Table 2 Regional relative frequencies and clinical data of LGMD subtypes in mainland China and Taiwan
Item | Our study | Meng Yu et al | Qi Zhang et al | Liang Wang et al | Wen-Chen et al |
|---|---|---|---|---|---|
Geographical distribution | Southeast China | North China | Northeast China | South China | Taiwanese |
Confirmed LGMD Family | 41 | 95 | 26 | 24 | 26 |
Number of cases | 50 | 105 | 26 | 30 | 40 |
Males: Females | 27:23 | 58:47 | 20:6 | 12:18 | 21:19 |
Age at onset (years) | 19.7 ± 9.3 | 19.1 ± 11.5 | 31.6 ± 14.0 | 12.0 ± 7.9 | 15.8 ± 11.8 |
Median age of onset years (range) | 19 (4–48) | 17 (2–47) | 28.5 (15–71) | 13 (1.5–29) | 14 (2–50) |
Cardiac dysfunction | 22.0% (9/41) | – | – | 33.3% (3/9) | 27.0% (10/37) |
Restrictive pulmonary defect | 15.4% (4/26) | – | – | – | 47.4% (18/38) |
Most Common subtype | Dysferlinopathy | Dysferlinopathy | Calpainopathy | Dysferlinopathy Sarcoglycanopathies | Dysferlinopathy Dystroglycanopathies |
 Calpainopathy | 29.30% | 24.80% | 46.20% | 16.70% | 11.50% |
 Dysferlinopathy | 36.60% | 49.50% | 38.40% | 29.20% | 23.10% |
 Sarcoglycanopathies | Not found | 9.50% | 7.70% | 29.20% | 15.40% |
 LGMD-R7/LGMD2G | 14.60% | Not found | Not found | Not found | 11.50% |
 Dystroglycanopathies | 12.20% | 3.80% | Not found | 8.30% | 23.10% |
 LGMD-R9/LGMD2I | 4.90% | 2.90% | Not found | 8.30% | 19.20% |