Pediatric anti-neutral glycosphingolipid antibodies-positive encephalomyeloradiculoneuropathy presenting with prominent brain demyelination.

BACKGROUND: Encephalomyeloradiculoneuropathy (EMRN) is characterized by progressive neurological symptoms in the central and peripheral nervous systems. The autoantibodies against neutral sphingolipids are disease-specific antibodies against EMRN. Although adults with EMRN typically present with symptoms of peripheral nervous system involvement, the symptoms in pediatric patients are not well understood. CASE: A 4-year-old boy was admitted to our hospital on the 10th day of fever due to poor oral intake and hyponatremia. The day after admission, he developed seizures and impaired consciousness and was transferred to our hospital. When he arrived at our hospital, he experienced disturbances in consciousness, neck rigidity, and opisthotonus. MRI of the head revealed scattered white matter lesions and pleocytosis in the cerebrospinal fluid (CSF). During treatment with intravenous methylprednisolone (IVMP), the patient developed diminished deep tendon reflexes in the lower extremities four days later, with no improvement in cervical stiffness or opisthotonos. Additional evaluations revealed enlarged cerebral white matter lesions on brain MRI, cauda equina enhancement on MRI of the spinal cord, axonal neuropathy in the bilateral tibial nerves, and positive anti-neutral glycosphingolipid (GSL) antibodies in both serum and CSF. Intensive immunomodulatory therapy, and neurorehabilitation, led to substantial neurological recovery within three months of onset. CONCLUSION: Pediatric antineutral GSL antibody-positive EMRN may initially present with extensive cerebral white matter lesions and delayed onset of peripheral radiculoneuropathy. Our case extends the disease spectrum of EMRN and may aid in the early diagnosis of EMRN in the pediatric population.

Static supply of different simulated flue gases for native microalgae cultivation in diluted cow manure digestate.

The application of microalgae to sequester CO2 from flue gases can be an interesting process since it can contribute to mitigate CO2 emission into the atmosphere. One obstacle of such application is the high CO2 concentration in the flue gases, which can lead to low pH in the cultivation medium and hence process failure. This study aims to investigate static CO2 gas supply for microalgae cultivation as a potential alternative that might allow applying different flue gases with different compositions and higher CO2 concentrations. Two sets of experiments were performed. First, the effect of increasing the amount of supplied carbon was tested. In the second experiment, the applicability of such system for different flue gases regarding their oxygen and carbon content was tested. In all experiments, 50 times diluted cow manure digestate was used as a culture medium. By increasing CO2 concentration up to 10% in the supplied air, microalgae growth productivity of 48.7 mg/L/d was achieved. A further improvement of microalgae growth was shown with increasing the gas/culture volume ratio. Microalgae productivity rate increased form 48.7 mg/L/d to 73.5 mg/L/d when the volume of gas increased from 47% to 81% of total volume. Applying CO2 in air (O2 content around 20%) or in N2 (O2 content less than 2%) didn't show any difference regarding inorganic carbon dissolution, pH, ammonium nitrogen removal, CO2 fixation or biomass productivity. Generally, it can be concluded that static gas supply for microalgae cultivation can allow the application of different flue gases from different industries with low or high O2 content and with CO2 concentration as high as 20%. According to our results, a microalgae cultivation system with continuous static gas supply was proposed.

Fatal X-linked lymphoproliferative disease type 1-associated limbic encephalitis with positive anti-alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor antibody.

BACKGROUND: X-linked lymphoproliferative disease type 1 (XLP1) is a rare monogenic immune dysregulation disorder caused by a deficiency of a signaling lymphocyte activation molecule-associated protein (SAP). While many patients with XLP1 present with fatal hemophagocytic lymphohistiocytosis upon Epstein Barr virus (EBV) infection, a small fraction present with limbic encephalitis in the absence of EBV infection. It is poorly understood why SAP deficiency may cause limbic encephalitis in XLP1. CASE: A 12-year-old boy presented with seizures, changes in personality, memory loss, and cognitive deficits during treatment for interstitial pneumonia. A diagnosis of limbic encephalitis was made. Despite treatment against CD8+ T cell-mediated autoimmunity with intravenous methylprednisolone, dexamethasone, intravenous immunoglobulin, plasma exchange, cyclosporine, weekly etoposide, mycophenolate mofetil, and adalimumab, encephalitis progressed until the patient died after one month of treatment intitiation. Post-mortem genetic testing revealed a de novo SH2D1A truncating mutation. Tests for EBV infection were negative. Initial spinal fluid revealed markedly elevated protein levels, mild pleocytosis, and elevation of two chemokines (C-X-C motif chemokine ligand [CXCL] 10 and CXCL 13). Moreover, initial spinal fluid was tested positive for anti-alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR) autoantibody. DISCUSSION: In XLP1-associated limbic encephalitis, anti-AMPAR autoantibody production by the dysregulated immune system due to SAP deficiency might be a pathogenic mechanism of central nervous system manifestations. In addition to the standard treatment for XLP1, targeted treatment against B-cell-mediated immunity might be indicated for patients with XLP1-associated limbic encephalitis.

An oldest-old non-small cell lung cancer patient with abscopal effect in a single lesion.

The abscopal effect without concomitant immunotherapy is a rare event, including among cases of lung cancer. Furthermore, the occurrence of limited abscopal effect for only a single lesion in the metastatic organ consistent with the irradiated organ would be an even more rare event. A 94-year-old man was diagnosed with advanced lung cancer with osteolytic bone metastases in his right iliac bone, and the right side of his axial vertebrae. After palliative radiation therapy to the right iliac lesion for pain relief without other anticancer therapy, the axial vertebral osteolytic lesion disappeared despite no reduction in the other lesions. This case furthers our understanding of the pathogenesis of the abscopal effect.

Prediction of leachate quantity and quality from a landfill site by the long short-term memory model.

The long short-term memory (LSTM) model was first applied in this study for the prediction of the leachate quantity and quality at a real landfill site. In our LSTM model, in the learning phase from July 2003 to March 2018, three input data items consisting of the daily precipitation (DP), the daily average temperature (DAT), and the accumulated amount of landfilled waste presented the quantity of leachate generated with high accuracy. The DAT was important for the landfill site, particularly in a snow area because it contributes to the leachate generated during the spring thaw with low precipitation. In the testing phase from April 2018 to March 2019, our LSTM model predicted the leachate generated with a mean absolute percentage error (MAPE) of 26.2%. The concentrations of biological oxygen demand, chemical oxygen demand, total nitrogen, calcium ion and chloride ion in leachate were presented in the learning phase by six input data items: DP, DAT, and the daily amount of landfilled waste (incineration residue, incombustible waste, business waste, and combustible waste) with high R2 values. In the testing phase, the quality of leachate was predicted with the MAPE between 11.8% and 30.2%. Another year data from April 2019 to March 2020 was used to verify accuracy of our model with no overfitting. This study showed the possibility of applying the LSTM model to future predictions of leachate quantity and quality from landfill sites with an acceptable error for daily operation.

Strategy of landfilled waste reduction by a distributed materials recovery facility system in Surabaya, Indonesia.

Slow progress in municipal waste reduction and landfill space scarcity lead to numerous environmental problems in Indonesia and developing countries. Surabaya, the role model of an environmental management city in Indonesia and other countries, is no exception. Despite the situation, Surabaya's initiative of deploying a distributed materials recovery facility (MRF) and its performance in recovering recyclables show a potential to be developed for addressing the landfill waste reduction issues. This study proposes a new strategy with small-sized distributed MRFs to achieve 30% landfilled waste reduction and reducing greenhouse gas (GHG) emissions, focusing on Surabaya as the case study. Scenario 1 merged three pairs of transfer stations which shows some indistinguishable optimizations and failed to meet the target. Both Scenario 2 and Scenario 3 added six years of landfill lifetime for reaching the target. However, the distributed MRF system and different transportation systems in Scenario 3 accomplished the goal with only 24 new MRFs, whereas Scenario 2 needs to upgrade 48 transfer stations into MRFs. Scenario 3 decreased the GHG emissions generation by 29%, possibly contributing to Indonesia's GHG emissions target of 0.2%.

Endobronchial brachytherapy with curative intent: the impact of reference points setting according to the bronchial diameter.

Endobronchial brachytherapy (EBB) is an effective treatment for endobronchial tumors. However, bronchial toxicity caused by over-irradiation remains problematic. To decrease bronchial toxicity, we developed a source-centralizing applicator for EBB. The purpose of the present study was to assess the efficacy and safety of EBB with varying reference dose points according to the bronchial diameter, using a source-centralizing applicator. We reviewed 15 patients with endobronchial carcinoma who were treated with curative intent using a combination of external beam radiotherapy (EBRT) and high-dose-rate EBB between 2005 and 2014. During each EBB session, we used a source-centralizing applicator that maintained the source-delivering catheter in the center of the bronchial lumen. Reference dose points were 5-7 mm from the source axis, depending on the bronchial diameter. The median radiation doses of EBRT and EBB were 40 Gy in 20 fractions and 18 Gy in 3 fractions, respectively. The median observation period was 36 months. The 3-year overall survival, progression-free survival and local control rates were 79%, 77% and 100%, respectively. Grade 2 radiation pneumonitis was observed in two cases. Bronchial toxicities, such as hemoptysis or the symptoms of chronic bronchitis, were not observed. EBB with varying reference dose points according to bronchial diameter, using a source-centralizing applicator, is a promising procedure that may be effective for tumor elimination and reducing toxicity to the bronchial wall.

The impact of epidermal growth factor receptor mutations on patterns of disease recurrence after chemoradiotherapy for locally advanced non-small cell lung cancer: a literature review and pooled analysis.

The purpose of this review was to evaluate the impact of epidermal growth factor receptor (EGFR) mutation status on disease recurrence in patients treated with chemoradiotherapy (CRT) for locally advanced non-small cell lung cancer (NSCLC). A literature search was conducted and a total of three studies were analyzed. There was no significant difference in the objective response rate between the EGFR mutation group and the EGFR wild-type group (odds ratios [OR] 1.46, 95% CI, 0.79-2.70, P = 0.228), and there was no significant difference in the incidence of disease recurrence (OR 1.37, 95% CI, 0.68-2.75, P = 0.379) between the two groups. There were significant difference in the incidence of local/locoregional progression (LP) (OR 0.35, 95% CI, 0.18-0.71, P = 0.003) and distant progression (DP) (OR 2.97, 95% CI, 1.59-5.54, P < 0.001). Brain metastasis (BM) was one of the main recurrence patterns of DP, and the incidence was significantly higher in the EGFR mutant group (OR 2.75, 95% CI, 1.43-5.31, P = 0.003). There were no statistically significant heterogeneities in these pooled analyses. The patterns of recurrence after CRT for locally advanced NSCLC were different according to EGFR mutation status. LP after CRT in patients with EGFR mutation was less frequent, but the high incidence of DP, especially BM, continued to be the major problem. On the other hand, LP continued to be the major problem in EGFR wild-type patients. In multimodality treatment for inoperable locally advanced NSCLC, we may need to consider different treatment strategies according to EGFR mutation status.

The impact of emphysema on dosimetric parameters for stereotactic body radiotherapy of the lung.

The purpose of this study was to evaluate the impact of emphysematous changes in lung on dosimetric parameters in stereotactic body radiation therapy (SBRT) for lung tumor. A total of 72 treatment plans were reviewed, and dosimetric factors [including homogeneity index (HI) and conformity index (CI)] were evaluated. Emphysematous changes in lung were observed in 43 patients (60%). Patients were divided into three groups according to the severity of emphysema: no emphysema (n = 29), mild emphysema (n = 22) and moderate to severe emphysema groups (n = 21). The HI (P < 0.001) and the CI (P = 0.029) were significantly different in accordance with the severity of emphysema in one-way analysis of variance (ANOVA). The HI value was significantly higher in the moderate to severe emphysema group compared with in the no emphysema (Tukey, P < 0.001) and mild emphysema groups (P = 0.002). The CI value was significantly higher in the moderate to severe emphysema group compared with in the no emphysema group (P = 0.044). In multiple linear regression analysis, the severity of emphysema (P < 0.001) and the mean material density of the lung within the PTV (P < 0.001) were significant factors for HI, and the mean density of the lung within the PTV (P = 0.005) was the only significant factor for CI. The mean density of the lung within the PTV was significantly different in accordance with the severity of emphysema (one-way ANOVA, P = 0.008) and the severity of emphysema (P < 0.001) was one of the significant factors for the density of the lung within the PTV in multiple linear regression analysis. Our results suggest that emphysematous changes in the lung significantly impact on several dosimetric parameters in SBRT, and they should be carefully evaluated before treatment planning.

Multimodality Treatment for Patients with Node-Positive Prostate Cancer: the Role of Radiation Therapy.

Prostate cancer is the secondary most frequently diagnosed cancer in the world. Although numerous prospective randomized trial have been conducted to guide the management of patients with localized or locally advanced prostate cancer, few clinical trials targeting node-positive prostate cancer have been reported. Therefore, there are still controversies in the optimal management of node-positive prostate cancer. Recently, efficacy of multimodality treatment, including radiation therapy (RT), for such patients has been reported in several articles. The results indicate potential benefit of RT both in adjuvant therapy after prostatectomy and in definitive therapy for node-positive prostate cancer. The aim in this article was to summarize the current evidence for RT and evaluate the role in multimodality treatment for patients with node-positive prostate cancer.

Roles of Valproic Acid in Improving Radiation Therapy for Glioblastoma: a Review of Literature Focusing on Clinical Evidence.

Glioblastoma (GBM) is the most common and aggressive type of primary brain neoplasm. The current standard therapy for GBM consists of maximal surgical resection within safe limits, followed by radiation therapy (RT) and chemotherapy with temozolomide. Despite advances in treatment, the prognosis of GBM remains poor. Epileptic seizure is one of the most common symptoms in patients with GBM. Valproic acid (VPA), a histone deacetylase inhibitor, is often used as an anti-epileptic drug in patients with brain neoplasms due to its effectiveness and low toxicity profile. Several in vivo and in vitro studies have indicated that VPA has radiosensitizing effects for gliomas and radioprotective influence on normal brain tissue or hippocampal neurons. The results of several retrospective studies have also indicated potential benefit to improve survival of patients with GBM. Moreover, the promising treatment results of a phase 2 trial of concurrent radiation therapy, temozolomide, and VPA for patients with GBM have been recently reported. The use of VPA in patients with GBM has thus recently receiving more attention. In this article, we review the role of VPA in radiation therapy for GBM, focusing on the clinical evidence.

Radiation-induced organizing pneumonia after stereotactic body radiotherapy for lung tumor.

The aim of this retrospective study was to investigate characteristics of organizing pneumonia (OP) after stereotactic body radiotherapy (SBRT) for lung tumor. Between September 2010 and June 2014, patients who were diagnosed as Stage I lung cancer and treated with SBRT at our institution were included in this study. A total of 78 patients (47 males with a median age of 80 years) were analyzed. The median follow-up period was 23 months. Five patients (6.4%) developed OP at 6-18 months after SBRT. The cumulative incidence of OP was 4.3% (95% confidence interval [CI], 1.1-11.0) and 8.2% (95% CI, 2.9-17.0) at 1 and 2 years, respectively. Tumor location (superior and middle lobe vs inferior lobe) was shown to be a borderline significant factor for the occurrence of OP ( P: = 0.069). In the subgroup analysis of patients with a radiographic follow-up period at least 6 months, or who died within 6 months after SBRT, 7 of 72 patients (9.7%) developed Grade 2 or 3 radiation pneumonitis (G2/3 RP) at 2-4 months after SBRT. A statistically significant association between G2/3 RP in the subacute phase and OP was shown ( P: = 0.040). In two of the five patients who developed OP, the symptoms and radiographic change were improved rapidly by corticosteroid administration. One patient had relapsed OP after suspending the treatment and re-administration was required. Three patients with minor symptoms were managed without corticosteroid administration and OP resolved without any relapse. The radiation-induced OP should be considered as one of the late lung injuries after SBRT for lung tumors.

Comparison of therapeutic results from radiofrequency ablation and stereotactic body radiotherapy in solitary lung tumors measuring 5 cm or smaller.

BACKGROUNDS: This retrospective study was conducted to compare the clinical outcomes of radiofrequency ablation (RFA) with those of stereotactic body radiotherapy (SBRT) in patients with lung tumors. METHODS: Local tumor progression, adverse events, and overall survival were compared in patients who underwent either RFA or SBRT for a single lung tumor measuring 5 cm or smaller. This study was approved by the institutional review boards of two institutions. Informed consent was waived. RESULTS: During September 2009 to June 2012, 48 patients [30 males and 18 females, with a mean age ± standard deviation (SD) of 75.0 ± 7.5 years] underwent RFA at one institution and 47 patients (21 males and 26 females, with a mean age ± SD of 77.0 ± 7.5 years) underwent SBRT in another. The mean maximum tumor diameter ± SD was 2.0 ± 0.8 cm (range 0.6-3.9 cm) in the RFA group, and 2.1 ± 0.9 cm (range 0.8-4.7 cm, p = 0.539) in the SBRT group. The RFA and SBRT groups showed similar 3-year local tumor progression [9.6%, 95% confidence interval (CI) 3.6-23.9% vs. 7.0%, 95% CI 0.2-20.2%, p = 0.746] and overall survival rates (86.4%, 95% CI 69.2-94.3% vs. 79.6%, 95% CI 60.6-90.1%, p = 0.738). No factor significantly affected local tumor progression. A maximum tumor size of 2 cm was identified as a prognostic factor in both univariate and multivariate analyses. No death was related to treatment procedures. Major complication rates (Grade 3 adverse events) of the RFA (10.4%, 5/48) and SBRT (8.5%, 4/47, p > 0.999) groups were similar. CONCLUSION: For lung tumor patients, lung RFA provided local tumor control and survival that were similar to those achieved using SBRT, with equal safety.

Scenario analysis of the benefit of municipal organic-waste composting over landfill, Cambodia.

This paper presents insight into the benefits of organic waste recycling through composting over landfill, in terms of landfill life extension, compost product, and mitigation of greenhouse gases (GHGs). Future waste generation from 2003 to 2020 was forecast, and five scenarios of organic waste recycling in the municipality of Phnom Penh (MPP), Cambodia, were carried out. Organic waste-specifically food and garden waste-was used for composting, and the remaining waste was landfilled. The recycling scenarios were set based on organic waste generated from difference sources: households, restaurants, shops, markets, schools, hotels, offices, and street sweeping. Through the five scenarios, the minimum volume reductions of waste disposal were about 56, 123, and 219 m(3) d(-1) in 2003, 2012, and 2020, respectively, whereas the maximum volume reductions in these years were about 325, 643, and 1025 m(3) d(-1). These volume reductions reflect a landfill life extension of a minimum of half a year and a maximum of about four years. Compost product could be produced at a minimum of 14, 30, and 54 tons d(-1) in 2003, 2012, and 2020, respectively, and at a maximum in those years of about 80, 158, and 252 tons d(-1). At the same time benefit is gained in compost product, GHG emissions could be reduced by a minimum of 12.8% and a maximum of 65.0% from 2003 to 2020. This means about 3.23 (minimum) and 5.79 million tons CO(2)eq (maximum) contributed to GHG mitigation. In this regard, it is strongly recommended that MPP should try to initiate an organic-waste recycling strategy in a best fit scenario.

Three-Dimensional Comparison in Palatal Forms Between Modified Presurgical Nasoalveolar Molding Plate and Hotz's Plate Applied to the Infants With Unilateral Cleft Lip and Palate.

The presurgical nasoalveolar molding plate appliance with stent (PNAM) extended from the palatal molding plate; to correct the nostril shape of infants with cleft lip and palate is well known. The PNAM appliance is based on the finding that a high degree of plasticity is maintained in the cartilage of infants during the first 6 weeks after birth. However, on the current PNAM protocol described by Grayson et al. the nasal stent is supposed to be an adjunct to the palatal molding plate after reducing the severity of the alveolar cleft width. We have used the modified Hotz's plate from the setup model and built up the nasal stent even before reducing the severity of the alveolar deformity. In this study we assess the effects of the modified Hotz's plate and the modified PNAM appliance for the alveolar and palatal form. The lateral deviation of the incisal point, the width of the palatal cleft, and the degree of curvature of the palatal vault were first evaluated on plaster models. The PNAM group is smaller on the lateral deviation of the incisal point than the modified Hotz's group. The decreased average width of the palatal cleft and curvature of the palate, was almost the same in both the modified Hotz's and PNAM groups. In comparison with the modified Hotz's plate, the modified PNAM appliance also improves the molding of the alveolar segments and reduces cleft width.