Lebensqualität bei Fabry-Patienten: Erhebung mit dem SF-36®Fragebogen Elisabeth Blohm Hintergrund: Der Morbus Fabry ist eine X-chromosomal vererbte, lysosomale Speichererkrankung bedingt durch den Mangel an dem Enzym α-Galaktosidase A. Die gesundheitsbezogene Lebensqualität von Fabry-Patienten ist im Vergleich zur Normalbevölkerung oder Patienten anderer chronischer Erkrankungen sowohl bei physischen als auch psychischen Aspekten reduziert. Es ist bekannt, dass für den Morbus Fabry typische Symptome, wie Schmerzen oder Dysfunktionen lebenswichtiger Organe, wie Herz- und Niereninsuffizienz, sowie frühzeitige Schlaganfälle zu einer signifikant verringerten gesundheitsbezogenen Lebensqualität beitragen. Fragestellung: Ziel dieser Arbeit war es, mit Hilfe des SF-36-Fragebogens die gesundheitsbezogene Lebensqualität einer großen Kohorte von Fabry-Patienten zum Zeitpunkt der ersten Vorstellung in einem spezialisierten Fabry Zentrum zu ermitteln. Gleichzeitig sollten begleitende Faktoren identifiziert werden, die mit den verschiedenen Dimensionen der psychischen und physischen Funktionsfähigkeit assoziiert sind. Dabei legten wir einen Schwerpunkt auf die Nierenfunktion. Methoden: Es wurden 99 Patienten mit Morbus Fabry eingeschlossen. Wir untersuchten die Daten des ersten Besuchs in unserem Fabry-Zentrum. Die Patientencharakteristika der Studienteilnehmer und die verschiedenen Skalen der HRQoL wurden über die CKD-Stadien unter Verwendung von ANOVA, Kruskal-Wallis-Test, χ²-Test und Fisher’s-exact-Test verglichen. Die mit den verschiedenen Dimensionen der HRQoL assoziierten Faktoren wurden mittels einer linearen Regressionsanalyse untersucht. Im multivariaten Modell wurden die folgenden Variablen in das Modell aufgenommen: Alter, Geschlecht, Nierenfunktion, Schmerzen, Schmerztherapie und vaskuläres Ereignis. Ergebnisse: Die meisten Patienten, besonders die meisten Frauen, hatten eine erhaltene Nierenfunktion, wohingegen Patienten mit eingeschränkter Nierenfunktion mit höherer Wahrscheinlichkeit männlich waren. Alle Patienten, die einer RRT erhielten, waren männlich; zwei von ihnen erhielten eine Nierentransplantation, sieben waren unter Dialyse. Eine eingeschränkte Nierenfunktion, besonders die Notwendigkeit einer RRT war über allen Skalen mit einer deutlichen Reduktion der HRQoL assoziiert. Desweiteren waren männliches Geschlecht, Schmerzen und Schmerztheapie signifikant und deutlich mit niedrigeren Werten in den SF-36-Skalen assoziiert. Im multivariaten Modell stellten sich eine eingeschränkte Nierenfunktion und Schmerzen als die Hauptfaktoren für reduzierte Werte in den physikalischen Kategorien heraus (körperliche Funktionsfähigkeit, körperliche Rollenfunktion, körperliche Schmerzen, allgemeine Gesundheitswahrnehmung und körperlicher Summenwert). Im Gegensatz dazu stellte sich heraus, dass die Notwendigkeit einer RRT mit reduzierter HRQoL in den psychischen und sozialen Kategorien assoziiert war. Schlussfolgerung: In dieser großen Kohorte von Fabry-Patienten aus einem Zentrum war die chronische Nierenerkrankung, besonders die Notwendigkeit einer RRT, ein entscheidender Faktor mir eine reduzierte HRQoL in physischen und psychisch/sozialen Aspekten des Lebens. Außerdem hatten Schmerzen eine unabhängige Beziehung mit niedrigeren Werten der physischen Skalen. Neben der Enzymersatztherapie könnten eine optimale Behandlung der Nierenerkrankung, sowie eine effektive Schmerztherapie helfen, die HRQoL von Fabry-Patienten zu verbessern. / Health Related Quality of Life in Patients with Fabry Disease: Investigation with the SF-36® questionnaire Elisabeth Blohm Background Fabry disease is an X-linked lysosomal storage disorder caused by a deficiency of the enzyme α-galactosidase A. Health related quality of life (HRQoL) in patients with Fabry disease is reduced when compared to the healthy population and to other chronic diseases in physical as well as mental aspects of well-being. Fabry specific symptoms such as pain, vital organ dysfunction including heart and renal failure or premature strokes are known to contribute significantly to impaired HRQoL Aims The purpose of the current study was to characterize HRQoL as assessed by the SF-36® questionnaire in a large cohort of patients with Fabry disease of a single center. We aimed to identify factors associated with the various dimensions of physical and mental functioning, with a focus on kidney function. Methods 99 patients with Fabry disease were enrolled. We analyzed data from the first visit at our center. Characteristics of study participants and the different scales of HRQoL were compared across CKD stages (eGFR >60 ml/min vs. eGFR <60 ml/min vs. renal replacement therapy) using ANOVA, Kruskal-Wallis test, χ²- test, and Fisher’s exact test. Factors associated with the various dimensions of HRQoL were examined using linear regression analysis. In multivariate analyses, the following variables were included in the model: age, gender, kidney function, pain, pain-therapy, and vascular event. Results Most of the patients, in particular most women, had preserved kidney function, whereas patients with impaired kidney function were more likely to be male. All patients receiving RRT were of male gender, of which two underwent kidney transplantation and seven received dialysis. Impaired kidney function, particularly the need of RRT, was associated with markedly reduced HRQoL across all scales. Furthermore, male gender, pain and pain-therapy were significantly and meaningfully associated with lower scores on the SF-36 scales. In multivariate modeling, impaired kidney function and pain emerged as the main factors associated with lower scores in physical aspects of life (physical functioning, role physical, bodily pain, general health, PCS). In contrast, only the need for RRT was found to be associated with reduced HRQoL in mental/social parameter dimensions. Conclusion In this large cohort of Fabry patients recruited in a single center, chronic kidney disease, particularly the need of RRT, was a crucial factor for reduced HRQoL in physical and mental/social aspects of life. Furthermore, pain was independently related to lower scores on physical scales. Aside from enzyme replacement therapy, optimal treatment of kidney disease and an effective pain therapy may help to improve HRQoL in Fabry patients.

No description available.

Chronic diseases such as diabetes have become increasingly prominent around the world. Diabetes care requires a combination of medication, exercise, diet, self-management education, and psychosocial care to reduce the risk of long-term complications. Following this strict regimen can have an adverse effect on the patient's quality of life. Quality of life is a broad concept that is difficult to define and study. This study focused on the health-related component of quality of life. Diabetes is reaching epidemic proportions in Kuwait. While the incidence of the disease is documented, little is known about its effect on the life of the Kuwaiti population. This is partly due to the lack of valid Arabic versions of health-related quality of life (HRQOL) instruments. The purpose of this study was to investigate the construct validity of two HRQOL measures in a sample of older adults in Kuwait with Type 2 diabetes. The two measures were the Short Form 36-Item Health Survey version 2.0, a generic measure, and the Problem Areas in Diabetes survey, a disease-specific measure. A total of 240 Kuwaiti citizens over the age of 55 years living in Kuwait City participated in this study. Participants were divided into two groups: the Diabetes Group included 120 older adults with diabetes and the Control Group included 120 older adults without diabetes. Three approaches were used to assess the construct validity of the Arabic versions of the SF-36v2 Health Survey and the Problem Areas in Diabetes survey: factor analysis, correlation, and known-group method. The results of this study support the use of the Arabic versions of the two surveys to measure HRQOL of older adults in Kuwait with Type 2 diabetes. Standards for construct validity were well met, with few exceptions. / Physical Therapy

<p>The aim with this thesis was to study cardiovascular disease and hypertension, use of drugs and health from an epidemiological perspective. Various methods - self-rated health (SRH), health related quality of life (HRQL) - the 36-item short form questionnaire (SF-36) - and health utility measurements - the rating scale (RS) and the time-trade off (TTO) methods - were employed.</p><p>Data from the Swedish Adoption/Twin Study of Aging (SATSA) in 1984, 1987, 1990, and 1993 as well as a general population survey conducted in Uppsala County in 1995 were used.</p><p>Persons who have cardiovascular disease, both with and without drug treatment, were found to have a lower SRH as compared to others in the population. Longitudinal analyses showed that SRH was relatively stable over time among persons with cardiovascular disease. Both having a low SRH and having cardiovascular disease were associated with a higher mortality rate.</p><p>Hypertensives were found to have a lower HRQL than do others in the general population as measured by the SF-36. The lowest scoring was found in the general health perception scale (GH), whereas role emotional (RE) and mental health (MH) were the scales least affected by hypertension.</p><p>Nearly 20 percent of the antihypertensive drug users reported side effects.The pattern of side effects was similar to that reported in clinical trials. Both hypertension itself and the drug treatment were found to have an impact on the patient's health-state utility as measured by the RS. Comparative analyses showed that health utilities and psychometric quality-of-life instruments were only moderately correlated among hypertensives. </p><p>The results also showed that inequalities in HRQL were present with respect to several sociodemographic factors. </p><p>In summary, this thesis revealed that persons with cardiovascular disease and/or with hypertension experience poorer health than others in the population. The poor health may be caused both by the disease and/or the drug treatment. The results in this thesis also suggested that special attention and care should be directed to persons with cardiovascular disease and/or hypertension reporting ill health. This especially is important given that low HRQL can be a riskfactor for subsequent cardiovascular events or complications which in turn might result in higher mortality rate.</p>

The aim with this thesis was to study cardiovascular disease and hypertension, use of drugs and health from an epidemiological perspective. Various methods - self-rated health (SRH), health related quality of life (HRQL) - the 36-item short form questionnaire (SF-36) - and health utility measurements - the rating scale (RS) and the time-trade off (TTO) methods - were employed. Data from the Swedish Adoption/Twin Study of Aging (SATSA) in 1984, 1987, 1990, and 1993 as well as a general population survey conducted in Uppsala County in 1995 were used. Persons who have cardiovascular disease, both with and without drug treatment, were found to have a lower SRH as compared to others in the population. Longitudinal analyses showed that SRH was relatively stable over time among persons with cardiovascular disease. Both having a low SRH and having cardiovascular disease were associated with a higher mortality rate. Hypertensives were found to have a lower HRQL than do others in the general population as measured by the SF-36. The lowest scoring was found in the general health perception scale (GH), whereas role emotional (RE) and mental health (MH) were the scales least affected by hypertension. Nearly 20 percent of the antihypertensive drug users reported side effects.The pattern of side effects was similar to that reported in clinical trials. Both hypertension itself and the drug treatment were found to have an impact on the patient's health-state utility as measured by the RS. Comparative analyses showed that health utilities and psychometric quality-of-life instruments were only moderately correlated among hypertensives. The results also showed that inequalities in HRQL were present with respect to several sociodemographic factors. In summary, this thesis revealed that persons with cardiovascular disease and/or with hypertension experience poorer health than others in the population. The poor health may be caused both by the disease and/or the drug treatment. The results in this thesis also suggested that special attention and care should be directed to persons with cardiovascular disease and/or hypertension reporting ill health. This especially is important given that low HRQL can be a riskfactor for subsequent cardiovascular events or complications which in turn might result in higher mortality rate.

Abstract The aim of this study was to evaluate the results of lumbar spine surgery and determine which factors modify outcome. A follow-up questionnaire, the Beck Depression Inventory (BDI, the Short Form 36 Health Survey (SF-36) and the Oswestry Low Back Disability Questionnaire (ODI) were sent to working-aged patients who had undergone lumbar spine surgery in the Oulu University Hospital between June, 2005 and May, 2008. Those with a BDI &#8805; 10 were further classified into either non-melancholic (NmDS) or melancholic depression (MDS) groups. Potential spinal cord stimulation (SCS) candidates were interviewed via telephone. The postal survey was sent to 814 patients, of which 537 (66%) replied. Of these, 361 had undergone disc surgery, 85 stabilizing surgery and 91 decompression. Pain intensity was milder, the frequency of pain more rare, functional disability minimal and quality of life better after disc surgery compared to stabilizing surgery and decompression, which are technically more demanding operations and the patients’ condition are often more serious. Altogether, 213 patients presented with depressive symptoms (DS) defined as having a BDI &#8805; 10, and these were further classified into NmDS (n = 153) and MDS (n = 60) subtypes. ODI differed between DS subtypes: those without DS had minimal, NmDS moderate and MDS severe functional disability. Pain was more frequent and more intense among DS patients. In particular, MDS patients suffered from pain, used more pain medication, but received less benefit from it. Disc surgery patients were divided according to body mass index (BMI): normal, pre-obese and obese. Pre-obese and obese patients gained weight during the follow-up. Obese patients had more DS and a worse functional outcome than normal-weighted or pre-obese patients. Of the entire cohort (n = 814), 21 patients received SCS. Eleven respondents underwent SCS treatment after they had replied. Features predicting SCS treatment were daily or continuous pain, higher pain intensity with predominant radicular pain, more severe pain-related functional disability, more DS and reduced benefit from pain medication. The time between lumbar surgery and implantation of a SCS device was extensive. Based on data from phone interviews, it appears that SCS was not offered to all potential candidates. In conclusion, the outcome of lumbar spine surgery was good after disc surgery but less favourable after more demanding stabilizing surgery or decompression. DS, especially of the MDS subtype, and obesity were more often seen in patients with a poorer surgical outcome. SCS treatment was used late and only for patients with very severe pain. / Tiivistelmä Tutkimuksen tarkoitus oli arvioida tuloksia lannerangan kirurgiassa ja kartoittaa tekijöitä, jotka vaikuttavat leikkaustulokseen. Seurantakysely, Beckin depressio¬kysely (BDI), SF-36 elämänlaatukysely ja Oswestryn toiminta¬kyky¬kysely (ODI), lähetettiin työikäisille Oulun yliopistollisessa sairaalassa kesäkuu 2005 - maaliskuu 2008 alaselkäleikatuille potilaille. Beckin depressioasteikko &#62; 10 luokiteltiin ei-melankolisesti masentuneisiin (NmDS) ja melankolisesti masen¬tuneisiin (MDS). Mahdolliset takajuostestimulaatio (TJS) -ehdokkaat haastateltiin puhelimitse. Postikysely lähetettiin 814 potilaalle, joista 537 (66%) vastasi. Näistä 361:lle tehtiin välilevytyräleikkaus, 85:lle stabiloiva leikkaus ja 91:lle juurikanavan avarrusleikkaus. Välilevytyräleikatuilla kipu oli lievempää, harvemmin esiintyvää, toiminnallinen haitta vähäisempää ja elämänlaatu parempaa verrattuna potilaisiin, joille tehtiin vaativampi stabiloiva tai juurikanavan avarrusleikkaus. Kaikkiaan 213 potilaalla oli depressio-oireita (DS, BDI &#8805; 10) ja nämä luokiteltiin edelleen NmDS (n = 153) ja MDS (n = 60) alaryhmiin. ODI erottui eri DS alatyyppien välillä: ei-DS-potilailla oli minimaalinen, NmDS-potilailla kohtuullinen ja MDS-potilailla vaikea toiminnallinen haitta. Kipua oli useammin ja voimakkaampana DS-potilailla. Erityisesti MDS-potilaat kärsivät kivuista, käyttivät enemmän kipulääkkeitä ja hyötyivät niistä vähemmän. Välilevytyräleikatut luokiteltiin painoindeksin (BMI) pohjalta normaaleihin, ylipainoisiin ja lihaviin. Ylipainoiset ja lihavat lihoivat seuranta-aikana. Lihavilla potilailla oli enemmän masennusta ja huonompi toiminnallinen tulos verrattuna normaaleihin ja ylipainoisiin. Koko tutkimusryhmässä (n = 814) 21 potilasta oli saanut TJS:n. Yksitoista vastaajaa sai TJS:n kyselytutkimuksen jälkeen. TJS:n saaneilla oli päivittäistä tai jatkuvaa, kovempaa ja pääasiassa jalkaan säteilevää kipua. Kipu aiheutti enemmän toiminnallista haittaa, enemmän masennusta ja nämä saivat vähemmän apua kipulääkityksestä. Aika leikkauksen ja TJS:n asennuksen välillä oli pitkä. Puhelinhaastattelun avulla saatu tieto osoittaa, ettei TJS-hoitoa tarjota kaikille potentiaalisille hyötyjille. Yhteenvetona voidaan todeta, että välilevytyräleikkauksen jälkeen tulos oli hyvä ja vaativampien stabiloivan ja juurikanavan avarrusleikkauksen jälkeen heikompi. DS, etenkin MDS ja lihavuus korostuivat huonommin toipuneissa. TJS-hoitoa käytettiin vain vaikeimmille tapauksille ja odotusajat olivat pitkät.