Very rare (< 1/1.106). Very low serum albumin concentration. There is a compensatory increase in the other plasma proteins so

that the total protein level is barely decreased. There is little peripheral edema except in the neonatal period. It is possible that this anomaly causes miscarriages and deaths in utero, which would explain the rarity of the condition.

From a biological point of view, high cholesterol, decrease of total calcium (but the ionized Ca is normal), increase in the total thyroxine level (but its free fraction is normal). Adult lipodystrophy of the limbs seems associated.

Anesthetic implications: 

increased effect of the drugs highly bound to albumin

References : 

-         Koot BGP, Houwen R, Pot D-J. 
Congenital analbuminaemia : biochemical and clinical implications. A case report and literature review. 
Eur J Pediatr 2004; 163: 664-70.

Updated: November 2019