Purpose : Diamond-Blackfan anemia (DBA) is a rare heterogeneous genetic disorder of infancy and early childhood. It is characterized by red cell aplasia, congenital anomalies, and a predisposition to cancer. Corticosteroids and red cell transfusions are the mainstays of therapy. We describe our experience of 6 cases of DBA that were encountered over a period of 16 years. Methods : Medical records of 6 patients diagnosed to have DBA and admitted to the Chonnam National University Hospital between 1992 and 2008 were retrospectively reviewed. Results : Three patients were males. The age at diagnosis ranged from 3 to 18 months (median, 5.5 months). Heart defects were observed in 4, polydactyly in 2, and strabismus in 1 patient. The median number of transfusions was 3 (range, 2-8). All patients responded to initial treatment with steroids and had a hemoglobin level ${\geq}9g/dL$ with a median of 12.5 days (range, 7-22 days). Three patients are currently not receiving steroid therapy. A minimum dose of oral prednisolone (<5 mg per day) was required to maintain hemoglobin ${\geq}9mg/dL$ in 3 cases. Red cell transfusion was infrequently required in 1 patient. In the median follow-up of 14 years, there was no development of malignancy. No significant side effects of steroids were found, except for short stature in 2. Conclusion : The majority of DBA patients achieved complete response and under maintenance therapy with low dose of steroids. Close observation is needed to monitor steroid side effects, cardiac function, and development of malignancy. A nation-wide survey is necessary to further characterize this rare disease in Korean children.
Journal of Physiology & Pathology in Korean Medicine
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v.24
no.4
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pp.661-667
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2010
Astragali Radix (AR) has been used in traditional medicinal herb, which is known to tonify the qi and blood. It has been asserted to be a tonic that can improve the function of the lungs, adrenal glands and the gastrointestinal tract, increase metabolism, promote healing and reduce fatigue. Also, Plantago asiatica (PA) has the anti-inflammatory, antiseptic, antitussive, cardiac, diuretic, expectorant, haemostatic effects, though it is considered to be less powerful than the seed. Recent research is showing that it is promising in lowering cholesterol and controlling diabetes. Obesity is characterized at the cellular level by an increase in the number and size of adipocytes differentiated from fibroblastic preadipocytes in adipose tissue. In the present study, we investigated the effects of 3 to 7 extracts of mixed medicinal herbs (water and ethanol extract of Astragali Radix; ARW and ARE, water and ethanol extract of Plantago asitica; PAW and PAE, and those mixed extracts; ARW+PAE, ARW+PAW and ARE+PAE) on adipogenesis and apoptosis in 3T3-L1 adipocytes by MTT assay, Oil-Red-O staining and TUNEL assay. And then, we determined total contents of phenolic compounds and flavonoid compounds in mixed herbal extracts. These result showed that herbal extracts (ARW, PAE and ARW+PAE) affected on adipogenesis and apoptosis in 3T3-L1 adipocytes among 3 to 7 extracts tested. Especially herbal mixed extracts (ARW+PAE) had the highest inhibition on differentiation of preadipocytes and lipoid accumulation of maturing adipocytes. And it did not affect cell viability of mature adipocytes, but herbal mixed extracts (ARW+PAE) increased apoptosis in mature adipocytes, as proved by highest concentration $200\;{\mu}g/m{\ell}$ using TUNEL assay. In addition, contents of total phenoilc compounds and flavonoids showed the highest level in ARE+PAE and PAE than ARW. These results suggest that this main target for adipocyte functions could be effective in improving the metabolic syndrome including obesity.
The successful cardiac transplantation depends partly on the donor heart preservation by a solution that will ensure recovery of myocardial function. The purpose of this study was to perform the evaluation of various preservation solutions and to accumulate the data on the requisites for ideal preservation solution. The experimental setup was the constant pressure Langendorffs perfusion system. Isolated rabbit hearts were perfused for 20minutes with unarm Krebs-Henseleit solution, stored for 4 hours in cold preservation solution after cardioplegia, and then were reperfused for 20minutes. The 4 experimental groups were prepared Hartmann's solution group (group 1, control), modified Euro-collins solution group(group II. MEC), modified University of Wisconsin group (group n, MUW), and CK solution(made by the author) group (group W, CK). The parameters for assessing the preservation ability were levels of enzymes in freezed myocardial tissues (lactate, creatine kinase-MB and adenosine deaminase), coronary flow. left ventricular developing pressure and dpldt. In conclusion, the ability of preservation for isolated rabbit heart was excellent in CK solution and modified University of Wisconsin solution, and poor in modified Euro-collins solution, compared with Hartmann solution. CK solution has low potassium concentrations(34.2mEq/L) and includes various substrates to be salutary on myocardial preservation. This fact may indicates the necessity of further refinements in selection or composition of electrolytes and substrates.
Transactions of the Korean Society of Mechanical Engineers B
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v.38
no.9
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pp.739-745
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2014
Although medication is given to heart disease patients, conventional medication alone is not sufficient to treat heart disease. However, it has been reported that left ventricular assist device (LVAD) transplantation is an effective bridge to heart transplantation by assisting cardiac function. This study used long-term animal testing and emergency situations with a bovine model (Holstein) and canine model (Labrador-retriever) to evaluate the biocompatibility of LibraHeart-I (LH-1), which is a bent-tube type of LVAD that was developed in a previous study. In the long-term animal testing with the bovine model, the subjects survived for 49 days with no irregularities observed in their complete blood cell counts or the vital sign tests that were carried out during the test period. In short-term animal testing with the canine model, it was observed that blood did not remain inside the LH-I even without power support from an external drive source. In this study, the biocompatibility of the LH-I that was developed in a previous study was verified by the ejection performance during long-term animal testing and emergency situations.
Background: Cardiopulmonary bypass during open heart surgery causes systemic inflammatory respose. IL-10 is an anti-inflammatory cytokine that inhibits inflammatory process and protects organ function by down regulation of pro-inflammatory cytokine release and maintenance of blood level balance with pro-inflammatory cytokines. Mateial and Method: Plasma IL-10 levels were measured and analyzed in 22 patients who underwent open heart surgery (11 cases of coronary artery bypass graft, 11 cases of valve replacement) under cardiopulmonary bypass since 1988 January to July at Department of Thoracic and Czardiovascular surgery, Yeungnam University Hospital. 1g of methylprednisolone was administrated to thirteen patients randomly. Blood samp.es were taken and collected at the time of induction of anesthesia, 10 min before cardiopulmonary bypass, 10 min after starting of CPB, 10 min aftr aortic cross clamping, 10 min after ACC release, and 10 min, 2 hours, and `5 hours after CPB respectively. The plasma levels of IL-10 were determined by enzyme-linked immunosorbent assays(ELISA). Wilcoxon-Raule Sum test was used for statistical analysis. Result: In all 22 patients, cardiopulmonary bypass time was used for statistical analysis. Result: In all 22 patients, cardiopulmonary bypass time was 171$\pm$41.4 min and aortic cross clamp time was 118$\pm$36.5 min. Peak IL-10 level was achieved at 10 min after ACC(361.0$\pm$52.81pg/ml) and was decreased sharply at 2 hours after CPB. Peak IL-10 level was correlated positively with aortic cross clamp time(p=0.011); however, it did not correlated with bypass time(p=0.181). In valve replacement group, mean IL-10 level at peak point was 567.89$\pm$107.69 pg/ml and was significantly higher than that of coronary artery bypass group(205.67$\pm$192.70 pg/ml)(p<0.001). ACC time in valve replacement group was significantly longer than that of coronary artery bypass group(p<0.01), however, bypass time was not(p=0.212). Thirteen patients with steroid pretreatment before starting of CPB showed relatively higher plasma IL-10 level than in control group, however, no statistical significance was noted(p=0.19). Conclusion: plasma level of IL-10 was increased in association with cardiopulmonary bypass and revealed peak at 10 min after ACC release. IL-10 level was correlated positively with ACC time. Therefore, systemic inflammatory respeonse in association with cardiopulmonary bypass could be decreased by reducing ACC time during cardiac surgery.
The purpose of this study was to investigate the changes of long term ECG response in a company with middle-aged male employees. Subjects were 60 men who were 40~55 years old. We enrolled 30 exercise group subjects into a 3-year exercise program. In measurement index, body composition was measured by % body fat and BMI. Exercise stress test analyses were measured using ST/HR slope and QRS vector. Statistical analysis was performed using analysis of repeated ANOVA. Results of this study were as follows: In ST/HR slope, the control group showed symptoms of ischemia after nine minutes of exercise. In the rest frontal axis of the QRS vector, the control group had a tendency towards right axis deviation. In the rest horizontal amplitude of the QRS vector, the control group had a tendency to show a significant decrease, but it was increased significantly in the exercise group. These findings suggest that inactive company workers was showed a decrease of exercise capacity, early diagnosis exercise-induced ST depression, and prolonged deviation of QRS vector, but that cardiac function could be elevated in active middle aged men through regular exercise program participation.
Myocardial tagging technique such as spatial modulation of magnetization (SPAMM) allows the study of myocardial motion with high accuracy. However, the accuracy of the estimation of tag intersection can be affected by tagline spacing. The aim of this study was to investigate the relationship between tagline spacing of SPAMM image and tagging contrast-to-noise ratio (CNR) in in-vivo study. Two healthy volunteers were undergone electrocardiographically triggered MR imaging with SPAMM-based tagging pulse sequence at a 1.5T MR scanner. Horizontally modulated stripe patterns were imposed with a range from 3.6 to 9.6 mm of tagline spacing. Images of the left ventricle(LV) wall were acquired at the mid-ventricle level during cardiac cycle with FE-EPI (TR/TE = 5.8/2.2 msec, FA= 10$^{\circ}$. Tagging CNR for each image was calculated with a software which developed in our group. During contraction, tagging CNR was more rapidly decreased in case of narrow tagline spacing than in case of wide tagline spacing. In the same heart phase, CNR was increased corresponding with tagline spacing. Especially, at the fully contracted heart phase, CNR was more rapidly increased than the other heart phases as a function of tagline spacing. The results indicated that the optimization of tagline spacing provides better tagging CNR in order to analyze the myocardial motion more accurately.
Lee, Sung Ryul;Noh, Su Jin;Pronto, Julius Ryan;Jeong, Yu Jeong;Kim, Hyoung Kyu;Song, In Sung;Xu, Zhelong;Kwon, Hyog Young;Kang, Se Chan;Sohn, Eun-Hwa;Ko, Kyung Soo;Rhee, Byoung Doo;Kim, Nari;Han, Jin
The Korean Journal of Physiology and Pharmacology
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v.19
no.5
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pp.389-399
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2015
Zinc has been considered as a vital constituent of proteins, including enzymes. Mobile reactive zinc ($Zn^{2+}$) is the key form of zinc involved in signal transductions, which are mainly driven by its binding to proteins or the release of zinc from proteins, possibly via a redox switch. There has been growing evidence of zinc's critical role in cell signaling, due to its flexible coordination geometry and rapid shifts in protein conformation to perform biological reactions. The importance and complexity of $Zn^{2+}$ activity has been presumed to parallel the degree of calcium's participation in cellular processes. Whole body and cellular $Zn^{2+}$ levels are largely regulated by metallothioneins (MTs), $Zn^{2+}$ importers (ZIPs), and $Zn^{2+}$ transporters (ZnTs). Numerous proteins involved in signaling pathways, mitochondrial metabolism, and ion channels that play a pivotal role in controlling cardiac contractility are common targets of $Zn^{2+}$. However, these regulatory actions of $Zn^{2+}$ are not limited to the function of the heart, but also extend to numerous other organ systems, such as the central nervous system, immune system, cardiovascular tissue, and secretory glands, such as the pancreas, prostate, and mammary glands. In this review, the regulation of cellular $Zn^{2+}$ levels, $Zn^{2+}$-mediated signal transduction, impacts of $Zn^{2+}$ on ion channels and mitochondrial metabolism, and finally, the implications of $Zn^{2+}$ in health and disease development were outlined to help widen the current understanding of the versatile and complex roles of $Zn^{2+}$.
Kim, Nari;Lee, Sang-Kyeong;Chung, Joon-Yong;Seog, Dae-Hyun;Kim, Euiyong;Jin Han
Proceedings of the Korean Biophysical Society Conference
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2003.06a
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pp.23-23
/
2003
It has been suggested that the impairment of smooth muscle cell (SMC) function by alterations in the $Ca^{2+}$-activated $K^{+}$ ( $K_{Ca}$ ) channels accounts for the reduction in coronary reserve during left ventricular hypertrophy (LVH). However, this hypothesis has not been fully investigated. The main goal of this study was to assess whether the properties of $K_{Ca}$ channels in coronary SMCs were altered during LVH. New Zealand white rabbits (0.8-1.0 kg) and Sprague-Dawley rats (300-400 g) were randomly selected to receive either an injection of isoproterenol (300 $\mu\textrm{g}$/kg body weight) or an equal volume of 0.9% saline (1 mL/kg body weight). The animals developed LVH 10 days after injection. In patch-clamp experiments, the unitary current amplitude and open probability for the $K_{Ca}$ channels were significantly reduced in LVH patches compared with control patches. The concentration-response curve of the $K_{Ca}$ channel to [C $a^{2+}$]$_{i}$ was shifted to the right. Inhibition of the $K_{Ca}$ channels with TEA was more pronounced in LVH cells than in the control cells. The whole-cell currents of $K_{Ca}$ channels were reduced during LVH. Western blot analysis indicated no differences in $K_{Ca}$ channel expression between the control and LVH coronary SM membranes. In contraction experiments, the effect of a high $K^{+}$concentration on the resting tension of the LVH coronary artery was greater than on that of the control. The effect of TEA on the resting tension of the LVH coronary artery was reduced as compared with the effect on the control. Our findings imply a novel mechanism for reduced coronary reserve during LVH.ing LVH.
From May 1, 1993 to May 31 1995, the authers studied retrospectively 211 patients who underwent cardiovascular operation with cardiopulmonary bypass(CPB). Because we were interested in new development of ARF(prevalence, mortality rate, and main risk factors), we performed a multivariate statistical analysis about data of patients with preoperative serum creatinine values of less than 1.5 mg/dL. Normal renal function before operation(serum creatinine level less than 1.5 mg/dL) was registered in 198(74%) patients. Of these, 27(14%) patients showed postoperative renal complication, including 20(10%) patients classified as renal dysfunction(serum creatinine level between 1.5 and 2.5 mg/dL) and 7(4%) patients as acute renal failure(serum creatinine level higher than 2.5 mg/dL). The mortality rate was 5.8% in normal patients, 5% in patients with renal dysfunction, and 43% when acute renal failure developed(p=0.036). Indeed, the renal impairment proved to be an independent predictor of mortality(odd ratio 2.52∼11.25), along with cardiovascular(odd ratio 4.20) and respiratory(odd ratio 2.18) complications. Multivariate analysis identified the following variables as independent risk factors for postoperative renal impairment : advanced age(odd ratio 1), need for emergency operation(odd ratio 3.78), low-output syndrome(odd ratio 3.66), respiratory complication(odd ratio 1.30), need for deep hypothermic circulatory arrest(odd ratio 1.4). The 13 patients(7%) with preoperative renal failure showed a significantly higher morbidity and mortality rate than those without renal complications before operation. We concluded that the likelihood of severe renal complications is resonably low in the patients undergoing cardiac operation without preexisting renal dysfunction, but associated mortality remains high. A prominant role of hemodynamic factor in the development of postoperative acute renal failure must be recognized during preoperative, intraoperative, and postoperative periods.
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