• Progress in Medical Physics
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• v.21 no.4
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• pp.354-359
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• 2010

The purpose of this study is to verify the dosimetric effect on real PTV (planning target volume) coverage and safety of OARs (organs at risk) at various image fusion protocol-based radiosurgery plan for pituitary adenomas. Real PTV coverage and its variation was acquired and maximum dose and the volume absorbing above threshold dose were also measured for verifying the safety of optic pathway and brainstem. The protocol that can reduce superior-inferior uncertainty by using both axial and coronal MR (magnetic resonance) image sets shows relatively lower values than that of case using only axial image sets. As a result, the image fusion protocol with both axial and coronal image sets can be beneficial to generate OAR-weighted radiosurgery plan.

• Journal of Korean Neurosurgical Society
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• v.29 no.10
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• pp.1345-1351
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• 2000

Objective : Around the sellar area, there are many important structures. But, the optimal radiation dosage for minimal toxicity to surrounding neural tissue has not been firmly established. The purpose of this study is to evaluate the radiosurgical outcome of juxtasellar tumors and to investigate the relationship between radiation dosage and toxicity to neural tissue. Method : Between May 1992 and June 2000, we treated 65 juxtasellar tumors by using the Leksell Gamma Knife. Among them, 52 patients who could be followed more than 1 year were included in this study. The radiosurgical dosage to the optic pathway, cavernous sinus, Meckel's cave, hypothalamus, pituitary gland and stalk, and brain stem was analyzed and correlated with clinical outcome. The mean follow-up period was 33.5 months(range 12.2- 99.0 months). Result : The clinical response rate was 69.2%. The volume response rate was 61.0% and the radiologic control rate was 92.7%. There were 4 complications(7.7%) of 2 trigeminal neuropathy, 1 abducens nerve palsy, and 1 trigeminal and transient abducens nerve palsy. The optic apparatus appeared to tolerate doses greater than 10Gy. The risk of cranial nerve complications in cavernous sinus seemed to be related to doses of more than 16Gy. In 3 of 4 patients who received more than 16Gy to cavernous sinus, the abducens or trigeminal neuropathy occurred. Also, one patient who received more than 15Gy to the Meckel's cave, trigeminal neuropathy developed. The hypothalamus, pituitary gland and stalk, and brain stem were relatively tolerable to radiation. Conclusion : Gamma Knife radiosurgery seems to be an effective method to control the growth of juxtasellar tumors. To avoid injury to surrounding important neural tissue, careful dose planning and further study for radiation toxicity to neural tissue were needed.

• Journal of Interdisciplinary Genomics
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• v.5 no.1
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• pp.5-11
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• 2023

β-ureidopropionase (β-UP) is an enzyme that catalyzes the final step in the pyrimidine degradation pathway, which converts β-ureidopropionate and β-ureidoisobutyrate into β-alanine and β-aminoisobutyrate, respectively. β-UP deficiency (UPB1D; OMIM # 613161) is an extremely rare autosomal recessive inborn error disease caused by a mutation in the UPB1 gene on chromosome 22q11. To date, approximately 40 cases of UPB1D have been reported worldwide, including one case in Korea. The clinical manifestations of patients with UPB1D are known to be diverse, with a very wide range of manifestations being previously reported; these manifestations include completely asymptomatic, urogenital and colorectal anomalies, or severe neurological involvement, including global developmental delay, microcephaly, early onset psychomotor retardation with dysmorphic features, epilepsy, optic atrophy, retinitis pigmentosa, severely delayed myelination, and cerebellar hypoplasia. Currently, diagnosis of UPB1D is challenging as neurological manifestations, MRI abnormalities, and biochemical analysis for pyrimidine metabolites in the urine, plasma, and cerebrospinal fluid also need to be confirmed by UPB1 gene mutations. Overall, treatment of patients with UPB1D is palliative as there is still no definitive curative treatment available.

• Journal of Korean Neurosurgical Society
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• v.49 no.2
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• pp.120-123
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• 2011

We present a rare case of optochiasmatic cavernous angioma (CA) that progressed despite radiation therapy. A 31-year-old female patient presented with sudden loss of left visual acuity and right homonymous hemianopsia. Magnetic resonance imaging (MRI) revealed a suprasellar mass and findings compatible with a craniopharyngioma or an optic glioma with bleeding. An open biopsy was conducted using the transcranial approach, and histological examination revealed gliosis. During the one-year follow-up period, imaging suggested intratumoral bleeding and the mass continued to grow. We recommended re-operation, but the patient refused due to fear of surgery. Consequently, the patient received fractionated radiation therapy (3,000 cGy) to the parasellar area. Despite the radiotherapy, the mass continued to grow for the following 6 years. The final MRI before definitive treatment revealed a multi lobulated, multistage hematoma with calcification in the parasellar area, extending into the third ventricle and midbrain. The patient ultimately underwent reoperation due to the growth of the tumor. The mass was completely removed with transcranial surgery, and the pathologic findings indicated a cavernous angioma (CA) without evidence of glioma. As shown in our case, patients may suffer intratumoral hemorrhage after biopsy and radiotherapy. This case places the value of biopsy and radiotherapy for a remnant lesion into question. It also shows that reaching the correct diagnosis is critical, and complete surgical removal is the treatment of choice.

• Molecules and Cells
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• v.46 no.7
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• pp.441-450
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• 2023

β-Catenin (Ctnnb1) has been shown to play critical roles in the development and maintenance of epithelial cells, including the retinal pigment epithelium (RPE). Ctnnb1 is not only a component of intercellular junctions in the epithelium, it also functions as a transcriptional regulator in the Wnt signaling pathway. To identify which of its functional modalities is critically involved in mouse RPE development and maintenance, we varied Ctnnb1 gene content and activity in mouse RPE lineage cells and tested their impacts on mouse eye development. We found that a Ctnnb1 double mutant (Ctnnb1^dm), which exhibits impaired transcriptional activity, could not replace Ctnnb1 in the RPE, whereas Ctnnb1^Y654E, which has reduced affinity for the junctions, could do so. Expression of the constitutively active Ctnnb1^∆ex3 mutant also suppressed the development of RPE, instead facilitating a ciliary cell fate. However, the post-mitotic or mature RPE was insensitive to the loss, inactivation, or constitutive activation of Ctnnb1. Collectively, our results suggest that Ctnnb1 should be maintained within an optimal range to specify RPE through transcriptional regulation of Wnt target genes in the optic neuroepithelium.

• Journal of Yeungnam Medical Science
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• v.31 no.2
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• pp.152-156
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• 2014

We report on a patient who showed visual recovery following bilateral occipital lobe infarct, as evaluated by follow up functional magnetic resonance imaging (fMRI) and diffusion tensor tractography (DTT). A 56-year-old female patient exhibited severe visual impairment since onset of the cerebral infarct in the bilateral occipital lobes. The patient complained that she could not see anything, although the central part of the visual field remained dimly at 1 week after onset. However, her visual function has shown improvement with time. As a result, at 5 weeks after onset, she notified that her visual field and visual acuity had improved. fMRI and DTT were acquired at 1 week and 4 weeks after onset, using a 1.5-T Philips Gyroscan Intera. The fiber number of left optic radiation (OR) increased from 257 (1-week) to 353 (4-week), although the fiber numbers for right OR were similar. No activation in the occipital lobe was observed on 1-week fMRI. By contrast, activation of the visual cortex, including the bilateral primary visual cortex, was observed on 4-week fMRI. We demonstrated visual recovery in this patient in terms of the changes observed on DTT and fMRI. It appears that the recovery of the left OR was attributed more to resolution of local factors, such as peri-infarct edema, than brain plasticity.

• Molecules and Cells
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• v.42 no.12
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• pp.893-905
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• 2019

Mitochondria are highly dynamic organelles that constantly undergo fission and fusion processes that closely related to their function. Disruption of mitochondrial dynamics has been demonstrated in acute kidney injury (AKI), which could eventually result in cell injury and death. Previously, we reported that augmenter of liver regeneration (ALR) alleviates renal tubular epithelial cell injury. Here, we gained further insights into whether the renoprotective roles of ALR are associated with mitochondrial dynamics. Changes in mitochondrial dynamics were examined in experimental models of renal ischemia-reperfusion (IR). In a model of hypoxia-reoxygenation (HR) injury in vitro, dynamin-related protein 1 (Drp1) and mitochondrial fission process protein 1 (MTFP1), two key proteins of mitochondrial fission, were downregulated in the Lv-ALR + HR group. ALR overexpression additionally had an impact on phosphorylation of Drp1 Ser637 during AKI. The inner membrane fusion protein, Optic Atrophy 1 (OPA1), was significantly increased whereas levels of outer membrane fusion proteins Mitofusin-1 and -2 (Mfn1, Mfn2) were not affected in the Lv-ALR + HR group, compared with the control group. Furthermore, the mTOR/4E-BP1 signaling pathway was highly activated in the Lv-ALR + HR group. ALR overexpression led to suppression of HR-induced apoptosis. Our collective findings indicate that ALR gene transfection alleviates mitochondrial injury, possibly through inhibiting fission and promoting fusion of the mitochondrial inner membrane, both of which contribute to reduction of HK-2 cell apoptosis. Additionally, fission processes are potentially mediated by promoting tubular cell survival through activating the mTOR/4E-BP1 signaling pathway.

• Journal of Korean Neurosurgical Society
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• v.30 no.9
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• pp.1115-1119
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• 2001

Objective : Management strategies for pediatric chiasmatic-hypothalamic gliomas(CHG) include surgery, irradiation, chemotherapy and a combination of these modalities. This study was performed in order to compare the efficacy of various methods of treatment and to describe its optimal management. Material and Method : We have reviewed the results of management of 6 children with a diagnosis of CHG, who were observed closely during the last 8 years. The patients were aged 7 months to 15 years. Our patients presented with diencephalic syndrome, endocrine dysfunction, and progressive visual loss. None of these had evidence of neurofibromatosis-1. Treatment consisted of surgery alone(2), surgry and irradiation(2), surgery, irradiation and chemotherapy(1), and surgery and chemotherapy(1). Results : Four children had large exophytic suprasellar tumors and two showed diffuse midline lesions. Obstructive hydrocephalus was present in all patients. Pathologic examination revealed anaplastic astrocytoma in 1 and lowgrade astrocytoma in 5. Two patients, recently treated with radiation therapy following radical subtotal resection, showed significant tumor reductions and good clinical status. Four patients had partial tumor resection. Of these patients, two developed disease stabilization during follow-up period of 7 and 8 years, respectively. Life-threatening complications were noted in remaining two patients. Conclusion : CHG may follow an unpredicatable course and show a various reponse to each treatment modality. Further studies are indicated to define the optimal method of treatment of CHG in childhood.

• Asian Pacific Journal of Cancer Prevention
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• v.16 no.12
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• pp.5019-5024
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• 2015

Background: The purpose of this study was to assess the dosimetric and clinical feasibility of volumetric modulated arc based hypofractionated stereotactic radiotherapy (RapidArc) treatment for large acoustic schwannoma (AS >10cc). Materials and Methods: Ten AS patients were immobilized using BrainLab mask. They were subject to multimodality imaging (magnetic resonance and computed tomography) to contour target and organs at risk (brainstem and cochlea). Volumetric modulated arc therapy (VMAT) based stereotactic plans were optimized in Eclipse (V11) treatment planning system (TPS) using progressive resolution optimizer-III and final dose calculations were performed using analytical anisotropic algorithm with 1.5 mm grid resolution. All AS presented in this study were treated with VMAT based HSRT to a total dose of 25Gy in 5 fractions (5fractions/week). VMAT plan contains 2-4 non-coplanar arcs. Treatment planning was performed to achieve at least 99% of PTV volume (D99) receives 100% of prescription dose (25Gy), while dose to OAR's were kept below the tolerance limits. Dose-volume histograms (DVH) were analyzed to assess plan quality. Treatments were delivered using upgraded 6 MV un-flattened photon beam (FFF) from Clinac-iX machine. Extensive pretreatment quality assurance measurements were carried out to report on quality of delivery. Point dosimetry was performed using three different detectors, which includes CC13 ion-chamber, Exradin A14 ion-chamber and Exradin W1 plastic scintillator detector (PSD) which have measuring volume of $0.13cm^3$, $0.009cm^3$ and $0.002cm^3$ respectively. Results: Average PTV volume of AS was 11.3cc (${\pm}4.8$), and located in eloquent areas. VMAT plans provided complete PTV coverage with average conformity index of 1.06 (${\pm}0.05$). OAR's dose were kept below tolerance limit recommend by American Association of Physicist in Medicine task group-101(brainstem $V_{0.5cc}$ < 23Gy, cochlea maximum < 25Gy and Optic pathway <25Gy). PSD resulted in superior dosimetric accuracy compared with other two detectors (p=0.021 for PSD.

• The Journal of Korean Society for Radiation Therapy
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• v.34
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• pp.43-50
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• 2022

Objective: The purpose of this study is to choose a treatment plan and equipment to maximize tangential irradiation to protect the normal brain tissues as much as possible during total scalp irradiation. Subjects and Methods: After zoning the total scalp of a phantom and selecting a target area for treatment, the study made a Helical TomoTherapy(HT) plan, a Helical TomoTherapy with a Complete Block(HTCB) plan, and a Volumetric Modulated Arc Therapy(VMAT) plan. All of these plans made sure that the volume of a treatment plan with 95% of a prescription dose(40 Gy) would not exceed 95% of the entire volume and that Dmax would not be more than 110% of the prescription dose. The therapy plans compared doses among organs at risk of damage including the brain. Doses in the brain tissues were assessed based on the volumetric criteria for normal tissues in Emami et al. Results: HT, HTCB, and VMAT had a dose of 21.68 Gy, 13.75 Gy, and 20.89 Gy, respectively, in brain tissues at D_33%, a dose of 7.06 Gy, 3.21 Gy, and 7.84 Gy, respectively, at D_67%, and a dose of 3.14 Gy, 1.75 Gy, and 3.84 Gy, respectively, at D_100%. They recorded a Dmean of 16.64 Gy, 11.78 Gy, and 16.64 Gy, respectively. These results show that the overall dose was low in the HTCB plan. When the volume of a low dose was calculated based on 5 Gy, they recorded 87%, 49%, and 96%, respectively, in V_5Gy. In addition, the maximum dose in the remaining organ(brain stem, hippocampus, and both lenses) except for the optic pathway was the lowest in HTCB Conclusion: The findings demonstrate that TomoTherapy with a complete block minimized a dose in organs at risk of damage including the brain and hippocampus on both sides and accordingly reduced the probability of side effects such as radiation-induced brain injuries and a resulting decrease in neurocognitive functions. In addition to total scalp irradiation, if additional studies on ring targets treated in various areas are conducted to establish the benefits of tangential irradiation, it is believed that TomoTherapy using Complete Block can be used to maximize tangential irradiation in treatment planning.