength and endurance in children with idiopathic inflammatory myopathies. It can be used in routine clinical care as well as therapeutic trials.

The effect of osteotomy on bowing and height in children with X-linked hypophosphatemia.

The clinical records of 40 patients with X-linked hypophosphatemia who were treated and followed for at least 36 months were examined retrospectively. The patients were divided into those treated with medication only (group A) and those treated with medication and surgery (group B). At follow-up, significant improvement in bowing angles was noted for group B patients compared with those treated medically. There was, however, no improvement in the height of children in group A, and there was a significant decrease in height among the children treated surgically (group B). Neither height nor bowing angles of femurs or tibias at presentation predicted the need for future osteotomies. However, children who eventually needed surgery were found to be obese at presentation significantly more frequently than children who did not require osteotomy.

Long term follow-up of children with mixed connective tissue disease.

Mixed connective tissue disease (MCTD) is characterized by features of more than one of the rheumatic disorders with antinuclear antibodies in a speckled pattern and with antibodies to nuclear ribonucleoprotein (nRNP). MCTD is uncommon in children and long-term follow-up studies in children are infrequently reported. A retrospective review of clinical experience at five pediatric rheumatology centers provided 11 patients who met the following inclusion criteria: (1) Kasukawa's criteria for MCTD1; (2) presentation younger than 18th birthday; (3) greater than five years of follow-up; (4) completion of data collection form. The widely varying outcomes of these 11 children with MCTD on long-term follow-up may lend doubt that this is a unique and distinctive rheumatologic disorder.

Medication choices in juvenile rheumatoid arthritis.

This survey was performed to review medication usage by pediatric rheumatologists in the care of patients with juvenile rheumatoid arthritis (JRA). Prospective data from 50 patients per physician with JRA were recorded by six pediatric rheumatologists in the Fall of 1993. Naproxen was used most frequently-in 48% of all patients. Next in order of frequency were methotrexate (39%), prednisone (15%), tolmetin (12%), indomethacin (11%) and folic acid (10%). Salicylates (acetylsalicylic acid, trisalicylate and salsalate) were used in 7%, and myochrysine was used in 2% of patients. Overall, nonsteroidal anti-inflammatory drugs were used in 93% of all patients, slower-acting antirheumatic drugs (SAARDs) were used in 54% and prednisone in 15%.Medication usage varied by disease type in predictable ways but also varied by physician in ways that could not be accounted for by population differences. Methotrexate was the most-often used of all SAARDs and supplanted myochrysine in JRA. Naproxen was the most often used NSAID in the treatment of JRA and had largely supplanted salicylates. With the arrival of practice guidelines, reasons for and impact of these changes (as well as the interesting variations between physicians) will need to be examined.

Myositis ossificans in a young child mimicking monoarthritis of the hip.

This report describes apparent acute monoarthritis of the left hip in a 6-year-old boy that was caused by myositis ossificans (MO). His initial presentation, with pain on movement of the left hip and an elevated sedimentation rate, led to consideration of an infection or inflammatory process affecting the left hip. Initial evaluation, including MRI and muscle biopsy, did not successfully lead to a diagnosis. MO became evident as an explanation for this child's problem on the 13th hospital day, 4 weeks after symptoms began, when typical radiologic manifestations became apparent on x-ray. This child's presentation, together with a review of recent literature, emphasizes that this condition a) is not as rare as previously described, b) occurs without a history of trauma, c) can be associated with acute phase reactants, and d) should be included in the differential diagnosis of apparent monoarthritis of the hip in children.

Interaction of neutrophils and endothelium in isolated coronary venules and arterioles.

This study reports measurements of porcine neutrophil dynamics in isolated microvessels. Porcine coronary venules and arterioles were isolated, cannulated, and perfused with fluorescently labeled neutrophils at a series of flow velocities. In venules (62.50 +/- 5.41 microns diam) under control conditions, rolling neutrophils were often observed at intraluminal flow velocities ranging from 600 to 6,000 microns/s, and the rolling fraction varied inversely as a function of flow velocity. There was no significant adherence under the control conditions at any of the various flow velocities. Pretreatment of the neutrophils with human recombinant complement 5a (C5a, 10(-8) M) increased adherence at low flow velocities but did not alter the rolling fraction. In contrast to venules, rolling neutrophils were not observed in arterioles (58.80 +/- 5.6 microns diam). Furthermore, neutrophils that were pretreated with C5a did not adhere to the arteriolar endothelium even at low flow velocities. We suggest that 1) isolated microvessels perfused with fluorescently labeled neutrophils are suitable models for the study of the interaction between neutrophils and the microvascular endothelium, 2) shear force plays an important role in neutrophil rolling in coronary venules but is not the major factor that prevents neutrophil rolling and adherence in arterioles, and 3) C5a causes neutrophil adherence in venules but not in arterioles, indicating that different mechanisms underlie the interaction between neutrophils and endothelium in venules and arterioles.

Severe insulin resistance and diabetes mellitus in mandibuloacral dysplasia.

Mandibuloacral dysplasia (MAD) is a syndrome with onset in midchildhood. The predominant characteristics of MAD include flexion contractures; mandibular hypoplasia; loss of body fat; atrophic, speckled skin; and progressive osteolysis of the clavicles. We studied three males with MAD. Each had lipodystrophy of the extremities, with sparing of the face and neck. All had moderate hyperlipidemia. In response to oral glucose, each had a diabetic response, with peak insulin levels between 2870 and 22,960 pmol/L. Insulin-stimulated glucose disposal was determined in two patients with MAD. At an insulin infusion rate of 120 mU/m2 per minute, glucose disposal was less than 25% of that measured at similar levels of insulinemia in nondiabetic control subjects, indicating marked insulin resistance in patients with MAD. The insulin resistance occurred without obesity, excessive levels of counterregulatory hormones, or anti-insulin-receptor antibodies. We suggest that MAD is a previously undescribed form of lipodystrophic insulin-resistant diabetes mellitus.

Treatment of aniline poisoning with exchange transfusion.

A case of aniline poisoning with methemoglobinemia unresponsive to methylene blue is described. Exchange transfusion proved successful. A rationale for the failure of methylene blue under these circumstances is described.

Down's syndrome.

We discuss the ethical, psychosocial, economic, and medical dimensions of the treatment and management of a child with Down's syndrome and a congenital heart defect.