Objective:To use the best evidence of noninvasive mechanical ventilation related pressure injury prevention in the clinic guided by integrated promoting action on research implementation in health services framework (i-PARIHS) and evaluate its effect.Methods:This study is an unsynchronized before and after control study. Convenience sampling method was used to select patients receiving non-invasive mechanical ventilation from 7 wards of Beijing Hospital from October 2019 to September 2021. A total of 575 patients receiving non-invasive mechanical ventilation from October 2019 to September 2020 were included in the control group and 602 patients from October 2020 to September 2021 were included in the trial group. The control group adopted the usual care measures, and the trial group applied the prevention program for noninvasive ventilation related facial pressure injuries. The incidence of pressure injury was compared between the two groups. Nurses in 7 wards were investigated before and after the intervention program to compare the changes of knowledge level.Results:In the control group, there were 354 males, 221 females, aged (77.13 ± 14.49) years old; in the trial group there were 392 males, 210 females, aged (75.60 ± 14.27) years old. The incidence of pressure injury in the trial group was lower than the control group, but showed no significant difference ( P>0.05). In the control group, 11 cases suffered pressure injury, including 5 cases in stage 2, 6 cases in stage 3 and above. There were 9 cases in the trial group, including 8 in stage 2 and 1 in stage 3 and above. The severity of pressure injury in the trial group was lower than that in the control group, and the difference was significant ( χ2 = 3.83, 4.11, both P<0.05). The scores of the nurse′pressure injury knowledge increased from (6.77 ± 1.53) points to (7.15 ± 1.47) points, with a significant difference ( t = -2.31, P<0.05). Conclusions:Management of the prevention of noninvasive mechanical ventilation related facial pressure injury through evidence-based practice can reduce its incidence, reduce its severity, and it is beneficial to improve the clinical nurses' prevention knowledge and clinical practice level of facial pressure injury related to non-invasive mechanical ventilation.

Patients with severe trauma require an extremely timely treatment and transfusion plays an irreplaceable role in the emergency treatment of such patients. An increasing number of evidence-based medicinal evidences and clinical practices suggest that patients with severe traumatic bleeding benefit from early transfusion of low-titer group O whole blood or hemostatic resuscitation with red blood cells, plasma and platelet of a balanced ratio. However, the current domestic mode of blood supply cannot fully meet the requirements of timely and effective blood transfusion for emergency treatment of patients with severe trauma in clinical practice. In order to solve the key problems in blood supply and blood transfusion strategies for emergency treatment of severe trauma, Branch of Clinical Transfusion Medicine of Chinese Medical Association, Group for Trauma Emergency Care and Multiple Injuries of Trauma Branch of Chinese Medical Association, Young Scholar Group of Disaster Medicine Branch of Chinese Medical Association organized domestic experts of blood transfusion medicine and trauma treatment to jointly formulate Chinese expert consensus on blood support mode and blood transfusion strategies for emergency treatment of severe trauma patients ( version 2024). Based on the evidence-based medical evidence and Delphi method of expert consultation and voting, 10 recommendations were put forward from two aspects of blood support mode and transfusion strategies, aiming to provide a reference for transfusion resuscitation in the emergency treatment of severe trauma and further improve the success rate of treatment of patients with severe trauma.

Objective:To evaluate the effect of clinical pathway implementation on medical efficiency and medical expenses of patients with two common rheumatic immune diseases " rheumatoid arthritis" and " ankylosing spondylitis" diseases by using diagnosis related group (DRG) related indicators.Methods:The data of patients with two common rheumatic immune diseases " rheumatoid arthritis" and " ankylosing spondylitis" included in the clinical pathway management from January 2017 to December 2019 in the Department of Rheumatology and Immunology of Jinhua Hospital, Zhejiang University School of Medicine were carried out. The impact of clinical pathway implementation on the average hospital stay, average cost and average drug cost of patients with the two diseases were analyzed and compared , so as to evaluate the effect of the implementation of the clinical pathway.Results:From the implementation of clinical pathway in 2017 to 2019, the number of patients admitted and total medical specialty services in the two groups of " rheumatoid arthritis" and " ankylosing spondylitis" increased year by year ( P<0.01). The average length of stay, average cost and average drug cost of patients in the " rheumatoid arthritis" disease group decreased year by year, with statistically significant differences between groups (all P<0.01). The average length of stay in the ankylosing spondylitis group was shortened year by year, and the difference was statistically significant ( P<0.01). Compared with 2017, the average cost in 2018 decreased significantly, and the difference was statistically significant ( P<0.01). There was no significant difference in average cost between 2018 and 2019 ( P>0.05). The average cost in 2018 was significantly higher than that in 2017 ( P<0.05). After analyzing the causes and optimizing the clinical pathway, the average cost in 2019 was significantly lower than that in 2018 ( P<0.01). Conclusions:Through the implementation of clinical pathways and continuous optimization of pathway connotation during use, the diagnosis and treatment efficiency of patients with " rheumatoid arthritis" and " ankylosing spondylitis" can be significantly improved, and medical costs can be reduced, which is in line with the current medical reform needs.

N

To investigate clinical characteristics of muscular sarcoidosis presenting muscle mass as initial symptom. The clinical data of 10 patients diagnosed with muscular sarcoidosis and admitted in the Second Affiliated Hospital of Zhejiang University School of Medicine from May 2014 to October 2020 were collected and retrospectively analyzed. All patients were female, including 2 patients with unilateral involvement of lower extremities, 7 patients with bilateral involvement of lower extremities, and 1 patient with bilateral involvement of upper and lower extremities. Lung lesions were presented in all patients, and 9 cases had mediastinal lymphadenopathy. Serum angiotensin-converting enzyme (sACE) was 57-118 IU/ml. No specific characteristics were found by ultrasound. Typical “dark-star” sign and “three-stripe” sign were shown in the magnetic resonance imaging (MRI). The pathology of striated muscle was non-necrotizing granulomatous inflammation. Three patients were not treated. In 7 patients treated with prednisone 0.5-1 mg ·kg -1·d -1, immunosuppressants were combined in 3 patients at initial and the other 4 patients later on. During the follow-up of 7-62 months, 1 patient was stable, 6 improved and 3 relapsed. Till now, 5 patients are still on the treatment of glucocorticoid and immunosuppressant.

Objective:To observe the clinical characteristics of primary retinitis pigmentosa (RP) complicated with glaucoma.Methods:A retrospective clinical study. From June 2008 to March 2020, the diagnosis of primary RP were included in the diagnosis confirmed by the eye examination of West China Hospital of Sichuan University included 4794 eyes of 2432 patients. Among them, 4679 eyes (97.2%, 2364/2432) were in 2364 cases with RP alone, and 115 eyes were in 68 cases with RP combined with glaucoma (2.80%, 68/2432). All affected eyes underwent best corrected visual acuity (BCVA) and intraocular pressure examination. The BCVA examination was carried out using the international standard visual acuity chart, which was converted into the logarithmic minimum angle of resolution (logMAR) visual acuity during statistics. The 67 eyes of 40 patients with RP and glaucoma with complete follow-up data were analyzed to observe the proportion of different glaucoma types, logMAR BCVA, intraocular pressure and other clinical characteristics, as well as treatment methods and post-treatment intraocular pressure control. After treatment, the intraocular pressure ≤21 mm Hg (1 mm Hg=0.133 kPa) was regarded as intraocular pressure (IOP) control; >21 mm Hg was regarded as uncontrolled IOP.Results:Among the 67 eyes of 40 cases with complete follow-up data, 5 cases (7 eyes) with primary open-angle glaucoma (10.45%, 7/67), 56 cases (58 eyes) with angle-closure glaucoma (ACG) (86.57%, 58/67), 4 cases (4 eyes) with neovascular glaucoma (5.97%, 4/67), 2 of them had both ACG and neovascular glaucoma. Among 58 ACG eyes, 17 eyes were acute ACG (25.37%, 17/67), 21 eyes were chronic ACG (31.34%, 21/67), and 2 eyes were suspicious angle closure (2.99%, 2/67), lens dislocation secondary to angle-closure glaucoma in 8 eyes (11.94%, 8/67), chronic angle-closure glaucoma after anti-glaucoma surgery, intraocular lens shift in 5 eyes (7.46%, 5/67), 5 eyes (7.46%, 5/67) secondary to glaucoma with true small eyeballs. The logMAR BCVA 3.50 of the affected eye,<3.50->2.00, ≤2.00-≥1.30,<1.30->1.00, ≤1.00- 0.52,<0.52 were 9 (13.43%, 9/67), 30 (44.78%, 30/ 67), 7 (10.45%, 7/67), 4 (5.97%, 4/67), 11 (16.42%, 11/67), 6 (8.96%, 6/67) eyes, which correspond to mean intraocular pressure were 32.31±11.67, 30.15±14.85, 28.17±13.19, 31.50±17.25, 18.71±8.85, 14.12±4.25 mm Hg. Among 67 eyes, 37eyes (55.22%, 37/67), 18eyes (26.86%, 18/67), and 6 (8.96%, 6/67) eyes underwent surgery, medication alone, and peripheral iris laser perforation treatment, respectively. The treatment of 6 eyes was abandoned (8.96%, 6/67). Malignant glaucoma occurred in 3 eyes (8.11 %, 3/37) after the operation, all of which were after trabeculectomy of the ACG eye. After treatment, intraocular pressure was controlled in 37 eyes (55.22%, 37/67), 19 eyes were not controlled (28.36%, 19/67), and 11 eyes were lost to follow-up (16.42%, 11/67).Conclusions:The incidence of glaucoma in patients with primary RP is 2.80%. ACG is more common, and the combined lens dislocation or intraocular lens shift is more common.

Objective:To construct a non-invasive mechanical ventilation-related facial pressure injury prevention scheme based on the evidence, so as to provide guidance for the prevention of facial pressure injury.Methods:On the basis of the previous evidence summary, the expert letter questionnaire for prevention of non-invasive mechanical ventilation related facial pressure injury was initially constructed. From November 2020 to March 2021, the Delphi method was used to construct an evidence-based non-invasive mechanical ventilation-related facial pressure injury prevention scheme.Results:The positive coefficient of expert for the two rounds of expert letter inquiries was 100%, the expert authority coefficient was 0.94, and the coefficient of variation was 0 to 0.29 and 0 to 0.22, respectively. The final non-invasive mechanical ventilation-related facial pressure injury prevention scheme included evaluation, prevention strategies, multidisciplinary teamwork, education and training and implementation procedures.Conclusions:Evidence-based non-invasive mechanical ventilation-related facial pressure injury prevention scheme has high reliability and strong clinical guidance for the prevention of facial pressure injuries, which is of great significance for improving quality of nursing and patient outcomes.

Objective:To explore the related influencing factors of interstitial pneumonia (IP) in B-cell non-Hodgkin lymphoma (B-NHL) patients treated with R-CHOP-like chemotherapy regimen.Methods:The clinical data of 377 CD20 + B-NHL patients in Minhang Branch of Fudan University Shanghai Cancer Hospital from January 2014 to June 2019 were retrospectively analyzed. According to whether rituximab was used, patients were divided into R-CHOP-like chemotherapy group (275 cases) and CHOP-like chemotherapy group (102 cases). The incidence of IP in patients stratified according to different clinical factors was analyzed, and logistic multivariate regression was used to analyze the risk factors of IP. Results:Thirty-eight out of 377 patients (10.08%) developed IP; the incidence rates of IP in the R-CHOP-like chemotherapy group and the CHOP-like chemotherapy group were 13.09% (36/275) and 1.96% (2/102), respectively, and the difference was statistically significant (χ 2 = 10.169, P < 0.01). There were statistical differences in the incidence rates of IP between patients with or without rituximab, with or without liposomal doxorubicin in the regimen, and with or without grade Ⅳ neutropenia occurred during the treatment [13.09% (36/275) vs. 1.96% (2/102), 18.18% (22/ 121) vs. 6.25% (16/256), 15.43% (27/175) vs. 5.45% (11/202), all P < 0.01]. Logistic regression analysis showed that the application of rituximab ( OR = 6.761, 95% CI 1.369-33.711, P = 0.020) and grade Ⅳ neutropenia ( OR = 7.443, 95% CI 2.132-8.199, P = 0.001) were independent risk factors for the occurrence of IP. Conclusions:R-CHOP-like chemotherapy regimen increases the occurrence of IP in patients with B-NHL. The use of rituximab and grade Ⅳ neutropenia are independent risk factors for the occurrence of IP.

OBJECTIVE: To analyze the results of screening for hereditary tyrosinemia (HT) in newborns and its clinical features and genotype. METHODS: The HT screening was conducted among 2 188 784 newborns from November 2013 to November 2018. The tyrosine (TYR)/ succinylacetone (SA) levels were detected by tandem mass spectrometry (MS-MS). The clinical characteristics, genetic results and following up data of identified patients were analyzed. RESULTS: The normal ranges (0.5%-95.5%) of TYR and SA were 34.5-280.0 μmol/L and 0.16-2.58 μmol/L, respectively. Three HT cases were confirmed with a detection rate of 1∶729 595. There was 1 case of tyrosinemia type Ⅰ (HTⅠ) (homozygous variations of c.455G>A in gene), 1 case of tyrosinemia type Ⅱ(HTⅡ) (heterozygous variations of c.890G>T and c.408+1G>A in gene), and 1 case of tyrosinemia type Ⅲ (HT Ⅲ) (homozygous variations of c.257T>C in gene). The variations of c.890G>T, c.4081G>A of and c.257T>C of were novel. The positive predictive value of the screening was 3.4%. Case 1 (HTⅠ) with TYR and SA values of 666.9 μmol/L and 3.87 μmol/L respectively, presented cholestasis, mild elevated of liver enzyme and lactic acid, who were although fed with TYR and phenylalanine free milk, but died at 2 months of age. Case 2 (HTⅡ) with higher TYR (625.6 μmol/L) and normal SA at screening, received medical milk treatment; during the 7 months of follow-up the baby showed normal score of Bayley assessment and normal TYR without eye and skin symptoms. Case 3 (HT Ⅲ) with TYR of 1035.3 μmol/L and normal SA at screening; during the 29 months of follow-up the value of TYR fluctuated from 532.1 μmol/L to 1060.3 μmol/L due to irregular medical milk treatment, while the score of Bayley assessment was normal. CONCLUSIONS HT is rare in the southern Chinese population, and the gene spectrum is scattered. Early treatment with nitisinone is recommended in children with HTⅠ, otherwise the prognosis is poor; the prognosis of children with HTⅡ is good when early treated with special diet; the prognosis of children with HTⅢ needs to be determined with more data.
Child ; Cyclohexanones ; therapeutic use ; Genotype ; Humans ; Infant ; Infant, Newborn ; Neonatal Screening ; Nitrobenzoates ; therapeutic use ; Tandem Mass Spectrometry ; Tyrosinemias ; diagnosis ; drug therapy ; genetics