Pre-hospital emergency care recommendations Call for a patient suffering from Bourneville’s tuberous sclerosis

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چکیده

Definition: Tuberous sclerosis is an autosomal dominant multisystemic genetic condition that can affect many organs: brain, skin, eye, heart, lungs and the kidney. It affects one in 7 to 8,000 people. Its main complications involve the nervous system (convulsions, intracranial hypertension), the kidneys (rupture of cysts and angiomyolipomas) and the lungs of adult females (rupture of bullae with lymphangioleiomyomatosis). It can be difficult to detect the clinical signs of these complications due to the intellectual deficiency or psychiatric problems that can be associated with the condition. A full clinical evaluation must be done if there is the slightest suspicion.

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تاریخ انتشار 2012