MIM 208 540, 615 415]

(reno-hepato-pancreatic dysplasia)

Extremely rare. Autosomal recessive transmission of a mutation of the NHHP3 gene on 3q22 (type 1) or of the NEK8 gene on 17q11.2 (type 2). Other mutations in the NHHP3 gene cause nephronophthisis, see this topic).

Association of renal, liver and pancreatic cysts, often lethal in the neonatal period.

Anesthetic implications: 

check renal and hepatic function

References : 

-         Vankalakunti M, Gupta K, Kakkar N, Das A.  
Renal-hepato-pancreatic dysplasia syndrome (Ivemark syndrome). 
Diagn Pathol 2007 : 2 :24

Updated: December 2020