Objective:To investigate the effect of microgravity-mediated Notch1 signaling on macrophage polarization on bone homeostasis.Methods:The animal model was constructed by tail-limb suspension(HLS)to simulate the microgravity environment.The animals were grouped into Control group,HLS group,HLS+NC group,HLS+si group,HLS+rhNF-κB group.ELISA was used to detect the content of TNF-α and IL-1β in serum of rats.TUNEL staining was used to detect the apoptosis of bone tissue.Immunofluo-rescence was used to detect the polarization of macrophages in bone tissue.The rat osteoblast CP-R091 microgravity model was con-structed by simulating the microgravity environment with a rotating wall bioreactor.The cell experiments were divided into Control group,HLS group,HLS+NC group,HLS+si group,HLS+rhNF-κB group.CCK-8 test was used to detect the proliferation activity of cells in each group,and AO test was used to test the apoptosis rate of cells in each group.PCR was used to detect the expression of os-teogenesis-related genes in bone tissues and cells.Western blot was used to detect the expression of Notch1,hair division-related en-hancer-1(HES-1),and Notch pathway ligand 1(Jagged1)in bone tissues and cells of each group.Results:Compared with control group,the contents of TNF-α and IL-1β in the serum of the rats in the HLS group,the apoptosis rate,and the proportion of M1 macro-phages were significantly increased.Compared with HLS group,the HLS+si group could obviously partially reverse the change trend of the above parameters,while HLS+rhNF-κB group significantly changed the above parameter values.Compared with control group,the proliferation activity of the cells in the HLS group was significantly reduced,and the apoptosis rate was significantly increased.Com-pared with HLS group,the HLS+si group could obviously partially reverse the change trend of the above parameters,while the HLS+rhNF-κB group made the above parameter values worse.The expressions of the osteogenesis-related genes collagen type Ⅰ(COL1),osteocalcin(OCN)and Runt-related transcription factor 2(RUNX2)in bone tissues and cells in the microgravity environment were significantly decreased,while the expressions of Notch-1,Hes-1 and Jagged1 were significantly increased,and the differences were statistically significant(all P<0.05).Conclusion:Microgravity-mediated Notch1 signaling regulates M1/M2 polarization of macro-phages,participates in cell proliferation and apoptosis in bone tissue,and affects the progress of bone homeostasis.

Objective:To explore the characteristics of abnormal chromosome karyotype of common external genital malformations in male children.Methods:A retrospective analysis of the clinical data of 2 408 children was made in outpatient or inpatient from January 2012 to December 2017. The mean age was (38±7) months. There were 1115 cases of hypospadias, 189 cases of cryptorchidism, 304 cases of micropenis, 681 cases of concealed penis and 119 cases of disorders of sex development. All children were tested for chromosome 550 band in peripheral blood, and the results of chromosome karyotype were analyzed.Results:A total of 131 cases of abnormal chromosome karyotypes were detected, with a detection rate of 5.4%, including 46 cases of chromosome number abnormalities, 85 cases of structural abnormalities, 64 cases of sex chromosome abnormalities and 67 cases of autosomal abnormalities. The karyotype of chromosome was 46, XY, inv (9) (p12q13) in 28 cases accounting for 21.4%. It was followed by 47, XXY, with 16 cases, accounting for 12.2%. The detection rate of chromosomal abnormalities in children with disorders of sex development was 12.6%(15 cases), hypospadias was 5.5%(61 cases), cryptorchidism was 5.3%(10 cases), micropenis was 4.9%(15 cases), and concealed penis was 4.4%(30 cases).Conclusions:Chromosomal abnormalities are not uncommon in male children with external genital malformations. Chromosome structural abnormalities are more common than quantitative abnormalities, and sex chromosome abnormalities account for a comparable proportion to autosomal abnormalities.

Objective:To retrospectively evaluate the clinical effect of mitral valve repair for rheumatic mitral stenosis.Methods:We retropectively analyze the clinical datd of 50 rheumatic mitral disease patients undergoing mitral valve repair from January 2016 to March 2019, the clinical outcome was compaired with those of patients undergoing mitral valve replacement. The operation time, cardiopulmonary bypass time, blood loss, ICU time, hospital stay, and postoperative cardiac function were analyzed, and followed up for 2 years to assess mitral regurgitation, cardiac function, and complication rates.Results:The time of cardiopulmonary bypass and ascending aorta occlusion in the valve repair group were longer than those in the valve replacement group ( P<0.05), and the postoperative ventilator assistance time, ICU stay time, and hospital stay were shorter than those in the valve replacement group ( P<0.05). After 2 years of follow-up, no patients died in the two groups. The rehospitalization rate in the valve repair group was lower than that in the replacement group ( P<0.05), and there was no significant difference in the reoperation rate between the groups ( P>0.05); There was 1 case (2%) of moderate mitral valve regurgitation in the mitral valve repair group, no moderate or severe mitral valve stenosis, no paravalvular leakage in the mitral valve replacement group, and no significant difference between the two groups ( P>0.05). The left ventricular end-diastolic diameter and left ventricular ejection fraction in the mitral valve repair group were significantly better than those in the mitral valve replacement group ( P<0.05). Conclusion:Mitral valve repair is effective in treating rheumatic mitral stenosis. It is beneficial to protect heart function, reduce postoperative anticoagulation complications, and does not increase the rate of reoperation. It is a safe, effective and feasible treatment.

Male sex differentiation is driven by 2 hormones produced by the fetal testis, testosterone and anti-Müllerian hormone(AMH), responsible for the regression of müllerian ducts in male fetuses. Mutations inactivating AMH or its receptor AMHR2 lead to the persistent müllerian duct syndrome(PMDS) in otherwise normally virilized 46, XY males. Further assessment was carried out when suspicion of PMDS arose from physical examination which revealed that the testis crossed to the contralateral side of the body. Further examination include ultrasound, AMH concentration, karyotype, and gene sequencing. Once PMDS is considered, there is no need to perform the gonads biopsy. The optical surgery methods include one-stage cryptorchidism and hernia curation, and at the same time.Stripping/destroying the mucosa of the retained müllerian remnants to reduce the risk of malignancy and, simultaneously, to prevent the damage to vas deference.

Objective:To investigate the therapeutic effect of dorsal skin-tightening technique on the correction of mild penile curvature in children with hypospadias.Methods:The clinical characteristics of hypospadias patients (95 cases) with mild penile curvature (<30°) after degloving penis during operation in our hospital from Jan 2017 to Sep 2020 were analyzed retrospectively. Group A: A new technique, reconstructing penile pubic angle at 12 o'clock position of penile dorsal side after degloving and suturing forskin outer and inner plate with tension at 12 o'clock position, was performed in dorsal skin-tightening group (43 cases). Gtoup B: while in dorsal midline tunica albuginea plication group (52 cases), Buck fascia was incised on dorsal midline area, following by tunica albuginea plication with one or two stitches. The patients in Group A were 0.4 to 1.5 years old, and the median age was 1.1 years, urethral orifice were located on distal shaft in 36 cases, proximal in 7 cases.The patients in Group B were 0.5 to 2.6 years old, and the median age was 1.5 years, urethral orifice were located on distal shaft in 41 cases, proximal in 11 cases. The penile ventral curvature degree was recorded during regular follow-up (postoperative 6 and 12 months), as well as postoperative complications.Results:Artificial erection test showed penile curvature was corrected during surgery by measuring with protractor. There was no chordee by measuring with the side photos in all patients during an average of 1.6 years follow-ups. There were 4 case of urethral fistula in Group B and 3 cases in Group A. No cases of urethrostenosis, diverticulum or concealed penis was recorded. The difference of postoperative complications had no statistical significance.Conclusion:Hypospadias with mild penile curvature could be effectively corrected by dorsal skin-tightening technique, which showed a good result of early follow-up.

BACKGROUND: Bronchial sleeve lobectomy is essential surgical approach to treat centralized lung cancer. It is the best reflected the principle of lung cancer surgery, "remove tumor completely while minimize pulmonary function loss". Bronchial pleural fistula (BPF) is not common but very severe complication of bronchial sleeve lobectomy, that is usually fatal. Present article is to explore clinical effect on prevention of bronchial pleural fistula (BPF) in bronchial sleeve lobectomy, by wrapping brachial anastomosis with pedicled pericardial fat flap. METHODS: Clinical data of 39 non-small cell lung cancer (NSCLC) patients who underwent surgical resection during January 2016 to May 2019 in Lung Cancer Center of West China Hospital, Sichuan University were collected and retrospectively analyzed. All of the patients underwent bronchial sleeve lobectomy and a brachial anastomosis wrapping with pedicled pericardial fat flap. RESULTS: All patients recovered well and were discharged within 6 d-14 d after operation. No BPF occurred, nor other severe complications, such as reoperation needing intrathoracic bleeding, several pneumonia and respiratory failure, and life threatening cardiac arrhythmia. Only one patient (1/39) had several anastomotic stenosis and consequential atelectasis of residual lung in operative side 6 months after surgery. CONCLUSIONS Wrapping bronchial anastomosis with pedicled pericardial fat flap in bronchial lobectomy for centralized NSCLC is a simple and effective approach to prevent BPF, thus safety of the operation could be significantly improved.

Disorders of sex development (DSD) refer to a group of diseases characterized by abnormal congenital development of chromosomes, gonad or genitals with different pathophysiological changes and clinical manifestations. DSD is more common in neonates and adolescents, and neonates often show genital abnormalities while adolescents show abnormal sexual development during puberty. It is the international consensus that the scope of DSD should include basic clinical evaluation (internal and external genitalia and endocrine hormones), diagnostic confirmation (chromosome, genetic diagnosis), psychological assessment for children and family, treatment (sex assignment, hormone replacement and surgical intervention), potential fertility protection and long-term follow-up, which require the expertise of pediatric endocrinology, pediatric urology, clinical psychology, genetic disciplines, medical images and other related disciplines; that is, individualized management of children with DSD requires an experienced multidisciplinary team (MDT). This article reviews the recent progress on the evaluation, diagnosis and management of disorders of sex development.
Consensus ; Disorders of Sex Development ; diagnosis ; therapy ; Humans ; Medicine ; trends ; Sexual Development

OBJECTIVE: To assess the value of fetal anteroposterior renal pelvic diameter (APD) in predicting antenatal hydronephrosis requiring surgical treatment after birth. METHODS: A total of 525 cases of antenatal hydronephrosis detected by prenatal ultrasonography (ultrasound index APD ≥ 4 mm in the second trimester and APD ≥ 7 mm in the third trimester) in Zhejiang Prenatal Diagnosis Center from June 2007 to June 2018 were retrospectively analyzed. ROC curve was used to analyze the relationship between these ultrasound indicators and the requirement for surgical treatment after birth. RESULTS: There were 162 cases (30.9%) diagnosed in the second trimester and 363 cases (69.1%) diagnosed in the third trimester; 131 cases were diagnosed pathologically after birth, of which 121 finally underwent surgical treatment. The area under ROC curve (AUC) of APD in middle pregnancy for prediction of requiring surgery 1-12 years after birth was 0.910; the cut-off value of APD was 8.45 mm with a sensitivity of 97.1%, specificity of 70.9%, positive predictive value (PPV) of 47.9%, and negative predictive value (NPV) of 98.9%. The AUC of APD in late pregnancy for prediction of requiring surgery 1-12 years after birth was 0.800; the cut-off value of APD was 12.25 mm with a sensitivity of 66.7%, specificity of 81.2%, PPV of 51.7%, and NPV of 89.1%. CONCLUSIONS APD in pregnancy can be used to predict whether the fetus with hydronephrosis needs surgical treatment after birth, and the prediction value of APD in the middle pregnancy is better.
Female ; Fetus ; diagnostic imaging ; Humans ; Hydronephrosis ; diagnostic imaging ; surgery ; Kidney Pelvis ; diagnostic imaging ; Pregnancy ; Retrospective Studies ; Ultrasonography

Objective: To explore the feasibility of gender assignment in 46,XY disorders of sex development (DSD) with severe undermasculinisation mainly based on molecular diagnosis. Methods: A retrospective study of 45 patients of 46, XY DSD with severe undermasculinisation were admitted between November 2015 and October 2018 at Children′s Hospital, Zhejiang University School of Medicine. The initial social gender were all female, of whom the external genital manifestations were Prader 0 to 2; the degree of masculinity was scored using external masculinisation score (EMS); the position and development of the gonads were examined by ultrasound, cystoscopy and laparoscopy, also including assessing the development of the Wolffian tube and the Müllerian tube. The level and ratio of testosterone to dihydrotestosterone before and after hCG stimulation were evaluated for the function of Leydig cell and 5α-reductase-2. Gender role scales and sandbox games were used to assess gender role behavior. Genital sensitivity to androgen stimulation was assessed; A panel including 163 genes related to gender development were determined by second-generation sequencing in all 45 patients. Finally, a multidisciplinary team (MDT) makes a gender assignment after a comprehensive analysis mainly based on the molecular etiological diagnosis. Results: Thirty-nine out of 45 patients (87%) had an identifiable genetic etiology, and the remaining 6 (13%) were negative for genetic testing. Forty-five patients had EMS less than or equal to 3 points. Sexual psychological assessment was performed in 39 patients, with male dominance in 24 (62%) and female dominance in 15 (38%). The gender assignment was 23 cases (51%) for male and 19 cases (42%) for female, and 3 cases (7%) were not completely determined. Conclusions Molecular diagnosis provides a strong basis for appropriate gender assignment of 46, XY DSD children with severe undermasculinisation. Based on molecular diagnosis, each DSD should be analyzed by professional MDT to analyze the clinical symptoms/signs, gonadal development, gonad tumor risk, external genital morphology, sexual psychological assessment, potential fertility opportunities, parental views, Social and cultural factors, etc. make appropriate gender assignment.

Objective To explore the change of urine proteins from nephrostomy tube and renal function in patients with very severe hydronephrosis after nephrostomy in order to determine the treatment strategy of either renal preservation or resection.Methods Retrospective analysis of clinical data from March 2015 to March 2017 of 28 cases with congenital unilateral severe hydronephrosis in children's hospital affiliated to Zhejiang University,including 19 boys and 9 girls,with 20 cases on the left and 8 on the right,an average age of 26.6 (0.3 to 122.0) months at the first consultation and 2 cases of urinary biochemical abnormalities.Front to rear diameter for puncture were between 5.7 and 7.6 cm,with an average of 6.7 cm,and the differential renal function (DRF) for the affected kidneys were 6.98 ％-9.89 ％ (average 8.65 ％) before puncture.The children underwent percutaneous renal perforation fistula.According to the recovery of renal function after perforation fistula,those who underwent nephrectomy were recruited as a nephrectomy group and the children whose kidney were preserved as kidney preservation group.Comparison 24 hours before and 3 months after puncture was made beteween groups regarding the proportion of urine,pH of the renal pelvis urine,oαl microglobulin (α1-MG),albumin (Alb),β2 microglobulin (β2-MG),immunoglobulin G (IgG),transferrin (TRF),and the recovery of the damaged renal function (direnal function DRF).Results Twenty-eight cases of percutaneous nephrolithotomy were successful without puncture complications,with 10 cases in the nephrectomy group and 18 cases in the renal preservation group.The difference of renal pelvis urine from the affected kidney of the nephrectomy group showed no statistical significance 24 hours before and 3 months after the procedure when comparing the following parameters,including urinary specific gravity (1.006 ± 0.007 vs.1.009 ± 0.005),pH value (7.74 ± 0.41 vs.7.70 ± 0.32),urine creatinine value [(462.20 ± 158.01) μmol/L and (449.20 ± 143.41) μmol/L],αl microglobulin [(14.03 ± 4.360) mg/L vs.(13.84 ± 2.55) mg/L],β2 microglobulin [(0.76 ± 0.28) mg/L vs.(0.65 ±0.14) mg/L],immunoglobulin G [(1 292.20 ± 303.50) mg/L vs.(1 175.33 ± 376.67) mg/L],and kidney function (DRF) [(8.86 ±0.67) ％ vs.(9.90 ± 1.26)％].While microalbumin [(8 644.40 ± 829.54) mg/L vs.(3 145.10 ± 1 445.02) mg/L] and transferrin [(445.70 ± 46.71) mg/L vs.(214.30 ± 40.13) mg/L,P ＜ 0.05] were significantly increased 3 months after than 24 h before the procedure in nephrostomy group.There were no significant differences of the urine specific gravity 24h before and 3 months after the procedure in the kidney preserved group (1.003 ± 0.003 vs.1.005 ± 0.003,P ＞ 0.05).The creatinine value of 3 months after the procedure was significantly increased than that 24 hours within the procedure [(654.50 ± 154.52tμmol/L) vs.(423.94 ± 172.74) μmol/L],and the urine pH (7.28 ± 0.32 vs.7.91 ± 0.56),α1 microglobulin [(5.85 ± 0.38) mg/L vs.(12.58 ± 3.40) mg/L],microalbumin [(571.50 ± 167.14) mg/L vs.(2 343.28 ± 576.22) mg/L],β2 microglobulin [(0.14 ±0.05)mg/L vs.(0.53 ±0.13) mg/L],immunoglobulin G [(247.38 ±75.29) mg/L vs.(1 026.44 ±245.42)mg/L],and transferrin [(67.64 ± 16.34) mg/L vs.(249.17 ±78.62) mg/L] were significantly decreased 3 months after than 24 hours before the procedure.The renal function was higher 3 months after than 24 hours within the procedure [(8.53 ± 0.80) ％ vs.(20.50 ± 7.87) ％,P ＜ 0.05].Conclusions The increase of creatinine value and the decrease of urine pH,α1 microglobulin,microalbumin,β 2 microglobulin,immunoglobulin G and transferrin in renal pelvis urine suggested that renal function recovered after renal puncture,which is important for next-step plan of treatment strategy.