Chylomicron retention disease, also known as Anderson's disease, is a rare hereditary hypocholesterolemic disorder, recessive inherited, characterized by nonspecific symptoms as abdominal distension, steatorrhea, and vomiting associated with failure to thrive. We describe a patient with failure to thrive, chronic diarrhea and steatorrhea who the diagnosis of chylomicron retention disease was established after several months of disease progression. The genetic study confirmed a homozygosity mutation in SAR1B gene, identifying a mutation never previous described [c.83_84delTG(p.Leu28Argfs*7)]. With this case report the authors aim to highlight for this very rare cause of failure to thrive and for the importance of an attempting diagnosis, in order to start adequate management with low fat diet supplemented with fat-soluble vitamins, reverting the state of malnutrition and avoiding possible irreversible and desvantating complications.
The pathophysiology and fundamental etiologic mechanism of inflammatory bowel disease (IBD) is not well understood even though therapeutic regimens and drugs are rapidly evolutionary. IBD has complicated connections with genetic, immunologic, gut microbial, environmental, and nutritional factors. It is not clearly well known to the physicians how to feed, what nutrients are more helpful, and what food to be avoided. This review discusses the issues of growth and important nutritional concerns in the management of IBD in childhood.
The purpose of this study is to evaluate the nutritional status of protein and lipids in the patients with alcoholic liver disease, to find an effective way of dietary management for patients with alcoholic liver disease and to obtain the materials for nutritional education for alcoholics. The subjects consist of 80 patients with alcoholic liver cirrhosis(ALC) and 12 patients with alcoholic fatty liver(AFL). The control group included 57 alcoholics without liver disease(A), 32 patients with viral liver cir rhosis(VLC) and 194 normal(NL). Biochemical evaluation of nutritional status was performed by ana lyzing the profiles of protein and lipids in blood samples. The results are summarized as follows: 1) The frequency of subjects below the normal range of serum total protein was 26.3% in ALC, 16.7% in AFL and 3.5% in A. Serum albumin was lower in 41.3% of ALC. 2) The alcoholics without liver disease showed significantly higher level of serum triglycerides, total cholesterol and LDL cholesterol than the other groups. The alcoholic subjects had lower HDL cholesterol than normal subjects. Overall, the protein and lipids status of the alcoholic subjects in this study was evaluated to be very poor on the basis of biochemical assessments. The results suggest that alcohol abuse and poor dietary intake could cause malnutrition. An extensive nutritional education should be emphasized for the alcohol consuming population. High quality of protein and other dietary intakes from early stage of the disease may be effective in nutritional therapy for the patient with alcoholic liver disease.
Objectives : The purpose of this descriptive observational study was to identify the factors influencing the nutritional status of patients with liver cancer receiving transarterial chemoembolization(TACE) Methods : A total of 100 participants were enrolled in this study. Data were collected and descriptive statistics and logistic regression were performed. Results : Among participants, 41.0% had a risk of malnutrition and 59.0% had a normal nutritional status. Statistically significant differences between the normal and risk groups were found for the following factors: occupation; economical status; regular exercise; underlying disease; adverse events right after TACE; current adverse events; duration after TACE; depression; and self-care performance. Underlying disease(OR=5.134, p=.005) and self-care performance(OR=0.931, p=.032) had statistically associated with nutritional status. Conclusions : The findings suggest that underlying disease and self-care performance influence the nutritional status among liver cancer patients receiving transcatheter arterial chemoembolization(TACE).
Purpose: This study was conducted to explore the mediating effect of nutritional status on the relationship between symptom experience and functional status of patients with Chronic Obstructive Pulmonary Disease (COPD). Methods: A total of 141 COPD patients visiting D hospital and I hospital in B city were enrolled in this study. Data were collected from January 2017 to July 2017. Outcome variables were measured by Mini Nutritional Assessment Short-Form (MNA-SF) for nutritional status, The Memorial Symptom Assessment Scale (MSAS) for symptom experience, and The Functional Performance Inventory Short Form (FPI-SF) for functional status. The data were analyzed with descriptive statistics, Pearson's correlation, and path analysis using SPSS/WIN 21.0 and AMOS 25. Results: There was a significant negative correlation between symptom experience and nutritional status (r=-.61, p<.001), a significant negative correlation between symptom experience and functional status (r=-.40, p<.001), and significant positive correlation between nutritional status and functional status (r=.47, p<.001). Symptom experience had indirect effects on functional status through nutritional status. Conclusion: For enhancing functional status in COPD patients, it is necessary to develop nursing intervention programs to enhance symptom management as well as nutritional status.
This study was intended to evaluate the overall effects of nutritional education on adults having two or more symptoms of chronic degenerative disease. A nine week nutritional education program was provided for 65 adults with chronic diseases. We assessed the changes in dietary knowledge, eating behavior and socio-psychological factors. When we evaluated the nutrient intakes of the subjects, their energy intake was 79.4% of the Korean Recommended Dietary Allowances (RDA). Their dietary intake of other nutrients was also below the RDA level except for Vitamin C. Their knowledge of dietary therapy was slightly improved after the implementing of nutritional education. The dietary behavior of ‘night snacks before sleep’was significantly improved. While the overall fear due to disease was significantly increased, self-efficacy was not improved. Self-efficacy for eating “three regular meals” and “choosing fruit, vegetable and grain” were significantly decreased. Family support for “buying food which is good for my health” was also significantly increased, whereas “advises me to eat appropriate foods for health” was decreased. Biochemical analysis indicated that blood levels of triglyceride, cholesterol and blood pressure improved after nutrition education. Therefore, we concluded that nutritional education program for people with chronic degenerative diseases could change the diet therapy knowledge, dietary behavior, and the fear due to disease, support from family and behavior intention toward the direction to improve the chronic disease condition. However, it did not improve self-efficacy. Our study also indicated that nutritional education strategies to improve self-efficacy should be an important aspect in a long term education plan for patients to establish desirable eating habits.
Assessment regarding the nutritional status of Parkinson's disease (PD) patients is important because their nutritional status influences the outcome of disease. The purpose of this study investigated the dietary habits, dietary quality, and quality of life for Korean PD patients according to their nutritional status. Seventy PD patients were recruited from K University Hospital (KMC IRB#0918-07) from February 2010 to October 2011. To diagnose PD, the United Kingdom Parkinson's Society Brain Bank standards were used by a neurology physician. The participants were interviewed and assessed using various tools, including a general questionnaire, anthropometrics, questionnaires for dietary habits, dietary diversity score (DVS), dietary variety score (DVS), diet quality index-international (DQI-I), beck depression inventory (BDI), the Korean version of mini mental state examination (K-MMSE), and 36-item short-form health survey (SF-36). The results of the study were as follows: 1) Most of the PD patients (69.0%) were at risk of malnutrition, and 8.5% and 22.5% of the subjects were malnourished and well-nourished, respectively. 2) Clinical symptoms (swallowing and chewing difficulties) were obvious, and the quality of diet (DDS, DVS, DQI-I) was low in the malnourished group (p < 0.01). 3) The malnourished group had significantly lower scores regarding SF-36 (quality of life) as well as that of cognitive function (p < 0.05). 4) The nutritional status of PD patients was significantly correlated with their depression (p < 0.05) and quality of life (p < 0.01). These results indicate that appropriate nutritional management will improve the nutritional status and quality of life as well as the cognitive function in Parkinson's disease patients.
Objectives: This study is to investigate and to test the overall level of nutritional knowledge. attitude. practice and health status of the residents in rural area. Methods: The interview survey was performed in March 2004 with structured questionnaires to 510 respondents of the residents who lived in Muan-Gun of Jeonnam province. The questionnaire was the abridged Scale of the Nutritional Knowledge Attitude Practice(SNKA) and health status as the morbidity, subjective health status, health management, and health examination. The covariate, F-test or t-test, and Chi-squire method were used for some of the cross-sectional data. Results: Average weight of nutritional knowledge was $10.64{\pm}5.98$ by SNKA of the 20 items(0-20). Average weight of nutritional attitude practice was $64.59{\pm}7.72$ by SNKA of the 20 items(20-80). The level of the nutritional knowledge was lower than the level of the nutritional attitude practice. 77.3% of the respondents have been health management, 49.6% of the respondents have been health examination. A majority of the respondents(31.3%) had disease, while 13.5% of the respondents had chronic disease, and 17.8% of the respondents had acute disease. The level of nutritional knowledge and general characteristics was positively correlated while the relationships were positive between nutritional knowledge and health status, and between nutritional attitude practice and health status. Conclusion: These results suggested that education programs of the nutritional knowledge were necessary for the residents of rural area. And behavior change of the nutritional knowledge were necessary for the residents of rural area. Further research would be required to specify the necessities and operation researches.
The purpose of this study was to analyze the nutritional status and dietary habits in predialysis patients of chronic renal failure(CRF). The patients group was composed of total 35 persons with chronic renal failure(diabetes exclusion), male 20, female 15 who were treated in the kidney internal department and the control group also composed of 35 persons, male 18, female 17 who were classified as normal by the medical examination. Their dietary habits, nutritional status and nutritional knowledge were investigated from two general hospitals in Inchon, middle of this year 2002. There were 31.4% of low weight patients (BMI below 20), 77.1% of anemia patients (serum hemoglobin below 12g/㎗), 6S.6% of hypertension patients with diastolic blood pressure over 90mmHg, 80% with systolic blood pressure over 140mmHg, 20% of hypercholesterolemia patients (serum cholesterol over 230mg/㎗), and 22.9% of hyperlipemia patients (serum triglyceride over 200mg/㎗). The cardiovascular disease seemed to be caused by the abnormality of lipid metabolism. The possibility of the bone disease was shown from patients of hyperphosphatemia (serum phosphorus over 4.7mg/㎗, 22.9%) and hypocalcemia (serum calcium below 8.4mg/㎗, 25.7%). Intake of insufficient calories which was caused by the lack of appetite affected on the nutritional status. The intake of most nutrients was not significantly different from the RDA for Koreans. Consequently, the patient groups took a lot of salt even after the diagnosis of CRF. But patients ate 6.lg of salt which were more than the recommended amount 2∼4g for patients with CRF. The patient groups, who had the experiences of nutritional counselling, had significantly higher nutritional knowledge related to CRF than control group. Unfortunately, patients could not have enough chances for nutritional counselling by the nutritionist even though they needed the nutritional informations and dietetic treatments. The continuous research is expected with regard to the detail plan for the improvement of nutritional support and the nutritional counselling because it is important to decide the requirements of nutrients for patients with kidney disease, considering the kidney function and status of nutrition.
This study was conducted to compare the nutritional status, nutrient intakes, and chronic disease risks of Chinese elderly people. MNA (Mini Nutritional Assessment) developed for elderly people was used to determine their nutritional status. In this study, participants consisted of 148 urban residents aged 65 years and over residing in three cities in Shandong Province, China. Participants were 67 (45.3%) men and 81 (54.7%) women, and average age was 72.8 years. According to MNA score, 77.7% of participants were 'well nourished', 22.3% were 'at risk of malnutrition', and 2% were 'malnourished'. Nutritional status was divided into two groups by MNA score as 'well nourished' and 'malnourished', which was formed with the combination of 'at risk of malnutrition' and 'malnourished'. Compared with the well nourished group, the malnourished group was older, and physical status indicators such as weight, BMI (Body Mass Index), and calf circumference of the malnourished group were much smaller. The malnourished group had higher prevalence rates of heart disease and stomach disorders while the well nourished group had a higher rate of hypertension. There was no significant difference between the malnourished group and well nourished group in nutritional intake below Chinese DRIs (Dietary Reference Intakes) among elder males. A higher proportion of elder females showed insufficient intakes of energy, protein, vitamin A, vitamin E, and zinc in the malnourished group compared to the well nourished group. The INQ (Index of Nutrition Quality) of calcium, magnesium, zinc, iodine, and most vitamins was less than 1 in both the malnourished and well nourished groups.
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