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1.
BMC Cancer ; 24(1): 462, 2024 Apr 13.
Article in English | MEDLINE | ID: mdl-38614966

ABSTRACT

BACKGROUND: Patients with metastatic gastric cancer (mGC) have poor prognosis. This real-world study aimed to describe treatment regimens and survival of mGC patients. METHODS: A retrospective analysis was conducted using anonymized German claims data (AOK PLUS) covering a period from 2010 to 2021. The study population included newly diagnosed mGC cases identified from 2011 to 2020. The index date was defined as the first diagnosis of metastasis on or after gastric cancer diagnosis. Therapy regimens were identified based on inpatient and outpatient data, and subsequently stratified by line of treatment. Survival analyses were conducted using the Kaplan-Meier method. RESULTS: The cohort consisted of 5,278 mGC incident cases (mean age: 72.7 years; male: 61.9%). Nearly half of the incident cases received mGC-related treatment (49.8%). Treated patients were more often male, younger, and had fewer comorbidities compared to untreated patients. Of the 2,629 mGC patients who started the first line of treatment (1LOT), 32.8% switched to 2LOT, and 10.2% reached 3LOT. Longer survival time was observed among disease-specific treated cases compared with untreated cases (median real-world overall survival (rwOS): 12.7 months [95%CI 12.1 - 13.3 months] vs. 3.7 months [95%CI 3.4 - 4.0 months]). CONCLUSION: Systemic therapy was not received in almost half of the mGC patients. In those patients, a very short median rwOS was observed. Treatment patterns were generally in line with the guideline recommendations, however, therapy switching rates and poor prognosis indicate high unmet needs also in the treated population.


Subject(s)
Splenic Neoplasms , Stomach Neoplasms , Humans , Male , Aged , Stomach Neoplasms/epidemiology , Stomach Neoplasms/therapy , Retrospective Studies , Inpatients , Outpatients , Germany/epidemiology
2.
Isr Med Assoc J ; 26(4): 211-215, 2024 Apr.
Article in English | MEDLINE | ID: mdl-38616664

ABSTRACT

BACKGROUND: On 7 April 1933, the Nazi Law for the Restoration of the Professional Civil Service was enacted. The law triggered the dismissal of most Jewish medical staff from German universities. A few Jewish professors in Berlin were permitted to continue their academic activity with restrictions. Those professors were gradually dismissed as laws and restrictions were enforced. OBJECTIVES: To identify the last Jewish medical professors who, despite severe restrictions, continued their academic duties and prepared students for their examinations in Berlin after the summer of 1933. METHODS: We reviewed dissertations written by the medical faculty of Berlin from 1933 to 1937 and identified Jewish professors who mentored students during those years. RESULTS: Thirteen Jewish tutors instructed dissertations for the medical examinations after the Nazi regime seized power. They were employees of different university hospitals, including the Jewish hospitals. We did not identify Aryan students instructed by Jewish professors. The professors were active in different medical disciplines. Half of the reviewed dissertations were in the disciplines of surgery and gynecology. The last Jewish tutors were dismissed in October 1935. However, some of their studies were submitted for examination after that date. CONCLUSIONS: After the Nazi regime seized power, academic activities and medical research by Jewish professors declined but did not stop. However, these professors worked with only Jewish students on their theses. Most dissertations were approved and examined after the Jewish academics were dismissed by the university, in some cases even after they left Germany.


Subject(s)
Education, Medical , Jews , Humans , Berlin , Germany , Judaism
4.
Rehabilitation (Stuttg) ; 63(2): 81-88, 2024 Apr.
Article in English | MEDLINE | ID: mdl-38626789

ABSTRACT

BACKGROUND AND PURPOSE: In the last decades, Germany has experienced a vast increase in the number of individuals applying for or receiving disability benefit. Only a small proportion of them intends to return to work. The present study aims to identify and describe the percentage of temporary disability pensioners, who have the intention to return to working life. METHODS: The final sample consisted of 496 insured individuals who were receiving temporary disability pension from the Regional German Pension Insurance (DRV) at that time. Participation was voluntary and anonymous. The following self-report instruments were administered: a. Evaluation of Contextual Factors; b. German Symptom Validation Test (BEVA); c. German Patient Health Questionnaire (PHQ-9); d. Readiness for Return-to-Work German Scale (RRTW). RESULTS: The results revealed that approximately 6% of participants were at the stage of "preparation", 31.9% at the stage of "contemplation", and more than half of the participants were at the stage of "pre-contemplation" according to the RRTW. Comparing the groups of "pre-contemplation" (Group 1) and "contemplation+preparation" (Group 2+3), no statistically significant differences were found, except for age. CONCLUSIONS: In conclusion, further research is required to identify possible factors which could predict the stage of RRTW. New interventions are needed that can raise the motivation for and success in the return to work of disability pensioners.


Subject(s)
Disabled Persons , Return to Work , Humans , Intention , Germany/epidemiology , Pensions
5.
Rehabilitation (Stuttg) ; 63(2): 89-99, 2024 Apr.
Article in German | MEDLINE | ID: mdl-38626790

ABSTRACT

PURPOSE: Based on the experience during the SARS-CoV-2 pandemic, the study aimed to derive facilitating and hindering factors in the implementation of medical rehabilitation during future pandemics in adolescents with scoliosis. METHODS: A qualitative study design with guided expert interviews was chosen. Twelve interviews with adolescents and seven interviews with physiotherapists were conducted. The evaluation was carried out using qualitative content analysis according to Mayring and inductive categorization. RESULTS: For 83.3% of the adolescents a therapy considering individual patient needs was a facilitator. Good information management (91.7%), continued availability of leisure activities (66.7%), and a high perception of safety (100%) were facilitating. 71.4% favoured outdoor therapy. The increased exertion caused by wearing a mask (91.7%) and the shortened therapy times (66.7%) were seen as barriers. For 75.0% of the adolescents, social contacts were more difficult. All therapists stated that communication between patients and therapists was more difficult. CONCLUSION: For successful rehabilitation during the SARS-CoV-2 pandemic, depending on weather conditions, therapy should be outside, as there is no need to wear mouth-nose protection outdoors. In addition, the physiotherapeutic treatment (Schroth therapy) should be carried out without mask due to the intensity of the exercise. Social contacts among all adolescents should be supported as much as possible. The leisure activities should provide a wide choice and enough free places. Continuation of certain hygienic measures could help to reduce the incidence of illnesses (e. g., gastrointestinal infections) in the future. These were barely noticed in rehabilitation facilities during the pandemic.


Subject(s)
COVID-19 , Scoliosis , Humans , Adolescent , SARS-CoV-2 , Pandemics , Germany/epidemiology
6.
Rehabilitation (Stuttg) ; 63(2): 119-130, 2024 Apr.
Article in German | MEDLINE | ID: mdl-38626791

ABSTRACT

As a result of increasingly individualized and multimodal therapy, prognosis of breast cancer has improved significantly over the last years. However, multimodal treatment and the use of new medications can lead to a variety of somatic, sometimes new, side effects such as fatigue, polyneuropathy or autoimmune toxicities. This and the oncological diagnosis lead to a high level of psychological distress in the women affected and often to subsequent psychological disorders (sleep/anxiety disorders, depression, ...). Both the diverse complaints after oncological therapy and the increasingly improved overall prognosis underline the importance of multimodal rehabilitation concepts to improve quality of life and successful professional reintegration.In the following, these secondary disorders after breast cancer, their multimodal therapy and their significance for social-medical performance assessment are presented in more detail.


Subject(s)
Breast Neoplasms , Female , Humans , Breast Neoplasms/psychology , Depression , Quality of Life/psychology , Germany , Anxiety/psychology , Anxiety/therapy , Fatigue/complications
7.
BMC Emerg Med ; 24(1): 62, 2024 Apr 14.
Article in English | MEDLINE | ID: mdl-38616266

ABSTRACT

BACKGROUND: Emergency medical service (EMS) workers face challenging working conditions that are characterized by high stress and a susceptibility to making errors. The objectives of the present study were (a) to characterize the psychosocial working conditions of EMS workers, (b) to describe the perceived quality of patient care they provide and patient safety, and (c) to investigate for the first time among EMS workers associations of psychosocial working conditions with the quality of patient care and patient safety. METHODS: For this cross-sectional study, we carried out an online survey among 393 EMS workers who were members of a professional organization. Working conditions were measured by the Demand-Control-SupportQuestionnaire (DCSQ) and seven self-devised items covering key stressors. Participants reported how often they perceived work stress to affect the patient care they provided and we inquired to what extent they are concerned to have made a major medical error in the last three months. Additionally, we used parts of the Emergency Medical Services - Safety Inventory (EMS-SI) to assess various specific errors and adverse events. We ran descriptive analyses (objective a and b) and multivariable logistic regression (objective c). RESULTS: The most common stressors identified were communication problems (reported by 76.3%), legal insecurity (69.5%), and switching of colleagues (48.9%) or workplaces (44.5%). Overall, 74.0% reported at least one negative safety outcome based on the EMS-SI. Concerns to have made an important error and the perception that patient care is impaired by work stress and were also frequent (17.8% and 12.7%, respectively). Most psychosocial working conditions were associated with the perception that patient care is impaired due to work stress. CONCLUSIONS: Work stress in EMS staff is pronounced and negative safety outcomes or potential errors are perceived to occur frequently. Poor psychosocial working conditions were only consistently associated with perceived impairment of patient care due to work stress. It seems necessary to reduce communication problems and to optimize working processes especially at interfaces between emergency services and other institutions. Legal insecurity could be reduced by clarifying and defining responsibilities. Communication and familiarity between team colleagues could be fostered by more consistent composition of squads.


Subject(s)
Emergency Medical Services , Occupational Stress , Humans , Cross-Sectional Studies , Patient Safety , Working Conditions , Germany , Occupational Stress/epidemiology
8.
Proc Natl Acad Sci U S A ; 121(17): e2307213121, 2024 Apr 23.
Article in English | MEDLINE | ID: mdl-38621134

ABSTRACT

In the past three decades, there has been a rise in young academy movements in the Global North and South. Such movements, in at least Germany and the Netherlands, have been shown to be quite effective in connecting scientific work with society. Likewise, these movements share a common goal of developing interdisciplinary collaboration among young scientists, which contributes to the growth of a nation's-but also global-scientific endeavors. This paper focuses on the young academy movement in the fourth-largest country hosting the biggest Muslim population in the world, which is also the third-most populous democracy: Indonesia. We observe that there has been rising awareness among the young generation of scientists in Indonesia of the need to advocate for the use of sciences in responding to upcoming and current multidimensional crises. Science advocacy can be seen in their peer-based identification of Indonesia's future challenges, encompassing the fundamental areas for scientific inquiry, discovery, and intervention. We focus on the Indonesian Young Academy of Sciences (ALMI) and its network of young scientists. We describe ALMI's science communication practice, specifically SAINS45 and Science for Indonesia's Biodiversity, and how they have been useful for policymakers, media, and school engagements. The article closes with a reflection on future directions for the young academy movement in Indonesia and beyond.


Subject(s)
Islam , Indonesia , Germany , Netherlands
9.
Science ; 384(6691): 13-14, 2024 Apr 05.
Article in English | MEDLINE | ID: mdl-38574124

ABSTRACT

Complex tools from 300,000-year-old deposit at Schöningen in Germany point to a "wood age".


Subject(s)
Neanderthals , Wood , Animals , Archaeology , Germany
10.
Int Rev Psychiatry ; 36(1-2): 104-115, 2024.
Article in English | MEDLINE | ID: mdl-38557349

ABSTRACT

This article presents a psychobiography study of the famous Greek photographer Ellie Sougioultzoglou-Seraidari (Nelly's) (1899-1998). Nelly's was born in Aidini (Asia Minor), and in her early years she experienced the tragic events of the Greek-Turkish War (1919-1923). She studied photography in Dresden (Germany), and worked in Athens (Greece) and New York (U.S.A.). A narrative and cultural psychology framework was adopted to explore the way that Nelly's constructed her life story. An adjusted version of the 'Life-Story Interview' (McAdams & Bowman, 2001) was used that distinguishes between redemption versus contamination narratives. The narrative analysis of autobiographical materials revealed turning points and a central life theme. The photographer appeared to construct her life story as a process of reinventing herself or managing 'rebirth'. This construction matches the dominant narrative of Greek refugees of Asia Minor, following what is known in European history as the Asia Minor 'Great Catastrophe' (1922). Both narratives are surviving and thriving stories of 'rebirth'. The importance of making meaning of life stories within a specific socio-cultural and historical context is emphasised. Practice and research suggestions are provided.


Subject(s)
Narration , Female , Humans , Germany
11.
Int J Public Health ; 69: 1606770, 2024.
Article in English | MEDLINE | ID: mdl-38586472

ABSTRACT

Objective: To explore the role of reminders in recruiting and maintaining participation in an online panel. Methods: 50,045 individuals from five German federal states were invited by regular mail to participate in the online study "Health-Related Beliefs and Healthcare Experiences in Germany." Those who did not respond to the first attempt received a postal reminder. Comparisons of sociodemographic characteristics and responses were made between first-attempt respondents and those who enrolled after the second letter. Results: After the initial letter, 2,216 (4.4%, 95%CI: 4.3%-4.6%) registered for the study; after a reminder 1,130 (2.5%, 2.3%-2.6% of those reminded) enrolled. Minor sociodemographic differences were observed between the groups and the content of the responses did not differ. Second-attempt respondents were less likely to participate in subsequent questionnaires: 67.3% of first-attempt vs. 43.3% of second-attempt respondents participated in their fourth survey. Recruitment costs were 79% higher for second-attempt respondents. Conclusion: While reminders increased the number of participants, lower cost-effectiveness and higher attrition of second-attempt respondents support the use of single invitation only for studies with a similar design to ours when the overall participation is low.


Subject(s)
Surveys and Questionnaires , Humans , Germany
12.
BMC Psychol ; 12(1): 192, 2024 Apr 08.
Article in English | MEDLINE | ID: mdl-38589939

ABSTRACT

BACKGROUND: While adverse impacts of climate change on physical health are well-known, research on its effects on mental health is still scarce. Thus, it is unclear whether potential impacts have already reached treatment practice. Our study aimed to quantify psychotherapists' experiences with patients reporting climate change-related concerns and their views on dealing with this topic in psychotherapy. METHODS: In a nationwide online survey, responses were collected from 573 psychotherapists from Germany. Therapists reported on the presence of such patients, their socio-demographic characteristics, and climate change-related reactions. Psychotherapists' views on dealing with this topic in psychotherapy were also assessed. Descriptive statistics were used to analyse the responses. RESULTS: About 72% (410/573) of psychotherapists indicated having had patients expressing concerns about climate change during treatment. Out of these therapists, 41% (166/410) stated that at least one patient sought treatment deliberately because of such concerns. Patients were mainly young adults with higher education. Most frequent primary diagnoses were depression, adjustment disorder, and generalized anxiety disorder. Psychotherapists having encountered such patients differed from those without such encounters in their views on potential functional impairment and the necessity to target the concerns in treatment. Although 79% (326/415) of all respondents felt adequately prepared by their current therapeutic skills, 50% (209/414) reported a lack of information on how to deal with such concerns in therapy. CONCLUSIONS: Results indicate that psychotherapists are frequently confronted with climate change-related concerns and regard the mental health impact of climate change on their patients as meaningful to psychotherapeutic care. Regular care could be improved by a continuous refinement of the conceptualization and knowledge of the mental health influences of climate change. This would allow providing tailored methods of assessing and addressing climate change-related concerns in practice.


Subject(s)
Climate Change , Psychotherapy , Young Adult , Humans , Psychotherapy/methods , Surveys and Questionnaires , Germany
13.
Inquiry ; 61: 469580241242793, 2024.
Article in English | MEDLINE | ID: mdl-38591360

ABSTRACT

Relaxation-related workplace interventions can be counterstrategies for stress due to office work and should be evaluated for success after implementation. This study reports on the evaluation and comparison of 3 relaxation techniques introduced as an intervention for health promotion. A Germany-wide multicenter evaluation of a 3-month relaxation intervention (30 min, once a week) including autogenic training, progressive muscle relaxation (PMR) or Tai Chi was conducted at 14 locations as an intervention study. Anonymous questionnaires (n = 244) were used to collect self-reports of perceived stress after the courses. Status before the courses (pre) and after completion (post) were reported at the end of the intervention. All 3 relaxation techniques were able to improve the subjective perception of stress (SPS) score. Autogenic training was reported to improve all singular items with large effects, whereas only 2 items showed change for Tai Chi and none for PMR. Singular items were also influenced by the location of the courses. All 3 methods were able to improve stress-related outcomes in terms of stress perception and individual aspects of stress management. For this specific intervention autogenic training yielded the largest and wide-ranging effects. Tai Chi was also effective, but additional effect due to an associated increase in physical activity were not found. For a workplace intervention PMR could not be recommended based on this study. The literature on workplace interventions is surprisingly scarce, and future studies should focus on the selection of appropriate participants while avoiding potential bias.


Subject(s)
Exercise , Workplace , Humans , Germany
14.
Arch Osteoporos ; 19(1): 26, 2024 Apr 09.
Article in English | MEDLINE | ID: mdl-38592546

ABSTRACT

Osteoporosis is the most common bone disorder. Our data gives an estimate of around 5.87 million cases of osteoporosis in the general German population in 2018. Only 30% of insured individuals who suffered an osteoporotic fracture and/or had a confirmed diagnosis of osteoporosis, received an appropriate prescription. PURPOSE: Osteoporosis is the most common bone disorder. It particularly affects elderly people and increases the risk of atraumatic fractures. The aim of this study was to estimate the prevalence of osteoporosis in the general German population aged ≥ 50 years and to collect data on the frequency of prescription of osteoporosis-specific medication in order to assess the treatment gap. METHODS: Retrospective analysis of anonymized data of individuals aged ≥ 50 years insured under statutory healthcare schemes from the database of the Institute for Applied Health Research Berlin (InGef) for 2018 (study population). Insured individuals with osteoporosis were identified based on osteoporosis diagnoses, osteoporosis-specific prescriptions, or osteoporotic fractures. Thus, we estimated the prevalence of osteoporosis in the general German population aged ≥ 50 years. The prevalence of diagnoses, fractures, and prescriptions was determined for the study population and stratified by age and gender. RESULTS: Within the study population of 1,599,299 insured individuals, a prevalence of osteoporosis of 15.9% was determined. This estimated approximately 5.87 million cases of osteoporosis for the general German population. 81.6% of the cases were women. Osteoporosis-specific prescriptions were received by 30.0% of the insured individuals in the study population who had been diagnosed with osteoporosis and/or suffered an osteoporotic fracture. CONCLUSIONS: Germany has a high prevalence of osteoporosis. Only a small portion of individuals who may require osteoporosis-specific treatment actually receive it.


Subject(s)
Bone Diseases , Osteoporosis , Osteoporotic Fractures , Aged , Humans , Female , Male , Osteoporotic Fractures/epidemiology , Retrospective Studies , Osteoporosis/epidemiology , Germany/epidemiology
15.
PLoS One ; 19(4): e0296976, 2024.
Article in English | MEDLINE | ID: mdl-38635523

ABSTRACT

Vaccination willingness against COVID-19 is generally perceived as low. Moreover, there is large heterogeneity across and within countries. As a whole, Germany has average vaccination rates compared to other industrialized countries. However, vaccination rates in the 16 different German federal states differ by more than 20 percentage points. We describe variation in vaccination rates on the level of the 400 German counties using data on all vaccinations carried out until December 2022. Around 52-72% of that variation can be explained by regional differences in demographic characteristics, housing, education and political party preferences. We find indications that the remaining part may be due to differences in soft factors such as risk aversion, trust in the German government, trust in science, and beliefs in conspiracy theories regarding the origins of the Corona virus. We conclude that improving the trust in science and the fight against conspiracy theories may possibly be effective tools to improve vaccination rates and effectively fight pandemics.


Subject(s)
COVID-19 , Humans , COVID-19/epidemiology , COVID-19/prevention & control , Affect , Developed Countries , Germany/epidemiology , Trust , Vaccination
16.
PLoS One ; 19(4): e0301892, 2024.
Article in English | MEDLINE | ID: mdl-38635596

ABSTRACT

Clinical assessment of body condition is crucial in captive and free-ranging reptiles, since a large percentage of diseases result from inadequate nutrition. However, preventive health care is restricted by the lack of a practical method for the assessment in tortoises. Pre-existing evaluation systems based on weight and shell measurements are laborious and ignore the clinical presentation of the animal. The present study aimed to facilitate the assessment by establishing a body condition score. A total of 373 Hermann's Tortoises (Testudo hermanni) (n = 281 tortoises kept as pets in Germany and n = 92 tortoises originating from a free-ranging population (68) or a rearing station (24) in France) were examined and data (weight (g), carapace length (cm), width (cm), height (cm)) were recorded in a standard protocol between October 2020 and October 2021. A modified version of a body condition score for Mojave Desert Tortoises (Gopherus agassizii) (1 = cachectic, 3 = ideal, 5 = obese) was utilized and tested against pre-existing shell measurement systems (Jackson's ratio, body condition index, volume condition index, circumferential product). German captive tortoises were significantly heavier and larger than French specimens. In the Spearman's correlation matrix, the body condition score showed a statistically significant correlation with all measurement methods in the total population of captive tortoises (Testudo hermanni boettgeri), with a medium correlation strength, and a lack of correlation in free-ranging tortoises (Testudo hermanni hermanni). However, individual animal data suggested misleading results of mathematical equations in terms of body condition. Clinical evaluation of tortoises, including a body condition score, should be considered essential to provide good healthcare and should be an integral part of general examination.


Subject(s)
Turtles , Animals , France , Germany , Delivery of Health Care
18.
JMIR Public Health Surveill ; 10: e47241, 2024 Apr 04.
Article in English | MEDLINE | ID: mdl-38573762

ABSTRACT

BACKGROUND: The VACCELERATE Pan-European Scientific network aims to strengthen the foundation of vaccine trial research across Europe by following the principles of equity, inclusion, and diversity. The VACCELERATE Volunteer Registry network provides access to vaccine trial sites across the European region and supports a sustainable volunteer platform for identifying potential participants for forthcoming vaccine clinical research. OBJECTIVE: The aim of this study was to approach members of patient advocacy groups (PAGs) across Europe to assess their willingness to register for the VACCELERATE Volunteer Registry and their perspectives related to participating in vaccine trials. METHODS: In an effort to understand how to increase recruitment for the VACCELERATE Volunteer Registry, a standardized survey was developed in English and translated into 8 different languages (Dutch, English, French, German, Greek, Italian, Spanish, and Swedish) by the respective National Coordinator team. The online, anonymous survey was circulated, from March 2022 to May 2022, to PAGs across 10 European countries (Belgium, Cyprus, Denmark, France, Germany, Greece, Ireland, Italy, Spain, and Sweden) to share with their members. The questionnaire constituted of multiple choice and open-ended questions evaluating information regarding participants' perceptions on participating in vaccine trials and their willingness to become involved in the VACCELERATE Volunteer Registry. RESULTS: In total, 520 responses were collected and analyzed. The PAG members reported that the principal criteria influencing their decision to participate in clinical trials overall are (1) the risks involved, (2) the benefits that will be gained from their potential participation, and (3) the quality and quantity of information provided regarding the trial. The survey revealed that, out of the 520 respondents, 133 individuals across all age groups were "positive" toward registering in the VACCELERATE Volunteer Registry, with an additional 47 individuals reporting being "very positive." Respondents from Northern European countries were 1.725 (95% CI 1.206-2.468) times more likely to be willing to participate in the VACCELERATE Volunteer Registry than respondents from Southern European countries. CONCLUSIONS: Factors discouraging participants from joining vaccine trial registries or clinical trials primarily include concerns of the safety of novel vaccines and a lack of trust in those involved in vaccine development. These outcomes aid in identifying issues and setbacks in present registries, providing the VACCELERATE network with feedback on how to potentially increase participation and enrollment in trials across Europe. Development of European health communication strategies among diverse public communities, especially via PAGs, is the key for increasing patients' willingness to participate in clinical studies.


Subject(s)
Patient Advocacy , Vaccines , Humans , Europe , France , Germany , Clinical Trials as Topic
19.
Orphanet J Rare Dis ; 19(1): 161, 2024 Apr 13.
Article in English | MEDLINE | ID: mdl-38615062

ABSTRACT

BACKGROUND: Acid sphingomyelinase deficiency (ASMD) is a rare, progressive, potentially fatal lysosomal storage disease that exhibits a broad spectrum of clinical phenotypes. There is a need to expand the knowledge of disease mortality and morbidity in Germany because of limited information on survival analysis in patients with chronic ASMD (type B or type A/B). METHODS: This observational, multicentre, retrospective cohort study was conducted using medical records of patients with the first symptom onset/diagnosis of ASMD type B or type A/B between 1st January 1990 and 31st July 2021 from four German medical centres. Eligible medical records were abstracted to collect data on demographic characteristics, medical history, hospitalisation, mortality, and causes of death from disease onset to the last follow-up/death. Survival outcomes were estimated using the Kaplan-Meier analysis. Standardised mortality ratio (SMR) was also explored. RESULTS: This study included 33 chart records of patients with ASMD type B (n = 24) and type A/B (n = 9), with a median (interquartile range [IQR]) age of 8.0 [3.0-20.0] years and 1.0 [1.0-2.0] years, respectively, at diagnosis. The commonly reported manifestations were related to spleen (100.0%), liver (93.9%), and respiratory (77.4%) abnormalities. Nine deaths were reported at a median [IQR] age of 17.0 [5.0-25.0] years, with 66.7% of overall patients deceased at less than 18 years of age; the median [IQR] age at death for patients with ASMD type B (n = 4) and type A/B (n = 5) was 31.0 [11.0-55.0] and 9.0 [4.0-18.0] years, respectively. All deaths were ASMD-related and primarily caused by liver or respiratory failures or severe progressive neurodegeneration (two patients with ASMD type A/B). The median (95% confidence interval [CI]) overall survival age since birth was 45.4 (17.5-65.0) years. Additionally, an SMR [95% CI] analysis (21.6 [9.8-38.0]) showed that age-specific deaths in the ASMD population were 21.6 times more frequent than that in the general German population. CONCLUSIONS: This study highlights considerable morbidity and mortality associated with ASMD type B and type A/B in Germany. It further emphasises the importance of effective therapy for chronic ASMD to reduce disease complications.


Subject(s)
Niemann-Pick Disease, Type A , Niemann-Pick Diseases , Adolescent , Adult , Child , Humans , Middle Aged , Young Adult , Germany/epidemiology , Morbidity , Niemann-Pick Disease, Type A/epidemiology , Niemann-Pick Disease, Type A/genetics , Niemann-Pick Diseases/epidemiology , Niemann-Pick Diseases/genetics , Retrospective Studies
20.
Pediatr Rheumatol Online J ; 22(1): 44, 2024 Apr 18.
Article in English | MEDLINE | ID: mdl-38637849

ABSTRACT

OBJECTIVE: To investigate the psychosocial burden in children and adolescents with juvenile rheumatic diseases during the COVID-19 pandemic. METHODS: As part of the multicentre observational KICK-COVID study linked to the National Pediatric Rheumatology Database, adolescents < 21 years and parents of children < 12 years with rheumatic diseases answered questions on perceptions of health risk (PHR) due to SARS-CoV2, stress, well-being (WHO-5) and symptoms of depression (PHQ-9) and anxiety (GAD-7). Data were collected at routine visits from June to December 2021 and assessed for association with demographic and clinical parameters, treatment and patient-reported outcomes by multivariable regression analyses. RESULTS: Data from 1356 individuals (69% female, 50% adolescents) were included. Median PHR on a numeric rating scale (NRS, 0-10) was 4 (IQR 2-6), median perceived stress was 3 (IQR 1-6). Adolescents reported a worse well-being with a significantly lower median WHO-5-score (60, IQR 40-76) than parents reported for their children < 12 years (80, IQR 68-84). Moderate to severe symptoms of depression and anxiety were reported by 14.3% and 12.3% of the adolescents, respectively. PHR was significantly higher in patients with systemic lupus erythematosus, methotrexate or biologic disease-modifying anti-rheumatic drug therapy than in patients without these characteristics, whereas lower WHO-5 or higher PHQ-9 or GAD-7 scores were only associated with poorer patient-reported health status and physical functioning. CONCLUSION: The perception of health risk due to SARS-CoV2 infection was not paralleled by an impairment of mental health, which were, however, significantly correlated with self-rated health status and functional capacity, highlighting the importance of patient-reported outcome assessment. TRIAL REGISTRATION: German Clinical Trials Register (DRKS), no. DRKS00027974. Registered on 27th of January 2022.


Subject(s)
COVID-19 , Rheumatic Diseases , Child , Humans , Adolescent , Female , Male , Depression/epidemiology , Depression/etiology , Pandemics , Prospective Studies , RNA, Viral , COVID-19/epidemiology , SARS-CoV-2 , Anxiety/epidemiology , Anxiety/etiology , Rheumatic Diseases/drug therapy , Rheumatic Diseases/epidemiology , Germany/epidemiology , Perception
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