The clinical manifestation and laboratory features of juvenile hyaline fibromatosis(JHF)are reposed in a 5-year-old girl.Clinical symptoms began at 1 year of age,which was characterized by multiple cutaneous nodules and gingival hyperplasia with normal intelligence.Histopathologic examination of a skin biopsy specimen showed that the epidermis was normal,the nodules were composed of spindle-shaped fibroblasts embedded in homogeneous,non-fibrous,eosinophilic substance,and normal tissue was disrupted.Moreover,the fibroblasts showed transparent cytoplasm arranged in an obscure bundle-like pattern.No cell atypia or necrosis was observed.PCR amplification and sequencing of the capillary morphogenesis gene-2(CMG2)revealed a homozygous splicing mutation(IVS 14+1G→T)in the patient,while both of her parents were hetemzygous for the mutation.JHF is inherited in this family in an autosomal recessive manner.

Objective To evaluate the quality of life of patients with Kaschin-Beck disease (KBD)receiving total knee arthroplasty (TKA) before and after the operation.Methods Clinical efficacy of 25 patients with KBD who underwent TKA in Department of Orthopaedics,Shaanxi Provincial People's Hospital from January 2015 to January 2017 was prospectively analyzed.A questionnaire survey on KBD quality of life (KBDQOL)was carried out to evaluate the quality of life of the patients before,6 months and 1 year after the surgery.Results The scores of physical function,activity limit,social support,mental status and total health scores of KBDQOL in 6 months after the surgery (30.60 ± 3.73,23.24 ± 2.03,15.16 ± 1.62,18.92 ± 2.89,12.80 ± 2.57) and 1 year after the surgery (32.00 ± 3.19,23.76 ± 1.59,15.60 ± 1.29,20.16 ± 2.67,17.28 ± 3.88) were significantly higher than those of before the surgery (18.84 ± 4.94,21.04 ± 2.72,12.80 ± 2.06,13.68 ± 3.42,7.92 ± 1.93,P ＜ 0.05).However,there was no significant difference in economic scores before,6 months and 1 year after the surgery (10.68 ± 2.98,10.60 ± 2.78,10.48 ± 2.80,P ＞ 0.05).Conclusions The quality of life after TKA in patients with KBD severe knee osteoarthritis is significantly better than that before the surgery.The KBDQOL questionnaire is an appropriate tool for evaluating the quality of life in KBD patients.

Shared decision-making as a new model of healthcare culture is gaining more and more popularity and attention among healthcare participants. The paper successively described the concept of shared decision-making and the key points of the steps of applying shared decision-making in obstetrics, and analyzed the internal and external conditions and problems of applying shared decision-making in obstetrics wards with the help of SWOT analysis, in order to provide reference for further research and clinical practice related to shared decision-making in obstetrics wards in the future.

Objective:To analyze the impact of adolescent pregnancy on maternal and infant outcomes.Methods:A retrospective cohort study was conducted on 5 765 parturbirths in Jining Medical College Hospital from January 1, 2019 to December 31, 2020. The parturbirths were divided into adolescent group (maternal age<20 years, 280 cases), age group 1 (maternal age 20-24 years, 1 733 cases) and age groups 2 (maternal age 25-34 years, 3 752 cases). All information was collected through the hospital′s electronic case system. General data, pregnancy characteristics and outcomes were compared among the three groups by analysis of variance (ANOVA), χ 2 tests and binary logistics regression analysis was used to analyze the impact of adolescent pregnancy on maternal and infant outcomes. Results:In the adolescent group, the proportion of women with an education of junior high school or below, rural residence, no fixed income, unmarried, and no history of induced abortion were all significantly higher than those in age group 1 and age group 2 (82.50% vs 17.37%, 14.37%; 59.29% vs 42.70%, 43.36%; 80.71% vs 15.52%, 14.71%; 75.71% vs 12.23%, 9.97%; 82.50% vs 71.84%, 71.91%) (all P<0.05); there was no significant differences in age at menarche, body mass index before pregnancy, and weight gain during pregnancy among the three groups (all P>0.05). The proportion of preterm birth, low birth weight infants and transferring to neonatal intensive care unit (NICU) in the adolescent group were all significantly higher than those in age group 1 and age group 2 (5.36% vs 1.10%, 1.57%; 5.00% vs 0.23%, 0.05%; 21.79% vs 6.12%, 15.17%); the incidence of anemia in pregnancy in the adolescent group was significantly higher than that in age group 1 (15.36% vs 9.75%), and the incidence of postpartum hemorrhage was significantly higher than that in the age group 2 (10.71% vs 6.08%). The incidence of failed vaginal trials leading to cesarean section, amniotic fluid contamination, and episiotomy was significantly lower in the adolescent group than those in age group 2 (8.57% vs 15.22%, 10.71% vs 18.10%, 33.95% vs 40.01%) (all P<0.05). The incidence of failed vaginal trials leading to cesarean section was inversely associated with gestational age (adolescent group, OR=0.252, 95% CI: 0.123-0.515; age group 1, OR=0.673, 95% CI: 0.567-0.799) (both P<0.05); the risks of low birth weight infants (adolescent group, OR=7.440, 95% CI: 3.426-16.156; age group 1, OR=0.103, 95% CI: 0.032-0.330) and transferring to the NICU (adolescent group, OR=1.661, 95% CI: 1.120-2.463; age group 1, OR=0.360, 95% CI: 0.290-0.448) showed a U-shaped distribution in different pregnancy age groups, they were both higher in the adolescent group than those in the age group 2 (both P<0.05); the risk of episiotomy (adolescent group, OR=0.002, 95% CI: 0-0.016; age group 1, OR=1.308, 95% CI: 1.151-1.485) showed an inverted U-shape distribution across the different pregnancy age groups, it was lower in the adolescent group than that in age group 2 (both P<0.05). Conclusion:Adolescent pregnancy is associated with a lower risk of conversion to cesarean section and episiotomy due to failed vaginal delivery, but may increase the risk of low birth weight infants and transferring to NICU.