• Journal of The Korean Society of Inherited Metabolic disease
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• v.20 no.2
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• pp.37-43
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• 2020

Phenylketonuria is the most prevalent disorder caused by an inborn error in aminoacid metabolism. It results from mutations in the phenylalanine hydroxylase (PAH) gene. If untreated or late treated, results in profound and irreversible mental disability. Newborn screening test identify patients with phenylketouria. The early initiation of a phenylalanine restricted diet very soon prevents most of the neuropsychiatric complications. However, the diet therapy is difficult to maintain and compliance is poor, especially in adolescents and adulthood. Since 2015, American Medical College of Medical Genetics and Genomics (ACMG) recommended more strong restrictive diet therapy for target blood level of phenylalanine (<360 umol/L). For over four decades the only treatment was a very restrictive low phenylalanine diet. This changed in 2007 with the approval of cofactor therapy (Tetrahydrobiopterin, BH4) which is effective in up to 30% of patients. Data from controlled clinical trials with sapropterin dihydrochloride indicate a similar occurrence of all-cause adverse events with this treatment and placebo. Large neutral aminoacids (LNAA) competes with phenylalanine for transport across the blood-brain-barrier and have a beneficial effect on executive functioning. A new therapy has just been approved that can be effective in most patients with PAH deficiency regardless of their degree of enzyme deficiency or the severity of their phenotype. Phenylalanine ammonia lyase (PAL-PEG) was approved in the USA by FDA in May of 2018 for adult patients with uncontrolled blood phenylalanine concentrations on current treatment. Nucleic acid therapy (therapeutic mRNA or gene therapy) is likely to provide longer term solutions with few side effects.

• Imaging Science in Dentistry
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• v.51 no.2
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• pp.117-127
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• 2021

Purpose: The aim of this study was to evaluate the volumetric characteristics of mucous retention cysts(MRCs) in the maxillary sinus and to analyze potential associations of MRCs with dentoalveolar pathologies. Materials and Methods: Cone-beam computed tomography (CBCT) scans exhibiting bilateral maxillary sinuses that were acquired from January 2016 to February 2019 were initially screened. A total of 227 scans(454 sinuses) that fulfilled the inclusion criteria were included. The presence, location, and volumetric characteristics of the diagnosed MRCs were evaluated on CBCT images using the 3D-Slicer software platform. The presence of MRCs was correlated with potential influencing factors including age, sex, and dentoalveolar pathology. For MRCs located on the sinus floor, factors with a potential impact on the volume, surface, and diameter were analyzed. Results: An MRC was present in 130 (28.6%) of the 454 sinuses. Most MRCs were located on the sinus walls and floor. The mean MRC volume, surface, and diameter were 551.21±1368.04 mm³, 228.09±437.56 mm², and 9.63±5.40 mm, respectively. Significantly more sinuses with associated endodontically treated teeth/periapical lesions were diagnosed with an MRC located on the sinus floor. For MRCs located on the sinus floor, endodontic status exhibited a significant association with increased volume, surface, and diameter. Conclusion: Periapical lesions might be a contributing factor associated with the presence and volume of MRCs located on the sinus floor. The 3D-Slicer software platform was found to be a useful tool for clinicians to analyze the size of MRCs before surgical interventions such as sinus floor elevation procedures.

• Journal of Veterinary Clinics
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• v.38 no.3
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• pp.163-168
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• 2021

A 6-year-old, spayed female, Maltese dog with tachypnea and dry cough was presented to Gyeongsang National University Veterinary Medical Teaching hospital. On physical examination, its respiration rate was 132 per minute. Decreased partial pressure of oxygen, partial pressure of carbon dioxide, and hyperlactatemia were found on arterial blood gas analysis. Its diastolic blood pressure was 80 mmHg. Auscultation revealed arrhythmia. Electrocardiogram revealed P pulmonale, P mitrale, and ventricular premature complexes. Thoracic radiographs revealed mild enlargement of both atrium and moderate enlargement of the left ventricular. There was also a moderate alveolar pattern in the right and caudal part of the left cranial lung lobe. Two-dimensional echocardiography showed enlargement of generalized four chambers without remarkable findings of valvular degeneration. M-mode echocardiography showed decreased left ventricular fractional shortening and enlarged left ventricular internal diameter at both end-systolic and end-diastolic. Color-flow Doppler imaging revealed eccentric turbulent flow starting below the left ventricular outflow tract and extending into the left atrium during systole. Spectral Doppler recordings revealed a high velocity flow through the mitral, tricuspid, aorta, and pulmonic regurgitation. Restrictive transmitral flow revealed high E-wave velocity, short E-wave deceleration time, and reduced A-wave velocity. There was also low ejection velocity thorough left ventricular out tract flow. Based on echocardiographic examination, dilated cardiomyopathy was the tentative diagnosis. The dog was medicated with inotropes, angiotensin converting enzyme inhibitor, and diuretics. At the 10-day following-up, the dog died suddenly. This report describes echocardiographic diagnosis and prognosis of dilated cardiomyopathy rarely reported in small breed dogs.

• Journal of Microbiology and Biotechnology
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• v.31 no.11
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• pp.1583-1590
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• 2021

Studies have demonstrated that PE_PGRS45 is constitutively expressed under various environmental conditions (such as nutrient depletion, hypoxia, and low pH) of the in vitro growth conditions examined, indicating that PE_PGRS45 protein is critical to the basic functions of Mycobacterium tuberculosis. However, there are few reports about the biochemical function and pathogenic mechanism of PE_PGRS45 protein. The fact that this M. tuberculosis gene is not easily expressed in E. coli may be mainly due to the high content of G+C and the use of unique codons. Fusion tags are indispensable tools used to improve the soluble expression of recombinant proteins and accelerate the characterization of protein structure and function. In the present study, His6, Trx, and His6-MBP were used as fusion tags, but only MBP-PE_PGRS45 was expressed solubly. The purification using His6-MBP tag-specific binding to the Ni column was easy to separate after the tag cleavage. We used the purified PE_PGRS45 to immunize New Zealand rabbits and obtained anti-PE_PGRS45 serum. We found that the titer of polyclonal antibodies against PE_PGR45 was higher than 1:256000. The result shows that purified PE_PGRS45 can induce New Zealand rabbits to produce high-titer antibodies. In conclusion, the recombinant protein PE_PGRS45 was successfully expressed in E. coli and specific antiserum was prepared, which will be followed by further evaluation of these specific antigens to develop highly sensitive and specific diagnostic tests for tuberculosis.

• Journal of the Korea Convergence Society
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• v.13 no.3
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• pp.353-362
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• 2022

This study was a randomized controlled trial to examine the effects of breast massage applied to women of childbearing age with cyclic mastagia on breast pain, breast hardness, nipple extensibility, breast size, and breast blood circulation. The study participants were 54 women with cyclic mastalgia who were recruited from Medical Center C, Women's Hospital M, department stores located in City D, and Internet cafes. The experimental treatment was to apply breast massage for a total of 30 minutes, 15 minutes each to the left and right breasts during the period of cyclic mastalgia. After experimental treatment, breast pain (F=49.16, p<.001), breast hardness (Rt t=8.93, p<.001; Lt t=-10.34, p<.001), nipple extensibility (Rt t=3.58, p<.001 ; Lt t=4.66, p<.001), breast size (Rt F=60.59, p<.001; Lt F=51.05, p<.001) and breast blood circulation (Rt t=-1.30, p=.201; Lt t=-2.82, p=.007) were significantly different between the two groups. In conclusion, breast massage performed in this study was effective in relieving breast pain, relieving breast hardness, and improving breast blood circulation in participants with cyclic mastalgia.

• Journal of Korean Neurosurgical Society
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• v.65 no.6
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• pp.779-789
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• 2022

Objective : To analyze the effects of the number and shape of fenestrations on the mechanical strength of pedicle screws and the effects of bone cement augmentation (BCA) on the pull-out strength (POS) of screws used in conventional BCA. Methods : For the control group, a conventional screw was defined as C1, a screw with cannulated end-holes was defined as C2, a C2 screw with six pinholes was defined as C3, and the control group type was set. Among the experimental screws, T1 was designed using symmetrically placed thru-hole type fenestrations with an elliptical shape, while T2 was designed with half-moon (HM)-shaped asymmetrical fenestrations. T3 and T4 were designed with single HM-shaped fenestrations covering three pitches and five pitches, respectively. T5 and T6 were designed with 0.6-mm and 1-mm wider fenestrations than T3. BCA was performed by injecting 3 mL of commercial bone cement in the screw, and mechanical strength and POS tests were performed according to ASTM F1717 and ASTM F543 standards. Synthetic bone (model #1522-505) made of polyurethane foam was used as a model of osteoporotic bone, and radiographic examinations were performed using computed tomography and fluoroscopy. Results : In the fatigue test, at 75% ultimate load, fractures occurred 7781 and 9189 times; at 50%, they occurred 36122 and 82067 times; and at 25%, no fractures occurred. The mean ultimate load for each screw type was 219.1±52.39 N for T1, 234.74±15.9 N for T2, 220.70±59.23 N for T3, 216.45±32.4 N for T4, 181.55±54.78 N for T5, and 216.47±29.25 N for T6. In comparison with C1, T1, T2, T3, T4, and T6 showed significantly different ultimate load values (p<0.05). However, when the values for C2 and the fenestrated screws were evaluated with an unpaired t test, the ultimate load value of C2 significantly differed only from that of T2 (p=0.025). The ultimate load value of C3 differed significantly from those of T1 and T2 (C3 vs. T1 : p=0.048; C3 vs. T2 : p<0.001). Linear correlation analysis revealed a significant correlation between the fenestration area and the volume of bone cement (Pearson's correlation coefficient r=0.288, p=0.036). The bone cement volume and ultimate load significantly correlated with each other in linear correlation analysis (r=0.403, p=0.003). Conclusion : Fenestration yielded a superior ultimate load in comparison with standard BCA using a conventional screw. In T2 screws with asymmetrical two-way fenestrations showed the maximal increase in ultimate load. The fenestrated screws can be expected to show a stable position for the formation of the cement mass.

• Biomolecules & Therapeutics
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• v.30 no.6
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• pp.553-561
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• 2022

Chronic myeloid leukemia (CML) is a slowly progressing hematopoietic cell disorder. Sphingosine kinase 1 (SPHK1) plays established roles in tumor initiation, progression, and chemotherapy resistance in a wide range of cancers, including leukemia. However, small-molecule inhibitors targeting SPHK1 in CML still need to be developed. This study revealed the role of SPHK1 in CML and investigated the potential anti-leukemic activity of hirsuteine (HST), an indole alkaloid obtained from the oriental plant Uncaria rhynchophylla, in CML cells. These results suggest that SPHK1 is highly expressed in CML cells and that overexpression of SPHK1 represents poor clinical outcomes in CML patients. HST exposure led to G2/M phase arrest, cellular apoptosis, and downregulation of Cyclin B1 and CDC2 and cleavage of Caspase 3 and PARP in CML cells. HST shifted sphingolipid rheostat from sphingosine 1-phosphate (S1P) towards the ceramide coupled with a marked inhibition of SPHK1. Mechanistically, HST significantly blocked SPHK1/S1P/S1PR1 and BCR-ABL/PI3K/Akt pathways. In addition, HST can be docked with residues of SPHK1 and shifts the SPHK1 melting curve, indicating the potential protein-ligand interactions between SPHK1 and HST in both CML cells. SPHK1 overexpression impaired apoptosis and proliferation of CML cells induced by HST alone. These results suggest that HST, which may serve as a novel and specific SPHK1 inhibitor, exerts anti-leukemic activity by inhibiting the SPHK1/S1P/S1PR1 and BCR-ABL/PI3K/Akt pathways in CML cells, thus conferring HST as a promising anti-leukemic drug for CML therapy in the future.

• Journal of Convergence for Information Technology
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• v.11 no.5
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• pp.199-205
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• 2021

This study was done to evaluate the dermatoxicity of zinc sulfate (ZnSO4) and the protective effect of Rubus parvifolius L. (RP) extract on cytotoxicity of ZnSO4, mordant in cultured SK-MEL-3 cells. For this study, it was done an antioxidative effect as DPPH-radical scavenging ability as well as the diminutive ability of total melanin with cell viability. ZnSO4 significantly decreased cell viability in dose-dependently, and it was mid-toxic. The ascorbic acid significantly increased cell viability damaged by ZnSO4-induced cytotoxicity. In the protective effect of RP extract on ZnSO4-induced cytotoxicity, RP extract significantly increased cell viability compared with ZnSO4-treated group, and also it showed both the DPPH-radical scavenging ability and the decrease of total amount of melanin. From these findings, the cytotoxicity of ZnSO4 is correlated with oxidative stress, and also RP extract effectively protected ZnSO4-induced cytotoxicity via antioxidative effect such as DPPH-radical scavenging ability with the whitening effect by the decrement of total amount of melanin. Conclusively, the natural ingredients like RP extract may be a useful agent for the improvement of antioxidative and whitening effects

• Journal of Chest Surgery
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• v.55 no.1
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• pp.10-19
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• 2022

Background: According to the eighth TNM (tumor-node-metastasis) staging system, the presence of separate tumor nodules in the same lobe is designated as a T3 descriptor. However, it remains unclear whether adjuvant chemotherapy confers survival advantages in this setting. Methods: We retrospectively identified 142 pathologic T3N0M0 patients with additional pulmonary nodules in the same lobe from a single-institutional database from 2004 to 2019. The main outcomes were overall survival and recurrence-free survival. Multivariable Cox regression was used to identify the benefit of adjuvant chemotherapy while adjusting for other variables. Results: Sixty-one patients received adjuvant chemotherapy (adjuvant group) and 81 patients did not receive adjuvant therapy after surgery (surgery-only group). There were no demonstrable differences between the 2 groups regarding hospital mortality and postoperative complications, indicating that treatment selection had not significantly occurred. However, the use of adjuvant chemotherapy was associated with improved 5-year overall survival (70% vs. 59%, p=0.006) and disease-free survival (60% vs. 46%, p=0.040). A multivariable Cox model demonstrated that adjuvant chemotherapy was associated with a survival advantage (adjusted hazard ratio, 0.54; p<0.001). In exploratory analyses of subgroups, the effect of adjuvant chemotherapy seemed to be insufficient in those with small main tumors (<4 cm). Conclusion: Adjuvant chemotherapy was associated with better survival in T3 cancers with an additional tumor nodule in the same lobe. However, the role of adjuvant chemotherapy in patient subgroups with small tumors or those without risk factors should be determined via large studies.

• Clinical and Experimental Reproductive Medicine
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• v.49 no.1
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• pp.2-8
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• 2022

Humanity is in the midst of the coronavirus disease 2019 (COVID-19) pandemic, and vaccines-including mRNA vaccines-have been developed at an unprecedented speed. It is necessary to develop guidelines for vaccination for people undergoing treatment with assisted reproductive technology (ART) and for pregnancy-related situations based on the extant laboratory and clinical data. COVID-19 vaccines do not appear to adversely affect gametes, embryos, or implantation; therefore, active vaccination is recommended for women or men who are preparing for ART. The use of intravenous immunoglobulin G (IVIG) for the treatment of immune-related infertility is unlikely to impact the effectiveness of the vaccines, so COVID-19 vaccines can be administered around ART cycles in which IVIG is scheduled. Pregnant women have been proven to be at risk of severe maternal and neonatal complications from COVID-19. It does not appear that COVID-19 vaccines harm pregnant women or fetuses; instead, they have been observed to deliver antibodies against severe acute respiratory syndrome coronavirus 2 (SARSCoV-2) to the fetus. Accordingly, it is recommended that pregnant women receive COVID-19 vaccination. There is no rationale for adverse effects, or clinical cases of adverse reactions, in mothers or neonates after COVID-19 vaccination in lactating women. Instead, antibodies to SARS-CoV-2 can be delivered through breast milk. Therefore, breastfeeding mothers should consider vaccination. In summary, active administration of COVID-19 vaccines will help ensure the safe implementation of ART, pregnancy, and breastfeeding.