ABSTRACT
Human T-cell leukemia virus type 1 (HTLV-1)-associated myelopathy/tropical spastic paraparesis (HAM/TSP) is a chronic neurodegenerative disease. This multicenter, randomized phase 3 study evaluated the efficacy and safety of 0.3 mg/kg intravenous mogamulizumab, a monoclonal antibody targeting-CC chemokine receptor 4, every 12 weeks in HAM/TSP patients. This study comprised a 24-week double-blind, placebo-controlled period, 24-week open-label period, and extension treatment period. The primary endpoint was the proportion of patients with a ≥ 1-grade improvement in the Osame motor disability score (OMDS). Secondary endpoints were changes in HTLV-1 proviral load, 10-m timed walk, cerebrospinal fluid (CSF) neopterin levels, and safety. The exploratory endpoint was CSF chemokine C-X-C motif ligand 10 (CXCL10) levels. Thirty-four and 33 patients were randomized to mogamulizumab and placebo arms, respectively. At the end of the double-blind period, no significant difference was found in the OMDS improvement rate or other secondary efficacy endpoints assessing motor activities. However, the mogamulizumab arm showed a significant decrease in HTLV-1 proviral load (- 59.39 ± 29.91% vs. placebo 2.32 ± 36.31%) and CSF neopterin (p < 0.001)/CXCL10 levels (p = 0.004). The baseline OMDS pattern and the 60-80% HTLV-1 proviral load reduction were sustained through the open-label and extension treatment periods. Although a higher incidence of rash (69.2%) was reported, the safety profile was similar compared with a previous phase 1/2a study. We found no significant difference in clinical benefit; however, mogamulizumab may provide long-term clinical benefit by preventing disease progression, as CSF neopterin/CXCL10 levels are associated with long-term prognosis in HAM/TSP.Clinical Trial Registration Number: NCT03191526 (registered date: 6-June-2017).
Subject(s)
Antibodies, Monoclonal, Humanized , Human T-lymphotropic virus 1 , Neopterin , Paraparesis, Tropical Spastic , Humans , Double-Blind Method , Antibodies, Monoclonal, Humanized/administration & dosage , Male , Middle Aged , Female , Paraparesis, Tropical Spastic/drug therapy , Paraparesis, Tropical Spastic/cerebrospinal fluid , Adult , Aged , Neopterin/cerebrospinal fluid , Human T-lymphotropic virus 1/drug effects , Chemokine CXCL10/cerebrospinal fluid , Viral Load/drug effects , Treatment OutcomeABSTRACT
Since even subtle mucosal changes may be depicted using virtual endoscopy created by the three-dimensional reconstruction of MDCT images, we developed a novel diagnostic imaging system that integrates and displays virtual enteroscopy, curved planar reconstruction, and a virtual unfolded view, the width of which changes with increases/decreases in the inner luminal diameter. The system is also equipped with artificial intelligence that superimposes and displays depressed areas, generates an automatic small bowel centerline that connects fragmented small bowel regions, and performs electronic cleansing. We retrospectively evaluated the diagnostic performance of this system for small bowel lesions in Crohn's disease, which were divided into two groups: endoscopically-observable and endoscopically-unobservable. Lesion detection rates for stenoses, longitudinal ulcers with a cobblestone appearance, and scars were excellent in both groups. This system, when used in combination with endoscopy, shows slight mucosal changes in areas in which an endoscope cannot reach due to strictures, thereby extending the range of observation of the small bowel. This system is a useful diagnostic modality that has the capacity to assess mucosal healing and provide extraluminal information.
ABSTRACT
We analyzed 20 patients diagnosed with autoimmune neurological diseases with seizure predominance. In these patients, we examined the usefulness of Antibody Prevalence in Epilepsy and Encephalopathy (APE2) score and Antibodies Contributing to Focal Epilepsy Signs and Symptoms (ACES) score in autoimmune encephalitis (AE) for facilitating early treatment. APE2 score was positive in 19 of 20 patients. ACES score was positive in 15 of 20 patients, and 4 of 5 of the patients with negative ACES score did not have AE. Comprehensive assessment including the use of the above scores is desirable in the early stage of AE.
Subject(s)
Autoantibodies , Encephalitis , Seizures , Humans , Autoantibodies/blood , Male , Female , Middle Aged , Encephalitis/immunology , Encephalitis/diagnosis , Encephalitis/therapy , Adult , Aged , Seizures/etiology , Seizures/immunology , Hashimoto Disease/immunology , Hashimoto Disease/diagnosis , Hashimoto Disease/complications , Biomarkers/blood , Early Medical Intervention , Young Adult , Adolescent , Aged, 80 and over , Severity of Illness IndexABSTRACT
Autoantibodies against LGI1 and Caspr2 complexed with voltage-gated potassium channels (VGKC) cause limbic encephalitis. Anti-LGI1 encephalitis progresses in a subacute course with memory impairment, disorientation, and focal epileptic seizures. Anti-LGI1 encephalitis is preceded by faciobrachial dystonic seizures (FBDS), which are specific involuntary movements and frequently complicated by hyponatremia due to the syndrome of inappropriate secretion of antidiuretic hormone (SIADH). Neutralization of LGI1 by anti-LGI1 antibodies reduces AMPA receptors and induces epileptic seizures and memory impairment. Anti-Caspr2 encephalitis (Morvan's syndrome) is associated with limbic symptoms, severe autonomic disorders, muscle cramps and burning extremity pain due to peripheral nerve hyperexcitability. Thymomas and other malignant tumors may be complicated, and search for them is necessary. Anti-Caspr2 antibodies bind to Caspr2 on the surface of afferent cells in the dorsal root ganglion, and internalization of VGKCs causes a decrease in the K+ current, resulting in neuronal hyperexcitation and severe pain. Early immunotherapeutic intervention may improve the prognosis of these diseases, and measuring these autoantibodies should be performed in the presence of specific clinical signs, even with normal cerebrospinal fluid findings.
Subject(s)
Encephalitis , Limbic Encephalitis , Potassium Channels, Voltage-Gated , Humans , Membrane Proteins , Nerve Tissue Proteins , Intracellular Signaling Peptides and Proteins/metabolism , Autoantibodies , Limbic Encephalitis/diagnosis , SeizuresABSTRACT
Mononuclear phagocytic cells (MPCs) are classified into monocytes (Mos)/macrophages and dendritic cells (DCs) based on their functions. Cells of MPCs lineage act as immune modulators by affecting effector cells, such as NK cells, T cells, and B cells. This study aimed to investigate the effects of Lacticaseibacillus paracasei strain Shirota (LcS) ingestion on peripheral MPCs, particularly on their expression of functional cell-surface molecules enhanced in healthy adults. Thus, twelve healthy office workers consumed a fermented milk drink containing 1.0 × 1011 cfu of LcS (LcS-FM) or a control unfermented milk drink (CM) once a day for 6 weeks. Peripheral blood mononuclear cells (PBMCs) were prepared from blood samples, and immune cells and functional cell-surface molecules were analyzed. We observed remarkable differences in the expression of HLAABC, MICA, CD40, and GPR43 in plasmacytoid DCs (pDCs) between the LcS-FM and CM groups, whereas no difference was found in CD86 or HLADR expression. The LcS-FM group exhibited higher CD40 expression in both conventional DCs (cDCs) and Mos, especially in type 2 conventional DCs (cDC2s) and classical monocytes (cMos); higher percentages of cMos, intermediate monocytes (iMos), and nonclassical monocytes; and higher numbers of cMos and iMos in PBMCs than the CM group. LcS ingestion increased the expression of HLAABC, MICA, CD40, and GPR43 in pDCs and CD40 in cDCs and Mos, particularly cDC2s and cMos. These results suggest that LcS modulates the function of MPCs that may lead to the regulation of immune effector functions in healthy adults.
ABSTRACT
Background: Satralizumab, a monoclonal antibody that recognizes interleukin-6 receptors, is known to reduce the relapse rate in neuromyelitis optica spectrum disorder (NMOSD), but its safety during pregnancy has not been established. We present the case of an NMOSD patient who safely completed pregnancy, parturition, and breastfeeding under satralizumab treatment. Importantly, satralizumab transfer to umbilical cord blood, infant serum, or breast milk was not observed. Case presentation: A 37-year-old Japanese female developed anti-aquaporin 4 antibody-positive NMOSD with left optic neuritis. Despite responding to steroid and azathioprine therapy, she experienced moon face and weight gain and desired the prompt reduction of the steroid dosage. She also wanted to conceive a child with a safe and preferably early pregnancy and parturition. Because pregnancy and parturition after the onset of NMOSD elevate the risk of relapse and miscarriage, treatment with satralizumab was initiated with the patient's consent. She experienced normal parturition and continued with satralizumab, steroid, and azathioprine treatments while breastfeeding without experiencing any relapses. Concentrations of satralizumab in the umbilical cord blood, infant serum, and breast milk were below the detection sensitivity. Conclusion: These findings suggest that satralizumab may be safe and effective for the perinatal management of NMOSD, especially when there are concerns about continuing pregnancy and the risk of relapse after parturition.
ABSTRACT
Subcutaneous implantable venous ports were placed in 414 patients between April 2016 and August 2021 for the purpose of breast cancer chemotherapy in our hospital. Although the internal jugular vein approach was selected to prevent fractures caused by pinch-off syndrome, catheter fracture occurred in 8 patients(1.9%). All patients were ADL-independent women aged 44-62 years(median, 50.5 years). The intravenous ports were placed on the side of the dominant and non-dominant hands in 4 and 4 patients, respectively. Six patients received perioperative chemotherapy, while 2 had advanced breast cancer. Catheter fractures occurred 17.7-54.2 months(median, 41.7 months)after placement. The fractures were discovered when the patients presented with one or more of the following conditions: poor backflow of blood (n=4), subcutaneous emphysema observed on CT(n=1), subcutaneous leakage of CT contrast media(n=3), and no sign or symptom(n=1). The fractures occurred in the clavicular subcutaneous part in all patients. Partial and complete fractures of the catheter occurred in 5 and 3 patients, respectively. In 3 patients with complete fracture of the catheter, catheter tips had strayed into the right atrium and were removed using percutaneous endovascular procedures.
Subject(s)
Breast Neoplasms , Catheterization, Central Venous , Central Venous Catheters , Breast Neoplasms/drug therapy , Breast Neoplasms/etiology , Breast Neoplasms/surgery , Catheterization, Central Venous/adverse effects , Catheters, Indwelling/adverse effects , Central Venous Catheters/adverse effects , Female , Heart Atria , Humans , Jugular VeinsABSTRACT
We conducted a questionnaire survey of patients with breast cancer who underwent implantation of a subcutaneous central venous port system for chemotherapy; we aimed to evaluate their satisfaction with the port and difficulties in daily life. Overall, 130 patients with breast cancer who underwent port implantation for chemotherapy between 2016 and 2018 responded to our survey. Although the overall satisfaction was high(78.5%), 82(63.1%)patients reported difficulties in daily life, such as"pain or discomfort,""irritation over the implanted area while wearing a seat belt,"and"unable to wear a bag across their bodies."Some patients(10.8%)responded that these negative factors were associated with difficulties in carrying out social roles. The proportion of patients who understood the explanation provided before port implantation was 80.0%, and the satisfaction of these patients tended to be high(85.6%).
Subject(s)
Breast Neoplasms , Catheterization, Central Venous , Breast Neoplasms/drug therapy , Catheterization, Central Venous/adverse effects , Catheters, Indwelling , Female , Humans , Patient Satisfaction , Personal Satisfaction , Surveys and QuestionnairesABSTRACT
BACKGROUND: Chemotherapy in childhood leukemia potentially induces brain lesions and neurological sequelae. Paroxysmal sympathetic hyperactivity (PSH) is known as a treatment-associated complication; however, the full clinical spectra of PSH remain to be elusive. CASE REPORT: A 5-year-old girl was diagnosed of acute myeloid leukemia (AML) M5. After the intensification therapy, she developed recurrent symptoms of episodic tachycardia, hypertension and perspiration lasting for several hours per day. The low-frequency-high-frequency ratio on Holter electrocardiography was rapidly increased from 0.84 to 2.24 at the onset of the paroxysmal event, whereas the video-monitoring electroencephalography (EEG) never identified ictal patterns of epileptiform discharges during the episodes. Thus, the diagnosis of PSH was given at 7 years of age. Myoclonic and generalized tonic-clonic seizures frequently appeared from 10 years of age, which poorly responded to anticonvulsants. EEG showed diffuse slow-wave bursts with multifocal spikes. Serial head magnetic resonance imaging (MRI) revealed diffuse cerebral and hippocampal atrophy, but not inflammatory lesions in the limbic system. CONCLUSION: We first demonstrate a pediatric case with PSH who developed drug-resistant epilepsy 3 years after the onset of PSH. Our data suggest the pathophysiological link of persistent PSH with chemotherapy-associated brain damage.
Subject(s)
Antineoplastic Agents/adverse effects , Autonomic Nervous System Diseases/chemically induced , Drug Resistant Epilepsy/chemically induced , Leukemia, Myeloid, Acute/drug therapy , Neurotoxicity Syndromes/etiology , Child , Female , HumansABSTRACT
Although diet is an important factor influencing gut microbiota, there are very few studies regarding that relationship in Japanese people. Here, we analyzed the relationship between habitual dietary intake surveyed by food frequency questionnaire and the quantitative features of gut bacteria by quantitative PCR and next generation sequencer in 354 healthy Japanese adults. The α-diversity of gut microbiota was positively correlated with the intake of mushrooms and beans and negatively correlated with the intake of grains. The ß-diversity was significantly associated with the intake of fruits, mushrooms, seaweeds, seafoods, and alcoholic beverages. Multiple linear regression analysis of the relationship between food groups associated with the diversity of gut microbiota and the number of gut bacteria at the genus level found 24 significant associations, including a positive association between alcoholic beverages and the number of Fusobacterium. These results support that habitual dietary intake influenced the diversity of gut microbiota and was strongly associated with the number of specific gut bacteria. These results will help us to understand the complex relationship between habitual diet and gut microbiota of the Japanese.
Subject(s)
Colony Count, Microbial/statistics & numerical data , Diet/statistics & numerical data , Eating/physiology , Feeding Behavior/physiology , Gastrointestinal Microbiome/physiology , Adult , Agaricales , Diet/methods , Diet Surveys , Edible Grain , Fabaceae , Female , Healthy Volunteers , High-Throughput Nucleotide Sequencing , Humans , Japan , Linear Models , Male , Middle Aged , Real-Time Polymerase Chain Reaction , Young AdultABSTRACT
BACKGROUND AND AIM: Magnifying endoscopy is useful for diagnosis of early gastrointestinal neoplasms by visualizing microvascular (MV) and microsurface (MS) structures of the mucosa when combined with image-enhanced endoscopy. However, precise control of the endoscope is needed because the depth of focus is narrow and the target may move. These problems may be overcome by the all-in-focus (AIF) technique, which was developed in the engineering field. The aim of the study was to evaluate magnifying endoscopic image with AIF algorithm. METHODS: Twenty gastric neoplasms were examined. Images were acquired at 80× magnification and converted to endoscopic images with an AIF algorithm (EI-AIF). The focus area and MV and MS patterns in the original image and the EI-AIF were compared on a 5-point Likert scale, where 5 indicates that the EI-AIF was superior. Intraclass correlation coefficients (ICCs) were used to assess the inter-evaluator reliability. An image quality measurement value was calculated for each image as an indicator of the degree of focus. RESULTS: The scores for focus area, MV, and MS were 4.78 ± 0.45 (ICC = 0.63), 4.12 ± 0.76 (ICC = 0.70), and 4.72 ± 0.52 (ICC = 0.45), respectively, with the EI-AIF significantly superior for all three items (P < 0.05 by Student's t-test). ICCs for the focus area and MV were > 0.60, indicating strong inter-evaluator reliability. Image quality measurement was higher for the EI-AIF compared with the original image in every case. CONCLUSIONS: Endoscopic observation with AIF algorithm gives a better image quality that allows easier evaluation of MV and MS patterns. This technique may resolve the difficulties with magnifying endoscopic observation.
Subject(s)
Algorithms , Gastroscopy/methods , Stomach Neoplasms/diagnostic imaging , Stomach Neoplasms/pathology , Adult , Aged , Aged, 80 and over , Female , Humans , Male , Middle AgedABSTRACT
Holographic video requires impractical bitrates for storage and transmission without data compression. We introduce an end-to-end compression pipeline for compressing holographic sequences with known ground truth motion. The compression strategy employs a motion compensation algorithm based on the rotational transformation of an angular spectrum. Residuals arising from the compensation step are represented using short-time Fourier transforms and quantized with uniform mid-rise quantizers whose bit depth is determined by a Lagrangian rate-distortion optimization criterion where the distortion metric is the mean squared error. Experiments use computer-generated holographic videos, and we report Bjøntegaard delta peak signal-to-noise ratio gains of around 20 dB when compared to traditional image/video codecs.
ABSTRACT
Whereas in FWDCs, a significantly larger volume and lower density of fluid in the paranasal sinuses (P = 0.0195 and P = 0.0104, respectively), lower density of fluid in the central airways (P = 0.0077), lower stomach content density (P = 0.0216), lower density in the left atrium (P = 0.0029), and a difference of density between the atria (P = 0.0019) were observed.
ABSTRACT
Many researchers suggested that ultrathin endoscopy improves patient acceptance of endoscopic examinations. However, ultrathin endoscopy provides less image resolution and luminous intensity. Therefore, we focused on the visibility of early gastric cancer on ultrathin endoscopy with Flexible spectral imaging color enhancement (FICE) in this study. Thirty-six patients with early gastric cancer were prospectively enrolled. One endoscopist performed the endoscopic examinations by white light conventional endoscopy (W-CE), white light ultrathin endoscopy (W-UE), FICE ultrathin endoscopy (F-UE) and white light plus FICE ultrathin endoscopy (WF-UE) in the patients. Four other endoscopists were asked to evaluate the visibility of gastric cancer on the W-CE, W-UE, F-UE and WF-UE images with a 5-point Likert scale. The lesions were classified as uncolored, normocolored or reddish. We examined the color difference between early gastric cancer and the surrounding mucosa. To examine the relationship between the color difference and the vessel density, we also measured the difference in vessel density using pathologic specimens stained with hematoxylin and eosin. The Likert score of WF-UE was significantly higher than those of the other three methods (p<0.001). The color difference of F-UE was higher than that of W-CE in the reddish group (p=0.049). The difference in vessel density was higher in the reddish group than in the normocolored group (p=0.048). In conclusion, the visibility of early gastric cancer from the surrounding mucosa using ultrathin endoscopy with FICE was better than that using white light conventional endoscopy, especially for reddish lesions.
Subject(s)
Endoscopy, Gastrointestinal/methods , Stomach Neoplasms/diagnostic imaging , Aged , Aged, 80 and over , Blood Vessels/diagnostic imaging , Female , Humans , Male , Middle Aged , Stomach Neoplasms/blood supplyABSTRACT
Evaluation of the pancreatic elastic modulus (PEM) using shear wave elastography (SWE) requires at least 5 measurements to ensure reproducibility. The aim of this study was to evaluate improvement in reproducibility of SWE, using the propagation display method in normal pancreas ([NP] phase 1) and to examine the differences in PEM between NP and chronic pancreatitis (CP), intraductal papillary mucinous neoplasm (IPMN) and autoimmune pancreatitis ([AIP] phase 2). In phase 1, the measurement success rate, median PEM in repeated measurements and appropriate number of SWE measurements were determined in 109 cases with NP. In phase 2, PEM was measured in CP (nâ¯=â¯10), IPMN (nâ¯=â¯31) and AIP (nâ¯=â¯5), using the required number of SWE measurements determined in phase 1. In phase 1, the measurement success rate was 93.9% (92/109 cases). The median PEM for NP was 14.6 kPa and the appropriate number of SWE measurements was at least 3. In phase 2, the median PEMs in CP, IPMN and AIP were 19.6, 18.1 and 17.2 kPa, respectively, with significant differences between NP and CP (pâ¯=â¯0.0133) and between NP and IPMN (pâ¯=â¯0.0436). Use of the propagation display method in SWE improves the reproducibility of measurement of PEM.
Subject(s)
Autoimmune Pancreatitis/diagnostic imaging , Elasticity Imaging Techniques/methods , Image Interpretation, Computer-Assisted/methods , Pancreatic Intraductal Neoplasms/diagnostic imaging , Pancreatitis, Chronic/diagnostic imaging , Adult , Aged , Aged, 80 and over , Autoimmune Pancreatitis/physiopathology , Diagnosis, Differential , Elastic Modulus/physiology , Female , Humans , Male , Middle Aged , Pancreas/diagnostic imaging , Pancreas/physiology , Pancreatic Intraductal Neoplasms/physiopathology , Pancreatitis, Chronic/physiopathology , Reproducibility of ResultsABSTRACT
We report a case of breast cancer with cartilaginous differentiation that responded well to chemotherapy, which completely eliminated distant metastasis. A 63-year-old woman visited our hospital complaining of a large hemorrhagic mass measuring 15 cm in diameter with ulceration of the left breast. Palpation revealed swelling of the left axillary and right supraclavicular (SC)lymph nodes, suggesting breast cancer metastasis. A CT scan revealed metastasis in the right lung measuring 2.5 cm in size. She underwent a total mastectomy with axillary dissection. The pathological findings were as follows; breast carcinoma with cartilaginous differentiation accompanied by a single lymph node metastasi(s 1/21)and skin involvement, ly0, v0, ER(-), PgR(-), HER2 0, Ki-67 80%. Four courses of AC therapy were administered as postoperative chemotherapy, which resulted in a decrease in the size of the SC lymph node to 1 cm. Subsequently, 12 courses of weekly paclitaxel yielded a complete response of the lung and SC lymph node metastasis. Oral administration of S-1 after paclitaxel therapy resulted in no recur- rence for 16 months after the operation.
Subject(s)
Breast Neoplasms , Axilla , Breast Neoplasms/pathology , Breast Neoplasms/therapy , Cartilage , Female , Humans , Lymphatic Metastasis , Mastectomy , Middle Aged , Neoplasm Recurrence, LocalABSTRACT
PURPOSE: To investigate the effectiveness of postmortem computed tomography in the diagnosis of drowning, focusing on the comparison of fresh water and salt water cases using three-dimensionally (3D) reconstructed data. MATERIALS AND METHODS: We examined features of drowning in 25 fresh water drowning cases (FWDCs; 13 men, 12 women; mean age 73.1 years; range 43-95 years), and compared these with 12 salt water drowning cases (SWDCs; 5 men, 7 women; mean age 66.0 years; range 55-77 years). Pulmonary opacities, volume and density (CT number) of accumulated fluid in the paranasal sinuses and central airways, volume of the stomach/stomach contents, and cardiac blood density were examined. RESULTS: In SWDCs, pulmonary ground-glass opacities with wholly thickened interstitium was frequently identified (P = 0.0274). Whereas in FWDCs, a significantly larger volume and lower density of fluid in the paranasal sinuses (P = 0.0195 and P = 0.0104, respectively), lower density of fluid in the central airways (P = 0.0077), lower stomach content density (P = 0.0216), lower density in the left atrium (P = 0.0029), and a difference of density between the atria (P = 0.0247) were observed. CONCLUSIONS: A lower density in the left atrium was observed in FWDCs compared to SWDCs. This finding may be helpful in differentiating between FWDCs and SWDCs.
Subject(s)
Drowning/diagnosis , Fresh Water , Imaging, Three-Dimensional/methods , Seawater , Tomography, X-Ray Computed/methods , Adult , Aged , Aged, 80 and over , Autopsy , Female , Heart/diagnostic imaging , Humans , Lung/diagnostic imaging , Male , Middle Aged , Paranasal Sinuses/diagnostic imaging , Respiratory System/diagnostic imaging , Stomach/diagnostic imagingABSTRACT
Importance: Although acquired autoimmune neuromyotonia (NMT) is associated with voltage-gated potassium channel (VGKC)-complex antibodies, to date there has been no systematic study of autoantibodies to the specific antigens leucine-rich glioma inactivated protein 1 (LGI1), contactin-associated protein 2 (CASPR2), and contactin 2 together with the full clinical syndrome, particularly pain and autonomic and central nervous system involvement. Objectives: To study the full spectrum of clinical features and serum autoantibodies in patients with NMT, including the effects of pain on quality of life. Design, Setting, and Participants: A cohort study of clinical features and serologic testing in 38 patients with electrophysiologically-confirmed NMT, reviewed clinically between February 2007 and August 2009, in the Universities of Sydney and Kagoshima and followed up across 2 to 4 years. Association of NMT with quality of life was researched in an independent, patient-led, online pain survey conducted from April 2012 to May 2012. Serologic analyses were performed in 2012, and final data analysis was performed in 2016. Main Outcomes and Measures: Clinical data and scores on the modified Rankin Scale (mRS), which measures disability on a range of 0 to 6, with 0 indicating normal and 6 indicating death, before and after treatments were combined with CASPR2, LGI1, and contactin 2 antibody status. Results: Among the 38-person NMT cohort, 25 (65.8%) were male and the median (range) age was 55 (12-85) years. Twenty-three (60.5%) were Japanese and 15 (39.5%) were of white race/ethnicity. Symptomatic treatments (mainly antiepileptic drugs) were used in most patients with mild disease (12 patients with mRS <3), whereas immunotherapies were successful in most patients with mRS scores greater than 2. Autoantibodies to VGKC-complex antigens (17 patients [45%]), bound to CASPR2 (5 [13%]), contactin 2 (5 patients, 1 with CASPR2 [13%]), LGI1 (2 [5%]), or both LGI1 and CASPR2 (6 [16%]). The last group of 6 patients had high mRS scores (mean [SD], 3.8 [1.7]), thymoma (4 patients), pain (5 patients), autonomic (6 patients) and sleep (5 patients) disturbance, suggesting Morvan syndrome. The 56 responders to the independent patient-led survey reported pain that could be severe, anatomically widespread, and that often resulted in unemployment, domestic problems, and poor quality of life. Conclusions and Relevance: The cohort study detailed underrecognized aspects of the clinical and serologic spectrum of NMT. The heterogeneity of clinical features and of specific antibodies limit associations, but the common existence of thymoma, pain, and autonomic and central nervous system features, often with both LGI1 and CASPR2 antibodies, should be better recognized to more completely address the range of comorbidities and consequences of the disease regarding quality of life.
Subject(s)
Autoantibodies/blood , Contactin 2/immunology , Isaacs Syndrome , Membrane Proteins/immunology , Nerve Tissue Proteins/immunology , Pain/physiopathology , Patient Reported Outcome Measures , Proteins/immunology , Adolescent , Adult , Aged , Aged, 80 and over , Child , Cohort Studies , Female , Humans , Intracellular Signaling Peptides and Proteins , Isaacs Syndrome/blood , Isaacs Syndrome/complications , Isaacs Syndrome/immunology , Isaacs Syndrome/physiopathology , Male , Middle Aged , Quality of Life , Young AdultABSTRACT
BACKGROUND AND STUDY AIMS: To decrease complications associated with preoperative endoscopic nasobiliary drainage (ENBD) for perihilar cholangiocarcinoma patients, we developed a modified 6-Fr ENBD catheter with multiple side holes (m-ENBD). The aim of this retrospective study was to compare the m-ENBD catheter with a conventional 7-Fr ENBD catheter (c-ENBD). PATIENTS AND METHODS: This study involved 371 patients with suspected perihilar cholangiocarcinoma who underwent ENBD using a c-ENBD catheter or an m-ENBD catheter. The effectiveness of each catheter and the incidence of complications were evaluated. Univariate and multivariate analyses followed by propensity score matching were performed. RESULTS: In 145 patients with total bilirubin levels ≥â2.0âmg/dL prior to drainage, these levels decreased to <â2.0âmg/dL after ENBD in 81.1â% of the c-ENBD patients and in 74.0â% of the m-ENBD patients ( P â=â0.325). Post-ENBD cholangitis occurred in 24.9â% of the c-ENBD patients and in 12.4â% of the m-ENBD patients ( P â=â0.006). After propensity score matching, the rate of post-ENBD cholangitis ( P â=â0.007) and the number of patients requiring subsequent or additional drainage ( P â=â0.030) were significantly lower in the m-ENBD group. CONCLUSION: The modified 6-Fr ENBD catheter was associated with a lower incidence of post-ENBD cholangitis than the conventional 7-Fr ENBD catheter, and the incidence of subsequent or additional drainage procedures was also decreased.
ABSTRACT
Fecal calprotectin (FC) has drawn attention as a biomarker in the evaluation of Crohn's disease (CD). However, few reports have provided a detailed examination of the relationship between small-bowel CD lesions and FC levels. The present study aimed to examine the entire small bowel using double-balloon endoscopy (DBE) and to determine the relationship between the endoscopic activity in small-bowel CD and FC levels. Twenty small-bowel CD patients, who underwent DBE, were prospectively enrolled. Endoscopic evaluation was based on the simple endoscopic score for CD, with the small bowel divided into four regions. This score was defined as the double-balloon endoscopic score for CD (DES-CD). Furthermore, to focus on mucosal membrane damage, we used the partial DES-CD (pDES-CD), in which presence of stenosis was excluded from DES-CD. DES-CD revealed a correlation with FC (γ = 0.691, P = 0.001) and C-reactive protein (CRP) (γ = 0.631, P = 0.003) levels. Furthermore, pDES-CD showed a correlation with the FC level (γ = 0.747, P < 0.001), erythrocyte sedimentation rate (γ = 0.492, P = 0.028), and the CRP level (γ = 0.605, P = 0.005). CD Activity Index and endoscopic score showed no correlation. Our results revealed a correlation between the endoscopic activity in small-bowel CD and FC levels. Furthermore, pDES-CD showed a strong correlation with FC levels. This may be because FC levels were elevated due to mucosal membrane damages, rather than stenoses.