• Journal of The Korean Society of Inherited Metabolic disease
• /
• v.18 no.1
• /
• pp.30-34
• /
• 2018

Niemann Pick type C disease (NPC) is an inherited progressive neurodegenerative disorder, due to defects of intracellular lipid trafficking and storage. Hepatosplenomegaly may prevail, while progressive neurodegenerative symptoms such as cerebellar involvement, dystonia, vertical supranuclear ophthalmoplegia, cataplexy, and eventually seizures starting at juvenile or late infantile period may accompany after normal early development. Here we describe a 3-year-old Korean boy with NPC who presented with splenomegaly at age 3. Liver biopsy showed characteristic foamy cell stained by periodic acid-schiff, and molecular analysis for NPC1 identified the compound heterozygous mutations, novel mutation of c.1631G>A (p.Trp544Ter) and c.2662C>T (p. Pro888Ser) as a known mutation. Filipin was strongly stained with unesterified cellular cholesterol in the patient's skin fibroblasts. The patient has received migulstat since age 3 years and his long-term outcome is needed to be observed.

• Journal of The Korean Society of Inherited Metabolic disease
• /
• v.15 no.1
• /
• pp.44-48
• /
• 2015

Mucopolysaccharidosis (MPS) IIIA is a lysosomal storage disorder caused by abnormalities of the enzyme Heparan N-sulfatase that is required for degradation of heparan sulfate. The patient in this study was a 5 year-old boy who presented with macrocephaly and developmental delay. Urinary excretion of glycosaminoglycan was increased (26 g/moL creatinine, reference range: <7 g/moL creatinine) and a distinct band of heparan sulfate was shown in electrophoresis. Heparan N-sulfatase activity was significantly decreased in skin fibroblasts (0.2 pmoL/min/mg protein, reference range: 9-64 pmoL/min/mg protein). PCR and direct sequencing analysis of the SGSH gene showed compound heterozygous mutations: c.1040C>T (p.S347F) and c.703G>A (p.D235N). This is the first report for a Korean patient with MPS IIIA who was confirmed by biochemical investigation and molecular genetic analyses.

• Horticultural Science & Technology
• /
• v.32 no.5
• /
• pp.636-644
• /
• 2014

This experiment examined the effects of preharvest Ca-chitosan spraying and short-term post-harvest treatment with high $pCO_2$ on the shelf-life of 'Seolhyang' strawberry fruit. Fruit firmness decreased when harvested late in the season and was little affected by Ca-chitosan spray treatments. Short-term treatment with high $pCO_2$ after harvest, however, significantly increased fruit firmness regardless of preharvest treatment. The combined treatment of Ca-chitosan spray and high $pCO_2$ led to an additional increase in shelf-life through delay of skin disorder, fruit decay incidence and firmness loss during simulated marketing. The residual effects of both treatments, however, were reduced when fruit were harvested late in the season. Fruit firmness was more affected by high $pCO_2$ treatment whereas decay and visual quality were affected by preharvest spraying with Ca-chitosan. Although more research is required to determine the optimum concentration of calcium and chitosan, and the optimal spray interval, our results show that the combined treatment of preharvest Ca-chitosan spray and postharvest high $pCO_2$ is effective in improving strawberry fruit shelf-life by increasing firmness and delaying decay.

• Journal of Veterinary Clinics
• /
• v.31 no.5
• /
• pp.371-375
• /
• 2014

This article describes the use of laparoscopic-assisted cystoscopy for removal of urinary calculi in five dogs. All dogs had micturition disorder due to urinary calculi. The surgical technique used was same in all cases. A urethral catheter passed into the urinary bladder through the urethra preoperatively. A 5-mm diameter cannula was placed in the ventral midline, 1 to 2 cm cranial to the umbilicus, and the 5-mm laparoscope was introduced via the cannula. A 10-mm diameter cannula was placed adjacent to the apex of the bladder under the visual guidance of laparoscopy. The bladder was then partially exteriorized through the 10-mm portal site, and a stab incision was performed on the bladder wall. The incisional margin of the bladder was sutured to the skin of the second portal site in $360^{\circ}$ simple continuous suture. A 2.7-mm diameter cystoscope with a sheath was introduced into the bladder lumen. The cystic and urethral calculi were removed under the visual guidance of cystoscopy with continuous fluid flushing. No major postoperative complications were identified. During the follow up period (range 7 to 21 months), no episodes of urinary dysfunction or recurrence of clinical signs were observed.

• The Journal of Korean Medicine Ophthalmology and Otolaryngology and Dermatology
• /
• v.10 no.1
• /
• pp.209-246
• /
• 1997

In the literatual study on the Ecthyma, the results were as follows. 1. The pathogenic factors of Ecthyma is divided three parts. One is exogenous pathogenic factors which including the wind evil, wind heat and toxic material produced by wetness evil. Another is endogenous pathogenic factors which including the declination of kidney-yang, impairment of the liver and kidney, the lower classes of yin-fire, accumulation of wetness-heat in the spleen asthenia, impairment of the liver and kidney, wetness-heat of three yang, asthenic heat-syndrome of three yin. The other is pathogenic factors neither endogenous nor exogenous which including the food and living, uncontrol sexual excess, anxiety and angry, injury of skin, injury of insects and animals. 2. Five viscera which was concerned with Ecthyma are liver, spleen and kidney. 3. Frequent region of Ecthyma are S-36(足三里) and C-7(陰交). External Ecthyma was rose to wetness-heat of three yang channel that cured easily. Internal Ecthyma was rose to asthenic heat-syndrome of three yin channel that cured hardly. 4. In the frequency of prescription, the most numerous prescription is Bojungikgitang(補中益氣湯) and the next are Kyukgigo(隔紙膏) and Yukmijihwanghwan(六味地滉丸). 5. In the frequency of medicine, the most medicine is Calomelas(輕粉) which included Hydrargyrum(水銀) and the next are Olibanum(乳香) and Resina Commiphorae Myrrhae(沒藥) which regulating vital energy and pain control medicine used that in order to destroy insects and remove polson. 6. In classification of the medical action, medicine of clearing away summer-heat and heat evil and activating blood circulation to dissipate blood stasis used to be very busy which in order to remove the disorder of vital energy for virulent heat-evil. 7. In classification of four characters, the most part is warm medicine, the next are cold and cool medicine and there is a few that is hot medicine. 8. In classification of five tastes, the most numerous tastes are bitter and acrid, the next are sweet, salty and sour tastes. 9. In classification of virulence of medicine, the most part is non-toxic, the next are weakly and deadly poison. 10. In classification of channel distribution, the most is the medicine that belongs to liver channel, the next are the lung, spleen, stomach and kidney channel.

• Journal of Korean Public Health Nursing
• /
• v.14 no.2
• /
• pp.431-445
• /
• 2000

In general the prevalence of old people is high and frequently have multiple diseases and symtoms requiring treatment. The nature of illness in the elderly has to be faced, and drugs do have an important part in the treatment of that illness. The purpose of this study were to describe health status and medication, and to provide some basic data for elderly's health education, especially for the right medication. Body mass index, self perceived health status, activities of daily living, disease and experience of past operation were surveyed to recognize the 249 elderly's actual health status. The degree of the elderly's understanding the way of medication, experience of side effects, experience of drug combination and incidence of drug adverse reaction along with drug combination were examined for medication. The aged $women(BMI; 10.7\pm13.3\%)$ overweighed the aged $men(BMI; 4.0\pm10.4\%)$. $69.0\%$ of them recognized their health good. Their activities of daily living were diminished following by the age group(p=0.0068) and relationship with self perceived health status were very significant(p=0.0005). They(192 elderly) suffered from multiple disease such as $osteoarthritis(49.5\%)$, $hypertension(32.0\%)$, gastric $disorder(16.1%)$, $diabetes(14.6\%)$, $osteomalacia(10.9\%)$, cardiovascular $disease(9.9\%)$, senile $cataract(5.7\%)$, skin $rash(5.2\%)$, liver $disease(4.2\%)$, kidney $disease(3.6\%)$, spinal cord $problem(3.6\%)$, respiratory $disease(2.1\%)$ $tuberculosis(1.0\%)\;etc(1.0\%).$ $28.3\%$ of them replied that they had an operation for appendictis senile cataract, peptic ulcer, spinal cord problem, pleurisy, hemorrhoid and the rest. Most of $them(87.4\%)$ knew the way very well how to use drugs, and $21.6\%$ of the replied 171 elderly experienced adverse drug reaction. Drug compliance rate were $high(83.6\%)$. In our study 56.9% of the 167 elderly took several medicine together. And $18.9\%$ of the 95 elderly who did drug combination had an experience of drug interaction. One person kept average 5.5 kinds of drugs at home among 243 elderly. They kept $digestives(79.4\%)$, $ointments(68.7\%)$, $vitamins(59.7\%)$, $analgesics(59.7\%)$, cold $medicines(45.3\%)$ antiarthritic $drugs(33.3\%)$, health $foods(27.7\%)$, antihypertensive $drugs(25.1\%)$, anti peptic ulcer $drugs(24.7\%)$, $laxatives(19.8\%)$, $antacids(16.5\%),\;antibiotics(l6.5\%)$, hypoglycemic $agents(10.3\%)$, cardiac $stimulants(7.0\%)$, $diuretics(4.5\%)$, antiarrhythmic $drugs(4.9\%)$, anti anginal $drugs(4.1\%)$, $hypnotics(3.3\%)$, $etc(38.3\%)$. With this result, we ascertain that polypharmacy in the elderly caused by multiple disease is common, which lead to drug interaction. So our task is to educate elderly how to use drugs in order to maximize their efficiency and to minimize their adverse effects.

• Journal of the Korean Home Economics Association
• /
• v.46 no.5
• /
• pp.97-109
• /
• 2008

Atopic dermatitis is the fastest growing skin disorder among children from infancy to adolescence in Korea. The side-effects of atopic dermatitis not only include physical discomfort, but also psychological trauma which ultimately affects the developmental growth of children. This study was conducted in order to investigate the behavioral characteristics of an atopic dermatitis sufferer. As part of this research, the relative influence of behavioral problems and, self-perceived competence were analyzed in relation to the social competence of an atopic child. In total, 301 atopic and non-atopic children, between 2 and 6 years of age, and their mothers and teachers participated in the study. From this number, 109 children had atopic dermatitis, while 192 children did not. Mothers were asked to complete a parent-report questionnaire that required information on parental stress, according to the scale parenting methodology of Abbdin(1990) and Cho(1999). Teachers were subjected to teacher-report questionnaires which included topics on social competence, socia-emotional assessment and behavioral problems of an atopic child. Furthermore, children also completed questionnaires on self-perceived competence. According to analysis on K-CBCL, ITSEA, social competence, self-perceived competence and parental stress, children with atopic dermatitis showed higher scores in depression/anxiety and depression/withdrawal, compared to children in the normal control group. In relation to parental stress, daily stress, parental role stress, and stress related disease, mothers with atopic dermatitis children exhibited higher scores. In establishing relationships among the related variables, atopic children who demonstrated more social competence were more likely to suffer less from withdrawal, attention problems, and depression/anxiety. Stress related disease in mothers with atopic children was positively related to attention problems of the child. In terms of relative influences, behavioral problems was the most significant variable, accounting for 23% of variance. Lower behavioral problems was positively related to more social competence. In summation, this study investigated the general characteristics of atopic children. In conclusion, atopic children and their mothers had difficulty in dealing with this disease. It is our belief that an atopic child would not only require physical treatment, but also need appropriate psychological care.

• Korean Journal of Plant Resources
• /
• v.22 no.6
• /
• pp.483-488
• /
• 2009

The main objective of this breeding program is to develop high yielding, disease resistance and good processing potato cultivar. 'Gui Valley' is a clonal selection resulting from a cross between 'ND2471-8' and 'Cona'. It has medium plant height and light green foliage. 'Gui Valley' has medium flowering habit and light pink flowers. 'Gui Valley' is a medium maturing potato cultivar and tubers are smooth, yellow skin, light yellow flesh, long-oval tuber shape, distinct red eyes with medium depth and medium dormancy. It has high level of tuber uniformity and good keeping quality. 'Gui Valley' demonstrates resistance to potato virus Y (PVY), soft rot, but moderately susceptible to late blight and common scab. It is also resistant to most of the internal and external physiological disorders particularly dehiscence, hollow heart and internal brown spot. The specific gravity of 'Gui Valley' is significantly higher (1.097) than that of 'Shepody' (1.078). 'Gui Valley' has suitable for processing mainly French fries and chips. This cultivar has high level of tuber uniformity and capable of yielding 37.6 $t{\cdot}ha^{-1}$, which is 18.2% higher than the control potato cultivar 'Shepody' under optimum agronomical practices.

• Journal of Life Science
• /
• v.26 no.8
• /
• pp.970-975
• /
• 2016

Acne vulgaris is a common chronic skin disorder that affects millions of people. The pathogenesis of acne has been known to be closely associated with the bacterium Propionibacterium acnes. Here we investigated the anti-acne activity of Lactobacillus plantarum K-1 BR by observing the expressions of proinflammatory cytokines, TNF-α, IFN-γ and IL-8, of human keratinocytes. When we applied heat-killed P. acnes to HaCaT cells, the inflammatory cytokines were induced by two- to four-fold compared to the normal control. When the bacteriocin, purified from L. plantarum K-1 BR, was pretreated to the HaCaT cells, the expression levels of TNF-α and IFN-γ stimulated by P. acnes significantly decreased to 25% and 30% of the induced levels, respectively. The IL-8 levels also significantly decreased with the concentration dependent manner of the bacteriocin. These results suggest that the bacteriocin from L. plantarum K-1 BR could reduce the expression levels of inflammatory cytokines and thus may relieve inflammations caused by acne.

• Journal of the Korean Child Neurology Society
• /
• v.26 no.4
• /
• pp.284-287
• /
• 2018

Tuberous sclerosis complex (TSC) is an autosomal dominant disorder caused by mutation of one of two genes, TSC1 (encoding hamartin, 9q34) and TSC2 (encoding tuberin, 16p13). It invades the central nervous system and various parts of the body, causing various symptoms. Crohn's disease (CD) is a chronic immune-mediated disease that has not been clearly elucidated. It is thought to be caused by an excessive immune response of the body to bacteria that normally exist in the digestive tract with genetic factors. No cases have been reported in which both of the above-mentioned diseases occurred simultaneously. We report a case of CD in a patient with TSC. A 12-year-old boy was brought to our hospital because of abdominal pain. Skin lesions were observed in the TSC. Fundus examination revealed a hamartoma in the right retina. Brain magnetic resonance imaging revealed a subendothelial giant cell astrocytoma (SEGA). On the basis of these findings, he was diagnosed as having TSC. Blood test results showed increased levels of inflammatory markers. On abdominal ultrasonography, his colon walls were observed to be thickened with increased vascularity of the proximal ascending colon, ileocecal valve, and terminal ileum. Colonoscopy revealed discontinuous ulcerations and inflammations of the ileum, IC valve, and cecum, similar to those found in CD. Everolimus was administered orally for the SEGA but was discontinued frequently owing to the exacerbation of CD. The possibility of CD should be kept in mind in patients with TSC considering to undergo treatment for SEGA.