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Allgrove syndrome is one of the rare hereditary disorders with autosomal recessive inheritance. Mutation in AAAS gene on chromosome 12q13 encoding ALADIN protein leads to alacrima, achalasia, adrenal insufficiency and multiple rare features. We present a case of allgrove syndrome diagnosed on history and examination with alacrima evident at the age of 6 months, achalasia and adrenal insufficiency at the age of 3 years and 11 years respectively, dental variations i.e., missing of permanent premolars and malformed lateral incisors at the age of 10-11 years. In this particular case report, association of this syndrome with congenitally missed permanent premolars and malformed lateral incisors has been established which is a novel and rare feature observed in allgrove syndrome patients. Patients with allgrove syndrome usually manifest alacrima, achalasia and adrenal insufficiency during the first two decades of life.

RAHMAN AHMAD, MARYAM, MUNEEBA AKBAR, Muhammad Ashfaq Zafar. (2021) A Case Report of Allgrove Syndrome with Hypodontia, Pakistan Pediatric Journal, Volume 45, Issue 1.
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