Chronic nonspherocytic hemolytic anemia (CNSHA) and glucose 6 phosphate dehydrogenase (G6PD) deficiency in a patient with familial amyloidotic polyneuropathy (FAP)

Summary A new glucose-6-phosphate dehydrogenase (G6PD) variant with severe erythrocytic G6PD deficiency and a unique pH optimum is described in a young patient with chronic nonspherocytic hemolytic anemia (CNSHA) and familial amyloidotic polyneuropathy (FAP). Chronic hemolysis was present in the absence of infections, oxidant drugs or ingestion of faba beans. Residual enzyme activity was about 2.6% and 63% of normal activity in erythrocytes and leucocytes, respectively. A molecular study using standard methods showed G6PD in the patient to have normal electrophoretic mobility (at pH 7.0, 8.0 and 8.8), normal apparent affinity for substrates (K_m, G6P and NADP) and a slightly abnormal utilization of substrate analogues (decreased deamino-NADP and increased 2-deoxyglucose-6-phosphate utilization). Heat stability was found to be markedly decreased (8% of residual activity after 20 min of incubation at 46°C) and a particular characteristic of this enzyme was a biphasic pH curve with a greatly increased activity at low pH. Although molecular characteristics of this variant closely resemble those of G6PD Bangkok and G6PD Duarte, it can be distinguished from these and all other previously reported variants by virtue of its unusual pH curve. Therefore the present variant has been designated G6PD Clinic to distinguish it from other G6PD variants previously described.     

The presence of nuclear families in prehistoric collective burials revisited: the bronze age burial of Montanissell Cave (Spain) in the light of aDNA

Ancient populations have commonly been thought to have lived in small groups where extreme endogamy was the norm. To contribute to this debate, a genetic analysis has been carried out on a collective burial with eight primary inhumations from Montanissell Cave in the Catalan pre-Pyrenees. Radiocarbon dating clearly placed the burial in the Bronze Age, around 3200 BP. The composition of the group-two adults (one male, one female), one young woman, and five children from both sexes-seemed to represent the structure of a typical nuclear family. The genetic evidence proves this assumption to be wrong. In fact, at least five out of the eight mitochondrial haplotypes were different, denying the possibility of a common maternal ancestor for all of them. Nevertheless, 50% of the inhumations shared haplogroup J, so the possibility of a maternal relationship cannot be ruled out. Actually, combining different analyses performed using ancient and living populations, the probability of having four related J individuals in Montanissell Cave would range from 0.9884 to 0.9999. Owing to the particularities of this singular collective burial (small number of bodies placed altogether in a hidden cave, the evidence of non-simultaneous interments, close dating and unusual grave goods), we suggest that it might represent a small group with a patrilocal mating system.     

Irrigation mix: how to include water sources when assessing freshwater consumption impacts associated to crops

Purpose

Impacts of activities related to freshwater use are gaining interest among the life cycle assessment (LCA) community and several approaches are nowadays available in the literature. However, the general trend still is to ignore the assessment of its impact or, luckily, its inclusion on the inventory. This paper describes a procedure to incorporate water source information at the inventory level and evaluate the influence of that profile on the environmental impact assessment level.

Methods

The methodology lies on two main elements: the “irrigation mix” concept and the freshwater ecosystem impact indicator already defined in the literature. By doing so, the results obtained can be easily integrated in LCA studies of irrigated crops, or more complex studies with agricultural ingredients, where only information regarding the amount (but not the origin) of irrigation water is available.

Results and discussion

The results make more visible the benefits associated to the use of nonconventional, artificial water sources, by quantifying the improvement achieved on the water stress of a specific basin. Besides, the irrigation mix gives a better picture of the real contribution of irrigation to other impact categories (here, the global warming potential). Finally, the results were applied in a LCA study of lettuce production (an irrigated product cultivated in the studied region), and the method was analyzed against the criteria defined by the International Reference Life Cycle Data System handbook.

Conclusions

The inclusion of the water mix in the inventory level (irrigation profile) as well as in the impact assessment level (water stress index) is straightforward to apply by LCA practitioners, resulting in a more realistic assessment of the impacts of freshwater consumption associated to crops. The implementation on a case study allowed the quantification of promoting alternative water sources in a region suffering from significant water stress as well as to improve knowledge on the environmental impact associated to freshwater consumed by one of the irrigated crops grown there. We recommend using the approach defined here in order to check its applicability to other river basins and products.     

Assessing potential desertification environmental impact in life cycle assessment. Part 2: agricultural case study in Spain and Argentina

Purpose

Land use in dry lands can result in a final stage where land is completely depleted or entirely degraded causing the desertification phenomenon. The first part (part 1) of this series of two articles proposed a methodology to include desertification in life cycle assessment (LCA). A set of variables to be measured in the life cycle inventory, characterization factors, and an impact assessment method for the life cycle impact assessment phase were proposed. This second part (part 2) aims at showing the application of the model proposed in part 1 on two case studies of agricultural activities.

Methods

The impact model proposed is applied to plots of land devoted to agricultural activities in two countries: Argentina and Spain. In the agricultural plots of Spain (1SP to 9SP), two crops were analyzed: winter wheat (Triticum aestivum) and rapeseed (RS, Brassica napus). Two crops were considered in the Argentinean case study: rapeseed (RS, B. napus) and digit grass (Digitaria eriantha) (10AR to 17AR). A bare soil state is considered in both countries as a reference state. Both case studies consider only the agricultural stage in the inventory of a complete life cycle assessment study. Both also consider only one impact category in life cycle environmental assessment: desertification impact due to land occupation.

Results and discussion

On the basis of the obtained results, it can be inferred that cultivating 1 ha of rapeseed and 1 ha of wheat has the same impact on the analyzed plots in Spain and improves the reference state conditions in 50 % of the cases. Moreover, rapeseed grown in Mendoza produces almost the same impact as in some of the Spanish plots. Normalized areas of plots could be useful to compare results in different regions of the world to avoid the influence of the area of occupation in results.

Conclusions

The proposed model implies a contribution of significant importance because so far there has not been an impact assessment tool for land use in dry lands within the LCA framework. The main strength of the proposed model is that it allows a simple way to quantify the desertification impact. Also, it is emphasized that the model can be adapted virtually without difficulty to the evaluation of all types of crops with different management practices in different regions in the life cycle impact assessment stage.     

Functional plasticity of the human humerus: Shape,rigidity, and muscular entheses

The relationship between the mechanical loading undergone by a bone and its form has been widely assumed as a premise in studies aiming to reconstruct behavioral patterns from skeletal remains. Nevertheless, this relationship is complex due to the existence of many factors affecting bone structure and form, and further research combining structural and shape characteristics is needed. Using two‐block PLS, which is a test to analyze the covariance between two sets of variables, we aim to investigate the relationship between upper‐limb entheseal changes, cross‐sectional properties, and contour shape of the humeral diaphysis. Our results show that individuals with strongly marked entheseal changes have increased diaphyseal rigidities. Bending rigidities are mainly related to entheseal changes of muscles that cross the shoulder. Moreover, the entheseal changes of muscles that participate in the rotation of the arm are related to mediolaterally flatter and ventrodorsally broader humeral shapes in the mid‐proximal diaphysis. In turn, this diaphyseal shape is related to diaphyseal rigidity, especially to bending loadings. The shape of the diaphysis of the rest of the humerus does not covary either with rigidity or with entheseal changes. The results indicate that large muscular scars, such as those found in the mid‐proximal diaphyses, seem to be related to diaphyseal shape, whereas this relationship is not seen for areas with less direct influences of powerful muscles. Am J Phys Anthropol 150:609–617, 2013. © 2013 Wiley Periodicals, Inc.     

Critical analysis of life cycle inventory datasets for organic crop production systems

Purpose

Organic agriculture (OA) has gained widespread popularity due to its view as a more sustainable method of farming. Yet OA and conventional agriculture (CA) can be found to have similar or varying environmental performance using tools such as life cycle assessment (LCA). However, the current state of LCA does not accurately reflect the effects of OA; thus the aim of the present study was to identify gaps in the inventory stage and suggest improvements.

Methods

This article presents for the first time a critical analysis of the life cycle inventory (LCI) of state-of-the-art organic crop LCIs from current and recommended LCA databases ecoinvent and AGRIBALYSE®. The effects of these limitations on LCA results were analyzed and detailed ways to improve upon them were proposed.

Results and discussion

Through this analysis, unrepresentative plant protection product (PPP) manufacturing and organic fertilizer treatment inventories were found to be the main limitations in background processes, due to either the lack of available usage statistics, exclusion from the study, or use of unrepresentative proxies. Many organic crop LCIs used synthetic pesticide or mineral fertilizer proxies, which may indirectly contain OA prohibited chemicals. The effect of using these proxies can contribute between 4–78% to resource and energy-related impact categories. In a foreground analysis, the fertilizer and PPP emission models utilized by ecoinvent and AGRIBALYSE® were not well adapted to organic-authorized inputs and used simplified modeling assumptions. These critical aspects can be transferred to respective LCAs that use this data, potentially yielding unrepresentative results for relevant categories. To improve accuracy and to contribute novel data to the scientific community, new manufacturing LCIs were created for a few of the missing PPPs, as well as recommendations for fertilizer treatment LCIs and more precise emission models for PPPs and fertilizers.

Conclusions

The findings in the present article add much needed transparency regarding the limitations of available OA LCIs, offers guidance on how to make OA LCIs more representative, allow for more accurate comparisons between conventional and OA, and help practitioners to better adapt LCA methodology to OA systems.

     

Functional implications of radial diaphyseal curvature

A recent study (Galtés et al.: Am J Phys Anthropol 135 (2008) 293-300) demonstrated that during pronation, pronator teres exerts a favorable force for radial lateral bending. On the basis of this finding, we hypothesized that the pattern of muscular loading exerted on the radius by this muscle might play a role as a mechanical stimulus involved in radial bowing. The current work relates the hypertrophy of the forearm muscles to the degree of lateral curvature of the radial diaphysis. The analysis is based on an original osteometrical index to estimate radial curvature, and it applies a visual reference method to grade the osteological appearance of 10 entheses of 104 radii from archaeological and contemporary samples. Using these morphological data as an indirect method to measure the association between muscular hypertrophy and bone curvature, this study reveals that the pattern of muscular loading exerted on the apex of the radial shaft by the pronator teres muscle may play an important role as a mechanical stimulus involved in diaphyseal bowing.     

LCA of a tomato crop in a multi-tunnel greenhouse in Almeria

Purpose

Protected crops have expanded significantly in the Mediterranean area over the last few decades as a successful means to provide abundant and high-quality produce. Although resources are generally used efficiently, greenhouse areas cause major environmental impacts. The aim of this work was to study, from an environmental point of view, the improvement capacity of greenhouse areas in the Mediterranean region and to assess several alternative agricultural practices to decrease their contribution to the environmental impacts in this system.

Materials and methods

The methodology used was life cycle assessment (LCA) based on a tomato crop grown in a multi-tunnel greenhouse in Almeria, on the southeast coast of Spain. The functional unit chosen was 1 ton of loose classic tomatoes. Five midpoint impact categories and one energy flow indicator were selected for their relevance. The agricultural practice alternatives evaluated were reduction of volume of substrate and fertilizers, extension of substrate and greenhouse life span and increase in renewable energy for electricity production.

Results and discussion

The results indicated that the main contributors to impact categories in the tomato production were structure, auxiliary equipment and fertilizers. Structure accounted for between 30 and 48?% of the contributions, depending on the impact category. The principal burdens in the auxiliary equipment stage were substrate and consumption of electricity. Fertilizers environmental impacts were due to emissions during their manufacture and application to the crop. In a best-case option, taking into account the best alternatives, contributions to the impact categories were reduced by between 17 and 30?%. The LCA methodology proved to be a useful tool to evaluate the environmental damage of this agricultural activity. The importance of including farm infrastructure in the assessment was demonstrated as it was a major contributor. The risk of eutrophication could be reduced by adjustment of the fertilizers?Cwater balance and implementation of a closed-loop irrigation system. Future technological improvements should be developed to increase yields and thereby directly reduce the environmental burdens per unit produce.

Conclusions

The present study served to assess the environmental impacts of a tomato crop in a multi-tunnel greenhouse on the coast of Almeria. The assessment was used to evaluate alternatives for improvement of cleaner production in greenhouse areas. Further research should focus on assuring the feasibility of the suggested options.