Osteomesopyknosis is a rare sclerosing bone disorder of autosomal dominant inheritance. We report a first case of osteomesopyknosis in Korea. A 16-year old girl complained of diffuse back pain for 1 year. We performed physical examination, biochemical investigations and imaging studies. A radiograph of spine revealed rugger-jersey vertebra and sandwich vertebra. Bone specific alkaline phosphatase, osteocalcin and C-terminal telopeptides of type I collagen were normal. Only an axial skeleton involvement was shown on the whole body bone scan. This patient was diagnosed to have osteomesopyknosis. Osteomesopyknosis is characterized by normal level of bone turnover marker and an axial bone involvement. Osteomesopyknosis can be occurred in Korea and needs to be considered when patients, especially young patients, suffer from back pain and have only axial osteosclerosis.
Alkaline Phosphatase ; Back Pain ; Bone Diseases ; Collagen Type I ; Female ; Humans ; Korea ; Osteocalcin ; Osteosclerosis ; Physical Examination ; Skeleton ; Spine ; Wills

PURPOSE: The present study was conducted to develop a mild vascular cognitive impairment (MVCI) assessment tool for patients with stroke and to examine its validity, reliability, and clinical adequacy. METHODS: Items of this tool were developed based on previously verified cognitive assessment tools. Face, content, and criterion (concurrent) validities, optimal cut-off score for differentiation of MVCI and normal cognitive function, clinical adequacy, internal consistency, and inter-rater reliability of the assessment tool were determined in 60 stroke patients at a university hospital located in Incheon, South Korea. RESULTS: The devised MVCI assessment tool contains 20 items which were designed to assess seven cognitive domains: orientation, memory, language, attention, reasoning/abstraction, visuospatial perception, and executive function/problem solving. Content, face, and construct validities were well supported. Clinical adequacy testing revealed that the overall probability of correctly discriminating MVCI using the MVCI assessment tool for stroke was 90.0%, which was statistically significant. Furthermore, a score of 23 was found to be the optimal cut-off score for MVCI. Internal consistency and inter-rater reliability were also well supported. CONCLUSIONS: The findings of this study indicate that the developed MVCI assessment tool for stroke could serve as a clinically useful tool for detecting MVCI and for properly assessing degree of cognitive impairment in stroke patients.
Aged ; Female ; Humans ; Male ; Middle Aged ; *Mild Cognitive Impairment/complications/diagnosis ; Neuropsychological Tests/*standards ; Psychometrics/*methods ; Reproducibility of Results ; Republic of Korea ; Sensitivity and Specificity ; Stroke/*complications

Purpose: This study aimed to describe the desperate situation where the clinician should make decisions to further manage patients having experienced adverse drug reaction (ADR) to lamotrigine that is indicated to not easily controlled neuropsychiatric diseases. Methods: A descriptive analysis was done by thoroughly reviewing medical records of patients who were reported to have ADR to lamotrigine in a regional drug-safety center between 2010 and 2018. Results: Eighty-four cases of lamotrigine-related ADRs occurred in 80 patients. Skin lesions were most commonly observed in 70 cases (83.3%) and 14 cases (16.7%) had severe ADRs. Sixty-three subjects (78.8%) discontinued lamotrigine, while 17 (21.3%) continued it.At the time of discontinuation, 30.0% were prescribed aromatic antiepileptic drugs. Among 4 subjects who were eventually prescribed lamotrigine again after a period of discontinuation, 3 (75.0%) experienced its recurrence. Among patients who had taken alternative medications, the incidence of ADRs was higher in those being prescribed aromatic antiepileptic drugs than in the others being prescribed other than aromatic antiepileptic drugs (P = 0.013). Regarding the control of underlying diseases, as many as 65 (86.7%) and 68 (90.7%) failed to reach maintaining the resolved state from 6 months and 12 months after the substitution, respectively. Conclusion Patients can be easily trapped between the recurrence of ADRs and the treatment failure to a certain drug like lamotrigine, in which we can hardly find a reasonable alternative to manage them.

Objective: To determine whether dynamic susceptibility contrast-enhanced (DSC) perfusion magnetic resonance imaging (MRI) can be used to evaluate posterior cerebral circulation in pediatric patients with moyamoya disease (MMD) who underwent anterior revascularization. Materials and Methods: This study retrospectively included 73 patients with MMD who underwent DSC perfusion MRI (age, 12.2 ± 6.1 years) between January 2016 and December 2020, owing to recent-onset clinical symptoms during the follow-up period after completion of anterior revascularization. DSC perfusion images were analyzed using a dedicated software package (NordicICE; Nordic NeuroLab) for the middle cerebral artery (MCA), posterior cerebral artery (PCA), and posterior border zone between the two regions (PCA-MCA). Patients were divided into two groups; the PCA stenosis group included 30 patients with newly confirmed PCA involvement, while the no PCA stenosis group included 43 patients without PCA involvement. The relationship between DSC perfusion parameters and PCA stenosis, as well as the performance of the parameters in discriminating between groups, were analyzed. Results: In the PCA stenosis group, the mean follow-up duration was 5.3 years after anterior revascularization, and visual disturbances were a common symptom. Normalized cerebral blood volume was increased, and both the normalized time-topeak (nTTP) and mean transit time values were significantly delayed in the PCA stenosis group compared with those in the no PCA stenosis group in the PCA and PCA-MCA border zones. TTP PCA (odds ratio [OR] = 6.745; 95% confidence interval [CI] = 2.665–17.074; P < 0.001) and CBVPCA-MCA (OR = 1.567; 95% CI = 1.021–2.406; P = 0.040) were independently associated with PCA stenosis. TTPPCA showed the highest receiver operating characteristic curve area in discriminating for PCA stenosis (0.895; 95% CI = 0.803–0.986). Conclusion nTTP can be used to effectively diagnose PCA stenosis. Therefore, DSC perfusion MRI may be a valuable tool for monitoring PCA stenosis in patients with MMD.

The objective of this study was to analyze the concurrent treatment effects of ursolic acid (UA) and low-intensity treadmill exercise and to confirm the effectiveness of UA as an exercise mimetic to safely improve muscle atrophy-related diseases using Sprague-Dawley (SD) rats with skeletal muscle atrophy. Significant muscle atrophy was induced in male SD rats through hind limb immobilization using casting for 10 days. The muscle atrophy-induced SD rats were group into four: SED, sedentary; UA, daily intraperitoneal UA injection, 5 mg/kg; EX, low-intensity (10–12 m/min, 0° grade) treadmill exercise; and UEX, daily intraperitoneal UA injection, 5 mg/kg, and low-intensity (10–12 m/min, 0° grade) treadmill exercise. After 8 weeks of treatment, endurance capacity was analyzed using a treadmill, and tissues were extracted for analysis of visceral fat mass, body weight, muscle mass, expression of muscle atrophy- and hypertrophy-related genes, and endurance capacity. Although the effects of body weight gain control, muscle mass increase, and endurance capacity improvement were inadequate in the UA group, significant results were confirmed in the UEX group. The UEX group had significantly reduced body weight and visceral fat, significantly improved mass of tibialis anterior and gastrocnemius muscles, and significantly decreased atrophy-related gene expression of MuRF1 and atrogin-1, but did not have significant change in hypertrophy-related gene expression of Akt and mTOR. The endurance capacity was significantly improved in the EX and UEX groups. These data suggest that concurrent treatment with low-intensity exercise and UA is effective for atrophy-related physical dysfunctions.
Aging ; Animals ; Atrophy ; Body Weight ; Extremities ; Gene Expression ; Humans ; Immobilization ; Intra-Abdominal Fat ; Male ; Muscle, Skeletal ; Muscles ; Muscular Atrophy* ; Rats* ; Rats, Sprague-Dawley

If we analogize any external physical force applied to victims of crimes involving violence, it would be possible to not only presume the mutual action between victims and suspects but also deduce more facts related to the cases. Therefore, in this study, defining the phenomenon of amino acid compounds in sweat spreading into clothes as impact marks, experiments using ninhydrin, 1,8-dizafluoren-9-one (DFO), 1,2-indanedione-zinc (1,2-IND-Zn) were conducted to determine developmental variations through change over time, which was not performed in previous studies. A 5-week period was set up including first damage as a variation factor, and materials in each action were developed using certain reagents. The level of specimen development depending on the change over time was identified. Thus, no changes were observed at each initial level of development.
Amino Acids* ; Clothing* ; Crime ; Indicators and Reagents* ; Ninhydrin ; Sweat ; Violence ; Weapons*

Signal transducer and activator of transcription 3 (STAT3) has a crucial role in various autoimmune disorders including, inflammatory bowel disease (IBD). Our previous study demonstrated that STAT3 activation by IL-6 in colonic epithelial cells exacerbates experimental ulcerative colitis. Activated T lymphocytes are also found in ulcerative colitis patients with intestinal inflammation, but the role of STAT3 in T cells remains elusive. To determine the STAT3 function of T cells in intestinal inflammation, we generated T cell-specific STAT3 knockout (KO) mice and used dextran sulfate sodium (DSS) to induce colitis. In this study, we demonstrated that T cell-specific STAT3 deletion alleviated DSS-induced colitis in mice, resulting in reduced histological scores and myeloperoxidase (MPO) activity. Importantly, the population of T cells in the spleen and lymph nodes was significantly decreased in the control and DSS-induced groups of STAT3 KO mice. In addition, STAT3 deficiency in T cells markedly reduced the production of interferon (IFN)-γ, IL-6, and IL-17A, whereas IL-10 secretion was increased. Collectively, the results suggest that STAT3 in T cells may be a therapeutic target in ulcerative colitis by balancing the immune response through T cell homeostasis.
Animals ; Colitis* ; Colitis, Ulcerative ; Colon ; Cytokines ; Dextran Sulfate* ; Dextrans* ; Epithelial Cells ; Homeostasis ; Humans ; Inflammation ; Inflammatory Bowel Diseases ; Interferons ; Interleukin-10 ; Interleukin-17 ; Interleukin-6 ; Lymph Nodes ; Mice ; Peroxidase ; Spleen ; STAT3 Transcription Factor ; T-Lymphocytes

The dystrophic type of neurofibromatosis is a well-known bizarre deformity of the spine. There has been little literature about spontaneous vertebral column dislocation in the thoracic spine with progressive neurological symptoms. The authors present a case of thoracic spine dislocation vertically and transversely with intact posterior elements. A 35-year-old woman had stooping and back pain for five years. She developed motor weakness of both lower extremities three months ago. Plain X-ray and three-dimensional CT scans showed dislocation between T5 and T6 vertebrae, that were which was translated transversely and vertically with severe resorption of vertebral pedicles of T5 and T6. After skeletal traction, motor weakness was improved. And we performed two-staged anterior and posterior fusion to stabilize severe kyphoscoliosis. The patient was improved neurologically with free ambulation state at 24-month follow-up. Combined anterior and posterior fusion was an effective method for the treatment spontaneous vertebral column dislocation in the thoracic spine with progressive neurological symptoms.
Adult ; Back Pain ; Congenital Abnormalities ; Dislocations* ; Female ; Follow-Up Studies ; Humans ; Lower Extremity ; Neurofibromatoses* ; Neurofibromatosis 1 ; Spine* ; Tomography, X-Ray Computed ; Traction ; Walking

Purpose: To develop and evaluate a deep learning technique to automatically segment bone structures in zero echo time (ZTE) for skull magnetic resonance imaging (MRI) in children. Materials and Methods: From January to December 2021, 38 bone ZTE MRIs from infants and children (age range, 1–31 months) were collected for model development.Mask images were generated by manually segmenting the craniofacial bone using a commercial segmentation program. Among them, 35 ZTE series were used to train the three-dimensional (3D)-nnUnet deep learning model and the remaining three series were used for model validation. A temporally different dataset of 19 ZTE bone MRIs obtained in May 2022 from infants and children (age range, 3–168 months) was used to determine the model’s performance. Dice similarity coefficient was calculated for each test case.From 3D volume rendering images, segmentation accuracy, overall image quality, and visibility of cranial sutures were subjectively evaluated on a 5-point scale and compared with ground truth data from manual segmentation. Reasons for segmentation failure were analyzed using axially segmented ZTE images. Results: For the test set, the mean Dice similarity coefficient was 0.985 ± 0.019. The segmentation accuracy was lower than the ground truth without showing a statistically significant difference between the two (3.39 ± 1.11 vs. 3.73 ± 0.77, p = 0.055). The overall image quality and suture visibility showed no significant difference (3.34 ± 0.75 vs.3.42 ± 0.69, p = 0.317; 3.55 ± 0.97 vs. 3.60 ± 0.95, p = 0.157). Common reasons for low segmentation accuracy were well-pneumatized sinuses, metal artifacts, skin at the vertex level, and bones too thin. Conclusion The deep learning-based automatic segmentation technique of bone ZTE MRIs showed comparable segmentation performance to manual segmentation. Using the deep learning-based segmentation results, acceptable 3D-volume rendering images of craniofacial bones were generated.

Mucin-producing tumors of the pancreas were first reported by Ohhashi and Takagi in 1980. Since then, many cases of intraductal papillary mucinous tumor (IPMT) of the pancreas, which is almost homonymous to mucin-producing tumors of the pancreas, have been reported. IPMTs are generally regarded as tumors with a favorable prognosis. Some IPMTs have invasiveness and this is always associated with a poor prognosis. Most IPMTs arise from the main pancreatic duct and IPMTs arising from the accessory pancreatic duct are relatively rare. Only 6 cases have been reported in the literature so far. Our patient was a 43-year old man who was admitted to the hospital due to recurrent pancreatitis. An endoscopic retrograde pancreatography revealed a patulous minor papilla orifice extruding mucin and a cystic lesion in a branch of the accessory duct. A pancreaticoduodenectomy was performed and a pathologic examination of the resected specimen showed intraductal papaillary mucinous neoplasm, low grade malignancy, in the accessory pancreatic duct and its branch. We herein report this interesting case with a review of the literature.
Adult ; Humans ; Mucins* ; Pancreas ; Pancreatic Ducts* ; Pancreaticoduodenectomy ; Pancreatitis ; Prognosis