• Journal of Yeungnam Medical Science
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• v.34 no.2
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• pp.285-289
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• 2017

Pheochromocytoma can present with various symptoms including cardiogenic shock and cardiac arrest. Particularly, in cases of cardiogenic shock of unknown origin, pheochromocytoma should be considered. A 20-year-old woman without any medical history visited our emergency department due to nausea, vomiting, headache, and chest pain. Echocardiography revealed severe left ventricular dysfunction. Mechanical ventilation and veno-arterial extracorporeal membrane oxygenation (ECMO) were implemented owing to her unstable vital signs. For unstable vital sign and cardiogenic shock in a young woman without any previous medical history, pheochromocytoma was considered and diagnosed based on elevated levels of catecholamine derivatives in a 24-hour urine sample. Cardiac function recovered and ECMO was discontinued on the 5th day of hospitalization. She later underwent an elective adrenalectomy and no recurrence was found during the follow-up period. We reported a case of pheochromocytoma which was presented with cardiogenic shock in a young woman with no concomitant disease, and successfully treated with ECMO followed by an elective adrenalectomy.

• The Korean Journal of Physiology and Pharmacology
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• v.3 no.2
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• pp.223-230
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• 1999

Alterations of cardiovascular function associated with various thyroid states have been studied. In hyperthyroidism left ventricular contractility and relaxation velocity were increased, whereas these parameters were decreased in hypothyroidism. The mechanisms for these changes have been suggested to include alterations in the expression and/or activity levels of various proteins; ${\alpha}-myosin$ heavy chain, ${\beta}-myosin$ heavy chain, ${\beta}-receptors,$ the guanine nucleotide-binding regulatory protein, and the sarcolemmal $Ca^{2+}-ATPase.$ All these cellular alterations may be associated with changes in the intracellular $Ca^{2+}$ concentration. The most important regulator of intracellular $Ca^{2+}$ concentration is the sarcoplasmic reticulum (SR), which serves as a $Ca^{2+}$ sink during relaxation and as a $Ca^{2+}$ source during contraction. The $Ca^{2+}-ATPase$ and phospholamban are the most important proteins in the SR membrane for muscle relaxation. The dephosphorylated phospholamban inhibits the SR $Ca^{2+}-ATPase$ through a direct interaction, and phosphorylation of phospholamban relieves the inhibition. In the present study, quantitative changes of $Ca^{2+}-ATPase$ and phospholamban expression and the functional consequences of these changes in various thyroid states were investigated. The effects of thyroid hormones on (1) SR $Ca^{2+}$ uptake, (2) phosphorylation levels of phospholamban, (3) SR $Ca^{2+}-ATPase$ and phospholamban protein levels, (4) phospholamban mRNA levels were examined. Our findings indicate that hyperthyroidism is associated with increases in $Ca^{2+}-ATPase$ and decreases in phospholamban levels whereas opposite changes in these proteins occur in hypothyroidism.

• Journal of Chest Surgery
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• v.33 no.4
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• pp.285-289
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• 2000

Background: For AVR using conventional prosthetic valves in adult patients with a narrow aortic root, aortic root enlargement is necessary to reduce postoperative pressure gradient across the aortic valve (ΔP). An evaluation of early and mid-term results of aortic root enlargement with AVR and echocardiographic follow up of ΔP and left ventricular function was performed. Method: From Aug. 1991 to Feb. 1998, eighteen patients aged 17 to 59 years(mean, 38$\pm$12 years) underwent Manouguian procedure with AVR. Aortic annular circumstance was enlarged 10.0mm to 18.0mm(mean, 12.6$\pm$6.3mm). Eight patients(44.0%) had NYHA class III status before operation, and seven cases of them underwent concomitant MVR. Valve pathology was ASr in 6 cases, AS in 4 cases, nd ASr+MSr in 8 cases. Replaced valve size was 21mm in 8 cases and 23 mm in 10 cases, and St. Jude Medical mechanical valve was used in 10 cases and Carbomedics in 8 cases. Result: Follow-up duration was 6 to 57 months (mean, 26$\pm$18 months), and total follow-up was 287 patient-year. There were one hospital death and one late death, therefore, actuarial survival rate was 85.7% at 56 months. Peak ΔP wad decreased significantly at postoperative mid-term period as 13$\pm$5mmHg, compared with thepreoperative one (42$\pm$8mmHg) (p<0.01). LVM(gm/$m^2$) was also diminished as 35.8%(115$\pm$36gm/$m^2$)at postoperative mid-term period, compared with preoperative one (179$\pm$56gm/$m^2$)(p<0.05). Conclusion: There were no specific complications related to the procedure. And we could have adequate enlargement of aortic annulus to suitable prosthetic valve that have no effect of patient-prosthese mismatch.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.14 no.1
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• pp.37-41
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• 2014

Fabry disease is an X-linked disease caused by deficiency of the lysosomal enzyme alpha-galactosidase A. Affected males present anhydrosis, acroparesthesia and angiokeratoma, and subsequently cardiac, cerebral and renal complications are followed. Females and atypical variants show heterogeneous clinical symptoms. In 2001, two recombinant enzymes were approved for Fabry disease: agalsidase alpha and agalsidase beta. Since the introduction of enzyme replacement therapy (ERT), the number of long-term follow-up studies has been reported. Long-term ERT showed effectiveness on renal function in patients with chronic kidney disease, decrease or stabilization of left ventricular mass, and improvement of pain and quality of life. However, there were limited effects on cerebrovascular events and their mortality. Current literatures on the clinical effect of ERT have reported limited datain adult patients who have already advanced disease. Therefore, further study for pre-symptomatic patients and atypical variants is needed to verify the impact of ERT. This review summarized recent progresses in ERT and limitations of long-term effect of ERT in patients with Fabry disease.

• Childhood Kidney Diseases
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• v.24 no.2
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• pp.107-114
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• 2020

Purpose: The purpose of this study was to evaluate the prevalence, clinical characteristics, and long-term clinical effects of hypertension in Korean childhood-onset systemic lupus erythematous (SLE) patients. Methods: The medical records of SLE patients, diagnosed by 2019 SLE European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) classification criteria, who visited Samsung Medical Center from January 2009 to May 2019 were reviewed. Disease activity and long-term damage were evaluated using the Modified Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) and the Pediatric Systemic Lupus International Collaborating Clinics/ACR Damage Index (Ped-SDI), respectively. The sex-, age- and height-blood pressure standards recommended by the American Academy of Pediatrics 2017 guideline was used to define hypertension. Results: A total of 32 patients were enrolled in this study. The median follow-up duration was 7.3 years and females were predominant. The median ages at SLE and hypertension diagnoses were 14.2 and 14.3 years, respectively. The biopsy-proven lupus nephritis was detected in 90.6% and 37.5% were class IV. During the follow-up, 12 patients (37.5%) had hypertension. Among them, 2 patients had 3 episodes of posterior reversible encephalopathy syndrome and 5 patients had left ventricular hypertrophy (LVH). Univariate analysis showed baseline hypertension was significantly correlated with a lower estimated glomerular filtration rate, higher body mass index and SLEDAI at baseline. The development of hypertension during the follow-up was significantly correlated with obesity, LVH, and higher Ped-SDI. Conclusion: Our study revealed that hypertension in pediatric SLE is associated with obesity and renal function at SLE diagnosis and could affect long-term damage.

• Clinical and Molecular Hepatology
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• v.24 no.4
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• pp.409-416
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• 2018

Background/Aims: Left ventricular diastolic dysfunction (LVDD) is an early manifestation of cardiac dysfunction in patients with liver cirrhosis (LC). However, the effect of LVDD on survival has not been clarified, especially in decompensated LC. Methods: We prospectively enrolled 70 patients with decompensated LC, including ascites or variceal bleeding, at Daejeon St. Mary's Hospital from April 2013 to April 2015. The cardiac function of these patients was evaluated using 2D echocardiography with tissue Doppler imaging. The diagnosis of LVDD was based on the American Society of Echocardiography guidelines. The primary endpoint was overall survival. Results: Forty-four patients (62.9%) had LVDD. During follow-up (22.3 months), 18 patients died (16 with LVDD and 2 without LVDD). The survival rate was significantly lower in patients with LVDD than in those without LVDD (31.1 months vs. 42.6 months, P=0.01). In a multivariate analysis, the Child-Pugh score and LVDD were independent predictors of survival. Moreover, patients with a ratio of early filling velocity to early diastolic mitral annular velocity (E/e') ${\geq}10$ (LVDD grade 2) had lower survival than patients with E/e' ratio < 10. Conclusions: The presence of LVDD is associated with poor survival in patients with decompensated LC. Therefore, it may be important to monitor and closely follow LVDD patients.

• The Korean Journal of Physiology and Pharmacology
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• v.27 no.4
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• pp.345-356
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• 2023

This study aimed to assess the effects of exogenous hydrogen sulfide (H₂S) on abdominal aorta coarctation (AAC) induced myocardial fibrosis (MF) and autophagy in rats. Forty-four Sprague-Dawley rats were randomly divided into control group, AAC group, AAC + H₂S group, and H₂S control group. After a model of rats with AAC was built surgically, AAC + H₂S group and H₂S group were injected intraperitoneally with H₂S (100 µmol/kg) daily. The rats in the control group and the AAC group were injected with the same amount of PBS. We observed that H₂S can improve left ventricular function and the deposition of myocardial collagen fibers, inhibit pyroptosis, down-regulate the expression of P-eif2α in myocardial tissue, and inhibit cell autophagy by activating the phosphatidylinositol 3-kinase (PI3K)/AKT1 signaling pathway (p < 0.05). In addition, angiotensin II (1 µM) H9c2 cardiomyocytes were injured in vitro experiments, and it was also observed that pyroptosis was inhibited after H₂S (400 µmol/kg) intervention, the expression of P-eif2α in cardiomyocytes was significantly down-regulated, and the PI3K/AKT1 signaling pathway was activated at the same time. Therefore, increasing the expression of P-eif2α reverses the activation of the PI3K/AKT1 signaling pathway by H₂S. In conclusion, these findings suggest that exogenous H₂S can ameliorate MF in rats with AAC by inhibiting pyroptosis, and the mechanism may be associated with inhibiting the phosphorylation of eif2α and activating the PI3K/AKT1 signaling pathway to inhibit excessive cell autophagy.

• Journal of Chest Surgery
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• v.38 no.9 s.254
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• pp.609-615
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• 2005

Background: Some controversy still exists concerning the operative indications of coronary fistulas. Nevertheless, a short-term and long-term outcomes are excellent with surgical interventions. In this study, we assessed our surgical results on this disease entity during the last 20 years. Anatomic diversity was described as well. Material and Method: From April 1986 to March 2005, 20 patients with coronary fistulas underwent surgical correction in Seoul National University Children's Hospital. Their medical records were reviewed retrospectively. Result: Twelve patients ($60\%$) were asymptomatic prior to surgery. All had electrocardiogram and echocardiogram and all but 3 had coro-nary angiogram preoperatively. Anatomically, none of them had two or more coronary fistulas. The sites of origin were left coronary system in 11 patients and right in 9. The draining sites were right ventricle in 11, right atrium in 3, left ventricle in 3, main pulmonary artery in 2, and superior vena cavae in 1. All of the involved, the coro-nary arteries were dilated or aneurismal. In 1 case, there was atherosclerotic change but no ischemic evidence in preoperative electrocardiogram. Operative techniques included external obliteration (13), internal obliteration (5), and both (2). External obliteration was done by ligation of the fistulous tract only in T patients, by fstula ligation plus plication in 3 and by plication or patch closure via fistulotomy in 3. There was no operative mortality. All of postoperative morbidities including transient sinus arrhythmia (2), complete atrioventricular block (1), decreased left ventricular function (2), ventricular tachycardia (1), pericarditis (1), and seizure (1) improved on discharge. The mean follow-up was 55.1$\pm$50.2 months (4.0 months${\~}$18.0 years) and there were no recurrences of fistula. There was 1 second operation for aortic root aneurysm, which developed after external patch closure of right coronary fistula. Conclusion: We demonstrated here that coronary fistulas can be cured with excellent clinical outcome and low operative risk under precise diagnosis. Understanding the anatomic diversity will help to construct surgical plans.

• The Korean Journal of Nuclear Medicine
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• v.23 no.2
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• pp.175-181
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• 1989

Visual assessment of regional wall motion abnormality (RWMA) by gated blood pol scan (GBPS) serves as an useful parameter in the diagnosis, functional evaluation, and follow up in various clinical settings, but are still subject to some inherent limitations. On important problem may be the interobserver as well as intraobsever variation that may well be present due to the subjective nature of the interpretations. This study was carried out to determine the reliability and reproducibility of visual assessments made in GBPSs, and to observe the degree to which the results would be influenced by observer variation. Fifty two patients with coronary heart disease had resting GBPS and contrast ventriculography within 4 days appall. Contrast ventriculography-showed normal wall motion in 6 patients and the remaining 46 had RWMA in one or more segments. The anterior and left anterolateral views of all 52 GBPSs were analyzed by three independent observers, who selected from 5 scales, their level of confidence that there was RWMA in that segment. Reciever operating characteristic (ROC) curves for each analysis was plotted and the area under the curve $(\theta)$ was used as a parameter representing each observer's performance in his interpretations. The findings of contrast ventriculographies were used as the standard for RWMA. The apical and inferoapical segments showed the best correlation with contrast ventriculography ($\theta=0.90-0.94$, 0.81-0.94, respectively), and the inferior wall showed the poorest correlation $(\theta=0.70-0.74)$. The interpretations of the inferior, septal, apical, and posteroinferior, segments showed no difference between the observers, but there was significantly better performance in assessment by observer A compared to that by B or C for the anterolateral segments ($\theta=0.87$, 0.78, 0.76, respectively. p<0.01 for A vs B, p<0.05 for A vs C), as well as when all segments were considered altogether ($\theta=0.88$, 0.83, 0.82, respectively. both p<0.05). This was also true for the infero-apical segment between A and C ($\theta=0.09$, 0.81, p<0.05). The intraobserver variation, however, did not appear significant, with only the inferior segment for observer B showing any significant difference when observer A and B repeated the analysis 10 days latter. There was no difference in assessing dyskinesia, with all observers showing a high performance ($\theta=0.98$, 0.87, 0.97, respectively). The visual assessment of left ventricular ejection fraction by all three observers correlated well with the calculated value from a semiautomated method (Spearman's r = 0.91, 0.83, 0.83. p<0.01, p<0.05, p < 0.05). The assessment of LV and RV size also correlated well between the three observers (Kendall's w = 0.80, 0.51, p<0.01 for both left and right ventricles). The above findings suggest that RWMA visually assessed by GBPS correlates well with that done by contrast ventriculography. And although the observer's experience or skill may influence the results in certain segments, visual analysis of GBPS may serve as a reliable and reproducible means for evaluating ventricular function.

• The Korean Journal of Nuclear Medicine
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• v.37 no.3
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• pp.147-152
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• 2003

Purpose: We evaluated radioisotope and sex-specific differences of normal limits for left ventricle volumes (LWs) and ejection fraction (EF) using myocardial perfusion gated SPECT (g-SPECT). Materials and Methods: Rest Tl-201/post-stress Tc-99m MIBI g-SPECT measurements with acquisitions of 8-frame were evaluated for 70 patients (mean age $55{\pm}14.56%$ female) who either had < 10% pretest likelihood of CAD (n = 12) or had normal coronary angiography (EF > 50%) (n = 58). LVEF, LWs were automatically determined by quantitative gated SPECT using QGS program. Results: Similar results were obtained for mean LVEF between Tc-99m MIBI ($62%{\pm}7%$ and Tl-201 ($63%{\pm}8%$) g-SPECT measurements. In Contrast, Tl-201 g-SPECT had significantly lower LWs values ($EDV;\;74{\pm}23mL,\;ESV;\;28{\pm}14mL$) than Tc-99m MIBI g-SPECT ($EDV;\;82{\pm}25mL,\;ESV;\;32{\pm}15mL$) (p<0.05). Women had significantly lower EDV ($Tc-99m\;MIBI;\;71{\pm}18mL,\;Tl-201;\;65{\pm}17mL$), and ESV values ($Tc-99m\;MIBI;\;27{\pm}10mL,\;Tl-201;\;23{\pm}8mL$) compared with EDV ($Tc-99m\;MIBI;\;96{\pm}27mL,\;Tl-201;\;85{\pm}24mL$), and ESV Values ($Tc-99m\;MIBI;\;40{\pm}17mL,\;Tl-201;\;36{\pm}16mL$) of men (p<0.05). Women had significantly higher LV EF Values ($65%{\pm}7%$) than men ($60%{\pm}8%$) by Tl-201 gated SPECT (p<0.05). Conclusion: These data suggest significant differeuces in normal limits for LWs and EF, according to genders and radiopharmaceutical. Therefore, the evaluation of cardiac function in patients should consider radioisotope and sex-matched normal values.