No abstract available.
Child* ; Epilepsy* ; Humans

No abstract available.
Primary Myelofibrosis*

No abstract available.
Aortic Stenosis, Supravalvular*

Augmentation cystoplasty is effective in the rehabilitation of the contracted bladder of small capacity caused by tuberculosis or interstitial cystitis refractory to more conservative methods of management. Genitourinary tuberculosis is the most common cause of contracted bladder in Korea and it may lead to considerable vesicoureteral reflux and progressive deterioration of renal function. The use of hemi-Kock ileal pouch with an afferent intussuscepted nipple is a suitable solution in patient in whom the bladder outflow tract provides adequate resistance and supratrigonal cystectomy is to be performed with construction of an antireflux mechanism. Herein, we report 4 cases of hemi-Kock ileocystoplasty.
Cystectomy ; Cystitis, Interstitial ; Humans ; Korea ; Nipples ; Rehabilitation ; Tuberculosis ; Urinary Bladder ; Vesico-Ureteral Reflux

No abstract available.
Breast Neoplasms* ; Breast* ; DNA* ; Oncogene Proteins* ; Oncogenes* ; Ploidies* ; Prognosis*

PURPOSE: Anal manometry is one of the most commonly used tests to evaluate fecal continence function. Advanced pressure parameters of the tests, such as fatigue rate index (FRI), resting rectoanal pressure gradient (RPG), cough index (CI), and radial asymmetry (RA) are recently devised to make up the inaccuracy of conventional parameters, but without solid verification. The object of this study is to investigate such parameters including conventional ones in incontinent patients compared with those in controls and to determine the significance of each parameter. METHODS: Forty-four patients with anal incontinence and 42 controls were studied. We examined conventional pressure parameters (mean resting pressure [MRP], maximum squeeze pressure [MXSP]) and advanced parameters (RPG, FRI, CI, RA), and compare those parameters with normal controls. Multiple logistic regression analysis and receiver operating characteristic curve analyses were also performed. RESULTS: No difference was detected in demographic factors between the two groups. Basic manometric parameters were significantly different between the two groups (MRP, 31.1+/-20.7 mmHg vs. 41.4+/-18.6 mmHg, P=0.0170; MXSP, 203.1+/-84.3 mmHg vs. 258.5+/-105.9 mmHg, P=0.0086). Among advanced parameters, RPG and FRI were significantly different (2.83+/-1.54 vs. 4.08+/-1.53, P=0.0003; 9+/-21.6 sec vs. 75.6+/-12.1 sec, P=0.0333), but CI and RA were not so different (0.74+/-0.45 vs. 0.61+/-0.33, P=0.1326; 22.7+/-7.0% vs. 21.1+/-7.6%, P=0.3244). Only RPG was significant in multiple logistic regression analysis (P=0.019). Areas under ROC curves were 0.65 for MRP, 0.65 for MXSP, 0.82 for RPG, and 0.73 for FRI. CONCLUSION: Among anal manometric pressure parameters, RPG and FRI as advanced parameters are more accurate than any other parameters in detecting fecal incontinence.
Cough ; Demography ; Fatigue ; Fecal Incontinence ; Humans ; Logistic Models ; Manometry ; ROC Curve

The management of patient undergoing thoracic aortic surgical procedures represents a major challenge for the anesthesiologists. Aortic cross-clamping and unclamping are particularly hszardous during the- course of the thoracic aortic operation. Proximal hypertension, myocardial ischemia and arrhythmia have been reported to occur after application of the aortic cross-clampmg. Removal of the cross-clamp often results in severe hypotension with subsequent myocardial, cerebral and renal ischemia. In this case, the patient was managed successfully using Regitine drip during the cross-clamp and slow release of clamp during unclamp, combined with slight overhydration just before unclamp.
Aortic Aneurysm, Thoracic* ; Arrhythmias, Cardiac ; Humans ; Hypertension ; Hypotension ; Ischemia ; Myocardial Ischemia ; Phentolamine

PURPOSE: To find the applicability of adjuvant radiotherapy for extrahepatic bile duct cancer (EBDC), we analyzed the pattern of failure and evaluate prognostic factors of locoregional failure after curative resection without adjuvant treatment. MATERIALS AND METHODS: In 97 patients with resected EBDC, the location of tumor was classified as proximal (n = 26) and distal (n = 71), using the junction of the cystic duct and common hepatic duct as the dividing point. Locoregional failure sites were categorized as follows: the hepatoduodenal ligament and tumor bed, the celiac artery and superior mesenteric artery, and other sites. RESULTS: The median follow-up time was 29 months for surviving patients. Three-year locoregional progression-free survival, progression-free survival, and overall survival rates were 50%, 42%, and 52%, respectively. Regarding initial failures, 79% and 81% were locoregional failures in proximal and distal EBDC patients, respectively. The most common site was the hepatoduodenal ligament and tumor bed. In the multivariate analysis, perineural invasion was associated with poor locoregional progression-free survival (p = 0.023) and progression-free survival (p = 0.012); and elevated postoperative CA19-9 (> or =37 U/mL) did with poor locoregional progression-free survival (p = 0.002), progression-free survival (p < 0.001) and overall survival (p < 0.001). CONCLUSION: Both proximal and distal EBDC showed remarkable proportion of locoregional failure. Perineural invasion and elevated postoperative CA19-9 were risk factors of locoregional failure. In these patients with high risk of locoregional failure, adjuvant radiotherapy could be considered to improve locoregional control.
Bile Duct Neoplasms ; Bile Ducts, Extrahepatic* ; Celiac Artery ; Cystic Duct ; Disease-Free Survival ; Follow-Up Studies ; Hepatic Duct, Common ; Humans ; Ligaments ; Mesenteric Artery, Superior ; Multivariate Analysis ; Prognosis ; Radiotherapy, Adjuvant* ; Recurrence ; Risk Factors ; Survival Analysis ; Survival Rate

Six transmembrane protein of prostate 2 (STAMP2) plays a key role in linking inflammatory and diet-derived signals to systemic metabolism. STAMP2 is induced by nutrients/feeding as well as by cytokines such as TNFalpha, IL-1beta, and IL-6. Here, we demonstrated that STAMP2 protein physically interacts with and decreases the stability of hepatitis B virus X protein (HBx), thereby counteracting HBx-induced hepatic lipid accumulation and insulin resistance. STAMP2 suppressed the HBx-mediated transcription of lipogenic and adipogenic genes. Furthermore, STAMP2 prevented HBx-induced degradation of IRS1 protein, which mediates hepatic insulin signaling, as well as restored insulin-mediated inhibition of gluconeogenic enzyme expression, which are gluconeogenic genes. We also demonstrated reciprocal expression of HBx and STAMP2 in HBx transgenic mice. These results suggest that hepatic STAMP2 antagonizes HBx-mediated hepatocyte dysfunction, thereby protecting hepatocytes from HBV gene expression.
Animals ; Female ; Gene Expression ; Gluconeogenesis/genetics ; Hep G2 Cells ; Humans ; Insulin/pharmacology/physiology ; Insulin Receptor Substrate Proteins/genetics/metabolism ; Insulin Resistance ; *Lipid Metabolism ; Liver/*metabolism/physiopathology ; Male ; Membrane Proteins/metabolism/*physiology ; Mice ; Mice, Inbred C57BL ; Mice, Inbred CBA ; Mice, Transgenic ; Oxidoreductases/metabolism/*physiology ; Phosphorylation ; Protein Binding ; Protein Processing, Post-Translational ; Proteolysis ; Receptor, Insulin/metabolism ; Trans-Activators/*physiology ; Transcriptional Activation

Epidermolysis bullosa simplex (EBS), characterized by a cleavage plane through basal keratinocytes, is caused by a mutation of the genes encoding keratin 5 and 14. It is often regarded as the least severe form of Epidermolysis bullosa. In 1886, Koebner described the seasonal blisterings predominantly on the palms and soles but occurring on the other sites of friction with clothes as well. His name is often used to describe EBS with the generalized blisterings. We have experienced a case of Koebner variant of EBS in a two-day old male neonate whose father had the same EBS. Hereby, we report his clinical, histopathological findings with a brief review of the literature.
Blister ; Epidermolysis Bullosa Simplex* ; Epidermolysis Bullosa* ; Fathers ; Friction ; Humans ; Infant, Newborn ; Keratin-5 ; Keratinocytes ; Male ; Seasons