Allgrove’s Syndrome: Two Case Reports and Review of Literature
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 Present...
| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
| Published: |
Mazandaran University of Medical Sciences
2015-07-01
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| Series: | Journal of Pediatrics Review |
| Online Access: | http://dx.doi.org/10.17795/jpr-2653 |
| _version_ | 1818041615963389952 |
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| author | Daniel Zamanfar Elika Shokri Shiva Shadani Soheila Shahmohammadi |
| author_facet | Daniel Zamanfar Elika Shokri Shiva Shadani Soheila Shahmohammadi |
| author_sort | Daniel Zamanfar |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-12-10T08:33:15Z |
| format | Article |
| id | doaj.art-22f8916eaa8c46338418e969950e9ceb |
| institution | Directory Open Access Journal |
| issn | 2322-4401 2322-4398 |
| language | English |
| last_indexed | 2024-12-10T08:33:15Z |
| publishDate | 2015-07-01 |
| publisher | Mazandaran University of Medical Sciences |
| record_format | Article |
| series | Journal of Pediatrics Review |
| spelling | doaj.art-22f8916eaa8c46338418e969950e9ceb2022-12-22T01:56:02ZengMazandaran University of Medical SciencesJournal of Pediatrics Review2322-44012322-43982015-07-013210.17795/jpr-2653Allgrove’s Syndrome: Two Case Reports and Review of LiteratureDaniel Zamanfar0Elika Shokri1Shiva Shadani2Soheila Shahmohammadi3Diabetes Research Center, Mazandaran University of Medical Sciences, Sari, IR IranFaculty of Medicine, Mazandaran University of Medical Sciences, Sari, IR IranFaculty of Medicine, Mazandaran University of Medical Sciences, Sari, IR IranMazandaran Pediatric Infectious diseases Research Center (MPIDRC), Mazandaran University of Medical Sciences, Sari, IR IranIntroduction: 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.http://dx.doi.org/10.17795/jpr-2653 |
| spellingShingle | Daniel Zamanfar Elika Shokri Shiva Shadani Soheila Shahmohammadi Allgrove’s Syndrome: Two Case Reports and Review of Literature Journal of Pediatrics Review |
| title | Allgrove’s Syndrome: Two Case Reports and Review of Literature |
| title_full | Allgrove’s Syndrome: Two Case Reports and Review of Literature |
| title_fullStr | Allgrove’s Syndrome: Two Case Reports and Review of Literature |
| title_full_unstemmed | Allgrove’s Syndrome: Two Case Reports and Review of Literature |
| title_short | Allgrove’s Syndrome: Two Case Reports and Review of Literature |
| title_sort | allgrove s syndrome two case reports and review of literature |
| url | http://dx.doi.org/10.17795/jpr-2653 |
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