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Cardiovascular pathology, including aortic root dilation at the level of sinus of Valsalva (SV), is one of the major causes of morbidity in paediatric patients with Marfan syndrome (MFS). β-Blocker (BB) is well established to slow aortic dilation in MFS. Less is known about the effectiveness of angiotensin II receptor blocker (ARB) on aortic dilation in paediatric patients with MFS.215 patients with MFS (9.01 ± 5.7 years) were subject to a standardised diagnostic programme. Aortic root dilation was evaluated and followed up by echocardiography. In 48 cases, BB and ARB effects on aortic root dilation were evaluated. Effect of treatment was measured by comparison of z scores of SV before and after treatment initiation.Treatment by ARB and BB leads to significant reduction of SV dilation (p0.05). The deviation of SV enlargement from normal as expressed by the rate of change in z scores was significantly reduced by a mean difference of -0.56 ± 0.71 z scores (p0.001) under ARB therapy and by a mean difference of -0.35 ± 0.68 z scores (p0.05) under BB therapy. The prophylactic effect of ARB and BB on aortic root dilation is similar in both groups (p0.05).Both concepts lead to a significant reduction of SV dilation. The effect of ARB and BB is similar. This is the first study concerning the comparison of ARB and BB in previously untreated paediatric patients with MFS. The results of the study show that both treatment strategies are beneficial in paediatric and adolescent patients.

Item does not contain fulltext AIM: Due to age-dependent manifestations, diagnosis of Marfan syndrome (MFS) in children and adolescents is sophisticated. Although revised Ghent criteria is a major step forward, its utility in children is still restricted due to expensive and technically advanced diagnostics. As early diagnosis submits long-term benefits concerning prognosis, the need of an appropriate diagnostic tool for risk stratification of suspected paediatric patients with Marfan is justified. METHODS AND RESULTS: Sixty paediatric patients with Marfan were subject to a standardized diagnostic programme. All clinical symptoms of the revised Ghent nosology were analysed concerning age at first clinical manifestation, prevalence and likelihood ratio for MFS. Symptoms with early onset, high prevalence and high positive likelihood ratio were identified and combined for a risk score called Kid-Short Marfan Score (Kid-SMS). Three risk categories for suspicion of Marfan syndrome were developed. Finally, the Kid-SMS was operated in 130 paediatric patients with suspected MFS. Kid-SMS identified significantly more suspected patients with Marfan compared with Ghent nosology, revised Ghent and genetics alone without oversensitivity. CONCLUSION: Whereas diagnosis of MFS in childhood is sophisticated, Kid-SMS is a useful tool for risk stratification of suspected paediatric patients with Marfan by easy executable diagnostics, especially for paediatricians and paediatric cardiologists.

Introduction. Anemia is prevalent in adult heart failure patients and appears to be an independent risk factor for morbidity and mortality. The purpose of this work is to determine the prevalence of anemia in children with heart failure from dilated cardiomyopathy (DCM) and to evaluate its influence on morbidity and mortality.Methods. A homogenous group of 58 children with congestive heart failure from DCM was evaluated for heart failure symptoms, appearance of anemia, hospitalization, age of first clinical appearance, necessity of transfusion, and death during medical attendance. Anemic and nonanemic patients were analyzed for differences in age distribution, morbidity, and mortality.Results. Anemia was present in 64% of DCM patients. Hospitalization secondary to heart failure was significantly elevated in heart failure patients with anemia (mean versus days per year, ). However, mortality was not elevated. Significant relations of age and prevalence of anemia or age and severity of anemia did not appear.Conclusion. Anemia is prevalent in pediatric patients with congestive heart failure from DCM and appears in all age classes. Hospitalization as a surrogate of morbidity is elevated in heart failure patients developing anemia, but mortality risk did not increase.

The constituents of attractiveness differ across the sexes. Many relevant traits are dimorphic, suggesting that they are the product of intersexual selection. However, direction of causality is generally difficult to determine, as aesthetic criteria can as readily result from, as cause, dimorphism. Women have proportionately smaller feet than men. Prior work on the role of foot size in attractiveness suggests an asymmetry across the sexes, as small feet enhance female appearance, yet average, rather than large, feet are preferred on men. Previous investigations employed crude stimuli and limited samples. Here, we report on multiple cross-cultural studies designed to overcome these limitations. With the exception of one rural society, we find that small foot size is preferred when judging women, yet no equivalent preference applies to men. Similarly, consonant with the thesis that a preference for youth underlies intersexual selection acting on women, we document an inverse relationship between foot size and perceived age. Examination of preferences regarding, and inferences from, feet viewed in isolation suggests different roles for proportionality and absolute size in judgments of female and male bodies. Although the majority of these results bolster the conclusion that pedal dimorphism is the product of intersexual selection, the picture is complicated by the reversal of the usual preference for small female feet found in one rural society. While possibly explicable in terms of greater emphasis on female economic productivity relative to beauty, the latter finding underscores the importance of employing diverse samples when exploring postulated evolved aesthetic preferences.

We estimate the impact of regulatory heterogeneity on agri-food trade using a gravity analysis that relies on detailed data on non-tariff measures (NTMs) collected by the NTM-Impact project. The data cover a broad range of import requirements for agricultural and food products for the EU and nine of its major trade partners. We find that trade is significantly reduced when importing countries have stricter maximum residue limits (MRLs) for plant products than exporting countries. For most other measures, due to their qualitative nature, we were unable to infer whether the importer has stricter standards relative to the exporter, and we do not find a robust relationship between these measures and trade. Our findings suggest that, at least for some import standards, harmonising regulations will increase trade. We also conclude that tariff reductions remain an effective means to increase trade even when NTMs abound.

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Drug-induced dyskinesia is a common phenomenon in Parkinson's disease (PD) and is often socially as well as physically disabling for patients. The Movement Disorders Society commissioned a task force to assess available clinical rating scales, critique their clinimetric properties, and make recommendations regarding their clinical utility. A task force composed six clinical researchers who systematically searched the literature for scales measuring dyskinesia in PD, evaluated the scales' previous use, performance parameters, and quality of validation data (if available). A scale was designated "Recommended" if the scale has been used in clinical studies beyond the group that developed it, has been specifically used in PD reports, and if clinimetric studies have established that it is a valid, reliable, and sensitive. "Suggested" scales met two of the above criteria and those meeting one were "Listed." Based on the systematic review, eight rating scales for dyskinesia that have either been validated or used in PD were identified. These were the Abnormal Involuntary Movement Scale (AIMS), The Unified Parkinson's Disease Rating Scale (UPDRS) part IV, the Obeso Dyskinesia Rating Scale, the Rush Dyskinesia Rating Scale, the Clinical Dyskinesia Rating Scale (CDRS), the Lang-Fahn Activities of Daily Living Dyskinesia Scale, the Parkinson Disease Dyskinesia Scale (PDYS-26), and the Unified Dyskinesia Rating Scale (UDysRS). Based on this review, at present two of the reviewed dyskinesia scales (AIMS and the Rush Dyskinesia Rating Scale) fulfill criteria for Recommended for use in PD populations, albeit weakly so; all of the remaining met criteria to be Suggested. However, the two most recent scales (PDYS-26 and UDysRS) have excellent clinimetric properties and appear to provide a reliable and valid assessment tool of dyskinesia in PD. If they are used successfully beyond the groups that developed them, both have the potential to be re-ranked as Recommended. As further testing of these scales in PD is warranted, no new scales are needed until the available scales are fully tested clinimetrically.

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The purposes of this study were to use delayed gadolinium-enhanced MRI of cartilage (dGEMRIC) to evaluate the zonal distribution of glycosaminoglycans (GAGs) in normal cartilage and repair tissue and to use 3-T MRI to monitor the GAG content in matrix-associated autologous chondrocyte transplants.Fifteen patients who underwent matrix-associated autologous chondrocyte transplantation in the knee joint underwent MRI at baseline and 3-T follow-up MRI 1 year later. Total and zonal changes in longitudinal relaxivity (deltaR1) and relative deltaR1 were calculated for repair tissue and normal hyaline cartilage and compared by use of analysis of variance.There was a significant difference between the mean deltaR1 of repair tissue and that of reference cartilage at baseline and follow-up (p0.001). There was a significant increase in deltaR1 value and a decrease in GAG content from the deep layer to the superficial layer in the reference cartilage and almost no variation and significantly higher values for the repair tissue at both examinations. At 1-year follow-up imaging, there was a 22.7% decrease in deltaR1 value in the deep zone of the transplant.T1 mapping with dGEMRIC at 3 T shows the zonal structure of normal hyaline cartilage, highly reduced zonal variations in repair tissue, and a tendency toward an increase in global and zonal GAG content 1 year after transplantation.

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