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1.
Zhonghua Er Ke Za Zhi ; 62(5): 462-466, 2024 May 02.
Artigo em Chinês | MEDLINE | ID: mdl-38623015

RESUMO

Objective: To analyze the clinical manifestations, pathology, and gene variant characteristics in children with progressive familial intrahepatic cholestasis type 3 (PFIC3). Methods: This retrospective study assessed the clinical manifestations, pathological features, gene variants, and prognosis data of 11 children with PFIC3 hospitalized in the Department of Hepatology, Fifth Medical Center, PLA General Hospital, from January 2015 to December 2022. Panel or whole exome sequencing was performed on the probands, followed by Sanger sequencing for verification within the family. Detected pathogenic variants were compared with known disease databases. Additionally, the new variants were predicted the deleteriousness and protein structure using relevant software to evaluate their pathogenicity. Results: Among the 11 PFIC3 children, 8 were boys and 3 were girls. The age of onset was 3.1 (0.2, 15.6) years. The main complaint of onset was different in the 11 patients;5 of them were abnormal liver function, 3 of them were liver and spleen enlargement, 2 of them were abdominal distension, and 1 of them was jaundice. Alanine aminotransferase, asparate aminotransferase and γ-glutamyltransferase increased in all the patients, which were(113±40), (150±44) and (270±156) U/L respectively. Moreover, direct bilirubin increased in 9 patients, and cholestasis was showed in 8 patients. All patients showed liver fibrosis on imaging, and 8 patients had cirrhosis. The pathological features of 8 cases by liver biopsy were as follows: 8 cases of fibrosis in the portal area, 7 cases of small bile duct hyperplasia, 4 cases of positive copper staining, and 5 cases of cirrhosis. A total of 17 ABCB4 gene variants were detected, including 9 new variants: c.589C>T(p.Q197X), c.1230+1G>A(Splicing), c.2914G>A(P.D972N), c.1058G>A(p.C353Y), c.956G>T(p.G319V), c.473T>A(p.L158Q), c.164T>C(p.L55S), c.2493G>C(p.R831S), and c.1150G>C(p.G384R). All 11 patients were treated with ursodeoxycholic acid and followed up for 5.1(0.6, 7.4) years. Among them, 4 cases of cirrhosis progressed continuously, 3 cases had liver transplantations, and the remaining 4 cases were stable after medical treatment. Conclusions: Children with PFIC3 have early onset, diverse clinical manifestations, rapid progression of fibrotic and cholestasis, as well as poor prognosis. Genetic testing helps to confirm the diagnosis.


Assuntos
Subfamília B de Transportador de Cassetes de Ligação de ATP/deficiência , Colestase Intra-Hepática , Sequenciamento do Exoma , Humanos , Masculino , Feminino , Colestase Intra-Hepática/genética , Colestase Intra-Hepática/diagnóstico , Estudos Retrospectivos , Criança , Pré-Escolar , Lactente , Adolescente , Mutação , Fígado/patologia , gama-Glutamiltransferase/sangue , Alanina Transaminase/sangue , Subfamília B de Transportador de Cassetes de Ligação de ATP/genética , Prognóstico , Aspartato Aminotransferases/sangue
2.
Zhonghua Kou Qiang Yi Xue Za Zhi ; 58(10): 991-995, 2023 Oct 09.
Artigo em Chinês | MEDLINE | ID: mdl-37818533

RESUMO

Orthognathic surgery changes the jaw position and occlusion, and also affects the original structure and function of the temporomandibular joint (TMJ). With the widespread development of orthognathic surgery, the impact of orthognathic surgery on the structure and function of the TMJ is increasingly valued, and the importance of the TMJ in orthognathic surgery is gradually recognized. Proper understanding the relationship between orthognathic surgery and TMJ not only helps to elucidate how the orthognathic surgery affects the condyle and causes temporomandibular disorders (TMD), but also has significant clinical significance in preventing and treating TMD in patients underwent orthognathic surgery.


Assuntos
Cirurgia Ortognática , Procedimentos Cirúrgicos Ortognáticos , Transtornos da Articulação Temporomandibular , Humanos , Procedimentos Cirúrgicos Ortognáticos/efeitos adversos , Articulação Temporomandibular/cirurgia , Transtornos da Articulação Temporomandibular/cirurgia , Transtornos da Articulação Temporomandibular/etiologia , Oclusão Dentária
3.
Zhonghua Kou Qiang Yi Xue Za Zhi ; 57(7): 708-715, 2022 Jul 02.
Artigo em Chinês | MEDLINE | ID: mdl-35790510

RESUMO

Objective: To evaluate the 2-dimension and 3-dimension changes of upper airway of patients who were diagnosed with idiopathic condylar resorption (ICR) and anterior open bite as well as received bilateral temporomandibular joint (TMJ) prostheses replacement or bimaxillary orthognathic surgery. Methods: This study is a retrospective study. Seventeen patients diagnosed as ICR and anterior open bite in Department of Orthognathic and TMJ surgery, West China Hospital of Sichuan University were selected (January 2018 to December 2021) and divided into bilateral TMJ protheses replacement group (group R, n=8) and orthognathic group (group O, n=9), according to which surgery they have performed. In order to compare variation of upper airway before and after surgery in different dimensions and sections within the same group or between groups, Spiral computed tomography data were obtained before (1 month) and after operation (10 to 12 months) to measure the total volume of airway (VT), the maximum sagittal area (MSA), the maximum cross-sectional area (MACA), the minimum cross-sectional area (MICA), the area of the most posterior plane(PPA), the area of soft-palate plane (SPA), the area of the most posterior point of tongue base plane (PTA), the area of the root of epiglottis plane (EA), the oropharyngeal airway volume (VO), the glossopharyngeal airway volume (VG) and the laryngeal airway volume (VL). Wilcoxon signed-rank test were used to complete statistical analyses for VO (T2),SPA (T2),ΔMSA,ΔMACA in group R as well as PTA (T1),EA (T2) in group O. Statistical analyses of other items were performed with student's t test. Results: VT, VO, VG, VL, MSA, MACA, MIC, PPA, PTA and EA of group R (T2) were significantly increased after TMJ prosthesis with Lefort I osteotomy (P<0.05). Meanwhile the VT, VO, VG, MSA, MACA, MICA, PPA and SPA of group O (T2) were significantly increased (P<0.05). There were significant difference in ΔVT and ΔVL between group R [(6 854.80±3 197.82) mm3, (2 252.85±1 527.96) mm3] and group O [(3 367.91±3 124.62) mm3, (413.21±1 244.44) mm3](t=2.27, P=0.038; t=2.74, P=0.015). Conclusions: Bilateral temporomandibular joint (TMJ) prostheses replacement and bimaxillary orthognathic surgery can both enlarge the areas and volumes of upper airway in patients who suffer from ICR and anterior open bite. Compared with bimaxillary orthognathic surgery, bilateral temporomandibular joint prostheses replacement plays a more pronounced role in enlargement and reconstruction of middle-inferior section of upper airway.

4.
Plant Dis ; 106(10): 2607-2617, 2022 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-35442048

RESUMO

Panax notoginseng round spot disease (PRSD), caused by Mycocentrospora acerina, is the main leaf disease occurring in cultured P. notoginseng. Aiming to find a safe and efficient control method for PRSD, we studied the disease characteristics of PRSD and the optimal growth conditions of M. acerina and evaluated the efficacy of rain-shelter cultivation in PRSD control. Moreover, we described M. acerina based on morphological characterization and molecular analyses (ITS, ACT, LSU, and TEF-1α). The optimum temperature for M. acerina conidial germination was found to be 14 to 22°C. Furthermore, leaf surface wetness for at least 4 h is required for conidial germination, and conidia can successfully infect P. notoginseng when the leaf wetness lasts for more than 8 h. Additionally, rainwater splashing determines the conidial transfection distance, which is less than 2 m. Finally, our study revealed that rain-shelter cultivation is an effective and simple physical prevention strategy to control PRSD, with an average efficacy of up to 100%.


Assuntos
Ascomicetos , Panax notoginseng , Folhas de Planta
6.
Zhonghua Kou Qiang Yi Xue Za Zhi ; 56(8): 764-768, 2021 Aug 09.
Artigo em Chinês | MEDLINE | ID: mdl-34404142

RESUMO

Objective: To comprehensively investigate the biomechanical properties of the temporomandibular joint (TMJ) disc and to perfect the mechanical testing system of the TMJ disc by conducting tests of compression, tension, cyclic compression, cyclic tension, creep and friction. Methods: Fifteen fresh goat heads (weighing 2.5-3.0 kg) were purchased from the market. They were all ordinary goats (9-12 months old, body weighing 18-21 kg) regardless of gender. Bilateral articular discs (a total of 30) were dissected within 30 minutes after execution. According to the national standard for mechanical testing of viscoelastic materials, fresh TMJ disc specimens of goat were prepared and tests were carried out in physiological conditions. The universal mechanical testing machine was utilized to test biomechanical properties of TMJ discs. Results: The compressive modulus of TMJ discs was (8.41±2.12) MPa and the tensile modulus was (9.54±3.26) MPa. The mechanical characteristics would be irreversibly altered once the load exceeded the physiological range. In addition, it underwent apparent creep relaxation under continuous strain (0.5 MPa or 3.0 MPa) and the surface friction coefficient of the TMJ discs (0.015+0.011) was much lower than that of general viscoelastic materials. Conclusions: The TMJ disc was a bio-viscoelastic structure with excellent tensile and compressive properties and its surface was extremely smooth in wet conditions.

7.
Zhonghua Zhong Liu Za Zhi ; 43(3): 318-323, 2021 Mar 23.
Artigo em Chinês | MEDLINE | ID: mdl-33752312

RESUMO

Objective: To investigate the effect of serum lipid level on prognosis of patients with small cell lung cancer (SCLC) at the initial treatment. Methods: The clinical data of patients with SCLC from 2012 to 2017 in our hospital were retrospectively analyzed. According to the standard of appropriate level and abnormal stratification of blood lipid in Chinese population, the lipids included total cholesterol (TC), triglyceride (TG), high-density lipoprotein cholesterol (HDLC) and low-density lipoprotein cholesterol (LDLC) at the time of initial treatment were grouped. Then the relationship between different lipid levels and clinicopathological characteristics was analyzed. Finally, Cox proportional hazard model was used to analyze the independent prognostic factors of patients. Results: A total of 129 patients with SCLC were included in this study. At the time of initial treatment, there were 90 (69.8%) cases whose TC < 5.2 mmol/L, while 39 (30.2%) cases ≥5.2 mmol/L; 95 (73.6%) cases whose TG <1.7 mmol/L, while 34 (26.4%) cases ≥1.7 mmol/L; 27 (20.9%) cases whose HDLC <1.0 mmol/L while 102 cases (79.1%) ≥1.0 mmol/L; 90 (69.8%) cases whose LDLC <3.4 mmol/L while 39 cases (30.2%) ≥3.4 mmol/L. The patients' triglyceride initial treatment was associated with their body mass index (P<0.05). The median disease-free survival (PFS) of SCLC patients was related with their serum TC level and clinical stage (P<0.05) and the overall survival (OS) was related with clinical stage of SCLC patients (P<0.05). The median PFS of SCLC patients in the TC <1.7 mmol/L group at the initial treatment was 10.5 months, significantly longer than 8.8 months of the TC ≥1.7 mmol/L group (P=0.024). The median OS of SCLC patients in the TG <1.7 mmol/L group at the initial treatment was 20.2 months, marginally longer than 15.6 months of the TG ≥1.7 mmol/L group (P=0.097). Multivariate analysis result showed that, the TG level was an independent risk factor of SCLC progression at the time of initial treatment (P=0.024). There was no significant correlation of TC, HDLC, LDLC and SCLC prognosis (P>0.05). Conclusion: TG level is an independent risk factor for the progression of SCLC at the time of initial treatment, and the increase of TG level indicates rapid disease progression and poor prognosis.


Assuntos
Neoplasias Pulmonares , Carcinoma de Pequenas Células do Pulmão , HDL-Colesterol , Humanos , Lipídeos , Prognóstico , Estudos Retrospectivos , Carcinoma de Pequenas Células do Pulmão/tratamento farmacológico , Triglicerídeos
8.
Eur Rev Med Pharmacol Sci ; 24(19): 10028-10035, 2020 10.
Artigo em Inglês | MEDLINE | ID: mdl-33090408

RESUMO

OBJECTIVE: Depletion of islet ß cells plays a crucial role in the onset of diabetes mellitus. Cell autophagy, as a self-healing process, contributes to maintaining metabolic homeostasis and can protect islet ß cells from apoptosis upon starvation or high glucose stress. However, the underlying regulatory network of the autophagic process in islet ß cells has not been fully explored. MATERIALS AND METHODS: Murine ß-TC3 cells treated with different concentrations of glucose, and wild-type or the Ser484 mutant human cell division cycle gene 14A (hCDC14A) was transfected. Cell viability, proliferation and autophagy as well as islet secretion were studied. The mTOR and AMPK signaling pathways were investigated by western blots. Zipper-interacting protein kinase was studied using mass spectrometry and immunoprecipitation. RESULTS: Overexpression of wild-type hCDC14A, but not the Ser484 mutant hCDC14A, promoted cell viability, proliferation and autophagy accompanied by enhanced islet secretion and reduced cell apoptosis via mTOR pathway inhibition as well AMPK pathway activation in ß-TC3 cells and vice versa. Furthermore, Zipper-interacting protein kinase (ZIPK), also known as DAPK3, was found to interact with hCDC14A primarily for Ser484 phosphorylation, and ZIPK knockdown could affect the phosphorylation of hCDC14A and weaken cell death or cell cycle modulation. CONCLUSIONS: Taken together, our results may provide new insight into the role of hCDC14A in the autophagy of islet ß cells and suggest the potential therapeutic value of hCDC14A phosphorylation in the prevention and treatment of diabetes.


Assuntos
Autofagia , Proteínas Quinases Associadas com Morte Celular/metabolismo , Ilhotas Pancreáticas/metabolismo , Proteínas Tirosina Fosfatases/metabolismo , Animais , Autofagia/efeitos dos fármacos , Linhagem Celular , Proteínas Quinases Associadas com Morte Celular/genética , Glucose/farmacologia , Ilhotas Pancreáticas/efeitos dos fármacos , Camundongos , Fosforilação/efeitos dos fármacos , Proteínas Tirosina Fosfatases/genética
9.
Zhonghua Gan Zang Bing Za Zhi ; 27(8): 604-609, 2019 Aug 20.
Artigo em Chinês | MEDLINE | ID: mdl-31594077

RESUMO

Objective: To investigate the curative effect of antiviral therapy and related factors influencing the curative affect in children with immune-tolerant phase chronic hepatitis B. Methods: From May 2014 to April 2015, 46 children with chronic hepatitis B, aged 1 to 16 years with immune-tolerant phase were enrolled as the treatment group. All cases in the treated group either received interferon alpha (3-5 MIU/m(2), once daily) in lamivudine combination (if HBV DNA decreased < 2 log(10)) or repeatedly received interferon-alpha alone (if HBV DNA decreased >2 log(10)) for 12 weeks. Interferon was discontinued at 72 weeks and followed-up period was continued with lamivudine for 24 weeks. At the same time, data of 23 cases of untreated children with immune-tolerant phase chronic hepatitis B were collected as the control group. The treatment group and the control group were divided into two age groups: 1-7 years old and 7-15 years old. Data measurements were compared using t-test, analysis of variance and single factor analysis methods, and the count data were analyzed by χ (2) test. Multiple logistic regression analysis was used to analyze the effects of different factors on response. Results: (1) There were 22 cases aged 1-7 years in the treatment group (47.8%) and 12 cases aged 1-7 years in the control group (52.2%). The cases of mother-to-child transmission (MTCT) in treatment and control group were 34 (73.9%) and 17 (73.9%), while children with normal baseline ALT in the treatment and control group were 18 (39.1%) and 10 (43.5%). (2) At the end of follow-up, 15 cases in the treatment group (32.6%) had HBeAg serological conversion. Among them, nine (19.6%) cases had HBsAg clearance or HB-Ag seroconversion with anti-HBs, and one (2.2%) case had HBsAg clearance, but both HBeAg and anti-HBe were positive. In the control group, one case had HBV DNA lower than the lower limit of detection level, and one case had HBeAg seroconversion without HBsAg clearance. (3) At the end of follow-up, the seroconversion rates of HBeAg in patients aged 1 to 7 years and patients aged 7 to 15 years were 45.5% and 20.8%, respectively (P = 0.078) and the clearance rates of HBsAg were 36.4% and 8.3% (P = 0.023). The serum conversion rates of normal and abnormal baseline alanine aminotransferase levels were 5.6% and 50.0% (P = 0.005), and the clearance rates of HBsAg were 5.6% and 32.1% (P = 0.077), respectively. There was no statistically significant difference in gender, mother-to-child transmission, HBV DNA genotyping and baseline HBsAg level in antiviral efficacy among children (P > 0.05). (4) HBsAg and HBeAg clearance occurred in 100% of patients at the end of follow-up who had HBsAg < 3 000 IU/ml at 24 weeks of treatment. (5) Multivariate logistic regression analysis showed that serum HBeAg conversion rate had relation with non-MTCT transmission and abnormal baseline alanine aminotransferase. Furthermore, HBsAg clearance rate was associated with the age of children. Conclusion: Sequential combination of interferon and lamivudine with a prolonged course can improve the HBV DNA negative conversion rate, HBeAg seroconversion rate, HBsAg loss rate and mild ALT abnormalities at baseline in children under the age of 7 years with immune-tolerant phase chronic hepatitis B.


Assuntos
Antivirais/uso terapêutico , Hepatite B Crônica/tratamento farmacológico , Interferons/uso terapêutico , Lamivudina/uso terapêutico , Adolescente , Criança , Pré-Escolar , DNA Viral/sangue , Quimioterapia Combinada , Antígenos de Superfície da Hepatite B/sangue , Antígenos E da Hepatite B/sangue , Humanos , Lactente , Transmissão Vertical de Doenças Infecciosas , Resultado do Tratamento
10.
Zhonghua Yi Xue Za Zhi ; 99(36): 2848-2854, 2019 Sep 24.
Artigo em Chinês | MEDLINE | ID: mdl-31550815

RESUMO

Objective: To explore the role and its molecular mechanism of miR-328 during the differentiation of embryonic stem cells (ESCs) into insulin-producing cells (IPCs) in vitro. Method: Mouse embryonic stem cell line-mESCs-Nanog-GFP was induced in conditioned medium and divided into negative control group, miR-328 agomir transfected group, miR-328 antagomir transfected group and transforming growth factor ß2 (TGF-ß2) siRNA transfected group. The function of IPCs was identified by real-time quantitative PCR (qPCR) detecting system and immunofluorescence in above-mentioned groups. Methods of qPCR, immunofluorescence and enzyme-linked immunosorbent assay (ELISA) were used to detect effects of overexpression and inhibition of miR-328 on differentiation of multilineage precursor cells. We predicted the binding sites of miR-328 and TGF-ß2 by performing the bioinformatics analysis. Dual luciferase reporter gene and Western blotting were employed to identify the regulatory relationship between miR-328 and TGF-ß2. Results: mESCs could be transfected with miR-328 agomir, with an efficacy of 70%-80%. Up-regulated miR-328 in MPCs reduced the RNA expression of several key transcription factors which were crucial for early pancreatic development. Additionally, the insulin released by IPCs decreased in response to glucose stimulation (all P<0.05). However, overexpression of miR-328 led to the decrease of protein level of insulin and Nkx6.1 (all P<0.05). Transfection of miR-328 antagomir had the opposite effects (P<0.05). The dual luciferase reporter gene assay revealed that miR-328 functioned via binding to the 3' non-coding region (3'-UTR) of the TGF-ß2. Western blotting indicated that miR-328 regulated protein expression. After knockdown of miR-328, the relative expression of TGF-ß2 was 1.00±0.01. After co-transfection of miR-328 antagomir and TGF-ß2 siRNA, the relative expression of TGF-ß2 was 0.80±0.03. After downregulating TGF-ß2, the relative expression of TGF-ß2 was 0.20±0.01. Knockdown of TGF-ß2 down-regulated the expression of early pancreatic transcription factors (P<0.05) and inhibited Pdx1(+)cell differentiation. Conclusion: miR-328 can inhibit the differentiation of ESCs into IPCs via binding to 3' UTR of TGF-ß2, and provide a new regulatory pathway for the treatment of diabetes with stem cells.


Assuntos
Células Secretoras de Insulina , Animais , Diferenciação Celular , Camundongos , MicroRNAs , Células-Tronco Embrionárias Murinas , Fator de Crescimento Transformador beta2
11.
Zhonghua Kou Qiang Yi Xue Za Zhi ; 54(8): 510-514, 2019 Aug 09.
Artigo em Chinês | MEDLINE | ID: mdl-31378027

RESUMO

Orthognathic surgery and temporomandibular joint (TMJ) surgery are two relatively independent disciplines in oral and maxillofacial surgery. Dentofacial deformities and TMJ diseases commonly coexist. The diagnosis and treatment of the diseases with coexisting dentofacial deformities and TMJ disorders is complex. Thus it is very important to comprehensively understand the common characteristics of orthognathic and TMJ surgery, which places a greater demand on oral and maxillofacial surgeons. The present work focuses on our thinking on common characteristics of TMJ and orthognathic surgery based on the managements of these diseases.


Assuntos
Deformidades Dentofaciais , Cirurgia Ortognática , Procedimentos Cirúrgicos Ortognáticos , Transtornos da Articulação Temporomandibular , Humanos , Articulação Temporomandibular/cirurgia , Transtornos da Articulação Temporomandibular/cirurgia
12.
Br J Biomed Sci ; 76(4): 178-183, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31210089

RESUMO

Introduction: Most patients with malignant solid tumours have abnormal blood coagulation and an abnormal peripheral blood count, but data on nasopharyngeal carcinoma is scare. We hypothesised abnormal coagulation indices and red cell distribution width (RDW) in this group that are linked to the tumour (T), lymph node (N) and metastatic aspects (M) of the patients.Methods: We recruited 740 newly diagnosed patients with nasopharyngeal carcinoma and 238 healthy controls, taking venous blood for prothrombin time, activated partial thromboplastin time (APTT), thrombin time, fibrinogen, fibrin degradation products (FDP), D-dimer, RD), platelets and platelet distribution width (PDW). In the patients, lab indices were analysed according to clinical stage.Results: All indices except thrombin time were significantly different between cases and controls (p < 0.001), and many predicted TNM classifications and early or late stage of the disease. In sensitivity/specificity analysis, the prothrombin time, APTT and PDW gave AUCs >0.7, and in combination gave an AUC of 0.88 (95% CI 0.86-0.91) for nasopharyngeal carcinoma. No index provided an AUC >0.7 for T or N classification, or early v late stage, but APTT, fibrinogen and FDP all gave AUCs ≥0.7 for predicting metastases. Together, these three indices gave an AUC of 0.84 (0.78-0.91).Conclusions: Routine coagulation indices can predict nasopharyngeal carcinoma, with the combination of prothrombin time, APTT and PDW being strongest. The combination of APTT, fibrinogen and FDPs provides a useful score to predict metastases. These indices should be considered in the diagnosis and staging of this disease.


Assuntos
Biomarcadores Tumorais/sangue , Produtos de Degradação da Fibrina e do Fibrinogênio/metabolismo , Fibrinogênio/metabolismo , Carcinoma Nasofaríngeo/diagnóstico , Neoplasias Nasofaríngeas/diagnóstico , Tempo de Protrombina , Adulto , Área Sob a Curva , Plaquetas/patologia , Estudos de Casos e Controles , Índices de Eritrócitos , Feminino , Humanos , Linfonodos/metabolismo , Linfonodos/patologia , Metástase Linfática , Masculino , Pessoa de Meia-Idade , Carcinoma Nasofaríngeo/sangue , Carcinoma Nasofaríngeo/patologia , Neoplasias Nasofaríngeas/sangue , Neoplasias Nasofaríngeas/patologia , Estadiamento de Neoplasias , Tempo de Tromboplastina Parcial , Contagem de Plaquetas , Valor Preditivo dos Testes , Tempo de Trombina
13.
Zhonghua Er Ke Za Zhi ; 57(1): 40-45, 2019 Jan 02.
Artigo em Chinês | MEDLINE | ID: mdl-30630230

RESUMO

Objective: To review and analyze the clinical and pathological data of children with autoimmune hepatitis (AIH). Methods: Medical records of 46 patients hospitalized in Pediatric Liver Diseases Treatment and Research Center, Fifth Medical Center, General Hospital of People's Liberation Army(PLA) from April 2012 to April 2018 were extracted. Medical data included type of AIH, clinical manifestations, biochemical parameters, liver biopsy results, and outcomes of treatment were analyzed retrospectively. Among 46 children, 19 were males and 27 were females. The age of onset was 10.1(1.4-18.0) years old. Chi-Square test, Rank sum test or t test were used for inter-group comparison. Results: There were 32 (70%)AIH-I cases and 14 (30%)AIH-Ⅱ cases (χ(2)=12.565, P=0.000). Among the 46 patients, there were 5 modes of onest: 17 cases (37%) had acute viral hepatitis-like presentation, 2 cases (4%) had fulminant hepatic failure, 9 cases (20%) had insidious onset, 5 cases (11%) showed cirrhosis and portal hypertension, and 13 cases (28%) were incidentally found to be due to elevated hepatic aminotransferases. Comorbidities including primary sclerotic cholangitis (n=3), primary biliary cholangitis (n=1), systemic lupus erythematosus (n=1) and inflammatory bowel disease (n=2), were all seen in AIH-Ⅰ cases. The elevated biochemical parameters of these patients were as follows: alanine aminotransferase (n=46), aspartate transminase (n=46), total bilirubin (n=35) γ-glutamyl transpeptadase (n=39), γ-globulin (n=32) and IgG (n=33). The γ-globulin and IgG levels were significantly higher in AIH-Ⅰ patients than those with AIH-Ⅱ((32±9)% vs. (23±8)%, t=3.217, P=0.002,(27±10) vs. (18±8)g/L, t=3.193, P=0.003, respectively). Thirty-nine patients received liver biopsy, among whom 22 (56%) with inflammation grade (G)≥3, 26(67%) with fibrosis stage (S) ≥3, and 7 with hepatic cirrhosis (S4) according to pathological analysis. Typical histopathological changes of AIH included: 36 cases of interfacial hepatitis (92%), 23 cases of lymphocyte/plasma cell infiltration (59%), 3 cases of rosette (8%). Forty patients received prednisolone monotherapy or combined with azathioprine after diagnosis. Complete remission was seen in 29 (72%) patients, partial remission in 10 (25%) patients and no response in 1 (3%) patient. Among complete remission patients, 15 (52%) had relapse in the process of prednisolone reduction. Repeated liver biopsy performed in 8 patients after treatment showed that hepatic inflammation and fibrosis were both improved in 6 patients, only inflammation was alleviated without fibrosis improvement in 1 patient, and neither inflammation nor fibrosis was improved in 1 case. The length of follow-up was 3.3 (0.3-10.5) years, and none of the 39 prednisolone-responded cases discontinued treatment successfully. Adverse effect of long-term prednisolone therapy included bilateral cataract (n=6), spinal fracture accompanied with delayed bone age development (n=1). Conclusions: AIH-Ⅰ is more common than AIH-Ⅱ in children, with diverse clinical characteristics. Most cases have progressive liver inflammation and fibrosis when diagnosed. Prednisolone monotherapy or combined with azathioprine could achieve both biochemical and pathological improvement, but relapse is inevitable during drug tapering, hence long-term treatment is essential.


Assuntos
Glucocorticoides , Hepatite Autoimune , Prednisolona , Adolescente , Criança , Feminino , Glucocorticoides/uso terapêutico , Hepatite Autoimune/complicações , Hepatite Autoimune/tratamento farmacológico , Hepatite Autoimune/patologia , Humanos , Cirrose Hepática , Masculino , Prednisolona/uso terapêutico , Estudos Retrospectivos , Resultado do Tratamento
14.
Br J Oral Maxillofac Surg ; 56(6): 525-530, 2018 07.
Artigo em Inglês | MEDLINE | ID: mdl-29887252

RESUMO

Mandibular distraction osteogenesis (DO) has been shown to lead to considerable improvement in obstruction of the posterior airway space in patients with ankylosis of the temporomandibular joint (TMJ), and our objective was to find out if we could confirm these findings. Seventeen patients had spiral computed tomographic (CT) scans before and after DO. After treatment, the overall posterior airway space was enlarged in all three sections of the airway (oropharyngeal, glossopharyngeal, and laryngeal). We then compared rates of change in the airway among the sections using 2-dimensional and 3-dimensional assessments, and found that the rate of change in 3-dimensional assessment of volume was significantly higher than that in the 2-dimensional (62% compared with 34%). We also found that the higher 3-dimensional rate of change came from changes in the oropharyngeal and glossopharyngeal sections, while there was no significant difference between the 2- and 3-dimensional rates of change in the laryngeal section. Because the laryngeal section had the most robust enlargement after DO in both the overall area of the posterior airway space (increased by 54%) and volume (increased by 73%), we concluded that 3-dimensional assessments were more sensitive to smaller changes in the airway space during the operation. This suggests that 3-dimensional assessments are preferable in the prediction and evaluation of the effects of DO on the posterior airway space.


Assuntos
Obstrução das Vias Respiratórias/cirurgia , Anquilose/cirurgia , Transtornos da Articulação Temporomandibular/cirurgia , Adulto , Anquilose/diagnóstico por imagem , Artroplastia/métodos , Cefalometria , Feminino , Humanos , Masculino , Osteogênese por Distração/métodos , Estudos Retrospectivos , Transtornos da Articulação Temporomandibular/diagnóstico por imagem , Tomografia Computadorizada Espiral , Resultado do Tratamento
15.
Eur Rev Med Pharmacol Sci ; 22(8): 2351-2355, 2018 04.
Artigo em Inglês | MEDLINE | ID: mdl-29762852

RESUMO

OBJECTIVE: To investigate the effects and mechanism of yes-associated protein 1 (YAP1) on thyroid carcinoma cells. MATERIALS AND METHODS: Quantitative Real-time PCR (qRT-PCR) and Western blot assay were used to detect the expression of YAP1 in normal thyroid cells (HT-ori3) and four types of thyroid carcinoma cells: FTC-133, IHH-4, TPC-1 and NPA. The cell lines with the highest expression of YAP1 were selected as the experimental materials. qRT-PCR and Western blot assay were used to detect the interference effect of si-YAP1. The cell proliferation and the effect on the PI3K-Akt signal pathway were examined by MTT and Western blot. RESULTS: The expression of YAP1 significantly increased in the thyroid carcinoma cell line compared with normal thyroid cells, among which the expression of YAP1 in TPC-1 was the highest. Quantitative PCR and Western blot results showed significant interference effects. The MTT assay indicated that YAP1 interference suppressed the proliferation of cells and the expression of p-Akt. CONCLUSIONS: The interference of YAP1 can inhibit the growth of thyroid cancer cells, and its mechanism may be associated with the PI3K-Akt signaling pathway.


Assuntos
Proteínas Adaptadoras de Transdução de Sinal/metabolismo , Fosfoproteínas/metabolismo , Interferência de RNA , Proteínas Adaptadoras de Transdução de Sinal/antagonistas & inibidores , Proteínas Adaptadoras de Transdução de Sinal/genética , Linhagem Celular Tumoral , Proliferação de Células , Humanos , Fosfatidilinositol 3-Quinases/metabolismo , Fosfoproteínas/antagonistas & inibidores , Fosfoproteínas/genética , Proteínas Proto-Oncogênicas c-akt/metabolismo , RNA Interferente Pequeno/metabolismo , Transdução de Sinais , Neoplasias da Glândula Tireoide/metabolismo , Neoplasias da Glândula Tireoide/patologia , Fatores de Transcrição , Proteínas de Sinalização YAP
16.
Br J Oral Maxillofac Surg ; 56(2): 139-143, 2018 02.
Artigo em Inglês | MEDLINE | ID: mdl-29395454

RESUMO

We know of no universally accepted classification for intracapsular condylar fractures. We propose here a new classification based on the concept of a "disc-condyle" unit, and validate the classification based on outcomes of treatment. From 1 January 2010 - 31 December 2014, 55 patients with unilateral intracapsular condylar fractures were classified into three types: type A has no reduction in mandibular height or displacement of the disc (n=7); type B has displacement of the disc with no reduction in mandibular height (n=17); and type C has reduced mandibular height with or without displacement of the disc (n=31). We treated types B and C by open reduction and fixation, while type A fractures were managed non-surgically. At six month follow-up, we found no significant differences in the vertical height of the ramus, mandibular deviation, protrusion, or lateral protrusion between the fractured and healthy sides. All patients had normal occlusion postoperatively and only one patient (type C) reported pain. Magnetic resonance imaging and computed tomography showed good osseous healing and disc-condylar relations in all cases. Our results show that this new classification of intracapsular condylar fractures is a safe and easy way to obtain satisfactory outcomes of treatment. However, it needs further independent validation.


Assuntos
Fixação de Fratura/métodos , Cápsula Articular/lesões , Côndilo Mandibular/lesões , Fraturas Mandibulares/classificação , Fraturas Mandibulares/terapia , Adolescente , Adulto , China , Feminino , Humanos , Imageamento por Ressonância Magnética , Masculino , Fraturas Mandibulares/diagnóstico por imagem , Pessoa de Meia-Idade , Tomografia Computadorizada por Raios X , Resultado do Tratamento
17.
Br J Oral Maxillofac Surg ; 56(2): 107-112, 2018 02.
Artigo em Inglês | MEDLINE | ID: mdl-29254874

RESUMO

Injury to the mandibular condyle in children usually leads to malocclusion and disharmony of facial growth. Our aim was to study the facial growth after reconstruction of the mandibular condyle using autogenous coronoid process grafts in children with unilateral ankylosis of the temporomandibular joint (TMJ). We followed up 10 growing patients with unilateral bony ankylosis of the TMJ who had been admitted to the West China Hospital of Stomatology, Sichuan University (Chengdu, China) between 1 January 2008 and 31 December 2012. There were three boys and seven girls, age range 5-12 years at the time of operation. In each case, patients were treated by gap arthroplasty, reconstruction of the condyle with an ipsilateral coronoid process, and interposition of the pedicled temporalis fascial flap during one operation. The mean (range) postoperative follow up was 4.73 (3-6) years. Postoperative panoramic radiographs were taken, and the growth of the mandibular height and length on the affected side was measured and compared with the healthy side. All patients had an uneventful, normal recovery. The mean (range) maximal mouth opening at the end of follow up was 35.6 (32-41) mm. Both the height of the ramus and the length of the mandible continued to grow after successful treatment of the ankylosis (using autogenous coronoid process grafts for reconstruction of the condyle) but the deficit in growth was not completely made up. The final height of the ramus on the affected side (at the end of follow up) had increased by 25% (p=0.012) and the final length of the mandible on the affected side by 26% (p=0.010) compared with immediately after operation. For comparison of the rate of growth, the increased height of the ramus of the affected side was 47% lower (p=0.003), while the increased length of the mandible on the affected side was 27% shorter (p=0.008) compared with the healthy side. The mandible on the affected side continued to grow after successful treatment of the ankylosis, but the growth deficit was not made up completely. The rate of growth of the affected mandible seemed to be less than on the undisturbed side even after treatment of the ankylosis.


Assuntos
Anquilose/cirurgia , Côndilo Mandibular/cirurgia , Reconstrução Mandibular/métodos , Desenvolvimento Maxilofacial , Transtornos da Articulação Temporomandibular/cirurgia , Artroplastia , Transplante Ósseo/métodos , Criança , Pré-Escolar , China , Feminino , Humanos , Masculino , Côndilo Mandibular/lesões , Radiografia Panorâmica , Retalhos Cirúrgicos , Resultado do Tratamento
18.
Int J Oral Maxillofac Surg ; 46(10): 1346-1351, 2017 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-28610820

RESUMO

Aglossia is a rare congenital abnormality, often associated with micrognathia and limb defects. Situs inversus totalis is also a rare congenital abnormality, defined as a mirror-image reversal of all the asymmetric organs of the thorax and abdomen. The concurrence of these two abnormalities has only been reported in eight similar cases in the literature. Although micrognathia and malocclusion were observed in all of these cases, few treatments were performed for the patients' dentofacial deformities. This report describes the case of a 7-year-old boy suffering from micrognathia, aglossia, and situs inversus totalis simultaneously, and the treatment for his micrognathia by mandibular symphyseal midline distraction osteogenesis, guided by virtual surgical planning and a three-dimensional printed surgical template. In a review of the literature, this is the first case of micrognathia associated with aglossia and situs inversus totalis that has been treated by mandibular symphyseal midline distraction osteogenesis for the dentofacial deformity.


Assuntos
Micrognatismo/cirurgia , Osteogênese por Distração/métodos , Situs Inversus , Língua/anormalidades , Anormalidades Múltiplas , Criança , Humanos , Masculino , Modelos Anatômicos , Impressão Tridimensional , Cirurgia Assistida por Computador
19.
Int J Oral Maxillofac Surg ; 46(7): 805-810, 2017 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-28372992

RESUMO

Mandibular distraction osteogenesis (MDO) is an effective treatment for tongue-based airway obstruction in children with severe Pierre Robin sequence. An investigation was performed to determine whether certain clinical factors influence the airway outcomes of MDO. A literature search of several databases was performed to identify studies providing individual patient data. Data extracted from the studies included patient sex, age at distraction, disease type, experience of any previous surgery on the airway, length of distraction, pre- and postoperative blood oxygen saturation nadir, and osteotomy design. Non-parametric tests and multivariate logistic regression analysis were conducted to investigate the potential interaction between these clinical factors and the efficacy of surgery. Five studies met the inclusion criteria, with data available for 73 individual patients. The results of the statistical analysis revealed that few of the factors investigated influenced the surgical efficacy in children with Pierre Robin sequence; the effect of the length of distraction was regarded as uncertain because of the limited amount of individual data available. In conclusion, no influencing factors were found, and according to this analysis, mandibular distraction may be a widely effective procedure. However, more well-designed studies and more individual data are needed to strengthen the results of this meta-analysis.


Assuntos
Obstrução das Vias Respiratórias/etiologia , Obstrução das Vias Respiratórias/cirurgia , Mandíbula/cirurgia , Osteogênese por Distração/métodos , Síndrome de Pierre Robin/complicações , Criança , Humanos , Mandíbula/anormalidades , Resultado do Tratamento
20.
Heredity (Edinb) ; 119(2): 95-106, 2017 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-28379211

RESUMO

Inferring past demography is a central question in evolutionary and conservation biology. It is, however, sometimes challenging to disentangle their roles of contemporary versus historical processes in shaping the current patterns of genetic variation in endangered species. In this study, we used both chloroplast microsatellite (cpSSR) loci and nuclear microsatellite (nSSR) loci to assess the levels of genetic differentiation, genetic effective population size, contemporary/historical levels of gene flow and demographic history for five populations sampled across the range of Dipteronia dyeriana, an endangered palaeoendemism from Southwestern China. We found that D. dyeriana had a mixed pattern of moderate genetic diversity and high inbreeding. Bayesian clustering divided D. dyeriana populations into two nSSR genetic clusters. Coalescent-based approximate Bayesian computation analyses suggest the western and eastern groups of D. dyeriana likely persisted in a long-term refuge in Southern China since the beginning of the last glacial period, whereas increasingly colder and arid climates at the onset of the last glacial maximum might have fostered the fragmentation of D. dyeriana within refugia. Following their divergence, the western group kept relatively stable effective population size, whereas the eastern group had experienced 500-fold population expansion during the Holocene. Although clear loss of genetic diversity by human activities was not suggested, recent habitat fragmentation has led to a reduction of population connectivity and increased genetic differentiation by ongoing genetic drift in isolated populations, possibly owing to decreased population size in recent dozen years. Finally, we discussed the implications of these results on conservation policies.


Assuntos
Fluxo Gênico , Variação Genética , Genética Populacional , Sapindaceae/genética , Teorema de Bayes , Biodiversidade , China , Conservação dos Recursos Naturais , DNA de Cloroplastos/genética , DNA de Plantas/genética , Espécies em Perigo de Extinção , Deriva Genética , Endogamia , Repetições de Microssatélites , Densidade Demográfica , Dinâmica Populacional
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