Primary biliary cirrhosis is frequently associated with a variety of disorders presumed to be autoimmune in nature, such as Sjogrens syndrome, scleroderma, rheumatoid arthritis, systemic lupus erythematosus, and autoimmune thyroiditis. Scleroderma has been recognized in association with primary biliary cirrhosis. Most cases present as the CREST (calcinosis cutis, Raynauds phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) syndrome. Isolated pulmonary hypertension develops in a small proportion of patients, nearly all of whom have limited cutaneous involvement. We report a case who has diffuse scleroderma associated with primary biliary cirrhosis and isolated pulmonary hypertension.
Arthritis, Rheumatoid ; Esophageal Motility Disorders ; Humans ; Hypertension, Pulmonary* ; Liver Cirrhosis, Biliary* ; Lupus Erythematosus, Systemic ; Scleroderma, Diffuse* ; Sjogren's Syndrome ; Thyroiditis, Autoimmune

PURPOSE: To determine correlation between stress fracture of the posterior tibia and flexor digitorum longusmuscle injury caused by athletic or sporting activity during adolescence. MATERIALS AND METHODS: Eleven casesdiagnosed as stress fracture after X-ray and MR imaging of the lower leg were reviewed. With regard to eachfracture, the following features were noted: age, sex, and athletic or sporting activity of the patient, and site.Using MR imaging techniques, axial and sagittal T1 and T2 weighted images were obtained in all cases and T1-GdDTPA images in seven. RESULTS: The activities undertaken were running (n=3), football (n=2), ballet (n=2),taekwando (n=1), badminton (n=1), field hockey (n=1), and basketball (n=1). MR images revealed localized corticalthickness (n=11), linear intramedullary callus showing a low signal on T1 and T2 weighted images (n=9), marrowhyperemia (n=7), and flexor digitorum longus muscle injury showing a high signal on T1-Gd DTPA and T2 weightedimage (n=7). CONCLUSION: Stress fracture of the posterior tibia might be induced by flexor digitorum longusmuscle activity induced by athletic or sporting activities during adoleseence.
Adolescent ; Basketball ; Bony Callus ; Football ; Fractures, Stress* ; Hockey ; Humans ; Leg ; Magnetic Resonance Imaging ; Pentetic Acid ; Racquet Sports ; Running ; Sports ; Tibia*

BACKGROUND: Tricuspid regurgitation (TR) is a common finding that can be detected with use of Doppler echocardiographic technique in patients with right ventricular dysfunction as well as in normal children, adolescents and adult. It is difficult to evaluate the right ventricular function by conventional method, including echocardiography, especially in case with TR. METHOD: To determine the degree and relationship of right ventricular function and duration of TR, we examined the 41 patients with TR associated with heart disease, group 2 (n=19) with no definitive evidence of right ventricular dysfunction nor significant pulmonary hypertension, and group 3 (n=22) with right ventricular dilatation or wall motion abnormality, or history of longstanding pulmonary hypertension or moderate or severe TR, and compared 59 normal subjects (group 1) with trivial or mild TR without definitive heart disease. Using Doppler echocardiography, duration of TR (TRD) and pulmonary ejection period (ET) is measured, and calculated the TR time interval (RTI) by the difference of TRD and ET divided by ET. RESULT: Pre-ejection period (PEP), ET and systolic time interval (STI, PEP/ET) of right ventricle are significantly prolonged in group 2 and 3 compare to those of group 1 (p<0.001 respectively), TRD is significantly prolonged in group 3 compare to those of group 1 and 2, and isovolumic contraction time (IRT), isovolumic relaxation time (IRT) and RTI are significantly different in each group and prolonged in group 2 and 3. RTI is significantly correlated to STI (r=0.56), ICT (r=0.75) and IRT (r=0.94), but independent to heart rate. CONCLUSION: We conclude that Doppler measurement of RTI (TRD-ET)/ET{=(ICT+IRT)/ET} is a simple and useful new index for the evaluation of RV function including systolic and diastolic function.
Adolescent ; Adult ; Child ; Dilatation ; Echocardiography ; Echocardiography, Doppler ; Evaluation Studies as Topic* ; Heart Diseases ; Heart Rate ; Heart Ventricles ; Humans ; Hypertension, Pulmonary ; Relaxation ; Systole ; Tricuspid Valve Insufficiency* ; Ventricular Dysfunction, Right ; Ventricular Function, Right

We report on an anesthetic experience with a 74-year-old female with Isaacs' syndrome, who underwent elective surgery for open rotator cuff repair. Isaacs' syndrome is a rare peripheral motor neuron disorder with clinical manifestations such as involuntary muscle twitching, cramps, mild weakness and increased sweating. To avoid prolonged neuromuscular blockade, the patient was observed with neuromuscular monitoring during total intravenous anesthesia with propofol, remifentanil, and atracurium. No adverse events were observed during the anesthetic management, and the patient recovered smoothly from the neuromuscular blockade. We describe the clinical characteristics of Isaacs' syndrome and its specific considerations in anesthesia.
Anesthesia ; Anesthesia, Intravenous ; Atracurium ; Female ; Humans ; Isaacs Syndrome ; Motor Neurons ; Muscle Cramp ; Muscle, Smooth ; Neuromuscular Blockade ; Neuromuscular Monitoring ; Piperidines ; Propofol ; Rotator Cuff ; Sweat ; Sweating

PURPOSE: We performed a multicenter, prospective study to evaluate the efficacy of imipramine and desmopressin to improve arousability and prevent nocturnal enuresis(NE). MATERIALS AND METHODS: The total of 48 children with NE were given questionnaires that included a scoring system for the assessing arousal from sleep. They were assigned into two groups: group 1(imipramine 25 mg, 3 girls and 16 boys, mean age 7.9 years), group 2(desmopressin 0.2 mg, 9 girls and 9 boys, mean age 7.5 years). The assessment of arousability was repeated 2 weeks and 4 weeks after medication. Eleven children were excluded because of incomplete data. RESULTS: Mean wetting events in group 1 decreased from 8.8 to 5.1 times(2 weeks) and 3.0 times(4 weeks)(p=0.009) versus 10.2 to 5.5 times(2 weeks) and 6.4 times(4 weeks)(p=0.007) for group 2. The mean threshold of arousability in group 1 was 4.9(baseline), 4.4(2 weeks), and 3.7(4 weeks), and, for group 2, 5.1(baseline), 4.8(2 weeks), and 4.8(4 weeks). The two groups were not different(p=0.14, p=0.73). CONCLUSIONS: Imipramine and desmopressin, which are commonly used in treating NE in Korea, influenced wetting events but not arousability.
Arousal ; Child ; Deamino Arginine Vasopressin ; Humans ; Imipramine ; Korea ; Nocturnal Enuresis ; Prospective Studies

Air injected into the epidural space may spread along the nerves of the paravertebral space. Depending on the location of the air, neurologic complications such as multiradicular syndrome, lumbar root compression, and even paraplegia may occur. However, cases of motor weakness caused by air bubbles after caudal epidural injection are rare. A 44-year-old female patient received a caudal epidural injection after an air-acceptance test. Four hours later, she complained of motor weakness in the right lower extremity and numbness of the S1 dermatome. Magnetic resonance imaging showed no anomalies other than an air bubble measuring 13 mm in length and 0.337 ml in volume positioned near the right S1 root. Her symptoms completely regressed within 48 hours.
Epidural Space ; Female ; Humans ; Hypesthesia ; Injections, Epidural ; Lower Extremity ; Magnetic Resonance Imaging ; Paraplegia

BACKGROUND/AIMS: Although transcatheter arterial chemoembolization (TACE) is a major treatment modality for unresectable hepatocellular carcinoma (HCC), acute hepatic failure after TACE is not rare. However, reports dealing with this important complication are not good enough and results are often variable. The purpose of this study was to evaluate the incidence and associated risk factors of acute hepatic failure after TACE. METHODS: From January 2001 to November 2004, six hundred and thirtytwo TACE sessions were performed in 377 patients (294 men and 83 women). Adriamycin mixed lipiodol solution and gelfoam were used for TACE. Various clinical and radiological factors before and after the procedure were reviewed retrospectively. Univariate and multivariate analyses were performed to evaluate the risk factors associated with the development of acute hepatic failure after TACE. RESULTS: Acute hepatic failure occurred in 76 (12.0%) of the 632 TACE sessions within 14 days. Univariate analysis revealed that Child-Pugh class, 1st TACE, total bilirubin level, number of involved segments, total size of tumor, presence of right portal vein thrombosis (PVT) or main PVT, involvement of segment 1, 5, 6, 7, modified UICC stage, and doses of chemotherapeutic agent were significantly different between the patients with or without hepatic failure after TACE. Among them, elevated total bilirubin (p=0.001, E (beta)=1.449), presence of right (p=0.035, E (beta)=2.109) or main (p=0.011, E (beta)=4.067) PVT were independently associated factors in multivariate analysis. CONCLUSIONS: The incidence of acute hepatic failure after TACE was 12.0%. Elevated bilirubin level and portal vein thrombosis could be considered as the predictive factors for acute hepatic failure after TACE in HCC patients.
Adult ; Aged ; Aged, 80 and over ; Antibiotics, Antineoplastic/adverse effects/therapeutic use ; Carcinoma, Hepatocellular/complications/pathology/*therapy ; Chemoembolization, Therapeutic/*adverse effects/methods ; Doxorubicin/adverse effects/therapeutic use ; Female ; Humans ; Incidence ; Iodized Oil/adverse effects/therapeutic use ; Liver Failure, Acute/epidemiology/*etiology ; Liver Function Tests ; Liver Neoplasms/complications/pathology/*therapy ; Male ; Middle Aged ; Multivariate Analysis ; Predictive Value of Tests ; Retrospective Studies ; Risk Factors ; Severity of Illness Index

Maternal alcohol abuse is thought to be the common cause of mental retardation. Especially, continuous alcohol consumption during critical period of brain development induce fetal alcohol effects. In this study, the authors investigated the effects of maternal alcohol drinking on the postnatal changes of BDNF contents and patterns of BDNF-containing neuron in neonatal rat brain, and, the influence of maternal thyroxine treatment on the brain of pups of alcohol abused mother. Pregnant rats were divided into three groups. Alcohol-fed group (n=4) received 35 calories of liquid alcohol diet daily from gestation day 6; control pair-fed group (n=4) was fed a liquid diet in dextrin replaced alcohol isocalorically; alcohol+T4 group (n=4) received 35 calories liquid alcohol diet and exogenous thyroxine (5 microgram/kg/day) subcutaneously. The amount of BDNF was significantly higher in the alcohol+T4 group as compared to the alcohol group at P7, P14 and P21, especially, alcohol+T4-exposed pups showed a significant increase of BDNF at P7. The decrease in BDNF was found in alcohol group compared to control pair-fed group at all ages. In alcohol+T4 group, BDNF-containing Purkinje cells exhibited mature pattern and monolayer arrangement at P14. Alcohol+T4 group showed mature pattern and numerical increase of BDNF-containing cells in cerebral cortex, hypothalamus and hippocampus at P7. The BDNF immunoreactivity of hippocampus continued to show prominent configuration in alcohol+T4 group at P28. These results indicate that the increase of the BDNF-containing neurons and BDNF amount in pups of thyroxinesupplemented alcohol-exposed dams as compared to control pair-fed and alcohol-exposed pups at P7, presumably suggest the early postnatal growth stimulatory effect of the exogenously supplemented thyroxine. Therefore, the increase of BDNF synthesis caused by maternal administration of exogenous thyroxine may ameliorate fetal alcohol effects, one of the ill effects as a result of the dysthyroid state following maternal alcohol abuse.
Alcohol Drinking ; Alcoholism ; Animals ; Brain* ; Brain-Derived Neurotrophic Factor ; Cerebral Cortex ; Critical Period (Psychology) ; Diet ; Hippocampus ; Humans ; Hypothalamus ; Immunohistochemistry ; Intellectual Disability ; Mothers* ; Neurons* ; Pregnancy ; Purkinje Cells ; Rats ; Thyroxine*

The Budd-Chiari syndrome is no longer a rare disease in the world, but there has been small cases reported until now in Korea. There are three forms of Budd-Chiari syndrome, among them membranous web in the inferior vena cava is the most common form in the oriental area. Authors have expereinced the Budd-Chiari syndrome, 49 year old female patient, caused by membranous web associated with the long thrombus in the inferior vena cava. She underwent side-to-side portacaval shunt and cavoatrial shunt using 19 mm diameter, 25 cm length polytetrafluoroetylene graft. Postoperative major morbidities were ARF and pneumonia. She has improved clinically and pathologically after operation. It is concluded that combined portacaval shunt and cavoatrial shunt has been effective to relieve the symptoms of Budd-Chiari syndrome caused by membranous web and inferior vena cava thrombotic occlusion.
Budd-Chiari Syndrome ; Female ; Humans ; Korea ; Middle Aged ; Pneumonia ; Portacaval Shunt, Surgical ; Rare Diseases ; Thrombosis ; Transplants ; Vena Cava, Inferior

PURPOSE: To identify potential predictive factors of incidental prostate cancer (IPca) in patients considering tissue-ablation treatment for benign prostatic hyperplasia (BPH). MATERIALS AND METHODS: From the 11 centers, 1,613 men who underwent transurethral resection of the prostate (TURP) or open prostatectomy were included. Before surgery, prostate biopsy was performed in all patients with prostate-specific antigen (PSA) > or =4.0 ng/ml or with abnormal digital rectal examination (DRE) findings. The patients with prostate cancer preoperatively or with PSA >20 ng/ml were excluded. As predictive factors of IPca, age, body mass index, PSA, DRE, and transrectal ultrasonography (TRUS) findings, including total prostate volume (TPV), transition zone volume (TZV), and the presence of hypoechoic lesions, were reviewed. PSA density (PSAD) and PSAD in the transition zone (PSAD-TZV) were calculated. RESULTS: IPca was diagnosed in 78 patients (4.8%). DRE findings, PSA, and TZV were independent predictive factors in the multivariate analysis. In the receiver operating characteristic curve analysis of PSA, PSAD, and PSAD-TZV, the area under the curve (AUC) was the largest for PSAD-TZV (AUC, 0.685). CONCLUSIONS: IPca was detected in 4.8% of the population studied. In addition to DRE findings, the combination of TZV and PSA can be useful predictive factors of IPca in patients considering tissue-ablation treatment as well as TURP.
Biopsy ; Body Mass Index ; Cyanoacrylates ; Digital Rectal Examination ; Humans ; Male ; Multivariate Analysis ; Prostate ; Prostate-Specific Antigen ; Prostatectomy ; Prostatic Hyperplasia ; Prostatic Neoplasms ; ROC Curve ; Transurethral Resection of Prostate