Reducing fracture incidence in children with osteogenesis imperfecta: contribution of orthotics to bisphosphonates treatment.

PURPOSE: To determine whether there was a reduction in fracture incidence amongst children with OI who were treated with both bisphosphonates and orthoses. OBJECTIVE: Was there an additional reduction in fracture incidence amongst children with Osteogenesis Imperfecta (OI) treated with both bisphosphonates and Hip-Knee-Ankle-Foot-Orthosis (HKAFO)? MATERIALS AND METHODS: Of the 129 OI patients treated from 1990 to 2017, retrospective data from 48 patients who participated in the bisphosphonates-orthosis regime were analyzed including the incidence of fractures and modalities of fracture treatment. RESULTS: Bisphosphonates usage was more frequent than bracing and there were more positive changes (smaller or equal number of fractures each year) than negative changes (more fractures each year); negative changes were scarce, explained by non-compliance with the use of bracing. Poisson regression models were significant for positive changes, whereas the interaction between them was borderline significant. The main finding is that the association between bisphosphonates usage and the number of positive changes was stronger among the patients who used braces more frequently and weaker among patients who used bracing less frequently. CONCLUSIONS: Bracing of OI patients has an additive effect on bisphosphonate treatment in fracture prevention which should lead to the reconsideration of a hybrid approach to OI management.

Two key goals of treatment of Osteogenesis Imperfecta (OI) patients include decreasing fracture incidence and improving function and independence as supported by a multi-disciplinary approach that combines medical, orthopaedic and rehabilitation treatments.Although the literature provides evidence that bisphosphonates reduce the frequency of fractures, there have not been reports of its effect when used with orthoses.Orthoses for OI patients have an additive effect on bisphosphonate treatment in fracture prevention.These results contribute to making an informed decision regarding this hybrid approach to OI management.

National study of newborn hearing screening: programme sensitivity and characteristics of undetected children.

UNLABELLED: The success of universal newborn hearing screening (UNHS) programmes is usually evaluated by determining the effect of the early detection of hearing loss on developmental outcome. However, in practice, these programmes do not detect all children with permanent childhood hearing impairment. In this study we determine the sensitivity of the current UNHS programme and analyse the characteristics of the children not detected by UNHS. We performed a nationwide, population-based, retrospective follow-up study in The Netherlands. All children born in 2003-05 and screened in a hearing screening programme (well babies and neonatal intensive care (NICU) graduates) were included for study. The main outcome measure was the sensitivity of the UNHS programme (based on the proportion of children known to have a permanent childhood hearing impairment in 2008 who were identified by UNHS). We also evaluated age at diagnosis, severity, and aetiology of hearing impairment in the children not detected by UNHS. We found that the sensitivity of the current UNHS programme was 0.83 (0.79 for well babies and 0.96 for NICU graduates). Permanent childhood hearing impairment was confirmed before 36 months of age in 96% of the study cohort. Of the children unidentified by the UNHS, > 50% had moderate hearing loss. No predominant cause of hearing impairment was found in these children. CONCLUSION: Our current UNHS programme identified the majority of children with a permanent hearing impairment of congenital cause.

[Where does BSE in the Netherlands come from?]. / Waar komt BSE in Nederland vandaan?

It is only in the last 5 years that the Netherlands has been confronted with cases of bovine spongiform encephalopathy (BSE). The cases diagnosed to date have not been clearly linked to imports from the United Kingdom. This article describes the various possible explanations for the Dutch cases. The risk factors involved, have either a connection with imported BSE, local origin of BSE, or both. These factors can also be divided into introductory risk and propagation risk, terms that were also used in an EU risk assessment study. Research at ID-Lelystad since the early 1990s and at IKC-Ede has tried to assess the relative importance of the various risk factors, the results of which are discussed in this paper. The paper does not deal with the specifics of the cases diagnosed to date, because of the absence of an in-depth epidemiological investigation, but provides a general assessment of the risk factors that might have played a role. Important factors have been, in addition to the initial imports of cattle and meat and bone-meal from the UK, the continuing imports from other countries with covert BSE and the cross-contamination within the animal feed production lines. Emphasis is on the period of the early and mid-1990s, the period in which most calves with diagnosed BSE were born.

A tentative exploration of the character of pediatric cochlear implant critique.

Deaf communities have resisted pediatric cochlear implants in many western countries since the introduction of the technique in the late 1970's. In this struggle they have been supported by quite a few scientists from a diverse, albeit social scientific background. The arguments of the latter have been the source of much misapprehension. In this paper we will explore this misapprehension by structurally probing for implicit conceptualisations of benefit and legitimation procedures in the discourse of proponents and opponents alike.

Three-year outcome of disruptive adolescents treated in a day program.

Adolescents (N = 38) who attended a multi modal day program were matched with a control group (N = 35) for age, gender, year of assessment, and on delinquent and aggressive scores. Parent and child were separately interviewed three years after initial assessment. Adolescents who attended the program functioned better overall and were more satisfied with treatment than controls. Both groups showed a poor outcome and a decrease in symptom scores. A diagnosis of conduct disorder, high delinquency scores and high total problem scores predicted a poorer outcome.

A survey of the smoking habits of physicians in Curacao in 1995 - abstract

We sent a self-administered WHO smoking survey to all 245 physicians working on the island of Curacao. A response rate of 61 percent was reached without sending reminder letters. 33 percent were current smokers, smoking was more prevalent in males (68 percent) than females (34 percent), and the mean daily consumption was 11.8 cigarettes. Smoking prevalence and smoking behaviour amongst Curacaoan physicians proved to be highly similar to the data that have been revealed by Dutch studies, even though the smoking prevalence within Curacaoan population is lower than in the Netherlands. These findings may support the hypothesis of professional socialisation in medical school. Curacaoan and Dutch physicians also expressed similar opinions towards smoking. Among doctors the knowledge of smoking and diseases were found to be suboptimal. Curacaoan doctors report they want more information about smoking cessation programmes. (AU).

Juvenile obsessive-compulsive disorder.

OBJECTIVE: To describe the characteristics of a series of children and adolescents with obsessive-compulsive disorder (OCD) and evaluate the outcome of treatment. METHOD: Type of symptoms, severity before and after treatment and factors associated with outcome were examined in a large group (n = 82) of consecutive OCD cases referred for treatment. RESULTS: Most children (95%) had both obsessions and compulsions. Symptoms had been present for 2 years on average. Seventy-one per cent (n = 57) of all eligible patients completed a combined behavioural and pharmacological protocol. Among these, there was a 68% remission rate and a 60% decrease of symptoms at 4 weeks. Comorbidity with oppositional defiant disorder and high aggression scores were associated with poor outcome. CONCLUSIONS: Juvenile OCD can be treated effectively in a standard clinical setting. Treatment programs of the kind described are accepted by young people. It remains to be seen whether in this age group a combined treatment produces better results than medication alone or cognitive-behaviour therapy alone.

Inter-rater reliability of global assessment of functioning in a clinical setting.

Four studies on the inter-rater reliability of a proposed Axis V version for DSM-IV and of the CGAS involving 162 child and adolescent patients and 20 clinicians showed moderate agreement (intraclass correlation: 0.53-0.66). This was comparable to previous versions of Axis V, but lower than that reported for the CGAS. More detailed description of anchor points did not increase reliability nor there were differences in agreement when rating current or previous functioning.