Autosomal recessive hereditary disorder	Congenital anomaly of eye	Developmental hereditary disorder	Genetic disease of glomerulus	Hereditary disorder of the visual system	Hereditary nephropathy	Steroid-resistant nephrotic syndrome
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Pierson syndrome (disorder)
	Pierson syndrome
	Microcoria and congenital nephrosis syndrome
	A rare primary glomerular disease characterized by the association of congenital nephrotic syndrome, early onset renal failure and ocular anomalies with microcoria and severe neurodevelopment deficits.

Id	723449004
Status	Primitive

Finding site	Glomerulus structure
Occurrence	Congenital
Pathological process	Pathological developmental process

Associated morphology	Morphologically abnormal structure
Finding site	Eye structure
Occurrence	Congenital
Pathological process	Pathological developmental process

Has interpretation	Below reference range
Interprets	Albumin measurement

Has interpretation	Above reference range
Interprets	Urine protein measurement

SNOMED CT to Orphanet simple map

RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set

Target	N04.8
Term	Nefrotisch syndroom; Anders gespecificeerd

Target	Q13.2
Term	Overige congenitale misvormingen van iris

SNOMED CT to ICD-10 extended map

Target	Q13.8
Rule	TRUE
Advice	ALWAYS Q13.8 \| POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
Correlation	SNOMED CT source code to target map code correlation not specified