Maffuccis syndrome was first reported by Maffucci, in 1881. This syndrome is manifested by multiple enchondromas and hemangiomas, characterized by non-hereditary mesenchymal neoplasia that has a propensity for malignant transformation. Involvement of long bones, particularly the bones of the hands and feet, is most common. The most important complication of this syndrome is the high frequency of chondr osarcomatous metaplasia. We report a case of Maffuccis syndrome in a 56-year-old woman with multiple hemangioma on both the hands and forearms, left foot and enchondromas of multiple bones. The disease started at the age of 8 and was associated with deformity, but malignant transformation was not found.
Chondroma ; Congenital Abnormalities ; Enchondromatosis ; Female ; Foot ; Forearm ; Hand ; Hemangioma ; Humans ; Metaplasia ; Middle Aged

PURPOSE: Barriers to cardiopulmonary resuscitation (CPR) education are magnified by relative cost and course availability. E-learning has emerged as a viable solution for continuous, on-demand training and organizational learning. We assessed the hypothesis that E-learning is a viable strategy for CPR training of the general public and sought to evaluate its effects on CPR quality compared to traditional classroom-based methods. METHODS: The E-learning program was specifically designed to teach basic life support skills, and consisted of 50 minutes internet lectures and simulation videos. The training session was freely available to twenty two officers in rural South Korea. The trainees were able to practice with a mannequin and an automated external defibrillator (AED) trainer at their place of employment over the course of 3 days. The control group was trained at a hospital by certified instructors using the same equipment during a 2 hour period. At the end of the course, the participant's skills were evaluated using a checklist and a skill performance test. RESULTS: Forty two subjects were enrolled finally with 19 and 23 belonging to the E-learning and the control groups, respectively. One E-learning trainee was excluded because he was absent from the skills test. The mean time to learn CPR and AED techniques was 29.0+/-24.5 minutes in the elearning group. The mean age of the E-learning group was significantly older than that of the control group (32.4+/-4.0 vs. 26.0+/-1.5, p<0.001). However, we did not find any significant differences in their weight, height or CPR educational status. Before the education sessions, the willingness to perform CPR and their confidence in performing CPR were not significantly different among the two groups. Regarding skill performance, there were no significant differences between the groups except the volume of ventilation. The control group showed a higher volume of ventilation than the elearning group (1,031.7+/-521.6 vs. 548.8+/-303.3, p=0.004). CONCLUSION: E-learning accompanied with appropriate practice can be a helpful tool for public CPR training. The demand for E-learning will increase, and this study shows that e-learning programs can be successful, yielding similar results as traditional, classroom-based training.
Cardiopulmonary Resuscitation ; Checklist ; Defibrillators ; Education, Distance ; Educational Status ; Employment ; Hypogonadism ; Internet ; Learning ; Lectures ; Life Support Care ; Manikins ; Mitochondrial Diseases ; Ophthalmoplegia ; Republic of Korea ; Ventilation

PURPOSE: Barriers to cardiopulmonary resuscitation (CPR) education are magnified by relative cost and course availability. E-learning has emerged as a viable solution for continuous, on-demand training and organizational learning. We assessed the hypothesis that E-learning is a viable strategy for CPR training of the general public and sought to evaluate its effects on CPR quality compared to traditional classroom-based methods. METHODS: The E-learning program was specifically designed to teach basic life support skills, and consisted of 50 minutes internet lectures and simulation videos. The training session was freely available to twenty two officers in rural South Korea. The trainees were able to practice with a mannequin and an automated external defibrillator (AED) trainer at their place of employment over the course of 3 days. The control group was trained at a hospital by certified instructors using the same equipment during a 2 hour period. At the end of the course, the participant's skills were evaluated using a checklist and a skill performance test. RESULTS: Forty two subjects were enrolled finally with 19 and 23 belonging to the E-learning and the control groups, respectively. One E-learning trainee was excluded because he was absent from the skills test. The mean time to learn CPR and AED techniques was 29.0+/-24.5 minutes in the elearning group. The mean age of the E-learning group was significantly older than that of the control group (32.4+/-4.0 vs. 26.0+/-1.5, p<0.001). However, we did not find any significant differences in their weight, height or CPR educational status. Before the education sessions, the willingness to perform CPR and their confidence in performing CPR were not significantly different among the two groups. Regarding skill performance, there were no significant differences between the groups except the volume of ventilation. The control group showed a higher volume of ventilation than the elearning group (1,031.7+/-521.6 vs. 548.8+/-303.3, p=0.004). CONCLUSION: E-learning accompanied with appropriate practice can be a helpful tool for public CPR training. The demand for E-learning will increase, and this study shows that e-learning programs can be successful, yielding similar results as traditional, classroom-based training.
Cardiopulmonary Resuscitation ; Checklist ; Defibrillators ; Education, Distance ; Educational Status ; Employment ; Hypogonadism ; Internet ; Learning ; Lectures ; Life Support Care ; Manikins ; Mitochondrial Diseases ; Ophthalmoplegia ; Republic of Korea ; Ventilation

Botryomycosis is a rare, chronic and suppurative di- sease that is often mistaken clinically and histologically for a fungal infection, because the histologic feature shows a cluster of bacteria found within an eosinophilic matrix or capsule, giving the appearance of granules mimicking the sulfur granules of actinomycosis. Staphy- lococcus aureus is the most common organism cultured from lesions of botryomycosis, but other bacteria have also been isolated and implicated, including Psudomonas aeruginosa, E. coli, Proteus spp., Bacillus spp.. In some case, multiple pathogenic bacteria were found on cul ture. This disease has two patterns of organ involve ment. The more common type is cutaneous type, which involves the skin, subcutaneous soft tissue, muscle and bone. The other is visceral type which involves the lungs, liver, tongue, orbit, bowel, brain, kidney or pros tate. We report a case of brain and pulmonary botry omycosis in a 60 year-old man, who have early lung cancer. Botryomycosis was diagnosed by brain mass removal, PCNA and bronchoscopy, and lung cancer was detected incidentally by bronchoscope. The patient was treated with the Penicillin G after operation of brain mass, and right pneumonectomy was done.
Actinomycosis ; Bacillus ; Bacteria ; Brain ; Bronchoscopes ; Bronchoscopy ; Eosinophils ; Humans ; Kidney ; Liver ; Lung Neoplasms* ; Lung* ; Middle Aged ; Oceans and Seas ; Orbit ; Penicillin G ; Pneumonectomy ; Proliferating Cell Nuclear Antigen ; Proteus ; Skin ; Sulfur ; Tongue

Background: Cadherin-11, a cell-to-cell adhesion molecule, is associated with higher tumor grade and decreased patient survival. The purpose of this study was to investigate the clinical significance of cadherin-11 expression in the progression and prognosis of a newly diagnosed primary glioblastoma (GBL). Methods: Between 2007 and 2016, 52 out of 178 patients diagnosed with a GBL and satisfied the following criteria: 1) a new primary GBL, 2) gross-total resection, 3) immunohistochemically-available tissue, and 4) standardized adjuvant treatment. Results: In terms of staining intensity, the low-intensity cadherin-11 group showed longer progression-free survival (PFS) than the high-intensity cadherin-11 group (median PFS, 12.0 months [95% CI, 11.1-12.9] vs. median PFS, 6.0 months [95% CI, 3.7-8.3]; p<0.001). The low-intensity cadherin-11 group revealed longer overall survival (OS) than the high-intensity cadherin-11 group (median OS, 20.0 months [95% CI, 11.8-16.6] vs. median OS, 15.0 months [95% CI, 11.8-18.2]; p=0.003). The staining intensity of cadherin-11 was a statistically significant factor in PFS and OS in terms of univariate and multivariate analyses (univariate analysis: p<0.001 and p=0.005; multivariate analysis: p<0.001 and p=0.005). Conclusion Our clinical study demonstrates high cadherin-11 expression may be associated with poor PFS and OS for a newly diagnosed primary GBL.

Autosomal dominant polycystic kidney disease (ADPKD) is one of most common hereditary disorders that is potentially fatal. To elucidate clinical and genetic characteristics of ADPKD in Korea, 166 patients were analyzed retrospectively. The results were as follows ; 1) The male-to-female ratio was 83: 83, and age of patients was 4813(meanSD) years. 6496 of patients have a family history of the ADPKD. 2) Most common symptom was flank and ab- dominal pain. In urinalysis, proteinuria(42%), hematuria(18%) were found. Other organ involvement included hepatic cysts(58%), pancreatic cysts(8%) and splenic cyst(296). 3) The age at diagnosis was 44% 12 years. Hypertension was observed in 65%, the age of detection was 45% 11 years. Azotemia was observed in 36%, the age of detection was 52 +/- 11 years. 4) Echocardiography showed left ventricular hypertrophy 5896(19/33), valve regurgitation 1596(5/33) and mitral valve prolapse 3%(1/33). Cerebrovascular events occurred in 14 patients(8.4%), and 6 patients confirmed cerebral aneurysm rupture. 5) 29 patients(17%) reached end stage renal fail- ure(ESRD), the age was 53 +/- 10 years. The provability of being alive and not having ESRD was 88% by age 50, 76% by age 58, and 48% by age 65. Sex and hepatic cyst were not associated significantly with the course of renal function(p>0.05). 6) 15 patients(9%) died, the age of death was 55 +/- 12 years. 6 patients died after reaching ESRD. The cause of death were was cancer(4), cerebrovascular event(3), cessation of dialysis(2), sudden death (2), liver cirrhosis(1), sepsis(1), suicide(1) and unknown(1). Results of our study revealed the complications of ADPKD in Korea, hypertension 6596, ESRD 17% and cerebrovascular event 8%. Functional survival was 88M by age 50, 76% by age 58, and 48M by age 65. The extrarenal manifestations are more important contributors to mortality than renal manifestations of ADPKD.
Azotemia ; Cause of Death ; Death, Sudden ; Diagnosis ; Echocardiography ; Humans ; Hypertension ; Hypertrophy, Left Ventricular ; Intracranial Aneurysm ; Kidney Failure, Chronic ; Korea* ; Liver ; Mitral Valve Prolapse ; Mortality ; Polycystic Kidney, Autosomal Dominant ; Retrospective Studies ; Rupture ; Urinalysis

BACKGROUND: Fine-needle aspiration (FNA) of the thyroid is a widely accepted confirmatory test for thyroid cancer with high sensitivity and specificity. FNA is a simple procedure that is learned by many clinicians to enable accurate diagnosis of thyroid cancer. However, it is assumed that because the FNA test is a relatively simple procedure, its cytologic results are reliable regardless of the operator's experience. The aim of this study was to evaluate the differences in the diagnostic indices of FNA between operators with different levels of experience. METHODS: A total of 694 thyroid FNA specimens from 469 patients were reviewed, and were separated based on the experience of the clinicians who performed the procedure. One hundred and ninety were categorized in the experienced group, and 504 in the inexperienced group. All FNA results were then compared with histological data from surgically resected specimens, and the sample adequacy and diagnostic accuracy of the groups were compared. RESULTS: The age, gender, and nodule size and characteristics were similar in both groups. The sample adequacy rate was not significantly different between the experienced and nonexperienced groups (96.3% vs. 95.4%, P=0.682). However, the non-experienced group had a higher false-negative rate than the experienced group (6.4% vs. 17.2%, P=0.038), and the sensitivity of the FNA test also tended to be lower in the nonexperienced group (95.6% vs. 88.9%, P=0.065). CONCLUSION: These results suggest that FNA operators who have less experience may miss cases of thyroid cancer by performing the procedure incorrectly. As such, the experience of the FNA operator should be considered when diagnosing thyroid cancer. When clinicians are being trained in FNA, more effort should be made to increase the accuracy of the procedure; therefore, enhanced teaching programs and/or a more detailed feedback system are recommended.
Biopsy, Fine-Needle* ; Diagnosis ; Diagnostic Errors* ; Humans ; Sensitivity and Specificity ; Thyroid Gland* ; Thyroid Neoplasms*

The efficacy and safety of ertapenem, 1 g once daily, were compared with that of ceftriaxone, 2 g once daily, for the treatment of adults with acute pyelonephritis (APN) and complicated urinary tract infections (cUTIs) in a prospective, multicenter, double-blinded, randomized study. After > or = 3 days of parenteral study therapy, patients could be switched to an oral agent. Of 271 patients who were initially stratified by APN (n = 210) or other cUTIs (n = 61), 66 (48.9%) in the ertapenem group and 71 (52.2%) in the ceftriaxone group were microbiologically evaluable. The mean duration of parenteral and total therapy, respectively, was 5.6 and 13.8 days for ertapenem and 5.8 and 13.8 days for ceftriaxone. The most common pathogen was Escherichia coli. At the primary efficacy endpoint 5-9 days after treatment, 58 (87.9%) patients in the ertapenem group and 63 (88.7%) in the ceftriaxone had a favorable microbiological response. When compared by stratum and severity, the outcomes in the two groups were equivalent. The frequency and severity of drug-related adverse events were generally similar in both treatment groups. The results indicate that ertapenem is highly effective and safe for the treatment of APN and cUTIs.
Acute Disease ; Anti-Bacterial Agents/*therapeutic use ; Ceftriaxone/*therapeutic use ; Double-Blind Method ; Humans ; Odds Ratio ; Pyelonephritis/complications/*drug therapy ; Republic of Korea ; Risk Factors ; Urinary Tract Infections/complications/*drug therapy ; beta-Lactams/*therapeutic use

BACKGROUND: The green fluorescent protein (GFP) from jelly fish, Aequorea victoria, has become a versatile reporter for monitoring gene expression in a variety of cells and organisms. Using GFP as a marker protein we studied whether there are any differencies in the expression patterns among organs in mouse after intravenous injection of adenovirus vectors with GFP gene. METHODS: Recombinant E1, E3-defective type 5 adenovirus vectors (2x10(8)/mouse) with CMV promoter and GFP gene were injected into mice via tail vein. On 3, 6, 9, 14, 21, 28 days after gene transfer, 5 mice per experiments were sacrificed by cervical dislocation and obtained liver, lung, heart, kidney, spleen, small intestine and bone. Half of them were examined by optical microscope after H-E stain. Another half were examined by fluorescent microscope after frozen section. Western blottings were done for each samples with anti-GFP monoclonal antibody and obtained GFP bands were quantitatively compared using Gel-Doc (Bio-Rad, USA) image analyzer. RESULTS: In all organs that we obtained, expression of GFPs are noticed 3 days after gene transfer and reached a maximum around 9th to 14th days, after then the intensities are slightly decreased but maintained until 28th days as determined by Western blotting. On fluorescent microscopic examination, GFPs are well and most frequently expressed on lung among all the examined organs. There are little expression of GFPs on liver parenchymal area around the sinusoids and central veins, although patchy expression of GFPs are observed along the liver capsules. GFPs are highly expressed around the splenic trabecula area but splenic pulp area, it is very sparsely expressed. GFPs are more frequently and highly expressed around the renal tubular area than gromerular area in kidneys. In small intestine, GFPs are expressed on mid portion of microvilli. GFPs are not expressed on myocardium except scanty expression on endocardium. Bone marrow showed GFPs but precise localization is difficult because bony spicules mashed bone marrow during the preparation of frozen section. No specific pathologic lesions possibly related with adenovirus administration are observed on microscopic examination of H-E stained specimens. CONCLUSIONS: GFPs can be detected in cells without the fixing and staining and a good marker to studying the kinetics and persistence of adenovirus mediated gene therapy. And there are different GFP expression patterns according to the organs after intravenous injection of adenovirus vectors with GFP gene in mouse.
Adenoviridae* ; Animals ; Blotting, Western ; Bone Marrow ; Capsules ; Dislocations ; Endocardium ; Fluorescence ; Frozen Sections ; Gene Expression ; Genetic Markers ; Genetic Therapy ; Heart ; Injections, Intravenous* ; Intestine, Small ; Kidney ; Kinetics ; Liver ; Lung ; Mice* ; Microvilli ; Myocardium ; Spleen ; Veins ; Victoria

PURPOSE: The objective of this study was to evaluate our institutional experience with veno-venous (VV) extracorporeal membrane oxygenation (ECMO) in patients with severe acute respiratory failure (ARF). MATERIALS AND METHODS: From January 2007 to August 2013, 31 patients with severe ARF that was due to various causes and refractory to mechanical ventilation with conventional therapy were supported with VV ECMO. A partial pressure of arterial oxygen (PaO2)/inspired fraction of oxygen (FiO2) <100 mm Hg at an FiO2 of 1.0 or a pH <7.25 due to CO2 retention were set as criteria for VV ECMO. RESULTS: Overall, 68% of patients survived among those who had received VV ECMO with a mean PaO2/FiO2 of 56.8 mm Hg. Furthermore, in trauma patients, early use of ECMO had the best outcome with a 94% survival rate. CONCLUSION: VV ECMO is an excellent, life-saving treatment option in patients suffering from acute and life-threatening respiratory failure due to various causes, especially trauma, and early use of VV ECMO therapy improved outcomes in these patients.
Acute Disease ; Adult ; Cause of Death ; *Extracorporeal Membrane Oxygenation ; Female ; Humans ; Male ; Middle Aged ; Respiratory Insufficiency/complications/*therapy ; Survival Analysis ; Treatment Outcome