Purpose To study the clinic-pathological features, differential diagnosis and prognosis of extragastrointestinal stromal tumor ( EGIST) arising in the vulva and the rectovaginal septum. Methods Clinical manifestations, pathological features, immunohisto-chemistry, gene mutations, treatment and prognosis were analyzed in 1 case of EGIST arising in the vulva and 2 cases of EGIST arising in the rectovaginal septum with review of related literature. Results Case 1 was a 59-years-old woman who was found to have a 4. 4 cm × 3 cm × 3 cm recurrent mass in the right vulva after 6 months of the first resection. Case 2 was a 58-years-old woman who presen-ted with a 7. 3 cm × 6. 1 cm × 4. 6 cm mass in the rectovaginal septum. Case 3 was a 41-year-old woman who presented with an 8. 6 cm × 7. 4 cm × 6. 7 cm mass in the rectovaginal septum. Histologically, the uniform spindle cells showed the interlacing fascicular, whirl-pool and palisade patterns with high cellular density. Mitotic figures were readily identified. Immunohistochemical evaluation revealed that the tumor cells exhibited strong and diffuse staining for CD117, CD34, NES, H-Caldesmon and DOG-1. Molecular analysis showed the gene mutation of c-Kit exon 11 in all 3 cases. Conclusion EGIST should be considered in the differential diagnosis of the mesenchymal tumors arising in the vulva and the rectovaginal septum. The immunohistochemical evaluation and molecular genetic tes-ting are crucial tools for the differential diagnosis and assessment of the prognosis and targeted therapy of EGIST.

Objective To prepare a new thrombus model by fluid model method , which is suitable for evaluation of mechanical thrombectomy devices for acute stroke.Methods The fluid model adopted the Chandler loop theory , while the static model adopted conventional method.Mechanical properties of thrombi prepared by fluid model and static model were measured by manual elongation test ( 15 samples in each group) and catheter injection test (15 samples in each group ).Histological structure was compared among the above thrombus models ( 5 samples in each group ) and specimens of five patients with stroke by hematoxylin-eosin staining method.Then, thrombi in fluid model were utilized to establish embolization of vessels in swine model ( two pigs ) for evaluation of radiopacity.Independent-Sample test was performed to compare the maximum tensile length of two methods , and Fisher′s exact test was used to compare the rate of thrombus fragmentation after catheter injection test.Results The maximum tensile length of two models were (4.28 ±0.23) and (3.16 ±0.13) cm, respectively, and the difference was statistically significant (t=16.38, P<0.01);After catheter injection, the fracture rates of thrombus were 13% (2/15) and 60%(9/15), which were also statistically significant (P<0.05).As to histological structure, thrombi prepared by fluid model showed mixed thrombus structure , which similar to thrombi of stroke patients , But thrombi prepared by static model were replete with erythrocytes under the microscope .A total of eight vessels ( lingual arteries and superficial cervical arteries ) in two swines were successfully occlusive ( TIMI 0 or 1 ) , and sufficient radiopacity of each injected thrombus was observed.Conclusions The thrombi prepared by fluid model not only have good mechanical stability and sufficient radiopacity , but their histological structure is similar to thromboemboli retrieved from cerebral arteries of patients with acute stroke.

BACKGROUND:Chondrocytes co-cultured with bone marrow stromal stem cells on the scaffold of platelet-rich plasma are found to proliferate, and besides proliferative growth, bone marrow stromal cells exhibit a tendency of differentiating into chondrocytes. OBJECTIVE:To study the effect of platelet-rich plasma and human umbilical cord mesenchymal stem cells (hUCMSCs) on cartilage repair. METHODS:Forty healthy New Zealand white rabbits were selected to establish models of cartilage defects, and then randomly divided into normal saline group, platelet-rich plasma group, hUCMSCs group and combination group. Platelet-rich plasma was prepared by using double centrifugations to prepare passage 3 hUCMSCs. After modeling, intra-articular injection of normal saline (0.5 mL), 12.5%platelet-rich plasma (0.5 mL), 1×107 hUCMSCs (0.5 mL), 12.5%platelet-rich plasma+1×107 hUCMSCs (total y 0.5 mL) was done in corresponding groups, respectively. After 12 weeks of modeling, the injured cartilage was grossly observed, and hematoxylin-eosin staining was used to observe cartilage repair under light microscope;according to the O'Driscol histologic standard, histological examination was performed. RESULTS AND CONCLUSION:The repair effect in the normal saline group was significantly better that in the platelet-rich plasma group, hUCMSCs group, combination group (P<0.05), while the platelet-rich plasma group and combination group also exhibit better outcomes than the hUCMSCs group (P<0.05). These findings indicate that both platelet-rich plasma and hUCMSCs can promote cartilage repair;moreover, platelet-rich plasma with or without hUCMSCs is superior to hUCMSCs alone in the cartilage repair.

OBJECTIVE: To explore the genetic etiology for a Chinese pedigree affected with Lesch-Nyhan syndrome. METHODS: Members of the pedigree who had visited the Genetic Counseling Clinic of Linyi People's Hospital on February 10, 2022 were selected as the study subjects. Clinical data and family history of the proband were collected, and trio-whole exome sequencing (trio-WES) was carried out for the proband and his parents. Candidate variants were verified by Sanger sequencing. RESULTS: Trio-WES revealed that both the proband and his cousin brother had harbored a hemizygous c.385-1G>C variant in intron 4 of the HPRT1 gene, which was unreported previously. A heterozygous c.385-1G>C variant of the HPRT1 gene was also found in the proband's mother, grandmother, two aunts, and a female cousin, whilst all phenotypically normal males in his pedigree were found to have a wild type for the locus, which has conformed to an X-linked recessive inheritance. CONCLUSION The heterozygous c.385-1G>C variant of the HPRT1 gene probably underlay the Lesch-Nyhan syndrome in this pedigree.
Male ; Humans ; Female ; Lesch-Nyhan Syndrome/genetics* ; Pedigree ; East Asian People ; Heterozygote ; Introns ; Mutation

Objective:To investigate the intervention effects of fracture liaison service (FLS)-based intervention in elderly patients with meniscus injury, to provide reference for clinical rehabilitation.Methods:A total of 86 elderly patients with meniscus injury from February 2018 to October 2019 in the Second Affiliated Hospital of Xi ′an Jiaotong University were divided into experimental group and control group according to random number table method. There were 43 cases in each group.The control group was given routine nursing, and the experimental group implemented FLS-based nursing intervention on the basis of the control group. The clinical effects were compared between the two groups by using knee function, knee joint mobility and degree of frailty. Results:Finally, 38 cases were included in the experimental group and 41 cases in the control group. There was no significant difference in knee function, knee joint mobility and degree of frailty between the two groups at discharge ( P>0.05). At 1, 3 months after discharge, the knee scores and knee joint mobility were (75.37 ± 4.68) points, (90.34 ± 3.02) points and (96.68 ± 8.11)°, (119.11 ± 7.92)° in the experimental group, higher than those in the control group (73.17 ± 3.92) points, (87.76 ± 2.93) points and (91.76 ± 7.75)°, (108.61 ± 7.72)°, the differences were statistically significant ( t values were 2.26-5.96, all P<0.05). The knee function scores and knee joint mobility of the two groups changed with time ( F=264.33, 279.54, both P<0.05). There were interaction effects between groups and time in the knee joint mobility of the two groups ( F=6.12, P<0.05). At 1, 3, 6 months after discharge, the physiological dimension scores and frailty total scores were 5.08 ± 1.34, 4.74 ± 1.10, 4.13 ± 0.88 and 8.32 ± 1.50, 7.82 ± 1.31, 6.82 ± 0.95 in the experimental group, lower than those in the control group 5.68 ± 1.15, 5.22 ± 0.85, 5.02 ± 0.76, 9.05 ± 1.28, 8.40 ± 0.89, 8.18 ± 0.90, the differences were statistically significant ( t values were 2.15-6.57, all P<0.05). At 6 months after discharge, the psychological and cognitive dimension scores were 0.98 ± 0.30 and 0.45 ± 0.24 in the experimental group, lower than those in the control group 1.32 ± 0.37 and 0.59 ± 0.22, the differences were statistically significant ( t=4.49, 2.82, both P<0.05). The physiological and cognitive dimension scores and total scores of the two groups changed with time ( F=30.61, 31.72, 38.50, all P<0.05). There were interaction effects between groups and time in the physiological demension scores and the frailty total scores of the two groups ( F=2.86, 4.03, both P<0.05). Conclusions:FLS-based intervention can promote the rehabilitation of knee joint function and alleviate the degree of frailty of elderly patients with meniscus injury.

Objective: To explore the influences of comprehensive nursing intervention on the caregivers of severely burned children. Methods: Eighty severely burned children, admitted to our department from November 2016 to November 2017 and conforming to the study criteria, were included in this study. They were divided into comprehensive group and control group according to the random number table, with 40 children in each group. One caregiver for each child was included in the same group. Children and caregivers in control group had routine nursing care while children and caregivers in comprehensive group had comprehensive nursing care on the base of routine nursing care, including comprehensive psychological nursing of children and caregivers, guidance of rehabilitation for caregivers throughout the entire recovery process, individualized propaganda and education for caregivers on account of disease of children, perfecting social support system, sustained attention of children during follow-up. Anxiety self-rating scale and social support rating scale were used to assess the levels of anxiety and social support of caregivers of children in the two groups at the time of admission (before intervention) and the second month after discharge (the third time follow-up, after intervention). On the day of discharge, the nursing service satisfaction questionnaire of our department was used to evaluate the nursing service satisfaction of caregivers of children. Data were processed with chi-square test and independent sample t test. Results: (1) Before intervention, the anxiety and social support scores of caregivers of children in control group were respectively (64.5±3.8) and (40.4±2.5) points, similar to (63.5±4.9) and (40.5±2.6) points in comprehensive group (t=0.988, 0.177, P>0.05). After intervention, the anxiety score [(47.2±1.9) points] of caregivers of children in comprehensive group was significantly lower than that of control group [(57.6±4.3) points, t=13.917, P<0.01], and the social support score [(45.3±1.9) points] was significantly higher than (35.9±2.1) points in control group (t=-21.282, P<0.01). After intervention, scores of objective support, subjective support, and social support utilization in the social support evaluation rating of caregivers of children in comprehensive group were significantly higher than those in control group (t=12.678, -11.079, -10.031, P<0.01). (2) At the time of discharge, scores of clinical care, propaganda and education content and form in the nursing service satisfaction questionnaire of caregivers of children in comprehensive group were significantly higher than those of control group (t=-14.757, -15.627, -33.221, P<0.01). Conclusions Comprehensive nursing intervention method can effectively reduce the anxiety of the caregivers of severely burned children, help them to make better use of social support system, and improve the satisfaction of caregivers in nursing services.

At present, there are many monitoring devices for vital signs parameters for clinical selection, but urine monitoring has not received enough attention. The traditional manual visual measurement, intermittent measurement of urine pH value, and other methods are still in use, so the authors designed a multifunctional urine bag. The urine bag can set up an alarm system per unit time according to patient′s age and condition as well as set up urine pH value detection area in hard plastic measuring bottle. This device can not only accurately monitor urine volume per unit time, but also hopefully realize the alarm automation of abnormal urine volume per unit time, as well as real-time dynamic monitoring of urine pH value. It is helpful for medical staff to make accurate judgment on patients′ condition changes, to guide the formulation and modification of clinical treatment plans, and to reduce the workload of clinical nursing staff to a certain extent.