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: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.

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.

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 investigate the tolerability of early enteral nutrition (EN), and to further explore the association of early EN with clinical outcome in critically ill patients with hemodynamic instability.Methods:The adult patients from Zhejiang Provincial People’s Hospital with an expected admission to ICU for at least 24 h were consecutively recruited from May 2014 to May 2016, and all clinical, laboratory, and survival data were prospectively collected. The AGI grade was daily assessed on the first week of ICU admission. Enteral nutrition (EN) started after 6 h of hemodynamic stability (MAP ≥ 65 mmHg) when the patients took vasoactive medication. The patients were divided into three groups based on the timing of EN initiation: early EN group (EN initiation within 48 h of ICU admission), late EN group (EN initiation at more than 48 h of ICU admission), and no initiation of enteral feeding within 7 days of ICU admission.Results:Of 201 patients enrolled, the mean age was 65.3 ± 16.4 years, APACHE II score was 22.4 ± 6.85, and 191 patients (95.0%) took mechanical ventilation. There were no differences in high gastric residual volume, diarrhea, and gastrointestinal (GI) bleeding between the early EN group and late EN group ( P>0.05). Whereas, patients in the no initiation of EN within 7 days of ICU admission had a lower prevalence of gastric residual volume (16.7% vs. 33.3%, P=0.05), but higher prevalence of GI bleeding (47.2% vs. 26.1%, P=0.02). Compared with those in the late EN group and in no initiation of EN within 7 days of ICU admission, patients in the early EN group had lower 28- (30.4% vs. 47.9% vs. 55.6%, P=0.01) and 60-day mortality rates (38.0% vs. 53.4% vs. 63.9%, P=0.017). Multivariate Cox regression analysis showed that the timing of EN initiation on the admission to ICU (early EN vs. late EN, χ 2≥5.83, P<0.05; early EN vs. no initiation of EN, χ 2≥7.90, P<0.01), serum creatinine ( χ 2=5.06, P<0.05), plasma albumin ( χ 2≥6.41, P<0.01), AGI grade ( χ 2≥8.15, P<0.01), and APACHE II score ( χ 2≥9.62, P<0.01) were independent predictors for 28- and 60-day mortality. Conclusions:Early EN on admission to ICU could be tolerated, and is significantly associated with lower risk of 28- and 60-day mortality in critically ill patients with vasoactive medication to maintain hemodynamic stability.

Objective:To explore the effects of 30° and 45° angles on the time and comfort of mechanical ventilation and extubation for patients undergoing abdominal surgery.Methods:A total of 64 patients who underwent oral tracheal intubation at SICU in the General Surgery Center of the Critical Care Medical Center of our hospital from January 2018 to November 2018 were selected. A total of 64 patients were randomly divided into group A and group B by random number table method, of which there were 31 people in group A and 33 people in group B. During the experiment, 1 people in group A and 3 people in group B experienced dyspnea during the withdrawal process and terminated treatment. Finally, 30 patients in group A and 30 patients in group B were selected. The bedside angle during group A treatment was 30°, and the bedside angle during group B treatment was 45°. The bedside angle card was used by both groups to measure the height of the bedside during the weaning. The two groups of patients were compared during the weaning period (improved Visual Analogue Scale score), weaning to extubation time, aspiration rate, intubation time, pressure ulcer rate, pulmonary function and hemodynamic changes after extubation.Results:Patients with ventilator to extubation time, during the machine during oxygenation index, pulled machine comfort score in group A were 117.50 (45.25, 189.00) min, (348.20±59.72) mmHg(1 mmHg=0.133 kPa), 1.00 (1.00, 2.00) points, group B were respectively 30.00 (13.50, 42.75) min, (314.60±67.13) mmHg and 3.00 (2.00, 3.00) points, two groups comparing the difference was statistically significant ( Z values was -2.411, -4.806, t value was 2.048, P<0.05 or 0.01). Conclusion:Lifting the bedside 30° during mechanical ventilation withdrawal in patients with abdominal surgery can shorten the time from patient withdrawal to extubation, improve the patient's oxygenation index, and improve the comfort during patient withdrawal. It can be used as an early patient for abdominal surgery. One of the auxiliary intervention measures for withdrawal.