Paroxysmal kinesigenic dyskinesia (PKD) is a diagnostic term for transient, involuntary abnormal movements triggered by sudden motions. The treatment for PKD differs from other paroxysmal dyskinesias, as it notably responds well to sodium channel blockers. We report a case of atypical PKD, coupled with paroxysmal exercise-induced dyskinesia (PED). Both PKD and PED in this patient showed a good response to oxcarbazepine. This case could be clinical evidence that paroxysmal dyskinesias could potentially be regarded as a spectrum disorder with overlapping features.

Background: Nilotinib is a tyrosine kinase inhibitor approved by the Ministry of Food and Drug Safety for frontline and 2nd line treatment of Philadelphia chromosome-positive chronic myeloid leukemia (Ph+ CML). This study aimed to confirm the safety and efficacy of nilotinib in routine clinical practice within South Korea. Methods: An open-label, multicenter, single-arm, 12-week observational post-marketing surveillance (PMS) study was conducted on 669 Korean adult patients with Ph + CML from December 24, 2010, to December 23, 2016. The patients received nilotinib treatment in routine clinical practice settings. Safety was evaluated by all types of adverse events (AEs) during the study period, and efficacy was evaluated by the complete hematological response (CHR) and cytogenetic response. Results: During the study period, AEs occurred in 61.3% (410 patients, 973 events), adverse drug reactions (ADRs) in 40.5% (271/669 patients, 559 events), serious AEs in 4.5% (30 patients, 37 events), and serious ADRs in 0.7% (5 patients, 8 events). Furthermore, unexpected AEs occurred at a rate of 6.9% (46 patients, 55 events) and unexpected ADRs at 1.2% (8 patients, 8 events). As for the efficacy results, CHR was achieved in 89.5% (442/494 patients), and minor cytogenetic response or major cytogenetic response was achieved in 85.8% (139/162 patients). Conclusion This PMS study shows consistent results in terms of safety and efficacy compared with previous studies. Nilotinib was well tolerated and efficacious in adult Korean patients with Ph + CML in routine clinical practice settings.

Purpose: Resuscitative endovascular balloon occlusion of the aorta (REBOA) has emerged as a salvage technique changing the paradigm in the management of noncompressible torso hemorrhage. However, training for the REBOA procedure is rarely performed. The endovascular training for REBOA (ET-REBOA) course was conducted to develop the endovascular skills of participants. Methods: Sixteen residents and 12 specialists participated in this educational course. All participants were provided with precourse learning materials. The ET-REBOA course consisted of 2 sections; an ultrasound-guided sheath insertion on the puncture model, and a balloon manipulation on the vascular circuit model. A 13-item procedure checklist and the time required to perform the procedure were examined. Pre/post self-reported confidence score and course satisfaction questionnaire were obtained. Results: Twenty-eight participants performed the 56 REBOA procedures. On the first attempt, the median total time for REBOA from ultrasound-guided vascular access to balloon inflation was 1,139 ± 250 seconds in the resident group and 828 ± 280 seconds in the specialist group. The median shortened time for completion was 273 seconds and 290 seconds respectively. A significant decrease in procedure task time was observed between first and second attempts in the resident group (P = 0.016), specialist group (P = 0.004), and in total among all participants (P < 0.001). Conclusion The ET-REBOA course significantly decreased the time taken to perform the REBOA procedure with high satisfaction of the participants. The course could be an effective curriculum for the development of endovascular skills for performing REBOA.

Angiomatoid Spitz nevus is a variant of melanocytic nevus with prominent vasculature. Due to its pathologic features, angiomatoid Spitz nevus in the vaginal wall is extremely rare. A 42-year-old woman presented to the hospital with abnormal vaginal bleeding. Vaginal examination revealed a 2×2-cm well-demarcated tumor on the posterior wall of the vagina. The mass was successfully removed by complete excision and was diagnosed as angiomatoid Spitz nevus on pathologic examination. We present the first reported case of vaginal angiomatoid Spitz nevus, which caused vaginal bleeding. Although angiomatoid Spitz nevus has many histopathological similarities with malignant melanoma, precise histopathological diagnosis is important for preventing overtreatment.
Adult ; Diagnosis ; Female ; Gynecological Examination ; Humans ; Medical Overuse ; Melanoma ; Nevus, Epithelioid and Spindle Cell ; Nevus, Pigmented ; Uterine Hemorrhage ; Vagina

To evaluate the carcinogenicity of p27 knockout (KO) mice with RNA-guided endonuclease (RGENs)-mediated p27 mutant exon I gene (IΔ), alterations in the carcinogenic phenotypes including tumor spectrum, tumor suppressor proteins, apoptotic proteins and cell cycle regulators were observed in p27 (IΔ) KO mice after treatment with 7,12-Dimethylbenz[a]anthracene (DMBA) and 12-O-tetradecanoylphorbol-13-acetate (TPA)(DT) for 5 months. The target region (544~571 nt) in exon I of the p27 gene was successfully disrupted in p27 (IΔ) KO mice using the RGEN-induced non-homologous end joining (NHEJ) technique. After DT exposure for 5 months, a few solid tumors (identified as squamous cell carcinoma) developed on the surface of back skin of DT-treated p27 (IΔ) KO mice. Also, squamous cell hyperplasia with chronic inflammation was detected in the skin dermis of DT-treated p27 (IΔ) KO mice, while the Vehicle+p27 (IΔ) KO mice and WT mice maintained their normal histological skin structure. A significant increase was observed in the expression levels of tumor suppressor protein (p53), apoptotic proteins (Bax, Bcl-2 and Caspase-3) and cell-cycle regulator proteins (Cyclin D1, CDK2 and CDK4) in the skin of DT-treated p27 (IΔ) KO mice, although their enhancement ratio was varied. Taken together, the results of the present study suggest that squamous cell carcinoma and hyperplasia of skin tissue can be successfully developed in new p27 (IΔ) KO mice produced by RGEN-induced NHEJ technique following DT exposure for 5 months.
9,10-Dimethyl-1,2-benzanthracene* ; Animals ; Carcinogenesis ; Carcinoma, Squamous Cell* ; Cell Cycle ; Dermis ; Epithelial Cells* ; Exons ; Hyperplasia* ; Inflammation ; Mice* ; Phenotype ; Skin ; Tumor Suppressor Proteins

The butanol extract of Asparagus cochinchinensis roots fermented with Weissella cibaria (BAfW) significantly suppressed the inflammatory response induced by lipopolysaccharide (LPS) treatment in RAW264.7 cells. To investigate the dose dependence and durability of BAfW on the anti-asthma effects, alterations in key parameters were measured in ovalbumin (OVA)-challenged Balb/c mice treated with the different doses of BAfW at three different time points. The number of immune cells, OVA-specific IgE level, thickness of respiratory epithelium and mucus score decreased significantly in a dose-dependent manner in response to treatment with 125 to 500 mg/kg BAfW (P < 0.05), although the highest level was detected in the 500 mg/kg treated group. Moreover, the decrease in these parameters was maintained from 24 to 48 h in the 500 mg/kg of BAfW treated group. At 72 h, the effects of BAfW on the number of immune cells, OVA-specific IgE level and thickness of respiratory epithelium partially disappeared. Overall, this study provides the first evidence that the anti-asthma effect of BAfW may reach the maximum level in OVA-challenged Balb/c mice treated with 500 mg/kg and that these effects can last for 48 h.
Animals ; Asthma* ; Fermentation ; Immunoglobulin E ; Mice ; Mucus ; Ovalbumin ; Respiratory Mucosa ; Therapeutic Uses* ; Weissella

A few clues about correlation between endoplasmic reticulum (ER) stress and mulberry (Morus alba) leaves were investigated in only the experimental autoimmune myocarditis and streptozotocin-induced diabetes. To investigate whether a novel extract of mulberry leaves fermented with Cordyceps militaris (EMfC) could suppress ER in fatty liver, alterations in the key parameters for ER stress response were measured in high fat diet (HFD)-induced obese C57L/6 mice treated with EMfC for 12 weeks. The area of adipocytes in the liver section were significantly decreased in the HFD+EMfC treated group as compared to the HFD+Vehicle treated group, while their level was higher in HFD+Vehicle treated group than No treated group. The level of the eukaryotic initiation factor 2 alpha (eIF2α) and inositol-requiring enzyme 1 beta (IRE1α) phosphorylation and CCAAT-enhancer-binding protein homologous protein (CHOP) expression were remarkably enhanced in the HFD+Vehicle treated group. However, their levels were restored in the HFD+EMfC treated group, although some differences were detected in the decrease rate. Similar recovery was observed on the ER stress-induced apoptosis. The level of Caspase-3, Bcl-2 and Bax were decreased in the HFD+EMfC and HFD+orlistat (OT) treated group compared to the HFD+Vehicle treated group. The results of the present study therefore provide first evidence that EMfC with the anti-obesity effects can be suppressed ER stress and ER stress-induced apoptosis in the hepatic steatosis of HFD-induced obesity model.
Adipocytes ; Animals ; Apoptosis ; Caspase 3 ; CCAAT-Enhancer-Binding Proteins ; Cordyceps* ; Diet, High-Fat ; Endoplasmic Reticulum Stress* ; Endoplasmic Reticulum* ; Eukaryotic Initiation Factor-2 ; Fatty Liver ; Liver ; Mice* ; Morus* ; Myocarditis ; Obesity ; Phosphorylation

Regulation of gastrointestinal hormones have been reported in animal models for constipation undergoing laxative therapy when administered herbal products. We undertook to investigate whether the laxative activity of gallotannin-enriched extracts isolated from Galla Rhois (GEGR) affects the regulation of gastrointestinal hormones, by examining the concentration of four hormones and the activation of their receptors in the loperamide (Lop)-induced constipation model. Stool parameters, including number, weight and water content, were significantly recovered in the Lop+GEGR treated group, relative to the Lop+vehicle treated group; however, food intake and water consumption were maintained at a constant level. Also, a similar recovery was detected for thickness of mucosa, muscle and flat luminal surface in the Lop+GEGR treated group. Furthermore, concentration of the four gastrointestinal hormones evaluated, namely, cholecystokinin (CCK), gastrin (GAS), somatostatin (SS) and motilin (MTL), were lower in the Lop+vehicle treated group than the No treated group, but were remarkably enhanced in the Lop+GEGR treated group. Moreover, the downstream signaling pathway of MTL and SS receptors were recovered after GEGR administration. Results of the present study therefore indicate that the laxative effects of GEGR treatment may be tightly related with the regulation of gastrointestinal hormones in the Lop-induced constipation model.
Animals ; Cholecystokinin ; Constipation* ; Drinking ; Eating ; Gastrins ; Gastrointestinal Hormones* ; Loperamide ; Models, Animal ; Motilin ; Mucous Membrane ; Phenobarbital ; Rats* ; Somatostatin ; Water

OBJECTIVE: To investigate the efficacy of portable microcurrent therapy device (PMTD) of the hip internal rotators in the treatment of in-toeing gait caused by increased femoral anteversion in children over 8 years of age. METHODS: Eleven children (22 legs; 4 boys and 7 girls; mean age, 10.4±1.6 years) with in-toeing gait caused by increased femoral anteversion were included in the present study. All children received 60 minutes of PMTD (intensity, 25 µA; frequency, 8 Hz) applied to the hip internal rotators daily for 4 weeks. Hip internal rotation (IR) angle, external rotation (ER) angle, and midmalleolar-second toe angle (MSTA) measurement during stance phase at transverse plane and Family Satisfaction Questionnaire, frequency of tripping and fatigue like pains about the PMTD were performed before treatment and at 4 weeks after initial PMTD treatment. Paired t-test and Fisher exact test were used for statistical analysis. RESULTS: Hip IR/ER/MSTA was 70.3°±5.4°/20.1°±5.5°/–11.4°±2.7°, and 55.7°±7.8°/33.6°±8.2°/–2.6°±3.8° before treatment and at 4 weeks after initial PMTD treatment, respectively (p<0.01). Ten of 11 (91%) children's family stated that they were generally satisfied with the PMTD treatment. The frequency of tripping and fatigue like pains was significantly lower at 4 weeks after PMTD treatment (p<0.05). Excellent inter-rater and intra-rater reliability was observed for repeated MSTA measurements between the examiners (k=0.91–0.96 and k=0.93–0.99), respectively. CONCLUSION: PMTD of the hip internal rotators can be effective in improving the gait pattern of children with in-toeing gait caused by increased femoral anteversion.
Bone Anteversion ; Child* ; Electric Stimulation Therapy ; Fatigue ; Female ; Gait* ; Hip ; Humans ; Leg ; Pilot Projects* ; Toes

BACKGROUND: Because conventional echocardiographic parameters have several limitations, strain echocardiography has often been introduced in clinical practice. However, there are also obstacles in using it in clinical practice. Therefore, we wanted to find the current status of awareness on using strain echocardiography in Korea. METHODS: We conducted a nationwide survey to evaluate current use and awareness of strain echocardiography from the members of the Korean Society of Echocardiography. RESULTS: We gathered total 321 questionnaires from 25 cardiology centers in Korea. All participants were able to perform or interpret echocardiographic examinations. All participating institutions performed strain echocardiography. Most of our study participants (97%) were aware of speckle tracking echocardiography and 185 (58%) performed it for clinical and research purposes. Two-dimensional strain echocardiography was the most commonly used modality and left ventricle (LV) was the most commonly used cardiac chamber (99%) for clinical purposes. Most of the participants (89%) did not think LV strain can replace LV ejection fraction (LVEF) in their clinical practice. The common reasons for not performing routine use of strain echocardiography was diversity of strain measurements and lack of normal reference value. Many participants had a favorable view of the future of strain echocardiography. CONCLUSION: Most of our study participants were aware of strain echocardiography, and all institutions performed strain echocardiography for clinical and research purposes. However, they did not think the LV strain values could replace LVEF. The diversity of strain measurements and lack of normal reference values were common reasons for not using strain echocardiography in clinical practice.
Cardiology ; Echocardiography* ; Heart Ventricles ; Korea* ; Reference Values