Ankylosis ; Humans ; Respiration Disorders ; Stomatognathic System Abnormalities ; Tongue ; abnormalities ; Trismus

Connective Tissue Growth Factor ; Humans ; Myocardial Infarction ; physiopathology ; Ventricular Remodeling

BACKGROUND: The real mechanism of systemic sclerosis is still not clear, it is found clinically that there are psychological stress factors of different forms before the attack.OBJECTIVE: To deeply investigate the psychosocial factors in patients with systemic sclerosis, and primarily analyze the role of psychological stress factors by comparing with national norms and controls as well as combining with clinical immunological test.DESIGN: A controlled observation.SETTING: The experiments were carried out in the Department of Geriatrics, the First Hospital Affiliated to Medical College, Zhejiang University and the Department of Surgery, Hangzhou Tongji Hospital.PARTICIPANTS: Between December 2002 and September 2005. 26 patients with systemic sclerosis (systemic sclerosis group), who received thoracic duct lymph drainage therapy, and 30 inpatients with chronic gastritis(control group) were selected from the same disease area of the Department of Internal Medicine, the First Hospital of Medical College, Zhejiang University.METHODS: All the patients filled the general information inventory, the contents included were age, gender, main symptoms, disease course, special examination, diagnosis and drug therapy. The psychosocial scale was used to evaluate the psychosocial factors. Life event scale (LES) was used to assess the life events stress, including profession, learning, marriage and love, family and child, economics, justice, interpersonal relationship and other common life events. The simplified coping style questionnaire (SCSQ)was applied to assess the coping styles, including 8 main components: confrontation, indifference, self-control, seeking help, self-blame, escape, planning and reassessment, and then the habitual coping styles were divided into negative ones and positive ones. The Eysenck personality questionnaire (EPQ) was used to evaluate the personality characters, it consisted of4 subscales: extraversion-introversion scale, neuroticism scale, psychoticism scale, lie and cover up scale. The fasting blood samples (3 mL) were drawn from the patients to detect serum levels of immunoglobulin G (IgG), immunoglobulin A (IgA) immunoglobulin M (IgM) and complement C3 with immunoturbidimetry, and the correlations between IgG and other factors were analyzed.MAIN OUTCOME MEASURES: The evaluative results of psychosocial scale and results of clinical immunological detection were mainly observed.RESULTS: All the 56 patients finished the scale survey and immunological detection, and all were involved in the analysis of results. The total number of life events, number of negative events and LEU value of negative events in LES were all significantly greater in the systemic sclerosis group than in the control group. For the coping style, the dimension of positive coping was less but that of negative coping was more in the systemic sclerosis group than in the control group. For EPQ, the scores of extraversion-introversion were lower but the scores of neuroticism in both males and females in the systemic sclerosis group were higher than in the control group and norms. For the immunological detection, the levels of lgG, lgA and lgM were all higher in the systemic sclerosis group than in the control group, but C3 level had insignificant difference between the two groups.IgG had negative correlations with the number of negative events, dimension of negative coping and the score of extraversion-introversion in EPQ.CONCLUSION: Patients with systemic sclerosis have obvious psychological stress, negative coping style, unstable mood and abnormal humoral immune function. Psychosocial stress has influence on immunology, it is indicated that psychological stress is closely correlated with the attack of systemic sclerosis.

Objective To evaluate the effect of self-management program in patients with rheumatoid arthritis(RA).Methods Self-management of RA patients was performed using a chronic disease self-management program (CDSMP).The health status,overall function,disease activity index,joint swelling and tenderness,joint pain score were analyzed at 12 and 24 weeks.Results At 12 and 24 weeks,health status,overall function,disease activity index,joint swelling and tenderness,joint pain score showed significant differences in two groups.Conclusion Self-management program may reduce complications,restore joint functions and improve social interactions,and improve quality of life.

Objective To evaluate the effect of self-management program in patients with rheumatoid arthritis(RA).Methods Self-management of RA patients was performed using a chronic disease self-management program (CDSMP).The health status,overall function,disease activity index,joint swelling and tenderness,joint pain score were analyzed at 12 and 24 weeks.Results At 12 and 24 weeks,health status,overall function,disease activity index,joint swelling and tenderness,joint pain score showed significant differences in two groups.Conclusion Self-management program may reduce complications,restore joint functions and improve social interactions,and improve quality of life.

Objective:To provide clinical references for the diagnosis and treatment of hemophilic pseudotumor (HPT) in maxillofacial region.Methods:Fourteen cases of HPT in maxillofacial region from the Department of Stomatology, Beijing Children′s Hospital from Jan 2009 to Jan 2019 were collected. Two cases were lost for follow-up and 12 patient,all boys, were finally followed up and included in the study. The patients aged from 13 months to 10 years old. The medical history, clinic manefestitions and the features of the radiology examination were recorded. The patients were treated by using replacement treatment first. If the conservative treatment was not effective, the patients then received operation combined with pereoperation replacement thearapy. The patients were followed up for 13 months to 10 years.There were 11 cases of hemophilia A, and 1 case of hemophilia B. Two cases were severe type, the others (10/12) were mild and moderate types. Only 1 case was diagnosed as hemophilia initially. Nine cases (9/12) were misdiagnosed as malignant tumors, 1 case was misdiagnosed as osteomyelitis and 1 case was misdiagnosed as hemangioma. Only 3 cases had identified history of trauma before.Results:All cases were treated with replacement therapy first, among which 10 cases were effective, 8 cases were cured by conservative therapy, 1 case had residual soft tissue fistula after conservative treatment and 1 case recurrented after conservative treatment for 8 months. Two patients with poor efficacy to the replacement treatment were performed operations and finally were cured.Conclusions:The misdiagnosis rate of HPT in maxillofacial region was high. The conservative factor replacement therapy could achieve good results in most children and could be used as the preferred treatment. If the conservative treatment was not effective, the surgical treatment was also a safe option.

Objective: Analyzing the characteristics of consonant errors in children with functional dysarthria in different age groups and the effect of speech training provides a reference for clinical treatment. Methods : This study followed medical ethics, and informed consent has been obtained from patients. Speech data from 388 patients with functional dysarthria were retrospectively studied. They were divided into two groups at the age of 6, namely, the preschool group (4-6 years old) of 226 patients and the school age group (6-13 years old, including 6 years old) of 162 patients. The characteristics of consonant pronunciation errors from four aspects were analyzed: average number of errors, pronunciation location, pronunciation method, and error type. One-on-one speech training was conducted, with a training frequency of once a week and once for 30 minutes. The training method was carried out in the order of phoneme training, syllable training, vocabulary training, sentence training, and short text and conversation training. The effects of speech training in the two groups were compared. Results: Analysis by pronunciation location: both age groups had the highest frequency of errors in tongue tip posterior sounds; the school age group had the lowest error frequency for labiodental consonants, and the preschool group had the lowest error frequency for bilabial consonants. According to the analysis of pronunciation mode, both age groups had the highest error frequency of aspirated affricate and the lowest error frequency of nasal sound. Analysis by error type: both age groups are mainly characterized by substitution and omission. Compared with the preschool group, most consonants of patients in the school group tend to improve in terms of pronunciation location, pronunciation mode, and error types. Compared with the preschool group, the two types of errors-palatalization and lateralization-increased in frequency in the school group, but the trend of increased lateralization was not statistically significant. After 6.7 and 5.5 sessions of speech training, the pronunciation of the preschool group and the school-age group significantly improved; the cure rate of the school-age group was 84.9% (118/139), and that of the preschool group was 77.1% (91/118). There was no statistically significant difference in the cure rate between the two groups. Conclusion Functional dysarthria may improve with age, but it may not completely self-heal. Children of different age groups can achieve good treatment results through scientific and reasonable speech training.

Objective:To evaluate the efficacy and influencing factors of surgery combined with neoadjuvant chemoradiotherapy in the treatment of children with non-orbital head and neck rhabdomyosarcoma (HNRMS).Methods:Information from 45 children diagnosed as non-orbital HNRMS and subjected to surgery combined with neoadjuvant chemoradiotherapy in Beijing Children′s Hospital affiliated to Capital Medical University from August 2017 to July 2021 was analyzed. The patients included 25 males and 20 females, aged from 1 to 17 years old. The primary tumor site, pathological subtype, clinical stage, risk group, therapeutic regimen, resection range and outcome of all cases were also collected. The survival curves were made using the Kaplan-Meier method and the potential prognostic factors were investigated by Cox regression analysis.Results:Fifteen (33.3%) of 45 children achieved negative surgical margin under complete tumor resection. The postoperative pathological results showed that there were 20 cases of embryonic subtype, 19 cases of alveolar subtype and 6 cases of spindle sclerosis subtype. The postoperative follow-up time ranged from 4 to 71 months, with a median of 26 months. During the follow-up period, 13 children died, among whom brain metastasis was the most common cause of death, accounting for 7/13. The 3-year overall survival rate was 67.6%. Multivariate analysis showed that non-embryonic subtype ( HR=6.26, 95% CI: 1.52-25.87, P=0.011) and failure to reach R0 resection ( HR=9.37, 95% CI: 1.18-74.34, P=0.034) were independent risk factors affecting overall survival rate. Conclusion:Surgery combined with neoadjuvant chemoradiotherapy can offer a good efficacy for children with non-orbital HNRMS. Non-embryonic subtype and resection without negative operative microscopic margins are independent risk factors for poor prognosis, and brain metastasis is the main cause of death in these children.

Objective:To analyze the treatment outcomes and prognoses of children with head and neck non-parameningeal rhabdomyosarcoma (HNnPM RMS).Methods:A retrospective analysis was performed on the clinical data of children with HNnPM RMS admitted to Beijing Children′s Hospital from September 2012 to September 2022. The clinical features, comprehensive treatment modes and prognoses of the patients were analyzed. The overall survival rate (OS) and event free survival rate (EFS) were calculated using the Kaplan-Meier method, and univariate analysis was performed using the Log-rank test.Results:A total of 70 children were included in this study, 38 males and 32 females, with a median age of 47 months (2-210 months). Pathological subtypes including the embryonal in 27 cases, the alveolar in 36 cases and the spindle cell and sclerosing in 7 cases. Thirty children (83.3%) with alveolar type were positive for FOXO1 gene fusion. All 70 children underwent chemotherapy, including 38 with neoadjuvant chemotherapy and 32 with adjuvant chemotherapy. Sixty of 70 children underwent surgery, of whom, 10 underwent two or more surgeries. There were 63 children underwent radiotherapy, including 54 with intensity-modulated radiation therapy, 4 with particle implantation and 5 with proton therapy. The median follow-up was 45 (5-113) months, the 5-year OS was 73.2%, and the 5-year EFS was 57.7%. Univariate analysis showed lymph node metastasis ( χ2=5.022, P=0.025), distant metastasis ( χ2=8.258, P=0.004), and high Intergroup Rhabdomyosarcoma Study (IRS) group ( χ2=9.859, P=0.029) as risk factors for poor prognosis. Before June 2016, the 5-year OS based on BCH-RMS-2006 scheme was 63.6%, and after 2016, the 5-year OS based on CCCG-RMS-2016 scheme was 79.6%. Conclusion:Multidisciplinary combined standardized treatment can offer good treatment outcome and prognosis for children with HNnPM RMS. Local control is a key to the efficacy of comprehensive treatment.

Objective To understand and improve the laboratory detection capacity of water manganese in testing organizations of Shaanxi Province． Methods The self-made manganese capacity testing samples were used as the reference in this proficiency testing program. The homogeneity of the samples was tested by single factor analysis of variance. The t-test method was used to evaluate the stability of the samples. The results of manganese in water provided by participant laboratories were analyzed by the robust statistical technique Z-score. Results A total of 136 laboratories participated in the proficiency testing program throughout the province. Among them, 129 laboratories (including retest laboratory) or 94.85% of total participating laboratories obtained satisfactory results. Conclusion The water quality monitoring agencies of our province had shown a strong detection capability for the determination of metal elements in drinking water, which ensures to provide safe water supply to the residents in Shaanxi Province.