This case report presents a 16-year-old male patient with deficiency of adenosine deaminase 2(DADA2). The patient had a history of Raynaud′s phenomenon with digital ulcers since childhood. As the disease progressed, the patient developed retinal vasculitis, intracranial hemorrhage, skin necrosis, severe malnutrition, refractory hypertension, and gastrointestinal bleeding. Genetic testing revealed compound heterozygous mutations in the ADA2 gene (paternal c.1072G > A pathogenic mutation + maternal c.1065C > A variant of unknown clinical significance). ADA2 enzyme activity was significantly reduced (0.1 U/L). A multidisciplinary treatment team developed an individualized plan to address key issues, including nutritional support, blood pressure control, rehabilitation, pain management, and balancing anticoagulation/bleeding risks. After systematic intervention, the patient′s condition showed partial improvement. This case reveals clinical challenges such as anticoagulation decisions and nutritional support of DADA2. It also emphasizes the importance of early molecular diagnosis, tumor necrosis factor-α inhibitor targeted therapy, and multidisciplinary collaboration in management, underlining the core value of precision medicine in rare disease management.

Objective:The evidence on the use of bedside ultrasound to assess muscle mass in critically ill adults was retrieved and screened, and the best evidence was summarized.Methods:A computer search was conducted for relevant literature on ultrasound measurement of muscle mass in critically ill adults in domestic and foreign databases such as BMJ Best Clinical Practice, UpToDate, PubMed, CNKI, and guide website and professional association website. The search time limit was from the establishment of the database to August 30, 2023. Literature quality was evaluated by four researchers trained in systematic evidence-based courses according to literature type.Results:A total of 15 literatures were included, including 2 guideline, 4 expert consensus, 5 systematic reviews and 4 randomized controlled studies. A total of 22 pieces of evidence were summarized, including 6 aspects: position and patient preparation, feasibility of implementation by nursing staff, selection of probe and matters needing attention, muscle positioning, evaluation of muscle structure by ultrasound and the guiding significance of ultrasound evaluation of muscle mass.Conclusions:The best evidence summary of bedside ultrasound assessment of muscle mass in critically ill adults summarized in this study is scientific and systematic, and provides evidence-based basis for establishing standardized ultrasound assessment procedures in clinic.

Objective : To investigate the role of early inhibition of Toll⁃like receptor 4 (TLR4) in regulating hippampal neuroimmune responseto adolescent ratsafter neonatal hypoxic⁃ischemic brain damage(HIBD) . Methods: Postnatal day 7 rats were randomized into controlgroup , hypoxic ischemia (HI) group , and HI + TAK⁃242( the specific inhibitor of TLR4)(TAK⁃242) group. The expression of TLR4 in rat hippocampus was detected by immunohistochemistry at 3 days after HI. Immunofluorescence were used to determine the number of Iba⁃1 + , GFAP + , CD161 + , MPO + and CD3 + cells in the hippocampus at 21 days after HI. Immunohistochemistry was used to detect ICAM⁃1 and C3a expression in the hippocampal CA1 region ; and Western blot was used to detect tumor necrosis factor interleukin IL⁃1β , TNF⁃α and IL⁃10 expression. Results : Compared with control group , significantly raised TLR4 expression was observed in the left hippocampal CA1 , CA3 and DG regions(P < 0. 01 or P < 0. 05) , while the expression in the TAK⁃242 group lowered compared to the HI group (P < 0. 05) . The number of GFAP + cells in the CA1 area of the hippocampus in the TAK⁃242 group of neonatal rats decreased compared to which in the HI group at 21 days after HI(P < 0. 05) , but the number of CD3 + T lymphocytes in the hippocampal CA1 area of new born rats in the HI group increased compared to which in the Control group (P < 0. 05) , but the difference between TAK⁃242 and the Control group was not statistically significant. The number of Iba⁃1 + cells , MPO + cells , CD161 + cells , the expression of ICAM⁃1 and C3a in hippocampal CA1 region , and the expression of TNF⁃α , IL⁃1β and IL⁃10 in hippocampus of rats were not different among groups at 21 days after HIBD. Conclusion Early inhibition of TLR4 may ameliorate adolescent neuroimmune disorders by reducing the increase of hippocampal astrocytesafter neonatal HIBD.

Objective:To summarizes the evidence of oral and nasal care for adult inpatients during nasal tube feeding and provide reference for clinical implementation.Methods:The relevant evidence on oral and nasal care of adult patients with nasal feeding were systematically searched on UpToDate, Joanna Briggs Institute (JBI) Evidence-based Healthcare Center, National Guideline Clearinghouse, National Institute for Health and Clinical Excellence, Medlive.cn, Cochrane Library, PubMed, Embase, Web of Science, China National Knowledge Infrastructure, Wanfang database and other databases and guideline websites. The retrieval period was from January 1, 2016 to August 28, 2020. After the methodological quality evaluation of the included clinical practice guidelines, expert consensus, systematic evaluation, clinical decision-making and evidence summary, the literature that met the standards was extracted and the evidence was summarized according to the professional clinical judgment of the researchers.Results:A total of 13 literatures were included, including 1 guideline, 2 expert consensus, 3 systematic evaluation, 2 clinical decision making and 5 evidence summaries. After sorting out, 16 best evidences were summarized from 4 aspects such as professional training of nursing staff, factors affecting oral and nasal hygiene, assessment and nursing of nasal, assessment and nursing of oral.Conclusions:Through the standardized evidence collection and formulation method, this paper summarizes the relevant evidence of oral and nasal nursing for patients with enteral nutrition through nasal tube feeding from 4 aspects. Medical staff should strengthen the training of professionals, find out the factors affecting the oral and nasal health of adult inpatients undergoing nasal tube feeding as soon as possible and take effective measures to evaluate and care the patients' oral and nasal cavity according to the best evidence.

Objective:To explore the construction and effect of biobank in biological research in nursing field.Methods:Through the establishment of a biological research plan for nursing in Affiliated Jinhua Hospital, Zhejiang University School of Medicine, the biobank in biological research for nursing was constructed from aspects of biological samples, instruments and equipment, operating software, biobanking team formation, personnel training and standardized process formulation.Results:In August 2020, the Nursing Department and Central Laboratory of Affiliated Jinhua Hospital, Zhejiang University School of Medicine established a biobank for biological research in the nursing field, formed a biobank team, conducted personnel training and formulated standardized procedures. From December 1, 2020 to June 30, 2021, biological samples were collected from 264 gastric cancer patients and 56 healthy controls, mainly including whole blood, serum and urine.Conclusions:The construction of biobank for biological research in nursing field can collect abundant biological samples, which lays a foundation for subsequent independent biological research in nursing field.

Objective:Analysis of influencing factors of enteral nutrition interruption in critically ill patients in general surgery department and its impact on prognosis.Methods:A total of 91 cases of critically ill patients in general surgery department were selected who were admitted to the general surgery of General Hospital of Eastern Theater Command of the Chinese People′s Liberation Army in Nanjing from June 2021 to March 2022 by convenient sampling method, demographic and enteral nutrition interruption data were collected,and patients were divided into enteral nutrition interruption group and enteral nutrition uninterrupted group to investigate the analysis of the factors of affecting enteral nutrition interruption and its impact on prognosis by Logistic regression analysis.Results:There were 59 cases in the enteral nutrition interruption group and 32 cases in the enteral nutrition uninterrupted group. There were statistically significant differences in gender, analgesic and sedatives, Gastro-kinetic agent and feeding intolerance between both groups ( χ2 values were 4.51-9.97, all P<0.05). Logistic regression analysis results showed that gender ( OR=4.566, 95%CI 1.332-15.657, P<0.05), analgesic and sedatives ( OR=3.437, 95%CI 1.112-10.621, P<0.05), and feeding intolerance ( OR=4.116, 95%CI 1.257-13.479, P<0.05) were the factors of enteral nutrition interruption. There were statistically significant differences between the two groups in the number of days of enteral nutrition up to goal in 3 days, 3-7 days and 7 days, albumin,length of stay in intensive care unit, total length of stay and hospitalization expenses between both groups ( Z values were -2.80 - -0.73, all P<0.05). Conclusions:Female, analgesic and sedatives and feeding intolerance are the risk factors of enteral nutrition interruption in critically ill patients in general surgery department, and enteral nutrition interruption has an adverse impact on the prognosis.Medical staff should avoid excessive use of analgesic and sedatives, and do well in feeding tolerance management to reduce the occurrence of enteral nutrition interruption.

Appendiceal epithelial neoplasms including benign and malignant, are clinically rare. There were quite a lot of changings in classification systems for them and different pathological diagnostic terminologies were used, resulted in confusions of understanding and communication for both pathologists and clinicians. Basically, appendiceal epithelial neoplasms include adenoma, serrated lesion and polyps, mucinous neoplasms, carcinoma and neuroendocrine neoplasms. Appendiceal mucinous neoplasms and goblet cell carcinoma are exclusively seen in the appendix. Though some appendiceal neoplasms are similar to those in large bowl, however, the molecular mechanism is different. The classification, pathological diagnosis and clinical significance of appendiceal epithelial neoplasms were summarized based on the fifth edition of WHO classification on digestive system tumors and other related literatures.

Appendiceal epithelial neoplasms including benign and malignant, are clinically rare. There were quite a lot of changings in classification systems for them and different pathological diagnostic terminologies were used, resulted in confusions of understanding and communication for both pathologists and clinicians. Basically, appendiceal epithelial neoplasms include adenoma, serrated lesion and polyps, mucinous neoplasms, carcinoma and neuroendocrine neoplasms. Appendiceal mucinous neoplasms and goblet cell carcinoma are exclusively seen in the appendix. Though some appendiceal neoplasms are similar to those in large bowl, however, the molecular mechanism is different. The classification, pathological diagnosis and clinical significance of appendiceal epithelial neoplasms were summarized based on the fifth edition of WHO classification on digestive system tumors and other related literatures.

Objective:To explore the effects of individualized exercise prescription in critically ill patients during enteral nutrition.Methods:From January to August 2018, we selected 120 critically ill patients admitted to the General Surgery Intensive Care Unit (ICU) of General Hospital of Eastern Theater Command. According to admission time, patients were divided into control group and observation group, with 60 cases in each group. Control group used the conventional chest physical therapy, exercise program for passive and active functional exercise of limbs. Observation group implemented the individualized exercise prescription to implement functional exercise orders. After daily rounds, doctors and nurses assessed the condition of the day by combining patient's basic condition and prescribed the individualized exercise prescription, including functional exercise items, requirements, dosages and frequency, performed by a full-time nurse. We compared the muscle strength of patients between two groups with the MRC scores, and observed the intolerance, number of interruptions of enteral nutrition feeding and the ICU hospitalization time during enteral nutrition between two groups.Results:After 168 hours and 366 hours, the differences of MRC scores between two groups were significant ( P<0.05) . After 14 days of intervention, the serum total protein, hemoglobin and body mass index (BMI) of observation group were higher than those of control group also with statistical differences ( P<0.05) . The number of cases of abdominal distension, gastric retention, the total number of intolerance and the number of feeding interruptions during enteral nutrition in the observation group were lower than those in the control group, and the differences were statistically significant ( P<0.05) . The ICU hospitalization time of patients of observation group and control group were (25.28±5.42) d and (27.94±5.65) d respectively also with a statistical difference ( P<0.05) . Conclusions:The individualized exercise prescription functional exercise intervention strategies is beneficial for critically ill patients to improve muscle strength, nutritional indicators, avoid rapid decline in BMI, reduce feeding intolerance and the number of feeding interruptions, ensure continuous implementation of enteral nutrition, and shorten patients' ICU hospitalization time so as to promote accelerated recovery of patients.

Objective:To explore the influences of different lying positions on vital signs and comforts in patients with tracheal intubation removed after intestinal fistula surgery.Methods:From October 2018 to June 2019, convenience sampling was used to select 150 patients with intestinal fistula surgery after general anesthesia in Intensive Care Unit of the General Hospital of Eastern Theater Command were selected as the research object. After waiting for the patient to wake up from anesthesia, the tracheal intubation was removed, and the lying position was changed after normal spontaneous breathing through the nose. Patients were randomly divided into 5 groups, 30 cases in each group. Group A was in the supine position; group B was in the lying position with the head of the bed raised by 15°; group C was in the lying position with the head of the bed raised by 30°; group D was in the lying position with the head of the bed raised by 45°; group E was in the lying position with the head of the bed raised by 15° to 45 °. At the beginning, the head of the bed was shaken to 15 °; and after two hours, the head of the bed was shaken to 30 °, and after another two hours, the head of the bed was shaken to 45 °. After 5 groups of patients fixed the angle of the lying position, when the patient complained of discomfort or the body position changed spontaneously, the patient changed the lying position. The duration of the lying position, uncomfortable symptoms and pain degree of abdominal incision were compared among 5 groups.Results:Patients with tracheal intubation removed after intestinal fistula surgery had a longer persistence in the 15° and 30° lying positions within 6 hours, respectively (72.27±12.41) min and (69.37±9.10) min. There were statistically significant differences in the persistence time of 5 different lying positions ( P<0.01) . The number of patients in group B and C with waist pain was less, and there were more patients with painless incisions, and there were statistically significant differences among 5 groups ( P<0.05) . Conclusions:Among patients with tracheal intubation removed and normal spontaneous breathing through the nose after intestinal fistula surgery, the 15° to 30° lying position within 6 hours after the operation can prolong the patient's adherence to the lying position, reduce the incidence of abdominal incision pain and waist pain, and reduce the probability of dizziness, nausea and vomiting.