Objective:To analyze the clinical features of anti-neurofascin-155 (NF155) antibody positive autoimmune nodopathy in children.Methods:This was a case series study. A total of 6 children who were diagnosed accurately as anti-NF155 antibody positive autoimmune nodopathy by cell immunofluorescence assay at the Children′s Hospital of Fudan University from January 2020 to December 2023 were collected. This study retrospectively analyzed 6 pediatric children′s clinical manifestations, laboratory and electrophysiological examination results, and treatment outcomes.Results:Among 6 children with anti-NF155 antibody positive autoimmune nodopathy, there were 4 boys and 2 girls. The onset age of 6 children ranged from 3 years and 8 months to 12 years. All 6 children had extremity weakness (more severe in the distal and the lower extremities than in the upper extremities), 5 children had sensory deficits such as numbness or pain in the extremities, 4 children had tremors and ataxia, 3 children had cranial nerve involvement. Among the 6 children, 4 children had protein-cell separation in cerebrospinal fluid examinations. Among the 6 children, 1 child had central nervous system demyelination, the brain magnetic resonance imaging showed multiple abnormal signals in the bilateral cerebral hemispheres. Four children showed motor and sensory nerve damage in electrophysiological examination, and 2 children only showed motor nerve damage. Three children showed myelin and axonal damage, and 3 children only showed axonal damage. Among the 6 children, 5 children were treated with intravenous immunoglobulin and steroids. Among them, 2 children underwent plasma exchange due to poor efficacy, and subsequently, rituximab was added. There was 1 child changed the treatment with olfatomumab since the symptoms did not significantly improve after using rituximab. After treatment for 4-15 months, 2 children had no clinical symptoms, 1 child had improvement in clinical symptoms, 2 children had no significant improvement in clinical symptoms, and 1 child who did not receive the immunotherapy had no significant change in clinical symptoms.Conclusions:Anti-NF155 antibody positive autoimmune nodopathy in children presents with varying degrees of clinical manifestations. It is mainly characterized by extremity weakness, numbness and pain, often accompanied bytremorsand ataxia. Some pediatric patients may also have central nervous system demyelination. Cerebrospinal fluid and electrophysiological examination are important auxiliary examination methods. If steroid therapy is not effective, plasma exchange and rituximab treatment should be used as soon as possible.

China prospective multicenter birth cohort (Prospective Omics Health Atlas birth cohort, POHA birth cohort) study was officially launched in 2022. This study, in collaboration with 12 participating units, aims to establish a high-quality, multidimensional cohort comprising 20 000 naturally conceived families and assisted reproductive families. The study involves long-term follow-up of parents and offspring, with corresponding biological samples collected at key time points. Through multi-omics testing and analysis, the study aims to conduct multi-omics big data research across the entire maternal and infant life cycle. The goal is to identify new biomarkers for maternal and infant diseases and provide scientific evidence for risk prediction related to maternal diseases and neonatal health.

This article is a summary of the Second China Breast Milk Science Conference, which was held in Beijing from August 5 to 7, 2022, with the theme of "Data sharing, method sharing and science sharing". The purpose of the conference is to summarize the latest progress in breast milk research, identify the unresolved issues, and jointly discuss the direction of future breast milk research. Firstly, we summarize the contents and purpose of breast milk scientific research and prospect of breast milk science. The second part focuses on the research status of breast milk composition and its health effects, and puts forward the future research direction. The third section focuses on the health effects of breastfeeding and scientific support and key aspects of breastfeeding promotion actions. The fourth part elaborates the specific methods of current scientific research of breast milk and emphasize the importance of method standardization and the idea of future methodological research. The fifth part consists of the strategy of feeding infants with medical conditions and ways to better promote the growth and development of these infants. The last part introduces the innovation, deficiencies, and future research directions of infant formula production technology in China. This conference demonstrate the importance of multidisciplinary communication, discussions and collaborations in clinical medicine, nutrition, perinatal health, food science, and policy-making in the scientific research of breast milk, and provides guidance for future multidisciplinary research on the physiology of lactation, the composition of breast milk, breastfeeding, and infants and young children nutrition.

Objective To study the clinical effects of continuous blood purification (CBP) in the treatment of newborns with severe sepsis and multiple organ dysfunction syndrome (MODS). Method From May 2013 to November 2018, the clinical data of infants with severe sepsis and MODS receiving CBP in the neonatal department of our hospital were retrospectively analysed. Changes of blood pressure, arterial partial pressure of oxygen/inhaled oxygen concentration (PaO2/FiO2), pH, serum potassium, sodium, urea nitrogen, creatinine, urine volume and maintenance dose of adrenaline at different time points before and after CBP were analysed. Result According to the inclusion and exclusion criteria, a total of 8 newborns with sepsis and MODS were enrolled in the study. One patient had the complication of perforated colon, and the other one had acute renal failure. The number of affected organs in these infants was 3～5. Six cases began CBP treatment within 1～5 days after admission, and the other two cases began CBP treatment on the 38th and 47th days after admission. The average treatment duration was (58.6±25.9) h. The effective rate of CBP in the treatment of severe sepsis with MODS was 75.0％(6/8). Blood pressure was increased at 6 h, 12 h, 24 h and 48 h after treatment and at the end of treatment. PaO2/FiO2 and blood pH were increased. The urine volume was increased at 24 h and 48 h after treatment and at the end of treatment (P<0.05). Serum potassium, urea nitrogen and creatinine were significantly decreased (P<0.05). The maintenance dose of adrenaline was also decreased significantly at 12 h after CBP (P<0.05), and withdrawn at 48 h after treatment. Only one case experienced membrane blockage during CBP. Dialysis was continued after the replacement of filtration membrane. No other complications existed. Conclusion CBP is effective in the treatment of neonatal severe sepsis with MODS. It can improve the circulation and renal function.

Objective: To summarize the effect of simultaneous orthognathic surgery along with mandibular ramus reconstruction using costochondral graft, for adult M3 hemifacial macrosomia. Methods: From November 2015 to October 2017, 5 adults diagnosed with M3 hemifacial macrosomia were treated. There were 3 males and 2 females, aged from 19 to 26 years. Le Fort Ⅰ osteotomy and SSRO with simultaneous mandibular ramus reconstruction using contralateral sixth or seventh costochondral graft was performed to correct the facial asymmetry and occluding relation. The data of clinical examination and CTs were collected at the time point of immediately postoperative, 1, 3, 6, 12 months after surgery. The facial symmetry, joint function, occlusion and 3D measurements in CT image reconstruction were analysed to evaluate the surgery outcome. Results: The length of rib and costal cartilage ranged from 47 mm to 67 mm. All the costal cartilage grafts survived, and 4 patients got primary healing. All patients were followed for 2-13 months (with the mean follow-up of 8 months). The ratio of ramus length of affected side to normal side was over 80%. The occlusion was stable. The facial structures were satisfactory after 6 months. Conclusions Orthognathic surgery with simultaneous mandibular ramus reconstruction using costochondral graft is suitable for the adult severe hemifacial macrosomia, with satisfactory cosmetic and functional results. This method is easily performed with reliable graft survival rate, aesthetic facial structure and stable occlusion.

Objective To investigate the CT spectral imaging features of benign and malignant ovarian tumors and to assess the value of spectral CT in differentiating between benign and malignant ovarian tumors.Methods This study was granted by the institutional review board with a waiver for informed consent.34 patients with ovarian tumor(11 benign tumors and 23 malignant tumors)underwent preoperative CT scanning that included arterial phase (AP)and venous phase (VP)with GSI mode.Measurements were performed on the GSI viewer.The iodine concentration (IC,100 ug/cm3)was saved,and the normalized iodine concentration (NIC)and the spectral curve(spectral Hounsfield unit curve)were recorded.The slope of the curve was calculated as the formula:(CT attenuation (40 keV)-CT attenuation(100 keV))/60.The CT value was measured at 70 keV in Mono image.CT values,the slope of the spectral curve,the iodine concentration and NIC were compared between the two groups by using independent sample t-test with software SPSS 2 1.0.Receiver operating characteristic (ROC)was used to determine the threshold of slope of the spectral curve,the iodine concentration and NIC for differentiating benign and malignant ovarian tumors in VP.Results The slope of the spectral curve,the iodine concentration and NIC of malignant ovarian tumors in VP were 1.72±0.77,14.50±6.44 (100 μg/cm3),and 0.33±0.12.The slope of the spectral curve,the iodine concentration and NIC of benign ovarian tumors in VP were 1.05±0.48,8.86±4.04 (100 μg/cm3),and 0.22±0.13.Significant differences were found between the slope of spectral curve,the iodine concentration and NIC of the malignant ovarian tumors and those of the benign ovarian tumors in VP (P=0.01).CT values in double phases revealed no difference between these two groups (P>0.05).Conclusion There is a significant difference between the solid component of benign ovarian tumors and that of malignant ovarian tumors in spectral CT.GSI scan mode can provide more information for the differential diagnosis of benign and malignant ovarian tumors.

Objective To observe the effects of TLR9 on the nude mouse transplanted tumor growth of human pancreanc cancer and its drug resistance.Methods The nude mouse transplated tumor of human pancreatic cancer PANC-1 was established and randomly divided into 6 groups for conducting the experiment:sterile normal saline group,TLR9 agonist,TLR9 inhibitor group,gemcitabine group,TLR9 inhibitor plus gemcitabine Bin group,TLR9 agonist plus gemcitabine.The tumor size and growth situation were recorded by the vernier caliper.The immunohistochemical method was used to detect tumor TLR9 receptor expression.The tumor growth,metastasis and paracancerous nssue invasion situation were observed by the magnetic resonance imaging (MRI).Results The volume and growth speed of resected tumor mass in the gemcitabine group,TLR9 agonist + gemcitabine group,TLR9 inhibitor plus gemcitabine group was significantly smaller than those in other groups (P＜0.05),which in the TLR9 agonist + gemcitabine group were significantly greater than those in the TLR9 inhibitor plus gemcitabine group and gemcitabine group (P＜0.05),the difference between the TLR9 inhibitor plus gemcitabine group and gemcitabine group had statistical significance (P＜0.05),while the difference among the TLR9 agonist group,TLR9 inhibitor group and normal saline group had no stastistical significance (P＞0.05).The tumor in mice at 7 weeks after planting showed oval shape with clear boundary by MRI observation,no obvious metastais and paracancerous invasion were seen in paracancerous nssues no statistically significant,5 weeks,6 weeks after planting,seven weeks mice observed in MRI,the tumor into an,state clearly that the transfer of the surrounding tissue,no significant vascular invasion,heart,liver,kidney disease.The TLR9 expression on the surface of tumor tissue was detected and identified.Conclusion Pancreatic cancer nude mouse transplated tumor has definitely positive expression of TLR9,TLR9 activation can significantly decrease the sensitivity of pancreatic cancer to chemotherapy,increases the drug resistance of tumor,on contrary promotes the tumor growth.

Objective Type A lamins are encoded by LMNA and a major component of the nuclear lamina, which have been suggested to play important roles in chromatin organization, transcription, DNA replication, and cell apoptosis. The aim of this study was to analyze the bioinformation of zebrafish lamins. Methods A phylogeny analysis was figured out with protein sequences of different species by Clustal X and MEGA 4?0 software. Then we compared the lamin protein sequences of different species with that of zebrafish by BLAST tool from NCBI. A figure of synteny analysis results was done with lamin sequence information of humans, murine and zebrafish cited from UCSC, Vega and Ensemble. Results The a?nalysis results showed that lmna, lmnb1, and lmnb2 genes of zebrafish are highly conservative and they may be homology of human LMNA, LMNB1 and LMNB2 genes. Conclusions Zebrafish lamins and human lamins have homologous sequence similarity, indicating that these two genes are orthologous genes.

BACKGROUND:As a noticeable tissue engineering material of polyhy droxyalka noates family, poly (3-hydroxybutyrate-co-4-hydroxybutyrate)(P3HB4HB) exhibitsgood biocompatibility, adhesion and mechanicalproperties, presenting aextensive application future in tissue-engineered research.
OBJECTIVE:To investigate the biocompat ibilityin vitroand ectopic osteogenic differentiationin vivoof P3HB4HB and human bone marrow mesenchymal stem cels.
METHODS:Passage 5human bone marrow mesenchymal stem cels transplanted ontothe three-dimensional P3HB4HB scaffoldwereincubated with osteogenic induction medium (test group)or with no osteogenic induction(control group), respectively. After 5-day incubation, thecelgrowth was assessed by acridine orange staining and scanning electron microscopy; after14-day incubation, both kinds of cel-scaffold composites were subcutaneously implanted into the nude mice. At 16 weeks after implantation, the cel-scaffold composites were removed to observeectopic osteogenic differentiationin vivousing hematoxylin-eosin staining, von Kossa staining and colagen type I immunohistochemical staining.
RESULTS AND CONCLUSION:Acridine orange staining showed that cels adhered wel on the surface of the scaffold;under thescanning electron microscope, induced celsgrew wel on the P3HB4HB scaffold and produced abundant extracelular matrixes. In addition, at 16 weeks after implantation, there were osteoidtissues in the test group, positive for von Kossa staining as wel as colagen type I immunohistochemical staining;furthermore, hematoxylin-eosin staining showednumerous osteoblasts and bone lacunas. In contrast, no bone tissues appeared in the control group. To conclude, P3HB4HB is a suitable material for bone tissue engineering.

Objective To explore the ability for constructing tissue engineering bone in vivo in complex scaffolds with PHB‐HHx as the scaffolds material and human umbilical cord mensenchymal stem cells (hUCMCs) as the seed cells .Methods hUCM‐SCs were inoculated into PHBHHx scaffolds to induce osteogenesis culture in vivo for two weeks ,the the induced group was the experimental group and those without instilling hUCMCs served as the control group ,the nude mouse subcutaneous implantation was performed .Then taking material at 1 ,3 ,5 months after implantation in vivo was performed for conducting HE ,collagenⅠim‐munohistochemical ,alkaline phosphatase staining and RT‐PCR .Results hUCMSCs showed good cellular adsorbability .The size and form in the experimental group basically maintained the original status ,and the osteogenesis specific indicators were positive ;but the control group did not keek the original status ,its volume was gradually shrunk until complete degradation ,and the osteogen‐esis specific indicators were negative .Conclusion The PHBHHx scaffolds combined with hUCMSCs has the capability of in vivo heterotopic constructing tissue engineering bone in nude mouse by in vitro osteogenic induction .