Gallbladder cancer is among the organs examined in mass screening for cancer using ultrasonography; the reported prevalence of gallbladder cancer in such screening of a general population was 0.011%, while the prevalence of gallbladder polyps was reported as 4.3 to 6.9%. Endoscopic ultrasonography is useful for the differential diagnosis of gallbladder tumors detected by mass screening, as well as for estimating the depth of tumor invasion and detecting abnormal connections between pancreatobiliary ducts. While a systematic approach leading to diagnosis by endoscopic ultrasonography is useful, recent advances of contrast-enhanced ultrasonography are expected to establish it as a new modality for early detection. At our hospital, 7 of 26 patients with abnormal connections between pancreatobiliary ducts developed gallbladder carcinoma (23.1%), and 7 of 48 patients with gallbladder carcinoma had abnormal connections between pancreatobiliary ducts (12.5%). Serial observation in patients with gallstones and prophylactic surgery in patients with abnormal connections between pancreatobiliary ducts are necessary.
Objective We evaluated patients with chronic hepatitis C (CHC) and compared the clinical and pathological features of steatosis and metabolic syndrome to identify the risk factors for CHC with severe fibrosis. Methods One hundred seventy-one patients with biopsy-confirmed CHC were included in the study: 90 males and 81 females, age 56.2±12.8 years; 46 with obesity (BMI≥25 kg/m2); 51 with hypertension; 36 with type 2 diabetes mellitus; and 20 with hypertriglyceridemia. Results Steatosis was detected in 79 patients (46%); 92 patients (54%) showed no steatosis. Seventy-four patients (43%) showed mild fibrosis and 97 patients (56%) showed severe fibrosis. The variables that were significantly associated with steatosis were obesity [odds ratio 2.160 (1.010-4.727), p=0.046] and type 2 diabetes [odds ratio 3.667 (1.559-8.430), p=0.027]. The variables that were significantly associated with severe fibrosis were older age [odds ratio 2.675 (1.309-5.464), p=0.007], obesity [odds ratio 2.156 (1.006-4.619), p=0.048] and type 2 diabetes [odds ratio 8.739 (2.845-26.846), p=0.0002]. Nagasaki (N) score (the total number of specific risk factors, namely an older age, obesity, and type 2 diabetes) was higher in the severe fibrosis group than in the mild fibrosis group (mild fibrosis: severe fibrosis=1.48±1.14 vs.2.66±94, p<0.001). Conclusion Metabolic syndrome factors, including obesity and diabetes, play a critical role in the pathogenesis of fibrosis in CHC. The N score was therefore found to be a significant predictor of severe fibrosis in CHC.
Objective This study evaluated the relationship between individual components in metabolic syndrome (MetS) and amino-terminal pro-brain natriuretic peptide (NT-proBNP). Methods A screening program for MetS in 2008 in Taiwan excluded subjects aged <30 years and pregnant women. Fasting glucose, insulin level, high-sensitivity C-reactive protein (hsCRP), and NT-proBNP were assessed. A propensity-score matching process was used to select subjects with and without MetS comparable in age, gender, body height, and serum creatinine levels. A multiple regression model was used to determine the association between individual components of MetS and NT-proBNP. Finally 270 subjects with MetS and another 270 matched subjects without MetS aged ≥30 years were included. Results The subjects with MetS had higher uric acid and hsCRP, but not NT-proBNP. Multiple regression model showed that log (NT-proBNP) was positively associated with systolic blood pressure (β=0.002 per mmHg, p=0.013), but negatively associated with body mass index (β=-0.017 per kg/m2, p=0.018), triglyceride (β=-0.00048 per mg/dL, p=0.020) and insulin level (β=-0.005 per mU/L, p=0.005). Log (NT-proBNP) was neutral to waist circumference, fasting glucose, high-density lipoprotein cholesterol, and diastolic blood pressure. Conclusion MetS was not associated with serum NT-proBNP concentrations due to the contradictory effects of each component.
Objective To determine success rates in achieving goal blood pressures by measuring several factors including lifestyle characteristics associated with therapeutic failures for target treatment goals. Methods and Patients This prospective observational cohort study (September 2008 to September 2010) collected information on blood pressure control status and healthy lifestyle practices listed in Belloc and Breslow's seven health practices through medical records and patients' self-administrated questionnaires among 1,264 of 1,753 Japanese hypertensive patients initially registered. Multivariate analysis was performed to estimate the association between lifestyle practices and "therapeutic failures" at the baseline survey. Results Median age and proportion of males were 74 years and 41.1%. Therapeutic failures were 68.3% in non-elderly patients (<65 years of age) without diabetes mellitus or chronic kidney disease, 62.6% in patients with these diseases or those who had myocardial infarction, 41.8% in elderly patients (≥65 years of age) without these diseases and only 28.6% in patients with cerebrovascular disease. The total number of healthy lifestyle items was inversely associated with therapeutic failures in multivariate analysis among both sexes. Conclusion The study revealed low achievement rates toward treatment goals, especially non-elderly patients without diabetes mellitus or chronic kidney disease, and patients with these diseases or those who had myocardial infarction. Our data indicated the necessity to improve physicians' awareness of the management of hypertension according to treatment guidelines and the importance of a healthy lifestyle to maintain goal blood pressure levels.
Objective We identified the prognostic relevance of pneumothorax in interstitial lung disease (ILD) patients and evaluated the efficacy and safety of autologous blood-patch pleurodesis. Methods We retrospectively reviewed 59 occurrences of pneumothorax in 34 ILD patients identified over a 12-year period. Results Air leakage ceased in 16 of 22 (72.7%) episodes after blood pleurodesis and in 11 of 14 (78.6%) episodes after chemical pleurodesis. Both the cure ratio and recurrence ratio in the cure episodes were comparable with those in the chemical pleurodesis group (p=0.99 and 0.99, respectively). In addition, there were no harmful events associated with blood pleurodesis. The median survival time after the first episode of pneumothorax was less than 9 months in patients with idiopathic interstitial pneumonia (IIP) and only around 3 years in the patients with other types of ILD, which have essentially favorable outcomes. Kaplan-Meier survival estimates were significantly worse in the patients with concomitant pneumomediastinum than in those without (p<0.05). A multivariate Cox regression analysis identified that the number of episodes of pneumothorax, IIP diagnosis and concomitant pneumomediastinum were independent predictors of death. Conclusion Autologous blood-patch pleurodesis is safe and worth considering as a first-line treatment for pneumothorax secondary to ILD. However, despite treatments, the prognosis after the onset of pneumothorax in ILD patients was found to be poor. In addition, concomitant pneumomediastinum may further worsen the prognosis.
Objective Although the rates of reported symptoms of Pandemic (H1N1) 2009 influenza virus infection are well studied, the course of progression of these symptoms is not clear. In this study, we carefully reviewed the progress of each patient after hospitalization and clarified the clinical course of the symptoms. Methods We retrospectively examined the clinical data of 16 consecutive patients who had been hospitalized during the early stages of an influenza epidemic and observed the clinical progression of their symptoms. Results Each symptom had a different time of onset and progression pattern. In roughly one-third of our patients, symptoms appeared before the onset of high fever. Acute respiratory symptoms tended to last longer than other symptoms; similarly, sore throat and cough lasted longer than rhinorrhea. The SpO2 of the patients with influenza showed a declining trend. The point at which minimum SpO2 levels were noted was approximately 1.5 days after onset of fever. Conclusion In this H1N1 epidemic, patients typically tended to experience general fatigue, sore throat, and cough before the onset of fever, with sore throat and cough lasting longer than the other symptoms. Most patients showed decreased SpO2 levels at -1.5 days after onset of fever.
Objective The aim of this study was to evaluate the clinical effectiveness of endoscopic bronchial occlusion (EBO) with endobronchial Watanabe spigots (EWSs), a type of silicone bronchial blocker, for managing prolonged pulmonary air leaks. Patients and Methods Between October 2002 and April 2010, 24 patients with surgically incurable pulmonary air leaks underwent EBO with EWSs. The spigot was grasped with forceps and inserted into the affected bronchus by using a flexible bronchoscope through an endotracheal or a tracheostomy tube. Results In each patient, at least one EWS (mean=2.8) was placed for air leaks due to pneumothorax (n=15), empyema (n=8), or postsurgical complications (n=1). Twelve patients (50%) had complete resolution of the air leaks and seven (29.2%) had a reduction in air leaks, but five (20.8%) showed no improvement. Twenty-three patients required thoracic drainage tubes, which were successfully removed after EBO in 15 patients (65.2%). Of the 24 patients, four experienced severe respiratory failure requiring mechanical ventilation but were successfully treated. Complications were spigot migration, atelectasis, pneumonia, and lung abscess, but none caused significant mortality. Conclusion EBO with EWSs seems to be a reasonable and manageable treatment option for patients with prolonged pulmonary air leaks, including those with severe respiratory failure requiring mechanical ventilation.
Objective Primary quadriceps weakness/atrophy is a rare disorder with variable etiologies; therefore, this disorder has been regarded as a clinical syndrome rather than a distinct entity. However, three affected patients of a Taiwanese family demonstrate a uniform pattern of quadriceps weakness and atrophy, their clinical manifestations and pattern of inheritance may suggest a new disease entity. Patients and Methods Three patients in a Taiwanese kindred with selective quadriceps weakness and atrophy, which began after age 40 years, were examined. To disclose the confines of this disorder, muscle CT scans, electromyography, nerve conduction studies and muscle biopsies were performed; and to unravel and better understand the nature of this disorder, histopathological, ultrastructural, immunocytochemical and genetic studies were carried out. Results In two patients with long-standing disease, muscle imaging showed marked atrophy and fat replacement of the anterior thigh muscles and electromyography showed a mixture of myopathic and neuropathic changes. Muscle histopathology on the mildly affected tibialis anterior showed myopathic changes with myofibrillar degeneration and secondary neurogenic alterations. Immunocytochemical staining was not diagnostic but excluded the dystrophinopathies and other well-known muscular dystrophies. Conclusion All previously identified diseases resulting in quadriceps weakness and atrophy have been ruled out and the present disorder appears to be a new disease entity of autosomal dominant late onset quadriceps myopathy.
Objective The Japanese Respiratory Society (JRS) last revised the guidelines for community-acquired pneumonia (CAP) in adults in 2005. These guidelines proposed new criteria (A-DROP) to assess the severity of pneumonia and to differentiate between typical bacterial pneumonia and atypical pneumonia. The goal of the present study was to evaluate the utility of the A-DROP criteria for these described purposes. Methods An observational survey was conducted between July 2006 and March 2007, and patients with CAP were prospectively surveyed using consecutive enrollment methods. Patients In total, 1,875 patients from 200 medical facilities throughout Japan were analyzed. Results The JRS 2005 A-DROP system was a good indicator of mortality in the patient population, and these results were significantly correlated with the Pneumonia Severity Index (PSI) of the Infectious Disease Society of America (IDSA). Among the various factors characterized, 'SpO2 of 90% or less (PaO2 of 60 Torr or less)' was the strongest predictor of mortality. In terms of the differential diagnosis between typical bacterial and atypical pneumonia, five of six JRS 2005 items were strongly and significantly correlated with a diagnosis of atypical pneumonia. Conclusion The JRS 2005 A-DROP system was accurate and clinically useful for the assessment of the severity of pneumonia and for the differentiation between typical bacterial pneumonia and atypical pneumonia.
We report a patient with alcoholic liver cirrhosis who had a 15 mm focal nodular hyperplasia (FNH)-like nodule in the liver. This FNH-like nodule was diagnosed as hepatocellular carcinoma (HCC) mainly based on hypervascularity during the hepatic arterial phase, washout pattern during the equilibrium phase and low signal intensity during the hepatobiliary phase in gadolinium-ethoxybenzyl-diethylenetriamine pentaacetic acid (Gd-EOB-DTPA)-enhanced MRI; it was surgically resected. Its histology exhibited hepatocyte hyperplasia, fibrous septa containing unpaired small arteries accompanied by reactive bile ductules, remarkable iron deposits and sinusoidal capillarization, and was compatible with the diagnosis of an FNH-like nodule. When we analyzed the images of the present nodule retrospectively, low signal intensity on in-phase and isosignal intensity on opposed-phase T1-weighted MRI may have reflected iron deposits in the FNH-like nodule. In addition, a low signal intensity on T2-weighted MRI and no detection in diffusion-weighted MRI may help in distinguishing FNH-like nodules from HCC, since these image findings are inconsistent with typical HCC. Immunohistochemical analysis revealed a markedly reduced expression of organic anion transporter (OATP) 1B3 in this nodule, which implied decreased Gd-EOB-DTPA uptake by hepatocytes and accounted for the low signal intensity during the hepatobiliary phase on Gd-EOB-DTPA-enhanced MRI. To the best of our knowledge this is the first report in which an FNH-like nodule was assessed for OATP1B3 expression.
We report a case of a 78-year-old woman with cardiac sarcoidosis with a history of syncope and palpitation. Further assessment with echocardiography, gadolinium-enhanced cardiovascular magnetic resonance (CMR) and histology led to a diagnosis of cardiac sarcoidosis. As the patient suffered from ventricular tachycardia (VT) despite active corticosteroid therapy, an implantable cardioverter-defibrillator (ICD) was positioned. She was also administered a beta blocker, but an electrical storm appeared every several days requiring ICD therapy. The drug-refractory VT was finally controlled with a catheter ablation session, during which we could detect the VT focus in the right ventricular outflow tract next to the aneurysm by using an electroanatomic mapping system (CARTO). Referring to echocardiographic and CMR images proved very useful in detecting the aneurysm using the CARTO system.
In April 2005, a 72-year-old woman with pulmonary sarcoidosis exhibited focal 18F-fluoro-2-deoxyglucose (18F-FDG) uptake in her heart on 18F-FDG positron emission tomography (PET). Although Japanese guidelines for diagnosing cardiac sarcoidosis were not met at this point, electrocardiography, echocardiography, and magnetic resonance imaging became diagnostic for cardiac sarcoidosis 1 year later. In the present case report, the potential of 18F-FDG PET in the early recognition of cardiac sarcoidosis in comparison with other imaging modalities is discussed.
Gitelman's syndrome (GS) is an autosomal recessive disorder; it is rarely inherited over several generations. A 16-year-old boy showed hypokalemia and hypocalciuria. Clinically, he was diagnosed as GS because of diuretic responsiveness to furosemide but not thiazide. Genetic testing disclosed he was a compound heterozygote (T180K/V677M) for the SLC12A3 gene. Unexpectedly, the patient's father also showed hypokalemia and hypocalciuria. The genetic analysis showed he had an L849H mutation in addition to T180K. The present pedigree showed an extremely rare case. Diuretic tests are useful diagnostic methods, and genetic testing is necessary for precise evaluation of complicated cases as in this family.
A patient was diagnosed with autoimmune thrombocytopenia and concurrent diffuse large B-cell lymphoma (DLBCL). Both autoimmune thrombocytopenia and DLBCL were successfully treated using chemotherapy, while steroid therapy, rituximab monotherapy, and high-dose parenteral glucocorticoid therapy combined with high-dose intravenous immunoglobulin failed to improve thrombocytopenia. A greater understanding of the pathogenesis and treatment options can only be definitively clarified by analyses of settings such as the present case, as autoimmune thrombocytopenia is uncommon in patients with malignant lymphoma and the etiology and optimal treatment remain unclear.
A 34-year-old man developed fever and headache, followed by finger tremor and gait disturbance, and was admitted to our hospital about two months after onset. Blood tests showed a white blood cell count of 32,600 /μL with an eosinophil count of 22,300 /μL. There was no evidence of allergic drug reaction or parasitic infection. Cerebrospinal fluid examination demonstrated mononuclear pleocytosis without eosinophils or atypical cells. Brain MRI showed symmetric lesions bilaterally in the medial temporal lobe, frontobasal and insular regions and medulla oblongata. Herpes simplex virus-DNA was negative in the cerebrospinal fluid. The patient died about four months after onset. Histopathologically, there was infiltration of T cells, B cells and macrophages throughout the whole brain, but eosinophils or atypical cells were absent. Immunohistochemistry for herpes simplex virus type 1 and human herpesvirus 6 was negative. This case suggests that fatal encephalitis may develop in association with hypereosinophilic syndrome.
Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by acute severe headache with or without additional neurological symptoms and reversible cerebral vasoconstriction. Unruptured aneurysm has been reported in some cases with RCVS. We report a severe case of a 53-year-old woman with RCVS having an unruptured cerebral aneurysm and presenting as cortical subarachnoid hemorrhage, reversible posterior leukoencephalopathy syndrome, and cerebral infarction. She was successfully treated with corticosteroids and a calcium channel blocker and the aneurysm was clipped. Her various complications are due to the responsible vasoconstriction that started distally and progressed towards proximal arteries. This case demonstrates the spectrum of presentations of RCVS, a clinically complicated condition.
Reading epilepsy is a rare form of reflex epilepsy that occurs exclusively during reading. In this report, we describe a patient in whom seizure was the initial symptom of idiopathic hypoparathyroidism. A 31-year-old man experienced seizures three times in different bookshops while browsing. Chvostek's sign was observed. Serum calcium and parathyroid hormone levels were decreased. Brain computed tomography revealed symmetrical calcification at the dentate nuclei of the cerebellum and the basal ganglia. The interictal electroencephalography was normal. There was no photoparoxysmal response. After amelioration of serum calcium level with oral administration of vitamin D, seizure attacks ceased without antiepileptic drugs. This case is, to our knowledge, the first report of reading epilepsy associated with hypocalcemia due to idiopathic hypoparathyroidism. The psychological stress of reading at bookshops without buying might have triggered seizures under CNS hyperexcitability due to hypocalcemia.
Immunoglobulin G4 (IgG4)-related tubulointerstitial nephritis (TIN) is often accompanied by autoimmune pancreatitis (AIP) or chronic sclerosing dacryoadenitis and sialoadenitis. However, IgG4-related TIN without AIP or lacrimal and/or salivary gland lesions has not been well recognized. Here, we report a case of IgG4-related TIN associated with hepatic inflammatory pseudotumor without AIP or lacrimal and/or salivary gland lesions. A 58-year-old Japanese man with epigastralgia underwent contrast-enhanced computed tomography (CT), which revealed multiple low-density lesions in both kidneys and a low density hepatic mass. Laboratory tests showed an extremely high level of serum IgG4. Percutaneous renal and hepatic biopsies showed diffuse infiltration of lymphocytes and IgG4-positive plasma cells with fibrosis in both tissues. Two months after administration of oral prednisolone, both lesions decreased in size on follow-up CT, and the serum creatinine level also improved. No recurrence has been detected for two years with a maintenance dose of prednisolone.
We report a case of rheumatoid arthritis (RA) with autoimmune hepatitis (AIH) and Sjogren syndrome (SjS) that was treated with the tumor necrosis factor (TNF) inhibitor, etanercept (ETN). Both RA activity and transaminase levels improved as a result of treatment. Follow-up liver biopsy showed improvement of hepatitis. Although the efficacy of anti-TNF for RA patients with AIH remains controversial, this case suggests that treatment with ETN may result in a favorable clinical course in a certain subset of patients with RA and AIH.
The DNA sequencing analyses of the 16S rRNA gene, rpoB and hsp65 were conducted to characterize six strains that had been identified as Mycobacterium xenopi by DNA-DNA hybridization (DDH) for past ten years in our hospital. The results revealed Mycobacterium heckeshornense infection in one of the six cases. A 47-year-old man, who had been treated for pneumonia, had pulmonary nontuberculous mycobacterial disease. The sputa from the patient were culture positive for mycobacterium in three times. And it was diagnosed as M. xenopiby DDH method. Chest X-ray showed fibrocavitary lesion in right upper lobe was successfully treated with clarithromycin for four weeks.