1.A Comparative Study of Three National Surveys on Biliary Atresia by the Korean Association of Pediatric Surgeons
Yeon Jun JEONG ; Dayoung KO ; Hyunhee KWON ; Ki Hoon KIM ; Dae Yeon KIM ; Soo-Hong KIM ; Wontae KIM ; Hae-Young KIM ; Hyun Young KIM ; Seong Chul KIM ; Younghyun NA ; Jung-Man NAMGOONG ; So Hyun NAM ; Junbeom PARK ; Jinyoung PARK ; Tae-Jun PARK ; Jeong-Meen SEO ; Ji-Young SUL ; Joonhyuk SON ; Hyun Beak SHIN ; Joohyun SIM ; Soo Min AHN ; Hee Beom YANG ; Jung-Tak OH ; Chaeyoun OH ; Joong Kee YOUN ; Sanghoon LEE ; Ju Yeon LEE ; Kyong IHN ; Hye Kyung CHANG ; Eunyoung JUNG ; Jae Hee CHUNG ; Yu Jeong CHO ; Yun Mee CHOE ; Soo Jin Na CHOI ; Seok Joo HAN ; In Geol HO ; Ji-Won HAN
Advances in Pediatric Surgery 2025;31(2):47-58
Purpose:
Biliary atresia (BA) is a rare but progressive cholangiopathy and the leading cause of pediatric liver transplantation worldwide. The Korean Association of Pediatric Surgeons (KAPS) has conducted three national surveys (2001, 2011, and 2023) to assess long-term trends in the diagnosis, treatment, and outcomes of BA. This study provides a comparative analysis of the 2nd and 3rd national surveys, with reference to selected findings from the 1st survey.
Methods:
This study included 453 patients from the 3rd national survey (2011–2021) and 435 patients from the 2nd survey (2001–2010), all of whom underwent Kasai portoenterostomy. Data were collected via electronic case report forms from pediatric surgical centers nationwide. Comparisons were made regarding demographics, clinical features, diagnostic patterns, operative details, follow-up outcomes, and survival. Kaplan–Meier analysis was used to evaluate long-term survival.
Results:
The mean number of BA patients per year remained stable between surveys (43.5 in the 2nd, 41.18 in the 3rd), though centralization of care increased, with 61.5% of cases managed by two major institutions in the 3rd survey. The median age at surgery decreased, and the use of preoperative imaging (especially magnetic resonance cholangiopancreatography) increased. The 10-year native liver survival rate declined from 59.8% to 53.7%, while overall 10-year survival improved slightly (92.9% to 93.2%). Postoperative complications, such as cholangitis and liver failure, persisted but were better categorized. The 3rd survey also reported improved mortality (4.9%) and reduced follow-up loss (11.5%) compared to the 2nd survey.
Conclusion
While overall survival after Kasai operation has remained high and even improved, native liver survival has slightly declined. The findings reflect earlier diagnosis, more consistent diagnostic imaging, and increasing centralization of care. These trends underscore the importance of long-term nationwide data collection in guiding future strategies for BA management in Korea.
2.Ataxin-3 Overexpression via Adeno-associated Viral Vector Injection in the Primate Cerebellum: A Novel Model of Spinocerebellar Ataxia Type 3
Keonwoo KIM ; Aryun KIM ; Jinyoung WON ; Junghyung PARK ; Kyung Seob LIM ; Chang-Yeop JEON ; Jisun MIN ; Jee-Hyun CHO ; Youngkyu SONG ; Bon-Sang KOO ; Gyu-Seo BAE ; Eunsu JEON ; Kang-Jin JEONG ; Sung-Hyun PARK ; Hwal-Yong LEE ; Won Seok CHOI ; Dong-Seok LEE ; Youngjeon LEE
Experimental Neurobiology 2025;34(6):248-262
Spinocerebellar ataxia type 3 (SCA3) is an autosomal-dominant neurodegenerative disorder caused by an expanded polyglutamine repeat in the ataxin-3 gene. The resulting mutant ataxin-3 protein forms intraneuronal inclusions that lead to neurodegeneration in the cerebellum and other brain regions. This study aimed to develop a novel nonhuman primate model of SCA3 to address the limitations of existing knock-in and transgenic models using an adeno-associated virus (AAV) to deliver the mutant gene. AAV viral vectors carrying mutant ataxin-3 were stereotaxically injected into the cerebellum of monkeys. The animals were monitored over an 8-week period, during which behavioral and neuroimaging assessments were conducted. This was followed by a detailed pathological examination. The AAV vector successfully spread throughout the cerebellum, and the expression of mutant ataxin-3 was confirmed. Neuroimaging revealed a reduction in N-acetylaspartate (NAA) levels, whereas histological analysis showed significant damage to the Purkinje cell layer. Notably, the monkeys exhibited sleep disturbances, a prodromal symptom commonly observed in human patients with SCA3. AAV-mediated delivery of mutant ataxin-3 can effectively replicate the key pathological and clinical features of SCA3 in primates. This approach offers a promising new model for studying disease mechanisms and evaluating potential therapies.
3.Tuberculous Pericarditis Mimicking a Malignant Pericardial Tumor:A Case Report
Ji Young PARK ; Ji-Yeon HAN ; Jinyoung PARK ; Gi Won SHIN ; Su Young YUN ; Mi Seon KANG ; Da Som KIM
Journal of the Korean Society of Radiology 2024;85(1):197-203
Tuberculous pericarditis is an extrapulmonary manifestation of tuberculosis that is most commonly associated with pericardial thickening, effusion, and calcification. We present a case of tuberculous pericarditis mimicking a malignant pericardial tumor in a 77-year-old male. CT revealed an irregular and nodular pericardial thickening. MRI revealed high signal intensity on T1-weighted fat-suppressed images and peripheral rim enhancement after gadolinium administration. MRI can be helpful in determining the differential diagnoses in cases of tuberculous pericarditis with nonspecific imaging findings.
4.Maxillary complete denture and mandibular fixed prosthesis restoration using CBCT based facebow transfer:a case report
JinYoung CHON ; Su-Jin AHN ; Suk Won LEE
The Journal of Korean Academy of Prosthodontics 2024;62(4):262-273
With the increasing use of CAD-CAM (computer-aided design-computer-aided manufacturing) methods for prosthesis fabrication, utilization of virtual articulators in CAD software has also increased. Among the various methods of positioning the maxilla in a virtual articulator, there are techniques that utilize CBCT (cone-beam computed tomography) or facial scans without the use of a facebow.In this case, a full-mouth rehabilitation was planned for a patient who exhibited multiple crown fractures and occlusal plane disharmony through maxillary complete denture and mandibular fixed prosthetic restoration. Radiopaque markers were added to the duplicated maxillary temporary denture to take closed-mouth impression, which was then scanned and positioned on the CBCT. On CBCT, hinge axis connecting the medial poles of both mandibular condyles along with the Frankfort horizontal plane was designated and utilized to perform virtual articulator mounting. Maxillary complete denture and mandibular fixed prostheses were fabricated, resulting in satisfactory aesthetic and functional outcomes.
5.Evaluating the Validity and Reliability of the Korean Version of the Scales for Outcomes in Parkinson’s Disease–Cognition
Jinse PARK ; Eungseok OH ; Seong-Beom KOH ; In-Uk SONG ; Tae-Beom AHN ; Sang Jin KIM ; Sang-Myung CHEON ; Yoon-Joong KIM ; Jin Whan CHO ; Hyeo-Il MA ; Mee Young PARK ; Jong Sam BAIK ; Phil Hyu LEE ; Sun Ju CHUNG ; Jong-Min KIM ; Han-Joon KIM ; Young-Hee SUNG ; Do Young KWON ; Jae-Hyeok LEE ; Jee-Young LEE ; Ji Seon KIM ; Ji Young YUN ; Hee Jin KIM ; Jin Yong HONG ; Mi-Jung KIM ; Jinyoung YOUN ; Hui-Jun YANG ; Won Tae YOON ; Sooyeoun YOU ; Kyum-Yil KWON ; Su-Yun LEE ; Younsoo KIM ; Hee-Tae KIM ; Joong-Seok KIM ; Ji-Young KIM
Journal of Movement Disorders 2024;17(3):328-332
Objective:
The Scales for Outcomes in Parkinson’s Disease–Cognition (SCOPA-Cog) was developed to assess cognition in patients with Parkinson’s disease (PD). In this study, we aimed to evaluate the validity and reliability of the Korean version of the SCOPACog (K-SCOPA-Cog).
Methods:
We enrolled 129 PD patients with movement disorders from 31 clinics in South Korea. The original version of the SCOPA-Cog was translated into Korean using the translation-retranslation method. The test–retest method with an intraclass correlation coefficient (ICC) and Cronbach’s alpha coefficient were used to assess reliability. Spearman’s rank correlation analysis with the Montreal Cognitive Assessment-Korean version (MOCA-K) and the Korean Mini-Mental State Examination (K-MMSE) were used to assess concurrent validity.
Results:
The Cronbach’s alpha coefficient was 0.797, and the ICC was 0.887. Spearman’s rank correlation analysis revealed a significant correlation with the K-MMSE and MOCA-K scores (r = 0.546 and r = 0.683, respectively).
Conclusion
Our results demonstrate that the K-SCOPA-Cog has good reliability and validity.
6.Tuberculous Pericarditis Mimicking a Malignant Pericardial Tumor:A Case Report
Ji Young PARK ; Ji-Yeon HAN ; Jinyoung PARK ; Gi Won SHIN ; Su Young YUN ; Mi Seon KANG ; Da Som KIM
Journal of the Korean Society of Radiology 2024;85(1):197-203
Tuberculous pericarditis is an extrapulmonary manifestation of tuberculosis that is most commonly associated with pericardial thickening, effusion, and calcification. We present a case of tuberculous pericarditis mimicking a malignant pericardial tumor in a 77-year-old male. CT revealed an irregular and nodular pericardial thickening. MRI revealed high signal intensity on T1-weighted fat-suppressed images and peripheral rim enhancement after gadolinium administration. MRI can be helpful in determining the differential diagnoses in cases of tuberculous pericarditis with nonspecific imaging findings.
7.Maxillary complete denture and mandibular fixed prosthesis restoration using CBCT based facebow transfer:a case report
JinYoung CHON ; Su-Jin AHN ; Suk Won LEE
The Journal of Korean Academy of Prosthodontics 2024;62(4):262-273
With the increasing use of CAD-CAM (computer-aided design-computer-aided manufacturing) methods for prosthesis fabrication, utilization of virtual articulators in CAD software has also increased. Among the various methods of positioning the maxilla in a virtual articulator, there are techniques that utilize CBCT (cone-beam computed tomography) or facial scans without the use of a facebow.In this case, a full-mouth rehabilitation was planned for a patient who exhibited multiple crown fractures and occlusal plane disharmony through maxillary complete denture and mandibular fixed prosthetic restoration. Radiopaque markers were added to the duplicated maxillary temporary denture to take closed-mouth impression, which was then scanned and positioned on the CBCT. On CBCT, hinge axis connecting the medial poles of both mandibular condyles along with the Frankfort horizontal plane was designated and utilized to perform virtual articulator mounting. Maxillary complete denture and mandibular fixed prostheses were fabricated, resulting in satisfactory aesthetic and functional outcomes.
8.Maxillary complete denture and mandibular fixed prosthesis restoration using CBCT based facebow transfer:a case report
JinYoung CHON ; Su-Jin AHN ; Suk Won LEE
The Journal of Korean Academy of Prosthodontics 2024;62(4):262-273
With the increasing use of CAD-CAM (computer-aided design-computer-aided manufacturing) methods for prosthesis fabrication, utilization of virtual articulators in CAD software has also increased. Among the various methods of positioning the maxilla in a virtual articulator, there are techniques that utilize CBCT (cone-beam computed tomography) or facial scans without the use of a facebow.In this case, a full-mouth rehabilitation was planned for a patient who exhibited multiple crown fractures and occlusal plane disharmony through maxillary complete denture and mandibular fixed prosthetic restoration. Radiopaque markers were added to the duplicated maxillary temporary denture to take closed-mouth impression, which was then scanned and positioned on the CBCT. On CBCT, hinge axis connecting the medial poles of both mandibular condyles along with the Frankfort horizontal plane was designated and utilized to perform virtual articulator mounting. Maxillary complete denture and mandibular fixed prostheses were fabricated, resulting in satisfactory aesthetic and functional outcomes.
9.Tuberculous Pericarditis Mimicking a Malignant Pericardial Tumor:A Case Report
Ji Young PARK ; Ji-Yeon HAN ; Jinyoung PARK ; Gi Won SHIN ; Su Young YUN ; Mi Seon KANG ; Da Som KIM
Journal of the Korean Society of Radiology 2024;85(1):197-203
Tuberculous pericarditis is an extrapulmonary manifestation of tuberculosis that is most commonly associated with pericardial thickening, effusion, and calcification. We present a case of tuberculous pericarditis mimicking a malignant pericardial tumor in a 77-year-old male. CT revealed an irregular and nodular pericardial thickening. MRI revealed high signal intensity on T1-weighted fat-suppressed images and peripheral rim enhancement after gadolinium administration. MRI can be helpful in determining the differential diagnoses in cases of tuberculous pericarditis with nonspecific imaging findings.
10.Maxillary complete denture and mandibular fixed prosthesis restoration using CBCT based facebow transfer:a case report
JinYoung CHON ; Su-Jin AHN ; Suk Won LEE
The Journal of Korean Academy of Prosthodontics 2024;62(4):262-273
With the increasing use of CAD-CAM (computer-aided design-computer-aided manufacturing) methods for prosthesis fabrication, utilization of virtual articulators in CAD software has also increased. Among the various methods of positioning the maxilla in a virtual articulator, there are techniques that utilize CBCT (cone-beam computed tomography) or facial scans without the use of a facebow.In this case, a full-mouth rehabilitation was planned for a patient who exhibited multiple crown fractures and occlusal plane disharmony through maxillary complete denture and mandibular fixed prosthetic restoration. Radiopaque markers were added to the duplicated maxillary temporary denture to take closed-mouth impression, which was then scanned and positioned on the CBCT. On CBCT, hinge axis connecting the medial poles of both mandibular condyles along with the Frankfort horizontal plane was designated and utilized to perform virtual articulator mounting. Maxillary complete denture and mandibular fixed prostheses were fabricated, resulting in satisfactory aesthetic and functional outcomes.

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