Objective:To analyze the incidence and clinical phenotype of the concomitant extragenital malformations in the patients with female reproductive tract anomalies.Methods:A retrospective study was conducted using clinical data of hospitalized patients diagnosed with uterine, cervical, or vaginal malformations from January 2003 to December 2022 in Peking Union Medical College Hospital. The malformations were classified according to American Society for Reproductive Medicine müllerian anomalies classification 2021, and in each type, the incidence and specific manifestations of concomitant extragnital malformations were analyzed.Results:A total of 444 patients were included. The overall incidence of concomitant extragenital malformations was 43.5% (193/444), including urinary system, skeletal system, and other system malformations. Renal malformations on the obstructed side were present in all patients with oblique vaginal septum syndrome (100.0%, 78/78). The total incidence of concomitant extragnital malformations was as high as 8/11 in uterus didelphys, 43.5% (10/23) in unicornuate uterus, 33.6% (79/235) in Mayer-Rokitansky-Küster-Hauser syndrome, 18.8% (6/32) in septate uterus and 18.5% (12/65) in cervical agenesis. Urinary system malformations (30.6%, 136/444) and skeletal system malformations (13.5%, 60/444) were the most common concomitant malformations in all types, in which, unilateral renal agenesis and scoliosis were the most common.Conclusions:Urinary and skeletal system malformations are important features of female reproductive tract anomalies. Urologic ultrasonography and spinal roentgenogram are recommended for all patients with female reproductive tract anomalies.

Objective:To explore the current status of medication adherence to immunosuppressants in lung transplant recipients and to analyze its correlation with medication beliefs and perceived social support.Methods:This was a cross-sectional study. From June 2022 to April 2023, totally 233 lung transplant recipients who were followed up in the Department of Lung Transplantation at China-Japan Friendship Hospital were selected by convenience sampling. The Basel Assessment of Adherence to Immunosuppressive Medications Scale (BAASIS), the Chinese version of the Beliefs about Medicines Questionnaire, and the Perceived Social Support Scale (PSSS) were used for the survey. Multiple linear regression analysis was conducted to explore the correlation between medication adherence to immunosuppressants, medication beliefs, and perceived social support in these lung transplant recipients.Results:A total of 233 questionnaires were distributed, with 213 valid responses received (91.42%). The incidence of non-adherence to immunosuppressants among the 213 transplant recipients was 41.78% (89/213), with the most common issue being not taking medication on time (27.23%, 58/213). Multiple linear regression analysis showed that age and perceived social support were influencing factors of medication adherence ( P<0.05) . Conclusions:The current level of medication adherence to immunosuppressants in lung transplant recipients is relatively poor. Medication adherence is correlated with age and the level of perceived social support. Healthcare providers should pay attention to medication adherence in younger patients and enhance their perceived social support to increase adherence to immunosuppressive medications.

OBJECTIVE: To explore the genetic basis for two children with sporadic neurofibromatosis type 1 (NF1) complicated with nephrotic syndrome (NS). METHODS: Clinical data of the children were collected. Both children were subjected to high-throughput sequencing, and candidate variants were verified by Sanger sequencing. RESULTS: Both children had café-au-lait macules, subaxillary freckle and Lisch nodules. Child 1 also had congenital tibiofibular pseudarthrosis on the left side. Genetic testing revealed that child 1 has harbored a heterozygous c.844C>T variant in the exon 8 of the NF1 gene, whilst child 2 has harbored a heterozygous c.1246C>T variant in the exon 11 of the NF1 gene. Both children were diagnosed with NF1 and have developed pronounced proteinuria, hypoalbuminemia, hypercholesterolemia and pitting edema at the ages of 3 and 10, respectively. Renal biopsy of child 2 has revealed minimal change nephropathy, and the diagnosis of nephrotic syndrome was established. Child 1 was treated with glucocorticoid, and child 2 was treated with glucocorticoid in combination with mycophenolate mofetil. The NS was relieved with no recurrence during 1 year's follow-up. CONCLUSION NF1 combined with NS is rare in the clinical settings. The prognosis of children with NF1 combined with minimal change nephropathy is relatively good. Detection of NF1 gene variant can facilitate early identification and diagnosis of NF1.
Child ; Humans ; Neurofibromatosis 1/genetics* ; Nephrotic Syndrome/genetics* ; Nephrosis, Lipoid ; Glucocorticoids ; Genetic Testing

OBJECTIVE: To explore the influencing factors of depressive symptom of employees in a mould-producing enterprises using two occupational stress models. METHODS: A total of 457 employees in a mould-producing enterprise were selected as study subjects using cluster sampling method. The Center for Epidemiological Study-Depression Scale, the Chinese version of Job Demand Content Questionnaire and the Effort-Reward Imbalance Questionnaire were used to investigate the level of depressive symptom and occupational stress by the job demand control(JDC) model and effort reward imbalance(ERI) model. RESULTS: Among the 457 employees, the median score of depressive symptom was 11.00, the detection rate of depressive symptom in the subjects was 23.4%(107/457). The incidence of occupational stress by the JDC and ERI models was 78.3%(358/457) and 62.6%(286/457), respectively. The rate of depressive symptom was statistically higher in the high occupational stress group than that of the low occupational stress group by the ERI model(26.6% vs 18.1%, P<0.05). The multivariate logistic regression analysis showed that both marital status and monthly income were influencing factors of depressive symptom by the JDC model(P<0.05). The marital status, monthly income and occupational stress were influencing factors of depressive symptom by the ERI model(P<0.05). The risk of depressive symptom was statistically higher in the high occupational stress group than that of the low occupational stress group(P<0.05). CONCLUSION: Employees in mould-producing enterprise have some depressive symptom. The marital status, monthly income and occupational stress by the ERI model are the main influencing factors of depressive symptom.

OBJECTIVE：To stud y the effects of different processed products of Whitmania pigra on hemorheology and coagulation indexes in acute blood stasis model rats. METHODS ：SD rats were randomly divided into blank group ，model group ， aspirin group ，W. pigra hang-dried product low- ，medium- and high-dose groups ，W. pigra talcum powder-ironed product low- ， medium- and high-dose groups ，W. pigra wine bran-processed product low- ，medium- and high-dose groups ，with 6 rats in each group. Except for blank group ，other groups received subcutaneous injection of epinephrine hydrochloride and ice water bath for 15 d to induce acute blood stasis model. From the 8th day of modeling ，rats in aspirin group were given aspirin 0.2 g/kg intragastrically. Rats in each dose group of W. pigra processed products were given relevant medicine 0.35，1.4，3.5 g/kg intragastrically（calculated by crude drug ）. Rats in blank group and model group were given constant volume of normal saline intragastrically， once a day ， for consecutive 8 days. Hemorheology indexes as whole blood viscosity （high， medium and low shearrate ），plasma viscosity ，erythrocyte com deformation index ，erythrocyte aggregation index ，hematocrit， and blood coagulation indexes as prothrombin time （PT）， mail：wcl19960125@163.com activated partial prothrombin time （APTT），thrombin time （TT）were determined. RESU LTS：Compared with blank group ，whole blood viscosity under different shear rates ，plasma viscosity ， erythrocyte aggregation index and hematocrit of model group were increased significantly ，while erythrocyte deformation index was significantly decreased ，PT，TT and APTT were significantly shortened （P＜0.01）. Compared with model group ，whole blood viscosity under different shear rates ，plasma viscosity ，erythrocyte aggregation index and hematocrit of aspirin group and W. pigra hang-dried product ，talcum powder-ironed product ，wine bran-processed product high-dose groups were decreased significantly ， while erythrocyte deformation index were significantly increased ，and PT （only W. pigra talcum powder-ironed products high-dose group），APTT（except for W. pigra hang-dried products high-dose group ）and TT were prolonged significantly. The whole blood viscosity of W. pigra hang-dried product medium-dose group under low shear rate ，and those of W. pigra talcum powder-ironed product low-dose ，wine bran-processed product medium-dose groups under low and medium shear rates were decreased significantly. Erythrocyte deformation index of W. pigra talcum powder-ironed product medium-dose group was increased significantly ，while erythrocyte aggregation index was decreased significantly ，and PT ，TT were prolonged significantly. APTT of W. pigra hang-dried product medium-dose group was prolonged significantly. Hematocrit of W. pigra wine bran-processed product low-dose group was decreased significantly （P＜0.05 or P＜0.01）. CONCLUSIONS ： W. pigra hang-dried， talcum powder-ironed and wine bran-processed product can effectively improve hemorheology indexes and prolong blood coagulation time.

OBJECTIVE: To detect genetic variant in a sib-pair with Finnish type congenital nephrotic syndrome (CNF). METHODS: Clinical data of the sib-pair was reviewed. Coding regions of the NPHS1 gene was analyzed for the sib-pair and both parents. RESULTS: The sister and brother respectively developed severe proteinuria 1 month and 28 days after birth, in addition with low serum albumin, hypercholesterolemia and severe edema, which were suggestive of CNF. Genetic testing identified that the sib-pair has both carried two heterozygous variants of NPHS1 gene, namely c.2605G>C (p.P869>A) and c.-61G>A, for which their father and mother were heterozygous carriers. CONCLUSION The c.2605G>C (p.869P>A) and c.-61G>A variants of the NHPS1 gene probably underlay the CNF in both sibs. The c.2605G>C(p.869P>A) was unreported previously.
Adult ; Female ; Humans ; Infant, Newborn ; Male ; Membrane Proteins/genetics* ; Mutation ; Nephrotic Syndrome/genetics* ; Siblings

Objective To evaluate the impact of gonadotropin-releasing hormone agonist(GnRH-a) used before surgery on natural pregnancy rates in patients with ovarian endometriomas. Methods In this retrospective study, 57 patients with ovarian endometriomas who had a consecutive laparoscopic surgery between June, 2010 to September, 2015 in Peking Union Medical College Hospital were included. Those patients were divided into preoperative GnRH-a treatment group(n=31)and non-GnRH-a treatment group (n=26). There were no differences in patients'characteristics between the two groups. All of them had a desire for natural pregnancy postoperatively. GnRH-a was no longer used after surgery. After the surgical procedure, the patients were observed over a period of 12 months, during which the frequency of natural pregnancy was assessed. The two groups were compared in terms of natural pregnancy rates. Results Totally 33 patients had natural pregnancy after surgery. The univariate analysis showed that the pregnancy rates of age, r-AFS stage, infertility, preoperative use of GnRH-a, tumor size, tumor side, deep infiltrating endometriosis and adenomyosis did not have statistically significant differences (all P>0.05). The two classified logistic regression showed that OR for preoperative use of GnRH-a was 0.250(95％CI: 0.064-0.978)with a statistical difference(P=0.046). Conclusion The use of GnRH-a preoperatively may have a negative effect on natural pregnancy rates of patients after surgery with ovarian endometriomas.

Objective To explore changes of urine transferrin (TFR),micro albumin (mALb),β2 microspheres protein (MG),α1MG and N-acetylβ-D amino group (NAG) in children with kidney injury induced by cytomegalovirus (CMV) infec?tion, and the outcome after treatment. Methods Fifty children with CMV infection were used as case group, and 35 chil?dren of convalescence stage of upper respiratory tract infection were used as control group. The serum levels of creatinine (Scr), blood urea nitrogen (BUN),β2MG, liver function, CMV-IgM, CMV-PCR and brainstem auditory (BAEP), head CT and urine routine test, urine TFR, mALb,β2MG,α2MG and NAG were detected. The sputum CMV-PCR was detected if childrencombined with CMV pneumonia. Ganciclovir (5mg/kg) was given to two groups, 1/12 h i.v. for 14 d. The urine TFR,mALb,β2MG, α2MG and NAG were detected again after treatment. Results There were no significant differences in the urine mALb and TFR between the two groups. The urine levels ofα1MG, NAG andβ2MG were higher in case group than those of control group. The urine levels ofα1MG, NAG andβ2MG were decreased after 2-week treatment in case group. There were no significant differences in urine mALb and TFR before and after treatment. Conclusion The combined detection ofβ2MG,α1MG and NAG can predict CMV kidney damage in children at a early stage.

Objective To investigate clinical outcome of laparoendoscopic single-site compared with traditional three-port in treatment of ovarian cystectomy.Method From February 2012 to June 2013,54 patients with ovarian cyst were randomized to the single-site group (n =24) or the three-port group (n =30) in Peking Union Medical College Hospital.The outcome of laparoscopic single-site and three-port ovarian cystectomy were evaluated.Operation time,blood loss,surgical complications,postoperative pain score,postoperative analgesic requirements,length of hospital stay,cosmetic satisfaction score,postoperative recovery and total cost were compared between both groups.Results No difference was found in blood loss,length of hospital stay,complications and total cost between the two groups(P ＞ 0.05).Operation time was (31-± 10) minute in three-port group and (40 ± 19) minute in single-site group.Visual analogue pain score was 5.6 ± 1.7 in three-port group and 2.7 ± 1.4 in single-site group,cosmetic satisfaction score was 92.5 ± 2.5 in three-port group and 97.4 ± 1.5 in single-site group.Those clinical parameter reached statistical difference (all P ＜ 0.05).Conclusion Laparoendoscopic single site surgery approach is feasible and safe to treat benign ovarian cyst disease.And it offers decreased pain and offer improved cosmesis.

Objective To explore the operation skills of laparoendoscopic single site surgery ( LESS) of total hysterectomy , to expand the application of LESS in the field of in gynecologic surgery . Methods A total of 23 cases of total hysterectomy were completed under laparoendoscopic single site surgery in Peking Union Medical College Hospital from January 2012 to August 2013, applying with combination of the traditional laparoscopic and special LESS apparatuses , intelligent electronic coagulation and excision device and the self-fixed absorbable sutures ( V-LOC).The datas of the patients during the operations and the follow-up were recorded and analyzed Results Twenty-three cases LESS-TH are accomplished.The weight of uterus was (230+38) g.The operation time was (73+22) minutes, with the intraoperative blood loss (99 ±53) ml.The postoperative intestinal function recovery time was (13 +4) hours, and postoperative hospitalized time was ( 1.8 ±0.6 ) days, with the postoperative pain visual analogue scale 3.9 ±1.6.There were no complications reported during intraoperative and postoperative time.All of our patients was finished the follow-up from 14 d to 2 months.As usual, vaginal discharge is normal, wound healed well, most of the wounds healing (19/23) hidden in umbillicus, no obvious scar, vaginal stub wound healed well Conclusions ( 1 ) LESS can accomplish total hysterectomy of uterus of gestational age less than 8 weeks safely and effectively .( 2 ) Combination of traditional laparoscopic and special LESS appatatuses , the use of intelligent energy devices and V-LOC to suture vaginal stub could make the total hysterectomy by LESS much easier and safer .