1.Evidence‑based Korean guidelines for the clinical management of multiple myeloma: addressing 12 key clinical questions
Sung‑Hoon JUNG ; Youngil KOH ; Min Kyoung KIM ; Jin Seok KIM ; Joon Ho MOON ; Chang‑Ki MIN ; Dok Hyun YOON ; Sung‑Soo YOON ; Je‑Jung LEE ; Chae Moon HONG ; Ka‑Won KANG ; Jihyun KWON ; Kyoung Ha KIM ; Dae Sik KIM ; Sung Yong KIM ; Sung‑Hyun KIM ; Yu Ri KIM ; Young Rok DO ; Yeung‑Chul MUN ; Sung‑Soo PARK ; Young Hoon PARK ; Ho Jin SHIN ; Hyeon‑Seok EOM ; Sang Eun YOON ; Sang Mee HWANG ; Won Sik LEE ; Myung‑won LEE ; Jun Ho YI ; Ji Yun LEE ; Ji Hyun LEE ; Ho Sup LEE ; Sung‑Nam LIM ; Jihyang LIM ; Ho‑Young YHIM ; Yoon Hwan CHANG ; Jae‑Cheol JO ; Jinhyun CHO ; Hyungwoo CHO ; Yoon Seok CHOI ; Hee jeong CHO ; Ari AHN ; Jong Han CHOI ; Hyun Jung KIM ; Kihyun KIM
Blood Research 2025;60():9-
Multiple myeloma (MM), a hematological malignancy, is characterized by malignant plasma cell proliferation in the bone marrow. Recent treatment advances have significantly improved patient outcomes associated with MM.In this study, we aimed to develop comprehensive, evidence-based guidelines for the diagnosis, prognosis, and treat‑ ment of MM. We identified 12 key clinical questions essential for MM management, guiding the extensive literature review and meta-analysis of the study. Our guidelines provide evidence-based recommendations by integrating patient preferences with survey data. These recommendations include current and emerging diagnostic tools, thera‑ peutic agents, and treatment strategies. By prioritizing a patient-centered approach and rigorous data analysis, these guidelines were developed to enhance MM management, both in Korea and globally.
2.Aplastic Anemia, Mental Retardation, and Dwarfism Syndrome Associated with Aldh2 and Adh5 Mutations
Bomi LIM ; Anna CHO ; Jaehyun KIM ; Sang Mee HWANG ; Soo Yeon KIM ; Jong-Hee CHAE ; Hyoung Soo CHOI
Clinical Pediatric Hematology-Oncology 2024;31(2):52-55
Aplastic anemia, mental retardation, and dwarfism (AMeD) syndrome, also known as aldehyde degradation deficiency (ADD) syndrome, is an autosomal recessive disorder caused by mutations in the ALDH2 and ADH5 genes, leading to decreased activity of the aldehyde dehydrogenase 2 (ALDH2) and alcohol dehydrogenase 5 (ADH5) enzymes, subsequently triggering enhanced cellular levels of formaldehyde and diverse multisystem manifestations. Herein, we present the case of a 7-year-old girl with AMeD syndrome, characterized by pancytopenia, developmental delay, microcephaly, epilepsy, and myelodysplastic syndrome. Whole-exome sequencing revealed compound heterozygous variants (c.832G>C and c.678delA) in the ADH5 gene and a heterozygous pathogenic variant (c.1510G>A) in the ALDH2 gene. This case underscores the complexity of AMeD syndrome, emphasizing the importance of genetic testing to ensure diagnosis and aid in the development of potential targeted therapeutic approaches.
3.Aplastic Anemia, Mental Retardation, and Dwarfism Syndrome Associated with Aldh2 and Adh5 Mutations
Bomi LIM ; Anna CHO ; Jaehyun KIM ; Sang Mee HWANG ; Soo Yeon KIM ; Jong-Hee CHAE ; Hyoung Soo CHOI
Clinical Pediatric Hematology-Oncology 2024;31(2):52-55
Aplastic anemia, mental retardation, and dwarfism (AMeD) syndrome, also known as aldehyde degradation deficiency (ADD) syndrome, is an autosomal recessive disorder caused by mutations in the ALDH2 and ADH5 genes, leading to decreased activity of the aldehyde dehydrogenase 2 (ALDH2) and alcohol dehydrogenase 5 (ADH5) enzymes, subsequently triggering enhanced cellular levels of formaldehyde and diverse multisystem manifestations. Herein, we present the case of a 7-year-old girl with AMeD syndrome, characterized by pancytopenia, developmental delay, microcephaly, epilepsy, and myelodysplastic syndrome. Whole-exome sequencing revealed compound heterozygous variants (c.832G>C and c.678delA) in the ADH5 gene and a heterozygous pathogenic variant (c.1510G>A) in the ALDH2 gene. This case underscores the complexity of AMeD syndrome, emphasizing the importance of genetic testing to ensure diagnosis and aid in the development of potential targeted therapeutic approaches.
4.Transradial Versus Transfemoral Access for Bifurcation Percutaneous Coronary Intervention Using SecondGeneration Drug-Eluting Stent
Jung-Hee LEE ; Young Jin YOUN ; Ho Sung JEON ; Jun-Won LEE ; Sung Gyun AHN ; Junghan YOON ; Hyeon-Cheol GWON ; Young Bin SONG ; Ki Hong CHOI ; Hyo-Soo KIM ; Woo Jung CHUN ; Seung-Ho HUR ; Chang-Wook NAM ; Yun-Kyeong CHO ; Seung Hwan HAN ; Seung-Woon RHA ; In-Ho CHAE ; Jin-Ok JEONG ; Jung Ho HEO ; Do-Sun LIM ; Jong-Seon PARK ; Myeong-Ki HONG ; Joon-Hyung DOH ; Kwang Soo CHA ; Doo-Il KIM ; Sang Yeub LEE ; Kiyuk CHANG ; Byung-Hee HWANG ; So-Yeon CHOI ; Myung Ho JEONG ; Hyun-Jong LEE
Journal of Korean Medical Science 2024;39(10):e111-
Background:
The benefits of transradial access (TRA) over transfemoral access (TFA) for bifurcation percutaneous coronary intervention (PCI) are uncertain because of the limited availability of device selection. This study aimed to compare the procedural differences and the in-hospital and long-term outcomes of TRA and TFA for bifurcation PCI using secondgeneration drug-eluting stents (DESs).
Methods:
Based on data from the Coronary Bifurcation Stenting Registry III, a retrospective registry of 2,648 patients undergoing bifurcation PCI with second-generation DES from 21 centers in South Korea, patients were categorized into the TRA group (n = 1,507) or the TFA group (n = 1,141). After propensity score matching (PSM), procedural differences, in-hospital outcomes, and device-oriented composite outcomes (DOCOs; a composite of cardiac death, target vessel-related myocardial infarction, and target lesion revascularization) were compared between the two groups (772 matched patients each group).
Results:
Despite well-balanced baseline clinical and lesion characteristics after PSM, the use of the two-stent strategy (14.2% vs. 23.7%, P = 0.001) and the incidence of in-hospital adverse outcomes, primarily driven by access site complications (2.2% vs. 4.4%, P = 0.015), were significantly lower in the TRA group than in the TFA group. At the 5-year follow-up, the incidence of DOCOs was similar between the groups (6.3% vs. 7.1%, P = 0.639).
Conclusion
The findings suggested that TRA may be safer than TFA for bifurcation PCI using second-generation DESs. Despite differences in treatment strategy, TRA was associated with similar long-term clinical outcomes as those of TFA. Therefore, TRA might be the preferred access for bifurcation PCI using second-generation DES.
5.Categorization of Meibomian Gland Dysfunction Using Lipid Layer Thickness and Meibomian Gland Dropout in Dry Eye Patients: A Retrospective Study
Phil Kyu LEE ; Jae Lim CHUNG ; Da Ran KIM ; Young Chae YOON ; SoonWon YANG ; Woong-Joo WHANG ; Yong-Soo BYUN ; HyungBin HWANG ; Kyung Sun NA ; HyunSoo LEE ; So Hyang CHUNG ; Eun Chul KIM ; YangKyung CHO ; Hyun Seung KIM ; Ho Sik HWANG
Korean Journal of Ophthalmology 2024;38(1):64-70
Purpose:
In the present study, we determined the prevalence of obstructive meibomian gland dysfunction (MGD), hyposecretory MGD, grossly normal MG, and hypersecretory MGD in patients with dry eye syndrome using lipid layer thickness (LLT) and MG dropout.
Methods:
Eighty-eight patients with dry eye syndrome were included in the study. Patients were categorized into four groups according to the LLT and weighted total meiboscore. The proportion of patients in each group was calculated. The age, sex, Ocular Surface Disease Index, LLT, Schirmer, tear film breakup time, cornea stain, weighted total meiboscore, expressibility, and quality of meibum were compared between the four groups.
Results:
Fifteen eyes (17.0%) had obstructive MGD, two eyes (2.3%) had hyposecretory MGD, 40 eyes (45.5%) had grossly normal MG, and 17 eyes (19.3%) had hypersecretory MGD. The obstructive MGD group was younger than the grossly normal MG group. In obstructive MGD, the ratio of men to women was higher than that of the other groups. However, Ocular Surface Disease Index, Schirmer, tear film breakup time, and corneal stain did not show statistically significant differences between the four groups. The meibum expressibility of the hyposecretoy MGD group was worse than those of the other groups. The meibum expressibility of the hyposecretoy MGD group was poor than those of the obstructive and hypersecretory MGD group.
Conclusions
This categorization was expected to help determine the best treatment method for dry eye syndrome, according to the MG status.
6.Aplastic Anemia, Mental Retardation, and Dwarfism Syndrome Associated with Aldh2 and Adh5 Mutations
Bomi LIM ; Anna CHO ; Jaehyun KIM ; Sang Mee HWANG ; Soo Yeon KIM ; Jong-Hee CHAE ; Hyoung Soo CHOI
Clinical Pediatric Hematology-Oncology 2024;31(2):52-55
Aplastic anemia, mental retardation, and dwarfism (AMeD) syndrome, also known as aldehyde degradation deficiency (ADD) syndrome, is an autosomal recessive disorder caused by mutations in the ALDH2 and ADH5 genes, leading to decreased activity of the aldehyde dehydrogenase 2 (ALDH2) and alcohol dehydrogenase 5 (ADH5) enzymes, subsequently triggering enhanced cellular levels of formaldehyde and diverse multisystem manifestations. Herein, we present the case of a 7-year-old girl with AMeD syndrome, characterized by pancytopenia, developmental delay, microcephaly, epilepsy, and myelodysplastic syndrome. Whole-exome sequencing revealed compound heterozygous variants (c.832G>C and c.678delA) in the ADH5 gene and a heterozygous pathogenic variant (c.1510G>A) in the ALDH2 gene. This case underscores the complexity of AMeD syndrome, emphasizing the importance of genetic testing to ensure diagnosis and aid in the development of potential targeted therapeutic approaches.
7.Aplastic Anemia, Mental Retardation, and Dwarfism Syndrome Associated with Aldh2 and Adh5 Mutations
Bomi LIM ; Anna CHO ; Jaehyun KIM ; Sang Mee HWANG ; Soo Yeon KIM ; Jong-Hee CHAE ; Hyoung Soo CHOI
Clinical Pediatric Hematology-Oncology 2024;31(2):52-55
Aplastic anemia, mental retardation, and dwarfism (AMeD) syndrome, also known as aldehyde degradation deficiency (ADD) syndrome, is an autosomal recessive disorder caused by mutations in the ALDH2 and ADH5 genes, leading to decreased activity of the aldehyde dehydrogenase 2 (ALDH2) and alcohol dehydrogenase 5 (ADH5) enzymes, subsequently triggering enhanced cellular levels of formaldehyde and diverse multisystem manifestations. Herein, we present the case of a 7-year-old girl with AMeD syndrome, characterized by pancytopenia, developmental delay, microcephaly, epilepsy, and myelodysplastic syndrome. Whole-exome sequencing revealed compound heterozygous variants (c.832G>C and c.678delA) in the ADH5 gene and a heterozygous pathogenic variant (c.1510G>A) in the ALDH2 gene. This case underscores the complexity of AMeD syndrome, emphasizing the importance of genetic testing to ensure diagnosis and aid in the development of potential targeted therapeutic approaches.
8.Ultrasonographic and Computed Tomographic Features of Dermoid Cyst of the Neck in a Dog:A Case Report
Seong-Ju OH ; Gunha HWANG ; Eun-Chae YUN ; Dongbin LEE ; Sung-Lim LEE ; Hee Chun LEE ; Tae Sung HWANG
Journal of Veterinary Clinics 2024;41(4):252-257
A 13-year-old castrated male mixed-breed dog presented for a health screening with a small nodule on the left hindlimb, which was revealed to be a mast cell tumor. A CT scan was performed to evaluate the mast cell tumor, and it incidentally revealed a well-marginated, fluid-attenuating, non-contrast enhanced mass in the deep ventral region of the left lower neck. Ultrasonography confirmed a well-capsulated mass with a cranio-ventrally protruding lesion extending into sternohyoid muscles. The outer layer of the mass had a hypoechoic thick wall and the internal parenchyma contained hyperechoic foci and multiple hyperechoic parallel lines. The mass was surgically resected. Histopathological examination confirmed a dermoid cyst characterized by cornifying squamous epithelial cells, keratinaceous debris, and hair shaft fragments. The dog showed no signs of recurrence or additional abnormalities three months post-surgery. This report highlights the importance of considering dermoid cysts in the differential diagnosis of neck masses on imaging examinations using CT or ultrasonography.
9.Impact of Adjuvant Hormone Therapy on Sleep, Physical Activity, and Quality of Life in Premenopausal Breast Cancer: 12-Month Observational Study
Seung Mi YEO ; Ji Young LIM ; Seok Won KIM ; Byung Joo CHAE ; Jonghan YU ; Jai Min RYU ; Ji Hye HWANG
Journal of Breast Cancer 2023;26(2):93-104
Purpose:
This study aimed to investigate the differences in sleep disturbance changes between patients receiving two hormone therapies (“tamoxifen plus ovarian function suppression group [T+OFS group]” versus “tamoxifen group [T group]”) and the chronological changes in sleep disturbances in each group.
Methods:
Premenopausal women with unilateral breast cancer who underwent surgery and were scheduled to receive hormone therapy (HT) with tamoxifen alone or with tamoxifen plus gonadotropin-releasing hormone (GnRH) agonist for ovarian function suppression were included. The enrolled patients wore an actigraphy watch for two weeks and completed questionnaires (insomnia, sleep quality, physical activity [PA], and quality of life [QOL]) at five time points: immediately before HT and 2, 5, 8, and 11 months after HT.
Results:
Among the 39 enrolled patients (21 and 18 patients in the T+OFS group and T group, respectively), 25 (17 and 8 patients in the T+OFS group and T group, respectively) were finally analyzed. There were no differences between the two groups in time-dependent changes in insomnia, sleep quality, total sleep time, rapid eye movement sleep rate, QOL, and PA;however, the severity of hot flashes was significantly higher in the T+OFS group than in the T group. Although the interaction between group and time was not significant, insomnia and sleep quality significantly worsened at 2–5 months of HT when changes over time were analyzed within the T+OFS group. In both the groups, PA and QOL were maintained without significant changes.
Conclusion
Unlike tamoxifen alone, tamoxifen plus GnRH agonist initially worsened insomnia and sleep quality, but gradually improved with long-term follow-up. Patients who initially experience insomnia during tamoxifen plus GnRH agonist administration can be reassured based on the results of this study, and active supportive care may be used during this period.
10.Possibility of Combined Meningitis in Under 90-Day-Old Infants With Urinary Tract Infection
Jun Ho HWANG ; Su Yeong KIM ; Na Mi LEE ; Dae Yong YI ; Sin Weon YUN ; Soo Ahn CHAE ; In Seok LIM ; Ji Young PARK
Pediatric Infection & Vaccine 2022;29(2):84-95
Purpose:
Urinary tract infections (UTIs) are the most common serious bacterial infections in young infants. Lumbar puncture (LP) has been used to diagnose coexisting meningitis in infants under 90 days of age with suspected UTI in many hospitals. However, the incidence of bacterial meningitis associated with UTIs is low. We aimed to describe the prevalence of concomitant bacterial meningitis in young infants with UTIs.
Methods:
The medical records of infants with the first episode of UTI admitted to the ChungAng University Hospital from January 2010 to December 2019 were retrospectively reviewed. Infants aged < 90 days who underwent LP with initial evaluation were included. Demographic and clinical features, laboratory findings, and imaging findings were collected and analyzed.
Results:
Eighty-six infants with UTIs were enrolled in the study. The median age was 61.5 days (interquartile range, 42.3–73.8 days) and boys (90.7%) were predominant. Escherichia coli was the most common pathogen (n=80, 93.0%) and followed by Klebsiella species (n=5, 5.8%). Fifteen (18.1%) specimens produced extended spectrum β-lactamase (ESBL). Five (5.8%) infants had positive blood culture results. Seven (8.1%) infants showed pleocytosis in the cerebrospinal fluid, but none had coexisting bacterial meningitis. Twenty-four (30.8%) infants showed renal dilatation or hydronephrosis on ultrasonography. Dimercaptosuccinic acid (DMSA) scans revealed cortical defects in 17 (21.3%) infants while voiding cystourethrography revealed vesicoureteral reflux in 6 (46.2%) infants.
Conclusion
Co-existing bacterial meningitis was not observed in young infants with UTIs. LP could not be routinely performed considering the clinical condition of <90 days old UTI patients.

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