Duplication 8q12: confirmation of a novel recognizable phenotype with duane retraction syndrome and developmental delay

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Duplication of 8q12 encompassing CHD7 is associated with a distinct phenotype but without duane anomaly.

Interstitial duplications of 8q12 encompassing CHD7 have recently been described as a new microduplication syndrome. Three 8q12 duplications have been reported with shared recognizable phenotype: Duane anomaly, developmental delay and dysmorphic facial features. We identified a 2.7 Mb duplication on chromosome 8q12 with SNP-array in a patient with growth delay, congenital heart defects, ear ano...

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Duane retraction syndrome in a Nigerian child

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Simultaneous Occurrence of Duane Retraction Syndrome with Marfan Syndrome

Marfan syndrome (MFS) is an autosomal dominant disorder of connective tissue, while Duane retraction syndrome (DRS) is a congenital cranial dysinnervation disorder (CCDD) which can be transmitted as autosomal dominant disorder in 5-10% of patients. In this paper, we present an 8-year-old girl who presented with left eye DRS and bilateral subluxation of the lens associated with MFS in absence of...

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Properties of saccades in Duane retraction syndrome.

PURPOSE To improve understanding of the binocular control of saccades by making high-resolution eye movement recordings in patients with unilateral Duane retraction syndrome (DRS) type I (marked by congenital absence of the sixth cranial nerve). METHODS Binocular eye movements were recorded in four patients in binocular viewing conditions during a saccade task. RESULTS Affected-side gaze sh...

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ژورنال

عنوان ژورنال: European Journal of Human Genetics

سال: 2012

ISSN: 1018-4813,1476-5438

DOI: 10.1038/ejhg.2011.243