ABSTRACT
Very early onset inflammatory bowel disease (VEO-IBD) in children refers to an IBD with the onset age of less than 6 years old, clinically characterized by recurrent colitis, perianal lesions, and nutrient absorption disorders.Different from adults, single gene mutation plays an important role in the pathogenesis of VEO-IBD.To date, about 70 single gene defects have been identified involving the pathogenesis of VEO-IBD, including epithelial barrier, neutrophil and phagocyte function, immune cell selection and activation, immunosuppressive mechanism, or apoptosis.Interleukin-10 (IL-10) is an anti-inflammatory cytokine that regulates innate and adaptive immunity, influences the expression of pro-inflammatory molecules and the function of multiple immune cells, and plays a vital role in the development and progression of IBD.Patients with defects in the IL-10 signaling pathway (IL-10 or IL-10 receptor deficiency) may develop life-threatening colitis as early as childhood.This article reviews the progress in the pathogenesis and treatment of VEO-IBD caused by IL-10 signaling pathway defects.
ABSTRACT
Objective To observe the effects of Huatan Quyu Decoction on cognitive function and the expressions of GABA and VILIP-1 in brain tissue of rats with cerebral small vessel disease;To discuss its mechanism for treatment on cerebral small vessel disease.Methods Totally 48 male SD rats were randomly divided into blank group,model group,Huatan Quyu Decoction low-and high-dosage groups,with 12 rats in each group.Except for the blank group,a rat model of cerebral small vessel disease was prepared by in vitro injection of homologous microemboli.Huatan Quyu Decoction low-and high-dosage groups were given Huatan Quyu Decoction 1.25 and 2.5 g/kg by gavage,the blank group and model group were gavage with equal amounts of distilled water for 28 consecutive days.Morris water maze experiment was conducted on day 1,7,14,and 28 after administration to evaluate the learning and memory abilities of rats,HE staining was used to observe pathological changes in hippocampal tissue,and immunohistochemical staining was used to detect the expressions of GABA and VILIP-1 proteins in brain tissue.Results Compared with the blank group,the escape latency of Morris water maze experiment in model group significantly prolonged(P<0.05),and the number of crossing platforms was significantly reduced(P<0.05);the arrangement of hippocampal tissue cells was disordered,gaps widen,and nuclei atrophy and necrosis,the GABA expression in brain tissue significantly decreased(P<0.05),while the VILIP-1 expression significantly increased(P<0.05).Compared with the model group,the escape latency of Morris water maze experiment in the Huatan Quyu Decoction low-and high-dosage groups significantly shortened(P<0.05)on day 7,14,and 28 of administration,and the number of crossing platforms significantly increased(P<0.05),GABA expression significantly increased(P<0.05),while VILIP-1 expression significantly decreased(P<0.05).Compared with the Huatan Quyu Decoction low-dosage group,the escape latency of Morris water maze experiment in Huatan Quyu Decoction high-dosage group decreased at various time points,and the number of crossing platforms increase,the pathological damage of hippocampal tissue was reduced,the expression of GABA in brain tissue increased,and the expression of VILIP-1 decreased,with statistical significance(P<0.05).Conclusion Huatan Quyu Decoction can increase the expression of GABA in brain tissue and inhibit the expression of VILIP-1,thereby improve the cognitive function of rats with cerebrovascular disease.
ABSTRACT
Objective:To assess the clinical significance of next-generation sequencing (NGS)-based IGH/ IGK gene rearrangement analysis versus flow cytometry (FCM) in diagnosing minimal residual disease (MRD) of children with acute B-cell lymphoblastic leukemia (B-ALL). Methods:Clinical data, NGS-MRD and FCM-MRD findings at the initial diagnosis and after induction chemotherapy of 85 children diagnosed as B-ALL in Children′s Hospital of Nanjing Medical University from July 2019 to July 2021, were retrospectively analyzed.The sensitivity of the two methods, and the positive rate were compared by χ2 test or Fisher′ s test.The correlation was identified by Spearman correlation analysis. Results:Dominant clone sequences were detected in all children at the initial diagnosis by NGS, while selection markers were identified by FCM in 75(88.2%) patients.Positive MRD rate detected by NGS-MRD was significantly higher than that of FCM-MRD at the same time point after induction chemotherapy[31.8%(27/85) vs.9.4%(8/85), P<0.001]. Compared with those of FCM-MRD, NGS-MRD had good sensitivity (100.0%), specificity (75.3%) and negative predictive value (100.0%), and the positive predictive value was 29.6%.MRD results detected by NGS were consistent with that of FCM ( r=0.569, P<0.001). By July 27, 2022, 2 patients with NGS-MRD (+ )FCM-MRD (-)relapsed during maintenance chemotherapy. Conclusions:NGS is highly consistent with FCM in the detection of MRD in children with B-ALL, which is more sensitive.The combination of NGS-MRD and FCM-MRD benefits more in monitoring MRD in children with B-ALL after induction chemotherapy.
ABSTRACT
Narrative medicine, an interdisciplinary subject merging medicine and the humanities, serves as a vital instrument in capturing, comprehending, and integrating the diverse perspectives of all stakeholders involved in the realm of illness. In the context of pediatric palliative care, the implementation of narrative medicine fosters effective doctor-patient communication and enhances the doctor-patient relationship. This article delves into the importance and practicality of narrative medicine in pediatric palliative care, shedding light on the hurdles faced during its implementation. The study highlights the pressing need for further extensive and in-depth research of narrative medicine in pediatric palliative care, aiming to provide evidence-based insights that can inform clinical interventions and decision-making processes.
ABSTRACT
With the progress of medical technology, the survival rate of children with cancer has been conti-nuously improved, and the quality of life of children cancer survivors has become the research direction of modern medicine.Particularly, fertility protection has attracted increasing attention, and some reproductive assistance technologies make it possible to preserve the fertility of cancer groups.In this article, the damage of pediatric cancer treatment to the fertility of male and female patients, existing reproductive protection methods and their effects were summarized.This paper provides a reference for the treatment of pediatric cancer patients and hopes to arouse more people′s awareness of fertility protection.
ABSTRACT
With the progress of medical technology, the survival rate of children with cancer has been conti-nuously improved, and the quality of life of children cancer survivors has become the research direction of modern medicine.Particularly, fertility protection has attracted increasing attention, and some reproductive assistance technologies make it possible to preserve the fertility of cancer groups.In this article, the damage of pediatric cancer treatment to the fertility of male and female patients, existing reproductive protection methods and their effects were summarized.This paper provides a reference for the treatment of pediatric cancer patients and hopes to arouse more people′s awareness of fertility protection.
ABSTRACT
Severe combined immunodeficiency(SCID)is a kind of severe primary immunodeficiency disease.Patients often develop symptoms after birth, which is characterized by recurrent and life-threatening infection, sometimes accompanied by varying degrees of dysplasia.According to the classification proposed by the PID expert Committee of the International Union of Immunological Societies, SCID is clinically classified by the number of lymphocytes.Early screening and intervention of SCID is significant to improve the prognosis.With the promotion of TREC and other early screening methods, the detection rate of SCID has significantly improved, but the accurate detection rate in different ethnic groups still needs further explored.Hematopoietic stem cell transplantation is a traditional therapy for radical cure of SCID.And the safety of gene therapy for SCID has been gradually guaranteed with continuous improvement during recent years.This article will review the classification, early screening methods and treatment progress of SCID.
ABSTRACT
Epstein-Barr(EB)virus is the first carcinogenic virus discovered in 1964.EBV infection is common in pediatrics and most of the infected children are asymptomatic.When the immune function of children becomes low or deficient, EBV may cause a series of benign or malignant lymphoproliferative diseases, such as infectious mononucleosis, chronic active EBV infection, hemophagocytic lymphohistiocytosis, post-transplant lymphoproliferative disorders, X-linked lymphoproliferative syndrome and so on.Most of these diseases have complex pathogenesis, diverse clinical manifestations, poor prognosis and high mortality.The only effective treatment is allogeneic hematopoietic stem cell transplantation.
ABSTRACT
Over the past decades, substantial improvements in diagnostics, treatment, techniques and pharmacotherapy have resulted in a steadily increasing population of childhood cancer survivors.Health-related quality of life (HRQoL) of survivors has gradually become the focus of attention.Survivors of cancer in children will face a range of problems, such as physical, psychological and social pressure brought by the disease, growth and development disorders, gonadal dysfunction and late effects (organ dysfunction and secondary cancer). Previous studies have shown that the HRQoL of cancer survivors is significantly lower than that of the normal people, but some studies dedicated that the cancer experience may have a positive impact on the HRQoL of survivors.The quality of life questionnaires is widely used to evaluate HRQoL from the aspects of physical health, mental health and social health.Information from observational, cross-sectional, but particularly longitudinal studies, is needed to further describe HRQoL of cancer survivors in children, as well as provide a foundation for the development of intervention studies.More relevant studies are expected to provide evidence-based basis in making clinical decisions.
ABSTRACT
Childhood hemophagocytic lymphohistiocytosis (HLH) can be classified into primary and secondary forms.With the progress of diagnosis and treatment of stratified treatment, the prognosis was improved significantly.Allogeneic hematopoietic stem cell transplantation (allo-HSCT) is recommended for the treatment of primary HLH, recurrent/refractory HLH and central nervous system involved HLH.With the development of transplantation technology, transplantation related mortality (TRM) has decreased significantly.In general, remission period of HLH is the best time for transplantation, while in some special situation HSCT can be done when HLH is still active.In addition to HLA matched sibling donors, the survival rates of unrelated matched donors and haploidentical donors were significantly improved.Most of the domestic institution choose the improved myeloablative conditioning as the conditioning regimen.