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1.
Nat Commun ; 15(1): 204, 2024 Jan 03.
Artigo em Inglês | MEDLINE | ID: mdl-38172118

RESUMO

Generating ion-photon entanglement is a crucial step for scalable trapped-ion quantum networks. To avoid the crosstalk on memory qubits carrying quantum information, it is common to use a different ion species for ion-photon entanglement generation such that the scattered photons are far off-resonant for the memory qubits. However, such a dual-species scheme can be subject to inefficient sympathetic cooling due to the mass mismatch of the ions. Here we demonstrate a trapped-ion quantum network node in the dual-type qubit scheme where two types of qubits are encoded in the S and F hyperfine structure levels of 171Yb+ ions. We generate ion photon entanglement for the S-qubit in a typical timescale of hundreds of milliseconds, and verify its small crosstalk on a nearby F-qubit with coherence time above seconds. Our work demonstrates an enabling function of the dual-type qubit scheme for scalable quantum networks.

2.
Artigo em Chinês | MEDLINE | ID: mdl-38212134

RESUMO

Objective: To investigate the efficacy and safety of endoscopic resection of infratemporal fossa mass and to determine the indications for surgery. Methods: A retrospective case series study was conducted, including a total of 29 patients who underwent endoscopic surgery to treat infratemporal fossa mass in the Department of Rhinology of Beijing Tongren Hospital, Capital Medical University, from April 2008 to December 2021. Ten males and 19 females were included in the study, with age of (46.5±13.7) years. Pre-and post-operative sinus CT, sinus or nasopharyngeal enhanced MRI were evaluated, respectively. The main outcome measurements were the total resection of mass and the incidence of surgery-related complications. Results: Among the 29 cases of infratemporal fossa mass, 22 were schwannomas, 3 were cysts, 2 were neurofibromas, 1 was pleomorphic adenoma and 1 was basal cell adenoma. Preoperative imaging showed well-defined lesion boundaries, and postoperative pathology confirmed the benign nature of all cases. The endoscopic transnasal approach was used in 28 patients, while the combination of the transnasal approach and the transoral approach was used in 1 patient. Complete tumor removal was achieved in all cases with a 100% resection rate. The average follow-up time was 38 months (7-168 months), and no tumor recurrence was observed. Conclusions: The Endoscopic transnasal approach is a safe and effective surgical approach for the treatment of benign tumors or masses in the infratemporal fossa.


Assuntos
Fossa Infratemporal , Neoplasias da Base do Crânio , Masculino , Feminino , Humanos , Adulto , Pessoa de Meia-Idade , Fossa Infratemporal/patologia , Estudos Retrospectivos , Neoplasias da Base do Crânio/cirurgia , Neoplasias da Base do Crânio/patologia , Recidiva Local de Neoplasia , Endoscopia/métodos
3.
Zhonghua Yi Xue Za Zhi ; 103(45): 3652-3657, 2023 Dec 05.
Artigo em Chinês | MEDLINE | ID: mdl-38018064

RESUMO

Objective: To analyze the risk factors of thrombosis in patients with JAK2V617F mutation positive myeloproliferative neoplasms (MPN). Methods: A total of 223 MPN patients with JAK2V617F mutation in the Second Hospital of Tianjin Medical University from September 2017 to May 2023 were retrospectively enrolled, including 111 males and 112 females, aged [M(Q1,Q3)] 57(21,66) years. According to the presence or absence of thromboembolism during follow-up, the patients were divided into thrombosis group (n=102) and non-thrombosis group (n=121). The clinical characteristics, laboratory characteristics, cytogenetics and other disease progression and survival of the two groups of patients were analyzed. As of March 31, 2023, the follow-up period [M (Q1, Q3)] was 6 (3, 10) years. The influencing factors of thrombosis in JAK2V617F positive MPN patients were analyzed by using the Cox risk model. Results: Among 223 JAK2V617F positive MPN patients, 144 were polycythemia vera (PV), 51 were essential thrombocythemia (ET) and 28 were primary myelofibrosis (PMF). The mutation rates of ASXL1 and BCORL1 genes in the thrombosis group were 19.6% (20/102) and 6.9% (7/102), respectively, which were higher than those in the non-thrombosis group [9.1% (11/121) and 0.8% (1/121)] (both P<0.05). The proportion of monocytes, C-reactive protein (CRP), interleukin-1ß (IL)-1ß, IL-8 and tumor necrosis factor-ß (TNF-ß) increased in the thrombosis group were higher than those in the non-thrombosis group (all P<0.05). Multivariate analysis showed that age≥60 years (HR=2.132, 95%CI: 1.376-3.303, P=0.001), history of thrombosis (HR=3.636, 95%CI: 2.121-6.202, P<0.001), ASXL1 mutation positive (HR=2.245, 95%CI: 1.093-3.231, P=0.022) and elevated TNF-ß (HR=2.009, 95%CI: 1.113-3.624, P=0.021) were risk factors for thrombosis in JAK2V617F positive MPN patients. Conclusions: In addition to age, history of thrombosis and positive ASXL1 mutation, elevated TNF-ß is also an influencing factor of thrombosis in JAK2V617F positive MPN patients. Intervention of inflammation may have a certain effect on the prevention and treatment of thrombosis.


Assuntos
Transtornos Mieloproliferativos , Policitemia Vera , Tromboembolia , Trombose , Masculino , Feminino , Humanos , Idoso , Pessoa de Meia-Idade , Estudos Retrospectivos , Linfotoxina-alfa/genética , Transtornos Mieloproliferativos/genética , Transtornos Mieloproliferativos/complicações , Policitemia Vera/complicações , Policitemia Vera/genética , Tromboembolia/complicações , Trombose/genética , Mutação , Fatores de Risco , Janus Quinase 2/genética
4.
Beijing Da Xue Xue Bao Yi Xue Ban ; 55(3): 558-562, 2023 Jun 18.
Artigo em Chinês | MEDLINE | ID: mdl-37291935

RESUMO

The patient was a 55-year-old man who was admitted to hospital with "progressive myalgia and weakness for 4 months, and exacerbated for 1 month". Four months ago, he presented with persistent shoulder girdle myalgia and elevated creatine kinase (CK) at routine physical examination, which fluctuated from 1 271 to 2 963 U/L after discontinuation of statin treatment. Progressive myalgia and weakness worsened seriously to breath-holding and profuse sweating 1 month ago. The patient was post-operative for renal cancer, had previous diabetes mellitus and coronary artery disease medical history, had a stent implanted by percutaneous coronary intervention and was on long-term medication with aspirin, atorvastatin and metoprolol. Neurological examination showed pressure pain in the scapularis and pelvic girdle muscles, and V- grade muscle strength in the proximal extremities. Strongly positive of anti-HMGCR antibody was detected. Muscle magnetic resonance imaging (MRI) T2-weighted image and short time inversion recovery sequences (STIR) showed high signals in the right vastus lateralis and semimembranosus muscles. There was a small amount of myofibrillar degeneration and necrosis, CD4 positive inflammatory cells around the vessels and among myofibrils, MHC-Ⅰ infiltration, and multifocal lamellar deposition of C5b9 in non-necrotic myofibrils of the right quadriceps muscle pathological manifestation. According to the clinical manifestation, imageological change, increased CK, blood specific anti-HMGCR antibody and biopsy pathological immune-mediated evidence, the diagnosis of anti-HMGCR immune-mediated necrotizing myopathy was unequivocal. Methylprednisolone was administrated as 48 mg daily orally, and was reduced to medication discontinuation gradually. The patient's complaint of myalgia and breathlessness completely disappeared after 2 weeks, the weakness relief with no residual clinical symptoms 2 months later. Follow-up to date, there was no myalgia or weakness with slightly increasing CK rechecked. The case was a classical anti-HMGCR-IMNM without swallowing difficulties, joint symptoms, rash, lung symptoms, gastrointestinal symptoms, heart failure and Raynaud's phenomenon. The other clinical characters of the disease included CK as mean levels >10 times of upper limit of normal, active myogenic damage in electromyography, predominant edema and steatosis of gluteus and external rotator groups in T2WI and/or STIR at advanced disease phase except axial muscles. The symptoms may occasionally improve with discontinuation of statins, but glucocorticoids are usually required, and other treatments include a variety of immunosuppressive therapies such as methotrexate, rituximab and intravenous gammaglobulin.


Assuntos
Doenças Autoimunes , Inibidores de Hidroximetilglutaril-CoA Redutases , Doenças Musculares , Miosite , Masculino , Humanos , Pessoa de Meia-Idade , Autoanticorpos , Miosite/diagnóstico , Músculo Esquelético/patologia , Inibidores de Hidroximetilglutaril-CoA Redutases/uso terapêutico , Necrose/patologia , Doenças Musculares/diagnóstico , Doenças Musculares/tratamento farmacológico
5.
Eur Ann Otorhinolaryngol Head Neck Dis ; 140(4): 191-192, 2023 08.
Artigo em Inglês | MEDLINE | ID: mdl-36775669
6.
Zhonghua Yu Fang Yi Xue Za Zhi ; 57(2): 293-300, 2023 Feb 06.
Artigo em Chinês | MEDLINE | ID: mdl-36797591

RESUMO

Wolfram syndrome is a rare genetic spectrum disorder characterized by diabetes insipidus, diabetes mellitus, optic atrophy, and deafness, accompanied by other variable clinical manifestations. At present, the prognosis of this syndrome is very poor, the specific molecular mechanism is not clear, effective treatments are lacking to delay, prevent or reverse the development of Wolfram syndrome, and many patients die prematurely due to severe neurological dysfunction. This increases the urgency of the research on the pathogenic molecular mechanism related to Wolfram syndrome and the development of new therapies. This article summarizes the research progress on the pathogenic molecular mechanism and treatment status of Wolfram syndrome, in order to provide reference for the further mechanism research, prevention and treatment of Wolfram syndrome.


Assuntos
Síndrome de Wolfram , Humanos , Síndrome de Wolfram/diagnóstico , Síndrome de Wolfram/genética , Síndrome de Wolfram/terapia , Resultado do Tratamento , Registros
7.
Zhonghua Er Ke Za Zhi ; 60(11): 1185-1190, 2022 Nov 02.
Artigo em Chinês | MEDLINE | ID: mdl-36319155

RESUMO

Objective: To compare the efficacy and safety of 2 low-dose rituximab regimens in the treatment of children with primary immune thrombocytopenia (ITP). Methods: A total of 90 ITP children admitted to the Hematology Oncology Center of Beijing Children's Hospital from January 2018 to March 2021 were enrolled in this prospective cohort study. In the single-dose group, rituximab was given with a single dose of 375 mg/m2 (maximum dose 600 mg). In the 4-dose group, rituximab was given with a dose of 100 mg weekly (if body weight of the patient ≥ 30 kg, increase dosage to 200 mg weekly) for 4 weeks. Wilcoxon Mann-Whitney test, Chi-square test and Fisher's exact test were used to analyze the difference in efficacy, safety and treatment burden between two groups. Results: Among the 90 children, 41 were male and 49 were female, and the age of medication was 6.8 (4.1,10.0) years. There were 27 cases in the single-dose group and 63 cases in the 4-dose group.There were no significant differences in overall response rate, complete response rate and partial response rate between the single-dose group and 4-dose group (41% (11/27) vs. 33% (21/63), 26% (7/27) vs. 19% (12/63), 15% (4/27) vs. 14%(9/63), χ2=0.45, 0.54, 0.00, all P>0.05). The single-dose group was earlier to get overall response than the 4-dose group (1 (1, 1) vs. 3 (2, 6) weeks, Z=-3.24, P=0.001). There were no significant differences in the sustained response rate, the overall response rate in 1 year, the complete response rate in 1 year, and the partial response rate in 1 year between the single-dose group and the 4-dose group (33% (9/27) vs. 30% (19/63), 30% (8/27) vs. 24% (15/63), 19% (5/27) vs. 14% (9/63), 11% (3/27) vs. 10% (6/63), χ2=0.09, 0.34, 0.04, 0.00, all P>0.05). There were no significant differences in the duration of overall response, recurrence rate within half a year and one year, recurrence time and rate of adverse events between the single-dose group and 4-dose group (all P>0.05). The number of hospitalizations, the duration of hospital stays and the dosage of the single-dose group were significantly lower than those of the 4-dose group (1 (1, 1) vs. 4 (4, 4) times, 5 (4, 7) vs. 8 (5, 8) d, 400 (250, 500) vs. 400 (400, 800) mg, Z=-8.67, -3.03, -4.05, all P<0.05). Conclusions: The single-dose rituximab regimen is comparable to 4-dose rituximab regimen in effectiveness and safety for treatment of children ITP, but more economical and convenient. The single-dose rituximab regimen is more suitable for the second-line treatment of children ITP.


Assuntos
Púrpura Trombocitopênica Idiopática , Criança , Feminino , Masculino , Humanos , Rituximab , Estudos Prospectivos , Peso Corporal , Hospitalização
9.
Artigo em Chinês | MEDLINE | ID: mdl-35266356

RESUMO

OBJECTIVE: To identify the differentially expressed proteins in different liver tissues in the mouse model of cystic echinococcosis (CE), so as to provide insights into the research and development of therapeutic drugs targeting CE. METHODS: Female Kunming mice at ages of 6 to 8 weeks were randomly assigned into the CE group and the control group. Mice in the CE group were intraperitoneally infected with 2 000 Echinococcus multilocularis protoscoleces, while mice in the control group were injected with the same volume of physiological saline. All mice in both groups were sacrificed after breeding for 350 d, and the lesions (the lesion group) and peri-lesion specimens (the peri-lesion group) were sampled from the liver of mice in the CE group and the normal liver specimens (the normal group) were sampled from mice in the control group for data independent acquisition (DIA) proteomics analysis, and the differentially expressed proteins were subjected to Gene Ontology (GO) term enrichment analysis and Kyoto Encyclopedia of Genes and Genomes (KEGG) pathway enrichment analysis. RESULTS: A total of 26 differentially expressed proteins were identified between the lesion group and the normal group and between the peri-lesion group and the normal group, including 8 up-regulated proteins and 18 down-regulated proteins. GO term enrichment analysis showed that these differentially expressed proteins were predominantly enriched in endoplasmic reticulum membrane (biological components), oxidoreductase activity (molecular function) and oxoacid metabolic process and monocarboxylic acid metabolic process (biological processes). KEGG pathway enrichment analysis revealed that the differentially expressed protein Acyl-CoA oxidase 1 (Acox1), which contributed to primary bile acid biosynthesis during the fatty acid oxidation, was involved in peroxisome signaling pathway, and the differentially expressed protein fatty acid binding protein 1 (Fabp1), which contributed to fatty acid transport, was involved in the peroxisome proliferator-activated receptor (PPAR) signaling pathway. CONCLUSIONS: Differentially expressed proteins are identified in the liver specimens between mouse models of CE and normal mice, and some differentially expressed proteins may serve as potential drug targets for CE.


Assuntos
Equinococose , Echinococcus multilocularis , Animais , Modelos Animais de Doenças , Equinococose/genética , Proteínas de Ligação a Ácido Graxo/metabolismo , Feminino , Fígado , Camundongos , Proteômica
10.
Artigo em Chinês | MEDLINE | ID: mdl-35266357

RESUMO

OBJECTIVE: To identify the differentially expressed proteins in different liver tissues in the mouse model of alveolar echinococcosis using high-resolution mass spectrometry with data independent acquisition (DIA), and to identify the key proteins contributing to the pathogenesis of alveolar echinococcosis. METHODS: Protoscoleces were isolated from Microtus fuscus with alveolar echinococcosis and the experimental model of alveolar echinococcosis was established in female Kunming mice aged 6 to 8 weeks by infection with Echinococcus multilocularis protoscoleces. Mice were divided into the experimental and control groups, and animals in the experimental group was injected with approximately 3 000 protoscoleces, while mice in the control group were injected with the same volume of physiological saline. Mouse liver specimens were sampled from both groups one year post-infection and subjected to pathological examinations. In addition, the lesions (the lesion group) and peri-lesion specimens (the peri-lesion group) were sampled from the liver of mice in the experimental group and the normal liver specimens (the normal group) were sampled from mice in the control group for DIA proteomics analysis, and the differentially expressed proteins were subjected to bioinformatics analysis. RESULTS: A total of 1 020 differentially expressed proteins were identified between the lesion group and the normal group, including 671 up-regulated proteins and 349 down-regulated proteins, and 495 differentially expressed proteins were identified between the peri-lesion group and the normal group, including 327 up-regulated proteins and 168 down-regulated proteins. The Kyoto Encyclopedia of Genes and Genomes (KEGG) pathway enrichment analysis revealed that these differentially expressed proteins were involved in peroxisome, peroxisome proliferator-activated receptor (PPAR) and fatty acid degradation pathways, and the peroxisome and PPAR signaling pathways were found to correlate with liver injury. Several differentially expressed proteins that may contribute to the pathogenesis of alveolar echinococcosis were identified in these two pathways, including fatty acid binding protein 1 (Fabp1), Acyl-CoA synthetase long chain family member 1 (Acsl1), Acyl-CoA oxidase 1 (Acox1), Enoyl-CoA hydratase and 3-hydroxyacyl CoA dehydrogenase (Ehhadh) and Acetyl-Coenzyme A acyltransferase 1B (Acaa1b), which were down-regulated in mice in the experimental group. CONCLUSIONS: A large number of differentially expressed proteins are identified in the liver of the mouse model of alveolar echinococcosis, and Fabp1, Acsl1, Acox1, Ehhadh and Acaa1b may contribute to the pathogenesis of alveolar echinococcosis.


Assuntos
Equinococose , Echinococcus multilocularis , Animais , Proteínas de Ligação a Ácido Graxo/metabolismo , Feminino , Fígado , Camundongos , Proteômica
11.
Zhonghua Yan Ke Za Zhi ; 58(1): 3-5, 2022 Jan 11.
Artigo em Chinês | MEDLINE | ID: mdl-34979785

RESUMO

Glaucoma is the world's leading cause of irreversible blindness. As a chronic disease, it requires lifelong follow-up and management. In the long-term follow-up of glaucoma patients, a diagnosis and treatment management system is helpful. Despite the availability of various means of informationalized record management, there remain problems and challenges, like a lack of unified file management standards, data incompatibility, and barriers to inter-hospital data sharing, which lead to the failure to establish a comprehensive and standardized glaucoma record for patients. It is necessary to form a standardized management system so as to improve the efficiency and accuracy of the diagnosis and treatment of glaucoma, better maintain the visual function of patients and reduce the burden on society and family.


Assuntos
Glaucoma , Atenção , Cegueira , Doença Crônica , Glaucoma/diagnóstico , Glaucoma/terapia , Humanos
12.
J Dairy Sci ; 104(12): 12496-12507, 2021 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-34593232

RESUMO

This study evaluated the effects of incremental tributyrin supplementation in pasteurized waste milk on growth performance, health, and blood metabolism of dairy calves before and after weaning. Forty-eight newborn female Holstein dairy calves (39.6 ± 2.75 kg; mean ± standard deviation) were blocked by age and randomly assigned to 3 treatments: pasteurized waste milk (1) without supplementation, (2) with 1 g/L of tributyrin products (unprotected solid powder; containing 35% tributyrin), or (3) with 2 g/L of tributyrin products. The calves were weaned on d 56 and were raised until d 77. Data were analyzed for the preweaning, postweaning, and overall periods. The results showed that starter intake and hay intake were not different among treatments in any period of the trial, but the crude protein intake tended to increase linearly with tributyrin supplementation during the overall period. Although tributyrin supplementation had no effects on body weight during preweaning and overall periods, body weight increased linearly with tributyrin supplementation postweaning. The average daily gain tended to increase linearly during postweaning and overall periods. No effects were observed on feed efficiency in any period. A positive linear relationship between body length and tributyrin supplementation was observed during the postweaning period, but no differences were found for the other body structural measurements in any period. The results of diarrhea showed that tributyrin concentration had a negative linear relationship with diarrhea frequency during preweaning and overall periods. The rectal temperature did not differ among treatments in any period, but a treatment × week effect for rectal body temperature was observed. For blood metabolism, tributyrin supplementation had no effects on insulin, growth hormone, total protein, albumin, or globulin. No differences were found in serum amyloid A concentration in any of the periods, yet haptoglobin concentration decreased linearly with increasing tributyrin concentration during postweaning and overall periods. Endothelin concentration showed a tendency to decrease linearly during preweaning and postweaning periods and decreased linearly with tributyrin supplementation during the overall period. An increasing tributyrin concentration was associated with a negative linear relationship with IL-1ß concentration during the preweaning period, and no differences were found in the other periods. The concentration of IL-6 and tumor necrosis factor α were not different among treatments in any of the periods. These data suggest that increasing the concentration of tributyrin in pasteurized waste milk could increase growth performance and health of dairy calves, and incremental tributyrin supplementation could linearly reduce haptoglobin, endothelin, and IL-1ß concentrations, indicating a positive effect of tributyrin on alleviating oxidative stress and inflammatory status of dairy calves. Calves fed pasteurized waste milk supplemented with tributyrin products (containing 35% tributyrin) at 2 g/L compared with 1 g/L of milk had more improved growth and health.


Assuntos
Ração Animal , Leite , Ração Animal/análise , Animais , Peso Corporal , Bovinos , Dieta , Suplementos Nutricionais , Feminino , Triglicerídeos , Desmame
13.
Zhonghua Er Ke Za Zhi ; 59(11): 968-972, 2021 Nov 02.
Artigo em Chinês | MEDLINE | ID: mdl-34711033

RESUMO

Objective: To summarize and analyze of the clinical and genetic characteristics of children with nonmuscle myosin heavy chain 9 (MYH9)-related disease (MYH9-RD). Methods: To screen the patients who were first diagnosed as "chronic/refractory immune thrombocytopenia (ITP) " from April 2016 to May 2019 in Beijing Children's Hospital by genetic and clinical examinations, then the clinical manifestation, laboratory examination and genetics results of 7 children diagnosed with MYH9-RD were collected and summarized retrospectively. Results: Among 7 children diagnosed with MYH9-RD, 3 were males and 4 females. The age of onset was 1.25 (0.41-6.16) years. The course of disease was 2.16 (0.41-8.59) years. The automatic platelet count was (9 (5-30))×109/L. All the cases were found with giant platelets under microscope,and the manual platelet count was (70 (30-100))×109/L. Four cases had skin hemorrhage or epistaxis and 3 cases had no bleeding. All 7 patients had received first-or second-line therapy of ITP, of whom 1 case received splenic embolization, and all the treatments mentioned above were ineffective. Finally, it was confirmed that all 7 patients had heterozygous missense mutations of MYH9 gene by next generation sequencing (NGS), including 2 pedigrees and 5 sporadic cases. Four sporadic mutations occurred in N-terminal globular head domain (HD), and 1 sporadic case with p.D1424N mutations occurred in the C-terminal tail domain (TD). One of the pedigrees also had p.D1424N mutation. The other familial case had a novel variant with one missense variant p.A44D caused by the c.131C>A transition. One of the two p.R702 mutations had kidney damage, and several relatives of the new p.A44D mutations had deafness. Conclusions: In this study, the spontaneous mutations of seven MYH9-RD were common, and all patients were misdiagnosed as ITP, whereas the bleeding was mild and immunotherapy was ineffective. The suspected disease can be identified earlier by manual visual platelet volume and count, which can be confirmed by genetic testing. It is more important to monitor the development of other organs damage instead of thrombocytopenia. For cases with p.R702 mutations the doctor should be aware of kidney damage, and for the cases with novel mutations p.A44D the doctor should be aware of hearing loss.


Assuntos
Perda Auditiva Neurossensorial , Cadeias Pesadas de Miosina , Criança , Pré-Escolar , Feminino , Perda Auditiva Neurossensorial/diagnóstico , Perda Auditiva Neurossensorial/genética , Humanos , Lactente , Masculino , Proteínas Motores Moleculares/genética , Mutação , Cadeias Pesadas de Miosina/genética , Estudos Retrospectivos , Trombocitopenia/congênito
14.
Zhonghua Wai Ke Za Zhi ; 59(8): 711-715, 2021 Aug 01.
Artigo em Chinês | MEDLINE | ID: mdl-34192865

RESUMO

Currently, thoracic endovascular aortic repair (TEVAR) is the first-line treatment for patients with complicated Stanford type B aortic dissections. However, TEVAR does not occlude the distal entry tear of dissections, and blood flow persists in the false lumen. Dissections might progress in some patients. Studies showed that distal entry tear increased the possibility of late aortic events during follow-up. Thus, treatment of distal entry tear is necessary in some high-risk patients after TEVAR. In this article, the current treatment strategies of distal entry tear are summarized, which include PETTICOAT, STABILISE, covered stent, fenestrated and branched stent-grafts, false lumen embolization, vascular occluder, and Knickerbocker. However, the number of the cases of most approaches is so limited that the indications and effectiveness need to be further studied. Selecting the right treatment for the right patient is of great importance.

15.
Zhonghua Er Ke Za Zhi ; 59(3): 212-217, 2021 Mar 02.
Artigo em Chinês | MEDLINE | ID: mdl-33657696

RESUMO

Objective: To investigate the clinical characteristics, treatment and prognosis of children with acquired thrombotic thrombocytopenic purpura (TTP). Methods: The clinical manifestations, laboratory examination, treatment and prognosis of 5 children with acquired TTP hospitalized in Beijing Children's Hospital, Capital Medical University from January 2016 to July 2019 were analyzed retrospectively. Results: There were 5 children with acquired TTP including 2 males and 3 females, with the onset age of 8.9(0.8-14.5) years, while 11 children with TTP in the same period. Thrombocytopenia and microangiopathic hemolytic anemia were found in all 5 patients. Only one patient had typical pentalogy of TTP, 3 patients had nervous system symptoms and 3 patients had fever, while renal impairment was relatively rare (1 case). Laboratory examination showed severe thrombocytopenia (7(4-14) ×109/L) and low level of hemoglobin (70(58-100)g/L) in all 5 children. Blood biochemical examination showed that total bilirubin (mainly indirect bilirubin) increased in 3 patients, lactate dehydrogenase increased in 5 patients, and urea nitrogen increased in 1 patient. Bone marrow smear showed megakaryocyte did not decrease. Plasma ADAMTS13 activity was 0 in all 5 patients while ADAMTS13 inhibitor was positive in 4 patients and negative in 1 patient. All 5 children received glucocorticoid therapy, rituximab was added in the early stage of the disease, and 3 children received plasma exchange. The time of platelet recovery to normal was 19 (9-29) days. One child had TTP recurrence after 9 months of treatment. The condition was stable after being treated with glucocorticoid and rituximab again. This case was finally diagnosed as systemic lupus erythematosus after more than 3 years followed up. By December 1, 2020, the follow-up time was 24(16-57) months.The clinical symptoms of all patients disappeared and the platelet level was stable at 159(125-269) ×109/L. Conclusions: Childhood acquired TTP is relatively rare, which can occur in all age groups. The clinical manifestations are mainly thrombocytopenia and microangiopathic hemolytic anemia, the plasma ADAMTS 13 activity and inhibitor test are helpful for the diagnosis of acquired TTP. Plasma exchange and rituximab are effective treatment. This disease requires long-term follow-up.


Assuntos
Anemia Hemolítica , Púrpura Trombocitopênica Trombótica , Adolescente , Criança , Feminino , Humanos , Masculino , Troca Plasmática , Púrpura Trombocitopênica Trombótica/diagnóstico , Púrpura Trombocitopênica Trombótica/terapia , Estudos Retrospectivos , Rituximab/uso terapêutico
17.
Zhonghua Zhong Liu Za Zhi ; 43(1): 108-112, 2021 Jan 23.
Artigo em Chinês | MEDLINE | ID: mdl-33472322

RESUMO

Objective: To estimate the incidence and mortality of corpus uteri cancer in China, 2015. Methods: Quality audit and evaluation of the data from 2015 cancer registration reported by 501 cancer registries were conducted, and 368 cancer registries were included in the analysis. The incidence rate and mortality rate of corpus uteri cancer were calculated according to the factors of the region (urban, rural, east, central, western), sex and age groups. The incidence and mortality of corpus uteri cancer with the 2015 population were estimated. Chinese standard population in 2000 and world Segi's population were used for the calculation of age-standardized rates (ASR) of incidence and mortality. Results: In 2015, 368 cancer registries included in the analysis covered a total of 309 553 499 populations in China, accounting for 22.52% of the national population. It is estimated that there were about 68 900 new cases of corpus uteri cancer in 2015, the incidence rate was 10.28/10(5), age-standardized incidence rates by Chinese standard population (ASR China) and world standard population (ASR world) were 6.86/10(5) and 6.66/10(5), respectively. The incidence rate of urban area (11.35/10(5)) was higher than that of rural area (8.90/10(5)), and the incidence of eastern region (12.12/10(5)) was higher than the central region (9.94/10(5)) and the western region (8.25/10(5)). It is estimated that in 2015, there were about 16 000 deaths of corpus uteri cancer, the mortality rate was 2.39/10(5), ASR China was 1.49/10(5), ASR world was 1.47/10(5). The mortality in urban areas (2.40/10(5)) is close to rural areas (2.39/10(5)); the mortality in central areas (2.55/10(5)) was higher than the eastern areas (2.32/10(5)) and the western areas (2.31/10(5)). Conclusions: In China, the incidence of corpus uteri cancer is on the rise and has a trend of youth, the burden of disease is gradually increasing, which threatens the health of women. Targeted prevention and control measures should be carried out in the different regions.


Assuntos
Neoplasias , População Rural , Adolescente , China/epidemiologia , Feminino , Humanos , Incidência , Sistema de Registros , População Urbana , Útero
20.
Zhonghua Liu Xing Bing Xue Za Zhi ; 41(5): 642-647, 2020 May 10.
Artigo em Chinês | MEDLINE | ID: mdl-32164401

RESUMO

The outbreak of COVID-19 has spread quickly across 114 countries/territories/areas in six continents worldwide and has been announced as a pandemic by WHO. This study analyzed global COVID-19 epidemiological trends, examined impact of the pandemic on global health security, diplomacy, and social environment in China, and provided short- and long-term strategic policy recommendations for China's subsequent preparedness and responses.


Assuntos
Infecções por Coronavirus/epidemiologia , Infecções por Coronavirus/prevenção & controle , Saúde Global/tendências , Pandemias/prevenção & controle , Pneumonia Viral/epidemiologia , Pneumonia Viral/prevenção & controle , COVID-19 , China/epidemiologia , Humanos , Políticas
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