1.Advancements in the understanding and management of histiocytic neoplasms
Kyung‑Nam KOH ; Su Hyun YOON ; Sung Han KANG ; Hyery KIM ; Ho Joon IM
Blood Research 2024;59():22-
Histiocytic neoplasms are rare diseases involving macrophages, dendritic cells, and monocytes. They include Langer‑ hans cell histiocytosis (LCH), Erdheim-Chester disease (ECD), Rosai-Dorfman disease (RDD), juvenile xanthogranuloma (JXG), and histiocytic sarcoma. Histiocytic neoplasms are characterized by varied clinical courses and prognoses, necessitating a nuanced understanding of their classification, epidemiology, and clinical manifestations. Genetic studies have revealed somatic mutations, predominantly in the MAPK pathway, suggesting a clonal neoplastic nature.This review covers the current understanding of histiocytic neoplasms, molecular pathophysiology, with a particular focus on mutations in genes such as BRAF, MAP2K1, and the PI3K-AKT signaling pathways, and evolving treatment strategies, especially focusing on LCH, ECD, RDD, and JXG. The treatment landscape has evolved with advancements in targeted therapies. BRAF inhibitors, such as vemurafenib and dabrafenib, have shown efficacy, especially in highrisk LCH cases; however, challenges remain, including relapse post-treatment discontinuation, and adverse effects.MEK inhibitors have also demonstrated effectiveness, and cobimetinib has recently been approved for use in adults.Further research is required to determine the optimal treatment duration and strategies for managing therapy inter‑ ruptions. Advancements in molecular genetics and targeted therapies have revolutionized the management of histio‑ cytic neoplasms. However, ongoing research is crucial for optimizing patient outcomes.
2.Advancements in the understanding and management of histiocytic neoplasms
Kyung‑Nam KOH ; Su Hyun YOON ; Sung Han KANG ; Hyery KIM ; Ho Joon IM
Blood Research 2024;59():22-
Histiocytic neoplasms are rare diseases involving macrophages, dendritic cells, and monocytes. They include Langer‑ hans cell histiocytosis (LCH), Erdheim-Chester disease (ECD), Rosai-Dorfman disease (RDD), juvenile xanthogranuloma (JXG), and histiocytic sarcoma. Histiocytic neoplasms are characterized by varied clinical courses and prognoses, necessitating a nuanced understanding of their classification, epidemiology, and clinical manifestations. Genetic studies have revealed somatic mutations, predominantly in the MAPK pathway, suggesting a clonal neoplastic nature.This review covers the current understanding of histiocytic neoplasms, molecular pathophysiology, with a particular focus on mutations in genes such as BRAF, MAP2K1, and the PI3K-AKT signaling pathways, and evolving treatment strategies, especially focusing on LCH, ECD, RDD, and JXG. The treatment landscape has evolved with advancements in targeted therapies. BRAF inhibitors, such as vemurafenib and dabrafenib, have shown efficacy, especially in highrisk LCH cases; however, challenges remain, including relapse post-treatment discontinuation, and adverse effects.MEK inhibitors have also demonstrated effectiveness, and cobimetinib has recently been approved for use in adults.Further research is required to determine the optimal treatment duration and strategies for managing therapy inter‑ ruptions. Advancements in molecular genetics and targeted therapies have revolutionized the management of histio‑ cytic neoplasms. However, ongoing research is crucial for optimizing patient outcomes.
3.Advancements in the understanding and management of histiocytic neoplasms
Kyung‑Nam KOH ; Su Hyun YOON ; Sung Han KANG ; Hyery KIM ; Ho Joon IM
Blood Research 2024;59():22-
Histiocytic neoplasms are rare diseases involving macrophages, dendritic cells, and monocytes. They include Langer‑ hans cell histiocytosis (LCH), Erdheim-Chester disease (ECD), Rosai-Dorfman disease (RDD), juvenile xanthogranuloma (JXG), and histiocytic sarcoma. Histiocytic neoplasms are characterized by varied clinical courses and prognoses, necessitating a nuanced understanding of their classification, epidemiology, and clinical manifestations. Genetic studies have revealed somatic mutations, predominantly in the MAPK pathway, suggesting a clonal neoplastic nature.This review covers the current understanding of histiocytic neoplasms, molecular pathophysiology, with a particular focus on mutations in genes such as BRAF, MAP2K1, and the PI3K-AKT signaling pathways, and evolving treatment strategies, especially focusing on LCH, ECD, RDD, and JXG. The treatment landscape has evolved with advancements in targeted therapies. BRAF inhibitors, such as vemurafenib and dabrafenib, have shown efficacy, especially in highrisk LCH cases; however, challenges remain, including relapse post-treatment discontinuation, and adverse effects.MEK inhibitors have also demonstrated effectiveness, and cobimetinib has recently been approved for use in adults.Further research is required to determine the optimal treatment duration and strategies for managing therapy inter‑ ruptions. Advancements in molecular genetics and targeted therapies have revolutionized the management of histio‑ cytic neoplasms. However, ongoing research is crucial for optimizing patient outcomes.
4.Safety and efficacy of nilotinib in adult patients with chronic myeloid leukemia: a post-marketing surveillance study in Korea
Seo-Yeon AHN ; Sang Kyun SON ; Gyu Hyung LEE ; Inho KIM ; June-Won CHEONG ; Won Sik LEE ; Byung Soo KIM ; Deog-Yeon JO ; Chul Won JUNG ; Chu Myoung SEONG ; Jae Hoon LEE ; Young Jin YUH ; Min Kyoung KIM ; Hun-Mo RYOO ; Moo-Rim PARK ; Su-Hee CHO ; Hoon-Gu KIM ; Dae Young ZANG ; Jinny PARK ; Hawk KIM ; Seryeon LEE ; Sung-Hyun KIM ; Myung Hee CHANG ; Ho Sup LEE ; Chul Won CHOI ; Jihyun KWON ; Sung-Nam LIM ; Suk-Joong OH ; Inkyung JOO ; Dong-Wook KIM
Blood Research 2022;57(2):144-151
Background:
Nilotinib is a tyrosine kinase inhibitor approved by the Ministry of Food and Drug Safety for frontline and 2nd line treatment of Philadelphia chromosome-positive chronic myeloid leukemia (Ph+ CML). This study aimed to confirm the safety and efficacy of nilotinib in routine clinical practice within South Korea.
Methods:
An open-label, multicenter, single-arm, 12-week observational post-marketing surveillance (PMS) study was conducted on 669 Korean adult patients with Ph + CML from December 24, 2010, to December 23, 2016. The patients received nilotinib treatment in routine clinical practice settings. Safety was evaluated by all types of adverse events (AEs) during the study period, and efficacy was evaluated by the complete hematological response (CHR) and cytogenetic response.
Results:
During the study period, AEs occurred in 61.3% (410 patients, 973 events), adverse drug reactions (ADRs) in 40.5% (271/669 patients, 559 events), serious AEs in 4.5% (30 patients, 37 events), and serious ADRs in 0.7% (5 patients, 8 events). Furthermore, unexpected AEs occurred at a rate of 6.9% (46 patients, 55 events) and unexpected ADRs at 1.2% (8 patients, 8 events). As for the efficacy results, CHR was achieved in 89.5% (442/494 patients), and minor cytogenetic response or major cytogenetic response was achieved in 85.8% (139/162 patients).
Conclusion
This PMS study shows consistent results in terms of safety and efficacy compared with previous studies. Nilotinib was well tolerated and efficacious in adult Korean patients with Ph + CML in routine clinical practice settings.
5.Gender-specific Effect of Micronutrient on Non-erosive Reflux Disease and Erosive Esophagitis
Su Youn NAM ; Bum Joon PARK ; Yeong Ah CHO ; Kum Hei RYU
Journal of Neurogastroenterology and Motility 2019;25(1):82-90
BACKGROUND/AIMS: The effect of dietary micronutrients on non-erosive reflux disease (NERD) and reflux esophagitis is unclear. We aim to evaluate the gender-specific effect of micronutrient on erosive esophagitis and NERD. METHODS: A total of 11 690 participants underwent endoscopy and completed 3-day recordings for dietary intake and questionnaires for reflux symptoms from 2004 to 2008. To evaluate the effect of dietary micronutrients on NERD or erosive esophagitis, adjusted regression analysis with odds ratio (OR) and 95% confidence interval (CI) was used. In addition, we performed gender-specific analysis. RESULTS: Prevalence of NERD and erosive esophagitis was 6.8% and 11.2% in men and 9.1% and 2.4% in women. In adjusted analysis, high intake of vitamin A (OR, 0.78; 95% CI, 0.64–0.96), retinol (OR, 0.73; 95% CI, 0.59–0.90), vitamin B2 (OR, 0.68; 95% CI, 0.54–0.87), vitamin B6 (OR, 0.75; 95% CI, 0.58–0.96), folic acid (OR, 0.77; 95% CI, 0.62–0.96), calcium (OR, 0.66; 95% CI, 0.53–0.82), and iron (OR, 0.68; 95% CI, 0.53–0.87) had an inverse association with NERD. However, erosive esophagitis has no relationship with micronutrients except vitamin C (OR, 0.78; 95% CI, 0.62–0.98). High dietary intake of calcium reduced the risk of NERD in men and high dietary intake of many micronutrients reduced NERD in women. CONCLUSIONS: While many dietary micronutrients reduced NERD, they had no effect on erosive esophagitis. The effect of micronutrient on NERD was more prominent in women than men.
Ascorbic Acid
;
Calcium
;
Endoscopy
;
Esophagitis
;
Esophagitis, Peptic
;
Female
;
Folic Acid
;
Gender Identity
;
Humans
;
Iron
;
Male
;
Micronutrients
;
Odds Ratio
;
Prevalence
;
Riboflavin
;
Vitamin A
;
Vitamin B 6
6.Multiple epidural fibrin glue patches in a patient with spontaneous intracranial hypotension:A case report
Yun Gu LEE ; Mijung SA ; In su OH ; Jeong Ae LIM ; Nam Sik WOO ; Jae hun KIM
Anesthesia and Pain Medicine 2019;14(3):335-340
BACKGROUND: Spontaneous intracranial hypotension (SIH) is a condition caused by spontaneous leakage of cerebrospinal fluid, with postural headache as the primary symptom. Orthostatic headache caused by SIH is often not resolved by conservative management. CASE: We performed 15 epidural blood patch treatments in a 43-year-old female patient; however, they were only transiently effective. To improve the patient's SIH and orthostatic headache, epidural fibrin glue patch treatment was attempted. Fibrin glue is a substance that can act as a bio-friendly adhesive by facilitating the coagulation cascade. In our case, 3 epidural fibrin glue patch treatments were performed and the symptoms completely resolved. CONCLUSIONS: The epidural fibrin glue patch may be beneficial for the treatment of refractory postural headaches caused by SIH.
Adhesives
;
Adult
;
Blood Patch, Epidural
;
Cerebrospinal Fluid
;
Female
;
Fibrin Tissue Adhesive
;
Fibrin
;
Headache
;
Humans
;
Intracranial Hypotension
7.Guidelines for the Surgical Management of Oral Cancer: Korean Society of Thyroid-Head and Neck Surgery
Young hoon JOO ; Jae keun CHO ; Bon seok KOO ; Minsu KWON ; Seong keun KWON ; Soon young KWON ; Min su KIM ; Jeong kyu KIM ; Heejin KIM ; Innchul NAM ; Jong lyel ROH ; Young min PARK ; Il seok PARK ; Jung je PARK ; Sung chan SHIN ; Soon hyun AHN ; Seongjun WON ; Chang hwan RYU ; Tae mi YOON ; Giljoon LEE ; Doh young LEE ; Myung chul LEE ; Joon kyoo LEE ; Jin choon LEE ; Jae yol LIM ; Jae won CHANG ; Jeon yeob JANG ; Man ki CHUNG ; Yuh seok JUNG ; Jae gu CHO ; Yoon seok CHOI ; Jeong seok CHOI ; Guk haeng LEE ; Phil sang CHUNG
Clinical and Experimental Otorhinolaryngology 2019;12(2):107-144
Korean Society of Thyroid-Head and Neck Surgery appointed a Task Force to provide guidance on the implementation of a surgical treatment of oral cancer. MEDLINE databases were searched for articles on subjects related to “surgical management of oral cancer” published in English. Results were restricted to systematic reviews, randomized control trials/controlled clinical trials, and observational studies. The quality of evidence was rated with use RoBANS (Risk of Bias Assessment Tool for Nonrandomized Studies) and AMSTAR (A Measurement Tool to Assess the Methodological Quality of Systematic Reviews). Evidence-based recommendations for practice were ranked according to the American College of Physicians grading system. Additional directives are provided as expert opinions and Delphi questionnaire when insufficient evidence existed. The Committee developed 68 evidence-based recommendations in 34 categories intended to assist clinicians and patients and counselors, and health policy-makers. Proper surgical treatment selection for oral cancer, which is directed by patient- and subsite-specific factors, remains the greatest predictor of successful treatment outcomes. These guidelines are intended for use in conjunction with the individual patient's treatment goals.
Advisory Committees
;
Bias (Epidemiology)
;
Carcinoma, Squamous Cell
;
Counseling
;
Expert Testimony
;
Humans
;
Mouth Neoplasms
;
Neck
;
Republic of Korea
8.Radiologic Comparison of Humeral Position according to the Implant Designs Following Reverse Shoulder Arthroplasty: Analysis between Medial Glenoid/Medial Humerus, Lateral Glenoid/Medial Humerus, and Medial Glenoid/Lateral Humerus Designs
Nam Su CHO ; Ju Hyun NAM ; Se Jung HONG ; Tae Wook KIM ; Myeong Gu LEE ; Jung Tae AHN ; Yong Girl RHEE
Clinics in Shoulder and Elbow 2018;21(4):192-199
BACKGROUND: The currently available reverse shoulder arthroplasty (RSA) designs can be classified into medial glenoid/medial humerus (MGMH), lateral glenoid/medial humerus (LGMH), and medial glenoid/lateral humerus (MGLH) prosthesis designs. The purpose of this study was to radiologically analyze the effect of different RSA designs on humeral position following RSA. METHODS: A total of 50 patients who underwent primary RSA were retrospectively analyzed. Among 50 patients, 33 patients (group A: MGMH) underwent RSA with Aequalis system (Wright, Inc, Bloomington, MN, USA), 6 (group B: LGMH) with Aequalis system using bony increased offset, and 11 (group C: MGLH) with Aequalis Ascend Flex system. The acromiohumeral distance, acromioepiphyseal distance (AED), lateral humeral offset (LHO), LHO from the center of rotation (LHO(COR)), and deltoid length were radiologically measured to quantify the distalization and lateralization of the humerus. RESULTS: The increment in postoperative AED was 19.92 ± 3.93 mm in group A, 24.52 ± 5.25 mm in group B, and 25.97 ± 5.29 mm in group C, respectively (p=0.001). The increment in postoperative LHO was 0.13 ± 6.30 mm, 8.00 ± 12.14 mm, and 7.42 ± 6.88 mm, respectively (p=0.005). The increment in postoperative LHOCOR was 20.76 ± 6.06 mm, 22.04 ± 5.15 mm, and 28.11 ± 4.14 mm, respectively (p=0.002). CONCLUSIONS: The radiologic analysis of the effect of different RSA designs on humeral position following RSA showed significant differences in the increment in postoperative AED, LHO, and LHO(COR) between the 3 groups. Therefore, MGLH design seems to be more effective for humeral distalization and lateralization compared to original Grammont design.
Arthroplasty
;
Humans
;
Humerus
;
Prosthesis Design
;
Retrospective Studies
;
Shoulder
9.Performance of Half-dose Chest Computed Tomography in Lung Malignancy Using an Iterative Reconstruction Technique.
Hee KANG ; Jung Gu PARK ; Se Kyoung PARK ; Beom Su KIM ; Ki Nam LEE ; Kyeung Seung OH
Kosin Medical Journal 2017;32(1):47-57
OBJECTIVES: The purpose of this study was to evaluate the performance of half-dose chest CT using an iterative reconstruction technique in patients with lung malignancies. METHODS: The Dual-source CT scans were obtained and half-dose datasets were reconstructed with 5 different strengths in 38 adults with lung malignancies. Two radiologists graded subjective image quality; noise, contrast and sharpness at the central/peripheral lung, mediastinum and chest wall of the reconstructed half-dose images, compared with those of standard-dose images, using a three-point scale. A lesion assessment; lesion conspicuity and diagnostic confidence, was also performed. The quantitative image noises; contrast-to-noise ratio (CNR) and signal-to-noise ratio (SNR) were measured and compared with those of standard-dose images. RESULTS: The subjective image noise in the half-dose images was less than that of the standard-dose images. The contrast in strengths 2 to 5 was superior, the sharpness of the lung parenchyma in strengths 3 to 5 was inferior, and the CNR/SNR in all strengths were higher than those of standard-dose images (P < 0.05). The improvement of subjective image noise and contrast, the decrease in sharpness, were correlated with strength level (P < 0.05). The lesion conspicuity in half-dose images of strengths 4 and 5 was decreased. The diagnostic confidence of the half-dose images of all strengths was comparable to that of the standard-dose images (P < 0.05). CONCLUSIONS: Half-dose chest CT images using an iterative reconstruction technique show decreased image noise, increased contrast, and diagnostic confidence comparable to standard-dose images. Images reconstructed with strength 2 and 3 appear to be the optimal choice in clinical practice.
Adult
;
Dataset
;
Humans
;
Lung*
;
Mediastinum
;
Noise
;
Signal-To-Noise Ratio
;
Thoracic Wall
;
Thorax*
;
Tomography, X-Ray Computed
10.Association between Serum 25-hydroxyvitamin D Levels and Type 2 Diabetes in Korean Adults
Hyun NAM ; Hye Yeon KIM ; Jin Su CHOI ; Sun Seog KWEON ; Young Hoon LEE ; Hae Sung NAM ; Kyeong Soo PARK ; So Yeon RYU ; Seong Woo CHOI ; Su Hyun OH ; Sun A KIM ; Min Ho SHIN
Chonnam Medical Journal 2017;53(1):73-77
Previous studies have suggested that a vitamin D deficiency increases the risk of type 2 diabetes. This study evaluated the association between serum vitamin D levels and type 2 diabetes in Korean adults. This study included 9,014 subjects (3,600 males and 5,414 females) aged ≥50 years who participated in the Dong-gu Study. The subjects were divided into groups in whom the serum vitamin D level was severely deficient (<10 ng/mL), deficient (10 to <20 ng/mL), insufficient (20 to <30 ng/mL) and sufficient (≥30 ng/mL). Type 2 diabetes was defined by a fasting blood glucose level of ≥126 mg/dL and/or an HbA1c proportion of ≥6.5% and/or self-reported current use of diabetes medication. Multiple logistic regression was performed to evaluate the association between vitamin D status and type 2 diabetes. The age- and sex-adjusted prevalence of type 2 diabetes was 22.6%, 22.5% and 18.4% and 12.7% for severely deficient, deficient, insufficient, and sufficient, respectively. Multivariate modeling revealed that subjects with insufficient or sufficient vitamin D levels were at a lower risk of type 2 diabetes than were subjects with deficient vitamin D levels [odds ratio (OR), 0.82; 95% confidence interval (CI), 0.71–0.94 and OR, 0.51; 95% CI, 0.35–0.74, respectively]. Higher serum vitamin D levels were associated with a reduced risk of diabetes in Korean adults, suggesting that vitamin D may play a role in the pathogenesis of diabetes.
Adult
;
Blood Glucose
;
Cross-Sectional Studies
;
Diabetes Mellitus, Type 2
;
Fasting
;
Humans
;
Logistic Models
;
Male
;
Prevalence
;
Vitamin D
;
Vitamin D Deficiency

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