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Jul 30, 2023, 9 tweets

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‘CHARGE’ is an acronym that describes a constellation of clinical features including

Coloboma,

Heart defects,

choanal Atresia,

Retardation (of growth and/or development),

Genitourinary malformation and

Ear abnormalities.

👉complex genetic syndrome, owing to the wide range of tissues/systems affected by mutations in the CHD7 gene

CHARGE syndrome remains a clinical diagnosis.

Absent/hypoplastic semicircular canals are present in the majority of patients with CHARGE and are highly predictive of the presence of a CHD7 mutation.

Early involvement of a cardiologist, ophthalmologist, endocrinologist, geneticist and ear, nose and throat surgeon is recommended.

***

Coloboma of the eye

Complete thymic aplasia rarely occurs but leads to severe combined immune deficiency.

Persistent lymphopenia in a patient with CHARGE must always be investigated

***

Choanal atresia...

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