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August 8, 2026Human GeneticsOpen Access

Analysis of the Russian FSHD registry (n=491) revealed that 76% of patients had genetic confirmation, with shoulder girdle weakness being the most common onset manifestation (45.2%).

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Population

491 participants with Facioscapulohumeral muscular dystrophy in the Russian FSHD Patient Registry, mean age…

Design

Cohort

Key result

Analysis of the Russian FSHD registry (n=491) revealed that 76% of patients had genetic confirmation, with shoulder girdle weakness being the most common onset manifestation (45.2%).

Authors

AKAnna KuchinaDSDarya SherstyukovaABArtem Borovikov

Discussion

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Overview

Registry data may aid FSHD stratification by onset; leaves open prospective validation before guiding care.

Study Design

Type

Cohort (n=491)

Structured PICO

P
Population
491 patients with facioscapulohumeral muscular dystrophy in the Russian national registry, 76% of whom had genetic confirmation.
O
Outcome
Clinical characteristics, genetic confirmation rates, D4Z4 repeat unit distribution, and disease progression trajectories

The Russian FSHD registry provides comprehensive characterization of a large national cohort, identifying distinct disease progression trajectories useful for patient stratification.

Cite This Study

Kuchina et al. (2026) conducted a cohort in Facioscapulohumeral muscular dystrophy (FSHD) (n=491). Facioscapulohumeral muscular dystrophy (FSHD) was evaluated on Clinical characteristics and disease progression trajectories. Analysis of the Russian FSHD registry (n=491) revealed that 76% of patients had genetic confirmation, with shoulder girdle weakness being the most common onset manifestation (45.2%).

synapsesocial.com/papers/6ab09eee53e47274bbe78db1https://doi.org/10.1007/s00439-026-02865-y
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