Spinal Cord Injury Model Systems: Review of Program and National Database From 1970 to 2015.

The Spinal Cord Injury Model Systems (SCIMS) centers have provided continuous, comprehensive multidisciplinary care for persons with spinal cord injury (SCI) in the United States since their inception in 1970. In addition, the research conducted and the analysis of data collected at these centers facilitate advances in the care and the overall quality of life for people with SCI. Over the past 45 years, the SCIMS program and National Spinal Cord Injury Database (NSCID) have undergone major revisions, which must be recognized in the planning, conduct, and interpretation of SCIMS research to prevent misinterpretation of findings.

The National Institute on Disability and Rehabilitation Research burn model system database: a tool for the multicenter study of the outcome of burn injury.

Advances in critical care and surgical management have significantly improved survival after burn injury over the past several decades. However, today, survival alone is an insufficient outcome. In 1994, the National Institute on Disability and Rehabilitation Research (NIDRR) created a burn model system program to evaluate the long-term sequelae of burn injuries.

Mutations in the Wolfram syndrome type 1 gene (WFS1) define a clinical entity of dominant low-frequency sensorineural hearing loss.

Objective: To describe low-frequency sensorineural hearing loss (LFSNHL) inherited as a dominant trait in 3 families and in 1 sporadic case.

Design: Longitudinal clinical study from 1968 to 2001.

Setting: Tertiary care hospital; field studies conducted by molecular genetic research laboratory.

Mutation of a transcription factor, TFCP2L3, causes progressive autosomal dominant hearing loss, DFNA28.

We ascertained a large American family with an autosomal dominant form of progressive non-syndromic sensorineural hearing loss. After excluding linkage to known deafness loci, we performed a genome-wide scan and found linkage to marker GAAT1A4 on chromosome 8q22 (LOD=5.12 at theta=0), and this locus was designated DFNA28.