True about nephrogenic diabetes insipidus in children –
In nephrogenic diabetes insipidus, level of the ADH is normal.
The defects lies in the distal tubule where the cells fail to respond to ADH.
Diabetes inspidus is associated with wolfram syndrome or DIDMOD syndrome not wilm's tumour.
Wolfram syndrome → Diabetes insidus, Diabetes mellitus, Optic atrophy, Deafness.
Decreased ADH secretion
Receptor insensitivity in kidney tubules
Decreased ADH
Commonly associated with WILM's tumor