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Cystic fibrosis (CF) is a complex disease which requires interdisciplinary care in specialized CF centres. In Germany, 97 paediatric and adult outpatient clinics agreed to report clinical data of their patients to a newly established registry, the Cystic Fibrosis Quality Assurance (CFQA) project. This article characterizes the design of the CFQA an...
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... With this in mind, published data suggest that malnutrition prevails in 12-74% of pediatric patients with CF [21][22][23][24][25][26]. Some earlier studies using the stricter cut-off of the 15th BMI percentile to identify malnutrition support similar findings, with malnutrition being diagnosed in 20-30% of pediatric patients [27][28][29][30]. Nevertheless, very few studies have examined the risk for malnutrition among pediatric patients with CF. ...
Malnutrition prevails in considerable proportions of children with Cystic Fibrosis (CF), and is often associated with adverse outcomes. For this, routine screening for malnutrition is pivotal. In the present cross-sectional study, we aimed to assess the risk for malnutrition in pediatric outpatients with CF. A total of 76 outpatients (44 girls, 11.9 ± 3.9 years old, 39.5% adolescents) were recruited and anthropometric, clinical, dietary and respiratory measures were collected. All outpatients were screened for malnutrition risk with a validated disease-specific instrument. Most children exhibited a low risk for malnutrition (78.9%), whereas none of the participants were characterized as having a high malnutrition risk. In the total sample, malnutrition risk was positively associated with age (r = 0.369, p = 0.001), and inversely related to the body mass index (r = −0.684, p < 0.001), height z-score (r = −0.264, p = 0.021), and forced expiratory volume (FEV 1 %, r = −0.616, p < 0.001). Those classified as having a low malnutrition risk were younger (p = 0.004), heavier (p < 0.001) and taller (p = 0.009) than their counterparts with a moderate risk. On the other hand, patients in the moderate risk group were more likely pubertal (p = 0.034), with a reduced mid-upper arm fat area (p = 0.011), and worse pulmonary function (p < 0.001). Interestingly, none of the children attaining ideal body weight were classified as having a moderate malnutrition. risk, whereas 37.5% of the patients allocated at the moderate risk group exhibited physiological lung function. In this cohort of outpatients with CF that were predominantly well-nourished and attained physiological lung function, malnutrition risk was identified only in small proportions of the sample. Our data support that patients that are older, pubertal, or have diminished fat mass are at greater risk for malnutrition.
... CF patients grow poorly at all ages, they are short up to early adulthood, but they achieve normal fi nal height in their mid-20s. Wiedemann et al. [ 23 ] stated that in a group of 4306 CF patients, the initially low height SDS increased with age and normal height was achieved by almost all patients when reaching the adult age. ...
The human growth pattern with different developmental stages is a result of evolutionary trade‐offs showing specific features. The prolonged childhood and adolescence with their typical growth spurts is associated with age‐ and sex‐specific development of maturation signs, and changes of body proportions and dimensions. Biological age can be estimated with different methods and helps to classify human growth‐specific biological age stages. These stages include typical body markers, but also sociocultural and cognitive signs. Age‐specific growth velocity patterns and changes in proportions and dimensions are genetically fixed. Humans exhibit phenotypic plasticity that can result in secular trends. Conventionally, increases of body height and earlier maturation are explained by better nutrition and living conditions. However, strategic growth adjustments are being considered as possible explanations for the secular increase in height. In addition, reductions in some skeletal proportions in contemporary populations are assumed to be the result of reduced physical activity.
... However, this explanation may be too simplistic. Some studies show that nowadays neither infectious diseases nor undernutrition (except of severe continuous starvation up to the very end of growth period) seem to influence final stature (Hermanussen, 2013;Wiedermann et al., 2001). Moreover, despite the findings that poor childhood health increases morbidity in later life, no support is found for using adult height as a proxy for the effects of childhood health experiences (Blackwella et al., 2001). ...
The aim of this study was to test the null hypothesis that no relationship between maximal living stature and adult lifespan had existed in prehistoric and historic Lithuanian populations. The sample analyzed consisted of 1713 skeletons of adult individuals who died between the 2nd century CE and the beginning of the 19th century CE, collected from 118 archaeological sites located in the current territory of Lithuania. A cumulative logit model was applied to model ordinal age-related changes in the auricular surface and the pubic symphysis (measures of lifespan) as a function of maximal length of femur (an indicator of maximal living stature), sex, burial site and its chronology as a proxy for the place of residence and period. The lack of strong association between adult lifespan and femur length was observed for males and females, various periods and places of residence. However, the results failed to reject convincingly the hypothesis that no relationship between these two variables had existed in preindustrial Lithuanian populations. In addition to concerns regarding sampling and measurement errors, heterogeneous frailty in early life and resulting selective mortality may partly explain the results obtained.
... According to BAZ, the patients were classified as underweight (BAZ < −2.0), attaining normal body weight (−2.0 ≤ BAZ < 1.0), overweight (BAZ ≥ 1.0) or obese (BAZ ≥ 2.0), while weight-for-height and height-for-age z-scores were used to define wasting and stunting [14]. Additional classification was considered regarding ideal BAZ for CF (BAZ ≥ 0.0) [10], and nutritional failure as BAZ < −1.04, a z-score corresponding to the 15th percentile of the BMI-for-age growth curves [15][16][17]. Waist circumference (WC) was measured according to the method proposed by the WHO [18], and abdominal obesity was diagnosed according to the International Diabetes Federation cutoffs [19]. On the left side of the body, mid-upper arm circumference (MUAC) was measured at the mid-point between the olecranon process and the acromium, while triceps skinfold thickness (TSK) values were taken 3 times (Harpenden Skinfold Calipers, Batty International, Burgess Hill, England) in order to attain a median value. ...
... Nutritional failure was apparent in 17.9% of the sample, lower compared to Italian (20.9%) [15] or German pediatric CF populations (28.6%) [16]. Additionally, our patients exhibited malnutrition in smaller proportions than that recorded in a previous Greek cohort of CF patients, given care in Northern Greece (17.9% vs. 22.1%) [23], although we should note that direct comparison with the aforementioned study is unavailable as the stated disparities may be attributed to using a more strict cut-off (15th BMI percentile over the 10th) to assess malnourishment in our study, as well as our study focusing only on the pediatric CF population. ...
Background: Pediatric cystic fibrosis (CF) patients suffer high rates of undernutrition, subject to several parameters. We aimed to assess growth and nutritional status of Greek children and adolescents with CF.
Methods: Eighty-four patients (35 boys) formed the sample. Anthropometrics and FEV1 were measured, growth and weight status were assessed. Body mass index (BMI), arm circumference (MUAC), fat (MUAFA) and muscle (MUAMA) were calculated.
Results: In the total sample, 6.0% of the patients were underweight, 4.8% stunted, 8.3% wasted and 17.9% in nutritional failure, whereas 59.5% attained the ideal BMI for CF. FEV1 positively associated with BMI (B=0.03, p≤0.003), weight (B=0.03, p≤0.003) and MUAMA z-scores (B=0.04, p≤0.005). Meconium ileus negatively associated with FEV1 (B=−14.17, p≤0.003) and stature (B=−0.65, p≤0.043). Pancreatic insufficiency negatively influenced MUAC and MUAFA z-scores (p≤0.05 for both).
Conclusions: The examined CF patients appear to be thriving. Unlike published research, the participants’ sex, gene mutation and acquisition of pathogens did not affect growth.
... CF patients grow poorly at all ages, they are short up to early adulthood, but they achieve normal fi nal height in their mid-20s. Wiedemann et al. [ 23 ] stated that in a group of 4306 CF patients, the initially low height SDS increased with age and normal height was achieved by almost all patients when reaching the adult age. ...
Growth is a dynamic process and has a characteristic pattern with three major growth spurts: the postnatal spurt followed by rapid growth deceleration during infancy and early childhood; the mild mid-growth spurt around the age of 6–7 years that however remains invisible in most velocity charts; and the adolescent growth spurt that parallels full sexual maturation. The maximum peak in height velocity of the adolescent spurt tends to be higher in early than in late-maturing individuals. Body weight follows a very similar pattern. Short-term growth is characterised by irregular sequences of mini growth spurts at random intervals of a few days. Measurements at intervals of less than 24 h are affected by circadian variation.
Growth is the increase of size (amplitude) over time. But individuals differ in developmental tempo: their calendar age may not parallel their biological age. Good clinical practice attempts to separately assess tempo and amplitude. Three techniques are currently available to assess the physical aspect of tempo: (1) assessing skeletal maturity, (2) assessing the state of pubertal maturity and (3) plotting multiple records of height SDS. Human beings follow a cephalocaudal (head-to-tail) gradient of growth. Adolescent growth proceeds in a distal-to-proximal pattern. Sitting height is a useful parameter to estimate the trunk–leg proportion. Particularly in younger children, the increase in body length largely reflects the increase in leg length.
Secular trends in developmental tempo and in adolescent growth exist leading to very characteristic population patterns of growth when recorded at different historic moments.
... Several quality management programmes have evolved, with Centre comparison as a primary motivation. Early registry development in Germany was called the Cystic Fibrosis Quality Assurance (CFQA) project [16], and occurred long before the idea of publishing Centre-specific summaries was deemed acceptable in other regions. Whereas most registries report survival into the middle adult years, a report from South America [17] is a sobering reminder that attention to the quality and delivery of care is of primary importance. ...
... There have been several key advances in this area over the past decade. German CF Centres have made quality improvement a major focus in their CF care [16,30]. This project was able to demonstrate first the temporal improvements in outcome and then improvements in care but importantly, it demonstrated variation in care practices among the 93 Centres in Germany. ...
Od początków leczenia mukowiscydozy (CF) dane pacjentów były zapisywane oraz analizowane w celu identyfikacji czynników prowadzących do bardziej korzystnych skutków leczenia. Ogromne repozytoria danych, takie jak Rejestr Mukowiscydozy w USA, stworzony w latach 60. XX w., umożliwiły nagłośnienie sukcesów w leczeniu oraz wyników pacjentów, jak również wdrożenie programów poprawy przez specjalistyczne ośrodki CF. Większe ilości danych dostępne w bazach danych ośrodków i rejestrach pacjentów w ostatnich dekadach spowodowały, że możliwe stało się porównywanie różnych terapii, standardów opieki, a także rejestrowanie danych. Zaczęto skupiać się na standardach opieki nad poszczególnymi pacjentami na różnych poziomach: pacjenta, ośrodka, regionalnym, krajowym oraz międzynarodowym. Publikacja ta stanowi przegląd dotyczący zarządzania jakością oraz kwestii jej poprawy na każdym z wyżej wymienionych poziomów, ze szczególnym uwzględnieniem wskaźników zdrowia, roli ośrodków CF, sieci regionalnych, krajowych polityk zdrowotnych, a także rejestracji i porównania międzynarodowych danych.
... Z upływem czasu wyewoluowało wiele programów zarządzania jakością, których główną motywacją były porównania między ośrodkami. Wczesny rozwój rejestru w Niemczech nazwano projektem Zapewnienia Jakości w zakresie CF (CFQA) [16] i miał on miejsce na długo, zanim pomysł opublikowania specyficznych dla ośrodków podsumowań zyskał akceptację w innych regionach. Podczas gdy większość rejestrów odnotowuje przeżycie pacjentów do wieku średniego, raport z Ameryki Południowej [17] stanowi trzeźwiące przypomnienie, że uwaga skierowana na jakość i dostarczaną opiekę ma kluczowe znaczenie. ...
... Na polu tym w ciągu ostatniej dekady miało miejsce kilka kluczowych postępów. Ośrodki CF w Niemczech postawiły poprawę jakości za swój główny cel w zakresie opieki nad chorymi na CF [16,30]. Projekt ten zademonstrował również pierwszą czasową poprawę w wynikach, a następnie w zakresie opieki, aleco szczególnie ważnewykazał różnice w praktykach pomiędzy 93 ośrodkami na terenie Niemiec. ...
... Much has been written in recent years about improving quality of care for patients with cystic fibrosis (CF) [1][2][3][4][5][6][7][8]. Evidence points to improved patient outcomes associated with the development of quality improvement processes in CF care sites [9,10], and benchmarking efforts in both the United States and Germany have identified structural factors and care processes within care sites that are associated with improved patient outcomes [4,6]. ...
Assessing cystic fibrosis (CF) patient quality of care requires the choice of an appropriate outcome measure. We looked systematically and in detail at pulmonary function outcomes that potentially reflect clinical practice patterns.
Epidemiologic Study of Cystic Fibrosis data were used to evaluate six potential outcome variables (2002 best FVC, FEV1, and FEF25-75 and rate of decline for each from 2000 to 2002). We ranked CF care sites by outcome measure and then assessed any association with practice patterns and follow-up pulmonary function.
Sites ranked in the top quartile had more frequent monitoring, treatment of exacerbations, and use of chronic therapies and oral corticosteroids. The follow-up rate of pulmonary function decline was not predicted by site ranking.
Different pulmonary function outcomes associate slightly differently with practice patterns, although annual FEV1 is at least as good as any other measure. Current site ranking only moderately predicts future ranking.
Copyright © 2014 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.
... Several quality management programmes have evolved, with Centre comparison as a primary motivation. Early registry development in Germany was called the Cystic Fibrosis Quality Assurance (CFQA) project [16], and occurred long before the idea of publishing Centre-specific summaries was deemed acceptable in other regions. Whereas most registries report survival into the middle adult years, a report from South America [17] is a sobering reminder that attention to the quality and delivery of care is of primary importance. ...
... There have been several key advances in this area over the past decade. German CF Centres have made quality improvement a major focus in their CF care [16,30]. This project was able to demonstrate first the temporal improvements in outcome and then improvements in care but importantly, it demonstrated variation in care practices among the 93 Centres in Germany. ...
Since the earliest days of cystic fibrosis (CF) treatment, patient data have been recorded and reviewed in order to identify the factors that lead to more favourable outcomes. Large data repositories, such as the US Cystic Fibrosis Registry, which was established in the 1960s, enabled successful treatments and patient outcomes to be recognized and improvement programmes to be implemented in specialist CF centres. Over the past decades, the greater volumes of data becoming available through Centre databases and patient registries led to the possibility of making comparisons between different therapies, approaches to care and indeed data recording. The quality of care for individuals with CF has become a focus at several levels: patient, centre, regional, national and international. This paper reviews the quality management and improvement issues at each of these levels with particular reference to indicators of health, the role of CF Centres, regional networks, national health policy, and international data registration and comparisons.
... [17][18][19][20][21] One unique model of group improvement is the German CF Quality Assurance project, which has specifically aimed to develop clinical goals for its participating programmes based on benchmarks derived from registry data. [22][23][24][25] Quality indicators were selected (airway cultures free of Pseudomonas aeruginosa, nutritional measures, lung function, and lack of serious complications). During two annual conferences, the highest-ranking programmes for these indices presented their treatment strategies, and the ensuing discussions led to the identification of clinical practices that programmes would aspire to adopt. ...
Benchmarking is the process of using outcome data to identify high-performing centres and determine practices associated with their outstanding performance. The US Cystic Fibrosis Foundation (CFF) Patient Registry contains centre-specific outcomes data for all CFF-certified paediatric and adult cystic fibrosis (CF) care programmes in the USA. The CFF benchmarking project analysed these registry data, adjusting for differences in patient case mix known to influence outcomes, and identified the top-performing US paediatric and adult CF care programmes for pulmonary and nutritional outcomes. Separate multidisciplinary paediatric and adult benchmarking teams each visited 10 CF care programmes, five in the top quintile for pulmonary outcomes and five in the top quintile for nutritional outcomes. Key practice patterns and approaches present in both paediatric and adult programmes with outstanding clinical outcomes were identified and could be summarised as systems, attitudes, practices, patient/family empowerment and projects. These included: (1) the presence of strong leadership and a well-functioning care team working with a systematic approach to providing consistent care; (2) high expectations for outcomes among providers and families; (3) early and aggressive management of clinical declines, avoiding reliance on 'rescues'; and (4) patients/families that were engaged, empowered and well informed on disease management and its rationale. In summary, assessment of practice patterns at CF care centres with top-quintile pulmonary and nutritional outcomes provides insight into characteristic practices that may aid in optimising patient outcomes.
