Ionic balance defects like: mechanosensory protein (PIEZO1) and the Gardos channel (KCNN4) defects are associated with dehydrated hereditary stomatocytosis 1 and 2, respectively; Rh-associated glycoprotein (RhAG) defects with overhydrated stomatocytosis; anion transporter (SLC4A1) defects with increased permeability to cations at low temperatures; adenosine triphosphate-binding cassette family member (ABCB6) defects with familial pseudohyperkalamia while glucose transporter(GLUT1) defects manifest with mental retardation, seizures, hepatosplenomegaly and cryohydrocytosis (20, 21, 22). This evidence concerns the gene KCNN4 and Hepatosplenomegaly.