Background: Hereditary hemolytic anemia (HHA) is a rare disease characterized by premature red blood cell (RBC) destruction due to intrinsic RBC defects. The RBC Disorder Working Party of the Korean Society of Hematology established and updated the standard operating procedure for making an accurate diagnosis of HHA since 2007. The aim of this study was to investigate a nationwide epidemiology of Korean HHA. Methods: We collected the data of a newly diagnosed pediatric HHA cohort (2007–2016) and compared this cohort's characteristics with those of a previously surveyed pediatric HHA cohort (1997–2006) in Korea. Each participant's information was retrospectively collected by a questionnaire survey. Results: A total of 369 children with HHA from 38 hospitals distributed in 16 of 17 districts of Korea were investigated. RBC membranopathies, hemoglobinopathies, RBC enzymopathies, and unknown etiologies accounted for 263 (71.3%), 59 (16.0%), 23 (6.2%), and 24 (6.5%) of the cases, respectively. Compared to the cohort from the previous decade, the proportions of hemoglobinopathies and RBC enzymopathies significantly increased (P < 0.001 and P = 0.008, respectively). Twenty-three of the 59 hemoglobinopathy patients had immigrant mothers, mostly from South-East Asia. Conclusion In Korea, thalassemia traits have increased over the past 10 years, reflecting both increased awareness of this disease and increased international marriages. The enhanced recognition of RBC enzymopathies is due to advances in diagnostic technique; however, 6.5% of HHA patients still do not have a clear diagnosis. It is necessary to improve accessibility of diagnosing HHA.

No abstract available.
Humans ; Intubation* ; Klippel-Feil Syndrome* ; Laryngoscopes*

BACKGROUND AND OBJECTIVES: In patients with ST-segment elevation myocardial infarction (STEMI) and multivessel disease, complete revascularization (CR) for non-culprit lesions is not routinely recommended. The aim of this study was to compare the clinical outcomes of multivessel compared with infarct-related artery (IRA)-only revascularization in patients undergoing primary percutaneous coronary intervention (PCI) for STEMI. SUBJECTS AND METHODS: From the Korean Acute Myocardial Infarction Registry (KAMIR) database, 1,094 STEMI patients with multivessel disease who underwent primary PCI with drug-eluting stents were enrolled in this study. The patients were divided into two groups: culprit-vessel-only revascularization (COR, n=827) group; multivessel revascularization, including non-IRA (MVR, n=267) group. The primary endpoint of this study included major adverse cardiac events (MACEs), such as death, myocardial infarction, or target or nontarget lesion revascularization at one year. RESULTS: There was no difference in clinical characteristics between the two groups. During the one-year follow-up, 102 (15.2%) patients in the COR group and 32 (14.2%) in the MVR group experienced at least one MACE (p=0.330). There were no differences between the two groups in terms of rates of death, myocardial infarction, or revascularization (2.1% vs. 2.0%, 0.7% vs. 0.8%, and 11.7% vs. 10.1%, respectively; p=0.822, 0.910, and 0.301, respectively). The MACE rate was higher in the incompletely revascularized patients than in the completely revascularized patients (15% vs. 9.5%, p=0.039), and the difference was attributable to a higher rate of nontarget vessel revascularization (8.6% vs. 1.8%, p=0.002). CONCLUSION: Although multivessel angioplasty during primary PCI for STEMI did not reduce the MACE rate compared with culprit-vessel-only PCI, CR was associated with a lower rate of repeat revascularization after multivessel PCI.
Angioplasty ; Arteries ; Coronary Artery Disease ; Drug-Eluting Stents ; Follow-Up Studies ; Glycosaminoglycans ; Humans ; Myocardial Infarction ; Percutaneous Coronary Intervention

BACKGROUND: Myocardial revascularization in patients with renal insufficiency is challenging to the cardiac surgeon, irrespective of utilizing extracorporeal circulation. This study aimed to compare the number of bypass grafts and the mid-term results and to evaluate independent survival predictors in patients with renal insufficiency undergoing on-pump or off-pump myocardial revascularization. MATERIALS AND METHODS: We retrospectively analyzed the data of 103 patients with renal insufficiency, who had isolated myocardial revascularization between January 1999 and January 2009. The patients were divided into two groups, the on-pump group and the off-pump group. RESULTS: The off-pump group received a significantly greater number of distal arterial grafts than the on-pump group. However, the mean number of total grafts, the degree of complete revascularization, and survival rate of the patients were not significantly different between the two groups. Multivariate analysis showed the independent predictors for reduced mid-term survival were the number of total grafts and postoperative periodic renal replacement therapy. Off-pump myocardial revascularization does not decrease the number of bypass grafts or influence on the mid-term results for patients with renal insufficiency, compared to on-pump myocardial revascularization. CONCLUSION: Myocardial revascularization with a large number of total grafts has a beneficial effect on survival in patients with renal insufficiency, irrespective of utilizing extracorporeal bypass.
Extracorporeal Circulation ; Humans ; Multivariate Analysis ; Myocardial Revascularization ; Postoperative Period ; Renal Insufficiency ; Renal Replacement Therapy ; Retrospective Studies ; Survival Rate ; Transplants

PURPOSE: Lymphatic spread of tumor is an important prognostic factor for patients with non-small cell lung carcinoma (NSCLC). Vascular endothelial growth factor-C (VEGF-C) and VEGF-D play important roles in lymphangiogenesis via the VEGF receptor 3 (VEGFR-3). We sought to determine whether VEGF-C, VEGF-D and VEGFR-3 are involved in the clinical outcomes of patients with resected NSCLC. MATERIALS AND METHODS: Using immunohistochemical staining, we investigated the protein expressions of VEGF-C, VEGF-D and VEGFR-3 in the tissue array specimens from patients who underwent resection for NSCLC. The immunoreactivity for p53 was also examined. The clinicopathological implications of these molecules were statistically analyzed. RESULTS: Analysis of a total of 118 specimens showed that VEGF-C, VEGF-D and their co-expression were significantly associated with more advanced regional lymph node metastasis (p=0.019, p=0.044 and p=0.026, respectively, N2 versus N0 and N1). A VEGFR-3 expression had a strong correlation with peritumoral lymphatic invasion (p=0.047). On the multivariate analysis for survival and recurrence, pathologic N2 lymph node metastasis was the only independent prognostic factor, but none of the investigated molecules showed any statistical correlation with recurrence and survival. CONCLUSIONS: The present study revealed that high expressions of VEGF-C and VEGF-D were strongly associated with more advanced regional lymph node metastasis in patients with resected NSCLC.
Carcinoma, Non-Small-Cell Lung ; Humans ; Lung ; Lymph Nodes ; Lymphangiogenesis ; Multivariate Analysis ; Neoplasm Metastasis ; Receptors, Vascular Endothelial Growth Factor ; Recurrence ; Vascular Endothelial Growth Factor A ; Vascular Endothelial Growth Factor C ; Vascular Endothelial Growth Factor D ; Vascular Endothelial Growth Factor Receptor-3 ; Vascular Endothelial Growth Factors

BACKGROUND: Emergence agitation frequently occurs after desflurane anesthesia in children.Nalbuphine, because of its sedative and analgesic properties, might be useful for the management of this side effect.We studied the effect of nalbuphine on recovery characteristics and emergence agitation after desflurane anesthesia in children for strabismus surgery. METHODS: 41 patients (3-14 yr) scheduled for pediatric strabismus surgery were included.All children received ketamine 0.5 mg/kg intravenously before entering the operating room.After intravenous induction with thiopental and rocuronium to facilitate endotracheal intubation, patients were randomly assigned to receive saline, or nalbuphine 0.2 mg/kg respectively. Anesthesia was maintained with desflurane 4-6% with N2O : O2 = 2 : 1.At the end of anesthesia, time to cough, extubation, movement, eye opening and discharge were recorded.Emergence agitation was recorded by three point rating scale. RESULTS: Agitation scores were significantly different between the two groups (P < 0.01).Time to extubation and movement were similar between two groups.Time to eye opening was significantly increased in nalbuphine group (P < 0.05).But, there was no difference in time to discharge from the recovery room to the ward between the two groups. CONCLUSIONS: In children undergoing strabismus surgery with desflurane anesthesia, nalbuphine 0.2 mg/kg administered immediately after induction reduced incidence of emergence agitation without delaying discharge from recovery room.
Androstanols ; Anesthesia ; Child ; Cough ; Dihydroergotamine ; Eye ; Eye Movements ; Humans ; Incidence ; Intubation, Intratracheal ; Isoflurane ; Ketamine ; Nalbuphine ; Recovery Room ; Strabismus ; Thiopental

BACKGROUND: Coronary artery disease (CAD) has recently become one of the major causes of mortality and morbidity in Korea. However, not much epidemiologic and demographic data has yet been reported. The purpose of this study was to investigate the clinical features as well as the prognostic factors of patients with CAD. METHODS: We prospectively enrolled 1,665 consecutive patients with CAD who had been admitted to the Catholic University Hospitals from December 1999 to April 2003. RESULTS: Acute myocardial infarction (AMI) was the most common cause of admission (n=715, 42.9%). Dyslipidemia, hypertension and smoking were the most common risk factors. More than 70% of the patients who underwent percutaneous coronary intervention (PCI) received stent implantation. A total of 965 (612 males) patients were followed at least for 6 months (the mean follow-up duration was 23.8+/-12.2 months). The incidence rates of major adverse cardiac events (MACE: cardiac death, acute myocardial infarction, target vessel revascularization) and cardiac death were 15.1% (n=146) and 2.2% (n=21), respectively. There was no difference in overall survival between the patients treated with medical therapy and those treated with PCI. By Cox regression analysis, the independent prognostic factors for MACE were PCI (95% CI: 1.75-4.85; p<0.01) and multivessel disease (95% CI: 1.03-2.04; p<0.05), and the independent prognostic factors for cardiac death were medical therapy (95% CI: 1.08-14.41; p<0.05) and old age (95% CI: 1.13-16.13; p<0.05). CONCLUSIONS: There was no difference in overall survival between the patients treated with medical therapy and those treated with PCI. However, PCI was superior to medical therapy for preventing death of the patients with acute coronary syndrome.
Acute Coronary Syndrome ; Coronary Artery Disease* ; Coronary Disease ; Coronary Vessels* ; Death ; Dyslipidemias ; Follow-Up Studies ; Heart* ; Hospitals, University ; Humans ; Hypertension ; Incidence ; Korea ; Mortality ; Myocardial Infarction ; Percutaneous Coronary Intervention ; Prognosis ; Prospective Studies ; Risk Factors ; Smoke ; Smoking ; Stents

BACKGROUND: Second-line chemotherapy offers advanced non-small cell lung cancer (NSCLC) patients a small, but significant increase in survival. Docetaxel is usually administered as a 3-week schedule, yet there is significant toxicity with this therapy. Therefore, a weekly schedule has been explored in several previous trials. In this retrospective study, we compared the efficacy and safety of a weekly schedule and a 3-week schedule of docetaxel monotherapy in a second-line setting. METHODS: Docetaxel was administered as 75 mg/m2 on day 1 every 3 weeks or as 37.5 mg/m2 on day 1 and 8 every 3 weeks until disease progression or severe toxicity developed. RESULTS: From October 2003 to March 2006, a total of 37 patients received docetaxel monotherapy and 36 patients could be evaluated. A total of 135 cycles were administered and then evaluated. The median overall survival was 13.3 months (95% confidence interval: 6.3~20.3) for the weekly schedule and 10.7 months (95% confidence interval: 8.3~13.0) for the 3-week schedule (p=0.41). The median time to progression was 3.0 months (95% confidence interval: 1.9~4.0) and 2.8 months (95% confidence interval: 1.0~4.6), respectively (p=0.41). The response rate was 16.7% for the weekly schedule and 21.1% for the 3-week schedule. The major form of hematologic toxicity was grade 3-4 neutropenia (3-week: 38.9%, weekly: 9.5%). The non-hematologic toxicities were similar between the two schedules. There were no treatment-related deaths. CONCLUSIONS: A docetaxel weekly schedule was very tolerable and it had comparable activity to that of the 3-week docetaxel schedule. Considering the efficacy and tolerability, a docetaxel weekly schedule can be an alternative schedule for the standard treatment of NSCLC in a second-line setting.
Adult ; Aged ; Antineoplastic Agents/*administration & dosage/adverse effects ; Carcinoma, Non-Small-Cell Lung/*drug therapy/pathology ; Drug Administration Schedule ; Female ; Humans ; Lung Neoplasms/*drug therapy/pathology ; Male ; Middle Aged ; Neoplasm Staging ; Retrospective Studies ; Taxoids/*administration & dosage/adverse effects ; Treatment Outcome

The perforation and migration of ingested sharp metallic bodies is a rare event. A perforation of the gastrointestinal tract is difficult to accurately and quickly diagnose when there is no peritonitis or abscess formation. Patients often present with no symptoms. The discovery of a foreign body on a radiological examination of the abdomen may be made incidentally. Moreover, a history of ingestion is usually difficult to obtain. Foreign bodies after perforation have been reported to migrate to any intra-abdominal site and to extra-abdominal sites in rare cases. We report one case of young man with ingested needle in which perforated silently and migrated to the lesser omentum. A 23-year-old man presented with an incidental foreign body. He has no definite ingestion history or psychiatric disorder, but was a heavily drinker. An abdominal plain X-ray showed a needle in the upper abdominal area. Computed tomography revealed the foreign body to be located beneath the liver and in the lesser omentum. The patient underwent a laparoscopic examination performed in the supine position. After creating a pneumoperitoneum by CO2 gas insufflation at 12 mmHg, 3 trochars (two 5-mm and 12-mm) were introduced at each subcostal and supraumbilical area. Surgical management using laparoscopic extraction was successful. The patient was discharged in good health on the 3rd day after the procedure. Laparoscopy can be used to remove ingested foreign bodies when surgery is indicated.
Abdomen ; Abscess ; Eating ; Foreign Bodies ; Gastrointestinal Tract ; Humans ; Insufflation ; Laparoscopy ; Liver ; Needles* ; Omentum* ; Peritonitis ; Pneumoperitoneum ; Stomach* ; Supine Position ; Young Adult

Recently the transcriptional up-regulation of human beta-defensin 2 (HBD-2) by lipopolysaccharide (LPS) was found to be associated with NF-kappaB binding site. Although the general mechanisms of NF-kappaB activation by LPS stimulation are well understood, less is known about the signal transduction pathway leading to LPS-induced NF-kappaB activation in human corneal epithelial (HCE) cells. The aim of this study was to investigate the intracellular signals involved in LPS-induced HBD-2 mRNA expression in HCE cells. Pretreatments of inhibitors for NF-kappaB, protein tyrosine kinase, p38 mitogen activated protein kinase (MAPK), and c-Jun N-terminal kinase (JNK) attenuated the LPS-induced NF-kappaB DNA binding activity and HBD-2 mRNA expression. Furthermore, pretreatments with inhibitors for protein kinase C (PKC), phosphatidylcholine-phospholipase C, phosphatidylinositol-phospholipase C, or phosphatidate phosphohydrolase prevented LPS-induced HBD-2 mRNA expression and HBD-2 prmoter-driven luciferase activity. However, the increased expression of HBD-2 mRNA and the increased DNA binding activity of NF-kappaB induced by LPS were not changed by the blockage of extracellular signal-regulated kinase (ERK) and of addition of antioxidants. Forskolin, a protein kinase A (PKA) agonist did not induce HBD-2 mRNA expression. These data demonstrate that LPS-induced HBD-2 mRNA expression via NF-kappaB is, at least in part, dependent on PKC, p38 MAPK, JNK, and protein tyrosine kinase status, but appears to be independent on PKA, ERK and ROS in HCE cells. Taken together, there may be more than one signaling pathways that lead to LPS-induced up-regulation of HBD-2 mRNA expression in HCE cells.
Antioxidants ; Binding Sites ; Colforsin ; Cyclic AMP-Dependent Protein Kinases ; DNA ; Epithelial Cells* ; Humans* ; JNK Mitogen-Activated Protein Kinases ; Luciferases ; NF-kappa B ; p38 Mitogen-Activated Protein Kinases ; Phosphatidate Phosphatase ; Phosphotransferases ; Protein Kinase C ; Protein Kinases ; Protein-Tyrosine Kinases ; RNA, Messenger* ; Signal Transduction ; Up-Regulation