Validation of the Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP INTEND)

被引：117

作者：

Glanzman, Allan M. ^{[1
]}

McDermott, Michael P. ^{[2
]}

Montes, Jacqueline ^{[3
]}

Martens, William B. ^{[2
]}

Flickinger, Jean

Riley, Susan ^{[4
]}

Quigley, Janet ^{[4
]}

Dunaway, Sally ^{[3
]}

O'Hagen, Jessica ^{[3
]}

Deng, Liyong ^{[3
]}

Chung, Wendy K. ^{[3
]}

Tawil, Rabi ^{[2
]}

Darras, Basil T. ^{[4
,5
]}

Yang, Michele ^{[6
,7
]}

Sproule, Douglas ^{[3
]}

De Vivo, Darryl C. ^{[3
]}

Kaufmann, Petra ^{[3
]}

Finkel, Richard S. ^{[8
]}

机构：

[1] Childrens Hosp Philadelphia, Dept Phys Therapy, Philadelphia, PA 19104 USA

[2] Univ Rochester, Med Ctr, Rochester, NY 14642 USA

[3] Columbia Univ, New York, NY USA

[4] Childrens Hosp, Boston, MA 02115 USA

[5] Harvard Univ, Sch Med, Cambridge, MA 02138 USA

[6] Univ Colorado, Denver, CO 80202 USA

[7] Childrens Hosp, Denver, CO 80218 USA

[8] Univ Penn, Perelman Sch Med, Philadelphia, PA 19104 USA

来源：

PEDIATRIC PHYSICAL THERAPY | 2011年 / 23卷 / 04期

关键词：

child development/physiology; child; disability evaluation; female; humans; infant; male; motor skills/physiology; outcome assessment (health care); preschool; physical therapy/methods; psychometrics/methods; severity of illness index; spinal muscular atrophies of childhood/diagnosis; spinal muscular atrophies of childhood/physiopathology; validity; SPINAL MUSCULAR-ATROPHY; SMN2 COPY NUMBER; DISEASE SEVERITY; NATURAL-HISTORY; SMA;

D O I：

10.1097/PEP.0b013e3182351f04

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

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)

引用

页码：322 / 326

页数：5

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