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Organ growth in Rett syndrome: a postmortem examination analysis
D D Armstrong1, J K Dunn, R J Schultz
1Baylor Rett Center and Department of Pathology, Baylor College of Medicine, Houston, Texas 77030, USA.
Pediatric Neurology
|March 19, 1999
Summary
Rett syndrome causes growth failure, particularly affecting brain development, distinguishing it from other developmental disorders. Organ growth in Rett syndrome shows a unique pattern, with the brain being preferentially impacted.
Area of Science:
- Neurology
- Developmental Biology
- Genetics
Background:
- Rett syndrome is a rare neurological disorder affecting females, characterized by severe cognitive and motor impairments.
- Early clinical signs include decelerated head growth and altered somatic development postnatally.
- The etiology of Rett syndrome remains largely unknown.
Purpose of the Study:
- To investigate the unique growth patterns in Rett syndrome by comparing organ weights.
- To determine if growth failure in Rett syndrome is distinct from other developmental disorders.
- To assess the preferential impact of growth deficits on the brain.
Main Methods:
- Analysis of organ weights from 39 postmortem studies of individuals with Rett syndrome.
- Comparison of these weights with normal organ weights for age- and height-matched females.
- Evaluation of occipitofrontal circumference (OFC) to assess brain growth deceleration.
Main Results:
- Rett syndrome exhibits a unique growth failure pattern compared to Down and Turner syndromes.
- Brain growth, indicated by OFC, decelerates postnatally, with no significant weight increase after age 4.
- Growth of heart, kidneys, liver, and spleen decelerates after 8-12 years but remains height-appropriate; adrenal weights are normal.
Conclusions:
- The brain appears to be preferentially affected in Rett syndrome, despite generalized growth deficits.
- Organ weight analysis reveals a distinct growth trajectory in Rett syndrome.
- Further research is needed to understand the underlying mechanisms of preferential brain growth impairment.
