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1.
Vascular ; 30(4): 779-786, 2022 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-34144653

RESUMEN

OBJECTIVES: To evaluate the safety and feasibility of microwave ablation for treating venous malformations (VMs) with severe localized intravascular coagulopathy (LIC). PATIENTS AND METHODS: Data for patients with the diagnosis of VMs coupled with severe LIC who underwent color Doppler-guided microwave dynamic ablation between January 2017 and June 2019 were retrospectively reviewed and analyzed. All patients had previously received sclerotherapy or other treatments with poor outcomes and gradual aggravation of coagulation abnormalities. Microwave treatment with "dynamic ablation" was performed with real-time color Doppler monitoring and was repeated if necessary after 3 months. Low-molecular-weight heparin (LMWH) was used to control consumptive coagulopathy. The therapeutic efficacy including coagulation function and lesion size was evaluated using the four-level scale developed by Achauer. RESULTS: Among 15 patients with extensive diffuse or multiple VMs, 10 patients presented with lesions in a single lower extremity, one in both lower extremities and the perineum, one in both upper extremities and the trunk, and three with multiple lesions. The patients underwent a total of 74 microwave ablation sessions, with an average of 4.9 sessions per person. Coagulation abnormalities were temporarily aggravated in 59 sessions within the first seven days post-ablation but improved to grade II (fair) a week later. From six months to three years after the ablation, the lesions improved to grade IV (excellent) in one patient, grade III (good) in six patients, and grade II (fair) in eight patients. Moreover, the coagulation function improved to grade IV in four patients, grade III in eight patients, and grade II in three patients, resulting in an efficiency rate of 80% (12/15). Post-ablation complications included fever, hemoglobinuria, and elevations in aspartate aminotransferase, lactate dehydrogenase, and alanine aminotransferase. The patients with fever and hemoglobinuria recovered after specific therapeutic measures, but elevations in aspartate aminotransferase, lactate dehydrogenase, and alanine aminotransferase recovered spontaneously without further interventions. CONCLUSIONS: Ablation coupled with anticoagulation can effectively treat VMs in patients with severe LIC and improve the long-term coagulation function.


Asunto(s)
Trastornos de la Coagulación Sanguínea , Microondas , Malformaciones Vasculares , Alanina Transaminasa/uso terapéutico , Aspartato Aminotransferasas/uso terapéutico , Trastornos de la Coagulación Sanguínea/complicaciones , Hemoglobinuria/complicaciones , Hemoglobinuria/tratamiento farmacológico , Heparina de Bajo-Peso-Molecular , Humanos , Lactato Deshidrogenasas , Microondas/uso terapéutico , Estudios Retrospectivos , Resultado del Tratamiento , Malformaciones Vasculares/complicaciones , Malformaciones Vasculares/diagnóstico por imagen , Malformaciones Vasculares/cirugía
2.
Int J Med Sci ; 18(9): 2008-2016, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-33850471

RESUMEN

Plexiform neurofibroma (pNF) in the head and neck is a characteristic feature in patients with neurofibromatosis type 1 (NF1) and is associated with significant disfigurement and psychological distress. Yes-associated protein (YAP), the key molecule involved in the Hippo pathway, is a vital transductor that regulates the proliferation and remyelinating of Schwann cells. The functional status of YAP and its feasibility as a potential target are still unknown in pNF. A total of 17 pNF tumor tissue specimens from the head and neck were collected at the Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine. Histologically, diagnosis of the Schwann cell region in pNF was achieved with hematoxylin-eosin staining, positive reactions for S100, SOX10, ERK and p-ERK, and low identification of Ki67 and SMA. Compared with normal nerve tissue, obviously increased nuclear YAP was detected in the Schwann cell region of pNF, with a mean nuclear staining rate of 67.11%. Based on the shNF1 Schwann cell model (the RSC96 cell line), with upregulated expression of RAS, ERK and p-ERK, p-YAP (Ser127) and p-YAP (Ser397) were significantly decreased and total YAP and nuclear YAP were increased. According to a confocal assay, the interference of shNF1 substantially promoted YAP nuclear translocation. Compared with control Schwann cells, the YAP inhibitor CA3 might have a more sensitive effect (IC50: NC=0.96±0.04, shNF1=0.71±0.02, P<0.05) on the shNF1 Schwann cell model than the classic MEK1/2 inhibitor selumetinib (IC50: NC=14.36±0.95, shNF1=24.83±0.98, P>0.05). For in vivo inhibition, the CA3 group and the selumetinib group displayed a similar inhibition effect with no significant difference. Increased nuclear translation and the functional state of YAP implies that the YAP-Hippo pathway might play an important role in the formation and remyelination of pNF. Compared with selumetinib, the YAP inhibitor can exhibit a similar but more sensitive effect on NF1-/- Schwann cells. These observations imply that YAP as a novel or adjuvant therapy target in the treatment of pNF.


Asunto(s)
Proteínas Adaptadoras Transductoras de Señales/metabolismo , Antineoplásicos/farmacología , Neurofibroma Plexiforme/genética , Neurofibromatosis 1/complicaciones , Células de Schwann/patología , Factores de Transcripción/metabolismo , Proteínas Adaptadoras Transductoras de Señales/antagonistas & inhibidores , Adolescente , Adulto , Animales , Antineoplásicos/uso terapéutico , Línea Celular Tumoral , Núcleo Celular/metabolismo , Proliferación Celular/efectos de los fármacos , Niño , Preescolar , Femenino , Regulación Neoplásica de la Expresión Génica/efectos de los fármacos , Humanos , Masculino , Ratones , Terapia Molecular Dirigida/métodos , Neurofibroma Plexiforme/tratamiento farmacológico , Neurofibroma Plexiforme/patología , Neurofibromatosis 1/tratamiento farmacológico , Neurofibromatosis 1/genética , Factores de Transcripción/antagonistas & inhibidores , Ensayos Antitumor por Modelo de Xenoinjerto , Proteínas Señalizadoras YAP , Adulto Joven
3.
Acta Pharmacol Sin ; 41(4): 572-580, 2020 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-31705124

RESUMEN

Stem cell therapy represents the potential alternative effective strategy for some diseases that lack effective treatment currently. Correspondingly, it is crucial to establish high-sensitive and reliable quantification assay for tracing exogenous cell migration. In the present study, we first used both bioluminescence imaging (BLI) indirect labeling (human norepinephrine transporter-luciferase reporter system) and 89zirconium (89Zr)-hNSCs direct labeling combined with positron emission tomography/computer tomography (PET/CT) system for tracking human neural stem cells (hNSCs) migration into the brain via nasal administration in preclinical study. But the above two methods failed to give the biodistribution profile due to their low sensitivity. Considering its superior sensitivity and absolute quantitation capability, we developed and validated the droplet digital PCR (ddPCR) targeting species-specific gene in frozen and paraffin sections, slices, and whole blood with the sensitivity of 100-200 hNSCs. Accurate and high throughput quantification could be performed using ddPCR with the coefficient of variation (CVs) of lower quality control (LQC) below 30%. In combination with immunohistochemistry and ddPCR, we confirmed the migration of hNSCs into the brain via nasal administration, which supported the efficacy of hNSCs in MPTP-treated mice, an animal model of Parkinson's disease. In conclusion, the present study is the first to report the application of ddPCR in the pharmacokinetics profile description of tracking of hNSCs in preclinical studies.


Asunto(s)
Células-Madre Neurales/citología , Enfermedad de Parkinson/terapia , Trasplante de Células Madre , 1-Metil-4-fenil-1,2,3,6-Tetrahidropiridina , Administración Intranasal , Animales , Modelos Animales de Enfermedad , Humanos , Ratones , Ratones Endogámicos C57BL , Imagen Óptica , Enfermedad de Parkinson/genética , Reacción en Cadena de la Polimerasa , Tomografía Computarizada por Tomografía de Emisión de Positrones , Distribución Tisular , Circonio/química
4.
Int J Med Sci ; 15(4): 385-394, 2018.
Artículo en Inglés | MEDLINE | ID: mdl-29511374

RESUMEN

A TIE2 mutation causing arginine-to-tryptophan substitution at residue 849 (TIE2-R849W) is commonly identified in heredofamilial venous malformation. However, there is no in vivo model to confirm the pathogenic role of TIE2-R849W. Humanized TIE2-R849W plasmid was constructed via PCR-mediated site-directed mutagenesis. After transcription and micro-injection, TIE2-R849W significantly induces multiple malformations in zebrafish: caudal vein plexus (CVP) defect, eye abnormalities, forebrain formation perturbations, and mandibular malformation. Histologically, these phenotypes accompany aphakia, confused retina plexiform layer, abnormal mandibular cartilage, ectopic myelencephalon proliferation and aberrant location of neurogliocytes. According to qRT-PCR, except for high expression of egfl7, the other CVP-related genes cd146, nr2f1a, and s1pr1 are not significantly different from control. TIE2-R849W also induced upregulation of the wnt signaling pathway. Gene array in vitro shows that under the effect of TIE2-R849W, consistent with high expression of pik3 and foxo1, high levels of egfl7, wnt9a, lrp5 and dkk1 were partly confirmed. This in vivo model directly identifies the venous-related pathogenic role of TIE2-R849W. Under up-regulation of TIE2-R849W, egfl7 could be considered a potential reason for venous defects. Moreover, the wnt pathway may perform an important role as a key trigger for head multi-malformations.


Asunto(s)
Malformaciones Vasculares/genética , Venas/patología , Proteínas de Pez Cebra/genética , Animales , Animales Modificados Genéticamente , Modelos Animales de Enfermedad , Regulación de la Expresión Génica/genética , Humanos , Mutación , Fosforilación , Receptor TIE-2/genética , Malformaciones Vasculares/patología , Venas/metabolismo , Pez Cebra/genética
5.
Int J Cancer ; 139(7): 1658-66, 2016 10 01.
Artículo en Inglés | MEDLINE | ID: mdl-27252149

RESUMEN

This meta-analysis was to evaluate the efficacy of current treatment modalities for kaposiform hemangioendothelioma and tufted angioma. A systematic review was performed using PubMed (Medline), Web of Science and Embase for clinical studies. The outcome was measured by pooled response rate with 95% confidence intervals (CIs), together with heterogeneity, subgroup analysis, sensitivity analysis and publication bias. Fifteen studies with 244 participants were included in this analysis. Vincristine therapy exhibited a relatively higher response rate (0.72; 95%CI, 0.64-0.79) compared with other therapies including systemic corticosteroid (0.27; 95%CI, 0.17-0.36), interferon (0.36; 95%CI, 0.24-0.48), radiotherapy (0.49; 95%CI, 0.26-0.73), embolization (0.66; 95%CI, 0.48-0.83), aspirin/ticlopidine (0.42; 95%CI, 0.06-0.78) and sirolimus (0.57; 95%CI, 0.00-0.10), in treating KHE/TA. Subgroup analysis indicated that the efficacy of systemic corticosteroids therapy was age-related. The pooled response rate was 0.15 (95%CI, 0.08-0.23) for participants 3.5 months of age and older compared with 0.35 (95% CI, 0.26-0.44) for participants less than 3.5 months. Regarding side effects, systemic corticosteroids treatment was 0.32 (95%CI, 0.15-0.50), vincristine modality was 0.16 (95%CI, 0.08-0.24) and interferon therapy was 0.28 (95%CI, 0.13-0.43). In conclusion, as one of the first reviews evaluating the effect of common therapies in the treatment of KHE/TA, our meta-analysis displayed that vincristine was more effective. Thus, vincristine was the most effective, providing evidence supporting the use of vincristine as a first-line therapy for KHE/TA.


Asunto(s)
Hemangioendotelioma/terapia , Hemangioma/terapia , Síndrome de Kasabach-Merritt/terapia , Sarcoma de Kaposi/terapia , Neoplasias Cutáneas/terapia , Ensayos Clínicos como Asunto , Hemangioendotelioma/tratamiento farmacológico , Hemangioma/tratamiento farmacológico , Humanos , Síndrome de Kasabach-Merritt/tratamiento farmacológico , Ensayos Clínicos Controlados Aleatorios como Asunto , Sarcoma de Kaposi/tratamiento farmacológico , Neoplasias Cutáneas/tratamiento farmacológico , Vincristina/uso terapéutico
6.
Nanomedicine ; 11(5): 1109-15, 2015 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-25791814

RESUMEN

Topical propranolol has been used for the therapy of superficial infantile hemangiomas (IH). A retrospective investigation was conducted in 50 patients to evaluate the clinical effect of a new type of topical nano-propranolol-dispersed hydrogel. Participants were treated 3 times per day for 2 weeks to 11 months. 68% of patients were female and 12% had received other treatments before therapy. The nano-propranolol 0.5% hydrogel was initiated at a mean age of 5.010 months and for a mean duration of 3.610 months. The response rate was 86%. No recurrence and rebound growth occurred after withdrawal of hydrogel. Slight side effects (application site itching, erosion and crusting) were observed in only 2 cases. All the local irritations were evaluated as mild and were tolerated without discontinuing the medication. We suggest that topical nano-propranolol hydrogel could be an alternative option for the treatment of uncomplicated superficial IH with satisfactory tolerability and optimal effectiveness. FROM THE CLINICAL EDITOR: The current recommended treatment for infantile hemangiomas is oral propranolol. Nonetheless, a small proportion of patients will have systemic side effects. In this article, the authors developed topical nano-propranolol hydrogel and tested this on clinical patients and found favorable response.


Asunto(s)
Antagonistas Adrenérgicos beta/uso terapéutico , Hemangioma/tratamiento farmacológico , Propranolol/uso terapéutico , Neoplasias Cutáneas/tratamiento farmacológico , Piel/efectos de los fármacos , Administración Tópica , Antagonistas Adrenérgicos beta/administración & dosificación , Antagonistas Adrenérgicos beta/efectos adversos , Femenino , Hemangioma/patología , Humanos , Hidrogeles/química , Lactante , Masculino , Nanoestructuras/química , Vehículos Farmacéuticos/química , Propranolol/administración & dosificación , Propranolol/efectos adversos , Estudios Retrospectivos , Piel/patología , Neoplasias Cutáneas/patología
7.
J Oral Maxillofac Surg ; 72(11): 2126-33, 2014 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-25201232

RESUMEN

PURPOSE: Piezosurgery has been used widely in oral and maxillofacial surgery, but there has been no report systematically describing an osteotomy method with piezosurgery for complicated mandibular third molar removal. The aim of this study was to introduce 4 osteotomy methods using piezosurgery and evaluate their effects. MATERIALS AND METHODS: A retrospective study was conducted of patients with a complicated impacted mandibular third molar requiring extraction. The predictor variable was the extraction technique. Four osteotomy methods using piezosurgery were tested according to different impaction types: method 1 involved complete bone removal; method 2 involved segmental bone removal; method 3 involved bone removal combined with tooth splitting; and method 4 involved block bone removal. Outcome variables were success rate, operative time, major complications (including nerve injury, mandible fracture, severe hematoma, or severe edema), and serious pyogenic infection. Data were analyzed using descriptive statistics. RESULTS: The study was composed of 55 patients with 74 complicated impacted mandibular third molars. All impacted mandibular third molars were removed successfully. The average surgical time was 15 minutes (range, 8 to 26 minutes). Thirty-eight molars (51.4%) were extracted by method 1, 18 molars (24.3%) by method 2, 12 molars (16.2%) by method 3, and 6 molars (8.1%) by method 4. Two cases (2.7%) developed postoperative infections and recovered within 1 week using drainage and antibiotic administration. CONCLUSION: The 4 osteotomy methods with piezosurgery provide effective ways of removing complicated impacted mandibular third molars.


Asunto(s)
Mandíbula/cirugía , Tercer Molar/cirugía , Osteotomía/métodos , Humanos , Estudios Retrospectivos
8.
J Craniofac Surg ; 23(1): e33-6, 2012 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-22337457

RESUMEN

Hemangiopericytoma (HPC) is an uncommon vascular tumor arising from pericytic cells with variable malignant potential. Primary HPCs of the bone are extremely rare; however, involvement of the condylar process has not been reported. We presented a 21-year-old female patient with low-grade malignant HPC in the right mandibular condyle. Clinical examination and imaging findings revealed a well-defined soft mass, encapsulating the mandibular condyle from behind. The lesion and mandibular condyle were removed simultaneously; then, the costochondral graft was used to reconstruct the defect. Histopathologic examinations of the specimen confirmed the diagnosis. After 2 years of follow-up, the patient is free of disease, and mouth opening returned to normal.


Asunto(s)
Hemangiopericitoma/diagnóstico , Cóndilo Mandibular/patología , Neoplasias Mandibulares/diagnóstico , Antígeno 12E7 , Antígenos CD/análisis , Moléculas de Adhesión Celular/análisis , Diagnóstico Diferencial , Femenino , Estudios de Seguimiento , Humanos , Imagen por Resonancia Magnética , Osteotomía/métodos , Rango del Movimiento Articular/fisiología , Tomografía Computarizada por Rayos X , Vimentina/análisis , Adulto Joven
9.
Ann Transl Med ; 10(11): 621, 2022 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-35813344

RESUMEN

Background: Infantile hemangioma (IH) is the most common benign vascular tumor of infancy and is proposed to arise from hemangioma stem cells (HemSCs). Therapies for IH include oral beta-blockers, surgery, and the delivery of novel therapeutic agents, such as bioactive microRNAs (miRNAs). However, in the extracellular environment, miRNA is easily hydrolyzed by RNase. miR-187-3p has previously been confirmed to promote or inhibit various malignancies, but its role in the development and progression of IH remains unclear. Methods: In this study, engineered exosomes (E-exos) were exploited to deliver miR-187-3p into HemSCs. The E-exos were generated by introducing miR-187-3p mimics into human adipose mesenchymal stem cell-derived exosomes (hAMSC-exos) via electroporation. The expression and secretion of miR-187-3p were examined by quantitative reverse transcription-polymerase chain reaction (qRT-PCR). Western blot analysis, transmission electron microscopy (TEM), and dynamic light scattering (DLS) were used to characterize the exosomes. The effects of the E-exos on HemSC viability were examined using the tube formation assay and the 3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyl-2H-tetrazolium bromide assay. Western blot analysis was used to evaluate the effects of E-exos on Notch-1, Notch-4, and Jagged-1 expression in HemSCs. Results: E-exos did not differ significantly from hAMSC-exos in terms of morphology, particle size, or surface markers. E-exos could be internalized by HemSCs, and the course of cellular uptake of E-exos was time dependent. After 12 hours of treatment, E-exos significant inhibited tube formation. Notch signaling was also inhibited by miR-187-3p loading by E-exos. E-exos showed excellent inhibitory effects against HemSC proliferation via Notch signaling. Conclusions: This study provides a foundation for using hAMSC-exos to optimize current clinical options to facilitate IH treatment and deliver therapeutic agents in the future.

10.
Childs Nerv Syst ; 27(4): 603-7, 2011 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-20821215

RESUMEN

OBJECTIVE: The aim of this study was to identify RASA1 mutation in Chinese population with sporadic Sturge-Weber syndrome (SWS). METHODS: Genomic DNA was obtained from peripheral blood of nine patients with sporadic SWS. The 25 exons, promoter regions (-1,000 bp) as well as intron-exon boundaries of RASA1 were amplified by polymerase chain reaction, and products were sequenced directly. RESULTS: A novel synonymous mutation (c.1229 G > A [p.K420K]) of RASA1 was identified in the present series. CONCLUSION: It implied that RASA1 may be not a virulence gene, but further study is needed to know RASA1 gene mutation in SWS patients.


Asunto(s)
Mutación , Síndrome de Sturge-Weber/genética , Proteína Activadora de GTPasa p120/genética , Adolescente , Adulto , Pueblo Asiatico/genética , Secuencia de Bases , Niño , Análisis Mutacional de ADN , Femenino , Humanos , Lactante , Masculino , Persona de Mediana Edad , Datos de Secuencia Molecular , Reacción en Cadena de la Polimerasa , Adulto Joven
12.
Ann Transl Med ; 9(5): 394, 2021 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-33842615

RESUMEN

BACKGROUND: Though infantile hemangioma (IH) is a common benign vascular tumor, its pathogenesis remains unclear. This study explored the function of hemangioma-derived stem cells (HemSCs) derived exosomes, which exerted an intercellular effect on hemangioma-derived endothelial cells (HemECs). METHODS: First, HemSCs and HemECs were extracted and cultured. HemSCs derived exosomes (HemSCs-exos) were harvested. miRNA sequencing and target prediction were used to explore differentially expressed miRNAs and potential binding targets. After HemECs were co-cultured with HemSCs-exos, a series of in vitro assays were then performed including cell counting kit-8 (CCK-8) assay, cell apoptosis assay, cell cycle assay and tube formation assay to evaluate proliferation, angiogenesis abilities, etc. qRT-PCR and Western blot were conducted to detect the expression level of target genes and proteins. RESULTS: After co-culturing with HemSCs-exos, proliferation, and angiogenesis abilities of HemECs were enhanced, while apoptosis and cell cycle arrest rate were decreased. MiR-196b-5p was observed to be significantly highly expressed in HemSCs-exos. CDKN1B was identified as the binding target of miR-196b-5p. HemECs' proliferation and angiogenesis abilities were elevated when co-cultured with exosomes from HemSCs transfected with miR-196b-5p mimic. In addition, apoptosis rate declined, and lower cells were arrested in G0/G1 phases. Cyclin E, bcl-2 were significantly highly expressed, whereas p27, Bax expression were significantly down-regulated. The positive effect of miR-196b-5p in HemSCs-exos was dramatically reversed when HemECs were transfected with oe-CDKN1B. CONCLUSIONS: The current study found a novel intercellular interaction between IH cells. Briefly, exosome-derived miRNA-196b-5p in HemSCs could facilitate proliferation and angiogenesis abilities, and attenuate apoptosis and cell cycle repression rate of HemECs by directly binding with CDKN1B.

13.
Ann Transl Med ; 9(2): 116, 2021 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-33569418

RESUMEN

BACKGROUND: Infantile hemangiomas (IHs) are the most frequently occurring pediatric lesions. Oral propranolol has been shown to be safe and effective in infants with IHs. Side effects such as sleep disturbances have been associated with propranolol. Atenolol is a hydrophilic, selective ß1-blocker and therefore may be not associated with side effects attributable to ß2-adrenergic receptor blockade and lipophilicity. However, the efficacy of atenolol in the treatment of IHs is poorly understood. The aim of this study was to evaluate the efficacy of atenolol in the treatment of proliferating IHs in a clinical cohort including 133 consecutive patients. METHODS: In this study, we enrolled 133 patients diagnosed as proliferating IHs from the routine clinical and referral practices of the authors. The procedures followed were in accordance with the ethical standards of the Institute Review Board of Shanghai Ninth People's Hospital and Helsinki Declaration. Clinical characteristics, including demographic data and clinical morphology, were collated. Responses to oral atenolol therapy were graded as: excellent, good, fair and poor. According to the reaction to atenolol treatment, additional medications or therapy were used for IH patients to achieve satisfactory clinical results. RESULTS: In this study, 128 (96.2%) of 133 IH patients responded to oral atenolol, and the response rate (RR) was significantly different for different ages of patients (P<0.05), with the youngest patients having the highest RR. The mean time of treatment was 4.9 months. Forty-one patients who exhibited residual hyperpigmentation or telangiectasia were further treated with timolol maleate cream (n=32) or pulsed dye laser (n=9). All the 41 patients showed positive response. No life-threatening complications were noted during and after oral atenolol. Only 4 (3.0%) of 133 patients developed minor complications including diarrhea. No agitation and bronchospasm were noted in our study. CONCLUSIONS: This study demonstrated that atenolol was effective in the treatment of IHs. Compared to propranolol, atenolol seems to have a similar effect on IHs. Furthermore, atenolol seems to be less frequently associated with potentially life-threatening side effects.

14.
Medicine (Baltimore) ; 100(1): e24146, 2021 Jan 08.
Artículo en Inglés | MEDLINE | ID: mdl-33429792

RESUMEN

ABSTRACT: Since 2008, oral propranolol has evolved as the first-line therapy for infantile hemangiomas (IHs). Meanwhile, oral atenolol gradually shows comparative effectiveness versus oral propranolol with few side effects. Here, we conducted a mobile internal survey among a group of Chinese clinicians about how they choose the dosage, dose regimen, and dose escalation methods of propranolol and atenolol for the treatment of IH.A mobile-ready internal survey on the application of oral propranolol and oral atenolol for IH in mainland China was performed and distributed to 333 potential clinicians from different levels of healthcare institutions in mainland China. Eighty-one doctors responded to the survey. All the respondents had the experience of treating IH with oral propranolol and 32 had the experience with oral atenolol.Most of the doctors from tertiary hospitals chose 2 mg/kg/d twice daily, while most of those with the experience of propranolol from private hospitals chose 1 mg/kg/d once daily. More doctors from tertiary hospitals had the experience of atenolol than those from private hospitals.Oral atenolol has become another medication intervention option for IH in mainland China. This survey is helpful to standardize and develop a guideline of oral atenolol therapy for IH.


Asunto(s)
Atenolol/farmacología , Hemangioma/tratamiento farmacológico , Propranolol/farmacología , Administración Oral , Antihipertensivos/farmacología , Antihipertensivos/uso terapéutico , Atenolol/uso terapéutico , China , Femenino , Hemangioma/complicaciones , Humanos , Lactante , Masculino , Propranolol/uso terapéutico , Encuestas y Cuestionarios , Resultado del Tratamiento
15.
J Vasc Surg Venous Lymphat Disord ; 9(4): 1007-1016.e7, 2021 07.
Artículo en Inglés | MEDLINE | ID: mdl-33248299

RESUMEN

OBJECTIVE: Common venous malformations (VMs) are a frequent sporadic subtype of vascular malformations. Given the TEK and PIK3CA mutations identified, this study aims to investigate the genetic landscape of VMs in the head and neck. METHODS: Patients from published sequencing studies related to common VMs were reviewed. Detailed data regarding clinical characteristics, sequencing strategies, and mutation frequency were synthesized. Lesion distribution of common VMs in the head and neck were further retrospectively analyzed by the pathologic database of the Department of Oral and Maxillofacial-Head and Neck Oncology, Shanghai Ninth People's Hospital. For the frequently affected sites in the head and neck, patients were selected for targeted sequencing with a designed vascular malformation-related gene panel or whole exome sequencing. Detected variants were analyzed by classical bioinformatic algorithms (SIFT23, PolyPhen-2 HDIV, LRT, MutationTaster, Mutation Assessor, and GERP++). To confirm the expression pattern of particular candidate gene, specimens were examined histochemically. Gene ontology enrichment analysis and a protein-protein interaction network were also constructed. RESULTS: Three hundred patients from eight sequencing studies related to common VMs were reviewed. The total prevalence rates of TEK and PIK3CA mutations were 41.3% and 26.7%, respectively. The most frequent TEK/PIK3CA mutations were TEK-L914F/PIK3CA-H1047R. TEK/PIK3CA mutations existed in 70.3% and 2.7% of VMs in the head and neck. In retrospective data from 649 patients carrying cervicofacial VMs at Shanghai Ninth Hospital, the most frequent sites were the maxillofacial region (lips, cheek, parotid-masseteric region, submandibular region) and the oral and oropharyngeal region (buccal mucosa, tongue). Targeted sequencing for 14 frequent lesions detected TEK variants in three patients (21.4%), but no PIK3CA mutations. On whole exome sequencing of two patients without TEK/PIK3CA mutations, CDH11 was the only shared deleteriously mutated gene. Bioinformatic analyses of CDH11 implied that genes involved in cellular adhesion and junctions formed a significant portion. CONCLUSIONS: Common VMs of the head and neck have a unique genetic landscape. Novel CDH11 and TEK variants imply that pathogenesis is mediated by the regulatory relationship between endothelial cells and extracellular components.


Asunto(s)
Fosfatidilinositol 3-Quinasa Clase I/genética , Células Endoteliales/fisiología , Cabeza/irrigación sanguínea , Mutación , Cuello/irrigación sanguínea , Receptor TIE-2/genética , Malformaciones Vasculares/genética , Cadherinas/genética , Humanos , Estudios Retrospectivos , Malformaciones Vasculares/patología
16.
Front Aging Neurosci ; 13: 650103, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-33776747

RESUMEN

Alzheimer's disease (AD) is a neurodegenerative disorder characterized by memory impairments, which has no effective therapy. Stem cell transplantation shows great potential in the therapy of various disease. However, the application of stem cell therapy in neurological disorders, especially the ones with a long-term disease course such as AD, is limited by the delivery approach due to the presence of the brain blood barrier. So far, the most commonly used delivery approach in the therapy of neurological disorders with stem cells in preclinical and clinical studies are intracranial injection and intrathecal injection, both of which are invasive. In the present study, we use repetitive intranasal delivery of human neural stem cells (hNSCs) to the brains of APP/PS1 transgenic mice to investigate the effect of hNSCs on the pathology of AD. The results indicate that the intranasally transplanted hNSCs survive and exhibit extensive migration and higher neuronal differentiation, with a relatively limited glial differentiation. A proportion of intranasally transplanted hNSCs differentiate to cholinergic neurons, which rescue cholinergic dysfunction in APP/PS1 mice. In addition, intranasal transplantation of hNSCs attenuates ß-amyloid accumulation by upregulating the expression of ß-amyloid degrading enzymes, insulin-degrading enzymes, and neprilysin. Moreover, intranasal transplantation of hNSCs ameliorates other AD-like pathology including neuroinflammation, cholinergic dysfunction, and pericytic and synaptic loss, while enhancing adult hippocampal neurogenesis, eventually rescuing the cognitive deficits of APP/PS1 transgenic mice. Thus, our findings highlight that intranasal transplantation of hNSCs benefits cognition through multiple mechanisms, and exhibit the great potential of intranasal administration of stem cells as a non-invasive therapeutic strategy for AD.

17.
World J Pediatr ; 17(2): 123-130, 2021 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-32851561

RESUMEN

Kasabach-Merritt phenomenon (KMP) is a rare disease that is characterized by severe thrombocytopenia and consumptive coagulation dysfunction caused by kaposiform hemangioendothelioma or tufted hemangioma. This condition primarily occurs in infants and young children, usually with acute onset and rapid progression. This review article introduced standardized recommendations for the pathogenesis, clinical manifestation, diagnostic methods and treatment process of KMP in China, which can be used as a reference for clinical practice.


Asunto(s)
Síndrome de Kasabach-Merritt/diagnóstico , Síndrome de Kasabach-Merritt/terapia , Niño , China/epidemiología , Diagnóstico Diferencial , Humanos , Síndrome de Kasabach-Merritt/epidemiología , Nivel de Atención
18.
Hua Xi Kou Qiang Yi Xue Za Zhi ; 38(6): 603-615, 2020 Dec 01.
Artículo en Zh | MEDLINE | ID: mdl-33377335

RESUMEN

Large general hospitals currently play an increasingly important role in the diagnosis and treatment for acute critical patients and difficult diseases because of the development of dual referral system and hierarchical diagnosis, as well as the formation of medical treatment alliance. Patients with oral cancers are often associated with systemic diseases, which increases the complexity of the condition. Thus, meeting the demand through the traditional single medical model is difficult. As such, a multidisciplinary team (MDT) model has been proposed and has achieved a good clinical effect. To standardize the application of this model, we organized an event in which relevant experts discussed and formulated a consensus to provide standardized suggestions on the MDT process and the diagnosis and treatment of common systemic diseases as reference for clinical practice.


Asunto(s)
Neoplasias de la Boca , Grupo de Atención al Paciente , Consenso , Humanos , Neoplasias de la Boca/diagnóstico , Neoplasias de la Boca/terapia , Derivación y Consulta
19.
Med Hypotheses ; 72(4): 453-4, 2009 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-19131174

RESUMEN

Gingival epulis represents a family of benign tumors and tumorlike masses of the gingiva. A spectrum of inflammatory and other reactive changes can be observed pathologically as granulation, fibrous or vascular tissue. It may occur at any age, but most commonly found in patients at their twenties and sixties. Women are more frequently affected than men. Surgical excision or removal is the treatment of choice, and wider resection including extraction of the involved teeth and adjacent tissues is often required in order to prevent recurrence. However, wider resection may result in cosmetic and functional problems. Epulis bears some similarities with hemangioma both clinically and histopathologically. Therefore, we hypothesize that intralesional injection of Pingyangmycin may be a safe and effective treatment for epulis. The efficacy of this treatment modality is worthy of further investigation.


Asunto(s)
Bleomicina/análogos & derivados , Neoplasias Gingivales/tratamiento farmacológico , Bleomicina/administración & dosificación , Bleomicina/uso terapéutico , Femenino , Humanos , Inyecciones Intralesiones , Masculino
20.
Zhonghua Yi Xue Za Zhi ; 89(44): 3130-4, 2009 Dec 01.
Artículo en Zh | MEDLINE | ID: mdl-20193276

RESUMEN

OBJECTIVE: To evaluate the short-term efficacy and safety of propranolol in the treatment of infantile hemangiomas. METHODS: Between October 2008 and May 2009, oral propranolol was applied to 58 infants with hemangiomas at a dose of 1.0 - 1.5 mg per kilogram of body weight per day in Linyi, Shandong and Shanghai. There were 19 males and 39 females 1 to 12 months old with a mean age of 4 months. The primary tumor size was 1.5 cm x 1.0 cm to 18.0 cm x 5.0 cm. Twenty-seven were superficial, nine deep-seated and 22 mixed. The tumors were located in head and neck (n = 41), trunk and extremities (n = 12), labium vulvae (n = 2), perianal region (n = 1), perineum (n = 1) and scrotum (n = 1). The patients were hospitalized for 7 to 10 days, continued medication at home and revisited every 2 weeks. The changes of tumor size, texture and color were monitored and recorded at a regular interval. The adverse effects after medication were observed and managed accordingly. The short-term results were evaluated using a 4-point scale system. RESULTS: At 24 hours post-medication, all the tumors decreased in density, color and size. The changes became conspicuous within 5 to 7 days. Seven patients had medication for 2 months, 22 for 3 months, 21 for 4 months and 8 for 5 months. The follow-up period was 5 to 9 months. The overall response was scale I (poor) in 1 patient (1.7%), scale II (moderate) in 12 patients (20.7%), scale III (good) in 35 patients (60.4%) and scale IV (excellent) in 10 patients (17.2%). Statistical analysis showed that the treatment response for deep-seated hemangiomas was significantly better than that for superficial hemangiomas (P < 0.05), but no significant difference was found among different primary sites (P > 0.05). The main adverse effects were bradycardia (100%), diarrhea (63.8%) and sleep change (30.2%), which resolved after expectant treatment without any significant sequel. No serious adverse effect was observed. CONCLUSIONS: Oral propranolol treatment at a low dose is a safe and effective regimen for infantile proliferating hemangiomas. And it can be used as the first-line therapeutic modality. The short-term efficacy is excellent while the side effects are minimal.


Asunto(s)
Hemangioma/tratamiento farmacológico , Propranolol/efectos adversos , Propranolol/uso terapéutico , Relación Dosis-Respuesta a Droga , Femenino , Humanos , Lactante , Masculino , Propranolol/administración & dosificación , Estudios Prospectivos , Resultado del Tratamiento
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