OBJECTIVE: Previous classification systems for juvenile idiopathic arthritis (JIA) were based on the number of joints involved and did not categorize homogenous disease entities. Therefore, JIA patients were reclassified retrospectively by applying rheumatoid factor (RF) and antinuclear antibody (ANA), which have been proven to constitute a homogenous disease entity. METHODS: The medical records of JIA patients were investigated retrospectively and reclassified into six categories using the new provisional classification. The nomenclature was based on Dr. Martini's proposal in the 23rd European Paediatric Rheumatology Congress (2016) at Genoa, Italy. New categories included systemic JIA (sJIA), RF-positive JIA (RF-JIA), early-onset ANA-positive JIA (eoANA-JIA), enthesitis/spondylitis-related JIA (ESR-JIA), “other JIA”, and “unclassified JIA”. RESULTS: Of a total of 262 JIA patients, 71 (27.1%) were reclassified as sJIA, 31 (11.8%) as RF-JIA, 22 (8.4%) as eoANA-JIA, 63 (24.0%) as ESR-JIA, 65 (24.8%) as “other JIA”, and 10 (3.8%) as “unclassified JIA”. A comparison of RF-JIA, eoANA-JIA, and ESR-JIA revealed significant differences in the gender ratio, age of disease onset, and the cumulative number and type of joints involved among the three groups. “Other JIA” comprised a significant proportion (24.8%) and warrants the need for further classification. The characteristics of the RF-positive patients were comparable to those of the anti-cyclic citrullinated peptide antibody-positive patients. The ANA positivity was lower (28.2%) than that in Western studies but showed similar clinical features. CONCLUSION: This is the first study applying RF and ANA to classify JIA without considering the joint counts. The six new categories include sJIA, RF-JIA, eoANA-JIA, ESR-JIA, “other JIA,” and “unclassified JIA”.
Antibodies, Antinuclear ; Arthritis, Juvenile ; Classification ; Humans ; Italy ; Joints ; Medical Records ; Retrospective Studies ; Rheumatoid Factor ; Rheumatology

OBJECTIVE: Our aim was to investigate the long term safety and efficacy of etanercept in children with juvenile idiopathic arthritis (JIA). METHODS: The study subjects were the 90 JIA patients treated with etanercept in the Department of Pediatrics, Hallym University Medical Center between January 2004 and December 2017. We retrospectively reviewed their medical records for age at diagnosis, duration of etanercept treatment, number of active joints, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and adverse events during treatment. RESULTS: Among the 90 patients, 38 (42.0%) were male and 52 (58.0%) were female; 15 (16.7%) had systemic onset, 41 (45.6%) had extended oligoarticular, 14 (15.6%) had rheumatoid factor-positive polyarticular, 18 (20.0%) had rheumatoid factor-negative polyarticular, and 2 (2.1%) had enthesitis-related arthritis. The median age at the start of etanercept treatment was 9 years (range, 3~18 years), and the median duration of etanercept treatment was 6 years (range, 0.5~13 years). The median number of active joints decreased from 9 to 0 after 6 months of etanercept treatment. The median CRP and ESR were within normal range after 3 months of treatment. Six patients experienced recurrence, 9 switched to other medications and 3 discontinued etanercept. Of the 14 reported adverse events, 1 was serious, and there were no tuberculosis infections or malignancies. CONCLUSION: Long-term treatment with etanercept is efficacious and safe for children with JIA. However, those with the systemic onset subtype appear to have low drug survival rate compared to those with other types of JIA.
Academic Medical Centers ; Arthritis ; Arthritis, Juvenile ; Blood Sedimentation ; C-Reactive Protein ; Child ; Diagnosis ; Etanercept ; Female ; Humans ; Joints ; Male ; Medical Records ; Pediatrics ; Recurrence ; Reference Values ; Retrospective Studies ; Survival Rate ; Tuberculosis

No abstract available.
Kidney Transplantation* ; Metabolism*

Atrial fibrillation (AF), the most common chronic arrhythmia in adults, is rarely reported in children. Moreover, most of the previously reported children with AF have comorbidities, such as structural heart diseases, rheumatic diseases, and thyroid diseases. This case report is about a healthy 9-year-old boy who was diagnosed with AF without cardiac anomaly. He visited the emergency department with chest pain and palpitation, lasting 2 hours. His electrocardiogram showed narrow-complex tachycardia, which led to the diagnosis of supraventricular tachycardia. The administration of adenosine revealed rapid irregular P waves. After electrical cardioversion, cardiac rhythm was converted to normal sinus rhythm. This case report suggests that when children with narrow-complex tachycardia visit the emergency department, the possibility of AF, in addition to supraventricular tachycardia, should be considered if the RR intervals are markedly irregular.
Adenosine ; Adult ; Arrhythmias, Cardiac ; Atrial Fibrillation ; Cardiovascular Diseases ; Chest Pain ; Child ; Comorbidity ; Diagnosis ; Electric Countershock ; Electrocardiography ; Emergency Service, Hospital ; Humans ; Male ; Pediatrics ; Rheumatic Heart Disease ; Tachycardia ; Tachycardia, Supraventricular ; Thyroid Diseases

The explanation of isolated-organ tuberculosis rests on the assumption that in the course of the lymphatic or hematogenous dissemination of bacilli, organisms may be rapidly destroyed in all other sites save for the particular tissue involved in the isolated tuberculous process. Tuberculosis can arise in all tissues having lymphatics or blood supply, but the disease causing biliary tract obstruction has been known to be rare. Recently, we experi-enced a case of isolated-organ tuberculosis causing common bile duct obstruction and periductal lymph node enlargement in a 46-year-old Korean male. An ultrasonography-guided percutaneous needle biopsy revealed a granulomatous inflammation of the lymph node. After 7 months of anti-tuberculous medication, the common bile duct obstruction and periductal lymph node enlargement disappeared completely in a follow up abdominal CT and ERCP.
Biliary Tract ; Biopsy, Needle ; Cholangiopancreatography, Endoscopic Retrograde ; Cholestasis ; Common Bile Duct* ; Follow-Up Studies ; Humans ; Inflammation ; Lymph Nodes ; Lymphadenitis* ; Male ; Middle Aged ; Tomography, X-Ray Computed ; Tuberculosis*

BACKGROUND AND OBJECTIVES: The effect of electrical stimulation (ES) on vestibular compensation was investigated for 28 days after unilateral labyrinthectomy (Lx) in 24 rats. MATERIALS AND METHOD: The rats were divided into 4 groups and were treated as follows: A) Lx only, B) Lx with ES (0.6 mA) for 2 days, C) Lx with ES (0.6 mA) for 7 days, D) Lx with ES (1.2 mA) for 2 days. ES with pulse wave (10 Hz) was applied to temporal portion bilaterally (8 hr/day). Cathodal currents were transmitted to the electrode on the destructive side, anodal to that on the intact side. Postoperatively, spontaneous nystagmus (SN) was recorded with the video camera and yaw and roll head tilt (YHT, RHT) were measured using photo images. Horizontal vestibulo-ocular reflex (VOR) was evaluated at various frequencies (0.05, 0.1, 0.5, 1, 2, 3 Hz with peak angular velocity of 40degrees/sec) with the magnetic search coil system. Locomotor movement was monitored with a video camera. RESULTS: The ES groups showed faster compensation in SN, YHT, and RHT than the Lx group. At 3 days after Lx, the ES groups showed significant improvement in the gain over the whole frequency and asymmetry at low frequency compared to that of the Lx group. But the ES group had no effect on compensation of gain and asymmetry on and after 7 days of post-labyrinthectomy. There was no significant difference in vestibular compensation with respect to duration and intensity of ES. ES had no significant effect on the mean velocity and mean deviation of locomotor movements. CONCLUSION: These results suggest that ES has a favorable effect on the suppression of early static symptoms but has no effect on the compensation of dynamic symptoms after the recovery of resting discharge of the vestibular nuclei neurons.
Animals ; Compensation and Redress* ; Ear, Inner ; Electric Stimulation* ; Electrodes ; Head ; Neurons ; Rats* ; Reflex, Vestibulo-Ocular ; Vestibular Nuclei

Background: This is the first clinical study conducted among Asian women using breast implants manufactured by an Asian company. Four-year data regarding the safety and efficacy of BellaGel breast implants have already been published, and we now report 6-year data. Methods: This study was designed to take place over 10 years. It included 103 patients who underwent breast reconstruction or augmentation using BellaGel breast implants. The rates of implant rupture and capsular contracture were measured and analyzed to evaluate the effectiveness of the breast implant. Results: At patients’ 6-year postoperative visits, the implant rupture and capsular contracture rates were 1.15% and 2.30%, respectively. The implant rupture rate was 3.77% among reconstruction cases and 0% among augmentation cases. The capsular contracture rate was 5.66% among reconstruction cases and 0.83% among augmentation cases. Conclusions The 6-year data from this planned 10-year study suggest that the BellaGel cohesive silicone gel-filled breast implant is an effective and safe medical device that can be used in breast reconstruction and augmentation.

BACKGROUND: To compare the mobilizing effects and toxicities of two different doses of cyclophosphamide (CY) plus lenograstim (glycosylated G-CSF), we performed a prospective randomized study by enrolling patients suffering with either high-risk Non-Hodgkin's lymphoma (NHL) or breast cancer undergoing ablative chemotherapy. METHODS: The NHL patients received 4 cycles of CHOP and the breast cancer patients received 2-3 cycles of FAC (FEC) adjuvant chemotherapy. Then, the patients were randomly allocated to receive CY 4 g/m2 (arm A) or 1.5 g/m2 (arm B) in combination with lenograstim. Large volume leukapheresis was carried out and it was continued daily until the target cell dose of 2x10 (6) CD34+ cell/kg was reached. RESULTS: Twenty-seven patients were enrolled in the study. The median number of leukaphereis sessions actually performed was 2.5 sessions in arm A and 3 sessions in arm B. The target cell dose was obtained with the median number of one leukapheresis session in both arms of the study (p=0.09). The collected number of CD34+ cells in the leukapheresis products was higher in arm A than arm B (22.4 vs. 9.9x10 (6) /kg, respectively, p=0.05). Grade III or IV leukopenia was present in 14/15 patients (94%) in arm A and in 1/12 patients (8%) in arm B (p< 0.0001). Grade III or IV thrombocytopenia was present in 8/15 patients (54%) in arm A, but this was not present in any patients of arm B (p=0.0004). Neutropenic fever occurred in 6/15 patients (40%) in arm A, and in 1/12 patients (8%) in arm B (p=0.09). The hematological recovery of the leukocytes and platelets after transplantation was not statistically different between the two doses. CONCLUSION: Low-dose CY plus lenograstim is a safe and effective mobilizing regimen.
Transplantation Conditioning ; Stem Cells/*drug effects ; Recombinant Proteins/administration & dosage/pharmacology ; Prospective Studies ; Myeloablative Agonists/*administration & dosage/pharmacology ; Middle Aged ; Male ; Lymphoma, Non-Hodgkin/*drug therapy ; Leukapheresis ; Humans ; *Hematopoietic Stem Cell Mobilization ; Granulocyte Colony-Stimulating Factor/*administration & dosage/pharmacology ; Female ; Drug Therapy, Combination ; Cyclophosphamide/*administration & dosage/pharmacology ; Chemotherapy, Adjuvant ; Breast Neoplasms/*drug therapy ; Adult

We conducted a phase II multicenter trial to estimate the response and survival of patients with newly diagnosed multiple myeloma to high dose melphalan therapy followed by autologous peripheral blood stem cell transplantation. Eligible patients who had undergone induction with vincristine, adriamycin and dexamethasone (VAD) should have adequate cardiac, pulmonary and renal function (creatinine <2 mg/dL). Melphalan at 200 mg/m2 was used as a conditioning regimen. Eighty patients were enrolled from 13 centers. The median age of the patients was 53 yr (range; 20 to 68 yr). The initial stage was IA/IIA/IIB/IIIA/IIIB in 3/8/1/54/14 patients, respectively. Beta2-microglobulin, CRP and LDH were increased in 74, 42 and 34% of the patients examined. Cytogenetic data were available in 30 patients, and 6 patients showed numeric or structural abnormalities. Two therapy-related mortalities occurred from infection. Among the 78 evaluable patients, CR/PR/MR/NC/PD were achieved in 48/26/2/1/1patients, respectively. After a median follow-up of 30 months, the median overall and event-free survivals were 66 months (95% CI: 20-112) and 24 months (95% CI: 18-29), respectively. This study verifies the efficacy and feasibility of high dose melphalan therapy with autologous stem cell transplantation in newly diagnosed multiple myeloma.
Adult ; Aged ; Antigens, CD34/biosynthesis ; Antineoplastic Agents, Alkylating/*therapeutic use ; C-Reactive Protein/biosynthesis ; Cell Survival ; Combined Modality Therapy ; Cytogenetics ; Disease-Free Survival ; Female ; Human ; Korea ; L-Lactate Dehydrogenase/biosynthesis ; Male ; Melphalan/*therapeutic use ; Middle Aged ; Multiple Myeloma/*therapy ; Peripheral Blood Stem Cell Transplantation/*methods ; Time Factors ; Transplantation, Autologous/*methods ; beta 2-Microglobulin/blood

BACKGROUND: Essential thrombocythemia (ET) has a chronic course, but its main clinical features are thrombosis and hemorrhage. We evaluated the clinical features, including the vascular complications in patients with ET, during the disease courses and we determined the predictable risk factors for major vascular complications. METHODS: From 1991 to 2004, the medical records for 69 patients with ET were retrospectively reviewed for evaluating the clinical features, including the vascular complications, and the predictable risk factors for major vascular complications were analyzed. RESULTS: Major vascular thrombotic and hemorrhagic complications were observed in 16 patients (23.2%) and 6 patients (8.7%) at the time of diagnosis, and in 13 (18.8%) and 9 patients (13.0%) during follow-up. The incidence of major vascular thromboses in the older group (age >60 years) was higher than that in the younger group (< or =60 years) (34.2% vs 9.7%, respectively, P=0.016) at the time of diagnosis. During follow-up, the major vascular thrombosis risk was increased in patients with a previous thrombosis history (37.5% vs 13.2%, respectively, P=0.029) and in patients with 2 or more combined cardiovascular risk factors (44.4% vs 15.0%, respectively, P=0.035). The probability of 10-year survival in patients with thrombo-hemorrhagic complications during the disease course was lower than that in patients without complication (60.5% vs 93.7%, respectively, P=0.046). CONCLUSION: Advanced age, a previous thrombosis history and the combined cardiovascular risk factors were the risk factors for major vascular thrombosis in patients with ET. Prevention of thrombo-hemorrhagic complications is the most important therapeutic goal. Treatment strategies according to risk factors ought to be prospectively investigated.
Diagnosis ; Follow-Up Studies ; Hemorrhage ; Humans ; Incidence ; Medical Records ; Retrospective Studies ; Risk Factors ; Thrombocythemia, Essential* ; Thrombosis