Allgrove Syndrome, also known as the Triple “A” or the AAA Syndrome is one of the many rare autosomal recessive congenital disorders well-studied by doctors. This syndrome was discovered by Dr. Jeremy Allgrove, a pediatric endocrinologist, together with his colleagues.
Triple A or AAA Syndrome literally defines by three disorders which begins with the letter A. These are Achalasia, Addisonianism and Alacrima. Let us discuss one by one each component.
- Achalasia. This is characterized by failure of the smooth muscles to relax specifically the smooth muscles located in the lower esophageal sphincter. This eventually leads to delayed food propulsion going to the stomach and subsequent enlargement of the esophageal area. Usual symptom include dysphagia or difficulty or discomfort when swallowing, vomiting and for pediatric patients, failure to thrive.
- Addisionianism or Addison’s disease or Adrenal insufficiency which is characterized by deficiency in the production of steroid hormones specifically cortisol. In some, production of the mineralocorticoid, aldosterone, is also affected resulting to impaired water retention, sodium regulation and potassium secretion. A patient may present with hypoglycemic seizures and shock.
- Alacrima. This is characterized by decreased or there is abnormality in the production of tears. Patients usually presents with ocular or eye symptoms and reveals decrease to no tears when crying.
Treatment modalities for patients diagnosed with Allgrove or Triple “A” or AAA Syndrome is as complex as the syndrome itself. For achalasia, this requires surgical intervention depending on the severity and the affected area. For Addison’s disease, steroid or mineralocorticoid supplement, depending on what is deficient is the best treatment plan. For alacrima, continuous eye lubricants must be applied.