Autosomal recessive ichthyosis	Genetic intellectual disability	Hereditary nephropathy	Multiple system malformation syndrome	Renal impairment	Short stature disorder
\|	\|	\|	\|	\|	\|

Ichthyosis, intellectual disability, dwarfism, renal impairment syndrome (disorder)
	Ichthyosis, intellectual disability, dwarfism, renal impairment syndrome
	Passwell Goodman Siprkowski syndrome
	Ichthyosis-intellectual disability-dwarfism-renal impairment syndrome is characterized by nonbullous congenital ichthyosis, intellectual deficit, dwarfism and renal impairment. It has been described in four members of one Iranian family. Transmission is autosomal recessive.

Id	733097003
Status	Primitive

Associated morphology	Hyperkeratosis
Finding site	Entire skin
Occurrence	Congenital
Pathological process	Pathological developmental process

Has interpretation	Impaired
Interprets	Renal function

Finding site	Kidney structure
Occurrence	Congenital
Pathological process	Pathological developmental process

Has interpretation	Abnormal
Interprets	Keratinization

Has interpretation	Impaired
Interprets	Intellectual ability

Has interpretation	Impaired
Interprets	Adaptation behavior

Has interpretation	Below reference range
Interprets	Body height

SNOMED CT to Orphanet simple map

RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set

Target	Q80.9
Term	Congenitale ichthyose, niet gespecificeerd

Target	F79.9
Term	Niet gespecificeerde zwakzinnigheid; Zonder vermelding van gedragsstoornissen

Target	E34.3
Term	Kleine gestalte, niet elders geclassificeerd

Target	N19
Term	Niet gespecificeerde nierinsufficiëntie

SNOMED CT to ICD-10 extended map

Target	Q87.1
Rule	TRUE
Advice	ALWAYS Q87.1 \| POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
Correlation	SNOMED CT source code to target map code correlation not specified