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Objective:To evaluate the predictive values of the Status Epilepticus in Pediatric Patients Severity Score (STEPSS) and END-IT score in the short-term prognosis of children with status epilepticus (SE).Methods:It was a retrospective study involving 103 children with SE who were admitted to the Qingdao Women and Children′s Hospital Affiliated to Qingdao University from January 1, 2012 to January 1, 2022.Glasgow Outcome Scale was used to evaluate the prognosis at discharge, and the children were divided into good prognosis group ( n=78) and poor prognosis group ( n=25). Risk factors for poor prognosis of SE in children were analyzed by Logistic regression.Receiver operating characteristic (ROC) curve was used to evaluate the prognostic values of STEPSS and END-IT score in children with SE. Results:Compared with those of the good prognosis group, significantly younger age [16 (9, 58) months vs.56 (21, 84) months, Z=-3.068, P=0.002], higher blood lactic acid levels [3.16 (2.43, 4.01) mmol/L vs.1.67 (1.32, 2.10) mmol/L, Z=-6.085, P<0.001], STEPSS scores [3.0(3.0, 4.0) points vs.1.0(1.0, 2.0) points, Z=-6.956, P<0.001], END-IT scores [3.0(1.5, 4.0) points vs.1.0(0, 1.0) points, Z=-5.502, P<0.001], proportion of developmental delay ( χ2=16.756, P<0.001), abnormal brain magnetic resonance imagine examination ( χ2=5.860, P=0.015), use of ventilator and multiple drugs (all P<0.001), and longer duration of anti-SE therapy time( Z=1.488, P=0.024) were detected in the poor prognosis group. Logistic regression analysis indicated that increased blood lactic acid ( OR=7.975, 95% CI: 2.705-23.518), increased drug types ( OR=14.562, 95% CI: 2.035-104.173), STEPSS scores( OR=8.914, 95% CI: 2.824-28.140) and END-IT scores ( OR=2.209, 95% CI: 1.046-4.667) were risk factors for the poor prognosis of SE in children.The area under the curve (AUC) of STEPSS in predicting the poor prognosis of SE in children was 0.939, with the cut-off value, sensitivity, specificity and Youden index of 2.5 points, 96.0%, 85.9% and 0.82, respectively.AUC of END-IT scores in predicting the poor prognosis of SE in children was 0.853, with the cut-off value, sensitivity, specificity and Youden index of 1.5 points, 76.0%, 75.6% and 0.52, respectively.AUC of STEPSS in predicting the poor prognosis of SE in children was significantly higher than that of END-IT scores ( U=36.91, P<0.05). The predictive value of STEPSS combined with END-IT was higher, and the sensitivity and negative predictive value of parallel test were 100.0%, while the specificity and positive predictive value of series test were 94.9% and 81.8%, respectively. Conclusions:STEPSS and END-IT scores may be used as predictors for the poor prognosis of SE in children.Their combination provides a better prediction.
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Objective:To investigate the mechanism of dexmetomidine (DEX) in improving lung injury in septic mice.Methods:Male C57BL/6 mice were randomly assigned to the blank group (NC), sham operation group (sham), cecal ligation and puncture group (CLP), and Dex treatment group (CLP+DEX), 36 mice per group. Mice in the CLP group were intraperitoneally injected with 1 mL sterile saline 15 min before CLP, and mice in the CLP + DEX group were intraperitoneally injected with 50 μg/kg DEX 15 min before CLP. The survival rate was recorded within 24 h after CLP. The mice were sacrificed at 0, 3, 6, 12, and 24 h after CLP, and lung tissues were collected. The expression levels of cytokines (IL-6, IL-1β, TNF-α) and lncRNA-HOTAIR in the lung of mice were detected by qPCR. RAW264.7 cell were cultured in vitro, LPS (100 ng/mL) and DEX (1 μ mol/L) were used to establish a cell model for studying the mechanism of Dex, and the expression of cytokines (IL-6, IL-1β, TNF-α) and lncRNA-HOTAIR in RAW264.7 cell model were detected by qPCR. In addition, the effect of lncRNA-HOTAIR on sepsis was explored in vivo and in vitro by knockdown or overexpression of HOTAIR.Results:The survival rate of the CLP+DEX group was higher than that of the CLP group within 24 h after surgery, and the levels of IL-6, IL-1β, and TNF-α in the lungs were significantly lower than those in the CLP group at 6, 12, and 24 h after surgery ( P<0.05). In addition, the level of lncRNA HOTAIR showed that the expression level of lncRNA HOTAIR in the lungs of mice were decreased after Dex treatment, and were decreased 1.1 times ( P<0.05), 4.0 times ( P<0.01) and 3.8 times ( P<0.01) at 6, 12, and 24 h, respectively. Compared with the NC group, knockdown of HOTAIR significantly decreased the levels of IL-1β, IL-6, and TNF-α in septic mice ( P<0.05), and overexpression of HOTAIR significantly increased the levels of IL-1β, IL-6, and TNF-α in septic mice ( P<0.01). Conclusions:DEX can reduce the production of inflammatory factors in the lungs of septic mice and improve the survival rate of septic mice. The mechanism may be related to the inhibition of HOTAIR expression.
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Objective:To establish the fingerprint of Bupleuri Radix with Ultra High Performance Liquid Chromatography (UPLC) method and combining Principal Component Analysis to evaluate the quality of Bupleuri Radix in different areas. Methods:Acquity UPLC BEH-C 18 (2.1 mm×100 mm, 1.7 μm) column was used with acetonitrile (A)-water (B) solution, gradient elution. The column temperature was 30 ℃, the flow rate was 0.3 ml/min, and the detection wavelength was 200 nm, injection volume 5 μl. Results:There were 7 common peaks in the UPLC fingerprints of 10 batches of medicinal materials, and the similarity was 0.940-0.975. Through the principal component analysis, the cumulative contribution rate of three main component factors was 90.977%,and comprehensive score of S5 (Hubei) was the highest with the best quality.Conclusions:There are certain quality differences of different areas in Bupleuri Radix. Through the combination of fingerprint and principal component analysis, it can provide reference for quality control, development and application of Bupleuri Radix.
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Objective:To prepare a peptide fluorescent probe based on aggregation-induced emission and to investigate its application in the detection of early caries.Methods:Eight aspartate-serine-serine (DSS) were combined with aggregation-induced emission material to prepare peptide fluorescent probes, and an artificial demineralization model was established in vitro. The samples were immersed in the peptide fluorescent probe solution for 1 min, and a fluorescence imaging system was applied to examine the tooth samples and collect images and fluorescence data. Scanning electron microscopy was also applied to observe the phenotype of the teeth, and electron microscopy was applied to detect the calcium-phosphorus ratio on the enamel surface of the teeth. Polarized light microscopy was also applied to observe the enamel area of the teeth. Results:The fluorescence intensity of demineralized teeth was clearly observed to be lower than that of normal teeth in the peptide fluorescent probe-treated area, and the difference was statistically significant ( P < 0.05). The results of scanning electron microscopy showed that the enamel surface of the demineralized group had more irregular pores, while the enamel surface of the undemineralized group was flatter with only some irregular accumulation of flakes. The results of polarized light microscopy showed that a clear birefringence could be observed in the enamel region of normal teeth, while a black area or the disappearance of the birefringence effect accompanied by a partial black dark shadow could be observed in the enamel region of demineralized teeth. Conclusions:An aggregation-induced luminescence-based peptide fluorescent probe was successfully prepared, which can precisely localize the enamel and show some application value in early caries detection.
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It is the main method to save the ischemic penumbra and improve the nerve function for the patients with ischemic stroke to realize the vascular recanalization within the time window. However, the possible hyperperfusion after successful recanalization will result in neurological deterioration and poor outcome. This article reviews the risk factors for cerebral hyperperfusion after vascular recanalization.
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OBJECTIVE@#To investigate whether gut microbiota disturbance after cardiopulmonary bypass (CPB) contributes to the development of perioperative neurocognitive disorders (PND).@*METHODS@#Fecal samples were collected from healthy individuals and patients with PND after CPB to prepare suspensions of fecal bacteria, which were transplanted into the colorectum of two groups of pseudo-germ-free adult male SD rats (group NP and group P, respectively), with the rats without transplantation as the control group (n=10). The feces of the rats were collected for macrogenomic sequencing analysis, and serum levels of IL-1β, IL-6 and TNF-α were measured with ELISA. The expression levels of GFAP and p-Tau protein in the hippocampus of the rats were detected using Western blotting, and the cognitive function changes of the rats were assessed with Morris water maze test.@*RESULTS@#In all the 3 groups, macrogenomic sequencing analysis showed clustering and clear partitions of the gut microbiota after the transplantation. The relative abundances of Klebsiella in the control group (P < 0.005), Akkermansia in group P (P < 0.005) and Bacteroides in group NP (P < 0.005) were significantly increased after the transplantation. Compared with those in the control group, the rats in group NP and group P showed significantly decreased serum levels of IL-1β, IL-6 and TNF-α and lowered expression levels of GFAP and p-Tau proteins (all P < 0.05). Escape platform crossings and swimming duration in the interest quadrant increased significantly in group NP (P < 0.05), but the increase was not statistically significant in group N. Compared with those in group P, the rats in group NP had significantly lower serum levels of IL-1β, IL-6 and TNF-α and protein expressions of GFAP and p-Tau (all P < 0.05) with better performance in water maze test (P < 0.05).@*CONCLUSION@#In patients receiving CPB, disturbances in gut mirobiota contributes to the development of PND possibly in relation with inflammatory response.
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Male , Animals , Rats , Rats, Sprague-Dawley , Cardiopulmonary Bypass , Gastrointestinal Microbiome , Interleukin-6 , Tumor Necrosis Factor-alpha , Neurocognitive DisordersABSTRACT
Abstract@#Allergic diseases can occur in all systems of the body, covering the whole life cycle, from children to adults and to old age, can be lifelong onset and even fatal in severe cases. Children account for the largest proportion of the victims of allergic disease, Children s allergies start from scratch, ranging from mild to severe, from less to more, from single to multiple systems and systemic performance, so the prevention and treatment of allergic diseases in children is of great importance, which can not only prevent high risk allergic conditions from developing into allergic diseases, but also further block the process of allergy. At present, there is no consensus on the management system of allergic children in kindergartens and primary schools. The "Consensus on Allergy Management and Prevention in Kindergartens and Primary Schools", which includes the organizational structure, system construction and management of allergic children, provides evidence informed recommendations for the long term comprehensive management of allergic children in kindergartens and primary schools, and provides a basis for the establishment of the prevention system for allergic children.
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Pustular psoriasis is a serious life-threatening disease, and patients usually show poor response to traditional treatments. In recent years, interleukin-17 and interleukin-23 inhibitors have shown favorable efficacy in the treatment of psoriasis. This review summarizes the latest progress in interleukin-17 and interleukin-23 inhibitors for the treatment of pustular psoriasis.
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Chronic spontaneous urticaria (CSU) is a recurrent systemic inflammatory disease. At present, there are few biological indicators that can effectively reflect CSU activity in clinical practice, and some indicators have not been widely used. To identify sensitive and easy-to-check indicators may be helpful for the diagnosis and treatment of as well as prediction of therapeutic efficacy in CSU. This review comprehensively summarizes current clinical application of indicators related to CSU activity, in order to provide a reference for clinical practice and related scientific research.
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Objective:To explore predictive factors for the efficacy of omalizumab in the treatment of refractory chronic spontaneous urticaria (CSU) .Methods:Totally, 40 patients with refractory CSU treated with omalizumab were enrolled from Department of Dermatology, the Second Affiliated Hospital of Soochow University from 2019 to 2021. Before treatment, clinical data including the urticaria activity score over 7 days (UAS7) and dermatology life quality index (DLQI) were collected; venous blood samples were collected for the detection of total immunoglobulin E (IgE) antibodies, eosinophil counts and basophil counts, anti-thyroid peroxidase (TPO) IgG antibody levels, mean platelet volume, as well as C-reactive protein (CRP) , D-dimer, complements C3 and C4, interleukin (IL) -2, IL-4, IL-6, IL-10, IL-17A, tumor necrosis factor (TNF) -α and interferon (IFN) -γ levels, and percentages of CD4 + T cells and CD8 + T cells; meanwhile, the autologous serum skin test (ASST) was performed. After 12-week treatment with omalizumab, 40 CSU patients were divided into well-responding group and poorly-responding group according to the UAS7 score, and the above laboratory indicators were compared between the two groups. For continuous variable indicators with significant differences, the accuracy of prediction and optimal cut-off values were determined by using the receiver operating characteristic (ROC) curve; for categorical variable indicators with significant differences, the sensitivity and specificity for the prediction of poor clinical response to omalizumab were calculated; correlations among the above indicators were analyzed by Pearson correlation analysis. Results:After 12-week treatment with omalizumab, 28 CSU patients responded well to omalizumab, and 12 responded poorly. Before treatment, the poorly-responding group showed significantly increased proportions of patients with eosinopenia (6/12) , basopenia (7/12) , decreased C3 (6/12) , decreased C4 (6/12) , positive anti-TPO IgG antibodies (5/12) and low total IgE levels (8/12) , increased proportion of CD4 + T cells (71.13% ± 3.26%) , and increased IL-17A levels (27.16 ± 9.75 pg/ml) compared with the well-responding group (14.3%, 10.7%, 14.3%, 7.1%, 10.7%, 14.3%, 60.33% ± 5.12%, 19.24 ± 10.84 pg/ml, respectively; all P < 0.05) , but decreased IL-6 levels compared with the well-responding group ( t = 5.75, P < 0.05) . According to the ROC analysis and calculation of sensitivity, specificity and accuracy, the above indicators showed high accuracy in predicting therapeutic effect of omalizumab, and the optimal cut-off values of IL-6, IL-17A, and CD4 + T cell proportion were 8.672 pg/ml, 23.415 pg/ml, and 67.95%, respectively. In addition, the IL-6 level was significantly positively correlated with the total IgE level in CSU patients at baseline ( r = 0.43, P = 0.006) . Conclusion:Before the selection of omalizumab for the treatment of refractory CSU, there is a need to detect the eosinophil and basophil counts, levels of complements C3, C4, anti-TPO IgG antibodies, total IgE, IL-17A and IL-6, and CD4 + T cell proportions to predict therapeutic effect of omalizumab, so as to determine whether omalizumab is suitable for the patients.
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Objective:To evaluate the role of α7 nicotinic acetylcholine receptor (α7nAChR) in penehyclidine hydrochloride-induced reduction of endotoxin-induced acute lung injury (ALI) in mice.Methods:Forty SPF healthy male C57BL/6 mice, aged 6-8 weeks, weighing 18-25 g, were divided into 4 groups ( n=10 each) using a random number table method: control group (group C), ALI group, penehyclidine hydrochloride group (PHC group), and α7nAChR inhibitor MLA group (MLA group). ALI was induced by intraperitoneal injection of lipopolysaccharide 15 mg/kg in anesthetized animals, while normal saline was given instead in group C. In PHC group, penehyclidine hydrochloride 2 mg/kg was intraperitoneally injected at 30 min before developing the model. MLA 10 mg/kg was intraperitoneally injected at 10 min before administration of penehyclidine hydrochloride in MLA group. Mice were sacrificed at 6 h after lipopolysaccharide administration, and lung tissues were collected for microscopic examination of the pathological changes (by HE staining) and for determination of the wet/dry weight ratio (W/D ratio), content of tumor necrosis factor-alpha (TNF-α), interleukin-1beta (IL-1β) and IL-10 (by enzyme-linked immunosorbent assay) and expression of α7nAChR (by Western blot). Results:Compared with C group, the W/D ratio and contents of TNF-α and IL-1β were significantly increased, the content of IL-10 was decreased, and the expression of α7nAChR was up-regulated in ALI, PHC and MLA groups ( P<0.05). Compared with ALI group, the W/D ratio and contents of TNF-α and IL-1β were significantly decreased, the content of IL-10 was increased, and the expression of α7nAChR was up-regulated in PHC group ( P<0.05). Compared with PHC group, the W/D ratio and contents of TNF-α and IL-1β were significantly increased, the content of IL-10 was decreased, and the expression of α7nAChR was down-regulated in MLA group ( P<0.05). Compared with ALI group, the pathological changes of lung tissues were significantly mitigated in PHC group, while this effect of PHC was partially reversed by α7nAChR inhibitor MLA. Conclusions:α7nAChR is involved in penehyclidine hydrochloride-induced reduction of endotoxin-induced ALI in mice.
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The gynecological malignancy has a high incidence and mortality. More efficient treatment methods still need to be explored to improve the survival benefits. Artificial intelligence (AI) aims to intelligently process the original problems by simulating the thinking way of human brain. It has obtained significant progress in gynecological malignancy, with great potential in the field of cancer diagnosis and treatment. This paper reviews the application of AI in the diagnosis and treatment of gynecological malignancy, and mainly introduces the research progress on AI in the radiotherapy. This paper mainly focuses on the key issues such as automatic delineation, dose prediction, radiotoxicity prediction and efficacy prediction, and discusses the current benefits and limitations of AI in radiotherapy of gynecological malignancy.
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Objective:To compare the nutritional evaluation methods and to analyze the clinical value of different nutritional evaluation indexes in acute lymphoblastic leukemia (ALL) pediatric patients during the stage of remission induction.Methods:The clinical data of 132 ALL pediatric patients who were diagnosed and treated with remission induction in Shanghai Children′s Medical Center, School of Medicine, Shanghai Jiao Tong University from January 2018 to December 2020 were collected by convenient sampling method. The body composition was measured by direct segmental multifrequency bioelectrical impedance measurement (DSM-BIA), and the height, weight, upper arm circumference and triceps skinfold thickness were also measured. The Screening Tool for the Assessment of Malnutrition in Pediatrics scores were calculated. The serum albumin value and the hemoglobin value were monitored. Using DSM-BIA as the reference standard, the accuracy of different nutritional evaluation indexes was investigated and analyzed.Results:During the stage of remission induction in ALL pediatric patients, there were 69 out of 132 children who had the muscle mass below normal. The muscle mass below normal was detected in 52.3% (69/132). And the upper arm circumference had the highest correlation and the minimal standard error with muscle mass measured by DSM-BIA ( R2=0.520, SE=0.180). Grouped with nutritional status by the upper arm circumference, the children with normal nutritional status had lower incidence of very severe neutropenia 76.8% (24/32) than 92.1% (70/76) with abnormal nutritional status ( χ2=6.14, P=0.013). Conclusions:During the stage of remission induction in ALL pediatric patients, the malnutrition rate is high. The upper arm circumference is a simple index which can accurately reflect the nutritional status of the ALL pediatric patients. It is suggested that the upper arm circumference should be used as the primary screening index of clinical nutrition by clinical nurses.
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Objective:To investigate the effect of oxytocin on neonatal instinctive behavior expression and breast-feeding.Methods:A total of 71 pairs of parturient women and their newborns who delivered in the delivery room of the Affiliated Hospital of Weifang Medical College from February to May 2021 were selected. According to whether oxytocin induced labor, they were divided into the oxytocin group and the control group. Widstr?m nine stages was used to observe and record neonatal behavior. Breastfeeding was followed up 3 days and 3 months after delivery.Results:Totally 35 pairs in each group were included. In the oxytocin group, the occurrence time of neonatal head or head turning, hand in mouth, body moving to nipple, lips touching areola, licking nipple, nipple and effective sucking were (18.2 ± 5.4), (27.8 ± 8.3), (31.0 ± 10.1), (44.3 ± 14.5), (47.2 ± 15.6), (49.4 ± 16.3), (48.3 ± 13.6) min, which were significantly later than those in the control group (15.3 ± 5.3), (21.0 ± 8.1), (24.3 ± 9.0), (34.0 ± 11.4), (37.2 ± 11.9), (38.6 ± 11.8), (39.6 ± 8.7) min. The difference was statistically significant ( t values ranged from -3.10 to -2.17, P<0.05). The duration of neonatal awakening, activity, crawling and familiarity in the oxytocin group were (6.9 ± 3.2), (18.9 ± 9.3), (13.6 ± 7.9), (9.2 ± 5.1) min, which were significantly longer than those in the control group (5.1 ± 2.8), (12.3 ± 7.1), (10.3 ± 5.3), (6.7 ± 4.3) min; sucking stage duration in the oxytocin group was (35.1 ± 7.2) min, which was significantly shorter than that in the control group (39.6 ± 7.1) min; all the differences were statistically significant ( t values ranged from -3.25 to 2.28, P<0.05). The times of exclusive breast-feeding in the oxytocin group were (2.8 ± 3.1), (4.5 ± 3.3), (6.9 ± 3.0) at 24, 48 and 72 h postnatal, and the exclusive breastfeeding rate at 3 months after birth was 77.1%(27/35), which were significantly lower than those (7.6 ± 3.6), (8.9 ± 2.7), (10.3 ± 2.0) and 82.9%(29/35) in the control group, the differences were statistically significant ( t=6.05, 6.11, 5.48, χ2=0.36, P<0.05). Conclusions:The use of oxytocin during labor may affect the expression of neonatal lactation behavior and negatively affect the breastfeeding.
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AMP-activated protein kinase (AMPK) is an energy sensor, which can regulate the energy metabolism of cells by regulating mitochondrial oxidation capacity and substrate utilization, affecting various pathways such as glucose and fatty acid transport. Recent studies have shown that energy metabolism disturbance is a "blasting fuse" leading to a series of secondary injuries after cerebral ischemia-reperfusion, including oxidative stress, acidosis, apoptosis, and excitotoxicity. AMPK participates in most pathophysiological processes of cerebral ischemia-reperfusion injury by regulating cellular energy metabolism. This article reviews the mechanism of action of AMPK signaling pathway in cerebral ischemia-reperfusion injury.
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Pediatric acute liver failure(PALF)is a rare and life-threatening clinical syndrome caused by multiple etiologies with high mortality, rapid progress and bad prognosis.It′s crucial that how to identify liver failure early and evaluate the operation opportunity of liver transplantation accurately.Traditional serum biomarkers can be used to diagnose and evaluate the conditions of PALF, and they can also provide suggestions for treatment.In recent years, some new biomarkers of PALF have been emerged.This review summarized the traditional biomarkers and new biomarkers according to liver′s biological functions, aiming to improve the ability of clinicians to diagnose and treat PALF.
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This article aims to review the clinical efficacy of existing non-pharmacological therapies for post-stroke depression （PSD）， in order to provide references for the comprehensive treatment of PSD. As a common complication of stroke， PSD seriously affects patients' rehabilitation and daily life， and brings a heavy burden to patients and their families. Non-pharmacological therapy is an important intervention approach for PSD. Therefore， this article gives a review on the non-pharmacological therapies for PSD including psychotherapy and physical therapy.
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Objective:To evaluate the diagnosis and critical value reporting of ruptured tubal pregnancy by ultrasound.Methods:The clinical and ultrasound data of 70 patients with ruptured tubal pregnancy diagnosed by surgery in Peking Union Medical College Hospital from August 2017 to May 2021 were retrospectively analyzed to evaluate the diagnosis and critical value reporting of ruptured tubal pregnancy by ultrasound.Results:Among the 70 patients, 68 patients underwent gynecological ultrasonography and two patients did not. Sixty-three cases (92.6%, 63/68) were accurately diagnosed as adnexal ectopic pregnancy mass, abdominal and pelvic effusion/blood clot, and 5 adnexal ectopic pregnancy masses (7.4%, 5/68) were missed.Among the 5 missed cases, 4 cases (80%, 4/5) were heterotopic pregnancy (2 cases of IVF-ET 2 embryos, 1 case of patient taking ovulation induction drugs, 1 case of gravida with twin family history) and 1 case (20%, 1/5) of single tubal pregnancy. Critical values were reported in the all 63 cases of ruptured tubal pregnancy diagnosed by preoperative ultrasound.Conclusions:Ultrasound could accurately diagnose tubal pregnancy and assess its rupture, and timely report the critical value, effectively guarantee the medical safety. The particularity and complexity of ultrasound diagnosis in early pregnancy with assisted reproductive technique deserve more attention.
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Objectives:To analyze the clinical features of microscopic polyangiitis (MPA), and observe the clinical outcomes of different pathological types.Methods:The clinical data of 61 patients with MPA in Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine from January 2010 to December 2019 were analyzed retrospectively. According to age, the patients were divided into ≥ 60 years old group (46 cases) and<60 years old group (15 cases). According to the initial serum creatinine, the patients were divided into ≥ 500 μmol/L group (18 cases) and<500 μmol/L group (43 cases). The basic data and laboratory examination results of the patients were recorded, and the disease activity was evaluated by Birmingham systemic vasculitis activity score (BVAS). Twenty-three patients with complete pathological data were pathologically classified and followed up to assess their clinical outcomes. The progression to end-stage renal disease (ESRD) or death was defined as the endpoint.Results:Ferritin in ≥60 years old group was significantly higher than that in<60 years old group: 452 (289, 792) μg/L vs. 210 (119, 451) μg/L, and there was statistical difference ( P<0.05). The fever rate, hemoglobin and platelets in creatinine ≥ 500 μmol/L group were significantly lower than those in creatinine<500 μmol/L group: 3/18 vs. 48.8% (21/43), 77.5 (62.8, 86.0) g/L vs. 85.0 (77.0, 104.0) g/L and 192 (147, 234) × 10 9/L vs. 257 (208, 365) × 10 9/L, the gastrointestinal involvement and BVAS in creatinine ≥ 500 μmol/L group were significantly higher than those in creatinine<500 μmol/L group: 16/18 vs. 25.6% (11/43) and 20.0 (16.0, 23.3) scores vs. 15.0 (12.0, 19.0) scores, and there were statistical differences ( P<0.05 or<0.01). Pearson correlation analysis result showed that BVAS was positively correlated with creatinine ( r = 0.42, P<0.01), negatively correlated with hemoglobin ( r = -0.42, P<0.01), but it had no correlation with erythrocyte sedimentation rate and platelets ( r = 0.05 and 0.04, P>0.05). Among the 23 patients with completed the clinical outcome statistics, endpoint events occurred in 5 of 6 patients with crescent renal pathology, and in 7 of 12 patients with severe renal interstitial injury. Kaplan-Meier survival curve analysis result showed that the average survival time in ESRD MPA patients was significantly shorter than that in non ESRD MPA patients (41.2 months vs. 63.5 months), and there was statistical difference ( χ2 = 0.48, P = 0.028). Conclusions:The clinical manifestations of elderly MPA patients are similar to those of young MPA patients. Creatinine≥500 μmol/L or anemia at initial onset indicate higher vasculitis activity in MPA. The prognosis of MPA patients with pathological manifestations of crescent or severe interstitial injury is poor, and the survival rate of ESRD is lower than that of non ESRD patients.
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Objective:To analyze the relationship between FAB morphological classification and World Health Organization (WHO) 2016 classification in children with acute erythroid leukemia(AEL), and to summarize the clinical features and prognosis.Methods:Clinical data of de nova childhood AEL patients from January 1, 2002 to December 31, 2019, in Pediatric Blood Disease Center, Institute of Hematology & Blood Disease Hospital were retrospectively analyzed.All of them were re-evaluated according to the WHO 2016 classification.Results:(1) A total of 20 patients were diagnosed as AEL by FAB classification.According to the criteria of WHO 2016, they were re-diagnosed as myelodysplastic syndromes (MDS)- refractory anemia with excess of blasts (11 cases), acute myeloid leukemia with MDS-related changes (3 cases), acute monocytic leukemia (1 case), and pure red leukemia (PEL, 5 cases). (2) Pathological hematopoiesis was frequently detected in bone marrow smears.Auer bodies were seen occasionally in some blasts.The most common antigen expressing were CD 117, CD 13, CD 33, CD 34, CD7, and CD 38.Karyotype analysis was performed in 18 cases successfully, involving 6 cases with abnormal karyotypes, including + 8, -7, 22p+ , t (3; 5: ? ), + 3q-, 15q-, and del (9)(q13). (3) Thirteen cases were treated by chemotherapy, and the one-course complete remission rate was 38.5%.By July 1, 2020, only 2 cases were alive without disease.The overall survival was 49 months and 11 months, respectively. Conclusions:Childhood AEL is susceptible to pathological hematopoiesis, poor response to early chemotherapy and poor prognosis.After re-evaluation according to WHO 2016 classification, most of them were diagnosed as MDS-related.Therefore, adjusting the suitable induction regimen with allogeneic hematopoietic stem cell transplantation may improve the prognosis.