No abstract available.
Dermatomyositis*

The 4p deletion syndrome, also known as Wolf-Hirschhorn syndrome, is a well-known genetic disorder caused by a partial deletion of the short arm of chromosome 4. The great variability in the extent of the 4p deletion and the possible contribution of additional genetic rearrangements leads to a wide spectrum of clinical manifestations. Herein, we present our experience with eight cases of 4p deletion syndrome, ascertained prenatally between 1998 and 2016 at our hospital.
Arm ; Chromosomes, Human, Pair 4 ; Prenatal Diagnosis* ; Wolf-Hirschhorn Syndrome*

Kawasaki disease is an acute vasculitis of unknown causes that occurs predominantly in infants and young children and produces coronary artery aneurysm. We have recently experienced a case of persistent Kawasaki disease in a 24 month-old-girl improved with pulsed doses of methylprednisolone. Even with an administration of intravenous gamma globulin(IVIG), she repeated the course of recovery and aggravation. After four times repeated doses of IVIG, additional intravenous methylprednisolone pulse therapy was tried and resulted in remarkable improvement. We reported the case with a brief review of the related literature.
Aneurysm ; Child ; Coronary Vessels ; Humans ; Immunoglobulins, Intravenous ; Infant ; Methylprednisolone* ; Mucocutaneous Lymph Node Syndrome* ; Vasculitis

PURPOSE: Pertussis is a highly communicable infectious disease in children with high mortality, especially in young infants. The incidence of pertussis in South Korea has decreased to about 10 cases a year in late '90s. Doubting previously reported incidences of pertussis, we designed this study to establish exact epidemiology and a diagnostic basis of pertussis. METHODS: From Mar. 2000 to Mar. 2001, polymerase chain reaction(PCR) and cultures of nasopharyngeal aspirates were taken from 49 patients who were clinically suspected of pertussis in Kangdong Sacred Heart Hospital. RESULTS:Among 49 patients suspected of pertussis, 10 cases showed positive results by PCR method. Four out of those 10 cases were positive in culture. The peak outbreak was noticed in the spring(Mar.-May) and the autumn(Sep.-Nov.). The mean age of the patients was 3.6 months. Eight of the 10 cases which were PCR positive results proved not to be immunized against pertussis. CONCLUSION: Most of the pertussis patients in our study were diagnosed as bronchiolitis, pneumonia or bronchitis at the beginning of the disease and only a few patients showed typical clinical manifestations of pertussis, including whooping. When the above results are taken together, we suggest the possibility that the actual prevalence of pertussis in South Korea might be higher than that of previous reports. The importance of pertussis screen tests should be emphasized in children with severe coughs.
Bronchiolitis ; Bronchitis ; Child* ; Communicable Diseases ; Cough ; Epidemiology ; Heart ; Humans ; Incidence ; Infant ; Korea ; Mortality ; Pneumonia ; Polymerase Chain Reaction ; Prevalence ; Whooping Cough*

Calcium pyrophosphate dihydrate (CPPD)crystal deposition disease is one of the most common causes of arthralgia in elderly.The acute form (pseudogout) may present as acute monoarticular or polyarticular arthritis.It is often accompanied by high fever and sometimes simulates acute infectious condition.The knee is the joint most frequently affected by pseudogout but other sites such as wrist,shoulder,ankle,elbow and hands may be affected.A few cases involving cervical and lumbar spine mistaken for CNS infection have also been described in foreign countries.We report here a case of CPPD crystal deposition disease mimicking meningitis.
Arthralgia ; Calcium Pyrophosphate* ; Calcium* ; Chondrocalcinosis ; Fever ; Hand ; Joints ; Knee ; Meningitis ; Spine

PURPOSE: The purpose of this study is to identify the epidemiologic and clinical characteristics of respiratory adenovirus infections in children, and to investigate the difference in the clinical features between single adenovirus infection and coinfection with adenovirus and other respiratory viruses. METHODS: A retrospective study was performed in 470 children hospitalized with respiratory adenovirus infections in Gwangmyeong Sungae Hospital between January 2013 and December 2013. RESULTS: The mean age of the patients was 46.2 months and the peak incidence was in the 12- to 24-month age group. The mean duration of hospitalization and fever were 4.5+/-1.1 and 4.5+/-9.2 days, respectively. Seasonally it had occurred throughout the year, but showed the highest prevalence in August and high prevalence in July, September, and October. The frequency of viral coinfection with other respiratory viruses was 39.6%. The age was significantly younger in coinfection group than in the single adenovirus infection group (P<0.001). The prevalence rates of bronchiolitis (P<0.001) and pneumonia (P=0.042) were significantly higher in the respiratory syncytial virus coinfection group. The coinfection rate was significantly higher in children aged less than 2 years (P<0.001), and the prevalence rates of bronchiolitis (P<0.001) and pneumonia (P<0.001) were also higher in the group aged less than 2 years than other age groups. CONCLUSION: Adenovirus is an important viral agent in hospitalized children with acute respiratory tract infection. Lower respiratory tract infections, such as bronchiolitis and pneumonia, and coinfection with other respiratory viruses were more frequently occurred in patients under 2 years of age. Further studies are needed to clarify whether coinfection with other respiratory viruses would increase the rate of lower respiratory tract infections in patients with respiratory adenoviral infections.
Adenoviridae Infections* ; Adenoviridae* ; Bronchiolitis ; Child ; Child, Hospitalized ; Coinfection ; Fever ; Hospitalization ; Humans ; Incidence ; Inpatients* ; Pneumonia ; Prevalence ; Respiratory Syncytial Viruses ; Respiratory Tract Infections ; Retrospective Studies ; Seasons

PURPOSE : To evaluate the compliance of patients who underwent complete resection of non-small cell lung cancer (NSCLC) with adjuvant chemotherapy. MATERIALS AND METHODS : Between January 2004 and May 2006, patients who underwent a complete resection for NSCLC were referred to oncologists for adjuvant chemotherapy. Three or 4 cycles of platinum-based adjuvant chemotherapy was then performed according to the protocol or the preference of the oncologists. RESULTS : Two hundred and thirty-two patients were enrolled in this study. The median age of the study group was 60.9 years and 76.7 % of the patients enrolled were male. 34.9%, 28.8% and 36.2% of the patients were in stage IB, II and III respectively. In addition, 142 of the patients (61.2%) completed all planned cycles, whereas 65 patients (28%) received no therapy. The causes of start failure for adjuvant chemotherapy consisted of decreased postoperative performance status (n=39), refusal (n=13) and distant metastasis at the initial follow-up (n=2). The causes of cessation during adjuvant chemotherapy included the occurrence of severe adverse effects (n=12), aggravation of the disease with newly developed metastasis (n=4) and others (n=6). The mortality related to the adjuvant chemotherapy was 1.3 % (n=3), all of the fatalities were due to pneumonia and sepsis. Univariate analysis showed that age, postoperative complications and pathologic staging were the significant factors that determined whether the adjuvant chemotherapy was completed. Multivariate analysis demonstrated statistically significant differences in compliance when age and pathologic staging were considered. CONCLUSION : Adjuvant chemotherapy for completely resected NSCLC was performed with satisfactory compliance in approximately 60% of the patients included in this study, and age plays an important role in the compliance of adjuvant chemotherapy. Elderly subsets will be examined to help determine the effect of age on compliance and outcome. In addition, the medical oncologist tended to complete the adjuvant chemotherapy for more advanced cases of lung cancer than for stage IB lung cancer
Aged ; Carcinoma, Non-Small-Cell Lung* ; Chemotherapy, Adjuvant* ; Compliance* ; Disulfiram ; Drug Therapy ; Follow-Up Studies ; Humans ; Lung Neoplasms ; Male ; Mortality ; Multivariate Analysis ; Neoplasm Metastasis ; Pneumonia ; Postoperative Complications ; Sepsis

OBJECTIVE: This study aims to investigate whether there are any notable etiologies for repeated biochemical pregnancy (RBP) and, if so, to compare those etiologies associated with repeated spontaneous abortion in infertile couples who have undergone in vitro fertilization (IVF). METHODS: Forty-four infertile couples who underwent IVF and experienced RBP were included in this study. RBP was defined as more than 2 early pregnancy losses that occurred before the detection of a gestational sac, with ectopic pregnancies specifically excluded by serial serum beta human chorionic gonadotropin evaluation. Forty-three infertile couples who underwent IVF and experienced recurrent spontaneous abortion (RSA) were included as a control group. Karyotype analysis, anatomic evaluation of uterus, endocrine and immunological evaluation were performed. In addition, the number of pregnant women confirmed by 12 weeks' gestation was compared between groups. RESULTS: Immunological factors (RSA: 20.9% vs. RBP: 29.5%, P=0.361), diminished ovarian reserve (RSA: 10.9% vs. RBP: 17%, P=0.552), and parental chromosomal abnormalities (RSA: 18.6% vs. RBP: 9.1%, P=0.218) were not different between groups. Additionally, the incidence of uterine factors (RSA: 11.6% vs. RBP: 4.6%, P=0.206), unknown cause (RSA: 48.8% vs. RBP: 54.5%, P=0.161), and the pregnancy outcome identified until 12 weeks' gestation (RSA: 46.5% vs. RBP: 38.6%, P=0.520) did not differ between groups. CONCLUSION: In the present study, the causes of RBP after IVF were similar to those of RSA. Accordingly, we suggest that efforts should be made to define the etiology of RBP, particularly for infertile couples, and that possible management strategies should be offered.
Abortion, Habitual ; Abortion, Spontaneous ; Biochemical Phenomena ; Chorionic Gonadotropin ; Chromosome Aberrations ; Family Characteristics* ; Female ; Fertilization in Vitro* ; Gestational Sac ; Humans ; Immunologic Factors ; In Vitro Techniques* ; Incidence ; Karyotype ; Ovarian Reserve ; Parents ; Pregnancy Outcome ; Pregnancy* ; Pregnancy, Ectopic ; Pregnant Women ; Uterus

OBJECTIVE: Severity of nausea and vomiting of pregnancy (NVP) is associated with adverse pregnancy outcomes and poorer quality of life (QOL). The aim of this study was to evaluate the severity of NVP and maternal well-being status using the Pregnancy-Unique Quantification of Emesis and Nausea (PUQE) scale in a Korean population. METHODS: A total of 527 pregnant women who were receiving prenatal care at 4 hospitals were asked to participate in the study between January 2015 and June 2015. The severity of NVP was evaluated by the PUQE scale and maternal well-being status was evaluated using the visual analogue scale (VAS). Statistical analyses were performed to determine the risk factors associated with NVP and the associations between the severity of NVP and QOL. RESULTS: Among the 472 eligible pregnant women, 381 (80.7%) were suffering from NVP during pregnancy. No significant differences (P>0.05) were observed in any of the variables between the 2 study groups, with the exception of smoking, alcohol consumption, and history of NVP. NVP history was found to be the most powerful risk factor (adjusted odds ratio, 11.6; 95% confidence interval, 4.7–28.7). The correlation coefficient (r) between the VAS scores of maternal well-being status and PUQE severity was −0.25 (r 2=0.062; P-< 0.001). CONCLUSION: In this study, an explicit decline in maternal well-being status was observed according to severity of NVP. The PUQE scale may be of help to clinicians, healthcare providers, and researchers because of its simplicity and usefulness as a tool for NVP evaluation.
Alcohol Drinking ; Female ; Health Personnel ; Humans ; Korea ; Nausea ; Odds Ratio ; Pregnancy Outcome ; Pregnancy ; Pregnant Women ; Prenatal Care ; Quality of Life ; Risk Factors ; Smoke ; Smoking ; Visual Analog Scale ; Vomiting

PURPOSE: To evaluate the success rate following amnioinfusion in pregnant women undergoing external cephalic version (ECV) after initial failure. METHODS: This prospective study enrolled 17 consecutive pregnant women from October 2013 to May 2015. ECV was performed with amnioinfusion after initial failure. The success rates of ECV and vaginal delivery, including pregnancy outcomes, were analyzed. RESULTS: ECV was performed at an average of 37.3±0.6 weeks of gestational age. Eight of seventeen patients were nulliparous. The estimated fetal weight was 2,688±279 g, and the amniotic fluid index was 6.4±2.6 cm. The overall success rate of ECV was 70.6% (12/17), and the success rates in nulliparous and multiparous women were 75.0% (6/8) and 66.7% (6/9), respectively. The rate of emergent cesarean section within 24 hours was 11.8% (2/17). Excluding one women who were lost to follow-up, the rate of normal vaginal delivery was 81.8% (9/11) among the women who had successful ECV. We did not observe any complications such as uterine rupture, placental abruption, or intrauterine fetal death. CONCLUSION: Although ECV with amnioinfusion after initial failure might help increase the success rate of ECV, it needs to be further evaluated in larger studies.
Abruptio Placentae ; Amniotic Fluid ; Breech Presentation ; Cesarean Section ; Female ; Fetal Death ; Fetal Weight ; Gestational Age ; Humans ; Lost to Follow-Up ; Pregnancy ; Pregnancy Outcome ; Pregnant Women* ; Prospective Studies ; Uterine Rupture ; Version, Fetal*