Huntington's disease (HD) is an autosomal dominant inherited disease with insidious onset and slow progression, mainly characterized by chorea-like symptom, intelligence decline, and psychiatric abnormalities. Cause of the disease is abnormal expansion of CAG trinucleotide repeat sequences in the first exon of the Huntington gene (HTT) on chromosome 4. Despite the clear etiology, currently, no effective therapeutic measures to control the disease progress is noted, and symptomatic treatment is still the main treatment in clinical practice. This article provides a brief overview of the current clinical trials, clinical challenges, and future development of HD gene therapy to provide references for subsequent related research.

This review aims to interpret the interventions to improve physical function for children and young people with cerebral palsy, thus providing relevant suggestions.Relevant literatures published before November 2018 were systematically searched in Cochrane library, CINAHL, and Embase MEDLINE using the Grading of Recommendations Assessment, Development and Evaluation method.Consult with international experts and patients to assess evidence and recommend it.Based on 3 systematic reviews, 30 randomized clinical trials, and 5 pre-and post-treatment studies, a total of 13 recommendations were given.The guidelines recommend that, in order to achieve functional stan-dards, intervention measures should include, patient-selected goals, full-task practice in real-life settings, support for family empowerment, and a team approach.The age, ability, and child/family preferences were all needed to be considered.In order to improve walking ability, ground walking and treadmill training can be carried out.Various methods can promote the realization of hand use goals hand use, including the two-hand exercise training, constraint-induced moverment therapy, goal-oriented training and cognitive therapy.In terms of patient self-care, the guideline proposed that, the combination of full-task practice and auxiliary equipment can improve the independence of self-care and reduce the burden of care givers.Leisure goals could be achieved by the combination of the practice of the entire task with strategies to address environmental, personal, and social barriers.The intervention of children and adolescents with cerebral palsy should take into consideration of patient selection and the goal of full-task practice.The child/family preference, age and ability should be considered when clinical workers selecting specific interventions.

Brachytherapy ; Carcinoma, Hepatocellular/drug therapy* ; Chemoembolization, Therapeutic ; Combined Modality Therapy ; Humans ; Iodine Radioisotopes ; Liver Neoplasms/drug therapy* ; Portal Vein ; Retrospective Studies ; Sorafenib/therapeutic use* ; Thrombosis ; Treatment Outcome

Developmental coordination disorder (DCD) is a developmental disorder that mainly affects children′s motor coordination.The prevalence of DCD in childhood is very high, and it seriously affects the physical and mental health of school-age children.In China, this disease has not yet been fully recognized and understood.There is a lack of standardized and unified diagnostic criteria, and the standardization of relevant clinical assessment tools and the establishment of normative models are insufficient.In addition, the clinical practice and experience of most health professionals in dealing with DCD appear to be limited.Based on international clinical practice recommendations on the definition, diagnosis, assessment, intervention, and psychosocial aspects of DCD issued by the European Academy of Childhood Disability in 2019, the recommendations related to the diagnosis, evaluation and intervention of DCD in the guidelines were compiled and interpreted in the light of the current clinical practice and research in China.The objective of this study is to bring DCD to the attention of more domestic medical experts through the interpretation of this international guideline and to provide preliminary guidance on the clinical diagnosis, assessment, and intervention of DCD.

Objective To investigate the epidemiological characteristics of cerebral palsy(CP)in children aged 1-6 years in China,including the incidence,prevalence,type of CP,etiology,prevention and rehabilitation status. Methods The survey was carried out by standard questionnaires,multi-center collaboration,stratified-cluster ran-dom sampling method.The surveyed adopted the following principles:streets in the city and villages in the rural areas, and the number of the urban and rural children was the same,and the proportion of children in each age group was balanced.The investigation areas included provinces and autonomous regions,including Heilongjiang,Beijing,Henan, Shandong,Shanxi,Shaanxi,Anhui,Hunan,Guangxi,Guangdong,Chongqing and Qinghai,and 323 858 children were in-vestigated.Results The incidence of CP was 2.48‰(155/62 591 cases),and the prevalence was 2.46‰(797/323 858 cases)(1-6 years old).The prevalence varied in different regions,in which the highest prevalence was 5. 40‰(54/9 998 cases)in Qinghai province,and the lowest prevalence was 1.04‰(47/45 133 cases)in Shandong province.The prevalence of the males(2.64‰,461/174 391 cases)was higher than that of the females(2.25‰, 336/149 467 cases),and the difference was statistically significant(P<0.05).The types of CP were spastic type (58.85%,469/797 cases),mixed type(13.17%,105/797 cases),dyskinetic(9.79%,78/797 cases),hypotonic (8.28%,66/797 cases),ataxia(6.25%,52/797 cases)and rigid(3.39%,27/797 cases)respectively in 797 CP children.The first three risk factors for CP were long -term exposure to harmful physical factors during pregnancy, whether there were birth defects among the three generations of relatives of the children,such as children's peers, parents or grandparents,whether there were birth defects among the children's peers,parents or grandparents,and neonatal jaundice or persistent jaundice.Among 797 CP children,79.67% of the children with CP were timely detected and treated in the local hospitals,while the other 19.93% of them were not timely treated.The places which could give them timely detection and early diagnosis and treatment were general hospitals(42.97%),Maternity and Infant Hospitals (27.03%)and Children's Hospitals(20.31%). The main rehabilitation methods for 797 children with CP were 34.58% in the hospitals or rehabilitation centers,31.61% in the communities(including at home),33.80% mainly in the medical institution,and in the communities they could also receive partially rehabilitation services. Conclusions The prevalence of CP in China is coincident with international levels.The prevalence rate of CP in males is higher than that in females.The types of CP distribution are accorded with international distribution characteristics.There were still some children with CP who could not receive timely detection and treatment.Rehabilitation at the medical institutions is the chief way and proper rehabilitation guidance should be carried out in the communities.

Autism spectrum disorders (ASD) is a neurodevelopmental condition characterized by altered social communication,alongside restricted and stereotyped behaviors and interests,bringing a heavy burden to the family and society.International experts develop the International Classification of Functioning,Disability and Health (ICF) core sets for individuals with ASD.The ICF core sets for ASD will be a tool to describe individual functioning comprehensively.

In order to optimize the teaching process, improve the teaching quality and cultivate of emergency nursing talents with comprehensive quality, we systematically designed the teaching process of foreign body airway obstruction first aid. From the design ideas and curriculum innovation, we focused on the use of a variety of teaching methods, and we combined it with modern information technology, which demonstrated the practicability and operability of first aid skill. This teaching practice has proved that the systematically teaching reform design is helpful to improve the learning efficiency of nursing students and cultivate the comprehensive quality of students' emergency response ability.

Objective To explore the effects ofBuzhong Yiqi Pills on HBV-related decompensated liver cirrhosis patients.Methods A total of 176 patients of HBV-related decompensated liver cirrhosis were enrolled in the study from January 2007 to January 2010 and were divided into treatment group (82 cases) and control group (94 cases) according to patient's wishes. Patients in both groups were given antiviral therapy. According to the liver function and complications, patients were given glycyrrhizin to protect liver, Kuhuang Injection to treat jaundice, and spironolactone and furosemide for diuretic treatment. Patients in the treatment group receivedBuzhong Yiqi Pills, one bag for each time, twice a day, four weeks as a treatment session, three sessions each year, with five-year follow-up. Effects ofBuzhong Yiqi Pills on the hepatorenal function, blood coagulation, blood routine, complications and survival rate in patients with decompensated liver cirrhosis were observed.ResultsBuzhong Yiqi Pills could effectively improve the hepatorenal function, blood routine and coagulation disorders of HBV-related decompensated liver cirrhosis patients (P<0.05,P<0.01). The rate of complications with hydrothorax and ascites (46.34% vs. 88.30%), upper gastrointestinal hemorrhage (39.02% vs. 69.15%), infection (31.71% vs. 57.45%), hepatic encephalopathy (23.17% vs. 54.26%), hepatorenal syndrome (6.10% vs. 18.09%) and chronic hepatic failure (9.76% vs. 25.53%) in the treatment group and the control group were with statistical significance (P<0.05,P<0.01). The five-year survival rates were significantly higher in the treatment group (79.27%) compared with the control group (64.89%), with statistical significance (χ2=5.353,P=0.021).ConclusionLong term use ofBuzhong Yiqi Pills can significantly decrease the complications of HBV-related decompensated cirrhosis and improve survival rate of patients.

Objective To explore the clinical significance of the serum brain?derived neurotrophic factor( BDNF) level in severe neonatal hyperbilirubinemia. Methods One hundred and twenty term and birth weight>2500 g infants admitted to the Neonatal Intensive Care Unit of Bethune nternational Peace Hospital of People Liberation Army were divided into severe hyperbilirubinemia group and control group according to their total bilirubin concentration. Total bilirubin( TBIL) concentration,BDNF and albumin in serum were determined in two groups. In addition, craniocerebral MRI was performed in severe neonatal hyperbilirubinemia before discharge. The correlation of the BDNF, TBIL, B/A, MRI results between severe hyperbilirubinemia group and control group were compared. Results The serum BDNF levels in severe hyperbilirubinemia group was ( 8. 84 ±3. 26) μg/L,significantly higher than that in control group((6. 24±1. 71) μg/L,t=3. 88,P<0. 05). In severe hyperbilirubinemia group,BDNF level was positively correlated with B/A level(r=0. 429,P<0. 05),but there was no correlation between BDNF and total bilirubin level(r=0. 278,P>0. 05). The serum BDNF level with craniocerebral MRI abnormal was ( 9. 53 ± 2. 77 ) μg/L, higher than that with craniocerebral MRI abnormal ((7. 81±3. 76) μg/L),but there was no statistical difference between them(t=1. 439,P>0. 05). Conclusion In severe neonatal hyperbilirubinemia, the body can secrete BDNF increasely. BDNF level is positivelycorrelated with B/ A level. As a marker of brain damage,BDNF is sensitive than craniocerebral MRI.

Secretory diarrhea provides a major health challenge worldwide, which is one of the most important reasons for children morbidity and death. The activation of Cl- channels in intestinal epithelial cells resulting in the excessive fluid secretion in the intestine is the main reason of diarrhea caused by enterotoxins. In diarrhea caused by cholera and the other bacterial enterotoxins, cystic fibrosis transmembrane conductance regulator ( CFTR) is the main cAMP-control Cl- channel to promote the fluid secretion in epithelial cells. Therefore, CFTR inhibitors are the new choices for secretory diarrhea. CFTR inhibitors include thiazolidinone, glycine hydrazide and quinoxalinedione chemical classes, and some components from natural plants also exhibit CFTR inhibition activity, however, further studies should be done.