Characteristics of autosomal dominant WFS1-associated optic neuropathy and its comparability to OPA1-associated autosomal dominant optic atrophy.

This study aims to describe the ophthalmic characteristics of autosomal dominant (AD) WFS1-associated optic atrophy (AD WFS1-OA), and to explore phenotypic differences with dominant optic atrophy (DOA) caused by mutations in the OPA1-gene. WFS1-associated diseases, or 'wolframinopathies', exhibit a spectrum of ocular and systemic phenotypes, of which the autosomal recessive Wolfram syndrome has been the most extensively studied. AD mutations in WFS1 also cause various phenotypical changes including OA.

[Guidelines in historical perspective].

The role of clinical practice guidelines has changed dramatically the past 50 years. Both beneficial, as also having some disadvantageous effects. Considering the international developments, such as a growing body of evidence and evolving methodologies, some limits might be in reach or already been reached.

Clinical outcomes of treatment with idebenone in Leber's hereditary optic neuropathy in the Netherlands: A national cohort study.

Purpose: The purpose of the study was to present results from a national Dutch cohort of patients with Leber's Hereditary Optic Neuropathy (LHON) treated with idebenone.

Methods: The multicentre, open-label, retrospective evaluation of the long-term outcome of idebenone treatment of Dutch LHON patients on visual function and on thickness of the retinal ganglion cell layer. Patients included in the analysis had a confirmed mutation in their mitochondrial DNA encoding either of the seven subunits of complex I, had a reported loss of vision in at least one eye and had a follow-up of more than 6 months after their treatment was started.

[New treatment option for Leber hereditary optic neuropathy: early diagnosis is required].

Background: Leber hereditary optic neuropathy (LHON) is an orphan disease which leads to painless subacute loss of central vision in both eyes. It develops mainly in young adults and is more common in males. It most often leads to lifelong blindness.

International Consensus Statement on the Clinical and Therapeutic Management of Leber Hereditary Optic Neuropathy.

Leber hereditary optic neuropathy (LHON) is currently estimated as the most frequent mitochondrial disease (1 in 27,000-45,000). Its molecular pathogenesis and natural history is now fairly well understood. LHON also is the first mitochondrial disease for which a treatment has been approved (idebenone-Raxone, Santhera Pharmaceuticals) by the European Medicine Agency, under exceptional circumstances because of the rarity and severity of the disease.

[The future of guidelines: an exploration based on the four perspectives of the PHSF-2014].

In the Dutch Public Health Status and Foresight report (PHSF), the Dutch National Institute for Public Health and the Environment explores the future of public health and health care in the Netherlands up to 2040. In the first article of a short series on the PHSF 2014, we briefly explained the study and four normative perspectives on public health. In the second article, we described the most important future trends up to 2030.

Prioritizing dermatoses: rationally selecting guideline topics.

Background: Clinical practice guideline (CPG) development starts with selecting appropriate topics, as resources to develop a guideline are limited. However, a standardized method for topic selection is commonly missing and the way different criteria are used to prioritize is not clear.

Objectives: To select and prioritize dermatological topics for CPG development and elucidate criteria dermatologists find important in selecting guideline topics.

A national study on adherence to a basal cell carcinoma guideline; development of a tool to assess guideline adherence.

Background: Clinical practice guideline implementation may be at variance with actual daily practice, as guideline adherence is a complex process depending on many actors and factors. Feedback regarding adherence is essential to monitor the effect that a guideline has in clinical practice and whether or not the quality of care is raised by implementation.

Objectives: Developing a tool for obtaining and giving nationwide feedback regarding adherence.

Summary of the Dutch S3-guidelines on the treatment of psoriasis 2011. Dutch Society of Dermatology and Venereology.

This document provides a summary of the Dutch S3-guidelines on the treatment of psoriasis. These guidelines were finalized in December 2011 and contain unique chapters on the treatment of psoriasis of the face and flexures, childhood psoriasis as well as the patient's perspective on treatment. They also cover the topical treatment of psoriasis, photo(chemo)therapy, conventional systemic therapy and biological therapy.

Prospective comparison of three guideline development methods for treatment of actinic keratosis.

Objective: To compare three methods of guideline development, to see whether using alternative evidence-based methods resulted in variation of recommendations for treating actinic keratosis.

Methods: Method 1 followed a standard multiple session evidence-based approach with a working group. In method 2 recommendations were formulated by a working group during a 2-day conference.

Off-label use of azathioprine in dermatology: a systematic review.

Objective: To summarize evidence regarding the effectiveness, efficacy, and safety of off-label azathioprine use in dermatology.

Data Sources: We searched the MEDLINE (1950-2009), EMBASE (1980-2009), and CENTRAL (1996-2009) databases on October 9, 2009. The main search terms were azathioprine and its synonyms.

Short- and long-term effects of a quality improvement collaborative on diabetes management.

Introduction: This study examined the short- and long-term effects of a quality improvement collaborative on patient outcomes, professional performance, and structural aspects of chronic care management of type 2 diabetes in an integrated care setting.

Methods: Controlled pre- and post-intervention study assessing patient outcomes (hemoglobin A1c, cholesterol, blood pressure, weight, blood lipid levels, and smoking status), professional performance (guideline adherence), and structural aspects of chronic care management from baseline up to 24 months. Analyses were based on 1,861 patients with diabetes in six intervention and nine control regions representing 37 general practices and 13 outpatient clinics.

[Guideline 'Diagnosis and treatment of inflammatory bowel disease in adults'. I. Diagnosis and treatment].

The Dutch national practice guideline 'Diagnosis and treatment of inflammatory bowel diseases (IBD) in adults' describes the multidisciplinary approach for adult patients with (suspected) IBD, recommended following analysis of the literature according to the principles of evidence based guideline development. The symptoms on first presentation of a patient with IBD are mainly connected with the localisation and severity of the disease and less with the resulting diagnosis 'Crohn's disease' or 'ulcerative colitis'. There is no test by which the diseases can be distinguished with certainty.

Intravenous immunoglobulin in recurrent-relapsing inflammatory optic neuropathy.

Objective: Recurrent-relapsing inflammatory optic neuropathy, including chronic relapsing inflammatory and autoimmune optic neuropathies, is rare, but can cause severe visual loss. Long-term steroids may preserve vision, yet side effects are frequent. We describe our experience with intravenous immunoglobulins (IVIg).

[Guideline 'Medicinal care for drug addicts in penal institutions'].

In the Netherlands, the policy on care for prisoners who are addicted to opiates is still heterogeneous. The recent guidelines entitled 'Medicinal care for drug addicts in penal institutions' should contribute towards unambiguous and more evidence-based treatment for this group. In addition, it should improve and bring the care pathways within judicial institutions and mainstream healthcare more into line with one another.

The guideline "consultation psychiatry" of the Netherlands Psychiatric Association.

Background: In 2008, the Netherlands Psychiatric Association authorized a guideline "consultation psychiatry."

Aim: To set a standard for psychiatric consultations in nonpsychiatric settings. The main objective of the guideline is to answer three questions: Is psychiatric consultation effective and, if so, which forms are most effective? How should a psychiatric consultations be performed? What increases adherence to recommendations given by the consulting psychiatrist?

Method: Systematic literature review.

[Advice on guidelines: planning for guideline development in the Netherlands].

There is a lack of shared responsibility and national steering in guideline development. The 'Regieraad' (advisory board on guideline development, established by the Dutch Health Secretary) hopes to facilitate the coordination of guideline development. Therefore, the board developed a shortlist of the 100 most important diseases and 25 important care processes, based on several accepted criteria.

[The practice guideline 'Consultation psychiatry' of the Dutch Psychiatric Association for psychiatric consultations in primary care and the hospital].

Psychiatric consultation in primary care as well as in the hospital is both effective and cost-effective if certain procedures are followed. With the professional guideline 'Consultation psychiatry', the Dutch Psychiatric Association aims at setting a standard for psychiatric consultations in non-psychiatric settings. In general practice, the psychiatric consultation is preferably embedded in 'collaborative care', an integrated care model including the general practitioner and a case manager (usually a nurse), with the consultant psychiatrist being regularly available for clearly defined indications.

Factors that influence the stroke care team's effectiveness in reducing the length of hospital stay.

Background And Purpose: The purpose of this study was to explore the effects of a quality improvement program for improving stroke care and the determinants of success at the team and hospital levels. Method- For 16 months, 23 multidisciplinary stroke service teams participated in a quality improvement collaborative designed to set up stroke services and reduce the length of hospital stay (LOHS). We monitored the LOHS and the discharge delay during the project and measured indicators of well organized stroke services at baseline and after the intervention.

Evidence for the impact of quality improvement collaboratives: systematic review.

Objective: To evaluate the effectiveness of quality improvement collaboratives in improving the quality of care.

Data Sources: Relevant studies through Medline, Embase, PsycINFO, CINAHL, and Cochrane databases.

Study Selection: Two reviewers independently extracted data on topics, participants, setting, study design, and outcomes.

[The history of evidence-based psychiatry and evidence-based guideline development].

Evidence-based medicine, evidence-based psychiatry and evidence-based development of clinical guidelines are all flourishing today because of developments that have taken place in both science and society. These concepts will be explained and placed in their context. Medical treatment is based on the one hand on scientific knowledge and experience, on the other hand on compassion and consideration for others.

[Guideline 'Precautionary measures for contrast media containing iodine'].

Annually, 0.5-1 million injections of contrast media containing iodine are administered in the Netherlands. Almost all contrast media nowadays are low-osmolar and nonionic.