Child ; Humans ; Mpox (monkeypox)/prevention & control* ; Disease Outbreaks

Objective:To investigate a new method for the reconstruction of hemifacial microsomia by sagittal osteotomy of the affected mandibular outer cortex combined with bone graft of mandibular outer cortex from healthy side.Methods:From March 2006 to March 2023, the clinical data of patients with hemifacial microsomia admitted to the Department of Craniomaxillofacial Surgery, Plastic Surgery Hospital, Chinese Academy of Medical Sciences were analyzed retrospectively. Preoperative diagnosis and surgical design were performed based on clinical manifestations and imaging findings. All cases were operated under general anesthesia. The affected mandibular outer cortex was previously split by an intraoral approach, and then the mandibular outer cortex of appropriate shape and size on the healthy side was harvested and grafted into the split bone space according to the preoperative design, following by internal rigid fixation. Complications, facial appearance improvement, and patient satisfaction were followed up. Photographs were taken preoperative, immediately postoperative and at the long-term(last) postoperative follow-up, and the severity of the deformity was analyzed. CT data from preoperative, immediate postoperative, and long-term follow-up visits were imported into Surgicase Proplan medical three-dimensional image workstation in Dicom format. The mandible was reconstructed using Segmentation, and the thickness of the mandible was measured during pre-operative, immediate post-operative and long-term follow-up visits. Anova with repeated measurement design was used to compare measurements and LSD test was used for multiple comparisons. The Kruskal-Wallis rank sum test were used to statistically analyze malformation severity. P< 0.05 is considered statistically significant. Results:A total of 39 patients were included in this study, including 13 females and 26 males, with an average age of (22.21±4.57) years (15-27 years). All patients were followed up for an average of (45.56±39.41) months (6-153 months) after surgery. The grafted mandibular outer cortex grows well with the adjacent bone tissue, and the mandibular angle and mandibular body are significantly wider. Of the 39 cases, 1 developed an infection 1 year after surgery, the titanium plate was exposed, and the patient healed after debridement and removal of the immobilizing splint. The facial appearance of the other patients improved significantly. Preoperative, immediate postoperative and long term follow up of mandibular thickness measurements were compared in pairs, and the differences were statistically significant (all P<0.05). The patient’s appearance satisfaction score: the preoperative score was [2.0(1.5, 2.0)] points, the immediate postoperative score was [4.0(4.0, 4.0)] points, the score of the last postoperative follow up was [4.0(4.0, 4.0)] points. There was statistical difference in satisfaction among the three groups ( P<0.01). The preoperative scores were compared with the scores of the immediate postoperative and the last postoperative follow-up respectively, and the differences were statistically significant( P<0.01). There was no statistical significance in satisfaction between the immediate postoperative score and the score of the last postoperative follow up ( P>0.05). Conclusion:The sagittal splitting osteotomy of the mandibular outer cortex is consistent with the features of mandibular anatomy, and provides a good condition for the grafting and healing of autogenous bone. Removing the outer cortex of the mandible on the healthy side not only increases the thickness of the affected side, but also decreases the width of the angle of the mandible on the healthy side, so as to effectively correct the asymmetric deformity of the mandible. The method is simple, with few complications and good results, and is one of the ideal treatments to correct hemofacial microsomia.

Objective:To investigate a new method for the reconstruction of hemifacial microsomia by sagittal osteotomy of the affected mandibular outer cortex combined with bone graft of mandibular outer cortex from healthy side.Methods:From March 2006 to March 2023, the clinical data of patients with hemifacial microsomia admitted to the Department of Craniomaxillofacial Surgery, Plastic Surgery Hospital, Chinese Academy of Medical Sciences were analyzed retrospectively. Preoperative diagnosis and surgical design were performed based on clinical manifestations and imaging findings. All cases were operated under general anesthesia. The affected mandibular outer cortex was previously split by an intraoral approach, and then the mandibular outer cortex of appropriate shape and size on the healthy side was harvested and grafted into the split bone space according to the preoperative design, following by internal rigid fixation. Complications, facial appearance improvement, and patient satisfaction were followed up. Photographs were taken preoperative, immediately postoperative and at the long-term(last) postoperative follow-up, and the severity of the deformity was analyzed. CT data from preoperative, immediate postoperative, and long-term follow-up visits were imported into Surgicase Proplan medical three-dimensional image workstation in Dicom format. The mandible was reconstructed using Segmentation, and the thickness of the mandible was measured during pre-operative, immediate post-operative and long-term follow-up visits. Anova with repeated measurement design was used to compare measurements and LSD test was used for multiple comparisons. The Kruskal-Wallis rank sum test were used to statistically analyze malformation severity. P< 0.05 is considered statistically significant. Results:A total of 39 patients were included in this study, including 13 females and 26 males, with an average age of (22.21±4.57) years (15-27 years). All patients were followed up for an average of (45.56±39.41) months (6-153 months) after surgery. The grafted mandibular outer cortex grows well with the adjacent bone tissue, and the mandibular angle and mandibular body are significantly wider. Of the 39 cases, 1 developed an infection 1 year after surgery, the titanium plate was exposed, and the patient healed after debridement and removal of the immobilizing splint. The facial appearance of the other patients improved significantly. Preoperative, immediate postoperative and long term follow up of mandibular thickness measurements were compared in pairs, and the differences were statistically significant (all P<0.05). The patient’s appearance satisfaction score: the preoperative score was [2.0(1.5, 2.0)] points, the immediate postoperative score was [4.0(4.0, 4.0)] points, the score of the last postoperative follow up was [4.0(4.0, 4.0)] points. There was statistical difference in satisfaction among the three groups ( P<0.01). The preoperative scores were compared with the scores of the immediate postoperative and the last postoperative follow-up respectively, and the differences were statistically significant( P<0.01). There was no statistical significance in satisfaction between the immediate postoperative score and the score of the last postoperative follow up ( P>0.05). Conclusion:The sagittal splitting osteotomy of the mandibular outer cortex is consistent with the features of mandibular anatomy, and provides a good condition for the grafting and healing of autogenous bone. Removing the outer cortex of the mandible on the healthy side not only increases the thickness of the affected side, but also decreases the width of the angle of the mandible on the healthy side, so as to effectively correct the asymmetric deformity of the mandible. The method is simple, with few complications and good results, and is one of the ideal treatments to correct hemofacial microsomia.

Objective:To investigate the clinical features,gene mutation profile,efficacy and prognostic factors of primary extranodal diffuse large B-cell lymphoma(EN-DLBCL).Methods:A retrospective analysis was performed for 382 patients with primary EN-DLBCL with complete clinical data who were treated in West China Hospital from January 2013 to January 2023,and their clinical characteristics,gene mutation profile,efficacy and prognostic factors were analyzed.Results:The median age of the 382 patients with EN-DLBCL was 56 (18-89 )years old.The male-to-female ratio was 1.12∶1,and the most common primary sites were gastrointestinal tract (31.7％),Wechsler ring (19. 1％)and breast gland (7.1％).A total of 51 gene mutations were fund,and the most common frequencies of gene mutations were TP53 (32.5％),MYD88 (32.5％),and CD79B (30.0％).The median follow-up was 63 months,and the 5-year progression-free survival (PFS)rate was 74.5％ and the 5-year overall survival (OS)rate was 89.6％. The adverse factors on PFS were as follows:>1 extranodal sites involvement (P<0.001),P53≥50％(P<0.001),hyper double expression(hDEL)of C-myc>50％/Bcl-2>70％(P<0.001).The adverse factors affecting the OS of patients were as follows:>1 extranodal sites involvement (P<0.001),P53≥50％(P<0.001),hDEL(P<0.001). Chemotherapy combined with local radiotherapy could improve PFS (P=0.041)and OS (P=0.003),while R-CHOP+X (molecule agents as BTKi、HDACi、Lenalidomide)failed to show a significant difference in PFS (P=0.075)and OS (P=0.767 ).Among the 40 patients who underwent next-generation sequencing at high risk,there was no significant in PFS (P=0.849)and OS (P=0.500)of patients with positive MYD88 and/or CD79B mutations (MCD subtype)treated with BTKi and patients with negative MYD88 and CD79B mutations.Conclusion:Primary EN-DLBCL can involve multiple organs or tissue sites.TP53,MYD88,and CD79B are the most common gene mutations.The efficacy of BTKi in patients with positive MCD subtypes at intermediate and high risk is not inferior to that in MCD-negative control patients.

OBJECTIVE: To preliminarily verify the effectiveness of self-designed artificial condyle-mandibular distraction (AC-MD) complex in the treatment of Pruzansky type ⅡB and Ⅲ hemifacial microsomia (HFM) through model test. METHODS: Five children with Pruzansky type ⅡB and Ⅲ HFM who were treated with mandibular distraction osteogenesis (MDO) between December 2016 and December 2021 were selected as the subjects. There were 3 boys and 2 girls wih an average age of 8.4 years (range, 6-10 years). Virtual surgery and model test of AC-MD complex were performed according to preoperative skull CT of children. The model was obtained by three-dimensional (3D) printing according to the children's CT data at a ratio of 1∶1. The occlusal guide plate was designed and 3D printed according to the children's toothpaste model. The results of the model test and the virtual surgery were matched in three dimensions to calculate the error of the residual condyle on the affected side, and the model test was matched with the actual skull CT after MDO to measure and compare the inclination rotation of the mandible, the distance between the condylar of the healthy side and the residual condyle of the affected side, and the lengthening length of the mandible. RESULTS: The error of residual condyle was (1.07±0.78) mm. The inclination rotation of the mandible, the distance between the condylar of the healthy side and the residual condyle of the affected side, and the lengthening length of the mandible after 3D printing model test were significantly larger than those after MDO ( P<0.05). CONCLUSION In the model test, the implantation of AC-MD complex can immediately rotate the mandible to the horizontal position and improve facial symmetry, and the residual condyle segment can be guided close to the articular fossa or the preset pseudoarticular position of the skull base after operation.
Male ; Child ; Female ; Humans ; Goldenhar Syndrome/surgery* ; Mandible/surgery* ; Osteogenesis, Distraction/methods* ; Printing, Three-Dimensional ; Facial Asymmetry/surgery*

We performed an extensively targeting metabolomic detecting using ultra-performance liquid chromatography-mass spectrometry (UPLC-MS/MS) to compare the secondary metabolites in Dang shen [Codonopsis pilosula (Franch.) Nannf.] from Shanxi and Gansu provinces. The findings showed that 161 secondary metabolites in 6 groups (phenolic acids, flavonoids, lignans and coumarins, alkaloids, terpenoids, others) were found from Dang shen in Changzhi city of Shanxi province and Dingxi city of Gansu province. There were 98 secondary metabolites which is differed significantly. In comparison to Dingxi city, 33 different secondary metabolites of Dang shen in Changzhi city had a greater relative content, whereas relative content of 65 different metabolites in Dingxi city was higher. Metabolic pathway enrichment analysis revealed that phenolic acids and flavonoids were significantly different in the secondary metabolites of Dang shen from different producing places. This may be one of the reasons for the difference in the quality of Dang shen in Shanxi and Gansu provinces. This work compared and analyzed the secondary metabolites of Dang shen from Dingxi city in Gansu province and Changzhi city in Shanxi province for the first time, which lays the foundation for further study on the quality of Dang shen.

Objective: To investigate the clinical characteristics, cytogenetics, molecular biology, treatment, and prognosis of patients with therapy-related myelodysplastic syndrome and acute myeloid leukemia (t-MDS/AML) secondary to malignancies. Methods: The clinical data of 86 patients with t-MDS/AML in West China Hospital of Sichuan University between January 2010 and April 2023 were retrospectively analyzed. The clinical characteristics, primary tumor types, and tumor-related therapies were analyzed. Results: The study enrolled a total of 86 patients with t-MDS/AML, including 67 patients with t-AML, including 1 patient with M(0), 6 with M(1), 27 with M(2), 9 with M(3), 12 with M(4), 10 with M(5), 1 with M(6), and 1 with M(7). Sixty-two patients could be genetically stratified, with a median overall survival (OS) of 36 (95% CI 22-52) months for 20 (29.9%) patients in the low-risk group and 6 (95% CI 3-9) months for 10 (14.9%) in the intermediate-risk group. The median OS time was 8 (95% CI 1-15) months in 32 (47.8%) patients in the high-risk group. For patients with non-acute promyelocytic leukemia (APL) and AML, the median OS of the low-risk group was 27 (95% CI 18-36) months, which was significantly longer than that of the non-low-risk group (χ(2)=5.534, P=0.019). All 9 APL cases were treated according to the initial treatment, and the median OS was not reached, and the 1-, 2-, and 3-year OS rates were 100.0%, (75.0±6.2) %, and (75.0±6.2) % respectively. Of the 58 patients with non-APL t-AML (89.7%), 52 received chemotherapy, and 16 achieved complete remission (30.8%) after the first induction chemotherapy. The 1-, 2-, and 3-year OS rates of the non-APL t-AML group were (42.0 ± 6.6) %, (22.9±5.7) %, and (13.4±4.7) %, respectively. The median OS of patients who achieved remission was 24 (95% CI 18-30) months, and the median OS of those who did not achieve remission was 6 (95% CI 3-9) months (χ(2)=10.170, P=0.001). Bone marrow CR was achieved in 7 (53.8%) of 13 patients treated with vineclar-containing chemotherapy, with a median OS of 12 (95% CI 9-15) months, which was not significantly different from that of vineclar-containing chemotherapy (χ(2)=0.600, P=0.437). In 19 patients with t-MDS, the 1-, 2-, and 3-year OS rates were (46.8±11.6) %, (17.5±9.1) %, and (11.7±9.1) % with a median OS of 12 (95% CI 7-17) months, which was not significantly different from that in t-AML (χ(2)=0.232, P=0.630) . Conclusions: Breast cancer, bowel cancer, and other primary tumors are common in patients with t-MDS/AML, which have a higher risk of adverse genetics. Patients with APL had a high induction remission rate and a good long-term prognosis, whereas patients without APL had a low remission rate and a poor long-term prognosis.
Humans ; Retrospective Studies ; Leukemia, Myeloid, Acute/drug therapy* ; Leukemia, Promyelocytic, Acute/therapy* ; Prognosis ; Myelodysplastic Syndromes/drug therapy* ; Neoplasms, Second Primary/drug therapy* ; Remission Induction ; Antineoplastic Combined Chemotherapy Protocols/therapeutic use*

Objective To investigate the relationship between digit ratio and schizophrenia in men from Ningxia Han nationality. Methods Using anthropometry, digital camera was used to obtain finger photographs of both hands of 216 male subjects (nonnal control; 116, schizophrenia patient; 100). The anatomical points were marked and the length of each finger of both hands were measured by computer image analysis software (Image-Pro Plus 6 . 0 ) . Differences of the mean digit ratio of left and right hand 2D:3D, 2D:4D, 2D:5D, 3D:4D, 3D:5D, 4D:5D between the control group and the patient group were compared. The correlation between 2D :4D and onset age in the patient group were analyzed. Results Except for left and right hand 3D :4D, digit ratios in patient group were significantly higher than those in control group (2D :4D and 3D :5D in both hands, right hand 3D :5D, p all<0. 001; 2D :3D and 4D :5D, left hand 3D :5D, P<0. 0 1 ) . Compared with the control group (left hand; 1%, right hand; 1%) , the percentage of 2D:4D Ss 1 were significantly higher ( P<0. 05) in the patient group (left hand; 6% , right hand; 7%). There were no conelations between 2D :4D mean value and onset age in patients group (P>0. 05). Conclusion Digit ratio is related to the occurrence of schizophrenia in Ningxia Han male.

Aim To observe the effect of thalidomide on the learning and memory ability and hippocampal tissue proteome of Alzheimer's disease(AD)mice,to screen the differential proteins of thalidomide in preventing and treating AD,the pathways involved in regulation,and to explore its possible mechanism. Methods The experimental mice were randomly divided into blank control group,model group,and thalidomide high and low dose groups. The drugs were administered by gavage every day for 21 days. After the administration,Morris water maze test was used to evaluate the learning and memory abilities of the mice,HE staining and Nissl staining were used to observe the pathological tissue morphology of the mouse hippocampus,ELISA was employed to detect the mitochondrial respiratory chain enzyme complex in mouse brain,and the Label-free proteomics method was used to screen different groups of hippocampal proteome proteins. Results The results of the Morris water maze showed that compared with the model group,the escape latency time of the drug group was significantly reduced,and the number of crossing the platform significantly increased(P<0.05). Thalidomide administration could improve the morphological structure of neurons in hippocampus,and could increase the activity of the mitochondrial respiratory chain complex ,Ⅱ, and of the brain tissues of AD mice(P<0.05). A total of 4 378 differential proteins were identified,which had a significant regulatory effect on the expression of 580 proteins in hippocampus of AD mice(P<0.05). Energy metabolism may jointly participate in the regulation of neurodegeneration pathways-changes in pathways such as various diseases and Alzheimer's disease. Conclusions Thalidomide can significantly improve the learning and memory function of AD model mice induced by Aβ

OBJECTIVE: This study is to obtain precise data on iron physiological requirements in Chinese children using single stable isotope tracer technique. METHODS: Thirty boys (10.6 ± 0.2 years) and 27 girls (10.4 ± 0.2 years) were received oral 6 mg ⁵⁷Fe each day for 5 consecutive days. Venous blood samples were subsequently drawn to examine the change of total iron concentration and ⁵⁷Fe abundance at day 0, 14, 28, 60, 90, 180, 360, 450, 540, 630, 720. The iron physiological requirement was calculated by iron loss combined with iron circulation rate once ⁵⁷Fe abundance stabilized in human body. RESULTS: The iron physiological requirement was significantly lower in boys than those values in girls (16.88 ± 7.12 vs. 18.40 ± 8.81 μg/kg per day, P < 0.05). Correspondingly, the values were calculated as 722.46 ± 8.43 μg/day for boys and 708.40 ± 7.55 μg/day for girls, respectively. Considering nearly 10% iron absorption rate, the estimated average iron physiological requirement was 6.0 mg/day in boys and 6.2 mg/day in girls. CONCLUSION This study indicate that iron physiological requirement could require more daily iron intake in girls as compare with the values in boys having the same body weight. These findings would be facilitate to the new revised dietary reference intakes.
Body Weight ; Child ; China ; Female ; Humans ; Iron ; Isotopes ; Male ; Nutritional Status