OBJECTIVE: To investigate the predictive value of endothelial activation and stress index (EASIX) for the prognosis of patients with mantle cell lymphoma (MCL). METHODS: A retrospective analysis was conducted to assess prognosis and compare the clinical features of patients diagnosed with MCL who were admitted to the Affiliated Hospital of Xuzhou Medical University from January 2010 to June 2023, had therapeutic indications and received standard treatment. RESULTS: A total of 66 patients were included and divided into high EASIX group and low EASIX group, according to a cutoff value of 0.97 determined by the receiver operating characteristic (ROC) curve. Multivariate Cox regression analysis showed that prealbumin <0.2 g/L, high EASIX, and ECOG PS score ≥2 were independent risk factors influencing overall survival (OS) in MCL patients. The median OS of patients in the high and low EASIX group was 13.0 and 37.5 months, and the median progression-free survival was 8.8 and 26.0 months, respectively. The proportions of patients with ECOG PS score ≥2 and prealbumin <0.2 g/L at onset significantly increased in the high EASIX group compared to those in the low EASIX group. CONCLUSION At the time of initial diagnosis, EASIX can serve as an independent prognostic indicator impacting OS in patients with MCL. Furthermore, patients in the high EASIX group experience a poorer prognosis and shorter survival duration compared with those in the low EASIX group.
Humans ; Lymphoma, Mantle-Cell/pathology* ; Prognosis ; Retrospective Studies ; Male ; Female ; Middle Aged ; Aged ; ROC Curve

Objective: To study the influence of steroid withdrawal protection strategy on height growth in pediatric patients after kidney transplantation. Methods: The prospective cohort study enrolled 40 stage 5 chronic kidney disease children receiving kidney transplantation from July 2017 to September 2022 at Guangzhou Women and Children's Medical Center. Based on the primary preoperative disease, patients with immune abnormality-associated glomerular diseases or unknown causes were assigned to the steroid maintenance group, in which patients received steroid tapering within 3 months after surgery to a maintenance dose of 2.5 to 5.0 mg/d. While patients with hereditary kidney disease or congenital urinary malformations were assigned to the steroid withdrawal group, in which patients had steroids tapered off within 3 months. The characteristics of height catch-up growth and clinical data were compared between the 2 groups at baseline, 6, 12, 18 and 24 months after kidney transplantation. T-test, repeated measurement of variance analysis, Mann-Whitney U test, and Fisher exact test were used for the comparison between the 2 groups. Results: Among the 40 children, 17 were males, 23 were females, 25 were in the steroid withdraw group ((7.8±2.8) years old when receiving kidney transplantation) and 15 cases were in the steroid maintenance group ((7.6±3.5) years old when receiving kidney transplantation). The study population was followed up for (26±12) months. The total dose per unit body weight of steroids in the steroid withdrawal group was lower than that in the steroid maintenance group ((0.13±0.06) vs. (0.36±0.19) mg/(kg·d), t=5.83, P<0.001). The height catch-up rate (ΔHtSDS) in the first year after kidney transplantation in the steroid withdraw and steroid maintenance groups was 1.0 (0.7, 1.4) and 0.4 (0.1, 1.0), respectively; in the second year, the ΔHtSDS in the steroid withdraw group was significantly higher than that in the steroid maintenance group (1.1 (0.2, 1.7) vs. 0.3 (0, 0.8), U=28.00, P=0.039). The HtSDS in the steroid withdrawal group at the five follow-up time points was -2.5±0.8, -2.0±0.8, -1.5±0.8, -1.3±0.9 and -0.5±0.3, respectively, while in the steroid maintenance was -2.4±1.3, -2.2±1.1, -2.0±1.0, -1.8±1.0 and -1.6±1.0, respectively. There were statistically significant differences in HtSDS at different follow-up time points in both 2 groups (F=19.81, P<0.01), but no statistical differences in overall impact between the 2 groups (F=1.13, P=0.204). The steroid treatment was interaction with the increase of follow-up time (F=3.62, P=0.009). At the 24^th month after transplantation, the HtSDS in the steroid withdrawal group was significantly higher than that in the steroid maintenance group (P=0.047). Six patients in the steroid withdrawal group experienced antibody-mediated immune rejection (AMR), while 3 did in the steroid maintenance group. Moreover, there was no significant difference in AMR between the two groups (χ²=0.06, P=0.814). Conclusion: The steroid withdrawal protection strategy favors the height catch-up growth in pediatric patients after kidney transplantation and does not increase the risk of postoperative antibody-mediated immune rejection.
Male ; Humans ; Child ; Female ; Child, Preschool ; Kidney Transplantation ; Prospective Studies ; Steroids/therapeutic use* ; Antibodies ; Body Weight

OBJECTIVES: To investigate the characteristics of auditory processing (AP) in preschool children with attention deficit hyperactivity disorder (ADHD) using Preschool Auditory Processing Assessment Scale (hereafter referred to as "auditory processing scale"). METHODS: A total of 41 children with ADHD and 41 typically developing (TD) children were assessed using the auditory processing scale, SNAP-IV rating scale, and Conners' Kiddie Continuous Performance Test (K-CPT). The auditory processing scale score was compared between the TD and ADHD groups. The correlations of the score with SNAP-IV and K-CPT scores were assessed. RESULTS: Compared with the TD group, the ADHD group had significantly higher total score of the auditory processing scale and scores of all dimensions except visual attention (P<0.05). In the children with ADHD, the attention deficit dimension score of the SNAP-IV rating scale was positively correlated with the total score of the auditory processing scale (r_s30=0.531, P<0.05; r_s27=0.627, P<0.05) as well as the scores of its subdimensions, including auditory decoding (r_s=0.628, P<0.05), auditory attention (r_s=0.492, P<0.05), and communication (r_s=0.399, P<0.05). The hyperactivity-impulsivity dimension score of the SNAP-IV rating scale was positively correlated with the hyperactivity-impulsivity dimension score of the auditory processing scale (r_s=0.429, P<0.05). In the children with ADHD, the attention deficit dimension score of the K-CPT was positively correlated with the total score (r_s30=0.574, P<0.05; r_s27=0.485, P<0.05) and the hyperactivity-impulsivity dimension score (r_s=0.602, P<0.05) of the auditory processing scale. CONCLUSIONS Preschool children with ADHD have the risk of AP abnormalities, and the auditory processing scale should be used early for the screening and evaluation of AP abnormalities in children.
Child, Preschool ; Humans ; Attention Deficit Disorder with Hyperactivity ; Schools ; Auditory Perception

Objective:To understand the dynamics of circulating Tfh (cTfh) and B cells, and their relationship in individuals with acute (AHI) and chronic HIV infection (CHI).Methods:HIV infected subjects and HIV negative healthy controllers (HC) were enrolled from the men who have sex with men(MSM) high-risk cohort at Beijing Youan hospital. Multicolor flow cytometry was used for the frequency and absolute number analysis of cTfh cell and B-cell subsets with AHI, CHI, and HC.Results:Compared to HC, AHI resulted in an increase in cTfh cell levels and persisted into CHI, however, there was no significant difference between AHI and CHI (AHI vs HC: 2.09±1.48 vs 0.26±0.38, t=5.25, P<0.001; CHI vs HC: 2.26±1.35 vs 0.26±0.38, t=6.25, P<0.001; AHI vs CHI: 2.09±1.48 vs 2.26±1.35, t=0.40, P=0.449). Among B cell subsets, activated memory (AM), tissue-like memory (TLM), and plasmablast (PB) cell levels increased [AM(AHI vs HC: 3.59±1.77 vs 0.83±0.44, t=6.65, P<0.001; CHI vs HC: 3.99±2.49 vs 0.83±0.44, t=5.46, P<0.001); TLM(AHI vs HC: 11.05±4.96 vs 1.30±0.93, t=8.45, P<0.001; CHI vs HC: 13.91±6.59 vs 1.30±0.93, t=8.28, P<0.001); PB( AHI vs HC: 3.01±2.50 vs 0.43±0.26, t=4.47, P<0.001; CHI vs HC: 1.88±1.57 vs 0.43±0.26, t=3.97, P<0.001)], whereas resting memory (RM) and na?ve mature (NM) cell levels decreased in both AHI and CHI [RM(AHI vs HC: 20.06±9.74 vs 25.43±10.91, t=1.70, P=0.040; CHI vs HC: 15.70±8.47 vs 25.43±10.91, t=3.29, P=0.003); NM(AHI vs HC: 55.71±13.88 vs 66.26±11.71, t=2.90, P=0.004; CHI vs HC: 58.33±14.47 vs 66.26±11.71, t=1.94, P=0.006)]. The levels of cTfh cells were positively correlated with those of AM ( r=0.67, P<0.001), classical memory (CM) ( r=0.59, P=0.001), RM ( r=0.47, P=0.010), and PB ( r=0.65, P<0.001), and negatively correlated with those of NM B cells in AHI ( r=-0.55, P=0.003). However, cTfh cells did not show any relationship with B cell subsets except for the positive correlation with PB ( r=0.56, P=0.003) in CHI. Conclusions:HIV infection drives the expansion of cTfh cells, which may in turn leads to perturbations of B cell differentiation during AHI stage.

Objective:To explore the clinical efficacy of disease-modifying drug nusinersen on children with spinal muscular atrophy.Methods:The baseline and longitudinal clinical data of 15 children who were treated with nusinersen in the Children′s Hospital, Zhejiang University School of Medicine from October 2019 to October 2021 were retrospectively collected. The general data (gender, age, genotype, and clinical classification, etc.), motor function, nutritional status, scoliosis and respiratory function were analyzed. Wilcoxon rank-sum test was used for comparing multi-system conditions before and after treatment.Results:The age of 15 cases (7 males, 8 females) was 6.8 (2.8, 8.3) years, with 2 cases of type 1, 6 cases of type 2, and 7 cases of type 3 respectively, and the course of disease was 55.0 (21.0, 69.0) months. After 9.0 (9.0, 24.0) months of treatment, the motor function scale evaluations of the Hammersmith neurological examination section 2 (13.0 (7.0, 23.0) vs. 18.0 (10.0, 25.0) scores, Z=-2.67, P=0.018) of 15 children, the Hammersmith functional motor scale expanded (38.0 (18.5, 45.5) vs. 42.0 (23.0, 51.0) scores, Z=-2.38, P=0.018), and the revised upper limb module (27.0 (19.5, 32.0) vs. 33.0 (22.5, 35.5) scores, Z=-2.52, P=0.012) of children with type 2 and 3 had significantly improved. Thirteen patients achieved clinically significant motor function improvement, and 2 of them had kept stable scale scores. Subjective reports also indicated that the muscle strength and daily exercise ability of these children improved after treatment, and no serious adverse reactions were reported. Supplemented by the multi-disciplinary team management, the levels of some indicators such as Cobbs angle of scoliosis and forced vital capacity all had significantly improved (all P<0.05). Conclusions:Nusinersen can improve the motor function of patients with 5q spinal muscular atrophy, which is also proved safe to be used in children. The drug treatment supplemented by the multi-disciplinary team management is helpful to improve the multi-system function of the children with spinal muscular atrophy.

Objective:To investigate the value of generative adversarial networks-based PET image reconstruction in improving the quality of low-dose 18F-FDG PET images and lesion detection in pediatric patients. Methods:Retrospective analysis of 61 PET images of children (38 males, 23 females, age (4.0±3.5) years) who underwent 18F-FDG total-body PET/CT imaging in Beijing Friendship Hospital, Capital Medical University from August 2021 to December 2021 was performed. The low-dose images (30 s, 20 s, 10 s) of all children extracted by list mode were input into the generative adversarial networks for deep learning (DL) reconstruction to obtain the corresponding simulated standard full-dose images (DL-30 s, DL-20 s, DL-10 s). The semi-quantitative parameters of the liver blood pool and primary lesion of standard full-dose 120 s, 30 s, 20 s, 10 s, DL-30 s, DL-20 s, and DL-10 s images were measured. The target-to-background ratio (TBR), contrast-to-noise ratio (CNR), and CV were calculated. The 5-point Likert scale was used for subjective scoring of image quality, and the detective abilities for positive lesions of each groups were compared. The sensitivities and positive predictive values of positive lesions detection were calculated. Mann-Whitney U test and Kruskal-Wallis rank sum test and χ2 test were used for data analyses. Results:CNR of the 30 s, 20 s, and 10 s groups were lower than those of DL-30 s, DL-20 s, and DL-10 s groups, respectively ( z values: -3.58, -3.20, -3.65, all P<0.05). Score of DL-10 s group was significantly lower than those of 120 s, DL-30 s and DL-20 s groups (4(3, 4), 5(4, 5), 4(4, 5), 4(4, 5); H=97.70, P<0.001). There were no significant differences in TBR, CNR, CV, SUV max and SUV mean of lesions and liver blood pool in 120 s, DL-30 s, DL-20 s, and DL-10 s groups ( H values: 0.00-6.76, all P>0.05). The sensitivities of positive lesion detection in DL-30 s, DL-20 s, and DL-10 s groups were 97.83%(225/230), 96.96%(223/230), 95.65%(220/230), respectively, and the positive predictive values were 96.57%(225/233), 93.70%(223/238), 84.94%(220/259), respectively. The positive predictive value in DL-10 s group was lower than those in DL-30 s and DL-20 s groups ( χ2=23.51, P<0.001). There were more false-positive and false-negative lesions detected by DL-10 s group than those of DL-30 s and DL-20 s groups in different sites. Conclusion:Based on the generative adversarial networks, the image quality of DL-20 s group is high and can meet the clinical diagnostic requirements.

OBJECTIVE: To deeply understand the clinical manifestation, laboratory examination characteristics, diagnosis and treatment of an eight p11 myeloproliferative syndrome (EMS) with rare phenotypes. METHODS: The clinical and laboratory characteristics and the process of allogeneic hematopoietic stem cell transplantation (allo-HSCT) were summarized in 1 rare EMS case involving T/B/myeloid cells. Meanwhile, 2 similar cases in the previous literature were also discussed. RESULTS: The bone marrow examination indicated that the patient with B-cell acute lymphocytic leukemia. The lymph node biopsy showed that the patient was T lymphoblastic/myeloid lymphoma. The 8p11 abnormality was found by the examination of bone marrow chromosomes. The RT-PCR examination showed that the BCR-ABL fused gene was negtive. The FGFR1 breakage was found by using the FISH with FGFR1 probe in lymph node. The Mutation of FMNL3, NBPF1 and RUNX1 genes was found by using the whole exome sequencing. The patient received allo-HSCT under CR2. By the follow-up till to September 2019, the patient survived without the above-mentioned disease. CONCLUSION EMS manifest as neoplasms involving T-lineage, B-lineage, and myeloid-lineage simultaneously is extremely rare. Although the FGFR1 gene-targeted therapy can be conducted, allo-HSCT should be actively considered.
Bone Marrow ; Chromosomes, Human, Pair 8 ; Formins ; Hematologic Neoplasms ; Humans ; Myeloproliferative Disorders/genetics* ; Phenotype ; Receptor, Fibroblast Growth Factor, Type 1/genetics* ; Translocation, Genetic

OBJECTIVE: To observe the clinical effect of platelet rich plasma (PRP) combined with β tricalcium phosphate bioceramic bone in the treatment of non traumatic necrosis of the femoral head in ARCO stageⅡ. METHODS: From January 2017 to December 2018, 100 patients (160 hips) with ARCO stageⅡnon traumatic necrosis of the femoral head were divided into PRP group and control group. In PRP group, 50 patients (80 hips), 22 males and 28 females, aged from 18 to 65 (43.47± 7.23) years, with a course of 4 to 18 (15.8±2.9) months, underwent core decompression and bone grafting combined with PRP implantation. There were 50 cases (80 hips) in the control group, including 27 males and 23 females, aged 20 to 63 (45.72± 7.43) years, and the course of disease was 6 to 19 (14.9±3.8) months. Hip X-ay film was followed up after operation. Harris score and VAS score were used to evaluate the curative effect, and the survival rate of hip joint was recorded. RESULTS: All patients had good wound healing, no infection, thrombosis and other complications. All patients were followed up for 12 to 14 (12.0±0.4) months. Twelve months after operation, the image expression of PRP group was better than that of control group( CONCLUSION Platelet-rich plasma(PRP) combined with artificialbone for core decompression and bone grafting can change the situation of simple artificial bone implantation and uncertain curative effect, improve the success rate of this operation, effectively reduce the collapse rate of femoral head necrosis in the early and middle stage, delay or even avoid hip replacement.
Adolescent ; Adult ; Aged ; Artemisinins ; Bone Transplantation ; Decompression, Surgical ; Female ; Femur Head/surgery* ; Femur Head Necrosis/surgery* ; Humans ; Male ; Middle Aged ; Naphthoquinones ; Platelet-Rich Plasma ; Treatment Outcome ; Young Adult

Objective To understand the epidemiological characteristics of COVID-19 epidemic in Huangpu District of Shanghai, and to provide scientific evidence for prevention and control of COVID-19. Methods Descriptive statistics were used to study the suspected and confirmed cases of COVID-19 reported from January 21 through March 10, 2020 in Huangpu District, Shanghai. Results A total of 120 suspected cases of COVID-19 were reported, of which 12 were diagnosed and 108 were excluded.The first confirmed case was reported on January 21, and the last case was on February 10; the majority (11/12) of the confirmed cases were reported from January 21 through February 1.The average duration of time from the symptom onset to the first medical visit was 2.6 days, whereas the average duration from the first medical visit to the hospital diagnosis was 2.2 days.There were 15 suspected cases with a confirmed history of residence or tourism in Wuhan, in which 6 were confirmed cases.Moreover, 5 suspected cases had a confirmed history of contact with other confirmed cases, in which 3 were confirmed cases.Thus, exposure in Wuhan and exposure to confirmed cases were the most significant risk factors at this stage of the epidemic. Conclusion The 12 cases identified in Huangpu District of Shanghai are all adults, half of whom had confirmed history of exposure in Wuhan.The first cluster of COVID-19 cases in Shanghai is documented in Huangpu District.Epidemiological investigation reveals that the confirmed cases might be infectious the day before the symptom onset.