Is it certain that citrin deficiency patients will develop AACD* at some point in their lives?

Based on the literature and personal communications, not all patients with citrin deficiency will develop Adolescent and Adult Citrin Deficiency (AACD)*. In Japan, where there is a high awareness of AACD*, its population frequency is 1:100,000 to 1:200,000, whereas the frequency of NICCD is approximately 1:17,000. It is not certain yet what actually triggers the onset of AACD*.

* Previously known as Citrullinemia type 2 (CTLN2)