Here we report the case of a 20-year-old female patient previously diagnosed with Hashimoto's thyroiditis and overt hypothyroidism, and who had been taking synthetic thyroxine (100micro/day) for eight months. She experienced intermittent dizziness and generalized weakness, and was diagnosed as having severe autoimmune hemolytic anemia (AIHA). We prescribed prednisolone treatment and continued synthetic thyroxine administration. Two years and five months later, she developed idiopathic thrombocytopenic purpura (ITP) and was diagnosed with Evans' syndrome. Thereafter, laparoscopic splenectomy was performed because her autoimmune hemolytic anemia was refractory and dependent on steroid therapy. The HLA genotypes of the patient were HLA-A*020101/A*2602, HLA-B*270502/B*5401, HLA-Cw*0102/Cw*020202, HLA-DRB1*0404/DRB1*0405, and HLA-DQB1*0302/DQ B1*0401. Hashimoto's thyroiditis is often associated with other nonendocrine autoimmune diseases, and antithyroid antibodies are frequently observed in Evans' syndrome (coexistence of AIHA and ITP). However, there is no report of Evans' syndrome developing in patients with overt hypothyroidism and Hashimoto's thyroiditis. This case suggests that three autoimmune diseases (AIHA, ITP, and Hashimoto's thyroiditis) might share a common immunogenetic pathway in pathogenesis.
Purpura, Thrombocytopenic/blood/*complications ; Humans ; Hashimoto Disease/*complications/radionuclide imaging ; Female ; Anemia, Hemolytic, Autoimmune/blood/*complications ; Adult

OBJECTIVES: This study was investigated the recovery characteristics of propofol-remifentanil and sevoflurane-remifentanil anesthesia for total thyroidectomy. METHODS: Eighty patients in ASA physical status 1 and 2 scheduled for total thyroidectomy were allocated randomly to either group P (n = 40) or group S (n = 40). Anesthesia was maintained with remifentanil effect site concentration (Ce) 1-3 ng/mL and propofol Ce 2-4 microg/mL in the group P, and was maintained with remifentanil Ce 1-3 ng/mL and sevoflurane 1.5-2% in the group S. Blood pressure, heart rate, and bispectral (BIS) index were measured during perioperative period. The times from discontinuance of anesthetic agent to eye opening, to extubation, and to stating name were measured. Postoperative complications were evaluated. RESULTS: There were no significant differences between group P and S on the blood pressure, heart rate, and recovery time. BIS index of group P showed lower than that of group S during operation (P < 0.05). The incidences of side effects were similar in the two groups, though the incidence of nausea was higher in the group S (P < 0.05). CONCLUSIONS: Propofol-remifentanil anesthesia was more advantageous than sevoflurane-remifentanil anesthesia for thyroidectomy in view of side effect incidences.
Anesthesia* ; Blood Pressure ; Heart Rate ; Humans ; Incidence ; Nausea ; Perioperative Period ; Postoperative Complications ; Propofol ; Thyroidectomy*

Hyperglycemic hyperosmolar state (HHS) is an acute complication mostly occurring in elderly type 2 diabetes mellitus (DM). Thyrotoxicosis causes dramatic increase of glycogen degradation and/or gluconeogenesis and enhances breakdown of triglyc-erides. Thus, in general, it augments glucose intolerance in diabetic patients. A 23-yr-old female patient with Graves' disease and type 2 DM, complying with methimazole and insulin injection, had symptoms of nausea, polyuria and generalized weakness. Her serum glucose and osmolarity were 32.7 mM/L, and 321 mosm/kg, respectively. Thyroid function tests revealed that she had more aggravated hyperthyroid status; 0.01 mU/L TSH and 2.78 pM/L free T3 (reference range, 0.17-4.05, 0.31-0.62, respectively) than when she was discharged two weeks before (0.12 mU/L TSH and 1.41 pM/L free T3). Being diagnosed as HHS and refractory Graves' hyperthyroidism, she was treated successfully with intravenous fluids, insulin and high doses of methimazole (90 mg daily). Here, we described the case of a woman with Graves' disease and type 2 DM developing to HHS.
Thyroid Function Tests ; Methimazole/therapeutic use ; Insulin/therapeutic use ; Hyperthyroidism/*complications/therapy ; Hyperglycemic Hyperosmolar Nonketotic Coma/*etiology ; Humans ; Graves Disease/*complications ; Fluid Therapy ; Female ; Diabetes Mellitus, Type 2/*complications ; Adult

Osteoclasts originated from hematopoietic stem cells are multi-nucleated cells that can resorb the bone matrix. Receptor activator of nuclear factor kappa-B (RANK)/RANK ligand (RANKL) signaling pathway is crucial for the differentiation and activation of osteoclasts. In this study, we investigated for the first time whether or not RANKL induced mitogen- and stress-activated kinase 1 (MSK1) phosphorylation at Ser 376. Activation of MSK1 was detected as soon as 5 min after RANKL stimulation and sparsely detected at 30 min after stimulation. RANKL-induced MSK1 phosphorylation occurred in a dose-dependent manner. MSK1 is known as a downstream signaling molecule of cAMP-dependent protein kinase (PKA). Treatment with the PKA inhibitor H89 significantly suppressed c-Fos and nuclear factor of activated T-cells, cytoplasmic 1 (NFATc1) induction upon RANKL stimulation. In addition, cAMP response element-binding protein (CREB) phosphorylation was extremely inhibited by H89 treatment. Mitogen-activated protein kinases (MAPKs) have been investigated for induction of MSK1 phosphorylation. Specific signaling pathway inhibitors for p38 and extracellular signal-regulated kinases (ERKs) significantly blocked RANKL-induced MSK1 activation. Finally, as a downstream effector of the p38-MSK1 pathway, c-Fos transcriptional activity was determined. RANKL-mediated elevation of c-Fos transcriptional activity was significantly suppressed by p38 inhibitor. Moreover, a dominant negative form of CREB suppressed activation of NFATc1. In conclusion, RANKL-stimulated MSK1 phosphorylation could play a role in induction of NFATc1 through CREB and c-Fos activation as a downstream molecule of p38, ERK MAPKs, and PKA. Our results support basic information for the development of osteoclast specific inhibitors.
Bone Matrix ; Cyclic AMP Response Element-Binding Protein ; Cyclic AMP-Dependent Protein Kinases ; Extracellular Signal-Regulated MAP Kinases ; Hematopoietic Stem Cells ; Mitogen-Activated Protein Kinases ; NFATC Transcription Factors ; Osteoclasts ; Phosphorylation ; Phosphotransferases

BACKGROUND/AIMS: During endoscopic retrograde cholangiopancreatography (ERCP), all efforts should be made to be aware of radiation hazards and to reduce radiation exposure. The aim of this study was to investigate the status of radiation protective equipment and the awareness of radiation exposure in health care providers performing ERCP in Korean hospitals. METHODS: A survey with a total of 42 questions was sent to each respondent via mail or e-mail between October 2010 and March 2011. The survey targeted nurses and radiation technicians who participated in ERCP in secondary or tertiary referral centers. RESULTS: A total of 78 providers from 38 hospitals responded to the surveys (response rate, 52%). The preparation and actual utilization rates of protective equipment were 55.3% and 61.9% for lead shields, 100% and 98.7% for lead aprons, 47.4% and 37.8% for lead glasses, 97.4% and 94.7% for thyroid shields, and 57.7% and 68.9% for radiation dosimeters, respectively. The common reason for not wearing protective equipment was that the equipment was bothersome, according to 45.7% of the respondents. CONCLUSIONS: More protective equipment, such as lead shields and lead glasses, should be provided to health care providers involved in ERCP. In particular, the actual utilization rate for lead glasses was very low.
Cholangiopancreatography, Endoscopic Retrograde ; Data Collection ; Delivery of Health Care ; Electronic Mail ; Eyeglasses ; Glass ; Health Personnel ; Humans ; Korea ; Postal Service ; Referral and Consultation ; Thyroid Gland

Purpose: Febrile neutropenia (FN) can cause suboptimal treatment and treatment-related mortality (TRM) in diffuse large B-cell lymphoma (DLBCL) patients treated with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone (R-CHOP). Materials and methods: We conducted a prospective cohort study to evaluate the effectiveness of pegfilgrastim prophylaxis in DLBCL patients receiving R-CHOP, and we compared them with the PROCESS cohort (n=485). Results: Since January 2015, 986 patients with DLBCL were enrolled. Pegfilgrastim was administered at least once in 930 patients (94.3%), covering 90.3% of all cycles. FN developed in 137 patients (13.9%) in this cohort (23.7% in the PROCESS cohort, p<0.001), and 4.2% of all cycles (10.2% in the PROCESS cohort, p<0.001). Dose delay was less common (≥3 days: 18.1% vs. 23.7%, p=0.015; ≥5 days: 12.0% vs. 18.3%, p=0.023) in this cohort than in the PROCESS cohort. The incidence of TRM (3.2% vs. 5.6%, p=0.047) and infection-related death (1.8% vs. 4.5%, p=0.004) was lower in this cohort than in the PROCESS cohort. The 4-year overall survival (OS) and progression-free survival (PFS) rates of the two cohorts were not different (OS: 73.0% vs. 71.9%, p=0.545; PFS: 69.5% vs. 68.8%, p=0.616). However, in patients aged ≥75 years, the 4-year OS and PFS rates were higher in this cohort than in the PROCESS cohort (OS: 49.6% vs. 33.7%, p=0.032; PFS: 44.2% vs. 30.3% p=0.047). Conclusion Pegfilgrastim prophylaxis is effective in the prevention of FN and infection-related death in DLBCL patients receiving R-CHOP, and it also improves OS in patients aged ≥75 years.

Purpose: Diffuse large B-cell lymphoma (DLBCL) is the most common hematologic malignancy worldwide. Although substantial improvement has been achieved by the frontline rituximab-based chemoimmunotherapy, up to 40%-50% of patients will eventually have relapsed or refractory disease, whose prognosis is extremely dismal. Materials and Methods: We have carried out two prospective cohort studies that include over 1,500 DLBCL patients treated with rituximab plus CHOP (#NCT01202448 and #NCT02474550). In the current report, we describe the outcomes of refractory DLBCL patients. Patients were defined to have refractory DLBCL if they met one of the followings, not achieving at least partial response after 4 or more cycles of R-CHOP; not achieving at least partial response after 2 or more cycles of salvage therapy; progressive disease within 12 months after autologous stem cell transplantation. Results: Among 1,581 patients, a total of 260 patients met the criteria for the refractory disease after a median time to progression of 9.1 months. The objective response rate of salvage treatment was 26.4%, and the complete response rate was 9.6%. The median overall survival (OS) was 7.5 months (95% confidence interval, 6.4 to 8.6), and the 2-year survival rate was 22.1%±2.8%. The median OS for each refractory category was not significantly different (p=0.529). Conclusion In line with the previous studies, the outcomes of refractory DLBCL patients were extremely poor, which necessitates novel approaches for this population.