No abstract available.
Osteotomy*

Differential diagnosis of invasive aspergillosis from other pulmonary fungal infections including mucormycosis is important because the treatment is pathogen-dependent. Clinically, invasive aspergillosis is often discriminated from other mold infections on the basis of typical histopathologic features in the biopsy specimen. However, biopsy alone is not always complete because different fungal species can display similar histopathologic features. Surrogate markers or molecular-based assays can be useful when the results of conventional diagnostic modalities are conflicting. Here, we present a case of invasive pulmonary aspergillosis histologically mimicking mucormycosis, which was confirmed by fungal polymerase chain reaction.
Aspergillosis ; Biomarkers ; Biopsy ; Diagnosis, Differential ; Fungi ; Invasive Pulmonary Aspergillosis* ; Lung Diseases, Fungal ; Mucormycosis* ; Polymerase Chain Reaction

High-resolution imaging techniques have increased the detection rate of adrenal incidentaloma. We developed a method of liquid chromatography-tandem mass spectrometry (LC-MS/MS) for detection of plasma free metanephrine (MN) and normetanephrine (NMN) and evaluated its analytical performance and clinical efficacy in differential diagnosis of adrenal incidentaloma. After solid-phase extraction, chromatographic isolation of the analytes and internal standard was achieved by column elution in the LC-MS/MS system. The analytes were detected in multiple-reaction monitoring mode by using positive electrospray ionization: MN, transition m/z 180.1-->165.1; NMN, m/z 166.1-->134.1. This method was validated for linearity, precision, accuracy, lower limits of quantification and detection, extraction recovery, and the matrix effect. Plasma concentrations of MN and NMN of 14 patients with pheochromocytoma were compared with those of 17 healthy volunteers, 10 patients with essential hypertension, and 60 patients with adrenal adenoma. The assay's linear range was 0.04-50.0 and 0.08-100.0 nmol/L for MN and NMN, respectively. Assay imprecision was 1.86-7.50%. The accuracy ranged from -7.50% to 2.00%, and the mean recovery of MN and NMN was within the range 71.5-95.2%. Our LC-MS/MS method is rapid, accurate, and reliable and useful for differential diagnosis of adrenal incidentaloma.
Adenoma ; Diagnosis, Differential* ; Healthy Volunteers ; Humans ; Hypertension ; Mass Spectrometry* ; Metanephrine ; Normetanephrine ; Pheochromocytoma ; Plasma*

BACKGROUND/AIMS: The aim of the study was to investigate the risk factors for biliary pancreatitis according to severity. METHODS: This study retrospectively reviewed 58 patients who underwent endoscopic retrograde cholangiopancreatography for the management of acute biliary pancreatitis between November 2001 and June 2004. The severity of pancreatitis was classified as severe or mild pancreatitis according to the Glasgow scale. Multiple clinical and radiological factors were analyzed for a relationship with the severity of pancreatitis and coexisting biliary pathology. RESULTS: Ten patients (17%) had severe pancreatitis (the SP group) and the remaining 48 patients (83%) had mild pancreatitis (the MP group). The diameter of the common bile duct CBD) and cystic duct, and the number and the size of gallstones were not significantly different between the two groups of patients. The number of patients without a periampullary diverticulum in the SP group (90.0%) was significantly higher than in the MP group (39.6%). Most of the SP patients (90.0%) had CBD stones (<5 mm) or CBD sludge, but the prevalence of CBD stones (<5 mm) or CBD sludge was lower in the MP group (54.2%, p=0.04). The absence of a periampullary diverticulum was identified as a risk factor according to severity by the use of logistic regression analysis (odds ratio=25; p=0.01). CONCLUSIONS: The development of severe biliary pancreatitis was influenced by risk factors such as a CBD stone less than 5 mm or sludge and the absence of a periampullary diverticulum.
Cholangiopancreatography, Endoscopic Retrograde ; Common Bile Duct ; Cystic Duct ; Diverticulum ; Gallstones ; Humans ; Logistic Models ; Pancreatitis* ; Pathology ; Prevalence ; Retrospective Studies ; Risk Factors* ; Sewage

Schaaf-Yang syndrome (SYS) is a rare genomic imprinting disorder caused by truncating mutations in the paternally derived MAGE family member L2 (MAGEL2) allele. It is also responsible for Prader-Willi syndrome, characterized by neonatal hypotonia, developmental delay, intellectual disability, respiratory distress in early infancy, and arthrogryposis. More than 250 individuals with approximately 57 different molecular variants have been reported since 2013, but the phenotype-genotype association in SYS is not yet fully understood. Here, we describe the case of a Korean patient diagnosed with SYS harboring a mutation in the paternal allele of MAGEL2: c.2895G>A, resulting in a protein change of p.Trp965*. The patient’s phenotype included respiratory distress, arthrogryposis, hypotonia, and feeding difficulty in the early neonatal period. Mild renal dysfunction and hearing impairment were observed during infancy.

BACKGROUND/AIMS: Nonalcoholic fatty liver disease (NAFLD) has recently been found to be a novel component of metabolic syndrome (MS), which is one of the leading causes of chronic liver disease. The serum alanine aminotransferase (ALT) and gamma-glutamyltransferase (GGT) levels are suggested to affect liver fat accumulation and insulin resistance. We assessed the associations of serum ALT and GGT concentrations within the reference ranges with MS and NAFLD. METHODS: In total, 1,069 subjects enrolled at the health promotion center of Wonkwang University Hospital were divided into 4 groups according to serum ALT and GGT concentrations levels within the reference ranges. We performed biochemical tests, including liver function tests and lipid profiles, and diagnosed fatty liver by ultrasonography. Associations of ALT and GGT concentrationgrading within the reference range with fatty liver and/or MS were investigated. RESULTS: The presence of MS, its components, and the number of metabolic abnormalities [except for high-density lipoprotein-cholesterol (HDL-C) and fasting blood glucose] increased with the ALT level, while the presence of MS, its components, and the number of metabolic abnormalities (except for HDL-C) increased with the GGT level. The odds ratios for fatty liver and MS increased with the ALT level (P<0.001 and P=0.049, respectively) and the GGT level (P=0.044 and P=0.039, respectively). CONCLUSIONS: Serum ALT and GGT concentrations within the reference ranges correlated with the incidence of NAFLD and MS in a dose-dependent manner. There associations need to be confirmed in large, prospective studies.
Adult ; Alanine Transaminase/*blood ; Cholesterol, HDL/blood ; Fatty Liver/*diagnosis/enzymology/ultrasonography ; Female ; Humans ; Liver Function Tests ; Male ; Metabolic Syndrome X/*diagnosis/enzymology ; Middle Aged ; Odds Ratio ; Reference Values ; Retrospective Studies ; gamma-Glutamyltransferase/*blood

PURPOSE: The purpose of this study is to determine the maximum tolerated dose (MTD), safety, pharmacokinetics, and recommended phase II dose of an oral drug composed of paclitaxel and HM30181A, which is an inhibitor of P-glycoprotein, in patients with advanced cancers. MATERIALS AND METHODS: Patients with advanced solid tumors received standard therapy were given the study drug at escalating doses, using a 3+3 design. The study drug was orally administered on days 1, 8, and 15, with a 28-day cycle of administration. The dose of paclitaxel was escalated from 60 to 420 mg/m2, and the dose of HM30181A was escalated from 30-210 mg/m2. RESULTS: A total of twenty-four patients were enrolled. Only one patient experienced a dose-limiting toxicity-a grade 3 neutropenia that persisted for more than 2 weeks, at 240 mg/m2 of paclitaxel. MTD was not reached. The maximum plasma concentration was obtained at a dose level of 300 mg/m2 and the area under the curve of plasma concentration-time from 0 to the most recent plasma concentration measurement of paclitaxel was reached at a dose level of 420 mg/m2. The absorption of paclitaxel tends to be limited at doses that exceed 300 mg/m2. The effective plasma concentration of paclitaxel was achieved at a dose of 120 mg/m2. Responses of 23 patients were evaluated; 8 (34.8%) had stable disease and 15 (65.2%) had progressive disease. CONCLUSION: The study drug appears to be well tolerated, and the effective plasma concentration of paclitaxel was achieved. The recommended phase II dose for oral paclitaxel is 300 mg/m2.
Absorption ; Humans ; Maximum Tolerated Dose ; Neutropenia ; P-Glycoprotein* ; Paclitaxel* ; Pharmacokinetics ; Plasma

Immunoglobulin G4 (IgG4)-related disease is a newly recognized condition characterized by fibroinflammatory lesions with dense lymphoplasmacytic infiltration, storiform-type fibrosis and obliterative phlebitis. The pathogenesis is not fully understood but multiple immune-mediated mechanisms are believed to contribute. This rare disease can involve various organs and pleural involvement is even rarer. We report a case of IgG4-related disease involving pleura. A 66-year-old man presented with cough and sputum production for a week. Chest radiography revealed consolidation and a pleural mass at right hemithorax. Treatment with antibiotics resolved the consolidation and respiratory symptoms disappeared, but the pleural mass was unchanged. Video-assisted thoracoscopic surgery was performed. Histopathology revealed dense lymphoplasmacytic infiltration and storiform fibrosis with numerous IgG4-bearing plasma cells. The serum IgG4 level was also elevated. Further examination ruled out the involvement of any other organ. The patient was discharged without further treatment and there is no evidence of recurrence to date.
Aged ; Anti-Bacterial Agents ; Autoimmune Diseases ; Cough ; Fibrosis ; Humans ; Immunoglobulin G ; Immunoglobulins* ; Phlebitis ; Plasma Cells ; Pleura ; Pleural Neoplasms ; Radiography ; Rare Diseases ; Recurrence ; Sputum ; Thoracic Surgery, Video-Assisted ; Thorax

We describe a 37-yr-old man who developed central pontine myelinolysis (CPM) after allogeneic hematopoietic stem cell transplantation (HSCT) for acute lymphoblastic leukemia. After HSCT, desquamation developed on the whole body accompanied by hyperbilirubinemia. The liver biopsy of the patient indicated graft-versus-host disease- related liver disease, and the dose of methylprednisolone was increased. Then, the patient developed altered mentality with eye ball deviation to the left, for which electroencephalogram and magnetic resonance imaging (MRI) scans were done. Brain MRI scan demonstrated the imaging findings consistent with central pontine myelinolysis and extrapontine myelinolysis. He did not have any hyponatremia episode during hospitalization prior to the MRI scan. To the best of our knowledge, presentation of CPM after allogeneic HSCT is extremely rare in cases where patients have not exhibited any episodes of significant hyponatremia. We report a rare case in which hepatic dysfunction due to graft-versus-host disease has a strong association with CPM after HSCT.
Adult ; Biopsy ; Brain/pathology ; Electroencephalography ; Graft vs Host Disease ; Hematopoietic Stem Cell Transplantation/*adverse effects ; Humans ; Hyperbilirubinemia/etiology ; Liver/pathology ; Magnetic Resonance Imaging ; Male ; Myelinolysis, Central Pontine/complications/*etiology ; Precursor Cell Lymphoblastic Leukemia-Lymphoma/complications/*therapy ; Time Factors ; Treatment Outcome

BACKGROUND/AIMS: The aim of this study was to evaluate the clinical features, radiologic findings, treatment modalities, and outcomes based on the origin of infection in patients with pyogenic liver abscesses. METHODS: We retrospectively analysed the medical records of 118 patients who were treated for pyogenic liver abscesses between January 2004 and December 2006. Of the 118 patients, 56 patients had biliary risk factors. RESULTS: The clinical characteristics were similar between the two groups, but the biliary risk group was characterized by an older age and the presence of jaundice. The size of the abscess was smaller in the group with biliary risk factors than in the cryptogenic group (p=0.02). Klebsiella pneumonia was the most common organism overall; however, Escherichia coli was more prevalent in the biliary risk group than in the cryptogenic group (p=0.03). Comparing the treatment modalities between the two groups, antibiotic therapy alone was more frequently used in the biliary group (p<0.01). The death rate was higher in the biliary group than in the non-biliary group (7.1 vs. 0.0%, p=0.04). The higher death rate in the biliary group was associated with multi-organ failure, absence of biliary drainage, and underlying diseases. CONCLUSIONS: There were differences between the two groups with respect to the size of the abscess, patient age, cultured organisms, and treatment modalities. The death rate was higher in the biliary group than the cryptogenic group.
Abscess ; Biliary Tract Diseases ; Drainage ; Escherichia coli ; Humans ; Jaundice ; Klebsiella ; Liver ; Liver Abscess, Pyogenic ; Medical Records ; Pneumonia ; Retrospective Studies ; Risk Factors