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2.
Zhonghua Er Ke Za Zhi ; 61(12): 1086-1091, 2023 Dec 02.
Artigo em Chinês | MEDLINE | ID: mdl-38018045

RESUMO

Objective: To analyze the clinical characteristics of children with multisystem inflammatory syndrome (MIS-C) associated with SARS-CoV-2 in China, and to improve the understanding of MIS-C among pediatricians. Methods: Case series study.Collect the clinical characteristics, auxiliary examinations, treatment decisions, and prognosis of 64 patients with MIS-C from 9 hospitals in China from December 2022 to June 2023. Results: Among the 64 MIS-C patients, 36 were boys and 28 were girls, with an onset age being 2.8 (0.3, 14.0) years. All patients suffered from fever, elevated inflammatory indicators, and multiple system involvement. Forty-three patients (67%) were involved in more than 3 systems simultaneously, including skin mucosa 60 cases (94%), blood system 52 cases (89%), circulatory system 54 cases (84%), digestive system 48 cases (75%), and nervous system 24 cases (37%). Common mucocutaneous lesions included rash 54 cases (84%) and conjunctival congestion and (or) lip flushing 45 cases (70%). Hematological abnormalities consisted of coagulation dysfunction 48 cases (75%), thrombocytopenia 9 cases (14%), and lymphopenia 8 cases (13%). Cardiovascular lesions mainly affected cardiac function, of which 11 patients (17%) were accompanied by hypotension or shock, and 7 patients (12%) had coronary artery dilatation.Thirty-six patients (56%) had gastrointestinal symptoms, 23 patients (36%) had neurological symptoms. Forty-five patients (70%) received the initial treatment of intravenous immunoglobulin in combination with glucocorticoids, 5 patients (8%) received the methylprednisolone pulse therapy and 2 patients (3%) treated with biological agents, 7 patients with coronary artery dilation all returned to normal within 6 months. Conclusions: MIS-C patients are mainly characterized by fever, high inflammatory response, and multiple organ damage. The preferred initial treatment is intravenous immunoglobulin combined with glucocorticoids. All patients have a good prognosis.


Assuntos
COVID-19 , Doenças do Tecido Conjuntivo , Aneurisma Coronário , Masculino , Criança , Feminino , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Coagulação Sanguínea , China/epidemiologia , Febre , Síndrome de Resposta Inflamatória Sistêmica/diagnóstico , Síndrome de Resposta Inflamatória Sistêmica/terapia
3.
Clin Radiol ; 78(10): 755-762, 2023 10.
Artigo em Inglês | MEDLINE | ID: mdl-37558538

RESUMO

AIM: To evaluate the diagnostic value and safety of ultrasound-guided core-needle biopsy for peripheral pulmonary lesions (PPLs). MATERIALS AND METHODS: PubMed, EMBASE, and the Cochrane Library for relevant were searched for studies published up to June 2022. The diagnostic accuracy of US-guided percutaneous transthoracic needle biopsy (PTNB) for the diagnosis of PPLs was evaluated using pooled sensitivity, specificity, diagnostic odds ratio (DOR), positive and negative likelihood ratios (PLR and NLR), and the area under the summary receiver operating characteristic curves value (SROC). RESULTS: The search included 12 original studies (3,830 procedures). For US-guided PTNB, the pooled sensitivity and specificity for the diagnosis of PPLs were 0.93 (95% confidence interval [CI]: 0.91-0.94) and 0.99 (95% CI: 0.96-1.00), respectively. The pooled estimates of the PLR, NLR, and DOR were 134.88 (95% CI: 24.88-731.74), 0.07 (95% CI: 0.06-0.09), and 1,814.95 (95% CI: 333.62-9,873.76), respectively. The area under the SROC curve was 0.95 (95% CI: 0.93-0.97). The overall complication rate was 3.6% (136 of 3,830), including self-limited haemoptysis and asymptomatic pneumothorax, and only six cases of pneumothorax requiring chest tube drainage and one case of severe bleeding were reported. CONCLUSIONS: US-guided core-needle biopsy is an excellent diagnostic tool for PPLs, with high accuracy and excellent technical performance and safety.


Assuntos
Pneumotórax , Humanos , Biópsia Guiada por Imagem , Biópsia com Agulha de Grande Calibre/efeitos adversos , Sensibilidade e Especificidade , Ultrassonografia de Intervenção
8.
Zhonghua Er Ke Za Zhi ; 59(12): 1038-1042, 2021 Dec 02.
Artigo em Chinês | MEDLINE | ID: mdl-34856662

RESUMO

Objective: To summarize the clinical characteristics and explore the risk factors of recurrent Kawasaki disease. Methods: In this retrospective study, reviewed 41 cases with recurrent Kawasaki disease in Department of Rheumatology and Immunology, Children's Hospital of Chongqing Medical University from January 2013 to January 2021. And another 123 children with Kawasaki disease who had no recurrence during at least 6 years of follow-up were assigned into control group. Furthermore, the risk factors of recurrence were derived by comparing the clinical characteristics of recurrent cases at their initial episodes with those of control cases by Chi-square test and the Mann-Whitney U test, followed by Logistic regression and receiver operating characteristic analysis. Results: There were 29 males and 12 females in 41 children with recurrent Kawasaki disease, 33 children (80%) suffered a recurrence within 2 years after the first episode and 8 children (20%) developed a recurrence after 2 years. Compared with the first episode, the second episode had lower white blood cell count (15.2 (12.8-18.8)×109 vs. 18.0 (14.9-23.4)×109/L, Z=-2.462, P=0.014) and rate of edema in extremities (54% (22/41) vs. 76% (31/41), χ2=4.321, P=0.038), shorter fever durations before intravenous immunoglobulin treatment (5.0 (5.0-6.0) vs. 6.0 (5.0-7.5) d, Z=-3.329, P=0.001) and higher levels of hemoglobin ((116±8) vs. (107±12)g/L, t=-4.124, P<0.05) and albumin((39±5) vs. (36±6) g/L, t=-3.009, P=0.004). Multivariate Logistic regression analysis showed that C-reaction protein>97.5 mg/L (OR=3.014, 95%CI 1.350-6.730, P=0.007), platelet >276 × 109/L (OR=4.099, 95%CI 1.309-12.838, P=0.015), intravenous immunoglobulin resistance (OR=9.239, 95%CI 1.178-72.477, P=0.034), Mycoplasma pneumoniae infection (OR=2.585, 95%CI 1.129-5.922, P=0.025) were independent risk factors for recurrent Kawasaki disease recurrence.The predictive model then was generated using these four risk factors. The receiver operating characteristic analysis showed that the area under curve was 0.732 (95%CI 0.647-0.817). When the cut-off was 0.241, the sensitivity and specificity were 63.4% and 70.7%, respectively. Conclusions: Children with Kawasaki disease should be followed up for at least 2 years after the first episode and should pay more attention to C-reactive protein. Children with Mycoplasma pneumoniae infection, intravenous immunoglobulin resistance, higher C-reactive protein and platelet at the first onset have a higher risk of recurrent Kawasaki disease.


Assuntos
Síndrome de Linfonodos Mucocutâneos , Pneumonia por Mycoplasma , Feminino , Humanos , Masculino , Síndrome de Linfonodos Mucocutâneos/complicações , Síndrome de Linfonodos Mucocutâneos/epidemiologia , Curva ROC , Estudos Retrospectivos , Fatores de Risco
9.
Zhonghua Er Ke Za Zhi ; 59(12): 1086-1087, 2021 Dec 02.
Artigo em Chinês | MEDLINE | ID: mdl-34856669
10.
Zhonghua Wai Ke Za Zhi ; 59(9): 738-745, 2021 Sep 01.
Artigo em Chinês | MEDLINE | ID: mdl-34404171

RESUMO

Objective: To investigate the causes,prevention and treatment of femoral artery puncture related complications caused by the application of resuscitative endovascular balloon occlusion of the aorta (REBOA) in the resection of pelvic and sacral tumors. Methods: Clinical data of 23 patients with femoral artery puncture related complications who received REBOA in the resection of pelvic and sacral tumors from August 2010 to August 2018 at the Musculoskeletal Tumor Center,Peking University People's Hospital were retrospectively analyzed.There were 8 males and 15 females,with the an age of (37.0±16.2) years (range:15 to 65 years).Arterial access via the Seldinger technique for REBOA was obtained in the right common femoral artery of 18 cases,and in the left of 6 cases.An arterial sheath with a diameter of 11 to 12 F(1 F≈0.33 mm) was used for the patient.The occurrence and treatment of postoperative complications were analyzed. Results: Acute femoral arterial thrombosis occurred in 18 patients,which was managed by open repair 48 hours postoperatively.Among the 349 patients admitted before 2015 who received hemostasis by compression after femoral artery sheath removal,12 patients (3.4%) developed acute femoral artery thrombosis.While the 476 patients admitted after 2015 who used a vascular stapler to close the femoral artery wound,6 patients (1.3%) developed acute femoral artery thrombosis.One case of retroperitoneal hematoma and 1 case of femoral pseudoaneurysm were found and surgically fixed.Postoperative follow-up was (40±18) months (range:13 to 108 months).Three cases with chronic lower extremity ischemia were confirmed by Doppler ultrasonography during 1 to 5 years follow-up.Two of them had minimal symptoms and denied further treatment,while the other one received femoral-femoral artery bypass surgery to restore distal flow for pain and numbness relief. Conclusions: Acute femoral arterial thrombosis was the most common femoral artery puncture.Technique refinement of REBOA,the use of percutaneous suture device and close follow-up can reduce the approach-specific complications,and help to detect and treat the complications timely,which may popularize the clinical application of REBOA.

11.
Zhonghua Er Ke Za Zhi ; 59(7): 576-581, 2021 Jul 02.
Artigo em Chinês | MEDLINE | ID: mdl-34405640

RESUMO

Objective: To explore the clinical characteristics and risk factors of pediatric patients with Wiskott-Aldrich syndrome (WAS). Methods: This was a case-control study. Clinical data of 165 cases of pediatric patients with WAS, who visited the Department of Rheumatology, Children's Hospital of Chongqing Medical University between January 2007 and August 2020 were retrospectively analyzed and divided into death group and survival group (control group) according to the prognosis in the follow-up. Two independent samples t-test, Welch approximate t-test, Mann-Whitney U test, Pearson χ² test, Yates corrected χ² test, or Fisher exact probability test were used for comparison between groups. Risk factors were analyzed by multivariate Logistic regression analysis. Results: A total of 165 patients with Wiskott-Aldrich syndrome were enrolled in this study, including 40 cases in the death group and 125 cases in the survival group. The WAS score was (4.1±0.8) score in the death group and (3.1±1.2) score in the survival group. The age was 19 (9, 28) months in the death group and 60 (36,86) in the survival group. The episode rates of recurrent infection and (or) severe infection, intracranial hemorrhage and eczema in the death group were significantly higher than those in the survival group (95.0% (38/40) vs.32.0% (40/125),25.0% (10/40) vs. 2.4% (3/125), 90.0% (36/40) vs. 72.0% (90/125), χ²=48.253, 18.325, 5.440, all P<0.05). Infection (22 cases, 55.0%) and intracerebral hemorrhage (15 cases, 37.5%) were the main causes of death, 3 cases (7.5%) died of severe graft-versus-host disease after transplantation. The Logistic regression model indicated that repeated infection and (or) severe infection and non-use of intravenous immunoglobulin (IVIG) replacement therapy were risk factors for death in Chinese WAS patients (OR values were 8.999 and 2.860, 95% CI were (2.041-39.667) and (1.375-5.950), respectively, all P<0.05). Conclusions: Recurrent and (or) severe infection is the main risk factor of death for WAS patietns. Regular IVIG treatment can improve the survival rate of patients with WAS.


Assuntos
Doença Enxerto-Hospedeiro , Síndrome de Wiskott-Aldrich , Estudos de Casos e Controles , Criança , Humanos , Lactente , Prognóstico , Estudos Retrospectivos , Fatores de Risco
12.
Zhonghua Er Ke Za Zhi ; 59(6): 495-500, 2021 Jun 02.
Artigo em Chinês | MEDLINE | ID: mdl-34102824

RESUMO

Objective: To analyze the clinical characteristics of congenital agammaglobulinemia and the efficacy of intravenous immunoglobulin (IVIG) replacement therapy for this disease. Methods: The basic characteristics, clinical manifestations, laboratory examinations, and outcomes of 114 patients with congenital agammaglobulinemia diagnosed in Children's Hospital of Chongqing Medical University from January 1988 to April 2020 were retrospectively analyzed. The efficacy of IVIG in improving the clinical symptoms between regular and irregular treatment groups were compared by χ2 test. To explore the clinical characteristics associated with delayed diagnosis and treatment, the patients were also stratified into following subgroups: non-cough, short-term cough and long-term cough groups, chronic lung disease and non-chronic lung disease groups, and arthritis and non-arthritis groups. The age at onset, age at diagnosis, time consumed for diagnosis, initial time of immunoglobulin replacement, dose of IVIG, IgG trough level between the above groups were compared by t test, F test or non-parametric test. Results: All the 114 patients were male, with the onset age of (22±18) months. The age at diagnosis was (89±54) months, time consumed in diagnosis was (63±46) months, and the initial time of immunoglobulin replacement was (75±45) months. A total of 66 patients had been followed up to April 2020, with a follow-up period of (54±41) months. Among these children, 42 (63.6%) received regular infusion, whose monthly IVIG dose was (538±105) mg/kg and IgG trough level was (5.8±1.5) g/L, whereas 24 patients (36.4%) were treated irregularly. There was no significant difference in the improvement rate of fever, cough, sinusitis, diarrhea, otitis media and arthritis between regular and irregular IVIG replacement groups (all P>0.05). Sixty-one out of the 66 patients (92.4%) had fever before IVIG treatment, whose fever episodes were significantly decreased after IVIG treatment (5 (2,12) vs. 0 (0, 1) per year, Z =-6.436, P<0.01). Sixty patients (90.9%) suffered from wet cough before treatment and 36 (54.5%) after treatment. Initial time of immunoglobulin replacement was significantly delayed in the long-term cough (27 cases) and short-term cough groups (9 cases) compared with non-cough group (18 cases) ((97±51) vs. (64±41) vs. (63±42) months, F=3.554, P=0.035). Twenty-nine patients (43.9%) were diagnosed with chronic lung disease, whose initial time of immunoglobulin replacement (103 (75,144) vs. 46 (26,64) months, Z=-4.330, P<0.01), age at diagnosis (103 (75,142) vs. 47 (31,68) months, Z=-3.486, P<0.01), and time consumed in diagnosis (91 (55,129) vs. 29 (10,41) months, Z =-4.386, P<0.01) were significantly later and longer than those in children without chronic lung disease (37 cases). In addition, thirty-two patients(48.5%) were diagnosed with arthritis, whose initial time of immunoglobulin replacement ((98±51) vs. (58±39) months,t=3.420, P=0.001) and time consumed in diagnosis ((74±49) vs. (44±40) months, t=2.600, P=0.010) were also significantly later and longer than those in children without arthritis (34 cases). Conclusions: After immunoglobulin replacement therapy, the clinical symptoms such as fever, sinusitis, diarrhea, and otitis media can be improved more or less. However, long-term wet cough, chronic lung disease, and arthritis are still prominent clinical problems, which could be controlled by standard immunoglobulin replacement therapy in some patients.


Assuntos
Agamaglobulinemia , Doenças Genéticas Ligadas ao Cromossomo X , Agamaglobulinemia/tratamento farmacológico , Criança , Pré-Escolar , Feminino , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Lactente , Masculino , Estudos Retrospectivos
13.
Zhonghua Er Ke Za Zhi ; 58(12): 966-972, 2020 Dec 02.
Artigo em Chinês | MEDLINE | ID: mdl-33256317

RESUMO

Objective: To elucidate the relationship between the myositis autoantibodies and clinical phenotypes of juvenile dermatomyositis (JDM). Methods: A total of 76 JDM patients admitted to the Children's Hospital of Chongqing Medical University from January 2017 to May 2020 were tested for myositis autoantibodies, including myositis-specific autoantibodies (MSAs) and myositis-associated autoantibodies (MAAs). Kruskal-Wallis test and logistic regression were used to analyze the relationship between the antibodies and clinical characteristics. Results: In the 76 cases, 37 were females and 39 males. Forty-three cases (53%) were MSAs positive, among which the most common subtypes were autoantibodies against nuclear matrix protein 2 (NXP2) (15/76, 20%), melanoma differentiation-associated protein 5 (MDA5) (13/76, 17%) and transcription intermediary factor 1 gamma (9/76, 11%). While 20 cases (26%) were positive for MAAs, among which anti-Ro-52 antibody (17/76, 22%) was the most common subtype. Sixteen patients (21%) had both MAAs and MSAs. The incidences of arthritis (9 cases), fever (8 cases), skin ulcers (5 cases) and macrophage activation syndrome (MAS) (1 case) were significantly higher in the patients with anti-MDA5 antibody among the antibody groups. Dysphasia (6 cases) and edema (8 cases) mainly occurred in patients with anti-NXP2 antibody. Children with anti-MDA5 antibody were more likely to develop arthritis (OR=10.636, 95%CI: 2.770-40.844, P=0.001), skin ulcers (OR=12.500, 95%CI: 2.498-62.522, P=0.002), fever (OR=5.600, 95%CI: 1.580-19.849, P=0.008) and interstitial lung disease (ILD) (OR=23.333, 95%CI: 4.750-114.616, P<0.01). In the patients with different subtypes of MSAs, the creatine kinase value was significantly lower in the anti-MDA5 group than those in the anti-aminoacyl-tRNA synthetase (P=0.03), anti-NXP2 (P<0.01), and MSAs-negative group (P=0.013). Conclusions: Most children with JDM have positive myositis autoantibodies, and will present with different clinical phenotypes based on different autoantibodies. And children with anti-MDA5 antibodies are likely to develop ILD and MAS. Therefore the detection of myositis-specific autoantibodies is important.


Assuntos
Autoanticorpos/imunologia , Dermatomiosite/classificação , Dermatomiosite/imunologia , Fenótipo , Criança , Feminino , Humanos , Doenças Pulmonares Intersticiais , Síndrome de Ativação Macrofágica , Masculino
15.
Zhonghua Er Ke Za Zhi ; 58(3): 223-227, 2020 Mar 02.
Artigo em Chinês | MEDLINE | ID: mdl-32135595

RESUMO

Objective: To analyze the clinical , immunological and genetic features of a child with BCL11B mutation induced neurodevelopmental disorder. Methods: The clinical data and genetic test of a child with BCL11B mutation hospitalized in the Department of Rheumatology and Immunology in Children's Hospital of Chongqing Medical University in December 2018 were extracted and analyzed. The literature was searched with "BCL11B mutation" and "immunodeficiency 49" as key words in Chinese databases and Pubmed until January 2019 was reviewed. Results: A male patient aged 3 years and 11 months with facial dysmorphisms and delayed language and motor development was admitted due to neurodevelopmental retardation over two years. Laboratory tests showed normal human immunoglobulin (IgG 12.90 g/L, IgA 1.02 g/L, IgM 1.15 g/L, IgE 532 000 U/L), Trec (228) and proliferation of T and B cells. The lymphocyte subsets revealeda reduced percentage of B cells (0.108) but normal absolute numbers (0.574×10(-3)/L), and an increased percentage (0.828) as well as absolute numbers (4.415×10(-3)/L) of T cells. A heterozygous BCL11B mutation was detected by sanger sequencing, showing a de novo frameshift mutation c.1887_c.1893delCGGCGGG in exon 4. Two papers were found which were all in English, with total of 14 patients(13 patients with complete information). Thirteen mutations were reposed, including 7 frameshift, 2 nonsense, 2 missense, and 2 chromosomal rearrangements; Thirteen patients had heterozygous mutations. All patients had delayed language and motor development and facial dysplasia which were mainly hypertelorism, thin eyebrows and small palpebral fissures. Some patients had dental anomalies, ametropia and allergy, and a few were combined with immune impairment, but without overt signs of immunodeficiency. Only one patient had multisystem anomalies and profound immune deficiency. Conclusions: BCL11B is essential for development of the nervous and the immune system. In this study, the de novo mutation of BCL11B gene resulted in neurodevelopmental and immunological disorders.


Assuntos
Transtornos do Neurodesenvolvimento , Fatores de Transcrição , Proteínas Supressoras de Tumor , Pré-Escolar , Heterozigoto , Humanos , Masculino , Mutação , Transtornos do Neurodesenvolvimento/genética , Proteínas Repressoras , Fatores de Transcrição/genética , Proteínas Supressoras de Tumor/genética
16.
Zhonghua Er Bi Yan Hou Tou Jing Wai Ke Za Zhi ; 54(11): 857-862, 2019 Nov 07.
Artigo em Chinês | MEDLINE | ID: mdl-31795548

RESUMO

Objective: To investigate the migration and invasion behaviors of Hep-2 after the targeted knockdown of yes-associated protein (YAP). Methods: Hep-2 cells were knock-downed for YAP by shRNA as YAP-shRNA group, Hep-2 treated with non-specific shRNA as YAP-NC group, and Hep-2 with no treatment as control. Glucose uptake and lactate production in the cells were examined to assess Warburg effect. The migration and invasion behaviors of cells in three groups were observed. The expressions of vimentin and E-cadherin were detected by RT-PCR and Western Blot. The statistical software GraphPad Prism 7.0 was used to analyze significance of data. Two tailed Student' s t-tests was used to determine significance when only two groups were compared. P values of less than 0.05 was considered statistically significant. Results: Downregulation of YAP led to a obvious decrease in glucose uptake [(18.51±1.72)%] and lactate production [103.40±8.32] in Hep-2 cells compared with control [(41.20±1.11)% and 743.69±19.49, t=19.20 and 52.33, respectively, both P<0.01] and YAP-NC group [(39.60±0.78)% and 705.22±17.20, t=19.34 and 54.56, respectively, both P<0.01]. Compared with the control group (78.32±4.04) and YAP-NC group (77.28±3.11), the scratch healing ability of Hep-2 cells was significantly decreased in YAP-shRNA group (44.71±4.68). The P value was less than 0.01 (t=9.42 and 10.04). The number of cells with YAP-shRNA (33.30±4.19) passing through compartments was remarkable fewer than the control group (133.71±6.72) and YAP-NC group (126.32±4.21). The P value was less than 0.01 (t=21.96 and 27.13). The expression of E-cadherin protein in cells of YAP-shRNA group (6.16±0.11) was up-regulated compared with control (0.97±0.10, t=35.70, P<0.01) and YAP-NC group (1.13±0.09, t=36.28, P<0.01), while the expression of vimentin protein in cells of YAP-shRNA group (1.08±0.09) was down-regulated compared with control (5.67±0.12, t=29.91, P<0.01) and YAP-NC group (5.51±0.12, t=29.04, P<0.01). Conclusions: The down-regulation of YAP in Hep-2 inhibits the migration and invasion of cells via suppressing Warburg and EMT program.


Assuntos
Proteínas Adaptadoras de Transdução de Sinal/genética , Neoplasias Laríngeas/genética , Neoplasias Laríngeas/metabolismo , Fatores de Transcrição/genética , Proteínas Adaptadoras de Transdução de Sinal/biossíntese , Caderinas/biossíntese , Linhagem Celular Tumoral , Movimento Celular , Proliferação de Células , Regulação para Baixo , Transição Epitelial-Mesenquimal , Humanos , Neoplasias Laríngeas/patologia , Invasividade Neoplásica , RNA Interferente Pequeno/genética , Fatores de Transcrição/biossíntese , Vimentina/biossíntese , Proteínas de Sinalização YAP
17.
Eur Rev Med Pharmacol Sci ; 22(20): 6609-6615, 2018 10.
Artigo em Inglês | MEDLINE | ID: mdl-30402832

RESUMO

OBJECTIVE: To investigate the effect of microRNA-210 on the spinal cord injury (SCI) and its underlying mechanism. MATERIALS AND METHODS: The mouse SCI model was established. Mice were randomly assigned into 4 groups, namely the sham operation group (sham group), surgery group (SCI group), surgery+NC group (SCI+NC group) and surgery+microRNA-210 overexpression group (SCI+microRNA-210 mimics group). The mRNA levels of microRNA-210 and the key genes in the JAK-STAT pathway of the four groups were detected by Real-Time Polymerase Chain Reaction (RT-PCR) at different time points. Protein levels of JAK2 and STAT3 in mice of the four groups were detected by Western blot. To investigate the role of microRNA-210 in SCI recovery, changes in the motor function of mice were detected. RESULTS: Grip strengths of right and left forelimbs in mice from the sham group were temporarily decreased at the early stage after surgery, which were gradually recovered to the preoperative levels on the 3rd postoperative day. However, mice in SCI group were unable to complete the grip strength determination at the early stage after surgery. Mice in SCI group were capable of grasping on the 7th postoperative day. Besides, grip strengths of mice in SCI group were remarkably lower than those of sham group until the end-point (on the 50th day). Furthermore, mRNA levels of microRNA-210 in mice of SCI group were decreased in a time-dependent manner (p<0.05). Higher grip strengths were observed in mice of SCI+microRNA-210 mimics group in comparison with those of SCI group and SCI+NC group (p<0.05). In addition, Western blot showed that protein levels of JAK2 and STAT3 in mice of SCI group were increased in a time-dependent manner (p<0.05). Moreover, protein levels of JAK2, STAT3, and MCP-1 in mice of SCI+NC group were remarkably higher than those in the sham group and SCI+microRNA-210 mimics group (p<0.05). CONCLUSIONS: MicroRNA-210 is down-regulated in SCI mice. Grip strengths of SCI mice can be recovered after microRNA-210 overexpression via inhibiting inflammatory response by the JAK-STAT pathway.


Assuntos
Inflamação/enzimologia , Janus Quinase 2/metabolismo , MicroRNAs/metabolismo , Fator de Transcrição STAT3/metabolismo , Traumatismos da Medula Espinal/enzimologia , Medula Espinal/enzimologia , Animais , Comportamento Animal , Modelos Animais de Doenças , Inflamação/genética , Inflamação/fisiopatologia , Camundongos , MicroRNAs/genética , Força Muscular , Recuperação de Função Fisiológica , Transdução de Sinais , Medula Espinal/fisiopatologia , Traumatismos da Medula Espinal/genética , Traumatismos da Medula Espinal/fisiopatologia
18.
Zhonghua Er Ke Za Zhi ; 56(8): 628-632, 2018 Aug 02.
Artigo em Chinês | MEDLINE | ID: mdl-30078247

RESUMO

Objective: To investigate the clinical, immunological, and molecular manifestations of nuclear factor kappa-B subunit 2 (NFκB2) gene mutation associated common variable immunodeficiency (CVID) . Methods: A 14-month-old boy diagnosed with NFκB2-mutated CVID was admitted into Children's Hospital of Chongqing Medical University in December 2015. The clinical manifestations, biochemical tests, immunological function, molecular features, treatment, and follow-up of the patient were analyzed. The Chinese and PUBMED databases were searched with the key words "NFκB2" and "immune deficiency" and related literatures were reviewed. Results: The patient had 4 episodes of pneumonias and one otitis media since the age of 6 months. The serum immunoglobulin levels were IgG 2.73 g/L, IgA<0.07 g/L, and IgM 0.12 g/L. The percentage of peripheral lymphocyte subsets demonstrated increased CD3(+)T lymphocyte (81.8%), increased CD4(+) naïve T cell (39.1%), normal B cell (14.1%), low switched memory B and plasmablast B (respectively 0.1% and 0), and lightly diminished natural killer(NK) cell (4.13%). Within the peripheral CD4(+)T cells, the percentage of regulatory T cells (1.49% (control 4.08%)), T follicular helper (3.66% (control 11.0%)), and T helper 17 (9.65% (control 15.7%)) were decreased, while the percentage of T helper 2 (60.9% (control 46.5%)) was elevated. T lymphocyte proliferative response and T cell receptor repertoire diversity were normal. NK-cell cytotoxic activity was impaired. The whole-exome sequencing harbored a de novo heterozygous nonsense mutation in exon 22 (c.2557C>T; p. Arg853X) in the C-terminus of NF-κB2. The western blotting confirmed the decreased expression of NF-κB2 (p52) protein. The patient received intravenous immunoglobulin infusion monthly (400-600 mg/kg), followed by improvement of pulmonary infection. After searching the databases, a total of 28 cases (1 Chinese and 27 non-Chinese) were identified. There were 12 cases of nonsense mutation (5 were gain-of-function mutation), and 8 cases of missense and frameshift mutations, respectively. The main clinical manifestation was respiratory infection, followed by autoimmune diseases such as alopecia and trachyonychia. Fifteen cases developed adrenocorticotrophic hormone (ACTH) deficiency. Conclusions: NF-κB2 signaling pathway played an important role in T and B lymphocyte differentiation, and NK-cell cytotoxic activity. NFκB2 mutation should be considered in cases with recurrent infections, hypogammaglobulinemia, and decreased memory B cells and plasma cells, especially when combined with ACTH deficiency.


Assuntos
Imunodeficiência de Variável Comum , Mutação , Subunidade p52 de NF-kappa B , Agamaglobulinemia , Imunodeficiência de Variável Comum/complicações , Imunodeficiência de Variável Comum/genética , Imunodeficiência de Variável Comum/imunologia , Heterozigoto , Humanos , Lactente , Masculino , Subunidade p52 de NF-kappa B/genética , Sequenciamento do Exoma
20.
Eur Rev Med Pharmacol Sci ; 22(14): 4604-4610, 2018 07.
Artigo em Inglês | MEDLINE | ID: mdl-30058697

RESUMO

OBJECTIVE: Acute kidney injury (AKI) is common in critically ill patients, and sepsis patients with AKI had a higher mortality rate. The aim of the present study was to determine the potential value of urinary miR-26b in the diagnosis of sepsis-associated AKI. PATIENTS AND METHODS: Urinary samples were collected from a cohort of 155 sepsis patients (68 AKI patients and 87 non-AKI patients) and 57 patients with non-infectious systemic inflammatory response syndrome (SIRS). The expression levels of urinary miR-26b were measured by RT-qPCR analysis. ROC curve analysis was performed to determine the diagnostic value. Pearson correlation analysis was performed to assess the levels of urinary miR-26b and several clinical parameters. Kaplan-Meier curves were plotted to show the impact of urinary miR-26b on the 28-day survival. RESULTS: Significantly increased urinary miR-26b levels were found in patients with sepsis-associated AKI. Urinary miR-26b had a sensitivity of 90.8% and specificity of 75.0% for distinguishing between AKI sepsis and non-AKI sepsis. Urinary miR-26b levels were closely correlated with clinical parameters reflecting the severity of the disease. Kaplan-Meier analysis revealed that sepsis patients with high urinary miR-26b levels had an elevated mortality rate. CONCLUSIONS: Taken together, these findings suggested that urinary miR-26b might be utilized as a potential biomarker for sepsis-associated AKI.


Assuntos
Injúria Renal Aguda/diagnóstico , Biomarcadores/urina , MicroRNAs/urina , Sepse/patologia , Injúria Renal Aguda/etiologia , Adulto , Idoso , Área Sob a Curva , Proteína C-Reativa/análise , China , Estudos de Coortes , Feminino , Humanos , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Curva ROC , Sensibilidade e Especificidade , Sepse/complicações , Sepse/mortalidade
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