• Title/Summary/Keyword: long SRT

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One-year experience of oral substrate reduction therapy in three patients with Gaucher disease type I

  • Sohn, Young Bae;Kim, Yewon;Moon, Ji Eun
    • Journal of Genetic Medicine
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    • v.17 no.2
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    • pp.62-67
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    • 2020
  • Purpose: Eliglustat is an oral substrate reduction therapy (SRT) approved for adults with Gaucher disease type I (GD1) who are extensive, intermediate, or poor CYP2D6 metabolizers. Here we report one-year experience of eliglustat switch therapy from long-term enzyme replacement therapy (ERT) in three adult patients with GD1. Materials and Methods: Medical history, clinical (hemoglobin concentration, platelet count, and bone mineral density) and biochemical parameters (angiotensin converting enzyme, total acid phosphatase, and lyso-gb1) of the patients were collected and evaluated by retrospective review of medical records at every 3, 6, or 12 month after switch to SRT. Results: Patient 1 was a 43-year old female diagnosed GD1 and her clinical and biochemical parameters were stabilized for more than 20 years by ERT. Due to the burden of regular hospital visit, she switched to SRT. During one-year of SRT, clinical parameters and biomarkers were maintained stable. However, after suffering acute febrile illness during SRT, she decided to re-switch to ERT due to concerns about drug interaction. Patient 2 was 41-year old male, younger brother of patient 1 and Patient 3 was 31-year old male. They switched to SRT in clinically stable condition with long-term ERT. The one-year SRT was tolerable without specific safety issue and the clinical parameters were maintained stable. Conclusion: One-year eliglustat therapy in three adult patients with GDI was generally tolerable and effective for maintaining the clinical parameters and biomarkers. However, the drug compliance, concurrent drug interactions, and long-term safety of eliglustat should be carefully monitored.

A Flexibility Assessment of Hamstring Muscle Length in 10year old Children as Affected the Long-sitting Position (1개 초등학교 3학년생의 슬괵근 유연성 평가)

  • Chu Min;Kwon Gy-Joon;Kang Sung-Guk;Kwon Hwal-Ran;Park Dong-Bin;Park Jeon-Jung
    • The Journal of Korean Physical Therapy
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    • v.12 no.1
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    • pp.73-78
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    • 2000
  • The Sit-and-Reach Test (SRT) is commonly nea to assess flexibility of the spine and length of the hamstring muscle, The purpose of this study was to describe hamstring muscle length as reflected by use of the SRT, the Hip Joint Angle (HJA) and Sack length(from C7 to S2) in children, to examine the correlation between Back length, SRT and HJA measurements and to examine gender differences. The 162 subjects were participated without known musculoskeletal and neurological impairments of their spine or lower exeremities. In the Long-silting position, the Back length, SRT and HJA measurements were obtained. A mean Back length value of 46.2cm, a mean SRT value of 29.4cm and a mean HJA of 77,0 degrees were obtained including all subjects. There was a strong correlation between the SRT and HJA measurements (r=.66). There were a difference between boys and girls in Back(p=.0019) and HJA measurements (p=.015). The results of this study suggest that measurements for the SRT and HJA were correlated than Back. The HJA measurements guide treatment more effectively than do Back length and SRT measurements.

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Effect of Sludge Age on the Toxicity of Cr6+, Zn2+, and Cd2+ in INT-Dehydrogenase Assay (INT-Dehydrogenase 시험법에서 슬러지 일령이 Cr6+, Zn2+ 및 Cd2+ 독성에 미치는 영향)

  • Ryu, Hong-Duck;Lee, Sang-Ill;Kim, Jong-Soo
    • Korean Journal of Environmental Agriculture
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    • v.27 no.4
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    • pp.382-388
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    • 2008
  • This study was initiated to elucidate the relation between the toxicity of $Cr^{6+}$, $Zn^{2+}$, and $Cd^{2+}$ and sludge age (Solids retention time, SRT). The effect of SRT on the toxicity of $Cr^{6+}$, $Zn^{2+}$, and $Cd^{2+}$ in activated sludge system was investigated with INT-dehydrogenase assay. Experimental results showed that the inhibitory effects of $Cr^{6+}$, $Zn^{2+}$, and $Cd^{2+}$ were reduced as the sludge age increased from SRT 5 d to SRT 25 d. It is noteworthy that the experimental results enabled to determine the relative toxicity of the tested metals depending on the sludge age. At the SRT of 5 and 9 days, the order of toxicity of the three metals to the activated sludge was $Cr^{6+}$ > $Zn^{2+}$ > $Cd^{2+}$ (the $IC_{50}$ value of $Cr^{6+}$, $Zn^{2+}$, and $Cd^{2+}$ was 16.15, 25.90, and 32.49 mg/L, respectively) and $Zn^{2+}$ > $Cr^{6+}$ > $Cd^{2+}$ (the $IC_{50}$ value of $Cr^{6+}$, $Zn^{2+}$, and $Cd^{2+}$ was 39.12, 27.93, and 42.31 mg/L, respectively), respectively. However, the toxicity of three metals was almost same at the SRT of 14 and 25 days (the range of $IC_{50}$ in SRT 14 and 25 days was from 49.80 mg/ L to 53.44 mg/L among three heavy metals). This results would be explained by that the quantity of biopolymer formed in short SRT was small, whereas that in long SRT was large. Consequently, it is recommended that sludge age be maintained at long SRT in order to avoid the toxicity inhibition of heavy metals such as $Cr^{6+}$, $Zn^{2+}$, and $Cd^{2+}$.

Long-term tolerance and outcomes for dose escalation in early salvage post-prostatectomy radiation therapy

  • Safdieh, Joseph J.;Schwartz, David;Weiner, Joseph;Weiss, Jeffrey P.;Rineer, Justin;Madeb, Isaac;Rotman, Marvin;Schreiber, David
    • Radiation Oncology Journal
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    • v.32 no.3
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    • pp.179-186
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    • 2014
  • Purpose: To study the long-term outcomes and tolerance in our patients who received dose escalated radiotherapy in the early salvage post-prostatectomy setting. Materials and Methods: The medical records of 54 consecutive patients who underwent radical prostatectomy subsequently followed by salvage radiation therapy (SRT) to the prostate bed between 2003-2010 were analyzed. Patients included were required to have a pre-radiation prostate specific antigen level (PSA) of 2 ng/mL or less. The median SRT dose was 70.2 Gy. Biochemical failure after salvage radiation was defined as a PSA level >0.2 ng/mL. Biochemical control and survival endpoints were analyzed using the Kaplan-Meier method. Univariate and multivariate Cox regression analysis were used to identify the potential impact of confounding factors on outcomes. Results: The median pre-SRT PSA was 0.45 ng/mL and the median follow-up time was 71 months. The 4- and 7-year actuarial biochemical control rates were 75.7% and 63.2%, respectively. The actuarial 4- and 7-year distant metastasis-free survival was 93.7% and 87.0%, respectively, and the actuarial 7-year prostate cancer specific survival was 94.9%. Grade 3 late genitourinary toxicity developed in 14 patients (25.9%), while grade 4 late genitourinary toxicity developed in 2 patients (3.7%). Grade 3 late gastrointestinal toxicity developed in 1 patient (1.9%), and grade 4 late gastrointestinal toxicity developed in 1 patient (1.9%). Conclusion: In this series with long-term follow-up, early SRT provided outcomes and toxicity profiles similar to those reported from the three major randomized trials studying adjuvant radiation therapy.

Relation between sludge properties and filterability in MBR: Under infinite SRT

  • Zhang, Haifeng;Wang, Bing;Yu, Haihuan;Zhang, Lanhe;Song, Lianfa
    • Membrane and Water Treatment
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    • v.6 no.6
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    • pp.501-512
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    • 2015
  • A laboratory-scale submerged membrane bioreactor (MBR) was continuously operated for 100 d at an infinite sludge retention time (SRT) with the aim of identifying possible relation between the filterability of mixed liquor and sludge properties, such as extracellular polymeric substances (EPS), soluble microbial products (SMP), viscosity of mixed liquor, zeta potential of flocs and particle size distributions (PSD). Research results confirmed that MBR can operate with a complete sludge retention ensuring good treatment performances for COD and $NH_3-N$. However, the long term operation (about 40 d) of MBR with no sludge discharge had a negative influence on sludge filterability, and an increase in membrane fouling rates with the time was observed. There as a strong correlation between the sludge filterability and the fouling rate. Among the different sludge properties parameters, the concentration SMP and EPS had a more closely correlation with the sludge filterability. The concentrations of SMP, especially SMP with MW above 10 kDa, had a strong direct correlation to the filterability of mixed sludge. The protein fractions in EPS were biodegradable and available for microorganism metabolism after about 60 days, and the carbohydrates in EPS had a significantly negative effect on sludge filterability in MBR at an infinite SRT.

An Evaluation Validity of the Silicone Replica Technique at Measurement on Fit of Fixed Dental Prostheses (고정성 보철물의 적합도 측정 시 사용하는 실리콘 복제본 기술의 정확도 평가)

  • Kim, Jae-Hong;Kim, Ki-Baek
    • Journal of dental hygiene science
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    • v.12 no.6
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    • pp.566-571
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    • 2012
  • Marginal and internal fit are very important factor for the clinical long-term success of fixed dental prostheses (FDPs). A variety of methods have been applied to the measuring marginal and internal fit of FDPs. The purpose of this study was to compare the marginal and internal fit of 2 different measuring methods silicone replica technique [SRT] and die cutting technique (DCT). SRT, the space between die and FDPs were filled with light-body silicone, then stabilized by medium-body silicone. DCT, the FDPs were cemented on definitive dies and embedded into epoxy. The silicone replicas and epoxies were sectioned mesio-distal and measured 4 locations (margin, rounded chamfer, axial, occlusal) and using a digital microscope at a magnification $\times$160. For the SRT the mean${\pm}$SDs fit (${\mu}m$) recorded were: margin $88.7{\pm}22.2$, rounded chamfer $90.6{\pm}25.0$, axial wall $61.5{\pm}21.8$, occlusal $134.9{\pm}30.8$. For the DCT the mean${\pm}$SDs fit (${\mu}m$) recorded were: margin $85.3{\pm}18.0$, rounded chamfer $85.4{\pm}24.7$, axial wall $62.0{\pm}21.3$, occlusal $131.7{\pm}30.5$. The mean${\pm}$SDs size (${\mu}m$) of SRT margin was $88.7{\pm}22.2$ and of DCT margin was $85.3{\pm}18.0$. There were no statistically significant difference (p<0.05) of the four parts between SRT and DCT. It is considered an appropriate case to use SRT at fit of FDPs.

Comparison study on membrane fouling by various sludge fractions with long solid retention time in membrane bioreactor

  • Sun, Darren Delai;Liu, Shushu
    • Membrane and Water Treatment
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    • v.4 no.3
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    • pp.175-189
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    • 2013
  • A membrane bioreactor (MBR) with sludge retention time (SRT) of 300 days was maintained for over 2 years. Polypropylene microfiltration (MF) membrane with pore size of 0.2 ${\mu}m$ was used in the MBR system. The fouling behaviors of various sludge fractions from the MBR were studied and sub-divided resistances were analyzed. It was observed that $R_{cp}$ was a dominant resistance during the filtration of activated sludge, contributing 63.0% and 59.6% to the total resistance for MBR and sequential batch reactor (SBR) respectively. On the other hand, $R_c$ played the significant role during the filtration of supernatant and solutes, varying between 54.54% and 67.18%. Compared with $R_{cp}$ and $R_c$, $R_{if}$ was negligible, and $R_m$ values remained constant at $0.20{\times}10^{12}m^{-1}$. Furthermore, resistances of all sludge fractions increased linearly with rising mixed liquor suspended solids (MLSS) concentration and growing trans-membrane pressure (TMP), while the relationship was inversed between fraction resistances and cross flow velocity (CFV). Among all fractions of activated sludge, suspended solid was the main contributor to the total resistance. A compact cake layer was clearly observed according to the field emission scanning electro microscopy (FE-SEM) images.

Clinical Outcomes of Intracranial Nonvestibular Schwannomas Treated with Linac-Based Stereotactic Radiosurgery and Radiotherapy

  • Puataweepong, Putipun;Dhanachai, Mantana;Hansasuta, Ake;Saetia, Kriangsak;Dangprasert, Somjai;Sitathanee, Chomporn;Yongvithisatid, Pornpan
    • Asian Pacific Journal of Cancer Prevention
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    • v.17 no.7
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    • pp.3271-3276
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    • 2016
  • Background: Intracranial nonvestibular schwannomas arising from various cranial nerves excluding CN VIII are uncommon. Recently, stereotactic radiosurgery (SRS) and fractionated stereotactic radiotherapy (SRT) have been widely reported as effective treatment modalities for nonvestibular schwannomas. The purpose of this study was to study the long term clinical outcome for nonvestibular schwannomas treated with both X-Knife and CyberKnife (CK) radiosurgery at one institution. Materials and Methods: From 2004 to 2013, fifty-two nonvestibular schwannoma patients were included in this study, 33 patients (63%) were treated with CK, and 19 (37%) were treated with X-Knife. The majority of the tumors were jugular foramen schwannomas (38%) and trigeminal schwannomas (27%). HSRT was given for 45 patients (86%), whereas CSRT was for 6 (12%) and SRS for 1 (2%). Results: The median pretreatment volume was $9.4cm^3$ (range, $0.57-52cm^3$). With the median follow up time of 36 months (range, 3-135), the 3 and 5 year progression free survival was 94 % and 88%, respectively. Tumor size was decreased in 13 (25%), stable in 29 (56%), and increased in 10 (19%). Among the latter, 3 (30%) required additional treatment because of neurologic deterioration. No patient was found to develop any new cranial nerve deficit after SRS/SRT. Conclusions: These data confirmed that SRS/SRT provide high tumor control rates with low complications. Large volume tumors and cystic expansion after radiation should be carefully followed up with neurological examination and MRI, because it may frequently cause neurological deterioration requiring further surgery.

Effect of Temperature on Nitritation using Effluent of Anaerobic Digester (혐기 소화조 유출수의 아질산화 반응에 온도가 미치는 영향)

  • Im, Jiyeol;Gil, Kyungik
    • Journal of Korean Society on Water Environment
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    • v.27 no.3
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    • pp.286-292
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    • 2011
  • Preparing for the Standards for Effluents which will be strengthen from 2012, many ways like remodellings and repairs of sewage treatment plant (STP) are considered. The treatment of the recycle water from the sludge treatment process contains high-strength organic compounds and nitrogen is considered as alternative. In the treatment of high-strength nitrogen, nitritation has more economic advantages than nitrification. In this study, lab-scale reactor was operated at the $35^{\circ}C$, $20^{\circ}C$ and $10^{\circ}C$ conditions using effluent of anaerobic digester to investigate the nitrogen removal by nitritation. Long-term stable nitritation was achieved at the $35^{\circ}C$, $20^{\circ}C$ but $10^{\circ}C$. In the stable nitritation states, nitrite conversion was higher at the high temperature of $35^{\circ}C$ than the room temperature of $20^{\circ}C$. Also shorter solid retention time (SRT) was needed to induce high nitrite conversion at the high temperature of $35^{\circ}C$. It was showed that temperature and SRT are important factors to induce nitritation.

Substrate reduction therapy as a new treatment option for patients with Gaucher disease type 1: A review of literatures

  • Sohn, Young Bae;Yoo, Han-Wook
    • Journal of Genetic Medicine
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    • v.13 no.2
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    • pp.59-64
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    • 2016
  • Gaucher disease type 1 (GD1) is an inherited lysosomal storage disorder caused by deficiency of acid ${\beta}$-glucosidase. The diminished enzyme activity leads to the accumulation of substrates and results in multi-systemic manifestations including hepatosplenomegaly, anemia, thrombocytopenia, and bone diseases. Enzyme replacement therapy (ERT) by infusion of recombinant protein has been the standard treatment for over 20 years. Despite the successful long-term treatment with ERT, several unmet needs remain in the treatment of GD1 such as severe pulmonary and skeletal manifestations. Substrate reduction therapy (SRT) reduces the accumulation of substrates by inhibiting their biosynthesis. Eliglustat, a new oral SRT, was approved in United States and Europe as a first-line therapy for treating adult patients with GD1 who have compatible CYP2D6 metabolism phenotypes. Although eliglustat is not yet available in Korea, introduction and summary of this new treatment modality are provided in this paper by review of literatures. Despite the fact that there are only limited studies to draw resolute conclusions, the current data demonstrated that eliglustat is not inferior to ERT in terms of its clinical efficacy. The approval of eligustat enables eligible adult GD1 patients to have the option of oral therapy although it still needs further studies on long-term outcomes. The individual patient should be assessed carefully for the choice of treatment modality when eliglustat becomes available in Korea. Furthermore, the clinical guidelines for Korean patients with GD1 regarding the use of eliglustat needs to be developed in near future.