Morning glory syndrome (MGS) is a rare congenital optic disc anomaly with a characteristic fundal finding with severe visual impairment. It may occur in association with various systemic manifestations, even though most of the reported cases were isolated. A 6-year-old male visited the nephrology clinic with a history of microscopic hematuria and at the age of 12 years, he was diagnosed thin glomerular basement membrane nephropathy by kidney biopsy. After the following years, the patient had progressive deterioration of visual acuity, and diagnosed as MGS. Whole Exome Sequencing of this patient and his mother revealed heterozygous COL4A4 mutations [c.81_86del (p.Ile29_Leu30del)]. It is more reasonable to consider MGS seen in this patient as a coincidental finding of autosomal dominant Alport syndrome. To our knowledge, this case represents the first case report of autosomal dominant Alport syndrome associated with MGS.

Operational tolerance (OT), defined as maintaining stable graft function without immunosuppression after transplant surgery, is an ideal goal for kidney transplant recipients (KTRs). Recent investigations have demonstrated the distinctive features of B cells, T cells, and dendritic cell-related gene signatures and the distributions of circulating lymphocytes in these patients; nonetheless, substantial heterogeneities exist across studies. This study was conducted to determine whether previously reported candidate gene biomarkers and the profiles of lymphocyte subsets of OT could be applied in Korean KTRs. Peripheral blood samples were collected from 153 patients, including 7 operationally tolerant patients. Quantitative real-time PCR and flow cytometry were performed to evaluate gene expression and lymphocyte subsets, respectively. Patients with OT showed significantly higher levels of B cell-related gene signatures (IGKV1D-13 and IGKV4-1), while T cell-related genes (TOAG-1) and dendritic cell-related genes (BNC2, KLF6, and CYP1B1) were not differentially expressed across groups. Lymphocyte subset analyses also revealed a higher proportion of immature B cells in this group. In contrast, the distributions of CD4⁺ T cells, CD8⁺ T cells, mature B cells, and memory B cells showed no differences across diagnostic groups. An OT signature, generated by the integration of IGKV1D-13, IGKV4-1, and immature B cells, effectively discriminated patients with OT from those in other diagnostic groups. Finally, the OT signature was observed among 5.6% of patients who had stable graft function for more than 10 years while on immunosuppression. In conclusion, we validated an association of B cells and their related signature with OT in Korean KTRs.
B-Lymphocytes ; Biomarkers ; Flow Cytometry ; Gene Expression ; Humans ; Immunosuppression ; Kidney Transplantation ; Kidney* ; Lymphocyte Subsets ; Lymphocytes ; Memory ; Precursor Cells, B-Lymphoid ; Real-Time Polymerase Chain Reaction ; RNA, Messenger ; T-Lymphocytes ; Transplant Recipients* ; Transplants

BACKGROUND: An automatic alarm system was developed was developed for unexpected vital sign instability in admitted patients to reduce staffing needs and costs related to rapid response teams. This was a pilot study of the automatic alarm system, the medical emergency system (MES), and the aim of this study was to determine the effectiveness of the MES before expanding this system to all departments. METHODS: This retrospective, observational study compared the performance of patients admitted to the pulmonary department at a single center using patient data from three 3-month periods (before implementation of the MES, December 2013–February 2014; after implementation of the MES, December 2014–February 2015 and December 2015–February 2016). RESULTS: A total of 571 patients were admitted to the pulmonary department during the three observation periods. During this pilot study, the MES automatically issued 568 alarms for 415 admitted patients. There was no significant difference in the rate of cardiopulmonary resuscitation (CPR) before and after application of the MES. The mortality rate also did not change. However, it appeared that CPR was prevented in four patients admitted from the general ward to the intensive care unit (ICU) during MES implementation. The median length of hospital stay and median length of ICU stay were not significantly different before and after MES implementation. CONCLUSIONS: Although we did not find a significant improvement in outcomes upon MES implementation, the CPR rate and mortality rate did not increase despite increased comorbidities. This was a small pilot study and, based on these results, we believe that the MES may have significant effects in longer-term and larger-scale studies.
Cardiopulmonary Resuscitation ; Clinical Alarms ; Comorbidity ; Critical Care ; Emergencies* ; Humans ; Intensive Care Units ; Internal Medicine ; Korea* ; Length of Stay ; Monitoring, Physiologic ; Mortality ; Observational Study ; Patients' Rooms ; Pilot Projects* ; Retrospective Studies ; Vital Signs

BACKGROUND: An automatic alarm system was developed was developed for unexpected vital sign instability in admitted patients to reduce staffing needs and costs related to rapid response teams. This was a pilot study of the automatic alarm system, the medical emergency system (MES), and the aim of this study was to determine the effectiveness of the MES before expanding this system to all departments. METHODS: This retrospective, observational study compared the performance of patients admitted to the pulmonary department at a single center using patient data from three 3-month periods (before implementation of the MES, December 2013–February 2014; after implementation of the MES, December 2014–February 2015 and December 2015–February 2016). RESULTS: A total of 571 patients were admitted to the pulmonary department during the three observation periods. During this pilot study, the MES automatically issued 568 alarms for 415 admitted patients. There was no significant difference in the rate of cardiopulmonary resuscitation (CPR) before and after application of the MES. The mortality rate also did not change. However, it appeared that CPR was prevented in four patients admitted from the general ward to the intensive care unit (ICU) during MES implementation. The median length of hospital stay and median length of ICU stay were not significantly different before and after MES implementation. CONCLUSIONS: Although we did not find a significant improvement in outcomes upon MES implementation, the CPR rate and mortality rate did not increase despite increased comorbidities. This was a small pilot study and, based on these results, we believe that the MES may have significant effects in longer-term and larger-scale studies.
Cardiopulmonary Resuscitation ; Clinical Alarms ; Comorbidity ; Critical Care ; Emergencies ; Humans ; Intensive Care Units ; Internal Medicine ; Korea ; Length of Stay ; Monitoring, Physiologic ; Mortality ; Observational Study ; Patients' Rooms ; Pilot Projects ; Retrospective Studies ; Vital Signs

Spontaneous myometrial contraction (SMC) in pregnant uterus is greatly related with gestational age and growing in frequency and amplitude toward the end of gestation to initiate labor. But, an accurate mechanism has not been elucidated. In human and rat uterus, all TRPCs except TRPC2 are expressed in pregnant myometrium and among them, TRPC4 are predominant throughout gestation, suggesting a possible role in regulation of SMC. Therefore, we investigated whether the TRP channel may be involved SMC evoked by mechanical stretch in pregnant myometrial strips of rat using isometric tension measurement and patch-clamp technique. In the present results, hypoosmotic cell swelling activated a potent outward rectifying current in G protein-dependent manner in rat pregnant myocyte. The current was significantly potentiated by 1microM lanthanides (a potent TRPC4/5 stimulator) and suppressed by 10microM 2-APB (TRPC4-7 inhibitor). In addition, in isometric tension experiment, SMC which was evoked by passive stretch was greatly potentiated by lanthanide (1microM) and suppressed by 2-APB (10microM), suggesting a possible involvement of TRPC4/5 channel in regulation of SMC in pregnant myometrium. These results provide a possible cellular mechanism for regulation of SMC during pregnancy and provide basic information for developing a new agent for treatment of premature labor.
Animals ; Female ; Gestational Age ; Humans ; Lanthanoid Series Elements ; Mice ; Muscle Cells ; Myometrium ; Obstetric Labor, Premature ; Osmotic Pressure ; Patch-Clamp Techniques ; Pregnancy ; Rats* ; Uterine Contraction* ; Uterus

Protease-activated receptor (PAR)-2 is expressed in endothelial cells and vascular smooth muscle cells. It plays a crucial role in regulating blood pressure via the modulation of peripheral vascular tone. Although some reports have suggested involvement of a neurogenic mechanism in PAR-2-induced hypotension, the accurate mechanism remains to be elucidated. To examine this possibility, we investigated the effect of PAR-2 activation on smooth muscle contraction evoked by electrical field stimulation (EFS) in the superior mesenteric artery. In the present study, PAR-2 agonists suppressed neurogenic contractions evoked by EFS in endothelium-denuded superior mesenteric arterial strips but did not affect contraction elicited by the external application of noradrenaline (NA). However, thrombin, a potent PAR-1 agonist, had no effect on EFS-evoked contraction. Additionally, omega-conotoxin GVIA (CgTx), a selective N-type Ca2+ channel (I(Ca-N)) blocker, significantly inhibited EFS-evoked contraction, and this blockade almost completely occluded the suppression of EFS-evoked contraction by PAR-2 agonists. Finally, PAR-2 agonists suppressed the EFS-evoked overflow of NA in endothelium-denuded rat superior mesenteric arterial strips and this suppression was nearly completely occluded by omega-CgTx. These results suggest that activation of PAR-2 may suppress peripheral sympathetic outflow by modulating activity of I(Ca-N) which are located in peripheral sympathetic nerve terminals, which results in PAR-2-induced hypotension.
Animals ; Blood Pressure ; Endothelial Cells ; Hypotension* ; Mesenteric Arteries ; Mesenteric Artery, Superior ; Muscle, Smooth ; Muscle, Smooth, Vascular ; Norepinephrine ; omega-Conotoxin GVIA ; Rats ; Receptor, PAR-2 ; Thrombin

A 6-year old, female, Schnauzer dog with chief complaint of tetraparesis was referred to the Veterinary Medicine Teaching Hospital of Chungnam National University. On physical examination, neulologic examinations and magnetic resonance imaging, this patient was diagnosed into fibrocartilaginous embolic myelopathy. Although conventional treatment such as prednisolone, cefotaxime and enrofloxacin revealed no improvement, this patient showed favorable therapeutic response by combined therapy with aquapuncture with prednisolone, modified moxibustion, herbal medicine and massage.
Animals ; Cefotaxime ; Dogs ; Female ; Fluoroquinolones ; Herbal Medicine ; Hospitals, Teaching ; Humans ; Magnetic Resonance Imaging ; Massage ; Moxibustion ; Physical Examination ; Prednisolone ; Spinal Cord Diseases ; Veterinary Medicine

OBJECTIVE: The aim of this study was to assess the clinical role of 18F-FDG PET/CT for the evaluation of lymph node metastasis in periorbital malignancies, compared with CT alone. MATERIALS AND METHODS: We analyzed eighteen PET/CT and CT scans in 15 patients with biopsy-proven periorbital malignancies. We compared the diagnostic capabilities of PET/CT and CT with regard to nodal metastasis by level-by-level analysis and by N staging prediction. The reference standards were surgical pathology (n = 7) from dissected lymph node specimens and the results from radiological follow-up (n = 11, mean 20.5 months; range 10-52 months). Moreover, any changes in patient care as prompted by PET/CT were recorded and compared with treatment planning for CT alone. RESULTS: PET/CT had a sensitivity of 100%, while CT had a sensitivity of 57% (p = 0.03) for nodal metastasis by level-by-level analysis. PET/CT had a specificity of 97%, positive predictive value of 93%, negative predictive value of 100%, and diagnostic accuracy of 98%, while the CT values for these same parameters were 97%, 89%, 82%, and 84%, respectively. PET/CT correctly predicted N staging with an accuracy of 100%, while CT was only 83% accurate (p = 0.01). Regarding the impact on patient care, the extent of surgery for regional lymph nodes and the treatment decision were modified by PET/CT in 39% of patients. CONCLUSION: PET/CT could provide useful information in the management of regional lymph node metastases in patients with periorbital malignancies.
Adult ; Aged ; Contrast Media ; Eye Neoplasms/pathology/*secondary ; Eyelid Neoplasms/pathology/secondary ; Female ; Fluorodeoxyglucose F18/*diagnostic use ; Humans ; Iohexol/analogs & derivatives/diagnostic use ; Lacrimal Apparatus Diseases/pathology ; Lymphatic Metastasis/diagnosis ; Male ; Middle Aged ; *Positron-Emission Tomography ; Radiopharmaceuticals/*diagnostic use ; Sensitivity and Specificity ; *Tomography, X-Ray Computed/methods

It was previously reported that the Korean predictive model could be used to identify patients at high risk of postoperative nausea and vomiting (PONV). This study investigated whether PONV in the high-risk and very high-risk patients identified by the Korean predictive model could be prevented by multiple prophylactic antiemetics. A total of 2,456 patients were selected from our previous PONV study and assigned to the control group, and 374 new patients were recruited consecutively to the treatment group. Patients in each group were subdivided into two risk groups according to the Korean predictive model: high-risk group and very high-risk group. Patients in the treatment group received an antiemetic combination of dexamethasone 5 mg (minutes after induction) and ondansetron 4 mg (30 min before the end of surgery). The incidences of PONV were examined at two hours after the surgery in the postanesthetic care unit and, additionally, at 24 hr after the surgery in the ward, and were analyzed for any differences between the control and treatment groups. The overall incidence of PONV decreased significantly from 52.1% to 23.0% (p< or =0.001) after antiemetic prophylaxis. Specifically, the incidence decreased from 47.3% to 19.4% (p< or =0.001) in the high-risk group and from 61.3% to 28.3% (p< or =0.001) in the very high-risk group. Both groups showed a similar degree of relative risk reductions: 59.0% vs. 53.8% in the high-risk and very high-risk groups, respectively. The results of our study showed that the antiemetic prophylaxis with the combination of dexamethasone and ondansetron was effective in reducing the occurrence of PONV in both high-risk and very high-risk patients.
Adult ; Anesthetics/adverse effects ; Antiemetics/*pharmacology ; Dexamethasone/administration & dosage ; Female ; Humans ; Incidence ; Korea ; Middle Aged ; Ondansetron/administration & dosage ; Postoperative Complications/prevention & control ; Postoperative Nausea and Vomiting/*prevention & control ; Postoperative Period ; Risk ; Risk Factors ; Treatment Outcome

BACKGROUND: Opioid delivered by epidural patient-controlled analgesia (PCA) is effective in relieving pain after surgery, but it is associated with side effects, such as nausea, vomiting, pruritus, respiratory depression, and urinary retention. The purpose of this study was to compare hydromorphone related side effects and the quality of analgesia when naloxone was added to epidural PCA regimen. METHODS: Fifty-two thoracotomy patients with PCA were allocated blindly into two groups. Patients in group H (n = 26) received continuous epidural hydromorphone (16microgram/ml) in 0.1% bupivacaine; patients in group N (n = 26) received an epidural infusion containing naloxone (2 microgram/ml) and hydromorphone (16microgram/ml) in 0.1% bupivacaine. The basal rate of PCA was 4 ml/hr and the demand dose was 1.5 ml with a lockout time of 15 min. Pain intensity, sedation, pruritus, nausea and vomiting, respiratory depression were checked at 6, 12, 24 hours postoperatively. RESULTS: The Visual Analog Scale (VAS) scores were significantly lower in group H than in group N. There were no significant differences in the overall incidence of pruritus, nausea and sedation between the two groups. CONCLUSIONS: Continuous epidural infusion of naloxone combined with hydromorpho-ne is not effective in reducing the incidence and severity of pruritus induced by epidural hydromorphone.
Analgesia ; Analgesia, Patient-Controlled* ; Bupivacaine ; Humans ; Hydromorphone* ; Incidence ; Naloxone* ; Nausea ; Passive Cutaneous Anaphylaxis ; Pruritus* ; Respiratory Insufficiency ; Thoracotomy ; Urinary Retention ; Visual Analog Scale ; Vomiting