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A study on clinical manifestations of tuberous sclerosis
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نویسنده
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Puri Neerja
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منبع
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journal of pakistan association of dermatologists - 2013 - دوره : 23 - شماره : 4 - صفحه:365 -370
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چکیده
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Objective: to document clinical features in patients of tuberous sclerosis. patients and methods: twenty clinically diagnosed cases of tuberous sclerosis were assessed clinically for features of tuberous sclerosis. results: males outnumbered females and male: female ratio was 2.3:1. adenoma sebaceum was the commonest (95%) cutaneous feature of tuberous sclerosis followed by ash leaf macule and shagreen patch each seen in 90% patients followed by koenen’s tumour seen in 75% patients. positive family history was seen in 25% patients. regarding the clinical spectrum of tuberous sclerosis it was seen that epileptic fits and cardiac complications were seen in 50% patients each, eye abnormalities including retinal astrocytomas were seen in 40% patients, mental retardation was seen in 25% patients followed by astrocytomas and pulmonary disease seen in 20% patients each. conclusion: tuberous sclerosis can present with diverse cutaneous and systemic manifestations. dermatologist may assist in early diagnosis of this multisystem genodermatosis.
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کلیدواژه
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Tuberous sclerosis ,shagreen patch ,adenoma sebaceum ,periungual fibroma ,angiofibromas.
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آدرس
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Punjab Health Systems Corporation, India
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Authors
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