Real-Life Effectiveness and Safety of Glecaprevir/Pibrentasvir for Korean Patients with Chronic Hepatitis C at a Single Institution

Background/Aims@#Glecaprevir/pibrentasvir (G/P) is a combination of direct-acting antiviral agents that is an approved treatment for chronic infections by all six hepatitis C virus (HCV) genotypes. However, there are limited data on the effect of G/P in Korean patients in actual real-world settings. We evaluated the real-life effectiveness and safety of G/P at a single institution in Korea. @*Methods@#This retrospective, observational, cohort study used sustained virologic response at 12 weeks after treatment completion (SVR12) as the primary effectiveness endpoint. Safety and tolerability were also determined. @*Results@#We examined 267 individuals who received G/P for chronic HCV infections. There were 148 females (55.4%), and the overall median age was 63.0 years (range, 25 to 87 years). Eightythree patients (31.1%) had HCV genotype-1 and 182 (68.2%) had HCV-2. A total of 212 patients (79.4%) were HCV treatment-naïve, 200 (74.9%) received the 8-week treatment, 13 (4.9%) had received prior treatment for hepatocellular carcinoma, 37 (13.7%) had chronic kidney disease stage 3 or higher, and 10 (3.7%) were receiving dialysis. Intention to treat (ITT) analysis indicated that 256 (95.9%) achieved SVR12. A modified ITT analysis indicated that SVR12 was 97.7% (256/262). Six patients failed therapy because of posttreatment relapse. SVR12 was significantly lower in those who received prior sofosbuvir treatment (p=0.002) and those with detectable HCV RNA at week 4 (p=0.027). Seventy patients (26.2%) experienced one or more adverse events, and most of them were mild. @*Conclusions@#These real-life data indicated that G/P treatment was highly effective and well tolerated, regardless of viral genotype or patient comorbidities.

The predictors of sustained virological response with sofosbuvir and ribavirin in patients with chronic hepatitis C genotype 2

Background/Aims@#Real-world, clinical practice data are lacking about sofosbuvir/ ribavirin (SOF/RBV) treatment of Korean patients with hepatitis C virus genotype 2 (HCV GT2) infection. This study investigated the efficacy and safety of SOF/RBV in Korean patients with HCV GT2 infection and clinical factors predicting sustained virological response 12 weeks (SVR12) after the end of SOF/RBV treatment. @*Methods@#A total of 181 patients with HCV GT2 with/without cirrhosis were treated with SOF/RBV for 16/12 weeks. Rapid virological response (RVR) was defined as non-detectable HCV RNA at 4 weeks. @*Results@#The RVR rate was 80.7% (146/181), the end of treatment response rate was 97.8% (177/181) and the SVR12 rate was 92.8% (168/181). Of eight patients with relapse, four did not achieve RVR. Three patients had a history of hepatocellular carcinoma (HCC). Multivariable analysis showed that RVR (p = 0.015) and no previous history of HCC (p = 0.007) were associated with SVR12. Factors significantly contributing to RVR included cirrhosis, creatinine concentration, and pre-treatment HCV RNA level. SVR12 rate was significantly higher in RVR (+) than RVR (–) patients (95.2% vs. 82.9%, p = 0.011) and also significantly higher in patients without than with a history of HCC (94.1% vs. 72.7%, p = 0.008). During treatment, 80/181 patients (44.2%) experienced mild to moderate adverse events, with 32 (17.7%) requiring RBV dose reductions due to anemia. @*Conclusions@#SOF/RBV treatment was effective and tolerable in HCV GT2 patients. RVR and no previous history of HCC were positive predictors of SVR12.

Real-Life Effectiveness and Safety of Glecaprevir/Pibrentasvir for Korean Patients with Chronic Hepatitis C at a Single Institution

Background/Aims@#Glecaprevir/pibrentasvir (G/P) is a combination of direct-acting antiviral agents that is an approved treatment for chronic infections by all six hepatitis C virus (HCV) genotypes. However, there are limited data on the effect of G/P in Korean patients in actual real-world settings. We evaluated the real-life effectiveness and safety of G/P at a single institution in Korea. @*Methods@#This retrospective, observational, cohort study used sustained virologic response at 12 weeks after treatment completion (SVR12) as the primary effectiveness endpoint. Safety and tolerability were also determined. @*Results@#We examined 267 individuals who received G/P for chronic HCV infections. There were 148 females (55.4%), and the overall median age was 63.0 years (range, 25 to 87 years). Eightythree patients (31.1%) had HCV genotype-1 and 182 (68.2%) had HCV-2. A total of 212 patients (79.4%) were HCV treatment-naïve, 200 (74.9%) received the 8-week treatment, 13 (4.9%) had received prior treatment for hepatocellular carcinoma, 37 (13.7%) had chronic kidney disease stage 3 or higher, and 10 (3.7%) were receiving dialysis. Intention to treat (ITT) analysis indicated that 256 (95.9%) achieved SVR12. A modified ITT analysis indicated that SVR12 was 97.7% (256/262). Six patients failed therapy because of posttreatment relapse. SVR12 was significantly lower in those who received prior sofosbuvir treatment (p=0.002) and those with detectable HCV RNA at week 4 (p=0.027). Seventy patients (26.2%) experienced one or more adverse events, and most of them were mild. @*Conclusions@#These real-life data indicated that G/P treatment was highly effective and well tolerated, regardless of viral genotype or patient comorbidities.

The predictors of sustained virological response with sofosbuvir and ribavirin in patients with chronic hepatitis C genotype 2

Background/Aims@#Real-world, clinical practice data are lacking about sofosbuvir/ ribavirin (SOF/RBV) treatment of Korean patients with hepatitis C virus genotype 2 (HCV GT2) infection. This study investigated the efficacy and safety of SOF/RBV in Korean patients with HCV GT2 infection and clinical factors predicting sustained virological response 12 weeks (SVR12) after the end of SOF/RBV treatment. @*Methods@#A total of 181 patients with HCV GT2 with/without cirrhosis were treated with SOF/RBV for 16/12 weeks. Rapid virological response (RVR) was defined as non-detectable HCV RNA at 4 weeks. @*Results@#The RVR rate was 80.7% (146/181), the end of treatment response rate was 97.8% (177/181) and the SVR12 rate was 92.8% (168/181). Of eight patients with relapse, four did not achieve RVR. Three patients had a history of hepatocellular carcinoma (HCC). Multivariable analysis showed that RVR (p = 0.015) and no previous history of HCC (p = 0.007) were associated with SVR12. Factors significantly contributing to RVR included cirrhosis, creatinine concentration, and pre-treatment HCV RNA level. SVR12 rate was significantly higher in RVR (+) than RVR (–) patients (95.2% vs. 82.9%, p = 0.011) and also significantly higher in patients without than with a history of HCC (94.1% vs. 72.7%, p = 0.008). During treatment, 80/181 patients (44.2%) experienced mild to moderate adverse events, with 32 (17.7%) requiring RBV dose reductions due to anemia. @*Conclusions@#SOF/RBV treatment was effective and tolerable in HCV GT2 patients. RVR and no previous history of HCC were positive predictors of SVR12.

A Case of Brain Herniation and Cerebrospinal Fluid Leakage during Endoscopic Marsupialization of Ethmoid Sinus Mucocele / 대한이비인후과학회지

Paranasal sinus mucocele is a slowly growing benign cystic lesion. It usually involves the frontal and ethmoid sinuses and can extend to adjacent structures, especially to the orbit, skull base and brain parenchyma. Prompt surgical intervention is needed when symptoms occur. Complete resection of mucocele is approached via endoscopic sinus surgery, while marsupialization is also widely considered. Recently, we encountered a case of spontaneous brain herniation and cerebrospinal fluid leakage during endoscopic marsupialization of ethmoid sinus mucocele. Herein, we report the case with a review of the literature.

A Case of Laryngeal Inflammatory Myofibroblastic Tumor / 대한두경부종양학회지

Inflammatory myofibrolastic tumor (IMT) is a rare borderline neoplasm. It frequently occurs in the lung but occasionally occurs in extrapulmonary sites such as the genitourinary tract, gastrointestinal tract, breast, salivary glands, sinonasal tract, orbit, and the central nervous system. Laryngeal involvement of IMT is very rare.A 61-year-old woman who complained of hoarseness persisting for 3 months visited our hospital. Laryngoscopy showed an elevated lesion in the right true vocal cord. Incisional biopsy was confirmed as larygeal inflammatory myofibrolastic tumor. We performed a transoral excision with CO2 LASER under suspension examination. Regional recurrence or distant metastasis was not observed after 9 months of follow-up. Herein we report a case of larygeal inflammatory myofibrolastic tumor that was treated with surgery alone, with a literature review.

A Case of Cervical Pott's Disease Presenting as Huge Retropharyngeal Abscess / 대한이비인후과학회지

Tuberculous spondylitis, also known as Pott's disease, is a disease involving the spine with progressive destruction. It most commonly involves the thoracic and lumbosacral spine and may result in severe deformity or neurologic deficit. Although cervical spine involvement is rare, it can cause life threatening event. Recently, 70-year-old man presented with progressive dyspnea and dysphagia. Physical examination and radiologic studies showed a huge retropharyngeal abscess with bony erosion and sclerotic change at the adjacent cervical spine. After incision and drainage, it was finally confirmed as a tuberculous abscess. Herein, we report our experience with literature review.

A Case of Cervical Pott's Disease Presenting as Huge Retropharyngeal Abscess / 대한이비인후과학회지

Tuberculous spondylitis, also known as Pott's disease, is a disease involving the spine with progressive destruction. It most commonly involves the thoracic and lumbosacral spine and may result in severe deformity or neurologic deficit. Although cervical spine involvement is rare, it can cause life threatening event. Recently, 70-year-old man presented with progressive dyspnea and dysphagia. Physical examination and radiologic studies showed a huge retropharyngeal abscess with bony erosion and sclerotic change at the adjacent cervical spine. After incision and drainage, it was finally confirmed as a tuberculous abscess. Herein, we report our experience with literature review.

A Rare Duodenal Subepithelial Tumor: Duodenal Schwannoma

Schwannomas are uncommon neoplasms that arise from Schwann cells of the neural sheath. Gastrointestinal schwannomas are rare among mesenchymal tumors of the gastrointestinal tract, and only a few cases have been reported to date. Duodenal schwannomas are usually discovered incidentally and achieving a preoperative diagnosis is difficult. Schwannomas can be distinguished from other subepithelial tumors on endoscopic ultrasonography; however, any typical endosonographic features of duodenal schwannomas have not been reported due to the rarity of these tumors. Immunohistochemistry is essential to distinguish schwannomas from gastrointestinal stromal tumors and leiomyomas. We report a case of duodenal schwannoma found incidentally during a health check-up endoscopy. On endoscopic ultrasonography, this tumor was suspected as a gastrointestinal stromal tumor; therefore, the patient underwent laparoscopic wedge resection of the tumor. Histopathology and immunohistochemistry confirmed that the duodenal lesion was a benign schwannoma.

Developing a Basic Scale for Workers' Psychological Burden from the Perspective of Occupational Safety and Health

BACKGROUND: Organizations are pursing complex and diverse aims to generate higher profits. Many workers experience high work intensity such as workload and work pressure in this organizational environment. Especially, psychological burden is a commonly used term in workplace of Republic of Korea. This study focused on defining the psychological burden from the perspective of occupational safety and health and tried to develop a scale for psychological burden. METHODS: The 48 preliminary questionnaire items for psychological burden were prepared by a focus group interview with 16 workers through the Copenhagen Psychosocial Questionnaire II and Mindful Awareness Attention Scale. The preliminary items were surveyed with 572 workers, and exploratory factor analysis, confirmatory factor analysis, and correlation analysis were conducted for a new scale. RESULTS: As a result of the exploratory factor analysis, five factors were extracted: organizational activity, human error, safety and health workload, work attitude, and negative self-management. These factors had significant correlations and reliability, and the stability of the model for validity was confirmed using confirmatory factor analysis. CONCLUSION: The developed scale for psychological burden can measure workers' psychological burden in relation to safety and health. Despite some limitations, this study has applicability in the workplace, given the relatively small-sized questionnaire.

Case of an Inflammatory Myofibroblastic Tumor of the Duodenum / 대한소화기학회지

An inflammatory myofibroblastic tumor (IMT) is a rare disease that can occur in a variety of locations, including the lung, orbit, parotid, pleura, and stomach. Despite multiple reports in various organs, a duodenal IMT is rare with limited case reports. We encountered a case of a 49-year-old male with a duodenal IMT. The patient underwent a laparoscopic wedge resection under the impression of a duodenal mesenchymal tumor, such as gastrointestinal stromal tumor, but the final diagnosis was a duodenal IMT. The patient was treated successfully with an oral nonsteroidal anti-inflammatory drug for the residual lesions. He was free of recurrence during the 12 month follow-up period.

Intraductal Papillary Mucinous Neoplasms of the Bile Duct Treated with Argon Plasma Coagulation / 대한췌담도학회지

Intraductal papillary mucinous neoplasm of the bile duct (IPMN-B) is frequently found and has recently been recognized as a precancerous lesion leading to invasive carcinoma characterized by papillary growth of the ductal epithelium with rich mucin production and cystic dilatation of the hepatic duct. Surgical resection is the treatment of choice to avoid malignant transformation. Despite a growing number of studies on IPMN-B, there are few reports of its treatment aside from surgery. A 75-year-old woman was incidentally diagnosed as IPMN-B. Considering the patient's age, comorbidity, and preference, we recommended an argon plasma coagulation (APC) as local ablation therapy rather than surgical resection. There was no evidence of remnant tumor on percutaneous transhepatic cholangioscopy 4 weeks after the ablation of tumor mass by using APC. We report a rare case of IPMN-B successfully treated with APC.

Intraductal Papillary Mucinous Neoplasms of the Bile Duct Treated with Argon Plasma Coagulation / 대한췌담도학회지

Intraductal papillary mucinous neoplasm of the bile duct (IPMN-B) is frequently found and has recently been recognized as a precancerous lesion leading to invasive carcinoma characterized by papillary growth of the ductal epithelium with rich mucin production and cystic dilatation of the hepatic duct. Surgical resection is the treatment of choice to avoid malignant transformation. Despite a growing number of studies on IPMN-B, there are few reports of its treatment aside from surgery. A 75-year-old woman was incidentally diagnosed as IPMN-B. Considering the patient's age, comorbidity, and preference, we recommended an argon plasma coagulation (APC) as local ablation therapy rather than surgical resection. There was no evidence of remnant tumor on percutaneous transhepatic cholangioscopy 4 weeks after the ablation of tumor mass by using APC. We report a rare case of IPMN-B successfully treated with APC.

Polymorphisms of the Reg1alpha Gene and Early Onset Type 2 Diabetes in the Korean Population / 당뇨병

BACKGROUND: The Reg gene has been reported to be expressed in regenerating islets and Reg1 protein to be up-regulated at an early stage of diabetes in mice. As human Reg1alpha is homologous with murine Reg1, we investigated whether common variants in Reg1alpha are associated with type 2 diabetes in the Korean population. METHODS: We sequenced the Reg1alpha gene to identify common polymorphisms using 24 Korean DNA samples. Of 11 polymorphisms found, five common ones (g.-385T>C [rs10165462], g.-36T>G [rs25689789], g.209G>T [rs2070707], g.1385C>G [novel], and g.2199G>A [novel]) were genotyped in 752 type 2 diabetic patients and 642 non-diabetic subjects. RESULTS: No polymorphism was associated with the risk of type 2 diabetes. However, g.-385C and g.2199A lowered the risk of early-onset type 2 diabetes, defined as a diagnosis in subjects whose age at diagnosis was 25 years or more but less than 40 years (odds ratio [OR], 0.721 [0.535 to 0.971] and 0.731 [0.546 to 0.977] for g.-385C and g.2199A, respectively) and g.1385G increased the risk of early-onset diabetes (OR, 1.398 [1.055 to 1.854]). Although adjusting for errors in multiple hypotheses-testing showed no statistically significant association between the three individual polymorphisms and early-onset diabetes, the haplotype H1, composed of g.-385C, g.1385C, and g.2199A, was associated with a reduced risk of early-onset diabetes (OR, 0.590 [0.396 to 0.877], P = 0.009). CONCLUSION: Polymorphisms in the Reg1alpha were not found to be associated with overall susceptibility to type 2 diabetes, though some showed modest associations with early-onset type 2 diabetes in the Korean population.

Effect of enamel matrix derivative (EMD, Emdogain(R)) on the differentiation of cultured human periodontal ligament cells and mesenchymal stem cells

Effect of Adipose Differentiation-Related Protein (ADRP) on Glucose Uptake of Skeletal Muscle / 당뇨병

BACKGROUND: Skeletal muscle is the most important tissue contributing to insulin resistance. Several studies have shown that accumulation of intramyocellular lipid is associated with the development of insulin resistance. Thus, proteins involved in lipid transport, storage and metabolism might also be involved in insulin action in skeletal muscle. Adipose differentiation-related protein (ADRP), which is localized at the surface of lipid droplets, is known to be regulated by peroxisome proliferator activated receptor gamma (PPARgamma). However, it is not known whether ADRP plays a role in regulating glucose uptake and insulin action in skeletal muscle. METHODS: ADRP expression in skeletal muscle was measured by RT-PCR and western blot in db/db mice with and without PPARgamma agonist. The effect of PPARgamma agonist or high lipid concentration (0.4% intralipos) on ADRP expression was also obtained in cultured human skeletal muscle cells. Glucose uptake was measured when ADRP was down-regulated with siRNA or when ADRP was overexpressed with adenovirus. RESULTS: ADRP expression increased in the skeletal muscle of db/db mice in comparison with normal controls and tended to increase with the treatment of PPARgamma agonist. In cultured human skeletal muscle cells, the treatment of PPARgamma agonist or high lipid concentration increased ADRP expression. siADRP treatment decreased both basal and insulin-stimulated glucose uptake whereas ADRP overexpression increased glucose uptake in cultured human skeletal muscle cells. CONCLUSION: ADRP expression in skeletal muscle is increased by PPARgamma agonist or exposure to high lipid concentration. In these conditions, increased ADRP contributed to increase glucose uptake. These results suggest that insulin-sensitizing effects of PPARgamma are at least partially achieved by the increase of ADRP expression, and ADRP has a protective effect against intramyocellular lipid-induced insulin resistance.

Hereditary Hemolytic Anemia in Korea: a Retrospective Study from 1997 to 2006

BACKGROUND: The aim of this study was to investigate the prevalence, clinical and laboratory findings of hereditary hemolytic anemia (HHA) in Korea from 1997 to 2006 and to develop the appropriate diagnostic approach for HHA. METHODS: By the use of questionnaires, information on the clinical and laboratory findings ofHHA diagnosed from 1997 to 2006 in Korea was collected and analyzed retrospectively. A total of 431 cases were enrolled in this study from 46 departments of 35 hospitals. RESULTS: The overall frequency of HHA did not change through the 10-year period for pediatrics but did show an increasing tendency for internal medicine. The overall male to female sex ratio did not show sex predominance (1.17:1), but a significant male predominance with a ratio of 1.49:1 was seen for pediatrics while a significant female predominance with a ratio of 1:1.97 was seen forinternal medicine. Of the total cases, 74.2% (282/431) were diagnosed before the age of 15 years. The etiologies of HHA were classified as red cell membrane defects, hemoglobinopathies, red cell enzyme deficiencies and unknown causes. There were 382 cases (88.6%) of red cell membrane defects with 376 cases (87.2%) of hereditary spherocytosis and 6 cases (1.4%) of hereditary elliptocytosis, 20 cases (4.6%) of hemoglobinopathies with 18 cases (4.2%) of beta-thalassemia, a case (0.2%) of alpha-thalassemia and a case (0.2%) of Hemoglobin Madrid, 7 cases (1.6%) of red cell enzyme deficiencies with 5 cases (1.2%) of glucose-6- phosphate dehydrogenase (G-6-PD) deficiency, a case (0.2%) of pyruvate kinase (PK) deficiency and a case (0.2%) of enolase deficiency, and 22 cases (5.1%) of unknown causes. The most common chief complaint in pediatric patients was pallor and that in adult patients was jaundice. In the red cell membrane defect group of patients, the level of hemoglobin was significantly higher than in adult patients. The mean corpuscular volume, mean corpuscular hemoglobin, corrected reticulocyte count, total and indirect bilirubin level and lactate dehydrogenase levels in the hemoglobinopathy group of patients were significantly lower than the values in the red cell membrane defect group of patients. The mean concentration of G-6-PD was 0.8+/-0.7U/1012RBC in the G-6-PD deficient patients, PK was 1.7U/1010 RBC in the PK deficient patient, and the level of enolase was 0.04U/g of Hb in the enolase deficient patient. CONCLUSION: The most prevalent cause of HHA in Korea during 1997 to 2006 was hereditary spherocytosis, but HHA by other causes such as hemoglobinopathy and red cell enzyme deficiency gradually increased with the development of molecular diagnostic methods and increasing general interest. However, the etiologies of HHA need to be pursued further in 5.1% of the patients. An systematic standard diagnostic approach is needed in a nationwide prospective study for correct diagnoses and appropriate management of HHA.

Current Status of Hematopoietic Stem Cell Transplantation in Korean Children

BACKGROUND: Hematopoietic stem cell transplantation (HSCT) is one of the most important armamentarium against various hematologic malignancies or some solid tumors. We investigated the number of patients who might need transplants and compared with that of actual transplants to conceptualize current status and circumstances of HSCTs in Korean children. METHODS: Questionnaires were sent to Korean Society of Hematopoietic Stem Cell Transplantation (KSHSCT) members who were taking care of children with malignancies or hematologic diseases. Almost all of the newly diagnosed patients between Jan, 1st and Dec, 31st, 2003 were enrolled in the study. RESULTS: Seven hundred forty eight children (male to female ratio = 1.4:1) were enrolled. The median age was 6.1 years old (8 days~28.8 years old). Malignant diseases consisted of 695 cases (92.9%), and among them almost half were hematologic malignancies. The participating members speculated that HSCTs should be indicated in 285 children (38.1%) which included 209 allogeneic, and 76 autologous transplants. In reality, however, allogeneic HSCTs were performed only in 140 children (67.0%) with the median interval of 5.9 month, and autologous transplants in 44 children (57.9%) with 8.3 month. In autologous setting, all the patients received peripheral blood stem cells (PBSCs), whereas bone marrow (61%), cord blood (34%), and PBSC (5%) were used in allogeneic HSCTs. Donor types were as follows: unrelated donor (37%), cord blood (34%), sibling donor (25%), and family (4%). The reasons for not performing HSCTs were unfavorable disease status or death, no availability of suitable donor, economical situation, and refusal by parental preferences. Under the strict insurance regulations, many transplants were not covered by insurance. More autologous transplants were performed without insurance coverage than allogeneic HSCTs (P=0.013). Those cases were advanced cases and HLA mismatch transplants for allogeneic setting, and relatively rare diseases still awaiting favorable results of transplants for autologous setting. CONCLUSION: HSCTs are essential part of treatment strategies for children with various diseases. Unfortunately, however, a third of patients who were in need of transplants did not receive HSCTs due to various reasons. It is necessary to expand unrelated donor pool or cord blood banks for the cases lacking HLA-identical sibling donors. Also medical insurances should cover HSCTs for rare diseases as well as for less favorable but novel situations where there are no suitable alternatives.

A Case of Pure Red Cell Anemia Complicated by Antiepileptic Drugs

A 12-year-old boy developed pure red cell anemia(PRCA) during a combination therapy of antiepileptic drugs(AEDs) for epilepsy. His complex partial seizure was intractable to monotherapy. During 7 months of treatment, he was treated with Vigabatrin, Carbamazepine and Valproate. While switching from Carbamazepine to Valproate, he presented anemia but with no jaundice. His hemoglobin was 4.1g/dl bone-marrow biopsy revealed erythroid hypoplasia with normal myelopoiesis and megakaryocytopoiesis, indicating PRCA. Rapid recovery from PRCA was observed 1 month after discontinuation of Valproate, without immunosuppressive therapy. Although the hematologic toxicity of AEDs is well documented, isolated cessation of red cell production is uncommon. Our observation suggests that the synergistic toxicity caused by Valproate and Carbamazepine may induce PRCA through the inhibitory effect beyond the differentiation stage of BFU-E and CFU-E.

Statistical Study on Congenital Anomalies

PURPOSE: Congenital anomalies are known as a major cause of neonatal death with prematurity and birth injuries. We surveyed the incidence of congenital anomalies among livebirths and stillbirths delivered at our hospital, and detected in neonatal and postneonatal period. METHODS: The statistical study was done about congenital anomalies among 9,569 deliveries (9,438 livebirths and 131 stillbirths) at Ulsan University Hospital during the periods of 5 years from Jan 1992 to Dec 1996. We reviewed delivery and newborn record, OPD chart, admission chart retrospectively. We investigated the incidence of congenital anomaly according to each year, period, outcome of delivery, sex, birth weight, gestational age, maternal age, Apgar score, multiple pregnancy, and each system. RESULTS: 1) The overall incidence of congenital anomalies was 5.0% (481 cases) among 9,569 deliveries. The annual incidence was 4.5% in 1992, 5.1% in 1993, 5.2% in 1994, 5.8% in 1995, 4.7% in 1996. 2) The incidence of congenital anomalies detected in neonatal period was 3.5% (333 cases) and 1.5% (148 cases) in postneonatal periods. 3) The incidence of congenital anomalies was 4.9% among live births and 9.2% among stillbirths. 4) The incidence of congenital anomalies was 5.3% in male and 4.7% in female. 5) The incidence of congenital anomalies among the babies, below 2,500g of birth weight was 7.7%, between 2,500g and 3,999g was 4.7%, and over 4,000g was 6.8N. 6) The incidence of congenital anomalies among the babies, below 32 weeks of gestational age was 12.9%, between 32 weeks and 36 weeks was 7.4%, between 37 weeks and 41 weeks was 4.7%, and over 42 weeks was 5.1%. 7) The babies born to mothers younger than 20 years of age have anomalies in 7.1%, mother between 20 years of age and 35 years of age in 5.0%, and older than 35 years of age in 6.5%. 8) The incidence of congenital anomalies according to Apgar score among live births between 0 and 3 was 9.8%, between 4 and 6 was 6.0%, and between 7 and 10 was 5.0%. 9) The incidence of congenital anomalies according to multiple pregnancy was 8.6%. 10)The systems of congenital anomalies in order of incidence were as follows; The digestive system (25.6%), the cardiovascular system (22.1%), the genitourinary system (18.9%), the musculoskeletal system (15.5%), the skin and soft tissue (11.7%), the central nervous system (1.6%), the respiratory system (l%), the others (3.6%). CONCLUSIONS: Regular examination and screening on congenital anomaly is required from newborn period to infant and childhood. In view of these considerations, the incidence of congenital anomaly may be increased more than the past reports because of development of recent diagnostic techniques.