IgG4-Related Lung Disease Exhibiting the Invasion into the Diaphragm: A Case Report

Immunoglobulin G4-related disease (IgG4-RD) is a fibroinflammatory condition which involves various organs. This is a very rare case of IgG4-related lung disease (IgG4-RLD) with the invasion into diaphragm. The patient was a 71-year-old man with a long-term exposure to asbestos who had a mass shadow in the left lower lung lobe, which was suspected to invade the left diaphragm on computed tomography (CT). Positron emission tomography (PET)/CT also presented an avid intake of fluorodeoxyglucose in the mass, which suspected lung cancer. Although bronchoscopic biopsy could not lead to the definite diagnosis, we performed left lower lobectomy combined with the resection of left diaphragm. The specimen showed the features of IgG4-RLD on pathology: the vein stenosis and fibrosis around the vein, the infiltration of IgG4-positive cells, and IgG cells to IgG4 cells ratio of 40%. Furthermore, there were inflammatory cells infiltrating to the diaphragm.     

Atrial Fibrillation-triggered Ventricular Fibrillation in a Patient with Early Repolarization Syndrome

A 54-year-old man with early repolarization syndrome (ERS) implanted with an implantable cardioverter-defibrillator (ICD) developed persistent atrial fibrillation (AF) three years after the implantation. Similarly, the remote monitoring system begun frequently detecting ventricular fibrillation (VF) and polymorphic ventricular tachycardia (PVT). Longer RR intervals were repeatedly observed just before the initiation of PVT/VF. Catheter ablation for AF successfully diminished both the PVT and VF events.     

Chylous Ascites Due to Hyperthyroidism and Heart Failure

Chylous ascites (CA) is the accumulation of fluid with a high triglyceride content in the peritoneal cavity. Only two cases in the literature have reported CA with hyperthyroidism. A 28-year-old previously healthy woman presented with gradual-onset abdominal swelling, exertional dyspnea, and diarrhea. Hyperthyroidism and heart failure were diagnosed using laboratory investigation and echocardiography. Ultrasonography revealed a large amount of ascites. The ascitic fluid was milky with elevated triglyceride levels. Treatment with anti-thyroid therapy and diuretics improved all symptoms, and the free triiodothyronine (T3) level normalized after five days. Hyperthyroidism and heart failure should be considered as reversible causes of CA.     

Endoscopic ultrasound-guided transmural drainage for pancreatic fistula or pancreatic duct dilation after pancreatic surgery

Background

Endoscopic ultrasound (EUS)-guided drainage is widely used to manage pancreatic pseudocysts. Several studies have reported the use of EUS-guided drainage for pancreatic fistula and stasis of pancreatic juice caused by stricture of the pancreatic duct after pancreatic resection.

Methods

At the authors’ hospital, 262 patients underwent surgery involving pancreatic resection from April 2005 to March 2010. In 90 of these patients (34%), a grade B or C postoperative pancreatic fistula developed that required additional treatment. The authors performed EUS-guided transmural drainage (EUS-TD) for six patients (2.1%) with a pancreatic fistula or dilation of the main pancreatic duct visible by EUS. Percutaneous drainage was provided for 18 patients (6.8%). The success rates for EUS-TD and percutaneous drainage were compared in a retrospective analysis.

Results

In all six cases, EUS-TD was performed successfully without complications. Five of the six patients were successfully treated with only one trial of EUS-TD. The final technical success rate was 100% for both EUS-TD and percutaneous drainage. Both the short- and long-term clinical success rates for EUS-TD were 100% and those for percutaneous drainage were 61.1 and 83%, respectively. The differences in these rates were not significant (short-term success, P?=?0.091 vs. long-term success, P?=?0.403). However, the time to clinical success was significantly shorter with EUS-TD (5.8?days) than with percutaneous drainage (30.4?days; P?=?0.0013) in the current series.

Conclusions

The EUS-TD approach appears to be a safe and technically feasible alternative to percutaneous drainage and may be considered as first-line therapy for pancreatic fistulas visible by EUS.     

Placebo controlled evaluation of Xilei San,a herbal preparation in patients with intractable ulcerative proctitis

Background and Aim: Topical mesalamine or corticosteroid has shown efficacy in patients with ulcerative proctitis, but patients often become refractory to these interventions. Xilei San is a herbal preparation with evidence of anti‐inflammatory effects. We evaluated the efficacy of topical Xilei San in ulcerative proctitis patients. Methods: In a double blind setting, 30 patients with intractable ulcerative proctitis despite ≥ 4 weeks of topical mesalamine or corticosteroid were randomly assigned to True (n = 15) and placebo (n = 15). Patients in True received suppository Xilei San (0.1 g/dose per day of Xilei San), the other 15 received placebo suppository. The initial efficacy was evaluated on day 14. Primary endpoint of the trial was avoiding relapse during 180 days, relapse meant recurrence of active disease. Riley's index was applied for endoscopic and histological evaluations, while patients' quality of life was evaluated by an inflammatory bowel disease questionnaire. Results: On day 14, the number of patients who achieved remission, clinical activity index ≤ 4 in True was significantly higher versus placebo (P < 0.04). Likewise, at day 180, an 81.8% of patients in True were without relapse versus 16.7% in placebo (P < 0.001). Further, significant endoscopic (P < 0.01), histological (P < 0.02) and inflammatory bowel disease questionnaire (P < 0.04) improvements were observed in True, but not in placebo. Conclusions: This is the first controlled investigation showing significant clinical and endoscopic efficacy for Xilei San in patients with intractable ulcerative proctitis. Topical Xilei San was well tolerated, and was without safety concerns.     

Voxel‐wise quantification of myocardial perfusion by cardiac magnetic resonance. Feasibility and methods comparison

The purpose of this study is to enable high spatial resolution voxel‐wise quantitative analysis of myocardial perfusion in dynamic contrast‐enhanced cardiovascular MR, in particular by finding the most favorable quantification algorithm in this context. Four deconvolution algorithms—Fermi function modeling, deconvolution using B‐spline basis, deconvolution using exponential basis, and autoregressive moving average modeling —were tested to calculate voxel‐wise perfusion estimates. The algorithms were developed on synthetic data and validated against a true gold‐standard using a hardware perfusion phantom. The accuracy of each method was assessed for different levels of spatial averaging and perfusion rate. Finally, voxel‐wise analysis was used to generate high resolution perfusion maps on real data acquired from five patients with suspected coronary artery disease and two healthy volunteers. On both synthetic and perfusion phantom data, the B‐spline method had the highest error in estimation of myocardial blood flow. The autoregressive moving average modeling and exponential methods gave accurate estimates of myocardial blood flow. The Fermi model was the most robust method to noise. Both simulations and maps in the patients and hardware phantom showed that voxel‐wise quantification of myocardium perfusion is feasible and can be used to detect abnormal regions. Magn Reson Med, 2012. © 2012 Wiley Periodicals, Inc.     

A Mother Speaks

Eosinophilic pneumonia was confirmed by bronchoalveolar lavage fluid examination and transbronchial lung biopsy. Aspergillus niger was cultured from the patient's pharyngeal swab and bronchoalveolar lavage fluid. Inhalation bronchoprovocation test with A. niger antigen was positive. Although the patient's condition improved promptly with 10 mg/day prednisolone administration, dry cough recurred approximately 2 months after completion of this therapy. Severe coughing disappeared on oral cleansing with 300 mg/day amphotericin B, and he recovered completely on 100 mg/day amphotericin B administration. Oral cleansing with amphotericin B may be efficacious in preventing relapses of eosinophilic pneumonia caused by allergic reaction to fungal antigen.     

The impact of traumatic brain injury on family members living with patients: a preliminary study in Japan and the UK

Purpose: To ascertain the views of families living with TBI patients about the nature of the problems experienced as a result of TBI, and to compare the views of Japanese family members (J-FM) and British family members (B-FM) in order to find out whether there were cultural differences in family response to TBI. Methods: Family members involved in providing care were identified by the patients. Face to face interviews were conducted with all 18 carers in B-FM and four carers in J-FM. The remaining eight carers in J-FM participated in the postal questionnaire. Questionnaires were developed to explore the nature of problems and the involvement of family such as social embarrassment. Results: Problems arising in families were almost the same reported from both groups. However families in B-FM were likely to know more about how to cope with these problems. Family members in J-FM reported more statistically significant increases in social embarrassment than those in B-FM. Conclusion: The preliminary results showed that family members living with TBI patients in both groups had experienced problems. Appropriate rehabilitation services should be developed to help families as well as TBI patients in Japan.     

Angiopoietin-1 promotes LYVE-1-positive lymphatic vessel formation

Angiopoietin (Ang) signaling plays a role in angiogenesis and remodeling of blood vessels through the receptor tyrosine kinase Tie2, which is expressed on blood vessel endothelial cells (BECs). Recently it has been shown that Ang-2 is crucial for the formation of lymphatic vasculature and that defects in lymphangiogenesis seen in Ang-2 mutant mice are rescued by Ang-1. These findings suggest important roles for Ang signaling in the lymphatic vessel system; however, Ang function in lymphangiogenesis has not been characterized. In this study, we reveal that lymphatic vascular endothelial hyaluronan receptor 1-positive (LYVE-1(+)) lymphatic endothelial cells (LECs) express Tie2 in both embryonic and adult settings, indicating that Ang signaling occurs in lymphatic vessels. Therefore, we examined whether Ang-1 acts on in vivo lymphatic angiogenesis and in vitro growth of LECs. A chimeric form of Ang-1, cartilage oligomeric matrix protein (COMP)-Ang-1, promotes in vivo lymphatic angiogenesis in mouse cornea. Moreover, we found that COMP-Ang-1 stimulates in vitro colony formation of LECs. These Ang-1-induced in vivo and in vitro effects on LECs were suppressed by soluble Tie2-Fc fusion protein, which acts as an inhibitor by sequestering Ang-1. On the basis of these observations, we propose that Ang signaling regulates lymphatic vessel formation through Tie2.