A congenital autosomal recessive form of adrenal insufficiency that is characterized by very low plasma ACTH and CORTISOL levels with no significant response to CORTICOTROPIN-RELEASING HORMONE. Affected neonatal patients may present with OBSTRUCTIVE JAUNDICE, severe HYPOGLYCEMIA, and SEIZURES. Mutations in the TBX19 gene have been identified. OMIM: 201400
