3 Diseases included under the pediatric neuromuscular umbrella Cerebral palsyCongenital muscular dystrophyCongenital myopathyDuchenne muscular dystrophySpina bifidaSpinal muscular atrophy
4 Why obtain accurate height measurements in pediatric neuromuscular disease? To trend growthTo trend and calculate Body Mass Index (BMI)To trend and calculate conversion from Forced Vital Capacity (FVC) to % predicted Forced Vital Capacity (FVC)While FVC provides important data, % predicted FVC may demonstrate earlier declines in respiratory capacity.
5 Ulna LengthMeasuring height in a uniform manner across all children with pediatric neuromuscular disease allows for:standard data collectionaggregate data and meta-analysisability to reach statistically significant results in rare disease analysis
6 Ulna length serves as a mathematical proxy for height. Why ulna length?In children with neuromuscular disease, it can be difficult to obtain an accurate height measurement due to:ContracturesScoliosisWeaknessUlna length serves as a mathematical proxy for height.
7 Measuring Ulna Length Upper Extremity Support and position the arm in pronationwith 90 – 110 degrees of elbow flexion.Palpate the distal tip of the ulnar styloid processPalpate superiorly on the wrist to avoid mistaking the tendon of the extensor carpi ulnaris for the distal tip of the ulna.Palpate the tip of the olecranon and place one arm of the segmometer on the olecranon.Place the other end of the segmometerat the tip of the ulnar styloid.Measure in cm, to the nearestmillimeter (e.g. 19.7cm)
8 Where can I find references to calculate height and % predicted FVC based on ulna length? Gauld LM, Kappers J, Carlin JB, Robertson CF. Height Prediction from ulna length. Dev Med Child Neurol Jul: 46 (7):Gauld LM, Kappers J, Carlin JB, Robertson CF. Prediction of childhood pulmonary function using ulna length. Am J Respir Crit Care Med Oct 1:168 (7): Epub 2003 Jul 17
9 What device is needed to measure ulna length? Segmometer