أعرض في EDS

Multiple Symmetric Lipomatosis: A Cross-Sectional Study to Investigate Clinical Features and Patients’ Quality of Life

التفاصيل البيبلوغرافية
العنوان: Multiple Symmetric Lipomatosis: A Cross-Sectional Study to Investigate Clinical Features and Patients’ Quality of Life
المؤلفون: Schiltz, Daniel, Mueller, Karolina, Ortner, Christine, Tschernitz, Sebastian, Anker, Alexandra, Felthaus, Oliver, Schreml, Julia, Koller, Michael, Prantl, Lukas, Schreml, Stephan
المصدر: Symmetry
Volume 13
Issue 10
Symmetry, Vol 13, Iss 1823, p 1823 (2021)
بيانات النشر: Multidisciplinary Digital Publishing Institute, 2021.
سنة النشر: 2021
مصطلحات موضوعية: multiple symmetric lipomatosis (MSL), Morbus Madelung, Launois Bençaude, benign symmetric lipomatosis (BSL), quality of life in MSL, ddc:610, endocrine system, fungi, QA1-939, 610 Medizin, Mathematics
الوصف: Within the subcutaneous adipose tissue diseases, multiple symmetric lipomatosis (MSL) (syn.: Launois Bensaude Syndrome, Morbus Madelung, benign symmetric lipomatosis) is rare. The pathogenesis of MSL remains unclear. We investigated the largest German cohort of MSL patients to obtain anamnestic data and quality of life with a standard questionnaire. Twenty-nine patients with confirmed MSL were included and filled in a questionnaire designed for this study. The questionnaire assessed common anamnestic factors, such as quality of life (EQ-5D-3L) and subjective treatment goals and success (“Patient-Benefit-Index-Lymphedema”, PBI-L). The gender distribution of the patients involved in the study was m/f: 1/4 (male: n = 6 (21%)
female n = 23 (79%)). While the exact pathophysiology of MSL remains unclear, a subset of patients’ positive family history suggests a strong genetic factor, sometimes compatible with autosomal dominant inheritance. Patients with MSL showed lower health states (EQ VAS Score: m = 51, sd = 24, range = 0–90) than the German norm population (m = 77). Around two thirds (68%) of patients reported relevant benefits of therapy (liposuction/lipectomy). In our cohort about one third of the patients reported a positive family history for MSL-like features. Additionally, at least in some patients, a strong genetic factor, compatible with autosomal dominant inheritance, seems a possible major driver of MSL development. Alcohol consumption and MSL development has to be regarded as a controversial issue. Patients suffering from MSL have a clear decrease in quality of life and a marked wish for treatment.
وصف الملف: application/pdf
اللغة: English
تدمد: 2073-8994
DOI: 10.3390/sym13101823
URL الوصول: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::3142c11b3ebffd8a81b0f5cd79432254
حقوق: OPEN
رقم الأكسشن: edsair.doi.dedup.....3142c11b3ebffd8a81b0f5cd79432254
قاعدة البيانات: OpenAIRE
الوصف
تدمد:20738994
DOI:10.3390/sym13101823