SynthesisAging2026
Life expectancy and causes of death in classical laminopathic progeroid syndromes: systematic review with individual-patient data synthesis.
Synthesis in Aging, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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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.
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Authors and funding
9 authors.
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Abstract
Classical laminopathic progeroid syndromes link nuclear-envelope defects to accelerated aging, but the specific prognosis for each subtype remains unclear. We conducted a PRISMA-guided systematic review and individual-patient data (IPD) synthesis (PROSPERO CRD420251080312; PubMed/Scopus to 16 July 2025). Genetically confirmed IPD constituted the primary cohort; causes of death were harmonized a priori and survival assessed by Kaplan-Meier with log-rank tests. We included 169 studies and two institutional cases, previously illustrated in the literature but not comprehensively characterized, yielding 158 genetically confirmed IPD (61 deaths, 97 censored). Median survival (years, 95% CI) was: HGPS 16.0 (13.42-19.0; n=60), MAD-B 37.0 (24-44; n=21), RD-LMNA 0.92 (0.096-2.50; n=8), and RD-ZMPSTE24 0.03 (0.014-0.047; n=38); MAD-A had no deaths (n=31). Cause-of-death profiles were subtype-specific: respiratory failure predominated in RD (36/45 deaths), cardiovascular causes in HGPS (6/11), and renal complications in MAD-B (4/5). Findings were robust in sensitivity analyses (including clinical cases, risk-of-bias exclusions, and center-specific checks). In sum, survival and mortality patterns differ markedly across classical laminopathic progeria; genetically confirmed IPD resolved by subtype provides more reliable estimates for clinical counseling and trial design in ultra-rare progeroid disorders.
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