Ectodermal dysplasia is a hereditary disease characterized by a congenital dysplasia of one or more ectodermal structures. Characteristic manifestations include scanty hair and eyebrows, pigmented and hyperkeratinized skin around the eyes and mouth, frontal bossing with prominent supraorbital ridges, nasal bridge depression and dental anomalies. Hyperthermia or unexplained high fever as a result of the deficiency of sweat glands is common medical history. Findings of intraoral structures are anodontia or oligodontia with conical crowns. Consequently, generalized spacing and loss of vertical dimension of occlusion. Interdisciplinary approach has been performed to treat a 10-year old boy with ectodermal dysplasia. Orthodontists and a prosthodontist worked together on this case, and the result was satisfactory.
Anodontia ; Child ; Crowns ; Depression ; Ectoderm* ; Ectodermal Dysplasia* ; Eyebrows ; Fever ; Genetic Diseases, Inborn ; Hair ; Humans ; Male ; Mouth ; Skin ; Sweat Glands ; Vertical Dimension

An appendicocolic fistula is a rare disease. It seems likely that the appendix becomes adherent to the viscus either before or after its perforation due to appendicitis, so surgeons may become confused when an emergency appendectomy in done. We report one case of an appendicocolic fistula to draw attention to the importance of this fistula and to describe the clinical, the diagnostic, and the therapeutic aspects of the disease.
Appendectomy ; Appendicitis ; Appendix ; Emergencies ; Fistula* ; Rare Diseases

PURPOSE: To identify the predictors of death from other causes in patients with localized renal cell carcinoma (RCC). MATERIALS AND METHODS: We identified 1,101 patients with pathologically confirmed T1 or T2 RCC with a follow-up duration of over 6 months. Survival according to the cause of death was evaluated by using the Kaplan-Meier analysis with log-rank test. Prognostic factors for death from other causes were assessed by multivariate analysis using the Cox proportional hazard regression model. Once the prognostic factors were identified, a risk-group variable was created by counting the number of unfavorable features present for each patient. RESULTS: The median follow-up was 62 months, and RCC-related death occurred in 50 patients (4.5%), whereas death from other causes occurred in 47 (4.3%). Patients who died from other causes had a higher American Society of Anesthesiologist (ASA) score (26.1% vs. 10.2%; p=0.044), older age (63.4 years vs. 55.0 years; p<0.001), smaller mass size (5.1 cm vs. 7.9 cm; p<0.001), and lower nuclear grade (p=0.003). In the multivariate Cox regression analysis, older age, higher ASA score, and lower body mass index were independent factors predicting death from other causes in patients with localized RCC. On the basis of the number of risk factors for death from other causes, the 5-year other-cause-specific survival was 98.3% (0 risk factors), 84.7% (1 risk factor), and 67.6% (2 or 3 risk factors), respectively (p<0.001). CONCLUSIONS: Older age, higher ASA score, and lower body mass index were independent predictors of death from other causes in patients with localized RCC.
Body Mass Index ; Carcinoma, Renal Cell ; Cause of Death ; Follow-Up Studies ; Humans ; Kaplan-Meier Estimate ; Multivariate Analysis ; Risk Factors ; Watchful Waiting

Purpose: Limited data exist on the use of low midline and transverse incisions for specimen extraction or stoma sites in laparoscopic rectal cancer surgery (LRCS). We compared the short-term and medium-term outcomes of these incisions and assessed whether wound complications in specimen extraction sites (SES) are increased by specimen extraction through the stoma site (SESS) in LRCS. Methods: From March 2010 to December 2017, 189 patients who underwent LRCS and specimen extraction through low abdominal incisions were divided into 2 groups: midline (n = 102) and transverse (n = 87), and perioperative outcomes were compared. Results: The midline group showed a higher frequency of temporary stoma formation (P = 0.001) and splenic flexure mobilization (P < 0.001) than the transverse group. The overall incisional hernia and wound infection rates in the SES were 21.6% and 25.5%, respectively, in the midline group and 26.4% and 17.2%, respectively, in the transverse group (P = 0.494 and P = 0.232, respectively). In patients who underwent SESS, the incisional hernia and wound infection rates of SES after stoma closure were 39.1% and 43.5%, respectively, in the midline group, and 35.5% and 22.6%, respectively, in the transverse group (P = 0.840 and P = 0.035, respectively). Conclusion In terms of incisional hernia and wound infection at the SES, a low midline incision may be used as a low transverse incision in patients without temporary stoma in LRCS. However, considering the high wound complication rates after stoma closure in patients with SESS in this study, SESS should be performed with caution in LRCS.

We investigated the role of Ca2+ and protein kinases/phosphatases in the stimulatory effect of insulin on glucose transport. In isolated rat adipocytes, the simple omission of CaCl2 from the incubation medium significantly reduced, but did not abolish, insulin-stimulated 2-deoxy glucose (2-DG) uptake. Pre-loading adipocytes with intracellular Ca2+ chelator, 5,5'-dimethyl bis (o-aminophenoxy)ethane-N,N,N'N' tetraacetic acetoxymethyl ester (5,5'-dimethyl BAPTA/AM) completely blocked the stimulation. Insulin raised intracellular Ca2+ concentration ((Ca2+)i) about 1.7 times the basal level of 72+/-5 nM, and 5,5'-dimethyl BAPTA/AM kept it constant at the basal level. This correlation between insulin-induced increases in 2-DG uptake and (Ca2+)i indicates that the elevation of (Ca2+)i may be prerequisite for the stimulation of glucose transport. Studies with inhibitors (ML-9, KN-62, cyclosporin A) of Ca2+-calmodulin dependent protein kinases/phosphatases also indicate an involvement of intracellular Ca2+. Additional studies with okadaic acid and calyculin A, protein phosphatase-1 (PP-1) and 2A (PP-2A) inhibitors, indicate an involvement of PP-1 in insulin action on 2-DG uptake. These results indicate an involvement of Ca2+-dependent signaling pathway in insulin action on glucose transport.
Adipocytes* ; Animals ; Cyclosporine ; Glucose* ; Insulin* ; Okadaic Acid ; Rats ; Staphylococcal Protein A

Purpose: This study aimed to develop a model for predicting pathologic extracapsular extension (ECE) and seminal vesicle invasion (SVI) while integrating magnetic resonance imaging-based T-staging (cTMRI, cT1c-cT3b). Materials and Methods: A total of 1,915 who underwent radical prostatectomy between 2006-2016 met the inclusion/exclusion criteria. We performed a multivariate logistic regression analysis as well as Bayesian network (BN) modeling based on possible confounding factors. The BN model was internally validated using 5-fold validation. Results: According to the multivariate logistic regression analysis, initial prostate-specific antigen (iPSA) (β=0.050, p < 0.001), percentage of positive biopsy cores (PPC) (β=0.033, p < 0.001), both lobe involvement on biopsy (β=0.359, p=0.009), Gleason score (β=0.358, p < 0.001), and cTMRI (β=0.259, p < 0.001) were significant factors for ECE. For SVI, iPSA (β=0.037, p < 0.001), PPC (β=0.024, p < 0.001), Gleason score (β=0.753, p < 0.001), and cTMRI (β=0.507, p < 0.001) showed statistical significance. BN models to predict ECE and SVI were also successfully established. The overall area under the receiver operating characteristic curve (AUC)/accuracy of the BN models were 0.76/73.0% and 0.88/89.6% for ECE and SVI, respectively. According to internal comparison between the BN model and Roach formula, BN model had improved AUC values for predicting ECE (0.76 vs. 0.74, p=0.060) and SVI (0.88 vs. 0.84, p < 0.001). Conclusion Two models to predict pathologic ECE and SVI integrating cTMRI were established and installed on a separate website for public access to guide radiation oncologists.

BACKGROUND: Small proportions of all the elderly stroke patients receive recombinant tissue plasminogen activator (r-tPA) therapy, although old age is not a proven contraindication to intravenous thrombolytic therapy for acute ischemic stroke. The purpose of this study was to identify reasons for exclusion from r-tPA therapy and factors associated with the decision of r-tPA use in elderly patients with acute ischemic stroke. METHODS: From the acute stroke registries of 22 domestic university hospitals taking the r-tPA therapy from January 2007 to May 2010, we extracted data of all acute ischemic stroke patients who were aged 80 or over and arrived within onset 3 hours. For all patients, we assessed the eligibility of r-tPA therapy using National Institute of Neurological Disorders and Stroke (NINDS) r-tPA trial criteria. For eligible patients, we compared all clinical variables between patients who were treated with r-tPA and those who were not, and analyzed potential factors related to the decision of r-tPA use. RESULTS: A total of 494 patients were included in this study. 255 patients (51.6%) were excluded by NINDS r-tPA trial criteria and the major reasons for exclusion were minor neurological deficit (53.7%) and clinical improvement (17.3%). Among 239 patients who were eligible for r-tPA, 162 (32.8%) patients received r-tPA and 77 (15.6%) did not. Multivariable analysis showed that younger age, shorter time-delay from onset to admission, non-smoker, no history of prior stroke, good pre-stroke functional status and severe initial neurological deficit were independently associated with the decision of r-tPA use in the elderly stroke patients predictors for r-tPA treatment. CONCLUSION: In very elderly patients, mild neurological deficit on arrival and rapid clinical improvement in neurological symptoms were the main reasons for exclusion from thrombolytic therapy.
Aged ; Hospitals, University ; Humans ; National Institute of Neurological Disorders and Stroke ; Registries ; Stroke ; Thrombolytic Therapy ; Tissue Plasminogen Activator

BACKGROUND: Although several hospital-based stroke studies were published, there has not been any reliable data representing the clinical characteristics of stroke in Korea. We analyzed the clinical characteristics of patients with ischemic stroke registered in the Korean Stroke Registry (KSR), which is the largest prospective hospital-based nation-wide stroke registry in Korea. METHODS: The KSR provided standardized protocols for collecting data, which includes the data of demographics, subtypes of stroke, risk factors, and neurological outcome at discharge. The brain imaging studies, including CT or MRI, were performed in all cases. RESULTS: KSR registered 10,811 patients of acute ischemic stroke between Nov. 2002 and Jun. 2004. The large-artery atherosclerosis was the most common subtype (37.3%), followed by small vessel occlusion (30.8%). Hypertension (65.4%) was the most common risk factor, followed by smoking (34.5%) and diabetes (28.3%). Although most of the hypertensive and diabetic patients had been diagnosed before the stroke, less than 45.4% and 32.5% of them were under regular control. The steno-occlusive lesion of extracranial carotid artery was only 29.3% and the ratio of intra- to extracranial artery disease was more than 2 in KSR. Only 20.5% of patients were admitted within 3 hours after stroke onset and 2.1% were treated with intravenous thrombolysis. In-hospital case-fatality was 5.2%, which is relatively comparable to those of previous studies. CONCLUSIONS: The KSR provided informative data in understanding the clinical characteristics of ischemic stroke in Korea. Further analysis of KSR will facilitate clinical trials and development of guidelines for the management of stroke patients.
Arteries ; Atherosclerosis ; Carotid Arteries ; Cerebrovascular Disorders ; Demography ; Epidemiology ; Humans ; Hypertension ; Korea ; Magnetic Resonance Imaging ; Neuroimaging ; Prospective Studies* ; Registries ; Risk Factors ; Smoke ; Smoking ; Stroke*