Creutzfeldt-Jakob disease(CJD)is a rapidly progressive and fatal neurodegenerative disorder caused by prions,with certain infectivity and iatrogenic transmission risks.With the rapid progress and application of new dia-gnostic biomarkers and detection methods,as well as the construction and improvement of surveillance and reporting systems,the detection of CJD in patients domestically and internationally has shown an increasing trend year by year.Due to its long incubation period and heterogeneity of early symptoms,early identification and diagnosis of the disease is difficult,increasing the risk of transmission within medical institutions.Currently,there is a lack of con-sensus on the infection prevention and control of CJD.In order to timely identify and diagnose CJD as well as effec-tively block its transmission in medical institutions,this consensus summarizes 15 clinical concerns and formulates 24 specific recommendations based on the latest domestic and international research findings and clinical evidence,as well as combines with clinical practice,aiming to standardize healthcare-associated infection prevention and control measures for CJD and reduce its transmission risk in medical institutions.

Creutzfeldt-Jakob disease(CJD)is a rapidly progressive and fatal neurodegenerative disorder caused by prions,with certain infectivity and iatrogenic transmission risks.With the rapid progress and application of new dia-gnostic biomarkers and detection methods,as well as the construction and improvement of surveillance and reporting systems,the detection of CJD in patients domestically and internationally has shown an increasing trend year by year.Due to its long incubation period and heterogeneity of early symptoms,early identification and diagnosis of the disease is difficult,increasing the risk of transmission within medical institutions.Currently,there is a lack of con-sensus on the infection prevention and control of CJD.In order to timely identify and diagnose CJD as well as effec-tively block its transmission in medical institutions,this consensus summarizes 15 clinical concerns and formulates 24 specific recommendations based on the latest domestic and international research findings and clinical evidence,as well as combines with clinical practice,aiming to standardize healthcare-associated infection prevention and control measures for CJD and reduce its transmission risk in medical institutions.

Objective:To investigate the clinical features of the initial phase of acute osteomyelitis in children with skin and soft tissue infection as the main sign.Methods:The clinical data of 154 children with skin and soft tissue infections as the main sign from July 2017 to February 2023 were retrospectively analyzed. According to MRI, 48 children with no signs of osteomyelitis and only simple skin and soft tissue infection were included in the non-osteomyelitis group, including 28 boys and 20 girls, aged 38.50 (12.00, 93.00) months; 106 children with acute osteomyelitis with skin and soft tissue infection as the main sign were included in the osteomyelitis group, including 65 boys and 41 girls, aged 49.50 (17.50, 87.00) months. The disease course, maximum body temperature at onset, inflammatory indicators (including white blood cell count, neutrophil count, C-reactive protein, erythrocyte sedimentation rate) examined within 24 h after admission were compared between the two groups, and receiver operating characteristic (ROC) curve was used to evaluate the efficacy of each index in diagnosing osteomyelitis.Results:The white blood cell counts in the non-osteomyelitis group and osteomyelitis group were 13.72 (10.19, 19.19) ×10 9 /L and 14.74 (10.63, 18.67) ×10 9 /L, and the neutrophil counts were 7.79 (5.62, 11.91) ×10 9 /L and 9.58 (5.77, 13.67) ×10 9 /L, the difference was not statistically significant ( Z=-0.68, P=0.495; Z=-1.24, P=0.216). The course of disease in the non-osteomyelitis group and osteomyelitis group was 5.00 (3.00, 7.00) d and 5.50 (4.00, 9.00) d ( Z=-2.03, P=0.042), and the maximum body temperature at the onset of the disease was 38.50 (36.65, 39.00) ℃ and 39.00 (38.50, 40.00) ℃ ( Z=-3.72, P<0.001), C-reactive protein was 23.26 (8.16, 47.67) mg/L and 69.27 (26.28, 111.03) mg/L ( Z=-4.52, P<0.001), erythrocyte sedimentation rate was 35.00 (24.25, 53.00) mm/1 h and 61.00 (43.00, 78.00) mm/1 h ( Z=-5.06, P<0.001), the differences were statistically significant. The proportion of patients with increased C-reactive protein was 70.8% (34/48) and 92.5% (98/106) in non-osteomyelitis group and osteomyelitis group, the proportion of patients with increased erythrocyte precipitation rate was 81.3% (39/48) and 100% (106/106), and the proportion of patients with fever was 66.7% (32/48) and 100% (106/106), respectively, the difference was statistically significant (χ 2=12.61, P<0.001; χ 2=21.11, P<0.001; χ 2=39.43, P<0.001). The sensitivity, specificity and area under the curve of osteomyelitis were 84.0%, 33.3% and 0.602, respectively. The maximum body temperature at onset was 99.1%, 35.4% and 0.687, and the C-reactive protein was 57.6%, 85.4% and 0.728, respectively. Erythrocyte sedimentation rates were 84.0%, 56.3% and 0.755, respectively. Multivariate logistic regression analysis indicated that the maximum body temperature was >37.6 ℃ [ OR=22.54, 95% CI (2.66, 190.81)] and C-reactive protein was >54.59 mg /L [ OR=4.23, 95% CI (1.63, 11.01)] was an independent risk factor for predicting osteomyelitis with skin and soft tissue infection as the main sign. Conclusion:Compared to simple skin and soft tissue infections, children with osteomyelitis had a higher proportion of fever, elevated C-reactive protein, and elevated erythrocyte sedimentation rate, a longer duration of illness, and higher elevations in temperature, C-reactive protein, and erythrocyte sedimentation rate. Length of onset, maximum body temperature at onset, C-reactive protein and erythrocyte sedimentation rate had certain diagnostic efficacy in determining the tendency of skin soft tissue infection to osteomyelitis. Maximum body temperature >37.6 ℃ and C-reactive protein >54.59 mg/L may independently predict the possibility of skin soft tissue infection as osteomyelitis, and prompt Magnetic Resonance Imaging is recommended for early diagnosis and treatment in such children.

OBJECTIVE: To analyze the clinical and genetic characteristics of a child with mucopolysaccharidoses type I. METHODS: Enzymatic and genetic testing were carried out for the child who was admitted due to contraction of fingers and flexion deformity of lower limbs. The child was subjected to target exome capture sequencing. Candidate variants were verified by Sanger sequencing of the child, her parents and two sisters. RESULTS: The child had featured facial dysmorphism, short stature, round head, short neck, corneal turbidity and skeletal deformity. Enzyme test was positive, and genetic testing revealed that she had harbored c.1049delA (p.N350Mfs*4) and c.1815dupT (p.V606Cfs*53) compound heterozygous variants of the IDUA gene, which were inherited from her mother and father, respectively. Her two sisters had each carried one of above variants. c.1815dupT was known to be pathogenic, whilst c.1049delA was not reported in Human Gene Mutation Database. CONCLUSION The compound heterozygous variants of the IDUA gene probably underlay the disease in this child, among which the c.1049delA (p.N350Mfs*4) is unreported previously.
Child ; Dwarfism ; Female ; Genetic Testing ; Humans ; Iduronidase ; Mucopolysaccharidosis I ; Mutation ; Exome Sequencing

Objective:To observe the changes of plasma nitric oxide (NO) and desmosine (DES) concentrations in animals under different decompression pressure loads, and to explore the role of NO and DES in quantitative evaluation of diving decompression injury.Methods:A total of 50 male SD rats were divided into five groups with 10 rats in each group by the random table method. Rats in Group 1 were not pressurized and were given air ventilation, and left the cabin after 120 min; after pressurized to 70 m for 70 min, 40 rats in the other four groups were decompressed at a constant rate to the normal pressure in 40 min (Group 2), 30 min (Group 3), 20 min (Group 4) and 10 min (Group 5), respectively. The changes of plasma NO and DES concentrations in rats after leaving the cabin were observed. A total of 40 male rabbits were divided into four groups with 10 rabbits in each group by the random table method. The rabbits were used to simulate fast buoyancy ascent escape from the depth of 150 m and the compression pressure doubled every four seconds. The four groups were decompressed at a constant rate of 6 m/s after being given different bottom time, i. e., 4 s (Group 1), 60 s (Group 2), 180 s (Group 3), and 300 s (Group 4). The changes of plasma NO and DES concentrations in the rabbits after leaving the cabin were analyzed.Results:Compared with the rats in the Group 1, the plasma NO and DES concentrations of the rats of the Group 5 significantly increased, with statistically significant differences ( P<0.05 or P<0.01), and the plasma DES concentration of the rats in the Group 4 also increased, with a statistically significant difference ( P<0.05). The plasma NO and DES concentrations in rats were positively correlated with the reciprocal of the decompression time at a constant rate (NO: r=0.683, P<0.01; DES: r=0.535, P<0.01) and showed significant linear regression relationships (NO: r2=0.467, P<0.01; DES: r2=0.287, P<0.01). Compared with the values before escape, the plasma NO concentrations in the rabbits of the Group 3 and 4 increased significantly, with statistically significant differences ( P<0.01), and the plasma DES concentrations in all four rabbit groups increased significantly, with statistically significant differences ( P<0.01). After leaving the cabin, compared with the rabbits in the Group 1, the plasma NO and DES concentrations in rabbits of the Group 3 and 4 increased significantly, with statistically significant differences (NO: P<0.05 or P<0.01; DES: P<0.01). After leaving the cabin, the plasma NO and DES concentrations in rabbits were positively correlated with the bottom time (NO: r=0.672, P<0.01; DES: r=0.702, P<0.01)and showed significant linear relationships (NO: r2=0.452, P<0.01; DES: r2=0.493, P<0.01). Conclusion:The plasma NO and DES concentrations can quantitatively indicate the damage caused by decompression pressure loads, which makes it possible to design decompression protocol and safety evaluation. The severity of decompression injuries is linearly related to the reciprocal of decompression time (or rate) that determines the decompression pressure load, which have reference value for the decompression rate control in the theoretical model of decompression.

Objective:To observe the changes of plasma nitric oxide (NO) and desmosine (DES) concentrations in animals under different decompression pressure loads, and to explore the role of NO and DES in quantitative evaluation of diving decompression injury.Methods:A total of 50 male SD rats were divided into five groups with 10 rats in each group by the random table method. Rats in Group 1 were not pressurized and were given air ventilation, and left the cabin after 120 min; after pressurized to 70 m for 70 min, 40 rats in the other four groups were decompressed at a constant rate to the normal pressure in 40 min (Group 2), 30 min (Group 3), 20 min (Group 4) and 10 min (Group 5), respectively. The changes of plasma NO and DES concentrations in rats after leaving the cabin were observed. A total of 40 male rabbits were divided into four groups with 10 rabbits in each group by the random table method. The rabbits were used to simulate fast buoyancy ascent escape from the depth of 150 m and the compression pressure doubled every four seconds. The four groups were decompressed at a constant rate of 6 m/s after being given different bottom time, i. e., 4 s (Group 1), 60 s (Group 2), 180 s (Group 3), and 300 s (Group 4). The changes of plasma NO and DES concentrations in the rabbits after leaving the cabin were analyzed.Results:Compared with the rats in the Group 1, the plasma NO and DES concentrations of the rats of the Group 5 significantly increased, with statistically significant differences ( P<0.05 or P<0.01), and the plasma DES concentration of the rats in the Group 4 also increased, with a statistically significant difference ( P<0.05). The plasma NO and DES concentrations in rats were positively correlated with the reciprocal of the decompression time at a constant rate (NO: r=0.683, P<0.01; DES: r=0.535, P<0.01) and showed significant linear regression relationships (NO: r2=0.467, P<0.01; DES: r2=0.287, P<0.01). Compared with the values before escape, the plasma NO concentrations in the rabbits of the Group 3 and 4 increased significantly, with statistically significant differences ( P<0.01), and the plasma DES concentrations in all four rabbit groups increased significantly, with statistically significant differences ( P<0.01). After leaving the cabin, compared with the rabbits in the Group 1, the plasma NO and DES concentrations in rabbits of the Group 3 and 4 increased significantly, with statistically significant differences (NO: P<0.05 or P<0.01; DES: P<0.01). After leaving the cabin, the plasma NO and DES concentrations in rabbits were positively correlated with the bottom time (NO: r=0.672, P<0.01; DES: r=0.702, P<0.01)and showed significant linear relationships (NO: r2=0.452, P<0.01; DES: r2=0.493, P<0.01). Conclusion:The plasma NO and DES concentrations can quantitatively indicate the damage caused by decompression pressure loads, which makes it possible to design decompression protocol and safety evaluation. The severity of decompression injuries is linearly related to the reciprocal of decompression time (or rate) that determines the decompression pressure load, which have reference value for the decompression rate control in the theoretical model of decompression.

Objective To investigate the effect of simulated 400 m He-O2 saturation diving on the pulmonary oxidative-antioxidative system in rats.Methods SD rats were exposed to 4.1 MPa He-O2 mixture for 24 h in an animal chamber. Normal air pressure was used in the control group .The histopathology , contents of malondialdehyde ( MDA) , 8-hydroxy-2′-deoxyguanosine(8-OHdG),and glutathione(GSH) and activities of superoxide dismutase (SOD) and glutathione peroxi-dase( GSH-Px) in pulmonary tissues were determined .Results No difference was found during pathological examination or in MDA, 8-OHdG and SOD activity.GSH and GSH-Px activity were decreased significantly (P<0.05) after hyperbaric exposure.Conclusion Exposure to 4.1 MPa He-O2 for 24 h may promote oxidative stress and reduce antioxidative capacity rather than cause obvious oxidative damage in rat lungs .