From Multiple Myeloma to Acute Myeloid Leukemia: A Case Report of a 61-year-old Woman after 8 Years of Chemotherapy and Immunotherapy.

BACKGROUND: As the second most prevalent hematologic malignancy, multiple myeloma (MM) affects plasma cells and is characterized by chromosomal abnormalities, particularly involving the immunoglobulin heavy chain switch region. MM represents a biologically and clinically heterogeneous hematological malignancy that serves as a clonal evolution model, exhibiting clonal heterogeneity throughout all stages from monoclonal gammopathy undetermined significance (MGUS) and smoldering multiple myeloma (SMM) to MM. Although significant progress has been made in the treatment of MM, leading to improved patient outcomes, concerns are arising regarding disease relapse due to the presence and selection of pre-existing resistant clones or selective pressure during therapy. CASE PRESENTATION: We present a case of multiple myeloma (MM) in a female patient, who underwent an 8-year course of treatment, including chemotherapy, immunomodulators, hematopoietic stem cell transplantation, CD38 monoclonal antibody, and chimeric antigen receptor T-cell (CAR-T), and was recently diagnosed with concurrent progressive MM and acute myeloid leukemia (AML). This patient has witnessed the evolution of MM treatment paradigms. CONCLUSION: In this course, disease relapses occurred twice, one of which was manifested by a light chain escape (LCE). Moreover, through the course of the disease in this patient, we review the process of clonal evolution that may be relevant.

Optimal Rituximab Monotherapy in Splenic Marginal Zone Lymphoma (SMZL): A Case Report and Brief Review.

INTRODUCTION: Splenic marginal zone Lymphoma (SMZL) is a rare, chronic B lymphocyte proliferative disease. Generally, SMZL is accompanied by circulating atypical villous lymphocytes, known as SMZL with villous lymphocytes. Rituximab is a chimeric monoclonal antibody to CD20; recent but limited studies have confirmed its effectiveness in treating SMZL. Given the low incidence and selection of treatment, statistical comparisons of rituximab monotherapy with other available treatment options with the full range of data from previous clinical studies remain sparse. Here, we report a case of SMZL with villous lymphocytes treated by rituximab monotherapy, which is especially infrequently reported. CASE REPORT: A 63-year-old Chinese female was presented to the hospital with complaints of splenomegaly and pain in the spleen area. Immunohistochemistry analysis was positive for IGH, IGK, and IGL clonal rearrangement. Villous lymphocytes were found in peripheral blood and bone marrow, along with further immunotyping results. The case was considered as SMZL with villous lymphocytes. Based on the SMZLSG prognosis assessment, we applied rituximab monotherapy. After eight cycles of rituximab treatment, the patient's condition improved markedly, with blood constituent and size of the spleen returning to normal levels, achieving complete response, with no significant side effect observed. DISCUSSION: The patient provides a typical SMZL with villous lymphocytes case treated with rituximab monotherapy. Currently, the main treatment options include splenectomy and rituximab. After synthesizing a series of current views, we put forward our opinion about the selection of therapy for SMZL patients in order to gain maximum benefits for patients in need of treatment. CONCLUSION: Our analysis found no statistically significant difference between rituximab monotherapy and rituximab combined with chemotherapy, while rituximab treatments resulted in better therapeutic effects than chemotherapy. Rituximab monotherapy has favorable therapeutic effects and minor adverse effects (AEs) in treating SMZL.

Computer simulation of the structural properties of fatty-acid modified PAMAM dendrimers at pH 5 and 7.

In this work, we performed coarse-grained molecular dynamics (CGMD) simulations of G3, G4, and G5 polyamidoamine (PAMAM) dendrimers grafting with fatty acid (FTA) chains. The FTA chains of varying length and grafting densities (50% and 100% of surface terminals) correspond to pH 7 and 5, respectively. Our findings suggested that the structural properties of dendrimers were determined by dendrimer generation, polymerization degrees, and pH. With one exception, the size of the FTA grafting dendrimer shrank after fatty acid attachment. Because of the protonation of the dendrimer's interior amines at low pH, the FTA chains are distributed at the dendrimer's surface group. At pH 7, the FTA chains that have aggregated in the interior of the dendrimer cause chain crowding. Our research provided references on drug encapsulation and the lower toxicity of these hydrophobically modified nanoparticles.

Factors Associated with Fertility Intention among Chinese Married Youth during the COVID-19 Pandemic.

Using the childbearing survey data from Hubei Province in March 2022, this article empirically analyzed the status quo of fertility intention and its influencing factors among Chinese married youth during the COVID-19 pandemic. In our analysis, fertility intention was operationalized as the ideal number of children and short-term fertility plan. Statistical results showed that the average ideal number of children stood at 1.652, which was lower than the population replacement level, whilst only 16.4% of married youth had a short-term fertility plan. By utilizing a binary logit regression model and the sheaf coefficient technique, we found that COVID-19-induced factors (i.e., change in the marital relationship during the epidemic, delayed pregnancy preparation due to vaccination) had a more stable effect on fertility intention, especially on short-term fertility planning. Parenting perception characteristics exerted a great impact on the ideal number of children but a relatively small impact on short-term fertility planning. Meanwhile, married youth with stable jobs and a high family income did not necessarily show stronger fertility intentions than those with fewer socioeconomic resources. In addition, the findings also reveal that the relative importance of fertility-influencing factors could vary at different fertile stages, which have valuable implications for population policy in Chinese contexts.

Role of humic substances and alkaline in phosphorus release from sludge pre-treated by (alkali-) hydrothermal.

The scarcity of phosphorus (P) resources makes the recovery of P urgent. Sludge is a secondary resource rich in P, and the release of P from it is a key step for recovery. Hydrothermal (HT) is currently a popular method for sludge pretreatment, and its combination with alkaline (alkali-hydrothermal, AHT) could reduce the energy consumption in treatment. This study tried to compare their P release profiles in treating activated sludge in which organic P (OP) and non-apatite inorganic P (NAIP) were co-existence. Apart from the OP release in cell lysis, P release from NAIP brought by the joint effect of OH- and humic substances (HS) formed in treatment was focused. The results showed that, compared to HT treatment, more P was released when OH- participated (AHT), and the peak P release was observed at 160 °C. Variation of P distribution in the treated sludge revealed that more P was released from NAIP in AHT than in HT. HS formed in treatments was extracted and characterized. The amount and the structure of the HS varied significantly with the treatment conditions, and there was a linear correlation ship between PO43--P release and the humic acid (HA) amount in HS. Mechanism study indicated there was a synergism between HS and OH- in promoting PO43--P release from NAIP. This study linked HS produced by sludge with P release, which provided a new perspective for subsequent P recovery from sludge.

Imbalance in the Gut Microbiota of Children With Autism Spectrum Disorders.

Background: Autism spectrum disorder (ASD) are complex behavioral changes manifesting early in childhood, which impacts how an individual perceives and socializes with others. The study aims to assess the disparities in gut microbiota (GM) amongst healthy controls and children with ASD. Methods: The study was performed on 25 children with ASD and 20 healthy children. Autistic symptoms were diagnosed and assessed with the Diagnostic and Statistical Manual for Mental Disorders and the Autism Treatment Evaluation Checklist (ATEC). Gastrointestinal (GI) symptoms were assessed with a GI Severity Index (GSI) questionnaire. The fecal bacteria composition was investigated by the high-throughput sequencing of the V3-V4 region of the 16S rRNA gene. The alpha diversity was estimated using the Shannon, Chao, and ACE indexes. The unweighted UniFrac analysis and the PCA plots were used to represent the beta diversity. LDA and LEfSe were used to assess the effect sizes of each abundant differential taxon. Results: Children with high GSI scores had much higher ATEC Total scores than those with lower GSI-scores. GI symptoms were strongly associated with symptoms of ASD. There was no difference in Chao, ACE, and Shannon indexes between ASD patients and healthy controls. Both groups showed a significant microbiota structure clustering in the plotted PCAs and significant differences in its composition at the family, order, genus, and phyla levels. There were also noteworthy overall relative differences in Actinobacteria and Firmicutes between both groups. Conclusions: This study shows the relationship between the clinical manifestations of Autistic symptoms and GI symptoms. ASD patients have dysbiosis of gut microbiota, which may be related to the onset of ASD. These findings may be beneficial for developing ASD symptoms by changing gut microbiota.

Identification of Key Genes and Pathways Associated with Hepatosplenic T-Cell Lymphoma (HSTCL) by Bioinformatics Analysis.

BACKGROUND: Prognosis of Hepatosplenic T-Cell Lymphoma (HSTCL) is very poor, while the molecular mechanism of this disease has rarely been investigated and remains mysterious. The aim of the study is to screen differentially expressed genes (DEGs) of patients with HSTCL and normal controls, explore the pathogenesis, and provide guidance for the gene diagnosis and precise treatment of HSTCL. METHODS: The genetic chip data GSE57520 of HSTCL was searched from the GEO database, and the quality control and DEGs screening were performed through BART online tools. In addition, FunRich software was used to perform gene enrichment and pathway analysis on the screened DEGs. Subsequently protein interaction network (PPI) was constructed via the STRING database and analyzed using the visual module of Cytoscape software. RESULTS: A total of 4,759 DEGs were obtained, including 2,501 up-regulated genes and 2,258 down-regulated genes (p < 0.05). The analysis of gene ontology (GO) showed that DEGs in cytology component (CC) mainly involved cytoplasm, nucleus, plasma membrane, Golgi apparatus, lysosome, and endoplasmic reticulum. Besides, DEGs in molecular function (MF) mainly included transcription factor activity, catalytic activity, transporter activity, transcription regulator activity, receptor signaling pathway complex, receptor activity. Moreover, DEGs in biological processes (BP) are mainly involved in base regulation, transport, energy pathways, metabolism, protein metabolism, and apoptosis. The results of the Kyoto Gene and Genome Encyclopedia (KEGG) analysis showed that the DEGs mainly include TRAIL, Beta1 integrin, integrin family, proteoglycan, S1P, and ErbB. Combined with Cytoscape software cytoHubba plug-in, protein interaction network (PPI) analysis showed that KIF20A, DLGAP5, PBK, TOP2A, ASPM, NEK2, KIF14, and DEPDC1B were the most abundant core genes. Module analysis showed that the three gene modules with the highest scores were mainly related to mitosis, epithelial cell adhesion and signal transduction, and the process of DNA damage. CONCLUSIONS: The DEGs of HSTCL patients versus healthy control groups were obtained through a variety of bioinformatics methods. KIF20A and DLGAP5 may become potential therapeutic targets for HSTCL. Also, the most abundant signaling pathway in DEGs was the tumor necrosis factor-related apoptosis-inducing ligand (TRAIL) - related pathway. Besides, related genes and expression characteristics of HSTCL pathogenesis were reanalyzed from distinctive perspectives, which might provide specific diagnostic markers and targeted therapy for HSTCL.

A multicenter study on the clinical features and risk factors of poor prognosis in neonatal necrotizing enterocolitis / 中华实用儿科临床杂志

Objective To explore the clinical features and risk factors of poor prognosis in neonatal necrotizing enterocolitis(NEC).Methods A retrospective study was carried out in the infants with NEC admitted to 6 cooperative hospitals in Guangdong Province between January 2005 and December 2014.The clinical features and risk factors of poor prognosis in preterm and full-term infants diagnosed NEC,early onset and late onset NEC were analyzed.Results A total of 449 cases who met the criteria were admitted during the study time.The mortality was 23.6％ (106/449 cases),of which the preterm group was 24.6％ (58/238 cases) while the full-term group was 22.7％ (48/211 cases),the early onset group was 22.1％ (45/204 cases) while the late onset group was 24.3％ (57/235 cases).The median number of NEC onset in preterm group was 11 d after birth while the number of the full-term group was 6 d.Full-term infants who diagnosed NEC were more likely to manifest themselves as abdominal distension (52.1％ vs.42.0％,x2 =4.597,P =0.032),vomiting(36.5％ vs.17.2％,x2 =21.428,P =0.000) and bloody stool(30.3％ vs.21.4％,x2 =4.653,P =0.031);but in the onset of NEC,preterm infants more likely to have feeding intolerance (21.0％ vs.12.8％,x2=5.309,P =0.021).The early onset group of full-term NEC was much common in twins or multiplets(9.4％ vs.1.1％,x2 =6.226,P =0.013),which rate of surgical therapy was much higher (41.0％ vs.27.0％,P =0.036) and the breast-feeding rate before NEC was lower than the late onset group(14.5％ vs.32.6％,x2 =9.500,P =0.002),the differences were statistically significant.The gestational age and birth weight were bigger in the early onset group of preterm NEC[(33.8 ±2.5) weeks vs.(32.2 ±2.8) weeks,t =4.261,P =0.000;(2.1 ±0.5) kg vs.(1.7 ± 0.5) kg,t =4.735,P =0.000)],but length of stay was shorter than the late onset group (18.0 d vs.26.5 d,P =0.000).Logistic regression analysis showed that the risk factors of poor prognosis of full-term NEC were shock,peritonitis and sepsis;while risk factors of poor prognosis of preterm NEC were small for gestational age infant,pulmonary hemorrhage,shock,intestinal perforation and sepsis;the risk factors of poor prognosis of the early onset group of full-term NEC was shock;while those of the late onset group were shock and peritonitis;the risk factors of poor prognosis in the early onset group of preterm NEC were shock and sepsis,while those in the late onset group were pulmonary hemorrhage,shock,intestinal perforation and sepsis.Conclusions Compared to the preterm NEC,the onset time of full-term NEC was earlier and the clinical manifestations were more typical.Early identification and management of shock,peritonitis,intestinal perforation,sepsis and pulmonary hemorrhage can reduce the risk of poor prognosis of neonate NEC.

Composition of gut microbiome in neonates with severe hyperbilirubinemia and its effect on bilirubin brain injury / 中华实用儿科临床杂志

Objective To study the composition of gut microbiome in neonates with severe bilinebinemia (serum total bilirubin ＞ 342 μmol/L),and to explore the relationship between gut microbiome and bilirubin brain injury.Methods A prospective study was conducted.The neonates with serum total bilirubin ＞ 342 μmol/L from September 2016 to March 2017 in Guangzhou Women and Children's Medical Center,Guangzhou Medical University,were enrolled in the study and 16S rDNA sequence analysis technology was used to detect the composition of gut microbiome in all subjects.According to the results of brain magnetic resonance imaging (MRI),brain stem auditory evoked potential (BAEP) and clinical manifestations,the subjects were divided into the brain injury group (26 cases) and no brain injury group (28 cases).The differences of the composition of gut microbiome between the 2 groups were compared,and the levels of unconjugated bilirubin in serum and cerebrospinal fluid were also compared.Results The level of unconjugated bilirubin in serum of the brain injury group was (463.51 ± 110.62) μmol/L,but in no brain injury group was(364.18 ±63.13) μmol/L,and there was significant difference between the 2 groups(t =4.090,P =0.000 1).The level of unconjugated bilirubin in the cerebrospinal fluid of the brain injury group was (9.53 ± 2.68) μmol/L,but in no brain injury group was (6.94 ± 2.31) μmol/L,and there was significant difference between the 2 groups (t =3.812,P =0.000 3).There was no correlation between the level of unconjugated bilirubin in the cerebrospinal fluid and serum between the 2 groups(r =0.137,0.081,all P ＞0.05).The abundance of gut microbiome in the brain injury group was lower than that in no brain injury group in genus level,among which Fusobacterium,Catabacter,Succinivibrio,Clostridium and Bacteroides were significantly different (all P ＜ 0.05).Conclusions The occurrence of bilirubin brain injury depends on the level of unconjugated bilirubin in serum cerebrospinal fluid,but it may be more directly dependent on the level of bilirubin in the cerebrospinal fluid.The diversity of gut microbiome in neonates with bilirubin brain injury was significantly lower than that in no brain injury group.The level of unconjugated bilirubin in cerebrospinal fluid may be related to the different blood-brain barrier permeability caused by different composition of gut microbiome.

Therapeutic effect of placenta-derived mesenchymal stem cells on hypoxic-ischemic brain damage in rats.

BACKGROUND: Oxidative stress is involved in the development of hypoxic-ischemic brain damage (HIBD). In this study, we investigated the therapeutic effects of placenta-derived mesenchymal stem cells (PD-MSCs) and explored the NF-E2-related factor-2/heme oxygenase-1 (Nrf2/HO-1) signaling pathway in treating HIBD. METHODS: P7 rats were subjected to hypoxic-ischemic brain injury and randomly divided into four groups (control, HIBD, HIBD+PD-MSCs, and HIBD+fibroblasts). Forty-eight hours after the induction of HIBD, 5×10(5) of PD-MSCs were injected into cerebral tissue in the HIBD+PD-MSCs group, while the same dose of fibroblasts were injected in the HIBD+fibroblasts group. Morris Water Maze, gross and pathological changes were tested at P28. The level of malondialdehyde (MDA) was detected in rats' hippocampus. RT-PCR and western blot analysis were used to evaluate the changes of Nrf2/HO-1. RESULTS: The HIBD group showed significantly longer escape latency and a lower frequency of original platform crossing in the Morris Water Maze compared with the control group. Rats receiving PD-MSCs showed significant improvement of HIBD. The pathological changes were evident after HIBD, but ameliorated in the PD-MSCs group. Compared with the control group, HO-1 and Nrf2 were up-regulated at gene and protein levels in the HI brain, beginning at 6 hours and peaking at 48 hours (P<0.05). The expression of HO-1 and Nrf2 in the PD-MSCs treatment group was more pronounced than in the HIBD group (P<0.01). PD-MSCs also decreased MDA production in the brain tissue. CONCLUSION: These results demonstrate that PD-MSCs have neuroprotective effect during the treatment of HIBD and that the mechanism may be partly due to alleviating oxidative stress.

Clinical analysis of neonatal congenital cystic lung lesions in 28 cases / 中国小儿急救医学

Objective To explore the clinical characteristics of neonatal congenital cystic lung lesions. Methods Between January 2008 and June 2014,total 28 cases diagnosed congenital cystic lung lesions in neonatal center of Guangzhou Women and Children's Hospital were collected.The clinical data were analyzed including manifestations,lesion characteristics,imaging,diagnosis,treatment and prognosis,and the related literature were reviewed.Results There were 20 male and 8 female,16 cases of lobar emphysema,7 cases of pulmonary seques-tration,4 cases of congenital cystic adenomatoid malformation and 1 case of bronchogenic cyst.Main symptoms were dyspnea(78.57%),cyanosis (39.29%),wheezing cough (17.86%),feeding difficulties (14.29%),fever (10.71%),asymptomatic(21.43%).Two cases combined with congenital heart disease,4 cases combined with other malformations(such as diaphragmatic hernia,laryngeal stridor,funnel chest,polycystic kidney).There were lesions in chest CT image,4 cases underwent lobectomy,other cases underwent conservative treatment.Seventeen cases got better and discharge,3 cases dead.Conclusion The most common type of congenital cystic lung lesions in neonate is lobar emphysema.Main manifestations are dyspnea,cyanosis.If we suspect congenital cyst-ic lung lesions,we should do chest CT or MRI scan soon ,also do echocardiography and ultrasound to exclude other malformations.The treatment effect is satisfactory,and the outcome need long time to follow up.

Clinical analysis of neonatal purulent meningitis caused by group B streptococcus / 中国小儿急救医学

Objective To explore the clinical characteristics and outcome of group B streptococcus (GBS) induced neonatal meningitis and to provide the guide for early diagnosis and appropriate treatment.Methods A retrospective chart review was performed.A total of 19 cases of neonatal purulent meningitis caused by GBS and 22 cases of neonatal purulent meningitis caused by Escherichia coli were identified in the NICU of Guangzhou Women and Children's Medical Center from Nov 1,2011 to Apr 31,2014.The clinical features,treatments and clinical turnover were analysed.Results GBS meningitis accounted for 24.7％ (19/77) of total bacterial positive cultures of blood or cerebral spinal fluid.The average time of progression to early-onset GBS meningitis of 6 early-onset cases mainly complaining of anhelation and groan,was (11.80 ± 11.34)h,and 83.3％ present within 24 hours;the main initial clinical symptoms of 13 late-onset cases[mean age (17.85 ± 7.77) d] were fever.Peripheral blood C-reactive protein concentration of GBS meningitis was significantly higher than that of Escherichia coli meningitis [(154.43 ± 88.64) mg/L vs.(67.52 ± 64.23) mg/L,P =0.001].Compared with Escherichia coli meningitis,the average length of stay in hospital and the recovery time of abnormal cerebral spinal fluid in neonates with GBS infection were both extended by more than 10 days.Conclusion The clinical manifestations of neonatal purulent meningitis caused by GBS are usually non-specific.It is associated with longer hospitalization and recovery time of abnormal cerebral spinal fluid.Antepartum prophylaxis,early diagnosis and therapy are vital for reducing the incidence of complications and mortality of neonatal GBS purulent meningitis.

A meta-analysis of Chinese herbal medicines for vascular dementia.

OBJECTIVE: To investigate the efficacy and safety of Chinese herbal medicines in the treatment of patients with vascular dementia. DATA RETRIEVAL: We retrieved publications from Cochrane Library (2004 to July 2011), PubMed (1966 to July 2011), the Chinese Science and Technique Journals Database (1977 to July 2011), the China National Knowledge Infrastructure (1979 to July 2011), Google Scholar (July 2011), and the Chinese Biomedical Database (1977 to July 2011) using the key words "Chinese medicine OR Chinese herbal medicine" and "vascular dementia OR mild cognition impair OR multi-infarct dementia OR small-vessel dementia OR strategic infarct dementia OR hypoperfusion dementia OR hemorrhagic dementia OR hereditary vascular dementia". SELECTION CRITERIA: Randomized controlled trials comparing Chinese herbal medicines with placebo/western medicine in the treatment of patients with vascular dementia were included. Diagnostic standards included Diagnostic and Statistical Manual of Mental Disorders-IV, and National Institute of Neurological Disorders and Stroke and Association Internationale pour la Recherché et l'Enseignement en Neurosciences. Two participants independently conducted literature screening, quality evaluation and data extraction. The quality of each trial was assessed according to the Cochrane Reviewers' Handbook 5.0. MAIN OUTCOME MEASURES: Effective rate, Mini-Mental State Examination scores, Hasegawa Dementia Scale scores, and incidence of adverse reactions. RESULTS: We identified 1 143 articles discussing the effects of Chinese medicine on vascular dementia. Thirty-one of these were included in the analysis. These studies involved a total of 2 868 participants (1 605 patients took Chinese medicine decoctions (treatment group); 1 263 patients took western medicine or placebo). The results of our meta-analysis revealed that Chinese herbal remedies in the treatment group were more efficacious than the control intervention (relative risk (RR) = 1.27; 95% confidence interval (CI): 1.18-1.38, P < 0.01). Mini-Mental State Examination scores were higher in patients taking Chinese herbal medicines than in those in the control group (weighted mean difference (WMD) = 2.83; 95%CI: 2.55-3.12, P < 0.01). Patients in the treatment group showed better disease amelioration than those in the control group (Hasegawa Dementia Scale scores; WMD = 2.41, 95%CI: 1.48-3.34, P < 0.01). There were also considerably fewer adverse reactions among those in the treatment group compared with those in the control group (RR = 0.20, 95%CI: 0.08-0.47, P < 0.01). CONCLUSION: Chinese herbal medicine appears to be safer and more effective than control measures in the treatment of vascular dementia. However, the included trials were generally low in quality. More well-designed, high-quality trials are needed to provide better evidence for the assessment of the efficacy and safety of Chinese medicines for vascular dementia.

Seven cases of neonatal Prader-Willi syndrome and a literature review / 中华实用儿科临床杂志

Objective To know more about the features of Prader-Willi syndrome (PWS) in neonates and to promote early interventions for PWS patients.Methods The clinical data from 7 PWS patients being definitely diagnosed by array comparative genomic hybridization(array-CGH) from Jan.2007 to Dec.2012 in Department of Neonatology,Guangzhou Women and Children's Medical Center were collected retrospectively.The data were analyzed with literature being reviewed.Results There were 7 cases described in this study,of which 6 cases were male and 1 case was female.All patients had the features of hypotonia,poor responses,feeding difficulties,less crying or weak crying.Seven cases had 5 or more of the following distinctive facial appearances:prominent forehead,narrow face,almond eyes,small mouth,downturned mouth corners,thin upper lip,and micromandible.Six cases of male children were small scrotum,among them 3 cases were cryptorchidism.Seven cases were diagnosed as PWS by array-CGH.Seven children were fed by using gastric tube,in which 4 cases accepted swallow training after admission,and their gastric tube lasted from 8 to 20 days [(13.0 ±5.1) d],3 cases did not receive continuous tube feeding but swallow training them of from 15 to 35 days [(18.0 ± 4.3) d].Other comprehensive therapy measures included offering the parents a detailed health education,informing and their gastric tube lasted the basic information about the disease,teaching the parents how to facilitate feeding and prevent asphyxia and other aspects of skills.This might increase the baby's passive activities and promote normal development of the baby's nutritional management measures to prevent excessive or inadequate nutritional intake.Multidisciplinary consultation was necessary including the intervention of mass language training,nutrition,neurology,psychiatry,psychology and osteology.Conclusions PWS newborns have the characteristics of hypotonia,poor responses,feeding difficulties,less crying or weak crying,genital hypoplasia and typical facial features.Genetic testing should be necessary for early diagnosis,as early diagnosis is benefitial for early intervention including comprehensive treatment measures such as swallowing training,which may improve the life quality of PWS patients,and improve the prognosis,and prevent growth retardation,obesity and other problems.

Clinical analysis of 17 cases of neonatal Kasabach-Merritt syndrome / 国际儿科学杂志

Objective To summarize the clinical characteristics and therapeutic approaches of Kasabach-Merritt syndrome (KMS).Methods The data from 17 KMS patients that had been definitely diagnosed from December,2007 to January,2012 in our tertiary center were collected retrospectively.Results We described 17 patients,13 of whom were male and 4 of whom were female,with an age range of 17 hours to 28 days.Of the 17 cases,4 cases had hemangioma of internal organs,13 on the surface of the body.All of them had thrombocytopenia and coagulation dysfunction.Seventeen cases were initially treated with corticosteroids.The responses were varied:excellent and rapid improvement (n =6),failure (n =11),recurrence (n =3).Then the cases of failure and recurrence accepted artery embolization:excellent and rapid improvement (n =8),failure (n =4),recurrence (n =1),with 1 giving up.At last,the cases resistant to artery embolization therapy accepted vincristine therapy.Four cases had rapid improvement,and 1 died from disseminated intravascular coagulation.In this study,a response rate to corticosteroids was 35.3％,and the recurrent rate was 50％.The response rate to artery embolization was 61.5％.Five patients unresponsive to hormone therapy and artery embolism were treated with vincristine,and the effective rate was 80％.Conclusion In the therapy of neonatal KMS,the resistant to corticosteroids is common.Combinative therapy of corticosteroids with artery embolization is recommended as the first-line therapy.Vincristine is suggested as a therapy when there is resistance to the other therapy.

[In vitro effects of Wnt3a gene modification on mitigating damage of mouse bone marrow mesenchymal stem cells induced by Ara-C].

This study was aimed to investigate the protective effect of Wit3a gene modification on mouse bone marrow mesenchymal stem cells against the injury induced by Ara-C. The gene-modified MSC steadily expressing Wnt3a were established by adenovirus system. The acute direct damage effects of different concentrations of Ara-C on the unmodified MSC and the gene-modified MSC were assessed by using an in vitro culture system, and the corresponding controls were set. The proliferation and apoptosis of MSC exposed to Ara-C were detected by cell count kit-8 (CCK-8) and flow cytometry. The expression of BCL-2 protein related with cell apoptosis was assayed by Western blot. The results indicated that as compared with unmodified MSC, Ara-C exhibited a less inhibitory effect on the proliferation of gene-modified MSC. There was obvious difference between unmodified MSC and gene-modified MSC (p < 0.05). The proliferation of gene-modified MSC began to recover at 72 hours after removal of Ara-C. However, unmodified MSC showed sustained suppression of proliferation after withdrawal of Ara-C. In apoptosis, the apoptosis rate of gene-modified MSC induced by Ara-C was significantly lower than those of unmodified MSC (p < 0.05). In addition, the expression levels of BCL-2 protein in gene-modified MSC were up-regulated compared with unmodified MSC (p < 0.05). It is concluded that Wnt3a gene modification can significantly mitigate the damage of mouse bone marrow MSC induced by Ara-C.

[Effect of Wnt3a-transduced bone marrow mesenchymal stem cells on the proliferation of T lymphocytes].

OBJECTIVE: To observe the effect of Wnt3a-transduced mouse bone marrow mesenchymal stem cells (MSC) on the proliferation of T lymphocytes. METHODS: MSC were isolated from C57BL/6 mouse bone marrow and expanded in vitro, then identified by flow cytometry and their differentiation capacity into osteocytes and adipocytes were determined. Recombinant plasmids containing Wnt3a gene, were transfected with lipofectamine into HEK293 cells by the AdEasy system. Viral particles were collected to infect MSC and adenovirus vector expressing GFP (Ad-GFP) was used as control. The expression of GFP in MSC was observed using fluorescence microscopy and the protein levels of Wnt3a and ß-catenin were determined by Western blot. Wnt3a-transduced and Ad-GFP transduced MSC were separately cocultured with spleen lymphocytes stimulated by ConA, at the ratio of 1:100, 1:50 or 1:10 respectively. The proliferation rate of T lymphocytes was estimated by Cell Cout Kit-8 (CCK-8) and the level of cytokine by ELISA. RESULTS: FCM analysis showed that the MSC were highly positive for CD90.2, CD44 and negative for CD34, CD45, they could differentiate into osteoblasts and adipocytes after induction; The titer of recombinant adenoviruses was up to 1 × 10(10) pfu/ml. After infected with the adenoviruses, MSC had the strongest GFP expression at 72 h and the efficiency of infection was 50%-60%. The expressions of Wnt3a and ß-catenin protein in the Wnt3a-transduced MSC were significantly increased. MSC could suppress the proliferation of T lymphocytes in a dose-dependent manner. When MSC cocultured with spleen lymphocytes at 1:10 ratio, T lymphocyte proliferation rate and the level of IFN-γ were (55.41 ± 1.75)% and (326.70 ± 14.41) pg/ml respectively in Ad-GFP transduced MSC group, while in Wnt3a-transduced MSC group, they were (37.27 ± 2.66)% and (218.80 ± 12.93) pg/ml respectively. There was no effect on the production of IL-2. CONCLUSION: Compared to Ad-GFP transduced MSC, Wnt3a-transduced MSC exhibit a more potent inhibitory effect on the proliferation of T lymphocytes.

[Effectiveness of Nao'an Capsule on stroke prevention among high risk population in Nanhui, Shanghai].

OBJECTIVE: To evaluate the effectiveness of stroke prevention among high risk population, using Nao'an Capsules. METHODS: Participants were selected from 696,558 residents in Nanhui, using county of Shanghai city. Individuals aged 35 years old and over with at least one risk factor exposure to stroke, received cerebral vascular hemodynamic examination. 18,271 cases meeting the criteria of cerebral vascular hemodynamic indexes accumulative score below 70 points were defined as individuals with high-risk and targets to receive intervention. According to the willingness of the participants, 10,313 cases received Nao'an Capsules for intensive intervention based on general intervention measures compared to 7958 cases only receiving general intervention. After the implementation of intervention, incidence and mortality rates of stroke and the effectiveness of three-year intervention were studied. RESULTS: Incidence of stroke in Nao'an Capsules group was significantly lower than that of the general intervention (P < 0.01) with 53.8% in males and 58.4% in females. The relative risk (RR) in two gender groups were 0.46 (0.33 - 0.64) and 0.39 (0.30 - 0.50) respectively. Multiple logistic regression analysis indicated that the history of hypertension, accumulative score of cerebral vascular hemodynamic indexes, age, gender and Nao'an Capsules intervention were the variables selected into the equation and significantly related to stroke. Among the variables, Nao'an Capsule was the strongest factor with a RR of 0.41. CONCLUSION: Incidence of stroke in Nao'an Capsule intervention group was significantly lower than that in the general intervention group after 3 years of intervention, suggesting that Nao'an Capsule intervention was the strongest factor affecting stroke occurrence in individuals at high-risk.