TO THE EDITOR: Genetic factors have been suggested to play an important role in the development of achalasia, and various candidate genes have been documented. ALADIN is one of the candidate genes. However, the study by Di Nardo et al has shown that no pathogenic mutations were detected in the ALADIN gene of 41 patients with isolated achalasia. In addition, Allgrove syndrome is a rare disorder,...

BACKGROUND Allgrove syndrome is a rare autosomal recessive condition characterized by adrenal insufficiency, achalasia, alacrima and occasionally autonomic disturbances. Mutations in the AAAS gene, on chromosome 12q13 have been implicated as a cause of this disorder. CASE(S) PRESENTATION We present various manifestations of this syndrome in two related families each with two affected siblings...

Triple A (Allgrove) syndrome is characterized by achalasia, alacrima, adrenal abnormalities and a progressive neurological syndrome. Affected individuals have between two and four of these relatively common clinical problems; hence the diagnosis is often difficult in all but the classical presentation. The inheritance is autosomal recessive, and most cases of triple A have no family history. Us...

Allgrove's or "4 A" syndrome is a rare autosomal recessive condition with alacrima, achalasia, autonomic disturbance, and ACTH insensitivity among other features. Recent studies have identified mutations in the AAAS, a candidate gene on chromosome 12q13 in such patients. Manifestations in adult patients are rarely reported. The syndrome usually presents during the first decade of life with dysp...

Allgrove syndrome is a very rare seen disorder with an autosomal recessive trait. The three characteristics of disease are alacrima, achalasia and adrenal failure. Alacrimia is the first manifestation in most cases. Achalasia and adrenal failure are seen later. Neurological involvement is rare and usually occurs in older age. A woman 22 years old admitted to our hospital with adrenal failure cr...

Triple A syndrome (Allgrove syndrome) is a rare inherited autosomal recessive disease with a typical triad including adrenocorticotrophic-hormone-resistant glucocorticoid insufficiency, reduced or absent tearing (alacrima) and achalasia and a wide range of symptoms can be detected due to multi organ involvement. This report describes the case of a Triple Asyndrome, a12 year-old boy with a histo...

triple a syndrome (allgrove syndrome) is a rare inherited autosomal recessive disease with a typical triad including adrenocorticotrophic-hormone-resistant glucocorticoid insufficiency, reduced or absent tearing (alacrima) and achalasia and a wide range of symptoms can be detected due to multi organ involvement. this report describes the case of a triple asyndrome, a12 year-old boy with a histo...

BACKGROUND Allgrove syndrome, or triple "A" syndrome (3A syndrome), is a rare autosomal recessive syndrome with variable phenotype, and an estimated prevalence of 1 per 1,000,000 individuals. Patients usually display the triad of achalasia, alacrima, and adrenocorticotropin (ACTH) insensitive adrenal insufficiency, though the presentation is inconsistent. CASE REPORT Here, the authors report a ...