PURPOSE: Endoscopic variceal sclerotherapy(EVS) has been considered the mainstay therapy for bleeding esophageal varices in adults. However, recent data have shown that endoscopic variceal ligation is just as efficacious and has fewer complications than EVS. Although there are many reports concerning EVL in adults, only a few studies have been done of children. METHODS: We performed EVL in 9 children under 6 years of age(mear age, 2.5 year) with esophageal varices. Outcome was assessed with respect to survival, rebleeding, and complications. RESULTS: The causes of portal hypertension were 3 cases of congenital hepatic fibrosis, 3 cases of biliary atresia, 2 cases of portal vein thrornbosis, and one case of portal vein fibromuscular dysplasia. The age at diagnosis ranged from 10 months to 6 years. The patients underwent a mean of 2.6+/- 0.7 sessions of EVL(ranging from two to four). The numbers of bands per person were 5.6+/-1.8, and the numbers of bands per session were 2.2+/-1.0 Two complications of esophageal rebleeding were noted, and none of the cases experienced symptoms of esophageal stenosis, nor gastroesophageal reflux. CONCLUSION: EVL is safe and effective in controlling variceal hemorrhage in children with portal hypertension, regardless of etiology. The cornplication rate is low and EVL is an acceptable and perhaps preferable altemative to EVS in children with esophageal varices. But regular periodic examination for recurrence of varices after eradication should be required.
Adult ; Biliary Atresia ; Child* ; Diagnosis ; Esophageal and Gastric Varices* ; Esophageal Stenosis ; Fibromuscular Dysplasia ; Fibrosis ; Gastroesophageal Reflux ; Hemorrhage ; Humans ; Hypertension, Portal ; Ligation* ; Portal Vein ; Recurrence ; Varicose Veins

PURPOSE: Recently, a number of rapid antigen detection tests have been available to diagnose group A streptococcal pharyngotonsillitis. The purpose of this study was to determine the sensitivity, specificity and consistency of the two rapid antigen detection tests. METHODS: Among the patients who visited our clinic from November 2001 to February 2002, 61 patients who had clinical findings of pharyngeal erythema or edema, pharyngeal exudates and soft palatine petechiae were enrolled in our study. A total of 61 patients were tested with rapid antigen detection tests and throat culture. BD LINK2TM Strep A(Becton, Dickinson and Company, U.S.A.) and QuickVue(R) In-LineTM(Quidel Corporation, U.S.A.) were selected for rapid antigen detection tests. RESULTS: Of the 61 patients tested, 22 patients were confirmed as group A streptococcal pharyngotonsillitis by throat culture. The BD LINK2TM Strep A had a sensitivity of 81.8% and a specificity 89.7%. The positive and negative predictive values were 81.8% and 89.7%, respectively. The QuickVue(R) In-LineTM had a sensitivity of 77.3% and a specificity of 100%. The positive and negative predictive values were 100% and 88.6%, respectively. The kappa values of BD LINK2TM Strep A and QuickVue(R) In-LineTM were 0.72 and 0.81, respectively. CONCLUSION: In addition to high sensitivity, specificity and consistency, both kits are easy to use and simple to interpret, and therefore have the potential to be used with backup throat culture for diagnosis of acute pharyngotonsillitis.
Diagnosis* ; Edema ; Erythema ; Exudates and Transudates ; Humans ; Pharynx ; Purpura ; Sensitivity and Specificity

The purpose of this study was to investigate whether sperm selection by hyaluronic acid (HA) binding could improve fertilization rate and embryo quality in intracytoplasmic sperm injection (ICSI) cycles. Two hundred nineteen oocytes obtained from eighteen women were injected with either HA-bound (n = 107) or conventionally selected spermatozoa (n = 112) in a randomized way. All of the participants were infertile couples who had normal sperm parameters but low fertilization rate in previous in vitro fertilization (IVF) cycle (n = 5) or experienced multiple IVF failures (n = 13). Lower fertilization (75.7% vs 83.0%) and cleavage rate on day 2 (72.9% vs 83.0%) was observed in oocytes injected with HA-bound spermatozoa than the conventional group, but the difference was not significant. Significantly lower cleavage rate was observed on day 3 in HA group (56.0% vs 69.6%, P = 0.038). Blastocyst formation rate and the number of transferred embryos were similar in both groups. In multiple IVF failure patients, significantly reduced fertilization rate (71.8% vs 85.3%, P = 0.046) and cleavage rate on day 2 (70.4% vs 85.3%, P = 0.029) and day 3 (53.5% vs 77.3%, P = 0.002) were noticed in HA group. Five women achieved pregnancy continuing more than 12 weeks after transfer (27.8%). Success of ICSI was not related with the number of embryos fertilized by HA-bound spermatozoa. Application of ICSI by sperm selection using HA binding is not helpful in couples with repeated poor fertilization or implantation despite normal sperm parameters.
Adult ; Blastocyst/cytology ; Embryo Transfer ; Female ; *Fertilization in Vitro ; Humans ; Hyaluronic Acid/*pharmacology ; Infertility, Male/therapy ; Male ; Oocytes/cytology/physiology ; Pregnancy ; Pregnancy Rate ; Prospective Studies ; *Sperm Injections, Intracytoplasmic ; Spermatozoa/*drug effects/physiology

Diagnostic exome sequencing (DES) is a powerful tool to analyze the pathogenic variants leading to development delay (DD) and intellectual disability (ID). Recently, heterozygous de novo mutation of the histone acetyltransferase encoding gene KAT6B has been recognized as causing a syndrome with congenital anomalies and intellectual disability, namely Say-Barber-Biesecker-Young-Simpson (SBBYS) syndrome. Here we report a case of SBBYS syndrome in a third generation Korean family affected with a missense mutation in KAT6B, c.2292C>T p.(His767Tyr) identified by DES. This is the first confirmed familial inherited mutation of the KAT6B reported worldwide. Our case emphasizes again the importance of basic physical examination and taking a family history. Furthermore, advances in genetic diagnostic tools are becoming key to identifying the etiology of DD and ID. This allows a physiatrist to predict the disease's clinical evolution with relative certainty, and offer an appropriate rehabilitation plan for patients.
Exome* ; Family Characteristics* ; Histone Acetyltransferases ; Humans ; Intellectual Disability ; Mutation, Missense ; Physical Examination ; Rehabilitation

BACKGROUND: The laryngeal mask airway Proseal (LMA-Proseal) is a renewed airway device with a modified cuff to improve the mask seal around the larynx. The classic LMA is known to have an incomplete mask seal during high positive pressure ventilation. Therefore, we examined whether the ventilation via a LMA-Proseal during laparoscopic surgery may be adequate and also observed the complications associated with a LMA-Proseal. METHODS: Thirty four patients undergoing elective gynecological laparoscopies were studied. After the insertion of a LMA-Proseal, we measured the peak airway pressure, plateau airway pressure, oropharyngeal leakage pressure, and the leakage around the neck before and after intra-abdominal CO2 insufflation. After extubation, we observed the complications, including the gastroesophageal regurgitation using a methylene blue staining method. RESULTS: The peak and plateau airway pressures after pneumoperitoneum increased significantly (P<0.05), compared with those before insufflation. However, the oropharyngeal leakage pressure was not changed and leakages around the neck were not observed. After extubation, a cuff stained with methylene blue occurred in 10 of 34 patients. Sore throat and dysphagia occurred in 8 and 4 of 34 patients, respectively. CONCLUSIONS: A LMA-Proseal during laparoscopic surgery permits adequate airway pressure, but does not protect the gastroesophageal regurgitation.
Deglutition Disorders ; Humans ; Insufflation ; Laparoscopy* ; Laryngeal Masks* ; Larynx ; Masks ; Methylene Blue ; Neck ; Pharyngitis ; Pneumoperitoneum ; Positive-Pressure Respiration ; Ventilation

We encountered a case of a rupture of an endotracheal tube cuff during robot-assisted thyroid surgery in a 35-year-old male patient. Two hours after commencing surgery, the bellows of the ventilator were not filled and a rupture of the endotracheal tube cuff was suspected. Once the robot-manipulator is engaged, the position of the operating table cannot be altered without removing it from the patient. Reintubation with direct laryngoscopy was performed with difficulty in the narrow space between the patient's head and robot-manipulator without moving the robot away from the patient. The rupture of the endotracheal tube cuff was confirmed by observing air bubbles exiting from the balloon in water. The patient was discharged 3 days after surgery without complications. In robot-assisted thyroid surgery, a preoperative arrangement of the robot away from the patient's head to obtain easy access to the patient is essential for safe anesthetic care.
Adult ; Anesthesia ; Head ; Humans ; Laryngoscopy ; Male ; Operating Tables ; Rupture ; Thyroid Gland ; Ventilators, Mechanical ; Water

Endobrochial tuberculosis which may result in stenosis of the bronchus, is a rare complication of pulmonary tuberculosis in children. We recently treated three children with endobronchial tuberculosis which presented different clinical manifestations and different response to the treatments. Endoscopic examinations revealed bronchial masses, in which biopsy and polymerase chain reaction(PCR) were consistent with endobronchial tuberculosis. We reviewed the presentation and treatment of endobronchial tuberculosis in 3 children.
Biopsy ; Bronchi ; Bronchoscopy* ; Child ; Constriction, Pathologic ; Diagnosis* ; Humans ; Tuberculosis* ; Tuberculosis, Pulmonary

Background/Aims: Recent evidence has identified the significance of type 2 iodothyronine deiodinase (DIO2) in various diseases. However, the role of DIO2 polymorphism in metabolic parameters in patients with hypothyroidism is not fully understood. Methods: We assessed the polymorphism of the DIO2 gene and various clinical parameters in 118 patients who were diagnosed with hypothyroidism from the Ansan-Anseong cohort of the Korean Genome and Epidemiology Study. Furthermore, we systematically analyzed Genotype-Tissue Expression (GTEx) data. Results: A total of 118 participants with hypothyroidism were recruited; 32 (27.1%) were homozygous for the Thr allele, 86 (73.9%) were homozygous for the Ala allele or heterozygous. Patients with hypothyroidism with DIO2 polymorphism without hypertension at baseline had higher incidence of hypertension compared to patients without DIO2 polymorphism. Analysis of the GTEx database revealed that elevation of DIO2 expression is associated with enhancement of genes involved in blood vessel regulation and angiogenesis. Conclusions Commonly inherited variation in the DIO2 gene is associated with high blood pressure and prevalence of hypertension in patients with hypothyroidism. Our results suggest that genetic variation in the hypothalamic-pituitary-thyroid pathway in influencing susceptibility to hypertension.

Adefovir dipivoxil (ADV) is a nucleotide used as long-term therapy of chronic hepatitis B. Many published reports have shown that long-term high-dose therapy with adefovir can be associated with proximal renal tubular dysfunction resulting in significant hypophosphatemia, renal insufficiency and osteomalacia. We have encountered two patients who developed evidence of hypophosphatemic osteomalacia while on long-term low-dose adefovir therapy for chronic hepatitis B. We report on its clinical features and its potential resolution with cessation of the drug and supplementation with phosphate. We also reviewed the other published cases associated with hypophosphatemic osteomalacia after low-dose adefovir therapy. The symptoms and the hypophosphatemia improved after cessation of the drug and supplementation with phosphate in most cases. Patients taking adefovir long-term should receive regular investigation of the phosphate level and renal function.
Fanconi Syndrome ; Hepatitis B, Chronic* ; Hepatitis, Chronic* ; Humans ; Hypophosphatemia ; Kidney Diseases ; Osteomalacia* ; Renal Insufficiency

BACKGROUND/AIMS: The apolipoprotein B/A-I ratio (ApoB/A-I) is a powerful clinical indicator of atherosclerosis. Although numerous reports have shown the effect of non-alcoholic fatty liver disease (NAFLD) on cardiovascular disease, few reports have examined the relationship between NAFLD and the ApoB/A-I ratio. The aim of the study was to determine the association between NAFLD and the ApoB/A-I ratio in prediabetic patients. METHODS: This cross-sectional study was performed with data obtained from 701 patients (mean age, 47.9+/-9.6 years) diagnosed with prediabetes. Serum lipid profiles including lipoprotein, apolipoprotein, and calculated ApoB/A-I ratio as well as metabolic syndrome parameters such as fasting glucose and insulin were measured in each subject. RESULTS: Among the 701 patients, 340 (48%) had NAFLD. The number of male patients was 490 (NAFLD+, 276; and NAFLD-, 214). The odds ratios for the prevalence of NAFLD increased according to the quartiles of the ApoB/A-I ratio (1.886, 2.245, and 2.587) (p<0.001). CONCLUSIONS: The prevalence of NAFLD correlated with high ApoB/A-I ratio, suggesting that NAFLD increases the risk for atherosclerosis progression in male prediabetic patients.
Apolipoprotein A-I ; Apolipoproteins ; Atherosclerosis ; Cardiovascular Diseases ; Cross-Sectional Studies ; Fasting ; Fatty Liver ; Glucose ; Humans ; Insulin ; Lipoproteins ; Male ; Odds Ratio ; Prediabetic State ; Prevalence