A generic method of engagement to elicit regional coastal management options

Stakeholder engagement is important for successful management of natural resources, both to make effective decisions and to obtain support. However, in the context of coastal management, questions remain unanswered on how to effectively link decisions made at the catchment level with objectives for marine biodiversity and fisheries productivity. Moreover, there is much uncertainty on how to best elicit community input in a rigorous manner that supports management decisions. A decision support process is described that uses the adaptive management loop as its basis to elicit management objectives, priorities and management options using two case studies in the Great Barrier Reef, Australia. The approach described is then generalised for international interest. A hierarchical engagement model of local stakeholders, regional and senior managers is used. The result is a semi-quantitative generic elicitation framework that ultimately provides a prioritised list of management options in the context of clearly articulated management objectives that has widespread application for coastal communities worldwide. The case studies show that demand for local input and regional management is high, but local influences affect the relative success of both engagement processes and uptake by managers. Differences between case study outcomes highlight the importance of discussing objectives prior to suggesting management actions, and avoiding or minimising conflicts at the early stages of the process. Strong contributors to success are a) the provision of local information to the community group, and b) the early inclusion of senior managers and influencers in the group to ensure the intellectual and time investment is not compromised at the final stages of the process. The project has uncovered a conundrum in the significant gap between the way managers perceive their management actions and outcomes, and community's perception of the effectiveness (and wisdom) of these same management actions.

Understanding Retail Productivity by Simulating Management Practices

Intelligent agents offer a new and exciting way of understanding the world of work. In this paper we apply agent-based modeling and simulation to investigate a set of problems in a retail context. Specifically, we are working to understand the relationship between human resource management practices and retail productivity. Despite the fact we are working within a relatively novel and complex domain, it is clear that intelligent agents could offer potential for fostering sustainable organizational capabilities in the future. Our research so far has led us to conduct case study work with a top ten UK retailer, collecting data in four departments in two stores. Based on our case study data we have built and tested a first version of a department store simulator. In this paper we will report on the current development of our simulator which includes new features concerning more realistic data on the pattern of footfall during the day and the week, a more differentiated view of customers, and the evolution of customers over time. This allows us to investigate more complex scenarios and to analyze the impact of various management practices.

In vivo and in vitro disappearance of energy and nutrients in novel carbohydrates and cereal grains by pigs

In vivo and in vitro experiments were conducted to determine digestibility of GE and nutrients, as well as DE and ME of carbohydrates fed to growing pigs. The objective of Exp. 1 was to determine the DE and ME of 4 novel carbohydrates fed to pigs. The 4 novel carbohydrates were 2 sources of resistant starch (RS 60 and RS 70), soluble corn fiber (SCF), and pullulan. These carbohydrates were produced to increase total dietary fiber (TDF) intake by humans. Maltodextrin (MD) was used as a highly digestible control carbohydrate. The DE and ME for RS 60 (1,779 and 1,903 kcal/kg, respectively), RS 75(1,784 and 1,677 kcal/kg, respectively), and SCF (1,936 and 1,712 kcal/kg, respectively) were less (P < 0.05) than for MD (3,465 and 3,344 kcal/kg, respectively) and pullulan (2,755 and 2,766 kcal/kg, respectively), and pullulan contained less (P < 0.05) DE and ME than MD. However, there was no difference in the DE and ME for RS 60, RS 75, and SCF. The varying degrees of small intestinal digestibility and differences in fermentability among these novel carbohydrates may explain the differences in the DE and ME among carbohydrates. Therefore, the objectives of Exp. 2 were to determine the effect of these 4 novel carbohydrates and cellulose on apparent ileal (AID) and apparent total tract (ATTD) disappearance, and hindgut disappearance (HGD) of GE, TDF, and nutrients when added to diets fed to ileal-cannulated pigs. The second objective was to measure the endogenous flow of TDF to be able to calculate the standardized ileal disappearance (SID) and standardized total tract (STTD) disappearance of TDF in the 4 novel fibers fed to pigs. Results of the experiment indicated that the AID of GE and DM in diets containing cellulose or the novel fibers was less (P < 0.05) than of the maltodextrin diet, but the ATTD of GE and DM was not different among diets. The addition of RS 60, RS 75, and SCF did not affect the AID of acid hydrolysed ether extract (AEE), CP, or ash, but the addition of cellulose and pullulan reduced (P < 0.01) the AID of CP. The average ileal and total tract endogenous losses of TDF were calculated to be 25.25 and 42.87 g/kg DMI, respectively. The SID of TDF in diets containing RS 60, SCF, and pullulan were greater (P < 0.01) than the SID of TDF in the cellulose diet, but the STTD of the SCF diet was greater (P < 0.05) than for the cellulose and pullulan diets. Results of this experiment indicate that the presence of TDF reduces small intestinal disappearance of total carbohydrates and energy which may reduce the DE and ME of diets and ingredients. Therefore, the objective of Exp. 3 was to determine the DE and ME in yellow dent corn, Nutridense corn, dehulled barley, dehulled oats, polished rice, rye, sorghum, and wheat fed to growing pigs and to determine the AID and ATTD of GE, OM, CP, AEE, starch, total carbohydrates, and TDF in these cereal grains fed to pigs. Results indicated that the AID of GE, OM, and total carbohydrates was greater (P < 0.001) in rice than in all other cereal grains. The AID of starch was also greater (P < 0.001) in rice than in yellow dent corn, dehulled barley, rye, and wheat. The ATTD of GE was greater (P < 0.001) in rice than in yellow dent corn, rye, sorghum, and wheat. With a few exceptions, the AID and ATTD of GE and nutrients in Nutridense corn was not different from the values for dehulled oats. Likewise, with a few exceptions, the AID, ATTD, and HGD of GE, OM, total carbohydrates, and TDF in yellow corn, sorghum, and wheat were not different from each other. The AID of GE and AEE in dehulled barley was greater (P < 0.001) than in rye. The ATTD of GE and most nutrients was greater (P < 0.001) in dehulled barley than in rye. Dehulled oats had the greatest (P < 0.001) ME (kcal/kg DM) whereas rye had the least ME (kcal/kg DM) among the cereal grains. Results of the experiment indicate that the presence of TDF and RS may reduce small intestinal digestibility of starch in cereal grains resulting in reduced DE and ME in these grains. Digestibility experiments involving animals are time consuming and expensive. Therefore, the objective of Exp. 4 was to correlate DM and OM digestibility obtained from 3 in vitro procedures with ATTD of GE and with the concentration of DE in 50 corn samples that were fed to growing pigs. The second objective was to develop a regression model that can predict the ATTD of GE or the concentration of DE in corn. The third objective was to evaluate the suitability of using the DaisyII incubator as an alternative to the traditional water bath when determining in vitro DM and OM digestibility. Results indicated that corn samples incubated with Viscozyme for 48 h in the DaisyII incubator improved (P < 0.001) the ability of the procedure to detect small differences in the ATTD of GE or to detect small differences in the concentration of DE in corn. Likewise, compared with using cellulase or fecal inoculum, the variability in the ATTD of GE and the variability in the DE in corn was better (R2 = 0.56; P < 0.05 and R2 = 0.53; P < 0.06, respectively) explained if Viscozyme was used than if cellulase or fecal inoculum was used. A validated regression model that predicted the DE in corn was developed using Viscozyme and with the corn samples incubated in the DaisyII incubator for a 48 h. In conclusion, this present work used the pig as a model for human gastrointestinal function and evaluates carbohydrates from 2 different nutritional perspectives – humans and animals. The addition of novel carbohydrates reduced the digestibility of energy in the diets without necessarily reducing the digestibility of other nutrients. Thus, supplementation of novel carbohydrates in the diets may be beneficial for the management of diabetes. Aside from diabetic management, cereal grains such as rye and sorghum, may also help in BW management because of there low caloric value, but for undernourished individuals, dehulled oats, dehulled barley, and rice are the ideal grains. From an animal nutrition standpoint, high concentration of dietary fiber is undesirable because it reduces feed efficiency. Therefore, the inclusion of feed ingredients that have a high concentration of dietary fiber is often limited in animal diets. Although in vivo determination is ideal, in vitro procedures are useful tools to determine caloric value of food and feed ingredients.

Optimizing Western Flower Thrips Management on French Beans by Combined Use of Beneficials and Imidacloprid

Western flower thrips (WFT), Frankliniella occidentalis (Pergande), is an important pest of vegetable crops worldwide and has developed resistance to many insecticides. The predatory mites Neoseiulus (=Amblyseius) cucumeris (Oudemans), the entomopathogenic fungus Metarhizium anisopliae (Metsch.), and an insecticide (imidacloprid) were tested for their efficacy to reduce WFT population density and damage to French bean (Phaseolus vulgaris L.) pods under field conditions in two planting periods. Metarhizium anisopliae was applied as a foliar spray weekly at a rate of one litre spray volume per plot while imidacloprid was applied as a soil drench every two weeks at a rate of two litres of a mixture of water and imidacloprid per m(2). Neoseiulus cucumeris was released every two weeks on plant foliage at a rate of three mites per plant. Single and combined treatment applications reduced WFT population density by at least three times and WFT damage to French bean pods by at least 1.7 times compared with untreated plots. The benefit-cost ratios in management of WFT were profitable with highest returns realized on imidacloprid treated plots. The results indicate that M. anisopliae, N. cucumeris, and imidacloprid have the potential for use in developing an integrated pest management program against WFT on French beans.

A gestão da informação sob a luz do Enterprise Content Management (ECM): um estudo de caso em uma universidade pública

This work involves the organization and content perspectives on Enterprise Content Management (ECM) framework. The case study at the Federal University of Rio Grande do Norte was based on ECM model to analyse the information management provided by the three main administrative systems: The Integrated Management of Academic Activities (SIGAA), Integrated System of Inheritance, and Contracts Administration (SIPAC) and the Integrated System for Administration and Human Resources (SIGRH). A case study protocol was designed to provide greater reliability to research process. Four propositions were examined in order to reach the specific objectives of identification and evaluation of ECM components from UFRN perspective. The preliminary phase provided the guidelines for the data collection. In total, 75 individuals were interviewed. Interviews with four managers directly involved on systems design were recorded (average duration of 90 minutes). The 70 remaining individuals were approached in random way in UFRN s units, including teachers, administrative-technical employees and students. The results showed the presence of many ECM elements in the management of UFRN administrative information. The technological component with higher presence was "management of web content / collaboration". But initiatives of other components (e.g. email and document management) were found and are in continuous improvement. The assessment made use of eQual 4.0 to examine the effectiveness of applications under three factors: usability, quality of information and offered service. In general, the quality offered by the systems was very good and walk side by side with the obtained benefits of ECM strategy adoption in the context of the whole institution

An ontology for risk management of digital collections

Maintaining accessibility to and understanding of digital information over time is a complex challenge that often requires contributions and interventions from a variety of individuals and organizations. The processes of preservation planning and evaluation are fundamentally implicit and share similar complexity. Both demand comprehensive knowledge and understanding of every aspect of to-be-preserved content and the contexts within which preservation is undertaken. Consequently, means are required for the identification, documentation and association of those properties of data, representation and management mechanisms that in combination lend value, facilitate interaction and influence the preservation process. These properties may be almost limitless in terms of diversity, but are integral to the establishment of classes of risk exposure, and the planning and deployment of appropriate preservation strategies. We explore several research objectives within the course of this thesis. Our main objective is the conception of an ontology for risk management of digital collections. Incorporated within this are our aims to survey the contexts within which preservation has been undertaken successfully, the development of an appropriate methodology for risk management, the evaluation of existing preservation evaluation approaches and metrics, the structuring of best practice knowledge and lastly the demonstration of a range of tools that utilise our findings. We describe a mixed methodology that uses interview and survey, extensive content analysis, practical case study and iterative software and ontology development. We build on a robust foundation, the development of the Digital Repository Audit Method Based on Risk Assessment. We summarise the extent of the challenge facing the digital preservation community (and by extension users and creators of digital materials from many disciplines and operational contexts) and present the case for a comprehensive and extensible knowledge base of best practice. These challenges are manifested in the scale of data growth, the increasing complexity and the increasing onus on communities with no formal training to offer assurances of data management and sustainability. These collectively imply a challenge that demands an intuitive and adaptable means of evaluating digital preservation efforts. The need for individuals and organisations to validate the legitimacy of their own efforts is particularly prioritised. We introduce our approach, based on risk management. Risk is an expression of the likelihood of a negative outcome, and an expression of the impact of such an occurrence. We describe how risk management may be considered synonymous with preservation activity, a persistent effort to negate the dangers posed to information availability, usability and sustainability. Risk can be characterised according to associated goals, activities, responsibilities and policies in terms of both their manifestation and mitigation. They have the capacity to be deconstructed into their atomic units and responsibility for their resolution delegated appropriately. We continue to describe how the manifestation of risks typically spans an entire organisational environment, and as the focus of our analysis risk safeguards against omissions that may occur when pursuing functional, departmental or role-based assessment. We discuss the importance of relating risk-factors, through the risks themselves or associated system elements. To do so will yield the preservation best-practice knowledge base that is conspicuously lacking within the international digital preservation community. We present as research outcomes an encapsulation of preservation practice (and explicitly defined best practice) as a series of case studies, in turn distilled into atomic, related information elements. We conduct our analyses in the formal evaluation of memory institutions in the UK, US and continental Europe. Furthermore we showcase a series of applications that use the fruits of this research as their intellectual foundation. Finally we document our results in a range of technical reports and conference and journal articles. We present evidence of preservation approaches and infrastructures from a series of case studies conducted in a range of international preservation environments. We then aggregate this into a linked data structure entitled PORRO, an ontology relating preservation repository, object and risk characteristics, intended to support preservation decision making and evaluation. The methodology leading to this ontology is outlined, and lessons are exposed by revisiting legacy studies and exposing the resource and associated applications to evaluation by the digital preservation community.

Project done for the Library Management course

The aim of Library Administration course led by Professor Gilda Chacon is that in addition to understanding the theory to be developed objectives for good management, learn to apply to a library or a department. Our group through this study sought to determine in what way the principles and purposes of the administration are applied in the Reference Department of the Central Library of the National University. Importantly, we believe that the Department of reference is the union between the user and bibliographic, and that its aim is to keep the user the right information. The theoretical part of the paper is prepared based on guided reading and class discussion of the basic literature presented in the course syllabus, information corresponding to the reference unit was obtained through collaboration that the staff gave us the same through interviews, written data, reports and so on.

Representativeness, legitimacy and power in public involvement in health-care management

Public participation in health-service management is an increasingly prominent policy internationally. Frequently, though, academic studies have found it marginalized by health professionals who, keen to retain control over decision-making, undermine the legitimacy of involved members of the public, in particular by questioning their representativeness. This paper examines this negotiation of representative legitimacy between staff and involved users by drawing on a qualitative study of service-user involvement in pilot cancer-genetics services recently introduced in England, using interviews, participant observation and documentary analysis. In contrast to the findings of much of the literature, health professionals identified some degree of representative legitimacy in the contributions made by users. However, the ways in which staff and users constructed representativeness diverged significantly. Where staff valued the identities of users as biomedical and lay subjects, users themselves described the legitimacy of their contribution in more expansive terms of knowledge and citizenship. My analysis seeks to show how disputes over representativeness relate not just to a struggle for power according to contrasting group interests, but also to a substantive divergence in understanding of the nature of representativeness in the context of state-orchestrated efforts to increase public participation. This divergence might suggest problems with the enactment of such aspirations in practice; alternatively, however, contestation of representative legitimacy might be understood as reflecting ambiguities in policy-level objectives for participation, which secure implementation by accommodating the divergent constructions of those charged with putting initiatives into practice.

Errors in preparation and medication management in nursing professionals

The preparation and administration of medications is one of the most common and relevant functions of nurses, demanding great responsibility. Incorrect administration of medication, currently constitutes a serious problem in health services, and is considered one of the main adverse effects suffered by hospitalized patients. Objectives: Identify the major errors in the preparation and administration of medication by nurses in hospitals and know what factors lead to the error occurred in the preparation and administration of medication. Methods: A systematic review of the literature. Deined as inclusion criteria: original scientiic papers, complete, published in the period 2011 to May 2016, the SciELO and LILACS databases, performed in a hospital environment, addressing errors in preparation and administration of medication by nurses and in Portuguese language. After application of the inclusion criteria obtained a sample of 7 articles. Results: The main errors identiied in the pr eparation and administration of medication were wrong dose 71.4%, wrong time 71.4%, 57.2% dilution inadequate, incorrect selection of the patient 42.8% and 42.8% via inadequate. The factors that were most commonly reported by the nursing staff, as the cause of the error was the lack of human appeal 57.2%, inappropriate locations for the preparation of medication 57.2%, the presence of noise and low brightness in preparation location 57, 2%, professionals untrained 42.8%, fatigue and stress 42.8% and inattention 42.8%. Conclusions: The literature shows a high error rate in the preparation and administration of medication for various reasons, making it important that preventive measures of this occurrence are implemented.

Human resource management reform in the Indonesian civil service : towards strategic fit?

Studies of strategic HRM have dominated HRM research over the last three decades. Focusing on the HRM-organisation performance relationship, researchers take various themes and perspectives in their approach to strategic HRM. Among these themes, two contrasting approaches of strategic HRM continue to flourish: first, the best practice approach suggests that certain HRM practices will have the same effect irrespective of context and, second, the best fit approach suggests that the choice of HRM practices should be designed in accordance with an organisations’ specific context. While there is little consensus on what constitutes strategic HRM, the most common feature agreed in this field is the notion of the strategic integration; aligning HRM practices with organisations’ overall strategic objectives (vertical fit) and with each other (horizontal fit). Utilising the best fit approach as its theoretical framework, this study examines how vertical and horizontal fit is practised in the Indonesian civil service and what factors likely influence the prevalence of vertical and horizontal fit in the Indonesian civil service context. This study is significant for two important reasons. Firstly, the literature suggests that there are limited studies examining the best fit concept in the civil sector despite its implementation in the private sector positively contributing to organisational performance improvement. Secondly, the study provides enlightenment on how the best fit approach could contribute to performance improvement in the Indonesian civil service. This is in line with the fact that negative images of the Indonesian civil service are continuously highlighted although various HRM reform initiatives have been put in place. To achieve the objectives of the study, the qualitative case study approach accompanied by semi-structured interviews was employed involving 53 senior officials and one focus group discussion from eight Indonesian government agencies, consisting of three central agencies mandated to manage human resources, the National Bureaucratic Reform Team and four line agencies from both central and local governments. Thematic analysis was employed for data analyses and NVIVO software was used to manage the data. The study suggests three main findings. First, various HRM initiatives in relation to the HRM reform have been introduced in the Indonesian civil service differentiating them from the old HRM practices. However, the findings indicate that some HRM policies are still contradicting and hinder vertical and horizontal fit. Second, despite the contradictory policies, vertical and horizontal fit can be seen in the line agencies which have been acknowledged as ‘reformed agencies’. This demonstrates that the line agencies play an important role in aligning HRM practices with the line agencies’ goals and objectives and with one another although they are bounded by HRM policies that are unlikely to support the vertical and horizontal fit concept. Third, factors influencing the prevalence of vertical and horizontal fit include knowledge of contemporary HRM in both central agencies and line agencies, commitment from the line agencies’ leaders, devolvement of HRM to the line agencies and the socio-political and economic environments of the Indonesian civil service. The findings of the study raise policy, practical and theoretical implications. In terms of policy implications, the study highlights the importance of fit in HRM policies to support the achievement of the line agencies’ goals. Therefore, when formulating an HRM policy, the central agencies need to ensure that the HRM policy is linked to line agencies’ goals and to other HRM policies. This is to ensure synchronisation among the policies and thus maximising the achievement of the line agencies’ goals. From the practical perspectives, the study highlights important points which can be learned by the central agencies in carrying out their strategic role with regard to the formulation of HRM policies; by the line agencies in maximising the contribution of HRM to the achievement of the goals and objectives of the agencies through the implementation of the best fit concept, and by the leaders of the agencies in providing continuous support to each of the involved parties in the line agencies and involving the HRM department in all agency’s strategic decision-making. In relation to the theoretical implication, it is clear that the best fit approach is not thoroughly applied due to factors discussed previously. However, this does not mean that the best fit concept cannot be implemented. As argued by McCourt & Ramgutty-Wong (2003), instead of adopting the whole concept of best fit, a modulated approach reflecting the best fit concept, such as selecting individual HRM practices and experimenting with devolution, is possible for civil service organisations which still embrace centralised HRM systems. As demonstrated in the findings, some of the line agencies being studied seem to be ready to adopt the best fit approach given that they have knowledge of the best fit concept, strong support from the top leader, less political intervention and less corruption, collusion, and nepotism practices in their HRM practices.

Pressure management in water distribution systems: current status, proposals, and future trends

Pressure management (PM) is commonly used in water distribution systems (WDSs). In the last decade, a strategic objective in the field has been the development of new scientific and technical methods for its implementation. However, due to a lack of systematic analysis of the results obtained in practical cases, progress has not always been reflected in practical actions. To address this problem, this paper provides a comprehensive analysis of the most innovative issues related to PM. The methodology proposed is based on a case-study comparison of qualitative concepts that involves published work from 140 sources. The results include a qualitative analysis covering four aspects: (1) the objectives yielded by PM; (2) types of regulation, including advanced control systems through electronic controllers; (3) new methods for designing districts; and (4) development of optimization models associated with PM. The evolution of the aforementioned four aspects is examined and discussed. Conclusions regarding the current status of each factor are drawn and proposals for future research outlined

Gamification in Skills Management

Employees are the human capital which, to a great extent, contributes to the success and development of high-performance and sustainable organizations. In a work environment, there is a need to provide a tool for tracking and following-up on each employees' professional progress, while staying aligned with the organization’s strategic and operational goals and objectives. The research work within this Thesis aims to contribute to improve employees' selfawareness and auto-regulation; two predominant research areas are also studied and analyzed: Visual Analytics and Gamification. The Visual Analytics enables the specification of personalized dashboard interfaces with alerts and indicators to keep employees aware of their skills and to continuously monitor how to improve their expertise, promoting simultaneously behavioral change and adoption of good-practices. The study of Gamification techniques with Talent Management features enabled the design of new processes to engage, motivate, and retain highly productive employees, and to foster a competitive working environment, where employees are encouraged to be involved in new and rewarding activities, where knowledge and experience are recognized as a relevant asset. The Design Science Research was selected as the research methodology; the creation of new knowledge is therefore based on an iterative cycle addressing concepts such as design, analysis, reflection, and abstraction. By collaborating in an international project (Active@Work), funded by the Active and Assisted Living Programme, the results followed a design thinking approach regarding the specification of the structure and behavior of the Skills Development Module, namely the identification of requirements and the design of an innovative info-structure of metadata to support the user experience. A set of mockups were designed based on the user role and main concerns. Such approach enabled the conceptualization of a solution to proactively assist the management and assessment of skills in a personalized and dynamic way. The outcomes of this Thesis aims to demonstrate the existing articulation between emerging research areas such as Visual Analytics and Gamification, expecting to represent conceptual gains in these two research fields.

The Effect of Stress Management Program Using Cognitive Behavior Approach on Mental Health of the Mothers of the Children With Attention Deficit Hyperactivity Disorder

Background: Attention deficit hyperactivity disorder is one of the most common psychiatric disorders in children. Objectives: The study aimed to evaluate the effectiveness of stress management program using cognitive behavior approach on mental health of the mothers of the children with attention deficit hyperactivity disorder. Patients and Methods: In this interventional study, 90 mothers of the children with attention deficit hyperactivity disorder were randomly allocated into three intervention, placebo, and control groups. The general health questionnaire was used to measure mental health. Besides, stress was assessed through the depression-anxiety-stress scale. The two instruments were completed at baseline, immediately after, and one month after the intervention by the mothers. Afterwards, within group comparisons were made using one-sample repeated measurement ANOVA. One-way ANOVA was used for inter group comparisons. Mothers in the placebo group only participated in meetings to talk and express feelings without receiving any interventions. Results: At the baseline, no significant difference was found among the three groups regarding the means of stress, anxiety, depression, and mental health. However, a significant difference was observed in the mean score of stress immediately after the intervention (P = 0.033). The results also showed a significant difference among the three groups regarding the mean score of mental health (P < 0.001). One month after the intervention, the mean difference of mental health score remained significant only in the intervention group (P < 0.001). Conclusions: The study findings confirmed the effectiveness of stress management program utilizing cognitive behavior approach in mental health of the mothers of the children with attention deficit hyperactivity disorder.

Experience of a Single Center in NTBC Use in Management of Hereditary Tyrosinemia Type I in Libya

Background: Hereditary Tyrosinemia type I (HTI) is a metabolic disease caused by deficiency of fumarylacetoacetate hydrolase enzyme. Objectives: This study reports beside its clinical and biochemical presentation, the outcome of NTBC [2- (2-nitro-4-trifloro-methylbenzoyl)-1, 3-cyclohexanedion] treatment of the disease and evaluates its biochemical markers in 16 pediatric Libyan patients. Patients and Methods: The diagnosis was based on presence of high tyrosine levels in blood and succinylacetone in urine. Results: The consanguinity rate was 81.2%, the median age at onset, at diagnosis and at starting treatment were 4.5, 8, and 9.5 months respectively. At presentation hepatomegaly, jaundice, rickets and high gamma glutamyl transferase (GGT) were observed in 87.5% of patients. All patients had extremely high alpha fetoprotein (AFP) and high alkaline phosphatase (ALP) levels. Fifteen patients were treated with NTBC, normalization of PT (Prothrombine time) was achieved in average in 14 days. The other biochemical parameters of liver function (transaminases, GGT, ALP, bilirubin and albumin) took longer to improve and several months to be normalized. Survival rate with NTBC was 86.6%. Patients who started treatment in a median of 3 months post onset observed a fast drop of AFP in 90.6% of patients (P = 0.003). Abnormal liver function and rickets were the common presentations, GGT was an early cholestatic sensitive test. ALP was constantly high even in asymptomatic patients. Conclusions: In HT1 a faster dropping of AFP is a marker of good prognosis.