Objective To investigate the short- and long-term efficacy and safety of infliximab (IFX) in intestinal Behçet's disease (BD) patients in a retrospective cohort study. Methods Among 43 consecutive patients with intestinal BD presenting at the same clinic, 15 with active disease and receiving standard treatment were given IFX infusions (5 mg/kg body weight) every eight weeks. The patients were clinically and endoscopically evaluated before treatment, then assessed after 10 weeks, 12 months and 24 months for a clinical response, defined as a significant improvement in intestinal symptoms and a reduced C-reactive protein (CRP) level. Results At week 10, 12 patients (80%) exhibited a response to IFX, with eight (53%) in remission with no intestinal symptoms and normal CRP levels. A response to IFX was maintained in seven of the 11 patients (64%) available at 12 months and in four of the eight patients (50%) available at 24 months. Of the seven patients receiving prednisolone at entry, five responders had their steroid doses reduced. Fulminant intestinal BD was predictive of an absence of response to IFX. The adverse effects comprised one infusion reaction and one case of fever, most likely related to IFX. Conclusion IFX is effective and safe in patients with refractory intestinal BD.
Objective To characterize hypertensive patients living in metropolitan cities in China. Methods This was a cross-sectional survey conducted in Beijing, Shanghai and Guangzhou. The eligibility criteria included outpatients 35-85 years of age with a systolic blood pressure (SBP) of ≥140 mmHg or a diastolic blood pressure (DBP) of ≥90 mmHg or both and/or patients receiving antihypertensive medications. The patients' demographic characteristics, medical history and findings of physical examinations, laboratory tests and cardiovascular imaging (i.e., ultrasonic cardiogram) were included in the survey. Risk stratification and the rate of hypertension control were evaluated. Results A total of 25,336 individuals were surveyed, of which 79.1% were from cardiology clinics and 51.8% were male hypertensives. The average SBP/DBP was 139.3±18.6/82.3±12.0 mmHg. The mean age was 63.6±11.5 years. The mean BMI was 25.1±3.8 kg/m2. Among the men, 55.9% had a waist circumference of >90 cm. Among the women, 50.9% had a waist circumference of >85 cm. The percentages of patients with diabetes mellitus, heart disease and cerebral vascular disease were 20.3%, 39.2% and 10.4%, respectively. The smoking rate was 17.6%. Overall, 60.9% of the patients were in the very high risk group. While 97.7% of the patients were receiving antihypertensive drug therapy, only 40.2% had controlled SBP/DBP (i.e., under 140/90 mmHg). The control rate was statistically higher in Beijing and Shanghai than in Guangzhou and among older patients than among younger patients (43% among the patients >75 years of age vs. 28.1% among the patients 35-45 years of age). Conclusion In Beijing, Shanghai and Guangzhou, most hypertensive patients have various cardiovascular risk factors and cardiovascular diseases. High blood pressure is not under appropriate control in all cases, especially among young hypertensives and patients living in Guangzhou city. Approaches designed to target multiple risk factors and concomitant cardiovascular diseases and boost the hypertension control rate are warranted.
Objective Left cardiac catheterization carries a risk of cerebral complications. We aimed to conduct a prospective comparative study to determine the frequency and clinical implications of cerebral microembolism following left cardiac catheterization. Methods One hundred and sixty-seven patients were enrolled into two groups: 111 patients who underwent left cardiac catheterization and 56 patients who underwent multidetector computed tomography (MDCT). MRI was performed within seven days after cardiac catheterization or MDCT, and acute cerebral embolism was detected on diffusion-weighted imaging (DWI). Old cerebral infarctions were detected on the initial T2-weighted imaging (T2WI), and new cerebral infarctions were detected on T2WI three months after the first MRI. Results On the initial MRI examinations, DWI-positive areas were observed in 20 cases (18%) and T2WI-positive areas were observed in only two cases (1.8%) after three months in the patients who underwent left cardiac catheterization. DWI-positive areas were observed in only one case (1.8%) after MDCT, while no T2WI-positive areas were observed three months later. No neurological abnormalities were detected in any patients with DWI-positive areas. Conclusion Left cardiac catheterization carries a risk of asymptomatic cerebral microembolism, as assessed on DWI; however, most microemboli disappear within three months without causing any neurological abnormalities.
Objective Obtaining an accurate histopathological diagnosis is mandatory for the optimal treatment of patients who are suspected of having recurrent lung cancer. The purpose of this retrospective study was to investigate the usefulness of endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) for the diagnosis of recurrent non-small cell lung cancer (NSCLC) among patients who undergo curative surgical resection. Methods Consecutive patients who underwent convex probe EBUS-TBNA for mediastinal or hilar lymph node and peribronchial lung parenchymal lesions between May 2009 and May 2011 were included. The diagnostic sensitivity, specificity, accuracy, positive predictive value (PPV) and negative predictive value (NPV) were calculated on a per-lesion and per-patient basis. Results Forty-two patients who were suspected of having recurrent NSCLC underwent EBUS-TBNA to assess 53 mediastinal and hilar lymph nodes and seven peribronchial lung parenchymal lesions. Among the 60 lesions, recurrence of malignancy was confirmed in 41 lesions on EBUS-TBNA (36 lymph nodes and five peribronchial lung lesions). On a per-lesion basis, the diagnostic sensitivity, specificity, accuracy, PPV and NPV of EBUS-TBNA for confirming recurrence were 95.3%, 100%, 96.6%, 100% and 88.9%, respectively. On a per-person basis, the diagnostic sensitivity, specificity, accuracy, PPV and NPV were 94.3%, 100%, 95.2%, 100% and 77.8%, respectively. No serious complications related to the procedures were observed. Conclusion Convex probe EBUS-TBNA is a sensitive method for diagnosing recurrent NSCLC in patients with lymph node and peribronchial lung parenchymal lesions. Therefore, EBUS-TBNA should be considered first for the cytopathological diagnosis of recurrent NSCLC.
Objective Thrombotic thrombocytopenic purpura (TTP) is a life-threatening generalized disease with pathological features that are termed thrombotic microangiopathies. Since the discovery of the von Willebrand factor-cleaving protease [a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13)], it is widely known that approximately two-thirds of TTP patients have a severe deficiency of ADAMTS13 activity due to gene mutations or acquired autoantibodies to this enzyme. However, the remaining one-third of TTP patients have only moderately reduced or almost normal ADAMTS13 activity. To elucidate the clinical characteristics and outcomes of these two types of TTP, we have retrospectively analyzed the cases of acquired TTP patients treated in a single institution from 2000 to 2011. Methods Our case studies include 11 TTP patients, of which 5 were considered idiopathic and 6 had cases of TTP associated with underlying diseases such as non-Hodgkin lymphoma or connective tissue diseases. Results These patients were treated with a combination therapy of plasma exchange and steroids and with several adjunctive therapeutic regimens including the on-label use of cyclophosphamide and cyclosporine and the off-label use of high-dose steroid or immunoglobulin with rituximab. Splenectomies were not performed. As a result of these treatments, 6 out of the 7 patients with ADAMTS13 activity deficient TTP achieved a complete remission without relapse, but the remaining 4 patients with non-ADAMTS13 activity deficient TTP all died without complete remission. Conclusion We present herein the detailed clinical courses of 11 patients with TTP and address our experiences with the efficacy of various therapeutic regimens. This case-oriented study should be helpful to the physicians who directly care for TTP patients, and may provide a future direction for developing a more efficient treatment modality.
Objective Disseminated intravascular coagulation (DIC) is often associated with infection and a poor outcome. In this study, useful markers for predicting poor outcomes were examined. Methods The frequency of DIC and organ failure, outcomes and hemostatic markers were prospectively evaluated in 242 patients with infections. Results Seventy-seven patients were diagnosed with DIC, 36 of whom recovered from the condition. The rate of DIC or resolution of DIC was highest in the patients with sepsis and lowest in the patients with respiratory infections. Mortality tended to be high in the patients with respiratory infections. The DIC score, sepsis-related organ failure assessment (SOFA) score, prothrombin time (PT) ratio and thrombin-antithrombin complex level were significantly high in the patients who did not recover from DIC. The age, DIC score, SOFA score, PT ratio and levels of thrombomodulin and plasminogen activator inhibitor (PAI)-I were significantly high in the non-survivors. Factors related to a poor outcome included resolution of DIC, the SOFA score, age and the PT ratio. Factors related to resolution of DIC included the SOFA score and age, while factors related to the SOFA score included the levels of PAI-I, leukocytes, fibrinogen, D-dimer and platelets. Conclusion The outcomes of septic patients primarily depend on the SOFA score and the resolution of DIC, which are related to organ failure.
Systemic AA amyloidosis is a complication of various inflammatory diseases including Crohn's disease (CD). Amyloid nephropathy is the most common clinical presentation of AA amyloidosis leading to renal failure, and affected patients often require hemodialysis and ultimately renal transplantation. We herein report the successful use of infliximab as maintenance therapy for amyloid nephropathy in a patient with CD. In the present patient, surgical treatment and infliximab infusion immediately induced a remission of CD, and scheduled infliximab therapy successfully maintained the patient's stable condition for three years, with a significant decrease in the serum creatinine level.
A 72-year-old man suffered from paraparesis with a sensory impairment and bladder and rectal disturbances. Magnetic resonance imaging T2-weighted images depicted a high-intensity lesion in the spinal cord that was consistent with myelitis. A blood examination revealed severe thrombocytopenia and liver dysfunction. No malignant cells were detected by peripheral smears or bone marrow biopsy. Systemic computed tomography detected hepatosplenomegaly and ascites but no lymphadenopathies. Transjugular liver biopsy (TJLB) safely confirmed a diagnosis of intravascular large B-cell lymphoma (IVLBCL), and the patient achieved a complete response following treatment with an appropriate chemotherapy. TJLB is therefore a timely and accurate diagnostic approach for IVLBCL, especially when a bleeding tendency and ascites are noted.
A previously healthy 18-year-old man was admitted to our hospital with abdominal pain in September 2010. We performed a percutaneous biopsy of multiple intrahepatic masses. A diagnosis of desmoplastic small round cell tumors was confirmed based on the presence of a polyphenotypic immunoprofile (positivity for EMA, vimentin, cytokeratin, desmin and WT1) and characteristic EWS-WT1 gene fusion. Because the mass had invaded the mesentery and the disease had disseminated to liver, the patient received palliative chemotherapy with carboplatin, paclitaxel, vincristine, doxorubicin, cyclophosphamide, ifosfamide, etoposide and irinotecan. The maximal response to the chemotherapy was a partial remission. The patient died 20 months after diagnosis.
We herein present the findings of the case of a 23-year-old man who was hospitalized for ventricular tachycardia (VT) with no previous history of cardiac disease or any family history of sudden death. Based on the clinical features as well as the echographic and MRI results, the patient was diagnosed with both acute viral myocarditis and arythmogenic right ventricular dysplasia (ARVD). The patient underwent implantation of an automatic cardioverter defibrillator. There was no recurrent VT during the 24 month follow-up. This case demonstrates the link between ARVD and myocarditis, and highlights the importance of conducting an RV assessment through a cardiac magnetic resonance (CMR) study in the context of arrhythmia and myocarditis.
A 63-year-old woman had mistakenly drunk detergent stored in a plastic bottle and was transported to our hospital via ambulance due to unconsciousness. In the emergency room, the monitoring electrocardiogram showed complete atrioventricular block and temporary pacing was thus started. Left ventriculography indicated midventricular Takotsubo cardiomyopathy, although coronary angiograms showed a normal appearance. The atrioventricular block was transient, and the reduced left ventricular wall motion gradually recovered. An electrophysiological study performed before discharge showed no abnormalities in the atrioventricular conduction system. In conclusion, we experienced a case of mid-ventricular Takotsubo cardiomyopathy complicated by transient complete atrioventricular block.
Coronary artery fistulae that drain into the left ventricle are extremely rare, and even fewer cases of fistulae involving all three of the coronary arteries have so far been reported. We herein report a 64-year-old woman with a unique pattern of coronary artery-left ventricular fistulae that involved all three of the coronary arteries. The multiple fistulae presented in a diffuse plexus-like arrangement. The fistulae resulted in a diastolic volume overload of the left ventricle (left-to-left shunt), as well as "coronary steal" with the shunting of blood away from the myocardium since the fistulae represented the path of least resistance.
Pulmonary tumor thrombotic microangiopathy is a lethal, yet difficult to diagnose, complication of gastrointestinal carcinoma. Even if properly diagnosed, there is no treatment, especially after a circulatory collapse. We herein report a case of pulmonary tumor thrombotic microangiopathy with circulatory failure due to pulmonary hypertension. The patient was temporarily successfully treated with imatinib, an inhibitor of the platelet-derived growth factor receptor. Pulmonary hypertension was dramatically ameliorated and the patient was able to be weaned from percutaneous cardiopulmonary support within 20 days of treatment. Imatinib may be effective for ameliorating pulmonary hypertension that is caused by pulmonary tumor thrombotic microangiopathy.
A 59-year-old man with recurrent oral cancer presented with severe pancytopenia, hyponatremia and hypoglycemia. Endocrine testing showed a partial primary adrenal insufficiency and primary hypothyroidism. The bone marrow biopsy showed a gelatinous transformation with hypocellularity and fat atrophy. His pancytopenia, hyponatremia and hypoglycemia resolved following treatment with corticosteroids and thyroid hormone replacement therapy. The follow-up bone marrow biopsy demonstrated a resolution of the gelatinous transformation. This case is a rare example of a patient with a primary insufficiency of the adrenal and thyroid glands that is associated with gelatinous bone marrow transformation (GMT). The GMT was resolved through the admimistration of corticosteroids and thyroid hormone replacement therapy.
We herein report the case of a patient with a juxtaglomerular cell tumor (JCT). Dynamic enhanced computed tomography revealed a small nodule on the surface of the lower pole of the right kidney. Selective renal venous sampling showed an elevated level of plasma renin activity (PRA) in the right lower pole renal vein only. We performed right partial nephrectomy and diagnosed the patient with JCT. Making a diagnosis of JCT is often difficult due to the small size of the tumor and the lack of lateralization of the PRA on renal venous sampling. This case highlights the importance of performing selective renal venous sampling for the preoperative diagnosis of JCT.
Preeclampsia is the most common hypertensive disorder to occur during pregnancy. A healthy 38-year-old primipara presented with pretibial edema at 33 weeks of gestation followed by the development of proteinuria at 36 weeks of gestation. She had no past medical history of hypertension and was normotensive during gestation. Her proteinuria persisted after delivery, and she was also hypoalbuminemic. A renal biopsy revealed a remodeling of the glomerular basement membrane (GBM) with double contours. Some of the glomerular segments showed endothelial swelling. Immunoperoxidase staining for C4b-binding protein was positive and Protein S was weakly detected in the GBM. Electron microscopy revealed an expansion of the subendothelial zone as well as mesangial cell interposition. This case suggests that glomerular endotheliosis may therefore sometimes be present despite the absence of hypertension.
A 37-year-old woman at 17 weeks of gestation who was first noted to have proteinuria and microscopic hematuria at 13 weeks of gestation was admitted to our hospital with proteinuria that progressed to nephrotic syndrome (NS). Despite the treatment with prednisolone, including methylprednisolone pulse therapy, the NS worsened. The patient underwent an elective abortion at 21 weeks of gestation, and the NS then went into partial remission. A renal biopsy revealed membranous nephropathy (MN). There was no evidence of secondary MN. This is the first reported case of subclinical idiopathic MN that first developed in pregnancy.
Focal segmental glomerulosclerosis (FSGS) is extremely rare among paraneoplastic nephrotic syndromes. We herein report a case of lung adenocarcinoma with nephrotic syndrome caused by paraneoplastic FSGS. A 68-year-old man visited our hospital for an evaluation of a right hilar mass on chest radiography and supraclavicular lymphadenopathy. Because an aspiration biopsy of the supraclavicular lymph node revealed adenocarcinoma, the patient was diagnosed with lung adenocarcinoma. He also had nephrotic syndrome, and the pathological findings of the renal biopsy demonstrated FSGS. Standard-dose carboplatin-containing chemotherapy led to a partial response for lung cancer and improved the patient's nephrotic syndrome without causing any adverse renal effects.
We herein report a case of hard metal lung disease that was successfully treated with inhaled corticosteroids. A 46-year-old man was admitted to our hospital due to coughing and an abnormal shadow on a chest radiograph. He had worked as a hard metal tool sharpener for five years. Chest computed tomography scans showed centrilobular micronodules and areas of ground-glass opacity in the bilateral lung fields. Video-associated lung biopsy specimens revealed bronchocentric interstitial pneumonia and cellular bronchiolitis. A high-energy dispersion X-ray microanalysis detected tungsten. The patient was diagnosed with hard metal lung disease. Inhaled corticosteroid therapy (800 μg of ciclesonide hydrofluoroalkane daily) resolved the patient's symptoms, elevated KL-6 level, abnormal areas of chest opacity and obstructive, restrictive and diffusion impairments.
Ankylosing spondylitis (AS) primarily affects the spine and axial skeleton. Various pulmonary manifestations have been reported; however, diffuse alveolar hemorrhage (DAH) has not been previously described in a patient with AS. A 49-year-old man with longstanding AS visited our hospital complaining of progressive dyspnea and hemoptysis. DAH was diagnosed based on the findings of chest computed tomography and bronchoscopy. No positive findings suggested any cause of DAH other than weakly positive results for perinuclear antineutrophil cytoplasmic antibodies on indirect immunofluorescence. Following the administration of steroid and plasmapheresis therapy, the patient's symptoms and chest radiographic findings improved. Further clinical case reports and investigations are needed to clarify whether DAH represents a possible pulmonary manifestation of AS.
A 78-year-old man presented with urinary retention and difficulty walking. Both legs showed muscle weakness, and he was experiencing lower body hypoesthesia. T2-weighted magnetic resonance imaging revealed lesions with high signal intensity and enhancement in the spinal cord and cerebrum. A cerebrospinal fluid specimen showed inflammatory changes, but negative cytology findings. Chest computed tomography revealed a tumor measuring 40 mm in diameter, and a lung biopsy revealed the presence of squamous cell carcinoma. We diagnosed the patient with paraneoplastic neurological syndrome related to lung cancer. The patient was treated with steroid pulse therapy and chemotherapy, which relieved the symptoms and enabled the patient to achieve an independent gait.
A 32-year-old man presented with a fever. A laboratory examination detected atypical lymphocytes and liver enzyme elevation. The serological tests for Epstein-Barr virus (EBV) were consistent with an acute infection pattern. Computed tomograpy showed bowel wall thickening, and colonoscopy revealed numerous ulcerations. The histological findings from the biopsy specimens from the colon were consistent with peripheral T-cell lymphoma (PTCL), and in situ hybridization detected EBER-1 in the atypical lymphocytes. Because his clinical and endoscopic abnormalities improved without medication, we diagnosed the patient with EBV-associated enteropathy. We herein report a rare case of EBV-associated enteropathy that required careful differentiation from PTCL.
Granulomatous amoebic encephalitis (GAE) is a rare but fatal infection. Due to its nonspecific symptoms and laboratory and neuroradiological findings, it is rarely diagnosed antemortem. We herein present the case of a 72-year-old Japanese woman who was diagnosed with GAE following the detection of a pathogen similar to Balamuthia mandrillaris under a microscopic examination of cerebrospinal fluid sediment and who achieved remission with combination antimicrobial therapy. There are no previous reports of pathogens similar to B. mandrillaris being detected in cerebrospinal fluid antemortem; therefore, this case may be used as a benchmark for further studies.
Tocilizumab (TCZ) was administered from 2004 to 2008 in a 52-year-old woman with rheumatoid arthritis (RA) refractory to methotrexate (MTX) as a clinical trial. TCZ therapy with MTX was resumed in March 2009 due to exacerbation of RA. The patient was an human T-lymphotropic virus type I (HTLV-I) carrier, and, in April 2011, a peripheral blood smear showed many atypical lymphocytes, thus leading to a diagnosis of adult T-cell leukemia (ATL). Complete remission of ATL was achieved with a standard therapeutic regimen.
We herein report the findings of a case of myelodysplastic syndrome that was complicated by septicemia and meningoencephalitis, both of which were caused by Bacillus cereus. In contrast to all of the previous cases of B. cereus that have been seen at our institution, this patient did not have any invasive devices, such as a central venous catheter, that could have acted as a conduit for a B. cereus infection. Although B. cereus-induced meningoencephalitis is often lethal, the immediate treatment with a regimen of antibiotics including vancomycin was effective in eradicating the infection and, therefore, in reversing both the septicemia and the meningoencephalitis.