BACKGROUND: Paraneoplastic neurological disorders associated with anti-Ri-antibodies, which are typically present with opsoclonus-myoclonus-ataxia. Most cases with anti-Ri-antibodyassociated paraneoplastic syndrome due to breast cancer occur in women - its occurrence in men is extremely rare. CASE REPORT: We present herein the case of a male patient with breast cancer who had atypical anti-Ri-antibody-associated paraneoplastic syndrome presenting as complete horizontal ophthalmoplegia, left trigeminal sensory symptoms, and truncal ataxia. Following the diagnosis of paraneoplastic syndrome, chemotherapy and immunomodulating treatment including intravenous immunoglobulin and oral prednisolone were administered. Although the patient was negative for serum anti-Ri-antibodies 14 weeks later, his symptoms persisted. CONCLUSIONS: To our knowledge, this is the first case report of ophthalmoplegia without opsoclonus-myoclonus in a male anti-Ri-antibody-positive patient with breast cancer.
Ataxia ; Breast ; Breast Neoplasms ; Female ; Humans ; Immunoglobulins ; Male ; Multiple Endocrine Neoplasia Type 1 ; Nervous System Diseases ; Ophthalmoplegia ; Paraneoplastic Syndromes ; Prednisolone

This study was carried out to derive and evaluate reference computed tomography (CT)-based indices for normal canine spine. CT and magnetic resonance images were acquired from 12 clinically normal Beagle dogs (normal group) and 50 dogs with 56 spinal disorders (patient group). Image acquisition regions were cervical spine (C2–T1), thoracic spine (T1–T13), and lumbar spine (L1–L7). Measured indices were: the ratios of width to height of the spinal cord including the dura matter (CR) and of the vertebral foramen (FR), and the ratio of the cross-sectional area of the spinal cord to that of the vertebral foramen (CFAR). Reliability analysis was performed to evaluate intermodality agreement. Student's t-tests and receiver operating characteristic curves were used to discriminate the normal and patient groups on CT. Intermodality agreements of the normal and patient groups were acceptable to excellent. The highest discriminating levels of CR at the vertebral body level and the intervertebral disc space level were 1.25 or more and 1.44 or more, respectively. FR and CFAR had the highest discriminating level at the cervical region. This report presents quantitative information on canine spinal morphometry; the obtained indices may be helpful for CT screening of dogs with spinal disorders.
Animals ; Dogs ; Humans ; Intervertebral Disc ; Magnetic Resonance Imaging ; Mass Screening ; ROC Curve ; Spinal Cord ; Spine

No abstract available.
Arthritis, Rheumatoid ; Bone Density* ; Estrogens* ; Etidronic Acid* ; Female ; Humans ; NF-kappa B ; Osteoporosis, Postmenopausal*

A nasal-type extranodal natural killer/T-cell lymphoma is considered an aggressive form of non-Hodgkin's lymphoma, with approximately half of all patients relapsing during the follow-up period, and most relapses occurring within the first 2 years of remission. Here we report an unusual case of a 42-year-old man who experienced recurrence in single pleura after 8 years of remission.
Adenosine Deaminase ; Adult ; Follow-Up Studies ; Humans ; Lymphoma* ; Lymphoma, Extranodal NK-T-Cell ; Lymphoma, Non-Hodgkin ; Pleura ; Pleural Effusion ; Recurrence*

Purpose: Dipeptidyl peptidase-4 (DPP-4) inhibitors lower blood glucose levels and enhance the function of pancreatic βcells. Yet, it is unknown whether posttransplant administration of DPP4 inhibitors is beneficial for pancreas transplant recipients. Methods: We thus retrospectively analyzed the records of 312 patients who underwent pancreas transplantation between 2000 and 2018 at Asan Medical Center (Seoul, Korea) and compared the metabolic and survival outcomes according to DPP-4 inhibitor treatment. Results: The patients were divided into the no DPP-4 inhibitor group (n = 165; no treatment with DPP-4 inhibitors or treated for <1 month) and the DPP-4 inhibitor group (n = 147; treated with DPP-4 inhibitors for ≥1 month). There were no significant differences in levels of glucose, hemoglobin A1c, and insulin between the 2 groups during 36 months of follow-up. However, the level of C-peptide was significantly higher in the DPP-4 inhibitor group at 1, 6, and 24 months posttransplant (all P < 0.05). Moreover, the DPP-4 inhibitor group had significantly higher rates of overall (log-rank test, P = 0.009) and death-censored (log-rank test, P = 0.036) graft survival during a 15-year follow-up. Conclusion Posttransplant DPP-4 inhibitor administration may help improve the clinical outcomes including β cell function after pancreas transplantation.

Purpose: There are increased therapeutic usages of rituximab in kidney transplantation (KT). However, few studies have evaluated the effect of rituximab on cancer development following KT. This study aimed to evaluate the effect of rituximab on the cancer occurrence and mortality rate according to each type of cancer. Methods: Five thousand consecutive recipients who underwent KT at our center were divided into era1 (1990–2007) and era2-rit– (2008–2018), and era2-rit+ (2008–2018) groups. The era2-rit+ group included patients who received single-dose rituximab (200–500 mg) as a desensitization treatment 1–2 weeks before KT. Results: The 5-year incidence rates of malignant tumors after KT were 3.1%, 4.3%, and 3.5% in the era1, era2-rit–, and era2-rit+ group, respectively. The overall incidence rate of cancer after transplantation among the 3 study groups showed no significant difference (P = 0.340). The overall cancer-related mortality rate was 17.1% (53 of 310). Hepatocellular carcinoma (HCC) had the highest mortality rate (61.5%) and relative risk of cancer-related death (hazard ratio, 8.29; 95% confidence interval, 2.40–28.69; P = 0.001). However, we found no significant association between rituximab and the incidence of any malignancy. Conclusion Our results suggest that single-dose rituximab for desensitization may not increase the risk of malignant disease or cancer-related mortality in KT recipients. HCC was associated with the highest risk of cancer-related mortality in an endemic area of HBV infection.

PURPOSE: The 2017 international consensus guidelines (ICG) for intraductal papillary mucinous neoplasm (IPMN) of the pancreas were recently released. Important changes included the addition of worrisome features such as elevated serum CA 19-9 and rapid cyst growth (>5 mm over 2 years). We aimed to clinically validate the 2017 ICG and compare the diagnostic performance between the 2017 and 2012 ICG. METHODS: This was a retrospective cohort study. During January 2000–January 2017, patients who underwent complete surgical resection and had pathologic confirmation of branch-duct or mixed-type IPMN were included. To evaluate diagnostic performance, the areas under the receiver operating curves (AUCs) were evaluated. RESULTS: A total of 448 patients were included. The presence of mural nodule (hazard ratio [HR], 9.12; 95% confidence interval [CI], 4.60–18.09; P = 0.001), main pancreatic duct dilatation (>5 mm) (HR, 5.32; 95% CI, 2.67–10.60; P = 0.001), thickened cystic wall (HR, 3.40; 95% CI, 1.51–7.63; P = 0.003), and elevated CA 19-9 level (>37 unit/mL) (HR, 5.25; 95% CI, 2.05–13.42; P = 0.001) were significantly associated with malignant IPMN. Malignant lesions showed a cyst growth rate >5 mm over 2 years more frequently than benign lesions (60.9% vs. 29.7%, P = 0.012). The AUC was higher for the 2017 ICG than the 2012 ICG (0.784 vs. 0.746). CONCLUSION: The new 2017 ICG for IPMN is clinically valid, with a superior diagnostic performance to the 2012 ICG. The inclusion of elevated serum CA 19-9 level and cyst growth rate to the 2017 ICG is appropriate.
Area Under Curve ; Carcinoma, Pancreatic Ductal ; Cohort Studies ; Consensus ; Dilatation ; Humans ; Mucins ; Pancreas ; Pancreatic Ducts ; Retrospective Studies

Purpose: Dipeptidyl peptidase-4 (DPP-4) inhibitors lower blood glucose levels and enhance the function of pancreatic βcells. Yet, it is unknown whether posttransplant administration of DPP4 inhibitors is beneficial for pancreas transplant recipients. Methods: We thus retrospectively analyzed the records of 312 patients who underwent pancreas transplantation between 2000 and 2018 at Asan Medical Center (Seoul, Korea) and compared the metabolic and survival outcomes according to DPP-4 inhibitor treatment. Results: The patients were divided into the no DPP-4 inhibitor group (n = 165; no treatment with DPP-4 inhibitors or treated for <1 month) and the DPP-4 inhibitor group (n = 147; treated with DPP-4 inhibitors for ≥1 month). There were no significant differences in levels of glucose, hemoglobin A1c, and insulin between the 2 groups during 36 months of follow-up. However, the level of C-peptide was significantly higher in the DPP-4 inhibitor group at 1, 6, and 24 months posttransplant (all P < 0.05). Moreover, the DPP-4 inhibitor group had significantly higher rates of overall (log-rank test, P = 0.009) and death-censored (log-rank test, P = 0.036) graft survival during a 15-year follow-up. Conclusion Posttransplant DPP-4 inhibitor administration may help improve the clinical outcomes including β cell function after pancreas transplantation.

Background: Administration of pancreatic endoplasmic reticulum kinase inhibitor (PERKi) improved insulin secretion and hyperglycemia in obese diabetic mice. In this study, autophagic balance was studied whether to mediate it. Methods: Human islets were isolated from living patients without diabetes. PERKi GSK2606414 effects were evaluated in the islets under glucolipotoxicity by palmitate. Islet insulin contents and secretion were measured. Autophagic flux was assessed by microtubule associated protein 1 light chain 3 (LC3) conversion, a red fluorescent protein (RFP)-green fluorescent protein (GFP)- LC3 tandem assay, and P62 levels. For mechanical analyses, autophagy was suppressed using 3-methyladenine in mouse islets. Small interfering RNA for an autophagy-related gene autophagy related 7 (Atg7) was transfected to interfere autophagy. Results: PERKi administration to mice decreased diabetes-induced P62 levels in the islets. Glucolipotoxicity significantly increased PERK phosphorylation by 70% and decreased insulin contents by 50% in human islets, and addition of PERKi (40 to 80 nM) recovered both. PERKi also enhanced glucose-stimulated insulin secretion (6-fold). PERKi up-regulated LC3 conversion suppressed by glucolipotoxicity, and down-regulated P62 contents without changes in P62 transcription, indicating enhanced autophagic flux. Increased autophagosome-lysosome fusion by PERKi was visualized in mouse islets, where PERKi enhanced ATG7 bound to LC3. Suppression of Atg7 eliminated PERKi-induced insulin contents and secretion. Conclusion This study provided functional changes of human islets with regard to autophagy under glucolipotoxicity, and suggested modulation of autophagy as an anti-diabetic mechanism of PERKi.

Background/Aims: We investigated chromosomal aberrations in patients with pancreatic ductal adenocarcinoma (PDAC) and intraductal papillary mucinous neoplasm (IPMN) by fluorescence in situ hybridization (FISH) to identify cytogenetic changes and molecular markers that may be useful for preoperative diagnosis. Methods: Tissue samples from 48 PDAC and 17 IPMN patients were investigated by FISH analysis using probes targeting chromosomes 7q, 17p, 18q, 20q, and 21q and the pericentromeric region of chromosome 18 (CEP18). Results: The PDAC samples harbored 17p deletion (95.8%), 18q deletion (83.3%), CEP18 deletion (81.2%), 20q gain (81.2%), 21q deletion (77.1%), and 7q gain (70.8%). The IPMN samples had 17p deletion (94.1%), CEP18 deletion (94.1%), 21q deletion (70.6%), 18q deletion (58.8%), 20q gain (58.8%), and 7q gain (58.8%). A significant difference in CEP18 gain was identified between the PDAC and IPMN groups (p=0.029). Detection of 17p or 18q deletion had the highest diagnostic accuracy (80.0%) for PDAC. Conclusions Chromosomal alterations were frequently identified in both PDAC and IPMN with similar patterns. CEP18 gain and 17p and 18q deletions might be involved in the later stages of PDAC tumorigenesis. Chromosome 17p and 18q deletions might be excellent diagnostic markers.