• Journal of Chest Surgery
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• v.29 no.11
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• pp.1218-1222
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• 1996

The reported incidence of postcardiotomy cardiogenic shock not responding to conventional therapy is still 0.1 to 0.8%. For this group of patients, more aggressive form of circulatory support must be employed. Centrifugal pumps are a ventricular assist device most commonly used on this purpose, due to low cost and easy availability. Currently, however, clinical experience of centrifugal pumps as a ventricular assist device is rarely reported in Korea. From January 1992 to January 1996, 2986 patients underwent cardiac operations on cardiopulmonary bypass at Seoul National University Hospital. Refractory postcardiotomy cardiac failure requring ventricular support with a Biomedicus centrifugal pump developed in ten of these patients. There were eight men and two women, ranged in age from nine years to 77 years with a mean of 50$\pm$20 years. The primary surgical procedures consisted of isolated coronary revascularization in four patients, combined coronary revascularization and aortic valve replacement in two, aortic dissection repair in two, pulmonary embolectomy in one, and heart transplantation in one. Of the ten patients, five had left ventricular assistance, one had right ventricular assistance, and four had biventricular assistance. Duration of ventricular assistance ranged from 24 to 175 hours, with a mean of 76$\pm$51 hours. Seven patients were weaned from ventricular assistance, and four of them discharged. The causes of death for nonsurvivors were progressive cardiac failure in two patients and multiorgan failure, intractable ventricular fibrillation, irreversible brain injury, and mechanical problem, respectively, in the other four. Survival was not predicted by time on cardiopulmonary bypass, aortic cross-clamp time, or duration of ventricular support. Major complications included bleeding(7), renal failure(6), infection(3) and neurologic complication(2). These results indicate that a centrifugal pump can provide reasonably satisfactory short-term circulatory support.

• Journal of Genetic Medicine
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• v.4 no.1
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• pp.38-44
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• 2007

Purpose : Menkes disease is an X-linked recessively inherited disorder caused by the mutation of the ATP7A gene encoding copper-transporting P-type AT Pase. The phenotypic features are progressive neurological degeneration, mental retardation, loose skin, and vascular complications. Early diagnosis and treatment are very important for the prognosis of Menkes disease. Here, we describe nov el mutations of the ATP7A gene and prenatal diagnosis by mutation analysis. Methods : Five unrelated Korean Menkes patients were included in this study. They presented with depigmented wool-like hair, progressive neurologic deterioration, and hypotonia in infancy. Serum copper and ceruloplasmin levels w ere decreased. Brain magnetic resonance imaging revealed tortuous intracranial vessels. Mutation analysis has been carried out using cDNA from cultured skin fibroblasts or genomic DNA from peripheral leukocytes. Prenatal diagnosis was performed in two cases using chorionic villi samples or amniocytes. Results : Four novel mutations have been identified from four different families; c.3511+1G>A (p.E1099_N1171delinsMfsX 18), c.4005+5 G>A (p.V1268_R1335del), c.1870_2172del (p.S624_Q724del), and c.3352 G>A (p.G1118S). T he remaining one was previously reported (c.1933 C>T (p.V 1268_R1335del)). On prenatal DNA analysis, one w as diagnosed as normal, while the other turned out to be a female heterozygote with p.S624_Q724del mutation of the ATP7A gene. Conclusion : We identified 4 novel mutations of the ATP7A gene. Prenatal diagnosis in families at risk is critical in order to choose preventiv e options including an early treatment with copper-histidine therapy or therapeutic termination. Most mutations of the ATP7A gene were frame-shift mutations and prenatal diagnosis has been successfully carried out.

• Journal of Chest Surgery
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• v.35 no.10
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• pp.712-723
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• 2002

Substantial alterations in cerebral blood flow(CBF) are known to occur during cardiopulmonary bypass(CPB). Many investigators have speculated that these changes may be responsible for both minor and major cerebral damages after CPB. More recently, these changes in CBF have been observed to be intimately related to the arterial carbon dioxide tension(Pa$CO_2$) maintained during CPB. The present study was prospectively designed to investigate the clinical effects of normocapnic and hypercapnic CPB on the cerebral oxygen metabolism in cardiac surgery Material and Method: Thirty-six adult patients scheduled for elective cardiac surgery were randomized to either normocapnic group (Pa$CO_2$35~40 mmHg, n＝18) or hypercapnic group(Pa$CO_2$, 45~55 mmHg, n＝18) with moderately hypothermic nonpulsatile CPB(nasopharyngeal temperature of 29~3$0^{\circ}C$). In each patient, middle cerebral artery blood flow velocity( $V_{MCA}$), cerebral arteriovenous oxygen content difference (C(a-v) $O_2$), cerebral oxygen extraction(COE), cerebral metabolic rate for oxygen(CMR $O_2$), cerebral oxygen transport( $T_{E}$ $O_2$), $T_{E}$ $O_2$/CMR $O_2$ ratio, cerebral desaturation(internal jugular bulb blood oxygen saturation $\leq$ 50%), and arterial and jugular bulb blood gas were evaluated throughout the operation. Postoperative neuropsychologic complications were assessed in all patients. All variables were compared between the two groups. Result: VMCA(169.13 $\pm$ 8.32 vs 153.11 $\pm$8.98%), TE $O_2$(1,911.17$\pm$250.14 vs 1,757.40$\pm$249.56), $T_{E}$ $O_2$,/CMR $O_2$ ratio(287.38$\pm$28.051 vs 246.77$\pm$25.84), $O_2$ tension in internal jugular bulb (41.66$\pm$9.19 vs 31.50$\pm$6.09 mmHg), and $O_2$saturation in internal jugular bulb(68.97$\pm$10.96 vs 58.12$\pm$12.11%) during CPB were significantly lower in normocapnic group(p＝0.03), whereas hypercapnic group had lower C(a-v) $O_2$(3.9$\pm$0.3 vs 4.9$\pm$0.3 mL/dL), COE(0.3$\pm$0.03 vs 0.4$\pm$0.03), CMR $O_2$(5.8 $\pm$0.5 vs 6.8$\pm$0.6), and arterial blood pH(7.36$\pm$0.09 vs 7.46$\pm$0.07, p＝0.04) during CPB. Hypercapnic group had lower incidence of cerebral desaturation than normocapnic group(3 vs 9 patients, p=0.03). Duration of the neuropsychologic complication(delirium) were shorter in hypercapnic group than in normocapnic group(36 vs 60 hrs, p＝0.009). Conclusion: These findings suggest that hypercapnic CPB may have salutary effects on the cerebral oxygen metabolism and postoperative neurologic outcomes in cardiac surgery.surgery.

• Radiation Oncology Journal
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• v.22 no.3
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• pp.165-176
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• 2004

Purpose: Firstly, to analyze facto in terms of radiation treatment that might potentially cause subfrontal relapse in two patients who had been treated by craniospinal irradiation (CSI) for medulloblastoma, Secondly, to explore an effective salvage treatment for these relapses. Materials and Methods: Two patients who had high-risk disease (T3bMl, T3bM3) were treated with combined chemoradiotherapy CT-simulation based radiation-treatment planning (RTP) was peformed. One patient who experienced relapse at 16 months after CSI was treated with salvage surgery followed by a 30.6 Gy IMRT (intensity modulated radiotherapy). The other patient whose tumor relapsed at 12 months after CSI was treated by surgery alone for the recurrence. To investigate factors that might potentially cause subfrontal relapse, we evaluated thoroughly the charts and treatment planning process including portal films, and tried to find out a method to give help for placing blocks appropriately between subfrotal-cribrifrom plate region and both eyes. To salvage subfrontal relapse in a patient, re-irradiation was planned after subtotal tumor removal. We have decided to treat this patient with IMRT because of the proximity of critical normal tissues and large burden of re-irradiation. With seven beam directions, the prescribed mean dose to PTV was 30.6 Gy (1.8 Gy fraction) and the doses to the optic nerves and eyes were limited to 25 Gy and 10 Gy, respectively. Results: Review of radiotherapy Portals clearly indicated that the subfrontal-cribriform plate region was excluded from the therapy beam by eye blocks in both cases, resulting in cold spot within the target volume, When the whole brain was rendered in 3-D after organ drawing in each slice, it was easier to judge appropriateness of the blocks in port film. IMRT planning showed excellent dose distributions (Mean doses to PTV, right and left optic nerves, right and left eyes: 31.1 Gy, 14.7 Gy, 13.9 Gy, 6.9 Gy, and 5.5 Gy, respectively. Maximum dose to PTV: 36 Gy). The patient who received IMRT is still alive with no evidence of recurrence and any neurologic complications for 1 year. Conclusion: To prevent recurrence of medulloblastoma in subfrontal-cribriform plate region, we need to pay close attention to the placement of eye blocks during the treatment. Once subfrontal recurrence has happened, IMRT may be a good choice for re-irradiation as a salvage treatment to maximize the differences of dose distributions between the normal tissues and target volume.

• Journal of Chest Surgery
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• v.35 no.6
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• pp.420-429
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• 2002

Moderate hypothermic cardiopulmonary bypass (CPB) has commonly been used in cardiac surgery. Several cardiac centers recently practice normothermic CPB in cardiac surgery, However, the clinical effect and safety of normothermic CPB on cerebral metabolism are not established and not fully understood. This study was prospectively designed to evaluate the clinical influence of normothermic CPB on brain metabolism and to compare it with that of moderate hypothermic CPB. Material and Method: Thirty-six adult patients scheduled for elective cardiac surgery were randomized to receive normothermic (nasopharyngeal temperature ＞34.5 $^{\circ}C$, n=18) or hypothermic (nasopharyngeal temperature 29~3$0^{\circ}C$, n=18) CPB with nonpulsatile pump. Middle cerebral artery blood flow velocity (VMCA), cerebral arteriovenous oxygen content difference (CAVO$_{2}$), cerebral oxygen extraction (COE), modified cerebral metabolic rate for oxygen (MCMRO$_{2}$), cerebral oxygen transport (TEO$_{2}$), cerebral venous desaturation (oxygen saturation in internal jugular bulb blood$\leq$50 %), and arterial and internal jugular bulb blood gas analysis were measured during six phases of the operation： Pre-CPB (control), CPB-10 min, Rewarm-1 (nasopharyngeal temperature 34 $^{\circ}C$ in the hypothermic group), Rewarm-2 (nasopharyngeal temperature 37 $^{\circ}C$ in the both groups), CPB-off and Post-CPB (skin closure after CPB-off). Postoperaitve neuropsychologic complications were observed in all patients. All variables were compared between the two groups. Result： VMCA at Rewarm-2 was higher in the hypothermic group (153.11$\pm$8.98%) than in the normothermic group (131.18$\pm$6.94%) (p＜0.05). CAVO$_{2}$ (3.47$\pm$0.21 vs 4.28$\pm$0.29 mL/dL, p＜0.05), COE (0.30$\pm$0.02 vs 0.39$\pm$0.02, p＜0.05) and MCMRO$_{2}$ (4.71 $\pm$0.42 vs 5.36$\pm$0.45, p<0.05) at CPB-10 min were lower in the hypothermic group than in the normothermic group. The hypothermic group had higher TEO$_{2}$ than the normothermic group at CPB-10 (1,527.60$\pm$25.84 vs 1,368.74$\pm$20.03, p＜0.05), Rewarm-2 (1,757.50$\pm$32.30 vs 1,478.60$\pm$27.41, p＜0.05) and Post-CPB (1,734.37$\pm$41.45 vs 1,597.68$\pm$27.50, p＜0.05). Internal jugular bulb oxygen tension (40.96$\pm$1.16 vs 34.79$\pm$2.18 mmHg, p＜0.05), saturation (72.63$\pm$2.68 vs 64.76$\pm$2.49 %, p＜0.05) and content (8.08$\pm$0.34 vs 6.78$\pm$0.43 mL/dL, p<0.05) at CPB-10 were higher in the hypothermic group than in the normothermic group. The hypothermic group had less incidence of postoperative neurologic complication (delirium) than the normothermic group (2 vs 4 patients, p＜0.05). Lasting periods of postoperative delirium were shorter in the hypothermic group than in the normothermic group (60 vs 160 hrs, p<0.01). Conclusion: These results indicate that normothermic CPB should not be routinely applied in all cardiac surgery, especially advanced age or the clinical situations that require prolonged operative time. Moderate hypothermic CPB may have beneficial influences relatively on brain metabolism and postoperative neuropsychologic outcomes when compared with normothermic CPB.