Dermatomyositis is a distinctive entity that is identified by a characteristic rash that accompanies or more often precedes proximal muscle weakness. There is a well recognized association between dermatomyositis and several cancers, such as ovarian cancer, lung cancer, pancreas cancer, stomach cancer and colorectal cancers and non-Hodgkin Lymphoma. But dermatomyositis associated with intrahepatic cholangiocarcinoma has not yet been reported in Korea. We experienced a case of dermatomyositis associated with infiltrative intrahepatic cholangiocarcinoma and we report on this unusual case along with reviewing the related literature.
Cholangiocarcinoma ; Colorectal Neoplasms ; Dermatomyositis ; Exanthema ; Korea ; Liver Neoplasms ; Lung Neoplasms ; Lymphoma, Non-Hodgkin ; Muscle Weakness ; Ovarian Neoplasms ; Pancreatic Neoplasms ; Stomach Neoplasms

OBJECTIVE: The aim of this study is as follows; First, to assess knowledge regarding cervical cancer, HPV and the acceptance of HPV vaccination among girls in their late teens in Seoul, Korea. Second, to find out factors affecting the acceptance of HPV vaccination. METHOD: From November to December 2006, we surveyed Ewha Womans University freshmen and third year female students of Keumran High school affiliated with Ewha Womans University. A written questionnaire was sent to 310 students and 275 students responded. The questionnaire was divided into three sections that sought information about the followings : (1) Demographic characteristics; (2) Knowledge regarding cervical Pap smear testing and cervical cancer; (3) Knowledge regarding HPV and HPV vaccines. We scored the questionnaire giving one point for each correct answer. We summed up scores of knowledge on each section and compared the scores according to the acceptance of HPV vaccination in target group. RESULTS: The mean score of knowledge regarding cervical Pap smear testing and cervical cancer, HPV and HPV vaccines were 5.31+/-1.48 and 3.11+/-2.09, respectively. None of the demographic characteristics influenced the acceptance of HPV vaccine. The level of knowledge regarding HPV and HPV vaccines significantly affected the acceptance of HPV vaccine, however, knowledge regarding cervical Pap smear testing and cervical cancer did not. CONCLUSION: This study emphasize the need for further education regarding HPV infection and HPV vaccination for improvement in HPV vaccine acceptance.
Adolescent* ; Education ; Female* ; Humans* ; Korea* ; Papillomavirus Vaccines ; Surveys and Questionnaire ; Seoul ; Uterine Cervical Neoplasms* ; Vaccination*

BACKGROUND/AIMS: Recently, many cases of vitamin K-dependent coagulopathy of unknown origin have been reported. Such patients lack any relevant family history and have no systemic disease, raising suspicion of superwarfarin intoxication. We evaluated individual risk factors causing coagulopathy and hemorrhagic symptoms in patients with suspected superwarfarin intoxication. In addition, we determined how to effectively treat vitamin K-dependent coagulopathy caused by suspected superwarfarin intoxication. METHODS: Seven patients with suspected superwarfarin intoxication who lacked any definitive history of rodenticide ingestion were included. Thirty-one patients initially diagnosed with rodenticide poisoning were also included. We performed a retrospective chart review of all subjects and examined clinical data including patient demographics and medical histories. RESULTS: Patients initially diagnosed with rodenticide poisoning were divided into two groups, one of which had a laboratory abnormality (prothrombin time [PT] > 13 seconds) and another group with PTs in the normal range. There was no significant difference between the two groups in any of age, gender, the extent of chronic alcohol consumption, the causative rodenticide, psychiatric problems, ingestion of drugs interacting with warfarin, the extent of intoxication, or the type of ingestion attempt. The albumin level of the former group was significantly lower than that of the latter group (p = 0.014). Furthermore, a significant difference between the two groups was evident in terms of simultaneous ingestion of rodenticide and alcohol (p = 0.023). CONCLUSIONS: Most patients with superwarfarin poisoning did not exhibit any complication. When such complications were evident, they were associated with serum albumin level and coingestion of rodenticide and alcohol.
4-Hydroxycoumarins/*poisoning ; Adolescent ; Adult ; Aged ; Aged, 80 and over ; Alcohol Drinking/adverse effects/blood ; Anticoagulants/*poisoning ; Blood Coagulation/*drug effects ; Child ; Child, Preschool ; Female ; Humans ; Male ; Middle Aged ; Partial Thromboplastin Time ; Prothrombin Time ; Republic of Korea ; Retrospective Studies ; Risk Factors ; Rodenticides/*poisoning ; Serum Albumin/metabolism ; Vitamin K/*blood ; Vitamin K Deficiency Bleeding/blood/*chemically induced/diagnosis/therapy ; Young Adult

BACKGROUND/AIMS: For many years, conventional treatment for multiple myeloma (MM) not ineligible for high-dose therapy has been the combination of oral melphalan and prednisone (MP). However, melphalan-based regimens are associated with numerous complications. Another alkylating agent, cyclophosphamide, has similar effects on MM and is associated with fewer reports of complications. Therefore, cyclophosphamide-based regimens have usually been used as salvage therapy in patients with refractory or relapsed MM, despite the development of newer drugs. The purpose of this report was to evaluate the efficacy and tolerability of cyclophosphamide and prednisone as a first-line therapy for MM. METHODS: For the period January 2002 to June 2012, we retrospectively analyzed 29 patients newly diagnosed with MM who underwent a treatment regimen consisting of intravenous cyclophosphamide (1,000 mg/kg) for 1 day and prednisone (100 mg) for 4 days. RESULTS: The rate of response to this regimen was 31.1 percent. The median progression-free survival (PFS) was 5.5 months and the median overall survival (OS) was 47.3 months. The regimen was well tolerated. Adverse effects of grades above III were as follows: anemia in seven patients (24.1%), neutropenia in five patients (17.2%), and thrombocytopenia in two patients (6.8%). These adverse effects were easily adjusted. No one developed a secondary malignancy or hemorrhagic cystitis. CONCLUSIONS: Although PFS was less than for the MP regimen, median OS was better than for the MP regimen. Furthermore, the cyclophosphamide-prednisone regimen was well tolerated, and the adverse effects that did occur were easily adjusted. The cyclophosphamide-prednisone combination regimen may represent an effective and well tolerated first-line therapy for non-transplant candidates with MM.
Anemia ; Antineoplastic Combined Chemotherapy Protocols ; Cisplatin ; Cyclophosphamide ; Disease-Free Survival ; Humans ; Melphalan ; Methotrexate ; Multiple Myeloma ; Neutropenia ; Prednisone ; Retrospective Studies ; Salvage Therapy ; Thrombocytopenia

Background: Cytoplasmic polyadenylation element binding (CPEB) proteins are sequencespecific RNA-binding proteins that control translation via cytoplasmic polyadenylation. We previously reported that CPEB1 or CPEB4 knockdown suppresses TAK1 and SMAD signaling in an in vitro study. Objective: This study aimed to investigate whether suppression of CPEB1 or CPEB4 expression inhibits scar formation in a mice model of acute dermal wound healing. Methods: CPEB1 and CPEB4 expression levels were suppressed by siRNA treatment. Skin wounds were created by pressure-induced ulcers in mice. Images of the wound healing were obtained using a digital camera and contraction was measured by ImageJ. mRNA and protein expression was analyzed using quantitative real time polymerase chain reaction and western blotting, respectively. Results: Wound contraction was significantly decreased by pre-treatment with CPEB1 or CPEB4 siRNA compared to the control. Suppression of CPEB1 or CPEB4 expression decreased TAK1 signaling by reducing the levels of TLR4 and TNF-α, phosphorylated TAK1, p38, ERK, JNK, and NF-κB-p65. Decreased levels of phosphorylated SMAD2 and SMAD3 indicated a reduction in SMAD signaling as well. Consequently, the expression of α-SMA, fibronectin, and type I collagen decreased. Conclusion CPEB1 siRNA or CPEB4 siRNA inhibit scar formation by modulating the TAK1 and SMAD signaling pathways. Our study highlights CPEB1 and CPEB4 as potential therapeutic targets for the treatment of scar formation.

BACKGROUND/AIMS: The dose of mycophenolate mofetil (MMF) has been reduced in Asia due to side effects associated with the conventional fixed dose of 2-3 g/day. We aimed to determine the pharmacokinetics of a reduced dose of MMF and to validate its feasibility in combination with tacrolimus in living-donor liver transplantation (LDLT). METHODS: Two sequential studies were performed in adult LDLT between October 2009 and 2011. First, we performed a prospective pharmacokinetic study in 15 recipients. We measured the area under the curve from 0 to 12 hours (AUC0-12) for mycophenolic acid at postoperative days 7 and 14, and we performed a protocol biopsy before discharge. Second, among 215 recipients, we reviewed 74 patients who were initially administered a reduced dose of MMF (1.0 g/day) with tacrolimus (trough, 8-12 ng/mL during the first month, and 5-8 ng/mL thereafter), with a 1-year follow-up. We performed protocol biopsies at 2 weeks and 1 year post-LDLT. RESULTS: In the first part of study, AUC0-12 was less than 30 mgh/L in 93.3% of cases. In the second, validating study, 41.9% of the recipients needed dose reduction or cessation due to side effects within the first year after LDLT. At 12 months post-LDLT, 17.6% of the recipients were administered a lower dose of MMF (0.5 g/day), and 16.2% needed permanent cessation due to side effects. The 1- and 12-month rejection-free survival rates were 98.6% and 97.3%, respectively. CONCLUSIONS: A reduced dose of MMF was associated with low blood levels compared to the existing recommended therapeutic range. However, reducing the dose of MMF combined with a low level of tacrolimus was feasible clinically, with an excellent short-term outcome in LDLT.
Adult ; Aged ; Area Under Curve ; Drug Therapy, Combination ; Female ; Follow-Up Studies ; Gastrointestinal Diseases/etiology ; Graft Rejection/prevention & control ; Humans ; Immunosuppressive Agents/blood/*pharmacokinetics ; Leukopenia/etiology ; Liver/pathology ; Liver Failure/*therapy ; *Liver Transplantation ; Male ; Middle Aged ; Mycophenolic Acid/adverse effects/*analogs & derivatives/blood/pharmacokinetics ; ROC Curve ; Retrospective Studies ; Tacrolimus/therapeutic use ; Tissue Donors

PURPOSE: To analyze the controversies surrounding therapeutic decision-making and the withholding of life- sustaining treatments, values held concerning therapeutic interventions of terminal cancer patients are compared between physicians and family members. MATERIALS AND METHODS: 42 advanced or terminal stage cancer patients were enrolled for the study. The questionnaires were administered to the duty doctor and the family of the patients. Questions included whether to use new agents with a 15% partial efficacy and whether to use opioid analgesics, intravenous nutrition, a feeding tube, antibiotics, and hemodialysis. Additionally, we asked about the administration of CPR, ventilator application, and euthanasia. If the family permitted, the same questionnaires were given to the patients. RESULTS: Of the 42 cases, 5 families refused to answer the questionnaire. Of the available 37 families, only 5 families permitted access to the patients. Of the 5 patients, 2 patients refused the questionnaire. Only 67.6% and 8.1% of families and the patients clearly understood the stage of cancer. The use of a new agent was accepted by 45.2% of the physicians and 45.9% of the families. The rankings of the acceptance of treatment in the physicians and in the families were similar. The concordance rate between the physicians and the families was lowest on ventilator application and CPR. 31% of the physicians and 43.2% of the families agreed on the issue of euthanasia. CONCLUSION: Values held on issues like therapeutic decision-making and the withholding of life-sustaining treatments in terminal cancer patients are discordant between physicians and family members. In order to resolve controversies on the role of physicians in end-of-life decisions, the values of physicians as well as patients and their family members should be considered in the final decision-making process.
Analgesics, Opioid ; Anti-Bacterial Agents ; Cardiopulmonary Resuscitation ; Euthanasia ; Humans ; Medical Futility ; Renal Dialysis ; Ventilators, Mechanical ; Withholding Treatment ; Surveys and Questionnaires

The heart disease during gestation complicates approximately 0.5-1.5% of pregnancies. The common cause of heart disease during gestation is acquired rheumatic valvular lesions and congenital heart defects. In contrast, infective endocarditis during pregnancy or the puerperium is quite rare, with fewer than 100 cases reported over the past 50 years. We present a case of bacterial endocarditis complicated by severe tricuspid valvular insufficiency and associated septic pulmonary emboli. Therapy consisted of cesarean section at 32 weeks gestation followed by tricuspid valvular replacement, removal of vegetation and primary closure of congenital ventricular septal defect.
Cesarean Section ; Endocarditis ; Endocarditis, Bacterial* ; Female ; Heart Defects, Congenital ; Heart Diseases ; Heart Septal Defects, Ventricular ; Postpartum Period ; Pregnancy*

To elucidate the mechanism of cyclic nucleotides, such as adenosine 3',5'-cyclic monophosphate (cAMP) and guanosine 3',5' -cyclic monophosphate (cGMP), in the regulation of human gastric motility, we examined the effects of forskolin (FSK), isoproterenol (ISO) and sodium nitroprusside (SNP) on the spontaneous, high K+ and acetylcholine (ACh)-induced contractions of corporal circular smooth muscle in human stomach. Gastric circular smooth muscle showed regular spontaneous contraction, and FSK, ISO and SNP inhibited its phasic contraction and basal tone in a concentration-dependent manner. High K+ (50 mM) produced sustained tonic contraction, and ACh (10 micrometer) produced initial transient contraction followed by later sustained tonic contraction with superimposed phasic contractions. FSK, ISO and SNP inhibited high K+-induced tonic contraction and also ACh-induced phasic and tonic contraction in a reversible manner. Nifedipine (1 micrometer), inhibitor of voltage-dependent L-type calcium current (VDCC(L)), almost abolished ACh-induced phasic contractions. These findings suggest that FSK, ISO and SNP, which are known cyclic nucleotide stimulators, inhibit smooth muscle contraction in human stomach partly via inhibition of VDCCL.
Acetylcholine ; Adenosine ; Calcium ; Contracts ; Forskolin ; Guanosine ; Humans ; Isoproterenol ; Muscle, Smooth ; Nifedipine ; Nitroprusside ; Nucleotides, Cyclic ; Relaxation ; Stomach

We elucidated the distribution of interstitial cells of Cajal (ICC) in human stomach, using cryosection and c-Kit immunohistochemistry to identify c-Kit positive ICC. Before c-Kit staining, we routinely used hematoxylin and eosin (HE) staining to identify every structure of human stomach, from mucosa to longitudinal muscle. HE staining revealed that the fundus greater curvature (GC) had prominent oblique muscle layer, and c-Kit immunostaining c-Kit positive ICC cells were found to have typical morphology of dense fusiform cell body with multiple processes protruding from the central cell body. In particular, we could observe dense processes and ramifications of ICC in myenteric area and longitudinal muscle layer of corpus GC. Interestingly, c-Kit positive ICC-like cells which had morphology very similar to ICC were found in gastric mucosa. We could not find any significant difference in the distribution of ICC between fundus and corpus, except for submucosa where the density of ICC was much higher in gastric fundus than corpus. Furthermore, there was no significant difference in the density of ICC between each area of fundus and corpus, except for muscularis mucosa. Finally, we also found similar distribution of ICC in normal and cancerous tissue obtained from a patient who underwent pancreotomy and gastrectomy. In conclusion, ICC was found ubiquitously in human stomach and the density of ICC was significantly lower in the muscularis mucosa of both fundus/corpus and higher in the submucosa of gastric fundus than corpus.
Eosine Yellowish-(YS) ; Gastrectomy ; Gastric Fundus ; Gastric Mucosa ; Hematoxylin ; Humans ; Immunohistochemistry ; Interstitial Cells of Cajal ; Mucous Membrane ; Muscles ; Stomach