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Objectives: To stratify patients with mixed connective tissue disease (MCTD) based on their immunophenotype., Methods: We analyzed the immunophenotype and transcriptome of 24 immune cell subsets from patients with MCTD, systemic lupus erythematosus (SLE), idiopathic inflammatory myopathy (IIM), and systemic sclerosis (SSc) from our functional genome database, ImmuNexUT (https://www.immunexut.org/). MCTD patients were stratified by employing machine learning models including Random Forest, trained by immunophenotyping data from SLE, IIM, and SSc patients. Transcriptomes were analyzed with gene set variation analysis (GSVA) and clinical features of MCTD subgroups were compared., Results: This study included 215 patients, including 22 patients with MCTD. Machine learning models, constructed to classify SLE, IIM, and SSc patients based on immunophenotyping, were applied to MCTD patients, resulting in 16 classified as SLE-immunophenotype and 6 as non-SLE-immunophenotype. Among MCTD patients, patients with the SLE-immunophenotype had higher proportions of Th1 cells [2.85% (interquartile range (IQR) 1.54-3.91) vs 1.33% (IQR 0.99-1.74) p= 0.027] and plasmablasts [6.35% (IQR 4.17-17.49) vs 2.00% (IQR 1.20-2.80) p= 0.010]. Notably, the number of SLE-related symptoms was higher in patients with the SLE-immunophenotype [2.0 (IQR 1.0-2.0) vs 1.0 (IQR1.0-1.0) p= 0.038]. Moreover, GSVA scores of interferon-α and -γ responses were significantly higher in patients with the SLE-immunophenotype in central memory CD8+ T cells, while hedgehog signalling was higher in non-SLE-immunophenotype patients in 5 cell subsets., Conclusion: This study describes the stratification of MCTD patients based on immunophenotyping, suggesting the presence of distinct immunological processes behind the clinical subtypes of MCTD., (© The Author(s) 2024. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

The sponge associated axiid shrimp Eiconaxius farreae Ortmann, 1891 was originally described based on specimens from Sagami Bay, central Japan. Morphological examination of many specimens of Eiconaxius collected from Sagami Bay and nearby areas, and genetic data from the mitochondrial COI and 16S rRNA genes have revealed that three species are represented. This finding led to a reappraisal of the identity of E. farreae and E. mortenseni Sakai, 1992, also originally described from Sagami Bay. It has been clarified that two species (E. farreae and an undescribed species) were mixed in the syntype material of E. farreae, and consequently, a lectotype of E. farreae is designated in the interest of nomenclatural stability. Here, E. farreae and E. mortenseni are redescribed, and the third species is described as new, E. reconditus. The new species is morphologically very similar to E. mortenseni. Differentiating characters among closely allied species are reassessed. Previous records of E. farreae are reviewed in light of the present findings.

Assessing human health risks associated with inhalation exposure of volatile chemical substances (VCSs) volatilized from contaminated soil requires quantitative evaluation of volatilization fluxes (VFs) and an understanding of how environmental factors impact VF generation. We developed a numerical model that considers advection-dispersion and VCSs volatilization in unsaturated soil, enabling VF prediction through parameter optimization using soil column tests. We conducted parametric analyses to assess how key parameters, such as soil particle size, contamination depth, temperature, and surface soil thickness affect VF generation. By analyzing VCS transport near the ground surface, we uncovered the mechanisms underlying VF generation. We also identified characteristic differences in VF generation behavior linked to soil particle size and gas saturation at the ground surface. Under specific soil particle size conditions, significant VF generation occurred even when contamination was deep underground. This was primarily observed when capillary effect was pronounced, and VCSs continued to be supplied to the ground surface through upward advection. Considering the significant impact of VF generation on human health, our parametric study provides valuable insights into relationships between different parameters and VF behavior, especially under varying ground surface temperatures and surface soil thicknesses. This study contributes to developing effective remediation and risk-reduction strategies. ENVIRONMENTAL IMPLICATION: This research examines the environmental implications of volatile chemical substances (VCSs), including hazardous materials like benzene and trichloroethylene, in contaminated soil. VCSs pose health risks when they volatilize from soil. The study quantifies volatilization fluxes (VF) and elucidates the environmental factors affecting VF generation. These findings are vital for effective environmental management. By comprehending the mechanisms governing VF generation, particularly regarding soil properties like particle size, this research enhances the effectiveness of soil contamination remediation and risk reduction. It emphasizes the essential need for a comprehensive VCS assessment in contaminated soils to protect both human health and the environment., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 The Authors. Published by Elsevier B.V. All rights reserved.)

Despite the recognised risks of human exposure to mercury (Hg), the drivers of gaseous elemental mercury (GEM) emissions from the soil remain understudied. In this study, we aimed to identify the environmental parameters that affect the GEM flux from soil and derive the correlations between environmental parameters and GEM flux. Principal component analysis (PCA), factor analysis (FA), and structural equation modelling (SEM) were performed on samples from forest and non-forest sites. The associated results revealed the impact of each environmental parameter on GEM flux, either due to the interaction between the parameters or as a coherent set of parameters. An introductory correlation matrix examining the relationship between two components showed a negative correlation between GEM flux and atmospheric pressure at the two sites, as well as strong correlations between atmospheric pressure and soil temperature. In cases of non-forest open sites with no trees, the PCA and FA results were consistent, indicating that atmospheric pressure, solar irradiance, and soil moisture-defined as primary causality-are largely independent drivers of GEM flux. In contrast, the PCA and FA results for the forest areas with high humidity, tree coverage, and shade were inconsistent, confirming the hypothesis that primary causality affects GEM flux rather than consequent parameters driven by primary causality, such as air and soil temperature and atmospheric humidity. The SEM results provided further evidence for primary and consequent causality as crucial drivers of the GEM flux. This study demonstrates the importance of key primary parameters, such as atmospheric pressure, solar irradiance, and soil moisture content, that can be used to predict mercury release from soils, as well as the importance of consequent parameters, such as air and soil temperature and atmospheric humidity. Monitoring the magnitude of these environmental parameters alone may facilitate the estimation of mercury release from soils and be useful for detailed modelling of soil-air Hg exchange., Competing Interests: Declaration of competing interest The authors declare the following financial interests/personal relationships which may be considered as potential competing interests:Monami Kondo reports financial support was provided by Japan Society for the Promotion of Science. Monami Kondo reports financial support was provided by Japan Science and Technology Agency., (Copyright © 2024. Published by Elsevier Ltd.)

A new species of alvinocaridid shrimp is reported, from the Northwest Eifuku hydrothermal vent field at 16191667 m depth on the Mariana Arc. A comprehensive phylogenetic reconstruction of Alvinocarididae based on the mitochondrial cytochrome c oxidase subunit I (COI) gene including this new species reveals the paraphyly of the genus Rimicaris Williams & Rona, 1986 with four other generaAlvinocaridinides, Manuscaris, Opaepele, and Shinkaicarisnested within it. We re-examine material of these four problematic genera, and synonymise them under Rimicaris whose diagnosis has been amended, in order to maintain a monophyletic Rimicaris. Our new species, Rimicaris cambonae sp. nov. is genetically close to Rimicaris loihi (Williams & Dobbs, 1995) comb. nov. (previously Opaepele loihi) with which it co-occurs, but can be morphologically distinguished by the less elevated dorsal surface of the rostrum, this being devoid of a median carina, a stronger pterygostomial tooth on the carapace, and a blunt rather than acuminate proximolateral process on the antennular stylocerite. Species previously assigned to the above listed, synonymized genera are also discussed, with new material examined for three key species: R. loihi, R. acuminata, and R. leurokolos. Further, Alvinocaridinides formosa Komai & Chan, 2010 and Manuscaris liui Wang & Sha, 2016 are synonymized under Rimicaris leurokolos (Kikuchi & Hashimoto, 2000) comb. nov. and R. acuminata (Komai & Tsuchida, 2015) comb. nov., respectively. Revised diagnoses are presented for R. loihi, R. acuminata, and R. leurokolos. After the present revision revision, Rimicaris now consists of 15 species.

Metacrimenes fenestra, a new palaemonid genus and species is described on the basis of an ovigerous female and one juvenile specimen from Suruga Bay, central Japan. The specimens were associated with the stalked crinoid Metacrinus rotundus Carpenter, 1885, collected at a depth of 131200 m. Photographs taken in situ indicate that the shrimp lives ectosymbiotically on its crinoid host. The greatly reduced bud-like exopod on the third maxilliped links the new genus to Mesopontonia Bruce, 1967 (Indo-West Pacific) and Waldola Holthuis, 1951 (East Pacific), in which the exopod is absent, but the presence of antennal spine on the carapace and the non-elongate, symmetrical second pereopods immediately distinguish Metacrimenes n. gen. from these genera. Molecular phylogenetic analysis using a dataset concatenating two mitochondrial markers (16S rRNA and COI genes) clusters the new genus with Paraclimenes gorgonicola (Bruce, 1967), two species of Mesopontonia and Brucecaris tenuis (Bruce, 1969). However, in Paraclimenes Bruce, 1995 and Brucecaris Marin & Chan, 2006, the third maxilliped exopod is normally developed, flagellum-like. Other differentiating characters between the new taxon and the presumably allied taxa are discussed.

Two species of munidid squat lobsters are newly reported from the North-West Pacific off Japan based on material collected by the recent cruise of R/V Kaimei (KM20-10C cruise) of the Japan Agency for Marine-Earth Science and Technology, exploring seamounts in the marine protected area (MPA): Gonionida kaimei n. sp. and Trapezionida psylla (Macpherson, 1994). Gonionida kaimei n. sp. closely resembles G. tangaroa (Ahyong, 2007) and G. rubrimana (Ahyong, 2007), but morphological characters and living colouration differentiate the new species from the latter two species. Genetic analysis using two molecular markers (mitochondrial COI and 16S rRNA genes) also supports the recognition of the new species. Trapezionida psylla has been recorded from the South-West Pacific, and thus the present material greatly extends the geographic range of the species to the North-West Pacific. The identity of the present specimens is also confirmed by the genetic data, as well as the morphological evidence. This study contributes to documenting the benthic invertebrate fauna of the Japanese MPA.

A new species of the thorid shrimp genus Heptacarpus Holmes, 1900, H. sadoi, is described and illustrated on the basis of six specimens from the southeastern coast of the Boso Peninsula, central Japan. All the specimens were collected from intertidal tide pools at low tide. Heptacarpus sadoi n. sp. belongs to the species group with epipods on the pereopods 13 and appears morphologically close to H. jordani (Rathbun, 1902) known from Japan and Korea. The new species is readily differentiated from H. jordani by the short rostrum with fewer dorsal teeth and the possession of only one spine on the distal margin of the basal article of the antennular peduncle. Genetic comparison using two mitochondrial markers (COI and 16S rRNA genes) also supports that the new taxon is distinct from H. jordani.

Examination of material from Japanese waters has disclosed that two species have been confounded under Tymolus uncifer (Ortmann, 1892) (Decapoda: Brachyura: Cyclodorippidae) in previous literature. The identity of Tymolus uncifer sensu stricto is clarified in reference to the lectotype, and the second taxon is herein attributed to T. melanommus (Doflein, 1904), which was originally recognised as a forma of T. uncifer with one specimen from off Kenya and one specimen from Japan. A lectotype is herein selected for T. melanommus in the interest of nomenclatural stability. Differentiating characters between the two species are assessed in detail, and those two species are compared also with T. glaucommus (Alcock, 1894), T. similis (Grant, 1905), T. brucei Tavares, 1991, T. daviei Tavares, 1997, T. hirtipes Tan & Huang, 2000 and T. globosus Spiridonov & Trkay, 2007, all of them are considered as valid. Sexual dimorphism and ontogenetic variation are seen in the relative length and stoutness of the pereopods 2 and 3 in T. uncifer and T. melanommus, but the morphometric characters derived from the two pereopods are useful as diagnostic at comparison between same sex in adults. Comparison of partial fragments of the mitochondrial COI and 16S rRNA gene sequences strongly supports that the two taxa are distinct. Previous records of T. uncifer, including fossil records, are reviewed based on the present findings. Tymolus uncifer and T. melanommus are restricted to Japanese waters at present.

A small collection of chirostyloid squat lobsters was studied, collected from seamounts on the Nishi-Shichito, West Mariana, and Central Mariana ridges, which have been collectively designated as marine protected areas (MPAs) of Japan. Specimens were collected using a remotely operated vehicle (ROV). Four species belonging to three families were identified, of which two are new to science: Uroptychus medius n. sp. (Chirostylidae), Eumunida smithii Henderson, 1885, E. treguieri de Saint Laurent & Poupin, 1996 (Eumunididae), and Sternostylus spiniger n. sp. (Sternostylidae). Identities of those species were assessed using an integrative approach based on morphological comparison and genetic analysis of two mitochondrial markers (COI and 16S rRNA genes). The two species of Eumunida represent the first records from Japanese waters.

A new species of the alpheid shrimp genus Athanas Leach, 1814 is described based on a holotype from Wakasa Bay, Sea of Japan side of Honshu, Japan, at a depth of 90 m. Athanas exilis n. sp. appears close to A. sydnyensis Anker & Ahyong, 2007, but is distinguished from the latter by some morphological features, including the different shape of the telson, the shorter antennular stylocerite, more strongly produced distal lamella of the antennal scaphocerite, and less developed armature of the merus of the major cheliped. Preliminary genetic analysis using the mitochondrial 16S rRNA gene was performed to compare interspecific genetic divergence within the genus.

A new species of the laomediid mud shrimp genus Naushonia Kingsley, 1897 is described and illustrated based on seven specimens collected from Okinawa Island, Ryukyu Islands, southwestern Japan. All specimens of Naushonia karashimai n. sp. were collected from burrows of a large axiidean shrimp, Neaxius acanthus (A. Milne-Edwards, 1879), which inhabits seagrass beds in inner reef lagoons. The new species is easily distinguished from all 16 known congeners by its tridentate rostrum and relatively narrow pereopod 1 palm with a proximally located pollex. Sequences of the mitochondrial 16S rRNA and COI genes were newly generated for five species and two species of Naushonia, including the new species, respectively. Preliminary phylogenetic analysis using sequences of the 16S rRNA gene was performed.

Background: Eosinophilic granulomatosis with polyangiitis (EGPA) is a form of systemic vasculitis with eosinophilic inflammation. However, existing classification criteria are all designed to classify EGPA among vasculitis and there is no established method distinguishing EGPA from other eosinophilic disorders. The aim of the present study was to propose a scoring system to differentiate EGPA among eosinophilic disorders., Methods: Non-supervised hierarchical clustering using Ward's method and principal component analysis (PCA) were performed for 19 clinical parameters of 58 patients with eosinophilia-related diseases at a tertiary university hospital. The newly proposed scoring system was externally validated in 40 patients at another tertiary institution., Results: Two distinct clusters were identified, and clinical features including peripheral neuropathy, asthma, skin involvement, lung involvement, rheumatoid factor (RF) positivity, myeloperoxidase (MPO)-anti-neutrophil cytoplasmic antibody (ANCA) positivity, IgE elevation, C-reactive protein (CRP) elevation, and vasculitis pathological findings were predominantly observed in one of these clusters (p < 0.05). Ten features defining the cluster with a high rate of vasculitis were weighted by PCA to create the E-CASE (EGPA classification among systemic eosinophilia) scoring system, on a 16-point scale. Based on the distribution of scores in the primary cohort, we defined an E-CASE score ≥12 as positive, ≤ 8 as negative, and 9-11 as undeterminable. The sensitivity and specificity of the E-CASE score in the validation cohort were 93.3% and 100%, respectively., Conclusions: We developed and verified a novel scoring system for differentiating EGPA from other types of eosinophilic disorders., (Copyright © 2022 Japanese Society of Allergology. Published by Elsevier B.V. All rights reserved.)

The alpheid shrimp genus Metabetaeus Borradaile, 1899 was previously known from two species associated with anchialine pools and caves, M. minutus (Whitelegge, 1897) and M. lohena Banner & Banner, 1960, and one species inhabiting in coral reef, M. mcphersonae Anker, 2010. In this study, a new species of the genus is described based on fourteen specimens collected from Shizuoka and Mie Prefectures, Honshu, central Japan. Metabetaeus lapillicola n. sp. inhabits interstitial spaces among small pebbles deposited in lower intertidal to upper subtidal zone, representing rather unusual habitat not only for Metabetaeus, but even for carideans. It is distinguished from three congeneric species by the presence of a pterygostomial spine on the carapace, greatly unequal chelipeds with the major one being substantially elongate, and the lack of a distinct distolateral spine on the uropodal endopod. Phylogenetic analyses using the mitochondrial 16S rRNA and COI genes support the recognition of the new species. The generic diagnosis of Metabetaeus is emended to accommodate the new species.

To identify the long-term risks of malignancy in patients with myositis-specific antibody (MSA)-positive idiopathic inflammatory myopathy (IIM). This retrospective cohort study included 216 IIM patients (aged > 18 years). Of these, 109 patients were positive for antibodies against anti-aminoacyl-tRNA synthetase (ARS), melanoma differentiation-associated gene 5 (MDA5), Mi-2, and transcriptional intermediary factor 1-γ (TIF1-γ). Age- and sex-matched standardized incidence ratios (SIRs) were calculated to compare the incidence of malignancy in IIM patients to that of the general population. The malignancy-free survival rate was estimated by Kaplan-Meier methods. Our study included 109 patients, 64 with anti-ARS, 28 with anti-MDA5, 9 with anti-Mi-2, and 8 with anti-TIF1-γ antibodies; 16 and 5 patients were diagnosed with a malignancy within 3 years before or after and within 4 to 10 years after their IIM onset, respectively. The SIRs of malignancy within 3 years of IIM onset for each MSA were calculated as follows: 2.12 (95% confidence interval [CI] 0.98-4.35) for anti-ARS, 1.87 (95% CI 0.48-4.97) for anti-MDA5, 2.11 (95% CI 0.11-13.69) for anti-Mi-2, and 9.30 (95% CI 2.98-25.58) for anti-TIF1-γ antibodies. The SIR at 4 to 10 years after IIM onset in patients with an anti-MDA5 antibody was 4.62 (95% CI 1.19-14.72); other MSAs did not have statistically significant SIRs. The long-term SIR of malignancy in patients with an anti-MDA5 antibody was 4.62 (95% CI 1.19-14.72), and the SIR among patients with an anti-TIF1-γ antibody within 3 years of IIM onset was 9.30 (95% CI 2.98-25.58). Screening for malignancies in patients with late phase of IIM and an anti-MDA5 antibody may be beneficial., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

A new palaemonid species, Periclimenes variabilis, is described and illustrated on the basis of four specimens from the Ritto Seamount, West Marina Ridge, northwestern Pacific, at depths of 642-666 m. Specimens of the new species were collected with an unidentified hexacoral of Primnoidae (Cnidaria), an antipatharian octocoral Leiopathes sp. (Cnidaria), and an unidentified crinoid (Echinodermata), suggesting association with those invertebrates. Morphological comparison and molecular phylogenetic analyses using the mitochondrial 16S ribosomal RNA and cytochrome C oxidase subunit I genes indicate that the new species is closest to Periclimenes uniunguiculatus Bruce, 1990 although the possession of more than two spiniform setae on either side of the telson in the new species is consistent with the generic diagnosis of Bathymenes Kou, Li & Bruce, 2016. In reference to the close relationship to the species currently assigned to Periclimenes Costa, 1844 and the unsettled taxonomy of the genus, the new species is provisionally assigned to Periclimenes. Differentiating characters between the new species and closely related taxa are discussed.

Objective: Clinically amyopathic dermatomyositis (CADM) patients frequently develop refractory interstitial lung disease (ILD), with a poor prognosis. We aimed to verify the efficacy and safety of plasma exchange (PE) treatment for ILD in CADM., Method: A retrospective case-control study was conducted to compare clinical outcomes with and without PE treatment in CADM-ILD patients refractory to combination therapy of high-dose glucocorticoids, calcineurin inhibitors, and cyclophosphamide. Among 19 enrolled patients, 11 were further treated with PE. We compared survival rates and other clinical characteristics. PE consisted of either fresh-frozen plasma or albumin as a replacement solution., Results: Basal clinical characteristics at diagnosis, including age, gender, serum ferritin, Krebs von den Lungen-6 (KL-6), C-reactive protein, and respiratory function tests, did not differ between the two groups. The survival rate for treatment with PE was higher than for treatment without PE (91% and 50%, respectively, p < 0.05). Among PE-treated patients, anti-melanoma differentiation-associated gene-5 (anti-MDA-5) antibody titre, ferritin, and KL-6 as serological activity markers were sustainably reduced only after initiating PE. Therapeutic intervention with PE reduced the frequency of exacerbation of ILD requiring methylprednisolone pulse therapy. The occurrence of bacterial, fungal, and cytomegalovirus infection did not differ between the groups with and without PE, and adverse events associated with PE resolved with appropriate intervention., Conclusion: Combination therapy with PE was associated with an improved survival rate, and may be effective for the management of refractory ILD in CADM patients. A personalized therapeutic strategy including PE could be introduced for fatal rapidly progressive ILD.

Gold (Au) mining area is known to be one of the major sources of toxic elements; however, the potential risks of toxic elements from abandoned Au mines to the surrounding river basin districts and human exposure pathways to toxic elements need to be clarified. In this study, the distribution and mobilities of nine toxic elements (As, Cd, Cu, Pb, Sb, Zn, Cr, Ni and V) in Kesennuma City, Tohoku Region in Japan, a typical Au-mining district with several river basins, were studied through a geochemical survey (including element total concentrations and water-/acid-leaching concentrations determinations, as well as GB calculations), and environmental assessment on these elements in soil, river sediment, and river water samples from the study area. The contamination evaluation by index of geo-accumulation (I geo ) and enrichment factor (EF) suggested that As, Cu, Ni and Sb enrichments were greatly observed in the vicinity of the abandoned Au mines; moreover, calculated GB upper values for Cu in the river sediment surpass that of Tohoku Region. It has been found in this study that each element has particular mobility, which eventually influences its exposure pathway to humans. For instance, As in soil and sediment poses adverse non-carcinogenic risks and unacceptable carcinogenic risks to especially children mainly through groundwater ingestion. To minimize the potential risks associated with exposure to toxic elements in Au-mining districts, effective risk management measures should be implemented around river system by Au-mining companies even after their long-time closures, based on the consideration of each element's mobility., (© 2022. The Author(s), under exclusive licence to Springer Nature B.V.)

The influence of flow channels on the leaching behavior of toxic elements in contaminated soil cannot be neglected in a column percolation test. This study presents a visualization of the flow channel formed in the soil and evaluated the relationship between the leaching behavior of soil components and flow. We conducted column percolation tests with two types of filling methods (Compaction and No compaction) and used X-ray computed tomography to visualize the soil structure and non-uniform flow. Additionally, the variations of flow in a cross section of water were evaluated using hydraulic conductivity based on differential pressure. Under No compaction, a flow occurred throughout the soil column at the beginning of the water passage, but a non-uniform flow emerged as the liquid-solid ratio increased; under Compaction, a non-uniform flow was formed from the beginning of water passage. The leaching behavior of the major components and toxic elements from soil with high adsorptive properties was significantly affected by the filling method up to a liquid-solid ratio of 2. These results suggest that the non-uniform flow formed in the column percolation test has a significant impact on the leaching of soil components., Competing Interests: The authors declare no conflict of interest., (© 2022 The Authors.)

A new species of the alpheid shrimp genus Salmoneus Holthuis, 1955 is described based on two specimens from the Seto Inland Sea, Japan. Salmoneus aduncus n. sp. is strikingly similar to S. rostratus Barnard, 1962, which is distributed widely in the tropical Indo-West Pacific, but the new species is distinguished from the latter by the proportionally wider telson, the less pronounced longitudinal grooves on the major cheliped palm, and the weaker occlusal teeth on the major cheliped fingers. The living colouration is also different between the two. Phylogenetic analysis using the mitochondrial 16S rRNA gene supports the recognition of the new species. Salmoneus is primarily distributed in tropical and subtropical waters, and in temperate waters in East Asia, only S. gracilipes Miya, 1972, has been the sole representative of the genus. The new species is the second to be recorded from the warm temperate areas in East Asia, although its real distribution remains to be clarified.

Protein-losing enteropathy (PLE) has been reported to be associated with various systemic autoimmune diseases. However, reports regarding PLE in ANCA-associated vasculitis (AAV) patients are limited. We herein aimed to describe the clinical characteristics of AAV with PLE. We conducted a retrospective chart review of patients who were diagnosed with AAV and who began treatment at the University of Tokyo Hospital between June 2003 and June 2020. Among 68 AAV patients, there were four patients (5.9%) with PLE, consisting of two patients with MPA, one patient with GPA, and one patient with EGPA. Clinical courses were described, and their data were compared with AAV patients without PLE. Demographic characteristics, disease activity, and the pattern of organ involvement were similar between patients with PLE and without PLE. Patients with PLE had hypocomplementemia more frequently than the patients without PLE (CH50 75.0% vs 1.8%, p < 0.001, C3 50.0% vs 1.8%, p = 0.01, C4 75.0% vs 3.5%, p = 0.001). Although hypoalbuminemia improved with immunosuppressive therapy for AAV, the improvement in hypoalbuminemia was slow in most cases. We also performed a systematic review on PLE associated with vasculitis. Thirteen reports were included, and Henoch-Schonlein Purpura patients with PLE also tended to have hypocomplementemia. In conclusion, PLE is a rare complication of AAV and complement system may associate with the mechanism of PLE., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

The ghost shrimp genus Callianassa Leach, 1814 is currently represented by six taxa, of which five are known from the eastern Atlantic and only C. persica Sakai, 2005 from the Persian Gulf. In this study, a new species of Callianassa is described and illustrated on the basis of material from the subtidal soft sediments (at 2628 m depth) in Wakasa Bay, western Japanese coast of the Sea of Japan. Callianassa ogurai n. sp. is the first species of the genus from the Pacific region. This study discusses characters differentiating the new species and other five congeneric species. Molecular phylogenetic analysis using the mitochondrial 16S rRNA gene supports the close relationship between the new species and C. subterranea (Montagu, 1808), warranting the generic assignment of the new species to Callianassa sensu stricto. We also explore the contentious taxonomic status of C. timiris (Sakai, Trkay, Beck Freiwald, 2015).

The diogenid hermit crab genus Pseudopaguristes McLaughlin, 2002 is currently represented by 15 species worldwide. In this study, we describe a new species of the genus, P. flavioculus, based on a unique male holotype from off Sarigan, Northern Mariana Islands, Micronesia, at depth of 131133 m. The new species appears similar to the type species of the genus, P. janetkae McLaughlin, 2002, but the lack of conspicuous spines on the dorsal face of the right cheliped carpus, the possession of a median row of tiny spines on the dorsal face of the left cheliped carpus, and different colour pattern of the cephalic appendages and the ambulatory legs distinguish P. flavioculus n. sp. from P. janetkae. Pseudopaguristes has been differentiated from the two allied genera, Paguristes Dana, 1851 and Areopaguristes Rahayu, 2012, by the possession of eight functional pairs of gills (versus 13 pairs in Paguristes and 12 pairs in Areopaguristes), but our examination of the new species and three other congeneric species available for study clarifies that the development of arthrobranchs on the maxillipeds 3 and chelipeds is rather variable in species currently assigned to Pseudopaguristes. Careful observation of the anterior arthrobranchs is recommended to avoid misinterpretation in the counts of gill numbers.

Comparison between materials from various localities in the North Pacific and North Atlantic, including specimens identified with Lebbeus polaris (Sabine, 1824) in previous studies, led the authors to reinstate Hippolyte St. Pauli Brandt, 1851 as a valid species of the thorid genus Lebbeus White, 1847 (Decapoda: Caridea), removed from the synonymy of L. polaris. Lebbeus sanctipauli is distinguished from L. polaris by the rostrum with fewer dorsal teeth in female and a better developed ventral blade in male, fewer dorsolateral spiniform setae on the telson (three or four, rarely five versus six to 12), the longer antennular stylocerite in females, stouter pereopods 35 with fewer accessory spiniform setae on pereopods 35 dactyli (three or four versus five to seven) and the possession of an epipod on the pereopod 3. Although L. polaris has been thought to have a circumpolar distribution, the occurrence of the species in the North Pacific Ocean needs to be verified.

Nawalparasi-West/Parasi is one of the severely affected districts in the Terai lowlands of Nepal by arsenic (As) contamination in groundwater, exceeding standards of 10 ppb (WHO) and 50 ppb (Nepal Drinking Water Standard). This study presents the spatial and temporal distribution of As across 6 km × 10 km region in Parasi via meteorological, hydrogeological, physio-chemical, and sedimentological investigations in 31 communities for about 5 years. In this study, water balance analysis was carried out for understanding the groundwater dynamics in the study area and its contribution to As elution. Gentle flow gradient and little to no infiltration was observed in the central region with relatively impervious silty clayey flood plain, where higher As concentrations were obtained compared to the northern Siwalik foothills and southern parts with coarser sediments. Similarly, higher As concentration (1048 ppb) was recorded in the drier pre-monsoon season than the wet season (529 ppb). The aquifer at 12 to 23 m depth feeding 73% wells in the study area exhibited higher As concentration in reduced environment as opposed to the oxidizing state at 5- to 6-m and 30- to 50-m deep aquifers. Other constituents such as Fe, B, and Cr and their relation with As were analyzed. The results of GERAS model analysis done for health risk assessment are also presented which show that under long-term exposure, the residents in Parasi were undertaking intolerable cancer risk of 1.1 to 6.4 × 10 -3 . This study further incorporates socio-economic sentiments vital to analyze, and propose sustainable and cheap countermeasures for immediate implementation to reduce As exposure and health risk in Nepal, which is also highly applicable for other affected regions in South Asian Region., (© 2022. The Author(s), under exclusive licence to Springer Nature Switzerland AG.)

There have been hundreds of reports on mutations in the NLRP3 gene related to NLRP3-associated autoinflammatory disease, but few of these mutations have occurred as both germline and somatic mosaic mutations. In this case-based review, we report a 68-year-old man with an NLRP3-associated autoinflammatory disease. He developed secondary amyloidosis, including a renal and colorectal presentation in his 50 s. Sequencing of the NLRP3 gene revealed an I574F somatic mosaic mutation, which has up to now only been reported in germline mutations. The patient was treated with canakinumab, which had great efficacy not only on the NLRP3-mediated inflammation, but also on the chronic renal failure and proteinuria provoked by secondary renal amyloidosis. To evaluate the effectiveness of canakinumab, we conducted a literature research on renal amyloidosis related to NLRP3-associated autoinflammatory disease treated with canakinumab. Although our patient had a relatively long medical history and greater amounts of proteinuria than other reported cases, canakinumab had great efficacy on renal impairment, in similar to other reported cases. Along with the first report of a late-onset I574F somatic mosaic mutation in NLRP3-associated autoinflammatory disease, this report demonstrates the effectiveness of canakinumab on renal amyloidosis, probably through the way that IL-1β blockade minimizes podocyte injury., (© 2022. The Author(s), under exclusive licence to International League of Associations for Rheumatology (ILAR).)

A new species of the deep-sea caridean genus Glyphocrangon A. Milne-Edwards, 1880, G. obtusis n.sp., is described and illustrated on the basis of the material collected in the South China Sea off the Pratas Island. It appears close to G. hakuhoae Takeda Hanamura, 1994 and G. robusta Komai, 2004 among the 93 described species in Glyphocrangon. From G. hakuhoae, the new species is distinguished by the rostrum with less developed convexity on the dorsolateral margin and lacking transverse septa, and the less elevated median carinae on the pleomeres 15. From G. robusta, the new species differs in lacking transverse septa at the rostrum, the fourth carina on the carapace with the two anterior parts unaligned and the posterior part divided into four lobes. Molecular genetic analysis using the barcoding segment of the mitochondrial COI gene supports the establishment of the new species.

Based on a collection of deep-water caridean shrimps captured by commercial baited traps and gill nets operated in Nemuro Strait, southwestern part of the Sea of Okhotsk, three new species of the thorid shrimp genus Lebbeus White, 1847, L. rufomaculosus n. sp., L. subtilis n. sp. and L. tenuipes n. sp., are described and illustrated. Integrative approach using morphological comparison and genetic analyses with the mitochondrial COI and 16S rRNA genes is taken to clarify specific identities of those newly recognized taxa. In addition, an aberrant male specimen of L. fujimotoi Matsuzaki, Hibino Komai, 2015 is reported, of which the identity is confirmed by genetic analyses.

The deep-sea caridean shrimp Bathypalaemonella pandaloides (Rathbun, 1906) (Bathypalaemonellidae) was heretofore known only from Hawaii (type locality) and French Polynesia. In this article, we report this rarely collected species on the basis of a single male specimen from the Ritto Seamount on the West Mariana Ridge, northwestern Pacific, at a depth of 657 m, collected by means of a remotely operated vehicle (ROV). The specimen is intact, enabling us to describe the characteristic major pereopod 2 of the species for the first time. The closest relative is B. zimmeri Balss, 1914, known only from the western Indian Ocean; fewer accessory spiniform setae on the pereopods 35 dactyli and the less produced distal lamella of the antennal scaphocerite distinguish B. pandaloides from B. zimmeri. K2P divergences of the mitochondrial COI and 16S rRNA genes in taxa attributed to Bathypalaemonella Balss, 1914 are presented. The present specimen was collected from an anthipatharian octocoral colony, Leiopathes sp. (Cnidaria), suggesting a commensal relationship.

Introduction: GATA1 mutation plays an important role in initiating transient abnormal myelopoiesis (TAM) and in the clonal evolution towards acute megakaryoblastic leukaemia (AMKL) associated with Down syndrome (DS). This study aimed to develop and validate the clinical utility of a complementary DNA (cDNA) analysis in parallel with the conventional genomic DNA (gDNA) Sanger sequencing (Ss), as an initial screening test for GATA1 mutations., Methods: GATA1 mutations were evaluated using both gDNA and cDNA in 14 DS patients using Ss and fragment analysis (FA), respectively., Results: The detection sensitivity of conventional gDNA sequencing was limited in low blast percentage TAM (LBP-TAM); however, cDNA-based Ss readily detected all the pathognomonic GATA1 mutations. The cDNA-based FA readily detected GATA1 frameshift mutation with a reliable sensitivity ranging from 0.005% to 0.01% of clonal cells., Conclusions: GATA1 mutations are heterogeneous; therefore, we would like to propose a dual cDNA and gDNA analysis as a standard diagnostic approach, especially for LBP-TAM. cDNA-based FA promises an excellent sensitivity for detecting frameshift GATA1 mutations in the longitudinal clonal evolution towards AMKL without using a patient specific primer., (© 2021 John Wiley & Sons Ltd.)

Introduction: To eliminate the disparity and maldistribution of physicians and medical specialty services, the development of diagnostic support for rare diseases using artificial intelligence is being promoted. Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is a rare disorder often requiring special knowledge and experience to diagnose. In this study, we investigated the possibility of differential diagnosis of IgG4-RD based on basic patient characteristics and blood test findings using machine learning., Methods: Six hundred and two patients with IgG4-RD and 204 patients with non-IgG4-RD that needed to be differentiated who visited the participating institutions were included in the study. Ten percent of the subjects were randomly excluded as a validation sample. Among the remaining cases, 80% were used as training samples, and the remaining 20% were used as test samples. Finally, validation was performed on the validation sample. The analysis was performed using a decision tree and a random forest model. Furthermore, a comparison was made between conditions with and without the serum IgG4 concentration. Accuracy was evaluated using the area under the receiver-operating characteristic (AUROC) curve., Results: In diagnosing IgG4-RD, the AUROC curve values of the decision tree and the random forest method were 0.906 and 0.974, respectively, when serum IgG4 levels were included in the analysis. Excluding serum IgG4 levels, the AUROC curve value of the analysis by the random forest method was 0.925., Conclusion: Based on machine learning in a multicenter collaboration, with or without serum IgG4 data, basic patient characteristics and blood test findings alone were sufficient to differentiate IgG4-RD from non-IgG4-RD., (© 2022. The Author(s).)

A new species of the alpheid shrimp genus Richalpheus Anker Jeng, 2006 is described on the basis of 10 specimens collected from three locations in Kagoshima Prefecture, Kyushu, Japan. Richalpheus laeuadigitus n. sp. appears close to R. palmeri Anker Jeng, 2006 and R. dahabensis Anker Dworschak, 2007 among the three known congeners, but differs from R. palmeri in the proportionally wider telson and the more proximally located tip of the antennular stylocerite, and from R. dahabensis in the different configuration of the major cheliped fingers, in particular the possession of a well-developed bulge-like ridge on the occlusal margin of the dactylus. Two sequences of the mitochondrial 16S rRNA gene from two of the paratypes are newly obtained, and compared with that of R. palmeri downloaded from the GenBank database. The new species is the fourth representative of Richalpheus. Possible association between the new species and other infaunal invertebrates is suggested, although not specified.

Understanding the diffusive transport behavior of volatile organic compounds (VOCs) in near-surface soils is important because soil VOC emissions affect atmospheric conditions and climate. Previous studies have suggested that temperature changes affect the transport behavior; however, the effect of these changes are poorly understood. Indeed, under dynamic temperature conditions, the change in VOC flux is much larger than that expected from the temperature dependency of the diffusion coefficient of VOCs in air. However, the mechanism is not well understood, although water in soil has been considered to play an important role. Here, we present the results of experiments for the upward vertical vapor-phase diffusive transport of two VOCs (benzene and tetrachloroethylene) in sandy soil under sinusoidal temperature variations of 20-30 °C, as well as its numerical representation. The results clarify that the unexpectedly large changes in emission flux can occur as a result of changes in the VOC concentration gradient due to VOC release (volatilization) from/trapping (dissolution) into water, and that such flux changes may occur in various environments. This study suggests the importance of a global evaluation of soil VOC emissions by continuous measurements in various soil environments and/or predictions through numerical simulations with thorough consideration of the role of water in dynamic soil environments., (© 2022. The Author(s).)

Polymyalgia rheumatica (PMR) is an inflammatory rheumatic disease characterized by stiffness and aching mainly in the shoulders, neck and hip girdles. The underlying pathogenesis of PMR involves myeloid lineage activation with a high expression of pattern recognition receptors. In addition, vaccination against severe acute respiratory syndrome coronavirus 2 with mRNA-1273 functions as both an immunogen and intrinsic adjuvant. It leads to the activation of innate immunity, resulting in antibody production. We herein report the first case of PMR-like syndrome seven days after mRNA-1273 vaccination. Reassuringly, the symptoms, such as pain of the neck, shoulder girdle and pelvic girdle, as well as elevated inflammatory markers were resolved within a month without glucocorticoid or immunosuppressant administration.

Soil moisture is a key factor for mercury (Hg) emission from soil. Despite its significance for Hg emissions, the effect of soil moisture on Hg flux and fractions has not been thoroughly investigated. The objective of this study was to elucidate the influences of soil moisture and temperature on Hg fluxes from soils and Hg fractions. A kinetic study was performed to measure Hg emission fluxes of six soil samples under different temperature (T) (15 °C, 20 °C, 25 °C, 30 °C, and 35 °C) and moisture conditions (0%, 10%, and 20% added water). The results showed that the Hg fluxes increased with increases in T and soil moisture. A linear correlation was found between ln (Hg emission flux) and 1/T for the six soil samples at different moisture contents (R 2  = 0.73-0.99). The range of activation energy (Ea) values was 25.31-57.86 kJ/mol. The Hg fractions in soils of different moisture content were determined by a sequential extraction method. The results demonstrated that soil moisture affected the Hg fractions in soils. The Ea values had different relationships with soil moisture in different soils. There were correlations between Ea and the elemental and mercuric sulfide fractions for air-dried soils. However, for moist soils, Ea was negatively correlated with the water-soluble and acid-soluble fractions. Collectively, the combination of the Hg emission kinetics and Hg fraction measurement of different moist soils indicated that Hg emission was affected by both total Hg concentration and Hg fractions., (Copyright © 2021 Elsevier Ltd. All rights reserved.)

The poorly known pinnotherid crab, Pinnixa haematosticta Sakai, 1934, is redescribed on the basis of female holotype and additional specimens, including males and females, from Japan. The male characters of the species are documented for the first time. The species is transferred to Indopinnixa Manning Morton, 1987 because the male pleonal somites 35 are functionally fused, the telson is wide, and the maxilliped 3 with the dactylus is distinctly longer than the propodus. Indopinnixa kumejima Naruse Maenosono, 2012 is morphologically very similar to I. haematosticta n. comb., but a molecular phylogenetic analysis using partial fragments of the mitochondrial 16S rRNA gene supports the hypothesis that the two taxa as distinct. Morphologically, the two species are differentiated by the shape of the pereopod 4 merus (proportionally wider in I. haematosticta n. comb. than in I. kumejima). Our findings suggest that I. haematosticta n. comb. and I. kumejima are geographically distinct: I. haematosticta appears restricted to the Japanese mainland (Honshu to Kyushu) and Ohsumi Islands, while I. kumejima occurs in the Ryukyu and Ogasawara Islands.

We describe three new species of hermit crab of the pagurid genera, Catapagurus A. Milne-Edwards, 1880, Nematopagurus A. Milne-Edwards Bouvier, 1892 and Pagurixus Melin, 1939, on the basis of material collected during the scientific cruise to the Northern Mariana Islands conducted by the U.S. National Oceanographic and Atmospheric Administration (NOAA) in 2015. Catapagurus tenuilamina n. sp., referred to the C. ensifer Henderson, 1893 species group, appears close to C. granulatus Edmondson, 1951, but differs from the latter in the more slender ultimate article of the antennular peduncle, more slender dactyli of pereopods 2 and 3, and the possession of two dorsal subdistal spines in the meri of the pereopods. Nematopagurus marianicus n. sp. resembles N. gardineri Alcock, 1905 and N. lewinsohni Trkay, 1986, but is distinguished from the latter two by the characteristic dense setation on the upper part of the left cheliped carpus and by the elongate dactyli of pereopods 2 and 3 that lack a row of bristle-like setae on the dorsal margins. Pagurixus eminens n. sp. is referred to the P. boninensis Melin, 1939 species group and appears closest to P. annulus Komai Poupin, 2013, but is differentiated from the latter in the shape and ornamentation of the right coxa of pereopod 5 in the male as well as details of the colour pattern on the chelipeds and ambulatory legs. Partial sequences of the mitochondrial 16S rRNA gene from the three new species were sequenced and deposited in the GenBank database for future genetic and phylogenetic study.

Idiopathic inflammatory myopathies (IIMs) are autoimmune diseases predominantly affecting proximal muscles; paraspinal muscle involvement is relatively rare. Because paraspinal myopathies do not always cause clinically evident symptoms, the diagnosis of IIMs with axial myopathies can be challenging. Anti-Ku autoantibodies, initially reported in polymyositis/systemic sclerosis overlap syndrome, are myositis-associated antibodies observed in patients with a wide variety of connective tissue diseases. Few reports have been published demonstrating predominant axial myopathy in IIM patients with anti-Ku antibodies. Herein, we investigated a previously healthy Japanese woman in her early 70s who presented with Raynaud's phenomenon, back pain, and exertional dyspnoea. The creatine kinase was elevated and antinuclear antibody staining was positive, but myositis-specific antibodies were negative. Magnetic resonance imaging revealed myocarditis and a wide range of axial muscle inflammation, including bilateral thoracolumbar paraspinal, infraspinatus, and trapezius muscles. The muscle biopsy was consistent with IIM. In addition, anti-Ku antibody was positive. The administration of prednisolone and tacrolimus quickly alleviated the symptoms, and the creatine kinase level returned to normal. The diagnosis of IIM was arduous in this case because she did not present with camptocormia, muscle weakness involving the proximal limbs was not apparent, and myositis-specific antibodies were negative. Whether axial myopathy and myocarditis are more prevalent in IIM patients with than without anti-Ku antibodies is uncertain. Clinicians should suspect axial myopathy and myositis-associated antibodies, such as anti-Ku antibodies, especially in patients in whom muscle weakness of the proximal limbs is not noticeable., (© Japan College of Rheumatology 2021. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Background: Multi-parametric flow cytometry (MFC) is a helpful tool for detecting neoplastic cells in malignant lymphoma; however, lymphoma cells can be difficult to detect when characteristic immunophenotypic abnormalities are not evident. We evaluated the stainability of VS38, which is used for multiple myeloma, in normal and abnormal B cells using MFC to develop a new strategy for detecting lymphoma cells., Methods: We compared the median fluorescence intensity of VS38 staining in lymphocytes from patients without hematopoietic neoplasms and in B cells from 26 patients with B cell lymphoma (BCL). To evaluate the performance of VS38 gating, we compared VS38-positive B cells with the percentages of BCL cells, and with the mutation ratios of MYD88 L265P measured by droplet digital PCR in patients with lymphoplasmacytic lymphoma (LPL)/Waldenström macroglobulinemia (WM)., Results: CD27-positive memory B cells were stained with VS38, whereas normal lymphocytes were faintly stained. Lymphoma cells were stained with VS38 in 11 of 12 patients with LPL/WM, 3 of 3 with chronic lymphocytic leukemia, 3 of 5 with mantle cell lymphoma, 2 of 4 with follicular lymphoma, and 1 of 1 with splenic marginal zone lymphoma. The percentages of VS38-positive B cells in VS38-positive BCL were equivalent to those of lymphoma cells and the mutation ratios of MYD88 L265P in LPL/WM., Conclusions: VS38 identified neoplastic cells in plasma cell disorders and BCL. This might improve the accuracy of BCL diagnosis, especially in patients with LPL/WM., (© 2021 International Clinical Cytometry Society.)

Competing Interests: Conflicts of interest and sources of funding: T.K. has received speaking fees from Tanabe Mitsubishi, Kissei, and received grants and speaking fees from GlaxoSmithKline. H.H. has received speaking fees and/or honoraria from Pfizer, Nihon pharmaceutical, Taiho, and Novartis. H.T. has received speaking fees from Sanofi. H.S. has received fees from Pfizer, Bristol-Myers Squibb, Eli Lilly, Sanofi, Abbvie, GlaxoSmithKline, Chugai, UCB, Takeda, Astellas, and Daiichi-Sankyo. K.F. has received speaking fees, and/or honoraria from Tanabe Mitsubishi, Bristol-Myers Squibb, Eli Lilly, Chugai, Jansen, Pfizer, Ono, AbbVie, Ayumi, Astellas, Sanofi, Novartis, Daiichi Sankyo, Eisai, Asahi Kasei, Japan Blood Products Organization, and Kowa, and has received research grants from Tanabe Mitsubishi, Bristol-Myers Squibb, Eli Lilly, Chugai, AbbVie, Ayumi, Astellas, Sanofi, Eisai, Tsumura & Co, and Asahi Kasei. There is no funding to declare for the research.

A new species of the processid shrimp genus Nikoides Paulson, 1875, N. subdistalis, is described and illustrated on the basis of five specimens collected from Kushimoto, Wakayama Prefecture, Japan, at depths of 712 m. The new species appears most similar to N. maldivensis Borradaile, 1915 among the 10 known congeners, but is notable in the unique dentition of the rostrum and the characteristic shape of the antennular stylocerite. A partial segment of the mitochondrial 16S rRNA gene was sequenced from one of the paratypes of the new species, and genetic divergence among four congeneric taxa, of which three was downloaded from the GenBank database, is shown. An identification key to 11 species of Nikoides, including the new species, is given.

Improving geothermal systems through hydraulic stimulation to create highly permeable fractured rocks can induce seismicity. Therefore, the technique must be applied at a moderate intensity; this has led to concerns of insufficient permeability enhancement. Adding chemical stimulation can mitigate these issues, but traditional methods using strong mineral acids have challenges in terms of achieving mineral dissolution over long distances and highly variable fluid chemistry. Here, we demonstrate a novel chemical stimulation method for improving the permeability of rock fractures using a chelating agent that substantially enhances the dissolution rate of specific minerals to create voids that are sustained under crustal stress without the challenges associated with the traditional methods. Applying this agent to fractured granite samples under confining stress at 200 °C in conjunction with 20 wt% aqueous solutions of sodium salts of environmentally friendly chelating agents (N-(2-hydroxyethyl)ethylenediamine-N, N', N'-triacetic acid and N, N-bis(carboxymethyl)-L-glutamic acid) at pH 4 was assessed. A significant permeability enhancement of up to approximately sixfold was observed within 2 h, primarily due to the formation of voids based on the selective dissolution of biotite. These results demonstrate a new approach for chemical stimulation., (© 2021. The Author(s).)

Examination of material from various Japanese localities clarified that the estuarine crab Ptychognathus glaber Stimpson, 1858 is possibly restricted to the Ogasawara Islands, Japan. Specimens from the Ryukyu Islands, thought to represent P. glaber, actually represent P. lipkei N.K. Ng, 2010 instead, previously known only from Cebu, the Philippines. Ptychognathus glaber is redescribed in detail, and differentiating characters between it and P. lipkei are reassessed. Previous literature records of P. glaber are reviewed in the light of our findings. Genetic analysis using partial sequences of the mitochondrial 16S rRNA gene suggests that P. glaber is genetically closer to P. ishii Sakai, 1939 than to P. lipkei, and thus P. ishii is also compared morphologically with P. glaber.

Objective: The immature platelet fraction (IPF) represents recently produced platelets in bone marrow and this parameter is increased in patient with primary immune thrombocytopenia (ITP). We investigated the associations between IPF and absolute immature platelet count (AIPC), and clinical parameters in systemic lupus erythematosus (SLE), which has more complex pathological mechanisms than in primary ITP., Methods: Patients with SLE were retrospectively reviewed at the University of Tokyo Hospital from May, 2012 to January, 2021. The correlations between clinical parameters and the number of immature platelets were assessed with Spearman's rank correlation coefficients. A multiple logistic regression model was used to identify the independent clinical parameters for IPF and AIPC. The difference in the distribution of time for a complete response (CR) after prednisolone (PSL) administration was also evaluated by log-rank test., Results: A total of 282 SLE patients were enrolled, and 12.41% of those patients showed thrombocytopenia. IPF correlated with clinical parameters such as platelet count (r = -0.58), AIPC (r = 0.64) and systemic lupus erythematosus disease activity index 2000 (SLEDAI-2K) (r = 0.24). SLEDAI-2K [odds ratio (OR) (per unit increase), 1.07; 95% CI, 1.013 - 1.13] and thrombocytopenia (OR, 32.23; 95% CI, 11.072 - 93.80) were independent clinical parameters to account for IPF increase. IPF correlated with the number of bone marrow megakaryocytes (n = 19, r = 0.57). Notably, the probability of CR in response to PSL in AIPC-high patients was higher than in AIPC-low patients (hazard ratio, 4.62; 95% CI, 1.07 - 20.02)., Conclusion: IPF correlated with disease activity of SLE and represented platelet production in the bone marrow, whereas AIPC predicted a rapid response to steroids in thrombocytopenic patients with SLE.