BACKGROUND:Family-centered care is fundamental in pediatric oncology, emphasizing holistic support for both the patient and their family throughout the cancer journey. However, disclosing a cancer diagnosis remains a complex and culturally sensitive process, with practices varying and being influenced by multiple factors. OBJECTIVES:This study aimed to explore parental disclosure patterns in children with cancer and identify influencing factors from both parent and child perspectives. METHODS:A descriptive qualitative study was conducted at a Chinese national children's medical center. Parents and children were recruited and interviewed, with data analyzed using thematic analysis to identify disclosure patterns and content analysis to explore influencing factors. RESULTS:Thirty-three participants (23 parents and 10 children) from 23 families were interviewed. Five distinct disclosure patterns were identified: "sharing it honestly," "revealing it gradually," "waiting for the right time," "keeping it to oneself," and "remaining silent indefinitely." Guided by the Social-Ecological Framework, we found disclosure patterns were influenced by individual (disease-related, child-related, and parental factors), interpersonal (family dynamics and close relationships), institutional (providers, care settings, and community), and societal (cultural norms and practice guidelines) factors. CONCLUSIONS:This study reveals the diverse and culturally influenced patterns of parental cancer disclosure, shaped by multilayered factors. Future research should focus on deepening understanding of these dynamics and identifying key elements to inform tailored interventions. IMPLICATIONS FOR PRACTICE:Healthcare providers can support families by tailoring approaches to disclosure patterns, addressing barriers, and fostering open communication, while advocating for culturally sensitive guidelines to strengthen parent-child relationships and provide quality care.

Background: Atopic dermatitis (AD) is a chronic, recurrent inflammatory disease associated with an unbalanced immune response in the upper layers of the skin tissue, mostly starting in childhood. As important factors in gene expression regulation, polymorphisms in interleukin (IL)-17A and IL-17F may be associated with the susceptibility and severity of AD. Methods: Blood samples and clinical information were obtained from 132 patients with AD and 100 healthy children. Using multiplex polymerase chain reaction and next-generation sequencing, five potential single-nucleotide polymorphisms (SNPs) of IL-17A and IL-17F were genotyped in all participants. The relationship between SNPs and susceptibility to or severity of AD was examined by analyzing haplotypes and genetic models. Results: The IL-17A rs3819025 polymorphism was substantially associated with higher AD risk in both the allele model (p = 0.03; odds ratio [OR] = 1.76; confidence interval [CI]: 1.05-2.95) and the dominant model (p = 0.04, OR = 1.85; CI: 1.03-3.33). There was no correlation between AD susceptibility and the IL-17A (rs2275913 and rs4711998) or IL-17F (rs763780 and rs12203736) SNPs (all p > 0.05). Additionally, the five IL-17A and IL-17F SNPs did not significantly differ across the mild-to-moderate and severe subgroups (all p > 0.05). Conclusions: The IL-17A/rs3819025 polymorphism was linked to the development of AD, whereas the IL-17F polymorphism was unrelated to the susceptibility to and severity of AD. The IL-17A polymorphism may provide valuable information to speculate on the susceptibility to AD in Chinese Han children.

Lymphatic invasion (LI) is extremely aggressive and induces worse prognosis among patients with colorectal cancer (CRC). Thus, it is critical to characterize the cellular and molecular mechanisms underlying LI in order to establish novel and efficacious therapeutic targets that enhance the prognosis of CRC patients. RNA-seq data, clinical and survival information of colon adenocarcinoma (COAD) patients were obtained from the TCGA database. In addition, three scRNA-seq datasets of CRC patients were acquired from the GEO database. Data analyses were conducted with the R packages. We assessed the tumor microenvironment (TME) differences between LI+ and LI- based scRNA-seq data, LI+ cells exhibited augmented abundance of immunosuppression and invasive subset. Marked extracellular matrix network activation was also observed in LI+ cells within SPP1+ macrophages. We revealed that an immunosuppressive and pro-angiogenic TME strongly enhanced LI, as was evidenced by the CD4+ Tregs, CD8+ GZMK+, SPP1+ macrophages, e-myCAFs, and w-myCAFs subcluster infiltrations. Furthermore, we identified potential LI targets that influenced tumor development, metastasis, and immunotherapeutic response. Finally, a novel LIRS model was established based on the expression of 14 LI-related signatures, and in the two testing cohorts, LIRS was also proved to have accurate prognostic predictive ability. In this report, we provided a valuable resource and extensive insights into the LI of CRC. Our conclusions can potentially benefit the establishment of highly efficacious therapeutic targets as well as diagnostic biomarkers that improve patient outcomes.

Abstract Background Aplastic anemia (AA) is a serious hematopoietic system disease in children. Although there is no standard treatment scheme for acquired non-severe aplastic anemia (NSAA) at present, cyclosporine (CsA) has always been the most commonly used first-line treatment drug for children NSAA. The number of children with AA is large in China. This study aimed to explore the effect, safety and prognosis analysis of CsA on Chinese children with acquired NSAA. Methods The clinical data of children with acquired NSAA who were diagnosed in the affiliated pediatric hospital of Fudan University from December 2010 to June 2023 and whose initial treatment was mainly oral CsA treatment (excluding the use of anti-thymocytes/anti-lymphocyte globulin) were analyzed. Results A total of 40 NSAA patients were enrolled, including 24 males and 16 females, with a median age of 7.0 years (2.5–16.5 years). Among them, 5 cases (12.5%) were blood transfusion dependent, including 1 case of red blood cell (RBC) dependence, 1 case of platelet (Plt) dependence, and 3 cases of both RBC and Plt dependence. Complete remission (CR) was achieved in 9 patients (22.5%), partial remission (PR) in 21 patients (52.5%), and none remission (NR) in 10 patients (25.0%). In PR patients, 5 patients progressed during treatment, and received hematopoietic stem cell transplantation (HSCT), 4 patients underwent hematopoietic reconstitution, and 1 patient died after transplantation; Among 10 NR patients, 4 patients progressed to SAA, of which 2 patients were still treated with oral medicine, and the other 2 patients were treated with HSCT for disease progression 2 and 4 years after treatment (1 patient died of viral brainstem encephalitis combined with sepsis after transplantation); One NR patient died of severe agranulocytosis; The other 5 cases were relatively stable after CsA treatment. The RBC count in CR group and PR group was significantly higher than that in NR group (P < 0.01 and P < 0.05, respectively). Hemoglobin (Hb) in CR group and PR group was higher than that in NR group (P < 0.05). Plt count in CR group was higher than that in NR group (P < 0.05). The average dosage of CsA in NR group was significantly higher than that in CR group (P < 0.05). The peak plasma concentration of CsA in NR group was significantly higher than that in CR group (P < 0.05). The increase of white blood cell (WBC) in CR patients and PR patients after 60 days of CsA treatment was higher than that in NR patients (P < 0.01). The Plt count of CR patients was higher than that of CR and PR patients (P < 0.01). There was no significant difference in WBC, ANC, absolute reticulocyte count (ARC), cellular immune function and CsA blood grain concentration at diagnosis among CR, PR and NR patients, and there was no difference in the increased values of ANC, RBC, Hb and ARC at 60 days after treatment. Conclusions CsA is an effective drug for the treatment of acquired NSAA in children, with good safety under therapeutic drug monitoring (TDM). The efficacy is related to the levels of RBC, Hb and Plt at the time of diagnosis and the response of WBC and Plt at 60 days after treatment.

IntroductionThe study aimed to explore the underlying immunologic mechanisms and immune-related biomarkers associated with alopecia areata (AA) development.Material and methodsExpression data from the GSE68801 dataset, comprising 60 individuals with alopecia areata (28 patchy-type AA (AAP), 23 alopecia universalis (AU), 9 alopecia totalis (AT)), and 36 normal controls (NC) were analyzed. The study investigated differentially expressed RNAs (DERs), immune infiltration, and immune-related modules. Functional enrichment analysis of overlapping DERs was conducted using DAVID. Additionally, overlapping pathways and genes identified in a co-expression network, along with data from the Comparative Toxicogenomics Database 2019 update, were screened.ResultsThere were 1708 lncRNAs and 17,326 mRNAs, along with 427 overlapping DERs among AAP, AU, AT, and NC, were identified. Subsequently, 17 biological processes significantly associated with inflammatory and immune responses, as well as 8 KEGG signaling pathways, including the chemokine and cytokine-cytokine receptor interaction pathway, were enriched. Notable differences in the infiltration of four T cell subtypes—activated CD8 T cells, effector memory CD8 T cells, regulatory T cells, as well as plasmacytoid dendritic cells—were observed compared to NC. Two modules were found to be significantly linked to disease stage progression and various T cell types. Functional analysis revealed significant enrichment of cytokine-cytokine receptor interaction and the T cell receptor signaling pathway among the genes involved in these modules. Furthermore, CXCL9 and CXCL10 were identified as key nodes associated with the disease.ConclusionsOur study indicates that AA is an autoimmune disease associated with T cells, with CXCL9 and CXCL10 emerging as significant prognostic factors in its development.

BACKGROUND:Internationally, early integration of palliative care in pediatric oncology has been widely recognized. However, little is known about the perspective of Chinese providers in this regard. OBJECTIVE:The aim of this study was to explore the perspective of Chinese providers on the early integration of palliative care in pediatric oncology. METHODS:This was a convergent mixed-methods study with a survey among 141 Chinese providers (101 nurses, 38 oncologists, and 2 social workers) and 12 individual interviews (5 oncologists, 5 nurses, and 2 social workers). RESULTS:Three categories existed by comparison and merging of quantitative and qualitative findings: (1) attitudes toward early integration of pediatric palliative care: 75% of the participants endorsed early integration because it would bring benefits to patients and their families-participants had concerns about misunderstandings of palliative care among other stakeholders; (2) patient-provider interactions relating to early integration: participants held contradictory views toward the impact on and influencers of early integration regarding patient-provider interactions; and (3) participants suggested a system to support early integration by addressing parents' misconceptions and providers' training, and institutional facilitation. CONCLUSIONS:Chinese pediatric oncology providers generally exhibit a reserved willingness toward the early integration of palliative care. They agree that palliative care would be beneficial but have concerns about providing structural support and addressing cultural influencers. IMPLICATIONS FOR PRACTICE:Findings of this study emphasize the significance of convening stakeholders and establishing a pediatric palliative care-friendly system in a developing country, particularly by addressing structural support, resource allocation, clarified responsibilities, and capacity building.

Introduction Atopic Dermatitis (AD) is a chronic inflammatory skin disease that affects almost 20% of children and 2 -10% of adults worldwide. Previous studies revealed that Interleukin-6 (IL-6) plays an essential role in autoimmune and chronic inflammatory diseases. This study aims to investigate the associations between IL-6 polymorphisms and AD. Methods Blood samples were collected from 132 AD patients and 100 controls, and single nucleotide polymorphisms (SNPs) in IL-6 (rs2069840 (C/G), rs2066992 (G/T), rs2069837 (A/G) and rs1800796 (G/C)) were analyzed using Multiplex PCR-Based Next Generation Sequencing (NGS). Results Results showed that the A/G genotype of IL-6/rs2069837 was significantly associated with a 1.933-fold increased risk of AD compared to those patients with A/A genotype (OR 1.933; 95%CI 1.086-3.438; p=0.024). The combined A/G-G/G genotype raised AD risk by 1.856 times compared to patients with the A/A genotype in dominant model (OR: 1.856; 95% CI: 1.056-3.261; p=0.030). No association was observed for 3 other SNPs and 4 haplotypes. Discussion These findings suggested that the A/G genotype of IL-6/rs2069837 was more susceptible to AD than A/A genotype in Chinese Han children, indicating the risk role of IL-6/rs2069837 in the occurrence of AD.

ObjectiveTo analyze the efficiency of unrelated umbilical cord blood transplantation (UCBT) in the treatment of hereditary leukodystrophy following busulfan- and cyclophosphamide-based myeloablative chemotherapy.MethodsA retrospective study was performed in patients with hereditary leukodystrophy who underwent UCBT after myeloablative chemotherapy between April 2015 and March 2020.ResultsThe study cohort included 12 pediatric patients (ten males), nine with cerebral adrenoleukodystrophy (ALD) and three with juvenile globoid cell leukodystrophy (GLD). All received HLA-matched or partially mismatched unrelated UCBT. There were no cases of graft rejection. Median neutrophil engraftment time was 20 days [12–33 days] and median platelet engraftment time was 29 days [14–65 days]. Median follow-up was 36 months [1–86 months], and the overall survival rate for patients with cerebral ALD and juvenile GLD after UCBT was 77.8% (7/9) and 100% (3/3), respectively. In patients with ALD, although lipid profiles (serum very-long-chain fatty acid) were improved post-UCBT, six patients demonstrated worse neurologic function score and performance status post-UCBT, and six patients had higher Loes scores at last follow-up compared with baseline. In patients with juvenile GLD, all patients showed stable neurologic function score and performance status despite the Loes score of one patient increased slightly after transplantation.ConclusionIn patients with cerebral ALD, patients with no or mild neurological symptoms can benefit from UCBT, while UCBT cannot reverse advanced disease. In patients with juvenile GLD, UCBT is safe and contributes to stabilize neurological function.

Purpose:To analyze the contribution of metagenomic next-generation sequencing (mNGS) in the guidance of clinical treatment and outcomes of infection during myelosuppression among children with hematological and neoplastic diseases.Patients and Methods:The clinical data and results of mNGS assay of febrile patients suspected of infection were retrospectively collected. The characteristics of pathogenic microorganisms and clinical course of myelosuppressed children with hematological diseases were summarized.Results:Our study included 70 patients (45 males) with a median age of 5 years (range: 0.5 to 13 y). During the study period, there were 96 events of suspected infection. According to comprehensive clinical diagnosis, 73 blood infections, 43 pneumonia and 2 urinary tract infections occurred. The positive rate of mNGS was significantly higher than that of traditional microbial detection (83.3% vs 17.7%). The main pathogens detected by mNGS were Pseudomonas aeruginosa, Acinetobacter, human herpesvirus, Candida and Aspergillus. The average duration of fever was 4.9 days and 11.6 days (P < 0.05), and the average cost of anti-infection treatment was RMB ¥28,077 and 39,898 (P < 0.05) among children received mNGS within 48 hours and more than 48 hours after the onset of infection symptoms.Conclusion:mNGS contributes to clinical management of children with infection during myelosuppression, especially among patients with negative traditional microbial detection. Early implementation of mNGS in children with symptoms has a tendency to reduce the time of infection, fever and the cost of treatment.

Background Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a malignant primary T-cell lymphoma that is challenging to distinguish from autoimmune disorders and reactive panniculitides. Delay in diagnosis and a high misdiagnosis rate affect the prognosis and survival of patients. The difficulty of diagnosis is mainly due to an incomplete understanding of disease pathogenesis. Methods We performed single-cell RNA sequencing of matched subcutaneous lesion tissue, peripheral blood, and bone marrow from a patient with SPTCL, as well as peripheral blood, bone marrow, lymph node, and lung tissue samples from healthy donors as normal controls. We conducted cell clustering, gene expression program identification, gene differential expression analysis, and cell-cell interaction analysis to investigate the ecosystem of SPTCL. Results Based on gene expression profiles in a single-cell resolution, we identified and characterized the malignant cells and immune subsets from a patient with SPTCL. Our analysis showed that SPTCL malignant cells expressed a distinct gene signature, including chemokines families, cytotoxic proteins, T cell immune checkpoint molecules, and the immunoglobulin family. By comparing with normal T cells, we identified potential novel markers for SPTCL (e.g., CYTOR, CXCL13, VCAM1, and TIMD4) specifically differentially expressed in the malignant cells. We also found that macrophages and fibroblasts dominated the cell-cell communication landscape with the SPTCL malignant cells. Conclusions This work offers insight into the heterogeneity of subcutaneous panniculitis-like T-cell lymphoma, providing a better understanding of the transcription characteristics and immune microenvironment of this rare tumor.

Background Hereditary leukodystrophies are diverse metabolic diseases caused by gene mutations that result in the abnormal development or degeneration of myelin. Transplantation of matched sibling donor umbilical cord blood (UCB) can slow progression and prolong survival in some cases. For patients without siblings, however, UCB transplantation from unrelated donors may be the only option. Methods This retrospective study assessed unrelated UCB transplantation (UCBT) efficacy following busulfan- and cyclophosphamide-based myeloablative chemotherapy. Results The study cohort included 12 pediatric patients (ten males), nine with adrenoleukodystrophy (ALD) and three with globoid cell leukodystrophy (GLD), treated between April 2015 and March 2020. All received HLA-matched or partially mismatched UCBT. Median age at UCBT was 7.2 years (range, [0.8–12.9 years]). There were no cases of graft rejection. Median neutrophil engraftment time was 20 days [12–33 days] and median platelet engraftment time was 29 days [14–65 days]. Median follow-up was 28 months [1–73 months], and overall survival rate was 83.3% (10/12). Seven patients had higher Loes scores post-transplantation, and two patients died of infection. Four patients with rapid neurological deterioration pre-UCBT exhibited worse neurological symptoms post-UCBT. In contrast, four patients with stable neurological symptoms pre-UCBT demonstrated symptom stability post-UCBT, and two with no neurological symptoms pre-UCBT were also symptom-free post-UCBT. Further, lipid profiles of surviving ALD patients were improved post-treatment. Conclusions Hereditary leukodystrophy patients with mild neurological symptoms can benefit from UCBT, while UCBT cannot reverse advanced disease.