Growth failure and outcome in Rett syndrome: Specific growth references

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Growth failure and outcome in Rett syndrome: specific growth references.

OBJECTIVES Prominent growth failure typifies Rett syndrome (RTT). Our aims were to 1) develop RTT growth charts for clinical and research settings, 2) compare growth in children with RTT with that of unaffected children, and 3) compare growth patterns among RTT genotypes and phenotypes. METHODS A cohort of the RTT Rare Diseases Clinical Research Network observational study participants was re...

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Feeding experiences and growth status in a Rett syndrome population.

OBJECTIVES Feeding difficulties in Rett syndrome are complex and multifactorial. In this study, we describe the feeding experiences in Rett syndrome and examine the factors affecting growth. MATERIALS AND METHODS Using questionnaire data related to a population-based cohort, ages 2 to 29 years (n = 201), we measured the feeding experiences, growth, and factors affecting growth (enteral nutrit...

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Reduced nerve growth factor in Rett syndrome postmortem brain tissue.

To determine whether reduced nerve growth factor (NGF) and/or its high affinity receptor, trkA, play a role in the pathophysiology of Rett syndrome (RS), we used immunohistochemistry in paraffin-embedded human autopsy brain tissue, to quantify NGF and trkA levels within the frontal cortex of 9 RS females and 10 female controls of similar age. The results showed a significant reduction of NGF ex...

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Growth failure secondary to moyamoya syndrome.

We describe a boy who presented at the age of 7 years with short stature due to hypopituitarism. Six months after starting appropriate hormone replacement treatment at the age of 8 he suffered his first generalised convulsion. Further neuroradiological investigation led to the diagnosis of moyamoya syndrome.

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Metabolic Fingerprints of Altered Brain Growth, Osmoregulation and Neurotransmission in a Rett Syndrome Model

BACKGROUND Rett syndrome (RS) is the leading cause of profound mental retardation of genetic origin in girls. Since RS is mostly caused by mutations in the MECP2 gene, transgenic animal models such as the Mecp2-deleted ("Mecp2-null") mouse have been employed to study neurological symptoms and brain function. However, an interdisciplinary approach drawing from chemistry, biology and neuroscience...

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ژورنال

عنوان ژورنال: Neurology

سال: 2012

ISSN: 0028-3878,1526-632X

DOI: 10.1212/wnl.0b013e31826e9a70