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allgrove’s syndrome: two case reports and review of literature
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نویسنده
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zamanfar daniel ,shokri elika ,shadani shiva ,shahmohammadi soheila
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منبع
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journal of pediatrics review - 2015 - دوره : 3 - شماره : 2 - صفحه:1 -5
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چکیده
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introduction: allgrove’s syndrome (as) is a rare cause of adrenal insufficiency inherited in an autosomal recessive pattern. usually the disease is manifested during the first decade of life with severe hypoglycaemia, which can lead to sudden death.case presentation: here we described two cases of allgrove’s syndrome; the first case was an 8-year-old male admitted to hospital for evaluation of seizure and gait abnormality and the second case was a 4-year-old boy admitted for evaluation of hyperpigmentation of his skin and buccal mucosa for eight months, also we made a review of literature.conclusions: alacrima is considered to be the earliest clinical manifestation of triple a syndrome and early recognition of glucocorticoid deficiency would prevent hypoglycaemic convulsions, neurological sequelae and death. a carful replacement of glucocorticoids is critical to avoid adrenal crisis and allow normal growth and development.
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کلیدواژه
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syndrome ,adrenal insufficiency ,alacrima ,case report
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آدرس
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diabetes research center, mazandaran university of medical sciences, sari, ir iran, IR Iran, faculty of medicine, mazandaran university of medical sciences, sari, ir iran, IR Iran, faculty of medicine, mazandaran university of medical sciences, sari, ir iran, IR Iran, mazandaran pediatric infectious diseases research center (mpidrc), mazandaran university of medical sciences, sari, ir iran, IR Iran
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Authors
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