Thymoma Exhibiting Spontaneous Regression With Cystic Change Due to Acute Infarction: A Case Report and Literature Review.

Spontaneous regression (SR) of thymoma is rare. We report a case of a surgically resected thymoma due to cystic changes owing to acute ischemic infarction with an increased anti-acetylcholine receptor antibody level. A 61-year-old male underwent a computed tomography (CT) scan, which showed a 4.9 cm anterior mediastinal tumor and slight right pleural effusion. Blood test results indicated an elevated white blood cell count of 13300/mL. One month later, an enhanced CT scan at our hospital showed spontaneous mediastinal tumor regression to 3.7 cm and no pleural effusion. The tumor contained homogeneous low-density areas on enhanced CT, which showed high intensity on T2-weighted magnetic resonance imaging, indicating cystic changes. He had no symptoms of myasthenia; however, his anti-acetylcholine receptor antibody level was slightly elevated (0.4 nmol/L). Suspecting a thymoma, an extended total thymectomy through a median sternotomy was performed. Histopathological analysis confirmed the diagnosis of thymoma type B2 and Masaoka stage I. SR is due to acute intratumoral infarction. At two years postoperatively, no tumor recurrence or development of myasthenia gravis was observed. Thymomas should be included in the differential diagnosis of anterior mediastinal tumors that regress spontaneously with cystic changes, pleural effusion, and an elevated inflammatory response. Mature cystic teratoma rupture should be differentiated, but preoperative biopsy is often challenging owing to necrotic and fibrous tissues; therefore, early surgical resection is required for diagnosis and treatment.

[Surgically Resected Cases of Mediastinal Ganglioneuroma Detected in Adults].

A ganglioneuroma is a rare, benign, neurogenic tumor originating from the sympathetic ganglion. Mediastinal ganglioneuroma are mostly detected in children, typically around 10 years of age, and are rarely identified in adults. Herein, we report two surgically resected cases of mediastinal ganglioneuroma in adults. In Case 1, a 53-year-old man, without any symptom, underwent a computed tomography, revealing a 3.2 cm well-defined paravertebral superior mediastinal tumor with long craniocaudal axis. In case 2, a 29-year-old woman presented with newly-developed ptosis and a history of left-sided facial hypohidrosis since the age of 10. Chest computed tomography (CT) revealed a 7.8 cm well-defined paravertebral superior mediastinal tumor with long craniocaudal axis. Both patients were initially suspected to have neurogenic tumors, particularly schwannomas. They underwent mediastinal tumor resections, requiring sympathetic nerve trunk dissection. Pathological examination confirmed the diagnosis of ganglioneuromas in both cases. Mediastinal ganglioneuroma must be differentiated from schwannoma, the most common neurogenic tumor in adults. Unlike schwannoma, ganglioneuroma cannot be enucleated, therefore attention should be focused on complications associated with sympathetic nerve trunk dissection, such as Horner's syndrome, hyperhidrosis, and arrhythmia. Identifying this rare entity and its characteristic imaging aids in preoperative differentiation, strategizing surgical approaches, and predicting complications.

Efficacy and Safety of the Natural Killer T Cell-Stimulatory Glycolipid OCH-NCNP1 for Patients With Relapsing Multiple Sclerosis: Protocol for a Randomized Placebo-Controlled Clinical Trial.

BACKGROUND: Multiple sclerosis (MS) is an autoimmune inflammatory disease of the central nervous system that causes myelin sheath damage and axonal degeneration. The glycolipid (2S, 3S, 4R)-1-O-(α-d-galactosyl)-2-tetracosanoylamino-1,3,4-nonaetriol (OCH-NCNP1 or OCH) exerts an immunoregulatory action that suppresses T helper (Th)1 cell-mediated immune responses through natural killer T cell activation, selective interleukin-4 production, and Th2 bias induction in human CD4-positive natural killer T cells. OBJECTIVE: This trial aims to investigate the efficacy and safety of the immunomodulator OCH in patients with relapsing MS through 24-week repeated administration. METHODS: This protocol describes a double-blind, multicenter, placebo-controlled, randomized phase II clinical trial that was initiated in September 2019. The participants were randomly assigned to either a placebo control group or an OCH-NCNP1 group and the investigational drug (3.0 mg) was orally administered once weekly for the 24-week duration. Major inclusion criteria are as follows: patients had been diagnosed with relapsing MS (relapsing-remitting and/or secondary progressive MS) based on the revised McDonald criteria or were diagnosed with MS by an attending physician as noted in their medical records; patients with at least two medically confirmed clinical exacerbations within 24 months prior to consent or one exacerbation within 12 months prior to consent; patients with at least one lesion suspected to be MS on screening magnetic resonance imaging (MRI); and patients with 7 points or less in the Expanded Disability Status Scale during screening. Major exclusion criteria are as follows: diagnosis of neuromyelitis optica and one of optic neuritis, acute myelitis, and satisfying at least two of the following three items: (1) spinal cord MRI lesion extending across at least three vertebral bodies, (2) no brain MRI lesions during onset (at least four cerebral white matter lesions or three lesions, one of which is around the lateral ventricle), and (3) neuromyelitis optica-immunoglobulin G or antiaquaporin-4 antibody-positive. Outcome measures include the primary outcome of MRI changes (the percentage of subjects with new or newly expanded lesions at 24 weeks on T2-weighted MRI) and the secondary outcomes annual relapse rate (number of recurrences per year), relapse-free period (time to recurrence), sustained reduction in disability (SRD) occurrence rate, period until SRD (time to SRD occurrence), no evidence of disease activity, and exploratory biomarkers from phase I trials (such as gene expression, cell frequency, and intestinal and oral microbiome). RESULTS: We plan to enroll 30 patients in the full analysis set. Enrollment was closed in June 2021 and the study analysis was completed in March 2023. CONCLUSIONS: This randomized controlled trial will determine whether OCH-NCNP1 is effective and safe in patients with MS as well as provide evidence for the potential of OCH-NCNP1 as a therapeutic agent for MS. TRIAL REGISTRATION: ClinicalTrials.gov NCT04211740; https://clinicaltrials.gov/study/NCT04211740. INTERNATIONAL REGISTERED REPORT IDENTIFIER (IRRID): DERR1-10.2196/46709.

Atypical type A thymoma component identified by pulmonary metastasectomy 11 years after surgery of type AB thymoma.

Atypical type A thymomas exhibit more aggressive features than conventional type A thymomas. Type AB thymomas rarely have atypical type A components. We report a rare case of type AB thymoma with an atypical type A component, that was identified after pulmonary metastasectomy 11 years after the primary surgery and long-term follow-up after recurrence. A 61-year-old female underwent extended thymectomy for an anterior mediastinal tumor 11 years prior and was diagnosed with type AB thymoma (Masaoka stage II). Five years ago, follow-up computed tomography showed well-circumscribed pulmonary nodules up to 1.0 cm in both lungs. All the pulmonary nodules grew slowly; however, one of the nodules grew to 1.6 cm, and thoracoscopic wedge resection was performed for diagnosis. Pathologically, the pulmonary nodule was consisted of type A thymoma component. Conventional type AB thymomas are usually locally aggressive neoplasms; thus, we reviewed the tissue slides of primary thymomas. Histologically, cytological atypia, hypercellularity, and increased mitosis are observed in the type A component. Consequently, the diagnosis was revised to a type AB thymoma with an atypical type A component. The pulmonary nodule exhibited the same atypical type A features. Pulmonary metastasectomy was performed two more times as volume-reduction surgery. The residual metastasis was located only in the lung with slow growth, 4 years after the first pulmonary resection; therefore, we followed up as an outpatient without treatment.

Multifocal Cholesterol Granulomas of the Thymus: A Case Report of Positron Emission Tomography- Positive Benign Tumor of the Anterior Mediastinum.

Cholesterol granulomas of the thymus are extremely rare benign entities. Herein, we describe a case of cholesterol granuloma in a 45-year-old man who presented with multifocal anterior mediastinal lesions on computed tomography (CT). Positron emission tomography (PET)-CT revealed increased fluorodeoxyglucose uptake, with a maximum standardized uptake value of 8.3. Diffusion-weighted magnetic resonance imaging (MRI) revealed extreme hypointensity. He underwent total thymectomy by median sternotomy for presumed multiple thymoma. On histopathological analysis, cholesterol granuloma of the thymus was confirmed, and the patient had an uneventful postoperative course without recurrence for 28 months. Cholesterol granuloma of the thymus is a PET-CT-positive benign tumor with unique histological findings. The MRI findings were indicative of hypocellularity associated with a benign entity, whereas the PET-CT findings corresponded to granulomatous inflammation. Therefore, concurrent use of PET-CT and MRI can be helpful in distinguishing between benign cholesterol granulomas and malignant anterior mediastinal tumors.

Pulmonary metastasis of matrix-producing carcinoma mimicking small cell lung cancer.

Matrix-producing carcinoma (MPC) of the breast is an extremely rare subtype of invasive breast cancer. MPC is characterized by the production of a cartilaginous or osseous matrix without spindle cells. It is an aggressive carcinoma, often presenting as triple-negative breast cancer. MPC frequently metastasizes to the lungs; however, it rarely reoccurs as a solitary pulmonary metastasis. We report a case of a 77-year-old non-smoking woman with a solitary pulmonary metastasis of MPC, which mimicked small cell lung cancer (SCLC). Initially, the pulmonary metastasis was misdiagnosed as SCLC based on its morphological and immunochemical features, including focal positivity for CD56 and thyroid transcription factor-1. Although the portion of the chondromyxoid matrix of the primary site was not small, that of the metastatic site was small. A focal cartilaginous matrix deposition of pulmonary metastasis from MPC could make it difficult to differentiate from SCLC. We should acknowledge that the portion of chondromyxoid matrix may differ between primary and distant metastatic sites in MPC.

Successful Video-Assisted Thoracoscopic Removal of a Chest Tube Protruding Deep Into the Pulmonary Hilum.

Tube thoracostomy is an invasive procedure frequently used to drain pleural fluid collections or to manage pneumothorax, wherein the lungs commonly sustain trauma. In some cases, deep pulmonary hilar injuries are managed by anatomical lung resections. A deep hilar injury with the chest tube protruding into the lung parenchyma is a rare complication of tube thoracostomy. We report the case of a patient with tube thoracostomy-related deep pulmonary laceration treated using video-assisted thoracic surgery (VATS). A 74-year-old man with a left-sided pneumonia-associated empyema underwent tube thoracostomy for drainage of intrathoracic purulent collection at another hospital; however, chest radiography and computed tomography (CT) revealed intrusion of the 22 Fr chest tube into the left lower lobe parenchyma for approximately 10 cm toward the pulmonary hilum, with the chest tube tip located near the left main bronchus and pulmonary artery. Although no massive intrapulmonary hemorrhage, pneumothorax, or pneumomediastinum was observed, multiple pyothoracic cavities were present. He was transferred to our hospital the following day in a hemodynamically stable condition. The next day, he underwent both surgical chest tube removal and decortication for empyema. Owing to the worsening of his physical condition and due to prolonged severe inflammation and lack of appetite, without any sign of bleeding or chest tube air leak, a two-port VATS with sparing of the lung parenchyma was attempted. After decortication, the penetrating chest tube was slowly removed. No hemorrhage or air leaks were observed at the site of penetration, requiring no sutures or dressing. Following his uneventful postoperative course, he was discharged on day 9. In selected cases, anatomic lung resection can be avoided, even for deep hilar injuries, depending upon the degree of intrapulmonary hemorrhage and the presence of air leak from the chest tube, and the CT scan findings.

Thymoma exhibiting spontaneous regression with developing myasthenia gravis: A case report.

Spontaneous regression (SR) of thymoma is rare. A 44-year-old man with right chest pain underwent computed tomography (CT), which showed an 11.0 cm mass in the anterior mediastinum and right pleural effusion. He refused surgery and was sent home without medication and additional treatment. One year later, the mass had regressed to 5.5 cm, and the right pleural effusion had disappeared. He was then lost to follow-up. Four years after the initial visit, he presented with diplopia and fatigue. A significant increase in his anti-acetylcholine receptor antibody levels led to myasthenia gravis (MG) diagnosis. CT revealed a regressed mediastinal mass (3.0 cm). After extended thymectomy, histologic analysis confirmed a thymoma type B2, Masaoka stage IIa. The SR was due to intratumoral infarction. This report is the first to describe MG developing during SR. Anterior mediastinal tumors undergoing SR should be differentiated from thymomas and MG perioperative development should be considered.

Extra-muscular manifestations in GNE myopathy patients: A nationwide repository questionnaire survey in Japan.

OBJECTIVE: GNE myopathy is a rare autosomal recessive adult-onset distal myopathy caused by biallelic pathogenic variants in GNE. Although some extra-muscular manifestations associated with GNE myopathy have been reported, little is known about whether they are disease-specific and how often they present. This study aimed to characterize extra-muscular manifestations of GNE myopathy. METHODS: We conducted a questionnaire survey of GNE myopathy patients registered in a national registry in Japan. The questionnaire requested information regarding idiopathic thrombocytopenia, cardiac involvement, respiratory involvement, sleep apnea syndrome (SAS), and psychiatric diseases. RESULTS: The response rate was 62.4% (126/198), yielding a total of 51 male and 75 female participants. Of the participants, 4.1% (5/123) had a diagnosis of idiopathic thrombocytopenia, and 16.3% (8/49) of males and 6.6% of females (5/76) had a diagnosis of SAS. In total, 0.8% (1/126) of participants had pervasive developmental disabilities and 14.7% (16/109) had a psychiatric disease. CONCLUSION: The frequencies of idiopathic thrombocytopenia and SAS among Japanese GNE myopathy patients were higher than those observed in the general Japanese population. Routine blood tests and evaluation of sleep-disordered breathing should be considered in order to better manage GNE myopathy patients.

Patient characteristics and burden of disease in Japanese patients with generalized pustular psoriasis: Results from the Medical Data Vision claims database.

Generalized pustular psoriasis (GPP) is a rare and severe systemic, neutrophilic skin disease. To date, accurate clinical profiling of patients with GPP remains poorly understood. In this study, we present the characteristics and estimate the burden of disease in patients with GPP compared with those with plaque psoriasis, in Japan. This retrospective study was conducted using the Medical Data Vision database between January 1, 2015, and December 31, 2019. Patients with at least one confirmed inpatient or outpatient diagnostic code for GPP (L40.1) or psoriasis vulgaris (L40.0) were included for analysis. The main outcome measures included comparisons of the prevalence of comorbidities, medication use, and healthcare resource utilization between patients with GPP, patients with plaque psoriasis, and a general population-matched cohort. In total, 718 patients with GPP and 27,773 patients with plaque psoriasis were identified. Patients with GPP were more likely to be female than those with plaque psoriasis (51.6% vs. 38.7%). During the 12-month follow-up period, patients with GPP were more likely to experience comorbidities than those with plaque psoriasis, including psoriatic arthritis, other forms of psoriasis, osteoporosis, interstitial pneumonia, and peptic ulcer disease. Medication use also differed between those with GPP and those with plaque psoriasis: patients with GPP were more likely to be prescribed antibiotics and psychiatric medication. Patients with GPP were also more likely to require more healthcare resource utilization with longer hospitalizations than those with plaque psoriasis. Overall, in Japan, patients with GPP have a higher burden of illness than those with plaque psoriasis.

Feasibility of intraoperative diagnosis of lung adenocarcinoma in situ to avoid excessive resection.

BACKGROUND: Limited lung resection is generally believed to be available for lung adenocarcinoma in situ (AIS). At our institute, intraoperative hematoxylin-eosin staining of frozen-section slides is routinely performed for evaluating tumor invasiveness after partial resection to avoid excessive lung resection. This study aimed to evaluate the feasibility and usefulness of intraoperative frozen-section diagnosis of AIS. METHODS: We retrospectively reviewed 143 patients with 151 AISs diagnosed by intraoperative frozen sections between 2012 and 2019 at our institute. All patients underwent limited resection because of the result of intraoperative frozen-section diagnosis. RESULTS: The total concordance rate between the diagnoses of AIS by intraoperative frozen sections and postoperative paraffin-embedded sections was 82.7% for 151 nodules. Although 21 minimally invasive adenocarcinomas (MIA) and 5 invasive adenocarcinomas were diagnosed as AIS intraoperatively, no patient had tumor recurrence after resection. Among 125 pathologically proven cases of AIS postoperatively, there were 67 (53.6%) radiologically invasive tumors including ground-glass nodules (GGNs) with part-solid component or pure-solid nodules. CONCLUSIONS: This intraoperative evaluation of frozen-section slides will help surgeons avoid excessive lung resection for AIS that was radiologically diagnosed as invasive adenocarcinoma. Intraoperative frozen-section diagnosis will provide to be clinically useful and lead to less invasive surgical treatment for lung nodules.

Lymphatic permeation and vascular invasion should not be integrated as lymphovascular invasion in lung adenocarcinoma.

OBJECTIVE: Lymphatic permeation (LY) and vascular invasion (VI) are well-known as postoperative prognostic factors in non-small cell lung cancer (NSCLC). Some reports use the term "lymphovascular invasion (LVI)" in reference to the integration of LY and VI. The purpose in this study is to elucidate whether NSCLC with LY or VI can be labeled as LVI-positive NSCLC or it should be treated as an LY- or VI-positive tumor, respectively. METHODS: We reviewed 601 completely resected lung adenocarcinomas and squamous cell carcinomas, and examined the respective prognostic and biological significance of LY and VI. RESULTS: Among 454 adenocarcinomas, multivariate survival analyses showed that LY and VI were unfavorable prognostic factors in stages II and III and stages I and II, respectively. Conversely, this relationship was not found among 147 squamous cell carcinomas. Patients with adenocarcinomas with LY and VI had a significantly worse prognosis than those with adenocarcinomas with LY or VI in stage II, but not in stage I. Among 149 recurrent adenocarcinomas, only VI had a strong effect on early recurrence and shorter post-recurrence survival. LY and VI were predictors for multiple organ recurrence of adenocarcinoma. The recurrence of adenocarcinoma with LY was high in the ipsilateral mediastinal lymph nodes, whereas VI was significantly correlated with distant metastasis to organs, such as the brain, liver, and adrenal gland. CONCLUSIONS: LY and VI have differing effects postoperative prognosis and recurrence-relevant events, suggesting that these pathological findings should not be integrated as LVI.

How long is cessation of preoperative smoking required to improve postoperative survival of patients with pathological stage I non-small cell lung cancer?

BACKGROUND: Smoking can cause non-small cell lung cancer (NSCLC). However, the effects of preoperative smoking on tumor progression are not well-known. In addition, the duration of smoking cessation that can provide NSCLC patients with smoking history similar postoperative prognosis as that of nonsmokers remains unknown. This study aimed to investigate the period of smoking cessation that may "compensate" for past smoking history regarding postoperative survival in cases of resected pathological stage I NSCLC by examining the relationship between clinicopathological factors and preoperative smoking. METHODS: We retrospectively examined clinicopathological factors including preoperative smoking status and postoperative survival in 453 patients with pathologically proven stage I NSCLC at our Institute. Smoking status was evaluated using the following four parameters: cigarettes per day, number of years of smoking, pack-years, and number of years since smoking cessation. RESULTS: Pathological factors that reflect tumor invasiveness including vascular invasion (VI) and pleural invasion (PL) were associated with the degree of preoperative smoking in adenocarcinomas, particularly invasive diameters of 2-3 cm. Such a relationship was not identified for non-adenocarcinomas. Heavy smoking status was significantly related to PL in lepidic or papillary predominant adenocarcinomas and to VI in acinar or solid predominant adenocarcinomas. Former smokers who quit smoking for ≥10 years had similar postoperative survival as non-smokers for adenocarcinoma ≤3 cm. CONCLUSIONS: Accumulative smoking habit correlated with VI and PL, particularly in 2-3 cm adenocarcinoma, whereas larger adenocarcinomas and non-adenocarcinomas of any size appear to grow and become invasive independent of preoperative smoking status. Longer smoking cessation ≥10 years can result in postoperative survival similar to that of non-smokers with adenocarcinomas ≤3 cm. Current smokers should quit smoking immediately to ensure longer survival even though they suffer from small-sized lung adenocarcinomas in the future.

Pregnancy in GNE myopathy patients: a nationwide repository survey in Japan.

BACKGROUND: GNE myopathy is an autosomal recessive adult-onset distal myopathy. While a few case reports have described the progression of GNE myopathy during pregnancy, to our knowledge, none have examined disease progression after delivery or obstetric complications. OBJECTIVE: This study aimed to reveal maternal complications, newborn complications, and the impact of pregnancy on disease progression in GNE myopathy patients. METHODS: We conducted a questionnaire survey on pregnancy, delivery, and newborns involving female GNE myopathy patients who are currently registered in a national registry in Japan. RESULTS: The response rate for the questionnaire survey was 60.0% (72/120). Of the 72 respondents, 44 (61.1%) had pregnancy experience (average, 1.8 pregnancies; 53 pregnancies before onset and 28 after onset). The incidence of threatened abortion was 26.9% among post-onset pregnancies, which was higher compared to those of the general Japanese population (p = 0.03). No other maternal or infant complications were commonly observed. Over 80% were unaware of changes in disease progression during pregnancy (mean age, 32.8 ± 3.5 years) or after delivery (32.9 ± 3.8 years), while 19.0% experienced disease exacerbation within a year after delivery (30.0 ± 1.0 years). Six patients developed myopathy within a year after delivery (29.7 ± 4.6 years), while none developed myopathy during pregnancy. CONCLUSIONS: There were no serious maternal or newborn complications, and subjective progression did not differ during or after delivery in the majority of GNE myopathy patients. However, our findings suggest the importance of considering the possibility of threatened abortion and disease progression after delivery.

Clinicopathological Factors Related to Recurrence Patterns of Resected Non-Small Cell Lung Cancer.

Even after complete resection, non-small cell lung cancer (NSCLC) shows preferential recurrence in the mediastinal lymph nodes, lungs, brain, bone, liver, and adrenal gland. However, the relationship between clinicopathological factors and recurrence patterns after resection has not been well-evaluated. Among 688 NSCLC cases with complete resection between 2004 and 2016, 233 cases recurred at our institute. On multivariate analyses, NSCLCs with lymph node metastasis and pulmonary metastasis at surgery commonly recurred in the mediastinal lymph nodes and lungs, respectively. Young age, adenocarcinoma, and vascular invasion were correlated with brain metastasis. Although no variable was associated with bone metastasis, vascular invasion was correlated with postoperative liver and adrenal gland metastasis. Pathologically proven stage II or III NSCLC, adenocarcinoma, and the presence of lymphatic permeation would result in multiple metastases. Vascular invasion, larger invasive size, and advanced stage were independent risk factors of early recurrence. Considering survival, vascular invasion, elderly age, and non-adenocarcinoma were unfavorable prognostic factors after recurrence. Some clinicopathological variables were correlated with organ-specific metastasis and post-recurrence survival. Particularly, vascular invasion was a biomarker of brain, liver, and adrenal gland metastases and a prognostic marker after recurrence among completely resected NSCLC. This information is useful for more frequent patient follow-up and identifying organ-specific distant metastasis.

Expectations and anxieties of Duchenne muscular dystrophy patients and their families during the first-in-human clinical trial of NS-065/NCNP-01.

Duchenne muscular dystrophy (DMD) is a recessive X-linked genetic disease caused by a mutation in the dystrophin gene. The new drug NS-065/NCNP-01 utilizing exon-skipping therapy targeting specific deletions has been used in a first-in-human trial for the treatment of DMD. We surveyed 10 pairs of DMD participants and their parents within this clinical trial via an iPad survey form and through interviews regarding their understanding of the trial, expectations, anxieties, and reasons for participating in the trial. Approximately half of the participants actively decided to participate of their own volition, and none considered quitting the trial. This indicates that participants participated more positively in this clinical trial than previously expected. However, some potential concerns were also revealed, with one being that the desire to please those around them might be more important to the DMD participants than the effects of the drug. Another issue is the possibility of biased information originating from the study subjects' parents; while seven out of 10 of the parents told their children that the study drug might work, only four of these parents also explained that it might not work. Only two study participants received an explanation concerning the drug's side effects from their parents. This result implies that caution should be taken when family expectations are high, and there is a possibility that subjects will be given biased information from their parents.

Quantitative computed tomography for predicting cardiopulmonary complications after lobectomy for lung cancer in patients with chronic obstructive pulmonary disease.

OBJECTIVES: In lung cancer resection, chronic obstructive pulmonary disease is a risk factor for post-operative complications. Few studies on post-operative complications of lung cancer resection have considered radiographic emphysematous change as an index. Here, we have examined the relationship between the regional ratio of the emphysematous area in pre-operative computed tomography images and cardiopulmonary complications in patients with chronic obstructive pulmonary disease who underwent lung cancer resection. METHODS: We retrospectively evaluated 159 patients with chronic obstructive pulmonary disease who underwent lobectomy for lung cancer at Shizuoka Cancer Center Hospital, Shizuoka, Japan, between 2002 and 2011. Pre-operative factors, including the proportion of the emphysematous area measured by computed tomography as a percentage of the low attenuation area (LAA%), as well as intraoperative factors were analyzed. Cardiopulmonary complications, including pyothorax, pneumonia and atelectasis, acute pulmonary injury, indwelling chest tube, long duration of oxygen supply, and arrhythmia, were evaluated. RESULTS: Cardiopulmonary complications were observed among 61 patients (38%). Univariate analysis revealed that patient age, percentage of forced expiratory volume in 1 s, LAA%, and volume of blood loss were significantly associated with cardiopulmonary complications. Multivariate analysis indicated patient age and LAA% as being significant independent predictors of cardiopulmonary complications. CONCLUSIONS: The regional ratio of the emphysematous area is useful for predicting cardiopulmonary complications in patients with chronic obstructive pulmonary disease who undergo lobectomy for lung cancer. In such patients who are also ≥ 70 years of age and exhibit LAA% ≥ 1.0%, careful intra- and post-operative management is warranted.

[Thoracic Paravertebral Block in Patients Ineligible for Epidural Block].

We retrospectively assessed the effectiveness and the safety of thoracic paravertebral block(PVB) in patients ineligible for epidural block (EP). Eleven PVB patients and 33 EP patients were enrolled. Postoperative pain was evaluated using a numerical rating scale (NRS). The mean NRS ± standard deviation at rest 24 and 48 hours after surgery were 1.36 ± 1.63 and 0.55 ± 1.03 in the PVB group and 1.07 ± 1.47 and 1.38 ± 1.31 in the EP group, respectively. There were no statistically significant differences in the NRS scores. Approximately 10% of the EP patients had complications such as hypotension, nausea and vomiting, or urinary retention. On the other hand, there were no adverse events in the PVB group. PVB can provide pain relief comparable to EP with a better side-effect profile. There were no technical complications associated with PVB. Thoracic PVB is an effective and safe method of postoperative analgesia for patients undergoing thoracic surgery with ineligibilities for EP.

Clinical trial network for the promotion of clinical research for rare diseases in Japan: muscular dystrophy clinical trial network.

BACKGROUND: Duchenne muscular dystrophy (DMD) is the most commonly inherited neuromuscular disease. Therapeutic agents for the treatment of rare disease, namely "orphan drugs", have recently drawn the attention of researchers and pharmaceutical companies. To ensure the successful conduction of clinical trials to evaluate novel treatments for patients with rare diseases, an appropriate infrastructure is needed. One of the effective solutions for the lack of infrastructure is to establish a network of rare diseases. METHODS: To accomplish the conduction of clinical trials in Japan, the Muscular dystrophy clinical trial network (MDCTN) was established by the clinical research group for muscular dystrophy, including the National Center of Neurology and Psychiatry, as well as national and university hospitals, all which have a long-standing history of research cooperation. RESULTS: Thirty-one medical institutions (17 national hospital organizations, 10 university hospitals, 1 national center, 2 public hospitals, and 1 private hospital) belong to this network and collaborate to facilitate clinical trials. The Care and Treatment Site Registry (CTSR) calculates and reports the proportion of patients with neuromuscular diseases in the cooperating sites. In total, there are 5,589 patients with neuromuscular diseases in Japan and the proportion of patients with each disease is as follows: DMD, 29 %; myotonic dystrophy type 1, 23 %; limb girdle muscular dystrophy, 11 %; Becker muscular dystrophy, 10 %. We work jointly to share updated health care information and standardized evaluations of clinical outcomes as well. The collaboration with the patient registry (CTSR), allows the MDCTN to recruit DMD participants with specific mutations and conditions, in a remarkably short period of time. CONCLUSION: Counting with a network that operates at a national level is important to address the corresponding national issues. Thus, our network will be able to contribute with international research activity, which can lead to an improvement of neuromuscular disease treatment in Japan.