• Korean Journal of Radiology
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• v.22 no.3
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• pp.366-375
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• 2021

Objective: To evaluate the radiological tumor response patterns and compare the response assessments based on immune-based therapeutics Response Evaluation Criteria in Solid Tumors (iRECIST) and RECIST 1.1 in metastatic clear-cell renal cell carcinoma (mccRCC) patients treated with programmed cell death-1 (PD-1) inhibitors. Materials and Methods: All mccRCC patients treated with PD-1 inhibitors at Henan Cancer Hospital, China, between January 2018 and April 2019, were retrospectively studied. A total of 30 mccRCC patients (20 males and 10 females; mean age, 55.6 years; age range, 37-79 years) were analyzed. The target lesions were quantified on consecutive CT scans during therapy using iRECIST and RECIST 1.1. The tumor growth rate was calculated before and after therapy initiation. The response patterns were analyzed, and the differences in tumor response assessments of the two criteria were compared. The intra- and inter-observer variabilities of iRECIST and RECIST 1.1 were also analyzed. Results: The objective response rate throughout therapy was 50% (95% confidence interval [CI]: 32.1-67.9) based on iRECIST and 30% (95% CI: 13.6-46.4) based on RECIST 1.1. The time-to-progression (TTP) based on iRECIST was longer than that based on RECIST 1.1 (median TTP: not reached vs. 170 days, p = 0.04). iRECIST and RECIST 1.1 were discordant in 8 cases, which were evaluated as immune-unconfirmed PD based on iRECIST and PD based on RECIST 1.1. Six patients (20%, 6/30) had pseudoprogression based on iRECIST, of which four demonstrated early pseudoprogression and two had delayed pseudoprogression. Significant differences in the tumor response assessments based on the two criteria were observed (p < 0.001). No patients demonstrated hyperprogression during the study period. Conclusion: Our study confirmed that the iRECIST criteria are more capable of capturing immune-related atypical responses during immunotherapy, whereas conventional RECIST 1.1 may underestimate the benefit of PD-1 inhibitors. Pseudoprogression is not rare in mccRCC patients during PD-1 inhibitor therapy, and it may last for more than the recommended maximum of 8 weeks, indicating a limitation of the current strategy for immune response monitoring.

• Korean Journal of Radiology
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• v.22 no.1
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• pp.118-130
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• 2021

Objective: This study aimed to investigate the blood-brain barrier (BBB) disruption in mild traumatic brain injury (mTBI) patients with post-concussion syndrome (PCS) using dynamic contrast-enhanced (DCE) magnetic resonance (MR) imaging and automatic whole brain segmentation. Materials and Methods: Forty-two consecutive mTBI patients with PCS who had undergone post-traumatic MR imaging, including DCE MR imaging, between October 2016 and April 2018, and 29 controls with DCE MR imaging were included in this retrospective study. After performing three-dimensional T1-based brain segmentation with FreeSurfer software (Laboratory for Computational Neuroimaging), the mean K^trans and v_p from DCE MR imaging (derived using the Patlak model and extended Tofts and Kermode model) were analyzed in the bilateral cerebral/cerebellar cortex, bilateral cerebral/cerebellar white matter (WM), and brainstem. K^trans values of the mTBI patients and controls were calculated using both models to identify the model that better reflected the increased permeability owing to mTBI (tendency toward higher K^trans values in mTBI patients than in controls). The Mann-Whitney U test and Spearman rank correlation test were performed to compare the mean K^trans and v_p between the two groups and correlate K^trans and v_p with neuropsychological tests for mTBI patients. Results: Increased permeability owing to mTBI was observed in the Patlak model but not in the extended Tofts and Kermode model. In the Patlak model, the mean K^trans in the bilateral cerebral cortex was significantly higher in mTBI patients than in controls (p = 0.042). The mean v_p values in the bilateral cerebellar WM and brainstem were significantly lower in mTBI patients than in controls (p = 0.009 and p = 0.011, respectively). The mean K^trans of the bilateral cerebral cortex was significantly higher in patients with atypical performance in the auditory continuous performance test (commission errors) than in average or good performers (p = 0.041). Conclusion: BBB disruption, as reflected by the increased K^trans and decreased v_p values from the Patlak model, was observed throughout the bilateral cerebral cortex, bilateral cerebellar WM, and brainstem in mTBI patients with PCS.

• The Journal of Korean Academy of Sensory Integration
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• v.21 no.3
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• pp.79-102
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• 2023

Objective : Autistic Spectrum Disorders(ASD) is a developmental disorder characterized by atypical sensory adaptation, communication problem, stereotyped behavior, and feeding disorders. The reasons for ASD feeding disorders are oral sensory motor, cognitive, behavioral, and social problems. Major symptoms include picky eating, selective eating, food refusal, food neophobia, limited food variety, and food aversion. ASD feeding disorders could be accompanied by various problems such as health and nutrition intake problems, feeding development, eating-related sociability, and family and caregiver stress. Feeding problems and disorders in ASD can present from birth. However, ASD is diagnosed by the age of 3, and there might be an appropriate treatment gap. Usually, symptoms of feeding disorders tend to decrease with age. However, the symptoms often remain, so early evaluation, intervention, and periodic checking are necessary. In this study, the general information about the feeding disorder characteristics of ASD, influencing factors, and intervention were described through a literature review. Conclusion : Sensory-based therapy and behavior-based therapies are generally used for feeding disorders in ASD. Sensory-based therapy is effective for food sensitivity and behavior-based therapy for food selection. As the symptoms of feeding disorders in ASD are diverse, a comprehensive approach includes play and participation, oral motor exercise, diet, and daily life. However, appropriate evaluation, intervention protocol, and guidelines for the treatment of feeding disorders in ASD are limited. Therefore, a complex approach based on a more systematic understanding is needed. Feeding rehabilitation specialists, such as occupational therapists, should provide appropriate evaluation and intervention.

• Journal of the Korean Society of Radiology
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• v.83 no.3
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• pp.508-526
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• 2022

Parkinson's disease (PD) is a movement disorder that develops due to degenerative loss of dopaminergic cells in the substantia nigra of the midbrain. Recent advances in MRI techniques have demonstrated various imaging findings that can reflect the underlying pathophysiological processes occurring in Parkinson's disease. Many imaging studies have shown that such findings can assist in the diagnosis of Parkinson's disease and its differentiation from atypical parkinsonism. In this review, we present MRI techniques that can be used in clinical assessment, such as nigrosome imaging and neuromelanin imaging, and we provide the detailed imaging features of Parkinson's disease reflecting nigrostriatal degeneration.

• Journal of the Korean Society of Radiology
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• v.81 no.4
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• pp.990-995
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• 2020

Chronic granulomatous disease (CGD) is an uncommon primary immune deficiency caused by phagocytes defective in oxygen metabolite production. It results in recurrent bacterial or fungal infections. Herein, we present a case of CGD with a large pulmonary granuloma in a neonate and review the imaging findings. The patient was a 24-day-old neonate admitted to the hospital with fever. A round opacified lesion was identified on the chest radiograph. Subsequent CT and MRI revealed a round mass with heterogeneous enhancement in the right lower lobe. There were foci of diffusion restriction in the mass. Surgical biopsy of the mass revealed chronic granuloma. Finally, the neonate was diagnosed with CGD caused by mutation of the gp91^phox gene. Herein, we present the clinical and imaging findings of this unusual case of CGD.

• Korean Journal of Radiology
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• v.24 no.7
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• pp.690-697
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• 2023

Objective: ¹⁸F-FP-CIT positron emission tomography (PET) is known for its high sensitivity and specificity for evaluating striatal dopamine transporter (DAT) binding. Recently, for the early diagnose of Parkinson's disease, many researchers focused on the diagnosis of synucleinopathy in organs involved in non-motor symptoms of Parkinson's disease. We investigated the feasibility of salivary gland uptake on ¹⁸F-FP-CIT PET as a new biomarker in patients with parkinsonism. Materials and Methods: A total of 219 participants with confirmed or presumed parkinsonism, including 54 clinically diagnosed idiopathic Parkinson's disease (IPD), 59 suspected and yet undiagnosed, and 106 with secondary parkinsonism, were enrolled. The standardized uptake value ratio (SUVR) of the salivary glands was measured on both early and delayed ¹⁸F-FP-CIT PET scans using the cerebellum as the reference region. Additionally, the delayed-to-early ratio (DE_ratio) of salivary gland was obtained. The results were compared between patients with different PET patterns. Results: The SUVR in early ¹⁸F-FP-CIT PET scan was significantly higher in patients with IPD pattern compared that in the non-dopaminergic degradation group (0.5 ± 0.19 vs. 0.6 ± 0.21, P < 0.001). Compared with the non-dopaminergic degradation group, the DE_ratio was significantly lower in patients with IPD (5.05 ± 1.7 vs. 4.0 ± 1.31, P < 0.001) or atypical parkinsonism patterns (5.05 ± 1.7 vs. 3.76 ± 0.96, P < 0.05). The DE_ratio was moderately and positively correlated with striatal DAT availability in both the whole striatum (r = 0.37, P < 0.001) and posterior putamen (r = 0.36, P < 0.001). Conclusion: Parkinsonism patients with an IPD pattern exhibited a significant increase in uptake on early ¹⁸F-FP-CIT PET and a decrease in the DE_ratio in the salivary gland. Our findings suggest that salivary gland uptake of dual-phase ¹⁸F-FP-CIT PET can provide diagnostic information on DAT availability in patients with Parkinson's disease.

• Journal of Radiation Industry
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• v.17 no.4
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• pp.471-479
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• 2023

In In-situ radioactivity measurement techniques, efficiency calibration models use predefined models to simulate a sample's geometry and radioactivity distribution. However, simplified efficiency calibration models lead to uncertainties in the efficiency curves, which in turn affect the radioactivity concentration results. This study aims to develop an efficiency calibration optimization methodology to improve the accuracy of in-situ gamma radiation measurements for byproducts from industrial facilities. To accomplish the objective, a drive mechanism for rotational measurement of an byproduct simulator and a sample was constructed. Using ISOCS, an efficiency calibration model of the designed object was generated. Then, the sensitivity analysis of the efficiency calibration model was performed, and the efficiency curve of the efficiency calibration model was optimized using the sensitivity analysis results. Finally, the radiation concentration of the simulated subject was estimated, compared, and evaluated with the designed certification value. For the sensitivity assessment of the influencing factors of the efficiency calibration model, the ISOCS Uncertainty Estimator was used for the horizontal and vertical size and density of the measured object. The standard deviation of the measurement efficiency as a function of the longitudinal size and density of the efficiency calibration model decreased with increasing energy region. When using the optimized efficiency calibration model, the measurement efficiency using IUE was improved compared to the measurement efficiency using ISOCS at the energy of ²²⁸Ac (911 keV) for the nuclide under analysis. Using the ISOCS efficiency calibration method, the difference between the measured radiation concentration and the design value for each simulated subject measurement direction was 4.1% (1% to 10%) on average. The difference between the estimated radioactivity concentration and the design value was 3.6% (1~8%) on average when using the ISOCS IUE efficiency calibration method, which was closer to the design value than the efficiency calibration method using ISOCS. In other words, the estimated radioactivity concentration using the optimized efficiency curve was similar to the designed radioactivity concentration. The results of this study can be utilized as the main basis for the development of regulatory technologies for the treatment and disposal of waste generated during the operation, maintenance, and facility replacement of domestic byproduct generation facilities.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.24 no.1
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• pp.17-25
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• 2024

Fabry disease (FD) is an X-linked lysosomal storage disorder. It is caused by mutations in the α-galactosidase A gene, which results in deficient or absent activity of α-galactosidase A (α-Gal A). This leads to a progressive accumulation of globotriaosylceramide (Gb3) in various tissues. Manifestations of Fabry disease include serious and progressive impairment of renal and cardiac function. In addition, patients experience pain, gastrointestinal disturbance, transient ischaemic attacks, and strokes. Additional effects on the skin, eyes, ears, lungs, and bones are often seen. Reduced life expectancy and deadly consequences are being caused by cardiac involvement. Chaperone therapy or enzyme replacement therapy (ERT) are two disease-specific treatments for FD. Thus, early detection of FD is critical for decreasing morbidity and mortality. Globotriaosysphingosine (lyso-Gb3) for identifying atypical FD variants and highly sensitive troponin T (hsTNT) for detecting cardiac involvement are both significant diagnostic indicators. This review aimed to offer a basic resource for the early diagnosis and update on the diagnosis of having FD. We will also provide a general diagnostic algorithm and information on ERT and its accompanying treatments.

• Clinical and Experimental Pediatrics
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• v.50 no.1
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• pp.47-51
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• 2007

Purpose : The early diagnosis of Kawasaki disease (KD) is difficult sometimes, especially in atypical or incomplete cases presenting 4 or less principal clinical features without coronary arterial lesions (coronary arterial ectasia or aneurysm). The authors investigated the incidence of echocardiographic abnormalities in patients with KD to discover whether abnormal echocardiographic findings might be helpful in the early diagnosis of KD. Methods : Echocardiography was done in the acute stage of 103 patients with KD (Kawasaki group) and 40 patients with other acute febrile illnesses (control group). Abnormal echocardiographic findings were classified into 4 categories and defined as follows; 1) significant pericardial effusion, 2) significant valvular dysfunctions, 3) left ventricular systolic dysfunction, 4) coronary arterial ectasia or aneurysm. Results : In the Kawasaki group, significant pericardial effusion was present in 24 patients (23.3 percent), significant valvular dysfunctions in 30 patients (29.1 percent), left ventricular systolic dysfunction in 10 patients (9.7 percent), and coronary arterial lesions in 27 patients (26.2 percent). In the control group, significant pericardial effusion was present in only one patient (2.5 percent). The number of patients with any one of echocardiographic abnormalities was 57 (55.3 percent) in Kawasaki group and one (2.5 percent) in control group. The sensitivity of echocardiography in the diagnosis of KD was 55.3 percent, the specificity 97.5 percent, the positive predictive value 98.3 percent, and the negative predictive value 45.9 percent. Conclusion : If abnormal echocardiographic findings, even if other than coronary arterial lesions, are confirmed in patients in whom KD is suspected, it seems desirable to inifiate specific treatment for KD.

• Journal of Chest Surgery
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• v.43 no.6
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• pp.681-686
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• 2010

Background: A chest computed-tomography has become more prevalent so that it is more common to detect small sized pulmonary nodules that have not been found in previous simple chest x-ray. If those detected nodules are undersized or located in pulmonary parenchyma, it is difficult to accomplish a biopsy since it is vulnerable to explore them either grossly or digitally. Thus, in our hospital, a thoracoscopic pulmonary wedge resection was performed after locating a lesion by means of hook wire with CT-guided. Material and Method: 31 patients (17 males and 14 female patients) from December in 2006 to June in 2010 became our subjects; their 34 pulmonary nodules were subjected to the thoracoscopic pulmonary wedge resection after locating a lesion by means of hook wire with CT-guided. Also we analyzed a possibility of hook wire dislocation, a frequency of conversion to open thoracotomy, time consumed to operation after location of a lesion, operation time, post operation complication, and histological diagnosis of the lesion. Result: 12 of 34 cases were ground glass lesion, whereas 22 cases of them were solitary pulmonary lesion. The median value of the lesion was 8mm in size (range: 3 to 23 mm), while the median value was 12.5 mm in depth (range: 1 to 34 mm). The median value of time consumed from location of the lesion to anesthetic induction was 86.5 minutes (41~473 minutes); furthermore the mean value of operation time was 103 minutes (25~345 minutes). Intrathoracic wire dislocation was found in one case, but a target lesion was successfully excised. Open thoracotomy was performed in four cases due to pleural adhesion. However, there was no case of conversion to open thoracotomy due to failure to detect a target lesion. In histological diagnosis, metastatic cancer were found in 15 cases, which were the most common, primary lung cancer were in 9 cases, non-specific inflammation were in 3 cases, tuberculosis inflammation were in 2 cases, lymph nodes were in 2 cases, active tuberculosis were in 1 case, atypical adenomatous hyperplasia was in 1 case and normal lung parenchymal finding was in 1 case, respectively. Conclusion: In our hospital, in order to accomplish a precise histological diagnosis of ground-glass lesion and pulmonary nodules in lung parenchyma, location of pulmonary nodules were exactly located with hook wire under chest computed-tomography, which was followed by lung biopsy. We concluded that this was an accurate, minimally invasive and valuable method to minimize the complications and increase of cost of medical service provided.