BACKGROUND Thrombopoietin (TPO) receptor agonist, Lusutrombopag, is recently released to treat thrombocytopenia in chronic liver disease (CLD). However, its effectiveness remains to be unclear. The purpose of this study was to clarify the efficacy of Lusutrombopag and identify the predictors associated with platelet count increase. METHODS Eighty CLD patients with thrombocytopenia were enrolled. The primary endpoint was the Lusutrombopag effect, which is the proportion of cases satisfying following; the platelet increase was enough (with an increase of greater than 1.0 × 104/μL from at baseline) and platelet transfusion was not required. The secondary endpoints were the response rate (which showed an increase of greater than 1.0 × 104/μL from at baseline), independent predictors of increased platelets, and the superiority of Lusutrombopag over platelet transfusion. RESULTS The primary endpoint was 93.8% (75 of 80 patients). The response rate was 96.2% (77 of 80). Factors indicative of renal function (BUN, creatinine, eGFR) significantly and negatively correlated with increase in platelets (p = 0.033, 0.049, 0.0014, respectively), and were also identified as independent predictors by multiple regression analysis (p = 0.049, 0.0023, 0.0016, respectively). The median increase in platelet count after Lusutrombopag was significantly higher than that after platelet transfusion (41000 vs. 12000 /μL, p = 0.015). CONCLUSION This study revealed that Lusutrombopag was more sufficiently effective in CLD patients compared with platelet transfusion and that renal function is independent predictor of increase. Factors indicative of renal function significantly and negatively correlated with increase in platelets.BACKGROUND Angioleiomyomas are usually small and relatively rare painful soft tissue tumors, and are usually less than 2 cm in diameter. Magnetic resonance (MR) imaging features of typical angioleiomyomas are not well understood, and their association with histologic subtypes is not clear. In the present study, MR images of average sized angioleiomyomas were compared with regard to specific histologic subtypes. METHODS We performed a retrospective review of medical records, MR imaging data, and histologic specimens of 18 consecutive patients with angioleiomyomas that were extirpated at our hospital between January 2006 and December 2013. RESULTS Most of the lesions showed homogenous iso-signal intensities compared to skeletal muscle on T1-weighted images; however, on T2-weighted images, solid and venous types showed heterogenous and iso- to slightly high-signal intensity, while the cavernous type showed high-signal intensity. Most of the lesions had a low-signal intensity rim, and two thirds of them had adjacent vessels. CONCLUSIONS Our results suggested that MR findings of angioleiomyoma vary depending on histologic subtypes. T2-weighted images of solid and venous types of angioleiomyoma show specific MR findings which allow for differentiation from other soft tissue tumors such as soft tissue sarcomas. These mostly show iso- to slightly high-signal intensity compared to skeletal muscle, while the cavernous type show non-specific findings. Thus, solid and venous types of angioleiomyoma could be diagnosed preoperatively via clinical findings and MR imaging.BACKGROUND 5-Aminolevulinic Acid (5-ALA) Photodiagnosis (PD) is an effective method to detect residual tumors during glioma surgery. However, the strength of fluorescence differs in every case even in the most malignant glioma such as glioblastoma. Also, false-negative fluorescence may result in tumor residue. We investigated the effect of ultrasound on the intracellular level of protoporphyrin IX (PpIX) and the expression level of ATP-binding cassette transporter 2 (ABCG2), which is thought to act as a membrane efflux pump of PpIX from cytosol. METHODS The malignant glioma established cell line, SNB19, U87MG and T98G were used for in vitro experiments. Ultrasound (1MHz, 3W/cm2, Duty cycle 10%) was irradiated on the cultured cells after administration of 5-ALA. The morphological changes of tumor cells were observed. The PpIX levels and the expression of ABCG2 were evaluated. RESULTS The glioma tumor cells showed transient morphological changes and attachment to the culture dish, while most cells survived and were restored to their original morphology within 6 hours. The PpIX expression levels increased in glioma cells after ultrasound irradiation, earlier and higher than 5-ALA alone. Concurrently the expressions of ABCG2, that increased after 5-ALA administration, were relatively reduced in ultrasound irradiated glioma cells. CONCLUSIONS The ultrasound irradiation of the malignant gliomas contributes to the stronger 5-ALA induced fluorescence by elevating intracellular PpIX levels. The suppression of ABCG2 expression by ultrasound may be one of the factors which contributes to the PpIX accumulation in glioma cells.OBJECT Petrous internal carotid aneurysm (PA) concomitant with a mass lesion and cranial nerve palsy is relatively rare. In recent years, flow-diverter stent implantation is being widely used as an alternative treatment for PA. However, alternative treatments are not available for all patients for various reasons, including the tortuosity of the parent carotid artery, allergies to contrast material, or high cost. It is, therefore, important to compare the relative outcomes of different treatment methods. Here, we have accordingly reviewed the available literature on the treatments for PA. METHODS We searched PubMed, MEDLINE, and other databases, such as OvidSP using the terms „aneurysm „, „carotid artery”, and „petrous ” and identified 221 articles. We also performed a literature review and discussed and compared the etiologies, symptoms, treatment methods, and clinical outcomes in patients with PA. RESULTS AND CONCLUSIONS The systematic review revealed that the onset of secondary aneurysms was generally manifested by bleeding (p less then 0.001), while the onset of primary aneurysms was manifested by cranial nerve deficits (p= 0.0014); the prognosis after treatment of 34 cranial nerve palsies in 25 cases was reported.IntroductionTo increase survival rates among patients with severe trauma from road traffic accidents, Japan launched the D-Call Net (DCN) system for dispatching doctors by helicopter utilizing Advanced Automatic Collision Notification technology in November 2015. As of October 2019, DCN has dispatched doctors 4 times.CasesCase 1 Canceled because trauma was mild. Case 2 Doctor made contact with 2 patients with moderate trauma 29 min earlier than would have occurred conventionally. This was the first case of doctor dispatch and patient treatment based on automotive engineering information worldwide. Case 3 An accident involving 3 severely injured patients activated DCN, enabling doctor-patient contact 20 min earlier than would have been possible conventionally. Case 4 DCN was utilized ineffectively.DiscussionAccording to 2008 data from Chiba Prefecture, in accidents where victims sustained severe trauma, the time from accidence occurrence to hospital arrival was 67 min, even with doctor dispatch by air ambulance („Doctor-Heli” [DH]). Accident information for faster doctor dispatch effectively improved survival rates. An algorithm was developed to assess trauma severity (severity probability) based on accident information. DCN dispatches doctors based on information, including accident site and severity probability, that is sent to smartphones of doctors, reducing the time from accident to DH request by approximately 17 min.DCN is the world’s first system for faster doctor dispatch to traffic accident sites based on automotive engineering information. It is crucial for improving survival rates and mitigating aftereffects.Hemophagocytic lymphohistiocytosis (HLH) associated with Epstein-Barr virus (EBV) infection ranges from self-limiting to severe/aggressive or fatal. We report the case of a 4-year-old boy with EBV-HLH with persistent fever, severe pancytopenia, hypertriglyceridemia, and hypofibrinogenemia. The levels of plasma cytokines and chemokines were measured using a Bio-Plex system on Days 1, 2, 3, 4, 5, and 8 following hospital admission. The administration of steroid and high-dose intravenous immunoglobulin (1 g/kg) did not alleviate the fever or reduce cytokine production; however, following etoposide (a known antineoplastic agent) administration, the fever decreased immediately, and the patient’ s general condition improved. The present study revealed that IL-6, IL-10, IL-8, MCP-1, IFN-γ, and TNF-α were suppressed by etoposide administration. In particular, production of IFN-γ sharply declined from 1,104.1 pg/mL to 101.5 pg/mL, and the IL-6 level also decreased from 229.8 pg/mL to 11.0 pg/mL the day after the initial etoposide administration. Subsequently, there was no recurrence of symptoms with dexamethasone, etoposide, and cyclosporine A treatment. The present case demonstrates that early etoposide administration is critical for successful treatment and indicates that etoposide exhibits a prompt inhibitory effect on cytokine production.A 65-year-old woman with spinocerebellar ataxia presented with generalized seizures due to subcortical hemorrhaging. Magnetic resonance imaging (MRI) revealed obstruction of the superior sagittal sinus. Despite treatment, she became comatose. MRI newly revealed subdural fluid collection and descent of the brainstem. Her history indicated a recent fall, prompting additional studies, which revealed lumbar fracture and cerebrospinal fluid (CSF) leaks. We performed an epidural blood patch, and her consciousness was fully restored in one month. This is the first report of cerebral venous thrombosis with CSF leaks in the lumbar region due to a fall injury.Small-bowel hemangiomas are a possible source of gastrointestinal bleeding for which there is no established treatment approach. In this report, we describe the case of a 58-year-old woman who presented with hematochezia and who was diagnosed with small bowel hemangioma. She was successfully treated using endoscopic sclerotherapy. Initial capsule endoscopy revealed bleeding in the ileum. Subsequent double-balloon enteroscopy showed a 2-cm, bluish-purple, ileal submucosal tumor with an overlying protrusion. The lesion was responsible for the hematochezia and was treated with intralesional injection of polidocanol. The hematochezia completely resolved and at 4 months after sclerotherapy, the size of the lesion was significantly reduced.Mutations in the TTC21B gene have been identified in patients with nephronophthisis and were recently found in some patients with focal segmental glomerulosclerosis. We herein report a Japanese boy with end-stage renal disease due to medullary polycystic kidney disease and primary focal segmental glomerulosclerosis. Next-generation sequencing detected a new compound heterozygous missense mutation in the TTC21B gene. His renal pathological findings and gene mutations have not been previously reported in patients with ciliopathy. For children with severe renal dysfunction, mutations in the TTC21B gene cause both ciliopathy characterized by bilateral polycystic kidney disease and primary focal segmental glomerulosclerosis.