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Secondary prophylaxis therapy: what are the benefits, limitations and unknowns?

Prophylaxis is widely recommended for the management of severe haemophilia A. Adoption of this approach has varied from country to country, and notably in the US prophylaxis is currently administered in fewer than one-half of severe cases. One implication is that a substantial segment of the severe haemophilia. A population will only be able to reap the potential benefits of prophylaxis if administered in the form of secondary prophylaxis. This therapeutic alternative has been less extensively investigated than primary prophylaxis, but nevertheless sufficient evidence has been reported to allow an assessment of the benefits and limitations of secondary prophylaxis. This evidence addresses the use of secondary prophylaxis in three contexts: (i). early secondary prophylaxis; (ii). delayed secondary prophylaxis and (iii). secondary prophylaxis in adults. In patients receiving early secondary prophylaxis studies in Sweden, the Netherlands, the UK and the US have demonstrated a reduction in the frequency of bleeding episodes and a subsequent low incidence of arthropathy. Additional reported benefits consist of reduced emergency room visits and hospitalizations. However, secondary prophylaxis is associated with an increased risk of the eventual development of arthropathy compared with primary prophylaxis. When delayed until school age or adolescence or until the development of frequent bleeding episodes under on-demand treatment, secondary prophylaxis generally appears to be unable to reverse all existing or developing joint damage. Nevertheless, multiple studies have shown that this therapy can retard further joint deterioration, reduce the frequency of haemorrhage, hospitalization and missed school days, improve physical function and capacity for self care, lessen restrictions on activities, reduce pain and enhance quality of life. Secondary prophylaxis in adults has been shown effective in reducing bleeding episodes. Adults under secondary prophylaxis can also experience improvements in joint condition, functional capacity and quality of life and a reduction in pain. Irrespective of age at initiation, long-term secondary prophylaxis appears to reduce the frequency of bleeding episodes even in patients with existing target joints whose bleeding diathesis persists during the early phases of secondary prophylactic therapy. In light of its potential benefits for substantial numbers of patients with severe haemophilia A, secondary prophylaxis should be considered to as a therapeutic option for patients not receiving primary prophylaxis.

Adolescent↗

The fate of early experience following developmental change: longitudinal approaches to individual adaptation in childhood.

2 strategies were used to investigate the continued impact of early experience and adaptation given subsequent experience and/or developmental change in a poverty sample (N = 190). Groups were defined whose adaptation was similar during the preschool years but consistently different earlier; then these 2 groups were compared in elementary school. In addition, a series of regression analyses was performed in which variance accounted for by near-in or contemporary predictors of adaptation in middle childhood was removed before adding earlier adaptation in subsequent steps. Children showing positive adaptation in the infant/toddler period showed greater rebound in the elementary school years, despite poor functioning in the preschool period. Regression analyses revealed some incremental power of early predictors with intermediate predictors removed. The results were interpreted as supporting Bowlby's thesis that adaptation is always a product of both developmental history and current circumstances. While this research cannot resolve such a complicated issue, it does point to the need for complex formulations to guide research on individual development.

Adaptation, Psychological↗

Teacher-mediated intervention after disaster: a controlled three-year follow-up of children's functioning.

BACKGROUND: Child survivors of a catastrophic earthquake in Turkey were evaluated three and a half years after the event, and three years after a sub-group participated in a teacher-mediated intervention developed by the authors. The goal of this follow-up study was to determine the long-term effectiveness of the original intervention. METHODS: Subjects who participated in the intervention were compared with a control group of children similar in terms of demographics, risk and exposure. All children were evaluated in terms of posttraumatic, grief and dissociative symptomatology, as well as adaptive functioning (academic performance, social behavior and general conduct). RESULTS: The severity of post-traumatic, grief and dissociative symptoms of the two groups was comparable. Teachers blind to group assignment rated participating children significantly higher than the control group in terms of adaptive functioning. CONCLUSIONS: Early post-disaster intervention addressing children and their educational milieu provides children with significant symptomatic reduction, allowing the mobilization of adaptive coping, thereby enhancing their overall functioning as observed in school.

Adaptation, Psychological↗

Maturation of cutaneous reflex responses recorded in the lower limb in man.

Cutaneous reflex responses from tibialis anterior muscle following electrical stimulation were recorded for 39 normal children between the ages of one and 16 years. In the first year of life stimulation produces a monophasic excitatory reflex response at short latency; in the second year it produces long- as well as short-latency increases in activity. Maturation of reflex function continues during the school years, with progressive emphasis on long- rather than short-latency components. These results are discussed in relation to mechanisms underlying the acquisition of motor skills during childhood.

Adolescent↗

Quality of life after pediatric intestinal transplantation: the perception of pediatric recipients and their parents.

The objective was to examine the perception of physical and psychosocial functioning of pediatric intestinal transplant recipients who are beyond the perioperative period and compare these with normal and chronically ill children. Child and parent forms of the Child Health Questionnaire were administered to all 29 pediatric intestinal transplant recipients between the ages of 5 and 18 years who had had a small bowel transplantation 1 year previous and had a functional allograft. Comparison was made with published norms and scores for pediatric patients on hemodialysis. Intestinal transplant recipients (on average 5 years after intestinal transplantation and at a mean age 11 years) reported similar scores in all domains compared with normal children. Parents of intestinal transplant recipients noted decreased function in several domains related to their child's general health, physical functioning, and the impact of the illness on parental time, emotions and family activities. Intestinal transplant recipients beyond the perioperative period perceive their physical and psychosocial functioning as similar to normal school children. Parental proxy assessments differ from the recipients, with the parent's perception of decreased general health and physical functioning for intestinal transplant recipients compared with norms.

Adolescent↗

Oral complaints and utilization of dental services in relation to general health factors in a 88-year-old Swedish population.

The aim of this study was to describe dental state, oral health problems, self-assessed ability to perform oral hygiene and utilization of oral health services in a sample of 88-year-old people (n = 374) and to relate these variables to functional ability, subjective health and drug consumption. The intention was also to identify barriers to utilization of dental care in non-institutionalized elderly people. The results of interviews showed that 46% of the subjects were dentate. Oral complaints were reported by 24% of the home living individuals. Poor health, medication and feelings of loneliness were factors associated with oral discomfort. Difficulties in performing oral hygiene activities were reported by 10% of the subjects and were correlated with functional impairments and feelings of loneliness. Utilization of dental services was correlated with dental state; 72% of the dentate and 8% of the edentulous subjects made regular dental visits. The main reason for not visiting a dentist was "no perceived need". Independent of dental state, some factors were significantly correlated to low demand for dental services; these included feelings of loneliness, functional impairments and low school education.

Activities of Daily Living↗

A review of mood disorders among juvenile offenders.

OBJECTIVES: This article provides an overview of what is known about the prevalence, diagnosis, and effective treatment of mood disorders among youths, particularly among juvenile offenders, and discusses the unique problems that arise for the delivery of treatment services. The relationship between mood disorders and disruptive or delinquent behaviors as well as the particular importance of proper diagnosis and treatment of mood disorders in this population are discussed. METHODS: A search was conducted of the MEDLINE and PsycINFO databases for articles that had been published since 1980 on mood disorders in the juvenile offender population as well as articles on adolescent mood disorders. RESULTS: The studies on the prevalence of mood disorders among juvenile offenders varied significantly in the methodology used and in the rates of prevalence found, although all studies showed that this population had high rates of mood disorders. The identification and effective treatment of mood disorders is critical because these disorders are a leading cause of suicide among adolescents and because mood disorders may contribute to or exacerbate delinquent and disruptive behaviors. CONCLUSIONS: Juvenile detainees have a constitutional right to needed mental health treatment. More comprehensive mental health services are required to ensure that juvenile offenders with mental illness are identified and cared for appropriately. Doing so not only will alleviate painful symptoms but may also contribute significantly to improvements in psychosocial functioning, interpersonal relations, and school performance and to decreases in delinquent, disruptive, and suicidal behaviors.

Adolescent↗

Evaluation and management of psychosomatic symptoms in adolescence.

Adolescent patients commonly experience symptoms such as headache, chest pain, abdominal pain, or dizziness that are psychophysiologic responses to stress, anxiety, and depression. Because most symptomatic adolescents initially visit medical providers, and not mental health professionals, the clinician is faced with the challenge of providing a comprehensive evaluation that is not merely focused on the symptom. In addition to a careful medical assessment, this evaluation must include a review of psychosocial functioning in the family, school, peer group, and community. Appropriate management may include supportive counseling, instruction in relaxation techniques, anti-depressant medication, and referral for psychotherapy.

Adolescent↗

Slow progression of juvenile metachromatic leukodystrophy 6 years after bone marrow transplantation.

Metachromatic leukodystrophy refers to a group of genetic neurologic diseases caused by deficiencies of the enzyme arylsulfatase A and the resulting accumulation of sulfatides in white matter. Bone marrow transplantation has been advocated as a treatment in an attempt to correct the enzyme deficiency. Such a transplant was performed in 1991 in a 16-year-old girl with a form of late juvenile metachromatic leukodystrophy caused by a homozygous P426L mutation in the arylsulfatase A gene. Engraftment was prompt and resulted in constant enzymatic normalization of circulating lymphocytes. The elevated urinary excretion of sulfatides remained unaffected. Clinical findings up until transplantation consisted of gait disturbances, impairment of cognitive functioning, and deterioration in school performance over several years. During a 6-year follow-up period, the patient's condition was subject to major fluctuations but, on the whole, findings showed slow neurologic and neurophysiologic deterioration. The clinical course observed after bone marrow transplantation probably more or less reflects the natural course expected in this form of late-onset metachromatic leukodystrophy.

Adolescent↗

Insulin pumps: what every school nurse needs to know.

The results of the Diabetes Control and Complications Trial revolutionized the care of people with Type 1 diabetes mellitus (DM). The era of "tight control" of blood sugars to decrease microvascular complications dawned. The subsequent technological development of insulin pumps has made it possible for individuals with Type 1 DM, as well as those with Type 2 who are insulin dependent, to keep their blood sugars in a more normal range. Children of all ages with Type 1 DM have been switching from multiple daily injections of insulin to insulin pumps. School nurses who have not had a child with a pump certainly will in the near future. It is important for school nurses to understand the function and possible complications of using an insulin pump to assist and support children in their transition to pump therapy. School nurses need to be aware of available technical support resources for insulin pumps should problems arise at school with pump management.

Adolescent↗

Quality of life of adolescent males with attention-deficit hyperactivity disorder.

Most psychosocial research on attention-deficit/hyperactivity disorder (ADHD) has focused on deficits in school, family, or behavioral functioning without incorporating perceived quality of life (QoL) or the adolescents' perspective. The Youth Quality of Life Instrument--Research Version (YQOL-R), was used to assess self-perceived QoL in a community sample of adolescents aged 11-18 years. Fifty-five adolescent males with a clinical diagnosis of ADHD were compared to a group of 107 adolescents with no chronic conditions (NCC) and a group of 52 adolescents with mobility impairments (MI). The adolescents with ADHD reported significantly lower perceived QoL scores, particularly in the Self and Relationship domains, than the NCC group. Their scores were similar to those from the group with MI, a group previously shown to have a substandard QoL. Interventions to improve self-esteem and social interactions might use QoL outcomes in evaluating effectiveness.

Adolescent↗

A cross-sectional analysis of video games and attention deficit hyperactivity disorder symptoms in adolescents.

BACKGROUND: Excessive use of the Internet has been associated with attention deficit hyperactivity disorder (ADHD), but the relationship between video games and ADHD symptoms in adolescents is unknown. METHOD: A survey of adolescents and parents (n = 72 adolescents, 72 parents) was performed assessing daily time spent on the Internet, television, console video games, and Internet video games, and their association with academic and social functioning. Subjects were high school students in the ninth and tenth grade. Students were administered a modified Young's Internet Addiction Scale (YIAS) and asked questions about exercise, grades, work, and school detentions. Parents were asked to complete the Conners' Parent Rating Scale (CPRS) and answer questions regarding medical/psychiatric conditions in their child. RESULTS: There was a significant association between time spent playing games for more than one hour a day and YIAS (p < 0.001), overall grade point average (p < or = 0.019), and the "Inattention" and "ADHD" components of the CPRS (p < or = 0.001 and p < or = 0.020, respectively). No significant association was found between body mass index (BMI), exercise, number of detentions, or the "Oppositional" and "Hyperactivity" components of CPRS and video game use. CONCLUSION: Adolescents who play more than one hour of console or Internet video games may have more or more intense symptoms of ADHD or inattention than those who do not. Given the possible negative effects these conditions may have on scholastic performance, the added consequences of more time spent on video games may also place these individuals at increased risk for problems in school.

Journal Article↗

Behavioral assessment of lead intoxication in children.

Current questions about lead exposure focus on the consequences of levels too low to have erupted into blatantly discernible defects. The present paper addresses two sets of interrelated problems derived from this issue. One is how to define the behavioral consequences of asymptomatic lead absorption, and the second focuses on behavioral assessment procedures. Current primary prevention programs emphasize environmental monitoring, and early detection programs emphasize lead body burden measurements. The evaluation of behavioral problems in school children as a function of body burden is rarely performed. Epidemiologic data indicate sufficient natural variability to determine the degree of association between indices of total body burden and behavior. Assessment procedures are described and research suggestions offered that sample concretely defined target behaviors in social environments.

Body Burden↗

Relationship of adherence to pediatric asthma morbidity among inner-city children.

OBJECTIVES: Morbidity from asthma among children is one of the most important US health concerns. This study examines the relationship of baseline nonadherence to subsequent asthma morbidity among inner-city children. METHODS: A multisite, prospective, longitudinal panel study was conducted of 1199 children who were aged 4 to 9 years and had asthma and their caregivers, most of whom were parents, in emergency departments and clinics at 8 research centers in 7 US metropolitan inner-city areas. Nine morbidity indicators were collected at 3, 6, and 9 months after baseline, including hospitalizations, unscheduled visits, days of wheeze/cough, and days of reduced activities. RESULTS: Children whose caregivers scored high on a new measure, Admitted Nonadherence, experienced significantly worse morbidity on 8 of the 9 measures. Children who scored high on a new Risk for Nonadherence measure experienced significantly worse morbidity on all 9 morbidity measures. Multiple and logistic regressions found that the adherence measures had independent significant effects on morbidity. Combining the measures improved estimates of morbidity: children whose caregivers were poor on either adherence measure had worse morbidity than those with good adherence on both, eg, rate of hospitalization was twice as high, they missed more than twice as much school, had poorer overall functioning, and experienced more days of wheezing and more restricted days of activity. CONCLUSIONS: Risk for Nonadherence and Admitted Nonadherence independently and jointly predicted subsequent asthma morbidity. Targeting risks for nonadherence may be an effective intervention strategy. Most risks can be controlled by physicians through reducing the complexity of asthma regimens, communicating effectively with caregivers about medication use, and correcting family misconceptions about asthma medication side effects.

Adult↗

Adverse reactions in children. Special considerations in prevention and management.

Adverse reactions occur in only 2 to 5% of children for whom drugs are prescribed. Most of those which do occur are mild and transient in nature. Practitioners who treat children should be familiar with the adverse effects of every drug they use and be certain that every drug prescribed is strictly necessary. Antimicrobials and antipyretic/analgesics are the drugs most commonly prescribed for children. Serious adverse reactions such as aplastic anaemia (with chloramphenicol) are fortunately rare. Erythromycin prescribed for intercurrent infection may precipitate signs of toxicity in children receiving carbamazepine or theophylline. Anticonvulsants impair cognitive function and thereby affect school performance. Phenothiazines and metoclopramide prescribed as antiemetics may cause spectacular dystonic posturings, although this adverse reaction is rapidly reversed with intravenous biperiden. The duration of drug therapy in paediatric practice should be limited to the minimum time compatible with full recovery. The management of adverse reactions in childhood hinges on early recognition and prompt withholding of the offending drug. Urgent resuscitation is essential when anaphylactic collapse occurs, but in most instances of adverse reaction symptomatic treatment of discomfort is all that is necessary. It is important that parents of a child who has suffered an adverse reaction be aware of this and know the name of the drug responsible. Practitioners who encounter an unreported adverse reaction have an obligation to notify the appropriate drug monitoring authority.

Analgesics↗

Use of intranasal cromolyn sodium for allergic rhinitis.

Allergic rhinitis affects 10% to 20% of Americans. It frequently coexists with other conditions, such as allergic conjunctivitis, sinusitis, and asthma, and is associated with impaired occupational function and performance in school, decreased quality of life, and increased health care costs. An efficacious agent with minimal adverse effects and a lack of drug interactions is needed to help simplify treatment of allergic rhinitis, especially in patients with comorbidities. Controlled studies of intranasal cromolyn sodium therapy for patients with seasonal and perennial allergic rhinitis are reviewed, and appropriate candidates for treatment with this agent are discussed. Cromolyn inhibits the degranulation of sensitized mast cells, thereby blocking the release of inflammatory and allergic mediators. It reduces symptoms of allergic rhinitis, and, when used prophylactically, cromolyn can prevent symptoms from occurring. Controlled studies comparing cromolyn with placebo, intranasal corticosteroids, and antihistamines have shown the efficacy of cromolyn in relieving rhinitis symptoms. In addition, because cromolyn is poorly absorbed systemically, it is well tolerated and not associated with drug interactions. Intranasal cromolyn has an excellent safety record, is available as an over-the-counter medication, and has been proved to be efficacious in patients with allergic rhinitis.

Administration, Intranasal↗