Impact of Frailty and Sarcopenia on Short- and Long-Term Outcomes in Elderly Patients Undergoing Radical Gastrectomy for Gastric Cancer.

BACKGROUND: The impact of frailty and sarcopenia in patients with gastric cancer is unclear. This study aimed to comprehensively examine the impact of frailty and sarcopenia on the short- and long-term outcomes in elderly patients undergoing radical gastrectomy for gastric cancer. METHODS: We retrospectively assessed 246 patients aged ≥ 65 years who underwent radical gastrectomy. Frailty and sarcopenia were assessed using the modified frailty index (mFI) and psoas muscle mass index (PMI), respectively. RESULTS: There were 30 (12.2%) and 60 (24.4%) patients with High-mFI and Low-PMI, respectively. As the age increased, both sexes showed significant correlations with PMI and mFI (r = - 0.238, 0.322, P = 0.003 and 0.002, respectively). High-mFI and Low-PMI did not affect the short-term outcomes. However, High-mFI was an independent risk factor for non-home discharge (P = 0.004) and was a significant predictor of 3- and 5-year overall survival (OS) (HR = 2.76 and 2.26; P = 0.002 and 0.005, respectively) and 1-, 3- and 5-year non-cancer-specific survival (non-CSS) (HR = 4.88, 8.05, and 4.01; P = 0.017, < 0.001, < 0.001, respectively). Low-PMI was a significant predictor of only 5-year OS (HR = 2.03, P = 0.003) and non-CSS (HR = 2.10, P = 0.020). CONCLUSIONS: Frailty is significant predictor of non-home discharge and 1-, 3-, 5-year OS and 3- and 5-year non-CSS. Sarcopenia is a significant predictor of 5-year OS and non-CSS. Preoperative assessment of both frailty and sarcopenia can help surgeons to select adequate treatment strategies for the elderly population.

Post-colostomy internal hernia of the stomach treated with laparoscopic gastropexy: a case report.

BACKGROUND: Internal hernias are formed by the protrusion of internal organs through an aperture formed congenitally or postoperatively. Internal hernias are most commonly associated with the small intestine. Only two cases of a post-sigmoid colostomy internal hernia of the stomach have been reported. This hernia arises from the space between the lifted sigmoid colon and the left abdominal wall. In the two aforementioned cases, treatment comprised suturing of the sigmoid colon to the lateral abdominal wall and changing of the intraperitoneal route to an extraperitoneal one. Herein, we present a very rare case who underwent laparoscopic gastropexy for a post-sigmoid colostomy internal hernia of the stomach. CASE PRESENTATION: Our patient, a 67-year-old woman, was undergoing chemoradiation for rectal cancer and planned to undergo abdominoperineal resection. However, tumor perforation resulted in a high fever and a right gluteal abscess; therefore, a sigmoid colostomy was performed through the intraperitoneal route in the left lower abdomen. One month after the surgery, the patient presented to our emergency room with vomiting, abdominal pain, and abdominal distension. Computed tomography revealed a markedly distended stomach caused by the obstruction of the pylorus secondary to the colostomy; laparoscopic gastropexy was performed subsequently and the postoperative course was uneventful. CONCLUSIONS: This is the first report on the laparoscopic gastropexy treatment of a post-sigmoid colostomy internal hernia of the stomach; our findings may help physicians manage such hernias.

What is necessary to shorten the operative time in initial introduction of robotic gastrectomy for gastric cancer?

INTRODUCTION: Robotic gastrectomy (RG) is a good alternative to laparoscopic gastrectomy, as it improves treatment outcomes and reduces the burden of technical difficulties; however, prolonged operative time may be a disadvantage. This study aimed to identify measures to shorten the operative time during the initial introduction of RG at an institution. METHODS: We assessed 33 patients with gastric cancer who underwent radical distal gastrectomy with Billroth-I reconstruction and divided them into three groups: laparoscopic distal gastrectomy (LDG), robotic distal gastrectomy in the early phase (RDG-E), and in the late phase (RDG-L). Operative time, six technical steps, and junk time, including the roll-in/roll-out, docking/undocking, and instrument exchange times, were compared among the groups. RESULTS: The median (range) overall operative times of LDG, RDG-E, and RDG-L were 248 (179-323), 304 (249-383), and 263 (220-367) min, respectively, but no significant differences were observed. For each surgical step of RG, RDG-L in suprapancreatic lymph node dissection was significantly shorter than that in RDG-E. The median (range) junk times of LDG, RDG-E, and RDG-L were 16.7 (12.7-26.4), 48.3 (38.6-67.7), and 42.0 (35.4-49.2) min, respectively. Junk time was significantly longer in RDG-L than in LDG (p = 0.003), but not significant between RDG-E and RDG-L. The learning curve effect of overall, console, and junk times were achieved in four cases of RDG. CONCLUSION: Junk time is a major factor in prolonging RDG operative time. However, to reduce the time after initial introduction, measures to promote robot-specific standardization and more effective use of robotic instruments are essential.

Case Report: A Rare Case of Elderly-Onset Adult-Onset Still's Disease in a Patient With Systemic Lupus Erythematosus.

The rare systemic inflammatory disorder 'adult-onset Still's disease (AOSD)' is characterized by recurrent fever, evanescent rash, arthralgia, and leukocytosis with neutrophilia. The Yamaguchi criteria are widely used to diagnose AOSD; these criteria can be used for diagnosis after a wide range of infectious, rheumatic, and neoplastic diseases have been excluded. AOSD generally does not overlap with other rheumatic diseases. We present the rare case of an 80-year-old Japanese woman who presented with arthralgia, fever, and skin rash during treatment for systemic lupus erythematosus (SLE), which was finally diagnosed as an overlap of AOSD. Blood tests revealed leukocytosis with neutrophilia, high C-reactive protein (CRP), and liver dysfunction. Her anti-ds-DNA antibody titer and serum complement titer were at the same level as before and remained stable. We suspected AOSD based on the high serum ferritin level but hesitated to diagnose AOSD because of the patient's SLE history. We measured serum interleukin (IL)-18; it was extremely high at 161,221 pg/mL, which was strongly suggestive of AOSD. We thus diagnosed AOSD complicated during the course of treatment for SLE. The patient's arthralgia and high CRP level persisted after we increased her oral prednisolone dose and added oral methotrexate, but her symptoms eventually improved with the addition of intravenous tocilizumab. We note that the presence of autoantibodies or other rheumatic diseases cannot be absolutely ruled out in the diagnosis of AOSD. Although high serum IL-18 levels are not specific for AOSD, the measurement of serum IL-18 may aid in the diagnosis of AOSD in similar rare cases.

Four-Year Teriparatide Followed by Denosumab vs. Continuous Denosumab in Glucocorticoid-Induced Osteoporosis Patients With Prior Bisphosphonate Treatment.

Objectives: In our previous 24-month study, we observed that teriparatide had some advantages over denosumab for bone mineral density (BMD) in glucocorticoid-induced osteoporosis (GIO) patients with prior bisphosphonate treatment. We conducted this extension study to investigate whether the advantage of teriparatide obtained in the first 2 years would be maintained after the switch to denosumab. Materials and Methods: We switched patients who had completed 24-month daily teriparatide treatment to denosumab (switch group, n=18) and compared their BMD every 6 months up to 48 months with the group who continued to receive denosumab (denosumab group, n=16). Results: At 48 months, the lumbar spine BMD was significantly increased from baseline in both groups (denosumab: 10.4 ± 8.7%, p<0.001; switch: 14.2 ± 6.8%, p<0.001). However, a significant increase in femoral neck BMD from baseline occurred only in the switch group (11.2 ± 14.6%, p<0.05); denosumab (4.1 ± 10.8%). The total hip BMD increased significantly from baseline in both groups (denosumab: 4.60 ± 7.4%, p<0.05; switch: 7.2 ± 6.9%, p<0.01). Femoral neck BMD was significantly increased in the switch versus the denosumab group (p<0.05). Conclusion: In GIO patients with prior bisphosphonate treatment, the advantage of teriparatide may be maintained after the treatment period. A continuous increase in BMD can be expected with teriparatide followed by denosumab.

Usefulness of Interleukin-18 as a Diagnostic Biomarker to Differentiate Adult-Onset Still's Disease With/Without Macrophage Activation Syndrome From Other Secondary Hemophagocytic Lymphohistiocytosis in Adults.

Background: Interleukin (IL)-18 is markedly elevated in systemic inflammatory diseases that cause the 'cytokine storm' such as adult-onset Still's disease (AOSD) and hemophagocytic lymphohistiocytosis (HLH). The differences in IL-18 between AOSD and HLH, especially in adults, is uncertain. Macrophage activation syndrome (MAS), a form of secondary HLH, is often difficult to differentiate cases of AOSD that include MAS from other secondary HLH. In this case-control study, we investigated whether serum IL-18 levels could be a useful biomarker for the differential diagnosis of AOSD with or without MAS (AOSD group) and other secondary HLH in adults (adult HLH group). Patients and Methods: We enrolled 46 patients diagnosed with AOSD including 9 patients with MAS and 31 patients in the adult HLH group, which excluded AOSD-associated MAS. The clinical features and laboratory data were compared between the AOSD and adult HLH groups. In addition, we subdivided the AOSD group (with or without MAS) and the adult HLH group (whether lymphoma-associated or not) and compared the four groups. A logistic regression analysis was used to identify factors with high efficacy in differentiating the two groups, followed by a receiver operating characteristic (ROC) curve analysis to evaluate the differential diagnostic ability of IL-18. We analyzed the correlation between IL-18 and various laboratory parameters in the AOSD group. Results: Serum IL-18 levels of patients in the AOSD groups were significantly higher than those of the adult HLH groups, and were closely correlated with ferritin, soluble interleukin-2 receptor (sIL-2R), and other laboratory data. Univariate and multivariate logistic regression analyses revealed that IL-18, sIL-2R, and 'arthralgia or arthritis' are independent factors useful in the differential diagnosis of AOSD from adult HLH. In the differential diagnosis of both groups, the area under the curve obtained from the ROC curve of IL-18 with a cutoff value of 18,550 pg/mL was 0.91 (95% confidence interval 0.83-1.00; sensitivity 90.3%, specificity 93.5%), and the differential diagnosis ability of IL-18 was superior to that of other laboratory data. Conclusions: IL-18 could be a useful biomarker for the differential diagnosis of AOSD and adult HLH.

FDG-PET/CT and Auricular Cartilage Biopsy Are Useful for Diagnosing with Relapsing Polychondritis in Patients without Auricular Symptoms.

Relapsing polychondritis (RP) is a rare autoimmune inflammatory disease characterized by recurrent inflammation and destruction of cartilage. Although auricular chondritis is a characteristic finding in RP, it can be difficult to diagnose in the absence of auricular symptoms. A 64-year-old Japanese male was referred to our hospital with fever and respiratory distress. Contrast-enhanced computed tomography (CT) revealed bronchial wall thickening and we suspected RP; however, he had no auricular symptoms and did not meet the diagnostic McAdam criteria for RP, so we used 18F-fluorodeoxyglucose positron emission tomography/CT (FDG-PET/CT) to search for other cartilage lesions. This analysis revealed FDG accumulation not only in the bronchial walls, but also in the left auricle. Instead of a bronchial biopsy using a bronchoscope, we performed a biopsy of the left auricular cartilage, which is considered a relatively less invasive site. Even though the auricle was asymptomatic, the pathology results revealed chondritis. He was diagnosed with RP, and his symptoms rapidly improved with corticosteroid therapy. A biopsy of asymptomatic auricular cartilage may be useful in the diagnosis of RP. FDG-PET/CT is a powerful tool for the early diagnosis of RP, identifying inflammatory areas even in the absence of symptoms, and guiding the selection of appropriate biopsy sites.

Propylthiouracil-Induced Antineutrophil Cytoplasmic Antibody-Associated Vasculitis after COVID-19 Vaccination.

We report the case of a patient who developed antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) after receiving the coronavirus disease 2019 (COVID-19) vaccine BNT162b (Pfizer-BioNTech). A 37-year-old Japanese woman had been taking propylthiouracil for Graves' disease. She had erythema on her forearm on the 12th day after receiving the first dose of the vaccine, fever on the 13th day, and redness and swelling of her left auricle on the 25th day. Her serum myeloperoxidase-ANCA and proteinase 3-ANCA levels, which were negative before the Graves' disease treatment, were elevated. She had unilateral auricular symptoms but no other typical relapsing polychondritis findings. She was diagnosed with propylthiouracil-induced AAV. She was treated with oral glucocorticoids, and her symptoms improved. Propylthiouracil is considered to be the main cause of the onset of AAV in this case, but it cannot be ruled out that BNT162b may have had some effect on the onset of the disease. Although the development of propylthiouracil-induced AAV in this case may have been incidental and unrelated to the vaccination, this report provides important data for evaluating the safety of the vaccine.

The Combination of Preoperative Skeletal Muscle Quantity and Quality is an Important Indicator of Survival in Elderly Patients Undergoing Curative Gastrectomy for Gastric Cancer.

BACKGROUND: The diagnosis of sarcopenia emphasizes both, the quantity and quality of skeletal muscle. However, the impact of the combination of muscle quantity and quality on long-term survival remains unclear. This study aimed to assess the impact of the combination of skeletal muscle quantity and quality on long-term outcomes in patients with gastric cancer who underwent curative resection. METHODS: We retrospectively assessed 242 patients aged ≥ 65 ears who underwent curative gastrectomy between 2006 and 2015. The psoas muscle mass index (PMI) and intramuscular adipose tissue content (IMAC) were measured on preoperative computed tomography as skeletal muscle quantity and quality, respectively. The sarcopenia stage was classified by the combination of preoperative skeletal muscle quantity and quality (non-sarcopenia, sarcopenia, and severe sarcopenia). Prognostic factors for the 5-year overall survival (OS), non-cancer-specific survival (non-CSS), and cancer-specific survival (CSS) were evaluated by multivariable Cox regression. RESULTS: The median follow-up period was 63.2 months. The non-sarcopenia, sarcopenia, and severe sarcopenia groups comprised 88, 121, and 33 patients (36.4%, 50.0%, and 13.6%), respectively. The severe sarcopenia group was older, and had a greater depth of invasion, than the non-sarcopenia group. Multivariable analysis revealed severe sarcopenia as an independent predictive indicator of OS (hazard ratio [HR] 4.01; 95% confidence interval [CI] 1.75 to 9.22) and non-CSS (HR 3.27; 95% CI 1.61 to 6.67), but not CSS. CONCLUSIONS: The combination of preoperative skeletal muscle quantity and quality was useful for predicting survival, especially death from other diseases, in elderly patients who underwent gastrectomy for gastric cancer.

Interleukin-18 in Inflammatory Kidney Disease.

Interleukin (IL)-18, a member of the IL-1 superfamily, is a pro-inflammatory cytokine that is structurally similar to IL-1ß. IL-18 promotes the production of interferon gamma (IFN-γ) and strongly induces a Th1 response. IL-18 drives the same myeloid differentiation factor 88 (MyD88)/nuclear factor kappa B (NF-κB) signaling pathway as IL-1ß. In physiological conditions, IL-18 is regulated by the endogenous inhibitor IL-18 binding protein (IL-18BP), and the activity of IL-18 is balanced. It is reported that in several inflammatory diseases, the IL-18 activity is unbalanced, and IL-18 neutralization by IL-18BP is insufficient. IL-18 acts synergistically with IL-12 to induce the production of IFN-γ as a Th1 cytokine, and IL-18 acts alone to induce the production of Th2 cytokines such as IL-4 and IL-13. In addition, IL-18 alone enhances natural killer (NK) cell activity and FAS ligand expression. The biological and pathological roles of IL-18 have been studied in many diseases. Here we review the knowledge regarding IL-18 signaling and the role of IL-18 in inflammatory kidney diseases. Findings on renal injury in coronavirus disease 2019 (COVID-19) and its association with IL-18 will also be presented.

Foxp3-Positive Regulatory T Cells Contribute to Antifibrotic Effects in Renal Fibrosis via an Interleukin-18 Receptor Signaling Pathway.

Renal interstitial fibrosis is a common lesion in the process of various progressive renal diseases. Interleukin (IL)-18 is a proinflammatory cytokine that plays an important role in the induction of Th1 responses and is associated with renal interstitial fibrosis, but the mechanism of fibrosis remains unclear. Here we used IL-18 receptor alpha knockout (IL-18Rα KO) mice to investigate the role of an IL-18Rα signaling pathway in renal fibrosis in a murine model of unilateral ureteral obstruction. IL-18 Rα KO mice showed decreased renal interstitial fibrosis and increased infiltration of CD4+ T cells and Foxp3+ regulatory T cells (Tregs) compared to wildtype (WT) mice. The expression of renal transforming growth factor beta 1 (TGF-ß1, which is considered an important cytokine in renal interstitial fibrosis) was not significantly different between WT and IL-18Rα KO mice. The adoptive transfer of CD4+ T cells from the splenocytes of IL-18Rα KO mice to WT mice reduced renal interstitial fibrosis and increased the number of Foxp3+ Tregs in WT mice. These results demonstrated that Foxp3+ Tregs have a protective effect in renal interstitial fibrosis via an IL-18R signaling pathway.

Encapsulated fat necrosis mimicking abdominal liposarcoma: A case report and literature review.

We report a case of an encapsulated fat necrosis without significant medical history. To differentiate from liposarcoma, it should be recognized that a half of abdominal encapsulated fat necrosis cases have a history of inflammation and surgery.

The Effectiveness and Retention Rate of Iguratimod in Japanese Rheumatoid Arthritis Patients with/without Methotrexate in Daily Medical Care.

(1) Background: We evaluated the clinical response of iguratimod (IGU) in patients with rheumatoid arthritis (RA) being treated with or without methotrexate (MTX) over 54 weeks. (2) Methods: 106 patients with RA undergoing IGU were retrospectively observed. RA patients were divided into those treated with MTX+IGU (n = 35) and those treated with IGU (n = 71). The primary endpoint was the clinical response of the Disease Activity Score assessing 28 joints with C-reactive protein (DAS28-CRP) differences in the changes from baseline to 54 weeks between MTX+IGU and IGU groups. Secondary endpoints, such as the clinical response, retention rate, and safety, were evaluated. (3) Results: The DAS28-CRP difference in the changes between the two groups were -0.2. DAS28-CRP were significantly reduced from the baseline in the MTX+IGU and IGU groups (-1.43 and -1.20 from baseline, respectively). The retention rates were 71.4% in the MTX+IGU groups and 59.2% in the IGU groups (p = 0.16). Adverse events were observed in a total of 6 (17.1%) MTX+IGU patients and 20 (28.2%) IGU patients (p = 0.21). (4) Conclusions: IGU therapy may be a useful treatment option for patients who cannot be treated with MTX.

Olfactory function deteriorates in patients with Parkinson's disease complicated with REM sleep behavior disorder.

INTRODUCTION: It is not concluded whether the association between olfactory dysfunction and REM sleep behavior disorder (RBD) were worsen cognitive function in patients with Parkinson's disease (PD). We sought to evaluate the impact of these symptoms in PD. METHODS: We examined 62 patients with PD using an olfactory test (Odor Stick Identification Test for Japanese: OSIT-J) and polysomnography (PSG). We divided the patients into 3 groups: PD with clinical RBD (n = 32), PD with subclinical RBD (n = 11), and PD with normal REM sleep (n = 19). We compared their clinical backgrounds, results of OSIT-J, autonomic functions, and cognitive functions such as Montreal cognitive assessment Japanese version (MoCA-J). Some factors associated with RBD were analyzed by multiple regression. RESULTS: There were significant differences in the results of OSIT-J, and autonomic and cognitive functions between the 3 groups. There were significant differences in the total OSIT-J score between the 3 groups (PD with clinical RBD: 3.3 ± 2.2, PD with subclinical RBD: 4.0 ± 2.6, PD with normal REM sleep: 6.7 ± 3.0, p < 0.001). Patients in the group with PD with clinical RBD had a significantly lower score than those with normal REM sleep (p < 0.001). Logistic regression analysis showed that OSIT-J score was significantly associated with RBD. The PD group with clinical RBD had more patients with mild cognitive impairment than the group with normal REM sleep. Multiple regression analysis revealed that olfactory dysfunction was correlated with MoCA-J. CONCLUSIONS: Olfactory dysfunction is associated with RBD. Especially, it is important to screen olfactory function in RBD complicated patients with PD in view of cognitive impairment.

Effects of denosumab versus teriparatide in glucocorticoid-induced osteoporosis patients with prior bisphosphonate treatment.

INTRODUCTION: Osteoporosis is one of the serious adverse effects associated with glucocorticoid therapy. Although bisphosphonates have been used for glucocorticoid-induced osteoporosis (GIO), some patients have shown an inadequate response. In such cases, denosumab or teriparatide are used. However, there is no consensus on which of these two drugs is superior. We prospectively compared denosumab's and teriparatide's effects on the bone mineral density (BMD) in GIO patients with prior bisphosphonate treatment. MATERIALS AND METHODS: After receiving oral bisphosphonates for ≥2 years, GIO patients with low T-score BMD (<-2.5) were switched from bisphosphonates to denosumab (n = 20) or daily teriparatide (n = 21). We measured the BMD (lumbar spine, femoral neck, and total hip) in both groups every 6 months for 24 months. RESULTS: At 24 months of treatment, the lumbar spine BMD increased significantly from baseline in both the denosumab and teriparatide groups (baseline vs. denosumab and teriparatide; 5.9 ± 5.6%, P < 0.001 and 7.9 ± 5.4%, P < 0.001). A significant increase in femoral neck BMD from baseline occurred only in the teriparatide group (6.6 ± 10.8%, P < 0.05); denosumab (1.5 ± 5.0%). No significant changes occurred in the total hip BMD from baseline in either group (-0.1 ± 5.6% and 3.3 ± 7.5%, respectively). There was no significant difference between the denosumab and teriparatide groups at 24 months in lumbar spine and femoral neck BMD, but was significantly higher in the teriparatide group at 12 months (P < 0.01 and P < 0.05 in the lumbar spine and femoral neck, respectively). CONCLUSION: Teriparatide might have some advantages over denosumab and be a good alternative for treating GIO patients with prior bisphosphonate treatment.

Impact of Screening for Salivary Gland by Ultrasonography.

BACKGROUND: Ultrasonography is superior to other imaging modalities for detecting salivary gland diseases. However, there have been no reports of the results of salivary gland screening with ultrasonography. In this study, the salivary glands were also observed during thyroid ultrasonography to determine the degree of salivary gland abnormalities detected by ultrasonography. METHODS: This study was conducted retrospectively using medical records. It assessed the association between the following abnormal findings detected during thyroid ultrasonography and their final diagnoses: atrophy/swelling, unclear demarcation from surrounding tissues, decreased salivary gland parenchyma echo level, heterogeneity of parenchyma, hypervascularity of salivary gland parenchyma, dilatation of the ducts, and a mass within the gland. RESULTS: Of the 908 patients who underwent thyroid ultrasonography, salivary gland abnormalities were detected in 36 (4.0%) patients. Of the 36 patients with abnormal ultrasonographic findings, 22 underwent further examination. Of the 22 patients, 16 received definitive diagnoses of salivary gland diseases.Salivary gland disorders were considered to be absent in patients with only heterogeneity of the salivary glands observed on ultrasonography. Salivary gland disorders in all patients with further abnormal ultrasonographic findings such as atrophy/swelling, unclear boundary, or hypervascularity in addition to internal heterogeneity were confirmed by further blood examinations and imaging studies. We were able to detect autoimmune sialadenitis such as Sjögren's syndrome and IgG4-related sialadenitis by ultrasonography in patients without obvious symptoms. CONCLUSION: Salivary gland screening during thyroid ultrasonography revealed abnormal findings including Sjögren's syndrome and IgG4-related sialadenitis in about 4% of the patients. Thus, ultrasonography may also be useful for early detection of autoimmune diseases of salivary glands.

Investigation of the Utility and Safety of Dynamic Computed Tomography with Vasodilators.

BACKGROUND: Dynamic computed tomography (CT) angiography is useful for evaluating of hepatic vascularity. Although vasodilators increase hepatic blood flow, the utility of dynamic CT with vasodilators is unclear. Here we investigated the utility and safety of dynamic CT with vasodilators. METHODS: A prospective case-control radiographic evaluation using abdominal dynamic CT with and without vasodilator was performed at a single center between October 2015 and September 2016. We compared the CT values in Hounsfield units of the aorta; celiac artery; and common, right, and left hepatic arteries in the arterial phase and the main trunk; right and left branches of the portal vein; and right, middle, and left hepatic veins in the portal phase with and without vasodilators. The region of interest was set in each element of the liver vasculature. Four radiological technologists independently and visually compared the scores of the portal vein (P-score) and hepatic vein (V-score) on a 5-point scale with and without vasodilators. RESULTS: The CT values of arteries and veins using vasodilators were significantly higher than those without vasodilators. With and without vasodilators, the P-scores were 3.1 ± 1.2 and 4.0 ± 1.1 (P < 0.05) and the V-scores were 3.3 ± 1.4 and 4.3 ± 1.0 (P < 0.05). Only one patient with vasodilator use had transient hypotension and recovered immediately without medication. CONCLUSION: Dynamic CT with vasodilators can provides better visualization of vascular structures.

High-frequency component in flash visual evoked potentials in type 3 Gaucher disease.

OBJECTIVE: To characterize the visual evoked potentials (VEP) in patients with type 3 Gaucher disease (GD) with or without progressive myoclonus epilepsy. METHODS: Three young adults with progressive myoclonus epilepsy (type 3a GD) and two children without progressive myoclonus epilepsy (type 3b GD) were enrolled. Flash visual and somatosensory evoked potentials (F-VEP and SEP, respectively) were retrospectively reviewed in all patients under enzyme replacement therapy. Pattern reversal visual evoked potentials (PR-VEP) were recorded in the type 3a group. RESULT: High-frequency components were provoked at early latencies on averaged F-VEP in all patients with type 3a and one patient with type 3b GD. Conversely, no activities were recorded in PR-VEP. The onset latency of the components began at 15.3-19.8 ms after flash stimulation. Four-five of the F-VEP examination the activities' duration overrode the N75 waveforms and were prolonged toward the P100 peak latency. The F-VEP amplitude did not differ between the type 3a and type 3b groups, while the SEP amplitude was higher in the type 3a than in the type 3b group. SIGNIFICANCE: High-frequency components on F-VEP using bandpass filter at 10-200 Hz may help assess augmented excitability in the visual cortex of type 3 GD patients.

Possible Relationship Between MYBL1 Alterations and Specific Primary Sites in Adenoid Cystic Carcinoma: A Clinicopathological and Molecular Study of 36 Cases.

BACKGROUND: Adenoid cystic carcinoma (ACC) is a relatively rare malignant neoplasm that occurs in salivary glands and various other organs. Recent studies have revealed that a significant proportion of ACCs harbor gene alterations involving MYB or MYBL1 (mostly fusions with NFIB) in a mutually-exclusive manner. However, its clinical significance remains to be well-established. METHODS: We investigated clinicopathological and molecular features of 36 ACCs with special emphasis on the significance of MYBL1 alterations. Reverse-transcription polymerase-chain reaction (RT-PCR) and fluorescence in-situ hybridization (FISH) were performed to detect MYB/MYBL1-NFIB fusions and MYBL1 alterations, respectively. Immunohistochemistry was performed to evaluate MYB expression in the tumors. The results were correlated with clinicopathological profiles of the patients. RESULTS: RT-PCR revealed MYB-NFIB and MYBL1-NFIB fusions in 10 (27.8%) and 7 (19.4%) ACCs, respectively, in a mutually-exclusive manner. FISH for MYBL1 rearrangements was successfully performed in 11 cases, and the results were concordant with those of RT-PCR. Immunohistochemically, strong MYB expression was observed in 23 (63.9%) tumors, none of which showed MYBL1 alterations. Clinicopathologically, a trend of a better disease-specific survival was noted in patients with MYBL1 alterations than in those with MYB-NFIB fusions and/or strong MYB expression; however, the difference was not significant. Interestingly, we found tumors with MYBL1 alterations significantly frequently occurred in the mandibular regions (P = 0.012). Moreover, literature review revealed a similar tendency in a previous study. CONCLUSION: Our results suggest that there are some biological or etiological differences between ACCs with MYB and MYBL1 alterations. Moreover, the frequent occurrence of MYBL1-associated ACC in the mandibular regions suggests that MYB immunohistochemistry is less useful in diagnosing ACCs arising in these regions. Further studies are warranted to verify our findings.