• Journal of Genetic Medicine
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• v.4 no.1
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• pp.22-37
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• 2007

The autosomal dominant spinocerebellar ataxias (SCAs) are a group of neurodegenerative diseases, clinically and genetically heterogeneous, characterized by degeneration of spinocerebellar pathways with variable involvement of other neural systems. At present, 27 distinct genetic forms of SCAs are known: SCA1-8, SCA10-21, SCA23, SCA25-28, DRPLA (dentatorubral-pallidoluysian atrophy), and 16q-liked ADCA (autosomal dominant cerebellar ataxia). Epidemiological data about the prevalence of SCAs are restricted to a few studies of isolated geographical regions, and most do not reflect the real occurrence of the disease. In general a prevalence of about 0.3-2 cases per 100,000 people is assumed. As SCA are highly heterogeneous, the prevalence of specific subtypes varies between different ethnic and continental populations. Most recent data suggest that SCA3 is the commonest subtype worldwide; SCA1, SCA2, SCA6, SCA7, and SCA8 have a prevalence of over 2%, and the remaining SCAs are thought to be rare (prevalence <1%). In this review, we highlight and discuss the SCA7. The hallmark of SCA7 is the association of hereditary ataxia and visual loss caused by pigmentary macular degeneration. Visual failure is progressive, bilateral and symmetrical, and leads irreversibly to blindness. This association represents a distinct disease entity classified as autosomal dominant cerebellar ataxia (ADCA) type II by Harding. The disease affectsprimarily the cerebellum and the retina by the moderate to severe neuronal loss and gliosis, but also many other central nervous system structures as the disease progresses. SCA7 is caused by expansion of an unstable trinucleotide CAG repeat in the ATXN7 gene encoding a polyglutamine (polyQ) tract in the corresponding protein, ataxin-7. Normal ATXN7 alleles contain 4-35 CAG repeats, whereas pathological alleles contain from 36->450 CAG repeats. Immunoblott analysis demonstrated that ataxin-7 is widely expressed but that expression levels vary among tissues. Instability of expanded repeats is more pronounced in SCA7 than in other SCA subtypes and can cause substantial lowering of age at onset in successive generations termed ‘anticipation’ so that children may become diseased even before their parents develop symptoms. The strong anticipation in SCA7 and the rarity of contractions should have led to its extinction within a few generations. There is no specific drug therapy for this neurodegenerative disorder. Currently, therapy remains purely symptomatic. Cellular models and SCA7 transgenic mice have been generated which constitute valuable resources for studying the disease mechanism. Understanding the pathogenetic mechanisms of neurodegeneration in SCAs should lead to the identification of potential therapeutic targets and ultimately facilitate drug discovery. Here we summarize the clinical, pathological, and genetic aspects of SCA7, and review the current understanding of the pathogenesis of this disorder. Further, we also review the potential therapeutic strategies that are currently being explored in polyglutamine diseases.

• Nutrition Research and Practice
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• v.10 no.1
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• pp.56-66
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• 2016

BACKGROUND/OBJECTIVES: Despite successes recorded in combating iodine deficiency, more than 2 billion people are still at risk of iodine deficiency disorders. Rural landlocked and mountainous areas of developing countries are the hardest hit, hence the need to explore and advance novel strategies such as biofortification. SUBJECTS/METHODS: We evaluated adoption, purchase, and consumption of iodine biofortified vegetable legumes (IBVL) using the theory of protection motivations (PMT) integrated with an economic valuation technique. A total of 1,200 participants from three land-locked locations in East Africa were recruited via multi-stage cluster sampling, and data were collected using two, slightly distinct, questionnaires incorporating PMT constructs. The survey also elicited preferences for iodine biofortified foods when offered at a premium or discount. Determinants of protection motivations and preferences for iodine biofortified foods were assessed using path analysis modelling and two-limit Tobit regression, respectively. RESULTS: Knowledge of iodine, iodine-health link, salt iodization, and biofortification was very low, albeit lower at the household level. Iodine and biofortification were not recognized as nutrient and novel approaches, respectively. On the other hand, severity, fear, occupation, knowledge, iodine status, household composition, and self-efficacy predicted the intention to consume biofortified foods at the household level; only vulnerability, self-efficacy, and location were the most crucial elements at the school level. In addition, results demonstrated a positive willingness-to-pay a premium or acceptance of a lesser discount for biofortification. Furthermore, preference towards iodine biofortified foods was a function of protection motivations, severity, vulnerability, fear, response efficacy, response cost, knowledge, iodine status, gender, age. and household head. CONCLUSIONS: Results lend support for prevention of iodine deficiency in unprotected populations through biofortification; however 'threat' appraisal and socio-economic predictors are decisive in designing nutrition interventions and stimulating uptake of biofortification. In principle, the contribution is threefold: 1) Successful application of the integrated model to guide policy formulation; 2) Offer guidance to stakeholders to identify and tap niche markets; 3) stimulation of rural economic growth around school feeding programmes.

• The Journal of the Acoustical Society of Korea
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• v.40 no.3
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• pp.201-212
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• 2021

The present study was conducted as a pilot investigation to suggest the standards of acoustic performance for classrooms suitable for incomplete hearing people such as children under 9 years of age. Subjective evaluations such as questionnaire and speech intelligibility test were conducted to 264 students at two elementary schools in Cheong-ju in order to analyze the characteristics of the listening environment in the classrooms of the lower grades in elementary school. The survey was undertaken with a total of 264 students at two elementary schools in Cheong-ju, and investigated their satisfaction with the classroom listening environment. As a result, students responded that the most helpful information type for understanding class content is the voice of teacher. In addition, the volume of the current teacher's voice is normal, and the level of clarity is highly satisfactory. As for the acoustic performance of the classroom, the opinion that the noise was normal and the reverberation was very short was found to be dominant in overall satisfaction with the listening environment. Meanwhile, as a result of speech intelligibility test using the word list selected for the lower grade students of elementary school, it could be inferred that the longitudinal axis distance from the sound source in the case of 8-year-olds is a factor that affects speech recognition.

• Asia-pacific Journal of Multimedia Services Convergent with Art, Humanities, and Sociology
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• v.7 no.7
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• pp.69-78
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• 2017

This study researches and analyses the state of the learning achievement assessment of the basic curriculum of the special education in the viewpoint of the individualized education pursuing for the learning and growing of each person, and discusses the political plan for support on the basis of the results. The object of study was 129 special school teachers working in B metropolitan city applying for the basic curriculum. For the measurement, the tool developed by the examination of FGI and the specialist on the basis of test research related to the evaluation tools of the learning achievement assessment for the special education was used. As the result of analysing the real state, to evaluate the individual learning and growing in the aspect of the plan, criteria, period of the evaluation, the special education teachers were approaching in detailed and precise quality, and guiding and using the evaluation results in the various ways in the prospective of the curriculum. The recognition on the sub-element in the each area of the measurement tools showed the difference by gender, age, career, academic achievement, school class, foundation type. On the basis of the results of the real state, as the political support plan, some advice were proposed, which were the expansion of the autonomy of making the curriculum achievement standard and the evaluation system, improving the field and system of the learning achievement assessment, establishing the support system to strengthen the evaluation competences in accord with the teachers' background variables, etc.

• The korean journal of orthodontics
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• v.29 no.1 s.72
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• pp.51-72
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• 1999

In orthodontic field, it is very important to understand the normal growth. Such an understanding can be derived from observation of normal growth in various samples from childhood to adulthood, and this builds a foundation from which growth abnormality or variation can be defined. Thus, a broad data collection of normal children, as well as basic study reviewing such data become necessary. The relationship between the mean values of cephalometric measurements in Growth and Development was studied among the groups(boys and girls) of Korean chidren from the ages 6-years to 16-years. 220 boys 170 girls were chosen as subjects : Cephalometric X-ray were taken for 3 years and hard tissue analysis on McNamara and Ricketts Analysis which was divided into measurements of 5 parts(Cranial base, Cranial base and Maxilla & Mandible, Maxilla and Mandible, Mandible, Dental measurements). The relationship of craniofacial growth was studied. The following conclusions were obtained: 1. There were statistically significant differences in anterior cranial base between the two sexes of 14 and 15-year grouips. 2. In comparison of growth amounts among different age groups, statistically singnificant difference in Posterior facial height exhisted among $10\~11,\;12\~13\;and\;14\~15$ year-old interval groups. This pattern increased with aging. 3. Na perpendicular to A showed earlier growth peak in females(11-12years) than males(12-13years). When horizontal measurements of point A and Pogonion are compared, mandibular growth appeared to be greater. 4. Maximum growth peak of cranial base and mandible was earlier in females (11-12years) than males(13-14 years). 5. Upper central incisor flared out with aging, and there were increases in lower incisor to A-Pg, and lower incisor inclination There was significant difference between the two sexes in 10-year-old group.

• Tuberculosis and Respiratory Diseases
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• v.65 no.4
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• pp.269-276
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• 2008

Background: Although the prevalence of tuberculosis infections (PTBI) is one of the basic epidemiologic indices, no survey has been carried out since 1995 because the nation-wide tuberculosis prevalence survey was changed to a surveillance system. Subjects without a BCG scar are examined in a tuberculin survey. However, it is very difficult to select these subjects under high vaccination coverage. It is important to evaluate the impact of BCG vaccinations on the tuberculin response and estimate the PTBI regardless of the BCG vaccination status. Methods: A nation-wide, school-based cross-sectional tuberculin survey was carried out among first graders in elementary school in 2006. A total of 5,148 children in 40 schools were selected by quota sampling. Tuberculin testing with 0.1 ml of two tuberculin units of PPD RT23 was carried out on 4,018 children. The maximum transverse diameter of induration was measured 48 to 72 hours later. The presence of a BCG scar was checked separately. Results: There were no BCG scars in 6.3% of the subjects. The mean induration size of tuberculin testing was $3.7{\pm}4.4mm$, which included 1,882 (46.8%) subjects with an induration size of 0 mm. The PTBI was 10.9% (439 subjects) using a cut-off point of ${\geq}10mm$ (conventional method). The annual risk of tuberculosis infections (ARTI) was 1.9% when the mean age of the subjects was assumed to be 6 years. There was no difference in the PTBI according to the presence or absence of a BCG scar [11.2% vs 7.6% (OR: 1.54, 95% CI: 0.98~2.43)]. Using a mirror image technique with 16 mm as the cut-off point, the PTBI and ARTI had decreased to 2.4% and 0.4% respectively. Conclusion: PTBI and ARTI, as estimated by conventional methods, appear to be high among BCG vaccinated children. A mirror image technique is more suitable for estimating the indices in a country with an intermediate burden of tuberculosis than the conventional method.

• Journal of the Korean Academy of Child and Adolescent Psychiatry
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• v.9 no.1
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• pp.54-66
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• 1998

Objectives：This study was to investigate the impact of parental psychiatric disorder on offspring's depression, anxiety, self concept, perception of familial relationship compared with offspring of normal control. In offsprings of parents with psychiatric disorder, this study explored whether their psychopathology, self concept, and perception of familial relationship were influenced by parent’s sex, onset time of parent’s psychiatric disorder and parent’s psychiatric diagnosis. Methods：52 offsprings aged 10-18 years of 39 psychiatric outpatient were surveyed from June, 1997 to April, 1998 and completed several questionnaaire, including Korean from of the Family Environment Scale, Korean form of the State-Trait Anxiety Inventory for Children, Korean form of Kovac’s Children’s Depression Inventory, and Korean form of Piers-Harris Children’s Self-Concept Scale. Their score was compared with offsprings’ of normals. In offsprings of parents with psychiatric disorder, they were compared according to parent’s sex, onset time of parent's psychiatric disorder and parent’s psychiatric diagnosis. Results：The results were as follows：1) Offsprings of parents with psychiatric disorder reported higher level of state anxiety and lower level of the FES expressive subscale than offsprings of normals(p<0.05). But they reported higher level of PHCSCS intellectual & school status subscale and popularity subscale than normals(p<0.05). 2) There were no differences in anxiety, depression, self concept, and perception of familial relationship between patient’s sex. 3) Offsprings less than 3 years old when parent’s psychiatric disorder had developed showed higher level of trait anxiety and lower level of FES control subscale than offsprings more than 3 years old (p<0.05). 4) There were no diferences in anxiety, depression, self concept, and perception of familial relationship between patient’ diagnostic groups(schizophrenia spectrum disorder-mood disoderneurosis). Conclusion：The finding indicated that self reported scale of anxiety and depression showed no significant difference between offsprings of psychiatric patients and offsprings of normals. In offsprings of parents with psychiatric disorder, parent’s sex and psychiatiric diagnosis had no influence on offspring’s psychopathology. But the offspring’s age(before 3 years old) when the parent’s psychiatriric disorder developed had influence on higher level of offspring’s trait anxiety. For further high risk group study, direct interview and evaluation of parent-child agreement or teacher-child agreement will be needed in longitudinal study.

• Journal of the korean academy of Pediatric Dentistry
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• v.28 no.3
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• pp.412-420
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• 2001

In the growing children with normal occlusion and class III malocclusion who need the early treatment to be helped to diagnose and to set up treatment plan by calculating the mean values of the lateral cephalometric measurements, cephalometric measurements by McNamara alalysis were achieved and compared on both the 84 elementary school children with normal occlusion and 83 class III malocclusion children at the age of 7 to 9 in Kwangju city and the results were as follows: 1. On the boys group, between normal occlusion and class III malocclusion, Effective maxillary length, Maxillomandibular differential, Facial axis, Nasion perpendicualr to pogonion, Point A perp to Mx 1, and A-Pog line to Mn 1 showed significant differences(p<0.05). 2. On the girls group, between normal occlusion and class III malocclusion significant differences were shown in Effective Maxillary length, Maxillomandibular differential, Mandibular plan angle, Nasion perpendicualr to Point A, Point A perp to Mx 1, A-Pog line to Mn 1(p<0.01). 3. There were no significant differences between normal occlusion and class III malocclusion of boys group but significant differences between them of girls group in Effective mandibular length(P<0.01). On the boys and girls group, Effective maxilla length of class III malocclusion was shorter than that of normal occlusion. 4. There were no significant differences between normal occlusion and class III malocclusion both in boys and girls at Anterior lower facial height. 5. There were significant differences between boys and girls both in class III malocclusion and normal occlusion at Anterior lower facial height and Mandibular plane angle(p<0.01).

• Journal of agricultural medicine and community health
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• v.8 no.1
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• pp.44-65
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• 1983

As a part of the epidemiological studies of clonorchiasis, this study was conducted to evaluate the current endemicity and the natural transition of the Clonorchis infection in Gimhae Gun and delta area a high endemic area in Korea in recent years, prior to the introduction of praziquantel which will eventually influence the status of the prevalence. The data obtained in this study in 1983 were evaluated for natural transition of the infection in comparison with those obtained 16 years ago in 1967 by the author(Kim, 1974). The areas of investigation, villages and schools surveyed, methods and techniques used in this study were the same as in 1967, except for the contents of the questionnaire for raw freshwater fish consumption by the local inhabitants. 1) The prevalence rate of clonorchiasis in the general population of the villages was 48.1% on the average out of a total of 484 persons examined. The average of those of the riverside-delta area was 65.2% and 43.0% in the inland area. Among the schoolchildren, the prevalence rate was 8.2% on the average out of a total of 1,423 examined. By area, the prevalence rate was 10.8% in the riverside delta area and 2.8% in the inland area. By sex, difference in the prevalence was seen only in the inhabitants of the inland area showing 52.4% in the male and 33.5% in the female. 2) In the natural transition of the infection, the prevalence rate among the inhabitants has decreased from 68.8% in 1967 to 48.1% in 1983, and in the schoolchildren from 56.4% in 1967 to 8.2% in 1983. The reduction rate was higher in the riverside-delta area than in the inland area. 3) In the prevalence rate by age, 11.9% was first seen in the 5-9 age group and the rate gradually increased up to 75.0% in the 50-59 age group. By sex, the rate was higher in the male than in the female in the 20-29 age group and over. 4) In the natural transition of the prevalence rate by age, the reduction rate of the infection during the past 16 years was greater in the younger age groups up to the 40-49 age group and reached the same level in the age group 50-59. Reduction was seen again in the age group over 60s. By sex, the reduction rate was greater in the female than in the male in the 20-29 age group and over. By area, the reduction rate was greater in the riverside delta area than in the inland area, particularly in the young age groups. 5) In the intensity of the infection among the cases, the mean egg out-put per mg feces per infected cases(EPmg) in the inhabitants was 6.3. EPmg of those of the river-side-delta area was 15.4 and that of the in-land was 2.8. On the other hand, in the schoolchildren, EPmg was 3.2, and no difference was seen between the two areas, the river-side-delta area and the inland area. 6) In the transition of the intensity of the infection by area, EPmg among the inhabitants inexplically increased from 7.8 in 1967 to 15.4 in 1983. This was probably caused by uneven specimen collection in the process of sampling the population. EPmg of the inhabitants in the inland area and those of the schoolchildren of both riverside delta and inland areas showed a similar decrease in the past 16 years. 7) The intensity of the infection by age showed a relatively low level in the 20-29 age group and below, and EPmg 5.1-9.5 was seen in the 30-39 age group and over. Sex, Epmg was 5.8 in the male and 4.7 the female. By in 8) In the transition of the intensity of the infection, EPmg decreased from 6.2 in 1967 to 5.4 in 1983. By age, in contrast to the figures of 1967 in which EPmg gradually increased with some fluctuation from 1.1 in the 0-4 age group to peak 10.5 in the 50-59 age group, in 1983 lower intensity of the infection was seen in the age group from 10-14 to 20-29 with the EPmg range of 0.6-2.7. 9) In the distribution of the clonorchiasis cases by the range of EPmg value, 43.2% of the cases were in 0.1 0.9 and 34.6% in 1.0-4.9. As a whole by cumulative percent, 44.6% of them were under 0.9 as light infection and 86.1% of them under 9.9 up to moderate infection. By sex, no difference was seen in Epmg. 10) In the transition of the distribution by the range of Epmg, the cases were distributed up to the range 80.0-99.9 in 1967 and to 60.0-79.9 in 1983. By cumulative percent, in the range of 0.1-0.9 and less, light infection, 34.3% of them were distributed in 1967 and 44.6% in 1983 with about 10% increase. In the range of 5.0-9.9 and less, up to moderate infection, 83.2% in 1967 and 86.1% in 1983 of the cases were seen, respectively. 11) The practice of raw freshwater fish consumption among the inhabitants seems to have decreased in recent years. Those who admitted to raw freshwater fish consumption in the last two years among the infected inhabitants were 59.3%, although 86.8% of them professed to have experience with raw freshwater fish consumption. 31.7% of those who have had experience of the raw freshwater fish consumption denied any further consumption in recent years. From an interview of 543 school-children, 24.1% of them admitted to an experience of raw freshwater fish consumption. However, those who have practised in the past two years comprized 17.9%. Those who denied raw freshwater fish consumption in recent years among those who had such experience were 26.0% out of 131 interviewed. The rate of raw freshwater fish consumption in both inhabitants and schoolchildren were higher in the male than in the female. On the contrary, the rate of those who did not practise in recent years among those who had experience of raw freshwater fish consumption was higher in the female than in the male. 12) The major reason for the reduction of raw freshwater fish consumption among the local inhabitants was the risk of the fluke infection. However, it has become apparent that such change of taste has resulted from water pollution impact which has affected throughout the areas of the freshwater systems in this locality since last several years. 13) In animal survey, Clonorchis infection was seen in 14.8% of 88 dogs examined and 3.7% of 27 house rats examined. It was noted that populations of dogs and cats have increased in the villages surveyed. Although the prevalence rate was lower in the present survey than those of 1967, the significance of the animals as the reservoir host has not changed. 14) Prevalence rate of Clonorchis infection by cercariae in the first intermediate host, Parafossarulus manchouricus, was 0.6% out of 517 snails examined. The infection rate was lower in comparison with 2.3% out of 2,124 examined in 1967. Moreover, sharp decreases in number and distribution of the intermediate host snails in many watershed areas of the huge freshwater systems in this locality seemed to reduce transmission of Clonorchis in connection with the intermediate host stage of its life cycle. 15) Clonorchis infection in the second intermediate fish hosts was relatively low. The mean number of Clonorchis metacercaria per fish in Pseudorasbora parva was 517 in 1983, whereas it was 1943 in 1968 through 1969. Environmental water pollution has also caused the decreased fish population density in these areas, and this has also apparently affected to the practice of raw freshwater fish consumption among the local inhabitants. 16) In conclusion, endemicity of Clonorchis infection in Gimhae Gum and delta area of the Nagdong River has sharply decreased during the past 16 years. The major cause of the regressive transition of the infection was the water pollution of the land water systems of this locality. The pollution has upset the ecosystems comprizing of the intermediate hosts of Clonorchis in many areas, and also affected to a significant extent to the discontinuance of the local inhabitants for raw freshwater fish consumption.

• Korean Journal of Clinical Pharmacy
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• v.15 no.1
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• pp.9-20
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• 2005

Cholestatic liver disease is a frequent complication of prolonged parenteral nutrition, especially in premature infants. Numerous factors have been cited as contributing to TPN associated cholestasis. However the exact etiology remains obscure. Ursodeoxycholic acid (UDCA) has been reported to be beneficial far children and adults with various chronic cholestatic liver disease. The aim of this prospective, randomized, double-blind, placebo-controlled study was to determine the preventive effects of UDCA administration during TPN. Seventeen pediatric patients (8 boys and 9 girls) undergoing TPN were assigned randomly to two groups, UDCA and placebo group. UDCA group (n=9) received 15 mg/kg/day UDCA and placebo group (n=8) received 15 mg/kg/day placebo enterally during the TPN period. Liver function tests (total bilirubin, aspartate aminotransferase, alanine aminotransferase, alkaline phosphatase) were per-formed before TPN and weekly or three times a week. The patients' weights, complete blood count, composition of TPN, and the infusion rate of TPN and lipid were monitored everyday. Calcium and phosphate were monitored twice a week. Between the UDCA and placebo groups, there were no differences in weight at the onset of TPN, birth weight, duration of TPN, respiratory distress syndrome associated with prematurity, age at the onset of TPN, gestational age, the number of days the patients received antibiotics, the number of patients received enteral nutritions and the composition of TPN. In contrast, there was a significant difference between the UDCA and placebo groups in alanine aminotransferase levels during TPN. It doesn't seem that UDCA administration during TPN correlates directly with improvement of liver function. But the preventive administration of UDCA may be effective in reducing liver enzyme, alanine aminotransferase and has no adverse effects.