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Publications in this journal

• Carbon Dioxide Embolism during Laparoscopic Surgery.

  Authors: Eun Young Park, Ja-Young Kwon, Ki Jun Kim

  Yonsei medical journal. 53(3):459-66.

  Clinically significant carbon dioxide embolism is a rare but potentially fatal complication of anesthesia administered during laparoscopic surgery. Its most common cause is inadvertent injection ofClinically significant carbon dioxide embolism is a rare but potentially fatal complication of anesthesia administered during laparoscopic surgery. Its most common cause is inadvertent injection of carbon dioxide into a large vein, artery or solid organ. This error usually occurs during or shortly after insufflation of carbon dioxide into the body cavity, but may result from direct intravascular insufflation of carbon dioxide during surgery. Clinical presentation of carbon dioxide embolism ranges from asymptomatic to neurologic injury, cardiovascular collapse or even death, which is dependent on the rate and volume of carbon dioxide entrapment and the patient's condition. We reviewed extensive literature regarding carbon dioxide embolism in detail and set out to describe the complication from background to treatment. We hope that the present work will improve our understanding of carbon dioxide embolism during laparoscopic surgery.

• The localizing and lateralizing value of auras in lesional partial epilepsy patients.

  Authors: Byoung Seok Ye, Yang-Je Cho, Sang Hyun Jang, Moon Kyu Lee, Byung In Lee, Kyoung Heo

  Yonsei medical journal. 53(3):477-85.

  Purpose: We investigated the localizing and lateralizing values of auras in patients with lesional partial epilepsy on an outpatient basis. Materials and Methods: A total of 276 subjects werePurpose: We investigated the localizing and lateralizing values of auras in patients with lesional partial epilepsy on an outpatient basis. Materials and Methods: A total of 276 subjects were retrospectively selected for this study if they had a unilateral single lobar lesion based on magnetic resonance image (MRI) results, and their scalp electroencephalography (EEG) findings were not discordant with the MRI-defined lobar localization and lateralization. According to the lesion locations, subjects were considered as having mesial temporal (MTLE), lateral temporal (LTLE), frontal (FLE), parietal (PLE), or occipital (OLE) lobe epilepsies. Auras were classified into 13 categories. Results: A hundred and seventy-six subjects (63.8%) had experienced at least one aura. FLE subjects had the fewest number of auras. Epigastric and psychic auras were frequent among MTLE subjects, while visual auras were common in those with PLE and OLE. Somatosensory auras and whole body sensations were more frequent in the subjects with PLE than those without. Autonomic auras were more common in MTLE subjects than in LTLE subjects. Dysphasic auras were more frequently found in left-sided epilepsies. Five pairs of aura categories showed concurrent tendencies, which were the epigastric and autonomic auras, autonomic and emotional auras, visual and vestibular auras, auditory and vestibular auras, and whole-body sensation and auditory auras. Autonomic and emotional auras had a concurrent tendency in left-sided epilepsies, but not in right-sided epilepsies. Conclusion: Our results support the previously known localizing value of auras, and suggest that dysphasic auras and the association of emotional and autonomic auras may have a lateralizing value.

• Genetic and epileptic features in rett syndrome.

  Authors: Hyo Jeong Kim, Shin Hye Kim, Heung Dong Kim, Joon Soo Lee, Young-Mock Lee, Kyo Yeon Koo, Jin Sung Lee, Hoon-Chul Kang

  Yonsei medical journal. 53(3):495-500.

  Purpose: Rett syndrome is a severe neurodevelopmental disorder in females. Most have mutations in the methyl-CpG-binding protein 2 (MECP2) gene (80-90%). Epilepsy is a significant commonlyPurpose: Rett syndrome is a severe neurodevelopmental disorder in females. Most have mutations in the methyl-CpG-binding protein 2 (MECP2) gene (80-90%). Epilepsy is a significant commonly accompanied feature in Rett syndrome. Our study was aimed at comprehensive analysis of genetic and clinical features in Rett syndrome patients, especially in regards to epileptic features. Materials and Methods: We retrospectively reviewed 20 patients who were diagnosed with MECP2 mutations at Severance Children's Hospital between January 1995 and July 2010. All patients met clinical criteria for Rett syndrome. Evaluations included clinical features, epilepsy classification, electroencephalography analysis, and treatment of seizures. Results: Ages ranged from 3.6 to 14.3 years (7.7±2.6). Fourteen different types of MECP2 mutations were found, including a novel in-frame mutation (1153-1188 del36). Fourteen of these patients (70.0%) had epilepsy, and the average age of seizure onset was 3.0±1.8 years. Epilepsy was diverse, including partial seizure in four patients (28.5%), secondarily generalized seizure in six (42.8%), generalized tonic seizure in two (14.3%), Lennox-Gastaut syndrome in one (7.1%), and myoclonic status in non-progressive encephalopathy in one (7.1%). Motor functions were delayed so that only 10 patients (50.0%) were able to walk independently: five (35.8%) in the epilepsy group and five (83.3%) in the non-epilepsy group. Average developmental scale was 33.5±32.8 in the epilepsy group and 44.4±21.2 in the non-epilepsy group. A clear genotype-phenotype correlation was not found. Conclusion: There is a tendency for more serious motor impairment and cognitive deterioration in Rett syndrome patients with epilepsy.

• Correlations between Coronary Plaque Tissue Composition Assessed by Virtual Histology and Blood Levels of Biomarkers for Coronary Artery Disease.

  Authors: Young-Guk Ko, Van Cuong Le, Bo Hyun Kim, Dong-Ho Shin, Jung-Sun Kim, Byeong-Keuk Kim, Donghoon Choi, Yangsoo Jang, Myeong-Ki Hong

  Yonsei medical journal. 53(3):508-16.

  Purpose: We investigated correlations of coronary plaque composition determined by virtual histology (VH) intravascular ultrasound (IVUS) and blood levels of biomarkers that represent thePurpose: We investigated correlations of coronary plaque composition determined by virtual histology (VH) intravascular ultrasound (IVUS) and blood levels of biomarkers that represent the vulnerability of coronary plaques. Materials and Methods: Pre- and postprocedural blood levels of high sensitivity C-reactive protein, soluble CD40 ligand (sCD40L), matrix metalloproteinase-9, and neopterin were measured in 70 patients with stable angina (SA) or unstable angina (UA) who were undergoing percutaneous coronary intervention (PCI) for single lesions. We evaluated the data for correlations between these biomarkers and necrotic core contents in PCI target lesions analyzed by VH. Results: Clinical characteristics, IVUS, VH, and biomarker blood levels were not different between the SA and the UA group except for more frequent previous statin use (52.3% vs. 23.1%, p=0.017) and lower remodeling index in the SA group (0.98±0.09 vs. 1.10±0.070, p<0.001). Among the biomarkers evaluated, only pre-PCI neopterin level showed a weakly significant correlation with the absolute volume of the necrotic core (r=0.320, p=0.008). Pre- and post-PCI blood levels of sCD40L (r=0.220, p=0.072; r=0.231, p=0.062) and post-PCI blood level of neopterin (r=0.238, p=0.051) showed trends toward weakly positive correlations with the absolute volume of necrotic core. Conclusion: We found a weakly positive correlation between the pre-PCI neopterin level and necrotic core volume in the PCI-target lesion. The clinical implications of our findings need to be investigated in further studies.

• Comparison of Optical Coherence Tomographic Assessment between First- and Second-Generation Drug-Eluting Stents.

  Authors: Byeong-Keuk Kim, Jung-Sun Kim, Junbeom Park, Young-Guk Ko, Donghoon Choi, Yangsoo Jang, Myeong-Ki Hong

  Yonsei medical journal. 53(3):524-9.

  Purpose: There is a lack of sufficient data in comparison of optical coherence tomographic (OCT) findings between first- and second-generation drug-eluting stents (DES). Compared to first-generationPurpose: There is a lack of sufficient data in comparison of optical coherence tomographic (OCT) findings between first- and second-generation drug-eluting stents (DES). Compared to first-generation (i.e., sirolimus- or paclitaxel-eluting stents), second-generation DESs (i.e., everolimus- or biolinx-based zotarolimus-eluting stents) might have more favorable neointimal coverage. Materials and Methods: Follow-up OCT findings of 103 patients (119 lesions) treated with second- generation DESs were compared with those of 139 patients (149 lesions) treated with first-generation DESs. The percentage of uncovered or malapposed struts, calculated as the ratio of uncovered or malapposed struts to total struts in all OCT cross-sections, respectively, was compared between the two groups. Results: Both DES groups showed similar suppression of neointimal hyperplasia (NIH) on OCT (mean NIH cross-sectional area; second- vs. first-generation=1.1±0.5 versus 1.2±1.0 mm², respectively, p=0.547). However, the percentage of uncovered struts of second-generation DESs was significantly smaller than that of first-generation DESs (3.8±4.8% vs.7.5±11.1%, respectively, p<0.001). The percentage of malapposed struts was also significantly smaller in second-generation DESs than in first-generation DESs (0.4±1.6% vs.1.4±3.7%, respectively, p=0.005). In addition, intra- stent thrombi were less frequently detected in second-generations DESs than in first-generation DESs (8% vs. 20%, respectively, p=0.004). Conclusion: This follow- up OCT study showed that second-generation DESs characteristically had greater neointimal coverage than first-generation DESs.

• Clinical Outcomes of Ductal Carcinoma In Situ of the Breast Treated with Partial Mastectomy without Adjuvant Radiotherapy.

  Authors: Seung Hyun Hwang, Joon Jeong, Sung Gwe Ahn, Hak Min Lee, Hy-De Lee

  Yonsei medical journal. 53(3):537-42.

  Purpose: Some recent trials suggest that postoperative adjuvant radiotherapy (RT) may be safely omitted after breast-conserving surgery (BCS) for some patients with ductal carcinoma in situ (DCIS).Purpose: Some recent trials suggest that postoperative adjuvant radiotherapy (RT) may be safely omitted after breast-conserving surgery (BCS) for some patients with ductal carcinoma in situ (DCIS). In this study, we reviewed clinical outcomes of patients with DCIS treated with partial mastectomy (PM) without adjuvant RT. Materials and Methods: Medical records of 28 patients (29 breasts) with DCIS who were treated with PM, but without RT, between April 1991 and December 2010 were retrospectively analyzed. Based on established criteria (2.0 cm or less in size and no comedonecrosis), 18 patients were treated without RT after PM. Seven patients (8 breasts) who did not receive RT due to refusal were also included in this study. Three other patients were excluded because data concerning comedonecrosis were not available. Results: For the 25 patients included in this study, the mean age of the 18 patients who met the criteria was 47.9±6.2 years, and 47.6±12.7 years for the 7 patients who did not. The mean sizes of the primary tumors were 0.6±0.4 cm and 0.9±0.3 cm, respectively, in these two groups. Among these 25 patients (26 breasts) treated without RT, we observed no ipsilateral breast tumor recurrence or mortality within a mean follow-up of 84 months. Conclusion: Based on this small number of cases, patients with DCIS, who were selected for tumor size less than 2 cm and absence of comedonecrosis, may be treated successfully with BCS; adjuvant RT may be omitted.

• Comparison of Pelvic Phased-Array versus Endorectal Coil Magnetic Resonance Imaging at 3 Tesla for Local Staging of Prostate Cancer.

  Authors: Bum Soo Kim, Tae-Hwan Kim, Tae Gyun Kwon, Eun Sang Yoo

  Yonsei medical journal. 53(3):550-6.

  Purpose: Several studies have demonstrated the superiority of endorectal coil magnetic resonance imaging (MRI) over pelvic phased-array coil MRI at 1.5 Tesla for local staging of prostate cancer.Purpose: Several studies have demonstrated the superiority of endorectal coil magnetic resonance imaging (MRI) over pelvic phased-array coil MRI at 1.5 Tesla for local staging of prostate cancer. However, few have studied which evaluation is more accurate at 3 Tesla MRI. In this study, we compared the accuracy of local staging of prostate cancer using pelvic phased-array coil or endorectal coil MRI at 3 Tesla. Materials and Methods: Between January 2005 and May 2010, 151 patients underwent radical prostatectomy. All patients were evaluated with either pelvic phased-array coil or endorectal coil prostate MRI prior to surgery (63 endorectal coils and 88 pelvic phased-array coils). Tumor stage based on MRI was compared with pathologic stage. We calculated the specificity, sensitivity and accuracy of each group in the evaluation of extracapsular extension and seminal vesicle invasion. Results: Both endorectal coil and pelvic phased-array coil MRI achieved high specificity, low sensitivity and moderate accuracy for the detection of extracapsular extension and seminal vesicle invasion. There were statistically no differences in specificity, sensitivity and accuracy between the two groups. Conclusion: Overall staging accuracy, sensitivity and specificity were not significantly different between endorectal coil and pelvic phased-array coil MRI.

• Prognostic factors for endotracheal silicone stenting in the management of inoperable post-intubation tracheal stenosis.

  Authors: So Yeon Lim, Hojoong Kim, Kyeongman Jeon, Sang-Won Um, Won-Jung Koh, Gee Young Suh, Man Pyo Chung, O Jung Kwon

  Yonsei medical journal. 53(3):565-70.

  Purpose: Stenting has been developed to deal with airway stenosis and is applicable in patients with post-intubation tracheal stenosis (PITS) in whom surgery would not be indicated. The purpose ofPurpose: Stenting has been developed to deal with airway stenosis and is applicable in patients with post-intubation tracheal stenosis (PITS) in whom surgery would not be indicated. The purpose of this study was to investigate the prognostic factors in inoperable patients in whom a silicone stent was inserted due to PITS. Materials and Methods: We retrospectively evaluated 55 PITS patients undergoing silicone stenting between January 2001 and December 2009. Results: Silicone stent was inserted to narrowed trachea after the combination of pre-dilatation including laser cauterization, mechanical bougienation and ballooning. Following airway stabilization, the stent could be removed successfully in 40% (22/55) of the patients after median 12 months of stenting. However, in 60% (33/55) of patients, the stent could not be removed successfully and surgical management was needed after initial stabilization. Multivariate analysis revealed that the stent could be successfully removed more frequently in those who do not have cardiovascular disease [odds ratio (OR)=12.195; p=0.036] and the intervention was performed within 6 months after intubation (OR=13.029; p=0.031). Conclusion: Among those patients undergoing silicone stenting due to PITS, the stent could be successfully removed when patients do not have cardiovascular disease and stented within 6 months after intubation.

• Estimating average glucose levels from glycated albumin in patients with end-stage renal disease.

  Authors: Jwa-Kyung Kim, Jung Tak Park, Hyung Jung Oh, Dong Eun Yoo, Seung Jun Kim, Seung Hyeok Han, Shin-Wook Kang, Kyu Hun Choi, Tae-Hyun Yoo

  Yonsei medical journal. 53(3):578-86.

  Purpose: In patients with diabetic end stage renal disease (ESRD), glycated albumin (GA) reflects recent glycemic control more accurately than glycated hemoglobin (HbA1c). We evaluated thePurpose: In patients with diabetic end stage renal disease (ESRD), glycated albumin (GA) reflects recent glycemic control more accurately than glycated hemoglobin (HbA1c). We evaluated the relationship between GA and average blood glucose (AG) level and developed an estimating equation for translating GA values into easier-to-understand AG levels. Materials and Methods: A total of 185 ESRD patients, including 154 diabetic and 31 non-diabetic participants, were enrolled (108 hemodialysis, 77 peritoneal dialysis). Patients were asked to perform four-point daily self-monitoring of capillary blood glucose (SMBG) at least three consecutive days each week for four weeks. Serum levels of GA, HbA1c and other biochemical parameters were checked at baseline, as well as at 4 and 8 weeks. Results: Approximately 74.3±7.0 SMBG readings were obtained from each participant and mean AG was 169.1±48.2 mg/dL. The correlation coefficient between serum GA and AG levels (r=0.70, p<0.001) was higher than that of HbA1c and AG (r=0.54, p<0.001). Linear regression analysis yielded the following equation: estimated AG (eAG) (mg/dL)=4.71×GA%+73.35, and with this formula, serum GA levels could be easily translated to eAG levels. Multivariate analysis revealed significant contributions of postprandial hyperglycemia (β=0.25, p=0.03) and serum albumin (β=0.17, p=0.04) in determining serum GA level, independent to other clinical parameters. Conclusion: Compared to HbA1c, serum GA levels were better correlated with AG levels. Using the estimating equation, an average blood glucose level of 155-160 mg/dL could be matched to a GA value of 18-19% in patients with ESRD.

• Endotoxin is not essential for the development of cockroach induced allergic airway inflammation.

  Authors: Yoo Seob Shin, Jung-Ho Sohn, Joo-Young Kim, Jae Hyun Lee, Sang-Heon Cho, Soo-Jong Hong, Joo-Shil Lee, Chein-Soo Hong, Jung-Won Park

  Yonsei medical journal. 53(3):593-602.

  Purpose: Cockroach (CR) is an important inhalant allergen and can induce allergic asthma. However, the mechanism by which CR induces airway allergic inflammation and the role of endotoxin in CRPurpose: Cockroach (CR) is an important inhalant allergen and can induce allergic asthma. However, the mechanism by which CR induces airway allergic inflammation and the role of endotoxin in CR extract are not clearly understood in regards to the development of airway inflammation. In this study, we evaluated whether endotoxin is essential to the development of CR induced airway allergic inflammation in mice. Materials and Methods: Airway allergic inflammation was induced by intranasal administration of either CR extract, CR with additional endotoxin, or endotoxin depleted CR extract, respectively, in BALB/c wild type mice. CR induced inflammation was also evaluated with toll like receptor-4 (TLR-4) mutant (C3H/HeJ) and wild type (C3H/HeN) mice. Results: Intranasal administration of CR extracts significantly induced airway hyperresponsiveness (AHR), eosinophilic and neutrophilic airway inflammation, as well as goblet cell hyperplasia in a dose-dependent manner. The addition of endotoxin along with CR allergen attenuated eosinophilic inflammation, interleukin (IL)-13 level, and goblet cell hyperplasia of respiratory epithelium; however, it did not affect the development of AHR. Endotoxin depletion in CR extract did not attenuate eosinophilic inflammation and lymphocytosis in BAL fluid, AHR and IL-13 expression in the lungs compared to CR alone. The attenuation of AHR, eosinophilic inflammation, and goblet cell hyperplasia induced by CR extract alone was not different between TLR-4 mutant and the wild type mice. In addition, heat inactivated CR extract administration induced attenuated AHR and eosinophilic inflammation. Conclusion: Endotoxin in CR extracts may not be essential to the development of airway inflammation.

• Single-stage removal of thoracic dumbbell tumors from a posterior approach only with costotransversectomy.

  Authors: Kei Ando, Shiro Imagama, Norimitsux Wakao, Kenichi Hirano, Ryoji Tauchi, Akio Muramoto, Hiroki Matsui, Tomohiro Matsumoto, Yukihiro Matsuyama, Naoki Ishiguro

  Yonsei medical journal. 53(3):611-7.

  Purpose: Thoracic dumbbell tumors are relatively rare, usually arising from neurogenic elements. Methods for surgical removal thereof remain controversial. The purpose of this study was to evaluatePurpose: Thoracic dumbbell tumors are relatively rare, usually arising from neurogenic elements. Methods for surgical removal thereof remain controversial. The purpose of this study was to evaluate the surgical results of a single-stage posterior approach with laminectomy and costotransversectomy only for the management of thoracic dumbbell tumors. Materials and Methods: Eight cases of thoracic large dumbbell tumor were analyzed retrospectively: seven men and one woman (mean age, 49 years). Pathologic findings included schwannoma in five patients, neurofibroma in two patients (Recklinghausen in one patient), and ganglioneuroma in one patient. All patients underwent single-stage removal of dumbbell tumors by a posterior approach followed by laminectomy and costotransversectomy combined with instrumentation. Clinical and radiologic outcomes were reviewed, thereafter. Results: Operative time ranged from 185 to 420 minutes (mean, 313 minutes), with estimated blood loss ranging from 71 to 1830 mL (mean, 658 mL). Postoperative complications included atelectasis in one case. All patients had tumors successfully removed with no neurological deterioration. Spinal deformities were not observed in any patients at the last follow-up (mean, 52 months), with instrumentation. Conclusion: Single-stage surgery with laminectomy and costotransversectomy may be useful for removing thoracic dumbbell tumors without a combined anterior approach.

• Altered cellular kinetics in the growth plate of the femoral head of spontaneously hypertensive rats.

  Authors: Hoon Park, Sun Young Kong, Hyun Woo Kim

  Yonsei medical journal. 53(3):625-33.

  Purpose: Pathologic changes in the growth plate remain unknown in Legg-Calvé-Perthes (LCP) disease. Spontaneously hypertensive rats have proven to be a good model for studying LCP disease. This studyPurpose: Pathologic changes in the growth plate remain unknown in Legg-Calvé-Perthes (LCP) disease. Spontaneously hypertensive rats have proven to be a good model for studying LCP disease. This study investigated the histopathologic changes and the expression of vascular endothelial growth factor in the growth plate of spontaneously hypertensive rats (SHR). Materials and Methods: Sixty SHR rats were divided into two groups: those showing osteonecrosis (SHR+n group: 32), and those showing normal ossification (SHR-n group: 28). Thirty Wister Kyoto rats served as a control. For histomorphological measurement, the length of each zone of the growth plate was measured. Cell kinetics was measured by 5-bromo-2'-deoxyuridin (BrdU) immunohistochemistry and transferase-mediated deoxyuridine triphosphate-biotin nick end labeling (TUNEL) assays. Vascular endothelial growth factor (VEGF) immunohistochemistry was used to identify of expression of VEGF. Results: The lengths of growth plates of the SHR+n group were significantly shorter in the initial growth period than those of the other groups. The lowest proliferative rate and the highest apoptosis rate were observed in the SHR+n group at the initial growth period. The expression of VEGF in the growth plate of the SHR group was lower than the control group, and it was lower in the SHR+n group than in the SHR-n group. Conclusion: The growth plate of the SHR+n group was found to be affected by disease process of ischemic necrosis of the femoral head, and this might explain the relative overgrowth of the greater trochanter in the later stages of LCP disease.

• Facial Nerve Paralysis due to Chronic Otitis Media: Prognosis in Restoration of Facial Function after Surgical Intervention.

  Authors: Jin Kim, Gu-Hyun Jung, See-Young Park, Won Sang Lee

  Yonsei medical journal. 53(3):642-8.

  Purpose: Facial paralysis is an uncommon but significant complication of chronic otitis media (COM). Surgical eradication of the disease is the most viable way to overcome facial paralysis therefrom.Purpose: Facial paralysis is an uncommon but significant complication of chronic otitis media (COM). Surgical eradication of the disease is the most viable way to overcome facial paralysis therefrom. In an effort to guide treatment of this rare complication, we analyzed the prognosis of facial function after surgical treatment. Materials and Methods: A total of 3435 patients with COM, who underwent various otologic surgeries throughout a period of 20 years, were analyzed retrospectively. Forty six patients (1.33%) had facial nerve paralysis caused by COM. We analyzed prognostic factors including delay of surgery, the extent of disease, presence or absence of cholesteatoma and the type of surgery affecting surgical outcomes. Results: Surgical intervention had a good effect on the restoration of facial function in cases of shorter duration of onset of facial paralysis to surgery and cases of sudden onset, without cholesteatoma. No previous ear surgery and healthy bony labyrinth indicated a good postoperative prognosis. Conclusion: COM causing facial paralysis is most frequently due to cholesteatoma and the presence of cholesteatoma decreased the effectiveness of surgical treatment and indicated a poor prognosis after surgery. In our experience, early surgical intervention can be crucial to recovery of facial function. To prevent recurrent cholesteatoma, which leads to local destruction of the facial nerve, complete eradication of the disease in one procedure cannot be overemphasized for the treatment of patients with COM.

• Phagocytosis and endocytosis of silver nanoparticles induce interleukin-8 production in human macrophages.

  Authors: Seungjae Kim, In-Hong Choi

  Yonsei medical journal. 53(3):654-7.

  Phagocytosis or endocytosis by macrophages is critical to the uptake of fine particles, including nanoparticles, in order to initiate toxic effects in cells. Here, our data enhance the understandingPhagocytosis or endocytosis by macrophages is critical to the uptake of fine particles, including nanoparticles, in order to initiate toxic effects in cells. Here, our data enhance the understanding of the process of internalization of silver nanoparticles by macrophages. When macrophages were pre-treated with inhibitors to phagocytosis, caveolin-mediated endocytosis, or clathrin-mediated endocytosis, prior to exposure to silver nanoparticles, Interleukin-8 (IL-8) production was inhibited. Although cell death was not reduced, the inflammatory response by macrophages was compromised by phagocytosis and endocytosis inhibitors.

• A Trp33Arg Mutation at Exon 1 of the MYH9 Gene in a Korean Patient with May-Hegglin Anomaly.

  Authors: Moon Ju Jang, Hyun-Jeong Park, So Young Chong, Ji Young Huh, In-Ho Kim, Ja-Hyun Jang, Hee-Jin Kim, Doyeun Oh

  Yonsei medical journal. 53(3):662-6.

  In this report, we describe a Korean patient with May-Hegglin anomaly from a mutation of the MYH9 gene. The proband was a 21-year-old man with thrombocytopenia. He did not have a bleeding tendency.In this report, we describe a Korean patient with May-Hegglin anomaly from a mutation of the MYH9 gene. The proband was a 21-year-old man with thrombocytopenia. He did not have a bleeding tendency. His neutrophil count was normal at 7490/mm³; however, the neutrophils contained abnormal basophilic inclusions in their cytoplasm. The platelet count was decreased at 15000/mm³ with giant platelets. Coagulation test results were not remarkable. Direct sequencing of MYH9 revealed that he was heterozygous for a mutation in exon 1, which was a 97T>A substitution mutation affecting codon 33, substituting tryptophan with arginine (Trp33Arg). Family study showed that both of his parents had normal phenotype and genotypes, indicating a de novo occurrence of the mutation in the proband.

• Cerebrovascular disease and stroke in korean male adolescent.

  Authors: Chang Hyun Oh, Joonho Chung, Dongkeun Hyun, Eunyoung Kim, Hyeonseon Park

  Yonsei medical journal. 53(3):467-76.

  Purpose: This study aimed to estimate the prevalence and incidence of cerebrovascular disease (CVD) and stroke in Korean male adolescents. Materials and Methods: The authors reviewed all medicalPurpose: This study aimed to estimate the prevalence and incidence of cerebrovascular disease (CVD) and stroke in Korean male adolescents. Materials and Methods: The authors reviewed all medical certificates, medical records, and radiologic images from the examinations of Korean military conscription from January 2008 to May 2011. Results: Of the 101156 examinees, 40 had CVD and stroke during adolescence. The overall prevalence and incidence of CVD and stroke was 39.54 cases per 100000 adolescents and 2.08 cases per 100000 adolescents per year, respectively and these were similar to the worldwide data. There were 3 cases of aneurysm, 3 cases of dural arteriovenous fistula, 11 cases of arteriovenous malformation, 4 cases of cavernous hemangioma, 4 cases of cerebrovascular infarction, 16 cases of Moyamoya disease, and 1 case of missing data. The incidence of arteriovenous malformation (0.57 cases per 100000 adolescents per year) was lower than the incidence for the worldwide general population. The incidence of Moyamoya disease was higher than that in any other country (15.82 cases per 100000 adolescents, vs. 0.83 cases per 100000 adolescents per year). Conclusion: We observed ischemic and hemorrhagic stroke, each accounting for approximately half of cases, and high incidence of Moyamoya disease with low incidence of arteriovenous malformation in Korean male adolescents.

• Lateralization of cognitive functions in aphasia after right brain damage.

  Authors: Ji-Wan Ha, Sung-Bom Pyun, Yu Mi Hwang, Hyunsub Sim

  Yonsei medical journal. 53(3):486-94.

  Purpose: The lateralization of cognitive functions in crossed aphasia in dextrals (CAD) has been explored and compared mainly with cases of aphasia with left hemisphere damage. However, comparing thePurpose: The lateralization of cognitive functions in crossed aphasia in dextrals (CAD) has been explored and compared mainly with cases of aphasia with left hemisphere damage. However, comparing the neuropsychological aspects of CAD and aphasia after right brain damage in left-handers (ARL) could potentially provide more insights into the effect of a shift in the laterality of handedness or language on other cognitive organization. Thus, this case study compared two cases of CAD and one case of ARL. Materials and Methods: The following neuropsychological measures were obtained from three aphasic patients with right brain damage (two cases of CAD and one case of ARL); language, oral and limb praxis, and nonverbal cognitive functions (visuospatial neglect and visuospatial construction). Results: All three patients showed impaired visuoconstructional abilities, whereas each patient showed a different level of performances for oral and limb praxis, and visuospatial neglect. Conclusion: Based on the analysis of these three aphasic patients' performances, we highlighted the lateralization of language, handedness, oral and limb praxis, visuospatial neglect and visuospatial constructive ability in aphasic patients with right brain damage.

• Correlations between Serum Inflammation Factors and Left Ventricular Remodeling in Acute ST Segment Elevation Myocardial Infarction.

  Authors: Jing Tan, Qi Hua

  Yonsei medical journal. 53(3):501-7.

  Purpose: To investigate the changes and correlations of the serum inflammation factors levels and left ventricular (LV) structure and function in patients with acute ST segment elevation myocardialPurpose: To investigate the changes and correlations of the serum inflammation factors levels and left ventricular (LV) structure and function in patients with acute ST segment elevation myocardial infarction (STEMI). Materials and Methods: A prospective study was performed on 70 STEMI patients and 70 control subjects. Serum levels of interleukin-6 (IL-6), soluble CD40 ligand (sCD40L), metalloproteinase- 9 (MMP-9) and tissue inhibitor of metalloproteinase-1 (TIMP-1) were measured by sandwich enzyme-linked immunosorbent assay (ELISA), and cardiac structure and function were assessed by echocardiography at admission and 3-year follow-up. Results: We found that the levels of serum IL-6, sCD40L and MMP-9 increased steadily among control subjects, remote myocardial infarction and acute STEMI patients, and the level of TIMP-1 elevated remarkly at 3-year follow-up visit in STEMI. The admission level of serum MMP-9 positively correlated with LV end-diastolic and end-diastole volume (r=0.294, p=0.022; r=0.269, p=0.036, respectively), and TIMP-1 positively correlated with E/A ratio (r=0.278, p=0.044) at 3-year follow-up. Conclusion: The study indicates that admission levels of serum MMP-9 and TIMP-1 closely correlated with left ventricular structure and function, which may be involved in the process of post-infarction remodeling of myocardium.

• Diagnostic patterns in the evaluation of patients presenting with syncope at the emergency or outpatient department.

  Authors: Gu Hyun Kang, Ju Hyeon Oh, June Soo Kim, Young Keun On, Hyoung Gon Song, Ik Joon Jo, Su Jin Kim, Su-Jin Bae, Tae Gun Shin

  Yonsei medical journal. 53(3):517-23.

  Purpose: Patterns of syncope evaluation vary widely among physicians and hospitals. The aim of this study was to assess current diagnostic patterns and medical costs in the evaluation of patientsPurpose: Patterns of syncope evaluation vary widely among physicians and hospitals. The aim of this study was to assess current diagnostic patterns and medical costs in the evaluation of patients presenting with syncope at the emergency department (ED) or the outpatient department (OPD) of a referral hospital. Materials and Methods: This study included 171 consecutive patients with syncope, who visited the ED or OPD between January 2009 and July 2009. Results: The ED group had fewer episodes of syncope [2 (1-2) vs. 2 (1-5), p=0.014] and fewer prodromal symptoms (81.5% vs. 93.3%, p=0.018) than the OPD group. Diagnostic tests were more frequently performed in the ED group than in the OPD group (6.2±1.7 vs. 5.3±2.0; p=0.012). In addition, tests with low diagnostic yields were more frequently used in the ED group than in the OPD group. The total cost of syncope evaluation per patient was higher in the ED group than in the OPD group [823000 (440000-1408000) won vs. 420000 (186000-766000) won, p<0.001]. Conclusion: There were some differences in the clinical characteristics of patients and diagnostic patterns in the evaluation of syncope between the ED and the OPD groups. Therefore, a selective diagnostic approach according to the presentation site is needed to improve diagnostic yields and to reduce the time and costs of evaluation of syncope.

• Myeloperoxidase expression in acute myeloid leukemia helps identifying patients to benefit from transplant.

  Authors: Yundeok Kim, Sulhee Yoon, Soo Jeong Kim, Jin Seok Kim, Jun-Won Cheong, Yoo Hong Min

  Yonsei medical journal. 53(3):530-6.

  Purpose: Despite extensive study, the use of allogeneic hematopoietic stem cell transplantation in acute myeloid leukemia (AML) vary considerably. The decision of which of these options to choose isPurpose: Despite extensive study, the use of allogeneic hematopoietic stem cell transplantation in acute myeloid leukemia (AML) vary considerably. The decision of which of these options to choose is complex and depends on both clinical and molecular variables as well as the availability and histocompatability of donor stem cells. So far there is no clear explanation on whether the expression of myeloperoxidase (MPO) relates to the prognosis of AML. Materials and Methods: We retrospectively analyzed the prognostic significance of the MPO expression in the 140 patients with diagnosed AML treated at a single institution. Results: In our study, MPO expression was associated with disease-free survival (DFS) and transplant was beneficial to overcome a negative prognostic effect of MPO-negative at diagnosis based upon the result that the DFS in patients received transplants are not significant between the MPO-positive group and MPO-negative group although DFS in all patients was different according to MPO expression. Conclusion: MPO expression at diagnosis helps to choose therapy for each AML patient and can differentiate AML patients who need transplantation.

• Recent changes in the clinicopathologic features of korean men with prostate cancer: a comparison with Western populations.

  Authors: Seok-Soo Byun, Sangchul Lee, Sang Eun Lee, Eunsik Lee, Seong Il Seo, Hyun Moo Lee, Han Yong Choi, Cheryn Song, Hanjong Ahn, Young Deuk Choi, Jin Seon Cho

  Yonsei medical journal. 53(3):543-9.

  Purpose: The aim of this study was to evaluate the recent changes in the clinicopathologic features of prostate cancer in Korea and to compare these features with those of Western populations.Purpose: The aim of this study was to evaluate the recent changes in the clinicopathologic features of prostate cancer in Korea and to compare these features with those of Western populations. Materials and Methods: We retrospectively reviewed the data of 1582 men undergoing radical prostatectomy for clinically localized prostate cancer between 1995 and 2007 at 10 institutions in Korea for comparison with Western studies. The patients were divided into two groups in order to evaluate the recent clinicopathological changes in prostate cancer: Group 1 had surgery between 1995 and 2003 (n=280) and Group 2 had surgery between 2004 and 2007 (n=1302). The mean follow-up period was 24 months. Results: Group 1 had a higher prostate-specific antigen level than Group 2 (10.0 ng/mL vs. 7.5 ng/mL, respectively; p<0.001) and a lower proportion of biopsy Gleason scores ≤6 (35.0% vs. 48.1%, respectively; p<0.001). The proportion of patients with clinical T1 stage was higher in Group 2 than in Group 1. Group 1 had a lower proportion of organ-confined disease (59.6% vs. 68.6%; p<0.001) and a lower proportion of Gleason scores ≤6 (21.3% vs. 33.0%; p<0.001), compared to Group 2. However, the relatively higher proportion of pathologic Gleason scores ≤6 in Group 2 was still lower than those of Western men, even though the proportion of organ-confined disease reached to that of Western series. Conclusion: Korean men with prostate cancer currently present better clinicopathologic parameters. However, in comparison, Korean men still show relatively worse pathologic Gleason scores than Western men.

• Evaluation of the UniCel™ DxI 800 Immunoassay Analyzer in Measuring Five Tumor Markers.

  Authors: Younhee Park, Yongjung Park, Jungyong Park, Hyon-Suk Kim

  Yonsei medical journal. 53(3):557-64.

  Purpose: Tumor marker concentrations in a given specimen measured by different analyzers vary according to assay methods, epitopes for antibodies used, and reagent specificities. Although greatPurpose: Tumor marker concentrations in a given specimen measured by different analyzers vary according to assay methods, epitopes for antibodies used, and reagent specificities. Although great effort in quality assessment has been instituted, discrepancies among results from different analyzers are still present. We evaluated the assay performance of the UniCel™ DxI 800 automated analyzer in measuring the alpha-fetoprotein (AFP), carcinoembryonic antigen (CEA), carbohydrate antigen (CA) 125, CA 15-3 and CA 19-9 tumor markers. Materials and Methods: The linearity and precision performance of the five tumor marker assays were evaluated, and concentrations of the respective markers as measured by DxI were compared to those measured by other conventional analyzers (ADVIA Centaur™ and Vitros™ ECi) using 200 specimens collected from 100 healthy persons and 100 patients with respective cancers. Results: The linear fits for all five tumor markers were statistically acceptable (F=4648 for AFP, F=15846 for CEA, F=6445 for CA 125, F=2285 for CA 15-3, F=7459 for CA 19-9; p<0.0001 for all). The imprecision of each tumor marker assay was less than 5% coefficient of variation, except for low and high concentrations of AFP. The results from UniCel™ DxI 800 were highly correlated with those from other analyzers. Conclusion: Our results demonstrate that UniCel™ DxI 800 has good linearity and precision performance for the tumor markers assayed in this study. However, there were discrepancies between assaying methods. Efforts to standardize tumor marker assays should be undertaken, and the redetermination of cut-off levels is necessary when developing methods of analyzing tumor markers.

• Hereditary thrombophilia in korean patients with idiopathic pulmonary embolism.

  Authors: Mirae Lee, Hye Jin No, Shin Yi Jang, Nari Kim, Seung Hyuk Choi, Hojoong Kim, Sun-Hee Kim, Hee-Jin Kim, Duk-Kyung Kim

  Yonsei medical journal. 53(3):571-7.

  Purpose: Hereditary thrombophilia (HT) is a major risk factor for idiopathic pulmonary embolism (iPE) and shows different prevalence among ethnic groups. The prevalence and clinical characteristicsPurpose: Hereditary thrombophilia (HT) is a major risk factor for idiopathic pulmonary embolism (iPE) and shows different prevalence among ethnic groups. The prevalence and clinical characteristics of HT in Korean patients with iPE were investigated. Materials and Methods: Patients with PE on computed tomography (CT) scan were recruited, and those with malignancy were excluded. Patients were divided into iPE and provoked PE (pPE) groups. The presence of HT in the iPE group was assessed by DNA sequencing of the corresponding gene in patients who had low levels of natural anticoagulants. The clinical characteristics of iPE with HT (iPE/HT+) were compared with those of iPE without HT (iPE/HT-) and pPE. Results: Out of 161 patients, 84 patients had iPE and 77 patients had pPE. Among 54 patients in the iPE group whose coagulation profiles were tested, 28 patients were diagnosed with HT (51.9%; 28/54). Compared with the iPE/HT- and pPE groups, the iPE/HT+ group showed the highest proportion of male patients (71.4%; p<0.001); the youngest mean age (44±14 years; p<0.001); and the highest frequencies for history of venous thromboembolism (64.3%; p<0.001), concurrent deep vein thrombosis (75.0%; p=0.021), and adverse clinical outcomes (42.9%, p<0.001). Protein C deficiency was the most common HT. On molecular genetic tests, causative mutation was identified in 13 patients. Conclusion: In this study of Korean patients, about half of the patients with iPE had HT. Patients with iPE and HT were mostly young males with deep venous thrombosis (DVT), previous venous thromboembolism (VTE), and frequent adverse clinical outcomes. Therefore, Korean patients with iPE should be tested for HT.

• Urinary hypoxia-inducible factor-1alpha levels are associated with histologic chronicity changes and renal functionin patients with lupus nephritis.

  Authors: Chunyang Ma, Jiali Wei, Feng Zhan, Ru Wang, Keying Fu, Xiaoping Wan, Zhuori Li

  Yonsei medical journal. 53(3):587-92.

  Purpose: Tubulointerstitial hypoxia in the kidney is considered a hallmark of injury and a mediator of the progression of tubulointerstitial fibrosis. Hypoxia-inducible factor-1alpha (HIF-1alpha), aPurpose: Tubulointerstitial hypoxia in the kidney is considered a hallmark of injury and a mediator of the progression of tubulointerstitial fibrosis. Hypoxia-inducible factor-1alpha (HIF-1alpha), a master transcription factor in cellular adaptation to hypoxia, regulates a wide variety of genes, some of which are closely associated with tissue fibrosis. The present study set out to characterize urinary HIF-1alpha expressions in patients with lupus nephritis (LN) and to explore whether urinary HIF-1alpha expressions are associated with histologic chronicity changes and renal function. Materials and Methods: Urinary HIF-1alpha levels were measured by enzyme-linked immunosorbent assays in 42 patients with LN and in 30 healthy controls. Activity and chronicity indexes as well as tubular HIF-1alpha expressions were analyzed for each specimen. Results: Urinary HIF-1alpha levels were higher in LN patients than in healthy controls (3.977±1.696 vs. 2.153±0.554 ng/mL, p<0.001) and were associated with histologic chronicity indexes (r=0.463, p<0.01) and eGFR (r=-0.324, p<0.05). However, urinary HIF-1alpha levels showed no correlation with histologic activity indexes, anti-dsDNA, ANA, complement 3 and 4 levels, proteinuria, systemic lupus erythematosis disease activity index, and WHO pathological classification (p>0.05). Conclusion: Urinary HIF-1alpha levels were elevated in LN patients and were associated with histologic chronicity changes and renal function, indicating that HIF-1alpha might contribute to histologic chronicity in LN.

• Health related quality of life in common variable immunodeficiency.

  Authors: Isabella Quinti, Cristina Di Pietro, Helene Martini, Anna Maria Pesce, Francesca Lombardi, Maddalena Baumghartner, Stefania Colantuono, Cinzia Milito, Stefano Tabolli

  Yonsei medical journal. 53(3):603-10.

  Purpose: To quantify the health related quality of life in primary immunodeficiency patients. Materials and Methods: We used generic health status and general psychological health questionnaires toPurpose: To quantify the health related quality of life in primary immunodeficiency patients. Materials and Methods: We used generic health status and general psychological health questionnaires to determine the range of issues that needed to be considered in examining the burden of common variable immunodeficiency (CVID). Results: The health status of patients with CVID was lower than that observed in normal subjects. Overall, Role-Physical and General Health scales correlated with a poorer clinical status. Surprisingly, the duration of disease did not influence health status. Being female, older, General Health Questionnaire-positive and alexithymic proved to be major risk factors associated with a poor health status. Patients with chronic lung disease and chronic diarrhea had the lowest values on the Medical Outcome Study, Short Form SF-36 (SF-36) scales. Disease severity perception was associated with the General Health Questionnaire and alexithymia status. Limitations in daily activities as a result of lower physical health were the major problems facing common variable immunodeficiency patients. Conclusion: Our data underlined the importance of conducting a periodical health related quality of life assessment on patients with primary antibody deficiencies and, moreover, stressed the necessity of providing psychological support to at risk patients.

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