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Table 5 Musculoskeletal abnormalities detected by pGALS in MPS type II

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

Manoeuvre tested pGALS instruction MPS II (n = 4)
Shoulder flexion Reach arms up 12/12 (100%)
Shoulder abduction, external rotation Hands behind neck 12/12 (100%)
Elbow extension Reach up 12/12 (100%)
Wrist flexion Hands together back to back 12/12 (100%)
Wrist extension Hands palm to palm 11/11 (100%)
MCP/DIP/PIP extension Hands out in front 12/12 (100%)
Cervical spine lateral flexion Touch ear to shoulder 12/12 (100%)
MCP, DIP, PIP extension Hands and wrists together 11/12 (92%)
TMJ excursion Open mouth wide and try to put 3 fingers inside 10/12 (83%)
MCP/DIP/PIP flexion Make a fist 10/12 (83%)
Elbow extension Hands out in front 9/11 (82%)
Forearm supination Turn hand over 9/11 (82%)
Ankle dorsiflexion Walk on heels 7/9 (78%)
Neck extension Look up to the ceiling 9/12 (75%)
Gait Observe patient walking 7/10 (70%)
Forward flexion of spine Bend forwards. Observe curvature of spine from all sides. 8/12 (67%)
Opposition of thumb and 3 rd -5 th fingers Touch tips of fingers with thumb 8/12 (67%)
Spinal deformity   5/9 (56%)
Opposition of thumb and index finger Touch tip of finger with thumb 5/12 (42%)
Elbow flexion Put hands behind neck 5/12 (42%)
Knee extension Bring ankle up to bottom 4/12 (33%)
Knee flexion 7/12 (58%)
Ankle plantar flexion Walk on tip-toes 2/9 (22%)
Elbow flexion Put hands and wrists together 2/11 (18%)
  1. Figure Key: ANA = antinuclear antibody; ESR = erythrocyte sedimentation rate; IDUA = iduronidase, alpha-L; JIA = juvenile idiopathic arthritis; NSAIDs = non-steroidal anti-inflammatory drugs; pGALS = paediatric Gait, Arms, Legs and Spine; RA = rheumatoid arthritis; uGAG = urinary glycosaminoglycans.