Vision-related quality of life, photoaversion, and optical rehabilitation in achromatopsia.

SIGNIFICANCE: We report on photoaversion and patient-reported quality of life in Danish patients with achromatopsia and evaluate the best optical rehabilitation. Our results contribute to the evaluation of outcome measures in therapy trials and aid in providing the best optical rehabilitation for patients with this and clinically similar conditions. PURPOSE: This study aimed to investigate the vision-related quality of life, the impact of photoaversion on daily living, and the best optical rehabilitation in a cohort of achromatopsia patients, including testing the hypothesis that red light-attenuating filters are generally preferred. METHODS: Patients with genetically verified achromatopsia were recruited. Investigations included the 25-item Visual Function Questionnaire and supplementary questions regarding photoaversion and visual aids. Patients were evaluated by a low vision optometrist and given the choice between different light-attenuating filters. First, two specially designed red and gray filters both transmitting 6% light, and then a pre-defined broader selection of filters. Best-corrected visual acuity and contrast sensitivity were measured without filters and with the two trial filters. RESULTS: Twenty-seven patients participated. Median 25-item Visual Function Questionnaire composite score was 73, with the lowest median score in the subscale near vision (58) and the highest in ocular pain (100). The majority of patients (88%) reported that light caused them discomfort, and 92% used aid(s) to reduce light. Ninety-six percent (26 of 27) preferred the gray filter to the red indoors; 74% (20 of 27) preferred the gray filter. Contrast sensitivity was significantly better with the gray filter compared with no filter (p=0.003) and the red filter (p=0.002). CONCLUSIONS: Our cohort has a relatively high vision-related quality of life compared with other inherited retinal diseases, but photoaversion has a large impact on visual function. Despite what could be expected from a theoretical point of view, red filters are not generally preferred.

Phenotypic characteristics of Danish patients with achromatopsia.

PURPOSE: To describe the phenotype of Danish patients with genetically verified achromatopsia (ACHM) with special focus on signs of progression on structural or functional parameters, and possible genotype-phenotype correlations. METHODS: Forty-eight patients were identified, with disease-causing variants in five different genes: CNGA3, CNGB3, GNAT2, PDE6C and PDE6H. Longitudinal evaluation was possible for 11 patients and 27 patients participated in a renewed in-depth phenotyping consisting of visual acuity assessment, optical coherence tomography (OCT), fundus autofluorescence, colour vision evaluation, contrast sensitivity, mesopic microperimetry and full-field electroretinography. Foveal morphology was evaluated based on OCT images for all 48 patients using a grading system based on the integrity of the hyperreflective photoreceptor band, the inner segment ellipsoid zone (ISe). Signs of progression were evaluated based on longitudinal data and correlation with age. RESULTS: We found a statistically significant positive correlation between OCT grade and age (Spearman ρ = 0.62, p < 0.0001) and we observed changes in the foveal morphology in 2 of 11 patients with ≥5 years of follow-up. We did not find any convincing correlation between age and functional parameters (visual acuity, retinal sensitivity and contrast sensitivity) nor did we find correlation between structural and functional parameters, or any clear genotype-phenotype correlation. CONCLUSIONS: Some patients with ACHM demonstrate signs of progressive foveal changes in OCT characteristics with increasing age. This is relevant in terms of possible new treatments. However, functional characteristics, such as visual acuity, remained stable despite changing foveal structure. Thus, seen from a patient perspective, ACHM can still be considered a non-progressive condition.

The Danish respiratory society guideline for long-term high flow nasal cannula treatment, with or without supplementary oxygen.

Introduction: Long-term High Flow Nasal Cannula (LT-HFNC), defined as High Flow Nasal Cannula treatment provided to patients with chronic pulmonary conditions during stable phases, has emerged as a home treatment in different categories of patients with chronic lung diseases in recent years. Methods: This paper summarizes the physiological effects of LT-HFNC and evaluates the clinical knowledge to date about treatment in patients with chronic obstructive lung disease, interstitial lung disease and bronchiectasis. The guideline is translated and summarized in this paper and presented unabridged as an appendix to the paper. Results: The paper describes the working process behind the Danish Respiratory Society's National guideline for treatment of stable disease, which has been written to support clinicians in both evidence-based decision making and practical issues concerning the treatment.

[Treatment limitations or discontinuation in patients with benign pulmonary diseases].

The decision to limit or discontinue treatment is a difficult issue, which all physicians will face. Timely communication with information on treatment possibilities and limitations, respectful listening to patients' and informal caregivers' wishes and early palliation is recommended in a stable phase. In some situations, it is better to stop life-prolonging treatment and optimise quality of life in patients with benign pulmonary diseases. Decision on treatment limitations or discontinuation is best taken at a conference and should be based on the patient's wishes, the disease stage and progression and potential reversible components.

Danish respiratory society position paper: palliative care in patients with chronic progressive non-malignant lung diseases.

Background:  Chronic non-malignant lung diseases such as chronic obstructive pulmonary disease (COPD) and interstitial lung diseases (ILD) result in reduced quality of life (QoL), a high symptom burden and reduced survival. Patients with chronic non-malignant lung disease often have limited access to palliative care. The symptom burden and the QoL of these patients resembles patients with cancer and the general palliative approach is similar. However, the disease trajectory is often slow and unpredictable, and the palliative effort must be built on accessibility, continuity and professional competences. The Danish Health Authority as well as the WHO recommends that there is access to palliative care for all patients with life-threatening diseases regardless of diagnosis. In 2011, the Danish Health Authority requested that the national medical societies would to formulate guidelines for palliation. Methods: In 2015, a group of members of the Danish Respiratory Society (DRS) was appointed for this purpose. It was composed of experienced ILD and COPD researchers as well as clinicians from different parts of Denmark. A literature review was made, a draft was prepared, and all recommendations were agreed upon unanimously. Results: The Danish version of the position paper was finally submitted for review and accepted by all members of DRS. Conclusion: In this position paper we provide recommendations on the terminology of chronic and terminal lung failure, rehabilitation and palliative care, advanced care planning, informal caregivers and bereavement, symptom management, the imminently dying patient, and organization of palliative care for patients with chronic non-malignant lung diseases.

[Opioids can modulate the immune system]. / Opioider påvirker immunsystemet.

Opioids can modulate and suppress the immune system through central mediated mechanisms. Morphine increases replication and spread of HIV-1. Evidence suggests that morphine can also enhance growth and spread of some cancer diagnoses like breast-, prostate- and non-small cell lung cancer. The mechanisms behind the effects of morphine are mainly mediated by inhibiting apoptosis of cancer cells and by stimulation of angiogenesis. Some other opioid agonists seem to be depleted from these effects. Prospective studies are needed to clarify the immunosuppressive effects of opioids in cancer pain management.

First Case of Liver Abscess in Scandinavia Due to the International Hypervirulent Klebsiella Pneumoniae Clone ST23.

This is the first case report from Scandinavia of a pyogenic liver abscess caused by a Klebsiella pneumoniae isolate belonging to the international hyper virulent clone ST23. The patient, an 85-year old Caucasian, had no history of foreign travel or any classical predisposing factors for infection. The isolate was hypermucoviscous of capsular serotype K1 and carried the virulence factors aerobactin, allS, kfu and rmpA.