This paper reports the clinical characteristics and ZNF526 gene mutation in a child with Dentici-Novelli neurodevelopmental syndrome, who was admitted to the Department of Pediatrics at the First Affiliated Hospital of Henan University of Traditional Chinese Medicine in June 2021, and reviews the relevant literature.The male child, 5 years and 1 month old, presented with distinctive facial features, intellectual and motor retardation, language development delays, scattered Mongolian spots on the back and buttocks, brain tissue involvement, visual impairment, and epilepsy.The whole-exome sequencing of the family revealed that the child suffered compound heterozygous mutations, c.1430G>T and c. 475C>T, in the ZNF526 gene.At present, 7 mutation sites in the ZNF526 gene have been reported globally to be associated with neurodevelopmental disorders, and the 2 mutations in this case have not been reported in the literature, broadening the phenotype and mutation spectrum of this disease.

Objective:To investigate the factors that influence the occurrence of complications following deep inferior epigastric perforator (DIEP) flap breast reconstruction, and to determine whether these complications have an impact on the patient's quality of life.Methods:The clinical and follow up data from patients who underwent DIEP flap breast reconstruction at the Department of Breast, Affiliated Cancer Hospital of Zhengzhou University from Dec 2019 to Mar 2023 were retrospectively analyzed.Results:A total of 85 patients underwent DIEP flap breast reconstruction, including 71 stage Ⅰ reconstructions and 14 stage Ⅱ reconstructions. Postoperative complications occurred in 22 cases 25.9%, including flap complications in 11 cases (12.9%) and abdominal donor site complications in 11 cases (12.9%). Univariate and multivariate analyses showed that the overall incidence of complications was associated with high BMI and early surgery ( P<0.05). Flap complications were associated with high BMI, surgery performed at initial phase, and the use of internal mammary vascular branches as recipient vessels ( P<0.05). Abdominal complications were associated with previous abdominal surgery scars ( P<0.05). BREAST-Q scores showed no significant differences between the surgical complication group and the no complication group in terms of breast satisfaction, mental health, physical health-chest, physical health-abdomen, satisfaction with abdomen, sexual health, etc. ( P>0.05). Conclusions:DIEP flap breast reconstruction has a significant learning curve. The patient's own clinical characteristics (such as BMI and abdominal incision scars) and intraoperative choices (such as the selection of recipient vessels) may influence the occurrence of postoperative complications. However, the presence of postoperative complications does not appear to have an impact on patient-reported outcomes.

Objective:To explore the efficacy of precise and empirical bismuth-containing quadruple therapy guided by clarithromycin sensitivity testing in the first eradication of Helicobacter pylori ( H. pylori) in Ningxia. Methods:From August 12, 2022 to March 22, 2023, 600 patients diagnosed as H. pylori-positive by 14C-urea breath test ( 14C-UBT) for the first time in People′s Hospital of Ningxia Hui Autonomous Region, Ningnan Hospital of People′s Hospital of Ningxia Hui Autonomous Region, Zhongwei People′s Hospital, Yanchi County People′s Hospital, and Pingluo People′s Hospital were selected, and divided into empirical treatment group (hereinafter referred to as the empirical group), genetic testing group (hereinafter referred to as the genetic group), and drug sensitivity testing group (hereinafter referred to as the drug sensitivity group) by using random number table with 200 patients in each group. The empirical group did not undergo drug sensitivity testing and genetic testing, while the genetic and drug sensitivity groups were confirmed to be sensitive to clarithromycin through genetic testing and drug sensitivity testing, and the patients with drug-resistant were excluded, respectively. All the patients of the 3 groups received the same clarithromycin bismuth-containing quadruple therapy. Intention-to-treat (ITT) and per-protocol (PP) analyses were performed to compare the eradication rates of H. pylori among 3 groups. Cost-effectiveness ratio (CER) and incremental cost-effectiveness ratio (ICER) were used for cost-effectiveness and sensitivity analysis based on the ITT. Chi-square test was used for statistical analysis. Results:There were 200, 126, and 168 patients included in the empirical group, genetic group, and drug sensitivity group in ITT analysis, and 190, 123, and 164 patients were enrolled in the 3 groups in PP analysis, respectively. The results of ITT analysis showed that the eradication rates of H. pylori in the empirical group, genetic group, and drug sensitivity group were 80.5% (161/200), 94.4% (119/126), and 95.2% (160/168), respectively. The results of PP analysis indicated that the eradication rates of H. pylori in the 3 groups were 84.7% (161/190), 96.7% (119/123), and 97.6% (160/164), respectively, and the differences were statistically significant ( χ2=25.39 and 24.93, both P<0.001). The H. pylori eradication rates of genetic group and drug sensitivity group were both higher than that of empirical group in ITT and PP analysis( χ2=12.40, 17.80, 11.42, and 17.13; all P<0.001). The cost-effectiveness analysis showed that the direct treatment cost of the empirical group, genetic group, and drug sensitivity group was 400.8, 729.2, and 779.2 yuan, respectively, and the CER was 4.98, 7.72, and 8.18 yuan/%, respectively. Compared to the empirical group, the ICER of the genetic group and drug sensitivity group was 23.6 and 25.7 yuan/%, respectively. The sensitivity analysis demonstrated that, when the cost of genetic testing reduced or increased by 20%, the ICER of the genetic group compared to the empirical group was 21.8 or 25.5 yuan/%, respectively. When the cost of drug sensitivity testing reduced or increased by 20%, the ICER of the drug sensitivity group compared to the empirical group was 23.3 or 28.2 yuan/%. When the cost of gastroscopy reduced or increased by 20%, the ICER of the genetic group compared to the empirical group was 20.8 or 26.5 yuan/%, and the ICER of the drug sensitivity group compared to the empirical group was 23.0 or 28.4 yuan/%, respectively. Conclusion:In Ningxia, if the clarithromycin bismuth-containing quadruple regimen is applied as the first H. pylori eradication regimen, in order to achieve the clinical eradication efficacy of H. pylori, and the patients can accept an additional payment of 23.6 or 25.7 yuan for each 1% increasing in the H. pylori eradication rate, then the precision treatment after clarithromycin resistance test is recommended.

Objective:To investigate the clinical application effect of internal mammary artery perforator (IMAP) as recipient vessels in breast reconstruction with deep inferior epigastric perforator flap (DIEPF) immediately after breast cancer surgery.Methods:From May 2020 to May 2023, a total of 18 patients with DIEPF breast reconstruction using IMAP as recipient vessels were selected from the Department of Breast Disease of Henan Cancer Hospital. The patients were 31 to 50 years old, with an average of 41.5 years old. The stages of breast cancer were cT is/1-2N 0-2M 0, and all of the patients received immediate breast reconstruction after the breast cancer surgery. The size of flaps were from 9.0 cm × 26.0 cm to 15.0 cm × 38.0 cm. Preoperative chest and abdominal wall CTA were performed to identify the location of intercostal space and the calibre of IMAP. Intraoperatively, the number of IMAP, the diameters of corresponding arteries and accompanying veins in the recipient site were recorded. All patients were included in the scheduled postoperative follow-up through outpatient clinic or via WeChat. The quality of flap survival was evaluated, the condition of breast appearance and recovery of the abdominal donor site were evaluated according to the breast cancer patient reported outcome measures (BREAST-Q). Results:All the 18 patients had the IMAP visualised in surgery, with 13 had the IMAP located at the second intercostal space and 3 at the third intercostal space. The other 2 patients were found with the IMAP located in both the second and third intercostal spaces, in which 1 was found that both of IMAP were suitable for anastomosis. It was also found that there was 1 accompanying vein in 15 breasts and 2 accompanying veins in 2 breasts. One breast had found without an accompanying vein. The diameters of arteries were 1.1 mm±0.1 mm and that of the veins were 1.8 mm±0.3 mm. The average follow-up period was 28 months, ranged from 6 to 40 months. Of the 18 flaps, 17 were completely survived. Venous compromise occurred in 1 flap due to extensive venous thrombosis, and it was replaced with a breast implant. No patient experienced concave deformities in the reconstructed breasts. Seventeen patients with an average BREAST-Q score of 94.4. One patient with a BREAST-Q score of 79.0.Conclusion:IMAP can serve as a reliable recipient vessel for immediate breast reconstruction with DIEPF after breast cancer surgery. With strict selection criteria, this technique could be put on further trials with larger sample size and multi-centres.

The presence of adenocarcinoma in urothelium is rare and mucinous adenocarcinoma is even rarer. A case of primary ureteral papillary mucinous adenocarcinoma was reported. The patient was admitted to hospital due to dull pain in the left lumbar abdomen with abdominal distension for 2 years and aggravation with fever for 1 week. CT examination revealed left ureteral calculi, severe left renal hydronephrosis, and renal cortical atrophy. The diagnosis was left ureteral calculus with hydronephrosis and left renal dysfunction. Left kidney puncture and drainage were performed first, followed by laparoscopic nonfunctional nephrectomy and ureterolithotomy. The pathological diagnosis was left ureteral mucinous adenocarcinoma. The patient refused further adjuvant therapy and died 16 months after surgery due to extensive tumor metastasis.

OBJECTIVE: To introduce a scout view scanning technique of back-forward bending CT (BFB-CT) in simulated surgical position for evaluating the remaining real angle and flexibility of thoracolumbar kyphosis secondary to old osteoporotic vertebral compression fracture. METHODS: A total of 28 patients with thoracolumbar kyphosis secondary to old osteoporotic vertebral compression fracture who met the selection criteria between June 2018 and December 2021 were included in the study. There were 6 males and 22 females with an average age of 69.5 years (range, 56-92 years). The injured vertebra were located at T ₁₀-L ₂, including 11 cases of single thoracic fracture, 11 cases of single lumbar fracture, and 6 cases of multiple thoracolumbar fractures. The disease duration ranged from 3 weeks to 36 months, with a median of 5 months. All patients received examinations of BFB-CT and standing lateral full-spine X-ray (SLFSX). The thoracic kyphosis (TK), thoracolumbar kyphosis (TLK), local kyphosis of injured vertebra (LKIV), lumbar lordosis (LL), and the sagittal vertical axis (SVA) were measured. Referring to the calculation method of scoliosis flexibility, the kyphosis flexibility of thoracic, thoracolumbar, and injured vertebra were calculated respectively. The sagittal parameters measured by the two methods were compared, and the correlation of the parameters measured by the two methods was analyzed by Pearson correlation. RESULTS: Except LL ( P>0.05), TK, TLK, LKIV, and SVA measured by BFB-CT were significantly lower than those measured by SLFSX ( P<0.05). The flexibilities of thoracic, thoracolumbar, and injured vertebra were 34.1%±18.8%, 36.2%±13.8%, and 39.3%±18.6%, respectively. Correlation analysis showed that the sagittal parameters measured by the two methods were positively correlated ( P<0.001), and the correlation coefficients of TK, TLK, LKIV, and SVA were 0.900, 0.730, 0.700, and 0.680, respectively. CONCLUSION Thoracolumbar kyphosis secondary to old osteoporotic vertebral compression fracture shows an excellent flexibility and BFB-CT in simulated surgical position can obtain the remaining real angle which need to be corrected surgically.
Male ; Female ; Humans ; Aged ; Fractures, Compression/surgery* ; Spinal Fractures/diagnostic imaging* ; Lumbar Vertebrae/surgery* ; Thoracic Vertebrae/surgery* ; Kyphosis/surgery* ; Osteoporotic Fractures/surgery* ; Lordosis ; Tomography, X-Ray Computed ; Retrospective Studies

Objective     To compare the clinical efficacy and safety of da Vinci robot with totally no tube (TNT) versus subxiphoid video-assisted thymectomy surgery (SVATS) in the treatment of thymic tumors. Methods     From 2019 to 2021, a retrospective analysis was conducted on patients with thymic tumor resection in the Department of Thoracic Surgery, General Hospital of Northern Theater Command. All patients underwent total thymectomy and mediastinal fat removal, and they were divided into a TNT group and a SVATS group according to the operation method. The intraoperative blood loss, conversion rate, postoperative visual analogue score (VAS), postoperative hospital stay time and postoperative complications were compared between the two groups. Results     We finally included 435 patiets. There were 168 patients with 83 males and 85 females at an average age of 61.920±9.210 years in the TNT group and 267 patients with 147 males and 120 females at an average age of 61.460±8.119 years in the SVATS group. There was no death or postoperative myasthenic crisis in both groups. There was no statistical difference in postoperative hospital stay (1.540±0.500 d vs. 3.400±0.561 d, P=0.000), intraoperative blood loss (13.450±5.498 mL vs. 108.610±54.462 mL, P=0.000), postoperative 24 h VAS score (4.960±1.757 points vs. 3.600±1.708 points, P=0.000), or postoperative complication rate (3.0% vs. 11.6%, P=0.001). Conclusion     TNT is a more efficient, safe, and effective surgical approach for treating thymic tumors, which can shorten hospital stay time and reduce postoperative complications. However, SVATS can minimize postoperative pain.

Objective:To observe the physiological effect of bi-level positive airway pressure (BiPAP) ventilation among stable chronic obstructive pulmonary disease (COPD) patients.Methods:This was a small sample size, exploratory, interventional study. A total of 10 outpatients with stable COPD were included from Department of Pulmonary and Critical Care Medicine of Zhujiang Hospital, Southern Medical University between January 2018 and December 2018. The BiPAP mode of noninvasive mechanical ventilation was adopted. The inspiratory positive airway pressure was gradually increased from 10 cmH 2O (1 cmH 2O=0.098 kPa) to 24 cmH 2O, and each time by 2 cmH 2O. The expiratory positive airway pressure remained unchanged at 4 cmH 2O. Baseline and test data were collected before and during the ventilation for comparison, including total respiratory cycle time (T tot), inspiratory time (T i), inspiratory time (T e), inspiratory tidal volume (V Ti); mouth pressure (P mo), esophageal pressure (P eso), transdiaphragmatic pressure (P di), esophageal pressure time product (PTP es), diaphragm pressure time product (PTP di), root mean square of electromyography of diaphragm (RMS), V e/RMS, inspiratory capacity (IC), the change in end-expiratory lung volume (ΔEELV) and dynamic PEEPi (PEEPi dyn). Results:All the 10 patients completed the trial. Compared to calm breathing, V Ti, V e, P mo, IC, ΔEELV score and V e/RMS increased significantly with increasing pressure levels (all P<0.05); T e only increased significantly at 20-22 cmH 2O pressure levels compared to calm breathing ( P<0.05). P di, PTP es, PTP di, RMS and RMS/RMS max decreased significantly with increasing levels (all P<0.05). PTP es and PTP di converged to 0 and no longer showed significant changes after the 18 cmH 2O pressure level. RMS and RMS/RMS max flattened out at pressure level greater than 16 cmH 2O. T i/T tot only significantly decreased at the 20 cmH 2O pressure level compared to calm breathing. PEEPi dyn showed a tendency to decrease and then increase with increasing pressure levels. Conclusion:BiPAP ventilation, at appropriate pressure levels, significantly relieves pulmonary ventilation disorders and reduces the load of respiratory muscle in patients with stable COPD.

OBJECTIVE: To investigate the changes in spinal-pelvic sagittal parameters from preoperative standing to prone position in old traumatic spinal fractures with kyphosis. METHODS: The clinical data of 36 patients admitted between December 2016 and June 2021 for surgical treatment of old traumatic spinal fractures with kyphosis, including 7 males and 29 females, aged from 50 to 79 years (mean, 63.9 years), were retrospectively analyzed. Lesion segments included 2 cases of T ₁₁, 12 cases of T ₁₂, 2 cases of T _{11, 12}, 4 cases of T ₁₂ and L ₁, 12 cases of L ₁, 2 cases of L ₂, 1 case of L _{2, 3}, and 1 case of L ₃. The disease duration ranged from 4 to 120 months, with an average of 19.6 months. Surgical procedures included Smith-Petersen osteotomy in 4 cases, Ponte osteotomy in 6 cases, pedicle subtraction osteotomy in 2 cases, and improved fourth level osteotomy in 18 cases; the remaining 6 cases were not osteotomized. The bone mineral density ranged from -3.0 to 0.5 T, with a mean of -1.62 T. The spinal-pelvic sagittal parameters from preoperative standing to prone positions were measured, including local kyphosis Cobb angle (LKCA), thoracic kyphosis (TK), lumbar lordosis (LL), sacral slope (SS), pelvic tilt (PT), and PI and LL mismatch (PI-LL). The kyphotic flexibility=(preoperative standing LKCA-preoperative prone LKCA)/preoperative standing LKCA×100%. Spinal-pelvic sagittal parameters were compared between standing position and prone position before operation, and Pearson correlation was used to judge the correlation between the parameters of standing position and prone position before operation. RESULTS: When the position changed from standing to prone, LKCA and TK decreased significantly ( P<0.05), while SS, LL, PT, and PI-LL had no significant difference ( P>0.05). Pearson correlation analysis showed that LL was significantly correlated with SS and PI-LL in both standing and prone positions ( P<0.05), and the correlation strength between LL and SS in prone position was higher than that in standing position. In the standing position, LKCA was significantly correlated with SS and PT ( P<0.05). However, when the position changed from standing to prone, the correlation between LKCA and SS and PT disappeared, while PT and PI-LL was positive correlation ( P<0.05). The kyphotic flexibility was 25.13%-78.79%, with an average of 33.85%. CONCLUSION For the patients of old traumatic spinal fractures with kyphosis, the preoperative LKCA and TK decrease significantly from standing position to prone position, and the correlation between spinal and pelvic parameters also changed, which should be taken into account in the formulation of preoperative surgical plan.
Male ; Female ; Humans ; Spinal Fractures/surgery* ; Standing Position ; Retrospective Studies ; Lumbar Vertebrae/surgery* ; Kyphosis/surgery* ; Lordosis/surgery*

Objective:To evaluate the efficacy of esketamine for patient-controlled intravenous analgesia (PCIA) in elderly patients undergoing modified radical mastectomy for breast cancer.Methods:Ninety elderly female patients, aged 65-78 yr, weighing 46-75 kg, of American Society of Anesthesiologists physical status Ⅱ or Ⅲ, undergoing elective modified radical surgery for breast cancer under general anesthesia, were divided into 2 groups ( n=45 each) using a random number table method: esketamine PCIA group (group E) and sufentanil PCIA group (group S). Anesthesia was induced with target-controlled infusion of propofol, intravenous atracurium besylate and sufentanil and maintained with target-controlled infusion of propofol and remifentanil and intermittent intravenous boluses of cis-benzenesulfonic acid atracurium.The patients were connected to an analgesic pump for PCIA at 10 min before completion of operation.The PCIA solution in group E contained esketamine 2 mg/kg, ketorolac tromethamine 90 mg and tropisetron 5 mg in 100 ml of normal saline.The PCIA solution in group S contained sufentanil 1 μg/kg, ketorolac tromethamine 90 mg and tropisetron 5 mg in 100 ml of normal saline.The PCA pump was set up with a 1.5 ml bolus dose, a 15 min lockout interval and background infusion at a rate of 1.5 ml/h, and the analgesia was performed until 48 h after operation.When numeric rating scale score ≥ 4 points and the efficacy of patient-controlled analgesia was not good, tramadol 100 mg was intravenously injected for rescue analgesia.Steward recovery scores were recorded at 4, 8, 24 and 48 h after operation.The requirement for rescue analgesia, effective pressing times of analgesic pump and time to first flatus were recorded within 48 h after operation.The nausea and vomiting, respiratory depression, dizziness and pruritus within 48 h after operation and delirium within 7 days after operation were recorded.The 40-item Quality of-Recovery scale was used to evaluate the early postoperative recovery of patients at 24 and 48 h after operation. Results:Compared with group S, the 40-item Quality of Recovery scale score was significantly increased at each time point, postoperative time to first flatus was shortened, the incidence of postoperative nausea and vomiting and pruritus was decreased ( P<0.05), and no significant change was found in the Steward recovery score at each time point after operation, effective pressing times of PCA and requirement for rescue analgesia in group E ( P>0.05). Conclusions:Esketamine provides better efficacy than sufentanil when used for PCIA in elderly patients undergoing modified radical mastectomy for breast cancer.