Preferred Label : Renal pseudohypoaldosteronism type 1;
ICD-11 definition : Renal pseudohypoaldosteronism type 1 (renal PHA1) is a mild form of primary mineralocorticoid
resistance restricted to the kidney. Clinical expression: in general, patients present
with a salt wasting syndrome in the neonatal period, with weight loss, failure to
thrive, vomiting and dehydration. Occasionally, polyhydramnios has been noted. Symptoms
of renal PHA1 usually improve in early childhood and older children are generally
asymptomatic with normal growth and psychomotor development.;
Origin ID : 1829275943;
Automatic exact mappings (from CISMeF team)
Renal pseudohypoaldosteronism type 1 (renal PHA1) is a mild form of primary mineralocorticoid
resistance restricted to the kidney. Clinical expression: in general, patients present
with a salt wasting syndrome in the neonatal period, with weight loss, failure to
thrive, vomiting and dehydration. Occasionally, polyhydramnios has been noted. Symptoms
of renal PHA1 usually improve in early childhood and older children are generally
asymptomatic with normal growth and psychomotor development.
