PhenomeGene — Human Disease Gene Website SeriesSLC9A3

Clinical Characteristics

Autosomal-recessive congenital secretory sodium diarrhea (CSD) is caused by biallelic loss‐of‐function or strongly hypomorphic SLC9A3mutations, presenting with polyhydramnios antenatally, neonatal watery sodium‐rich diarrhea, metabolic acidosis, growth failure, and sometimes later bowel inflammation.