Objective:To explore the screening value of platelet parameters from blood cell analysis for MYH9-related disorders(MYH9-RD).Methods:A cross-sectional study was conducted with 38 patients diagnosed with MYH9-RD at Shenzhen Children's Hospital from May 1, 2016, to August 31, 2024, including 24 males and 14 females; the median age was 11.5 (3.8, 35) years; categorized by gene mutation location into "head region" ( n=8 ) and "tail region" ( n=30); and by clinical manifestations into " isolated hematological manifestations" ( n=16) and "hematological manifestations with extra-hematological involvement"( n=22). The control groups included 39 cases of immune thrombocytopenia (ITP), 38 cases of acute lymphoblastic leukemia (ALL), and 40 healthy individuals. Platelet-related parameters were detected by hematology analyzer, and platelet counts and sizes were confirmed by manually counting and microscopic observation. Kruskal-Wallis test was used to compare platelet parameters between MYH9-RD and control groups. Receiver operating characteristic (ROC) curves were used to assess the diagnostic efficacy of platelet parameters for MYH9-RD. Results:In MYH9-RD patients the median value of mean platelet volume (MPV) was 13.4 (11.2, 14.7) fl, immature platelet fraction (IPF) was 52.7% (43.5%, 58.0%), platelet large cell ratio(PLCR) was 57.6 %(45.0%, 62.9%), and microscopic large platelet ratio (PLCR-M) was 30.0% (25.0%, 30.0%).And those values weresignificantly higher than in ITP, ALL, and healthy controls (all P<0.05). Patients with MYH9 gene "head region" mutations had a lower platelet count [24.5 (15.0, 47.5)×10 9/L]than those with "tail region" mutations [69.0 (49.5, 86.3) ×10 9/L]( Z=-3.493, P<0.001), but a higher IPF ( t=2.024, P=0.044).Patients with "extra-hematological involvement had a lower platelet count than those with "isolated hematological manifestations" ( t=-2.015, P=0.043). The optimal cutoff value for diagnosing MYH9-RD with IPF was 26.7%, with a sensitivity of 100% and specificity of 98.7%; the area under the curve was 0.999 (95% CI 0.995-1.000), which was superior toMPV, PLCR and PLCR-M parameters. Conclusion:IPF is superior to other platelet parameters sush as MPV,showing high diagnostic efficacy in distinguishing MYH9-RD from ITP and ALL. It can be used as a simple and effective indicator for early screening of MYH9-RD.

Objective To observe the impact of low-dose oxycodone-nalbuphine on pain mediator release in patient-controlled intravenous analgesia(PCIA)following laparoscopic radical resection of rectal cancer.Methods 120 patients who underwent laparoscopic radical surgery for colorectal cancer in our hospital from October 2022 to September 2023 were selected and randomly divided into the control group and the experimental group(60 cases in each).PCIA was administered within 48 h postoperatively,formulated as 10 mg hydromorphone and 40 mg nalbuphine diluted in saline to 100 ml.No background dose was administered in the control group,and a background dose of 1 ml/h was administered in the test group.Compare the number of effective PCIA compressions,the rate of remedial analgesia,the rate of remedial antiemesis,the dosage of analgesic pump,Visual Analogue Scale(VAS),Ramsay sedation score,the release of serum pain mediators and the occurrence of postoperative adverse reactions in patients within ≤24 hours and 24-48 hours.Results VAS Scores:The VAS scores during activity in the experimental group at 24 h and 48 h after surgery(2.21±0.25,1.78±0.44)were lower than those in the control group(2.58±0.23,2.12±0.27)(both P＜0.05).The VAS scores at rest in the experimental group at 12 h,24 h,and 48 h after surgery(2.52±0.28,2.14±0.26,1.75±0.32)were lower than those in the control group(3.17±0.39,2.68±0.33,2.04±0.21)(P＜0.05).Pump Usage:The amounts of analgesic pump used in the experimental group at 24 h and 48 h after surgery[(27.64±0.28)ml,(49.11±0.25)ml]were higher than those in the control group[(12.06±0.33)ml,(20.15±0.36)ml)](both P＜0.001).PCIA Pressing Times:The effective pressing times of PCIA in the experimental group within ≤24 h and 24-48 h(5.89±0.31,4.73±0.28)were lower than those in the control group(7.28±0.38,6.21±0.37)(both P＜0.001).Rescue Analgesia and Antiemesis Rates:The rescue analgesia rate(11.67％vs 28.33％,P=0.023)and rescue antiemesis rate(5.00％vs 18.33％,P=0.023)in the experimental group were both lower than those in the control group.Ramsay Sedation Scores:The Ramsay sedation scores in the experimental group at 12 h,24 h,and 48 h after surgery(2.18±0.31,1.74±0.42,1.46±0.14)were lower than those in the control group(2.43±0.48,2.12±0.32,2.03±0.38)(P＜0.001).In the experimental group,substance P[(35.26±5.27)pg/ml,(26.37±4.17)pg/ml],neuropeptide Y[(147.28±27.43)pg/ml,(127.26±18.49)pg/ml],and prostaglandin E2[(56.48±3.69)at 24 hours and 48 hours after the operation 9)The levels of pg/ml,(42.47±5.35)pg/ml and norepinephrine[(3.12±0.48)ng/ml,(1.57±0.19)ng/ml]were all lower than those of the control group[(41.27±6.48)pg/ml,(34.37±4.86)pg/ml,(35.26±5.27 pg/ml,(26.37±4.17)pg/ml,(62.47±4.26)pg/ml,(53.95±4.73)pg/ml,(3.64±0.64)ng/ml,(2.16±0.26)ng/ml,(P＜0.001)].Conclusion Low-dose oxycodone-nalbuphine in patient-controlled intravenous analgesia following laparoscopic radical resection of rectal cancer can improve analgesic effects and reduce serum pain mediator release.

Objective:To explore the feasibility and preliminary clinical experience of robot-assisted pelvic exenteration (PE) in the treatment of locally advanced (LARC) and recurrent (LRRC) rectal cancer.Method:A descriptive case series research method was adopted. Inclusion criteria included: (1) Age 18-80 years old; (2) Preoperative puncture biopsy performed through endoscopy, and a pathological diagnosis of rectal malignant tumor; (3) Preoperative imaging examinations confirming locally advanced (cT4b stage) or locally recurrent rectal cancer, with tumor location in the pelvic cavity; (4) Physical condition: ECOG score ≤1 point, and radical resection being feasible after assessment. The data for five patients with LARC or LRRC who underwent pelvic exenteration (PE) using the da Vinci robotic surgical system in the Department of Anorectal Surgery, the Second Affiliated Hospital of Naval Medical University (Shanghai Changzheng Hospital) from January, 2024 to January, 2025 were collected retrospectively. The mean age was (46.8±7.7) years, with 3 males and 2 females, who comprised 2 cases of LARC and 3 cases of LRRC. Two patients received preoperative radiotherapy, and 4 patients received preoperative chemotherapy. The average body mass index was (21.5±2.7) kg/m2. According to the American Society of Anesthesiologists (ASA) classification, 2 cases were grade II and 3 cases were grade III.Results:All patients with LARC or LRRC successfully underwent robot-assisted PE. The average operation time was (496.4±139.5) minutes; the average intraoperative blood loss was (72.0±29.5) ml; the average postoperative exhaust time was (50.0 ±13.6) hours; and the average postoperative defecation time was(64.2±15.3) hours. Mean early postoperative VAS pain scores was (3.6±1.5) points. Three patients underwent primary intestinal anastomosis, and 2 patients underwent colonic single-lumen ostomy. All 5 patients underwent urinary system reconstruction, among which 2 underwent ureterovesical reimplantation, 1 underwent percutaneous ureterostomy, 1 underwent ileal conduit replacement of bladder, and 1 underwent direct bladder suture. After surgery, except for 1 case of pelvic infection with effusion (Clavien-Dindo grade IIIa), there were no obvious postoperative complications. Postoperative pathological results showed that all patients achieved R0 resection, including 1 case of T4a stage and 4 cases of T4b stage (all involving urogenital organs or tissues), and 3 cases of N0 stage and 2 cases of N1 stage, with a maximum tumor diameter of (4.7±1.9) cm. The median postoperative follow-up time was 11 (range 7 to 17) months, and no patient experienced local recurrence.Conclusion:The above short-term preliminary results of robot-assisted PE in the treatment of LARC or LRRC within the pelvic cavity indicate that it is both safe and feasible.

Objective To observe the impact of low-dose oxycodone-nalbuphine on pain mediator release in patient-controlled intravenous analgesia(PCIA)following laparoscopic radical resection of rectal cancer.Methods 120 patients who underwent laparoscopic radical surgery for colorectal cancer in our hospital from October 2022 to September 2023 were selected and randomly divided into the control group and the experimental group(60 cases in each).PCIA was administered within 48 h postoperatively,formulated as 10 mg hydromorphone and 40 mg nalbuphine diluted in saline to 100 ml.No background dose was administered in the control group,and a background dose of 1 ml/h was administered in the test group.Compare the number of effective PCIA compressions,the rate of remedial analgesia,the rate of remedial antiemesis,the dosage of analgesic pump,Visual Analogue Scale(VAS),Ramsay sedation score,the release of serum pain mediators and the occurrence of postoperative adverse reactions in patients within ≤24 hours and 24-48 hours.Results VAS Scores:The VAS scores during activity in the experimental group at 24 h and 48 h after surgery(2.21±0.25,1.78±0.44)were lower than those in the control group(2.58±0.23,2.12±0.27)(both P＜0.05).The VAS scores at rest in the experimental group at 12 h,24 h,and 48 h after surgery(2.52±0.28,2.14±0.26,1.75±0.32)were lower than those in the control group(3.17±0.39,2.68±0.33,2.04±0.21)(P＜0.05).Pump Usage:The amounts of analgesic pump used in the experimental group at 24 h and 48 h after surgery[(27.64±0.28)ml,(49.11±0.25)ml]were higher than those in the control group[(12.06±0.33)ml,(20.15±0.36)ml)](both P＜0.001).PCIA Pressing Times:The effective pressing times of PCIA in the experimental group within ≤24 h and 24-48 h(5.89±0.31,4.73±0.28)were lower than those in the control group(7.28±0.38,6.21±0.37)(both P＜0.001).Rescue Analgesia and Antiemesis Rates:The rescue analgesia rate(11.67％vs 28.33％,P=0.023)and rescue antiemesis rate(5.00％vs 18.33％,P=0.023)in the experimental group were both lower than those in the control group.Ramsay Sedation Scores:The Ramsay sedation scores in the experimental group at 12 h,24 h,and 48 h after surgery(2.18±0.31,1.74±0.42,1.46±0.14)were lower than those in the control group(2.43±0.48,2.12±0.32,2.03±0.38)(P＜0.001).In the experimental group,substance P[(35.26±5.27)pg/ml,(26.37±4.17)pg/ml],neuropeptide Y[(147.28±27.43)pg/ml,(127.26±18.49)pg/ml],and prostaglandin E2[(56.48±3.69)at 24 hours and 48 hours after the operation 9)The levels of pg/ml,(42.47±5.35)pg/ml and norepinephrine[(3.12±0.48)ng/ml,(1.57±0.19)ng/ml]were all lower than those of the control group[(41.27±6.48)pg/ml,(34.37±4.86)pg/ml,(35.26±5.27 pg/ml,(26.37±4.17)pg/ml,(62.47±4.26)pg/ml,(53.95±4.73)pg/ml,(3.64±0.64)ng/ml,(2.16±0.26)ng/ml,(P＜0.001)].Conclusion Low-dose oxycodone-nalbuphine in patient-controlled intravenous analgesia following laparoscopic radical resection of rectal cancer can improve analgesic effects and reduce serum pain mediator release.

Objective:To explore the feasibility and preliminary clinical experience of robot-assisted pelvic exenteration (PE) in the treatment of locally advanced (LARC) and recurrent (LRRC) rectal cancer.Method:A descriptive case series research method was adopted. Inclusion criteria included: (1) Age 18-80 years old; (2) Preoperative puncture biopsy performed through endoscopy, and a pathological diagnosis of rectal malignant tumor; (3) Preoperative imaging examinations confirming locally advanced (cT4b stage) or locally recurrent rectal cancer, with tumor location in the pelvic cavity; (4) Physical condition: ECOG score ≤1 point, and radical resection being feasible after assessment. The data for five patients with LARC or LRRC who underwent pelvic exenteration (PE) using the da Vinci robotic surgical system in the Department of Anorectal Surgery, the Second Affiliated Hospital of Naval Medical University (Shanghai Changzheng Hospital) from January, 2024 to January, 2025 were collected retrospectively. The mean age was (46.8±7.7) years, with 3 males and 2 females, who comprised 2 cases of LARC and 3 cases of LRRC. Two patients received preoperative radiotherapy, and 4 patients received preoperative chemotherapy. The average body mass index was (21.5±2.7) kg/m2. According to the American Society of Anesthesiologists (ASA) classification, 2 cases were grade II and 3 cases were grade III.Results:All patients with LARC or LRRC successfully underwent robot-assisted PE. The average operation time was (496.4±139.5) minutes; the average intraoperative blood loss was (72.0±29.5) ml; the average postoperative exhaust time was (50.0 ±13.6) hours; and the average postoperative defecation time was(64.2±15.3) hours. Mean early postoperative VAS pain scores was (3.6±1.5) points. Three patients underwent primary intestinal anastomosis, and 2 patients underwent colonic single-lumen ostomy. All 5 patients underwent urinary system reconstruction, among which 2 underwent ureterovesical reimplantation, 1 underwent percutaneous ureterostomy, 1 underwent ileal conduit replacement of bladder, and 1 underwent direct bladder suture. After surgery, except for 1 case of pelvic infection with effusion (Clavien-Dindo grade IIIa), there were no obvious postoperative complications. Postoperative pathological results showed that all patients achieved R0 resection, including 1 case of T4a stage and 4 cases of T4b stage (all involving urogenital organs or tissues), and 3 cases of N0 stage and 2 cases of N1 stage, with a maximum tumor diameter of (4.7±1.9) cm. The median postoperative follow-up time was 11 (range 7 to 17) months, and no patient experienced local recurrence.Conclusion:The above short-term preliminary results of robot-assisted PE in the treatment of LARC or LRRC within the pelvic cavity indicate that it is both safe and feasible.

Objective:To explore the screening value of platelet parameters from blood cell analysis for MYH9-related disorders(MYH9-RD).Methods:A cross-sectional study was conducted with 38 patients diagnosed with MYH9-RD at Shenzhen Children's Hospital from May 1, 2016, to August 31, 2024, including 24 males and 14 females; the median age was 11.5 (3.8, 35) years; categorized by gene mutation location into "head region" ( n=8 ) and "tail region" ( n=30); and by clinical manifestations into " isolated hematological manifestations" ( n=16) and "hematological manifestations with extra-hematological involvement"( n=22). The control groups included 39 cases of immune thrombocytopenia (ITP), 38 cases of acute lymphoblastic leukemia (ALL), and 40 healthy individuals. Platelet-related parameters were detected by hematology analyzer, and platelet counts and sizes were confirmed by manually counting and microscopic observation. Kruskal-Wallis test was used to compare platelet parameters between MYH9-RD and control groups. Receiver operating characteristic (ROC) curves were used to assess the diagnostic efficacy of platelet parameters for MYH9-RD. Results:In MYH9-RD patients the median value of mean platelet volume (MPV) was 13.4 (11.2, 14.7) fl, immature platelet fraction (IPF) was 52.7% (43.5%, 58.0%), platelet large cell ratio(PLCR) was 57.6 %(45.0%, 62.9%), and microscopic large platelet ratio (PLCR-M) was 30.0% (25.0%, 30.0%).And those values weresignificantly higher than in ITP, ALL, and healthy controls (all P<0.05). Patients with MYH9 gene "head region" mutations had a lower platelet count [24.5 (15.0, 47.5)×10 9/L]than those with "tail region" mutations [69.0 (49.5, 86.3) ×10 9/L]( Z=-3.493, P<0.001), but a higher IPF ( t=2.024, P=0.044).Patients with "extra-hematological involvement had a lower platelet count than those with "isolated hematological manifestations" ( t=-2.015, P=0.043). The optimal cutoff value for diagnosing MYH9-RD with IPF was 26.7%, with a sensitivity of 100% and specificity of 98.7%; the area under the curve was 0.999 (95% CI 0.995-1.000), which was superior toMPV, PLCR and PLCR-M parameters. Conclusion:IPF is superior to other platelet parameters sush as MPV,showing high diagnostic efficacy in distinguishing MYH9-RD from ITP and ALL. It can be used as a simple and effective indicator for early screening of MYH9-RD.

Objective:To explore the risk factors for lymphoproliferative disorders (PTLD) in children with thalassemia major (TM) after allogeneic hematopoietic stem cell transplantation (allo-HSCT).Methods:This was a retrospective case-control study.A total of 482 children with TM who underwent allo-HSCT at Shenzhen Children′s Hospital between January 2020 and December 2022 were selected and classified into the PTLD and non-PTLD groups according to the occurrence of PTLD.The risk factors for PTLD after allo-HSCT in children with TM were analyzed, and the diagnostic efficiency of relevant risk factors for PTLD was analyzed by receiver operating characteristic (ROC) curve.Results:A total of 25 out of 482 patients (5.2%, 25/482) developed PTLD about 114 (54-271) days after allo-HSCT.Among them, 12 cases (12/25, 48.0%) occurred within 100 days, and 22 cases (22/25, 88.0%) occurred within 1 year after allo-HSCT.Univariate analysis showed that there were significant differences in gender composition, type of transplant donor, number of natural killer cells and B lymphocytes in peripheral blood at 30 days after allo-HSCT, positive rate of plasma Epstein-Barr virus deoxyribonucleic acid (EBV-DNA) and incidence rate of acute graft-versus-host disease (aGVHD) between the 2 groups (all P<0.05).Multivariate Logistic regression analysis showed that female ( OR=3.196, 95% CI: 1.144-8.929), positive plasma EBV-DNA ( OR=17.523, 95% CI: 5.449-56.344) and aGVHD ( OR=3.156, 95% CI: 1.161-8.575) were independent risk factors for PTLD after allo-HSCT in TM children (all P<0.05).The ROC curve analysis showed that positive plasma EBV-DNA had an excellent accuracy in predicting the occurrence of PTLD after allo-HSCT (sensitivity was 0.796, specificity was 0.800, area under the curve was 0.803).If combined with aGVHD and gender, the area under the curve for the prediction of PTLD increased to 0.831. Conclusions:Female, positive plasma EBV-DNA and aGVHD are independent risk factors for PTLD after allo-HSCT in children with TM.It provides useful early warnings for the prediction and prevention of PTLD.

Objective:To explore the consistency of peripheral whole blood and venous serum procalcitonin (PCT) levels, and the value of peripheral whole blood PCT in evaluating pediatric bacterial infection.Methods:This multicenter cross-sectional parallel control study was conducted in 11 children′s hospital. All the 1 898 patients older than 28 days admitted to these hospitals from March 2018 to February 2019 had their peripheral whole blood and venous serum PCT detected simultaneously with unified equipment, reagent and method. According to the venous serum PCT level, the patients were stratified to subgroups. Analysis of variance and chi-square test were used to compare the demographic characteristics among groups. And the correlation between the peripheral blood and venous serum PCT level was investigated by quantitative Pearson correlation analysis.The PCT resultes were also converted into ranked data to further test the consistency between the two sampling methods by Spearman′s rank correlation test. Furthermore, the ranked data were converted into binary data to evaluate the consistency and investigate the best cut-off of peripheral blood PCT level in predicting bacterial infection.Results:A total of 1 898 valid samples were included (1 098 males, 800 females),age 27.4(12.2,56.7) months. There was a good correlation between PCT values of peripheral whole blood and venous serum ( r=0.97 , P<0.01). The linear regression equation was PCT?venous serum=0.135+0.929×PCT peripheral whole blood. However, when stratified to 5 levels, PCT results showed diverse and unsatisfied consistency between the two sampling methods ( r=0.51-0.92, all P<0.01). But after PCT was converted to ordinal categorical variables, the stratified analysis showed that the coincidence rate of the measured values by the two sampling methods in each boundary area was 84.9%-97.1%. The dichotomous variables also showed a good consistency (coincidence rate 96.8%-99.3%, Youden index 0.82-0.89). According to the severity of disease, the serum PCT value was classified into 4 intervals（<0.5、0.5-<2.0、2.0-<10.0、≥10.0 μg/L）, and the peripheral blood PCT value also showed a good predictive value (AUC value was 0.991 2-0.997 9). The optimal cut points of peripheral whole blood PCT value 0.5、1.0、2.0、10.0 μg/L corresponding to venous serum PCT values were 0.395, 0.595, 1.175 and 3.545 μg/L, respectively. Conclusions:There is a good correlation between peripheral whole blood PCT value and the venous serum PCT value, which means that the peripheral whole blood PCT could facilitate the identification of infection and clinical severity. Besides, the sampling of peripheral whole blood is simple and easy to repeat.