• Journal of The Korean Society of Inherited Metabolic disease
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• v.17 no.3
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• pp.92-95
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• 2017

Fabry disease (FD) is an X-linked lysosomal storage disorder caused by an ${\alpha}$-galactosidase A (GLA, MIM 300644) enzyme deficiency due to pathogenic variants in the ${\alpha}$-galactosidase A gene (GLA). The disease leads to accumulation of globotriaosylceramide (Gb3) and related glycophospholipids affecting nearly all major organ systems, with the primary sites damaged by Gb3 including renal glomeruli, myocardium, neurons of the dorsal ganglion and autonomic nervous system, and vascular endothelial and smooth muscle. Progressive deposition in these organ systems present with various clinical manifestations including acroparesthesia, renal failure and heart failure. Here, we report a Chinese male diagnosed with Fabry disease in his late $4^{th}$ decades showing improvement of acroparesthesia during enzyme replacement therapy (ERT). A 48-year-old Chinese man who presented with chronic recurrent severe burning pain in his fingers and toes since the age of 10, with worse involvement of the former visited to our clinic for further evaluation. His medical history included a transient ischemic attack aged 40 and diagnosed with stage 4-5 chronic kidney disease aged 47. In the family history, the patient's brother was found to be have Fabry disease 1 month before his visit. Except for his brother, all other members of the family are healthy. Based on his medical history and family history, he was strongly suspicious for Fabry disease. He was found to have a galactose-alpha-1,3-galactose level 4.96 (Reference range, 42.5-67.9) suggestive of Fabry disease. The followed sequencing of GLA coding region in our patient revealed hemizyosity for the mutation c.988C>T (Q330X) in Exon 7. Since ERT start, he showed significant improvement in his symptoms of burning sensation of fingers and toes. On the contrary, due to deteriorating kidney function even with ERT, he is considered for kidney transplantation. Despite of diagnostic delay until late 4th decades, ERT showed a potential improvement of acroparesthesia in our patient. However, late start of ERT can lead to poor outcome in multiorgan function. Therefore, early diagnosis with high index of suspicion followed by continuous ERT with regular monitoring have an impact on quality of life in Fabry disease.

• Korean Journal of Ecology and Environment
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• v.37 no.4 s.109
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• pp.411-422
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• 2004

A limnological survey was conducted in a reservoir, Lake Hoengsung located in Kangwondo, Korea, from July 2000 to September 2001 on the monthly basis. Phosphorus loading from the watershed was estimated by measuring total phosphorus concentration in the main tributary. Secchi disc transparency, epilimnetic (0-5 m) turbidity, chlorophyll a (Chl-a), total phosphorus (TP), total nitrogen(TN) and silica concentration were in the range of 0.9-3.5 m, 0.1-8.5 NTU, 0.3-32.4 mgChl $m^{-3}$, 5-46 mgP $m^{-3}$, 0.83-3.55 mgN $L^{-1}$ and 0.5-9.6 mgSi $L^{-1}$, respectively. Green algae and cyanobacteria dominated phytoplankton community in warm seasons, from July through October, 2000. In July a green alga (Scenedesmus sp.) was dominant with a maximum cell density of 10,480 cells mL. Cyanobacteria (Microcystics sp.) dominated in August and September with cell density of 3,492 and 295 cells mL ,respectively. Species diversity of phytoplankton was highest (2.22) in July. The trophic state of the reservoir can be classified as eutrophic on the basis of TP, Chl-a, and Secchi disc transparency. Because TP concentration was high in flood period, most of phosphorus loading was concentrated in rainy season. TP loading was calculated by multiplying TP and flow rate. The dam managing company measured inflow rate of the reservoir daily, while TP was measured by weekly surveys. TP of unmeasured days was estimated from the empirical relationship of TP and the flow rate of the main tributary; $TP=5.59Q^{0.45}\;(R^2=0.47)$. Annual TP loading was calculated to be 4.45 tP $yr^{-1}$, and the areal P loading was 0.77 gP $m^{-2}\;yr^{-1}$ which is similar to the critical P loading for eutrophication by Vollenweider's phosphorus model, 0.72 gP $m^{-2}\;yr^{-1}$.

• Tuberculosis and Respiratory Diseases
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• v.41 no.2
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• pp.111-119
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• 1994

Background: Because of the common etiologic factor, such as smoking, lung cancer and chronic obstructive pulmonary disease are often present in the same patient. The preoperative prediction of remaining pulmonary function after the resectional surgery is very important to prevent serious complication and postoperative respiratory failure. $^{99m}Tc$-MAA perfusion scan has been used for the prediction of postoperative pulmonary function, but it may be inaccurate in case of large V/Q mismatching. We compared $^{99m}Tc$-DTPA radioaerosol inhalation scan with $^{99m}Tc$-MAA perfusion scan in predicting postoperative lung function. Method: Preoperative inhalation scan and/or perfusion scan were performed and pulmonary function test were performed preoperatively and 2 month after operation. We predicted the postoperative pulmonary functions using the following equations. Postpneurnonectomy $FEV_1$=Preop $FEV_1x%$ of total function of lung to remain Postlobectomy $FEV_1$=Preop $FEV_1{\times}$(% of total 1-function of affected lung${\times}$$\frac{Number\;of\;segments\;to\;be\;resected}{Number\;of\;segments\;of\;affected\;lung})$ Results: 1) The inhalation scan showed good correlations between measured and predicted $FEV_1$, FVC and $FEF_{25-75%}$. (correlation coefficiency; 0.94, 0.91, 0.87 respectively). 2) The perfusion scan also showed good correlations between measured and predicted $FEV_1$, FVC and $FEF_{25-75%}$. (correlation coefficiency; 0.86, 0.72, 0.87 respectively). 3) Among three parameters, $FEV_1$ showed the best correlations in the prediction by lung scans. 4) Comparison between inhalation scan and perfusion scan in predicting pulmonary function did not show any significant differneces except FVC. Conclusion: The inhalation scan and perfusion scan are very useful in the prediction of postoperative lung function and don't make a difference in the prediction of pulmonary function a1though the former showed a better correlation in FVC.

• Asian-Australasian Journal of Animal Sciences
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• v.21 no.1
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• pp.107-119
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• 2008

To determine the true digestible phosphorus (TDP) requirement of growing pigs, two experiments were designed with the experimental diets containing five true digestible P levels (0.16%, 0.20%, 0.23%, 0.26% and 0.39%) and the ratio of total calcium to true digestible P (TDP) kept at 2:1. In Experiment 1, five barrows (Duroc${\times}$Landrace${\times}$Yorkshire) with an average initial body weight of 27.9 kg were used in a $5{\times}5$ Latin-square design to evaluate the effect of different dietary P levels on the digestibility and output of P and nitrogen. In Experiment 2, sixty healthy growing pigs (Duroc${\times}$Landrace${\times}$Yorkshire) with an average body weight (BW) of 21.4 kg were assigned randomly to one of the five dietary treatments (12 pigs/diet), and were used to determine the true digestible phosphorus (TDP) requirement of growing pigs on the basis of growth performance and serum biochemical indices. The results indicated that the true digestibility of P increased (p<0.05) linearly with increasing dietary TDP level below 0.26%. The true P digestibility was highest (56.6%) when dietary TDP was 0.34%. Expressed as g/kg dry matter intake (DMI), fecal P output increased (p<0.05) linearly with increasing P input. On the basis of g/kg fecal dry matter (DM), fecal P output was lowest for Diet 4 and highest (p<0.05) for Diet 5. The apparent digestibility of crude protein (CP) did not differ (p>0.05) among the five diets, with the average nitrogen output of 12.14 g/d and nitrogen retention of 66% to 74% (p>0.05), which suggested that there was no interaction between dietary P and CP protein levels. During the 28-d experimental period of Experiment 2, the average daily gain (ADG) of pigs was affected by dietary TDP levels as described by Eq. (1): $y=-809,532x^4+788,079x^3-276,250x^2+42,114x-1,759$; ($R^2=0.99$; p<0.01; y = ADG, g/d; x = dietary TDP, %), F/G for pigs by Eq. (2): $y=3,651.1x^4-3,480.4x^3+1,183.8x^2-172.5x+10.9$ ($R^2=0.99$; p<0.01; y = F/G; x = dietary TDP, %), and Total P concentrations in serum by Eq. (3): $y=-3,311.7x^4+3,342.7x^3-1,224.6x^2+195.6x-8.7$ (R2 = 0.99; p<0.01; y = total serum P concentration and x = dietary TDP, %). The highest ADG (782 g/d), the lowest F/G (1.07) and the highest total serum P concentration (3.1 mmol/L) were obtained when dietary TDP level was 0.34%. Collectively, these results indicate that the optimal TDP requirement of growing pigs is 0.34% of the diet at a total Ca to TDP ratio of 2:1.

• Asian Pacific Journal of Cancer Prevention
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• v.14 no.5
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• pp.3075-3078
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• 2013

Background: KLK3 gene products, like human prostate-specific antigen (PSA), are important biomarkers in the clinical diagnosis of prostate cancer (PCa). G protein-coupled receptor RFX6, C2orf43 and FOXP4 signaling plays important roles in the development of PCa. However, associations of these genes with PCa in northern Chinese men remain to be detailed. This study aimed to investigate their impact on occurrence and level of malignancy. Methods: All subjects were from Beijing and Tianjin, including 266 cases with prostate cancer and 288 normal individuals as controls. We evaluated associations between clinical covariates (age at diagnosis, prostate specific antigen, Gleason score, tumor stage and aggressive) and 6 candidate PCa risk loci, genotyped by PCR- high resolution melting curve and sequencing methods. Results: Case-control analysis of allelic frequency of PCa associated with PCa showed that one of the 6 candidate risk loci, rs339331 in the RFX6 gene, was associated with reduced risk of prostate cancer (odds ratio (OR) = 0.73, 95% confidence interval (CI) =0.57-0.94, P = 0.013) in northern Chinese men. In addition, subjects with CX (CC+TC) genotypes had a decreased risk for prostrate cancer compared to those carrying the TT homozygote (OR =0.64, 95% CI = 0.45- 0.90, P = 0.008). The TT genotype of 13q22 (rs9600079, T) was associated with tumor stage (P=0.044, OR=2.34, 95% CI=0.94-5.87). Other SNPs were not significantly associated with clinical covariates in prostate cancer (P > 0.05). Conclusions. rs339331 in the RFX6 gene may be associated with prostate cancer as a susceptibility locus in northern Chinese men.

• Journal of Oral Medicine and Pain
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• v.35 no.2
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• pp.123-133
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• 2010

The purpose of this study is to investigate Tension-type headache associated with pericranial tenderness and Headache attributed to temporomandibular joint disorder among Characteristic pain index, Disability points, Chronic pain grade, Depression index, Nonspecific physical symptoms (pain items included), Nonspecific physical symptoms (pain items excluded), Jaw disability score using RDC/TMD Axis II. Eighty five patients who visited the Kyung Hee University Dental Hospital were diagnosed as Tension-type headache associated with pericranial tenderness(n=48) and Headache attributed to temporomandibular joint disorder(n=37) by the International Classification of Headache Disorders, 2nd Edition, and were administered the Korean versions of the RDC/TMD Axis II- Characteristic pain index, Disability points, Chronic pain grade, Depression index, Nonspecific physical symptoms (pain items included), Nonspecific physical symptoms (pain items excluded), Jaw disability score. Then it was analyzed statistically by SPSS(ver. 10.0). T-test, The Wilcoxon-signed rank test and Mann-Whitney U test (p<0.05) were used. There were no significant differences in Characteristic pain index, Disability points, Chronic pain grade, Depression index, Nonspecific physical symptoms (pain items included), Nonspecific physical symptoms (pain items excluded), Jaw disability score between two groups. Tension type headache associated with pericranial tenderness and Headache attributed to temporomandibular joint disorder showed similar Axis II feature. Tension-type headache associated with pericranial tenderness may be related to Headache attributed to temporomandibular joint disorder.

• Tuberculosis and Respiratory Diseases
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• v.50 no.1
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• pp.106-116
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• 2001

Background : Pulmonary thromboembolism is relatively frequent and potentially fatal. However, it is commonly misdiagnosed. The incidence of pulmonary thromboembolism is not decreasing despite advances in diagnosis and effective prophylatic measures. Its potential for significant sequela necessitates a prompt diagnosis and treatment. Unfortunately, there are many difficulties and problems regarding accurate diagnosis. There is a low prevalence of deep vein thrombosis and pulmonary thromboembolism in Korea and only few reports on this subject are available. Method : The clinical features of 36 patients, who were diagnosed with pulmonary thromboembolism at the Korea University medical center, were reviewed. Results : 1) There was no significant difference in prevalence between men an women, and the mean age was 50.9 years in men 59.2 years in women. 2) The frequent causes of pulmonary thromboembolism were malignancies (22.2%), surgery (22.2%), and heart disease(8.2%). Specific causes were not identified in 33.3%. 3) The most common symptom was dyspnea(72.2%), and the most common sign was tachypnea(61.1%). 4) The EKG findings were normal in 28.6%, an S1Q3T3 pulmonale pattern in 25.7%, ST or QRS changes in others. 5) The chest X-ray findings indicated pulmonary infiltration in 37.5%, cardiomegaly in 15.6%, pleural effusion in 12.5%, and normal in 27.8%. The perfusion lung scan showed a high probability in 66.7%, and intermediate or low probability in 33.3%. 6) The pulmonary arterial pressure(PAP) in the high probability groups was 57.9mmHg with a higher mortality rate(35%). Conclusion : Pulmonary thromboembolism is not uncommon in Korea and its clinical features do not differ greatly from thase reported in the literature. When pulmonary thromboemblism of unknown causes are diagnosed, a search for an occult malignancy is recommended. Rapid diagnosis and treatment are achieved when thromboemblism is suspected.

• Journal of Animal Science and Technology
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• v.49 no.4
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• pp.491-500
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• 2007

Two experiments were used to determine the effects of environment-friendly diets on growth performance, fecal excretion, nitrogen excretion and emission gases in manure for growing pigs. In experiment 1, ninety six crossed pigs(Landrace×Yorkshire×Duroc) were allocated into four treatments. Treatments were AME(adequate ME diet, 3,265 kcal/kg), LME(lower ME diet, 3,100 kcal/kg), LME 0.05(lower ME diet+α- galactosidase & β-mannanase 0.05%) and LME 0.10(lower ME diet+α-galactosidase & β-mannanase 0.10%). Pigs fed AME diet had lower ADFI(Average Daily Feed Intake) than pigs fed other diets(p<0.05). DM(Dry Matter) digestibility in pigs fed AME and LME 0.10 diets had greater than pigs fed LME diet(p<0.05). Energy digestibility is higher in pigs fed AME and LME 0.10 diets than other treatments(p<0.05). In experiment 2, twenty four crossbred pigs(33.71 kg average BW) were used in a 14-d metabolism experiment. The pigs were housed in individual cages equipped with plastic bed flooring. Treatments were CP(Crude protein) 18% without Bacillus sp., CP 18% diet+Bacillus sp. 0.05%, CP 14% without Bacillus sp. and CP 14% diet+Bacillus sp. 0.05%. Nitrogen intake was higher for CP 18% diets than CP 14% diets(p<0.05). DM, N(Nitrogen) and energy digestibility were affected by probiotics(p<0.05). With the high CP in diets, Energy and N digestibility, urine N percent, urine N excretion and total N excretion were increased significantly compared to low CP in diets(p<0.05). Among the treatments, DM and N digestibilities, feces N excretion, N absorption were decreased significantly(p<0.05), however, feces excretion, feces N, urine N percent, urine N excretion and total N excretion were increased significantly(p<0.05) when pigs fed without probiotics diets compare to pigs fed with probiotics diets. DM and N digestibility, feces excretion, feces N excretion, urine N percent, urine N excretion, total N excretion, N absorption and N adsorption ratio were CP×probiotic interactions in p<0.05. Ammonia(p<0.01) and H2S(p<0.05) in manure were lower in CP 14% diets than CP 18% diets. Also, ammonia and H2S in manure were CP×probiotic interactions in p<0.05. In conclusion, low energy and reduction of CP dietary added enzyme and probiotics improved nutrient digestibility and reduced odors emission in manure for growing pigs.

• Molecules and Cells
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• v.21 no.1
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• pp.52-62
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• 2006

In previous reports we demonstrated that ginsenosides, active ingredients of Panax ginseng, affect some subsets of voltage-dependent $Ca^{2+}$ channels in neuronal cells expressed in Xenopus laevis oocytes. However, the major component(s) of ginseng that affect cloned $Ca^{2+}$ channel subtypes such as ${\alpha}_{1C}$(L)-, ${\alpha}_{1B}$(N)-, ${\alpha}_{1A}$(P/Q)-, ${\alpha}_{1E}$(R)- and ${\alpha}_{1G}$(T) have not been identified. Here, we used the two-microelectrode voltage clamp technique to characterize the effects of ginsenosides and ginsenoside metabolites on $Ba^{2+}$ currents ($I_{Ba}$) in Xenopus oocytes expressing five different $Ca^{2+}$ channel subtypes. Exposure to ginseng total saponins (GTS) induced voltage-dependent, dose-dependent and reversible inhibition of the five channel subtypes, with particularly strong inhibition of the ${\alpha}_{1G}$-type. Of the various ginsenosides, $Rb_1$, Rc, Re, Rf, $Rg_1$, $Rg_3$, and $Rh_2$, ginsenoside $Rg_3$ also inhibited all five channel subtypes and ginsenoside $Rh_2$ had most effect on the ${\alpha}_{1C}$- and ${\alpha}_{1E}$-type $Ca^{2+}$ channels. Compound K (CK), a protopanaxadiol ginsenoside metabolite, strongly inhibited only the ${\alpha}_{1G}$-type of $Ca^{2+}$ channel, whereas M4, a protopanaxatriol ginsenoside metabolite, had almost no effect on any of the channels. $Rg_3$, $Rh_2$, and CK shifted the steady-state activation curves but not the inactivation curves in the depolarizing direction in the ${\alpha}_{1B}$- and ${\alpha}_{1A}$-types. These results reveal that $Rg_3$, $Rh_2$ and CK are the major inhibitors of $Ca^{2+}$ channels in Panax ginseng, and that they show some $Ca^{2+}$ channel selectivity.

• Radiation Oncology Journal
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• v.20 no.1
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• pp.41-52
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• 2002

Purpose : 3D conformal radiotherapy, the optimum dose delivered to the tumor and provided the risk of normal tissue unless marginal miss, was restricted by organ motion. For tumors in the thorax and abdomen, the planning target volume (PTV) is decided including the margin for movement of tumor volumes during treatment due to patients breathing. We designed the respiratory gating radiotherapy device (RGRD) for using during CT simulation, dose planning and beam delivery at identical breathing period conditions. Using RGRD, reducing the treatment margin for organ (thorax or abdomen) motion due to breathing and improve dose distribution for 3D conformal radiotherapy. Materials and Methods : The internal organ motion data for lung cancer patients were obtained by examining the diaphragm in the supine position to find the position dependency. We made a respiratory gating radiotherapy device (RGRD) that is composed of a strip band, drug sensor, micro switch, and a connected on-off switch in a LINAC control box. During same breathing period by RGRD, spiral CT scan, virtual simulation, and 3D dose planing for lung cancer patients were peformed, without an extended PTV margin for free breathing, and then the dose was delivered at the same positions. We calculated effective volumes and normal tissue complication probabilities (NTCP) using dose volume histograms for normal lung, and analyzed changes in doses associated with selected NTCP levels and tumor control probabilities (TCP) at these new dose levels. The effects of 3D conformal radiotherapy by RGRD were evaluated with DVH (Dose Volume Histogram), TCP, NTCP and dose statistics. Results : The average movement of a diaphragm was 1.5 cm in the supine position when patients breathed freely. Depending on the location of the tumor, the magnitude of the PTV margin needs to be extended from 1 cm to 3 cm, which can greatly increase normal tissue irradiation, and hence, results in increase of the normal tissue complications probabiliy. Simple and precise RGRD is very easy to setup on patients and is sensitive to length variation (+2 mm), it also delivers on-off information to patients and the LINAC machine. We evaluated the treatment plans of patients who had received conformal partial organ lung irradiation for the treatment of thorax malignancies. Using RGRD, the PTV margin by free breathing can be reduced about 2 cm for moving organs by breathing. TCP values are almost the same values $(4\~5\%\;increased)$ for lung cancer regardless of increasing the PTV margin to 2.0 cm but NTCP values are rapidly increased $(50\~70\%\;increased)$ for upon extending PTV margins by 2.0 cm. Conclusion : Internal organ motion due to breathing can be reduced effectively using our simple RGRD. This method can be used in clinical treatments to reduce organ motion induced margin, thereby reducing normal tissue irradiation. Using treatment planning software, the dose to normal tissues was analyzed by comparing dose statistics with and without RGRD. Potential benefits of radiotherapy derived from reduction or elimination of planning target volume (PTV) margins associated with patient breathing through the evaluation of the lung cancer patients treated with 3D conformal radiotherapy.