Yang, Sungmin;Choi, Hyo Won;Kang, Yun Koo;Lee, Jin-Sung;Namgoong, Mee Kyung
Journal of The Korean Society of Inherited Metabolic disease
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v.20
no.2
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pp.55-62
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2020
A 22-month-old girl who had taken iron supplements due to iron deficiency anemia, presented bloody mucoid stool for one month. She had a bruise at the right periorbital area due to minor trauma and hepatosplenomegaly. Laboratory studies showed anemia, thrombocytopenia, elevated alkaline phosphatase (ALP), hypophosphatemia, decreased haptoglobin, hypocomplementemia, negative direct/indirect Coomb's test, normal vitamin D3 level and high PTHi. Wrist x-ray showed no signs of rickets. The abdominal ultrasound showed only accessory spleen. Tandem mass spectrometry was normal. During follow up, bloody stool regressed after seven days of withdrawal of iron supplement and cow milk, and the total CO2 level had been within 15-20 mEq/L with normal anion gap. NGS (next generation sequencing) panel test for evaluation of renal tubular acidosis showed negative results. After low dose steroid and vitamin D supplements under the impression of hypocomplementemic vasculitis, thrombocytopenia, C3/C4, decreased haptoglobin, and elevated ALP level became normal. At 57 months of age, laboratory findings showed elevated liver enzyme, ALP and gamma-glutamyl transferase again. And liver cirrhosis with splenomegaly and diffuse renal disease were reported with abdomen CT scan. Liver biopsy reported macro- and micronodular cirrhosis. Urine organic acid profile showed elevated succinylacetone level. Whole exome sequencing revealed novel compound heterozygous mutations (NM_00137.2:c.107T>C, NM_00137, 2:c.614T>C) in FAH gene and confirmed by Sanger sequencing. Consequently, the patient was diagnosed as chronic hereditary tyrosinemia type I. She started low phenylalanine/tyrosine diet and nitisinone treatment. Our case had presented symptoms very slowly, which is the first case of chronic tyrosinemia type I in South Korea.
As medical facilities are usually built at urban areas, special concrete aggregates and evaluation methods are needed to optimize the design of concrete walls by balancing density, thickness, material composition, cost, and other factors. Carbon treatment rooms require a high radiation shielding requirement, as the neutron yield from carbon therapy is much higher than the neutron yield of protons. In this case study, the maximum carbon energy is 430 MeV/u and the maximum current is 0.27 nA from a hybrid particle therapy system. Hospital or facility construction should consider this requirement to design a special heavy concrete. In this work, magnetite is adopted as the major aggregate. Density is determined mainly by the major aggregate content of magnetite, and a heavy concrete test block was constructed for structural tests. The compressive strength is 35.7 MPa. The density ranges from 3.65 g/cm3 to 4.14 g/cm3, and the iron mass content ranges from 53.78% to 60.38% from the 12 cored sample measurements. It was found that there is a linear relationship between density and iron content, and mixing impurities should be the major reason leading to the nonuniform element and density distribution. The effect of this nonuniformity on radiation shielding properties for a carbon treatment room is investigated by three groups of Monte Carlo simulations. Higher density dominates to reduce shielding thickness. However, a higher content of high-Z elements will weaken the shielding strength, especially at a lower dose rate threshold and vice versa. The weakened side effect of a high iron content on the shielding property is obvious at 2.5 µSv=h. Therefore, we should not blindly pursue high Z content in engineering. If the thickness is constrained to 2 m, then the density can be reduced to 3.3 g/cm3, which will save cost by reducing the magnetite composition with 50.44% iron content. If a higher density of 3.9 g/cm3 with 57.65% iron content is selected for construction, then the thickness of the wall can be reduced to 174.2 cm, which will save space for equipment installation.
This study examined the chemical composition of lotus root and functionally evaluated a fermented lotus root drink. Electron-donating ability using DPPH along with nitrite-scavenging ability were used to compare the antioxidative activities of unfermented and fermented lotus root drinks. The electron-donating abilities of the unfermented lotus root drink (1%) and fermented lotusroot drink (1%) were 22.55% and 23.88%, respectively. At pH 6.0, the nitrite-scavenging abilities of the unfermented lotus root drink and the fermented lotus root drink (100%) were 27.64% and 40.3%, respectively, and their scavenging ability increased in a dose-dependent manner at all pH values. In order to study the anti-obesity effects of the two drinks, male Sprague-Dawley rats were divided into four groups (A: basal diet, B: high fat diet, C: high fat diet+unfermented lotus root drink, D: high fat diet+fermented lotus root drink). Net weight gains were not significantly different among the four groups. Plasma total cholesterol concentrations significantly decreased in the groups receiving the unfermented and fermented lotus root drinks. Also, plasma total lipid and triglyceride contents were lower in the groups receiving the unfermented and fermented lotus root drinks as compared to the high fat diet group; however, the differences among the three groups were not significant.
Jun Sung Bae;Chae Won Lee;Chan Yeong Yang;Eun Ha Jeong;Areum Kim;Young-Sik Chae;Jung-Jin Park;Kwan Ha Park
Journal of fish pathology
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v.36
no.2
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pp.293-302
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2023
This study evaluated the acute toxicity induced by oxolinic acid (OA), neomycin-oxytetracycline combination (NEO-OTC) and florfenicol (FF) administered to guppy (Poecilia reticulata) by comparing standard formulations with commercial preparations (COOaqua curinpotion, COOaqua terafinpotion and COOaqua profenpotion, respectively) for ornamental fish at concentrations of 2-4%. NEO-OTC exhibited the highest acute toxicity in guppy, with no difference observed between the pstandard formulation and commercial preparation (LC50 = 126.08 mg/L and 112.44 mg/L, respectively). OA acute toxicity was assessed in the form of sodium salt, with an LC50 of 504.61 mg/L for the standard formulation and a slightly increased toxicity of 316.11 mg/L for the commercial preparation. In contrast, no mortality was observed during the 96-hour exposure period with the standard formulation in the form of oxolinic acid. The acute toxicity of FF was measured to be above 1,000 mg/L for the standard formulation; however, the commercial preparation significantly increased to 158.53 mg/L. These results indicate that toxicity can significantly increase in commercial formulations, especially those with low levels of active ingredients. This is presumed to be attributed to the organic solvents or solubilizing agents present in the commercial preparations, which may enhance toxicity. Additionally, guppy infected with Aeromonas salmonicida were effectively protected against mortality by administering OA, NEO-OTC and FF at concentrations of 50 mg/L, 100 mg/L and 15 mg/L, respectively, for 2 hours and at half the dose for 24 hours. This result indicates that liquid formulations containing low concentrations of antibiotics may partially increase toxicity, but there is no problem in effectively treating diseases in ornamental fish.
[ $\underline{Purpose}$ ]: To determine the patterns of evaluation and treatment in patients with breast cancer after mastectomy and treated with radiotherapy. A nationwide study was performed with the goal of improving radiotherapy treatment. $\underline{Materials\;and\;Methods}$: A web- based database system for the Korean Patterns of Care Study (PCS) for 6 common cancers was developed. Randomly selected records of 286 eligible patients treated between 1998 and 1999 from 17 hospitals were reviewed. $\underline{Results}$: The ages of the study patients ranged from 20 to 80 years (median age 44 years). The pathologic T stage by the AJCC was T1 in 9.7% of the cases, T2 in 59.2% of the cases, T3 in 25.6% of the cases, and T4 in 5.3% of the cases. For analysis of nodal involvement, N0 was 7.3%, N1 was 14%, N2 was 38.8%, and N3 was 38.5% of the cases. The AJCC stage was stage I in 0.7% of the cases, stage IIa in 3.8% of the cases, stage IIb in 9.8% of the cases, stage IIIa in 43% of the cases, stage IIIb in 2.8% of the cases, and IIIc in 38.5% of the cases. There were various sequences of chemotherapy and radiotherapy after mastectomy. Mastectomy and chemotherapy followed by radiotherapy was the most commonly performed sequence in 47% of the cases. Mastectomy, chemotherapy, and radiotherapy followed by additional chemotherapy was performed in 35% of the cases, and neoadjuvant chemoradiotherapy was performed in 12.5% of the cases. The radiotherapy volume was chest wall only in 5.6% of the cases. The volume was chest wall and supraclavicular fossa (SCL) in 20.3% of the cases; chest wall, SCL and internal mammary lymph node (IMN) in 27.6% of the cases; chest wall, SCL and posterior axillary lymph node in 25.9% of the cases; chest wall, SCL, IMN, and posterior axillary lymph node in 19.9% of the cases. Two patients received IMN only. The method of chest wall irradiation was tangential field in 57.3% of the cases and electron beam in 42% of the cases. A bolus for the chest wall was used in 54.8% of the tangential field cases and 52.5% of the electron beam cases. The radiation dose to the chest wall was $45{\sim}59.4\;Gy$ (median 50.4 Gy), to the SCL was $45{\sim}59.4\;Gy$ (median 50.4 Gy), and to the PAB was $4.8{\sim}38.8\;Gy$, (median 9 Gy) $\underline{Conclusion}$: Different and various treatment methods were used for radiotherapy of the breast cancer patients after mastectomy in each hospital. Most of treatment methods varied in the irradiation of the chest wall. A separate analysis for the details of radiotherapy planning also needs to be followed and the outcome of treatment is needed in order to evaluate the different processes.
After cardiac surgery, it has been recognized that various complications were associated with injured humoral and cellular immunity by cardiopulmonary bypass(CPB). Especially, in postoperative pulmonary dysfunction, transpulmonary leukostasis followed complement activation and inflammatory responses are major pathogen. Some studies have showed that pretreated-corticosteroids before CPB protected postoperative pulmonary dysfunction. Corticosteroids may inhibit complement and leukocyte activation. On based previous studies, present investigator determined changes of leukocyte counts and transpulmonary leukostasis during cardiac surgery and postoperative periods. For the evaluation of postoperative pulmonary function and edema, $PaO_2$ and chest X-ray were compared between pre-CPB and post-CPB. Fever and other parameters were also observed postoperatively. The aim of this study was to define for the prophylactic effects of corticosteroid(Solu-Medrol: 30mg/kg) on all the researched parameters. This study was prospectively designed with randomized-blind fashion for 50 patients undergoing cardiac surgery. According to the purpose of study, all patients were divided into placebo and steroid group. : Placebo group was 25 patients received normal saline(not corticosteroid), and steroid group was 25patients received corticosteroid(Solu-Medrol: 30mg/kg) before initiation of CPB. The results of study were summarized as follows. 1. Total peripheral leukocyte counts decreased significantly at 5 minutes of CPB in all patients(P<0.01), and began to increase progressively at later periods of CPB with neutrophilia. The significant rise remained at postoperative 7th day(P<0.05). 2. During partial CPB, transpulmonary leukostasis occurred in placebo group(P<0.001), whereas it was prevented in steroid group. 3. In both groups, peripheral lymphocyte counts were stable during CPB, but began to reduce at time of intensive care unit(ICU) and the lymphocytopenia remained until postoperative 3rd day. The lymphocyte counts recovered on postoperative 7th day. 4. In both groups, peripheral counts of monocyte were relatively stable in the early peroid of CPB, and increased gradually in the later periods of CPB. This significant monocytosis remained throughout postoperlative periods(P<0.05). 5. The mean value of postoperative $paO)_2$ was lower than that of pre-CPB in placebo group(P=0.01) but didn't significant in steroid group(P=0.90). In the incidence of pulmonary edema signs and fever, placebo group was higher than steroid group(P=0.001, p=0.01, respectively). However mechanical respiratory supporting and care periods at intensive care unit were not significant difference between two groups(P>.0.05).With the above results, the investigator concluded that leukocyte activation and pulmonary sequestration were caused by cardiac surgery with CPB and demonstrated that high dose corticosteroid will provide prophylactic effect for pulmonary leukostasis and higher neutrophilia. These effects may ameliorate postoperative pulmonary dysfunction and contribute to postoperative less morbidity. However, further study should be performed because postoperative lymphocytopenia continued for 3 days in both groups, which may suspected damage or suppression of cell-mediated immunity with used corticosteroid.
Yoo Jeong Hyun;Kim Sung Sook;Lee Kyung Ja;Rhee Chung Sik
Radiation Oncology Journal
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v.15
no.2
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pp.79-95
/
1997
Purpose : Phospholipase C(PLC) isozymes play significant roles in signal transduction mechanism. $PLC-\gamma$ 1 is one of the key regulatory enzymes in signal transduction for cellular proliferation and differentiation. Ras oncoprotein, EGFR, and PKC are also known to be involved in cell growth. The exact mechanisms of these signal transduction following irradiation, however, were not clearly documented Thus, this study was Planned to determine the biological significance of PLC, ras oncoprotein, EGFR, and PKC in damage and regeneration of rat intestinal mucosa following irradiation. Material and Method : Sixty Sprague-Dawley rats were irradiated to entire body with a single dose of 8Gy. The rats were divided into S groups according to the sacrifice days after irradiation. The expression of PLC, ras oncoprotein, EGFR and PKC in each group were examined by the immunoblotting and immunohistochemistry. The histopathologic findings were observed using H&I stain, and the mitoses for the evidence of regeneration were counted using the light microscopy & PCNA kit. The Phosphoinositide(PI) hydrolyzing activity assay was also done for the indirect evaluation of $PLC-\gamma$ 1 activity. Results: In the immunohistochemistry , the expression of $PLC-{\beta}$ was negative for all grøups. The expression of $PLC-{\gamma}1$ was highest in the group III followed by group II in the proliferative zone of mucosa. The expression of $PKC-{\delta}1$ was strongly positive in group 1 followed by group II in the damaged surface epithelium. The above findings were also confirttled in the immunoblotting study. In the immunoblotting study, the expressions of $PLC-{\beta}$, $PLC-{\gamma}1$, and $PKC-{\delta}1$ were the same as the results of immunohis-tochemistry. The expression of ras oncoprctein was weakly positive in groups II, III and IV. The of EGFR was the highest in the group II, III, follwed by group IV and the expression of PKC was weakly positive in the group II and III. Conclusion: $PLC-{\gamma}1$ mediated signal transduction including ras oncoprotein, EGFR, and PKC play a significant role in mucosal regeneration after irradiation. $PLC-{\delta}1$ mediated signal transduction might have an important role in mucosal damage after irradiation. Further studies will be necessary to confirm the signal transduction mediating the $PKC-{\delta}1$.
Purpose: To study the effect of recombinant human epidermal growth factor (rhEGF) on oral mucositis induced by cisplatin and radiotherapy in a mouse model. Materials and Methods: Twenty-four ICR mice were divided into three groups-the normal control group, the no rhEGF group (treatment with cisplatin and radiation) and the rhEGF group (treatment with cisplatin, radiation and rhEGF). A model of mucositis induced by cisplatin and radiotherapy was established by injecting mice with cisplatin (10 mg/kg) on day 1 and with radiation exposure (5 Gy/day) to the head and neck on days $1{\sim}5$. rhEGF was administered subcutaneously on days -1 to 0 (1 mg/kg/day) and on days 3 to 5 (1 mg/kg/day). Evaluation included body weight, oral intake, and histology. Results: For the comparison of the change of body weight between the rhEGF group and the no rhEGF group, a statistically significant difference was observed in the rhEGF group for the 5 days after day 3 of. the experiment. The rhEGF group and no rhEGF group had reduced food intake until day 5 of the experiment, and then the mice demonstrated increased food intake after day 13 of the of experiment. When the histological examination was conducted on day 7 after treatment with cisplatin and radiation, the rhEGF group showed a focal cellular reaction in the epidermal layer of the mucosa, while the no rhEGF group did not show inflammation of the oral mucosa. Conclusion: These findings suggest that rhEGF has a potential to reduce the oral mucositis burden in mice after treatment with cisplatin and radiation. The optimal dose, number and timing of the administration of rhEGF require further investigation.
[ $\underline{Purpose}$ ]: This study analyzed the tumor response, overall survival, progression free survival and related prognostic factors in patients with muscle invasive bladder cancer subjected to bladder preserving treatment. $\underline{Materials\;and\;Methods}$: Between August 1995 and June 2004, 37 patients with muscle invasive (transitional cell carcinoma, clinically stage T2-4) bladder cancer were enrolled for the treatment protocol of bladder preservation. There were 33 males and 4 females, and the median age was 67 years (range $38{\sim}86\;years$). Transurethral resection of the bladder (TURB) was performed in 17 patients who underwent complete resection. The median radiation dose administered was 64.8 Gy (range $55.8{\sim}67\;Gy$). The survival rate was calculated by the Kaplan-Meier method. $\underline{Results}$: An evaluation of the response rate was determined by abdomen-pelvic CT and cystoscopy at three months after radiotherapy. A complete response was seen in 17 patients (46%). The survival rate at three years was 54.7%, with 54 months of median survival (range $3{\sim}91$ months). During the study, 17 patients died and 13 patients had died from bladder cancer. The progression free survival rate at three years was 37.2%. There were 24 patients (64.9%) who had disease recurrence: 16 patients (43.2%) had local recurrence, 6 patients (16.2%) had a distant recurrence, and 2 patients (5.4%) had both a local and distant recurrence. The survival rate (p=0.0009) and progression free survival rates (p=0.001) were statistically significant when compared to the response rate after radiotherapy. $\underline{Conclusion}$: The availability of complete TURB and appropriate chemoradiotherapy were important predictors for bladder preservation and survival.
Kim, Jia;Hong, Gun Chul;Lee, Hyeok;Choi, Seong Wook
The Korean Journal of Nuclear Medicine Technology
/
v.18
no.1
/
pp.43-48
/
2014
Purpose: In the PET/CT images, The SUV (standardized uptake value) enables the quantitative assessment according to the biological changes of organs as the index of distinction whether lesion is malignant or not. Therefore, It is too important to enter parameters correctly that affect to the SUV. The purpose of this study is to evaluate an allowable error range of SUV as measuring the difference of results according to input errors of Activity, Weight, uptake Time among the parameters. Materials and Methods: Three inserts, Hot, Teflon and Air, were situated in the 1994 NEMA Phantom. Phantom was filled with 27.3 MBq/mL of 18F-FDG. The ratio of hotspot area activity to background area activity was regulated as 4:1. After scanning, Image was re-reconstructed after incurring input errors in Activity, Weight, uptake Time parameters as ${\pm}5%$, 10%, 15%, 30%, 50% from original data. ROIs (region of interests) were set one in the each insert areas and four in the background areas. $SUV_{mean}$ and percentage differences were calculated and compared in each areas. Results: $SUV_{mean}$ of Hot. Teflon, Air and BKG (Background) areas of original images were 4.5, 0.02. 0.1 and 1.0. The min and max value of $SUV_{mean}$ according to change of Activity error were 3.0 and 9.0 in Hot, 0.01 and 0.04 in Teflon, 0.1 and 0.3 in Air, 0.6 and 2.0 in BKG areas. And percentage differences were equally from -33% to 100%. In case of Weight error showed $SUV_{mean}$ as 2.2 and 6.7 in Hot, 0.01 and 0.03 in Tefron, 0.09 and 0.28 in Air, 0.5 and 1.5 in BKG areas. And percentage differences were equally from -50% to 50% except Teflon area's percentage deference that was from -50% to 52%. In case of uptake Time error showed $SUV_{mean}$ as 3.8 and 5.3 in Hot, 0.01 and 0.02 in Teflon, 0.1 and 0.2 in Air, 0.8 and 1.2 in BKG areas. And percentage differences were equally from 17% to -14% in Hot and BKG areas. Teflon area's percentage difference was from -50% to 52% and Air area's one was from -12% to 20%. Conclusion: As shown in the results, It was applied within ${\pm}5%$ of Activity and Weight errors if the allowable error range was configured within 5%. So, The calibration of dose calibrator and weighing machine has to conduct within ${\pm}5%$ error range because they can affect to Activity and Weight rates. In case of Time error, it showed separate error ranges according to the type of inserts. It showed within 5% error when Hot and BKG areas error were within ${\pm}15%$. So we have to consider each time errors if we use more than two clocks included scanner's one during the examinations.
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