This article focuses on a case study of sitosterolemia in a child who initially presented with hemolytic anemia and thrombocytopenia. Sitosterolemia is a rare autosomal recessive lipid metabolism disorder, difficult to diagnose due to its non-typical clinical manifestations. The 8-year-old patient was initially misdiagnosed with pyruvate kinase deficiency. Comprehensive biochemical and molecular biology analyses, including gene sequencing, eventually led to the correct diagnosis of sitosterolemia. This case highlights the complexity and diagnostic challenges of sitosterolemia, emphasizing the need for increased awareness and accurate diagnosis in patients presenting with similar symptoms.

OBJECTIVE:This research seeks to analyse the immunomodulatory impacts of adrenomedullin (ADM) on macrophages induced by bacterial lipopolysaccharide and to investigate the influence of macrophage-conditioned media from various stimulating factors on the biological activity of dental pulp stem cells (DPSCs) in vitro. METHODS:The polarisation effect of ADM on macrophages was analysed through cell immunofluorescence staining and flow cytometry. Potential mechanisms were explored through transcriptomics and metabolomics. The impact of different macrophage-conditioned media on the biological activity of DPSCs was evaluated through western blotting, Realtime fluorescence quantitative, alkaline phosphatase activity assay, and eosin red staining. Each experiment was performed with 3 biological and 3 technical duplicate measurements. Statistical analysis was performed with t test and one-way ANOVA, and mathematical significance defined as P < .05. RESULTS:ADM can reverse polarisation of macrophages towards M2 phenotype by Lipopolysaccharide and the conditioned media of ADM-induced M2 polarised macrophages significantly enhances the proliferation and differentiation of DPSCs. The mechanism may involve the metabolic reprogramming of macrophages by ADM, specifically promoting the metabolic shift from glycolysis to mitochondrial oxidative phosphorylation in Lipopolysaccharide-induced macrophages. CONCLUSION:These results indicate that ADM is involved in suppressing inflammation and enhancing the proliferation and differentiation of DPSCs by reprogramming macrophage metabolism.

Objective: To analyze the mid-term efficacy of the China Net Childhood Lymphoma mature B-cell lymphoma 2017 (CNCL-B-NHL-2017) regimen in treating children with high-grade B-cell lymphoma (HGBL). Methods: Clinical and pathological data of HGBL children aged≤18 years admitted to 16 hospitals of the Chinese Children's Lymphoma Collaborative Group (CNCL) from May 2017 to April 2021 were collected retrospectively. They were divided in to high-grade B-cell lymphoma with double hit/triple hit (HGBL-DH/TH) group and high-grade B-cell lymphoma non-specified (HGBL-NOS) group, according to the 2016 version of the World Health Organization (WHO) Hematopoietic and Lymphoid Tissues Cancer Classification. Both groups of patients were treated with stratified chemotherapy by risk according to the CNCL-B-NHL-2017 scheme. The deadline for follow-up was December 31, 2023. All the patients were examined by chromosome fluorescence in situ hybridization (FISH), and the rearrangement of genes MYC, BCL-2 and BCL-6 was confirmed. The clinical and pathological characteristics of patients at disease onset were analyzed, and the therapeutic effects of patients in different clinical stages and risk groups were compared. Survival analysis was drawn by Kaplan Meier method, the log-rank test was used to compare the differences in the cumulative survival rate between different groups, and multivariate Cox regression model was used to identify the prognostic factors. Results: A total of 62 patients were included, with an onset age [M(Q1, Q3)] of 7 (4, 11) years, including 48 males and 14 females. There were 11 (17.7%) patients in stageⅡ, 33(53.2%)patients in stage Ⅲ and 18(29.1%)patients in stage Ⅳ. FISH testing showed that 4 cases (6.5%) were HGBL-DH and 3 (4.8%) were HGBL-TH. The remaining 55 cases (88.7%) were HGBL-NOS, with 18 cases accompanied by MYC rearrangement. There were 7 cases in the HGBL-DH/TH group and 55 cases in the HGBL-NOS group. Thirteen cases (20.9%) were treated with the B1 regimen, 3 cases (4.8%) with B2 regimen, 37 cases (59.6%) with C1 regimen, and 9 cases (14.7%) with the C2 regimen. Forty-eight cases (77.4%) received rituximab therapy at the same time. Five cases (8.0%) progressed during treatment. The follow-up time [M(Q1, Q3)] was 43.5 (36.1, 53.7) months. The complete remission rate was 91.9% (57/62). The 3 year overall survival rate was 93.5% and event-free survival (EFS) rate was 91.9%. The 3-year overall survival rate in the HGBL-NOS group was higher than that in the HGBL-DH/TH group (96.3% vs 71.4%, P=0.011). The 3-year EFS rate of the HGBL-NOS group was higher than that of the HGBL-DH/TH group (94.5% vs 71.4%, P=0.037). In the HGBL-NOS subgroup, the overall survival rate of children with MYC rearrangement was lower (100% vs 88.9%,P=0.039). Multivariate Cox regression analysis showed that central invasion (HR=6.05, 95%CI: 1.96-38.13, P=0.046) was a risk factor for overall survival. Conclusion: CNCL-B-NHL-2017 regimen shows significant effects in the treatment of pediatric HGBL, with a good prognosis.

Further evidence is needed to explore the impact of high-altitude environments on the neurologic function of neonates. Non-invasive techniques such as cerebral near-infrared spectroscopy and amplitude-integrated electroencephalography can provide data on cerebral oxygenation and brain electrical activity. This study will conduct multiple cerebral near-infrared spectroscopy and amplitude-integrated electroencephalography monitoring sessions at various time points within the first 3 days postpartum for healthy full-term neonates at different altitudes. The obtained data on cerebral oxygenation and brain electrical activity will be compared between different altitudes, and corresponding reference ranges will be established. The study involves 6 participating centers in the Chinese High Altitude Neonatal Medicine Alliance, with altitude gradients divided into 4 categories: 800 m, 1 900 m, 2 400 m, and 3 500 m, with an anticipated sample size of 170 neonates per altitude gradient. This multicenter prospective cohort study aims to provide evidence supporting the impact of high-altitude environments on early brain function and metabolism in neonates.

Background: The aim of this case was to understand and treat the intricate root canal anatomy observed in complex maxillary first premolars, particularly those exhibiting three root canals, emphasizing the significance of understanding root canal morphological variations in their treatment. Conclusions: This case reported the methods of treating three-root canal maxillary first premolars, including warm vertical compression technique, root canal microsurgery, and the application of rubber dam. The utilization of dental operating microscopes, various surgical strategies, and key assessments of X-rays and cone beam computed tomography (CBCT) scans were all essential steps for achieving accurate and safe root canal therapy.

Epilepsy is a multifaceted neurological syndrome characterized by recurrent, spontaneous, and synchronous seizures. The pathogenesis of epilepsy, known as epileptogenesis, involves intricate changes in neurons, neuroglia, and endothelium, leading to structural and functional disorders within neurovascular units and culminating in the development of spontaneous epilepsy. Although current research on epilepsy treatments primarily centers around anti-seizure drugs, it is imperative to seek effective interventions capable of disrupting epileptogenesis. To this end, a comprehensive exploration of the changes and the molecular mechanisms underlying epileptogenesis holds the promise of identifying vital biomarkers for accurate diagnosis and potential therapeutic targets. Emphasizing early diagnosis and timely intervention is paramount, as it stands to significantly improve patient prognosis and alleviate the socioeconomic burden. In this review, we highlight the changes and molecular mechanisms of the neurovascular unit in epileptogenesis and provide a theoretical basis for identifying biomarkers and drug targets.

牙髓再生组织工程技术是基于生物学、材料学和工程学等多个学科的交叉研究,通过构建组织细胞-生物材料三维空间复合体,植入患牙髓腔,以促进无髓患牙的血运重建和组织细胞的增殖分化,并最终形成修复性牙髓-牙本质复合体.组织工程技术在牙髓再生的治疗中有巨大应用前景,有望取代根管治疗成为损伤牙髓修复的主流方法.本文对牙髓再生组织工程技术构建的仿生支架和体外模型进行了综述,以期对组织工程技术在牙髓再生中的作用有更深入的理解和新的启示.

Glucocorticoid-induced osteoporosis (GIOP) is a skeletal disease characterized by decreased bone strength and increased fracture risk associated with long-term glucocorticoid use. GIOP is the most common secondary osteoporosis that critically affects the quality of life of patients. Currently, the incidence of GIOP in China remains high, with insufficient awareness and lack of prevention and treatment norms. Therefore, the Chinese Rheumatology Association has established this standard based on domestic and international experience, with the aim of raising awareness of prevention and treatment among clinicians, guiding the standardized diagnosis and treatment of this disease, and improving the overall prognosis of patients with GIOP.

BACKGROUND:To explore the application of magnetic resonance imaging (MRI) and diffusion-weighted imaging (DWI) in the diagnosis of hypoxic-ischemic encephalopathy and kernicterus in premature infants.METHODS:Fifty-eight premature infants with hypoxic-ischemic encephalopathy and fifty-eight premature infants with kernicterus who were examined and treated in our hospital between January 2018 and January 2021 were assigned to the observation group or the control group. All patients were examined by MRI imaging and DWI imaging, and the examination results were compared between the two groups.RESULTS:No significant differences were found in sensitivity, specificity, positive predictive value, or negative predictive value between the observation group and the control group (P>0.05). MRI could clearly visualize the signal changes of patients, whereas DWI did not show any signal changes. There was no significant difference between MRI and DWI in the diagnosis of hypoxic-ischemic encephalopathy in premature infants. Further, there was no significant difference in the diagnostic performance of MRI between the observation group and the control group (P>0.05). However, the diagnostic performance of DWI in the control group was better than that in the observation group, and the difference was statistically significant (P<0.05).CONCLUSIONS:MRI and DWI imaging have high detection rates for the diagnosis of hypoxic-ischemic encephalopathy and kernicterus in premature infants. These imaging methods can benefit the treatment of premature infants and have important clinical application value.

目的:探讨鼻中隔后端犁骨切除后鼻孔成形术治疗先天性后鼻孔闭锁的疗效。方法:回顾性分析深圳市儿童医院2017年3月至2019年6月收治的5例先天性后鼻孔闭锁患儿的资料，其中男2例，女3例；年龄33 d至2岁4个月；2例双侧先天性后鼻孔闭锁（CHARGE综合征），1例单侧后鼻孔闭锁和单侧后鼻孔狭窄（Crouzon综合征）；2例单侧先天性后鼻孔闭锁。所有病例在3.0 mm 0度耳内镜经鼻腔切除鼻中隔后端骨质、去除犁骨、闭锁板骨质并打磨患侧翼内板，连通双侧后鼻孔。术后不放置长期扩张支架。术后2周、1、3、6、12个月常规随访，最长随访超过2年。观察手术疗效。结果:5例患儿均恢复良好的鼻腔通气功能，随访期间未见后鼻孔造孔出现再次狭窄和闭锁。无其他异常并发症出现。结论:耳内镜下鼻中隔后端、犁骨、闭锁板切除术治疗后鼻孔闭锁可行，且术后可以不放置长期扩张支架，手术疗效确定，造孔无再次狭窄或闭锁。

本文介绍了采用支撑喉镜内镜下经皮单侧声带外移缝合固定术治疗的3例新生儿双侧声带麻痹患儿的诊疗经验。3例患儿出生后即有喉喘鸣、重度呼吸困难以及低氧血症,需要呼吸支持治疗,出生后3 d内均确诊。支撑喉内镜下利用2个10 ml注射器的针头,经皮穿针置入4-0不可吸收缝线,把线引出到颈外打结固定,外移一侧声带后部,扩大声门开放气道,缓解呼吸困难。3例患儿术后2～3周喉喘鸣、呼吸困难均有明显改善,均无需气管切开术。其中2例患儿出院时能正常呼吸和正常喂养,1例患儿能正常呼吸但需要勺子喂养。随访至2019年11月,有1例患儿双侧声带运动恢复,另2例患儿双侧声带运动暂未见恢复。

Obstructive sleep apnea syndrome (OSAS) is a common clinical disease and easily overlooked by clinicians and patients,which is due to partial collapse and airflow obstruction caused.The tendency of airway collapse can be used to quantify the airway pressure.It is closely related to sleep apnea syndrome and has certain clinical significance for the etiology,diagnosis and treatment of OSAS.

Early childhood caries (ECC) is a considerable pediatric and public health problem worldwide. Preceding studies have focused primarily on bacterial diversity at the taxonomic level. Although these studies have provided significant information regarding the connection between dental caries and oral microbiomes, further comprehension of this microbial community's ecological relevance is limited. This study identified the carbon source metabolic differences in dental plaque between children with and without ECC. We compared the microbial community functional diversity in 18 caries-free subjects with 18 severe ECC patients based on sole carbon source usage using a Biolog assay. The anaerobic microbial community in the ECC patients displayed greater metabolic activity than that of the control group. Specific carbon source metabolism differed significantly between the two groups. Subjects from the two groups were well distinguished by cluster and principal component analyses based on discriminative carbon sources. Our results implied that the microbial functional diversity between the ECC patients and healthy subjects differed significantly. In addition, the Biolog assay furthered our understanding of oral microbiomes as a composite of functional abilities, thus enabling us to identify the ecologically relevant functional differences among oral microbial communities.

Pediatric PulmonologyVolume 19, Issue 1 p. 67-68 Correspondence To the editor: On the effect of oxygen inhalation on pulmonary artery pressure evaluated by doppler echocardiography in pneumonia on infants and children Wang Yan-Hua MD, Wang Yan-Hua MD Department of Pediatrics, First Hospital Department of Pediatrics, People's Hospital, Beijing Medical University, Beijing, P.R. ChinaSearch for more papers by this authorDu Jun-Bao MD, Du Jun-Bao MD Department of Pediatrics, First Hospital Department of Pediatrics, People's Hospital, Beijing Medical University, Beijing, P.R. ChinaSearch for more papers by this authorLi Jin-Yun MD, Li Jin-Yun MD Department of Pediatrics, First Hospital Department of Pediatrics, People's Hospital, Beijing Medical University, Beijing, P.R. ChinaSearch for more papers by this authorZhao Yi MD, Zhao Yi MD Department of Pediatrics, First Hospital Department of Pediatrics, People's Hospital, Beijing Medical University, Beijing, P.R. ChinaSearch for more papers by this author Wang Yan-Hua MD, Wang Yan-Hua MD Department of Pediatrics, First Hospital Department of Pediatrics, People's Hospital, Beijing Medical University, Beijing, P.R. ChinaSearch for more papers by this authorDu Jun-Bao MD, Du Jun-Bao MD Department of Pediatrics, First Hospital Department of Pediatrics, People's Hospital, Beijing Medical University, Beijing, P.R. ChinaSearch for more papers by this authorLi Jin-Yun MD, Li Jin-Yun MD Department of Pediatrics, First Hospital Department of Pediatrics, People's Hospital, Beijing Medical University, Beijing, P.R. ChinaSearch for more papers by this authorZhao Yi MD, Zhao Yi MD Department of Pediatrics, First Hospital Department of Pediatrics, People's Hospital, Beijing Medical University, Beijing, P.R. ChinaSearch for more papers by this author First published: January 1995 https://doi.org/10.1002/ppul.1950190113AboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat No abstract is available for this article. Volume19, Issue1January 1995Pages 67-68 RelatedInformation