The Texas Rett Syndrome Registry maintains the largest population-based registry of cases and potential cases of Rett syndrome in the world. The most precise estimate of the prevalence of Rett syndrome of 1 per 22800 (0.44/10000) females aged 2 through 18 years of age was generated from this Registry. In addition, the first prevalence figures for black and Hispanic female cases were estimated. Registry cases are actively ascertamed from multiple sources. Registry staff identify presumptive cases from review of information provided to the Registry by the parent or guardian. Preliminary diagnostic evaluation includes standardized review of medical records and videotape of key behaviors. Diagnosis is confirmed at clinical evaluation. The active surveillance system is monitored with the two-source capture-recapture methodology and case ascertainment is projected. The 1990 prevalence estimate of Rett syndrome indicates that the syndrome occurs less frequently than previously estimated. Until a biologic marker for Rett syndrome is identified or a standard definition for an incident case of Rett syndrome is designated, the prevalence of Rett syndrome will remain a major investigative issue of its epidemiology, and the Registry will be an important, systematic mean to gather case material for clinical and laboratory studies providing the foundation for the development of preventive interventions.
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February 1993
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February 01 1993
Epidemiology of Rett Syndrome: A Population-Based Registry
Claudia A. Kozinetz;
Claudia A. Kozinetz
From The Rett Center, Department of Pediatrics, Baylor College of Medicine, Houston, TX
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Martha L. Skender;
Martha L. Skender
From The Rett Center, Department of Pediatrics, Baylor College of Medicine, Houston, TX
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Nancy MacNaughton;
Nancy MacNaughton
From The Rett Center, Department of Pediatrics, Baylor College of Medicine, Houston, TX
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Mary Jane Almes;
Mary Jane Almes
From The Rett Center, Department of Pediatrics, Baylor College of Medicine, Houston, TX
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Rebecca J. Schultz;
Rebecca J. Schultz
From The Rett Center, Department of Pediatrics, Baylor College of Medicine, Houston, TX
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Alan K. Percy;
Alan K. Percy
From The Rett Center, Department of Pediatrics, Baylor College of Medicine, Houston, TX
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Daniel G. Glaze
Daniel G. Glaze
From The Rett Center, Department of Pediatrics, Baylor College of Medicine, Houston, TX
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Pediatrics (1993) 91 (2): 445–450.
Article history
Received:
June 04 1992
Accepted:
October 06 1992
Citation
Claudia A. Kozinetz, Martha L. Skender, Nancy MacNaughton, Mary Jane Almes, Rebecca J. Schultz, Alan K. Percy, Daniel G. Glaze; Epidemiology of Rett Syndrome: A Population-Based Registry. Pediatrics February 1993; 91 (2): 445–450. 10.1542/peds.91.2.445
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