Pneumocystis jirovecii pneumonia (PJP) is an opportunistic pulmonary infection that commonly occurs in immunocompromised children. We report a case of infantile leukemia complicated by PJP and review the relevant literature. A summary and analysis of 10 infantile leukemia patients with PJP infection (9 cases reported in the literature and 1 case from our center) showed that PJP mostly occurred in the early stages of chemotherapy (80%, 8/10). The main clinical manifestations were dyspnea (100%, 10/10) and hypoxemia (50%, 5/10), while pulmonary imaging findings lacked specificity. In most cases (50%, 5/10), diagnosis was established by identifying pathogens in bronchoalveolar lavage fluid under microscopy. In our case, diagnosis was confirmed using targeted next-generation sequencing (tNGS) of bronchoalveolar lavage fluid. Treatment with intravenous sulfamethoxazole complex was administered in 8 patients, all of whom eventually recovered. PJP may occur in the early stages of chemotherapy for infantile leukemia, thus early prevention is necessary. tNGS facilitates early diagnosis of PJP, and sulfamethoxazole complex remains an effective therapeutic option.
Humans ; Infant ; Bronchoalveolar Lavage Fluid/microbiology* ; Immunocompromised Host ; Leukemia/complications* ; Pneumocystis carinii/isolation & purification* ; Pneumonia, Pneumocystis/diagnosis* ; Trimethoprim, Sulfamethoxazole Drug Combination/therapeutic use*

Objective:To observe the efficacy and safety of adrenocorticotropic hormone (ACTH) therapy in children with steroid dependent nephrotic syndrome (SDNS)/frequently relapsed nephrotic syndrome (FRNS).Methods:The clinical data of children with SDNS/FRNS who received treatment with prednisone acetate tablets were retrospectively collected from June 2019 to June 2023 in the Nephrology Department of Xi′an Children′s Hospital. The children were divided into glucocorticoid+ACTH group and glucocorticoid group, according to whether ACTH was used or not. The differences in cortisol, total cholesterol and 24 hour urinary protein quantity between 2 groups of children at baseline and follow-up endpoints were compared, and the effectiveness (the proportion of no recurrence and discontinuation of glucocorticoid) and occurrence of adverse reactions were evaluated.Results:A total of 39 patients with SDNS/FRNS were included in this study, with 21 cases in the glucocorticoid+ACTH group and 18 cases in the glucocorticoid group. Among the 39 children, there were 33 cases of SDNS and 6 cases of FRNS, respectively. The proportion of baseline low cortisol levels was 76.9% (30/39). The proportion of cortisol levels returning to normal after ACTH treatment in the glucocorticoid+ACTH group was 76.2% (16/21). The baseline and follow-up endpoint for cortisol levels in the glucocorticoid+ACTH group were 28.0(19.8, 51.5) μg/L and 79.9(58.9, 113.0) μg/L, respectively. The baseline and follow-up endpoint for cortisol levels in the glucocorticoid group were 21.0(15.8, 37.4) μg/L and 25.3(18.2, 51.4) μg/L, respectively. In the 2 groups of cortisol levels, there was statistically significant difference in the interaction effect between time and group ( Wald χ2=11.595, P=0.001), there was a statistically significant difference at the follow-up endpoint between the 2 groups ( Wald χ2=19.462, P<0.001), and the difference was statistically significant in the time effect of the glucocorticoid+ACTH group ( Wald χ2=21.100, P<0.001). The baseline and follow-up endpoint for total cholesterol in the glucocorticoid+ACTH group were 4.95(4.23, 5.26) mmol/L and 4.38(4.04, 5.24) mmol/L, respectively. The baseline and follow-up endpoint for total cholesterol in the glucocorticoid group were 4.80 (4.17, 5.28) mmol/L and 5.74 (5.04, 6.88) mmol/L, respectively. In the 2 groups of total cholesterol, there was statistically significant difference in the interaction effect between time and group ( Wald χ 2=9.842, P=0.002), there was statistically significant difference at the follow-up endpoint between the 2 groups ( Wald χ 2=12.187, P<0.001), the difference was statistically significant between the 2 groups in the time effect at baseline and the follow-up endpoint (glucocorticoid+ACTH group: Wald χ 2=6.488, glucocorticoid group: Wald χ2=7.112; all P<0.05). The baseline and follow-up endpoint for 24 hour urinary protein quantity in the glucocorticoid+ACTH group were 115 (105, 128) mg/d and 121 (113, 128) mg/d, respectively. The baseline and follow-up endpoint for 24 hour urinary protein quantity in the glucocorticoid group were 118 (113, 125) mg/d and 138 (119, 2 100) mg/d, respectively. In the 2 groups of 24 hour urinary protein quantity, there was statistically significant difference in the interaction effect between time and group ( Wald χ2=7.743, P=0.005), there was statistically significant difference at the follow-up endpoint between the 2 groups ( Wald χ2=7.779, P=0.005), and the difference was statistically significant in the time effect of the glucocorticoid group ( Wald χ2=13.331, P<0.001). The proportion of no recurrence (17/21) and discontinuation of oral glucocorticoid (16/21) in the glucocorticoid+ACTH group were higher than those in the glucocorticoid group (the proportion were both 6/18), and the differences between the 2 groups were statistically significant (the chi square values were 9.084 and 7.240, respectively; all P<0.01). No adverse reactions occurred in the glucocorticoid group. The incidence of adverse reactions in the glucocorticoid+ACTH group was 14.3% (3/21), of which 2 cases developed generalized urticaria and 1 case developed hypertension. Conclusions:ACTH has a good efficacy and safety in children with SDNS/FRNS. The results of this study need to be further validated by increasing the sample size and conducting multicenter studies.

Objective:To observe the efficacy and safety of adrenocorticotropic hormone (ACTH) therapy in children with steroid dependent nephrotic syndrome (SDNS)/frequently relapsed nephrotic syndrome (FRNS).Methods:The clinical data of children with SDNS/FRNS who received treatment with prednisone acetate tablets were retrospectively collected from June 2019 to June 2023 in the Nephrology Department of Xi′an Children′s Hospital. The children were divided into glucocorticoid+ACTH group and glucocorticoid group, according to whether ACTH was used or not. The differences in cortisol, total cholesterol and 24 hour urinary protein quantity between 2 groups of children at baseline and follow-up endpoints were compared, and the effectiveness (the proportion of no recurrence and discontinuation of glucocorticoid) and occurrence of adverse reactions were evaluated.Results:A total of 39 patients with SDNS/FRNS were included in this study, with 21 cases in the glucocorticoid+ACTH group and 18 cases in the glucocorticoid group. Among the 39 children, there were 33 cases of SDNS and 6 cases of FRNS, respectively. The proportion of baseline low cortisol levels was 76.9% (30/39). The proportion of cortisol levels returning to normal after ACTH treatment in the glucocorticoid+ACTH group was 76.2% (16/21). The baseline and follow-up endpoint for cortisol levels in the glucocorticoid+ACTH group were 28.0(19.8, 51.5) μg/L and 79.9(58.9, 113.0) μg/L, respectively. The baseline and follow-up endpoint for cortisol levels in the glucocorticoid group were 21.0(15.8, 37.4) μg/L and 25.3(18.2, 51.4) μg/L, respectively. In the 2 groups of cortisol levels, there was statistically significant difference in the interaction effect between time and group ( Wald χ2=11.595, P=0.001), there was a statistically significant difference at the follow-up endpoint between the 2 groups ( Wald χ2=19.462, P<0.001), and the difference was statistically significant in the time effect of the glucocorticoid+ACTH group ( Wald χ2=21.100, P<0.001). The baseline and follow-up endpoint for total cholesterol in the glucocorticoid+ACTH group were 4.95(4.23, 5.26) mmol/L and 4.38(4.04, 5.24) mmol/L, respectively. The baseline and follow-up endpoint for total cholesterol in the glucocorticoid group were 4.80 (4.17, 5.28) mmol/L and 5.74 (5.04, 6.88) mmol/L, respectively. In the 2 groups of total cholesterol, there was statistically significant difference in the interaction effect between time and group ( Wald χ 2=9.842, P=0.002), there was statistically significant difference at the follow-up endpoint between the 2 groups ( Wald χ 2=12.187, P<0.001), the difference was statistically significant between the 2 groups in the time effect at baseline and the follow-up endpoint (glucocorticoid+ACTH group: Wald χ 2=6.488, glucocorticoid group: Wald χ2=7.112; all P<0.05). The baseline and follow-up endpoint for 24 hour urinary protein quantity in the glucocorticoid+ACTH group were 115 (105, 128) mg/d and 121 (113, 128) mg/d, respectively. The baseline and follow-up endpoint for 24 hour urinary protein quantity in the glucocorticoid group were 118 (113, 125) mg/d and 138 (119, 2 100) mg/d, respectively. In the 2 groups of 24 hour urinary protein quantity, there was statistically significant difference in the interaction effect between time and group ( Wald χ2=7.743, P=0.005), there was statistically significant difference at the follow-up endpoint between the 2 groups ( Wald χ2=7.779, P=0.005), and the difference was statistically significant in the time effect of the glucocorticoid group ( Wald χ2=13.331, P<0.001). The proportion of no recurrence (17/21) and discontinuation of oral glucocorticoid (16/21) in the glucocorticoid+ACTH group were higher than those in the glucocorticoid group (the proportion were both 6/18), and the differences between the 2 groups were statistically significant (the chi square values were 9.084 and 7.240, respectively; all P<0.01). No adverse reactions occurred in the glucocorticoid group. The incidence of adverse reactions in the glucocorticoid+ACTH group was 14.3% (3/21), of which 2 cases developed generalized urticaria and 1 case developed hypertension. Conclusions:ACTH has a good efficacy and safety in children with SDNS/FRNS. The results of this study need to be further validated by increasing the sample size and conducting multicenter studies.

Objective To detect the expressions of intercellular adhesion molecule-1(ICAM-1)and nu-clear factor(NF)-κB in hepatic tissues of the patients with chronic hepatitis B,and to analyze their correlation with the hepatic inflammatory activity and fibrosis degree.Methods The liver biopsy specimens from 66 pa-tients with hepatitis B and 10 non-hepatopathic controls were selected,and immunohistochemistry and in situ hybridization were used to detect ICAM-1 and NF-κB expression levels in different liver tissues.Results The positive rate of ICAM-1 and NF-κB expression in liver tissues of the patients with chronic hepatitis B was higher than that in normal liver tissues,and the difference was statistically significant(P＜0.05).The expres-sion of ICAM-1 and NF-κB in the patients with hepatitis B was positively correlated with the inflammatory ac-tivity and fibrosis degree(r=0.493,0.496,P＜0.01;r=0.580,0.519,P＜0.01).Conclusion ICAM-1 and NF-κB in the patients with chronic hepatitis B are highly expressed,which is useful in judging the hepatic in-flammatory activity and fibrosis degree.

BACKGROUND:Endothelial injury is one of the causes of cardiovascular diseases.Human induced pluripotent stem cells are easy to obtain,have strong differentiation ability,and have less exclusiveness,and their endothelial differentiated cells can be used as ideal cells for cardiovascular disease research. OBJECTIVE:To investigate the effect and mechanism of calycosin on endothelial differentiation of human induced pluripotent stem cells and to provide technical support for microvascular regeneration. METHODS:Human induced pluripotent stem cells were divided into control group and calycosin group(1.25,2.5 μg/mL),and growth factors were added to induce single-layer endothelial differentiation.After the induction of differentiation for 8 days,the positive rate of endothelial cell marker CD144 was detected by flow cytometry.Fluorescent expressions of CD144 and CD31 were detected by the immunofluorescence method.Lentivirus RNAi GFP puromycin was used to silence human-induced pluripotent stem cell Piezo1 mRNA followed by endothelial directed differentiation.After 8 days of differentiation,the positive rate of CD144 in differentiated cells was detected by flow cytometry.The mRNA expression levels of CD144,Piezo1 and MEK were detected by qPCR. RESULTS AND CONCLUSION:(1)Compared with the control group,the positive rate of CD144 was significantly increased in the 1.25 and 2.5 μg/mL calycosin groups(P<0.05).The expressions of CD144,Piezo1,and MEK mRNA were increased in the 2.5 μg/mL calycosin group(P<0.05).The fluorescence expressions of CD144(P<0.01)and CD31(P<0.001)were significantly increased in the 2.5 μg/mL calycosin group.(2)Compared with the shNT group,CD144 positive rate and CD144,Piezo1,MEK mRNA expressions were significantly increased in the shNT + calycosin 1.25,2.5 μg/mL groups(P<0.05).Compared with the shPiezo1 group,the positive rate of CD144 and mRNA expressions of CD144,Piezo1 and MEK had no significant changes in the shPiezo1+calycosin 1.25,2.5 μg/mL groups(P>0.05).(3)It is concluded that 2.5 μg/mL calycosin promotes the differentiation of human-induced pluripotent stem cells into endothelial lineages.Calycosin promotes the downstream MEK expression,thereby promoting the endothelial differentiation of human induced pluripotent stem cells by targeting the expression level of Piezo1.

Objective To investigate the diagnostic value of immunophenotype in distinguishing acute promyelocytic leukemia(APL)from HLA-DR negative acute myeloid leukemia(AML)using flow cytometry.Methods A retrospective observational study was con-ducted including 42 APL patients and 28 newly diagnosed or relapsed HLA-DR negative AML patients admitted to our hospital from 2014 to 2024.Immunophenotype analysis was performed on bone marrow aspirate samples using flow cytometry.The positive expression rates of CD64,MPO,CD7,CD11c,CD9,CD123 and other antigens were compared between the two groups using the Chi-square test.The diagnostic efficiency of the CD9/123 and CD64+MPO+CD7 CD11c-models for APL was evaluated using receiver operating charac-teristic(ROC)curves.Results The HLA-DR negative AML group exhibited significantly lower positive rates of CD64,CD9 and MPO(P＜0.05),and higher positive rates of CD11c and CD7(P＜0.05)compared to APL group.The CD64+MPO+CD7-CD11c-model had an area under the curve(AUCROC)of 0.859,sensitivity of 93.8％and specificity of 75.0％for distinguishing APL.The CD9/CD123 expression pattern had AUCROC of 0.919,sensitivity of 83.3％and specificity of 84.0％for APL diagnosis.The combined CD9/123 and CD64+MPO+CD7-CD11c-model had AUCROC of 0.955,sensitivity of 83.3％and specificity of 100％.Conclusion The combined CD9/123 and CD64+MPO+CD7-CD11c-expression pattern may serve as a helpful tool for differentiating APL from HLA-DR negative AML.

Objective:To compare the short-term efficacy of drug-coated balloon and common balloon in the treatment of with below-the-ankle arteriosclerosis obliterans (ASO).Methods:The clinical and follow-up data of 115 patients (118 limbs) with lower extremity ASO of Rutherford 4-6 admitted to the Department of Peripheral Vascular Diseases, Xiyuan Hospital, China Academy of Chinese Medical Sciences from Jun 2021 to Jun 2023 were retrospectively analyzed.Results:Patients were divided into group of 52 limbs using paclitaxel-coated balloon and group of 66 limbs using common balloon. During the 6-month follow-up period, The total effective rate of wound surface was 98.1% in the drug-coated balloon group and 92.4% in the common balloon group. The difference was statistically significant ( P<0.05); the increase of ankle-brachial index (ABI) and toe-brachial index (TBI) in the drug-coated balloon group were significantly better than common balloon group, and the difference was statistically significant ( P<0.01). No major adverse events occurred in the two groups during the 6-month follow-up period. Compared with the common balloon group, the drug-coated balloon group had a higher limb salvage rate (100.0% vs. 98.5% ) and a lower target vessel reconstruction rate (7.7% vs.18.2%), though the difference was not statistically significant ( P>0.05). Conclusion:Compared with the common balloon, the drug-coated balloon is more effective in the treatment of patients with wound healing rate and vascular patency rate in Rutherford 4-6 grade below-the-ankle artery lesions.

Hypoxia is associated with the occurrence and development of many diseases in clinical settings.Cell hypoxia not only serves as a vital marker for disease advancement,but also plays a pivotal role in exacerbating the disease process,and improving tissue hypoxia may thus provide new strategies for the treatment of related diseases.Further investigation of these diseases at the cellular and molecular levels requires the establishment of a cellular hypoxia model.Current extensively employed hypoxic cell models can be categorized primarily into three types:chemical hypoxia,physical hypoxia,and glucose deprivation hypoxia models.This article reviews the various types of hypoxic cell models and scrutinizes their applications and limitations in disease research.

Hypoxia is associated with the occurrence and development of many diseases in clinical settings.Cell hypoxia not only serves as a vital marker for disease advancement,but also plays a pivotal role in exacerbating the disease process,and improving tissue hypoxia may thus provide new strategies for the treatment of related diseases.Further investigation of these diseases at the cellular and molecular levels requires the establishment of a cellular hypoxia model.Current extensively employed hypoxic cell models can be categorized primarily into three types:chemical hypoxia,physical hypoxia,and glucose deprivation hypoxia models.This article reviews the various types of hypoxic cell models and scrutinizes their applications and limitations in disease research.

Objective:To evaluate the efficacy and safety of rituximab(RTX) as remission-mainten-ance therapy in antineutrophil cytoplasmic antibody(ANCA) associated vasculitis(AAV).Methods:Patients with AAV, including granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA), treated with rituximab (RTX) in Peking Union Medical College Hospital during September 2005 to June 2021 were included into this study. Clinical data, relapse rate, time of first relapse and adverse events were collected and analyzed. The cumulative relapse rate was calculated by Kaplan-Meier, t test, and Man-Whithey U test and chi-square were used to compare differences between two groups. Results:① Thirty-nine AAV patients were enrolled, including 36 GPA and 3 MPA. During the 20(3, 104) months follow-up, 59.0%(23/39) patients had suffered relapses. The time for first relapse was 11(3, 42) months after remission. ② There were no difference in the relapse rate [60.0%(18/30) vs 55.6%(5/9), χ2=0.06, P=1.000), the time of first relapse [15(3, 42) vs 10(9, 30), Z=0.45, P=0.678], CD19 + B [23.5 (5, 148) cell/μl vs 3(2, 15) cell/μl, Z=0.57, P=0.605] and serum IgG [7.09(5.13, 13.90) g/L vs 9.72(5.32, 12.0) g/L, Z=0.36, P=0.770] between standard dose and low-dose groups. The rate of major relapse-free was significantly less in patients treated with standard dose than patients with reduced dose of RTX {87.1%[95% CI(73.4%, 100.8R%)] vs 64.3%[95% CI(23.1%, 105.4%)], χ2=7.59, P=0.006}. ③ There were no difference in relapse rate [50.0%(3/6) vs 60.6%(20/33), χ2=0.24, P=0.674], time of first relapse [23(6, 25) vs 11(3, 42), Z=0.05, P=0.982], CD19 + B[35(15, 50) cell/μl vs 10(0, 148) cell/μl, Z=0.95, P=0.382] and serum IgG[6.70(5.91, 7.49) g/L vs 7.69(3.78, 13.90) g/L, Z=0.48, P=0.700] between the fixed interval dosage and the on-demand dosage groups. There was no difference in the rate of major relapse-free between the two groups (100% vs 77.8%, χ2=1.79, P=0.181). ④ The incidence of infusion reaction was 5.1%(2/39) and infection was 20.5%(8/39). Serum IgG level was 4.37(3.78, 13.4) g/L at infection. There was no difference in safety between the standard and low-dose groups or between fixed interval and on-demand dosage groups ( P>0.05). Conclusion:There is no significant difference in relapse rate bet-ween the standard RTX dose and low-dose RTX induction therapy group, but the major relapse rate is sign-ificantly reduced in the standard dose RTX therapy. The relapse rate of fixed intervals dosage group is similar to that of on-demand dosage group. The safety profile of the standard dose and low-dose induction therapy groups or fixed intervals and on-demand dosage groups is similiar.