Congenital insensitivity to pain with anhidrosies (CIPA) is one of the exceedingly rare hereditary sensory autonomic neuropathies (HSAN). There are five types of HSAN according to hereditary transmission, natural history, clinical manifestations, and pathologic findings. The CIPA corresponds to type IV and is characterized by episodes of recurrent fever with generalized anhidrosis, insensitivity to pain and temperature with self-multilation, and mental retardation. We report a 14 month old girl with the clinical symptoms and neuropathological findings of CIPA without family history. She presented with episodes of recurrent fever and self-mutilation of her tongue. Clinical, laboratory and pathologic aspects of this patient are discussed along with review of the literature.
Female ; Fever ; Hereditary Sensory and Autonomic Neuropathies* ; Humans ; Hypohidrosis ; Infant ; Intellectual Disability ; Natural History ; Pain Insensitivity, Congenital* ; Tongue

Coronary stenting for long complex lesion is effective but associated with complication. We compared the results of stenting between with multiple conventional stenting group (group A) and with single long stenting group (group B). Fifty patients were prospectively and randomly enrolled: 25 patients for each group. Each group showed no significant differences of clinical characteristics. One patient died of heart failure in each group, not associated with the procedure itself. One patients had cerebrovascular accident in each group. Five patients had major bleeding (2, group A; 3, group B). Angiographic success rate was 100% in each group and procedural success rate was 96% and 100% in group A and B, respectively. Angiographic and clinical restenosis rate at 6 months follow-up were 60%, 36% in group A and 65%, 44% in group B, respectively (p=S). Multivariate analysis showed that several factors affected the angiographic restenosis rate as follows; a) male gender (M:F=76.9%:25.0%, P<0.001), b) AMI (AMI:stable angina pectoris=72.7%:66.7%, P<0.001), c) lesion length d) residual stenosis. In conclusion, there were no statistical differences of restenosis and complication rate between the two groups. Our data support single long stenting is acceptable and economically more favorable for long diffuse lesion, compared to multiple conventional stenting.
Constriction, Pathologic ; Coronary Stenosis* ; Coronary Vessels* ; Follow-Up Studies ; Heart Failure ; Hemorrhage ; Humans ; Male ; Multivariate Analysis ; Prospective Studies ; Stents* ; Stroke

No abstract available.
Humans ; Intubation* ; Ludwig's Angina*

Purpose: Glioblastoma (GBM) is one of the most lethal human tumors with a highly infiltrative phenotype. Our previous studies showed that GBM originates in the subventricular zone, and that tumor-derived mesenchymal stem-like cells (tMSLCs) promote the invasiveness of GBM tumorspheres (TSs). Here, we extend these studies in terms of ventricles using several types of GBM patient-derived cells. Materials and Methods: The invasiveness of GBM TSs and ventricle spheres (VSs) were quantified via collagen-based 3D invasion assays. Gene expression profiles were obtained from microarray data. A mouse orthotopic xenograft model was used for in vivo experiments. Results: After molecular and functional characterization of ventricle-derived mesenchymal stem-like cells (vMSLCs), we investigated the effects of these cells on the invasiveness of GBM TSs. We found that vMSLC-conditioned media (CM) significantly accelerated the invasiveness of GBM TSs and VSs, compared to the control and even tMSLC-CM. Transcriptome analyses revealed that vMSLC secreted significantly higher levels of several invasiveness-associated cytokines. Moreover, differentially expressed genes between vMSLCs and tMSLCs were enriched for migration, adhesion, and chemotaxis-related gene sets, providing a mechanistic basis for vMSLC-induced invasion of GBM TSs. In vivo experiments using a mouse orthotopic xenograft model confirmed vMSLCinduced increases in the invasiveness of GBM TSs. Conclusion Although vMSLCs are non-tumorigenic, this study adds to our understanding of how GBM cells acquire infiltrative features by vMSLCs, which are present in the region where GBM genesis originates.

Background: In recent years, implant-based breast reconstruction has been performed because of its simplicity, short operation time, and rapid recovery of patients. Several studies have reported treatment methods for implant surgery-related infection, which is a serious complication. The aim of this study was to introduce our strategy for salvaging infected implants and to evaluate its effectiveness.
Methods: The authors performed a retrospective study of 145 cases from 132 patients who underwent implant-based breast reconstruction from January 2012 to December 2018. Empirical antibiotics were immediately administered to patients with suspected infections. The patients then underwent salvage treatment including appropriate antibiotics, ultrasonography-guided aspiration, debridement, antibiotic lavage, and implant exchange through a multidisciplinary approach. Patient demographics, operative data, duration until drain removal, adjuvant treatment, and complications were analyzed.
Results: The total infection rate was 5.5% (8/145). A longer indwelling catheter period and adjuvant treatment were significantly associated with infection. The salvage treatment showed a success rate of 87.5% (7/8). Seven patients who received early aggressive salvage treatment recovered from infection. One patient with methicillin-resistant Staphylococcus aureus, who received salvage treatment 11 days after symptom onset, did not respond to drainage and antibiotic treatment. That patient subsequently underwent explantation.
Conclusions In implant-based breast reconstruction, prevention of infection is of the utmost importance. However, if an infection is suspected, proactive empirical antibiotic therapy and collaboration with the necessary departments are required. Through a multidisciplinary approach and proactive early management, swift and appropriate salvage should be performed.

Purpose: A critical indicator of the overall survival of patients with high-grade glioma is the successful isolation of tumor mesenchymal stem-like cells (tMSLCs), which play important roles in glioma progression. However, attempts to isolate tMSLCs from surgical specimens have not always been successful, and the reasons for this remain unclear. Considering that the amount of surgical high-grade glioma specimens varies, we hypothesized that larger surgical specimens would be better for tMSLC isolation. Materials and Methods: We assessed 51 fresh, high-grade glioma specimens and divided them into two groups according to the success or failure of tMSLC isolation. The success of tMSLC isolation was confirmed by plastic adherence, presenting antigens, tri-lineage differentiation, and non-tumorigenicity. Differences in characteristics between the two groups were tested using independent two sample t-tests, chi-square tests, or Kaplan-Meier survival analysis. Results: The mean specimen weights of the groups differed from each other (tMSLC-negative group: 469.9±341.9 mg, tMSLC positive group: 546.7±618.9 mg), but the difference was not statistically significant. The optimal cut-off value of specimen weight was 180 mg, and the area under the curve value was 0.599. Conclusion Our results suggested a minimum criterion for specimen collection, and found that the specimen amount was not deeply related to tMSLC detection. Collectively, our findings imply that the ability to isolate tMSLCs is determined by factors other than the specimen amount.

Purpose: A critical indicator of the overall survival of patients with high-grade glioma is the successful isolation of tumor mesenchymal stem-like cells (tMSLCs), which play important roles in glioma progression. However, attempts to isolate tMSLCs from surgical specimens have not always been successful, and the reasons for this remain unclear. Considering that the amount of surgical high-grade glioma specimens varies, we hypothesized that larger surgical specimens would be better for tMSLC isolation. Materials and Methods: We assessed 51 fresh, high-grade glioma specimens and divided them into two groups according to the success or failure of tMSLC isolation. The success of tMSLC isolation was confirmed by plastic adherence, presenting antigens, tri-lineage differentiation, and non-tumorigenicity. Differences in characteristics between the two groups were tested using independent two sample t-tests, chi-square tests, or Kaplan-Meier survival analysis. Results: The mean specimen weights of the groups differed from each other (tMSLC-negative group: 469.9±341.9 mg, tMSLC positive group: 546.7±618.9 mg), but the difference was not statistically significant. The optimal cut-off value of specimen weight was 180 mg, and the area under the curve value was 0.599. Conclusion Our results suggested a minimum criterion for specimen collection, and found that the specimen amount was not deeply related to tMSLC detection. Collectively, our findings imply that the ability to isolate tMSLCs is determined by factors other than the specimen amount.

PURPOSE: Although quantification of amyloid positron emission tomography (PET) is important for evaluating patients with cognitive impairment, its routine clinical use is hampered by complicated preprocessing steps and required MRI. Here, we suggested a one-step quantification based on deep learning using native-space amyloid PET images of different radiotracers acquired from multiple centers.METHODS: Amyloid PET data of the Alzheimer Disease Neuroimaging Initiative (ADNI) were used for this study. A training/validation consists of 850 florbetapir PET images. Three hundred sixty-six florbetapir and 89 florbetaben PET images were used as test sets to evaluate the model. Native-space amyloid PET images were used as inputs, and the outputs were standardized uptake value ratios (SUVRs) calculated by the conventional MR-based method.RESULTS: The mean absolute errors (MAEs) of the composite SUVR were 0.040, 0.060, and 0.050 of training/validation and test sets for florbetapir PETand a test set for florbetaben PET, respectively. The agreement of amyloid positivity measured by Cohen's kappa for test sets of florbetapir and florbetaben PET were 0.87 and 0.89, respectively.CONCLUSION: We suggest a one-step quantification method for amyloid PET via a deep learning model. The model is highly reliable to quantify the amyloid PET regardless of multicenter images and various radiotracers.
Alzheimer Disease ; Amyloid ; Cognition Disorders ; Humans ; Learning ; Magnetic Resonance Imaging ; Methods ; Neuroimaging ; Positron-Emission Tomography

PURPOSE: Although quantification of amyloid positron emission tomography (PET) is important for evaluating patients with cognitive impairment, its routine clinical use is hampered by complicated preprocessing steps and required MRI. Here, we suggested a one-step quantification based on deep learning using native-space amyloid PET images of different radiotracers acquired from multiple centers. METHODS: Amyloid PET data of the Alzheimer Disease Neuroimaging Initiative (ADNI) were used for this study. A training/validation consists of 850 florbetapir PET images. Three hundred sixty-six florbetapir and 89 florbetaben PET images were used as test sets to evaluate the model. Native-space amyloid PET images were used as inputs, and the outputs were standardized uptake value ratios (SUVRs) calculated by the conventional MR-based method. RESULTS: The mean absolute errors (MAEs) of the composite SUVR were 0.040, 0.060, and 0.050 of training/validation and test sets for florbetapir PETand a test set for florbetaben PET, respectively. The agreement of amyloid positivity measured by Cohen's kappa for test sets of florbetapir and florbetaben PET were 0.87 and 0.89, respectively. CONCLUSION We suggest a one-step quantification method for amyloid PET via a deep learning model. The model is highly reliable to quantify the amyloid PET regardless of multicenter images and various radiotracers.