963 resultados para inborn-errors


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Weighing lysimeters are the standard method for directly measuring evapotranspiration (ET). This paper discusses the construction, installation, and performance of two (1.52 m × 1.52 m × 2.13-m deep) repacked weighing lysimeters for measuring ET of corn and soybean in West Central Nebraska. The cost of constructing and installing each lysimeter was approximately US $12,500, which could vary depending on the availability and cost of equipment and labor. The resolution of the lysimeters was 0.0001 mV V-1, which was limited by the data processing and storage resolution of the datalogger. This resolution was equivalent to 0.064 and 0.078 mm of ET for the north and south lysimeters, respectively. Since the percent measurement error decreases with the magnitude of the ET measured, this resolution is adequate for measuring ET for daily and longer periods, but not for shorter time steps. This resolution would result in measurement errors of less than 5% for measuring ET values of ≥3 mm, but the percent error rapidly increases for lower ET values. The resolution of the lysimeters could potentially be improved by choosing a datalogger that could process and store data with a higher resolution than the one used in this study.

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Purpose To provide a summary of the classic paper "Differences in the accommodation stimulus response curves of adult myopes and emmetropes" published in Ophthalmic and Physiological Optics in 1998 and to provide an update on the topic of accommodation errors in myopia. Summary The accommodation responses of 33 participants (10 emmetropes, 11 early onset myopes and 12 late onset myopes) aged 18-31 years were measured using the Canon Autoref R-1 free space autorefractor using three methods to vary the accommodation demand: decreasing distance (4 m to 0.25 cm), negative lenses (0 to -4 D at 4 m) and positive lenses (+4 to 0 D at 0.25 m). We observed that the greatest accommodation errors occurred for the negative lens method whereas minimal errors were observed using positive lenses. Adult progressing myopes had greater lags of accommodation than stable myopes at higher demands induced by negative lenses. Progressing myopes had shallower response gradients than the emmetropes and stable myopes; however the reduced gradient was much less than that observed in children using similar methods. Recent Findings This paper has been often cited as evidence that accommodation responses at near may be primarily reduced in adults with progressing myopia and not in stable myopes and/or that challenging accommodation stimuli (negative lenses with monocular viewing) are required to generate larger accommodation errors. As an analogy, animals reared with hyperopic errors develop axial elongation and myopia. Retinal defocus signals are presumably passed to the retinal pigment epithelium and choroid and then ultimately the sclera to modify eye length. A number of lens treatments that act to slow myopia progression may partially work through reducing accommodation errors.

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The thesis studies the translation process for the laws of Finland as they are translated from Finnish into Swedish. The focus is on revision practices, norms and workplace procedures. The translation process studied covers three institutions and four revisions. In three separate studies the translation process is analyzed from the perspective of the translations, the institutions and the actors. The general theoretical framework is Descriptive Translation Studies. For the analysis of revisions made in versions of the Swedish translation of Finnish laws, a model is developed covering five grammatical categories (textual revisions, syntactic revisions, lexical revisions, morphological revisions and content revisions) and four norms (legal adequacy, correct translation, correct language and readability). A separate questionnaire-based study was carried out with translators and revisers at the three institutions. The results show that the number of revisions does not decrease during the translation process, and no division of labour can be seen at the different stages. This is somewhat surprising if the revision process is regarded as one of quality control. Instead, all revisers make revisions on every level of the text. Further, the revisions do not necessarily imply errors in the translations but are often the result of revisers following different norms for legal translation. The informal structure of the institutions and its impact on communication, visibility and workplace practices was studied from the perspective of organization theory. The results show weaknesses in the communicative situation, which affect the co-operation both between institutions and individuals. Individual attitudes towards norms and their relative authority also vary, in the sense that revisers largely prioritize legal adequacy whereas translators give linguistic norms a higher value. Further, multi-professional teamwork in the institutions studied shows a kind of teamwork based on individuals and institutions doing specific tasks with only little contact with others. This shows that the established definitions of teamwork, with people co-working in close contact with each other, cannot directly be applied to the workplace procedures in the translation process studied. Three new concepts are introduced: flerstegsrevidering (multi-stage revision), revideringskedja (revision chain) and normsyn (norm attitude). The study seeks to make a contribution to our knowledge of legal translation, translation processes, institutional translation, revision practices and translation norms for legal translation. Keywords: legal translation, translation of laws, institutional translation, revision, revision practices, norms, teamwork, organizational informal structure, translation process, translation sociology, multilingual.

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Purpose This study evaluated the impact of a daily and weekly image-guided radiotherapy protocols in reducing setup errors and setting of appropriate margins in head and neck cancer patients. Materials and methods Interfraction and systematic shifts for the hypothetical day 1–3 plus weekly imaging were extrapolated from daily imaging data from 31 patients (964 cone beam computed tomography (CBCT) scans). In addition, residual setup errors were calculated by taking the average shifts in each direction for each patient based on the first three shifts and were presumed to represent systematic setup error. The clinical target volume (CTV) to planning target volume (PTV) margins were calculated using van Herk formula and analysed for each protocol. Results The mean interfraction shifts for daily imaging were 0·8, 0·3 and 0·5 mm in the S-I (superior-inferior), L-R (left-right) and A-P (anterior-posterior) direction, respectively. On the other hand the mean shifts for day 1–3 plus weekly imaging were 0·9, 1·8 and 0·5 mm in the S-I, L-R and A-P direction, respectively. The mean day 1–3 residual shifts were 1·5, 2·1 and 0·7 mm in the S-I, L-R and A-P direction, respectively. No significant difference was found in the mean setup error for the daily and hypothetical day 1–3 plus weekly protocol. However, the calculated CTV to PTV margins for the daily interfraction imaging data were 1·6, 3·8 and 1·4 mm in the S-I, L-R and A-P directions, respectively. Hypothetical day 1–3 plus weekly resulted in CTV–PTV margins of 5, 4·2 and 5 mm in the S-I, L-R and A-P direction. Conclusions The results of this study show that a daily CBCT protocol reduces setup errors and allows setup margin reduction in head and neck radiotherapy compared to a weekly imaging protocol.

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Near infrared spectroscopy (NIRS) combined with multivariate analysis techniques was applied to assess phenol content of European oak. NIRS data were firstly collected directly from solid heartwood surfaces: in doing so, the spectra were recorded separately from the longitudinal radial and the transverse section surfaces by diffuse reflectance. The spectral data were then pretreated by several pre-processing procedures, such as multiplicative scatter correction, first derivative, second derivative and standard normal variate. The tannin contents of sawmill collected from the longitudinal radial and transverse section surfaces were determined by quantitative extraction with water/methanol (1:4, by vol). Then, total phenol contents in tannin extracts were measured by the Folin-Ciocalteu method. The NIR data were correlated against the Folin-Ciocalteu results. Calibration models built with partial least squares regression displayed strong correlation - as expressed by high determination correlation coefficient (r2) and high ratio of performance to deviation (RPD) - between measured and predicted total phenols content, and weak calibration and prediction errors (RMSEC, RMSEP). The best calibration was provided with second derivative spectra (r2 value of 0.93 for the longitudinal radial plane and of 0.91 for the transverse section plane). This study illustrates that the NIRS technique when used in conjunction with multivariate analysis could provide reliable, quick and non-destructive assessment of European oak heartwood extractives.

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Road traffic accidents are a large problem everywhere in the world. However, regional differences in traffic safety between countries are considerable. For example, traffic safety records are much worse in Southern Europe and the Middle East than in Northern and Western Europe. Despite the large regional differences in traffic safety, factors contributing to different accident risk figures in different countries and regions have remained largely unstudied. The general aim of this study was to investigate regional differences in traffic safety between Southern European/Middle Eastern (i.e., Greece, Iran, Turkey) and Northern/Western European (i.e., Finland, Great Britain, The Netherlands) countries and to identify factors related to these differences. We conducted seven sub-studies in which I applied a traffic culture framework, including a multi-level approach, to traffic safety. We used aggregated level data (national statistics), surveys among drivers, and data on traffic accidents and fatalities in the analyses. In the first study, we investigated the influence of macro level factors (i.e., economic, societal, and cultural) on traffic safety across countries. The results showed that a high GNP per capita and conservatism correlated with a low number of traffic fatalities, whereas a high degree of uncertainty avoidance, neuroticism, and egalitarianism correlated with a high number of traffic fatalities. In the second, third, and fourth studies, we examined whether the conceptualisation of road user characteristics (i.e., driver behaviour and performance) varied across traffic cultures and how these factors determined overall safety, and the differences between countries in traffic safety. The results showed that the factorial agreement for driver behaviour (i.e., aggressive driving) and performance (i.e., safety skills) was unsatisfactory in Greece, Iran, and Turkey, where the lack of social tolerance and interpersonal aggressive violations seem to be important characteristics of driving. In addition, we found that driver behaviour (i.e., aggressive violations and errors) mediated the relationship between culture/country and accidents. Besides, drivers from "dangerous" Southern European countries and Iran scored higher on aggressive violations and errors than did drivers from "safe" Northern European countries. However, "speeding" appeared to be a "pan-cultural" problem in traffic. Similarly, aggressive driving seems largely depend on road users' interactions and drivers' interpretation (i.e., cognitive biases) of the behaviour of others in every country involved in the study. Moreover, in all countries, a risky general driving style was mostly related to being young and male. The results of the fifth and sixth studies showed that among young Turkish drivers, gender stereotypes (i.e., masculinity and femininity) greatly influence driver behaviour and performance. Feminine drivers were safety-oriented whereas masculine drivers were skill-oriented and risky drivers. Since everyday driving tasks involve not only erroneous (i.e., risky or dangerous driving) or correct performance (i.e., normal habitual driving), but also "positive" driver behaviours, we developed a reliable scale for measuring "positive" driver behaviours among Turkish drivers in the seventh study. Consequently, I revised Reason's model [Reason, J. T., 1990. Human error. Cambridge University Press: New York] of aberrant driver behaviour to represent a general driving style, including all possible intentional behaviours in traffic while evaluating the differences between countries in traffic safety. The results emphasise the importance of economic, societal and cultural factors, general driving style and skills, which are related to exposure, cognitive biases as well as age, sex, and gender, in differences between countries in traffic safety.

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Alzheimer's disease (AD) is characterized by an impairment of the semantic memory responsible for processing meaning-related knowledge. This study was aimed at examining how Finnish-speaking healthy elderly subjects (n = 30) and mildly (n=20) and moderately (n = 20) demented AD patients utilize semantic knowledge to performa semantic fluency task, a method of studying semantic memory. In this task subjects are typically given 60 seconds to generate words belonging to the semantic category of animals. Successful task performance requires fast retrieval of subcategory exemplars in clusters (e.g., farm animals: 'cow', 'horse', 'sheep') and switching between subcategories (e.g., pets, water animals, birds, rodents). In this study, thescope of the task was extended to cover various noun and verb categories. The results indicated that, compared with normal controls, both mildly and moderately demented AD patients showed reduced word production, limited clustering and switching, narrowed semantic space, and an increase in errors, particularly perseverations. However, the size of the clusters, the proportion of clustered words, and the frequency and prototypicality of words remained relatively similar across the subject groups. Although the moderately demented patients showed a poor eroverall performance than the mildly demented patients in the individual categories, the error analysis appeared unaffected by the severity of AD. The results indicate a semantically rather coherent performance but less specific, effective, and flexible functioning of the semantic memory in mild and moderate AD patients. The findings are discussed in relation to recent theories of word production and semantic representation. Keywords: semantic fluency, clustering, switching, semantic category, nouns, verbs, Alzheimer's disease

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In a musical context, the pitch of sounds is encoded according to domain-general principles not confined to music or even to audition overall but common to other perceptual and cognitive processes (such as multiple pattern encoding and feature integration), and to domain-specific and culture-specific properties related to a particular musical system only (such as the pitch steps of the Western tonal system). The studies included in this thesis shed light on the processing stages during which pitch encoding occurs on the basis of both domain-general and music-specific properties, and elucidate the putative brain mechanisms underlying pitch-related music perception. Study I showed, in subjects without formal musical education, that the pitch and timbre of multiple sounds are integrated as unified object representations in sensory memory before attentional intervention. Similarly, multiple pattern pitches are simultaneously maintained in non-musicians' sensory memory (Study II). These findings demonstrate the degree of sophistication of pitch processing at the sensory memory stage, requiring neither attention nor any special expertise of the subjects. Furthermore, music- and culture-specific properties, such as the pitch steps of the equal-tempered musical scale, are automatically discriminated in sensory memory even by subjects without formal musical education (Studies III and IV). The cognitive processing of pitch according to culture-specific musical-scale schemata hence occurs as early as at the sensory-memory stage of pitch analysis. Exposure and cortical plasticity seem to be involved in musical pitch encoding. For instance, after only one hour of laboratory training, the neural representations of pitch in the auditory cortex are altered (Study V). However, faulty brain mechanisms for attentive processing of fine-grained pitch steps lead to inborn deficits in music perception and recognition such as those encountered in congenital amusia (Study VI). These findings suggest that predispositions for exact pitch-step discrimination together with long-term exposure to music govern the acquisition of the automatized schematic knowledge of the music of a particular culture that even non-musicians possess.

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Exposure assessment studies conducted in developing countries have been based on fixed-site monitoring to date. This is a major deficiency, leading to errors in estimating the actual exposures, which are a function of time spent and pollutant concentrations in different microenvironments. This study quantified school children’s daily personal exposure to ultrafine particles (UFP) using real-time monitoring, as well as volatile organic compounds (VOCs) and NO2 using passive sampling in rural Bhutan in order to determine the factors driving the exposures. An activity diary was used to track children’s time activity patterns, and difference in mean exposure levels across sex and indoor/outdoor were investigated with ANOVA. 82 children, attending three primary schools participated in this study; S1 and S2 during the wet season and S3 during the dry season. Mean daily UFP exposure (cm-3) was 1.08 × 104 for children attending S1, 9.81 × 103 for S2, and 4.19 × 104 for S3. The mean daily NO2 exposure (µg m-3) was 4.27 for S1, 3.33 for S2 and 5.38 for S3 children. Likewise, children attending S3 also experienced higher daily exposure to a majority of the VOCs than those attending S1 and S2. Time-series of UFP personal exposures provided detailed information on identifying sources of these particles and quantifying their contributions to the total daily exposures for each microenvironment. The highest UFP exposure resulted from cooking/eating, contributing to 64% of the daily exposure, due to firewood combustion in houses using traditional mud cookstoves. The lowest UFP exposures were during the hours that children spent outdoors at school. The outcomes of this study highlight the significant contributions of lifestyle and socio-economic factors in personal exposures and have applications in environmental risk assessment and household air pollution mitigation in Bhutan.

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Cereal grain is one of the main export commodities of Australian agriculture. Over the past decade, crop yield forecasts for wheat and sorghum have shown appreciable utility for industry planning at shire, state, and national scales. There is now an increasing drive from industry for more accurate and cost-effective crop production forecasts. In order to generate production estimates, accurate crop area estimates are needed by the end of the cropping season. Multivariate methods for analysing remotely sensed Enhanced Vegetation Index (EVI) from 16-day Moderate Resolution Imaging Spectroradiometer (MODIS) satellite imagery within the cropping period (i.e. April-November) were investigated to estimate crop area for wheat, barley, chickpea, and total winter cropped area for a case study region in NE Australia. Each pixel classification method was trained on ground truth data collected from the study region. Three approaches to pixel classification were examined: (i) cluster analysis of trajectories of EVI values from consecutive multi-date imagery during the crop growth period; (ii) harmonic analysis of the time series (HANTS) of the EVI values; and (iii) principal component analysis (PCA) of the time series of EVI values. Images classified using these three approaches were compared with each other, and with a classification based on the single MODIS image taken at peak EVI. Imagery for the 2003 and 2004 seasons was used to assess the ability of the methods to determine wheat, barley, chickpea, and total cropped area estimates. The accuracy at pixel scale was determined by the percent correct classification metric by contrasting all pixel scale samples with independent pixel observations. At a shire level, aggregated total crop area estimates were compared with surveyed estimates. All multi-temporal methods showed significant overall capability to estimate total winter crop area. There was high accuracy at pixel scale (>98% correct classification) for identifying overall winter cropping. However, discrimination among crops was less accurate. Although the use of single-date EVI data produced high accuracy for estimates of wheat area at shire scale, the result contradicted the poor pixel-scale accuracy associated with this approach, due to fortuitous compensating errors. Further studies are needed to extrapolate the multi-temporal approaches to other geographical areas and to improve the lead time for deriving cropped-area estimates before harvest.

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Near infrared (NIR) spectroscopy, usually in reflectance mode, has been applied to the analysis of faeces to measure the concentrations of constituents such as total N, fibre, tannins and delta C-13. In addition, an unusual and exciting application of faecal NIR [F.NIR] analyses is to directly predict attributes of the diet of herbivores such as crude protein and fibre contents, proportions of plant species and morphological components, diet digestibility and voluntary DM intake. This is an unusual application of NIR spectroscopy insofar as the spectral measurements are made, not on the material of interest [i.e. the diet), but on a derived material (i.e. faeces). Predictions of diet attributes from faecal spectra clearly depend on there being sufficient NIR spectral information in the diet residues present in faeces to describe the diet, although endogenous components of faeces such as undigested debris of micro-organisms from the rumen and Large intestine and secretions into the gastrointestinal tract wilt also contribute spectral information. Spectra of forage and of faeces derived from the forage are generally similar and the observed differences are principally in the spectral regions associated with constituents of forages known to be of low, or of high, digestibility. Some diet components (for example, ureal which are likely to be entirely digested apparently cannot be predicted from faecal NIR spectra because they cannot contribute to faecal spectra except through modifying the microbial and endogenous components. The errors and robustness of F.NIR calibrations to predict the crude protein concentration and digestibility of the diet of herbivores are generally comparable with those to directly predict the same attributes in forage from NIR spectra of the forage. Some attributes of the animal, such as species, gender, pregnancy status and parasite burden have been successfully discriminated into classes based on their faecal NIR spectra. Such discrimination was likely associated with differences in the diet selected and/or differences in the metabolites excreted in the faeces. NIR spectroscopy of faeces has usually involved scanning dried and ground samples in monochromators in the 400-2500nm or 1100-2500nm ranges. Results satisfactory for the purpose have also been reported for dried and ground faeces scanned using a diode array instrument in the 800-1700nm range and for wet faeces and slurries of excreta scanned with monochromators. Chemometric analysis of faecal spectra has generally used the approaches established for forage analysis. The capacity to predict many attributes of the diet, and some aspects of animal physiology, from NIR spectra of faeces is particularly useful to study the quality and quantity of the diet selected by both domestic and feral grazing herbivores and to enhance production and management of both herbivores and their grazing environment.

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In a study that included C-4 tropical grasses, C-3 temperate grasses and C-3 pasture legumes, in vitro dry matter digestibility of extrusa, measured as in vitro dry matter loss (IVDML) during incubation, compared with that of the forage consumed, was greater for grass extrusa but not for legume extrusa. The increase in digestibility was not caused by mastication or by the freezing of extrusa samples during storage but by the action of saliva. Comparable increases in IVDML were achieved merely by mixing bovine saliva with ground forage samples. Differences were greater than could be explained by increases due to completely digestible salivary DM. There was no significant difference between animals in relation to the saliva effect on IVDML and, except for some minor differences, similar saliva effects on IVDML were measured using either the pepsin-cellulase or rumen fluid-pepsin in vitro techniques. For both C-4 and C-3 grasses the magnitude of the differences were inversely related to IVDML of the feed and there was little or no difference between extrusa and feed at high digestibilities (>70%) whereas differences of more than 10 percentage units were measured on low quality grass forages. The data did not suggest that the extrusa or saliva effect on digestibility was different for C-3 grasses than for C-4 grasses but data on C-3 grasses were limited to few species and to high digestibility samples. For legume forages there was no saliva effect when the pepsin-cellulase method was used but there was a small but significant positive effect using the rumen fluid-pepsin method. It was concluded that when samples of extrusa are analysed using in vitro techniques, predicted in vivo digestibility of the feed consumed will often be overestimated, especially for low quality grass diets. The implications of overestimating in vivo digestibility and suggestions for overcoming such errors are discussed.

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Objectives. The sentence span task is a complex working memory span task used for estimating total working memory capacity for both processing (sentence comprehension) and storage (remembering a set of words). Several traditional models of working memory suggest that performance on these tasks relies on phonological short-term storage. However, long-term memory effects as well as the effects of expertise and strategies have challenged this view. This study uses a working memory task that aids the creation of retrieval structures in the form of stories, which have been shown to form integrated structures in longterm memory. The research question is whether sentence and story contexts boost memory performance in a complex working memory task. The hypothesis is that storage of the words in the task takes place in long-term memory. Evidence of this would be better recall for words as parts of sentences than for separate words, and, particularly, a beneficial effect for words as part of an organized story. Methods. Twenty stories consisting of five sentences each were constructed, and the stimuli in all experimental conditions were based on these sentences and sentence-final words, reordered and recombined for the other conditions. Participants read aloud sets of five sentences that either formed a story or not. In one condition they had to report all the last words at the end of the set, in another, they memorised an additional separate word with each sentence. The sentences were presented on the screen one word at a time (500 ms). After the presentation of each sentence, the participant verified a statement about the sentence. After five sentences, the participant repeated back the words in correct positions. Experiment 1 (n=16) used immediate recall, experiment 2 (n=21) both immediate recall and recall after a distraction interval (the operation span task). In experiment 2 a distracting mental arithmetic task was presented instead of recall in half of the trials, and an individual word was added before each sentence in the two experimental conditions when the participants were to memorize the sentence final words. Subjects also performed a listening span task (in exp.1) or an operation span task (exp.2) to allow comparison of the estimated span and performance in the story task. Results were analysed using correlations, repeated measures ANOVA and a chi-square goodness of fit test on the distribution of errors. Results and discussion. Both the relatedness of the sentences (the story condition) and the inclusion of the words into sentences helped memory. An interaction showed that the story condition had a greater effect on last words than separate words. The beneficial effect of the story was shown in all serial positions. The effects remained in delayed recall. When the sentences formed stories, performance in verification of the statements about sentence context was better. This, as well as the differing distributions of errors in different experimental conditions, suggest different levels of representation are in use in the different conditions. In the story condition, the nature of these representations could be in the form of an organized memory structure, a situation model. The other working memory tasks had only few week correlations to the story task. This could indicate that different processes are in use in the tasks. The results do not support short-term phonological storage, but instead are compatible with the words being encoded to LTM during the task.

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Raw data from SeaScan™ transects off Wide Bay (south Queensland) taken in August 2007 as part of a study of ecological factors influencing the distribution of spanner crabs (Ranina ranina). The dataset (comma-delimited ascii file) comprises the following fields: 1. record number 2. date-time (GMT) 3. date-time (AEST) 4. latitude (signed decimal degrees) 5. longitude (decimal degrees) 6. speed over ground (knots) 7. depth (m) 8. seabed roughness (v) 9. hardness (v) Indices of roughness and hardness (from the first and second echoes respectively) were obtained using a SeaScan™ 100 system (un-referenced) on board the Research Vessel Tom Marshall, with the ship’s Furuno FCV 1100 echo sounder and 1 kW, 50 kHz transducer. Generally vessel speed was kept below about 14 kt (typically ~12 kt), and the echo-sounder range set to 80 m. The data were filtered to remove errors due to data drop-out, straying beyond system depth limits (min. 10 m), or transducer interference.

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A model of root water extraction is proposed, in which a linear variation of extraction rate with depth is assumed. Five crops are chosen for simulation studies of the model, and soil moisture depletion under optimal conditions from different layers for each crop is calculated. Similar calculations are also made using the constant extraction rate model. Rooting depth is assumed to vary linearly with potential evapotranspiration for each crop during the vegetative phase. The calculated depletion patterns are compared with measured mean depletion patterns for each crop. It is shown that the constant extraction rate model results in large errors in the prediction of soil moisture depletion, while the proposed linear extraction rate model gives satisfactory results. Hypothetical depletion patterns predicted by the model in combination with a moisture tension-dependent sink term developed elsewhere are indicated.