Epidemiologic evaluation of pulmonary paragonimiasis in Japan using a Japanese nationwide administrative database.

BACKGROUND: Paragonimiasis is a parasitic disease primarily contracted through consumption of undercooked freshwater crustaceans or wild boar meat. Large-scale nationwide epidemiological data on paragonimiasis are lacking. In this study, we aimed to investigate the nationwide epidemiology of hospitalized patients with paragonimiasis in Japan using a comprehensive nationwide Japanese administrative database. METHODS: We evaluated the Japanese Diagnosis Procedure Combination (DPC) data of patients diagnosed with pulmonary paragonimiasis between April 1, 2012 and March 30, 2020. The patients' address and information, including age, sex, treatment (medication: praziquantel; surgery: open thoracotomy or intracranial mass extirpation), Japan coma scale, comorbidities, and length of hospital stay, were extracted. RESULTS: Of the 49.6 million hospitalized patients, data were extracted on 73 patients with paragonimiasis, of whom 36 were male and 37 were female. The mean age was 49.7 years and the mean length of stay was 12.5 days. The most frequent comorbidity was pleural effusion (31.5 %), followed by pneumothorax (13.7 %). The sites of ectopic paragonimiasis in organs other than the lung included the liver (5.5 %), skin (4.1 %), and brain (2.7 %). Geographically, most patients were from the Kyushu region (54.8 %), followed by the Kanto region (22.0 %). Fukuoka Prefecture had the highest number of patients (22.0 %) by prefecture. During the study period, an average of 9.1 patients/year were hospitalized with lung paragonimiasis in Japan. CONCLUSION: Paragonimiasis has not completely disappeared in Japan; thus, physicians should be aware of paragonimiasis in the Kyushu region, especially in the Fukuoka Prefecture.

Non-contrast magnetic resonance angiography for systemic artery evaluation in Kawasaki disease.

BACKGROUND: Kawasaki disease (KD) is a systemic vasculitis; systemic arteries other than the coronary arteries should therefore also be evaluated. This study investigated the feasibility of evaluating coronary aneurysms, systemic artery aneurysms (SAAs), and cerebrovascular diseases in patients with KD using non-contrast magnetic resonance angiography (NC-MRA). METHODS: Coronary artery protocols, including coronary magnetic resonance angiography (MRA) and vessel wall imaging, were performed in 57 examinations of 28 patients. Systemic artery protocol, including SAA scans and head MRA, along with coronary artery protocol, were performed in 42 examinations of 42 patients. The image quality of the SAAs was evaluated on a 4-point scale. Examination time and sedation dosage were compared between the protocols. The presence of SAAs and cerebrovascular disease was also evaluated. RESULTS: The image quality score of SAAs was 4 (interquartile range [IQR]: 4-4) for the aorta, 4 (IQR: 3-4) for the subclavian artery, 4 (IQR: 3-4) for the renal artery, and 3 (IQR: 3-4) for the iliac artery. No differences were found between examination time (47.0 [IQR: 43.0-61.0] min vs. 51.0 [IQR: 45.0-60.0] min, p = 0.48) and sedative dose (4.63 [IQR: 3.93-5.79] mg/kg vs. 4.21 [IQR: 3.56-5.71] mg/kg, p = 0.37) between the protocols. Systemic artery protocol detected SAAs in three patients (7.1%), and cerebrovascular disease was not detected. CONCLUSIONS: Evaluating the coronary and systemic arteries in patients with KD using NC-MRA on a single examination was possible without compromising examination time or sedation dose. The systemic artery protocol was useful in finding SAAs.

Ovulation patterns affect the offspring sex ratios and change with the women's age.

BACKGROUND: The aim of this study was to evaluate whether women's ages at conception and the ratio of male to female infants are associated with various ovulation patterns. METHODS: An observational clinical study was conducted in private OB/GYN clinics. Infertile women with regular menstrual cycles receiving intrauterine insemination (IUI) and/or in-vitro fertilization (IVF) had their ovulation patterns monitored in three consecutive spontaneous cycles receiving infertility treatment in the third cycle. Ovulation patterns were also observed in women with slight ovulation disorders during IUI and/or IVF in clomiphene citrate stimulated cycles. All the pregnant women's ages at conception and their respective offspring sex ratios were compared to various ovulation patterns. Statistical evaluation was performed using ANOVA, unpaired t test, χ2 test or Fisher's exact test, heterogeneity χ2 test, odds ratios at 95% confidence intervals and logistic regression. RESULTS: Contralateral ovulation (i.e. ovulation jumping from ovary to the other) was more often observed in relatively younger women, who showed a higher probability of having a boy than after ipsilateral ovulation. There was a significantly higher frequency of boys being conceived following three consecutive ovulations with a left-left-right (LLR) ovulation pattern, while three ovulations from the left ovary (LLL) were associated with a higher frequency of girls. We also found two consecutive menstrual cycles the left-right (LR) ovulation pattern showed a similar significant difference compared to the left-left (LL) ovulation. Both the infertile and infertile + fertile women groups showing right-sided ovulation, regardless of age, showed significantly higher offspring sex ratio compared to left-sided ovulation, which was not observed in the group of fertile women alone. CONCLUSIONS: LLR, LR and contralateral ovulation happens more often in younger women and favors male offspring in infertile women. Right-sided ovulation favors male offspring in infertile and infertile + fertile women, which was not observed in the group of fertile women.

It is of interest to evaluate whether ovulation patterns change with women's ages at conception and affect offspring sex ratios. An observational clinical study was conducted in private OB/GYN clinics. Infertile women with regular menstrual cycles receiving intrauterine insemination (IUI) and/or in-vitro fertilization (IVF) had their ovulation patterns monitored in three consecutive spontaneous cycles receiving infertility treatment in the third cycle. Ovulation patterns were also observed in women with slight ovulation disorder during IUI and/or IVF in clomiphene citrate stimulated cycles. Women's ages and offspring sex ratios were compared to various ovulation patterns. The contralateral ovulation was more often observed in relatively younger women, who showed a higher probability of having a boy than following a pregnancy after ipsilateral ovulation. There was a significantly higher frequency of boys being conceived following three consecutive ovulations with after a left-left-right (LLR) ovulation pattern, while three ovulations from the left ovary (LLL) were associated with a higher frequency of girls. Only considering two consecutive menstrual cycles the left-right (LR) ovulation pattern showed a similar significant difference compared to the left-left (LL) ovulation. Both infertile and infertile + fertile women showing right-sided ovulation, regardless of age, showed significantly higher offspring sex ratio compared to left-sided ovulation, which was not observed in the group of fertile women alone. LLR, LR and contralateral ovulation happens more often in younger women and favor male offspring in infertile women. Right-sided ovulation favors male offspring in infertile and infertile + fertile women, which was not observed in the group of fertile women.

Exposure to PM2.5 is a risk factor for acute exacerbation of surgically diagnosed idiopathic pulmonary fibrosis: a case-control study.

BACKGROUND: Short-term exposure to ozone and nitrogen dioxide is a risk factor for acute exacerbation (AE) of idiopathic pulmonary fibrosis (AE-IPF). The comprehensive roles of exposure to fine particulate matter in AE-IPF remain unclear. We aim to investigate the association of short-term exposure to fine particulate matter with the incidence of AE-IPF and to determine the exposure-risk time window during 3 months before the diagnosis of AE-IPF. METHODS: IPF patients were retrospectively identified from the nationwide registry in Japan. We conducted a case-control study to assess the correlation between AE-IPF incidence and short-term exposure to eight air pollutants, including particulate matter < 2.5 µm (PM2.5). In the time-series data, we compared monthly mean exposure concentrations between months with AE (case months) and those without AE (control months). We used multilevel mixed-effects logistic regression models to consider individual and institutional-level variables, and also adjusted these models for several covariates, including temperature and humidity. An additional analysis with different monthly lag periods was conducted to determine the risk-exposure time window for 3 months before the diagnosis of AE-IPF. RESULTS: Overall, 152 patients with surgically diagnosed IPF were analyzed. AE-IPF was significantly associated with an increased mean exposure level of nitric oxide (NO) and PM2.5 30 days prior to AE diagnosis. Adjusted odds ratio (OR) with a 10 unit increase in NO was 1.46 [95% confidence interval (CI) 1.11-1.93], and PM2.5 was 2.56 (95% CI 1.27-5.15). Additional analysis revealed that AE-IPF was associated with exposure to NO during the lag periods lag 1, lag 2, lag 1-2, and lag 1-3, and PM2.5 during the lag periods lag 1 and lag 1-2. CONCLUSIONS: Our results show that PM2.5 is a risk factor for AE-IPF, and the risk-exposure time window related to AE-IPF may lie within 1-2 months before the AE diagnosis. Further investigation is needed on the novel findings regarding the exposure to NO and AE-IPF.

Insufficient development of vessels and alveoli in lungs of infants with trisomy 18-Features of pulmonary histopathological findings from lung biopsy.

The aim of this study was to evaluate the features of pulmonary histopathological changes in cases of trisomy 18 complicated with congenital heart disease and pulmonary arterial hypertension. Twenty-eight patients with trisomy 18 underwent open lung biopsy at the time of primary operation in our hospital between 2008 and 2019. We compared these histopathological findings with those from previously described groups without trisomy 18. Mean age at primary cardiac surgery was 37 days (range, 9-69 days). According to the Heath-Edwards (HE) classification, 1, 8, 12, and 5 patients were graded as 0, 1, 2, and 3, respectively, whereas 2 patients were not classifiable due to medial defects in the small pulmonary arteries (MD). Four (14.3%) and 13 (46.4%) patients presented with MD and hypoplasia of the small pulmonary arteries (HS). Fifteen (53.6%) and 21 (75.0%) patients presented with alveolar hypoplasia (AH) and alveolar wall thickening (AT). MD, HS, and AH in trisomy 18 were present frequently, differing significantly from previous reports. These findings might be associated with congenital inadequate development of vessels and alveoli in the lung, contributing to a high risk of PAH in trisomy 18.

A fatal case of COVID-19-associated invasive pulmonary aspergillosis.

A 79-year-old Japanese man with polymyalgia rheumatica was admitted to hospital with coronavirus disease (COVID-19). On admission, he was treated with ciclesonide inhalation, ivermectin, and meropenem. He was intubated 6 days after admission, and methylprednisolone therapy was initiated (1000 mg/day). Hypoxemia and chest radiographic findings temporarily improved. However, chest computed tomography showed bilateral ground-glass attenuations, multiple nodules, and consolidation. Aspergillus fumigatus was cultured from the tracheal aspirate and he was diagnosed with COVID-19-associated invasive pulmonary aspergillosis (CAPA) and treated with liposomal amphotericin B. However, he died 28 days after admission.

Temporal echocardiographic assessment of pulmonary hypertension in idiopathic pulmonary fibrosis patients treated with nintedanib with or without oxygen therapy.

BACKGROUND: Nintedanib is an inhibitor of receptor tyrosine kinases, including vascular endothelial growth factor receptor, but its effects on pulmonary hypertension (PH) in idiopathic pulmonary fibrosis (IPF) patients with chronic hypoxia were unclear. METHODS: This study included a nintedanib prospective study and historical control study. In the nintedanib prospective study, pulmonary artery systolic pressure (PASP) measured using transthoracic echocardiography was evaluated at six points during 48 weeks in 16 IPF patients in whom nintedanib was started. In the historical control study, adjusted annual change in PASP was compared between patients treated with (n = 16) and without (n = 15) nintedanib. RESULTS: In the nintedanib prospective study, the mean PASP at 48 weeks after starting nintedanib was significantly higher compared to that at baseline. When IPF patients were divided into two groups, IPF patients with or without long-term oxygen treatment (LTOT), mean PASP at 48 weeks was significantly higher than that at baseline only in IPF patients receiving LTOT (P = 0.001). In the historical control study, adjusted annual change in PASP in IPF patients treated with nintedanib was significantly lower than that in patients treated with no antifibrotic agents when considering patients without LTOT (0.26 mmHg vs 7.05 mmHg; P = 0.011). CONCLUSIONS: We found differential effects of nintedanib on PH between IPF patients with or without LTOT. Nintedanib may have a disadvantageous effect on PH in IPF patients with LTOT. Conversely, nintedanib treatment may be beneficial to PH in IPF patients without LTOT.

Hard Metal Lung Disease with Favorable Response to Corticosteroid Treatment: A Case Report and Literature Review.

Hard metal lung disease (HMLD) is a pneumoconiosis caused by occupational exposure to hard metals such as tungsten carbide and cobalt, but the treatment strategies for HMLD have not been well established. A 68-year-old Japanese man with occupational history as a grinder of hard metals for 18 years referred to our hospital because of dry cough and dyspnea. A chest computed tomography (CT) on admission revealed centrilobular micronodules, ground-glass opacities, and reticular opacities in the peripheral zone of both lungs. Mineralogic analyses of lung tissues detected components of hard metals, such as tungsten, titanium and iron, and the same metals were also detected in the sample of the dust of his workplace. Thus, the patient was diagnosed as having HMLD based on occupational exposure history and radiologic and mineralogic analyses of the lung. Corticosteroid therapy was initiated, which resulted in partial improvements in his symptoms, radiological and pulmonary functional findings. In a review of the 18 case reports of HMLD treated with corticosteroids, including our case, the majority of patients (77.8%) showed favorable responses to corticosteroid treatment. Furthermore, the presence of fibrotic changes, such as reticular opacity, in radiological examinations was associated with the resistance to corticosteroids. In conclusion, the majority of patients with HMLD are expected to favorable response to corticosteroid treatment, whereas chest CT findings such as fibrotic changes may be predictive of the resistance of corticosteroid treatment. Lastly, proper prevention of hard metal exposure is most important as the first step.

[A Case of Left Upper Lobe Lung Cancer Successfully Diagnosed by Transesophageal Endoscopic Ultrasound with Bronchoscope-Guided Fine Needle Aspiration Alone].

A 58-year-old Japanese woman with fever and cough visited A hospital. Her chest X-ray and CT showed a tumor attached to the mediastinum in the left upper lobe with mediastinal lymphadenopathy (#4R). After an introduction from A hospital to our hospital, endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) for the mediastinal lymphadenopathy and a simultaneous approach to the mass lesion in the left upper lobe were performed. In spite of twice aspiration by EBUS-TBNA for mediastinal lymphadenopathy, we failed to obtain enough specimens, and, as the mass lesion in the left upper lobe was invisible in the endobronchial ultrasound, we could not approach it. Then using the same ultrasound bronchoscope, we subsequently performed a transesophageal endoscopic ultrasound with bronchoscope-guided fine needle aspiration (EUS-B-FNA) to the mass lesion in the left upper lobe twice, with the result that sufficient tissues were obtained. Then we changed from the ultrasound bronchoscope to a normal bronchoscope and we performed brushing and transbronchial lung biopsy for the left upper lobe mass lesion. Pathological results revealed that only the specimens obtained by EUS-B-FNA were diagnostic for adenocarcinoma; the other specimens obtained using EBUS-TBNA and normal bronchoscope failed to be diagnostic. EUS-B-FNA in combination with EBUS-TBNA has been recommended for the diagnosis of mediastinal and near-mediastinal lesions in the guidelines of the American College of Chest Physicians in 2013, but EUS-B-FNA has not been widely used in Japan. As shown in our present patient who was successfully diagnosed as having lung cancer by EUS-B-FNA alone, respiratory physicians should be aware of being skillful at performing EUS-B-FNA to accurately and effectively approach target lesions.

[A Case of Drug-Induced Lung Injury Caused by Kamikihito].

An eighty-nine-year-old Japanese male was admitted to our hospital due to dry cough and dyspnea. Respiratory symptoms appeared soon after an administration of an oriental medicine, Kamikihito for tinnitus. Upon admission, chest computed tomography showed patchy consolidations and ground-glass opacities in the right upper lobe of the lungs, and ground-glass opacities in the bilateral lower lobes. Sulbactam-ampicillin combination (SBT/ABPC, 3 g × 2/day) was started in addition to the change or cessation of several other drugs, including Kamikihito, resulting in respiratory symptoms and chest radiographic exacerbations. Bronchoalveolar lavage fluid obtained from the right S3 showed an increase in the total cell number of lymphocytes. A drug lymphocyte stimulation test (DLST) for Kamikihito was also positive. Kamikihito-induced lung injury was most likely, and treatment with prednisolone (50 mg/day) was started. His respiratory symptoms and chest radiographic findings improved rapidly soon after initiating oral prednisolone. This is the first report of Kamikihito-induced lung injury.

[Evaluation of the Influence of the Experience and Training of EBUS-TBNA on Diagnostic Rate and Safety].

Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) has been widely used in Japan. The guidelines of the American College of Chest Physicians has recommended that EBUS-TBNA should be performed by well-trained operators who can perform highly accurate procedures, but the indicators of the degree of experience and training are unclear. In our department, physicians who do not have enough experience perform EBUS-TBNA under the supervision of bronchoscopic instructors who have EBUS-TBNA techniques (Board Certified Member of the Japan Society for Respiratory Endoscopy) after guidance and training in EBUS-TBNA using a simulator as an operator and helper. In order to evaluate the influence of the experience and training of EBUS-TBNA on diagnostic accuracy and safety, we retrospectively compared the diagnostic accuracy and safety of EBUS-TBNA performed by physicians within one year of experience of EBUS-TBNA and those performed by physicians with more than one year of experience. A total of 111 cases (148 lesions) who were eventually diagnosed as having primary lung cancer and underwent EBUS-TBNA in our department between April 2014 and January 2016 were divided into two groups. Group A (43 cases, 57 lesions) was examined by third-year doctors within one year of experience of EBUS-TBNA, and group B (68 cases, 91 lesions) was examined by doctors with four or more years of experience and with more than one year of experience of EBUS-TBNA. Diagnostic rate, examination time, and complications were evaluated. There were no significant differences between the two groups in the diagnostic rate (A, 89.5% vs. B, 90.1%, P = 1.0) or examination time (A, 27 min vs. B, 23 min, P = 0.149), and no complications were observed in either group. This study suggests that even less-experienced physicians may safely perform EBUS-TBNA as well as moderately-experienced physicians with more than 1 year experience of EBUS-TBNA with similar diagnostic rates when proper training and supervision are supplied.

Radiation Dose Reduction With a Low-Tube Voltage Technique for Pediatric Chest Computed Tomographic Angiography Based on the Contrast-to-Noise Ratio Index.

INTRODUCTION: The aim of this study was to evaluate the radiation dose and image quality at low tube-voltage pediatric chest computed tomographic angiography (CTA) that applies the same contrast-to-noise ratio (CNR) index as the standard tube voltage technique. MATERIALS AND METHODS: Contrast-enhanced chest CTA scans of 100 infants were acquired on a 64-row multidetector computed tomography (MDCT) scanner. In the retrospective study, we evaluated 50 images acquired at 120 kVp; the image noise level was set at 25 Hounsfield units. In the prospective study, we used an 80-kVp protocol; the image noise level was 40 Hounsfield units because the iodine contrast was 1.6 times higher than on 120-kVp scans; the CNR was as in the 120-kVp protocol. We compared the CT number, image noise, CT dose index volume (CTDIvol), and the dose-length product on scans acquired with the 2 protocols. A diagnostic radiologist and a pediatric cardiologist visually evaluated all CTA images. RESULTS: The mean CTDIvol and the mean dose-length product were 0.5 mGy and 7.8 mGy-cm for 80- and 1.2 mGy and 20.8 mGy-cm for 120-kVp scans, respectively (P < .001). The mean CTDIvol was 42% lower at 80 kVp than at 120 kVp, and there was no significant difference in the visual scores assigned to the CTA images (P = .28). CONCLUSIONS: With the CNR index being the same at 80-kVp and 120-kVp imaging, the radiation dose delivered to infants subjected to chest CTA can be reduced without degradation of the image quality.

Clinical Features of Acute and Fulminant Myocarditis in Children　- 2nd Nationwide Survey by Japanese Society of Pediatric Cardiology and Cardiac Surgery.

BACKGROUND: The 1st nationwide survey by the Japanese Society of Pediatric Cardiology and Cardiac Surgery of acute or fulminant myocarditis (AMC/FMC) in children revealed that the survival rate of FMC was only 51.6%. The 2nd nationwide survey was performed to evaluate the recent outcomes of pediatric myocarditis.Methods and Results:Questionnaires regarding patients aged ≤18 years with AMC/FMC during the period from January 2006 to December 2011 were mailed. A total of 221 cases (age 6.5±5.3 years, 116 boys and 105 girls) were reported. There were 145 (65.6%) and 74 cases (33.5%) of AMC/FMC, respectively; the type of myocarditis was not reported in the remaining 2 cases (0.9%). Viruses were identified in 56 cases (25.3%), including coxsackie B in 9 and influenza A in 8. Histopathology by either endomyocardial biopsy or autopsy was obtained in 38 cases (19.2%). Intravenous immunoglobulin was effective in 49 (34.3%) of 143 cases. Steroid therapy was effective in 20 (32.8%) of 61 cases. Mechanical circulatory support was given in 54 cases (24.4%) and 94.2% of them were patients with FMC. The survival rates for the whole study population, acute myocarditis, and FMC were 75.6%, 91.0%, and 48.6%, respectively. CONCLUSIONS: The survival rate of children with myocarditis was almost identical to that of 10 years ago. (Circ J 2016; 80: 2362-2368).

Medial defects of the small pulmonary arteries in fatal pulmonary hypertension in infants with trisomy 13 and trisomy 18.

Congestive heart failure is a major cause of early death in patients with trisomy 13 or 18 and congenital heart disease (CHD). Pulmonary artery banding for these patients early in life is preferred to protect the lungs from high pulmonary flow rates and improve survival. We performed open lung biopsies in 11 patients with trisomy 13 or 18 accompanied by CHD and severe pulmonary artery hypertension (PAH) between 2009 and 2011. Two (18.2%) of these 11 patients had medial defects of the small pulmonary arteries. One patient with trisomy 13 and an atrial septal defect developed lung hemorrhage and lung edema at the age of 9 months and died at the age of 13 months. The lumens of the small pulmonary arteries of the other patient with trisomy 18 and a ventricular septal defect became occluded due to the intimal proliferation of fibrous tissues at the age of 2 months. This patient died at the age of 27 months. The deaths of both patients were associated with heart-related factors. Patients with medial defects are vulnerable to intimal proliferation in the small pulmonary arteries. More patients with trisomy 13 or 18 and CHD might have similar pulmonary vascular changes. The small pulmonary arteries of patients with trisomy 13 and 18 should be further analyzed.

Cardiac computed tomography angiography with and without bolus tracking methods in infants with congenital heart disease.

The study investigated radiation dose, vascular computed tomography (CT) enhancement and image quality of cardiac computed tomography angiography (CCTA) with and without bolus tracking (BT) methods in infants with congenital heart disease (CHD). The volume CT dose index (CTDIvol) and dose length product (DLP) were recorded for all CT scans, and the effective dose was obtained using a conversion factors. The CT number for the ascending aorta (AO) and pulmonary artery (PA), image noise of muscle tissue and contrast-to-noise ratio (CNR) were measured and calculated. The median values in the groups with and without BT were 2.20 mGy versus 0.44 mGy for CTDIvol, 8.10 mGy·cm versus 6.20 mGy·cm for DLP, and 0.66 mSv versus 0.51 mSv for effective dose (p < 0.001). There were no statistical differences in vascular CT enhancement, image noise, and CNR. CCTA without BT methods can reduce the radiation dose while maintaining vascular CT enhancement and image quality compared to CCTA with BT methods.

Corrigendum: Use of the index of pulmonary vascular disease for predicting longterm outcome of pulmonary arterial hypertension associated with congenital heart disease.

[This corrects the article DOI: 10.3389/fcvm.2023.1212882.].

Overexpression of T-bet gene regulates murine autoimmune arthritis.

OBJECTIVE: To clarify the role of T-bet in the pathogenesis of collagen-induced arthritis (CIA). METHODS: T-bet-transgenic (Tg) mice under the control of the CD2 promoter were generated. CIA was induced in T-bet-Tg mice and wild-type C57BL/6 (B6) mice. Levels of type II collagen (CII)-reactive T-bet and retinoic acid receptor-related orphan nuclear receptor γt (RORγt) messenger RNA expression were analyzed by real-time polymerase chain reaction. Criss-cross experiments using CD4+ T cells from B6 and T-bet-Tg mice, as well as CD11c+ splenic dendritic cells (DCs) from B6 and T-bet-Tg mice with CII were performed, and interleukin-17 (IL-17) and interferon-γ (IFNγ) in the supernatants were measured by enzyme-linked immunosorbent assay. CD4+ T cells from B6, T-bet-Tg, or T-bet-Tg/IFNγ-/- mice were cultured for Th17 cell differentiation, then the proportions of cells producing IFNγ and IL-17 were analyzed by fluorescence-activated cell sorting. RESULTS: Unlike the B6 mice, the T-bet-Tg mice did not develop CIA. T-bet-Tg mice showed overexpression of Tbx21 and down-regulation of Rorc in CII-reactive T cells. Criss-cross experiments with CD4+ T cells and splenic DCs showed a significant reduction in IL-17 production by CII-reactive CD4+ T cells in T-bet-Tg mice, even upon coculture with DCs from B6 mice, indicating dysfunction of IL-17-producing CD4+ T cells. Inhibition of Th17 cell differentiation under an in vitro condition favoring Th17 cell differentiation was observed in both T-bet-Tg mice and T-bet-Tg/IFNγ-/- mice. CONCLUSION: Overexpression of T-bet in T cells suppressed the development of autoimmune arthritis. The regulatory mechanism of arthritis might involve dysfunction of CII-reactive Th17 cell differentiation by overexpression of T-bet via IFNγ-independent pathways.

Use of the index of pulmonary vascular disease for predicting long-term outcome of pulmonary arterial hypertension associated with congenital heart disease.

Aims: Limited data exist on risk factors for the long-term outcome of pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD-PAH). We focused on the index of pulmonary vascular disease (IPVD), an assessment system for pulmonary artery pathology specimens. The IPVD classifies pulmonary vascular lesions into four categories based on severity: (1) no intimal thickening, (2) cellular thickening of the intima, (3) fibrous thickening of the intima, and (4) destruction of the tunica media, with the overall grade expressed as an additive mean of these scores. This study aimed to investigate the relationship between IPVD and the long-term outcome of CHD-PAH. Methods: This retrospective study examined lung pathology images of 764 patients with CHD-PAH aged <20 years whose lung specimens were submitted to the Japanese Research Institute of Pulmonary Vasculature for pulmonary pathological review between 2001 and 2020. Clinical information was collected retrospectively by each attending physician. The primary endpoint was cardiovascular death. Results: The 5-year, 10-year, 15-year, and 20-year cardiovascular death-free survival rates for all patients were 92.0%, 90.4%, 87.3%, and 86.1%, respectively. The group with an IPVD of ≥2.0 had significantly poorer survival than the group with an IPVD <2.0 (P = .037). The Cox proportional hazards model adjusted for the presence of congenital anomaly syndromes associated with pulmonary hypertension, and age at lung biopsy showed similar results (hazard ratio 4.46; 95% confidence interval: 1.45-13.73; P = .009). Conclusions: The IPVD scoring system is useful for predicting the long-term outcome of CHD-PAH. For patients with an IPVD of ≥2.0, treatment strategies, including choosing palliative procedures such as pulmonary artery banding to restrict pulmonary blood flow and postponement of intracardiac repair, should be more carefully considered.

A new scoring system for predicting in-hospital death after lung cancer surgery (the SABCIP score) using a Japanese nationwide administrative database.

BACKGROUND: We aimed to develop and validate a new risk scoring tool for predicting in-hospital mortality after lung cancer surgery. METHODS: We retrospectively identified patients admitted for lung cancer surgery from a nationwide administrative database in Japan and randomly divided them into derivation and validation cohorts. In the derivation cohort, we performed logistic regression analysis to determine predictive variables and developed a risk scoring tool by proportionally weighting the regression coefficients and assigning points to each variable. In both cohorts, we evaluated the predictive performance of the score using the c-index and showed the in-hospital mortality at each risk score. RESULTS: In total, 64 175 patients (32 170 and 32 005 patients in the derivation and validation cohort, respectively) were enrolled, including 115 (0.4%) and 119 (0.4%) in-hospital patient deaths in the derivation and validation cohorts, respectively. Following the multivariate regression analysis, we selected six variables to create the SABCIP score, a risk scoring tool named after the parameters on which it is based, namely male sex, age ≥ 75 years, body mass index <18.5, clinical stage ≥3, interstitial lung disease, and procedure type (sleeve resection, chest wall resection, or pneumonectomy). The c-index of the score was 0.82 and 0.80 in the derivation and validation cohorts, respectively, which represents a better or equal discrimination performance compared with previous scoring tools. In-hospital mortality increased as the score increased in both cohorts. CONCLUSION: The SABCIP score is a simple and useful predictor of in-hospital mortality in patients after lung cancer surgery.

Pitfall for systemic artery aneurysms evaluation using electrocardiogram-gated subtracted three-dimensional fast spin echo sequence of magnetic resonance imaging in patients with Kawasaki disease.

Kawasaki disease (KD) is described as a syndrome that causes both coronary and systemic artery aneurysms (SAAs). This report describes the pitfall for SAAs' evaluation when using electrocardiogram (ECG)-gated subtracted three-dimensional fast spin echo (3D FSE) sequence of magnetic resonance imaging in KD patients. A 12-year-old male was diagnosed with KD at 3 months of age. We acquired ECG-gated 3D FSE images in the diastole and systole phases with coronal sections. Subtraction was then performed from diastolic phase imaging to systolic phase imaging. A 15.5 mm right axillary artery aneurysm and an 8.0 mm left axillary artery aneurysm were identified with ECG-gated 3D FSE in the diastolic phase. However, we observed signal loss in the right axillary artery aneurysm when subtraction was performed to selectively detect arteries; further, the brachial artery was poorly detected. ECG-gated subtracted 3D FSE sequence of magnetic resonance imaging can compromise the image quality of both aneurysm and peripheral artery images when detecting SAAs.