Purpose: Recently, there has been a worldwide increase in the prevalence of food allergies in children and it may cause nutritional imbalance and poor quality of life for growing children due to dietary restrictions. This study was conducted to quantitatively measure the life burden and related factors of mothers who are the primary caretakers of food-allergic children. Moreover, we generated data for use as a scientific basis for the development of a disease management program to reduce the burden on life from raising food-allergic children. Methods: Mothers of 2,005 children aged 2 years or older and enrolled in Seoul Metropolitan Atopy Free Schools in 2016 who have had diagnosis of food allergy in their lifetime were surveyed. The burden on life of parents with food-allergic children was measured using 17 questions from the Food Allergy Quality of Life-Parental Burden (FAQL-PB) questionnaire, and subjects were asked to respond with a scale of 1 ~ 7. Results: We analyzed the effects of sociodemographic factors of parents and children and the accompanying allergic disease factors on the lives of parents. The number of restricted food items due to food allergy of a child and accompanying diagnosis of atopic dermatitis had a positive (+) effect on the life burden of the parents. Conclusion: Accurate oral food challenge for food allergies should be administered to prevent excessive restriction of food intake. Moreover, efforts should be taken to prevent morbidity accompanying food allergy and relieve symptoms of asthma, allergic rhinitis and atopic dermatitis.
Forty-five Large White gilts were used to study the effect of energy intake from 28 to 176 d of age on body composition and reproductive development. From 28 to 60 d, the gilts were fed ad libitum a 16.6 MJ DE/kg, 24% crude protein and 1.3% total lysine diet. From 61 d of age three dietary treatments were used; 1) ad libitum access to feed (15.6 MJ DE/kg, 21% crude protein and 1.07% total lysine) (H), 2) feed offered at 75% (M) of the previous days intake of H, and 3) feed offered at 60% (L) of the previous days intake of H. ADG from 61 to 176 d of age was (p<0.05) affected by treatment. Although live weight at 176 d of age did not differ (p>0.1) the H gilts had higher (p<0.08) carcass weights than the M or L gilts. Back fat depths were similar (p>0.1) for all treatments at 115 d of age, however by 176 d of age M and H gilts were fatter (p<0.1) than L gilts. The mean lipid deposition (LD) from 115 to 176 d of age for L gilts (78.9 g/d) was less (p<0.05) than for M gilts (143.6 g/d) and H gilts (135.6 g/d). There were no differences between treatments for protein deposition (PD) over the same period. More (p<0.05) H gilts (n=8) attained puberty (first observed estrus) than either M gilts or L gilts (n=4 for both). Follicle numbers were similar (p>0.1) across treatments. For gilts that attained puberty, H gilts had fewer (p<0.05) follicles (13.5) than M gilts (19.7) and L gilts (21.3). For gilts with follicular development, H gilts had the heaviest (458.7 g) reproductive tract weight (RTW). However, for those that attained puberty, L gilts had the heaviest RTW. RTW were lowest for those with no follicular development. Energy restriction had a negative impact on puberty attainment, i.e. it took longer to reach puberty. However, for gilts that attained puberty, the number of follicles was greater for those on r feed intakes. It would appear that rate of fat deposition, but not necessarily the total amount of fat, plays an important role in puberty attainment.
The purpose of this study was to investigate the effects of the nutrition therapy and drug(simvastatin) treatment on the levels of blood lipids for the hyperlipidemic patients according to genetic polymorphism of apo CIII. Subjects of the study consisted of 43(male: 9, female: 34 )hyperlipidemic patients registered to Kyung Hee Medical Center, Intakes of nutrients for the subjects were determined by 24-hr recall method through a personal interviews. The subjects were instructed to take the hypolipidemic and hypocholesterolemic diets. Compliance was monitored through food records, personal interview, and body-weight measurements. The patients changed their basal diet containing 40. 1g fat(182.7mg cholesterol./day) to a diet containing 30.7g fat(139.2mg cholesterol/day) after 12 weeks of nutrition therapy. The distribution of genotypes for apo CIII by Msp I was 32.5% of TT, 50.0% of TC, and 17.5% of CC. aT the beginning of the study, the level of blood triglyceride was the highest for patients with TT and TC. Following the nutrition therapy, patients with CC exhibited the largest drop of blood triglyceride. Following simvastatin treatment with nutrition therapy, blood LDL-cholesterol decreased in most of the patiens with TT. Meanwhile the distribution of genotypes for apo CII by the Fok I was 10.0% of TT, 47.5% of TC, and 42.5% of CC. The level of blood triglyceride was the highest for patients with CC. Following the nutrition therapy, patients with TC exhibited a significant drop for the blood triglyceride. Following simvastatin treatment with nutrition therapy the level of blood LDL-cholesterol decreased in most of the patients with CC. We concluded that dietary habits and food have been changed by nutrition therapy. And blood lipid levels have been decreased by the restriction of intakes of energy, fat and cholesterol. There was variation in the levels of blood lipids according to apo CII polymorphisms. The level of blood lipids responded to nutrition therapy and drug treatment in different was according to genetic polymorphisms. Accordingly, the choice of individualized therapy based on the patient\\`s genetic polymorphism is very important for effective therapy(Korean J Nutrition 33(8) : 813-823, 2000)
Journal of The Korean Society of Inherited Metabolic disease
/
v.23
no.2
/
pp.39-44
/
2023
Hereditary tyrosinemia type 1 (HT-1) is a metabolic disorder caused by biallelic pathogenic variants in the fumarylacetoacetate hydrolase (FAH) gene, which impairs the function of the FAH enzyme, resulting in the accumulation of tyrosine's toxic metabolites in hepatocytes and renal tubular cells. As a consequence, individuals with HT-1 exhibit symptomatic manifestations. Rapid diagnosis and treatment of HT-1 can prevent short-term death and long-term complications. A 15-day-old boy presented to the outpatient department with elevated levels of tyrosine on his newborn screening tests conducted at the age of 3 and 10 days, respectively. Further blood tests revealed increased levels of alpha-fetoprotein and amino acids including tyrosine and threonine. Urine organic acid tests indicated a significant elevation in tyrosine metabolites, as well as the presence of succinylacetone (SA), which led to the diagnosis of HT-1. Two pathogenic and likely pathogenic variants of FAH compatible with HT-1 were also detected. He began a tyrosine-restricted diet at one month old and received nitisinone (NTBC) at two months old. With continued treatment, the patient's initially elevated AFP level, detection of SA in the urine, and mild hepatomegaly showed improvement. During four years and seven months of treatment, there were no exceptional complications apart from an increase in tyrosine levels and a delay in speech. We report a case of tyrosinemia type 1 detected through newborn screening, treated with dietary restriction and NTBC, with a good prognosis.
Journal of The Korean Society of Inherited Metabolic disease
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v.17
no.1
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pp.11-17
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2017
Purpose: Phenylketonuria (PKU) results from a deficiency of phenylalanine hydroxylase (PAH). The mutation of the PAH gene results in decreased phenylalanine hydroxylase enzyme activity in hyperphenylalaninemia (HPA) patients. This study reports ten cases of patients with the benign HPA genotype c.158G>A (p.Arg53His, R53H) variant in the PAH gene and aims to evaluate the clinical significance of the R53H variant. Methods: Ten Korean patients with the HPA genotype the R53H variant were included in this study. A retrospective medical record review was conducted. We characterized the phenotypes of the patients with HPA with the R53H variant using the following system: classic PKU, moderate PKU, mild PKU, Mild HPA, and benign HPA. Results: Five patients had the R53H variant with the "Pathogenic" variants (R413P, R241C, $Y356^*$, c.442-1G>A, $Y325^*$), Two patients had the "Likely pathogenic" variants ($W187^*$, A259T), Two patients had the "Uncertain significance" variants (R53H, G344D), and One patient had the "Not provided" variant (c.1066-14C>G). Nine patients genotyped with the R53H variant were the patient with benign HPA and One patient genotyped with the R53H homozygote was within normal range of plasma phenylalanine. None of the ten patients required dietary restriction of phenylalanine or pharmacotherapy to maintain their plasma phenylalanine levels and showed no clinical symptoms of HPA. Conclusion: Ten patients with HPA genotype the R53H variant were the patient with benign HPA and showed no clinical symptoms of HPA. Thus, the R53H variant, which was previously classified as an "Uncertain significance" mutation in HPA patients, should be re-classified as "Benign."
Journal of The Korean Society of Inherited Metabolic disease
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v.16
no.2
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pp.79-85
/
2016
Purpose: This study aimed to analyze genetic mutations, clinical manifestations, and treatment of patients with benign HPA in Korea. Methods: This case series study involved ten HPA patients who were referred to our hospital because of high phenylalanine concentration. We investigated their demographic features, clinical manifestations, and mutations of the PAH gene through direct DNA sequencing. Results: Among ten patients with benign HPA, two pairs of patients were related (father-daughter, mother-daughter relationship) cases, and all of them showed no specific clinical manifestations or notable past history. Their plasma phenylalanine levels ranged between 1.2 and 4.2 mg/dL. In the tetrahydrobiopterin (BH4) loading test, all patients were nonresponsive to BH4. In the confirmation test of PAH mutation analysis, we identified eleven different alleles out of twelve. The most common allele was R53H (c.158G> A). In addition, two novel PAH gene mutations, V423A (c.1268T>C) and V51A (c.152T>C), were identified. Although the patients did not receive any pharmacologic treatment or continuous phenylalanine restriction dietary therapy, their neurocognitive development was normal. Moreover, on serial outpatient follow-up tests, all patients maintained phenylalanine levels below 6 mg/dL. Conclusion: This study is the first in Korea to analyze benign HPA patients. All patients with benign HPA could maintain phenylalanine levels below 6 mg/dL with normal neurocognitive development, without continuous therapy. Therefore, performing mutation analysis and distinguishing benign HPA from phenylketonuria (PKU) are important to help improve life quality in patients with benign HPA by avoiding unnecessary lifelong therapy.
Packages have been used for a wide variety of purposes, for protection, for display, for transportation of goods, or for keeping personal belongings. According to the demands of society and the times, liquor packages have been specialized and have appeared in almost every shape and size without restriction to cine particular type of material. In spite of its rapid development and wide application in our society, liquor package design has rarely been considered as a subject of comprehensive study. Majoring in package design, I have become especially interested in the area of liquor package design. I would like to explore liquor package design from several aspects. With the advent of new market and the rise of a new consumer society, advertising and mass media have expanded rapidly. While convenience of use is not a major issue, serving size certainly are quality, appeal of heritage and health concerns. Heritage is a major consumer appeal in Whisky, Beer, Wine and spirits. Designers have drawn heavily on the tradition of alcoholic products, have used type and graphics to create the illusion of heritage for new products. A sidelight to the heritage aspect of spirits package is the evolution of outer boxes for international liquors. International liquors package design illustrated the past and current themes. The design is contemporary and spare. Colored panels correlated to the liquor flavor used on clean white, black, gold boxes. While this research does not deny the impact of structural innovation and convenience package design , it does deny the existence of a graphic plateau. It is assumed therefore, that development in technology can facilitate communication between East and West. This can be accomplished because as containers of products are used in social setting, their form will gradually apply strong influence to the need for economical, easily handled, easily utilized packaging. Typically, ethnic package designs are those packages containing products which are prepared and marketed to a category of people who are prepared and marketed to a culture traits. They are liquor products sold in the metropolitan New York area which are marketed specially to Asians, Hispanics, or Eurpean population. These cultural groups share numerous traits including religion, language, dietary habits and traditional drinking styles. Therefore, the products which are familiar or common in their native countries are often imported or marketed there to serve them. These packages and products are frequently found on the shelves of supermarkets in predominantly ethnic areas. That is Korea, Japan if packaging is correctly design it would appeal to the American market. My research is that oriental beverage -Soju is good example of this precept. Assumedly, there must be a degree of subjectivity since it is a mean in which the consumers can relate to its advertising. This degree to relate and identify is the degree to which the package will be remembered and purchased. Subjectivity is intimately related to purchases since there is no such thing as a rational purchase in a society that operates on mass consumption. It is essential that packages become more personal human, entertaining, and more like advertising in order to maximize merchandising potential.
Coronary artery disease has increased in Korea as the country enters the aged society. It is well known that the incidence of coronary artery disease is related to aging, hypertension, diabetes, hyperlipidemia, and dietary habit. For effective treatment of significant coronary stenosis, close coordination between cardiac surgery and cardiology team is essential. Especially cardiologists' decision whether to do the stent placement or CABG is very important because the cardiologists usually start to consult the patients for their treatment. Recently, non-surgical interventions(that is stent placement) in cardiology field have dramatically increased as the national insurance system removed the limitation of the number of stents deployed. However, accidents are often caused by inappropriate use of stents, especially in patients with triple coronary disease or left main disease with heavy coronary calcifications. Another aspect of stent placement is to cope with an emergency case in the event of coronary rupture or pericardial tamponade during coronary interventions without cardiac surgeons. In the past two years, the Korea Consumer Agency (Consumer Dispute Coordination Committee) analyzed eight cases of medical dispute settlement. Only two hospitals were manned with both cardiologists and cardiac surgeons. Seven patients died of procedures of stenting and five patients died on the day of the procedure. Among the 8 cases, 5 cases showed 3 vessel disease and the rest of the cases had either severe calcification, complete occlusion or poor coronary antomies for stenting According to a 2017 national data registry of coronary stenting, less than 3 drug-eluting stents were implanted in 98% of all patients. In 2015, the number of stent procedures was 38,922, and approximately in 800 (2%) cases, more than four stents were used per patient. We emphasize that it is necessary to seriously consider the cost-benefit analysis between stent and CABG. The patient has the right to choose the right procedure by asking the liability of 'instruction explanation obligation'. He should be well informed of the pros and cons of both procedures to avoid overuse of stent. It can be solved by intimate discussion of individual cases with the cardiac surgeon and the patient. Unilateral dialogue with the patient, forceful restriction on the number of stenting, lack of surgeon's backup in difficult cases should all be avoided. It is also necessary to solve the problem not only at the hospital level, such as multidisciplinary integrated medical care, but also a nationwide solution such as expanding cardiac surgeons as essential personnel to public officials.
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