Evidence mapPaperPMID 40671313Full record

ArticleClinical endocrinology2025

Potential Impact of Parental Origin of Inheritance on the Clinical Presentation of Familial Partial Lipodystrophy Type 2 Syndrome.

Donatella Gilio, Ozge Besci, Natália Rossin Guidorizzi, Merve Celik Guler, Ilgin Yildirim Simsir, Caterina Pelosini, Giovanni Ceccarini, Korcan Demir, Baris Akinci, Ferruccio Santini and 3 more

Abstract read
In one paragraph

Article in Clinical endocrinology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.

0numbers the graph read from it
0cells of the map it votes in
1citing papers in PubMed
field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

1 citing paper in PubMed.

  1. Article
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

13 authors.

Donatella GilioEndocrinology and Diabetes Division, Department of Internal Medicine, Caswell Diabetes Institute and Metabolism, University of Michigan Medical School, Ann Arbor, Michigan, USA.
Ozge BesciEndocrinology and Diabetes Division, Department of Internal Medicine, Caswell Diabetes Institute and Metabolism, University of Michigan Medical School, Ann Arbor, Michigan, USA.
Natália Rossin GuidorizziDivision of Internal Medicine, University of São Paulo, Ribeirão Preto, Brazil.
Merve Celik GulerEndocrinology and Diabetes Division, Department of Internal Medicine, Caswell Diabetes Institute and Metabolism, University of Michigan Medical School, Ann Arbor, Michigan, USA.ORCID https://orcid.org/0000-0003-1112-7640
Ilgin Yildirim SimsirDivision of Endocrinology and Metabolism, Department of Internal Medicine, Ege University, Izmir, Türkiye.
Caterina PelosiniEndocrinology Unit, Obesity and Lipodystrophy Center, University Hospital of Pisa, Pisa, Italy.
Giovanni CeccariniEndocrinology Unit, Obesity and Lipodystrophy Center, University Hospital of Pisa, Pisa, Italy.
Korcan DemirDivision of Pediatric Endocrinology, Dokuz Eylul University, Izmir, Türkiye.
Baris AkinciTechnological Research Program, Izmir Biomedicine and Genome Center & DEPARK, Dokuz Eylul University Health Campus, Izmir, Türkiye.ORCID https://orcid.org/0000-0002-8634-4845
Ferruccio SantiniEndocrinology Unit, Obesity and Lipodystrophy Center, University Hospital of Pisa, Pisa, Italy.
ECLip (European Consortium of Lipodystrophy)
Maria Cristina Foss-FreitasEndocrinology and Diabetes Division, Department of Internal Medicine, Caswell Diabetes Institute and Metabolism, University of Michigan Medical School, Ann Arbor, Michigan, USA.
Elif A OralEndocrinology and Diabetes Division, Department of Internal Medicine, Caswell Diabetes Institute and Metabolism, University of Michigan Medical School, Ann Arbor, Michigan, USA.ORCID https://orcid.org/0000-0002-9171-1144

Funding

Mechanisms of adipocyte loss in laminopathy-induced lipodystrophy in mice and humansR01DK125513 · UNIVERSITY OF MICHIGAN AT ANN ARBOR · 2025 to 2025
$659k
National Institute of Diabetes and Digestive and Kidney Diseases (Grant 2R01DK125513), and Dokuz Eylul University-Research Fund (Project Number TUI-2023-3126).NIDDK NIH HHS R01 DK125513
6 · The paper itself

Abstract

contextFamilial partial lipodystrophy type 2 (FPLD2) is a rare autosomal dominant disorder caused by pathogenic variants in the LMNA gene. The influence of parental inheritance on clinical presentation has not been fully explored.

objectiveTo investigate the influence of maternal versus paternal inheritance of LMNA variants on the clinical and metabolic phenotype of patients with FPLD2. DESIGN, PATIENTS, MEASUREMENTS: This retrospective cohort study included 83 patients with FPLD2 from four different centres. Clinical, biochemical, and body composition data were analysed. Patients were grouped based on maternal (maternal inheritance group; n = 49) or paternal (paternal inheritance group; n = 34) inheritance of LMNA variants. Statistical comparisons were made between the groups.

resultsPatients in the maternal inheritance group had a younger current age (35 (33) vs. 48 (22) years, p = 0.042) and earlier diagnosis of lipodystrophy (22 (30) vs. 36 (25) years, p = 0.044) compared to those in the paternal inheritance group. Body fat percentages in the arms (23.8 (6.5) % vs. 21.0 (6.2) %, p = 0.034) and trunk (32.1 (10.3) % vs. 28.5 (6.1) %, p = 0.024) were higher in maternal inheritance group. Fatty liver disease (79% vs. 57%, p = 0.029) and pancreatitis (26% vs. 8%, p = 0.033) were more prevalent in paternal inheritance group.

conclusionParental lineage may influence the phenotype of FPLD2: patients with a maternally inherited LMNA variant tend to preserve more adipose tissue in the upper body, while those with a paternally inherited variant experience greater adipose tissue loss in that region, often associated with more severe metabolic complications. These findings highlight the importance of contemplating parental lineage as a relevant factor when evaluating the clinical presentation and management of patients with FPLD2.

Indexed as

Lamin Type ALipodystrophy, Familial PartialMaternal InheritancePaternal InheritanceAdultBody CompositionFemaleHumansMaleMiddle AgedPhenotypeRetrospective StudiesYoung AdultLamin Type ALMNA protein, humanbody compositiondual X‐ray absorptiometryfat masslean massLMNAparent‐of‐origin effectpartial lipodystrophy

Identifiers

PMID40671313
PMCPMC12413679

What Socratic holds

Textmetadata
LicenceCC BY-NC-ND
Read underepoch 390

Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the Socratic graph.