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BackgroundJoubert syndrome and mitochondrial disease are rare congenital diseases in which a wide range of symptoms affects multiple organs. Patients with these diseases present characteristic symptoms related to the musculoskeletal, respiratory, and neurological systems, which make it difficult for anesthesiologists to manage the patient’s airway and choose appropriate anesthetic drugs. Case A 13-year-old male patient with Joubert syndrome and mitochondrial disease underwent elective surgery to insert a continuous ambulatory peritoneal dialysis catheter. Anesthesia was induced and maintained with propofol, remifentanil, and rocuronium. An I-gel was inserted to secure the airway; however, the fitting did not work properly, so the patient was intubated. The operation was completed without any major problems, and the intubated patient was transferred to the intensive care unit. ConclusionsAnesthesiologists should determine the method of anesthesia and prepare for unintended complications based on a full understanding of these congenital diseases.

Additional file 3: Figure S3. Tuning parameter log (lambda) selection during ten-fold cross validation (a) and L1-norm value (b) used for the regression model.

Additional file 2: Figure S2. Summary statistics of differentially hyper- (a), and hypomethylated (b) probes, and hypermethylated regions (c) in nonresponders according to their location. Differentially hypomethylated regions are now shown due to the lack of them.

Additional file 5: Figure S5. X-tile plots of the methylation patterns of the eight genes used in the regression model for a total of 141 samples across immunotherapy (SMC and IDIBELL) [56]. Each plot represents an association between individual gene methylation level and survival. The cut point, which separates high- and low-risk groups, represents the highest χ2-value obtained from the Kaplan-Meier survival analysis and log-rank test. Green and red indicate direct and inverse relationships, respectively, between methylation and survival.

Additional file 1: The effect of HLA-B*51 on clinical manifestations of BD. (DOCX 14 KB)

Additional file of Associations between the HLA-Apolymorphism and the clinical manifestations of Behcet's disease

Additional file 4: Figure S4. ROC curve analysis of the risk score for predicting progression-free survival greater than six months (a) and one year (b).

Additional file 1: Figure S1. Survival plots of previously known biomarkers. (a) tumor mutation burden (b) neo-antigen load (c) aneuploidy level (d) PD-L1 expression. Mutation burden, neo-antigen load, and aneuploidy level were calculated with exome-sequencing data (n = 60) and PD-L1 expression was obtained from RNA-sequencing data (n = 28).

Introduction Adult type 2 diabetes (T2DM) threatens public health and most patients manage their diabetic condition while in the community. As it is challenging for patients to properly manage diabetes alone, caregiver involvement in T2DM patient care is encouraged. This study aimed to examine the association between caregiver involvement in T2DM education within a community and the patients’ diabetes care outcomes (e.g., glycated hemoglobin (HbA1c) level, behavior, or hospitalization). Methods The available scientific literature in PubMed, Cochrane, EMBASE, and CINAHL was searched. The methodological quality of bias was assessed using the Cochrane risk of bias tool. Results A total of 13 out of 741 published studies were synthesized in this review. There is evidence that caregiver involvement in T2DM education is effective in the reduction of HbA1C and BMI, but not necessarily effective in reducing lipids. Study results indicate that caregiver related interventions can significantly improve patient diabetes knowledge, physical activity, and self-efficacy, but results were more mixed regarding medication adherence. Risk of bias analysis classified the majority of studies (77%) to be moderate or high quality. Conclusion This review aimed to explore the association between caregiver involvement in adult T2DM education in the community and patients’ diabetes care outcomes. The findings show an improvement in biological and behavioral self-management outcomes with caregivers involved in T2DM education, though no studies examined the direct association between complications or hospital readmission. Future research focused on tailored interventions and longer follow-up of patient outcomes are recommended.

Objective The purpose of this study is to describe and evaluate the results of the GAFINET(Gyeonggi Acute Febrile Illness Surveillance Network) operated for one year. Introduction After MERS outbreak in 2015, the provincial government and infectious disease control center (GIDCC) initiated an emergency department (ED) based Gyeonggi-do provincial acute febrile illness (AFI) surveillance network (GAFINet) to monitor for a subsequent outbreak of emerging or imported infectious diseases since September 2016. Following pilot operation from September to December 2016, the operation was run for one year from June 2017 to May 2018. GAFINet Initiative involves ten hospitals, consisted of four university-affiliated hospitals and six provincial medical centers in Gyeonggi-do province. These hospitals participated in this network voluntarily. Methods Periodic surveillance for finding AFI patients in ED of participating hospitals was performed prospectively (Figure 2). AFI was defined as 1) fever: body temperature ≥38 °C at admission, or 2) chief complaint of febrile or chilling sensation. Demography of patients and chief complaints were investigated in this first step. Cases were classified into six categories based on their clinical diagnoses: 1) respiratory AFI [AFRI], 2) gastroenteric AFI [AFGI], 3) exanthematic AFI [AFEI], 4) other infectious AFI, 5) non-infectious AFI, and 6) unclassified AFI. Participating infectious diseases specialists regularly reviewed and reformed this classification via web based system. Nosocomial AFI cases or the patients transferred from another hospital were excluded. When a patient had a history of international travel or he/she were undiagnosed in three days after ED admission, more comprehensive information including history and final diagnosis were obtained. For a baseline data, age- and sex-stratified ED visits were also gathered weekly. The proportion of AFI cases per 1000 visits was determined for one week period and analyzed by febrile diseases categories with age-stratification. Characteristics of cases with international travel histories or undiagnosed cases were also described separately. The results were presented to participating researchers as visualized dashboards in Web-based systems. Also we compared the trend and peak time of GAFINET data with the surveillance data of KCDC for influenza, hand foot and mouth disease, and Tsutsugamushi disease. Results The total number of patients in the emergency room in 10 hospitals was 366,695 for one year. Among them, 40,897 patients were diagnosed with acute febrile illness (11.2%). 47.8% were under 10 years of age, and 508 - 1,769 patients were fever - related patients per weak. The number of patients with foreign travel was 0 - 11 in each week, and the number of patients with unknown fever in the week was 0 - 6. 1 to 9 years of age accounted for the largest proportion (27.6% - 48.3%) of acute febrile patients. The most common infectious diseases were acute febrile respiratory disease, and other acute febrile infections and acute febrile gastrointestinal disease are followed. In acute febrile respiratory disease and acute febrile illness, seasonal pattern was seen. A total of 157 patients with acute febrile illnesses with overseas travel ability, 0 - 11 persons per week, consistently occurred. Acute gastroenteritis (AGE) was the most frequent diagnosis in 28 patients (17.8%), fever of unkonwn origin(FUO) was 25(15.9%), Acute pharyngotonsilitis 17(10.8%), viral infection 17 (10.8%), Influenza 7 (4.5%) and colitis 5 (3.2%). The cause of fever was not found in 30 of the acute febrile patients with overseas travel ability. A total of 77 patients with acute febrile disease were admitted to the hospital. In comparison with national surveillance data, the trend of occurrence of influenza in GAFINet and national data was similar. Both data peaked in the first week of 2018. Also trend of hand-foot and mouth disease(HFMD) was similar in two data. But peak weak was little different in both data(CDC at 30 weeks, GAFINett at 29 weeks in 2017). In GAFINet, the final diagnosis was confirmed as Tsutsugamushi disease in total 43 cases. The number of reported cases was small, and the epidemic peaked at the same time as the CDC surveillance data, but the outbreak occurred in the same period (October to November). Conclusions GAFINet has both the characteristics of indicator based surveillance and event based surveillance. Data were collected over a period of one year to examine the feasibility and applicability of the indicator based surveillance. In comparison with national surveillance data, some feasibility was verified by similar trends, but the necessity of operating regional surveillance data still needs to be discussed. It should also be noted that some diseases have different peak times of one week. The role of event based surveillance is mainly aimed at surveillance of fever patients with international travel history. The advantage of the GAFINet's classification system in the emergency room of participating hospitals is that the patient's cases of fever were constantly monitored. However, there is still a limit to the lack of budget and manpower in the samping and labaratory test. Real time visual feedback of surveillance data helped to increase participation and discuss the results.

본 연구에서는 청소년의 인터넷 사용유형을 측정하는 문항들을 요인분석으로 요약한 후 이요인점수를 바탕으로 군집분석을 실시하여 인터넷 사용유형의 하위 집단을 분류하고 각 집단이 정신건강 지표, 자기효능감, 자기조절 학습능력 상에서 유의한 차이가 있는지를 밝힘으로써 각 군집의 특성에 맞는 상담·교육적 개입에 대한 시사점을 얻고자 하였다. 연구대상은 서울 및 경기 소재의 남녀 중학생 239명과 남녀 고등학생 269명이었고 자료 분석을 위해 인터넷 사용유형 군집을 독립변인으로 하고 정신건강, 자기효능감, 자기조절 학습능력을 종속변인으로 하여 군집분석 및 다변량 분산분석을 실시하였다. 그 결과 4개의 군집이 적합한 것으로 나타났다. 군집 1은 ``게임/채팅형``, 군집 2는 ``학습/여가/다운로드형``, 군집 3은 ``학습/커뮤니티형``, 군집 4를 ``다목적사용형``으로 명명하였으며, 4개의 인터넷 사용유형에 따른 정신건강, 자기효능감 및 자기조절 학습능력 상의 차이가 유의하였다. 청소년들의 인터넷 사용유형별 특징을 파악함으로써 인터넷 사용동기와 잠재적 중독성을 파악하고 각 유형에 적합한 교육프로그램 개발에 대해 논의하였다.