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Wolman disease

Wolman disease (also known as Wolman’s disease, Wolman’s syndrom, and acid lipase deficiency) is a rare severe lipid storage disease that is usually fatal by age 1. This autosomal recessive disorder is marked by accumulation of cholesteryl esters (normally a transport form of cholesterol) and triglycerides (a chemical form in which fats exist in the body) that can build up significantly and cause damage in the cells and tissues. Both males and females are affected by this severe disorder. more...

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Infants are normal and active at birth but quickly develop progressive mental deterioration, hepatosplenomegaly (enlarged liver and grossly enlarged spleen), distended abdomen, gastrointestinal problems including steatorrhea (excessive amounts of fats in the stools), jaundice, anemia, vomiting and calcium deposits in the adrenal glands, causing them to harden.

There is no specific treatment for Wolman disease. Patients with anemia may require blood transfusions. In some patients, the enlarged spleen must be removed to improve cardiopulmonary function. Restricting one’s diet does not prevent lipid buildup in cells and tissues.

Wolman disease is named after Moshe Wolman.

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ASSESSING THE VALUES STRUCTURE AMONG UNITED ARAB EMIRATES UNIVERSITY STUDENTS
From Social Behavior and Personality, 1/1/04 by Simadi, Fayez A

The purpose of this study is to clarify the values structure among United Arab Emirates University students in general and in terms of gender and citizenship. The study sample size was 242 males and 353 females. Results indicated that religious and cognitive values came first in the structure, while social and economic values came in last. This structure was a little different in terms of citizenship and gender. These results are discussed on the basis of the literature.

A growing body of evidence indicates that deep-rooted changes in world view are taking place. These changes seem to be reshaping the economic, political, and social life in societies and the world. The most important body of evidence comes from the World Values Survey (WVS), which measured the values and beliefs of the public in all six inhabited continents in 1981, 1990 and 1995. The WVS has detected a pattern of systematic changes in values and motivations among those advanced industrial societies. These changes reflect economic and technological changes that have tremendously reduced the likelihood that people will die prematurely from starvation and disease (Inglehart, 2000).

In fact, the United Arab Emirates (UAE) have gone through dramatic changes in the last few decades because of the oil boom in the mid-seventies, which caused the UAE to move from a tribal system to the stage of operating as a state. However, traditional values still exist to some degree in UAE society (Wraikat & Simadi, 2001).

Values are defined as characteristics of individuals that clarify what is preferred, what is selected as being important and that then guides a person's life (Rokeach, 1973; Schwartz, 1992). The study of values is the main issue in the development of both individuals and societies. At the individual level, value priorities are the key to a person's beliefs, attitudes, and behavior, specifying what is preferred. At the cultural level, value structures of different cultural groups enable one to realize attributes characteristic of that particular culture (Aygun et al., 2002).

In one of the best-known approaches in the study of values, Rokeach (1973) defined values as modes of conduct and end states - namely instrumental and terminal values. He divided instrumental values into two categories: moral values are focused on interpersonal (e.g., helpfulness), and competence values with interpersonal (e.g., logical) modes of conduct. Rokeach classifies values as personal and social: personal values include self-centered values and self-respect, and social values include socially centered values such as equality, and world-at-peace.

There are several definitions of value: Allport, Vernon and Lindzey (1952) defined values as emotional-mental judgment toward some phenomenon. These researchers designed the first values instrument. Mouly (1982) defined values as a hypothetical concept, which illustrates the individual by work motivation toward some subjects, events, thoughts and other people. Rokeach (1973) considered values as being permanent beliefs about nature, behaviors and goals of life.

In Arabic literature Zahran (1982) defined values as a generalized emotional and mental judgment toward people, things, subjects and social events in life. Abu Neel (1985) defined values as a complicated system of evaluation of people, including positive and negative aspects, and with the evaluations starting from emotional or mental acceptance or rejection of subjects, individuals, and social events.

Schwartz (1992) assumed that values develop from three universal needs of individuals: biological, interactional, and social. These researchers and others derived motivationally distinct types of values from those universal requirements: self-direction, stimulation, hedonism, achievements, power, security, conformity, tradition, benevolence, and universalism. Moreover, they classified those values types into two dimensions according to conflicts and compatibility: openness to change (self-direction, stimulation) versus conservatism (conformity, tradition and security), and self-enhancement (power, achievement and hedonism) versus self-transcendence (universalism, benevolence). As a result, studies conducted among different cultures have revealed similar values types (Bond, 1988; Feather, 1991).

For this study the research considers values as: individual's mental judgment about things, people and social events; and this judgment determines the relationship between the individual and the objects. Values represent many things such as the person's view about purity, believing in God, benefit, democracy and so on. Operationally values will be measured by establishing a scale previously used by Attoum and Khasawneh (1999). This instrument has been used to determine the values structure of UAE university (UAEU) students. Moreover, this instrument will clarify the differences amoung students in their scores on the social, religious, political, aesthetic, economic and cognitive values domains.

Most Arabic researchers (Abu Neel, 1985; Khalefeh, 1992; Zahran, 1982) have agreed on the characteristics and sources of values as follows:

1. Values have a hierarchical order in terms of their priority in life.

2. Values have a high stability or they are difficult to change.

3. Values are affected by the individual's culture, so the environment can usually strengthen or weaken values.

4. Values can determine the individual's experiences, and thus affect his/her way of thinking.

Eagly and Chaiken (1993) have stated that values and attitudes share various components, described as follows: a) the cognitive component, which is made up of thoughts and information about objects, b) the emotional component, which comprises emotions and feelings about a subject, c) the behavioral component, which encompasses acts and responses of the individual toward the object.

In regard to the sources of values, as we said above, values are learned and gained through human environment interaction with numerous sources. (Almaghrabi, 1994, Newcomb, 1965, Zahran, 1982) have discriminated between sources of values. In the Arabic Moslem society, the first source of values is religion, which includes values that derive from the three main religions (Judaism, Christianity and Islam) - such as prayer and charity. The second source is social values, which are gained by the individual's interaction with his society - such as achievement, and democracy. In fact, those researchers concluded that social values are less stable than religious values.

Researchers have discussed various types of human values such as achievement, autonomy, religiosity, equality, frankness and generosity. These values have been classified by different scholars. The best-known classification was made by Allport, Vernon and Lindzey (1952). They classified values into six domains: cognitive, social, religious, political, economic and beauty. In general, each individual conceives his/her values in terms of priority or in terms of importance (Wolman, 1975).

Values can be developed through 3 stages. First, the acceptance stage: the individual takes values for granted. Second, the preference stage: the individual prefers and accepts values without being convinced. Third, the obligation stage: the individual is obligated to accept the values and refuses any violation of what they are about (Korathwohl, 1994).

Traditionally, the UAE society as a part of Arab Moslem society, has been characterized by close interpersonal relationships. The individual has a network of close ties, including the nuclear family, relatives, and close neighbors. The traditional socialization process emphasizes obedience, closeness, and loyalty to parents rather than independence and self-reliance. These characteristics came from traditional Islam instructions (Wraikat & Simadi, 2001).

However, with social change, different living styles and orientations toward life began to emerge. Although the basic family structure appears to be nuclear, it serves the functions of an extended family in terms of providing social, emotional, and material support, and thus may operate as a functionally extended family, and as a main resource of belonging and loyalty. But with recent changes in lifestyle, there are many resources of values - such as achievement, individuality, importance of planning - which have developed for Arab men, who are more interested in science, achievement, economic, freedom, autonomy, enjoyment and less concerned with traditional values (Wraikat & Simadi, 2001).

The gender role in the socialization process may serve as an important predictor of differences in values preferences. Accordingly, men tend to display more genetic and instrumental orientations (e.g., achievement and personal orientation) in their self-description and in their values preferences whereas women tend to show more concern for communal and expressive values (e.g., relationships with social orientation) (Aygun et al., 2002; Feather, 1991; Kashima et al., 1995; Wojciszke, 1997). In the present study we investigated the structure of values in UAEU students, in terms of gender and nationality to explore how their values are distributed and how they are ordered (top to bottom values).

The importance of the present study stems from the need to explore more about the UAEU students' thinking and their priorities in life. Furthermore, little research interest has been shown in such populations especially with regard to psychological and social studies. For this research the values instrument was defined as an abstract mental-emotional judgment of things, people and events in terms of desirability, that is, which is more desirable (Attoum & Khasawneh, 1999). The level of education was defined as study level (freshman and senior), while the discipline was defined in terms of humanities and sciences colleges. The present study asked the following questions:

- How are the values of UAE university students distributed within values structure in the cognitive, religious, social, political, aesthetic, and economic aspects?

- Is there an effect of gender on the distribution of value structure among UAE university students?

- Does the values structure differ by citizenship?

METHOD

POPULATION AND SAMPLE

The population for this study consisted of all the students in UAEU, a number that was estimated to be about 14,372 in October 2002, as stated by the Registration Department Statistics Report of UAEU (2002). The general population of the university was distributed throughout 8 colleges, which are: Humanities and Social Sciences, Medicine, Law, Education, Engineering, Administration, Information Technology, Sciences and Agriculture. The majority of the students are female.

For the study sample, data were obtained from 595 students, 242 males and 353 females. The males and females were selected randomly. Most of them were in the Humanities and Social Sciences College (70%), 81% of them were in the age group up to and including 25 years old and 84% were UAE nationals. It is important to note that about 70% of the sample were from the Humanities College because there is a similar percentage in the total number of students in UAEU. Table 1 shows the distribution of the sample based on sex and college type.

INSTRUMENTS

The instrument of value structure was used to measure the values among UAEU students. Scores of each item ranged from 0 to 3, where a score of 0 represented no choice and 3 represented choice of the aspect of value. Thus lower grades would indicate less choice of value aspect and higher grades would indicate a greater choice of value aspect. There were 60 pairs of items used to measure individual differences on this instrument. The value structure instrument required subjects to choose between two alternatives on each item. The instrument has 6 domains, each domain has 20 items, so the highest score will be 60 and the lowest will be 0. This instrument was developed by Attoum and Khasawneh (1999) based on the instrument of Allport, Vernon and Lindzey (1952). The instrument has 6 domains of values: religious, social, political, economic, cognitive, and aesthetic.

The researchers verified the content validity of the instrument through systematic examination of the translated Arabic version in terms of representation in the behavior domain to be measured and language clarity. Two sociologists and two psychologists examined the Arabic language of the scale and the required changes were made accordingly.

For construct validity and reliability the researchers took 71 students randomly from UAEU. To achieve construct validity, Pearson's correlation coefficient was calculated between each item score and total score for each of the 6 values domains. The researchers agreed on two standards to confirm the validity of the item: correlation coefficients must be above .20 and the item must be significant at level (p = 0.05), (Robinson, Shaver & Wrightsman, 1991). The results confirmed that all items were valid (r =. 20 to .75).

To test the reliability of the instrument, two standards were used. Cronbach's alpha, and the test retest after two weeks on the total sample of 71 students. The results of reliability estimates are shown in Table 2.

RESULTS

To test the first question about how the above-mentioned domains of the UAEU community are distributed within the values structure, means (M) and standard deviations (SD) of the sample were calculated and ordered as shown in Table 3.

Data in Table 3 showed that religious values came first among UAEU students, followed by cognitive, political, aesthetic, social, and with economic values in the lowest scoring position. These results imply that religious values are the basic source of students' judgment in several social life issues, while cognitive and political values were common as a second source of judgment, aesthetic and social values were common as a third source while economic values were the least important source of judgment.

To test whether or not the differences between means of the domains were significant, a multiple paired t-test was used. The test results shown in Table 4 revealed a significant difference between the mean of religious values and the mean of cognitive, political, aesthetic, social, and economic values. Negative significant difference was found between the cognitive values mean compared with aesthetic, social, and economic means. Moreover, the results revealed that the political values mean differed signficantly from the means obtained for the aesthetic and economic domains. These differences showed that political values were more highly esteemed, while the difference between the political values and the social values means showed that social values were held in greater eteem than were politcal values. Finally, there was a significant difference between the aesthetic and economic values means - which showed that economic values were held in greater esteem than were aessthetic values.

To investigate the impact of gender on value structure, an F test was conducted and results are shown in Table 5.

Data in Table 5 reveal that among males, the religious values were the highest domain (M=37.59), followed by political values (M=27.46), followed by cognitive values (M=25.86), then economic values (M=24.95), aesthetic values (M=22.17), and social values scored lowest (M=21.60). Among females results were different, as the religious values scored highest (M=40.27), followed by cognitive (M=27.68), then political values (M=25.77), then aesthetic values (M=25, 00), then social values (M=24.71), and the economic values scored lowest (M=18.99). For the test of differences between males and females on value structure domains, results of the F test revealed that there are significant differences between means of males and females in all value domains. The mean differences in religious, social, aesthetic, and cognitive values showed that these domains were held in higher esteem by females, while the mean differences in economic, and political values showed that these domains here more highly esteemed by males.

To test if there was a difference between Emirates nationals and students who are non-Emiratis in their priority of values mean an F test was conducted to clarify the difference. These results are shown in Table 6.

Data in Table 6 illustrate that value structure among Emirates students was highest for the religious domain (M= 39.81), followed by cognitive (M=26.87), followed by the political (M=26.30), followed by the aesthetic (M=23.94), followed by the social (M=23.62) and the economic domain was the least important (M=20.98). Among the non-Emiratis, the religious domain came first (M=34.92), followed by the political domain (M=27.31), followed by the cognitive (M=27.23), then the economic domain (M=24.50), then the aesthetic domain (M=23.15), and the social domain was the least important (M=22.62). The F test results revealed that Emirates students significantly overemphasized religious values more than did non-Emiratis (F=17.49, p £ .000). The non-Emiratis students significantly overemphasized economic values (F=14.23, p £ .000).

DISCUSSION

The findings of this study show that religious values are the most important for the UAEU student community, followed by cognitive, political, aesthetic, social and economic values. In addition, comparison tests showed that significant mean differences exist between religious and all other value domains. There are also differences between cognitive and the following three values: aesthetic, social and economic. Moreover, there is a difference between political and the following three values: aesthetic, social, and economic. Similarly, there is a difference between economic value and both aesthetic and social value.

The above results about priority of values for UAEU students reflect an important indicator about students' cognitive style and their preferences about these issues and value domains. The results indicate that Islamic values ranked first with significant difference from all other values, which implies that Islam is still a main source of judgment among the UAEU student community. This result is not unexpected because it is consistent with UAEU philosophy which emphasizes Islamic tradition in modern life. This philosophy is thought to help students to develop talents to deal successfully with modern life expectations. It is worth mentioning that these results are in harmony with the studies of Albatsh and Abderhaman (1990) and Attoum and Khasawneh (1999) in some Jordans universities which indicated that religious values appeared as a central source of judgment and preference for college students.

The cognitive values domain was second as a source of judgment and preference, with significant mean differences from aesthetic, social and economic values. This result confirmed another dimension of behavior for students at UAEU, because cognitive values are considered as a main priority for the university and students, through the emphasis on scientific research and development of mental and cognitive abilities for students in several fields (Attoum & Khasawneh, 1999).

Political values ranked third in priority with significant mean difference from aesthetic, social and economic values. This result confirmed students' interest in politics at local, national and international levels with particular interest in the national issue of the Israeli-Arab conflict and Iraq problem (Attoum & Khasawneh, 1999).

The fourth level of priority was the aesthetic values, with significant difference from social values. This result reflected moderate interest by UAEU students in aesthetic issues, who have more interest in religious, cognitive and political values as sources of their judgment and preference, considering their age and stage (Almaghrabi, 1994).

Social values rated as a fifth level of priority for students, a result which reflects a profound social change in UAE society. This social change has created a kind of conflict between past and present customs and beliefs. The new UAE generation seems to experience this conflict, seems to adhere to individualism and is more open to new experiences than were UAE older generations. These results clarified the preference of UAE youth for new social life styles (Wraikat & Simadi, 2001).

Economic values came last in priority, a result that is very predictable because of UAE high living standards. As is very well known, the GNP in UAE is above $17,000.00 a year (UNDP 2002), and theoretically there are no poor people in this country, so money is not an issue.

Moreover, the results revealed that there is a significant difference between the means of males and females in all their values. These differences in political and economic values means were higher for males, while the differences of means in cognitive, religious, aesthetic and social values were higher for females. This result is consistent with the literature in general (Albatsh & Abderhanan, 1990; Attoum & Khasawneh, 1999; Attoum & Khasawneh, 1999; Omari et al, 1983; Shaw, 1981) and is logical in UAE society, because man, traditionally, is still responsible for woman economically.

In regard to the values structure among UAEU students in terms of citizenship, the results revealed that there is a significant mean difference between Emirates and non-Emirates students in some values. The difference was significant in religious and economic values. Religious values were higher among Emirates students, while economic values ranked high among non- Emirates students. This result is expected, because Emirates society is still conservative and places great emphasis on customs and traditions, while the non-Emirates individuals came to this country to gain income and to improve their lifestyle, so, it is to be expected that they will give more attention to money than Emirates people do (Wraikat & Simadi, 2001).

ENGLISH REFERENCES

Allport, G., Vernon, P. , & Lindzey, A. (1952). A study of values: Manual of directions (revised edition). Boston Houghton, Mifflinco.

Agun, K., Inamo, E., & Olcy, E. (2002). Value domains of Turkish adults and university students. Journal of Social Psychology, 142(3), 333-353.

Bond, M. H. (1988). Finding universal dimensions of individual variation in multicultural studies of values: The Rokeach and Chinese value surveys. Journal of Personality and Social Psychology, 55, 1009-1015.

Eagly, A., & Chaiken, S. (1993). The psychology of attitudes. Fort Worth, USA: Harcourt Brace Publishers

Feather, N. T. (1991). Human values, global self-esteem, and belief in a just world. Journal of Personality, 59, 83-107.

Inglehart, R. (2000). Globalization and postmodern values. The Washington Quarterly, 23(1), 215-228

Kashima, Y., Kim, U., Gelfand, M. J., Yamaguchi, K. Y., Vchoi, S. C., & Yuki, M. (1995). Culture, gender and self: A perspective from individualism collectivism research. Journal of Personality and Social Psychology, 69, 925-937,

Korathwohl, D. (1994). Taxonomy of education objectives. New York: David Mekay

Mouly, J. (1982). Psychology for teaching. London: Allyn and Bacon, Inc.

Newcomb, T. (1965). Social psychology. London: Routledge & Kegan Paul Ltd.

Robinson, G. B. Shaver P. R., & Wrightsman, L. S. (1991). Measures of personality and social psychological aptitude. California 92101: Academic Press, Inc.

Rokeach, M. (1973). The nature of human values. New York: The Free Press.

Schwartz, S.H. (1992) Universals in the content and structure of values. In M. Zanna (Ed.), Advances in experimental social psychology, 25, 1-62. San Diego, CA: Academic Press.

Shaw, M. (1981). Group dynamics: The psychology of small group behavior, (3rd ed.). New York: McGraw-Hill Book Company.

United Nations Report (UNDP), (2001).

Wojciszke, B. (1997). Parallels between competence versus morality-related traits and individualistic versus collectivistic values. European Journal of Social Psychology, 27, 245-256.

Wolman, B. (1975). Dictionary of behavioral science. London: The Macmillan Press.

Wraikat A., & Simadi, F. (2001). The Arabian modern man. International Journal of Sociology and Social Policy, 21(11), 41-56.

ARABIC REFERENCES

Abu Neel, M. (1985). Social psychology, Part 1. Dar Alnahdah, Beirut.

Albatsh & Abderhanan. (1990). Value structure of university of Jordan students. Dirastat, 17(3), 92-136.

Almaghrabi, K. (1994). Behavior of individual and group in the organization. Dar Al fikir: Amman.

Attoum, A., & Khasawneh, A. (1999). Value matrix of Al-albayt university students. Al Manor, 1(1), 3-54 (Arabic Version).

Khalefeh, A. (1992). Promotion of values: A psychological study. Higher Council of Arts and Culture, Kuwait.

Omari, K., Jaradat, D., & Nashawati, A. (1983). Value structure of Yarmouk University. Abhath Al Yarmouk, 1(1), 119-142.

United Arab Emirates University. (2003). Statistical Report, First Term (2002-2003), Registration Department, UAEU.

Zahran, H. (1982). Values in education. Alam AlMarefah, (2nd ed.), Kuwait.

FAYEZ A. SIMADI

United Arab Emirates University and Jordan University of Science and Technology

MOHAMMAD A. KAMALI

United Arab Emirates University

Fayez A. Simadi, PhD, Associate Professor of Social Psychology, Department of Sociology, Faculty of Humanities and Social Scienxes, United Arab Emirates University, Ai-Ain, United Arab Emirates and Jordan University of Science and Technology, Irbid, Jordan. Mohammad A. Kamali, PhD, Assistant Professor of Psychology, Department of Psychology, Faculty of Humanities and Social Sciences, United Arab Emirates University, Al-Ain, United Arab Emirates.

Appreciation is due to reviewers including: Chau-Kui Cheung, Department of Applied Social Studies, City University of Hong Kong, Tat Chee Avenue, Kowloon, Hong Kong; Email: ; Mousa Alnabhan, Associate Professor, Methodology, Measurement and Assessment, Psychology Department, United Arab Emirates University, P.O. Box 1777, Al-Ain, UAE; Email: ; Dr. Adnan Atoum, Department of Psychology, Yarmouk University, Irbid, Jordan; Email: .

Please address correspondence to: Mohammad A. Kamali, Department of Psychology, College of Humanities, United Arab Emirates University, P.O. Box. 17771, Al-Ain, United Arab Emirates (UAE). Phone: (Office) +9713-7064773, (Mobile) +97150-6232920; Fax: +9713-7671705; Email:

Copyright Society for Personality Research, Incorporated 2004
Provided by ProQuest Information and Learning Company. All rights Reserved

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