A Reappraisal of the Architectural Legacy of King-Stadholder William III and Queen Mary II: Taste, Passion and Frenzy

This article reappraises the architectural legacy of King William III and Queen Mary II on the basis of an examination of all of the building projects relating to castles and palaces in Great Britain and the Low Countries during their reign. In both countries William and Mary were continuously renovating and adding additions to already existing castles and palaces as well as creating new ones, always simultaneously combining various projects. The authors propose that the extent of William and Mary’s architectural endeavours has so far been underestimated, primarily because these have not been assessed as an ensemble. Similarly, the monarchs’ great interest in the interior of their residences, and especially in their painting collections, has not been sufficiently acknowledged. This article brings together two academic traditions at both sides of the North Sea: on the basis of primary sources such as the diaries of Constantijn Huygens Jr, travel accounts and probate inventories, both the motivation for their frantic building can be discerned, as well as the quality, scope and cultural agency of the architectural and art programmes of William and Mary

Training clinicians in how to use patient-reported outcome measures in routine clinical practice

Introduction: Patient-reported outcome measures (PROs) were originally developed for comparing groups of people in clinical trials and population studies, and the results were used to support treatment recommendations or inform health policy, but there was not direct benefit for the participants providing PROs data. However, as the experience in using those measures increased, it became obvious the clinical value in using individual patient PROs profiles in daily practice to identify/monitor symptoms, evaluate treatment outcomes and support shared decision-making. A key issue limiting successful implementation is clinicians’ lack of knowledge on how to effectively utilize PROs data in their clinical encounters. Methods: Using a change management theoretical framework, this paper describes the development and implementation of three programs for training clinicians to effectively use PRO data in routine practice. The training programs are in three diverse clinical areas (adult oncology, lung transplant and paediatrics), in three countries with different healthcare systems, thus providing a rare opportunity to pull out common approaches whilst recognizing specific settings. For each program, we describe the clinical and organizational setting, the program planning and development, the content of the training session with supporting material, subsequent monitoring of PROs use and evidence of adoption. The common successful components and practical steps are identified, leading to discussion and future recommendations. Results: The results of the three training programs are described as the implementation. In the oncology program, PRO data have been developed and are currently evaluated; in the lung transplant program, PRO data are used in daily practice and the integration with electronic patient records is under development; and in the paediatric program, PRO data are fully implemented with around 7,600 consultations since the start of the implementation. Conclusion: Adult learning programs teaching clinicians how to use and act on PROs in clinical practice are a key steps in supporting patient engagement and participation in shared decision-making. Researchers and clinicians from different clinical areas should collaborate to share ideas, develop guidelines and promote good practice in patient-centred care

De architectuur en beleving van de hermitage (1770-1860):De hut, de heremiet(pop) en de hang naar eenzaamheid in de Nederlandse landschapstuin

De hermitage is een weinig onderzocht en begrepen tuinsieraad. De hermitage als plek om je in terug te trekken kwam voor van de oudheid tot diep in de negentiende eeuw, omdat deze mensen in verschillende perioden aansprak en eenvoudig kon worden aangepast aan de specifieke wensen van de adellijke, religieuze of intellectuele eigenaar en de smaak van de tijd. Dit artikel presenteert nieuw onderzoek naar de hermitage op de Nederlandse buitenplaats, op basis van een analyse van verkoopadvertenties, primair bronnenonderzoek (voornamelijk reisbeschrijvingen) en literatuuronderzoek. De hermitage was een wijdverbreid fenomeen in Nederland. Met de huidige inventarisatie is het aantal bekende buitenplaats-hermitages bijna verdriedubbeld van 36 naar ruim honderd. Op basis van deze nieuwe, grotere dataset bleek het mogelijk met meer precisie uitspraken te doen over de periode waarin de hermitage populair was, over de verspreiding van de hermitage in Nederland, de locatie in de tuin, het uiterlijk en de betekenis van de hermitage. Door de zoektocht in digitaal ontsloten historische kranten zijn vooral de iets latere, kleinere hermitages ontdekt. Waar Gelderland voorheen werd beschouwd als de voornaamste hermitageprovincie, is dit beeld met de huidige stand van wetenschap gekanteld in het voordeel van Holland. De burgerlijke signatuur van de hermitage is hiermee versterkt: de hermitage kwam het meest voor op de buitenplaatsen van stedelijke regenten gelegen in de nabijheid van de stad. Hermitages werden in de beginjaren vooral gezien als rariteit, en later als vast onderdeel van een landschappelijk aangelegd park. Maar ze vormden nooit het meest belangrijke tuinsieraad van een landschapspark. Uit het onderzoek blijkt dat vrijwel altijd is gekozen voor een geïsoleerde ligging. Het interieur van de hermitages was sober en bestond in de regel uit een of meerdere stoelen, een bank of bed, een doodshoofd of doodskist, een zandloper, boeken en een (houten) heremietpop. Dat de inrichting van de hermitage sterk gestandaardiseerd was, blijkt ook uit evaluaties van kluizenaarshutten in reisverslagen. De populariteit van de hermitage suggereert dat Nederlanders zeker niet ongevoelig waren voor de Romantiek, zoals nog altijd vaak wordt aangenomen. Het feit dat de bronnen zo weinig bewijs leveren voor cynisme ten aanzien van het fenomeen van de hermitage en de heremiet bevestigt deze these. Tegelijkertijd voorzag de kluizenaarshut in een meer universele behoefte, in plaats van een romantische: door de tijd heen zijn er altijd vertrekken gecreëerd waar mensen – meestal uit de elite – zich konden terugtrekken. In die zin was de hermitage een pendant van het kabinet of de studio. Wat overbleef na het opdrogen van de verheven gevoelens en gedachten van de Romantiek, was de wandeling naar de hermitage en bij aankomst de rust en het uitzicht. De Nederlandse hermitage reflecteert in alles de Nederlandse buitenplaats: beide waren vooral burgerlijk, wijdverbreid, stedelijk, bescheiden in omvang en aankleding

Views of patients and parents of children with genetic disorders on population-based expanded carrier screening

Objective Faster and cheaper next generation sequencing technologies have enabled expansion of carrier screening for recessive disorders, potentially facilitating population-based implementation regardless of ancestry or family history. Little is known, however, about the attitudes regarding population-based carrier screening among families with genetic disorders. This study assessed views among parents and patients with a recessive disorder and parents of children with Down syndrome (DS) on expanded carrier screening (ECS). Method In total, 85 patients with various recessive disorders, 110 parents of a child with a recessive disorder and 89 parents of a child with DS participated in an online survey in the Netherlands. Severity of recessive disorders was classified as mild/moderate or severe/profound. Results The majority of the (parents of) patients with a recessive disorder had a positive attitude towards population-based ECS, including screening for their own or their child's disorder. DS parents were significantly less positive towards ECS. Subgroup analyses showed that the severity of the disorder, rather than being a patient or parent, influences the attitudes, beliefs and intention to participate in ECS. Conclusion Our findings have important implications for future implementation initiatives as they demonstrate the different perspectives from people with experiential knowledge with genetic disorders

Health-related quality of life of children with first onset steroid-sensitive nephrotic syndrome

This study assessed HRQoL and emotional and behavioral difficulties (EBD) and associated variables in children with first onset SSNS. While relapsing steroid-sensitive nephrotic syndrome (SSNS) in children is associated with lower health-related quality of life (HRQoL), little is known about first onset. Four weeks after onset, children (2–16 years) and/or their parents who participated in a randomized placebo-controlled trial, completed the Pediatric Quality of Life Inventory 4.0 (PedsQL) and Strengths and Difficulties Questionnaire (SDQ) to measure HRQoL and EBD, respectively. Total and subscale scores and the proportion of children with impaired HRQoL (&gt; 1 SD below the mean of the reference group) or SDQ clinical scores (&lt; 10th and &gt; 90th percentile) were compared to the Dutch general population (reference group). Regression analyses were used to identify associated variables. Compared to the reference group, children 8–18 years reported significantly lower total HRQoL, and physical and emotional functioning. A large proportion (&gt; 45%) of these children had impaired HRQoL. There were no differences in HRQoL between children 2–7 years and the reference group, except for higher scores on social functioning (5–7 years). Similar proportions of SSNS and reference children scored within the clinical range of SDQ subscales. Age, sex, and steroid side-effects were negatively associated with HRQol and/or EBD. Conclusion: This study showed that HRQoL and EBD are affected in children of different ages with first onset SSNS. This calls for more awareness from healthcare providers and routinely monitoring of HRQoL and EBD in daily clinical care to prevent worsening of symptoms. Clinical trial registry: Netherlands Trial Register (https://trialsearch.who.int/ ; NTR7013), date of registration: 02 June 2018. What is Known: • Health-related quality of life (HRQoL) is lower and emotional and behavioral difficulties (EBD) is more affected in children with frequently-relapsing and steroid-dependent nephrotic syndrome. What is New: • HRQoL and EBD are affected in children with first onset steroid-sensitive nephrotic syndrome compared to a reference group of the Dutch general population. • To what extent HRQoL and EBD are affected depends on the age of the patient.</p

The experience of taking methotrexate for juvenile idiopathic arthritis: results of a cross-sectional survey with children and young people

Background: Children and young people (CYP) with juvenile idiopathic arthritis (JIA) are known to have impaired health-related quality of life (HRQoL), which is improved significantly for many by treatment with methotrexate (MTX). However, a significant proportion of CYP experience difficulties in taking MTX, which may reduce its potential benefits for HRQoL. The aim of this research was to examine how CYP with JIA perceive MTX treatment and how this relates to HRQoL. Methods: CYP aged 8-16 years taking MTX for JIA completed an adapted Parent Adherence Report Questionnaire, which contains 100mm visual analogue scales, to assess difficulty taking MTX, adherence, frequency of negative reactions and helpfulness of MTX. They also completed the Pediatric Quality of Life Inventory (PedsQL) Generic and Rheumatology scales. We collected data on age, gender, JIA course, disease duration, MTX duration of use, route and dose. Number of inflamed and limited joints were indicators of disease severity. Results: 116 CYP participated. Most considered MTX helpful (median 87; interquartile range (IQR) 50.75–98) and reported adherence was high (median 98; IQR 90–100). There was greater variability on scores for difficulty (median 22; IQR 2–69) and frequency of negative reactions (median 14.5; IQR 1.25–80). Mean (S.D.) scores on the PedsQL Physical and Psychosocial subscales were 71.63 (24.02) and 71.78 (19.59) respectively, indicating poorer HRQoL than that reported by healthy children. After controlling for demographic and disease variables, poorer physical HRQoL was significantly accounted for by greater difficulty in taking MTX. Poorer psychosocial HRQoL was significantly accounted for by subcutaneous MTX administration, a lower rating of MTX helpfulness and a greater reported difficulty in taking MTX. Conclusions: Taking MTX for JIA was viewed as helpful by most CYP but HRQoL was poorer in those who reported greater difficulty in taking MTX

Dutch–Flemish translation of nine pediatric item banks from the Patient-Reported Outcomes Measurement Information System (PROMIS)®

Purpose: The Patient-Reported Outcomes Measurement Information System (PROMIS®) is a new, state-of-the-art assessment system for measuring patient-reported health and well-being of adults and children. It has the potential to be more valid, reliable, and responsive than existing PROMs. The items banks are designed to be self-reported and completed by children aged 8–18 years. The PROMIS items can be administered in short forms or through computerized adaptive testing. This paper describes the translation and cultural adaption of nine PROMIS item banks (151 items) for children in Dutch–Flemish. Methods: The translation was performed by FACITtrans using standardized PROMIS methodology and approved by the PROMIS Statistical Center. The translation included four forward translations, two back-translations, three independent reviews (at least two Dutch, one Flemish), and pretesting in 24 children from the Netherlands and Flanders. Results: For some items, it was necessary to have separate translations for Dutch and Flemish: physical function—mobility (three items), anger (one item), pain interference (two items), and asthma impact (one item). Challenges faced in the translation process included scarcity or overabundance of possible translations, unclear item descriptions, constructs broader/smaller in the target language, difficulties in rank ordering items, differences in unit of measurement, irrelevant items, or differences in performance of activities. By addressing these challenges, acceptable translations were obtained for all items. Conclusion: The Dutch–Flemish PROMIS items are linguistically equivalent to the original USA version. Short forms are now available for use, and entire item banks are ready for cross-cultural validation in the Netherlands and Flanders

Late effects in pediatric allogeneic hematopoietic stem cell transplantation for nonmalignant diseases: proxy- and patient-reported outcomes

Survival rates in pediatric hematopoietic stem cell transplantation (HSCT) for nonmalignant diseases have improved due to advances in conditioning regimens, donor selection, and prophylaxis and treatment of infections and graft-versus-host disease. Insight into the long-term patient-reported outcomes (PROs) after pediatric HSCT for nonmalignant disease is lacking but essential for optimal shared decision making, counseling, and quality of care. The purpose of this research was to determine long-term patient-reported outcomes in allogeneic pediatric HSCT for nonmalignant diseases and to compare these results with Dutch reference data. This single-center cohort study evaluated PROs (PedsQL 4.0, PROMIS item banks), self- or proxy-reported, among patients at >= 2 years after pediatric allogeneic HSCT for nonmalignant disease. Mean scores were compared with those of the Dutch general population. Of 171 eligible patients, 119 participated, for a 70% response rate. The median patient age was 15.8 years (range, 2 to 49 years), and the median duration of follow-up was 8.7 years (range, 2 to 34 years). Indications for HSCT included inborn errors of immunity (n = 41), hemoglobinopathies (n = 37), and bone marrow failure (n = 41). Compared with reference data, significantly lower scores were found in adolescents (age 13 to 17 years) on the Total, Physical Health, and School Functioning PedsQL subscales. Significantly more Sleep Disturbance was reported in children (age 8 to 18 years). On the other hand, significantly better scores were seen on PROMIS Fatigue (age 5 to 7 years) and Pain Interference (age 8 to 18 years) and, in adults (age 19 to 30 years), on Depressive Symptoms and Sleep Disturbance. This study showed better or comparable very long-term PROs in patients after pediatric HSCT for nonmalignant diseases compared with the reference population. Children and adolescents seem to be the most affected, indicating the need for supportive care to prevent impaired quality of life and, more importantly, to amplify their long-term well-being. (c) 2022 The American Society for Transplantation and Cellular Therapy. Published by Elsevier Inc. This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/)Metabolic health: pathophysiological trajectories and therap