Kimura's disease is a chronic inflammatory soft tissue disorder with peripheral eosinophila and high serum IgE. It might be associated with nephrotic syndrome. We report a 6-year-old boy with Kimura's disease and the concurrent steroid dependant minimal change nephrotic syndrome. With levamisole treatment fot 24 months, tumor progression and relapse of the associated nephrotic syndrome were not observed.
Child ; Humans ; Immunoglobulin E ; Levamisole ; Nephrosis, Lipoid ; Nephrotic Syndrome ; Recurrence

Ketosis-prone diabetes includes heterogeneous disease groups characterized by provoked or unprovoked ketoacidosis (ketosis), with a typical phenotype of autoimmune type 1 diabetes. As the incidence of obesity and type 2 diabetes is universally increasing, the rate of type 2 diabetes in diabetic ketoacidosis in children and adolescents is exptected to increase rapidly. The clinical presentation of atypical ketoacidosis with type 2 diabetes has been reported mostly in adults. We recently experienced a case of a 10-year-old obese girl with new-onset type 2 diabetes who initially presented with severe diabetic ketoacidosis, and introduce it with literature reviews.
Adolescent ; Adult ; Child ; Diabetes Mellitus, Type 2 ; Diabetic Ketoacidosis ; Humans ; Incidence ; Ketosis ; Obesity ; Phenotype

Ketosis-prone diabetes includes heterogeneous disease groups characterized by provoked or unprovoked ketoacidosis (ketosis), with a typical phenotype of autoimmune type 1 diabetes. As the incidence of obesity and type 2 diabetes is universally increasing, the rate of type 2 diabetes in diabetic ketoacidosis in children and adolescents is exptected to increase rapidly. The clinical presentation of atypical ketoacidosis with type 2 diabetes has been reported mostly in adults. We recently experienced a case of a 10-year-old obese girl with new-onset type 2 diabetes who initially presented with severe diabetic ketoacidosis, and introduce it with literature reviews.
Adolescent ; Adult ; Child ; Diabetes Mellitus, Type 2 ; Diabetic Ketoacidosis ; Humans ; Incidence ; Ketosis ; Obesity ; Phenotype

OBJECTIVE: To compare the T1-weighted spectral presaturation with inversion-recovery sequences (T1 SPIR) with T2-weighted turbo spin-echo sequences (T2 TSE) on 3T magnetic resonance arthrography (MRA) in the evaluation of the subscapularis (SSC) tendon tear with arthroscopic findings as the reference standard. MATERIALS AND METHODS: This retrospective study included 120 consecutive patients who had undergone MRA within 3 months between April and December 2015. Two musculoskeletal radiologists blinded to the arthroscopic results evaluated T1 SPIR and T2 TSE images in separate sessions for the integrity of the SSC tendon, examining normal/articular-surface partial-thickness tear (PTTa)/full-thickness tear (FTT). Diagnostic performance of T1 SPIR and T2 TSE was calculated with arthroscopic results as the reference standard, and sensitivity, specificity, and accuracy were compared using the McNemar test. Interobserver agreement was measured with kappa (κ) statistics. RESULTS: There were 74 SSC tendon tears (36 PTTa and 38 FTT) confirmed by arthroscopy. Significant differences were found in the sensitivity and accuracy between T1 SPIR and T2 TSE using the McNemar test, with respective rates of 95.9–94.6% vs. 71.6–75.7% and 90.8–91.7% vs. 79.2–83.3% for detecting tear; 55.3% vs. 31.6–34.2% and 85.8% vs. 78.3–79.2%, respectively, for FTT; and 91.7–97.2% vs. 58.3–61.1% and 89% vs. 78–79.3%, respectively, for PTTa. Interobserver agreement for T1 SPIR was almost perfect for T1 SPIR (κ = 0.839) and substantial for T2 TSE (κ = 0.769). CONCLUSION: T1-weighted spectral presaturation with inversion-recovery sequences is more sensitive and accurate compared to T2 TSE in detecting SSC tendon tear on 3T MRA.
Arthrography* ; Arthroscopy ; Humans ; Magnetic Resonance Imaging ; Retrospective Studies ; Sensitivity and Specificity ; Tears* ; Tendons*

OBJECTIVES: To design a cloud computing-based Healthcare Software-as-a-Service (SaaS) Platform (HSP) for delivering healthcare information services with low cost, high clinical value, and high usability. METHODS: We analyzed the architecture requirements of an HSP, including the interface, business services, cloud SaaS, quality attributes, privacy and security, and multi-lingual capacity. For cloud-based SaaS services, we focused on Clinical Decision Service (CDS) content services, basic functional services, and mobile services. Microsoft's Azure cloud computing for Infrastructure-as-a-Service (IaaS) and Platform-as-a-Service (PaaS) was used. RESULTS: The functional and software views of an HSP were designed in a layered architecture. External systems can be interfaced with the HSP using SOAP and REST/JSON. The multi-tenancy model of the HSP was designed as a shared database, with a separate schema for each tenant through a single application, although healthcare data can be physically located on a cloud or in a hospital, depending on regulations. The CDS services were categorized into rule-based services for medications, alert registration services, and knowledge services. CONCLUSIONS: We expect that cloud-based HSPs will allow small and mid-sized hospitals, in addition to large-sized hospitals, to adopt information infrastructures and health information technology with low system operation and maintenance costs.
Commerce ; Computer Systems ; Decision Support Systems, Clinical ; Delivery of Health Care* ; Electronic Health Records ; Information Services ; Medical Informatics ; Medical Order Entry Systems ; Privacy ; Soaps ; Social Control, Formal

PURPOSE: Perigraft seroma and excessive fluid leaks are well known complications after the use of expanded polytetrafluoroethylene(ePTFE, Gore-Tex(R)) graft. The purpose of this study is to evaluate the effectiveness of precoating the grafts with fibrin glue in the patients underwent a modified Blalock-Taussig shunt. METHODS: Among 51 consecutive infants underwent modified Blalock-Taussig shunt between June 2000 and August 2003, 49 patients underwent 53 shunt procedures through thoracotomy were included for analysis. Their median age was 0.9 months(2 days-5.9 months), median body weight was 3.5(2.2-7.9) kg. The ePTFE grafts precoated with fibrin glue were used in 20 procedures. Perioperative variables of the precoated group were compared with those of a control group(n=33) using t-test and Fisher's exact test. RESULTS: There was no operative mortality in the both groups. No significant difference between the groups could be found in the preoperative variables, postoperative morbidity, and the incidence of perigraft seroma, which developed only in 1 case in the control group. The size of the grafts was significantly smaller in the precoated group(P<0.01). The total amount of pleural drainage per body weight of the precoated group was significantly less than that of the control group(19.7+/-25.2 versus 5.5+/-4.9 mL/kg; P<0.01). CONCLUSION: Precoating an ePTFE graft with fibrin glue may reduce perigraft leak after a modified Blalock-Taussig shunt, although the impact of the size of the graft used and the native pulmonary artery requires further investigation.
Blalock-Taussig Procedure ; Blood Vessel Prosthesis ; Body Weight ; Drainage ; Endoleak* ; Fibrin Tissue Adhesive ; Humans ; Incidence ; Infant ; Mortality ; Polytetrafluoroethylene* ; Postoperative Complications ; Pulmonary Artery ; Seroma ; Thoracotomy ; Tissue Adhesives ; Transplants

Despite efforts to treat critically ill patients who require continuous renal replacement therapy (CRRT) due to acute kidney injury (AKI), their mortality risk remains high. This condition may be attributable to complications of CRRT, such as arrhythmias. Here, we addressed the occurrence of ventricular tachycardia (VT) during CRRT and its relationship with patient outcomes. Methods: This study retrospectively enrolled 2,397 patients who started CRRT due to AKI from 2010 to 2020 at Seoul National University Hospital in Korea. The occurrence of VT was evaluated from the initiation of CRRT until weaning from CRRT. The odds ratios (ORs) of mortality outcomes were measured using logistic regression models after adjustment for multiple variables. Results: VT occurred in 150 patients (6.3%) after starting CRRT. Among them, 95 cases were defined as sustained VT (i.e., lasting ≥30 seconds), and the other 55 cases were defined as non-sustained VT (i.e., lasting <30 seconds). The occurrence of sustained VT was associated with a higher mortality rate than a nonoccurrence (OR, 2.04 and 95% confidence interval [CI], 1.23–3.39 for the 30- day mortality; OR, 4.06 and 95% CI, 2.04–8.08 for the 90-day mortality). The mortality risk did not differ between patients with non-sustained VT and nonoccurrence. A history of myocardial infarction, vasopressor use, and certain trends of blood laboratory findings (such as acidosis and hyperkalemia) were associated with the subsequent risk of sustained VT. Conclusion: Sustained VT occurrence after starting CRRT is associated with increased patient mortality. The monitoring of electrolytes and acid-base status during CRRT is essential because of its relationship with the risk of VT.