Tourette's syndrome: a treatable tic.

Tourette''s syndrome, or Gilles de la Tourette''s disease, is a disorder characterized by involuntary tic-like muscular movements, compulsive behaviour and involuntary vocalization of sounds, words or profanities. It begins in childhood and may persist for life, with a varied pattern and course. Recent studies indicate an organic basis for the disorder, and an abnormality of dopamine or purine metabolism has been suggested. The treatment of choice is haloperidol administration; most patients do well with low or moderate doses for long periods. Because these patients are often mistakenly regarded as anxious, psychoneurotic or hysterical, correct diagnosis is important if they are to be treated appropriately and regarded properly in the home, school and society.     

Withholding the artificial administration of fluids and food from elderly patients with dementia: ethnographic study

ObjectiveTo clarify the practice of withholding the artificial administration of fluids and food from elderly patients with dementia in nursing homes.DesignQualitative, ethnographic study in two phases.Setting10 wards in two nursing homes in the Netherlands.Participants35 patients with dementia, eight doctors, 43 nurses, and 32 families.ResultsThe clinical course of dementia was considered normal and was rarely reason to begin the artificial administration of fluids and food in advanced disease. Fluids and food seemed to be given mainly when there was an acute illness or a condition that needed medical treatment and which required hydration to be effective. The medical condition of the patient, the wishes of the family, and the interpretations of the patients'' quality of life by their care providers were considered more important than living wills and policy agreements.ConclusionsDoctors'' decisions about withholding the artificial administration of fluids and food from elderly patients with dementia are influenced more by the clinical course of the illness, the presumed quality of life of the patient, and the patient''s medical condition than they are by advanced planning of care. In an attempt to understand the wishes of the patient doctors try to create the broadest possible basis for the decision making process and its outcome, mainly by involving the family.

What is already known on this topic

Debate has focused on whether it is beneficial to withhold the artificial administration of fluids and food from patients with advanced dementia

What this study adds

The course of dementia, the patient''s quality of life, and the patient''s current medical condition influence doctors'' decision making more than advanced planning of careDoctors try to create the broadest possible basis for the decision making process and its outcome, mainly by involving the family     

Volunteers,doctors take palliative care into the community.

The face of palliative care is changing. In Ontario''s St. Catharines region there has been a concerted effort to make it more of a community-based procedure. A local college even teaches a 2-year course in palliative care. The trend is expected to continue because Canadians are living longer, and more frail elderly people will be dying at home. Dr. Sandra Hartman, a palliative-care consultant, says physicians interested in palliative care must remember that there is more to it than providing medical assistance. She considers bereavement counseling for the patient''s family a necessary part of follow-up preventive care.     

Pet Ownership in Childhood and Socio-Emotional Characteristics,Work Values and Professional Choices in Early Adulthood

ABSTRACT

The main goal of this study, partly retrospective in character, was to explore if and how pet ownership in childhood is related to several indicators of socio-emotional development, as well as work values and professional choices in early adulthood. The sample consisted of 356 students (200 females, 156 males) from different colleges of the University of Zagreb, representing helping (n=200) and non-helping (n=156) professions. The mean age of the students was 21 years. Seventy-four percent of the participants had had a pet during childhood, mostly dogs. Participants who had owned a pet during childhood reported quite a strong attachment to it. In addition, pets were rated lower than family members and friends, but higher than television, relatives and neighbors in terms of the social support derived from them during childhood. Discriminant analysis was performed in order to examine whether young adults – those who had owned a pet during childhood and those who hadn't – differed in emotional and motivational characteristics, and whether there was a correlation with their chosen subject of study. Pet ownership in childhood was a grouping variable, while measures of current socio-emotional functioning (empathy, prosocial behavior, social anxiety and loneliness), value orientations (self-actualisation, individualistic, social, utilitarian and adventurous) and chosen subject of study (helping or non-helping profession) were predictors. A significant discriminant function was obtained. Correlations between discriminating variables and discriminant function showed that young adults who had had a pet during childhood were more empathetic, more prone to choose helping professions, and more oriented towards social values than those who did not have a pet during childhood.     

THE RATE OF SENESCENCE OF THE DOMESTIC FOWL AS MEASURED BY THE DECLINE IN EGG PRODUCTION WITH AGE

Data are presented showing that the course of decline of egg production with age in the domestic fowl from the time laying begins up to and including 8 years follows an exponential law, that is, each year''s egg production is a constant percentage of the preceding year''s production (88 per cent in the group of fowl studied). Since the exponential law is the same as the law of monomolecular change in chemistry, and since the course of egg production with age may be taken as an index of the course of senescence of organs, or tissues limiting egg production, it is suggested that this exponential law of egg production substantiates the idea that senescence is a physicochemical process the course of which is limited by a chemical reaction. It is shown that the exhaustion of the oocytes is not likely to be the factor limiting the course of egg production.     

Child–Companion Animal Attachment Bonds in Single and Two-Parent Families

ABSTRACT

This study addressed the issue of whether children who potentially have reduced access to parental resources have a stronger attachment bond with a dog, compared with children who have greater access. The study compared children in single-parent families with those in two-parent families on level of attachment to their family dog. Parents judged children's level of attachment to dogs by completing the Companion Animal Bonding Scale. The children's ages ranged between three and twelve years. Overall, children in single-parent families did show significantly higher levels of attachment to dogs than children in two-parent families. A comparison of attachment to dogs across family type showed that children in the early childhood stage in single-parent families had significantly higher levels of bonding with dogs than children in the early childhood stage in two-parent families. For the middle childhood stage there was no significant difference between family type and attachment to dogs. Comparisons within family type showed mixed results, and there were no significant gender differences in attachment levels across family type. Findings are discussed in light of attachment theory, family systems theory and implications for child development.     

Nonmarital Fertility,Family Structure,and the Early School Achievement of Young Children From Different Race/Ethnic and Immigration Groups

Working from a life course perspective, this study examined the links between mothers' fertility and relationship statuses and children's early school achievement and how these links varied by race/ethnicity and immigration status. Analyses of nationally representative data from the Early Childhood Longitudinal Study–Kindergarten Cohort revealed that children born to unmarried women scored lower than children of married women on math tests in kindergarten and first grade. This pattern was most attributable to associated differences in family income and parent education, and it was moderated by women's marital and relationship statuses after having their children. Evidence also suggested that the academic risks of some family structure pattern relative to continuously married parents might have been more pronounced for White children.     

Chronic inflammatory bowel disease in childhood.

The diagnosis of Crohn''s disease in childhood has been facilitated by the use of fibreoptic endoscopy with biopsies, complemented by double-contrast radiology. Clinical suspicion leads initially to several relevant blood tests. These are followed by endoscopy and multiple colonic biopsies or barium follow-through studies depending on whether large-bowel or small-bowel disease is suspected. The present approach to diagnosis is based on corroborative investigative techniques-endoscopy, radiology, and histology, The availability of paediatric colonoscopes of small diameter should make it possible for paediatricians to perform limited examinations, but when more extensive endoscopy is indicated the child should be referred to special centres.     

NCL diseases — clinical perspectives

The neuronal ceroid lipofuscinoses (NCLs) are lysosomal storage disorders and together are the most common degenerative brain diseases in childhood. They are a group of disorders linked by the characteristic accumulation of abnormal storage material in neurons and other cell types, and a degenerative disease course. All NCLs are characterized by a combination of dementia, epilepsy, and motor decline. For most childhood NCLs, a progressive visual failure is also a core feature. The characteristics of these symptoms can vary and the age at disease onset ranges from birth to young adulthood. Genetic heterogeneity, with fourteen identified NCL genes and wide phenotypic variability render diagnosis difficult. A new NCL classification system based on the affected gene and the age at disease onset allows a precise and practical delineation of an individual patient's NCL type. A diagnostic algorithm to identify each NCL form is presented here. Precise NCL diagnosis is essential not only for genetic counseling, but also for the optimal delivery of care and information sharing with the family and other caregivers. These aspects are challenging because there are also potential long term complications which are specific to NCL type. Therefore care supported by a specifically experienced team of clinicians is recommended. As the underlying pathophysiological mechanism is still unclear for all NCL forms, the development of curative therapies remains difficult. This article is part of a Special Issue entitled: The neuronal ceroid lipofuscinoses or Batten Disease.     

THE CHILD'S SEXUAL LIFE—A Consideration of Clinical Implications

A healthy sexual life begins in childhood, and the groundwork for later difficulties also begins in childhood. For the child, sexuality is a rather general, pleasurable excitation, not a specific genital stimulation. Excessive attention to the perineal area, operation upon or injury to the genital area and injections are vicissitudes which may have an unhealthy influence on later sexual development. Healthy and informed parental attitudes are the key here, just as they are in the normal exhibitionism, curiosity and intense emotional attachments to parents. Parents infect children, healthily or unhealthily, with their attitudes. In healthy growth exhibitionism and peeping become transformed in socially acceptable ways. In unhealthy growth, either perversions or strong reactions like over-modesty or shame result. Masturbation is common and transitory in most children. Parents, and especially pediatricians to whom parents turn, have a golden opportunity to direct healthy growth by being well informed about the infant and child''s sexual growth and thus be enabled to advise upon or manage the common developmental phenomena with good commonsense and patience. Infants and children do not enter the world possessing the morals, standards or inhibitions of adults.     

The contribution of vaccination to global health: past,present and future

Vaccination has made an enormous contribution to global health. Two major infections, smallpox and rinderpest, have been eradicated. Global coverage of vaccination against many important infectious diseases of childhood has been enhanced dramatically since the creation of WHO''s Expanded Programme of Immunization in 1974 and of the Global Alliance for Vaccination and Immunization in 2000. Polio has almost been eradicated and success in controlling measles makes this infection another potential target for eradication. Despite these successes, approximately 6.6 million children still die each year and about a half of these deaths are caused by infections, including pneumonia and diarrhoea, which could be prevented by vaccination. Enhanced deployment of recently developed pneumococcal conjugate and rotavirus vaccines should, therefore, result in a further decline in childhood mortality. Development of vaccines against more complex infections, such as malaria, tuberculosis and HIV, has been challenging and achievements so far have been modest. Final success against these infections may require combination vaccinations, each component stimulating a different arm of the immune system. In the longer term, vaccines are likely to be used to prevent or modulate the course of some non-infectious diseases. Progress has already been made with therapeutic cancer vaccines and future potential targets include addiction, diabetes, hypertension and Alzheimer''s disease.     

Childhood Behavioral Problems Predict Young Adults' BMI and Obesity: Evidence From a Birth Cohort Stud

We examined whether behavioral problems in childhood and adolescence are associated with young adults' BMI and obesity, and tested whether childhood behavioral problems have a greater impact on young adults' obesity than adolescent behavioral problems. The data were from the Mater‐University of Queensland Study of Pregnancy (MUSP) and Its Outcomes, a population‐based birth cohort study commenced in Brisbane, Australia, in 1981. A subsample of 2,278 children for whom we had prospective information on their behavioral problems at ages 5 and 14 and measured BMI, and its categories (normal, overweight, and obese) at age 21 was chosen. Young adults who experienced behavioral problems at ages 5 or 14 had a greater average BMI and were more likely to be obese compared to young adults without behavioral problems at both ages. The childhood onset group was at greater risk of becoming obese by age 21 compared to the adolescent onset group (P = 0.04). These associations remained consistent after adjusting for a variety of potential covariates including maternal characteristics (i.e., demographics and life style), child dietary patterns, family meals, television (TV) watching, and participation in sports and exercise at 14 years. Childhood as well as persistent behavioral problems during childhood and adolescence predicts young adults' BMI and obesity. Although further studies are needed to confirm this association, there is a need for close monitoring of children presenting with behavioral problems.     

A Novel Technic for High School Magazines

Inspired by ideas that Levi-Strauss (1955) presented in Tristes Tropiques, the author discusses music and early childhood in Brazil from educational, historical, anthropological, and sociological perspectives. She begins with a short history of early childhood education in Brazil, including changes in educational policies, teacher preparation, and recent statistics. Next, a brief history of music teaching and learning is followed by a discussion of Brazilian children's current musical practices. In this section, the author discusses teacher training, curriculum, religion, wealth, local culture, and the role of the media. The author concludes with suggestions for the development of both early childhood music education policy and practice in Brazil.     

Hereditary pancreatitis: report of an affected Canadian kindred and review of the disease.

Hereditary pancreatitis is an autosomal dominant disease with no other known cause. It usually begins in childhood and is characterized by recurrent attacks of abdominal pain of variable intensity and duration, followed by symptom-free periods. The diagnosis is usually made in early adult life, when pancreatic insufficiency and calcifications appear. Complications are less frequent than in nonhereditary chronic pancreatitis. There are also differences between the two forms of chronic pancreatitis in sex incidence, etiologic factors and life expectancy. In a Canadian kindred three generations are affected with hereditary pancreatitis; there are four definite and four suspected cases. More than 40 affected kindreds, including 195 proven cases and 190 suspected cases, have now been reported in the literature. Thus, hereditary pancreatitis should be considered in the differential diagnosis of chronic relapsing pancreatitis of unknown cause as well as recurrent abdominal pain in childhood.     

稳定期慢性阻塞性肺疾病患者家庭运动训练现状调查及其影响因素的Logistic回归分析

目的:探讨稳定期慢性阻塞性肺疾病(COPD)患者家庭运动训练现状调查及其影响因素的Logistic回归分析。方法:收集2018年1月～2019年12月期间于本院进行治疗的稳定期COPD患者172例为调查研究对象,根据患者家庭运动训练进行情况将患者分为非运动训练组和运动训练组,对两组患者的一般资料、临床资料等进行统计对比,并采用单因素和多因素Logistic回归分析对影响稳定期COPD患者家庭运动训练进行情况的因素进行分析探讨。结果:在随访调查中,有59例患者进行了家庭运动训练,运动康复训练的普及率为34.30%。经单因素分析,两组患者在性别、年龄、体质指数(BMI)、居住地、婚姻状况、基础疾病、职业对比无显著性差异(P0.05),而两组患者在住院次数、病程、文化程度、照顾情况、肺康复指导对比有显著性差异(P0.05)。经多因素Logistic回归分析显示,COPD患者的住院次数、病程、文化程度、照顾情况、肺康复指导是影响患者进行家庭运动训练的主要因素(P0.05)。结论:我院稳定期COPD患者随访期间家庭运动训练的普及率普遍偏低,且运动方式、强度、频率、规律性等均欠佳。运动训练现状受到患者的住院次数、病程长短、文化程度高低、有无照顾者、有无肺康复指导等因素影响。     

Rural-urban differences in maternal responses to childhood fever in South East Nigeria

Background

Childhood fevers due to malaria remain a major cause of morbidity and mortality among under-five children in Nigeria. The degree of vulnerability perceived by mothers will affect their perception of the severity and threat of their child''s fever and the patterns of health care use. This study was undertaken to compare maternal responses to childhood fever in urban and rural areas of Enugu, south east Nigeria.

Methodology/Principal Findings

Data was collected with pre-tested interviewer-administered questionnaires from 276 and 124 urban and rural households respectively. In each household, only one woman aged 15–49 years who had lived in each of the urban and rural communities for at least one year and had at least one child less than 5 years old was interviewed. Malaria was mentioned as the commonest cause of childhood fevers. Rural mothers were more likely to recognize danger signs and symptoms than urban mothers. Rural mothers use more of informal than formal health services, and there is more home management of the fever with urban than rural mothers. Chloroquine, ACT, SP and Paracetamol are the main drugs given at home for childhood fevers, but the rural mothers were more likely to use leftover drugs from previous treatment to treat the fevers than urban mothers. The urban respondents were also more likely to use a preventive measure. Urban mothers sought actions faster than rural mothers and the total cost of treatment was also higher in urban areas.

Conclusions/Significance

Both urban and rural mothers are aware that malaria is the major cause of childhood fevers. Although rural mothers recognize childhood fever and danger signs better than urban mothers, the urban mothers'' responses to fever seem to be better than that for rural mothers. These responses and differences may be important for geographical targeting by policy makers for malaria interventions.     

Age at onset versus family history and clinical outcomes in 1,665 international bipolar-I disorder patients

Early onset in bipolar disorder (BPD) has been associated with greater familial risk and unfavorable clinical outcomes. We pooled data from seven international centers to analyze the relationships of family history and symptomatic as well as functional measures of adult morbidity to onset age, or onset in childhood (age <12), adolescence (12-18), or adulthood (19-55 years). In 1,665 adult, DSM-IV BPD-I patients, onset was 5% in childhood, 28% in adolescence, and 53% at peak ages 15-25. Adolescent and adult onset did not differ by symptomatic morbidity (episodes/year, percentage of months ill, co-morbidity, hospitalization, suicide attempts) or family history. Indications of favorable adult functional outcomes (employment, living independently, marriage and children, and a composite measure including education) ranked, by onset: adult > adolescent > child. Onset in childhood versus adolescence had more episodes/year and more psychiatric co-morbidity. Family history was most prevalent with childhood onset, similar over onset ages 12-40 years, and fell sharply thereafter. Multivariate modeling sustained the impression that family history and poor functional, but not symptomatic, outcomes were associated with younger, especially childhood onset. Early onset was more related to poor functional outcomes than greater symptomatic morbidity, with least favorable outcomes and greater family history with childhood onset.     

Wilson's disease: a common liver disorder?

In two sibships 7 of 24 siblings were homozygous for Wilson''s disease. In family A, the largest kindred of this recessively inherited disease thus far reported, the proband presented with chronic active hepatitis, one sibling died of cirrhosis, a second had clinical evidence of chronic liver disease and two others had biochemical and histologic changes in liver biopsy specimens. In family B the proband had cirrhosis and portal hypertension and one sibling had biochemical and histologic evidence of liver disease. All six living patients had low serum concentrations of ceruloplasmin and copper and a high 24-hour urinary excretion of copper, which was greatly increased by administration of D-penicillamine. None showed neurologic abnormalities and only one had Kayser-Fleischer rings (detectable only by slit-lamp examination). Each patient had an erythrocyte sedimentation rate (ESR) of 8 mm/h or less. After 3 and 2 years, respectively, of D-penicillamine therapy the conditions of the two probands had improved. Liver function became normal in three siblings, and no abnormalities developed in the remaining one. Thus, since Wilson''s disease may present with chronic active hepatitis or cirrhosis with a normal ESR and without ocular or neurologic signs, it may be a more common cause of liver disease in young people than has been appreciated.     

草原生态补助奖励政策牧民满意度及影响因素研究——基于甘肃青藏高原区与西部荒漠区的实证

草原生态补偿是我国建设生态文明的必由之路之一。牧民对草原生态补助奖励政策的满意度,一定程度上反映了补奖政策实施的绩效。甘肃省针对不同牧区特点,实施了差别化的补奖标准。选取甘肃青藏高原区和西部荒漠区两个不同草原牧区类型351户牧民为调研对象,结合入户深度访谈和调查问卷,构建满意度评价指标体系,测定了不同牧区牧民对草原生态补奖政策的满意度,借助Logistic模型分析不同牧区牧民政策满意度与影响因素。结果表明:(1)牧民对草原生态补奖政策总体满意度偏低,满意度综合指数为67.24%。(2)牧民生计多样化对政策评价具有重要影响。西部荒漠区牧民收入来源较青藏高原区多样化,畜牧业所占比重低于青藏高原区,政策实施对牧民收入影响较小,青藏高原区牧民满意度为62.43%,低于西部荒漠区的72.35%。(3)牧民对政策作用的认知、家庭规模以及对政策的了解,是影响两个区域牧民对政策满意度的共同因素,但两个区域呈现出较大差异性。因此,在后续政策实施中,实施"精准补偿"措施,多样化补偿标准和补偿方式,加强政策宣传和引导,示范引领安置牧民,延长畜牧业产业链,实现畜牧产业"三产"融合,逐步实现草原生态保护、牧业转型发展和牧民增收三方共赢。     

神经母细胞瘤免疫治疗研究进展

神经母细胞瘤是最常见的儿童肿瘤疾病之一,对患者及其家属来了巨大的精神痛苦和经济损失,传统治疗方法对该疾病的预前及术后都效果极差,对该肿瘤的治疗一直是医学界研究的热点和难点。免疫治疗近年来在肿瘤疾病治疗中体现出来的有效、安全、毒副作用小等特点越来越引起临床科学界的重视,科学家对通过免疫疗法治愈神经母细胞瘤的研究进展迅速,并已经取得部分有效的治疗性研究结果,值得进行综述,但目前尚未有对该方面研究的综述出现。本文就近年来神经母细胞瘤治疗研究的热点——免疫逃逸的机理研究、神经母细胞瘤疫苗、免疫类药物开发等进行综述。可以预见,未来神经母细胞瘤免疫治疗的前景广阔,也将有越来越多的针对神经母细胞瘤的商品化免疫性药物、肿瘤疫苗出现,这些药物将对该疾病的治疗具有显著的疗效。