The core pathogenesis of pediatric IgA vasculitis （IgAV） is considered to involve qi movement disorder， with the failure of clear-turbid separation and internal generation of turbid pathogens. Accordingly， the viewpoint of "qi disorder causing turbidity" is proposed. In clinical practice， the disease is divided into the acute， persistent， and stable stages. The acute stage is characterized by impaired dispersion of wei （卫） qi and the internal generation of heat turbidity， while the persistent stage by congestion of ying （营） qi and retention of stasis-turbidity， and the stable stage by depletion of pectoral qi with lingering latent turbidity. In clinical practice， regulating qi and transforming turbidity is taken as the basic therapeutic principle， and the self-formulated Tiaoqi Huazhuo Decoction （调气化浊汤） is used as the basic prescription for staged treatment with flexible modifications. Specifically， the acute stage is treated with the method of dispersing wei qi and clearing heat-turbidity； the persistent stage is recommended to harmonize ying qi and purging stasis-turbidity； and the stable stage should focus on securing pectoral qi and venting latent turbidity.

OBJECTIVE To optimize the extraction process of polysaccharides from Abelmoschus manihot flowers， and to investigate the antioxidant， moisturizing and hygroscopicity activities of the polysaccharides.METHODS The optimal ratio of composite enzymes was determined by orthogonal experiments， and the extraction process of polysaccharides was optimized by single-factor experiments and Box-Behnken response surface methodology， and validated. The scavenging rates of 2，2′-azino-bis （3-ethylbenzothiazoline-6-sulfonic acid） （ABTS） free radicals， hydroxyl free radicals， and superoxide anion free radicals， as well as the total reducing ability of the polysaccharides were investigated by the ABTS method， Fenton-salicylic acid colorimetric method， pyrogallol autoxidation method， and potassium ferricyanide reduction method， respectively. The moisturizing and hygroscopicity activities of the polysaccharides were evaluated by the weighing method.RESULTS The optimal ratio of composite enzymes was 136 000 U of papain， 125 000 U of pectinase，and 5 000 U of cellulase. The optimal extraction process was as follows： enzymatic hydrolysis time of 2.8 h， enzymatic hydrolysis temperature of 57.50 ℃， pH of the sodium dihydrogen phosphate-disodium hydrogen phosphate buffer was 5.5， and solid-to-liquid ratio of 1∶40. The results of three experiments showed that the average polysaccharides extraction rate of 23.595%， with an RSD of 0.90%. The scavenging rates of ABTS free radicals， hydroxyl free radicals， superoxide anion free radicals， and total reducing ability were 81.759%， 87.110%， 54.244%， and 1.129 （the mass concentrations of polysaccharides were 10、 5、 10， and 10 mg/mL， respectively）， respectively. At relative humidity of 43% for 12 h， the moisturizing rate and hygroscopicity rate of polysaccharides were 99.387% and 100.478%， respectively； at relative humidity of 83% for 12 h， they were 99.764% and 100.455%， respectively.CONCLUSIONS The optimal extraction process of polysaccharides from A.manihot flowers based on the composite enzymes is stable and feasible； the polysaccharides obtained under this process have good antioxidant， moisturizing， and hygroscopicity activities.

[This corrects the article DOI: 10.1016/j.apsb.2022.05.019.].

Objective:To evaluate the efficacy of eculizumab in treating hematopoietic stem cell transplantation-associated thrombotic microangiopathy (TA-TMA) .Methods:This retrospective study included 11 patients who developed TA-TMA after allogeneic hematopoietic stem cell transplantation and subsequently received eculizumab treatment at Peking University People′s Hospital between June 2018 and May 2024. The incidence of TA-TMA, treatment details, and clinical outcomes were analyzed.Results:Among the 11 included patients [4 males, 7 females; median age: 29 years (range: 9-56) ], underlying diseases were severe aplastic anemia (SAA) in 5 patients, acute lymphoblastic leukemia (ALL) in 3 patients, and acute myeloid leukemia (AML) in 3 patients. The median time to TA-TMA diagnosis was 48 days post-transplantation (range: 4-213 days), and all patients met the diagnostic criteria for high-risk TA-TMA. The median interval from TA-TMA diagnosis to the initiation of eculizumab treatment was 12 days (range: 1-56 days). Patients received a median of 3 doses of eculizumab (range: 1-14). Ten of the 11 patients were assessed as having no response (NR) to eculizumab at the end of treatment or at death. One patient achieved a partial response (PR) but subsequently died after TA-TMA relapsed due to infection. At the last follow-up, all patients were either lost to follow-up or had died. The median follow-up duration was 88 days (range: 33-326 days), and the median time from TA-TMA diagnosis to the last follow-up was 31 days (range: 21-113 days) .Conclusion:Eculizumab demonstrated poor efficacy in this TA-TMA cohort. This might be attributable to the critical and complex condition of the patients, delayed initiation of eculizumab treatment, and insufficient dosage.

Objective:To evaluate the diagnostic value of targeted next-generation sequencing (tNGS) of throat swab samples for detecting community-acquired respiratory viruses (CARV) in patients with hematological diseases.Methods:Clinical and laboratory data from 64 episodes involving patients with hematological diseases and suspected infections—who underwent both pharyngeal swab tNGS and CARV polymerase chain reaction (PCR) testing concurrently—were retrospectively analyzed. The cases were drawn from the Department of Hematology, Peking University People’s Hospital, between September 2023 and April 2024. Concordance between tNGS and CARV PCR results, as well as the diagnostic performance of tNGS in detecting CARV, were evaluated.Results:Among the 64 episodes, 29 were clinically diagnosed with respiratory tract infections, including one case of cytomegalovirus pneumonia and 28 CARV-positive cases. The remaining 35 episodes involved patients with fever or respiratory symptoms attributed to other causes, including 14 with extrapulmonary infections and 21 with noninfectious etiologies. The median follow-up duration was 215.5 days (range: 7-271 days). PCR detected 24 strains of seven CARV types, whereas tNGS detected 25 strains of eight CARV types. Using PCR results as the reference standard, the sensitivity, specificity, positive predictive value, negative predictive value and accuracy of tNGS were 85.0%, 88.6%, 77.3%, 92.9%, and 87.5%, respectively. The two methods showed good concordance (Kappa=0.717, P<0.001) . Conclusion:Pharyngeal swab tNGS may serve as a viable alternative to PCR for diagnosing CARV infections in patients with hematological diseases.

Objective:To evaluate the efficacy of eculizumab in treating hematopoietic stem cell transplantation-associated thrombotic microangiopathy (TA-TMA) .Methods:This retrospective study included 11 patients who developed TA-TMA after allogeneic hematopoietic stem cell transplantation and subsequently received eculizumab treatment at Peking University People′s Hospital between June 2018 and May 2024. The incidence of TA-TMA, treatment details, and clinical outcomes were analyzed.Results:Among the 11 included patients [4 males, 7 females; median age: 29 years (range: 9-56) ], underlying diseases were severe aplastic anemia (SAA) in 5 patients, acute lymphoblastic leukemia (ALL) in 3 patients, and acute myeloid leukemia (AML) in 3 patients. The median time to TA-TMA diagnosis was 48 days post-transplantation (range: 4-213 days), and all patients met the diagnostic criteria for high-risk TA-TMA. The median interval from TA-TMA diagnosis to the initiation of eculizumab treatment was 12 days (range: 1-56 days). Patients received a median of 3 doses of eculizumab (range: 1-14). Ten of the 11 patients were assessed as having no response (NR) to eculizumab at the end of treatment or at death. One patient achieved a partial response (PR) but subsequently died after TA-TMA relapsed due to infection. At the last follow-up, all patients were either lost to follow-up or had died. The median follow-up duration was 88 days (range: 33-326 days), and the median time from TA-TMA diagnosis to the last follow-up was 31 days (range: 21-113 days) .Conclusion:Eculizumab demonstrated poor efficacy in this TA-TMA cohort. This might be attributable to the critical and complex condition of the patients, delayed initiation of eculizumab treatment, and insufficient dosage.

Objective:To evaluate the diagnostic value of targeted next-generation sequencing (tNGS) of throat swab samples for detecting community-acquired respiratory viruses (CARV) in patients with hematological diseases.Methods:Clinical and laboratory data from 64 episodes involving patients with hematological diseases and suspected infections—who underwent both pharyngeal swab tNGS and CARV polymerase chain reaction (PCR) testing concurrently—were retrospectively analyzed. The cases were drawn from the Department of Hematology, Peking University People’s Hospital, between September 2023 and April 2024. Concordance between tNGS and CARV PCR results, as well as the diagnostic performance of tNGS in detecting CARV, were evaluated.Results:Among the 64 episodes, 29 were clinically diagnosed with respiratory tract infections, including one case of cytomegalovirus pneumonia and 28 CARV-positive cases. The remaining 35 episodes involved patients with fever or respiratory symptoms attributed to other causes, including 14 with extrapulmonary infections and 21 with noninfectious etiologies. The median follow-up duration was 215.5 days (range: 7-271 days). PCR detected 24 strains of seven CARV types, whereas tNGS detected 25 strains of eight CARV types. Using PCR results as the reference standard, the sensitivity, specificity, positive predictive value, negative predictive value and accuracy of tNGS were 85.0%, 88.6%, 77.3%, 92.9%, and 87.5%, respectively. The two methods showed good concordance (Kappa=0.717, P<0.001) . Conclusion:Pharyngeal swab tNGS may serve as a viable alternative to PCR for diagnosing CARV infections in patients with hematological diseases.

Objective:To conjecture the correlation between fetal hydrothorax(FHT)and pregnancy outcome through the analysis of 95 fetuses with hydrothorax and their mothers.Methods:In this case series study, 95 FHT patients admitted to the Third Affiliated Hospital of Zhengzhou University from January 2016 to October 2022 were retrospectively analyzed.According to the pregnancy outcome, these patients were divided into the induced labor group (13 patients) and the delivery group (82 patients). General data and genetic examinations of patients in the induced labor group were analyzed to explore their pathogenesis and genetics.According to the neonatal Apgar score, patients in the delivery group were divided into the normal group and the asphyxia group.Fifteen indicators including the maternal age, gestational week at first diagnosis, maternal complications, FHT location, FHT severity, FHT outcome during pregnancy, fetal ascites, hydrops fetalis, other abnormal fetal structures, amniotic fluid volume, intrauterine treatment, gestational week of delivery, mode of delivery, sex of the newborn, and newborn birth weight in the 2 groups were comparatively analyzed by the chi-square test.With the neonatal Apgar score as the dependent variable, variables that had statistical significance during the univariate analysis were included in the regression analysis, and a multivariate binary Logistic regression equation was established to explore the factors affecting the pregnancy outcome.Results:There were significant differences in the FHT outcome during pregnancy (16/22/13 cases vs.2/18/11 cases, χ2=6.994, P=0.030), FHT severity (27/24 cases vs.9/22 cases, χ2=4.475, P=0.034), hydrops fetalis (14/37 cases vs.23/8 cases, χ2=17.012, P=0.001), amniotic fluid volume (21/30 cases vs.24/7 cases, χ2=10.228, P=0.001), intrauterine treatment (19/32 cases vs.2/29 cases, χ2=9.603, P=0.002), gestational week of delivery[(38.15±2.05) weeks vs.(34.83±3.14) weeks, t=5.748, P=0.001], and newborn birth weight[(3 325.00±637.41) g vs.(2 714.58±837.99) g, t=3.727, P=0.001]between the normal and asphyxia groups(all P<0.05). Among them, hydrops fetalis ( OR=7.070, P=0.020) and severe FHT ( OR=6.927, P=0.043) were risk factors for neonatal Apgar scores.Intrauterine treatment ( OR=0.062, P=0.027) and gestational week of delivery( OR=0.577, P=0.022) could be used as a protective factor for neonatal Apgar scores. Conclusions:Diagnosis of FHT during the early gestational weeks and multiple fetal structural abnormalities, especially hydrops fetalis, have higher probabilities of abnormal genetic examinations, and the fetal prognosis was poor.It is recommended to carry out chromosomal karyo type and microarray tests, as well as whole exome sequencing and detection of genetic syndromes(if necessary), to avoid unnecessary fetal treatment and improve the survival rate of perinatal infants after intrauterine treatment.The poor prognosis is related to hydrops fetalis and severe FHT; however, timely intrauterine treatment and prolonging pregnancy can improve the pregnancy outcome and increase the survival rate of perinatal infants.

Objective Ultrasound categories of Breast Imaging Reporting and Data System(BI-RADS)are an important outcome used in the evaluation of the characteristics of breast lesions.In this study,we aim to explore the potential influencing factors that may cause the progression of BI-RADS categories.Methods Based on the cervical cancer and breast cancer screening cohort in Shuangliu District,Chengdu from 2015 to 2021,we collected ultrasound and questionnaire results.Progression was defined as changing from BI-RADS category 1 or 2 to 3 or above,or from the initial screening result of BI-RADS 3 or above to a higher category in subsequent ultrasound screenings.The Cox proportional hazards regression model was used to explore potential influencing factors for BI-RADS classification progression.A restricted cubic spline was used to explore the nonlinear association between the variables and the risk of progression.Results A total of 7 069 participants were included in the analysis and followed up for 23 580 person-years.Compared with other age groups,the proportion of participants of BI-RADS category 3 and above was highest in the 40-44 age group(24.83％).Cox regression analysis showed that postmenopausal women had a lower risk of BI-RADS progression than premenopausal women did(hazard ratio[HR]:0.65,95％confidence interval[CI]:0.44-0.97).In postmenopausal women,late menopause(HR=1.16,95％CI:1.03-1.30)was identified as a risk factor for BI-RADS progression.Obesity in premenopausal women was negatively associated with the risk of progression(HR=0.43,95％CI:0.21-0.88).Conclusion The risk factors for progression in ultrasound BI-RADS categories overlap with the risk factors for breast cancer.A risk score based on the high-risk factors for BI-RADS progression can be constructed to optimize screening programs for high-risk populations and improve the efficiency of breast cancer screening.

Objective:To further improve the understanding of paroxysmal nocturnal hemoglobinuria (PNH), we retrospectively analyzed and summarized the clinical characteristics, treatment status, and survival status of patients with PNH in Zhejiang Province.Methods:This study included 289 patients with PNH who visited 20 hospitals in Zhejiang Province. Their clinical characteristics, comorbidity, laboratory test results, and medications were analyzed and summarized.Results:Among the 289 patients with PNH, 148 males and 141 females, with a median onset age of 45 (16-87) years and a peak onset age of 20-49 years (57.8% ). The median lactic dehydrogenase (LDH) level was 1 142 (604-1 925) U/L. Classified by type, 70.9% (166/234) were classical, 24.4% (57/234) were PNH/bone marrow failure (BMF), and 4.7% (11/234) were subclinical. The main clinical manifestations included fatigue or weakness (80.8%, 235/289), dizziness (73.4%, 212/289), darkened urine color (66.2%, 179/272), and jaundice (46.2%, 126/270). Common comorbidities were hemoglobinuria (58.7% ), renal dysfunction (17.6% ), and thrombosis (15.0% ). Moreover, 82.3% of the patients received glucocorticoid therapy, 70.9% required blood transfusion, 30.7% used immunosuppressive agents, 13.8% received anticoagulant therapy, and 6.3% received allogeneic hematopoietic stem cell transplantation. The 10-year overall survival (OS) rate was 84.4% (95% CI 78.0% -91.3% ) . Conclusion:Patients with PNH are more common in young and middle-aged people, with a similar incidence rate between men and women. Common clinical manifestations include fatigue, hemoglobinuria, jaundice, renal dysfunction, and recurrent thrombosis. The 10-year OS of this group is similar to reports from other centers in China.