Validation of the Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP INTEND)
infant; Pediatrics; Rehabilitation; validity; outcome assessment; child; female; male; disease severity; (health care); atrophies of childhood/diagnosis; child development/physiology; childhood/physiopathology; disability evaluation; humans; motor skills/physiology; natural-history; physical therapy/methods; preschool; psychometrics/methods; severity of illness index; sma; smn2 copy number; spinal muscular; spinal muscular atrophies of; spinal muscular-atrophy; tone and motor problems; SMA1; tool development; scale development; CHOP INTEND
Purpose: Preliminary validation of the Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP INTEND) for motor skill assessment in spinal muscular atrophy type I. Methods: A total of 27 subjects 3 to 260 months old (mean = 49, SD = 69) with spinal muscular atrophy-I were evaluated with the CHOP INTEND. Subjects were evaluated as part of a multicenter natural history study. Results: CHOP INTEND scores and age were significantly correlated (r = -0.51, P = .007; 2 survival of the motor neuron [SMN] 2 gene copies, n = 16, r = -0.60, 3 SMN2 gene copies, n = 9, r = -0.83). Respiratory support and CHOP INTEND scores were correlated (r = -0.74, P <.0001, n = 26). The CHOP INTEND and age regression in patients with 2 copies versus 3 copies of SMN2 approached significance (P = .0711, n = 25). Subjects who required respiratory support scored significantly lower (mean = 15.5, SD = 10.2 vs mean = 31.2, SD = 4.2, P <.0001, n = 27). Correlation with motor unit number estimation and combined motor unit activation were not significant. Conclusion: The CHOP INTEND reflects measures of disease severity and supports continued exploration of the CHOP INTEND. (Pediatr Phys Ther 2011; 23: 322-326)
Glanzman A M; McDermott M P; Montes J; Martens W B; Flickinger J; Riley S; Quigley J; Dunaway S; O'Hagen J; Deng L Y; Chung W K; Tawil R; Darras B T; Yang M; Sproule D; De Vivo D C; Kaufmann P; Finkel R S; Pediatric Neuromuscular Clinical Research; Muscle Study Group
Pediatric Physical Therapy
2011
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1097/PEP.0b013e3182351f04" target="_blank" rel="noreferrer noopener">10.1097/PEP.0b013e3182351f04</a>
Gastrostomy tube feeding in children with cerebral palsy: a prospective, longitudinal study
Child; Female; Humans; infant; Male; Treatment Outcome; Longitudinal Studies; Time Factors; Demography; adolescent; Preschool; infant; Newborn; retrospective studies; Enteral Nutrition/methods; Anthropometry/methods; Body Height/physiology; Cerebral Palsy/classification/nursing/surgery; Child Development/physiology; Gastrostomy/methods; Head/growth & development; Nutritional Status/physiology; Weight Gain/physiology
We report a longitudinal, prospective, multicentre cohort study designed to measure the outcomes of gastrostomy tube feeding in children with cerebral palsy (CP). Fifty-seven children with CP (28 females, 29 males; median age 4y 4mo, range 5mo to 17y 3mo) were assessed before gastrostomy placement, and at 6 and 12 months afterwards. Three-quarters of the children enrolled (43 of 57) had spastic quadriplegia; other diagnoses included mixed CP (6 of 57), hemiplegia (3 of 57), undiagnosed severe neurological impairment (3 of 57), ataxia (1 of 57), and extrapyramidal disorder (1 of 57). Only 7 of 57 (12%) could sit independently, and only 3 of 57 (5%) could walk unaided. Outcome measures included growth/anthropometry, nutritional intake, general health, and complications of gastrostomy feeding. At baseline, half of the children were more than 38D below the average weight for their age and sex when compared with the standards for typically-developing children. Weight increased substantially over the study period; the median weight z score increased from -3 before gastrostomy placement to -2.2 at 6 months and -1.6 at 12 months. Almost all parents reported a significant improvement in their child's health after this intervention and a significant reduction in time spent feeding. Statistically significant and clinically important increases in weight gain and subcutaneous fat deposition were noted. Serious complications were rare, with no evidence of an increase in respiratory complications.
2005
Sullivan P; Juszczak E; Bachlet AM; Lambert B; Vernon-Roberts A; Grant HW; Eltumi M; McLean L; Alder N; Thomas AG
Developmental Medicine And Child Neurology
2005
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
Journal Article
<a href="http://doi.org/10.1111/j.1469-8749.2005.tb01095.x" target="_blank" rel="noreferrer">10.1111/j.1469-8749.2005.tb01095.x</a>