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Clinical and Laboratory Features of Korean Mucopolysaccharidoses (MPSs) (한국 뮤코 다당체 침착증 환자에 대한 임상적 고찰)

  • Sohn, Woo Yun;Lee, Jee Hyun;Paik, Kyung Hoon;Kwon, Eun Kyoung;Kim, Ahn Hee;Jin, Dong Kyu
    • Clinical and Experimental Pediatrics
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    • v.48 no.10
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    • pp.1132-1138
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    • 2005
  • Purpose : The mucopolysaccharidoses (MPSs) are a heterogeneous group of lysosomal storage disorders. They are caused by a deficiency of the enzymes involved in the degradation of glycosaminoglycans. Early recognition is important because recombinant enzyme replacement therapy is now available for MPS. We studied the clinical characteristics of 80 MPS children with the object of determining the epidemiological, clinical and radiological features in Korean MPS children. Methods : Diagnosis of MPS was confirmed by skin fibroblast enzyme analysis in 80 patients between February 1995 and December 2004. Charts were retrospectively reviewed for clinical and radiological findings, as well as for intelligence and speech evaluations. Results : Hunter syndrome (MPS type II) was the most prevalent type, appearing in 51/80 cases (64 %), followed by Sanfilippo syndrome (MPS III-18%), Hurler syndrome (MPS I-15%), and Morquio syndrome (MPS IV-4%). The average age at diagnosis was 5.5 years (range 1 to 20), and the male-to-female ratio was 4.7 : 1. Typical radiographic changes were observed in 45/54 cases (83%). Mitral regurgitation was the most common cardiac defect. Moderate to profound mental retardation and hearing loss were present in 14/35 cases (56%) and 33/38 cases (82%), respectively. Four MPS II patients had bone marrow transplantation, with mixed outcomes. Five MPS I patients are currently on enzyme replacement therapy. Conclusion : Our study showed a high proportion of MPS II cases (64%), which may represent population variability. By studying the clinical features of these patients, we hope to alert pediatricians of the warning signs of MPS.

Clinical Studies of Measles Prevalence in Western Kyungnam District in 2000 (2000년도 경남 서부 지역에 유행한 홍역의 임상양상)

  • Kwon, Byoung O;Ju, Hye Young;Kim, Jeong Hee;Yoo, Hwang Jae;Kim, Chun Soo
    • Clinical and Experimental Pediatrics
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    • v.45 no.3
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    • pp.331-338
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    • 2002
  • Purpose : To investigate the feature of the range of measles in 2000, we studied epidemics of the measles in western Kyungnam District. Methods : Among 489 patients under 15 years of age who visited or were admitted to in our hospital from January to December in 2000, we selected 344 patients whose measles IgM antibody was positive. We investigated age, monthly incidence, clinical features, vaccination history, and measles IgG antibody. Results : Patients under 12 months of age occupied the largest proportion(36.9%). Patients firstly occurred in May and geometrically increased from October. Fever, cough and rashes were observed in all patients but conjunctivitis in 54.9%, Koplik spot in 23.8%. The first vaccination ratio was 41.8%. Compared with the number of patients and the first vaccination ratio, the first vaccination ratio was the lowest under 12 months of age. After that age group, the number of patients was decreased as the first vaccination ratio was increased. Among 152 patients sampled for measles IgG antibody, 35 patients had received the first vaccination and 6 patients had received the second vaccination. Among 35 patients who received the first vaccination, 22(62.9%) patients were negative of measles IgG antibody. Among 6 patients who received the second vaccination, 3(50%) patients were negative. Conclusions : Measles patients under 12 months still dominated. Therefore, routine vaccination of single measles vaccine, is currently done at 6 month when measles are prevalent, should be considered. High first vaccination failure suggests problems of vaccines itself, transport and storage rather than vaccination methods. Therefore a thorough investigation should be made.

Evaluation of the Breast plan using the TLD and Mosfet for the skin dose (열형광선량계(TLD)와 MOSFET을 이용한 유방암 방사선치료계획에 대한 피부선량 평가)

  • Kim, seon myeong;Kim, young bum;Bak, sang yun;Lee, sang rok;Jeong, se young
    • The Journal of Korean Society for Radiation Therapy
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    • v.27 no.2
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    • pp.107-113
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    • 2015
  • Purpose : The measurement of skin dose is very important that treatment of breast cancer. On account of the cold or hot dose as compared with prescription dose, it is necessary to analyse the skin dose occurring during the various plan of the breast cancer treatment. At our hospital, we want to apply various analyses using a diversity of dosimeters to the breast cancer treatment. Subjectss and Methods : In the study, the anthropomorphic phantom is used to find out the dose difference of the skin(draining site), scar and others occurring from the tangential treatment plan of breast cancer. We took computed tomography scan of the anthropomorphic phantom and made plans for the treatment planing using open and wedge, Field-in-Field, Dose fluence. Using these, we made a comparative analysis of the dose date points by using the Eclipse. For the dose comparison, we place the anthropomorphic phantom in the treatment room and compared the measurement results by using the TLD and MOSFET on the dose data points. Results : On the central point of treatment planing basis, the upward and downward skin dose measured by the MOSFET was the highest when the fluence was used. The skin dose of inner and outer was distinguished from the figure(5.7% ~ 10.3%) when the measurements were fulfilled by using TLD and MOSFET. The other side of breast dose was the lowest in the open beam, on the other hand, is highest in the Dose fluence plan. In the different kinds of treatment, the dose deviation of inner and outer was the highest, and so this was the same with the TLD and MOSFET measurement case. The outer deviation was highest in the TLD, and the Inner'was highest in the MOSFET. Conclusion : Skin dose in relation to the treatment plan was the highest in the planing using the fluence technique in general and it was supposed that the high dose had been caused by the movement of the MLC. There's some differences among the all the treatment planning, but the sites such as IM node occurring the lack of dose, scar, drain site are needed pay close attention. Using the treatment planning of dose fluence is good to compensate the lack of dose, but It increases the dose of the selective range rather than the overall dose. Therefore, choosing the radiotherapy technique is desirable in the lights of the age and performance of the patient.

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A Study on Calibration Procedures for Ir-192 High Dose Rate Brachytherapy Sources (고선량률(HDR) 근접치료의 동위원소 Ir-192에 대한 측정방법에 관한 고찰)

  • Baek, Tae-Seong;Lee, Seung-Wook;Na, Soo-Kyong
    • The Journal of Korean Society for Radiation Therapy
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    • v.19 no.1
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    • pp.19-26
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    • 2007
  • Purpose: To compare of the accuracy among various measurement procedures of HDR Brachytherapy, and to evaluate the clinical suitability and usefulness of alternative PMMA (polymethylmethacrylateplastics: $C_5H_8O_2$) plate phantom without any additional cost due to the purchase of measuring apparatus. Materials and Methods: We made a comparative study on three types of measuring systems: well type chamber, source calibration jig, and PMMA plate phantom. Farmer type chamber was used for source calibration jig method and PMMA plate phantom method. Measurement was done 5 times each in comparison with the measurement values from manufacturer. Measurement results from experiment were compared with that from the manufacturer which is offered with the source whenever a source is substituted by a new one and evaluate the accuracy of source activity. Results: As a consequence of Ir-192 source measurement using well type chamber, source calibration jig and PMMA plate phantom, RMS (Root Mean Square) values for the relative error are 0.6%, 1.57%, 2.1%, respectively, compared with the data from manufacturer. And the mean errors with standard deviation are given $-0.2{\pm}0.5%$, $0.97{\pm}1.23%$, $-0.89{\pm}1.87%$ respectively. Conclusion: From the results shown by the three types of measurement system (well type chamber, source calibration jig, and PMMA plate phantom), the measurement with well type chamber produced the best accuracy. It turns out that we can also use the alternative system of PMMA plate phantom clinically without purchasing any additional particular apparatus since the system does not exceed the recommendation of AAPM (American Association of Physicists in Medicine), which requires the error range of within ${\pm}5%$.

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Species Composition and Biomass of Marine Algal Community in the Vicinity of Yonggwang Nuclear Power Plant on the West Coast of Korea (서해안 영광원자력발전소 주변 해조군집의 종조성과 생물량)

  • KIM Young Hwan;HUH Sung-Hoi
    • Korean Journal of Fisheries and Aquatic Sciences
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    • v.31 no.2
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    • pp.186-194
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    • 1998
  • Species composition and biomass of intertidal benthic algae were studied at the coast of Yonggwang Nuclear Power Plant area and its adjacent stations over 4 seasons (October 1995-August 1996), Of 68 species identified, 7 were Cyanophyta, 12 were Chlorophyta, 14 were Phaeophyta and 35 were Rhodophyta. The largest number of algal species (44) was found at Sangnok, the northernmost station of the study area, whereas the smallest number of species (15) was found from Tongho, ca. 13 km north of the power plant site. Number of species showed highest during the spring (44) and minimum was recorded in autumn (28). Biomass per unit area showed maximum in spring ($189.5\;g\;dry\;wt{\cdot}m^{-2}$ in average) and minimum in winter ($107.9\;g\;dry\;wt{\cdot}m^{-2}$ in average). Biomass values exhibited a wide range of variation among the stations, ranging from a low of $22.0\;g\;dry\;wt{\cdot}m^{-2}$ in annual average at Tongho to a high of $295.7g\;dry\;wt{\cdot}m^{-2}$ in average at Sangnok. Dominant species in biomass were Corallina pilulifera, Sargassum thunbergii, Gymnogongrus flabelliformis and Enteromorpha compressa. There have been little variation in the dominant algal species around the power plant site during the past 10 years and also these algae appeared throughout the west coast of Korea with higher frequency.

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Regeneration Processes of Nutrients in the Polar Front Area of the last Sea IV. Chlorophyll a Distribution, New Production and the Vertical Diffusion of Nitrate (동해 극전선역의 영양염류 순환과정 IV. Clorophyll a 분포, 신생산 및 질산염의 수직확산)

  • MOON Chang-Ho;YANG Sung-Ryull;YANG Han-Soeb;CHO Hyun-Jin;LEE Seung-Yong;KIM Seok-Yun
    • Korean Journal of Fisheries and Aquatic Sciences
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    • v.31 no.2
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    • pp.259-266
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    • 1998
  • A study on the biological and chemical characteristics in the middle last Sea of Korea was carried out at 31 stations in October $11\~18$, 1995 on board the R/V Tam-Yang. The chlorophyll a concentration, new and regenerated production, and the vertical diffusion of nitrate from the thermocline structure were investigated. From the vertical distribution of chlorophyll a, subsurface maxima were observed near the thermorline at most stations including the frontal zone, except at the southern stations where the maximum chloropyll a concentration occurred at the surface, The nanophytoplankton was the most dominant fraction comprising $83.5\%$ of total phytoplankton cell numbers, but netphytoplankton were common at the southern stations where the dominant species were Rhizosolenia sp. Nitrogenous new production and regenerated productions were measured using the stable isotope $^{15}N$ nitrate and ammonia uptake method. The vertically integrated nitrogen production varied between 8.470 and $72.945\;mg\;N\;m^{-2}\;d^{-1}$. The f-ratio, which is the traction of new production from primary production, waried between 0.03 and 0.72, indicating that $3\%$ to $72\%$ of primary production was supported by the input of nutrients from below the euphotic zone and the rest are supported by ammonia recycled within the euphotic layer. This range of f-ratio encompasses from extremely oligotrophic to eutrophic area characteristics. The differences in productivity and f-ratio among stations were related to frontal structure and the bottom topography. The values were high near the frontal zone and low outside of it, and the station near Ulleng Island showed the highest f-ratio. Vertical diffusion coefficients were calculated from both the water column stability (Kz-1) of King and Devol's equation (1979) and new nitrogen requirement (Kz-2). The values of Kz-2 ($0.11\~0.55\;cm^2/s$) were relatively low compared to the values reported previously.

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Nutritional Status of Continuous Ambulatory Peritoneal Dialysis Patients (지속성복막투석 환자의 영양상태에 관한 연구)

  • Park, Jin-Kyung;Son, Sook-Mee
    • Journal of Nutrition and Health
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    • v.39 no.7
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    • pp.624-640
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    • 2006
  • Dialysis patients are at risk of malnutrition not only because of losses of nutrients during peritoneal dialysis but also because of anorexia that results in inadequate nutrient intakes. The aim of this study was to estimate the nutritional status of 154 patients receiving continuous ambulatory peritoneal dialysis (CAPD), especially focused on protein-energy malnutrition and vitamin and mineral status. The mean age of the subjects was $5.12\;{\pm}\;12.4\;y$ with educational years of $12.3\;{\pm}\;0.4\;y$ for male and $9.6\;{\pm}\;0.4\;y$ for female. The mean duration of dialysis was $22.7\;{\pm}\;21.7\;mo$. The causes of renal failure included diabetes (32.7), chronic glomerulonephritis (15.0%), and hypertension (8.5%). The main complications associated with chronic renal failure were hypertension (86.1%), diabetes (35.4%) and liver disease (9.0%). The mean daily energy intake was $1216.8\;{\pm}\;457.3\;kcal$ and increased to $1509.2\;{\pm}\;457.2\;kcal$ when added with the energy from dextrose in dialysate. The latter was still much lower than estimated energy requirement but energy intake per kg of body weight (28.1 kcal/1 g) was within the range of that recommended for CAPD patients' diet therapy (25 - 30 kcal/kg). The average daily intake of protein was $49.2\;{\pm}\;25.1\;g$ with 37.6% of the patients showing their intakes less than Estimated Average Requirement. The average protein intake per kg of weight was 0.9 g/kg, which is less than that recommended for CAPD patients (1.2-1.5g/kg) with mean serum albumin level $3.2\;{\pm}\;0.5\;g/dl$. The proportion of the patients with dietary calcium intake less than EAR was 90.9%, but when added with supplementary calcium (phosphorus binder), most patients showed their total calcium intake between EAR and UL. Fifty percent of the patients were observed with dietary iron intake less than EAR, however most patients revealed their total iron intake with supplementation above UL. The addition of folic acid with supplementation increased mean total folic intake to $1126.0\;{\pm}\;152.4\;{\mu}g$ and ninety eight percent of the subjects showed their total folic acid intake above UL. The prevalence of anemia was 83.1 % assessed with hemoglobin level, even with high intakes of iron with supplementation. Thirty four percent of the patients showed their fasting blood glucose was not under control $(\geq\;126\;mg/dl)$ even with medication or insulin probably due to dextrose from dialysate. The mean blood lipid levels were within the reference levels of hyperlipidemia, but with 72.1 % of the patients showing lower HDL-C. In conclusion, Fairly large proportion of the patients were observed with protein malnutrition with low intake of protein and serum albumin level. Few patients showed their vitamins and minerals intake less than EAR with supplementation. For iron and folic acid, their intakes were increased to above UL for large proportion of he patients. However, more than eighty percent of the patients were still anemic associated with decreased renal function. The serum blood glucose and lipid level were not under control for some patients with medication. It seems that supplementation and medications that patients are taking should be considered for dietary consulting of CAPD patients.

A Study on The Relation between Psychological Stress and Stress Hormone, Nutritional Status of Patients with Non-Insulim Dependent Diabetes Mellitus (인슐린 비의존형 당뇨병 환자의 심리적 스트레스와 스트레스호르몬 및 영양상태와의 상관관계에 관한 연구)

  • 문수재
    • Journal of Nutrition and Health
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    • v.29 no.8
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    • pp.889-898
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    • 1996
  • This study estimated the re!ation between psychological stress and stress hormones, nu­t tritional status of patients with non-insulin dependent diabetes mellitus(NIDDM). Psycho­l logical stress such as depr'ession and anxiety in 34 diabetics was analyzedin relation to nutrient intake, blood components such as fasting blood sugar(FBS), hemoglobin AIC, stress hormones a and amino acids. The IeveIs of depression and anxiety were measured by The center for ep­i idemiological studies-depc$pm$111.49pg/ml for total catecholamine ( (norepmephrine and epinephrine) and 233.95 $pm$73.99pg/ml for norepinephrine, 94.03$pm$75. 9 97pg/ml for epinephrine, 13.lS$pm$5.55pl/dl for cortisol and 171.50$pm$62.50pg/ml for gul c cagon respectlveIy. The leveIs of stress hormones in diabetics such as total catecholamine, norep­i mephrine cortisol and glucagon were significantly higher than those in normal control. The lev­el of epmephrine was higher in diabetics but the diffierenee was not significant. The calorie m t take in diabetics was 1762$pm$292keal which is S1.4% lower than Korean recommended dietary a allowances(RDA). Calcium intake was slightly low but other nutrients intakeswere higher than R RDA. The value of fasting blood sugar(FBS), usual fasting blood sugar(usual FBS) which refteet a average FBS during 3 months and hemoglobin Al C in diabetics was 1S4.1S$pm$74.22mg/dl, 177.76$pm$42.77mg/dl and S.S4$pm$2.S2% respec디VeIy. The distribution of plasma amino acids in d diabetics was generally in the normal range. The leveI of anxiety in diabetics was positively cor­related with norepinephrine, concentration and usual FBS. The levels of glucagon, usual FBS a and hemoglobin Aj C were pOSI디veIy correlated with the branched chaimamino acid(BCM : leucme, isoleucme and valine)

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A Comparative Study on the Electrophysiological Properties of Medial and Lateral Spinoreticular Tract Cells in Cats (고양이의 내측 및 외측 척수망상로 세포의 전기생리학적 비교연구)

  • Lee, Suk-Ho;Jun, Jae-Yeol;Park, Choon-Ok;Goo, Yong-Sook;Kim, Jun;Sung, Ho-Kyung
    • The Korean Journal of Physiology
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    • v.24 no.1
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    • pp.181-194
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    • 1990
  • Antidromically activated spinoreticular tract (SRT) cell units in the lumbosacral enlargement of ${\alpha}-chloralose$ anesthetized cats were classified as medial and lateral SRT units according to the location of their axonal termination. Identified SRT units were tested fer antidromic conduction velocity, laterality of their axonal projection, the location in spinal gray, peripheral receptive field, the response pattern to graded mechanichal stimulation and the responsiveness to $A{\delta}$ and C volley of the peripheral nerve. 1) The 59% of 34 medial SRT units were recorded in ipsilateral side to the antidromic stimulation site, but 60% of the 47 lateral SRT units projected to contralateral side. 2) Most of the medial SRT cells and rostral ventrolateral medulla (RVLM)-projecting lateral SRT cells were recorded in lamina VII & VIII. The LRN (lateral reticular nucleus)-projecting SRT cells, however, distributed through all the laminae except superficial ones (I & II). 3) The identified SRT units were classified as low theshold (LT), deep, high threshold (HT), wide dynamic range (WDR) cells, based on the response patterns to graded mechanical stimuli. The proportion of SRT units which receive noxious input was 37.5%, 25% and 75% in the medial, LRN-projecting and RVLM SRT group, respectively. 4) There was no significant difference in the mean conduction velocities between the 3 groups. But the deep cells had significantly higher velocity than that of the HT cells. The above results show that the peripheral inputs to the SRT units are different in the 3 groups: medial, LRN & RVLM SRT group. Especially in case of the SRT cells projecting to RVLM which is a probable candidate fur the integration center of various pressor reflexes such as somatosympathetic reflex, the noxious informations occupy higher proportion of input to them than in other groups. Therefore the noxious information transmitted through the lateral SRT destined for RVLM is expected to play a role in somatosymapthetic reflex.

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A study on the fixation of heavy metals with modified soils in the landfill liner (개량혼합토를 이용한 폐기물 매립지 차수층의 중금속 고정능력에 관한 연구)

  • 노회정;이재영
    • Journal of Soil and Groundwater Environment
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    • v.7 no.2
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    • pp.63-71
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    • 2002
  • The authors selected the modified soil method, and then performed the geotechnical and environmental laboratory test, and evaluated whether the modified soil liner could be accepted as a barrier layer in landfill. Unlike the results of the natural soil(CL), those of the hydraulic conductivity test of stabilized soil met the standard value. According to these results, the optimal mixing ratio of a mixture(cement : bentonite : stabilizing agent) was 90 : 60 : 1 with mass ratio(kg) for 1㎥ with soil, and it was possible to use poor quality bentonite. B\circled2 because of a little difference from results with high quality bentonite. B\circled1. The Cation Exchange Capacity(CEC) of the modified soil was increased about 1.5 times compared with the natural soil; however. the change of CEC with a sort of additives was not detected. In order to observe the change of the chemical components and crystal structures, the natural and the modified soils with the sorts of additives were measured by the XRF(X-Ray Flourescence Spectrometer) and SEM, but there was no significant change. The artificial leachate with the heavy meals ($Pb^{2+}$ , $Cu^{2+}$, $Cd^{2+}$ Zn$^{2+}$ 100mg/L) was passed through the natural soil and modified soils in columns. In the natural soil, Cd$^{2+}$ and $Zn^{2+}$ were identified, simultaneously the pH of outflow was lower, and then came to the breakthrough point. The removal efficiency of the natural soil was showed in order of following : $Pb^{2+}$$Cu^{2+}$ > $Zn^{2+}$ > $Cd^{2+}$ On the other hand, modified soils were not showed the breakthrough condition like the result of the natural soil. The modified soil with the lower quality bentonite, B\circled2(column3) was more stable with respect to chemical attack than that with the higher bentonite, B\circled1(column2) because the change range of outflow pH in columns was less than that of outflow pH in column2. In addition, the case of adding the stabilizing agent(column4) was markedly showed the phenomena.ena.