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.

This study summarized the nursing experience of midline catheter insertion in 12 patients with a body mass index≥50,providing clinical references.Key nursing interventions included:pre-insertion patient positioning was adjusted to semi-Fowler's or sitting position based on respiratory status.The puncture sites were chosen at the middle or distal third of the upper arm with elbow flexion.The widened tourniquets and extended-length needles/catheter sheaths were used according to vascular depth and puncture angle.The modified pre-insertion length measurement and real-time ultrasound-guided vascular puncture were applied.The catheter with crisscross elastic bandage was secured post-insertion.All 12 patients successfully underwent catheterization,with devices removed after completing intravenous therapy and blood sampling.

Objective Using event-related potentials(ERPs)technology to study the effect of long term emotional distress on selective attention distraction inhibition function in college students and its neuroelectrophysiological mechanism.Methods The Eysenck personality questionnaire(EPQ)adult version was used to screen the high and low neuroticism groups among college students,and 35 subjects in each group were included in the long term emotional distress group and the emotional smoothness control group,respectively,and the response time,correct rate,N2 and P3 amplitude and latency results of the participants to complete the negative priming paradigm task were collected and analyzed.Results Compared with the control group,① the long term emotional distress group showed a prolonged response trend(P=0.072).② the long term emotional distress group had a prolonged N2 and P3 latency(P<0.05).Conclusion Selective attention distraction inhibition in college students with long term emotional distress decreased,and the decline mechanism may be related to the decline of inhibition processing and attention resource allocation ability.

Objective To investigate the effect of Qi stagnation on inhibitory control function and its neuroelectrophysiological mechanism in college students.Methods The population of peace and Qi depression was screened from college students through the scale of"Classification and Judgment Criteria of Traditional Chinese Medicine Constitution",and 35 subjects were included in each group.Combined with event-related potential technology,the two groups were collected to complete the Flanker paradigm behavioral indicators(accuracy,response time)and EEG data indicators(amplitude and latency of N2 and P3 components),and the behavioral differences and neuroelectrophysiological mechanisms between the two groups under the Flanker paradigm were explored.Results Compared with the flat group,the reaction time of the gas depression group was longer(P=0.07).Compared with the consistent dondition,the reaction time was longer under the inconsistent condition(P<0.001),and the accuracy rate was lower under the inconsistent condition significantly(P<0.001).Compared with the peaceful group,the N2 amplitude of the air depression group was higher(P<0.05).There was a statistically significant difference in the main effect of brain regions in the N2 latency period(P<0.001).Frontal lobe zero(Fz)in frontal region was greater than Frontal lobe central zero(FCz)in frontal region and greater than Central zero(Cz)in central region(P<0.05).There was a statistically significant difference in the main effect of brain region(P<0.001).FCz in the frontal central region was significantly greater than that in the frontal region Fz(P<0.001),and the difference was statistically significant in the central region Cz was significantly greater than that in the frontal region(P<0.001).The incubation period of P3 component was significantly smaller than that of inconsistent stimulation(P<0.001).Conclusion Qi stagnation has a negative effect on the inhibitory control function of college students,and the decline of conflict monitoring and control ability is one of the possible mechanisms.

Objective:To explore the values of reticulin fiber staining (RFS) in evaluating bone marrow (BM) involvement of lymphoma and in grading of BM biopsy from adult lymphoma patients.Methods:Retrospectively,354 cases of adult lymphoma were collected from November 2023 to May 2024 at Peking University Cancer Hospital. BM samples were stained with RFS and immunohistochemical staining (IHC), and flow cytometry (FCM) was also performed with the BM aspirations simultaneously. RFS was graded according to the European Consensus, as high grade (grade 2-3) indicating BM involvement in the study. BM involvement was considered as definite if no less than two positive findings among IHC, FCM, and RFS. Statistical analyses were performed via SPSS software (V23.0).Results:In this series, 52.3% (185/354) of the patients were male; 35.0% (124/354) aged >60 years; BM involvements were found in 34.5% (122/354) cases with high grade of RFS, which, in turn, were lymphoblastic leukemia/lymphoma (ALL/LBL) group (4/4), indolent B-cell lymphoma (IndBCL) group (49.1%, 53/108), transformed B-cell lymphoma (TrBCL) group (2/5), invasive B-cell lymphoma (InvBCL) group (26.5%, 41/155), T and NK cell lymphoma (TNKCL) group (27.3%, 12/44) and classical Hodgkin lymphoma (CHL) group (26.3%, 10/38); if classified by specific types, T-ALL/LBL (2/2), B-ALL/LBL (2/2) and CLL/SLL (8/10) ranked top three. In terms of the positive rate of BM involvement evaluated by RFS, no significant difference was seen between either gender or age groups ( χ2=3.416, P=0.332 and χ2=4.200, P=0.241); however, significant differences were observed between different lymphoma groups and types ( χ2=29.961, P=0.012 and χ2=102.546, P<0.001, respectively). BM invasion rates indicated by IHC and FCM were 25.4% (90/354) and 13.8% (49/354), respectively. The overall BM invasion rate was 24.3% (86/354), and the sensitivity of RFS, IHC, and FCM was 90.8%, 97.8%, and 55.8%, and specificity was 84.1%, 99.6%, and 98.9%, respectively. Overall, the concordance rate of RFS with IHC and FCM was 83.6% and 74.0%, respectively, including 85.8% and 74.2% for InvBCL group, 79.6% and 75.0% for IndBCL group, 84.1% and 75.0% for TNKCL group, 81.6% and 73.7% for CHL group, 5/5 and 2/5 for TrBCL group, and 4/4 and 3/4 for ALL/LBL group. Conclusions:In the evaluation of BM involvement status of adult lymphoma, high sensitivity and specificity are observed by RFS, and high concordance is also noted with both IHC and FCM. Thus, the BM infiltrating status of adult lymphoma could be evaluated more accurately by a combined usage of the three methods.

Objective Using event-related potentials(ERPs)technology to study the effect of long term emotional distress on selective attention distraction inhibition function in college students and its neuroelectrophysiological mechanism.Methods The Eysenck personality questionnaire(EPQ)adult version was used to screen the high and low neuroticism groups among college students,and 35 subjects in each group were included in the long term emotional distress group and the emotional smoothness control group,respectively,and the response time,correct rate,N2 and P3 amplitude and latency results of the participants to complete the negative priming paradigm task were collected and analyzed.Results Compared with the control group,① the long term emotional distress group showed a prolonged response trend(P=0.072).② the long term emotional distress group had a prolonged N2 and P3 latency(P<0.05).Conclusion Selective attention distraction inhibition in college students with long term emotional distress decreased,and the decline mechanism may be related to the decline of inhibition processing and attention resource allocation ability.

Objective To investigate the effect of Qi stagnation on inhibitory control function and its neuroelectrophysiological mechanism in college students.Methods The population of peace and Qi depression was screened from college students through the scale of"Classification and Judgment Criteria of Traditional Chinese Medicine Constitution",and 35 subjects were included in each group.Combined with event-related potential technology,the two groups were collected to complete the Flanker paradigm behavioral indicators(accuracy,response time)and EEG data indicators(amplitude and latency of N2 and P3 components),and the behavioral differences and neuroelectrophysiological mechanisms between the two groups under the Flanker paradigm were explored.Results Compared with the flat group,the reaction time of the gas depression group was longer(P=0.07).Compared with the consistent dondition,the reaction time was longer under the inconsistent condition(P<0.001),and the accuracy rate was lower under the inconsistent condition significantly(P<0.001).Compared with the peaceful group,the N2 amplitude of the air depression group was higher(P<0.05).There was a statistically significant difference in the main effect of brain regions in the N2 latency period(P<0.001).Frontal lobe zero(Fz)in frontal region was greater than Frontal lobe central zero(FCz)in frontal region and greater than Central zero(Cz)in central region(P<0.05).There was a statistically significant difference in the main effect of brain region(P<0.001).FCz in the frontal central region was significantly greater than that in the frontal region Fz(P<0.001),and the difference was statistically significant in the central region Cz was significantly greater than that in the frontal region(P<0.001).The incubation period of P3 component was significantly smaller than that of inconsistent stimulation(P<0.001).Conclusion Qi stagnation has a negative effect on the inhibitory control function of college students,and the decline of conflict monitoring and control ability is one of the possible mechanisms.

Objective:To explore the values of reticulin fiber staining (RFS) in evaluating bone marrow (BM) involvement of lymphoma and in grading of BM biopsy from adult lymphoma patients.Methods:Retrospectively,354 cases of adult lymphoma were collected from November 2023 to May 2024 at Peking University Cancer Hospital. BM samples were stained with RFS and immunohistochemical staining (IHC), and flow cytometry (FCM) was also performed with the BM aspirations simultaneously. RFS was graded according to the European Consensus, as high grade (grade 2-3) indicating BM involvement in the study. BM involvement was considered as definite if no less than two positive findings among IHC, FCM, and RFS. Statistical analyses were performed via SPSS software (V23.0).Results:In this series, 52.3% (185/354) of the patients were male; 35.0% (124/354) aged >60 years; BM involvements were found in 34.5% (122/354) cases with high grade of RFS, which, in turn, were lymphoblastic leukemia/lymphoma (ALL/LBL) group (4/4), indolent B-cell lymphoma (IndBCL) group (49.1%, 53/108), transformed B-cell lymphoma (TrBCL) group (2/5), invasive B-cell lymphoma (InvBCL) group (26.5%, 41/155), T and NK cell lymphoma (TNKCL) group (27.3%, 12/44) and classical Hodgkin lymphoma (CHL) group (26.3%, 10/38); if classified by specific types, T-ALL/LBL (2/2), B-ALL/LBL (2/2) and CLL/SLL (8/10) ranked top three. In terms of the positive rate of BM involvement evaluated by RFS, no significant difference was seen between either gender or age groups ( χ2=3.416, P=0.332 and χ2=4.200, P=0.241); however, significant differences were observed between different lymphoma groups and types ( χ2=29.961, P=0.012 and χ2=102.546, P<0.001, respectively). BM invasion rates indicated by IHC and FCM were 25.4% (90/354) and 13.8% (49/354), respectively. The overall BM invasion rate was 24.3% (86/354), and the sensitivity of RFS, IHC, and FCM was 90.8%, 97.8%, and 55.8%, and specificity was 84.1%, 99.6%, and 98.9%, respectively. Overall, the concordance rate of RFS with IHC and FCM was 83.6% and 74.0%, respectively, including 85.8% and 74.2% for InvBCL group, 79.6% and 75.0% for IndBCL group, 84.1% and 75.0% for TNKCL group, 81.6% and 73.7% for CHL group, 5/5 and 2/5 for TrBCL group, and 4/4 and 3/4 for ALL/LBL group. Conclusions:In the evaluation of BM involvement status of adult lymphoma, high sensitivity and specificity are observed by RFS, and high concordance is also noted with both IHC and FCM. Thus, the BM infiltrating status of adult lymphoma could be evaluated more accurately by a combined usage of the three methods.

This study summarized the nursing experience of midline catheter insertion in 12 patients with a body mass index≥50,providing clinical references.Key nursing interventions included:pre-insertion patient positioning was adjusted to semi-Fowler's or sitting position based on respiratory status.The puncture sites were chosen at the middle or distal third of the upper arm with elbow flexion.The widened tourniquets and extended-length needles/catheter sheaths were used according to vascular depth and puncture angle.The modified pre-insertion length measurement and real-time ultrasound-guided vascular puncture were applied.The catheter with crisscross elastic bandage was secured post-insertion.All 12 patients successfully underwent catheterization,with devices removed after completing intravenous therapy and blood sampling.

Objective:To construct an evaluation framework with detailed indices for demonstration units (wards) of enteral nutrition nursing, in order to improve the competence of nurses in enteral nutrition nursing and inform the specialized development of enteral nutrition demonstration units (wards).Method:On the basis of literature review and expert discussion, a preliminary draft was developed, and the Delphi expert consultation method was used to conduct two rounds of consultation with 15 clinical experts in the field of enteral nutrition nursing from 15 tertiary hospitals.Results:The effective response rates of questionnaires in two rounds of consultations were both 100%. The first round of expert consultation showed an authority coefficient of 0.90 and a coefficient of variation of 0 to 0.167, while the second round showed an authority coefficient of 0.93 and a coefficient of variation of 0 to 0.113. The Kendall harmony coefficients were 0.338 and 0.368, respectively. Finally, the evaluation framework with detailed indices for the demonstration unit (ward) of enteral nutrition nursing was formed, which consisted of 3 primary indicators, 16 secondary indicators, 54 tertiary indicators, and 62 detailed items.Conclusions:The evaluation framework we developed for the demonstration unit of enteral nutrition nursing follows the diagnosis and treatment process of enteral nutrition management for inpatients, including the triad of structure, process, and outcome. The framework is objective and practical, and can inform the daily practice of enteral nutrition nursing demonstration units (wards) and the development of enteral nutrition nursing specialties.