Triple-A syndrome
Medical condition / From Wikipedia, the free encyclopedia
Dear Wikiwand AI, let's keep it short by simply answering these key questions:
Can you list the top facts and stats about Triple-A syndrome?
Summarize this article for a 10 year old
Triple-A syndrome or AAA syndrome is a rare autosomal recessive congenital disorder. In most cases, there is no family history of AAA syndrome.[2] The syndrome was first identified by Jeremy Allgrove and colleagues in 1978; since then just over 100 cases have been reported.[3] The syndrome is called Triple-A due to the manifestation of the illness which includes achalasia (a dysfunction of the esophagus), addisonianism (adrenal insufficiency of primary type), and alacrima (insufficiency of tears). Alacrima is usually the earliest manifestation.[4] Neurodegeneration or atrophy of the nerve cells and autonomic dysfunction may be seen in the disorder; therefore, some have suggested the disorder be called 4A syndrome.[5] It is a progressive disorder that can take years to develop the full-blown clinical picture.[6] The disorder also has variability and heterogeneity in presentation.[7]
Triple A syndrome | |
---|---|
Other names | Achalasia–addisonianism–alacrima syndrome or Allgrove syndrome[1] |
MRI of the brain of 12-year-old boy with triple-A syndrome showing hypoplastic lacrimal glands (yellow arrows) | |
Specialty | Endocrinology |