Relationship between the floating toe and the center-of-pressure position in an upright posture among students.

[Purpose] The incidence of floating toes in children is increasing. Although the anteroposterior center of pressure in children is present posteriorly, its relationship with the floating toe is unclear. This study aimed to clarify the relationship between the position of the anteroposterior center of pressure and the floating toe in an upright posture in children. [Participants and Methods] In this cross-sectional study, a Win-Pod (Medicapteurs) platform was used to measure the position of the anteroposterior center of pressure in 208 boys and 195 girls from Japanese elementary schools. Using images of the plantar footprint, floating toes were assessed and the floating toe score was calculated. [Results] The anteroposterior center of pressure position was situated 32.3 ± 8.2% from the heel. The floating toe score of all the participants was 3.5 ± 2.4, with a very high rate of 98%. The floating toe score had a significant, moderate correlation with age, height, weight, and the anteroposterior center-of-pressure position. Multivariate analysis revealed an association between the floating toe score and the anteroposterior center-of-pressure position, height, and weight. [Conclusion] There is significant relationship between the anteroposterior center-of-pressure position and the floating toe score in an upright posture in Japanese elementary school students.

Zamamiphidins B and C, Manzamine-Related Alkaloids from an Amphimedon sp. Marine Sponge Collected in Okinawa.

Zamamiphidins B (1) and C (2), two new manzamine-related alkaloids with an unprecedented fused diazahexacyclic ring system, were isolated from an Amphimedon sp. marine sponge collected in Okinawa. The structures of zamamiphidins B (1) and C (2) including the relative configurations were elucidated on the basis of spectroscopic data.

Response of an Indicator Species, Dryopteris crassirhizoma, to Temporal and Spatial Variations in Sika Deer Density.

Identifying appropriate indicator species for the impact of deer on forest vegetation is crucial for forest management in deer habitats and is required to be sensitive to temporal and spatial variations in deer density. Dryopteris crassirhizoma was selected as a new indicator to evaluate the response to these variations. We examined the population-level characteristics, morphological characteristics at the individual level, and grazing intensity of D. crassirhizoma at temporally different deer density sites in Hokkaido, Japan. The response of D. crassirhizoma to spatial variation in deer density was also examined within and between two regions in Hokkaido, Japan. Although the population-level characteristics and morphological characteristics did not significantly respond to short-term decreases in deer density, grazing intensity significantly decreased with decreasing deer density. The grazing intensity was also positively related to the spatial variation of deer density within both regions, but the estimated coefficient of the grazing intensity differed between regions. We concluded that D. crassirhizoma can be a useful indicator species of the impact of deer on forest vegetation. The grazing intensity of the indicator species was sensitive to temporal and spatial variations in deer density within the region.

Rapid Improvement in Jaundice Using Transdermal Isosorbide Tape as a Nitric Oxide Donor in Two Adult Patients with Transplantation-associated Microangiopathy Related to Graft-versus-host Disease.

Two adult patients with acute leukemia developed transplantation-associated microangiopathy (TAM) related to graft-versus-host disease (GVHD). Both patients were resistant to standard therapy for TAM and GVHD, which led to markedly elevated serum total bilirubin levels of 47.5 and 10.6 mg/dL, respectively. Transdermal isosorbide tape as a nitric oxide donor was applied to Patients 1 and 2 on post-transplantation days 60 and 66, respectively, which rapidly improved their jaundice after 1 day. This is the first report to describe the efficacy of transdermal isosorbide tape for adult patients with jaundice associated with TAM related to GVHD.

Lessons Learned in Practice with Li-Fraumeni Syndrome: LFS-Related Breast Cancer Treatment Strategy and Establishment of a Surveillance System.

We herein present the case of a 33-year-old woman with no family history of metachronous bilateral breast cancer and osteosarcoma, diagnosed with Li-Fraumeni syndrome (LFS), which is a rare autosomal dominant hereditary cancer syndrome associated with a germline TP53 variant. She was diagnosed with left distal femoral osteosarcoma at the age of 16, and metachronous bilateral breast cancer at the ages of 29 and 33. When the third cancer was diagnosed, a hereditary tumor syndrome was suspected and the patient was referred to our genetic outpatient clinic. There was no family history of the 'core' cancers for LFS, but since the patient met Chompret's criteria, germline TP53 genetic testing was performed with the patient's will. A pathogenic variant, TP53:c.216dupC (p.Val73ArgfsX76) was found in exon 4 of the gene. This case is didactic because radiotherapy was performed on the first breast cancer before the diagnosis of LFS was made; radiation should be avoided if there are other options in LFS because of the inability to repair DNA damage. As a lesson learned, oncologists reaffirmed the importance of being aware of hereditary tumors from the keywords "multiple," "young," "familial," and "rare," and consulting the genetic department. In addition, surveillance using whole-body magnetic resonance imaging is recommended in LFS. However, this system is not yet provided nationwide, but we have newly settled it in our hospital.

[Examination of Criterion Validity about the Standard Language Test of Aphasia].

The Standard Language Test of Aphasia (SLTA) is the most frequently used comprehensive aphasia rating scale in Japan. Although the SLTA has been verified for reliability, verification for validity is inadequate. The purpose of this study was to examine criterion-related validity of the SLTA. The SLTA was performed on patients who had passed 3months or more after onset of the aphasia-causing disease such as stroke, and the Japanese version of the Western Aphasia Battery (WAB) was subsequently performed. We investigated age, gender, disease, and calculated Spearman's rank correlation coefficient for total score and each item of the SLTA and the WAB. There were 20participants (14males, 6females), with a mean age of 68.5±12.5years. Correlations of the SLTA and the WAB were as follows: SLTA total index score and WAB aphasia quotient; r=0.870 (P<0.001), SLTA Writing factor and WAB (VI) Writing; r=0.852 (P<0.001), SLTA writing instructions and WAB writing instructions; r=0.807 (P<0.001). Many of the correlations of Z-scores between sub-tests were r≥0.7. The SLTA has criteria-related validity and now the aphasia test that has been tested for reliability and validity. (Received July 22, 2019; Accepted March 4, 2020; Published July 1, 2020).

[Successful cord blood transplantation for myelodysplastic syndrome with Behçet disease-like refractory stomatitis and multiple ileocecal ulcerations].

A 12-year-old boy was diagnosed with aplastic anemia. He was followed as an outpatient without medication, and his cytopenia improved after several years. When he was 26 years old, an annual medical checkup revealed leukocytopenia, and at the age of 31 years, he was diagnosed with myelodysplastic syndrome (MDS), refractory cytopenia with multilineage dysplasia. Chromosomal analysis of his bone marrow cells revealed trisomy 8. Ten months after being diagnosed with MDS, he developed refractory stomatitis. Two months later, he experienced abdominal pain and bloody stool, and simple punched-out ulcers similar to intestinal Behçet's disease (BD) were noted in the terminal ileum on colonoscopy. Steroids, mesalazine, and adalimumab were ineffective. Nineteen months after the MDS diagnosis, he underwent cord blood transplantation from an HLA 1-locus mismatched unrelated donor in accordance with a non-myeloablative pretransplant conditioning regimen. The patient's stomatitis and ileocecal ulcers improved following the transplantation. Currently, both MDS and BD-like symptoms are in complete remission at 36 months post transplantation, and the patient continues to take low-dose oral tacrolimus for chronic skin GVHD. Allogeneic hematopoietic stem cell transplantation could become a therapeutic choice for MDS associated with BD, even if refractory intestinal BD symptoms are present.

[Clinical characteristics of vascular adverse events and significance of peripheral artery disease as a risk factor in chronic myeloid leukemia patients treated with nilotinib].

Vascular adverse events (VAEs) in chronic myeloid leukemia (CML) patients treated with nilotinib (NIL) has become a; however, studies on strategies to prevent VAEs remain limited. Therefore, the present study investigated VAEs in 19 CML patients treated with NIL at our hospital. The median age of the patients was 65 years and median follow-up period was 55 months after the initiation of NIL. VAEs occurred in 8 patients (peripheral artery disease (PAD), n=6; cerebral infarction (CI), n=3; coronary artery disease (CAD), n=4). The median elapsed time from the initiation of NIL to VAEs was 42 months. The 4-year cumulative incidence of VAEs was 23.5%. Majority of the patients with VAEs were smokers (P=0.074). All the six patients with PAD were diagnosed on the basis of the ankle-brachial index (ABI<0.9) in the asymptomatic phase; 4 of these patients had other VAEs (CI, n=1; CAD, n=2; CI and CAD, n=1). However, antecedent asymptomatic PAD was diagnosed even before CAD was diagnosed in two patients. Nevertheless, in cardiology, extensive studies have indicated that asymptomatic PAD is a risk factor for the development of cardiovascular events. In conclusion, for the effective management of CML patients treated with NIL, a routine screening with ABI to diagnose asymptomatic PAD may be beneficial in preventing severe VAEs.

[Neurolymphomatosis due to enteropathy-associated T-cell lymphoma clinically diagnosed by FDG-PET/CT and subsequently confirmed by autopsy].

A 59-year-old man who complained of abdominal pain was referred to our hospital. Computed tomography (CT) revealed mesenteric lymph node swelling and intestinal perforation. Histopathological study of the resected ileum and lymph node demonstrated diffuse proliferation of medium-sized atypical lymphocytes. Immunohistochemistry results were positive for cluster of differentiation (CD) 3, CD8, and CD56 cells, negative for CD5 and CD4 cells, and negative for Epstein-Barr virus-encoded RNA-fluorescent in situ hybridization (EBER-FISH). It also revealed the expression of γδ T-cell receptors. On the basis of these findings, enteropathy-associated T-cell lymphoma (EATL) was diagnosed. Although the patient received two courses of cyclophosphamide, hydroxydaunorubicin, oncovin, and prednisone (CHOP) and dexamethasone, etoposide, ifosfamide, and carboplatin (DeVIC) therapy, facial nerve and lower limb paralysis manifested. Magnetic resonance imaging (MRI) and lumbar puncture revealed central nervous system invasion of the EATL. Despite intrathecal chemotherapy and high-dose cytarabine therapy, the patient's neurological symptoms deteriorated. Fluorodeoxyglucose positron emission tomography (FDG-PET) /CT scan showed the accumulation of FDG along both median and sciatic nerves, and he was diagnosed with neurolymphomatosis (NL). He died on day 120 after admission. Autopsy specimens exhibited infiltration of lymphoma cells in the median and sciatic nerves. Although only one case of suspected NL in a patient with type 2 EATL has been previously reported, we clinically diagnosed NL using FDG-PET/CT and confirmed the diagnosis by autopsy. This case is valuable in terms of the pathological diagnosis of NL.

Successful management of intraperitoneal bleeding with platelet apheresis and von Willebrand factor supplementation in a patient with essential thrombocythemia and acquired von Willebrand syndrome.

A 36-year-old woman with essential thrombocythemia (ET) was admitted to our hospital for acute lower abdominal pain. Given no family history of bleeding disorder, she was diagnosed with acquired von Willebrand syndrome. Despite having a medical history of venous thrombosis, she had never been treated for ET because of her preferences. On admission, CT scan revealed massive hemorrhage in the ascending colon with the leakage of a contrast agent. Furthermore, a delayed enhancement of fluid collection in the Douglas fossa followingcontrast CT indicated bloody ascites. Laboratory data revealed elevated platelets (1,569×103/µl) and reduced von Willebrand factor (VWF) :RCo (32%) and VWF:Ag (48%). Platelet apheresis was initiated, combined with the infusion of VWF-containing concentrates and cytoreductive therapy with hydroxyurea. Three days after admission, her platelet count decreased to 992×103/µl after the second round of platelet apheresis. CT scan revealed no hemorrhage, which implied hemostasis. Because of the absence of symptoms, she was discharged 23 days after admission. These results suggest that platelet apheresis, combined with infusion of VWF-containing concentrates and cytoreductive therapy with hydroxyurea, is an effective approach for the treatment of acquired von Willebrand syndrome characterized by emergent bleeding concomitant with ET.

Unraveling the linguistic nature of specific autobiographical memories using a computerized classification algorithm.

In the present study, we explored the linguistic nature of specific memories generated with the Autobiographical Memory Test (AMT) by developing a computerized classifier that distinguishes between specific and nonspecific memories. The AMT is regarded as one of the most important assessment tools to study memory dysfunctions (e.g., difficulty recalling the specific details of memories) in psychopathology. In Study 1, we utilized the Japanese corpus data of 12,400 cue-recalled memories tagged with observer-rated specificity. We extracted linguistic features of particular relevance to memory specificity, such as past tense, negation, and adverbial words and phrases pertaining to time and location. On the basis of these features, a support vector machine (SVM) was trained to classify the memories into specific and nonspecific categories, which achieved an area under the curve (AUC) of .92 in a performance test. In Study 2, the trained SVM was tested in terms of its robustness in classifying novel memories (n = 8,478) that were retrieved in response to cue words that were different from those used in Study 1. The SVM showed an AUC of .89 in classifying the new memories. In Study 3, we extended the binary SVM to a five-class classification of the AMT, which achieved 64%-65% classification accuracy, against the chance level (20%) in the performance tests. Our data suggest that memory specificity can be identified with a relatively small number of words, capturing the universal linguistic features of memory specificity across memories in diverse contents.

Early diagnosis and successful treatment of disseminated toxoplasmosis after cord blood transplantation.

A 66-year-old woman with refractory angioimmunoblastic T-cell lymphoma underwent cord blood transplantation. Prior to transplantation, a serological test for Toxoplasma gondii-specific IgG antibodies was positive. On day 96, she exhibited fever and dry cough. Chest CT showed diffuse centrilobular ground glass opacities in both lungs. The reactivation of T. gondii was identified by the presence of parasite DNA in peripheral blood and bronchoalveolar lavage fluid. Moreover, brain MRI revealed a space occupying lesion in the right occipital lobe. Therefore, disseminated toxoplasmosis was diagnosed. She received pyrimethamine and sulfadiazine from day 99. The lung and brain lesions both showed improvement but the PCR assay for T. gondii DNA in peripheral blood was positive on day 133. On day 146, she developed blurred vision and reduced visual acuity, and a tentative diagnosis of toxoplasmic retinochoroiditis was made based on ophthalmic examination results. As agranulocytosis developed on day 158, we decided to discontinue pyrimethamine and sulfadiazine and the treatment was thus switched to atovaquone. Moreover, we added spiramycin to atovaquone therapy from day 174, and her ocular condition gradually improved. In general, the prognosis of disseminated toxoplasmosis after hematopoietic stem cell transplantation (HSCT) is extremely poor. However, early diagnosis and treatment may contribute to improvement of the fundamentally dismal prognosis of disseminated toxoplasmosis after HSCT.

[Discordant lymphoma of duodenal EBV-positive peripheral T-cell lymphoma not otherwise specified and ileal diffuse large B-cell lymphoma].

A 79-year-old woman was admitted with a 5-kg weight loss and anorexia. Computed tomography showed diffuse lymphadenopathy, and thickening of the duodenal and ileal walls. The patient then underwent biopsy of these sites. Pathological examination revealed duodenal Epstein-Barr virus (EBV)-positive peripheral T cell lymphoma-not otherwise specified (PTCL-NOS) and EBV-negative ileal diffuse large B-cell lymphoma (DLBCL) to be present simultaneously. Combination chemotherapy including rituximab produced a reduction of the duodenal EBV-positive PTCL-NOS lesion, but had no effect on the EBV-negative ileal DLBCL lesion. Thereafter, new lymphadenopathy, high fever, and lactate dehydrogenase (LD) elevation developed, complicated by pneumonia. The patient died due to rapid deterioration of the lymphoma and pneumonia on day 108 after initiation of treatment. EBV-positive PTCL-NOS is reportedly rare and the prognosis is poor. Moreover, EBV-negative ileal DLBCL was diagnosed simultaneously. This case is considered to have had an extremely rare discordant lymphoma, although the exact etiology of its development remains unknown. We speculate that age-related disorders of the immune system and HCV infection may have been associated with the pathogenic mechanism of lymphomagenesis in this case.

Spatial heterogeneity in the carrying capacity of sika deer in Japan.

Carrying capacity is 1 driver of wildlife population dynamics. Although in previous studies carrying capacity was considered to be a fixed entity, it may differ among locations due to environmental variation. The factors underlying variability in carrying capacity, however, have rarely been examined. Here, we investigated spatial heterogeneity in the carrying capacity of Japanese sika deer ( Cervus nippon ) from 2005 to 2014 in Yamanashi Prefecture, central Japan (mesh with grid cells of 5.5×4.6 km) by state-space modeling. Both carrying capacity and density dependence differed greatly among cells. Estimated carrying capacities ranged from 1.34 to 98.4 deer/km 2 . According to estimated population dynamics, grid cells with larger proportions of artificial grassland and deciduous forest were subject to lower density dependence and higher carrying capacity. We conclude that population dynamics of ungulates may vary spatially through spatial variation in carrying capacity and that the density level for controlling ungulate abundance should be based on the current density level relative to the carrying capacity for each area.

[Primary graft failure in a patient with refractory acute myeloid leukemia successfully treated with modified 'one-day'-based preparative regimen followed by cord blood transplantation].

A 32-year-old woman with acute myeloid leukemia failed to achieve remission with two courses of induction chemotherapy, and she received cord blood transplantation (CBT) in a non-remission state, using an HLA-matched cord blood (CB) graft after a conditioning regimen of fludarabine (Flu) at 125 mg/m² + melphalan at 140 mg/m² + total body irradiation (TBI) at 4 Gy. Chimerism analysis of the bone marrow (BM) cells performed on day 21 after CBT revealed 99% of these cells to be the recipient type. We diagnosed the patient as having graft failure (GF), and then carried out a second CBT using an HLA-matched male CB graft on day 29 after the first CBT. The conditioning regimen (modified 'one-day'-based regimen) consisted of Flu at 30 mg/m² (3 days) + cyclophosphamide (CY) at 2 g/m² (1 day) + TBI 2 Gy. She achieved neutrophil engraftment on day 18. FISH analysis of BM cells on day 13 showed 96% to be of male origin. She has remained in complete remission for 18 months, to date, since the salvage CBT. This case suggests that salvage CBT following a modified 'one-day'-based regimen may preserve a strong graft versus leukemia effect.

[Chromosomally integrated human herpesvirus-6 requiring differential diagnosis of reactivation after allogeneic hematopoietic stem cell transplantation].

Human herpesvirus-6 (HHV-6) is known to cause critical encephalitis, as a central nervous system infection, in some hematopoietic stem cell transplantation (HSCT) recipients. Chromosomally integrated human herpesvirus-6 (CIHHV-6) persistently shows HHV-6 DNA in blood, but this does not necessarily suggest active infection. The true clinical significance in HSCT is not clear. The prevalence of CIHHV-6 in Japan is reportedly 0.21%. We herein report two HSCTs: from a CIHHV-6-positive donor to a negative recipient and from a negative donor to a positive recipient. In the CIHHV-6-positive donor case, the recipient's plasma, which had been negative for HHV-6 before HSCT, became positive after transplantation and the level then remained high, although the subject was asymptomatic. In the CIHHV-6-positive recipient case, the patient's plasma viral load was high just after transplantation, although the subject was asymptomatic, and the load gradually decreased after engraftment. Antivirals had no effect on the viral load in either case. We should consider CIHHV-6 when the HHV-6 DNA load in blood persists asymptomatically after HSCT, to avoid misdiagnosis of reactivated HHV-6 infection and overuse of antivirals. It is also useful to monitor HHV-6 DNA in blood before HSCT, to distinguish HHV-6 reactivation from CIHHV-6.

Bone marrow metastasis of rhabdomyosarcoma mimicking acute leukemia: a case report and review of the literature.

Bone marrow metastasis of rhabdomyosarcoma has been reported to be difficult to distinguish from acute leukemia. We herein describe a case of rhabdomyosarcoma with bone marrow metastasis mimicking acute lymphoblastic leukemia. A 29-year-old woman was admitted with thrombocytopenia, blast-like cells in the peripheral blood and a coagulation disorder. Bone marrow aspirates showed 94.8% blast-like cell infiltration (CD45(-), myeloperoxidase(-), and CD56(+)), and CT scan revealed the presence of an infiltrating mass in the nasal cavity. Based on a biopsy of the nasal cavity, the patient was diagnosed with rhabdomyosarcoma exhibiting bone marrow metastasis. She received chemotherapy, followed by radiation therapy, and has since remained alive for 26 months, as of the last follow-up.

[Successful long-term control of recurrent primary central nervous system anaplastic large cell lymphoma after autologous hematopoietic stem cell transplantation with concurrent whole brain and spinal cord radiotherapy].

A 24-year-old woman was hospitalized with seizures in 2002. Magnetic resonance imaging demonstrated an intraspinal mass and inhomogeneous gadolinium enhancement along the cerebrospinal meninges. Cerebrospinal fluid (CSF) cytology showed large atypical cells expressing CD2, cytoplasmic CD3, CD7, CD13 and CD30. The patient was finally diagnosed with primary central nervous system anaplastic large cell lymphoma (ALCL). She completed 5 courses of methotrexate (MTX)/ procarbazine (PCZ)/ vincristine (VCR) (MPV) chemotherapy, followed by 2 courses of high dose cytarabine (AraC) and achieved a complete remission. In 2003, she suffered from headache. CSF analysis showed atypical lymphoid cells expressing CD 30. First CNS relapse was diagnosed. She then underwent autologous peripheral blood stem cell transplantation (auto-PBSCT) after administration of thiotepa, buslfan, and cyclophosphamide. However, second CNS relapse occurred in 2004. She received 5 courses of MPV chemotherapy followed by 36 Gy of craniospinal irradiation. Although there was no recurrence of the CNS disease, a third relapse was detected in the right breast in 2009. Pathological and immunohistochemistry analysis revealed ALK-1 positive ALCL. She was treated with 6 courses of cyclophosphamide/adriamycin/vincristine/predonine (CHOP) chemotherapy and 30.6 Gy of local radiation therapy. She has remained in remission for 6 years, to date, since the last therapy and has an excellent quality of life.

[Therapy-related chronic myelogenous leukemia following RFM therapy in a patient with follicular lymphoma].

Therapy-related myelodysplastic syndrome and acute myelogenous leukemia are increasingly being recognized as treatment complications in patients receiving chemotherapy or radiotherapy for previous neoplasms. However, therapy-related chronic myelogenous leukemia is relatively rare. A 61-year-old woman with a history of radiation therapy for breast cancer had previously, in 2007, received 4 courses of chemotherapy (RFM: rituximab, fludarabine, and mitoxantrone) for follicular lymphoma. In 2010, she was diagnosed with chronic-phase chronic myelogenous leukemia (CML) with Philadelphia chromosome but no other cytogenetic anomalies. Although a complete cytogenetic response (CCyR) was achieved with imatinib therapy, she developed leukocytosis with lymphoblasts and lymphoid crisis was diagnosed in January 2013. G-banded karyotyping showed 45, XX, -7, t, (9;22)(q33;q11.2). Unrelated bone marrow stem cell transplantation was performed after she had achieved a CCyR with dasatinib therapy. Polymerase chain reaction detected no major bcr/abl transcript in her bone marrow 42 days after transplantation. The majority of secondary leukemias resulting from the use of cytotoxic drugs can be divided into two well-defined groups depending on whether the patient has received alkylating agents or topoisomerase II inhibitors. However, concerns regarding the leukemogenic potential of fludarabine-based chemotherapy are growing. The potential risk of therapy-related leukemias including CML needs to be considered following fludarabine-based chemotherapy.