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Hirschsprung disease (aetiology (absence or dysfunction of... (fibronectin…
Hirschsprung disease
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pathophysiology
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in the absence of ENS reflexes, control of the intestinal smooth muscle is extrinsic, with the cholinergic (excitatory) predominant over the adrenergic (inhibition) leading to an overall increase in sm ms tone.
the loss of intrinsic enteric relaxing impulses, the increased ms tone is unopposed --> imbalance of sm ms contractility --> uncoordinated peristalsis --> functional obstruction
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prognosis
90% satisfaction following repair, but ongoing issues with continence
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defintion
developmental disorder characterised by the absence of the ganglia of the distal colon, resulting in a functional obstruction.
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