Schiltz, Daniel ORCID: 0000-0003-1096-9336, Mueller, Karolina, Ortner, Christine, Tschernitz, Sebastian, Anker, Alexandra, Felthaus, Oliver, Schreml, Julia, Koller, Michael, Prantl, Lukas ORCID: 0000-0003-2454-2499 and Schreml, Stephan ORCID: 0000-0002-2820-1942 (2021). Multiple Symmetric Lipomatosis: A Cross-Sectional Study to Investigate Clinical Features and Patients' Quality of Life. Symmetry-Basel, 13 (10). BASEL: MDPI. ISSN 2073-8994

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Abstract

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.</p>

Item Type: Journal Article
Creators:
CreatorsEmailORCIDORCID Put Code
Schiltz, DanielUNSPECIFIEDorcid.org/0000-0003-1096-9336UNSPECIFIED
Mueller, KarolinaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Ortner, ChristineUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Tschernitz, SebastianUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Anker, AlexandraUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Felthaus, OliverUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Schreml, JuliaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Koller, MichaelUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Prantl, LukasUNSPECIFIEDorcid.org/0000-0003-2454-2499UNSPECIFIED
Schreml, StephanUNSPECIFIEDorcid.org/0000-0002-2820-1942UNSPECIFIED
URN: urn:nbn:de:hbz:38-572521
DOI: 10.3390/sym13101823
Journal or Publication Title: Symmetry-Basel
Volume: 13
Number: 10
Date: 2021
Publisher: MDPI
Place of Publication: BASEL
ISSN: 2073-8994
Language: English
Faculty: Unspecified
Divisions: Unspecified
Subjects: no entry
Uncontrolled Keywords:
KeywordsLanguage
LAUNOIS-BENSAUDE; STEM-CELLS; EXPRESSION; DEFECTMultiple languages
Multidisciplinary SciencesMultiple languages
URI: http://kups.ub.uni-koeln.de/id/eprint/57252

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