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Table 2 Musculoskeletal abnormalities detected by pGALS (number of abnormal observations to total observations)

From: Assessment of musculoskeletal abnormalities in children with mucopolysaccharidoses using pGALS

Manoeuvre tested pGALS instruction All MPS (n = 18 patients) 1203 total observations MPS I (n = 13) MPS II (n = 4) Mannosidosis (n = 1)
Shoulder abduction, external rotation Hands behind neck 49/54 (91%) 35/39 (90%) 12/12 (100%) 2/3 (67%)
Shoulder flexion Reach arms up 48/54 (89%) 36/39 (93%) 12/12 (100%) 0/3 (0%)
Wrist flexion Hands together back to back 41/54 (76%) 27/39 (69%) 12/12 (100%) 2/3 (67%)
Elbow extension Reach up 39/53 (74%) 26/39 (90%) 12/12 (100%) 1/2 (50%)
TMJ excursion Open mouth wide and try to put 3 fingers inside 39/54 (72%) 28/39 (72%) 10/12 (83%) 1/3 (33%)
MCP, DIP, PIP extension Hands and wrists together 37/54 (69%) 24/39 (62%) 11/12 (92%) 2/3 (67%)
Forward flexion of spine Bend forwards. Observe curvature of spine from all sides 31/50 (62%) 21/35 (60%) 8/12 (67%) 2/3 (67%)
Spinal deformity Observe patient standing and then bending forwards 28/45 (62%) 22/34 (65%) 5/9 (56%) 1/2 (50%)
Gait Observe patient walking 28/45 (62%) 20/32 (63%) 7/10 (70%) 1/3 (33%)
Wrist extension Hands palm to palm 33/53 (60%) 22/39 (56%) 11/11 (100%) 0/3 (0%)
MCP/DIP/PIP extension Hands out in front 29/52 (56%) 17/37 (46%) 12/12 (100%) 0/3 (0%)
Cervical spine lateral flexion Touch ear to shoulder 29/53 (55%) 17/38 (45%) 12/12 (100%) 0/3 (0%)
Elbow extension Hands out in front 22/40 (55%) 12/27 (44%) 9/11 (82%) 1/2 (50%)
Ankle dorsiflexion Walk on heels 24/45 (53%) 15/33 (45%) 7/9 (78%) 2/3 (67%)
Neck extension Look up to the ceiling 28/53 (53%) 19/38 (50%) 9/12 (75%) 0/3 (0%)
Forearm supination Turn hand over 20/42 (48%) 10/30 (33%) 9/11 (82%) 1/1 (100%)
Opposition of thumb and 3 rd -5 th fingers Touch tips of fingers with thumb 23/54 (43%) 14/39 (36%) 8/12 (67%) 1/3 (33%)
MCP/DIP/PIP flexion Make a fist 20/54 (37%) 10/39 (26%) 10/12 (83%) 0/3 (0%)
Elbow flexion Put hands behind neck 12/42 (29%) 6/27 (33%) 5/12 (42%) 1/3 (33%)
Knee extension Bring ankle up to bottom 12/44 (27%) 7/29 (24%) 4/12 (33%) 1/3 (33%)
Knee flexion 11/45 (24%) 4/30 (13%) 7/12 (58%) 0/3 (0%)
Opposition of thumb and index finger Touch tip of finger with thumb 10/54 (19%) 5/39 (13%) 5/12 (42%) 0/3 (0%)
Elbow flexion Put hands and wrists together 7/53 (13%) 5/39 (13%) 2/11 (18%) 0/3 (0%)
Ankle plantar flexion Walk on tip-toes 4/45 (9%) 2/33 (6%) 2/9 (22%) 0/3 (0%)
  1. Key: DIP = distal interphalangeal; MCP = metacarpophalangeal; PIP = proximal interphalangeal; pGALS = paediatric Gait, Arms, Legs and Spine; TMJ = temporomandibular joint.