Objective:To detect pathogenic gene mutations in a patient with CARD14-associated papulosquamous eruption (CAPE) and his family, and to evaluate the short-term and long-term efficacy and safety of ustekinumab treatment.Methods:Clinical data were collected from a 6-year-old child presenting with erythroderma secondary to pityriasis rubra pilaris and from his family members. Peripheral blood samples were collected from the patient, his parents and younger brother, and DNA was extracted from these peripheral blood samples. A next-generation skin-targeted sequencing panel was used to detect gene mutations, and Sanger sequencing was performed for verification. Genomic DNA was also extracted from 100 unrelated healthy controls using the same method.Results:A heterozygous mutation c.349+1G>A in intron 6 of the CARD14 gene was identified in the proband and his mother, but was not identified in his father, younger brother or 100 healthy controls, leading to a diagnosis of CAPE in the proband. The patient received subcutaneous injections of ustekinumab at a dose of 45 mg at weeks 0 and 4, and every 12 weeks thereafter. After 1-week treatment, the skin lesions and symptoms improved markedly. No adverse events were reported during the 18-month treatment and follow-up.Conclusion:The heterozygous mutation c.349+1G>A in the CARD14 gene may be the cause of the disease in this family, and ustekinumab was effective and safe for the treatment of CAPE.

Objective:To detect pathogenic gene mutations in a patient with CARD14-associated papulosquamous eruption (CAPE) and his family, and to evaluate the short-term and long-term efficacy and safety of ustekinumab treatment.Methods:Clinical data were collected from a 6-year-old child presenting with erythroderma secondary to pityriasis rubra pilaris and from his family members. Peripheral blood samples were collected from the patient, his parents and younger brother, and DNA was extracted from these peripheral blood samples. A next-generation skin-targeted sequencing panel was used to detect gene mutations, and Sanger sequencing was performed for verification. Genomic DNA was also extracted from 100 unrelated healthy controls using the same method.Results:A heterozygous mutation c.349+1G>A in intron 6 of the CARD14 gene was identified in the proband and his mother, but was not identified in his father, younger brother or 100 healthy controls, leading to a diagnosis of CAPE in the proband. The patient received subcutaneous injections of ustekinumab at a dose of 45 mg at weeks 0 and 4, and every 12 weeks thereafter. After 1-week treatment, the skin lesions and symptoms improved markedly. No adverse events were reported during the 18-month treatment and follow-up.Conclusion:The heterozygous mutation c.349+1G>A in the CARD14 gene may be the cause of the disease in this family, and ustekinumab was effective and safe for the treatment of CAPE.

Objective:To investigate the application value of computer-assisted preoperative planning (CAPP) in the treatment of AO/OTA type-C distal femoral fractures.Methods:A retrospective analysis was conducted on 150 patients with AO/OTA type-C distal femur fractures from January 2010 to December 2021 using the less invasive stabilization system-distal femur (LISS-DF). Patients were divided into a non-CAPP group and a CAPP group based on whether computer-assisted preoperative planning was utilized. The non-CAPP group included 81 patients (56 males, 25 females) with an age range of 39 to 67 years (mean 54.9±5.8 years), consisting of 22 type-C1 fractures, 35 type-C2 fractures, and 24 type-C3 fractures. The CAPP group comprised 69 patients (50 males, 19 females) with an age range of 45 to 63 years (mean 53.9±4.6 years), including 18 type-C1 fractures, 28 type-C2 fractures, and 23 type-C3 fractures. The study recorded the time of preoperative planning and compared the surgical duration, intraoperative blood loss, times of fluoroscopy intraoperatively, and hospital stay length between the two groups. At the last follow-up, knee function was evaluated using the Hospital for Special Surgery (HSS) knee score and pain was assessed using the visual analogue scale (VAS).Results:The preoperative planning time of CAPP was 22.5±3.4 min (range, 17-31 min). There were statistically significant differences between non-CAPP group and CAPP group in terms of surgical duration (non-CAPP, 110.9±7.7 min; CAPP, 94.4±6.3 min), intraoperative blood loss (non-CAPP, 299.3±34.2 ml; CAPP, 224.1±22.0 ml), times of fluoroscopy intraoperatively (non-CAPP, 11.3±3.1; CAPP, 6.7±2.2), and hospital stay length (non-CAPP, 12.8±3.2 d; CAPP, 6.4±1.9 d) ( P<0.001). All patients were followed up, with the CAPP group having a follow-up duration of 17.7±3.3 months and the non-CAPP group having 18.1±3.7 months. Both groups of patients achieved clinical healing of fractures. The healing time for fractures in the CAPP group and the non-CAPP group were 13.9±1.1 weeks and 14.0±1.3 weeks, respectively, with no statistically significant difference ( t=0.699, P=0.490). At the last follow-up, the average HSS score and VAS score of the patients in the CAPP group were 86.6±3.4 points and 0.9±0.4 points, which were both better than those of the non-CAPP group 79.2±5.1 points and 1.3±0.5 points ( P<0.001). No patients in either group experienced complications related to delayed fracture healing, nonunion, or internal fixation failure. Conclusion:When using LISS -DF steel plate to treat complex distal femoral fractures, CAPP can assist the operator in efficiently and accurately completing preoperative design. Compared with traditional preoperative planning, it can reduce surgical time, surgical trauma, and achieve more satisfactory postoperative outcomes.

Objective:To summarize the clinical features of out-of-hospital extracorporeal cardiopulmonary resuscitation(ECPR) patients and analyze the factors associated with discharge survival.Methods:The data of 54 patients with ECPR in the Emergency Center of the Second Hospital Affiliated to Zhejiang University School of Medicine from January 2020 to December 2023 were analyzed. Patients were divided into the survival group and death group based on their survival status within 30 days of discharge. Kaplan Meier method was used to draw the survival curve, and COX regression analysis was used to analyze the relevant factors of survival.Results:(1) A total of 54 patients with ECPR were included in this study. 17 (31.5%) cases were discharged alive, of which 10 (58.8%) had a good neurological prognosis, while 37 case (68.5%) died.(2) Kaplan-Meier survival analysis showed that patients who received first-witness CPR, had a lower-extremity perfusion line established, underwent IABP, achieved ROSC, had normal pH, and had an initial pupillary light reflex had a longer median survival time ( P < 0.05). (3) Univariate COX regression analysis showed that statistically differences in survival time among patients with ECMO times, hypoperfusion times, initial pupil sizes, and APACHEⅡ scores ( P < 0.05); (4) Multivariate COX regression analysis showed that APACHEⅡ score, initial pupil size, and IABP were independent predictors of survival of patients discharged from the hospital with emergency ECPR; (5) The concordance index of the COX regression model was 0.921. Conclusions:The survival model demonstrates good predictive accuracy. Healthcare professionals can use the APACHEⅡscore and initial pupil size on admission as important reference indicators to assist in determining patient prognosis, and to timely initiate IABP treatment, in conjunction with the patient’s condition, to improve the survival rate of patients discharged from the hospital.

Objective:To analyze the application value of three-dimensional computed tomography (3-D CT) reconstruction technique in the evaluation of related predictive parameters of osteoporotic hip fractures.Methods:From February 2014 to March 2019, 106 patients with hip fracture who underwent dual-energy X-ray bone density test of T≤ -2.5 SD on the hip were analyzed retrospectively. There were 58 males and 48 females; Age 73.50±5.59 years old (range, 65-88 years old); Height 170.12±7.43 cm (range, 152-187 cm); Weight (69.26±11.85) kg (range, 37-94 kg); Body mass index 23.60±2.37 kg/m 2 (range, 18.3-28.9 kg/m 2); There were 32 cases of femoral neck fractures, 68 cases of intertrochanteric fractures and 6 cases of subtrochanteric fractures. Anatomic parameters of the hip were the cortical thickness index (CTI), hip axis length (HAL), and femoral offset (FO). The above-mentioned predictive parameters of the hip fractures were measured based on X-ray and 3-D CT reconstruction images. Comparison of the two methods were analyzed using paired t test, liner Pearson analysis, and Bland-Altman analysis. Pearson analysis was used to evaluate correlation between CTI, HAL, and FO and T value. Results:106 cases were compared and analyzed with the measured values of X-ray and 3-D CT reconstruction images according to the preset measurement scheme. The mean value of CTI measured by conventional X-ray measurement method was 0.31±0.05, and the mean value of CTI measured by 3-D CT reconstruction images was 0.31±0.05. The result was not statistically significant ( t=0.938, P=0.35). The mean values of HAL, FO measured by conventional X-ray measurement method were 123.35±12.74 mm, 43.99±2.81 mm, and the mean values of HAL, FO measured by 3-D CT reconstruction images were 121.11±14.51 mm, 40.73±3.11 mm. The results were statistically different ( t=2.578, 18.502; all P< 0.05). The Bland-Altman results showed that there was no difference in the consistency of the two measurement methods for CTI while the measurement results of HAL, FO were quite different. CTI and HAL showed strong correlations with the T scores respectively ( r=0.784, -0.748; P< 0.001). Conclusion:Based on 3-D CT reconstruction technique, accurate measurement of related predictive parameters of osteoporotic hip fractures can be realized, which provides theoretical basis for the evaluation of osteoporotic hip fractures in the elderly and the formulation of accurate rehabilitation treatment plan.

Objective:To investigate the application value of three-dimensional morphological measurement in the treatment of displaced intra-articular calcaneal fractures in the elderly.Methods:A retrospective case series study was conducted to analyze the clinical data of 44 patients with displaced intra-articular calcaneal fractures admitted to East Hospital Affiliated to Tongji University from January 2015 to January 2018. There were 33 males and 11 females, aged 60-88 years (mean, 67.2 years). According to Sanders classification, 35 patients were with type II fractures and 9 with type III fractures. All patients were evaluated with digital technology and received open reduction and internal fixation. The operation time, intraoperative blood loss, intraoperative fluoroscopy frequency and complications were recorded. Three-dimensional morphological parameters of calcaneal fractures were compared before and at latest follow-up, including horizontal length of the calcaneus, height of the posterior articular facet, height and length of the anterior process, Gissane angle, and B?hler angle. The visual analogue scale (VAS) and American Orthopedic Foot and Ankle Society (AOFAS) ankle-hind foot function score were also compared.Results:All patients were followed up for 12-30 months (mean, 17.1 months). There were 31 patients with posterior articular facet depression more than 2 mm, and 13 patients showed the back shift of the whole or part of posterior articular facet more than 3 mm and varying degree of fracture of the upper or lateral wall part of calcaneus body. The operative time was 55-105 minutes (mean, 85.1 minutes). The intraoperative blood loss was 30-130 ml (mean, 74.9 ml). The intraoperative fluoroscopy frequency was 5-8 times (mean, 6.5 times). During the operation, the internal fixation was not changed. Two patients suffered from skin incision infection after operation. At latest follow-up, the horizontal length of the calcaneus, height of the posterior articular facet, Gissane angle and B?hler angle were significantly improved from that before operation [(76.9±4.1)mm vs. (75.5±5.2)mm, (24.2±2.1)mm vs. (22.5±2.9)mm, (29.0±6.2)° vs. (18.3±5.5)°, (124.8±7.2)° vs. (107.6±8.5)°, respectively] ( P<0.01). No significance was found in the height and length of the anterior process ( P>0.05). At latest follow-up, the VAS and AOFAS ankle-hind foot function score were also significantly improved compared to preoperation [(1.1±0.9)points vs. (4.4±0.9)points, (86.4±6.2)points vs. (49.9±6.3)points, respectively] ( P<0.01). AOFAS ankle-hind foot function score was excellent in 13 patients, good in 26, fair in 5, with the excellent and good rate of 87%. Conclusion:Three-dimensional morphological measurement can fully evaluate the damage of calcaneal fractures and provide data reference for the preoperative planning of open reduction and internal fixation in the elderly, so as to restore the calcaneus structure, relieve pain and improve ankle function.

Objective: evaluate the efficacy of a single medial locking plate and lag screws based on computer-assisted pre-operation plan (CAPP) for the treatment of pilon fractures. Methods: Between 2013 and 2016, data of 27 patients with pilon fractures who were treated using a single medial locking and lag screws based on CAPP were retrospectively analyzed. All the cas-es were AO/OTA 43-C type pilon fractures. According to the Rüedi-Allgöwer classification, there were 5 cases of type Ⅱ and 22 cases of type Ⅲ. Among 27 patients, 19 patients had fibula fracture. CAPP was performed for all fractures, including simulated re-duction, virtual surgery, and implantation of internal fixation. The time of CAPP, intraoperative realization of preoperative plan, op-eration time, intraoperative blood loss, and pre- and post-operative range of motion of ankle were recorded. Functional outcomes at final follow up were assessed using American Orthopedic Foot and Ankle Society (AOFAS) ankle hindfoot scale, visual analogue scale (VAS). Results: The mean time of CAPP was 25.8 minutes (range, 14-30 minutes). The average operating time was 138.5 minutes. The average intraoperative blood loss was 225.9 ml. All the twenty-seven patients were followed up for an average period of 18.6 months. The average AOFAS score, VAS score at the final follow-up was 82.9, 2.7 points respectively, which was signifi-cantly improved compared with those before operation (t=-19.358, 7.720; all P< 0.001). Conclusion CAPP system is convenient and efficient, which can facilitate the treatment of type C pilon fractures with a single medial locking plate and lag screws. In addi-tion, satisfactory clinical outcomes can be obtained.

Objective To explore the effect of early enteral nutritional (EEN) support rate of reaching the standard on the prognosis of mechanical ventilation (MV) patients with fulminant myocarditis. Methods The clinical data of 17 MV patients with fulminant myocarditis admitted to Intensive Care Unit (ICU) of Yinzhou Hospital Affiliated to Ningbo University Medical College from February 11, 2015 to May 15, 2018 were analyzed retrospectively, and according to whether the 60% calculated nutritional target value of early enteral nutrition (EEN) was achieved within 7 days of treatment or not, they were divided into an EEN support standard group (10 cases) and a non-standard group (7 cases). The clinical data of MV time, length of stay in ICU, total hospitalization time, acute physiology and chronic health evaluation Ⅱ (APACHE Ⅱ) score and albumin (Alb) and prealbumin (PA) on the date of entering into ICU and on the date getting out of ICU were collected in the two groups, the difference of above indexes were compared between the two groups. Results The MV time, length of stay in ICU and the total hospitalization time in EEN support standard group were obviously shorter than those in EEN support non-standard group [MV time (hours): 93.59±32.11 vs. 131.07±45.34, length of stay in ICU (days): 14.78±5.24 vs. 19.21±6.78, total hospitalization stay (days): 21.28±5.62 vs. 27.19±4.82, all P < 0.05]. In comparisons between the two groups, the APACHE Ⅱ scores on discharge from ICU and the difference values in Alb, PA respectively between levels on date entering into ICU and getting out of ICU were of no statistical significant differences [APACHE Ⅱ score out of ICU: 6.72±2.14 vs. 7.21±2.15, Alb difference value between levels entering into ICU and getting out of ICU (g/L): 3.59±2.23 vs. 4.18±1.93, PA difference value as above mentioned (mg/L): 20.81±12.13 vs. 16.07±17.34, all P > 0.05]. Conclusion The standard EEN support for patients with acute fulminant myocarditis undergoing MV can shorten MV duration, length of stay in ICU and total hospitalization time.

Objective To observe the effect of early enteral nutrition (EN) on immune function in patients with severe acute pancreatitis (SAP). Methods Nineteen patients with SAP admitted to Yinzhou People's Hospital of Ningbo City from March 11, 2015 to December 16, 2016 were enrolled, they were divided into two groups according to the different times of EN given, 11 patients who were early supported with EN were assigned in the research group and another 8 patients whose EN support was delayed were included in the control group. The patients in two groups were treated with routine non-operative western medicine after admission, and the jejunal nutritional tube was placed in the nasal cavity for EN administration. The research group was given early EN beginning at 72 hours after admission, on the first day, 250 mL sugar saline was administered at a rate of 60 mL/h, and on the second day and afterward, it was changed to 200 mL Ruineng (a commercial kind of EN); in the control group, the EN began on 7 to 10 days after admission, using the same principle and method as the research group; EN was given for 3 weeks in both groups. Venous blood was collected from each patient before and after the EN support, and immunoglobulin (IgG, IgM and IgA) levels were determined by immunoturbidimetry, the time of improved Marshall score ＜ 1 was observed. Results The levels of immunoglobulin IgG (g/L: 11.13±2.56 vs. 8.17±1.12), IgM (g/L: 1.71±0.96 vs. 0.76±0.71) and IgA (g/L: 3.74±1.85 vs. 2.13±0.13) in the research group after treatment were significantly higher than those before treatment (all P ＜ 0.05);the changes in the above indicators before and after treatment in the control group were not obvious [IgG (g/L): 8.32±0.93 vs. 8.21±1.04, IgM (g/L): 0.87±0.73 vs. 0.81±0.66, IgA (g/L): 2.15±0.37 vs. 2.11±0.17]. The levels of IgG, IgM and IgA in the research group after treatment were significantly higher than those in the control group (all P ＜ 0.05), the time of Marshall score ＜ 1 was siginificantly shorter than the research group than that in control group (days: 12.31±1.27 vs. 16.18±1.13, P ＜ 0.05). Conclusion Administration of EN as early as possible can effectively enhance the immune function of patients with SAP and improve their prognosis.

Objective To compare the efficacy of early and delayed removal of debridement implant for infection after internal fixation of tibial fracture.Methods A retrospective case control study was conducted on the clinical data of 27 patients with tibial fractures who received plate or screw internal fixation admitted to the East Hospital Affiliated to Tongji University from March 2005 to September 2016.There were 21 males and six females,aged 18-81 years [(41.6 ± 14.3)years].According to the treatment methods,the patients were divided into the delayed implant removal group (Group A,10 patients) and the early implant removal group (Group B,17 patients).Group A was given debridement and anti infection treatment followed by continuous dressing change,and the implant was removed after the fractures were healed.Group B was given debridement and implant removal after one month of anti infection treatment and continuous dressing change when the infection was not clearly controlled.Patients with stable fracture ends were given only negative pressure closed drainage (VSD),and those with instable fracture ends were given external fixation and VSD.The time from infection to implant removal,the time of infection control,the fracture nonunion rate,the chronic bone infection rate,the knee joint function score of the American Hospital for Special Surgery (HSS),and the American Orthopedic Foot and Ankle Society (AOFAS) ankle hindfoot scale were compared between the two groups.Results All patients were followed up for 13-47 months,with the average of 28.4 months.There were significant differences between Group A and Group B in terms of the time from infection to implant removal [(49.9 17.1) weeks ∶ (19.3 ± 9.2) weeks],the time of infection control [(85.3 ±78.3)days∶ (6.3 ±2.8)days],fracture nonunion rate (30％ ∶ 0),and the chronic osteomyelitis incidence (30％ ∶ 0) (all P ＜0.05).No significant differences were found in HSS knee joint function score and AOFAS ankle hindfoot scale between the two groups (both P ＞ 0.05).Conclusion For patients with postoperative infection after internal fixation for tibial fracture,early thorough debridement and implant removal can quickly control the infection and reduce the incidence of nonunion and osteomyelitis.