Objective. To explore the clinical and pathological features of microscopic polyangiitis (MPA) in children. Methods. A retrospective analysis was performed of patients with pediatric MPA in our hospital over 10 years. Results. Data for 20 patients were collected; 16 patients had primary MPA (4 boys, 12 girls), with a median age of 8.9 years at the time of disease onset; 4 patients, all female, had antithyroid drug (ATD)-associated MPA, with an age range of 12.5 to 16.2 years at the time of disease onset. All patients exhibited renal involvement. Renal biopsies were performed in 14 patients. Fibrinoid exudation and necrosis of the glomerular capillaries were observed in all biopsy specimens. Crescents and scleroses were noted in 92.9% and 85.7% of these cases, respectively. The most frequent extrarenal organs involved were lungs, followed by the central nervous system (CNS), skin, and digestive system. Ninety percent of patients were positive for perinuclear antineutrophil cytoplasmic antibody, 94.1% were positive for myeloperoxidase, and 88.2% were positive for both. Forty-five percent of the patients had received steroid plus cyclophosphamide (CTX) pulse therapy for more than 3 months, and varying degrees of remission had been achieved in 88.9% of the patients. Conclusion. Both primary and ATD-associated MPA showed a female predisposition. Renal involvement was the most frequently observed condition, followed by involvement of lungs. CNS involvement was not rare in these pediatric patients. The efficacy of steroid plus CTX as induction therapy was evident in these patients.
Objective
To analyze the echocardiographic diagnosis of left atrial isomerism, and to explore the clinical prognosis and prognosis of such patients.
Methods
From January 2010 to October 2018, 47 cases of left atrial isomerism confirmed by echocardiography at Beijing Anzhen Hospital Affiliated to Capital Medical University were collected. The echocardiographic features, classification, surgical treatment, and prognosis were analyzed retrospectively.
Results
Among 47 patients with left atrial isomerism, 35 (35/47, 74.5%) had complex malformations, 10 (10/47, 21.3%) had simple malformations, and 2 (2/47, 4.2%) were not complicated with malformations. Among the 35 cases with complex malformations, 16 were polytypic in lung blood and 19 were hypotypic. Among the 47 patients with left atrial isomerism, 2 had abnormal visceral position, and 14 had abnormal cardiac position, including 11 (11/47, 23.4%) cases of right heart and 3 (3/47, 6.4%) cases of middle heart; 38 were complicated with polysplenic malformation (38/47, 80.9%) and 1 complicated with asymptomatic malformation; 42 (42/47, 89.4%) were complicated with bronchial and pulmonary malformations; 47 were combined with absence of the hepatic segment of the inferior vena cava (47/47, 100.0%); 23 (23/47, 48.9%) had abnormal superior vena cava connection; 47 (47/47, 100.0%) had abnormal hepatic venous connection; 6 (6/47, 12.8%) had abnormal pulmonary venous drainage; 9 (9/47, 19.1%) had abnormal electrocardiogram or dynamic electrocardiogram findings; 26 underwent orthodontic or palliative surgery, of which 21 had complicated malformations and 5 had simple malformations. Among the 26 cases undergoing surgical treatment, 4 died, all of whom had complicated malformations.
Conclusion
Left atrial isomerism patients are often complicated with cardiovascular and other organ malformations, which can be diagnosed by echocardiography. Echocardiography plays an important role in the diagnosis of left atrial isomerism and the accurate evaluation of associated malformations, as well as in the formulation of clinical treatment plans and the evaluation of the prognosis of patients.
Key words:
Heart defects, congenital; Echocardiography, stress
The pathogenesis of proteinuria in Alport syndrome (AS) remains unclear. Vascular endothelial growth factor A (VEGFA) is a key regulator of the glomerular filtration barrier (GFB). This study explored the expression of VEGFA in the glomeruli and its accumulation in the glomerular basement membrane (GBM) and their relationship with podocyte injury and proteinuria in Alport syndrome (AS). Clinical data and renal tissues of control patients (11 cases) and AS patients (25 cases) were included. AS patients were further divided into 2 groups according to the quantities of their urinary protein: mild to moderate proteinuria group (proteinuria <50 mg/kg/d, 15 cases) and heavy proteinuria group (proteinuria ≥50 mg/kg/d, 10 cases). The expression and distribution of VEGFA and VEGF receptor 2 (VEGFR2) in the GFB, the phosphorylation of VEGFR2 (p-VEGFR2) and nephrin (p-nephrin), and the expression of synaptopodin and nephrin in the glomeruli were detected by immune electron microscopy and/or immunofluorescence, and their relationships to proteinuria in AS patients were analyzed. The accumulation of VEGFA in the GBM was increased in AS patients. The expression of VEGFA and the levels of p-VEGFR2 and p-nephrin in glomeruli were increased and were positively correlated with the degree of proteinuria in AS patients. The expression of synaptopodin and nephrin were decreased and were negatively correlated with the degree of proteinuria in AS patients. The over expressed VEGFA in the glomeruli and its accumulation in the GBM may activate the VEGFA-VEGFR2 and nephrin signaling pathways and lead to podocyte injury and occurrence of proteinuria in AS.
To study the clinical and pathological features of Denys-Drash syndrome (DDS).Three DDS cases who were treated in our department from December 2009 to June 2011 were subjected to this study by reviewing of literature.Both case 1 and case 2 were female, with karyotype 46, XX. Case 3 was male with bilateral cryptorchidism. The ages of nephropathy onset of the three cases were 1 year and 9 months, 2 years and 8 moths, and 3 months respectively. Proteinuria in case 2 and case 3 were evidenced to be resistant to steroid. Case 1 was partially responsive to tacrolimus, plasma albumin and cholesterol were improved, although proteinuria was persistent after Tacrolimus was administered. Remission was achieved in case 2 after administration of cyclosporine A and later tacrolimus, and her renal function remains normal till present (4 years and 9 months). Residue renal histology revealed diffused mesangial sclerosis (DMS) in all three patients. All of the three patients had developed right unilateral Wilms tumor. A novel WT1 missense mutation exon 9 c.1213C > G was detected in case 1. WT1 exon 9 c.1168C > T nonsense mutation and exon 8 c.1130A > T missense mutation were detected in case 2 and case 3, respectively.The clinical manifestation of nephropathy in DDS is variable. The majority present with early onset nephropathy and reach renal failure before the age of 4 years. But in a few patients, nephropathy can also be present much later and progress slowly. Proteinuria in DDS is resistant to steroid but is responsive to calcineurin inhibitors, including Cyclosporine A. The effectiveness of tacrolimus was also observed in this study. DDS is evidently caused by WT1 mutation. DMS is the characteristic renal pathological change in DDS.
A method to prepare carbon-coated aluminum nanoparticles and carbon quantum dots by arc ablation of electrodes in liquid nitrogen environment is proposed. The thermal arc plasma is initiated by contacting and releasing of the electrodes. The target products are formed by evaporation-condensation process and the small droplets sputtering from the molten electrode under the continuous arc ablation. Without any vacuum devices, the carbon-coated aluminum nanoparticles and carbon quantum dots were obtained in a single step. The carbon-coated aluminum nanoparticles were characterized by X-ray diffraction, high resolution transmission electron microscopy, energy dispersive spectroscopy, Raman analysis, and dynamic light scattering method. The effect of different currents on the particle size of carbon-coated aluminum nanoparticles was investigated. The results indicates that the obtained carbon-coated aluminum nanoparticles are encapsulated by several graphene layers. In the range of 20A - 35A, the particle size becomes larger with increasing current. The carbon quantum dots in the products are separated and confirmed by HAADF-STEM and HRTEM. This method is promising for the fabrication of carbon-coated aluminum nanoparticles and carbon quantum dots.
A spinel LiMn2O4 is investigated via Raman spectroscopy at 514.5-nm excitation and X-ray diffraction. The dependence of Raman spectra on the different irradiated laser powers is determined and found to be different from that at 632.8-nm excitation. Based on our extensive analysis, our experimental results can be attributed to the laser heating effect, which reduces the Mn4+ cation concentration in the local area. Consequently, the decrease in average Mn valence in the local area unavoidably induces the Jahn-Teller effect and local lattice distortion, which accounts for the evolution of the measured Raman spectra of spinel LiMn2O4.
Preeclampsia (PE) is a special complication during pregnancy, which can cause severe maternal complications and lead the cause of maternal and perinatal death. So far, the etiology and pathogenesis of the disease is still not very clear. Currently, microRNAs (miRNAs) are reported to be the key regulators in the development of PE.The miR-199a-5p expression was detected by qRT-PCR. The expression of vascular endothelial growth factor A (VEGFA), placental growth factor (PLGF) and activating transcription factor 3 (ATF-3) were detected by qRT-PCR and Western blot. Transwell-invasion assay wasused to assess the effects of miR-199a-5p, PLGF and ATF-3 on the invasion of HTR-8/SVneo and TEV-1cell lines. Western blot and qRT-PCR were used to assess the related molecular mechanisms. Dual luciferase reporter assay was used to detect the interaction between miR-199a-5p and VEGFA.Here, weinitially demonstrated that in PE tissues, miR-199a-5p expression was higher than that in normal tissues, while there was sharp reduction in VEGFA. In placental tissues of PE patients, miR-199a-5p exhibited a negatively correlation with VEGFA. The invasion of HTR-8/SVneo and TEV-1 cells was suppressed by miR-199a-5p through direct inhibition of VEGFA expression. In addition, PE tissues were associated with sharp reduction in the protein levels of PLGF, ATF-3 and histone deacetylase 6 (HDAC6) compared with the normal tissues. We further proved that over-expression of PLGF could also promote HTR-8/SVneo and TEV-1 cells invasion through up-regulating ATF-3 expression and down-regulating DNM3 opposite strand (DNM3os) and miR-199a-5p expression. Lastly, we also found that tubacin suppressed HTR-8/SVneo and TEV-1 cells invasion via regulation of miR-199a-5p and VEGFA expression.Our data demonstrated the role of miR-199a-5p in the preeclampsia, and proved that miR-199a-5p could act as a potential therapeutic target for the treatment of PE.
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