We experienced a case of eyelid neurofibroma in von Recklinghausen's disease, which had a typical sign, cafe-au-lait spots on generalized skin. The patient was a five year old girl who had no family history, and whose mentality was subnormal. The right eyelid, in which the neurofibroma involved, was thickened, and was ptotic. The surgical correction of the right eyelid ptosis was not satisfied.
Blepharoptosis ; Cafe-au-Lait Spots ; Eyelids* ; Female ; Humans ; Neurofibroma* ; Neurofibromatosis 1 ; Skin

Tetrasomy for the short arm of chromosome 12 (Pallister-Killian syndrome) is an uncommon mosaic aneuploidy, which may present in the prenatal period with an ultrasonographically detected fetal abnormalities or following karyotyping for maternal age and other causes. In this syndrome the chromosome abnormalities, isochromosome is present in amniocyte with a much greater percentage than fetal lymphocyte. The most consistent reported prenatal ultrasound findings for tetrasomy 12p include polyhydramnios with short femurs and a diaphragmatic hernia. We report a case identified by prenatal karyotyping diagnosis.
Aneuploidy ; Arm ; Chromosome Aberrations ; Chromosomes, Human, Pair 12 ; Diagnosis ; Femur ; Hernia, Diaphragmatic ; Isochromosomes ; Karyotyping ; Lymphocytes ; Maternal Age ; Mosaicism ; Polyhydramnios ; Tetrasomy* ; Ultrasonography

Terlipressin has splanchnic vasoconstrictive effects, and is generally used for the management of gastroesophageal variceal bleeding secondary to liver cirrhosis. Terlipressin is a synthetic arginine vasopressin (AVP) analog containing a nonapeptide sequence. Terlipressin has increased selectivity for the V1 receptor, compared with AVP; hence, it is considered to be a safe vasoconstrictor. However, side effects such as hyponatremia and seizure, although very rare, have been reported. Hyponatremia related to terlipressin may be caused by the syndrome of inappropriate antidiuresis (SIAD), which is a disorder of sodium and water balance characterized by hypotonic hyponatremia without elevation of the antidiuretic hormone level. Here, we report a case of hyponatremic seizure induced by an infusion of terlipressin in a 52-year-old female who had isolated gastric variceal bleeding secondary to alcoholic liver cirrhosis.
Arginine Vasopressin ; Esophageal and Gastric Varices ; Female ; Hemorrhage* ; Humans ; Hyponatremia ; Liver Cirrhosis* ; Liver Cirrhosis, Alcoholic ; Middle Aged ; Receptors, Vasopressin ; Seizures* ; Sodium

BACKGROUND AND OBJECTIVES: Patients on oral anticoagulation therapy (OAT) require regular monitoring of the prothrombin time (PT) and dosage adjustment to prevent thromboembolic diseases without the risk of hemorrhage. Portable self PT monitors have been recently developed because the standard PT measurements are complicated and take considerable time. This study compared the International normalized ratio (INR) results that were obtained with using the CoaguChek XS device (Roche Diagnostic, Mannheim, Germany) with those obtained using a standard laboratory method Sysmex CA-1500 (Sysmex Corporation, Kobe, Japan) in the patients on OAT and also a healthy control group. SUBJECTS AND METHODS: 100 outpatients on OAT and 20 healthy controls were enrolled on a volunteer basis after providing informed consent at the Dong-A University Hospital. The outpatients and the healthy control group provided us the INR measurements with using both the CoaguChek XS and the Sysmex CA-1500. RESULTS: The coefficients of variation for CoaguChek XS and Sysmex CA-1500 were less than 10%. The PT (INR) results of CoaguChek XS and Sysmex CA-1500 were 2.0+/-0.7 and 2.2+/-0.7, respectively (p<0.001). There was a good correlation between CoaguChek XS and Sysmex CA-1500 (r=0.974, p<0.001). On the regression analysis, the slope of the regression line was 0.9197 and the y-intercept was 0.0058. On the Bland-Altman analysis, the INR mean difference (bias) between the two methods (CoaguChek XS INR-Sysmex CA-1500 INR) was -0.2 and the limit of agreement was +0.168~-0.568. CONCLUSION: The measurement with using CoaguChek XS has high repeatability, rapid availability and good accuracy that are comparable to the standard laboratory method. Therefore, CoaguChek XS can be a valuable tool for the self-monitoring of patients on OAT.
Anticoagulants ; Avena ; Hemorrhage ; Humans ; Informed Consent ; International Normalized Ratio ; Outpatients* ; Prothrombin Time* ; Prothrombin* ; Volunteers

Mucormycosis is an extremely rare but potentially life-threatening fungal infection. Mucormycosis of the gastrointestinal tract manifests with features similar to ischemic colitis. A 48-year-old man with end-stage renal disease due to diabetic nephropathy underwent deceased donor kidney transplantation. He complained of abdominal pain and distension on postoperative day 17. A computed tomography (CT) scan revealed symmetrical wall thickening of the ascending colon, which was consistent with ischemic colitis. However, a follow-up CT scan showed a localized wall-off colon perforation in the hepatic flexure and segmental mural gas in the ascending colon. Microscopic examination obtained from a surgical specimen demonstrated numerous fungal hyphae and spores in the mucosa and submucosa. A total colectomy was performed, but the patient died 36 days later due to multiple organ failure, despite antifungal agents. Clinicians should be informed about fungal infection, such as colonic mucormycosis mimicking ischemic colitis, in kidney transplant patients with diabetes mellitus, and treatment should be initiated at the earliest.
Abdominal Pain ; Antifungal Agents ; Colectomy ; Colitis, Ischemic* ; Colon* ; Colon, Ascending ; Diabetes Mellitus ; Diabetic Nephropathies ; Follow-Up Studies ; Gastrointestinal Tract ; Humans ; Hyphae ; Kidney Failure, Chronic ; Kidney Transplantation ; Kidney* ; Middle Aged ; Mucormycosis* ; Mucous Membrane ; Multiple Organ Failure ; Spores ; Tissue Donors ; Tomography, X-Ray Computed ; Transplant Recipients*

BACKGROUND/AIMS: The incidence of variant angina (VA) is relatively high in Korea compared with western countries, but its long-term clinical outcomes are not well defined. METHODS: Patients who underwent ergonovine provocation tests at the cardiac catheterization laboratory of Chonnam National University Hospital between 1996 and 2011 were enrolled in this study (n = 1162). Of them, 686 patients with positive ergonovine provocation tests were divided into two groups: patients with cardiac events (Group I: 153 patients, 52.4 +/- 11.0 years, M: F = 103: 50) and those without (Group II: 533 patients, 51.6 +/- 10.7 years, M: F = 350: 183). The mean follow-up duration was 40.2 +/- 38.0 months. Cardiac events were defined as cardiac death, recurrent ischemia, rehospitalization, myocardial infarction, and follow-up angiography. Clinical findings, laboratory and coronary angiographic characteristics were compared between the groups. RESULTS: A history of smoking was more common in Group I than in Group II (45.8% vs. 36.3%, p = 0.037). The levels of low-density lipoprotein cholesterol (119.4 +/- 35.3 vs. 111.1 +/- 32.2 mg/dL, p = 0.010) were higher in Group I than in Group II. According to Cox proportional hazard regression analysis, the major predictive factor for cardiac events during clinical follow-up was smoking (HR 1.80, 95% CI 1.036-3.126, p = 0.037). CONCLUSIONS: A history of smoking was the only independent risk factor for cardiac events during a long-term clinical follow-up in Korean patients with variant angina.
Angina Pectoris, Variant ; Angiography ; Cardiac Catheterization ; Cardiac Catheters ; Cholesterol ; Coronary Artery Disease ; Death ; Ergonovine ; Follow-Up Studies ; Humans ; Incidence ; Ischemia ; Korea ; Lipoproteins ; Myocardial Infarction ; Risk Factors ; Smoke ; Smoking

BACKGROUND: THe Korean Society for Nosocomial Infection Control (KOSNIC) orfanized the Korean Nosocomial Infections Surveillance System (KONIS) to establish a nationwide database of Nosocomial infection (NI) rate in the intensive care units (ICUs) of Korean hospitals. This report is a summary of the data from July through September 2006. METHODS: The KONIS performed a prospective sruveillance for nosocomial urinary tract infections (UTI), bloodstream infections (BSI), and pneumonia (PNEU) at 76 ICUs in 44 hospitals. NI rates were calculated as the numbers of infections per 1,000 patient-days or device-days. RESULTS: A total of 846 nosocomial infections were fOlllld during the study period: 407 UTIs (397 cases were urinary catheter-associated), 204 BSIs (182 were central line-associated), and 235 PNEUs (161 were ventilator-associated). The rate of urinary catheter-associated UTIs was 4.61 cases per 1,000 device-days and urinary catheter utilization ratio was 0.83. The rate of central line-associated BSIs was 3.16 and the utilization ratio was 0.55. The rate of ventilator-associated PNEUs was 3.80 and the utilization ratio was 0.41. Although the ventilator utilization ratio was lower in the hospitals with 400-699 beds than in the hospitals with more than 900 beds, the rate of ventilator-associated pneumonia was higher in the smaller hospitals than in the larger ones. The rates of all three device-associated infections were the highest in the neurosurgical ICUs and the rates were the lowest in the surgical ICUs. CONCLUSION: This study may contribute to the development of effective strategies for NI control according to the size of hospital and the type of ICUs.
Cross Infection* ; Intensive Care Units ; Pneumonia ; Pneumonia, Ventilator-Associated ; Prospective Studies ; Urinary Catheters ; Urinary Tract Infections ; Ventilators, Mechanical

BACKGROUND AND OBJECTIVES: Acute myocardial infarction (AMI) at a young age less than 40 years is an uncommon condition and it is characterized by multiple cardiovascular risk factors. We analyzed the risk factors of restenosis in AMI patients who underwent percutaneous coronary intervention (PCI), and these patients were under the age of 40 years. SUBJECTS AND METHODS: Between January 1997 and December 2006, 88 out of the 121 young AMI patients (mean age: 35.6+/-4.0 years, 115 males) who underwent follow-up coronary angiography after PCI were divided into two groups: the patients without restenosis (group I: n=62, mean age: 35.6+/-3.9 years, 60 males) and the patients with restenosis (group II: n=26, mean age: 36.3+/-3.8 years, 23 males). The clinical and coronary angiographic characteristics were compared between the two groups. RESULTS: Smoking (79.3%) was the most common risk factor in all the patients. The baseline clinical characteristics and baseline laboratory findings were not different between the two groups. There was no significant difference in the sex ratio (p=0.124). The Thrombolysis In Myocardial Infarction (TIMI) flows were not different between the two groups. The level of homocysteine (hcy) was significantly decreased from 12.4+/-8.8 micronmol/L to 9.3+/-3.8 micronmol/L in group I (p=0.011), but this was not changed significantly in group II (p=0.062). According to multiple logistic regression analysis, a high triglyceride level (>200 mg/dL) was an independent predictor of restenosis (p=0.046). CONCLUSION: A high level of serum triglyceride is a predictive factor of restenosis after PCI in young age patients with acute myocardial infarction.
Coronary Angiography ; Coronary Restenosis ; Follow-Up Studies ; Homocysteine ; Humans ; Logistic Models ; Myocardial Infarction* ; Percutaneous Coronary Intervention* ; Prognosis ; Risk Factors ; Sex Ratio ; Smoke ; Smoking ; Triglycerides