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137 results on '"Hong, Che Ry"'

1. Somatic uniparental disomy mitigates the most damaging EFL1 allele combination in Shwachman-Diamond syndrome

Author

Lee, Sangmoon, Shin, Chang Hoon, Lee, Jawon, Jeong, Seong Dong, Hong, Che Ry, Kim, Jun-Dae, Kim, Ah-Ra, Park, Boryeong, Son, Soo Jin, Kokhan, Oleksandr, Yoo, Taekyeong, Ko, Jae Sung, Sohn, Young Bae, Kim, Ok-Hwa, Ko, Jung Min, Cho, Tae-Joon, Wright, Nathan T., Seong, Je Kyung, Jin, Suk-Won, Kang, Hyoung Jin, Kim, Hyeon Ho, and Choi, Murim

Published
2021
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5. The dialkyl resorcinol stemphol disrupts calcium homeostasis to trigger programmed immunogenic necrosis in cancer

Author

Ji, Seungwon, Lee, Jin-Young, Schrör, Jan, Mazumder, Aloran, Jang, Dong Man, Chateauvieux, Sébastien, Schnekenburger, Michael, Hong, Che Ry, Christov, Christo, Kang, Hyoung Jin, Lee, Youngjo, Han, Byung Woo, Kim, Kyu-Won, Shin, Hee-Young, Dicato, Mario, Cerella, Claudia, König, Gabriele M., Orlikova, Barbora, and Diederich, Marc

Published
2018
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15. Effectiveness and Safety of Clofarabine Monotherapy or Combination Treatment in Relapsed/Refractory Childhood Acute Lymphoblastic Leukemia: A Pragmatic, Non-interventional Study in Korea

Author

Choi, Jung Yoon, primary, Hong, Che Ry, additional, Hong, Kyung Taek, additional, Kang, Hyoung Jin, additional, Kim, Seongkoo, additional, Lee, Jae Wook, additional, Jang, Pil Sang, additional, Chung, Nack-Gyun, additional, Cho, Bin, additional, Kim, Hyery, additional, Koh, Kyung-Nam, additional, Im, Ho Joon, additional, Seo, Jong Jin, additional, Hahn, Seung Min, additional, Han, Jung Woo, additional, Lyu, Chuhl Joo, additional, Yang, Eu Jeen, additional, Lim, Young Tak, additional, Yoo, Keon Hee, additional, Koo, Hong Hoe, additional, Kook, Hoon, additional, Jeon, In Sang, additional, Cho, Hana, additional, and Shin, Hee Young, additional

Published
2021
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18. Pharmacokinetic and Pharmacodynamic Characteristics of Tripegfilgrastim, a Pegylated G‐CSF, in Pediatric Patients with Solid Tumors.

Author

Lee, Soyoung, Hong, Kyung Taek, Moon, Seol Ju, Choi, Jung Yoon, Hong, Che Ry, Shin, Hee Young, Cho, Joo‐Youn, Jang, In‐Jin, Yu, Kyung‐Sang, Oh, Jaeseong, and Kang, Hyoung Jin

Subjects
GRANULOCYTES, GRANULOCYTE-colony stimulating factor, CHILD patients, PHARMACOKINETICS, AGE groups
Abstract

A long‐acting granulocyte colony‐stimulating factor, tripegfilgrastim, was approved in Korea for the prevention of chemotherapy‐induced neutropenia in adult patients. In this study, we evaluated the pharmacokinetics, pharmacodynamics, and safety of tripegfilgrastim in pediatric patients. A phase I, open‐label, single ascending‐dose study was performed in pediatric patients with solid tumors or lymphoma (ClinicalTrials.gov, NCT02963389). The patients were stratified according to age groups (aged 6 to 12 or 12 to 19 years) and received a single subcutaneous dose of tripegfilgrastim 60 μg/kg or 100 μg/kg. Tripegfilgrastim was administered 24 hours after the end of the chemotherapy, and serial blood sampling and safety monitoring were conducted. Twenty‐seven patients with solid tumors were enrolled in this study. Tripegfilgrastim was detectable in plasma for an extended period (terminal half‐life > 40 hours), and plasma concentrations increased slightly less than dose proportionally. The mean duration of grade 4 neutropenia was reduced as the average tripegfilgrastim concentration during the initial neutrophil recovery process increased. No substantial differences in the pharmacokinetic and pharmacodynamic responses were observed between the two age groups. When stratified by body weight, weighing more than 45 kg has a higher risk of a prolonged neutropenia period when receiving the lower dose (60 μg/kg) of tripegfilgrastim. Tripegfilgrastim was generally safe and well‐tolerated in the pediatric patients. These results justify further clinical investigations of tripegfilgrastim at 100 μg/kg dose in pediatric patients. [ABSTRACT FROM AUTHOR]

Published
2022
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19. Somatic uniparental disomy with a rare EFL1 variant causes Shwachman-Diamond syndrome through dysregulating ribosomal protein synthesis

Author

Lee, Sangmoon, Shin, Chang Hoon, Hong, Che Ry, Kim, Jun-Dae, Kim, Ah-Ra, Lee, Jawon, Kokhan, Oleksandr, Yoo, Taekyeong, Sohn, Young Bae, Kim, Ok-Hwa, Ko, Jung Min, Cho, Tae-Joon, Wright, Nathan T., Jin, Suk-Won, Kang, Hyoung Jin, Kim, Hyeon Ho, and Choi, Murim

Subjects
congenital, hereditary, and neonatal diseases and abnormalities
Abstract

We present three unrelated Korean Shwachman-Diamond syndrome (SDS) patients that carry an incomplete but identical homozygous EFL1 p.Thr1069Ala variant due to a bone marrow-specific mosaic uniparental disomy (UPD) in chromosome 15. This rare variant is found in 0.017% of East Asians and is asymptomatic in a heterozygous status, but harbors a hypomorphic effect, leading to 80S assembly of ribosomal protein (RP) transcripts. We propose a novel somatically-induced pathogenesis mechanism and EFL1 dysfunction that eventually leads to aberrant translational control and ribosomopathy.

Published
2018
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21. Somatic uniparental disomy mitigates the most damagingEFL1allele combination in Shwachman-Diamond syndrome

Author

Lee, Sangmoon, primary, Shin, Chang Hoon, additional, Lee, Jawon, additional, Jeong, Seong Dong, additional, Hong, Che Ry, additional, Kim, Jun-Dae, additional, Kim, Ah-Ra, additional, Son, Soo Jin, additional, Kokhan, Oleksandr, additional, Yoo, Taekyeong, additional, Ko, Jae Sung, additional, Sohn, Young Bae, additional, Kim, Ok-Hwa, additional, Ko, Jung Min, additional, Cho, Tae-Joon, additional, Wright, Nathan T., additional, Seong, Je Kyung, additional, Jin, Suk-Won, additional, Kang, Hyoung Jin, additional, Kim, Hyeon Ho, additional, and Choi, Murim, additional

Published
2018
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25. WHIM Syndrome With a Novel CXCR4 Variant in a Korean Child

Author

Shin, Dong Woo, primary, Park, Si Nae, additional, Kim, Sung-Min, additional, Im, Kyongok, additional, Kim, Jung-Ah, additional, Hong, Kyung Taek, additional, Choi, Jung Yoon, additional, Hong, Che Ry, additional, Park, Kyung Duk, additional, Shin, Hee Young, additional, Kang, Hyoung Jin, additional, Kim, Hyun Kyung, additional, and Lee, Dong Soon, additional

Published
2017
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27. Pediatric patients undergoing hematopoietic stem cell transplantation can greatly benefit from a novel once-daily intravenous busulfan dosing nomogram

Author

Rhee, Su-jin, primary, Lee, Ji Won, additional, Yu, Kyung-Sang, additional, Hong, Kyung Taek, additional, Choi, Jung Yoon, additional, Hong, Che Ry, additional, Park, Kyung Duk, additional, Shin, Hee Young, additional, Song, Sang Hoon, additional, Kang, Hyoung Jin, additional, and Lee, Howard, additional

Published
2017
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29. Pharmacokinetic Analysis of Continuous Intravenous Infusion of Lipo-Prostaglandin E1 As a Prophylaxis of Hepatic Veno-Occlusive Disease after Hematopoietic Stem Cell Transplantation

Author

Hong, Che Ry, primary, Choi, Yewon, additional, Oh, Jaeseong, additional, Sunwoo, Jung, additional, Hong, Kyung Taek, additional, Choi, Jung Yoon, additional, Yu, Kyung-Sang, additional, Park, Kyung Duk, additional, Shin, Hee Young, additional, and Kang, Hyoung Jin, additional

Published
2016
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31. Targeted busulfan and fludarabine-based conditioning for bone marrow transplantation in chronic granulomatous disease

Author

Ju, Hee Young, primary, Kang, Hyoung Jin, additional, Hong, Che Ry, additional, Lee, Ji Won, additional, Kim, Hyery, additional, Song, Sang Hoon, additional, Yu, Kyung-Sang, additional, Jang, In-Jin, additional, Park, June Dong, additional, Park, Kyung Duk, additional, Shin, Hee Young, additional, Kim, Joong-Gon, additional, and Ahn, Hyo Seop, additional

Published
2016
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32. Association of systolic blood pressure drop with intravenous administration of itraconazole in children with hemato-oncologic disease

Author

Lee,Hyoung Jin, Lee,Bongjin, Park,June Dong, Jeong,Hyung Joo, Choi,Yu Hyeon, Ju,Hee Young, Hong,Che Ry, Lee,Ji Won, Kim,Hyery, Suh,Dong In, Park,Kyung Duk, Kang,Hyoung Jin, Shin,Hee Young, Ahn,Hyo Seop, Lee,Hyoung Jin, Lee,Bongjin, Park,June Dong, Jeong,Hyung Joo, Choi,Yu Hyeon, Ju,Hee Young, Hong,Che Ry, Lee,Ji Won, Kim,Hyery, Suh,Dong In, Park,Kyung Duk, Kang,Hyoung Jin, Shin,Hee Young, and Ahn,Hyo Seop

Abstract

Hyeong Jin Lee,1,* Bongjin Lee,2,* June Dong Park,2 Hyung Joo Jeong,2 Yu Hyeon Choi,2 Hee Young Ju,1 Che Ry Hong,1 Ji Won Lee,1 Hyery Kim,1 Dong In Suh,3 Kyung Duk Park,1 Hyoung Jin Kang,1 Hee Young Shin,1 Hyo Seop Ahn1 1Department of Pediatrics, Cancer Research Institute, 2Division of Pediatric Intensive Care, Department of Pediatrics, 3Division of Pulmonology, Department of Pediatrics, Seoul National University College of Medicine, Seoul National University, Seoul, South Korea *These authors contributed equally to this work Purpose: Although few adverse effects have been reported for itraconazole, a widely used antifungal therapy for febrile neutropenia, we found intravenous (IV) itraconazole to be associated with serious cases of blood pressure (BP) drop. We therefore evaluated the incidence and risk factors for BP drop during IV administration of the drug.Materials and methods: We reviewed the medical records of children with hemato-oncologic disease who were treated with IV itraconazole from January 2012 to December 2013. By analyzing systolic BP (SBP) measurements made from 4 hours before through to 4 hours after itraconazole administration, we evaluated the changes in SBP and the risk factors for an SBP drop, especially clinically meaningful (≥20%) drops.Results: Itraconazole was administered 2,627 times to 180 patients. The SBP during the 4 hours following itraconazole administration was lower than during the 4 hours before administration (104 [53.0–160.33 mmHg] versus 105 [59.8–148.3 mmHg]; P<0.001). The decrease in SBP was associated with the application of continuous renal replacement therapy (CRRT) (P=0.012) and the use of inotropic (P=0.005) and hypotensive drugs (P=0.021). A clinically meaningful SBP drop was seen in 5.37% (141 out of 2,627) of the administrations, and the use of inotropics (odds ratio [OR] 6.70, 95% confidence interval [CI] 3.22–13.92; P<0.001), reducing the dose of inotropic

Published
2015

33. Association of systolic blood pressure drop with intravenous administration of itraconazole in children with hemato-oncologic disease

Author

Park, June Dong, primary, Lee, Hyoung Jin, additional, Lee, Bongjin, additional, Jeong, Hyung Joo, additional, Choi, Yu Hyeon, additional, Ju, Hee Young, additional, Hong, Che Ry, additional, Lee, Ji Won, additional, Kim, Hyery, additional, Suh, Dong In, additional, Park, Kyung Duk, additional, Kang, Hyoung Jin, additional, Shin, Hee Young, additional, and Ahn, Hyo Seop, additional

Published
2015
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36. Very Late Relapse of Bilateral Retinoblastoma

Author

Choi, Jung Yoon, primary, Kang, Hyoung Jin, additional, Lee, Ji Won, additional, Ju, Hee Young, additional, Hong, Che Ry, additional, Kim, Hyery, additional, Yu, Young Suk, additional, Park, Sung-Hye, additional, Cheon, Jung-Eun, additional, Park, Kyung Duk, additional, and Shin, Hee Young, additional

Published
2015
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40. Hemophagocytic lymphohistiocytosis diagnosed by brain biopsy

Author

Ju, Hee Young, primary, Hong, Che Ry, additional, Kim, Sung Jin, additional, Lee, Ji Won, additional, Kim, Hyery, additional, Kang, Hyoung Jin, additional, Park, Kyung Duk, additional, Shin, Hee Young, additional, Chae, Jong-Hee, additional, Phi, Ji Hoon, additional, Cheon, Jung-Eun, additional, Park, Sung-Hye, additional, and Ahn, Hyo Seop, additional

Published
2015
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50. Somatic uniparental disomy mitigates the most damaging EFL1allele combination in Shwachman-Diamond syndrome

Author

Lee, Sangmoon, Shin, Chang Hoon, Lee, Jawon, Jeong, Seong Dong, Hong, Che Ry, Kim, Jun-Dae, Kim, Ah-Ra, Park, Boryeong, Son, Soo Jin, Kokhan, Oleksandr, Yoo, Taekyeong, Ko, Jae Sung, Sohn, Young Bae, Kim, Ok-Hwa, Ko, Jung Min, Cho, Tae-Joon, Wright, Nathan T., Seong, Je Kyung, Jin, Suk-Won, Kang, Hyoung Jin, Kim, Hyeon Ho, and Choi, Murim

Abstract

Shwachman-Diamond syndrome (SDS; OMIM: #260400) is caused by variants in SBDS(Shwachman-Bodian-Diamond syndrome gene), which encodes a protein that plays an important role in ribosome assembly. Recent reports suggest that recessive variants in EFL1are also responsible for SDS. However, the precise genetic mechanism that leads to EFL1-induced SDS remains incompletely understood. Here we present three unrelated Korean SDS patients that carry biallelic pathogenic variants in EFL1with biased allele frequencies, resulting from a bone marrow-specific somatic uniparental disomy (UPD) in chromosome 15. The recombination events generated cells that were homozygous for the relatively milder variant, allowing for the evasion of catastrophic physiological consequences. Still, the milder EFL1variant was solely able to impair 80S ribosome assembly and induce SDS features in cell line and animal models. The loss of EFL1resulted in a pronounced inhibition of terminal oligo-pyrimidine element-containing ribosomal protein transcript 80S assembly. Therefore, we propose a more accurate pathogenesis mechanism of EFL1 dysfunction that eventually leads to aberrant translational control and ribosomopathy.

Published
2021
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