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Cardiac function associated with home ventilator care in Duchenne muscular dystrophy
Lee, Sangheun,Lee, Heeyoung,Eun, Lucy Youngmin,Gang, Seung Woong The Korean Pediatric Society 2018 Clinical and Experimental Pediatrics (CEP) Vol.61 No.2
Purpose: Cardiomyopathy is becoming the leading cause of death in patients with Duchenne muscular dystrophy because mechanically assisted lung ventilation and assisted coughing have helped resolve respiratory complications. To clarify cardiopulmonary function, we compared cardiac function between the home ventilator-assisted and non-ventilator-assisted groups. Methods: We retrospectively reviewed patients with Duchenne muscular dystrophy from January 2010 to March 2016 at Gangnam Severance Hospital. Demographic characteristics, pulmonary function, and echocardiography data were investigated. Results: Fifty-four patients with Duchenne muscular dystrophy were divided into 2 groups: home ventilator-assisted and non-ventilator-assisted. The patients in the home ventilator group were older ($16.25{\pm}1.85years$) than those in the nonventilator group ($14.73{\pm}1.36years$) (P=0.001). Height, weight, and body surface area did not differ significantly between groups. The home ventilator group had a lower seated functional vital capacity ($1,038{\pm}620.41mL$) than the nonventilator group ($1,455{\pm}603.2mL$). Mean left ventricular ejection fraction and fractional shortening were greater in the home ventilator group, but the data did not show any statistical difference. The early ventricular filling velocity/late ventricular filling velocity ratio ($1.7{\pm}0.44$) was lower in the home ventilator group than in the nonventilator group ($2.02{\pm}0.62$. The mitral valve annular systolic velocity was higher in the home ventilator group (estimated ${\beta}$, 1.06; standard error, 0.48). Patients with Duchenne muscular dystrophy on a ventilator may have better systolic and diastolic cardiac functions. Conclusion: Noninvasive ventilator assistance can help preserve cardiac function. Therefore, early utilization of noninvasive ventilation or oxygen may positively influence cardiac function in patients with Duchenne muscular dystrophy.
( Sangheun Lee ),( Jung Yoen Lee ),( Beom Kyung Kim ),( Seung Up Kim ),( Jun Yong Park ),( Do Young Kim ),( Hye Jin Ku ),( Kwang Hyub Han ),( Sang Hoon Ahn ) 대한간학회 2013 춘·추계 학술대회 (KASL) Vol.2013 No.1
Background/Aims: Tenofovir disoproxil fumarate (TDF) is a potent antiviral agent in both treatment-naive and lamivudineresistant chronic hepatitis B. However, antiviral efficacy of TDF has not been studied in suboptimal response to various rescue therapies due to multiple antiviral resistances. Here, we investigated the viral kinetics and response of TDF monotherapy in CHB patients with lamivudine resistance (LAM-R), adefovirresistance (ADV-R) and/or entecavir-resistance (ETV-R). Methods: From December 2012 to April 2013, 164 patients with suboptimal response to current rescue therapies were switched to TDF monotherapy and followed up at least 3 months. All patients had either LAM-R (n=105) or multipledrug resistance (MDR) including LAM-R+ADV-R (n=18), LAM-R+ETV-R (n=38), LAM-R+ADV-R+ETV-R (n=3). The mean reduction in serum HBV DNA levels and virologic response, defined as serum HBV DNA levels of < 60 IU/ml, were analyzed according to LAM-R or MDR. Results: At baseline, patients` mean serum HBV DNA level was 5.2 log10 (range; 2.3-8.2) IU/mL and 5.0 log10 (range; 2.2- 8.2) IU/mL in LAM-R and MDR group, respectively. At week 12, mean serum HBV DNA levels was significantly reduced from baseline in both LAM-R group (-2.8 log10 IU/mL, P<0.001) and MDR group (-2.5 log10 IU/mL, P<0.001). In MDR group, the speed of viral suppression was not different among the type and number of HBV mutations (LAM-R + ADV-R, -2.6; LAM-R + ETV-R, -2.3; LAM-R + ADV-R + ETV-R, -2.5; P<0.05 of all). However, the proportion of virologic response was higher in LAM-R group than that of MDR group (n=46, 43.8% vs. n=16, 27.1%, P=0.034) at Week 12, which means that viral suppression was dramatically achieved in some LAM-R patients. Conclusion: The switch to TDF monotherapy showed a rapid viral suppression in suboptimal responders with LAM-R and MDR with comparable efficacy. A longer follow-up study is undergoing in this patients` group
Liver Inflammation and Fibrotic Burden Are Associated with Endocrine Metabolism
( Sangheun Lee ),( Jin Woo Park ),( Ja Sung Choi ),( Ki Jun Han ) 대한간학회 2017 춘·추계 학술대회 (KASL) Vol.2017 No.1
Aims: Liver is one of main organs to control the glucose and lipid metabolism. We aimed to investigate that liver inflammation is associated with HbA1c and triglyceride which represent the degree of glucose and lipid control, respectively. Methods: Data from the Korean National Health and Nutrition Examination Surveys (KNHANES) 2010-2015 database were analyzed. Aspartate aminotransferase (AST), alanine aminotransferase (ALT) and FIB-4 were used for the evaluation of liver inflammation and fibrosis. Significant glucose and lipid metabolism disorders were defined as high HbA1c (≥6.5%) and high triglyceride (≥200mg/dL). Results: A total 30,741,128 participants was analyzed. The FIB-4 was significantly higher in subjects with high HbA1c (fully adjusted OR 1.05; 95% CI 1.002-1.10; p = 0.0374), and the prevalence of subjects with both AST (≥80 IU/L) and ALT (≥80 IU/L) were significantly higher in high HbA1c (fully adjusted OR 2.89; 95% CI 1.83-4.45; p = 2.70E-06 and 2.25; 95% CI 1.67-3.04; p = 7.71E-08, respectively). The prevalence of subjects with both AST (≥80 IU/L) and ALT (≥80 IU/L) were also significantly higher in high triglyceride (fully adjusted OR 2.72; 95% CI 1.89-3.88; p = 3.92E-08 and 2.12; 95% CI 1.67- 2.68; p = 5.63E-10, respectively). However, FIB-4 did not show a significant association with high triglyceride (fully adjusted OR 1.02; 95% CI 0.98-1.06; p = 0.2645). Conclusions: AST (≥80 IU/L), ALT (≥80 IU/L) and FIB-4 increase showed significant relationships with high HbA1c and triglyceride levels. The control liver inflammation and fibrosis may improve the endocrine metabolism disorder.
( Sangheun Lee ),( Beom Kyung Kim ),( Seung Up Kim ),( Ja Kyung Kim ),( Hyun Woong Lee ),( Soo Young Park ),( Jun Yong Park ),( Do Young Kim ),( Sang Hoon Ahn ),( Chae Yoon Chon ),( Kwang Hyub Han ) 대한간학회 2013 춘·추계 학술대회 (KASL) Vol.2013 No.1
Background/Aims: Sorafenib is recommended as a standard treatment for advanced hepatocellular carcinoma (HCC). However, in Asia, owing to its high cost and marginal therapeutic benefit, there have been few studies focusing patients treated with sorafenib as the first-line therapy. This study investigated the efficacy of sorafenib as the first-line therapy in Korean patients with advanced HCC. Methods: From 2007 to 2012, 86 patients with advanced HCC from 5 hospitals showing Barcelona Clinic Liver Cancer stage C were treated with sorafenib as the first-line therapy. The overall survival (OS) and progression-free survival (PFS) were analyzed. Results: Among entire cohort, the median OS and PFS were 5.0 (range, 4.0-5.9) and 3.2 (range, 2.6-3.7) months, respectively. Regarding OS, Child-Pugh class A (6.0 vs. 2.8 months), maximal tumor diameter <5cm (6.0 vs. 4.3 months), the baseline α-fetoprotein (AFP) < 200 ng/mL (5.8 vs. 4.1 months), and the advent of hand foot skin reaction (HFSR) of ≥ grade 2 (5.9 vs. 4.0 months) were independent favorable predictors for OS (all P<0.05). Similarly, regarding PFS, Child-Pugh class A (4.3 vs. 2.1 months), maximal tumor diameter < 5 cm (3.9 vs. 2.8 months), the baseline AFP <200 ng/mL (5.6 vs. 2.8 months) and the advent of HFSR of ≥ grade 2 (4.5 vs. 2.7 months) were independent favorable predictors (all P<0.05). Conclusion: Sorafenib shows a significant survival benefit in Korean patients with treatment-naive advanced HCC. Favorable predictors in our study can be used to identify the subpopulation in whom sorafenib can maximize its efficacy.
Phosphate Is Associated with the Severity of Acute Hepatitis A
( Sangheun Lee ),( Young Hoon Choi ),( Ki Jun Han ),( Ja Sung Choi ),( Jin Woo Park ),( Hyun-jeong Han ) 대한간학회 2018 춘·추계 학술대회 (KASL) Vol.2018 No.1
Aims: Acute hepatitis A (AH-A) is one of the most common forms of viral hepatitis, and distinct clinical features are associated with the prodromal, icteric, and recovery phases. This study was designed to investigate the correlations of various clinical parameters with severity in AH-A patients in each of these 3 phases. Methods: The medical records of 455 patients diagnosed with AH-A were retrospectively reviewed. The prodromal, icteric, and recovery phases were defined by the patterns of changes observed after admission in aspartate aminotransferase (AST), alanine aminotransferase (ALT), and bilirubin levels. Clinical parameters, including phosphate levels, were analyzed to identify their associations with the peak levels of AST, ALT, and bilirubin. Results: Of the patients, 129 (28.4%) were admitted in the prodromal phase, 187 (41.1%) in the icteric phase, and 139 (30.5%) in the recovery phase. Phosphate levels showed an inverse relationship with the peak AST and ALT levels in the prodromal phase (P=0.011 and P=0.005, respectively). Prothrombin time (PT, %) showed a negative relationship with peak AST levels throughout the prodromal, icteric, and recovery phases (P=0.039, P=0.028, and P=0.001, respectively), the peak ALT level in the prodromal phases (P=0.038) and the peak bilirubin level in the icteric phase (P=0.029). Conclusions: In conclusion, the baseline phosphate, AST, and ALT levels, as well as PT and the platelet count, were correlated with the peak levels of AST, ALT, and bilirubin in patients with AH-A.
( Sangheun Lee ),( Jun Yong Park ),( Kijun Song ),( Do Young Kim ),( Beom Kyung Kim ),( Seung Up Kim ),( Hye Jin Ku ),( Kwang-hyub Han ),( Sang Hoon Ahn ) 대한소화기학회 2015 Gut and Liver Vol.9 No.6
Background/Aims: The aim of this study was to evaluate the estimated glomerular filtration rate (eGFR) during telbivudine (LdT) versus entecavir (ETV) treatment in chronic hepatitis B (CHB) patients with underlying comorbidities such as diabetes mellitus (DM), hypertension, and cirrhosis. Methods: From 2010 to 2012, 116 CHB patients treated with LdT and 578 treated with ETV were compared in this real-practice cohort.The mean changes in eGFR (Modification of Diet in Renal Disease [MDRD] formula) from baseline to months 6, 12, and 18 were analyzed using a linear mixed model. Results: In LdT-treated patients, the mean eGFR increased by 7.6% at month 18 compared with the eGFR at baseline (MDRD formulain mL/min/1.73 m2). However, in ETV-treated patients, the mean eGFR decreased by 4.1% at month 18 compared with the eGFR at baseline. In the LdT-treated patients with DM, hypertension, cirrhosis or low eGFR <90 mL/min/1.73 m2, the mean eGFR showed a steady improvement, whereas the mean eGFR was reduced in the same subgroups of ETV-treated patients. Conclusions: The eGFR gradually increased over time during LdT treatment, especially in patients with mild abnormal eGFR at baseline, and in those with DM, hypertension,and cirrhosis, whereas a reduction in eGFR was seen with ETV treatment. (Gut Liver 2015,9:776-783)
TACE 및 Adjuvant Brivanib 치료로 완치된 뒤 21개월 후 재발성 간암으로 이식한 증례
이상헌 ( Sangheun Lee ),김미나 ( Mi Na Kim ),전영은 ( Young Eun Chon ),김범경 ( Beom Kyung Kim ),김승업 ( Seung Up Kim ),박준용 ( Jun Yong Park ),김도영 ( Do Young Kim ),안상훈 ( Sang Hoon Ahn ),전재윤 ( Chae Yoon Chon ),한광협 ( 대한간암학회 2013 대한간암학회지 Vol.13 No.1
Hepatocelluar carcinoma (HCC) is the most common primary liver cancer in the world and the most prevalent cancer among patients liver cirrhosis. The management of HCC depends on tumor stage and the degree of liver dysfunction. Patients with intermediate-stage HCC are ineligible for surgical or local ablative treatments. Current treatment guidelines recommend trans-arterial chemoembolization (TACE) for intermediate stage of HCC. However, tumor recurrence after TACE is universal and the survival benefit is relatively small. Hence, new strategies are needed to improve the outcome of HCC patients undergoing TACE. Recently, the combination of target agents with TACE has shown promising overall survival in advanced HCC. It is necessary to investigate new treat strategy how to increase treatment outcome of advanced HCC by new treat strategy.